PN 76001 RN 00356 AN 77263078 AU Laszlo-A. Penzes-P. Gyurkovits-K. TI Mucoviscidosis: total amylase activity of serum and mixed saliva in homozygous and heterozygous subjects. SO Acta-Paediatr-Acad-Sci-Hung. 1976. 17(4). P 293-6. MJ AMYLASES: an. CYSTIC-FIBROSIS: me. MN ADULT. AMYLASES: bl. CHILD. CYSTIC-FIBROSIS: bl, fg. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MALE. SALIVA: an. AB Total amylase activity of serum and mixed saliva was studied in homozygotes and heterozygotes for mucoviscidosis and in healthy subjects. Mean serum total activity was 269.0 +/- 113.7 U/l in the homozygotes, exceeding in nearly 50% of the cases the values given in the literature and those observed in the normal controls. The difference against the control group was significant (P less than 0.05). Mean serum total amylase activity of heterozygotes agreed with the mean value for the healthy group (203.5 +/- 79.5 U/l) without a significant difference. Total amylase activity in the saliva of homozygotes (148.700 +/- 65.700 U/l) was higher than in the heterozygotes /118.300 +/- 74.200 U/l) of the healthy children (51.700 +/- 26.500 U/l). The difference between the homozygous and healthy groups was strongly significant (P less than 0.01), and that between the heterozygous group and the combined healthy children and adult groups was also significant (P less than 0.05). In the heterozygotes, salivary amylase activity was slightly elevated but not significantly different from the control group and did not result in a change in serum total amylase activity. RF 001A CESKA M EXPERIENTIA 25 555 969 001B CESKA M CLIN CHIM ACTA 26 437 969 001C CESKA M CLIN CHIM ACTA 26 445 969 002 CHERNICK WS J PEDIATR 65 694 964 003 DI SANTAGNESE PA PEDIATRICS 22 507 958 004 MCDOUGALL C PEDIATRICS 5 114 950 005 GAUTIER E HELV PAEDIATR ACTA 17 415 962 006 GYURKOVITS K ACTA PAEDIATR ACAD SCI HUNG 15 49 974 007 HADORN B J PEDIATR 73 39 968 008 KAMARYT J Z KLIN CHEM KLIN BIOCHEM 8 564 970 009 KLEINBAUM H Z KINDERHEILK 90 7 964 010 MANDEL ID AM J DIS CHILD 113 431 967 011 MAYER WB ACTA PAEDIATR SCAND 63 389 974 012 NICORY C BIOCHEM J 6 387 922 014 ROSSITER MA ACTA PAEDIATR SCAND 63 389 974 015 SEARCY RL AM J CLIN PATHOL 46 582 966 016 SZCZEPANSKI Z Z KINDERHEILK 113 297 972 PN 76002 RN 00357 AN 77062364 AU Baran-D. Loeb-H. Mozin-M-J. TI Efficiency of two pancreatic extracts in patients with cystic fibrosis. SO Acta-Paediatr-Belg. 1976 Apr-Jun. 29(2). P 117-22. MJ CYSTIC-FIBROSIS: me. INTESTINAL-ABSORPTION: de. LIPIDS: me. NITROGEN: me. PANCREATIC-EXTRACTS: tu. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt. FEMALE. HUMAN. INFANT. MALE. AB Nitrogen- and lipid absorption coefficients were studied in 62 patients with cystic fibrosis, aged 2/12 to 12 years, and compared with the results obtained in 20 normal children. In 59 of the 62 patients nitrogen- and lipid absorption were grossly abnormal. Age-linked differences were not found. The efficacy of two pancreatic enzymes preparations was found to be similar. Both preparations produced the same significant improvement in the absorption of proteins and lipids. RF 001 BEST EB SOUTH MED J 53 1091 960 002 HAINES RD JAMA 180 1000 962 003 HARRIS R ARCH DIS CHILD 30 424 955 004 JENSEN SB ACTA PAEDIATR SCAND 45 70 956 005 LAPEY A J PEDIATR 84 328 974 006 ROSS CAC ARCH DIS CHILD 30 316 955 007 SHOHL AT J PEDIATR 23 267 943 008 SNEDECOR GW STATISTICAL METHODS 967 009 TREMOLIERES J CAHIERS NUTR DIETET 1 13 966 CT 1 MALMENDIER CL HELV PAEDIATR ACTA 32 115 977 2 ROGIERS V PEDIATR RES 14 1088 980 3 ROGIERS V CLIN CHIM ACTA 105 105 980 4 TOLCKMITT W KLIN PAEDIATR 193 365 981 5 ROGIERS V EUR J PEDIATR 141 39 983 PN 76003 RN 00358 AN 76133375 AU Skorecki-K. Levison-H. Crozier-D-N. TI Bronchial lability in cystic fibrosis. SO Acta-Paediatr-Scand. 1976 Jan. 65(1). P 39-44. MJ BRONCHI: pp. CYSTIC-FIBROSIS: pp. EXERTION. MN ADOLESCENCE. ADULT. ASTHMA: pp. CHILD. FEMALE. FORCED-EXPIRATORY-VOLUME. HUMAN. HYPERSENSITIVITY-IMMEDIATE. MALE. MAXIMAL-MIDEXPIRATORY-FLOW-RATE. PEAK-EXPIRATORY-FLOW-RATE. RESPIRATORY-AIRFLOW. VITAL-CAPACITY. AB We investigated the airway's response to exercise in cystic fibrosis (CF). Due to the reported high incidence of allergy in CF we tried to demonstrate the well documented post-exercise bronchospasm of asthma in patients with CF. Subjects ran on a treadmill for 6 minutes at a speed of 3 miles per hour and a gradient of 15 degrees. For the 12 controls studied, the mean % rise in peak expiratory flow rate (PEFR) during exercise was 4.7% and the mean % fall in PEFR during recovery was 0.7%. For the eight atopic asthmatics studied the mean % rise in PEFR was 12.2% and the mean % fall in PEFR was 33.7%. For the 29 patients with CF, the mean % rise in PEFR was 20.3% and the mean % fall in PEFR was 3.1%. Patients with CF showed no statistically significant post-exercise constriction compared with controls, while asthmatic patients showed a post-exercise % fall in PEFR significantly greater than seen in controls (p < 0.001) or in CF patients (p < 0.001). No patient with CF showed post-exercise bronchospasm, nor was there any difference between patients with CF who had positive skin tests to common allergens and those who did not in their pattern of bronchial liability. It is suggested that the general desensitization therapy should not be instituted in patients with CF merely on the basis of positive skin tests, without more stringent criteria such as characteristic seasonal variation in respiratory symptomatology, family history of atopy and positive bronchial inhalation provocation tests. RF 001 DAY G ARCH DIS CHILD 48 355 973 002 GODFREY S J ALLERGY CLIN IMMUNOL 52 199 973 003 HEIMLICH EM J ALLERGY CLIN IMMUNOL 37 103 966 004 JONES RS BR J DIS CHEST 56 78 962 005 JONES RS ARCH DIS CHILD 38 539 963 006 KULCZYCKI LL JAMA 175 358 961 007 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 008 SILVERMAN M ARCH DIS CHILD 47 882 972 CT 1 MELLIS CM PEDIATRICS 61 446 978 2 MITCHELL I J PEDIATR 93 744 978 3 ANON LANCET 1 708 979 4 PRICE JF CLIN ALLERGY 9 563 979 5 ZAMBIE MF ANN ALLERGY 42 290 979 6 LARSEN GL AM REV RESPIR DIS 119 399 979 7 SVENONIUS E RESPIRATION 40 226 980 8 HOLZER FJ ARCH DIS CHILD 56 455 981 9 LOUGHLIN GM CHEST 79 206 981 10 EGGLESTON PA J ALLERGY CLIN IMMUNOL 73 666 984 11 RICHARDSON VF ACTA PAEDIATR SCAND 73 75 984 12 CANNY GJ ACTA PAEDIATR SCAND 74 451 985 13 SLY RM MED SCI SPORTS EXER 18 314 986 PN 76004 RN 00359 AN 77084757 AU Waring-W-W. TI Current management of cystic fibrosis. SO Adv-Pediatr. 1976. 23. P 401-38. (REVIEW). MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. AEROSOLS. AMYLASES: tu. BRONCHODILATOR-AGENTS: tu. CALORIC-INTAKE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dh, di, dt. DIETARY-FATS. DIETARY-PROTEINS. DRAINAGE. HUMAN. INFANT. IRRIGATION. LIPASE: tu. PARENTERAL-FEEDING. PEPTIDE-HYDROLASES: tu. REVIEW. TRIGLYCERIDES. SUPPORT-U-S-GOVT-NON-P-H-S. VITAMINS: tu. WATER. EX Although forty years have passed since the discovery of the clinical entity called (for lack of a better name) cystic fibrosis, its treatment remains both symptomatic and controversial. Moreover, the efficacy of therapy has been difficult to evaluate for several reasons: the lack of a suitable animal model for therapeutic experimentation, the protean manifestations of the disease, the natural variations in its severity, the prolonged periods of observation that are required by its very chronicity and frequently the lack of satisfactory clinical parameters by which to score response. Stated in its simplest form, the treatment of cystic fibrosis is directed towards the long-term control of salt loss, malabsorption and bronchial obstruction, the management of several complications of the disease and the establishment of a positive therapeutic environment that minimizes anxiety and depression. The purpose of this review are to help the pediatric practitioner gain security in his day-to-day management of children with the disease and to advocate an effective working relationship between the practitioner and a regional cystic fibrosis or pediatric pulmonary center for long-term patient management. Problems in diagnosis, treatment of salt loss, the prevention and treatment of malabsorption, the prevention and treatment of bronchial obstruction, the prevention and treatment of certain complications, and the psychosocial support of the patient and family are discussed. RF 001 SARSFIELD JK ARCH DIS CHILD 50 463 975 002 WARING WW AM REV RESPIR DIS 104 166 971 003 GIBSON LE CLIN PEDIATR 12 450 973 004 GIBSON LE PEDIATRICS 23 545 959 005 ANON GAP CONF REP PROB SWEAT TESTI 975 006 ANON STUDY EVAL OF TEST FOR CF 975 007 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 008 HUANG NN GUIDE TO DRUG THERAPY IN PATI 974 009 ANON CF Q ANNOTATED REFERENCES 010 ANON DIRECTORY OF CF CARE TEACHING 011 SHWACHMAN H IN: SHIRKEY HC 651 975 012 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 013 ANON AM REV RESPIR DIS PART 2 110 1 974 014 MANGOS JA SCIENCE 158 135 967 015 BERGNER A PEDIATRICS 55 814 975 016 DOLAN TF JR AM REV RESPIR DIS 110 812 974 017 WARING WW MINI GUIDE TO LUNG DISEASE 1-4 018 ALLAN JD AM J DIS CHILD 126 22 973 019 KUO PT J CLIN INVEST 44 1924 965 020 GRACEY M ARCH DIS CHILD 44 401 969 021 GRACEY M ARCH DIS CHILD 45 445 970 022 OURSO SR MINI GUIDE TO LUNG DISEASE 8 023 OURSO SR MORE COOKING TREASURES 976 024 BARCLAY RPC ARCH DIS CHILD 50 490 975 025 MICHENER WM PEDIATR CLIN NORTH AM 17 373 970 026 FOX HA PEDIATRICS 52 14 973 027 HARRIS JA RESPIR CARE 20 1164 975 028 MELLINS RB AM REV RESPIR DIS SUPPL 110 137 974 029 MOTOYAMA EK IN: MANGOS JA 335 973 030 LORIN MI AM J PHYS MED 50 215 971 031 DENTON R DIS CHEST 28 123 955 032 MATTHEWS LW J PEDIATR 65 558 964 033 MATTHEWS LW PEDIATRICS 39 176 967 034 DOERSHUK CF PEDIATRICS 41 723 968 035 WOLFSDORF J PEDIATRICS 43 799 969 036 BAU SK PEDIATRICS 48 605 971 037 ALDERSON PO J PEDIATR 84 479 974 038 NORMAN AP PRACTITIONER 206 786 971 039 WARING WW PEDIATR RES 6 431 972 040 CHANG N AM REV RESPIR DIS 107 672 973 041 ROSENBLUTH M CLIN RES 19 805 971 042 MOTOYAMA EK PEDIATRICS 50 299 972 043 MATTHEWS LW IN: MANGOS JA 303 973 044 GIBSON LE AM REV RESPIR DIS SUPPL 110 100 974 045 CHANG N AM REV RESPIR DIS 106 867 972 046 LAWSON D BR MED J 1 317 965 047 SOLOMONS CC PEDIATRICS 47 384 971 048 LIEBERMAN J JAMA 205 312 968 049 WILLIAMS MH JR AM REV RESPIR DIS SUPPL 110 122 974 050 KULCZYCKI LL PROC INT CONG PEDIATR 13TH 577 971 051 RUCKER RW CF CLUB ABST 974 052 HACKETT RR ANESTHESIOLOGY 26 248 965 053 RAMIREZ RJ ANN INTERN MED 63 819 965 054 KYLSTRA JA AM REV RESPIR DIS 103 651 971 055 MUGGENBURG BA J APPL PHYSIOL 38 922 975 057 CEZEAUX G JR JAMA 199 15 967 058 BRAUNSTEIN MS CHEST 66 96 974 059 HUBER GL AM REV RESPIR DIS 104 337 971 060 MUGGENBURG BA AM REV RESPIR DIS 106 219 972 061 LAWSON D IN: LAWSON D PROC 5TH INT CF 225 969 062 MARKS MI J PEDIATR 79 822 971 063 HAWLEY HB CURR THER RES 16 414 974 064 HOFF GE SCAND J INFECT DIS 6 333 974 065 STERN RC CLIN PEDIATR 11 521 972 066 RUCKER RW PEDIATRICS 54 358 974 067 LIFSCHITZ MI AM J DIS CHILD 116 633 968 068 STOWE SM AM REV RESPIR DIS 111 611 975 069 ROYCE SW PEDIATRICS 8 255 951 070 MONSET-COUCHARD M CURR PROB PEDIATR NO 4 5 975 071 SYMCHYCH PS ARCH PATHOL 92 409 971 072 WHITMAN V PEDIATRICS 55 83 975 073 KELMINSON LL PEDIATRICS 39 24 967 074 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 CT 1 STERN RC AM REV RESPIR DIS 118 821 978 2 ROSENSTEIN BJ JAMA 240 1987 978 3 HOLSCLAW DS CLIN CHEST MED 1 407 980 4 SACK J ISR J MED SCI 16 417 980 5 CHRYMKO M AM J HOSP PHARM 37 713 980 6 GOTZ M EUR J RESPIR DIS 61 122 980 7 PASSERO MA CLIN PEDIATR 20 264 981 8 LAUGHLIN JJ PEDIATRICS 68 203 981 9 DAVID TJ LANCET 2 1204 982 10 MITCHELL EA AUST PAEDIATR J 18 40 982 11 THOMASSEN MJ J PEDIATR 104 352 984 12 KUHN RJ CLIN PHARMACY 4 555 985 PN 76005 RN 00360 AN 76181528 AU Nadler-H-L. TI Prenatal detection of genetic defects. SO Adv-Pediatr. 1976. 22. P 1-81. (REVIEW). MJ HEREDITARY-DISEASES: di. PRENATAL-DIAGNOSIS: mt. MN ADRENAL-HYPERPLASIA-CONGENITAL: di. ALPHA-FETOPROTEINS: an. AMINO-ACID-METABOLISM-INBORN-ERRORS: di. AMNIOCENTESIS: mt. AMNIOSCOPY: mt. CARBOHYDRATE-METABOLISM-INBORN-ERRORS: di. CENTRAL-NERVOUS-SYSTEM-DISEASES: di, fg. CHROMOSOME-ABNORMALITIES: di. CYSTIC-FIBROSIS: di. FEMALE. GENES-RECESSIVE. HEMOGLOBINOPATHIES: di. HUMAN. HYPERCHOLESTEROLEMIA: di, fg. LIPID-METABOLISM-INBORN-ERRORS: di. MUCOPOLYSACCHARIDOSIS: di. PREGNANCY. REVIEW. SEX-CHROMOSOME-ABNORMALITIES: di. ULTRASONICS: du. EX The intrauterine detection of genetic disorders represents one of the exciting advances in medicine. The major emphasis in this chapter will be a detailed review of the newer approaches toward antenatal detection of congenital malformations and hemoglobinopathies as well as an overview of the experience and problems of antenatal detection for chromosomal aberrations and inborn errors of metabolism. Approaches to prenatal diagnosis of genetic disorders include amniocentesis, visualization of the fetus, and fetal blood sampling. Prenatal detection of genetic disorders has become a reality. The area is evolving so rapidly that the major indications for intrauterine diagnosis have substantially changed within the past year. Prior to this time, maternal age, previous Down's syndrome, inborn errors of metabolism and X-linked recessive disorders were the primary criteria, with approximately 85% of the cases being used for cytogenetic analysis. Within the past year, in a number of laboratories, about half of the referrals for amniocentesis have been for central nervous system defects. Certainly with the development of technics for fetal blood sampling, this distribution of the frequencies of indication may, once again, change substantially. Most probably for the next few years cytogenetic abnormalities and neural tube defects will be the most common indications for amniocentesis. Amniocentesis will continue to be offered to many patients with inborn errors of metabolism and probably will be available for monitoring of hemoglobinopathies and other disorders of fetal blood as well as for accurate knowledge of the health of the fetus. RF 001 BALLANTYNE JW MANUAL OF ANTENATAL PATHOL HY 902 002 DORFMAN A ANTENATAL DIAGNOSIS 972 003 MILUNSKY A PRENATAL DIAGNOSIS OF HEREDIT 973 004 EMERY AEH ANTENATAL DIAGNOSIS OF GENET 973 005 HARRIS H PRENATAL DIAG SELECTIVE ABORT 974 006 BURTON BK AM J OBSTET GYNECOL 118 718 974 007 MILUNSKY A JAMA 230 232 974 009 DORAN TA AM J OBSTET GYNECOL 118 314 974 010 ROBINSON A AM J OBSTET GYNECOL 116 937 973 011 FREDA VJ BULL NY ACAD MED 42 475 966 012 QUEENAN JT CLIN OBSTET GYNECOL 9 491 966 013 BURNETT RG J IOWA MED SOC 58 130 958 014 LILEY AW NZ MED J 59 581 960 015 CREASMAN WT JAMA 204 949 968 017 FUCHS F CLIN OBSTET GYNECOL 9 565 966 019 JAVERT CT SPONTANEOUS AND HABITUAL ABOR 957 020 ANON NATIONAL AMNIOCENTESIS REG 021 GEYER VH Z KLIN CHEM KLIN BIOCHEM 8 145 970 022 TORNQVIST A ACTA OBSTET GYNECOL SCAND 50 79 971 023 SALAFSKY IS AM J OBSTET GYNECOL 111 1046 971 024 NATELSON S AMNIOTIC FLUID 974 025 NADLER HL J PEDIATR 74 132 969 026 NADLER HL LANCET 2 1277 969 027 SCHNECK L LANCET 1 582 970 028 RATTAZZI MC IN: DORFMAN A 207 972 029 NADLER HL LANCET 2 369 970 030 HUG G LANCET 1 1002 970 031 GERBIE AB AM J OBSTET GYNECOL 114 314 972 032 MELANCON S SCIENCE 173 627 971 033 BARNHISEL ML J CELL PHYSIOL 76 7 970 034 KABACK MM IN: DORFMAN A 81 972 035 NADLER HL BIOCHEM GENET 2 119 968 036 KABACK MM PEDIATR RES 5 366 971 037 UHLENDORF BW SCIENCE 160 1007 968 038 SHIH VE LANCET 2 45 970 039 SHIH VE CLIN CHIM ACTA 27 73 970 040 RYAN CA EXP CELL RES 71 388 972 041 NADLER HL N ENGL J MED 282 596 970 042 GERBIE AB AM J OBSTET GYNECOL 109 765 971 044 GOLBUS MS AM J OBSTET GYNECOL 118 897 974 045 MILUNSKY A N ENGL J MED 283 1370 970 046 HSU LYF AM J DIS CHILD 125 290 973 047 MILUNSKY A J PEDIATR 79 303 971 048 KOHN G LANCET 2 778 970 049 MILUNSKY A LANCET 2 979 970 050 KOHN G PEDIATR RES 1 461 967 051 KLINGER HP J BIOPHYS BIOCHEM CYTOL 8 345 960 052 BOOK JA J MED GENET 5 224 968 053 SCHLEGEL RJ CYTOGENETICS 5 430 968 054 KARDON NB J PEDIATR 80 297 972 055 RIIS P LANCET 2 180 960 056 RIIS P IN: MOORE KL 966 057 CEDERQVIST LL CLIN OBSTET GYNECOL 13 159 970 058 CASPERSSON T CHROMOSOMA 30 215 970 059 DANES BS SCIENCE 149 987 965 060 MILUNSKY A N ENGL J MED 281 1128 969 061 MATALON R LANCET 1 83 970 062 OMURA K TOHOKU J EXP MED 111 87 973 063 CRAWFURD MDA J MED GENET 10 144 973 064 DANES BS LANCET 1 946 970 065 MATALON R LANCET 1 798 972 066 HARPER PS J MED GENET 11 123 974 067 FRATANTONI JC PROC NAT ACAD SCI USA 60 699 968 068 FRATANTONI JC N ENGL J MED 280 686 969 069 BOOTH CW PEDIATRICS 53 396 974 070 FRATANTONI JC SCIENCE 162 570 968 071 MATALON R BIOCHEM BIOPHYS RES COMMUN 47 959 972 072 BACH G PROC NAT ACAD SCI USA 69 2048 972 073 BACH G FED PROC 32 483 973 074 MATALON R PEDIATR RES 7 384 973 075 OBRIEN JS PROC NAT ACAD SCI USA 69 1720 972 076 VON FIGURA K BIOCHEM BIOPHYS RES COMMUN 48 262 972 077 MATALON R BIOCHEM BIOPHYS RES COMMUN 61 759 974 078 MATALON R PEDIATR RES 9 314 975 079 SLY WS J PEDIATR 82 249 973 080 WIRTSCHAFTER ZT AM J OBSTET GYNECOL 76 1219 958 081 LEVY HL PEDIATR RES 3 113 969 082 COCKBURN F J OBSTET GYNAECOL BR COMMONW 80 10 973 083 SAIFER A CLIN CHEM 16 891 970 084 EMERY AEH LANCET 1 1307 970 085 SCOTT CR PEDIATR RES 6 659 972 086 TADA K TOHOKU J EXP MED 113 169 974 087 MORROW G 3RD J PEDIATR 77 120 970 088 GOMPERTZ D PEDIATRICS 54 511 974 089 MAHONEY MJ IN: DORFMAN A 95 972 090 MAHONEY MJ PEDIATR RES 7 342 973 091 FRIMPTER GW AM J DIS CHILD 113 115 967 092 SCHULMAN JD J PEDIATR 77 468 970 093 SCHNEIDER JA PEDIATR RES 7 291 973 094 SCHNEIDER JA N ENGL J MED 290 878 974 096 WILLCOX P ARCH DIS CHILD 49 209 974 097 GOODMAN SI J CLIN GENET 4 236 973 098 ROERDINK FH PEDIATR RES 7 863 973 101 DANCIS J IN: DORFMAN A 123 972 102 HSIA YR PROC AM PEDIATR SOC ANNU MTG 26 970 103 GOMPERTZ D LANCET 1 1009 973 104 WENDEL U HUMANGENETIK 19 127 973 105 ELSAS LJ METABOLISM 23 569 974 106 HARTLAGE PL J PEDIATR 85 86 974 107 FLEISHER LD J PEDIATR 85 677 974 108 BITTLES AH J MED GENET 10 120 973 109 HERS HG BIOCHEM J 86 11 963 110 NITOWSKY HM J LAB CLIN MED 69 472 967 111 ANGELINI C N ENGL J MED 287 948 972 112 KOSTER JF CLIN CHIM ACTA 40 294 972 113 COX RP LANCET 2 893 970 114 STEINITZ K ISR J MED SCI 3 411 967 115 SALAFSKY IS J PEDIATR 79 794 971 116 GALJAARD H CLIN CHIM ACTA 49 361 973 117 JUSTICE P BIOCHEM BIOPHYS RES COMMUN 39 301 970 118 HOWELL RR PROC AM PEDIATR SOC ANNU MTG 130 970 119 HUIJING F AM J HUM GENET 21 275 969 120 MIGEON BR AM J HUM GENET 26 360 974 121 KROOTH RS J EXP MED 113 1155 961 122 NADLER HL PEDIATRICS 42 912 968 123 FENSOM AH BR MED J 2 386 974 124 HILL HZ SCIENCE 179 1136 973 125 BENSON PF LANCET 1 106 973 126 DURAND P LANCET 1 1198 968 127 VAN HOOF F LANCET 1 1198 968 130 OCKERMAN PA LANCET 2 239 967 131 KJELLMAN B J PEDIATR 75 366 969 132 OBRIEN JS J PEDIATR 75 167 969 133 OBRIEN JS AM J DIS CHILD 109 338 965 134 OKADA S SCIENCE 160 1002 968 135 SINGER HS N ENGL J MED 282 571 970 136 SLOAN HR PEDIATR RES 3 532 969 137 THOMAS GH J LAB CLIN MED 74 725 969 138 LOWDEN JA N ENGL J MED 288 225 973 139 KABACK MM J PEDIATR 82 1037 973 140 OBRIEN JS N ENGL J MED 284 893 971 141 WOLFE LS NEUROLOGY (MINN) 20 23 970 142 SINGER HS AM J HUM GENET 24 454 972 143 BOOTH CW PEDIATRICS 52 521 973 144 OKADA S SCIENCE 165 698 969 145 OBRIEN JS SCIENCE 172 61 971 146 FRIEDLAND J PROC SOC EXP BIOL MED 136 1297 971 147 RICHARDSON BJ CLIN GENET 4 376 973 148 NAVON R AM J HUM GENET 25 287 973 149 SANDHOFF K LIFE SCI 7 283 968 150 OKADA S AM J HUM GENET 23 55 971 151 DESNICK RJ MIDWEST SOC PEDIAT RES PAPER 972 152 OKADA S CLIN RES 19 216 971 153 BRADY RO BIOCHEM BIOPHYS RES COMMUN 18 221 965 154 BEUTLER E AM J HUM GENET 23 62 971 156 BRADY RO PROC NAT ACAD SCI USA 55 366 966 157 FREDRICKSON DS IN: STANBURY JB 783 972 158 EPSTEIN CJ AM J HUM GENET 23 533 971 159 SUZUKI K PROC NAT ACAD SCI USA 66 302 970 160 SUZUKI K BIOCHEM BIOPHYS RES COMMUN 45 1363 971 161 ELLIS WG ARCH NEUROL 29 253 973 162 JATZKEWITZ H J NEUROCHEM 16 19 969 163 KABACK MM N ENGL J MED 282 1336 970 164 PORTER MT PROC NAT ACAD SCI USA 62 887 969 165 PERCY AK SCIENCE 161 594 968 166 AUSTIN J ARCH NEUROL 14 259 966 167 AUSTIN J ARCH NEUROL 13 593 965 168 MURPHY JV IN: BERNSOHN J 67 971 169 VAN DER HAGEN CB CLIN GENET 4 256 973 170 LEROY JG N ENGL J MED 288 1365 973 171 BORRESON AL CLIN GENET 4 442 973 172 BOOTH CW J PEDIATR 86 560 975 173 BRADY RO N ENGL J MED 276 1163 967 174 BRADY RO SCIENCE 172 174 971 175 KINT JA SCIENCE 167 1268 970 176 BEUTLER E AM J HUM GENET 24 237 972 177 WOOD S AM J HUM GENET 24 250 972 178 GALJAARD H CLIN GENET 5 368 974 179 HERNDON JH J CLIN INVEST 48 1017 969 180 HERNDON JH N ENGL J MED 281 1034 969 181 NADLER HL AM J OBSTET GYNECOL 103 710 969 182 DAWSON G SCIENCE 170 556 970 183 LEROY JG BIRTH DEF ORIG ART SER 5 174 969 184 GLASER JH PEDIATR RES 7 342 973 185 WARREN RJ PEDIATR RES 7 343 973 186 PHILIPPART M PEDIATR RES 9 316 975 187 KOHN G PEDIATR RES 9 314 975 188 NADLER HL N ENGL J MED 282 302 970 189 AULA P HUMANGENETIK 25 307 974 190 SEEGMILLER JE SCIENCE 155 1682 967 191 ROSENBLOOM FM LANCET 2 305 967 192 FUJIMOTO WY LANCET 2 511 968 193 DEMARS R SCIENCE 164 1303 969 194 BOYLE JA SCIENCE 169 688 970 195 JEFFCOATE TNA LANCET 2 553 965 196 NICHOLS J LANCET 1 83 970 197 FUCHS F AM J OBSTET GYNECOL 99 806 967 198 CATHRO DM LANCET 1 732 969 199 NICHOLS J LANCET 2 1068 967 200 FRASIER SL J PEDIATR 84 738 974 201 GOLDSTEIN JL AM J HUM GENET 26 199 974 202 KHACHADURIAN AK LEB MED J 26 325 973 203 BEAUDET AL J LAB CLIN MED 84 54 974 204 DANES BS LANCET 1 1061 968 205 NADLER HL LANCET 2 84 969 206 BOWMAN BH SCIENCE 164 325 969 207 SPOCK A PEDIATR RES 1 173 967 208 DANES BS J EXP MED 136 1313 972 209 BARNETT DR FED PROC 32 677 973 210 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 211 CONOVER JH CLIN RES 21 531 973 212 BOWMAN BH CLIN GENET 4 461 973 213 BERATIS NG PEDIATR RES 7 958 973 214 GIBLETT ER LANCET 2 1067 972 215 DISSING J LANCET 2 1316 972 216 HIRSCHHORN R LANCET 2 1217 973 217 HIRSCHHORN R LANCET 1 73 975 218 ROMEO G BIOCHEM GENET 4 659 970 219 REGAN JD SCIENCE 174 147 971 220 RAMSAY CA LANCET 2 1109 974 221 LORBER J PEDIATRICS 53 307 974 222 STEIN SC PEDIATRICS 54 553 974 223 CARTER CO J MED GENET 10 209 973 224 CASSADY G AM J OBSTET GYNECOL 97 395 967 225 LEE TY AM J OBSTET GYNECOL 107 917 970 226 EMERY AEH J OBSTET GYNAECOL BR COMMONW 79 154 972 227 EMERY AEH PROC INT CONG PEDIATR 14TH 974 228 EMERY AEH LANCET 1 970 973 229 BERGSTRAND CG SCAND J CLIN LAB INVEST 8 174 956 230 GITLIN D J CLIN INVEST 45 1826 966 231 SEPPALA M AM J OBSTET GYNECOL 114 595 972 232 ADINOLFI A BIOCHIM BIOPHYS ACTA 251 197 971 233 ABELEV GI ADV CANCER RES 14 295 971 234 MILUNSKY A J PEDIATR 84 889 974 235 SELLER MJ DEVELOP MED CHILD NEUROL 16 369 974 236 BROCK DJH LANCET 2 197 972 237 SEPPALA M CLIN PERINATOL 1 293 974 238 SEPPALA M ANN MED EXP BIOL FENNIAE 45 16 967 239 SEPPALA M LANCET 1 155 973 240 SEPPALA M OBSTET GYNECOL 42 701 973 241 GUIBAUD S LANCET 1 1261 973 242 WHYLEY GA J OBSTET GYNAECOL BR COMMONW 81 459 974 243 SEPPALA M OBSTET GYNECOL 42 613 973 244 SEPPALA M J OBSTET GYNAECOL BR COMMONW 81 827 974 245 SEPPALA M AM J OBSTET GYNECOL 119 2701 974 246 DEBRUIJN AWA LANCET 1 525 975 247 MILUNSKY A PEDIATR RES 9 360 975 248 KJESSLER B LANCET 1 432 975 249 BROCK DJH CLIN CHIM ACTA 57 315 974 250 BROCK DJH LANCET 2 923 973 251 ISHIGURO T LANCET 2 1214 973 252 ISHIGURO T AM J OBSTET GYNECOL 121 539 975 253 WALDMANN TA LANCET 2 1112 972 254 SIMONS MJ LANCET 1 1234 974 255 NISHI S CANCER RES 30 2507 970 256 KANG KY CLIN CHIM ACTA 42 175 972 257 ZELTZER PM LANCET 2 7854 974 258 BROCK DJH LANCET 1 745 975 259 MACRI JN NATURE NEW BIOL 246 89 973 260 CLAUSEN J PROC SOC EXP BIOL MED 107 170 961 261 HEITMANN R DTSCH Z NERVENHEILK 188 187 967 262 MACRI JN LANCET 1 14 974 263 MACRI JN LANCET 1 1109 974 264 OLSSON JE LANCET 2 347 974 265 OLSSON JE J NEUROCHEM 21 1153 973 266 HOCHWALD GM PROC SOC EXP BIOL MED 109 91 962 267 LATERRE EC CLIN CHIM ACTA 10 197 964 268 PENNY R AUST J EXP BIOL MED SCI 49 111 971 269 OLSSON JE J NEUROCHEM 21 625 973 270 SUTHERLAND GR LANCET 2 1098 973 271 NELSON MM LANCET 1 504 974 272 RUSSELL JGB J OBSTET GYNAECOL BR COMMONW 76 345 969 273 HARTLEY JB ST MARYS HOSPITAL MANCHESTER 966 274 PAPP Z LANCET 1 729 973 275 CAMPBELL S LANCET 2 1226 972 276 THIERY M LANCET 1 599 973 277 MICHELL RC J OBSTET GYNAECOL BR COMMONW 80 1100 973 278 FRIGOLETTO FD OBSTET GYNECOL 44 286 974 279 NOONAN CD AM J OBSTET GYNECOL 101 929 968 280 OMENN GS AM J HUM GENET 24 31A 972 281 QUEENAN JT OBSTET GYNECOL 35 648 970 282 AGUERO O SURG GYNECOL OBSTET 130 649 970 283 AGUERO O AM J OBSTET GYNECOL 107 971 970 284 ERBSLOH J ARCH GYNAEKOL 173 160 942 285 WIESENHAAN PF AM J OBSTET GYNECOL 113 819 972 286 OGITA S OBSTET GYNECOL 43 887 974 287 BAUM G FUND MEDICAL ULTRASONOGRAPHY 974 288 GARRETT WJ AUST NZ J OBSTET GYNAECOL 10 7 970 289 HOULTON MCC J OBSTET GYNAECOL BR COMMONW 81 818 974 290 WESTIN B LANCET 2 872 954 291 WESTIN B ACTA PAEDIATR SCAND 46 117 957 292 MANDELBAUM B AM J OBSTET GYNECOL 98 1140 967 293 EMERY AEH IN: HARRIS M 31 970 294 SCRIMGEOUR JB IN: EMERY AEH 40 973 295 VALENTI C AM J OBSTET GYNECOL 114 561 972 296 HOBBINS JC AM J OBSTET GYNECOL 118 1069 974 297 BENZIE RJ AM J OBSTET GYNECOL 121 460 975 298 MOTULSKY AG N ENGL J MED 288 31 973 299 OSKI FA HEMATOLOGIC PROBLEMS IN THE N 972 300 BRINKMAN R J PHYSIOL (LOND) 85 117 933 301 WALKER J ARCH DIS CHILD 30 111 955 302 DRESCHER H WIEN KLIN WOCHENSCHR 32 92 954 303 HOLLENBERG MD SCIENCE 174 698 971 304 BASCH RS BLOOD 39 530 972 305 PATARYAS HA BLOOD 39 688 972 306 KAN YW N ENGL J MED 287 1 972 307 KAZAZIAN HH JR N ENGL J MED 289 58 973 308 CIVIDALLI G PEDIATR RES 8 553 974 309 VALENTI C AM J OBSTET GYNECOL 115 851 973 310 KAN YW LANCET 1 79 974 311 KAN YW BLOOD 43 411 974 312 HOBBINS JC N ENGL J MED 290 1065 974 313 CHANG H N ENGL J MED 290 1067 974 314 ALTER BP PEDIATR RES 9 320 975 315 BROCK DJH LANCET 2 1077 974 316 HARRIS M FOGARTY INT CENT PROC 6 970 317 LEJEUNE J AM J HUM GENET 22 121 970 318 CROW JF ADV HUM GENET 8 5 972 319 RAMSEY P THOMIST 37 174 973 320 KASS L IN: HILTON B 973 CT 1 GUSTAVSON KH NEUROPADIATRIE 8 293 977 2 GOLDMAN B BR J OBSTET GYNAECOL 84 808 977 3 COOPERMAN EM CAN MED ASSOC J 116 1339 977 4 VALLE D JOHNS HOPKINS MED J 141 35 977 5 DANIEL A MED J AUST 2 352 978 6 MILES JH PEDIATR CLIN NORTH AM 25 593 978 7 SELL RR SOC BIOL 25 288 978 8 MAHONEY MJ CLIN PERINATOL 6 255 979 9 SIMPSON JL CLIN OBSTET GYNAECOL 6 259 979 10 NORBY S DAN MED BULL 26 353 979 11 TEICHLERZALLEN D CLIN GENET 18 257 980 12 STEPHENSON SR AM J OBSTET GYNECOL 141 319 981 PN 76006 RN 00361 AN 77019322 AU Hawkins-E. Singer-D-B. TI The adrenal cortex in cystic fibrosis of the pancreas. SO Am-J-Clin-Pathol. 1976 Oct. 66(4). P 710-4. MJ ADRENAL-CORTEX: pa. ADRENAL-GLANDS: pa. CYSTIC-FIBROSIS: pa. MN ADRENAL-CORTEX: me. ALDOSTERONE: bi. CHILD-PRESCHOOL. CHLORIDES: me. CYSTIC-FIBROSIS: me. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. SODIUM: me. AB The morphologies of the adrenal cortical zones of 25 children with cystic fibrosis and 24 control patients are presented. A technic for objective measurement of these zones is described. Hyperplasia of the zona glomerulosa in the children with cystic fibrosis is significant and may be related to chronic loss of salt in the sweat or demands placed on renal salt-conserving mechanisms. RF 001 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 002 BECH K ACTA PATH MICROBIOL SCAND (A) 80 615 972 003 CHODOS DD AM J DIS CHILD 110 76 965 004$ COOKE CR J LAB CLIN MED 82 784 971 005 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 006 DI SANTAGNESE PA JAMA 172 2014 960 007 DI SANTAGNESE PA PEDIATRICS 12 549 953 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 009 FINBERG L J PEDIATR 79 499 971 010 GOTTLIEB RP J PEDIATR 79 930 971 011 KESSLER WR PEDIATRICS 8 648 951 012 MCCOMBS ML TEX REP BIOL MED 31 615 973 013 MONTALVO JM J CLIN ENDOCRINOL METAB 28 582 968 014 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 015 ROBSON AM J PEDIATR 79 42 971 016 SIEGENTHALER P ARCH DIS CHILD 39 61 964 017 STOLL E HELV PAEDIATR ACTA 25 234 970 018 SYMINGTON T PATHOLOGY HUMAN ADRENAL GLAND 66 969 019 TALAMO RC MED TIMES 97 139 969 CT 1 RAPAPORT R J PEDIATR 98 768 981 2 FORSYTH JS SCOTT MED J 27 333 982 3 FERGUSSON RJ ACTA PAEDIATR SCAND 75 124 986 PN 76007 RN 00362 AN 77063079 AU Abernathy-R-S. TI Bulging fontanelle as presenting sign in cystic fibrosis. Vitamin A metabolism and effect on cerebrospinal fluid pressure. SO Am-J-Dis-Child. 1976 Dec. 130(12). P 1360-2. MJ CYSTIC-FIBROSIS: co. INTRACRANIAL-PRESSURE. SKULL. VITAMIN-A-DEFICIENCY: co. MN CASE-REPORT. CHILD-PRESCHOOL. DIAGNOSIS-DIFFERENTIAL. HUMAN. MALABSORPTION-SYNDROMES: co. MALE. VITAMIN-A-DEFICIENCY: di. VITAMIN-A: me. AB A 51/2-month-old infant had the single problem of a bulging fontanelle. A diagnosis of cystic fibrosis with secondary hypovitaminosis A was made by the findings of high sweat chloride values and a low serum carotene level. A greatly accelerated rate of weight gain following the addition of pancreatic enzyme supplements confirmed the presence of malabsorption. The infant developed characteristic fibrosis pulmonary disease at 20 months of age. Animal studies have shown vitamin A deficiency to be associated with increased cerebrospinal fluid (CSF) pressure, diminished absorption of CSF, and pathological findings of thickening and infiltration with mucopolysaccharides of the dura mater around the arachnoid villi. RF 001 KEATING JP PEDIATRICS 46 41 970 002 CORNFELD D PEDIATRICS 10 33 952 003 BASS MH J PEDIATR 47 690 955 004 BUCHHEIT WA N ENGL J MED 280 938 969 005 MOORE LA AM J PHYSIOL 130 684 940 006 MILLEN JW LANCET 2 679 954 007 EATON HD AM J CLIN NUTR 22 1070 969 008 CALHOUN MC J DAIRY SCI 50 1486 967 009 HAYES KC ARCH OPHTHALMOL 80 777 968 010 COUSINS RJ J NUTR 97 409 969 011 DANES BS J EXP MED 124 1181 966 012 HURT HD J DAIRY SCI 50 1941 967 013 MADDUX GW J NUTR 104 478 974 014 GORGACZ EJ J NUTR 101 1541 971 015 ROELS OA JAMA 214 1097 970 CT 1 EATON ML AM J HOSP PHARM 35 1099 978 2 CHASE HP J PEDIATR 95 337 979 3 ROACH ES PEDIATRICS 66 622 980 4 MAGAZZU G RIV ITAL PEDIATR 7 73 981 5 PARK RW GASTROENTEROLOGY 81 1143 981 6 KASARSKIS EJ NEUROLOGY 32 1292 982 7 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 PN 76008 RN 00363 AN 76228819 AU Jones-R-E. Giammona-S-T. TI Intrapleural injection of quinacrine for pneumothorax in a child with cystic fibrosis. SO Am-J-Dis-Child. 1976 Jul. 130(7). P 777-8. MJ CYSTIC-FIBROSIS: co.PNEUMOTHORAX: dt. QUINACRINE: ad. MN CARBENICILLIN: tu. CASE-REPORT. CHILD. GENTAMICINS: tu. HUMAN. INJECTIONS: mt. MALE. MEDIASTINAL-EMPHYSEMA: co, dt. PLEURA. PNEUMOTHORAX: su. PSEUDOMONAS-INFECTIONS: dt. QUINACRINE: tu. THORAX: su. THORACIC-SURGERY. EX Pneumothorax occurs in 2% to 4% of patients with cystic fibrosis following rupture of subpleural blebs into the pleural space. Death can occur, and recurrences have been reported. Therapeutic procedures include needle aspiration, closed thoracotomy drainage, open thoracotomy with pleural scarification, or intrapleural instillation of irritants. When injected into the pleural space, the antimalarial drug quinacrine hydrochloride evokes an inflammatory response; the resultant fibrosis produces a tight adherence of the visceral and pleural surfaces. Quinacrine therapy for pneumothorax has been frequently used in adults, but there are few pediatric case reports. RF 001 LIFSCHITZ MI AM J DIS CHILD 116 633 968 002 BOAT TF JAMA 209 1498 969 003 CATTANEO SM J THORAC CARDIOVASC SURG 66 467 973 004 KATTWINKEL J JAMA 226 557 973 005 BORDA I JAMA 201 1049 967 006 BORJA ER CANCER 31 899 973 CT 1 RICH RH J PEDIATR SURG 13 237 978 2 FURMAN WR AM REV RESPIR DIS 121 137 980 3 KISTLER I HELV PAEDIATR ACTA 36 495 982 4 MCLAUGHLIN FJ J PEDIATR 100 863 982 5 SCHUSTER SR J PEDIATR SURG 18 492 983 PN 76009 RN 00364 AN 76203208 AU Isenberg-J-N. LHeureux-P-R. Warwick-W-J. Sharp-H-L. TI Clinical observations on the biliary system in cystic fibrosis. SO Am-J-Gastroenterol. 1976 Feb. 65(2). P 134-41. MJ BILIARY-TRACT-DISEASES: ra. CYSTIC-FIBROSIS: ra. MN ADOLESCENCE. ADULT. BILIARY-TRACT-DISEASES: di. BIOPSY. CHILD. CHILD-PRESCHOOL. CHOLECYSTOGRAPHY. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. INFANT. LIVER-CIRRHOSIS-BILIARY: ra. LIVER. MALE. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB The biliary tree of 66 patients with cystic fibrosis was examined by conventional roentgenographic methods. Forty-five per cent of the oral cholecystograms were judged abnormal by our criteria. A study was considered abnormal if there was no visualization or if there was visualization of a microgallbladder or structural abnormality including marginal irregularities, septate gallbladder or cholelithiasis. Intravenous cholangiography was used to further study the 22 patients who did not visualize on the oral study. Again, anatomic abnormalities were prevalent but six patients in this group had normal appearing gallbladders. Abdominal pain, a frequent symptom in cystic fibrosis, was not associated with roentgenographic abnormality. No correlation was seen between the external biliary tree abnormalities and multifocal biliary cirrhosis which was present in 40% of these patients. Further, no correlation was seen between serum gamma glutamyl transpeptidase levels and either of these lesions. RF 001 POGUE RE MINN MED 52 279 969 002 WARWICK WJ CF DATA REGISTRY COMM REPORT 974 003 GRAND RJ CLIN PEDIATR 9 588 970 004 KOPEL FB GASTROENTEROLOGY 62 483 972 005 FEIGELSON J NOUV PRESSE MED 1 1899 972 006 WALKER WA PEDIATRICS 40 946 967 007 SHARP HL CF CLUB ABST 4 968 008 DANKS DM ANN HUM GENET 28 323 965 009 BAKER HL JR RADIOLOGY 74 239 960 010 HODGSON JR RADIOL CLIN NORTH AM 8 85 970 011 WISE RE RADIOL CLIN NORTH AM 4 521 966 012 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 013 JONES MD J PEDIATR 53 172 958 014 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 015 SLOTH K ACTA PAEDIATR SCAND 61 380 972 016 LAPEY A J PEDIATR 84 328 974 017 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 018 SHARP HL GAP CONF REP PATHOL OF CF 972 019 OPPENHEIMER EH J PEDIATR 86 683 975 CT 1 LHEUREUX PR AM J ROENTGENOL 128 953 977 2 SANTAGNESE PAD AM J MED 66 121 979 3 DAVIDSON GP J CLIN PATHOL 33 390 980 4 PSACHAROPOULOS HT LANCET 2 78 981 5 JACOBS WH AM J GASTROENTEROL 76 342 981 6 LAMBERT JR GASTROENTEROLOGY 80 169 981 7 PARK RW GASTROENTEROLOGY 81 1143 981 8 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 9 MOWAT AP CLIN GASTROENTEROL 11 171 982 10 TSCHOLAKOFF D FORTSCHR GEB RONTG NUKL 137 18 982 11 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 12 DOHERTY DE SOUTH MED J 76 1580 983 13 BASS S GASTROENTEROLOGY 84 1592 983 14 HODSON ME POSTGRAD MED J 60 225 984 15 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 16 WEIZMAN Z GUT 27 1043 986 17 MCHUGO JM BR J RADIOL 60 137 987 PN 76010 RN 00365 AN 77085034 AU Lederberg-S. Sackett-D. TI Distribution of complement C3 variants in individuals with cystic fibrosis. SO Am-J-Hum-Genet. 1976 Nov. 28(6). P 597-601. MJ COMPLEMENT-3. COMPLEMENT. CYSTIC-FIBROSIS: fg. VARIATION-GENETICS. MN ELECTROPHORESIS-AGAR-GEL. FEMALE. GENE-FREQUENCY. HUMAN. MALE. PHENOTYPE. AB The gene frequency for slow and fast electrophoretic variants of complement C3 in Caucasian individuals with cystic fibrosis was similar to the values expected for unaffected controls, thereby ruling out a suspected differential involvement of these phenotypes with the disease. In one family, cystic fibrosis and complement C3 phenotypes segregated independently. RF 001 SPOCK A PEDIATR RES 1 173 967 002 BOWMAN BH SCIENCE 164 325 969 003 MANGOS JA SCIENCE 158 135 967 004 KAISER D LANCET 1 1003 970 005 LEDERBERG S IN: MANGOS JA 259 976 006 MULLER-EBERHARD HJ ANNU REV BIOCHEM 44 697 975 007 DI SANTAGNESE PA N ENGL J MED 277 1287 967 008 CONOVER JH LANCET 2 1501 973 009 CONOVER JH LIFE SCI 14 253 974 010 CONOVER JH LANCET 1 47 975 011 RAO GJS J PEDIATR 80 573 972 012 RAO GJS PEDIATR RES 8 684 974 013 WILSON GB PEDIATR RES 10 87 976 014 POLLEY MJ J MED GENET 11 249 974 015 LIEBERMAN J LANCET 1 1230 974 016 LIEBERMAN J AM REV RESPIR DIS 109 399 974 017 WRIGHT SW AM J HUM GENET 20 157 968 018 KULCZYCKI LL AM J DIS CHILD 127 64 974 019 ALPER CA J CLIN INVEST 47 2181 968 020 AZEN EA SCIENCE 162 905 968 021 ALPER CA IN: PEETERS H 17 295 969 022 AZEN EA BIOCHEM GENET 3 215 969 023 VISAKORPI JK ISR J MED SCI 9 1156 973 024 ARVILOMMI H HUMANGENETIK 18 253 973 025 FARHUD DD HUMANGENETIK 17 57 972 026 DAVIS BJ ANN NY ACAD SCI 121 404 964 027 HOLZHAUER RJ AM J HUM GENET 28 602 976 CT 1 HOLZHAUER RJ AM J HUM GENET 28 602 976 2 SCHIOTZ PO HUM HERED 28 293 978 3 GOTZ M EUR J PEDIATR 127 133 978 4 JAKEL HP BIOL ZENTRALBL 98 55 979 5 SCACCHI R HUM HERED 31 183 981 6 BERNAL JE HUM HERED 36 97 986 PN 76011 RN 00366 AN 77085035 AU Holzhauer-R-J. Van-Ess-J-D. Schwartz-R-H. TI Third component of complement in cystic fibrosis. SO Am-J-Hum-Genet. 1976 Nov. 28(6). P 602-6. MJ COMPLEMENT-3. COMPLEMENT. CYSTIC-FIBROSIS: fg. POLYMORPHISM-GENETICS. MN ELECTROPHORESIS-AGAR-GEL. FEMALE. HETEROZYGOTE. HUMAN. MALE. PEDIGREE. PHENOTYPE. AB In a study of C3 levels and phenotypes in 64 cystic fibrosis (CF) patients, 92 CF parents, 64 normal siblings, and 126 healthy controls, significant elevations of mean C3 levels were found in CF patients, their parents, and in one genetic sub-group of their siblins (SS females). C3 concentration in CF patients correlated with the degree of clinical impairment as measured by Shwachman-Kulczycki (S-K) score. No significant differences were found in the prevalences of C3 phenotypes or the S and F gene frequencies among the groups studied. RF 001 POLLEY MJ J MED GENET 11 249 974 002 CONOVER JH LANCET 2 1501 973 003 CONOVER JH LIFE SCI 14 253 974 004 LIEBERMAN J LANCET 1 1230 974 005 HANN S LANCET 2 520 974 006 SCANLIN TF JR LANCET 1 1382 974 007 TAUSSIG LM CF CLUB ABST 17 21 976 008 SERGENT JS CLIN RES 24 389A 976 009 ALPER CA J CLIN INVEST 47 2181 968 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 TEISBERG P VOX SANG 19 47 970 012 LEDERBERG S AM J HUM GENET 28 597 976 CT 1 LEDERBERG S AM J HUM GENET 28 597 976 2 SCHIOTZ PO HUM HERED 28 293 978 3 MOSS RB AM REV RESPIR DIS 121 23 980 4 SCACCHI R HUM HERED 31 183 981 5 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 6 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 7 BERNAL JE HUM HERED 36 97 986 PN 76012 RN 00367 AN 76250273 AU Townes-P-L. Moore-W-D. White-M-R. TI Amylase polymorphism: studies of sera and duodenal aspirates in normal individuals and in cystic fibrosis. SO Am-J-Hum-Genet. 1976 Jul. 28(4). P 378-89. MJ AMYLASES: me. CYSTIC-FIBROSIS: en. DUODENUM: se. POLYMORPHISM-GENETICS. MN AMYLASES: bl. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. HUMAN. PANCREAS: en. SALIVA: en. SUPPORT-U-S-GOVT-P-H-S. AB Prior genetic studies of the human pancreatic amylase (Amy2) locus have been directed principally to the electrophoretic analysis of serum and urine, on the assumption that these fluids receive negligible contributions from the salivary (Amy 1) locus. In support of that assumption was the observation that the isozyme bands were lacking in patients with cystic fibrosis and in a postpancreatectomy patient. We have examined the sera of 97 patients having cystic fibrosis and find normal levels of serum amylase. On electrophoresis, three-quarters of the cystic fibrosis patients have a pattern (F- pattern) not observed in normal sera. The pattern is characterized by the absence of Pa 1. Comparative electrophoresis and mixing experiments indicate that the F-pattern is of salivary origin and is unmasked in cystic fibrosis by the absence of a pancreatic contribution. The normal serum pattern is considered to be an admixture of salivary and pancreatic amylase. On the assumption that duodenal fluids might more closely reflect the pancreatic (Amy 2) locus, electrophoretic studies were performed on 148 normal individuals and 37 individuals with cystic fibrosis. Electrophoretic phenotypes in duodenal aspirates are more complex than previously reported in studies of urine and serum; presumably because of the higher concentrations of amylase in the aspirates. Comparative electrophoresis and mixing experiments indicate that the phenotypes observed in duodenal aspirates also reflect admixture of pancreatic and salivary amylase. This recognition of pancreatic and salivary admixture in sera fortunately does not alter our prior understanding of the genetics of the Amy 2 polymorphism. The extensive studies which led to the delineation of the Amy 2 polymorphism were essentially based on the presence or absence of a variant band which proves now to be outside the zone of admixture. RF 001 KAMARYT J HUMANGENETIK 1 579 965 002 KAMARYT J HUMANGENETIK 3 41 966 003 BOETTCHER B ANAL BIOCHEM 28 510 969 004 DE LA LANDE FA ENZYMOLOGIA 37 335 969 005 BOETTCHER B ENZYMOLOGIA 40 234 970 006 WARD JC AM J HUM GENET 23 403 971 007 MERRITT AD NATURE NEW BIOL 239 243 972 008 MERRITT AD AM J HUM GENET 25 510 973 009 MERRITT AD AM J HUM GENET 25 510 973 010 FRIDHANDLER L CLIN CHEM 18 1493 972 011 KLEIN B CLIN CHEM 16 32 970 012 TOWNES PL J PEDIATR 75 221 969 013 WEBB DR AM REV RESPIR DIS 108 918 973 014 BERK JE AM J DIG DIS 11 695 966 015 HOBBS JR IN: BERGMEYER HU 909 974 016 TAKEUCHI T CLIN CHIM ACTA 54 137 974 017 OJALA K SCAND J CLIN LAB INVEST 35 163 975 018 KAMARYT J Z KLIN CHEM KLIN BIOCHEM 7 51 969 019 LEVITT MD ANN INTERN MED 71 919 969 020 DUANE WC J CLIN INVEST 50 156 971 021 ADLERCREUTZ H CLIN CHIM ACTA 43 187 973 022 DI SANTAGNESE PA PEDIATRICS 22 507 958 023 BARBERO GJ PEDIATRICS 22 945 958 024 KARN RC ISOZYMES 4 745 975 025 TOWNES PL BIRTH DEF ORIG ART SER 8 95 972 026 LILLIBRIDGE CB J PEDIATR 82 279 973 CT 1 MERRITT AD ADV HUM GENET 8 135 977 2 TAUSSIG LM AM J HUM GENET 29 408 977 3 TOWNES PL AM J HUM GENET 29 408 977 4 WATSON RR AM J CLIN NUTR 30 599 977 5 GILLARD BK PEDIATR RES 12 868 978 6 BERK JE AM J GASTROENTEROL 69 417 978 7 CASOLA L CLIN CHIM ACTA 94 83 979 8 TOWNES PL AM J DIS CHILD 135 248 981 9 CLINK D AM SURGEON 48 422 982 10 BARNETT JL SOUTH MED J 79 832 986 PN 76013 RN 00368 AN 77020143 AU Neuburger-N. Levison-H. Kruger-K. TI Transit time analysis of the forced expiratory vital capacity in cystic fibrosis. SO Am-Rev-Respir-Dis. 1976 Oct. 114(4). P 753-9. MJ CYSTIC-FIBROSIS: pp. VITAL-CAPACITY. MN ADOLESCENCE. ADULT. ANALYSIS-OF-VARIANCE. CHILD. FEMALE. FORCED-EXPIRATORY-VOLUME. HUMAN. MALE. PEAK-EXPIRATORY-FLOW-RATE. AB Transit time analysis of the forced expiratory vital capacity maneuver was applied to 37 patients with cystic fibrosis 8 to 22 years of age. This analysis divides the vital capacity into segments of air and assigns a transit time to each segment. The characteristics of the distribution of these transit times are used as measurements of pulmonary function. The quantities were compared with the forced vital capacity, 1-sec forced expiratory volume, ratio of 1-sec forced expiratory volume to vital capacity, peak flow, forced expiratory flow during the middle half of the forced vital capacity, maximal expiratory flow at 25 per cent of the vital capacity, specific airway conductance, and arterial PO2 of these patients. The standard deviation of the transit times was the most frequently abnormal. The mean transit time had the largest range of values in terms of its own standard deviation, and it was the best single estimate of over-all lung function because it correlated almost equally with large and small airway function. The coefficient of cariance of transit times was specific in detecting abnormality of small airways and was as sensitive in the detection of minimal lung disease as the standard deviation of transit times. RF 001 FISH J AM REV RESPIR DIS 109 700 974 002 NEUBURGER N J APPL PHYSIOL 40 329 976 003 MANSELL A J APPL PHYSIOL 33 711 972 004 MEAD J J APPL PHYSIOL 22 95 967 005 MACKLEM PT J APPL PHYSIOL 22 395 967 CT 1 ZAPLETAL A BULL EUR PHYSIOPATH RESP 15 575 979 2 JORDANOGLOU J THORAX 34 187 979 3 LIANG A THORAX 34 194 979 4 BELLO R THORAX 35 225 980 5 JORDANOGLOU J THORAX 35 375 980 6 JANSEN JM AM REV RESPIR DIS 122 221 980 7 NICKERSON BG AM REV RESPIR DIS 122 859 980 8 JORDANOGLOU J THORAX 37 304 982 9 MILLER MR THORAX 37 205 982 10 EISER NM BULL EUR PHYSIOPATH RESP 19 495 983 11 QUANJER PH BULL EUR PHYSIOPATH RESP 19 1 983 12 ELDHAHER AHG MED BIOL ENG COMPUT 21 277 983 13 BUIST AS AM REV RESPIR DIS 127 714 983 14 OSMANLIEV DP BULL EUR PHYSIOPATH RESP 20 285 984 15 INCALZI RA RESPIRATION 48 145 985 16 PEDERSEN OF J APPL PHYSIOL 58 1305 985 17 MILLER MR AM REV RESPIR DIS 132 1041 985 18 CHINN DJ BULL EUR PHYSIOPATH RESP 22 461 986 PN 76014 RN 00369 AN 77020098 AU Galant-S-P. Rucker-R-W. Groncy-C-E. Wells-I-D. Novey-H-S. TI Incidence of serum antibodies to several Aspergillus species and to Candida albicans in cystic fibrosis. SO Am-Rev-Respir-Dis. 1976 Aug. 114(2). P 325-31. MJ ANTIBODIES-FUNGAL: an. ASPERGILLUS: im. CANDIDA-ALBICANS: im. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ASPERGILLUS-NIGER: im. ASPERGILLUS-FUMIGATUS: im. ASTHMA: im. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. HUMAN. IGE: an. INFANT. MALE. PRECIPITIN-TESTS. AB The incidence of serum IgE antibodies to several species of aspergillus and the incidence of serum precipitins to these species and to Candida albicans were determined in 22 children with cystic fibrosis, 17 children with bronchial asthma, and 18 control subjects. IgE antibodies were found in 50 per cent of the patients with cystic fibrosis and in 29 per cent of the patients with bronchial asthma; none was found in the control subjects. Precipitins to aspergillus species were present in 59 per cent of the patients with cystic fibrosis, 35 per cent of the patients with bronchial asthma, and 11 per cent of the control subjects. Greater differences were noted when the total number of precipitin bands in each group was compared. There was a trend for the more severe cases of cystic fibrosis (based on the Shwachman-Kulczycki score) to have a greater incidence of precipitins and a lower frequency of IgE antibodies to aspergillus than the milder cases. Candida precipitins were found more frequently in patients with cystic fibrosis (45 per cent) than in patients with bronchial asthma (18 per cent); none was found in the control group. The potential role of fungal hypersensitivity in cystic fibrosis and the therapeutic implications are discussed in light of these findings. RF 001 DI SANTAGNESE PA IN: VAUGHAN VC III 905 971 002 SHWACHMAN H IN: KENDIG EL JR 1 524 972 003 WARREN CPW CLIN ALLERGY 5 1 975 004 MEARNS MB LANCET 1 538 967 005 SCHWARTZ RH AM J DIS CHILD 120 432 970 006 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 007 MEARNS MB THORAX 20 385 965 008 BATTEN JC MOD PROBL PEDIATR 10 227 967 009 ORES CN CF CLUB ABST 16 48 975 010 LEWISTON NJ CF CLUB ABST 47 973 011 PEPYS J HYPERSENSITIVITY DIS LUNGS DU 969 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 OUCHTERLONY O ACTA PATH MICROBIOL SCAND 32 231 953 014 PEPYS J AM REV RESPIR DIS 89 842 964 015 WIDE L LANCET 2 1105 967 016 HOFFMAN DR J ALLERGY CLIN IMMUNOL 54 165 974 017 FINEGOLD SM AM J MED 27 463 959 018 YOUNG RC MEDICINE (BALTIMORE) 49 147 970 019 ARBESMAN CE IN: GOODFRIEND L 163 973 020 PEPYS J HYPERSENSITIVITY DIS LUNGS DU 969 021 KULCZYCKI LL JAMA 175 358 961 022 RACHELEFSKY GS AM J DIS CHILD 128 355 974 023 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 024 CRACCO G PROC EWGCF 5TH ANNU MTG 16 974 025 MCFARLANE H BR MED J 1 423 975 026 BARDANA EJ JR AM J DIS CHILD 129 1164 975 027 KATZENSTEIN AL AM REV RESPIR DIS 111 497 975 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 COLLINS RF AM J MED TECHNOLOGY 44 826 978 3 NOVEY HS J DIAL 3 349 979 4 MARSH PB MYCOPATHOLOGIA 69 67 979 5 NELSON LA AM REV RESPIR DIS 120 863 979 6 MOSS RB AM REV RESPIR DIS 121 23 980 7 MARKS MI J PEDIATR 98 173 981 8 MOSS RB J PEDIATR 99 215 981 9 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 10 WONNE R CLIN ALLERGY 15 455 985 11 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 12 MALFROOT A PEDIATR INFECT DIS 5 376 986 PN 76015 RN 00370 AN 76229764 AU Wood-R-E. Boat-T-F. Doershuk-C-F. TI Cystic fibrosis. SO Am-Rev-Respir-Dis. 1976 Jun. 113(6). P 833-78. (REVIEW). MJ CYSTIC-FIBROSIS. MN BIOLOGICAL-TRANSPORT. CYSTIC-FIBROSIS: co, pp, th. EXOCRINE-GLANDS: pp. FEMALE. GASTROINTESTINAL-DISEASES: et. GENITAL-DISEASES-MALE: et. GENITAL-DISEASES-FEMALE: et. HUMAN. LUNG-DISEASES: et, pa. MALE. MUCUS: me. PANCREAS: pp. PROGNOSIS. PSYCHOLOGY. REVIEW. SOCIAL-ADJUSTMENT. SWEAT. SUPPORT-U-S-GOVT-P-H-S. EX Cystic fibrosis is the most frequent lethal genetic syndrome among white children and is the cause of much of the chronic progressive pulmonary disease encountered in children. Because of the increasing adolescent and adult population of patients with cystic fibrosis and their psychosocial, employment, and treatment needs, this review, whenever possible, will emphasize aspects pertinent to older patients. No known biochemical or structural defect will account for all the pathophysiologic phenomena of cystic fibrosis. Thus, the definition of the disease rests on the clinical findings; cystic fibrosis is a syndrome, and not yet a disease. Incidence figures for cystic fibrosis are necessarily minimal. The best diagnostic efforts currently available fail to detect all those who die at a young age or who have mild symptoms. Several pieces of evidence indicate that cystic fibrosis is transmitted as an autosomal recessive trait. An autosomal recessive inheritance for cystic fibrosis suggests a discrete biochemical or structural defect. To date no single lesion has been proved to provide a unifying hypothesis for the pathophysiology of cystic fibrosis. Exocrine gland dysfunction, mucociliary transport, immunology, ciliary factors and transport mechanisms are discussed. The manifestations of cystic fibrosis involve many organ systems and the pattern of presentation is variable. Respiratory, gastrointestinal, genitourinary, and sweat gland manifestations are discussed. There are at least 3 possible approaches to screening for cystic fibrosis: sweat testing, evaluation of pancreatic function, or detection of some product of the defective gene. Prenatal diagnosis and heterozygote testing, diagnosis in adults, and diagnostic problems are also examined. Treatment includes prevention, pulmonary therapy, and gastrointestinal therapy. Pulmonary and other complications and their treatment are discussed, as well as psychosocial aspects, and evaluation and prognosis of cystic fibrosis. RF 001 ROCHHOLZ EL KINDERLIED UND KINDERSPIEL AU 280 857 002 LANDSTEINER K ZENTRALBL ALLG PATHOL 16 903 905 003 GARROD AE Q J MED 6 242 913 004 BLACKFAN KD J PEDIATR 3 679 933 005 BLACKFAN KD J PEDIATR 13 627 938 006 FANCONI G WIEN MED WOCHENSCHR 86 753 936 007 ANDERSEN DH AM J DIS CHILD 56 344 938 009 ANDERSEN DH AM J DIS CHILD 70 100 945 010 HARRIS R ARCH DIS CHILD 30 424 955 011 GIBSON LE PEDIATRICS 23 545 959 012 DENTON R AM J DIS CHILD 82 433 951 013 DOYLE B PHYS THER REV 39 24 959 014 THACKER EW POSTURAL DRAINAGE AND RESPIRA 959 015 MATTHEWS LW J PEDIATR 65 558 964 016 DOERSHUK CF PEDIATRICS 36 675 965 017 WRIGHT SW AM J HUM GENET 20 157 968 018 KULCZYCKI LL AM J DIS CHILD 127 64 974 019 ANTONELLI M FRACASTORO (VERONA) 63 207 970 020 GOODCHILD MC ARCH DIS CHILD 49 739 974 021 SELANDER P ACTA PAEDIATR SCAND 51 65 962 022 REIDERMAN MI GENETIKA (SOV GENETICS) 7 148 971 023 STEINBERG AG AM J HUM GENET 12 416 960 024 DI SANTAGNESE PA N ENGL J MED 277 1287 967 025 VIVELL O MONATSSCHR KINDERHEILKD 111 62 963 026 BRUNECKY Z J MED GENET 9 33 972 027 BERNHEIM M PEDIATRIE 16 17 961 028 CARTER CO MOD PROBL PEDIATR 10 372 967 029 PUGH RJ ARCH DIS CHILD 42 544 967 030 HOUSTEK J REV MED LIEGE 22 421 967 031 SULTZ HA AM J PUBLIC HEALTH 56 1461 966 032 DANKS DM ANN HUM GENET 28 323 965 033 HALL BD J MED GENET 5 262 968 034 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 035 MERRITT AD J LAB CLIN MED 60 998 962 036 HONEYMAN MS AM J HUM GENET 17 461 965 037 GOODMAN HO AM J HUM GENET 4 59 952 038 SUPER M S AFR MED J 49 818 975 039 HARRIS RL PEDIATRICS 41 733 968 040 MACDOUGALL LG LANCET 2 409 962 041 WANG CI N ENGL J MED 279 1216 968 042 IKAI K ACTA PAEDIATR JAP 7 23 965 043 KANDAR HR ANTISEPTIC 58 863 961 044 REDDY CR J PEDIATR 75 522 969 045 COHEN LF GAP CONF REP PROB REPRO PHYSI 6 975 046 DANES BS J EXP MED 129 775 969 047 BEARN AG IN: MANGOS JA 21 973 048 DANES BS AM J HUM GENET 23 297 971 049 CONNEALLY PM TEX REP BIOL MED 31 639 973 050 TAYSI K N ENGL J MED 281 1108 969 051 REED GB J PATHOL 101 251 970 052 KULCZYCKI LL CLIN PEDIATR 3 692 964 053 BATTEN J LANCET 1 1348 963 054 ORZALESI MM ACTA PAEDIATR SCAND 52 267 963 055 HALLETT WY AM REV RESPIR DIS 92 714 965 056 BEARN AG CLIN GASTROENTEROL 2 515 973 057 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 058 KNUDSON AG JR AM J HUM GENET 19 388 967 059 VIRTANEN S J PEDIATR 68 139 966 060 STEINBERG AG AM J HUM GENET 8 177 956 061 GOTZ M Z KINDERHEILK 117 183 974 062 MILUNSKY A PEDIATRICS 42 501 968 063 SMITH DW LANCET 2 309 968 064 LINDENBAUM RH J MED GENET 9 232 972 065 TAUSSIG LM AM J DIS CHILD 125 495 973 066 FLUGE G AM J DIS CHILD 127 760 974 067 BURNELL RH AM J DIS CHILD 127 746 974 068 LEWIS MB AUSTRALAS J DERMATOL 13 105 972 069 LOX CD ARIZ MED 31 508 974 070 BITAR J J PEDIATR 71 123 967 071 MATTHEWS LW AM REV RESPIR DIS 88 199 963 072 CHERNICK WS PEDIATRICS 24 739 959 073 KOPITO L J PEDIATR 68 313 966 074 KOPITO LE FERTIL STERIL 24 499 973 075 HADORN B J PEDIATR 73 39 968 076 ZOPPI G ACTA PAEDIATR SCAND 59 692 970 077 RULE AH FERTIL STERIL 21 515 970 078 DEARBORN DG IN: MANGOS JA 179 976 079 GIBSON LE PEDIATRICS 48 695 971 080 MANGOS JA TEX REP BIOL MED 31 651 973 081 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 082 PAUNIER L PEDIATRICS 52 446 973 083 KAISER D PEDIATR RES 5 167 971 084 CHAUNCEY HH ARCH ORAL BIOL 7 707 962 085 CHERNICK WS J PEDIATR 59 890 961 086 SHWACHMAN H ANN NY ACAD SCI 93 600 962 087 BOTELHO SY J PEDIATR 83 601 973 088 DI SANTAGNESE PA PEDIATRICS 22 507 958 089 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 090 MANDEL ID AM J DIS CHILD 110 646 965 091 SCHULZ IJ J CLIN INVEST 48 1470 969 092 MANGOS JA PEDIATR RES 1 436 967 093 MANGOS JA SCIENCE 158 135 967 094 MANGOS JA PEDIATR RES 2 378 968 095 KAISER D IN: MANGOS JA 247 973 096 GUGLER EC J PEDIATR 71 585 967 097 BOAT TF PEDIATR RES 8 531 974 098 WARTON KL BR MED J 3 570 971 099 SATIR B J CELL BIOL 56 153 973 100 BRADDOCK LI CF CLUB ABST 17 969 101 LUNDGREN DW CLIN CHIM ACTA 62 357 975 102 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 103 MUNRO GF J BIOL CHEM 247 1272 972 104 MANDEL ID AM J DIS CHILD 113 431 967 105 CHERNICK WS J PEDIATR 65 694 964 106 RAO GJS J PEDIATR 80 573 972 107 BOAT TF IN: MANGOS JA 165 976 108 LILLIBRIDGE CB PEDIATRICS 53 913 974 109 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 110 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 111 LORIN MI BIORHEOLOGY 9 27 972 112 BETTELHEIM FA BIORHEOLOGY 8 129 971 113 STURGESS JM CLIN SCI 38 145 970 114 POTTER JL AM REV RESPIR DIS 99 909 969 115 SNARY D BIOCHEM BIOPHYS RES COMMUN 40 844 970 116 DEMAN J BIOCHIM BIOPHYS ACTA 297 486 973 117 FRIBERG S AM REV RESPIR DIS 108 1010 973 119 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 120 HAVEZ R CLIN CHIM ACTA 17 281 967 121 HAVEZ R CLIN CHIM ACTA 17 463 967 122 HAVEZ R IN: PEETERS H 16 343 968 123 DEGAND P IN: PEETERS H 16 361 968 124 HAVEZ R BULL SOC CHIM BIOL 51 245 969 125 ROUSSEL P CLIN CHIM ACTA 36 315 972 126 LAMBLIN G CLIN CHIM ACTA 36 329 972 127 DEGAND P BULL PHYSIOPATH RESPIR NANCY 9 199 973 128 ROUSSEL P J BIOL CHEM 250 2114 975 129 BOAT TF AM REV RESPIR DIS 110 428 974 130 LAMB D BR J DIS CHEST 66 239 972 131 RUBIN LS J PEDIATR 63 1120 963 132 BOAT TF CHEST 67 325 975 133 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 134 MANGOS JA PEDIATR RES 3 562 969 135 STURGESS JM BR J EXP PATHOL 54 388 973 136 MARTINEZ JR PEDIATR RES 9 463 975 137 MARTINEZ JR PEDIATR RES 9 470 975 138 SANCHIS J N ENGL J MED 288 651 973 139 WOOD RE AM REV RESPIR DIS 111 733 975 140 WARING WW N ENGL J MED 288 681 973 141 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 142 HILDING AC AM J PHYSIOL 191 404 957 143 VANAS A S AFR MED J 46 347 972 144 MAGID SL ARCH OTOLARYNGOL 86 212 967 145 SADE J AM REV RESPIR DIS 102 48 970 146 KING MA AM REV RESPIR DIS 110 740 974 147 LUCAS AM ARCH OTOLARYNGOL 20 518 934 148 BLAKE J J BIOMECH 8 179 975 149 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 150 YEATES DB ARCH DIS CHILD 51 28 976 151 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 152 BOXERBAUM B AM REV RESPIR DIS 108 777 973 153 SCHWARTZ RH AM J DIS CHILD 111 408 966 154 SOUTH MA J PEDIATR 71 645 967 155 HALBERT SP PEDIATRICS 26 792 960 156 BURNS MW LANCET 1 270 968 157 HABBOUSHE C PEDIATRICS 48 973 971 158 MAY JR ARCH DIS CHILD 47 908 972 159 HOIBY N SCAND J RESPIR DIS 56 38 975 160 CONOVER JH LANCET 2 1501 973 161 POLLEY MJ J MED GENET 11 249 974 162 PENNINGTON JE AM J MED 58 629 975 163 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 164 FALK GA AM REV RESPIR DIS 105 14 972 165 TURNER-WARWICK M BR J DIS CHEST 61 113 967 166 GORDON DS AM REV RESPIR DIS 108 127 973 167 SPOCK A PEDIATR RES 1 173 967 168 BOWMAN BH SCIENCE 164 325 969 169 CHERRY JD J PEDIATR 79 937 971 170 BESLEY GTN J MED GENET 6 278 969 171 POSSELT HG Z KINDERHEILK 110 93 971 172 MCCOMBS ML CLIN GENET 1 171 970 173 CONOVER JH PEDIATR RES 7 220 973 174 DANES BS J EXP MED 136 1313 972 175 DOGGETT RG NATURE NEW BIOL 243 251 973 176 IACOCCA VF J PEDIATR 79 508 971 177 BOWMAN BH SCIENCE 167 871 970 178 DANES BS J EXP MED 137 1538 973 179 SCHMOYER IR LIFE SCI PART 2 11 1037 972 180 BERATIS NG PEDIATR RES 7 958 973 181 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 182 BARNETT DR TEX REP BIOL MED 31 691 973 183 CONOVER JH PEDIATR RES 7 224 973 184 BARNETT DR TEX REP BIOL MED 31 703 973 185 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 186 CONOVER JH LIFE SCI 14 253 974 187 BOKISCH VA J CLIN INVEST 49 2427 970 188 LIEBERMAN J AM REV RESPIR DIS 111 100 975 189 RAO GJS SCIENCE 177 610 972 190 LIEBERMAN J AM REV RESPIR DIS 109 399 974 191 RAO GJS PEDIATR RES 8 684 974 192 CONOVER JH LANCET 1 1194 973 193 WILSON GB PEDIATR RES 9 635 975 194 ALTLAND K HUMANGENETIK 28 207 975 195 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 196 FARRELL PM PEDIATR RES 10 127 976 197 COHEN FL J MED GENET 11 253 974 198 WOOD RE LANCET 2 1452 973 199 ANON GAP CONF REP CILIARY INHIBITO 973 200 COLE CH PEDIATR RES 6 616 972 201 BALFE JW SCIENCE 162 689 968 202 LAPEY A PEDIATR RES 4 478 970 203 FEIG SA PEDIATR RES 8 594 974 204 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 205 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 206 MCEVOY FA CLIN CHIM ACTA 54 195 974 207 QUISSELL DO NATURE 247 115 974 208 GIBBS GE PEDIATR RES 1 24 967 209 BENKE PJ LANCET 1 182 972 210 FLETCHER DS CLIN CHIM ACTA 44 5 973 211 FITZPATRICK DF NATURE NEW BIOL 235 173 972 212 DUFFY MJ CLIN CHIM ACTA 49 397 973 213 BAIG MM J PEDIATR 86 72 975 214 CHANGUS JE AM J PATHOL 80 317 975 215 BENKE PJ PROC SOC EXP BIOL MED 137 1283 971 216 TAYLOR A PEDIATR RES 8 861 974 217 COLE CH PEDIATR RES 9 763 975 218 TAUSSIG LM LANCET 1 1367 972 219 BROWN GA LANCET 2 639 971 220 MORIN CL BIOMEDICINE EXPRESS 19 133 973 221 ARAKI H PEDIATR RES 9 932 975 222 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 223 ZUELZER WW PEDIATRICS 4 53 949 224 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 225 REID L MOD PROBL PEDIATR 10 195 967 226 ESTERLY JR THORAX 23 670 968 227 BOWDEN DH AM J MED 38 226 965 228 WENTWORTH P THORAX 23 582 968 229 LAMARRE A PEDIATRICS 50 291 972 230 LANDAU LI AM REV RESPIR DIS 108 593 973 231 ZAPLETAL A PEDIATRICS 48 64 971 232 FOX WW PEDIATRICS 54 293 974 233 MELLINS RB PEDIATRICS 44 315 969 234 MANSELL A AM REV RESPIR DIS 109 190 974 235 BEIER FR AM REV RESPIR DIS 94 430 966 236 COOK CD PEDIATRICS 24 181 959 237 GODFREY S ARCH DIS CHILD 46 144 971 238 ZELKOWITZ PS J PEDIATR 74 393 969 239 DAY G ARCH DIS CHILD 48 355 973 240 LEVISON H IN: MANGOS JA 3 976 240A LIFSCHITZ MI AM REV RESPIR DIS 99 399 969 241 BOUHUYS A J APPL PHYSIOL 30 670 971 242 MELLINS RB PEDIATRICS 41 560 968 243 LANDAU LI AM REV RESPIR DIS 111 725 975 244 HODSON CJ CLIN RADIOL 13 54 962 245 SCHWARTZ EE AM J ROENTG RAD THER NUCL MED 122 708 974 246 DI SANTAGNESE PA DIS CHEST 27 654 955 247 HUANG NN J PEDIATR 59 512 961 248 IACOCCA VF AM J DIS CHILD 106 315 963 249 LAWSON D IN: WATT PJ 69 970 250 MEARNS MB ARCH DIS CHILD 47 902 972 251 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 252 REYNOLDS HY ANN INTERN MED 82 819 975 253 DOGGETT RG J PEDIATR 68 215 966 254 DOGGETT RG INFECT IMMUN 6 628 972 255 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 256 HOLSCLAW DS CLIN PEDIATR 9 346 970 257 LLOYD-STILL JD PEDIATRICS 53 678 974 258 SHWACHMAN H PEDIATRICS 30 389 962 259 RULON JT ARCH OTOLARYNGOL 78 192 963 260 HARRIS GBC POSTGRAD MED 34 251 963 261 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 111 1 962 262 HOLSCLAW DS AM J DIS CHILD 109 101 965 263 DONNISON AB PEDIATRICS 37 833 966 264 BERDON WE N ENGL J MED 277 585 967 265 MCPARTLIN JF ARCH DIS CHILD 47 207 972 266 FISHER OD ARCH DIS CHILD 29 262 954 267 CORDONNIER JK SURGERY 54 667 963 268 HUNTON DB GASTROENTEROLOGY 50 99 966 269 HOLSCLAW DS PEDIATRICS 48 51 971 270 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 271 KOPEL FB GASTROENTEROLOGY 62 483 972 272 HADORN B CAN MED ASSOC J 98 377 968 273 LAPEY A J PEDIATR 84 328 974 274 REEMTSMA K PEDIATRICS 22 525 958 275 GRYBOSKI JD GASTROENTEROLOGY 45 633 963 276 ANTONOWICZ I PEDIATRICS 42 492 968 277 WEBER AM N ENGL J MED 289 1001 973 278 HEIZER WD N ENGL J MED 285 257 971 279 MOSS AJ J PEDIATR 86 295 975 280 LEE PA JAMA 228 585 974 281 DOLAN TF JR CLIN PEDIATR 9 295 970 282 TORSTENSON OL PEDIATRICS 45 857 970 283 PETERSEN RA AM J DIS CHILD 116 662 968 284 UNDERWOOD BA PEDIATR RES 6 26 972 285 BLANC WA PEDIATRICS 22 494 958 286 KULCZYCKI LL N ENGL J MED 259 409 958 287 TAUSSIG LM RADIOLOGY 106 369 973 288 ALTERMAN K AM J DIS CHILD 101 210 961 289 BRAY PF AM J DIS CHILD 126 78 973 290 DOLAN TF JR CLIN PEDIATR 14 862 975 291 CRAIG JM AM J DIS CHILD 93 357 957 292 SHARP HL CF CLUB ABST 4 968 293 DI SANTAGNESE PA PEDIATRICS 18 387 956 294 VALMAN HB ARCH DIS CHILD 46 805 971 295 COOPER HS JOHNS HOPKINS MED J 135 268 974 296 BOAT TF CLIN PEDIATR 13 505 974 297 KATTWINKEL J J PEDIATR 82 234 973 298 WEBSTER R ARCH DIS CHILD 28 343 953 299 ROVSING H ACTA RADIOL DIAGN STOCKH 14 588 973 300 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 301 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 302 SHWACHMAN H PEDIATRICS 55 86 975 303 BARBERO GJ PEDIATRICS 29 788 962 304 WARWICK WJ 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CURRENT MICROBIOL 10 323 984 210 KOTTKE TE PREV MED 13 47 984 211 BLYTHE SA CLIN BIOCHEM 17 277 984 212 BOYD RL PEDIATR RES 18 1028 984 213 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 214 SPEERT DP PEDIATR RES 18 431 984 215 MCKEVITT AI J CLIN MICROBIOL 19 291 984 216 TANNENBAUM CS ANTIMICROB AGENTS CHEMOTHER 25 673 984 217 SCHIFF JB ANTIMICROB AGENTS CHEMOTHER 26 1 984 218 WILSON GB CLIN GENET 26 331 984 219 MCCLURE HM ADV VET SCI COMP MED 28 267 984 220 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 221 SMITH MJ THORAX 39 369 984 222 WELLS AL J FOOD NUTR 41 65 984 223 SPEERT DP INFECT IMMUN 43 1006 984 224 ORENSTEIN DM J APPL PHYSIOL 57 408 984 225 HODSON ME POSTGRAD MED J 60 225 984 226 JACKSON AH EUR J RESPIR DIS 65 114 984 227 LEWIS MI S AFR MED J 65 641 984 228 FARRELL PM PEDIATRICS 73 115 984 229 LINDEMANS J ACTA PAEDIATR SCAND 73 768 984 230 FICK RB J CLIN INVEST 74 236 984 231 KLINGER JD J CLIN INVEST 74 1669 984 232 PENNINGTON JE AM J MED 76 34 984 233 ROSENSTEIN BJ SOUTH MED J 77 1383 984 234 EFTHIMIOU J BR J DIS CHEST 78 299 984 235 DAVIS PB CHEST 85 802 984 236 THOMASSEN MJ J PEDIATR 104 352 984 237 WHEELER WB J PEDIATR 104 695 984 238 HENKE KG AM REV RESPIR DIS 129 708 984 239 WALL MA AM REV RESPIR DIS 129 557 984 240 PIER GB J IMMUNOL 133 734 984 241 SHERBROCKCOX V CARBOHYD RES 135 147 984 242 GILLARD BK AM J DIS CHILD 138 577 984 243 HANDELSMAN DJ N ENGL J MED 310 1670 984 244 ROOMANS GM ANN NY ACAD SCI 428 121 984 245 AUERBACH HS LANCET 2 686 985 246 KLINGER JD DIAGN MICROBIOL INFECT DIS 3 149 985 247 FOULSTON C J PEDIATR GASTROENTEROL NUTR 4 143 985 248 GOLD R PEDIATR INFECT DIS 4 172 985 249 KUHN RJ CLIN PHARMACY 4 555 985 250 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 251 DAVIS PB SEM RESPIR MED 6 261 985 252 LESTER LA SEM RESPIR MED 6 285 985 253 MISCHLER EH SEM RESPIR MED 6 271 985 254 ORENSTEIN DM SEM RESPIR MED 6 252 985 255 NEAL JL EVOLUTIONARY THEORY 7 153 985 256 SHRYOCK TR CURRENT MICROBIOL 12 91 985 257 SWOBODNIK W J CLIN ULTRASOUND 13 469 985 258 TOMASHEFSKI JF HUM PATHOL 16 253 985 259 FARRELL PM PEDIATR RES 19 104 985 260 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 261 KNOWLES MR PEDIATR RES 19 676 985 262 FINKELSTEIN SM METH INF MED 24 192 985 263 REED MD ANTIMICROB AGENTS CHEMOTHER 27 583 985 264 IRVIN RT CAN J MICROBIOL 31 268 985 265 REYNOLDS HY DISEASE A MONTH 31 1 985 266 BARDON A RES COMMUN CHEM PATH PHARM 47 449 985 267 BLACKWOOD LL INFECT IMMUN 47 840 985 268 ITANO M RESPIRATION 47 220 985 269 DORING G INFECT IMMUN 49 557 985 270 SORDELLI DO INFECT IMMUN 49 265 985 271 LUZAR MA INFECT IMMUN 50 577 985 272 BHAGAT RG ANN ALLERGY 54 297 985 273 KOMIYAMA K ORAL SURG 59 590 985 274 WINKLER U KLIN WSCHR 63 490 985 275 COALSON JJ BR J EXP PATHOL 66 205 985 276 SORDELLI DO EUR J RESPIR DIS 66 31 985 277 HAPONIK EF EUR J RESPIR DIS 67 381 985 278 SORDELLI DO EUR J RESPIR DIS 67 118 985 279 RAVIA Y HUM GENET 71 294 985 280 CEDER O ACTA PAEDIATR SCAND 74 102 985 281 COLLINS JE ACTA PAEDIATR SCAND 74 423 985 282 SCOGGIN CH POSTGRAD MED 77 243 985 283 BLUMER JL AM J MED 79 37 985 284 STUTTS MJ PROC NAT ACAD SCI USA 82 6677 985 285 COTTON DJ CHEST 87 217 985 286 STOKES DC CHEST 87 785 985 287 BROWN DT ANN OTOL RHINOL LARYNGOL 94 3 985 288 TABLAN OC J PEDIATR 107 382 985 289 TOMASHEFSKI JF ARCH PATHOL LAB MED 109 910 985 290 THOMASSEN MJ AM REV RESPIR DIS 131 791 985 291 ZACH MS AM REV RESPIR DIS 131 537 985 292 SZEINBERG A AM REV RESPIR DIS 132 766 985 293 TOURNIER JM AM REV RESPIR DIS 132 524 985 294 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 295 DALEY L J IMMUNOL 134 3089 985 296 BROWN J AM J DIS CHILD 139 836 985 297 WILMOTT RW AM J DIS CHILD 139 669 985 298 HILL DJS MED J AUST 143 230 985 299 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 300 OBERWALDNER B PEDIATR PULMONOL 2 358 986 301 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 302 OHMAN DE EUR J CLIN MICROBIOL 5 6 986 303 FRASER IM CLIN CHEST MED 7 131 986 304 KNOWLES MR CLIN CHEST MED 7 285 986 305 LEIGH MW EXP LUNG RES 10 153 986 306 KOHLER D ATEMWEGS LUNGENKRANKH 12 358 986 307 FRIEND PA J INFECT 13 55 986 308 PADGETT PJ CURRENT MICROBIOL 14 187 986 309 PEDERSEN SS J ANTIMICROB CHEMOTHER 17 505 986 310 BALTIMORE RS PEDIATR RES 20 1085 986 311 STUTTS MJ PEDIATR RES 20 1316 986 312 MACGEORGE J J MED MICROBIOL 21 331 986 313 SHRYOCK TR J CLIN MICROBIOL 23 513 986 314 KRIEG DP J CLIN MICROBIOL 24 986 986 315 MAHANEY MC ARCH ORAL BIOL 31 363 986 316 WARWICK WJ CLIN CHEM 32 850 986 317 JACOBSEN LE J CAN ASSOC RADIOL 37 17 986 318 FINKELSTEIN SM J CHRON DIS 39 195 986 319 HOIBY N ANNU REV MICROBIOL 40 29 986 320 MAHANEY MC HUM BIOL 58 445 986 321 DORING G CLIN EXP IMMUNOL 64 597 986 322 RODMAN HM MEDICINE 65 389 986 323 KOHLER D EUR J RESPIR DIS 69 319 986 324 MATTHYS H EUR J RESPIR DIS 69 311 986 325 BECK R ACTA PAEDIATR SCAND 75 639 986 326 KURLAND G PEDIATRICS 78 1097 986 327 ORENSTEIN DM PEDIATRICS 78 621 986 328 KUZEMKO JA J ROY SOC MED 79 2 986 329 ROSENSTEIN BJ SOUTH MED J 79 319 986 330 RUBIO TT AM J MED 81 73 986 331 GOWEN CW J PEDIATR 108 517 986 332 SMITH AL J PEDIATR 108 866 986 333 TOMASHEFSKI JF AM REV RESPIR DIS 133 535 986 334 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 335 MENTZ WM AM REV RESPIR DIS 134 938 986 336 STUTTS MJ AM REV RESPIR DIS 134 17 986 337 ROSENSTEIN BJ AM J DIS CHILD 140 966 986 338 KRUHLAK RT WEST J MED 145 196 986 339 PLOPPER CG ANAT EMBRYOL 174 167 986 340 TRIBBLE CG ANN SURG 204 677 986 341 DASGUPTA MK J CLIN IMMUNOL 7 51 987 342 SPEERT DP J HOSP INFECT 9 11 987 343 SHRYOCK TR CURRENT MICROBIOL 14 251 987 344 KOMIYAMA K CAN J MICROBIOL 33 27 987 345 NIELSEN OH CURR THER RES CLIN EXP 41 367 987 346 HOOKE AM INFECT IMMUN 55 99 987 347 COTTON CU J CLIN INVEST 79 80 987 348 HENDERSON WR J ALLERGY CLIN IMMUNOL 79 543 987 349 NUTMAN J J IMMUNOL 138 3481 987 350 DERETIC V J BACTERIOL 169 351 987 351 EMMETT M PROC SOC EXP BIOL MED 184 74 987 PN 76016 RN 00371 AN 76182691 AU Isenberg-J-N. Sahlstrom-S-J. Sharp-H-L. TI The glycoprotein-degrading potential of peripheral leukocytes in cystic fibrosis. SO Am-Rev-Respir-Dis. 1976 Apr. 113(4). P 567-9. MJ CYSTIC-FIBROSIS: bl. GLYCOPROTEINS: me. LEUKOCYTES: en. MN ACETYLGLUCOSAMINIDASE: bl. ADOLESCENCE. ADULT. ASPARTYLGLUCOSYLAMINASE: bl. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. FEMALE. FUCOSIDASE: bl. GALACTOSIDASES: bl. HUMAN. IN-VITRO. LYSOSOMES: en. MALE. MANNOSIDASES: bl. SUPPORT-U-S-GOVT-P-H-S. AB In this study, the potential problems and discrepancies in cill culture techniques were avoided by isolating leukocytes from the peripheral blood of patients with cystic fibrosis and healthy human volunteers and preparing homogenates to determine the specific activities of 5 lysosomal enzymes involved in the degradation of glycoproteins. Four lysosomal hydrolases involved in the breakdown of the carbohydrate side chain were assayed using artificial substrates. The fifth enzyme, aspartylglucosamine amido hydrolase, which cleaves the linkage between this side chain and the peptide backbone, was assayed with natural substrate. No differences in enzyme activities between the 2 leukocyte sources were observed. Because no enzyme activities were deficient and no compensatory increases were noted, we concluded that the thick, glycoprotein-rich, exocrine secretions in cystic fibrosis cannot be attributed to a defect in their catabolism. RF 001 DISCHE Z ANN NY ACAD SCI 93 526 962 002 KNAUFF RE CLIN CHIM ACTA 19 245 968 004 BARRETT AJ IN: DINGLE JT 116 973 005 LOWRY OH J BIOL CHEM 193 265 951 006 WIESMANN UN J PEDIATR 77 685 970 007 KRAUS I PEDIATRICS 47 1010 971 008 GIBBS GE SCIENCE 167 993 970 009 RUSSELL SB J MED GENET 8 441 971 010 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 011 SOMER H CLIN CHIM ACTA 60 219 975 012 LOUISOT P CLIN CHIM ACTA 48 373 973 013 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 2 MARGOLIES R PEDIATR RES 17 931 983 PN 76017 RN 00372 AN 77085824 AU Corey-M. Levison-H. Crozier-D. TI Five- to seven-year course of pulmonary function in cystic fibrosis. SO Am-Rev-Respir-Dis. 1976 Dec. 114(6). P 1085-92. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. EXPIRATORY-RESERVE-VOLUME. FEMALE. FOLLOW-UP-STUDIES. HUMAN. MALE. RESPIRATION. RESPIRATORY-FUNCTION-TESTS. SEX-FACTORS. AB The pulmonary function records of 132 patients with cystic fibrosis followed for 5 to 7 years were reviewed. Changes in forced vital capacity, 1-sec forced expiratory volume, mean forced expiratory flow during the middle half of the forced vital capacity, and 1-sec forced expiratory volume as a percentage of forced vital capacity were examined. There was considerable variation in the rates of change, but the general pattern was consistent with a theory of exponential decline, mean forced expiratory flow during the middle half of the forced vital capacity showing the earliest and most dramatic changes. The pulmonary function of 33 patients (25 per cent) remained stable or improved throughout follow-up, possibly reflecting mild forms of lung disease or the efficacy of therapy. Twenty of these patients (15 per cent) maintained completely normal pulmonary function. The rate of decline in pulmonary function values, with progress of the disease, was steeper in the female patients. RF 001 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 002 HUANG NN AM J DIS CHILD 120 289 970 003 SHWACHMAN H PEDIATRICS 46 335 970 004 DOERSHUK CF PEDIATRICS 36 675 965 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 TAUSSIG LM J PEDIATR 82 380 973 007 MEARNS MB THORAX 28 537 973 008 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 009 FEATHERBY EA AM REV RESPIR DIS 102 737 970 010 EMIRGIL C AM J MED 51 504 971 011 SEELY JE J APPL PHYSIOL 36 34 974 012 HIGGINS MW AM REV RESPIR DIS 108 258 973 013 SNEDECOR GW STATISTICAL METHODS 967 014 BATES DV AM REV RESPIR DIS 108 1043 973 015 DIENER CF AM REV RESPIR DIS 111 719 975 CT 1 DAVIDSON GP J PEDIATR 93 976 978 2 MELLIS CM AM REV RESPIR DIS 117 911 978 3 ZAPLETAL A BULL EUR PHYSIOPATH RESP 15 575 979 4 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 5 KRAEMER R HELV PAEDIATR ACTA 34 417 979 6 SANTAGNESE PAD AM J MED 66 121 979 7 MILLIGAN DWA J PEDIATR 95 24 979 8 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 9 SVENONIUS E RESPIRATION 40 226 980 10 RUSSELL NJ BR J DIS CHEST 76 35 982 11 GASKIN K J PEDIATR 100 857 982 12 MCLAUGHLIN FJ J PEDIATR 100 863 982 13 NOLAN G J PEDIATR 101 626 982 14 REDDING GJ AM REV RESPIR DIS 126 31 982 15 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 16 WILMOTT RW ARCH DIS CHILD 58 835 983 17 WELLS AL J FOOD NUTR 41 65 984 18 LEWIS MI S AFR MED J 65 641 984 19 WHEELER WB J PEDIATR 104 695 984 20 WANG EEL N ENGL J MED 311 1653 984 21 DAVIS PB SEM RESPIR MED 6 261 985 22 LAMBERTY JM ANAESTHESIA 40 448 985 23 WIESEMANN HG MONATSSCHR KINDERHEILKD 133 726 985 24 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 25 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 26 MILLER RW MED PEDIATR ONCOL 14 202 986 27 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 28 PRICE JF J ROY SOC MED 79 10 986 29 WANG EEL PEDIATR INFECT DIS J 6 256 987 30 ANDREASSON B ACTA PAEDIATR SCAND 76 70 987 PN 76018 RN 00373 AN 77132888 AU Yaffe-S-J. TI Developmental factors influencing interactions of drugs. SO Ann-NY-Acad-Sci. 1976. 281. P 90-7. MJ AGING. DRUGS: me. MN BLOOD-PROTEINS: me. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. FEMALE. FETUS: me. GASTROINTESTINAL-SYSTEM: me. HUMAN. HYPERBILIRUBINEMIA: me. INFANT. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: me. INTESTINAL-ABSORPTION. PREGNANCY. PROTEIN-BINDING. SUPPORT-U-S-GOVT-P-H-S. EX The author has attempted to summarize the current state of knowledge regarding dispositional processes in infants and children of various ages. The traditional processes of absorption, distribution, metabolism, and excretion are described not only in newborn infants but also in infants and children of other age groups. Knowledge of the effects of age and the effects of diseases, which are often unique to each stage of postnatal development, upon drug action and disposition is a vital and necessary prerequisite to a discussion of interactions of drugs of abuse in the pediatric population. It is essential that the developmental status of the host be taken into consideration when examining interactions of drugs in infants and children. The mechanisms of interactions are similar to those seen in the adult patient. The developmental status of the pediatric patient also plays a significant role in the manifestations of disease seen in sick infants and children. These are often profound and consequently may play a major role in modifying drug action and interaction. RF 001 JUSKO WJ PEDIATRICS 45 945 970 002 KRASNER J ANN NY ACAD SCI 226 101 973 003 RANE A J PEDIATR 78 877 971 004 WINSBERG BG IN: FORREST IS 974 005 CIANCIO SG J PERIODONTOL 43 411 972 006 HOPPEL CC IN: MORSELLI S 975 007 STERN L AM J DIS CHILD 120 26 970 009 ALVARES AP CLIN PHARMACOL THER 17 179 975 010 JUSKO WJ PEDIATRICS 56 1038 975 011 REIDENBERG MM ANN NY ACAD SCI 281 1 976 012 HURWITZ A ANN NY ACAD SCI 281 98 976 PN 76019 RN 00374 AN 77064517 AU Boat-T-F. Cheng-P-W. Iyer-R-N. Carlson-D-M. Polony-I. TI Human respiratory tract secretion. Mucous glycoproteins of nonpurulent tracheobronchial secretions, and sputum of patients with bronchitis and cystic fibrosis. SO Arch-Biochem-Biophys. 1976 Nov. 177(1). P 95-104. MJ BRONCHITIS: me. CYSTIC-FIBROSIS: me. GLYCOPROTEINS. MUCUS: se. RESPIRATORY-SYSTEM: se. SPUTUM: se. MN ADOLESCENCE. AMINO-ACIDS: an. CASE-REPORT. FEMALE. FUCOSE: an. GALACTOSE: an. GLYCOPROTEINS: se. HEXOSAMINES: an. HUMAN. SIALIC-ACIDS: an. SULFURIC-ACIDS: an. SUPPORT-U-S-GOVT-P-H-S. AB Mucous glycoproteins were isolated by agarose gel filtration from nonpurulent tracheobronchial secretions and purulent sputum which had been reduced, carboxymethylated and, in the case of purulent secretions, treated with deoxyribonuclease. The solubilized and purified glycoproteins were fractioned on diethylaminoethyl cellulose into two major (I, II) and two minor (Ia, III) blood group active components. Components I and II had similar carbohydrate and amino acid compositions which were typical for human blood group substances. These two components did differ in several respects. Component I contained 1.4-2.6% sulfate and did not inhibit influenza virus hemagglutination while component II contained 7.1-7.8% sulfate and was a potent inhibitor of virus hemagglutination. Component II also migrated more rapidly on sodium dodecyl sulfate-3.3% acrylamide gel electrophoresis. Components I and II in purulent secretions displayed only minor compositional differences from their counterparts in nonpurulent secretions. Component II was more abundant in two sputum samples from subjects with cystic fibrosis than in purulent bronchitic secretions or in nonpurulent secretions. RF 001 YEAGER H JR AM J MED 50 493 971 002 TOREMALM NG ACTA OTOLARYNGOL STOCKH SUPPL 158 43 960 003 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 004 HAVEZ R CLIN CHIM ACTA 17 281 967 005 HAVEZ R CLIN CHIM ACTA 17 463 967 006 HAVEZ R BULL SOC CHIM BIOL 51 245 969 007 HAVEZ R IN: PEETERS H 16 343 968 008 ROUSSEL P CLIN CHIM ACTA 36 315 972 009 LAMBLIN G CLIN CHIM ACTA 36 329 972 010 DEGAND P BULL PHYSIOPATH RESPIR NANCY 9 199 973 011 MASSON PL BIOCHIM BIOPHYS ACTA 111 466 965 012 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 013 ROUSSEL P J BIOL CHEM 250 2114 975 014 ROBERTS GP EUR J BIOCHEM 50 265 974 015 LAMBLIN G BIOCHIM BIOPHYS ACTA 322 372 973 016 BROGAN TD BIOCHEM J 71 125 959 017 BISERTE G LILLE MED 6 52 961 018 BUKANTZ SC J ALLERGY 29 29 958 019 BROGAN TD BR J EXP PATHOL 41 288 960 020 LOWRY OH J BIOL CHEM 193 265 951 021 BURTON K BIOCHEM J 62 315 956 022 ANTONOPOULOS CA ACTA CHEM SCAND 16 1521 962 023 COTLOVE E J LAB CLIN MED 51 461 958 024 DUBOIS M ANAL CHEM 28 350 956 025 BOAT TF AM REV RESPIR DIS 110 428 974 026 DAVIS BJ ANN NY ACAD SCI 121 404 964 027 REISFELD RA NATURE 195 281 962 028 WEBER K METHODS ENZYMOL 26 3 972 029 GLOSSMAN H J BIOL CHEM 246 6339 971 030 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 031 PRICE VE J BIOL CHEM 171 477 947 032 IYER RN ARCH BIOCHEM BIOPHYS 142 101 971 033 ROVIS L BIOCHEMISTRY 12 1955 973 034 LUCAS AM ARCH OTOLARYNGOL 20 518 934 035 SPIRO RG ADV PROT CHEM 27 349 973 036 GOTTSCHALK A IN: GOTTSCHALK A 2 810 972 037 WATKINS WM IN: GOTTSCHALK A 830 972 038 HOLDEN KG BIOCHEMISTRY 10 3105 971 039 PITT-RIVERS R BIOCHEM J 109 825 968 040 SPRINGER GF PROC NAT ACAD SCI USA 64 635 969 041 SNARY D BIOCHEM BIOPHYS RES COMMUN 40 844 970 042 MOORE S J BIOL CHEM 238 235 963 043 CRESTFIELD AM J BIOL CHEM 238 622 963 044 JONES R HISTOCHEM J 5 19 973 045 DEGAND P BIOCHIM BIOPHYS ACTA 320 318 973 046 NEUBERGER A IN: GOTTSCHALK A 1 474 972 047 KEAL EE THORAX 27 643 972 048 CARLSON DM IN: AMINOFF D 229 970 049 LEV R AM J PATHOL 46 23 965 050 LAMB D BR J DIS CHEST 66 239 972 CT 1 BOAT TF PEDIATR RES 11 977 977 2 CREETH JM BIOCHEM J 167 557 977 3 GALLAGHER JT TRENDS BIOCHEM SCI 3 38 978 4 ALHADEFF JA CLIN GENET 14 189 978 5 FORSTNER JF DIGESTION 17 234 978 6 MARTIN GP GUT 19 103 978 7 CLAMP JR BR MED BULL 34 25 978 8 CREETH JM BR MED BULL 34 17 978 9 CHENG PW ANAL BIOCHEM 85 276 978 10 ROUSSEL P LUNG 154 241 978 11 SACHDEV GP BIOCHIM BIOPHYS ACTA 536 184 978 12 BOAT TF BIOCHIM BIOPHYS ACTA 540 127 978 13 MAWHINNEY TP PEDIATR RES 13 760 979 14 FELDHOFF PA BIOCHEMISTRY 18 2430 979 15 ROSE MC BIOCHEMISTRY 18 4030 979 16 LAMBLIN G BIOCHIMIE 61 23 979 17 KAIZU T COMP BIOCHEM PHYSIOL (B) 62 195 979 18 JAKEL HP BIOL ZENTRALBL 98 55 979 19 HERZBERG MC J BIOL CHEM 254 1487 979 20 LIAO TH BIOCHIM BIOPHYS ACTA 577 442 979 21 CLARK JN BIOCHIM BIOPHYS ACTA 588 357 979 22 SACHDEV GP BIOCHEM MED 24 82 980 23 LAST JA ENVIRON HEALTH PERSPECT 35 131 980 24 BOAT TF FED PROC 39 3067 980 25 SHELHAMER JH J CLIN INVEST 66 1400 980 26 MAWHINNEY TP ANAL BIOCHEM 101 112 980 27 ESTEP JA J APPL PHYSIOL 50 383 981 28 CHENG PW ANAL BIOCHEM 117 301 981 29 BROWN DT AM REV RESPIR DIS 124 285 981 30 SHERMAN JM AM REV RESPIR DIS 124 476 981 31 BHASKAR KR J BIOL CHEM 256 7583 981 32 HOUDRET N BIOCHIM BIOPHYS ACTA 668 413 981 33 ADLER KB EXP LUNG RES 3 69 982 34 TABAK LA J ORAL PATHOL 11 1 982 35 BLITZER MG PEDIATR RES 16 203 982 36 BOAT TF PEDIATR RES 16 792 982 37 WOODWARD H BIOCHEMISTRY 21 694 982 38 WILLIAMS IP EUR J RESPIR DIS 63 510 982 39 SNYDER CE CARBOHYD RES 105 87 982 40 GALLAGHER JT ADV EXP MED BIOL 144 335 982 41 REID L ADV EXP MED BIOL 144 369 982 42 MIAN N BIOCHEM J 201 533 982 43 DOUGLAS WHJ ANAT REC 202 285 982 44 CHENG PW J BIOL CHEM 257 6251 982 45 REID L EXP LUNG RES 4 157 983 46 FRATES RC PEDIATR RES 17 30 983 47 MARGOLIES R PEDIATR RES 17 931 983 48 WESLEY A PEDIATR RES 17 65 983 49 VERDUGO P BIORHEOLOGY 20 223 983 50 BLOOMFIELD VA BIOPOLYMERS 22 2141 983 51 KOHLER D KLIN WSCHR 61 243 983 52 CHACE KV CLIN CHIM ACTA 132 143 983 53 HOUDRET N BIOCHIM BIOPHYS ACTA 758 24 983 54 WOOD RE SEM RESPIR MED 5 336 984 55 SHELHAMER JH EXP LUNG RES 7 149 984 56 ROUSSEL P BIOCHEM SOC TRANS 12 617 984 57 LLOYD C IN VITRO 20 416 984 58 WINGERT WE BIOCHEMISTRY 23 690 984 59 VISHWANATH S INFECT IMMUN 45 197 984 60 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 61 STELANDER M EUR J RESPIR DIS 65 5 984 62 KALINER M J ALLERGY CLIN IMMUNOL 73 318 984 63 BASBAUM CB PROC NAT ACAD SCI USA 81 4419 984 64 JAHNKE GD PROC NAT ACAD SCI USA 81 578 984 65 VERDUGO P CIBA FOUND SYMP 109 212 984 66 TABAK LA CARBOHYD RES 135 117 984 67 BHASKAR KR EXP LUNG RES 9 289 985 68 MIKKELSEN A INT J BIOMEDICAL COMPUT 17 215 985 69 RAM BP CRC CRIT REV BIOCHEM 17 257 985 70 CARLSTEDT I ESSAYS BIOCHEM 20 40 985 71 CHACE KV BIOCHEMISTRY 24 7334 985 72 MIKKELSEN A BIOPOLYMERS 24 1683 985 73 REDDY MS J DENT RES 64 33 985 74 CHENG PW BIOCHEM J 227 405 985 75 KOHLER D ATEMWEGS LUNGENKRANKH 12 358 986 76 ROOMANS GM J SUBMICROSC CYTOL 18 613 986 77 MILLS AN BR J EXP PATHOL 67 821 986 78 KOHLER D EUR J RESPIR DIS 69 319 986 79 KALINER M AM REV RESPIR DIS 134 612 986 80 LEIGH MW AM REV RESPIR DIS 134 784 986 81 CHENG PW CARBOHYD RES 149 253 986 82 LHERMITTE M LUNG 164 217 986 83 BASBAUM CB ARCH BIOCHEM BIOPHYS 249 363 986 84 MAWHINNEY TP J CHROMATOGR 351 91 986 85 MAWHINNEY TP J BIOL CHEM 262 2994 987 PN 76020 RN 00375 AN 76267483 AU Mayo-J-W. Wallace-W-M. Matthews-L-W. Carlson-D-M. TI Quantitation of submandibular proteins resolved from normal individuals and children with cystic fibrosis. SO Arch-biochem-biophys. 1976 Aug. 175(2). P 507-13. MJ CYSTIC-FIBROSIS: me. PROTEINS: me. SUBMANDIBULAR-GLAND: me. MN ADOLESCENCE. AMYLASES: me. CHILD. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FEMALE. HUMAN. KINETICS. MALE. SUPPORT-U-S-GOVT-P-H-S. AB Submandibular secretions collected from children with cystic fibrosis (CF) showed increased protein concentrations (milligrams/milliliter) and increased amylase specific activity (units/milligram of protein) relative to normals secretions. These differences between normal (N) and CF secretions were as follows: protein, 1.25 plus or minus 0.51 (N), 1.75 plus or minus 0.35 (CF) (P < 0.02); and amylase, 58 plus or minus 18 (N), 80 plus or minus 19 (CF) (P < 0.001). To determine the basis for elevated protein in CF saliva, several major proteins resolved by polyacrylamide disc gel electrophoresis were quantitated by densitometry. These included four phosphoproteins (PP), serum albumin, an acid phosphatase-containing fraction, amylase, and an unidentified protein referred to as PI-7.1. Together, these proteins comprise greater than 75% of the total protein in the secretion. Differences in individual protein concentrations (milligrams/milliliter) resolved from normal and CF secretions, respectively, were as follows: PP2, 0.02 plus or minus 0.01, 0.03 plus or minus 0.02 (NS, not significant); PP3, 0.06 plus or minus 0.04, 0.05 plus or minus 0.03 (NS); acid phosphatase fraction, 0.06 plus or minus 0.04, 0.12 plus or minus 0.07 (P < 0.05); amylase, 0.09 plus or minus 0.04, 0.27 plus or minus 0.16 (P < 0.01); and pI-7.1, 0.04 plus or minus 0.02, 0.13 plus or minus 0.08 (P < 0.02). Amylase, the most significant contributor to the elevated protein, comprised 26% of the total protein of normal secretions and 38% of the total protein of CF secretions. Thus, our results do not support the concept of a generalized increase in all organic components in CF submandibular secretions but, rather, increases in specific proteins, namely amylase, component pI-7.1, and an acid phosphatase-containing fraction. RF 001 CHERNICK WS J PEDIATR 59 890 961 002 CHERNICK WS J PEDIATR 65 694 964 003 MANDEL ID AM J DIS CHILD 113 431 967 004 BLOMFIELD J ARCH DIS CHILD 48 267 973 005 BLOMFIELD J AUST PAEDIATR J 9 232 973 006 BLOMFIELD J GUT 14 558 973 007 MAYO JW FED PROC 29 409 970 008 MAYO JW ARCH BIOCHEM BIOPHYS 161 134 974 009 DAVIS BJ ANN NY ACAD SCI 121 404 964 010 REISFELD RA NATURE 195 281 962 011 KRUSKI AW ANAL BIOCHEM 60 431 974 012 LOWRY OH J BIOL CHEM 193 265 951 013 BERNFELD P METHODS ENZYMOL 1 149 955 014 MAYO JW ARCH BIOCHEM BIOPHYS 163 498 974 015 HSIU J BIOCHEMISTRY 3 61 964 016 BOAT TF PEDIATR RES 8 531 974 017 GOROVSKY MA ANAL BIOCHEM 35 359 970 018 DAWES C ARCH ORAL BIOL 19 887 974 019 WALLACH D EUR J BIOCHEM 21 433 971 020$ FISCHER EH IN: BOYER PD 4 322 960 021 BDOLAH A BIOCHEM BIOPHYS RES COMMUN 8 266 962 022 SPEIRS R ARCH ORAL BIOL 19 747 974 023 CHERNICK WS MOD PROBL PEDIATR 10 125 967 024 MARTINEZ JR PEDIATR RES 9 470 975 CT 1 GILLARD BK PEDIATR RES 12 868 978 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 ROOMANS GM ANN NY ACAD SCI 428 121 984 4 VONEULER AM EXP MOL PATH 43 142 985 5 DAVIS PB J DENT RES 66 667 987 6 MCCARTHY D BR DENT J 162 148 987 PN 76021 RN 00376 AN 76230443 AU Hochman-H-I. Feins-N-R. Rubin-R. Gould-J. TI Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis. SO Arch-Dis-Child. 1976 May. 51(5). P 390-1. MJ ALKALOSIS: et. CYSTIC-FIBROSIS: co. DIARRHEA-INFANTILE: et. MN CASE-REPORT. CHLORIDES: me. FECES: an. FEMALE. HUMAN. ILEOSTOMY: ae. INFANT-NEWBORN. AB A case of hypochloraemic metabolic alkalosis in an infant with chloride losing ileostomy drainage and cystic fibrosis is described. It is speculated that intestinal loss of chloride played a major role in the development of metabolic alkalosis. RF 001 AARONSON I ARCH DIS CHILD 46 479 971 002 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 003 DAVIDSON AG AUST PAEDIATR J 8 187 972 004 DESOUSA RC J CLIN INVEST 53 465 974 005 DI SANTAGNESE PA JAMA 172 2014 960 006 GOTTLIEB RP J PEDIATR 79 930 971 007 KESSLER WR PEDIATRICS 8 648 951 008 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 009 SCHWARTZ WB N ENGL J MED 279 630 968 CT 1 HOCHMAN HI PEDIATR CLIN NORTH AM 26 803 979 2 BECKERMAN RC PEDIATRICS 63 580 979 3 NUSSBAUM E AM J DIS CHILD 133 965 979 4 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 PN 76022 RN 00377 AN 76276665 AU Lacroix-J. TI Letter: Life tables for cystic fibrosis. SO Arch-Dis-Child. 1976 Aug. 51(8). P 645. MJ CYSTIC-FIBROSIS: mo. LIFE-EXPECTANCY. MN AGE-FACTORS. CYSTIC-FIBROSIS: di. HUMAN. INFANT. EX I read the article by Drs. Robinson and Norman on life tables for cystic fibrosis with much interest. They express their disappointment at not finding any difference between the life expectancy of children presenting during the first year and that of all cases. In fact the former may be a little worse. Is it not what should be expected? Since infection is known to be present as early as the first month of life, the diagnosis is probably late in nearly all cases. Do the authors not point out the necessity of an evaluation of neonatal screening? On the other hand, and logically, one can expect that a very late diagnosis is consistent nowadays with a very mild case - and the best therapeutic results! Shwachman's report of patients over 17 years of age seems to support this belief: only 12% of the series had been diagnosed before one year of age. Therefore I think we should evaluate the course of late presenting cases (after one year of age for instance) separately since a large delay in diagnosis may be a clue to the mildness of the disease. RF 001 ROBINSON MJ ARCH DIS CHILD 50 962 975 002 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 003 SHWACHMAN H PEDIATRICS 36 689 965 PN 76023 RN 00378 AN 76204630 AU Griffiths-A-D. Bull-F-E. Dykes-P. TI Effect of gestational length on albumin content of meconium. SO Arch-Dis-Child. 1976 Apr. 51(4). P 321-3. MJ ALBUMINS: an. GESTATIONAL-AGE. MECONIUM: an. MN CYSTIC-FIBROSIS: di. FALSE-POSITIVE-REACTIONS. FEMALE. HUMAN. INFANT-NEWBORN. INFANT-PREMATURE. OCCULT-BLOOD. PREGNANCY. AB During a screening programme for the detection of CF using the meconium albumin technique, the overall false-positive rate was found to be approximately 1%. When the gestational age of the infants was taken into account the false-positive rate was found to be significantly higher in preterm (8%) as compared to term infants (0.55%). This was due largely but not solely to the presence of occult blood. Possible explanations for these findings are discussed and attention drawn to the limitation of meconium albumin content as a screening technique for CF in preterm infants. RF 001 BUCHANAN DJ PEDIATRICS 9 304 952 002 BULL FE ARCH DIS CHILD 49 602 974 003 CAIN ARR ARCH DIS CHILD 47 131 972 004 FORD-JONES AEA ARCH DIS CHILD 50 238 975 005 GREEN MN PEDIATRICS 41 989 968 006 GREEN MN PEDIATRICS 21 635 958 007 KOLMER JA APPROVED LABORATORY TECHNIC 266 952 008 PROSSER R ARCH DIS CHILD 49 597 974 009 SCHUTT WH ARCH DIS CHILD 43 178 968 010 WERNER B ACTA PAEDIATR SCAND SUPPL 6 35 1 948 011 WISER WC PEDIATRICS 33 115 964 CT 1 UXA F MINERVA PEDIATR 31 831 979 2 RYLEY HC ARCH DIS CHILD 54 92 979 PN 76024 RN 00379 AN 76204624 AU Robinson-P-G. Elliot-R-B. TI Cystic fibrosis screening in the newborn. SO Arch-Dis-Child. 1976 Apr. 51(4). P 301-4. MJ CYSTIC-FIBROSIS: di. ENZYME-TESTS. FECES: en. MN CHYMOTRYPSIN: an. HUMAN. INFANT-NEWBORN. SWEAT: en. TRYPSIN: an. AB A new technique of measuring stool enzyme activity on dry specimens of faeces from newborn children at 4-5 days of age has detected 3 cases of cystic fibrosis in the first 6000 tests. No known cases of cystic fibrosis have been missed. Additionally, one case of pancreatic achylia of at least 4 months' duration has been detected. It is proposed that the detection of cystic fibrosis by this technique is sufficiently practical to be acceptable as a worthwhile newborn screening programme. The screening test has been in use in Auckland for over a year and is now being set up in Hamilton, Wellington, and Dunedin (New Zealand), and Sydney (Australia). RF 001 BARBERO GJ AM J DIS CHILD 112 536 966 002 GIBSON LE PEDIATRICS 23 545 959 003 PROSSER R ARCH DIS CHILD 49 597 974 004 ROBINSON PG NZ MED J 79 1024 974 005 ROBINSON PG CLIN CHIM ACTA 62 225 975 006 RYLEY HC ARCH DIS CHILD 49 901 974 007 SCHWARTZ IL J CLIN INVEST 35 114 956 008 SHWACHMAN H PEDIATRICS 46 335 970 009 SHWACHMAN H AM J DIS CHILD 92 347 956 CT 1 CROSSLEY JR LANCET 2 1093 977 2 ARVANITAKIS C GASTROENTEROLOGY 74 932 978 3 EVANS RT J CLIN PATHOL 34 911 981 4 CRISTOL P SEM HOP PARIS 58 449 982 PN 76025 RN 00380 AN 76276664 AU Ryley-H-C. Neale-L-M. Prosser-R. Dodge-J. TI Screening for cystic fibrosis by analysis of serum protein in faeces. SO Arch-Dis-Child. 1976 Aug. 51(8). P 641-3. MJ ALBUMINS: an. ALPHA-1-ANTITRYPSIN: an. CYSTIC-FIBROSIS: di. FECES: an. MN COMPARATIVE-STUDY. HUMAN. MECONIUM: an. AB Faecal specimens from 51 infants free of cystic fibrosis (CF) and from 9 infants with the disease were analysed for albumin and alpha1- antitrypsin content. Faeces from infants with no CF had a mean albumin content of less than 0-1 mg/g dry weight and a mean albumin: alpha1-antitrypsin ratio value of less than 0-1. Faeces from infants with CF had, with one exception, an albumin content of more than 2-0 mg/g dry weight and a ratio value greater than 3-0. It was subsequently found that the duodenal aspirate from the child with CF but whose faeces had a low albumin content and ratio value, had tryptic activity though at a much reduced level compared to the activity in aspirates from healthy infants. RF 001 RYLEY HC ARCH DIS CHILD 49 901 974 002 RYLEY HC CLIN CHIM ACTA 64 117 975 CT 1 RYLEY HC ARCH DIS CHILD 54 92 979 2 MASTELLA G RIV ITAL PEDIATR 7 581 981 PN 76026 RN 00381 AN 76276646 AU Mukherji-R-N. Moss-P-H. Heffernan-C-K. TI Is cystic fibrosis an acid mucopolysaccharidosis?. SO Arch-Dis-Child. 1976 Jul. 51(7). P 563-5. MJ CYSTIC-FIBROSIS: cl. MUCOPOLYSACCHARIDOSIS: cl. MYOCARDIAL-DISEASES: cl. MN CASE-REPORT. CYSTIC-FIBROSIS: pa. FEMALE. HUMAN. INFANT. MUCOPOLYSACCHARIDOSIS: pa. MYOCARDIAL-DISEASES: pa. MYOCARDIUM: pa. AB A case of cystic fibrosis with myocardial fibrosis and acid mucopolysaccharide deposits in the myocardium is described. The case supports the theory that cystic fibrosis may be a genetic acid mucopolysaccharidosis. RF 001 BARNES GL AUST PAEDIATR J 6 81 970 002 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 003 JOHANSEN PG LANCET 1 455 968 004 MCGIVEN AR ARCH DIS CHILD 37 656 962 005 MOSQUERA F ARCH ARGENT PEDIATR 63 450 965 006 NEZELOF C ARCH FR PEDIATR 16 1035 959 007 OPPENHEIMER EH LAB INVEST 30 411 974 008 POWELL LW JR VA MED 84 178 957 CT 1 HIDE DW ARCH DIS CHILD 52 163 977 2 ZIMMERMANN A HELV PAEDIATR ACTA 37 183 982 PN 76027 RN 00382 AN 76276634 AU Warner-J-O. Taylor-B-W. Norman-A-P. Soothill-J-F. TI Association of cystic fibrosis with allergy. SO Arch-Dis-Child. 1976 Jul. 51(7). P 507-11. MJ CYSTIC-FIBROSIS: co. HYPERSENSITIVITY: co. MN ADOLESCENCE. ALLERGENS. ASPERGILLUS: im. BREAST-FEEDING. CHILD. CLADOSPORIUM: im. FEMALE. HAY-FEVER: co. HUMAN. HYPERSENSITIVITY: fg. HYPERSENSITIVITY-IMMEDIATE. INFANT. INTRADERMAL-TESTS. LUNG-VOLUME-MEASUREMENTS. MALE. PEAK-EXPIRATORY-FLOW-RATE. RESPIRATORY-TRACT-INFECTIONS: co. AB Immediate skin hypersensitivity to various inhalant allergens was present in 59% of 123 children with cystic fibrosis (CF), a much higher percentage than in the general population. This is consistent with the idea that atopy arises as a result of impaired handling of antigen at mucosal surfaces. The allergic CF children had more chest infections, a worse chest x-ray appearance, and lower peak expiratory flow rates. Allergic diseases were also frequent in the CF obligate heterozygotes (32% of mothers and 26% of fathers). It is suggested that the heterozygotes may also have a mucosal abnormality resulting in defective antigen handling. RF 001 BLAIR H CLIN ALLERGY 4 389 974 002 BOWMAN BH SCIENCE 164 325 969 003 CARR RD ARCH DERMATOL 89 27 964 004 CHANG N AM REV RESPIR DIS 106 867 972 005 CHRISPIN AR PEDIATR RADIOL 2 101 974 006 CRACCO G PROC EWGCF 5TH ANNU MTG 16 974 007 DAY G ARCH DIS CHILD 48 355 973 008 FEATHERBY EA CAN MED ASSOC J 102 835 970 009 HORN MEC CHEST SUPPL 63 44 973 010 KULCZYCKI LL JAMA 175 358 961 011 LANDAU LI J PEDIATR 82 863 973 012 LIFSCHITZ MI AM REV RESPIR DIS 99 399 969 013 MEARNS MB LANCET 1 538 967 014 RACHELEFSKY GS AM J DIS CHILD 128 355 974 015 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 016 SPOCK A PEDIATR RES 1 173 967 017 TAYLOR B LANCET 2 111 973 018 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 019 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 020 WARREN CPW CLIN ALLERGY 5 1 975 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 WARNER JO CLIN ALLERGY 7 385 977 3 DESPRES P NOUV PRESSE MED 7 2566 978 4 SILVERMAN M ARCH DIS CHILD 53 873 978 5 TURNER MW ARCH DIS CHILD 53 631 978 6 MELLIS CM PEDIATRICS 61 446 978 7 MITCHELL I J PEDIATR 93 744 978 8 ANON LANCET 1 708 979 9 PRICE JF CLIN ALLERGY 9 563 979 10 CARSWELL F MONOGR PAEDIATR 10 144 979 11 LEUPOLD W MONOGR PAEDIATR 10 119 979 12 CARSWELL F CLIN EXP IMMUNOL 35 141 979 13 ZAMBIE MF ANN ALLERGY 42 290 979 14 SANTAGNESE PAD AM J MED 66 121 979 15 LARSEN GL AM REV RESPIR DIS 119 399 979 16 NELSON LA AM REV RESPIR DIS 120 863 979 17 SLY PD AUST PAEDIATR J 16 205 980 18 VANASPEREN PP AUST PAEDIATR J 16 53 980 19 HODSON ME THORAX 35 801 980 20 ORMEROD LP THORAX 35 768 980 21 SKOV PS ALLERGY 35 23 980 22 TOBIN MJ THORAX 35 232 980 23 TOBIN MJ THORAX 35 807 980 24 BRUETON MJ ARCH DIS CHILD 55 348 980 25 GEORGE RH ARCH DIS CHILD 55 910 980 26 MOSS RB AM REV RESPIR DIS 121 23 980 27 LARSEN GL AM J DIS CHILD 134 1143 980 28 CROMWELL O LANCET 2 164 981 29 REEN DJ CLIN ALLERGY 11 571 981 30 SHEN J INFECT IMMUN 32 967 981 31 HOLZER FJ ARCH DIS CHILD 56 455 981 32 CLARKE CW BR J DIS CHEST 75 15 981 33 VANASPEREN P AM J DIS CHILD 135 815 981 34 WILSON GB MED HYPOTHESES 8 527 982 35 PITCHERWILMOTT RW ARCH DIS CHILD 57 582 982 36 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 37 PRIGENT F ANN DERMATOL VENEREOL 109 341 982 38 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 39 ANON LANCET 2 257 983 40 MURPHY MB THORAX 39 179 984 41 LAUFER P J ALLERGY CLIN IMMUNOL 73 44 984 42 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 43 LAUFER P CUTIS 35 557 985 44 LAUFER P CUTIS 36 245 985 45 BERKIN KE EUR J RESPIR DIS 67 103 985 46 HORDVIK NL AM REV RESPIR DIS 131 889 985 47 WILMOTT RW AM J DIS CHILD 139 669 985 48 DINWIDDIE R J ROY SOC MED 79 6 986 49 PIEDRA P J PEDIATR 108 817 986 PN 76028 RN 00383 AN 76251662 AU Yeates-D-B. Sturgess-J-M. Kahn-S-R. Levison-H. Aspin-N. TI Mucociliary transport in trachea of patients with cystic fibrosis. SO Arch-Dis-Child. 1976 Jan. 51(1). P 28-33. MJ CYSTIC-FIBROSIS: pp. TRACHEA: pp. MN ADOLESCENCE. ADULT. CHILD. CILIA. CYSTIC-FIBROSIS: bl, co. FEMALE. HUMAN. MALE. SPUTUM: se. AB Mucociliary tracheal transport rates were measured in 20 patients with cystic fibrosis, in whom these rates ranged from 0 to 12.8 mm/min. The patients were divided into 3 roughly equal groups on the basis of their transport rates. (1) Those in whom no abnormality in mucociliary transport was detected in the trachea; (2) those in whom normal transport rates were measured but in whom abnormalities such as cessation, or reversal of bolus movement were observed; (3) those in whom no normal transport rates were observed. In the first group the rates were similar to those observed in a population of healthy adults. These normal rates were observed in some patients who had a productive cough. The mean mucociliary tracheal transport rate increased with increasing maximum midexpiratory flow. Those patients with a low Shwachman score and poor arterial oxygen tension tended to fall into groups 2 and 3. In the ciliary dyskinesia assay in rabbit trachea the serum from the patients with the higher transport rates tended to initiate more rapid discharge of material from the epithelium and ciliary dyskinesia. RF 001 CAMNER P AM REV RESPIR DIS 108 131 973 002 CAMNER P IN: HERS JFP 236 973 003 CHARMAN J BIORHEOLOGY 9 185 972 004 CONOVER JH PEDIATR RES 7 220 973 005 DENTON R PEDIATRICS 25 611 960 006 DUBOIS AB J CLIN INVEST 35 322 956 007 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 008 FEATHER EA BR J DIS CHEST 64 192 970 009 HOORN B ARCH GESAMTE VIRUSFORSCH 18 210 966 010 LAMB D BR J DIS CHEST 66 239 972 011 MATTHEWS LW AM REV RESPIR DIS 88 199 963 012 POTTER JL ANN NY ACAD SCI 106 692 963 013 REID L MOD PROBL PEDIATR 10 195 967 014 SAKAKURA Y ANN OTOL RHINOL LARYNGOL 82 203 973 015 SANCHIS J N ENGL J MED 288 651 973 016 SPOCK A PEDIATR RES 1 173 967 017 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 018 THOMSON ML N ENGL J MED 289 749 974 019 WOOD RE PEDIATR RES 8 471 974 020 YEATES DB INT J APPL RADIAT ISOTOP 25 578 974 021 YEATES DB FED PROC 33 365 974 022 YEATES DB J APPL PHYSIOL 39 487 975 023 ZUELZER WW PEDIATRICS 4 53 949 CT 1 CZEGLEDYNAGY E LAB INVEST 35 588 976 2 SORENSEN RU INFECT IMMUN 18 735 977 3 WONG JW PEDIATRICS 60 146 977 4 ROSSMAN CM J PEDIATR 90 579 977 5 LOPEZVIDRIERO MT BR MED BULL 34 63 978 6 KOLLBERG H SCAND J RESPIR DIS 59 297 978 7 BASSETT PG COMPUT BIOL MED 9 97 979 8 GABRIDGE MG PEDIATR RES 13 31 979 9 NAGY EC PEDIATR RES 13 729 979 10 AFZELIUS BA INT REV EXP PATHOL 19 1 979 11 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 12 MOSSBERG B ACTA PHARMACOL TOXICOL 44 41 979 13 CAMNER P SCAND J RESPIR DIS 60 56 979 14 PAVIA D CLIN RESP PHYSIOL 16 335 980 15 LIPPMANN M BR J IND MED 37 337 980 16 CAMNER P CLIN SCI 59 79 980 17 MOSSBERG B EUR J RESPIR DIS 61 51 980 18 KATZ SM AM J CLIN PATHOL 73 682 980 19 STURGESS JM N ENGL J MED 303 318 980 20 SORENSEN RU PEDIATR RES 15 14 981 21 RUTLAND J THORAX 36 654 981 22 MORROW PE CHEST 80 809 981 23 SORENSEN RU AM REV RESPIR DIS 123 37 981 24 MITCHELL EA AUST PAEDIATR J 18 40 982 25 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 26 SORENSEN RU EUR J RESPIR DIS 64 524 983 27 JACKSON AH CLIN SCI 65 P 3 983 28 RUTLAND J AM REV RESPIR DIS 128 1030 983 29 KATZ S CELL CALC 5 421 984 30 CAMNER P ATEMWEGS LUNGENKRANKH 10 237 984 31 JACKSON AH EUR J RESPIR DIS 65 114 984 32 DAVIS PB SEM RESPIR MED 6 261 985 33 LEVANDOWSKI RA J LAB CLIN MED 106 428 985 PN 76029 RN 00384 AN 77086296 AU Lawson-D. Porter-J. TI Serum precipitins against respiratory tract pathogens in 522 "normal" children, and 48 cases of cystic fibrosis treated with cloxacillin. SO Arch-Dis-Child. 1976 Nov. 51(11). P 890-1. MJ CLOXACILLIN: tu. CYSTIC-FIBROSIS: im. PRECIPITINS: an. RESPIRATORY-TRACT-INFECTIONS: im. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt. HUMAN. INFANT. INFANT-NEWBORN. EX Well children show a steady increase of staphylococcal precipitins throughout life. Lower incidences are found for precipitins against H. influenzae, Pneumococcus, and haemolytic streptococcus. Precipitins against Proteus, Pseudomonas, and Klibsiella are rare. There are marked differences between the normal children and the cloxacillin-treated children with cystic fibrosis in respect to Pseudomonas and H. influenzae which rise to over 50% of cystic fibrosis children in the third quinquennium. However, the development of staphylococcal precipitins can be prevented by continuous antistaphylococcal therapy. RF 001 BURNS MW LANCET 1 223 968 002 BURNS MW LANCET 1 354 967 003 BURNS MW LANCET 1 270 968 CT 1 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 PN 76030 RN 00385 AN 76252000 AU Usatin-M-S. Wigger-H-J. TI Peliosis hepatis in a child. SO Arch-Pathol-Lab-Med. 1976 Aug. 100(8). P 419-21. MJ CYSTIC-FIBROSIS: co. LIVER-DISEASES: pa. LIVER: pa. MN AGE-FACTORS. AUTOPSY. CASE-REPORT. CHILD. CYSTIC-FIBROSIS: pa. HUMAN. LIVER-DISEASES: co. MALE. AB Peliosis hepatis, which has previously only been reported in adults, occurred in a chronically ill 11-year-old boy with cystic fibrosis. As in cases in adults, chronic illness, long-term anabolic steroid therapy, and right ventricular congestive failure may well have been the causative factors in this case. Histologically, the phlebectatic and parenchymal types, which were presumed to be separate, were found conjointly in this case. This is compatible with its origin from severe right ventricular congestive failure. The rarity of peliosis, however, indicates that one or more as yet unknown factors might be operative in its development. RF 001 YANOFF M ARCH PATHOL 77 159 964 002 WAGNER E ARCH HEILKD 2 369 861 003 SCHOENLANK W VIRCHOWS ARCH PATHOL ANAT 222 358 916 004 ZAK FG AM J PATHOL 26 1 950 005 CAROLI J SEM HOP PARIS 40 1709 964 006 WEIR MR TEX REP BIOL MED 27 1105 969 007 ROSS RC PATHOL EUR 7 273 972 008 BURGER RA AM J CLIN PATHOL 22 569 962 009 GORDON BS AM J CLIN PATHOL 33 156 960 010 KINTZEN W CAN MED ASSOC J 83 860 960 011 MCGIVEN AR J PATHOL 101 283 970 012 BERNSTEIN MS N ENGL J MED 284 1135 971 013 NAEIM F ARCH PATHOL 95 284 973 014 DELAGE C UNION MED CAN 102 1888 973 015 BAGHERI SA ANN INTERN MED 81 610 974 016 TRITES AEW ARCH PATHOL 63 183 957 017 SEAWRIGHT AA AUST VET J 47 91 971 018 WOLSTENHOLME JT PROC SOC EXP BIOL MED 74 659 950 019 BAGHERI SA GASTROENTEROLOGY 64 879 973 020 RUEBNER BH AM J PATHOL 60 247 970 021 TUCHWEBER B J MED (BASEL) 4 327 973 022 BERGS VV SCIENCE 158 377 967 023 ORANDI M CAN MED ASSOC J 96 1219 967 CT 1 PARADINAS FJ HISTOPATHOLOGY 1 225 977 2 ODIEVRE M ARCH FR PEDIATR 34 654 977 3 TAXY JB HUM PATHOL 9 331 978 4 BANK JI ACTA PAEDIATR SCAND 67 105 978 5 LUDWIG J DIG DIS SCI 24 785 979 6 KARASAWA T ACTA PATHOL JAP 29 457 979 7 WILLEN H VIRCHOWS ARCH PATHOL ANAT HIS 383 233 979 8 KAWAMOTO S ARCH PATHOL LAB MED 104 444 980 9 RYAN AJ FED PROC 40 2682 981 10 BROCHARD P ANN MED INTERNE (PARIS) 132 195 981 11 ITO T ENDOSCOPY 14 14 982 12 TUCKER RA DRUG INTEL CLIN PHARM 16 569 982 13 SPECH HJ Z GASTROENTEROL 20 710 982 14 CRAGG A PEDIATR RADIOL 14 340 984 15 WAKABAYASHI T ACTA PATHOL JAP 34 1079 984 16 GORDON SC J HEPATOL 2 475 986 PN 76031 RN 00386 AN 76135028 AU Changus-J-E. Pitot-H-C. TI Cystic fibrosis. A dilemma in the metabolic pathogenesis of genetic disease. SO Arch-Pathol-Lab-Med. 1976 Jan. 100(1). P 7-11. (REVIEW). MJ CYSTIC-FIBROSIS: me. MN ANIMAL. CELLS-CULTURED. CYSTIC-FIBROSIS: pa, fg. CYTOPLASM: ul. FIBROBLASTS: ul. HUMAN. IGG: an. INFANT-NEWBORN. LEUKOCYTES: ul. MUCINS: me. MUCOPOLYSACCHARIDES: me. PROTEIN-BINDING. RABBITS. REVIEW. SALIVA: an. SALIVARY-GLANDS: ul. SWEAT: an. AB The fundamental pathological observations in cystic fibrosis (CF) were made over three decades ago. More recently, physiological findings have directed investigation toward a plethora of humoral substances and their possible role in disturbances of the secretory processes in CF. The application of the techniques of molecular biology and of tissue culture is beginning to increase our knowledge about the so-called CF factors and their diverse effects on epithelial and ciliated surfaces. Emphasis in current research is on the chemical characterization of the CF factors, the metabolism of cells cultured from patients with CF, and the biochemical events controlling the processes of secretion. Some current theories as to the metabolic pathogenesis of CF are considered in the context of existing findings. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 FARBER S ARCH PATHOL 37 238 944 004 FARBER S J MICH MED SOC 44 587 945 005 SWENEY LR ARCH PATHOL 86 413 968 007 LEV R AM J PATHOL 46 23 965 008 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 009 MENGUY R GASTROENTEROLOGY 59 257 970 010 DOGGETT RG J CLIN PATHOL 24 270 971 011 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 012 GUGLER EC J PEDIATR 71 585 967 013 GUGLER EC J PEDIATR 73 548 968 014 DISCHE Z ANN NY ACAD SCI 93 526 962 015 DI SANTAGNESE PA PEDIATRICS 19 252 957 016 BENKE PJ PROC SOC EXP BIOL MED 137 1283 971 017 CHERNICK WS MOD PROBL PEDIATR 10 125 967 018 CHERNICK WS J PEDIATR 59 890 961 019 CHERNICK WS J PEDIATR 65 694 964 020 DI SANTAGNESE PA PEDIATRICS 12 549 953 021 MANGOS JA PEDIATR RES 1 436 967 022 MANGOS JA PEDIATR RES 2 378 968 023 BENKE PJ LANCET 1 182 972 024 TAYLOR A PEDIATR RES 8 861 974 025 RAO GJS J PEDIATR 80 573 972 026 SPOCK A PEDIATR RES 1 173 967 027 BESLEY GTN J MED GENET 6 278 969 028 BOWMAN BH SCIENCE 164 325 969 029 SPOCK A GAP CONF REP CILIARY INHIBITO 973 030 BOAT TF AM REV RESPIR DIS 110 428 974 031 BALFE JW SCIENCE 162 689 968 032 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 033 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 034 FITZPATRICK DF NATURE NEW BIOL 235 173 972 035 FEIG SA PEDIATR RES 8 594 974 036 MORIN CL BIOMEDICINE EXPRESS 19 133 973 037 TAUSSIG LM LANCET 1 1367 972 038 BROWN GA LANCET 2 639 971 039 WILSON GB PEDIATR RES 9 635 975 040 NOUSIA-ARVANITAKIS S CF CLUB ABST 14 43 973 041 BOWMAN BH IN: MANGOS JA 29 973 042 CONOVER JH LANCET 1 1194 973 043 WOOD RE LANCET 2 1452 973 044 CONOVER JH LANCET 2 1501 973 045 DANES BS LANCET 1 1061 968 046 DANES BS J EXP MED 129 775 969 047 LIE SO PEDIATR RES 7 13 973 048 LIE SO PROC NAT ACAD SCI USA 69 2361 972 049 DANES BS J EXP MED 136 1313 972 050 BERATIS NG PEDIATR RES 7 958 973 051 DANES BS AM J HUM GENET 25 323 973 052 DANES BS J EXP MED 137 1538 973 053 BARNETT DR TEX REP BIOL MED 31 703 973 054 CROW JF IN: NEEL JV 23 965 055 DANES BS LANCET 2 765 973 056 RENNERT OM LANCET 2 1091 973 057 BARTMAN J J PEDIATR 76 430 970 058 MATALON R LANCET 2 838 969 059 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 060 WIESMANN UN J PEDIATR 77 685 970 061 KRAUS I PEDIATRICS 47 1010 971 062 BENKE PJ N ENGL J MED 284 731 971 063 ROBERTSON JA LANCET 1 1256 974 064 DANES BS BIOCHEM GENET 12 359 974 065 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 066 QUISSELL DO NATURE 247 115 974 068 FLETCHER DS CLIN CHIM ACTA 44 5 973 069 BAIG MM J PEDIATR 86 72 975 070 RENNERT OM IN: MANGOS JA 41 973 071 COHEN LF PEDIATR RES 9 312 975 072 HANN S LANCET 2 520 974 073 LIEBERMAN J LANCET 1 1230 974 074 RAO GJS SCIENCE 177 610 972 075 LIEBERMAN J AM REV RESPIR DIS 109 399 974 076 COBURN MD AM REV RESPIR DIS 110 368 974 077 RAO GJS PEDIATR RES 8 684 974 078 LOUISOT P CLIN CHIM ACTA 48 373 973 079 SINGER L CLIN BIOCHEM 7 146 974 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 CALLAHAN JW PEDIATR RES 11 1166 977 3 SULLIVAN JL BIOCHEM GENET 15 1125 977 4 NEUTRA MR GASTROENTEROLOGY 75 701 978 5 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 6 GABRIDGE MG PEDIATR RES 13 31 979 7 PEARSON RD PEDIATR RES 13 834 979 8 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 9 LOPEZCORELLA E PATOLOGIA (MEXICO CITY) 18 167 980 10 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 PN 76032 RN 00387 AN 77133875 AU Phelan-P-D. Steiner-N. TI Assessing obstructive airways disease in children. Which test is best?. SO Aust-Paediatr-J. 1976 Jun. 12(2). P 72-6. MJ ASTHMA: di. CYSTIC-FIBROSIS: di. RESPIRATORY-AIRFLOW. MN ADOLESCENCE. CHILD. COMPARATIVE-STUDY. FORCED-EXPIRATORY-FLOW-RATES. HUMAN. AB Tests for airways obstruction based on a forced expiration were carried out in 60 children with asthma and 15 with cystic fibrosis to determine which test or tests were the best in identifying children with airways disease, to determine the severity of disease and to document response to therapy. Maximum expiratory flow, measured from a maximum expiratory flow volume curve, and maximum mid-expiratory flow measured from a spirogram, were the most sensitive tests in detecting early disease. Maximum expiratory flow measured from the maximum expiratory flow volume curve, combined with measurement of lung volumes, gave the best overall estimate of severity. Peak expiratory flow rate measured with a Peak Flow Meter, because of its simplicity of measurement, was the most useful test for routine use in the clinic and consulting room to monitor severity and response to therapy. RF 001 CONNOLLY NM J ASTHMA RES 8 31 970 002 FEATHERBY EA AM REV RESPIR DIS 102 737 970 003 HILL DJ ARCH DIS CHILD 47 874 972 004 LANDAU LI AM REV RESPIR DIS 108 593 973 005 LANDAU LI AUST NZ J MED 3 557 973 006 MEAD J J APPL PHYSIOL 22 95 967 007 POLGAR G PULMONARY FUNCTION TESTING IN 971 009 WENG TR AM REV RESPIR DIS 99 719 969 010 WOOLCOCK AJ LANCET 2 1323 965 011 ZAPLETAL A J APPL PHYSIOL 26 308 969 CT 1 MITCHELL EA AUST PAEDIATR J 18 40 982 2 CASTELLO D RIV ITAL PEDIATR 11 479 985 PN 76033 RN 00388 AN 77133876 AU Mellis-C-M. Landau-L-I. TI Ventilatory disturbance in children with obstructive lung disease. SO Aust-Paediatr-J. 1976 Jun. 12(2). P 77-82. MJ ASTHMA: pp. CYSTIC-FIBROSIS: pp. LUNG: pp. MN CHILD. HUMAN. RESPIRATORY-FUNCTION-TESTS. AB Disease in the small airways leads to alteration in the slope of phase III of the single breath nitrogen washout following a breath of 100% oxygen. Normal children and those with asthma and cystic fibrosis were studied by this technique. Measurement of the slope of phase III of the single breath washout appeared to be a sensitive indicator of abnormal pulmonary function in children with these diseases. Results obtained in many children with cystic fibrosis suggested that the upper lung regions were predominantly affected. RF 001 ALDERSON PO RADIOLOGY 111 151 974 002 ANTHONISEN NR CLIN SCI 35 495 968 003 COOPER DM AM REV RESPIR DIS 109 519 974 004 DOLLFUSS RE RESPIR PHYSIOL 2 234 967 005 FOWLER WS J APPL PHYSIOL 2 283 949 006 MCFADDEN ER JR J APPL PHYSIOL 25 365 968 CT 1 LANDAU LI THORAX 34 217 979 PN 76034 RN 00389 AN 76277421 AU Harrison-S-P. TI Compliance or non-compliance with treatment?. SO Australas-Nurses-J. 1976 May. 4(11). P 37. MJ CYSTIC-FIBROSIS: th. PATIENT-COMPLIANCE. MN CHILD. HUMAN. PUBLIC-HEALTH-NURSING. EX In a situation in which a patient, currently undergoing medical treatment at home requiring compliance and fairly rigid discipline for its effectiveness, is found by the health visitor not to be carrying out the prescribed treatment, should the health visitor's role always be to attempt to influence the patient to comply with the required treatment or are there occasions when it might be acceptable for her to disregard this positive role? To illustrate such a situation, the real-life experience of two families will be discussed. RF 001 NELSON WE TEXTBOOK OF PEDIATRICS 854 969 002 HARRISSON SP THESIS 975 003 THOMAS EJ J HLTH HUM BEHAV 7 2 966 PN 76035 RN 00390 AN 77065277 AU Hosli-P. Erickson-R-P. Vogt-E. TI Prospects for prenatal diagnosis of cystic fibrosis: induction of biochemical abnormalities in fibroblasts from patients with cystic fibrosis by a urinary glycoprotein. SO Biochem-Biophys-Res-Commun. 1976 Nov 22. 73(2). P 209-16. MJ ALKALINE-PHOSPHATASE: me. CYSTIC-FIBROSIS: di. GLYCOPROTEINS: ur. MN CYSTIC-FIBROSIS: me. ENZYME-INDUCTION: de. FEMALE. FIBROBLASTS: en. GLUCOSIDASES: me. GLYCOPROTEINS: du, pd. HETEROZYGOTE. HUMAN. MALE. PREGNANCY. PRENATAL-DIAGNOSIS. AB Alkaline phosphatase activity, assayed on a per cell basis with an ultra micro-method, can be increased up to 7-fold in fibroblast cultures derived from patients with cystic fibrosis by induction with a urinary glycoprotein. Fibroblasts from normal and heterozygous individuals are not significantly induced. There is a suggestion of heterogeneity among cases as reflected in varying baseline alkaline phosphatase and alpha-glucosidase levels. RF 001 DANES BS J EXP MED 129 775 969 002 HOSLI P PRENATAL DIAGNOSIS NEWSLETTER 1 10 972 003 HOSLI P PURINE METABOLISM IN MAN 811 974 004 HOSLI P TECNOMARA 972 005 HOSLI P ADV EXP MED BIOL 68 1 976 006 MAXFIELD M J CLIN INVEST 41 455 962 007 STEVENSON FK CLIN CHIM ACTA 23 441 969 008 FINNIGAN JA BIOCHIM BIOPHYS ACTA 236 52 971 009 CROSBY P HISTOCHEM J 3 223 971 010 TAMM I J EXP MED 95 71 952 011 MAXFIELD M GLYCOPROTEINS 446 964 012 HOSLI P IN: MOTULSKY AG 226 974 013 ANTONOWICZ I PEDIATR RES 6 803 972 CT 1 HOSLI P CLIN CHEM 23 1476 977 2 HOSLI P HUM GENET 37 195 977 3 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 4 OWEN E J MOL MED 3 203 978 5 UITENDAAL MP HUM HERED 28 151 978 6 ERICKSON RP AM J HUM GENET 30 A 28 978 7 HOSLI P HUM GENET 41 169 978 8 BRADY RO ANNU REV BIOCHEM 47 687 978 9 HOSLI P ACTA PAEDIATR SCAND 67 617 978 10 UITENDAAL MP ANAL BIOCHEM 84 147 978 11 VANNEUVILLE FJ ARCH INT PHYSIOL BIOCHIM 86 817 978 12 SHWACHMAN H AM J DIS CHILD 132 1112 978 13 CAREY WF MED J AUST 2 604 979 14 HOSLI P LANCET 2 543 979 15 CASOLA L MONOGR PAEDIATR 10 96 979 16 DIMATTEO G MONOGR PAEDIATR 10 19 979 17 HOSLI P MONOGR PAEDIATR 10 90 979 18 KAISER E CLIN BIOCHEM 12 208 979 19 HOSLI P CLIN GENET 15 487 979 20 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 21 CASOLA L CLIN CHIM ACTA 94 83 979 22 JAKEL HP BIOL ZENTRALBL 98 55 979 23 HOSLI P FEBS LETTERS 104 271 979 24 WIJCIK L BIOCHIM BIOPHYS ACTA 585 374 979 25 DAVIS PB PEDIATR RES 14 83 980 26 AITKEN DA J MED GENET 17 187 980 27 FASTH A ACTA PAEDIATR SCAND 69 189 980 28 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 29 HARRIS A CLIN GENET 20 315 981 30 JAKEL HP BIOL ZENTRALBL 100 273 981 31 HOSLI P EUR J PEDIATR 137 116 981 32 DODGE JA ARCH DIS CHILD 57 774 982 33 CEDER O SCANN ELECTRON MICROSC 1982 723 982 34 JAKEL HP BIOMED BIOCHIM ACTA 42 1123 983 35 BUYS CHCM CLIN CHIM ACTA 136 229 984 36 ANON LANCET 1 1199 985 37 PORTER WH CLIN CHEM 32 652 986 38 BOUE A HUM GENET 74 288 986 PN 76036 RN 00391 AN 76278044 AU Shapira-E. Ben-Yoseph-Y. Nadler-H-L. TI Decreased formation of alpha 2-macroglobulin-protease complexes in plasma of patients with cystic fibrosis. SO Biochem-Biophys-Res-Commun. 1976 Aug 9. 71(3). P 864-70. MJ ALPHA-MACROGLOBULINS: me. CYSTIC-FIBROSIS: me. PEPTIDE-HYDROLASES: bl. MN CYSTIC-FIBROSIS: en, fg. GEL-DIFFUSION-TESTS. HETEROZYGOTE. HUMAN. KINETICS. MACROMOLECULAR-SYSTEMS. PAPAIN. PRECIPITIN-TESTS. PROTEIN-BINDING. TRYPSIN. SUPPORT-U-S-GOVT-P-H-S. AB Alpha2-macroglobulin from patients with cystic fibrosis is shown to have reduced binding with papain, trypsin, and thrombin. The obligate heterozygotes for cystic fibrosis revealed intermediate values between the controls and the patients. Since papain and trypsin are not plasma endopeptidases, it becomes evident that the absence of alpha2-macroglobulin-protease complex in cystic fibrosis is due to a molecular defect within the macroglobulin. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 RAO GJS J PEDIATR 80 573 972 003 RAO GJS PEDIATR RES 8 684 974 004 RAO GJS PEDIATR RES 9 739 975 005 WILSON GB PEDIATR RES 10 87 976 007 BARRETT AJ BIOCHEM J 133 709 973 008 MANCINI G IMMUNOCHEMISTRY 2 235 965 009 HUNTER WM NATURE 194 495 962 010 TRAVIS J J BIOL CHEM 240 1962 965 011 GLASER AN J BIOL CHEM 236 2948 961 012 WINZOR DJ ARCH BIOCHEM BIOPHYS 104 202 964 013 LAURELL CB IN: PUTNAM FW 246 975 CT 1 RAO GJS PEDIATR RES 11 981 977 2 SHAPIRA E CLIN CHIM ACTA 78 359 977 3 SHAPIRA E J BIOL CHEM 252 7923 977 4 WILSON GB PEDIATR RES 12 801 978 5 ALHADEFF JA CLIN GENET 14 189 978 6 CHOY H BIOCHEM BIOPHYS RES COMMUN 82 1325 978 7 GUY GJ CLIN CHIM ACTA 87 63 978 8 VIRCA GD ANAL BIOCHEM 89 274 978 9 ROMEO G NATURE 274 909 978 10 APPLEGARTH DA LANCET 2 1236 979 11 ROMEO G PEDIATR RES 13 1030 979 12 WILSON GB PEDIATR RES 13 1079 979 13 JAKEL HP BIOL ZENTRALBL 98 55 979 14 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 15 COMINGS DE AM J HUM GENET 32 273 980 16 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 17 ROMEO G J LAB CLIN MED 95 116 980 18 BURDON MG CLIN CHIM ACTA 100 225 980 19 PARSONS M CLIN CHIM ACTA 100 215 980 20 SCHIDLOW DV AM REV RESPIR DIS 121 31 980 21 BENYOSEPH Y PEDIATR RES 15 839 981 22 BLITZER MG J PEDIATR GASTROENTEROL NUTR 1 289 982 23 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 24 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 25 BURY AF IRCS MED SCI BIOCHEM 10 255 982 26 BLITZER MG PEDIATR RES 16 203 982 27 ROBERTS RC PEDIATR RES 16 416 982 28 BRIDGES MA CLIN CHIM ACTA 118 33 982 29 TUMMLER B CLIN CHIM ACTA 125 219 982 30 BUCHANAN KD SCAND J GASTROENTEROL 18 155 983 31 BRIDGES MA ANN NY ACAD SCI 421 360 983 32 SHAPIRA E ANN NY ACAD SCI 421 352 983 33 HJELM M CLIN BIOCHEM 17 284 984 34 EAGER KB PEDIATR RES 18 999 984 35 ANON LANCET 2 249 985 PN 76037 RN 00392 AN 77022473 AU Bowman-B-H. Barnett-D-R. TI Recent advances in cystic fibrosis research. SO Birth-Defects. 1976. 12(6). P 197-212. (REVIEW). MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. AGGLUTINATION. BIOLOGICAL-TRANSPORT. CHILD. CHILD-PRESCHOOL. CHLORIDES: me. CILIA: de. COMPLEMENT-3: me. COMPLEMENT: me. CYSTIC-FIBROSIS: bl, di, me, oc, pa. DEPRESSION-CHEMICAL. HUMAN. INFANT. INFANT-NEWBORN. MUCUS: me. PRENATAL-DIAGNOSIS. RESEARCH. REVIEW. SODIUM: me. STAINS-AND-STAINING. SWEAT: me. SUPPORT-U-S-GOVT-P-H-S. EX Cystic fibrosis at one time was considered to be a lethal condition with death occurring at birth or within the first year of life. With the advent of antibiotic therapy, pancreatic enzyme replacement, extensive physical therapy and generally improved medical treatment, many patients now survive to adulthood. Clinical manifestations in CF vary from patient to patient and may include pulmonary disease, malnutrition, pancreatic insufficiency and defective ion transport. The most reliable laboratory method for diagnosis of CF is testing for elevated sweat electrolyte levels. Estimates of the incidence of CF vary from 1 in 2000 to 1 in 3500 among white populations of mid-European heritage. The disease is rare in black populations and almost non-existent in Orientals. Since 1967 there has been an intensive search for a circulating factor or factors which may be directly or indirectly responsible for the malfunctions manifest in this disease. Sweat factors, serum factors, metachromasia, bioassay variations, and properties of the ciliary inhibitor are discussed. Several groups working in CF research have centered their attention on the serum complement system. Agglutination and mucus factors, transport studies, enzyme studies, and prenatal diagnosis are also examined. RF 001 FANCONI G WIEN MED WOCHENSCHR 86 753 936 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 004 WARWICK WJ J ASTHMA RES 5 277 968 005 BRUSILOW SW ANNU REV MED 21 99 970 006 DOERSHUK CF GAP CONF REP DATA REG CONF 971 008 MCCOMBS ML TEX REP BIOL MED 31 615 973 009 DENNING CR PEDIATRICS 41 7 968 010 KAPLAN E N ENGL J MED 279 65 968 012 BLOMFIELD J ARCH ORAL BIOL 19 1153 974 013 KUO PT J PEDIATR 60 394 962 014 ROSENLUND ML NATURE 251 719 974 015 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 016 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 017 STEPHAN U PEDIATRICS 55 35 975 018 BRUNECKY Z J MED GENET 9 33 972 019 MERRITT AD J LAB CLIN MED 60 998 962 020 STEINBERG AG AM J HUM GENET 12 416 960 021 KULCZYCKI LL CLIN PEDIATR 3 692 964 022 OPPENHEIMER EH PEDIATRICS 42 547 968 023 FEINGOLD J ANN GENET (PARIS) 17 257 974 024 DANKS DM ANN HUM GENET 28 323 965 025 WRIGHT SW AM J HUM GENET 20 157 968 026 KNUDSON AG JR AM J HUM GENET 19 388 967 027 STUART AB LANCET 2 1521 974 028 COHEN FL J MED GENET 11 253 974 029 MANGOS JA SCIENCE 158 135 967 030 MANGOS JA PEDIATR RES 1 436 967 031 MANGOS JA TEX REP BIOL MED 31 651 973 032 SPOCK A PEDIATR RES 1 173 967 033 BOWMAN BH SCIENCE 164 325 969 034 BESLEY GTN J MED GENET 6 278 969 035 CONOVER JH PEDIATR RES 7 220 973 036 CONOVER JH PEDIATR RES 7 224 973 037 DANES BS J EXP MED 129 775 969 038 CONOVER JH LANCET 1 1194 973 040 CONOVER JH LANCET 2 1501 973 041 BERATIS NG PEDIATR RES 7 958 973 042 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 043 BARNETT DR TEX REP BIOL MED 31 703 973 044 DANES BS J EXP MED 137 1538 973 045 BARNETT DR TEX REP BIOL MED 31 697 973 046 BOWMAN BH SCIENCE 167 871 970 047 DOGGETT RG NATURE NEW BIOL 243 251 973 048 DOGGETT RG TEX REP BIOL MED 31 685 973 049 DANES BS J EXP MED 136 1313 972 050 BOWMAN BH CLIN GENET 4 461 973 051 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 052 CONOVER JH LIFE SCI 14 253 974 053 LIEBERMAN J AM REV RESPIR DIS 111 100 975 054 HANN S LANCET 2 520 974 055 POLLEY MJ J MED GENET 11 249 974 056 BARNETT DR PEDIATR RES 8 687 974 057 WILSON GB LIFE SCI 15 551 974 058 WILSON GB CLIN CHIM ACTA 49 79 973 059 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 060 BOWMAN BH FED PROC 31 842 972 061 KHAN MA TEX REP BIOL MED 31 665 973 062 BALFE JW SCIENCE 162 689 968 063 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 064 LAPEY A PEDIATR RES 5 446 971 065 COLE CH PEDIATR RES 6 616 972 066 FEIG SA PEDIATR RES 8 594 974 067 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 068 MCEVOY FA CLIN CHIM ACTA 54 195 974 069$ QUISSELL DO ANN NY ACAD SCI 203 237 972 070 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 071 BAGLIA FA CF CLUB ABST 23 975 072 BROWN GA LANCET 2 639 971 073 BENKE PJ LANCET 1 182 972 074 FLETCHER DS CLIN CHIM ACTA 44 5 973 075 LOUISOT P CLIN CHIM ACTA 48 373 973 076 RAO GJS J PEDIATR 80 573 972 077 RAO GJS PEDIATR RES 8 684 974 078 LIEBERMAN J AM REV RESPIR DIS 109 399 974 079 RENNERT OM CLIN PEDIATR 11 351 972 080 KLAGSBRUN M IN: MANGOS JA 53 973 081 RENNERT OM IN: MANGOS JA 41 973 082 NADLER HL LANCET 2 84 969 083 NEUFELD EF REPORT ON STAINING FOR METACH 972 084 JACOBSON CB CF CLUB ABST 23 974 085 BOLTON WE AM J HUM GENET 27 394 975 086 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 CT 1 KOLLBERG H ACTA PAEDIATR SCAND 68 639 979 2 ANON CAN MED ASSOC J 121 1193 979 PN 76038 RN 00393 AN 76254571 AU Cohen-L-F. Lundgren-D-W. Farrell-P-M. TI Distribution of spermidine and spermine in blood from cystic fibrosis patients and control subjects. SO Blood. 1976 Sep. 48(3). P 469-75. MJ CYSTIC-FIBROSIS: bl. SPERMIDINE: bl. SPERMINE: bl. MN CELL-FRACTIONATION. CELL-SEPARATION. CITRATES: bl. COMPARATIVE-STUDY. HEPARIN: bl. HUMAN. MALE. POLYAMINES: bl. AB Previous studies have shown an abnormality of the spermidine-to- spermine (Spd/Spm) ratio in whole blood of cystic fibrosis homo-and heterozygotes. To investigate Spd and Spm distribution amoung blood components as a possible cause of the abnormality, blood was fractionated using Rabinowitz's glass bead technique and Boyum's Ficoll-Hypaque method. Free (unconjugated) polyamines were extracted with perchloric acid and quantitated on an amino acid analyzer. In controls, mean +/- SEM concentrations in nmoles/10(9) cells of Spd and Spm, respectively, were 1.02 +/- 0.08 and 0.894 +/- 0.28 for erythrocytes; 126 +/- 31 and 357 +/- 105 for lymphocytes; 36 +/- 16 and 240 +/- 33 for granulocytes; and less than 0.5 and less than 0.5 nmoles/ml for plasma. When converted to the concentration in whole blood, it was found that greater than 90% of Spd and over 70% of Spm was associated with erythrocytes. While the higher cellular concentration in leukocytes was not unexpected, the fact that Spd and Spm in whole blood were primarily associated with erythrocytes was a new finding. Comparison with controls revealed that the Spd/Spm ratio in both whole blood and erythrocytes was significantly higher in the group of cystic fibrosis patients. RF 001 TABOR H ADV ENZYMOL 36 203 972 002 COHEN SS INTRODUCTION TO POLYAMINES 971 003 RUSSELL DH IN: RUSSELL DH 1 973 004 SCHIMPFF SC IN: RUSSELL DH 395 973 005 RENNERT OM IN: MANGOS JA 41 973 006 LUNDGREN DW CLIN CHIM ACTA 62 357 975 007 TAUSSIG LM J PEDIATR 82 380 973 008 RABINOWITZ Y BLOOD 23 811 964 009 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 010 ROSENTHAL SM J PHARMACOL EXP THER 116 131 956 011 TABOR H PHARMACOL REV 16 245 964 012 GRAZIANO KD BIOMEDICINE 21 197 974 013 DESSER H CLIN CHIM ACTA 63 243 975 CT 1 RUSSELL DH CLIN CHEM 23 22 977 2 INOUE H SEIKAGAKU 49 411 977 3 LUNDGREN DW J PEDIATR 90 1034 977 4 ALHADEFF JA CLIN GENET 14 189 978 5 DUNZENDORFER U CANCER RES 38 2321 978 6 SAEKI Y J CHROMATOGR 145 221 978 7 ROSENBLUM MG SCIENCE 200 1496 978 8 LUNDGREN DW AM J PHYSIOL 234 E451 978 9 SAVORY J LANCET 2 1136 979 10 TAKAMI H LANCET 2 912 979 11 RUSSELL DH PEDIATR RES 13 1137 979 12 WILSON GB J LAB CLIN MED 93 800 979 13 RATTENBURY JM CLIN CHIM ACTA 95 61 979 14 JAKEL HP BIOL ZENTRALBL 98 55 979 15 MILANO G ONCODEVELOP BIOL MED 1 215 980 16 SHIPE JR ANN CLIN LAB SCI 10 128 980 17 BAYLIN SB PEDIATR RES 14 921 980 18 GAHL WA PEDIATR RES 14 118 980 19 LEISTI S PEDIATR RES 14 339 980 20 SCHAEFER GB PEDIATR RES 14 580 980 21 SWENDSEID ME LIFE SCI 26 533 980 22 THEOHARIDES TC LIFE SCI 27 703 980 23 DUNZENDORFER U ARZNEIMITTEL FORSCH DRUG RES 30-2 1801 980 24 LORIA D MED (BUENOS AIRES) 40 607 980 25 TAKAMI H BR J CANCER 41 751 980 26 UEHARA N CANCER 45 108 980 27 UEHARA N GANN 71 393 980 28 SHIPE JR CRC CRIT REV CLIN LAB SCI 16 1 981 29 MOULINOUX JP LIFE SCI 29 955 981 30 RUSSELL DH NEUROLOGY 31 80 981 31 CHENG PW J BIOL CHEM 257 6251 982 32 RUSSELL DH CRC CRIT REV CLIN LAB SCI 18 261 983 33 TOKUNAGA A HIROSHIMA J MED SCI 32 127 983 34 TOKUNAGA A HIROSHIMA J MED SCI 32 113 983 35 EKSTROM J ACTA PHYSIOL SCAND 119 287 983 36 FUJIHARA S J CHROMATOGR 277 53 983 37 CHAYEN R CELL BIOCHEM FUNCT 2 15 984 38 SHULL GE J THEOR BIOL 110 461 984 39 VORONCHIKHINA LD LAB DELO 443 985 40 RAM BP CRC CRIT REV BIOCHEM 17 257 985 41 MOULINOUX JP CLIN CHIM ACTA 145 77 985 42 VORONCHIKHINA LD VOPR MED KHIM 32 43 986 43 OTA DM INT J CANCER 38 245 986 44 TADOLINI B BIOCHEM BIOPHYS RES COMMUN 134 1365 986 45 MOULINOUX JP EUR J CANCER CLIN ONCOL 23 237 987 46 ISOBE K BIOCHEM MED METAB BIOL 37 110 987 PN 76039 RN 00394 AN 76115480 AU Gibbons-A. Allan-J-D. Holzel-A. McFarlane-H. TI Cell-mediated immunity in patients with cystic fibrosis. SO Br-Med-J. 1976 Jan 17. 1(6002). P 120-2. MJ CYSTIC-FIBROSIS: im. IMMUNITY-CELLULAR. MN ADOLESCENCE. ADULT. ANTIGENS. ANTIGENS-FUNGAL. ASPERGILLUS-FUMIGATUS: im. CELL-MIGRATION-INHIBITION. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt. FEMALE. GUINEA-PIGS. HUMAN. INFANT. LEUKOCYTES: im. LUNG: im. MACROPHAGES: im. MALE. PANCREAS: im. PREDNISOLONE: tu. PSEUDOMONAS-AERUGINOSA: im. AB Leucocytes from 26 patients with cystic fibrosis (CF) and 18 healthy controls were investigated by migration inhibition induced by a variety of antigens. In patients with CF cell-mediated immunity was found to human lung and pancreatic tissue extracts as well as to Aspergillus fumigatus, Pseudomonas aeruginosa, and food antigens but not to brain, heart, or kidney. Those patients with the severest form of the disease had the greatest impairment of cell-mediated immunity, but this impairment could be reversed by steroid treatment. Cell- mediated cytotoxicity may also be concerned in the pathogenesis of CF. RF 001 MURRAY MJ ANN INTERN MED 53 548 960 002 STEIN AA J PEDIATR 65 495 964 003 MEARNS MB THORAX 20 385 965 004 MEARNS MB LANCET 1 538 967 005 WARREN CPW CLIN ALLERGY 5 1 975 006 MCFARLANE H BR MED J 1 423 975 007 SOBORG M ACTA MED SCAND 181 247 967 008 MORLEY J IN: WEIR DM 2 20 973 009 PEPYS J PROC INT CONG ALLERGOLOGY 6 221 968 010 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 011 CRACCO G PROC EWGCF 5TH ANNU MTG 16 974 012 GELL PGH CLINICAL ASPECTS OF IMMUNOLOG 968 013 MCFARLANE H CF NEWS 10 6 975 CT 1 FEIGHERY C BR MED J 1 524 976 2 MCFARLANE H LANCET 1 1241 976 3 ANON NUTR REV 34 210 976 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 5 RAEBURN JA PROC NUTR SOC 36 77 977 6 GORMAN NT J NATL CANCER INST 61 1095 978 7 GOTZ M EUR J PEDIATR 127 133 978 8 HOIBY N ACTA PAEDIATR SCAND 68 495 979 9 HODSON ME THORAX 35 801 980 10 MIELIVERGANI G ARCH DIS CHILD 55 696 980 11 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 31 199 983 12 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 31 217 983 13 PORWOLL JM INFECT IMMUN 40 670 983 14 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 15 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 PN 76040 RN 00395 AN 76254863 AU Williams-A-J. McKiernan-J. Harris-F. TI Letter: Heat prostration in children with cystic fibrosis. SO Br-Med-J. 1976 Jul 31. 2(6030). P 297. MJ CYSTIC-FIBROSIS: co. HEAT-EXHAUSTION: co. MN CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. HYPONATREMIA: co. MALE. EX We wish to report three cases of hyponatraemic dehydration in children with cystic fibrosis admitted to this hospital in late June and early July this year. These three children with cystic fibrosis clearly illustrate the danger of the present unusual climatic conditions. The necessity of salt supplementation in these environmental circumstances should be emphasised to all concerned and, above all, to parents. The danger of the administration of salt-free glucose solutions also should be borne in mind. In the past the case has been argued for a safe electrolyte solution that would be commercially available. In the absence of such a readily available form of electrolyte-glucose solution parents will need explicit instructions on how the salt supplement should be administered. RF 001 ANON BR MED J 1 125 971 PN 76041 RN 00396 AN 77023000 AU Hodson-M-E. Mearns-M-B. Batten-J-C. TI Meconium ileus equivalent in adults with cystic fibrosis of pancreas: a report of six cases. SO Br-Med-J. 1976 Oct 2. 2(6039). P 790-1. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: et. MN ACETYLCYSTEINE: tu. ADOLESCENCE. ADULT. CASE-REPORT. COLON: ra. DIABETES-MELLITUS: co. ENEMA. FEMALE. HUMAN. INTESTINAL-OBSTRUCTION: dt, su. INTESTINE-SMALL: ra, su. MALE. AB Eleven episodes of "meconium ileus equivalent" have been seen in six adults with cystic fibrosis of the pancreas. Three patients were initially treated surgically; one died and the other two developed serious postoperative chest infections. Six episodes were successfully treated medically with acetylcysteine orally and by enema, nasogastric suction, and intravenous fluids. Operation should be avoided if possible, and maintenance treatment with acetylcysteine may be necessary to prevent relapse. RF 001 DONNISON AB PEDIATRICS 37 833 966 002 LEVY E ARCH DIS CHILD 26 335 951 003 FISHER OD ARCH DIS CHILD 29 262 954 004 FANCONI A HELV PAEDIATR ACTA 15 566 960 005 HUNTON DB GASTROENTEROLOGY 50 99 966 006 KOPEL FB GASTROENTEROLOGY 62 483 972 007 DI SANTAGNESE PA PEDIATRICS 19 252 957 008 JOHANSEN PG BIOCHEM J 87 63 963 009 THOMAIDIS TS J PEDIATR 63 444 963 010 HALLBERG D ACTA CHIR SCAND 128 201 964 011 SNYDER WH JR PEDIATRICS 34 72 964 012 GARNHAM JR GUT 5 256 964 013 MULLINS F JAMA 192 741 965 014 LILLIBRIDGE CB J PEDIATR 71 887 967 015 GRACEY M ARCH DIS CHILD 44 404 969 016 HANDWERGER S N ENGL J MED 281 451 969 CT 1 PRESCOTT LF LANCET 2 432 977 2 ANGERPOINTNER T Z KINDERCHIR GRENZGEB 24 99 978 3 BENGHIAT A BR J SURG 65 215 978 4 HOWIE AD SCOTT MED J 24 193 979 5 SAUVEGRAIN J J RADIOL ELECTROL MED NUCL 60 3 979 6 SANTAGNESE PAD AM J MED 66 121 979 7 ROGERS LF RADIOLOGY 133 807 979 8 FERRARI V EUR J RESPIR DIS 61 151 980 9 REDMOND AOB PRACTITIONER 224 295 980 10 PILLING DW BR J RADIOL 54 562 981 11 PARK RW GASTROENTEROLOGY 81 1143 981 12 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 13 ZENTLERMUNRO PL BR J HOSP MED 28 653 982 14 ROSENSTEIN BJ J PEDIATR GASTROENTEROL NUTR 2 299 983 15 BRECHER DB AM FAM PHYSICIAN 28 187 983 16 AZMY AF Z KINDERCHIRURGIE 38 253 983 17 ROSENBERG E KLIN PAEDIATR 195 323 983 18 HANLY JG BR MED J 286 1411 983 19 MEARNS MB ARCH DIS CHILD 60 272 985 20 BERKIN KE EUR J RESPIR DIS 67 103 985 21 ISPIZUA AU REV CLIN ESP 176 417 985 22 CLEGHORN GJ LANCET 1 8 986 23 ZENTLERMUNRO P LANCET 1 328 986 24 KOHLER JA J CLIN HOSP PHARM 11 21 986 25 OHALLORAN SM ARCH DIS CHILD 61 1128 986 26 RUBINSTEIN S PEDIATRICS 78 473 986 27 WELLER PH J ROY SOC MED 79 36 986 28 BAILEY DJ PEDIATRICS 79 281 987 PN 76042 RN 00397 AN 76137278 TI Letter: Cell-mediated immunity in patients with cystic fibrosis. SO Br-Med-J. 1976 Feb 28. 1(6008). P 524. MJ IMMUNITY-CELLULAR. MN CYSTIC-FIBROSIS: dt. HUMAN. IMMUNITY-CELLULAR: de. PREDNISOLONE: pd. EX We agree with the comment of Dr Feighery and Mr Woods that a low migration index signifies sensitivity to an antigen which can be achieved only by functionally active immunocompetent cells. However, we must emphasise that the functions of active immunocompetent cells may or may not be impaired, depending upon the antigens to which such cells are sensitised. Since comparative cells from age-matched controls and from blood donors did not show such sensitisation to those antigens to which the cystic fibrosis cells were sensitised then there is no doubt that the normal function of these immunocompetent cells in the patients with CF is indeed impaired. When one considers that steroids are known immunosuppressants it is certainly not surprising that treatment of the CF patients with prednisolone caused a decreased sensitivity of these sensitised CF cells to the antigens. Indeed, therapeutic concentrations of steroids were able to reduce the ability of sensitised spleen cells and of lymph-node cells to destroy target cells in vitro. Apart from this inhibition of sensitised cells by steroids, 90% of sensitised lymphocytes were killed when prednisolone 1 mg/l was present during the sensitisation phase. In addition it was observed in man that if cortisol or prednisolone was given two hours before abrading the skin, then there occurred a marked reduction of emigrations of both mononuclear cells and polymorphs into the area, thus inhibiting the mobilisation of macrophages. We believe that the situation with regard to steroid therapy and inhibition of migration of leukocytes from CF patients is similar. RF 001 GIBBONS A BR MED J 1 120 976 002 ROSENAU W J IMMUNOL 89 422 962 003 COHEN IR J EXP MED 132 1055 970 004 BOGGS DR AM J PATHOL 44 763 964 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 PN 76043 RN 00398 AN 76207946 AU Cooperman-E-M. TI Cystic fibrosis: physiology gone wrong. SO Can-Med-Assoc-J. 1976 Jun 19. 114(12). P 1082-3. MJ CYSTIC-FIBROSIS: pp. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: pp. INTESTINAL-SECRETIONS: ph. INTUSSUSCEPTION: pp. EX Cystic fibrosis (CF), like syphilis, could accurately be described as "the great imitator." Many organ systems are involved, and a clear understanding of the disease and its many complications is a useful exercise in understanding human physiology. CF could be called thick-secretion disease, because all exocrine fluids, like pancreatic juice, are thick, or it could be called multimystery disease, because there are so many unanswered questions about it. Many manifestations of CF are understandable complications of malabsorption, malnutrition and the physical and chemical effects of abnormal exocrine secretion; others, including the susceptibility to recurrent nasal polyps, the hypersalinized sweat, low female fertility and male sterility, given present knowledge, do not make physiologic sense. RF 001 KAPLAN E N ENGL J MED 279 65 968 002 NOLAN AJ CAN MED ASSOC J 114 142 976 003 ANDERSEN DH AM J DIS CHILD 56 344 938 004 DARLING RC AM J MED SCI 225 67 953 005 SHWACHMAN H ANN NY ACAD SCI 93 600 962 006 GIBSON LE PEDIATRICS 23 545 959 007 KOPEL FB GASTROENTEROLOGY 62 483 972 008 BERK RN RADIOLOGY 106 377 973 009 DONNISON AB PEDIATRICS 37 833 966 010 THOMAIDIS TS J PEDIATR 63 444 963 011 SHWACHMAN H N ENGL J MED 286 1300 972 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 DI SANTAGNESE PA PEDIATRICS 18 387 956 014 FALLIS JC CAN MED ASSOC J 114 38 976 015 HOLSCLAW DS PEDIATRICS 48 51 971 016 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 PN 76044 RN 00399 AN 76163065 AU Webb-T. Harnden-D-G. TI The transformation by simian virus 40 of cells from patients with mucopolysaccharidosis and from normal controls. SO Cancer-Res. 1976 Feb. 36(2 Pt 1). P 298-301. MJ CELL-TRANSFORMATION-NEOPLASTIC. MUCOPOLYSACCHARIDOSIS. SV40-VIRUS. MN ADSORPTION. CELL-LINE. CELLS-CULTURED. COMPARATIVE-STUDY. CYSTIC-FIBROSIS. FIBROBLASTS. GANGLIOSIDOSIS. GAUCHERS-DISEASE. AB Fibroblasts derived from individuals with mucopolysaccharidosis, an inborn error of metabolism, have been found to be more easily transformed by simian virus 40 than are cells derived from normal individuals. The increased susceptibility does not seem to depend upon changes in glycoprotein at the cell surface. Repeated observations were necessary to demonstrate these differences, and we do not believe that this test is suitable for routine screening for cancer susceptibility. RF 001 AARONSON SA J VIROL 6 470 970 002 AARONSON SA NATURE 228 359 970 003 AARONSON SA VIROLOGY 36 254 968 004 DANES BS J EXP MED 123 1 966 005 KERSEY JH PROC NAT ACAD SCI USA 69 980 972 006 KOPROWSKI H J CELL COMP PHYSIOL 59 281 962 007 LEVIN S ISR J MED SCI 8 133 972 008 MILLER RW LANCET 1 81 969 009 MUKERJEE D CANCER RES 30 1769 970 010 MUKERJEE D CANCER RES 32 1518 972 011 MUKERJEE D IN: BURDETTE WJ 314 970 012 POTTER AM BR J CANCER 31 348 975 013 SHEIN HM PROC NAT ACAD SCI USA 48 1164 962 014 SNYDER AL LANCET 1 586 970 015 TODARO GJ NAT CANCER INST MONOGRAPH 29 271 969 016 TODARO GJ SCIENCE 153 1252 966 017 TODARO GJ PROC SOC EXP BIOL MED 124 1232 967 018 VELDHUISEN G LANCET 2 529 970 019 YOUNG D LANCET 1 294 971 020 YOUNG D EUR J CANCER 7 337 971 CT 1 WEBB T BR J CANCER 36 583 977 2 WEBB T CANCER RES 37 997 977 3 LI FP CANCER 39 2633 977 4 CONRAD GW J BIOL CHEM 252 6861 977 5 SACK GH IN VITRO 17 1 981 6 HAFEZ M CANCER 55 2434 985 PN 76045 RN 00400 AN 76208332 AU Schneyer-C-A. Wilborn-W-H. TI Effects of cystic fibrosis serum on the rat parotid gland. SO Cell-Tissue-Res. 1976 Jun 11. 169(1). P 111-21. MJ CYSTIC-FIBROSIS: bl. PAROTID-GLAND: de. MN ANIMAL. ATROPHY. CHILD. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. MITOSIS: de. PAROTID-GLAND: im, ul. RATS. TISSUE-EXTRACTS: pd. SUPPORT-U-S-GOVT-P-H-S. AB Injections of serum from human patients with cystic fibrosis into adult rats caused pronounced structural modifications and increased mitotic rate in the parotid gland. Mitotic rate was increased from a low level of 0.02/1,000 acinar cells in parotid glands of adult rats to 6.5/1,000 acinar cells after 2 or 3 days of serum injection. At the light and electron microscopic levels, significant acinar cell atrophy and degranulation were observed. Cellular necrosis, and increases in quantity of lysosome-like dense bodies, mast cells, and macrophages were also detected. These changes are suggestive of tissue response to injurious foreign protein. Furthermore, the fact that normal sera pronounced the same kind of effects (but greatly reduced in extent) strengthens the view that these effects result from the immunologic response of the host organ to foreign antigen. Since, however, the responses of the rat parotid to cystic fibrosis serum were considerably more marked than those elicited by normal serum, the rat parotid may thus have potential usefulness in assaying for the presence of human cystic fibrosis factor. RF 001 BLOMFIELD J GUT 14 558 973 002 BOWMAN BH IN: MANGOS JA 29 973 003 BOWMAN BH SCIENCE 164 325 969 004 BURTON K BIOCHEM J 62 315 956 005 CERIOTTI GJ J BIOL CHEM 214 59 955 006 CONOD EJ PEDIATR RES 9 724 975 007 DALCQ AM IN: DE REUCK AVS 963 008 DOGGETT RG TEX REP BIOL MED 31 685 973 009 DEDUVE C ANNU REV PHYSIOL 28 435 966 010 EL-MOFTY S EXPERIENTIA 30 950 974 011 ERSHOFF BH PROC SOC EXP BIOL MED 110 262 965 012 HERZBERG VL AM J HUM GENET 24 46A 972 013 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 014 RUTBERG U ACTA ODONT SCAND SUPPL 30 19 11 961 015 SCHNEYER CA IN: GOSS R 972 016 SIMSON JV Z ZELLFORSCH MIKROSK ANAT 101 175 969 017 SPOCK A PEDIATR RES 1 173 967 018 WEIL-MALBERBE H METHODS BIOCHEM ANAL 16 293 968 019 WILBORN WH Z ZELLFORSCH MIKROSK ANAT 103 1 970 020 WILBORN WH Z ZELLFORSCH MIKROSK ANAT 130 471 972 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 2 OLIVER C AM J ANAT 155 185 979 3 HAND AR ARCH ORAL BIOL 26 369 981 4 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 5 QWARNSTROM EE J ORAL PATHOL 12 430 983 6 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 7 ROOMANS GM ANN NY ACAD SCI 428 121 984 8 VONEULER AM EXP MOL PATH 43 142 985 PN 76046 RN 00401 AN 77001949 AU Wallwork-J-C. McFarlane-H. TI The SIgA system and hypersensitivity in patients with cystic fibrosis. SO Clin-Allergy. 1976 Jul. 6(4). P 349-58. MJ CYSTIC-FIBROSIS: co. HYPERSENSITIVITY: et. IGA. IGA-SECRETORY. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im. DYSGAMMAGLOBULINEMIA: et. FEMALE. HUMAN. IGA: an. IGA-SECRETORY: an. IGE: an. IMMUNOGLOBULINS-J-CHAIN: an. INFANT. MALE. SALIVA: im. SPUTUM: im. AB A number of investigations have been used for the first time to examine the secretory IgA (SIgA) system in different body fluids from patients with cystic fibrosis (CF). Free J-chain was detected in all the sputum specimens examined. The isolated free secretory component (SC) from CF sputum differed in electrophoretic mobility from the SC isolated from normal human colostrum. In addition the free SC from some CF saliva formed precipitin lines of partial identity with normal human saliva or colostrum. A higher proportion of CF sera (33%) than of normal sera (10%) contained free SC. These investigations suggest that there may be some defect in the synthesis and/or the assembly of the SIgA immunoglobulins, which if confirmed, may help to explain the impaired Type I and Type III allergic manifestations in patients with CF. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 003 CUATRECASAS P PROC NAT ACAD SCI USA 61 636 968 004 GRABAR P IN: GRABAR P 3 964 005 KOHN J J CLIN PATHOL 23 733 970 006 MANCINI G IMMUNOCHEMISTRY 2 235 965 007 MEINKE GC J IMMUNOL 112 1401 974 008 MCFARLANE H BR MED J 1 423 975 009 NEVILLE DM J BIOL CHEM 246 6328 971 010 OUCHTERLONY O IN: KALTOS P 5 1 958 011 TAYLOR B LANCET 2 111 973 012 THOMPSON RA LANCET 2 517 962 013 TOMASI TB JR J IMMUNOL 112 2274 974 014 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 015 WARNER JO PROC EWGCF 5TH ANNU MTG 975 CT 1 RAEBURN JA PROC NUTR SOC 36 77 977 2 HOIBY N MONOGR PAEDIATR 10 138 979 3 PRYJMA J MONOGR PAEDIATR 10 125 979 4 HOIBY N ACTA PAEDIATR SCAND 68 495 979 5 HODSON ME THORAX 35 801 980 6 TOBIN MJ THORAX 35 807 980 7 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 8 MOSS RB CLIN EXP IMMUNOL 47 301 982 9 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 10 FICK RB J CLIN INVEST 74 236 984 11 HANCOCK REW J INFECT DIS 149 220 984 12 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 76047 RN 00402 AN 76234309 AU Shakib-F. Stanworth-D-R. Smalley-C-A. Brown-G-A. TI Elevated serum IgG4 levels in cystic fibrosis patients. SO Clin-Allergy. 1976 May. 6(3). P 237-40. MJ CYSTIC-FIBROSIS: im. IGG: an. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. HYPERSENSITIVITY-IMMEDIATE: di. INFANT. MALE. AB The quantitative measurement of the IgG subclass composition of the sera from sixteen patients with cystic fibrosis has revealed grossly elevated levels of IgG4 in seven patients. The possible significance of this observation is discussed in relation to recent reports of a high incidence of immediate-type hypersensitivity in such patients. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 ANDERSON BR NATURE 217 174 968 003 BERATIS NG PEDIATR RES 7 958 973 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 CONOVER JH PEDIATR RES 7 224 973 006 DANES BS J EXP MED 137 1538 973 007 DEVEY M THESIS 974 008 MORELL A J PEDIATR 80 960 972 009 MCLAUGHLAN P PROC INT IMMUNOL CONG 2ND 974 010 PARISH WE LANCET 2 591 970 011 PARISH WE IN: BRENT L 4 974 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 SHAKIB F J IMMUNOL METH 8 17 975 014 SPOCK A PEDIATR RES 1 173 967 015 STANWORTH DR CLIN ALLERGY 3 37 973 016 VAN DER GIESSEN M CLIN EXP IMMUNOL 21 501 975 CT 1 MCFARLANE H CLIN ALLERGY 7 279 977 2 SHAKIB F BR J DERMATOL 97 59 977 3 TURNER MW ARCH DIS CHILD 53 631 978 4 SHAKIB F CLIN ALLERGY 9 597 979 5 CARSWELL F MONOGR PAEDIATR 10 144 979 6 CARSWELL F CLIN EXP IMMUNOL 35 141 979 7 SHAKIE F RIC CLIN LAB 10 561 980 8 TOBIN MJ THORAX 35 807 980 9 LEA T ACTA DERM VENEREOL SUPPL 92 1980 73 980 10 MARGOLIES R PEDIATR RES 16 181 982 11 SCHLUEDERBERG A YALE J BIOL MED 55 317 982 12 WUTHRICH B DERMATOLOGICA 166 229 983 13 STANLEY PJ CLIN EXP IMMUNOL 58 703 984 14 HEINER DC AM J MED 76 1 984 15 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 16 SHRYOCK TR CURRENT MICROBIOL 12 91 985 17 MOSS RB MONOGR ALLERGY 19 202 986 18 MOSS RB PEDIATR RES 20 453 986 19 SHRYOCK TR J CLIN MICROBIOL 23 513 986 20 FICK RB AM REV RESPIR DIS 133 418 986 21 SCHUR PH ANN ALLERGY 58 89 987 22 MOSS RB CHEST 91 522 987 PN 76048 RN 00403 AN 77024509 AU Tocci-P-M. McKey-R-M-Jr. TI Laboratory confirmation of the diagnosis of cystic fibrosis. SO Clin-Chem. 1976 Nov. 22(11). P 1841-4. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CHLORIDES: me. CYSTIC-FIBROSIS: me. DIAGNOSIS-LABORATORY. ELECTRODES. FEMALE. HUMAN. INFANT. IONTOPHORESIS. MALE. METHODS. PHOTOMETRY. PILOCARPINE: du. POTASSIUM: me. QUALITY-CONTROL. SODIUM: me. SWEAT: me. SUPPORT-U-S-GOVT-NON-P-H-S. AB The recent commercial introduction of a method for detecting albumin in meconium makes screening for cystic fibrosis feasible for many hospitals. If the tests is adopted, confirmatory tests should be available. Quantitative analyses of sweat for sodium by flame photometry and for chloride by silver titration and ion-sleective electrodes are now used as confirmatory tests. We compare results of these confirmatory methods applied to presons with cystic fibrosis, respiratory disorders, or digestive disorders, and to control subjects. RF 001 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 002 DOERSHUK CF J PEDIATR 65 677 964 003 SHWACHMAN H PEDIATRICS 46 335 970 004 STEPHAN U IN: MANGOS JA 281 973 005 BUCHANAN DJ PEDIATRICS 9 304 952 006 RULE AH PEDIATRICS 45 847 970 007 SITZMANN FC PADIATRISCHE PRAXIS 11 85 972 008 DARLING RC AM J MED SCI 225 67 953 009 DI SANTAGNESE PA PEDIATRICS 12 549 953 010 SHWACHMAN H ADV PEDIATR 7 249 955 011 GIBSON LE PEDIATRICS 23 545 959 012 SHWACHMAN H PEDIATRICS 30 167 962 013 GIBSON LE GAP CONF REP PROB SWEAT TESTI 975 014 ANON J PEDIATR 88 711 976 015 KOPITO L PEDIATRICS 43 794 969 016 SHWACHMAN H PEDIATRICS 32 85 963 017 GALEN RS BEYOND NORMALITY THE PREDICTI 975 018 COLEMAN RL CLIN CHEM 19 1319 973 CT 1 WARWICK WJ CLIN CHEM 24 2050 978 2 BOWLING SA AM J MED TECHNOLOGY 48 25 982 3 RANDOLPH VS AM J MED TECHNOLOGY 48 15 982 4 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 PN 76049 RN 00404 AN 76234389 AU Tarnoky-A-L. Bayliss-V-M. Bowen-H-J. Burnow-R-N. Sherington-J. TI The use of electrolyte measurements in the detection of cystic fibrosis. SO Clin-Chim-Acta. 1976 Jun 15. 69(3). P 505-13. MJ CHLORIDES: me. CYSTIC-FIBROSIS: di. SODIUM: me. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg, me. FEMALE. HETEROZYGOTE. HUMAN. MALE. MIDDLE-AGE. NAILS: me. PILOCARPINE: du. PROBABILITY. SALIVA: me. SWEAT: me. AB Six tests recommended for use in the diagnosis of cystic fibrosis (CF) have been compared in the same subjects. The tests were carried out on 165 normal persons, 64 known cases of CF, their 67 parents and 18 sibs. The tests measured sodium in fingernails by activation, sodium in saliva and chloride in saliva and in thermal and pilocarpine-induced sweat by means of ion-specific electrodes, and chloride in pilocarpine-sweat by the standard titrimetric method. The tests ranged in complexity from simple screening methods to individual clinical procedures. None of the simpler tests matched the standard pilocarpine method in diagnostic efficiency, whether they were used singly or in combination. RF 001 BRIMBLECOMBE FSW LANCET 2 1428 973 002 GIBSON LE PEDIATRICS 23 545 959 003 KOPITO L PEDIATRICS 43 794 969 004 BUFFATTI G FRACASTORO (VERONA) 63 307 970 005 GURSON CT HELV PAEDIATR ACTA 28 165 973 006 SZABO L CLIN CHEM 19 727 973 007 TARNOKY AL CLIN BIOCHEM 4 29 971 008 SCHALES O J BIOL CHEM 140 879 941 009 LAWSON D ARCH DIS CHILD 44 715 969 010 BOWEN HJM INT ATOMIC ENERGY AGENCY REP 122 145 970 011 BOX GEP J ROY STATIST SOC (B) 26 211 964 012 KENDALL MG ADVANCED THEORY OF STATISTICS 3 966 013 LACHENBRUCH PA TECHNOMETRICS 10 1 968 014 KOLLBERG H ACTA PAEDIATR SCAND 63 405 974 015 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 CT 1 BRAY PT CLIN CHIM ACTA 80 333 977 2 TARNOKY AL J ROY SOC MED 73 73 980 3 HEELEY AF CLIN CHEM 29 2011 983 PN 76050 RN 00405 AN 76163916 AU Bardon-A. Slerakowska-H. Shugar-D. TI Purification and properties of human acid-thermostable ribonucleases, and diagnosis of childhood pancreatic fibrosis. SO Clin-Chim-Acta. 1976 Mar 15. 67(3). P 231-43. MJ CYSTIC-FIBROSIS: di. RIBONUCLEASES: me. MN CHILD. COPPER: pd. CYSTIC-FIBROSIS: en. DUODENUM: en. HUMAN. KINETICS. LIVER: en. ORGAN-SPECIFICITY. PANCREAS: en. HYDROGEN-ION-CONCENTRATION. RIBONUCLEASES: bl, ip. SPLEEN: en. ZINC: pd. AB Acid-thermostable ribonucleases were isolated from human pancreas, duodenal contents, liver, spleen, serum and urine, and purified 15-- 1000-fold. The pH optima, ionic requirements, and some of the specificity requirements, of these enzymes were investigated. The isolated enzymes formed two distinct groups: (a) The ribonucleases of the pancreas, duodenal contents and fraction A of serum and urine exhibit a pH optimum of 8.5, are inhibited by An2+ and Cu2+, and relatively rapidly hydrolyze the synthetic substrate uridine 3'- (alpha-naphthylphosphate); (b) the ribonucleases of the liver and spleen, and of fractions B of the serum and urine, with a pH optimum of 7, are less sensitive to An2+ and Cu2+, and exhibit negligible activity versus uridine 3'-(alpha-naphthylphosphate). Determination of the serum level of pancreatic-type ribonuclease activity, with the use of uridine 3'-(alpha-naphthylphosphate) or RNA as substrates, appears to be a valid diagnostic tool for pancreatic fibrosis in children. RF 001 ZAN-KOWALCZEWSKA M BIOCHIM BIOPHYS ACTA 341 138 974 002 ALPERS DH J BIOL CHEM 242 5617 967 003 DAVIDSON SJ EXP CELL RES 67 171 971 004 DAVIDSON SJ J CELL BIOL 59 213 973 005 DELANEY R BIOCHEMISTRY 2 438 963 006 NASKALSKI J PRZEGL LEK 29 394 972 007 NASKALSKI J PRZEGL LEK 29 548 972 008 SZNAJD J FOLIA MED CRACOV 14 297 972 009 NASKALSKI J PRZEGL LEK 29 295 972 010 CONNOLLY JH BR J EXP PATHOL 43 402 962 011 LEEPER RD J CLIN ENDOCRINOL METAB 23 426 963 012 BARDON A ACTA BIOCHIM POLON 14 341 967 013 SZER W BIOCHEM PREP 10 139 963 014 KOLE R ACTA BIOCHIM POLON 18 187 971 015 REISFELD RA NATURE 195 281 962 016 GABRIEL O IN: JAKOBY WV 22 565 971 017 DAVIS MT ANALYST 84 248 959 018 BEARD JR J BIOL CHEM 239 4186 964 019 RAZZELL WE METHODS ENZYMOL 6 236 963 020 SUNG SC J BIOL CHEM 237 506 962 021 BESSEY OA J BIOL CHEM 196 175 952 022 LOWRY OH J BIOL CHEM 193 265 951 023 KAPLAN HS J BIOL CHEM 222 907 956 024 GORDON J ARCH BIOCHEM BIOPHYS 112 421 965 025 BERMAN LB PROC SOC EXP BIOL MED 97 175 958 026 SCHULTZE-JENA BS IN: KOCH E 43 964 027 RESSLER N NATURE 210 695 966 028 HOUCK JC ARCH BIOCHEM BIOPHYS 73 384 958 029 POLATNICK J PROC SOC EXP BIOL MED 105 486 960 030 GUPTA S ARCH BIOCHEM BIOPHYS 101 88 963 031 EICHEL HJ ARCH BIOCHEM BIOPHYS 107 197 964 032 BARNARD EA NATURE 221 340 969 CT 1 SZCZESNAKACZMAREK A EXPERIENTIA 32 1499 976 2 BARDON A BIOCHIM BIOPHYS ACTA 438 461 976 3 KOLE R BIOCHIM BIOPHYS ACTA 438 540 976 4 REDDI KK PREPAR BIOCHEM 7 283 977 5 SIERAKOWSKA H PROG NUCLEI ACID RES MOL BIOL 20 59 977 6 RABIN EZ CLIN CHIM ACTA 78 235 977 7 NEUWELT EA BIOCHEM J 163 419 977 8 GAHL WA BIOCHEM MED 19 294 978 9 NEUWELT EA CANCER RES 38 88 978 10 WARSHAW AL SURGERY 86 227 979 11 AKSOY M EUR J CANCER 16 1041 980 12 BARDON A CLIN CHIM ACTA 101 17 980 13 CRANSTON JW BIOCHIM BIOPHYS ACTA 616 239 980 14 THOMAS JM ELECTROPHORESIS 2 104 981 15 REDDI KK CLIN BIOCHEM 14 191 981 16 BLANK A BIOCHEMISTRY 20 2261 981 17 SUGIYAMA RH BIOCHEMISTRY 20 2268 981 18 CRANSTON JW CLIN CHEM 27 1362 981 19 IWAMA M J BIOCHEM 89 1005 981 20 THOMAS JM CLIN CHIM ACTA 111 199 981 21 THOMAS JM CLIN CHIM ACTA 111 185 981 22 MARET A C R ACAD SCI SER III 293 741 981 23 WEICKMANN JL J BIOL CHEM 257 8705 982 24 FABRIS C IRCS MED SCI BIOCHEM 11 775 983 25 KUROKAWA E CLIN CHIM ACTA 128 83 983 26 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 27 WEICKMANN JL CANCER RES 44 1682 984 28 BARDON A ACTA PAEDIATR SCAND 73 263 984 29 HISHIKI S CLIN CHIM ACTA 136 155 984 30 THOMAS JM CLIN CHIM ACTA 142 73 984 31 DEPRISCO R BIOCHIM BIOPHYS ACTA 788 356 984 32 CEDER O ACTA PAEDIATR SCAND 74 102 985 33 NIWATA Y J BIOCHEM 97 923 985 34 BEINTEMA JJ MOL BIOL EVOLUT 3 262 986 35 JUNG K J CLIN CHEM CLIN BIOCHEM 24 243 986 36 SHAPIRO R BIOCHEMISTRY 25 7255 986 37 HITOI A J BIOCHEM 101 29 987 PN 76051 RN 00406 AN 76234394 AU Biswas-S. Norman-A-P. Baffoe-G. Graves-L. TI Prolactin, growth hormone and alpha-fetoprotein in children with cystic fibrosis. SO Clin-Chim-Acta. 1976 Jun 15. 69(3). P 541-2. MJ ALPHA-FETOPROTEINS: me. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: me. PROLACTIN: bl. SOMATOTROPIN: bl. MN ADULT. CHILD. FEMALE. HUMAN. MENSTRUATION. MICROCHEMISTRY. PREGNANCY. RADIOIMMUNOASSAY: mt. EX Prolactin is credited to have a role in the maintenance of electrolytic and water balance by the kidneys although its effect on the composition of sweat is unknown. We assayed prolactin, growth hormone and alpha-fetoprotein in the sera of 69 children with cystic fibrosis attending the Hospital for Sick Children. Sera from 12 children without cystic fibrosis were used as control. It appears that children with cystic fibrosis have serum levels of prolactin, GH and alpha-FP similar to those without this disease and that children have significantly higher levels of alpha-FP than women of reproductive age. RF 001 BECKER DJ J CLIN ENDOCRINOL METAB 41 782 975 002 PIMSTONE BL LANCET 2 799 966 003 CHANDRA RK BR MED J 1 714 975 004 SMITH JA BR MED J 2 392 975 005 WALLWORK JC BR MED J 2 392 975 006 BROCK DJH BR MED J 2 392 975 007 FITZSIMMONS JS BR MED J 3 544 975 008 KNOPFLE G BR MED J 4 459 975 009 BISWAS S J ENDOCRINOL 54 251 975 010 RODECK CH BR J OBSTET GYNAECOL 83 24 976 CT 1 BISWAS S CLIN CHIM ACTA 73 363 976 2 BISWAS S BR J OBSTET GYNAECOL 83 683 976 3 ALHADEFF JA CLIN GENET 14 189 978 4 BISWAS S FERTIL STERIL 30 200 978 5 MONTGOMERY ACV J ROY SOC MED 72 489 979 6 HUBBARD VS AM J DIS CHILD 134 317 980 7 PEDERSEN PS ACTA PAEDIATR SCAND 72 757 983 8 PREECE MA CLIN ENDOCRINOL METAB 15 453 986 PN 76052 RN 00407 AN 76234440 AU Forstner-G. Koheil-A. Forstner-J. TI Isoelectric focusing of serum in cystic fibrosis: failure to distinguish between homozygote and heterozygote sera. SO Clin-Chim-Acta. 1976 Aug 2. 70(3). P 459-62. MJ BLOOD-PROTEINS: me. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. MN ADOLESCENCE. ADULT. BLOOD-PROTEIN-ELECTROPHORESIS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. ISOELECTRIC-FOCUSING. MALE. MIDDLE-AGE. PEDIGREE. AB Thin-layer isoelectric focusing was performed on samples of sera from patients with Cystic Fibrosis, siblings and obligate heterozygotes (parents), and children without cystic fibrosis (controls). The protein band with an isoelectric point of pH 5.48, previously reported to be absent in homozygote cystic fibrosis sera, was found to have a pI of 5.25. It was present in approximately one-half of the homozygote and heterozygote sera tested, and absent from 18 percent of control sera. The presence of this band is not therefore a reliable marker for the normal gene, and cannot be used to identify the heterozygous carrier for cystic fibrosis. RF 001 SPROUL A J PEDIATR 69 759 966 002 SPOCK A PEDIATR RES 1 173 967 003 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 004 FORSTNER GG CAN MED ASSOC J 113 550 975 005 WILSON GB CLIN CHIM ACTA 49 79 973 006 WILSON GB PEDIATR RES 10 87 976 007 ALTLAND K HUMANGENETIK 28 207 975 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 76053 RN 00408 AN 77002035 AU Norgaard-Pedersen-B. Axelsen-N-H. TI Alpha-fetoprotein-like activity in sera from patients with malignant and non-malignant disease and healthy individuals. SO Clin-Chim-Acta. 1976 Sep 6. 71(2). P 343-7. MJ ALPHA-FETOPROTEINS: me. FETAL-PROTEINS: me. LIVER-DISEASES: me. MN COMPARATIVE-STUDY. CYSTIC-FIBROSIS: me. FEMALE. HEPATITIS: me. HUMAN. IMMUNOELECTROPHORESIS. LIVER-CIRRHOSIS: me. LIVER-NEOPLASMS: me. PREGNANCY. AB A new method, radio-crossed immunoelectrophoresis, demonstrates alpha- fetoprotein (AFP) in sera with a sensitivity of 1 mug/1. By this method AFP with alpha mobility was not found in sera from healthy individuals, patients with chronic active hepatitis and cirrhosis, primary biliary cirrhosis, secondary liver cancer and cystic fibrosis. In some of the sera, AFP was elevated when measured by conventional radioimmunoassay method and the sera contained an AFP- like substance with gamma mobility when analyzed by radio-crossed immunoelectrophoresis. The nature of this gamma substance is still obscure and needs further investigation. RF 001 ABELEV GI ADV CANCER RES 14 295 971 002 NORGAARD-PEDERSEN B ACTA PATH MICROBIOL SCAND 83 275 975 003 RUOSLAHTI E BR MED J 2 527 974 004 BELANGER L PATHOL BIOL (PARIS) 21 449 973 005 WALDMANN TA LANCET 2 1112 972 006 CHANDRA RK BR MED J 1 714 975 007 SEPPALA M LANCET 1 375 972 008 RUOSLAHTI E NATURE 235 161 972 009 RUOSLAHTI E TRANSPLANT REV 20 38 974 010 NORGAARD-PEDERSEN B CLIN CHIM ACTA 48 345 973 011 LEEK AE COLLOQ INSERM ALPHA-FETOPRO 563 974 012 DAMBRUYANT C J IMMUNOL METH 7 387 975 013 MORGAN CR DIABETES 13 579 964 014 PAGE LB CLIN CHIM ACTA 34 55 971 015 FLORIN-CHRISTENSEN A BR MED J 2 94 973 CT 1 BROCK DJH PROG MED GENET 2 1 977 2 NORGAARDPEDERSEN B SCAND J CLIN LAB INVEST 37 46 977 3 BJERRUM OJ BIOCHIM BIOPHYS ACTA 472 135 977 4 NORGAARDPEDERSEN B CANCER 41 2315 978 5 SCHULTZ H CANCER 42 2182 978 6 ADINOLFI M ADV HUM GENET 9 165 979 7 RUOSLAHTI E ADV CANCER RES 29 275 979 8 SOKOLENKO AA BULL EXP BIOL MED 88 1093 979 9 NORGAARDPEDERSEN B CLIN GENET 17 355 980 PN 76054 RN 00409 AN 77067410 AU Anderson-L-C. Smith-Q-T. Shapiro-B-L. TI Cellular glycosaminoglycans in lymphocytes from patients with cystic fibrosis. SO Clin-Chim-Acta. 1976 Nov 15. 73(1). P 63-5. MJ CYSTIC-FIBROSIS: bl. GLYCOSAMINOGLYCANS: bl. LYMPHOCYTES: me. MN ELECTROPHORESIS-CELLULOSE-ACETATE. GALACTOSAMINE: an. GLUCOSAMINE: an. HUMAN. AB Cellular glycosaminoglycans were isolated from lymphocytes from patients with cystic fibrosis and controls. The isolated glycosaminoglycans were fractionated by cellulose acetate electrophoresis, analyzed for glucosamine and galactosamine content, and subjected to hydrolysis with bovine testicular hyaluronidase. The total glycosaminoglycan content, the per cent glucosamine and galactosamine, and the distribution of cellular glycosaminoglycans in circulating lymphocytes in cystic fibrosis were no different from controls. RF 001 DANES BS BIOCHEM GENET 12 359 974 002 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 003 LINDE A CALC TISS RES 12 281 973 004 BREEN M ANAL BIOCHEM 35 146 970 005 MURPHY D CLIN CHIM ACTA 53 145 974 006 SCHUBERT M PRIMER ON CONNECTIVE TISS 68 968 007 MURATA K CLIN CHIM ACTA 57 115 974 008 TANIGUCHI N BIOCHEM MED 11 217 974 009 WELCH DW PEDIATR RES 9 698 975 CT 1 CAPEAU J BIOMEDICINE 28 124 978 2 HUESO P REV ESP FISIOL 34 339 978 3 LATIF KA NUTR REP INT 24 471 981 4 SHAPIRO BL SCIENCE 216 417 982 5 ASTAKHOVA TA TER ARKH 59 62 987 PN 76055 RN 00410 AN 76256081 AU Alhadeff-J-A. Tennant-L. Obrien-J-S. TI Altered isoenzyme patterns of liver alpha-L-fucosidase in cystic fibrosis. SO Clin-Genet. 1976 Aug. 10(2NA-NA-760903-760909). P 63-72. MJ CYSTIC-FIBROSIS: en. DISACCHARIDASES: me. FUCOSIDASE: me. ISOENZYMES: me. LIVER: en. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT-NEWBORN. ISOELECTRIC-FOCUSING. MALE. MIDDLE-AGE. NEURAMINIDASE: pd. SIALIC-ACIDS: an. AB The isoenzyme pattern of alpha-L-fucosidase was studied by isoelectric focusing in livers from seven patients with cystic fibrosis and in normal and pathological (GM1-gangliosidosis, Type II and Sanfilippo disease) controls. The controls had very reproducible patterns consisting of seven isoenzymes of alpha-L-fucosidase with the most neutral from (I) representing a small proportion of the total activity. All seven of the cystic fibrosis livers had altered alpha-L-fucosidase isoenzyme patterns. The chemical relationship of the seven isoenzymes of normal liver alpha-L-fucosidase was investigated using neuramindase. The five most acidic forms of alpha- L-fucosidase appear to be related to the most neutral form by sialic acid residues. Since the isoenzymes of liver alpha-L-fucosidase appear to be related by sialic acid residues, it is possible that the altered alpha-L-fucosidase isoenzyme patterns found in cystic fibrosis livers may result from aberrant sialylation. RF 001 ALHADEFF JA CLIN CHIM ACTA 57 307 974 002 ALHADEFF JA J BIOL CHEM 250 7106 975 003 ALHADEFF JA DEVELOP BIOL 47 319 975 004 ANTONOWICZ I PEDIATR RES 6 803 972 005 BOAT TF CLIN PEDIATR 13 505 974 006 BUTTERWORTH J CLIN CHIM ACTA 41 367 972 007 DISCHE Z PEDIATRICS 24 74 959 008 DISCHE Z AM J DIS CHILD 102 733 961 009 DISCHE Z ANN NY ACAD SCI 93 526 962 010 DOMINICK HC KINDERHEILK 119 261 975 011 FILLIAT M PATHOL BIOL (PARIS) 21 13 973 012 GILLY R PEDIATRIE 29 465 974 013 GOLDSTONE AP FEBS LETTERS 13 68 971 014 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 015 KATTWINKEL J J PEDIATR 82 234 973 016 KIM YS J CLIN INVEST 51 2033 972 017 KINT JA ARCH INTERN PHYSIOL BIOCHIM 79 633 971 018 KNAUFF RE CLIN CHIM ACTA 19 245 968 019 KRAUS I PEDIATRICS 47 1010 971 020 LIEBERMAN J AM REV RESPIR DIS 109 399 974 021 LOBECK CC IN: STANBURY JB 1605 972 022 LOUISOT P CLIN CHIM ACTA 48 373 973 023 LOWRY OH J BIOL CHEM 193 265 951 024 MANDEL ID AM J DIS CHILD 113 431 967 025 MARCHI AG HELV PAEDIATR ACTA 28 427 973 026 MCCOMBS ML TEX REP BIOL MED 31 615 973 027 MOOKERJEA S CAN J BIOCHEM 50 738 972 028 NEAL JL UNION MED CAN 102 1465 973 029 RAO GJS PEDIATR RES 8 684 974 030 ROSSITER MA ACTA PAEDIATR SCAND 63 389 974 031 SHAPIRO BL LANCET 2 1020 974 032 SINGER L CLIN BIOCHEM 7 146 974 033 TAUSSIG LM PEDIATRICS 54 229 974 034 TURNER BM AM J HUM GENET 27 651 975 035 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 CALLAHAN JW PEDIATR RES 11 1166 977 2 ALHADEFF JA CLIN GENET 13 417 978 3 ALHADEFF JA CLIN GENET 13 207 978 4 ALHADEFF JA CLIN GENET 14 189 978 5 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 6 ALHADEFF JA CLIN CHIM ACTA 89 469 978 7 CASOLA L MONOGR PAEDIATR 10 96 979 8 ALHADEFF JA BIOCHEM BIOPHYS RES COMMUN 86 787 979 9 SCANLIN TF CLIN CHIM ACTA 91 197 979 10 WILLCOX P CLIN CHIM ACTA 91 81 979 11 BUTTERWORTH J CLIN CHIM ACTA 92 109 979 12 CASOLA L CLIN CHIM ACTA 94 83 979 13 JAKEL HP BIOL ZENTRALBL 98 55 979 14 SCANLIN TF CLIN CHEST MED 1 424 980 15 ALHADEFF JA CLIN CHIM ACTA 105 131 980 16 JAKEL HP BIOL ZENTRALBL 100 273 981 17 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 18 HULTBERG B CLIN CHIM ACTA 112 167 981 19 SCANLIN TF CLIN CHIM ACTA 114 269 981 20 HARRIS A CLIN CHIM ACTA 116 171 981 21 MARGOLIES R PEDIATR RES 17 931 983 22 GUEANT JL CLIN CHIM ACTA 143 217 984 PN 76056 RN 00411 AN 76186625 AU Danes-B-S. Hodson-M-E. Batten-J. TI Cystic fibrosis: a cell culture study on an adult patient population. SO Clin-Genet. 1976 May. 9(5). P 527-32. MJ CYSTIC-FIBROSIS: fg. PHENOTYPE. MN ADOLESCENCE. ADULT. CELLS-CULTURED. FEMALE. FIBROBLASTS: pa. HUMAN. MALE. SKIN: pa. AB Skin fibroblast cultures from an adult population of cystic fibrosis (CF) patients have been studied to determine any possible genetic or clinical significance of the two classes described for cultured CF skin fibroblasts (Danes 1973). On the basis of clinical course, the 46 adult patients from 43 unrelated families studied were divided into two groups (40 typical, 6 atypical). The cultured fibroblasts from 37 of the 40 patients with typical CF were Class I (metachromatic, cystic fibrosis factor activity (CFFA) in the culture medium, and metabolic cooperation with normal fibroblasts), and the cultures from the remaining three patients were Class II (ametachromatic, no CFFA in the culture medium and no metabolic cooperation with metachromatic CF fibroblasts). During the 4-year research period one of the 37 patients from Class I and all three Class II patients with typical CF succumbed. The cultured skin fibroblasts from two of the six patients who had an atypical course for CF were Class I and four were Class II. When the parents of three of these atypical CF Class II patients were studied, the cultured fibroblasts from one parent in each family in each family were Class I and the other Class II. The affected offspring from such Class I-II matings may represent genetic compounds. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BERATIS NG PEDIATR RES 7 958 973 003 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 004 DANES BS BIRTH DEF ORIG ART SER 8 114 972 005 DANES BS LANCET 2 765 973 006 DANES BS J EXP MED 129 775 969 007 DANES BS AM J HUM GENET 23 297 971 008 GRAHAM JB AM J MED SCI 225 46 953 010 MCKUSICK VA AM J HUM GENET 25 446 973 011 MCKUSICK VA LANCET 1 993 972 012 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 CT 1 DANES BS CLIN GENET 11 83 977 2 DANES BS CLIN GENET 13 327 978 3 JAKEL HP BIOL ZENTRALBL 98 55 979 4 WINGE P DAN MED BULL 29 358 982 PN 76057 RN 00412 AN 78042375 AU Chilla-R. Arglebe-C. Lubahn-H. Doering-K-M. TI 'Fast isoamylases' in parotid saliva of heterozygous carriers of cystic fibrosis. SO Clin-Otolaryngol. 1976. 1(4). P 309-13. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: ge. HETEROZYGOTE. ISOAMYLASE: ge. SALIVA: en. MN ADULT. CHILD. CYSTIC-FIBROSIS: fg. FEMALE. HUMAN. MALE. PAROTID-GLAND: en. AB An increased number of 'fast isoamylases' is found in the parotid saliva of children with cystic fibrosis and their heterozygous parents who are clinically healthy. 'Fast isoamylases' are generated, at least in part, from the 6 isoamylases of the basic pattern by deamidation of asparagine and glutamine residues. This basic pattern is present in the parotid saliva of all subjects we have tested so far. A shift in the equilibrium between amidation and deamidation processes is discussed in terms of its possible significance for the pathogenesis of cystic fibrosis and for ageing. RF 002 ARGLEBE C CLIN OTOLARYNGOLOGY 1 249 976 003 KELLER PJ BIOCHEMISTRY 10 4867 971 004 LOWRY OH J BIOL CHEM 193 265 951 005 STREET HV CLIN CHIM ACTA 1 256 956 006 ARGLEBE C LARYNGOL RHINOL OTOL 54 542 975 007 VAN KLEEF FSM NATURE 258 264 975 008 ROBINSON AB PROC NAT ACAD SCI USA 66 753 970 009 GREENBERG DM METABOLIC PATHWAYS 2 968 PN 76058 RN 00413 AN 76209110 AU Dolan-T-F-Jr. TI Hemolytic anemia and edema as the initial signs in infants with cystic fibrosis. Consider this diagnosis even in absence of pulmonary symptoms. SO Clin-Pediatr (Phila). 1976 Jul. 15(7). P 597-600. MJ ANEMIA-HEMOLYTIC: co. CYSTIC-FIBROSIS: di. EDEMA: co. PANCREATIC-DISEASES: di. MN CASE-REPORT. CYSTIC-FIBROSIS: co. DIET. HUMAN. INFANT. INFANT-NEWBORN. MALE. PANCREATIC-DISEASES: co. VITAMIN-E-DEFICIENCY: co. AB This is a report of five infants eventually proven to have cystic fibrosis of the pancreas, who presented with hemolytic anemia and edema. Since the sweat test is often unreliable in edematous states, the possibility of cystic fibrosis should be considered. Use of either a protein hydrolysate or addition of pancreatic enzymes will improve nutritional status, and when edema disappears, a definite sweat test can be performed. Evidence of vitamin E deficiency in some of the cases is presented, and the possibility of this as a cause of anemia is raised. RF 001 SHAHIDI NT J PEDIATR 59 533 961 002 LEE PA JAMA 228 585 974 003 FLEISHER DS J PEDIATR 64 341 964 004 OSKI FA J PEDIATR 70 211 967 005 HASSAN H AM J CLIN NUTR 19 147 966 006 RITCHIE JH N ENGL J MED 279 1185 968 007 BLANC WA PEDIATRICS 22 494 958 008 MELHORN DK BLOOD 37 438 971 009 HARRIS PL AM J CLIN NUTR 13 385 963 010 WILLIAMS ML N ENGL J MED 292 887 975 011 MELHORN DK J PEDIATR 79 569 971 CT 1 CHASE HP J PEDIATR 95 337 979 2 CONGDON PJ POSTGRAD MED J 57 453 981 3 PARK RW GASTROENTEROLOGY 81 1143 981 4 FRATES RC AM J DIS CHILD 135 719 981 5 TURKEL SB CLIN PERINATOL 9 613 982 6 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 7 FARRELL PM J DENT CHILD 50 385 983 8 DWORKIN B J PARENT ENTERAL NUTR 11 38 987 PN 76059 RN 00414 AN 76256271 AU Warwick-W-J. Hansen-L-G. Sharp-H. TI Absorption of vitamin A in patients with cystic fibrosis. Absorption is best with emulsified vitamin A alcohol. SO Clin-Pediatr (Phila). 1976 Sep. 15(9). P 807-10. MJ CYSTIC-FIBROSIS: me. INTESTINAL-ABSORPTION. VITAMIN-A: me. MN ADOLESCENCE. ADULT. ALCOHOL-ETHYL: me. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: dt. EMULSIONS. FEMALE. HUMAN. INFANT. MALE. PALMITATES: me. VITAMIN-A-DEFICIENCY: dt. VITAMIN-A: ad. VITAMIN-A: bl. AB Vitamin A absorption tests using vitamin A palmitate and alcohol separately in oil and oil-water emulsions were done on 43 patients with cystic fibrosis. Patients were given 7,000 units of vitamin A per kilogram of body weight with a fatty breakfast. Pancreatic enzymes were not given with the test meal and were withheld for five hours from start of test. Blood was drawn before administration of the vitamin and at three and five hours after administration. Serum vitamin A levels were estimated using the Carr-Price technique. The percentages of patients with normal vitamin A absorption were 85 with vitamin A alcohol in oil-water emulsion, 61 with vitamin A alcohol dissolved in oil, 64 with vitamin A palmitate in oil-water emulsion, and 19 with vitamin A palmitate in oil. The number of stools per day is an inverse indicator of retention time in the intestine. Absorption of fat soluble vitamins is always abnormal when a patient has four or more stools a day. The observations that cystic fibrosis patients with abnormal liver biopsies have poor absorption of vitamin A were not statistically significant. The question of the effect of cirrhosis in cystic fibrosis on vitamin A absorption remains unresolved. RF 001 FITZGERALD O GUT 2 261 961 002 FITZGERALD O GUT 3 74 962 003 FITZGERALD O GUT 3 264 962 004 CARR FH BIOCHEM J 20 497 926 005 MAY CD AM J DIS CHILD 59 1167 940 006 WALKER WA PEDIATRICS 40 946 967 007 LEWIS JM J PEDIATR 31 496 947 008 KAGAN BM J CLIN INVEST 29 141 950 009 BARNES BC J CLIN INVEST 29 982 950 010 FOX HJ J LAB CLIN MED 34 1140 949 011 HAMRE CJ AM J MED SCI 220 183 950 CT 1 CHASE HP J PEDIATR 95 337 979 2 PARK RW GASTROENTEROLOGY 81 1143 981 3 FARRELL PM J DENT CHILD 50 385 983 4 VANBOVEN M J AM ACAD DERMATOL 12 822 985 5 RASMUSSEN M J PEDIATR GASTROENTEROL NUTR 5 397 986 PN 76060 RN 00415 AN 76234881 AU Weeke-B. Flensborg-E-W. Jacobsen-L. Jorgensen-B-A. Lykkegaard-E. Hoiby-N. TI Immunochemical quantitation of 18 proteins in sera from patients with cystic fibrosis: concentrations correlated to class of fibroblast metachromasia, clinical and radiological lung symptoms. SO Dan-Med-Bull. 1976 Jun. 23(3). P 155-60. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. FIBROBLASTS: me. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. ENGLISH-ABSTRACT. FEMALE. FLUORESCENT-ANTIBODY-TECHNIC. HUMAN. IMMUNOELECTROPHORESIS. LUNG-DISEASES: bl. MALE. EX Sera from 32 patients with cystic fibrosis classified according to fibroblast metachromasia into three classes have been analysed immunochemically for the concentrations of 18 serum proteins. In addition, the clinical and radiological status of the lungs were evaluated, using two scoring systems. Eleven of 16 serum proteins differed from the values of a thousand-donor serum pool. The most pronounced differences from the control sera were elevated acute-phase proteins, notably haptoglobin, alpha1-antichymotrypsin, IgG, and IgA, and low serum albumin and alpha2-HS-glycoprotein. Patients from metachromatic class III presented the most pronounced rise in acute-phase proteins and, furthermore, significantly decreased complement C3 and IgM, IgG and IgA were normal in class III patients. These differences from the control sera possibly reflect inflammatory tissue damage involving immune reactions. The clinical score was positively correlated to the radiologic score, and both these scores were positively correlated to the concentrations of eight and seven, respectively, of the serum proteins, and negatively correlated to three of the serum proteins. Based upon the correlations between the scores and the concentrations of some of the serum proteins, it is recommended that longitudinal prospective studies follow individual patients for the concentrations in serum of haptoglobin, albumin, IgA, IgG, C3, and, eventually, IgM and alpha1-antichymotrypsin. The present results suggest that these serum proteins will yield valuable information as regards prognosis and therapy of the lung disease in CF. RF 001 BELFRAGE S ACTA MED SCAND SUPPL 173 5 963 002 BERG T ACTA PAEDIATR SCAND 58 513 969 003 BROGAN TD THORAX 30 72 975 004 BUCKLEY RH J CLIN INVEST 55 157 975 005 CONOVER JH LIFE SCI 14 253 974 006 CONOVER JH LANCET 2 1501 973 007 CONOVER JH LANCET 1 47 975 008 DANES BS J EXP MED 129 775 969 009 DANES BS AM J HUM GENET 23 297 971 010 DANES BS J EXP MED 137 1538 973 011 HANN S LANCET 2 520 974 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 014 HOIBY N ACTA PAEDIATR SCAND 63 843 974 015 HOIBY N SCAND J RESPIR DIS 56 38 975 016 LANGGARD H ACTA PAEDIATR SCAND 57 255 968 017 LIEBERMAN J LANCET 1 1230 974 018 LUNDH B ACTA UNIV LUND II 24 1 965 019 LYKKEGAARD E DAN MED BULL 21 232 974 020 LYKKEGAARD E DAN MED BULL 21 241 974 021 LYKKEGAARD E DAN MED BULL 22 169 975 022 LYKKEGAARD E DAN MED BULL 22 175 975 023 LYKKEGAARD E DAN MED BULL 22 177 975 024 MCFARLANE H BR MED J 1 423 975 025 MULLER HE DTSCH MED WSCHR 93 120 968 026 MINCHIN-CLARKE HG THORAX 25 423 970 027 NIELSEN HG CLIN CHIM ACTA 36 133 972 028 NORMAN AP PROC EWGCF 2ND ANNU MTG 971 029 NYMAN M SCAND J CLIN LAB INVEST SUPPL 39 5 959 030 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 031 SCHWARTZ RH AM J DIS CHILD 111 408 966 032 STROBER W PEDIATRICS 43 416 969 033 TANNER JM ARCH DIS CHILD 41 613 966 034 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 035 WEEKE B ACTA MED SCAND 192 149 972 036 WEEKE B THESIS 1 973 037 WEEKE B SCAND J IMMUNOL SUPPL 1 2 37 973 038 WEEKE B SCAND J IMMUNOL SUPPL 2 1 48 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 TURNER MW ARCH DIS CHILD 53 631 978 3 GOTZ M EUR J PEDIATR 127 133 978 4 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 7 FICK RB CLIN CHEST MED 2 91 981 8 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 9 MARGOLIES R PEDIATR RES 16 181 982 10 BERNINGER RW METABOLISM 31 299 982 11 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 12 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 13 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 14 EMMETT M PROC SOC EXP BIOL MED 184 74 987 PN 76061 RN 00416 AN 77003984 AU Paulsen-E-P. Koury-M. TI Hemoglobin AIc levels in insulin-dependent and -independent diabetes mellitus. SO Diabetes. 1976. 25(2 SUPPL). P 890-6. MJ DIABETES-MELLITUS: bl. MN ACIDOSIS-DIABETIC: bl. ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. DIABETES-MELLITUS: dt. DIABETES-MELLITUS-INSULIN-DEPENDENT: bl. DIABETES-MELLITUS-INSULIN-DEPENDENT: ci. ERYTHROCYTES: me. FEMALE. HUMAN. INFANT. INSULIN: tu. MALE. OBESITY-IN-DIABETES: bl. PREDNISONE: ae. PREGNANCY-IN-DIABETES: bl. PREGNANCY. AB Unusual increases in the minor hemoglobin components (Hb AIa, b, c) known to be elevated in diabetes mellitus were found in states of relative or absolute insulinopenia: diabetic ketoacidosis, steroid- induced diabetes, insulin-dependent diabetes in cystic-fibrosis patients, and cystic fibrosis occurring in infants who have a marked suppression of insulin secretion. In ketoacidotic diabetics, it required at least a month for high Hb AI levels (16.9 +/- 2.6 per cent) to stabilize at nonacidotic levels (12.8 +/- 0.3 per cent), suggesting that decreases occur only as new red cells form under conditions less favorable to Hb AI synthesis. Abnormal amounts os Hb A and Hb AI resisted removal from diabetic red-cell membranes by low ionic buffers but yielded to hypotonic Tris buffer. Their removal resulted in simultaneous elution of peripheral and integral membrane proteins. It is suggested that Hb so firmly bound could reduce membrane elasticity and cell deformability, characteristics so vital to normal red cell movement through the microvasculature. RF 001 ALLEN DW J AM CHEM SOC 80 1628 958 002 HOLMQUIST WR BIOCHEMISTRY 5 2489 966 003 BOOKCHIN RM BIOCHEM BIOPHYS RES COMMUN 32 86 968 004 BUNN HF BIOCHEM BIOPHYS RES COMMUN 67 103 975 005 BUNN HF J CLIN INVEST 49 1088 970 006 HUISMAN TH J LAB CLIN MED 60 302 962 007 RAHBAR S BIOCHEM BIOPHYS RES COMMUN 36 838 969 008 TRIVELLI LA N ENGL J MED 284 353 971 009 PAULSEN EP METABOLISM 22 269 973 010 DITZEL J ACTA PAEDIATR SCAND 64 355 975 011 DITZEL J ACTA MED SCAND SUPPL 578 59 975 012 KOENIG RJ PROC NAT ACAD SCI USA 72 3687 975 013 KOENIG RJ DIABETES 25 1 976 014 DRABKIN DL J BIOL CHEM 164 703 946 015 DODGE JT ARCH BIOCHEM BIOPHYS 100 119 963 016 LOWRY OH J BIOL CHEM 193 265 951 017 PAULSEN EP PEDIATR RES 7 325 973 018 MILNER AD ARCH DIS CHILD 44 351 969 019 BEISSWENGER PJ SCIENCE 168 596 970 020 NICOLSON GL J CELL BIOL 59 395 973 021 MARFEY P IN: JAMIESON GA 309 969 022 DITZEL J IN: DITZEL J 123 970 CT 1 ANON BR MED J 1 1555 977 2 PAULSEN EP MED COLL VIRGINIA Q 13 26 977 3 PALMBERG PF DIABETES 26 703 977 4 NG RCK GASTROENTEROLOGY 73 804 977 5 DITZEL J ACTA PAEDIATR SCAND SUPPL 270 1977 112 977 6 ANON BR MED J 1 1373 978 7 DAVIS RE MED J AUST 1 530 978 8 DITZEL J BR MED J 1 741 978 9 MOSES RG MED J AUST 2 160 978 10 BECCARIA L AM J HEMATOL 4 367 978 11 JUHAN I NOUV PRESSE MED 7 759 978 12 HANDELSMAN DJ AUST NZ J MED 8 R 1 978 13 GONEN B DIABETOLOGIA 15 1 978 14 ABRAHAM EC DIABETES 27 931 978 15 DAVIS JE DIABETES 27 102 978 16 DITZEL J METABOLISM 27 927 978 17 GRAF RJ DIABETES 27 834 978 18 MCMILLAN DE DIABETES 27 895 978 19 RASKIN P METABOLISM 27 235 978 20 WIDNESS JA DIABETES 27 1182 978 21 MAQUART F BIOMEDICINE EXPRESS 29 208 978 22 DAUMERIEGOFFINET C ACTA CLIN BELG 33 240 978 23 SCHROTER W HELV PAEDIATR ACTA 33 535 978 24 LESPERANCE FA MED CLIN NORTH AM 62 767 978 25 WIDNESS JA BR J OBSTET GYNAECOL 85 812 978 26 WIDNESS JA J PEDIATR 92 8 978 27 FRASER DM BR MED J 1 979 979 28 FRIEDMAN S HEMOGLOBIN 3 411 979 29 BROWNLEE M ATHEROSCLEROSIS REV 4 29 979 30 JUHAN I NOUV PRESSE MED 8 4083 979 31 MCDONALD JM HUM PATHOL 10 279 979 32 ANGELI A ACTA DIABETOL LAT 16 295 979 33 BRESSLER R DRUGS 17 461 979 34 MOLNAR GD DIABETOLOGIA 17 5 979 35 DIX D CLIN CHEM 25 877 979 36 GRUBER CA CLIN CHEM 25 1970 979 37 HAMBURGER S J AM MED WOM ASSOC 34 109 979 38 POLLAK A BIOL NEONATE 36 185 979 39 DITZEL J FED PROC 38 2484 979 40 GIACOMELLI F LAB INVEST 40 460 979 41 FADEL HE OBSTET GYNECOL 54 322 979 42 SPICER KM J CLIN INVEST 64 40 979 43 LIND T BR J OBSTET GYNAECOL 86 210 979 44 KLUJBER L EUR J PEDIATR 132 289 979 45 OSHAUGHNESSY R AM J OBSTET GYNECOL 135 783 979 46 BEYLOT M LYON MED 242 179 979 47 DANEMAN D DIABETOLOGIA 19 423 980 48 DAVIES DM AUST NZ J OBSTET GYNAECOL 20 147 980 49 HATRON PY LILLE MED 25 213 980 50 STUART J J CLIN PATHOL 33 417 980 51 GENEZ VS TER ARKH 52 142 980 52 BYYNY RL POSTGRAD MED 68 191 980 53 KJAERGAARD JJ ACTA ENDOCRINOL 94 25 980 54 JOHNSON MW CLIN CHIM ACTA 104 319 980 55 GOEBEL FD MUNCH MED WOCHENSCHR 122 1731 980 56 SCHROTER W EUR J PEDIATR 134 95 980 57 WIDNESS JA AM J OBSTET GYNECOL 136 1024 980 58 BROOKS AP BR MED J 281 707 980 59 OUGHTON J HORM METAB RES 11 112 981 60 LANTZ B HORM METAB RES 13 660 981 61 MONNIER LH DIABETOLOGIA 20 12 981 62 ISOGAI Y SCAND J CLIN LAB INVEST 41 171 981 63 JUHAN I SCAND J CLIN LAB INVEST 41 159 981 64 BROWNLEE M ANNU REV BIOCHEM 50 385 981 65 SCHULTZ TA AM J MED 70 373 981 66 GARNER A J ROY SOC MED 74 427 981 67 DITZEL J EUR J PEDIATR 137 171 981 68 GOEBEL FD RES EXP MED 179 133 981 69 MANSANI FE INT J BIOL RES PREGNANCY 3 180 982 70 OKADA Y DIABETOLOGIA 22 212 982 71 JUHANVAGUE I NOUV REV FR HEMATOL 24 191 982 72 ARNQVIST H SCAND J CLIN LAB INVEST 42 265 982 73 BACHEM MG KLIN WSCHR 60 497 982 74 CARNEY RM DIABETES CARE 6 378 983 75 JACQUES C PRESSE MED 12 673 983 76 WORTH R DIABETOLOGIA 25 482 983 77 NAKAYOSHI N JAP J OPHTHALMOL 27 255 983 78 KAMADA T DIABETES 32 585 983 79 CZECH A J CHRON DIS 36 803 983 80 GARLICK RL J CLIN INVEST 71 1062 983 81 MAYER TK CLIN CHIM ACTA 127 147 983 82 KAIBARA M INT J GYNAECOL OBSTET 22 221 984 83 GOMO ZAR ANN CLIN BIOCHEM 22 362 985 84 FEIGE A Z GEBURTSHILFE PERINATOL 189 13 985 85 PAGANO G CLIN HEMORHEOL 6 303 986 86 DURRUTY P REV MED CHIL 114 1123 986 87 STUART J CLIN HEMORHEOL 7 239 987 PN 76062 RN 00417 AN 76235423 AU Rossipal-E. Mlekusch-W. TI Steatorrhea, a simple and rapid method of diagnosis. SO Eur-J-Pediatr. 1976 Jul 12. 122(4). P 297-302. MJ CELIAC-DISEASE: di. MN ADOLESCENCE. CELIAC-DISEASE: di. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FECES: an. HUMAN. INFANT. LIPIDS: an. METHODS.PNEUMONIA: di. RESPIRATORY-TRACT-INFECTIONS: di. AB A simple and rapid method for the diagnosis of steatorrhea is described. The method is based on the assessment of the difference of specific gravity and lipophilic property between a normal stool and that in steatorrhea, using a benzene-NaBr system. The stool samples are dried, pulverized, and then investigated. Steatorrhea is defined either as a fat or more in a 24-hour sample. Stool samples of 71 infants and children were examined. Among these were 23 healthy subjects, 10 with upper respiratory tract infections, and 6 with pneumonia. A further 31 children had celiac disease, 9 of them were on a gluten-free diet, and one child had cystic fibrosis. The procedure described renders single stool samples suitable for the assessment of steatorrhea. As a screening test the method proved to be rapid and accurate in the diagnosis of steatorrhea. RF 001 VAN DE KAMER JH J BIOL CHEM 177 347 949 002 ROSSIPAL E KLIN PAEDIATR 184 385 972 003 SHMERLING DH PEDIATRICS 46 690 970 CT 1 BORKENSTEIN M PAEDIATR PAEDOL 13 145 978 PN 76063 RN 00418 AN 76235422 AU Emrich-H-M. Heitz-J. Katz-S. Thom-R. TI Cystic fibrosis: normal volumes of red cells. Model experiments on the pathomechanism of the disease. SO Eur-J-Pediatr. 1976 Jul 12. 122(4). P 293-6. MJ CYSTIC-FIBROSIS: bl. ERYTHROCYTES. MN CYSTIC-FIBROSIS: pp. ERYTHROCYTES: de. GRAMICIDINS: pd. HUMAN. ION-EXCHANGE. PERMEABILITY. POTASSIUM: ph. SODIUM: ph. SWEAT. VALINOMYCIN: pd. AB Normal values of mean cell volume (M.C.V.) and of distribution of single cell volumes (S.C.V.) have been observed in erythrocytes of patients with cystic fibrosis using an electronical particle-volume analyzer (sheath flow detector). From these results a strong defect in red cell salt transport seems improbable. Valinomucin induces shrinking (by increase of K+ permeability) and gramicidin D induces swelling (by increase of Na+ permeability) of normal erythrocytes. Addition of C.F. sweat to normal erythrocytes induces no volume change. From this result no influence of the "C.F. factor" on passive ion permeability is concluded. RF 001 BALFE JW SCIENCE 162 689 968 002 COLE CH PEDIATR RES 6 616 972 003 EMRICH HM Z NATURFORSCH 24B 1144 969 004 EMRICH HM PEDIATR RES 2 464 968 005 FEIG SA CLIN RES 22 234A 974 006 FEIG SA PEDIATR RES 8 594 974 007 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 008 KAISER D PEDIATR RES 5 167 971 009 MANGOS JA SCIENCE 158 135 967 010 PRESSMAN BC PROC NAT ACAD SCI USA 58 1949 967 011 DI SANTAGNESE PA PEDIATRICS 12 549 953 012 THOM R TELEFUNKEN HOCHFREQUENZ TECHN 972 CT 1 GALEY WR PEDIATR RES 14 1269 980 2 WARWICK WJ AM J MED TECHNOLOGY 48 539 982 PN 76064 RN 00419 AN 76235421 AU Nikolaejek-W-P. Emrich-H-M. TI ALPHA-Amylase activity in sweat and serum of patients with cystic fibrosis of the pancreas. SO Eur-J-Pediatr. 1976 Jul 12. 122(4). P 289-91. MJ AMYLASES: an. CYSTIC-FIBROSIS: en. SWEAT: en. MN ADOLESCENCE. AMYLASES: bl. CHILD. CHILD-PRESCHOOL. HUMAN. AB The hypothesis by Doggett and Harrison, according to which alpha- amylase is the pathogenic factor of the exocrinopathy in cystic fibrosis (C.F.), is investigated.No elevation of alpha-amylase in sweat and serum of C.F. patients, as compared with controls of similar age, is observed. It is concluded that the "C.F. factor" cannot be identified with alpha-amylase. RF 001 DOGGETT RG NATURE NEW BIOL 243 251 973 002 EMRICH HM PFLUEGERS ARCH 290 298 966 003 LOBECK CC IN: STANBURY JB 1605 972 004 MANGOS JA SCIENCE 158 135 967 005 MANGOS JA PEDIATR RES 2 378 968 006 MANGOS JA PEDIATR RES 1 436 967 007 RICHTERICH R KLINISCHE CHEMIE THEORIE UND 968 CT 1 TAKACS O ACTA PAEDIATR ACAD SCI HUNG 18 21 977 2 DAVIDSON GP PEDIATR RES 12 967 978 3 SCHRODER CP EUR J PEDIATR 128 49 978 4 CASOLA L CLIN CHIM ACTA 94 83 979 5 QUINTON PM ANNU REV MED 34 429 983 PN 76065 RN 00420 AN 76235415 AU Knopfle-G. Rottauwe-H-W. Lehmann-F-G. TI Serum alpha1-fetoprotein in cystic fibrosis. SO Eur-J-Pediatr. 1976 Jul 12. 122(4). P 241-8. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN ADULT. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MALE. RADIOIMMUNOASSAY. AB In 38 children and adolescents with cystic fibrosis, in 19 normal children and 62 healthy adults the serum alpha1-fetoprotein concentrations were measured by radioimmunoassay. In cystic fibrosis patients 97.5% and in normal children 95% of the alpha1-fetoprotein values were within the normal range for healthy adults (1--9 ng/ml). Critical judgement of the reported findings in literature and our own results demonstrate that the investigation of alpha1-fetoprotein in the serum cannot serve for detecting homozygotes of cystic fibrosis genes or heterozygote carriers. RF 001 GITLIN D J CLIN INVEST 45 1826 966 002 ADINOLFI M DEVELOP MED CHILD NEUROL 9 609 967 003 MARKLEIN G Z KINDERHEILK 113 327 972 004 SEPPALA M LANCET 1 375 972 005 VAN FURTH R NATURE 222 1296 969 006 PURVES LR S AFR MED J 46 1290 972 007 RUOSLAHTI E INT J CANCER 8 374 971 008 RUOSLAHTI E NATURE 235 161 972 009 MASSEYEFF R IN: MASSEYEFF R 313 974 010 SEPPALA M AM J OBSTET GYNECOL 112 208 972 011 ABELEV GI CANCER RES 28 1344 968 012 SMITH JB AM J MED 51 767 971 013 RUOSLAHTI E J NATL CANCER INST 49 623 972 014 GASSNER M SCHWEIZ MED WOCHENSCHR 102 465 972 015 LEHMANN FG KLIN WOCHENSCHR 49 609 971 016 RUOSLAHTI E SCAND J GASTROENTEROL 8 197 973 017 GEFFROY Y PRESSE MED 78 1107 970 018 MASOPUST J PROTIDES BIOL FLUIDS 18 239 971 019 LEHMANN FG INTERNIST (BERL) 13 332 972 020 KOHN J LANCET 2 334 974 021 ZELTZER PM LANCET 1 373 974 022 BELANGER L PATHOL BIOL (PARIS) 21 457 973 023 BELANGER L PATHOL BIOL (PARIS) 21 449 973 024 WALDMANN TA LANCET 2 1112 972 025 SELLER MJ BR MED J 2 524 974 026 BROCK DJH LANCET 2 197 972 027 ALLAN LD LANCET 2 522 973 028 HARRIS R LANCET 1 429 974 029 NEVIN NC LANCET 1 1383 973 030 KJESSLER B LANCET 1 432 975 031 SEPPALA M AM J OBSTET GYNECOL 115 48 973 032 CHANDRA RK BR MED J 1 714 975 033 SMITH JA BR MED J 2 392 975 034 WALLWORK JC BR MED J 2 392 975 035 BROCK DJH BR MED J 2 392 975 036 FITZSIMMONS JS BR MED J 3 544 975 037 BELANGER L BR MED J 4 759 975 038 KNOPFLE G BR MED J 4 459 975 039 NISHI S GANN MOGR CANCER RES 14 79 973 040 ISHII M GANN MOGR CANCER RES 14 89 973 041 LEHMANN FG CLIN CHIM ACTA 33 197 971 042 LEHMANN FG Z KLIN CHEM KLIN BIOCHEM 9 309 971 043 MANCINI G COLLOQ PROTIDES BIOL FLUIDS 11 370 965 044 SIZARET P J BIOL STANDARDS 3 201 975 045 LEHMANN FG Z KLIN CHEM KLIN BIOCHEM 11 339 973 046 SACHS L ANGEWANDTE STATISTIK 974 047 CRAIG JM AM J DIS CHILD 93 357 957 048$ FEIGELSON J PEDIATRIE 8 417 972 049 DI SANTAGNESE PA PEDIATRICS 18 387 956 050 ROBERTS WC AM J MED 32 324 962 051 SWIFT PN PROC R SOC MED 56 923 963 052 WEBSTER R ARCH DIS CHILD 28 343 953 CT 1 KNOPFLE G KLIN PAEDIATR 189 207 977 2 ADINOLFI M ADV HUM GENET 9 165 979 PN 76066 RN 00421 AN 76140667 AU Lebenthal-E. Antonowicz-I. Shwachman-H. TI Enterokinase and trypsin activities in pancreatic insufficiency and diseases of the small intestine. SO Gastroenterology. 1976 Apr. 70(4). P 508-12. MJ ENTEROPEPTIDASE: me. INTESTINAL-DISEASES: en. INTESTINAL-MUCOSA: en. INTESTINE-SMALL: en. PANCREATIC-DISEASES: en. PEPTIDE-PEPTIDOHYDROLASES: me. TRYPSIN: me. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. DIARRHEA: en. DISACCHARIDASES: df. FOOD-HYPERSENSITIVITY: en. HYPOPROTEINEMIA: en. INFANT. MALABSORPTION-SYNDROMES: en. MILK-PROTEINS: ae. STREPTOKINASE: df. TRYPSIN: df. AB The interrelationship of enterokinase and trypsin activities were investigated in 133 infants and children with a variety of gastrointestinal and pancreatic disorders. Fourteen patients with diarrhea and grade II mucosal injury revealed a significant (P less than 0.01) reduction of enterokinase, trypsin, and disaccharidase activites as compared to 59 children with normal mucosa. Nine patients with cystic fibrosis and pancreatic insufficiency had normal mucosal enterokinase activity and elevated intraluminal enterokinase activity with very low or no trypsin activity. Patients with hypoproteinemia and gastrointestinal protein loss, associated with intestinal lymphangiectasia (4 patients) and intestinal lymphoid nodular hyperplasia (3 patients), had normal or insignificant decrease of enterokinase and trypsin activities. In patients with steatorrhea, a normal sweat test, normal intestinal mucosa, and absent trypsin activity, two entities were defined. One group (3 patients) was diagnosed as Schwachman-Diamond syndrome with pancreatic insufficiency and normal mucosal and intraluminal enterokinase activity. The second group (2 patients) with absent mucosal and intraluminal enterokinase activity and normal lipase and amylase activities was diagnosed as congenital enterokinase deficiency. RF 001 KUNITZ M J GEN PHYSIOL 22 429 939 002 MAROUX S J BIOL CHEM 246 5031 971 003 NORDSTROM C BIOCHIM BIOPHYS ACTA 242 209 971 004 HADORN B LANCET 1 812 969 005 TARLOW MJ ARCH DIS CHILD 45 651 970 006 HADORN B SWED NUTR FOUND SYMP 11 107 973 007 NIESSEN KH KINDERHEILK 121 49 973 008 RUTGEERSTS L TIJDSCHR GASTROENTEROL 15 379 972 009 LEBENTHAL E PEDIATRICS 56 585 975 010 HADORN B GASTROENTEROLOGY 66 548 974 011 WOODLEY JF GUT 13 900 972 012 GOLDMAN AS PEDIATRICS 32 425 963 013 WALDMANN TA GASTROENTEROLOGY 50 422 966 014 ANTONOWICZ I PEDIATRICS 49 847 972 015 SHWACHMAN H J PEDIATR 65 645 964 016 TOWNLEY RRW PEDIATRICS 36 911 965 017 DAHLQVIST A ANAL BIOCHEM 7 18 964 018 LOWRY OH J BIOL CHEM 193 265 951 019 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 020 EGGERMONT E ACTA GASTROENTEROL BELG 34 655 971 021 NORDSTROM C LANCET 1 933 972 022 TAKANO K OKAJIMAS FOLIA ANAT JPN 48 15 971 CT 1 LEBENTHAL E CLIN GASTROENTEROL 6 397 977 2 LEBENTHAL E PEDIATR RES 11 282 977 3 HERMONTAYLOR J GUT 18 259 977 4 MORIN CL PEDIATRICS 60 114 977 5 ANTONOWICZ I GASTROENTEROLOGY 72 1299 977 6 RINDERKNECHT H AM J DIG DIS 23 327 978 7 FREEMAN HJ ANNU REV MED 29 99 978 8 KWONG WKL GASTROENTEROLOGY 74 1277 978 9 THEIL KW J AM VET MED ASSOC 173 548 978 10 SCHMIDT E CLIN BIOCHEM 12 247 979 11 SCHMIDT E J CLIN CHEM CLIN BIOCHEM 17 693 979 12 TSAGIKYAN TA VOPR MED KHIM 25 161 979 13 BERG NO ACTA PAEDIATR SCAND 68 275 979 14 HATCH TF J NUTR 109 1874 979 15 HERMAN RH WEST J MED 130 95 979 16 BURKE V J MED MICROBIOL 13 103 980 17 DOCKTER G MONATSSCHR KINDERHEILKD 128 732 980 18 NIESSEN KH MONATSSCHR KINDERHEILKD 128 301 980 19 LENTZE MJ LANCET 2 504 982 20 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 21 LEBENTHAL E DIGESTION 23 39 982 22 LENTZE MJ J PEDIATR GASTROENTEROL NUTR 2 S252 983 23 BOSSMANN B J CLIN CHEM CLIN BIOCHEM 21 1 983 24 LOJDA Z HISTOCHEMISTRY 78 251 983 25 GHISHAN FK GASTROENTEROLOGY 85 727 983 26 GREEN JR J PEDIATR GASTROENTEROL NUTR 3 630 984 27 KAI H J PEDIATR GASTROENTEROL NUTR 3 328 984 28 HJELM M CLIN BIOCHEM 17 284 984 29 BOSSMANN B ACTA HISTOCHEM 74 217 984 30 MANN NS GASTROENTEROLOGY 86 384 984 31 DOCKTER G MONATSSCHR KINDERHEILKD 132 589 984 32 CALVIN RT J PEDIATR GASTROENTEROL NUTR 4 949 985 33 NEWMAN BM AM J PHYSIOL 250 G103 986 PN 76067 RN 00422 AN 76165918 AU Stern-R-C. Stevens-D-P. Boat-T-F. Doershuk-C-F. Izant-R-J-Jr. Matthews-L-W. TI Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting. SO Gastroenterology. 1976 May. 70(5 PT.1). P 645-9. MJ CYSTIC-FIBROSIS: co. LIVER-DISEASES: et. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. ESOPHAGEAL-AND-GASTRIC-VARICES: et. FEMALE. HEMORRHAGE-GASTROINTESTINAL: et. HUMAN. HYPERSPLENISM: et. HYPERTENSION-PORTAL: co, et, su. INFANT. LIVER-DISEASES: oc, su. MALE. OHIO. PORTACAVAL-SHUNT-SURGICAL. SUPPORT-U-S-GOVT-P-H-S. AB Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18- year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension. RF 001 FARBER S ARCH PATHOL 37 238 944 002 WEBSTER R ARCH DIS CHILD 28 343 953 003 DI SANTAGNESE PA PEDIATRICS 18 387 956 004 CRAIG JM AM J DIS CHILD 93 357 957 005 SLOTH K ACTA PAEDIATR SCAND 61 380 972 006 ROBERTS WC AM J MED 32 324 962 007 OPPENHEIMER EH J PEDIATR 86 683 975 008 TYSON KRT J PEDIATR SURG 3 271 968 009 DANIELSON GK JAMA 195 217 966 010 GRAND RJ CLIN PEDIATR 9 588 970 011 WILROY RS JR J PEDIATR 68 67 966 012 VALMAN HB ARCH DIS CHILD 46 805 971 013 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 014 KATTWINKEL J J PEDIATR 82 234 973 015 BOAT TF CLIN PEDIATR 13 505 974 016 RESNICK RH GASTROENTEROLOGY 67 843 974 017 CONN HO GASTROENTEROLOGY 67 1065 974 018 KOPEL FB GASTROENTEROLOGY 62 483 972 019 DALE DC J INFECT DIS SUPPL 130 143 974 020 ZIEGLER EJ J INFECT DIS SUPPL 130 145 974 CT 1 STERN RC J PEDIATR 89 406 976 2 SCOTT J AM J MED 63 488 977 3 STERN RC ANN INTERN MED 87 188 977 4 ROY CC N ENGL J MED 297 1301 977 5 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 6 SANTAGNESE PAD AM J MED 66 121 979 7 WOOD RE SOUTH MED J 72 189 979 8 VANVROONHOVEN TJ SURG GYNECOL OBSTET 149 559 979 9 CUNNINGHAM DG J COMPUT ASSIST TOMOGR 4 151 980 10 ARBORGH B SCAND J GASTROENTEROL 15 73 980 11 MIELIVERGANI G ARCH DIS CHILD 55 696 980 12 STERN RC AM J DIS CHILD 134 267 980 13 PSACHAROPOULOS HT LANCET 2 78 981 14 LAMBERT JR GASTROENTEROLOGY 80 169 981 15 PARK RW GASTROENTEROLOGY 81 1143 981 16 CLARKE M BR MED J 283 1263 981 17 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 18 STERN RC LANCET 1 1401 982 19 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 20 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 21 DOHERTY DE SOUTH MED J 76 1580 983 22 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 23 HUBBARD VS SEM RESPIR MED 6 299 985 24 BERNARD O CLIN GASTROENTEROL 14 33 985 25 SCHRODER R SCHWEIZ MED WOCHENSCHR 115 828 985 26 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 27 HULTCRANTZ R HEPATOLOGY 6 881 986 28 FEIST D WIEN KLIN WOCHENSCHR 98 547 986 29 KUMARISUBAIYA S J PEDIATR GASTROENTEROL NUTR 6 71 987 30 VARMA RR SEM LIVER DIS 7 59 987 PN 76068 RN 00423 AN 76188462 AU Javitt-N-B. TI Cholestasis in infancy. Status report and conceptual approach. SO Gastroenterology. 1976 Jun. 70(6). P 1172-81. MJ CHOLESTASIS: th. MN ALPHA-1-ANTITRYPSIN: df. ANION-EXCHANGE-RESINS: tu. BACTERIAL-INFECTIONS: co. BILE-DUCTS: ab. BILE-DUCTS: su. BILE-DUCTS-INTRAHEPATIC: pp. BILE: se. CHOLESTASIS: pa, pp, su. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. PHENOBARBITAL: tu. RECURRENCE. SUPPORT-U-S-GOVT-P-H-S. EX This review is concerned with the disturbances in bile flow that occur early in life. Although cholestasis is defined physiologically, it can be considered to have morphological features. Morphological cholestasis is the presence of bile pigments in histopathological sections of the liver and is most easily recognized as bile plugs or thrombi in bile canaliculi. Physiological cholestasis, a reduction in bile flow, is inferred from the finding of an elevated serum bile acid. This inference is derived from the known positive correlation between canalicular bile flow and bile acid excretion that has been found to occur in all species this far examined, including man. An increase in the amount of bile acid in plasma implies a reduction in the hepatic transport of the primary conjugated bile acids of man and therefore a decrease in associated canalicular bile flow. Classifications of cholestatic syndromes, functional abnormalities, and therapy for cholestasis syndromes are discussed. RF 001 SCHEUER P LIVER BIOPSY INTERPRETATION 22 973 002 SCHERSTEN T EUR J CLIN INVEST 1 242 971 003 KAPLOWITZ N AM J MED 54 725 973 004 JAVITT NB PEDIATR RES 7 119 973 005 SJOVALL K CLIN CHIM ACTA 13 207 966 006 SANDBERG DH PEDIATR RES 4 262 970 007 SANDBERG DH J LIPID RES 6 182 965 008 ALI SS CAN J BIOCHEM 48 1054 970 009 SCHWARZ HP CLIN CHIM ACTA 50 197 974 010 WATKINS JB N ENGL J MED 288 431 973 011 NORMAN A ACTA PAEDIATR SCAND 62 161 973 012 LANDING BH IN: GALL EA 480 972 013 ROLLESTON HD BR MED J 1 758 901 014 ODELL GB IN: JAMES AE JR 290 974 015 LANDING BH IN: BECKER FF 821 975 016 CRAIG JM ARCH PATHOL 54 321 952 017 STRAUSS L ARCH PATHOL 86 317 968 018 ALPERT LI N ENGL J MED 280 16 969 019 BROUGH AJ PEDIATRICS 43 519 969 020 BROUGH AJ HUM PATHOL 5 507 974 022 JAVITT NB POSTGRAD MED J 50 354 974 023 YEUNG CY PEDIATRICS 50 812 972 024 JAVITT N IN: GORESKY CA 401 975 025 SHARP HL PEDIATR RES 5 274 971 026 BACK P HOPPE SEYLERS Z PHYSIOL CHEM 354 83 973 027 LAVY U GASTROENTEROLOGY 65 556 973 028 JAVITT NB IN: PAUMGARTNER G 355 973 029 MAKINO I LIPIDS 8 47 973 030 STIEHL A EUR J CLIN INVEST 4 59 974 031 RUDMAN D J CLIN INVEST 36 530 957 032 CAREY JB JR J CLIN INVEST 37 1494 958 033 WOOTON IDP LANCET 2 1049 959 033 JAVITT NB IN: MATERN S 123 974 034 LILLY JR SURGERY 75 664 974 035 LEES W ARCH DIS CHILD 41 188 966 036 LEE SS ARCH SURG 99 19 969 037 WATTS DR ARCH SURG 108 592 974 038 SHARP HL J PEDIATR 71 723 967 039 AHRENS EH JR PEDIATRICS 8 628 951 040 JAVITT NB IN: SCHIFF L 111 975 041 GUTSTEIN S ISR J MED SCI 4 36 968 042 CLAYTON RJ AM J DIS CHILD 117 112 969 043 LINARELLI LG J PEDIATR 81 484 972 044 JUBERG RC PEDIATRICS 38 819 966 045 WATSON GH ARCH DIS CHILD 48 459 973 046 ALAGILLE D J PEDIATR 86 63 975 047 MILLARD M J MAINE MED ASSOC 62 243 971 048 EYSSEN H BIOCHIM BIOPHYS ACTA 273 212 972 049 WEBER A PEDIATRICS 50 73 972 050 CAREY JB JR J BIOL CHEM 238 855 963 051 NORMAN A ACTA PAEDIATR SCAND 58 59 969 052 NORMAN A ACTA PAEDIATR SCAND 62 264 973 053 HAAS L ARCH DIS CHILD 43 438 968 054 HAMBOURG M ANN PEDIATR (PARIS) 18 695 971 055 LUDERS D MONATSSCHR KINDERHEILKD 121 717 973 056 TYGSTRUP N ACTA MED SCAND 185 523 969 057 AAGENAES O ARCH DIS CHILD 43 646 968 058 SIGSTAD H ACTA MED SCAND 188 213 970 059 SHARP HL J PEDIATR 78 491 971 060 DANKS DM AUST PAEDIATR J 1 193 965 061 ESCOBEDO MB CLIN PEDIATR 13 656 974 062 SEELER RA ILL MED J 143 347 973 063 JEAN R ARCH FR PEDIATR 27 849 970 064 VALMAN HB ARCH DIS CHILD 46 805 971 065$ TAYLOR WF AM J DIS CHILD 46 805 971 066 OPPENHEIMER EH J PEDIATR 86 683 975 067 REID IS ARCH DIS CHILD 48 952 973 068 PORTO SD AM J DIS CHILD 117 684 969 069 LEVINE G ARCH PATHOL 95 267 973 070 AKERS DR SURGERY 71 817 972 071 HSIA DYY METABOLISM 16 419 967 072 SCRIVER CR AM J DIS CHILD 113 41 967 073 YU JS ARCH DIS CHILD 46 306 971 074 SHARP HL J LAB CLIN MED 73 934 969 075 AAGENAES O ACTA PAEDIATR SCAND 61 632 972 075 JOHNSON AM IN: BECKER FF 974 076 KATTAMIS C PEDIATRICS 54 157 974 077 KATTAMIS C ARCH DIS CHILD 48 133 973 078 GILLESPIE A LANCET 2 1081 970 079 SCHWEITZER IL GASTROENTEROLOGY 65 277 973 080 TOLENTINO P LANCET 1 398 971 081 VASSELLA F AM J DIS CHILD 123 300 972 082 ALTSCHULER G LANCET 2 1206 973 083 PORTER CA BR MED J 3 435 972 084 ZELTZER PM LANCET 1 373 974 085 MORRISSEY KP SURGERY 74 116 973 086 SHARP HL J PEDIATR 70 46 967 087 MAKSOUD JG PEDIATRICS 48 966 971 088$ POLEY JR J LAB CLIN MED 3 325 973 089 POLEY JR J PEDIATR SURG 7 660 972 090 HARDISON WG J LAB CLIN MED 77 811 971 091 PAUMGARTNER G BULL NY ACAD MED 51 455 970 092 GREIM H GASTROENTEROLOGY 63 846 972 093 EINARSSON K CLIN SCI MOL MED 46 183 974 094 HARKAVY M IN: SALHANIK HA 11 969 095 LOTTSFELDT FI N ENGL J MED 269 186 963 096 WEST RJ ARCH DIS CHILD 48 370 973 097 SHARP HL IN: GELLIS SS 224 973 098 COHEN BD N ENGL J MED 280 1362 969 099 ERLINGER S GASTROENTEROLOGY 66 281 974 100 MACAROL V J CLIN INVEST 49 1714 970 101 BARNHART J IN: PAUMGARTNER G 315 973 102 STIEHL A N ENGL J MED 286 858 972 103 SHARP HL J PEDIATR 81 116 972 104 KROVETZ LJ SURGERY 47 468 960 105 KROVETZ LJ SURGERY 47 453 960 106 HAYES DM J PEDIATR 71 598 967 107 LLANA-NAVAINO R REV MED CHIR MAL FOIE 46 137 971 108 THALER MM AM J DIS CHILD 116 262 968 109 RAVITCH MM MED TIMES 95 586 967 110 FONKALSRUD EW AM SURGEON 37 389 971 111 KOOP CE J PEDIATR SURG 10 169 975 112 LILLY J PEDIATRICS 55 12 975 113 KASAI M J PEDIATR SURG 10 173 975 114 GARDNER LI N ENGL J MED 290 406 974 115 CAMPBELL DP J PEDIATR SURG 9 329 974 116 MIYADA M SURGERY 76 234 974 117 STERLING JA INT SURG 46 228 966 118 ZACHERY RB PRACTITIONER 210 627 973 119 DE LORIMIER AA N ENGL J MED 288 1284 973 120 HAYES DM SURG CLIN NORTH AM 53 1257 973 121 ALAGILLE D AM J DIS CHILD 123 287 972 122 LANDING BH PROG PEDIATR SURG 6 113 974 CT 1 RODGERS BM GASTROENTEROLOGY 71 710 976 2 UTILI R GASTROENTEROLOGY 71 1109 976 3 JAVITT NB CLIN GASTROENTEROL 6 219 977 4 ROY CC CLIN GASTROENTEROL 6 377 977 5 WATKINS JB CLIN GASTROENTEROL 6 201 977 6 DELEZE G HELV PAEDIATR ACTA 32 29 977 7 LLOYDSTILL JD PEDIATRICS 59 626 977 8 CHIPRUT R GASTROENTEROLOGY 72 772 977 9 MATHIS RK J PEDIATR 90 864 977 10 SCHMEISSER W SCHWEIZ MED WOCHENSCHR 107 1613 977 11 EVANS J BR J DERMATOL 98 469 978 12 ABERNATHY CO MED HYPOTHESES 5 641 979 13 CALANDI C MINERVA PEDIATR 31 1357 979 14 JAVITT NB POSTGRAD MED 65 120 979 15 GOLDBERG DM AM J CLIN PATHOL 71 557 979 16 LLOYDSTILL JD AM J CLIN PATHOL 71 444 979 17 MANGINELLO FP J PEDIATR 94 296 979 18 BRANSKI D J CLIN GASTROENTEROL 2 251 980 19 KAPLINSKY C PEDIATRICS 65 782 980 20 DOCKTER G MONATSSCHR KINDERHEILKD 128 326 980 21 ROTTHAUWE HW MONATSSCHR KINDERHEILKD 128 284 980 22 FRANKEN P ZENTRALBL VETERINARMED (A) 28 276 981 23 FINNI K BIOL NEONATE 40 264 981 24 BARNES S J CLIN INVEST 68 775 981 25 KOCOSHIS SA J PEDIATR 99 436 981 26 FINNI K EUR J PEDIATR 138 53 982 27 SPIVAK W J PEDIATR GASTROENTEROL NUTR 2 381 983 28 WATKINS JB J PEDIATR GASTROENTEROL NUTR 2 365 983 29 GANGL A ACTA MED AUSTRIACA 10 107 983 30 TUCHWEBER B TOXICOL LETT 19 107 983 31 WATSON S CLIN PEDIATR 22 30 983 32 MATHIS U GASTROENTEROLOGY 85 674 983 33 LEWITTES M HEPATOLOGY 4 486 984 34 WALSHE JM SEM LIVER DIS 4 252 984 35 WEAVER LT ACTA PAEDIATR SCAND 73 155 984 36 BECKER M EUR J PEDIATR 143 41 984 37 BOHLES H KLIN PAEDIATR 198 435 986 38 BOHLES H INFUSIONSTHER KLIN ERNAHR 14 3 987 39 COX KL PEDIATR RES 21 170 987 PN 76069 RN 00424 AN 76188872 AU Weber-A-M. Roy-C-C. Chartrand-L. Lepage-G. Dufour-O-L. Morin-C-L. Lasalle-R. TI Relationship between bile acid malabsorption and pancreatic insufficiency in cystic fibrosis. SO Gut. 1976 Apr. 17(4). P 295-9. (REVIEW). MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: pp. MALABSORPTION-SYNDROMES: me. PANCREAS: pp. MN ADOLESCENCE. ADULT. BICARBONATES: pd. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: co, me. FECES: an. HUMAN. INFANT. INTESTINAL-ABSORPTION: de. LIPIDS: me. REVIEW. TRIGLYCERIDES: pd. AB Bile acid loss (mg/m2 24h) in the stools of 43 cystic fibrosis (CF) children with pancreatic insufficiency was 751-1 +/- 48-3, while that of six without clinical evidence of pancreatic disease (133-4 +/- 15- 9) did not differ from values in 25 controls (109-8 +/- 9-8). There was a good correlation between the degree of bile acid (BA) and fat sequestration. Concomitant changes in bile acid and fat loss were observed in the one group of six patients studied on and off pancreatic enzymes as well as in a second group of seven children treated with pancreatic supplements and maintained on a normal diet followed by a low fat diet supplemented with medium chain triglycerides. Administration of NA bicarbonate led to a significant decrease in fat loss (15-8 +/- 2-7 leads to 10-3 +/- 1-9) without any simultaneous change in bile acid excretion (533-1 +/- 58-3 leads to 500-4 +/- 58-6). Qualitative bile acid patterns in controls, in infants after an ileal resection, and in patients with CF or with coeliac disease showed that the percentage of primary BA followed closely the total amount excreted except in situations where antibiotics were administered. The exact mechanism for the increased loss of BA in CF is unknown. It is found in all age groups and is related to the presence and degree of pancreatic insufficiency. The possibility that unhydrolysed triglycerides may interfere with the intestinal absorption of bile acid needs further confirmation. RF 001 AMMON HV J CLIN INVEST 53 205 974 002 ANNEGERS JH ARCH INTERN MED 93 9 954 003 BONIN A J PEDIATR 83 594 973 004 BORGSTROM B J LIPID RES 8 598 967 005 EASTWOOD MA GUT 13 845 972 006 FIASSE R GASTROENTERO 3RD ANN CONG ABS 974 007 FLOCH MH GASTROENTEROLOGY 61 228 971 008 GO VLW GASTROENTEROLOGY 58 638 970 009 GRACEY M ARCH DIS CHILD 45 445 970 010 HARO EN CLIN RES 12 207 964 011 HEIZER WD BULL JOHNS HOPKINS HOSP 116 261 965 012 HOFMANN AF GASTROENTEROLOGY 62 918 972 013 HOLT PR DISEASE A MONTH 6 19 971 014 KATTWINKEL J PEDIATRICS 50 133 972 015 KUO PT J CLIN INVEST 44 1924 965 016 MALLORY A GASTROENTEROLOGY 64 26 973 017 REDINGER RN GASTROENTEROLOGY 64 610 973 018 RICOUR C ARCH FR PEDIATR 29 668 972 019 VAN DE KAMER JH J BIOL CHEM 177 347 949 020 VEEGER W N ENGL J MED 267 1341 962 021 WATKINS JB GASTROENTEROLOGY 67 835 974 022 WEBER AM CLIN CHIM ACTA 39 524 972 023 WEBER AM GASTROENTEROLOGY 68 1066 975 024 WEBER AM N ENGL J MED 289 1001 973 CT 1 ROY CC CLIN GASTROENTEROL 6 377 977 2 AURICCHIO S PEDIATR RES 11 997 977 3 LEFEBVRE D EXPERIENTIA 33 616 977 4 LIPSON AH ARCH DIS CHILD 52 428 977 5 SCOTT J AM J MED 63 488 977 6 ROY CC GASTROENTEROLOGY 72 1123 977 7 KERN F GASTROENTEROLOGY 73 631 977 8 ROY CC GASTROENTEROLOGY 73 631 977 9 WATKINS JB GASTROENTEROLOGY 73 1023 977 10 ROY CC N ENGL J MED 297 1301 977 11 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 12 SMALLEY CA ARCH DIS CHILD 53 477 978 13 CUMMINGS JH J CLIN INVEST 61 953 978 14 STARKEY BJ MONOGR PAEDIATR 10 12 979 15 ROY CC LIPIDS 14 811 979 16 LOESCHKE K INTERNIST 20 369 979 17 DUTTA SK DIG DIS SCI 24 529 979 18 ROY CC AM J CLIN NUTR 32 2404 979 19 HARRIES JT ARCH DIS CHILD 54 19 979 20 LLOYDSTILL JD AM J CLIN PATHOL 71 444 979 21 REGAN PT GASTROENTEROLOGY 77 285 979 22 MISCHLER EH AM J DIS CHILD 133 632 979 23 ROY CC PROC SOC EXP BIOL MED 161 105 979 24 ROULET M PEDIATR RES 14 1360 980 25 HUBBARD VS AM J CLIN NUTR 33 2281 980 26 GORIUP U HELV PAEDIATR ACTA 35 177 980 27 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 28 BOYLE BJ GASTROENTEROLOGY 78 950 980 29 BALISTRERI WF J PEDIATR 96 582 980 30 PARK RW GASTROENTEROLOGY 81 1143 981 31 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 32 COLOMBO C RIV ITAL PEDIATR 8 77 982 33 FONDACARO JD PEDIATR RES 16 494 982 34 GRAHAM DY DIG DIS SCI 27 485 982 35 HEUBI JE GASTROENTEROLOGY 83 804 982 36 HEUBI JE GASTROENTEROLOGY 83 1156 982 37 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 38 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 39 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 40 COLOMBO C DIG DIS SCI 28 306 983 41 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 42 GOTZ M ATEMWEGS LUNGENKRANKH 10 594 984 43 ZENTLERMUNRO PL GUT 25 500 984 44 PERRY RS CLIN PHARMACY 4 161 985 45 ROBB TA GUT 26 1246 985 46 ZENTLERMUNRO PL GUT 26 892 985 47 SETCHELL KDR CLIN CHIM ACTA 151 101 985 48 SINAASAPPEL M ACTA PAEDIATR SCAND SUPPL 317 1985 22 985 49 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 50 WEIZMAN Z GUT 27 1043 986 51 LEROY C DIG DIS SCI 31 911 986 52 DUTTA SK GASTROENTEROLOGY 91 1243 986 PN 76070 RN 00425 AN 76236387 AU Desai-L-S. Lazarus-H. Morrissey-G-W. Wulff-U-C. Foley-G-E. Shwachman-H. TI Differences in the frequency distribution of DNA and dry mass per cell in lymphocytes derived from cystic fibrosis patients. SO Haematologica (Pavia). 1976 Jun. 61(2). P 157-69. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. DNA: an. LYMPHOCYTES: an. MN ADOLESCENCE. ADULT. CELL-LINE. CHILD. FEMALE. HUMAN. KARYOTYPING. MALE. MIDDLE-AGE. SUPPORT-U-S-GOVT-P-H-S. AB The DNA and dry mass content of lymphocytes of normal controls, heterozygotic and cystic fibrosis patients have been studied utilizing biophysical instrumentation. The lymphocytes from CF had Feulgen-DNA mean values significantly lower than the normal controls. DNA values for CF lymphocytes exhibited two sub-population values. The mean dry mass (total protein) values exhibited greater intercellular variability among CF cells. The dry mass:DNA ratio was higher in CF than in normal controls, and this ratio appeared to increase concomitantly with advance of disease. RF 001 ANDERSEN DH ANN NY ACAD SCI 93 500 962 002 ANTONOWICZ I PEDIATR RES 6 803 972 003 ATKIN NB BR J CANCER 13 773 959 004 ATKIN NB BR J CANCER 13 773 959 005 BLUME RS BLOOD 33 821 969 006 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 007 BURZYNSKI NJ J DENT RES 52 164 973 008 CASPERSSON T EXP CELL RES 28 621 962 009 CASPERSSON T EXP CELL RES 32 553 963 010 CHOI KW SCIENCE 170 89 970 011 CONOVER JH PEDIATR RES 7 224 973 012 DANES BS J EXP MED 129 775 969 013 DANES BS J EXP MED 123 1 966 014 DANES BS J EXP MED 124 1181 966 015 GARCIA AM IN: WIED GL 2 966 016 DESAI LS CHROMOSOMA 38 329 972 017 EAGLE H SCIENCE 130 432 959 018 FOLEY GE PROC SYMP FUND CANC RES 21ST 65 968 019 FRANZER CS EXP CELL RES 4 316 953 020 GAHRTON G CANCER RES 29 1076 969 021 DEITCH AD IN: WIED GL 2 966 022 INUI N CYTOLOGIA 35 280 970 023 LAZARUS H IN: ROTHBLAT GH 2 170 972 024 LEUCHTENBERGER C IN: DANIELLI JF 1 219 968 025 LOMAKKA G ACTA HISTOCHEM SUPPL 6 47 965 026 LOMAKKA G ACTA HISTOCHEM SUPPL 6 393 965 027 MCCARTHY RE EXP CELL RES 43 564 966 028 OBRIEN JS FED PROC 30 956 971 029 SHEETER S BIRTH DEF ORIG ART SER 9 138 973 030 SHWACHMAN H PEDIATRICS 36 689 965 031 SNEDECOR GW STATISTICAL METHODS 967 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 76071 RN 00426 AN 76237304 AU Schaub-J. Remberger-K. Endres-W. Bremer-H-J. TI Galactosemia with endogenous production of galactose-1-phosphate and with cystic fibrosis-like appearance at autopsy. SO Helv-Paediatr-Acta. 1976 Jun. 31(1). P 67-76. MJ CYSTIC-FIBROSIS: co. GALACTOSEMIA: bl. LIVER-CIRRHOSIS: co. MN AUTOPSY. CASE-REPORT. CYSTIC-FIBROSIS: pa. ERYTHROCYTES: me. GALACTOSEMIA: co, dh. HUMAN. INFANT. JEJUNUM: pa. LIVER-CIRRHOSIS: pa. LIVER: pa. MALE. PANCREAS: pa. AB In an infant with galactosemia high levels of galactose-1-phosphate in red blood cells and of blood galactose were observed under a "galactose-free'' diet. The child did not thrive and developed a liver cirrhosis. At the age of 5 months he died unexpectedly. Post mortem examination revealed in the pancreas and the small intestine changes suggestive of a cystic fibrosis. Since the exogenous administration of galactose by diet could be excluded the endogenous production of significant amounts of galactose-1-phosphate has to be considered. RF 001 BREMER HJ HELV PAEDIATR ACTA 27 525 972 002 BRODEHL J BEIHEFT ZUM ARCHIV FUR KINDER 969 003 DONNELL GN BIOCHEM MED 1 29 967 004 GITZELMANN R PEDIATRICS 36 231 965 005 GITZELMANN R PEDIATR RES 3 279 969 006 GITZELMANN R BIOCHIM BIOPHYS ACTA 372 374 974 007 GITZELMANN R IN: HOMMES FA 25 975 009 HARRIS RC PEDIATR RES ABST 78 1 220 967 010 HSIA DYY GALACTOSEMIA 969 011 KALCKAR HM BIOCHIM BIOPHYS ACTA 20 262 956 012 KAPLAN E N ENGL J MED 279 65 968 013 KIRKMAN HN J LAB CLIN MED 56 161 960 014 KISSANE JM PATHOLOGY OF INFANCY AND CHIL 320 975 015 POTTER EL PATHOLOGY OF FETUS AND INFANT 142 962 016 PROSSER R ARCH DIS CHILD 49 597 974 017 SONNINO S J BIOL CHEM 241 1009 966 018 SPACKMAN DH ANAL CHEM 30 1190 958 019 TSUBOI KK J BIOL CHEM 244 1008 969 020 ZIMMERMAN HM PROGRESS IN NEUROPATHOLOGY 973 PN 76072 RN 00427 AN 77206026 AU Wood-R-E. TI Pseudomonas: the compromised host. SO Hosp-Pract. 1976 Aug. 11(8). P 91-100. MJ PSEUDOMONAS-INFECTIONS. MN ANIMAL. BLOOD-TRANSFUSION. CHILD. CYSTIC-FIBROSIS: co. DOGS. GRANULOCYTES: tr. CROSS-INFECTION. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES. INFANT-PREMATURE. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: co, th. RESPIRATORY-TRACT-INFECTIONS. SKIN-DISEASES-INFECTIOUS. TRANSPLANTATION-HOMOLOGOUS. AB Although the incidence of pseudomonas infection is low in the total hospital population, certain conditions predispose to such infection, which can be life-threatening. Patients with cystic fibrosis are a special group, since a majority have chronic pulmonary infections with pseudomonas, often of a mucoid variant rarely found in other patients. Several approaches are being tried in CF patients, but none shows much promise thus far. RF 000 FARMER JJ 3RD HOSP PRACT 11 63 976 000 LIU PV HOSP PRACT 11 139 976 001 REYNOLDS HY ANN INTERN MED 82 819 975 002 YOUNG LS CRC CRIT REV CLIN LAB SCI 3 291 972 003 MOREHEAD CD AM J DIS CHILD 124 564 972 004 HUANG NN J PEDIATR 59 512 961 005 PENNINGTON JE AM J MED 55 155 973 006 BOXERBAUM B AM REV RESPIR DIS 108 777 973 007 HALL JH ARCH DERMATOL 97 312 968 CT 1 THOMASSEN MJ PEDIATR RES 13 1085 979 2 WOOD RE SOUTH MED J 72 189 979 3 THOMASSEN MJ PEDIATR RES 14 715 980 4 THOMASSEN MJ INFECT IMMUN 33 512 981 5 SORDELLI DO CLIN IMMUNOL IMMUNOPATHOL 22 153 982 6 THOMASSEN MJ INFECT IMMUN 38 802 982 7 WINNIE GB INFECT IMMUN 38 1088 982 8 OFFREDOHEMMER C ANN MICROBIOL (PARIS) A134 281 983 9 WOODS DE REV INFECT DIS 5 S715 983 10 SCHILLER NL PEDIATR RES 17 747 983 11 SORENSEN RU EUR J RESPIR DIS 64 524 983 12 KEREN G J INFECT 9 22 984 13 NICAS TI CAN J MICROBIOL 31 387 985 14 NICAS TI J CLIN MICROBIOL 23 967 986 15 WOODS DE J CLIN MICROBIOL 24 260 986 16 SOKOL PA INFECT IMMUN 51 896 986 PN 76073 RN 00428 AN 77206023 AU Davidson-M. TI GI problems in children: I. SO Hosp-Pract. 1976 Aug. 11(8). P 47-55. MJ INTESTINAL-DISEASES: di, th. MN CELIAC-DISEASE: di, th. CHILD. CHILD-PRESCHOOL. COLITIS-ULCERATIVE: di, th. CYSTIC-FIBROSIS: di, th. DISACCHARIDASES: df. CROHN-DISEASE: di, th. HUMAN. INFANT. INFANT-NEWBORN. INFANT-PREMATURE. LYMPHANGIECTASIS-INTESTINAL: di, th. AB The first of two articles focuses on a number of malabsorptive disorders and on inflammatory bowel diseases as causes of persistent diarrhea. Diagnosis and treatment of the different entities are detailed. RF 001 DAVIDSON M IN: BARNETT HL 972 002 SILVERBERG M IN: BARNETT HL 972 003 DAVIDSON M IN: BARNETT HL 972 004 ANDERSON CM PEDIATRIC GASTROENTEROLOGY 975 005 DAVIDSON M J PEDIATR 66 545 965 006 SHWACHMAN H IN: SLEISENGER MH 1206 973 007 DAVIDSON M IN: SLEISENGER MH 973 008 DAVIDSON M J PEDIATR 69 1027 966 009 SHINER M LANCET 1 136 975 PN 76074 RN 00429 AN 76166525 AU Bedrossian-C-W. Greenberg-S-D. Singer-D-B. Hansen-J-J. Rosenberg-H-S. TI The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. SO Hum-Pathol. 1976 Mar. 7(2). P 195-204. MJ CYSTIC-FIBROSIS: pa. LUNG: pa. MN ADOLESCENCE. ADULT. AGE-FACTORS. BRONCHI: pa. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. MUCOUS-MEMBRANE: pa. PULMONARY-EMPHYSEMA: pa. AB The autopsies of 82 patients with cystic fibrosis were reviewed with respect to pathologic changes in the lungs and their respective prevalence among different age groups. Although bronchitis, mucopurulent plugging, and bronchopneumonia were almost universally present among children of all ages, epithelial metaplasia and bronchiectasis were rarer among infants and progressively more prevalent in older age groups. Emphysema was absent in patients under two years of age and affected 11 per cent of the patients two to six years of age and 40 per cent of the patients older than six years, but was never of a severe degree by the point count method. Pulmonary hemorrhage, although uncommon, was usually associated with prominent arterial vessels in walls of bronchiectatic airways. Quantitative assessment of bronchial glands revealed Reid indices significantly higher in patients with cystic fibrosis when compared to noncystic fibrosis patients, but there was no increase in these indices with the age of the patients. Glandular hypertrophy, predominance of mucous acini within glands, and goblet cell hyperplasia of the bronchial mucosa all suggest an explanation for the copious mucous secretion of patients with cystic fibrosis. However, it was not possible to ascertain whether these findings reflect a general exocrine defect of such patients or whether they were merely a response to chronic airway infection, even though the latter is a more plausible assumption. RF 001 SHWACHMAN H PEDIATRICS 36 689 965 002 TALAMO RC MED TIMES 97 139 969 003 ADDINGTON WW CHEST 59 306 971 004 REID L MOD PROBL PEDIATR 10 195 967 005 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 006 GREENBERG SD AM J CLIN PATHOL 41 658 964 007 REID L THORAX 15 132 960 008 FIELD WEH BR J DIS CHEST 62 11 968 009 WENTWORTH P THORAX 23 582 968 010 MATSUBA K AM REV RESPIR DIS 105 908 972 011 THURLBECK WM THORAX 19 436 964 012 ESTERLY JR THORAX 23 670 968 014 AUERBACH O AM REV RESPIR DIS 82 640 960 015 DI SANTAGNESE PA N ENGL J MED 277 1399 967 016 LEVITSKY S JAMA 213 125 970 017 SYMCHYCH PS ARCH PATHOL 92 409 971 018 GOLDRING RM J PEDIATR 65 501 964 PN 76076 RN 00430 AN 76215350 AU Benke-P-J. TI Biochemistry of cystic fibrosis: an overview. pp. 157-64. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. MJ CYSTIC-FIBROSIS: me. MN CELLS-CULTURED. CYSTIC-FIBROSIS: bl. GLYCOPROTEINS: me. IGG. MONOGRAPH. MUCOPOLYSACCHARIDES: me. SODIUM: me. SUPPORT-U-S-GOVT-NON-P-H-S. EX Cystic fibrosis has been a difficult problem to solve. One amino acid is not found in elevated concentrations in the urine, one abnormal sugar or polysaccharide is not found in secretions of the bronchial tree which causes pulmonary damage, and a unique material which inhibits sodium transport has not yet been identified in sweat. Instead of viewing the current state of CF research as chaotic, perhaps we should say in a positive way that the diversity of ideas stimulates thinking, and in the process of sorting out the primary defect, a wide spectrum of pathophysiologic changes will be recognized. Recently a positive-charged polypeptide associated with immunoglobulin G has been identified which may be responsible for the ciliary dyskinesis effect of CF serum. This material should demonstrate the same properties when bound to immunoglobulin G from control sera. It would be interesting if it were identical with the basic protein recently found only in the serum and IgG fraction of CF patients and carriers. Further work may identify how the polypeptide affects cell membrane surfaces, and whether a defective peptidase, perhaps an arginine esterase, fails to break down the polypeptide. A unifying hypothesis could then emerge and tie biochemical and physiological findings together. RF 001 FITZPATRICK DF NATURE NEW BIOL 235 173 972 002 MCEVOY FA CLIN CHIM ACTA 54 195 974 004 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 005 GIBBS GE SCIENCE 167 993 970 006 MARCHI AG HELV PAEDIATR ACTA 28 427 973 007 BENKE PJ N ENGL J MED 284 731 971 008 KRAUS I PEDIATRICS 47 1010 971 009 ROSENLUND ML NATURE 251 719 974 010 KUO PT J CLIN INVEST 44 1924 965 011 EMERY AEH LANCET 2 80 975 012 MCEVOY FA LANCET 2 236 975 013 FITZPATRICK DF NATURE NEW BIOL 242 113 973 014 DUFFY MJ NATURE NEW BIOL 246 151 973 015 SMITH QT NATURE NEW BIOL 240 56 972 016 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 017 COHEN LF PEDIATR RES 9 312 975 018 MCEVOY FA PEDIATR RES 9 721 975 019 CAMPBELL RA CLIN RES 23 144A 975 020 RENNERT OM CLIN PEDIATR 11 351 972 021 RENNERT OM PEDIATRICS 50 485 972 022 KLAGSBRUN M PEDIATR RES 8 205 974 023 HODES ME PEDIATR RES 8 212 974 024 BALFE JW SCIENCE 162 689 968 025 LAPEY A PEDIATR RES 5 446 971 026 FEIG SA PEDIATR RES 8 594 974 027 MANGOS JA IN: DI SANTAGNESE PA 109 964 028 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 029 MANGOS JA PEDIATR RES 1 436 967 030 CONOVER JH LANCET 2 1501 973 031 HANN S LANCET 2 520 974 032 LIEBERMAN J AM REV RESPIR DIS 111 100 975 033 MAXFIELD M J CLIN INVEST 41 455 962 034 FRIEDMANN T J LAB CLIN MED 70 404 967 035 EYLAR E GAP CONF REP CELL TISS CULT 8 971 036 SINGER L CLIN BIOCHEM 7 146 974 037 LOUISOT P CLIN CHIM ACTA 48 373 973 038 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 039 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 040 BOLTON WE AM J HUM GENET 27 394 975 041 SOLOMONS CC PEDIATRICS 47 384 971 042 KATTWINKEL J PEDIATRICS 50 133 972 043 RAO GJS SCIENCE 177 610 972 044 LIEBERMAN J PEDIATR RES 3 571 969 045 TALAMO RC PEDIATR RES 6 430 972 046 SPOCK A PEDIATR RES 1 173 967 047 BOWMAN BH SCIENCE 164 325 969 048 CHERRY JD J PEDIATR 79 937 971 049 FARRELL PM PEDIATR RES 8 467 974 050 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 051 SHAPIRO BL LANCET 2 1020 974 052 RAO GJS PEDIATR RES 8 684 974 053 COBURN MD AM REV RESPIR DIS 110 368 974 054 LIEBERMAN J AM REV RESPIR DIS 109 399 974 055 DANES BS LANCET 1 1061 968 056 TAYSI K N ENGL J MED 281 1108 969 057 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 058 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 059 RAO GJS J PEDIATR 80 573 972 060 ANTONOWICZ I PEDIATR RES 6 803 972 061 BOHME B HELV PAEDIATR ACTA 27 607 972 062 BOXERBAUM B AM REV RESPIR DIS 108 777 973 063 CONOD EJ PEDIATR RES 9 724 975 064 WILSON GB CLIN CHIM ACTA 49 79 973 065 WILSON GB PEDIATR RES 9 635 975 066 LOWE CU SCIENCE 153 1124 966 067 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 068 NEUFELD EF REPORT ON STAINING FOR METACH 972 069 ROBERTSON JA LANCET 1 1256 974 070 CAUDILL M LANCET 1 32 974 071 WELCH DW PEDIATR RES 9 698 975 072 WIESMANN UN J PEDIATR 77 685 970 073 DANES BS BIOCHEM GENET 12 359 974 074 LAMBLIN G CLIN CHIM ACTA 36 329 972 075 CHOU L PEDIATR RES 9 312 975 076 PALLAVICINI JC J PEDIATR 77 280 970 077 BENKE PJ LANCET 1 182 972 078 FLETCHER DS CLIN CHIM ACTA 44 5 973 079 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 080 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 081 RAO GJS PEDIATR RES 9 739 975 CT 1 SCHAAP T ISR J MED SCI 14 201 978 2 LAST JA J LAB CLIN MED 91 328 978 3 HALLINAN F MED HYPOTHESES 7 793 981 4 CAREY WF MED J AUST 2 528 983 5 TRAVERS H AM J OBSTET GYNECOL 146 338 983 PN 76077 RN 00431 AN 76215349 AU Schaap-T. Cohen-M-M. TI A proposed model for the inheritance of cystic fibrosis. pp. 291-307. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. MJ CYSTIC-FIBROSIS: fg. MODELS-BIOLOGICAL. MN ALLELES. CAUCASOID-RACE. CONSANGUINITY. GENE-FREQUENCY. GENES. GENES-RECESSIVE. GENOTYPE. HETEROZYGOTE. HUMAN. MONGOLOID-RACE. MONOGRAPH. MUTATION. PEDIGREE. POLYMORPHISM-GENETICS. SELECTION-GENETICS. SEX-FACTORS. EX To geneticists, cystic fibrosis, perhaps the most frequently encountered genetic disease among Caucasians of European origin, presents an enigmatic dilemma on various levels. Those problem areas which complicate our understanding of this disease include methods of diagnosis, ascertainment, and analysis and interpretation of data. In spite of an apparently clear-cut mode of inheritance (autosomal recessive), several conceptual difficulties exist in the acceptance of this formal transmission pattern. The most pressing problem of all, perhaps, is the population genetics of CF. The fact that a lethal gene may be maintained at such elevated frequencies and restricted to given population groups represents a seemingly genetic paradox. Our aim is to review some of the problems encountered in a genetic study of CF and to propose a theoretical model which may help in their elucidation. Topics discussed include problems of diagnosis, ascertainment, and data analysis; family studies and inferences as to the mode of inheritance; population studies; and a proposed model for the inheritance of CF. The main tenet of this model states that CF is determined by dominant alleles at two autosomal loci, with interaction between them. RF 001 DANKS DM ANN HUM GENET 28 323 965 001 CONOVER JH LIFE SCI 14 253 974 002 ANDERSEN DH AM J DIS CHILD 72 62 946 003 WRIGHT SW AM J HUM GENET 20 157 968 003 RAO GJS PEDIATR RES 8 684 974 004 BRUNECKY Z J MED GENET 9 33 972 004 BLOMFIELD J GUT 14 558 973 005 CROW JF IN: NEEL JV 23 965 005 CONOVER JH LANCET 1 1194 973 006 LOWE CU AM J DIS CHILD 78 349 949 007 GOODMAN HO AM J HUM GENET 4 59 952 008 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 009 ROBERTS GBS ANN HUM GENET 24 127 960 010 STEINBERG AG AM J HUM GENET 12 416 960 011 SELANDER P ACTA PAEDIATR SCAND 51 65 962 012 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 013 HOUSTEK J REV MED LIEGE 22 421 967 014 MERRITT AD J LAB CLIN MED 60 998 962 015 LEVIN S IN: GOLDSCHMIDT E 294 963 016 HONEYMAN MS AM J HUM GENET 17 461 965 017 PUGH RJ ARCH DIS CHILD 42 544 967 018 HALL BD J MED GENET 5 262 968 019 SULTZ HA AM J PUBLIC HEALTH 58 491 968 020 ANTONELLI M FRACASTORO (VERONA) 63 207 970 021 STEINBERG AG AM J HUM GENET 8 162 956 022 CONNEALLY PM TEX REP BIOL MED 31 639 973 023 BERNHEIM M PEDIATRIE 16 17 961 024 REDDY CR J TROP MED HYG 73 59 970 025 SALEM M J MED LIBAN 15 61 962 026 HARRIS RL PEDIATRICS 41 733 968 027 OPPENHEIMER EH PEDIATRICS 42 547 968 028 YAMAMOTO T SHONIKA RINSHO 10 1003 957 029 KOBAYASHI R NIPON SHONIKAGAKKAI ZASSHI 65 597 961 030 HAMAMOTO E NIPON SHONIKAGAKKAI ZASSHI 65 502 961 031 IKAI K ACTA PAEDIATR JAP 7 23 965 032 KADOWAKI H ARCH JAP CHIRUG 32 437 963 033 KOMI N BULL TOKYO MED DENT UNIV 13 1 966 034 KANDAR HR ANTISEPTIC 68 563 971 035 WANG CI CF CLUB ABST 9 57 968 036 ZUELZER WW PEDIATRICS 4 53 949 037 DI SANTAGNESE PA ANN INTERN MED 54 482 961 038 PILEGGI A DELAWARE MED J 34 97 962 039 KULCZYCKI LL CLIN PEDIATR 3 692 964 040 SENECAL JJ BULL MED AFRI OCCID FRANC 11 95 954 041 GROVE SS S AFR J LAB CLIN MED 5 113 959 042 MACDOUGALL LG LANCET 2 409 962 043 HEFFER ET NY STATE J MED 74 2355 974 044 WRIGHT SW AM J HUM GENET 20 157 968 045 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 046 DANES BS AM J HUM GENET 23 297 971 047 KNUDSON AG JR AM J HUM GENET 19 388 967 048 ANDERSON CM MOD PROBL PEDIATR 10 381 967 049 DODGE JA LANCET 1 572 975 050 CRAWFURD MDA HEREDITY 29 126 972 051 STUART AB LANCET 2 1521 974 052 HALLETT WY AM REV RESPIR DIS 92 714 965 CT 1 COX DW PEDIATR CLIN NORTH AM 26 467 979 2 MACDONALD MJ DIABETES 29 110 980 3 ROMEO G RIV ITAL PEDIATR 7 201 981 4 ROMEO G EUR J PEDIATR 137 119 981 5 SING CF AM J MED GENET 13 179 982 6 BUCHANAN KD SCAND J GASTROENTEROL 18 155 983 7 WOOD RE SEM RESPIR MED 5 336 984 8 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 9 ROMEO G AM J HUM GENET 37 338 985 10 FISHER JH AM REV RESPIR DIS 132 1149 985 11 STOLLINGER O WIEN MED WOCHENSCHR 135 307 985 12 BRAGANZA JM MED HYPOTHESES 20 233 986 13 BOUE A HUM GENET 74 288 986 PN 76078 RN 00432 AN 76215355 AU Talamo-R-C. Stiehm-E-R. Schwartz-R-H. TI Immunologic aspects of cystic fibrosis. pp. 195-217. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: im. MN ALPHA-1-ANTITRYPSIN: me. ANTIBODIES. ASPERGILLOSIS: et. B-LYMPHOCYTES: im. COMPLEMENT: me. CYSTIC-FIBROSIS: co. FACTOR-XII. HLA-ANTIGENS. HUMAN. HYPERSENSITIVITY: et. IGA: me. IGD: me. IGG: me. IGM: me. IMMUNE-COMPLEX-DISEASE. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: co. LUNG: pp. MONOGRAPH. PANCREATIC-EXTRACTS: im. PHAGOCYTOSIS. PSEUDOMONAS-INFECTIONS: et. RESPIRATORY-TRACT-INFECTIONS: et. REVIEW. STAPHYLOCOCCAL-INFECTIONS: et. T-LYMPHOCYTES: im. EX Because of the prominent role of infection in cystic fibrosis, and because of the genetic nature of both cystic fibrosis and specific immune defects, considerable effort has been expended in determining if there are hereditary immune defects in cystic fibrosis patients that render them susceptible to infection. Against a congenital immune defect is the fact that the infections nearly always are localized to the lung with the same two organisms, rather than infections at multiple sites involving multiple organisms. Specific immune mechanisms in the B and T cell systems are examined. Nonspecific host defense mechanisms include the complement system, phagocytosis, pulmonary clearance, the pulmonary barrier to the systemic spread of infection, protease inhibitors, and factor XIII-dependent systems. Hypersensitivity states include allergy and atopy, allergic aspergillosis, immune complex disease, and hypersensitivity to pancreatic extracts. The presence or absence of specific antigen components and the association of HL-A antigens are also discussed. RF 001 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 002 GREEN MN AM J DIS CHILD 100 365 960 003 SCHWARTZ RH AM J DIS CHILD 111 408 966 004 YOHE RM ANN ALLERGY 30 627 972 005 COLLINS-WILLIAMS C ANN ALLERGY 25 177 967 006 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 007 RACHELEFSKY GS AM J DIS CHILD 128 355 974 008 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 009 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 010 FALCHUK ZM PEDIATRICS 51 49 973 011 GUGLER EC J PEDIATR 73 548 968 012 SOUTH MA J PEDIATR 71 645 967 013 RULE AH PEDIATRICS 48 601 971 014 HALBERT SP PEDIATRICS 26 792 960 015 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 016 HALBERT SP MOD PROBL PEDIATR 10 144 967 017 SCHWARTZ RH AM J DIS CHILD 120 432 970 018 HABBOUSHE C PEDIATRICS 48 973 971 019 DIAZ F J INFECT DIS 121 269 970 020 PENNINGTON JE J INFECT DIS SUPPL 130 159 974 021 MCFARLANE H BR MED J 1 423 975 022 RENNERT OM CLIN PEDIATR 11 351 972 023 HUNICKI VZ KLIN WOCHENSCHR 75 862 963 024 AMMANN AJ MEDICINE (BALTIMORE) 50 223 971 025 BELTOAS E J LANCET 85 254 965 026 BITAR J J PEDIATR 71 123 967 027 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 028 CONOVER JH LANCET 2 1501 973 029 HANN S LANCET 2 520 974 030 LIEBERMAN J AM REV RESPIR DIS 111 100 975 031 SCANLIN TF JR LANCET 1 1382 975 032 CONOVER JH LIFE SCI 14 253 974 033 POLLEY MJ GAP CONF REP CILIARY INHIBITO 973 034 BOHME B HELV PAEDIATR ACTA 27 607 972 035 BOXERBAUM B AM REV RESPIR DIS 108 777 973 036 ROSSMAN CM PEDIATR RES 8 469 974 037 WOOD RE AM REV RESPIR DIS 111 733 975 038 TALAMO RC PEDIATRICS 56 91 975 039 SCHWARTZ RH CF CLUB ABST 19 972 040 SHARP HL IN: MANGOS JA 229 973 041 HOIBY N ACTA PAEDIATR SCAND 63 843 974 042 LIEBERMAN J PEDIATR RES 3 571 969 043 RAO GJS J PEDIATR 80 573 972 044 RAO GJS SCIENCE 177 610 972 045 RAO GJS PEDIATR RES 8 684 974 046 TALAMO RC PEDIATR RES 7 170A 972 047 KOMP DM CHEST 58 501 970 048 COOMBS RRA IN: GELL PGH 761 975 049 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 050 KULCZYCKI LL JAMA 175 358 961 051 PEPYS J J ROY COLL PHYSICIANS LOND 2 42 967 052 HENDERSON AH THORAX 23 501 968 053 MEARNS MB THORAX 20 385 965 054 BATTEN JC MOD PROBL PEDIATR 10 227 967 055 ORES CN CF CLUB ABST 16 48 975 056 MEARNS MB LANCET 1 538 967 057 BURNS MW LANCET 1 270 968 058 DOLAN TF JR AM REV RESPIR DIS 110 812 974 059 BERGNER A PEDIATRICS 55 814 975 060 RAUNIO V IN: DI SANTAGNESE PA 339 964 061 TALAMO RC IN: DI SANTAGNESE PA 356 964 062 BLANC WA IN: DI SANTAGNESE PA 327 964 063 URIEL J IN: DI SANTAGNESE PA 243 964 064 LOWE CU SCIENCE 153 1124 966 065 BROGLIO AL CF CLUB ABST 967 066 SCHWARTZ RH J LAB CLIN MED 70 725 967 067 GREEN MN PEDIATRICS 21 635 958 068 GREENE EL INT ARCH ALLERGY 40 184 971 069 STEPHAN U PEDIATRICS 55 35 975 070 BREWERTON DA LANCET 1 904 973 071 MACKAY IR LANCET 2 793 972 072 FALCHUK ZM J CLIN INVEST 51 1602 972 073 WALFORD RL HISTOCOMPATIBILITY TESTING 271 970 074 POLYMENIDIS Z LANCET 2 1452 973 075 VLADUTIU AO IMMUNOGENETICS 1 305 974 076 PATTERSON PR CF CLUB ABST 975 077 BACH FH SCIENCE 176 1024 972 CT 1 DISANTAGNESE PA N ENGL J MED 295 597 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 BUESCHER ES J PEDIATR 93 530 978 4 SORENSEN RU J PEDIATR 93 201 978 5 SEALE TW J CLIN MICROBIOL 9 72 979 6 CHURCH JA J PEDIATR 95 272 979 7 MCCARTHY MM CLIN PEDIATR 19 746 980 8 FICK RB J IMMUNOL METH 38 103 980 9 CHURCH JA ANN ALLERGY 45 217 980 10 WILSON GB J CLIN INVEST 66 1010 980 11 HARPER TB LUNG 157 219 980 12 MATTHEWS WJ N ENGL J MED 302 245 980 13 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 14 WELCH MJ PEDIATRICS 67 664 981 15 MOSS RB J PEDIATR 99 215 981 16 SORENSEN RU AM REV RESPIR DIS 123 37 981 17 DANIELE RP AM REV RESPIR DIS 124 738 981 18 VANGEFFEL R IMMUNOL LETTERS 5 155 982 19 WILSON GB MED HYPOTHESES 8 527 982 20 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 21 GRAFT DF AM REV RESPIR DIS 125 540 982 22 HARPER TB AM REV RESPIR DIS 126 540 982 23 GUIDOTTI TL AM J MED SCI 283 157 982 24 SORENSEN RU EUR J RESPIR DIS 64 524 983 25 FICK RB J CLIN INVEST 74 236 984 26 AUERBACH HS LANCET 2 686 985 27 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 76079 RN 00433 AN 76215360 AU Denning-C-R. Gluckson-M-M. Mohr-I. TI Psychological and social aspects of cystic fibrosis. pp. 127-51. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS. MN CYSTIC-FIBROSIS: fg, th. DENIAL-PSYCHOLOGY. FAMILY-PLANNING. GENETIC-COUNSELING. HETEROZYGOTE. HUMAN. MASS-SCREENING. MONOGRAPH. PSYCHOLOGY. QUESTIONNAIRES. REVIEW. SOCIOECONOMIC-FACTORS. SOCIOLOGY. STRESS-PSYCHOLOGICAL. UNITED-STATES. EX Cystic fibrosis is a disease in which there are enormous and sometimes overwhelming psychological and social problems encountered by the patient and his family. The present "state of the art" as it pertains to the psychological and social aspects of cystic fibrosis are examined. Attitudes towards cystic fibrosis, implications of a genetic diagnosis, heterozygote screening, and projections for the future are discussed. RF 001 ANDERSEN DH CHILDREN 7 11 960 002 TURK J PEDIATRICS 34 67 964 003 LAWLER RH CAN MED ASSOC J 94 1043 966 004 SPOCK A NC MED J 27 426 966 005 KULCZYCKI LL CLIN PROC CHILD HOSP DC 25 320 969 006 MCCOLLUM AT J PEDIATR 77 571 970 007 TROPAUER A AM J DIS CHILD 119 424 970 008 MEYEROWITZ JH SOC SCI MED 1 249 967 009 PLESS IB J PEDIATR 79 351 971 010 MATTSSON A PEDIATRICS 50 801 972 011 GAYTON WF AM J DIS CHILD 126 856 973 012 BEVERIDGE J AUST PAEDIATR J 9 214 973 013 BURTON L PRACTITIONER 210 247 973 014 ALLAN JL AUST PAEDIATR J 10 136 974 015 MCCRAE WM IN: APLEY J 4 157 974 016 WINDER A IN: PATTERSON PR 973 017 ROSENLUND ML ANN INTERN MED 78 959 973 018 FARKAS A AM J ORTHOPSYCHIATRY 43 259 973 019 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 020 MANGOS JA FUNDAMENTAL PROB CF AND RELAT 973 021 SHWACHMAN H PEDIATRICS 36 689 965 022 HECHT F N ENGL J MED 287 464 972 023 STEINHAUER PD PEDIATR CLIN NORTH AM 21 825 974 024 EMERY AEH BR MED J 1 724 973 025 LEONARD CO N ENGL J MED 287 433 972 026 CARTER CO LANCET 1 281 971 027 PEARN JH J MED GENET 10 129 973 028 MYERS JK DECADE LATER FOLLOW UP OF SOC 235 968 029 ANON MONTHLY VITAL STAT REPORT USD 24 4 975 030 KABACK MM PROG MED GENET 10 103 974 031 WOOD RE LANCET 2 1452 973 032 ANON GRANTS ADMIN MANUAL DHEW PUB 971 033 SHIH VE LAB TECH FOR DETECTION HEREDI 973 034 LEVY HL ADV HUM GENET 4 1 973 035 SHWACHMAN H PEDIATRICS 46 335 970 036 GEORGE L ARCH DIS CHILD 46 139 971 037 STEPHAN U IN: MANGOS JA 281 973 038 WARWICK WJ IN: MANGOS JA 973 039 STEPHAN U PEDIATRICS 55 35 975 040 ANON GENETIC SCREENING PROG PRIN A 975 041 ANON WHO TECH REP SER NO 401 968 042 WILSON JMG PRINCIPLES PRACTICE SCREENING 968 043 THORNER RM PUBLIC HEALTH MONOGRAPH DHEW 10 967 044 LAPPE M N ENGL J MED 286 1129 972 045 INGLEFINGER FJ N ENGL J MED 287 465 972 046 LUBS HA IN: HILTON B 973 047 MOTULSKY AG ISR J MED SCI 9 1341 973 CT 1 FALKMAN C ACTA PAEDIATR SCAND SUPPL 264 1977 7 977 2 DROTAR D PEDIATRICS 67 338 981 3 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 4 MOLLERING M KLIN PAEDIATR 198 369 986 PN 76080 RN 00434 AN 76215357 AU Esterly-J-R. Oppenheimer-E-H. Landing-B-H. TI Pathology of cystic fibrosis. pp. 115-24. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: pa. MN ADRENAL-MEDULLA: pa. BRONCHI: pa. CYSTIC-FIBROSIS: co. DIABETES-MELLITUS: et. DOWNS-SYNDROME: co. EXOCRINE-GLANDS: pa. GENES. HEMOPTYSIS: et. HEMOSIDERIN: me. HUMAN. KIDNEY-MEDULLA: pa. LIPOFUSCIN: me. LUNG: pa. MONOGRAPH. PANCREAS: pa. REVIEW. VITAMIN-A-DEFICIENCY: pa. EX The varied morphologic lesions in cystic fibrosis each result from obstruction of one type or another, documented or presumed. They are modified by the age of the individual, the severity and the conditions peculiar to each tissue. These lesions, plus the functional changes in sweat electrolytes, membrane transport and serum factor affecting syncrony of cilia, represent the diverse actions of a genetic defect, the intermediate stages of which are as yet unknown. Neither the diversity of abnormalities, their range in severity, nor differences in the age of onset argue convincingly for a trigger mechanism or acquire rather than inborn deficiency. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 FARBER S ARCH PATHOL 37 238 944 003 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 004 OPPENHEIMER EH J PEDIATR 86 683 975 005 DI SANTAGNESE PA N ENGL J MED 279 103 968 006 LANDING BH ARCH PATHOL 88 569 969 007 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 008 FIELD WEH BR J DIS CHEST 62 11 968 009 ESTERLY JR THORAX 23 670 968 010 VAWTER GF GAP CONF REP PATHOL OF CF 9 972 011 LANDING BH ANN NY ACAD SCI 93 518 962 012 TAYLOR BW ARCH DIS CHILD 49 133 974 013 KOPITO L PEDIATR RES 6 427 972 014 GLISSON F RACHITIDE SIVE MORBO PUERI 650 015 BLACKFAN KD J PEDIATR 3 679 933 016 PETERSEN RA AM J DIS CHILD 116 662 968 017 SMITH FR J LAB CLIN MED 80 423 972 018 DE LUCA L CANCER 30 1326 972 019 BLANC WA PEDIATRICS 22 494 958 020 WALTERS TR AM J DIS CHILD 124 641 972 022 KOMP DM CHEST 58 501 970 023 TABOLIN VA VOPR OKHR MATERIN DET 17 41 972 024 BOLANDE RP ARCH PATHOL 95 172 973 025 WANG CI CF CLUB ABST 968 026 OPPENHEIMER EH JOHNS HOPKINS MED J 131 351 972 027 MILUNSKY A PEDIATRICS 42 501 968 028 WANG CI PEDIATRICS 48 442 972 029 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 111 1 962 030 GIBSON LE J PEDIATR 81 193 972 031 OPPENHEIMER EH IN: MCKUSICK VA 7 108 972 032 OPPENHEIMER EH ARCH PATHOL 96 149 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 GOTZ M EUR J PEDIATR 127 133 978 3 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 4 JALOWAYSKI AA RESPIRATION 43 249 982 5 COALSON JJ BR J EXP PATHOL 66 205 985 6 HOIBY N ANNU REV MICROBIOL 40 29 986 PN 76081 RN 00435 AN 76215363 AU Butcher-F-R. TI Relevance of cyclic nucleotides and calcium to cystic fibrosis. pp. 243-58. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: ph. CALCIUM: ph. CYSTIC-FIBROSIS: me. GUANOSINE-CYCLIC-MONOPHOSPHATE: ph. PANCREAS: se. SALIVARY-GLANDS: se. MN ADENYL-CYCLASE: me. AMYLASES: se. ANIMAL. CALCIUM: pd. CELLS-CULTURED. CILIA: pp. CYSTIC-FIBROSIS: pp. GLYCOGEN: me. MICROTUBULES: pp. MONOGRAPH. PARASYMPATHOMIMETICS: pd. POTASSIUM: me. PROTEIN-KINASES: me. RATS. REVIEW. SECRETIN: ph. SYMPATHOMIMETICS: pd. SUPPORT-U-S-GOVT-P-H-S. EX The general area of research at the biochemical and molecular levels related to cystic fibrosis has suffered from the lack of a suitable animal model. Although some animal models have been proposed, these have not yet been tested. In attempts to circumvent this problem several investigators have turned to cell culture techniques to study various aspects of cystic fibrosis. These studies have included the use of red blood cells, lymphocytes, skin fibroblasts and cultured nasal polyps. There are enough positive indications from this approach to indicate that it might be fruitful. Consequently, a final aspect of this article will be a consideration of the use of cell cultures for future investigations into the possible metabolic lesions in cystic fibrosis. Control of cyclic nucleotide levels in parotid, the role of calcium, microfilaments and microtubules in secretion, and the use of cell cultures as an approach to the study of biochemical defects in cystic fibrosis are discussed. RF 001 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 001 DANES BS J EXP MED 136 1313 972 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 002 MARTINEZ JR PEDIATR RES 9 463 975 003 MARTINEZ JR PEDIATR RES 9 470 975 003 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 004 BDOLAH A BIOCHEM BIOPHYS RES COMMUN 18 452 965 004 PALLAVICINI JC J PEDIATR 77 280 970 005 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 005 BATZRI S J BIOL CHEM 248 361 973 006 DANES BS J EXP MED 129 775 969 006 MONNARD P EUR J PHARMACOL 23 306 973 007 MALAMUD D BIOCHIM BIOPHYS ACTA 279 3731 972 007 KLAGSBRUN M PEDIATR RES 8 205 974 008 QUISSELL DO NATURE 247 115 974 008 DURHAM JP BIOCHIM BIOPHYS ACTA 372 196 974 009 BUTCHER FR BIOCHIM BIOPHYS ACTA 392 82 975 009 DUFFEY MJ NATURE 249 94 974 010 BENZ L BR J PHARMACOL 46 66 972 010 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 011 BARTMAN J J PEDIATR 76 430 970 011 DESCHODT-LANCKMAN M GASTROENTEROLOGY 68 318 975 012 BAIG MM J PEDIATR 86 72 975 012$ CASE RM J PHYSIOL (LOND) 223 972 013 BURGEU ASV HANDBOOK OF PHYSIOLOGY 561 967 013 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 014 SCHULTZ G PROC NAT ACAD SCI USA 70 3889 973 014 CAUDILL M LANCET 1 32 974 015 DUFFY MJ CLIN CHIM ACTA 49 397 973 016 RASMUSSEN H CRIT REV BIOCHEM 1 95 972 016 DUFFY MJ CLIN CHIM ACTA 50 97 974 017 SELINGER Z BIOCHIM BIOPHYS ACTA 203 335 970 017 BOAT TF AM REV RESPIR DIS 110 428 974 018 HARFIELD B CAN J PHYSIOL PHARMACOL 5 997 973 018 DANES BS BIOCHEM GENET 12 359 974 019 CONOVER JH PEDIATR RES 7 224 973 019 FRANKS DJ FEBS LETTERS 42 267 974 020 DOUGLAS WW J PHYSIOL (LOND) 165 528 963 021 SELINGER Z J BIOL CHEM 248 369 973 022 SCHRAMM M IN: CECCARELLI B 2 974 025 HEISLER S BIOCHIM BIOPHYS ACTA 279 561 972 026 KANNO T J PHYSIOL (LOND) 226 353 972 027 CASE RM J PHYSIOL (LOND) 235 75 973 028 MATTHEWS EK J PHYSIOL (LOND) 234 689 973 029 SELINGER Z ANN NY ACAD SCI 185 395 971 031 CHRISTOPHE J IN: CECCARELLI B 2 974 032 SCHRAMM M NATURE NEW BIOL 240 203 972 033 BUTCHER FR BIOCHEM BIOPHYS RES COMMUN 48 23 972 034 BUTCHER FR J CELL BIOL 60 519 974 035 WILLIAMS JA FED PROC 34 1324 975 036 BAUDUIN H J CELL BIOL 66 165 975 037 ALLISON AC IN: CECCARELLI B 2 974 038 HSIE AW PROC NAT ACAD SCI USA 68 358 971 039 YAHARA I PROC NAT ACAD SCI USA 72 1579 975 040 BHISEY AN EXP CELL RES 64 419 971 041 GOODMAN DBP PROC NAT ACAD SCI USA 67 652 970 042 SOIFER D J NEUROCHEM 24 21 975 043 SLOBODA RD PROC NAT ACAD SCI USA 72 177 975 044 PIRAS R PROC NAT ACAD SCI USA 72 1161 975 045 SHELANSKI ML PROC NAT ACAD SCI USA 70 765 973 046 EDELMAN GM PROC NAT ACAD SCI USA 70 1442 973 047 ROSENBLITH JZ PROC NAT ACAD SCI USA 70 1625 973 048 OLIVER JM NATURE 253 471 975 049 WOOD RE AM REV RESPIR DIS 111 733 975 050 BESLEY GTN J MED GENET 6 278 969 051 DOGGETT RG NATURE NEW BIOL 243 250 973 052 ADSHEAD PC ANN NY ACAD SCI 253 192 975 053 ECKERT R J PHYSIOL (LOND) 226 699 972 054 CHERNICK WS MOD PROBL PEDIATR 10 125 967 056 PICKERING R ARCH ORAL BIOL 20 153 975 057 ASPRAY DW ANAL BIOCHEM 66 353 975 058 IGNARRO LJ PROC NAT ACAD SCI USA 71 2027 974 059 NEISSMAN G NATURE NEW BIOL 231 131 971 060 ZURIER RB J CELL BIOL 58 27 973 061 SMITH RJ PROC NAT ACAD SCI USA 72 108 975 062 GOLDSTEIN IM BIOCHEM BIOPHYS RES COMMUN 60 807 974 063 ZURIER RB PROC NAT ACAD SCI USA 70 844 973 064 ZURIER RB J CLIN INVEST 53 297 974 065 BARTMAN J J PEDIATR 76 430 970 066 STAHL M J PEDIATR 84 821 974 067 BERATIS NG PEDIATR RES 7 958 973 068 MCEVOY FA CLIN CHIM ACTA 54 195 974 069 BAIG MM J PEDIATR 86 72 975 070 PALLAVICINI JC J PEDIATR 77 280 970 071 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 072 PASTAN IH ANNU REV BIOCHEM 44 491 975 073 GRANNER DK BIOCHEM BIOPHYS RES COMMUN 46 1516 972 074 COFFINO P J CELL PHYSIOL 85 603 975 075 MACKENZIE CW J BIOL CHEM 249 5755 974 CT 1 NEUTRA MR GASTROENTEROLOGY 75 701 978 2 THEOHARIDES TC LIFE SCI 27 703 980 3 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 4 SORSCHER EJ LANCET 1 368 982 5 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 PN 76082 RN 00436 AN 76215362 AU Farrell-P-M. Lundgren-D-W. TI Recent observations concerning RNA methylation and polyamine metabolism in cystic fibrosis. pp. 223-41. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. MJ CYSTIC-FIBROSIS: me. POLYAMINES: me. RNA: me. MN ARGININE: an. CELL-LINE. ERYTHROCYTES: me. FEMALE. HUMAN. MALE. MENSTRUATION. METHYLATION. MONOGRAPH. PROGESTERONE: bl. PUTRESCINE: an. RNA-RIBOSOMAL: me. RNA-TRANSFER: me. SEX-FACTORS. SPERMIDINE: bl. SPERMINE: bl. SWEAT: an. EX Determination of fibroblast proliferation rates and RNA methylation patterns in our laboratory has revealed no alteration in cells from cystic fibrosis patients. Blood polyamines are located predominantly in erythrocytes with low concentrations in plasma; sweat has likewise been shown to have a low content of spermidine and spermine. Whole blood polyamine concentrations fluctuate from one individual to another and are especially variable in females subject to the hormonal changes of the menstrual cycle. Because of the variability in the spermidine and spermine content of blood, it is useful to express such data on the basis of the spermidine/spermine ratio; however, results must be analyzed according to sex and, in the case of females, should be interpreted in relationship to the menstrual cycle. Spermidine/spermine ratios are significantly elevated in whole blood extracts and isolated erythrocytes from male CF homo- and heterozygotes. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 VEGHELYI PV AM J DIS CHILD 90 28 955 003 PARSA I FED PROC 31 166 972 004 FLEISHER DS J PEDIATR 64 349 964 005 RENNERT OM PEDIATRICS 50 485 972 006 KERR SJ ADV ENZYMOL 36 1 972 007 STARR JL PHYSIOL REV 49 623 969 008 KLAGSBRUN M J BIOL CHEM 247 7443 972 009 TABOR H PHARMACOL REV 16 245 964 010 ROSENTHAL SM PROC SOC EXP BIOL MED 80 432 952 011 SEILER N HOPPE SEYLERS Z PHYSIOL CHEM 354 589 973 012 STEVENS L ANN NY ACAD SCI 171 827 970 013 STURMAN JA PEDIATR RES 8 231 974 014 OKA T J BIOL CHEM 249 7647 974 015 RUSSELL DH BIOCHEM J 128 1109 972 016 MUNRO GF J BACTERIOL 118 952 974 017 IGARASHI K BIOCHEM BIOPHYS RES COMMUN 64 897 975 018 HEBY O FEBS LETTERS 50 1 975 019 MARTON LJ IN: RUSSELL DH 367 973 020 DENTON MD IN: RUSSELL DH 373 973 021 NISHIOKA K CLIN CHIM ACTA 57 155 974 022 MANGOS JA AMINO ACID METABOLISM AND CF 7 971 023 RENNERT OM CLIN PEDIATR 11 351 972 024 RENNERT OM IN: MANGOS JA 41 973 025 KLAGSBRUN M IN: MANGOS JA 53 973 026 KLAGSBRUN M PEDIATR RES 8 205 974 027 HODES ME PEDIATR RES 8 212 974 028 LUNDGREN DW CLIN CHIM ACTA 62 357 975 029 MUNRO GF CF CLUB ABST 16 7 975 030 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 031 WRIGHT SW AM J HUM GENET 20 157 968 032 TODARO GJ J CELL COMP PHYSIOL 62 257 963 033 KLAGSBRUN M J BIOL CHEM 248 2612 973 034 TABOR H ANAL BIOCHEM 55 457 973 036 COHEN LF PEDIATR RES 9 312 975 037 RABINOWITZ Y BLOOD 23 811 964 039 GORDON RS J APPL PHYSIOL 31 713 971 040 PALLAVICINI JC J PEDIATR 77 280 970 041 BARNETT DR TEX REP BIOL MED 31 691 973 042 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 043 FLETCHER DS CLIN CHIM ACTA 44 5 973 044 RUSSELL DH ENDOCRINOLOGY 88 1397 971 045 ROSENTHAL SM J PHARMACOL EXP THER 116 131 956 046 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 047 LOUISOT P CLIN CHIM ACTA 48 373 973 048 PETER WH HOPPE SEYLERS Z PHYSIOL CHEM 354 1146 973 049 MCEVOY FA CLIN CHIM ACTA 54 195 974 050 COLE CH PEDIATR RES 6 616 972 051 INOUE H J BIOCHEM (TOKYO) 75 679 974 052 MARTINEZ JR PEDIATR RES 9 463 975 053 KNOECKI D ARCH BIOCHEM BIOPHYS 169 192 975 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 PN 76083 RN 00437 AN 76215365 AU Levison-H. Godfrey-S. TI Pulmonary aspects of cystic fibrosis. pp. 3-24. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: pp. RESPIRATION. MN ADOLESCENCE. AIRWAY-RESISTANCE. CHILD. CYSTIC-FIBROSIS: th. EXERTION. FEMALE. HELIUM. HUMAN. MALE. MAXIMAL-EXPIRATORY-FLOW-RATE. MAXIMAL-EXPIRATORY-FLOW-VOLUME-CURVES. MONOGRAPH. OXYGEN. PHYSICAL-FITNESS. PHYSICAL-THERAPY. PLETHYSMOGRAPHY-WHOLE-BODY. REVIEW. SEX-FACTORS. VENTILATION-PERFUSION-RATIO. EX Defective mucociliary transport has been implicated in the pathogenesis of the lung disease in cystic fibrosis, based on several observations. First, patients with the disease secrete more mucus than normal subjects, as indicated by gland hypertrophy, and frequently retain it in the lung. Second, the mucus is said to have altered biochemical and rheological properties; however, although the physiochemical properties of mucus are altered in all lung diseases, little histochemical or rheological difference has been found in mucus from patients with CF and other hypersecretory conditions such as chronic bronchitis and bronchiectasis. Third, a factor in the serum of CF patients inhibits normal ciliary activity in explain glands of ciliated epithelium; and infection, which damages human ciliated epithelium in culture, could further reduce mucociliary transport. Measurement of pulmonary function in cystic fibrosis, physical fitness and response to exercise in cystic fibrosis, control of breathing in patients with cystic fibrosis, regional lung function in cystic fibrosis, and therapy of cystic fibrosis are discussed. RF 001 ZUELZER WW PEDIATRICS 4 53 949 002 REID L MOD PROBL PEDIATR 10 195 967 003 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 004 MATTHEWS LW AM REV RESPIR DIS 88 199 963 005 POTTER JL ANN NY ACAD SCI 106 692 963 006 DENTON R PEDIATRICS 25 611 960 007 LAMB D BR J DIS CHEST 66 239 972 008 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 009 CHARMAN J BIORHEOLOGY 9 185 972 010 FEATHER EA BR J DIS CHEST 64 192 970 011 SPOCK A PEDIATR RES 1 173 967 012 CONOVER JH PEDIATR RES 7 220 973 013 CAMNER P AM REV RESPIR DIS 108 131 973 014 CAMNER P IN: HERS JFP 236 973 015 SAKAKURA Y ANN OTOL RHINOL LARYNGOL 82 203 973 017 SANCHIS J N ENGL J MED 288 651 973 018 THOMSON ML N ENGL J MED 289 749 974 019 WOOD RE AM REV RESPIR DIS 111 733 975 020 MACKLEM PT PHYSIOL REV 51 368 971 021 LAMARRE A PEDIATRICS 50 291 972 022 COOPER DM AM REV RESPIR DIS 109 519 974 023 MCCARTHY DS AM J MED 52 747 972 024 MANSELL A AM REV RESPIR DIS 109 190 974 025 HUTCHEON M AM REV RESPIR DIS 110 458 974 026 LAPIERRE JG CAN PAED SOC ANN MTG ABST 61 975 027 EMIRGIL C AM J MED 51 504 971 028 DOERSHUK CF AM REV RESPIR DIS 109 452 974 029 PHELAN PD ARCH DIS CHILD 44 393 969 031 GOLDRING RM J PEDIATR 65 501 964 032 GODFREY S EXERCISE TESTING IN CHILDREN 974 033 GODFREY S ARCH DIS CHILD 46 144 971 034 JONES NL CLIN SCI 31 39 966 035 DAVIES CT HUM BIOL 44 195 972 036 ANDERSON SD BR J DIS CHEST 69 1 975 037 DAY G ARCH DIS CHILD 48 355 973 039 LLOYD-STILL JD PEDIATRICS 53 678 974 040 COUNAHAN R ARCH DIS CHILD 50 477 975 041 WHITELAW WA RESPIR PHYSIOL 23 181 975 044 FEATHERBY EA CAN MED ASSOC J 102 835 970 045 CHANG N AM REV RESPIR DIS 107 672 973 046 MOTOYAMA EK IN: MANGOS JA 335 973 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 ORENSTEIN DM AM J DIS CHILD 131 973 977 4 SEALE TW J CLIN MICROBIOL 9 72 979 5 GUIDOTTI TL RESPIRATION 44 351 983 6 WHEELER WB J PEDIATR 104 695 984 7 EICHLER I DTSCH MED WSCHR 111 896 986 PN 76084 RN 00438 AN 76215361 AU Doershuk-C-F. Wood-R-E. Boat-T-F. TI Pulmonary therapy for cystic fibrosis. pp. 25-37. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: th. EXERCISE-THERAPY. EXPECTORANTS: tu. RESPIRATORY-THERAPY. MN AEROSOLS. AIRWAY-OBSTRUCTION: th. ASPERGILLOSIS: dt. BREATHING-EXERCISES. BRONCHODILATOR-AGENTS: tu. CYSTIC-FIBROSIS: co, dt. ENDOSCOPY. HEMOPTYSIS: th. HUMAN. IRRIGATION. LUNG-DISEASES: dt, et. LUNG: su. MONOGRAPH.PNEUMOTHORAX: dt. PSEUDOMONAS-INFECTIONS: dt. REVIEW. SUPPORT-U-S-GOVT-P-H-S. WATER. EX This report summarizes selected aspects of pulmonary therapy for cystic fibrosis. Since the results of chronic bronchopulmonary infection are the cause of death in most CF patients, improved patient survival patterns may possibly be attributed in large part to improvements in antibiotic therapy and increased therapeutic aggressiveness. Mist tent therapy was defined and initiated in the 1950s, but later studies of mist tent therapy have not shown beneficial response in many patients selected for study. Intermittent aerosol therapy continues to be employed by many physicians to deliver medication rather than bulk liquid to the bronchial mucosa. Pulmonary endoscopy and lavage, expectorants, allergic aspergillosis, pneumothorax, lobectomy, and hemoptysis are also examined. RF 001 MATTHEWS LW J PEDIATR 65 558 964 002 MEARNS MB ARCH DIS CHILD 47 5 972 003 DOERSHUK CF ARCH DIS CHILD 47 984 972 004 ANON AM REV RESPIR DIS PART 2 110 1 974 005 HUANG NN J PEDIATR 59 512 961 006 IACOCCA VF AM J DIS CHILD 106 315 963 007 MEARNS MB ARCH DIS CHILD 47 902 972 008 MAY JR ARCH DIS CHILD 47 908 972 009 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 010 BOXERBAUM B AM REV RESPIR DIS 108 777 973 011 WOOD RE AM REV RESPIR DIS 111 733 975 012 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 013 BURNS MW LANCET 1 270 968 014 HOIBY N SCAND J RESPIR DIS 56 38 975 015 LAWSON D IN: WATT PJ 69 970 016 DOERSHUK CF IN: GREEN M 707 968 017 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 018 MARKS MI J PEDIATR 79 822 971 019 HUANG NN J PEDIATR 78 338 971 020 PENNINGTON JE J INFECT DIS 128 63 973 022 KLASTERSKY J CHEST 61 117 972 023 DENTON R AM J DIS CHILD 82 433 951 024 DOERSHUK CF PEDIATRICS 41 723 968 025 PARKS CR J PEDIATR 76 305 970 026 MATTHEWS LW PEDIATRICS 39 176 967 027 MOTOYAMA EK PEDIATRICS 50 299 972 028 CHANG N AM REV RESPIR DIS 107 672 973 029 WOLFSDORF J PEDIATRICS 43 799 969 030 BAU SK PEDIATRICS 48 605 971 031 YEATES DB AM REV RESPIR DIS 107 602 973 032 ALDERSON PO J PEDIATR 84 479 974 033 WEST JB NATURE 189 588 961 034 MORROW PE AM REV RESPIR DIS SUPPL 110 88 974 035 PARKS CR AM REV RESPIR DIS 104 99 971 036 PARKS CR AM REV RESPIR DIS 108 513 973 037 GOLDBERG IS ARCH INTERN MED 131 88 973 038 KING MA AM REV RESPIR DIS 110 740 974 039 DULFANO MJ AM REV RESPIR DIS 112 341 975 040 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 041 DULFANO MJ AM REV RESPIR DIS 107 130 973 042 ROSENBLUTH M ARCH DIS CHILD 49 606 974 043 ANON AM REV RESPIR DIS PART 2 110 7 974 044 PHELAN PD ARCH DIS CHILD 44 393 969 045 LANDAU LI J PEDIATR 82 863 973 046 BARKER R J PEDIATR 80 396 972 047 CARSON S AM REV RESPIR DIS SUPPL 93 86 966 048 LAKE KB CHEST 68 62 975 049 MELLINS RB AM REV RESPIR DIS SUPPL 110 137 974 050 LORIN MI AM J PHYS MED 50 215 971 051 MOTOYAMA EK IN: MANGOS JA 335 973 052 SHARP JT AM REV RESPIR DIS SUPPL 110 154 974 053 SACKNER MA AM REV RESPIR DIS 111 62 975 054 CEZEAUX G JR JAMA 199 15 967 055 KYLSTRA JA AM REV RESPIR DIS 103 651 971 056 ALTMAN RP J PEDIATR SURG 8 809 973 057 FORBES J LANCET 2 767 957 058 HIRSCH SR CHEST 63 9 973 059 SCHWARTZ RH AM J DIS CHILD 120 432 970 060 PEPYS J AM REV RESPIR DIS 80 167 959 061 SLAVIN RG AM J MED 47 306 969 062 HENDERSON AH THORAX 23 501 968 063 BOAT TF JAMA 209 1498 969 064 LIFSCHITZ MI AM J DIS CHILD 116 633 968 065 STOWE SM AM REV RESPIR DIS 111 611 975 066 KULCZYCKI LL N ENGL J MED 257 203 957 067 DOERSHUK CF ANESTH ANALG CLEVE 51 413 972 068 GOTTLIEB LS CHEST 67 482 975 069 FELLOWS KE RADIOLOGY 114 551 975 PN 76085 RN 00439 AN 76215356 AU Boat-T-F. Cheng-P-W. TI Mucous glycoproteins. pp. 165-77. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ BRONCHI: se. CYSTIC-FIBROSIS: me. GLYCOPROTEINS: se. MUCUS: se. TRACHEA: se. MN AMINO-ACIDS: an. ATROPINE: pd. BRONCHITIS. CARBOHYDRATES: an. COMPARATIVE-STUDY. EPINEPHRINE: pd. GLYCOPROTEINS: an. HUMAN. METHACHOLINE-COMPOUNDS: pd. MONOGRAPH. MUCUS: an. MURAMIDASE: se. NOREPINEPHRINE: pd. REVIEW. TERBUTALINE: pd. SUPPORT-U-S-GOVT-P-H-S. EX Early investigators employed histochemical, chemical and physiochemical techniques in an attempt to identify differences between cystic fibrosis and control mucous secretions. Several mucous glycoprotein abnormalities were suggested by their studies. In retrospect, none of these studies achieved their goal of describing a fundamental mucous glycoprotein abnormality peculiar to CF. Improved methods have been devised for the collection of mucous glycoproteins in human respiratory tract secretions and the study of their chemical and physical properties. Therefore, it is now possible to make preliminary comparisons between human tracheobronchial mucous glycoproteins from CF and non-CF sources. General properties of mucous glycoproteins, human tracheobronchial mucous glycoproteins, comparison of CF and non-CF tracheobronchial mucous glycoprotein, and suggested directions for future studies of CF mucous secretions are discussed. RF 001 DI SANTAGNESE PA PEDIATRICS 19 252 957 002 DISCHE Z PEDIATRICS 24 74 959 003 ROELFS RE AM J DIS CHILD 113 419 967 004 CHERNICK WS ANN NY ACAD SCI 106 698 963 005 DISCHE Z AM J DIS CHILD 102 733 961 006 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 007 LEV R AM J PATHOL 46 23 965 008 LAMB D BR J DIS CHEST 66 239 972 009 GUGLER EC J PEDIATR 71 585 967 010 POTTER JL ANN NY ACAD SCI 106 692 963 011 BOAT TF AM REV RESPIR DIS 110 428 974 012 BOAT TF PEDIATR RES 8 531 974 013 SPIRO RG ADV PROT CHEM 27 349 973 014 GOTTSCHALK A GLYCOPROTEINS THEIR COMPOSITI 972 015 LLOYD KO BIOCHEMISTRY 7 2976 968 016 ANDERSON B ARCH BIOCHEM BIOPHYS 145 490 971 017 LUNDBLAD A ARCH BIOCHEM BIOPHYS 148 291 972 018 ROVIS L BIOCHEMISTRY 12 5340 973 019 ROVIS L BIOCHEMISTRY 12 5340 973 020 GOODWIN SD EUR J BIOCHEM 47 371 974 021 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 022 HAVEZ R CLIN CHIM ACTA 17 281 967 023 HAVEZ R CLIN CHIM ACTA 17 463 967 024 HAVEZ R IN: PEETERS H 16 343 968 025 DEGAND P IN: PEETERS H 16 361 968 026 HAVEZ R BULL SOC CHIM BIOL 51 245 969 027 ROUSSEL P CLIN CHIM ACTA 36 315 972 028 LAMBLIN G CLIN CHIM ACTA 36 329 972 029 DEGAND P BULL PHYSIOPATH RESPIR NANCY 9 199 973 030 ROUSSEL P J BIOL CHEM 250 2114 975 031 ROBERTS GP EUR J BIOCHEM 50 265 974 032 BOAT TF PEDIATR RES 7 607 973 033 WOOD RE AM REV RESPIR DIS 111 733 975 034 WAGNER BM AM J DIS CHILD 99 61 960 035 BOAT TF CHEST 67 325 975 036 SLOMIANY BL J BIOL CHEM 247 5062 972 037 PECK HD ADV ENZYMOL 39 651 974 038 KALINER M N ENGL J MED 289 277 973 039 YEAGER H JR CLIN RES 23 34A 975 040 FRIBERG S AM REV RESPIR DIS 108 1010 973 041 DULFANO MJ AM REV RESPIR DIS 112 341 975 042 OSSERMAN EF J EXP MED 124 921 966 043 LOWRY OH J BIOL CHEM 193 265 951 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 SANTAGNESE PAD N ENGL J MED 295 481 976 3 BOAT TF PEDIATR RES 11 977 977 4 NEUTRA MR LAB INVEST 36 535 977 5 WANNER A AM REV RESPIR DIS 116 73 977 6 BARTON AD PROC SOC EXP BIOL MED 156 8 977 7 LAST JA J LAB CLIN MED 91 328 978 8 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 9 HOSLI P MONOGR PAEDIATR 10 90 979 10 BOAT TF FED PROC 39 3067 980 11 MATTHEWS WJ N ENGL J MED 302 245 980 12 LIEBERMAN J J LAB CLIN MED 97 646 981 13 TABAK LA J ORAL PATHOL 11 1 982 14 MORIARTY CM J MED 13 257 982 15 CHENG PW J BIOL CHEM 257 6251 982 16 FRATES RC PEDIATR RES 17 30 983 17 KNOWLES M J CLIN INVEST 71 1410 983 18 KATZ S CELL CALC 5 421 984 19 WINGERT WE BIOCHEMISTRY 23 690 984 20 RAM BP CRC CRIT REV BIOCHEM 17 257 985 21 REDDY MS J DENT RES 64 33 985 22 CHENG PW BIOCHEM J 227 405 985 23 SMITH AC CLIN GASTROENTEROL 15 815 986 24 CHENG PW CARBOHYD RES 149 253 986 PN 76086 RN 00440 AN 76215358 AU Mangos-J-A. Bargman-G-J. Martinez-J-R. Rennert-O-M. TI Physiology and pharmacology of secretion and cystic fibrosis. A review of past developments and projections into the future. pp. 311-36. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: pp. EXOCRINE-GLANDS: se. MN ADENOSINE-TRIPHOSPHATE: me. ANIMAL. BODY-FLUIDS: se. CALCIUM: me. CHLORIDES: me. DISEASE-MODELS-ANIMAL. EXOCRINE-GLANDS: ir. GLUCOSE: se. HUMAN. MEMBRANE-POTENTIALS. MICE. MONOGRAPH. NEURAL-TRANSMISSION. NEUROSECRETION. OSMOLAR-CONCENTRATION. PANCREAS: se. POLYAMINES: ph. POTASSIUM: me. REVIEW. SALIVARY-GLANDS: se. SODIUM: me. SWEAT-GLANDS: se. SWEAT: se. UREA: se. WATER: me. EX Cystic fibrosis is a recessively inherited metabolic disease which is characterized by a universal dysfunction in the exocrine glands of affected individuals. This dysfunction results in abnormalities of the composition and/or the physiochemical behavior of the various exocrine gland products. Although significant advances have been made in our understanding of exocrine gland physiology and pharmacology, many questions remain unanswered and significant gaps exist in our knowledge of exocrine gland function. Perhaps clarification of these issues may lead to better understanding of the pathogenesis of cystic fibrosis and to development of better forms of therapy for the patients. The purpose of this article is to review past and present developments in this area of biomedical research and to point out areas of exocrine gland physiology and pharmacology where more knowledge is needed in order to clarify the nature of a disease like cystic fibrosis. Composition of the acinar fluid, secretory mechanisms, modification of ductal fluid, ionic dependence and energy requirements of the secretory process, calcium metabolism in exocrine glands, and exocrine gland dysfunction in cystic fibrosis are discussed. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 DI SANTAGNESE PA N ENGL J MED 277 1344 967 003 LOBECK CC IN: STANBURY JB 1605 972 005 MANGOS JA IN: MANGOS JA 221 973 006 MARTINEZ JR IN: DI SANTAGNESE PA 129 966 007 MARTINEZ JR PFLUEGERS ARCH 290 124 966 008 MARTINEZ JR PHYSIOLOGIST 10 244 967 009 MARTINEZ JE IN: BOTELHO SY 20 969 010 MANGOS JA PFLUEGERS ARCH 291 99 966 011 MANGOS JA AM J PHYSIOL 221 496 971 012 MANGOS JA AM J PHYSIOL 225 18 973 013 MANGOS JA AM J PHYSIOL 225 450 973 014 MANGOS JA PROC SOC EXP BIOL MED 146 321 974 015 SCHULZ IJ PFLUEGERS ARCH 284 360 965 016 SCHULZ IJ J CLIN INVEST 48 1470 969 017 SCHULZ IJ PFLUEGERS ARCH 308 277 969 018 YOUNG JA PFLUEGERS ARCH 291 85 966 019 YOUNG JA PFLUEGERS ARCH 327 285 971 020 PETERSEN OH IN: GIEBISCH G 207 971 021 PETERSEN OH PHIL TRANS R SOC LOND BIOL 262 307 971 022 PETERSEN OH IN: EMMELIN N 21 972 023 PETERSEN OH ACTA PHYSIOL SCAND SUPPL 381 1 972 024 SCRATCHERD T GUT 14 592 973 025 SWANSON CH J GEN PHYSIOL 62 407 973 026 PETERSEN OH J MEMBR BIOL 16 353 974 027 MANGOS JA AM J PHYSIOL 218 1329 970 028 MANGOS JA AM J PHYSIOL 224 1260 973 029 MANGOS JA AM J PHYSIOL 225 683 973 030 SCHNEYER CA IN: THORN NA 42 974 031 GARRETT JR IN: THORN NA 487 974 032 EMMELIN N HANDBOOK OF PHYSIOLOGY 2 967 033 BATZRI S J BIOL CHEM 248 361 973 034 POHTO P J ORAL THER PHARMACOL 4 467 968 035 BATZRI S J BIOL CHEM 248 356 973 037 MANGOS JA AM J PHYSIOL 229 553 975 038 MANGOS JA AM J PHYSIOL 229 560 975 039 MANGOS JA AM J PHYSIOL 229 566 975 040 THOMAS JE HANDBOOK OF PHYSIOLOGY 2 955 967 041 TAUSSIG LM J PEDIATR 79 1034 971 042 AMSTERDAM A PROC NAT ACAD SCI USA 69 3028 973 043 PETERSEN OH EXPERIENTIA 29 160 973 044 PETERSEN OH NATURE NEW BIOL 244 73 973 045 PETERSEN OH IN: USSING HH 478 973 046$ NISHIYAMA A J PHYSIOL (LOND) 283 145 974 047 NISHIYAMA A IN: THORN NA 216 974 048 NISHIYAMA A EXPERIENTIA 29 161 973 049 ROBISON GA CYCLIC AMP 971 050 GOLDBERG ND IN: WEISSMAN G 185 975 051 DOUGLAS WW BR J PHARMACOL 34 451 968 052 RASMUSSEN H SCIENCE 170 404 970 053 AMSTERDAM A J CELL BIOL 41 753 969 054 JAMIESON JD J CELL BIOL 48 503 971 055 KANNO T IN: THORN NA 278 974 056 BYRT PN BIOCHIM BIOPHYS ACTA 148 215 967 057 DREISBACH RH PROC SOC EXP BIOL MED 126 279 967 058 SCHRAMM M J BIOL CHEM 245 3225 970 059 PALADE GE SCIENCE 189 347 975 060 LIEBOW C AM J PHYSIOL 226 1077 974 061 LIEBOW C SCIENCE 189 472 975 062 YOUNG JA PFLUEGERS ARCH 295 157 967 063 YOUNG JA PFLUEGERS ARCH 319 185 970 064 FIELD MJ PFLUEGERS ARCH 345 207 973 065 SCHNEYER LH AM J PHYSIOL 215 664 968 066 SCHNEYER LH AM J PHYSIOL 217 1324 969 067 KNAUF H IN: GEIBISH G 198 970 068 FIELD MJ THESIS 972 069 MARTIN CJ PFLUEGERS ARCH 327 303 971 070 YOUNG JA PFLUEGERS ARCH 327 285 971 071 MARTIN CJ PFLUEGERS ARCH 341 131 971 072 MARTIN CJ IN: THORN NA 549 974 073 MARTINEZ JR EUR J PHARMACOL 18 375 972 074 DENNIS AR PROC ARUST PHYSIOL PHARMACOL 4 45 973 075 CASE RM IN: BOTELHO SY 39 969 076 JANOWITZ HD CIBA FOUND SYMP EXOCRINE PANC 115 962 077 CASE RM J PHYSIOL (LOND) 196 133 968 078 WORMSLEY KG GASTROENTEROLOGY 54 197 968 079 MANGOS JA AM J PHYSIOL 224 1235 973 080 PETERSEN OH J PHYSIOL (LOND) 210 205 970 081 DOUGLAS WW J PHYSIOL (LOND) 165 528 963 082 MARTINEZ JR EXPERIENTIA 28 167 972 083 SELINGER Z J BIOL CHEM 248 369 973 084 PETERSEN OH J PHYSIOL (LOND) 208 431 970 085 CASE RM J PHYSIOL (LOND) 235 75 973 086 MATTHEWS EJ J PHYSIOL (LOND) 231 283 973 087 BABAD H EUR J BIOCHEM 1 96 967 088 FEINSTEIN H EUR J BIOCHEM 13 158 970 089 RIDDERSTAP AS AM J PHYSIOL 216 547 969 090 CASE RM IN: THORN NA 344 974 091 WINDELER AS JR ARCH ORAL BIOL 11 1043 966 092 DREISBACH RH AM J PHYSIOL 196 645 959 093 NEILSEN SP J PHYSIOL (LOND) 223 685 972 094 DREISBACH RH BIOCHEM J 82 71 962 095 DREISBACH RH PROC SOC EXP BIOL MED 116 953 964 096 SCHNEYER LH AM J PHYSIOL 226 821 974 097 DI SANTAGNESE PA PEDIATRICS 12 549 953 098 MANGOS JA TEX REP BIOL MED 31 651 973 099 MANGOS JA SCIENCE 158 135 967 100 KAISER D PEDIATR RES 5 167 971 101 HADORN B CAN MED ASSOC J 98 377 968 102 JOHANSEN PG LANCET 1 455 968 103 CHERNICK WS J PEDIATR 59 890 961 104 CHERNICK WS J PEDIATR 65 694 964 105 CHERNICK WS ANN NY ACAD SCI 106 698 963 106 CHERNICK WS MOD PROBL PEDIATR 10 125 967 107 BARBERO GJ IN: DI SANTAGNESE PA 208 964 108 BARBERO GJ CF CLUB ABST 4 967 109 MANDEL ID AM J DIS CHILD 113 431 967 110 MANDEL ID AM J DIS CHILD 110 646 965 111 MARMAR J GASTROENTEROLOGY 50 551 966 112 GUGLER EC J PEDIATR 71 585 967 113 RUBIN LS J PEDIATR 63 1120 963 114 MANGOS JA PEDIATR RES 1 436 967 115 MANGOS JA PEDIATR RES 2 378 968 116 TAYLOR A PEDIATR RES 8 861 974 117 LUTZ RJ IN: GABELNICK HL 119 973 118 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 119 BETTELHEIM FA BIORHEOLOGY 8 129 971 120 LORIN MI BIORHEOLOGY 9 27 972 121 KHAN MA TEX REP BIOL MED 31 665 973 122 SELYE HR SCIENCE 133 44 961 123 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 124 MARTINEZ JR PEDIATR RES 9 470 975 125 MARTINEZ JR PEDIATR RES 9 463 975 126 IMMKEN L CF CLUB ABST 16 9 975 127 PIVETTA OH J HERED 64 301 973 131 SMITH T BIOCHEM J 84 292 962 132 ELFERINK J Z NATURFORSCH 30 117 975 133 FULLER D BIOCHEM J 150 557 975 134 RUSSELL DH GORDON RES CONF POLYAMINES 975 135 PROCTOR MS J INVEST DERMATOL 65 409 975 136 CASE RM IN: THORN NA 344 974 137 RASMUSSEN H SCIENCE 170 404 970 CT 1 DISANTAGNESE PA N ENGL J MED 295 534 976 2 DANN LG LANCET 2 405 978 3 HALLINAN F MED HYPOTHESES 7 793 981 4 MANGOS JA J DENT RES 60 19 981 5 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 6 HARE ER PEDIATR RES 19 938 985 7 SCHONI MH PEDIATR RES 19 47 985 PN 76087 RN 00441 AN 76215364 AU Dearborn-D-G. TI Water and electrolytes of exocrine secretions. pp. 179-91. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ BICARBONATES: me. CALCIUM: me. CYSTIC-FIBROSIS: me. EXOCRINE-GLANDS: se. SODIUM: me. WATER: me. MN BRONCHI: se. ESCHERICHIA-COLI: me. GLYCOPROTEINS: me. HUMAN. MONOGRAPH. MUCUS: se. CHOLECYSTOKININ: pd. POLYAMINES: me. REVIEW. SECRETIN: pd. SWEAT: se. TRACHEA: se. SUPPORT-U-S-GOVT-P-H-S. EX It has been recognized that the levels of water and electrolytes are deranged in many, if not all, of the exocrine secretions of cystic fibrosis patients. The sodium and chloride levels in serous secretions from CF patients are elevated. In contrast, the more protein-rich or mucoid, isotonic secretions (viz, from the pancreas and tracheobronchial mucus glands) are found to have sodium concentrations near normal or even somewhat decreased. However, these secretions have significantly reduced water content. The abnormalities in hypotonic secretions appear to rise from a decreased Na+ reabsorption in the ducts. The low water content of the isotonic secretions of CF patients appears to result from decreased secretion of water and electrolytes. Early studies on tracheobronchial secretions from CF patients led investigators to suggest that the primary defect may be a decrease in the levels of water and monovalent ions. In an attempt to provide a unifying hypothesis, it has been suggested that an increased calcium concentration in the various exocrine secretions leads to a "hyperpermeability" of the mucus linings and thus to an excessive loss of water and small ions. The concentration of calcium in exocrine secretions appears to follow the concentration of proteins and especially the mucous glycoproteins. It was suggested that calcium may be an important factor in the increased viscosity and insolubility of CF mucus. Organic ions are also potential perturbants of macromolecular structure. In addition to a possible perturbant role, polyamines have a potential role in water and electrolyte transport. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 DI SANTAGNESE PA N ENGL J MED 277 1287 967 003 JOHANSEN PG LANCET 1 455 968 004 MANGOS JA PEDIATR RES 1 436 967 005 MANGOS JA SCIENCE 158 135 967 006 MANGOS JA PEDIATR RES 2 378 968 007 KAISER D PEDIATR RES 5 167 971 008 MANGOS JA TEX REP BIOL MED 31 651 973 009 KAISER D EUR J CLIN INVEST 4 261 974 011 HADORN B AUST PAEDIATR J 4 8 968 012 HADORN B CAN MED ASSOC J 98 377 968 013 HADORN B J PEDIATR 73 39 968 014 ZOPPI G ACTA PAEDIATR SCAND 59 692 970 015 CHERNICK WS PEDIATRICS 24 739 959 016 CHERNICK WS ANN NY ACAD SCI 106 698 963 017 MATTHEWS LW AM REV RESPIR DIS 88 199 963 018 POTTER JL AM REV RESPIR DIS 96 83 967 019 KOPITO LE FERTIL STERIL 24 499 973 020 KOPITO LE FERTIL STERIL 24 512 973 021 GIBSON LE PEDIATRICS 48 695 971 022 BOAT TF PEDIATRICS 50 343 972 023 GOEBELL H KLIN WOCHENSCHR 48 1330 970 024 BOTELHO SY J PEDIATR 83 601 973 025 MANDEL ID CLIN PEDIATR 8 161 969 026 ULLRICH KJ IN: THORN NA 423 974 027 SELINGER Z BIOCHIM BIOPHYS ACTA 203 326 970 028 WALLACH D EUR J BIOCHEM 21 433 971 029 BLOMFIELD J ARCH DIS CHILD 48 267 973 030 WARNER RR J CELL BIOL 64 54 975 032 SATIR B J CELL BIOL 56 153 973 033 EMRICH HM PEDIATR RES 2 464 968 034 WIESMANN UN LANCET 2 510 972 035 GUGLER EC J PEDIATR 71 585 967 036 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 037 BOAT TF PEDIATR RES 8 531 974 038 WARTON KL BR MED J 3 683 971 039 KATCHALSKY A BIOPHYS J SUPPL 9 4 9 964 040 MANDEL ID AM J DIS CHILD 113 431 967 041 RULE AH FERTIL STERIL 21 515 970 042 LIAPPIS N CLIN CHIM ACTA 48 233 973 043 LEWIS RW LIPIDS 6 859 971 044 MCEVOY FA PEDIATR RES 9 721 975 048 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 049 ZELLER EA IN: BOYER PD 8 313 963 051 MUNRO GF J BIOL CHEM 247 1272 972 052 SEILER N HOPPE SEYLERS Z PHYSIOL CHEM 354 589 973 053 TABOR H ADV ENZYMOL 36 203 972 054 JAKOWSKA S CF AND RELATED HUMAN AND ANIM 970 055 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 056 LOBECK CC MOD PROBL PEDIATR 10 41 967 057 DI SANTAGNESE PA MOD PROBL PEDIATR 10 54 967 058 GIBBS GE IN: MCINTOSH R 145 960 059 PAUNIER L PEDIATRICS 52 446 973 060 CHAUNCEY HH ARCH ORAL BIOL 7 707 962 061 WOTMAN S ARCH ORAL BIOL 16 663 971 062 BLOMFIELD J AUST PAEDIATR J 10 75 974 063 MANDEL ID AM J DIS CHILD 110 646 965 064 CHERNICK WS J PEDIATR 59 890 961 065 SHWACHMAN H ANN NY ACAD SCI 93 600 962 066 DI SANTAGNESE PA PEDIATRICS 22 507 958 067 BRAND-AURABAN A J INVEST DERMATOL 58 14 972 068 STOLL E HELV PAEDIATR ACTA 25 234 970 069 KOPITO L J PEDIATR 68 313 966 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 3 SEALE TW PEDIATR RES 14 1398 980 4 WILL PC PEDIATR RES 14 1245 980 5 BOAT TF FED PROC 39 3067 980 6 WELCH MJ PEDIATRICS 67 664 981 7 KNOWLES M N ENGL J MED 305 1489 981 8 SORSCHER EJ LANCET 1 368 982 9 SEALE TW ANN CLIN LAB SCI 12 415 982 10 BOGART BI PEDIATR RES 16 223 982 11 KNOWLES M J CLIN INVEST 71 1410 983 12 CHACE KV CLIN CHIM ACTA 132 143 983 13 QUINTON PM N ENGL J MED 308 1185 983 14 DEARBORN DG N ENGL J MED 309 796 983 15 BOUCHER RC PEDIATR RES 18 1336 984 16 CROWTHER RS BIORHEOLOGY 21 253 984 17 KNOWLES MR PEDIATR RES 19 676 985 18 COLLIE G IN VITRO CELL DEVEL BIOL 21 597 985 19 KNOWLES MR CLIN CHEST MED 7 285 986 20 BOUCHER RC J CLIN INVEST 78 1245 986 21 FEIGAL RJ ANN NY ACAD SCI 488 82 986 PN 76088 RN 00442 AN 76215359 AU Lederberg-S. TI Polypeptide mediators of cystic fibrosis. pp. 259-71. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: me. PEPTIDES: me. MN ANIMAL. BIOLOGICAL-ASSAY. BLOOD-PROTEINS: an, me. CELLS-CULTURED. COMPLEMENT-3: me. COMPLEMENT-5: me. DISEASE-MODELS-ANIMAL. HETEROZYGOTE. HUMAN. IGG. MODELS-BIOLOGICAL. MOLECULAR-WEIGHT. MONOGRAPH. PEPTIDES: an. PROTEINURIA. RATS. REVIEW. SALIVA: me. EX Although complex models involving multiple loci may be plausible, the high frequency of cystic fibrosis in Caucasians is probably best explained by selective advantage for the heterozygote carrier. In short, an analogy of cystic fibrosis with hemoglobin S sickle cell anemia may be greater than with Tay-Sachs disease in terms of molecular mechanisms. Protein and polypeptide differences have been reported for the humoral fluids of cystic fibrosis patients and their heterozygotic relatives in connected with selected bioassay systems. These directions relate to a model here proposed for the intermediate stages of the physiology of cystic fibrosis, which builds on a large body of elegant experimental work. The basic tenet is that the cells of cystic fibrosis heterozygotes and normal individuals are in a stimulatory loop. Chronic reserpine treatment exaggerates in the normal rat a loop similar to the one exaggerated by genetic differences in the human, elevating to pathological levels an otherwise normal process. RF 001 STEINBERG AG AM J HUM GENET 8 162 956 002 DI SANTAGNESE PA N ENGL J MED 277 1287 967 003 LOBECK CC IN: STANBURY JB 1605 972 004 MANGOS JA FUNDAMENTAL PROB CF AND RELAT 973 005 MCCOMBS ML TEX REP BIOL MED 31 615 973 006 COHEN SS INTRODUCTION TO POLYAMINES 971 007 LEDERBERG S GAP CONF REP STRAT GEN DIS 15 972 008 RENNERT OM IN: MANGOS JA 41 973 010 MUNRO GF CF CLUB ABST 16 7 975 011 BDOLAH A BIOCHEM BIOPHYS RES COMMUN 18 452 965 012 CECCARELLI B ADV CYTOPHARMACOLOGY 2 974 013 RUSSELL DH PROC NAT ACAD SCI USA 72 1482 975 014 HSIE AW PROC NAT ACAD SCI USA 68 358 971 015 WHITFIELD JF IN VITRO 8 257 973 016 RASMUSSEN H CRIT REV BIOCHEM 1 95 972 017 SCHULTZ G PROC NAT ACAD SCI USA 70 3889 973 018 DANKS DM ANN HUM GENET 28 323 965 019 KNUDSON AG JR AM J HUM GENET 19 388 967 020 CONNEALLY PM TEX REP BIOL MED 31 639 973 021 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 022 SPOCK A PEDIATR RES 1 173 967 023 BOWMAN BH SCIENCE 164 325 969 024 BESLEY GTN J MED GENET 6 278 969 025 DANES BS J EXP MED 136 1313 972 026 SCHMOYER IR LIFE SCI PART 2 11 1037 972 027 CONOVER JH PEDIATR RES 7 220 973 028 MANGOS JA SCIENCE 158 135 967 029 MANGOS JA PEDIATR RES 1 436 967 030 KAISER D PEDIATR RES 5 167 971 031 KAISER D LANCET 1 1003 970 032 BOWMAN BH SCIENCE 167 871 970 033 BARNETT DR TEX REP BIOL MED 31 709 973 034 HERZBERG V J CLIN INVEST 52 2732 973 035 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 036 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 037 BARNETT DR TEX REP BIOL MED 31 703 973 038 DANES BS J EXP MED 137 1538 973 039 BARNETT DR TEX REP BIOL MED 31 691 973 040 BARNETT DR TEX REP BIOL MED 31 697 973 041 DOGGETT RG CF CLUB ABST 41 971 042 MCNEELY CM CF CLUB ABST 16 6 975 043 MANGOS JA PEDIATR RES 2 378 968 044 POLLEY MJ J MED GENET 11 249 974 045 CONOVER JH PEDIATR RES 7 224 973 046 BERATIS NG PEDIATR RES 7 958 973 047 CONOVER JH LIFE SCI 14 253 974 048 CONOVER JH CLIN RES 21 531 973 049 CONOVER JH LANCET 1 1194 973 050 CONOVER JH LANCET 2 1501 973 051 LIEBERMAN J LANCET 1 1230 974 052 LIEBERMAN J AM REV RESPIR DIS 111 100 975 053 HANN S LANCET 2 520 974 054 CONOVER JH LANCET 1 47 975 055 CONOVER JH PEDIATR RES 9 394 975 056 RAO GJS J PEDIATR 80 573 972 057 RAO GJS PEDIATR RES 8 684 974 058 LIEBERMAN J AM REV RESPIR DIS 109 399 974 059 LIEBERMAN J PEDIATR RES 3 571 969 060 RAO GJS SCIENCE 177 610 972 061 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 062 ADSHEAD PC ANN NY ACAD SCI 253 192 975 063 KATCHALSKY A BIOPHYS J SUPPL 9 4 9 964 064 QUINTON PM J CELL BIOL 56 787 973 065 KRAUS I PEDIATRICS 47 1010 971 066 ANTONOWICZ I PEDIATR RES 6 803 972 067 ESTENSEN RD NATURE 248 347 974 068 GOLDSTEIN IM BIOCHEM BIOPHYS RES COMMUN 60 807 974 069 BARTMAN J J PEDIATR 76 430 970 070 AMSTERDAM A J CELL BIOL 41 753 969 071 PICKERING R ARCH ORAL BIOL 20 153 975 CT 1 DISANTAGNESE PA N ENGL J MED 295 534 976 2 WANNER A AM REV RESPIR DIS 116 73 977 PN 76089 RN 00443 AN 76215354 AU Barbero-G-J. Shwachman-H. Grand-R. Woodruff-C. TI Gastrointestinal and nutritional manifestations of cystic fibrosis. pp. 83-111. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: co. GASTROINTESTINAL-DISEASES: et. NUTRITION-DISORDERS: et. MN AMINO-ACIDS: me. BODY-COMPOSITION. CYSTIC-FIBROSIS: dh. DIABETES-MELLITUS: et. DIETARY-FATS: me. GALLBLADDER: pa. GASTROINTESTINAL-MOTILITY. GROWTH. HUMAN. INTESTINAL-ABSORPTION. INTESTINAL-OBSTRUCTION: et. INTUSSUSCEPTION: et. LACTOSE-INTOLERANCE: et. LIVER-CIRRHOSIS: et. MONOGRAPH. NITROGEN: me. PANCREATIC-DISEASES: et. REVIEW. TRIGLYCERIDES: me. VITAMINS: me. EX The purpose of this paper is to summarize the present state of our knowledge of the gastrointestinal manifestations and absorption and metabolism of nutrients in patients with cystic fibrosis. The gastrointestinal manifestations include pancreatic exocrine insufficiency, pancreatic calcification and stones, diabetes, liver and gall bladder abnormalities, intestinal obstruction, and complications of malabsorption. Nutritional aspects of cystic fibrosis include growth retardation and body composition, fat and nitrogen absorption, amino acid absorption, medium-chain triglyceride absorption, and deficiency of fat-soluble vitamins. The critical questions for projection into the future fall into three categories: intestinal absorption, gastrointestinal function and experimental design. RF 001 SHWACHMAN H ADV PEDIATR 7 249 955 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 FARBER S ARCH PATHOL 37 238 944 004 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 005 SHWACHMAN H PEDIATR CLIN NORTH AM 1 389 954 006 SHINER M LANCET 1 17 956 007 DI SANTAGNESE PA PEDIATRICS 12 549 953 009 COZZETTO FJ PEDIATRICS 32 228 963 011 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 012 GRAND RJ CLIN PEDIATR 9 588 970 013 KOPEL FB GASTROENTEROLOGY 62 483 972 014 BERK RN RADIOLOGY 106 377 973 015 TUCKER AS AM J ROENTG RAD THER NUCL MED 89 1048 963 016 MADDOCK CL AM J DIS CHILD 66 370 943 017 SHWACHMAN H PEDIATRICS 55 86 975 018 HADORN B J PEDIATR 73 39 968 019 SHWACHMAN H AM J DIS CHILD 92 347 956 020 JOHANSEN PG LANCET 1 455 968 022 BEARDMORE M BR MED J 2 1383 966 023 DI SANTAGNESE PA N ENGL J MED 277 1287 967 024 HANDWERGER S N ENGL J MED 281 451 969 025 ROSAN RC AM J DIS CHILD 104 625 962 026 ROSAN RC METABOLISM 12 553 963 027 CRAIG JM AM J DIS CHILD 93 357 957 028 DI SANTAGNESE PA PEDIATRICS 18 387 956 029 OPPENHEIMER EH J PEDIATR 86 683 975 030 KATTWINKEL J J PEDIATR 82 234 973 031 LEBENTHAL E GASTROENTEROLOGY 67 807 974 032 GREEN MN AM J DIS CHILD 100 365 960 033 FEIGELSON J ACTA PAEDIATR SCAND 61 337 972 034 TYSON KRT J PEDIATR SURG 3 271 968 035 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 036 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 037 WEBER AM N ENGL J MED 289 1001 973 038 WATKINS JB GASTROENTEROLOGY 68 1087 975 039 PARKINS RA LANCET 2 851 963 040 SHWACHMAN H N ENGL J MED 286 1300 972 041 HOLSCLAW DS J PEDIATR SURG 9 867 974 042 ALTERMAN K AM J DIS CHILD 101 210 961 043 DONNISON AB PEDIATRICS 37 833 966 044 HOLSCLAW DS AM J DIS CHILD 109 101 965 045 NEUHAUSER EBD AM J ROENTG RAD THER 51 421 944 046 BERNSTEIN JG AM J DIS CHILD 99 804 960 047 OPPENHEIMER EH PEDIATR RES 7 339 973 048 NOBLETT HR J PEDIATR SURG 4 190 969 049 WAGGET J J PEDIATR 77 407 970 050 KOPITO L J PEDIATR 68 313 966 051 GREEN MN PEDIATRICS 21 635 958 052 GREEN MN PEDIATRICS 41 989 968 053 STEPHAN U PEDIATRE 10 63 974 054 STEPHAN U PEDIATRICS 55 35 975 055 BROWN PM N ENGL J MED 263 544 960 056 HUNTON DB GASTROENTEROLOGY 50 99 966 057 LILLIBRIDGE CB J PEDIATR 71 887 967 058 GRACEY M ARCH DIS CHILD 44 404 969 059 MULLINS F JAMA 192 741 965 060 ANDERSON CM PEDIATRIC GASTROENTEROLOGY 975 061 HOLSCLAW DS PEDIATRICS 48 51 971 062 KULCZYCKI LL N ENGL J MED 259 409 958 063 SHAHIDI NT J PEDIATR 59 533 961 064 PITTMAN FE AM J DIS CHILD 108 360 964 065 FLEISHER DS J PEDIATR 64 349 964 066 DOLAN TF JR CLIN PEDIATR 9 295 970 067 TORSTENSON OL PEDIATRICS 45 857 970 068 ANTONOWICZ I PEDIATRICS 42 492 968 069 GIBBONS ISE ARCH DIS CHILD 44 63 969 070 LEBENTHAL E AM J CLIN NUTR 28 595 975 071 ANTONOWICZ I PEDIATRICS 49 847 972 072 LLOYD-STILL JD PEDIATRICS 53 678 974 073 SHWACHMAN H PEDIATRICS 46 3 970 074 LLOYD-STILL JD PEDIATRICS 54 306 974 075 LAPEY A J PEDIATR 84 328 974 076 DOOLEY RR J PEDIATR 74 95 969 078 SHOHL AT J PEDIATR 23 267 943 079 ANDERSEN DH AM J DIS CHILD 69 221 945 080 CHUNG AW PEDIATRICS 7 491 951 081 ROSS CAC ARCH DIS CHILD 30 316 955 082 HARRIS R ARCH DIS CHILD 30 424 955 083 JENSEN SB ACTA PAEDIATR SCAND 45 70 956 084 SHMERLING DH PEDIATRICS 46 690 970 086 NIESSEN KH KLIN PAEDIATR 185 271 973 087 KATTWINKEL J PEDIATRICS 50 133 972 088 SOONG CS GASTROENTEROLOGY 63 748 972 089 GIARDINI O N ENGL J MED 286 1059 972 090 DEREN JJ N ENGL J MED 288 949 973 091 TONZ O MOD PROBL PEDIATR 10 259 967 092 WEISS S ARCH KINDERHEILK 174 254 966 093 ROMANO C LANCET 1 657 966 094 WEST CD AM J DIS CHILD 72 251 946 095 CHRISTENSEN HN J CLIN INVEST 28 319 949 096 GOULD BS AM J DIS CHILD 91 584 956 097 ANFANGER H AM J DIS CHILD 77 425 949 098 MORIN CL BIOMEDICINE EXPRESS 19 133 973 099 SEAKINS JWT IN: LAWSON D PROC 5TH INT CF 275 969 100 FERNANDES JH J CLIN INVEST 41 488 962 101 HUANG NN IN: SENIOR JR 207 968 102 GRACEY M IN: LAWSON D PROC 5TH INT CF 288 969 103 UNDERWOOD BA PEDIATR RES 6 26 972 104 SMITH FR J LAB CLIN MED 80 423 972 105 KATTWINKEL J J PEDIATR 82 234 973 106 MULLER DPR BIOCHEM J 112 28P 969 107 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 108 BLANC WA PEDIATRICS 22 494 958 109 DARBY CW ARCH DIS CHILD 48 72 973 110 FARRELL PM ACTA PAEDIATR SCAND 64 150 975 111 HARRIES JT ARCH DIS CHILD 46 341 971 112 DOLAN TF JR J PEDIATR 77 515 970 113 WALTERS TR AM J DIS CHILD 124 641 972 114 LUKENS JN AM J DIS CHILD 124 639 972 115 ALLAN JD AM J DIS CHILD 126 22 973 116 BERRY HK AM J DIS CHILD 129 165 975 117 SHERMAN JO J PEDIATR 86 518 975 118 FILLER RM N ENGL J MED 281 589 969 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 SUPER M S AFR MED J 52 991 977 3 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 4 BENDIG DW J PEDIATR 100 536 982 5 MISCHLER EH AM J DIS CHILD 136 1060 982 6 SUPER M LANCET 2 1200 983 7 JEFFREY I J CLIN PATHOL 36 1292 983 8 WELKON CJ AM J DIS CHILD 139 805 985 PN 76090 RN 00444 AN 76215353 AU Bowman-B-H. TI Factors related to cystic fibrosis. pp. 277-84. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS. PROTEINS. MN ANIMAL. BIOLOGICAL-ASSAY. CELLS-CULTURED. CILIA. COMPLEMENT-1. COMPLEMENT-3. CYSTIC-FIBROSIS: bl, fg, ur. DOGS. GENES. GLYCOPROTEINS. HUMAN. IMMUNOGLOBULINS. MEMBRANE-POTENTIALS. MOLECULAR-WEIGHT. MONOGRAPH. REVIEW. SALIVA. EX A variety of bioassays have identified molecules circulating in cystic fibrosis genotypes and synthesized in vitro by cultured cells from these genotypes by functional disturbances observed in the assays employed. Preliminary characterizations of these factors are in general agreement and the properties include a small molecular weight polypeptide within the range of 4000 to 10000 MW, a heat-labile protein having a cationic charge, and a protein present in saliva, serum, urine (and perhaps sweat) which is synthesized in vitro by fibroblasts, short-term lymphocytes and long-term lymphoid lines. The relationship of the factor or factors to the pathogenesis of cystic fibrosis is presently unknown, but its presence in cystic fibrosis genotypes appears to be related to the basic defect. RF 001 MANGOS JA SCIENCE 158 135 967 002 MANGOS JA PEDIATR RES 1 436 967 003 MANGOS JA TEX REP BIOL MED 31 651 973 004 SPOCK A PEDIATR RES 1 173 967 005 BESLEY GTN J MED GENET 6 278 969 006 BOWMAN BH SCIENCE 164 325 969 007 BOWMAN BH SCIENCE 167 871 970 008 DANES BS J EXP MED 136 1313 972 009 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 010 MCNEELY CM CF CLUB ABST 16 6 975 011 CONOVER JH PEDIATR RES 7 220 973 012 CONOVER JH PEDIATR RES 7 224 973 013 BERATIS NG PEDIATR RES 7 958 973 014 BOWMAN BH CLIN GENET 4 461 973 015 SCHMOYER IR LIFE SCI PART 2 11 1037 972 016 BARNETT DR TEX REP BIOL MED 31 709 973 017 HERZBERG V J CLIN INVEST 52 2732 973 018 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 019 CONOVER JH LIFE SCI 14 253 974 020 BARNETT DR TEX REP BIOL MED 31 703 973 021 DANES BS J EXP MED 137 1538 973 022 BARNETT DR TEX REP BIOL MED 31 697 973 023 WILSON GB CLIN CHIM ACTA 49 79 973 024 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 025 BOWMAN BH FED PROC 31 842 972 026 KAHN MA TEX REP BIOL MED 31 665 973 027 COHEN FL J MED GENET 11 253 974 028 WILSON GB LIFE SCI 15 551 974 029 POLLEY MJ J MED GENET 11 249 974 CT 1 WILSON GB PEDIATR RES 11 317 977 2 IMPERO JE PEDIATR RES 12 108 978 3 SEALE TW J CLIN MICROBIOL 9 72 979 4 WILSON GB MED HYPOTHESES 8 527 982 5 SEALE TW ANN CLIN LAB SCI 12 415 982 PN 76091 RN 00445 AN 76215352 AU Nadler-H-L. TI Enzyme studies. pp. 285-90. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: en. ESTERASES: me. PEPTIDE-PEPTIDOHYDROLASES: me. MN ARGININE. ESTERASES: df. KALLIKREIN: me. MONOGRAPH. PAROTID-GLAND: en. REVIEW. SALIVA: en. TRYPSIN-INHIBITORS: pd. EX It has been reported that saliva and serum of patients contain macromolecular "factors" which are presumably cationic and inhibit transport of electrolytes, induce dyskinesis or degranulate sensitized polymorphonuclear leukocytes and contain polypeptide moieties. The difference between levels of trypsin-like activity of mixed saliva of patients with cystic fibrosis and controls has been confirmed. A number of studies have been carried out in order to determine if a deficiency of proteolytic activity might account for the presence of cystic fibrosis serum factors. It was presumed that the observed partial deficiency of arginine esterase activity might well represent the total deficiency of one of several arginine esterases assayed under the experimental conditions. Although it was postulated that the deficiency of arginine esterase activity is related to the elevation of the levels of polypeptide or protein "factors", the hydrolysis of peptide bands by activated plasma could not be demonstrated, probably due to the limitations of the methods employed. The deficiency of plasma arginine esterase in cystic fibrosis has been questioned and confirmed in another laboratory. RF 001 MANGOS JA SCIENCE 158 135 967 002 MANGOS JA PEDIATR RES 1 436 967 003 SPOCK A PEDIATR RES 1 173 967 004 BOWMAN BH SCIENCE 164 325 969 005 BOWMAN BH SCIENCE 167 871 970 007 DOGGETT RG TEX REP BIOL MED 31 685 973 008 MANGOS JA PEDIATR RES 2 378 968 009 RAO GJS J PEDIATR 80 573 972 010 RAO GJS SCIENCE 177 610 972 011 SCHONI M EUR J CLIN INVEST 5 153 975 012 TAYLOR A PEDIATR RES 8 861 974 013 RAO GJS PEDIATR RES 8 684 974 014 RAO GJS PEDIATR RES 9 739 975 015 COLMAN RW J CLIN INVEST 48 23 969 016 COLMAN RW PROG HEMATOL 7 255 971 017 BARRETT AJ BIOCHEM J 131 809 973 018 GLOVER G J BIOL CHEM 246 4594 971 019 GROSKOPF WR J BIOL CHEM 244 359 969 020 CHASE T JR BIOCHEMISTRY 8 2212 969 021 TALAMO RC PEDIATR RES 6 430 972 022 LIEBERMAN J PEDIATR RES 3 571 969 023 LIEBERMAN J AM REV RESPIR DIS 109 399 974 024 COBURN MD AM REV RESPIR DIS 110 368 974 025 CONOVER JH LIFE SCI 14 253 974 026 LIEBERMAN J AM REV RESPIR DIS 111 100 975 CT 1 HALLINAN F MED HYPOTHESES 7 793 981 2 DANDONA P THORAX 36 60 981 3 WILSON GB MED HYPOTHESES 8 527 982 PN 76092 RN 00446 AN 76215351 AU Liebman-J. Lucas-R-V. Moss-A. Rosenthal-A. TI Cor pulmonale and related cardiovascular effects of cystic fibrosis. pp. 41-80. SO In: Mangos JA, Talamo RC, ed. Cystic fibrosis: projections into the future. New York, Stratton, 1976. WI 820 C9995 1976. (REVIEW). MJ CYSTIC-FIBROSIS: co. PULMONARY-HEART-DISEASE: et. MN ADOLESCENCE. ADULT. CHILD. DIGOXIN: tu. DIURETICS: tu. ECHOCARDIOGRAPHY. FUROSEMIDE: tu. HEART-ENLARGEMENT: et. HEART: pp. HUMAN. MONOGRAPH. MYOCARDIAL-CONTRACTION. MYOCARDIUM: pa. OXYGEN-INHALATION-THERAPY. OXYGEN: bl. PROGNOSIS. PULMONARY-HEART-DISEASE: di, th. RESPIRATION. REVIEW. TOLAZOLINE: tu. VASCULAR-RESISTANCE. VECTORCARDIOGRAPHY. EX Cor pulmonale was defined by a committee of the World Health Organization as "right ventricular hypertrophy, secondary to disease of the lung parenchyma or pulmonary vasculature, or due to abnormalities of pulmonary function." It has been estimated that 70% of children dying with cystic fibrosis have cor pulmonale, though as children live longer, the number may be higher. Clinical diagnosis, management, prognosis, echocardiography, echocardiography as a measure of left ventricular dysfunction, and a study of 94 ambulatory and hospitalized cystic fibrosis patients are discussed. The study establishes that in patients with cystic fibrosis, left ventricular dysfunction and hypertrophy occur and are related to increasing severity of pulmonary involvement. RF 001 ANON CIRCULATION 27 594 963 002 FISHMAN AP HOSP PRACT 6 101 971 003 SIASSI B J PEDIATR 78 794 971 004 SHWACHMAN H AM J DIS CHILD 96 6 958 005 MOSS AJ J PEDIATR 67 797 965 006 FERRER MI BULL NY ACAD MED 41 942 965 007 LIEBMAN J CIRCULATION 35 552 967 008 LIEBMAN J IN: MOSS AJ 183 968 009 LIEBMAN J CHEST 63 218 973 010 HAYES CJ PEDIATRICS 52 561 973 011 WILLIAMS JF JR AM J CARDIOL 16 534 965 012 CROWELL JW AM J PHYSIOL 206 313 964 013 HERNANDEZ A AM J DIS CHILD 115 576 968 014 MOSS AJ J PEDIATR 86 295 975 015 WHITMAN V PEDIATRICS 55 83 975 016 CAMPBELL EJM LANCET 1 1184 960 017 NOBLE MIM LANCET 2 257 966 019 GOLDRING RM J PEDIATR 65 501 964 020 KELMINSON LL PEDIATRICS 39 24 967 021 FISHER BJ CF CLUB ABST 971 022 FISHER BJ PROC AM PEDIATR SOC ANNU MTG 299 971 026 SAKAMOTO T JAPAN HEART J 15 360 974 027 HIRSCHFELD S CIRCULATION 51 304 975 028 HIRSCHFELD S CIRCULATION 52 641 975 029 BISHOP JM MED CLIN NORTH AM 57 771 973 030 FISHMAN AP N ENGL J MED 285 402 971 031 FRANK MJ CIRCULATION 47 798 973 032 DAVIES H CHEST 58 8 970 033 WILLIAMS JF JR J CLIN INVEST 47 1143 968 034 RAO BS AM J MED 45 229 968 035 BAUM GL N ENGL J MED 285 361 971 036 BLAND JW JR AM J CARDIOL 23 830 969 037 LUKE MJ PEDIATRICS 37 762 966 038 BARNES GL AUST PAEDIATR J 6 81 970 039 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 040 HOLMAN RL PEDIATRICS 24 34 959 041 WEISSLER AM AM HEART J 62 367 961 042 WEISSLER AM AM J CARDIOL 23 577 969 043 WEISSLER AM AM J CARDIOL 15 153 965 044 WEISSLER AM AM J MED SCI 259 4 970 045 LEVY AM CIRCULATION 46 816 972 046 GOLDE D CIRCULATION 42 1029 970 047 HARRIS LC AM J CARDIOL 14 448 964 048 MARTIN CE CIRCULATION 44 419 971 049 GARRARD CL JR CIRCULATION 42 455 970 050 WALLACE AG CIRC RES 12 611 963 051 WEISSLER AM CIRCULATION 37 149 968 052 WEISSLER AM AM J CARDIOL 17 768 966 053 FEIGENBAUM H ECHOCARDIOGRAPHY 972 054 FEIGENBAUM H ARCH INTERN MED 129 461 972 055 GIBSON DG BR HEART J 35 128 973 056 FORTUIN NJ CIRCULATION 44 575 971 057 MURRAY JA AM J CARDIOL 30 252 972 058 ABBASI AS N ENGL J MED 289 118 973 059 COOK CD J PEDIATR 59 710 961 060 MURRAY AB J PEDIATR 62 186 963 061 GOODMAN DJ AM J CARDIOL 33 438 974 062 GRAHAM TP JR PEDIATR CLIN NORTH AM 18 1109 971 063 MARSHALL RJ CARDIAC FUNCTION IN HEALTH AN 968 064 TAJIK AJ CIRCULATION 46 36 972 065 ZAKY A CIRCULATION 37 789 968 066 POPP RL CIRCULATION 49 428 974 067 ZIMMERMAN HA DIS CHEST 20 286 951 068 KELLY DT CIRCULATION 44 403 971 069 ALPERT JS CIRCULATION 50 317 974 070 FLUCK DC BR HEART J 28 92 966 071 MICHELSON N DIS CHEST 38 435 960 072 BOWDEN DH AM J MED 38 226 965 073 ROWLATT UF PEDIATR CLIN NORTH AM 10 499 963 074 DUCHAK JM JR AM J CARDIOL 29 628 972 075 MCLAURIN LP CIRCULATION 48 801 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 HIRSCHFELD SS CHEST 75 351 979 3 CHIPPS BE J PEDIATR 95 379 979 4 MATTHAY RA BR HEART J 43 474 980 5 MORIN DP PEDIATRICS 65 44 980 6 STERN RC AM J DIS CHILD 134 267 980 7 FOWLER RS J ELECTROCARDIOL 14 319 981 8 MATTHAY RA MED CLIN NORTH AM 65 489 981 9 JACOBSTEIN MD CHEST 80 399 981 10 NEWTH CJL AM REV RESPIR DIS 124 463 981 11 MOSS AJ PEDIATRICS 70 728 982 12 GOODING CA J PEDIATR 105 384 984 13 BENSON LN AM REV RESPIR DIS 130 987 984 14 CANNY GJ AM REV RESPIR DIS 130 822 984 15 STANBROOK HS AM REV RESPIR DIS 130 81 984 16 GEGGEL RL AM REV RESPIR DIS 131 531 985 17 RUTISHAUSER M WIEN KLIN WOCHENSCHR 98 728 986 PN 76093 RN 00447 AN 76236895 AU Hann-S. Holsclaw-D-S. TI Interactions of Pseudomonas aeruginosa with immunoglobulins and complement in sputum. SO Infect-Immun. 1976 Jul. 14(1). P 114-7. MJ COMPLEMENT-3. COMPLEMENT. CYSTIC-FIBROSIS: im. IMMUNOGLOBULINS. PSEUDOMONAS-AERUGINOSA: im. SPUTUM: im. MN ADOLESCENCE. ADULT. ANTIGEN-ANTIBODY-REACTIONS. CHILD. CHILD-PRESCHOOL. COMPLEMENT-3: an. COMPLEMENT: an. CYSTIC-FIBROSIS: mi. FEMALE. HUMAN. IGA: an. IGG: an. IGM: an. IMMUNOGLOBULINS: an. MALE. SPUTUM: mi. AB The interactions of Pseudomonas aeruginosa with humoral factors in the sputum of patients with cystic fibrosis were investigated by using an indirect immunofluorescent technique. Fluorescein- conjugated, monovalent antiserum specific to heavy chains of human immunoglobulin A (IgA), IgG, or IgM and to complement C3 were used. All strains of P. aeruginosa recovered from the sputum specimens of patients with cystic fibrosis were found to be coated with antibodies of IgA, IgG, and IgM classes and with C3. The specificity of the antibody coating was determined. The fluorescence was most intense with IgA and was followed in intensity by IgG, IgM, and C3. No difference was noted between rough and mucoid strains of P. aeruginosa. When the subcultured P. aeruginosa was incubated with the sputum eluates, a similar pattern of fluorescence was demonstrated, indicating that these humoral factors are present in the sputum and that the coating process can take place in the lower respiratory tract of the patients. By single radial immunodiffusion, significant quantities of the humoral factors in the sputum eluates were detected. These findings suggest that P. aeruginosa is opsonized in sputum of patients with cystic fibrosis. RF 001 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 002 BOXERBAUM B AM REV RESPIR DIS 108 777 973 003 BOYDEN SV IN: WOLSTENHOLME GEW 190 976 004 GOTZE O J EXP MED 134 90S 971 005 GREEN GM N ENGL J MED 276 421 967 006 GREEN GM J CLIN INVEST 43 769 964 007 GUGLER EC J PEDIATR 73 548 968 008 HUANG NN J PEDIATR 59 512 961 009 IACOCCA VF AM J DIS CHILD 106 315 963 010 ISHIZAKA T J IMMUNOL 97 716 966 011 JOHNSTON RB JR J EXP MED 129 1275 969 012 MARCER V RIV CLIN PEDIATR 83 135 970 013 OREN R J CELL BIOL 17 486 963 014 REYNOLDS HY GAP CONF REP PSEUDOMON IN CF 7 974 015 SMITH MR PROC NAT ACAD SCI USA 63 1151 969 016 SOUTH MA J PEDIATR 71 645 967 017 THOMAS V N ENGL J MED 290 588 974 018 WILLIAMS RC JR IMMUNOLOGY 17 249 969 019 WINKELSTEIN JA J PEDIATR 82 747 973 020 YOUNG LS J INFECT DIS 126 257 972 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 SORENSEN RU INFECT IMMUN 18 735 977 3 SORENSEN RU J PEDIATR 93 201 978 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 5 SHAW JO LAB INVEST 42 547 980 6 HOMMA JY JAP J EXP MED 50 149 980 7 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 8 KOLB WP AM REV RESPIR DIS 123 226 981 9 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 10 OLIVER AM J CLIN LAB IMMUNOL 10 221 983 11 SCHILLER NL PEDIATR RES 17 747 983 12 SORENSEN RU EUR J RESPIR DIS 64 524 983 13 PIER GB J INFECT DIS 150 223 984 14 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 15 PIEDRA P J PEDIATR 108 817 986 PN 76094 RN 00448 AN 76238105 AU Tavormina-J-B. Kastner-L-S. Slater-P-M. Watt-S-L. TI Chronically ill children. A psychologically and emotionally deviant population?. SO J-Abnorm-Child-Psychol. 1976. 4(2). P 99-110. MJ ADAPTATION-PSYCHOLOGICAL. CHRONIC-DISEASE. SOCIAL-ADJUSTMENT. MN ACHIEVEMENT. ADOLESCENCE. ADULT. ASTHMA: co. CHILD-BEHAVIOR-DISORDERS: co. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DEFENSE-MECHANISMS. DEPENDENCY-PSYCHOLOGY. DIABETES-MELLITUS-INSULIN-DEPENDENT: co. FEMALE. HEARING-DISORDERS: co. HUMAN. INTERNAL-EXTERNAL-CONTROL. MALE. PERSONAL-SATISFACTION. PERSONALITY-INVENTORY. PROJECTIVE-TECHNICS. SELF-CONCEPT. SEX-FACTORS. SOCIAL-DESIRABILITY. AB The study evaluated the psychosocial functioning levels of a group of chronically ill (diabetic, asthmatic, cystic fibrotic, and hearing- impaired) children across a battery of standardized personality instruments. The assessments were performed to provide a rigorous test of the popular hypothesis that chronically ill children are especially vulnerable to psychopatholgy. In contrast to this sterotype, results across measures demonstrated the normalcy rather than the deviance of these children. Although exceptions were noted, the children's functional strengths and coping abilities noticeably outweighed their weaknesses. RF 001 BAKWIN H BEHAVIOR DISORDERS IN CHILDRE 972 002 DILLER L IN: WOLMAN BB 972 003 EYSENCK SBG JR EYSENCK PERSONAL INVENTOR 963 004 FARBER B SOCIOMETRY 20 117 957 005 FARBER B CHILD DEVELOPMENT 24 959 006 GAYTON WF AM J DIS CHILD 126 856 973 008 GREEN M IN: GREEN M 965 009 KNOWLES HC JR MED CLIN NORTH AM 55 1007 971 010 LANYON RI J CONSULT CLIN PSYCHOL 35 1 970 011 LOUGHLIN WC AM J DIS CHILD 68 13 944 012 MATTSSON A PEDIATRICS 50 801 972 013 MATTSSON A J AM ACAD CHILD PSYCHIAT 11 558 972 014 NOWICKI S JR J CONSULT CLIN PSYCHOL 40 148 973 015 PIERS EV PIERS HARRIS CHILDRENS SELF C 967 016 PURCELL K IN: WOLMAN BB 972 017 RAINER JD ADV PSYCHOSOMATIC MED 3 167 963 018 SINES JO MANUAL FOR THE MISSOURI CHILD 971 019 SWIFT CR PSYCHOSOM MED 29 555 967 020 WERRY JS IN: QUAY HC 972 021 WINER BJ STATISTICAL PRIN IN EXP 101 962 CT 1 BEDELL JR J PSYCHOSOM RES 21 237 977 2 GAYTON WF PEDIATRICS 59 888 977 3 SULLIVAN BJ PSYCHOSOM MED 41 127 979 4 KELLERMAN J J PEDIATR 97 126 980 5 DONALD MW J NEUROL NEUROSURG PSYCHIAT 44 641 981 6 DROTAR D PEDIATRICS 67 338 981 7 LEWIS BL J PEDIATR 101 636 982 8 STEINHAUSEN HC J AM ACAD CHILD PSYCHIAT 22 559 983 9 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 10 CERRETO MC PEDIATR CLIN NORTH AM 31 689 984 11 PLESS IB PEDIATR CLIN NORTH AM 31 33 984 12 VIEWEG BW J CLIN PSYCHOL 40 1382 984 13 VERMA A J NEUROL NEUROSURG PSYCHIAT 47 414 984 14 SLIJPER FME PROG BRAIN RES 61 417 984 15 LEWISTON NJ SEM RESPIR MED 6 321 985 16 BRESLAU N J AM ACAD CHILD PSYCHIAT 24 87 985 17 GRAHAM P DEVELOP MED CHILD NEUROL 27 389 985 18 FIELDING D J PSYCHOSOM RES 29 457 985 19 SIMMONS RJ PSYCHOSOM MED 47 111 985 20 HELLER A AM J DIS CHILD 139 257 985 21 CARRGREGG MRC MED J AUST 143 503 985 22 KOVACS M DIABETES CARE 9 472 986 23 JAMISON RN J CHRON DIS 39 609 986 24 COWEN L PEDIATRICS 77 745 986 25 NOLAN T J PEDIATR 109 201 986 PN 76095 RN 00449 AN 76190785 AU Carson-J-A. Gormican-A. TI Disease-medication relationships in altered taste sensitivity. SO J-Am-Diet-Assoc. 1976 Jun. 68(06). P 550-3. MJ DRUG-THERAPY: ae. NUTRITION. TASTE-DISORDERS: et. MN ANESTHETICS: pd. ANOREXIA: et. DYSAUTONOMIA-FAMILIAL: co. CYSTIC-FIBROSIS: co. DEFICIENCY-DISEASES: co. ENDOCRINE-DISEASES: co. HUMAN. KIDNEY-DISEASES: co. NEOPLASMS: co. NERVOUS-SYSTEM-DISEASES: co. NUTRITION: de. SJOGRENS-SYNDROME: co. TASTE: de. WOUNDS-AND-INJURIES: co. EX Reviewing evidence of abnormal taste acuity elicits several factors which can change a patient's taste acuity. Consideration of his diagnosis, his nutritional status, and the treatment he is receiving or has received can offer further understanding of his perceptions of food. Future studies will furnish more information about how the patient's ability to taste and smell may be altered and thus affect his attitude toward food. RF 001 RUBINI ME AM J CLIN NUTR 27 223 974 002 PANGBORN RM IN: KARE MR 967 003 HENKIN RL BORDENS NUTR REV 28 71 967 004 GREEN RF SCHIZOPHRENIA 2 70 970 005 GREEN RF JAMA 218 1303 971 006 HODGES RE IN: WHITE PL 972 007 COHEN IK JAMA 223 915 973 008 HAMBIDGE KM PEDIATR RES 6 868 972 009 HENKIN RI PROC NAT ACAD SCI USA 62 30 969 010 ANON POSTGRAD MED 53 30 973 011$ HENKIN RI JAMA 223 914 971 012 KING ER VA MED 98 652 971 013 FLETCHER GH RADIATION THERAPY IN THE MANA 962 014 TEWFIK HH PRESENTATION DIET THERAPY USA 973 015 MACCARTHY-LEVENTHAL F LANCET 2 1138 959 016 BONANNI G NUNT RADIOL 31 383 965 017 KALMUS H J LARYNGOL OTOL 73 180 959 018 HANSEN D WEGNEISER FUR DIE FACHAERZTLI 18 133 970 019 CONGER AD RADIAT RES 37 31 969 020 HENKIN RI J CLIN INVEST 42 727 963 021 HENKIN RI J CLIN ENDOCRINOL METAB 27 1436 967 022 HENKIN RI J CLIN ENDOCRINOL METAB 28 624 968 023 WEISS VALBRANCA G RIV CLIN MED 5 46 946 024 SCHAUPP H ARCH KLIN EXP OHR NAS KEHLK H 195 179 969 025 SCHELLING JL LANCET 1 508 965 026 DEWYS WD ANN NY ACAD SCI 230 427 974 027 ABASOV IT SOV MED 25 47 961 028 SIMON JA MASTERS RESEARCH REPORT 974 029 HENKIN RI IN: BOSMA JF 967 030 HENKIN RI NATURE 227 965 970 032 HENKIN RI ANN INTERN MED 76 375 972 033 HENKIN RI SCIENCE 138 1107 962 034 HENKIN RI IN: BOSMA JF 970 035 BRANDENBURG JH FACIAL NERVE PARALYSIS (TAPE) 973 036 KALMUS H CHEMICAL SENSES IN HEALTH 960 037 SUMNER D BRAIN 90 187 967 038 GROSSMAN SP IN: HERXHEIMER A 968 039 MATA F J NEUROPSYCH 4 315 963 040 FOGAN L ANN INTERN MED 74 795 971 041 HENKIN RI LANCET 2 1268 967 042 DUFFIELD JE BR MED J 1 491 973 043$ SCOTT PJ NZ MED J 59 296 970 044 SHAFAR J LANCET 1 83 965 045 RABE F MED WELT 16 711 970 046 HALLMAN BL JAMA 152 322 953 047 SCHNEEBURG NG JAMA 149 1091 952 CT 1 BURLESON WR AM J HOSP PHARM 35 584 978 2 MATTES RD AM J CLIN NUTR 41 672 985 PN 76096 RN 00450 AN 76190784 AU Rickard-K. Brady-M-S. Hempel-J. Gresham-E. TI Care of children with conditions characterized by high nutritional risks. SO J-Am-Diet-Assoc. 1976 Jun. 68(06). P 546-50. MJ CYSTIC-FIBROSIS: dh. HEART-DEFECTS-CONGENITAL: dh. SPINA-BIFIDA: dh. MN CHILD-NUTRITION. CHILD. CHILD-PRESCHOOL. DIET-SODIUM-RESTRICTED. DIETARY-FATS. DIETARY-PROTEINS. ENZYMES: tu. HEALTH-EDUCATION. HUMAN. INFANT. INFANT-NEWBORN. PATIENTS: ed. POTASSIUM. SODIUM. VITAMINS: tu. WATER-ELECTROLYTE-BALANCE. PATIENT-EDUCATION. EX A key to any successful preventive nutritional program is long-term follow up, reinforcement, and early intervention when necessary. The dietitian must evaluate the effectiveness of her approach through careful monitoring of various parameters of growth, such as length, weight, head circumference, and skinfold measurements. It is essential that she be aware of the advantages, limitations, and complications of any dietary program. We have described only three examples of complex nutritional problems encountered in the intensive care nursery. There are many more equally challenging and frustrating medical and surgical disorders which demand the expertise of the dietitian for successful management. The dietitian of the future cannot rely on knowledge alone, but must utilize considerable ingenuity and creativity in managing these complicated nutritional enigmas if acceptance and effectiveness are to be maintained. RF 001 RICKARD K J AM DIET ASSOC 66 592 975 002 FOMON SJ IN: FOMON SJ 472 974 003 BERGMANN KE IN: FOMON SJ 974 004 FOMON SJ INFANT NUTRITION 974 005 ROOK GD BR HEART J 35 87 973 006 SELVARAJ RJ AM J CLIN NUTR 25 166 972 007 KOPEL FB GASTROENTEROLOGY 62 483 972 008 WEIHOFEN DM J AM DIET ASSOC 54 206 969 009 ANTONOWICZ I PEDIATRICS 42 492 968 010 HUANG NN IN: SENIOR JR 207 968 PN 76097 RN 00451 AN 76259953 AU Govan-J-R. TI Antibiotic therapy and cystic fibrosis: increased resistance of mucoid Pseudomonas aeruginosa to carbenicillin. SO J-Antimicrob-Chemother. 1976 Jun. 2(2). P 215-7. MJ ANTIBIOTICS: tu. CARBENICILLIN: pd. CYSTIC-FIBROSIS: dt. PSEUDOMONAS-AERUGINOSA: de. MN CYSTIC-FIBROSIS: mi. DRUG-RESISTANCE-MICROBIAL. HUMAN. EX The present communication reports the effect of the bacterial mucus on the activity of antibodies on strains of P. aeruginosa. Mucoid variants of P. aeruginosa obtained from the non-mucoid strains PAO 381 and PAT 404 by selection for phenotypic resistance to the virulent phage E 79 were more resistant to carbenicillin than the non-mucoid parent strains. Mucoid P. aeruginosa strains isolated from two CF patients were more resistant to carbenicillin than non-mucoid strains isolated from the same samples of sputum. When the experiments already described were repeated with oxytetracycline, in contrast to our experience with carbenicillin, mucoid strains were found to be more sensitive than non-mucoid strains. To prove the hypothesis that mucoid strains can be selected in vivo by carbenicillin therapy it will be necessary to show that the level of carbenicillin in CF secretions is less than the minimum inhibitory concentrations of mucoid strains but greater than that of non-mucoid strains. We are now investigating additional CF isolates in conjunction with the range of antibiotics used in chemotherapy of CF patients. RF 001 BURNS MW LANCET 1 270 968 002 DOGGETT RG APPL MICROBIOL 18 936 969 003 DOGGETT RG J PEDIATR 68 215 966 004 GOVAN JRW J MED MICROBIOL 8 513 975 005 MARKS MI J PEDIATR 79 822 971 006 WILLIAMS RJ J MED MICROBIOL 6 409 973 007 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 BROWN MRW J ANTIMICROB CHEMOTHER 3 198 977 3 JONES SE J PHARMACY PHARMACOL 29 P 69 977 4 GOVAN JRW J ANTIMICROB CHEMOTHER 4 233 978 5 MARKOWITZ SM INFECT IMMUN 22 530 978 6 HOLLOWAY BW MICROBIOL REV 43 73 979 7 BRANSON D AM J MED TECHNOLOGY 45 405 979 8 GOVAN JRW J GEN MICROBIOL 110 229 979 9 SAVITSKAYA KI ZH MIKROBIO EPIDEMIO IMMUNOBI 1979 96 979 10 MARKOWITZ SM J ANTIMICROB CHEMOTHER 6 251 980 11 MARTIN AJ ARCH DIS CHILD 55 604 980 12 CHOPRA I ADV MICROB PHYSIOL 23 183 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 14 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 15 OMBAKA EA REV INFECT DIS 5 S880 983 16 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 17 ANWAR H FEMS MICROBIOL LETTERS 16 247 983 18 TURNOWSKY F FEMS MICROBIOL LETTERS 17 243 983 19 ANWAR H BIOCHIM BIOPHYS ACTA 761 119 983 20 TANNENBAUM CS ANTIMICROB AGENTS CHEMOTHER 25 673 984 21 MODAI J SEM HOP PARIS 61 2325 985 22 WOODS DE J INFECT DIS 151 581 985 23 DERETIC V J BACTERIOL 165 510 986 PN 76098 RN 00452 AN 76259939 AU Raeburn-J-A. TI Antibiotic management of cystic fibrosis. SO J-Antimicrob-Chemother. 1976 Jun. 2(2). P 107-9. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. RESPIRATORY-TRACT-INFECTIONS: dt. MN RESPIRATORY-TRACT-INFECTIONS: co, mi. EX Cystic fibrosis is the most common autosomal recessive disorder in Great Britain, Central Europe and North America and it affects 1 in 2000 live born children. Does the antibiotic treatment in cystic fibrosis differ from the treatment of respiratory infection in other conditions? The cystic fibrosis patient not only suffers progressive deterioration in respiratory function; he often has dangerous complicating conditions culminating in super-infection by highly resistant organisms. The basic principle of antibiotic therapy must be rigidly applied in cystic fibrosis. The first principle is to identify the pathogen and its susceptibility to antibiotics. The second principle is to ensure that the chosen antibiotic reaches the site of infection. The third principle is that the antibiotic therapy ought to complement the immunological defences of the patient. A fourth principle is that no therapy must be given without first deciding upon the criteria which will be acceptable as indicating clinical improvement. RF 001 BEARN AG CLIN GASTROENTEROL 2 515 973 002 BRUMFITT W J ANTIMICROB CHEMOTHER 1 163 975 003 DIAZ F J INFECT DIS 121 269 970 004 FORBES IJ AUST NZ J MED 2 160 971 005 FORSGREN A IN: BRENT L 345 974 006 LAWSON D IN: WATT PJ 69 970 007 LEHRER RI J CLIN INVEST 50 2498 971 008 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 972 009 MAY JR ARCH DIS CHILD 47 908 972 010 MCCRAE WM IN: APLEY J 4 157 974 011 MCCRAE WM SCOTT MED J 19 187 974 013 MEARNS MB IN: MANGOS JA 345 973 014 RAEBURN JA POSTGRAD MED J 47 366 971 015 RAEBURN JA SCAND J INFECT DIS 5 135 973 016 RAEBURN JA IN: DAIKOS GK 2 730 974 017 SOLBERG CO LANCET 2 727 972 CT 1 RAEBURN JA PROC NUTR SOC 36 77 977 2 GOVAN JRW J ANTIMICROB CHEMOTHER 4 233 978 3 GRAY JA SCOTT MED J 24 141 979 4 MICHALSEN H CHEMOTHERAPY 26 135 980 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 6 HODSON ME BR J DIS CHEST 77 71 983 PN 76099 RN 00453 AN 76167755 AU Barranco-S-C. Bolton-W-E. Haenelt-B-R. Abell-C-W. TI Differences in the incorporation of thymidine into DNA of normal and cystic fibrosis fibroblasts in vitro. SO J-Cell-Physiol. 1976 May. 88(1). P 33-41. MJ CYSTIC-FIBROSIS: me. DNA: bi. THYMIDINE: me. MN BROMODEOXYURIDINE: pd. CADMIUM. CELL-DIVISION. CELL-LINE. COMPARATIVE-STUDY. GENOTYPE. KINETICS. THYMIDINE-KINASE: me. THYMINE-NUCLEOTIDES: bi. URIDINE: me. AB Although similar fractions of cells were in the S phase of the cell cycle, normal human skin fibroblasts were shown to incorporate more than twice the 3HTdR into their DNA in vitro than did cells obtained from individuals with cystic fibrosis (CF). Obligate heterozygotes incorporated an intermediate amount of the DNA precursor. Studied were initiated to determine the basis of the differential incorporation of 3HTdR among the genotypes. An analog of thymidine, BUdR, produced varied effects on the growth kinetics of the three genotypes. The growth of cells in BUdR resulted in a 50% increase in the population doubling times of all three genotypes, and caused the cell morphology to change from a spindle shape to one in which the cells became broadened and flat, with numerous cytoplasmic projections extending for distances of several cell diameters. The activities of thymidine kinase and the participation of the exogenous and de novo pathways in the synthesis of TMP were found to be approximately the same in all three genotypes. The data suggest that an alteration in the transport of thymidine into the cells may account for the differences in TdR incorporation into DNA, and this may be associated with other changes in cystic fibrosis that are apparently membrane associated. RF 001 BARRANCO SC CANCER RES 33 691 973 002 BOLTON WE AM J HUM GENET 27 394 975 003 BUKOVSKY J CANCER RES 25 358 965 004 DANES BS AM J HUM GENET 25 323 973 005 FURLONG N ANAL BIOCHEM 5 515 963 006 HAHN GM EXP CELL RES 49 285 968 007 HOWARD A HEREDITY SUPPL 6 261 953 008 HUMPHREY RM DNA STRAND SCISSION AND REJOI 570 970 009 MATALON R LANCET 2 838 969 010 MENDELSOHN ML J NATL CANCER INST 28 1015 962 011 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 012 STUBBLEFIELD E EXP CELL RES 48 652 967 013 TOBEY RA J CELL BIOL 46 151 970 014 TOLIVER A EXP CELL RES 53 506 968 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 YOKOYAMA M PEDIATR RES 11 765 977 3 GROSS S CLIN CHEM 23 299 977 4 BARRANCO SC J NATL CANCER INST 59 1685 977 5 EPSTEIN J PROC NAT ACAD SCI USA 74 1676 977 6 KATZ S RES COMMUN CHEM PATH PHARM 19 491 978 7 JAKEL HP BIOL ZENTRALBL 98 55 979 8 ROSCHER AA PEDIATR RES 14 261 980 9 KATZ S CLIN CHIM ACTA 100 245 980 10 THOMPSON KVA GERONTOLOGY 29 97 983 PN 76100 RN 00454 AN 77071904 AU Tannenbaum-P-J. Posner-A-S. Mandel-I-D. TI Formation of calcium phosphates in saliva and dental plaque. SO J-Dent-Res. 1976 Nov-Dec. 55(6). P 997-1000. MJ CALCIUM-PHOSPHATES: an. DENTAL-PLAQUE: an. HYDROXYAPATITES: an. SALIVA: an. MN ADOLESCENCE. ADULT. ASTHMA: me. CALCIUM-CARBONATE: an. CHILD. CYSTIC-FIBROSIS: me. DENTAL-CALCULUS: an. HUMAN. SUPPORT-U-S-GOVT-P-H-S. AB This is an X-ray diffraction study of the mineral phases in saliva and early dental plaque. The salivas studied came from patients with cystic fibrosis (CF), those with asthma, and heavy and light calculus formers. One-week old plaque was studied from individuals who are heavy, moderate, and light calculus formers. RF 001 CHERNICK WS J PEDIATR 59 890 961 002 MANDEL ID CLIN PEDIATR 8 161 969 003 BLOMFIELD J ARCH DIS CHILD 48 267 973 004 GUGLER EC J PEDIATR 71 585 967 005 BOAT TF PEDIATR RES 8 531 974 006 WARTON KL BR MED J 3 570 971 007 BLOMFIELD J ARCH ORAL BIOL 19 1153 974 008 BERTHOLD H DTSCH ZAHNAERNTZL Z 25 7 970 009 JENSEN TA J DENT RES 33 741 973 010 KAUFMAN HW CALC TISS RES 11 97 973 011 LEGEROS RI J DENT RES 53 45 974 012 SCHROEDER HE ARCH ORAL BIOL 11 1 966 013 ROWLES SL IN: BLACKWOOD HJJ 176 973 014 MANDEL ID J PERIODONT RES 9 211 974 015 ROWLES SL DENT PRACTIT DENT RES 15 2 964 016 WESTERDEN EM J DENT RES 37 749 958 017 HAY DI ARCH ORAL BIOL 12 937 967 CT 1 DRIESSENS FCM J BIOL BUCCALE 8 239 980 2 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 PN 76101 RN 00455 AN 77071903 AU Jagels-A-E. Sweeney-E-A. TI Oral health of patients with cystic fibrosis and their siblings. SO J-Dent-Res. 1976 Nov-Dec. 55(6). P 991-6. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: dt. ORAL-HEALTH. PANCREATIN: tu. MN CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. DENTAL-CARIES: et. FEMALE. HUMAN. MALE. ORAL-HYGIENE. TETRACYCLINE: ae. TOOTH-DISCOLORATION: et. SUPPORT-U-S-GOVT-P-H-S. AB Caries prevalence, oral hygiene index, submandibular gland hypertrophy, occlusion, dental hypoplasia, and staining of permanent teeth were evaluated in 63 patients with cystic fibrosis (CF) who were maintained on a regimen of broad-spectrum antibiotics and oral pancreatin, and the findings were compared with those of their near- aged siblings. Gland hypertrophy and dental staining were increased in patients with CF whereas caries was significantly decreased. No other significant differences were found. RF 001 SHWACHMAN H PEDIATRICS 46 335 970 002 SHWACHMAN H PEDIATR CLIN NORTH AM 3 295 956 003 SHWACHMAN H ANTIBIOT ANN 692 958 004 ZEGARELLI EV ORAL SURG 24 62 967 005 YULE A AUST DENT J 15 519 970 006 SWALLOW JN ARCH DIS CHILD 42 311 967 007 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 008 KNUTSON J PUBLIC HEALTH REP 59 253 944 009 GRUEBBEL A J DENT RES 23 163 944 010 GREENE JC JADA 68 25 964 011 HANDELMAN S J ORAL THER 2 338 966 012 LITTLETON N JADA 68 520 964 013 WELLS H AM J PHYSIOL 208 877 965 014 SWEENEY EA J DENT RES 44 973 964 015 MANGOS JA J PEDIATR 74 823 969 016 BAUMHAMMERS A ARCH ORAL BIOL 13 353 968 017 JENSEN AL JADA 59 923 959 018 ENNEVER J J PERIODONTOL 32 331 961 019 PORTER PJ ANTIMICROB AGENTS CHEMOTHER 5 668 965 CT 1 FISCHER EG J PEDIATR 95 385 979 2 PRIMOSCH RE ORAL SURG 50 301 980 3 MAHANEY MC ARCH ORAL BIOL 31 363 986 PN 76102 RN 00456 AN 77208522 AU Baylis-J-M. TI Experiences in a paediatric gastroenterology clinic. SO J-Hum-Nutr. 1976 Oct. 30(5). P 327-32. MJ DIETETICS. GASTROINTESTINAL-DISEASES: dh. MN CARBOHYDRATE-METABOLISM-INBORN-ERRORS: dh. CELIAC-DISEASE: dh. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dh. ENGLAND. FEMALE. GASTROINTESTINAL-DISEASES: et. GLUTEN: ae. OUTPATIENT-CLINICS-HOSPITAL. HUMAN. MILK: ae. PEDIATRICS. EX The Paediatric Gastroenterology Clinic at this hospital was started to follow children with gastroenterological problems after discharge from hospital and in particular to monitor their clinical and dietary progress. Other dietitians who see an opening for this work in their hospitals may find our experiences to be of interest. Gluten-free diets, cow's milk free diets, sugar intolerance, cystic fibrosis, and non-specific cases are discussed. RF 001 BAKER PG BR MED J 1 486 975 002 CUNNINGHAM K NUTRITION 27 23 973 003 FRANCIS DEM DIETS FOR SICK CHILDREN 974 004 KUITUNEN P ARCH DIS CHILD 50 351 975 005 TANNER JM IN: HOLZER A 958 PN 76103 RN 00457 AN 77007715 AU Wright-P-F. Khaw-K-T. Oxman-M-N. Shwachman-H. TI Evaluation of the safety of amantadine-HC1 and the role of respiratory viral infections in children with cystic fibrosis. SO J-Infect-Dis. 1976 Aug. 134(2). P 144-9. MJ AMANTADINE: tu. CYSTIC-FIBROSIS: co. INFLUENZA: pc. RESPIRATORY-TRACT-INFECTIONS: pc. VIRUS-DISEASES: pc. MN ADOLESCENCE. ADULT. AMANTADINE: ae. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. FEMALE. HUMAN. INFANT. MALE. PLACEBOS. RESPIRATORY-TRACT-INFECTIONS: et. SUPPORT-U-S-GOVT-P-H-S. VIRUS-DISEASES: et. AB Amantadine-HC1, an antiviral drug clinically effective against most strains of influenza A virus, was evaluated in a double-blind trial in 153 children with cystic fibrosis during the initial appearance of influenza A/England/42 virus in the New England area. Infection with this variant strain of influenza virus did not reach epidemic proportions during the study, so that the effectiveness of amantadine in this study population could not be fully assessed. However, the potential symptomatic and biochemical toxicity of amantadine was carefully monitored in a pediatric population. Serologic screening by complement fixation tests indicated that respiratory viruses may be important pathogens in exacerbations of respiratory disease in patients with cystic fibrosis. RF 001 DAVIES WL SCIENCE 144 862 964 002 TOGO Y JAMA 203 1089 968 003 SMORODINSTSEV AA JAMA 213 1448 970 004 WINGFIELD WL N ENGL J MED 281 579 969 005 ANON AAP COMMITTEE INFECT DIS REPT 140 974 006 QUILLIGAN JJ JR J PEDIATR 69 572 969 007 ANON RECOMMENDED METHOD FOR THE US 971 008 SEVER JL J IMMUNOL 88 320 962 009 BIANDRATE P J CHROMATOGR 74 31 972 010 ANON INFLUENZA RESPIRATORY DISEASE 974 011 KATTWINKEL J J PEDIATR 82 234 973 012 BLEIDNER WE J PHARMACOL EXP THER 150 484 965 013 MCLAREN C LANCET 1 1157 973 014 CHANOCK RM JAMA 176 647 961 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 TERMEULEN V MONATSSCHR KINDERHEILKD 127 367 979 3 GLEZEN WP REV INFECT DIS 2 408 980 4 BECKER Y PROG MED VIROL 26 1 980 5 FISHAUT M AM J DIS CHILD 134 321 980 6 FEIGELSON J NOUV PRESSE MED 10 955 981 7 HALL CB AM J MED 70 670 981 8 PETERSEN NT ACTA PAEDIATR SCAND 70 623 981 9 GLEZEN WP PEDIATR RES 17 1029 983 10 AMBROSINI PJ J CLIN PSYCHOPHARMACOL 4 223 984 11 EFTHIMIOU J THORAX 39 150 984 12 WANG EEL N ENGL J MED 311 1653 984 13 MISCHLER EH SEM RESPIR MED 6 271 985 14 SHIELDS WD NEUROLOGY 35 579 985 15 FRIEND PA J INFECT 13 55 986 16 STROOBANT J J ROY SOC MED 79 19 986 17 WANG EEL PEDIATR INFECT DIS J 6 256 987 18 THOMPSON J J MED VIROL 21 249 987 PN 76104 RN 00458 AN 77007743 AU Crozier-D-N. Khan-S-R. TI Tobramycin in treatment of infections due to Pseudomonas aeruginosa in patients with cystic fibrosis. SO J-Infect-Dis. 1976 Aug. 134 Suppl. P S187-90. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: dt. TOBRAMYCIN: tu. MN ADOLESCENCE. ADULT. CHILD. CHRONIC-DISEASE. FEMALE. HUMAN. LUNG-DISEASES: dt. MALE. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: co. TOBRAMYCIN: pd. AB The effectiveness and safety of tobramycin against infections due to Pseudomonas aeruginosa in patients with cystic fibrosis were studied in 15 patients with moderate-to-severe exacerbation of chronic pulmonary infection. Seventeen courses of treatment were given. Tobramycin (5-7.5 mg/kg per day) was administered intravenously in three divided doses per day (seven to 21 days). All specimens of sputum yielded moderate-to-heavy growth of P. aeruginosa; all isolates were inhibited by 4 mug of tobramycin/ml, and in seven cases isolates were not inhibited by 4 mug of gentamicin/ml. The mean levels of tobramycin in the blood were 4.4 mug/ml 0.5-1 hr and 0.8 mug/ml 7.5-8 hr after administration. All patients received an antistaphylococcal agent before, during, and after therapy. All patients except for two received 750 mg of carbenicillin/kg per day intravenously in six divided doses and 1 g of carbenicillin in a mask for inhalation three times a day. Both clinical and radiological improvement was noted in all patients. The white blood cell count, which was elevated in seven patients, returned to normal, and levels of blood gases improved. P. aeruginosa was eliminated from the sputum in five cases. No adverse side effects were noted. RF 001 PRESTON DA ANTIMICROB AGENTS CHEMOTHER 11 322 971 002 ANON TOBRAMYCIN PRODUCT MONOGRAPH 975 CT 1 NEU HC MED J AUST 2 13 977 2 CIPOLLE RJ THER DRUG MONITOR 2 359 980 3 MARTIN AJ ARCH DIS CHILD 55 604 980 4 HYATT AC J PEDIATR 99 307 981 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 6 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 7 MENDELMAN PM AM REV RESPIR DIS 132 761 985 8 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 76105 RN 00459 AN 77007744 AU McCrae-W-M. Raeburn-J-A. Hanson-E-J. TI Tobramycin therapy of infections due to Pseudomonas aeruginosa in patients with cystic fibrosis: effect of dosage and concentration of antibiotic in sputum. SO J-Infect-Dis. 1976 Aug. 134 Suppl. P S191-3. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: dt. SPUTUM: me. TOBRAMYCIN: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. PSEUDOMONAS-INFECTIONS: co. TOBRAMYCIN: ad, me. AB Established respiratory infections with mucoid Pseudomonas aeruginosa in patients suffering from cystic fibrosis were treated with conventional as well as larger doses of tobramycin. The infection was eradicated in four of the 17 patients treated, but the duration of follow-up study of one patient was short. It appeared that treatment was most successful in those patients in whom the highest peak concentrations of tobramycin in sputum were obtained. No side effects were noted, even when tobramycin was given in large doses for two weeks. RF 001 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 002 MCCRAE WM IN: APLEY J 4 157 974 003 WOOD RE GAP CONF REP PSEUDOMON IN CF 4 974 004 MARKS MI J PEDIATR 79 822 971 005 HUANG NN J PEDIATR 78 338 971 006 HAWLEY HB CURR THER RES 16 414 974 007 RAEBURN JA POSTGRAD MED J 47 366 971 008 RAEBURN JA SCAND J INFECT DIS 5 135 973 CT 1 NEU HC MED J AUST 2 13 977 2 GOVAN JRW J ANTIMICROB CHEMOTHER 4 233 978 3 NEU HC INFECTION 8 S 62 980 4 MARTIN AJ ARCH DIS CHILD 55 604 980 5 WIENTZEN R AM J DIS CHILD 134 1134 980 6 THYS JP J ANTIMICROB CHEMOTHER 8 29 981 7 MOMBELLI G ANTIMICROB AGENTS CHEMOTHER 19 72 981 8 VAKOUTIS J AM J HOSP PHARM 38 1477 981 9 MOMBELLI G SCHWEIZ MED WOCHENSCHR 111 123 981 10 MALMBORG AS SCAND J INFECT DIS 1981 64 981 11 RIFF L SEM PERINATOL 6 155 982 12 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 14 HODSON ME BR J DIS CHEST 77 71 983 15 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 16 KUHN RJ CLIN PHARMACY 4 555 985 17 CHRYSTYN H J CLIN HOSP PHARM 10 219 985 18 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 19 CONWAY SP ACTA PAEDIATR SCAND 74 107 985 20 HORREVORTS AM CHEST 88 260 985 21 MOMBELLI G SCHWEIZ MED WOCHENSCHR 115 93 985 22 MENDELMAN PM AM REV RESPIR DIS 132 761 985 23 MARLIN GE AM REV RESPIR DIS 134 1209 986 24 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 76106 RN 00460 AN 77007745 AU Parry-M-F. Neu-H-C. TI Tobramycin and ticarcillin therapy for exacerbations of pulmonary disease in patients with cystic fibrosis. SO J-Infect-Dis. 1976 Aug. 134 Suppl. P S194-7. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. LUNG-DISEASES: dt. PENICILLINS: tu. PSEUDOMONAS-INFECTIONS: dt. TICARCILLIN: tu. TOBRAMYCIN: tu. MN ADOLESCENCE. ADULT. CHILD. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. MALE. PSEUDOMONAS-AERUGINOSA: de. TICARCILLIN: pd. TOBRAMYCIN: pd. AB Patients who had cystic fibrosis and acute infectious exacerbations of pulmonary disease produced by Pseudomonas aeruginosa were treated with a combination of tobramycin and ticarcillin. P. aeruginosa recovered from patients was inhibited by lower concentrations of both of these drugs than of gentamicin or carbenicillin. Thirteen courses of treatment were administered to 11 patients (mean, 14.4 days). A favorable response was seen in 11 of 12 completed courses of treatment. Improvement was associated with decreases in white blood cell count, temperature, and sedimentation rate. Adverse reactions were uncommon. Although P. aeruginosa was not eradicated from the sputum, the clinical results suggest that the combination of tobramycin and ticarcillin may be particularly useful for treatment of acute exacerbations of pulmonary disease in patients with cystic fibrosis from whom P. aeruginosa is isolated. RF 001 MAY JR ARCH DIS CHILD 47 908 972 002 HUANG NN J PEDIATR 78 338 971 003 MARKS MI J PEDIATR 79 822 971 004 KLASTERSKY J CLIN PHARMACOL THER 14 104 973 005 HAWLEY HB CURR THER RES 16 414 974 006 NEU HC ANTIMICROB AGENTS CHEMOTHER 10 385 970 008 BAUER AW AM J CLIN PATHOL 45 493 966 009 NEU HC APPL MICROBIOL 21 66 971 010 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 CIPOLLE RJ THER DRUG MONITOR 2 359 980 2 NEU HC INFECTION 8 S 62 980 3 PRINCE AS J PEDIATR 97 148 980 4 HYATT AC J PEDIATR 99 307 981 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 6 SZAFF M ACTA PAEDIATR SCAND 72 651 983 7 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 8 CONWAY SP ACTA PAEDIATR SCAND 74 107 985 9 HORREVORTS AM CHEST 88 260 985 10 JEWETT CV J PEDIATR 106 669 985 PN 76107 RN 00461 AN 76216212 AU Lorin-M-I. Gaerlan-P-F. Mandel-I-D. Denning-C-R. TI Composition of nasal secretion in patients with cystic fibrosis. SO J-Lab-Clin-Med. 1976 Jul. 88(1). P 114-7. MJ CYSTIC-FIBROSIS: me. MUCUS: an. NASAL-MUCOSA: se. MN ADOLESCENCE. ADULT. ALBUMINS: an. CHILD. HUMAN. IGA: an. IGG: an. PHOSPHATES: an. POTASSIUM: an. PROTEINS: an. SODIUM: an. SUPPORT-U-S-GOVT-P-H-S. AB Quantitative examination of nasal secretion in patients with cystic fibrosis revealed a significantly greater than normal concentration of calcium, a finding in keeping with the hypothesized importance of this ion in the pathophysiology of the disease. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 SHWACHMAN H IN: KENDIG EL JR 1 524 972 003 GIBSON LE PEDIATRICS 23 545 959 004 DI SANTAGNESE PA PEDIATRICS 12 549 953 005 EMRICH HM MOD PROBL PEDIATR 10 58 967 006 GUGLER EC MOD PROBL PEDIATR 10 95 967 007 MANDEL ID AM J DIS CHILD 113 431 967 008 WOTMAN S ARCH ORAL BIOL 16 663 971 009 GUGLER EC J PEDIATR 71 585 967 010 GIBSON LE PEDIATRICS 48 695 971 011 CHERNICK WS PEDIATRICS 24 739 959 012 MATTHEWS LW AM REV RESPIR DIS 88 199 963 013 TREMBLE GE LARYNGOSCOPE 59 639 949 014 LORIN MI J LAB CLIN MED 80 275 972 015 CHEN PS ANAL CHEM 28 1756 956 016 FOLIN O J BIOL CHEM 73 627 927 017 HEIDELBERGER M SCIENCE 97 405 943 018 MANCINI G IMMUNOCHEMISTRY 2 235 965 019 GRIFO S PRACT OTORHINOLARYNGOL 33 394 971 PN 76108 RN 00462 AN 76096193 AU Wolf-R-O. Taussig-L-M. Ross-M-E. Wood-R-E. TI Quantitative evaluation of serum pancreatic isoamylases in cystic fibrosis. SO J-Lab-Clin-Med. 1976 Jan. 87(1). P 164-8. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: bl. ISOAMYLASE: bl. PANCREAS: en. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, co. HUMAN. INFANT. INFANT-NEWBORN. ISOAMYLASE: an. PANCREATIC-DISEASES: en, co, bl. SALIVARY-GLANDS: en. AB The amylase in sera of 20 patients with cystic fibrosis of the pancreas (CFP), 17 of whom had pancreatic insufficiency (PI), and of 34 normal control subjects was separated into its pancreatic and salivary components and quantitated. Electrophoretic separation was achieved on polyacrylamide disc gels with subsequent sectioning of the gel columns. The amylase content in international units per liter (IU/L.) was obtained for each section by the insoluble blue starch substrate method. The mean total serum amylase of the CFP patients with PI was significantly lower than the control group. A highly significant decrease was found when the pancreatic isoamylase was considered alone. The salivary isoamylase level remained essentially the same. Three patients with CFP but without PI demonstrated higher than normal pancreatic amylase values in the serum. These patients may be in an early stage of a progressive involvement of the pancreas in which there is an increased release of amylase. This method of electrophoretic separation and quantitation of the serum amylase is sensitive and efficient and allows the simultaneous processing of multiple specimens. The results obtained agree with previously published ratios of serum isoamylases. Our method should be valuable in the study of other diseases of the exocrine pancreas and salivary glands. RF 001 TAUSSIG LM PEDIATRICS 54 229 974 002 WOLF RO AM J CLIN PATHOL 54 214 970 003 ROSS ME J LAB CLIN MED 83 750 974 004 WOLF RO AM J CLIN PATHOL 49 871 968 005 FRIDHANDLER L CLIN CHEM 18 1493 972 006 KAMARYT J CESK PEDIATR 25 78 970 008 MELNYCK A CAN J MED TECHNOL 35 8 973 CT 1 WOLF RO AM J CLIN PATHOL 65 1022 976 2 MERRITT AD ADV HUM GENET 8 135 977 3 OTSUKI M EUR J PEDIATR 125 175 977 4 DAVIDSON GP PEDIATR RES 12 967 978 5 GILLARD BK PEDIATR RES 12 868 978 6 HUBBARD VS J PEDIATR 92 685 978 7 FORSTNER GG PEDIATR RES 13 1077 979 8 WOLF RO PEDIATR RES 13 1076 979 9 GILLARD BK CLIN CHEM 25 1919 979 10 WOOD RE SOUTH MED J 72 189 979 11 GILLARD BK PEDIATR RES 14 1168 980 12 WOLF RO PEDIATR RES 14 968 980 13 TARNOKY AL J ROY SOC MED 73 73 980 14 PARK RW GASTROENTEROLOGY 81 1143 981 15 TOBIN MJ IR J MED SCI 150 325 981 16 STERN RC LANCET 1 1401 982 17 TSIANOS EB LANCET 1 856 982 18 BROWN RC J CLIN PATHOL 35 547 982 19 STERN RC GASTROENTEROLOGY 82 707 982 20 TSIANOS EB CLIN CHIM ACTA 124 13 982 21 MASARYK TJ DIG DIS SCI 28 874 983 22 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 23 GILLARD BK AM J DIS CHILD 138 577 984 24 MAILLIE AJ SCAND J GASTROENTEROL 21 941 986 25 WOLF RO CLIN CHEM 32 296 986 PN 76109 RN 00463 AN 76263347 AU Barton-A-D. Ryder-K. Lourenco-R-V. Dralle-W. Weiss-S-G. TI Inflammatory reaction and airway damage in cystic fibrosis. SO J-Lab-Clin-Med. 1976 Sep. 88(3). P 423-6. MJ BRONCHIECTASIS: pa. BRONCHITIS: pa. CYSTIC-FIBROSIS: pa. MN BRONCHI: pa. CHRONIC-DISEASE. DNA: an. EPITHELIUM: pa. GAMMA-GLUTAMYLTRANSFERASE: an. HUMAN. LEUKOCYTES: pa. SPUTUM: an. SUPPORT-U-S-GOVT-P-H-S. AB In cystic fibrosis there is chronic infection and inflammatory reaction in the airways, accompanied by destruction and shedding of airway epithelium. Leukocytes migrate into the airways and some disintegrate, liberating deoxyribonucleoprotein that is incorporated into the gel structure of the bronchial mucus. We compared the status of these processes in cystic fibrosis with that in chronic bronchitis and bronchiectasis, by examining the sputum raised from the lower airways. Measurements also were made on sputum induced in normal subjects. The results indicate that migration of leukocytes into the airways and shedding of damaged airway epithelium were minimal in the normal subjects; they were significant in the patients with chronic bronchitis, higher in those with bronchiectasis, and still higher in those with cystic fibrosis. The large increases found in the total content of DNA and solids in the cystic fibrosis sputum were due to increases in the insoluble fraction containing the whole leukocytes and particulate debris that remained when the sputum mucus gel was solubilized with mercaptoethanol. Despite the large increases in the total content of DNA and solids, the contents of mucus gel components and of deoxyribonucleoprotein from disintegrated leukocytes actually present in the mucus gel structure of the cystic fibrosis sputum were not significantly higher than in the sputum from the patients with chronic bronchitis or brochiectasis. RF 001 PECORA DV N ENGL J MED 258 71 958 002 WHITE JR IN: COPLEY A 259 960 003 ANON AM REV RESPIR DIS 85 762 962 004 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 005 HAVEZ R CLIN CHIM ACTA 17 281 967 006 BURTON K BIOCHEM J 62 315 956 007 BARTON AD PROC SOC EXP BIOL MED 146 99 974 008 PUCHELLE E BULL PHYSIOPATH RESPIR NANCY 9 237 973 009 LOPATA M AM REV RESPIR DIS 110 730 974 010 HAVEZ R EXP ANNU BIOCHIM MED 32 121 973 011 MACKAY M EXP CELL RES 49 533 968 012 CHERNICK WS PEDIATRICS 24 739 959 013 MATTHEWS LW AM REV RESPIR DIS 88 199 963 014 GLENNER GG J HISTOCHEM CYTOCHEM 10 481 962 015 CROFTON J RESPIRATORY DISEASES 969 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 WANNER A AM REV RESPIR DIS 116 73 977 3 BARTON AD PROC SOC EXP BIOL MED 156 8 977 4 JAKEL HP BIOL ZENTRALBL 98 55 979 5 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 6 WILSON GB J CLIN INVEST 68 171 981 7 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 8 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 9 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 76110 RN 00464 AN 77121095 AU Goodchild-M-C. Edwards-J-H. Glenn-K-P. Grindey-C. Harris-R. Mackintosh-P. Wentzel-J. TI A search for linkage in cystic fibrosis. SO J-Med-Genet. 1976 Dec. 13(6). P 417-9. MJ CYSTIC-FIBROSIS: fg. LINKAGE-GENETICS. MN CHILD. HUMAN. AB Linkage between the locus for cystic fibrosis and other genetic markers was explored in 40 families from Birmingham and 20 from Manchester. No strong evidence was found for linkage with any of the markers examined. There was evidence against close linkage with ABO, HLA, and Rh. RF 001 CARTER CO IN: BODIAN M 49 952 002$ CHAUTARD-FREIRE-MAIA ANN HUM GENET 38 191 974 003 DANKS DM ANN HUM GENET 28 323 965 004 EDWARDS JH CLIN GENET 3 371 972 005 EDWARDS JH HISTOCOMPATIBILITY TESTING 972 006 FELLOUS M C R ACAD SCI (D)(PARIS) 272 3356 971 007 GOODCHILD MC ARCH DIS CHILD 48 684 973 008 LAMM LU ANN HUM GENET 38 383 975 009 MORTON NE AM J HUM GENET 7 277 955 010 POLYMENIDIS Z LANCET 2 1452 973 011 STEINBERG AG AM J HUM GENET 8 177 956 012 STEINBERG AG AM J HUM GENET 8 162 956 013 TERASAKI PI NATURE 204 998 964 014 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 SAFWENBERG J TISSUE ANTIGENS 10 287 977 2 HERROD HG J PEDIATR 94 676 979 3 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 4 BOWMAN BH HUM HERED 31 248 981 5 EDWARDS JH LANCET 1 1020 984 6 ANON LANCET 2 249 985 7 EIBERG H CLIN GENET 27 206 985 8 EIBERG H CLIN GENET 28 265 985 9 POVEY S CYTOGENET CELL GENET 40 67 985 10 SCAMBLER P HUM GENET 69 250 985 11 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 12 MOSS RB MONOGR ALLERGY 19 202 986 13 SCHMIEGELOW K CLIN GENET 29 374 986 14 FARRALL M AM J HUM GENET 38 75 986 15 SCAMBLER PJ AM J HUM GENET 38 567 986 16 SCAMBLER PJ CYTOGENET CELL GENET 41 62 986 PN 76111 RN 00465 AN 76145694 TI Report of the committe for a study for evaluation of testing for cystic fibrosis. SO J-Pediatr. 1976 Apr. 88(4 Pt 2). P 711-50. (REVIEW). MJ CYSTIC-FIBROSIS: di. MN ADULT. CHILD. CYSTIC-FIBROSIS: fg, me. DUODENUM: an. FEMALE. FINANCING-GOVERNMENT. FINANCING-ORGANIZED. GENETIC-COUNSELING. HUMAN. INFANT-NEWBORN. METHODS. MUCINS: me. MUCOUS-MEMBRANE: me. PANCREAS: me. PANCREATIC-JUICE: an. PREGNANCY. PRENATAL-DIAGNOSIS. PROTEINS: me. RESEARCH. REVIEW. SODIUM-CHLORIDE: an. SWEAT-GLANDS: me. SWEAT: an. SUPPORT-U-S-GOVT-P-H-S. UNITED-STATES. EX The Committee for a Study for Evaluation of Testing for Cystic Fibrosis was established in August 1974 to assess the reliability of the data on cystic fibrosis "factors" in serum and exocrine secretions and in cell culture; evaluate the role of the cystic fibrosis "factors" in the detection of the heterozygote and in the diagnosis of cystic fibrosis in the homozygote in utero, during the neonatal period, and thereafter; evaluate the various screening tests for the diagnosis of cystic fibrosis in the newborn infant that have been proposed in this country and abroad, about which there is considerable controversy; and evaluate the sweat test, which as currently performed leads to diagnostic errors, both positive and negative. Section 1 of this report presents what is known about the clinical aspects of cystic fibrosis; Section 2 summarizes the diverse facets of research in cystic fibrosis, including the sources of its support; and Section 3 is a critical analysis of the state of the art for testing for cystic fibrosis. Section 4 comprises the Committee's recommendations, which it is hoped will be of use to both clinicians and researchers in the various settings where cystic fibrosis is diagnosed, treated, and studied. RF 001 CONNEALLY PM TEX REP BIOL MED 31 639 973 002 HALLETT WY AM REV RESPIR DIS 92 714 965 003 KNUDSON AG JR AM J HUM GENET 19 388 967 005 CORNET JM IN: BARNETT HL 1208 972 006 DI SANTAGNESE PA PEDIATRICS 12 549 953 007 SHWACHMAN H PEDIATRICS 36 689 965 008 DI SANTAGNESE PA MOD PROBL PEDIATR 10 135 967 009 SHWACHMAN H PEDIATRICS 46 335 970 010 SHWACHMAN H HOSP PRACT 9 143 974 011 ANON CF FND 1973 REP SURVI STUD PA 975 012 DISCHE Z PEDIATRICS 24 74 959 013 JOHANSEN PG BIOCHEM J 87 63 963 014 KOPITO LE FERTIL STERIL 24 512 973 015 BOAT TF AM REV RESPIR DIS 110 428 974 016 SPOCK A PEDIATR RES 1 173 967 017 SANCHIS J N ENGL J MED 288 651 973 018 ROSSMAN CM PEDIATR RES 8 469 974 019 QUINLAN MF AM REV RESPIR DIS 99 13 969 020 YEATES DB J APPL PHYSIOL 39 487 975 022 SACKNER MA J APPL PHYSIOL 34 495 973 023 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 024 WOOD RE AM REV RESPIR DIS 111 733 975 025 MANGOS JA PEDIATR RES 1 436 967 026 SCHULZ IJ J CLIN INVEST 48 1470 969 027 SIMOPOULOS AP PEDIATR RES 5 626 971 028 MANGOS JA TEX REP BIOL MED 31 651 973 029 DI SANTAGNESE PA IN: DOWNEY JA 25 974 030 LEWISTON NJ CF CLUB ABST 15 59 974 031 COHEN FL J MED GENET 11 253 974 032 SMOLLER M AM J DIS CHILD 98 277 959 033 DI SANTAGNESE PA N ENGL J MED 277 1287 967 034 DANES BS J EXP MED 129 775 969 035 DANES BS LANCET 2 765 973 036 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 038 TAUSSIG LM LANCET 1 1367 972 039 KAISER D LANCET 1 1003 970 040 KAISER D PEDIATR RES 5 167 971 041 BROWN GA LANCET 2 639 971 042 LOBECK CC IN: STANBURY JB 1605 972 043 KAISER D IN: MANGOS JA 247 973 044 TAYLOR A PEDIATR RES 8 861 974 045 LOCKHART LH TEX REP BIOL MED 31 631 973 046 CONOVER JH PEDIATR RES 7 220 973 047 CONOVER JH PEDIATR RES 7 224 973 048 BERATIS NG PEDIATR RES 7 958 973 049 CONOVER JH LIFE SCI 14 253 974 050 MANGOS JA PEDIATR RES 2 378 968 051 MANGOS JA IN: MANGOS JA 221 973 052 DOGGETT RG TEX REP BIOL MED 31 685 973 054 DANES BS J EXP MED 136 1313 972 055 LIEBERMAN J AM REV RESPIR DIS 111 100 975 056 SCANLIN TF JR LANCET 1 1382 975 057 POLLEY MJ J MED GENET 11 249 974 059 RAO GJS J PEDIATR 80 573 972 060 RAO GJS SCIENCE 177 610 972 061 RAO GJS PEDIATR RES 8 684 974 063 HATHAWAY WE BLOOD 26 521 965 064 HATTERSLEY PG BR J HAEMATOL 18 411 970 066 WILSON GB CF CLUB ABST 15 1 974 067 WILSON GB PEDIATR RES 9 635 975 068 RENNERT OM PEDIATRICS 50 485 972 069 RENNERT OM IN: MANGOS JA 41 973 070 LUNDGREN DW CF CLUB ABST 15 2 974 071 KLAGSBRUN M IN: MANGOS JA 53 973 072 HODES ME PEDIATR RES 8 212 974 073 MCEVOY FA PEDIATR RES 9 721 975 074 COHEN LF PEDIATR RES 9 312 975 075 SCHWERT GW BIOCHIM BIOPHYS ACTA 16 570 955 076 HADORN B J PEDIATR 73 39 968 077 LAPEY A J PEDIATR 84 328 974 078 SLEGERS JFG PFLUEGERS ARCH 279 265 964 079 SCHULZ IJ PFLUEGERS ARCH 284 360 965 080 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 081 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 082 ANON GAP CONF REP PROB SWEAT TESTI 975 083 KOPITO L PEDIATRICS 43 794 969 085 SHWACHMAN H PEDIATRICS 32 85 963 086 SHWACHMAN H J PEDIATR 66 432 965 087 WARWICK WJ CF CLUB ABST 16 58 975 088 BRIMBLECOMBE FSW LANCET 2 1428 973 089 BRAY PT ARCH DIS CHILD 48 825 973 090 GRAND RJ J PEDIATR 70 357 967 091 NADLER HL LANCET 2 84 969 092 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 093 BOLTON WE AM J HUM GENET 27 394 975 094 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 095 ANON GENETIC SCREENING PROG PRIN A 975 096 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 097 GEORGE L ARCH DIS CHILD 46 139 971 098 KULCZYCKI LL AM J DIS CHILD 127 64 974 099 WILSON JMG PUBLIC HEALTH PAPERS 34 968 100 KOPITO L N ENGL J MED 272 504 965 101 SHWACHMAN H N ENGL J MED 255 999 956 102 KULCZYCKI LL AM J DIS CHILD 100 174 960 103 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 104 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 105 STEPHAN U PEDIATRICS 55 35 975 106 LEVY HL IN: MANGOS JA 261 973 107 GREEN MN PEDIATRICS 21 635 958 108 WISER WC PEDIATRICS 33 115 964 109 GREEN MN PEDIATRICS 41 989 968 110 PROSSER R ARCH DIS CHILD 49 597 974 111 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 114 GIAMMONA ST AM REV RESPIR DIS 102 834 970 115 ANON NIH APPROPR FY 1972 REPORT 34 972 116 GIBSON LE PEDIATRICS 23 545 959 CT 1 RYLEY HC LANCET 2 365 976 2 SANTAGNESE PAD N ENGL J MED 295 481 976 3 TENKATE LP INT J EPIDEMIOL 6 23 977 4 THOMAS JM PEDIATR RES 11 1148 977 5 THOMAS JM PEDIATR RES 11 138 977 6 GOTZ M PAEDIATR PAEDOL 12 413 977 7 ANDORSKY M AM J DIG DIS 22 56 977 8 BRAY PT CLIN CHIM ACTA 80 333 977 9 DESAI N J PEDIATR 91 447 977 10 LYRENE RK J PEDIATR 91 681 977 11 ANON LANCET 2 1032 978 12 WILSON GB PEDIATR RES 12 801 978 13 BARNES GL AUST PAEDIATR J 14 78 978 14 WARWICK WJ CLIN CHEM 24 2050 978 15 HOLTZMAN NA PEDIATR CLIN NORTH AM 25 411 978 16 MILLER WA SOUTH MED J 71 201 978 17 BRESLOW JL SCIENCE 201 180 978 18 SANTAGNESE PAD AM J MED 66 121 979 19 WARWICK WJ AM J CLIN PATHOL 72 142 979 20 DAVIDSON AGF N ENGL J MED 300 1164 979 21 DAVIS PB AM J MED 69 643 980 22 RYLEY HC J CLIN PATHOL 34 906 981 23 RYLEY HC J CLIN PATHOL 34 179 981 24 PARK RW GASTROENTEROLOGY 81 1143 981 25 SCHWACHMAN H J PEDIATR 98 576 981 26 BRESLOW JL N ENGL J MED 304 1 981 27 LITTLEFIELD JW N ENGL J MED 304 44 981 28 KNOWLES M N ENGL J MED 305 1489 981 29 SORSCHER EJ LANCET 1 368 982 30 STERN RC LANCET 1 1401 982 31 SEALE TW ANN CLIN LAB SCI 12 415 982 32 WIESEMANN HG MONATSSCHR KINDERHEILKD 130 96 982 33 BONFORTE RJ LANCET 1 71 983 34 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 35 COURY AJ CLIN CHEM 29 1593 983 36 HANSEN RC ARCH DERMATOL 119 51 983 37 BONFORTE RJ AM REV RESPIR DIS 127 392 983 38 DAVIS PB AM REV RESPIR DIS 128 34 983 39 MASOERO G AM J DIS CHILD 137 167 983 40 BOUCHER RC LUNG 161 1 983 41 FRANCKX J HELV PAEDIATR ACTA 39 347 984 42 DAVIS PB CHEST 85 802 984 43 SCHONI MH J PEDIATR 104 691 984 44 WARWICK WJ CLIN CHEM 32 850 986 45 LITTLEWOOD JM J ROY SOC MED 79 55 986 46 ROBERTS DD ARCH BIOCHEM BIOPHYS 245 292 986 PN 76112 RN 00466 AN 76122145 AU Morin-C-L. Roy-C-C. Lasalle-R. Bonin-A. TI Small bowel mucosal dysfunction in patients with cystic fibrosis. SO J-Pediatr. 1976 Feb. 88(2). P 213-6. MJ CYSTIC-FIBROSIS: co. INTESTINAL-MUCOSA: me. INTESTINE-SMALL: me. MALABSORPTION-SYNDROMES: et. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYCLOLEUCINE: me. CYSTIC-FIBROSIS: me, en. DIPEPTIDASES: me. DISACCHARIDASES: me. HUMAN. INTESTINAL-MUCOSA: en. INTESTINE-SMALL: en. LYSINE: me. MALABSORPTION-SYNDROMES: me, en. PHENYLALANINE: me. CELIAC-DISEASE: me, en. AB Jejunal biopsies were obtained from 37 children with cystic fibrosis, 16 with gluten-induced enteropathy, and 18 control subjects for the following studies: (1) disaccharidase activity, (2) L-ALA-L-Phe hydrolase activity, and (3) intestinal uptake of three 14C-labeled amino acids. Values were significantly reduced in the three determinations in patients with gluten-induced enteropathy as compared to control subjects. Lactase and L-ALA-L-Phe hydrolase activities were significantly reduced (p less than 0.01) in CF patients as compared to control subjects. Definite hypolactasia was also observed in 23% of the children with CF. Uptake of lysine was normal in CF patients whereas that of phenylalanine and cycloleucine was reduced as compared to control subjects. This study suggests an intestinal component to the malabsorption of patients with CF. RF 001 WEBER AM N ENGL J MED 289 1001 973 002 DEREN JJ N ENGL J MED 288 949 973 003 REEMTSMA K PEDIATRICS 22 525 958 004 GIBBONS ISE LANCET 1 877 967 005 ANTONOWICZ I PEDIATRICS 42 492 968 006 LAPEY A J PEDIATR 84 328 974 007 JENSEN SB ACTA PAEDIATR SCAND 45 70 956 008 HAINES RD JAMA 180 1000 962 009 SHWACHMAN H J PEDIATR 65 645 964 010 DAHLQVIST A ANAL BIOCHEM 7 18 964 011 HEIZER WD J CLIN INVEST 48 210 969 012 LOWRY OH J BIOL CHEM 193 265 951 013 MORIN CL J CLIN INVEST 50 1961 971 014 THIER SO J CLIN INVEST 44 442 965 015 WINER BJ STATISTICAL PRIN IN EXP 101 962 016 WELSH JD ARCH INTERN MED 120 261 967 017 NEWCOMER AD GASTROENTEROLOGY 66 754 974 018 PLOTKIN GR N ENGL J MED 271 1037 964 019 ARTHUR AB ARCH DIS CHILD 41 519 966 020 DAHLQVIST A ACTA PAEDIATR SCAND 59 621 970 021 BERG NO GASTROENTEROLOGY 59 575 970 022 DOUGLAS AP LANCET 2 491 968 023 DOUGLAS AP GUT 11 15 970 024 SILK DBA GUT 15 1 974 025 CRAFT IL GUT 9 425 968 026 SCHEDL HP J CLIN INVEST 47 417 968 027 BAYLESS TM AM J CLIN NUTR 22 181 969 028 BEDINE MS GASTROENTEROLOGY 65 735 973 029 HEIZER WD BIOCHIM BIOPHYS ACTA 185 409 969 030 HEIZER WD BIOCHIM BIOPHYS ACTA 264 450 972 031 MATTHEWS DM GUT 6 411 965 032 FREYE HB J PEDIATR 64 575 964 033 DISCHE Z PEDIATRICS 24 74 959 CT 1 BUTS JP UNION MED CAN 105 1660 976 2 ROY CC CLIN GASTROENTEROL 6 377 977 3 MORIN CL PEDIATRICS 60 114 977 4 MORIN CL LANCET 1 1102 979 5 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 6 HARRIES JT ARCH DIS CHILD 54 19 979 7 BERG NO ACTA PAEDIATR SCAND 68 275 979 8 COX KL J PEDIATR 94 488 979 9 ANDRIA G PEDIATR RES 14 812 980 10 MITCHELL EA AUST PAEDIATR J 17 89 981 11 DEBIEVILLE F ACTA PAEDIATR SCAND 70 33 981 12 PARK RW GASTROENTEROLOGY 81 1143 981 13 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 14 FONDACARO JD PEDIATR RES 16 494 982 15 AURICCHIO S J PEDIATR GASTROENTEROL NUTR 2 S304 983 16 MILLA PJ GUT 24 818 983 17 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 18 FONDACARO JD LIFE SCI 35 1431 984 19 BERTRAND JM J PEDIATR 104 41 984 20 FRASE LL GASTROENTEROLOGY 88 478 985 21 EGGERMONT E ACTA PAEDIATR SCAND SUPPL 317 1985 16 985 22 MOORE MC AM J CLIN NUTR 44 33 986 23 PENNY DJ ARCH DIS CHILD 61 1127 986 24 RUBINSTEIN S PEDIATRICS 78 473 986 25 DODGE JA J ROY SOC MED 79 27 986 PN 76113 RN 00467 AN 76122172 AU Boyle-I-R. di-SantAgnese-P-A. Sack-S. Millican-F. Kulczycki-L-L. TI Emotional adjustment of adolescents and young adults with cystic fibrosis. SO J-Pediatr. 1976 Feb. 88(2). P 318-26. MJ CYSTIC-FIBROSIS. EMOTIONS. STRESS-PSYCHOLOGICAL. MN ACTIVITIES-OF-DAILY-LIVING. ADOLESCENCE. ADULT. ATTITUDE-TO-DEATH. ATTITUDE. BODY-IMAGE. COMMUNICATION. FEMALE. HUMAN. INTELLIGENCE. INTERPERSONAL-RELATIONS. MALE. PARENTS. SEX-MATURATION. AB Twenty-seven adolescents and young adults with cystic fibrosis were studied to evaluate the phychological impact of this chronic illness. At first glance, most patients appeared to function adequately on a daily basis. However, four sources of psychological stress, leading to emotional disturbance, were identified: altered physical appearance causing distorted body images and denial of sexuality, strained interpersonal relationships resulting in isolation and mental strain, conflicts in upbringing, and increased awareness of the future and of death. Guidelines for the physician treating these young adults and their families include: (1) encouragement for greater involvement by the patient's father; (2) assisting the mother to find outside interests and to allow more independence to the patient; (3) stressing communication about cystic fibrosis within the home; (4) emphasizing outside activity for each patient; (5) repeated discussions of the patient's concerns while emphasizing his strengths; (6) anticipation of problems, specific to cystic fibrosis, such as sterility in males; and (7) encouragement of interpatient communication. RF 001 MEYEROWITZ JH SOC SCI MED 1 249 967 002 TURK J PEDIATRICS 34 67 964 003 TROPAUER A AM J DIS CHILD 119 424 970 004 MCCOLLUM AT J PEDIATR 77 571 970 005 KULCZYCKI LL CLIN PROC CHILD HOSP DC 25 320 969 006 LAWLER RH CAN MED ASSOC J 94 1043 966 007 CYTRYN L IN: ANTHONY EJ 37 973 008 SPOCK A NC MED J 27 426 966 009 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 TAUSSIG LM J PEDIATR 82 380 973 013 PINKERTON P DEVELOP MED CHILD NEUROL 12 207 970 014 MURAWSKI B DIABETES 19 259 970 015 KAUFMAN RV PEDIATRICS 48 123 971 016 CAMPBELL WA J UROL 104 930 970 017 KURTZ R J CLIN PSYCHOL 26 149 970 018 BERNSTEIN DM AM J PSYCHIATRY 127 1189 971 019 ABRAM HS AM J PSYCHIATRY 127 1199 971 021 AGLE DP MODERN TREATMENT 5 111 968 022 BROWNE JW AM J ORTHOPSYCHIATRY 30 730 960 023 MATTSSON A AM J PSYCHIATRY 122 1349 966 CT 1 HERROD HG J PEDIATR 91 276 977 2 GOLDBERG RT ARCH PHYS MED REHABIL 60 369 979 3 STRAUSS GD AM J DIS CHILD 133 301 979 4 ZELTZER L J PEDIATR 97 132 980 5 HUBBARD VS AM J DIS CHILD 134 317 980 6 STRAUSS GD J CHRON DIS 34 141 981 7 BYWATER EM ARCH DIS CHILD 56 538 981 8 DROTAR D PEDIATRICS 67 338 981 9 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 10 STEINHAUSEN HC J AM ACAD CHILD PSYCHIAT 22 559 983 11 FIRTH M DEVELOP MED CHILD NEUROL 25 466 983 12 WELLS AL J FOOD NUTR 41 65 984 13 COWEN L PSYCHOSOM MED 46 363 984 14 DEWET B S AFR MED J 65 526 984 15 LANDON C AM J DIS CHILD 138 388 984 16 SIMMONS RJ PSYCHOSOM MED 47 111 985 17 GOLDBERG RT ARCH PHYS MED REHABIL 66 492 985 18 KADEN GG AM J DIS CHILD 139 995 985 19 PUMARIEGA AJ J AM ACAD CHILD PSYCHIAT 25 269 986 20 FITZPATRICK C DEVELOP MED CHILD NEUROL 28 589 986 21 FITZPATRICK C DEVELOP MED CHILD NEUROL 28 596 986 22 NOLAN T PEDIATRICS 77 229 986 23 MEARNS MB J ROY SOC MED 79 51 986 24 NOLAN T J PEDIATR 109 201 986 25 MOLLERING M KLIN PAEDIATR 198 369 986 PN 76114 RN 00468 AN 76193175 AU Oppenheimer-E-H. Schwartz-A. TI Easy bruisability and terminal coma in a "normal" 5-month-old infant. SO J-Pediatr. 1976 Jun. 88(6). P 1049-53. MJ CEREBRAL-HEMORRHAGE: pa. CYSTIC-FIBROSIS: pa. MN CASE-REPORT. CEREBRAL-HEMORRHAGE: et. COMA. HUMAN. INFANT. MALE. VITAMIN-K-DEFICIENCY: co. EX The patient was a 5-month-old white male admitted in coma via transferral from another hospital. Clinical discussion argues that this child had acquired coagulopathy due to vitamin K deficiency and that this was secondary to the fat malabsorption that occurs in cystic fibrosis. The left third nerve palsy and contralateral hemiparesis in a patient with rapid neurologic deterioration suggest a mass lesion in the left cerebral hemisphere with uncal herniation. Pathologic discussion also suggests intracranial hemorrhage, massive on left, accompanied by cystic fibrosis with severe pancreatic lesions and bleeding disorder, probably due to vitamin K deficiency. RF 001 BAEHNER RL N ENGL J MED 275 524 966 002 MOSESSON MW N ENGL J MED 278 815 968 003 COOPER HA MAYO CLIN PROC 46 521 971 004 NAMMACHER MA J PEDIATR 76 549 970 005 DAM H ACTA MED SCAND 112 210 942 006 GOLDMAN HI PEDIATRICS 44 745 969 007 MOSS MH AM J DIS CHILD 117 540 969 008 WALTERS TR AM J DIS CHILD 124 641 972 009 TORSTENSON OL PEDIATRICS 45 857 970 010 DOLAN TF JR J PEDIATR 77 515 970 011 KOMP DM CHEST 58 501 970 012 DANNAWI N CLIN RES 22 84A 974 CT 1 CARPENTIERI U SOUTH MED J 71 854 978 2 CABALLERO FM PEDIATRICS 65 161 980 3 ROSENSTEIN BJ PEDIATRICS 73 877 984 4 LANE PA J PEDIATR 106 351 985 PN 76115 RN 00469 AN 76216936 AU Laraya-Cuasay-L-R. Cundy-K-R. Huang-N-N. TI Pseudomonas carrier rates of patients with cystic fibrosis and of members of their families. SO J-Pediatr. 1976 Jul. 89(1). P 23-6. MJ CARRIER-STATE: mi. CYSTIC-FIBROSIS: mi. PSEUDOMONAS-INFECTIONS. MN ADOLESCENCE. ANTIBIOTICS: pd. ANTIBODIES-BACTERIAL: an. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. NOSE: mi. PSEUDOMONAS-AERUGINOSA: de, ip. PSEUDOMONAS-INFECTIONS: co, fg. PYOCINS. SEROTYPING. SKIN: mi. SUPPORT-U-S-GOVT-P-H-S. AB The majority (86.6%) of patients with cystic fibrosis were found to be carriers of Pseudomonas aeruginosa. None of them, however, carried P. aeruginosa in their nares. In contrast, none of the non-CF family members of the patients with CF were carriers of P. aeruginosa. For example, only 4 of 468 cultures from skin, throat, and nares of the family members were positive for P. aeruginosa. Isolations of P. aeruginosa from the same CF patients were often of the same pyocine type. No specific pyocine type of P. aeruginosa was predominant in patients with CF. Isolations of P. aeruginosa from siblings with CF may or may not be of the same pyocine type as that of the family proband. Colonization of a patient with CF by P. aeruginosa is not a threat to the non-CF members of the family. RF 001 HUANG NN J PEDIATR 59 512 961 002 HUANG NN J PEDIATR 62 36 963 003 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 004 HUANG NN J PEDIATR 78 338 971 005 FARMER JJ 3RD PROC AM SOC MICROBIOL ANN MTG 261 123 972 006 FARMER JJ 3RD APPL MICROBIOL 18 760 969 007 SCHLICHTER JG AM HEART J 34 209 947 008 HUANG NN CF CLUB ABST 11 19 970 009 DIAZ F J INFECT DIS 121 269 970 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 STERN RC JAMA 239 2676 978 3 BEAUDRY PH J PEDIATR 97 144 980 4 STERN RC LANCET 1 1401 982 5 BAKER NR CURRENT MICROBIOL 7 35 982 6 SPEERT DP J PEDIATR 101 227 982 7 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 8 ZIMAKOFF J J HOSP INFECT 4 31 983 9 HASTIE AT INFECT IMMUN 40 506 983 10 KESTEL FG KLIN PAEDIATR 195 330 983 11 BUCKMIRE FLA MICROBIOS 41 49 984 12 DUNNE WM MICROBIOS 43 193 985 13 MARCUS H INFECT IMMUN 47 723 985 14 DUNNE WM APPL ENVIRON MICROBIOL 50 562 985 15 LUZAR MA INFECT IMMUN 50 572 985 16 LUZAR MA INFECT IMMUN 50 577 985 17 LINDEMANN RA J DENT RES 64 54 985 18 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 19 PADGETT PJ CURRENT MICROBIOL 14 187 986 20 HINGLEY ST INFECT IMMUN 51 254 986 21 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 22 HARDY KA J PEDIATR 109 51 986 23 DUNNE WM DIAGN MICROBIOL INFECT DIS 6 165 987 24 SPEERT DP J HOSP INFECT 9 11 987 25 OGLE JW J INFECT DIS 155 119 987 PN 76116 RN 00470 AN 76096876 AU Papp-Z. Karsai-T. Gyurkovits-K. TI Letter: Protein level in the meconium of a homozygotic and a heterozygotic infant with cystic fibrosis. SO J-Pediatr. 1976 Jan. 88(1). P 151. MJ CYSTIC-FIBROSIS: me. MECONIUM: an. PROTEINS: an. MN COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INFANT-NEWBORN. MALE. EX In our patient, the second child of a married couple heterozygous for cystic fibrosis , the meconium protein content was higher than that of controls. He has no symptoms of cystic fibrosis. The determination of sodium and chloride ions in the sweat excluded homozygosity; the bromide test led us to conclude the child to be a heterozygote. In our patient the protein content of the meconium, the majority of which was albumin, was two- to threefold that of the controls. This elevated protein content, however, was not characteristic of homozygosity. Our results lead us to conclude that a high protein content of meconium does not unconditionally indicate the homozygotic state for cystic fibrosis. It would appear that heterozygosity for cystic fibrosis is not completely without abnormality. Our findings perhaps explain "false positive" results recorded in the literature. High-protein content in the meconium may be useful in the identification of heterozygotes too. RF 001 BUCHANAN DJ PEDIATRICS 9 304 952 002 SCHUTT WH ARCH DIS CHILD 43 178 968 003 GREEN MN PEDIATRICS 41 989 968 004 PROSSER R ARCH DIS CHILD 49 597 974 005 KOLLBERG H ARCH DIS CHILD 47 836 972 006 BULL FE ARCH DIS CHILD 49 602 974 CT 1 PAPP Z CLIN GENET 11 431 977 PN 76117 RN 00471 AN 76263960 AU Cohen-L-F. Farrell-P-M. Lundgren-D-W. di-SantAgnese-P-A. TI Electrolyte values of sweat obtained by local and whole body collection methods in cystic fibrosis patients. SO J-Pediatr. 1976 Sep. 89(3). P 430-3. MJ CHLORIDES: an. CYSTIC-FIBROSIS: me. SODIUM: an. SWEAT: an. MN ADULT. HUMAN. MALE. EX The composition of sweat and its relationship to the condition and site of collection are of interest in environmental physiology, dermatology, and the study of cystic fibrosis. This study was undertaken to compare the concentrations of sodium and chloride in whole body sweat of normal control patients and of CF patients obtained by thermal stimulation with samples collected locally by pilocarpine iontophoresis. Electrolyte concentrations in whole body sweat samples correlated in a highly significant fashion with sodium and chloride values found in arm patch sweat. Patients with CF, however, showed significantly higher sodium and chloride concentrations in arm patch sweat and whole body sweat, and increased sodium excretion rates. RF 001 SCHULZ IJ J CLIN INVEST 48 1470 969 002 MANGOS JA PEDIATR RES 1 436 967 003 KAISER D PEDIATR RES 5 167 971 004$ LADILL WSS J PHYSIOL (LOND) 107 465 948 005 GORDON RS J APPL PHYSIOL 31 713 971 006 GOCHBERG SH PEDIATRICS 18 701 956 007 SIBINGA MS J APPL PHYSIOL 18 1226 963 008 CAGE GW J CLIN INVEST 45 1373 966 CT 1 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 2 HEELEY AF CLIN CHEM 29 2011 983 3 FELLMANN N EUR J APP PHYSI OCCUP PHYSIOL 54 511 985 PN 76118 RN 00472 AN 76263954 AU Stern-R-C. Boat-T-F. Doershuk-C-F. Tucker-A-S. Primiano-F-P-Jr. Matthews-L-W. TI Course of cystic fibrosis in 95 patients. SO J-Pediatr. 1976 Sep. 89(3). P 406-11. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FEMALE. FOLLOW-UP-STUDIES. HUMAN. LUNG-DISEASES: co. MALE. SUPPORT-U-S-GOVT-NON-P-H-S. AB The course of 95 patients with cystic fibrosis is presented. Survivors have a mean follow-up period of over 14 years (minimum: 13 years). Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only one has died and none is disabled. In contrast, of the other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 26 have died. Mortality and morbidity has been greater in females. Possible factors contributing to the improving prognosis include early diagnosis, aggressive management with comprehensive care, easy access to specialized care, and improved antimicrobial therapy. RF 001 DOERSHUK CF J PEDIATR 65 677 964 002 MATTHEWS LW J PEDIATR 65 558 964 003 SHWACHMAN H AM J DIS CHILD 96 615 958 004 HUANG NN AM J DIS CHILD 120 289 970 005 LAWSON D ARCH DIS CHILD 47 1 972 006 MEARNS MB ARCH DIS CHILD 47 251 972 007 MATTHEWS LW IN: MANGOS JA 303 973 008 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 009 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 010 GELLIS SS YEARBOOK OF PEDIATRICS 289 971 011 GELLIS SS YEARBOOK OF PEDIATRICS 176 972 012 GELLIS SS YEARBOOK OF PEDIATRICS 168 973 013 WARWICK WJ STATISTICAL ANALYSIS OF NATIO 974 014 CUTLER SJ J CHRON DIS 8 699 958 015 STOWE SM AM REV RESPIR DIS 111 611 975 016 STERN RC GASTROENTEROLOGY 70 645 976 017 RACHELEFSKY GS AM J DIS CHILD 128 355 974 CT 1 STERN RC J PEDIATR 89 412 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 LUCK SR J THORAC CARDIOVASC SURG 74 834 977 4 STERN RC ANN INTERN MED 87 188 977 5 KNOKE JD PEDIATR RES 12 676 978 6 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 7 ZAPLETAL A BULL EUR PHYSIOPATH RESP 15 575 979 8 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 9 FRYDMAN MI J CHRON DIS 32 211 979 10 RYLEY HC ARCH DIS CHILD 54 92 979 11 SANTAGNESE PAD AM J MED 66 121 979 12 WEISMAN Y J PEDIATR 95 416 979 13 PIEPSZ A J NUCL MED 21 909 980 14 MULLERWIEFEL DE MONATSSCHR KINDERHEILKD 128 692 980 15 MOSHANG T AM J DIS CHILD 134 1139 980 16 STERN RC AM J DIS CHILD 134 267 980 17 PASSERO MA CLIN PEDIATR 20 264 981 18 PEDERSEN PS ACTA PAEDIATR SCAND 70 507 981 19 LAMBERT JR GASTROENTEROLOGY 80 169 981 20 REITER EO AM J DIS CHILD 135 422 981 21 REITER EO CLIN ENDOCRINOL 16 127 982 22 KISTLER I HELV PAEDIATR ACTA 36 495 982 23 HELLSING K ACTA PAEDIATR SCAND 71 827 982 24 REDDING GJ AM REV RESPIR DIS 126 31 982 25 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 26 FRATES RC AM J DIS CHILD 136 279 982 27 WARWICK WJ ACTA PAEDIATR SCAND SUPPL 301 1982 27 982 28 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 29 PEDERZINI F RIV ITAL PEDIATR 9 445 983 30 ANON LANCET 1 663 984 31 SATEGNAGUIDETTI C GASTROENTEROL CLIN BIOL 8 141 984 32 PHELAN P ARCH DIS CHILD 59 71 984 33 LEWIS MI S AFR MED J 65 641 984 34 SEALE TW ANN CLIN LAB SCI 15 152 985 35 CONWAY SP ACTA PAEDIATR SCAND 74 107 985 36 DUHAMEL JF ARCH FR PEDIATR 43 229 986 37 MAHANEY MC HUM BIOL 58 445 986 38 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 39 LITTLEWOOD JM J ROY SOC MED 79 55 986 40 KATZ JN J PEDIATR 108 352 986 PN 76119 RN 00473 AN 76263955 AU Stern-R-C. Doershuk-C-F. Boat-T-F. Tucker-A-S. Primiano-F-P-Jr. Matthews-L-W. TI Course of cystic fibrosis in black patients. SO J-Pediatr. 1976 Sep. 89(3). P 412-7. MJ CYSTIC-FIBROSIS: di. NEGROID-RACE. MN CHILD. CHILD-PRESCHOOL. FEMALE. FOLLOW-UP-STUDIES. HUMAN. INFANT. MALE. RESPIRATORY-FUNCTION-TESTS. SUPPORT-U-S-GOVT-NON-P-H-S. AB Seventeen black patients showed typical sweat gland, gastrointestinal, and pulmonary manifestations of cystic fibrosis. There was an unusually high incidence of meconium ileus equivalent (35%). Three infant siblings of patients in this study had died of cystic fibrosis prior to referral of a family member to this center. One patient died at age four years after emotional problems interfered with the treatment program; status asthmaticus played a major role in his terminal respiratory failure. The remainder of the patients have had substantially less morbidity from pulmonary disease than a comparable, much larger group of white patients. Only one patient, age 28, who is married and employed full-time has severe pulmonary involvement as assessed by pulmonary function studies and chest roentgenogram. The 16 surviving patients have a mean age of 13 1/2 years after a mean follow-up period of 10 years. If black patients with cystic fibrosis survive infancy, they then, as a group, may have a relatively good prognosis. RF 001 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 002 STEINBERG AG AM J HUM GENET 12 416 960 003 KULCZYCKI LL AM J DIS CHILD 127 64 974 004 DI SANTAGNESE PA ANN INTERN MED 54 482 961 005 PILEGGI A DELAWARE MED J 34 97 962 006 MACDOUGALL LG LANCET 2 409 962 007 LEVIN SE S AFR MED J 41 482 967 009 OPPENHEIMER EH PEDIATRICS 42 547 968 010 KULCZYCKI LL CLIN PEDIATR 3 692 964 011 SENECAL JJ BULL MED AFRI OCCID FRANC 11 95 954 012 MATTHEWS LW J PEDIATR 65 558 964 014 DUBOIS AB J CLIN INVEST 35 322 956 015 DUBOIS AB J CLIN INVEST 35 322 956 016 SHWACHMAN H AM J DIS CHILD 96 615 958 017 CUTLER SJ J CHRON DIS 8 699 958 018 STERN RC J PEDIATR 89 406 976 019 ROBERTS GBS ANN HUM GENET 24 127 960 020 SHWACHMAN H N ENGL J MED 286 1300 972 021 DOLAN TF JR CLIN PEDIATR 14 862 975 CT 1 HEFFER ET J PEDIATR 90 324 977 2 FLANIGAN RC J PEDIATR SURG 13 435 978 3 ZAPLETAL A BULL EUR PHYSIOPATH RESP 14 265 978 4 KULCZYCKI LL J PEDIATR 92 855 978 5 LOPEZCORELLA E PATOLOGIA (MEXICO CITY) 18 167 980 6 ROSENSTEIN BJ AM J DIS CHILD 134 72 980 7 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 8 ROSENBERG E KLIN PAEDIATR 195 323 983 9 ROSENSTEIN BJ AM J DIS CHILD 140 966 986 PN 76120 RN 00474 AN 77008518 AU Anthony-Di-Sant-Agnese-P. Blanc-W-A. TI Infarction of the common bile duct in an infant after surfical exploration for jaundice: a possible cause of extrahepatic biliary atresia. SO J-Pediatr-Surg. 1976 Aug. 11(4). P 543-5. MJ BILIARY-TRACT-DISEASES: et. COMMON-BILE-DUCT: bs. INFARCTION: et. CHOLESTASIS: su. SURGERY-OPERATIVE: ae. MN BILIARY-TRACT: pa. CASE-REPORT. CHOLANGITIS: co. CYSTIC-FIBROSIS: co. HEPATIC-DUCT-COMMON: pa. HUMAN. INFANT. CHOLESTASIS: co. MALE. AB An infant explored for obstructive jaundice died 6 days postoperatively and was found to have extensive infarction of the common duct. This would have gone undetected had the patient survived and without careful sampling at autopsy. The incidence of this unsuspected complication is unknown. Surgical infarction may be one cause of fibrosis and secondary atresia of the extra-hepatic bile ducts. RF 001 LANDING BH PROG PEDIATR SURG 6 113 974 002 GELLIS SS PEDIATRICS 55 8 975 003 KOOP CE PEDIATRICS 55 8 975 004 LOUW JH J PEDIATR SURG 1 8 966 005 CAMERON GR J PATHOL BACTERIOL 35 769 932 006 BENNETT DE PEDIATRICS 33 735 964 007 SHORTER RB MAYO CLIN PROC 36 148 961 008 HOLDER TM AM J SURG 107 458 964 009 BRENT RL J PEDIATR 61 111 962 010 THALER MM AM J DIS CHILD 116 262 968 011 GELLIS SS AM J DIS CHILD 88 285 954 012 NORRIS WT AM J SURG 94 321 957 013 SANTULLI TV PEDIATRICS 26 27 960 CT 1 SCHWEIZER P MONATSSCHR KINDERHEILKD 128 292 980 2 TODANI T Z KINDERCHIRURGIE 38 400 983 PN 76121 RN 00475 AN 76171335 AU Holsclaw-D-S. Habboushe-C. TI Occult appendiceal abscess complicating cystic fibrosis. SO J-Pediatr-Surg. 1976 Apr. 11(2). P 217-21. MJ ABSCESS: co. APPENDICITIS: co. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. APPENDICITIS: di, su. CASE-REPORT. CHILD. CHILD-PRESCHOOL. HUMAN. MALE. AB Three case histories illustrate the diagnostic dilemma encountered whenever a patient with CF who is receiving antibiotics is evaluated for abdominal pain. Although acute appendicitis with perforation and abscess formation is not a common complication of CF, it occurs more frequently than is generally appreciated. The large number of abdominal situations in CF which can cause pain confused with but not typical of acute appendicitis. The true underlying condition is further masked by the concurrent use of antibiotics for pulmonary infection. A higher index of suspicion is needed to rule out acute appendicitis in a patient with cystic fibrosis and abdominal pain. A greater awaremess of the possibility of occult appendiceal abscess may help to avoid this complication. RF 001 GRAND RJ CLIN PEDIATR 9 588 970 002 KOPEL FB GASTROENTEROLOGY 62 483 972 003 HOLSCLAW DS J PEDIATR SURG 9 867 974 004 SNYDER WH JR PEDIATRICS 34 72 964 005 HOLSCLAW DS PEDIATRICS 48 51 971 006 SHWACHMAN H PEDIATRICS 55 86 975 008 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 HOLGERSEN LD AM J DIS CHILD 122 288 971 011 WILKINSON RH AM J DIS CHILD 118 687 969 012 HATTEN LE SOUTH MED J 66 803 973 013 STORER EH IN: SCHWARTZ S 1021 969 014 GONGAWARE RD J PEDIATR SURG 8 33 973 015 BELL MJ AM J DIS CHILD 129 138 975 016 SHANDLING B J PEDIATR SURG 9 79 974 017 SHWACHMAN H N ENGL J MED 286 1300 972 CT 1 OESTREICH AE J PEDIATR SURG 17 191 982 2 ROSENBERG E KLIN PAEDIATR 195 323 983 3 MCCARTHY VP GASTROENTEROLOGY 86 564 984 4 GEORGE DH HUM PATHOL 18 75 987 PN 76122 RN 00476 AN 76097309 AU Carter-R-A. TI The value of screening in paediatrics. SO J-R-Coll-Physicians-Lond. 1976 Jan. 10(2). P 153-60. MJ MASS-SCREENING. PEDIATRICS. MN ALPHA-FETOPROTEINS: df. ALPHA-1-ANTITRYPSIN: df. AMINO-ACID-METABOLISM-INBORN-ERRORS: di. CYSTIC-FIBROSIS: di. EVALUATION-STUDIES. GALACTOSEMIA: di. GLUCOSEPHOSPHATE-DEHYDROGENASE-DEFICIENCY: di. HARTNUP-DISEASE: di. HIP-DISLOCATION-CONGENITAL: di. HOMOCYSTINURIA: di. HUMAN. HYPOTHYROIDISM: cn. INFANT. INFANT-NEWBORN. MAPLE-SYRUP-URINE-DISEASE: di. PHENYLKETONURIA: di. EX Population screening is the carrying out of some test or procedure on every individual within a population defined by such limits as geography, administration, race, sex or age. At risk screening is the carrying out of such a test or procedure on a person or persons for whom there already exists some medical reason to suppose that the findings may be positive. This review of conditions for which population screening may be considered is not exhaustive, but it serves to show the central dilemma in this field, which is that, apart from congenital dislocation of the hip, phenylketonuria and possibly homocystinuria, none seems to show quite strong enough a case to be considered worthwhile screening for on its own. RF 001 ABULID J J CLIN ENDOCRINOL METAB 39 263 974 002 BARNES ND ARCH DIS CHILD 50 497 975 003 ANON BR MED J 3 489 971 004 ANON BR MED J 1 527 974 005 BROCK DJH LANCET 2 923 973 006 BROCK DJH LANCET 2 197 972 007 DACOU-VOUTETAKIS C ARCH DIS CHILD 48 826 973 008 DEALL AM BR MED J 3 639 971 009 DICKSON LG CLIN CHEM 19 301 973 010 DOW PA AM J CLIN PATHOL 61 333 974 011 ENDRE L LANCET 1 631 974 012 GUTHRIE R US DHEW CHILDRENS BUREAU PUBL 965 013 HARRIS R LANCET 1 429 974 014 HAWCROFT J HEALTH TRENDS 6 72 974 015 HOLLAND WW LANCET 2 1494 974 016 HOLT KS LANCET 2 1057 974 017 HOLTON JB ANN CLIN BIOCHEM 9 118 972 018 KLEIN AH LANCET 2 77 974 019 KLEIN A PEDIATR RES 5 399 971 021 LAURENCE KM LANCET 2 939 974 022 LEEK AE LANCET 1 876 974 023 LEEK AE LANCET 2 1151 974 024 LEEK AE LANCET 1 385 973 025 LINDSAY G ANN CLIN BIOCHEM 9 115 972 026 ANON BR MED J 4 7 968 027 MITTMAN C CHEST 60 214 971 028 RAINE DN ANN CLIN BIOCHEM 6 29 969 029 RAINE DN BR MED J 2 329 972 030 RAINE DN BR MED J 3 7 972 032 RAINE DN LANCET 2 996 974 033 RAINE DN TREATMENT OF INHERITED ME 975 034 SCRIVER CR LANCET 2 230 964 035 SEAKINS JWT TREATMENT OF INBORN ERRORS OF 973 036 SELLER MJ LANCET 1 428 974 037 SIMILA S ARCH DIS CHILD 50 565 975 038 STEPHAN U LANCET 1 367 975 039 SUN T AM J CLIN PATHOL 62 725 974 040 THAMBIPILLAI S AM J CLIN PATHOL 63 836 975 041 UDDIN DE JAMA 227 1408 974 042 WALD NJ LANCET 1 765 974 043 WHITBY LG LANCET 2 819 974 044 ANON SCREENING FOR INBORN ERRORS O 968 CT 1 CHALMERS RA ANN CLIN BIOCHEM 14 149 977 2 SMILKSTEIN G J MED EDUC 53 766 978 3 NELSON JH WEST J MED 129 243 978 PN 76123 RN 00477 AN 76122997 AU Blanck-R-R. Mendoza-E-M. TI Fertility in a man with cystic fibrosis. SO JAMA. 1976 Mar 29. 235(13). P 1364. MJ CYSTIC-FIBROSIS. FERTILITY. MN ADULT. CASE-REPORT. CYSTIC-FIBROSIS: co. HUMAN. LUNG: pp. MALE. PANCREAS: pp. RESPIRATORY-FUNCTION-TESTS. SEMEN: an. SPERM-MOTILITY. INFERTILITY-MALE: et. EX Our patient had well-compensated and late-appearing cystic fibrosis manifested by moderately decreased exercise, tolerance and a normal semen analysis, and he had presumably fathered one child. It is important to again emphasize the physician's obligation to determine fertility in any male cystic fibrosis patient, particularly with late-onset of symptoms, and to provide appropriate genetic counseling. RF 001 ROSENLUND ML ANN INTERN MED 78 959 973 002 KAPLAN E N ENGL J MED 279 65 968 003 DENNING CR PEDIATRICS 41 7 968 004 FEIGELSON J ARCH FR PEDIATR 26 937 969 005 TAUSSIG LM N ENGL J MED 287 586 972 006 SHWACHMAN H PEDIATRICS 32 85 963 PN 76124 RN 00478 AN 76122937 AU Lober-C-W. Seigler-H-F. Spock-A. TI Cystic fibrosis in a black woman. SO JAMA. 1976 Mar 15. 235(11). P 1140-1. MJ CYSTIC-FIBROSIS: oc. NEGROID-RACE. MN ADULT. AGE-FACTORS. CASE-REPORT. CYSTIC-FIBROSIS: di. FECES-IMPACTED: di. FEMALE. HEMOPTYSIS: di. HUMAN. INTESTINE-LARGE: ra. RECTAL-PROLAPSE: di. EX This article reports a case of a 22-year-old unmarried black woman with cystic fibrosis. There is only one report in the literature of cystic fibrosis in a black person over 20 years old. Due in part to the deficiency of pancreatic exocrine secretions and the production of a copious amount of highly viscous mucus by gastrointestinal glands, patients with cystic fibrosis are prone to recurrent intestinal obstruction, intussusception, and volvulus, as well as abdominal pain and masses. Shwachman and Holsclaw firmly state that the diagnosis of cystic fibrosis may be made on the histologic appearance of the appendix. RF 001 KULCZYCKI LL AM J DIS CHILD 127 64 974 002 DI SANTAGNESE PA ANN INTERN MED 54 482 961 003 SENECAL JJ BULL MED AFRI OCCID FRANC 11 95 954 004 GROVE SS S AFR J LAB CLIN MED 5 113 959 005 MACDOUGALL LG LANCET 2 409 962 006 LEVIN SE S AFR MED J 41 482 967 007 ZUELZER WW PEDIATRICS 4 53 949 008 PILEGGI A DELAWARE MED J 34 97 962 009 OPPENHEIMER EH PEDIATRICS 42 547 968 010 HEFFER ET NY STATE J MED 74 2355 974 011 BROWN PM N ENGL J MED 263 544 960 012 BECK AR GASTROENTEROLOGIA 106 84 966 013 MURTHY MSN OHIO STATE MED J 69 768 973 014 HOLSCLAW DS J PEDIATR SURG 9 867 974 015 DOLAN TF JR CLIN PEDIATR 14 862 975 016 SHWACHMAN H N ENGL J MED 286 1300 972 PN 76125 RN 00479 AN 77031958 AU Reynolds-H-Y. Di-SantAgnese-P-A. Zierdt-C-H. TI Mucoid Pseudomonas aeruginosa. A sign of cystic fibrosis in young adults with chronic pulmonary disease?. SO JAMA. 1976 Nov 8. 236(19). P 2190-2. MJ CYSTIC-FIBROSIS: di. LUNG-DISEASES: di. PSEUDOMONAS-AERUGINOSA: ip. PSEUDOMONAS-INFECTIONS: di. MN ADOLESCENCE. ADULT. AGE-FACTORS. CASE-REPORT. CHILD. CHRONIC-DISEASE. FEMALE. FOLLOW-UP-STUDIES. HUMAN. MALE. SPUTUM: mi. AB Two adult patients with cystic fibrosis had late onset of symptoms with principal involvement only of the lungs. The absence of other typical features of cystic fibrosis confused the diagnosis. Recovery of mucoid Pseudomonas aeruginosa from sputum cultures helped to establish the diagnosis in these patients, because these mucoid isolates are rarely obtained from subjects who do not have cystic fibrosis. Recent findings, which have identified the mucoid isolates as predominantly a single strain of P aeruginosa and have documented its widespread carriage in the United States by patients with cystic fibrosis, point to an interesting epidemiologic relationship between this bacterium and this disease. RF 001 CETIN ET J BACTERIOL 89 1432 965 002 DOGGETT RG LANCET 1 236 971 003 SHWACHMAN H AM J DIS CHILD 96 6 958 004 GIBSON LE PEDIATRICS 23 545 959 005 FISHER MW J BACTERIOL 98 835 969 006 HOMMA JY JAPAN J EXP MED 41 89 971 007 ZIERDT CH J CLIN MICROBIOL 1 521 975 008 ZIERDT CH J BACTERIOL 87 1003 964 009 REYNOLDS HY ANN INTERN MED 82 819 975 010 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 011 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 012 ELSTON HR AM J CLIN PATHOL 48 519 967 013 ANDERSON CM ARCH DIS CHILD 35 581 960 014 GIBSON LE CLIN PEDIATR 12 450 973 015 BOAT TF PATIENT CARE 120 975 CT 1 MARKOWITZ SM INFECT IMMUN 22 530 978 2 KULCZYCKI LL JAMA 240 30 978 3 SANTAGNESE PAD AM J MED 66 121 979 4 MARRIE TJ J INFECT DIS 139 357 979 5 HOLSCLAW DS CLIN CHEST MED 1 407 980 6 MARKOWITZ SM J ANTIMICROB CHEMOTHER 6 251 980 7 DAVIS PB PEDIATR RES 14 83 980 8 FICK RB CLIN CHEST MED 2 91 981 9 REYNOLDS HY CLIN CHEST MED 2 103 981 10 CARLONE S MICROBIOLOGICA 4 215 981 11 OHMAN DE INFECT IMMUN 33 142 981 12 MASHIMO K JAP J EXP MED 51 271 981 13 MARKS MI J PEDIATR 98 173 981 14 MACONE AB N ENGL J MED 304 1445 981 15 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 16 RIVERA M AM REV RESPIR DIS 126 833 982 17 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 18 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 19 JOHNSON SR OBSTET GYNECOL 61 S 2 983 20 BOUCHER RC LUNG 161 1 983 21 FLOURNOY DJ METH FIND EXP CLIN PHARMACOL 6 487 984 22 KILBOURN JP CURRENT MICROBIOL 11 19 984 23 BOSSO JA DRUG INTEL CLIN PHARM 18 825 984 24 PIER GB J IMMUNOL 133 734 984 25 MCCARTHY VP ARCH INTERN MED 144 408 984 26 ORENSTEIN DM SEM RESPIR MED 6 252 985 27 REYNOLDS HY DISEASE A MONTH 31 1 985 28 ROTH RM SOUTH MED J 78 573 985 29 WOODS DE J INFECT DIS 151 581 985 30 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 31 WOODS DE CLIN INVEST MED 9 108 986 32 PEDERSEN SS J ANTIMICROB CHEMOTHER 17 505 986 33 STUTTS MJ AM REV RESPIR DIS 134 17 986 PN 76126 RN 00480 AN 76172577 AU Nikolajek-W-P. Emrich-H-M. TI pH of sweat of patients with cystic fibrosis. SO Klin-Wochenschr. 1976 Mar 15. 54(6). P 287-8. MJ CYSTIC-FIBROSIS: me. SWEAT: an. MN BICARBONATES: me. HUMAN. HYDROGEN-ION-CONCENTRATION. SWEATING. AB pH of the sweat from patients with cystic fibrosis and in controls was measured as a function of the sweat-rate using a fluorescence-pH- indicator (umbelliferone). In both populations sweat is acid at low sweat-rates and alkaline at high ones. The results do not favour an abnormality of the ductal H+-secretion as the pathomechanism of cystic fibrosis. RF 001 EMRICH HM PFLUEGERS ARCH 290 311 966 002 EMRICH HM PEDIATR RES 2 464 968 003 KAISER D PFLUEGERS ARCH 349 63 974 004 KAISER D MED WELT 25 243 974 005 GRUNHAGEN HH Z NATURFORSCH 25B 373 970 006 EMRICH HM PFLUEGERS ARCH 290 298 966 007 SPOCK A PEDIATR RES 1 173 967 008 MANGOS JA SCIENCE 158 135 967 CT 1 BIJMAN J PEDIATR RES 21 79 987 2 BIJMAN J PFLUGERS ARCH 408 505 987 PN 76127 RN 00481 AN 77054785 AU Czegledy-Nagy-E. Sturgess-J-M. TI Cystic fibrosis: effects of serum factors on mucus secretion. SO Lab-Invest. 1976 Dec. 35(6). P 588-95. MJ CYSTIC-FIBROSIS: pp. MN ANIMAL. CILIA: pa. CYSTIC-FIBROSIS: bl, pa. HUMAN. RABBITS. TRACHEA: pa, ul. AB The effects of serum from children with cystic fibrosis and from normal children on the mucus-secreting, ciliated epithelium have been investigated in vitro using explanted tissue from rabbit lung. By optical and scanning electron microscopy, a sequence of structural changes is observed after incubation with cystic fibrosis serum; this sequence does not occur with normal serum. The earliest changes involve swelling of the goblet cells, with subsequent discharge of mucus onto the epithelial surface. This is followed by disruption of the normally rapid and synchronized ciliary activity. Mucus gradually extends over the surface entangling cilia. Finally, some shedding of ciliated cells occurs from the epithelium. These findings suggest that factors in cystic fibrosis serum cause discharge of mucus leading to a disturbance of the normal ciliary activity in the rabbit lung. It is postulated that such changes result in dysfunction of the mucociliary clearance mechanism and that this dysfunction may be a contributory factor to the pathogenesis of lung disease. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BOWMAN BH IN: MANGOS JA 29 973 004 BOWMAN BH SCIENCE 164 325 969 005 CONOVER JH PEDIATR RES 7 220 973 006 CONOVER JH LANCET 1 1194 973 007 CONOVER JH LIFE SCI 14 253 974 008 CRAWFURD MDA IN: LAWSON D PROC 5TH INT CF 42 969 009 CZEGLEDY-NAGY E PROC MICROSC SOC CAN 2 76 975 010 FORSTNER GG CAN MED ASSOC J 113 550 975 011 KILBURN KH AM REV RESPIR DIS 98 449 968 012 RAO GJS PEDIATR RES 8 684 974 013 SANCHIS J N ENGL J MED 289 749 974 014 SPOCK A PEDIATR RES 1 173 967 015 STANNERS CP NATURE NEW BIOL 230 52 971 017 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 SCHWACHMAN H N ENGL J MED 296 1519 977 2 FORSTNER JF DIGESTION 17 234 978 3 NAGY EC PEDIATR RES 13 729 979 4 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 NICOSIA SV SCIENCE 206 698 979 7 KENNEDY JR PEDIATR RES 14 1173 980 8 KURLANDSKY LE PEDIATR RES 14 1263 980 9 BATTISTA SP ARCH ENVIRON HEALTH 35 239 980 10 PEATFIELD AC THORAX 35 232 980 11 BOWMAN BH FED PROC 39 3195 980 12 WILSON GB J CLIN INVEST 66 1010 980 13 FLEMING N EXPERIENTIA 37 139 981 14 SPECIAN RD AM J ANAT 160 461 981 15 BERGAN T SCAND J INFECT DIS 1981 7 981 16 MORIARTY CM J MED 13 257 982 17 BOAT TF PEDIATR RES 16 792 982 18 REID L ADV EXP MED BIOL 144 369 982 19 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 20 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 21 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 22 QUISSELL DO PEDIATR RES 17 899 983 23 RUTLAND J AM REV RESPIR DIS 128 1030 983 24 MCPHERSON MA CLIN CHIM ACTA 135 181 983 25 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 26 WOOTEN MW J CELL PHYSIOL 121 490 984 27 VONEULER AM LIFE SCI 37 2399 985 28 VONEULER AM EXP MOL PATH 43 142 985 PN 76128 RN 00482 AN 76148660 AU Antonowicz-I. Ishida-S. Shwachman-H. TI Letter: Screening for cystic fibrosis. SO Lancet. 1976 Apr 3. 1(7962). P 746-7. MJ CYSTIC-FIBROSIS: di. INFANT-NEWBORN-DISEASES: di. MN ALBUMINS: du. COMPARATIVE-STUDY. FALSE-POSITIVE-REACTIONS. HUMAN. INFANT-NEWBORN. LACTOSE: du. MECONIUM: an. PROTEINS: an. SWEAT: an. EX Screening for cystic fibrosis (C.F.) in the newborn has been in vogue since the introduction of a rapid simple test based in the presence of albumin in the meconium of C.F. babies. False-positive tests have been observed in prematurity or when the specimen is contaminated with blood. We have reduced the number of false positives by additional studies on the meconium, including detection of lactase. Lactase activity can be detected in meconium from babies with C.F. but not in meconium for healthy babies. In testing 200 normal meconiums with a negative albumin test we noted 3 positive tests for lactase. These 3 patients did not have C.F. These false-positive lactase tests could be distinguished by the addition of distilled water to the meconium instead of lactose. This simple test may provide an additional or alternative method for detection of C.F. in a screening programme. When this test is used in addition to the albumin test the number of false-positive albumin tests will be reduced. RF 001 STEPHAN U PEDIATRICS 55 35 975 002 ANTONOWICZ I PEDIATRICS 56 782 975 003 SHWACHMAN H ANN NY ACAD SCI 93 600 962 CT 1 RYLEY HC LANCET 2 365 976 2 GRIFFITHS AD ARCH DIS CHILD 51 901 976 3 RYLEY HC MONOGR PAEDIATR 10 151 979 4 STEPHAN U MONOGR PAEDIATR 10 41 979 5 BERRY HK AM J DIS CHILD 134 930 980 6 MASTELLA G RIV ITAL PEDIATR 7 581 981 7 MACHILL G BIOL ZENTRALBL 100 449 981 8 DODGE JA ARCH DIS CHILD 57 774 982 9 NAYLOR EW SEM PERINATOL 9 232 985 PN 76129 RN 00483 AN 76266330 AU Ryley-H-C. Neale-L-M. Bray-P-T. TI Letter: False-negative meconium tests for cystic fibrosis. SO Lancet. 1976 Aug 14. 2(7981). P 365-6. MJ CYSTIC-FIBROSIS: di. MECONIUM: an. MN EVALUATION-STUDIES. FALSE-NEGATIVE-REACTIONS. HUMAN. INFANT-NEWBORN. EX Two reviews have questioned the value of meconium tests for the routine screening of cystic fibrosis because of the high proportion of false-positive and (perhaps more importantly) false-negative results. We wish to report our findings on false-negative results. We are evaluating various methods of meconium screening including the Boehringer Mannheim test strip, a semiquantitative test for proteins, electroimmunoassay of albumin, and estimation of the albumin/alpha1-antirypsin ratio. The figures are too small to draw any conclusions between the apparent relationship of false-negative results and a family history of the disease, but we feel that the results illustrate that little reliance should be put on a negative meconium test. This is not to say that the meconium test is valueless. In our study, at least 40% of infants with cystic fibrosis had a positive meconium-test result but did not have meconium ileus and thus the possibility of cystic fibrosis in these infants was recognised within a few days after birth and usually well before definitive clinical signs of the disease were manifest. RF 001 BRIMBLECOMBE FSW LANCET 2 1428 973 002 ANON J PEDIATR 88 711 976 003 ANTONOWICZ I LANCET 1 746 976 004 RYLEY HC CLIN CHIM ACTA 64 117 975 005 STEPHAN U PEDIATRICS 55 35 975 CT 1 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 PN 76130 RN 00484 AN 77076041 AU Conover-J-H. Conod-E-J. Gaerlan-P-F. Bogart-B-I. TI Calcium flux and cystic fibrosis [letter]. SO Lancet. 1976 Dec 18. 2(7999). P 1362-3. MJ CALCIUM: me. CYSTIC-FIBROSIS: me. MN A-23187: pd. ANIMAL. BIOLOGICAL-ASSAY. CELL-MEMBRANE-PERMEABILITY: de. CILIA: ph. HUMAN. RABBITS. TISSUE-CULTURE. TRACHEA: pa. EX In an effort to determine whether increased permeability to calcium ions could explain the cystic fibrosis-serum ciliary dyskensia factor response in the tracheal test system, we selectively increased the membrane permeability of the tracheal epithelium to calcium ions by adding calcium ionophore A23187 to the tracheal culture medium. These studies determined that calcium ionophore A23187 in culture medium produced a change in mucociliary function in the tracheal test system indistinguishable from that promoted by C.F. sera. These investigations, which are now being extended to include the ultrastructural alterations induced in the epithelium of the rabbit tracheal bioassay system, point to an important role for calcium as a cofactor in the production of the C.D.F. response generated by the C.F sera. The C.F. serum substance seems to act at the cellular-membrane level, promoting membrane-permeability changes (at least with respect to calcium) in the rabbit tracheal test system. Such a mechanism would not necessarily result in a net physiological imbalance of calcium in the rabbit tracheal system but only local changes in calcium concentration that would subsequently alter secretion and microtubular function. Since C.F. serum, like sweat and saliva from these same patients, can promote ionic fluxes, perhaps there is a common factor in all these body-fluids which is related to the basic defect in C.F. It is tempting to speculate that such a factor produces a membrane-associated phenomenon that may be generally representative of the in-vivo C.F. disorder and as such may reflect the nature of the cellular and glandular abnormalities of the disease. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 MANGOS JA PEDIATR RES 1 436 967 003 MANGOS JA SCIENCE 158 135 967 004 WOTMAN S ARCH ORAL BIOL 16 663 971 005 CONOVER JH PEDIATR RES 7 220 973 006 COCHRANE DE PROC NAT ACAD SCI USA 71 408 974 007 SATIR P SCIENCE 190 586 975 CT 1 KENNEDY JR PEDIATR RES 14 1173 980 2 KATZ S CELL CALC 5 421 984 PN 76131 RN 00485 AN 76195104 AU McFarlane-H. Allan-J-D. TI Letter: Cystic fibrosis heterozygosity in pathogenesis of allergy. SO Lancet. 1976 Jun 5. 1(7971). P 1241. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HYPERSENSITIVITY: et. MN ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: et. HUMAN. HYPERSENSITIVITY: di, fg. IGE: an. EX The article by Dr Warner and colleagues may be of great significance to our understanding of the aetiology and pathogenesis of cystic fibrosis (C.F.), and accords with our observations that allergic symptoms and positive skin tests are more common in C.F. homozygotes than in healthy controls. Apart from the fact that 59% of our C.F. homozygotes gave a positive skin test to one or more of 40 different classes of Bencard antigens, the main one being Aspergillus fumigatus, at least 85% of the C.F. heterozygotes had a strong positive history of allergy, of which the mother and her maternal progenitors, followed by the siblings, accounted for most. Further studies are required to establish the relationship, if any, between the gene responsible for the allergic manifestation in the population and the C.F. phenotype. RF 001 WARNER JO LANCET 1 990 976 002 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 003 ALLAN JD CLIN ALLERGY 5 255 975 004 MCFARLANE H BR MED J 1 423 975 005 GIBBONS A BR MED J 1 120 976 CT 1 GYURKOVITS K LANCET 1 203 977 2 MCFARLANE H CLIN ALLERGY 7 279 977 3 SILVERMAN M ARCH DIS CHILD 53 873 978 4 CARSWELL F MONOGR PAEDIATR 10 144 979 5 CARSWELL F CLIN EXP IMMUNOL 35 141 979 6 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 7 MOSS RB AM REV RESPIR DIS 121 23 980 8 CLARKE CW BR J DIS CHEST 75 15 981 9 WILSON GB MED HYPOTHESES 8 527 982 PN 76132 RN 00486 AN 78070537 AU Bentley-D. Moore-A. Shwachman-H. TI Finger Clubbing: A quantitative survey by analysis of the shadowgraph. SO Lancet. 1976 Jul 24. 2(7978). P 164-7. MJ NAILS. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: di. MN ADOLESCENCE. ADULT. ASTHMA: co. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. HEART-DEFECTS-CONGENITAL: co. HUMAN. METHODS. SUPPORT-U-S-GOVT-P-H-S. AB Finger-clubbing has been measured from the magnified silhouette of the right index finger (shadowgram) in 125 subjects, comprising children and young adults. The profile and hyponychial angles were both found to be good discriminators of clubbing and to be independent of age and sex. The normal profile angle was 168.3 degrees +/- 3.6 and the mean normal hyponychial angle was 180.1 degrees +/- 4.2. Patients with cystic fibrosis and cyanotic congenital heart-disease were found to have grossly elevated values of these angles. Some asthmatics were found to have a moderate degree of clubbing with significantly elevated values of these angles. The shadowgram proved a convenient objective method for recording the degree and progress of fingerclubbing in clinical practice. RF 001 TROUSSEAU A CONNAISSANCES MED CHIR 351 833 002 ADAMS F IN: GENUINE WORKS OF HIPP 1 249 849 003 LOVIBOND JL LANCET 1 363 938 004 LOVELL RRH CLIN SCI 9 299 950 005 BIGLER FC AM J PATHOL 34 237 958 006 CUDKOWICZ L BR J TUBERC DIS CHEST 51 14 957 007 STAVEM P LANCET 2 7 959 008 CARROLL DG TRANS AM CLIN CLIMAT ASSOC 83 198 972 009 NEUFELD O OHIO STATE MED J 48 834 952 010 MELLINS RB CIRCULATION 33 143 966 011 REGAN GM LANCET 1 530 967 012 WARING WW AM REV RESPIR DIS 104 166 971 013 BENTLEY D BR MED J 3 43 970 014 SLY RM ANN ALLERGY 30 575 972 015 PYKE DA LANCET 2 352 954 016 WARING WW CF CLUB ABST 967 CT 1 SINNIAH D J PEDIATR 92 597 978 2 KITIS G BR MED J 2 825 979 3 SINNIAH D ARCH DIS CHILD 54 145 979 4 ROTHSCHILD BM AM J GASTROENTEROL 73 503 980 5 BLUMSOHN D HEART LUNG 10 1069 981 6 SHNEERSON JM BR J DIS CHEST 75 75 981 7 PITTSTUCKER TJ ARCH DIS CHILD 61 576 986 PN 76133 RN 00487 AN 76173255 AU Lipson-A. TI Letter: Infecting dose of Salmonella. SO Lancet. 1976 May 1. 1(7966). P 969. MJ SALMONELLA: py. MN CYSTIC-FIBROSIS: dt. DRUG-CONTAMINATION. GASTROENTERITIS: mi. CROSS-INFECTION: mi. HUMAN. INFANT. PANCREATIN: ae. SALMONELLA-INFECTIONS: mi. EX An outbreak of salmonellosis in patients consuming pancreatin in the U.K. prompted a study at this hospital which included determination of the infecting dose in children with cystic fibrosis known to have contracted a S. schwarzengrund enteritis from contaminated porcine powdered pancreatin. We found that infection was especially prevalent in very young cystic-fibrosis patients who were receiving broad-spectrum antibiotic treatment for chest infection. All had their pancreatin added to meals immediately before consumption. Susceptibility to most infections depends on many factors and variables. It is necessary to take into account such things as age and condition of the patient when discussing the actual number of organisms causing an infection. RF 000 DAOUST JY LANCET 1 866 976 004 ROWE B BR MED J 4 51 975 006 LANG DJ N ENGL J MED 276 829 967 007 MCCULLOUGH NB J INFECT DIS 88 276 951 008 MCCULLOUGH NB J INFECT DIS 89 209 951 009 MCCULLOUGH NB J INFECT DIS 89 259 951 010 HORNICK RB N ENGL J MED 288 686 973 011 BOHNHOFF M J INFECT DIS 111 117 962 012 BENNETT IL ANNU REV MED 10 1 959 CT 1 CRAUN GF J WATER POLLUT CONTROL FED 49 1268 977 2 DICKINSON RJ SCOTT MED J 23 23 978 3 TURNBULL PCB CLIN GASTROENTEROL 8 663 979 4 SILLIKER JH J FOOD PROTECT 43 307 980 5 PALMER SR LANCET 1 881 981 6 BLASER MJ REV INFECT DIS 4 1096 982 7 SCOTT E J HYG LOND 89 279 982 8 LEE A ADV MICROB ECOL 8 115 985 PN 76134 RN 00488 AN 76173321 AU Warner-J-O. Norman-A-P. Soothill-J-F. TI Cystic fibrosis heterozygosity in the pathogenesis of allergy. SO Lancet. 1976 May 8. 1(7967). P 990-1. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HYPERSENSITIVITY: et. MN ALLERGENS: du. COMPARATIVE-STUDY. FEMALE. HOMOZYGOTE. HUMAN. HYPERSENSITIVITY: di, fg. MALE. SKIN-TESTS. AB 47% of cystic fibrosis (C.F.) heterozygotes had positive prick skin tests of 1 or more of 9 antigens and 53% had histories of allergic disease, both occurring significantly more often than in a control group. Since 1 in 20 of the population are C.F. carriers, this would contribute to allergy in the community. RF 001 TAYLOR B LANCET 2 111 973 002 MEARNS MB LANCET 1 538 967 003 WARREN CPW CLIN ALLERGY 5 1 975 004 ALLAN JD CLIN ALLERGY 5 255 975 006 BLAIR H CLIN ALLERGY 4 389 974 007 COUNAHAN R ARCH DIS CHILD 50 477 975 008 SMITH JM MED CLIN NORTH AM 48 3 974 009 SPOCK A PEDIATR RES 1 173 967 010 BESLEY GTN J MED GENET 6 278 969 011 CAPLIN I ANN ALLERGY 31 320 973 CT 1 GYURKOVITS K LANCET 1 203 977 2 MCFARLANE H CLIN ALLERGY 7 279 977 3 RAEBURN JA PROC NUTR SOC 36 77 977 4 WILLCOX HNA IMMUNOGENETICS 6 209 978 5 SILVERMAN M ARCH DIS CHILD 53 873 978 6 TURNER MW ARCH DIS CHILD 53 631 978 7 PRICE JF CLIN ALLERGY 9 563 979 8 CARSWELL F MONOGR PAEDIATR 10 144 979 9 CARSWELL F CLIN EXP IMMUNOL 35 141 979 10 ZAMBIE MF ANN ALLERGY 42 290 979 11 VANASPEREN PP AUST PAEDIATR J 16 53 980 12 HODSON ME THORAX 35 801 980 13 ORMEROD LP THORAX 35 768 980 14 SKOV PS ALLERGY 35 23 980 15 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 16 TOBIN MJ THORAX 35 807 980 17 WANG A ARCH DIS CHILD 55 130 980 18 HOLZER FJ ARCH DIS CHILD 56 455 981 19 CLARKE CW BR J DIS CHEST 75 15 981 20 ZETTERSTROM O BR MED J 283 1215 981 21 MANKU MS PROSTAGLANDINS LEUKOTR MED 9 615 982 22 ZETTERSTROM O J ALLERGY CLIN IMMUNOL 75 594 985 PN 76135 RN 00489 AN 77010669 TI The later years of cystic fibrosis [editorial]. SO Lancet. 1976 Sep 18. 2(7986). P 614-5. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co, th. FOLLOW-UP-STUDIES. HUMAN. EX According to Schwachman and Holsclaw, a cystic-fibrosis child who does not present with meconium ileus and who receives optimum care now has an 80% chance of reaching adulthood. The main feature needing continuous medical care in the older CF patient is persisting, and often progressive, chest disease. CF patients often owe their lives to paediatrician and to parents who have persevered with an arduous and exacting treatment regimen. Now that such patients are reaching adult life, we must continue to offer the best possible support. RF 013 SHWACHMAN H N ENGL J MED 281 500 969 014 SHWACHMAN H PEDIATRICS 36 689 965 015 SHWACHMAN H CF CLUB ABST 13 974 016 GRACEY M AUSTRALAS ANN MED 18 91 969 017 MITCHELL-HEGGS PF Q J MED 45 479 976 018 PROSSER R ARCH DIS CHILD 49 597 974 019 STEPHAN U PEDIATRICS 55 35 975 020 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 PN 76136 RN 00490 AN 77055460 AU Bowman-B-H. TI Current biochemical approaches in cystic fibrosis research. SO Life-Sci. 1976 Nov 1. 19(9). P 1289-96. (REVIEW). MJ CYSTIC-FIBROSIS. MN BIOLOGICAL-TRANSPORT. CILIA: ph. CYSTIC-FIBROSIS: co, fg, me. GASTROINTESTINAL-DISEASES: et. GLYCOPROTEINS: bi. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IONS. LUNG-DISEASES: et. MUCUS: me. POLYAMINES: me. PROTEINS: me. REVIEW. SODIUM: me. SULFATES: me. SWEAT: an. SUPPORT-U-S-GOVT-P-H-S. EX The clinical abnormalities in cystic fibrosis can be categorized into three headings: pulmonary disease, digestive disorders, and ion transport abnormalities. Research directions in cystic fibrosis have evolved from observations of the characteristic malfunctions inherent in the disease. These include defects in mucociliary function, mucous secretion, membrane transport and glycoprotein biosynthesis. Drawing from what is known about the bases of other inherited diseases, one could predict that cystic fibrosis results from an abnormal functional protein, as in sickle cell anemia; an enzyme deficiency, as in phenylketonuria; or a deficiency of a normal inhibitor, as in hereditary angioedema. RF 001 STEINBERG AG AM J HUM GENET 12 416 960 002 MCCOMBS ML TEX REP BIOL MED 31 615 973 003 LEVISON H IN: MANGOS JA 3 976 004 BARBERO GJ IN: MANGOS JA 83 976 005A FANCONI G WIEN MED WOCHENSCHR 86 753 936 005B ANDERSEN DH AM J DIS CHILD 56 344 938 006 DI SANTAGNESE PA PEDIATRICS 12 549 953 007 MANGOS JA PEDIATR RES 1 436 967 008 HIRSCHHORN K IN: MANGOS JA 11 973 009 KNUDSON AG JR AM J HUM GENET 19 388 967 011 SPOCK A PEDIATR RES 1 173 967 012 BOWMAN BH SCIENCE 167 871 970 013 MCCOMBS ML CLIN GENET 1 171 970 014 BARNETT DR TEX REP BIOL MED 31 703 973 015 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 016 BARNETT DR TEX REP BIOL MED 31 697 973 017 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 018 BERATIS NG PEDIATR RES 7 958 973 019 BESLEY GTN J MED GENET 6 278 969 020 CONOVER JH PEDIATR RES 7 224 973 021 DANES BS J EXP MED 136 1313 972 022 DANES BS J EXP MED 137 1538 973 023 MANGOS JA PEDIATR RES 1 436 967 024 MANGOS JA TEX REP BIOL MED 31 651 973 025 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 026 BOWMAN BH IN: MANGOS JA 29 973 027 WOOD RE AM REV RESPIR DIS 111 733 975 029 DANES BS AM J HUM GENET 25 323 973 031 RAO GJS PEDIATR RES 9 739 975 032 LIEBERMAN J AM REV RESPIR DIS 109 399 974 033 CONOD EJ PEDIATR RES 9 724 975 035 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 036 COHEN LF PEDIATR RES 9 312 975 037 DANES BS J EXP MED 129 775 969 038 DANES BS J EXP MED 136 1313 972 039 CONOVER JH LANCET 1 1194 973 040 BOAT TF AM REV RESPIR DIS 110 428 974 041 BALFE JW SCIENCE 162 689 968 042 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 043 LAPEY A PEDIATR RES 5 446 971 044 COLE CH PEDIATR RES 6 616 972 045 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 046 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 GABRIDGE MG PEDIATR RES 13 31 979 2 AFZELIUS BA INT REV EXP PATHOL 19 1 979 3 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 4 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 5 BERG U ACTA PAEDIATR SCAND 71 833 982 6 ARVIDSSON A ACTA PAEDIATR SCAND 72 293 983 7 RUDICK VL J CELL PHYSIOL 118 67 984 8 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 PN 76137 RN 00491 AN 77034202 TI Screening for cystic fibrosis--further thoughts [editorial]. SO Med-J-Aust. 1976 Aug 7. 2(6). P 194-5. MJ CYSTIC-FIBROSIS: oc. INFANT-NEWBORN-DISEASES: oc. MASS-SCREENING. MN ALBUMINS: an. ENZYME-TESTS. HUMAN. INFANT-NEWBORN. MECONIUM: an. EX Screening of the newborn for genetically determined diseases is one thing when sought by parents who know they are likely to pass a defective gene to their offspring; it is another thing altogether to apply screening tests to all newborns, irrespective of individual risks. In Australia, in the foreseeable future, the best hope for infants and children born with cystic fibrosis lies in the application of the proven sweat test technique whenever any of the numerous presenting features of the disease crops up. RF 001 ROBINSON PG ARCH DIS CHILD 51 301 976 002 ROBINSON PG MED J AUST 1 901 976 PN 76138 RN 00492 AN 76174003 TI Screening newborn infants for cystic fibrosis. SO Med-Lett-Drugs-Ther. 1976 Apr. 18(8). P 35-6. MJ CYSTIC-FIBROSIS: di. MN CYSTIC-FIBROSIS: fg. FALSE-NEGATIVE-REACTIONS. FALSE-POSITIVE-REACTIONS. GENETIC-COUNSELING. HUMAN. INFANT-NEWBORN. MECONIUM: an. PROGNOSIS. EX The Boehringer Mannheim test meconium strip advertised for detection of cystic fibrosis was, like many other diagnostic aids, marketed before its reliability was proven. The test is positive in many infants who do not have cystic fibrosis, and may be negative in those who have the disease. It is therefore unacceptable for routine screening of newborn infants for cystic fibrosis. RF 001 NOLAN AJ CAN MED ASSOC J 114 142 976 002 BRIMBLECOMBE FSW LANCET 2 1428 973 003 ANTONOWICZ I PEDIATRICS 56 782 975 004 STEPHAN U PEDIATRICS 55 35 975 005 ANON J PEDIATR 88 711 976 PN 76139 RN 00493 AN 76149472 AU Rennert-O-M. TI RNA methylation and polyamine metabolism in cystic fibrosis. SO Minerva-Pediatr. 1976 Jan 28. 28(2). P 62-8. MJ CYSTIC-FIBROSIS: me. POLYAMINES: me. RNA: me. MN ANIMAL. CATTLE. DOGS. HETEROZYGOTE. HOMOZYGOTE. HUMAN. METHYLATION. PREGNANCY. RATS. EX The autosomal recessively inherited disorder cystic fibrosis is clinically identified in patients by the pathognomonic finding of increased electrolyte content in the exocrine secretion sweat. Two other observations merit mention. First, the demonstration by Magos of the existence of a factor in CF sweat which interfered with reabsorption of sodium in the rat parotid. Secondly is the existence of a CF serum component which interfered with ciliary rhythm in organ culture systems. Our investigations in cystic fibrosis have been oriented towards the establishment of a possible pedigree of causes which will unite all these observations and secondly to develop a possible method for heterozygote detection. Cystic fibrosis RNA is relatively undermethylated in comparison with contrast cultures. In addition, CF heterozygote fibroblast cultures also appear undermethylated. Our interpretation of these data do not imply a primary defect of RNA methylases in cystic fibrosis but rather suggested a defect elsewhere in biochemical sequences involving methionine. PN 76140 RN 00494 AN 76174070 AU Martinez-J-R. TI Recent advances in research on cystic fibrosis. SO Mo-Med. 1976 Apr. 73(4). P 173-8. MJ CYSTIC-FIBROSIS. MN ANIMAL. CYSTIC-FIBROSIS: et, me. EXOCRINE-GLANDS: se. RATS. RESEARCH. EX Advances have been made in two areas of cystic fibrosis research at the University of Missouri School of Medicine. First, investigations have improved understanding of the normal mechanisms that underline the production of exocrine secretions. The second involves recent observations relating to important efforts to develop methods for heterozygote detection. The author concludes that efforts should be made to shift emphasis in research from qualitative bioassays to more reliable detection systems. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 MARTINEZ JR PEDIATR RES 9 463 975 003 MARTINEZ JR PEDIATR RES 9 470 975 004 IMMKEN L CF CLUB ABST 16 9 975 006 ADSHEAD PC ANN NY ACAD SCI 253 192 975 007 SPOCK A PEDIATR RES 1 173 967 008 CONOVER JH PEDIATR RES 7 224 973 009 DOGGETT RG TEX REP BIOL MED 31 685 973 010 MANGOS JA PEDIATR RES 1 436 967 011 MANGOS JA TEX REP BIOL MED 31 651 973 012 FARRELL PM PEDIATR RES 10 127 976 013 CHEUNG ATW PEDIATR RES 10 144 976 014 RENNERT OM IN: MANGOS JA 41 973 CT 1 WILSON GB MED HYPOTHESES 8 527 982 PN 76141 RN 00495 AN 78071429 AU Buchwald-M. Mapleson-J-L. TI Adenosine 3':5'-cyclic AMP in fibroblasts from patients with cystic fibrosis and its relationship to secretion. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 165-74. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: bi. CYSTIC-FIBROSIS: me. MN ADENOSINE-CYCLIC-MONOPHOSPHATE: an. ADOLESCENCE. ADULT. CELLS-CULTURED. CHILD. FEMALE. FIBROBLASTS: de, me. HUMAN. INFANT. ISOPROTERENOL: pd. MALE. EX Because of the known involvement of cyclic AMP in the control of secretion in exocrine and other mammalian cells, we have studied cyclic AMP metabolism in cultured cystic fibrosis cells in the hopes of demonstrating such an abnormality. A detailed comparison of 5 CF and 5 control strains showed that the CF cells synthesized 3 times more intracellular cyclic AMP than controls. In this paper, we present data on an additional 10 CF and 10 control strains. Measurement of intracellular cyclic AMP did not provide the same clear-cut differences between the CF and control strains, as we had previously observed. The CF strains synthesized more cyclic AMP than controls but the difference was not as large as in our earlier study and there is considerable overlap between the two groups. It is not clear why these two studies should have yielded such disparate results. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 005 BUTCHER FR IN: MANGOS JA 243 976 006 DANES BS LANCET 1 1061 968 007 DANES BS J EXP MED 129 775 969 008 DANES BS NATURE 222 685 969 009 DANES BS J EXP MED 136 1313 972 010 CONOVER JH PEDIATR RES 7 224 973 011 BERATIS NG PEDIATR RES 7 958 973 012 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 013 STURGESS JM BR J EXP PATHOL 54 388 973 014 MARTINEZ JR PEDIATR RES 9 463 975 015 MARTINEZ JR PEDIATR RES 9 470 975 016 RASMUSSEN H SCIENCE 170 404 970 017 BERRIDGE MJ ADV CYCLIC NUCLEO RES 6 1 975 018 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 019 GOLDSTEIN S DIABETES 18 545 969 020 STANNERS CP NATURE NEW BIOL 230 52 971 021 AMSTERDAM A J CELL BIOL 63 1037 974 022 MANGOS JA AM J PHYSIOL 229 553 975 023 MCKUSICK VA ANNU REV GENET 7 435 974 024 LAYMAN DL PROC NAT ACAD SCI USA 68 454 971 025 MATALON R PROC NAT ACAD SCI USA 56 1310 966 026 SEAR CHJ BIOCHEM BIOPHYS RES COMMUN 71 379 976 027 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 028 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 029 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 030 DANES BS BIOCHEM GENET 12 359 974 031 WIESMANN UN J PEDIATR 77 685 970 032 WELCH DW PEDIATR RES 9 698 975 033 PETERS HD POL J PHARMACOL PHARM 26 41 974 034 PALADE GE SCIENCE 189 347 975 035 GOGGINS JF J BIOL CHEM 247 5759 972 036 MANNER G CONN TISS RES 2 167 974 037 KOYAMA H J CELL PHYSIOL 87 189 976 038 LORANS G EUR J BIOCHEM 50 563 975 PN 76142 RN 00496 AN 78071428 AU Lamblin-G. Lafitte-J-J. Lhermitte-M. Degand-P. Roussel-P. TI Mucins from cystic fibrosis sputum. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 153-64. MJ CYSTIC-FIBROSIS: pp. MUCINS: an. SPUTUM: an. MN ADULT. BRONCHIECTASIS: pp. BRONCHITIS: pp. CHILD. CHRONIC-DISEASE. GLYCOPEPTIDES: an. HUMAN. MUCUS: an. EX A biochemical study of bronchial mucins in sputum from patients with cystic fibrosis was undertaken in order to determine their chemical features and to compare them with bronchial mucins secreted in other physiological or pathological conditions. The mucins studied in the present work were obtained from the insoluble and fibrillar part of sputum. If bronchial mucins are the main glycoproteins in the fibrillar mucus, these data suggest that there is a high proportion of acidic mucins within the bronchial secretions from CF patients. The predominance of acidic mucins may not be a specific feature of CF since the sputum from the 2 patients with other diseases had a similar mucin distribution. As far as CF is concerned, it is presently impossible to state whether the bronchial mucins represent normal mucins of the child or structurally abnormal molecules. RF 001 WAREMBOURG H PROC COLLOQUE INT PATHOLOGI 181 968 002 GOERKE J BIOCHIM BIOPHYS ACTA 344 241 974 003 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 004 MASSON PL BIOCHIM BIOPHYS ACTA 111 466 965 005 RYLEY HC BIOCHIM BIOPHYS ACTA 271 300 972 006 HAVEZ R EXP ANNU BIOCHIM MED 32 121 973 007 BROGAN TD BIOCHEM J 71 125 959 008 LAMBLIN G CLIN CHIM ACTA 36 329 972 009 LAMBLIN G BIOCHIM BIOPHYS ACTA 322 372 973 010 ROUSSEL P J BIOL CHEM 250 2114 975 011 POTTER JL ANN NY ACAD SCI 106 692 963 012 ROVIS L BIOCHEMISTRY 12 5340 973 013 BAIG MM J BIOL CHEM 247 6111 972 014 CARLSON DM J BIOL CHEM 243 616 968 015 KABAT EA BIOCHEMISTRY 4 1632 965 016 ROVIS L BIOCHEMISTRY 12 5340 973 017 ROBERTS GP EUR J BIOCHEM 50 265 974 018 LAFITTE JJ C R ACAD SCI (PARIS) 281 1901 975 019 LHERMITTE M BIOCHIMIE 58 367 976 020 BAKER AP BIOCHEM MED 14 42 975 021 LAMB D COLLOQ INT PATHOL THORAC LILL 143 968 022 LEV R AM J PATHOL 46 23 965 023 REID L MOD PROBL PEDIATR 10 195 967 024 DEMAILLE J BULL SOC CHIM FRANCE 1965 3506 965 CT 1 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 PN 76143 RN 00497 AN 78071427 AU Boat-T-F. Cheng-P-W. Wood-R-E. TI Tracheobronchial mucus secretion in vivo and in vitro by epithelial tissues from cystic fibrosis and control subjects. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 141-52. MJ BRONCHI: se. CYSTIC-FIBROSIS: pp. MUCUS: se. TRACHEA: se. MN ADOLESCENCE. ADULT. BRONCHITIS: pp. CHRONIC-DISEASE. EPITHELIUM: se. FEMALE. GLYCOPROTEINS: an. HUMAN. IN-VITRO. ORGAN-CULTURE. SPUTUM: an. EX This paper reviews some of our efforts to compare mucous glycoproteins from the tracheobronchial tract of cystic fibrosis patients with those of control subjects. We have used secretions from several sources, including sputum, bronchial washings obtained with the aid of the fiberoptic bronchoscope, tracheobronchial aspirates through endotracheal or tracheostomy tubes and, finally, secretions discharged by human tracheobronchial epithelial explants. Solubilization and fractionation of secretions; properties of the major mucous glycoprotein components; composition of major components from non-infected, bronchitic and CF lungs; and organ culture of human tracheobronchial epithelium are discussed. RF 001 LITT M BIORHEOLOGY 11 111 974 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 LAMBLIN G CLIN CHIM ACTA 36 329 972 004 BROGAN TD BR J EXP PATHOL 41 288 960 005 HAVEZ R CLIN CHIM ACTA 17 281 967 006 BOAT TF AM REV RESPIR DIS 110 428 974 007 BOAT TF ARCH BIOCHEM BIOPHYS 117 95 976 008 BOAT TF IN: MANGOS JA 165 976 009 SPIRO RG ADV PROT CHEM 27 349 973 010 SPRINGER GF PROC NAT ACAD SCI USA 64 635 969 011 HOORN B ACTA PATH MICROBIOL SCAND S183 966 012 BOAT TF PEDIATR RES 7 607 973 013 BOAT TF CHEST 67 325 975 014 SEGAL MS ASTHMA ALLERGY 2 17 975 015 WARREN L J BIOL CHEM 234 1971 959 016 ANTONOPOULOS CA ACTA CHEM SCAND 16 1521 962 PN 76144 RN 00498 AN 78071426 AU Sturgess-J-M. TI Bronchial mucus secretion in cystic fibrosis. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 129-40. MJ BRONCHI: se. CYSTIC-FIBROSIS: pp. MUCUS: se. MN ANIMAL. BRONCHI: ul. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: bl. HUMAN. IN-VITRO. INFANT-NEWBORN. LUNG-DISEASES: pp. MICROSCOPY-ELECTRON-SCANNING. MUCOUS-MEMBRANE: de, se. ORGAN-CULTURE. RATS. EX Mucus is the primary aetiological factor in the development of pulmonary disease in cystic fibrosis. Biochemical studies on bronchial secretions have not yet confirmed any qualitative difference in composition or in the nature of the glycoproteins, the major component of secretion. With the lack of any clear evidence of a basic abnormality in the nature of mucus, attention has been directed to the possible defect in the control of mucus secretion. This encompasses alteration in the normal regulation of the autonomic nervous system or to its stimulation either at the cellular or intracellular control level. In recent years, attention has focused on the presence of ciliotoxic factors in serum of children with cystic fibrosis, and their possible role in the pathophysiology of cystic fibrosis. Control of mucus secretion, submucosal glands, goblet cells, experimental models of lung disease in cystic fibrosis, the role of serum factors in mucus secretion, and the mucus blanket in chronic lung disease are discussed. RF 001 REID L MOD PROBL PEDIATR 10 195 967 002 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 003 MELLINS RB PEDIATRICS 44 315 969 004 FARBER S ARCH PATHOL 37 238 944 005 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 006 YEATES DB ARCH DIS CHILD 51 28 976 007 SPOCK A PEDIATR RES 1 173 967 008 MEYRICK B THORAX 24 729 969 009 REID L IN: LIEBOW AA 87 968 010 BRANTIGAN OC AM REV RESPIR DIS 80 194 959 011 STURGESS JM BR J EXP PATHOL 54 388 973 012 HAVEZ R CLIN CHIM ACTA 17 281 967 013 STURGESS J EXP MOL PATH 16 362 972 014 STURGESS JM CLIN SCI 43 533 972 015 BOAT TF CHEST 67 325 975 016 DE HALLER R IN: EMERY JL 94 969 017 FLOREY HW BR J EXP PATHOL 13 269 932 018 BOYD EM CAN MED ASSOC J 82 821 960 019 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 020 MANGOS JA PEDIATR RES 1 436 967 021 MARTINEZ JR PEDIATR RES 9 463 975 022 HOLZEL A LANCET 1 822 962 023 DI SANTAGNESE PA N ENGL J MED 295 481 976 024 JOHANSEN PG LANCET 1 455 968 025 FORSTNER JF PEDIATR RES 10 609 976 027 BOWMAN BH SCIENCE 167 871 970 028 CONOVER JH LIFE SCI 14 253 974 029 SCHMOYER IR LIFE SCI PART 2 11 1037 972 030 FORSTNER GG CAN MED ASSOC J 113 550 975 031 RAO GJS PEDIATR RES 9 739 975 032 CONOVER JH LANCET 2 1501 973 033 CZEGLEDY-NAGY E LAB INVEST 35 588 976 035 HERZBERG V J CLIN INVEST 52 2732 973 036 MCFARLANE H BR MED J 1 423 975 037 STURGESS JM AM REV RESPIR DIS 115 819 977 039 EBERT RV AM REV RESPIR DIS 112 491 975 CT 1 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 PN 76145 RN 00499 AN 78071425 AU Lopez-Vidriero-M-T. Reid-L. TI Pathophysiology of mucus secretion in cystic fibrosis. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 120-8. MJ BRONCHI: se. CYSTIC-FIBROSIS: pp. MUCUS: se. MN ADOLESCENCE. ADULT. BRONCHI: pa. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. GLYCOPROTEINS: se. HUMAN. IN-VITRO. INFANT. INFANT-NEWBORN. ORGAN-CULTURE. SPUTUM: an. TRACHEA: se. AB Abnormality of mucus secretion, either biochemical or physical, has long been claimed to be the primary cause of pulmonary complications - obstruction and infection - in patients suffering from cystic fibrosis (CF). This review will draw in large measure on the experience of Prof. L. Reid and her research team who over the last 15 years have studied mucus-secreting structures and their secretions in the normal and diseased lung from various angles: development, structure and ultrastructure, histochemistry organ culture, biochemical analysis and rheological characterization. RF 001 TAPPAN V ANN NY ACAD SCI 106 722 963 002 REID L ADV INTERN MED 12 256 964 003 MEYRICK B THORAX 24 729 969 004 LAMB D J PATHOL 98 213 969 005 JONES R HISTOCHEM J 5 19 973 006 JONES R HISTOCHEM J 5 19 973 007 LAMB D HISTOCHEM J 4 91 972 008 LAMB D BR J DIS CHEST 66 239 972 009 REID L MOD PROBL PEDIATR 10 195 967 010 LAMB D BR J DIS CHEST 66 239 972 011 LEV R AM J PATHOL 46 23 965 012 STURGESS J EXP MOL PATH 16 362 972 013 MEYRICK B J CELL BIOL 67 320 975 014 REID L IN: BALLS M 463 976 015 STURGESS JM CLIN SCI 43 533 972 017 KEAL EE THESIS 970 018 MEARNS MB ARCH DIS CHILD 43 528 968 019 LOPEZ-VIDRIERO MT THESIS 976 020 STURGESS J RHEOL ACTA 10 36 971 021 CHARMAN J BIORHEOLOGY 9 185 972 022 MITCHELL-HEGGS PF BIORHEOLOGY 11 417 974 CT 1 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 PN 76146 RN 00500 AN 78071422 AU Carlson-D-M. TI Mucous glycoproteins. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 1-10. MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: an. MUCUS: an. MN AMINO-ACIDS: an. ANIMAL. GLYCOPROTEINS: bi. HUMAN. MAMMALS. OLIGOSACCHARIDES: an, bi. SWINE. EX While there is widespread acceptance that mucous glycoprotein production is correlated with the pathogenesis of cystic fibrosis, as yet relatively little is known about the chemistry and control of synthesis and secretion of these heteromacromolecules. This presentation describes briefly the chemistry and biosynthesis of glycoproteins in general, but is focused on the mucous glycoproteins. The chemistry of glycoproteins, carbohydrate to amino acid linkages, carbohydrate structural analysis, biosynthesis, and regulation are discussed. RF 001 JAKOWSKA S CF AND RELATED HUMAN AND ANIM 970 002 ROSEMAN S CHEM PHYS LIPIDS 5 270 970 003 SCHACHTER H IN: HOROWITZ MI 975 004 JENTOFT N IN: MARTONOSI A 2 343 976 005 KORNFELD R ANNU REV BIOCHEM 45 217 976 006 NUENKE RH J BIOL CHEM 236 2452 961 007 JOHANSEN PG BIOCHEM J 78 518 961 008 SPIRO RG ADV PROT CHEM 27 349 973 009 ANDERSON B ARCH BIOCHEM BIOPHYS 148 304 972 010 SPIRO RG J BIOL CHEM 249 5704 974 011$ BAHL OP J BIOL CHEM 244 575 972 012 ARIMA T J BIOL CHEM 247 1825 972 013 THOMAS DB J BIOL CHEM 244 5943 969 014 CLAMP JR IN: GOTTSCHALK A 612 972 015 CARLSON DM J BIOL CHEM 243 616 968 016 IYER RN ARCH BIOCHEM BIOPHYS 142 101 971 017 LEHMAN D FED PROC FED AM SOC EXP BIOL 33 1497 974 018 CHOI HV J BIOL CHEM 249 932 974 019 CARLSON DM ANAL BIOCHEM 20 195 967 020 BELLA A JR J CHROMATOGR 51 314 970 021 BHATTI T BIOCHIM BIOPHYS ACTA 222 339 970 022 BAIG MM J BIOL CHEM 247 6111 972 023 SWELLEY CC J AM CHEM SOC 85 2497 963 024 HAKOMORI S J BIOCHEM (TOKYO) 55 205 964 025 LINDBERG B METHODS ENZYMOL 28 178 972 026 CARLSON DM IN: ROSSI E 304 968 027 CARLSON DM J BIOL CHEM 248 5742 973 028 MCGUIRE EJ IN: AMINOFF D 461 970 029 MONTGOMERY R IN: GOTTSCHALK A 518 972 030 SCHACHTER H J BIOL CHEM 246 5321 971 031 ROVIS L BIOCHEMISTRY 12 5340 973 032 GINSBURG V ADV ENZYMOL 26 35 964 033 RUTTER WJ J CELL PHYSIOL SUPPL 72 1 968 034 PICTET RL HANDBOOK OF PHYSIOLOGY SECT 7 1 25 972 035 CARLSON DM ARCH BIOCHEM BIOPHYS 152 605 973 036 RONZIO RA ARCH BIOCHEM BIOPHYS 159 777 973 037 TURKINGTON RW J BIOL CHEM 246 1814 971 PN 76147 RN 00501 AN 78071432 AU Newhouse-M-T. Rossman-C-M. Dolovich-J. Dolovich-M-B. Wilson-W-M. TI Impairment of mucociliary transport in cystic fibrosis. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 190-8. MJ CILIA: ph. CYSTIC-FIBROSIS: pp. MUCUS: ph. MN HUMAN. IGG. IN-VITRO. INFLAMMATION: pp. NASAL-MUCOSA: pp. RESPIRATORY-SYSTEM: pp. EX Over the past few years, studies from various laboratories have suggested the presence of a factor(s) in cystic fibrosis serum causing widespread in vitro abnormalities of components of the lung defence mechanism particularly with respect to macrophage dysfunction, quantitative and qualitative alterations in mucus, and ciliary motility. The possible in vivo role of ciliary dysfunction in the pathogenesis of the disease is of particular clinical interest. Since an in vitro ciliary dyskinesia factor had been previously demonstrated and pulmonary complications are the hallmark of CF, yet clearance in most of our clinically stable CF subjects was relatively well maintained, this suggested to us that a mucociliary transport abnormality might be present intermittently. In order to explore this further, we designed nasal mucociliary clearance studies to determine whether CF serum might exhibit MCT inhibition in vivo. From these studies, we concluded that there was a substance(s) in CF serum which had the capacity to slow MCT. Whether this effect is on the cilia themselves or via overproduction of normal or abnormal mucus is not known. RF 001 BOXERBAUM B AM REV RESPIR DIS 108 777 973 002 YEATES DB ARCH DIS CHILD 51 28 976 003 GIBSON LE LANCET 2 189 970 004 SPOCK A PEDIATR RES 1 173 967 005 BOWMAN BH SCIENCE 164 325 969 006 BESLEY GTN J MED GENET 6 278 969 007 WOOD RE LANCET 2 1452 973 008 CHRISTENSEN J IN: LAWSON D PROC 5TH INT CF 49 969 009 CHERRY JD J PEDIATR 79 937 971 010 SANCHIS J N ENGL J MED 288 651 973 011 WOOD RE AM REV RESPIR DIS 111 733 975 012 NEWHOUSE M IN: MANGOS JA 319 973 013 SANCHIS J J APPL PHYSIOL 33 757 972 014 SANCHIS J BULL PHYSIOPATH RESPIR NANCY 9 325 973 015 ROSSMAN CM J PEDIATR 90 579 977 016 PROCTOR DF IN: DAVIES CN 2 25 967 017 BOWMAN BH SCIENCE 167 871 970 018 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 019 DANES BS J EXP MED 137 1538 973 020 ISHIZAKA K J IMMUNOL 100 554 968 021 WANNER A CHEST 61 287 972 022 KARTAGENER M BEITR KLIN TUBERK 83 489 933 023 CAMNER P AM REV RESPIR DIS 112 807 975 024 AFZELIUS BA SCIENCE 193 317 976 PN 76148 RN 00502 AN 78071433 AU Aspin-N. Wong-J-W. Yeates-D-B. Levison-H. TI Mucociliary clearance in cystic fibrosis. SO Mod-Probl-Paediatr. 1976 Oct 24-27. 19. P 199-206. MJ CILIA: ph. CYSTIC-FIBROSIS: pp. MUCUS: ph. MN ADULT. AEROSOLS. ANIMAL. HUMAN. POSTURE. RABBITS. RADIOACTIVE-TRACERS. RESPIRATORY-SYSTEM: pp. SCINTILLATION-COUNTING. TRACHEA: pp. EX If we are to fully understand the factors which control mucociliary clearance throughout the human lung and the interrelationships between clearance and disease, it will be necessary to measure mucociliary clearance rates in airways at all levels within the lung. During the past few years, technical means have been sought whereby the rates of mucus clearance within individual airways can be measured. In this laboratory, we have developed a relatively noninvasive technique for measuring mucociliary tracheal transport rates. We measured MTTRs in 20 patients with cystic fibrosis. The MTTRs did not correlate well with the degree of pulmonary disease in these patients. The measurements of MTTR would suggest that a deranged mucociliary clearance mechanism is not a necessary condition for patients with CF. We have also been interested in evaluating the effectiveness of the therapy used in the treatment of CF. RF 001 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 002 SADE J AM REV RESPIR DIS 102 48 970 003 ALBERT RE ARCH INTERN MED 131 115 973 004 PROCTOR DF AM REV RESPIR DIS 115 97 977 005 SACKNER MA J APPL PHYSIOL 34 495 973 006 WOOD RE AM REV RESPIR DIS 111 733 975 007 FRIEDMAN M AM REV RESPIR DIS 115 67 977 008 YEATES DB J APPL PHYSIOL 39 487 975 009 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 PASSERO MA CLIN PEDIATR 20 264 981 PN 76149 RN 00503 AN 76242808 AU Di-SantAgnese-P-A. Davis-P-B. TI Research in cystic fibrosis (first of three parts). SO N-Engl-J-Med. 1976 Aug 26. 295(9). P 481-5. (REVIEW). MJ CYSTIC-FIBROSIS. MN CAUCASOID-RACE. CYSTIC-FIBROSIS: fg, me. EXOCRINE-GLANDS: se. GENES-RECESSIVE. GLYCOPROTEINS: me, ur. GLYCOSAMINOGLYCANS: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. RESEARCH. REVIEW. SUPPORT-U-S-GOVT-P-H-S. VISCOSITY. EX This review describes the major directions in cystic fibrosis research in the last nine years. There have been no further reports of cystic fibrosis in native African Negroes. It has been generally accepted that cystic fibrosis follows simple autosomal recessive transmission. It is difficult, however, to explain maintenance of a lethal gene at such high frequency. Nothing is known of the chromosomal localization of the trait. At present there is no simple and reliable method of heterozygote detection. The secretions of patients with cystic fibrosis are often not copious but exhibit a peculiar rheologic property. Several groups have emphasized decreased water content of cystic fibrosis secretions in the pathogenesis of the disease. There is no consistent abnormality of glycoproteins or glycosaminoglycans to explain why mucus secretions precipitate and obstruct the organ passages. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 LOBECK CC IN: STANBURY JB 1605 972 005 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 006 SHWACHMAN H IN: KENDIG EL JR 1 524 972 007 DI SANTAGNESE PA IN: DOWNEY JA 25 974 008 WOOD RE AM REV RESPIR DIS 113 833 976 009 ANTONELLI M FRACASTORO (VERONA) 63 207 970 010 KULCZYCKI LL AM J DIS CHILD 127 64 974 011 WRIGHT SW AM J HUM GENET 20 157 968 012 CONNEALLY PM TEX REP BIOL MED 31 639 973 013 HIRSCHHORN K IN: MANGOS JA 275 976 014 LAPEY A J PEDIATR 84 328 974 015 DANES BS J EXP MED 129 775 969 016 DANES BS J EXP MED 136 1313 972 017 POLYMENIDIS Z LANCET 2 1452 973 018 SCHWARTZ RH PEDIATR RES 9 299 975 019 BOCHKOVA DN GENETICS 11 154 975 020 ANON J PEDIATR 88 711 976 021 BETTELHEIM FA BIORHEOLOGY 8 129 971 022 LORIN MI BIORHEOLOGY 9 27 972 023 LIEBERMAN J AM REV RESPIR DIS 97 654 968 024 KHAN MA TEX REP BIOL MED 31 665 973 025 HADORN B CAN MED ASSOC J 98 377 968 026 KOPITO LE FERTIL STERIL 24 512 973 027 JOHANSEN PG LANCET 1 455 968 028 GIBSON LE PEDIATRICS 48 695 971 029 WOTMAN S ARCH ORAL BIOL 16 663 971 030 WIESMANN UN LANCET 2 510 972 031 FORSTNER JF PEDIATR RES 10 609 976 032 BOAT TF PEDIATR RES 8 531 974 033 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 034 BOAT TF AM REV RESPIR DIS 110 428 974 035 REID L MOD PROBL PEDIATR 10 195 967 036 ROUSSEL P J BIOL CHEM 250 2114 975 037 BROGAN TD THORAX 30 72 975 038 CLEAVE AJ BIOCHIM BIOPHYS ACTA 285 208 972 039 STEVENSON FK CLIN CHIM ACTA 23 441 969 040 CROSBY P HISTOCHEM J 3 223 971 041 BAIG MM J PEDIATR 86 72 975 042 CHANGUS JE AM J PATHOL 80 317 975 043 LOUISOT P CLIN CHIM ACTA 48 373 973 044 BAKER AP BIOCHEM MED 10 387 974 045 LUNDGREN DW CLIN CHIM ACTA 62 357 975 046 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 047 SINGER L CLIN BIOCHEM 7 146 974 048 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 049 MENGUY R GASTROENTEROLOGY 59 257 970 050 BOAT TF IN: MANGOS JA 165 976 051 WILSON RG CLIN CHIM ACTA 36 189 972 052 LANGGARD H ACTA PAEDIATR SCAND 57 255 968 053 CONSTANTOPOULOS G J PEDIATR 78 806 971 CT 1 POLONOVSKI C PEDIATRE 12 379 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 ANON BR MED J 1 596 977 4 SUPER M LANCET 2 1288 977 5 HUNT LA J SUPRAMOL STRUCT 7 213 977 6 THOMAS JM PEDIATR RES 11 1148 977 7 NEUTRA MR LAB INVEST 36 535 977 8 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 9 SCANLIN TF BIOCHEM BIOPHYS RES COMMUN 79 869 977 10 BRAY PT CLIN CHIM ACTA 80 333 977 11 HERROD HG J PEDIATR 91 276 977 12 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 13 DOERING KM EUR J PEDIATR 126 185 977 14 HODSON ME BR MED J 1 971 978 15 ANON LANCET 2 1032 978 16 FIGARELLA C GASTROENTEROL CLIN BIOL 2 343 978 17 OWEN E J MOL MED 3 203 978 18 DAVIS PB PEDIATR RES 12 703 978 19 IMPERO JE PEDIATR RES 12 108 978 20 BOIS E CLIN GENET 14 73 978 21 SCHAAP T ISR J MED SCI 14 201 978 22 PEARSON RD HEALTH LAB SCI 15 32 978 23 MARKOWITZ SM INFECT IMMUN 22 530 978 24 RAO GJS ENZYME 23 314 978 25 PURKISS P CLIN CHEM 24 714 978 26 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 27 HARRIES JT BR MED BULL 34 75 978 28 HOSLI P HUM GENET 41 169 978 29 KOLLBERG H SCAND J RESPIR DIS 59 297 978 30 NEUTRA MR GASTROENTEROLOGY 75 701 978 31 WILSON GB J LAB CLIN MED 92 463 978 32 VAUGHAN WJ SCIENCE 199 783 978 33 BRESLOW JL SCIENCE 201 180 978 34 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 35 HOSLI P LANCET 2 543 979 36 SHIER WT MED HYPOTHESES 5 661 979 37 SEALE TW J CLIN MICROBIOL 9 72 979 38 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 39 FEIGAL RJ PEDIATR RES 13 764 979 40 GABRIDGE MG PEDIATR RES 13 31 979 41 PEARSON RD PEDIATR RES 13 834 979 42 PIVETTA OH PEDIATR RES 13 1160 979 43 RUSSELL DH PEDIATR RES 13 1137 979 44 WILL PC PEDIATR RES 13 1129 979 45 LAM LFH LIFE SCI 24 2483 979 46 SORDELLI DO LIFE SCI 24 2003 979 47 COX DW PEDIATR CLIN NORTH AM 26 467 979 48 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 49 TUCKER RD IEEE TRANS BIOMED ENG 26 607 979 50 BROOKS FP MAYO CLIN PROC 54 475 979 51 SANTAGNESE PAD AM J MED 66 121 979 52 KOLLBERG H ACTA PAEDIATR SCAND 68 639 979 53 WOOD RE SOUTH MED J 72 189 979 54 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 55 SCANLIN TF CLIN CHIM ACTA 91 197 979 56 CHASE HP J PEDIATR 95 337 979 57 JAKEL HP BIOL ZENTRALBL 98 55 979 58 HOSLI P FEBS LETTERS 104 271 979 59 LEPRAT R ANN MICROBIOL (PARIS) B131 209 980 60 HOLSCLAW DS CLIN CHEST MED 1 407 980 61 SCANLIN TF CLIN CHEST MED 1 424 980 62 IANNACCONE G PEDIATR RADIOL 9 85 980 63 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 64 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 65 DAVIS PB PEDIATR RES 14 83 980 66 GILLARD BK PEDIATR RES 14 1168 980 67 WILL PC PEDIATR RES 14 1245 980 68 LASZLO A ACTA PAEDIATR ACAD SCI HUNG 21 69 980 69 CHASE HP METABOLISM 29 365 980 70 PRIMOSCH RE ORAL SURG 50 301 980 71 GLORIABOTTINI F HUM GENET 54 79 980 72 LLOYDSTILL JD PEDIATRICS 65 1010 980 73 WILSON GB J CLIN INVEST 66 1010 980 74 ADRIAN TE GASTROENTEROLOGY 79 460 980 75 DAVIS PB J LAB CLIN MED 96 75 980 76 DUFFY MJ CLIN CHIM ACTA 103 233 980 77 FODER B CLIN CHIM ACTA 104 187 980 78 MALER T FEBS LETTERS 121 153 980 79 WILLI UV AM J ROENTGENOL 134 1005 980 80 BALTIMORE RS J INFECT DIS 141 238 980 81 ROSENSTEIN BJ JOHNS HOPKINS MED J 147 188 980 82 TABACHNIK NF J PHARMACOL EXP THER 214 246 980 83 DAVIS PB N ENGL J MED 302 1453 980 84 MATTHEWS WJ N ENGL J MED 302 245 980 85 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 86 FICK RB CLIN CHEST MED 2 91 981 87 REYNOLDS HY CLIN CHEST MED 2 103 981 88 STEGMAYR B ULTRASTRUCTURAL PATHOL 2 357 981 89 ANTONELLI M RIV ITAL PEDIATR 7 769 981 90 BANCHINI G PEDIATR RES 15 1073 981 91 BENYOSEPH Y PEDIATR RES 15 839 981 92 IMPERO JE PEDIATR RES 15 940 981 93 SANDERSON MJ PEDIATR RES 15 219 981 94 SORENSEN RU PEDIATR RES 15 14 981 95 RUDIN DO BIOL PSYCHIAT 16 373 981 96 PIVETTA OH LIFE SCI 28 2207 981 97 CAVALIER SJ NEUROLOGY 31 714 981 98 FERNANDES PB INFECT IMMUN 33 527 981 99 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 100 RUTLAND J THORAX 36 654 981 101 ANON NUTR REV 39 14 981 102 CONGDON PJ POSTGRAD MED J 57 453 981 103 FICK RB J CLIN INVEST 68 899 981 104 WILSON GB J CLIN INVEST 68 171 981 105 TEGNER H ACTA PAEDIATR SCAND 70 629 981 106 REZNIK VM PROC NAT ACAD SCI USA 78 7143 981 107 MARKS MI J PEDIATR 98 173 981 108 LLOYDSTILL JD J PEDIATR 99 580 981 109 JAKEL HP BIOL ZENTRALBL 100 273 981 110 LEMANSKE RF AM REV RESPIR DIS 123 622 981 111 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 112 ROSCHER AA J PHARMACOL EXP THER 216 419 981 113 BYLUND DB J PHARMACOL EXP THER 218 134 981 114 MALER T J BIOL CHEM 256 1420 981 115 BRESLOW JL N ENGL J MED 304 1 981 116 SORSCHER EJ LANCET 1 368 982 117 STERN RC LANCET 1 1401 982 118 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 119 PALING MR SKELETAL RADIOL 8 63 982 120 AONO M IRCS MED SCI BIOCHEM 10 159 982 121 TABAK LA J ORAL PATHOL 11 1 982 122 FIGARELLA C EUR J CLIN INVEST 12 145 982 123 MORIARTY CM J MED 13 257 982 124 BLITZER MG PEDIATR RES 16 203 982 125 BOGART BI PEDIATR RES 16 223 982 126 PATTON CJ PEDIATR RES 16 1035 982 127 MITCHELL EA AUST PAEDIATR J 18 40 982 128 MATSON JA J REPROD MED 27 373 982 129 SHAPIRO BL AM J HUM GENET 34 846 982 130 OHMAN DE INFECT IMMUN 37 662 982 131 SLOMIANY BL J DENT RES 61 1163 982 132 KOLLBERG H EUR J RESPIR DIS 63 101 982 133 MOSS AJ PEDIATRICS 70 728 982 134 KOLLBERG H ACTA PAEDIATR SCAND 71 197 982 135 HUBBARD VS J AM DIET ASSOC 80 127 982 136 KEMP T ARCH INT PHYSIOL BIOCHIM 90 B123 982 137 SNYDER CE CARBOHYD RES 105 87 982 138 STURGESS J AM J PATHOL 106 303 982 139 TSCHOLAKOFF D FORTSCHR GEB RONTG NUKL 137 18 982 140 KRAEMER R EUR J PEDIATR 138 172 982 141 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 142 KATZ SM JAMA 248 2284 982 143 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 144 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 145 SHAWKER TH J ULTRASOUND MED 2 439 983 146 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 147 NEU HC J ANTIMICROB CHEMOTHER 11 1 983 148 CERQUETTI MC IMMUNOL COMMUN 12 375 983 149 HARD R TISSUE CELL 15 217 983 150 CARMAGNOL F PEDIATR RES 17 181 983 151 FRATES RC PEDIATR RES 17 30 983 152 MARMON L J PEDIATR SURG 18 811 983 153 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 154 CEDER O CLIN GENET 23 298 983 155 DAVIS PB J CHRON DIS 36 269 983 156 NEWHOUSE MT EUR J RESPIR DIS 64 151 983 157 PEDERSEN M EUR J RESPIR DIS 64 118 983 158 NEVE J ACTA PAEDIATR SCAND 72 437 983 159 ALLEN JM GASTROENTEROLOGY 85 1379 983 160 RUDICK VL J CELL PHYSIOL 115 143 983 161 BARDON A CLIN CHIM ACTA 133 311 983 162 ROSENBERG E KLIN PAEDIATR 195 323 983 163 SLOMIANY A J BIOL CHEM 258 8535 983 164 MINER C J PHYSIOL (LOND) 338 P 63 983 165 SHAPIRA E ANN NY ACAD SCI 421 352 983 166 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 167 SEYMOUR CA BIOESSAYS 1 38 984 168 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 169 VLADUTIU GD IRCS MED SCI BIOCHEM 12 515 984 170 BERGHOUT AGRV PEDIATR RES 18 1017 984 171 BOYD RL PEDIATR RES 18 1028 984 172 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 173 WILSON GB CLIN GENET 26 331 984 174 MCCLURE HM ADV VET SCI COMP MED 28 267 984 175 TAWARA K RES COMMUN CHEM PATH PHARM 43 515 984 176 ALBAZZAZ FJ RESPIRATION 46 88 984 177 HODSON ME POSTGRAD MED J 60 225 984 178 BARDON A ACTA PAEDIATR SCAND 73 263 984 179 SATO K J CLIN INVEST 73 1763 984 180 MCNEILL WF AM J CLIN PATHOL 81 742 984 181 DAVIS PB CHEST 85 802 984 182 WAUTERS J ARCH INT PHYSIOL BIOCHIM 92 B115 984 183 RUDICK VL J CELL PHYSIOL 118 67 984 184 WOOTEN MW J CELL PHYSIOL 121 490 984 185 GILLARD BK AM J DIS CHILD 138 577 984 186 RICE DH WEST J MED 140 238 984 187 HANCOCK REW J INFECT DIS 149 220 984 188 BRADY RC EXP CELL RES 150 141 984 189 GOLDBERG JB J BACTERIOL 158 1115 984 190 SATO K AM J PHYSIOL 247 R646 984 191 SWOBODNIK W J CLIN ULTRASOUND 13 469 985 192 CASSANO WF MED HYPOTHESES 18 51 985 193 WOOTEN MW IN VITRO CELL DEVEL BIOL 21 207 985 194 REYNOLDS HY DISEASE A MONTH 31 1 985 195 DUNNE WM MICROBIOS 43 193 985 196 JACQUOT J INFECT IMMUN 47 555 985 197 DUNNE WM APPL ENVIRON MICROBIOL 50 562 985 198 TABLAN OC J PEDIATR 107 382 985 199 HILL DJS MED J AUST 143 230 985 200 FICK RB J INFECT DIS 151 589 985 201 BRADY RC AM J PHYSIOL 248 G 54 985 202 COLE DEC J CHROMATOGR 337 267 985 203 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 204 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 205 BRIDGES MA PEDIATR RES 20 356 986 206 REGELMANN WE PEDIATR RES 20 619 986 207 RUPP GM MED HYPOTHESES 20 245 986 208 PORTER WH CLIN CHEM 32 652 986 209 SULLIVAN MM CHEST 90 239 986 210 HUNSINGER RN CLIN CHIM ACTA 156 165 986 211 BATTINO M FEBS LETTERS 199 155 986 212 DASGUPTA MK J CLIN IMMUNOL 7 51 987 213 DAVIS PB J DENT RES 66 667 987 214 TANDLER B J DENT RES 66 398 987 215 GEDSCHOLD J HUM GENET 75 277 987 PN 76150 RN 00504 AN 76242767 AU di-SantAgnese-P-A. Davis-P-B. TI Research in cystic fibrosis (second of three parts). SO N-Engl-J-Med. 1976 Sep 2. 295(10). P 534-41. (REVIEW). MJ CYSTIC-FIBROSIS: me. MN ANIMAL. BIOLOGICAL-TRANSPORT. CELL-MEMBRANE: me. CHLORIDES: me. CILIA: ph. COMPLEMENT-3. CYSTIC-FIBROSIS: bl, pa. FEMALE. GLYCOSAMINOGLYCANS: me. HUMAN. IGG: me. LYSOSOMES: en. MALE. METHYLATION. POLYAMINES: bl. PROTEIN-BINDING. REVIEW. RNA: me. SALIVA: an. SODIUM: me. SUBCELLULAR-FRACTIONS: en. SWEAT-GLANDS: me. TISSUE-CULTURE. TOXINS: ip. EX Tissue-culture studies involving metachromasia, glycosaminoglycans, RNA methylation, cell membranes, and ciliatoxic activity in medium are described. Although not fulfilling the hopes of a quick route to discovery of the basic defect in this disease that were raised by the first findings, the study of fibroblasts in cystic fibrosis has given important information. The evidence at present indicates that lysosomes and probably other intracellular organelles are normal in morphology and function in cystic fibrosis. Several humoral abnormalities have been found in cystic fibrosis: those that influence ciliary activity in experimental models; those that affect membrane transport; and the abnormality in polyamine distribution. A number of abnormal humoral factors have been found in cystic fibrosis. The limitations in their interpretation are serious: the assays used to delineate these abnormalities are often not universally reproducible, some because of their inherent variability and others because they are technically forbidding. Therefore, unified definition of the so-called "cystic fibrosis factors" is not possible at present. RF 054 DANES BS LANCET 1 1061 968 055 BEARN AG N ENGL J MED 282 102 970 056 TAYSI K N ENGL J MED 281 1108 969 057 KRAUS I PEDIATRICS 47 1010 971 058 MILUNSKY A N ENGL J MED 281 1128 969 059 ANON SUBCOMMITTEE ON METACHROMASIA 972 060 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 061 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 062 WIESMANN UN J PEDIATR 77 685 970 063 WELCH DW PEDIATR RES 9 698 975 064 RENNERT OM IN: MANGOS JA 41 973 065 KLAGSBRUN M IN: MANGOS JA 53 973 066 KLAGSBRUN M PEDIATR RES 8 205 974 067 HODES ME PEDIATR RES 8 212 974 068 FLETCHER DS CLIN CHIM ACTA 44 5 973 069 QUISSELL DO NATURE 247 115 974 070 BENKE PJ LANCET 1 182 972 071 CHOU L PEDIATR RES 10 176 976 072 DAVIS PB PEDIATR RES 10 364 976 073 BERATIS NG PEDIATR RES 7 958 973 074 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 075 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 076 SIMOPOULOS AP PEDIATR RES 6 355 972 077 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 078 BOLTON WE AM J HUM GENET 27 394 975 079 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 080 PALLAVICINI JC J PEDIATR 77 280 970 081 STEELE MW INT RES COMMUN SYST 2 1614 974 082 SPICER SS CF CLUB ABST 976 083 FARRELL PM ACTA PAEDIATR SCAND 64 150 975 084 BARTMAN J J PEDIATR 76 430 970 085 ANTONOWICZ I PEDIATR RES 6 803 972 086 BUTTERWORTH J CLIN CHIM ACTA 41 367 972 087 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 088 WILSON RG CLIN CHIM ACTA 36 113 972 089 MARCHI AG HELV PAEDIATR ACTA 28 427 973 090 GIBBS GE SCIENCE 167 993 970 091 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 092 GIBBS GE J INVEST DERMATOL 51 200 968 093 EVANS BW AM J DIS CHILD 127 660 974 094 BENKE PJ N ENGL J MED 284 731 971 095 FILLIAT M PATHOL BIOL (PARIS) 21 13 973 096 RUSSELL SB J MED GENET 8 441 971 097 SLY WS J PEDIATR 82 249 973 098 SHAPIRO BL LANCET 2 1020 974 099 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 100 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 101 SPOCK A PEDIATR RES 1 173 967 102 BOWMAN BH SCIENCE 164 325 969 103 BESLEY GTN J MED GENET 6 278 969 104 COHEN FL J MED GENET 11 253 974 105 CHERRY JD J PEDIATR 79 937 971 106 PUSSELT H Z KINDERHEILK 100 93 971 107 CHRISTENSEN J IN: LAWSON D PROC 5TH INT CF 49 969 108 CONOVER JH LANCET 1 1194 973 109 SCHMOYER IR LIFE SCI PART 2 11 1037 972 110 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 111 BARNETT DR TEX REP BIOL MED 31 709 973 112 DANES BS J EXP MED 137 1538 973 113$ BERATIS NG PEDIATR RES 8 687 974 114 HERZBERG V J CLIN INVEST 52 2732 973 115 POLLEY MJ J MED GENET 11 249 974 116 CONOVER JH LANCET 2 1501 973 117 RAO GJS PEDIATR RES 8 684 974 118 LIEBERMAN J AM REV RESPIR DIS 111 100 975 119 LEDERBERG S IN: MANGOS JA 259 976 120 WOOD RE LANCET 2 1452 973 121 FARRELL PM PEDIATR RES 10 127 976 122 CONOD EJ PEDIATR RES 9 724 975 123 WILSON GB PEDIATR RES 9 635 975 124 MANGOS JA PEDIATR RES 1 436 967 125 MANGOS JA PEDIATR RES 2 378 968 126 KAISER D PEDIATR RES 5 167 971 127 TAYLOR A PEDIATR RES 8 861 974 128 MANGOS JA TEX REP BIOL MED 31 651 973 129 TAUSSIG LM LANCET 1 1367 972 130 SCHULZ IJ J CLIN INVEST 48 1470 969 131 MANGOS JA IN: MANGOS JA 311 976 132 ARAKI H PEDIATR RES 9 932 975 133 MORIN CL BIOMEDICINE EXPRESS 19 133 973 134 BROWN GA LANCET 2 639 971 135 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 136 OKA T J BIOL CHEM 249 7647 974 137 LUNDGREN DW PROC SOC EXP BIOL MED 152 81 976 138 RUSSELL DH CANCER RES 31 1555 971 139 COHEN LF PEDIATR RES 9 312 975 140 MCEVOY FA PEDIATR RES 9 721 975 141 MUNRO GF CF CLUB ABST 16 7 975 142 PETER WH HOPPE SEYLERS Z PHYSIOL CHEM 354 1146 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 THOMAS JM PEDIATR RES 11 1148 977 3 WILSON GB PEDIATR RES 11 986 977 4 WILSON GB PEDIATR RES 11 317 977 5 NEUTRA MR LAB INVEST 36 535 977 6 SCANLIN TF BIOCHEM BIOPHYS RES COMMUN 79 869 977 7 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 8 DOERING KM EUR J PEDIATR 126 185 977 9 WILSON GB NATURE 266 463 977 10 HODSON ME BR MED J 1 971 978 11 FIGARELLA C GASTROENTEROL CLIN BIOL 2 343 978 12 OWEN E J MOL MED 3 203 978 13 DAVIS PB PEDIATR RES 12 703 978 14 IMPERO JE PEDIATR RES 12 108 978 15 PEARSON RD HEALTH LAB SCI 15 32 978 16 MARKOWITZ SM INFECT IMMUN 22 530 978 17 RAO GJS ENZYME 23 314 978 18 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 19 NEUTRA MR GASTROENTEROLOGY 75 701 978 20 WIEDERHOLD ML BRAIN RES 156 369 978 21 BRESLOW JL SCIENCE 201 180 978 22 HOSLI P LANCET 2 543 979 23 SEALE TW J CLIN MICROBIOL 9 72 979 24 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 25 GABRIDGE MG PEDIATR RES 13 31 979 26 THOMASSEN MJ PEDIATR RES 13 1085 979 27 LAM LFH LIFE SCI 24 2483 979 28 SORDELLI DO LIFE SCI 24 2003 979 29 COX DW PEDIATR CLIN NORTH AM 26 467 979 30 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 31 BROOKS FP MAYO CLIN PROC 54 475 979 32 SANTAGNESE PAD AM J MED 66 121 979 33 KOLLBERG H ACTA PAEDIATR SCAND 68 639 979 34 WOOD RE SOUTH MED J 72 189 979 35 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 36 SCANLIN TF CLIN CHIM ACTA 91 197 979 37 CHASE HP J PEDIATR 95 337 979 38 JAKEL HP BIOL ZENTRALBL 98 55 979 39 HOSLI P FEBS LETTERS 104 271 979 40 LEPRAT R ANN MICROBIOL (PARIS) B131 209 980 41 HOLSCLAW DS CLIN CHEST MED 1 407 980 42 SCANLIN TF CLIN CHEST MED 1 424 980 43 IANNACCONE G PEDIATR RADIOL 9 85 980 44 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 45 DAVIS PB PEDIATR RES 14 83 980 46 DAVIS PB PEDIATR RES 14 863 980 47 GILLARD BK PEDIATR RES 14 1168 980 48 WILL PC PEDIATR RES 14 1245 980 49 PRIMOSCH RE ORAL SURG 50 301 980 50 GLORIABOTTINI F HUM GENET 54 79 980 51 WILSON GB J CLIN INVEST 66 1010 980 52 DAVIS PB J LAB CLIN MED 96 75 980 53 ROSENSTEIN BJ JOHNS HOPKINS MED J 147 188 980 54 DAVIS PB N ENGL J MED 302 1453 980 55 MATTHEWS WJ N ENGL J MED 302 245 980 56 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 57 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 58 FICK RB CLIN CHEST MED 2 91 981 59 REYNOLDS HY CLIN CHEST MED 2 103 981 60 STEGMAYR B ULTRASTRUCTURAL PATHOL 2 357 981 61 ANTONELLI M RIV ITAL PEDIATR 7 769 981 62 BANCHINI G PEDIATR RES 15 1073 981 63 IMPERO JE PEDIATR RES 15 940 981 64 SANDERSON MJ PEDIATR RES 15 219 981 65 FERNANDES PB INFECT IMMUN 33 527 981 66 ANON NUTR REV 39 14 981 67 FICK RB J CLIN INVEST 68 899 981 68 WILSON GB J CLIN INVEST 68 171 981 69 TEGNER H ACTA PAEDIATR SCAND 70 629 981 70 LIEBERMAN J J LAB CLIN MED 97 646 981 71 MARKS MI J PEDIATR 98 173 981 72 LLOYDSTILL JD J PEDIATR 99 580 981 73 JAKEL HP BIOL ZENTRALBL 100 273 981 74 LEMANSKE RF AM REV RESPIR DIS 123 622 981 75 ROSCHER AA J PHARMACOL EXP THER 216 419 981 76 BYLUND DB J PHARMACOL EXP THER 218 134 981 77 BRESLOW JL N ENGL J MED 304 1 981 78 SORSCHER EJ LANCET 1 368 982 79 STERN RC LANCET 1 1401 982 80 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 81 TABAK LA J ORAL PATHOL 11 1 982 82 BLITZER MG PEDIATR RES 16 203 982 83 PATTON CJ PEDIATR RES 16 1035 982 84 MITCHELL EA AUST PAEDIATR J 18 40 982 85 SHAPIRO BL AM J HUM GENET 34 846 982 86 OHMAN DE INFECT IMMUN 37 662 982 87 SLOMIANY BL J DENT RES 61 1163 982 88 KOLLBERG H EUR J RESPIR DIS 63 101 982 89 KOLLBERG H ACTA PAEDIATR SCAND 71 197 982 90 HUBBARD VS J AM DIET ASSOC 80 127 982 91 KEMP T ARCH INT PHYSIOL BIOCHIM 90 B123 982 92 STURGESS J AM J PATHOL 106 303 982 93 KRAEMER R EUR J PEDIATR 138 172 982 94 KATZ SM JAMA 248 2284 982 95 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 96 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 97 NEU HC J ANTIMICROB CHEMOTHER 11 1 983 98 CERQUETTI MC IMMUNOL COMMUN 12 375 983 99 HARD R TISSUE CELL 15 217 983 100 FRATES RC PEDIATR RES 17 30 983 101 BUCHANAN KD SCAND J GASTROENTEROL 18 155 983 102 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 103 CEDER O CLIN GENET 23 298 983 104 DAVIS PB J CHRON DIS 36 269 983 105 NEVE J ACTA PAEDIATR SCAND 72 437 983 106 BARDON A CLIN CHIM ACTA 133 311 983 107 ROSENBERG E KLIN PAEDIATR 195 323 983 108 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 109 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 110 VLADUTIU GD IRCS MED SCI BIOCHEM 12 515 984 111 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 112 WILSON GB CLIN GENET 26 331 984 113 MCCLURE HM ADV VET SCI COMP MED 28 267 984 114 MESHULAM T CLIN IMMUNOL IMMUNOPATHOL 32 151 984 115 TAWARA K RES COMMUN CHEM PATH PHARM 43 515 984 116 HODSON ME POSTGRAD MED J 60 225 984 117 BARDON A ACTA PAEDIATR SCAND 73 263 984 118 SATO K J CLIN INVEST 73 1763 984 119 MCNEILL WF AM J CLIN PATHOL 81 742 984 120 DAVIS PB CHEST 85 802 984 121 GILLARD BK AM J DIS CHILD 138 577 984 122 HANCOCK REW J INFECT DIS 149 220 984 123 GOLDBERG JB J BACTERIOL 158 1115 984 124 SATO K AM J PHYSIOL 247 R646 984 125 CASSANO WF MED HYPOTHESES 18 51 985 126 REYNOLDS HY DISEASE A MONTH 31 1 985 127 JACQUOT J INFECT IMMUN 47 555 985 128 FRASE LL GASTROENTEROLOGY 88 478 985 129 TABLAN OC J PEDIATR 107 382 985 130 FICK RB J INFECT DIS 151 589 985 131 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 132 BRIDGES MA PEDIATR RES 20 356 986 133 RUPP GM MED HYPOTHESES 20 245 986 134 SULLIVAN MM CHEST 90 239 986 135 DASGUPTA MK J CLIN IMMUNOL 7 51 987 136 DAVIS PB J DENT RES 66 667 987 137 TANDLER B J DENT RES 66 398 987 138 GEDSCHOLD J HUM GENET 75 277 987 139 EMMETT M PROC SOC EXP BIOL MED 184 74 987 PN 76151 RN 00505 AN 76242784 AU di-SantAgnese-P-A. Davis-P-B. TI Research in cystic fibrosis (third of three parts). SO N-Engl-J-Med. 1976 Sep 9. 295(11). P 597-602. (REVIEW). MJ CYSTIC-FIBROSIS: me. MN ALPHA-FETOPROTEINS: me. ANIMAL. CILIA: pp. CYSTIC-FIBROSIS: di, im, mi, pp. ELECTROLYTES: me. ENDOCRINE-GLANDS: pp. FEMALE. HETEROZYGOTE. HUMAN. INFECTION: im. KALLIKREIN: me. KININS: me. MUCUS: se. PEPTIDE-HYDROLASES: ai. PREGNANCY. PRENATAL-DIAGNOSIS. PSEUDOMONAS-AERUGINOSA: ip. RESPIRATORY-SYSTEM: mi. REVIEW. SALIVARY-GLANDS: me. SODIUM: me. STAPHYLOCOCCUS-AUREUS: ip. TEARS: me. TRACHEA: pp. EX Recent autonomic nervous system studies in cystic fibrosis are outlined. Miscellaneous observations regarding salivary and tear glands, calcium, the kallikrein-kinin system, protease inhibitor, alpha-fetoprotein, and endocrine glands are discussed. Because more than 90 per cent of patients with cystic fibrosis die of progressive pulmonary involvement, complicated by infection with Staphylococcus aureus or Pseudomonas aeruginosa (or both), the question of immunologic deficit is frequently raised. Extensive investigations have shown the immune system in cystic fibrosis to be intact. Studies regarding pulmonary ciliary clearance and bacteriology are also outlined. Despite extensive investigations, the basic defect of cystic fibrosis is still unknown. What then is the explanation of the two defects in homozygotes that lead to most clinical manifestations - the sweat electrolyte defect and the abnormality in physiochemical behavior of mucous secretions? The former is satisfactorily explained by a sodium-reabsorption-inhibitory factor present in sweat and saliva of patients. As for the mucous-secretion abnormality, the effect of the increasing calcium concentration in decreasing the solubility of glycoproteins suggests that this mechanism might be responsible for the precipitation and obstruction of organ passages, which gives rise to most symptoms in cystic fibrosis. Although there is an excellent diagnostic test for homozygotes (sweat test), one cannot rely on any of the present biologic assays for determination of heterozygosity or antenatal diagnosis. RF 143 BOLANDE RP ARCH PATHOL 95 172 973 144 STURGESS JM BR J EXP PATHOL 54 388 973 145 MARTINEZ JR PEDIATR RES 9 463 975 146 MARTINEZ JR PEDIATR RES 9 470 975 147 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 148 BUCHWALD M AM J HUM GENET 27 22A 975 149 WIESMANN UN J PEDIATR 76 444 970 150 BLOMFIELD J ARCH DIS CHILD 48 267 973 151 BOTELHO SY J PEDIATR 83 601 973 152 SIMOPOULOS AP PROC AM PEDIATR SOC ANNU MTG 201 971 153 FITZPATRICK DF NATURE NEW BIOL 235 173 972 154 SMITH QT NATURE NEW BIOL 240 56 972 155 DUFFY MJ NATURE NEW BIOL 246 151 973 156 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 157 MCEVOY FA CLIN CHIM ACTA 54 195 974 158 RAO GJS J PEDIATR 80 573 972 159 RAO GJS SCIENCE 177 610 972 160 COBURN MD AM REV RESPIR DIS 110 368 974 161 LIEBERMAN J PEDIATR RES 3 571 969 162 LIEBERMAN J AM REV RESPIR DIS 109 399 974 163 TALAMO RC PEDIATR RES 6 430 972 164 WEISS AS J CLIN INVEST 53 622 974 165 SCHWARTZ RH CF CLUB ABST 19 972 166 SHARP HL IN: MANGOS JA 229 973 167 TALAMO RC PEDIATRICS 56 91 975 168 SCHWARTZ RH PEDIATR RES 9 299 975 169 TALAMO RC IN: MANGOS JA 195 976 170 CHANDRA RK BR MED J 1 714 975 171 SMITH JA BR MED J 2 392 975 172 WALLWORK JC BR MED J 2 392 975 173 BROCK DJH BR MED J 2 392 975 174 DOLAN TF JR J PEDIATR 79 684 971 175 ROSEN SW J CLIN ENDOCRINOL METAB 32 410 971 176 HANDWERGER S N ENGL J MED 281 451 969 177 SIMOPOULOS AP PEDIATR RES 5 626 971 178 MURAD F J CLIN ENDOCRINOL METAB 40 552 975 179 ROSAN RC AM J DIS CHILD 104 625 962 180 WILMSHURST EG PEDIATRICS 55 75 975 181 MILUNSKY A AM J DIS CHILD 121 15 971 182 KJELLMAN NIM ARCH DIS CHILD 50 205 975 183 STAHL M J PEDIATR 84 821 974 184 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 185 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 186 SCHWARTZ RH AM J DIS CHILD 120 432 970 187 PENNINGTON JE AM J MED 58 629 975 188 DI SANTAGNESE PA DIS CHEST 27 654 955 189 MCFARLANE H BR MED J 1 423 975 190 GUGLER EC J PEDIATR 73 548 968 191 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 192 FALCHUK ZM PEDIATRICS 51 49 973 193 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 194 BOXERBAUM B AM REV RESPIR DIS 108 777 973 195 ROSSMAN CM PEDIATR RES 8 469 974 196 SANCHIS J N ENGL J MED 288 651 973 197 ASPIN NA PEDIATR RES 9 393 975 198 WOOD RE AM REV RESPIR DIS 111 733 975 199 MAY JR ARCH DIS CHILD 47 908 972 200 ZIERDT CH J CLIN MICROBIOL 1 521 975 201 DOGGETT RG APPL MICROBIOL 18 936 969 202 CONOVER JH LIFE SCI 14 253 974 CT 1 POLONOVSKI C PEDIATRE 12 379 976 2 AZIZI F N ENGL J MED 295 1381 976 3 RAMIREZ P N ENGL J MED 295 1381 976 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 5 ANON BR MED J 1 596 977 6 THOMAS JM PEDIATR RES 11 1148 977 7 LONGNECKER DS ENVIRON HEALTH PERSPECT 20 105 977 8 SCANLIN TF BIOCHEM BIOPHYS RES COMMUN 79 869 977 9 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 10 DOERING KM EUR J PEDIATR 126 185 977 11 FIGARELLA C GASTROENTEROL CLIN BIOL 2 343 978 12 OWEN E J MOL MED 3 203 978 13 DAVIS PB PEDIATR RES 12 703 978 14 IMPERO JE PEDIATR RES 12 108 978 15 PEARSON RD HEALTH LAB SCI 15 32 978 16 MARKOWITZ SM INFECT IMMUN 22 530 978 17 RAO GJS ENZYME 23 314 978 18 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 19 KOLLBERG H SCAND J RESPIR DIS 59 297 978 20 NEUTRA MR GASTROENTEROLOGY 75 701 978 21 VAUGHAN WJ SCIENCE 199 783 978 22 BRESLOW JL SCIENCE 201 180 978 23 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 24 HOSLI P LANCET 2 543 979 25 SEALE TW J CLIN MICROBIOL 9 72 979 26 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 27 GABRIDGE MG PEDIATR RES 13 31 979 28 THOMASSEN MJ PEDIATR RES 13 1085 979 29 LAM LFH LIFE SCI 24 2483 979 30 SORDELLI DO LIFE SCI 24 2003 979 31 COX DW PEDIATR CLIN NORTH AM 26 467 979 32 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 33 BROOKS FP MAYO CLIN PROC 54 475 979 34 SANTAGNESE PAD AM J MED 66 121 979 35 KOLLBERG H ACTA PAEDIATR SCAND 68 639 979 36 WOOD RE SOUTH MED J 72 189 979 37 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 38 SCANLIN TF CLIN CHIM ACTA 91 197 979 39 CHASE HP J PEDIATR 95 337 979 40 WEISMAN Y J PEDIATR 95 416 979 41 JAKEL HP BIOL ZENTRALBL 98 55 979 42 HOSLI P FEBS LETTERS 104 271 979 43 LEPRAT R ANN MICROBIOL (PARIS) B131 209 980 44 HOLSCLAW DS CLIN CHEST MED 1 407 980 45 SCANLIN TF CLIN CHEST MED 1 424 980 46 IANNACCONE G PEDIATR RADIOL 9 85 980 47 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 48 CASSINO RJJ PEDIATR RES 14 1212 980 49 DAVIS PB PEDIATR RES 14 83 980 50 GILLARD BK PEDIATR RES 14 1168 980 51 WILL PC PEDIATR RES 14 1245 980 52 WOODS DE INFECT IMMUN 30 694 980 53 PRIMOSCH RE ORAL SURG 50 301 980 54 SHIMAMOTO K J CLIN ENDOCRINOL METAB 51 840 980 55 GLORIABOTTINI F HUM GENET 54 79 980 56 WILSON GB J CLIN INVEST 66 1010 980 57 DAVIS PB J LAB CLIN MED 96 75 980 58 ROSENSTEIN BJ JOHNS HOPKINS MED J 147 188 980 59 BYLUND DB NATURE 285 229 980 60 DAVIS PB N ENGL J MED 302 1453 980 61 MATTHEWS WJ N ENGL J MED 302 245 980 62 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 63 FICK RB CLIN CHEST MED 2 91 981 64 REYNOLDS HY CLIN CHEST MED 2 103 981 65 ANTONELLI M RIV ITAL PEDIATR 7 769 981 66 BANCHINI G PEDIATR RES 15 1073 981 67 IMPERO JE PEDIATR RES 15 940 981 68 SANDERSON MJ PEDIATR RES 15 219 981 69 PIVETTA OH LIFE SCI 28 2207 981 70 FERNANDES PB INFECT IMMUN 33 527 981 71 OHMAN DE INFECT IMMUN 33 142 981 72 THOMASSEN MJ INFECT IMMUN 33 512 981 73 ANON NUTR REV 39 14 981 74 FICK RB J CLIN INVEST 68 899 981 75 WILSON GB J CLIN INVEST 68 171 981 76 TEGNER H ACTA PAEDIATR SCAND 70 629 981 77 CLARKE CW BR J DIS CHEST 75 15 981 78 JACOBS WH AM J GASTROENTEROL 76 342 981 79 LIEBERMAN J J LAB CLIN MED 97 646 981 80 MARKS MI J PEDIATR 98 173 981 81 LLOYDSTILL JD J PEDIATR 99 580 981 82 JAKEL HP BIOL ZENTRALBL 100 273 981 83 LEMANSKE RF AM REV RESPIR DIS 123 622 981 84 SORENSEN RU AM REV RESPIR DIS 123 37 981 85 ROSCHER AA J PHARMACOL EXP THER 216 419 981 86 BYLUND DB J PHARMACOL EXP THER 218 134 981 87 BRESLOW JL N ENGL J MED 304 1 981 88 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 89 SORSCHER EJ LANCET 1 368 982 90 STERN RC LANCET 1 1401 982 91 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 92 VANGEFFEL R IMMUNOL LETTERS 5 155 982 93 PALING MR SKELETAL RADIOL 8 63 982 94 TABAK LA J ORAL PATHOL 11 1 982 95 MORIARTY CM J MED 13 257 982 96 JAGGER KS J CLIN MICROBIOL 15 1054 982 97 BLITZER MG PEDIATR RES 16 203 982 98 PATTON CJ PEDIATR RES 16 1035 982 99 MITCHELL EA AUST PAEDIATR J 18 40 982 100 SHAPIRO BL AM J HUM GENET 34 846 982 101 BOYCE JR INFECT IMMUN 37 695 982 102 OHMAN DE INFECT IMMUN 37 662 982 103 WINNIE GB INFECT IMMUN 38 1088 982 104 DODGE JA ARCH DIS CHILD 57 774 982 105 KOLLBERG H EUR J RESPIR DIS 63 101 982 106 KOLLBERG H ACTA PAEDIATR SCAND 71 197 982 107 HUBBARD VS J AM DIET ASSOC 80 127 982 108 KEMP T ARCH INT PHYSIOL BIOCHIM 90 B123 982 109 STURGESS J AM J PATHOL 106 303 982 110 KRAEMER R EUR J PEDIATR 138 172 982 111 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 112 KATZ SM JAMA 248 2284 982 113 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 114 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 115 NEU HC J ANTIMICROB CHEMOTHER 11 1 983 116 CERQUETTI MC IMMUNOL COMMUN 12 375 983 117 HARD R TISSUE CELL 15 217 983 118 FRATES RC PEDIATR RES 17 30 983 119 JAGGER KN J CLIN MICROBIOL 17 55 983 120 SCHILLER NL PEDIATR RES 17 747 983 121 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 122 CEDER O CLIN GENET 23 298 983 123 DAVIS PB J CHRON DIS 36 269 983 124 NEVE J ACTA PAEDIATR SCAND 72 437 983 125 BARDON A CLIN CHIM ACTA 133 311 983 126 HANNE LF J BACTERIOL 154 383 983 127 ROSENBERG E KLIN PAEDIATR 195 323 983 128 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 129 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 130 SHAWKER TH J ULTRASOUND MED 3 267 984 131 VLADUTIU GD IRCS MED SCI BIOCHEM 12 515 984 132 RABKIN CS ANN NEUROL 15 608 984 133 BOYD RL PEDIATR RES 18 1028 984 134 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 135 SPEERT DP PEDIATR RES 18 431 984 136 ANWAR H FEMS MICROBIOL LETTERS 24 235 984 137 MCCLURE HM ADV VET SCI COMP MED 28 267 984 138 TAWARA K RES COMMUN CHEM PATH PHARM 43 515 984 139 HODSON ME POSTGRAD MED J 60 225 984 140 BARDON A ACTA PAEDIATR SCAND 73 263 984 141 SATO K J CLIN INVEST 73 1763 984 142 MCNEILL WF AM J CLIN PATHOL 81 742 984 143 DAVIS PB CHEST 85 802 984 144 GILLARD BK AM J DIS CHILD 138 577 984 145 HANCOCK REW J INFECT DIS 149 220 984 146 GOLDBERG JB J BACTERIOL 158 1115 984 147 SATO K AM J PHYSIOL 247 R646 984 148 CASSANO WF MED HYPOTHESES 18 51 985 149 MITCHELL EA AUST PAEDIATR J 21 127 985 150 REYNOLDS HY DISEASE A MONTH 31 1 985 151 JACQUOT J INFECT IMMUN 47 555 985 152 LUZAR MA INFECT IMMUN 50 577 985 153 LUZAR MA INFECT IMMUN 50 572 985 154 FRIEDMAN HZ GASTROENTEROLOGY 88 808 985 155 TABLAN OC J PEDIATR 107 382 985 156 MENDELMAN PM AM REV RESPIR DIS 132 761 985 157 FICK RB J INFECT DIS 151 589 985 158 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 159 BRIDGES MA PEDIATR RES 20 356 986 160 RUPP GM MED HYPOTHESES 20 245 986 161 KUZEMKO JA J ROY SOC MED 79 2 986 162 SULLIVAN MM CHEST 90 239 986 163 DASGUPTA MK J CLIN IMMUNOL 7 51 987 164 DAVIS PB J DENT RES 66 667 987 165 TANDLER B J DENT RES 66 398 987 166 GEDSCHOLD J HUM GENET 75 277 987 167 EMMETT M PROC SOC EXP BIOL MED 184 74 987 PN 76152 RN 00506 AN 76220253 AU Stapleton-F-B. Kennedy-J. Nousia-Arvanitakis-S. Linshaw-M-A. TI Hyperuricosuria due to high-dose pancreatic extract therapy in cystic fibrosis. SO N-Engl-J-Med. 1976 Jul 29. 295(5). P 246-8. MJ CYSTIC-FIBROSIS: dt. URIC-ACID: ur. MN ADMINISTRATION-ORAL. CASE-REPORT. CHILD-PRESCHOOL. HUMAN. MALE. PANCREATIC-EXTRACTS: ad, ae, tu. PANCREATIN: ad. PURINES: ae, an. URINATION-DISORDERS: et. AB Dysuria, uric acid crystalluria and hyperuricosuria developed in a child with cystic fibrosis and normal serum uric acid. Hyperuricosuria in this patient and two other children was directly related to ingestion of large amounts of pancreatic extract. In these three children, reducing pancreatic extract dosage by 85 percent lowered their purine intake by 307, 225, and 148 mg, respectively; urinary uric acid excretion decreased by 245, 239, and 158 mg. Overmedication resulted from parents' decisions to increase enzyme dosages. In our cystic fibrosis clinic, 15 of 32 patients screened at random were taking higher than the prescribed dose of pancreatic enzymes, and 14 of these 15 children were hyperuricosuric. On the basis of this information, we suggest that the minimal effective dose of pancreatic extract should be determined and adhered to for each child with cystic fibrosis to avoid potential renal injury from hyperuricosuria. RF 001 ANON BR MED J 2 161 970 002 GRIEBSCH A ADV EXP MED BIOL 41B 443 974 003 EMERSON BT NEPHRON 14 62 975 004 SCHNEIDER WC J BIOL CHEM 161 293 945 005 WEBB JM J BIOL CHEM 230 1023 958 006 UCHIDA T IN: CANTONI GL 3 966 007 WILSON DM KIDNEY INT 4 331 974 008 SEEGMILLER JE IN: COHEN AS 265 967 009 MICHENER WM AM J DIS CHILD 113 195 967 010 ROBSON AM J PEDIATR 79 42 971 011 LITTMAN A N ENGL J MED 281 201 969 012 MEYERS A AM J DIS CHILD 129 1011 975 CT 1 ANON LANCET 2 73 977 2 NOUSIAARVANITAKIS S J PEDIATR 90 302 977 3 DIMAGNO EP N ENGL J MED 296 1318 977 4 GAHL WA N ENGL J MED 297 1349 977 5 CLAIN JE S AFR MED J 53 582 978 6 NOUSIAARVANITAKIS S J PEDIATR 92 734 978 7 STAPLETON FB J PEDIATR 92 911 978 8 DAVIDSON GP J PEDIATR 93 976 978 9 WOOD RE SOUTH MED J 72 189 979 10 NEWMAN AJ J PEDIATR 94 594 979 11 FORSTNER GG J PEDIATR 95 331 979 12 GAHL WA J PEDIATR 95 330 979 13 ROBB TA LANCET 2 544 980 14 SACK J ISR J MED SCI 16 417 980 15 NIESSEN KH MONATSSCHR KINDERHEILKD 128 301 980 16 SAGRANSKY DM AM J DIS CHILD 134 319 980 17 GOW R LANCET 2 1071 981 18 CHO YW J CLIN PHARMACOL 21 224 981 19 PARK RW GASTROENTEROLOGY 81 1143 981 20 TOLCKMITT W KLIN PAEDIATR 193 365 981 21 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 22 MITCHELL EA AUST PAEDIATR J 18 114 982 23 SHAW DW NZ MED J 95 424 982 24 MISCHLER EH AM J DIS CHILD 136 1060 982 25 MCGUIRE S IR J MED SCI 151 253 982 26 GRENDELL JH CLIN GASTROENTEROL 12 551 983 27 SORKIN EM DRUG INTEL CLIN PHARM 17 110 983 28 DELUMLEY L ARCH FR PEDIATR 40 723 983 29 STAPLETON FB J PEDIATR 102 88 983 30 LANKISCH PG CLIN GASTROENTEROL 13 985 984 31 CHEN YT N ENGL J MED 311 128 984 32 PERRY RS CLIN PHARMACY 4 161 985 33 SPINO M J PEDIATR 107 64 985 34 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 35 DAUDON M ANN BIOL CLIN 44 25 986 36 RUBINSTEIN S PEDIATRICS 78 473 986 37 PHILLIPS BM J ROY SOC MED 79 44 986 PN 76153 RN 00507 AN 76196171 TI Letter: Cystic fibrosis: description and genetics. SO N-Engl-J-Med. 1976 Jul 22. 295(4). P 231. MJ CYSTIC-FIBROSIS. MN CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HUMAN. EX It is regrettable that the excellent article by Bowman and Mangos on cystic fibrosis should be marred by a minor, yet unfortunately common, error. The risks of producing a child with cystic fibrosis in Table 1 would have been essentially correct if they had been stated in fractional terms. They are, however, stated in odds forms, and therefore understatements of the true risks. The risk when both parents are known to be heterozygous, for example, is stated as 1:4. This would mean that the second alternative has four times as much likelihood of occurring as the first, and it is clearly not true. When both parents are heterozygous the odds are three times as likely that the child will be normal as affected. This is usually stated as a risk of 1/4, or 1:3, that the child will be affected. RF 001 BOWMAN BH N ENGL J MED 294 937 976 PN 76154 RN 00508 AN 77034612 TI Cystic fibrosis: thyroid function and alpha fetoprotein [letter]. SO N-Engl-J-Med. 1976 Dec 9. 295(24). P 1381-2. MJ ALPHA-FETOPROTEINS. CYSTIC-FIBROSIS: pp. THYROID-GLAND: pp. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. MALE. EX Our recent studies showed normal serum thyroxine but lower serum total and free tri-iodothyronine and higher serum thyrotropin concentration in children with cystic fibrosis than in controls matched for age and sex. Furthermore, administration of iodides to children with cystic fibrosis resulted in goiter decrease in serum thyroxine and increase in serum thyrotropin concentration. Our studies point to multiple derangement in thyroid hormone economy in children with cystic fibrosis. Peripheral deiodination of thyroxine to tri-iodothyronine may be defective, probably as a result of a state of malnutrition. An intrinsic abnormality may also be present in the thyroid gland. Iodide, which is frequently used in this disease, may enhance this defect, occasionally producing hypothyroidism. Therefore, the thyroid, like other organs of endodermal origin (lung and pancreas), may be involved in cystic fibrosis. Thyroid function should be examined in all children with cystic fibrosis, and if iodides are administered, replacement doses of thyroid hormones should also be given to prevent hypothyroidism. - A recent review of cystic fibrosis referred to the report by Chandra et al. that the serum level of alpha fetoprotein (AFP) is increased in patients with cystic fibrosis (CF) or heterozygotes who bear the CF gene. The report of Chandra et al. had prompted us to assess the level of AFP in cystic fibrosis, and we wish to record here briefly our negative observations. On the whole, these results suggest that AFP is not increased in cystic fibrosis. - We thought that the complications of therapy with iodides or other agents was beyond the scope of our review, and thus we did not discuss the very interesting observations of the high rate of goiter after iodide therapy in CF patients. Whether this phenomenon indicates a subclinical thyroid defect related to CF per se or is a secondary effect of chronic illness in youth is still unsettled. RF 001 DOLAN TF JR J PEDIATR 79 684 971 001 DI SANTAGNESE PA N ENGL J MED 295 597 976 001 DI SANTAGNESE PA N ENGL J MED 295 597 976 002 DOLAN TF JR J PEDIATR 79 684 971 002 AZIZI F TRANS ASSOC AM PHYSICIANS 87 111 974 002 CHANDRA RK BR MED J 1 714 975 003 FALLIERS CJ J ALLERGY 38 183 966 003 AZIZI F TRANS ASSOC AM PHYSICIANS 87 111 974 003 BAUMGARTIN A CLIN IMMUNOL IMMUNOPATHOL 6 42 976 004 HOLTER H LANCET 2 184 975 005 WALLWORK JC BR MED J 2 392 975 006 BROCK DJH BR MED J 2 392 975 007 FITZSIMMONS JS BR MED J 3 544 975 008 SMITH JA BR MED J 2 392 975 CT 1 SACK J ISR J MED SCI 19 17 983 PN 76155 RN 00509 AN 76149940 AU Zegers-B-J. Maertzdorf-W-J. Van-Loghem-E. Mul-N-A. Stoop-J-W. Van-Der-Laag-J. Vossen-J-J. Ballieux-R-E. TI Kappa-chain deficiency. An immunoglobulin disorder. SO N-Engl-J-Med. 1976 May 6. 294(19). P 1026-30. MJ ANTI-ANTIBODIES: an. DYSGAMMAGLOBULINEMIA: im. IGA: an. IMMUNOGLOBULINS-KAPPA-CHAIN. IMMUNOGLOBULINS-LIGHT-CHAIN. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: im. MN ANTIBODY-FORMATION. ANTIBODY-SPECIFICITY. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, fg, im. DIABETES-MELLITUS-INSULIN-DEPENDENT: co. DYSGAMMAGLOBULINEMIA: co, fg. HUMAN. IGG: ip. IGM: ip. IMMUNOELECTROPHORESIS. LINKAGE-GENETICS. MALABSORPTION-SYNDROMES: co. MALE. PHENOTYPE. AB Since kappa-chain deficiency is an unusual condition, we studied the clinical and laboratory findings in a patient with this deficiency. The patient had cystic fibrosis with concurrent malabsorption, diabetes mellitus and IgA deficiency. The serum levels of IgM and IgG were 0.85 and 7.22 mg per milliliter, respectively. Kappa type IgM and IgG was not present in serum and external secretions; gamma, mu and lambda chains were probably polyclonal in character. Antibodies against kappa chains were not detected in either the patient or the mother. Plasma cells containing kappa-type immunoglobulins were absent in jejunum samples and bone marrow; kappa-chainbearing B lymphocytes could not be detected in blood and bone marrow. The serum of one of the patient's sisters contained trace amounts of kappa-type immunoglobulins. The patient displays a complete absence of kappa- type immunoglobulins, probably owing to a genetic defect. RF 001 HOBBS JR ADV CLIN CHEM 14 219 971 002 NATVIG JB ADV IMMUNOL 16 59 973 003? SELIGMANN M REV FR ETUD CLIN BIOL 12 604 967 004 BERNIER GM BLOOD 40 795 972 005 BARANDUN S BLOOD 47 79 976 007 MAERTZDORF W EUR J CLIN INVEST 2 294 972 008 VAN DER GIESSEN M CLIN EXP IMMUNOL 21 501 975 009 VAN LOGHEM E IN: WEIR DM 1 973 010 MUL NAJ EUR J CLIN INVEST 5 63 975 011 HIJMANS W CLIN EXP IMMUNOL 4 457 969 012 VOSSEN JM ANN NY ACAD SCI 254 262 975 013 HIJMANS W IN: PEETERS H 181 973 014 ROSAN RC AM J DIS CHILD 104 625 962 015 PENNY R J PEDIATR 78 512 971 016 VYAS GN CLIN GENET 1 45 970 017 NADORP JHS EUR J CLIN INVEST 3 317 973 018 BALLIEUX RE ADV EXP MED BIOL 45 423 973 019 LAWTON AR CONTEMP TOP IMMUNOBIOL 3 193 974 020 VAN LOGHEM E EUR J IMMUNOL 4 57 974 021 HOOD L IN: STERZL J 283 970 022 NUSSENZWEIG V J EXP MED 126 727 967 023 CAPRA JD PROC NAT ACAD SCI USA 69 40 972 024 FRANKLIN EC J IMMUNOL 107 1527 971 CT 1 SOLOMON A SCAND J IMMUNOL 5 685 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 VANTHIEL DH ANN INTERN MED 86 10 977 4 COIFFIER B LYON MED 237 399 977 5 COOPER MD SPRINGER SEMIN IMMUNOPATHOL 1 265 978 6 SMITH WI DIABETES 27 1092 978 7 HENDRICKX GFM ACTA PAEDIATR SCAND 68 187 979 8 MILLER ME AM J HEMATOL 8 333 980 9 HODDINOTT S DIABETOLOGIA 23 326 982 10 VANLOGHEM E J CLIN INVEST 72 1918 983 11 ROSEN FS N ENGL J MED 311 300 984 12 WEDGWOOD RJ CLIN IMMUNOL ALLERGY 5 301 985 13 STAVNEZER J NUCLEIC ACIDS RES 13 3495 985 14 STAVNEZERNORDGREN J SCIENCE 230 458 985 15 OLIVIERO S J IMMUNOGENET 13 3 986 16 MOSS RB MONOGR ALLERGY 19 202 986 17 MONTECUCCO C ARTHRITIS RHEUM 29 1532 986 18 RENCKENS ALJM CLIN CHEM 32 2147 986 19 BUCKLEY RH CLIN IMMUNOL IMMUNOPATHOL 40 13 986 20 MONTECUCCO C HAEMATOLOGICA 71 237 986 PN 76156 RN 00510 AN 76149897 AU Bowman-B-H. Mangos-J-A. TI Current concepts in genetics. Cystic fibrosis. SO N-Engl-J-Med. 1976 Apr 22. 294(17). P 937-8. MJ CYSTIC-FIBROSIS: fg. MN ALBUMINS: an. CYSTIC-FIBROSIS: di. FEMALE. FETAL-DISEASES: di. GENETIC-COUNSELING. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: di. MECONIUM: an. PREGNANCY. PRENATAL-DIAGNOSIS. RATS. SUPPORT-U-S-GOVT-P-H-S. EX Cystic fibrosis has attracted the attention of biomedical investigators because it is the most prevalent chronic disease inherited in Caucasian children. Carriers of the gene leading to cystic fibrosis occur quite frequently in the Caucasian population. A major aim in research on cystic fibrosis is the development of a test that will offer reliable detection of the heterozygote and of the fetus with cystic fibrosis. Considerable numbers of patients with this disease are not diagnosed until after death. The therapy of patients with cystic fibrosis is still symptomatic and includes appropriate diet (or diets), pancreatic enzyme and vitamin supplementation, treatment of chronic obstructive pulmonary involvement and chronic infections by antibiotics, physical therapy and inhalation therapy, psychoemotional support, and vocational rehabilitation. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 DI SANTAGNESE PA PEDIATRICS 12 549 953 003 BOWMAN BH TEX REP BIOL MED 31 611 973 CT 1 THOMAS JM PEDIATR RES 11 1148 977 2 YOKOYAMA M PEDIATR RES 11 765 977 3 LONGNECKER DS ENVIRON HEALTH PERSPECT 20 105 977 4 HARTUNG J SOC BIOL 24 192 977 5 EVENSON MA ANAL CHEM 49 R 16 977 6 FINLEY PR AM J CLIN PATHOL 69 615 978 7 STRAUSS GD AM J DIS CHILD 133 301 979 8 STRAUSS GD J CHRON DIS 34 141 981 9 MCNEELY MC PEDIATR RES 16 21 982 10 LONGNECKER DS AM J PATHOL 107 103 982 11 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 12 NAYLOR SL AM J HUM GENET 39 707 986 PN 76157 RN 00511 AN 77011660 AU Newhouse-M. Sanchis-J. Bienenstock-J. TI Lung defense mechanisms (first of two parts). SO N-Engl-J-Med. 1976 Oct 28. 295(18). P 990-8. (REVIEW). MJ LUNG: ph. MN AIRWAY-OBSTRUCTION: pp. ANIMAL. BRONCHI: ph, se. BRONCHITIS: pp. CILIA: ph, ul. COMPLEMENT: ph. COUGH: pp. CYSTIC-FIBROSIS: pp. EXOCRINE-GLANDS: ph. FILTRATION. HUMAN. LUNG: im, me, pp, ul. MOTION. MUCUS: ph. PARTICLE-SIZE. REFLEX. RESPIRATION. RESPIRATORY-AIRFLOW. REVIEW. SMOKING: pp. TRACHEA: me, se. EX One of the most important functions of the lung, aside from its role in gas exchange and metabolism, is the provision of an essential biologic barrier between man and his environment. Of fundamental importance is the way in which the airways and lung parenchyma prevent entry of and neutralize or remove injurious agents, tasks performed so efficiently that the lung is normally sterile from the first bronchial division to terminal lung units. Inhaled particles may be deposited in the respiratory tract as a result of impaction, sedimentation, Brownian motion, turbulent diffusion, and, to a minimal degree, electrostatic forces. Particles deposited between the posterior two thirds of the nasal cavity and the nasopharynx and from the larynx to the terminal bronchioles land on airways lined by mucus-covered, ciliated epithelium and are removed by means of mucociliary transport mechanisms. The structure of the mucociliary transport system, physical aspects of mucus, nonspecific soluble factors in airways secretions, and cilia and mucociliary transport are discussed. RF 001 BOUHUYS A PHYSIOLOGY ENVIRONMENT AND LU 974 002 HATCH TF PULMONARY DEPOSITION AND RETE 964 003 OWEN PR IN: WOLSTENHOLME GEW 969 004 WEHNER AP CHEST 60 468 971 005 LANDAHL HD BULL MATH BIOPHYS 12 161 950 006 MOORMAN WJ ARCH ENVIRON HEALTH 26 153 973 007 LANDAHL HD ARCH IND HYG 1 36 950 008 STRANDBERG LG ARCH ENVIRON HEALTH 9 160 964 009 SPEIZER FE ARCH ENVIRON HEALTH 12 725 966 010 VAUGHAN TR ARCH ENVIRON HEALTH 19 45 969 011 PROCTOR DF IN: DAVIES CN 2 25 967 012 PROCTOR DF IN: FENN WO 1 309 964 013 MORROW PE HEALTH PHYS 2 366 960 014 HOFFMAN RA J APPL PHYSIOL 38 955 975 015 SCHROTER RC RESPIR PHYSIOL 7 341 969 016 LANDAHL HD BULL MATH BIOPHYS 12 43 950 017 MUIR DCF ANN OCCUP HYG 10 161 967 018 COFFIN DL ANN OCCUP HYG 15 219 972 019 AMDUR MO ARCH ENVIRON HEALTH 16 460 968 020 BOREN HG ENVIRON RES 1 178 967 021 BOREN HG ARCH ENVIRON HEALTH 8 119 964 022 WILKENING MH REV SCI INSTRUM 23 13 952 023 MCJILTON C SCIENCE 182 503 973 024 AMDUR MO ARCH ENVIRON HEALTH 16 460 968 025 AMDUR MO ARCH ENVIRON HEALTH 23 459 971 026 DALHAMN T AIR WATER POLLUT 7 517 963 027 DALHAMN T AIR WATER POLLUT 7 531 963 028 MERCER TT AEROSOL SCI 1 9 970 029 MORROW PE IN: BOUHUYS A 299 970 030 TIMBRELL V IN: MERCER TT 972 031 BEECKMANS JM INT J ENVIRON STUD 1 31 970 032 NADEL JA J APPL PHYSIOL 17 861 962 033 NADEL JA J APPL PHYSIOL 20 164 965 034 ZUSKIN E N ENGL J MED 290 660 974 035 WIDDICOMBE JG J APPL PHYSIOL 17 613 962 036 NADEL JA ARCH ENVIRON HEALTH 16 171 968 037 OLSEN CR J APPL PHYSIOL 20 202 965 038 NADEL JA ARCH ENVIRON HEALTH 7 179 963 039 GOUGH J INDIAN MED SURG 6 285 960 040 ROSS BB J APPL PHYSIOL 8 264 955 041 NEWHOUSE M IN: MANGOS JA 319 973 042 LOUDEN RG AM REV RESPIR DIS 96 666 967 043 WHITTENBERGER JL TRANS NAT TUBERC ASS 48TH MTG 952 044 JONES JG CLIN SCI 37 343 969 045 JONES JG BR J ANAESTH 42 280 970 046 WELLS WF AIRBORNE CONTAGION AND AIR HY 955 047 SANCHIS J J APPL PHYSIOL 33 757 972 048 RHODIN JAG AM REV RESPIR DIS SUPPL 3 93 1 966 049 BUCHER U THORAX 16 219 961 050 MATSUBA K AM REV RESPIR DIS 107 552 973 051 REID LM THORAX 10 199 955 052 SLEIGH MA INT REV CYTOL 25 31 969 053 SATIR P SCI AM 231 44 974 054 KINOSITA H PHYSIOL REV 47 53 967 055 KILBURN KH AM REV RESPIR DIS 98 449 968 056 REID L THORAX 15 132 960 057 NIEWOEHNER DE N ENGL J MED 291 755 974 058 MEYRICK B THORAX 24 729 969 059 CHARMAN J BIORHEOLOGY 9 185 972 060 DULFANO MJ AM REV RESPIR DIS 112 341 975 061 BANG BG ANN NY ACAD SCI 106 625 963 062 GUDBJERG CE ACTA RADIOL 42 269 954 063 ANON BULL PHYSIOPATH RESPIR NANCY 9 471 973 064 BOYD EM RESPIRATORY TRACT FLUID 972 065 YEAGER H JR AM J MED 50 493 971 066 DULFANO MJ SPUTUM FUNDAMENTALS AND CLINI 974 067 CHERNICK WS PEDIATRICS 24 739 959 068 DENTON R AM REV RESPIR DIS 98 380 968 069 MEYER FA BULL PHYSIOPATH RESPIR NANCY 9 259 973 070 DULFANO MJ AM REV RESPIR DIS 104 88 971 071 GIBSON LE LANCET 2 189 970 072 LUCAS AM ARCH OTOLARYNGOL 20 518 934 073 BANG BG PROC SOC EXP BIOL MED 106 516 961 074 SPICER SS IN: DULFANO MJ 22 973 075 RYLANDER R ARCH INTERN MED 126 496 970 076 WEIBEL ER MORPHOMETRY OF THE HUMAN LUNG 963 077 NEGUS V BR MED J 2 723 961 078 REYNOLDS HY J LAB CLIN MED 84 559 974 079 LAURELL CB SCAND J CLIN LAB INVEST 15 132 963 080 ERIKSSON S ACTA MED SCAND SUPPL 432 1 965 081 TALAMO RC N ENGL J MED 278 345 968 082 TALAMO RC J ALLERGY CLIN IMMUNOL 48 240 971 083 WARD PA J CLIN INVEST 52 516 973 084 TOURVILLE DR J EXP MED 129 411 969 085 MASSON PL PROTIDES BIOL FLUIDS 16 633 968 086 MASSON PL J EXP MED 130 643 969 087 ORAM JD BIOCHIM BIOPHYS ACTA 170 351 968 088 BULLEN JJ BR MED J 1 69 972 089 TOMASI TB JR ADV IMMUNOL 9 1 968 090 WHEELOCK EF ADV IMMUNOL 16 123 973 091 SADE J AM REV RESPIR DIS 102 48 970 092 SLEIGH MA AM REV RESPIR DIS 93 16 966 093 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 094 LUCHSINGER PC AM REV RESPIR DIS 97 1046 968 095 DALHAMN T ACTA PHYSIOL SCAND SUPPL 36 123 956 096 HILDING AC TRANS AM ACAD OPHTHALMOL OTOL 65 475 961 097 DADAIAN JH AM REV RESPIR DIS 103 808 971 098 GOSSELIN RE AM REV RESPIR DIS 93 41 966 099 LAURENZI GA J OCCUP MED 15 175 973 100 LAURENZI GA AM REV RESPIR DIS 103 800 971 101 BARCLAY AE J PHYSIOL (LOND) 90 482 937 102 HILDING AC ARCH ENVIRON HEALTH 6 61 963 103 YEATES DB J APPL PHYSIOL 39 487 975 104 WOOD RE AM REV RESPIR DIS 111 733 975 105 HILDING AC AM J PHYSIOL 191 404 957 106 REASOR MJ FED PROC ABST NO 898 33 365 974 107 SANCHIS J IN: WALTON WH 972 108 SANCHIS J N ENGL J MED 288 651 973 109 CAMNER P ARCH ENVIRON HEALTH 26 294 973 110 BOOKER DV NATURE 215 30 967 111 MARIN MG J APPL PHYSIOL 27 385 969 112 LAURENZI GA N ENGL J MED 279 333 968 113 SACKNER MA ANN INTERN MED 82 40 975 114 DALHAMN T ARCH OTOLARYNGOL 70 166 959 115 FALK HL J NATL CANCER INST 23 999 959 116 WANNER A ARCH ENVIRON HEALTH 27 370 973 117 SAKAKURA Y PROC SOC EXP BIOL MED 140 870 972 118 ALBERT RE ARCH ENVIRON HEALTH 18 738 969 119 LOURENCO RV J CLIN INVEST 50 1411 971 120 CAMNER P ARCH ENVIRON HEALTH 26 90 973 121 CAMNER P ARCH ENVIRON HEALTH 25 60 972 122 WESTERGAARD O ARCH KLIN EXP OHR NAS KEHLK H 203 179 973 123 CAMNER P ARCH ENVIRON HEALTH 23 421 971 124 YEATES DB J APPL PHYSIOL 39 487 975 125 DALHAMN T BR J IND MED 13 110 956 126 FRENCH JG ARCH ENVIRON HEALTH 27 129 973 127 CARNOW BW JAMA 214 894 970 128 LAWTHER PJ THORAX 25 525 970 129 HOLMA B ACTA MED SCAND SUPPL 473 1 967 130 GIORDANO AM JR ARCH ENVIRON HEALTH 25 443 972 131 KILBURN KH ARCH ENVIRON HEALTH 14 77 967 132 BALLANGER JJ PROC ASPEN EMPHYSEM CONF 11TH 968 133 DALHAMN T ACTA PHYSIOL SCAND 58 287 963 134 HIRSCH JA ARCH ENVIRON HEALTH 30 249 975 135 ANDERSEN I ARCH ENVIRON HEALTH 28 31 974 136 WOLFF RK ARCH ENVIRON HEALTH 30 521 975 137 WOLFF RK FED PROC ABST NO 951 34 387 975 138 ROSSMAN CM PEDIATR RES 8 469 974 139 WANNER A FED PROC ABST NO 1169 34 427 975 140 LANDA JF J APPL PHYSIOL 38 946 975 141 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 142 REASOR MJ FED PROC ABST NO 898 33 365 974 143 CAMNER P ARCH ENVIRON HEALTH 29 220 974 144 GREEN GM J CLIN INVEST 43 769 964 145 LAURENZI GA AM REV RESPIR DIS SUPPL 3 93 134 966 146 SELLERS TF J EXP MED 114 237 961 147 HERS JFP AM REV RESPIR DIS SUPPL 3 93 162 966 148 CAMNER P AM REV RESPIR DIS 108 131 973 149 JARSTRAND C AM REV RESPIR DIS 110 415 974 150 SCHWARZMANN SW ARCH INTERN MED 127 1037 971 151 CAMNER P AM REV RESPIR DIS 112 807 975 CT 1 NEWHOUSE M N ENGL J MED 295 1486 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 ANON BR MED J 1 1179 977 4 JUNOD AF BULL EUR PHYSIOPATH RESP 13 729 977 5 VOISIN C BULL EUR PHYSIOPATH RESP 13 3 977 6 DANNENBERG AM J RETICULOENDOTHEL SOC 22 273 977 7 LAFORCE FM ANESTHESIOLOGY 47 195 977 8 BRIGGS DD MED CLIN NORTH AM 61 1163 977 9 TILSON MD SURGERY 82 130 977 10 BREEZE RG AM REV RESPIR DIS 116 705 977 11 FOURNIER G LYON MED 238 59 977 12 COIL JA INFECT IMMUN 21 412 978 13 DEMARIA TF INFECT IMMUN 21 114 978 14 DOMINGUEZDEVILLOTA E ACTA ANAESTHESIOL SCAND 22 227 978 15 REASOR MJ J APPL PHYSIOL 45 190 978 16 NEWHOUSE MT CHEST 73 936 978 17 RUFFIN RE CHEST 73 501 978 18 HARVEY RP AM REV RESPIR DIS 117 695 978 19 MCLEOD E AM REV RESPIR DIS 118 561 978 20 REYNOLDS HY LUNG 155 225 978 21 BATEMAN JRM LANCET 1 294 979 22 REYNOLDS HY NOUV PRESSE MED 8 2057 979 23 KUTZ SA ENVIRON RES 19 405 979 24 NEWTH CJL PEDIATR CLIN NORTH AM 26 617 979 25 MARCUCCI F MINERVA PEDIATR 31 1379 979 26 CHOPRA SK THORAX 34 493 979 27 DONALDSON SS CANCER 43 2036 979 28 DICKERMAN JD BLOOD 54 354 979 29 COOPER JM AUST J EXP BIOL MED SCI 57 279 979 30 REYNOLDS HY CHEST 75 239 979 31 TISI GM AM REV RESPIR DIS 119 293 979 32 GONG H AM REV RESPIR DIS 120 821 979 33 DUNNILL MS J PATHOL 128 221 979 34 ETIEVANT M ANN IMMUNOL (PARIS) D131 13 980 35 COGGINS CRE TOXICOLOGY 16 83 980 36 COIL JA J SURG RES 28 18 980 37 ZANENLIM OG J CLIN PATHOL 33 474 980 38 NIELSEN H ANAESTHESIA 35 703 980 39 WALKER RD AM J VET RES 41 1015 980 40 REYES MP MED CLIN NORTH AM 64 363 980 41 BERRY EM LANCET 1 1159 981 42 BOUCHER RC CLIN CHEST MED 2 377 981 43 CHANANA AD J RETICULOENDOTHEL SOC 29 127 981 44 HEBERT JC J SURG RES 31 337 981 45 HESSE H ARZNEIMITTEL FORSCH DRUG RES 31-1 716 981 46 RUSSELLJONES GJ INT ARCH ALLERGY APPL IMMUNOL 66 316 981 47 COLLINS FM VET IMMUNOL IMMUNOPATHOL 3 5 982 48 BIENENSTOCK J BULL EUR PHYSIOPATH RESP 18 153 982 49 NIJHUISHEDDES JMA EUR J RESPIR DIS 63 249 982 50 NAKHOSTEEN JA DTSCH MED WSCHR 107 1713 982 51 HEIDBRINK PJ AM REV RESPIR DIS 125 517 982 52 WILKIE BN J AM VET MED ASSOC 181 1074 982 53 CHRETIEN J SCHWEIZ MED WOCHENSCHR 36 983 54 CLAYPOOL WD SURV SYNTH PATHOL RES 2 34 983 55 GILMOUR IJ UROL CLIN NORTH AM 10 65 983 56 HEBERT JC J TRAUMA 23 1 983 57 CADIEUX A ENVIRON HEALTH PERSPECT 51 287 983 58 JAY SJ MED CLIN NORTH AM 67 1251 983 59 LARSEN GL CHEST 83 S 1 983 60 LEUENBERGER P SCHWEIZ MED WOCHENSCHR 113 1006 983 61 ALBLAS ABV AM REV RESPIR DIS 128 276 983 62 CHRISTENSEN TG SURV SYNTH PATHOL RES 3 201 984 63 ELLENBERGER MA VET IMMUNOL IMMUNOPATHOL 7 315 984 64 GAUNT PN INT ANESTHESIOL CLIN 22 113 984 65 BIENENSTOCK J ANNU REV MED 35 49 984 66 LAFORCE FM INFECT IMMUN 45 692 984 67 PANCIERA RJ AM J VET RES 45 2538 984 68 RUSSELLJONES GJ AUST J EXP BIOL MED SCI 62 1 984 69 PENNINGTON JE AM J MED 76 34 984 70 HERROD HG SOUTH MED J 77 1007 984 71 ZIMMERLI W SCHWEIZ MED WOCHENSCHR 114 1074 984 72 SHENNIB H ARCH SURG 119 274 984 73 MCKIERNAN BC J AM VET MED ASSOC 185 1155 984 74 WINCHESTER JW NUCL INSTR METH PHYS RES (B) 231 360 984 75 LIGGITT HD VET CLIN NORTH AM F ANI PRACT 1 347 985 76 LEVISON ME REV INFECT DIS 7 S656 985 77 PRUETER JC VET CLIN NORTH AM S ANI PRACT 15 1085 985 78 REYNOLDS HY DISEASE A MONTH 31 1 985 79 TRIGO E AM J VET RES 46 1098 985 80 TOEWS GB INFECT IMMUN 48 343 985 81 HARADA RN CHEST 87 247 985 82 SONE S BIOCHIM BIOPHYS ACTA 823 227 985 83 WANNER A CLIN CHEST MED 7 247 986 84 NAIR P J HOSP INFECT 8 96 986 85 KOHLER D ATEMWEGS LUNGENKRANKH 12 358 986 86 STRATTON CW HEART LUNG 15 226 986 87 MARIN MG PHARMACOL REV 38 273 986 88 TAYLOR RG THORAX 41 295 986 89 OHMANN HB INFECT IMMUN 51 344 986 90 REISER J ARCH DIS CHILD 61 88 986 91 WOLFF RK ENVIRON HEALTH PERSPECT 66 223 986 92 LAFORCE FM J INFECT DIS 154 959 986 93 HEILMANN P J VET MED (B) 34 126 987 PN 76158 RN 00512 AN 77057479 AU Norman-A-P. TI Cystic fibrosis. SO Nurs-Times. 1976 Nov 18. 72(46). P 1804-6. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, di. GROWTH-DISORDERS: et. HUMAN. INFANT. INFANT-NEWBORN. MECONIUM. AB Gone are the days when it was thought that the prognosis was hopeless - at least three quarters of these children should live past the age of 19, especially now that the disease can be diagnosed at birth. The diagnosis and treatment of cystic fibrosis are explained. PN 76159 RN 00513 AN 76243160 TI Immunologic mechanisms in cystic fibrosis. SO Nutr-Rev. 1976 Jul. 34(7). P 210-1. (REVIEW). MJ CYSTIC-FIBROSIS: im. IMMUNITY. LUNG-DISEASES: et. MN ANTIBODY-FORMATION. ANTIGENS-BACTERIAL. BACTERIAL-INFECTIONS: et. CELL-MIGRATION-INHIBITION. HUMAN. IMMUNITY-CELLULAR. LUNG-DISEASES-OBSTRUCTIVE: et. MACROPHAGES: im. MUCOUS-MEMBRANE: im. PHAGOCYTOSIS. REVIEW. SPUTUM: im. AB Patients with cystic fibrosis appear to have normal immunocompetence although they are unusually susceptible to infections of the lung. RF 001 SCHWARTZ RH AM J DIS CHILD 111 408 966 002 OLIVIERI M PEDIATRIA (NAPOLI) 81 453 973 003 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 004 SOUTH MA J PEDIATR 71 645 967 005 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 006 FALCHUK ZM PEDIATRICS 51 49 973 007 OGRADY LR CF CLUB ABST 16 975 008 HANN S CF CLUB ABST 47 974 009 HILL HR J PEDIATR 84 55 974 010 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 011 HANN S CF CLUB ABST 24 975 012 GIBBONS A BR MED J 1 120 976 013 MCFARLANE H BR MED J 1 423 975 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 76160 RN 00514 AN 76268238 AU Robinson-P-G. Elliott-R-B. Fraser-J. TI Cystic fibrosis in New Zealand: incidence and mortality data. SO NZ-Med-J. 1976 Apr 28. 83(562). P 268-70. MJ CYSTIC-FIBROSIS: oc. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mo. ETHNIC-GROUPS. HOSPITALIZATION. HUMAN. INFANT. INTESTINAL-OBSTRUCTION: mo. MECONIUM. NEW-ZEALAND. RESIDENCE-CHARACTERISTICS. AB The incidence of and mortality from cystic fibrosis in New Zealand for the years 1965-1971 have been reviewed. Data were obtained from hospital admission and death records. All of the 171 cases were of European parentage except for one part-Maori child. The minimum incidence of cystic fibrosis in the European population (one in 3185 live births) is comparable with that in other populations of similar background but somewhat lower than that derived by Becroft (1968). The data show that, during the period of the survey, approximately 30 percent of cystic fibrosis suffereres died at less than 12 months of age. RF 001 ANTONELLI M FRACASTORO (VERONA) 63 207 970 002 BECROFT DMO NZ MED J 68 113 968 003 CONNEALLY PM TEX REP BIOL MED 31 639 973 004 DONNISON AB PEDIATRICS 37 833 966 005 MERRITT AD J LAB CLIN MED 60 998 962 006 ROBINSON PG NZ MED J 79 1024 974 007 SELANDER P ACTA PAEDIATR SCAND 51 65 962 008 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 CROSSLEY JR LANCET 2 1093 977 2 WESLEY AW NZ MED J 98 321 985 PN 76161 RN 00515 AN 77035343 AU Mandel-I-D. Wotman-S. TI The salivary secretions in health and disease. SO Oral-Sci-Rev. 1976. (8). P 25-47. (REVIEW). MJ SALIVA: se. SECRETORY-RATE: de. MN AMYLASES: an. BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: pp. DENTAL-CARIES: pc. DIGITALIS-GLYCOSIDES: pd. ELECTROLYTES: an. GLYCOPROTEINS: an. HUMAN. HYPERTENSION: pp. IGA-SECRETORY: an. MOUTH-DISEASES: pc. MURAMIDASE: an. PAROTID-GLAND: ir. PHYSICAL-STIMULATION. REVIEW. SALIVA: an, en, ph, re. SALIVARY-GLAND-DISEASES: me. SALIVARY-PROTEINS: an. SPECIMEN-HANDLING. SUBMANDIBULAR-GLAND: ir. AB The potential usefulness of analysis of the salivary secretions in diagnosis and prognosis is beginning to be explored in depth. The preliminary work already undertaken indicates that modern methods applied to this secretion may provide information that is different from that obtained in other body fluids. Saliva is collected at the point of its manufacture and, therefore, is unaffected by collection or storage in the body. It is the product both of protein synthesis within the glands and of most of the known water and electrolyte exchange mechanisms. Salivary composition is affected by both autonomic and hormonal stimuli. As the specific influence of each of these factors is better understood, studies of this fluid will provide important clues to the understanding of disease and the evaluation of therapy. There are few places in the body where it is possible directly, utilizing a non-invasive technique, to examine the product of a large number of important biological processes. It is obvious that careful handling of collection and analytic techniques are essential if these secretions are to be utilized. Future investigations in clinical situations should take full advantage of the strong base of knowledge of the physiology of these glands. Development of this field depends on careful clinical investigations designed to make full use of our current knowledge. RF 001 ABELSON DC J ORAL MED 29 41 974 002 ABELSON DC J DENT RES SPEC ISS A A389 54 975 003$ BAIG MM J IMMUNOL 11 1826 973 004 BELLANTI JA PHAGOCYTIC CELL IN HOST 975 005 GAERLAN PF CF CLUB ABST 40 974 006 BERTRAM U ACTA ALLERGOL 19 458 964 007 BERTRAM U ACTA ODONT SCAND SUPPL 25 49 967 008 BERNFELD P METHODS ENZYMOL 1 149 955 009 BLAIR-WEST JR HANDBOOK OF PHYSIOLOGY 2 967 010 BLATT IM ANN OTOL RHINOL LARYNGOL 67 595 958 011 BLATT IM TRANS AM ACAD OPHTHALMOL OTOL 66 723 962 012 BLOCK PL NY STATE DENT J 28 116 962 013 BLOMFIELD J ARCH DIS CHILD 48 267 973 014 BOCHNER F ARCH NEUROL 31 57 974 015 BOGDONOFF MD J PSYCHOSOM RES 5 170 961 016 BOLTE HD VERH DTSCH GES INN MED 973 017 BRANDTZAEG P ACTA PATH MICROBIOL SCAND 79 189 971 018 BRANDTZAEG P ARCH ORAL BIOL 16 1295 971 019 BRANDTZAEG P IMMUNOLOGY 26 1101 974 020 BRANDTZAEG P SCAND J HAEMATOL SUPPL 12 1 970 021 BROWN CC PSYCHOPHYSIOL 7 66 970 022 BRUNNER HR N ENGL J MED 286 441 972 023 CALDWELL RC BIOCHIM BIOPHYS ACTA 101 157 965 024 CARAWAY WT AM J CLIN PATHOL 32 97 959 025 CHAUNCEY HH J DENT RES 33 321 954 026 CHANEY AL CLIN CHEM 8 130 962 027 CHEN PS ANAL CHEM 28 1756 956 028 CHISHOLM DM BR DENT J 135 393 973 029 CONN JW J LAB CLIN MED 45 6 955 030 DAWES C J DENT RES 49 1263 970 031 DAWES C J PHYSIOL (LOND) 220 529 972 032 ELLISON SA HANDBOOK OF PHYSIOLOGY 531 967 033 ERICSSON Y ACTA ODONT SCAND 17 131 959 034 GIBSON LE CLIN PEDIATR 12 450 973 035 GIBSON LE GAP CONF REP PROB SWEAT TESTI 975 036 GOLDMAN AS J PEDIATR 82 1082 973 037 GOW BS J DENT RES 44 885 965 038 HAMMER SM AM COLL CARDIOL 973 039 HAWKINS GR J DENT RES 42 105 963 040 HAYES CJ PROC AM PEDIATR SOC ANNU MTG 974 041 HENKIN RI J APPL PHYSIOL 22 316 967 042 HENKIN RI JAMA 217 4 971 043 HENKIN RI ANN INTERN MED 76 375 972 044 HILTON SM J PHYSIOL (LOND) 134 471 956 045 HOEPRICH P ANTIMICROB AGENTS CHEMOTHER 5 330 974 046 IRIBE K J NIHON UNIV SCH DENT 11 34 969 047 JENKINS GN IN: WOLSTENHOLME GEW 192 965 048 JENKINS GN ARCH ORAL BIOL 11 1203 966 049 JOSELOW M ARCH ENVIRON HEALTH 17 35 968 050 JUSKO WJ RES COMMUN CHEM PATH PHARM 10 189 975 051 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 052 KAHN N IADR PROGRAM ABST 169 969 053 KOYSOOKO R CLIN PHARMACOL THER 15 454 974 054 KRAUS FW ARCH ORAL BIOL 16 781 971 055 KRAKOWA P POL MED J 5 895 966 056 KULLANDER S ACTA ENDOCRINOL 48 329 965 057 KUTSCHER AH NY STATE J MED 68 28 968 058 LASHLEY KS J EXP PSYCHOL 1 461 916 059 LAULER D N ENGL J MED 267 1136 962 060 MANDEL ID PROC SOC EXP BIOL MED 115 959 964 061 MANDEL ID AM J DIS CHILD 113 431 967 062 MANDEL ID J PERIODONTOL 38 310 967 063 MANDEL ID ARCH ORAL BIOL 14 1433 969 064 MANDEL ID CLIN PEDIATR 8 161 969 065 MANDEL ID IN: GRANT DA 99 972 066 MANDEL ID J DENT RES PRG ABST 672 52 973 067 MANDEL ID IN: MERGENHAGEN S 973 068 MANDEL ID J DENT RES 53 246 974 069 MANDEL ID J PERIODONT RES 9 211 974 070 MARDER MZ J DENT RES SPEC ISS A A391 54 975 071 MCDONALD RC THESIS 950 072 MERGENHAGEN S COMPARATIVE IMMUNOLOGY OF 973 073 MORRISON M IN: PERSON P 968 074 MULROW PJ IN: WESSON LG 465 520 969 075 OEMRAWSINGH I ARCH ORAL BIOL 19 615 974 076 OPPENHEIM FG HELV ODONT ACTA 16 22 972 077 OPPENHEIM F IADR ABST 52 973 078 RAUCH S IN: GORLIN R 970 079 SCHNEYER LH J DENT RES 34 257 955 080 SHANNON IL ACTA ENDOCRINOL 46 393 964 081 SHANNON IL N ENGL J MED 271 37 964 082 SHANNON IL J DENT RES 51 6 972 083 SHANNON IL SALIVA COMPOSITION AND 974 084 SIEGEL IA J PHARMACOL EXP THER 187 518 973 085 SMITH TW J CLIN INVEST 49 2377 970 086 STROMINGER JL SCIENCE 156 213 967 087 SWANSON M CIRCULATION 47 736 973 088 TARBET WJ IADR ABST 92 968 089 TAUBMAN M SALIVARY RES GRP IADR 152 973 090 TOMASI TB JR ADV IMMUNOL 9 1 968 091 TRUELOVE EL J DENT RES 46 1400 967 092 VOLKER JF J DENT RES 25 9 947 093 WARD R LANCET 2 726 972 094 WEINSTEIN E ARCH ORAL BIOL 17 375 972 095 WOLF RD ARCH ORAL BIOL 16 1357 971 096 WOTMAN S J DENT RES SPEC ISS A A52 54 975 097 WOTMAN S J ORAL THER 3 239 967 098 WOTMAN S ARCH INTERN MED 124 477 969 099 WOTMAN S ARCH INTERN MED 126 248 970 100 WOTMAN S N ENGL J MED 285 871 971 101 WOTMAN S J APPL PHYSIOL 35 322 973 102 WOTMAN S IADR ABST 673 973 103 WOTMAN S POSTGRAD MED 53 73 973 104 WOTMAN S J PERIODONTOL 44 278 973 105 ZALL KM ANAL CHEM 28 1165 956 106 ZENGO AN ARCH ORAL BIOL 16 557 971 CT 1 KLOCK B SCAND J DENT RES 85 56 977 2 TAMARIN A J ORAL PATHOL 8 293 979 3 SKURK A ARCH ORAL BIOL 24 739 979 4 GERMAINE GR INFECT IMMUN 26 991 979 5 KLOCK B SCAND J DENT RES 87 129 979 6 GABAY EL J ORAL REHABIL 7 435 980 7 MANDEL ID J ORAL PATHOL 9 92 980 8 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 9 SLOMIANY BL ARCH ORAL BIOL 25 749 980 10 EPSTEIN SR J PERIODONTOL 51 336 980 11 IZUTSU K J DENT RES 59 1192 980 12 TWETMAN S SWED DENT J 5 9 981 13 ITO H MECH AGEING DEV 15 177 981 14 MORMANN JE CARIES RES 15 166 981 15 ECKERSALL PD ARCH ORAL BIOL 26 727 981 16 BAUM BJ LIFE SCI 29 1143 981 17 COLE MF INFECT IMMUN 31 998 981 18 GERMAINE GR INFECT IMMUN 31 598 981 19 SPIELMAN A ORAL SURG 51 144 981 20 SMITH QT J PERIODONTOL 52 673 981 21 BAUM BJ J DENT RES 60 1292 981 22 KUYATT BL J DENT RES 60 936 981 23 ITO H BIOCHEM BIOPHYS RES COMMUN 98 275 981 24 BAUM BJ J BIOL CHEM 256 9731 981 25 EDGAR WM J ORAL PATHOL 11 252 982 26 JONEJA JM ARCH ORAL BIOL 27 51 982 27 MARTINEZ JR ARCH ORAL BIOL 27 443 982 28 SLOMIANY BL ARCH ORAL BIOL 27 803 982 29 KARLSSON A J TROP PEDIATR 28 230 982 30 BAUM BJ J GERONTOL 37 392 982 31 EDGAR WM J DENT RES 61 1126 982 32 SLOMIANY BL J DENT RES 61 1163 982 33 DONATSKY O SCAND J DENT RES 90 157 982 34 STENBERG T SCAND J DENT RES 90 472 982 35 SLOMIANY BL ARCH ORAL BIOL 28 711 983 36 WIESENFELD D AUST DENT J 28 87 983 37 DISTLER W J DENT RES 62 87 983 38 ROLLA G SCAND J DENT RES 91 186 983 39 OHLSSON M HOPPE SEYLERS Z PHYSIOL CHEM 364 1323 983 40 MARSHALL T ELECTROPHORESIS 5 245 984 41 CROSSNER CG SWED DENT J 8 271 984 42 OSTERBERG T J ORAL REHABIL 11 157 984 43 STUCHELL RN OTOL CLIN NORTH AM 17 697 984 44 BENARYEH H J PSYCHIATR RES 18 299 984 45 OHURA K J PERIODONT RES 19 21 984 46 BENARYEH H ISR J MED SCI 20 197 984 47 BORGEAT F PSYCHOPHYSIOL 21 690 984 48 HEFT MW J DENT RES 63 1182 984 49 OGLESBEE LH CLIN CHIM ACTA 143 135 984 50 BENARYEH H GERODONTOLOGY 4 77 985 51 MORGAN BLG J ORAL PATHOL 14 37 985 52 SLOMIANY BL PROG LIPID RES 24 311 985 53 RUDNEY JD ARCH ORAL BIOL 30 765 985 54 LAIBLE NJ INFECT IMMUN 48 720 985 55 RUDNEY JD INFECT IMMUN 49 469 985 56 YOSHIDA Y ORAL SURG 59 426 985 57 LORBER M JAMA 253 778 985 58 SCOTT J GERODONTOLOGY 5 149 986 59 HAKANSSON B J ORAL REHABIL 13 235 986 60 RAVALD N J CLIN PERIODONTOL 13 758 986 61 AHLQWIST M COMMUNITY DENT ORAL EPIDEMIOL 14 358 986 62 YONTCHEV E INT J ORAL MAXILLOFAC SURG 15 687 986 63 JENZANO JW ARCH ORAL BIOL 31 627 986 64 SLOMIANY BL ARCH ORAL BIOL 31 463 986 65 PASTER BJ INFECT IMMUN 52 378 986 66 FOX PC ORAL SURG 61 243 986 67 HANDELMAN SL ORAL SURG 62 26 986 68 AGUIRRE A ARCH ORAL BIOL 32 297 987 69 EDGERTON M J PROSTHET DENT 57 57 987 70 SCHUBERT MM J DENT RES 66 680 987 PN 76162 RN 00516 AN 77124688 AU Sturgess-J-M. Moscarello-M-A. TI Alterations in the Golgi complex and glycoprotein biosynthesis in normal and diseased tissues. SO Pathobiol-Annu. 1976. 6. P 1-29. (REVIEW). MJ GLYCOPROTEINS: bi. GOLGI-APPARATUS: me. MN ADOLESCENCE. ADULT. ANIMAL. CHILD. CHILD-PRESCHOOL. CYCLOHEXIMIDE: pd. CYSTIC-FIBROSIS: me, pa. ENDOPLASMIC-RETICULUM: me. FREEZE-FRACTURING. GOLGI-APPARATUS: de, ul. HUMAN. LIVER-DISEASES: me. LIVER-DISEASES: pa. LIVER: me, pa. MICROSCOPY-ELECTRON. NEPHROSIS: me, pa. RATS. REVIEW. EX The Golgi complex has an important role in the regulation and normal development of the cell, contributing to anabolic functions including the assembly and secretion of macromolecules and to catabolic functions, in the synthesis of lysosomes. The central role of the Golgi complex in the biosynthesis of glycoproteins has been demonstrated. The purpose of this review is to focus attention on the structural and functional correlation of the Golgi complex in the liver, in relation to its involvement in disease. The hepatic parenchymal cell has been used widely as a model to study biosynthetic, absorptive and secretory functions of epithelial cell types. The studies are grouped under five broad headings: structural organization; development and differentiation; ultrastructural changes in pathologic conditions; alterations in glycoprotein biosynthesis; and alterations in glycosyltransferases. RF 001 DEDUVE C ANNU REV PHYSIOL 28 435 966 002 CARO LG J CELL BIOL 20 473 964 003 JAMIESON JD PROC NAT ACAD SCI USA 55 424 966 004 MAHLEY RW J LIPID RES 10 433 969 005 HAMILTON RL LAB INVEST 16 305 967 006 NEUTRA M J CELL BIOL 30 119 966 007 SJOSTRAND FS IN: DALTON AJ 968 008 PALADE GE IN: HAYASHI T 64 959 009 WHALEY WG SCIENCE 175 596 972 010 SCHACHTER H J BIOL CHEM 245 1090 970 011 EYLAR EH J THEOR BIOL 10 89 966 012 COOK GMW FED PROC 23 274 964 013 COOK GMW BIOCHIM BIOPHYS ACTA 101 57 965 014 WALDENSTROM J ACTA MED SCAND 117 216 944 015 SCHULTZE HE NATURFORSCH 10B 463 955 016 PETERKOFSKY A J IMMUNOL 76 237 956 017 ROWE DS BIOCHEM J 67 435 957 018 SPIRO RG N ENGL J MED 281 991 969 019 SCHACHTER H IN: FISHMAN WH 3 2 973 020 SPIRO RG J BIOL CHEM 235 2860 960 021 LEWIN PK EXP MOL PATH 8 21 968 022 SARCIONE EJ CANCER RES 27 2025 967 023 KUHLENSCHMIDT MS LAB INVEST 31 413 974 024 HUDGIN RL CAN J BIOCHEM 49 61 971 025 ITO S J CELL BIOL 27 475 965 026 RAMBOURG A INT REV CYTOL 31 87 971 027 STURGESS JM J MEMBR BIOL 12 367 973 028 BEAMS HW INT REV CYTOL 23 209 968 029 NORTHCOTE DH ENDEAVOR 30 26 971 030 MORRE DJ IN: REINERT J 971 031 CUNNINGHAM WP IN: LASKIN AI 974 032 CUNNINGHAM WP J CELL BIOL 63 1018 974 033 STURGESS JM PROC INT CONG ELECTR MICR 194 974 034 CLAUDE A J CELL BIOL 47 745 970 035 BRUNI C AM J PATHOL 46 691 965 036 ZEIGEL RF J CELL BIOL 15 45 962 037 MOSCARELLO MA BIOCHIM BIOPHYS ACTA 320 123 972 038 STAEHELIN LA J CELL SCI 7 787 970 039 HEREWARD FW J CELL SCI 13 621 973 040 BRANTON D PHIL TRANS R SOC LOND BIOL 261 133 971 042 STURGESS JM J CELL BIOL 55 524 972 043 ROHR HP LAB INVEST 24 128 971 045 WEIBEL ER J CELL BIOL 42 68 969 046 WIENER J J CELL BIOL 37 47 968 047 STAUBLI W J CELL BIOL 42 92 969 048 RIEDE UN VIRCHOWS ARCH CELL PATHOL 9 16 971 049 BOLENDER RP J CELL BIOL 56 746 973 051 FLICKINGER CJ J ULTRASTRUCT RES 27 344 969 052 KOHN RR PRINCIPLES OF MAMMALIAN A 971 053 STURGESS JM LAB INVEST 31 6 974 054 BALIS JU OVERDRUK TYDSCHRIFR GASTORO E 7 133 964 055 SALLNER G J CELL BIOL 30 73 966 056 CHIU HF LAB INVEST 30 305 974 057 DALLNER G J CELL BIOL 30 97 966 058 BROWN RM JR J CELL BIOL 41 109 969 059 TRUMP BF IN: LAVIA MF 975 060 STEINER JW INT REV EXP PATH 3 65 964 061 TRUMP BF IN: RICHTER GW 84 971 062 SVBODA D CANCER RES 28 1703 968 063 JEZEQUEL AM GUT 15 737 974 064 THIERY JP REV INT HEPATOL 12 207 962 065 SCHAFFNER F GASTROENTEROLOGY 37 565 959 066 ORLANDI F ACTA HEPATO SPLENOL 9 155 962 067 ORLANDI F ANN NEUROL PSICHIE ANN DESPID 55 1 961 068 SCHAFFNER F IN: MARTINI GA 962 069 BIEMPICA L LAB INVEST 24 110 971 070 DAVID H Z ZELLFORSCH MIKROSK ANAT 53 857 961 071 SALOMON JC BULL ASSOC FRANC CANCER 53 139 962 072 OBERLING C ANN ANAT PATHOL 1 401 956 073 REYNOLDS ES J CELL BIOL 19 139 963 075 STURGESS JM LAB INVEST 31 6 974 076 ARCASOY M AM J PATHOL 52 841 968 077 JEZEQUEL AM ANN ANAT PATHOL 3 512 958 078 HRUBAN Z J ULTRASTRUCT RES 8 236 963 079 SHINOZUKA H LAB INVEST 18 72 968 080 DAVID H BEITR PATHOL ANAT 134 463 966 081 STEINER JW AM J PATHOL 43 677 963 082 CAMAIN R ANN ANAT PATHOL 4 220 959 083 WEISBLUM B J CELL BIOL 12 313 962 084 DAVID H ACTA BIOL MED GER 4 159 960 085 JEZEQUEL AM PROG LIVER DIS 2 303 966 086 OZAKI Y ACTA SCHOL MED UNIV KYOTO 33 159 956 087 HARKIN JC AM J PATHOL 34 559 958 088 KOVACS E CAN J BIOCHEM 48 39 970 089 MOSCARELLO MA BIOCHIM BIOPHYS ACTA 127 373 966 090 HRUBAN Z LAB INVEST 14 70 965 091 MOLLENHAUER HH EXP MOL PATH 11 113 969 092 MICHAEL AF LAB INVEST 11 266 962 093 FISHER ER AM J PATHOL 52 869 968 094 SALOMON MI ARCH INTERN MED 115 704 967 095 NEUHAUS DW EXP MOL PATH 2 183 973 096 HORNE CHW CLIN SCI 42 743 972 097 SHIBATA S LAB INVEST 27 457 972 098 PIERCE GB LAB INVEST 21 27 969 099 ROY AK J HISTOCHEM CYTOCHEM 20 89 972 100 ROSENMANN E BEITR PATHOL ANAT 149 227 973 101 DERR RF PROC SOC EXP BIOL MED 125 248 967 102 KUHN R NATURWISSENSCHAFTEN 35 125 948 103 MOSCARELLO MA CAN J BIOCHEM 45 136 967 104 DARKEN MA PHARMACOL REV 16 223 964 105 REID IM LAB INVEST 23 119 970 106 SPIRO RG J BIOL CHEM 241 1271 966 107 HERSCOVICS A BIOCHEM J 112 709 969 109 AKERBLOM HK HORM METAB RES 4 15 972 110 DARCY DA BR J EXP PATHOL 46 155 965 111 DARCY DA BR J EXP PATHOL 49 614 968 112 DARCY DA BR J EXP PATHOL 48 608 967 113 JAMIESON JC CAN J BIOCHEM 53 401 975 114 TRELOAR M J BIOL CHEM 249 6628 974 115 KIM YS J CLIN INVEST 51 2033 972 116 MOOKERJEA S CAN J BIOCHEM 50 738 972 117 SCHACHTER H BIOCHEM BIOPHYS RES COMMUN 45 1011 971 118 NOVIKOFF AB FED PROC 21 1130 962 119 ESSNER E J CELL BIOL 15 289 962 120 WEISBLUM B J CELL BIOL 12 313 962 121 WHALEY WG IN: LOCKE M 964 122 ASHTON FE CAN J BIOCHEM 48 841 970 123 KRAMER MF J CELL BIOL 52 147 972 124 JAMIESON JD J CELL BIOL 39 580 968 125 ORRENIUS S J CELL BIOL 28 181 966 126 MOOKERJEA S CAN J BIOCHEM 45 825 967 127 NEUHAUS DW PROC SOC EXP BIOL MED 117 244 964 128 HRUBAN Z LAB INVEST 27 62 972 129 D LA IGLESIA FA LAB INVEST 30 539 974 CT 1 BROADWELL RD J CELL BIOL 90 474 981 2 AKISAKA T HISTOCHEMISTRY 76 539 982 PN 76163 RN 00517 AN 76243687 AU Cahill-L-T. TI Impairment of natural defenses- II: Endogenous causes: diseases, malignancies, malnutrition, organ transplants. SO Pediatr-Ann. 1976 Jul. 5(7). P 457-64. MJ IMMUNOLOGIC-DEFICIENCY-SYNDROMES. INFECTION: et. MN ADRENAL-GLAND-DISEASES: co. ANEMIA-SICKLE-CELL: co. CHILD. CYSTIC-FIBROSIS: co. GRAFT-REJECTION: co. HUMAN. IMMUNOSUPPRESSION: ae. NEOPLASMS: co. NUTRITION-DISORDERS: co. TRANSPLANTATION-HOMOLOGOUS: ae. UREMIA: co. EX This article provides evidence that opportunistic infection can be expected in chronically debilitated, immunosuppressed persons. The onset of an infectious disease in these patients can be sudden or insidious with few prodromal signs. Anorexia, malaise, fever, and vomiting are nonspecific symptoms that these children may manifest because of their primary illness or its treatment. Any insult to natural defense barriers may cause serious infections in the compromised host. An infected wound from an insect bite, easily controlled by local measures in a healthy person, may become a focus for septicemia in a child with leukemia. Despite the diagnosis and appropriate treatment of the specific infection, the immunosuppressed person is still subject to overwhelming infection by resistant opportunistic organisms until the natural defense mechanisms are restored. An awareness of the vulnerability of the compromised host is the key to effective diagnosis and management of the various complications of infectious disease. RF 001 SONG YS AM J PATHOL 33 331 957 002 PEARSON HA N ENGL J MED 281 923 969 003 ERAKLIS AJ N ENGL J MED 276 1225 967 004 RAVRY M ANN INTERN MED 77 11 972 005 ROWLEY DA J IMMUNOL 65 515 950 006 SASLOW S N ENGL J MED 261 120 959 007 ROBINSON MG N ENGL J MED 274 1006 966 008 BARRETT-CONNOR E MEDICINE (BALTIMORE) 50 97 971 009 HOOK EW N ENGL J MED 257 403 957 010 RUBIN HM AM J DIS CHILD 116 83 968 011 SHULMAN ST N ENGL J MED 287 164 972 012 WILFERT CM PEDIATRICS 42 840 968 013 LANG PA TEX REP BIOL MED 24 107 966 014 KIRKPATRICK CH J EXP MED 119 727 964 015 MING PL FED PROC 27 432 968 016 COHN ZA BACT REV 24 96 960 017 WUEPPER KD CLIN EXP IMMUNOL 2 71 967 018 CHIGREN RA LANCET 2 688 967 020 MILLER ME J PEDIATR 81 979 972 021 VAN OSS CJ INFECT IMMUN 4 54 971 022 VEJLSGAARD R ACTA MED SCAND 193 337 973 023 BAKER RD DIABETES 9 143 960 024 MARTIN FP J PEDIATR 44 437 954 025 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 026 SPOCK A PEDIATR RES 1 173 967 027 BOWMAN BH SCIENCE 164 325 969 028 CONOVER JH LANCET 1 1194 973 029 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 030 STRAUSS RR CANCER RES 30 480 970 031 HOLLAND JF BLOOD 37 499 971 032 ARMSTRONG D MED CLIN NORTH AM 55 3 971 033 LOURIA DB ANN INTERN MED 67 261 967 034 SINCORICS JG CANCER 25 663 970 035 GOOCH WM CANCER 28 984 971 036 WOLLMAN MR J PEDIATR 86 376 975 037 WILBUR JR CANCER 36 765 975 038 CLAMAN HN N ENGL J MED 287 388 972 039 CLINE MJ SCIENCE 153 1135 966 040 FAUVE RM J EXP MED 125 807 967 041 RINEHART JJ N ENGL J MED 292 236 975 042 BOGGS DR AM J PATHOL 44 763 964 043 MAKMAN MH J BIOL CHEM 243 1485 968 044 HUGHES WT CANCER 36 2004 975 045 BRAZINSKY JH JAMA 209 1527 969 046 HUGHES WT PEDIATR CLIN NORTH AM 21 583 974 047 WELLER TH N ENGL J MED 285 203 971 048 OLDING LB J EXP MED 141 561 975 049 FEIGIN RD J PEDIATR 87 677 975 050 SCRIMSHAW NS AM J MED SCI 237 367 959 051 KLAINER AS AM J MED SCI 258 431 968 052 JOSE DG LANCET 1 263 976 PN 76164 RN 00518 AN 76175338 AU Suberman-R-I. TI Constipation in children. SO Pediatr-Ann. 1976 Jan. 5(1). P 32-48. MJ CONSTIPATION: et. MN ABNORMALITIES: ra. ANUS-IMPERFORATE: ra. ASCARIASIS: ra. CHILD. COLON: ra. COLONIC-DISEASES: ra. CONSTIPATION: ra. CYSTIC-FIBROSIS: ra. FEMALE. GASTROINTESTINAL-MOTILITY. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: et. INTESTINAL-OBSTRUCTION: ra. MALE. MEGACOLON: ra. PSYCHOPHYSIOLOGIC-DISORDERS. URETERAL-DISEASES: ra. EX Although constipation is not well defined, when it is brought to the attention of a physician, it should raise certain questions. In a newborn, is obstipation due to Hirschsprung's disease, meconium ileus, or intermittent volvulus? Is obstipation in a child with a mass in the cecum due to cystic fibrosis - i.e., meconium ileus equivalent? Has the formula been mixed correctly? Does the patient have spinal defects? Has the child been on medications? Is the child constipated for psychogenic reasons? All of us who deal with children should consider all of these entities in each of our patients, so as not to miss those several diseases with grave but preventable consequences. RF 001 BERDON WE RADIOLOGY 90 466 968 002 GANS SL J PEDIATR SURG 5 511 970 003 THOMPSON W RADIOLOGY 113 693 974 004 BERDON WE AM J ROENTG RAD THER NUCL MED 93 432 965 005 SHEY WL AM J ROENTG RAD THER NUCL MED 112 105 971 006 THOMAS CG JR ANN SURG 171 796 970 007 BERDON WE RADIOLOGY 96 375 970 008 NOBLETT HR J PEDIATR SURG 4 190 969 009 GROSSMAN H AM J ROENTG RAD THER NUCL MED 97 227 966 010 SWISCHUK LE AM J ROENTG RAD THER NUCL MED 103 339 968 011 FRIEDLAND GW RADIOLOGY 103 159 972 012 BURKE EC AM J DIS CHILD 117 668 969 013 GROSSMAN H IN: KAUFMAN HJ 970 014 CULP WC RADIOLOGY 115 9 975 015 GARRARD SD PEDIATRICS 10 479 952 016 KOTTMEIR PK PEDIATRICS 36 572 965 PN 76165 RN 00519 AN 76197001 AU Rosenthal-A. Tucker-C-R. Williams-R-G. Khaw-K-T. Strieder-D. Shwachman-H. TI Echocardiographic assessment of cor pulmonale in cystic fibrosis. SO Pediatr-Clin-North-Am. 1976 May. 23(2). P 327-44. MJ CYSTIC-FIBROSIS: co. ECHOCARDIOGRAPHY. PULMONARY-HEART-DISEASE: di. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. ELECTROCARDIOGRAPHY. FEMALE. HUMAN. LUNG-VOLUME-MEASUREMENTS. MALE. PULMONARY-HEART-DISEASE: et. AB The early clinical recognition and assessment of cor pulmonale are frequently difficult. To evaluate the usefulness of echocardiographic measurement of right ventricular dimension and septal motion in assessing cor pulmonale, we studied 94 patients with cystic fibrosis of varying severity. There was a significant relationship between increasing right ventricular dimension index (RVDI) and (1) decreasing clinical score, vital capacity, forced expiratory volume in one second (FEV1), and maximal midexpiratory flow rate (MMFR); and (2) increasing residual volume and thoracic gas volume. There was no correlation between right ventricular dimension index and PaO2. These findings were supported by electrocardiogram and pathologic evidence of right ventricular involvement. Abnormal septal motion was observed more frequently in patients with severe obstructive airway disease as measured by decreased vital capacity, FEV1, and MMFR. The data show that there is echocardiographic evidence of mild right ventricular enlargement and/or hypertrophy much earlier in the course of cystic fibrosis than previously suspected and that the echocardiogram is a useful tool in the evaluation of cor pulmonale in patients with cystic fibrosis. RF 001 BEIER FR AM REV RESPIR DIS 94 430 966 002 BISHOP JM MED CLIN NORTH AM 57 771 973 003 COOK CD PEDIATRICS 24 181 959 004 DIAMOND MA CIRCULATION 43 129 971 005 DI SANTAGNESE PA N ENGL J MED 277 1287 967 006 DORNHORST AC LANCET 1 746 952 007 FEATHERBY EA AM REV RESPIR DIS 102 737 970 008 FEIGENBAUM H ECHOCARDIOGRAPHY 972 009 FRANKLIN DL CIRC RES 10 17 962 010 GOLDRING RM J PEDIATR 65 501 964 011 GOODMAN DJ AM J CARDIOL 33 438 974 012 HIRSCHFELD S CIRCULATION 52 641 975 013 JOHNSON DH PEDIATR RES 8 350 974 014 KEANE JF PEDIATR RES 8 351 974 015 LAMARRE A PEDIATRICS 50 291 972 016 LANGE RL CIRCULATION 33 763 966 017 LIEBMAN J CIRCULATION 35 552 967 018 LIEBMAN J CHEST 63 218 973 019 LIEBMAN J IN: MANGOS JA 41 976 020 MCCANN WD JAMA 221 1243 972 021 MCDONALD IG BR HEART J 34 1137 972 022 MEYER RA PROG CARDIOVASC DIS 15 341 973 023 MOSS AJ J PEDIATR 67 797 965 024 NANDA NC CIRCULATION 50 575 974 025 REILLY BJ RADIOLOGY 98 281 971 026 ROWLATT UF PEDIATR CLIN NORTH AM 10 499 963 027 ROYCE SW PEDIATRICS 8 255 951 028 RUSHKIN J CIRCULATION 48 79 973 029 SHWACHMAN H AM J DIS CHILD 96 6 958 030 SIASSI B J PEDIATR 78 794 971 031 SPECTOR WS HANDBOOK OF BIOLOGIC DATA 162 956 032 STROBER W PEDIATRICS 43 416 969 033 SYMCHYCH PS ARCH PATHOL 92 409 971 034 TAJIK AJ CIRCULATION 46 36 972 035 THOMAS AJ BR HEART J 34 653 972 036 WAYMAN AE CIRCULATION 50 905 974 037 ANON WHO TECH REP SER NO 213 961 CT 1 ROSENTHAL A PEDIATRICS 59 919 977 2 RYSSING E ACTA PAEDIATR SCAND 66 753 977 3 SANTAGNESE PAD AM J MED 66 121 979 4 ALLEN HD CHEST 75 428 979 5 HIRSCHFELD SS CHEST 75 351 979 6 CHIPPS BE J PEDIATR 95 379 979 7 MATTHAY RA BR HEART J 43 474 980 8 TANG SC CHIN MED J 93 779 980 9 LESTER LA J PEDIATR 97 742 980 10 FRANCIS PWJ AM J DIS CHILD 134 734 980 11 FOWLER RS J ELECTROCARDIOL 14 319 981 12 RABINOVITCH M BR HEART J 45 198 981 13 BERGER HJ AM J CARDIOL 47 950 981 14 MATTHAY RA MED CLIN NORTH AM 65 489 981 15 JACOBSTEIN MD CHEST 80 399 981 16 NEWTH CJL AM REV RESPIR DIS 124 463 981 17 MOSS AJ PEDIATRICS 70 728 982 18 COATES AL CHEST 82 543 982 19 JOHNSON SR OBSTET GYNECOL 61 S 2 983 20 GOODING CA J PEDIATR 105 384 984 21 BENSON LN AM REV RESPIR DIS 130 987 984 22 MICHAEL JR AM REV RESPIR DIS 130 516 984 23 LESTER LA SEM RESPIR MED 6 285 985 24 PANIDIS IP J AM COLL CARDIOL 6 701 985 PN 76166 RN 00520 AN 76197021 AU Allars-H-M. Cockayne-D-J. Blomfield-J. Rush-A-R. Van-Lennep-E-W. Brown-J-M. TI Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. II. Electron microscopy and electrophoresis. SO Pediatr-Res. 1976 Jun. 10(6). P 584-94. MJ COLLOIDS: me. CRYSTALLIZATION. CYSTIC-FIBROSIS: me. PAROTID-GLAND: me. SALIVA: me. MN ADOLESCENCE. ADULT. CALCIUM-PHOSPHATES: me. CHILD. COLD. EDTA: pd. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. GUANIDINES: pd. HUMAN. MICROSCOPY-ELECTRON. PAROTID-GLAND: pa. SALIVA: de. NEPHELOMETRY-AND-TURBIDIMETRY. UREA: pd. AB Centrifuged pellets of turbid parotid saliva from cystic fibrosis (CF) patients and non-CF subjects, obtained from saliva kept at 2 degrees for 10 min, had the electron microscope appearance of amorphous, round particles, and were thought to be colloidal aggregates of organic material. Drops of turbid saliva, from samples incubated for 2 hr at 2 degrees or 37 degrees, additionally contained discrete, electron-dense crystals having well defined angular morphology: usually cubic, retangular, or approximately hexagonal. The inhibitors, urea, guanidine hydrochloride, and EDTA, resulted in no crystals being observed. Selected area electron diffraction from individual crystals showed predominantly hexagonal, rectangular patterns could be indexed as coming from hydroxyapitite. A transition from the hexagonal to the rectangular pattern and back to the hexagonal pattern could be obtained from individual crystals tilted in the electron microscope. The square diffraction pattern may be from octa-calcium or brushite. Polyacrylamide gel disc electrophoresis of the parotid saliva indicated that the sparingly soluble proteins in the 2 degree and 37 degree pellets comprised proline-rich proteins and a calcium-precipitable, trichloroacetic acid (TCA)-precipitable phosphoprotein, which fluoresced with amido schwarz and Coomassie brilliant blue G250. RF 001 ALLARS HM PEDIATR RES 10 578 976 002 AZEN EA SCIENCE 180 1067 973 003 BEEVERS CA ACTA CRYSTAL 11 273 958 004 BLOMFIELD J AUST PAEDIATR J 10 75 974 005 BLOMFIELD J GUT 14 558 973 006 BLOMFIELD J PEDIATR RES 10 574 976 007 BLOMFIELD J ARCH ORAL BIOL 19 1153 974 008 BLOMFIELD J ARCH DIS CHILD 48 267 973 009 BOAT TF PEDIATR RES 8 531 974 010 BROWN WE J AM CHEM SOC 79 5318 957 011 BROWN WE NATURE 196 1050 962 012 CANCRO LP PROC IADR 48TH GENERAL MTG 970 013 DAVIS BJ ANN NY ACAD SCI 121 404 964 014 EANES ED CALC TISS RES 12 143 973 015 FRANCIS MD CALC TISS RES 6 335 971 016 FRONDEL C AM MINERAL 26 145 941 017 FUREDI-MILHOFER H CALC TISS RES 8 142 971 018 HAY DI ARCH ORAL BIOL 18 1517 973 019 HAY DI ARCH ORAL BIOL 18 1517 973 020 JOHANSEN PG LANCET 1 455 968 021 KAMIYA M SEIKAGAKU 45 327 973 022 KARNOVSKY MJ J CELL BIOL 27 137A 965 023 LARSEN S EXPERIENTIA 27 483 971 024 LEACH SA J DENT RES 53 310 974 025 MAYO JW ARCH BIOCHEM BIOPHYS 161 134 974 026 MCKENZIE HA ADV PROT CHEM 22 55 967 027 MEYER JL CALC TISS RES 10 91 972 028 NOBLE RW J AM CHEM SOC 87 2236 965 029 NYLEN MU J CELL BIOL 18 109 963 030 PALLAVICINI JC ATTACHMENT FOR THE BUCHLER AP 964 031 POSNER AS PHYSIOL REV 49 760 969 032 REISNER AH ANAL BIOCHEM 64 509 975 033 RIVA A ANAT REC 176 149 973 034 SCHROEDER HE HELV ODONT ACTA 9 73 965 035 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 036 STEVE-BOCCIARELLI D J MICROSC 16 21 973 037 TERMINE JD CALC TISS RES 10 171 972 038 TERMINE JD ARCH BIOCHEM BIOPHYS 140 307 970 039 WARTON KL BR MED J 3 570 971 040 WAUGH DF J AM CHEM SOC 87 2246 965 041 WOTMAN S J PERIODONTOL 44 278 973 042 YOUNG RA ARCH ORAL BIOL 11 699 966 CT 1 ALLARS HM PEDIATR RES 10 578 976 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 PN 76167 RN 00521 AN 76268606 AU Shapira-E. Rao-G-J. Wessel-H-U. Nadler-N-L. TI Absence of an alpha two-macroglobulin-protease complex in cystic fibrosis. SO Pediatr-Res. 1976 Sep. 10(9). P 812-7. MJ ALPHA-MACROGLOBULINS: df. ARGININE: bl. CYSTIC-FIBROSIS: en. ESTERASES: bl. MN HUMAN. HYDROLYSIS. IMMUNE-SERA. IMMUNOELECTROPHORESIS. IMMUNOLOGIC-TECHNICS. RABBITS: im. AB The present study using immunologic methodology confirms previous observations from this laboratory of an absence of a protease component with arginine esterase activity in plasma of patients with cystic fibrosis. In this study, the pooled plasma from control individuals was activated and partially purified after adsorption on columns of soybean trypsin inhibitor conjugated to Sepharose 4B followed by elution with benzamidine. The fraction was further purified by isoelectrofocusing on polyacrylamide gels. Proteins around the pI range of 5.5 were eluted and utilized to prepare an antiserum. Immunoelectrophoresis of activated plasma samples from control subjects and patients with cystic fibrosis was performed utilizing the antiserum. In controls, four precipitin arcs with residual esterase activity were observed, whereas only three were seen in plasma from patients with cystic fibrosis. Double gel diffusion experiments using specific antisera ruled out the presence of trypsin, chymotrypsin, plasminogen, prothrombin, C1 esterase, alpha one-trypsin inhibitor, and inter-alpha-trypsin inhibitor in the concentrated benzamidine eluate. The antisera to alpha two- macroglobulin gave an immunoprecipitate which was readily stained for proteolytic activity. On immunoelectrophoresis, the alpha two- macroglobulin precipitin band corresponded to the band absent in plasma of patients with cystic fibrosis. In contrast, the alpha two- macroglobulin levels were similar in plasma of control subjects and patients with cystic fibrosis. Using the antiserum to the protein fractith proteolytic activity could be demonstrated in control plasma. One specific enzyme-active "rocket" was absent in plasma of patients with cystic fibrosis. In a double blind study of 15 control samples and 15 samples from patients with cystic fibrosis, a specific "rocket" was shown to be present in 13 control samples and absent in 14 cystic fibrosis samples. alpha two-Macroglobulin was determined by both an immunologic procedure and by its trypsin binding (trypsin protein esterase concentration). The ratio of the immunologic assay to the biologic activity assay was 90 for the normal plasma samples and only 65 for cystic fibrosis samples. RF 001 AXEN R NATURE 214 1302 967 002 BAGDASARIAN A J CLIN INVEST 54 1444 974 003 BARRETT AJ BIOCHEM J 133 709 973 004 BOWMAN BH SCIENCE 164 325 969 005 CONOVER JH PEDIATR RES 7 220 973 006 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 007 GANROT PO CLIN CHIM ACTA 14 493 966 008 HAMBERG U EUR J BIOCHEM 40 439 973 009 HARPEL PC J EXP MED 138 508 973 010 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 011 KAUZMANN W J AM CHEM SOC 75 5154 953 012 LAURELL CB ANAL BIOCHEM 15 45 966 013 LEVY HB PROC SOC EXP BIOL MED 103 250 960 014 LOWRY OH J BIOL CHEM 193 265 951 015 MANCINI G IMMUNOCHEMISTRY 2 235 965 016 MANGOS JA PEDIATR RES 1 436 967 017 OUCHTERLONY O ACTA PATH MICROBIOL SCAND 25 186 948 018 RAO GJS J PEDIATR 80 573 972 019 RAO GJS PEDIATR RES 8 684 974 020 RAO GJS PEDIATR RES 9 739 975 021 RAO GJS SCIENCE 177 610 972 022 SHAPIRA E J LAB CLIN MED 77 877 971 024 SHAPIRA E ISR J MED SCI 10 1086 974 025 SPOCK A PEDIATR RES 1 173 967 026 WILSON GB PEDIATR RES 10 87 976 CT 1 RAO GJS PEDIATR RES 11 981 977 2 WILSON GB PEDIATR RES 11 317 977 3 SHAPIRA E CLIN CHIM ACTA 78 359 977 4 SHAPIRA E J BIOL CHEM 252 7923 977 5 WILSON GB NATURE 266 463 977 6 SCHAAP T ISR J MED SCI 14 201 978 7 RAO GJS ENZYME 23 314 978 8 PEARSON RD PEDIATR RES 13 834 979 9 ROMEO G PEDIATR RES 13 1030 979 10 VANLEUVEN F PEDIATR RES 13 1384 979 11 DAVIS PB PEDIATR RES 14 83 980 12 WALSH MMJ PEDIATR RES 14 353 980 13 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 14 ROMEO G J LAB CLIN MED 95 116 980 15 SCHIDLOW DV AM REV RESPIR DIS 121 31 980 16 NELLES LP BIOCHIM BIOPHYS ACTA 623 46 980 17 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 18 HULTBERG B CLIN CHIM ACTA 112 167 981 19 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 20 BURY AF IRCS MED SCI BIOCHEM 10 255 982 21 BURY AF PEDIATR RES 16 613 982 22 ROBERTS RC PEDIATR RES 16 416 982 23 BRIDGES MA CLIN CHIM ACTA 118 33 982 24 VEREMEENKO KN UKR BIOKHIM ZH 55 218 983 25 BRIDGES MA ANN NY ACAD SCI 421 360 983 26 SHAPIRA E ANN NY ACAD SCI 421 352 983 27 EAGER KB PEDIATR RES 18 999 984 PN 76168 RN 00522 AN 76243782 AU Kopito-L-E. Shwachman-H. TI The pancreas in cystic fibrosis: chemical composition and comparative morphology. SO Pediatr-Res. 1976 Aug. 10(8). P 742-9. MJ CYSTIC-FIBROSIS: pa. PANCREAS: pa. MN ADOLESCENCE. ADULT. CALCIUM: me. CHILD. CHILD-PRESCHOOL. COPPER: me. FATTY-ACIDS: me. FEMALE. HISTOCYTOCHEMISTRY. HUMAN. INFANT. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: me. MAGNESIUM: me. MALE. ORGAN-WEIGHT. PANCREAS: me. PANCREATIC-DUCTS: me, pa. WATER-ELECTROLYTE-IMBALANCE: me. ZINC: me. AB Sections of pancreas from 16 individuals who died with cystic fibrosis (CF) were classified by morphometric criteria into four categories in increasing order of pancreatic involvement. The concentration of acini, islets, main ducts, lobular ducts, connective tissue, and fat was compared with control levels. The results show that in the least involved pancreases, from neonates who died under 5 months of age, acini were reduced to 33% of control levels and the following were increased: islets, to 410%, lobular ducts, to 250%; and main ducts, to 1700% of controls. With increasing severity of the pancreatic disease the acini were further reduced to 5% and lobular ducts to 37% of control levels, respectively. Main ducts increased by 19-fold, and fatty infiltration accounted for more than 25% of the fresh weight of the pancreas in 9 of the 16 specimens. Comparative biochemical studies of 35 fibrocystic pancreases were quantitatively related to the severity of the pancreatic involvement as follows. Water and volatile matter, normally accounting for 80 +/-% of the weight of the fresh pancreas, was reduced to less than 30% in the most affected organs. The concentration of zinc diminished from near normal mean levels of 193 mugZn/g dry pancreas to 10% of this amount in the severely involved pancreas. Elevated concentrations of calcium, amounting to over 10 times control level, were found in obstructed ductal structures. Calcium was depleted from pancreatic sections adjacent to the obstructions. The following biochemical indicators were significantly different in their mean levels in the 35 fibrocystic pancreases when compared with the 17 controls: (P less than or equal to 0.001) fat, water, zinc, calcium, copper, magnesium, potassium, and sodium (P less than or equal to 0.01). RF 001 ALLEN RA AM J PATHOL 31 337 955 002 BLANC WA GAP CONF REP PATHOL OF CF 972 003 BLOMFIELD J GUT 14 558 973 004 BUSCH H YALE J BIOL MED 29 596 957 005 CHERNICK WS ANN NY ACAD SCI 106 698 963 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 DONNISON AB PEDIATRICS 37 833 966 008 FORSTNER JF CLIN RES 21 1033 973 009 HADORN B CAN MED ASSOC J 98 377 968 010 HSU JM SCIENCE 153 882 966 011$ KLEINER IS BIOCHEMISTRY 962 012 KOPITO L ATOM ABSORP SPECTRO CLIN CHE 3 970 013 KOPITO L J PEDIATR 68 313 966 014 KOPITO LE FERTIL STERIL 24 512 973 015 RASMUSSEN H SCIENCE 170 404 970 016 RULE AH FERTIL STERIL 21 515 970 017 SHWACHMAN H AM J DIS CHILD 92 347 956 018 SHWACHMAN H IN: SLEISENGER MH 1206 973 019 TING KAI LI IN: PRASAD AS 966 020 VALLEE BL IN: ANSON ML 10 955 CT 1 SCHWACHMAN H N ENGL J MED 296 1519 977 2 ROLLA G ACTA PATH MICROBIOL SCAND (A) 86 83 978 3 ROMEO G NATURE 274 909 978 4 SANTAGNESE PAD AM J MED 66 121 979 5 IMRIE JR AM J PATHOL 95 697 979 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 IANNACCONE G PEDIATR RADIOL 9 85 980 8 GILLARD BK PEDIATR RES 14 1168 980 9 LOPEZCORELLA E PATOLOGIA (MEXICO CITY) 18 167 980 10 LLOYDSTILL JD PEDIATRICS 65 1010 980 11 SPEHLROBBERECHT M ANN RADIOL (PARIS) 24 49 981 12 CAVALIER SJ NEUROLOGY 31 714 981 13 LAMBERT JR GASTROENTEROLOGY 80 169 981 14 PARK RW GASTROENTEROLOGY 81 1143 981 15 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 16 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 17 MASOERO G AM J DIS CHILD 137 167 983 18 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 19 GOSDEN CM LANCET 2 541 984 20 KATZ S CELL CALC 5 421 984 21 IANNUCCI A HUM PATHOL 15 278 984 22 GILLARD BK AM J DIS CHILD 138 577 984 23 NEAL JL EVOLUTIONARY THEORY 7 153 985 24 GRAHAM N CLIN RADIOL 36 199 985 25 BOLLBACH R EUR J PEDIATR 144 167 985 26 DURIE PR PEDIATR RES 20 209 986 27 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 28 DAVIS PB J DENT RES 66 667 987 PN 76169 RN 00523 AN 76221607 AU Gahl-W-A. Changus-J-E. Pitot-H-C. TI The effect of spermidine and spermine on proliferation in vitro of fibroblasts from normal and cystic fibrosis patients. SO Pediatr-Res. 1976 May. 10(5). P 531-5. MJ CELL-DIVISION: de. CYSTIC-FIBROSIS: pa. SPERMIDINE: pd. SPERMINE: pd. MN DEPRESSION-CHEMICAL. FIBROBLASTS: cy, de, gd. HUMAN. SUPPORT-U-S-GOVT-P-H-S. AB The effects of spermidine and spermine at varying concentrations upon the replicative ability of human fibroblasts in cell culture have been studied. The average concentrations of spermidine causing a 50% inhibition of prolifertion (ID50) after 3 days of growth for three normal cell strains and three strains derived from patients with cystic fibrosis (CF) were 4.4 X 10(-6) +/- 1.2 M and 6.2 X 10(-6) +/- 2.1 M, respectively. The values for spermine were 2.0 X 10(-6) +/- 0.5 M for normal and 2.2 X 10(-6) +/- 0.1 M for fibroblasts from cystic fibrosis patients. No significant difference between the replicative ability of normal and CF cell strains was seen over a wide range of polyamine concentrations employed for a period of up to 3 days. RF 001 ALARCON RA ARCH BIOCHEM BIOPHYS 106 240 964 002 ALARCON RA ARCH BIOCHEM BIOPHYS 137 365 970 003 ALARCON RA J THEOR BIOL 37 159 972 004 ALARCON RA ARCH BIOCHEM BIOPHYS 94 540 961 005 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 006 BACHRACH U BIOCHIM BIOPHYS ACTA 134 174 967 007 BLASCHKO H J PHYSIOL (LOND) 145 124 959 009 COHEN SS INTRODUCTION TO POLYAMINES 971 010 COHEN LF PEDIATR RES 9 312 975 011 DYKSTRA WG SCIENCE 149 428 965 012 GIBSON LE PEDIATRICS 23 545 959 013 GOLDSTEIN J EXP CELL RES 37 494 965 014 HAM RG BIOCHEM BIOPHYS RES COMMUN 14 34 964 015 HAM RG IN VITRO 10 119 974 016 HERBST EJ ANN NY ACAD SCI 171 691 970 017 HIGGINS ML J CELL PHYSIOL 74 149 969 018 HIRSCH JG J EXP MED 97 345 953 019 HIRSCH JG J EXP MED 95 191 952 020 JANNE J ACTA PHYSIOL SCAND SUPPL 300 1 967 021 KIMES BW BIOCHIM BIOPHYS ACTA 228 223 971 022 LOBECK CC IN: STANBURY JB 1605 972 023 LUNDGREN DW CF CLUB ABST 15 2 974 024 MAGER J BIOCHIM BIOPHYS ACTA 36 529 959 025 MUNRO GF CF CLUB ABST 16 7 975 026 OTSUKA H J CELL SCI 9 71 971 027 POHJANPELTO P EXP CELL RES 80 137 973 028 POHJANPELTO P NATURE NEW BIOL 235 247 972 030 RENNERT OM IN: MANGOS JA 41 973 031 ROZANSKY R J GEN MICROBIOL 10 11 954 032 RUSSELL DH IN: RUSSELL DH 1 973 033 RUSSELL DH CANCER RES 31 248 971 034 TABOR CW J BIOL CHEM 239 2194 964 035 TABOR CW J BIOL CHEM 208 645 954 036 TABOR H PHARMACOL REV 16 245 964 CT 1 LIVINGSTON JN J BIOL CHEM 252 560 977 2 GAHL WA CHEMBIOL INTERACT 22 91 978 3 GAHL WA IN VITRO 15 252 979 4 CLO C BIOCHEM J 182 641 979 5 SEALE TW ARCH BIOCHEM BIOPHYS 198 164 979 6 WEBBER MM CELL BIOL INT REP 4 185 980 7 GAHL WA PEDIATR RES 14 118 980 8 SWANSON TL IN VITRO 16 761 980 9 JENSEN PKA IN VITRO 18 867 982 10 HENLE KJ CANCER RES 46 175 986 PN 76170 RN 00524 AN 76197011 AU Wu-J-T. Herbst-J-J. Bray-P-F. TI Elevated plasma levels of carcinoembryonic antigen in cystic fibrosis. SO Pediatr-Res. 1976 Apr. 10(4). P 235-8. MJ CARCINOEMBRYONIC-ANTIGEN: an. CYSTIC-FIBROSIS: im. MN CHILD. CYSTIC-FIBROSIS: bl, ur. HUMAN. SALIVA: im. SALIVATION. SUPPORT-U-S-GOVT-P-H-S. AB Elevated levels of plasma CEA were found in patients with cystic fibrosis. The elevations were well correlated with the Shwachman scores of the patients. Parallel changes of the levels of plasma CEA and clinical conditions of four patients were also observed in the follow-up studies. Close clinical examination indicated that the elevated plasma CEA of cystic fibrosis patients may be derived from sputum which is produced in abnormal amounts. This hypothesis is further supported by the very high content and the chromatographic profile of the CEA activity of sputum from patients with cystic fibrosis. RF 001 BOAT TF PEDIATR RES 8 531 974 002 BOWMAN BH AM J HUM GENET 25 16A 973 003 GOLD P J NATL CANCER INST 45 219 970 004 GOLD P J EXP MED 121 439 965 005 HANSEN HJ HUM PATHOL 5 139 974 006 LAMBLIN G CLIN CHIM ACTA 36 329 972 007 LAURENCE DJR BR MED J 3 605 972 008 LOGERFO P N ENGL J MED 285 138 971 009 MARTIN F J NATL CANCER INST 50 1375 974 010 ROGALSKY VY BULL EXP BIOL 12 64 971 011 ROGALSKY VY LANCET 1 1322 973 012 ROGALSKY VY LANCET 2 729 974 013 ROUSSEL P CLIN CHIM ACTA 36 315 972 014 ROUSSEL P J BIOL CHEM 250 2114 975 015 SHWACHMAN H AM J DIS CHILD 96 6 958 016 THOMSON MP PROC NAT ACAD SCI USA 64 161 969 018 WU JT J NATL CANCER INST 53 1589 974 CT 1 EGAN ML CANCER 40 458 977 2 ALHADEFF JA CLIN GENET 14 189 978 3 MACSWEEN JM PEDIATR RES 14 187 980 4 MULDER H ANTICANCER RES 3 247 983 5 WU JT CLIN CHEM 29 2049 983 6 WU JT CLIN CHIM ACTA 130 47 983 PN 76171 RN 00525 AN 76197015 AU Shapiro-B-L. Smith-Q-T. Warwick-W-J. TI Letter: Serum glutathione reductase and cystic fibrosis. SO Pediatr-Res. 1976 Apr. 10(4). P 250. MJ CYSTIC-FIBROSIS: bl. GLUTATHIONE-REDUCTASE: bl. MN HUMAN. EX We have obtained additional data which support the specificity of the reported serum glutathione reductase (GR) changes in cystic fibrosis (CF). As in previous series, mean GR in CF was 20% greater than in controls (P < 0.005). No difference was found between CF and controls for either lactate dehydrogenase (LDH) or glutamic-pyruvic transaminase (GPT). It appears that some underlying process or processes such as leakage into serum from cellular damage as reflected in raised LDH and GPT might lead to raised GR as demonstrated by positive correlations in controls. The absence of a positive correlation between these enzymes and GR in the CF sample as well as normal values for LDH and GPT in CF suggests that the raised serum GR in CF is not attributable to tissue leakage from damaged cells secondary to the disease. RF 001 SHAPIRO BL PEDIATR RES 9 885 975 PN 76172 RN 00526 AN 76197019 AU Blomfield-J. Rush-A-R. Allars-H-M. Brown-J-M. TI Parotid gland function in children with cystic fibrosis and child control subjects. SO Pediatr-Res. 1976 Jun. 10(6). P 574-8. MJ CYSTIC-FIBROSIS: pp. PAROTID-GLAND: pp. MN ADOLESCENCE. AMYLASES: an. CALCIUM: an. CHILD. CHILD-PRESCHOOL. CITRATES: pd. FEMALE. HUMAN. MALE. PAROTID-GLAND: de. PHOSPHATES: an. POTASSIUM: an. SALIVA: an. SALIVATION. SODIUM: an. STIMULATION-CHEMICAL. AB Parotid saliva was collected from 22 children with cystic fibrosis (CF) and 21 control children. Stimulation was by 0.1 ml 5% citric acid on the tongue every 15 sec over a period of 10 min, and samples were collected for 2-min intervals. Stimulated saliva showed a lag period of low amylase release, and an activated period of higher amylase release. The major difference between CF and child control parotid saliva was in the elevated calcium concentrations in CF subjects. Flow rate and amylase, although higher in the CF group, were not raised to a statistically significant level. There were higher mean values for sodium and inorganic phosphate in CF children but statistical significance was minimal. Potassium values were almost identical in CF and control saliva. In the activated parotid saliva samples of both control and CF subjects there were positive correlations between flow rate and calcium, flow rate and sodium, anylase and calcium, and sodium and calcium, and negative correlations between sodium and potassium. The parotid function test has no value as a diagnostic test for cystic fibrosis, as even calcium values show too much overlap with control saliva to be of diagnostic worth. RF 001 ALLARS HM PEDIATR RES 10 578 976 002 ALLARS HM PEDIATR RES 10 584 976 003 BLOMFIELD J AUST PAEDIATR J 10 75 974 004 BLOMFIELD J CF CLUB ABST 15 29 974 006 CHAUNCEY HH ARCH ORAL BIOL 7 707 962 007 CHEN PS ANAL CHEM 28 1756 956 008 DAWES C CARIES RES 1 333 967 009 DAWES C ARCH ORAL BIOL 14 277 969 010 GOEBELL H GUT 13 477 972 011 GOW BS J DENT RES 44 885 965 012 JOHNSTON WH ARCH DIS CHILD 31 477 956 013 KUTSCHER AH AM J DIS CHILD 110 643 965 014 LAWSON D ARCH DIS CHILD 44 715 969 015 MANDEL ID AM J DIS CHILD 113 431 967 016 MANDEL ID AM J DIS CHILD 110 646 965 017 MANGOS JA PEDIATR RES 1 436 967 018 MARMAR J GASTROENTEROLOGY 50 551 966 019 MATTHEWS EK J PHYSIOL (LOND) 234 689 973 020 SAGGERS BA ARCH DIS CHILD 42 187 967 021 SCHNEYER LH PHYSIOL REV 52 720 972 022 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 023 WALLACH D EUR J BIOCHEM 21 433 971 024 WIESMANN UN J PEDIATR 76 444 970 025 WOTMAN S ARCH ORAL BIOL 16 663 971 026 WOTMAN S J PERIODONTOL 44 278 973 CT 1 ALLARS HM PEDIATR RES 10 578 976 2 ALLARS HM PEDIATR RES 10 584 976 3 GILLARD BK PEDIATR RES 12 868 978 4 SETSER ME LAB INVEST 41 256 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 FEIGAL RJ NATURE 278 276 979 7 WOOD JH METH FIND EXP CLIN PHARMACOL 4 255 982 8 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 9 KATZ S CELL CALC 5 421 984 10 ROOMANS GM ANN NY ACAD SCI 428 121 984 11 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 PN 76173 RN 00527 AN 76197020 AU Allars-H-M. Blomfield-J. Rush-A-R. Brown-J-M. TI Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. I. Physicochemistry. SO Pediatr-Res. 1976 Jun. 10(6). P 578-84. MJ COLLOIDS: me. CRYSTALLIZATION. CYSTIC-FIBROSIS: me. PAROTID-GLAND: me. SALIVA: me. MN ADOLESCENCE. ADULT. CALCIUM: me. CHEMISTRY-PHYSICAL. CHILD. COLD. EDTA: pd. GUANIDINES: pd. HEATING. HUMAN. PHOSPHATES: me. PROTEINS: me. SALIVA: de. NEPHELOMETRY-AND-TURBIDIMETRY. UREA: pd. AB Two types of turbidity were found in parotid saliva from both cystic fibrosis (CF) patients and non-CF subjects. On cooling saliva, a rapidly forming, reversible, cold-dependent turbidity appeared in increasing amounts with decreasing temperature and increasing protein concentration. At 37 degrees, a slowly forming, stable turbidity appeared in increased amounts in parotid saliva samples containing increased amounts of calcium. The 2 degree centrifuged pellet consisted predominantly of protein, whereas the 37 degree pellet contained calcium, inorganic phosphate, and protein. The cold- dependent turbidity at 2 degrees was not inhibited by EDTA, but 37 degrees turbidity was dramatically inhibited. Urea and guanidine hydrochloride reduced 2 degree turbidity, and, to a lesser extent, inhibited 37 degree turbidity. The tendency towards higher levels of protein, amylase, and calcium in CF compared with child control parotid saliva (4, 6) causes a greater incidence and degree of turbidity formation in saliva of CF patients. In this paper only the nature of the turbidity has been investigated, not its relative occurrence in each group of subjects. RF 001 ALLAN J DIGESTION 11 428 974 002 ALLARS HM PEDIATR RES 10 584 976 003 AZEN EA SCIENCE 180 1067 973 004 BLOMFIELD J AUST PAEDIATR J 10 75 974 005 BLOMFIELD J GUT 14 558 973 006 BLOMFIELD J PEDIATR RES 10 574 976 008 BLOMFIELD J ARCH ORAL BIOL 19 1153 974 009 BOAT TF PEDIATR RES 8 531 974 010 BOURSNELL JC J REPROD FERTIL 36 91 974 011 CANCRO LP IADR ABST 107 970 012 CHEN PS ANAL CHEM 28 1756 956 013 DAWES C CARIES RES 1 333 967 014 DAWES C ARCH ORAL BIOL 14 277 969 015 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 016 GOW BS J DENT RES 44 885 965 017 GRON P ARCH ORAL BIOL 18 1365 973 018 GRON P ARCH ORAL BIOL 18 1385 973 019 HAY DI ARCH ORAL BIOL 18 1517 973 020 JOHANSEN PG LANCET 1 455 968 021 LOWRY OH J BIOL CHEM 193 265 951 022 MCKENZIE HA ADV PROT CHEM 22 55 967 023 NOBLE RW J AM CHEM SOC 87 2236 965 024 OAKENFULL DG PROC AUST BIOCHEM SOC 7 20 974 025 OPPENHEIMER EH J PEDIATR 75 806 969 026 ROBERTS TK J REPROD FERTIL 35 595 973 027 RULE AH FERTIL STERIL 21 515 970 028 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 029 TERMINE JD CALC TISS RES 15 81 974 030 TISCORNIA O DIGESTION 11 172 974 031 WAUGH DF J AM CHEM SOC 87 2246 965 032 WOTMAN S J PERIODONTOL 44 278 973 CT 1 ALLARS HM PEDIATR RES 10 584 976 2 GILLARD BK CLIN CHEM 23 2279 977 3 JAKEL HP BIOL ZENTRALBL 98 55 979 4 FEIGAL RJ NATURE 278 276 979 5 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 PN 76174 RN 00528 AN 76127229 AU Chou-L. Nadler-H. TI Absence of a serum factor in patients with cystic fibrosis. SO Pediatr-Res. 1976 Mar. 10(3). P 176-8. MJ CYSTIC-FIBROSIS: bl. MN CELL-MEMBRANE: me. CELLS-CULTURED. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: me. FIBROBLASTS: me. FUCOSE: me. HUMAN. SKIN: me. AB A serum factor was demonstrated in normal individuals which (1) enhances the incorporation of L-[3H]fucose into cultured human skin fibroblasts, (2) is nondialyzable, (3) is heat labile at 50 degrees, (4) is present in the noneuglobulin fraction, and (5) appears to be deficient in serum from patients with cystic fibrosis. The specific activity of L-[3H]fucose incorporated into skin fibroblasts from normal individuals in the presence of serum from 12 control subjects was 3,337 +/- 168 cpm/mg protein in contrast to 2,294 +/- 172, the activity obtained either in the presence of serum from 10 age-matched patients with cystic fibrosis or in the absence of serum. These differences were significant at P less than 0.001. In comparison, no significant difference was detected in the amount of L-[3H]fucose incorporated into skin fibroblasts derived from normal individuals and patients with cystic fibrosis. The plasma membrane of cultured skin fibroblasts derived from patients with cystic fibrosis appears to be grossly unaltered in its protein and L-fucose labeling pattern. RF 001$ ATKINSON PH J BIOL CHEM 210 5162 971 002 BAIG MM J PEDIATR 86 72 975 003 BESLEY GTN J MED GENET 6 278 969 004 BOSMANN HB ARCH BIOCHEM BIOPHYS 130 573 969 005 BOWMAN BH SCIENCE 164 325 969 006 BROWN GA LANCET 2 639 971 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 009 FAIRBANKS G BIOCHEMISTRY 10 2606 971 010 FITZPATRICK DF NATURE NEW BIOL 235 173 972 011 KAISER D PEDIATR RES 5 167 971 012 LOWRY OH J BIOL CHEM 193 265 951 013 MANGOS JA PEDIATR RES 1 436 967 014 NADLER HL NATURE 213 1261 967 015 NAITOH Y SCIENCE 166 1633 969 016 SPOCK A PEDIATR RES 1 173 967 017 WARREN L IN: DAVIS BD 967 CT 1 DISANTAGNESE PA N ENGL J MED 295 534 976 2 BANCHINI G PEDIATR RES 15 1073 981 PN 76175 RN 00529 AN 77057102 AU Smith-Q-T. Hamilton-M-J. Shapiro-B-L. TI Simple technique for detection of cystic fibrosis heterozygotes and homozygotes. SO Pediatr-Res. 1976 Dec. 10(12). P 999-1000. MJ CYSTIC-FIBROSIS: di. MN BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. HETEROZYGOTE. HOMOZYGOTE. HUMAN. ISOELECTRIC-FOCUSING. STAINS-AND-STAINING. EX We examined serum proteins from 18 CF subjects and 14 control individuals. We were unable to detect any consistent differences between the stained serum protein patterns of the CF and the control subjects. These patterns show greater resolution of proteins than those previously published, but, in contrast, no consistent differences between CF and control subjects. In addition, we have modified the published methods with procedures which would enhance the likelihood of electrophoretic detection of an abnormal protein with the reported properties. A portion of the samples analyzed by the published procedures also were analyzed by each of these modifications. None of the modified procedures showed consistent differences between serum proteins from the CF and the control subjects. No explanation is readily apparent for the difference between results of Thomas et al. and our own results on the one hand and the previously reported results on the other. We attempted to rigorously reproduce the isoelectric focusing techniques given in the literature. However, isoelectric focusing in polyacrylamide gel can be affected by small technical differences and, thus, factors essential to reproducing the techniques may not have been evident in the published procedure. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 THOMAS JM CF CLUB ABST 17 19 976 003 WILLIAMSON AR IN: WEIR DM 973 004 WILSON GB PEDIATR RES 9 635 975 005 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 WILSON GB PEDIATR RES 10 1001 976 2 THOMAS JM PEDIATR RES 11 1148 977 3 WILSON GB PEDIATR RES 11 139 977 4 WILSON GB PEDIATR RES 11 986 977 5 SCHOLEY J PEDIATR RES 12 800 978 6 WILSON GB PEDIATR RES 12 801 978 7 TULLY GW PEDIATR RES 13 1078 979 8 WILSON GB PEDIATR RES 13 1079 979 9 MANSON JC LANCET 1 330 980 10 NEVIN GB HUM GENET 56 387 981 11 HALLINAN FM CLIN CHIM ACTA 117 103 981 12 BULLOCK S CLIN GENET 21 336 982 13 BURY AF CLIN CHIM ACTA 118 45 982 14 ANON LANCET 2 249 985 15 JAMIESON A HUM GENET 70 168 985 16 GETLIFFE KA J INHERIT METAB DIS 9 348 986 PN 76176 RN 00530 AN 76101892 AU Wilson-G-B. Fudenberg-H-H. TI Studies on cystic fibrosis using isoelectric focusing. II. Demonstration of deficient proteolytic cleavage of alpha2-macroglobulin in cystic fibrosis plasma. SO Pediatr-Res. 1976 Feb. 10(2). P 87-96. MJ ALPHA-MACROGLOBULINS: me. CYSTIC-FIBROSIS: bl. PEPTIDE-HYDROLASES: df. MN ALPHA-MACROGLOBULINS: ip, an, df. ARGINASE: df, bl. BLOOD-PROTEINS: ip. HUMAN. ISOELECTRIC-FOCUSING. PEPTIDE-HYDROLASES: bl. PROTEIN-BINDING. TRYPSIN: bl. AB A protein with an isoelectric point (pI) of 5.48 was found to be deficient in plasma from most cystic fibrosis (CF) homozygotes and obligate heterozygote carriers of CF as compared with normal control plasma. Purification of the protein with a pI of 5.48 from normal plasma was performed using ammonium sulfate precipitation, DEAE- cellulose and CM-cellulose chromatography, Sephadex G-200- gel filtration, starch block electrophoresis, and Sepharose 4B gel filtration. The purified protein migrated as a single band on polyacrylamide gel electrophoresis, and displayed a single arc on immunoelectrophoresis against polyvalent antiserum to whole human serum. Results from various techniques used in its characterization indicate that this protein is a fragment of alpha2-macroglobulin (alpha2M) which is derived from alpha2M by proteolytic cleavage of intact alpha2M subunits. Quantitation of alpha2M levels in plasma indicated no significant differences between levels of alpha2M in CF homozygote, obligate heterozygote carrier, or normal control plasma samples. Quantitation of arginine esterase activity in plasma treated with cloroform and ellagic acid indicated that both the total arginine esterase activity and that fraction of arginine esterase activity inhibited by soybean trypsin inhibitor (SBTI) were decreased in most CF homozygote and obligate heterozygote plasma samples relative to normal control values. The results of this study indicate that plasma samples from CF homozygotes and obligate heterozygote carriers for CF show deficient proteolytic cleavage of alpha2M as compared with normal control plasma, and suggest that a structural abnormality in alpha2M or a deficiency in plasma proteolytic activity may be responsible for this deficiency in proteolysis. RF 001 BARNETT DR PEDIATR RES 8 687 974 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BARRETT AJ BIOCHEM J 133 709 973 004 BERATIS NG PEDIATR RES 7 958 973 005 BOWMAN BH IN: MANGOS JA 29 973 006 BOWMAN BH CLIN GENET 4 461 973 007 BOWMAN BH SCIENCE 167 871 970 008 COHEN LF PEDIATR RES 9 312 975 009 COLMAN RW J CLIN INVEST 48 23 969 010 CONNEALLY PM TEX REP BIOL MED 31 639 973 011 CONOVER JH PEDIATR RES 7 220 973 012 CONOVER JH LANCET 1 47 975 013 CONOVER JH LIFE SCI 14 253 974 014 DAVIS BJ ANN NY ACAD SCI 121 404 964 015 FAIRBANKS G BIOCHEMISTRY 10 2606 971 016 GANROT PO CLIN CHIM ACTA 13 518 966 017 GANROT PO CLIN CHIM ACTA 13 597 966 018 GANROT PO CLIN CHIM ACTA 16 328 967 019 GANROT PO ACTA CHEM SCAND 21 595 967 020 HARPEL PC J EXP MED 132 329 970 021 HARPEL PC J EXP MED 138 508 973 022 HIRSCHHORN K IN: MANGOS JA 11 973 023 JONES JM BIOCHEM J 127 187 972 024 KAPLAN AP MICROVASC RES 8 97 974 025 KUNKEL HG IN: BIER M 225 959 026 LANCHANTIN GF PROC SOC EXP BIOL MED 121 444 966 027 LASKOWSKI M IN: BOYER PD 3 375 971 028 LIEBERMAN J LANCET 1 1230 974 029 LIEBERMAN J CF CLUB ABST 44 975 030 LOWRY OH J BIOL CHEM 193 265 951 031 MANGOS JA PEDIATR RES 2 378 968 032 MARGOLIS J J PHYSIOL (LOND) 151 238 960 033 MCCOMBS ML TEX REP BIOL MED 31 615 973 034 MCCONNELL DJ J CLIN INVEST 51 1611 972 035 MOLENAAR J J IMMUNOL 112 1444 974 036 MUNRO GF CF CLUB ABST 16 7 975 037 OUCHTERLONY O IN: WEIR DM 191 973 038 RAO GJS PEDIATR RES 8 684 974 039 RAO GJS SCIENCE 177 610 972 040 RATNOFF OD ADV IMMUNOL 10 145 969 041 RATNOFF OD IN: GOOD RA 135 971 042 RAYMOND S ANN NY ACAD SCI 121 350 964 043 RENNERT OM IN: MANGOS JA 41 973 044 SAUNDERS R J CLIN INVEST 50 2376 971 045 SIEGELMAN AM ARCH BIOCHEM BIOPHYS 97 159 962 046 STEINBACH M REV FR ETUD CLIN BIOL 13 179 968 047 STEINBACH M BIOCHIM BIOPHYS ACTA 154 228 968 048 WILSON GB THESIS 974 050 WILSON GB PEDIATR RES 9 635 975 051 WILSON GB LIFE SCI 15 551 974 052 WILSON GB CLIN CHIM ACTA 49 79 973 053 CAUDILL M LANCET 2 307 973 CT 1 SHAPIRA E PEDIATR RES 10 812 976 2 WILSON GB PEDIATR RES 10 1001 976 3 LEDERBERG S AM J HUM GENET 28 597 976 4 WILSON GB TEX REP BIOL MED 34 51 976 5 FORSTNER G CLIN CHIM ACTA 70 459 976 6 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 7 RAO GJS PEDIATR RES 11 981 977 8 WILSON GB PEDIATR RES 11 139 977 9 WILSON GB PEDIATR RES 11 317 977 10 ARNAUD P J IMMUNOL METH 16 221 977 11 CHAN KYH CLIN CHIM ACTA 74 71 977 12 SHAPIRA E CLIN CHIM ACTA 78 359 977 13 GOLDSMITH GH J LAB CLIN MED 89 131 977 14 SHAPIRA E J BIOL CHEM 252 7923 977 15 WILSON GB NATURE 266 463 977 16 WILSON GB PEDIATR RES 12 801 978 17 ALHADEFF JA CLIN GENET 14 189 978 18 RAO GJS ENZYME 23 314 978 19 VIRCA GD ANAL BIOCHEM 89 274 978 20 WILSON GB J LAB CLIN MED 92 463 978 21 ROMEO G NATURE 274 909 978 22 PEARSON RD PEDIATR RES 13 834 979 23 ROMEO G PEDIATR RES 13 1030 979 24 VANLEUVEN F PEDIATR RES 13 1384 979 25 WILSON GB PEDIATR RES 13 1079 979 26 JAKEL HP BIOL ZENTRALBL 98 55 979 27 KURECKI T ANAL BIOCHEM 99 415 979 28 BARRETT AJ BIOCHEM J 181 401 979 29 SCANLIN TF CLIN CHEST MED 1 424 980 30 DAVIS PB PEDIATR RES 14 83 980 31 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 32 ROMEO G J LAB CLIN MED 95 116 980 33 BURDON MG CLIN CHIM ACTA 100 225 980 34 PARSONS M CLIN CHIM ACTA 100 215 980 35 SCHIDLOW DV AM REV RESPIR DIS 121 31 980 36 NELLES LP BIOCHIM BIOPHYS ACTA 623 46 980 37 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 38 WILSON GB J CLIN INVEST 68 171 981 39 VANLEUVEN F J BIOL CHEM 256 9016 981 40 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 41 WILSON GB MED HYPOTHESES 8 527 982 42 BURY AF IRCS MED SCI BIOCHEM 10 255 982 43 BLITZER MG PEDIATR RES 16 203 982 44 BURY AF PEDIATR RES 16 613 982 45 ROBERTS RC PEDIATR RES 16 416 982 46 BRIDGES MA CLIN CHIM ACTA 118 33 982 47 BRIDGES MA CLIN CHIM ACTA 118 21 982 48 TUMMLER B CLIN CHIM ACTA 125 219 982 49 BACK SA BIOCHEM MED 30 34 983 50 BACK SA J CHROMATOGR 278 43 983 51 BRIDGES MA ANN NY ACAD SCI 421 360 983 52 SHAPIRA E ANN NY ACAD SCI 421 352 983 53 WILSON GB ANN NY ACAD SCI 421 404 983 54 HJELM M CLIN BIOCHEM 17 284 984 55 EAGER KB PEDIATR RES 18 999 984 56 MARYNEN P BIOCHIM BIOPHYS ACTA 799 187 984 57 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 PN 76177 RN 00531 AN 76101889 AU Cheung-A-T. Jahn-T-L. TI High speed cinemicrographic studies on rabbit tracheal (ciliated) epithelia: cytolytic effect of cystic fibrosis serum on tracheal epithelial cells. SO Pediatr-Res. 1976 Feb. 10(2). P 144-47. MJ CILIA: ph. CYSTIC-FIBROSIS: bl. TRACHEA: cy. MN ANIMAL. CELL-MOVEMENT. EPITHELIUM: cy. MOTION-PICTURES. PHOTOMICROGRAPHY. RABBITS. TRACHEA: ph. EPITHELIUM: cy. AB High speed cinemicrographs are made on the ciliary activity of rabbit tracheal cilia with the aid of Nomarski optics. The detailed nature of the ciliary beat pattern is determined from slow motion analysis of the high speed cinemicrographs (1-3). Such documented forms of ciliary beat pattern and the physiologic state of the tracheal epithelia are utilized as basic reference controls to investigate the physiologic effects of cystic fibrosis serum on ciliated epithelia and the cystic fibrosis tracheal mucus stagnation phenomenon. Careful analyses of the high speed cinemicrographs reveal that cystic fibrosis serum has no effect on the rhythm and beat pattern of rabbit tracheal cilia. However, it is shown in controlled procedures that cystic fibrosis serum has a cytolytic effect on the tracheal epithelial cells and also at the cell junctions. Ciliary dyskinesis, as described in previous reports (6-9), is actually a secondary effect of cytolysis and cell destruction. RF 001 CHEUNG ATW DISSERTATION 973 006 CONOVER JH PEDIATR RES 7 220 973 007 KOPEL FB CF CLUB ABST 32 972 008 SPOCK A PEDIATR RES 1 173 967 009 SPOCK A MOD PROBL PEDIATR 10 200 967 CT 1 MARTINEZ JR MO MED 73 173 976 2 WILSON GB PEDIATR RES 11 143 977 3 BOGART BI PEDIATR RES 12 15 978 4 GABRIDGE MG PEDIATR RES 13 31 979 5 VERDUGO P PEDIATR RES 13 131 979 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 BOROVIKOVA TM TER ARKH 52 125 980 8 TEGNER H ACTA PAEDIATR SCAND 70 629 981 9 MARUYAMA I JAP J PHARMACOL 32 237 982 10 RUTLAND J AM REV RESPIR DIS 128 1030 983 11 CHEUNG ATW J MED PRIMATOL 15 37 986 12 HENNESSY SJ J APPL PHYSIOL 60 2109 986 PN 76178 RN 00532 AN 76101886 AU Farrell-P-M. Fox-G-N. Spicer-S-S. TI Determination and characterization of ciliary ATPase in the presence of serum from cystic fibrosis patients. SO Pediatr-Res. 1976 Feb. 10(2). P 127-35. MJ ADENOSINE-TRIPHOSPHATE: me. CILIA: en. CYSTIC-FIBROSIS: en. EPITHELIUM: en. EPITHELIUM: cy. MN ADENOSINE-TRIPHOSPHATASE: me. ANIMAL. CHROMATOGRAPHY: mt. CYSTIC-FIBROSIS: bl. ELECTROLYTES: pd. HUMAN. HYDROLYSIS. IN-VITRO. MICROSCOPY-ELECTRON. OYSTERS: an. RABBITS. AB The purpose of this investigation was twofold: (1) to identify and characterize the enzymatic ATP hydrolysis system of epithelial cilia, and (2) to develop a quantitative, biochemical test for the ciliotoxic cystic fibrosis (CF) factor based on inhibition of ATP utilization by ciliary preparations. Our rationale for selecting this system for CF factor analysis relates to the tight and essential mechanochemical coupling of functioning cilia. Using rabbit tracheal epithelium as the source, a high molecular weight (greater than 200,000) ATPase was identified, partially purified, and extensively characterized. The properties of this protein were similar to those observed in previous studies of others with flagellar and ciliary dynein (the motility-associated ATPase) isolated from microorganisms. Analysis of the pH profile revealed a broad range of high enzymatic activity between 6.5 and 9. Studies with potential cation activators showed that the enzyme is activated equally by either Ca2+ or Mg2+ in equimolar concentrations. No activation occurred in the presence of Zn2+, Na+, H+, or Na+ plus K+ and the effect of Mg2+ or Ca2+ was not inhibited by Na+, K+, or Na+ plus K+. The enzyme hydrolyzed Mg2+- containing solutions of UTP, CTP, and ADP at 51-54% the rate of ATP dephosphorylation, whereas Mg-deoxy-ATP was hydrolyzed 79% as effectively as ATP. Using a newly devised, analytical technique with [gamma-32P]ATP as the substrate, the ATP hydrolysis of various ciliary preparations from rabbit trachea and oyster gill (including motile suspensions) was monitored in the presence of sera from CF homo- and heterozygotes. Reproducible rates of ATP dephosphorylation averaging 27 nmol/min/mg protein were demonstrable with homogenates of ciliated epithelium. None of the test systems evaluated, however, were capable of demonstrating CF-related differences in ATPase activity or ATP utilization. Although these attemps have been unsuccessful thus far, the approach described in this report provides an example of an objective, quantitative, biochemical assessment of ciliary function. RF 001 AIELLO EL PHYSIOL ZOOL 23 120 960 002 BARGMAN GJ PEDIATR RES 8 464 974 003 BESLEY GTN J MED GENET 6 278 969 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 BOWMAN BH SCIENCE 164 325 969 006 BROKAW CJ SCIENCE 156 76 967 007 BROKAW CJ ARCH BIOCHEM BIOPHYS 125 770 968 008 BROKAW CJ ARCH BIOCHEM BIOPHYS 142 91 971 009 CHERRY JD J PEDIATR 79 937 971 010 CHEUNG ATW CF CLUB ABST 14 28 973 011 COLE CH PEDIATR RES 6 616 972 012 CONOVER JH PEDIATR RES 7 220 973 013 CONOVER JH PEDIATR RES 7 224 973 014 DI SANTAGNESE PA N ENGL J MED 277 1287 967 015 ECKERT R SCIENCE 176 473 972 016 EISENBERG E J BIOL CHEM 245 2451 970 017 FAIRBANKS G BIOCHEMISTRY 10 2606 971 018 FISKE CH J BIOL CHEM 66 375 925 019 GRAY J PROC R SOC LOND BIOL 96 95 924 020 GIBBONS IR J BIOL CHEM 241 5500 966 021 GIBBONS IR J CELL BIOL 54 365 972 022 GIBBONS BH J CELL BIOL 54 75 972 023 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 024 KINOSITA H PHYSIOL REV 47 53 967 025 LOWRY OH J BIOL CHEM 193 265 951 026 MANGOS JA PEDIATR RES 1 436 967 027 MANGOS JA GAP CONF REP CILIARY INHIBITO 2 973 028 POSSELT HG Z KINDERHEILK 110 93 971 029 SKOU JC PHYSIOL REV 45 596 965 030 SPOCK A PEDIATR RES 1 173 967 031 TAYLOR A PEDIATR RES 8 861 974 032 WARNER FD ADV CELL MOL BIOL 2 193 972 033 WACHSTEIN M J HISTOCHEM CYTOCHEM 8 387 960 034 WATANABE T BIOCHEM BIOPHYS RES COMMUN 52 195 973 035 WETZEL MG J CELL BIOL 30 299 966 036 WOOD RE LANCET 2 1452 973 CT 1 MARTINEZ JR MO MED 73 173 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 DISANTAGNESE PA N ENGL J MED 295 534 976 4 BOGART BI PEDIATR RES 12 15 978 5 KURLANDSKY LE PEDIATR RES 14 1263 980 6 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 PN 76179 RN 00533 AN 76197024 AU Forstner-J-F. Forstner-G-G. TI Effects of calcium on intestinal mucin: implications for cystic fibrosis. SO Pediatr-Res. 1976 Jun. 10(6). P 609-13. MJ CALCIUM: pd. CYSTIC-FIBROSIS: me. INTESTINAL-SECRETIONS: de. MUCINS: me. MN ANIMAL. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. RATS. SOLUBILITY. ULTRACENTRIFUGATION. VISCOSITY. AB A major feature of the disease cystic fibrosis is the excessive concentration of mucus within ducts and glands of mucous-producing organs. Some mucous secretions also show an elevation in calcium concentration. Using purified rat intestinal goblet cell mucin as a model mucin, we have investigated the effect of millimolar additions (1-25 mM) of CaCl2 on the physical properties of the mucin. Isotonicity of incubation media was preserved in order to mimic in vivo conditions. CaCl2 (8-15mM) caused a 15-33% decrease in viscosity, no change in electrophoretic mobility in acrylamide gels, and a 20-30% decrease in solubility of the mucin. Solubility changes were reversed by the addition of EDTA (20 mM) to incubations. Insolubility was also produced in incubations of mucin with a mixture of soluble intestinal contents (NaCl washings). These findings strongly suggest that the mucin became smaller and more dense as calcium was added, a process most probably achieved by loss of intramolecular water. RF 001 ALLEN A GUT 13 666 972 002 BARTOLINI A J MEMBR BIOL 13 283 973 003 BELLA A JR ARCH BIOCHEM BIOPHYS 150 679 972 004 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 005 BOAT TF PEDIATR RES 8 531 974 006 BUDDECKE E IN: GOTTSCHALK A 966 007 DI SANTAGNESE PA N ENGL J MED 277 1287 967 008 FORSTNER JF BIOCHIM BIOPHYS ACTA 386 283 975 009 FORSTNER JF CAN J BIOCHEM 51 1154 973 010 FORSTNER JF BIOCHEM J 124 563 971 011 FORSTNER JF J CELL SCI 12 585 973 012 GIBSON LE PEDIATRICS 48 695 971 013 GUGLER EC J PEDIATR 71 585 967 014 JAKOWSKA S CF AND RELATED HUMAN AND ANIM 970 015 JOOS RW PROC SOC EXP BIOL MED 124 1268 967 016 NEGUS V PROC R SOC MED 60 75 967 017 PIGMAN W ANN ANAT PATHOL 17 227 972 018 ROBERTS GP EUR J BIOCHEM 50 265 974 019 ROUSSEL P J BIOL CHEM 250 2114 975 020 TAYLOR M LARYNGOSCOPE 84 612 974 021 WARNER RR J CELL BIOL 64 54 975 022 WARTON KL BR MED J 3 570 971 CT 1 SANTAGNESE PAD N ENGL J MED 295 481 976 2 RAO GJS PEDIATR RES 11 981 977 3 WOOD DL PEDIATR RES 11 827 977 4 NEUTRA MR LAB INVEST 36 535 977 5 KATZ S PEDIATR RES 12 1033 978 6 ALHADEFF JA CLIN GENET 14 189 978 7 FORSTNER JF DIGESTION 17 234 978 8 KATZ S RES COMMUN CHEM PATH PHARM 19 491 978 9 NAKAMURA J CHEM PHARMACEUT BULL 26 857 978 10 NEUTRA MR GASTROENTEROLOGY 75 701 978 11 LIST SJ BIOCHEM J 175 565 978 12 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 13 MARRIOTT C BIORHEOLOGY 16 331 979 14 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 15 SCANLIN TF CLIN CHEST MED 1 424 980 16 DAVIS PB PEDIATR RES 14 83 980 17 KATZ S CLIN CHIM ACTA 100 245 980 18 LAMONT JT BIOCHIM BIOPHYS ACTA 629 553 980 19 HALLINAN F MED HYPOTHESES 7 793 981 20 HUTH SW ARCH OPHTHALMOL 99 1628 981 21 TABAK LA J ORAL PATHOL 11 1 982 22 MURAKAMI T CHEM PHARMACEUT BULL 30 659 982 23 FORSTNER G ADV EXP MED BIOL 144 199 982 24 MARRIOTT C ADV EXP MED BIOL 144 89 982 25 ZENTLERMUNRO PL BR MED J 287 501 983 26 KATZ S CELL CALC 5 421 984 27 KARLSSON S J MED GENET 21 441 984 28 ALBAZZAZ FJ RESPIRATION 46 88 984 29 ALBAZZAZ FJ J APPL PHYSIOL 59 1191 985 30 MULLER RM SCANN ELECTRON MICROSC 1985 1583 985 31 ALBAZZAZ FJ CLIN CHEST MED 7 259 986 32 SMITH AC CLIN GASTROENTEROL 15 815 986 33 RUBINSTEIN S PEDIATRICS 78 473 986 34 FEIGAL RJ ANN NY ACAD SCI 488 82 986 PN 76180 RN 00534 AN 77057091 AU Wilson-G-B. Arnaud-P. Monsher-M-T. Fudenberg-H-H. TI Detection of cystic fibrosis protein by electrofocusing. SO Pediatr-Res. 1976 Dec. 10(12). P 1001-2. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ISOELECTRIC-FOCUSING. MN HETEROZYGOTE. HOMOZYGOTE. HUMAN. ISOELECTRIC-FOCUSING: mt. EX Detection of cystic fibrosis protein (CFP) using out method involves rather exacting requirements for the collection of serum samples and their subsequent analysis by electrofocusing. Minor variations in the methods described for sample collection and storage may cause degradation and loss of CFP and this by itself could lead to erroneous results since many CF samples would appear to be CFP negative, making it impossible to observe a consistent difference between a group of CF samples and normal control samples by electrofocusing. From the results presented by Thomas et al. and Figure 1 of the letter by Smith et al., it is obvious that neither group has reproduced the protein handling patterns presented in either of our previous reports. The pH gradients are not the same, the location of the sample pads relative to the anode are not the same, and the protein banding patterns are totally different. We question the validity of the techniques used, claims of having reproduced our method for detecting CFP, or supposed improvements designed to enhance the detection of CFP. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 AWDEH ZL NATURE 219 66 968 003 CONOVER JH PEDIATR RES 7 220 973 004 SMITH QT PEDIATR RES 10 999 976 005 THOMAS JM CF CLUB ABST 17 19 976 006 WILLIAMSON AR IN: WEIR DM 973 007 WILSON GB PEDIATR RES 10 87 976 008 WILSON GB PEDIATR RES 9 635 975 009 WILSON GB CLIN CHIM ACTA 49 79 973 010 WILSON GB CLIN RES 24 295 976 CT 1 THOMAS JM PEDIATR RES 11 1148 977 2 WILSON GB PEDIATR RES 11 986 977 3 WILSON GB PEDIATR RES 11 317 977 4 WILSON GB PEDIATR RES 11 139 977 5 WILSON GB NATURE 266 463 977 6 SCHOLEY J PEDIATR RES 12 800 978 7 WILSON GB PEDIATR RES 12 801 978 8 JAMIESON A HUM GENET 70 168 985 PN 76181 RN 00535 AN 77035582 AU Gillard-B-K. Feig-S-A. Harrison-G-M. Nelson-T-E. TI Cystic fibrosis: enzymatic detection of a ciliostatic factor. SO Pediatr-Res. 1976 Nov. 10(11). P 907-10. MJ AMYLASES. CYSTIC-FIBROSIS: en. GLUCOSYLTRANSFERASES: ai. GLYCOGEN-DEBRANCHING-ENZYME: ai. MN AMYLASES: ai, me. DIALYSIS. HUMAN. POLYAMINES: pd. SALIVA: an, en. AB The ciliostatic cystic fibrosis (CF) factor has been investigated in order to develop an enzymatic assay for its detection. In saliva, this factor is associated with alpha-amylase. Removal or addition of the factor by dialysis does not affect the alpha-amylase catalytic activity of CF or control (normal) saliva. Crude preparations of the dialyzable factor from CF saliva are ciliostatic. Alpha-Amylase- catalyzed starch hydrolysis from both CF and control saliva was found to be insensitive to hydroxyalkylamine inhibitors so this method could not be used to detect differences in CF and control amylase. Since another carbohydrase, mammalian debranching enzyme, is much more sensitive to inhibition, the effect of the isolated crude factor on this enzyme was determined. The mean values for percentage of inhibition of debranching enzyme activity by fractions obtained from saliva of CF patients, obligate heterozygotes, and control subjects are: 32% (27 samples), 13% (21 samples), and 6% (11 samples), respectively. Fifteen of the CF values exceed the maximum 13% inhibition observed for controls. Indeed, five samples almost completely inhibited debranching enzyme. This inhibitory action provides the basis of an enzymatic assay for the detection of the ciliostatic CF factor. RF 001 CAUDILL M LANCET 2 307 973 002 BISHOP SH CF CLUB ABST 14 25 973 003 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 004 BOWMAN BH SCIENCE 164 325 969 005 ANON GAP CONF REP CILIARY INHIBITO 2 973 006 DANES BS CLIN GENET 7 128 975 007 DANES BS J EXP MED 136 1313 972 008 DANES BS CLIN GENET 8 85 975 009 DI SANTAGNESE PA N ENGL J MED 277 1287 967 010 DOGGETT RG TEX REP BIOL MED 31 685 973 011 DOGGETT RG NATURE NEW BIOL 243 251 973 012 FISHER EH BIOCHEM PREP 8 27 961 014 KAISER D LANCET 1 1003 970 015 LOWRY OH J BIOL CHEM 193 265 951 016 MANGOS JA SCIENCE 158 135 967 017 MANGOS JA PEDIATR RES 1 436 967 018 MCCOMBS ML TEX REP BIOL MED 31 615 973 019 MCNEELY CM CF CLUB ABST 16 6 975 020 MILLER GL ANAL CHEM 31 964 959 021 NELSON TE CF CLUB ABST 7 974 022 NELSON TE FED PROC 33 1311 974 023 NELSON TE BIOCHEMISTRY 8 1419 969 024 REESE ET CARBOHYD RES 18 381 971 025 ROBYT JF IN: RADLEY JA 431 968 026 SPOCK A PEDIATR RES 1 173 967 027 WATTS TE ANAL BIOCHEM 49 479 972 CT 1 GILLARD BK BIOCHEMISTRY 16 3978 977 2 GILLARD BK CLIN CHEM 23 2279 977 3 GILLARD BK PEDIATR RES 12 868 978 4 IMPERO JE PEDIATR RES 12 108 978 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 7 SEALE TW PEDIATR RES 14 1398 980 8 IMPERO JE PEDIATR RES 15 940 981 9 SEALE TW ANN CLIN LAB SCI 12 415 982 10 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 PN 76182 RN 00536 AN 76197029 AU Thompson-F-E. Quissell-D-O. Williams-C-H. Martinez-J-R. TI The chronically reserpinized rat as a possible animal model for cystic fibrosis. IV. The protein composition of pulmonary lavage fluid. SO Pediatr-Res. 1976 Jun. 10(6). P 632-5. MJ CYSTIC-FIBROSIS: ci. DISEASE-MODELS-ANIMAL. PROTEINS: me. PULMONARY-SURFACTANT: me. RESERPINE. MN ADULT. ANIMAL. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: me. GLYCOPROTEINS: me. HUMAN. IRRIGATION. MALE. MIDDLE-AGE. MOLECULAR-WEIGHT. RATS. AB Lung lavage samples obtained from patients with cystic fibrosis (CF) had significantly higher levels of total protein per ml lavage fluid (0.49 vs. 0.30 mg/ml). A significant increase in the absolute and relative amounts of a low molecular weight glycoprotein (15,000 mol wt) was noted in lavage specimens from CF patients. Reserpine-treated rats also showed a significant increase in the total protein recovered in the lung lavage fluid with a 233% increase in the absolute and relative amounts of a low molecular weight glycoprotein (15,000 mol wt). Thus, reserpine induced changes in the secretions of the lung of the rat which are similar to those observed in samples obtained from the lung of CF patients. RF 001 ANDERSEN DH ANN NY ACAD SCI 93 500 962 002 BARBERO GJ IN: DI SANTAGNESE PA 208 964 003 CHAKRIN LW AM REV RESPIR DIS 108 69 973 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 DI SANTAGNESE PA N ENGL J MED 277 1344 967 006 DI SANTAGNESE PA N ENGL J MED 277 1399 967 007 FENNER C ANAL BIOCHEM 63 595 975 008 FAIRBANKS G BIOCHEMISTRY 10 2606 971 009 IMMKEN L CF CLUB ABST 16 9 975 010 LOWRY OH J BIOL CHEM 193 265 951 011 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 012 MARTINEZ JR PEDIATR RES 9 463 975 013 MARTINEZ JR PEDIATR RES 9 470 975 014 RAMIREZ RJ DIS CHEST 50 581 966 015 WEBER K J BIOL CHEM 244 4406 969 016 WILLIAMS CH CLIN RES 23 533A 975 CT 1 DAVIS PB PEDIATR RES 12 703 978 2 PERLMUTTER J PEDIATR RES 12 188 978 3 SIMSON JAV LAB INVEST 39 157 978 4 MARTINEZ JR PEDIATR RES 13 1156 979 5 MAWHINNEY TP PEDIATR RES 13 760 979 6 SORDELLI DO LIFE SCI 24 2003 979 7 SETSER ME EXP MOL PATH 31 413 979 8 SETSER ME LAB INVEST 41 256 979 9 MAWHINNEY TP PEDIATR RES 14 872 980 10 MORTON D PEDIATR RES 14 18 980 11 MCCURDY RE PEDIATR RES 15 1308 981 12 SNIDER GL MED CLIN NORTH AM 65 647 981 13 FORSTNER J AM J PHYSIOL 241 G443 981 14 CUTLER LS VIRCHOWS ARCH PATHOL ANAT HIS 392 185 981 15 SHIFFMAN ML PEDIATR RES 16 104 982 16 MODRZAKOWSKI MC CAN J MICROBIOL 29 1339 983 17 WERLIN SL EXP MOL PATH 39 24 983 18 BOYD RL PEDIATR RES 18 1028 984 PN 76183 RN 00537 AN 76151240 TI Airline travel for children with chronic pulmonary disease. SO Pediatrics. 1976 Mar. 57(3). P 408-10. MJ AEROSPACE-MEDICINE. CYSTIC-FIBROSIS. LUNG-DISEASES-OBSTRUCTIVE. MN ALTITUDE. ATMOSPHERIC-PRESSURE. CARBON-DIOXIDE: bl. CHILD. CHRONIC-DISEASE. HUMAN. OXYGEN: bl. AB Air travel for children with chronic pulmonary disease is usually safe, but the potential hazards must be considered. The high altitude, obviously, is that which provides the potential problem. Before specifically relating the problem to the children, a few words are in order concerning airline procedures and policies. RF 001 ANON ARCH ENVIRON HEALTH 2 124 961 002 ALTMAN PL HANDBOOK OF RESPIRATION AND C 971 003 HURTADO A J APPL PHYSIOL 1 304 948 004 LENFANT C N ENGL J MED 284 1298 971 CT 1 FRATES RC AM J DIS CHILD 131 687 977 2 CISSIK JH AVIAT SPACE ENVIRON MED 52 312 981 3 WALDMAN JD J AM COLL CARDIOL 2 1158 983 4 GONG H AM REV RESPIR DIS 130 980 984 5 DESMOND KJ CAN MED ASSOC J 131 325 984 PN 76184 RN 00538 AN 76175387 AU Chase-H-P. TI Fatty acids, prostaglandins, and cystic fibrosis. SO Pediatrics. 1976 Apr. 57(4). P 441-2. MJ CYSTIC-FIBROSIS: dt. FATTY-ACIDS: tu. PROSTAGLANDINS: bi. MN CYSTIC-FIBROSIS: me. HUMAN. EX The papers by Elliott and by Campbell et al. suggest a new approach toward etiology, pathophysiology, and therapy in patients with cystic fibrosis. As in Elliott's original report of the effects of intravenous fatty acid therapy for CF, the patients in the present report fared remarkably well, particularly in regard to weight gain. The paper of Campbell et al. suggests that oxygen does not simply diffuse across membranes, but that instead its transport is influenced by the fatty acid composition of the membrane. Elliott's suggestion that the disarranged fatty acid metabolism may be related to altered prostaglandin production is attractive. These studies do not prove whether fatty acid and/or prostaglandin alterations are secondary to intestinal fat malabsorption or are related to the primary metabolic defect in CF. RF 001 ELLIOTT RB PEDIATRICS 57 474 976 002 CAMPBELL IM PEDIATRICS 57 480 976 003 ELLIOTT RB ARCH DIS CHILD 50 76 975 004 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 005 ANON NUTR REV 32 19 974 006 BUCHWALD M AM J HUM GENET 27 22A 975 CT 1 BARNESS LA PEDIATRICS 58 638 976 2 SANJURJO P LANCET 1 752 977 3 ALLUE X PEDIATRICS 61 924 978 4 HAMDI I MONOGR PAEDIATR 10 84 979 5 RUDIN DO MED HYPOTHESES 8 17 982 6 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 7 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 PN 76185 RN 00539 AN 76175395 AU Campbell-I-M. Crozier-D-N. Caton-R-B. TI Abnormal fatty acid composition and impaired oxygen supply in cystic fibrosis patients. SO Pediatrics. 1976 Apr. 57(4). P 480-6. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS: me. OXYGEN: bl. MN CELL-MEMBRANE: ph. CYSTIC-FIBROSIS: bl. ERYTHROCYTES: ul. HEMOGLOBINS: me. HUMAN. LINOLEIC-ACIDS: me. LUNG: pp. OLEIC-ACIDS: me. OXYHEMOGLOBINS: bi. PALMITATES: me. AB Impaired oxygen supply and deteriorating health, in cystic fibrosis patients, correlates with abnormal changes in the fatty acid composition of blood lipids. As the proportion of oleates increases and that of linoleates decreases, erythrocyte membrane interference with the formation of intracellular oxyhemoglobin increases and arterial oxygen pressure decreases. The physical-chemical basis for these changes seems to be that oleic and linoleic acid differ in their ability to undergo reversible oxygenation in response to changes in oxygen pressure. The oxygen complex of linoleic acid dissociates at relatively high pressures, whereas that of oleic dissociates only at low pressures. Accordingly, excessive substitution of oleic for linoleic acid in membrane lipids would be expected to decrease the intracellular oxygen pressure to a level where hemoglobin oxygenation and any other oxygen-requiring processes would be impaired. RF 001 KUO PT J PEDIATR 60 394 962 002 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 003 ROSENLUND ML NATURE 251 719 974 004 CAMPBELL IM CF CLUB ABST 16 974 005 CAMPBELL IM LIPIDS 9 916 974 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 KUKSIS A LIPIDS 2 217 967 008 GODINEZ RI J LIPID RES 14 138 973 009 QUARFORDT SH LIPIDS 8 522 973 010 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 011 DEBEER JJ EUR J BIOCHEM 47 591 974 012 HOLLEY RW PROC NAT ACAD SCI USA 71 3976 974 013 ELLIOTT RB AUST PAEDIATR J 8 217 972 014 ELLIOTT RB PEDIATR RES 7 427 973 015 ELLIOTT RB ARCH DIS CHILD 50 76 975 016 PRESS M LANCET 1 597 974 CT 1 CHASE HP PEDIATRICS 57 441 976 2 HUBBARD VS LANCET 2 1302 977 3 DAVIS PB PEDIATR RES 12 703 978 4 BONTA IL BIOCHEM PHARMACOL 27 1611 978 5 NEUTRA MR GASTROENTEROLOGY 75 701 978 6 LEMEN RJ AM REV RESPIR DIS 117 639 978 7 CAMPBELL IM CAN J BIOCHEM 57 1099 979 8 CHASE HP PEDIATRICS 64 207 979 9 WOOD RE SOUTH MED J 72 189 979 10 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 11 GALEY WR PEDIATR RES 14 1269 980 12 ROGIERS V PEDIATR RES 14 1088 980 13 HUBBARD VS AM J CLIN NUTR 33 2281 980 14 COATES AL ACTA PAEDIATR SCAND 69 353 980 15 DAVIS PB J LAB CLIN MED 96 75 980 16 HUBBARD VS CLIN CHIM ACTA 102 115 980 17 OLLEY PM AM J DIS CHILD 134 688 980 18 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 19 HOLMAN RT AM J CLIN NUTR 34 1534 981 20 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 21 PARK RW GASTROENTEROLOGY 81 1143 981 22 ALLING C EUR J PEDIATR 137 197 981 23 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 24 GASKIN K J PEDIATR 100 857 982 25 MATKOVICS B CLIN CHIM ACTA 125 59 982 26 HUBBARD VS EUR J PEDIATR 141 68 983 27 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 28 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 29 CAMPBELL IM LANCET 2 107 987 30 HUBBARD VS LIPIDS 22 424 987 PN 76186 RN 00540 AN 76175394 AU Elliott-R-B. TI A therapeutic trial of fatty acid supplementation in cystic fibrosis. SO Pediatrics. 1976 Apr. 57(4). P 474-9. MJ CYSTIC-FIBROSIS: dt. FATTY-ACIDS: tu. MN BODY-WEIGHT. CHYMOTRYPSIN: me. CYSTIC-FIBROSIS: en. FECES: en. HUMAN. INFANT. LINOLEIC-ACIDS: tu. SODIUM: me. SWEAT: me. TRYPSIN: me. AB Seven children with cystic fibrosis (CF) have been treated for at least one year with intravenously administered soya oil emulsion. In all, an improvement of at least one biochemical abnormality in character with the disease appeared. The children's clinical course remains benign. This course is remarkably better than that of other children with CF treated without Intralipid in Auckland in the same period, though a placebo effect cannot be discounted. It is postulated that intravenous supplementation with essential fatty acid in CF may in turn partially correct an error of metabolism of prostaglandins present in the disease. RF 001 ELLIOTT RB ARCH DIS CHILD 50 76 975 002 KUO PT J PEDIATR 60 394 962 003 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 005 GIBSON LE PEDIATRICS 23 545 959 006 SCHWARTZ IL J CLIN INVEST 35 114 956 007 ROBINSON PG CLIN CHIM ACTA 62 225 975 008 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 009 MORRISON WR J LIPID RES 5 600 964 010 SHWACHMAN H PEDIATRICS 46 335 970 011 AHLUWALIA B J NUTR 92 205 967 012 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 013 ELLIOTT RB PEDIATR RES 7 427 974 014 ROBINSON GA ANNU REV BIOCHEM 37 149 968 015 HANSEN AE J NUTR 66 565 958 016 SAMUELS CE PROSTAGLANDINS 10 617 975 CT 1 CHASE HP PEDIATRICS 57 441 976 2 SANJURJO P LANCET 1 752 977 3 CROSSLEY JR LANCET 2 1093 977 4 HUBBARD VS LANCET 2 1302 977 5 MCNIFF BL AM J HOSP PHARM 34 1080 977 6 ROSENLUND ML PEDIATRICS 59 428 977 7 CHASE HP LANCET 2 236 978 8 DAVIDSON GP AUST PAEDIATR J 14 80 978 9 PHELAN PD AUST PAEDIATR J 14 61 978 10 BONTA IL BIOCHEM PHARMACOL 27 1611 978 11 ALLUE X PEDIATRICS 61 924 978 12 NEUTRA MR GASTROENTEROLOGY 75 701 978 13 HUNT CE J PEDIATR 92 603 978 14 LEMEN RJ AM REV RESPIR DIS 117 639 978 15 VAUGHAN WJ SCIENCE 199 783 978 16 FRIEDMAN Z SEM PERINATOL 3 341 979 17 BERG U MONOGR PAEDIATR 10 1 979 18 CHASE HP PEDIATRICS 64 207 979 19 LLOYDSTILL JD PEDIATRICS 64 50 979 20 WOOD RE SOUTH MED J 72 189 979 21 CHASE HP J PEDIATR 95 337 979 22 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 23 KARP RJ CLIN CHEST MED 1 375 980 24 ROGIERS V PEDIATR RES 14 1088 980 25 HUBBARD VS AM J CLIN NUTR 33 2281 980 26 COATES AL ACTA PAEDIATR SCAND 69 353 980 27 FRIEDMAN Z AM J DIS CHILD 134 397 980 28 OLLEY PM AM J DIS CHILD 134 688 980 29 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 30 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 31 RIVERS JPW BR MED BULL 37 59 981 32 ALLING C EUR J PEDIATR 137 197 981 33 LEVI N RIV ITAL PEDIATR 8 695 982 34 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 35 GASKIN K J PEDIATR 100 857 982 36 HARPER TB AM REV RESPIR DIS 126 540 982 37 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 38 MERRITT RJ ADV NUTR RES 5 77 983 39 ROGIERS V EUR J PEDIATR 141 39 983 40 LESTER LA J PARENT ENTERAL NUTR 10 289 986 41 MISCHLER EH PEDIATR RES 20 36 986 42 GILLJAM H SCAND J CLIN LAB INVEST 46 511 986 PN 76187 RN 00541 AN 76243835 AU Katz-A-J. Falchuk-Z-M. Schwachman-H. TI The coexistence of cystic fibrosis and celiac disease. SO Pediatrics. 1976 May. 57(5). P 715-21. MJ CELIAC-DISEASE: co. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ALKALINE-PHOSPHATASE: me. CASE-REPORT. CELIAC-DISEASE: fg, pa. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg, pa. DUODENUM: pa. FEMALE. GLIADIN: me. HISTOCOMPATIBILITY-TESTING. HLA-ANTIGENS. HUMAN. MALE. ORGAN-CULTURE. SUPPORT-U-S-GOVT-P-H-S. AB Two patients with cystic fibrosis (CF) who subsequently developed celiac disease (CD) are described. Since organ culture of intestinal mucosa has been used to establish an in vitro model for the study of CD, we utilized this opportunity to determine whether duodenal mucosa obtained from each of these two patients and their immediate families differed in its organ culture behavior from mucosa obtained from patients with CD alone. Additionally, as specific HL-A types are associated with CD, we used HL-A typing to determine whether the two patients with CF-CD differed genetically from patients with CD alone. One of our patients was HL-A8, the most common type associated with CD; the other was HL-A12, as are many of the non-HL-A8 celiac patients. The response in organ culture of the mucosa of these two patients was the same as the response in organ culture of the mucosa from patients with CD alone. These and other data suggest that CD occurring in patients with CF is no different than CD occurring alone. RF 001 GEE S ST BART HOSP REP 24 17 881 002 SHWACHMAN H PEDIATR CLIN NORTH AM 1 389 954 003 GOODCHILD MC ARCH DIS CHILD 48 684 973 004 SHWACHMAN H CF CLUB ABST 4 23 963 005 HIDE DW ARCH DIS CHILD 44 533 969 006 TAYLOR BW ARCH DIS CHILD 48 692 973 007 NORDIO S MINERVA MED 33 967 008 FRANKLIN JP AM J DIG DIS 19 149 974 009 BRANDBORG LL GASTROENTEROLOGY 37 1 959 010 CROSBY WH AM J DIG DIS 2 236 957 011 FALCHUK ZM J CLIN INVEST 51 1602 972 012 MITTAL KK TRANSPLANTATION 6 913 968 013 FALCHUK ZM J CLIN INVEST 53 487 974 014 BROWNING TH J CLIN INVEST 48 1423 969 015 FRAZER AC LANCET 2 252 959 016 DAHLQVIST A ANAL BIOCHEM 22 99 968 017 BESSEY OA J BIOL CHEM 164 321 946 018 LOWRY OH J BIOL CHEM 193 265 951 019 DE GRUCHY GC CLINICAL HAEMATOLOGY IN MEDIC 970 020 ANDERSEN DH ANN NY ACAD SCI 93 500 962 021 TRIER JS N ENGL J MED 283 1245 970 022 FALCHUK ZM IN: HEKKENS WTJM 974 023 MOOG F ANN NY ACAD SCI 166 447 969 024 STROBER W LANCET 2 459 974 025 NELSON DL CLIN RES 22 695A 974 026 FERGUSON R GUT 15 834 974 027 MACDONALD WC N ENGL J MED 272 448 965 028 ANTONOWICZ I PEDIATRICS 42 492 968 029 ANTONOWICZ I PEDIATRICS 49 847 972 CT 1 SAFWENBERG J TISSUE ANTIGENS 10 287 977 2 BERG NO ACTA PAEDIATR SCAND 68 275 979 3 PARK RW GASTROENTEROLOGY 81 1143 981 4 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 5 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 6 RUDDY RM CLIN PEDIATR 26 83 987 PN 76188 RN 00542 AN 76101935 AU Solomons-C. TI Letter: Questions regarding arginine hydrochloride inhalation. SO Pediatrics. 1976 Jan. 57(1). P 166. MJ ARGININE: ad. CYSTIC-FIBROSIS: dt. MN ARGININE: tu. HUMAN. EX With regard to the recent study of Dietzsch et al. on the use of arginine hydrochloride inhalation in cystic fibrosis patients, I wish to point out that these investigators acted irresponsibly in exposing their patients for an appreciable period (seven weeks) to a preparation which is known to be irritating. I would suggest that the deleterious effects seen by Dietzsch et al. are due to their inattention to experimental detail and lack of communication with other investigators. - Dietzsch et al. give the impression in their article that all arginine solution are detrimental to the lung. We wish to point out that L-arginine hydrochloride buffered with a sodium solution is irritative to the airway of cystic fibrosis patients. However, no comparison studies were done with L-arginine buffered with free base. - The fate of these children with cystic fibrosis depends on the quality of the inhalation treatment. This means if you replace a good mucolytic drug with a better one you step by step approach the ideal mucolytic solution. We think it to be unethical to use other, less effective, drugs or solutions in a control group (e.g., saline or distilled water). We did not state that arginine solutions are "irritative" or that arginine treatment is "dangerous." The N-acetylcysteine solution, which was successfully used before the treatment of our patients, was buffered with sodium hydroxide to a pH of 7.0. The quantity of sodium which was administered during each inhalation amounts to only 0.04 mval of sodium so that an injurious pharmacological effect can be excluded. Both drugs were well tolerated and had no "deleterious" effects. RF 001 DIETZSCH HJ PEDIATRICS 55 96 975 002 SOLOMONS CC PEDIATRICS 47 384 971 PN 76189 RN 00543 AN 77035665 AU Shapiro-G-G. Bamman-J. Kanarek-P. Bierman-C-W. TI The paradoxical effect of adrenergic and methylxanthine drugs in cystic fibrosis. SO Pediatrics. 1976 Nov. 58(5). P 740-3. MJ CYSTIC-FIBROSIS: dt. ISOPROTERENOL: tu. THEOPHYLLINE: tu. MN ADMINISTRATION-ORAL. ADOLESCENCE. ADULT. AEROSOLS. CHILD. CLINICAL-TRIALS. COMPARATIVE-STUDY. DOSE-RESPONSE-RELATIONSHIP-DRUG. HUMAN. ISOPROTERENOL: ad, ae. RESPIRATORY-AIRFLOW: de. RESPIRATORY-FUNCTION-TESTS. THEOPHYLLINE: ad, ae. AB This study examined the effect of aerosolized isoproterenol and oral theophylline on pulmonary function in cystic fibrosis (CF) to determine how often these drugs are beneficial and whether there is a correlation between response to isoproterenol and response to oral theophylline. After obtaining baseline pulmonary function tests before and after isoproterenol aerosol, 12 patients with CF were put on a double-blind trial of high-dosage theophylline (10 mg/kg three times per day) or placebo for four weeks and were then switched to the alternate regimen for the next four weeks. Patients had weekly evaluations of pulmonary function and plasma theophylline determinations. Gastrointestinal side effects were common with theophylline. The responses to aerosolized bronchodilators were inconsistent from week to week. After aerosolized bronchodilator, five of 12 patients had an average increase of 10% or more in FEF45%- 55% and FEF70%-80%. While taking oral theophylline, five patients had a 10% or more decrease in FEF 45%-55% and six patients had a decrease in FEF70%-80%. We conclude that there was no concordance between response to isoproterenol and theophylline, and that adrenergic and methylxanthine drugs must be used with caution in patients with CF. RF 001 FEATHERBY EA CAN MED ASSOC J 102 835 970 002 ZAPLETAL A PEDIATRICS 48 64 971 003 LANDAU LI J PEDIATR 82 863 973 004 COOPERMAN EM CAN MED ASSOC J 105 580 971 005 FREEDMAN BJ THORAX 26 46 971 006 BUTTS WC J LAB CLIN MED 84 451 974 007 WEINBERGER MM J PEDIATR 84 421 974 008 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 009 BIERMAN CW ASTHMA IN CHILDHOOD CURRENT T 975 010 POLGAR G PULMONARY FUNCTION TESTING IN 971 CT 1 LARSEN GL AM REV RESPIR DIS 119 399 979 2 ROSCHER AA PEDIATR RES 14 261 980 3 LARSEN GL AM J DIS CHILD 134 1143 980 4 GEORGITIS JW ANN ALLERGY 48 175 982 5 HOWARTH PH ADVER DRUG REAC ACUT POIS REV 2 25 983 6 HENDELES L PHARMACOTHERAPY 3 2 983 7 DAVIS PB J CHRON DIS 36 269 983 8 VALET SB ANN ALLERGY 50 161 983 9 GODFREY S PEDIATRICS 72 517 983 10 DAVIS PB J PEDIATR 102 177 983 11 WEINBERGER M J ALLERGY CLIN IMMUNOL 73 525 984 12 DAVIS PB CHEST 85 802 984 13 KUHN RJ CLIN PHARMACY 4 555 985 14 MISCHLER EH SEM RESPIR MED 6 271 985 15 GODFREY S ARCH DIS CHILD 60 997 985 16 HORDVIK NL AM REV RESPIR DIS 131 889 985 17 ZACH MS AM REV RESPIR DIS 131 537 985 18 DARGA LL PEDIATR PULMONOL 2 82 986 19 KNOWLES MR CLIN CHEST MED 7 285 986 20 SEALE TW PHARMACOL BIOCHEM BEHAV 24 1333 986 PN 76190 RN 00544 AN 76268646 AU Beveridge-J. TI Letter: Intralipid and cystic fibrosis. SO Pediatrics. 1976 Sep. 58(3). P 465. MJ CYSTIC-FIBROSIS: dh. INFANT-FOOD. MN ELECTROLYTES: me. HUMAN. INFANT-NEWBORN. SWEAT: me. EX Stimulated by Professor R. B. Elliott of Auckland,Australia, we have administered Intralipid regularly to a small number of babies in the last two years. The volume used has been 10 ml of the 10% solution per kilogram of body weight. This volume is administered every three weeks, the infusion taking approximately three hours. The four entrants in the trial are all doing well clinically. CT 1 HUBBARD VS LANCET 2 1302 977 2 LLOYDSTILL JD PEDIATRICS 64 50 979 3 ROGIERS V PEDIATR RES 14 1088 980 4 HUBBARD VS AM J CLIN NUTR 33 2281 980 5 MISCHLER EH PEDIATR RES 20 36 986 PN 76192 RN 00545 AN 77012878 AU Hardwick-D-F. Dimmick-J-E. TI Metabolic cirrhoses of infancy and early childhood. SO Perspect-Pediatr-Pathol. 1976. 3. P 103-44. (REVIEW). MJ LIVER-CIRRHOSIS: pa. METABOLISM-INBORN-ERRORS: pa. MN AMINO-ACID-METABOLISM-INBORN-ERRORS: pa. CYSTIC-FIBROSIS: pa. CYSTINOSIS. FRUCTOSE-INTOLERANCE: me, pa. GALACTOSEMIA: di, pa. GENETICS-MEDICAL. GLYCOGENOSIS-4: pa. HEPATOLENTICULAR-DEGENERATION: pa. HUMAN. IATROGENIC-DISEASE. INFANT. INFANT-NEWBORN. KIDNEY-POLYCYSTIC: di. LIPID-METABOLISM-INBORN-ERRORS: pa. LIVER-CIRRHOSIS: cn. LIVER: pa. MUCOPOLYSACCHARIDOSIS: pa. NIEMANN-PICK-DISEASE: pa. REVIEW. TYROSINE: me. EX Cirrhosis of the liver in infancy and early childhood is not a common phenomenon. However, because of the attendant morbidity and frequent mortality, it represents a significant diagnostic and therapeutic problem in this age group. This review will present briefly the metabolic cirrhoses of infancy, discuss currently proposed pathogenetic mechanisms and outline diagnostic techniques that may aid the pathologist in his approach to the problem of metabolic cirrhosis of the very young. Hereditary tyrosinemia, galactosemia, hereditary fructose intolerance, alpha1-antitrypsin deficiency, glycogenesis type IV (amylopectinosis), fibrocystic disease of the pancreas, Niemann-Pick disease, Gaucher's disease, mucopolysaccharidosis (Hurler-Hunter syndrome), Wolman's disease, hemochromatosis, Wilson's disease, cystinosis, the cerebrohepatorenal syndrome of Zellweger, Indian childhood cirrhosis, infantile polycystic disease of the liver and kidneys, congenital hepatic fibrosis, iatrogenic metabolic cirrhoses, and idiopathic congenital cirrhosis are discussed. RF 001 FORBES JG TRANS PATHOL SOC LOND 57 347 906 002 POYNTON FJ ARCH DIS CHILD 1 1 926 003 HARRELL GT AM J DIS CHILD 59 1301 940 004 KELLER PD J PEDIATR 34 588 949 005 CRAIG JM AM J DIS CHILD 90 299 955 006 PEACE R ARCH PATHOL 61 107 956 007 LANDING BH ANN NY ACAD SCI 111 399 963 008 GALL EA AM J CLIN PATHOL 26 1398 956 009 JONES E SOUTH MED J 60 57 967 010 JONES EL MISS MED 66 724 969 011 BUIST NRM IN: NYHAN WL 160 974 012 KENNAWAY NG PEDIATR RES 5 287 971 013 MEDES G BIOCHEM J 26 917 932 014 WOOLF LI IN: GJESSING LR 966 015 SCRIVER CR AM J DIS CHILD 113 41 967 016 GJESSING LR LANCET 2 1132 965 017 FLOYD JS J CLIN INVEST 45 1487 966 018 HARDWICK DF IN: BROWN H 974 019 HARDWICK DF METABOLISM 19 381 970 020 PERRY TL PEDIATRICS 36 236 965 021 GAULL GE PEDIATR RES 4 337 970 022 PERRY TL J MENT DEFIC RES 11 246 967 023 SHINOZUKA H AM J PATHOL 64 241 967 024 COX R LAB INVEST 29 54 973 025 GENTZ J ACTA PAEDIATR SCAND 58 383 969 026 JEVTIC MM AM J CLIN PATHOL 61 413 974 027 PARTINGTON MW CAN MED ASSOC J 97 1059 967 028 GENTZ J SCAND J CLIN LAB INVEST 29 115 972 029 GARROD AE LANCET 2 1 908 030 HSIA DYY J PEDIATR 59 872 961 031 BELL LS ARCH PATHOL 49 393 950 032 SMETANA HF AM J CLIN PATHOL 38 3 962 033 SUZUKI A ARCH PATHOL 82 602 966 034 SHIH VE N ENGL J MED 284 753 971 035 BEUTLER E J LAB CLIN MED 68 646 966 036 GITZELMANN R PEDIATR RES 1 14 967 037 SEGAL S IN: HSIA DYY 969 038 BAKER L J PEDIATR 68 551 966 039 SCHAPIRA F BIOCHEM BIOPHYS RES COMMUN 33 451 969 040 NG WG ANN HUM GENET 37 1 973 041 SIDBURY JB JR HELV PAEDIATR ACTA 14 317 959 042 SIDBURY JB JR IN: HSIA DYY 969 043 NADLER HL IN: HSIA DYY 127 969 044 HILL HZ SCIENCE 179 1136 973 045 FROESCH ER IN: STANBURY JB 131 972 046 SCHWARTZ R J CLIN INVEST 43 333 964 047 JEUNE M PEDIATRIE 16 605 961 048 PERHEENTUPA J ANN PEDIATR (PARIS) 8 221 962 049 LEVIN B AM J MED 45 826 968 050 MAENPAA PH SCIENCE 161 1253 968 051 RAIVIO KD BIOCHEM PHARMACOL 18 2625 969 052 GOLDBATT PJ LAB INVEST 23 378 970 053 PHILLIPS MJ AM J MED 44 910 968 054 YU DT LAB INVEST 31 85 974 055 LAURELL CB SCAND J CLIN LAB INVEST 15 132 963 056 SHARP HL J LAB CLIN MED 73 934 969 057 GANROT PO SCAND J CLIN LAB INVEST 19 205 967 058 TALAMO RC J PEDIATR 79 20 971 059 GLASGOW JFT AM J MED 54 181 973 060 ALPER CA PEDIATRICS 46 937 970 061 JOHNSON AM PEDIATRICS 46 921 970 062 AAGENAES O ACTA PAEDIATR SCAND 61 632 972 063 PORTER CA BR MED J 3 435 972 064 AAGENAES O POSTGRAD MED J 50 365 974 065 COTTRALL K POSTGRAD MED J 50 376 974 066 WILKINSON EJ J PEDIATR 85 159 974 067 FAGERHOL MK CLIN CHIM ACTA 16 199 967 068 KUEPPERS F AM J HUM GENET 25 677 973 069 FAGERHOL MK ACTA PATH MICROBIOL SCAND 70 421 967 070 COOK PJL POSTGRAD MED J 50 362 974 071 DELELLIS RA ARCH PATHOL 94 308 972 072 BELL OF NATURE 243 410 973 073 KUHLENSCHMIDT MS LAB INVEST 31 413 974 074 ERIKSSON S N ENGL J MED 292 176 975 075 ERIKSSON S N ENGL J MED 292 925 975 076 GANS H IN: MITTMAN C 972 077 HUG G IN: GALL EA 973 078 MCADAMS AJ HUM PATHOL 5 463 974 079 ANDERSEN DH LAB INVEST 5 11 956 080 SIDBURY JB JR BULL JOHNS HOPKINS HOSP 111 157 962 081 HOLLEMAN LWJ LAB INVEST 15 357 966 082 REED GB LAB INVEST 19 546 968 083 SCHOCHET SS ARCH PATHOL 90 354 970 084 CORI GT HARVEY LECTURES 48 145 953 085 BROWN BI PROC NAT ACAD SCI USA 56 725 966 086 SIDBURY JB JR PEDIATRICS 27 103 961 087 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 088 CRAIG JM AM J DIS CHILD 93 357 957 089 OPPENHEIMER EH J PEDIATR 86 683 975 090 CLAIREAUX AE ARCH DIS CHILD 31 22 956 091 BERNSTEIN JG AM J DIS CHILD 99 804 960 092 OPPENHEIMER EH ARCH PATHOL 96 149 973 093 COOPER HS JOHNS HOPKINS MED J 135 268 974 094 FARBER S ARCH PATHOL 37 238 944 095 VALMAN HB ARCH DIS CHILD 46 805 971 096 LANDING BH IN: GALL EA 973 097 WEBSTER R ARCH DIS CHILD 28 343 953 098 DI SANTAGNESE PA PEDIATRICS 18 387 956 099 CROCKER AC MEDICINE (BALTIMORE) 37 1 958 100 BLOOM W AM J PATHOL 1 595 925 101 PICK L AM J MED SCI 185 601 933 102 IVEMARK BI ACTA PAEDIATR SCAND 52 391 963 103 FREDRICKSON DS IN: STANBURY JB 783 972 104 LANDING BH PROG PEDIATR SURG 6 113 974 105 LANDING BH AM J PATHOL 33 1 957 106 BERNSTEIN JG J PEDIATR 55 577 959 107 DRUKKER A PEDIATRICS 45 1017 970 108 GINSBURG SJ J PEDIATR 82 1046 973 109 SCHNEIDER EL J PEDIATR 81 1134 972 110 RODGERS CL PEDIATRICS 7 53 951 111 JAVETT SN J PEDIATR 68 810 966 112 PICK L AM J MED SCI 185 453 933 113 STRAUS R AM J CLIN PATHOL 17 761 947 114 STRAUSS L AM J PATHOL 24 855 948 115 LINDSAY S AM J DIS CHILD 76 239 948 116 HENDERSON JL ARCH DIS CHILD 27 230 952 117 HAUST MD J HISTOCHEM CYTOCHEM 9 79 961 118 SCHWARZ H CAN MED ASSOC J 66 375 952 119 ABRAMOV A AM J DIS CHILD 91 282 956 120 WOLMAN M PEDIATRICS 28 742 961 121 CROCKER AC PEDIATRICS 35 627 965 122 MARSHALL WC ARCH DIS CHILD 44 331 969 123 LOUGH J ARCH PATHOL 89 103 970 124 PATRICK AD NATURE 222 1067 969 125 PERKIN KW AM J MED 39 118 965 126 FEINBERG R J CLIN PATHOL 33 480 960 127 WILSON SAK BRAIN 34 2925 912 128 LEVI AJ LANCET 2 575 967 129 STRICKLAND GT Q J MED 42 619 973 130 ANDERSON PJ AM J PATHOL 36 483 960 131 MCINTYRE N N ENGL J MED 276 439 967 132 REED GB ARCH PATHOL 93 249 972 134 STILL WJS ARCH DIS CHILD 30 354 955 135 BICKEL H ACTA PAEDIATR SCAND SUPPL 90 1 952 136 KING WB JR STANFORD MED BULL 10 162 962 137 PASSARGE E J PEDIATR 71 691 967 138 PUNNETT HH J PEDIATR 73 602 968 139 SMITH DW J PEDIATR 67 617 965 140 JAN JE AM J DIS CHILD 119 274 970 141 PATTON RG AM J DIS CHILD 124 840 972 142 DANKS DM J PEDIATR 86 382 975 143 VITALE L N ENGL J MED 280 642 969 144 GOLDFISCHER S SCIENCE 182 62 973 145 VOLPE JJ ACTA NEUROPATH (BERL) 20 175 972 146 BERNSTEIN J BIRTH DEF ORIG ART SER 10 35 974 147 SINGH A LANCET 1 587 961 148 SMETANA HF PEDIATRICS 28 107 961 149 NATAK NC INDIAN J MED RES 60 246 972 150 KERR DNS Q J MED 30 91 961 151 MURRAY-LYON IM GASTROENTEROLOGY 64 653 973 152 BLYTH H J MED GENET 8 257 971 153 LIEBERMAN E MEDICINE (BALTIMORE) 50 277 971 154 IVEMARK BI ACTA PAEDIATR SCAND 48 1 959 155 PARTIN JC GASTROENTEROLOGY 67 107 974 156 PEDEN VH J PEDIATR 78 180 971 157 WIGGER JH CITED IN: J PEDIATR 81 145 972 158 DRISCOLL JM JR J PEDIATR 81 145 972 159 ROGER R J PEDIATR 86 264 975 160 THALER MM PEDIATRICS 33 721 964 161 ALPERT LI N ENGL J MED 280 16 969 162 STRAUSS L BILIARY TRACT ANOMALIES THE R 972 163 KURNICK H HUM PATHOL 5 223 974 164 HARDWICK DF AM J PATHOL 62 56 971 165 COEN R AM J DIS CHILD 119 383 970 166 WEINBERG AG AM J DIS CHILD 119 290 970 167 SHIRAKI K AM J DIS CHILD 119 315 970 168 POPPER H GASTROENTEROLOGY 4 315 975 169 RAPPAPORT AM MICROVASC RES 6 212 973 CT 1 FISCH RO J PEDIATR 93 592 978 2 RUSHTON DI DIAGN HISTOPATHOL 4 17 981 3 MOWAT AP CLIN GASTROENTEROL 11 171 982 4 SPIVAK W J PEDIATR GASTROENTEROL NUTR 2 381 983 5 DENNEHY PH J PEDIATR 106 161 985 6 BLISARD KS HUM PATHOL 17 376 986 7 AGAMANOLIS DP NEUROLOGY 36 674 986 8 ZIMMERMANN A ARCH PATHOL LAB MED 110 136 986 PN 76193 RN 00546 AN 76269924 AU Tecklin-J-S. Holsclaw-D-S-Jr. TI Bronchial drainage with aerosol medications in cystic fibrosis. SO Phys-Ther. 1976 Sep. 56(9). P 999-1003. MJ ACETYLCYSTEINE: tu. CYSTIC-FIBROSIS: th. DRAINAGE. ISOPROTERENOL: tu. MN ADOLESCENCE. ADULT. AEROSOLS. BRONCHI: de, se. CHILD. DRUG-EVALUATION. DRUG-THERAPY-COMBINATION. HUMAN. RESPIRATORY-FUNCTION-TESTS. SUPPORT-U-S-GOVT-P-H-S. AB Examined in this study are the immediate effects of bronchial drainage preceded by the inhalation of aerosol solutions of a mucolytic agent, a bronchodilator, and their combination upon four measures of pulmonary function in 20 patients with cystic fibrosis. On three separate occasions, pulmonary function was tested preceding and following treatments consisting of one of the above three aerosol solutions followed by bronchial drainage. The order of solutions used was random and each subject received one treatment with each solution. The greatest overall improvement in function was seen with the bronchodilator and bronchial drainage. The combination of the bronchodilator/mucolytic agents and bronchial drainage was the next most effective technique. The mucolytic agent, when used alone with bronchial drainage, decreased function. Comparison of the pulmonary function scores indicated a significant difference among the three treatments. RF 001 ANON AM REV RESPIR DIS PART 2 110 1 974 002 TECKLIN JS PHYS THER 55 1081 975 003 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 004 LORIN MI AM J PHYS MED 50 215 971 005 MOTOYAMA EK IN: MANGOS JA 335 973 006 REAS HW J PEDIATR 65 542 964 007 STAMM SJ DIS CHEST 47 414 965 008 FEATHERBY EA CAN MED ASSOC J 102 835 970 009 CHANG N AM REV RESPIR DIS 106 867 972 010 ALLEN GW AM REV RESPIR DIS 104 61 971 011 MITCHELL M AM REV RESPIR DIS 96 685 967 012 HIRSCH SR THORAX 25 737 970 CT 1 TECKLIN JS AM REV RESPIR DIS 114 1038 976 2 DREISIN RB J LAB CLIN MED 93 674 979 3 TABACHNIK E AM REV RESPIR DIS 122 97 980 PN 76194 RN 00547 AN 76176175 AU Mattimore-J-M. TI Differentiating the cause of wheezing in infants. SO Postgrad-Med. 1976 Apr. 59(4). P 116-22. MJ AUSCULTATION. LUNG-NEOPLASMS: di. RESPIRATION. MN AORTA-THORACIC: ab. BRONCHI. ESOPHAGEAL-ACHALASIA: di. CASE-REPORT. CYSTIC-FIBROSIS: di. CYSTS: cn, di. DIAGNOSIS-DIFFERENTIAL. ESOPHAGEAL-NEOPLASMS: di. ESOPHAGUS. FEMALE. FOREIGN-BODIES: di. HEART-DEFECTS-CONGENITAL: di. HUMAN. INFANT. INFANT-NEWBORN. LIPOMA: di. LUNG-NEOPLASMS: cn. MALE. RESPIRATORY-HYPERSENSITIVITY: di. RESPIRATORY-TRACT-DISEASES: di. RESPIRATORY-TRACT-INFECTIONS: di. TRACHEOESOPHAGEAL-FISTULA: di. VIRUS-DISEASES: di. EX It cannot be emphasized too strongly that all infants who wheeze do not have asthma. Because of the life-threatening consequences of failure to detect some of the clinical entities that present with wheezing, the clinician should thoroughly investigate the infant who wheezes. Cases illustrating some causes of wheezing, additional causes of wheezing, and approaches to diagnosis are discussed. RF 001 GERRARD JW ACTA PAEDIATR SCAND SUPPL 234 1 973 002 MCINTOSH K J PEDIATR 82 578 973 003 HOGG JC N ENGL J MED 282 1283 970 004 HILMAN BC ANN ALLERGY 25 622 967 005 DEES SC JAMA 175 365 961 006 MUSTARD WT IN: BENSON CD 1 962 007 BROOKS JW IN: KENDIG EL JR 1 972 008 SHAHIDI NT J PEDIATR 59 533 961 009 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 010 AMMANN AJ RECURRENT INFECTION IN HYPERS 975 011 KATZ RM N ENGL J MED 288 233 973 012 LANG WR BR MED J 1 73 969 013 BECROFT DMO J CLIN PATHOL 24 72 971 014 CUMMING GR J PEDIATR 78 250 971 015 POLMAR SH PEDIATRICS 50 279 972 PN 76195 RN 00548 AN 76269694 AU Buchwald-M. TI Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis. SO Proc-Natl-Acad-Sci-USA. 1976 Aug. 73(8). P 2899-903. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: me. CYSTIC-FIBROSIS: me. ISOPROTERENOL: pd. SKIN: me. MN AGE-FACTORS. CELL-DIVISION. CELLS-CULTURED. DOSE-RESPONSE-RELATIONSHIP-DRUG. FIBROBLASTS. HUMAN. RECEPTORS-ADRENERGIC. TIME-FACTORS. AB To determine if the abnormalities of exocrine secretion characteristic of cystic fibrosis could be investigated in vitro, I studied the synthesis of 3':5'-cyclic AMP after isoproterenol stimulation in skin fibroblasts derived from patients with cystic fibrosis and from normal individuals. Comparison of normal and cystic fibrosis cells showed that the latter had 2- to 5-fold greater levels of intracellular cyclic AMP after stimulation with isoproterenol. The difference between the strains was observed at every stage of the culture cycle and was specific to stimulation by beta-adrenergic agonists. It could not be accounted for by different dose- or time- response curves nor by leakage of cyclic AMP into the medium. The increased sensitivity to catecholamines may reflect an intrinsic genetic property of cystic fibrosis cells, and it may be feasible to use this system to study the biochemical basis of the genetic defect in cystic fibrosis. RF 001 MCKUSICK VA MENDELIAN INHERIT IN MAN 975 002 DANES BS J EXP MED 129 775 969 003 DANES BS J EXP MED 136 1313 972 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 BERATIS NG PEDIATR RES 7 958 973 006 CONOVER JH PEDIATR RES 7 224 973 007 SPOCK A PEDIATR RES 1 173 967 008 BOWMAN BH SCIENCE 164 325 969 009 ANTONOWICZ I PEDIATR RES 6 803 972 010 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 011 PALLAVICINI JC J PEDIATR 77 280 970 012 DANES BS BIOCHEM GENET 12 359 974 013 BARTMAN J J PEDIATR 76 430 970 014 CHERNICK WS J PEDIATR 65 694 964 015 BLOMFIELD J GUT 14 558 973 016 BLOMFIELD J AUST PAEDIATR J 10 75 974 017 BARBERO GJ IN: DI SANTAGNESE PA 208 964 018 SEIFERT G IN: SCHNEYER LH 967 019 MARTINEZ JR PEDIATR RES 9 470 975 020 SCHRAMM M IN: MANGOS JA 215 973 021 ROBISON GA CYCLIC AMP 971 022 RAO GJS NATURE NEW BIOL 232 253 971 023 FRANKLIN TJ NATURE NEW BIOL 246 119 973 024 HASLAM RJ BIOCHEM J 144 253 974 025 KELLY LA J BIOL CHEM 249 3098 974 026 MANGANIELLO VC BIOCHIM BIOPHYS ACTA 362 509 974 027 FRANKLIN TJ MOL PHARMACOL 11 485 975 028 GOLDSTEIN S DIABETES 18 545 969 029 STANNERS CP NATURE NEW BIOL 230 52 971 030 GILMAN AG PROC NAT ACAD SCI USA 67 305 970 031 GILMAN AG J BIOL CHEM 248 6610 973 032 SHEPPARD H BIOCHEM PHARMACOL 20 2128 971 033 LOWRY OH J BIOL CHEM 193 265 951 034 FURCHGOTT RF FED PROC 29 1352 970 CT 1 DAVIDSON GP J CLIN INVEST 60 1402 977 2 EPSTEIN J PROC NAT ACAD SCI USA 74 1676 977 3 EPSTEIN J SOMATIC CELL GENET 4 451 978 4 DAVIS PB PEDIATR RES 12 703 978 5 WRIGHT RK PEDIATR RES 12 830 978 6 ERICKSON RP AM J HUM GENET 30 A 28 978 7 ROSCHER A MONOGR PAEDIATR 10 77 979 8 HOSLI P CLIN GENET 15 487 979 9 GNEGY ME AM J HUM GENET 31 A 48 979 10 PECKER F CLIN SCI 57 313 979 11 JAKEL HP BIOL ZENTRALBL 98 55 979 12 KURZ JB SCIENCE 206 1317 979 13 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 14 DAVIS PB PEDIATR RES 14 863 980 15 ROSCHER AA PEDIATR RES 14 261 980 16 AITKEN DA J MED GENET 17 187 980 17 KURZ JB PEDIATR RES 15 1328 981 18 GNEGY ME BIOCHEM MED 26 294 981 19 MARKOVAC J BIOCHEM MED 26 299 981 20 GALANT SP J CLIN INVEST 68 253 981 21 MCSWIGAN JD PROC NAT ACAD SCI USA 78 7670 981 22 MAPLESON JL J CELL PHYSIOL 109 215 981 23 ROSCHER AA J PHARMACOL EXP THER 216 419 981 24 CEDER O SCANN ELECTRON MICROSC 1982 723 982 25 BUCHWALD M CLIN BIOCHEM 17 143 984 26 VOLICER L NEUROBIOL AGING 6 35 985 27 KOPELMAN H N ENGL J MED 312 329 985 28 HONEGGER UE BIOCHEM PHARMACOL 35 1899 986 PN 76196 RN 00549 AN 76198413 AU Seeff-L-B. Zimmerman-H-J. TI Relationship between hepatic and pancreatic disease. SO Prog-Liver-Dis. 1976. 5. P 590-608. (REVIEW). MJ LIVER-DISEASES: co. PANCREATIC-DISEASES: co. MN ASCITES: et. CYSTIC-FIBROSIS: co. DIABETES-MELLITUS: co. DRUG-THERAPY: ae. GRANULOMA: co. HEMOCHROMATOSIS: co. HEPATITIS-A: co. HUMAN. KWASHIORKOR: co. LIVER-CIRRHOSIS: co, et. LIVER-DISEASES: et. MYCOSES: co. PANCREATIC-DISEASES: et. PANCREATITIS: co. PARASITIC-DISEASES: co. REVIEW. VIRUS-DISEASES: co. AB A voluminous literature has dealt with the variety of pathologic entities that may effect the liver and the pancreas. Much of it includes poorly or vaguely documented observations. Only a few facets have been subjected to experimental study. Despite the resultant difficulty in unraveling reported and inferred relationships between the two organs, the available information has been analyzed according to the following arbitrary categorization: (1) involvement of the liver in pancreatic disease, (2) involvement of the pancreas in hepatic disease, (3) involvement of both organs in systemic disease. RF 001 STEIGMANN F AM J GASTROENTEROL 33 454 960 002 MORRELL MT BR J SURG 57 814 970 003 LLOYD-JONES W ANN SURG 176 163 972 004 COMFORT MW GASTROENTEROLOGY 21 54 952 005 MCELROY R AM J MED 52 228 972 006 LOBECK CC IN: STANBURY JB 1300 966 007 CRAIG JM AM J DIS CHILD 93 357 957 008 DI SANTAGNESE PA PEDIATRICS 18 387 956 009 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 010 FARBER S ARCH PATHOL 37 238 944 011 WEBSTER R ARCH DIS CHILD 28 343 953 012 GATZIMOS CD AM J DIS CHILD 89 182 955 013 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 014 KATTWINKEL J J PEDIATR 82 234 973 015 ANDERSON C ARCH DIS CHILD 31 31 956 016 STEIN AA ANN SURG 157 516 963 017 LONGNECKER DS ARCH PATHOL 80 148 965 018 TRUHART H PATHOLOGIE PT 1 902 019 ELOESSER L MITT GRENZGEB D MED U CHIR 18 195 907 020 SCHILLER W SURG GYNECOL OBSTET 72 70 941 021 MACKENZIE WC ANN R COLL SURG ENGL 15 220 954 022 SUSSMAN HM AM J GASTROENTEROL 50 315 968 023 COMFORT MW GASTROENTEROLOGY 6 239 946 024 PHILLIPS AM ARCH INTERN MED 93 337 954 025 SANES J ARCH INTERN MED 85 980 950 026 FISHER ER SURGERY 37 213 955 027 DOS REIS L ARCH SURG 87 604 963 028 ANDERSON MC ARCH SURG 92 664 966 029 KITAMURA O AM J SURG 126 379 973 030 RUSH B JR SURGERY 31 349 952 031 THAL AP AM J SURG 105 708 963 032$ LUKASH WM JAMA 191 927 966 033 FOULK WT GASTROENTEROLOGY 35 375 958 034 BUTLER ML SOUTH MED J 66 700 973 035 WEINSTEIN BR ANN INTERN MED 58 245 963 036 FRIEDEN JH ARCH SURG 90 422 965 037 LEGER L NOUV PRESSE MED 33 2159 972 038 EDMONDSON HA AM J PATHOL 26 37 950 039 WOLDMAN EE JAMA 169 1281 959 040 COLE WH SURGERY 8 19 940 041 OWOR R AFR J MED SCI 3 205 972 042 MASIH B NY STATE J MED 71 2312 971 043 SUMMERSKILL WHJ LANCET 2 686 959 044 TYGSTRUP N ACTA MED SCAND 185 523 969 045 SALAZAR FA MED ANN DC 43 533 974 046 YALE CE JAMA 217 317 971 047 SUTTON JP ARCH SURG 100 623 970 048 LONGSTRETH GF ANN INTERN MED 75 903 971 049 RIGNAULT D SURGERY 63 571 968 050 GREGORY PB GASTROENTEROLOGY 62 436 972 051 BRAGANZA JM CLIN GASTROENTEROL 1 219 972 052 DONOWITZ M MEDICINE (BALTIMORE) 53 183 974 053 CREUTZFELDT W IN: POPPER H 3 371 970 054 VON MERING J ARCH J EXP PATHOL PHARMACOL 26 371 889 055 ALLAN FN BR J EXP PATHOL 5 75 924 056 HERSHEY JM AM J PHYSIOL 98 74 931 057 BEST CH J PHYSIOL (LOND) 79 94 933 058 DRAGSTEDT LR AM J PHYSIOL 117 175 936 059 CLOWES GHA JR AM J PHYSIOL 165 628 951 060 ZIMMERMAN HJ J LAB CLIN MED 36 922 950 061 LUCAS CC IN: HOLMAN RT 10 1 967 062 YOUNG FG LANCET 2 372 937 064 MAURIAC P PARIS MED 2 525 935 065 ZIMMERMAN HJ J LAB CLIN MED 36 912 950 066 DROLLER H BR MED J 1 623 945 067 WELLER E DTSCH MED WSCHR 74 1223 949 068 SHERWIN R N ENGL J MED 290 239 974 069 ZIMMERMAN HJ IN: SAMTER M 340 972 070 ERBER WF GUT 13 996 972 071 LISNEY AA PROC R SOC MED 37 165 943 072 JOSKE RA ROY MELBOURNE HOSP CLIN REP 25 20 955 073 ACHORD JL JAMA 205 129 968 074 HAM JM AM J DIG DIS 18 1079 973 075 GEOKAS MC CALIF MED 117 1 972 076 PARBHOO SP GUT 14 428 973 077 WANDS JR JOHNS HOPKINS MED J 133 156 973 078 FRANZINI C J CLIN PATHOL 18 775 965 079 LAVERDANT C ANN MED INTERNE PARIS 122 343 971 080 CONRAD ME AM J GASTROENTEROL 42 47 964 081 RAKE MO LANCET 1 533 970 082 CRANDALL LA ARCH NEUROL PSYCHIATR 27 367 932 083 JOHNSON TA ARCH INTERN MED 66 62 940 085 STINSON JC JR AM J CLIN PATHOL 22 117 952 086 NETIK J BULL MEM ECOLE NAT MED PHARM 5 412 957 087 STEIGMANN F AM J GASTROENTEROL 38 40 962 088 SOBEL JH GASTROENTEROLOGY 45 341 963 089 BUTT EM AM J CLIN PATHOL 42 437 964 090 VAN GOIDSENHOVEN GE AM J DIG DIS 8 160 963 091 ZIEVE L AM J DIG DIS 12 303 967 092 FERRI S RIV GASTROENTEROL 20 244 968 093 DAVIS AE MED J AUST 1 508 969 094 GOEBELL H DTSCH MED WSCHR 95 808 970 095 WILLIAMS CN CAN MED ASSOC J 105 1143 971 096 SARLES H GASTROENTEROLOGY 66 604 974 097 SOLOMON N AM J DIG DIS 19 253 974 098 MYHRE J J LAB CLIN MED 34 1671 949 099 WISLOSKI LR N ENGL J MED 275 322 966 100 TILNEY NL N ENGL J MED 274 1051 966 101 PAPPENHEIMER AM J EXP MED 94 45 951 102 OBRIEN PK BR MED J 2 1529 965 103 CRAIGHEAD JE AM J PATHOL 48 375 966 104 PLATT H VIROLOGY 9 484 959 105 SERRANO-RIOS M DIGESTION 2 262 969 106 MOTT CB AM J DIG DIS 17 583 972 107 LOUW JH BR J SURG 53 510 966 108 KLATSKIN G YALE J BIOL MED 23 207 950 109 CHAUN H AM J DIG DIS 17 725 972 110 CALVIN C PRESSE MED 79 2230 971 111 NIDUS BD ANN INTERN MED 63 663 965 112 BARRETT PVD N ENGL J MED 268 36 963 113 BLOCK MB N ENGL J MED 282 380 970 114 GUERRA M JAMA 200 552 967 115 PINCUS IJ IN: BOCKUS HL 960 965 116 CORNISH AL N ENGL J MED 265 673 961 117 NOGUEIRA JR GASTROENTEROLOGY 62 1040 972 118 FARBER E PROC SOC EXP BIOL MED 74 838 950 119 SCHRIER RW JAMA 194 564 965 120 ZUBROD CG PEDIATRICS 45 555 970 121 WHALLEY PJ JAMA 189 357 964 122 VILLA-TREVINO S J BIOL CHEM 238 1757 963 123 HATFIELD AK AM J DIG DIS 17 167 972 124 DAVIES JNP LANCET 1 317 948 125 TROISIER BULL SOC ANAT PARIS 44 231 871 126 GRACE ND GASTROENTEROLOGY 64 1257 974 127 SHELCON JH HEMOCHROMATOSIS 935 128 POWELL LW GASTROENTEROLOGY 64 889 973 129 POWELL LW NATURE 250 333 974 130 MACDONALD RG HEMOCHROMATOSIS AND HEMOSIDER 964 131 PURTILO DT CANCER 32 458 973 132 BECKER D N ENGL J MED 263 367 960 133 DYMOCK IW AM J MED 52 203 972 134 CHARLTON RW BR MED J 2 1427 964 135 TAPPER EJ AM J MED SCI 255 46 968 136 BALCERZAK SP GASTROENTEROLOGY 53 257 967 137 CONN HO GASTROENTEROLOGY 62 61 972 138 KOFF RS GASTROENTEROLOGY 63 834 972 139 POUNDER RE PROC R SOC MED 67 323 974 140 WILK PJ AM J GASTROENTEROL 60 273 973 141 LONGMIRE WP AM J SURG 125 661 972 CT 1 GRANT DAW BIOCHEM J 198 315 981 2 BATMAN PA HISTOPATHOLOGY 9 237 985 3 CASTELLANO G REV ESP ENFERM APAR DIG 68 67 985 4 GRANT D INT J PANCREATOL 1 167 986 PN 76197 RN 00550 AN 76245035 AU Mitchell-Heggs-P. Mearns-M. Batten-J-C. TI Cystic fibrosis in adolescents and adults. SO Q-J-Med. 1976 Jul. 45(179). P 479-504. MJ CYSTIC-FIBROSIS: pp. MN ADOLESCENCE. ADULT. ASPERGILLUS: ip. CYSTIC-FIBROSIS: co. FEMALE. GASTROINTESTINAL-DISEASES: co. GROWTH. HUMAN. LUNG-DISEASES: co. MALE. PSEUDOMONAS-AERUGINOSA: ip. PUBERTY. RESPIRATION. SODIUM: an. SPUTUM: mi. STAPHYLOCOCCUS: ip. SWEAT: an. AB Forty-five patients (25 male and 20 female) over 12 years of age with cystic fibrosis have been studied clinically, radiologically and physiologically. Their mean age at the first visit was 17 years; they were followed for a mean period of 4 years and attended at least every six months. The first symptom which developed before the age of five in 42 of the 45 patients was respiratory. Thirty-two of the 45 patients had severe lung disease (Group III) at the start of the study of the seven patients died during the study. Cough and sputum were almost universal, 23 had haemoptyses and eight pneumothoraces. Staphylococcus pyogenes, Haemophilus influenzae and Pseudomonas aeruginosa were the common pathogens isolated from sputum and the increasing prevalence of the latter was again confirmed. Acquisition of the mucoid strain of pseudomonas signified poor prognosis. Established infection was never eradicated. Forty-three patients had evidence of pancreatic insufficiency; in all but one patient the symptoms were mild and five patients abandoned dietary restriction and pancreatin without ill effect. Seven patients had symptoms of partial bowel obstruction (meconium ileus equivalent) but only one required surgical relief. The liver was enlarged in seven patients and the spleen was felt in three. Three patients had diabetes mellitus. The influence of cystic fibrosis on growth and development is reported--the growth spurt is late in the majority but growth failure is not confined to those with severe lung infection or malabsorption and in these circumstances remains unexplained. Mean weight was low in relation to height and puberty was delayed in both sexes. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH J CHRON DIS 7 58 958 003 ANDERSON CM ARCH DIS CHILD 35 581 960 004 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 005 BOXERBAUM B AM REV RESPIR DIS 108 777 973 006 ANON BR MED J 4 310 974 007 BURNS MW LANCET 1 270 968 008 BURTON L PRACTITIONER 210 247 973 009 CATTANEO SM J THORAC CARDIOVASC SURG 66 467 973 010 CROFTON J RESPIRATORY DISEASES 969 011 DANES BS J EXP MED 129 775 969 012 DENNING CR PEDIATRICS 41 7 968 013 DI SANTAGNESE PA PEDIATRICS 18 387 956 014 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 015 FEATHERBY EA IN: LAWSON D PROC 5TH INT CF 232 969 016 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 017 FEIGELSON J NOUV PRESSE MED 1 1899 972 018 GODFREY S ARCH DIS CHILD 46 144 971 019 GRACEY M ARCH DIS CHILD 44 404 969 020 GRACEY M AUSTRALAS ANN MED 18 91 969 021 GRAND RJ JAMA 195 993 966 022 GOODCHILD MC ARCH DIS CHILD 48 684 973 023 GREEN OC J CLIN ENDOCRINOL METAB 27 1059 967 024 HODSON CJ CLIN RADIOL 13 54 962 025 HOLSCLAW DS J PEDIATR 76 829 970 026 HUANG NN PEDIATR RES 7 428 973 027 KAPLAN E N ENGL J MED 279 65 968 028 KATTWINKEL J JAMA 226 557 973 029 KREISSL T Z KINDERHEILK 113 93 972 030 LANDAU LI J PEDIATR 82 863 973 031 LAPEY A J PEDIATR 84 328 974 032 LIFSCHITZ MI AM J DIS CHILD 116 633 968 033 MARSHALL WA ARCH DIS CHILD 45 13 970 034 MAY JR ARCH DIS CHILD 47 908 972 035 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 036 MCCRAE WM SCOTT MED J 19 187 974 037 MCINTOSH R ACTA PAEDIATR SCAND SUPPL 100 43 469 954 038 MCPARTLIN JF BR J SURG 60 707 973 039 MEARNS MB LANCET 1 538 967 040 MEARNS MB ARCH DIS CHILD 47 902 972 041 MEARNS MB THORAX 28 537 973 042 MEARNS MB IN: MANGOS JA 345 973 043 MILNER AD ARCH DIS CHILD 44 351 969 044 MITCHELL-HEGGS PF THORAX 25 165 970 045 NATH AP THORAX 29 223 974 046 REID L IN: MANGOS JA 195 973 047 ROSAN RC AM J DIS CHILD 104 625 962 048 ROSS CAC ARCH DIS CHILD 30 428 955 049 ROVSING H ACTA RADIOL DIAGN STOCKH 14 588 973 050 SIEGENTHALER P JAMA 186 1178 963 051 SHWACHMAN H PEDIATRICS 36 689 965 052 SPROUL A J PEDIATR 65 664 964 053 TANNER JM STANDARDS FOR SKELETAL AGE 972 054 TANNER JM ARCH DIS CHILD 41 454 966 055 TAYLOR BW ARCH DIS CHILD 48 692 973 056 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 057 WARREN CPW CLIN ALLERGY 5 1 975 058 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 CT 1 ANON LANCET 2 614 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 SEATON A SCOTT MED J 22 99 977 4 RAEBURN JA PROC NUTR SOC 36 77 977 5 SAKULA A BR J DIS CHEST 71 295 977 6 OWEN E J MOL MED 3 49 978 7 STERN RC AM REV RESPIR DIS 118 821 978 8 ANON BR MED J 2 626 979 9 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 10 SANTAGNESE PAD AM J MED 66 121 979 11 ALLAN JL MED J AUST 1 600 980 12 HOLSCLAW DS CLIN CHEST MED 1 407 980 13 HODSON ME THORAX 35 801 980 14 ORMEROD LP THORAX 35 768 980 15 BRUETON MJ ARCH DIS CHILD 55 348 980 16 FICK RB CLIN CHEST MED 2 91 981 17 CHO YW J CLIN PHARMACOL 21 224 981 18 CONGDEN PJ ARCH DIS CHILD 56 708 981 19 SCHWARTZ RH J ALLERGY CLIN IMMUNOL 68 290 981 20 JACOBS WH AM J GASTROENTEROL 76 342 981 21 REITER EO AM J DIS CHILD 135 422 981 22 REITER EO CLIN ENDOCRINOL 16 127 982 23 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 24 SHAWKER TH J ULTRASOUND MED 2 439 983 25 NEVILLE E THORAX 38 929 983 26 GUIDOTTI TL RESPIRATION 44 351 983 27 HODSON ME POSTGRAD MED J 60 225 984 28 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 29 LESTER LA SEM RESPIR MED 6 285 985 30 SEALE TW ANN CLIN LAB SCI 15 152 985 31 HANLY JG Q J MED 56 377 985 32 BERKIN KE EUR J RESPIR DIS 67 103 985 33 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 34 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 35 PREECE MA CLIN ENDOCRINOL METAB 15 453 986 36 MAHANEY MC ARCH ORAL BIOL 31 363 986 37 MAHANEY MC HUM BIOL 58 445 986 38 STEAD RJ CLIN ENDOCRINOL 26 187 987 PN 76198 RN 00551 AN 77150040 AU Dillman-D-E. TI Cystic fibrosis: a new challenge for the patient-care team. SO Respir-Ther. 1976 May-Jun. 6(3). P 25-7. MJ CYSTIC-FIBROSIS: th. RESPIRATORY-THERAPY. PATIENT-CARE-TEAM. MN HUMAN. AB To date, most cases of cystic fibrosis have been treated in pediatric hospitals. With changes and advances in treatment, patients who were once considered terminally ill are now living into adulthood. The stresses that result from chronic illness are considered herein from the standpoint of the social caseworker, who plans a crucial role on the patient-care team. New problems regarding the personal development of the patient are presented, with particular emphasis on the role of a CF patient as a parent. The role of the respiratory therapist in the care of such patients is unique. This is due to the extensiveness of patient contact, both verbal and physical, and to the primary threat that pulmonary disease poses to the patient with CF. The therapist's ability to cope with the problem, while aiding the family and patient to do the same, becomes crucial to the child's rehabilitation. The requirements for fulfilling this role are educational and developmental in nature, and should be pursued as a model for future patient care. PN 76199 RN 00552 AN 77015073 AU Paster-S-B. TI Nasal polyposis and sinusitis in cystic fibrosis. SO Rocky-Mt-Med-J. 1976 Sep-Oct. 73(5). P 261-3. MJ CYSTIC-FIBROSIS: co. NASAL-POLYPS: co. NOSE-NEOPLASMS: co. SINUSITIS: co. MN ADOLESCENCE. ADULT. CASE-REPORT. CHILD. HUMAN. MALE. AB Cystic fibrosis is now appearing in milder form or partial expression of the disease. The occurrence of nasal polyposis and sinusitis in patients with abnormal chest x-rays may be the clue to its diagnosis. RF 001 SCHWARTZ EE AM J ROENTG RAD THER NUCL MED 122 708 974 002 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 003 SHWACHMAN H PEDIATRICS 30 389 962 004 ANON J PEDIATR 88 711 976 005 DI SANTAGNESE PA PEDIATRICS 12 549 953 006 ANON J PEDIATR 88 711 976 CT 1 DAVID TJ J ROY SOC MED 79 23 986 PN 76200 RN 00553 AN 77038213 AU Wilson-G-B. Burdash-N-M. Arnaud-P. Monsher-M-T. Fudenberg-H-H. TI Carcinoembryonic antigen and cystic fibrosis protein in blood from cystic fibrosis homozygotes and heterozygote carriers. SO Scand-J-Immunol. 1976. 5(6-7). P 829-36. MJ CARCINOEMBRYONIC-ANTIGEN. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. BLOOD-PROTEINS: an. BLOOD-SPECIMEN-COLLECTION. CARCINOEMBRYONIC-ANTIGEN: an. CARRIER-PROTEINS: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, fg. GASTROINTESTINAL-NEOPLASMS: im. GEL-DIFFUSION-TESTS. HETEROZYGOTE. HOMOZYGOTE. HUMAN. ISOELECTRIC-FOCUSING. RADIOIMMUNOASSAY. SUPPORT-U-S-GOVT-P-H-S. AB Carcinoembryonic antigen (CEA) activity was measured by radioimmunoassay in blood from cystic fibrosis (CF) homozygotes, heterozygote carriers of CF, normal healthy controls, and other patient controls with carcinomas involving gastrointestinal organs. All samples were also screened by electrofocusing for cystic fibrosis protein (CFP), a metabolic marker previously shown to be associated with the CF gene. Significantly increased levels of CEA activity were found in all CFP-positive groups; however, with one exception all patient controls with marked increases in CEA activity were CFP- negative. Immunodiffusion of perchloric acid extracts of CEA-like material from heterozygote carrier blood indicated that the CEA-like material, which was elevated in homozygotes and heterozygotes for CF, showed only partial identity with two separate CEA preparations obtained from colon carcinomas and was not identical to either A, B, or O(H) blood group substances. This glycoprotein material did, however, react with three different anti-CEA antisera. Our finding of an abnormally increased glycoprotein in cystic fibrosis, taken together with previous reports demonstrating abnormalities in the carbohydrate portion of glycoproteins found in various exocrine secretions in CF, further suggests that the primary defect in this disease is manifested partly as a defect in glycoprotein metabolism. This defect may result from an abnormality in one or more of the glycosyltransferases, possibly caused by a more primary defect in polyamine metabolism. RF 001 ANDERSEN DH ANN NY ACAD SCI 93 500 962 002 BANJO C CANCER RES 34 2114 974 003 BIRD GWG J MED GENET 12 174 975 004 BOAT TF PEDIATR RES 8 531 974 005 BURTIN P CANCER RES 33 3299 973 006 CONOVER JH PEDIATR RES 7 220 973 007 DARCY DA BR J CANCER 28 147 973 008 DAVIDSON AG LANCET 2 1393 973 009 DI SANTAGNESE PA N ENGL J MED 277 1287 967 010 DISCHE Z PEDIATRICS 24 74 959 011 CAUDILL M LANCET 2 307 973 012 ESTERLY NB AM J DIS CHILD 123 200 972 013 GOLD P J EXP MED 121 439 965 014 GOLD P J EXP MED 122 467 965 015 HANSEN HJ HUM PATHOL 5 139 974 016 LAMBLIN G CLIN CHIM ACTA 36 329 972 017 LEV R AM J PATHOL 46 23 965 018 LOUISOT P CLIN CHIM ACTA 48 373 973 019 MACH JP IMMUNOCHEMISTRY 9 1031 972 020 MACSWEEN JM BR J CANCER 26 356 972 021 MANDEL ID AM J DIS CHILD 113 431 967 022 MANGIAROTTI-MARCHI MA MED CHIR DIG 3 141 974 023 MANGOS JA SCIENCE 158 135 967 024 MANGOS JA PEDIATR RES 1 436 967 025 MARTIN F INT J CANCER 6 352 970 026 MCCOMBS ML TEX REP BIOL MED 31 615 973 027 NEVILLE AM INT J CANCER 14 1 974 028 POTTER JL ANN NY ACAD SCI 106 692 963 029 RENNERT OM ANN CLIN LAB SCI 3 1 973 030 ROELFS RE AM J DIS CHILD 113 419 967 031 ROGALSKY VY LANCET 1 1322 973 032 ROUSSEL P J BIOL CHEM 250 2114 975 033 SHWACHMAN H IN: KENDIG EL JR 541 967 034 VONKLEIST S PROC NAT ACAD SCI USA 69 2492 972 036 WILSON GB PEDIATR RES 9 635 975 037 WOOD RE AM REV RESPIR DIS 113 833 976 038 ZANCHECK Z N ENGL J MED 286 83 972 CT 1 WILSON GB PEDIATR RES 11 986 977 2 WILSON GB NATURE 266 463 977 3 WILSON GB PEDIATR RES 12 801 978 4 ALHADEFF JA CLIN GENET 14 189 978 5 MACSWEEN JM PEDIATR RES 14 187 980 6 WILSON GB CLIN GENET 26 331 984 PN 76201 RN 00554 AN 76199237 AU Hoiby-N. Hoff-G-E. Jensen-K. Lund-E. TI Serological types of Diplococcus pneumoniae isolated from the respiratory tract of children with cystic fibrosis and children with other diseases. SO Scand-J-Respir-Dis. 1976. 57(1). P 37-40. MJ CYSTIC-FIBROSIS: mi. RESPIRATORY-SYSTEM: mi. STREPTOCOCCUS-PNEUMONIAE. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. SEROTYPING. SPUTUM: mi. STREPTOCOCCUS-PNEUMONIAE: ip. AB The distribution of serological types of D. pneumoniae was investigated in 40 strains isolated from 26 children with cystic fibrosis and 57 strains isolated from 39 children with other diseases. All strains were isolated from sputum or tracheal secretion. The strains from cystic fibrosis patients belonged to 14 different serological types, the most prevalent were 19F, 19A and 3. The strains from the other group of children belonged to 20 different serological types, the most prevalent were 23F, 19F and 11A. The differences between the two groups of patients as to the prevalences of types were small, and it is concluded that no special serological types of D. pneumoniae are associated with cystic fibrosis. RF 001 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 002 HOIBY N SCAND J RESPIR DIS 56 38 975 003 LUND E BULL WHO 23 5 960 004 LUND E INT J SYSTEM BACT 20 321 970 006 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 HOIBY N SCAND J RESPIR DIS 58 65 977 3 BRANSON D AM J MED TECHNOLOGY 45 224 979 4 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 6 FRIEND PA J INFECT 13 55 986 PN 76202 RN 00555 AN 77038272 AU Hoiby-N. Kilian-M. TI Haemophilus from the lower respiratory tract of patients with cystic fibrosis. SO Scand-J-Respir-Dis. 1976. 57(3). P 103-7. MJ CYSTIC-FIBROSIS: mi. HAEMOPHILUS. RESPIRATORY-SYSTEM: mi. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HAEMOPHILUS-INFLUENZAE: ip. HAEMOPHILUS: cl, ip. HUMAN. LARYNX: mi. MALE. SPUTUM: mi. AB Fifty-six non-haemolytic Haemophilus strains were isolated during current bacteriological examinations from material from the lower respiratory tract of 39 patients with cystic fibrosis during a 6- month period. Except for six strains which belonged to Haemophilus parainfluenzae, all strains were identified as Haemophilus influenzae. Biotype I of H. influenzae was the predominating taxon (38%) and was especially related to patients with recurrent or prolonged colonization. Only two strains were capsulated. An unexpectedly high percentage of the strains produced lysine decarboxylase. The significance of this property in the pathogenesis of the respiratory tract colonization is discussed. RF 001 DENNY FW J INFECT DIS 129 93 974 002 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 003 HOLDAWAY MD LANCET 1 358 967 004 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 005 HOIBY N SCAND J RESPIR DIS 56 38 975 006 KILBOURN JP LANCET 2 439 969 007 KILIAN M ACTA PATH MICROBIOL SCAND 80 571 972 008 KILIAN M J GEN MICROBIOL 93 9 976 009 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 010 MAY JR ARCH DIS CHILD 47 908 972 011 MEARNS MB ARCH DIS CHILD 47 902 972 012 SAGGERS BA J CLIN PATHOL 23 262 970 013 WHITE A PRINCIPLES OF BIOCHEMISTRY 964 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 HOIBY N SCAND J RESPIR DIS 58 65 977 3 OILL PA ARCH INTERN MED 139 985 979 4 CHAD ZH PEDIATRICS 66 9 980 5 IVANOVA NM TER ARKH 53 131 981 6 LISTON TE SOUTH MED J 75 753 982 7 TILLER FW ZENTRALBL BAKT MIKROB HYG (A) 253 236 982 8 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 9 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 10 KILBOURN JP AM REV RESPIR DIS 128 1093 983 11 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 12 NELSON JD J PEDIATR 106 1030 985 13 LEVY J ANTIMICROB AGENTS CHEMOTHER 29 474 986 PN 76203 RN 00556 AN 76246160 AU McCrae-W-M. TI Treatment of pseudomonas infections in cystic fibrosis using tobramycin. SO Scott-Med-J. 1976 Apr. 21(2). P 68-9. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: dt. TOBRAMYCIN: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. TOBRAMYCIN: ad. EX It was decided to treat all seventeen cystic fibrosis patients under our care who were known to have active infection with mucoid strains of Pseudomonas, using large intravenous doses of tobramycin. By the objective observations made, results were not so satisfactory. Changes in white blood cell count and erythrocyte sedimentation rate seemed often to conflict with clinical impression of improvement. However, there was closer agreement between subjective judgements, weight gain and respiratory function changes. Eradication of Pseudomonas from the sputum was achieved in four cases, although the duration of follow-up in one was very short. RF 001 HAWLEY HB CURR THER RES 16 414 974 002 HUANG NN J PEDIATR 78 338 971 003 MARKS MI J PEDIATR 79 822 971 004 RAEBURN JA POSTGRAD MED J 47 366 971 005 RAEBURN JA SCAND J INFECT DIS 5 135 973 CT 1 BROGDEN RN DRUGS 12 166 976 2 FRIIS B SCAND J INFECT DIS 11 211 979 3 HOWIE AD SCOTT MED J 24 193 979 4 HODSON ME BR J DIS CHEST 77 71 983 PN 76204 RN 00557 AN 77215997 AU Begleiter-M-L. Burry-V-F. Harris-D-J. TI Prevalence of divorce among parents of children with cystic fibrosis and other chronic diseases. SO Soc-Biol. 1976 Fall. 23(3). P 260-4. MJ CHRONIC-DISEASE. CYSTIC-FIBROSIS. DIVORCE. MN ATTITUDE-TO-HEALTH. CHILD. HUMAN. MISSOURI. PARENT-CHILD-RELATIONS. AB Data on the prevalence of divorce and separation among parents of children with cystic fibrosis and other chronic diseases indicate that marital breakdown is no more prevalent among these couples than it is in a general population. For couples who attended genetic counseling clinics or had children with spina bifida or leukemia, the divorce rate is lower than the United States national average. For parents of children with cystic fibrosis, the divorce rate is the same as the national average. The high recurrence risk for cystic fibrosis may deter many parents from further reproduction. We speculate that the inability to plan more children may be the factor responsible for the higher prevalence among these parents compared to those of children with other chronic diseases. RF 001 ALLAN JL AUST PAEDIATR J 10 136 974 002 CARTER CO LANCET 1 281 971 003 EMERY AEH BR MED J 1 724 973 004 FREESTON BM DEVELOP MED CHILD NEUROL 13 456 971 005 FRIEDMAN SB PEDIATRICS 32 610 963 006 GLUCKSON MM AM J HUM GENET 27 39A 975 007 HAMOVITCH MB PARENT AND THE FATALLY IL 964 008 HARE EH BR MED J 2 757 966 009 MCCOLLUM AT J PEDIATR 77 571 970 010 MCCRAE WM LANCET 2 141 973 011 OAKLEY GR NC MED J 27 186 966 012 REYNOLDS BD CLIN GENET 5 177 974 013 SORENSON JR AAAS SYMPOSIUM 972 014 STEHBENS JA J CLIN PSYCHOL 30 394 974 015 TURK J PEDIATRICS 34 67 964 016 ANON CENSUS OF POPULATION MARITAL 970 017 WALKER JH DEVELOP MED CHILD NEUROL 13 462 971 CT 1 HARTUNG J SOC BIOL 24 192 977 2 LANSKY SB PEDIATRICS 62 184 978 3 SILBERT AR PEDIATRICS 69 747 982 4 DEWET B S AFR MED J 65 526 984 5 THELIN T ACTA PSYCHIATR SCAND 71 636 985 PN 76205 RN 00558 AN 77127511 AU Levitt-R-G. Forrest-J-V. Sagel-S-S. TI Roentgenographic recognition of bronchoceles. SO South-Med-J. 1976 Dec. 69(12). P 1556-8. MJ BRONCHIECTASIS: ra. MN ADOLESCENCE. ADULT. ASPERGILLOSIS: co. ASPERGILLUS-FUMIGATUS. ASTHMA: co. BRONCHI: ab. BRONCHIAL-NEOPLASMS: co. BRONCHIECTASIS: et. BRONCHOGRAPHY. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DIAGNOSIS-DIFFERENTIAL. FEMALE. HUMAN. MALE. XENON-RADIOISOTOPES: du. AB Bronchoceles often have characteristic plain film, tomographic, and bronchographic appearances, and they may trap air. Roentgenographic recognition of bronchoceles can allow them to be distinguished from neoplasms and sometimes help avoid unnecessary surgery. RF 001 LEMIRE P AM J ROENTG RAD THER NUCL MED 110 687 970 002 MCCARTHY C CLIN RADIOL 21 366 970 003 WARING WW PEDIATRICS 39 166 967 004 TALNER L AM J ROENTG RAD THER NUCL MED 110 675 970 005 GENEREUX GP J CAN ASSOC RADIOL 22 71 971 006 FENNESSY J RADIOL CLIN NORTH AM 11 371 973 007 REES DO CLIN RADIOL 21 62 970 008 WADDELL J THORAX 20 214 965 009 HUTCHESON J AM J CLIN PATHOL 33 427 960 010 FRASER RG DIAGNOSIS OF DISEASES OF CHES 970 011 SLAVIN RG J ALLERGY 46 150 970 CT 1 ARONBERG DJ AM J ROENTGENOL 132 23 979 PN 76206 RN 00559 AN 77127499 AU Lober-C-W. TI Manifestations of cystic fibrosis in adults [letter]. SO South-Med-J. 1976 Nov. 69(11). P 1525. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: di. HUMAN. EX Cystic fibrosis (CF) is a recessive autosomal genetic disease in which exocrine glands secrete highly viscous mucus. Variable genetic penetrance ("organ sparing") results in a broad spectrum of clinical presentations. The diagnosis of CF is usually established by the presence of chronic obstructive lung disease and/or gastrointestinal dysfunction, together with elevated levels of sweat electrolytes (chloride > 60 mEq/liter), markedly diminished duodenal pancreatic enzyme concentrations, or pathologic changes in tissue. Family history may occasionally be helpful. The metabolic basis of CF is unknown. The classic stereotype of the CF patient is an infant with a chronic cough, diarrhea, and growth failure. Improved medical care is enabling many of these patients to reach adulthood, and increased diagnostic suspicion is resulting in identification of the disease in more adults. Based upon analysis of over 11,000 cases of CF reported during the year 1974 by 119 Cystic Fibrosis Centers, the National Cystic Fibrosis Foundation estimated that the probability of an individual with CF surviving to age 20 was approximately 39%. Although most of these adults are currently under the care of pediatricians, the development of medical, psychiatric, surgical, or obstetric complications may necessitate the involvement of other professionals. A joint study of cystic fibrosis in adults conducted by Duke Medical Center and the NIH has shown that the majority of affected adults, including those diagnosed early in life, are married and work full- or part-time. Many manifest few if any symptoms until relatively late in life. Since this study was retrospective, the reported frequencies of the clinical findings tend to underestimate, in all instances, the actual incidence in the population studied. The reported incidence of CF in the United States has been conservatively estimated at 1:2,000 live births. If all adults with clubbing, infertility, and other symptoms were evaluated for CF, the true incidence would certainly be higher. RF 001 ANON CF FND 1974 REP SURVI STUD PA 976 002 LOBER CW PROC AM COLL CHEST 40TH AN MT 974 003 DI SANTAGNESE PA IN: DOWNEY JA 25 974 PN 76207 RN 00560 AN 77060022 AU Litt-M. Khan-M-A. TI Detection of cystic fibrosis heterozygotes using the zeta potential reduction method. SO Tex-Rep-Biol-Med. 1976. 34(1). P 151-4. MJ CYSTIC-FIBROSIS: di. SALIVA: an. MN ADULT. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. GENES. HETEROZYGOTE. HOMOZYGOTE. HUMAN. METHODS. AB Parotid saliva samples from cystic fibrosis homozygotes, heterozygotes and normal individuals were tested by the zeta potential technique for secretion factor in a double-blind experiment. There was no overlap between zeta potential reduction of the control group and the cystic fibrosis test groups, indicating that the zeta potential test may be detecting a unique component in saliva of cystic fibrosis genotypes. RF 001 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 002 CONOVER JH PEDIATR RES 7 220 973 003 KHAN MA TEX REP BIOL MED 31 665 973 004 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 PN 76208 RN 00561 AN 77060026 AU Novak-R-A. Abell-C-W. TI L-fucose metabolism in cystic fibrosis fibroblasts. SO Tex-Rep-Biol-Med. 1976. 34(1). P 199-207. MJ CYSTIC-FIBROSIS: me. FUCOSE: me. MN CHROMATOGRAPHY. COMPARATIVE-STUDY. FIBROBLASTS: me. GLYCOPEPTIDES: me. HUMAN. MOLECULAR-WEIGHT. SUPPORT-U-S-GOVT-P-H-S. AB The capacity of skin fibroblasts from cystic fibrosis (CF) and non-CF individuals to incorporate exogenous L-fucose into various metabolic pools was compared. Incorporation into intracellular acid-soluble pools and extracellular glycopeptides was measured by a dual-label co- chromatographic technique, whereas total macromolecular incorporation was measured by a single-label procedure. Although CF and normal fibroblasts incorporate exogenous fucose into intracellular acid- soluble and high molecular weight compounds in a similar manner, CF cells appear to be characterized by an increased incorporation of label into low molecular weight extracellular glycopeptides. RF 001 BAUER U ANN PAEDIATR 194 236 960 002 BOLTON WE AM J HUM GENET 27 394 975 003 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 004 CHERNICK WS ANN NY ACAD SCI 106 698 963 005 DANES BS J EXP MED 129 775 969 006 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 007 DI SANTAGNESE PA PEDIATRICS 19 252 957 008 DISCHE Z AM J DIS CHILD 102 733 961 009 FLETCHER DS CLIN CHIM ACTA 44 5 973 010 JOHANSEN PG ANN NY ACAD SCI 106 755 963 011 JOHANSEN PG BIOCHEM J 87 63 963 012 KAUFMAN RL EXP CELL RES 50 127 968 013 LOBECK CC IN: STANBURY JB 1605 972 014 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 015 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 016 POTTER JL ANN NY ACAD SCI 106 692 963 017 ROBERTS RM J CELL BIOL 59 86 973 020 TRUJILLO JL BIOCHIM BIOPHYS ACTA 230 610 971 021 WIESMANN UN J PEDIATR 77 685 970 PN 76209 RN 00562 AN 77060025 AU Rennert-O-M. Frias-J. Shukla-J-B. TI Polyamine metabolism in cystic fibrosis. SO Tex-Rep-Biol-Med. 1976. 34(1). P 187-97. MJ CYSTIC-FIBROSIS: me. POLYAMINES: me. MN ANIMAL. ELECTROLYTES: bl. HAMSTERS. SPERMIDINE: bl. SUPPORT-U-S-GOVT-P-H-S. AB Increased blood levels of spermidine and an increased spermidine/spermine ratio is documented in 31 patients with cystic fibrosis (CF). Some 29 percent of CF patients overlap with the control group. An abnormality of urinary free polyamines or their metabolic derivatives related to electrolyte transport, serum ciliostatic factors, and the autonomic nervous system are elaborated upon. RF 001 NOUSIA-ARVANITAKIS S CF CLUB ABST 5 972 002 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 003 BARBERO GJ IN: DI SANTAGNESE PA 966 004 BARNETT DR TEX REP BIOL MED 31 691 973 005 BARNETT DR TEX REP BIOL MED 31 703 973 006 BOQUIST L LAB INVEST 21 96 969 007 BOWMAN BH SCIENCE 164 325 969 008 BYUS C GORDON RES CONF POLYAMINES 975 009 COHEN LF PEDIATR RES 9 334 975 010 DOGGETT RG NATURE NEW BIOL 243 251 973 011 DOGGETT RG TEX REP BIOL MED 31 685 973 012 ELFERINK J Z NATURFORSCH 30 117 975 013 FARRELL PM CF CLUB ABST 974 014 FLEISHER DS J PEDIATR 64 341 964 015 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 016 INOUE H J BIOCHEM (TOKYO) 75 679 974 017 KAISER D PEDIATR RES 5 167 971 018 KOSSOROTOW A BIOCHEM J 144 21 974 019 MANGOS JA SCIENCE 158 135 967 020 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 021 MARTINEZ JR PEDIATR RES 9 463 975 022 MARTINEZ JR PEDIATR RES 9 470 975 023 MILLS J MOL PHARMACOL 2 311 966 025 PARSA I FED PROC 31 166 972 027 RENNERT OM IN: MANGOS JA 41 973 030 SEILER N J CHROMATOGR 63 97 971 031 SMITH T BIOCHEM J 84 292 962 032 SPOCK A PEDIATR RES 1 173 967 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 MIALE TD MED PEDIATR ONCOL 3 209 977 3 WRIGHT RK PEDIATR RES 12 830 978 4 DUNZENDORFER U CANCER RES 38 2321 978 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 DESSER H ZENTRALBL VETERINARMED (A) 27 45 980 7 THEOHARIDES TC LIFE SCI 27 703 980 8 DESSER H ONCOLOGY 37 376 980 9 RENNERT OM CLIN CHIM ACTA 103 375 980 10 KIMBERLY MM ANAL CHEM 53 789 981 11 DESSER H ADV POLYAMINE RES 4 49 982 12 CASTI A J ENDOCRINOLOGICAL INVEST 5 263 982 13 DESSER H ONKOLOGIE 5 36 982 14 EKSTROM J ACTA PHYSIOL SCAND 119 287 983 15 CHAYEN R CELL BIOCHEM FUNCT 2 15 984 16 SCALABRINO G MECH AGEING DEV 26 149 984 17 PALES JL SCAND J HAEMATOL 32 241 984 PN 76210 RN 00563 AN 77060024 AU Arvanitakis-S. Mangos-J. Mcsherry-N-R. Rennert-O. TI Effect of polyamines and cystic fibrosis serum on glucose transport. SO Tex-Rep-Biol-Med. 1976. 34(1). P 175-86. MJ CYSTIC-FIBROSIS: bl. GLUCOSE: me. POLYAMINES: pd. MN ADULT. ANIMAL. CHILD. EPITHELIUM: me. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IN-VITRO. JEJUNUM: me. MALE. RATS. SPERMIDINE: pd. AB The effect of plasma or serum from homozygotes and heterozygotes for the cystic fibrosis (CF) gene on the active uptake of 3-0-14C-methyl- D-glucose (3-0-14C-MDG) by rat jejunal epithelium was studied. Furthermore, the role of the polyamine, spermidine, and its products of metabolic degradation on glucose transport were investigated, and a relationship to the pathogenesis of membrane dysfunction in cystic fibrosis was postulated. Glucose transport in everted rat jejunal rings was used in the study. Results were expressed as 3-0-14C-MDG concentration ratio between the intracellular (ICF) and the extracellular fluid spaces (ECF) of the jejunal rings at the end of a 60 min incubation period. The mean ratio obtained from incubations of the rat jejunal rings in medium consisting of Krebs-Ringer- bicarbonate buffer and the labeled sugar was considered as 100% uptake. When plasma or serum, with or without spermidine, was mixed with the medium in a volume ratio of 1:3, a decrease in the active uptake of 3-0-14C-MDG was observed, expressed as percent inhibition. Percent inhibition of 3-0-14C-MDG uptake obtained when the rat jejunal rings were incubated in normal plasma was compared to that obtained with plasma from cystic fibrosis genotypes. It was found that: 1) plasma from 25 homozygous children had greater inhibitory effect on glucose uptake than plasma from 26 normal children; 2) plasma from 9 heterozygous women had greater inhibitory effect than that from 6 normal women; 3) the inhibitory effect of plasma from 3 homozygous children was not influenced by dialysis; 4) the inhibitory effects of paired plasma and serum samples from 9 homozygotes were comparable; 5) spermidine added to the incubating electrolyte solution did not affect glucose transport; 6) the addition of spermidine to reaction mixtures containing normal plasma potentiated the inhibitory effect; and 7) mixing and incubation of fresh bovine serum with reaction mixtures containing plasma from homozygotes decreased the inhibitory effect. The predominant inhibitory effect of plasma or serum from homozygotes and heterozygotes for the CF gene appears to be related to a nondialyzeable molecule(s). It does not seem to reflect the presence of high plasma glucose levels in cystic fibrosis nor to be the result of competitive inhibition between sugars. It does not seem to be the result of sodium or other electrolyte differences. A similar inhibitory effect is acquired by normal plasma after the addition of spermidine. On the other hand, plasma from CF homozygotes loses its inhibitory effect after incubation with fresh bovine serum. These findings may indicate that products of metabolic degradation of spermidine are responsible for the inhibitory effect of glucose transport and suggest the possibility of a role in abnormal polyamine metabolism in the pathogenesis of cystic fibrosis. RF 001 BACHRACH U J GEN VIROL 13 415 971 002 BOWMAN BH SCIENCE 164 325 969 003 BOWMAN BH SCIENCE 167 871 970 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 BREMER HJ CLIN CHIM ACTA 32 407 971 006 BROWN GA LANCET 2 639 971 007 CRANE RK ABSORPTION OF SUGARS HANDBOOK 3 968 008 DESJEUX JF EUROPEAN SOC PEDIAT RES MTG 970 009 MCEWEN C J BIOL CHEM 240 2003 965 010 FITZPATRICK DF NATURE NEW BIOL 235 173 972 011 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 012 KAISER D PEDIATR RES 5 167 971 013 KIMES BW BIOCHIM BIOPHYS ACTA 228 223 971 014 KREMZNER LT BIOCHEM PHARMACOL 19 2541 970 015 LOWRY OH J BIOL CHEM 193 265 951 016 MANGOS JA SCIENCE 158 135 967 017 MANGOS JA PEDIATR RES 1 436 967 018 MANGOS JA PEDIATR RES 2 378 968 019 OLSEN WA J CLIN INVEST 49 96 970 020 PEGG AE BIOCHIM BIOPHYS ACTA 232 630 971 021 RAO GJS J PEDIATR 80 573 972 022 RAO GJS SCIENCE 177 610 972 023 RAINA A PROC NAT ACAD SCI USA 55 1587 966 024 RENNERT OM CLIN PEDIATR 11 351 972 025 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 026 SPOCK A PEDIATR RES 1 173 967 027 TABOR H PHARMACOL REV 16 245 964 028 TABOR CW J BIOL CHEM 239 2194 964 029 WALTER V CLIN RES 19 204 971 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 WRIGHT RK PEDIATR RES 12 830 978 3 WILL PC PEDIATR RES 13 1129 979 4 THEOHARIDES TC LIFE SCI 27 703 980 5 SHIPE JR CRC CRIT REV CLIN LAB SCI 16 1 981 6 KIMBERLY MM ANAL CHEM 53 789 981 7 SCALABRINO G ADV CANCER RES 36 1 982 8 GENEDANI S LIFE SCI 38 1293 986 PN 76211 RN 00564 AN 77060023 AU Baur-P-S. Brinkley-B-R. Bowman-B-H. TI Effects of cystic fibrosis serum ciliary inhibitor on oyster gill ultrastructure: analysis by scanning and transmission electron microscopy. SO Tex-Rep-Biol-Med. 1976. 34(1). P 155-74. MJ BLOOD-PROTEINS. CILIA: ul. CYSTIC-FIBROSIS: bl. MODELS-BIOLOGICAL. OYSTERS: ul. MN ANIMAL. CELLS-CULTURED. CILIA: ph. CYSTIC-FIBROSIS: me. GILLS: ul. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MICROSCOPY-ELECTRON-SCANNING. AB Ultrastructural analysis including scanning and transmission electron microscopic studies (SEM and TEM) were carried out on oyster gill tissue after exposure either to serum fractions from individuals homozygous or heterozygous for cystic fibrosis (CF), to comparable serum fractions from normal individuals, or to sea water. In 4 of the experiments examined topologically (SEM), the CF sera (either heterozygous or homozygous) stimulated the production of mucus that was found in close association with the cilia. The association of excessive mucus with the cell surface could be responsible, in part, for the well-known inhibition of ciliary activity by a factor in CF serum. In 3 additional SEM experiments involving shorter treatment times, very little difference could be observed between homozygous CF, heterozygous CF, and normal serum-fraction-treated oyster tissues. In parallel experiments, ultrathin sections of gill tissue were examined by means of TEM. Those samples that were responsive, as determined by TEM, displayed several characteristic features, including enlarged and partially exuded goblet cells, altered mucus structure along with twisted and matted cilia. An overall swelling of the gill filament was also observed in the responsive tissues. From TEM analysis no detectable alteration in fine structure was apparent in gill tissues that were treated with sera from heterozygous or normal individuals. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 ANDERSON TF NY ACAD SCI SER II 13 130 951 003 BESLEY GTN J MED GENET 6 278 969 004 BARNETT DR TEX REP BIOL MED 31 691 973 005 BOWMAN BH TEX REP BIOL MED 31 611 973 006 BOWMAN BH IN: MANGOS JA 29 973 007 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 008 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 009 BOWMAN BH SCIENCE 164 325 969 010 BOWMAN BH SCIENCE 167 871 970 011 CHERRY JD J PEDIATR 79 937 971 012 CONOVER JH PEDIATR RES 7 220 973 013 GALSTOFF PS FISHERY BULL 64 1 964 014 LUFT JH J BIOPHYS BIOCHEM CYTOL 9 409 961 015 SPOCK A PEDIATR RES 1 173 967 CT 1 WARD JB TEX REP BIOL MED 34 11 976 2 BOWMAN BH CLIN GENET 12 333 977 3 FORSTNER JF DIGESTION 17 234 978 4 GABRIDGE MG PEDIATR RES 13 31 979 5 NAGY EC PEDIATR RES 13 729 979 6 BOWMAN BH TEX REP BIOL MED 38 47 979 7 KENNEDY JR PEDIATR RES 14 1173 980 8 BOWMAN BH FED PROC 39 3195 980 9 SANDERSON MJ PEDIATR RES 15 219 981 10 TEGNER H ACTA PAEDIATR SCAND 70 629 981 11 MCPHERSON MA CLIN CHIM ACTA 135 181 983 12 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 13 WOOTEN MW J CELL PHYSIOL 121 490 984 PN 76212 RN 00565 AN 77060027 AU Carson-S-D. Harper-B-L. Barnett-D-R. Kurosky-A. Lankford-B-J. Bowman-B-H. TI Electrophoretic studies of the cystic fibrosis ciliary inhibitor and its interaction with immunoglobulin G. SO Tex-Rep-Biol-Med. 1976. 34(1). P 210-9. MJ BLOOD-PROTEINS: me. CILIA: ph. CYSTIC-FIBROSIS: bl. IGG: me. MN CYSTIC-FIBROSIS: me. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FIBROBLASTS: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MOLECULAR-WEIGHT. SUPPORT-U-S-GOVT-P-H-S. AB The cystic fibrosis ciliary inhibitor (CFCI) has been partially purified from serum and plasma of cystic fibrosis (CF) homozygotes and heterozygotes, and from media of cultured fibroblasts derived from cystic fibrosis genotypes. Characterization and comparison of fractions containing the CFCI were carried out by polyacrylamide gel electrophoresis. Gel electrophoresis confirmed previous molecular weight estimations of 4,500 to 11,000 for the CFCI and provided an estimate of the number of proteins present in the fractions. Low molecular weight proteins from serum and media were combined with IgG preparations. No specific binding to IgG by the media fraction containing the CFCI could be demonstrated by the techniques employed. There was decreased binding of the low molecular weight serum fraction containing CFCI to native IgG molecules from cystic fibrosis patients as compared to IgG from normal individuals. However, IgG from CF individuals demonstrated increased binding of the cfci- containing low molecular weight serum fraction after gel filtration in the presence of guanidinium chloride. This suggests: 1) that very low concentrations of CFCI are present in media fractions; and 2) that native CF IgG cannot bind the low molecular weight CFCI fractions to the same degree as native IgG from normals or CF IgG that has been dissociated from non-covalently bound components. RF 001 BARNETT DR TEX REP BIOL MED 31 703 973 002 BARNETT DR TEX REP BIOL MED 31 709 973 003 BOLTON WE AM J HUM GENET 27 394 975 004 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 005 BOWMAN BH SCIENCE 164 325 969 006 BOWMAN BH SCIENCE 167 871 970 007$ CUATRECASAS P PROC NAT ACAD SCI USA 70 485 970 008 CUATRECASAS P PROC NAT ACAD SCI USA 61 636 968 009 DANES BS J EXP MED 136 1313 972 010 DANES BS J EXP MED 137 1538 973 011 HARPER BL TEX REP BIOL MED 34 73 976 012 JOUSTRA M PROTIDES BIOL FLUIDS 17 511 969 013 PALACIOS R J BIOL CHEM 247 2316 972 014 PANYIM S ARCH BIOCHEM BIOPHYS 130 337 969 015 SWANK RT ANAL BIOCHEM 39 462 971 PN 76213 RN 00566 AN 77060021 AU Danes-B-S. TI Expression of the cystic fibrosis genotype in cultured somatic cells. SO Tex-Rep-Biol-Med. 1976. 34(1). P 135-50. MJ CYSTIC-FIBROSIS: fg. MN CELL-MEMBRANE: me. CELLS-CULTURED. CYSTIC-FIBROSIS: me, pa. FIBROBLASTS: me. GENOTYPE. HEPARIN: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYBRID-CELLS. LEUKOCYTES: me. LYSOSOMES: me. MUCOPOLYSACCHARIDES: me. PINOCYTOSIS. STAINS-AND-STAINING. AB Observations on the characteristics of the cell with the cystic fibrosis (CF) genotype in culture are reviewed. Although numerous and diverse abnormalities have been described, none were specific for the CF gene. The relevance of each of these abnormalities to the clinical syndrome known as CF is discussed, emphasizing that the value thus far of such cell culture research has been to learn how the CF gene influences cellular function. RF 001 BAIG MM J PEDIATR 86 72 975 002 BARKA T HISTOCHEMISTRY THEORY PRACTIC 84 963 003 BARNETT DR TEX REP BIOL MED 31 691 973 004 BARNETT DR PEDIATR RES 8 687 974 005 BARNETT DR TEX REP BIOL MED 31 697 973 006 BARNETT DR TEX REP BIOL MED 31 703 973 007 BARNETT DR TEX REP BIOL MED 31 709 973 008 BARTMAN J J PEDIATR 76 430 970 009 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 010 BERATIS NG PEDIATR RES 7 958 973 010A BOLTON WE AM J HUM GENET 27 394 975 011 BOWMAN BH IN: MANGOS JA 29 973 012 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 013 BOWMAN BH CLIN GENET 4 461 973 014 BOWMAN BH SCIENCE 164 325 969 015 BOWMAN BH SCIENCE 167 871 970 016 BOWMAN BH AM J HUM GENET 25 16A 973 017 CAUDILL M LANCET 1 32 974 018 CHOU L PEDIATR RES 9 312 975 019 CONOVER JH PEDIATR RES 7 224 973 020 CONOVER JH PEDIATR RES 7 220 973 021 CONOVER JH LANCET 2 1501 973 022 CONOVER JH LANCET 1 1194 973 023 CONOVER JH LIFE SCI 14 253 974 024 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 025 DANES BS BIRTH DEF ORIG ART SER 8 114 972 026 DANES BS LANCET 2 765 973 027 DANES BS LANCET 2 655 974 028 DANES BS CLIN GENET 7 128 975 029 DANES BS IN: KELLY S 297 976 031 DANES BS BIOCHEM GENET 12 359 974 032 DANES BS J EXP MED 129 775 969 033 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 034 DANES BS J EXP MED 136 1313 972 035 DANES BS CLIN GENET 8 85 975 036 DANES BS J EXP MED 137 1538 973 037 DEMARS R NAT CANCER INST MONOGRAPH 13 181 964 038 DI FERRANTE N CONNECT TISSUE RES 1 93 972 039 DI SANTAGNESE PA N ENGL J MED 277 1287 967 040 DOGGETT RG NATURE NEW BIOL 243 251 973 041 FLETCHER DS CLIN CHIM ACTA 44 5 973 042 FRATANTONI JC PROC NAT ACAD SCI USA 60 699 968 043 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 045 KLAGSBRUN M PEDIATR RES 8 205 974 046 KRAUS I PEDIATRICS 47 1010 971 047 LIE SO PROC NAT ACAD SCI USA 69 2361 972 048 MCMANUS SP J MED GENET 11 216 974 049 MATALON R PROC NAT ACAD SCI USA 56 1310 966 050 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 051 MILUNSKY A N ENGL J MED 281 1128 969 052 NADLER HL LANCET 2 84 969 054 PALLAVICINI JC J PEDIATR 77 280 970 055 POLLEY MJ J MED GENET 11 249 974 056 RAFF EC J CELL PHYSIOL 74 235 969 057 RENNERT OM CLIN PEDIATR 11 351 972 058 RENNERT OM PEDIATRICS 50 485 972 059 ROBERTSON JA LANCET 1 1256 974 060 RUSSELL SB J MED GENET 8 441 971 061 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 062 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 063 SPOCK A PEDIATR RES 1 173 967 064 SYLVEN B ACTA HISTOCHEM SUPPL 1 79 958 065 TAYSI K N ENGL J MED 281 1108 969 066 WIESMANN UN J PEDIATR 77 685 970 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 DANES BS CLIN GENET 13 327 978 3 WILSON GB J LAB CLIN MED 92 463 978 4 WILSON GB PEDIATR RES 13 1079 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 7 WILSON GB J CLIN INVEST 66 1010 980 8 TEGNER H ACTA PAEDIATR SCAND 70 629 981 PN 76214 RN 00567 AN 77060020 AU Baur-P-S. Bolton-W-E. Barranco-S-C. TI Electron microscopy and microchemical analysis of cystic fibrosis diploid fibroblasts in vitro. SO Tex-Rep-Biol-Med. 1976. 34(1). P 114-34. MJ CYSTIC-FIBROSIS: pa. MN ADULT. ANIMAL. CALCIUM: an. CELL-DIVISION. CELL-LINE. CELLS-CULTURED. CHILD. CYSTIC-FIBROSIS: me. CYTOPLASM: ul. DIPLOIDY. FEMALE. FIBROBLASTS: ul. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INFANT. MALE. SULFUR: an. SUPPORT-U-S-GOVT-P-H-S. AB Fibroblasts derived from cystic fibrosis homozygotes and heterozygotes were compared to normal fibroblasts topologically, ultrastructurally, and microchemically. Topological examinations, by means of scanning electron microscopy (SEM) revealed no significant differences between the 3 genotypes surveyed. The cells were generally flattened and/or fusiform structures that were more or less devoid of surface details. However, the dividing cells, in all of the populations surveyed, were found to have a more or less spherical configuration involving a highly complicated surface. The surface manifestations included blebs, ridges, and microvilli. Representative samples of the cells in all stages of growth, lag phase, exponential phase, and plateau phase, were surveyed in this study. Ultrastructurally, the fibroblasts of the 3 genotypes were found to have similar cytological detail. Highly infolded nuclei, distinct organelle components, and surface details, also seen in the SEM study, were observed. Numerous bundles of microfilaments were noted within the cytoplasm. Metachromatic granules were observed in cells from all 3 genotypes. Microchemical analysis, by means of energy dispersive X-ray analysis, demonstrated slight but recognizable differences in the elemental composition of the 3 genotypes. Of notable interest were the peak intensities of calcium and sulfur. The CF homozygous cells presented higher values for both of the elements when compared to the values observed in the CF heterozygous and normal cell populations. Although consistent differences could be observed in the CF genotypes when compared to the non-CF cells, no attempt was made to quantitate the concentrations of each of the elements within the cells. RF 001 ANDERSON TF NY ACAD SCI SER II 13 130 951 002 BARNETT DR TEX REP BIOL MED 31 691 973 003 BARTMAN J J PEDIATR 76 430 970 004 BEARN AG N ENGL J MED 282 102 970 005 BRADDOCK LI CF CLUB ABST 31 970 006 BOLTON WE AM J HUM GENET 27 394 975 007 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 008 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 009 BOWMAN BH SCIENCE 167 871 970 010 BOYDE A EXP CELL RES 71 313 972 011 CHERNICK WS MOD PROBL PEDIATR 10 125 967 012 DANES BS LANCET 2 765 973 013 DUNN MJ BIOCHIM BIOPHYS ACTA 352 96 974 014 EVERHART LP J CELL BIOL 60 442 974 015 GIBSON LE PEDIATRICS 48 695 971 016 GOLDBLOOM RG PEDIATRICS 48 693 971 017 GUGLER EC J PEDIATR 71 585 967 018 HODGES GM J CELL SCI 11 233 972 019 KAMENSKY E AM J PATHOL 73 59 973 020 LIM R SCIENCE 185 63 974 021 LUFT JH J BIOPHYS BIOCHEM CYTOL 9 409 961 022 PAWELETZ N CYTOBIOLOGIE 8 228 974 023 PORTER KR J CELL BIOL 57 815 973 024 PORTER KR J CELL BIOL 59 633 973 025 PORTER KR CELL 2 145 974 026 WARTON KL BR MED J 3 570 971 PN 76215 RN 00568 AN 77060019 AU Ward-J-B-Jr. TI Cultured cells in cystic fibrosis research: A review. SO Tex-Rep-Biol-Med. 1976. 34(1). P 11-36. (REVIEW). MJ CYSTIC-FIBROSIS: pa. MN ADENOSINE-CYCLIC-MONOPHOSPHATE: me. ANIMAL. BIOLOGICAL-TRANSPORT. BODY-FLUIDS: an. CELL-DIVISION. CELL-MEMBRANE: me. CELLS-CULTURED. CILIA. COLLAGEN: me. CYSTIC-FIBROSIS: bl, me. ELECTROLYTES: me. FIBROBLASTS. GLUCOSYLTRANSFERASES: me. GLYCOGEN: me. HUMAN. LYSOSOMES: en. MUCOPOLYSACCHARIDES: me. OYSTERS. PROTEINS: me. RABBITS. REVIEW. RNA-TRANSFER-METHYLTRANSFERASES: me. SUPPORT-U-S-GOVT-P-H-S. AB Cystic fibrosis (CF) is a common inherited disorder which is characterized by the production of exocrine secretions with elevated ion content and abnormally viscous mucus. Over the last few years cells obtained from the peripheral blood or cultured from tissues of cystic fibrosis patients have been used increasingly in the study of the disease. Investigations of the following properties of cystic fibrosis cells are reviewed: morphology, ultrastructure, growth kinetics, cellular metachromasia, the production of ciliary inhibitors, cellular composition, plasma membrane composition, the transport of inorganic ions and small organic molecules, lysosomal enzyme content, and RNA methylation. Studies of the effects on cultured cells and erythrocyte membranes of factors in CF cell culture medium and biological fluids from CF patients are discussed. RF 001 ANTONOWICZ I PEDIATR RES 6 803 972 002 BAFALLUY E LANCET 1 973 971 003 BAIG MM J PEDIATR 86 72 975 004 BALFE JW SCIENCE 162 689 968 005 BARNETT DR TEX REP BIOL MED 31 691 973 006 BARNETT DR TEX REP BIOL MED 31 697 973 007 BARNETT DR TEX REP BIOL MED 31 703 973 008 BARTMAN J J PEDIATR 76 430 970 009 BAUR PS TEX REP BIOL MED 34 155 976 010 BENKE PJ LANCET 1 182 972 011 BENKE PJ PROC SOC EXP BIOL MED 137 1283 971 012 BERATIS NG PEDIATR RES 7 958 973 013 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 014 BOLTON WE AM J HUM GENET 27 394 975 014A BOLTON WE TEX REP BIOL MED 34 97 976 015 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 016 BOWMAN BH LANCET 1 404 974 017 BOWMAN BH CLIN GENET 4 461 973 018 BOWMAN BH SCIENCE 164 325 969 019 BOWMAN BH SCIENCE 167 871 970 020 BOXERBAUM B AM REV RESPIR DIS 108 777 973 021 BUCHWALD M AM J HUM GENET 27 22A 975 022 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 023 CHANGUS JE AM J PATHOL 80 317 975 024 COLE CH PEDIATR RES 6 616 972 025 COLE CH PEDIATR RES 9 763 975 026 CONOD EJ PEDIATR RES 9 724 975 027 CONOVER JH PEDIATR RES 7 224 973 028 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 029 DANES BS LANCET 2 765 973 030 DANES BS LANCET 1 1061 968 031 DANES BS J EXP MED 129 775 969 032 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 033 DANES BS J EXP MED 136 1313 972 034 DANES BS CLIN GENET 8 85 975 035 DANES BS J EXP MED 137 1538 973 036 DANES BS AM J HUM GENET 23 297 971 037 DANES BS NATURE 222 685 969 038 DI SANTAGNESE PA N ENGL J MED 277 1287 967 039 DUFFY MJ CLIN CHIM ACTA 50 97 974 040 DUNHAM ET J CLIN INVEST 49 1804 970 042 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 043 FEIG SA PEDIATR RES 8 594 974 044 FITZPATRICK DF NATURE NEW BIOL 235 173 972 045 FLETCHER DS CLIN CHIM ACTA 44 5 973 046 ANON GAP CONF REP CELL TISS CULT 6 971 047 GIBBS GE SCIENCE 167 993 970 048 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 049 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 050 HODES ME PEDIATR RES 8 212 974 051 HOFFMAN JF AM J MED 41 666 966 052 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 053 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 054 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 055 JACOBSON CB CF CLUB ABST 23 974 056 KLAGSBRUN M IN: MANGOS JA 53 973 057 KLAGSBRUN M PEDIATR RES 8 205 974 058 KRAUS I PEDIATRICS 47 1010 971 059 LAPEY A PEDIATR RES 5 446 971 060 LIE SO PROC NAT ACAD SCI USA 69 2361 972 061 LOBECK CC PROC INT CONF RES PATHOG 3RD 107 966 062 LOUISOT P CLIN CHIM ACTA 48 373 973 063 MANGOS JA TEX REP BIOL MED 31 651 973 064 MANGOS JA SCIENCE 158 135 967 065 MARCHI AG HELV PAEDIATR ACTA 28 427 973 066 MCMANUS SP IR J MED SCI 143 227 974 067 MATALON R LANCET 2 838 969 068 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 069 MCEVOY FA CLIN CHIM ACTA 54 195 974 070 MILUNSKY A N ENGL J MED 281 1128 969 071 NADLER HL J PEDIATR 74 823 969 072 NADLER HL LANCET 2 84 969 073 NEUFELD EF REPORT ON STAINING FOR METACH 972 074 PALLAVICINI JC J PEDIATR 77 280 970 075 POST RL J BIOL CHEM 235 1796 960 076 QUISSELL DO NATURE 247 115 974 077 RAFF EC J CELL PHYSIOL 74 235 969 078 REED GB J PATHOL 101 251 970 079 RENNERT OM CLIN PEDIATR 11 351 972 080 RENNERT OM IN: MANGOS JA 41 973 081 RENNERT OM PEDIATRICS 50 485 972 082 ROBERTSON JA LANCET 1 1256 974 083 RUSSELL SB J MED GENET 8 441 971 084 SCHATZMANN HJ J PHYSIOL (LOND) 201 369 969 085 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 086 SPOCK A PEDIATR RES 1 173 967 087 STEELE MW PEDIATR RES 5 393 971 088 TAUSSIG LM LANCET 1 1367 972 089 TAYSI K N ENGL J MED 281 1108 969 090 WELCH DW PEDIATR RES 9 698 975 091 WIESMANN UN J PEDIATR 77 685 970 CT 1 WARD JB TEX REP BIOL MED 34 83 976 2 ANON BR MED J 1 596 977 3 HOSLI P MONOGR PAEDIATR 10 90 979 4 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 PN 76216 RN 00569 AN 77060018 AU Mayo-B-J. Klebe-R-J. Lankford-B-J. Morris-N-R. Barnett-D-R. Bowman-B-H. TI Somatic cell genetic studies of cystic fibrosis. SO Tex-Rep-Biol-Med. 1976. 34(1). P 107-11. MJ CYSTIC-FIBROSIS: fg. MN ANIMAL. BLOOD-PROTEINS: ip. CELL-LINE. CILIA: ph. CLONE-CELLS. CULTURE-MEDIA. CYSTIC-FIBROSIS: me. ELECTROPHORESIS-STARCH-GEL. GLUCOSEPHOSPHATE-DEHYDROGENASE: an. HUMAN. HYBRID-CELLS: en. MICE. OYSTERS. SUPPORT-U-S-GOVT-P-H-S. AB The expression of the gene coding for the cystic fibrosis ciliary inhibitor has been examined in somatic cell hybrid clonal lines. Hybrids were produced by the fusion of cultured skin fibroblasts from cystic fibrosis patients with mouse RAG and LM (TK-) cell lines. The ciliary inhibitor was detected by the oyster ciliary assay in several primary hybrid clones but not detected in others, indicating the segregation of an associated nuclear gene. RF 001 BOLTON WE AM J HUM GENET 27 394 975 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 003 BOWMAN BH SCIENCE 164 325 969 004 CONOVER JH PEDIATR RES 7 224 973 005 DANES BS AM J HUM GENET 25 323 973 006 DAVIDSON RL NATURE 205 1170 965 007 KIT S EXP CELL RES 31 297 963 008 KLEBE RJ J CELL BIOL 45 74 970 009 LITTLEFIELD JW SCIENCE 145 709 964 010 NICHOLS EA J HISTOCHEM CYTOCHEM 21 1066 973 011 NYHAN WL PEDIATR RES 1 5 967 012 PUCK RR J EXP MED 103 273 956 013 WEISS MC PROC NAT ACAD SCI USA 58 1104 967 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 PN 76217 RN 00570 AN 77060017 AU Bowman-B-H. Barnett-D-R. TI Introduction: Current investigations and theories on the genetic defect in cystic fibrosis. SO Tex-Rep-Biol-Med. 1976. 34(1). P 1-9. (REVIEW). MJ CYSTIC-FIBROSIS: fg. MN ANIMAL. CILIA: ph. CYSTIC-FIBROSIS: en, me. GENES. HUMAN. MUCUS: ph. RABBITS. REVIEW. SUPPORT-U-S-GOVT-P-H-S. EX Recent research in cystic fibrosis has suggested at least two theories regarding the genetic defect underlying this prevalent disease: that a mutant gene in cystic fibrosis genotypes is responsible for the synthesis of an altered protein that interferes with membrane transport and mucociliary activity; and that a mutant gene in cystic fibrosis genotypes is responsible for an enzyme deficiency leading to the presence of undegraded metabolites that interfere with membrane transport. Circulating factors in cystic fibrosis and their effects on membrane transport and mucociliary systems are discussed, as are the search for an enzymatic deficiency and investigations of altered metabolic pathways. Comparison of somatic cells from cystic fibrosis and normal genotypes is also outlined. RF 001 ARVANITAKIS S TEX REP BIOL MED 34 175 976 002 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 003 BALFE JW SCIENCE 162 689 968 004 BARGMAN GJ TEX REP BIOL MED 34 37 976 005 BARNETT DR TEX REP BIOL MED 31 703 973 006 BARRANCO SC J CELL PHYSIOL 88 33 976 007 BAUR PS TEX REP BIOL MED 34 155 976 008 BAUR PS TEX REP BIOL MED 34 155 976 009 BERATIS NG PEDIATR RES 7 958 973 010 BOLTON WE TEX REP BIOL MED 34 97 976 011 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 012 BOWMAN BH RECENT ADVANCES IN CF RESEARC 12 976 013 BOWMAN BH LANCET 1 404 974 014 BOWMAN BH FED PROC 31 842 972 015 BUCHWALD M AM J HUM GENET 27 22A 975 016 CARSON SD TEX REP BIOL MED 34 209 976 017 COHEN LF PEDIATR RES 9 334 975 018 COLE CH PEDIATR RES 9 763 975 019 CONOD EJ PEDIATR RES 9 724 975 020 CONOVER JH LANCET 2 1501 973 021 CONOVER JH TEX REP BIOL MED 34 45 976 022 CONOVER JH PEDIATR RES 7 224 973 023 DANES BS TEX REP BIOL MED 34 135 976 024 DANES BS J EXP MED 136 1313 972 025 DANES BS J EXP MED 137 1538 973 027 GRISHAM CM BIOCHEMISTRY 12 2635 973 028 GURD JW BIOCHEM J 122 43P 971 029 HARPER BL TEX REP BIOL MED 34 73 976 030 LIEBERMAN J AM REV RESPIR DIS 109 399 974 031 LITT M TEX REP BIOL MED 34 151 976 032 MANGOS JA SCIENCE 158 135 967 033 MAYO BJ TEX REP BIOL MED 34 107 976 034 NEWHOUSE M IN: MANGOS JA 319 973 035 NOVAK RA TEX REP BIOL MED 34 199 976 036 RAO GJS PEDIATR RES 9 739 975 037 RENNERT OM TEX REP BIOL MED 34 187 976 039 SANCHIS J N ENGL J MED 288 651 973 040 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 041 SPOCK A PEDIATR RES 1 173 967 042 BOWMAN BH TEX REP BIOL MED 31 611 973 043 TOWNES PL AM J HUM GENET 27 91A 975 044 WARD JB JR TEX REP BIOL MED 34 11 976 045 WIDNELL CC PROC NAT ACAD SCI USA 61 1050 968 046 WILSON GB TEX REP BIOL MED 34 51 976 047 WOOD RE AM REV RESPIR DIS 111 733 975 CT 1 ANON BR MED J 1 596 977 2 KOLLBERG H ACTA PAEDIATR SCAND 68 639 979 3 THOMAS JM CLIN CHIM ACTA 111 199 981 PN 76218 RN 00571 AN 77060028 AU Bargman-G-J. TI Larval brine shrimp (Nauplii): a potentially useful model to study cystic fibrosis. SO Tex-Rep-Biol-Med. 1976. 34(1). P 37-43. MJ CYSTIC-FIBROSIS: me. MODELS-BIOLOGICAL. SHRIMP: me. MN ANIMAL. CYSTIC-FIBROSIS: fg. ELECTROLYTES: me. EXOCRINE-GLANDS: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. LARVA. OXYGEN-CONSUMPTION. SALIVA: me. AB One of the most consistent characteristics of cystic fibrosis is the abnormal handling of electrolytes by exocrine glands. The present study has examined the possibility that diminished respiratory energy formation is a primary biochemical error responsible for this abnormality. Using oxygen consumption of intact larval brine shrimp, quantitative differences have been observed from reaction of mixed mouth saliva between CF heterozygotes and CF homozygotes. In addition, information demonstrating a correlation between a biochemical abnormality and disease severity was observed. The differences observed in inhibition by saliva from both CF homozygotes and obligate heterozygotes may occur as the result of 1) interference with electron transfer from organic fuel molecules to molecular oxygen or 2) interference with the mechanism responsible for generating the biochemical reducing power necessary for multiple biosynthetic reactions. RF 001 DANES BS J EXP MED 129 775 969 002 CONOVER JH LANCET 1 1122 973 003 LOBECK CC IN: STANBURY JB 1605 972 004 BARGMAN GJ PEDIATR RES 8 464 974 005 CONTE FP J COMP PHYSIOL 80 239 972 006 CONTE FP J COMP PHYSIOL 82 277 973 007 EWING RD J COMP PHYSIOL 88 217 974 008 EWING RD J COMP PHYSIOL 80 247 972 009 HOOTMAN SR J COMP PHYSIOL 79 97 972 010 SHAPIRO SS BIOMETRICS 21 1030 965 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 GABRIDGE MG PEDIATR RES 13 31 979 3 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 76219 RN 00572 AN 77060029 AU Conover-J-H. Conod-E-J. Hirschhorn-K. TI On the nature of the defect in cystic fibrosis. SO Tex-Rep-Biol-Med. 1976. 34(1). P 45-50. MJ CYSTIC-FIBROSIS: bl. MN AMNIOTIC-FLUID: me. ANAPHYLATOXINS: me. ANIMAL. BIOLOGICAL-ASSAY. CILIA: ph. COMPLEMENT-3: an. CYSTIC-FIBROSIS: en, fg. FIBROBLASTS: me. HUMAN. IGG: me. LEUKOCYTES: me. LYMPHOCYTES: me. RABBITS. SUPPORT-U-S-GOVT-P-H-S. AB Sera and lymphocyte culture media derived from cystic fibrosis (CF)- affected and carrier subjects contain ciliary dyskinesia factor (CDF) detected by our rabbit tracheal bioassay. In addition, we also find CDF in fibroblast media from these same donors and in amniotic fluid cell media derived from CF carrier or affected fetuses. In these latter instances, the media were inactive in the bioassay, but became active when mixed with purified IgG. In all instances, CDF activity was eliminated by the addition of anti-IgG. We have separated a low molecular weight fraction, between 1,000 and 10,000 M.W., from CF sera and culture media which is inactive in the bioassay until IgG is added. Presumptive and indirect evidence indicates that this fraction behaves similarly to the complement derived anaphylatoxin C3a. In addition, we have found activity in sera from CF patients and, to a lesser extent, carriers that induces degranulation of cytochalasin-B- treated human polymorphonuclear leukocytes. Since this activity appears to be in a different molecular species from that containing CDF, we postulate that the primary defect in CF is the deficiency of an enzyme whose substrates include a family of membrane-active molecules. RF 001 BERATIS NG PEDIATR RES 7 958 973 002 BLOMFIELD J GUT 14 558 973 004 CONOVER JH PEDIATR RES 7 220 973 005 CONOVER JH PEDIATR RES 7 224 973 006 CONOVER JH LANCET 1 1194 973 007 CONOVER JH LANCET 2 1501 973 008 CONOVER JH LIFE SCI 14 253 974 009 DANKS DM ANN HUM GENET 28 323 965 010 DAVIS AT PROC SOC EXP BIOL MED 137 161 971 011 GOLDSTEIN IM J IMMUNOL 111 33 973 012 LOBECK CC IN: STANBURY JB 1605 972 013 MANGOS JA PEDIATR RES 1 436 967 014 MCCOMBS ML CLIN GENET 1 171 970 015 RAO GJS SCIENCE 177 610 972 016 SPOCK A PEDIATR RES 1 173 967 017 VALLOTA EH J EXP MED 137 1109 973 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 BOGART BI PEDIATR RES 12 15 978 3 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 4 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 5 WILSON GB MED HYPOTHESES 8 527 982 6 WOOTEN MW IN VITRO CELL DEVEL BIOL 21 207 985 PN 76220 RN 00573 AN 77060030 AU Wilson-G-B. Fudenberg-H-H. TI Further purification and characterization of serum proteins used to detect cystic fibrosis genotypes by isoelectric focusing. SO Tex-Rep-Biol-Med. 1976. 34(1). P 51-71. MJ BLOOD-PROTEINS: ip. CYSTIC-FIBROSIS: bl. ISOELECTRIC-FOCUSING. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CHROMATOGRAPHY-GEL. CYSTIC-FIBROSIS: fg. FEMALE. GENOTYPE. HUMAN. INFANT. MALE. MOLECULAR-WEIGHT. SUPPORT-U-S-GOVT-P-H-S. AB Sera from cystic fibrosis (CF) homozygotes and obligate heterozygotes contain a CF factor (gamma CF factor) not found by isoelectric focusing in thin-layer polyacrylamide gels in most normal control sera. In addition, sera from most obligate heterozygotes lack another protein (bland B, C, or D) that is commonly found in sera from most normal and cystic fibrosis individuals. A standardized, biophysical assay is described that employs isoelectric focusing for the detection of both CF homozygotes and heterozygotes based on the analysis of whole serum for the presence of the gamma CF factor and bands B, C, and D. Results of analyzing sera from selected CF patients by isoelectric focusing indicated that there is a general correlation between the amount of the gamma CF factor and the clinical severity of the disease. Partial purification and characterization of the gamma CF factor and protein bands B, C, and D was accomplished by using DEAE-cellulose chromatography, Sephadex G- 200 gel filtration, sequential molecular filtration through a series of Amicon Diaflo ultrafiltration membranes, affinity chromatography, and cellulose acetate electrophoresis. The gamma CF factor is a cationic protein with a pI of 8.46+/-0.05, has gamma electrophoretic mobility, a molecular weight between 3,500 and 10,000, and apparently exists in CF serum in 2 forms (free in solution and complexed to IgG). Bands B, C, and D are cationic proteins with pI values of 7.85 to 8.10, have gamma electrophoretic mobility and a molecular weight of approximately 100,000-150,000. RF 001 BARNETT DR PEDIATR RES 8 687 974 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BERATIS NG PEDIATR RES 7 958 973 004 BOWMAN BH IN: MANGOS JA 29 973 005 BOWMAN BH CLIN GENET 4 461 973 006 BOWMAN BH SCIENCE 167 871 970 007 CONOVER JH PEDIATR RES 7 220 973 008 CONOVER JH LANCET 1 47 975 009 CONOVER JH LANCET 1 1194 973 010 CONOVER JH LIFE SCI 14 253 974 011 GRAIG LC IN: WILLIAMS CA 2 119 968 012 CUATRECASAS P PROC NAT ACAD SCI USA 61 636 968 013 DANES BS J EXP MED 137 1538 973 014 EDER J J IMMUNOL METH 2 67 972 015 FAHEY JL J IMMUNOL 94 84 965 016 FUCHS S IN: WEIR DM 1 973 017 GIBSON LE PEDIATRICS 23 545 959 018 LOCKHART LH TEX REP BIOL MED 31 631 973 019 MANCINI G IMMUNOCHEMISTRY 2 235 965 020 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 021 SHWACHMAN H IN: KENDIG EL JR 541 967 022 SPOCK A PEDIATR RES 1 173 967 023 WILSON GB DISSERT ABST INTERNAT 35 479 974 024 WILSON GB PEDIATR RES 10 87 976 025 WILSON GB PEDIATR RES 9 635 975 026 WILSON GB CF CLUB ABST 15 1 974 027 WILSON GB CLIN CHIM ACTA 49 79 973 028 WILSON GB CLIN RES 24 295 976 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 WILSON GB PEDIATR RES 11 139 977 3 WILSON GB PEDIATR RES 11 317 977 4 WILSON GB PEDIATR RES 11 986 977 5 WILSON GB NATURE 266 463 977 6 WILSON GB PEDIATR RES 12 801 978 7 WILSON GB PEDIATR RES 13 1079 979 8 BLITZER MG PEDIATR RES 16 938 982 9 BLITZER MG PEDIATR RES 16 203 982 10 SHAPIRA E ANN NY ACAD SCI 421 352 983 PN 76221 RN 00574 AN 77060031 AU Harper-B-L. Barnett-D-R. Bissett-J-D. TI Immunochemical studies of the plasma and cultured fibroblast media fractions containing the cystic fibrosis ciliary inhibitor. SO Tex-Rep-Biol-Med. 1976. 34(1). P 73-82. MJ CILIA: ph. CYSTIC-FIBROSIS: me. MN ANIMAL. BLOOD-PROTEINS: an. CULTURE-MEDIA. CYSTIC-FIBROSIS: bl. FIBROBLASTS: me. HEMAGGLUTINATION-TESTS. HOMOZYGOTE. HUMAN. IGG: an. IMMUNE-SERA. LIPOPROTEINS-HDL: an. LIPOPROTEINS-LDL: an. RABBITS. SERUM-ALBUMIN: an. AB The cystic fibrosis ciliary inhibitor (CFCI) has been fractionated from plasma of cystic fibrosis (CF) homozygotes and from the media of cultured fibroblasts derived from CF homozygotes. Plasma and fibroblast media from normal controls have been fractionated in an identical manner. Fractions from plasma and fibroblast culture media that demonstrate ciliary inhibitory activity contain several proteins in a molecular weight range of approximately 5,000-11,000. These proteins have been partially characterized by immunochemical analysis with antisera to 33 human serum proteins. Immunological determinants of albumin, C3 (but not C3a), C4, C5, alpha1-lipoprotein, beta- lipoprotein, beta2-microglobulin and immunoglobulin light chains have been detected by hemagglutination in fractions of CF plasma that inhibited ciliary activity and in analogous fractions from normal sera. None of the proteins were detected in media of cultured fibroblasts from either genotype. Since the same proteins and protein fragments were identified in both CF and normal plasma fractions, and were not detected in CF fibroblast media, it appears that none of these proteins can be identified as the CFCI. Identification of these proteins will permit further purification of the CFCI by immunochemical methods. RF 001 AOKI K BIOCHEMISTRY 14 3566 975 002 BARNETT DR PEDIATR RES 8 687 974 003 BARNETT DR TEX REP BIOL MED 31 703 973 004 BARNETT DR CF CLUB ABST 5 974 005 BARNETT DR TEX REP BIOL MED 31 709 973 006 BELLON F BIOCHEM J 147 585 975 007 BERATIS NG PEDIATR RES 7 958 973 008 BOLTON WE AM J HUM GENET 27 394 975 009 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 010 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 011 BOWMAN BH SCIENCE 164 325 969 012 BOWMAN BH SCIENCE 167 871 970 013 CARSON SD TEX REP BIOL MED 34 209 976 014 CONOD EJ PEDIATR RES 9 724 975 015 CONOVER JH LANCET 2 1501 973 016 CONOVER JH LIFE SCI 14 253 974 017 CUNNINGHAM BA BIOCHEMISTRY 12 4811 973 018 DANES BS J EXP MED 137 1538 973 019 JOUSTRA M PROTIDES BIOL FLUIDS 17 511 969 020 LACHMAN PJ IN: WEIR DM 973 021 LIEBERMAN J AM REV RESPIR DIS 111 100 975 022 ONKELINX E IMMUNOLOGY 16 35 969 023 PETERS T JR ADV CLIN CHEM 13 37 970 024 PERPER RJ PROC SOC EXP BIOL MED 125 575 967 025 SCANLIN TF JR LANCET 1 1382 974 026 WILSON WD BIOCHEMISTRY 10 1772 971 027 WEIR DM HANDBOOK OF EXPERIMENTAL IMMU 973 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 CARSON SD TEX REP BIOL MED 34 209 976 3 BURDON MG CLIN GENET 17 249 980 4 BOWMAN BH FED PROC 39 3195 980 5 CARSON SD BIOCHIM BIOPHYS ACTA 667 23 981 6 CARSON SD PEDIATR RES 16 13 982 PN 76222 RN 00575 AN 77060032 AU Ward-J-B-Jr. Bowman-B-H. TI Surface enzymes in cultured fibroblasts from cystic fibrosis patients. SO Tex-Rep-Biol-Med. 1976. 34(1). P 83-96. MJ CYSTIC-FIBROSIS: en. MN ADENOSINE-MONOPHOSPHATE. ADENOSINE-TRIPHOSPHATASE: an. ANIMAL. CELL-FRACTIONATION. FIBROBLASTS: en. GLUCOSEPHOSPHATE-DEHYDROGENASE: an. HUMAN. NITROPHENYL-PHOSPHATASE: an. OYSTERS. PHOSPHATASES: an. SUPPORT-U-S-GOVT-P-H-S. AB Membrane function was examined in cultured cells from cystic fibrosis patients by assaying several enzymes on intact skin fibroblasts attached to culture dishes. This technique required few cells and minimized disruption of cellular organization. Comparison of enzyme activities of intact and broken cells showed that 12% of total glucose-6-phosphate dehydrogenase, a cytoplasmic enzyme, was measurable using intact cells, while all adenosine monophosphatase was measurable using intact cells. Alkaline paranitrophenylphosphatase activity was divided between the cell surface and interior. Substrate competition experiments indicated that substrate specificities for adenosine monophosphatase and paranitrophenylphosphatase activities were different. Adenosine monophosphatase activities of 2 control and 2 cystic fibrosis strains fluctuated similarly during the cell culture cycle. The apparent Km values relative to adenosine monophosphate were similar in all strains. A chromatographic fraction of serum from a cystic fibrosis patient that was inhibitory to oyster ciliary activity had no effect on adenosine monophosphatase activity of normal fibroblasts. Furthermore, fractions of media from cystic fibrosis homozygote and heterozygote fibroblast cultures were not inhibitory to adenosine monophosphatase activities of intact normal fibroblasts or of part iculate fractions prepared from them. In light of previous studies that showed that factors from cystic fibrosis serum of culture medium disrupted specific membrane activities, it is proposed that the cystic fibrosis factor interacts with the plasma membrane, interfering most conspicuously with the protein functions that are sensitive to changes in their membrane environment. RF 001 BERATIS NG PEDIATR RES 7 958 973 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BOLTON WE AM J HUM GENET 27 394 975 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 006 BOWMAN BH SCIENCE 164 325 969 007 BOWMAN BH SCIENCE 167 871 970 008 BUTLER KW BIOCHEM BIOPHYS RES COMMUN 40 770 970 009 CUATRECASAS P ANNU REV BIOCHEM 43 169 974 010 DANES BS J EXP MED 129 775 969 011 DANES BS J EXP MED 136 1313 972 012 DEPIERRE J IN: LEPOW IH 55 972 013 DEPIERRE JW SCIENCE 183 1096 974 014 DI SANTAGNESE PA N ENGL J MED 277 1287 967 015 EDIDIN M SYMP SOC EXP BIOL 28 1 974 016 FISKE CH J BIOL CHEM 66 375 925 017 GRISHAM CM BIOCHEMISTRY 12 2635 973 018 GURD JW BIOCHEM J 122 43P 971 019 HAM RG EXP CELL RES 29 515 963 020 JOHANSEN PG LANCET 1 455 968 021 LOWRY OH J BIOL CHEM 193 265 951 022 MANGOS JA SCIENCE 158 135 967 023 MANGOS JA PEDIATR RES 2 378 968 024 MANGOS JA PEDIATR RES 1 436 967 025 PLAGEMANN PGW BIOCHIM BIOPHYS ACTA 344 263 974 026 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 027 SHEPPARD JR J CELL PHYSIOL 85 163 975 028 SINGER SJ SCIENCE 175 720 972 029 TENNEY SR ARCH BIOCHEM BIOPHYS 126 53 968 030 WARD JB JR TEX REP BIOL MED 34 11 976 031 WIDNELL CC PROC NAT ACAD SCI USA 61 1050 968 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 PN 76223 RN 00576 AN 77060033 AU Bolton-W-E. Barranco-S-C. TI Review of the growth characteristics of cystic fibrosis and normal human diploid fibroblasts in vitro. SO Tex-Rep-Biol-Med. 1976. 34(1). P 97-105. MJ CYSTIC-FIBROSIS: pa. MN CELL-DIVISION. CELL-LINE. DIPLOIDY. FIBROBLASTS: pa. HETEROZYGOTE. HOMOZYGOTE. HUMAN. KINETICS. MODELS-BIOLOGICAL. TIME-FACTORS. SUPPORT-U-S-GOVT-P-H-S. AB The growth kinetics of human diploid skin fibroblasts derived from cystic fibrosis (CF) homozygotes, CF heterozygotes and normal individuals was determined. The population doubling times increased with time in culture, and no difference was observed between the 3 genotypes tested. The cell cycle times remained constant through the 10th subculture, while the growth fraction, or fraction of cells in the cell cycle, decreased with culture time; however, changes in the growth fraction and population doubling time appear to be related to cellular senescence in vitro rather than to cystic fibrosis. RF 001 BARRANCO SC CANCER RES 33 691 973 002 BARRANCO SC CANCER RES 35 1194 975 003 BARNETT DR TEX REP BIOL MED 31 691 973 004 BESLEY GTN J MED GENET 6 278 969 005 BOLTON WE AM J HUM GENET 27 394 975 006 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 007 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 008 DANES BS J EXP MED 136 1313 972 009 DELLORCO RT EXP CELL RES 84 363 974 010 DELLORCO RT EXP CELL RES 77 356 973 011 GOLDSTEIN S EXP CELL RES 88 359 974 012 HAM RG EXP CELL RES 29 515 963 013 HAY RJ ADV GERONTOL RES 2 121 967 014 HAY RJ EXP GERONTOL 3 35 968 015 HAYFLICK L EXP CELL RES 37 614 965 016 HAYFLICK L EXP CELL RES 25 585 961 017 HAYFLICK L EXP GERONTOL 5 291 970 018 HODES ME PEDIATR RES 8 212 974 019 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 020 HOWARD A HEREDITY SUPPL 6 261 953 021 KLAGSBRUN M PEDIATR RES 8 205 974 022 MACIEIRA-COELHO A EXP CELL RES 42 673 966 023 MCHALE JS EXP GERONTOL 6 89 971 024 MENDELSOHN ML J NATL CANCER INST 28 1015 962 025 PUCK TT J EXP MED 106 145 957 026 REED GB J PATHOL 101 251 970 027 RENNERT OM CLIN PEDIATR 11 351 972 028 RUSSELL SB J MED GENET 8 441 971 029 SPOCK A PEDIATR RES 1 173 967 030 TAYSI K N ENGL J MED 281 1108 969 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 WARD JB TEX REP BIOL MED 34 11 976 3 FEIGAL RJ PEDIATR RES 13 764 979 PN 76224 RN 00577 AN 77105326 AU Clarke-C-W. TI Aspects of serum and sputum antibody in chronic airways obstruction. SO Thorax. 1976 Dec. 31(6). P 702-7. MJ AIRWAY-OBSTRUCTION: im. ANTIBODIES: an. SPUTUM: im. MN BRONCHITIS: im. CHRONIC-DISEASE. CYSTIC-FIBROSIS: im. HUMAN. IGA: an. IGG: an. IGM: an. SPUTUM: mi. AB Immunoglobulin levels and precipitating antibody against a range of microbial antigens were measured in simultaneously collected serum and sputum samples from patients with chronic bronchitis (11), cystic fibrosis (9), bronchiectasis (9), and asthma (4). Sputum was prepared by dialysis and high-speed centrifugation methods. Results showed that it was possible to detect precipitating antibody in the sputum, and the rate was increased when both methods were used. A discrepancy was noted between the detection rate in the sputum and serum. This, combined with the lack of correlation between sputum and serum immunoglobulins, lack of relationship between bronchial inflammation and sputum immunoglobulins, and the lack of IgM in the sputum suggested that the antibody and immunoglobulin were locally produced. Sputum IgA (7S) in patients with chronic bronchitis was significantly lower (P less than 0-05) than that found in patients with cystic fibrosis and bronchiectasis. Significant differences (P less than 0- 05) were also noted in serum IgG levels between patients with chronic bronchitis, bronchiectasis, and cystic fibrosis while serum IgM levels in patients with chronic bronchitis were significantly lower (P less than 0-05) when compared to serum levels in patients with cystic fibrosis. The presence of precipitating antibody in the sputum raises the possibility that type III reactions may be important in the pathogenesis of these conditions. RF 001 AUSTRIAN R YALE J BIOL MED 40 400 968 002 BIBERFELD G ACTA PATH MICROBIOL SCAND (B) 79 599 971 003 BRANEFORS-HELANDER P ARCH ALLERG APPL IMMUN 44 585 973 004 BROCKLEHURST WE IN: ORIE NGM 279 970 005 BROGAN TD THORAX 30 72 975 006 BRUMFITT W LANCET 2 1306 957 007 CLARKE CW POSTGRAD MED J 51 147 975 008 COLTON T STATISTICS IN MEDICINE 174 974 009 DEUSCHL H CLIN EXP IMMUNOL 16 401 974 010 FALK GA AM REV RESPIR DIS 105 14 972 011 GOTZE O J EXP MED 134 90S 971 012 GUMP DW ARCH INTERN MED 132 847 973 013 KATZ RM N ENGL J MED 288 233 973 014$ KILBURN KH CHEST SUPPL 61S 65 4 974 015 LEES AW LANCET 2 1112 959 016 MCFARLANE H BR MED J 1 423 975 017 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 018 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 972 019 MAY JR POSTGRAD MED J 51 144 975 020 MEDICI TC AM REV RESPIR DIS 103 784 971 021 MEREDITH WM BASIC MATHEMATICAL AND STATIS 288 967 022 PARISH WE SYMP SOC GEN MICROBIOL 22 157 972 023 REED CE YALE J BIOL MED 40 507 968 024 RYLEY HC BR J EXP PATHOL 49 625 968 025 RYLEY HC J CLIN PATHOL 26 852 973 026 SHORE SL THORAX 28 721 973 027 SMITH H SYMP SOC GEN MICROBIOL 22 1 972 028 SZETIVANYI I IN: SAMTER M 1 356 971 029 TAGER I N ENGL J MED 292 563 975 030 VAN DER ZAWAN JC CLIN ALLERGY 5 225 975 031 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 032 WARREN CPW AM REV RESPIR DIS 109 672 974 033 WITTELS EH AM REV RESPIR DIS 109 502 974 CT 1 CLARKE CW CLIN ALLERGY 7 527 977 2 TURNBULL LS CLIN EXP IMMUNOL 32 226 978 3 NASH DR ANN CLIN RES 11 49 979 4 PALUCH E INT J CANCER 23 42 979 5 MAYANSKY AN ZH MIKROBIO EPIDEMIO IMMUNOBI 1979 14 979 6 STOCKLEY RA THORAX 35 202 980 7 STOCKLEY RA J IMMUNOL METH 38 151 980 8 STOCKLEY RA AM REV RESPIR DIS 122 959 980 9 PUCHELLE E BIORHEOLOGY 18 659 981 10 CLARKE CW THORAX 36 665 981 11 PRYJMA J ANN ALLERGY 54 60 985 PN 76225 RN 00578 AN 76154742 AU Kerr-A-A. TI Dead space ventilation in normal children and children with obstructive airways diease. SO Thorax. 1976 Feb. 31(1). P 63-9. MJ AIRWAY-OBSTRUCTION: pp. RESPIRATORY-DEAD-SPACE. MN ADOLESCENCE. ASTHMA: pp. BODY-HEIGHT. CARBON-DIOXIDE: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. HUMAN. INSPIRATORY-CAPACITY. LUNG: ph, pp. RESPIRATORY-FUNCTION-TESTS: mt. TIDAL-VOLUME. AB Anatomical dead space was measured in 72 normal children aged from 5 to 16 years, using the single breath method. There was a linear increase in this measurement with height, weight, and end-inspiratory lung volume. Physiological dead space was measured in 52 normal children using the Bohr equation and substituting a rebreathing PCO2 for alveolar PCO2. There was a parallel increase in this measurement with height, weight, and end-inspiratory lung volume. The difference between the two dead space measurements constitutes the alveolar dead space and was constant over the whole age range at 45 +/- 22 ml. The ratio of physiological dead space to tidal volume was 33-6 +/-4-6% and was unaltered by age or change in lung volume. The effect of airways obstruction on the dead space volumes was studied in 36 children with asthma and 28 with cystic fibrosis. Physiological dead space increased with increasing airways obstruction. Anatomical dead space remained constant in spite of marked increases in lung volume associated with the airways obstruction. RF 001 BARTELS J J CLIN INVEST 33 41 954 002 BEAUDRY PH AM REV RESPIR DIS 95 248 967 003 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 004 COLLIER CR J APPL PHYSIOL 9 25 956 005 COMROE JH JR LUNG CLINICAL PHYSIOLOGY AND 962 006 COOK CD J CLIN INVEST 34 975 955 007 COOK CD IN: SMITH RM 32 968 008 DOWNES JJ PEDIATR CLIN NORTH AM 19 423 972 009 DUBOIS AB J CLIN INVEST 35 322 956 010 FOWLER WS AM J PHYSIOL 154 405 948 011 HARRIS EA CLIN SCI MOL MED 15 375 973 012 HART MC J APPL PHYSIOL 18 519 963 013 LEVINE G N ENGL J MED 282 1277 970 014 LEVISON H AM REV RESPIR DIS 101 972 970 015 LIFSHAY A J APPL PHYSIOL 31 478 971 016 MCEVOY JDS BR MED J 4 687 974 017 MELLEMGAARD K ACTA PHYSIOL SCAND 67 10 966 018 NELSON NM PEDIATRICS 30 963 962 019 OLSEN CR J APPL PHYSIOL 23 27 967 020 POLGAR G PULMONARY FUNCTION TESTING IN 971 021 RADFORD EP JR J APPL PHYSIOL 7 451 955 022 RAINE JM J APPL PHYSIOL 18 284 963 023 ROBINSON S ARBEITSPHYSIOLOGIE 10 251 938 024 SHEPARD RH J APPL PHYSIOL 18 284 957 025 STRANG LB CLIN SCI 21 107 961 026 YOUNG AC J APPL PHYSIOL 8 91 955 CT 1 SWINSCOW TDV BR MED J 2 680 976 2 HANCOX AJ MED BIOL ENG COMPUT 20 58 982 3 REISER J PEDIATR PULMONOL 2 237 986 PN 76226 RN 00579 AN 77060072 AU Matthay-M-A. Matthay-R-A. Mills-D-M. Lakshminarayan-S. Cotton-E. TI Hypertrophic osteoarthropathy in adults with cystic fibrosis. SO Thorax. 1976 Oct. 31(5). P 572-5. MJ CYSTIC-FIBROSIS: co. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: di. MN ADULT. ARTHRITIS: di. CASE-REPORT. CYSTIC-FIBROSIS: ra. DIAGNOSIS-DIFFERENTIAL. FIBULA: ra. HUMAN. MALE. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: ra. TIBIA: ra. AB Three adult patients with cystic fibrosis presented with arthralgia, and investigation for the usual causes of arthritis proved negative. Radiographs of long bones revealed periostitis and new bone fromation characteristic of hypertrophic osteoarthropathy. Symptomatic improvement occurred after analgesic and anti-inflammatory therapy. In patients with cystic fibrosis and bone or joint pain, the diagnosis of hypertrophic osteoarthropathy should be considered and long bone radiographs obtained. RF 001 BAMBERGER E WIEN KLIN WOCHENSCHR 2 226 889 002 COATES EO JR DIS CHEST 49 195 966 003 FISCHER DS MEDICINE 43 459 956 004 GOODMAN DH ANN ALLERGY 25 224 967 005 GROSSMAN H AM J DIS CHILD 107 1 964 006 HAMMARSTEN JF ARCH INTERN MED 99 421 957 007 JONES JD MAYO CLIN PROC 45 768 970 008 KOPEL FB GASTROENTEROLOGY 62 483 972 009 MARIE P REV MEDEC 10 1 890 010 SHAPIRO RF CHEST 63 912 972 011 STANLEY NN BR HEART J 33 469 971 012 TREVER RW ANN INTERN MED 48 660 958 CT 1 ANON BR MED J 2 785 977 2 SAGRANSKY DM AM J DIS CHILD 134 319 980 3 DELUMLEY L ARCH FR PEDIATR 40 723 983 4 BRADFORD R BR J DIS CHEST 78 275 984 5 BRAUDE S BR MED J 288 822 984 6 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 7 SUMMERS GD BR J RHEUMATOL 25 393 986 8 PHILLIPS BM J ROY SOC MED 79 44 986 9 COHEN AM AM J DIS CHILD 140 74 986 PN 76227 RN 00580 AN 76272091 AU Islam-N. TI The differential diagnosis of bronchial asthma in children. SO Trop-Doct. 1976 Jul. 6(3). P 105-7. MJ ASTHMA: di. MN ACUTE-DISEASE. BRONCHIOLITIS-VIRAL: di. BRONCHITIS: di. BRONCHOGRAPHY. CHILD. CYSTIC-FIBROSIS: di. DIAGNOSIS-DIFFERENTIAL. FOREIGN-BODIES: ra. HELMINTHIASIS: di. HUMAN. PERIARTERITIS-NODOSA: di. TUBERCULOSIS-PULMONARY: di. AB Bronchial asthma in children is not uncommon in the tropics. Conditions simulating this disease in the tropics have been discussed with a view to arriving at a diagnosis without sophisticated investigations. RF 001 HASAN M DISSERTATION 975 002 ISLAM N TROPICAL EOSINOPHILIA 96 964 003 MOSLER CITED IN: LEUCKART R 2 222 876 004 TULLIS DCH N ENGL J MED 282 370 970 PN 76228 RN 00581 AN 77129620 AU Bieri-J-G. Farrell-P-M. TI Vitamin E. SO Vitam-Horm. 1976. 34. P 31-75. (REVIEW). MJ VITAMIN-E: me. MN ANIMAL. ANTIOXIDANTS: me. BLOOD-COAGULATION. CORONARY-DISEASE: me. CYSTIC-FIBROSIS: me. DIET. DIETARY-FATS: me. ERYTHROCYTES: me. FATTY-ACIDS-UNSATURATED: me. HEMOGLOBINS: me. HUMAN. INFANT-NEWBORN. INFANT-PREMATURE. LIPOPROTEINS: me. LYMPH: me. MALABSORPTION-SYNDROMES: me. PROTEIN-CALORIE-MALNUTRITION: me. PROTEINS: bi. RETROLENTAL-FIBROPLASIA: me. REVIEW. CELIAC-DISEASE: me. VITAMIN-E-DEFICIENCY: me, th. VITAMIN-E: tu. EX In this review, we make a deliberate attempt to put all the information on vitamin E requirement, absorption, metabolism, and function in both animals and man in better perspective. The large clinical trials in which vitamin E was given at pharmacological levels for presumed medical indications, as well as trials of vitamin E for bona fide deficiency disease in man, will be extensively reviewed. Nutritional aspects discussed include dietary intake, biological activity, the evaluation of nutritional status, and the relationship to dietary polyunsaturated fatty acids. Metabolism concerns include absorption, transport and deposition, and cellular function. Clinical aspects include deficiency states, approaches to therapy, and possible pharmacological effects. RF 001 ABRAMS AA N ENGL J MED 272 1080 965 002 ABRAMS BA ARCH DIS CHILD 48 721 973 003 ANDERSON TW CAN MED ASSOC J 110 401 974 004 ANDERSON TW AM J CLIN NUTR 27 1174 974 005 ARMSTRONG D AM NEUROL ASSOC 98 3 973 006 BAER S JAMA 139 733 949 007 BAKER SJ BLOOD 32 717 968 008 BARNESS LA AM J CLIN NUTR 21 40 968 009 BASSEN FA BLOOD 5 381 950 010 BAUERNFEIND JC AM J CLIN NUTR 27 234 974 011 BIERI JG IN: DE LUCA HF 307 969 012 BIERI JG ANN NY ACAD SCI 203 181 972 013 BIERI JG J AM DIET ASSOC 62 147 973 014 BIERI JG FED PROC 34 913 975 015 BIERI JG PROC SOC EXP BIOL MED 149 500 975 016 BIERI JG J AM DIET ASSOC 66 134 975 017 BIERI JG J NUTR 100 557 970 018 BIERI JG INT J VITAM RES 40 344 970 019 BIERI JG PROC SOC EXP BIOL MED 120 554 965 020 BIERI JG PROC SOC EXP BIOL MED 117 131 964 021 BIERI JG J NUTR 106 124 976 022 BINDER HJ N ENGL J MED 273 1289 965 023 BLANC WA PEDIATRICS 22 494 958 024 BLOMSTRAND R INT J VITAM RES 38 328 968 025 BOYD AM J BONE JOINT SURG 31B 325 949 026 BRUBACHER G WISS VEROEFF DTSCH GES ERNAEH 16 50 967 027 BUNNELL RH AM J CLIN NUTR 17 1 965 028 BUNYAN J BIOCHEM J 77 47 960 029 BURK RF US ARMY MED RES NUTR LAB REP 335 973 030$ CANTAROW A CLIN BIOCHEM 180 962 031 CATIGNANI GL BIOCHEM BIOPHYS RES COMMUN 67 66 975 032 CATIGNANI GL IN: MACHLIN LJ 976 033 CATIGNANI GL PROC NAT ACAD SCI USA 71 1966 974 034 CHADD MA INT J VITAM RES 40 610 970 035 CHEEKE PR CAN J ANIM SCI 49 169 969 036 CURETON TK AM J PHYSIOL 179 628 954 037 CURRAN JS PEDIATR RES 9 364 975 038 DAM H VITAM HORM 20 527 962 039 DARBY CW ARCH DIS CHILD 48 72 973 040 DARBY WJ PROC SOC EXP BIOL MED 63 310 946 041 DARBY WJ ANN NY ACAD SCI 52 328 949 042 DAVIES T CLIN CHIM ACTA 24 431 969 043 DAVIS KC AM J CLIN NUTR 25 933 972 044 DE LEON AS PEDIATR CLIN NORTH AM 17 309 970 045 DE VILLERS A CAN J BIOCHEM 51 450 973 046 DICKS-BUSHNELL MW AM J CLIN NUTR 20 262 967 047 DILLEY RA J MEMBR BIOL 2 317 970 048 DINNING JS VITAM HORM 20 511 962 049 DIPLOCK AT AM J CLIN NUTR 27 995 974 050 DI SANTAGNESE PA N ENGL J MED 277 1399 967 051 EVARTS RP LIPIDS 9 860 974 052 FARRELL PM AM J CLIN NUTR 28 1381 975 053 FARRELL PM ACTA PAEDIATR SCAND 64 150 975 054 FERGUSON ME J NUTR 55 205 955 055 FILER LJ JR AM J CLIN NUTR 21 3 968 056 FILER LJ JR PEDIATRICS 8 328 951 057 ANON RECOMMENDED DIETARY ALLOWANCE 968 058 ANON RECOMMENDED DIETARY ALLOWANCE 974 059 FUKUBA H IN: SHIMAZONO N 63 972 060 FUKUBA H PROC SYMP ON INFLUENCE OF ENV 975 061 GALLO-TORRES HE LIPIDS 5 379 970 062 GLOOR U HELV CHIM ACTA 49 2303 966 063 GOLDBLOOM RB CAN MED ASSOC J 82 1114 960 064 GOLDBLOOM RB PEDIATRICS 32 36 963 065 GORDON HH AM J DIS CHILD 62 328 941 066 GORDON HH IN: WOHL MG 238 968 067 GORDON HH AM J DIS CHILD 90 669 955 068 GORDON HH PEDIATRICS 21 673 958 069 GROSS S ANN NY ACAD SCI 203 141 972 070 HAEGER K VASA 2 280 973 071 HAEGER K AM J CLIN NUTR 27 1179 974 072 HALSTEAD CH AM J CLIN NUTR 22 1371 969 073 HAMILTON M LANCET 1 367 953 074 HARRIES JT ARCH DIS CHILD 46 341 971 075 HARRIS PL PROC SOC EXP BIOL MED 107 381 961 076 HARRIS RS VITAM HORM 20 603 962 077 HASHIM SA AM J CLIN NUTR 21 7 968 078 HASSAN H AM J CLIN NUTR 19 147 966 079 HERTING DC AM J CLIN NUTR 22 147 969 080 HODGES RE J AM DIET ASSOC 62 638 973 081 HOEKSTRA WG FED PROC FED AM SOC EXP BIOL 34 2083 975 082 HORWITT MK AM J CLIN NUTR 8 451 960 083 HORWITT MK VITAM HORM 20 541 962 084 HORWITT MK AM J CLIN NUTR 27 1182 974 085 HORWITT MK VITAM HORM 26 487 968 086 HORWITT MK ANN NY ACAD SCI 203 223 972 087 HYMAN CB ANN NY ACAD SCI 232 211 974 088 IKEHATA H VITAMINS 38 253 968 089 JAGER FC ANN NY ACAD SCI 203 199 972 090 JOHNSON L AM J CLIN NUTR 27 1158 974 091 JOHNSON P LIFE SCI 1 115 962 092 KANNO C AGRIC BIOL CHEM 34 878 970 093 KATER RMH AM J CLIN NUTR 23 913 970 094 KATTWINKEL J J PEDIATR 82 234 973 095 KAYDEN HJ ANNU REV MED 23 285 972 096 KAYDEN HJ TRANS ASSOC AM PHYSICIANS 78 334 965 097 KELLEHER J BR J NUTR 24 1033 970 098 KERNER I AM J DIS CHILD 99 597 960 099 KINSEY VE ARCH OPHTHALMOL 56 481 956 100 KINSEY VE AM J OPHTHALMOL 34 1259 951 101 KLATSKIN G J CLIN INVEST 31 159 952 102 KULAPONGS P IN: OLSON RE 263 975 103 LARSSON H PHARMACOL CLIN 1 72 968 104 LEONARD PJ AM J CLIN NUTR 20 795 967 105 LEONARD PJ AM J CLIN NUTR 24 388 971 106 LEONARD PJ GUT 7 578 966 107 LEVIN S PEDIATRICS 27 578 961 108 LIVINGSTONE PD LANCET 2 602 958 109 LO SS ARCH DIS CHILD 48 360 973 110 LOSOWSKY MS ANN NY ACAD SCI 203 212 972 111 LUCY JA ANN NY ACAD SCI 203 4 972 112 MCCORMICK EC J LIPID RES 1 221 960 113 MACDOUGALL LG J PEDIATR 80 775 972 114 MACHLIN LF PROC SOC EXP BIOL MED 149 275 975 115 MACKENZIE JB PEDIATRICS 13 346 954 116 MCLAREN DS AM J CLIN NUTR 22 863 969 117 MACMAHON MT CLIN SCI 38 197 970 118 MACMAHON MT EUR J CLIN INVEST 1 161 970 119 MACMAHON MT EUR J CLIN INVEST 1 288 970 120 MCWHIRTER WR ACTA PAEDIATR SCAND 64 446 975 121 MAJAJ AS AM J CLIN NUTR 12 374 963 122 MARKS J VITAM HORM 20 573 962 123 MASON KE IN: SEBRELL WH JR 5 293 972 124 MELHORN DK J LAB CLIN MED 74 798 969 125 MELHORN DK J PEDIATR 79 581 971 126 MELHORN DK BLOOD 37 438 971 127 MELLORS A BR J NUTR 20 69 966 128 MENGEL CE J CLIN INVEST 46 1715 967 129 MINOT AS J LAB CLIN MED 29 772 944 130 MODELL CB BR MED J 3 259 974 131 MOLENAAR I PROC NAT ACAD SCI USA 61 982 969 132 MOLENAAR I VITAM HORM 30 45 972 133 MOYER WT PEDIATRICS 6 893 950 134 MULLER DPR BIOCHEM J 112 28P 969 135 MULLER DPR GUT 15 966 974 136 MUNSAT TL JAMA 226 1536 973 137 MUSTAJOKI P JAMA 221 714 972 138 NAIR PP ARCH INTERN MED 128 411 971 139 NEWMARK HL J PHARM SCI 64 655 975 140 NITOWSKY HM AM J DIS CHILD 92 164 956 141 NITOWSKY HM AM J CLIN NUTR 10 368 962 142 NORDOY A J LIPID RES 16 386 975 143 ANON NUTRITION SURVEY OF EAST PAKI 966 144 OLSON RE AM J CLIN NUTR 20 604 967 145 OLSON RE CIRCULATION 48 179 973 146 OLSON RE AM J CLIN NUTR 27 1117 974 147 OLSON RE IN: OLSON RE 275 975 148 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 149 OSKI FA J PEDIATR 70 211 967 150 OSKI FA AM J CLIN NUTR 21 45 968 151 OSKI FA HEMATOLOGIC PROBLEMS IN THE N 972 152 OWENS WC AM J OPHTHALMOL 32 1 949 153 PANOS TC AM J CLIN NUTR 21 15 968 154 PATNAIK RN EXPERIENTIA 31 1023 975 155 PEAKE IR BIOCHIM BIOPHYS ACTA 260 679 972 156 PEARSON CK BR J NUTR 24 581 970 157 POUKKA RKH LIPIDS 5 757 970 158 RAJARAM OV BIOCHEM J 140 509 974 159 RAMIREZ I AM J CLIN NUTR 26 1045 973 160 RATCLIFFE AH LANCET 2 1128 949 161 RAVIN IS N ENGL J MED 240 331 949 162 RICHARDS JE PEDIATRICS 20 92 957 163 RINZLER SH CIRCULATION 1 288 950 164 ROSE CS AM J PHYSIOL 168 414 952 165 RUBINSTEIN HM CLIN CHIM ACTA 23 1 968 166 SANDSTEAD HH AM J CLIN NUTR 17 27 965 167 SARTAIN P SOUTH MED J 60 1371 967 168 SCHMANDKE H INT Z VITAMINFORSCH 34 312 964 169 SCRIMSHAW NS JAMA 164 555 957 170 SCRIVER CR PEDIATRICS 46 493 970 171 SHARMAN IM BR J NUTR 26 265 971 172 SHUTE WE MED REC 160 91 947 173 SILBER R J CLIN INVEST 48 2089 969 174 SIMON ER J CLIN INVEST 43 1311 964 175 SMITH CL BR J NUTR 26 89 971 176 STEINER M J CLIN INVEST 57 732 976 177 STOCKMAN JA SEMIN HEMATOL 12 163 975 178 TAYLOR BW ARCH DIS CHILD 48 657 973 179 THANANGKUL O AM J CLIN NUTR 18 379 966 180 THOMPSON JN AM J CLIN NUTR 26 1349 973 181 UNDERWOOD BA PEDIATR RES 6 26 972 182 VOGELSANG A NATURE 157 772 946 183 VOGTMANN H NUTR METAB 13 271 971 184 WATSON CJ ARCH INTERN MED 131 698 973 185 WEBER F NUTR DIETA 8 54 966 186 WEBER F Z ERNAEHRUNGSWISS 245 1 964 187 WEINBERG T AM J PATHOL 34 565 958 188 WEISS P AM J MED SCI 258 275 969 189 WHITAKER JA AM J CLIN NUTR 20 783 967 190 WILLIAMS HTG SURG GYNECOL OBSTET 132 662 971 191 WITTING LA AM J CLIN NUTR 25 257 972 192 WITTING LA J NUTR 82 19 964 193 WITTING LA AM J CLIN NUTR 28 571 975 194 WO CKW AM J CLIN NUTR 28 808 975 195 WOODRUFF CW AM J CLIN NUTR 4 597 956 196 WRIGHT SW PEDIATRICS 7 386 951 197 ZANNOS-MARIOLEA L BR J HAEMATOL 26 193 974 CT 1 DONOVAN DH TOXICOL LETT 1 135 977 2 HOFFMANNOSTENHOF O TRENDS BIOCHEM SCI 2 284 977 3 TANGNEY CC CONTRACEPTION 17 499 978 4 CHO S J NUTR SCI VITAMINOL 24 221 978 5 FARRELL PM AM J CLIN NUTR 31 1720 978 6 EHRENKRANZ RA N ENGL J MED 299 564 978 7 LOW H BIOCHIM BIOPHYS ACTA 515 141 978 8 OGUNMEKAN AO AM J CLIN NUTR 32 2269 979 9 VATASSERY GT AM J CLIN NUTR 32 2061 979 10 ASTRUP H INT J VIT NUTR RES 49 186 979 11 PHELPS DL PEDIATRICS 63 933 979 12 TURUNEN S COMP BIOCHEM PHYSIOL (A) 63 455 979 13 KONINGS AWT RADIAT RES 80 494 979 14 EHRENKRANZ RA J PEDIATR 95 873 979 15 FARRELL PM J PEDIATR 95 869 979 16 COHEN N J AM CHEM SOCIETY 101 6710 979 17 VISSER CM BIOORGAN CHEM 9 411 980 18 PARRISH DB CRC CRIT REV FOOD SCI NUTR 13 161 980 19 KAJIWARA M HETEROCYCLES 14 1995 980 20 BAKER H NUTR REP INT 21 531 980 21 PRASAD JS AM J CLIN NUTR 33 606 980 22 BOUGLE D ANN NUTR ALIMENT 34 505 980 23 NADIGER HA BR J NUTR 44 211 980 24 OLSON GL J ORGANIC CHEM 45 803 980 25 KILIAN PL BIOCHEM BIOPHYS RES COMMUN 93 409 980 26 BURTON GW J AM CHEM SOCIETY 102 7791 980 27 LAMBERT JR ARCH PATHOL LAB MED 104 201 980 28 EHRENKRANZ RA AM J DIS CHILD 134 1157 980 29 BERTONIFREDDARI C MECH AGEING DEV 16 169 981 30 HESS HH LAB ANIM SCI 31 482 981 31 BELL EF AM J CLIN NUTR 34 414 981 32 JANSSON L AM J CLIN NUTR 34 8 981 33 JANSSON L ACTA PAEDIATR SCAND 70 297 981 34 BURTON GW J AM CHEM SOCIETY 103 6472 981 35 BIERI JG J NUTR 111 458 981 36 NADIGER HA CLIN CHIM ACTA 116 9 981 37 BATINICHABERLE I Z ACKER PFLANZEN 150 147 981 38 NODA Y NEUROBIOL AGING 3 173 982 39 SKLAN D NUTR REP INT 25 499 982 40 WELCH SC J MED CHEM 25 81 982 41 WIMALASENA J BIOCHEM PHARMACOL 31 3455 982 42 MCKENNA MC AM J CLIN NUTR 35 1010 982 43 WEERAPRADIST W ORAL SURG 54 304 982 44 SHKLAR G J NATL CANCER INST 68 791 982 45 PUKLIN JE OPHTHALMOLOGY 89 96 982 46 GUGGENHEIM MA J PEDIATR 100 51 982 47 SKLAN D J NUTR 112 1394 982 48 EHRENKRANZ RA ANN NY ACAD SCI 393 452 982 49 FARRELL PM ANN NY ACAD SCI 393 96 982 50 GUGGENHEIM MA ANN NY ACAD SCI 393 84 982 51 JANSSON L ACTA PAEDIATR SCAND SUPPL 296 1982 110 982 52 TERAO S J CHEM SOC PERKIN TRANS I 1982 2909 982 53 PANATTA GB NUTR REP INT 27 811 983 54 VATASSERY GT AM J CLIN NUTR 37 1020 983 55 KIM JE J PERIODONTOL 54 305 983 56 MATHER JP ACTA ENDOCRINOL 102 470 983 57 TAJIMA K BIOCHEM BIOPHYS RES COMMUN 115 1002 983 58 BURTON GW ARCH BIOCHEM BIOPHYS 221 281 983 59 BIERI JG N ENGL J MED 308 1063 983 60 FULLER PA NUTR RES 4 333 984 61 TAKEDA K CRIT CARE MED 12 957 984 62 VOST A LIPIDS 19 423 984 63 MARTINEZ FE J TROP PEDIATR 30 92 984 64 GUTCHER GR AM J CLIN NUTR 40 1078 984 65 BOUGLE D ARCH FR PEDIATR 41 63 984 66 BIERI JG J AM OIL CHEM SOC 61 1917 984 67 LABADARIOS D S AFR MED J 66 477 984 68 LABADARIOS D S AFR J SCI 80 427 984 69 NAPOLI JL ARCH BIOCHEM BIOPHYS 230 194 984 70 GUTCHER GR J PEDIATR GASTROENTEROL NUTR 4 604 985 71 CARPENTER D SEM NEUROL 5 271 985 72 CARPENTER D SEM NEUROL 5 283 985 73 LIBONDI T OPHTHALM RES 17 42 985 74 OBARA H CAN ANAESTH SOC J 32 358 985 75 OGUNMEKAN AO TROP GEOGR MED 37 175 985 76 MINO M AM J CLIN NUTR 41 631 985 77 MCGEE CD AM J CLIN NUTR 42 432 985 78 SYVAOJA EL INT J VIT NUTR RES 55 159 985 79 KALVARIA I INT J PANCREATOL 1 119 986 80 PIERI C ARCH GERONTOL GERIATR 5 21 986 81 GUTCHER G SEM PERINATOL 10 196 986 82 VANDEWOUDE MG J PARENT ENTERAL NUTR 10 303 986 83 HERBETH B CLIN CHEM 32 1756 986 84 ARANDA JV PEDIATR CLIN NORTH AM 33 583 986 PN 76229 RN 00582 AN 78098519 AU Euler-A-R. Ament-M-E. TI Crohn's disease - a cause of arthritis, oxalate stones and fistulae in cystic fibrosis. SO West-J-Med. 1976 Oct. 125(4). P 315-7. MJ CYSTIC-FIBROSIS: co. CROHN-DISEASE: co. MN ADOLESCENCE. ARTHRITIS-RHEUMATOID: et. CASE-REPORT. HUMAN. INTESTINAL-FISTULA: et. MALE. URETERAL-CALCULI: et. EX In most patients with cystic fibrosis growth failure occurs because of malabsorption or moderate to severe pulmonary disease, or both. Occasionally, growth lag is seen in patients that is disproportionate to the severity of the primary disease. Profound growth failure is a major characteristic of Crohn's disease in children. This report documents the first association of these two diseases in the same patient in the United States and shows how Crohn's disease alters the gastrointestinal manifestations of cystic fibrosis. An adolescent patient with cystic fibrosis, anemia, fistulae, arthritis and calcium oxalate stones was proven to have Crohn's disease by microscopic evaluation of surgically resected terminal ileum and colon. RF 001 EHRENPREIS TH ACTA PAEDIATR SCAND 60 209 971 002 LAPEY A J PEDIATR 84 328 974 003 MATTHEWS LW J PEDIATR 65 558 964 004 LINDSLEY CB ARTHRITIS RHEUM 14 398 971 005 WILLIAMS JA CLIN GASTROENTEROL 1 469 972 006 KYLE J PROC INT CONG GASTROENTER 8TH 968 007 BOWDEN DH AM J MED 38 226 965 008 SMITH A J CLIN GASTROENTEROL 1 443 972 009 MCCAFFERY TD PEDIATRICS 45 386 970 010 DYER N CLIN GASTROENTEROL 1 449 972 011 CHADWICK V N ENGL J MED 289 172 973 012 SMITH L N ENGL J MED 286 1371 972 013 EARNEST D J CLIN INVEST 51 26A 972 014 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 015 DOERSHUK CF IN: GREEN M 707 968 016 MONK M GASTROENTEROLOGY 53 198 967 CT 1 SAGRANSKY DM AM J DIS CHILD 134 319 980 2 DELUMLEY L ARCH FR PEDIATR 40 723 983 3 BERKIN KE EUR J RESPIR DIS 67 103 985 4 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 5 OJEDA VJ DIS COLON RECTUM 29 567 986 6 PHILLIPS BM J ROY SOC MED 79 44 986