PN 78001 RN 00782 AN 78231664 AU Mei-Liu-H. TI Reactive neuroaxonal dystrophy in children. Clinical pathological correlation. SO Acta-Neuropathol (Berl). 1978 Jun 30. 42(3). P 237-41. MJ BRAIN-DISEASES: pa. MN AUTOPSY. BILE-DUCTS: ab. BRAIN-DISEASES: ci, et. BRAIN-STEM: pa. CHILD. CYSTIC-FIBROSIS: co. HEART-FAILURE-CONGESTIVE: co. HUMAN. KIDNEY-FAILURE-CHRONIC: co. NUTRITION-DISORDERS: co. SPINAL-CORD: pa. TIME-FACTORS. VINCRISTINE: ae. AB The aim of the present study was to determine the frequency and etioloical factors of neuroaxonal dystrophy (N.D.) in brainstem and spinal cord of children with various non-neurological diseases. The materials used in this study consisted of 266 consecutive autopsies from 1974-1976 and an additional 13 cases from previous years. By far, the most common cause of N.D. in children was chemotherapeutic drugs, particularly vincristine, used for the treatment of malignant tumors. Approximately 90% of these children developed N.D. and the changes ranged from mild to severe. Approximately two-thirds of children with cystic fibrosis, congestive heart failure and chronic renal failure developed N.D. and the changes were mostly mild. Only one-third of patients with congenital biliary atresia and malnutrition developed N.D. and the changes were always mild. The frequency and severity of N.D. increased in patients who had prolonged clinical courses; no N.D. was seen in patients who died from acute cases such as trauma, acute infection, intoxication, acute renal failure or prematurity. This type of N.D. may be considered as reactive to a wide variety of injurious factors such as drug toxicity, malnutrition, chronic hypoxia and chronic renal failure, either alone or in combination. In contrast to previous reports of a low incidence of N.D. in children, there has been a sharp increase in recent years due to the advent of chemotherapy. RF 001 BERARD-BADIER M ACTA NEUROPATH (BERL) 28 261 974 002 BORISY GG J CELL BIOL 34 525 967 003 BRANNON W ACTA NEUROPATH (BERL) 9 1 967 004 CHOU SM ACTA NEUROPATH (BERL) 3 428 964 005 FUJISAWA K ACTA NEUROPATH (BERL) 8 255 967 006 JELLINGER K GERMAN MEDICAL MONTHLY BD 13 341 968 007$ LAMPERT P J NEUROPATH EXP NEUROL 23 60 964 008 LIU HM ACTA NEUROPATH (BERL) 37 207 977 009 LIU HM NEUROLOGY (MINN) 24 547 974 010 LUI HM ACTA NEUROPATH (BERL) 27 201 974 011 MARTIN JJ EUR NEUROL 8 239 972 012 PENTSCHEW A ACTA NEUROPATH (BERL) 1 313 962 013 SEITELBERGER F ACTA NEUROPATH (BERL) SUPPL 5 17 971 014 SEITELBERGER F PROC CONG NEUROPATH 1ST 3 323 952 015 SUNG JH J NEUROPATH EXP NEUROL 25 341 966 016 SUNG JH J NEUROPATH EXP NEUROL 25 341 966 017 WISNIEWSKI H J CELL BIOL 38 224 968 CT 1 AICARDI J BRAIN 102 727 979 2 GRAFE MR J NEUROPATHOL EXP NEUROL 39 555 980 3 SUNG JH J NEUROPATHOL EXP NEUROL 39 584 980 4 SUNG JH J NEUROPATHOL EXP NEUROL 40 37 981 5 TOMIWA K J NEUROL 229 267 983 6 ROBAIN O J NEUROL NEUROSURG PSYCHIAT 47 65 984 7 WISNIEWSKI KE ACTA NEUROPATH (BERL) 66 68 985 PN 78002 RN 00783 AN 78120444 AU Kraemer-R. Rudeberg-A. Hadorn-B. Rossi-E. TI Relative underweight in cystic fibrosis and its prognostic value. SO Acta-Paediatr-Scand. 1978 Jan. 67(1). P 33-7. MJ BODY-WEIGHT. CYSTIC-FIBROSIS: mo. MN CHILD. HUMAN. PROGNOSIS. AB On the basis of observations in 117 children with cystic fibrosis, seen from January 1956 to June 1976, it is demonstrated that the relative underweight (weight loss corrected for height) is most pronounced in children with predominantly pulmonary sypmtoms. The degree of underweight closely correlates inversely with survival. Because of its prognostic value, it is recommended that this clinical parameter be included in the checkups which are periodically carried out on children suffering from cystic fibrosis. RF 001 COOPERMAN EM CAN MED ASSOC J 105 580 971 002 CUTLER SJ J CHRON DIS 8 699 958 003 DOERSHUK CF J PEDIATR 65 677 964 004 FRIOLET B THER UMSCH 31 578 974 005 GEORGE L ARCH DIS CHILD 46 139 971 006 HADORN B SCHWEIZ RUNDSCHAU MED 14 454 973 007 HOGGER GP HELV PAEDIATR ACTA 30 151 975 008 HUANG NN AM J DIS CHILD 120 289 970 009 KERREBIJN KF PROC INT CF CONG 7TH 976 010 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 271 977 011 KRAEMER R HELV PAEDIATR ACTA 32 107 977 012 LAPEY A J PEDIATR 84 328 974 013 ROBINSON MJ ARCH DIS CHILD 50 962 975 014 SHWACHMAN H AM J DIS CHILD 96 6 958 015 SHWACHMAN H HOSP PRACT 9 143 974 016 SHWACHMAN H PEDIATRICS 46 335 970 017 SPROUL A J PEDIATR 65 664 964 018 TAUSSIG LM J PEDIATR 82 380 973 019 TANNER JM ARCH DIS CHILD 41 454 966 020 WARWICK WJ J CHRON DIS 28 609 975 CT 1 SANTAGNESE PAD AM J MED 66 121 979 2 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 3 KARP RJ CLIN CHEST MED 1 375 980 4 COATES AL ACTA PAEDIATR SCAND 69 353 980 5 SHEPHERD R J PEDIATR 97 351 980 6 GOW R LANCET 2 1071 981 7 BELL L J CAN DIET ASSOC 42 62 981 8 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 9 MILLER M AM J CLIN NUTR 36 492 982 10 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 11 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 12 GIUNTA A RIV ITAL PEDIATR 9 171 983 13 POSSELT HG ATEMWEGS LUNGENKRANKH 10 345 984 14 HOLT TL IRCS MED SCI BIOCHEM 12 370 984 15 WELLS AL J FOOD NUTR 41 65 984 16 BERTRAND JM J PEDIATR 104 41 984 17 MANSELL AL J PEDIATR 104 700 984 18 CANCIANI M J PEDIATR GASTROENTEROL NUTR 4 735 985 19 DAVIS PB SEM RESPIR MED 6 261 985 20 MISCHLER EH SEM RESPIR MED 6 271 985 21 GRAHAM N CLIN RADIOL 36 199 985 22 HOLT TL AM J CLIN NUTR 41 1061 985 23 SCHRODER R SCHWEIZ MED WOCHENSCHR 115 828 985 24 WILSON DO AM REV RESPIR DIS 132 1347 985 25 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 26 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 27 NEIJENS HJ ACTA PAEDIATR SCAND SUPPL 317 1985 38 985 28 ANON LANCET 1 249 986 29 LEVY L J PEDIATR GASTROENTEROL NUTR 5 97 986 30 SOUTTER VL CLIN GASTROENTEROL 15 137 986 31 DUHAMEL JF ARCH FR PEDIATR 43 229 986 32 OLOUGHLIN E AM J CLIN NUTR 43 732 986 33 MOORE MC AM J CLIN NUTR 44 33 986 34 MAHANEY MC HUM BIOL 58 445 986 35 SCHAAD UB ACTA PAEDIATR SCAND 75 128 986 36 DODGE JA J ROY SOC MED 79 27 986 37 MARCOTTE JE CHEST 90 375 986 38 SHEPHERD RW J PEDIATR 109 788 986 39 STEAD RJ CLIN ENDOCRINOL 26 187 987 40 GUELPA G SCHWEIZ MED WOCHENSCHR 117 168 987 PN 78003 RN 00784 AN 78231746 AU Nielsen-H-E. Lundh-S. Jacobsen-S-V. Hoiby-N. TI Hypergammaglobulinemic purpura in cystic fibrosis. SO Acta-Paediatr-Scand. 1978 Jul. 67(4). P 443-7. MJ CYSTIC-FIBROSIS: co. PURPURA-HYPERGLOBULINEMIC: co. MN ADOLESCENCE. ADULT. CHRONIC-DISEASE. CYSTIC-FIBROSIS: bl, im. FEMALE. HAEMOPHILUS-INFECTIONS: co. HAEMOPHILUS-INFLUENZAE. HUMAN. LEG. MALE. PNEUMONIA: co. PURPURA-HYPERGLOBULINEMIC: bl, im. SPUTUM: mi. AB Four patients are presented aged 14 to 20 years with cystic fibrosis and recurrent purpura of the legs. They have polyclonal increase of Ig but no intermediate complexes demonstrable by ultracentrifugation. The 4 patients differ from other patients with cystic fibrosis by a rapid deterioration of the clinical condition after the establishment of permanent pulmonary infection, and also by their proneness to Haemophilus influenzae infections. The clinical and laboratory findings are compatible with the diagnosis of Waldenstrom's hypergammaglobulinemic purpura. The heterogeneity of this syndrome is discussed. RF 001 ANON BR MED J 1 7 973 002 CAPRA JD MEDICINE (BALTIMORE) 50 125 971 003 CARR RD ARCH DERMATOL 94 536 966 004 DANES BS CLIN GENET 8 85 975 005 FARRELL C SCAND J IMMUNOL 4 673 975 006 HOIGNE R IN: DUKES NG 551 975 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 008 HOIBY N SCAND J RESPIR DIS 56 38 975 009 HOIBY N SCAND J RESPIR DIS 58 65 977 010 JACOBS JC J PEDIATR 87 91 975 011 JOHNSON KJ J CLIN INVEST 54 349 974 012 KATTAMIS C HELV PAEDIATR ACTA 25 421 970 013 KYLE RA MEDICINE (BALTIMORE) 50 113 971 014 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 015 SCHWARTZ HJ JAMA 187 230 964 016 STRAUSS WG N ENGL J MED 260 857 959 017$ WALDENSTROM JG NORD MED 20 2288 943 018 WALDENSTROM JG ACTA MED SCAND SUPPL 142 931 952 019 WALDENSTROM JG MONOCLONAL AND POLYCLONAL HYP 968 020 WIDMER U MINERVA PEDIATR 27 104 975 021 ZAHARIA L ACTA MED SCAND 199 429 976 022 ZAVALA DC J ALLERGY CLIN IMMUNOL 56 450 975 CT 1 SOTER NA J PEDIATR 95 197 979 2 CELADA A HELV PAEDIATR ACTA 35 569 980 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 5 SCHIDLOW DV ARCH DIS CHILD 59 377 984 6 CIRIELLO MM HAEMATOLOGICA 69 435 984 7 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 8 SUMMERS GD BR J RHEUMATOL 25 393 986 9 HOIBY N ANNU REV MICROBIOL 40 29 986 10 PHILLIPS BM J ROY SOC MED 79 44 986 PN 78004 RN 00785 AN 78120454 AU Roomans-G-M. Afzelius-B-A. Kollberg-H. Forslind-B. TI Electrolytes in nails analysed by X-ray microanalysis in electron microscopy. Considerations on a new method for the diagnosis of cystic fibrosis. SO Acta-Paediatr-Scand. 1978 Jan. 67(1). P 89-94. MJ CYSTIC-FIBROSIS: di. ELECTROLYTES: an. NAILS: an. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. EVALUATION-STUDIES. HUMAN. METHODS. MICROSCOPY-ELECTRON. SODIUM: an. ELECTRON-PROBE-MICROANALYSIS. AB Patients with cystic fibrosis (CF) have an increased concentration of sodium in their nails. Hitherto, only neutron activation analysis has been considered for the diagnosis of cystic fibrosis by analysis of electrolytes in nails. It has been thoroughly tested methodologically and clinically. However, the intrinsic advantages of X-ray microanalysis and the results obtained in this study suggest that this method, after further testing, may be a useful diagnostic acid for cystic fibrosis. In comparison with neutron activation analysis, X-ray microanalysis has the advantage of simultaneously giving the concentrations of several elements and may be accessible at any hospital with an electron microscope fitted with the necessary equipment. Nails of CF-patients are here shown to have increased concentrations of Na, K and Cl, which will make the diagnosis of cystic fibrosis more reliable. The possibility of using sulphur as a reference element may eliminate the weighing procedure necessary in neutron activation analysis. RF 001 BERG T NORTHERN PEDIATR CONG 15 967 002 CHANDLER JA J MICROSC 106 291 976 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 FITE LE PROC INT CONF MOD TRENDS 147 968 005 FORSLIND B ACTA DERM VENEREOL (STOCKH) 50 161 970 006 FORSLIND B J INVEST DERMATOL 67 273 976 007 GEORGE L ARCH DIS CHILD 46 139 971 008 GIBSON LE PEDIATRICS 23 545 959 009 HALL TA MICROSCOPIC BIOL CELL 22 271 975 010 HARRISON WW CLIN CHIM ACTA 36 483 972 011 HEIN W MIKROCHIM ACTA WIEN SUPPL 5 1 974 012 HEINTZ PH THESIS 967 013 JOHNSON GF PEDIATRICS 47 88 971 014 KANABROCKI E J NUCL MED 9 478 968 015 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 016 KOLLBERG H ACTA PAEDIATR SCAND 63 405 974 017 KOPITO L N ENGL J MED 272 504 965 018 MORGAN AJ J MICROSC 104 271 975 019 SHWACHMAN H PEDIATRICS 46 335 970 CT 1 STEPHAN U MONOGR PAEDIATR 10 41 979 2 GHADIALLY FN PATHOLOGY 11 95 979 3 KUYPERS GAJ HISTOCHEMISTRY 69 145 980 4 DAVID H BIOL ZENTRALBL 101 715 982 5 FORSLIND B SCANN ELECTRON MICROSC 1982 1715 982 6 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 7 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 8 ROOMANS GM ANN NY ACAD SCI 428 121 984 9 FORSLIND B SCANN ELECTRON MICROSC 1984 183 984 10 CHAPMAN AL EUR J RESPIR DIS 66 218 985 11 BAKER D SCANN ELECTRON MICROSC 1985 659 985 12 DJALDETTI M CLIN SCI 72 669 987 13 DJALDETTI M DERMATOLOGICA 174 114 987 PN 78005 RN 00786 AN 79018054 AU Hosli-P. Kollberg-H. Vogt-E. TI Reliable diagnosis of the major type of cystic fibrosis with fibroblast cultures. A double blind study. SO Acta-Paediatr-Scand. 1978 Sep. 67(5). P 617-20. MJ CYSTIC-FIBROSIS: di. FIBROBLASTS: en. GLYCOPROTEINS: du. MN ADOLESCENCE. ADULT. ALKALINE-PHOSPHATASE: me. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. CYSTIC-FIBROSIS: en. DOUBLE-BLIND-METHOD. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. ISOPROTERENOL: du. MALE. MIDDLE-AGE. SKIN: en. THEOPHYLLINE: du. AB A double blind study has been carried out to demonstrate that the most common type of cystic fibrosis (CF) can be reliably diagnosed with skin-derived fibroblast cultures. Alkaline phosphatase (AIP) activity has been measured in 6 normal controls, 12 CF-heterozygotes and 6 CF-homozygotes before and after stimulation with Tamm-Horsfall- glycoprotein (THP), isoproterenol and theophyline ("THP-induction test"). The mean AIP-activities after THP-induction were 8.8, 12.7 and 34.6 for the three different genotypes respectively. There was no overlap between the values of CF-homozygotes on the one hand, and the values of CF-heterozygotes and normal controls on the other hand. All 24 specimens were correctly diagnosed in the present double blind study, indicating the very high degree of reliability of the THP- induction test in the detection of the predominant type of cystic fibrosis with fibroblast cultures. Normal controls and CF- heterozygotes could not be discriminated on an individual basis, but as a group the CF-heterozygotes displayed higher AIP-values. RF 001 BARTMAN J J PEDIATR 76 430 970 002 DANES BS J EXP MED 129 775 969 003 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 004 GIBSON LE PEDIATRICS 23 545 959 005 HICKMAN S BIOCHEM BIOPHYS RES COMMUN 49 992 972 006 HOSLI P TISSUE CULTIVATION ON PLASTIC 972 007 HOSLI P IN: MOTULSKY AG 226 974 008 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 009 HOSLI P ADV EXP MED BIOL 68 1 976 010 HOSLI P CLIN CHEM 23 1476 977 011 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 013 HOSLI P HUM GENET 41 169 978 015 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 016 LIGHTBODY J LANCET 1 451 971 017 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 018 PALLAVICINI JC J PEDIATR 77 280 970 019 WARWICK WJ PEDIATRICS 34 621 964 CT 1 MAHONEY MJ CLIN PERINATOL 6 255 979 2 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 3 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 4 DAVIS PB PEDIATR RES 14 83 980 5 CHASE HP METABOLISM 29 365 980 6 FASTH A ACTA PAEDIATR SCAND 69 189 980 7 CEDER O SCANN ELECTRON MICROSC 1982 723 982 8 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 9 KHALID BAK CLIN ENDOCRINOL 18 407 983 10 CEDER O CLIN GENET 23 298 983 11 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 12 BUYS CHCM CLIN CHIM ACTA 136 229 984 PN 78006 RN 00787 AN 79018052 AU Katznelson-D. TI Increased intracranial pressure in cystic fibrosis. SO Acta-Paediatr-Scand. 1978 Sep. 67(5). P 607-9. MJ CYSTIC-FIBROSIS: co. PSEUDOTUMOR-CEREBRI: et. MN CASE-REPORT. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. RESPIRATORY-INSUFFICIENCY: co, et. VENOUS-PRESSURE. AB Three cystic fibrosis infants with severe respiratory distress had increased intracranial pressure (with bulging fontanels) which cleared parri passu with improvement in the chest condition. It is proposed that the intracranial hypertension is a result of raised venous pressure, itself secondary to the intrathoracic obstruction to venous return, consequent on the bronchial obstructive disease. RF 001 BRAY PF AM J DIS CHILD 126 78 973 002 DEES SC PEDIATRICS 23 1143 959 003 FIELDS JP J PEDIATR 58 74 961 004 JOHNSTON I BRAIN 97 289 974 005 JOHNSTON I BRAIN 97 301 974 006 LOBECK CC IN: STANBURY JB 1605 972 007 MARIE J AM J DIS CHILD 87 731 954 008 SHWACHMAN H IN: KENDIG EL JR 1 524 972 009 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 TAUSSIG LM ACTA PAEDIATR SCAND 68 615 979 2 MAGAZZU G RIV ITAL PEDIATR 7 73 981 3 ROACH ES PEDIATRICS 68 300 981 4 PALMER EA J TOXICOL CUTAN OCUL TOXICOL 1 181 982 PN 78007 RN 00788 AN 78141570 AU Matsuo-T. Ikeda-T. Mori-Y. Nonaka-M. Fujiwara-H. Yun-K. TI Cystic fibrosis of the pancreas in a human fetus. SO Acta-Pathol-Jpn. 1978 Jan. 28(1). P 77-82. MJ CYSTIC-FIBROSIS: em. MN CYSTIC-FIBROSIS: pa. FEMALE. GESTATIONAL-AGE. HUMAN. PANCREAS: pa. PREGNANCY. AB Postmortem examination of a 26 week old (postmenstrual) human fetus delivered by Cesarean section revealed meconium ileus and many swollen mucus-secreting cells in the gastro-intestinal mucosa. The pancreas showed extensive fibrosis, acinar destruction and dilatation of ducts containing eosinophilic casts. Mucous glands of the lungs also revealed mucous cells swollen and distended with their secretory products. The patient is believed to have cystic fibrosis of the pancreas, suggesting that the pathologic manifestations of the disease may begin early in fetal life. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 003 BOYER PH PEDIATRICS 16 778 955 004 JOHANSEN PG LANCET 1 455 968 005 KEENE MFL LANCET 1 767 929 006 LOBECK CC IN: STANBURY JB 1605 972 007 MARKS BL LANCET 1 365 960 008 WARKANY J CONGENITAL MALFORMATIONS NOTE 732 971 PN 78008 RN 00789 AN 78141584 AU Rolla-G. Melsen-B. Sonju-T. TI Is sulphated blood-group substance in pancreas a factor in cystic fibrosis?. SO Acta-Pathol-Microbiol-Scand [A]. 1978 Jan. 86(1). P 83-6. MJ BLOOD-GROUPS. CYSTIC-FIBROSIS: bl. GLYCOPROTEINS: bl. MN ANIMAL. AUTORADIOGRAPHY. CALCIUM: bl. CERCOPITHECUS. MACACA-FASCICULARIS. ANTHROPOIDEA. PANCREAS: me. SULFATES: me. AB The present study showed that the pancreas of two monkey species contained sulphated blood-group substance. Such strongly anionic molecules are known to bind calcium, and their solutions form gels in the presence of cations. It is suggested that such a mechanism may be involved in cystic fibrosis. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 HAUG A ACTA CHEM SCAND 24 843 970 003 KOPITO LE PEDIATR RES 10 742 976 004 LAMB D BR J DIS CHEST 66 239 972 005 LUDWIG H SCAND J IMMUNOL 6 724 977 006 ROLLA G ARCH ORAL BIOL 20 341 975 007 SMIDSROD O CHEM SOC FARADAY DISC 57 263 974 008 SZULMAN AE J EXP MED 111 785 960 009 SONJU T ACTA PATH MICROBIOL SCAND 79 95 971 010 SONJU T ARCH ORAL BIOL 19 897 974 011 SONJU T CARIES RES 8 113 974 012 SONJU T ACTA PATH MICROBIOL SCAND 83 215 975 013 WATKINS WM IN: GOTTSCHALK A 830 972 014 WEISS A KOLLOID ZEITSCHRIFT 158 22 958 015 WOOD RE AM REV RESPIR DIS 113 833 976 016 YOSIZAWA Z IN: GOTTSCHALK A 5 1000 972 CT 1 MICHALSEN H MONOGR PAEDIATR 10 148 979 PN 78009 RN 00790 AN 79038683 AU Schiotz-P-O. Nielsen-H. Hoiby-N. Glikmann-G. Svehag-S-E. TI Immune complexes in the sputum of patients with cystic fibrosis suffering from chronic Pseudomonas aeruginosa lung infection. SO Acta-Pathol-Microbiol-Scand [C]. 1978 Feb. 86(1). P 37-40. MJ ANTIGEN-ANTIBODY-COMPLEX. CYSTIC-FIBROSIS: co. PNEUMONIA: co. PSEUDOMONAS-INFECTIONS: co. SPUTUM: im. MN ADOLESCENCE. ADULT. CHILD. CHRONIC-DISEASE. COMPLEMENT-FIXATION-TESTS. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. IGG. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. PNEUMONIA: im. PSEUDOMONAS-INFECTIONS: im. RHEUMATOID-FACTOR. AB 12 cystic fibrosis (CF) patients chronically infected with mucoid P. aeruginosa and presenting multiple precipitins in serum against this bacterium and 12 patients without P. aeruginosa infection were examined for occurrence of soluble immune complexes in their sputum sol phase by a complement consumption assay and a solid phase rheumatoid factor binding assay. The correlation between the results obtained in the two assays was significant (r = 0.625, p less than 0.01). The patients chronically infected with P. aeruginosa showed a significantly (p less than 0.01) higher frequency of immune complex activity in their sputum sol phase, as compared to the patients without P. aeruginosa lung infection. These findings point to the possibility that chronic lung infection with mucoid P. aeruginosa in CF may be an immune complex disease. RF 001 BROGAN TD THORAX 30 72 975 002 COCHRANE CG IN: ZWEIFACH BW 3 85 974 003 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 004 GALLIN JI IN: KIRKPATRICK CH 161 976 005 GIBSON LE PEDIATRICS 23 545 959 007 HIRSCHHORN R IN: ZWEIFACH BW 1 259 974 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 010 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 011 HOIBY N ACTA PAEDIATR SCAND 63 843 974 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 013 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 014 HOIBY N SCAND J RESPIR DIS 56 38 975 015 HOIBY N SCAND J RESPIR DIS 58 65 977 016 LEBER PD IN: ZWEIFACH BW 3 401 974 017 NIELSEN H ACTA PATH MICROBIOL SCAND (C) 84 261 976 018 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 019 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 020 STALENHEIM G IMMUNOCHEMISTRY 10 501 973 021 THORELL JI BIOCHIM BIOPHYS ACTA 251 363 971 022 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 023 TURK JL BOLL IST SIEROTER MILAN SUPPL 53 208 974 CT 1 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 2 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 3 HOIBY N ACTA PAEDIATR SCAND 68 495 979 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 5 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 345 979 7 BERDISCHEWSKY M PEDIATR RES 14 830 980 8 HODSON ME THORAX 35 801 980 9 SKOV PS ALLERGY 35 23 980 10 MATTHEWS WJ N ENGL J MED 302 245 980 11 SORENSEN RU PEDIATR RES 15 14 981 12 CHURCH JA CHEST 80 405 981 13 MOSS RB J PEDIATR 99 215 981 14 DANIELE RP AM REV RESPIR DIS 124 738 981 15 PERMIN H ALLERGY 37 93 982 16 MOSS RB CLIN EXP IMMUNOL 47 301 982 17 PITCHERWILMOTT RW ARCH DIS CHILD 57 577 982 18 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 19 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 20 ANON LANCET 2 257 983 21 SCHILLER NL PEDIATR RES 17 747 983 22 DORING G INFECT IMMUN 42 197 983 23 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 24 SPEERT DP PEDIATR RES 18 431 984 25 DORING G ACTA PATH MICROB IMMU SCA (C) 92 307 984 26 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 27 MISCHLER EH SEM RESPIR MED 6 271 985 28 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 29 WOODS DE J INFECT DIS 151 581 985 30 KHARAZMI A EUR J CLIN INVEST 16 143 986 31 HOIBY N ANNU REV MICROBIOL 40 29 986 32 PIEDRA P J PEDIATR 108 817 986 33 MOSS RB AM REV RESPIR DIS 133 648 986 34 DASGUPTA MK J CLIN IMMUNOL 7 51 987 PN 78010 RN 00791 AN 79121553 AU Lebenthal-E. TI Pancreatic function and disease in infancy and childhood. SO Adv-Pediatr. 1978. 25. P 223-61. (REVIEW). MJ CYSTIC-FIBROSIS: pp. PANCREAS: ph. PANCREATIC-DISEASES: pp. PANCREATITIS: et. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. DIGESTION. HUMAN. INFANT. INFANT-NEWBORN. PANCREAS: ab, en. PANCREATIC-DISEASES: dt, en. PANCREATIC-NEOPLASMS: pp. PANCREATITIS: th. REVIEW. STARCH. SYNDROME. EX Normal development of the pancreas is of great importance in the neonate and infant. This review evaluates the development of exocrine pancreatic function, the hormonal control of this function, the interrelationship of pancreatic enzymes and pancreatic adaptation to food. The functional changes that occur in different disease processes of the pancreas affecting the infant will also be discussed. Since the treatment of pancreatic exocrine insufficiency with orally administered pancreatic enzymes in children is often more difficult than expected, the practical approach to enzyme treatment will be discussed. The morphological development and congenital malformations of the pancreas, tumors of the pancreas, the normal function of the exocrine pancreas, the development of the exocrine pancreatic function, developmental aspects of amylase activity and starch digestion, dietary adaptation of the exocrine pancreas, pancreatic function tests, pancreatic insufficiency, cystic fibrosis, Shwachman syndrome, isolated pancreatic enzyme deficiencies, the treatment of exocrine pancreatic insufficiency, pancreatitis in childhood, and the treatment of acute pancreatitis are discussed. RF 001 ABRUZZO JL ANN SURG 147 921 958 002 ADAMS JT SURGERY 63 877 968 003 AKERS DR SURGERY 71 817 972 004 ALLAN BJ ARCH BIOCHEM BIOPHYS 136 529 970 005 ANTONOWICZ I GASTROENTEROLOGY 72 1299 977 006 ANDERSON TA J LAB CLIN MED 79 31 972 007 APPEL MF ARCH SURG 108 63 974 008 AREY LB DEVELOPMENTAL ANATOMY 7TH ED 965 009 AURICCHIO S PEDIATRICS 39 853 967 010 AURICCHIO S PEDIATRICS 35 944 965 011 BABKIN BP SECRETORY MECHANISMS OF THE D 54 950 012 BACHRACH WH GASTROENTEROLOGY 63 890 972 013 BALZER E Z GASTROENTEROL 5 239 967 014 BANKS PA GASTROENTEROLOGY 61 382 971 015 BARBERO GJ AM J DIS CHILD 112 536 966 016 BARBEZAT GO PEDIATRICS 42 77 968 017 BARTHOLOMEW C BR MED J 1 666 970 018 BECKER WF ANN SURG 163 892 966 019 BEN ABDELJLIL A BIOCHEM BIOPHYS RES COMMUN 10 112 963 020 BENZONANA G BIOCHIM BIOPHYS ACTA 105 121 965 021 BERK JE JAMA 199 98 967 022 BISHOP RP AM J GASTROENTEROL 49 112 968 023 BLAINEY JD CLIN SCI 32 377 967 024 BLOCK MB N ENGL J MED 282 380 970 025 BLUMENTHAL HT ANN SURG 27 533 961 026 BODIAN M ACTA PAEDIATR SCAND 53 282 964 027 BONIN A J PEDIATR 83 594 973 028 BORGSTROM B BIOCHIM BIOPHYS ACTA 242 509 971 029 BROOKS FP N ENGL J MED 286 300 972 030 BUNNELL CE J PEDIATR 80 465 972 031 CAREY MC GUT 9 700 968 032 CASTLEMAN B N ENGL J MED 286 1353 972 033 CHRISTOPHE J GUT 7 298 966 034 CHRISTOPHE J HORM METAB RES 3 393 971 035 COMFORT MW GASTROENTEROLOGY 21 54 952 036 CORNET E J CHIR (PARIS) 84 527 962 037 COTTON PB LANCET 1 53 972 038 DAGORN JC GASTROENTEROLOGY 73 42 977 039 DELACHAUME-SALEM E BIOL GASTROENTEROL 2 135 970 040 DESNUELLE P CIBA FOUND SYMP 90 962 041 DEVIZIA B J PEDIATR 86 50 975 042 DIMAGNO EP N ENGL J MED 296 1318 977 043 DI SANTAGNESE PA PEDIATRICS 15 683 955 044 DI SANTAGNESE PA ACTA PAEDIATR SCAND 63 163 974 045 DIVINCENTI FC J TRAUMA 8 1004 968 046 DREILING DA GASTROENTEROLOGY 30 382 956 047 DOLAN RV ARCH SURG 109 762 974 048 DONOWITZ M J PEDIATR 87 241 975 049 DRUMMEY GD N ENGL J MED 264 85 961 050 ANON LANCET 1 205 975 051 ELLIOTT GB CAN J SURG 11 357 968 052 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 053 FARMER RG AM J MED 54 161 973 054 FELDMAN M JAMA 148 893 952 055 FOMON SJ AM J CLIN NUTR 28 1299 970 056 FRABLE WJ CANCER 27 667 971 057 FREDRICKSON DS IN: STANBURY JB 545 972 058 FREDRICKSON DS N ENGL J MED 276 34 967 059 FREY C PEDIATRICS 32 93 963 060 FRINGLE EM PROC R SOC MED 61 776 968 061 FRUCHT DA AM J DIS CHILD 92 182 956 062 GEOKAS MC CALIF MED 117 1 972 063 GIBBS GE PEDIATRICS 5 941 950 064 GIEDION A FORTSCHR GEB ROENTG NUKL 108 51 968 065 YOUNGS G GUT 13 154 972 066 GERATZ JD AM J PHYSIOL 219 705 970 067 GO VLW J CLIN INVEST 49 1558 970 068 GOULD BS AM J DIS CHILD 91 584 956 069 GRACES L PEDIATRICS 41 789 968 070 GRAHAM DY N ENGL J MED 296 1314 977 071 GREENBERGER NJ MEDICINE (BALTIMORE) 45 161 966 072 GROSFELD J ARCH SURG 101 370 970 073 GROSS JB AM J MED 33 358 962 074 GROSS RE SURGERY OF INFANCY AND CHILDH 953 075 GRYBOSKI J MAJ PROB CLIN PEDIATR 13 450 975 076 GUNDERSEN AE J PEDIATR SURG 4 478 969 077 HADORN B IN: ANDERSON CM 289 975 078 HADORN B CAN MED ASSOC J 98 377 968 079 HADORN B LANCET 1 812 969 080 HADORN B J PEDIATR 73 39 968 081 HAYS D ANN SURG 153 103 961 082 HENDREN WH ARCH DIS CHILD 40 132 965 083 HUDSON E ARCH INTERN MED 125 314 970 084 HONG SS AM J PHYSIOL 191 71 957 085 HOWARD JM ANN SURG 165 293 967 086 HOWARD F BR J NUTR 17 281 963 087 JACKSON CE AM J MED 43 727 967 088 JAMIESON JD J CELL BIOL 34 597 967 089 JANOWITZ HD ANN INTERN MED 74 444 971 090 JODL J ACTA PAEDIATR SCAND 64 619 975 091$ JOHNSON WC ANN SURG 9 562 970 092 JORDAN SC J PEDIATR 91 211 977 093 KARJOO M ARCH DIS CHILD 48 143 973 094 KATTWINKEL J PEDIATRICS 51 55 973 095 KAWANISHI H N ENGL J MED 289 357 973 096 KEENE MFL LANCET 1 767 929 097 KELLER PJ J BIOL CHEM 242 281 967 098 KIESSWETTER WB SURGERY 36 146 954 099 KILMAN JW SURGERY 55 455 964 100 KLEIN B CLIN CHEM 16 32 970 101 KLEITSCH WP ARCH SURG 71 795 955 102 KONIJN AM AM J PHYSIOL 218 1113 970 103 KLUMPP TG AM J DIS CHILD 40 1215 930 104 KREJS GJ AM J DIG DIS 22 280 977 105 KUNITZ M J GEN PHYSIOL 22 429 939 106 LAGERLOF HO ACTA MED SCAND SUPPL 128 1 942 107 LEBENTHAL E PEDIATRICS 56 585 975 108 LEBENTHAL E GASTROENTEROLOGY 70 508 976 109 LEBENTHAL E BIOCHIM BIOPHYS ACTA 497 558 977 110 LIEBERMAN J GASTROENTEROLOGY 50 183 966 111 LIECHTY RD JAMA 230 1538 974 112 LILLIBRIDGE CB J PEDIATR 82 279 973 113 LIESTYNA JA AM J DIS CHILD 107 644 964 114 LIEBOW C SCIENCE 189 472 975 115 LIFTON LJ JAMA 229 47 974 116 LINDBERG T ACTA PAEDIATR SCAND 63 805 974 117 LONG WB GASTROENTEROLOGY 71 589 976 118 LOUVARD D BIOCHIM BIOPHYS ACTA 309 127 973 119 LOWE CU AM J DIS CHILD 82 459 951 120 LUNDH G GASTROENTEROLOGY 42 275 962 121 LYMAN RL J NUTR 79 28 963 122 MACGREGOR IL GASTROENTEROLOGY 72 195 977 123 MALAISSE-LAGAE F SCIENCE 190 795 975 124 MALLARD RE J PEDIATR 91 445 977 125 MALONE JI J PEDIATR 85 825 974 126 MARCHIS-MOUREN G ARCH BIOCHEM BIOPHYS 83 309 959 127 MARDH PA BR MED J 2 240 973 128 MARTIN DU PAN R INT Z VITAMINFORSCH 1 67 971 129 MARTINEZ W ANN SURG 147 1 958 130 MATSUMOTO Y SURGERY 76 827 974 131 MCELROY R AM J MED 52 228 972 132 MOORE JG AM J DIG DIS 16 97 971 133 MORENS DM AM J DIS CHILD 128 401 974 134 MOYNAN RW J PEDIATR 65 711 964 135 NAGEL W PHYSIOL CHEM 340 1 965 136 NEELEY WA ANN SURG 173 657 971 137 NORDSTROM C BIOCHIM BIOPHYS ACTA 242 209 971 138 NORMAN A ACTA PAEDIATR SCAND 61 571 972 139 NORTHRUP WF SURGERY 71 27 972 140 OTHERSON HB J TRAUMA 8 535 968 141 PALADE GE IN: DE REUCK AVS 962 142 PALADE GE SCIENCE 189 347 975 143 PATTEN BM HUMAN EMBRYOLOGY 968 144 PAVLOV JP ARBEIT DER VERDEUUNGSDRUS 48 898 145 PERRIER CV GASTROENTEROLOGY 44 493 963 146 PETERSEN H SCAND J GASTROENTEROL 5 555 970 147 PRATT CB J PEDIATR 77 474 970 148 REETSMA K SURGERY 42 22 957 149 REGAN PT GASTROENTEROLOGY 72 A95 977 150 RICKHAM PP ARCH DIS CHILD 29 80 954 151 RICOUR C ACTA PAEDIATR SCAND 63 168 974 152 RIEMENSCHNEIDER TA PEDIATRICS 41 428 968 153 ROBINSON MJ PEDIATRICS 48 232 971 154 ROSSI E HELV PAEDIATR ACTA 8 530 953 155 ROTHMAN SS NATURE 213 460 967 156 ROTHMAN SS AM J PHYSIOL 217 504 969 157 ROTHMAN SS SCIENCE 190 747 975 158 SAID SI N ENGL J MED 293 155 975 159 SALMAN AJ PROC SOC EXP BIOL MED 126 694 967 160 SAUNDERS JHB BR MED J 1 418 977 161 SCHMIDT H CLIN ORTHOP 69 135 970 162 SHANKLIN DR N ENGL J MED 266 1097 962 163 SHELDON W ARCH DIS CHILD 39 268 964 164 SHMERLING DH HELV PAEDIATR ACTA 24 547 969 165 SHWACHMAN H J PEDIATR 63 835 963 166 SHWACHMAN H J PEDIATR 65 645 964 167 SHWACHMAN H AM J DIS CHILD 92 347 956 168 SHWACHMAN H IN: SLEISENGER MH 1206 973 169 SHWACHMAN H BIRTH DEF ORIG ART SER 8 46 972 170 SHWACHMAN H PEDIATRICS 55 86 975 171 SHWACHMAN H ADV PEDIATR 7 249 955 172 SIBERT JR GUT 16 81 975 173 SIBERT JR GUT 16 81 975 174 SIBERT JR ARCH DIS CHILD 50 443 975 175 SKUDE G ACTA PAEDIATR SCAND 65 145 976 176 SNOOK JT J NUTR 87 297 965 177 SNOOK JT AM J PHYSIOL 221 1383 971 178 SOLOMON AK FED PROC 11 722 952 179 STOVER SL J PEDIATR 73 235 968 180 SWAN DC LANCET 1 60 966 181 TACHIBANA T JAPAN J OBSTET GYNECOL 10 27 927 182 TACHIBANA T JAPAN J OBSTET GYNECOL 10 40 927 183 TACHIBANA T JAPAN J OBSTET GYNECOL 11 92 928 184 TAUSSIG LM PEDIATRICS 54 229 974 185 THEODORIDES T J CHIR (PARIS) 87 445 964 186 UDALL JN J PEDIATR 88 819 976 187 URSING B BR MED J 3 524 973 188$ WALTERS RL AM J SURG 3 364 966 189 WARSHAW AL N ENGL J MED 292 325 975 190 WEIJERS HA ACTA PAEDIATR SCAND 42 24 953 191 WELCH KJ TRAUMATIC LESIONS OF ABDOMEN 6 962 192 WEITZMAN JJ SURGERY 57 309 965 193 WERNER B ACTA PAEDIATR SCAND SUPPL 6 35 1 948 194 WHALEN J AM J SURG 121 16 971 195 WHITE TT AM J SURG 129 132 970 196 WITTE CL JAMA 203 1068 968 197 WOLF RO OBSTET GYNECOL 41 337 973 198 WOODLEY JF GUT 13 900 972 199$ WORMSLEY KG GASTROENTEROLOGY 1 27 972 200 WORMSLEY KG GUT 13 398 972 201 WORNING H SCAND J GASTROENTEROL 1 268 966 202 WRIGHT E SURGERY 69 389 971 203 ZOPPI G ACTA PAEDIATR SCAND 59 692 970 204 ZOPPI G PEDIATR RES 6 880 972 205 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 CT 1 LEBENTHAL E PEDIATR RES 14 1356 980 2 LEBENTHAL E J PEDIATR 97 389 980 3 LEBENTHAL E PEDIATR RES 15 1240 981 4 CARREL O HELV PAEDIATR ACTA 36 405 981 5 LEBENTHAL E AM J GASTROENTEROL 75 436 981 6 ANON J AM DIET ASSOC 78 443 981 7 ANDERSEN DW PEDIATR RES 16 304 982 8 BOWLING SA AM J MED TECHNOLOGY 48 25 982 9 RANDOLPH VS AM J MED TECHNOLOGY 48 15 982 10 ADRIAENSSENS K ARCH DIS CHILD 57 553 982 11 LONGNECKER DS AM J PATHOL 107 103 982 12 SVENDSEN LS ACTA VET SCAND SUPPL 78 1982 1 982 13 LEBENTHAL E J PEDIATR 102 1 983 14 ANDERSEN DW J NUTR 113 430 983 15 COURTOIS P ANN BIOL CLIN 43 127 985 16 STEGINK LD METABOLISM 35 519 986 17 ANDERSEN DW J NUTR 117 274 987 PN 78011 RN 00792 AN 79038961 AU Griscom-N-T. TI Total body opacification. SO AJR. 1978 Nov. 131(5). P 919-25. (REVIEW). MJ IODIZED-OILS: du. RADIOGRAPHY: mt. MN ABSCESS: ra. AGE-FACTORS. ASCITES: ra. CHILD-PRESCHOOL. COMMON-BILE-DUCT: ab. CYSTIC-FIBROSIS: co. CYSTS: ra. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: ra. INTESTINAL-OBSTRUCTION: cn, ra. IODIZED-OILS: ad, ae. LIVER-DISEASES: ra. DIATRIZOATE-MEGLUMINE: du. RADIOGRAPHY: ae. REVIEW. TECHNOLOGY-RADIOLOGIC. URETERAL-OBSTRUCTION: ra. AB Total body opacification is the phenomenon whereby introduction of iodinated contrast material into the blood stream renders structures differentially radiopaque according to their blood supply. The term implies the physiology: it is the whole body which is opacified, in proportion to the iodine in its plasma and extravascular extracellular fluid at the time of the radiograph. To the density inherent in the tissue before injection is added the opacity of the iodine circulating through and transuding into it. Just as tissues, even those of "water density," have minor intrinsic differences in x-ray absorption, so they may also have minor differences in blood flow and transudation into the extracellular fluid; the combination of these minor differences often makes the invisible visible. RF 001 ALFIDI R AM J ROENTG RAD THER NUCL MED 124 199 975 002 NEW PFJ RADIOLOGY 110 109 974 003 MATEGRANO VC RADIOLOGY 125 135 977 004 BIRNHOLZ JC RADIOLOGY 105 303 972 005 PHILLIPS JC RADIOLOGY 112 17 974 006 IMRAY TJ AM J ROENTG RAD THER NUCL MED 125 60 975 007 WIRTANEN GW RADIOLOGY 108 51 973 008 HEGEDUS V INVEST RADIOL 9 56 974 009 KORMANO M RADIOLOGY 121 379 976 010 GOODING CA RADIOLOGY 102 77 972 011 MESSINA AV RADIOLOGY 120 345 976 012 SHANSER JD RADIOL SOC NORTH AM ANNU MTG 977 013 LACHMAN RS PEDIATR RADIOL 3 117 975 014 YOUNG LW ANN RADIOL (PARIS) 17 391 974 015 MONCADA R AM J ROENTGENOL 129 583 977 016 MARTIN DJ BR J RADIOL 45 185 972 017 EFFMANN EL RADIOL SOC NORTH AM ANNU MTG 975 018 GRISCOM NT AM J ROENTG RAD THER NUCL MED 93 447 965 019 RABINOWITZ JG J CAN ASSOC RADIOL 24 226 973 020 FELLOWS KE RADIOL CLIN NORTH AM 10 349 972 021 ROSENFIELD N RADIOLOGY 114 113 975 022 KURLANDER GJ RADIOLOGY 89 1075 967 023 BERDON WE AM J ROENTG RAD THER NUCL MED 118 18 973 024 ROBINSON AE RADIOLOGY 122 749 977 025 ANON N ENGL J MED 295 608 976 026 CROWE JE AM J ROENTGENOL 130 167 978 027 LEAPE LL PEDIATRICS 47 101 971 028 MEYERS MA RADIOLOGY 117 539 975 029 GENEREUX GP J CAN ASSOC RADIOL 21 242 970 030 MONCADA R AM J ROENTGENOL 129 583 977 031 MORIN ME RADIOLOGY 121 307 976 032 BALIKIAN JP AM J ROENTG RAD THER NUCL MED 122 692 974 033 HATFIELD PM AM J ROENTG RAD THER NUCL MED 119 687 973 034 LOVE L AM J ROENTG RAD THER NUCL MED 122 299 974 035 LILIENFELD RM J UROL 115 123 976 036 MITTY HA AM J ROENTG RAD THER NUCL MED 115 630 972 037 SMITH AM AM J ROENTG RAD THER NUCL MED 104 389 968 038 KURTI DJ AM J ROENTG RAD THER NUCL MED 119 576 973 039 RABUSHKA SE RADIOLOGY 109 549 973 040 MORGAN H RADIOLOGY 97 301 970 041 BRILL PW AM J ROENTGENOL 127 289 976 042 STANLEY P PEDIATR RADIOL 5 58 976 043 BALSYS R INVEST RADIOL ABSTR 12 393 977 044 BARBARIC ZL RADIOLOGY 123 587 977 045 BENTON C RADIOLOGY 104 61 972 046 GRISCOM NT J PEDIATR SURG 1 76 966 047 DUNBAR JS RADIOL CLIN NORTH AM 10 367 972 048 WITTEN DM AM J ROENTG RAD THER NUCL MED 119 832 973 049 MARTIN DJ RADIOL CLIN NORTH AM 13 359 975 050 TALNER LB CONTRIB NEPHROL 5 3 977 051 STANDEN JR AM J ROENTG RAD THER NUCL MED 93 473 965 052 ANSELL G BR J RADIOL 43 395 970 053 DOUST BD RADIOLOGY 104 557 972 054 MOREAU JF RADIOLOGY 115 329 975 055 ELKIN M RADIOLOGY 116 259 975 056 DUNBAR JS AM J ROENTG RAD THER NUCL MED 110 520 970 057 GRISCOM NT RADIOLOGY 106 385 973 058 OCONNOR JF AM J ROENTG RAD THER NUCL MED 90 63 963 059 HOLT JF YEARBOOK OF RADIOLOGY 965 060 GOODING CA AM J ROENTG RAD THER NUCL MED 123 802 975 061 MCALISTER W SOC PEDIATR RADIOL ANNU MTG 978 062 BORRA S N ENGL J MED 284 592 971 063 BERGMAN LA N ENGL J MED 279 1277 968 CT 1 CREMIN BJ AM J ROENTGENOL 132 857 979 2 MAHMUD F AM J ROENTGENOL 133 1180 979 3 DEAN PB INVEST RADIOL 15 S164 980 4 AUSTIN RM CLIN RADIOL 31 697 980 5 KATZBERG RW RADIOLOGY 134 297 980 6 MORIN ME CRC CRIT REV DIAGN IMAGING 15 237 981 7 NEWHOUSE JH AM J ROENTGENOL 136 463 981 8 AXIOTIS CA J CLIN GASTROENTEROL 5 541 983 9 MINDELZUN RE SEM ROENTGENOL 19 259 984 PN 78012 RN 00793 AN 78253890 AU Strauss-A. Mendes-E. TI Exocrine disorder in asthmatics: demonstration of high sweat chloride levels in chronic patients and their relatives. SO Allergol-Immunopathol (Madr). 1978 Jan-Feb. 6(1). P 19-24. MJ ASTHMA: pp. CHLORIDES: an. CYSTIC-FIBROSIS: pp. SWEAT: an. MN ADOLESCENCE. ADULT. ASTHMA: et, fg. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co, di. FEMALE. HUMAN. INFANT. MALE. MIDDLE-AGE. RESPIRATORY-HYPERSENSITIVITY: et. AB In 87 patients with svere chronic asthma, the sweat chloride (Cl) level was determined, and increased concentrations were found in 12 (13.7%). In 76% of the relatives of these patients increased sweat Cl levels were also found. The authors give data of their clinical and allergy study and conclude that 13.7% of the patients under investigation had clinical characteristics of asthma, allergy and a functional exocrine disorder. The exocrinopathy may be an important factor in the pathophysiology of certain types of asthma. RF 001 CAPLIN I ANN ALLERGY 31 320 973 002 ANON IN: PORTER R 38 174 971 003 COUNAHAN R ARCH DIS CHILD 50 477 975 004 FIGUEIRA F REV PAUL MED 51 99 967 005 KOPITO L PEDIATRICS 43 794 969 006 MEARNS MB LANCET 1 538 967 007 RACHELEFSKY GS AM J DIS CHILD 128 355 974 008 SPOCK A PEDIATR RES 1 173 967 009 STRAUSS A J PEDIATR RIO DE JANEIRO 35 12 970 010 WARNER JO LANCET 1 990 976 011 WOOD RE AM REV RESPIR DIS 113 833 976 PN 78013 RN 00794 AN 78142110 AU Jacob-R-A. Sandstead-H-H. Solomons-N-W. Rieger-C. Rothberg-R. TI Zinc status and vitamin A transport in cystic fibrosis. SO Am-J-Clin-Nutr. 1978 Apr. 31(4). P 638-44. MJ CYSTIC-FIBROSIS: me. VITAMIN-A: me. ZINC: me. MN ADOLESCENCE. CHILD. HAIR: an. HUMAN. RETINOL-BINDING-PROTEINS: bl. SERUM-ALBUMIN: me. AB Zinc status and the retinol transport system were examined in 18 retinol supplemented cystic fibrosis (CF) patients and 40 age-matched controls. Plasma vitamin A was significantly lower in the CF group as compared to the controls and correlated positively with plasma retinol-binding protein (RBP) in both the CF and control groups. Plasma zinc of the CF group was not significantly lower than controls whereas hair zinc was. Plasma zinc was positively correlated with plasma RBP, vitamin A, and albumin in the CF group but not in the controls. Plasma concentrations of vitamin A, RBP, albumin, and zinc decreased with age in the CF group but not in the controls. The data support previous suggestions that low plasma vitamin A levels in CF are due to defects in the retinol transport system. The zinc status of the CF groups as a whole was judged to be low-normal however a subgroup of CF patients were in the marginal to deficient category. This subgroup also had lower levels of plasma vitamin A and RBP. The data suggest that zinc may be a contributing factor in the low plasma vitamin A/RBP levels of CF patients with marginal or deficient zinc status. RF 001 ANDERSEN DH J PEDIATR 15 763 939 002 KANAI MA J CLIN INVEST 47 2025 968 003 SMITH FR J CLIN INVEST 49 1754 970 004 UNDERWOOD BA PEDIATR RES 6 26 972 005 SMITH FR J LAB CLIN MED 80 423 972 006 SMITH JC JR SCIENCE 181 954 973 007 SMITH JE J LAB CLIN MED 84 692 974 008 HALSTED JA LANCET 1 322 970 009 BROWN ED J NUTR 106 563 976 010 SMITH JC JR J NUTR 106 569 976 011 HUBER AM J NUTR 105 1486 975 012 CARNEY SM J NUTR 106 1773 976 013 SINHA SN AM J CLIN PATHOL 54 570 970 014 NEELD JB J NUTR 79 454 963 015 KLEVAY LM AM J CLIN NUTR 23 284 970 016 PALIN HD PEDIATR RES ABST 343 10 358 976 017 KNOEPFLE G Z KINDERHEILK 119 279 975 018 HENKIN RI AM J MED SCI 264 401 972 019 KIRCHGESSNER M IN: PRASAD AS 1 189 976 020 MORRISON SA AM J CLIN NUTR 30 612 977 021 BEISEL WR ANN INTERN MED 67 744 967 022 PEKAREK RS PROC SOC EXP BIOL MED 138 728 971 023 SANDSTEAD HH IN: PRASAD AS 33 976 024 BEISEL WR IN: PRASAD AS 1 87 976 025 HALSTED JA GASTROENTEROLOGY 67 193 974 026 MUTO Y J BIOL CHEM 247 2542 972 027 SMITH FR AM J CLIN NUTR 26 973 973 028 SMITH FR AM J CLIN NUTR 26 982 973 029 SMITH FR J CLIN INVEST 50 2426 971 030 DI SANTAGNESE PA PEDIATRICS 18 387 956 031 CRAIG JM AM J DIS CHILD 93 357 957 032 STERLING K J CLIN INVEST 30 1238 951 033 PITTMAN FE AM J DIS CHILD 108 360 964 034 STROBER W PEDIATRICS 43 416 969 035 GIROUX EL BIOINORG CHEM 5 211 976 CT 1 PALIN D AM J CLIN NUTR 32 1253 979 2 PEKAREK RS AM J CLIN NUTR 32 1466 979 3 SOLOMONS NW AM J CLIN NUTR 32 856 979 4 ATUKORALA S BR J CANCER 40 927 979 5 CHASE HP J PEDIATR 95 337 979 6 CASPER RC AM J CLIN NUTR 33 1801 980 7 SOLOMONS NW AM J CLIN NUTR 33 2031 980 8 MAGAZZU G RIV ITAL PEDIATR 7 73 981 9 ANDREWS WS J PEDIATR SURG 16 284 981 10 HOOPER PL AM J CLIN NUTR 34 970 981 11 SOLOMONS NW AM J CLIN NUTR 34 462 981 12 GORDON EF J PEDIATR 99 341 981 13 HANSEN MA ANNU REV NUTRITION 2 151 982 14 MIKHAIL MM EUR J CLIN INVEST 12 345 982 15 SOLOMONS NW AM J CLIN NUTR 35 1048 982 16 SOLOMONS NW J AM DIET ASSOC 80 115 982 17 DELUCA F EUR J PEDIATR 138 327 982 18 VANDERHOOF JA DIG DIS SCI 28 300 983 19 HANSEN RC ARCH DERMATOL 119 51 983 20 BALY DL AM J CLIN NUTR 40 199 984 21 HUBBARD VS SEM RESPIR MED 6 308 985 22 DELVES HT CLIN ENDOCRINOL METAB 14 725 985 23 VANWOUWE JP SCI TOTAL ENVIRON 42 149 985 24 RASMUSSEN M J PEDIATR GASTROENTEROL NUTR 5 397 986 PN 78014 RN 00795 AN 78208255 AU Finley-P-R. Dye-J-A. Lichti-D-A. Byers-J-M-3d. Williams-R-J. TI A modified ion-selective electrode method for measurement of chloride in sweat. SO Am-J-Clin-Pathol. 1978 Jun. 69(6). P 615-8. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. SWEAT: an. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. ELECTRODES: is. HUMAN. INFANT. AB A modified method of analysis of sweat chloride concentration with an ion-selective electrode is presented. The original method of sweat chloride analysis proposed by the Orion Research Corporation (Cambridge, Massachusetts 02139) is inadequate because it produces erratic and misleading results. The modified method was compared with the reference quantitative method of Gibson and Cooke. In the modified method, individual electrode pads are cut and placed in the electrodes rather than using the pads supplied by the company; pilocarpine nitrate (2,000 mg/l) is used in place of pilocarpine HCl (640 mg/l); sodium bicarbonate as the weak electrolyte is used instead of K2SO4. A 10-minute period for sweat accumulation is employed rather than a zero-time collection as in the original Orion method. The modification has been studied for reproducibility in individuals, reproducibility between right and left arm in individuals; it has been compared extensively with the quantitative method of Gibson and Cooke, both in normal individuals and in patients with cystic fibrosis. There is excellent agreement between the modified method and the quantitative reference method. There appears to be a slight bias toward higher concentrations of chloride from the right arm compared with the left arm, but this difference is not medically significant. RF 001 BOWMAN BH N ENGL J MED 294 937 976 002 BRIMBLECOMBE FSW LANCET 2 1428 973 003 DI SANTAGNESE PA AM J MED 15 777 953 004 GIBSON LE PEDIATRICS 23 545 959 005 IBBOTT FA STANDARD METH CLIN CHEM 5 101 965 006 ANON GAP CONF REP PROB SWEAT TESTI 975 007 ANON J PEDIATR 88 711 976 008 ROSENLUND ML CRC CRIT REV CLIN LAB SCI 3 257 972 009 SHWACHMAN H ANN NY ACAD SCI 93 600 962 010 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 011 SZABO L CLIN CHEM 19 727 973 012 WARWICK WJ CF CLUB ABST 16 58 975 PN 78015 RN 00796 AN 78121163 AU Rosenstein-B-J. TI Cystic fibrosis presenting with the meconium plug syndrome. SO Am-J-Dis-Child. 1978 Feb. 132(2). P 167-9. MJ CYSTIC-FIBROSIS: di. INFANT-NEWBORN-DISEASES: di. INTESTINAL-OBSTRUCTION: di. MECONIUM. MN CASE-REPORT. CYSTIC-FIBROSIS: co, ra. DIAGNOSIS-DIFFERENTIAL. FEMALE. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: et, ra. INTESTINAL-OBSTRUCTION: et, ra. MALE. MEGACOLON: co, di. SYNDROME. AB The meconium plug syndrome (MPS) is a common cause of low intestinal obstruction in newborn infants. Usually, it is benign and not associated with other intestinal dysfunction. We describe the cases of three infants in whom there was intestinal obstruction consistent with the MPS and in which cystic fibrosis (CF) was later documented. We suggest that there is an association between the MPS and CF, and that the condition of all infants with the MPS be evaluated for the possibility of both Hirschsprung's disease and CF. RF 001 CLATWORTHY HW SURGERY 39 131 956 002 ELLIS DG J PEDIATR SURG 1 54 966 003 SWISCHUK LE AM J ROENTG RAD THER NUCL MED 103 339 968 004 MIKITY VG RADIOLOGY 88 740 967 005 GILLIS DA CAN MED ASSOC J 92 225 965 006 GROSSMAN H AM J ROENTG RAD THER NUCL MED 97 227 966 007 EMERY JL ARCH DIS CHILD 32 17 957 008 CHING NPH ARCH SURG 90 65 965 009 SOKAL MM N ENGL J MED 286 823 972 010 BRUNS WT AM J DIS CHILD 131 71 977 011 POOLE CA J PEDIATR SURG 11 1011 976 012 GREEN MN PEDIATRICS 41 989 968 013 DOLAN TF JR J PEDIATR SURG 9 821 974 014 STEPHAN U PEDIATRICS 55 35 975 CT 1 HEN J PEDIATRICS 66 466 980 2 ROSENSTEIN BJ AM J DIS CHILD 134 72 980 3 STARSHAK RJ GASTROINTEST RADIOL 6 75 981 4 PARK RW GASTROENTEROLOGY 81 1143 981 5 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 6 MULLER F PRENAT DIAGN 5 109 985 7 ABRAMSON SJ SEM ROENTGENOL 22 97 987 8 HALL SL CLIN PEDIATR 26 191 987 PN 78016 RN 00797 AN 78121196 AU Gunn-T. Belmonte-M-M. Colle-E. Dupont-C. TI Edema as the presenting symptom of cystic fibrosis: difficulties in diagnosis. SO Am-J-Dis-Child. 1978 Mar. 132(3). P 317-8. MJ CYSTIC-FIBROSIS: co. EDEMA: et. MN CHLORIDES: an. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. HYPOPROTEINEMIA: et. INFANT. MALE. SWEAT: an. EX Hypoproteinemia and edema may develop in infants with cystic fibrosis whatever their diet although prior ingestion of soy bean formula appears to be a predisposing factor, and a negative sweat chloride test may be present in a CF child during the hypoproteinemic phase of the illness. The diagnosis cannot be excluded until a negative test with adequate secretion of sweat is obtained after edema and hypoproteinemia have resolved. RF 001 FLEISHER DS J PEDIATR 64 341 964 002 MULNE A PEDIATR RES ABST 342 10 358 976 003 BILLE BSV ACTA PAEDIATR SCAND 44 435 955 004 GOLDMAN AS J PEDIATR 59 301 961 005 ANDERSON C MED J AUST 2 195 966 006 MASI M INT ANESTHESIOL CLIN 53 55 971 007 MACLEAN WC JR J PEDIATR 83 86 973 008 LEE PA JAMA 228 585 974 009 VLACHOS P J PEDIATR 84 926 974 010 DOLAN TF JR CLIN PEDIATR 9 295 970 011 LOCK J J PEDIATR 84 912 974 012 PAXSON CL JR JAMA 230 1257 974 013 BASS HN PEDIATRICS 59 126 977 014 SHAHIDI NT J PEDIATR 59 533 961 015 ROGER R J PEDIATR 86 264 975 CT 1 NIELSEN OH J PEDIATR GASTROENTEROL NUTR 1 355 982 2 TURKEL SB CLIN PERINATOL 9 613 982 3 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 4 CHRISTOFFEL KS J PEDIATR 107 231 985 PN 78017 RN 00798 AN 79059455 AU Townes-P-L. Kopelman-A-E. TI Meconium plug syndrome, cystic fibrosis, and exocrine pancreatic deficiency [letter]. SO Am-J-Dis-Child. 1978 Oct. 132(10). P 1043-4. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: et. MECONIUM. PANCREATIC-DISEASES: co. MN CASE-REPORT. HUMAN. INFANT-NEWBORN. SYNDROME. EX We have observed severe meconium plug syndrome (MPS) in a newborn infant who was found during the neonatal period to have extensive exocrine pancreatic deficiency. Cystic fibrosis was excluded by several sweat chloride tests. Our observation of MPS in a patient with exocrine pancreatic deficiency suggests that the MPS observed in the CF patients reported by Rosenstein is probably caused by pancreatic deficiency. The frequent occurrence of meconium ileus (MI) in CF and the rarity of MPS in CF is curious, since they may be different manifestations of the same pathologic process. Conceivably, MPS may occur with total absence of pancreatic enzymes (as in our patient and in a few patients with CF). Low levels of enzymes in CF may limit expression to meconium ileus. We wish to suggest the evaluation of exocrine pancreatic function in these patients even if CF has been excluded by sweat tests. - There is conflicting, albeit fragmentary, data suggesting that exocrine pancreatic deficiency may not be the sole cause of meconium obstruction in CF. Several investigators have suggested that MI may be the result of abnormal protein and mucoprotein secretions by the intestinal goblet cells and not from lack of pancreatic enzymes alone. While these observations suggest that exocrine pancreatic deficiency may not play a major role in all instance of meconium obstruction, it is important to note that there may be little correlation between the histologic appearance of the pancreas and exocrine function. RF 001 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 001 ROSENSTEIN BJ AM J DIS CHILD 132 167 978 002 THOMAIDIS TS J PEDIATR 63 444 963 002 GROSSMAN H AM J ROENTG RAD THER NUCL MED 97 227 966 003 OPPENHEIMER EH ARCH PATHOL 96 149 973 CT 1 ROSENSTEIN BJ AM J DIS CHILD 134 72 980 PN 78018 RN 00799 AN 78208347 AU Spiro-R-K. Landman-M-E. TI Meconium ileus equivalent. SO Am-J-Gastroenterol. 1978 Mar. 69(3 Pt 1). P 302-6. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: et. MN ACETYLCYSTEINE: tu. ADOLESCENCE. ADULT. AGE-FACTORS. CASE-REPORT. CHILD. CHILD-PRESCHOOL. FECES-IMPACTED: co. HUMAN. ILEUM: pa, ra. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: pa. MALE. MECONIUM. RESPIRATION-ARTIFICIAL. AB A case of meconium ileus equivalent in a 30-year old man is presented. The pathology and therapy for the condition are discussed. RF 001 HUNTON DB GASTROENTEROLOGY 50 99 966 002 DAVIDSON M HOSP PRACT 2 47 976 003 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 004 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 005 QUINLAN MF AUSTRALAS ANN MED 16 84 967 006 SIGLER RM MAYO CLIN PROC 40 477 965 007 ROWE MI AM J SURG 125 185 973 008 SCHILLER M AM J SURG 122 22 971 009 BROWN PM N ENGL J MED 263 544 960 010 NANKIN P AM J SURG 123 609 972 011 LILLIBRIDGE CB J PEDIATR 71 887 967 CT 1 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 2 ROSENBERG E KLIN PAEDIATR 195 323 983 3 RUBINSTEIN S PEDIATRICS 78 473 986 PN 78019 RN 00800 AN 78184587 AU Parry-M-F. Neu-H-C. TI A comparative study of ticarcillin plus tobramycin versus carbenicillin plus gentamicin for the treatment of serious infections due to gram-negative bacilli. SO Am-J-Med. 1978 Jun. 64(6). P 961-6. MJ ANTIBIOTICS: tu. CARBENICILLIN: tu. GENTAMICINS: tu. GRAM-NEGATIVE-AEROBIC-BACTERIA: de. PENICILLINS: tu. PSEUDOMONAS-INFECTIONS: dt. TICARCILLIN: tu. TOBRAMYCIN: tu. MN COMPARATIVE-STUDY. CYSTIC-FIBROSIS: dt. DRUG-RESISTANCE-MICROBIAL. DRUG-THERAPY-COMBINATION. HUMAN. KIDNEY-FUNCTION-TESTS. KIDNEY: de. PNEUMONIA: dt. SEPTICEMIA: dt. AB The combination of ticarcillin plus tobramycin (TT) or carbenicillin plus gentamicin (CG) was used to treat 82 patients with severe systemic gram-negative infection in a prospective, randomized study. Pseudomonas aeruginosa was the primary pathogen in 7 (93 per cent) of these patients. Patients treated with TT responded more frequently (92 per cent or 37 of 40) than patients treated with CG (71 per cent or 30 of 42) (p is less than 0.05). This difference was primarily due to a greater response to TT in patients with pulmonary infections (93 per cent versus 68 per cent) and infections due to Pseudomonas (92 per cent versus 70 per cent). Severity of underlying disease was also an important determinant of response. Except for a greater incidence of hepatotoxicity with CG (23 per cent versus 3 per cent; p is less than 0.02), there was no difference in toxicity, colonization with drug-resistant microorganisms or superinfection between the two treatment groups. The combination of TT appears to be superior to CG for the treatment of pulmonary infections due to Pseudomonas aeruginosa. RF 001 ANDRIOLE VT J INFECT DIS 129 124 974 002 LUMISH RM J INFECT DIS 133 538 976 003 SCOTT RE ANTIMICROB AGENTS CHEMOTHER 10 646 976 004 MYEROWITZ RL J INFECT DIS 124 239 971 005 MCCABE WR BULL NY ACAD MED 51 1084 975 006 KLASTERSKY J J INFECT DIS 129 187 974 007 KLASTERSKY J ANTIMICROB AGENTS CHEMOTHER 7 640 975 008 PARRY MF J PEDIATR 90 144 977 009 FELD R J INFECT DIS 135 61 977 010 SMITH CR N ENGL J MED 296 349 977 011 NEU HC ANTIMICROB AGENTS CHEMOTHER 10 385 970 012 NEU HC J INFECT DIS SUPPL 134 3 976 013 NEU HC ANTIMICROB AGENTS CHEMOTHER 8 457 975 014 PARRY MF J INFECT DIS 133 46 976 015 MCHENRY MC ANN INTERN MED 74 192 971 016 BAUER AW AM J CLIN PATHOL 45 493 966 017 NEU HC APPL MICROBIOL 21 66 971 018 FU KP ANTIMICROB AGENTS CHEMOTHER 10 511 976 019 PARRY MF ANTIMICROB AGENTS CHEMOTHER 9 625 976 020 SHWACHMAN H AM J DIS CHILD 96 6 958 021 PARRY MF J INFECT DIS SUPPL 134 194 976 022 HEWITT WL POSTGRAD MED J SUPPL 7 50 55 974 023 LIPNER HI J LAB CLIN MED 86 183 975 024 BROWN CH 3RD N ENGL J MED 291 265 974 025 PARRY MF J INFECT DIS 134 476 976 026 LAU WK AM J MED 62 959 977 CT 1 STEWART C DRUG INTEL CLIN PHARM 13 692 979 2 MEYER M CAN J MICROBIOL 25 1232 979 3 HOPEFL AW AM J HOSP PHARM 36 178 979 4 CHOW M CONN MED 43 25 979 5 PANCOAST SJ AM J MED 67 747 979 6 ANDRIOLE VT J LAB CLIN MED 94 196 979 7 PENNINGTON JE J INFECT DIS 140 881 979 8 NEU HC INFECTION 8 S 62 980 9 KOHLER RB J CLIN MICROBIOL 12 39 980 10 BROGDEN RN DRUGS 20 325 980 11 SORKNESS R AM J HOSP PHARM 37 555 980 12 YOUNG LS J ANTIMICROB CHEMOTHER 8 121 981 13 BELL WE ANN NEUROL 9 313 981 14 HOPEFL AW DRUG INTEL CLIN PHARM 15 697 981 15 ELLIS PP CAN J OPHTHALMOL 16 79 981 16 MOZZANA R HAEMATOLOGICA 66 634 981 17 BRINKER KR J LAB CLIN MED 98 292 981 18 HYATT AC J PEDIATR 99 307 981 19 ELIOPOULOS GM REV INFECT DIS 4 282 982 20 BELL WE SEM PERINATOL 6 1 982 21 PARRY MF J ANTIMICROB CHEMOTHER 9 273 982 22 PENNINGTON JE ANTIMICROB AGENTS CHEMOTHER 22 406 982 23 ABRAMOWITZ PW AM J HOSP PHARM 39 1176 982 24 TENENBAUM MJ MED CLIN NORTH AM 66 17 982 25 GRAFT DF J PEDIATR 100 497 982 26 KOHLER RB ARCH INTERN MED 142 1335 982 27 BODEY GP REV INFECT DIS 5 279 983 28 SAVOIA D DRUGS UNDER EXP CLIN RES 9 143 983 29 CHIN NX J ANTIMICROB CHEMOTHER 11 33 983 30 SRINIVASAN S INFECTION 11 291 983 31 BINT AJ J ANTIMICROB CHEMOTHER 12 115 983 32 GASPARETTO A J ANTIMICROB CHEMOTHER 12 171 983 33 SHLAES DM PEDIATR CLIN NORTH AM 30 121 983 34 KAHLMETER G J ANTIMICROB CHEMOTHER 13 9 984 35 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 36 ENG RHK INT J DERMATOL 23 153 984 37 YOUNG LS AM J MED 76 61 984 38 MOORE RD AM J MED 77 657 984 39 SCULLY BE ARCH INTERN MED 144 57 984 40 ACAR JF REV INFECT DIS 7 S513 985 41 LEVISON ME REV INFECT DIS 7 S656 985 42 SCULLY BE REV INFECT DIS 7 S669 985 43 CONE LA ANTIMICROB AGENTS CHEMOTHER 28 33 985 44 BALTCH AL AM J MED 79 8 985 45 WILSON CB J PEDIATR 106 1049 985 46 HANNO P UROL CLIN NORTH AM 13 577 986 47 ARPI M CHEMOTHERAPY 32 68 986 48 EVANS DA AM J MED 80 39 986 PN 78020 RN 00801 AN 78254310 AU Knox-G-E. Palmer-M-D. Huddleston-J-F. TI Fetal cystic fibrosis presenting as dystocia due to midgut volvulus with lethal perforation. SO Am-J-Obstet-Gynecol. 1978 Jul 15. 131(6). P 698-9. MJ CYSTIC-FIBROSIS: di. DYSTOCIA: di. FETAL-DISEASES: di. MN ADULT. DIAGNOSIS-DIFFERENTIAL. DYSTOCIA: et. FEMALE. HUMAN. INTESTINAL-OBSTRUCTION: co. PERITONITIS: co, et. PREGNANCY. RUPTURE-SPONTANEOUS. EX Fetal or neonatal volvulus is a rare event and is usually associated with an anatomic defect. Massive abdominal distension secondary to a ruptured or unruptured volvulus has never been reported as a cause of obstructed labor. The present report documents such a case, secondary to chemical peritonitis resulting from a ruptured midgut volvulus. This case demonstrates an unusual presentation of presumed cystic fibrosis with meconium ileus and volvulus which led to ischemic infarction and perforation of the small bowel. This case likewise emphasizes the need to consider the presence of other fetal anomalies, such as omphalocele or gastroschisis, when the usual maneuvers fail to relieve shoulder dystocia. RF 001 KRASNA IH J PEDIATR SURG 8 615 973 002 SROUJI MN J PEDIATR SURG 9 779 974 CT 1 SHALEV J J MED GENET 20 229 983 PN 78021 RN 00802 AN 78078929 AU Davis-S-D. Bruns-W-T. TI Effects of sputum from patients with cystic fibrosis on the activity in vitro of 5 antimicrobial drugs on Pseudomonas aeruginosa. SO Am-Rev-Respir-Dis. 1978 Jan. 117(1). P 176-8. MJ ANTIBIOTICS: pd. CYSTIC-FIBROSIS. PSEUDOMONAS-AERUGINOSA: de. SPUTUM. MN CARBENICILLIN: pd. GENTAMICINS: pd. HUMAN. MICROBIAL-SENSITIVITY-TESTS. NEOMYCIN: pd. POLYMYXIN-B: pd. TOBRAMYCIN: pd. SUPPORT-U-S-GOVT-P-H-S. AB By in vitro tests on 12 strains of Pseudomonas aeruginosa, sputum from patients with cystic fibrosis sharply increased the minimal bactericidal concentrations of polymyxin B and neomycin. Sputum had a lesser effect on tests with gentamicin and tobramycin and essentially none on tests with carbenicillin. RF 001 NEWTON BA NATURE 172 160 953 002 NEWTON BA J GEN MICROBIOL 10 491 954 003 POTTER JL ANN NY ACAD SCI 106 692 963 004 POTTER JL PEDIATRICS 36 714 965 005 GARROD LP J CLIN PATHOL 22 534 969 006 SAGGERS BA J CLIN PATHOL 23 266 970 007 KUNIN CM J INFECT DIS 121 55 970 008 DAVIS SD J INFECT DIS 123 392 971 009 DAVIS SD J INFECT DIS 124 610 971 010 CHEN CH ANTIMICROB AGENTS CHEMOTHER 2 331 972 011 DAVIS SD APPL MICROBIOL 23 775 972 012 DAVIS SD ANTIMICROB AGENTS CHEMOTHER 1 466 972 013 DAVIS SD CHEMOTHERAPY 19 243 973 014 DIENSTAG J ANTIMICROB AGENTS CHEMOTHER 1 41 972 015 ZIMELIS VM J INFECT DIS 127 663 973 016 BRYANT RE ANTIMICROB AGENTS CHEMOTHER 6 702 974 017 D AMATO RF ANTIMICROB AGENTS CHEMOTHER 7 596 975 018 LENNETTE EH MANUAL OF CLINICAL MICROBIOLO 974 019 RELLER LD J INFECT DIS 130 454 974 020 HUANG NN GUIDE TO DRUG THERAPY IN PATI 974 CT 1 ALEXANDER MR CHEST 75 675 979 2 VAUDAUX P J ANTIMICROB CHEMOTHER 8 17 981 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 4 MAXWELL D J ANTIMICROB CHEMOTHER 11 203 983 5 STACK BHR THORAX 40 358 985 6 MENDELMAN PM AM REV RESPIR DIS 132 761 985 7 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 PN 78022 RN 00803 AN 78164287 AU Lemen-R-J. Gates-A-J. Mathe-A-A. Waring-W-W. Hyman-A-L. Kadowitz-P-D. TI Relationships among digital clubbing, disease severity, and serum prostaglandins F2alpha and E concentrations in cystic fibrosis patients. SO Am-Rev-Respir-Dis. 1978 Apr. 117(4). P 639-46. MJ CYSTIC-FIBROSIS: di. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: et. PROSTAGLANDINS-E: bl. PROSTAGLANDINS-F: bl. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, co. FEMALE. HUMAN. MALE. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: bl. SUPPORT-U-S-GOVT-P-H-S. VITAL-CAPACITY. AB Nine patients with cystic fibrosis but without digital clubbing (Group A) were matched, prospectively, by sex and approximate age to 9 cystic fibrosis patients with digital clubbing (Group B) and to 9 normal persons (control subjects). Patients in Group B had significantly (P less than 0.05) lower clinical scores and forced vital capacity than did those in Group A, indicating more severe pulmonary disease in the former; however, other spirometer tests of pulmonary function revealed no differences between Groups A and B. The degree of digital clubbing had significant (P less than 0.05) linear relationships to forced vital capacity (r = -0.73) and clinical scores (r = 0.853) for Groups A and B. Plasma concentrations of prostaglandins F2alpha and E were significantly increased (P less than 0.05) in both Group A (X +/- SE, 0.48 +/- 0.03 and 0.87 +/- 0.10 ng per ml, respectively) and Group B (X +/- SE, 0.68 +/- 0.04 and 1.81 +/- 0.16 ng per ml, respectively) compared to the control group (X +/- SE, 0.14 +/- 0.01 and 0.39 +/- 0.02 ng per ml, respectively). Group B had significantly larger concentrations than did Group A; however, plasma concentrations of prostaglandin 15-keto-13, 14- dihydro metabolite were not different in Groups A and B, and were significantly smaller than in the control group. These studies suggest that the degree of digital clubbing in cystic fibrosis is related to the severity of the pulmonary involvement and that the prostaglandin system may play an important role in this disease. RF 001 BIGLER FC AM J PATHOL 34 237 958 002 LOVELL RRH CLIN SCI 9 299 950 003 DEGOWIN EL BEDSIDE DIAGNOSTIC EXAMINATIO 546 965 004 MELLINS RB CIRCULATION 33 143 966 005 WIERMAN WH JAMA 155 1459 954 006 WARING WW AM REV RESPIR DIS 104 166 971 007 MAUER EF AM HEART J 34 852 947 008 TURNER-WARWICK M THORAX 18 238 963 009 MCLAUGHLIN GE ANN INTERN MED 67 579 967 010 BASHOUR FA J LAB CLIN MED 58 613 961 011 GREEN K LANCET 2 1419 974 012 MATHE AA IN: RAMSWELL PW 3 169 977 013 WOOD RE AM REV RESPIR DIS 113 833 976 014 SHWACHMAN H AM J DIS CHILD 96 6 958 015 LEMEN RG IN: KENDIG EL JR 166 977 016 LEVINE L ADV BIOL SCI 9 71 973 017 MATHE AA ACTA PHYSIOL SCAND SUPPL 98 441 155 976 018 MATHE AA BIOCHEM PHARMACOL 26 181 977 019 SNEDECOR GW STATISTICAL METHODS 967 020 REID L MOD PROBL PEDIATR 10 195 967 021 ZUELZER WW PEDIATRICS 4 53 949 022 BEIER FR AM REV RESPIR DIS 94 430 966 023 COOK CD PEDIATRICS 24 181 959 024$ SIEGLER RL J PEDIATR 81 734 977 025 FERREIRA SH NATURE 216 868 967 026 BOWDEN DH AM J MED 38 226 965 027 ROSENTHAL A PEDIATRICS 59 588 977 028 LIEBERMAN J ANN INTERN MED 82 806 975 029 HALL GH J APPL PHYSIOL 21 923 966 030$ SAMUELSSON B ANNU REV BIOCHEM 898 669 975 031 NAKANO J PROC SOC EXP BIOL MED 144 506 973 032 HABBOUSHE C PEDIATRICS 48 973 971 033 SAID SI FED PROC 32 1972 973 034 KUO PT J PEDIATR 60 394 962 035 ROSENLUND ML PEDIATRICS 59 428 977 036 ELLIOTT RB PEDIATRICS 57 474 976 037 ELLIOTT RB PEDIATR RES 7 427 973 038 CAMPBELL IM PEDIATRICS 57 480 976 039 BAKHLE YS PHYSIOL REV 54 1007 974 040 LIEBERMAN J AM REV RESPIR DIS 109 743 974 CT 1 DODGE JA LANCET 2 475 978 2 SCHRIJEN F CLIN SCI MOL MED 55 485 978 3 LLOYDSTILL JD PEDIATRICS 64 50 979 4 GIRI SN J PHARMACOL METHODS 4 335 980 5 GIRI SN ENVIRON RES 21 467 980 6 CHANDLER DB PROSTAGLANDINS MED 7 571 981 7 METZ SA METABOLISM 30 299 981 8 GODARD P RESPIRATION 42 43 981 9 SHNEERSON JM BR J DIS CHEST 75 75 981 10 LEMANSKE RF AM REV RESPIR DIS 123 622 981 11 MARTINEZLAVIN M ARTHRITIS RHEUM 25 1186 982 12 LAVI Y J THORAC CARDIOVASC SURG 84 373 982 13 CHANDLER DB PROSTAGLANDINS LEUKOTR MED 11 11 983 14 MOON MA PROSTAGLANDINS 25 615 983 15 HANSEN RC ARCH DERMATOL 119 51 983 16 CHANDLER DB AM REV RESPIR DIS 128 71 983 17 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 18 DAVIS PB J LAB CLIN MED 104 203 984 19 RUSSI EW BR MED J 288 1160 984 20 SPRINCE NL N ENGL J MED 310 375 984 21 GIRI SN EXP LUNG RES 9 119 985 22 CHANDLER DB PROSTAGLANDINS LEUKOTR MED 19 139 985 23 LEUNG FW WEST J MED 142 345 985 24 MATUCCICERINIC M J RHEUMATOL 13 834 986 25 MISCHLER EH PEDIATR RES 20 36 986 26 PITTSTUCKER TJ ARCH DIS CHILD 61 576 986 27 BENDVOLD E J REPROD FERTIL 78 311 986 28 OZAKI T J APPL PHYSIOL 62 219 987 PN 78023 RN 00804 AN 78185256 AU Stern-R-C. Wood-R-E. Boat-T-F. Matthews-L-W. Tucker-A-S. Doershuk-C-F. TI Treatment and prognosis of massive hemoptysis in cystic fibrosis. SO Am-Rev-Respir-Dis. 1978 May. 117(5). P 825-8. MJ CYSTIC-FIBROSIS: co. HEMOPTYSIS: th. MN ADOLESCENCE. ADULT. ANTIBIOTICS: tu. BLOOD-TRANSFUSION. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: th. DRAINAGE. FEMALE. HEMOPTYSIS: et, mo. HUMAN. MALE. OHIO. PHYSICAL-THERAPY. PROGNOSIS. RECURRENCE. SUPPORT-U-S-GOVT-P-H-S. VITAMIN-K: tu. AB Massive hemoptysis (volume of expectorated blood > 300 ml in 24 hours) occurred in 38 (5 per cent) of 728 patients with cystic fibrosis. Twenty-two of the patients were in the midst of a relatively long-lasting flare-up of pulmonary infection. There was no other consistent precipitating event. All but 7 of the patients were admitted after the initial episode and were treated with parenteral antimicrobial drugs. Postural drainage was resumed as rapidly as possible after cessation of acute bleeding. Vitamin K was administered to all patients. Volumes of expectorated blood were as large as 2,500 ml with the initial episode. Blood pressure changes occurred in 4 patients. Five patients received blood transfusions. Bleeding stopped without surgical intervention in all patients, usually within 4 days. The 38 patients had a total of 85 episodes of bleeding. No surgical treatment was undertaken at this center. One patient underwent a lobectomoy at another hospital but continued to bleed postoperatively. Ten patients died (mean survival after hemoptysis, 2.5 years; range, 3 months to 6 years). This survival rate is comparable to that of another group of patients with equally severe lung disease who had never bled profusely. The other 28 patients remain alive from 7 months to 20 years after their initial episode. Our dat suggest that massive hemoptysis complicating cystic fibrosis may not be as ominous an event as has been believed in the past. Because of the tendency of most patients to stop bleeding without aggressive surgical intervention, future studies of new therapeutic measures for massive hemoptysis must have appropriate untreated control patients who receive only medical management. RF 001 HOLSCLAW DS J PEDIATR 76 829 970 002 LEVITSKY S JAMA 213 125 970 003 FELLOWS KE RADIOLOGY 114 551 975 004 ORES CN AM REV RESPIR DIS 99 790 969 005 KHAW K-T CF CLUB ABST 977 006 REMY J RADIOLOGY 122 33 977 007 DOERSHUK CF J PEDIATR 65 677 964 008 SHWACHMAN H AM J DIS CHILD 96 6 958 009 WOOD RE AM REV RESPIR DIS 113 833 976 010 MACK JF BR J RADIOL 38 422 965 011 SAW EC CHEST 70 589 976 012$ SAHEBJAMI H CHEST 69 131 977 CT 1 REMY J NOUV PRESSE MED 7 4306 978 2 FINK RJ CHEST 74 643 978 3 WOOD RE SOUTH MED J 72 189 979 4 MARACHE P ANN RADIOL (PARIS) 23 337 980 5 FAIRFAX AJ BR J DIS CHEST 74 345 980 6 TOM LWC ANN OTOL RHINOL LARYNGOL 89 419 980 7 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 8 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 9 MAGEE G LUNG 160 165 982 10 MARMON L J PEDIATR SURG 18 811 983 11 PORTER DK J THORAC CARDIOVASC SURG 86 409 983 12 BOBROWITZ ID ARCH INTERN MED 143 1343 983 13 PORTER DK ARCH INTERN MED 143 287 983 14 DAVIS PB CHEST 85 802 984 15 LESTER LA SEM RESPIR MED 6 285 985 16 BERKIN KE EUR J RESPIR DIS 67 103 985 17 TAFRESHI M NY STATE J MED 85 38 985 PN 78024 RN 00805 AN 78122210 AU Lopez-Vidriero-M-T. Reid-L. TI Chemical markers of mucous and serum glycoproteins and their relation to viscosity in mucoid and purulent sputum from various hypersecretory diseases. SO Am-Rev-Respir-Dis. 1978 Mar. 117(3). P 465-77. MJ BRONCHIAL-DISEASES: me. CYSTIC-FIBROSIS: me. GLYCOPROTEINS: an. MUCUS: an. SPUTUM: an. MN ASTHMA: me. BRONCHIECTASIS: me. BRONCHITIS: me. CHRONIC-DISEASE. FEMALE. FUCOSE: an. HUMAN. MALE. SIALOGLYCOPROTEINS: an. VISCOSITY. AB Mucus and serum are always present in sputum. The concentrations of their markers were measured in mucoid and purulent sputum from patients with chronic bronchitis, asthma, cystic fibrosis, or bronchiectasis. Differences in the concentrations of both mucous and serum glycoproteins were greater among different macroscopic types of sputum than among diseases for the same macroscopic type. Comparison between diseases showed that sputum from patients with asthma had the widest variation in concentrations of markers of mucous glycoprotein and dry macromolecular weight. When the astham group was further analyzed, it became apparent that patients with extrinsic asthma without chronic bronchitis represented a homogeneous group, whereas those with intrinsic asthma, with or without chronic bronchitis, were responsible for the wide variation. The concentrations of markers of mucous and serum glycoproteins in a sputum sample were correlated with the apparent viscosity of the sputum to establish the relative contribution of the glycoproteins to the viscosity of sputum. In mucoid sputum, the mucous glycoprotein was most important in determining the viscosity, whereas when in infected sputum, both mucous and serum components contributed to the viscosity. RF 001 DEGAND P BULL PHYSIOPATH RESPIR NANCY 9 199 973 002 BOAT TF IN: DULFANO MJ 243 973 003 ROBERTS GP EUR J BIOCHEM 50 265 974 004 CREETH JM BIOCHEM J 167 557 977 005 BROGAN TD THORAX 26 418 971 006 BROGAN TD THORAX 30 72 975 007 SPIRO SG J CLIN PATHOL 28 60 975 008 KEAL EE POSTGRAD MED J 47 171 971 009 LOPEZ-VIDRIERO MT THORAX 28 401 973 010 PUCHELLE E BULL PHYSIOPATH RESPIR NANCY 9 237 973 011 ROBERTS GP ARCH BIOCHEM BIOPHYS 173 528 976 012 LITT M BIORHEOLOGY 11 111 974 013 MARRIOTT C BIORHEOLOGY 11 119 974 014 MARRIOTT C BIORHEOLOGY 11 129 974 015 LITT M BIORHEOLOGY 13 37 976 016 CHEN TM BIORHEOLOGY 13 211 976 017 FORBES J LANCET 2 767 957 018 MUNIES R J PHARM SCI 57 824 968 019 LIEBERMAN J AM REV RESPIR DIS 97 654 968 020 DAVIS SS BULL PHYSIOPATH RESPIR NANCY 9 47 973 021 ANON LANCET 2 775 965 022 PORTER R CIBA FOUND STUDY GROUP 38 971 023 ANDERSON CM MED J AUST 1 419 958 024 CHARMAN J BIORHEOLOGY 9 185 972 025 WARREN L J BIOL CHEM 234 1971 959 026 GIBBONS MN ANALYST 80 268 955 027 ANTONOPOULOS CA ACTA CHEM SCAND 16 1521 962 028 DAS I FED EUR BIOCHEM SOC 9TH MTG 438 974 029 PALMER KNV BR J DIS CHEST 64 185 970 031 LAMB D HISTOCHEM J 4 91 972 032 JONES R HISTOCHEM J 5 19 973 033 LAMB D THESIS 969 034 KEAL EE THESIS 970 035 LAMB D J PATHOL 98 213 969 036 LAMB D BR J DIS CHEST 66 239 972 037 JONES R J PATHOL 116 1 975 038 JONES R PROC INT SYMP ON MUCUS I 397 976 039 MCCARTHY C Q J EXP PHYSIOL 49 85 964 040 LAMB D J PATHOL BACTERIOL 96 87 968 041 JONES R BR MED J 2 142 972 042 JONES R BR J EXP PATHOL 54 229 973 043 MATTHEWS LW AM REV RESPIR DIS 88 199 963 044 DENNIS EG J ALLERGY 35 464 964 045 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 046 HOUSTON JC THORAX 8 207 953 047 KEAL EE IN: STEIN M 223 975 048 EARLE BV THORAX 8 195 953 049 GLYNN AA THORAX 15 142 960 050 GORDON WI PROC SYMP NATURE OF ASTHMA 25 964 051 VIDAL J COLLOQ INT PATHOL THORAC LILL 69 968 052 DUNNILL MS IN: STEIN M 213 975 053 TURIAF J J FRANC MED CHIR THORAC 12 290 958 054 GIBBONS RA BIOCHEM J 73 209 959 055 GIBBONS RA BIOCHEM J 73 209 959 056 PUCHELLE E BIORHEOLOGY 12 219 975 057 MEYER FA BIORHEOLOGY 13 49 976 058 KEAL EE THORAX 27 643 972 CT 1 LOPEZVIDRIERO MT THORAX 34 512 979 2 WANNER A AM J MED 67 477 979 3 LOPEZVIDRIERO MT SCAND J RESPIR DIS 1979 33 979 4 SHIMURA S BIORHEOLOGY 17 363 980 5 BATTISTA SP ARCH ENVIRON HEALTH 35 239 980 6 REID LM ENVIRON HEALTH PERSPECT 35 113 980 7 KING M FED PROC 39 3080 980 8 KING M EUR J RESPIR DIS 61 39 980 9 LOPEZVIDRIERO MT EUR J RESPIR DIS 61 21 980 10 LOPEZVIDRIERO MT J COMP PATHOL 90 415 980 11 KING M PEDIATR RES 15 120 981 12 PUCHELLE E BIORHEOLOGY 18 659 981 13 BAUER E RES COMMUN CHEM PATH PHARM 34 547 981 14 MIRRAKHIMOV MM TER ARKH 53 110 981 15 SNYDER CE CARBOHYD RES 105 87 982 16 MORRIS JB ANAL BIOCHEM 121 129 982 17 REID L ADV EXP MED BIOL 144 369 982 18 REID L EXP LUNG RES 4 157 983 19 HARBITZ O EUR J RESPIR DIS 65 512 984 20 MORETTI M EUR J RESPIR DIS 65 365 984 21 STELANDER M EUR J RESPIR DIS 65 5 984 22 GREENSTONE M BR J DIS CHEST 79 9 985 23 BROWN DT ANN OTOL RHINOL LARYNGOL 94 3 985 24 BROFELDT S AM REV RESPIR DIS 133 1138 986 25 MORETTI M THORAX 42 229 987 26 ROGERS DF CLIN SCI 72 629 987 PN 78025 RN 00806 AN 79101945 AU Stern-R-C. Boat-T-F. Orenstein-D-M. Wood-R-E. Matthews-L-W. Doershuk-C-F. TI Treatment and prognosis of lobar and segmental atelectasis in cystic fibrosis. SO Am-Rev-Respir-Dis. 1978 Nov. 118(5). P 821-6. MJ ATELECTASIS: et. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. ATELECTASIS: di, th. BRONCHOSCOPY. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. PROGNOSIS. SUPPORT-U-S-GOVT-P-H-S. AB Lobar atelectasis occurred in 30 (4.1 per cent) and segmental atelectasis occurred in 6 (0.8 per cent) of 728 patients with cystic fibrosis. The right lung was involved in all 11 patients less than 5 years of age and in 18 of 26 episodes in 19 older patients. Five of the younger patients died within 5 years of the initial episode. Bronchoscopy (with or without local lavage) performed in addition to intensified medical therapy did not appear to improve the outcome. Future studies of therapeutic measures (including bronchoscopy) for treatment of atelectasis complicating cystic fibrosis must include appropriate control patients who receive only medical therapy. RF 001 DI SANTAGNESE PA PEDIATRICS 12 178 953 002 LLOYD-STILL JD PEDIATRICS 53 678 974 003 WOOD RE AM REV RESPIR DIS 113 833 976 004 WARING WW ADV PEDIATR 23 401 976 005 SHWACHMAN H PEDIATRICS 46 335 970 006 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 007 JAY SJ N ENGL J MED 293 798 975 008 FOX HM AM REV RESPIR DIS 108 469 973 009 ABDULMAJID OA THORAX 31 635 976 010 WELLONS HA AM J DIS CHILD 130 301 976 011 DEES SC JAMA 197 8 966 012 LIU PV HOSP PRACT 11 139 976 013 MITCHELL-HEGGS PF Q J MED 45 479 976 014 BEDROSSIAN CWM HUM PATHOL 7 195 976 015 HOLSCLAW DS CLIN PEDIATR 9 346 970 CT 1 REDDING GJ PEDIATR CLIN NORTH AM 31 891 984 2 HODSON ME POSTGRAD MED J 60 225 984 3 LESTER LA SEM RESPIR MED 6 285 985 4 REDDING GJ PEDIATR RES 19 552 985 5 NUSSBAUM E CLIN PEDIATR 24 379 985 6 ABMAN SH J PEDIATR 107 933 985 7 SHERMAN JM PEDIATR PULMONOL 2 244 986 8 TOMASHEFSKI JF AM REV RESPIR DIS 133 535 986 PN 78026 RN 00807 AN 79081157 AU Brackenridge-C-J. TI The seasonal variation of births of offspring from couples heterozygous for cystic fibrosis. SO Ann-Hum-Genet. 1978 Oct. 42(2). P 197-201. MJ CYSTIC-FIBROSIS: fg. SEASONS. MN AUSTRALIA. BIRTH-RATE. FEMALE. FERTILITY. GENES. HETEROZYGOTE. HUMAN. INFANT-NEWBORN. MALE. STATISTICS. TEMPERATURE. AB To test whether the month of birth of patients with cystic fibrosis (CF) is seasonally distributed as reported in a Dutch survey, published Australian data were examined and no difference in the monthly variation was found between births of CF patients and their unaffected sibs. When the two groups were combined and analysed by periodic regression using successively higher Fourier terms, a significant fit to the observed frequencies was obtained with a bimodal curve. Possession of a single CF gene seems therefore sufficient for expression of the cyclic variation. In substantial agreement with the Dutch series, maximum frequencies were found to occur in March or April and in September. It is suggested that one or both parents may be subject to variable fertility mediated by a temperature-dependent mechanism. The relevance of a periodic fertility pattern in preserving the CF gene in the population by selective heterozygote advantage is discussed. RF 001 BLISS CI STATISTICS IN BIOLOGY 2 219 970 002 DANKS DM ANN HUM GENET 28 323 965 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 EDWARDS JH ANN HUM GENET 25 89 961 005 FROGGATT P BR J PREV SOC MED 25 119 971 006 GIBSON LE PEDIATRICS 23 545 959 007 GUMBEL EJ J AM STATIST ASSOC 49 267 954 008 KESSLER WR PEDIATRICS 8 648 951 009 LOWE CU AM J DIS CHILD 78 349 949 010 MIETTINEN OS BR HEART J 32 103 970 011 PIKE MC BR MED J 2 395 967 012 SHWACHMAN H N ENGL J MED 255 999 956 013 TEN KATE LP INT J EPIDEMIOL 6 23 977 CT 1 BRACKENRIDGE CJ AM J MED GENET 5 303 980 2 BRACKENRIDGE CJ AM J MED GENET 5 295 980 3 ROMEO G RIV ITAL PEDIATR 7 201 981 4 DAVID TJ J MED GENET 18 299 981 5 PASSARGE E EUR J PEDIATR 143 54 984 6 MITCHELL RJ HUM BIOL 57 213 985 PN 78027 RN 00808 AN 78078968 AU Andrews-C. Mango-M. Venuto-R-C. TI Cystic fibrosis in adults [letter]. SO Ann-Intern-Med. 1978 Jan. 88(1). P 128-9. MJ CYSTIC-FIBROSIS: di. MN ADULT. CASE-REPORT. CYSTIC-FIBROSIS: co. HEAT-EXHAUSTION: et. HUMAN. HYPONATREMIA: et. MALE. EX An adult male was recently discovered to have cystic fibrosis after a unique clinical presentation not mentioned in the review article. This patient with recurrent episodes of heat exhaustion and hyponatremia had no extraordinary source of Na+ loss other than his sweat. This case represents an important new presentation of cystic fibrosis. Most internists view this disease as one seen only in chronically ill children. RF 001 STERN RC ANN INTERN MED 87 188 977 002 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 003 WOOD RE AM REV RESPIR DIS 113 833 976 PN 78028 RN 00809 AN 79061093 AU Schaad-U. Kraemer-R. Gaze-H. Hadorn-B. TI One-hour blood-xylose in cystic fibrosis. SO Arch-Dis-Child. 1978 Sep. 53(9). P 756-7. MJ CYSTIC-FIBROSIS: bl. XYLOSE: bl. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. TIME-FACTORS. AB One-hour blood-xylose concentrations after an oral xylose load were measured in children with cystic fibrosis (CF) and healthy controls. The mean of the 1-hour blood-xylose values was significantly increased in the group with CF. The finding confirms an earlier observation by Rolles et al. (1973). Its significance is not at present understood but it suggests that small intestinal function should be further investigated in CF. RF 001 COLOMBO JP IN: RICHTERICH R 462 978 002 GIBBONS ISE ARCH DIS CHILD 44 63 969 003 ROE JH J BIOL CHEM 173 507 948 004 ROLLES CJ LANCET 2 1043 973 005 SCHAAD U ARCH DIS CHILD 53 420 978 006 SCHAAD U HELV PAEDIATR ACTA 30 331 975 CT 1 FRASE LL GASTROENTEROLOGY 88 478 985 2 PENNY DJ ARCH DIS CHILD 61 1127 986 3 LECLERCQFOUCART J J PEDIATR GASTROENTEROL NUTR 6 66 987 PN 78029 RN 00810 AN 79061100 AU Ahuja-A-S. Mann-N-M. TI Cimetidine in cystic fibrosis [letter]. SO Arch-Dis-Child. 1978 Sep. 53(9). P 766. MJ CIMETIDINE: tu. CYSTIC-FIBROSIS: dt. GUANIDINES: tu. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FATS: an. FECES: an. FEMALE. HUMAN. MALE. EX A pilot study has examined the effect of cimetidine on steatorrhea in children with pancreatic insufficiency due to CF who were receiving pancreatic supplements. Administration of cimetidine with pancreatic supplements reduced the faecal fat excretion in 4 out of 5 children, in 3 cases by more than 50%. In Case 4 steatorrhea increased on cimetidine but without any symptoms and we have no explanation for it. None of the children had any side effects from the drug. Further studies are needed to determine the indications of cimetidine in the dietary management of CF. RF 001 HEIZER WD BULL JOHNS HOPKINS HOSP 116 261 965 002 KATTWINKEL J PEDIATRICS 50 133 972 003 REGAN PT GASTROENTEROLOGY 72 A95 977 CT 1 SCHONI M HELV PAEDIATR ACTA 36 359 981 2 BELL L J CAN DIET ASSOC 42 62 981 PN 78030 RN 00811 AN 78164870 AU Smalley-C-A. Addy-D-P. Anderson-C-M. TI Limitations of the sweat test [letter]. SO Arch-Dis-Child. 1978 Apr. 53(4). P 351-2. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHILD. CHLORIDES: an. HUMAN. PANCREAS: pp. SODIUM: an. EX We are in agreement with Schwarz et al. when they state that the sweat test has some limitations in the diagnosis of cystic fibrosis (CF) and that the diagnosis is not justified on the basis of an abnormal sweat test unless there is other supporting evidence of the disease. In our experience a much greater problem of sweat test accuracy occurs in hospitals where the test is carried out on a very much smaller number of patients and where the laboratory has a much lesser degree of experience than that of the authors. We have had a significant number of patients referred to our regional children's hospital who have had abnormal sweat electrolyte measurements elsewhere, but unequivocally normal measurements when repeated by a laboratory carrying out a large number of tests. We have not needed to go to the extent of serial collections of sweat and of giving spironolactone as have the authors. If we obtain a test within the 'grey area' on repeated occasions, we carefully seek other evidence of the disease, particularly evidence of pancreatic disease. - We do not advocate the general use of spironolactone: the merit of this drug in our experiments on certain patients was merely to support our suspicion of temporary hyperaldosteronism, a condition which might have led to erroneous conclusions in the absence of K or Cl determinations. RF 001 SCHWARZ V ARCH DIS CHILD 52 870 977 002 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 003 HADORN B CAN MED ASSOC J 98 377 968 PN 78031 RN 00812 AN 79040865 AU Turner-M-W. Warner-J-O. Stokes-C-R. TI Immunological studies in cystic fibrosis. SO Arch-Dis-Child. 1978 Aug. 53(8). P 631-8. MJ CYSTIC-FIBROSIS: im. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. HUMAN. HLA-ANTIGENS: an. IGE: an. IMMUNOGLOBULINS: an. INFANT. RESPIRATORY-HYPERSENSITIVITY: co, im. AB Evidence is presented to support the concept that much of the allergy in cystic fibrosis (CF) is IgE mediated. Total IgE levels were higher in allergic than in nonallergic CF patients. Levels were also higher in those patients who had had the greatest number of chest infections in the preceding 12 months. IgE antibody levels to Dermatophagoides pteronyssinus, Timothy grass pollen, and Aspergillus fumigatus were higher in those with positive results from skin tests to these allergens. The serum IgG, IgM, and IgA levels of allergic and nonallergic CF patients did not differ but the overall mean values for IgG and iGM were higher than those reported for healthy British children. The highest levels tended to be present in patients with the greatest number of recent major chest infections and the difference was significant for IgG. 16 patients had IgA levels >2 SD below the reported means for age-matched controls and 11 of these were nonallergic. IgA levels were also higher in patients who had recently experienced major chest infections. 45 of the patients were tissue types for HLA A and B antigens but no significant clinical associations with single antigens were observed. The antigen phenotype A1 + B8 was more common in patients with multiple allergic symptoms than in those with a single allergy or merely a positive result from a skin test. Nonsignificant increases of W19 in patients with frequent infections and of A2 in patients presenting with meconium ileus were also noted. The data presented do not permit a choice to be made between the alternative concepts of allergy as a primary abnormality in CF, and allergy arising secondary to infection. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 COOMBS RRA IN: GELL PGH 761 975 003 FAHEY JL J IMMUNOL 94 84 965 004 GEBHARD RL LANCET 2 760 973 005 HOBBS JR ASSOC CLIN PATHOLOGISTS BROAD 1 970 006 KAISER GI HLA AND DISEASE 252 976 007 ANON LANCET 2 1291 975 008 MACKAY IR LANCET 2 793 972 009 MCNEISH AS LANCET 1 668 973 010 MEARNS MB LANCET 1 538 967 011 MICKEY MR TISSUE ANTIGENS 1 57 971 012 MITTAL KK TRANSPLANTATION 6 913 968 013 NELSON SD TISSUE ANTIGENS 4 361 974 014 NYE L CLIN ALLERGY 5 13 975 015 PARISH WE LANCET 2 591 970 016 PIRSKANEN R ANN CLIN RES 4 304 972 017 POLYMENIDIS Z LANCET 2 1452 973 018 SHAKIB F CLIN ALLERGY 6 237 976 019 SOOTHILL JF CLIN ALLERGY 6 305 976 020 STANWORTH DR CLIN ALLERGY 3 37 973 021 SVEJGAARD A TRANSPLANT REV 22 3 975 022 TAYLOR B LANCET 2 111 973 023 TURNER MW CLIN EXP IMMUNOL 27 43 977 024 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 025 WARNER JO LANCET 1 990 976 026 WARNER JO ARCH DIS CHILD 51 507 976 027 WARREN CPW CLIN ALLERGY 5 1 975 028 WEEKE B DAN MED BULL 23 155 976 029 WIDE L LANCET 2 1105 967 CT 1 VANASPEREN PP AUST PAEDIATR J 16 53 980 2 ORMEROD LP THORAX 35 768 980 3 BRUETON MJ ARCH DIS CHILD 55 348 980 4 HARPER TB LUNG 157 219 980 5 DINWIDDIE R PRACTITIONER 224 291 980 6 MATTHEWS WJ N ENGL J MED 302 245 980 7 REEN DJ CLIN ALLERGY 11 571 981 8 PITCHERWILMOTT RW ARCH DIS CHILD 57 577 982 9 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 10 GUIDOTTI TL AM J MED SCI 283 157 982 11 ANON LANCET 2 257 983 12 AUERBACH HS LANCET 2 686 985 13 MOSS RB MONOGR ALLERGY 19 202 986 14 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 15 CANNY GJ THORAX 41 221 986 16 FICK RB AM REV RESPIR DIS 133 418 986 17 DASGUPTA MK J CLIN IMMUNOL 7 51 987 18 MOSS RB CHEST 91 522 987 PN 78032 RN 00813 AN 79081404 AU Yassa-J-G. Prosser-R. Dodge-J-A. TI Effects of an artificial diet on growth of patients with cystic fibrosis. SO Arch-Dis-Child. 1978 Oct. 53(10). P 777-83. MJ CYSTIC-FIBROSIS: dh. FOOD-FORMULATED. MN ADOLESCENCE. AGE-DETERMINATION-BY-SKELETON. BODY-HEIGHT. BODY-WEIGHT. CEPHALOMETRY. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. FEMALE. HUMAN. MALE. SKINFOLD-THICKNESS. AB The effects of an artificial diet on growth were examined in a controlled trial on 43 patients with cystic fibrosis. 28 patients received the diet for 12 months. Comparison was made between their growth before, during, and after dietary treatment. Growth changes were also assessed in 15 other patients who received a conventional diet. Artificial dietary treatment led to some improvement in height, weight, subscapular skinfold thickness, and a disproportionate advance of bone age for the group as a whole. Only 10 (36%) patients had a 'clinically' significant improvement in height, weight, or both- -i.e. more than 0.5 standard deviation score. Greatest improvements were in young and mildly affected patients. It is proposed that the future use of such an unpleasant and expensive diet should be restricted to a few selected cases, rather than given as routine treatment. RF 001 ALLAN JD AM J DIS CHILD 126 22 973 002 BERRY HK AM J DIS CHILD 129 165 975 003 CLARKE CW Q J MED 17 358 924 004 DOERSHUK CF J PEDIATR 65 677 964 005 HARPER MH MED J AUST 2 663 930 006 LOBER CW AM J DIS CHILD 129 1239 975 007 PASSINI F DTSCH MED WSCHR 45 851 919 008 ROBINSON MJ ARCH DIS CHILD 50 962 975 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 SPROUL A J PEDIATR 65 664 964 011 TANNER JM IN: FORFAR JO 270 973 012 TANNER JM ARCH DIS CHILD 50 142 975 013 TANNER JM ASSESSMENT OF SKELETAL MATURI 975 014 TANNER JM ARCH DIS CHILD 41 454 966 CT 1 CHASE HP J PEDIATR 95 337 979 2 KASPER H LEBER MAGEN DARM 10 269 980 3 KORETZ RL GASTROENTEROLOGY 78 393 980 4 SHEPHERD R J PEDIATR 97 351 980 5 MILLER M AM J CLIN NUTR 36 492 982 6 PENCHARZ PB J PEDIATR GASTROENTEROL NUTR 2 400 983 7 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 8 BERTRAND JM J PEDIATR 104 41 984 9 MANSELL AL J PEDIATR 104 700 984 10 CANCIANI M J PEDIATR GASTROENTEROL NUTR 4 735 985 11 HEINE W Z KLIN MED 40 181 985 12 HOLT TL AM J CLIN NUTR 41 1061 985 13 RUSSELL RI PROC NUTR SOC 44 87 985 14 MEARNS MB ARCH DIS CHILD 60 272 985 15 CHALMERS DM ACTA PAEDIATR SCAND 74 114 985 16 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 17 NEIJENS HJ ACTA PAEDIATR SCAND SUPPL 317 1985 38 985 18 LESTER LA J PARENT ENTERAL NUTR 10 289 986 19 SOUTTER VL CLIN GASTROENTEROL 15 137 986 20 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 21 DUHAMEL JF ARCH FR PEDIATR 43 229 986 22 OLOUGHLIN E AM J CLIN NUTR 43 732 986 23 MOORE MC AM J CLIN NUTR 44 33 986 24 GOODCHILD MC J ROY SOC MED 79 32 986 PN 78033 RN 00814 AN 78122876 AU Godfrey-S. Mearns-M. Howlett-G. TI Serial lung function studies in cystic fibrosis in the first 5 years of life. SO Arch-Dis-Child. 1978 Jan. 53(1). P 83-5. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. RESPIRATORY-FUNCTION-TESTS. AB Lung mechanics were studied in 8 infants with cystic fibrosis at 6 months of life and radiosotopic lung function was measured in 5 of them at 5 years of age. The children who were initially asymptomatic had normal lung mechanics in infancy but the 2 restudied later had abnormal radioisotopic lung function. The symptomatic children showed abnormalities in infancy and more marked changes later. It is concluded that the lungs in cystic fibrosis are probably normal initially and that damage occurs later even in the absence of symptoms. RF 001 DUBOIS AB J CLIN INVEST 35 322 956 002 HOWLETT G ARCH DIS CHILD 47 707 972 003 LAMARRE A PEDIATRICS 50 291 972 004 PHELAN PD ARCH DIS CHILD 44 393 969 005 RONCHETTI R ARCH DIS CHILD 50 595 975 CT 1 KRAEMER R HELV PAEDIATR ACTA 34 417 979 2 PEDERZINI F RIV ITAL PEDIATR 9 445 983 3 GODFREY S PEDIATRICS 72 517 983 4 TEPPER RS AM REV RESPIR DIS 135 1075 987 PN 78034 RN 00815 AN 79040866 AU Sacher-M. Kobsa-A. Shmerling-D-H. TI PABA screening test for exocrine pancreatic function in infants and children. SO Arch-Dis-Child. 1978 Aug. 53(8). P 639-41. MJ P-AMINOBENZOIC-ACID: du. AMINOBENZOIC-ACIDS: du. INFANT-NEWBORN. PANCREAS: ph. PANCREATIC-DISEASES: di. MN P-AMINOBENZOIC-ACID: ur. ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CHYMOTRYPSIN: me. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. INFANT. MALE. PANCREAS: pp. PANCREATIC-DISEASES: et, ur. AB P-Amino-benzoic acid (PABA) is split specifically by pancreatic chymotrypsin from the synthetic tripeptide N-benzoyl-L-tyrosyl-PABA. The urinary excretion of absorbed PABA serves as an index for exocrine pancreatic function. The peptide (0.015 g/kg) was administered orally to 20 controls (aged between 5 months and 16 years), 6 patients with exocrine pancreatic insufficiency caused by cystic fibrosis (CF), and 9 newborn infants. In the controls the mean 6-hour PABA recovery was 58.5% (+/- 11.2 SD). Recovery in patients with CF was lower (P less than 0.001) with no overlap. In newborn infants the mean 6-hour PABA recovery was 23.4 (+/- 17.7 SD); overlapping in 3 instances with the results in CF patients. This simple, noninvasive test thus appears promising and merits further investigation in younger infants, especially newborns. RF 001 ARVANITAKIS C LANCET 1 663 976 002 GYR K GUT 17 27 976 003 GYR K GASTROENTEROLOGY 68 488 975 004 HADORN B J PEDIATR 73 39 968 005 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 006 IMONDI AR GUT 13 726 972 007 SMITH HW J CLIN INVEST 24 388 945 CT 1 IMONDI AR BIOCHEM MED 22 198 979 2 SACHER M J PEDIATR 94 159 979 3 WALLER SL AUST NZ J MED 10 351 980 4 LANKISCH PG DTSCH MED WSCHR 105 1418 980 5 LANG C SCHWEIZ MED WOCHENSCHR 110 522 980 6 DOCKTER G MONATSSCHR KINDERHEILKD 128 732 980 7 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 8 OSSWALD P MONATSSCHR KINDERHEILKD 128 340 980 9 HOEK FJ GUT 22 8 981 10 LANKISCH PG HEPATOGASTROENTEROLOGY 28 333 981 11 LANG C BR J SURG 68 771 981 12 PARK RW GASTROENTEROLOGY 81 1143 981 13 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 14 LIBEER JC CLIN CHIM ACTA 115 119 981 15 DOCKTER G EUR J PEDIATR 135 277 981 16 KAI H J PEDIATR GASTROENTEROL NUTR 1 445 982 17 LANKISCH PG GUT 23 777 982 18 LENTZE MJ J PEDIATR GASTROENTEROL NUTR 2 S252 983 19 RYAN ME CLIN GASTROENTEROL 12 533 983 20 GOLDBERG DM CLIN PHYSIOL BIOCHEM 2 249 984 21 SATO M J PEDIATR GASTROENTEROL NUTR 3 415 984 22 TOSKES PP PHARMACOTHERAPY 4 74 984 23 LANKISCH PG CLIN GASTROENTEROL 13 717 984 24 SCHONI M PEDIATR RES 18 66 984 25 HUBBARD VS DIG DIS SCI 29 881 984 26 HODSON ME POSTGRAD MED J 60 225 984 27 GILLARD BK AM J DIS CHILD 138 577 984 28 BELLENTANI S EUR J PEDIATR 143 145 984 29 SCOTTA MS CLIN BIOCHEM 18 233 985 30 MEE AS GUT 26 1257 985 31 RIEU D ANN PEDIATR (PARIS) 32 205 985 32 NIEDERAU C GASTROENTEROLOGY 88 1973 985 33 HEYMAN MB GASTROENTEROLOGY 89 685 985 34 WEIZMAN Z GASTROENTEROLOGY 89 596 985 35 GHARBO SA ANAL BIOCHEM 148 228 985 36 SCHARPE S CLIN CHEM 33 5 987 37 SARLES J ARCH FR PEDIATR 44 311 987 PN 78035 RN 00816 AN 78255586 AU Smalley-C-A. Brown-G-A. Parkes-M-E. Tease-H. Brookes-V. Anderson-C-M. TI Reduction of bile acid loss in cystic fibrosis by dietary means. SO Arch-Dis-Child. 1978 Jun. 53(6). P 477-82. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: dh. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, me. FECES: an. HUMAN. PANCREATIN: tu. CELIAC-DISEASE: dh, et. TRIGLYCERIDES: tu. AB On a 'normal' diet increased faecal bile acid excretion was found in 14 of 16 children with cystic fibrosis who had steatorrhoea, but excretion was normal in 2 such children without steatorrhoea. The 16 children with steatorrhoea took 3 regimens of diet and therapy: a 'normal' diet with pancreatic enzyme supplements, a diet of reduced long-chain triglycerides with added medium-chain triglycerides, and the same diet with added pancreatic enzyme supplements. On each of these three regimens steatorrhoea and faecal bile acid loss were significantly less than on no treatment, with the lowest excretions occurring on the diet of reduced long-chain triglycerides with added medium-chain triglycerides and pancreatic enzyme supplements. Although a reduction in steatorrhoea was nearly always accompanied by a decrease in bile acid excretion, the initial bile acid loss was very variable and could not be predicted for any given degree of steatorrhoea. This suggests that at least one other factor, possibly liver disease or bile acid pool size, influences bile acid loss in the faeces. RF 001 ANDERSON CM MOD PROBL PEDIATR 10 326 967 002 ANDERSON CM LANCET 1 836 952 003 ANSANELLI V FRACASTORO (VERONA) 63 637 970 004 BAGHERI SA GASTROENTEROLOGY 66 878 974 005 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 006 DI SANTAGNESE PA PEDIATRICS 18 387 956 007 FOSBROOKE AS CLIN CHIM ACTA 20 517 968 008 GOODCHILD MC ARCH DIS CHILD 50 769 975 009 GRACEY M ARCH DIS CHILD 45 445 970 010 HADORN B CAN MED ASSOC J 98 377 968 011 HOFMANN AF IN: SENIOR JR 9 968 012 IWATA T J BIOCHEM (TOKYO) 56 424 964 013 LEYLAND FC ARCH DIS CHILD 44 170 969 014 MURPHY GM IN: ANDERSON CM 667 975 015 REDINGER RN GASTROENTEROLOGY 64 610 973 016 ROLLER RJ GASTROENTEROLOGY 72 661 977 017 SHERR HP AM J CLIN NUTR 27 1369 974 018 SHWACHMAN H PEDIATRICS 36 689 965 019 TYSON KRT J PEDIATR SURG 3 271 968 020 VAN DE KAMER JH J BIOL CHEM 177 347 949 021 WATKINS JB GASTROENTEROLOGY 67 835 974 022 WEBER AM CLIN CHIM ACTA 39 524 972 023 WEBER AM N ENGL J MED 289 1001 973 024 WEBER AM GASTROENTEROLOGY 68 1066 975 025 WEBER AM GUT 17 295 976 026 WEBSTER R ARCH DIS CHILD 28 343 953 CT 1 STARKEY BJ MONOGR PAEDIATR 10 12 979 2 JONAS A J PEDIATR 95 366 979 3 BALISTRERI WF J PEDIATR 96 582 980 4 COLOMBO C RIV ITAL PEDIATR 8 77 982 5 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 6 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 7 COLOMBO C DIG DIS SCI 28 306 983 8 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 9 SETCHELL KDR CLIN CHIM ACTA 151 101 985 10 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 11 LEROY C DIG DIS SCI 31 911 986 PN 78036 RN 00817 AN 78255587 AU Bray-P-T. Clark-G-C. Moody-G-J. Thomas-G. Thomas-J-D. TI Sweat testing for cystic fibrosis. Diagnostic screening with a combination chloride ion-selective electrode. SO Arch-Dis-Child. 1978 Jun. 53(6). P 483-6. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. INFANT-NEWBORN-DISEASES: di. SWEAT: an. MN AGE-FACTORS. ELECTRODES. FEMALE. HUMAN. INFANT-NEWBORN. MALE. METHODS. AB Screening of sweat chloride in newborn infants with the Orion Skin Chloride Measuring System and incorporating some procedural innovations is described. The results indicate that while diagnostic screening for cystic fibrosis can be readily undertaken, the test with the chloride ion-selective electrode is best left at least to the second day of life or later because of insufficient sweating in very young babies. Measurements on 2 babies with cystic fibrosis are also discussed. RF 001 ARMSTRONG D ORION RES INC NEWSLETTER 3 28 971 002 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 003 BRAY PT CLIN CHIM ACTA 77 69 977 004 BRAY PT CLIN CHIM ACTA 80 333 977 005 CHERIAN AG CLIN CHEM 17 652 971 006 GIBSON LE GAP CONF REP PROB SWEAT TESTI 975 007 GIBSON LE PEDIATRICS 23 545 959 008 GURSON CT HELV PAEDIATR ACTA 28 165 973 009 KOPITO L PEDIATRICS 43 794 969 CT 1 BRAY PT ARCH DIS CHILD 53 918 978 2 LITTLEWOOD JM ARCH DIS CHILD 61 1041 986 3 BECK R ACTA PAEDIATR SCAND 75 639 986 PN 78037 RN 00818 AN 79081443 AU Griffiths-A-D. Bull-F-E. TI Sweat testing for cystic fibrosis [letter]. SO Arch-Dis-Child. 1978 Nov. 53(11). P 918. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. SWEAT: an. MN CYSTIC-FIBROSIS: oc. FALSE-NEGATIVE-REACTIONS. HUMAN. INFANT-NEWBORN. MASS-SCREENING. EX Bray et al. conclude that the mass screening of newborn babies with chloride ion-selective electrodes is a feasible method for the diagnosis of cystic fibrosis; they also suggest that this procedure might be used in conjunction with meconium analysis to reduce the incidence of false positive results. In 1972 we reported the details of a child with proved cystic fibrosis in whom raised sweat sodium but normal sweat chloride levels were obtained during treatment with cloxacillin. Attention was drawn to the implication of the finding with regard to screening procedures, and the suggestion was made that, in at least some children with cystic fibrosis, the cloxacillin radical might replace the chloride ion in the sweat. When mass screening for cystic fibrosis is to be undertaken using methods based on the estimation of sweat chloride levels, it is therefore prudent to inquire whether the infant is receiving medication from any source and, if so, to interpret the results with caution. - These are instances of false-negative sweat chloride readings, but there is no evidence yet from our 1205 tests of any negative cystic fibrosis sweat chloride; the statistical chance of this is in any case small. These several cases suggest a need for a systematic investigation of the effect of medication on sweat ion levels in general as well as in cystic fibrosis cases. RF 001 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 002 BRAY PT ARCH DIS CHILD 53 483 978 003 DI SANTAGNESE PA GAP CONF REP PROB SWEAT TESTI 9 975 004 GRIFFITHS AD ARCH DIS CHILD 47 132 972 PN 78038 RN 00819 AN 79081430 AU Silverman-M. Hobbs-F-D. Gordon-I-R. Carswell-F. TI Cystic fibrosis, atopy, and airways lability. SO Arch-Dis-Child. 1978 Nov. 53(11). P 873-7. MJ AIRWAY-OBSTRUCTION: co. CYSTIC-FIBROSIS: co. HYPERSENSITIVITY-IMMEDIATE: co. MN ADULT. ALLERGENS. ASPERGILLUS-FUMIGATUS: im. CHILD. CYSTIC-FIBROSIS: pp. EXERTION. FEMALE. HUMAN. MALE. PEAK-EXPIRATORY-FLOW-RATE. PROSPECTIVE-STUDIES. AB In a survey of cystic fibrosis (CF) in the Avon area, 48 children with CF from 40 families together with 71 of their parents were studied by spirometry, exercise tests, and pinch tests. A control group of 42 young adults was similarly tested; control data for children were taken from previously published work. The prevalence of atopy (any positive prick test) in children with CF was 48%. Sensitivity to grass pollens and house dust mite was no more common in these children (29%) than in a normal population (34%). Hypersensitivity to Aspergillus fumigatus was found in 35% of children with CF and was associated with severe lung disease. The parents had a normal pattern and prevalence of atopy. Exercise- induced airways obstruction was present in only 22% of children with CF; its association with severe lung disease rendered interpretation difficult. The parents had a normal response to exercise. Both hypersensitivity to A. fumigatus and exercise-induced airways lability had the features of acquired characteristics. There was nothing in the present study to support the hypothesis that the possession of a CF gene predisposed to atopy. RF 001 ABBOTT V CAN MED ASSOC J 64 419 951 002 ALLAN JD CLIN ALLERGY 5 255 975 003 ANDERSON SD THESIS 972 004 ANDERSON SD THORAX 26 396 971 005 ASTRAND PO TEXTBOOK OF WORK PHYSIOLOGY 970 006 CHRISPIN AR PEDIATR RADIOL 2 101 974 007 COGSWELL JJ BR J DIS CHEST 69 177 975 008 COTES JE LUNG FUNCTION 975 009 COUNAHAN R ARCH DIS CHILD 50 477 975 010 DAY G ARCH DIS CHILD 48 355 973 011 FEATHERBY EA CAN MED ASSOC J 102 835 970 012 GODFREY RC CLIN ALLERGY 6 79 976 013 GODFREY S BR J DIS CHEST 64 15 970 014 JONES RS ARCH DIS CHILD 50 909 975 015 KULCZYCKI LL JAMA 175 358 961 016 LANDAU LI J PEDIATR 82 863 973 017 MCFARLANE H LANCET 1 1241 976 018 PEPYS J BR J HOSP MED 14 412 975 019 RACHELEFSKY GS AM J DIS CHILD 128 355 974 020 SILVERMAN M THESIS 973 021 SILVERMAN M ARCH DIS CHILD 47 882 972 022 SWINSCOW TDV STATISTICS AT SQUARE ONE 977 023 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 024 WARNER JO ARCH DIS CHILD 51 507 976 025 WARREN CPW CLIN ALLERGY 5 1 975 026 ZAPLETAL A PEDIATRICS 48 64 971 CT 1 PRICE JF CLIN ALLERGY 9 563 979 2 VANASPEREN PP AUST PAEDIATR J 16 53 980 3 ORMEROD LP THORAX 35 768 980 4 BRUETON MJ ARCH DIS CHILD 55 348 980 5 MOSS RB AM REV RESPIR DIS 121 23 980 6 VANASPEREN P AM J DIS CHILD 135 815 981 7 HENRY RL AUST PAEDIATR J 18 110 982 8 PITCHERWILMOTT RW ARCH DIS CHILD 57 582 982 9 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 10 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 11 WILSON N J INT BIOMED INFOR DATA 5 37 984 12 WONNE R CLIN ALLERGY 15 455 985 13 LAUFER P CUTIS 36 245 985 14 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 15 WILMOTT RW AM J DIS CHILD 139 669 985 16 DARGA LL PEDIATR PULMONOL 2 82 986 17 LAROCHE D J PEDIATR GASTROENTEROL NUTR 5 404 986 PN 78039 RN 00820 AN 79164993 AU Rudnik-J. Kurzawa-R. Gawel-J. Majewska-Zalewska-H. Zebrak-J. TI Activity of serum inhibitory proteases in bronchial secretions from children with various chronic non-specific respiratory diseases. SO Arch-Immunol-Ther-Exp (Warsz). 1978. 26(1-6). P 717-22. MJ ALPHA-1-ANTITRYPSIN: me. BRONCHI: en. BRONCHIAL-DISEASES: en. PROTEASE-INHIBITORS: me. MN BRONCHI: se. BRONCHIECTASIS: en. BRONCHITIS: en. CYSTIC-FIBROSIS: en. HUMAN. RESPIRATORY-TRACT-INFECTIONS: en, pa. AB In a group of 223 children with various chronic, non-specific respiratory diseases, the levels of serum inhibitory proteolytic enzymes in bronchial secretions was measured. Activity of inhibitory proteolytic enzymes was analyzed according to the nature of bronchial lesions observed at bronchoscopy and character and cellular composition of bronchial secretion. Statistically significant increase of the mean inhibitory value in comparison to values in the control group, was found in children with recurrent bronchitis (a-1- AT), cystic fibrosis (a-1-AT and a-1-X), chronic bronchitis (a-1-X), bronchiectases (a-1-X) and in children with obstructive bronchitis (a- 2-M). RF 001 BERGMANN K Z ERKR ATMUNGSORGANE 136 3 973 002 BROGAN TD THORAX 26 418 971 003 BROGAN TD THORAX 30 72 975 004 COHEN AB J CLIN INVEST 52 2793 973 005 ERIKSSON S ACTA MED SCAND 175 197 964 006 FEIGELSON J ANN PEDIATR (PARIS) 18 704 971 008 HOCHSTRASSER K BAYER SYMPOSIUM V PROTEINASE 111 974 009 HOCHSTRASSER K RESPIRATION 31 343 974 010 HOCHSTRASSER K HOPPE SEYLERS Z PHYSIOL CHEM 353 221 972 011 GUNTHER W HUSTEN AND AUSWURF 972 012 HUTCHISON DCS BR J DIS CHEST 67 171 973 013 KIKINDJANIN V ALLERGIE IMMUNOL 22 17 976 014 KLEIBAUER JP REV FR MAL RESP 1 923 973 015 KOSMIDER ST PNEUM POL 44 937 976 016 KURZAWA R GRUZLICA 743 189 975 017 LAINE A CLIN CHIM ACTA 67 159 976 018 LINDH E ACTA UNIV UPSALM 232 975 019 LOWRY OH J BIOL CHEM 193 265 951 020 MANCINI G IMMUNOCHEMISTRY 2 235 965 021 MICHEL FB NOUV PRESSE MED 3 2245 974 022 MITTMAN C AM REV RESPIR DIS 105 430 972 023 MOSHER DF J CLIN INVEST 60 1036 977 024 NEIMANN N PEDIATRIE 49 351 974 025 RASCHE B KLIN WOCHENSCHR 55 795 977 026 RASCHE B PNEUMONOLOGIE 149 157 973 027 RASCHE B PNEUMONOLOGIE 144 10 971 028 RYLEY HC J CLIN PATHOL 26 852 973 029 RUDNIK J J PEDIATR POL 51 1279 976 030 STEINBUCH M BAYER SYMPOSIUM V PROTEINASE 78 974 031 TALAMO RC ANN NY ACAD SCI 221 234 974 032 TOMASI TB JR J IMMUNOL 112 2274 974 PN 78040 RN 00821 AN 79061368 AU Ranga-V. Kleinerman-J. TI Structure and function of small airways in health and disease. SO Arch-Pathol-Lab-Med. 1978 Dec. 102(12). P 609-17. (REVIEW). MJ BRONCHI. MN AGING. AIRWAY-RESISTANCE. ANIMAL. BRONCHI: pa, ph, pp. BRONCHIAL-DISEASES: di. BRONCHITIS: pa. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. DOGS. GRAVITATION. HUMAN. INFANT. LUNG-COMPLIANCE. LUNG-VOLUME-MEASUREMENTS. MAXIMAL-EXPIRATORY-FLOW-RATE. PULMONARY-EMPHYSEMA: pa. RESPIRATORY-TRACT-DISEASES: pa. REVIEW. SMOKING. AB During the last two decades, great strides have been made in our understanding of the functional aspects of airflow in the periphery of the lung. It seems that the small airways are the important site of obstruction in a variety of chronic respiratory disorders associated with airflow obstruction. This review deals with the anatomic and functional aspects of small airways in normal and diseased lungs. In particular, the basis of obstruction or narrowing that is not dependent on intrinsic airway lesion is reviewed. The variety of pathologic changes in small airways observed in these diseases are outlined along with physiologic tests that are currently used to detect dysfunction at a stage long before they produce symptoms or alter standard tests of lung function. RF 001 GOUGH J PROC R SOC MED 45 576 952 002 MATSUBA K AM REV RESPIR DIS 104 516 971 003 WEST JB J APPL PHYSIOL 14 753 959 004 PEDLEY TJ RESPIR PHYSIOL 9 387 970 005 AMBERSON JB TRANS ASSOC AM PHYSICIANS 60 92 947 006 SPAIN DM AM REV TUBERC 68 24 953 007 WEIBEL ER MORPHOMETRY OF THE HUMAN LUNG 963 008 HORSEFIELD K J APPL PHYSIOL 24 373 968 009 SMITH P THORAX 29 147 974 010 KUHN C IN: BOUHUYS A 91 976 011 BREEZE RG AM REV RESPIR DIS 116 705 977 012 BASSET F Z ZELLFORSCH MIKROSK ANAT 116 45 971 013 EL-BERMANI AL-W AM J ANAT 137 12 973 014 HUNG K-S J ULTRASTRUCT RES 43 426 973 015 HUGHES JMB J APPL PHYSIOL 32 25 972 016 BRISCOE WA J CLIN INVEST 37 1279 958 017 MILIC-EMILI J J APPL PHYSIOL 21 749 966 018 GLAZIER JB J APPL PHYSIOL 23 694 967 019 WEST JB J APPL PHYSIOL 32 332 972 020 TURNER JM J APPL PHYSIOL 25 664 968 021 NIEWOEHNER DE J APPL PHYSIOL 36 412 974 022 MACKLEM PT RESPIR PHYSIOL 8 191 970 023 MACKLEM PT J APPL PHYSIOL 22 395 967 024 HOGG JC N ENGL J MED 278 1355 968 025 WOOLCOCK AJ J CLIN INVEST 48 1097 969 026 OLSON DE J APPL PHYSIOL 28 482 970 027 HOGG JC N ENGL J MED 282 1283 970 028 HIGH RH PEDIATR CLIN NORTH AM 4 183 957 029 KILBURN KH LAB INVEST 28 55 973 030 MAHVI D AM J PATHOL 86 559 977 031 LULLMANN-RAUCH R VIRCHOWS ARCH CELL PATHOL 11 167 972 032 SNIDER GL AM REV RESPIR DIS 85 666 962 033 BIGNON J AM REV RESPIR DIS 99 669 969 034 BIGNON J THORAX 25 556 970 035 MATSUBA K AM J PATHOL 67 265 972 036 DEPIERRE A CHEST 62 699 972 037 MATSUBA K AM REV RESPIR DIS 107 552 973 038 ZUELZER WW PEDIATRICS 4 53 949 039 WHITWELL F THORAX 7 213 952 040 ANON HEALTH CONSEQUENCES OF SM 972 041 ANDERSON AE JR ARCH ENVIRON HEALTH 12 569 966 042 SEELY JE SCIENCE 172 741 971 043 NIEWOEHNER DE N ENGL J MED 291 755 974 044 BODE FR AM J MED 59 43 975 045 INGRAM RH BULL PHYSIOPATH RESPIR NANCY 7 195 971 046 DAYMAN H J CLIN INVEST 30 1175 951 047 FRY DL AM J MED 29 672 960 048 MEAD J J APPL PHYSIOL 22 95 967 049 MACKLEM PT J APPL PHYSIOL 20 653 965 050 MARTIN RR AM REV RESPIR DIS 111 119 975 051 SCHILDER DP J CLIN INVEST 42 1705 963 052 HUTCHEON M AM REV RESPIR DIS 110 458 974 053 MACKLEM PT J CLIN INVEST 44 897 965 054 DESPAS PJ J CLIN INVEST 51 3235 972 055 MCCARTHY DS AM J MED 52 747 972 056 ANTHONISEN NR RESPIR PHYSIOL 8 58 969 057$ OTIS AB J APPL PHYSIOL 22 395 967 058 HOWELL JBL IN: STRETTON TB 157 976 CT 1 BASKERVILLE A NZ VET J 29 235 981 2 EBERT RV BR J DIS CHEST 75 277 981 3 LINHARTOVA A ARCH PATHOL LAB MED 106 499 982 4 MARIN ML LUNG 160 257 982 5 KILBURN KH ARCH ENVIRON HEALTH 38 277 983 PN 78041 RN 00822 AN 78186162 AU Beckerman-R-C. Taussig-L-M. TI The spectrum of cystic fibrosis in children and adults. SO Ariz-Med. 1978 Apr. 35(4). P 246-9. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. EX Cystic fibrosis, an autosomal recessive disorder, remains the most common lethal genetic disease of Caucasian people. The diagnosis of CF is usually made based upon the clinical presentation of chronic respiratory and/or gastrointestinal symptoms, a positive sweat electrolyte test and, often, a family history of the disorder. The manifestations of chronic cough, recurrent pneumonias, bronchiectasis with clubbing, pneumothorax, hemoptysis, and malabsorption secondary to pancreatic insufficiency are so well associated with cystic fibrosis that the diagnosis is usually not delayed. However, it is often not appreciated by physicians that CF may be associated with a broad spectrum of less common manifestations as well as extreme variability in severity of illness. Lack of familiarity with these manifestations may delay diagnosis until adolescence or adulthood. First, there seems to be mild clinical expression of the disorder in some patients (genetic variability). Second, the subtle manifestations of CF are not widely appreciated by physicians in practice. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 MITCHELL-HEGGS PF Q J MED 45 479 976 004 NADLER HL IN: STANBURY JB 1683 978 005 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 006 TAUSSIG LM PRACTICE OF PEDIATRICS 976 007 WOOD RE AM REV RESPIR DIS 113 833 976 PN 78042 RN 00823 AN 78256546 AU Davidson-G-P. Phelan-P-D. Townley-R-R. TI A controlled trial using intravenous infusion of soya oil emulsion in the treatment of children with cystic fibrosis. SO Aust-Paediatr-J. 1978 Jun. 14(2). P 80-2. MJ CYSTIC-FIBROSIS: dt. OILS: ad. MN CLINICAL-TRIALS. EMULSIONS. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. INFUSIONS-PARENTERAL. MALE. OILS: tu. SOY-BEANS. AB Five children with cystic fibrosis were given repeated intravenous infusions of soya oil emulsion (Intralipid) for 12 to 18 months and the effects on growth, health, sweat chloride and pancreatic enzyme activity compared with a control group. No beneficial effect was demonstrated and it is recommended that this form of therapy has no place in the routine treatment of children with cystic fibrosis. It is possible that treatment may have modified the degree of weight loss noted after 12 months of age. The lack of other beneficial effects and the problems involved in long-term Intralipid infusion should preclude the use of this form of therapy in the routine treatment of children with cystic fibrosis. RF 001 ELLIOTT RB ARCH DIS CHILD 50 76 975 002 ELLIOTT RB PEDIATRICS 57 474 976 003 FAJANS SS HANDBOOK OF PHYSIOLOGY SECT 7 1 473 972 004 GIBSON LE PEDIATRICS 23 545 959 005 WATSON EH GROWTH AND DEVELOPMENT OF CHI 96 967 006 WILLIAMS HE RESPIRATORY ILLNESS IN CHILDR 975 CT 1 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 2 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 3 ROGIERS V EUR J PEDIATR 141 39 983 4 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 PN 78043 RN 00824 AN 78256539 AU Phelan-P-D. TI Management of cystic fibrosis [editorial]. SO Aust-Paediatr-J. 1978 Jun. 14(2). P 61-2. MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. CHILD. HUMAN. PATIENT-CARE-TEAM. EX Controversy still surrounds the most appropriate method of management of children with cystic fibrosis. The reason for much of the uncertainty about management of cystic fibrosis is that many therapeutic approaches have been proposed without properly conducted controlled trials. With the current approaches to therapy, at least 80% of children with cystic fibrosis can expect to reach adult life, most with minimal or no lung damage. However, this dramatic improvement in survival does not indicate that cystic fibrosis is now an easy condition to manage and that it no longer requires specialised care. RF 001 ELLIOTT RB ARCH DIS CHILD 50 76 975 002 ELLIOTT RB PEDIATRICS 57 474 976 CT 1 NOLAN G J PEDIATR 101 626 982 PN 78044 RN 00825 AN 78256545 AU Barnes-G-L. McNaught-S. TI False negative sweat tests with apparently adequate sweat collections. SO Aust-Paediatr-J. 1978 Jun. 14(2). P 78-9. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHILD. CHLORIDES: an. FALSE-NEGATIVE-REACTIONS. HUMAN. SODIUM: an. AB Sweat tests collected on two or more occasions from normal children and children with cystic fibrosis have been analysed according to the weight of sweat collected. In five cases collections of 50-99 mg of sweat gave false negative results. Although 50-100 mg of sweat is considered adequate by some authorities our results do not support this. The study confirms the view that at least 100 mg of sweat must be collected to give reliable results. RF 001 BRAY PT CLIN CHIM ACTA 80 333 977 002 ANON J PEDIATR 88 711 976 003 ANON GAP CONF REP PROB SWEAT TESTI 975 004 GIBSON LE PROC FOR QUANTITATIVE ION 975 005 SCHWARZ V ARCH DIS CHILD 52 870 977 CT 1 COLLINS JE ACTA PAEDIATR SCAND 74 423 985 2 CHRISTOFFEL KS J PEDIATR 107 231 985 PN 78045 RN 00826 AN 78256549 AU Field-P. TI Cystic fibrosis: a review of clinic patients. SO Aust-Paediatr-J. 1978 Jun. 14(2). P 91-4. MJ CYSTIC-FIBROSIS: th. RESPIRATORY-TRACT-INFECTIONS: pc. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. HUMAN. INFANT. AB The response of 72 patients to an intensive antimicrobial prophylactic programme is presented in this review. Treatment was first commenced in 1968 and was evaluated during the last 4 months of 1975. The assessment confirmed a favourable clinical impression. RF 001 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 NOLAN G J PEDIATR 101 626 982 PN 78046 RN 00827 AN 79021079 AU Conover-J-H. Conod-E-J. TI The influence of cystic fibrosis serum and calcium on secretion in the rabbit tracheal mucociliary apparatus. SO Biochem-Biophys-Res-Commun. 1978 Aug 29. 83(4). P 1595-601. MJ CALCIUM: pd. CYSTIC-FIBROSIS: bl. IGG: ph. MUCUS: se. TRACHEA: se. MN A-23187: pd. ANIMAL. CYSTIC-FIBROSIS: fg, pp. EPITHELIUM: se. HETEROZYGOTE. RABBITS. TISSUE-CULTURE. TRACHEA: de. AB Cystic Fibrosis serum or its isolated component IgG fraction and calcium ionophore A23187 all produced a quantitatively greater increase of mucus glycoprotein secretion in the rabbit tracheal epithelium than did control serum or its isolated component IgG fraction. These values were determined by dry weight secretion per gram of tissue and on subsequent sialic acid content of secretions. This demonstrable increase in mucus production represents a measurable difference in the functioning of the cultured mucociliary apparatus due to the influence of cystic fibrosis serum. RF 001 BOGART BI PEDIATR RES 11 131 977 002 WILSON GB PEDIATR RES 11 143 977 003 SPOCK A PEDIATR RES 1 173 967 004 CONOVER JH PEDIATR RES 7 220 973 005 CONOVER JH LANCET 2 1362 976 006 BOGART BI PEDIATR RES 12 15 978 007 WARREN L J BIOL CHEM 234 1971 959 008 SCHNEIDER WC METHODS ENZYMOL 3 680 957 009 CONOD EJ PEDIATR RES 11 45 977 010 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 011 LOWRY OH J BIOL CHEM 193 265 951 CT 1 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 2 DAVIS PB IRCS MED SCI BIOCHEM 8 413 980 3 DAVIS PB PEDIATR RES 14 83 980 4 KENNEDY JR PEDIATR RES 14 1173 980 5 PHIPPS RJ EUR J RESPIR DIS 61 33 980 6 WILSON GB J CLIN INVEST 66 1010 980 7 HALLINAN F MED HYPOTHESES 7 793 981 8 ADLER KB LAB INVEST 45 372 981 9 BOAT TF PEDIATR RES 16 792 982 10 BOGART BI PEDIATR RES 16 223 982 11 QUISSELL DO PEDIATR RES 17 899 983 12 MCPHERSON MA CLIN CHIM ACTA 135 181 983 13 KATZ S CELL CALC 5 421 984 14 BARBIERI EJ BR J PHARMACOL 82 199 984 15 RUSSI EW CHEST 86 475 984 16 WOOTEN MW J CELL PHYSIOL 121 490 984 17 NEAL JL EVOLUTIONARY THEORY 7 153 985 PN 78047 RN 00828 AN 79020917 AU Choy-H. Applegarth-D-A. Davidson-A-G. Wong-L-T. TI Anti-plasmin (alpha2-macroglobulin) activity of plasma from cystic fibrosis patients. SO Biochem-Biophys-Res-Commun. 1978 Jun 29. 82(4). P 1325-8. MJ ALPHA-MACROGLOBULINS: me. CYSTIC-FIBROSIS: bl. MN CASEINS. FIBRINOLYSIN: ai. HUMAN. AB We have studied the plasim-alpha2-macroglobulin interaction using plasma from cystic fibrosis patients. In this system alpha2-macroglobulin from cystic fibrosis plasma does not differ from controls in its ability to bind with and inhibit plasmin. RF 001 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 002 SHAPIRA E CLIN CHIM ACTA 78 359 977 003 CRAWFORD GPM CLIN SCI MOL MED 51 215 976 004 SGOURIS JT VOX SANG 5 357 960 005 ALKAERSIG N J BIOL CHEM 234 32 959 006 NORMAN PS J EXP MED 106 423 957 007 NORMAN PS J EXP MED 108 639 958 008 HARPEL PC PROC 5TH CONG INT SOC THROM H 975 009 BARRETT AJ BIOCHEM J 133 709 973 CT 1 DAVIS PB PEDIATR RES 14 83 980 2 ROMEO G J LAB CLIN MED 95 116 980 3 PARSONS M CLIN CHIM ACTA 100 215 980 4 RICHMAN JBY CAN J BIOCHEM 59 519 981 5 BURY AF IRCS MED SCI BIOCHEM 10 255 982 6 ROBERTS RC PEDIATR RES 16 416 982 7 BACK SA BIOCHEM MED 30 34 983 8 SHAPIRA E ANN NY ACAD SCI 421 352 983 9 BRUCE MC AM REV RESPIR DIS 132 529 985 PN 78048 RN 00829 AN 79082306 AU Banschbach-M-W. Karam-A-G. Love-P-K. Hilman-B-C. TI Cystic fibrosis serum promotes [45Ca] uptake by normal human leukocytes. SO Biochem-Biophys-Res-Commun. 1978 Oct 30. 84(4). P 922-7. MJ CALCIUM: bl. CYSTIC-FIBROSIS: pp. LEUKOCYTES: me. MN BIOLOGICAL-TRANSPORT-ACTIVE. HUMAN. AB A two-fold increase in [45Ca] labeling was observed when normal human leukocytes were incubated in the presence of serum obtained from patients with cystic fibrosis. The degree of [45Ca] labeling of leukocytes isolated from patients with cystic fibrosis was also significantly greater than that observed for leukocytes that had been isolated from normal individuals. RF 001 BOGART BI PEDIATR RES 11 131 977 002 BOGART BI PEDIATR RES 12 15 978 003 OCHSMAN JL GAP CONF REP CALC ION CELL SE 976 004 DAVIS PB PEDIATR RES 12 703 978 005 RASMUSSEN H ADV CYCLIC NUCLEO RES 5 375 975 006 ANDERSON LE ANAL BIOCHEM 51 173 973 007 SCHREURS VVAM BIOCHIM BIOPHYS ACTA 419 320 976 008 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 009 SHAPIRO BL CLIN CHIM ACTA 82 125 978 010 WILKOWSKE CJ MAYO CLIN PROC 52 616 977 011 NEU HC BULL NY ACAD MED 54 141 978 012 MAREN TH ANNU REV PHARMACOL TOXICOL 16 309 976 013 WILSON GB NATURE 266 463 977 CT 1 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 2 DAVIS PB IRCS MED SCI BIOCHEM 8 413 980 3 DAVIS PB PEDIATR RES 14 83 980 4 DAVIS PB J LAB CLIN MED 96 75 980 5 BOGART BI PEDIATR RES 16 223 982 6 FEIGAL RJ LIFE SCI 30 93 982 7 SHAPIRO BL SCIENCE 216 417 982 8 KATZ S CELL CALC 5 421 984 9 BOUCHER RC PEDIATR RES 18 1336 984 10 VONRUECKER AA PEDIATR RES 18 594 984 11 HART DA IRCS MED SCI BIOCHEM 14 213 986 12 KEMP T PEDIATR RES 20 520 986 13 FEIGAL RJ ANN NY ACAD SCI 488 82 986 14 ZAKRZEWSKI JT BR J CLIN PHARMACOL 23 19 987 PN 78049 RN 00830 AN 78186681 AU Gahl-W-A. Chesney-J. Pitot-H-C. TI Serum ribonuclease levels in patients with cystic fibrosis. SO Biochem-Med. 1978 Apr. 19(2). P 294-7. MJ CYSTIC-FIBROSIS: en. RIBONUCLEASES: bl. MN CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. PANCREAS: en. SUPPORT-U-S-GOVT-P-H-S. EX Cystic fibrosis is an autosomal recessive disease, usually fatal before the third decade, characterized by chronic obstructive lung disease, elevated sweat electrolyte concentrations, and pancreatic insufficiency. The present investigation was carried out to determine if P-type serum RNase activity was reduced in CF patients 3 to 24 years of age. No difference in serum RNase activity was found among the groups of 17 normals, 21 CF patients, and 5 CF heterozygotes. It appears that observations of decreased RNase activity in the serum of young children with pancreatic insufficiency cannot be extended to older children, at least without withholding pancreatic supplements. RF 001 LEVY CC LIFE SCI 17 311 975 002 BARDON A CLIN CHIM ACTA 67 231 976 003 REDDI KK BIOCHEM BIOPHYS RES COMMUN 67 110 975 004 ALPERS DH J BIOL CHEM 242 5617 967 005 SCHMUKLER M J BIOL CHEM 250 2206 975 006 ZAN-KOWALCZEWSKA M BIOCHIM BIOPHYS ACTA 341 138 974 007 BARDON A BIOCHIM BIOPHYS ACTA 438 461 976 008 ZIMMERMAN SB ANAL BIOCHEM 10 444 965 CT 1 WARSHAW AL SURGERY 86 227 979 2 ISAACS P DIGESTION 22 101 981 3 THOMAS JM CLIN CHIM ACTA 111 199 981 4 WEICKMANN JL J BIOL CHEM 257 8705 982 5 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 PN 78050 RN 00831 AN 79104083 AU Antonowicz-I. Ishida-S. Shwachman-H. TI Studies in meconium in cystic fibrosis: the activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase. SO Biol-Neonate. 1978. 34(5-6). P 225-30. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: me. MECONIUM: en. PHOSPHATASES: me. MN ACID-PHOSPHATASE: me. ALKALINE-PHOSPHATASE: me. FUCOSIDASE: me. GLUCURONIDASE: me. HUMAN. INFANT-NEWBORN. MANNOSIDASES: me. AB The specific activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase were studied in meconium from infants with cystic fibrosis (CF) and control subjects. The study revealed significant variations in the specific activity of the enzymes except for acid phosphatase. The variations were not uniform. The activities of alpha-D-mannosidase, beta-glucuronidase and alkaline phosphatase were markedly decreased (p less than 0.001, p less than 0.002, p less than 0.001, respectively), while the activity of beta-D-fucosidase was significantly increased (p less than 0.001) in meconium from the infants with CF. It is suggested that the decreased activity of alpha-D-mannosidase and beta- glucuronidase might contribute to the accumulation of the abnormal substances in CF meconium. The highly increased activity of beta-D- fucosidase raises the possibility of an additional or alternative method for screening newborns for CF using meconium as the test material. RF 001 ANDERSEN H ACTA PATH MICROBIOL SCAND 61 377 964 002 ANDERSEN DH AM J DIS CHILD 72 62 946 003 ANTONOWICZ I GASTROENTEROLOGY 67 51 974 004 ANTONOWICZ I PEDIATRICS 56 782 975 005 ANTONOWICZ I PEDIATR RES 6 803 972 006 BARRETT AJ IN: DINGLE JT 116 973 007 BIERRING F ACTA PATH MICROBIOL SCAND 61 365 964 008 BUCHANAN DJ PEDIATRICS 9 304 952 009 CAIN ARR ARCH DIS CHILD 47 131 972 010 CHERNICK WS ANN NY ACAD SCI 106 698 963 011 CROW JF IN: NEEL JV 23 965 012 DANKS DM ANN HUM GENET 28 323 965 013 DISCHE Z AM J DIS CHILD 102 733 961 014 DISCHE Z PEDIATRICS 24 74 959 015 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 016 FRASER D CLIN CHIM ACTA 59 301 975 017 GREEN MN PEDIATRICS 21 635 958 018 GREEN MN PEDIATRICS 41 989 968 019 JOHANSEN PG ANN NY ACAD SCI 106 755 963 020 KNAUFF RE PROC SOC EXP BIOL MED 127 801 968 021 KOPITO L J PEDIATR 68 313 966 022 LEV R HISTOCHEMIE 29 103 972 023 LEVVY GA BIOCHEM J 80 433 961 024 LINHARDT K IN: BERGMEYER HU 783 965 025 LOWRY OH J BIOL CHEM 193 265 951 026 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 027 ROELFS RE AM J DIS CHILD 113 419 967 028 ROMEO D BIOCHIM BIOPHYS ACTA 159 194 968 029 RULE AH PEDIATRICS 45 847 970 030 RULE AH PEDIATRICS 48 601 971 031 RYLEY HC ARCH DIS CHILD 49 901 974 032 SCHACHTER H CAN J BIOCHEM 43 381 965 033 SCHUTT WH ARCH DIS CHILD 43 178 968 034 SHWACHMAN H ANN NY ACAD SCI 93 600 962 035 SIEGAL S NONPARAMETRIC STATISTICS 956 036 STEINBERG AG AM J HUM GENET 12 416 960 037 STEPHAN U PEDIATRICS 55 35 975 038 TAPPEL AL J BIOL CHEM 242 2463 967 039 VAN HOOF F IN: HERS HG 973 040 WATZKA M VERH ANAT GES 66 971 CT 1 HSIEH MC CLIN CHEM 27 388 981 2 LAMBOTTE C J GENET HUM 29 85 981 3 CABEZAS JA INT J BIOCHEM 15 243 983 4 RYLEY HC CLIN CHIM ACTA 135 49 983 PN 78051 RN 00832 AN 79083227 AU Summitt-R-L. TI Wolffian duct anomalies [editorial]. SO Birth-Defects. 1978. 14(6C). P 54-5. MJ CYSTIC-FIBROSIS: fg. VAS-DEFERENS: ab. WOLFFIAN-DUCT. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. INFANT-NEWBORN. MALE. MIDDLE-AGE. PHENOTYPE. INFERTILITY-MALE. EX The fact that a serum factor in cystic fibrosis patients inhibits ciliary movement of cells of isolated tracheae, and the observation that ciliary movement is associated with pinocytotic activity led Ohno et al. to suggest that this defect is responsible for aplasia or hypoplasia of the wolffian duct structures in males with cystic fibrosis. While Ohno's hypothesis lacks definitive proof, the hypoplasia or aplasia of the vas in males with cystic fibrosis is extremely common, producing sterility in affected males. RF 001 KAPLAN E N ENGL J MED 279 65 968 002 VALMAN HB LANCET 2 566 969 003 OHNO S HEREDITAS 69 107 971 PN 78052 RN 00833 AN 78103772 AU Mathieu-J-P. Stack-B-H. Dick-W-C. Buchanan-W-W. TI Pulmonary infection and rheumatoid arthritis. SO Br-J-Dis-Chest. 1978 Jan. 72(1). P 57-61. MJ ARTHRITIS-RHEUMATOID: co. BRONCHIECTASIS: co. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. ARTHRITIS-RHEUMATOID: im. FEMALE. HUMAN. MALE. RESPIRATORY-FUNCTION-TESTS. RHEUMATOID-FACTOR: an. AB Five of eleven patients with bronchiectasis and/or cystic fibrosis developed a polyarthritis with positive tests for rheumatoid factor. Possible mechanisms of this complication are discussed. RF 001 ALARCON-SEGOVIA D MEDICINE (BALTIMORE) 46 1 967 002 ARONOFF A BR MED J 2 228 955 003 BERKSON J PROC STAFF MTG MAYO CLIN 30 319 955 004 BRINKMAN GH AM REV TUBERC PULM DIS 80 732 959 005 GROSSMAN H AM J DIS CHILD 107 1 964 006 HARRIS J ARTHRITIS RHEUM 4 47 961 007 ISAACS AJ TUBERCLE 55 135 974 008 KELLGREN JH PRACTITIONER 193 257 964 009 KUNKEL HG ARTHRITIS RHEUM 1 289 958 010 LEE SL ARCH INTERN MED 117 620 966 011 MAINLAND D AM HEART J 45 644 953 012 MURRAY HW AM J MED 58 229 975 013 PONCET A POLYARTHRITE TUBERCULEU 897 014 SINGER JM ARTHRITIS RHEUM 4 124 961 015 VIRSHUP AM ARTHRITIS RHEUM 16 388 973 016 VOGL A AM J MED 18 51 955 017 WALKER WC Q J MED 36 239 967 018 WEINSTEIN MP AM J DIS CHILD 127 125 974 019 WHITE C BR MED J 2 1284 953 CT 1 HOWIE AD SCOTT MED J 24 193 979 2 SAGRANSKY DM AM J DIS CHILD 134 319 980 3 SUTTON PP ANN RHEUM DIS 41 47 982 4 DELUMLEY L ARCH FR PEDIATR 40 723 983 5 HILTON AM THORAX 39 185 984 6 SCHIDLOW DV ARCH DIS CHILD 59 377 984 7 BAMJI A SCAND J RHEUMATOL 14 15 985 8 BERKIN KE EUR J RESPIR DIS 67 103 985 9 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 10 PHILLIPS BM J ROY SOC MED 79 44 986 PN 78053 RN 00834 AN 78145446 AU Benghial-A. TI Meconium ileus equivalent an adult: a case report. SO Br-J-Surg. 1978 Mar. 65(3). P 215. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: co. INTESTINE-SMALL. MN ACETYLCYSTEINE: tu. ADULT. CASE-REPORT. HUMAN. INTESTINAL-OBSTRUCTION: dt. MALE. AB A case is described of meconium ileus equivalent presenting as a small bowel obstruction in an adult patient with cystic fibrosis. The importance of conservative management with acetylcysteine and intravenous fluids is emphasized. RF 001 FISHER OD ARCH DIS CHILD 29 262 954 002 GRACEY M ARCH DIS CHILD 44 404 969 003 HODSON ME BR MED J 2 790 976 004 HUNTON DB GASTROENTEROLOGY 50 99 966 005 LILLIBRIDGE CB J PEDIATR 71 887 967 PN 78054 RN 00835 AN 78104639 AU Harries-J-T. TI Meconium in health and disease. SO Br-Med-Bull. 1978 Jan. 34(1). P 75-8. (REVIEW). MJ CYSTIC-FIBROSIS: me. MECONIUM: an. MN BILE-ACIDS-AND-SALTS: an. BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: co. GLYCOPROTEINS: an. HEMOGLOBINS: an. HUMAN. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: et, me. MECONIUM: en. REVIEW. STEROIDS: an. EX Meconium can be defined as the first stools passed by a newborn infant, the first stool being passed within 24 hours of birth by more than 90% of newborns. This paper will review current knowledge on the sources of meconium, and its composition in health and disease states. The precise origin of the components of meconium has not been defined. The qualitative and quantitative patterns of the individual constituents of meconium in health are considered: water and inorganic elements, enzymes, plasma proteins, glycoproteins, lipids, haemoglobin metabolites, steroids and bile acids, and sterols. Meconium from infants with cystic fibrosis has been more extensively studied than in any other disease and the paper concentrates on this entity. The composition of meconium in cystic fibrosis, screening tests for cystic fibrosis, the diagnosis of cystic fibrosis, and meconium ileus are discussed. RF 001 ANTONOWICZ I PEDIATRICS 56 782 975 002 BUCHANAN DJ J BIOL CHEM 192 251 951 003 BUCHANAN DJ PEDIATRICS 9 304 952 004 COTE RH IN: WOLLHEIM E 1 489 962 005 COTE RH IN: AMINOFF D 249 970 006 COTE RH BIOCHEM J 153 63 976 007 DI SANTAGNESE PA N ENGL J MED 295 481 976 008 EGGERMONT E BIOL NEONATE 10 266 966 009 FARBER S J PEDIATR 24 387 944 010 FRASER D CLIN CHIM ACTA 59 301 975 011 FRENCH J BIOCHEM J 120 393 970 012 GIBSON LE PEDIATRICS 23 545 959 013 GREEN MN PEDIATRICS 41 989 968 014 GUSTAFSSON JA EUR J BIOCHEM 22 246 971 015 HALL IC AM J DIS CHILD 47 1270 934 016 HURWITT ES AM J DIS CHILD 64 443 942 017 KARLSON P INTRODUCTION TO MODERN BIOCHE 314 968 018 KINSELLA RA JR J CLIN ENDOCRINOL METAB 32 801 971 019 KNAUFF RE PROC SOC EXP BIOL MED 127 801 968 020 KOPITO L J PEDIATR 68 313 966 021 LIEBERMAN J GASTROENTEROLOGY 50 183 966 022 OPPENHEIMER EH ARCH PATHOL 96 149 973 023 PRITCHARD JA OBSTET GYNECOL 28 606 966 024 PROSSER R ARCH DIS CHILD 49 597 974 025 RULE AH PEDIATRICS 45 847 970 026 RULE AH PEDIATRICS 48 601 971 027 RYLEY HC ARCH DIS CHILD 49 901 974 028 SCHACHTER H CAN J BIOCHEM 43 381 965 029 SCHUTT WH ARCH DIS CHILD 43 178 968 030 SCHWARZ E ANN PAEDIATR 181 306 953 031 ACHWIDT W VERH ANAT GES 66 55 971 032 SHARP HL PEDIATR RES 5 274 971 033 SMITH CA PHYSIOLOGY OF THE NEWBORN 235 959 034 SPRINGER GF BIOCHEMISTRY 5 3254 966 035 YOUNG DM PROC SOC EXP BIOL MED 99 673 958 CT 1 KARLSSON KA J BIOL CHEM 256 3512 981 2 KATZ S DIGESTION 24 98 982 3 FRANCOUAL J ANN BIOL CLIN 40 227 982 4 STPYREK J J STEROID BIOCHEM 18 341 983 5 FRANCOUAL J CLIN CHEM 29 2054 983 6 SLOMIANY A J BIOL CHEM 258 8535 983 7 HANLY JG BR MED J 286 1411 983 8 DEHNER LP HUM PATHOL 17 807 986 9 MELLANDER L CLIN EXP IMMUNOL 63 555 986 10 CLARK DA J PEDIATR 110 765 987 PN 78055 RN 00836 AN 78104725 AU Dodge-J-A. Yassa-J-G. TI Zinc deficiency syndrome in a British youth with cystic fibrosis. SO Br-Med-J. 1978 Feb 18. 1(6110). P 411. MJ CYSTIC-FIBROSIS: co. ZINC: df. MN ADULT. CASE-REPORT. GENITALIA-MALE. GROWTH-DISORDERS: et. HUMAN. MALE. SEX-MATURATION. SYNDROME. EX A syndrome of dwarfism and retarded sexual development among Iranian and Egyptian teenage boys has been shown to be due to zinc deficiency. A British patient with cystic fibrosis and nephrotic syndrome showed growth retardation and hypogonadism, associated with low plasma zinc concentrations. The combination of cystic fibrosis and nephrotic syndrome appears to be fortuitous, and undoubtedly adverse effects on zinc homoeostasis were cumulative. Nevertheless, zinc deficiency should be considered when somatic and sexual growth failure occur in patients with cystic fibrosis. RF 001 PRASAD AS ARCH INTERN MED 111 407 963 002 HALSTED JA LANCET 1 322 970 003 EVANS GW IN: PRASAD AS 976 004 SANDSTEAD HH IN: PRASAD AS 137 976 005 STEELE TH J LAB CLIN MED 78 1019 971 CT 1 ATKINSON RL ANN INTERN MED 89 491 978 2 AGGETT PJ MONOGR PAEDIATR 10 8 979 3 WALRAVENS PA WEST J MED 130 133 979 4 SHAW JCL AM J DIS CHILD 133 1260 979 5 KYNAST G J PERINATAL MED 8 171 980 6 SOLOMONS NW AM J CLIN NUTR 33 2031 980 7 PARSONS HG PEDIATRICS 66 812 980 8 MITCHELL EA NZ MED J 92 6 980 9 SOLOMONS NW AM J CLIN NUTR 34 462 981 10 BEDDOES V PRACTITIONER 225 557 981 11 HANSEN MA ANNU REV NUTRITION 2 151 982 12 SOLOMONS NW AM J CLIN NUTR 35 1048 982 13 SOLOMONS NW J AM DIET ASSOC 80 115 982 14 PATRICK J CRC CRIT REV CLIN LAB SCI 20 95 984 15 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 16 DODGE JA J ROY SOC MED 79 27 986 PN 78056 RN 00837 AN 78145601 AU Hodson-M-E. TI Diseases of the respiratory system: Bronchiectasis and cystic fibrosis. SO Br-Med-J. 1978 Apr 15. 1(6118). P 971-3. MJ CYSTIC-FIBROSIS. MN BRONCHIECTASIS: co, et, th. CYSTIC-FIBROSIS: co, di, th. GENETIC-COUNSELING. HUMAN. EX Bronchiectasis is characterised by dilatation of the bronchi. This dilation may be generalised or confined to a single segment or lobe of the lung, and it may be tubular, fusiform (spindle shaped), or saccular (cystic). The aetiology, symptoms, diagnosis, treatment, complications, and prevention of bronchiectasis are considered. Cystic fibrosis affects many exocrine glands throughout the body. Presentation and diagnosis, treatment, complications and their treatment, management of associated social problems, and death due to cystic fibrosis are discussed. RF 001 HODSON ME BR MED J 2 790 976 CT 1 TODD JW BR MED J 1 1281 978 2 FLOWER KA BR MED J 2 630 979 3 FLOWER KA MONOGR PAEDIATR 10 54 979 4 REDDING GJ AM REV RESPIR DIS 126 31 982 5 ANON LANCET 2 257 983 6 HODSON ME BR J DIS CHEST 77 71 983 PN 78057 RN 00838 AN 78145788 AU Norman-A-P. TI Bronchiectasis and cystic fibrosis [letter]. SO Br-Med-J. 1978 May 6. 1(6121). P 1217. MJ BRONCHIECTASIS: th. CYSTIC-FIBROSIS: th. MN CHILD. HUMAN. PROGNOSIS. EX The otherwise excellent article on bronchiectasis and cystic fibrosis by Dr Margaret Hodson is seriously marred by the failure to emphasise that the advice on management and drug dosage is directed throughout to the adult and adolescent, and not to the child. Firstly, tetracycline should not be prescribed for young children for obvious reasons; secondly, the dosage of all antibiotics recommended is high for infants and small children. The final paragraph gives a pessimistic impression of an undoubtedly distressing condition by stressing the early mortality of the past and of many of the older patients of today, without noting the effect that early diagnosis - perhaps as a result of neonatal screening - together with more effective treatment may have in the future on the quality of life and length of life. RF 001 HODSON ME BR MED J 1 971 978 PN 78058 RN 00839 AN 79043563 AU Walker-L. TI My fight for life. SO Can-Nurse. 1978 Nov. 74(10). P 28-9. MJ ATTITUDE-TO-DEATH. CYSTIC-FIBROSIS: px. MN ADULT. CASE-REPORT. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. PNEUMONIA: co. PULMONARY-EMPHYSEMA: et, px. EX A patient with cystic fibrosis discusses her experiences with the disease. PN 78059 RN 00840 AN 78188959 AU Breslow-J-L. Epstein-J. Fontaine-J-H. TI Dexamethasone-resistant cystic fibrosis fibroblasts show cross-resistance to sex steroids. SO Cell. 1978 Apr. 13(4). P 663-9. MJ CYSTIC-FIBROSIS. DEXAMETHASONE: pd. ESTRADIOL: pd. PROGESTERONE: pd. STANOLONE: pd. MN CELL-SURVIVAL: de. CELLS-CULTURED. DEXAMETHASONE: me. DRUG-RESISTANCE. FEMALE. FIBROBLASTS: de, me. HUMAN. MALE. SUPPORT-U-S-GOVT-NON-P-H-S. AB Diploid skin fibroblasts derived from individuals with the autosomal recessive disease, cystic fibrosis (CF), were shown previously to be significantly more resistant to the cytotoxicity of dexamethasone, a glucocorticoid hormone, than were normal human fibroblasts. Here cystic fibrosis fibroblasts are also shown to be more resistant than normal human fibroblasts to the cytotoxic effects of the sex hormones, 17 beta-estradiol, dihydrotestosterone and progesterone. Since cells are believed to contain different receptors for each of the steroid hormones, it is not probable than the resistance of CF cells to these hormones results from a receptor deficiency. This was shown by the fact that CF cells were found to exhibit the same receptor activity as normal cells for 3-H-dexamethasone. Furthermore, neither normal human nor CF fibroblasts could be demonstrated to contain detectable receptor activity for 3H-17 beta-estradiol. In addition, the studies of fibroblast killing by hormones led to the further interesting observation that normal human diploid fibroblasts, regardless of the sex of the tissue donor, are sensitive to killing by each of the sex hormones. These findings suggest that the cytotoxic effects of the steroid hormones may be observed independently of the specific hormone receptors. The studies reported here thus suggest that the resistance of CF cells to the different steroid hormones is probably the result of a defect in a pathway in cellular steroid hormone metabolism other than that involving receptors. RF 001 BAXTER JD SCIENCE 171 189 971 002 BOURGEOIS S CELL 11 423 977 003 BRESLOW JL EXP CELL RES 110 399 977 004 CHAN L N ENGL J MED 294 1322 976 005 CONNEALLY PM TEX REP BIOL MED 31 639 973 006 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 007 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 008 FREUND JE MODERN ELEMENTARY STATISTICS 967 009 GORSKI J ANNU REV PHYSIOL 38 425 976 010 GRIFFIN JE J CLIN INVEST 57 1342 976 011 HORWITZ KB STEROID 26 785 975 012 KAUFMAN M J CLIN INVEST 58 345 976 013 KEENAN BS J CLIN ENDOCRINOL METAB 38 1143 974 014 LEUNG K ANNU REV PHYSIOL 37 245 975 015 LOWRY OH J BIOL CHEM 193 265 951 016 MEYER WJ III PROC NAT ACAD SCI USA 72 1469 975 017 MIDDLEBROOK JL ENDOCRINOLOGY 100 271 977 018 NEIFELD JP J BIOL CHEM 252 2972 977 019 ROUSSEAU GG J STEROID BIOCHEM 6 75 975 020 SCATCHARD G ANN NY ACAD SCI 51 660 949 021 SIBLEY CH CELL 2 221 974 022 YAMAMOTO KR PROC NAT ACAD SCI USA 71 3901 974 023 YAMAMOTO KR IN: GREEP RO 32 3 976 CT 1 EPSTEIN J SOMATIC CELL GENET 4 451 978 2 BRESLOW JL SCIENCE 201 180 978 3 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 4 BRESLOW JL J CELL PHYSIOL 99 343 979 5 KURZ JB SCIENCE 206 1317 979 6 THOMPSON EB J STEROID BIOCHEM 12 193 980 7 DAVIS PB PEDIATR RES 14 83 980 8 CHASE HP METABOLISM 29 365 980 9 KOEFFLER HP CANCER RES 40 563 980 10 DEMEYTS P CIRC RES 46 I 3 980 11 BRESLOW JL SCIENCE 207 1007 980 12 PEDERSEN PS ACTA PAEDIATR SCAND 70 507 981 13 KELLER GH ARCH BIOCHEM BIOPHYS 211 321 981 14 BRESLOW JL N ENGL J MED 304 1 981 15 SEALE TW ANN CLIN LAB SCI 12 415 982 16 MINGUELL JJ ARCH BIOL MED EXP 15 457 982 17 JUNEJA HS EXP HEMATOL 12 221 984 18 LANGHOFF E PEDIATR RES 18 488 984 19 GROSS NJ AM REV RESPIR DIS 129 805 984 20 PINSKY L ADV HUM GENET 16 299 987 PN 78060 RN 00841 AN 79126049 AU Treton-J. Boucays-A. Dersakissian-H. Boue-A. Courtois-Y. TI The repair of DNA single strand breaks in human cells with genetical disorders. SO Cell-Biol-Int-Rep. 1978 Jul. 2(4). P 403-10. MJ DNA-REPAIR. DNA: re. DNA-SINGLE-STRANDED: bi. MN ADULT. CELL-LINE. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS. FIBROBLASTS. HUMAN. LESCH-NYHAN-SYNDROME. RETINOBLASTOMA. TRISOMY. X-RAYS. AB The aim of this work was to make a preliminary examination of some hereditary diseases, related to precocious aging in order to determine whether the defects involved resulted from an impairment of the DNA repair capacity. To test for an impairment in DNA single strand capacity, in cells from patients with different diseases, we have followed the rate of the DNA after X-irradiation by sedimentation on alkaline sucrose gradients. It was found that all cells observed from Lesh Nyhan, cystic fibrosis, trisomy 15 et 21, retinoblastoma, were able to repair the single strand breaks in their DNA to the same extent and at the same initial rate. RF 001 BOUE A IN VITRO 11 409 975 002 BRADLEY MO MUTATION RES 37 279 976 003 CLEAVER JE PROC NAT ACAD SCI USA 63 428 969 004 COUNIS MF DEVELOP BIOL 57 47 977 005 CURE S MOLEC CELLUL MECH AGING 27 47 973 006 EPSTEIN JL PROC NAT ACAD SCI USA 70 977 973 007 EPSTEIN J BIOCHEM BIOPHYS RES COMMUN 59 850 974 008 GIANNELLI F NATURE 265 446 977 009 HART RN IN: CUTLER RG 9 134 976 010 HIGURASHI M PEDIATR RES 6 514 972 011 KATO H NATURE 260 447 976 012 LAMBERT B ACTA MED SCAND 200 433 976 013 LINN S PROC NAT ACAD SCI USA 73 2818 976 014 LITTLE JB GERONTOLOGY 22 28 976 015 PATERSON MC NATURE 260 444 976 016 STRAUSS BS LIFE SCI 15 1685 974 017 TAYLOR AMR NATURE 258 427 975 018 TAYLOR AMR NATURE 260 441 976 019 TICE RR IN: CUTLER RG 9 60 976 020 TRETON JA EXP CELL RES 102 410 976 021 TRETON JA IN: COURTOIS Y 60 193 976 022 TROSKO JE IN: CUTLER RG 9 168 976 023 WEICHSELBAUM RR NATURE 266 726 977 CT 1 LEONARD JC MUTATION RES 105 417 982 PN 78061 RN 00842 AN 79106567 AU Fink-R-J. Doershuk-C-F. Tucker-A-S. Stern-R-C. Boat-T-F. Matthews-L-W. TI Pulmonary function and morbidity in 40 adult patients with cystic fibrosis. SO Chest. 1978 Dec. 74(6). P 643-7. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. CYSTIC-FIBROSIS: mi. FEMALE. HAEMOPHILUS-INFLUENZAE: ip. HUMAN. LUNG-VOLUME-MEASUREMENTS. MALE. PSEUDOMONAS-AERUGINOSA: ip. RESIDUAL-VOLUME. RESPIRATORY-FUNCTION-TESTS. SEX-FACTORS. STAPHYLOCOCCUS-AUREUS: ip. AB Pulmonary function and cardiopulmonary complications were studied in a group of 40 patients with cystic fibrosis who reached the age of 25 years. Mean values for vital capacity (VC), functional residual capacity, residual volume (RV), the ratio of RV over total lung capacity (RV/TLC), conductance, and the ratio of the forced expiratory volume in one second over VC were abnormal. There was a variable pattern of progression from patient to patient. The men differed from the women only in that they had a significantly larger TLC and inspiratory capacity than the women. The resultant preservation of VC may have an advantage for survival in those patients in whom it is observed. Pseudomonas aeruginosa was encountered with increasing frequency with age. Massive hemoptysis did not result in early death. The occurrence of rightsided heart failure secondary to cor pulmonale, with or without respiratory failure, was a poor prognostic sign. RF 001 ANON CF FND 1976 REP SURVI STUD PA 978 002 STERN RC ANN INTERN MED 87 188 977 003 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 004 MATTHEWS LW J PEDIATR 65 558 964 005 COMROE JH JR LUNG CLINICAL PHYSIOLOGY AND 962 006 DUBOIS AB J CLIN INVEST 35 322 956 007 HELLIESEN PJ PEDIATRICS 22 80 958 008 DOERSHUK CF J PEDIATR 65 677 964 009 SHWACHMAN H AM J DIS CHILD 96 615 958 010 STOWE SM AM REV RESPIR DIS 111 611 975 011 STERN RC AM REV RESPIR DIS 117 825 978 012 HOLSCLAW DS J PEDIATR 76 829 970 013 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 CT 1 HOLSCLAW DS CLIN CHEST MED 1 407 980 2 MATTHAY RA BR HEART J 43 474 980 3 FRANCIS PWJ AM J DIS CHILD 134 734 980 4 FICK RB CLIN CHEST MED 2 91 981 5 MATTHAY RA MED CLIN NORTH AM 65 489 981 6 GUIDOTTI TL RESPIRATION 44 351 983 7 HODGES P J AM DIET ASSOC 84 664 984 8 DAVIS PB SEM RESPIR MED 6 261 985 9 WIESEMANN HG MONATSSCHR KINDERHEILKD 133 726 985 10 KRUHLAK RT WEST J MED 145 196 986 PN 78062 RN 00843 AN 78147498 AU Ewing-C-W. TI Role of the fiberoptic bronchoscope in lung lavage of patients with cystic fibrosis. SO Chest. 1978 May. 73(5 Suppl). P 750-4. MJ BRONCHOSCOPY. CYSTIC-FIBROSIS: co. IRRIGATION. LUNG. RESPIRATORY-INSUFFICIENCY: th. MN ADOLESCENCE. ADULT. CHILD. FEMALE. IN-VITRO. MALE. RESPIRATORY-INSUFFICIENCY: et. EX This study compared the use of the fiberoptic bronchoscope with other lavage techniques under general anesthesia, and of techniques using the rigid bronchoscope. Seventy-seven procedures were performed on 39 patients with cystic fibrosis. Their vital capacities were improved in 46 of the 75 valid measurements. Arterial blood gas levels showed improvements in 14 of the 33 values obtained. A prospective study is needed which incorporates the values of pre- and postoperative MMEV, RV/TLC, flow volume curves and arterial blood gas values. An evaluation of the complications and ease of operation may be of greater value. RF 001 DOERSHUK CF PEDIATRICS 36 675 965 002 KLINE BS J EXP MED 21 311 915 003 WINTERNITZ MC IN: CONTRIB MED BIOL RES 1255 919 004 VICENTE G REV PROGR DE LA CLINICA 928 005 THOMPSON HT LANCET 2 8 964 006 LEFEMINE AA ANN THORAC SURG 4 308 967 007 HACKETT RR ANESTHESIOLOGY 26 248 965 008 RAMIREZ RJ ANN INTERN MED 63 819 965 009 RAMIREZ RJ DIS CHEST 50 581 966 010 CEZEAUX G JR JAMA 199 15 967 011 KYLSTRA JA AM REV RESPIR DIS 103 651 971 012 ALTMAN RP J PEDIATR SURG 8 809 973 013 MILLIS RM CHEST 71 508 977 014 SACKNER MA AM REV RESPIR DIS 111 62 975 015 SACKNER MA CHEST 62 705 972 016$ DAHM LA CHEST 12 593 977 017 HOLSCLAW DS J PEDIATR 76 829 970 018 ALBERTINI RE JAMA 230 1666 974 019 BARRETT CR JR AM REV RESPIR DIS 109 429 974 020 KARETZKY MS NY STATE J MED 74 62 974 021 LOBER CW CHEST 68 382 975 022 BRAUNSTEIN MS CHEST 66 96 974 023 BEIER FR AM REV RESPIR DIS 94 430 966 CT 1 EMSLANDER HP INTERNIST 21 11 980 2 BURNS DM AM REV RESPIR DIS 127 695 983 3 SHERMAN JM PEDIATR PULMONOL 2 244 986 PN 78063 RN 00844 AN 78147499 AU Quick-C-A. Warwick-W. TI Bronchoscopy and lavage in management of pulmonary complications of cystic fibrosis. SO Chest. 1978 May. 73(5 Suppl). P 755-8. MJ BRONCHOSCOPY. CYSTIC-FIBROSIS: co. IRRIGATION. LUNG-DISEASES: th. LUNG. MN ADOLESCENCE. CASE-REPORT. FEMALE. HUMAN. LUNG-DISEASES: et. EX This article presents our experience with bronchoscopy as a therapeutic and diagnostic tool in the management of patients with cystic fibrosis. The results from therapeutic bronchoscopy and bronchial irrigation in cystic fibrosis patients have been quite impressive. Levels of blood gas after the procedure have generally shown improvement. The vital capacity and FEF1 in general have shown small but consistent improvements. The most dramatic effect has been the patient's own assessment of the operation. These patients state that their breathing is less labored in that it is less rapid and much deeper. Bronchoscopy offers an effective therapeutic tool in the management of pulmonary complications of cystic fibrosis. Unfortunately, the benefit is short-lived and repeated bronchoscopic procedures are often necessary. It must be accepted that there is a point in time when the cardiopulmonary state of a patient's cystic fibrosis may have progressed beyond salvageable limits. It is our conviction that earlier bronchoscopy and thorough bronchial irrigation may prevent the critical pulmonary event which is followed inevitably by progressive deterioration of the pulmonary status of patients with cystic fibrosis. CT 1 EMSLANDER HP INTERNIST 21 11 980 2 ROTHMANN BF ANN OTOL RHINOL LARYNGOL 91 641 982 3 FITZPATRICK SB AM J DIS CHILD 137 595 983 4 MOAZAM F J PEDIATR SURG 20 398 985 5 SHERMAN JM PEDIATR PULMONOL 2 244 986 PN 78064 RN 00845 AN 79002962 AU Mikkelsen-C. Waechter-E. Crittenden-M. TI Cystic fibrosis: a family challenge. SO Child-Today. 1978 Jul-Aug. 7(4). P 22-6. MJ CYSTIC-FIBROSIS: px. FAMILY. MN CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. MALE. EX To learn how families cope with cystic fibrosis, we interviewed 18 families who gave us vivid descriptions of the day-to-day process of living with this devastating disease. The families and the disease are described. Pre-diagnosis and diagnosis, home management and treatment, family communication about CF, the reactions of siblings, relationships with peers, hospitalization, and sources of support are discussed. RF 002 WAECHTER E IN: GODIN A 972 003 WOOD RE AM REV RESPIR DIS 113 833 976 005 MCCOLLUM AT J PEDIATR 77 571 970 CT 1 DEWET B S AFR MED J 65 526 984 2 DEWET B S AFR MED J 67 292 985 PN 78065 RN 00846 AN 79044102 AU Warwick-W-J. Hansen-L. TI Measurement of chloride in sweat with the chloride-selective electrode. SO Clin-Chem. 1978 Nov. 24(11). P 2050-3. MJ CHLORIDES: an. SWEAT: an. MN CYSTIC-FIBROSIS: di. ELECTRODES. HUMAN. INFANT-NEWBORN. METHODS. PILOCARPINE: du. EX We describe here an improved method for the sweat test with the chloride-selective electrode, after sweat production is stimulated with pilocarpine, which gives results comparable to the more tedious procedure of Gibson and Cooke. Cystic fibrosis should never be diagnosed solely on the results of any kind of sweat test; the diagnosis must be consistent with the results of the physical examination, history, and other studies, including a repeat of the two sweat tests at another time. Only by clinical examination and repeated sweat tests can a physician confirm or rule out this diagnosis. RF 001 SHWACHMAN H PEDIATRICS 30 167 962 002 GIBSON LE PEDIATRICS 23 545 959 003 GIBSON LE CLIN PEDIATR 12 450 973 004 HANSEN L MINN MED 50 1191 967 005 WARWICK WJ CF CLUB ABST 5 975 006 WARWICK WJ PEDIATRICS 36 261 965 007 HANSEN L AM J CLIN PATHOL 49 834 968 008 KOPITO L PEDIATRICS 43 794 969 009 SZABO L CLIN CHEM 19 727 973 010 GURSON CT HELV PAEDIATR ACTA 28 165 973 011 STEINRUD J DAN MED BULL 21 251 974 012 WARWICK WJ JAMA 198 59 966 013 CHERIAN AG CLIN CHEM 17 652 971 014 BRAY PT CLIN CHIM ACTA 77 69 977 015 WARWICK WJ CLIN CHEM 24 381 978 016 BRAY PT CLIN CHIM ACTA 80 333 977 017 ANON J PEDIATR 88 711 976 018 TOCCI PM CLIN CHEM 22 1841 976 019 MACLEAN WC JR J PEDIATR 83 86 973 020 VLACHOS P J PEDIATR 84 926 974 021$ WARWICK WJ CF CLUB ABST 12 21 974 CT 1 WEBSTER HL CLIN CHEM 27 385 981 2 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 3 COURY AJ CLIN CHEM 29 1593 983 4 MILLER ME CLIN CHEM 31 1715 985 PN 78066 RN 00847 AN 78213515 AU Guy-G-J. Butterworth-J. TI Carboxypeptidase A activity of cultured skin fibroblasts and relationship to cystic fibrosis. SO Clin-Chim-Acta. 1978 Jul 1. 87(1). P 63-9. MJ CARBOXYPEPTIDASES: me. CYSTIC-FIBROSIS: en. SKIN: en. MN CARBOXYPEPTIDASES: ai. CELLS-CULTURED. ENZYME-ACTIVATION: de. FIBROBLASTS: en. HUMAN. KINETICS. LYSOSOMES: en. AB Properties of carboxypeptidase A of cultured skin fibroblasts from control and cystic fibrosis patients were studied using alpha-N- carbobenzoxy-L-glutamyl-L-tyrosine as substrate. Carboxypeptidase A was inhibited by thiomersal, cyanide, iodoacetate and N- ethylmaleimide in a similar manner for control and cystic fibrosis fibroblasts. Both trypsin and dithiothreitol treatment activated the enzyme, but 1,10-phenanthroline inhibited only in the presence of dithiothreitol. Both Zn2+ and Co2+ reversed this inhibition. Trypsin treatment of carboxypeptidase A produced a form of the enzyme having a higher KM value for both control and cystic fibrosis fibroblasts. Dithiothreitol treatment of control fibroblasts resulted in a form with similar properties to the trypsin activated form, but cystic fibrosis fibroblasts yielded a variant form with even higher KM and Vmax values. Since other properties were similar, it seems likely that this difference reflected binding of a molecule to the enzyme rather than of a defect in the enzyme. RF 001 BUTTERWORTH J CLIN CHIM ACTA 44 295 973 002 BUTTERWORTH J CLIN GENET 9 505 976 003 BUTTERWORTH J CLIN GENET 12 297 977 004 LOWRY OH J BIOL CHEM 193 265 951 005 TAYLOR SL BIOCHIM BIOPHYS ACTA 341 99 974 006 ROTH M ANAL CHEM 43 880 971 007 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 008 RAO GJS PEDIATR RES 9 739 975 009 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 010 RAO GJS J PEDIATR 80 573 972 011 BERGSTROM K SCIENCE TOOLS 24 8 977 012 BARRETT AJ BIOCHEM J 133 709 973 013 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 CT 1 BUTTERWORTH J CLIN CHIM ACTA 97 39 979 2 BUTTERWORTH J ANAL BIOCHEM 106 156 980 3 BUTTERWORTH J CLIN CHIM ACTA 108 143 980 4 BUTTERWORTH J J INHERIT METAB DIS 5 9 982 5 BUTTERWORTH J CLIN CHIM ACTA 122 51 982 PN 78067 RN 00848 AN 78189501 AU Davis-P-B. TI Urinary cyclic nucleotides in adult male cystic fibrosis patients. SO Clin-Chim-Acta. 1978 Jul 15. 87(2). P 285-8. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: ur. CYSTIC-FIBROSIS: ur. GUANOSINE-CYCLIC-MONOPHOSPHATE: ur. MN ADULT. BODY-SURFACE-AREA. CREATININE: ur. HUMAN. MALE. MIDDLE-AGE. REFERENCE-VALUES. AB Urinary adenosine 3':5' (cyclic) monophosphate and guanosine 3':5' (cyclic)-monophosphate excretion are significantly elevated in adult male cystic fibrosis patients compared to normal men when values are expressed in ways which consider the smaller size of the cystic fibrosis subjects of the same age. Cystic fibrosis patients excreted 3.22 +/- 1.16 nmol adenosine 3':5' (cyclic)monophosphate/g creatinine per 24 h vs. 1.97 +/- 0.43 for normal men (p less than 0.02); and cystic fibrosis patients excreted 0.50 +/- 0.16 nmol guanosine 3':k' (cyclic)monophosphate/g creatinine per 24 h vs. 0.30 +/- 0.07 for normal men (p less than 0.05). Subjects were all adults who had completed linear growth and sexual development, thus eliminating any possible effects of slower maturation of cystic fibrosis patients, and all subjects were male, thus avoiding the fluctuation of urinary cyclic nucleotides with the menstrual cycle, problems which had complicated interpretation of previous studies. RF 001 SIMOPOULOS AP PROC AM PEDIATR SOC ANNU MTG 201 971 002 MURAD F J CLIN ENDOCRINOL METAB 40 552 975 003 TAYLOR AL J CLIN ENDOCRINOL METAB 30 316 970 004 HAMADAH K BIOCHEM SOC TRANS 2 461 974 005 TAUSSIG LM J PEDIATR 82 380 973 006 BROWN BL BIOCHEM J 121 561 971 007$ STEINER AL ADV CYCLIC NUCLEO RES 3 89 972 008 MURAD F ADV CYCLIC NUCLEO RES 3 355 973 009 MURAD F N ENGL J MED 286 1382 972 010 STAHL M J PEDIATR 84 821 974 CT 1 AURBACH GD ADV CYCLIC NUCLEO RES 12 1 980 2 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 3 HUNT NH CLIN SCI 58 463 980 4 EBSTEIN RP ISR J MED SCI 21 302 985 PN 78068 RN 00849 AN 78082699 AU Schuttringer-G. Zinterhofer-L. TI Automated immunoprecipitation of meconium albumin for cystic fibrosis screening in the newborn. SO Clin-Chim-Acta. 1978 Feb 1. 83(1-2). P 109-16. MJ ALBUMINS: an. CYSTIC-FIBROSIS: di. MECONIUM: an. MN CYSTIC-FIBROSIS: pc. FALSE-POSITIVE-REACTIONS. HUMAN. INFANT-NEWBORN. MASS-SCREENING. PRECIPITIN-TESTS: is, mt. AB Cystic fibrosis is the most frequent substantially lethal inherited disease in the United States. Newborn screening for cystic fibrosis has been suggested because early diagnosis permits genetic counselling of parents and improved treatment and prognosis for cystic fibrosis patients. The fact that cystic fibrosis newborns have abnormally high meconium albumin seems to offer the best screening approach. Because we have had equivocal and false positive results with meconium albumin test strips, we have developed a nephelometric automated immunoprecipitation method for the quantitative and specific analysis of meconium albumin. On a total of 3895 meconiums so far examined, eleven albumin elevations of undetermined origin have been detected. Nine of these were associated with prematurity. There have been, so far, no detected true positives and no known false negatives. The normal range for meconium albumin is in agreement with previously published ranges determined using manual immunochemical methods. This method is rapid and economic. The question of sensitivity, specificity and predictive value of this and other detection methods is discussed. RF 001 DI SANTAGNESE PA IN: DOWNEY JA 25 974 002 THOMPSON JS GENETICS IN MEDICINE 58 973 003 DI SANTAGNESE PA N ENGL J MED 277 1287 967 004 GEORGE L ARCH DIS CHILD 46 139 971 005 SHWACHMAN H PEDIATRICS 46 335 970 006 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 007 BUCHANAN DJ PEDIATRICS 9 304 952 008 GREEN MN PEDIATRICS 21 635 958 009 WISER WC PEDIATRICS 33 115 964 010 GREEN MN PEDIATRICS 41 989 968 011 SCHUTT WH ARCH DIS CHILD 43 178 968 012 CAIN ARR ARCH DIS CHILD 47 131 972 013 PROSSER R ARCH DIS CHILD 49 597 974 014 STEPHAN U PEDIATRICS 55 35 975 015 BULL FE ARCH DIS CHILD 49 602 974 016 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 017 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 018 ANON MEDICAL LETTER ON DRU 18 35 967 019 HARDY JD ARCH DIS CHILD 48 316 973 020 GALEN RS BEYOND NORMALITY THE PREDICTI 975 021 KINGSBURY FB J LAB CLIN MED 11 981 926 CT 1 RYLEY HC ARCH DIS CHILD 54 92 979 2 MASTELLA G RIV ITAL PEDIATR 7 581 981 3 FORREST DC ARCH DIS CHILD 56 151 981 4 DODGE JA ARCH DIS CHILD 57 774 982 5 NAYLOR EW SEM PERINATOL 9 232 985 PN 78069 RN 00850 AN 78063089 AU Shapiro-B-L. Feigal-R-J. Laible-N-J. Biros-M-H. Warwick-W-J. TI Doubling time alpha-aminoisobutyrate transport and calcium exchange in cultured fibroblasts from cystic fibrosis and control subjects. SO Clin-Chim-Acta. 1978 Jan 2. 82(1-2). P 125-31. MJ AMINOISOBUTYRIC-ACIDS: me. CALCIUM: me. CYSTIC-FIBROSIS: me. MN BIOLOGICAL-TRANSPORT. BIOLOGICAL-TRANSPORT-ACTIVE. CELL-DIVISION. FEMALE. FIBROBLASTS: me. HUMAN. KINETICS. MALE. SUPPORT-U-S-GOVT-P-H-S. AB Population doubling time, kinetics of transport of alpha- aminoisobutyrate (AIB) and calcium (Ca) exchange were studied in skin fibroblast monolayers obtained from 5 subjects with cystic fibrosis (CF) and 5 age- and sex-matched controls. Population doubling time as estimated from cell count, protein and DNA was no different in the two groups. KM, Vmax, maximal uptake and time of half maximal uptake of AIB were no different in the two groups. Intracellular Ca pool size based on exchange of 45Ca with unlabelled Ca was significantly greater in monolayers from CF subjects. RF 001 OYAMA VI PROC SOC EXP BIOL MED 91 305 956 002 SWITZER BR CLIN CHIM ACTA 32 203 971 003 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 004 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 005 BOLTON WE AM J HUM GENET 27 394 975 006 FLETCHER DS CLIN CHIM ACTA 44 5 973 007 BAUR PS TEX REP BIOL MED 34 113 976 008 MANGOS JA PEDIATR RES ABST 10 357 976 CT 1 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 2 FEIGAL RJ PEDIATR RES 13 764 979 3 LAM LFH LIFE SCI 24 2483 979 4 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 5 SWENEY LR J MICROSC 115 151 979 6 SHAPIRO BL SCIENCE 203 1251 979 7 FEIGAL RJ NATURE 278 276 979 8 OWEN K CANCER RES 40 3167 980 9 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 10 GNEGY ME BIOCHEM MED 26 294 981 11 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 12 SORSCHER EJ LANCET 1 368 982 13 FEIGAL RJ LIFE SCI 30 93 982 14 SHAPIRO BL AM J HUM GENET 34 846 982 15 SHAPIRO BL SCIENCE 216 417 982 16 FONDACARO JD LIFE SCI 32 1449 983 17 HARRIS A CLIN CHIM ACTA 128 41 983 18 WALLER RL LIFE SCI 35 775 984 19 WALLER RL CELL CALC 6 245 985 20 RUPP GM MED HYPOTHESES 20 245 986 21 MORRISSEY SM DIGESTION 34 28 986 22 FEIGAL RJ ANN NY ACAD SCI 488 82 986 PN 78070 RN 00851 AN 78063085 AU Brock-D-J. Barron-L. Manson-J. McCrae-W-M. TI Serum alphafetoprotein in cystic fibrosis of the pancreas. SO Clin-Chim-Acta. 1978 Jan 2. 82(1-2). P 101-3. MJ ALPHA-FETOPROTEINS: me. CYSTIC-FIBROSIS: bl. MN HETEROZYGOTE. HUMAN. RADIOIMMUNOASSAY. AB Serum alphafetoprotein concentrations were measured by three different types of radioimmunoassay in 30 patients with cystic fibrosis of the pancreas and in 55 controls. The highest value obtained in cystic patient was 10.2 ng/ml and in a control 10.8 ng/ml. These are within published normal limits. Previously reported large increases in serum AFP in patients with cystic fibrosis and in heterozygote carriers have not been confirmed. RF 001 CHANDRA RK BR MED J 1 714 975 002 BROCK DJH BR MED J 2 392 975 003 WALLWORK JC BR MED J 2 392 975 004 FITZSIMMONS JS BR MED J 3 544 975 005 BELANGER L BR MED J 4 759 975 006 BROCK DJH CLIN CHIM ACTA 57 315 974 007 BROCK DJH LANCET 1 745 975 008 VINCE JD BR J OBSTET GYNAECOL 82 718 975 009 FORRESTER PI CLIN CHIM ACTA 64 317 975 010 HUNTER WM IN: WEIR DM 973 CT 1 GOSDEN C BR MED J 2 1186 978 2 GOSDEN C J MED GENET 15 262 978 3 BROCK DJH BR J OBSTET GYNAECOL 85 575 978 4 RODECK CH LANCET 1 949 980 5 BROCK DJH BR J OBSTET GYNAECOL 87 582 980 6 BROCK DJH BR J OBSTET GYNAECOL 89 348 982 PN 78071 RN 00852 AN 79044172 AU Kenny-D. Cooke-A. Tempany-E. McGeeney-K-F. TI Activity of serum alpha-amylases in cystic fibrosis. SO Clin-Chim-Acta. 1978 Nov 1. 89(3). P 429-33. MJ ALPHA-AMYLASE: bl. AMYLASES: bl. CYSTIC-FIBROSIS: en. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. HUMAN. MATHEMATICS. PANCREAS: en. SALIVA: en. AB A new method which uses a differential inhibitor to measure pancreatic and salivary type alpha-amylases (EC 3.2.1.1) was applied to serum samples from 46 cystic fibrosis (CF) patients (age range 4- 14 years) and 50 controls of the same age group. The levels of pancreatic type amylase were lower in the CF patients (median 26.5 I.U./1) than the controls (median 81.5) (P less than 0.001). The results for salivary-type enzyme, however, did not support the previously reported finding of higher than usual levels in CF patients. This discrepancy is probably due to differences in analytical methods. It is felt that this procedure will be of value in the investigation of patients for cystic fibrosis and other pancreatic disorders. RF 001 BURTON P GUT 1 111 960 002 LUNDH G GASTROENTEROLOGY 42 275 962 003 SKUDE G SCAND J GASTROENTEROL 10 577 975 004 OTSUKI M CLIN CHIM ACTA 79 1 977 005 TAKEUCHI T CLIN CHIM ACTA 60 207 975 006 BERK JE AM J GASTROENTEROL 63 457 975 007 ODONNELL MD BIOCHIM BIOPHYS ACTA 422 159 976 008 ODONNELL MD CLIN CHEM 23 560 977 009 CONOVER WG PRACTICAL NONPARAMETRIC STATI 971 010 SKUDE G ACTA PAEDIATR SCAND 65 145 976 CT 1 ODONNELL MD CLIN CHIM ACTA 104 265 980 2 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 3 TOBIN MJ IR J MED SCI 150 325 981 4 BROWN RC J CLIN PATHOL 35 547 982 5 MORGENSTERN T MONATSSCHR KINDERHEILKD 132 661 984 6 GILLARD BK AM J DIS CHILD 138 577 984 7 MOLLERPETERSEN J CLIN CHIM ACTA 157 151 986 PN 78072 RN 00853 AN 79044177 AU Alhadeff-J-A. TI Lysosomal hydrolases in cystic fibrosis livers. SO Clin-Chim-Acta. 1978 Nov 1. 89(3). P 469-73. MJ CYSTIC-FIBROSIS: en. HYDROLASES: me. LIVER: en. LYSOSOMES: en. MN ACETYLGLUCOSAMINIDASE: me. HUMAN. IN-VITRO. MANNOSIDASES: me. NEURAMINIDASE: me. SUPPORT-U-S-GOVT-P-H-S. AB The specific activity of several lysosomal hydrolases in normal and cystic fibrosis (CF) livers has been investigated. Neuraminidase activity with respect to two natural substrates (fetuin and bovine submaxillary mucin) is normal in CF livers. No significant differences were found between CF and normal livers in 4- methylumbelliferyl-alpha-D-mannosidase specific activities determined at three different pH values (4.3, 5.65 and 6.5) corresponding to lysosomal, golgi and cytosolic components of the enzyme, respectively. The specific activities of p-nitrophenyl-beta-D- mannosidase and 4-methylumbelliferyl-beta-N-acetylglucosaminidase were also both similar in CF and normal livers. RF 001 NADLER HL IN: STANBURY JB 1683 978 002 MCCOMBS ML TEX REP BIOL MED 31 615 973 003 WRIGHT SW AM J HUM GENET 20 157 968 004 ALHADEFF JA CLIN GENET 13 417 978 005 OPHEIM DJ J BIOL CHEM 253 1017 978 006 PHILLIPS NC CLIN CHIM ACTA 55 11 974 007 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 008 WILSON RG CLIN CHIM ACTA 36 113 972 009 CALLAHAN JW PEDIATR RES 11 1166 977 010 LOWRY OH J BIOL CHEM 193 265 951 011 WARREN L J BIOL CHEM 234 1971 959 012 PHILLIPS N BIOCHEM J 151 469 975 013 OKADA S AM J HUM GENET 23 55 971 014 TULSIANI DRP J BIOL CHEM 245 1821 970 015 ALHADEFF JA CLIN GENET 10 63 976 016 ALHADEFF JA MOL CELL BIOCHEM 18 33 977 017 SPIRO RG ADV PROT CHEM 27 349 973 018 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 019 ANTONOWICZ I PEDIATR RES 6 803 972 CT 1 MINAMI R CLIN CHIM ACTA 96 107 979 2 MEYER DM BIOCHEM BIOPHYS RES COMMUN 103 1302 981 3 ALHADEFF JA BIOCHEM J 201 95 982 4 DENTANDT WR BIOCHEM MED 31 287 984 5 HULTCRANTZ R HEPATOLOGY 6 881 986 6 PORTER WH CLIN CHEM 32 652 986 PN 78073 RN 00854 AN 78105815 AU Alhadeff-J-A. Cimino-G. TI Cystic fibrosis liver sialyltransferase. SO Clin-Genet. 1978 Feb. 13(2). P 207-12. MJ CYSTIC-FIBROSIS: en. LIVER: en. SIALYLTRANSFERASES: me. TRANSFERASES: me. MN HUMAN. HYDROGEN-ION-CONCENTRATION. ISOELECTRIC-FOCUSING. LIVER-DISEASES: en. TEMPERATURE. AB The activity of sialytransferase with regard to the glycoprotein substrates asialofetuin and asialo-ovine submaxillary mucin was determined in normal, pathological control, and cystic fibrosis liver homogenates. Cystic fibrosis and pathological livers have about 40% of the average normal specific activity for sialytransferase. Several properties of cystic fibrosis sialytransferase were investigated and compared to those of the normal liver enzyme (Alhadeff et al. 1977). The pH optima curves were similar, but cystic fibrosis sialyltransferase appears to be more thermolabile than the normal liver enzyme. Isoelectric focusing studies revealed that the three most basic forms of sialyltransferase which are found in normal livers are deficient or absent in most cystic fibrosis liver. The data suggest that altered glycoprotein-sialyltransferases may be present in cystic fibrosis livers, probably a secondary effect due to general liver pathology. RF 001 ALHADEFF JA BIOCHIM BIOPHYS ACTA 484 307 977 002 ALHADEFF JA CLIN CHIM ACTA 57 307 974 003 ALHADEFF JA DEVELOP BIOL 47 319 975 004 ALHADEFF JA CLIN GENET 10 63 976 005 GOLDSTONE AP FEBS LETTERS 13 68 971 006 KATTWINKEL J J PEDIATR 82 234 973 007 LOBECK CC IN: STANBURY JB 1605 972 008 LOWRY OH J BIOL CHEM 193 265 951 009 MCCOMBS ML TEX REP BIOL MED 31 615 973 010 MOOKERJEA S CAN J BIOCHEM 50 738 972 011 MURPHY WH BIOCHIM BIOPHYS ACTA 52 349 961 012 SCHACHTER H IN: FISHMAN WH 3 2 973 013 SPIRO RG J BIOL CHEM 235 2860 960 014 SPIRO RG ADV PROT CHEM 27 349 973 015 TETTAMANTI G ARCH BIOCHEM BIOPHYS 124 41 968 016 THORPE R FEBS LETTERS 54 89 975 017 WARREN L J BIOL CHEM 234 1971 959 CT 1 ALHADEFF JA CLIN GENET 13 417 978 2 ALHADEFF JA CLIN GENET 14 189 978 3 LAPINA EB BIOORGAN KHIM 5 1720 979 4 ANFIMOVA ML BIOORGAN KHIM 7 1209 981 5 HULTBERG B CLIN CHIM ACTA 112 167 981 6 MARGOLIES R PEDIATR RES 17 931 983 7 PASCALI VL EUR J PEDIATR 143 133 984 PN 78074 RN 00855 AN 79002464 AU Bois-E. Feingold-J. Demenais-F. Runavot-Y. Jehanne-M. Toudic-L. TI Cluster of cystic fibrosis cases in a limited area of Brittany (France). SO Clin-Genet. 1978 Aug. 14(2). P 73-76. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: oc. FEMALE. FRANCE. GENE-FREQUENCY. HUMAN. MALE. PEDIGREE. AB Cystic fibrosis in the northern sector of the French "departement" of Finistere is 1:1787 live births. Within this sector a concentration of the disease was found in a small area. The minimal frequency in this area, from 1946 to 1972, was calculated as 1 per 377 live births, the gene frequency being 0.0515. Genealogic analysis, going back to the beginning of the 18th century, showed a relationship between 8 of the 10 families to which the patients belonged. The origin of the deleterious genes may be explained by a least five primary ancestral couples living in the 18th century. Random drift is the most probable explanation for the concentration of cystic fibrosis in this region. RF 001 ANDERSON CM MOD PROBL PEDIATR 10 381 967 002 CONNEALLY PM TEX REP BIOL MED 31 639 973 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 FEINGOLD J ANN GENET (PARIS) 17 257 974 005 GOURVIL F NOMS DE FAMILLE BRETONS D- 970 006 HIRSCHHORN K IN: MANGOS JA 275 976 007 KNUDSON AG JR AM J HUM GENET 19 388 967 008 RAO DC AM J HUM GENET 25 594 973 009 RUNAVOT Y THESIS 975 010 TEN KATE LP INT J EPIDEMIOL 6 23 977 011 WAGENER DK AM J HUM GENET 27 348 975 CT 1 COX DW PEDIATR CLIN NORTH AM 26 467 979 2 DEMENAIS F REV EPIDEMIOL SANTE PUBLIQUE 27 5 979 3 KLINGER KW HUM GENET 65 94 983 4 PASCALETGUIDON MJ CLIN GENET 26 39 984 5 KLINGER KW SEM RESPIR MED 6 243 985 6 LIVINGSTONE FB HUM BIOL 59 59 987 PN 78075 RN 00856 AN 79023672 AU Alhadeff-J-A. TI Glycoproteins and cystic fibrosis: a review. SO Clin-Genet. 1978 Oct. 14(4). P 189-201. (REVIEW). MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: me. MN CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en. FUCOSE: me. FUCOSIDASE: me. GLYCOPROTEINS: ip. HUMAN. REVIEW. SUPPORT-U-S-GOVT-P-H-S. EX This article is a review of the major research of the last 20 years on abnormal glycoproteins and abnormal glycoprotein metabolism in cystic fibrosis. Its purpose is to focus attention on an area considered by the author to be of great importance for elucidating the primary biochemical defect in CF. The majority of the studies on glycoproteins in CF have been done on crude mixtures of glycoproteins from tissues or tissue fluids, secretions or excretions. Very few studies have compared the properties and composition of purified glycoproteins from CF patients and controls. Comparative studies on the enzymes involved in the degradation and biosynthesis of the carbohydrate portions of glycoproteins in CF patients and normal controls are discussed. Several studies have suggested abnormalities in CF alpha-L-fucosidase and CF L-fucose metabolism. There are several studies which indirectly suggest that abnormalities in glycoproteins or their metabolism may be involved in the pathogenesis of cystic fibrosis. Fruitful areas for future investigation are comparison of the properties and composition of specific glycoproteins purified to homogeneity from CF and control tissues, particularly with regard to carbohydrate composition; comparison of the activities and properties of the enzymes involved in both the degradation and biosynthesis of glycoproteins in CF and control tissues; and studies of the effects of increased electrolyte concentration on the physical-chemical properties of glycoproteins. RF 001 ALHADEFF JA CLIN GENET 13 207 978 002 ALHADEFF JA BIOCHIM BIOPHYS ACTA 484 307 977 003 ALHADEFF JA MOL CELL BIOCHEM 18 33 977 004 ALHADEFF JA J BIOL CHEM 250 7106 975 005 ALHADEFF JA CLIN GENET 10 63 976 006 ALHADEFF JA DEVELOP BIOL 47 319 975 007 ALHADEFF JA CLIN GENET 13 417 978 008 ANDERSEN DH AM J DIS CHILD 56 344 938 009 ANTONOWICZ I PEDIATR RES 6 803 972 010 ASHWELL G ADV ENZYM MOLEC BIOL 41 99 974 011 BAKER AP FED PROC 33 1300 974 012 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 013 BAUER U ANN PAEDIATR 194 236 960 014 BESLEY GTN J MED GENET 6 278 969 015 BISWAS S CLIN CHIM ACTA 69 541 976 016 BLACKFAN KD J PEDIATR 13 627 938 017 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 018 BOAT TF AM REV RESPIR DIS 110 428 974 019 BOAT TF PEDIATR RES 8 531 974 020 BOWMAN BH SCIENCE 164 325 969 021 BROCK DJH BR MED J 2 392 975 022 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 023 BUTTERWORTH J CLIN CHIM ACTA 41 367 972 024 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 025 CHANDRA RK BR MED J 1 714 975 026 COHEN LF BLOOD 48 469 976 027 CROSBY P HISTOCHEM J 3 223 971 028 DAVIS PB CF CLUB ABST 7 976 029 DI SANTAGNESE PA PEDIATRICS 12 549 953 030 DISCHE Z PEDIATRICS 24 74 959 031 DISCHE Z ANN NY ACAD SCI 93 526 962 032 DISCHE Z AM J DIS CHILD 102 733 961 033 FILLIAT M PATHOL BIOL (PARIS) 21 13 973 034 FITZSIMMONS JS BR MED J 3 544 975 035 FORSTNER JF PEDIATR RES 10 609 976 036 FORSTNER JF CF CLUB ABST 10 976 037 FRIEDMANN T J LAB CLIN MED 70 404 967 038 GIBSON LE PEDIATRICS 23 545 959 039 GIBSON LE PEDIATRICS 48 695 971 040 GOLDSTONE AP LIFE SCI 9 1341 970 041 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 042 GUGLER EC J PEDIATR 71 585 967 043 GUHA AK CLIN BIOCHEM 10 153 977 044 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 045 KIM YS J CLIN INVEST 51 2033 972 046 KNAUFF RE CLIN CHIM ACTA 19 245 968 047 KNOPFLE G BR MED J 4 459 975 048 KOLLBERG H ACTA PAEDIATR SCAND 62 279 973 049 KOPITO L NATURE 202 501 964 050 KRAUS I PEDIATRICS 47 1010 971 051 KUTTY KM CF CLUB ABST 45 976 052 LOBECK CC IN: STANBURY JB 1605 972 053 LOUISOT P CLIN CHIM ACTA 48 373 973 054 LUNDGREN DW CLIN CHIM ACTA 62 357 975 055 MANDEL ID AM J DIS CHILD 113 431 967 056 MANDEL ID AM J DIS CHILD 110 646 965 057 MARCHI AG HELV PAEDIATR ACTA 28 427 973 058 MAXFIELD M J CLIN INVEST 41 455 962 059 MCCOMBS ML TEX REP BIOL MED 31 615 973 060 MCEVOY FA PEDIATR RES 9 721 975 061 MCNEELY MC AM J HUM GENET 29 73A 977 062 MOOKERJEA S CAN J BIOCHEM 50 738 972 063 NADLER HL IN: STANBURY JB 1683 978 064 NEUTRA MR LAB INVEST 36 535 977 065 NOVAK RA TEX REP BIOL MED 34 199 976 066 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 067 PAPP Z CLIN GENET 11 431 977 068 PEARSON RD PEDIATR RES 11 462 977 069 POTTER JL ANN NY ACAD SCI 106 692 963 070 RAO GJS CLIN RES 24 436A 976 071 ROELFS RE AM J DIS CHILD 113 419 967 072 RUSSELL SB J MED GENET 8 441 971 073 SCANLIN TF JR PEDIATR RES ABST 549 11 463 977 074 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 075 SCHACHTER H BIOCHEM SOC SYMP 40 57 974 076 SCHACHTER H IN: FISHMAN WH 3 2 973 077 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 078 SCHWARTZ RH J LAB CLIN MED 70 725 967 079 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 080 SHAPIRA E J BIOL CHEM 252 7923 977 081 SHAPIRA E PEDIATR RES 10 812 976 082 SHARON N COMPLEX CARBOHYDRATES THEIR C 975 083 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 084 SINGER L CLIN BIOCHEM 7 146 974 085 SMITH JA BR MED J 2 392 975 086 SPIRO RG ADV PROT CHEM 27 349 973 087 SPOCK A PEDIATR RES 1 173 967 088 STEVENSON FK CLIN CHIM ACTA 23 441 969 089 TALAMO RC J PEDIATR 65 480 964 090 TOUSTER O MOL CELL BIOCHEM 2 169 973 091 WALLWORK JC BR MED J 2 392 975 092 WELCH DW PEDIATR RES 9 698 975 093 WILSON GB SCAND J IMMUNOL 5 829 976 094 WILSON GB PEDIATR RES 10 87 976 095 WILSON RG CLIN CHIM ACTA 36 113 972 096 WRIGHT SW AM J HUM GENET 20 157 968 097 WU JT PEDIATR RES 10 235 976 CT 1 SHIER WT MED HYPOTHESES 5 661 979 2 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 3 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 4 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 5 MALER T FEBS LETTERS 121 153 980 6 HALLINAN F MED HYPOTHESES 7 793 981 7 BENYOSEPH Y PEDIATR RES 15 839 981 8 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 9 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 10 BLITZER MG PEDIATR RES 16 203 982 11 BLITZER MG PEDIATR RES 16 938 982 12 MCNEELY MC PEDIATR RES 16 21 982 13 LITIN BS BIOL NEONATE 42 228 982 14 SNYDER CE CARBOHYD RES 105 87 982 15 LONGNECKER DS AM J PATHOL 107 103 982 16 BENYOSEPH Y BIOCHIM BIOPHYS ACTA 718 172 982 17 FRATES RC PEDIATR RES 17 30 983 18 SLOMIANY A J BIOL CHEM 258 8535 983 19 KARLSSON S J MED GENET 21 441 984 20 HART DA THROMB HAEMOST 51 212 984 21 GUEANT JL CLIN CHIM ACTA 143 217 984 22 PASCALI VL EUR J PEDIATR 143 133 984 23 PORTER WH CLIN CHEM 32 652 986 PN 78076 RN 00857 AN 78189549 AU Alhadeff-J-A. Watkins-P. Freeze-H. TI Purification and characterization of altered cystic fibrosis liver alpha-L-fucosidase. SO Clin-Genet. 1978 May. 13(5). P 417-24. MJ CYSTIC-FIBROSIS: en. FUCOSIDASE: ip. LIVER: en. MN CARBOHYDRATES: an. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FUCOSIDASE: an, me. HUMAN. ISOELECTRIC-FOCUSING. SUPPORT-U-S-GOVT-P-H-S. AB alpha-L-Fucosidase (E.C.3.2.4.51) from two cystic fibrosis livers has been purified and characterized. Purification was accomplished by an affinity chromatographic procedure previously used for normal liver alpha-L-fucosidase. Characterization of the two cystic fibrosis alpha- L-fucosidases indicated that they were very similar to normal liver alpha-L-fucosidase with regard to pH optima profiles, Michaelis constants (Km's), subunit structure and antigenicity. However, gas liquid chromatographic analysis revealed altered carbohydrate compositions for both the cystic fibrosis alpha-L-fucosidases. The three major sugars found in normal purified liver alpha-L-fucosidase (mannose, N-acetylglucosamine and sialic acid) were reduced in the cystic fibrosis alpha-L-fucosidases, on average, to 51%, 44% and 32%, respectively, of their normal amounts. RF 001 ALHADEFF JA CLIN GENET 13 207 978 002 ALHADEFF JA MOL CELL BIOCHEM 18 33 977 003 ALHADEFF JA J BIOL CHEM 250 7106 975 004 ALHADEFF JA CLIN CHIM ACTA 57 307 974 005 ALHADEFF JA CLIN GENET 10 63 976 006 ALHADEFF JA DEVELOP BIOL 47 319 975 007 CLAMP JR METHODS BIOCHEM ANAL 19 229 971 008 DISCHE Z PEDIATRICS 24 74 959 009 DISCHE Z ANN NY ACAD SCI 93 526 962 010 DISCHE Z AM J DIS CHILD 102 733 961 011 ETCHISON JR VIROLOGY 60 217 974 012 GUHA AK CLIN BIOCHEM 10 153 977 013 KNAUFF RE CLIN CHIM ACTA 19 245 968 014 LAEMMLI UK NATURE 227 680 970 015 LINEWEAVER H J AM CHEM SOC 56 658 934 016 LOBECK CC IN: STANBURY JB 1605 972 017 LOWRY OH J BIOL CHEM 193 265 951 018 MCCOMBS ML TEX REP BIOL MED 31 615 973 019 ROELFS RE AM J DIS CHILD 113 419 967 020 SCANLIN TF JR PEDIATR RES ABST 549 11 463 977 021 SPIRO RG ADV PROT CHEM 27 349 973 022 THORPE R FEBS LETTERS 54 89 975 023 TURNER BM AM J HUM GENET 27 651 975 024 WARREN L J BIOL CHEM 234 1971 959 CT 1 ALHADEFF JA CLIN GENET 14 189 978 2 ALHADEFF JA CLIN CHIM ACTA 89 469 978 3 ALHADEFF JA BIOCHEM BIOPHYS RES COMMUN 86 787 979 4 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 5 FREEZE H BIOCHEM J 177 749 979 6 SCANLIN TF CLIN CHEST MED 1 424 980 7 ALHADEFF JA CLIN CHIM ACTA 105 131 980 8 BENYOSEPH Y PEDIATR RES 15 839 981 9 JAKEL HP BIOL ZENTRALBL 100 273 981 10 HARRIS A CLIN CHIM ACTA 116 171 981 11 ALHADEFF JA CLIN CHIM ACTA 117 227 981 12 MALER T J BIOL CHEM 256 1420 981 13 BLITZER MG PEDIATR RES 16 203 982 14 SCANLIN TF BIOCHEMISTRY 21 491 982 15 MARGOLIES R PEDIATR RES 17 931 983 16 RUDICK VL J CELL PHYSIOL 115 143 983 17 BOURASSA C CLIN CHIM ACTA 129 263 983 18 ALHADEFF JA CLIN CHIM ACTA 134 1 983 19 SCANLIN TF PEDIATR RES 19 368 985 20 HERMELIN B CELL MOL BIOL 33 83 987 PN 78077 RN 00858 AN 78189535 AU Danes-B-S. Beck-B. Flensborg-E-W. TI Cystic fibrosis: cell culture classes in a Danish population. SO Clin-Genet. 1978 Apr. 13(4). P 327-34. MJ CYSTIC-FIBROSIS: fg. MN AGE-FACTORS. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. DENMARK. HUMAN. INFANT. AB The cystic fibrosis (CF) culture phenotype of dermal fibroblasts (metachromasia, metabolic cooperation) of a group of 131 Danish CF patients and their families were studied to determine the distribution of the two CF culture classes and their prognostic significance. Of these, 62.6% (82) were Class I, 31.3% (41) Class II and 61.1% (8) were proposed to be genetic compounds. The occurrence of Class II was twice that found in a group of patients from New York (13%) and Minnesota (18%). The prognosis for Class II CF patients was considered to be poorer as: (1) The initial diagnosis was made earlier in Class II than in Class I or the compounds (63% of Class II were diagnosed in the first year of life, as compared to 35% in Class I and 13% of the compounds). (2) Only 5% of the Class II patients survived over the age of 15 years, both being deceased at the end of the study in 1976, whereas 24% of Class I and 63% of the compounds were over 15 years at the end of the study. This research added further evidence for genetic heterogeneity within the clinical syndrome, cystic fibrosis. RF 001 BERATIS NG PEDIATR RES 7 958 973 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 003 DANES BS BIRTH DEF ORIG ART SER 8 114 972 004 DANES BS LANCET 2 765 973 005 DANES BS CLIN GENET 7 128 975 006 DANES BS TEX REP BIOL MED 34 135 976 007 DANES BS AM J HUM GENET 23 297 971 008 DANES BS CLIN GENET 9 527 976 009 DANES BS CLIN GENET 11 83 977 011 LYKKEGAARD E DAN MED BULL 22 177 975 012 LYKKEGAARD E DAN MED BULL 22 169 975 013 LYKKEGAARD E DAN MED BULL 22 175 975 014 WOOD RE LANCET 2 1452 973 CT 1 WINGE P DAN MED BULL 29 358 982 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 78078 RN 00859 AN 78189631 AU Erkkila-J-C. Warwick-W-J. Bradford-D-S. TI Spine deformities and cystic fibrosis. SO Clin-Orthop. 1978 Mar-Apr. (131). P 146-50. MJ CYSTIC-FIBROSIS: co. SPINAL-DISEASES: co. SPINE: ab. MN ADOLESCENCE. ADULT. AGE-DETERMINATION-BY-SKELETON. AGE-FACTORS. BLOOD-PROTEINS. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. FEMALE. GROWTH-DISORDERS: et. HUMAN. KYPHOSIS: cn. MALE. SCOLIOSIS: cn. AB Patients with cystic fibrosis have an increased risk for developing kyphosis and scoliosis. The risk for both kyphosis and scoliosis increases with age. The severity of kyphosis did not appear greater in the older age groups. Patients with retarded bone age may be more likely to have a significant spinal deformity. The presence of spine deformity does not correlate with the severity of pulmonary involvement, height, weight, serum protein and albumin in patients with cystic fibrosis. RF 002 BRADFORD DS J BONE JOINT SURG 56A 740 974 003 BROOKS HL ORTHOP REV 1 17 972 004 BRUSZEWSKI J CHIR NARZAD RUCHU ORTOP POL 22 115 972 005 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 006 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 007 GREULICH WW RADIOGRAPHIC ATLAS OF SKELETA 959 008 KANE WJ CLIN ORTHOP 69 216 970 009 KEMP FH BR J RADIOL 21 449 948 010 KEMP FH BR J SOC MED 2 66 948 011 MATTHEWS LW IN: MANGOS JA 303 973 012 SCHEUERMANN HW UGESKR LAEGER 82 385 920 013 SHANDS AR JR J BONE JOINT SURG 37A 1243 955 014 SORENSEN KH SCHEUERMANN'S JUVENILE KYPHOS 964 015 WARWICK WJ MOD PROBL PEDIATR 10 353 967 016 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 017 WYNNE-DAVIES R J BONE JOINT SURG 50B 24 968 CT 1 PALING MR SKELETAL RADIOL 8 63 982 2 LOGVINOFF MM CLIN PEDIATR 23 389 984 3 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 PN 78079 RN 00860 AN 78168093 AU McFarlene-H. Allan-J-D. Malhotra-A. Hozel-A. Wallwork-J-C. TI The precipitins and hypersensitivity reactions in cystic fibrosis. SO Compr-Ther. 1978 Apr. 4(4). P 61-7. MJ CYSTIC-FIBROSIS: im. HYPERSENSITIVITY: im. PRECIPITINS. MN COMPLEMENT-3. ELECTROPHORESIS-AGAR-GEL. FLUORESCENT-ANTIBODY-TECHNIC. HUMAN. IGA-SECRETORY. IGE. OVALBUMIN: im. SALIVA: im. SERUM-ALBUMIN-BOVINE: im. SPUTUM: im. EX The large number and wide variety of precipitins detected in the sputum of cystic fibrosis patients suggest that the precipitins may be nonprotective and even harmful to the patients. These sputum precipitins were directed principally against food, fungi, bacteria, and human body tissue. On the other hand, precipitins to Staphylococcus alpha-hemolysin was the main type of immunoprecipitin detected in CF serum and was found in 60% of the serum specimens examined. It seems likely that the precipitins in patients with CF may be involved in the formation of immune complexes which, in conjunction with complement, may be responsible for the respiratory and gastrointestinal lesions that are common in patients with CF. The immune complexes may be the result of excess food and bacterial antigens escaping across the gut and lungs as a result of some abnormality of the SIgA system. Over 80% of the sputum specimens tested gave positive precipitins in agar gel against human seminal fluid and colostrum, suggesting that some of the antibodies formed in CF patients could be directed against host tissue, and may account for an autoimmune-type reaction in CF. Crossreaction between the high-titer antibody to Staphylococcus and basement membrane of lungs and pancreas, with complement activation, may occur in CF and required further study. The high number of B-cells, as detected by antibody, coated complement, activated sheep erythrocytes, and EAC rosettes, may be partly responsible for the synthesis of the variety of antibodies detected in CF patients. RF 001 DOGGETT RG J PEDIATR 68 215 966 002 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 003 SCHWARTZ RH CF CLUB ABST 9 965 004 BURNS MW LANCET 1 270 968 005 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 006 PEPYS J LANCET 2 134 968 007 URIEL J BULL SOC CHIM BIOL 48 969 966 008 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 009 MCFARLANE H BR MED J 1 423 975 010 MELLSTEDT H CLIN EXP IMMUNOL 15 309 973 011 JOHANSSEN SGO IMMUNOLOGY 10 265 968 PN 78080 RN 00861 AN 78149018 AU Forstner-J-F. TI Intestinal mucins in health and disease. SO Digestion. 1978. 17(3). P 234-63. (REVIEW). MJ INTESTINAL-MUCOSA. MUCINS. MN CARCINOMA: me. CHEMISTRY. CYSTIC-FIBROSIS: me. GASTROINTESTINAL-DISEASES: me. HORMONES: ph. HUMAN. INTESTINAL-MUCOSA: cy, me, ph. MUCINS: bi, me, ph, se. NEUROREGULATORS: ph. REVIEW. AB Intestinal mucins are complex glycoproteins which are secreted from goblet cells, and form a gel-like covering over the mucosal surface. They are assumed to provide lubrication and protection of the underlying epithelium against potentially injurious chemicals, enzymes, bacteria and dietary constituents. Recent advances in our understanding of mucin structure, secretion and functional properties are reviewed in this paper. Implications for diseases such as cystic fibrosis, peptic ulcer, malignancy and inflammatory bowel disease are briefly discussed. RF 001$ ABT AB ARCH PATHOL 100 301 976 002 ALLEN A BIOCHEM J 106 306 968 003 ALLEN A FARADAY CHEM SOC 57 210 974 004 ALLEN A GUT 13 666 972 005 ALLEN A BIOCHIM BIOPHYS ACTA 338 364 974 006 ALLEN A BIOCHEM SOC TRANS 2 630 974 007 ALPERS DH GASTROENTEROLOGY 64 471 973 008 ANDERSON CG BR J EXP PATHOL 20 25 939 009 ANDRE F ACTA GASTROENTEROL BELG 37 469 974 010 ARONSON NN IN: GOTTSCHALK A 1211 972 011 BAUR PS TEX REP BIOL MED 34 155 976 012 BELLA A JR ARCH BIOCHEM BIOPHYS 150 679 972 013 BENNETT G J CELL BIOL 60 258 974 014 BHASKAR KR BIOCHEM J 143 669 974 015 BLOCH KJ FED PROC FED AM SOC EXP BIOL 36 1054 977 016 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 017 BOAT TF AM REV RESPIR DIS 110 428 974 018 BOGART BI J ULTRASTRUCT RES 52 139 975 019 BOTTIN C BIOL GASTROENTEROL 6 307 973 020 BRAIN MC BR J HAEMATOL 18 183 970 021 CALVER GA CAN J MICROBIOL 22 832 976 022 CAMARRI E ARZNEIMITTEL FORSCH 22 768 972 023 CARLSON DM J BIOL CHEM 241 2984 966 024 CARLSON DM J BIOL CHEM 243 616 968 025 CHANG WWL AM J ANAT 144 39 975 026 CLAUSER H IN: GOTTSCHALK A 1151 972 027 CLAUSER H IN: AMINOFF D 479 970 028 CRAWFORD BJ CAN J ZOOL 52 1427 974 029 CREETH JM MOD PROBL PEDIATR 19 34 977 030 CREETH JM BIOCHEM J 143 159 974 031 CULLING CFA J CLIN PATHOL 28 656 975 032 CZEGLEDY-NAGY E LAB INVEST 35 588 976 033 DEKANSKI JB BR J PHARMACOL 52 464 974 034 DEKANSKI JB BR J PHARMACOL 55 387 975 035 DI GIROLAMO R APPL ENVIRON MICROBIOL 33 19 977 036 DI SANTAGNESE PA N ENGL J MED 277 1287 967 037 DUNSTONE JR BIOCHIM BIOPHYS ACTA 101 300 965 038 EASTWOOD GL GASTROENTEROLOGY 64 383 973 039 ELLIS DB BIOCHIM BIOPHYS ACTA 276 105 972 040 FAILLARD H IN: GOTTSCHALK A 1246 972 041 FILIPE MI BR J CANCER 32 60 975 042 FLOREY H PROC R SOC LOND BIOL 143 144 955 043 FLOREY HW GASTROENTEROLOGY 43 326 962 044 FORD A J BIOL STANDARDS 4 353 976 045 FORSTNER GG CAN J PHYSIOL PHARMACOL 51 122 973 046 FORSTNER JF PEDIATR RES 10 609 976 047 FORSTNER JF MOD PROBL PEDIATR 19 54 977 048 FORSTNER JF CAN J BIOCHEM 51 1154 973 050 FREEMAN JA ANAT REC 154 121 966 051 FUKUDA M BIOCHEM BIOPHYS RES COMMUN 64 465 975 052 GALLAGHER JT PROC R SOC LOND BIOL 192 49 975 053 GELMAN RA BIOCHIM BIOPHYS ACTA 427 627 976 054 GIBBONS RA BIOCHEM J 73 209 959 055 GIBBONS RA NATURE 200 665 963 056 GIBBONS RA IN: AMINOFF D 307 970 057 GIBBONS RJ ADV EXP MED BIOL 45 315 974 058 GIBSON LE PEDIATRICS 48 695 971 059 GINSBURG V ANNU REV BIOCHEM 38 371 969 060 GORALSKI A CELL TISSUE RES 160 551 975 061 GORDON HA EXPERIENTIA 30 214 974 062 GOTTSCHALK A IN: GOTTSCHALK A 1082 972 063 GOTTSCHALK A IN: GOTTSCHALK A 529 972 064 HARTIALA K J BIOL CHEM 195 251 952 065 HEATLEY NG GASTROENTEROLOGY 37 313 959 066 HERNANDEZ FJ DIS COLON RECTUM 19 63 976 067 HOLLANDER F ARCH INTERN MED 93 107 954 068 HOROWITZ MI IN: CODE CF 1063 967 069 HOROWITZ MI ANN NY ACAD SCI 140 784 967 070 HOSKINS LC GASTROENTEROLOGY 54 218 968 071 HOSKINS LC J CLIN INVEST 57 63 976 072 HOSKINS LC J CLIN INVEST 57 63 976 073 HOSKINS LC GASTROENTEROLOGY 54 210 968 074 JABBAL I CAN J BIOCHEM 54 707 976 075 JENNINGS MA Q J EXP PHYSIOL 41 131 956 076 KASHKET S J BACTERIOL 112 1127 972 077 KEEFER CS J CLIN INVEST 17 23 938 078 KELLAWAY IW J PHARM PHARMACOL 27 281 975 079 KENT PW BIOCHEM J 106 645 968 080 KIM SK J ULTRASTRUCT RES 38 371 972 081 KLAAS J J PARASIT 60 907 974 082 KLEINMAN HK BIOCHIM BIOPHYS ACTA 354 17 974 083 KOGA A Z ZELLFORSCH MIKROSK ANAT 139 463 973 084 KOKAS E COMPAR BIOCHEM PHYSIOL 22 81 967 085 KORNFELD R ANNU REV BIOCHEM 45 217 976 086 KRAMER MF HISTOCHEM J 7 511 975 087 KUPCHELLA CE TRANS NY ACAD SCI 34 351 972 088 LAMBLIN G BIOCHIM BIOPHYS ACTA 322 372 973 089 LAMBLIN G BULL EUR PHYSIOPATH RESP 13 175 977 090 LAMBERT R DIGESTION 5 116 972 091 LAMONT JT GASTROENTEROLOGY 72 82 977 092 LAWFORD GR CAN J BIOCHEM 45 507 967 093 LENT CM SCIENCE 179 693 973 094 LEWIS RW LIPIDS 5 947 970 095 LEWIS RW LIPIDS 8 321 973 096 LHERMITTE M BIOCHIMIE 58 367 976 097 LIAU YH DIGESTION 14 372 976 098 LINDSTEDT G J EXP MED 121 201 965 099 LLOYD KO PROC NAT ACAD SCI USA 61 1470 968 100 LUKIE BE BIOCHIM BIOPHYS ACTA 261 353 972 101 LUKIE BE BIOCHIM BIOPHYS ACTA 261 353 972 102 LUKIE BE BIOCHIM BIOPHYS ACTA 273 390 972 103 LUKIE BE BIOCHIM BIOPHYS ACTA 338 345 974 104 MACDERMOTT RP J CLIN INVEST 54 545 974 105 MACHIN J SCIENCE 183 759 974 106 MACPHERSON B DIGESTION 14 424 976 107 MARTIN F DIGESTION 1 165 968 108 MARTINEZ JR PEDIATR RES 9 463 975 109 MCGUIRE EJ IN: AMINOFF D 461 970 110 MEDLINE A HUM PATHOL 7 693 976 111 MENGUY R ANN NY ACAD SCI 140 797 967 112 MERZEL J AM J ANAT 124 281 969 113 MOON HW LAB INVEST 25 133 971 114 OATES MDG CARBOHYD RES 34 115 974 115 ODOHERTY PJA INT J BIOCHEM 6 435 975 116 OEMRAWSINGH I ARCH ORAL BIOL 19 753 974 117 OEMRAWSINGH I ARCH ORAL BIOL 21 755 976 118 NEGUS V PROC R SOC MED 60 75 967 119 NEMOTO T BIOCHIM BIOPHYS ACTA 192 37 969 120 NEUTRA M SCI AM 220 100 969 121 NEUTRA M J CELL BIOL 30 119 966 122 NEUTRA M J CELL BIOL 30 137 966 123 NUNGESTER WJ J INFECT DIS 59 11 936 124 PALAY SL IN: PALAY SL 305 958 125 PIGMAN W ANN ANAT PATHOL 17 227 972 126 PIGMAN W EUR J BIOCHEM 32 148 973 127 PIGMAN W IN: BLANAU 143 973 128 PINEO GF ANN NY ACAD SCI 230 262 974 129 PINEO GF J LAB CLIN MED 82 255 973 130 POTTER JL ANN NY ACAD SCI 106 692 963 131 RAINSFORD KD EXPERIENTIA 15 1172 976 132 REES DA BIOCHEM J 126 257 972 133 ROBERTS GP EUR J BIOCHEM 50 265 974 134 ROBERTS GP ARCH BIOCHEM BIOPHYS 173 528 976 135 ROBINSON DS BIOCHEM J 147 55 975 136 RODRIGUES MM ARCH OPHTHALMOL 89 493 973 137 ROSEMAN S MOD PROBL PEDIATR 10 244 967 138 ROSSIGNOL B FEBS LETTERS 43 241 974 139 ROSSIGNOL B FEBS LETTERS 21 189 972 140 ROUSSEL P J BIOL CHEM 250 2114 975 141 SCHACHTER H BIOCHEM SOC SYMP 40 57 974 142 SCHRAGER J GUT 12 559 971 143 SCHRAGER J ARCH ORAL BIOL 19 1215 974 144 SHACKLEFORD JM IN: AMINOFF D 223 970 145 SHARON N REV BIOCHEM 35 485 966 146 SHERR H GASTROENTEROLOGY 72 1130 977 147 SHILLINGFORD JS BIOCHEM SOC TRANS 2 1104 973 148 SHORA W GASTROENTEROLOGY 68 480 975 149 SLAGLE GW DIS COLON RECTUM 19 253 976 150 SOERGEL KH GASTROENTEROLOGY 47 610 964 151 SPIRO RG N ENGL J MED 281 991 969 152 SPIRO RG REV BIOCHEM 39 559 970 153 STAHL GH BIOCHEM J 136 845 973 154 STARKEY BJ BIOCHEM J 141 633 974 155 STEINBERG SE GASTROENTEROLOGY 68 309 975 156 STROMBECK DR INFECT IMMUN 10 1266 974 157 STURGESS JM CLIN SCI 43 533 972 158 SZULMAN AE ANNU REV MED 17 307 966 159 TAYLOR M LARYNGOSCOPE 84 612 974 160 TRIER JS IN: CODE CF 3 1125 968 161 TUPPY H IN: GOTTSCHALK A 403 972 162 WAECHTER CJ REV BIOCHEM 45 95 976 163 WALDRON-EDWARD D PATHOL BIOL (PARIS) 24 521 976 164 WALDRON-EDWARD D GASTROENTEROLOGY 59 671 970 165 WATKINS WM IN: SMELLIE RMS 125 974 166 WILLIAMS RC INFECT IMMUN 11 711 975 167$ WINDMUELLER HG J BIOL CHEM 249 5070 975 168 WINET H J EXP BIOL 64 283 976 169 WINSNES R ACTA PATH MICROBIOL SCAND (C) 84 77 976 170 WOLD JK ACTA CHEM SCAND (B) 29 703 975 171 WONG YC EXP MOL PATH 23 132 975 173 YOSIZAWA Z IN: GOTTSCHALK A 5 1000 972 CT 1 GAD A SCAND J GASTROENTEROL 14 94 979 2 ZHEREV S AGRESSOLOGIE 20 107 979 3 QURESHI R J CLIN INVEST 64 1149 979 4 SPECIAN RD J CELL BIOL 85 626 980 5 NIMMERFALL F BIOCHEM BIOPHYS RES COMMUN 94 960 980 6 BONERANDI JJ ANN DERMATOL VENEREOL 107 51 980 7 BALAZS M VIRCHOWS ARCH PATHOL ANAT HIS 387 193 980 8 PRIZONT R J CLIN INVEST 67 336 981 9 SPECIAN RD AM J ANAT 160 461 981 10 TABAK LA J ORAL PATHOL 11 1 982 11 SPICER SS HUM PATHOL 13 343 982 12 ECKNAUER R Z GASTROENTEROL 20 150 982 13 SLOMIANY BL ARCH ORAL BIOL 27 803 982 14 GIBBONS RJ AM J CLIN NUTR 36 276 982 15 HOUNSELL EF MED BIOL 60 227 982 16 SLOMIANY BL J DENT RES 61 1163 982 17 NEUTRA MR CHEST 81 S 14 982 18 FERACCI H GASTROENTEROLOGY 82 317 982 19 BEYER EC J CELL BIOL 92 28 982 20 SCHACHTER H ADV EXP MED BIOL 144 3 982 21 TRAYNOR OJ ADV EXP MED BIOL 144 225 982 22 NEUTRA MR AM J PHYSIOL 242 G380 982 23 SPECIAN RD AM J PHYSIOL 242 G370 982 24 CASTRO GA AM J PHYSIOL 243 G321 982 25 SLOMIANY BL J BIOL CHEM 257 9561 982 26 PETERS MW BIOCHIM BIOPHYS ACTA 693 417 982 27 KURIHARA H BIOMED RES 4 549 983 28 ALLEN A TRENDS BIOCHEM SCI 8 169 983 29 DONALDSON JD EUR SURG RES 15 11 983 30 MA J PATHOLOGY 15 385 983 31 SLOMIANY BL ARCH ORAL BIOL 28 711 983 32 SCHULTE BA J HISTOCHEM CYTOCHEM 31 391 983 33 TRAYNOR OJ CANCER 51 1847 983 34 POON H CAN J BIOCHEM CELL BIOL 61 868 983 35 LAMONT JT GASTROENTEROLOGY 84 306 983 36 STEPHEN AM GASTROENTEROLOGY 85 589 983 37 MOUWEN JMVM DTSCH TIERARZ WSCHR 90 477 983 38 SAROSIEK J BIOCHEM BIOPHYS RES COMMUN 115 1053 983 39 WESLEY AW CARBOHYD RES 115 151 983 40 WITAS H CARBOHYD RES 120 67 983 41 ZENTLERMUNRO PL BR MED J 287 501 983 42 SHIMANO T ANN NY ACAD SCI 417 97 983 43 SLOMIANY A BIOCHIM BIOPHYS ACTA 750 253 983 44 YAMAMOTO A J PHARMACOBIO DYN 7 728 984 45 LINDAHL M VET MICROBIOL 9 249 984 46 REID PE HISTOCHEM J 16 235 984 47 ALLEN A SCAND J GASTROENTEROL 19 101 984 48 SAROSIEK J SCAND J GASTROENTEROL 19 150 984 49 BORSCH G KLIN WSCHR 62 699 984 50 OHARA S COMP BIOCHEM PHYSIOL (B) 79 325 984 51 FORSTNER J CIBA FOUND SYMP 109 61 984 52 NEUTRA MR CIBA FOUND SYMP 109 20 984 53 MURTY VLN BIOCHEM BIOPHYS RES COMMUN 121 521 984 54 SALMONOWITZ E AM J ROENTGENOL 142 721 984 55 KITADA M CLIN CHIM ACTA 144 173 984 56 STITH BJ J COMP PHYSIOL (B) 155 89 984 57 SLOMIANY A J BIOL CHEM 259 4743 984 58 SAROSIEK J ANN NY ACAD SCI 435 575 984 59 BASBAUM C GASTROENTEROL CLIN BIOL 9 45 985 60 GOLDERMAN L ISR J MED SCI 21 410 985 61 SEGAWA K JAP J MED 24 244 985 62 GONZALEZ EA FEMS MICROBIOL LETTERS 29 115 985 63 ALLEN A DIG DIS SCI 30 S 55 985 64 LASZEWICZ W DIGESTION 31 47 985 65 COHEN PS INFECT IMMUN 48 139 985 66 NEVOLA JJ INFECT IMMUN 50 152 985 67 CHNG HS J PHARM SCI 74 399 985 68 BELL AE GASTROENTEROLOGY 88 269 985 69 LOURENS JM TIJDSCHR DIERGENEESKD 110 755 985 70 SLOMIANY A BIOCHEM BIOPHYS RES COMMUN 132 299 985 71 ENE MD CLIN CHIM ACTA 153 165 985 72 BRADY RC AM J PHYSIOL 248 G 54 985 73 CARLSTEDTDUKE B EUR J CLIN MICROBIOL 5 634 986 74 SAITOH H J PHARMACOBIO DYN 9 1008 986 75 SALOMONOWITZ E GASTROINTEST RADIOL 11 93 986 76 TOOFANIAN F LAB ANIM SCI 36 157 986 77 HALAMA AK TIERARZ UMSCH 41 340 986 78 LAUX DC INFECT IMMUN 52 18 986 79 CARLSTEDTDUKE B ACTA PATH MICROB IMMU SCA (B) 94 293 986 80 SMITH PL AM J PHYSIOL 250 G432 986 81 MOURICOUT MA INFECT IMMUN 55 1216 987 82 SNYDER JD INT ARCH ALLERGY APPL IMMUNOL 82 351 987 83 SHERMAN P AM J PATHOL 126 527 987 PN 78081 RN 00862 AN 79045223 AU Rao-G-J. Platt-M-W. Nadler-H-L. TI Reaction of 4-methylumbelliferylguanidinobenzoate with proteases in plasma of patients with cystic fibrosis. SO Enzyme. 1978. 23(5). P 314-9. MJ CYSTIC-FIBROSIS: en. HYMECROMONE: du. PEPTIDE-HYDROLASES: df. UMBELLIFERONES: du. MN ADOLESCENCE. ARGININE: aa, du. BINDING-SITES. CHILD. CHILD-PRESCHOOL. GUANIDINES: du. HUMAN. HYMECROMONE: aa. IN-VITRO. ISOELECTRIC-FOCUSING. MOLECULAR-WEIGHT. PEPTIDE-HYDROLASES: bl. SUPPORT-U-S-GOVT-P-H-S. AB Protease activity, assayed using 4- methylumbelliferylguanidinobenzoate, an active site titrant of certain proteases, is significantly deficient in plasma of patients with cystic fibrosis. The deficiency can be demonstrated with both chloroform-ellagic acid activated plasma in which the proteases can hydrolyze esters of arginine and unactivated plasma in which the proteases have negligible activity towards these esters. The deficiency can also be demonstrated by separation of the proteases by isoelectric focusing on polyacrylamide gels or by chromatography on agarose columsn. Since protease deficiency can be demonstrated with unactivated plasma, the deficiency in cystic fibrosis is probably due to a reduced number of protease molecules rather than their decreased catalytic efficiency. RF 001 BENDER ML J AM CHEM SOC 88 5890 966 002 CHAN KYH CLIN CHIM ACTA 74 71 977 003 CHASE T JR BIOCHEMISTRY 8 2212 969 004 COBURN MD AM REV RESPIR DIS 110 368 974 005 DI SANTAGNESE PA N ENGL J MED 295 481 976 006 JAMESON GW BIOCHEM J 131 107 973 007 KERR MA BIOCHEMISTRY 14 5088 975 008 OUCHTERLONY O ACTA PATH MICROBIOL SCAND 25 186 948 009 RAO GJS J PEDIATR 80 573 972 010 RAO GJS PEDIATR RES 8 684 974 011 RAO GJS PEDIATR RES 9 739 975 012 RAO GJS SCIENCE 177 610 972 013 SCHONI M EUR J CLIN INVEST 5 153 975 014 SEALE TW PEDIATR RES ABST 552 11 463 977 015 SHAPIRA E CLIN CHIM ACTA 78 359 977 016 SHAPIRA E PEDIATR RES 10 812 976 017 BARRETT AJ BIOCHEM J 133 709 973 018 WILSON GB PEDIATR RES 10 87 976 CT 1 BROCK DJH LANCET 1 1245 979 2 WALSH MM LANCET 1 622 979 3 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 4 RAO GJS ENZYME 24 280 979 5 WALSHPLATT M ENZYME 24 224 979 6 NADLER HL LANCET 2 96 980 7 WALSH MMJ PEDIATR RES 14 353 980 8 NADLER HL PEDIATRICS 66 690 980 9 PARSONS M CLIN CHIM ACTA 100 215 980 10 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 11 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 12 LLOYDSTILL JD J PEDIATR 99 580 981 13 NADLER HL AM J OBSTET GYNECOL 141 885 981 14 BROCK DJH RIC CLIN LAB 12 231 982 15 SCHWARTZ M CLIN CHIM ACTA 124 213 982 16 TUMMLER B CLIN CHIM ACTA 125 219 982 17 GREEN JR EUR J PEDIATR 139 35 982 18 BRANCHINI BR PEDIATR RES 17 850 983 19 BRIDGES MA ANN NY ACAD SCI 421 360 983 20 HODSON ME POSTGRAD MED J 60 225 984 21 BUYS CHCM CLIN CHIM ACTA 136 229 984 22 MARYNEN P BIOCHIM BIOPHYS ACTA 799 187 984 23 PERINI JM BULL EUR PHYSIOPATH RESP 21 569 985 24 QURESHI AR J PEDIATR 106 913 985 PN 78082 RN 00863 AN 78238710 AU Endres-W. Wuttge-B. TI Occurrence of secondary cystathioninuria in children with inherited metabolic disorders, liver diseases, neoplasms, cystic fibrosis and celiac disease. SO Eur-J-Pediatr. 1978 Aug 17. 129(1). P 29-35. MJ CELIAC-DISEASE: ur. CYSTATHIONINE: ur. CYSTIC-FIBROSIS: ur. LIVER-DISEASES: ur. METABOLISM-INBORN-ERRORS: ur. NEOPLASMS: ur. MN ADOLESCENCE. AGE-FACTORS. BILE-DUCTS: ab. CHILD. CHILD-PRESCHOOL. CYTOMEGALIC-INCLUSION-DISEASE: ur. HUMAN. INFANT. AB Secondary cystathioninuria was found in two of 46 children suffering from tumors, leukemia, liver disease, inherited metabolic disorders, cystic fibrosis and celiac disease. Of these two patients, one had congenital biliary atresia and the other cytomegalovirus infection. Seven further children had only moderately elevated excretion of cystathionine. It is suggested that secondary cystathioninuria is uncommon in the disease investigated. RF 001 ENDRES W Z KINDERHEILK 110 46 971 003 FOURMAN P ARCH DIS CHILD 41 273 966 004 GEISER CF CANCER 22 856 968 005 GJESSING LR LANCET 2 1281 963 006 GJESSING LR SCAND J CLIN LAB INVEST 17 513 965 007 GJESSING LR RECENT RESULTS CANCER RES 2 26 966 008 LIEBERMAN E PEDIATRICS 40 828 967 009 SCRIVER CR PEDIATRICS 31 240 963 010 SHAW KNF AM J DIS CHILD 113 119 967 011 STUDNITZ W ACTA PAEDIATR SCAND 58 173 969 012 STUDNITZ W ACTA PAEDIATR SCAND 59 80 970 013 VOUTE PA JR CLIN CHIM ACTA 22 373 968 CT 1 SHIN YS CLIN CHIM ACTA 127 77 983 PN 78083 RN 00864 AN 78214700 AU Schroder-C-P. Emrich-H-M. TI Ultramicro-electrophoresis of protein in sweat from patients with cystic fibrosis of the pancreas and controls. SO Eur-J-Pediatr. 1978 May 22. 128(1). P 49-56. MJ CYSTIC-FIBROSIS: me. PROTEINS: an. SWEAT: an. MN ADOLESCENCE. BINDING-SITES. CELL-MEMBRANE. CHILD. CHILD-PRESCHOOL. ELECTROPHORESIS: mt. HUMAN. HYDROGEN-ION-CONCENTRATION. SALIVA. VISCOSITY. AB 46 sweat-samples from 32 children with cystic fibrosis of the pancreas (C.F.) and 35 samples from 23 control-children were collected with glass micro-capillaries. Protein was determined by ultramicro-electrophoresis. Results: 1. Protein was detected in 48% of the samples (C.F. and controls). 2. At pH 2.3, C.F.-sweat showed at least one more band than control-sweat. 3. At pH 8.9, C.F.-sweat occasionally showed one more band than the control-group. 4. At pH 2.3, more protein was found at the electrophoresis start point using C.F.-sweat, whereas at pH 8.9 the opposite was found. 5. At pH 2.3, protein was found in fewer samples than at pH 8.9. The "C.F.-factor" is postulated to represent a basic polyelectrolyte which induces the following pathogenic mechanisms: a) Aggregation of proteins giving rise to a high viscosity of secretions, such as saliva. b) Binding to the cell-membrane of the glandular epithelium, thus inducing a disturbance of active NaCl-reabsorption (e.g. by reduction of luminal passive Na+-influx). RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 BARNETT DR TEX REP BIOL MED 31 709 973 003 BOWMAN BH AM J HUM GENET 25 16A 973 004 DOGGETT RG NATURE NEW BIOL 243 250 973 005 EMRICH HM PFLUEGERS ARCH 290 298 966 006 GIBSON LE PEDIATRICS 23 545 959 007$ GROSSBACH U IN: GLICK D 3 975 008 HEICK HMC CLIN RES 21 1037 973 009 JIRKA M NATURE 199 283 963 010 KAISER D PEDIATR RES 5 167 971 011 LOBECK CC IN: STANBURY JB 1605 972 012 MCEVOY FA PEDIATR RES 9 721 975 013 MANGOS JA SCIENCE 158 135 967 014 MANGOS JA PEDIATR RES 1 436 967 015 MANGOS JA PEDIATR RES 2 378 968 016 MANGOS JA TEX REP BIOL MED 31 651 973 017 NIKOLAJEK WP EUR J PEDIATR 122 289 976 018 TAYLOR A PEDIATR RES 8 861 974 PN 78084 RN 00865 AN 78106794 AU Gotz-M. Lubec-G. TI Complement in cystic fibrosis. SO Eur-J-Pediatr. 1978 Jan 17. 127(2). P 133-9. MJ COMPLEMENT: an. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. COMPLEMENT-3: an. COMPLEMENT-4: an. FEMALE. HUMAN. INFANT. MALE. PSEUDOMONAS-AERUGINOSA. SEX-FACTORS. SPUTUM: mi. AB Complement components C3, C4, and C3A were estimated in 30 patients with cystic fibrosis aged 1 to 21 years (M:F = 16:14) and were compared with results in 40 healthy, age-matched subjects. The influences of the clinical score, sputum microbiology, and the patients' sex were also investigated. In contrast to most previous communications, this paper shows that, compared to the control group, a significant decrease of C3 (P less than 0.001) and C4 (P less than 0.02) was observed whereas C3A levels were not altered. There were no increases in complement. Shwachman-scores above or below 70 did not influence the complement levels, nor did exacerbations of the disease change the levels. No influence of the patients' sex could be shown. Pseudomonas aer. in the sputum was clearly associated with complement defects (14/18). Alternative-pathway involvement of complement activation could be demonstrated in 32%. The results make complement activation due to pulmonary infection most likely. The defects observed probably represent secondary changes. RF 001 ALPER CA N ENGL J MED 288 601 973 002 ALPER CA IN: NETER E 975 003 BEDROSSIAN CWM HUM PATHOL 7 195 976 004 CHARLESWORTH JA J CLIN INVEST 53 1578 974 005 CONOVER JH LANCET 2 1501 973 006 CONOVER JH LANCET 1 47 975 007 CONOVER JH LIFE SCI 14 253 974 008 DANES BS J EXP MED 129 775 969 009 ESTERLY JR IN: MANGOS JA 115 976 010 FEARON DT N ENGL J MED 292 937 975 011 FUST G CLIN IMMUNOL IMMUNOPATHOL 5 293 976 012 GIBBONS A BR MED J 1 120 976 013 GIBSON LE PEDIATRICS 23 545 959 014 GOETZ M PROC EWGCF 6TH ANNU MTG 975 015 HANN S LANCET 2 520 974 016 HANSSON LO LANCET 2 874 975 017 HARPER BL CF CLUB ABST 17 20 976 018 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 019 HOLZHAUER RJ CF CLUB ABST 17 22 976 020 JOHNSTON RB J PEDIATR 90 169 977 021 KAY AB LANCET 2 916 974 022 LEDERBERG S AM J HUM GENET 28 597 976 023 LEDERBERG S AM J HUM GENET 28 597 976 024 MCFARLANE H BR MED J 1 423 975 025 OSOFSKY SG J PEDIATR 90 180 977 026 PALMER DG AUST NZ J MED 6 349 976 027 POLMAR SH IN: KIRKPATRICK CH 191 976 028 ROSSMAN CM J PEDIATR 90 579 977 028A ROY CC PEDIATR CLIN GASTROENTEROL 975 029 SCANLIN TF JR LANCET 1 1382 974 030 STEPHAN U PEDIATRICS 55 35 975 031 TAUSSIG LM CF CLUB ABST 17 21 976 032 WEEKE B DAN MED BULL 23 155 976 033 WOOD RE AM REV RESPIR DIS 111 733 975 034 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 2 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 3 HODSON ME THORAX 35 801 980 4 MOSS RB AM REV RESPIR DIS 121 23 980 5 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 6 FICK RB J CLIN INVEST 68 899 981 7 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 8 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 9 FICK RB PEDIATR RES 20 1258 986 PN 78085 RN 00866 AN 78107013 AU Watts-R-W. TI Progress in screening for inborn errors of metabolism. SO Experientia. 1978 Feb 15. 34(2). P 143-52. MJ GENETIC-SCREENING. MASS-SCREENING. METABOLISM-INBORN-ERRORS: di. MN ADULT. LIPOIDOSIS: di. ANEMIA-SICKLE-CELL: di. CARBOXYLIC-ACIDS: ur. CYSTIC-FIBROSIS: di. ENZYME-TESTS. FEMALE. GALACTOSEMIA: di. GLYCOSIDES: bl. HUMAN. HYPOTHYROIDISM: cn, di. INFANT. INFANT-NEWBORN. JEWS. LYMPHOCYTES: me. MALE. OLIGOSACCHARIDES: ur. PHENYLKETONURIA: di. PREGNANCY. THALASSEMIA: di. AB The inborn errors of matabolism are a series of individually rare biochemical anomalies some of which cause serious clinical manifestations. They are of great interest to biochemists and geneticists, as well as to paediatricians and internist for whom they often present special diagnostic and therapeutic problems. The study of the inborn errors of metabolism also has implications in the fields of epidemiology and social medicine. The number of known inborn errors of metabolism has increased rapidly in recent years, and others, as yet unidentified, presumably await recognition. Only a few of these conditions can be treated now, but the realisation that early diagnosis is essential in order to achieve good results has stimulated interest in the possibility of examining either whole populations or selected predisposed groups of individuals for biochemical differences which characterise particular inherited metabolic diseases. This article reviews some recent developments with particular reference to the indications for such screening programmes and progress in the identification of previously unknown inborn errors of metabolism in otherwise homogeneous population groups. The inborn errors of metabolism are due to single gene mutations. Recognition of the asymptomatic individuals who are heterozygous for the abnormal gene causing the disease may be important clinically and the identification has to be considered as one aspect of metabolic screening for the inborn errors of metabolism. RF 001 WATTS RWE LANCET 1 368 975 002 PURKISS P PROC INT SYMP GLYCOCON 3RD 19 975 005 BROWN E METABOLISM 26 1047 977 006 CHALMERS RA ANALYST 97 224 972 007 CHALMERS RA ANALYST 97 951 972 008 CHALMERS RA ANALYST 97 958 972 009 HEALY MJR J CHROMATOGR 87 365 973 010 LAWSON AM ADV MASS SPECTROMET 6 235 974 011 LAWSON AM BIOMED MASS SPECTROMET 1 199 974 012 CHALMERS RA CHEM BRIT 11 290 975 013 LEVY HL N ENGL J MED 291 1214 974 014 CLAYTON BE IN: RAINE DN 1 1 974 015 KOMROWER GM PEDIATRICS 53 182 974 016 SEEGMILLER JE IN: NATELSON S 291 974 017 SCRIVER CR LANCET 2 230 964 018 SMITH DW PEDIATRICS 19 1011 957 019 MAN EB J PEDIATR 63 926 963 020 DUTAU G ANN PEDIATR (PARIS) 22 315 975 021 RAITI S ARCH DIS CHILD 46 692 971 022 KLEIN AH J PEDIATR 81 912 972 023 FISHER DA J PEDIATR 89 692 976 024 CHOPRA IJ N ENGL J MED 293 740 975 025 LAWSON D ARCH DIS CHILD 44 715 969 026 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 027 BRAY PT CLIN CHIM ACTA 77 69 977 028 STEPHAN U PEDIATRICS 55 35 975 029 RYLEY HC ARCH DIS CHILD 49 901 974 030 GIBSON LE PEDIATRICS 23 545 959 031 SPOCK A PEDIATR RES 1 173 967 032 BOWMAN BH TEX REP BIOL MED 31 611 973 033 GLUECK CJ J LAB CLIN MED 82 467 973 034 KWITEROVICH PO JR LANCET 1 118 973 035 ADRIAENSSENS K PROTIDES BIOL FLUIDS 19 265 971 036 DARMADY J PROTIDES BIOL FLUIDS 19 26A 971 037 DARMADY JM BR MED J 2 685 972 038 OSE L LANCET 2 625 975 039 SAIFER A IN: NATELSON S 353 974 040 KABACK MM PROG MED GENET 10 103 974 041 CHILDS B AM J HUM GENET 28 537 976 042 CHILDS B AM J HUM GENET 28 550 976 043 KABACK MM HUM HERED 27 186 977 044 PADEH B HUM HERED 27 200 977 045 EVANS PR IN: RAINE DN 93 977 046 KAN YW LANCET 1 269 977 047 NATHAN DG BR J HAEMATOL SUPPL 31 143 975 048 CHALMERS RA ANN CLIN BIOCHEM 14 149 977 049 LAWSON AM CLIN CHEM 22 1283 976 050 CHALMERS RA CLIN CHEM 22 1288 976 051 CHALMERS RA CLIN CHEM 22 1292 976 052 ROBERTS GE BIOCHEM SOC SPEC PUBL 1 7 973 053 LA DU BN J BIOL CHEM 230 251 958 054 KAUFMAN S N ENGL J MED 293 785 975 055 BARTHOLOME K PEDIATR RES 9 899 975 056 WATTS RWE NATURE 201 396 963 057 POLLACK VE J LAB CLIN MED 66 564 965 058 LA DU BN BIOCHEM BIOPHYS RES COMMUN 7 398 962 059 RYMAN BE IN: WILKINSON JH 976 060 SCRIVER CR AMINO ACID METABOL DISORDERS 973 061 GIBBS DA ANN CLIN BIOCHEM 14 157 977 063 LYON MF AM J HUM GENET 14 135 962 064 WITKOP CJ JR AM J HUM GENET 22 55 970 065 GARTLER SM ANN HUM GENET 33 171 969 066 GARTLER SM SCIENCE 172 572 971 067 WATTS RWE IN: HOLTON J 975 068 SPENCE MW J MED GENET 14 91 977 070 RAINE DN J CLIN PATHOL SUPPL 27 8 974 CT 1 PURKISS P CLIN CHEM 24 669 978 2 LEHNERT W MONATSSCHR KINDERHEILKD 127 665 979 3 FARRIAUX JP ARCH FR PEDIATR 37 367 980 4 ISSAQ HJ J LIQUID CHROMATOGR 4 2233 981 5 BECKER K MONATSSCHR KINDERHEILKD 129 556 981 6 DHARESHWAR SS INDIAN J MED RES 76 716 982 PN 78086 RN 00867 AN 79046205 AU Neutra-M-R. Trier-J-S. TI Rectal mucosa in cystic fibrosis. Morphological features before and after short term organ culture. SO Gastroenterology. 1978 Oct. 75(4). P 701-10. MJ CYSTIC-FIBROSIS: pa. INTESTINAL-MUCOSA: pa. RECTUM: pa. MN ADOLESCENCE. ADULT. AUTORADIOGRAPHY. BIOPSY. CHILD. EPITHELIUM: pa. HUMAN. IN-VITRO. MUCUS: an. ORGAN-CULTURE. SUPPORT-U-S-GOVT-P-H-S. AB Histological changes observed in the large intestinal mucosa of cystic fibrosis (CF) patients, including crypt enlargement as well as intracellular and extracellular accumulation of mucus, have been considered by some to be useful diagnostic signs of this disease. The extent of these changes, however, has not been evaluated quantitatively. In this study, quantitative stereological methods were applied to biopsies of rectal mucosa from 5 CF patients, 5 sibling controls, and 2 normal adults, to measure goblet cell numbers, crypt luminal volume, and volume of intracellular mucus. It was found that CF crypt lumina were variably dilated, but that the average relative volume of intracellular mucus and the numbers of goblet cells in CF mucosae were comparable to those of sibling and adult controls. In CF biopsies, however, surface columnar absorptive cells consistently contained putative lipid droplets and rapidly accumulated additional large lipid droplets during short term organ culture. Lipid droplets were not observed in the same cell types of control biopsies before or after short term organ culture. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 004 NEUTRA MR LAB INVEST 36 535 977 005 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 006 DONNISON AB PEDIATRICS 37 833 966 007 EIDELMAN S CF CLUB ABST 5 964 008 JABRO IH J LANCET 86 385 966 009 PARKINS RA LANCET 2 851 963 010 SHWACHMAN H N ENGL J MED 286 1300 972 011 HAGE E ACTA PATH MICROBIOL SCAND (A) 80 345 972 012 DALTON AJ ANAT REC 121 281 955 013 SHANTZ A STAIN TECHNOL 40 279 965 014 WEIBEL ER IN: HAYAT MA 3 239 973 015 BROWNING TH J CLIN INVEST 48 1423 969 016 EASTWOOD GL GASTROENTEROLOGY 64 375 973 017 JODL J ACTA PAEDIATR SCAND 60 367 971 018 GEAR EV JR GASTROENTEROLOGY 55 522 968 019 THOMAIDIS TS J PEDIATR 63 444 963 020 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 021 FORSTNER JF PEDIATR RES 10 609 976 022 JOHANSEN PG LANCET 1 455 968 023 BENNETT G J CELL BIOL 46 409 970 024 FORSTNER GG J BIOL CHEM 245 3584 970 025 DOBBINS WO AM J CLIN NUTR 22 257 969 026 CARDELL RR J CELL BIOL 34 123 967 027 PALAY SL PROC INT SYMP LIPID TRANSPOR 33 964 028 SABESIN SM LIPIDS 10 840 975 029 BINDER HJ PROC SOC EXP BIOL MED 131 1119 969 030 MELNICK CS GASTROENTEROLOGY 52 985 967 031 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 032 KESSLER JI GASTROENTEROLOGY 68 1068 975 033 CLARK SB J LIPID RES 14 581 973 034 SNIPES R LAB INVEST 18 179 968 035 ODOHERTY PJA LIPIDS 8 249 973 036 GLICKMAN RM GASTROENTEROLOGY 70 347 978 037 JACOP JS J CELL BIOL 70 158A 976 038 ELLIOTT RB PEDIATRICS 57 474 976 039 CAMPBELL IM PEDIATRICS 57 480 976 040 BUTCHER FR IN: MANGOS JA 243 976 CT 1 PARK RW GASTROENTEROLOGY 81 1143 981 2 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 3 GOLDMAN H HUM PATHOL 13 981 982 4 HOWDLE PD CLIN SCI 65 105 983 5 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 6 BRADY RC EXP CELL RES 150 141 984 PN 78087 RN 00868 AN 78150401 AU Pearson-R-D. Lubin-A-H. TI A simple method for estimating a 'heparin binding capacity' of human serum. SO Health-Lab-Sci. 1978 Jan. 15(1). P 32-7. MJ BLOOD-PROTEINS: me. HEPARIN: me. PROTEIN-BINDING. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: bl. FEMALE. HEPARIN: an, bl. HUMAN. MALE. METHODS. MIDDLE-AGE. AB A method for estimating a 'heparin binding capacity' of human serum is described. When human serum is diluted with a low ionic strength, heparin-containing buffer at pH 5.5, protein-heparin electrostatic complexes form in solution with subsequent formation of insoluble aggregates which can be collected by centrifugation. Quantitative determination of the relative amounts by weight of protein and heparin in the insoluble heparin-protein aggregates permits estimation of a combining ratio at which serum proteins bind with heparin to precipitate from solution. This weight combining ratio of protein and heparin is a quantitative measure of the total affinity for heparin of all proteins in serum which bind heparin at pH 5.57 to form an insoluble complex. An unusually high affinity for heparin by an abnormal serum protein or an increase in amount of a normally- occuring, high heparin-affinity, serum protein would alter the average protein: heparin combining ratio and increase the 'heparin- binding capacity' of human serum. The converse would be true for serum proteins having a low affinity for heparin, lowering the 'heparin-binding capacity' of human serum. The described method was used to evaluate the 'heparin-binding capacity' of serum proteins in normal individuals and in persons with cystic fibrosis. RF 001 BITTER T ANAL BIOCHEM 4 330 962 002 DI SANTAGNESE PA LANCET 2 765 967 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 DISCHE Z J BIOL CHEM 167 189 947 005 DISCHE Z J BIOL CHEM 175 595 948 006 EHRLICH J J PHARM SCI 62 517 973 007 GOODMAN LS PHARMACOLOGIC BASIS OF THE TH 1446 970 008 HOMAN JDH BIOCHIM BIOPHYS ACTA 2 333 948 009 JAKOVCIC S J PEDIATR 62 25 963 010 LASKER SE ARCH BIOCHEM BIOPHYS 115 360 966 011 LINDAHL U IN: BALAZS EA 2 943 970 012 LOWRY OH J BIOL CHEM 193 265 951 013 MARCINIAK E J LAB CLIN MED 84 344 974 014 PEARSON RD CF CLUB ABST 17 976 015 SLACK J J LAB CLIN MED 59 302 962 CT 1 PEARSON RD PEDIATR RES 13 834 979 2 DUPOUY JP J PHYSIOL (PARIS) 76 631 980 3 VERPOORTE JA INT J BIOCHEM 13 1151 981 4 MARGOLIES R PEDIATR RES 16 181 982 5 MARGOLIES R PEDIATR RES 17 931 983 6 DUPOUY JP ANN ENDOCRINOL PARIS 47 429 986 PN 78088 RN 00869 AN 79109072 AU Marchi-A-G. Marchi-M-A. Cigui-I. Uxa-F. Perticarari-S. Nordio-S. TI Further studies on lymphocyte beta-glucuronidase abnormality in cystic fibrosis. Possible relations to carboxypeptidase B-like activity. SO Helv-Paediatr-Acta. 1978 Dec. 33(6). P 517-25. MJ CARBOXYPEPTIDASES: bl. CYSTIC-FIBROSIS: en. GLUCURONIDASE: bl. LYMPHOCYTES: en. MN CHILD. CYSTIC-FIBROSIS: di, fg. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. LYMPHOCYTE-TRANSFORMATION. MALE. PHYTOHEMAGGLUTININS: pd. AB The presence in the serum of both cystic fibrosis (CF) homozygotes and heterozygotes of a factor inhibiting the response of lymphocyte lysosome beta-glucuronidase activity to in vitro phytohaemagglutinin (PHA) stimulation is confirmed. Studying lymphocyte beta- glucuronidase activity on PHA stimulation represents a way to confirm CF diagnosis and to screen CF heterozygotes. For technical complexity, however, the method cannot be used for mass screening, but it can be useful for confirming the diagnosis in suspected cases. Relationships between serum factor inhibiting the effect of PHA on beta-glucuronidase, ciliary dyskinesia factor and carboxypeptidase B- like activity are discussed. RF 001 CONOVER JH LANCET 2 1501 973 002 CONOVER JH PEDIATR RES 7 220 973 003 CONOVER JH PEDIATR RES 7 224 973 004 CONOVER JH LIFE SCI 14 253 974 005 DI SANTAGNESE PA N ENGL J MED 295 481 976 006 ERDOS EG BIOCHEM PHARMACOL 13 893 964 007 FOLK JE J BIOL CHEM 235 2272 960 008 LIEBERMAN J AM REV RESPIR DIS 111 100 975 009 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 010 MANGIAROTTI-MARCHI MA MINERVA PEDIATR 23 1847 971 011 MARCHI AG MINERVA PEDIATR 23 1842 971 012 MARCHI AG HELV PAEDIATR ACTA 28 427 973 013 MARCHI AG ACTA PAEDIATR BELG 29 67 976 014 NORDIO S ACTA PAEDIATR SCAND 63 664 974 CT 1 JAKEL HP BIOL ZENTRALBL 100 273 981 PN 78089 RN 00870 AN 79047858 AU Schwarz-H-P. Kraemer-R. Thurnheer-U. Rossi-E. TI Liver involvement in cystic fibrosis. A report of 9 cases. SO Helv-Paediatr-Acta. 1978 Nov. 33(4-5). P 351-64. MJ CYSTIC-FIBROSIS: co. LIVER-DISEASES. MN ADOLESCENCE. CASE-REPORT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. LIVER-CIRRHOSIS: pa. LIVER-CIRRHOSIS-BILIARY: pa. LIVER-DISEASES: pa. LIVER: pa. MALE. AB 9 out of 204 unselected cystic fibrosis (CF) patients seen at the Department of Pediatrics, University of Berne, Switzerland, over the last 20 years had clinically overt liver disease. In 7 patients liver cirrhosis was demonstrated (3.4%). Focal biliary cirrhosis was the pertinent finding in 3 cases, whereas another 3 showed unspecific nodular cirrhosis. One infant presented with prolonged obstructive jaundice due to partial extrahepatic atresia and delayed passing of meconium. Two other patients had steatosis. A review of the literature dealing with clinical and laboratory findings in CF patients with liver disease is given. RF 001 ALHADEFF JA CLIN GENET 10 63 976 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 BERNSTEIN JG AM J DIS CHILD 99 804 960 004 BOAT TF CLIN PEDIATR 13 505 974 005 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 006 COOPER HS JOHNS HOPKINS MED J 135 268 974 007 CRAIG JM AM J DIS CHILD 93 357 957 008 DI SANTAGNESE PA PEDIATRICS 18 387 956 009 DI SANTAGNESE PA N ENGL J MED 295 481 976 010 DONATH A HELV PAEDIATR ACTA 25 634 970 011 DUBOIS RS GASTROENTEROLOGY 72 1052 977 012$ EKLUND A IN: PAUMGARTNER G 241 977 013 EXSS R DTSCH MED WSCHR 97 425 972 014 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 015 FEIGELSON J ACTA PAEDIATR SCAND 61 337 972 016 FEIGELSON J NOUV PRESSE MED 1 1899 972 017 FEIGELSON J ACTA PAEDIATR SCAND 61 337 972 018 GATZIMOS CD AM J DIS CHILD 89 182 955 019 GIEGLER I ANN ANAT PATHOL 5 115 960 020 GOODCHILD MC ARCH DIS CHILD 50 769 975 021 GOODCHILD MC ARCH DIS CHILD 50 813 975 022 ISENBERG JN GASTROENTEROLOGY 66 887 974 023 JEAN R ARCH FR PEDIATR 27 849 970 024 KATTWINKEL J J PEDIATR 82 234 973 025 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 271 977 026 KRAEMER R HELV PAEDIATR ACTA 32 107 977 027 LITT IF PEDIATR CLIN NORTH AM 20 945 973 028 OPPENHEIMER EH LANCET 2 1031 973 029 OPPENHEIMER EH J PEDIATR 86 683 975 030 ORENSTEIN DM AM J DIS CHILD 131 973 977 031 ROLLER RJ GASTROENTEROLOGY 72 661 977 032 ROSENSTEIN BJ J PEDIATR 91 1022 977 033 ROY CC N ENGL J MED 297 1301 977 034 SCHWARZ HP CLIN CHIM ACTA 50 197 974 035 SHIER KJ CAN MED ASSOC J 89 645 963 036 SHWACHMAN H AM J DIS CHILD 96 6 958 037 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 038 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 039 SOLOWAY RD AM J DIG DIS 16 1082 971 040 STERN RC GASTROENTEROLOGY 70 645 976 041 STERN RC J PEDIATR 89 406 976 042 STERN RC ANN INTERN MED 87 188 977 043 TALAMO RC AM J DIS CHILD 115 74 968 044 TANNER JM ARCH DIS CHILD 41 454 966 045 TAYLOR WF AM J DIS CHILD 123 161 972 046 VALMAN HB ARCH DIS CHILD 46 805 971 047 WARWICK WJ JAMA 238 2159 977 048 WATKINS JB GASTROENTEROLOGY 73 1023 977 049 WEBER AM N ENGL J MED 289 1001 973 050 WEBER AM GUT 17 295 976 051 WILROY RS JR J PEDIATR 68 67 966 CT 1 CUNNINGHAM DG J COMPUT ASSIST TOMOGR 4 151 980 2 BALISTRERI WF J PEDIATR 97 689 980 3 MAGAZZU G RIV ITAL PEDIATR 7 73 981 4 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 5 MCCLURE HM ADV VET SCI COMP MED 28 267 984 6 BERNARD O CLIN GASTROENTEROL 14 33 985 7 FEIGELSON J ANN PEDIATR (PARIS) 32 218 985 8 SCHRODER R SCHWEIZ MED WOCHENSCHR 115 828 985 PN 78090 RN 00871 AN 78193578 AU Warwick-W-J. TI The incidence of cystic fibrosis in Caucasian populations. SO Helv-Paediatr-Acta. 1978 Jun. 33(2). P 117-25. MJ CAUCASOID-RACE. CYSTIC-FIBROSIS: oc. MN HUMAN. AB Estimates of the newborn frequency of cystic fibrosis in different Caucasian groups range from 4 times more to 40 times less common than the generally accepted figure of 1:2000. Current meconium screening trials which may be effective in populations with the incidence equal to or greater than 1:2000, may be useful for populations with an incidence as low as 1:7000 only after maximum improvement of the methods. Once the true incidence or the variable incidence is proven for Caucasian populations, screening trails in Negro, Oriental and Indian populations will be required. RF 001 ANTONELLI M FRACASTORO (VERONA) 63 207 970 002 BECROFT DMO NZ MED J 68 113 968 003 BOCHKOVA DN PEDIATRIIA 12 24 974 004 BOZKOWA K PEDIATR POLSKA 46 677 971 005 BOZKOWA K PROC EWGCF 1ST ANNU MTG 970 006 BRUNECKY Z J MED GENET 9 33 972 007 BRUNECKY Z KINDERAERZTL PRAX 38 514 970 008 BRUNECKY Z CESK PEDIATR 25 74 970 009 CAIN ARR ARCH DIS CHILD 47 131 972 010 CARTER CO PHYSIOTHERAPY 61 240 975 011 CARTER CO MOD PROBL PEDIATR 10 372 967 012 CONNEALLY PM AMER SOC HUM GENET PROGRAM 42 966 013 CONNEALLY PM TEX REP BIOL MED 31 639 973 014 DANKS DM ANN HUM GENET 28 323 965 015 DIETZSCH HS PROC EWGCF 6TH ANNU MTG 975 016 FEINGOLD J CF Q ANNOTATED REFERENCES 13 15 974 017 FEINGOLD J ANN GENET (PARIS) 17 257 974 018 GALEN RS BEYOND NORMALITY THE PREDICTI 975 019 GILLY R ANN PEDIATR (PARIS) 20 5 973 020 GILLY R ARCH FR PEDIATR 28 49 971 021 GOTTSCHALK B PROC INT CF CONG 7TH 38 976 022 GURSON CT HELV PAEDIATR ACTA 28 165 973 023 HALL BD J MED GENET 5 262 968 024 HALL BD IN: LAWSON D PROC 5TH INT CF 82 969 025 HANNA BL IN: US PUB HEAL SERV PUB1163 7 963 026 HONEYMAN MS AM J HUM GENET 17 461 965 027 HONEYMAN MS CONN HLTH BULL 75 275 961 028 HOUSTEK J REV MED LIEGE 22 421 967 029 HOUSTEK J CESK PEDIATR 17 445 962 030 HOUSTEK J CAS LEK CES 104 201 965 031 KAISER D PROC INT CF CONG 7TH 33 976 032 KOLLBERG H PROC EWGCF 1ST ANNU MTG 970 033 KOLLBERG H PROC INT CF CONG 7TH 32 976 034 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 035 KULCZYCKI LL AM J DIS CHILD 100 174 960 036 LEVIN S IN: GOLDSCHMIDT E 294 963 037 MERRITT AD J LAB CLIN MED 60 998 962 038 MIGLIORI V MINERVA MED 64 4906 973 039 NEVANLINNA HR HEREDITAS 71 195 972 040 NIELSEN EL UGESKR LAEGER 133 1017 971 041 NIELSEN EL ACTA PAEDIATR SCAND 61 377 972 042 OREILLY D INT J EPIDEMIOL 3 247 974 043 PROSSER R ARCH DIS CHILD 49 597 974 044 PUGH RJ ARCH DIS CHILD 42 544 967 045 RACHINSKU SU PEDIATRIIA 3 60 973 046 REIDERMAN MI GENETIKA (SOV GENETICS) 7 148 971 047 RIGHETTI ABB PROC INT CF CONG 7TH 36 976 048 ROBERTS GBS ANN HUM GENET 24 127 960 049 ROBINSON PG PROC INT CF CONG 7TH 40 976 050 ROJO M PROC INT CF CONG 7TH 36 976 051 SELANDER P ACTA PAEDIATR SCAND 51 65 962 052 SIMANKOVA N ANN GENET (PARIS) 7 84 964 053 STEINBERG AG IN: GOLDSCHMIDT E 133 963 054 STEINBERG AG AM J HUM GENET 12 416 960 055 SULTZ HA AM J PUBLIC HEALTH 56 1461 966 056 SULTZ HA AM J PUBLIC HEALTH 58 491 968 057 SUPER M S AFR MED J 49 818 975 058 TEN KATE LP THESIS 975 059 TEN KATE LP INT J EPIDEMIOL 6 23 977 061 TODD RM MOD PROBL PEDIATR 10 387 967 062 TRIMBLE BK ANN HUM GENET 38 199 974 063 WRIGHT SW AM J HUM GENET 20 157 968 064 WRIGHT SW HAWAII MED J 27 229 968 065 ZYCHOWICZ C PEDIATR POLSKA 40 289 965 CT 1 ALLAN JL AUST PAEDIATR J 16 270 980 2 JONES MB J CHRON DIS 33 697 980 3 ROMEO G RIV ITAL PEDIATR 7 201 981 4 WARWICK WJ MINN MED 64 553 981 5 KLINGER KW HUM GENET 65 94 983 6 MCCLURE HM ADV VET SCI COMP MED 28 267 984 7 KARJOO M J TROP PEDIATR 30 195 984 8 ALLAN JL ACTA PAEDIATR SCAND 74 286 985 9 KNOPFLE G KLIN PAEDIATR 197 481 985 10 KNOPFLE G KLIN PAEDIATR 197 13 985 11 GEDSCHOLD J HUM GENET 75 277 987 PN 78091 RN 00872 AN 78149805 AU Seaman-W-B. TI The case of the abnormal duodenum. SO Hosp-Pract. 1978 Mar. 13(3). P 80-1. MJ CYSTIC-FIBROSIS: co. DUODENAL-DISEASES: ra. MN ADULT. CHILD. DUODENAL-DISEASES: et. HUMAN. MALE. EX Intermittent epigastric pain, partially relieved by food, was the reason for examining the upper gastrointestinal tract in a 30-year-old man. The second portion of the duodenum was strikingly abnormal. The patient had a history of daily, multiple loose stools since birth. At age 12 a diagnosis of small bowel malabsorption due to fibrocystic disease of the pancreas was established. In view of this history, the duodenal changes were considered to be a manifestation of fibrocystic disease of the pancreas. At the present time the cause of these duodenal abnormalities is unknown. RF 001 TAUSSIG LM RADIOLOGY 106 369 973 002 BERK RN RADIOLOGY 106 377 973 CT 1 MABOGUNJE OA SURGERY 90 114 981 PN 78092 RN 00873 AN 78149840 AU Hosli-P. Vogt-E. TI Reliable detection of cystic fibrosis in skin-derived fibroblast cultures. SO Hum-Genet. 1978 Mar 17. 41(2). P 169-73. MJ ALKALINE-PHOSPHATASE: bi. CYSTIC-FIBROSIS: di. FIBROBLASTS: en. MN CELLS-CULTURED. ENZYME-INDUCTION. GLYCOPROTEINS. HETEROZYGOTE. HUMAN. ISOPROTERENOL. PREGNANCY. PRENATAL-DIAGNOSIS. SKIN: cy. THEOPHYLLINE. AB The specific induction of alkaline phosphatase with Tamm-Horsfall glycoprotein, isoproterenol, and theophylline in skin-derived fibroblast cultures from patients with cystic fibrosis permits one to reliably discriminate between cystic fibrosis patients on the one hand, and heterozygotes and normals on the other. It was found the fibroblast-like and intermediary types of amniotic fluid-derived cells behave essentially like skin-derived fibroblasts. These findings imply that if different amniotic fluid cell types can be reliably separated, prenatal diagnosis of cystic fibrosis should become feasable in the near future. RF 001 BOXER LA N ENGL J MED 295 1041 976 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 HOSLI P TISSUE CULTIVATION ON PLASTIC 972 004 HOSLI P IN: MOTULSKY AG 226 974 005 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 006 HOSLI P ADV EXP MED BIOL 68 1 976 007 HOSLI P CLIN CHEM 23 1476 977 008 HOSLI P HUM HERED 27 185 977 CT 1 HOSLI P ACTA PAEDIATR SCAND 67 617 978 2 HOSLI P MONOGR PAEDIATR 10 90 979 3 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 4 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 7 AITKEN DA J MED GENET 17 187 980 8 FASTH A ACTA PAEDIATR SCAND 69 189 980 9 STRATTON CJ J CLIN CHEM CLIN BIOCHEM 19 852 981 10 HARRIS A CLIN GENET 20 315 981 11 JAKEL HP BIOL ZENTRALBL 100 273 981 12 HOSLI P EUR J PEDIATR 137 116 981 13 CEDER O SCANN ELECTRON MICROSC 1982 723 982 14 LEGGE M CHIN MED J 97 671 984 15 AITKEN DA PRENAT DIAGN 5 119 985 16 PINO JA ANAL CHEM 57 295 985 PN 78093 RN 00874 AN 78192839 AU Schiotz-P-O. Hoiby-N. Morling-N. Sorensen-H. TI C3 polymorphism in a Danish cystic fibrosis population and its possible association with antibody response. SO Hum-Hered. 1978. 28(4). P 293-300. MJ ANTIBODIES-BACTERIAL. COMPLEMENT-3: ge. CYSTIC-FIBROSIS: im. POLYMORPHISM-GENETICS. MN ADOLESCENCE. ADULT. ANTIBODIES-BACTERIAL: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg. DENMARK. FEMALE. GENE-FREQUENCY. HUMAN. INFANT. INFANT-NEWBORN. MALE. PHENOTYPE. PSEUDOMONAS-INFECTIONS: im. AB The C3 types of human serum are reported for a material of 113 Danish cystic fibrosis patients, age 0-30 years. The frequency of the C3F gene was 0.2832 which was significantly higher (p less than 0.0005) than the frequency found in a control group of 224 healthy babies (C3F = 0.1585). It also differed significantly (p less than 0.01) from the C3F gene frequency of 0.1780 found in 177 blood donors, age 20-24 years. A significant association between any of the C3 phenotypes and the most serious infection in cystic fibrosis, chronic mucoid P. aeruginosa infection, or the antibody response against these bacteria was not found. RF 001 BRONNESTAM R HUM HERED 23 206 973 002 BRONNESTAM R HUM HERED 23 214 973 003 BURNS MW LANCET 1 270 968 004 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 005 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 006 DOGGETT RG INFECT IMMUN 6 628 972 007 FARHUD DD HUMANGENETIK 17 57 972 008 GIBSON LE PEDIATRICS 23 545 959 009 HOFF GE SCAND J INFECT DIS 6 333 974 010 HOLZHAUER RJ AM J HUM GENET 28 602 976 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 015 HOIBY N SCAND J RESPIR DIS 58 65 977 016 LEDERBERG S AM J HUM GENET 28 597 976 017 SHWACHMAN H CLIN PEDIATR 14 1115 975 018 SORENSEN H HUM HERED 25 279 975 019 SORENSEN H HUM HERED 25 284 975 020 TEISBERG P VOX SANG 19 47 970 CT 1 SCACCHI R HUM HERED 31 183 981 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 3 BERNAL JE HUM HERED 36 97 986 4 SCHUR PH ANNU REV MED 37 333 986 PN 78094 RN 00875 AN 79217453 AU Boat-T-F. Cheng-P. TI Mucous glycoproteins in cystic fibrosis. pp. 108-21. SO In: Walborg EF, ed. Glycoproteins and glycolipids in disease processes. Washington, American Chemical Society, 1978. QU 55 S9801g 1978. (REVIEW). MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: me. MUCUS: me. MN ANIMAL. CARBOHYDRATES: an. CELLS-CULTURED. HUMAN. MONOGRAPH. RABBITS. REVIEW. SULFURIC-ACIDS: me. TRACHEA: me. SUPPORT-U-S-GOVT-P-H-S. EX Cystic fibrosis, the most common lethal genetic trait of Caucasians, causes much of the chronic progressive lung disease in children and young adults. It is also responsible for intestinal obstruction at birth (meconium ileus), pancreatic insufficiency with maldigestion and failure to grow, and obstructive liver disease in this age group. Furthermore, obliteration of the vas deferens causes aspermia and infertility in most males with CF. Individuals with CF secrete sweat which contains excessive amounts of chloride and sodium. Diagnostic criteria include detection of elevated chloride levels in sweat of individuals with typical pulmonary, gastrointestinal, or genital manifestations of the disease. There are several, well documented alterations in the mucous secretions of individuals with cystic fibrosis, any of which alone or in combination, may interfere with mucous clearance from the lung or from passageways of other organs. It seems clear that additional studies to define basic aspects of normal mucous biochemistry, physiology and pharmacology must precede or parallel studies of CF mucous secretions if data pertinent to CF pathophysiology are to be generated. Particular needs include an understanding of the chemical determinants of physical and rheological properties of these secretions, and elucidation of mechanisms for control of sulfated glycoprotein biosynthesis. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 MAYO BJ TEX REP BIOL MED 34 107 976 004 CONOD EJ PEDIATR RES 11 45 977 005 MANGOS JA PEDIATR RES 2 378 968 006 DEARBORN DG IN: MORRIS D 273 978 007 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 008 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 009 RAO GJS PEDIATR RES 9 739 975 010 SHAPIRA E J BIOL CHEM 252 7923 977 011 DAVIS SS BULL PHYSIOPATH RESPIR NANCY 9 47 973 012 POTTER JL AM REV RESPIR DIS 99 909 969 013 KHAN MA BIOCHIM BIOPHYS ACTA 444 369 976 014 REID L MOD PROBL PEDIATR 10 195 967 015 ZUELZER WW PEDIATRICS 4 53 949 016 REID L IN: PORTER R 32 45 968 017 OPPENHEIMER EH ARCH PATHOL 96 149 973 018 BOWMAN BH SCIENCE 164 325 969 019 BOGART BI PEDIATR RES 11 131 977 020 CZEGLEDY-NAGY E LAB INVEST 35 588 976 021 BOAT TF AM REV RESPIR DIS 110 428 974 022$ BAKER AP GAP CONF REP 976 023 BOAT TF MOD PROBL PEDIATR 19 141 977 024 BAKER AP AM REV RESPIR DIS 115 811 977 025 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 026 BOAT TF IN: MANGOS JA 165 976 027 NEUTRA MR LAB INVEST 36 535 977 028 STURGESS JM CLIN SCI 43 533 972 029 DEARBORN DG IN: MANGOS JA 179 976 030 WOOD RE AM REV RESPIR DIS 111 733 975 031 FORSTNER JF PEDIATR RES 10 609 976 032 GUGLER EC J PEDIATR 71 585 967 033 BOAT TF PEDIATR RES 8 531 974 034 BAILLEUL V CLIN CHIM ACTA 74 115 977 035 DEGAND P BULL PHYSIOPATH RESPIR NANCY 9 199 973 036 ROBERTS GP ARCH BIOCHEM BIOPHYS 173 528 976 037 SHIH CK AM REV RESPIR DIS 115 989 977 038 SNARY D BIOCHEM BIOPHYS RES COMMUN 40 844 970 039 HILL HD J BIOL CHEM 252 3791 977 040 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 041 ROUSSEL P J BIOL CHEM 250 2114 975 042 CHENG PW FED PROC 35 1444 976 043 LOPEZ-VIDRIERO MT IN: BRAIN JD 289 977 044 GOTTSCHALK A BIOCHIM BIOPHYS ACTA 46 91 961 045 MEYER FA BIOCHIM BIOPHYS ACTA 392 223 975 046 DEMAN J BIOCHIM BIOPHYS ACTA 297 486 973 048 LAMBLIN G BULL EUR PHYSIOPATH RESP 13 175 977 050 DISCHE Z PEDIATRICS 24 74 959 051 ABLETT S IN: RESING H 344 976 052 JEFFERY PK IN: BRAIN JD 5 193 977 053 BOAT TF IN: MANGOS JA 165 976 055 CHENG PW ANAL BIOCHEM 85 276 978 056 BOAT TF CHEST 67 325 975 CT 1 MORIARTY CM J MED 13 257 982 2 LITIN BS BIOL NEONATE 42 228 982 3 BENYOSEPH Y BIOCHIM BIOPHYS ACTA 718 172 982 4 SLOMIANY A J BIOL CHEM 258 8535 983 5 RUDICK VL J CELL PHYSIOL 118 67 984 PN 78095 RN 00876 AN 78241012 AU Sahu-S. Lynn-W-S. TI Lipid composition of sputum from patients with asthma and patients with cystic fibrosis. SO Inflammation. 1978 Mar. 3(1). P 27-36. MJ ASTHMA: me. CYSTIC-FIBROSIS: me. LIPIDS: an. SPUTUM: an. MN ADOLESCENCE. ADULT. CHILD. FATTY-ACIDS: an. GLYCOLIPIDS: an. HUMAN. PHOSPHOLIPIDS: an. PULMONARY-SURFACTANT: an. SUPPORT-U-S-GOVT-P-H-S. AB Lipids from the sputum of patients with asthma and with cystic fibrosis were isolated and characterized. In both cases, lipids constituted approximately 30% of the dry material. Phosphatidlycholine was the most abundant lipid, Significant amounts of phosphatidylethanolamine and phosphatidylglycerol were present. Hexosyl ceramides, sphingomyelin, phosphatidylinositol, lysophosphatidylcholine, and lysophosphatidylethanolamine were present as minor lipid components. Apperciable quantities of neutral lipids were present, of which triglycerides and cholesterol were the main constituents. Phosphatidylcholine, sphingomyelin, and phosphatidylgycerol were highly saturated. Large amounts of phosphatidylcholine containing mostly palmitic acid, particularly in the asthmatic sputum, suggest htat this highly saturated. Large amounts of phosphatidylcholine containing mostly palmitic acid, particularly in the asthmatic sputum, suggests that this highly saturated phospholipid is synthesized in the upper airways for reasons other than its beneficial surface-active properties in the alveoli. RF 001 CHERNICK WS PEDIATRICS 24 739 959 002 MATTHEWS LW AM REV RESPIR DIS 88 199 963 003 RAMIREZ RJ AM J MED 45 502 968 004 LEWIS RW LIPIDS 6 859 971 005 SAHU S AM REV RESPIR DIS 114 177 976 006 SAHU S AM REV RESPIR DIS 115 233 977 007 GALLAI-HATCHARD JJ BIOCHIM BIOPHYS ACTA 116 532 966 008 ADAMS EP CHEM PHYS LIPIDS 1 368 967 009 FOLCH J J BIOL CHEM 226 497 957 010 ROUSER G IN: MARINETTI GV 1 99 967 011 ESSELMAN WJ METHODS ENZYMOL 28 140 972 012 VIOQUE E J AM OIL CHEM SOC 39 63 962 013 SKIPSKI VP METHODS ENZYMOL 14 530 969 014 DITMER JC METHODS ENZYMOL 14 482 969 015 ROONEY SA J LIPID RES 16 418 975 016 TARLOV AR J BIOL CHEM 240 49 965 017 BARTLETT GR J BIOL CHEM 234 466 959 018 FARKAS J J LIPID RES 14 344 973 019 VANHANDEL E J LAB CLIN MED 50 152 957 020 RUDEL LL J LIPID RES 14 364 973 021 LAUTER CJ J LIPID RES 3 136 962 022 TAO RVP J LIPID RES 14 16 973 023 BAXTER CF LIPIDS 4 243 969 024 HEPPLESTON AG BR J EXP PATHOL 55 384 974 025 BODY DR LIPIDS 6 625 971 026 HURST DJ RESPIR PHYSIOL 17 72 973 027 ROONEY SA BIOCHIM BIOPHYS ACTA 360 56 974 028 GRAY GM BIOCHIM BIOPHYS ACTA 144 519 967 029 HENDERSON RF LIPIDS 7 492 972 030 KING RJ AM J PHYSIOL 224 788 973 031 GODINEZ RI BIOCHEMISTRY 14 830 975 032 HALLMAN M J LIPID RES 17 257 976 033 KLAUS MH PROC NAT ACAD SCI USA 47 1858 961 034 CLEMENTS JA SCIENCE 169 603 970 CT 1 GALABERT C CLIN RESP PHYSIOL 17 197 981 2 HARPER TB AM REV RESPIR DIS 126 540 982 3 SLAYTER HS EUR J BIOCHEM 142 209 984 PN 78096 RN 00877 AN 79129714 AU Ten-Kate-L-P. Feenstra-de-Gooyer-I. Ploeg-de-Groot-G. Gouw-W-L. Anders-G-J. TI Should we screen all newborns for cystic fibrosis?. SO Int-J-Epidemiol. 1978 Dec. 7(4). P 323-30. MJ CYSTIC-FIBROSIS: oc. MASS-SCREENING. MN ALBUMINS: an. ATTITUDE-OF-HEALTH-PERSONNEL. FALSE-NEGATIVE-REACTIONS. FALSE-POSITIVE-REACTIONS. HUMAN. INFANT-NEWBORN. MECONIUM: an. NETHERLANDS. PEDIATRICS. QUESTIONNAIRES. AB To assess the value of detecting albumin in meconium as a screening procedure for cystic fibrosis [CF] 68,000 meconium samples were examined by BM Meconium Test, single radial immunodiffusion and benzidine reaction. The specificity and sensitivity of this combination of tests were 99.67% and 78.57% respectively. The prevalence of CF at birth was confirmed as 1:3600 in this country. This low prevalence resulted in a relatively high number of false positives. Therefore, a positive test result has a low predictive value [3.39%] and this is a serious drawback of the method. The experiences and opinions of 37 local paediatricians about the screening programme were evaluated by a simple questionnaire. Gold's decision rule was applied. The least relative cost of misclassification justifying a mass-screening programme was 3 times higher than the actual relative cost as suggested by the aggregate opinion of paediatricians in the region. These results support the view that with the methods used screening may have more disadvantages than not screening. RF 001 TEN KATE LP INT J EPIDEMIOL 6 23 977 002 BRIMBLECOMBE FSW LANCET 2 1428 973 003 GOLD RJM ANN HUM GENET 37 315 974 004 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 005 BULL FE ARCH DIS CHILD 49 602 974 006 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 007 STEPHAN U PEDIATRICS 55 35 975 008 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 009 PROSSER R ARCH DIS CHILD 49 597 974 010 GALEN RS BEYOND NORMALITY THE PREDICTI 975 011 TINSCHMANN P PROC EWGCF 5TH ANNU MTG 974 012 GRIFFITHS AD ARCH DIS CHILD 51 321 976 013 HUANG NN AM J DIS CHILD 120 289 970 014 SHWACHMAN H PEDIATRICS 46 335 970 015 WOOD RE AM REV RESPIR DIS 113 833 976 016 ORENSTEIN DM AM J DIS CHILD 131 973 977 017 RYLEY HC CLIN CHIM ACTA 64 117 975 018 ANTONOWICZ I PEDIATRICS 56 782 975 019 ANTONOWICZ I LANCET 1 746 976 020 CROSSLEY JR LANCET 2 1093 977 CT 1 ANDERS GJPA J GENET HUM 29 23 981 2 SZIBOR R Z KLIN MED 40 313 985 3 DANKERTROELSE JE LANCET 1 326 986 4 DANKERTROELSE JE ACTA PAEDIATR SCAND 76 209 987 PN 78097 RN 00878 AN 79108929 AU Ekvall-S. Mitchell-A. TI The effect of supplemental vitamin E on vitamin A serum levels in cystic fibrosis. SO Int-J-Vitam-Nutr-Res. 1978. 48(4). P 324-32. MJ CYSTIC-FIBROSIS: dt. VITAMIN-A: bl. VITAMIN-E: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CREATINE: ur. CREATININE: ur. CYSTIC-FIBROSIS: me, ur. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. MALE. VITAMIN-A: tu. VITAMIN-E: bl. AB Effects of the ingestion of vitamin E with vitamin A on the serum levels of these vitamins and the urinary creatine to creatinine ratios of 18 children with cystic fibrosis disease of the pancreas (CF) was studied. After the daily ingestion for two months of a specially prepared capsule containing 5000 IU vitamin A palmitate and 100 mg d alpha-tocopherol acetate in aqueous dispersible form there was a significant increase in vitamin A as well as vitamin E levels of serum taken 3 1/2 hr postprandially. Creatine to creatinine ratios in single voiding of urine decreased. RF 001 BAKER H AM J CLIN NUTR 20 850 967 002 BAUERNFEIND JC AM J CLIN NUTR 27 234 974 003 BIERI JG PROC SOC EXP BIOL MED 117 131 964 004 BLANC WA PEDIATRICS 22 494 958 005 BONSNES RW J BIOL CHEM 158 581 945 006 CLARK LC JR AM J DIS CHILD 81 774 951 007 DAVIES AW NATURE 147 794 941 008 GOLDBLOOM RB PEDIATRICS 32 36 963 009 GORDON HH PEDIATRICS 21 673 958 010 GREEN J NUTR ABSTR REV 38 321 969 011 KATZ LN EXPERIMENTAL ATHEROSCLEROSIS 953 012 KERNER I AM J DIS CHILD 99 597 960 013 LEWIS JS AM J CLIN NUTR 26 136 973 014 MAJAJ AS AM J CLIN NUTR 12 374 963 015 MEDWADOWSKI B THESIS 964 016 NEELD JB J NUTR 79 454 963 017 NITOWSKY HM AM J CLIN NUTR 10 368 962 018 RIVERS JPW LANCET 2 642 975 019 RODRIQUEZ MS J NUTR 102 908 972 020 UNDERWOOD BA BULL NY ACAD MED 47 34 971 021 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 022 WO CKW AM J CLIN NUTR 28 808 975 CT 1 WOODRUFF CW AM J CLIN NUTR 44 384 986 PN 78098 RN 00879 AN 78170950 AU OHalloran-E-T. TI Cystic fibrosis--an eleven year survey. SO Ir-Med-J. 1978 Apr 21. 71(6). P 184-7. MJ CYSTIC-FIBROSIS. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, oc, th. FEMALE. HUMAN. INFANT. IRELAND. MALE. PROGNOSIS. TIME-FACTORS. EX The purpose of this communication is to review our experiences with children with cystic fibrosis seen from January 1964 to January 1975 and to analyse the factors which have contributed to the improved prognosis which has occurred in this period. The numbers of new patients have increased from six in 1964 to 12 in 1974. Both sexes have been equally affected. Respiratory infections, failure to thrive and diarrhoea were the predominant presenting features in that order, and thus any one of these presenting features should indicate that cystic fibrosis should at least be considered and excluded. Each patient was assessed monthly at the clinic during which attendance formal physiotherapy was carried out and the parents were instructed in the techniques of percussion and drainage. Forty-two of our sixty-four patients are still alive and thirty-three of those fall into the excellent category, seven into the good and one each into the fair and poor. During the period of this review there has been an obvious improvement with a fall in morbidity and mortality and with the increasing proportion of patients now in the "excellent" group. We attribute these facts to earlier diagnosis, regular and careful follow-up and a supervised therapeutic programme including parent education and participation. It would seem, therefore, that early diagnosis and active treatment prolong life and give better health. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH PEDIATRICS 3 406 949 003 DODGE JA BR MED J 2 192 975 004 GEORGE L ARCH DIS CHILD 46 139 971 005 MITCHELL-HEGGS PF Q J MED 45 479 976 006 MEARNS MB ARCH DIS CHILD 46 139 972 007 ROBINSON MJ ARCH DIS CHILD 50 962 975 008 SHWACHMAN H ADV PEDIATR 7 249 955 009 SHWACHMAN H PEDIATRICS 46 335 970 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 WARWICK WJ MOD PROBL PEDIATR 10 353 967 PN 78099 RN 00880 AN 78170976 AU Katznelson-D. Ben-Yishay-M. TI Cystic fibrosis in Israel: clinical and genetic aspects. SO Isr-J-Med-Sci. 1978 Feb. 14(2). P 204-11. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. AUTOPSY. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, fg, oc. ETHNIC-GROUPS. FAMILY-PLANNING. GENETIC-COUNSELING. HUMAN. INFANT. ISRAEL. AB A total of 140 cases of cystic fibrosis in 110 families was identified in Israel during the years 1946--75, and there was reason to suspect inadequate ascertainment. Cystic fibrosis is frequent among the Ashkenazic and Arab populations with an incidence approximating that for Caucasians of European ancestry (1:1,800 to 1:4,000 live births). It is about half as frequent in the Sephardim, rare in the Iraqi or Yemenite Jews and perhaps absent in Iranian Jews. The clinical picture of cystic fibrosis in Israel in all the ethnic groups was found to be the same as elsewhere in the world. In- depth.interviews of 50 families were conducted to assess their understanding of the genetics of the disease and their attitude toward family planning. RF 001 LEVIN S IN: GOLDSCHMIDT E 294 963 002 STEPHAN U PEDIATRICS 55 35 975 003 PUGH RJ ARCH DIS CHILD 42 544 967 004 ANON FACTS ABOUT ISRAEL 1975 975 005 ANON STATISTICAL ABST OF ISRAEL 975 006 GOLDSCHMIDT AE ANN HUM GENET 24 191 960 007 BEARN AG CLIN RES 17 462 969 008 SHWACHMAN H PEDIATRICS 46 335 970 009 WING JP HUM HERED 24 323 974 010 ADAM A ISR J MED SCI 9 1383 973 011 CARTER CO LANCET 1 281 971 012 LEONARD CO N ENGL J MED 287 433 972 CT 1 SCHAAP T ISR J MED SCI 14 201 978 2 KATZNELSON D ISR J MED SCI 17 687 981 3 KATZNELSON D LANCET 1 112 982 4 HOLLANDER DH MED HYPOTHESES 8 191 982 5 STURGESS JM AM J MED GENET 22 383 985 6 KOLLBERG H J TROP PEDIATR 32 293 986 7 NAZER H TRANS R SOC TROP MED HYG 80 348 986 PN 78100 RN 00881 AN 78170975 AU Schaap-T. Hodes-M-E. Cohen-M-M. TI Cystic fibrosis--problems and prospects [editorial]. SO Isr-J-Med-Sci. 1978 Feb. 14(2). P 201-3. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: di. HUMAN. LINKAGE-GENETICS. RESEARCH. EX Cystic fibrosis, an autosomal recessive trait, is the most common hereditary lethal condition among Caucasians, approximating a prevalence of 1 per 2500 births with a carrier frequency of 1:25 in populations originating from West Europe. The markedly elevated frequency of CF in a considerable proportion of the human race presents a genetic enigma for which several solutions have been proposed. Genetic heterogeneity, heterozygote advantage, reduced penetrance, genetic drift, and linkage or epistasis are discussed. The basic defect in CF and its fundamental biochemical and physiological ramifications are unknown. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 BEARN AG CLIN GASTROENTEROL 2 515 973 003 CONNEALLY PM TEX REP BIOL MED 31 639 973 004 KATZNELSON D ISR J MED SCI 14 204 978 005 POLLEY MJ J MED GENET 11 249 974 006 DANES BS BIRTH DEF ORIG ART SER 8 114 972 007 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 008 FEINGOLD J ANN GENET (PARIS) 17 257 974 009 CROW JF IN: NEEL JV 23 965 010 STUART AB LANCET 2 1521 974 011 SUPER M S AFR MED J 49 818 975 012 RAO DC AM J HUM GENET 25 594 973 013 WAGENER DK AM J HUM GENET 27 348 975 014 MANGOS JA PEDIATR RES 1 436 967 015 SPOCK A PEDIATR RES 1 173 967 016 BOWMAN BH SCIENCE 164 325 969 017 WOOD RE LANCET 2 1452 973 018 BENKE PJ IN: MANGOS JA 157 976 019 WILSON GB CLIN CHIM ACTA 49 79 973 020 THOMAS JM PEDIATR RES 11 1148 977 021 RAO GJS PEDIATR RES 9 739 975 022 CHAN KYH CLIN CHIM ACTA 74 71 977 023 SHAPIRA E PEDIATR RES 10 812 976 PN 78101 RN 00882 AN 79067429 AU Pinney-M-A. TI Review of cystic fibrosis. SO Issues-Compr-Pediatr-Nurs. 1978 Aug. 3(2). P 54-65. MJ CYSTIC-FIBROSIS. MN CHILD. CYSTIC-FIBROSIS: di, th. HUMAN. PARENTS. PEDIATRIC-NURSING. RESPIRATORY-THERAPY. EX Cystic fibrosis is an inherited disease affecting mainly Caucasians. All exocrine glands are obstructed, causing multiple manifestations. The most lethal aspect is the pulmonary involvement. Early diagnosis and treatment influence prognosis. The sweat test is the diagnostic tool; it must be done in a center where it is performed frequently and where quality control is available. Specific therapy varies from center to center, but all agree on a consistent, comprehensive approach to management. One must be acutely aware of the psychosocial aspects when caring for children with cystic fibrosis. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 003 LEVISON H IN: MANGOS JA 3 976 004 WAGGET J J PEDIATR 77 407 970 006 GIBSON LE PEDIATRICS 23 545 959 007 MELLINS RB AM REV RESPIR DIS SUPPL 110 137 974 009 WARING WW ADV PEDIATR 23 401 976 010 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 011 DROTUR DA PEDIATRICS 56 710 975 013 MATTSSON A PEDIATRICS 50 801 972 014 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 015 DOOLEY RR AM J OBSTET GYNECOL 118 971 974 PN 78102 RN 00883 AN 78067357 AU Reitman-A-A. Faber-R-D. TI A cephalometric study of patients with cystic fibrosis. SO J-Am-Dent-Assoc. 1978 Jan. 96(1). P 83-7. MJ CYSTIC-FIBROSIS. MAXILLOFACIAL-DEVELOPMENT. MN ADOLESCENCE. ADULT. CEPHALOMETRY. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. HUMAN. MALE. SKULL: gd. AB Children with cystic fibrosis have skeletal maturation and epiphyseal anomalies similar to those in children with nutritional problems. However, these patients have been found to have normal somatic development if treatment is begun early. In a group of children with cystic fibrosis undergoing medical treatment, cephalometric measurements compared favorably with those established by normal standards. RF 001 HUANG NN AM J DIS CHILD 120 289 970 002 SHWACHMAN H AM J DIS CHILD 96 6 958 003 GORLIN RJ THOMAS ORAL PATHOLOGY 995 970 004 SPROUL A J PEDIATR 65 664 964 005 KREISSL T Z KINDERHEILK 113 93 972 006 GREEN OC ENDOCRINOL METAB J CLIN 27 1059 967 007 MILUNSKY A AM J DIS CHILD 121 15 971 008$ DENNIS JL JAMA 94 855 965 009 SNODGRASS RM AM J ROENTG RAD THER NUCL MED 74 6 955 010 TAUSSIG LM AM J DIS CHILD 125 495 973 PN 78103 RN 00884 AN 78217979 AU Govan-J-R. Fyfe-J-A. TI Mucoid Pseudomonas aeruginosa and cystic fibrosis: resistance of the mucoid from to carbenicillin, flucloxacillin and tobramycin and the isolation of mucoid variants in vitro. SO J-Antimicrob-Chemother. 1978 May. 4(3). P 233-40. MJ ANTIBIOTICS: pd. CARBENICILLIN: pd. CLOXACILLIN: aa. CYSTIC-FIBROSIS: mi. FLOXACILLIN: pd. PSEUDOMONAS-AERUGINOSA: ip. TOBRAMYCIN: pd. MN CHEMISTRY-PHYSICAL. DRUG-RESISTANCE-MICROBIAL. HUMAN. POLYSACCHARIDES-BACTERIAL: an. PSEUDOMONAS-AERUGINOSA: de. AB Mucoid strains of Pseudomonas aeruginosa isolated from the sputa of patients with cystic fibrosis or in vitro through selection with phage were more resistant to carbenicillin, flucloxacillin and tobramycin and more sensitive to tetracycline than related non-mucoid strains. The observations led to the development of a technique for the isolation of mucoid variants in vitro based on enhanced resistance to carbenicillin. Mucoid variants were isolated from various strains of Ps. aeruginosa and the exopolysaccharide found to be similar to that obtained from mucoid Ps. aeruginosa isolated from patients with cystic fibrosis. RF 001 BRUMFITT W J ANTIMICROB CHEMOTHER 1 163 975 002 DOGGETT RG APPL MICROBIOL 18 936 969 003 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 004 DOGGETT RG J PEDIATR 68 215 966 005 EVANS LR J BACTERIOL 116 915 973 006 GOVAN JRW J MED MICROBIOL 8 513 975 007 GOVAN JRW J ANTIMICROB CHEMOTHER 2 215 976 008 GOVAN JRW IN: NORRIS JR 10 59 978 009 HOLLOWAY BW BACT REV 33 419 969 010 IACOCCA VF AM J DIS CHILD 106 315 963 011 LAWSON D IN: WATT PJ 69 970 012 MCCRAE WM J INFECT DIS SUPPL 134 191 976 013 MARKS MI J PEDIATR 79 822 971 014 MARTIN DR J MED MICROBIOL 6 111 973 015 MAY JR J MED MICROBIOL 6 77 973 016 PHILLIPS I J MED MICROBIOL 2 9 969 017 RAEBURN JA J ANTIMICROB CHEMOTHER 2 107 976 018 SONNENSCHEIN C ZENTRALBL BAKTERIOL 104 365 927 019 STANISICH V GENETICS 61 327 969 020 WILLIAMS RJ J MED MICROBIOL 6 409 973 021 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 HOLLOWAY BW MICROBIOL REV 43 73 979 2 GOVAN JRW J GEN MICROBIOL 110 229 979 3 THOMASSEN MJ J INFECT DIS 140 873 979 4 DEMKO CA CURRENT MICROBIOL 4 69 980 5 MARKOWITZ SM J ANTIMICROB CHEMOTHER 6 251 980 6 CHENG KJ CAN J MICROBIOL 26 1104 980 7 LAM J INFECT IMMUN 28 546 980 8 LIEBERMAN MM INFECT IMMUN 29 489 980 9 FYFE JAM J GEN MICROBIOL 119 443 980 10 BRISOU JF C R ACAD SCI (D)(PARIS) 290 1421 980 11 FICK RB CLIN CHEST MED 2 91 981 12 SLACK MPE LANCET 2 502 981 13 COSTERTON JW CRC CRIT REV MICROBIOL 8 303 981 14 IRVIN RT ANTIMICROB AGENTS CHEMOTHER 19 1056 981 15 CHENG KJ CAN J MICROBIOL 27 461 981 16 BLACKWOOD LL INFECT IMMUN 32 443 981 17 COSTERTON JW ANNU REV MICROBIOL 35 299 981 18 MARKS MI J PEDIATR 98 173 981 19 GOVAN JRW J GEN MICROBIOL 125 217 981 20 BERGAN T SCAND J INFECT DIS 1981 7 981 21 BAKER NR CURRENT MICROBIOL 7 35 982 22 SLACK MPE J ANTIMICROB CHEMOTHER 10 368 982 23 PIGGOTT NH EUR J APP MICROBIO BIOTECHNOL 16 131 982 24 SUTHERLAND IW ADV MICROB PHYSIOL 23 79 982 25 BOYCE JR INFECT IMMUN 37 840 982 26 OHMAN DE INFECT IMMUN 37 662 982 27 HOOKE AM INFECT IMMUN 38 136 982 28 MONTIE TC INFECT IMMUN 38 1296 982 29 EXNER M ZENTRALBL BAKT MIKROB HYG (B) 176 425 982 30 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 31 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 32 COSTERTON JW REV INFECT DIS 5 S867 983 33 GOVAN JRW REV INFECT DIS 5 S874 983 34 OMBAKA EA REV INFECT DIS 5 S880 983 35 COSTERTON JW PROG FOOD NUTR SCI 7 91 983 36 OLIVER AM J CLIN LAB IMMUNOL 10 221 983 37 TURNOWSKY F FEMS MICROBIOL LETTERS 17 243 983 38 HACKING AJ J GEN MICROBIOL 129 3473 983 39 DALHOFF A ZENTRALBL BAKT MIKROB HYG (A) 254 379 983 40 COSTERTON JW REV INFECT DIS 6 S608 984 41 GRISTINA AG ORTHOP CLIN NORTH AM 15 517 984 42 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 43 NACUCCHIO MC PEDIATR RES 18 295 984 44 CHAN R J CLIN MICROBIOL 19 8 984 45 WINGENDER J FEMS MICROBIOL LETTERS 21 63 984 46 COSTERTON JW DEVELOP IND MICROBIOL 25 363 984 47 TANNENBAUM CS ANTIMICROB AGENTS CHEMOTHER 25 673 984 48 LAMBE DW CAN J MICROBIOL 30 809 984 49 MILLS J INFECT IMMUN 43 359 984 50 HANSEN JB APPL ENVIRON MICROBIOL 47 704 984 51 VOGEL F DTSCH MED WSCHR 109 1148 984 52 FYFE JAM J GEN MICROBIOL 130 825 984 53 JARMAN TR ARCH MICROBIOL 137 231 984 54 GOLDBERG JB J BACTERIOL 158 1115 984 55 DARZINS A J BACTERIOL 159 9 984 56 LINKER A J BACTERIOL 159 958 984 57 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 58 MARTY N MED MALAD INFECT 15 604 985 59 SHABTAI J DEVELOP IND MICROBIOL 26 291 985 60 IRVIN RT CAN J MICROBIOL 31 268 985 61 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 33 515 985 62 SUTHERLAND IW ANNU REV MICROBIOL 39 243 985 63 MARCUS H INFECT IMMUN 47 723 985 64 SHABTAI Y APPL ENVIRON MICROBIOL 49 192 985 65 LUZAR MA INFECT IMMUN 50 577 985 66 KOMIYAMA K ORAL SURG 59 590 985 67 WINKLER U KLIN WSCHR 63 490 985 68 GRISTINA AG J BONE JOINT SURG 67A 264 985 69 DUMA RJ AM J MED 78 154 985 70 GRISTINA AG SURGERY 98 12 985 71 LORY S CURR TOP MICROBIOL IMMUNOL 118 53 985 72 PULLIAM L J INFECT DIS 151 153 985 73 DARZINS A J BACTERIOL 161 249 985 74 OHMAN DE J BACTERIOL 162 1068 985 75 GOVAN JRW MICROBIOL SCI 3 302 986 76 SCHMITT DD J VASC SURG 3 732 986 77 WEBB LX J VASC SURG 4 16 986 78 MCEACHRAN DW J MICROBIOL METH 5 99 986 79 FLOURNOY DJ METH FIND EXP CLIN PHARMACOL 8 391 986 80 FRIEND PA J INFECT 13 55 986 81 MACGEORGE J J MED MICROBIOL 21 331 986 82 KRIEG DP J CLIN MICROBIOL 24 986 986 83 PITT TL J ROY SOC MED 79 13 986 84 DERETIC V J BACTERIOL 165 510 986 85 DERETIC V BIO TECHNOL 5 469 987 86 GROSS M J PHYTOPATH 118 276 987 PN 78104 RN 00885 AN 78129864 AU Lewis-S-M. Evans-J-W. Jalowayski-A-A. TI Continuous distributions of specific ventilation recovered from inert gas washout. SO J-Appl-Physiol. 1978 Mar. 44(3). P 416-23. MJ NITROGEN. RESPIRATION. MN ADULT. AGING. CHILD. CYSTIC-FIBROSIS: pp. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: pp. LUNG: ph. MATHEMATICS. RESPIRATORY-DEAD-SPACE. TIDAL-VOLUME. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB We describe a new technique for recovering continuous distributions of ventilation (V) as a function of tidal ventilation/volume ratio (V/V0) from the nitrogen washout. The analysis yields a continuous distribution of V as a function of V/V0 represented as fractional ventilations of 50 compartments plus dead space. The procedure was verified by recovering known distributions from data to which noise had been added. Using an apparatus to control the subject's tidal volume and FRC, mixed expired N2 data gave the following results: a) the distributions of young, normal subjects were narrow and unimodal with a mean ln standard deviation of 0.56 plus or minus 0.13; b) those of subjects over age 40 were broader (ln SD 0.86 plus or minus 0.19) with more poorly ventilated units; c) patients with pulmonary disease of all descriptions showed enlarged dead space; d) patients with cystic fibrosis showed multimodal distributions with the bulk of the ventilation going to overventilated units; and e) patients with obstructive lung diseases fell into several classes, three of which are illustrated. These results suggest that our approach is well suited for clinical investigation. RF 001 BECKLAKE MR THORAX 7 111 952 002 BOUHUYS A PHYSIOL REV 39 731 959 003 EVANS JW BULL MATH BIOPHYS 32 59 970 004 EVANS JW BULL MATH BIOPHYS 29 711 967 005 EVANS JW J APPL PHYSIOL 42 889 977 006 FOWLER WS J APPL PHYSIOL 2 283 949 007 GOMEZ DM PROC NAT ACAD SCI USA 49 312 963 008 GOMEZ DM J APPL PHYSIOL 19 683 964 009$ GOMEZ DM J EXP MED 193 514 965 010 GOMEZ DM AM REV RESPIR DIS 93 1 966 011 HASHIMOTO T J APPL PHYSIOL 23 203 967 012 HEISE M RESPIRATION 31 310 974 013$ JALAWALA SA J CLIN INVEST 53 188 975 014 LAWSON CL SOLVING LEAST SQUARES PROBLEM 974 015 MARTIN CJ RESPIR PHYSIOL 21 157 974 016 NAKAMURA T J APPL PHYSIOL 21 227 966 017 NYE RE J APPL PHYSIOL 16 115 961 018 OKUBO T J APPL PHYSIOL 24 658 968 019 OLSZOWKA AJ RESPIR PHYSIOL 25 191 975 020 PESLIN R J APPL PHYSIOL 30 462 971 021 ROSSING RG J APPL PHYSIOL 21 1907 966 022 SCHWARZL F PHYSICA 18 791 952 023 TSUNODA S J APPL PHYSIOL 32 644 972 024 WAGNER PD J APPL PHYSIOL 36 588 974 025 YOUNG AC J CLIN INVEST 53 1178 974 CT 1 LEWIS SM J APPL PHYSIOL 44 424 978 2 BUTLER JP J APPL PHYSIOL 46 47 979 3 EVANS JW MATH BIOSCI 46 233 979 4 WAGNER PD J APPL PHYSIOL 46 579 979 5 MARTIN CJ J APPL PHYSIOL 47 319 979 6 LAMEDICA G RESPIRATION 39 333 980 7 WAGNER PD ANNU REV PHYSIOL 42 235 980 8 AZZARO SH IEEE TRANS BIOMED ENG 28 755 981 9 DEVRIES WR J APPL PHYSIOL 51 1122 981 10 LEWIS SM J APPL PHYSIOL 51 1463 981 11 PIMMEL RL J APPL PHYSIOL 51 1581 981 12 OZANNE GM ANESTHESIOLOGY 54 413 981 13 WAGNER PD BULL EUR PHYSIOPATH RESP 18 361 982 14 JALOWAYSKI AA RESPIRATION 43 249 982 15 LEWIS SM RESPIR PHYSIOL 50 111 982 16 HUTCHISON AA AM REV RESPIR DIS 125 28 982 17 BRONIKOWSKI TA INT J BIOMEDICAL COMPUT 14 411 983 18 MULLER E BULL EUR PHYSIOPATH RESP 19 P100 983 19 RUBIN DZ J APPL PHYSIOL 56 708 984 20 BATES JHT IEEE TRANS BIOMED ENG 32 478 985 21 BRUNNER JX J APPL PHYSIOL 59 1008 985 22 HARF A AM REV RESPIR DIS 132 350 985 23 BUCHANAN PR CLIN PHYS PHYSIOL MEASURE 7 237 986 24 CRAWFORD ABH J APPL PHYSIOL 61 2108 986 25 BATES JHT RADIAT RES 107 307 986 26 BUCHANAN PR AM REV RESPIR DIS 133 A384 986 27 BATES JHT MED BIOL ENG COMPUT 25 131 987 PN 78105 RN 00886 AN 78129865 AU Lewis-S-M. TI Emptying patterns of the lung studied by multiple-breath N2 washout. SO J-Appl-Physiol. 1978 Mar. 44(3). P 424-30. MJ MODELS-BIOLOGICAL. RESPIRATION. MN ADULT. AGING. CHILD. CYSTIC-FIBROSIS: pp. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: pp. LUNG-VOLUME-MEASUREMENTS. LUNG: ph, pp. NITROGEN. RESPIRATORY-DEAD-SPACE. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB Changes in the nitrogen concentration seen during the single-breath nitrogen washout reflect changes in relative flow (V) from units with differing ventilation/volume ratios (V/V0). The multiple-breath washout provides sufficient data for the V for units with varying V/V0 to be plotted as a function of the volume expired. Flow from the dead space may also be determined. In young normals the emptying patterns are narrow and unimodal throughout the alveolar plateau with little or no flow from the dead space at the end of the breath. Older normals show more flow from the dead space, particularly toward the end of the breath, and some show a high V/V0 mode early in the breath. Patients with obstructive lung disease have a high flow from the dead space which is present throughout the breath. A well ventilated mode at the end of the breath is seen in some obstructed subjects. Patients with cystic fibrosis showed a poorly ventilated mode appearing at the end of the breath as well as a very high dead space. RF 001$ BAKER LG RESPIR PHYSIOL 21 119 974 002 BALL WC JR J CLIN INVEST 41 519 962 003 DOLLFUSS RE RESPIR PHYSIOL 2 234 967 004 ENGEL LA J APPL PHYSIOL 35 9 973 005 EVANS JW BULL MATH BIOPHYS 32 59 970 006 EVANS JW BULL MATH BIOPHYS 29 711 967 007 EVANS JW J APPL PHYSIOL 42 889 977 008 FOWLER WS AM J PHYSIOL 154 405 948 009 FOWLER WS J APPL PHYSIOL 2 283 949 010 FOWLER WS J CLIN INVEST 31 40 952 011 LEWIS SM J APPL PHYSIOL 44 416 978 012 MCCARTHY DS AM J MED 52 747 972 013 MARTIN CJ RESPIR PHYSIOL 21 157 974 014 MILLETTE B J APPL PHYSIOL 27 587 969 015 PAIVA M J APPL PHYSIOL 35 401 973 016 POWER G J APPL PHYSIOL 27 701 969 017 SCHERER PW BULL MATH BIOPHYS 34 393 972 018 SIKAND R J APPL PHYSIOL 21 1331 966 019 SUDA Y J APPL PHYSIOL 29 480 970 020 TSUNODA S J APPL PHYSIOL 32 644 972 021 WAGNER PD J APPL PHYSIOL 36 588 974 022 YOUNG AC J CLIN INVEST 53 1178 974 CT 1 LAMEDICA G RESPIRATION 39 333 980 2 JALOWAYSKI AA RESPIRATION 43 249 982 3 RUBIN DZ J APPL PHYSIOL 56 708 984 4 CUMMING G CLIN PHYSIOL 5 33 985 5 BUCHANAN PR CLIN PHYS PHYSIOL MEASURE 7 237 986 PN 78107 RN 00887 AN 79048678 AU Sparham-P-D. Lobban-D-I. Speller-D-C. TI Isolation of Staphylococcus aureus from sputum in cystic fibrosis. SO J-Clin-Pathol. 1978 Oct. 31(10). P 913-8. MJ CYSTIC-FIBROSIS: mi. SPUTUM: mi. STAPHYLOCOCCUS-AUREUS: ip. MN ADOLESCENCE. BACTERIOLOGICAL-TECHNICS. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CULTURE-MEDIA. HUMAN. PSEUDOMONAS-AERUGINOSA: ip. SONICATION. AB The success in the isolation of Staphylococcus aureus of different methods of sputum processing was investigated in 60 specimens collected from 14 patients with cystic fibrosis during a seven-month period. Fifty specimens (83%) from 11 patients yielded Staph. aureus by one or more methods. Direct plating of purulent portions of sputum on to media designed for general use in respiratory infections gave unsatisfactory results (35% yield of Staph. aureus). Some increase in isolations was obtained with preliminary liquefaction of sputum; but the best results were given by the addition of a medium selective for staphylococci (mannitol salt agar, BBL) or by initial sonication of sputum (each 83% yield). Seven of the 11 strains of Staph. aureus were thymidine-dependent and otherwise atypical in laboratory characteristics; these were isolated from patients who had received co-trimoxazole. RF 001 ALDER VG J APPL BACT 25 436 962 002 BABER KG J MED LAB TECH 26 391 969 003 CHRISTIE R J PATHOL BACTERIOL 51 189 940 004 CRUICKSHANK R MEDICAL MICROBIOLOGY 2 975 005 GEORGE RH LANCET 2 1081 977 006 HUHTANEN CN J DAIRY SCI 49 1008 966 007 MCINTOSH AF PROCESS BIOCHEM 6 22 971 008 MAY JR ARCH DIS CHILD 47 908 972 009 MEAD GR TUBERCLE 45 370 964 010 MEARNS MB ARCH DIS CHILD 47 902 972 011 MILES AA J HYGIENE 38 732 938 012 PHILLIPS I J MED MICROBIOL 2 9 969 013 RAWLINS GA LANCET 2 538 953 014 WILKINSON PJ J CLIN PATHOL 30 417 977 015 WILLIAMS RF IN: WILLIAMS RF 20 977 CT 1 WILLIAMS RF CURR MED RES OPIN 6 43 979 2 HOWDEN R MED LAB SCI 38 29 981 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 4 FRIEND PA J INFECT 13 55 986 PN 78108 RN 00888 AN 78152669 AU Morris-L-J. Mascia-A-V. Farnsworth-P-B. TI Cystic fibrosis: making a correct and early diagnosis. SO J-Fam-Pract. 1978 Apr. 6(4). P 749-56. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. CASE-REPORT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, fg. DIABETES-MELLITUS: co. DIAGNOSIS-DIFFERENTIAL. FEMALE. HUMAN. INFANT. MALABSORPTION-SYNDROMES: di. MALE. RECTAL-PROLAPSE: et. AB Cystic fibrosis is detected with increasing frequency in older children, adolescents, and even young adults. The quality of life and longevity in patients with cystic fibrosis is more favorable the earlier a diagnosis is made and a therapeutic regimen begun. This report presents and reviews five cases in chich the diagnosis of cystic fibrosis was made after the age of six years. Emphasis is placed on the variability of presenting signs and symptoms and the problems that can arise in confirming a suspected diagnosis of cystic fibrosis. RF 001 DOERSHUK CF PEDIATRICS 36 675 965 002 DI SANTAGNESE PA N ENGL J MED 277 1287 967 003 STEINBERG AG AM J HUM GENET 12 416 960 004 DI SANTAGNESE PA JAMA 172 2065 960 005 ANDERSON CM ARCH DIS CHILD 35 581 960 006 DARLING RC AM J MED SCI 225 67 953 007 WOOD RE AM REV RESPIR DIS 113 833 976 008 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 009 SHWACHMAN H PEDIATRICS 30 389 962 010 MCCOLLUM AT J PEDIATR 77 571 970 011 GIBSON LE J PEDIATR 81 193 972 012 ELIAN E N ENGL J MED 264 13 961 013 SHWACHMAN H ANN NY ACAD SCI 93 600 962 014 GIBSON LE PEDIATRICS 23 545 959 015 KULCZYCKI LL N ENGL J MED 259 409 958 016 TOMASHEFSKI JF CHEST 57 28 970 017 MILUNSKY A PEDIATRICS 42 501 968 018 HIDE DW ARCH DIS CHILD 44 533 969 019 GOODCHILD MC ARCH DIS CHILD 48 684 973 020 TAYLOR BW ARCH DIS CHILD 48 692 973 021 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 022 MEARNS MB ARCH DIS CHILD 47 902 972 023 SHWACHMAN H PEDIATRICS 55 86 975 024 ROSAN RC AM J DIS CHILD 104 625 962 025 GEORGE L ARCH DIS CHILD 46 139 971 026 GREEN MN PEDIATRICS 41 989 968 027 STEPHAN U PEDIATRICS 55 35 975 028 SHWACHMAN H PEDIATRICS 30 167 962 CT 1 WARWICK WJ J CHRON DIS 33 685 980 2 CHINO ES AM SURGEON 50 70 984 PN 78109 RN 00889 AN 78152730 AU Cloutier-M-M. Mangos-J-A. TI The challenges of cystic fibrosis. SO J-Fla-Med-Assoc. 1978 Apr. 65(4). P 259-63. MJ CYSTIC-FIBROSIS. MN CYSTIC-FIBROSIS: di, fg, th. HUMAN. AB Cystic fibrosis is an autosomal recessive genetic disorder of the exocrine glands which has a frequency of approximately 1:1,500 live births. This disease presents challenges to the physician and the health care team. The Pediatric Pulmonary Center of the University of Florida in Gainesville, in coordination with Cystic Fibrosis Centers and clinics throughout the state, is helping physicians and patients to recognize and manage this chronic disease. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 FANCONI G WIEN MED WOCHENSCHR 86 753 936 003 DI SANTAGNESE PA AM J MED 15 777 953 004 GIBSON LE PEDIATRICS 23 545 959 005 BAU SK PEDIATRICS 48 605 971 006 DUDLEY JP CF CLUB ABST 976 007 LANDAU LI J PEDIATR 82 863 973 008 MANGOS JA PEDIATR RES 2 378 968 009 SPOCK A PEDIATR RES 1 173 967 010 BOWMAN BH SCIENCE 164 325 969 PN 78110 RN 00890 AN 78243783 AU Klinger-J-D. Straus-D-C. Hilton-C-B. Bass-J-A. TI Antibodies to proteases and exotoxin A of Pseudomonas aeruginosa in patients with cystic fibrosis: Demonstration by radioimmunoassay. SO J-Infect-Dis. 1978 Jul. 138(1). P 49-8. MJ ANTIBODIES-BACTERIAL: ip. CYSTIC-FIBROSIS: im. EXOTOXINS: im. PEPTIDE-HYDROLASES: im. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. FEMALE. HUMAN. INFANT. MALE. PSEUDOMONAS-AERUGINOSA: en, py. RADIOIMMUNOASSAY. VIRULENCE. AB Sera from 33 patients with cystic fibrosis and two pediatric patients being treated for chronic pulmonary infections not related to cystic fibrosis and six sera or serum pools from uninfected individuals were tested with a microtiter radioimmunoassay for reactivity against exotoxin A and two proteases from Pseudomonas aeruginosa. Exotoxin A was purified from a low-protease strain of P. aeruginosa and shown to have adenosine diphosphate-ribose transferase activity and mouse lethality. Proteases were purified from an isolate of P. aeruginosa from a patient with cystic fibrosis and had proteolytic activity against elastin and collagen in an assay employing dimethylated protein substrates. The antibody responses of the patients detected using 125I-labeled antibody to human immunoglobulin were correlated with clinical evaluations expressed as a composite score based on pulmonary findings, case histories, growth and nutrition, and chest X rays. Values in the radioimmunoassay for patients' sera were compared with those of a control serum pool and expressed as the ratio of counts per minute (cpm) in patient serum to the cpm in the control pool. Inverse correlations were found between these ratios for each of the pseudomonas exoproducts and clinical scores; highest ratios occurred in patients showing the lowest clinical scores. These results confirm that proteases and exotoxin A of P. aeruginosa are produced in cystic fibrosis pulmonary infections due to P. aeruginosa and suggest that they may serve as significant virulence factors in these chronic infectious states. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 IACOCCA VF AM J DIS CHILD 106 315 963 003 MAY JR ARCH DIS CHILD 47 908 972 004 MEARNS MB ARCH DIS CHILD 47 902 972 005 HOIBY N ACTA PAEDIATR SCAND 63 843 974 006 MARKS MI J PEDIATR 79 822 971 007$ ELLER JJ AM J MED SUPPL 62 179 977 008 KREGER AS INFECT IMMUN 9 828 974 009 SCHULTZ DR INFECT IMMUN 10 128 974 010 PAVLOVSKIS OR IN: SCHLESSINGER D 252 975 011 PAVLOVSKIS OR J INFECT DIS 133 253 976 012 MIDDLEBROOK JL CAN J MICROBIOL 23 183 977 013 BURNS MW LANCET 1 270 968 014 DIAZ F J INFECT DIS 121 269 970 015 HABBOUSHE C PEDIATRICS 48 973 971 016 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 017 SEIDMON EJ J PEDIATR 87 528 975 018 POLLACK M INFECT IMMUN 14 942 976 019 POLLACK M J CLIN MICROBIOL 6 58 977 020 LIU PV J INFECT DIS 128 514 973 021 IGLEWSKI BH INFECT IMMUN 15 138 977 022 MORIHARA K J BIOL CHEM 240 3295 965 023 PURCELL RH APPL MICROBIOL 26 478 973 024 ROSENTHAL JD J IMMUNOL 109 171 972 025 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 026 DOWNIE NM BASIC STATISTICAL METHODS 970 027 COLLIER RJ J BIOL CHEM 246 1496 971 028 VASIL ML INFECT IMMUN 16 353 977 029 FREDRICK JF ANN NY ACAD SCI 121 305 964 030 LIN Y J BIOL CHEM 244 789 969 031 LIU PV J INFECT DIS 116 481 966 032 IGLEWSKI BH PROC NAT ACAD SCI USA 72 2284 975 033 MORIHARA K INFECT IMMUN 15 679 977 034 CARRICK L JR BIOCHIM BIOPHYS ACTA 391 422 975 CT 1 GRAY L INFECT IMMUN 23 150 979 2 MORIHARA K INFECT IMMUN 24 188 979 3 CASH HA AM REV RESPIR DIS 119 453 979 4 KOSTIALA AAI J MED MICROBIOL 13 201 980 5 BERDISCHEWSKY M PEDIATR RES 14 830 980 6 JENSEN SE CAN J MICROBIOL 26 77 980 7 JENSEN SE CAN J MICROBIOL 26 87 980 8 HOMMA JY JAP J EXP MED 50 149 980 9 BEAUDRY PH J PEDIATR 97 144 980 10 SANAI Y FEBS LETTERS 120 131 980 11 CROSS AS J INFECT DIS 142 538 980 12 JENSEN SE J BACTERIOL 144 844 980 13 FICK RB CLIN CHEST MED 2 91 981 14 BLACKWOOD LL INFECT IMMUN 32 443 981 15 CRYZ SJ INFECT IMMUN 32 759 981 16 PENNINGTON JE J CLIN INVEST 68 1140 981 17 WOODS DE J CLIN INVEST 68 1435 981 18 MARKS MI J PEDIATR 98 173 981 19 WOODS DE J INFECT DIS 143 784 981 20 DORING G ZENTRALBL BAKT MIKROB HYG (A) 249 89 981 21 OBERNESSER HJ ZENTRALBL BAKT MIKROB HYG (A) 249 76 981 22 WRETLIND B SCAND J INFECT DIS 1981 13 981 23 CROWE KE J CLIN MICROBIOL 15 115 982 24 JAGGER KS J CLIN MICROBIOL 15 1054 982 25 BAKER NR IN VITRO 18 369 982 26 BAKER NR CAN J MICROBIOL 28 248 982 27 WOODS DE INFECT IMMUN 36 1223 982 28 WEINER RN EXP MOL PATH 37 249 982 29 DORING G ZENTRALBL BAKT MIKROB HYG (A) 252 239 982 30 OBERNESSER HJ ZENTRALBL BAKT MIKROB HYG (A) 252 248 982 31 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 32 POLLACK M REV INFECT DIS 5 S979 983 33 WOODS DE REV INFECT DIS 5 S715 983 34 WRETLIND B REV INFECT DIS 5 S998 983 35 JAGGER KN J CLIN MICROBIOL 17 55 983 36 BRYAN LE J CLIN MICROBIOL 18 276 983 37 CUKOR G J CLIN MICROBIOL 18 457 983 38 CASH HA CAN J MICROBIOL 29 448 983 39 KLINGER JD INFECT IMMUN 39 1377 983 40 LYERLY DM INFECT IMMUN 40 113 983 41 DORING G INFECT IMMUN 42 197 983 42 DORING G J INFECT DIS 147 744 983 43 CAPLAN DB REV INFECT DIS 6 S705 984 44 SPEERT DP PEDIATR RES 18 431 984 45 BOROWSKI RS J CLIN MICROBIOL 19 736 984 46 LONDON CJ APPL ENVIRON MICROBIOL 47 75 984 47 GRANSTROM M ACTA PAEDIATR SCAND 73 772 984 48 DORING G ACTA PATH MICROB IMMU SCA (C) 92 307 984 49 WOOTEN MW J CELL PHYSIOL 121 490 984 50 HANCOCK REW J INFECT DIS 149 220 984 51 PARMELY MJ J EXP MED 160 1338 984 52 GRANSTROM M EUR J CLIN MICROBIOL 4 197 985 53 KLINGER KW EUR J CLIN MICROBIOL 4 201 985 54 PITT TL EUR J CLIN MICROBIOL 4 190 985 55 SAELINGER CB EUR J CLIN MICROBIOL 4 170 985 56 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 57 DORING G INFECT IMMUN 49 557 985 58 LUZAR MA INFECT IMMUN 50 577 985 59 WINKLER U KLIN WSCHR 63 490 985 60 LORY S CURR TOP MICROBIOL IMMUNOL 118 53 985 61 BRUCE MC AM REV RESPIR DIS 132 529 985 62 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 63 WOODS DE CLIN INVEST MED 9 108 986 64 BALTIMORE RS PEDIATR RES 20 1085 986 65 WOODS DE J CLIN MICROBIOL 24 260 986 66 HINGLEY ST INFECT IMMUN 54 379 986 67 PITT TL J ROY SOC MED 79 13 986 68 VASIL ML J PEDIATR 108 800 986 69 MOSS RB AM REV RESPIR DIS 133 648 986 70 PARMELY MJ J IMMUNOL 137 988 986 71 DASGUPTA MK J CLIN IMMUNOL 7 51 987 72 DORING G INFECTION 15 47 987 PN 78111 RN 00891 AN 80076215 AU Dann-L-G. Blau-K. TI Studies on the basic defect in cystic fibrosis: the kinin hypothesis. SO J-Inherited-Metab-Dis. 1978. 1(4). P 179-81. MJ CYSTIC-FIBROSIS: pp. KININS: ph. MN EXOCRINE-GLANDS: pp. HUMAN. MODELS-BIOLOGICAL. EX It is our hypothesis that in cystic fibrosis there is a genetically determined specific deficiency in the activity of either the glandular kallikrein or in its activating enzyme, and that this defect is the primary defect in the disease. The immediate consequence of either of these defects is the absence of kallidin and the resulting failure of reabsorption. The fact that there may be mutations affecting distinct enzymes could account for the genetic heterogeneity of cystic fibrosis. A consequence of a defective kallikrein system would be accumulation of a precursor related to the low molecular weight kininogen and we propose that this compound is the ciliary-dyskinesia factor. RF 001 BENKE PJ IN: MANGOS JA 157 976 002 CHAN KYH CLIN CHIM ACTA 74 71 977 003 DANN LG LANCET 2 405 978 004 DIETL T HOPPE SEYLERS Z PHYSIOL CHEM 359 499 978 005 EMRICH HM PEDIATR RES 2 464 968 006 GARRETT JR ARCH ORAL BIOL 12 1417 967 007 KAISER D LANCET 1 1003 970 008 LOBECK CC IN: STANBURY JB 1605 972 009 MCKUSICK VA MENDELIAN INHERIT IN MAN 468 978 010 MANGOS JA AM J PHYSIOL 224 1235 973 011 MANGOS JA PEDIATR RES 2 378 968 012 MANGOS JA IN: MANGOS JA 311 976 013 MUNGER BL J BIOPHYS BIOCHEM CYTOL 11 385 961 014 NUSTAD K GEN PHARMAC 9 1 978 015 ORSTAVIK TB ACTA HISTOCHEM 54 183 975 016 POTTER JL ANN NY ACAD SCI 106 692 963 017 RAO GJS PEDIATR RES 9 739 975 018 SELINGER Z IN: CARAFOLI E 139 975 019 SCHRAMM M J CYCLIC NUCLEOTIDES RES 1 181 975 020 SUTCLIFFE CH PROC R SOC MED 61 297 968 021 ULLRICH KJ IN: THORN NA 423 974 022 WEBSTER ME BIOCHEM PHARMACOL 12 511 963 PN 78112 RN 00892 AN 78243862 AU Wilson-G-B. Fudenberg-H-H. TI Separation of ciliary dyskinesia substances found in serum and secreted by cystic fibrosis leukocytes and lymphoid cell lines, using protein A--Sepharose CL-4B. SO J-Lab-Clin-Med. 1978 Sep. 92(3). P 463-82. MJ BACTERIAL-PROTEINS. CILIA: an. CYSTIC-FIBROSIS: bl. LEUKOCYTES: se. MN CELL-LINE. CELL-MOVEMENT. CELLS-CULTURED. CILIA: ph. GENOTYPE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYDROGEN-ION-CONCENTRATION. IGG: me. IN-VITRO. PROTEIN-BINDING. SERUM-ALBUMIN. SERUM-ALBUMIN-BOVINE. STAPHYLOCOCCUS. SUPPORT-U-S-GOVT-P-H-S. AB Cell-free culture medium from PHA-stimulated PBL cultures or long-term lymphoid cell lines from CF homozygotes or obligate heterozygotes, as well as serum from such individuals, can produce three distinct changes in the ciliary beat of rabbit tracheal ciliated epithelium: (1) increased beat frequency, (2) CD, and (3) ciliostasis. The ability to produce both 1 and 2 seems to be characteristic for leukocyte cultures from CF genotypes: 46 of 49 CF cultures produced both effects, as compared with none of 16 cultures from normal controls and 1 of 10 from non-CF patient controls with bronchial asthma. CTA was not specific for CF PBL cultures, since 13% of normal control and 60% of the non-CF patient control cultures produced ciliostasis. Similar findings were obtained when serum from the same individuals or culture medium from long-term lymphoid cell lines was tested. Using protein A from Staphylococcus aureus covalently coupled to Sepharose CL-4B as a reagent, we separated the CDAs present in serum and in culture medium from PBL and lymphoid cultures. Protein A specifically adsorbs IgG and binds it under mild conditions at physiological pH. The CDAs are noncovalently bound to IgG at physiological pH and can be co-purified with IgG. The IgG-CDA complexes can be readily desorbed from protein A-Sepharose CL-4B at pH 3.0, and under these conditions the CDAs are dissociated from IgG, retain all their activity, and can be separated by chromatography on Bio-Gel P-10 or Sephadex G-200. Three distinct activities were removed from serum or PBL cultures which showed both CDA and CTA, and two were obtained from samples which showed only CDA. One CDA believed to be specific for CF (CF-CDA) was separated from CF serum and PBL or lymphoid cell culture medium. This CF-CDA was complexed noncovalently to IgG (not subclass IgG3), had a molecular weight of 4000 to 6000, was stable as acid pH, was found in biological fluids from both homozygotes and heterozygotes for CF, was shown not to be C3a (anaphylatoxin), did not require IgG for activity, and was positively charged at pH values more acidic than 8.0. RF 001 SPOCK A PEDIATR RES 1 173 967 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 WOOD RE AM REV RESPIR DIS 111 733 975 004 WOOD RE AM REV RESPIR DIS 113 833 976 005 BOWMAN BH SCIENCE 164 325 969 006 BESLEY GTN J MED GENET 6 278 969 007 BERATIS NG PEDIATR RES 7 958 973 008 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 009 DANES BS J EXP MED 136 1313 972 010 CONOVER JH PEDIATR RES 7 224 973 011 DANES BS CLIN GENET 7 128 975 012 WILSON GB CLIN RES 25 34 977 014 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 015 DANES BS J EXP MED 137 1538 973 016 DANES BS TEX REP BIOL MED 34 135 976 017 WILSON GB PEDIATR RES 11 317 977 018 CONOD EJ PEDIATR RES 11 45 977 019 GLADE PR AM J PATHOL 60 483 970 020 CONOVER JH LANCET 2 1501 973 021 CONOVER JH PEDIATR RES 7 220 973 022 WILSON GB PEDIATR RES 11 143 977 023 WILSON GB IN: RADOLA BJ 337 977 024 WILSON GB NATURE 266 463 977 025 HJELM H FEBS LETTERS 28 73 972 026 WILSON GB PEDIATR RES 9 635 975 027 WILSON GB PEDIATR RES 11 139 977 028 POLET H J EXP MED 142 949 975 029 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 030 BRODER SW BLOOD 35 539 970 032 GRABAR P BIOCHIM BIOPHYS ACTA 10 193 953 033 LAURELL CB ANAL BIOCHEM 15 45 966 034 FUDENBERG HH BASIC IMMUNOGENETICS 977 035 VYAS GN J IMMUNOL 100 274 968 036 PANDEY JP J IMMUNOGENET 5 122 978 037 BOHLEN P ARCH BIOCHEM BIOPHYS 155 213 973 038 KRONVALL G J IMMUNOL 103 828 969 040 SPRAGG J ANN NY ACAD SCI 209 372 973 041 AMAUD P TRANS ASSOC AM PHYSICIANS 39 205 976 042 CONOVER JH LIFE SCI 14 253 974 043 CONOD EJ PEDIATR RES 9 724 975 044 VALLOTA EH J EXP MED 137 1109 973 045 BARNETT DR TEX REP BIOL MED 31 709 973 046 WILSON GB PEDIATR RES 10 87 976 047 SHAPIRA E J BIOL CHEM 252 7923 977 CT 1 WILSON GB PEDIATR RES 13 1079 979 2 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 3 WILSON GB J CLIN INVEST 66 1010 980 4 MOSS RB AM REV RESPIR DIS 121 23 980 5 WILSON GB PROC SOC EXP BIOL MED 164 105 980 6 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 7 WILSON GB J CLIN INVEST 68 171 981 8 WILSON GB CLIN GENET 26 331 984 PN 78113 RN 00893 AN 79049543 AU Moller-N-E. Thomsen-J. TI Mucocele of the paranasal sinuses in cystic fibrosis. SO J-Laryngol-Otol. 1978 Nov. 92(11). P 1025-7. MJ CYSTIC-FIBROSIS: co. MUCOCELE: et. PARANASAL-SINUS-DISEASES: et. MN CASE-REPORT. CHILD. FEMALE. HUMAN. MALE. AB Two children below 9 years of age with mucocele and cystic fibrosis are reported. Mucocele in this age-group is very rare and is probably pathognomonic of cystic fibrosis. RF 001 CARDESA CITED IN: ACTA OTO RINO LARIN 3 251 970 002 DAWES JDK IN: SCOTT-BROWN 3 266 971 003 MENDELSOHN RS ARCH OTOLARYNGOL 79 312 964 004 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 005 RITTER FN IN: COATES GM 3 15 955 006 ROBERTSON DM AM J OPHTHALMOL 68 845 969 007 RULON JT ARCH OTOLARYNGOL 78 192 963 008 STOOL S TRANS AM ACAD OPHTHALMOL OTOL 70 811 966 009 TALLENT A CAHIERS OTO RHINO LARYNGOL 5 7 970 CT 1 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 78114 RN 00894 AN 78201635 AU Calbreath-D-F. TI Screening for cystic fibrosis. SO J-Nurs-Care. 1978 Jul. 11(7). P 8. MJ CYSTIC-FIBROSIS: di. MN HUMAN. INFANT-NEWBORN. EX Cystic fibrosis is a great unknown. There is a lot of information about the disease, but no common denominator, nothing to pull all the pieces together. It represents both a diagnostic and a therapeutic challenge. Progress is being made and perhaps someday we will be able to understand this complex entity. PN 78115 RN 00895 AN 79153473 AU Morrissey-S-M. Tymvios-M-C. TI Acid mucins in human intestinal goblet cells. SO J-Pathol. 1978 Dec. 126(4). P 197-208. MJ CYSTIC-FIBROSIS: me. INFANT-NEWBORN-DISEASES: me. INTESTINE-SMALL: me. MUCINS: me. MN CELL-COUNT. CYSTIC-FIBROSIS: pa. DUODENUM: me. HISTOCYTOCHEMISTRY. HUMAN. ILEUM: me. INFANT. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: pa. INTESTINE-SMALL: pa. JEJUNUM: me. AB Acid mucins in goblet cells of the duodenum, jejunum and ileum of neonates and infants with cystic fibrosis (CF) and without CF were measured by scanning microdensitometry after alcian blue staining according to the protocol of McCarthy and Reid (1963) which characterises four groups of acidic mucins. In CF infants over 6 mth of age, but not in controls, there was an increase along the gut from duodenum to ileum of both weakly acidic and strongly acidic sulphomucins. In the ileum the increase was in total mucins from 6 mth previously in the same CF patients and this difference could be accounted for by an increase of sialidase-resistant mucins. The increase in sulphomucins was more marked at the tip than at the base of the villi. In CF neonates there was significant difference in the quantities of acidic mucins. The question whether the mucins of CF are chemically abnormal or merely accumulated to an abnormal extent is probably best investigated by analysis of sialidase-resistant mucins and sulphomucins of the ileum and strongly acidic sulphomucins of the duodenum and jejunum in CF infants over 6 mth of age. RF 001 ANDERSEN DH ANN NY ACAD SCI 93 500 962 002 BAUER U ANN PAEDIATR 194 236 960 003 BELANGER L ANN NY ACAD SCI 106 364 963 004 DISCHE Z PEDIATRICS 24 74 959 005 FARBER S J MICH MED SOC 44 587 945 006 FREYE HB J PEDIATR 64 575 964 007 GIBBONS RA EXCERPTA MED INT CONG SERIES 133 695 966 008 GOTTSCHALK A CHEM BIOL SIALIC ACID REL 960 009$ GRAIG JM IN: RESEARCH ON CF 25 959 010 GRECO V GUT 8 491 967 011 JOHANSEN PG ANN NY ACAD SCI 106 755 963 012 JOHANSEN PG J PATHOL 99 299 969 013 JONES R HISTOCHEM J 5 19 973 014 KENT PW BIOCHEM J 87 38 963 015 LAMB D THESIS 969 016 LAMB D J PATHOL 98 213 969 017 LAMB D HISTOCHEM J 4 91 972 018 LANDING BH ROSS PAEDIATR RES CONF 18TH 63 956 019 LEV R LAB INVEST 14 2080 966 020 LEV R AM J PATHOL 46 23 965 021 MCCARTHY C Q J EXP PHYSIOL 49 81 964 022 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 023 POTTER JL ANN NY ACAD SCI 106 692 963 024 ROELFS RE AM J DIS CHILD 113 419 967 025 DI SANTAGNESE PA N ENGL J MED 277 1287 967 026 SCHRAGER J NATURE 201 702 964 027 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 028 SPICER SS AM J CLIN PATHOL 33 453 970 029 THOMAIDIS TS J PEDIATR 63 444 963 030 WAGNER BM AM J DIS CHILD 99 61 960 CT 1 HOUGH L J PHYSIOL (LOND) 291 P 9 979 2 MORRISSEY SM GUT 22 788 981 3 ADLER KB LAB INVEST 45 372 981 4 FORSTNER G ADV EXP MED BIOL 144 199 982 5 WESLEY A PEDIATR RES 17 65 983 6 MORRISSEY SM GUT 24 909 983 7 REID L INT REV EXP PATHOL 24 335 983 8 JACOBS LR DIG DIS SCI 28 422 983 9 JEFFREY I J CLIN PATHOL 36 1292 983 10 HOLLANDERS D BR J CLIN PRACT 37 112 983 11 SLOMIANY A BIOCHIM BIOPHYS ACTA 750 253 983 12 REID PE HISTOCHEM J 16 253 984 13 JEFFREY I J CLIN PATHOL 37 957 984 14 HOLLANDERS D J PHYSIOL (LOND) 348 P 8 984 15 CLEGHORN GJ LANCET 1 8 986 16 MORRISSEY SM DIGESTION 34 28 986 17 HOLLANDERS D BR J CLIN PRACT 40 74 986 PN 78116 RN 00896 AN 78153026 AU Buts-J-P. Morin-C-L. Roy-C-C. Weber-A. Bonin-A. TI One-hour blood xylose test: a reliable index of small bowel function. SO J-Pediatr. 1978 May. 92(5). P 729-33. MJ INTESTINAL-MUCOSA: pp. INTESTINE-SMALL: pp. XYLOSE: du. MN CELIAC-DISEASE: di. CYSTIC-FIBROSIS: pp. FECES: an. HUMAN. INTESTINAL-DISEASES: di. LIPIDS: an. MALABSORPTION-SYNDROMES: di. RETROSPECTIVE-STUDIES. TIME-FACTORS. XYLOSE: bl. AB A retrospective analysis of oral D-xylose tolerance tests in 435 pediatric patients was performed. A significant difference was found between 126 normal subjects and 47 untreated children with celiac disease one hour after load. The one-hour value was found to be more reliable than was fecal fat analysis in screening children for celiac disease. Sex, age, weight, and body surface did not influence the results of the one-hour value in the control subjects. Forty-eight children with cystic fibrosis had one-hour xylose levels within the normal range, but the means at 90, 120, and 180 minutes after load exceeded significantly (P less than 0.01) those of the controls. In a nonceliac group of 63 children with abnormally low xylose levels, almost all were found to have clinical conditions compatible with upper small bowel mucosal impairment. This study shows that a single estimation of xylose in blood one hour after load is a reliable index of small bowel mucosal function. It is a good screening test for celiac disease and small bowel disorders producing malabsorption in children. RF 001 HAWKINS KI CLIN CHEM 16 749 970 002 JONES WO J PEDIATR 62 50 963 003 MEEUWISSE GW ACTA PAEDIATR SCAND 54 33 965 004 MCCRAE WM ARCH DIS CHILD 38 571 963 005 SAMMONS HG GUT 8 348 967 006 FINLAY JM ANN INTERN MED 61 411 964 007 BENSON JA JR N ENGL J MED 256 335 957 008 ROLLES CJ LANCET 2 1043 973 009 ROLLES CJ ARCH DIS CHILD 50 359 975 010 SLADEN GE BR MED J 3 223 973 011 LAMABADUSURIYA SP ARCH DIS CHILD 50 34 975 012 GOLDMAN AS PEDIATRICS 32 425 963 013 DAVIDSON M J PEDIATR 69 1027 966 014 ROE JH J BIOL CHEM 173 507 948 015 LANZKOWSKY P JAMA 186 517 963 016 VAN DE KAMER JH J BIOL CHEM 177 347 949 017 SHMERLING DH PEDIATRICS 46 690 970 018 FORDTRAN JS N ENGL J MED 267 274 962 019 BUTTERWORTH CE JR N ENGL J MED 261 157 959 020 SEGAL S J CLIN INVEST 38 407 959 021 CASPARY WF GASTROENTEROLOGY 63 531 972 022 GLUCKMAN RF GASTROENTEROLOGY 44 828 963 023 KENDALL MJ BR MED J 1 533 971 024 JACOBSON ED AM J MED 28 524 960 025 EGAN-MITCHELL B ARCH DIS CHILD 47 238 972 026 OPPENHEIMER EH J PEDIATR 86 683 975 027 DI SANTAGNESE PA PEDIATRICS 18 387 956 028 WYNGAARDEN JB J CLIN INVEST 36 1395 957 029 BRIEN FS GASTROENTEROLOGY 20 287 952 030 SANTIAGO-BORRERO PJ PEDIATRICS 48 59 971 031 GOLDSTEIN F GASTROENTEROLOGY 59 380 970 032 ROBILLARD JE UNION MED CAN 102 1496 973 CT 1 MORIN CL LANCET 1 1102 979 2 CHRISTIE DL J PEDIATR 94 509 979 3 LIEBMAN WM J PEDIATR 94 508 979 4 MORIN CL J PEDIATR 94 509 979 5 COBDEN I GUT 21 512 980 6 PARK RW GASTROENTEROLOGY 81 1143 981 7 LEBENTHAL E J PEDIATR 98 681 981 8 KEKOMAKI M J PEDIATR GASTROENTEROL NUTR 1 369 982 9 MONTGOMERY RD TRANS R SOC TROP MED HYG 76 25 982 10 RYAN ME CLIN GASTROENTEROL 12 533 983 11 SAVILAHTI E ARCH DIS CHILD 58 246 983 12 PERKKIO M ACTA PAEDIATR SCAND 73 680 984 13 FITZGERALD JF PEDIATR INFECT DIS 4 6 985 14 BONAMICO M RIV ITAL PEDIATR 11 172 985 15 CHATTI N UNION MED CAN 114 60 985 16 LOMBARD KA PEDIATRICS 77 396 986 17 LEVINE JJ AM J DIS CHILD 141 435 987 PN 78117 RN 00897 AN 79069434 AU Davidson-G-P. Hassel-F-M. Crozier-D. Corey-M. Forstner-G-G. TI Iatrogenic hyperuricemia in children with cystic fibrosis. SO J-Pediatr. 1978 Dec. 93(6). P 976-8. MJ CYSTIC-FIBROSIS: bl. URIC-ACID: bl. MN ADOLESCENCE. ADULT. AGE-FACTORS. CHILD. CYSTIC-FIBROSIS: dt. HUMAN. PANCREAS: en. PANCREATIC-EXTRACTS: ae, tu. PURINES: ae. EX We report the frequent presence of hyperuricemia in a group of children with cystic fibrosis who were known to be consuming large dosages of pancreatic extract as part of their therapy. Hyperuricemia was more common in older patients who had been treated for a longer period of time. Clarification of the role of renal function and other factors in the production of hyperuricemia awaits a careful study of the relationship of purine load to urate excretion at various ages in these patients. RF 001 STAPLETON FB N ENGL J MED 295 246 976 002 NOUSIA-ARVANITAKIS S J PEDIATR 90 302 977 003 COREY M AM REV RESPIR DIS 114 1085 976 004 HARKNESS RA ARCH DIS CHILD 44 773 969 005 ENDOZIEN JC NATURE 228 180 970 006 STAPLETON FB PEDIATR RES 11 558 977 007 KAUFMAN JM J PEDIATR 73 583 968 008 WINBERG J ACTA PAEDIATR SCAND 48 443 959 009 GUTMAN AB TRANS ASSOC AM PHYSICIANS 74 353 961 010 KLINENBERG J NEPHRON 14 88 975 CT 1 GAHL WA J PEDIATR 95 330 979 2 ROBB TA LANCET 2 544 980 3 BELL L J CAN DIET ASSOC 42 62 981 4 BOHLES H HELV PAEDIATR ACTA 37 267 982 5 MISCHLER EH AM J DIS CHILD 136 1060 982 6 CHEN YT N ENGL J MED 311 128 984 7 PERRY RS CLIN PHARMACY 4 161 985 8 ZENTLERMUNRO PL GUT 26 892 985 9 SPINO M J PEDIATR 107 64 985 10 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 11 GOODCHILD MC J ROY SOC MED 79 32 986 12 PHILLIPS BM J ROY SOC MED 79 44 986 PN 78118 RN 00898 AN 78153070 AU Kulczycki-L-L. Schauf-V. TI Incidence of cystic fibrosis in black children--revisited [letter]. SO J-Pediatr. 1978 May. 92(5). P 855. MJ CYSTIC-FIBROSIS: oc. MN BLACKS. CHILD. DISTRICT-OF-COLUMBIA. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: oc. NEW-YORK-CITY. EX In the February, 1977, issue of The Journal, Dr. Heffer comments on publications concerning the occurrence of cystic fibrosis (CF) in black children. He "feels" that CF occurs more frequently than we have reported. Our ten-year search in the Washington, D.C., area shows a minimum incidence of cystic fibrosis among black newborn infants as one per 17,000. Since 1972, we have identified an additional four black patients with cystic fibrosis in black children. This is consistent with the previously reported incidence of one in 17,000. Therefore, we disagree with Heffer, that this is a common disease among blacks. RF 001 HEFFER ET J PEDIATR 90 324 977 002 HEFFER ET NY STATE J MED 74 2355 974 003 KULCZYCKI LL AM J DIS CHILD 127 64 974 004 KULCZYCKI LL CLIN PEDIATR 3 692 964 005 STERN RC J PEDIATR 89 412 976 006 DI SANTAGNESE PA ANN INTERN MED 54 482 961 PN 78119 RN 00899 AN 78153027 AU Nousia-Arvanitakis-S. Arvanitakis-C. Desai-N. Greenberger-N-J. TI Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by the synthetic peptide N-benzoyl-L-tyrosyl-p-aminobenzoic acid. SO J-Pediatr. 1978 May. 92(5). P 734-7. MJ P-AMINOBENZOIC-ACID: du. AMINOBENZOIC-ACIDS: du. CYSTIC-FIBROSIS: co. PANCREATIC-DISEASES: di. MN P-AMINOBENZOIC-ACID: aa, ur. ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CHYMOTRYPSIN: me. FECES: an, en. HUMAN. LIPIDS: an. PANCREATIC-DISEASES: et, ur. SUPPORT-U-S-GOVT-P-H-S. AB The synthetic peptide N-benzoyl-L-tyrosyl-p-aminobenzoic acid is specifically cleaved by chymotrypsin to Bz-Ty and PABA. The liberated PABA is absorbed and excreted in the urine. Accordingly, PABA recovery reflects intraluminal chymotrypsin activity and is an index of exocrine pancreatic function. This test was evaluated in 24 patients with cystic fibrosis to determine its role in the diagnosis of exocrine pancreatic insufficiency. Cumulative percent PABA recovery in six hours was significantly lower in CF patients compared with the control group. No overlap was noted between the two groups. There was good correlation between PABA recovery, fecal chymotrypsin activity, and coefficient of fat absorption. These findings indicate that PABA recovery is significantly reduced in patients with CF and steatorrhea and may prove a practical and reliable test of pancreatic insufficiency. RF 001 KOPEL FB GASTROENTEROLOGY 62 483 972 002 STAPLETON FB N ENGL J MED 295 246 976 003 IMONDI AR GUT 13 726 972 004 ARVANITAKIS C LANCET 1 663 976 005 GYR K GUT 17 27 976 006 BORNSCHEIN W CLIN CHIM ACTA 67 21 976 007 TAUSSIG LM J PEDIATR 82 380 973 008 VAN DE KAMER JH J BIOL CHEM 177 347 949 009 IMONDI AR ANAL BIOCHEM 54 199 973 010 BRATTON AC J BIOL CHEM 128 537 939 011 SMITH HW J CLIN INVEST 24 388 945 012 BARBERO GJ AM J DIS CHILD 112 536 966 013 DYCK WP AM J DIG DIS 12 310 967 014 GREENGARD P IN: GOODMAN LS 1661 970 015 ZAROFONETIS CJD J INVEST DERMATOL 11 359 948 CT 1 IMONDI AR DIG DIS SCI 24 214 979 2 MALIS F J PEDIATR 94 942 979 3 SACHER M J PEDIATR 94 159 979 4 LANKISCH PG DTSCH MED WSCHR 105 1418 980 5 LANG C SCHWEIZ MED WOCHENSCHR 110 522 980 6 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 7 NIESSEN KH MONATSSCHR KINDERHEILKD 128 301 980 8 OSSWALD P MONATSSCHR KINDERHEILKD 128 340 980 9 SCHONBERGER W MONATSSCHR KINDERHEILKD 128 195 980 10 HOEK FJ GUT 22 8 981 11 LANKISCH PG HEPATOGASTROENTEROLOGY 28 333 981 12 LANG C BR J SURG 68 771 981 13 PARK RW GASTROENTEROLOGY 81 1143 981 14 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 15 WOLF C SCHWEIZ MED WOCHENSCHR 111 343 981 16 LIBEER JC CLIN CHIM ACTA 115 119 981 17 KAI H J PEDIATR GASTROENTEROL NUTR 1 445 982 18 LANKISCH PG GUT 23 777 982 19 RYAN ME CLIN GASTROENTEROL 12 533 983 20 DELCHIER JC GUT 24 318 983 21 HEELEY AF CLIN CHEM 29 2011 983 22 TOSKES PP GASTROENTEROLOGY 85 565 983 23 GOLDBERG DM CLIN PHYSIOL BIOCHEM 2 249 984 24 TOSKES PP PHARMACOTHERAPY 4 74 984 25 LANKISCH PG CLIN GASTROENTEROL 13 717 984 26 HUBBARD VS DIG DIS SCI 29 881 984 27 GILLARD BK AM J DIS CHILD 138 577 984 28 BELLENTANI S EUR J PEDIATR 143 145 984 29 ORENSTEIN DM SEM RESPIR MED 6 252 985 30 SCOTTA MS CLIN BIOCHEM 18 233 985 31 BORNSCHEIN W Z GASTROENTEROL 23 247 985 32 KOREN G DIG DIS SCI 30 928 985 33 RIEU D ANN PEDIATR (PARIS) 32 205 985 34 SCHIOTZ PO ACTA PAEDIATR SCAND 74 460 985 35 NIEDERAU C GASTROENTEROLOGY 88 1973 985 36 HEYMAN MB GASTROENTEROLOGY 89 685 985 37 WEIZMAN Z GASTROENTEROLOGY 89 596 985 38 SCHARPE S CLIN CHEM 33 5 987 39 SARLES J ARCH FR PEDIATR 44 311 987 PN 78120 RN 00900 AN 79069407 AU Vitullo-B-B. Rochon-L. Seemayer-T-A. Beardmore-H. de-Belle-R-C. TI Intrapancreatic compression of the common bile duct in cystic fibrosis. SO J-Pediatr. 1978 Dec. 93(6). P 1060-1. MJ COMMON-BILE-DUCT. CYSTIC-FIBROSIS: co. MN AUTOPSY. CASE-REPORT. CHOLESTASIS: et. COMMON-BILE-DUCT: pa. CYSTIC-FIBROSIS: pa. HUMAN. INFANT. MALE. PRESSURE. AB We report an infant with cystic fibrosis presenting at birth with meconium ileus who developed prolonged cholestatic jaundice from compression of the common bile duct and the pancreatic duct by advanced pancreatic fibrosis. Extrahepatic biliary obstruction of this nature has not been previously described in cystic fibrosis. RF 001 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 002 OPPENHEIMER EH J PEDIATR 86 683 975 003 TALAMO RC AM J DIS CHILD 115 74 968 004 JEAN R ARCH FR PEDIATR 27 849 970 005 VALMAN HB ARCH DIS CHILD 46 805 971 CT 1 LAMBERT JR GASTROENTEROLOGY 80 169 981 2 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 3 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 4 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 5 TEDESCO FJ GASTROINTEST ENDOSC 32 25 986 6 PATRICK MK J PEDIATR 108 101 986 PN 78121 RN 00901 AN 78088954 AU Antonowicz-I. Lebenthal-E. Schwachman-H. TI Disaccharidase activities in small intestinal mucosa in patients with cystic fibrosis. SO J-Pediatr. 1978 Feb. 92(2). P 214-9. MJ CYSTIC-FIBROSIS: en. DISACCHARIDASES: me. INTESTINAL-MUCOSA: en. INTESTINE-SMALL: en. MN ALPHA-GLUCOSIDASES: me. ATROPHY. BETA-GALACTOSIDASES: me. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. HUMAN. INTESTINAL-MUCOSA: pa. INTESTINE-SMALL: pa. AB The disaccharidase activities in small intestinal biopsies were related to the morphology of the mucosa and the ages of 63 patients with cystic fibrosis and 177 healthy control subjects of Caucasian origin. In patients with CF and in the healthy control subjects under 5 years of age with normal intestinal mucosa, no low lactase activity was found. In those patients with CF who were over 5 years of age, one group had high and one group had low lactase activity, as occurs in healthy Caucasian control subjects of the same age. This finding supports the view that in patients with CF, lactase deficiency is not related to the disease entity. In patients with or without CF who had the same degree of mucosal atrophy, the decrease of disaccharidase activities followed the same pattern, indicating that enzyme activities are affected to the same extent by the damage of the mucosa. In patients with CF with pancreatic insufficiency, the disaccharidase activities were significantly (P less than 0.001) higher when compared to those in control subjects of the same age and ethnic group, although the increase was not uniform in all patients with cystic fibrosis. RF 001 AURICCHIO S LANCET 2 324 963 002 WESER E N ENGL J MED 273 1070 965 003 ANDERSON AFR AM J DIG DIS 9 91 942 004 CHALFIN D AM J DIG DIS 12 81 967 005 SHEEHY TW LANCET 2 1 965 006 BINDER HJ AM J DIG DIS 11 858 966 007 FRAZER AC LANCET 1 503 966 008 LITMAN A ISR J MED SCI 4 110 968 009 KOJECKY Z AM J PROCTOL 19 204 968 010 NORDIO S ANN PAEDIATR 206 287 966 011 PETERNAL WW GASTROENTEROLOGY 48 299 965 012 COZZETTO FJ PEDIATRICS 32 228 963 013 GRYBOSKI JD GASTROENTEROLOGY 45 633 963 014 JONES RHT LANCET 2 120 964 015 ANTONOWICZ I PEDIATRICS 42 492 968 016 CUATRECASAS P LANCET 1 14 965 017 COOK GC LANCET 1 725 966 018 GILAT T AM J DIG DIS 15 895 970 019 KRETCHMER N LANCET 2 392 971 020 HUANG SS SCIENCE 160 83 966 021 BAYLESS TM JAMA 197 968 966 022 BAYLESS TM JOHNS HOPKINS MED J 121 54 967 023 ALZATE H AM J CLIN NUTR 22 122 969 024 REDDY V AM J CLIN NUTR 25 114 972 025 LEBENTHAL E GASTROENTEROLOGY 67 807 974 026 WELSH JD MEDICINE (BALTIMORE) 49 257 970 027 SIMMONS FJ AM J DIG DIS 18 595 973 028 JOHNSON JD ADV PEDIATR 22 197 975 029 DAHLQVIST A BIOL SOC TRANS 3 227 975 030 LEBENTHAL E AM J CLIN NUTR 28 595 975 031 ALPERS DH BIOCHIM BIOPHYS ACTA 401 28 975 032 SHWACHMAN H AM J DIS CHILD 96 6 958 033 DAHLQVIST A ANAL BIOCHEM 7 18 964 034 TOWNLEY RRW PEDIATRICS 36 911 965 035 LOWRY OH J BIOL CHEM 193 265 951 036 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 037 PENA AS GUT 10 1052 969 038 GUDMAND-HOYER E GUT 11 338 970 039 TANDON R AM J DIG DIS 16 845 971 040 PENA AS GASTROENTEROLOGY 64 400 973 041 GUDMAND-HOYER E SCAND J GASTROENTEROL 10 209 975 042 ARVANITAKIS C AM J DIG DIS 19 417 974 043 SEETHARAM B LIFE SCI 18 89 976 044 ANTONOWICZ I PEDIATRICS 56 782 975 CT 1 SCHMIDT E CLIN BIOCHEM 12 247 979 2 SCHMIDT E J CLIN CHEM CLIN BIOCHEM 17 693 979 3 KEDINGER M ENZYME 24 96 979 4 COX KL J PEDIATR 94 488 979 5 LEBENTHAL E AM J DIS CHILD 133 21 979 6 BROWN KH AM J CLIN NUTR 33 1054 980 7 MITCHELL EA AUST PAEDIATR J 17 89 981 8 BRANSKI D DIGESTION 22 8 981 9 MCNAMARA D BIOMEDICINE EXPRESS 35 122 981 10 LEBENTHAL E PEDIATRICS 67 828 981 11 PARK RW GASTROENTEROLOGY 81 1143 981 12 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 13 CATALA J DIGESTION 24 234 982 14 BOUNOUS G GASTROENTEROLOGY 82 1457 982 15 GUTSCHMIDT S J PEDIATR GASTROENTEROL NUTR 2 677 983 16 ROSSI E ANN ALLERGY 53 649 984 17 SCHWARTZ M PRENAT DIAGN 5 145 985 18 CREUTZFELDT W SCAND J GASTROENTEROL 20 45 985 19 FRASE LL GASTROENTEROLOGY 88 478 985 20 RUBINSTEIN S PEDIATRICS 78 473 986 PN 78122 RN 00902 AN 78132308 TI Pancreatitis in children [letter]. SO J-Pediatr. 1978 Apr. 92(4). P 685-6. MJ PANCREATITIS: fg. MN AMYLASES: bl. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, di. FEMALE. HUMAN. INFANT. PANCREATITIS: et. PREGNANCY. EX "Hereditary pancreatitis" is important for at least three reasons: (1) It is probably not an uncommon form of pancreatitis in children and young adults; (2) the course of the disorder may be chronic, with substantial long-term morbidity, including an apparently increased risk for pancreatic cancer; and (3) once having made the correct diagnosis, occurrence among siblings and/or offspring (and perhaps other relatives) of the proband may be expected. Hereditary pancreatitis is an autosomal dominant trait with apparently complete penetrance, though markedly variable expressivity. It would be reasonable to point out that hereditary pancreatitis should be entertained whenever an alternative diagnosis is not documented, even without a positive family history. - We agree with the statement by Jordan and Ament in a recent article that "acute pancreatitis is one of the rarer causes of abdominal pain in children and is among the least reported and characterized." However, their discussion of this problem is surprisingly incomplete. Familial pancreatitis, one of the most important causes of pancreatitis in childhood, is not even mentioned; cystic fibrosis is barely acknowledged. These two conditions as well as hypolipoproteinemia and ascariasis (also not mentioned) should be included in the differential diagnosis of pancreatitis in children and of otherwise unexplained abdominal pain in this age group. - We appreciate the concern that we did not diagnose hereditary pancreatitis in any of our patients. None of the ten patients with "idiopathic pancreatitis" had a family history of pancreatitis documented in their charts. Cystic fibrosis (CF) is not a common cause of pancreatitis in our center. We chose to omit ascariasis from our differential diagnosis because in the developed countries it is unlikely to be a common cause of pancreatitis. RF 001 JORDAN SC J PEDIATR 91 211 977 002 RICCARDI VM ARCH INTERN MED 135 822 975 003 KATTWINKEL J PEDIATRICS 51 55 973 003 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 004 SCRIVER CR AMINO ACID METABOL DISORDERS 973 004 SHWACHMAN H PEDIATRICS 55 86 975 005 SKUDE G SCAND J GASTROENTEROL 10 577 975 006 TYE JG J MED GENET 13 96 976 007 WOLF RO J LAB CLIN MED 87 164 976 008 SKUDE G ACTA PAEDIATR SCAND 65 145 976 009 SCHIOTZ PO CLIN GENET 11 43 977 CT 1 WOLF RO PEDIATR RES 13 1076 979 2 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 3 MASARYK TJ DIG DIS SCI 28 874 983 PN 78123 RN 00903 AN 79007007 AU Buescher-E-S. Winkelstein-J-A. TI The ability of bacteria to activate the terminal complement components in serum of patients with cystic fibrosis. SO J-Pediatr. 1978 Sep. 93(3). P 530-1. MJ BACTERIA. COMPLEMENT. CYSTIC-FIBROSIS: im. MN CYSTIC-FIBROSIS: co, mi. HUMAN. IN-VITRO. LUNG-DISEASES: et. PSEUDOMONAS-AERUGINOSA. RECURRENCE. STAPHYLOCOCCUS-AUREUS. SUPPORT-U-S-GOVT-P-H-S. EX The terminal complement components, C3 to C9, contribute to the host's defense against infection. This study was performed to determine if defective activation of C3 to C9 by bacteria contributes to the pathogenesis of the recurrent pulmonary infections in cystic fibrosis. When each of the bacteria was incubated in the serum of patients with cystic fibrosis for 30 minutes, the consumption of available C3 was the same as in the sera of the control patients and of the two patients with chronic, severe bronchiectasis. Based on the results of the present study, it is unlikely that defective activation of C3 to C9 contributes to the pathogenesis of the increased susceptibility to pulmonary infections of these patients. RF 001 TALAMO RC IN: MANGOS JA 195 976 002 POLLEY MJ J MED GENET 11 249 974 003 LYRENE RK J PEDIATR 91 681 977 004 WINKELSTEIN JA J IMMUNOL 108 1681 972 CT 1 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 2 HODSON ME THORAX 35 801 980 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 4 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 78124 RN 00904 AN 79049980 AU Mitchell-I. Corey-M. Woenne-R. Krastins-I-R. Levison-H. TI Bronchial hyperreactivity in cystic fibrosis and asthma. SO J-Pediatr. 1978 Nov. 93(5). P 744-8. MJ ASTHMA: pp. BRONCHI: de. CYSTIC-FIBROSIS: pp. METHACHOLINE-COMPOUNDS: pd. MN ADOLESCENCE. ADULT. ASTHMA: im. BRONCHI: im, pp. CHILD. CYSTIC-FIBROSIS: fg, im. DOSE-RESPONSE-RELATIONSHIP-DRUG. FEMALE. FORCED-EXPIRATORY-VOLUME. HISTAMINE: pd. HUMAN. MALE. AB We studied 113 patients with CF and compared their responsiveness to inhaled methacholine to that of 50 asthmatic children and 26 normal children. Positive responses to MCh occurred in 51% of the patients with CF and 98% of those with asthma. There was a significant correlation between a positive response to MCh in CF and poor pulmonary function. There was no relationship between allergic rhinitis or positive allergen skin tests and a positive MCh response. Dose-response curves and time course to MCh challenge differed in CF and asthma. Bronchial hyperreactivity is thus common in CF but is different from that in asthma. RF 001 COUNAHAN R ARCH DIS CHILD 50 477 975 002 WARNER JO ARCH DIS CHILD 51 507 976 003 LANDAU LI J PEDIATR 82 863 973 004 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 005 CHAI H J ALLERGY CLIN IMMUNOL 56 323 975 006 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 007 MELLIS CM PEDIATRICS 61 446 978 008 MERCER TT ANN ALLERGY 23 314 965 009 WENG TR AM REV RESPIR DIS 99 879 969 010 NIE NH STAT PACKAGE FOR THE SOCIAL S 975 011 SNEDECOR GW STATISTICAL METHODS 967 012 HALUSZKA J RESPIRATION 32 217 975 013 EMPEY DW AM REV RESPIR DIS 113 131 976 014 RUBINFELD AR AM REV RESPIR DIS 115 381 977 015 SWIFT DL IN: LAWSON D PROC 5TH INT CF 969 016 DOLOVICH MB J APPL PHYSIOL 40 468 976 017 NELSON LA CF CLUB ABST 18 977 CT 1 ANON LANCET 1 708 979 2 MIHALAS LS J PEDIATR 94 1013 979 3 MILLIGAN DWA J PEDIATR 95 24 979 4 KATTAN M N ENGL J MED 301 664 979 5 BURDON JGW MED J AUST 2 77 980 6 SLY PD AUST PAEDIATR J 16 205 980 7 KATTAN M THORAX 35 531 980 8 ORMEROD LP THORAX 35 768 980 9 TOBIN MJ THORAX 35 807 980 10 MOSS RB AM REV RESPIR DIS 121 23 980 11 GURWITZ D AM REV RESPIR DIS 122 95 980 12 LARSEN GL AM J DIS CHILD 134 1143 980 13 DAVIS PB N ENGL J MED 302 1453 980 14 FANTA CH MED CLIN NORTH AM 65 473 981 15 GALANT SP J CLIN INVEST 68 253 981 16 GURWITZ D J PEDIATR 98 551 981 17 MOSS RB J PEDIATR 99 215 981 18 LEMANSKE RF AM REV RESPIR DIS 123 622 981 19 VANASPEREN P AM J DIS CHILD 135 815 981 20 RUSSELL NJ BR J DIS CHEST 76 35 982 21 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 22 WONNE R ATEMWEGS LUNGENKRANKH 9 104 983 23 NEIJENS HJ PEDIATR CLIN NORTH AM 30 829 983 24 DAVIS PB J CHRON DIS 36 269 983 25 SCHEINMANN P SEM HOP PARIS 59 2969 983 26 LEWIS MI S AFR MED J 65 641 984 27 MCFADDEN ER J ALLERGY CLIN IMMUNOL 73 413 984 28 DAVIS PB AM REV RESPIR DIS 129 911 984 29 SELTZER J AM REV RESPIR DIS 129 790 984 30 MISCHLER EH SEM RESPIR MED 6 271 985 31 WONNE R CLIN ALLERGY 15 455 985 32 MITCHELL I ANN ALLERGY 54 233 985 33 HORDVIK NL AM REV RESPIR DIS 131 889 985 34 DARGA LL PEDIATR PULMONOL 2 82 986 35 MACLUSKY IB PEDIATR PULMONOL 2 94 986 36 DAVIS PB HORM METAB RES 18 217 986 37 DAVIES RJ J ALLERGY CLIN IMMUNOL 78 1031 986 38 SULLIVAN MM CHEST 90 239 986 39 BARNES PJ AM REV RESPIR DIS 134 1289 986 40 STEAD RJ PROSTAGLANDINS LEUKOTR MED 26 91 987 41 GALVEZ RA J ALLERGY CLIN IMMUNOL 79 331 987 PN 78125 RN 00905 AN 78220043 AU Sorensen-R-U. Stern-R-C. Polmar-S-H. TI Lymphocyte responsiveness to Pseudomonas aeruginosa in cystic fibrosis: Relationship to status of pulmonary disease in sibling pairs. SO J-Pediatr. 1978 Aug. 93(2). P 201-5. MJ CYSTIC-FIBROSIS: im. LYMPHOCYTES: im. LYMPHOPENIA: et. PNEUMONIA: im. PSEUDOMONAS-INFECTIONS: im. MN ADOLESCENCE. ADULT. AGGLUTINATION-TESTS. ANTIGENS-BACTERIAL. CHILD. CYSTIC-FIBROSIS: fg. FEMALE. HUMAN. IMMUNITY-CELLULAR. IN-VITRO. LYMPHOPENIA: im. MALE. PNEUMONIA-STAPHYLOCOCCAL: im. PSEUDOMONAS-AERUGINOSA: im. STAPHYLOCOCCAL-INFECTIONS: im. STAPHYLOCOCCUS-AUREUS: im. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB Lymphocyte proliferative responses to Pseudomonas aeruginosa and Staphylococcus aureus were evaluated in six sibling pairs with cystic fibrosis. In each pair, one sibling had advanced clinical disease, whereas the other sibling was in good clinical condition. Three in this latter group had no clinically apparent Pseudomonas bronchitis. In all cases, the average responses to Pseudomonas isolated from each sibling pair were lower in the sibling with advanced clinical disease. This difference was not observed in the responses to Staphylococcus. Normal plasma or plasma from patients with CF in good clinical condition does not restore the responses in patients with advanced clinical disease. However, plasma from patients with low or no responses to Pseudomonas inhibits the responses of responding siblings. A progressive specific lymphocyte unresponsiveness to Pseudomonas may play an important role in the increasing destructiveness of chronic pulmonary Pseudomonas infection in cystic fibrosis. RF 001 TALAMO RC IN: MANGOS JA 195 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 004 BOXERBAUM B AM REV RESPIR DIS 108 777 973 005 THOMASSEN MJ J RETICULOENDOTHEL SOC 22 38A 977 006 HANN S INFECT IMMUN 14 114 976 007 HOIBY N SCAND J RESPIR DIS 56 38 975 008 SCHWARTZ RH AM J DIS CHILD 111 408 966 009 SOUTH MA J PEDIATR 71 645 967 010 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 011 GREEN GM AM REV RESPIR DIS 102 691 970 012 GREEN GM J EXP MED 119 167 964 013 KALTREIDER HB AM REV RESPIR DIS 113 347 976 014 WARR GA AM REV RESPIR DIS 108 371 973 015 SORENSEN RU INFECT IMMUN 18 735 977 016 THORSBY E HISTOCOMPATIBILITY TESTING 655 970 017 DOERSHUK CF J PEDIATR 65 677 964 018 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 THOMASSEN MJ PEDIATR RES 13 1085 979 2 CHURCH JA J PEDIATR 95 272 979 3 MCCARTHY MM CLIN PEDIATR 19 746 980 4 HODSON ME THORAX 35 801 980 5 CHURCH JA ANN ALLERGY 45 217 980 6 BEAUDRY PH J PEDIATR 97 144 980 7 MOSS RB AM REV RESPIR DIS 121 23 980 8 BALTIMORE RS J INFECT DIS 141 238 980 9 HARPER TB LUNG 157 219 980 10 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 11 SORENSEN RU PEDIATR RES 15 14 981 12 RUBIN HR CELL IMMUNOL 57 307 981 13 MARKS MI J PEDIATR 98 173 981 14 MOSS RB J PEDIATR 99 215 981 15 SORENSEN RU AM REV RESPIR DIS 123 37 981 16 FROLAND SS SCAND J INFECT DIS 1981 72 981 17 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 18 VANGEFFEL R IMMUNOL LETTERS 5 155 982 19 SEALE TW ANN CLIN LAB SCI 12 415 982 20 GOTZ M CLIN EXP IMMUNOL 50 178 982 21 HARPER TB AM REV RESPIR DIS 126 540 982 22 PORWOLL JM INFECT IMMUN 40 670 983 23 CAPLAN DB REV INFECT DIS 6 S705 984 24 PARMELY MJ J EXP MED 160 1338 984 25 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 26 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 27 DAVIS PB SEM RESPIR MED 6 261 985 28 KURLAND G PEDIATRICS 78 1097 986 29 PIEDRA P J PEDIATR 108 817 986 PN 78126 RN 00906 AN 78220060 AU Rosenstein-B-J. Plotnick-L-P. Blasco-P-A. TI Iodide-induced hypothyroidism without a goiter in an infant with cystic fibrosis. SO J-Pediatr. 1978 Aug. 93(2). P 261-2. MJ CYSTIC-FIBROSIS: co. HYPOTHYROIDISM: ci. POTASSIUM-IODIDE: ae. MN CASE-REPORT. CYSTIC-FIBROSIS: dt. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. POTASSIUM-IODIDE: tu. SUPPORT-U-S-GOVT-NON-P-H-S. AB It has been suggested that patients with cystic fibrosis may have an intrinsic abnormality of thyroid function that may be enhanced by the administration of iodides. We wish to report an infant with CF in whom hyperthyroidism developed, in the absence of a goiter, following short-term iodide therapy. RF 001 DOLAN TF JR J PEDIATR 79 684 971 002 AZIZI F TRANS ASSOC AM PHYSICIANS 87 111 974 003 BEGG TB Q J MED 32 351 963 004 SEGALL-BLANK M CF CLUB ABST 21 977 005 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 CHABROLLE JP ARCH FR PEDIATR 36 80 979 PN 78127 RN 00907 AN 78220113 AU Rich-R-H. Warwick-W-J. Leonard-A-S. TI Open thoracotomy and pleural abrasion in the treatment of spontaneous pneumothorax in cystic fibrosis. SO J-Pediatr-Surg. 1978 Jun. 13(3). P 237-42. MJ CYSTIC-FIBROSIS: co. PLEURA: su. PNEUMOTHORAX: su. THORAX: su. THORACIC-SURGERY. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INTUBATION. MALE. METHODS. PNEUMOTHORAX: dt, et. QUINACRINE: tu. RETROSPECTIVE-STUDIES. AB The various treatments of spontaneous pneumothorax in cystic fibrosis are examined over a 15-yr period. Open thoracotomy with pleural abrasion is compared to observation, tube thoracostomy, and tube thoracostomy with instillation of quinacrine. Open thoracotomy with pleural abrasion has been performed 31 times in 20 patients. Discussion centers around selection of patients, preoperative preparation, operative technique, and postoperative care and follow- up, including analysis of pulmonary function studies. In our experience, open thoracotomy with pleural abrasion is a safe and effective method that should be utilized in the management of pneumothorax in patients with cystic fibrosis. RF 001 GRAHAM WP 3RD SURG GYNECOL OBSTET 122 373 966 002 SCHUSTER SR J THORAC CARDIOVASC SURG 48 750 964 003 HOLSCLAW DS CLIN PEDIATR 9 346 970 004 HOLSCLAW DS MINN MED 52 1547 969 005 MEARNS MB ARCH DIS CHILD 47 499 972 006 GIBBS GE MINN MED 52 1433 969 007 WYLIE RH J THORAC CARDIOVASC SURG 48 770 964 008 LIFSCHITZ MI AM J DIS CHILD 116 633 968 009 BOAT TF JAMA 209 1498 969 010 SHWACHMAN H MINN MED 52 1521 969 011 JONES RE AM J DIS CHILD 130 777 976 012 KATTWINKEL J JAMA 226 557 973 013 BROOKS JW ANN SURG 177 798 973 014 SAHA SP ANN THORAC SURG 19 561 975 015 MITCHELL-HEGGS PF THORAX 25 165 970 016 STOWE SM AM REV RESPIR DIS 111 611 975 CT 1 SCHUSTER SR J PEDIATR SURG 18 492 983 2 WEEDEN D THORAX 38 737 983 3 ROBB KL J ECON ENTOMOL 77 1288 984 4 MISRA M BULL INST ZOOL ACAD SINICA 24 85 985 PN 78128 RN 00908 AN 78244407 AU Flanigan-R-C. Stern-R-C. Izant-R-J-Jr. Filston-H-C. TI Cystic fibrosis in a black infant: presentation with meconium ileus and volvulus. SO J-Pediatr-Surg. 1978 Aug. 13(4). P 435-6. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: su. INTESTINE-SMALL: su. MECONIUM. MN CASE-REPORT. COLON: ab. DUCTUS-ARTERIOSUS-PATENT: co. FEMALE. HUMAN. ILEOSTOMY. ILEUM: su. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co. JEJUNUM: su. NEGROID-RACE. PREGNANCY. AB This is a case report of meconium ileus complicated by intestinal volvulus and infarction in a black infant. RF 001 STERN RC J PEDIATR 89 412 976 002 KULCZYCKI LL CLIN PEDIATR 3 692 964 003 KULCZYCKI LL AM J DIS CHILD 127 64 974 004 WAGGET J J PEDIATR SURG 5 649 970 005 KALAYOGLU M J PEDIATR SURG 6 290 971 006 BISHOP HC ANN SURG 145 410 957 CT 1 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 PN 78129 RN 00909 AN 79050049 AU Shigemoto-H. Endo-S. Isomoto-T. Sano-K. Taguchi-K. TI Neonatal meconium obstruction in the ileum without mucoviscidosis. SO J-Pediatr-Surg. 1978 Oct. 13(6). P 475-9. MJ CYSTIC-FIBROSIS. ILEUM. INFANT-NEWBORN-DISEASES: th. INTESTINAL-OBSTRUCTION: th. MECONIUM. MN CASE-REPORT. CONTRAST-MEDIA: tu. ENEMA. FEMALE. HUMAN. HYPERTONIC-SOLUTIONS. HYPERTROPHY: et. ILEUM: pa. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: et, pa. INTESTINAL-MUCOSA: se. INTESTINAL-OBSTRUCTION: et, pa. MALE. MUCUS: se. AB Two newborns with intestinal obstruction of the terminal ileum without mucoviscidosis were cured by Gastrografin enema. A further case of a premature infant showed, at autopsy, a large intestine containing normal meconium, but an ileum which was plugged with tenacious meconium containing PAS-positive and alcian blue-negative mucus. Our 3 cases were similar to cases of "meconium disease" reported by Rickham and of "meconium plug in the small intestine" reported by Emery. The primary cause of this disease lies in the large amount of PAS-positive mucus secreted from goblet cells of the ileal mucosa and the hypertrophy of lymph nodules in the ileal submucosa. RF 001 LEUBNER H PEDIATRICS 15 135 955 002 DI SANTAGNESE PA PEDIATRICS 22 507 958 003 BERTI G ARCH KINDERHEILK 24 463 898 004 CLATWORTHY HW SURGERY 39 131 956 005 RICKHAM PP AM J SURG 109 173 965 006 KORNBLITH BA AM J PATHOL 5 249 929 007 HURWITT ES AM J DIS CHILD 64 443 942 008 EMERY JL ARCH DIS CHILD 32 17 957 009 ZACHARY RB ARCH DIS CHILD 32 22 957 010 DOLAN TF JR J PEDIATR SURG 9 821 974 CT 1 SIEGEL MJ RADIOLOGY 132 79 979 2 YOSHIKAWA Y ACTA PATHOL JAP 31 845 981 3 HILLEMEIER AC PEDIATRICS 69 325 982 4 TAL A CLIN PEDIATR 24 460 985 5 SAMUEL N J ULTRASOUND MED 5 425 986 6 KING A PEDIATR RES 20 536 986 PN 78130 RN 00910 AN 78221008 AU Lloyd-Still-J-D. TI Assisted ventilation in cystic fibrosis [letter]. SO JAMA. 1978 Aug 25. 240(8). P 739. MJ CYSTIC-FIBROSIS: co. RESPIRATION-ARTIFICIAL. RESPIRATORY-INSUFFICIENCY: th. MN HUMAN. INFANT. RESPIRATORY-DISTRESS-SYNDROME: th. RESPIRATORY-INSUFFICIENCY: et. EX My colleagues and I reviewed the diagnosis, management, and prognosis in 17 cases of infants with cystic fibrosis and severe respiratory distress who were admitted to the Children's Hospital Medical Center, Boston, between 1968 and 1972. There was a 60% mortality overall. Six of the 17 infants were treated with assisted ventilation, of whom only one survived. Our subsequent experience with assisted ventilation has shown no improvement in the survival of these infants. RF 000 DAVIS PB JAMA 239 1851 978 001 LLOYD-STILL JD PEDIATRICS 53 678 974 PN 78131 RN 00911 AN 79050797 AU Garrison-D-W. TI Parents' understanding of genetic risk data in genetic counseling [letter]. SO JAMA. 1978 Dec 8. 240(24). P 2631-2. MJ CYSTIC-FIBROSIS: fg. GENETIC-COUNSELING. MN HUMAN. RISK. EX Relatively little has been published on the parents' ability to grasp the importance of empirical risk data and Mendelian-type probabilities. The basic problem is that for many reasons, consumers do not know what the recurrent risks are for the various genetic diseases, not only cystic fibrosis. As is so often the problem in health care, the most effective means to communicate health care information is not used or even known. Until the problem of communication of probability can be worked out, I would like to make some suggestions in trying to help those who are provided with probability data. Always record probability values discussed in the genetic counseling session. If dealing with a family physician, the information should become a part of the medical history for future reference. When providing documentation of probabilities, always include the interpretation of such probabilities through diagrams, charts, and other simple means. Use available pamphlets and brochures provided by various organizations such as foundations and departments of health. RF 000 CHECK W JAMA 240 819 978 001 LEONARD CO N ENGL J MED 287 433 972 002 CARTER CO LANCET 1 281 971 CT 1 WHITTEN CF AM J HUM GENET 33 802 981 PN 78132 RN 00912 AN 78197203 AU Kulczycki-L-L. Murphy-T-M. Bellanti-J-A. TI Pseudomonas colonization in cystic fibrosis. A study of 160 patients. SO JAMA. 1978 Jul 7. 240(1). P 30-4. MJ BRONCHI: mi. CARRIER-STATE: mi. CYSTIC-FIBROSIS: co. PSEUDOMONAS-AERUGINOSA: ip. PSEUDOMONAS-INFECTIONS: co. MN ADOLESCENCE. ADULT. ANTIBIOTICS: pd, tu. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mi. DISEASE-SUSCEPTIBILITY. DRUG-RESISTANCE-MICROBIAL. FOLLOW-UP-STUDIES. HUMAN. INFANT. PSEUDOMONAS-AERUGINOSA: de, gd. PSEUDOMONAS-INFECTIONS: dt. SUPPORT-U-S-GOVT-NON-P-H-S. AB We investigated the role of Pseudomonas aeruginosa colonization in the respiratory tracts of cystic fibrosis (CF) patients to relate the effect of this colonization to progression of bronchial airway pathologic conditions and to the patients' clinical progress, and to identify predisposing factors to persistence of P aeruginosa colonization and bronchial tree damage. Half of 160 CF patients studied had persistent P aeruginosa respiratory tract colonization; the other half had none. Pseudomonas aeruginosa seems to have an exclusive propensity for the respiratory tract and may appear at any age. Treatment with antibiotics, including aminoglycosides, failed to eradicate P aeruginosa. The continuous use of antibiotics seemed to contribute to the persistence of P aeruginosa and the appearance of mucoid strains of P aeruginosa. RF 001 REYNOLDS HY JAMA 236 2190 976 002 ELSTON HR AM J CLIN PATHOL 48 519 967 003 HARRISON GM CF CLUB ABST 16 42 975 004 LARAYA-CUASAY LR J PEDIATR 89 23 976 005 GIBSON LE PEDIATRICS 23 545 959 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 ALTMAN RP J PEDIATR SURG 8 809 973 008 WARING WW PEDIATRICS 39 166 967 009 DOGGETT RG LANCET 1 236 971 010 REYNOLDS HY ANN INTERN MED 82 819 975 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 012 ZIERDT CH J CLIN MICROBIOL 1 521 975 013 BELLANTI JA PROC INT CF CONG 7TH 20 976 014 PENNINGTON JE J INFECT DIS 131 158 975 015 MILLIS RM CHEST 71 508 977 016 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 017 HUANG NN J PEDIATR 59 512 961 018 BURNS MW LANCET 1 270 968 019 DOERSHUK CF IN: MANGOS JA 976 CT 1 KULCZYCKI LL MONOGR PAEDIATR 10 27 979 2 LOENINGBAUCKE VA J PEDIATR 95 630 979 3 MCCARTHY MM CLIN PEDIATR 19 746 980 4 LAM J INFECT IMMUN 28 546 980 5 BEAUDRY PH J PEDIATR 97 144 980 6 WIENTZEN R AM J DIS CHILD 134 1134 980 7 BALTIMORE RS J INFECT DIS 141 238 980 8 CARLONE S MICROBIOLOGICA 4 215 981 9 KOEPP LH CURRENT MICROBIOL 6 383 981 10 MOSS RB J PEDIATR 99 215 981 11 BAKER NR CURRENT MICROBIOL 7 35 982 12 BAKER NR IN VITRO 18 369 982 13 SORDELLI DO CLIN IMMUNOL IMMUNOPATHOL 22 153 982 14 BAKER NR CAN J MICROBIOL 28 248 982 15 BALTIMORE RS HELV PAEDIATR ACTA 37 547 982 16 BOYCE JR INFECT IMMUN 37 695 982 17 BOYCE JR INFECT IMMUN 37 840 982 18 OHMAN DE INFECT IMMUN 37 662 982 19 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 20 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 21 BODEY GP REV INFECT DIS 5 279 983 22 CROSS A REV INFECT DIS 5 S837 983 23 GOVAN JRW REV INFECT DIS 5 S874 983 24 BALTIMORE RS PEDIATR RES 17 952 983 25 CUKOR G J CLIN MICROBIOL 18 457 983 26 BOYD RL INFECT IMMUN 39 1403 983 27 HASTIE AT INFECT IMMUN 40 506 983 28 DORING G INFECT IMMUN 42 197 983 29 PENKETH A AM REV RESPIR DIS 127 605 983 30 HACKING AJ J GEN MICROBIOL 129 3473 983 31 BOUCHER RC LUNG 161 1 983 32 ADLER KB JAMA 249 1615 983 33 CHAN R J CLIN MICROBIOL 19 8 984 34 PENKETH A THORAX 39 299 984 35 BUCKMIRE FLA MICROBIOS 41 49 984 36 KUHN RJ CLIN PHARMACY 4 555 985 37 EFTIMIADI C DRUGS UNDER EXP CLIN RES 11 241 985 38 OLIVER AM J CLIN LAB IMMUNOL 17 85 985 39 IRVIN RT CAN J MICROBIOL 31 268 985 40 STACK BHR THORAX 40 358 985 41 JACQUOT J INFECT IMMUN 47 555 985 42 MARCUS H INFECT IMMUN 47 723 985 43 SORDELLI DO INFECT IMMUN 49 265 985 44 WINKLER U KLIN WSCHR 63 490 985 45 LINDEMANN RA J DENT RES 64 54 985 46 SORDELLI DO EUR J RESPIR DIS 67 118 985 47 TABLAN OC J PEDIATR 107 382 985 48 BROWN J AM J DIS CHILD 139 836 985 49 GOVAN JRW MICROBIOL SCI 3 302 986 50 FRIEND PA J INFECT 13 55 986 51 PADGETT PJ CURRENT MICROBIOL 14 187 986 52 PEDERSEN SS J ANTIMICROB CHEMOTHER 17 505 986 53 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 54 ADLER KB AM J PATHOL 125 501 986 55 STUTTS MJ AM REV RESPIR DIS 134 17 986 56 LONNQVIST B J INFECT DIS 153 175 986 57 DUNNE WM DIAGN MICROBIOL INFECT DIS 6 165 987 58 BOUCHER RC TOXICOL APPL PHARMACOL 87 264 987 59 GOLDBERG JB J BACTERIOL 169 1349 987 PN 78133 RN 00913 AN 78174206 AU Stern-R-C. Boat-T-F. Abramowsky-C-R. Matthews-L-W. Wood-R-E. Doershuk-C-F. TI Intermediate-range sweat chloride concentration and Pseudomonas bronchitis. A cystic fibrosis variant with preservation of exocrine pancreatic function. SO JAMA. 1978 Jun 23. 239(25). P 2676-80. MJ BRONCHITIS: co. CHLORIDES: an. CYSTIC-FIBROSIS: fg. PSEUDOMONAS-INFECTIONS: co. SWEAT: an. MN ADOLESCENCE. BRONCHITIS: et. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, di. DIAGNOSIS-DIFFERENTIAL. FEMALE. GENES. HUMAN. MALE. PEDIGREE. SUPPORT-U-S-GOVT-P-H-S. AB We studied the clinical and laboratory characteristics of seven patients with sweat chloride concentration consistently between 40 and 60 mEq/liter. Each has chronic Pseudomonas bronchitis, and all lack digestive symptoms. Laboratory findings indicate the preservation of exocrine pancreatic function. The patients include two of five children in one family and two of four in another. In a third family, one of five siblings has an intermediate sweat chloride concentration, but another has a typical fibrosis value (105 mEq/liter). One patient died of respiratory failure; results of an autopsy showed bronchiolectasis typical of cystic fibrosis, but minimal pancreatic changes. The data suggest a genetic basis for this variant of cystic fibrosis. These patients may be homozygous for a portion of a closely linked multigene cystic fibrosis locus or may have modifier genes that ameliorate the pancreatic and sweat lesions. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 SHWACHMAN H ADV PEDIATR 7 249 955 003 GIBSON LE PEDIATRICS 23 545 959 004 WOOD RE AM REV RESPIR DIS 113 833 976 005 COGSWELL JJ ARCH DIS CHILD 49 520 974 006 SARSFIELD JK ARCH DIS CHILD 50 463 975 007 OTSUKI M CLIN CHIM ACTA 22 439 976 008 SHWACHMAN H AM J DIS CHILD 92 347 956 009 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 010 LOBECK CC PEDIATRICS 30 172 962 011 SHWACHMAN H PEDIATRICS 36 689 965 012 TAUSSIG LM PEDIATRICS 54 229 974 013 SHWACHMAN H PEDIATRICS 55 86 975 014 DENNING CR PEDIATRICS 41 7 968 015 ANDERSON CM ARCH DIS CHILD 35 581 960 016 GHARIB R AM J DIS CHILD 108 499 964 017 LARAYA-CUASAY LR J PEDIATR 89 23 976 018 KISSMEYER-NIELSEN F NATURE 224 75 969 019 FAGERHOL MK PROG MED GENET 7 96 970 020 GALDSTON M AM REV RESPIR DIS 107 718 973 CT 1 ANON LANCET 2 1032 978 2 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 3 DAVIS PB AM J MED 69 643 980 4 SHWACHMAN H ARCH DIS CHILD 56 137 981 5 COLTEN HR N ENGL J MED 304 831 981 6 STERN RC LANCET 1 1401 982 7 BARBERO GJ J PEDIATR 100 914 982 8 GASKIN K J PEDIATR 100 857 982 9 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 10 GUIDOTTI TL RESPIRATION 44 351 983 11 BLYTHE SA CLIN BIOCHEM 17 277 984 12 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 13 FISHER JH AM REV RESPIR DIS 132 1149 985 14 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 15 BROWN RF SOUTH MED J 79 1430 986 16 ROSENSTEIN BJ SOUTH MED J 79 319 986 17 FERNALD GW SEM ROENTGENOL 22 87 987 PN 78134 RN 00914 AN 78153979 AU Davis-P-B. di-SantAgnese-P-A. TI Assisted ventilation for patients with cystic fibrosis. SO JAMA. 1978 May 5. 239(18). P 1851-4. MJ CYSTIC-FIBROSIS: th. RESPIRATION-ARTIFICIAL. MN ACUTE-DISEASE. ADOLESCENCE. ADULT. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. COMPARATIVE-STUDY. HUMAN. INFANT. LUNG-DISEASES-OBSTRUCTIVE: th. REMISSION-SPONTANEOUS. RESPIRATORY-INSUFFICIENCY: th. TIME-FACTORS. AB Forty-six patients with cystic fibrosis (CF), 1 month to 32 years of age, had 51 episodes of assisted ventilation. Thirty-five (69%) died after 1 to 395 days of mechanical ventilation and six others (12%) died in the hospital 1 to 42 days after assisted ventilation therapy was discontinued. Only three patients survived longer than one year after discharge. These results are much poorer than those reported for patients with chronic obstructive pulmonary disease of other causes probably because of the different natural history and response to therapy in CF. Patients with CF whose conditions progressively deteriorated despite optimal therapy and no longer respond to antibiotic and physical therapy are not good candidates for mechanical ventilation. The occasional patient with good baseline status in whom acute respiratory failure develops should be considered for assisted ventilation. RF 001 ZWILLICH CW AM J MED 57 161 974 002 PETTY TL JAMA 233 34 975 003 ROGERS RM CHEST SUPPL 62 95 972 004 ODONOHUE WJ CHEST 58 603 970 005 JESSEN O LANCET 2 9 967 006 WEISS EB ANN INTERN MED 67 556 967 007 BRADLEY RD LANCET 1 854 964 008 ANON GAP CONF REP PULMONARY COMPLI 10 972 009 ANON GAP CONF REP CHRON BRONC CF 17 977 010 SHWACHMAN H N ENGL J MED 296 1519 977 011 BIGELOW DB IN: PETTY TL 240 974 012 DI SANTAGNESE PA IN: DOWNEY JA 25 974 013 DAVIS PB AM REV RESPIR DIS SUPPL 115 278 977 014 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 015 ALBERTINI RE CHEST 67 134 975 016 SALISBURY BG THORAX 30 441 975 017 ROGERS RM N ENGL J MED 286 1230 972 CT 1 SANTAGNESE PAD AM J MED 66 121 979 2 WOOD RE SOUTH MED J 72 189 979 3 PETTY TL SEM RESPIR MED 3 263 982 4 KNAUS WA JAMA 249 1059 983 5 NUSSBAUM E CLIN PEDIATR 24 379 985 6 SCOGGIN CH POSTGRAD MED 77 243 985 7 MAKE BJ CLIN CHEST MED 7 679 986 8 ODONOHUE WJ CHEST 90 S 1 986 PN 78135 RN 00915 AN 79007746 AU Bonforte-R-J. TI Variability in cystic fibrosis [letter]. SO JAMA. 1978 Oct 20. 240(17). P 1855. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: di. DIAGNOSIS-DIFFERENTIAL. HUMAN. EX All of us who care for patients with cystic fibrosis (CF) are well aware of the varying degrees of clinical involvement they have. It has long been known that whereas the most patients have pulmonary involvement of one degree or another, approximately 15% to 20% of patients will not have any pancreatic involvement. In the 80% to 85% of patients with pancreatic involvement, the degree of involvement can vary greatly. If we can accept the concept of clinical heterogeneity for the pulmonary and gastrointestinal systems, there is no reason why we should doubt the existence of clinical variability for the other major organ system affected by this disease, the sweat glands. RF 000 STERN RC JAMA 239 2676 978 PN 78136 RN 00916 AN 79007795 AU Rosenstein-B-J. Langbaum-T-S. Gordes-E. Brusilow-S-W. TI Cystic fibrosis. Problems encountered with sweat testing. SO JAMA. 1978 Oct 27. 240(18). P 1987-8. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHLORIDES: an. FALSE-NEGATIVE-REACTIONS. FALSE-POSITIVE-REACTIONS. HUMAN. IONTOPHORESIS. PILOCARPINE: du. SPECIMEN-HANDLING: st. AB The sweat test is the only practical and reliable laboratory test for confirmation of the diagnosis of cystic fibrosis. Among 234 patients referred to The Johns Hopkins Hospital for sweat testing, 62 had had a previous test; 29 tests had been reported as negative, and 33 tests had been reported as positive. Results of quantitative pilocarpine- iontophoresis sweat tests at this center led to a change in diagnosis in 27 (43.5%) of these 62 patients. Most of the errors were false- positive. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 DARLING RC AM J MED SCI 225 67 953 003 GIBSON LE PEDIATRICS 23 545 959 004 ANON J PEDIATR 88 711 976 005 KOPITO L PEDIATRICS 43 794 969 006 SHWACHMAN H J PEDIATR 66 432 965 007 ANON GAP CONF REP PROB SWEAT TESTI 975 008 MACLEAN WC JR J PEDIATR 83 86 973 009 GIBSON LE CLIN PEDIATR 12 450 973 010 BRAY PT CLIN CHIM ACTA 80 333 977 011 WARING WW ADV PEDIATR 23 401 976 CT 1 PRIEST JB JAMA 242 1970 979 2 SCHWACHMAN H J PEDIATR 98 576 981 3 WONG LTK GUT 23 744 982 4 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 5 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 6 KIRK JM ANN CLIN BIOCHEM 20 369 983 7 ORENSTEIN DM SEM RESPIR MED 6 252 985 8 CHRISTOFFEL KS J PEDIATR 107 231 985 9 TAGLE S REV MED CHIL 113 758 985 10 ROSENSTEIN BJ CLIN PEDIATR 26 78 987 PN 78137 RN 00917 AN 79175879 AU Cho-Y-J. Oh-Y-H. Abe-C. Homma-J-Y. Usui-M. Matuhasi-T. TI IgE antibody production to exoenzymes and common antigen (OEP) of Pseudomonas aeruginosa in mice. SO Jpn-J-Exp-Med. 1978 Dec. 48(6). P 491-6. MJ ANTIBODY-FORMATION. IGE: bi. PANCREATOPEPTIDASE: im. PEPTIDE-HYDROLASES: im. PSEUDOMONAS-AERUGINOSA: im. MN ANIMAL. ANTIBODIES-BACTERIAL: im. ANTIGENS-BACTERIAL: im. CYSTIC-FIBROSIS: im. ENDOTOXINS: an. FEMALE. HUMAN. MALE. MICE. PSEUDOMONAS-AERUGINOSA: en. TOXOIDS: im. AB IgE antibody production to exoenzymes and common antigen (OEP) of P. aeruginosa was studied in mice that were injected with the antigens incorporated into water-in-oil-in-water (w/o/w) emulsion or Al(OH)3 gel. OEP and protease toxoid (PT) elicited IgE antibody response but elastase toxoid (ET) did not. Capacity of the OEP to produce IgE antibody was reduced remarkably by it's protease treatment, which suggested that the capacity lies in the protein portion of OEP. The w/o/w emulsion was less effective than Al(OH)3 gel in adjuvanticity to elicit IgE antibody response, but the emulsion enhanced the IgM and/or IgG antibody response to PT and maintained a constant level for a long period. These findings may suggest that IgE antibody response to some components of P. aeruginosa could be induced in man. High serum level of IgE was observed in some cases of cystic fibrosis caused by P. aeruginosa infection, although the IgE antibody activity has not yet been determined. RF 001 SORENSEN RU INFECT IMMUN 18 735 977 002 HOMMA JY JAPAN J EXP MED 45 361 975 003 DANNEMAN PJ INFECT IMMUN 14 694 976 004 MATUHASI T NATURE NEW BIOL 245 213 973 005 HOMMA JY ANIMAL PLANT MICROBIOL TOXINS 1 499 976 006 ABE C JAPAN J EXP MED 47 393 977 007 TANAMOTO K J BIOCHEM (TOKYO) 83 711 978 008 MORIHARA K BIOCHIM BIOPHYS ACTA 73 113 963 009 MORIHARA K J BIOL CHEM 240 3295 965 010 MORIHARA K AGRIC BIOL CHEM 39 1123 975 011 HOMMA JY JAPAN J EXP MED 48 111 978 012 MOTA I LIFE SCI 8 813 969 013 TOMIYAMA T JAPAN J EXP MED 43 185 973 014 KIMURA J JAPAN J EXP MED 48 149 978 CT 1 ABE C JAP J EXP MED 51 71 981 2 PAUWELS R ALLERGY 38 239 983 3 HOMMA JY J CLIN MICROBIOL 20 855 984 PN 78138 RN 00918 AN 79155504 AU Araki-H. Nakamura-K. TI Effect of sera from cystic fibrosis homozygotes and heterozygotes on glucose metabolism in Vero cells. SO Lancet. 1978 Apr 15. 1(8068). P 793-4. MJ CYSTIC-FIBROSIS: bl. GLUCOSE: me. MN ANIMAL. CARBON-DIOXIDE. CELL-LINE. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. KIDNEY. ANTHROPOIDEA. AB The effect on intracellular production of carbon dioxide from glucose of sera from cystic fibrosis (C.F.) homozygotes and heterozygotes was determined in an established cell line (Vero cell) by a double-blind assay. CO2 production was reduced when cells were incubated with an ammonium-sulphate-precipitated fraction of sera from C.F. homozygotes and heterozygotes but not by a similar fraction from sera of healthy donors. RF 001 BALFE JW SCIENCE 162 689 968 002 MANGOS JA PEDIATR RES 2 378 968 003 SPOCK A PEDIATR RES 1 173 967 004 LAPEY A PEDIATR RES 5 446 971 005 FEIG SA PEDIATR RES 8 594 974 006 FITZPATRICK DF NATURE NEW BIOL 235 173 972 007 DUFFY MJ NATURE NEW BIOL 246 151 973 008 ARAKI H PEDIATR RES 9 932 975 009 YASUMURA T NIPPON RINSHO 21 1201 963 010 WARSHAW JB CELL BIOL 52 283 972 011 GOLDNER AM J GEN PHYSIOL 53 362 969 012 ARAKI H PROC INT CF CONG 7TH 28 976 013$ BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 14 253 975 014 DANES BS J EXP MED 137 1538 973 CT 1 NAKAMURA K J NATL CANCER INST 77 1035 986 PN 78139 RN 00919 AN 78245770 AU Dodge-J-A. Hamdi-I. TI Abnormal levels of prostaglandins and fatty acids in cystic fibrosis [letter]. SO Lancet. 1978 Aug 26. 2(8087). P 475. MJ CYSTIC-FIBROSIS: me. LINOLEIC-ACIDS: df. PROSTAGLANDINS-E: bi. PROSTAGLANDINS-F: bi. MN HUMAN. EX Like Dr Chase and Dr Dupont we have found essential fatty-acid deficiency, usually subclinical, in cystic fibrosis patients. However, we feel that their data on prostaglandin synthesis may have other interpretations. In patients with deficiency of linoleic acid, oleic acid is used an alternative substrate; and instead of dihomogammalinoleic acid, an alternative 20:3 fatty acid is produced. We have found increased amounts of this substance in the plasma of patients with cystic fibrosis. We agree with the authors' conclusion that levels of linoleic acid are specifically related to P.G. production in children with cystic fibrosis. It remains to be shown whether children with cystic fibrosis have a primary abnormality of fatty-acid metabolism or whether the observed disturbances of prostaglandin metabolism are secondary to malabsorption of dietary essential fatty acids. In either case, certain clinical features of the disease may well be explained by prostaglandin disturbances. RF 000 CHASE HP LANCET 2 236 978 001 YASSA JG PROC INT CF CONG 7TH 323 976 002 ROBINSON PG PROC INT CF CONG 7TH 107 976 003 VAN EVERT WE PROSTAGLANDINS 15 268 978 004 LEMEN RJ AM REV RESPIR DIS 117 639 978 CT 1 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 PN 78140 RN 00920 AN 78245706 AU Smalley-C-A. Addy-D-P. Anderson-C-M. TI Does that child really have cystic fibrosis?. SO Lancet. 1978 Aug 19. 2(8086). P 415-7. MJ CYSTIC-FIBROSIS: di. MN AMYLASES: an. BICARBONATES: an. CHILD. CHILD-PRESCHOOL. CHLORIDES: an. CHOLECYSTOKININ: du. EVALUATION-STUDIES. FALSE-POSITIVE-REACTIONS. HUMAN. LIPASE: an. PANCREAS: pp. SECRETIN: du. SODIUM: an. SWEAT: an. TRYPSIN: an. AB A diagnosis of cystic fibrosis was incorrectly made after false- positive sweat tests in 14 children. 13 of these children had been tested at hospitals where it seems likely that sweat tests were not done very often. All the children had normal sweat-electrolyte values when the test was repeated at a regional paediatric centre where approximately 250 sweat tests are done each year. In 5 cases, detailed testing of pancreatic function was normal. None of the children had typical chest disease and only 2 had gastrointestinal symptoms. In the absence of the typical clinical features of the disease a diagnosis of cystic fibrosis should be made with extreme caution and only after meticulous testing of both sweat electrolytes and pancreatic function. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 SCHWARZ V IN: DAVIS JA 545 974 003 GIBSON LE J PEDIATR 81 193 972 004 SCHWARZ V ARCH DIS CHILD 52 870 977 005 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 006 ANON J PEDIATR 88 711 976 007 GIBSON LE PEDIATRICS 23 545 959 008 SCHALES O J BIOL CHEM 140 879 941 009 HADORN B J PEDIATR 73 39 968 010 SCHWERT GW J BIOL CHEM 172 221 948 011 VAN DE KAMER JH J BIOL CHEM 177 347 949 012 HADORN B CAN MED ASSOC J 98 377 968 013 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 CT 1 ANON LANCET 2 1032 978 2 STAGG BH ANN CLIN BIOCHEM 16 147 979 3 DANDONA P THORAX 36 60 981 4 ANON LANCET 2 1196 982 5 DAVID TJ LANCET 2 1204 982 6 GASKIN KJ PEDIATR RES 16 554 982 7 WONG LTK GUT 23 744 982 8 BROWN RC J CLIN PATHOL 35 547 982 9 HEELEY AF CLIN CHEM 29 2011 983 10 LITTLEWOOD JM BR MED J 287 501 983 11 CARTER EP ARCH DIS CHILD 59 919 984 12 PERINI JM BULL EUR PHYSIOPATH RESP 21 569 985 13 GREEN A ANN CLIN BIOCHEM 22 171 985 14 CUMMING JGR ARCH DIS CHILD 61 573 986 15 LITTLEWOOD JM ARCH DIS CHILD 61 1041 986 16 DAVIDSON GP ACTA PAEDIATR SCAND 75 145 986 17 ROSENSTEIN BJ CLIN PEDIATR 26 78 987 PN 78141 RN 00921 AN 78245699 AU Dann-L-G. Blau-K. TI Exocrine-gland function and the basic biochemical defect in cystic fibrosis. SO Lancet. 1978 Aug 19. 2(8086). P 405-7. MJ CYSTIC-FIBROSIS: et. SALIVARY-GLANDS: me. MN ABSORPTION. ARGININE. CALCIUM: me. CELL-MEMBRANE: me. CHLORINE: me. CYSTIC-FIBROSIS: en, me. ESTERASES: df. HUMAN. KININS: bi. MODELS-BIOLOGICAL. SALIVARY-GLANDS: pa, se. SODIUM: me. AB A biochemical link is proposed between secretion and reabsorption in exocrine glands, in the form of a factor (kinin E) which initiates ion reabsorption. Activation of this factor involves arginine esterases, and in cystic fibrosis these may be defective, preventing the formation of kinin E. In cystic fibrosis stimulation of exocrine glands produces abnormal reabsorption of ions, leading to the physiological abnormalities and clinical manifestations of the disease. RF 001 PETERSEN OH PHIL TRANS R SOC LOND BIOL 262 307 971 002 GARRETT JR ARCH ORAL BIOL 12 1417 967 003 FOX RH J PHYSIOL (LOND) 142 2119 958 004 ULLRICH KJ IN: THORN NA 423 974 005 MANGOS JA AM J PHYSIOL 224 1235 973 006 SUTCLIFFE CH PROC R SOC MED 61 297 968 007 MANGOS JA IN: MANGOS JA 311 976 008 RAO GJS SCIENCE 177 610 972 009 CHAN KYH CLIN CHIM ACTA 74 71 977 010 COBURN MD AM REV RESPIR DIS 110 368 974 011 RAO GJS PEDIATR RES 8 684 974 012 RAO GJS PEDIATR RES 9 739 975 013 NUSTAD K GEN PHARMAC 9 1 978 014 FRAKI JE ACTA DERM VENEREOL (STOCKH) 50 321 970 015 BEILENSON S J PHYSIOL (LOND) 199 303 968 016 GARRETT JR IN: THORN NA 487 974 017$ FINK E ANAL CHEM 290 183 978 018 BOWMAN BH CLIN GENET 12 333 977 019 MANGOS JA SCIENCE 158 135 967 020 MANGOS JA PEDIATR RES 2 378 968 021 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 022 MARTINEZ JR PEDIATR RES 9 470 975 023 WHITELAW A LANCET 2 1288 977 024 POTTER JL ANN NY ACAD SCI 106 692 963 025 GUGLER EC J PEDIATR 71 585 967 CT 1 BROCK DJH LANCET 1 1245 979 2 DANN LG LANCET 2 907 979 3 WILSON GB PEDIATR RES 13 1079 979 4 TARNOKY AL J ROY SOC MED 73 73 980 5 DUFFY MJ CLIN CHIM ACTA 103 233 980 6 STEGMAYR B ULTRASTRUCTURAL PATHOL 2 357 981 7 HALLINAN F MED HYPOTHESES 7 793 981 8 BURY AF PEDIATR RES 16 613 982 9 GREEN JR EUR J PEDIATR 139 35 982 10 HEELEY AF CLIN CHEM 29 2011 983 11 HARE ER PEDIATR RES 19 938 985 12 RUPP GM MED HYPOTHESES 20 245 986 PN 78142 RN 00922 AN 78112941 AU Kilbourn-J-P. TI Bacterial content and ionic composition of sputum in cystic fibrosis [letter]. SO Lancet. 1978 Feb 11. 1(8059). P 334. MJ BACTERIA. CYSTIC-FIBROSIS. ELECTROLYTES. SPUTUM. MN BACTERIA: ip. CALCIUM: an. CANDIDA-ALBICANS: ip. CYSTIC-FIBROSIS: mi. ELECTROLYTES: an. HUMAN. POTASSIUM: an. SODIUM: an. SPUTUM: an, mi. EX Burns and May proposed the following pattern for the bacteriology of cystic fibrosis: (1) Staphylococcus aureus is the initial bacterial pathogen, and damage caused by it renders the lungs susceptible to infection by other species. (2) Control of S. aureus by antibiotics allows the influx of Haemophilus influenzae. (3) Pseudomonas aeruginosa is introduced and continued chemotherapy encourages its establishment. (4) P. aeruginosa supplants S. aureus and H. influenzae, becoming the sole pathogen. I have examined these ideas by quantitative culture of sputum from 20 patients seen at the Cystic Fibrosis Treatment and Research Center, University of Oregon Health Sciences Center from 1965 to 1977. The results accord with the original hypothesis of Burns and May. In 13 of 20 patients the sputum bacterial flora changed as the hypothesis of Burns and May predicted. This preliminary data suggests that one of the primary sputum abnormalities in cystic fibrosis may be increased Ca++ which makes the sputum susceptible to colonisation by S. aureus. After infection the Ca++ concentration is reduced and the lung becomes susceptible to colonisation by other microorganisms including H. influenzae, P. aeruginosa, and Escherichia coli. RF 001 BURNS MW LANCET 1 270 958 002 MAY JR ARCH DIS CHILD 47 908 972 003 KILBOURN JP AM REV RESPIR DIS 98 810 968 004 KILBOURN JP LANCET 2 878 970 005 KILBOURN JP LANCET 1 405 974 006 MATTHEWS LW AM REV RESPIR DIS 88 199 963 007 POTTER JL AM REV RESPIR DIS 96 83 967 008 POTTER JL ANN NY ACAD SCI 106 692 963 009 CHERNICK WS PEDIATRICS 24 739 959 CT 1 BOYCE JR LANCET 2 268 980 2 KILBOURN JP PEDIATR RES 14 259 980 3 LINKE HAB MICROBIOS 27 107 980 4 BOYCE JR INFECT IMMUN 37 695 982 5 BOYCE JR INFECT IMMUN 37 840 982 6 PIER GB J INFECT DIS 147 494 983 7 KILBOURN JP CURRENT MICROBIOL 11 19 984 8 MULLER RM SCANN ELECTRON MICROSC 1985 1583 985 PN 78143 RN 00923 AN 78091015 AU Sparham-P-D. Lobban-D-I. Speller-D-C. TI Thymidine-requiring Staphylococcus aureus [letter]. SO Lancet. 1978 Jan 14. 1(8055). P 104-5. MJ STAPHYLOCOCCUS-AUREUS: gd. THYMIDINE. MN CYSTIC-FIBROSIS: mi. DRUG-RESISTANCE-MICROBIAL. HUMAN. TRIMETHOPRIM: ad. EX We confirm that thymidine-requiring (thy-) Staphylococcus aureus strains are common in patients with cystic fibrosis. In 7 months, we isolated Staph. aureus from 11 of 14 patients with C.F. Despite long-term co-trimoxazole Staph. aureus tends to persist in the sputum even though isolates do not require thymidine at first and are trimethoprim-sensitive in vitro. Staph. aureus is less commonly isolated, and of less significance, in most other chest conditions for which co-trimoxazole is given. These factors may explain why thy- staphylococci are recognised more frequently in C.F. than in other diseases. RF 000 GEORGE RH LANCET 2 1081 977 002 WILLIAMS RF PROC EWGCF 6TH ANNU MTG 4 975 CT 1 WILLIAMS RF CURR MED RES OPIN 6 43 979 2 STURM AW ANT V LEEUW J MICROBIOL SEROL 47 92 981 3 LACEY RW J MED MICROBIOL 15 403 982 4 KING CH J CLIN MICROBIOL 18 79 983 PN 78144 RN 00924 AN 78222230 AU Chase-H-P. Dupont-J. TI Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. SO Lancet. 1978 Jul 29. 2(8083). P 236-8. MJ CYSTIC-FIBROSIS: bl. LINOLEIC-ACIDS: bl. PROSTAGLANDINS-E: bl. PROSTAGLANDINS-F: bl. MN CHILD. CLINICAL-TRIALS. CYSTIC-FIBROSIS: dt, pp. FEMALE. HUMAN. LINOLEIC-ACIDS: pd, tu. MALE. PROSTAGLANDINS-E: bi. PROSTAGLANDINS-F: bi. STIMULATION-CHEMICAL. AB 12 children with cystic fibrosis (C.F.) had lower levels of the essential fatty acid, linoleic acid, in plasma and in red blood cells, than did control children, and production of prostaglandin F2alpha (P.G.F2alpha) was higher than in controls. After 10 months of oral linoleic-acid supplementation in 6 of the children with C.F., the linoleic-acid levels in plasma, red cells, and platelets were higher and P.G.F2alpha production was lower than in the 6 children with C.F. who received a placebo lipid. Prostaglandin F2alpha is associated with bronchoconstriction and its increased production in children with C.F. might be causally related to their chronic pulmonary disease. RF 001 KUO PT J PEDIATR 60 394 962 002 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 003 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 004 RIVERS JPW LANCET 2 642 975 005 HUBBARD VS LANCET 2 1302 977 006 ELLIOTT RB PEDIATRICS 57 474 976 007 SILVER MJ J PROSTAGLANDINS 1 429 972 009 PASARGIKLIAN M IN: SAMUELSSON B 1 461 976 010 NELSON GJ IN: NELSON GJ 10 972 012 MCCOSH FJ J PROSTAGLANDINS 12 471 976 013 DUPONT J FED PROC 37 445 978 014 PACE-ASCIAK C BIOCHIM BIOPHYS ACTA 152 784 968 CT 1 HORROBIN DF MED HYPOTHESES 5 599 979 2 HORROBIN DF MED HYPOTHESES 5 365 979 3 CHASE HP PEDIATRICS 64 207 979 4 CHASE HP J PEDIATR 95 337 979 5 HORROBIN DF MED HYPOTHESES 6 277 980 6 GALEY WR PEDIATR RES 14 1269 980 7 CHASE HP METABOLISM 29 365 980 8 HUBBARD VS J PEDIATR 96 421 980 9 SHEPHERD R J PEDIATR 97 351 980 10 HUBBARD VS CLIN CHIM ACTA 102 115 980 11 ROGIERS V CLIN CHIM ACTA 105 105 980 12 OLLEY PM AM J DIS CHILD 134 688 980 13 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 14 RIVERS JPW BR MED BULL 37 59 981 15 PARK RW GASTROENTEROLOGY 81 1143 981 16 LEMANSKE RF AM REV RESPIR DIS 123 622 981 17 ALLING C EUR J PEDIATR 137 197 981 18 CHASE HP J PEDIATR GASTROENTEROL NUTR 1 49 982 19 LEVI N RIV ITAL PEDIATR 8 695 982 20 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 21 ROGIERS V PEDIATR RES 16 761 982 22 HARPER TB AM REV RESPIR DIS 126 540 982 23 HUBBARD VS EUR J PEDIATR 141 68 983 24 ROGIERS V EUR J PEDIATR 141 39 983 25 HUANG YS PROSTAGLANDINS LEUKOTR MED 15 223 984 26 ROGIERS V PEDIATR RES 18 704 984 27 HORROBIN DF BIOMED BIOCHIM ACTA 43 S114 984 28 MANSELL AL J PEDIATR 104 700 984 29 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 30 HUBBARD VS SEM RESPIR MED 6 308 985 31 FARRELL PM PEDIATR RES 19 104 985 32 CHRISTOPHE A ANN NUTR METAB 29 239 985 33 MAAK B Z KLIN MED 40 1115 985 34 WILSON DO AM REV RESPIR DIS 132 1347 985 35 GALLAND L J AM COLL NUTR 5 213 986 36 LESTER LA J PARENT ENTERAL NUTR 10 289 986 37 MISCHLER EH PEDIATR RES 20 36 986 38 DUHAMEL JF ARCH FR PEDIATR 43 229 986 39 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 40 CARLSTEDTDUKE J PROC NAT ACAD SCI USA 83 9202 986 41 DUPONT J N ENGL J MED 314 718 986 42 HARE WR BIOCHEM SOC TRANS 15 497 987 43 HUBBARD VS LIPIDS 22 424 987 44 STEAD RJ PROSTAGLANDINS LEUKOTR MED 26 91 987 45 YOUNGBERG CA DIG DIS SCI 32 472 987 PN 78145 RN 00925 AN 78155356 AU Lieberman-J. TI Lectins and cystic fibrosis [letter]. SO Lancet. 1978 May 6. 1(8071). P 985-6. MJ CONCANAVALIN-A. CYSTIC-FIBROSIS: pp. MN ANIMAL. CYSTIC-FIBROSIS: et. EXOCRINE-GLANDS: se. HUMAN. MUCUS: se. RATS. EX Dr Freed and Dr Buckley have shown that a lectin, concanavalin A (Con A), causes severe rhinitis, sinusitis, and increased mucus secretion when inhaled by man or small-intestinal colic, foul-smelling flatus, and mucus-coated stools if ingested. It is somewhat surprising that the authors suggest that a suitable inhaled or ingested lectin may be useful in treating patients with cystic fibrosis, since the very symptoms produced by the ingested lectin resemble those associated with the disease. A more attractive recommendation would be for patients with cystic fibrosis to avoid substances containing lectins such as Con A to avoid stimulating additional mucus release. The underlying pathogenetic defect in cystic fibrosis may be inappropriate, excessive stimuli by lectin-like substances. These observations suggest that the increase in the protein content of lymphocytes is stimulated by a plasma factor present in patients with cystic fibrosis and in carriers of the cystic fibrosis gene. These observations, plus those of Freed and Buckley, might suggest that cystic fibrosis is a disease involving a plasma factor with lectin or Con A-like activity which stimulates the secretion of mucus and increases the enzyme content of circulating or cultured lymphocytes. - Professor Lieberman confuses cause with effect. If there is excessive mucus production in cystic fibrosis we suggest that this is because the secretion is so difficult to remove from the mucosal surface. Goblet cells and mucus glands become clogged with hyperviscid mucus which, not being removed, stimulates further mucus production. To deprive the patient of the mucotractive value of his dietary lectins would be a therapeutic mischief equivalent to prescribing a cough-suppresant for a productive cough. RF 000 FREED DLJ LANCET 1 585 978 001 MARCHI AG HELV PAEDIATR ACTA 28 427 973 002 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 CT 1 LIEBERMAN J J LAB CLIN MED 97 646 981 PN 78146 RN 00926 AN 79052016 TI Cystic-fibrosis variants--or variations? [editorial]. SO Lancet. 1978 Nov 11. 2(8098). P 1032-3. MJ CYSTIC-FIBROSIS: di. MN BRONCHITIS: di. CHLORIDES: an. CHRONIC-DISEASE. DIAGNOSIS-DIFFERENTIAL. HUMAN. PANCREAS: pp. PSEUDOMONAS-INFECTIONS: di. STAPHYLOCOCCAL-INFECTIONS: di. SWEAT: an. EX Cystic fibrosis is inherited by single autosomal transmission. That, at least, is the general view; but from time to time other suggestions are offered. Stern et al. describe what they believe may be a genetic variant. The affected patients, they suggest, are homozygous for a portion of a closely linked multigenic cystic fibrosis locus, or have modifier genes ameliorating the pancreatic and sweat lesions. Do these patients really have CF? And, if so, are Stern et al. justified in suggesting that they differ genetically from the "classic" CF patient? This hypothesis may not be correct but, until we understand the basic fundamental defect, it may be unwise to divide CF into different genetic types on the basis of clinical variations. RF 001 DANKS DM ANN HUM GENET 28 323 965 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 DANES BS J EXP MED 129 775 969 004 STERN RC JAMA 239 2676 978 005 HADORN B CAN MED ASSOC J 98 377 968 006 ZOPPI G ACTA PAEDIATR SCAND 59 692 970 007 ANON J PEDIATR 88 711 976 008 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 009 SCHWARZ V ARCH DIS CHILD 52 870 977 010 SMALLEY CA LANCET 2 415 978 011 MANGOS JA CF PROJECTIONS INTO THE FUTUR 976 012 HIRSCHHORN K IN: MANGOS JA 305 976 013 CONOVER JH LIFE SCI 14 253 974 CT 1 DAVIS PB AM J MED 69 643 980 2 TARNOKY AL J ROY SOC MED 73 73 980 PN 78147 RN 00927 AN 79052040 AU Kibel-M-A. TI Sweat tests in cystic fibrosis [letter]. SO Lancet. 1978 Nov 11. 2(8098). P 1050. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHILD. CHLORIDES: an. ECZEMA: di. FALSE-POSITIVE-REACTIONS. HUMAN. SODIUM: an. EX Dr Smalley and her colleagues rightly stress that the commonest reason for abnormally high sweat-electrolyte levels, apart from cystic fibrosis, is faulty technique. A further cause of false-positive results, and potentially more important than the list of rare disorders usually cited, is atopic dermatitis. Dr M. Ampola and I investigated ten children with eczema at the Tufts New England Medical Center, Boston, and found higher sodium and/or chloride levels in sweat obtained from affected skin than in sweat from normal skin in the same individuals. In four of these atopic children, levels within the diagnostic range for cystic fibrosis were obtained (above 60 mmol/l). RF 000 SMALLEY CA LANCET 2 415 978 001 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 002 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 003 ANON J PEDIATR 88 711 976 CT 1 DAVID TJ LANCET 2 1204 982 2 RUDDY RM CLIN PEDIATR 26 83 987 PN 78148 RN 00928 AN 79031281 AU Galabert-C. Filliat-M. Chazalette-J-P. TI Fatty-acid composition of serum-lecithins in cystic-fibrosis patients without steatorrhoea [letter]. SO Lancet. 1978 Oct 21. 2(8095). P 903. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-UNSATURATED: an. PHOSPHATIDYLCHOLINES: bl. MN HUMAN. PANCREAS: me. EX We have investigated the fatty-acid composition of serum-lecithins from four groups of C.F. patients, defined by their pancreatic function evaluated by 72-h quantitative stool fat and determination of the coefficient of fat absorption. C.F. patients without pancreatic insufficiency and not on pancreatic extract had significantly higher percentages of linoleic acid than did patients of any other group. The altered fatty-acid composition of serum-lipid fractions seems to be a secondary consequence of the pancreatic insufficiency commonly associated with C.F. disease and not a direct metabolic defect as some have suggested. RF 001 HUBBARD VS LANCET 2 1302 977 002 KUO PT J PEDIATR 60 394 962 003 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 004 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 005 ELLIOTT RB ARCH DIS CHILD 50 76 975 CT 1 ROGIERS V CLIN CHIM ACTA 105 105 980 2 ROGIERS V PEDIATR RES 16 761 982 3 HARPER TB AM REV RESPIR DIS 126 540 982 4 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 5 HUBBARD VS EUR J PEDIATR 141 68 983 6 ROGIERS V EUR J PEDIATR 141 39 983 7 ROGIERS V PEDIATR RES 18 704 984 8 FARRELL PM PEDIATR RES 19 104 985 9 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 10 STEAD RJ PROSTAGLANDINS LEUKOTR MED 26 91 987 PN 78149 RN 00929 AN 79071612 AU Ellam-S-V. TI Improvement on the safety of sweat-test equipment. SO Med-Biol-Eng-Comput. 1978 Sep. 16(5). P 595-6. MJ ACCIDENT-PREVENTION. IONTOPHORESIS: is. SAFETY. SWEAT: an. MN BURNS-ELECTRIC: pc. CYSTIC-FIBROSIS: me. HUMAN. EX Patients with cystic fibrosis have a higher concentration of sodium and chloride in their sweat than normals. This observation led to the introduction of an iontophoresis technique for stimulating the sweat glands with pilocarpine. Occasionally, electrical burns result from defective equipment or from an incorrect or badly applied test procedure. A new design of equipment is presented which eliminates the possibility of a burn during the test. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 GIBSON LE PEDIATRICS 23 545 959 003 SCHWARZ V ARCH DIS CHILD 43 695 968 PN 78150 RN 00930 AN 79052693 AU Elliott-R-B. TI Cystic fibrosis screening in the newborn [editorial]. SO Med-J-Aust. 1978 Jul 29. 2(3). P 95. MJ CYSTIC-FIBROSIS: pc. MASS-SCREENING: mt. MN HUMAN. INFANT-NEWBORN. EX Many attempts have been made to establish a suitable screening test for cystic fibrosis for newborns. Recently, a new method has been described by Crossley and her associates. It is based on measuring pancreatic trypsin in a sample of faeces collected on to filter paper on the 4th or 5th day of life. The false-negative rate of the new test has not yet been determined, but older children with CF and pancreatic achylia all gave abnormal results when tested. The disadvantage of the test, as with the meconium albumin test, is that about 10% of children with CF are said not to have pancreatic achylia. Whilst the method needs more extensive evaluation, it appears technically more satisfactory than others so far described. RF 001 ELLIOTT RB REC ADELAIDE CHILD HOSP 1 359 977 002 PROSSER R ARCH DIS CHILD 49 597 974 003 CROSSLEY JR LANCET 2 1093 977 PN 78151 RN 00931 AN 79072550 AU Moses-R-G. Bowen-T. Edwards-R. TI Cystic fibrosis and fast moving haemoglobin [letter]. SO Med-J-Aust. 1978 Aug 12. 2(4). P 160-1. MJ CYSTIC-FIBROSIS: bl. HEMOGLOBINS: an. MN CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. EX We have measured fast moving components of haemoglobin (FMH) in nine children with cystic fibrosis without any clinical evidence of diabetes mellitus. The nine patients with cystic fibrosis had a mean FMH of 9.3 which differed significantly from 97 controls with a mean FMH of 7.6. There was no correlation between FMH and age or FMH and duration of disease. The above results raise the question as to what extent the FMH is a reflection of hyperglycaemia or insulinopenia. RF 001 PAULSEN EP DIABETES 25 890 976 002 KOENIG RJ N ENGL J MED 295 417 976 003 MILNER AD ARCH DIS CHILD 44 351 969 PN 78152 RN 00932 AN 78113434 AU Holzel-A. TI Malabsorption. SO Midwife-Health-Visit-Community-Nurse. 1978 Mar. 14(3). P 79-81. MJ MALABSORPTION-SYNDROMES: pp. MN CELIAC-DISEASE: pp. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. DIETARY-CARBOHYDRATES. HUMAN. INFANT. LACTOSE-INTOLERANCE: pp. SUCROSE. EX Malabsorption in infancy and childhood can roughly be defined as a condition of inadequate growth in spite of seemingly adequate nutrition and is accompanied by excessive excretion of food components in the stools. Acquired lactase deficiency, the introduction of sugar in the diet, coeliac disease, and cystic fibrosis are discussed. PN 78153 RN 00933 AN 79093734 AU Watts-R-W. TI Recent advances in screening for inborn errors of metabolism. SO Monogr-Hum-Genet. 1978. 9. P 204-11. MJ METABOLISM-INBORN-ERRORS: di. MN ANEMIA-SICKLE-CELL: oc. CRETINISM: oc. CYSTIC-FIBROSIS: oc. HETEROZYGOTE-DETECTION. HUMAN. METABOLISM-INBORN-ERRORS: fg. PHENYLKETONURIA: oc. TAY-SACHS-DISEASE: oc. EX A screening test is any diagnostic procedure which we apply without a specific clinical indication from the point of view of the individual patient concerned. Non-selective (whole population) screening for inborn errors of metabolism, selective screening for inborn errors of metabolism, screening for nonamino organic acidurias, and selective screening for inborn errors of metabolism because of genetic indications are discussed. The main points which emerge for the future from this review are the need to develop multidisease screening programmes to detect the very rare diseases; that postnatal, prenatal and carrier screening should be considered and developed collectively; and there us an urgent needs to develop screening for thallasaemia, sickle cell disease, cystic fibrosis and congenital hypothyroidism. RF 001 BROWN E METABOLISM 26 1047 977 002 CHALMERS RA CLIN CHEM 22 1292 976 003 CHALMERS RA CLIN CHEM 22 1288 976 004 CHALMERS RA ANN CLIN BIOCHEM 14 149 977 005 CHILDS B AM J HUM GENET 28 537 976 006 CHILDS B AM J HUM GENET 28 550 976 007 EVANS PR IN: RAINE DN 93 977 008 FISHER DA J PEDIATR 89 692 976 009 GIBBS DA ANN CLIN BIOCHEM 14 157 977 011 KABACK MM HUM HERED 27 186 977 012 KABACK MM PROG MED GENET 10 103 974 013 KAN YW LANCET 1 269 977 014 KOMROWER GM PEDIATRICS 53 182 974 015 LAWSON AM CLIN CHEM 22 1283 976 016 NATHAN DG BR J HAEMATOL SUPPL 31 143 975 017 PADEH B HUM HERED 27 200 977 018 PURKISS P PROC INT SYMP GLYCOCON 3RD 19 975 019 SEEGMILLER JE IN: NATELSON S 291 974 020 WATTS RWE LANCET 1 368 975 CT 1 HAIDER M CLIN BIOCHEM CONTEM THEO TECH 2 145 982 PN 78154 RN 00934 AN 79032102 AU Gordeuk-V. Keeports-R. Rexrode-W. TI Cystic fibrosis in adults [letter]. SO N-Engl-J-Med. 1978 Nov 16. 299(20). P 1137. MJ CYSTIC-FIBROSIS: oc. MN ADULT. AGE-FACTORS. CASE-REPORT. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. EX Long considered a childhood disease, cystic fibrosis has been recognized with increasing frequency in adults. In the case reported below the diagnosis was made in an adult. The initial diagnosis of cystic fibrosis in patients over 30 years of age has rarely been reported. Our patient, in whom the diagnosis of cystic fibrosis was first made at 48 years if age, serves to emphasize the point that physicians should consider cystic fibrosis in the differential diagnosis of adults with unexplained chronic obstructive lung disease. RF 001 WARWICK WJ JAMA 238 2159 977 002 SHWACHMAN H N ENGL J MED 296 1519 977 003 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 CT 1 DAVIDSON AGF N ENGL J MED 300 1164 979 PN 78155 RN 00935 AN 79053053 AU Bennion-L-J. Grundy-S-M. TI Risk factors for the development of cholelithiasis in man (second of two parts). SO N-Engl-J-Med. 1978 Nov 30. 299(22). P 1221-7. (REVIEW). MJ CHOLELITHIASIS: et. MN ADOLESCENCE. ADULT. AGE-FACTORS. ANEMIA-HEMOLYTIC: co. BILE: mi. CHOLELITHIASIS: ci, fg, oc. CLOFIBRATE: ae. CONTRACEPTIVES-ORAL: ae. CYSTIC-FIBROSIS: co. DIET: ae. ESTROGENS: ae. FEMALE. GASTROINTESTINAL-DISEASES: co. HUMAN. INFECTION: co. LIVER-CIRRHOSIS-ALCOHOLIC: co. MALE. OBESITY: co. PREGNANCY. REVIEW. RISK. EX The demographic characteristics of persons at high risk for pigment gallstones differ from those is whom cholesterol stones are likely to develop. Conditions associated with pigment gallstones include hemolytic anemia, alcoholic cirrhosis, and infected bile. Conditions associated with cholesterol gallstones include obesity, diet, blood-cholesterol-lowering drugs, disorders of the gastrointestinal tract, female sex hormones, and genetic and ethnic influences. Problem areas such as case finding and common disorders and pregnancy are discussed, along with clinical implications (diagnosis, prevention, and therapy). RF 049 SAMPLINER RE N ENGL J MED 283 1358 970 050 SIEVERS ML JAMA 182 570 962 051 THISTLE JL MAYO CLIN PROC 46 603 971 052 SUTOR DJ GUT 12 55 971 053 GROSS DMB J PATHOL BACTERIOL 32 503 929 054 TROTMAN BW AM J DIG DIS 20 735 975 055 MARINOVIC J REV MED CHIL 100 1320 972 056 MIYAKE H DIGESTION 1 219 968 057 YAGI T TOHOKU J EXP MED 72 117 960 058 NAKAYAMA F AM J SURG 120 794 970 059 KAMEDA H GASTROENTEROLOGY 46 109 964 060 MATSUSHIRO T GASTROENTEROLOGY 72 630 977 061 GLENN F ANN SURG 153 411 961 062 CAMERON JL ANN SURG 174 702 971 063 MERENDINO KA ANN SURG 177 694 973 064 TAMALET E J MED FR 21 159 932 065 JORDAN RA GASTROENTEROLOGY 33 952 957 066 DEWEY KW RADIOLOGY 96 385 970 067 BATES GC GASTROENTEROLOGY 21 104 952 068 BOUCHIER IAD GUT 10 705 969 069 NICHOLAS P GASTROENTEROLOGY 63 112 972 070 VAN DER LINDEN W ACTA CHIR SCAND SUPPL 269 1 961 071 FRIEDMAN GD J CHRON DIS 19 273 966 072 STURDEVANT RAL N ENGL J MED 288 24 973 073 MABEE TM SURGERY 79 460 976 074 SARLES H SEM HOP PARIS 33 3424 957 075 SARLES H AM J DIG DIS 14 531 969 076 SARLES H AM J DIG DIS 15 251 970 077 SARLES H SCAND J GASTROENTEROL 6 189 971 078 SCHREIBMAN PH AM SOC CLIN INVE 66TH ANN MTG 72A 974 079 QUINTAO E J LIPID RES 12 233 971 080 SARLES H SCAND J GASTROENTEROL 5 603 970 081 DAM H Z ERNAEHRUNGSWISS 10 178 971 082 DENBESTEN L SURGERY 73 266 973 083 MIETTINEN M ANN CLIN RES 8 111 976 084 DAM H SCAND J CLIN LAB INVEST 19 367 967 085 WATANABE N ARCH SURG 85 136 962 086 GRUNDY SM J CLIN INVEST 55 269 975 087 HEATON KW CLIN GASTROENTEROL 2 67 973 088 BURKITT DP BR MED J 1 274 973 089 POMARE EW AM J DIG DIS 21 521 976 091 RAYMOND TL J CLIN INVEST 60 1429 977 092 ANON N ENGL J MED 296 1185 977 093 COOPER J LANCET 1 1083 975 094 PERTSEMLIDIS D GASTROENTEROLOGY 66 565 974 095 THISTLE JL GASTROENTEROLOGY 61 488 971 096 HORLICK L CIRCULATION 43 299 971 097 GRUNDY SM J LIPID RES 13 531 972 098 WOOD PD METABOLISM 21 107 972 099 DAM H Z ERNAEHRUNGSWISS 10 188 971 100 MEIHOFF WE J CLIN INVEST 47 261 968 101 HEATON KW GASTROENTEROLOGY 55 5 968 102 DOWLING RH GUT 13 415 972 103 COHEN S GASTROENTEROLOGY 60 237 971 104 BAKER AL AM J DIG DIS 19 109 975 105 MARKS JW AM J DIG DIS 22 1097 977 106 HEATON KW BR MED J 3 494 969 107 PELLERIN D J PEDIATR SURG 10 35 975 108 ROVSING H ACTA RADIOL DIAGN STOCKH 14 588 973 109 ROY CC N ENGL J MED 297 1301 977 110 WEBER AM N ENGL J MED 289 1001 973 111 GOODCHILD MC ARCH DIS CHILD 50 769 975 112 WATKINS JB GASTROENTEROLOGY 67 835 974 113 ANON LANCET 1 1399 973 114 ANON ORAL CONTRACEPTIVES AND HEALT 57 974 115 STOLLEY PD AM J EPIDEMIOL 102 197 975 116 ANON N ENGL J MED 290 15 974 117 BENNION LJ N ENGL J MED 294 189 976 118 TRITAPEPE R N ENGL J MED 295 961 976 119 ANON FDA DRUG BULL 8 12 978 120 NILSSON S ACTA CHIR SCAND 132 275 966 121 HORN G BR MED J 2 732 956 122 BENNION LJ METABOLISM 27 961 978 123 BENNION LJ CLIN RES 26 496A 978 124 BENNION LJ N ENGL J MED 297 709 977 125 NILSSON S ACTA CHIR SCAND 133 648 967 126 WILCOX LL CLIN RES 20 223 972 127 ZAHOR Z SCAND J GASTROENTEROL 9 3 974 128 BILL K N ENGL J MED 284 694 971 129 REDINGER RN ARCH INTERN MED 130 618 972 130 THISTLE JL N ENGL J MED 284 144 971 131 JACKSON CD SURGERY 46 853 959 132 VAN DER LINDEN W ACTA GENET STATIST MED 15 159 965 133 DANZINGER RG MAYO CLIN PROC 47 762 972 134 BAINTON D N ENGL J MED 294 1147 976 135 WILLIAMS CN CAN MED ASSOC J 117 758 977 136 BENNION LJ N ENGL J MED 296 1365 977 137 KAYE MD LANCET 1 1228 971 138 RIEGEL C JAMA 105 1343 935 139 POTTER MG JAMA 106 1070 937 140 LARGE AM AM J MED SCI 239 713 960 CT 1 BARKER DJP BR MED J 2 1389 979 2 GRUNDY SM EUR J CLIN INVEST 9 179 979 3 AYUB A SURG CLIN NORTH AM 59 1095 979 4 REDINGER RN POSTGRAD MED 65 56 979 5 ANDERSEN JM GASTROENTEROLOGY 77 1146 979 6 INGELFINGER FJ N ENGL J MED 300 917 979 7 PADOVA CD METH FIND EXP CLIN PHARMACOL 2 177 980 8 CAPRON JP GASTROENTEROL CLIN BIOL 4 63 980 9 DEVORE GR CLIN PERINATOL 7 349 980 10 HUIJBREGTS AWM EUR J CLIN INVEST 10 443 980 11 DIPADOVA C EUR SURG RES 12 57 980 12 ANGELIN B SCAND J GASTROENTEROL 15 849 980 13 HONORE LH J CLIN PHARMACOL 20 338 980 14 DEMARQUEZ JL AGRESSOLOGIE 21 137 980 15 PETERS H INTERNIST 21 559 980 16 CADIER L ANN RADIOL (PARIS) 23 428 980 17 SHAFFER EA CAN J SURG 23 517 980 18 HONORE LH J REPROD MED 25 187 980 19 HONORE LH J CHRON DIS 33 465 980 20 SCHLIERF G ATHEROSCLEROSIS 36 323 980 21 DIEHL AK J NATL CANCER INST 65 1209 980 22 MCMICHAEL AJ J NATL CANCER INST 65 1201 980 23 DIEHL AK SOUTH MED J 73 438 980 24 AHLBERG J GASTROENTEROLOGY 79 90 980 25 MOCK DM J PEDIATR 96 878 980 26 HONORE LH ARCH SURG 115 62 980 27 PAUMGARTNER G MUNCH MED WOCHENSCHR 122 1223 980 28 PAUMGARTNER G MUNCH MED WOCHENSCHR 122 1001 980 29 WILLIAMS CN CAN MED ASSOC J 122 664 980 30 NIESSEN KH MONATSSCHR KINDERHEILKD 128 551 980 31 SCHWEIGHOFER S AM J DIS CHILD 134 622 980 32 BRAVERMAN DZ N ENGL J MED 302 362 980 33 COHEN S N ENGL J MED 302 397 980 34 DIEHL AK LANCET 2 187 981 35 DIPADOVA C METH FIND EXP CLIN PHARMACOL 3 155 981 36 ANGELIN B SCAND J GASTROENTEROL 16 1015 981 37 SCHLIERF G DIGESTION 21 44 981 38 AHLBERG J J LIPID RES 22 404 981 39 STEVEN MM GUT 22 592 981 40 CAPRON JP DIG DIS SCI 26 523 981 41 DIPADOVA C DIG DIS SCI 26 1095 981 42 SOUSTEK Z ACTA MORPHOL ACAD SCI HUNG 29 127 981 43 RUDERMAN NB AM J CLIN NUTR 34 1617 981 44 PIMSTONE NR SURG CLIN NORTH AM 61 865 981 45 KLATSKY AL CIRCULATION 64 32 981 46 MOK HYI GASTROENTEROLOGY 81 340 981 47 LEE SP SCIENCE 211 1429 981 48 TURLEY SD J BIOL CHEM 256 2438 981 49 RUDERMAN NB INT J OBES 6 151 982 50 TUCKER LE INT J OBES 6 247 982 51 CHILDS B AM J MED GENET 13 319 982 52 ZSIGMOND G EUR SURG RES 14 344 982 53 LEISS O SCAND J GASTROENTEROL 17 587 982 54 HENEGOUWEN GPV NETH J MED 25 89 982 55 USCANGA L REV INVEST CLIN 34 341 982 56 TAKEUCHI N ATHEROSCLEROSIS 42 129 982 57 PODDA M ARCH DIS CHILD 57 956 982 58 BRAUN B KLIN WSCHR 60 1357 982 59 EVERSON GT GASTROENTEROLOGY 82 711 982 60 HEUBI JE GASTROENTEROLOGY 82 1295 982 61 LOWENFELS AB GASTROENTEROLOGY 83 672 982 62 KRISHNAMURTHY GT WEST J MED 137 87 982 63 SHUMAN WP AM J ROENTGENOL 138 1 982 64 KORENMAN SG ARCH INTERN MED 142 1131 982 65 SASTIC JW SURG GYNECOL OBSTET 155 209 982 66 KERN F SEM LIVER DIS 3 87 983 67 ZAK RA SEM LIVER DIS 3 132 983 68 BOUCHIER IAD CLIN GASTROENTEROL 12 25 983 69 DOWLING RH CLIN GASTROENTEROL 12 125 983 70 BORGNAPIGNATTI C ITAL J GASTROENTEROL 15 228 983 71 KESANIEMI YA SCAND J GASTROENTEROL 18 897 983 72 WHITING MJ GUT 24 11 983 73 LEISS O KLIN WSCHR 61 579 983 74 GILAT T GASTROENTEROLOGY 84 242 983 75 MESSING B GASTROENTEROLOGY 84 1012 983 76 TURLEY SD GASTROENTEROLOGY 84 253 983 77 LEISS O FETTE SEIFEN ANSTRICHM 85 447 983 78 MEDINA E REV MED CHIL 111 668 983 79 MCMICHAEL AJ AM J EPIDEMIOL 118 620 983 80 ADYE B WEST J MED 139 471 983 81 BOUCHIER IAD BR MED J 286 415 983 82 DOTY JE J PARENT ENTERAL NUTR 8 263 984 83 WHITING MJ INT J OBES 8 681 984 84 ANGELIN B EUR J CLIN INVEST 14 73 984 85 FAED EM DRUG METAB REV 15 1213 984 86 DIPADOVA C SCAND J GASTROENTEROL 19 820 984 87 BOUCHIER JAD GUT 25 1021 984 88 WERNER D GUT 25 269 984 89 ACALOVSCHI M ACTA GASTROENTEROL BELG 47 381 984 90 ADAMI HO BR J CANCER 49 235 984 91 DEDERER YM KLIN MED 62 14 984 92 ADAMTHWAITE DN S AFR MED J 65 572 984 93 WHITING MJ GASTROENTEROLOGY 86 243 984 94 TURLEY SD GASTROENTEROLOGY 87 284 984 95 FRIED GM SURGERY 95 284 984 96 VOGELBERG KH DTSCH MED WSCHR 109 1712 984 97 MULLIGAN SC IR J MED SCI 153 106 984 98 SCRAGG RKR BR MED J 288 1113 984 99 RHOMBERG HP BR MED J 289 1002 984 100 MORRISON WB VET PATHOL 22 285 985 101 BOLONDI L GUT 26 734 985 102 SPITZ MR J SURG ONCOL 30 6 985 103 DIEHL AK J CHRON DIS 38 1019 985 104 LEISS O KLIN WSCHR 63 1163 985 105 NILSELL K GASTROENTEROLOGY 89 287 985 106 BORTNICHAK EA AM J EPIDEMIOL 121 19 985 107 PIXLEY F BR MED J 291 11 985 108 EINARSSON K N ENGL J MED 313 277 985 109 ABATE MA DRUG INTEL CLIN PHARM 20 106 986 110 DIDONATO P GUT 27 23 986 111 TURLEY SD J LIPID RES 27 486 986 112 OKOLICSANYI L DRUGS 31 430 986 113 MARMON L POSTGRAD MED 79 181 986 114 SANDLER RS GASTROENTEROLOGY 91 157 986 115 WYSOWSKI DK AM J EPIDEMIOL 123 532 986 116 KEANE P SURG GYNECOL OBSTET 163 555 986 117 TURLEY SD BIOCHIM BIOPHYS ACTA 879 28 986 118 WINSHIP KA ADVER DRUG REAC ACUT POIS REV 6 37 987 119 HEATON KW MOL ASP MED 9 89 987 120 LEE SS J CLIN GASTROENTEROL 9 65 987 121 PITT HA AM J SURG 153 233 987 122 SCHULMAN A RADIOLOGY 162 425 987 PN 78156 RN 00936 AN 78247032 AU Romeo-G. Parsons-M. Bossen-A. Blessing-Moore-J. Cavalli-Sforza-L. TI Trypsin-binding IgG in cystic fibrosis. SO Nature. 1978 Aug 31. 274(5674). P 909-11. MJ ANTIBODIES: an. CYSTIC-FIBROSIS: im. TRYPSIN: im. MN CYSTIC-FIBROSIS: th. HUMAN. IGG. IMMUNOGLOBULINS-FAB. PANCREATIC-EXTRACTS: im, tu. SUPPORT-U-S-GOVT-P-H-S. EX It has been observed that most cystic fibrosis patients lack an alpha2 macroglobulin-protease complex and that binding of trypsin and other proteases to alpha2 macroglobulin is lower in plasma of CF patients than in that of normal controls. We therefore decided to study the interaction of trypsin with plasma proteins of CF patients. Here we report that trypsin-binding IgG (TbIgG) is present in 80% of CF patients and in 30% of their mothers, but not in 97% of normal controls or in other relatives of CF patients. Experimental evidence supports the antibody nature of TbIgG, which is presumably produced against the porcine trypsin ingested by CF patients as pancreatic extracts for substitutive enzyme therapy. RF 001 WILSON GB PEDIATR RES 10 87 976 002 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 003 HUNTER WM NATURE 194 495 962 004 DAIGER S PROC NAT ACAD SCI USA 72 2076 975 005 STANWORTH DR IN: WEIR DM 10 1 973 006 KOPITO LE PEDIATR RES 10 742 976 007 TWAROG FJ J ALLERGY CLIN IMMUNOL 59 35 977 008 DOLAN TF JR AM REV RESPIR DIS 110 812 974 010 GENELL S HOPPE SEYLERS Z PHYSIOL CHEM 358 115 977 011 LIEBOW C SCIENCE 189 472 975 012 ELIAS E LANCET 2 66 977 013 AARON R IMMUNOCHEMISTRY 3 451 966 014 ERICKSON RP IMMUNOCHEMISTRY 11 41 974 015 RAO GJS SCIENCE 177 610 972 016 RAO GJS PEDIATR RES 8 684 974 017 CHAN KYH CLIN CHIM ACTA 74 71 977 018 TALAMO RC PEDIATR RES 6 430 972 019 LIEBERMAN J AM REV RESPIR DIS 109 399 974 020 GOLDSMITH GH J LAB CLIN MED 89 131 977 021 ROMEO G AM J HUM GENET 29 92A 977 CT 1 VANLEUVEN F PEDIATR RES 13 1384 979 2 DAVIS PB PEDIATR RES 14 83 980 3 DENARO M PEDIATR RES 14 1086 980 4 TARNOKY AL J ROY SOC MED 73 73 980 5 ROMEO G J LAB CLIN MED 95 116 980 6 PARSONS M CLIN CHIM ACTA 100 215 980 7 MOSS RB AM REV RESPIR DIS 121 23 980 8 SCHIDLOW DV AM REV RESPIR DIS 121 31 980 9 BLITZER MG J PEDIATR GASTROENTEROL NUTR 1 289 982 10 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 11 DURIE PR J PEDIATR GASTROENTEROL NUTR 1 337 982 12 ROBERTS RC PEDIATR RES 16 416 982 13 MOSS RB CLIN EXP IMMUNOL 47 301 982 14 BRIDGES MA ANN NY ACAD SCI 421 360 983 15 LAROCHE D J PEDIATR GASTROENTEROL NUTR 5 404 986 16 MOORE DJ CLIN BIOCHEM 19 303 986 17 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 18 BLANDIN C J BIOPHYS BIOMECAN 11 5 987 PN 78157 RN 00937 AN 78248435 AU Phillips-B-M. TI Cystic fibrosis: the challenge in research and management. SO Nurs-Mirror. 1978 Aug 24. 147(8). P 13-5. MJ CYSTIC-FIBROSIS: nu. MN ADOLESCENCE. CHILD. CYSTIC-FIBROSIS: co, px. HUMAN. RESEARCH. EX Cystic fibrosis is a common and serious disease, and presents a challenge in management to the paediatrician, nurse, dietitian, physiotherapist, and all other members of the caring team. The outstanding research challenge is to discover the basic defect, and if this is not treatable to find a method of identifying carriers and affected infants in utero. In this task, the work of the Cystic Fibrosis Research Trust, both in raising money for research and for supporting and advising patients, is much appreciated. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 SPOCK A PEDIATR RES 1 173 967 003 WOLFSDORF J PEDIATRICS 43 799 969 004 PARKS CR AM REV RESPIR DIS 108 513 973 PN 78158 RN 00938 AN 78248436 AU Duberley-J-D. King-H. TI Cystic fibrosis: the nurse-teacher and carer for mother and child. SO Nurs-Mirror. 1978 Aug 24. 147(8). P 15-8. MJ CYSTIC-FIBROSIS: nu. PARENT-CHILD-RELATIONS. PATIENTS: ed. PATIENT-EDUCATION. MN CHILD. HOME-NURSING. HUMAN. INTERPERSONAL-RELATIONS. NURSE-PATIENT-RELATIONS. TERMINAL-CARE. EX The authors explain the importance of nursing care being directed to all members of the family involved in the care of the cystic fibrosis child and, in particular, the mother. RF 001 BIVALEC LM NURS CLIN NORTH AM 2 109 976 002 BURTON L FAMILY LIFE OF SICK CHILDREN 975 003 HARRISSON SP FAMILIES IN STRESS 977 004 TURK J PEDIATRICS 34 67 964 005 QUINT JC NURSE AND THE DYING PATIE 967 PN 78159 RN 00939 AN 78248437 AU Mallinson-B-M. TI Cystic fibrosis: 7 rules for physiotherapy. SO Nurs-Mirror. 1978 Aug 24. 147(8). P 18-9. MJ CYSTIC-FIBROSIS: nu. PHYSICAL-THERAPY. MN CHILD. HUMAN. INFANT. POSTURE. EX The author explains why treatment, although time-consuming, must be carried out every day. PN 78160 RN 00940 AN 78248433 TI A positive approach to cystic fibrosis [editorial]. SO Nurs-Mirror. 1978 Aug 24. 147(8). P 1. MJ CYSTIC-FIBROSIS: nu. MN CHILD. FAMILY. HOME-NURSING. HUMAN. EX The child with cystic fibrosis presents a heart-breaking problem to parents, since the disease is inherited equally from both mother and father, and there is the possibility that other children will be similarly afflicted. Perhaps the most valuable advice the nurse can give to parents is to adopt at all times a positive approach to their problem. PN 78161 RN 00941 AN 78136652 AU Russell-G. TI Cystic fibrosis--1. Clinical aspects. SO Nurs-Times. 1978 Mar 23. 74(12). P 486-9. MJ CYSTIC-FIBROSIS: di. MN CYSTIC-FIBROSIS: th. HUMAN. INFANT. PROGNOSIS. EX Cystic fibrosis is a fatal inherited disease involving many different organs. At one time most affected children died within a few years of birth but modern management has led to a marked improvement in prognosis and, of course, to an increase in the number of affected older children and young adults. Cystic fibrosis is, therefore, no longer a disease encountered only in paediatric units, but with increasing survival into teens and twenties affected patients are now appearing in adult units. The aetiology and pathology, the clinical picture, diagnosis, treatment, and prognosis are discussed. PN 78162 RN 00942 AN 78115481 AU Russell-G. TI Cystic fibrosis--2: genetic and social aspects. SO Nurs-Times. 1978 Mar 30. 74(13). P 538-41. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: di. FEMALE. GENETIC-COUNSELING. HUMAN. INFANT-NEWBORN. PARENTS: ed. PREGNANCY. PRENATAL-DIAGNOSIS. PROGNOSIS. RESEARCH. EX The inheritance of cystic fibrosis and its impact on the patient and on the community are examined. Inheritance, genetic counseling, antenatal diagnosis, screening for cystic fibrosis, psychosocial aspects, employment prospects, and the Cystic Fibrosis Research Trust are discussed. RF 001 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 002 BURTON L PRACTITIONER 210 247 973 003 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 004 GIBBS GE PAEDIATRICIAN 1 133 972 005 MCCRAE WM IN: APLEY J 4 157 974 006 SHWACHMAN H PEDIATRICS 46 335 970 PN 78163 RN 00943 AN 79115400 AU Lavy-U. Bauer-C-H. TI Pathophysiology of failure to thrive in gastrointestinal disorders. SO Pediatr-Ann. 1978 Nov. 7(11). P 743-9. MJ GASTROINTESTINAL-DISEASES: co. GROWTH-DISORDERS: et. MN ADOLESCENCE. CELIAC-DISEASE: co. CHILD. COLITIS-ULCERATIVE: co. CYSTIC-FIBROSIS: co. ENTERITIS: co. ESOPHAGEAL-DISEASES: co. ESOPHAGUS: ab. HUMAN. INFANT. INFANT-NEWBORN. LIVER-DISEASES: co. MALABSORPTION-SYNDROMES: co. MEGACOLON: co. MOUTH-ABNORMALITIES: co. SYNDROME. AB What will be our GI approach to a child with FTT syndrome? Detailed history and physical examination will give us the clue and often the probable diagnosis. Several laboratory tests are helpful in establishing the fact that there is malabsorption. Among them are a complete blood count with smear, quantitative stool fat excretion, serum protein and chemistry screen panel, prothrombin time, and oral tolerance and absorption--i.e., of glucose, iron, vitamin A, and xylose. Specialized procedures may be used to nail down the diagnosis: radiology, biopsy, duodenal intubation, etc. These should never be employed as routine screening tests, however. In outlining a comprehensive and successful therapy, the attending physician will find it helpful to consider the particular pathophysiologic mechanisms of a specific disease. Exact diagnosis makes the therapy both rational and effective. RF 001 ANDERSON CM IN: ANDERSON CM 975 002 BEEKEN WL PEDIATRICS 52 69 973 003 CARRE IJ CLIN PROC CHILD HOSP DC 25 123 969 004 CASTLEMAN B N ENGL J MED 289 147 973 005 DAVIDSON M J PEDIATR 69 1027 966 006 DAVIDSON M IN: BARNETT HL 972 007 DAVIDSON M IN: SLEISENGER MH 973 008 KATTWINKEL J PEDIATRICS 51 55 973 009 OLSEN WA N ENGL J MED 285 1358 971 010 PRINSEN JE J PEDIATR SURG 10 97 975 011 SHWACHMAN H PEDIATRICS 46 335 970 012 SILVERBERG M IN: BARNETT HL 972 013 SILVERBERG M PEDIATR ANN 6 977 014 SMITH DW RECOGNIZABLE PATTERNS OF HUMA 970 015 TRIER JS N ENGL J MED 285 1470 971 PN 78164 RN 00944 AN 78072649 AU Holsclaw-D-S-Jr. TI Recognition and management of patients with cystic fibrosis. SO Pediatr-Ann. 1978 Jan. 7(1). P 9-10, 12-6, 21-2 passim. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: th. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, di, pa, th. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: et. LIVER: pa. LUNG: pa. MECONIUM. MIDDLE-AGE. PANCREAS: pa. RESPIRATORY-TRACT-INFECTIONS: dt. INFERTILITY: et. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. EX Cystic fibrosis is the most common lethal genetic disease in childhood, and the broad range of its clinical expression can involve practically every specialty of medicine. The pathology of cystic fibrosis, its clinical features, diagnosis, and treatment are discussed. The pediatrician will not want to attempt to provide exclusive care for the CF patient. These patients require the many supports that only a specialized center will have. Chest surgeons, pediatric surgeons, otolaryngologists, cardiologists, gastroenterologists - in fact, all fields of medicine and all medical specialties - may be needed for the various clinical problems associated with the disease. RF 001 MCKUSICK VA MENDELIAN INHERIT IN MAN 975 002 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 HOLSCLAW DS CLIN PEDIATR 9 346 970 005 SHWACHMAN H PEDIATRICS 30 389 962 006 ANDERSEN DH AM J DIS CHILD 56 344 938 007 CRAIG JM AM J DIS CHILD 93 357 957 008 HOLSCLAW DS AM J DIS CHILD 109 101 965 009 SHWACHMAN H N ENGL J MED 286 1300 972 010 HOLSCLAW DS J UROL 106 568 971 011 HOLSCLAW DS PEDIATRICS 48 442 971 012 KOPITO LE FERTIL STERIL 24 512 973 013 OPPENHEIMER EH J PEDIATR 77 991 970 014 WOOD RE AM REV RESPIR DIS 113 833 976 015 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 016 DOGGETT RG J PEDIATR 68 215 966 017 KULCZYCKI LL N ENGL J MED 259 409 958 018 VALMAN HB ARCH DIS CHILD 46 805 971 019 KESSLER WR PEDIATRICS 8 648 951 020 ANON J PEDIATR 88 711 976 021 GIBSON LE PROC FOR QUANTITATIVE ION 975 022 SHWACHMAN H AM J DIS CHILD 96 6 958 023 WARING WW ADV PEDIATR 23 401 976 024 HUANG NN GUIDE TO DRUG THERAPY IN PATI 974 025 TECKLIN JS PHYS THER 55 1081 975 026 STERN RC CLIN PEDIATR 11 521 972 027 HANDWERGER S N ENGL J MED 281 451 969 028 HOLSCLAW DS J PEDIATR 76 829 970 029 HOLSCLAW DS PEDIATRICS 48 51 971 030 SIASSI B J PEDIATR 78 794 971 031 KELMINSON LL PEDIATRICS 39 24 967 032 GAYTON WF AM J DIS CHILD 126 856 973 033 GAYTON WF PEDIATRICS 59 888 977 034 BOYLE IR J PEDIATR 88 318 976 035 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 CT 1 CHO YW J CLIN PHARMACOL 21 224 981 2 STANGHELLE JK ACTA PAEDIATR SCAND 72 935 983 PN 78165 RN 00945 AN 78072646 AU Huang-N-N. TI The use of new antibiotic agents for chronic pulmonary disease. SO Pediatr-Ann. 1978 Jan. 7(1). P 56, 58-60, 62 passim. MJ ANTIBIOTICS: tu. BACTERIAL-INFECTIONS: dt. RESPIRATORY-TRACT-INFECTIONS: dt. MN AGAMMAGLOBULINEMIA: co. AMINOGLYCOSIDES: tu. ASTHMA: co. CEPHALOSPORINS: tu. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: co. PENICILLINS: tu. PNEUMONIA-ASPIRATION: co. RESPIRATORY-TRACT-INFECTIONS: et. SULFAMETHOXAZOLE: tu. TETRACYCLINES: tu. TRIMETHOPRIM: tu. AB Modern advances in the development of antimicrobial agents and in chemotherapy have made available potent aminoglycoside antibiotics, with more effective ones to come. Their effectiveness against P. aeruginosa is a great contribution to patients with cystic fibrosis and other chronic disorders. The development of carbenicillin has augmented the effectiveness of the aminoglycoside antibiotics. Ticarcillin is similar to carbenicillin and will play a similar role in antibiotic therapy. The cephalosporins serve as alternative agents principally for their antistaphylococcal activity. We are urgently in need of a potent antibiotic agent against P. aeruginosa that can be given by the oral route. The TMP-SMX combination is a potent chemotherapeutic agent that can be administered by the oral route. It is effective in the treatment of P. carinii pneumonia, which is very common owing to the extended survival of patients with leukemia and other malignancies or with allografts who are prone to develop infections with immunosuppressive therapy. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 CUMMING WA RADIOLOGY 105 387 972 003 BARTLETT JG CHEST 68 560 975 004 KIM IG LARYNGOSCOPE 83 347 973 005 GOSINK BB AM J ROENTG RAD THER NUCL MED 117 816 973 006 LARAYA-CUASAY LR AM REV RESPIR DIS 109 703 974 007 BAUER AW AM J CLIN PATHOL 45 493 966 008 STRATFORD BC LANCET 1 378 974 009 MENDELSON J ANTIMICROB AGENTS CHEMOTHER 9 633 976 010 MEYERS RM ARCH OTOLARYNGOL 92 160 970 011 KOSSEK JC J LAB INVEST 30 48 974 012 BOBROW SN JAMA 222 1546 972 013 HOLMES RK ANTIMICROB AGENTS CHEMOTHER 6 253 974 014 HOUANG ET LANCET 1 423 976 015 NEU HC J INFECT DIS SUPPL 134 3 976 016 HUANG NN INFECTION SUPPL 4 465 976 017 MARKS MI J CLIN PHARMACOL 15 246 975 018 HUANG NN CLINICAL EXPERIENCE WITH AMIK 976 019 KHAN AJ CURR THER RES 19 479 976 020 KLASTERSKY J J MED MICROBIOL 7 465 974 021 LEVISON ME ANTIMICROB AGENTS CHEMOTHER 5 667 974 022 MCLAUGHLIN JE LANCET 1 261 971 023 RIFF LJ ARCH INTERN MED 130 887 972 024 LEVINE BB J INFECT DIS SUPPL 128 364 973 025 TUANO SB ANTIMICROB AGENTS CHEMOTHER 6 101 966 026 BURTON JR JAMA 229 679 974 027 THORNHILL TS APPL MICROBIOL 17 457 969 028 STORE SD J POSTGRAD MED 21 121 975 029 KIRBY WMM J INFECT DIS SUPPL 128 341 973 030 STANFORD HC J INFECT DIS SUPPL 122 9 970 031 HUANG NN J PEDIATR 78 338 971 032 BROWN CH 3RD N ENGL J MED 291 265 974 033 ERVIN ER ANTIMICROB AGENTS CHEMOTHER 9 94 976 034 PARRY MF J PEDIATR 90 144 977 035 PARRY MF J INFECT DIS SUPPL 134 194 976 036 NEU HC ANTIMICROB AGENTS CHEMOTHER 8 457 975 037 HUANG NN ANTIMICROB AGENTS CHEMOTHER 6 127 966 038 PINES A PRACTITIONER 213 727 974 039 BROGDEN RN DRUGS 9 251 975 040 GROSSMAN ER PEDIATRICS 47 567 971 041 FRIMPTER GW JAMA 184 111 963 042 KABU SB CLIN PHARMACOL THER 9 550 968 043 BERGAN T ACTA MED SCAND 192 483 972 044 LEWIN EB J INFECT DIS SUPPL 128 618 973 045 STORE SD J POSTGRAD MED 21 114 975 046 KNIGHT D LANCET 2 717 975 047 HUGHES WT CANCER 36 2004 975 048 HUGHES WT N ENGL J MED 295 726 976 PN 78166 RN 00946 AN 78072644 AU Holsclaw-D-S-Jr. Keith-H-H. Palmer-J. TI Meconium screening for cystic fibrosis. SO Pediatr-Ann. 1978 Jan. 7(1). P 29-30, 35, 38-40. MJ CYSTIC-FIBROSIS: di. MASS-SCREENING. MECONIUM: an. MN ALBUMINS: an. CYSTIC-FIBROSIS: oc. FALSE-NEGATIVE-REACTIONS. HUMAN. INFANT-NEWBORN. METHODS. REAGENT-STRIPS. SWEAT: an. SUPPORT-U-S-GOVT-NON-P-H-S. UNITED-STATES. AB In our hands, the BMC-Test Meconium has been a significant step towards the goal of developing an ideal newborn screening test for CF. It is easily performed, is highly specific, has reasonably high sensitivity, and--given its limitation of identifying only patients with CF who have intrauterine pancreatic insufficiency--is the best method of screening newborns for CF that has been devised to date. Many technical problems remain to be solved before the test can be endorsed without reservation. Because the test, as currently constituted, does not identify all potential subjects for further testing who might have CF, it should not be made mandatory. It is, however, better than other available screening methods for CF and can be praised for that benefit. The concept has indicated a valuable new direction for mass-screening possibilities and perhaps can, by future modification, be made sensitive enough to warrant universal usage. RF 001 ANON GAP CONF REP PROB SWEAT TESTI 975 002 STEPHAN U NEONATAL MASS SCREENING FOR C 976 003 LANDSTEINER K ZENTRALBL ALLG PATHOL 16 903 905 004 KORNBLITH BA AM J PATHOL 5 249 929 005 FARBER S AM J DIS CHILD 64 953 942 006 FARBER S J PEDIATR 24 387 944 007 RAPOPORT S SCIENCE 112 150 950 008 BUCHANAN DJ PEDIATRICS 9 304 952 009 GREEN MN PEDIATRICS 21 635 958 010 WISER WC PEDIATRICS 33 115 964 011 SCHUTT WH ARCH DIS CHILD 43 178 968 012 GREEN MN PEDIATRICS 41 989 968 013 DAVIDSON AG BR MED J 4 362 971 014 CAIN ARR ARCH DIS CHILD 47 131 972 015 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 016 SZIBOR R HELV PAEDIATR ACTA 28 359 973 017 SZIBOR R DTSCH GESUNDHEITSW 28 2252 973 018 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 019 BRUNS WT AM J DIS CHILD 131 71 977 020 ANON PROC EWGCF 5TH ANNU MTG 974 021 STEPHAN U PEDIATRICS 55 35 975 022 MCKUSICK VA MENDELIAN INHERIT IN MAN 167 968 023 ANON CF FND 1974 REP SURVI STUD PA 976 024 SCRIVER CR PEDIATRICS 58 757 976 025 HOLTZMAN NA JAMA 229 667 974 026 FISHER DA J PEDIATR 89 692 976 PN 78167 RN 00947 AN 78157263 AU Savoca-C-J. Brasch-R-C. Gooding-C-A. Gamsu-G. TI The right paratracheal stripe in children. SO Pediatr-Radiol. 1978 Feb 28. 6(4). P 203-7. MJ THORACIC-RADIOGRAPHY. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: ra. HUMAN. INFANT. LYMPHOSARCOMA: ra. MALE. NEOPLASM-RECURRENCE-LOCAL: ra. REFERENCE-VALUES. PLEURAL-EFFUSION: ra. SALIVARY-GLAND-NEOPLASMS: ra. SUBMANDIBULAR-GLAND: ra. SUPPORT-U-S-GOVT-P-H-S. AB The width of the right paratracheal stripe (RPS) has been established in normal adults but not in normal children. The thymus and great vessels are relatively larger in children than in adults and could obscure or widen the RPS. We found that obscuration does occur and, therefore, the RPS is less often measurable in children than in adults. Widening by the thymus and great vessels, however, does not occur. The width of the RPS in normal children, 0.5 to 3.0 mm, is slightly less than in adults. From this study of normal children and our subsequent experience with pediatric patients, we conclude that in a child an RPS 4 mm or wider is reliable evidence of disease affecting the trachea, mediastinum, or pleura. RF 001 SAVOCA CJ RADIOLOGY 122 295 977 CT 1 NEUFANG KFR LYMPHOLOGY 15 132 982 PN 78168 RN 00948 AN 79054260 AU Moffat-R-E. Sobonya-R-E. Chang-C-H. TI Childhood sarcoidosis with fatal cor pulmonale. SO Pediatr-Radiol. 1978 Sep 26. 7(3). P 180-2. MJ LUNG-DISEASES: di. PULMONARY-HEART-DISEASE: di. SARCOIDOSIS: di. MN ADOLESCENCE. CASE-REPORT. CYSTIC-FIBROSIS: di. DIAGNOSIS-DIFFERENTIAL. FEMALE. HUMAN. LUNG-DISEASES: ra. LUNG: ra. PULMONARY-HEART-DISEASE: ra. SARCOIDOSIS: ra. AB A 14 year-old white girl who developed fatal cor pulmonale from severe pulmonary sarcoidosis had a previous clinical diagnosis of cystic fibrosis. Chest radiographs demonstrated marked pulmonary fibrosis without the changes of severe bronchiectasis that would be anticipated with cystic fibrosis. RF 001 HAUSER H OKLA STATE MED ASSOC 39 395 946 002 KENDIG EL JR CHEST 70 351 976 003 KIRKS DR AM J ROENTG RAD THER NUCL MED 117 777 973 004 KOERNER SK N ENGL J MED 293 268 975 005 MCGOVERN JP ADV PEDIATR 8 97 956 PN 78169 RN 00949 AN 79011613 AU Wilson-G-B. Fudenberg-H-H. TI Is cystic fibrosis protein a diagnostic marker for individuals who harbor the defective gene? [letter]. SO Pediatr-Res. 1978 Jul. 12(7). P 801-4. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. HETEROZYGOTE-DETECTION. MN HUMAN. SUPPORT-U-S-GOVT-P-H-S. EX It has been almost 5 years since the publication of our original report describing a "cystic fibrosis factor" (now designated "cystic fibrosis protein" or CFP) detected in the serum of most homozygotes and heterozygotes for cystic fibrosis when analyzed using isoelectric focusing in thin layer polyacrylamide gels. The recent report by Scholey et al. confirms that CFP is present in the majority of CF sera and that it can be detected by our methods. Before discussing whether the CFP can be used as a diagnostic marker for cystic fibrosis, we would like to examine more closely some of the requirements of the method which seemed to have been critical for the detection of CFP by Scholey et al. We would like to point out that Scholey and his coworkers adhered closely to most of our guidelines during their attempts to detect CFP. The exact nature of CFP and the other ciliary dyskinesia factors is only partially known, and all aspects of their metabolism are not yet understood. Therefore, it may be some time before we comprehend the incomplete concordance found between CFP and the pathogenesis of CF. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 AWDEH ZL NATURE 219 66 968 003 DANES BS CLIN GENET 11 83 977 004 SCHOLEY J PEDIATR RES 12 800 978 005 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 006 SMITH QT PEDIATR RES 10 999 976 007 THOMAS JM PEDIATR RES 11 138 977 008 THOMAS JM PEDIATR RES 11 1148 977 009 WARNER JO LANCET 1 990 976 010 WILSON GB PEDIATR RES 11 986 977 011 WILSON GB PEDIATR RES 10 1001 976 014 WILSON GB SCAND J IMMUNOL 5 829 976 015 WILSON GB CLIN RES 26 501 978 016 WILSON GB PEDIATR RES 10 87 976 017 WILSON GB TEX REP BIOL MED 34 51 976 018 WILSON GB ADV POLYMINE RES 2 281 978 019 WILSON GB PEDIATR RES 9 635 975 020 WILSON GB CLIN CHIM ACTA 49 79 973 021 WILSON GB IN: RADOLA BJ 337 977 022 WILSON GB PEDIATR RES 11 139 977 023 ANON J PEDIATR 88 711 976 CT 1 TULLY GW PEDIATR RES 13 1078 979 2 WILSON GB PEDIATR RES 13 1079 979 3 MANSON JC LANCET 1 330 980 4 NEVIN GB HUM GENET 56 387 981 5 HALLINAN FM CLIN CHIM ACTA 117 103 981 6 BULLOCK S CLIN GENET 21 336 982 7 BROCK DJH HUM GENET 60 30 982 8 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 9 WILSON GB CLIN GENET 26 331 984 10 HODSON ME POSTGRAD MED J 60 225 984 11 KLINGER KW SEM RESPIR MED 6 243 985 12 KIRKPATRICK C PEDIATR RES 19 1341 985 13 JAMIESON A HUM GENET 70 168 985 14 KUZEMKO JA J ROY SOC MED 79 2 986 PN 78170 RN 00950 AN 78157307 AU Impero-J-E. Harrison-G-M. Nelson-T-E. TI Cystic fibrosis: isolation and physical properties of a salivary cystic fibrosis factor. SO Pediatr-Res. 1978 Feb. 12(2). P 108-14. MJ CYSTIC-FIBROSIS: me. SALIVA: an. SALIVARY-PROTEINS: ip. MN ALPHA-AMYLASE: ip, pd. ANIMAL. CILIA: de. GLYCOGEN-DEBRANCHING-ENZYME: ai. GLYCOGEN. HEPARIN. HUMAN. HYDROGEN-ION-CONCENTRATION. IN-VITRO. METHODS. OYSTERS. RABBITS. SALIVARY-PROTEINS: pd. TEMPERATURE. SUPPORT-U-S-GOVT-P-H-S. AB A ciliostatic factor has been isolated from cystic fibrosis (CF) saliva by dialyzing it from purified alpha-amylase prepared by a glycogen-complex method. This method of isolating the CF factor is an improvement over the previously employed heparin procedure. The activity of the isolated factor is proportional with concentration using the oyster gill ciliostatic assay and in its inhibition of mammalian glycogen debranching enzyme. The ciliostatic action of the factor can be reversed by heparin under certain conditions. The type of inhibition of the debranching enzyme by the isolated CF factor indicates that its chemical structure is similar to that observed with hydroxyalkylamines and polyamine metabolites. Physical properties of the isolated factor indicate that it is of low molecular weight and is labile as a function of pH and temperature. At neutral pH the conditions under which it is maintained have a direct effect on the length of time that it is stable. RF 001 ARAKI H PEDIATR RES 9 932 975 002 ASANO T AM J PHYSIOL 207 415 964 003 BERNFELD P METHODS ENZYMOL 1 149 955 004 BESLEY GTN J MED GENET 6 278 969 005 BISHOP SH CF CLUB ABST 14 25 973 006 BOWMAN BH IN: MANGOS JA 277 976 007 BOWMAN BH SCIENCE 164 325 969 008 ANON GAP CONF REP CILIARY INHIBITO 2 973 009 CONOVER JH PEDIATR RES 7 220 973 010 DANES BS CLIN GENET 7 128 975 011 DANES BS J EXP MED 136 1313 972 012 DANES BS CLIN GENET 8 85 975 013 DEARBORN DG GAP CONF REP POLYAMINES 19 976 014 DI SANTAGNESE PA N ENGL J MED 295 481 976 015 DOGGETT RG TEX REP BIOL MED 31 685 973 016 DOGGETT RG NATURE NEW BIOL 243 250 973 018 DUBOIS M ANAL CHEM 28 350 956 019 FISHER EH BIOCHEM PREP 8 27 961 020 GILLARD BK PEDIATR RES 10 907 976 021 GILLARD BK BIOCHEMISTRY 16 3978 977 022 KAISER D LANCET 1 1003 970 023 KIMES BW BIOCHIM BIOPHYS ACTA 228 223 971 024 LEVITZKI A BIOCHIM BIOPHYS ACTA 81 101 964 025 LOBECK CC IN: STANBURY JB 1605 972 026 LOCKHART LH TEX REP BIOL MED 31 631 973 027 LOWRY OH J BIOL CHEM 193 265 951 028 LOYTER A BIOCHIM BIOPHYS ACTA 65 200 962 029 MANGOS JA SCIENCE 158 135 967 030 MANGOS JA PEDIATR RES 2 378 968 031 MANGOS JA PEDIATR RES 1 436 967 032 MCCOMBS ML TEX REP BIOL MED 31 615 973 033 MCNEELY CM CF CLUB ABST 16 6 975 034 MILLER GL ANAL CHEM 31 964 959 035 NELSON TE CF CLUB ABST 7 974 036 NELSON TE FED PROC 33 1311 974 037 NELSON TE BIOCHEMISTRY 8 1419 969 038 NELSON TE FED PROC 32 627 973 039 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 040 SPOCK A PEDIATR RES 1 173 967 041 TAYLOR A PEDIATR RES 8 861 974 042 WATTS TE ANAL BIOCHEM 49 479 972 043 WILSON GB PEDIATR RES 9 635 975 044 WOOD RE LANCET 2 1452 973 CT 1 IMPERO JE PEDIATR RES 15 940 981 2 CEDER O SCANN ELECTRON MICROSC 1982 723 982 3 BOGART BI J CHROMATOGR 381 29 986 PN 78171 RN 00951 AN 78248077 AU Platt-M-W. Rao-G-J. Nadler-H-L. TI Reaction of 4-methylumbelliferylguanidinobenzoate with cultivated human skin fibroblasts derived from patients with cystic fibrosis. SO Pediatr-Res. 1978 Aug. 12(8). P 874-7. MJ BIOLOGICAL-ASSAY. CYSTIC-FIBROSIS: en. HYMECROMONE: du. PEPTIDE-HYDROLASES: me. UMBELLIFERONES: du. MN CELLS-CULTURED. FIBROBLASTS: en. GUANIDINES: du. HUMAN. HYMECROMONE: aa. SKIN: en. SUPPORT-U-S-GOVT-P-H-S. AB Protease activity in cultivated human skin fibroblasts has been quantitated using 4-methylumbelliferylguanidinobenzoate (MUGB), an active site titrant of trypsin-like proteases (7). The reaction of the proteases with MUGB was complete in 1 hr, inhibited both by benzamidine and (p-nitrophenyl)-p'-guanidinobenzoate, but not by p- hydroxymercuribenzoate. The extent of reaction was proportional to protein concentration and independent of MUGB concentration. This activity was present in the particulate fraction of the cell. The mean "titre" values (nanomoles of 4-methylumbelliferone released per mg protein) of the proteases in fibroblasts from eight controls (N), 8 obligate heterozygotes (H), and 14 patients with cystic fibrosis (CF) were: N, 1.27 +/- 0.11; H, 0.82 +/- 0.12; CF, 0.66 +/- 0.10. The differences in the "titre" values for N:CF and N:H were significant (p less than 0.001) as were those for H:CF (p less than 0.01). The mean "titre" value obtained for cultivated control amniotic fluid cells was 1.29 +/- 0.17. These data indicate a reduction in the MUGB- reactive proteases in skin fibroblasts derived from patients with CF when compared either to control or to obligate heterozygotes. These data are consistent with our earlier suggestion (11, 15) that decreased proteolytic levels in the tissues and fluids of patients with CF may be a generalized phenomenon. RF 001 BARRETT AJ BIOCHEM J 131 809 973 002 BROWN CR BIOCHEM J 149 147 975 003 CALLAHAN JW PEDIATR RES 11 1166 977 004 CHASE T JR BIOCHEM BIOPHYS RES COMMUN 29 508 967 005 CHASE T JR BIOCHEMISTRY 8 2212 969 006 HATCHER VB BIOCHEM BIOPHYS RES COMMUN 76 602 977 007 JAMESON GW BIOCHEM J 131 107 973 008 KERR MA BIOCHEMISTRY 14 5088 975 009 LOWRY OH J BIOL CHEM 193 265 951 010 NADLER HL NATURE 213 1261 967 011 WALSH-PLATT M AM J HUM GENET 29 111A 977 012 RAO GJS J PEDIATR 80 573 972 013 RAO GJS PEDIATR RES 8 684 974 014 RAO GJS PEDIATR RES 9 739 975 CT 1 BROCK DJH LANCET 1 1245 979 2 WALSH MM LANCET 1 622 979 3 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 4 WALSHPLATT M ENZYME 24 224 979 5 NADLER HL LANCET 2 96 980 6 NADLER HL PEDIATRICS 66 690 980 7 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 8 TUMMLER B CLIN CHIM ACTA 125 219 982 9 GREEN JR EUR J PEDIATR 139 35 982 10 BROCK DJH PRENAT DIAGN 3 1 983 11 BRIDGES MA ANN NY ACAD SCI 421 360 983 12 CEDER O ACTA PAEDIATR SCAND 1983 1 983 13 BUYS CHCM CLIN CHIM ACTA 136 229 984 PN 78172 RN 00952 AN 78201164 AU Knoke-J-D. Stern-R-C. Doershuk-C-F. Boat-T-F. Matthews-L-W. TI Cystic fibrosis: the prognosis for five-year survival. SO Pediatr-Res. 1978 May. 12(5). P 676-9. MJ CYSTIC-FIBROSIS: mo. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. PROBABILITY. PROGNOSIS. SUPPORT-U-S-GOVT-P-H-S. AB Statistical discriminant analysis is applied to 41 concomitant variables obtained during the first year of study of 226 patients with cystic fibrosis. A discriminant function based on six variables is developed which can be used as a predictive index. This index estimates the probability of 5-year survival for an individual patient and can also be used to classify patients into one of two groups: (1) will live for 5 years or (2) will die within 5 years. Speculation A classification rule for cystic fibrosis is presented which correctly assigns 90% of the 5-year survivors and 84% of those who expire. Such a classification scheme is useful for clinical and research purposes. RF 001 AFIFI AA AM J CARDIOL 33 826 974 002 AFIFI AA N ENGL J MED 285 1497 971 003 CHANG PC J AM STATIST ASSOC 69 336 974 004 DIXON WJ BMDP: BIOMEDICAL COMPUTER PRO 975 005 DOERSHUK CF J PEDIATR 65 677 964 006 LACHENBRUCH PA DISCRIMINANT ANALYSIS 975 007 LACHENBRUCH PA TECHNOMETRICS 10 1 968 008 LURIA MH ANN INTERN MED 85 561 976 009 MATTHEWS LW J PEDIATR 65 558 964 010 MCCABE GP JR TECHNOMETRICS 17 103 975 011 MOORE DH J AM STATIST ASSOC 68 399 973 012 SHUBIN H CARDIOVASC RES 2 329 968 013 SHWACHMAN H AM J DIS CHILD 96 6 958 014 STERN RC J PEDIATR 89 406 976 015 TAUSSIG LM J PEDIATR 82 380 973 CT 1 KRAEMER R HELV PAEDIATR ACTA 34 417 979 2 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 3 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 4 DAVIS PB SEM RESPIR MED 6 261 985 PN 78173 RN 00953 AN 78248067 AU Wright-R-K. Buehler-B-A. Schott-S-N. Rennert-O-M. TI Spermine and spermidine, modulators of the cell surface enzyme adenylate cyclase. SO Pediatr-Res. 1978 Aug. 12(8). P 830-3. MJ ADENYL-CYCLASE: ai. CYSTIC-FIBROSIS: en. SPERMIDINE: pd. SPERMINE: pd. MN ADULT. CELL-MEMBRANE: de, en. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pa. FIBROBLASTS: de. HUMAN. IN-VITRO. INFANT-NEWBORN. SUPPORT-U-S-GOVT-P-H-S. AB Adenylate cyclase activity was measured in membrane preparations of cultured fibroblasts from controls and patients with cystic fibrosis. Enzyme activity increased as the transition from exponential growth to confluence occurred; sodium fluoride-stimulated activity more markedly displayed this relationship than basal cyclase activity. The in vitro addition of spermine (1 X 10(-6) to 2 X 10(-3) M) to membrane preparations caused inhibition of basal and sodium fluoride- stimulated enzyme activity, with 50% inhibition of basal activity occurring at 10(-6) M spermine. Spermidine (10(-4) M) caused 15--25% inhibition of adenylate cyclase activity. The increase in fibroblast adenylate cyclase activity during the transition from exponential growth was comparable in cells obtained from cystic fibrosis patients and control subjects. Basal and sodium-fluoride stimulated adenylate cyclase activity as well as inhibition of this enzyme activity by spermidine and spermine were undistinguishable between the different cell genotypes. A potential modulation of cellular proliferative activity through polyamine interaction with the adenylate cyclase system is postulated. RF 001 ANDERSON WB ADV CYCLIC NUCLEO RES 5 681 975 002 AMATRUDA J BIOCHIM BIOPHYS ACTA 373 266 974 003 ARVANITAKIS S TEX REP BIOL MED 34 175 976 004 BUEHLER B PEDIATR RES 11 186 977 005 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 006 CHUN P BIOCHEM BIOPHYS RES COMMUN 69 1095 976 007 COHEN LF PEDIATR RES 9 334 975 008 CUETRECASAS P ADV CYCLIC NUCLEO RES 5 79 975 009 DYKSTRA WG SCIENCE 149 428 965 010 FRANKS DJ FEBS LETTERS 42 267 974 011 HOLTTA E BIOCHEM BIOPHYS RES COMMUN 54 350 973 012 JOHNSON RA J BIOL CHEM 250 6599 975 013 KOSSOROTOW A BIOCHEM J 144 21 974 014 LOWRY OH J BIOL CHEM 193 265 951 015 MANGOS JA IN: MANGOS JA 311 976 016 ONEILL J BIOCHIM BIOPHYS ACTA 404 243 975 017 OTTEN J J BIOL CHEM 247 7082 972 018 PROCTOR MS J INVEST DERMATOL 65 409 975 019 RAINA A MED BIOL 53 121 975 020 RENNERT OM TEX REP BIOL MED 34 187 976 021 RICHARDS GM ANAL BIOCHEM 57 369 974 022 ROSENTHAL S BIOCHIM BIOPHYS ACTA 363 141 974 023 RUSSELL DH PROC NAT ACAD SCI USA 60 1420 968 024 SALOMON Y ANAL BIOCHEM 58 541 974 025 TASHIMA Y BIOCHEM BIOPHYS RES COMMUN 66 1344 975 026 WOLFF J J BIOL CHEM 250 6897 975 027 WRIGHT RK ARCH DERMATOL 107 47 973 CT 1 ILLIANO G BULL MOL BIOL MED 4 155 979 2 CANELLAKIS ES CURR TOP CELL REGUL 15 155 979 3 TINTNER R J NEUROCHEM 33 1067 979 4 ALLEN JC EUR J BIOCHEM 102 153 979 5 GAHL WA PEDIATR RES 14 118 980 6 KUROSHIMA A JAP J PHYSIOL 30 935 980 7 KIMBERLY MM ANAL CHEM 53 789 981 PN 78174 RN 00954 AN 79011612 AU Scholey-J. Applegarth-D-A. Davidson-A-G. Wong-L-T. TI Detection of cystic fibrosis protein by electrofocusing [letter]. SO Pediatr-Res. 1978 Jul. 12(7). P 800. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. MN HUMAN. ISOELECTRIC-FOCUSING. EX Wilson et al. reported a biophysical assay for cystic fibrosis that employed isoelectric focusing of serum proteins. Their work appeared to establish the presence of a definitive marker for CF serum in the form of a protein band that was not present in normal serum. We tried to repeat their work and found that the background color of the fresh electropherograms made it rather difficult to detect unequivocally the presence or absence of bands in the appropriate pH region in CF vs. control serum. However, when the gels were photographed using Kodak Panatomic X film and a Wratten 25 A red filter, the color response and contrast of the film eliminated this interference to a great extent and it was much easier to detect the putative band. We agree partially with the observations of Wilson and Fudenberg, but would like to stress that the technique is not a diagnostic test. RF 001 SMITH QT PEDIATR RES 10 999 976 002 THOMAS JM PEDIATR RES 11 1148 977 003 WILSON GB PEDIATR RES 11 986 977 004 WILSON GB PEDIATR RES 10 1001 976 005 WILSON GB PEDIATR RES 9 635 975 006 WILSON GB PEDIATR RES 11 139 977 CT 1 TULLY GW PEDIATR RES 13 1078 979 2 WILSON GB PEDIATR RES 13 1079 979 3 MANSON JC LANCET 1 330 980 4 WILSON GB LANCET 2 313 980 5 NEVIN GB HUM GENET 56 387 981 6 HALLINAN FM CLIN CHIM ACTA 117 103 981 7 BULLOCK S CLIN GENET 21 336 982 8 BROCK DJH HUM GENET 60 30 982 9 BURY AF CLIN CHIM ACTA 118 45 982 10 GRATAROLI R PEDIATR RES 18 130 984 11 ANON LANCET 2 249 985 12 JAMIESON A HUM GENET 70 168 985 13 QURESHI AR J PEDIATR 106 913 985 14 HAYWARD C J IMMUNOL METH 91 117 986 PN 78175 RN 00955 AN 78157324 AU Perlmutter-J. Martinez-J-R. TI The chronically reserpinized rat as a possible model for cystic fibrosis. VII. Alterations in the secretory response to cholecystokinin and to secretin from the pancreas in vivo. SO Pediatr-Res. 1978 Mar. 12(3). P 188-94. MJ CYSTIC-FIBROSIS: pp. DISEASE-MODELS-ANIMAL. PANCREATIC-JUICE: se. RESERPINE: pd. MN AMYLASES: se. ANIMAL. CHOLECYSTOKININ: pd. ELECTROLYTES: se. MALE. ORGAN-WEIGHT: de. PANCREATIC-JUICE: de. PROTEINS: se. RATS. SECRETIN: pd. TIME-FACTORS. AB The chronically reserpinized rat has been proposed as an animal model for cystic fibrosis on the basis of morphologic and secretory alterations in the submaxillary gland and of abnormalities in pulmonary secretions. In this investigation, the volume and composition of pancreatic juice from reserpine treated rats (0.5 mg/kg/day) have been compared to those of untreated controls after stimulation with purified cholecystokinin (0.1 microgram/kg body weight) and with crude and purified preparations of secretin (6 microns/100 g body weight) infused iv for 30-min periods. The results demonstrate that the treated animals secreted a significantly lower volume of pancreatic juice after stimulation with these secretagogues. Flow rates were also significantly reduced after stimulation with cholecystokinin and crude secretin. Protein and amylase outputs in response to cholecystokinin were smaller than in control animals after unrestritced feedings, but greater after a 24-hr fast. Total bicarbonate output was also reduced after stimulation with either crude or purified secretin and the normal excretion patterns for bicarbonate and chloride were either absent of reversed in the secretin-stimulated pancreatic juice of the treated animals. Whole pancreas homogenates from the treated animals showed significant increases in Ca++ and protein content. These results indicate that chronic administration of reserpine alters the secretion of water, protein, and bicarbonate from the rat pancreas and that it affects several of the exocrine glands involved in cystic fibrosis. These findings lend support to the concept of an animal model for the human disease. The chronic administration of reserpine to rats has been shown to induce alterations in salivary and pulmonary secretions which resemble those seen in patients with cystic fibrosis. The concept of an animal model has been proposed on the basis of these observations. The pancreas is another exocrine gland prominently involved in cystic fibrosis and if it can be demonstrated that reserpine administration also induces alterations in pancreatic secretion, the concept of the animal model would be strengthened considerably. RF 001 BOGART BI PEDIATR RES 11 131 977 002 CASE RM J PHYSIOL (LOND) 235 75 973 003 DI SANTAGNESE PA N ENGL J MED 277 1287 967 004 EIMERL S J BIOL CHEM 249 3991 974 005 HADORN B IN: LAWSON D PROC 5TH INT CF 56 969 006 IMMKEN L CF CLUB ABST 16 9 975 007 LOWRY OH J BIOL CHEM 193 265 951 008 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 009 MANGOS JA AM J PHYSIOL 221 496 971 010 MANGOS JA PROC SOC EXP BIOL MED 146 321 974 011 MARTINEZ JR PEDIATR RES 9 463 975 012 MARTINEZ JR PEDIATR RES 9 470 975 013 SEWELL WA J PHYSIOL (LOND) 252 379 975 014 SHAW HM AUST J BIOL SCI 25 147 972 015 SHAW HM J EXP PHYSIOL 58 229 973 016 THOMPSON FE PEDIATR RES 10 632 976 017 WILLIAMS JA BIOCHEM BIOPHYS RES COMMUN 60 542 974 018 WOOD DL PEDIATR RES 11 827 977 CT 1 SIMSON JAV LAB INVEST 39 157 978 2 MARTINEZ JR PEDIATR RES 13 1156 979 3 MAWHINNEY TP PEDIATR RES 13 760 979 4 SETSER ME LAB INVEST 41 256 979 5 DAVIS PB PEDIATR RES 14 83 980 6 MAWHINNEY TP PEDIATR RES 14 872 980 7 MORTON D PEDIATR RES 14 18 980 8 MCCURDY RE PEDIATR RES 15 1308 981 9 CUTLER LS VIRCHOWS ARCH PATHOL ANAT HIS 392 185 981 10 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 11 SHIFFMAN ML PEDIATR RES 16 104 982 12 MACLEOD RM DEVELOP NEUROSCI 16 271 983 13 MARTINEZ JR PEDIATR RES 17 523 983 14 SHIFFMAN ML PEDIATR RES 17 486 983 15 MODRZAKOWSKI MC CAN J MICROBIOL 29 1339 983 16 LINGARD JM AM J PHYSIOL 245 G690 983 17 LOGIN IS BRAIN RES 260 99 983 18 CASTEELS R J PHYSIOL (LOND) 340 403 983 19 BOYD RL PEDIATR RES 18 1028 984 20 CHUNG KS PEDIATR RES 18 1300 984 21 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 22 MARTINEZ JR PEDIATR RES 19 711 985 23 HAZLETT D PEDIATR RES 20 1236 986 PN 78176 RN 00956 AN 78248075 AU Gillard-B-K. Markman-H-C. Feig-S-A. TI Differences between cystic fibrosis and normal saliva alpha-amylase as a function of age and sex. SO Pediatr-Res. 1978 Aug. 12(8). P 868-72. MJ ALPHA-AMYLASE: me. AMYLASES: me. CYSTIC-FIBROSIS: en. SALIVA: en. MN ADOLESCENCE. ADULT. AGE-FACTORS. ALPHA-AMYLASE: an. CHILD. COMPARATIVE-STUDY. FEMALE. HUMAN. MALE. MIDDLE-AGE. PARENTS. SALIVA: an. SALIVARY-PROTEINS: an. SEX-FACTORS. SUPPORT-U-S-GOVT-P-H-S. AB Alpha-amylase activity and protein concentration of cystic fibrosis (CF), obligate heterozygote (H), and normal (N) saliva have been evaluated with respect to genotype, age, sex and clinical status. "Factors" present in CF saliva had no detectable effect on salivary amylase activity under reaction conditions optimized for both competitive and noncompetitive inhibition. Tris [tris-(hydoxy-methyl)aminomethane] inhibited amylase activity with a Ki = 59 plus or minus 10 mM, but CF "factors" had no detectable effect on either substrate or Tris binding to alpha-amylase. Alpha-amylase concentrationand protein content of CF saliva had bimodal distributions. Thirty-six percent of the CF patients were found to be amylase hypersecretors, with amylase concentration 3 or more SD above the N mean. Amylase hypersecretion was marginally correlated with both genetic factors (with parent values, P = 0.075) and clinical status (with Scwachman scores, P = 0.068). All the hypersecretors were in the 14-24 year age group. Amylase concentration for this age was significant higher in CF samples (mean plus or minus SD), 82 plus or minus 37 starch units/ml (n = 12), than in N samples, 33 plus or minus 17 starch unites/ml (n = 13), as measured by the Student t-test (P < 0.001). Salivary protein content of 14- to 24-year-old CF males was elevated compared to all other groups [1.57 plus or minus 0.76 (n = 5) vs. 0.63 plus or minus 0.47 (n = 20); Student t-test, P < 0.005]. Mean specific activity of male saliva dropped sharply as age increased from 10-15 years, from over 200 to less than 75 starch units/mg protein. Mean normal female specific activity values were essentially constant [97 plus or minus 50 (n = 18) starch units/mg protein] from 5-30 years of age. CF male specific activity values decreased similarly to the N male values from preadolescence to adolescence, but the mean CF female values peaked later, at 17 years, with a maximum of 171 plus or minus 58 (n = 4). This is significantly higher than all the other groups in the 16- to 20-year age range: 63 plus or minus 30 (n = 9), P < 0.005. These data emphasize the need for age- and sex-matched controls in all studies of salivary composition. We were unable to detect any effect of a salivary CF factor on the enzymatic activity of alpha-amylase. Hypersecretion of salivary alpha-amylase was observed in 36% of CF patients. This may reflect alterations in secretory function basic to CF, or be secondary to the disease process and result from decreased secretion from the mucous salivary glands or deterioration of pancreatic function. RF 001 BISHOP SH CF CLUB ABST 14 25 973 002 BLOMFIELD J AUST PAEDIATR J 10 75 974 003 BLOMFIELD J PEDIATR RES 10 574 976 004 BOWMAN BH SCIENCE 164 325 969 005 DI SANTAGNESE PA N ENGL J MED 277 1344 967 006 DIXON M BIOCHEM J 55 170 953 007 DIXON WJ BMDP: BIOMEDICAL COMPUTER PRO 975 008 DOGGETT RG NATURE NEW BIOL 243 250 973 009 DOGGETT RG TEX REP BIOL MED 31 685 973 010 FISHER EH BIOCHEM PREP 8 27 961 011 GILLARD BK PEDIATR RES 10 907 976 012 GILLARD BK CLIN CHEM 23 2279 977 013 GILLARD BK BIOCHEMISTRY 16 3978 977 014 ANON J PEDIATR 88 711 976 015 LEHNINGER AL BIOCHEMISTRY 14 197 975 016 LOWRY OH J BIOL CHEM 193 265 951 017 MANDEL ID AM J DIS CHILD 113 431 967 018 MAYO JW ARCH BIOCHEM BIOPHYS 175 507 976 019 MCNEELY CM CF CLUB ABST 16 6 975 020 MILLER GL ANAL CHEM 31 964 959 021 NELSON TE CF CLUB ABST 15 7 974 022 POULSEN JH J PHYSIOL (LOND) 264 323 977 023 ROBYT JF IN: RADLEY JA 431 968 024 SHWACHMAN H CF CLUB ABST 16 19 975 025 SHWACHMAN H AM J DIS CHILD 96 6 958 026 SKUDE G SCAND J GASTROENTEROL 10 577 975 027 SMITH QT PROC SOC EXP BIOL MED 153 241 976 028 SPOCK A PEDIATR RES 1 173 967 029 TOWNES PL AM J HUM GENET 28 378 976 030 WOLF RO J LAB CLIN MED 87 164 976 031 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 GILLARD BK PEDIATR RES 14 1168 980 2 PARK RW GASTROENTEROLOGY 81 1143 981 3 GILLARD BK CLIN CHEM 29 1119 983 PN 78177 RN 00957 AN 78157291 AU Bogart-B-I. Conod-E-J. Gaerlan-P-F. Conover-J. TI The biologic activities of cystic fibrosis serum. II. Ultrastructural aspects of the effect of cystic fibrosis sera and calcium ionophore A23187 on rabbit tracheal explants. SO Pediatr-Res. 1978 Jan. 12(1). P 15-24. MJ A-23187: pd. ANTIBIOTICS: pd. CYSTIC-FIBROSIS: bl. TRACHEA: de. MN ANIMAL. CALCIUM: me, ph. CILIA: ul. CYSTIC-FIBROSIS: pp. EPITHELIUM: ul. MALE. RABBITS. TRACHEA: ul. AB Ultrastructural and cytochemical observations indicate that both cystic fibrosis (CF) sera and calcium ionophore A23187 induce a swelling or an increase in the size and possibly the number of secondary lysosomes and an increase in mucus secretion in epithelium of the rabbit tracheal bioassay system. Extended incubation of the rabbit tracheal explants with either CF or control sera produces a cytotoxic effect on the tracheal epithelium, but only after the termination of the normal bioassay time period. Comparative ultrastructural study of the effect of both CF sera and calcium ionophore A23187 on the rabbit tracheal bioassay system indicates that increased membrane permeability to calcium may be important in the production of the ciliary dyskinesia response by CF serum factor(s) in the rabbit tracheal bioassay system. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 BARRETT LA CANCER RES 36 1003 976 003 BAUR PS TEX REP BIOL MED 34 155 976 004 BOGART BI J HISTOCHEM CYTOCHEM 16 572 968 005 BOGART BI J ULTRASTRUCT RES 52 139 975 007 CHERRY JD J PEDIATR 79 937 971 008 CHEUNG ATW PEDIATR RES 10 144 976 009 COCHRANE DE PROC NAT ACAD SCI USA 71 408 974 010 CONOVER JH PEDIATR RES 7 220 973 011 CONOVER JH TEX REP BIOL MED 34 45 976 012 DAEMS WT IN: DINGLE JT 1 64 969 013 DETER RL J CELL BIOL 33 437 967 014 DIRKSEN ER CANCER RES 28 906 968 015 DI SANTAGNESE PA PEDIATRICS 12 549 953 016 DI SANTAGNESE PA N ENGL J MED 277 1287 967 017 EIMERL S J BIOL CHEM 249 3991 974 018 ERRICSSON JLE IN: DINGLE JT 2 345 969 019 FARQUHAR MG IN: DINGLE JT 2 462 969 020 FARRELL PM PEDIATR RES 10 127 976 021 GALLIN JI J CELL BIOL 62 594 974 022 GILLESPIE E J CELL BIOL 50 544 971 023 GINN FL AM J PATHOL 53 1041 968 024 JACQUES PJ IN: DINGLE JT 2 395 969 025 KAGAYAMA M J CELL BIOL 62 519 974 026 KIRSCHNA MW J SUPRAMOL STRUCT 2 412 974 027 KURIYAMA R J BIOCHEM (TOKYO) 75 463 975 028 LOBECK CC IN: STANBURY JB 1605 972 029 LUFT JH J BIOPHYS BIOCHEM CYTOL 9 409 961 030 MIRA-MOSER F EUR J CLIN INVEST 6 103 976 031 MOTOKAWA T J CELL BIOL 66 377 975 032 NEUTRA M J CELL BIOL 30 119 966 033 PALADE GE IN: HAYASHI T 64 959 034 PRESSMAN BC FED PROC 32 1698 973 035 RAVAZZOLA M LAB INVEST 35 425 976 036 REED PW J BIOL CHEM 247 6970 972 037 SATIR P SCIENCE 190 586 975 038 SELINGER Z PROC NAT ACAD SCI USA 71 128 974 039 SHELANSKI ML PROC NAT ACAD SCI USA 70 765 973 040 SOMLYO AP J CELL BIOL 66 425 975 041 SPOCK A PEDIATR RES 1 173 967 042 TRUMP BF IN: TRUMP BF 1 975 043 TRUMP BF IN: RICHTER GW 84 971 044 TRUMP BF IN: KARBE F 548 974 045 WEISENBERG RC SCIENCE 177 1104 972 046 WONG DT ARCH BIOCHEM BIOPHYS 156 578 973 CT 1 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 2 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 3 KENNEDY JR PEDIATR RES 14 1173 980 4 SANDERSON MJ PEDIATR RES 15 219 981 5 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 6 CEDER O SCANN ELECTRON MICROSC 1982 723 982 7 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 8 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 9 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 10 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 11 KATZ S CELL CALC 5 421 984 12 NEAL JL EVOLUTIONARY THEORY 7 153 985 13 VONEULER AM EXP MOL PATH 43 142 985 14 BOGART BI J CHROMATOGR 381 29 986 15 ROOMANS GM SCANN ELECTRON MICROSC 1986 165 986 PN 78178 RN 00958 AN 78157289 AU Grinwald-P-M. Segal-M-B. TI Effect of saliva from cystic fibrosis patients and from normal subjects on red blood cell sodium transport. SO Pediatr-Res. 1978 Jan. 12(1). P 1-3. MJ CYSTIC-FIBROSIS: me. ERYTHROCYTES: me. SALIVA: ph. SODIUM: me. MN ADULT. BIOLOGICAL-TRANSPORT. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. MALE. OUABAIN: pd. SODIUM: bl. AB Saliva, whether taken from patients with cystic fibrosis or from normal subjects, caused an increase in red blood cell Na+ efflux (in the presence or absence of ouabain) of 19-29% as compared with non- saliva controls. However, there was no significant difference between the effects of cystic fibrosis saliva and normal saliva. RF 001 BALFE JW SCIENCE 162 689 968 002 DI SANTAGNESE PA N ENGL J MED 277 1344 967 003 DUFFY MJ LANCET 2 136 972 004 HOFFMAN JF ANN NY ACAD SCI 137 566 966 005 KAISER D LANCET 1 1003 970 006 LAPEY A PEDIATR RES 5 446 971 007 MANGOS JA PEDIATR RES 1 436 967 008 SACHS JR J CLIN INVEST 46 65 967 009 TAUSSIG LM LANCET 1 1367 972 CT 1 SEALE TW PEDIATR RES 14 1398 980 2 BERGHOUT AGRV PEDIATR RES 18 1017 984 PN 78179 RN 00959 AN 79073912 AU Davidson-G-P. Koheil-A. Forstner-G-G. TI Salivary amylase in cystic fibrosis: a marker of disordered autonomic function. SO Pediatr-Res. 1978 Oct. 12(10). P 967-70. MJ AMYLASES: bl. CYSTIC-FIBROSIS: en. SALIVA: en. MN CHILD. CYSTIC-FIBROSIS: me. EATING. FECES: an. HUMAN. ISOENZYMES: bl. LIPIDS: an. PANCREAS: en. PANCREATIC-DISEASES: en. SYNDROME. AB Total serum amylase was determined in 83 patients with cystic fibrosis (CF) (14 of whom had no steatorrhea), 21 control patients, and 6 patients with Shwachman's syndrome. Patients with CF who had steatorrhea and therefore lacked pancreatic function had the same serum levels pre- and postprandially as the control subjects, whereas patients without steatorrhea and, therefore, with evidence of pancreatic function, had significantly higher levels. The patients with Shwachman's syndrome had significantly lower total serum amylase than the three other patient groups. Only the patients with Shwachman's syndrome demonstrated a significant postprandial rise in activity. Only the salivary isoenzyme was detected in patients with Shwachman's syndrome or CF and apparently absent pancreatic function, whereas CF patients without steatorrhea had both salivary and pancreatic isoenzymes. These results demonstrate that patients with CF have an increase in circulating salivary amylase isoenzyme whereas patients with Shwachman's syndrome do not. The increase probably reflects an autonomic hyperstimulation of salivary secretion which is primary to CF, and unrelated to the state of pancreatic activity. RF 001 BARBERO GJ PEDIATRICS 29 788 962 002 BARBERO GJ CF CLUB ABST 4 967 003 BLOMFIELD J AUST PAEDIATR J 10 75 974 004 BLOMFIELD J PEDIATR RES 10 574 976 005 BONGIOVANNI AM LAB INVEST 10 956 961 006 CARAWAY WT AM J CLIN PATHOL 32 97 959 007 GIBBS GE PROC SOC EXP BIOL MED 77 251 951 008 HAGLUND H METHODS BIOCHEM ANAL 19 1 971 009 JANOWITZ HD AM J MED 27 924 959 010 MANDEL ID AM J DIS CHILD 113 431 967 011 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 012 MARMAR J GASTROENTEROLOGY 50 551 966 013 NIKOLAJEK WP EUR J PEDIATR 122 289 976 014 ODONNELL MD CLIN CHEM 23 560 977 015 ROY CC PEDIATR CLIN GASTROENTEROL 975 016 RUBIN LS PEDIATRICS 38 865 966 017 SHWACHMAN H J PEDIATR 65 645 964 018 SHWACHMAN H PEDIATRICS 55 86 975 019 SKUDE G SCAND J GASTROENTEROL 10 577 975 020 SKUDE G SCAND J GASTROENTEROL 11 525 976 021 SUN DCH GASTROENTEROLOGY 45 203 963 022 TAUSSIG LM PEDIATRICS 54 229 974 023 VESTERBERG O ACTA CHEM SCAND 20 820 966 024 WOLF RO J LAB CLIN MED 87 164 976 025 WORMSLEY KG SCAND J GASTROENTEROL 4 623 969 CT 1 WOLF RO PEDIATR RES 13 1076 979 2 QUINTON PM NATURE 279 551 979 3 GILLARD BK PEDIATR RES 14 1168 980 4 ROULET M PEDIATR RES 14 1360 980 5 WOLF RO PEDIATR RES 14 968 980 6 DAVIDSON GP J CLIN PATHOL 33 390 980 7 PARK RW GASTROENTEROLOGY 81 1143 981 8 ROSCHER AA J PHARMACOL EXP THER 216 419 981 9 MITCHELL EA AUST PAEDIATR J 18 118 982 10 GOLDBERG DM CLIN PHYSIOL BIOCHEM 2 249 984 11 MORGENSTERN T MONATSSCHR KINDERHEILKD 132 661 984 12 MAILLIE AJ SCAND J GASTROENTEROL 21 941 986 13 LIU P J CAN ASSOC RADIOL 37 279 986 PN 78180 RN 00960 AN 79073920 AU Katz-S. TI Calcium and sodium transport processes in patients with cystic fibrosis. I. A specific decrease in Mg2+-dependent, Ca2+-adenosine triphosphatase activity in erythrocyte membranes from cystic fibrosis patients. SO Pediatr-Res. 1978 Nov. 12(11). P 1033-8. MJ ADENOSINE-TRIPHOSPHATASE-CALCIUM: me. CALCIUM: me. CYSTIC-FIBROSIS: en. ERYTHROCYTE-MEMBRANE: en. ERYTHROCYTES: en. MAGNESIUM: me. SODIUM: me. MN ADENOSINE-TRIPHOSPHATASE-SODIUM-POTASSIUM: me. ADOLESCENCE. ADULT. FEMALE. HUMAN. MALE. AB Calcium-ATPase activity (Mg2+-dependent Ca2+-ATPase, ATP phosphohydrolase, EC 3.6.1.3) in erythrocyte membrane preparations from cystic fibrosis (CF) patients was greatly reduced compared to erythrocyte membranes from control subjects. The Km for calcium was found to be similar in the two groups; however, the Vmax, the maximal rate of activation of the Ca2+-ATPase, is reduced by 50% in the erythrocyte membrane preparations of the CF patients (P less than 0.001). In contrast, the Mg2+-ATPase activity of erythrocyte membranes from CF patients was unchanged compared to the control subjects. No difference in the Na+,K+-ATPase activity in erythrocyte membranes from CF patients compared to control patients could be observed. This indicates that the Ca2+-ATPase activity noted in CF erythrocytes is not part of a generalized membrane or membrane-bound enzyme alteration. It remains to be determined whether this alteration in Ca2+-ATPase activity is directly related to a defect in calcium transport in these cells and is a generalized phenomenon in CF present in cell types more directly involved in secretion. RF 001 BAKER PF IN: BUTLER JAV 179 972 002 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 003 BLOMFIELD J ARCH DIS CHILD 48 267 973 004 BLOSTEIN R J BIOL CHEM 243 1957 968 005 BLOSTEIN R J BIOL CHEM 245 270 970 006 BOAT TF PEDIATR RES 8 531 974 007 BOND GH BIOCHIM BIOPHYS ACTA 323 592 973 008 CALDWELL PG IN: CUTHBERT AW 10 973 009 CHERNICK WS J PEDIATR 59 890 961 010 COLE CH PEDIATR RES 6 616 972 011 DI SANTAGNESE PA N ENGL J MED 277 1344 967 012 DOUGLAS WW BR J PHARMACOL 34 451 968 013 FEIG SA PEDIATR RES 8 594 974 014 FORSTNER JF PEDIATR RES 10 609 976 015 GIBSON LE PEDIATRICS 48 695 971 016 GLYNN IM BIOCHEM J 90 147 964 017 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 018 KATZ S CF CLUB ABST 18 9 977 019 KATZ S BIOCHIM BIOPHYS ACTA 389 314 975 020 KATZ AM ADV CYCLIC NUCLEO RES 5 453 975 021 LEE KS J GEN PHYSIOL 54 713 969 022 LOWRY OH J BIOL CHEM 193 265 951 023 MANDEL ID CLIN PEDIATR 8 161 969 024 MCEVOY FA CLIN CHIM ACTA 54 195 974 025 POST RL METHODS ENZYMOL 10 762 967 026 RUSSELL JM J GEN PHYSIOL 63 144 974 027 SCHATZMANN HJ J PHYSIOL (LOND) 235 551 973 028 WIESMANN UN CF CLUB ABST 9 12 968 029 WINS P BIOCHIM BIOPHYS ACTA 120 341 966 030 ZIPKIN I CF CLUB ABST 11 57 970 CT 1 GOPINATH RM AM J HEMATOL 7 303 979 2 ANSAH TA CELL CALC 1 195 980 3 SCANLIN TF CLIN CHEST MED 1 424 980 4 RAESS BU J PHARMACOL METHODS 4 273 980 5 DAVIS PB PEDIATR RES 14 83 980 6 ANSAH TA PROC WEST PHARMACOL SOC 23 389 980 7 KATZ S CLIN CHIM ACTA 100 245 980 8 FODER B CLIN CHIM ACTA 104 187 980 9 KATZ S CELL CALC 2 545 981 10 LARSEN FL NATURE 294 667 981 11 SORSCHER EJ LANCET 1 368 982 12 WARWICK WJ AM J MED TECHNOLOGY 48 539 982 13 REINILA M ANAL BIOCHEM 124 19 982 14 APPLEGARTH DA MED HYPOTHESES 11 277 983 15 FONDACARO JD LIFE SCI 32 1449 983 16 HARRIS A CLIN CHIM ACTA 128 41 983 17 MINER C J PHYSIOL (LOND) 338 P 63 983 18 NORDT FJ ANN NY ACAD SCI 416 651 983 19 SEYMOUR CA BIOESSAYS 1 38 984 20 KATZ S CELL CALC 5 421 984 21 BERGHOUT AGRV PEDIATR RES 18 1017 984 22 DEARBORN DG PEDIATR RES 18 890 984 23 VONRUECKER AA PEDIATR RES 18 594 984 24 WALLER RL LIFE SCI 35 775 984 25 PETTEGREW JW J MAGNET RESON 57 185 984 26 CLOUGH DL CLIN CHIM ACTA 138 259 984 27 WALLER RL CELL CALC 6 245 985 28 SUTER S PEDIATR RES 19 346 985 29 ALBAZZAZ FJ J APPL PHYSIOL 59 1191 985 30 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 31 ALLEN BG CELL CALC 7 161 986 32 BRIDGES MA PEDIATR RES 20 356 986 33 DAVIS PB PEDIATR RES 20 1290 986 34 RUPP GM MED HYPOTHESES 20 245 986 35 MORRISSEY SM DIGESTION 34 28 986 36 HUNSINGER RN CLIN CHIM ACTA 156 165 986 37 SCHONI MH CELL CALC 8 53 987 PN 78181 RN 00961 AN 78225004 AU Davis-P-B. Braunstein-M. Jay-C. TI Decreased adenosine 3':5'-monophosphate response to isoproterenol in cystic fibrosis leukocytes. SO Pediatr-Res. 1978 Jun. 12(6). P 703-7. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: bl. CYSTIC-FIBROSIS: bl. ISOPROTERENOL: pd. LEUKOCYTES: me. MN ADOLESCENCE. ADULT. ANTIBIOTICS. CYSTIC-FIBROSIS: fg. DIGOXIN. FEMALE. GRANULOCYTES: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. LEUKOCYTES: de. MALE. MIDDLE-AGE. PANCREATIC-EXTRACTS. PROSTAGLANDINS-E: pd. RECEPTORS-ADRENERGIC-BETA: ph. AB Leukocyte preparations from cystic fibrosis (CF) patients produced markedly less adenosine 3':5'-monophosphate (cAMP) in response to isoproterenol than normal cells. Both stimulation ratio and net isoproterenol-stimulated accumulation of cAMP were significantly (P < 0.001) lower in mixed leukocyte preparations from 20 CF patients (29 trials) compared to 21 normal subjects (49 trials). There was no significant correlation of results with clinical score, and no differences in the CF group attributable to medications or presence of pancreatic insufficiency. CF heterozygotes had mean stimulation ratio and mean net isoproterenol-stimulated cAMP intermediate betweem normal and CF. There were no statistically significant differences among normal subjects, CF patients, and CF heterozygotes in basal cAMP or prostaglandin E1-stimulated cAMP. Purified mononuclear cells from five normal persons and seven CF homozygotes had the same basal and prostaglandin E1-stimulated cAMP, but isoproterenol-stimulated cAMP was markedly depressed (P < 0.01) in the CF cells. Granulocytes from six normal persons and nine CF patients also had the same basal and prostaglandin E1-stimulated cAMP, but isoproterenol-stimulated cAMP was decreased (P < 0.05) in the CF samples. These results clearly demonstrate a difference between normal and CF leukocytes in cAMP response to beta-adrenergic stimulation. The diminised cAMP response to beta-adrenergic stimulation in CF is probably related to the presence of a CF gene, and may interact with other genetic abnormalities to produce the clinical syndrome of CF. RF 001 ATKINSON JP J CYCLIC NUCLEOTIDES RES 2 297 976 002 BOLANDE RP ARCH PATHOL 95 172 973 003 BOURNE HR J PHARMACOL EXP THER 178 1 971 004 BOYD EM CAN MED ASSOC J 82 821 960 005 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 006 BRAUNSTEIN M CF CLUB ABST 28 975 007 BROWN BL ADV CYCLIC NUCLEO RES 2 25 972 008 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 009 BUSSE WW J ALLERGY CLIN IMMUNOL 58 586 976 010 CAMPBELL IM PEDIATRICS 57 480 976 011 CHERNICK WS MOD PROBL PEDIATR 10 125 967 012 CHERNICK WS J PEDIATR 59 890 961 013 CONOLLY ME J CLIN INVEST 58 1307 976 015 DAVIS PB FED PROC 36 409 977 016 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 156 970 017 DI SANTAGNESE PA N ENGL J MED 295 481 976 018 ENGELHARD VH PROC NAT ACAD SCI USA 73 4482 976 019 FARBER S AM J DIS CHILD 64 953 942 021 LEFKOWITZ RJ N ENGL J MED 295 323 976 022 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 023 MARTINEZ JR PEDIATR RES 9 463 975 024 MARTINEZ JR PEDIATR RES 9 470 975 025 MCEVOY FA LANCET 2 236 975 026 MICKEY J J BIOL CHEM 250 5727 975 027 MURAD F J CLIN ENDOCRINOL METAB 40 552 975 028 PARKER DW J CLIN INVEST 52 48 973 029 PATEL KR CLIN ALLERGY 4 311 974 030 RUBIN LS PEDIATRICS 38 865 966 031 SCHAAP T IN: MANGOS JA 291 976 032 SIMOPOULOS AP PEDIATR RES 6 355 972 033 SNIDER DE J CLIN INVEST 58 524 976 034 STURGESS JM BR J EXP PATHOL 54 388 973 035 TALLMAN JF PROC NAT ACAD SCI USA 74 873 977 036 TAUSSIG LM J PEDIATR 82 380 973 037 THOMPSON FE PEDIATR RES 10 632 976 038 WILLIAMS LT J CLIN INVEST 57 149 976 039 WRIGHT R PEDIATR RES 10 373 976 040 ZENSER TV J BIOL CHEM 251 7431 976 CT 1 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 2 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 3 LAKE CR CLIN CHIM ACTA 92 141 979 4 DAVIS PB IRCS MED SCI BIOCHEM 8 413 980 5 AURBACH GD ADV CYCLIC NUCLEO RES 12 1 980 6 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 7 DAVIS PB PEDIATR RES 14 83 980 8 DAVIS PB PEDIATR RES 14 863 980 9 ROSCHER AA PEDIATR RES 14 261 980 10 AITKEN DA J MED GENET 17 187 980 11 DAVIS PB J LAB CLIN MED 96 75 980 12 LEMANSKE RF AM REV RESPIR DIS 122 213 980 13 DAVIS PB N ENGL J MED 302 1453 980 14 MARKOVAC J BIOCHEM MED 26 299 981 15 JOHNSON DL RES COMMUN CHEM PATH PHARM 32 377 981 16 GALANT SP J CLIN INVEST 68 253 981 17 LEMANSKE RF AM REV RESPIR DIS 123 622 981 18 BYLUND DB J PHARMACOL EXP THER 218 134 981 19 FRASER CM N ENGL J MED 305 1165 981 20 ROAN Y PEDIATR RES 16 591 982 21 MOTULSKY HJ N ENGL J MED 307 18 982 22 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 23 DAVIS PB J CHRON DIS 36 269 983 24 DAVIS PB J CLIN INVEST 71 1787 983 25 CABRINI G RIV ITAL PEDIATR 10 405 984 26 STESSMAN J J GERONTOL 39 667 984 27 SATO K J CLIN INVEST 73 1763 984 28 EBSTEIN RP ISR J MED SCI 21 302 985 29 MITCHELL EA AUST PAEDIATR J 21 127 985 30 ERICKSON RP AM J HUM GENET 37 124 985 31 KOPELMAN H N ENGL J MED 312 329 985 32 DAVIS PB HORM METAB RES 18 217 986 33 DAVIS PB PEDIATR RES 20 1290 986 34 BOUCHER RC J CLIN INVEST 78 1245 986 35 SCHONI MH EUR J PEDIATR 145 80 986 36 SPAIDE RF AM J OPHTHALMOL 103 204 987 PN 78182 RN 00962 AN 78225040 AU Allue-X. Sanjurjo-P. TI Triglyceride levels after Intralipid infusion in children with cystic fibrosis. SO Pediatrics. 1978 Jun. 61(6). P 924-5. MJ CYSTIC-FIBROSIS: bl. DIETARY-FATS: ad. GLYCERIN: ad. PHOSPHATIDYLCHOLINES: ad. TRIGLYCERIDES: bl. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: th. DRUG-COMBINATIONS. FATTY-ACIDS-ESSENTIAL: bl. FATTY-ACIDS-NONESTERIFIED: bl. FEMALE. HUMAN. MALE. PARENTERAL-FEEDING. EX Infusions of soybean oil emulsion (Intralipid) have been reported to correct essential fatty acid (EFA) deficiency in patients with cystic fibrosis of the pancreas. This article reports the immediate effects of Intralipid infusion in patients with CF in an attempt to ascertain through biochemical data whether the use of Intralipid at the reported dose is free of untoward effects. Six patients with CF were placed in a protocol that called for periodic infusions with soybean oil emulsion containing EFAs. The first three patients received 2 gm/kg of body weight of Intralipid over a four-hour period. The other three patients received half that dose over the same period. The first three patients developed symptoms of paleness, nausea and mild discomfort two to three hours after the infusion. Further information is needed to determine the best method of correction of EFA deficiency in children with CF. RF 001 ELLIOTT RB PEDIATRICS 57 474 976 002 TAUSSIG LM J PEDIATR 82 380 973 003 SANJURJO P LANCET 1 752 977 004 EGGSTEIN M KLIN WOCHENSCHR 44 267 966 005 CALDWELL MD J PEDIATR 81 894 972 006 SHENNAN AT J PEDIATR 91 134 977 007 PRESS M LANCET 1 597 974 008 TASHIRO T J PEDIATR SURG 10 203 975 009 FRIEDMAN Z PEDIATRICS 58 650 976 010 CHASE HP PEDIATRICS 57 441 976 CT 1 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 2 MERRITT RJ ADV NUTR RES 5 77 983 PN 78183 RN 00963 AN 78225020 AU Bureau-M-A. McDougall-D-M. Beaudry-P-H. Belmonte-M-M. TI Late effect of nocturnal mist tent therapy related to the severity of airway obstruction in children with cystic fibrosis. SO Pediatrics. 1978 Jun. 61(6). P 842-6. MJ AIRWAY-OBSTRUCTION: th. CYSTIC-FIBROSIS: co. RESPIRATORY-THERAPY. MN ADOLESCENCE. AIRWAY-OBSTRUCTION: et. CHILD. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. MALE. MAXIMAL-MIDEXPIRATORY-FLOW-RATE. AB To evaluate the long-term effect of nocturnal mist tent therapy on the progression of airway obstruction in children with cystic fibrosis (CF) of varying severity, two matched groups each consisting of 24 children with CF were studied during 18 months on mist tent therapy and 18 months of therapy. The progression in airway obstruction was measured by change in serial measurements of maximal midexpiratory flow (MMEF), from which a regression equation of MMEF against time was obtained for each individual. Changes in MMEF value with or without therapy were compared in patients matched for severity of disease as indicated by initial MMEF values. For the group as a whole no differences were found in the progression of the airway obstruction whether the patients received mist tent therapy or not. This therapy failed to benefit any of the groups of children with CF who had early, moderate, or advanced airway obstruction as judged from their initial MMEF value. It is concluded that nocturnal mist tent therapy neither decreases airway obstruction nor prevents its progression in children with CF. RF 001 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 002 CHANG N AM REV RESPIR DIS 107 672 973 003 TAUSSIG LM J PEDIATR 84 619 974 004 JEHANNE M ANN PEDIATR (PARIS) 21 595 974 005 NORMAN AP PRACTITIONER 206 786 971 006 MATTHEWS LW J PEDIATR 65 558 964 007 MATTHEWS LW PEDIATRICS 39 176 967 008 AVERY ME PEDIATRICS 39 160 967 009 WOLFSDORF J PEDIATRICS 43 799 969 010 ALDERSON PO J PEDIATR 84 479 974 011 FEATHERBY EA AM REV RESPIR DIS 102 737 970 012 BEAUDRY PH AM REV RESPIR DIS 95 248 967 013 FOX WW PEDIATRICS 54 293 974 CT 1 LOURENCO RV ARCH INTERN MED 142 2299 982 PN 78184 RN 00964 AN 78157375 AU Mellis-C-M. Levison-H. TI Bronchial reactivity in cystic fibrosis. SO Pediatrics. 1978 Mar. 61(3). P 446-50. MJ ASTHMA: co. BRONCHI: de. CYSTIC-FIBROSIS: co. HISTAMINE: pd. MN ADOLESCENCE. ADULT. AEROSOLS. AIRWAY-RESISTANCE. BRONCHI: pp. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pp. FEMALE. FORCED-EXPIRATORY-VOLUME. HISTAMINE: ad. HUMAN. MALE. MAXIMAL-MIDEXPIRATORY-FLOW-RATE. SKIN-TESTS. SPIROMETRY. AB There is considerable dispute regarding the prevalence of asthma in patients with cystic fibrosis (CF). We studied 50 patients with CF and compared their responsiveness to inhaled histamine with that of asthmatic children. The incidence of positive responses to inhaled histamine was 24% in the patients with CF and more than 90% in the asthmatic patients. We found no correlation, in the patients with CF, between the histamine response and several indexes of atopy (clinical allergic disease and positive allergen skin tests). Positive responders to histamine were not evenly distributed over the disease spectrum of CF. Instead, positive responses occurred only in those patients whose prechallenge pulmonary function was abnormal. We conclude that the heightened bronchial reactivity in patients with CF reflects the severity of their underlying lung disease rather than the presence of coexistent asthma. RF 001 COUNAHAN R ARCH DIS CHILD 50 477 975 002 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 003 WARNER JO ARCH DIS CHILD 51 507 976 004 ABBOTT V CAN MED ASSOC J 64 419 951 005 DERBES VJ J ALLERGY 28 287 957 006 LANDAU LI J PEDIATR 82 863 973 007 HEIMLICH EM J ALLERGY CLIN IMMUNOL 37 103 966 008 DAY G ARCH DIS CHILD 48 355 973 009 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 010 ITKIN IH J ALLERGY CLIN IMMUNOL 40 245 967 011 PARKER CD ARCH INTERN MED 115 452 965 012 DEKOCK MA J APPL PHYSIOL 21 185 966 013 ANON LANCET 2 691 975 014 CHAI H J ALLERGY CLIN IMMUNOL 56 323 975 015 MERCER TT ANN ALLERGY 23 314 965 016 DUBOIS AB J CLIN INVEST 35 322 956 017 WENG TR AM REV RESPIR DIS 99 879 969 018 HALUSZKA J RESPIRATION 32 217 975 019 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 020 RUBINFELD AR AM REV RESPIR DIS 115 381 977 021 DOLOVICH MB J APPL PHYSIOL 40 468 976 022 HOGG JC N ENGL J MED 278 1355 968 023 EMPEY DW AM REV RESPIR DIS 113 131 976 024 GIMENO F THORAX 29 16 974 CT 1 MITCHELL I J PEDIATR 93 744 978 2 LEUPOLD W MONOGR PAEDIATR 10 119 979 3 ZAMBIE MF ANN ALLERGY 42 290 979 4 LARSEN GL AM REV RESPIR DIS 119 399 979 5 NELSON LA AM REV RESPIR DIS 120 863 979 6 BURDON JGW MED J AUST 2 77 980 7 SLY PD AUST PAEDIATR J 16 205 980 8 VANASPEREN PP AUST PAEDIATR J 16 53 980 9 TOBIN MJ THORAX 35 807 980 10 TABACHNIK E AM REV RESPIR DIS 122 97 980 11 LARSEN GL AM J DIS CHILD 134 1143 980 12 PULLAN CR BR MED J 280 364 980 13 DOLOVICH J THORAX 36 641 981 14 HOLZER FJ ARCH DIS CHILD 56 455 981 15 FANTA CH MED CLIN NORTH AM 65 473 981 16 FREEDMAN PM J ALLERGY CLIN IMMUNOL 67 59 981 17 SMYTH JA PEDIATRICS 68 336 981 18 LEMANSKE RF AM REV RESPIR DIS 123 622 981 19 BECHTEL JJ AM REV RESPIR DIS 124 759 981 20 VANASPEREN P AM J DIS CHILD 135 815 981 21 MYERS JR JAMA 246 225 981 22 GEORGITIS JW ANN ALLERGY 48 175 982 23 EIGEN H PEDIATRICS 70 698 982 24 PULLAN CR BR MED J 284 1665 982 25 NADEL JA ADV INTERN MED 28 207 983 26 EASTON JG ANN ALLERGY 50 171 983 27 NELSON HS ANN ALLERGY 51 488 983 28 PRATTER MR CHEST 84 42 983 29 LEE DA BR MED J 286 1256 983 30 MOK JYQ ARCH DIS CHILD 59 299 984 31 PEARLMAN DS AM J DIS CHILD 138 459 984 32 HAIDER SA BR MED J 289 109 984 33 RUBIN BK PEDIATR INFECT DIS 4 88 985 34 DAVIS PB SEM RESPIR MED 6 261 985 35 WONNE R CLIN ALLERGY 15 455 985 36 MAPP CE ANN ALLERGY 54 424 985 37 MITCHELL I ANN ALLERGY 54 233 985 38 KONIG P ANN ALLERGY 55 95 985 39 RIVLIN J EUR J RESPIR DIS 67 286 985 40 CROPP GJA CHEST 87 S 55 985 41 ENARSON DA CHEST 87 452 985 42 HORDVIK NL AM REV RESPIR DIS 131 889 985 43 DARGA LL PEDIATR PULMONOL 2 82 986 44 CHAY OM AUST NZ J MED 16 644 986 45 MELILLO G EUR J RESPIR DIS 69 282 986 46 PIEDRA P J PEDIATR 108 817 986 PN 78185 RN 00965 AN 79032974 AU Rimsza-M-E. Hernried-L-S. Kaplan-A-M. TI Hemorrhagic retinopathy in a patient with cystic fibrosis. SO Pediatrics. 1978 Sep. 62(3). P 336-8. MJ CYSTIC-FIBROSIS: co. RETINAL-HEMORRHAGE: et. MN ACUTE-DISEASE. ADULT. ALTITUDE. ANOXIA: co. CASE-REPORT. FEMALE. HUMAN. RESPIRATORY-FUNCTION-TESTS. RISK. VISION-DISORDERS: et. AB A 21-year-old woman with a history of chronic lung disease secondary to cystic fibrosis (CF) developed an acute hemorrhagic retinopathy during exposure to moderately high altitude. Although retinal hemorrhages are known to occur in patients with CF, we speculate that the retinopathy in this case was partially related to altitudinal change and that patients with chronic hypoxemia may be predisposed to high-altitude retinopathy at much lower altitudes. RF 001 BRUCE GM ARCH OPHTHALMOL 63 391 960 002 SPALTER HF J PEDIATR OPHTHALMOL 8 6 971 003 CHAZAN BI J PEDIATR 77 86 970 004 WONG VG AM J OPHTHALMOL 59 763 965 005 LIETMAN PA JAMA 189 924 964 006 COCKE JG JR J PEDIATR 68 27 966 007 STRAUSS RG PEDIATRICS 43 297 969 008 HOUSTON CS LANCET 2 758 975 009 SINGH I N ENGL J MED 280 175 969 010 FRAYSER R N ENGL J MED 282 1183 970 011 SHULTS WT ARCH OPHTHALMOL 93 404 975 012 RENNIE D ARCH OPHTHALMOL 93 395 975 CT 1 BOLDER PM ANESTHESIOLOGY 61 595 984 2 SPAIDE RF AM J OPHTHALMOL 103 204 987 PN 78186 RN 00966 AN 78094640 AU Gilmore-J-P. Davis-M. Gibbs-G-E. TI Influence of cystic fibrotic and heterozygous serum on rat jejunum. SO Proc-Soc-Exp-Biol-Med. 1978 Jan. 157(1). P 70-4. MJ CYSTIC-FIBROSIS: bl. SODIUM: me. MN BIOLOGICAL-TRANSPORT. CYSTIC-FIBROSIS: di, fg. HETEROZYGOTE. HUMAN. JEJUNUM: me. MEMBRANE-POTENTIALS. RATS. EX The rat jejunum bioassay has been shown to be capable of identifying both the cystic fibrotic homozygote and heterozygote and therefore would appear to have great potential as a screening test for the latter. It is suggested that the cystic fibrosis factor does not influence sodium reabsorption by inhibiting an active sodium reabsorption mechanism but rather by inhibiting passive sodium entry into the cell. RF 001 WOOD RE LANCET 2 1452 973 002 MANGOS JA PEDIATR RES 1 436 967 003 ARAKI H PEDIATR RES 9 932 975 004 ARAKI H PROC INT CF CONG 7TH 28 976 005 USSING HH ACTA PHYSIOL SCAND 61 484 964 006 ARMITAGE P STATISTICAL METHODS IN MEDICA 971 007 KAISER D PEDIATR RES 5 167 971 CT 1 TUCKER RD PEDIATR RES 13 1371 979 2 WILL PC PEDIATR RES 13 1129 979 3 TUCKER RD IEEE TRANS BIOMED ENG 26 607 979 PN 78187 RN 00967 AN 78179933 AU Katz-S. TI Calcium and sodium transport processes in patients with cystic fibrosis 2. Mg2+- dependent, Ca2+ ATPase activity in fibroblast membrane preparations from cystic fibrosis patients and controls. SO Res-Commun-Chem-Pathol-Pharmacol. 1978 Mar. 19(3). P 491-503. MJ ADENOSINE-TRIPHOSPHATASE: me. CALCIUM: me. CYSTIC-FIBROSIS: me. SODIUM: me. MN ADOLESCENCE. CELL-MEMBRANE: en. CELLS-CULTURED. CHILD. FIBROBLASTS: en, ul. HUMAN. MAGNESIUM: me, pd. AB Mg2+-dependent Ca2+-ATPase activity was determined in membrane preparations of fibroblasts grown from skin biopsies of cystic fibrosis patients and age-matched controls. This enzyme was stimulated by increasing free calcium concentrations with an apparent Kdiss for calcium of approximately 45 micron. Although there was a great deal of variation in Ca2+-ATPase activity observed between individual strains, there was a significant decrease in the maximal activation of the Ca2+-ATPase in membrane preparations of fibroblasts obtained from cystic fibrosis patients compared to the controls (P less than 0.05). This observation indicates that decreased Ca2+- ATPase activity is a generalized phenomenon in cystic fibrosis found in more than one cell-type. This decrease in Ca2+-ATPase activity may have a number of implications that may explain some of the manifestations of the disease. RF 001 BAKER PF IN: BUTLER JAV 179 972 002 BARRANCO SC J CELL PHYSIOL 88 33 976 003 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 004 BLOMFIELD J ARCH DIS CHILD 48 267 973 005 BLOSTEIN R J BIOL CHEM 243 1957 968 006 BOAT TF PEDIATR RES 8 531 974 007 BOND GH BIOCHIM BIOPHYS ACTA 323 592 973 008 CALDWELL PG IN: CUTHBERT AW 10 973 009 CHERNICK WS J PEDIATR 59 890 961 010 DANES BS J EXP MED 129 775 969 011 DI SANTAGNESE PA N ENGL J MED 277 1344 967 012 DOUGLAS WW BR J PHARMACOL 34 451 968 013 FLETCHER DS CLIN CHIM ACTA 44 5 973 014 FORSTNER JF PEDIATR RES 10 609 976 015 GIBSON LE PEDIATRICS 48 695 971 016 GLYNN IM BIOCHEM J 90 147 964 017 KATZ S CF CLUB ABST 18 9 977 018 KATZ S BIOCHIM BIOPHYS ACTA 389 314 975 019 KROOTH RS MED CLIN NORTH AM 53 795 969 020 LEWIS AE BIOSTATISTICS 966 021 LOWRY OH J BIOL CHEM 193 265 951 022 MANDEL ID CLIN PEDIATR 8 161 969 023 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 024 POST RL METHODS ENZYMOL 10 762 967 025 QUISSELL DO NATURE 247 115 974 026 ROUFOGALIS BD BIOCHIM BIOPHYS ACTA 318 360 973 027 RUSSELL JM J GEN PHYSIOL 63 144 974 028 SCHATZMANN HJ J PHYSIOL (LOND) 235 551 973 029 SHIGEKAWA M J BIOL CHEM 251 6984 976 CT 1 ANSAH TA CELL CALC 1 195 980 2 ANSAH TA PROC WEST PHARMACOL SOC 23 389 980 3 THEOHARIDES TC LIFE SCI 27 703 980 4 KATZ S CLIN CHIM ACTA 100 245 980 5 FODER B CLIN CHIM ACTA 104 187 980 6 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 7 GNEGY ME BIOCHEM MED 26 294 981 8 SORSCHER EJ LANCET 1 368 982 9 HARRIS A CLIN CHIM ACTA 128 41 983 10 KATZ S CELL CALC 5 421 984 11 ALLEN BG CELL CALC 7 161 986 12 BRIDGES MA PEDIATR RES 20 356 986 13 MORRISSEY SM DIGESTION 34 28 986 PN 78188 RN 00968 AN 79014649 AU Super-M. TI Cystic fibrosis in Southern Africa. Including the preparation of a register of carriers and potential carriers. SO S-Afr-Med-J. 1978 Jul 1. 54(1). P 18-21. MJ CYSTIC-FIBROSIS: oc. MN CHILD. CYSTIC-FIBROSIS: fg. FEMALE. HUMAN. MALE. MEDICAL-RECORDS-PROBLEM-ORIENTED. NAMIBIA. ZIMBABWE. SOUTH-AFRICA. WHITES. AB Little has been published on cystic fibrosis (CF) in Whites in southern Africa, and no figures as to incidence exist. A register of CF patients, their parents (obligatory carriers), siblings, uncles, aunts and first cousins (potential carriers) has been compiled for southern Africa. The degree of co-operation shown by colleagues and by families whose addresses have been provided by them, and possible reasons for non-co-operation are discussed. From the numbers and birth dates of patients a rough estimate of the incidence in the Republic of South Africa, South West Africa and Rhodesia has been made. In all three regions, but especially in South Africa, incidence is likely to have been underestimated. Details available from the register include the number of CF patients alive and dead, those who presented with meconium ileus, the number of affected patients per family, consanguinity among the parents or grandparents, the frequency with which identical surnames were encountered, and the sibship sizes of all those on the register. Towns and districts with a population rich in the CF gene are mentioned. The number of potential carriers has been determined, so that they can be screened when a practicable detection test is devised. The register has answered a number of questions about CF in southern Africa. It has focused attention on the disease in the region and played a major catalytic role in the formation of the Southern African Cystic Fibrosis Association. RF 001 GROVE SS S AFR J LAB CLIN MED 5 113 959 002 LEVIN SE S AFR MED J 41 482 967 003 SUPER M S AFR MED J 49 818 975 004 SUPER M S AFR MED J 50 238 976 005 BARBERO GJ IN: MANGOS JA 83 976 006 MACKENZIE DJM S AFR MED J 50 2083 975 007 CARTER CO ABC OF MEDICAL GENETICS 969 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 009 BOTHA MC S AFR MED J SUPPL 1 46 22 972 010 SUPER M LANCET 2 1288 977 011 SUPER M THESIS 978 012 OLIVIER SP PIONIERTREKKE NA GAZALAND 59 943 013 MEYER ET IN: GEDENKBOEK VAN DIE DORSLA 5 974 014 EMERY AEH ELEMENTS OF MEDICAL GENET 103 975 CT 1 SUPER M MONOGR PAEDIATR 10 106 979 2 SUPER M LANCET 2 1200 983 3 BOTHA MC S AFR MED J 64 609 983 PN 78189 RN 00969 AN 79159331 AU Kollberg-H. Mossberg-B. Afzelius-B-A. Philipson-K. Camner-P. TI Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function. SO Scand-J-Respir-Dis. 1978. 59(6). P 297-306. MJ BRONCHIAL-DISEASES: di. CILIA. CYSTIC-FIBROSIS: di. LUNG-DISEASES-OBSTRUCTIVE: et. MN ADULT. BRONCHI: pp. BRONCHIAL-DISEASES: pa, pp. CILIA: ph, ul. CLINICAL-TRIALS. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pa, pp. DOUBLE-BLIND-METHOD. FEMALE. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: pa, pp. MALE. RESPIRATORY-FUNCTION-TESTS. RESPIRATORY-TRACT-INFECTIONS: pa, pp. SPIROMETRY. SYNDROME. TERBUTALINE: tu. AB Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of cilia. The results were compared with those of patients with congenitally immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all the seven CF patients who succeeded in inhaling the test aerosol. Ciliary ultrastructure from a deceased CF patient was normal. Patients with the immotile cilia syndrome had no substantial clearance and defective cilia. The CF patients coughed more during the clearance measurements than any other group studied earlier, and their coughing was effective. One patient succeeded in avoiding coughing in both measurements and had faster clearance when he got terbutaline than when he got the vehicle. Although younger, the CF patients tended to be more obstructed in their lungs and more handicapped than the patients suffering from the immotile-cilia syndrome. The latter patients had more discomfort from rhinitis, sinusitis and otitis than had the CF patients. An impairment of the mucociliary transport rate is hence unlikely to be a primary pathogenic factor for the respiratory tract disease in CF patients. RF 001 AFZELIUS BA SCIENCE 193 317 976 002 BERGLUND E ACTA MED SCAND 173 185 963 003 CAMNER P ENVIRON PHYSIOL 1 137 971 004 CAMNER P SCAND J RESPIR DIS 54 272 973 005 CAMNER P AM REV RESPIR DIS 108 131 973 006 CAMNER P AM REV RESPIR DIS 112 807 975 007 CAMNER P ARCH ENVIRON HEALTH 31 79 976 008 ELIASSON R N ENGL J MED 297 1 977 009 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 010 GIBSON LE PEDIATRICS 23 545 959 011 JARSTRAND C AM REV RESPIR DIS 110 415 974 012 KARTAGENER M BEITR KLIN TUBERK 83 489 933 013 KOLLBERG H THESIS 974 014 MOSSBERG B SCAND J RESPIR DIS 57 281 976 015 MOSSBERG B SCAND J RESPIR DIS 57 119 976 016 MOSSBERG B SCAND J RESPIR DIS 59 1 978 017 MOSSBERG B SCAND J RESPIR DIS 59 55 978 018 PEDERSEN H NATURE 262 494 976 019 PHILIPSON K J AEROLOL SCI 4 51 977 021 SANCHIS J N ENGL J MED 288 651 973 022 DI SANTAGNESE PA N ENGL J MED 277 1287 967 023 DI SANTAGNESE PA N ENGL J MED 295 481 976 024 SHWACHMAN H AM J DIS CHILD 96 6 958 025 THOMSON ML N ENGL J MED 289 749 973 026 WOOD RE AM REV RESPIR DIS 111 733 975 027 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 AFZELIUS B BR MED J 2 674 979 2 PAVIA D CLIN RESP PHYSIOL 16 335 980 3 AFZELIUS BA THORAX 35 401 980 4 CAMNER P CLIN SCI 59 79 980 5 MOSSBERG B EUR J RESPIR DIS 61 8 980 6 MOSSBERG B EUR J RESPIR DIS 61 51 980 7 MOSSBERG B EUR J RESPIR DIS 61 18 980 8 MOSSBERG B EUR J RESPIR DIS 61 47 980 9 RUTLAND J THORAX 36 654 981 10 TEGNER H ACTA PAEDIATR SCAND 70 629 981 11 AFZELIUS BA AM REV RESPIR DIS 124 107 981 12 CORKEY CWB AM REV RESPIR DIS 124 544 981 13 KONRADOVA V EUR J RESPIR DIS 63 516 982 14 MOSSBERG B EUR J RESPIR DIS 63 111 982 15 YARNAL JR POSTGRAD MED 71 195 982 16 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 17 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 18 FERGUSON JA AM PHARM 23 48 983 19 MOSSBERG B EUR J RESPIR DIS 64 129 983 20 NEWHOUSE MT EUR J RESPIR DIS 64 151 983 21 RUTLAND J AM REV RESPIR DIS 128 1030 983 22 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 23 KATZ S CELL CALC 5 421 984 24 NEWHOUSE MT SEM RESPIR MED 5 341 984 25 WOOD RE SEM RESPIR MED 5 336 984 26 CAMNER P ATEMWEGS LUNGENKRANKH 10 237 984 27 CANET E PRESSE MED 13 1607 984 28 BECKER B RESPIRATION 46 180 984 29 ROSSMAN CM AM REV RESPIR DIS 129 161 984 30 HANDELSMAN DJ N ENGL J MED 310 3 984 31 AFZELIUS BA CRC CRIT REV BIOCHEM 19 63 985 32 SATO T EXP MOL PATH 43 13 985 33 KOHLER D ATEMWEGS LUNGENKRANKH 12 358 986 34 WILSON R THORAX 41 453 986 35 SVARTENGREN M EUR J RESPIR DIS 68 267 986 36 KOHLER D EUR J RESPIR DIS 69 319 986 37 MOSSBERG B EUR J RESPIR DIS 69 295 986 38 WILSON R J INFECT DIS 153 376 986 39 ZWAS ST J NUCL MED 28 161 987 40 SYKES DA THORAX 42 256 987 PN 78190 RN 00970 AN 78096506 AU Vaughan-W-J. Lindgren-F-T. Whalen-J-B. Abraham-S. TI Serum lipoprotein concentrations in cystic fibrosis. SO Science. 1978 Feb 17. 199(4330). P 783-6. MJ CYSTIC-FIBROSIS: bl. LIPOPROTEINS: bl. MN ADOLESCENCE. ADULT. CARRIER-STATE: bl. CHILD. CHILD-PRESCHOOL. ELECTROPHORESIS-AGAR-GEL. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FEMALE. HUMAN. LIPOPROTEINS-HDL: bl. LIPOPROTEINS-LDL: bl. LIPOPROTEINS-VLDL: bl. MALE. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB Two major classes of lipoproteins, low density and high density, are decreased in the serum of patients with cystic fibrosis; major apoproteins are also decreased. Since essential fatty acids and certain fat-soluble vitamins depend on lipoproteins for transport in the serum, knowledge of lipoprotein levels in cystic fibrosis patients could prove valuable in understanding (i) the basis for the abnormally low serum levels of these fatty acids and vitamins and (ii) the effects of therapies involving these molecules. RF 001 DANKS DM ANN HUM GENET 28 323 965 002A DI SANTAGNESE PA N ENGL J MED 295 481 976 002B DI SANTAGNESE PA N ENGL J MED 295 597 976 003 WIESE HF AM J CLIN NUTR 18 155 966 003 KUO PT J CLIN INVEST 44 1924 965 004 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 004 ROSENLUND ML NATURE 251 719 974 005 LOBECK CC IN: STANBURY JB 1605 972 006 MCCORMICK EC J LIPID RES 1 221 960 006 KRINSKY NI ARCH BIOCHEM BIOPHYS 73 233 958 006 KUTSKY RJ HANDBOOK OF VITAMINS AND HORM 36 973 006 SKIPSKI VP IN: NELSON GJ 471 972 009 FERRO-LUZZI AMES G J BIOL CHEM 249 634 974 009 VAUGHAN WJ GERONTOLOGY 23 110 977 010 WEBER K J BIOL CHEM 244 4406 969 011 MORRISETT JD ANNU REV BIOCHEM 44 879 975 012 LINDGREN FT IN: NELSON GJ 181 972 014 HATCH FT J LAB CLIN MED 81 946 973 017 DYERBERY J SCAND J CLIN LAB INVEST 31 473 973 017 SRINIVASAN SR CIRCULATION 54 309 976 018 LOPEZ-S A AM J CLIN NUTR 20 808 967 018 JOHNSON BC J CHRON DIS 18 147 965 019 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 021 WILSON DE J CLIN INVEST 51 1051 972 022 THOMPSON GR CLIN SCI MOL MED 45 583 973 024 SMITH FR J LAB CLIN MED 80 423 972 025 HOLVEY DN MERCK MANUAL OF DIAGNOSIS 739 972 026 KESSLER JI J BIOL CHEM 245 5281 970 026 GLICKMAN RM J CLIN INVEST 52 2910 973 026 GLICKMAN RM SCIENCE 193 1254 976 027 ELLIOTT RB PEDIATRICS 57 474 976 027 ELLIOTT RB ARCH DIS CHILD 50 76 975 027 ROSENLUND ML PEDIATRICS 59 428 977 CT 1 CHASE HP J PEDIATR 95 337 979 2 YI PI BLOOD 57 1055 981 3 PARK RW GASTROENTEROLOGY 81 1143 981 4 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 5 KRAUSS RM MED CLIN NORTH AM 66 403 982 6 SCHONI M PEDIATR RES 18 66 984 7 ABMAN SH J PEDIATR GASTROENTEROL NUTR 5 393 986 PN 78191 RN 00971 AN 78204217 AU Breslow-J-L. Epstein-J. Fontaine-J-H. Forbes-G-B. TI Enhanced dexamethasone resistance in cystic fibrosis cells: potential use for heterozygote detection and prenatal diagnosis. SO Science. 1978 Jul 14. 201(4351). P 180-2. MJ CYSTIC-FIBROSIS: pp. DEXAMETHASONE: to. MN AMNIOTIC-FLUID: cy. CELL-SURVIVAL: de. CYSTIC-FIBROSIS: di, fg. DRUG-RESISTANCE. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. PREGNANCY. PRENATAL-DIAGNOSIS. SUPPORT-U-S-GOVT-P-H-S. AB Cultured skin fibroblasts from patients with cystic fibrosis (CF) are more resistant to dexamethasone toxicity than are normal cells. We now report that, when fibroblasts cultured from obligate CF heterozygotes are exposed to dexamethasone, they have an intermediate survival compared to normal and homozygous CF cells. When dexamethasone survival was tested on cells from four patients undergoing amniocentesis, cells from a woman at risk of producing a child with CF showed significant dexamethasone resistance, similar to that of fibroblasts derived from lnown CF homozygotes; the other amniotic cell specimens showed dexamethasone sensitivity similar to that of normal skin fibroblasts. These data suggest that the dexamethasone resistance previously observed in skin fibroblasts may also be useful in the prenatal diagnosis of CF. RF 001 ANON J PEDIATR 88 711 976 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 004 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 005 BRESLOW JL CELL 13 663 978 006 BRESLOW JL EXP CELL RES 110 399 977 008 FREUND JE MODERN ELEMENTARY STATISTICS 967 CT 1 HOSLI P LANCET 2 543 979 2 MAHONEY MJ CLIN PERINATOL 6 255 979 3 KURNIT DM ANNU REV GENET 13 235 979 4 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 5 SHWACHMAN H J PEDIATR 95 661 979 6 HOSLI P FEBS LETTERS 104 271 979 7 KURZ JB SCIENCE 206 1317 979 8 SCANLIN TF CLIN CHEST MED 1 424 980 9 DAVIS PB PEDIATR RES 14 83 980 10 TARNOKY AL J ROY SOC MED 73 73 980 11 BARDON A CLIN CHIM ACTA 101 17 980 12 BRESLOW JL SCIENCE 207 1007 980 13 DANIEL A HUM GENET 57 96 981 14 KELLER GH ARCH BIOCHEM BIOPHYS 211 321 981 15 BRESLOW JL N ENGL J MED 304 1 981 16 LITTLEFIELD JW N ENGL J MED 304 44 981 17 SEALE TW ANN CLIN LAB SCI 12 415 982 18 KHALID BAK CLIN ENDOCRINOL 18 407 983 19 KHALID BAK MOL CELL ENDOCRINOL 30 303 983 20 LANGHOFF E PEDIATR RES 18 488 984 21 NELSON MM S AFR MED J 66 688 984 22 GROSS NJ AM REV RESPIR DIS 129 805 984 23 BOUE A PRESSE MED 14 575 985 24 POLANSKY J INVEST OPHTHALMOL VIS SCI 26 805 985 25 RAVIA Y HUM GENET 71 294 985 26 BOUE A HUM GENET 74 288 986 27 KUZEMKO JA J ROY SOC MED 79 2 986 PN 78192 RN 00972 AN 78204186 AU Rosenblum-M-G. Durie-B-G. Beckerman-R-C. Taussig-L-M. Russell-D-H. TI Cystic fibrosis: decreased conjugation and excretion of [14C]spermidine. SO Science. 1978 Jun 30. 200(4349). P 1496-7. MJ CYSTIC-FIBROSIS: me. SPERMIDINE: me. MN HUMAN. SPERMIDINE: bl, ur. SUPPORT-U-S-GOVT-P-H-S. AB Free and conjugated [14C]spermidine were measured in plasma samples from normal individuals and cystic fibrosis patients. Within 4 minutes, the 14C-labeled material in the plasma from normal individuals was 70 percent conjugated compared to no detectable conjugation by cystic fibrosis patients. Further, the patients excreted only 11 to 13 percent of the [14C]spermidine in their urine within 72 hours whereas normal excretion was 60 to 76 percent. In both cases, the labeled material was in a conjugated form. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 DI SANTAGNESE PA PEDIATRICS 12 549 953 003 BOWMAN BH SCIENCE 164 325 969 003 TAUSSIG LM IN: KELLEY VC 4 1 976 004 LUNDGREN DW CLIN CHIM ACTA 62 357 975 005 COHEN LF BLOOD 48 469 976 007 RUSSELL DH CLIN CHEM 23 22 977 008 ROSENBLUM MG CANCER RES 37 47 977 009 ROSENBLUM MG PROC AM ASSOC CANCER RES 17 15 976 010 SEILER N NEUROCHEM RES 1 469 976 CT 1 ROSENBLUM MG CANCER RES 38 3161 978 2 CAMPBELL RA LANCET 1 724 979 3 RUSSELL DH PEDIATR RES 13 1137 979 4 CANELLAKIS ES CURR TOP CELL REGUL 15 155 979 5 AIGNERHELD R PROC NAT ACAD SCI USA 76 6652 979 6 AIGNERHELD R PHYSIOL CHEM PHYS 12 389 980 7 MELVIN MAL PHYSIOL CHEM PHYS 12 431 980 8 BAYLIN SB PEDIATR RES 14 921 980 9 SWENDSEID ME LIFE SCI 26 533 980 10 THEOHARIDES TC LIFE SCI 27 703 980 11 BARDON A CLIN CHIM ACTA 101 17 980 12 SHIPE JR CRC CRIT REV CLIN LAB SCI 16 1 981 13 FAN MZ PHYSIOL CHEM PHYS MED NMR 15 57 983 14 FAN MZ PHYSIOL CHEM PHYS MED NMR 15 69 983 15 TAKAGI T J CHROMATOGR 272 279 983 16 NORDLANDER JE J ORGANIC CHEM 49 133 984 PN 78193 RN 00973 AN 78250613 AU Epstein-J. Breslow-J-L. Fitzsimmons-M-J. Vayo-M-M. TI Pleiotropic drug resistance in cystic fibrosis fibroblasts: increased resistance to cyclic AMP. SO Somatic-Cell-Genet. 1978 Jul. 4(4). P 451-64. MJ CYSTIC-FIBROSIS: pp. DIBUTYRYL-CYCLIC-AMP: pd. MN CELL-LINE. CELL-SURVIVAL: de. DRUG-RESISTANCE. ISOPROTERENOL: pd. SUPPORT-U-S-GOVT-P-H-S. 3-5-CYCLIC-AMP-PHOSPHODIESTERASE: me. AB In previous studies, cystic fibrosis (CF) fibroblasts were demonstrated to be resistant to the cytotoxic effects of ouabain, dexamethasone, and the sex hormones, dihydrotestosterone, 17beta- estradiol, and progesterone. We now show that CF fibroblasts also exhibit greatly increased resistance to the cytotoxic effects of exogenous dibutyryl cyclic AMP (cAMP), as well as to isoproterenol and theophylline, drugs which are known to increase endogenous levels of cAMP. CF cells were also shown to have normal amounts of (3H)cAMP binding to protein kinase as well as normal amounts of cAMP- stimulated protein kinase activity. Phosphodiesterase in CF cells was also found to be stimulated by cAMP to the same degree as in normal cells. These findings suggest that there is no detectable protein kinase deficiency in CF cells. cf cells thus appear to be unlike some cAMP-resistant mutants described by others which are defective in protein kinase activity and cAMP regulation of phosphodiesterase levels. The cross-resistance of CF fibroblasts to ouabain, steroid hormones, and cAMP may provide a unique opportunity to study the biochemical events involved in the metabolism of these drugs as well as the basic biochemical defect in a common human genetic disease. RF 001 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 002 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 003 BRESLOW JL CELL 13 663 978 004 BRESLOW JL EXP CELL RES 110 399 977 005 LELIEVRE L EXP CELL RES 104 191 977 006 LOWRY OH J BIOL CHEM 193 265 951 007 INSEL PA SCIENCE 190 896 975 008 GILMAN AG PROC NAT ACAD SCI USA 67 305 970 009 BEAVO JA J BIOL CHEM 245 5649 970 010 MANGANIELLO VC BIOCHIM BIOPHYS ACTA 362 509 974 011 APPLEMAN MM ADV CYCLIC NUCLEO RES 3 65 973 012 KUO JF PROC NAT ACAD SCI USA 64 1349 969 013 ERLICHMAN J J BIOL CHEM 248 7607 973 014 BEAVO JA IN: DRUMMOND P 5 241 975 015 DANIEL V PROC NAT ACAD SCI USA 70 76 973 016 COFFINO P J CELL PHYSIOL 85 603 975 017 BOURNE HR J CELL PHYSIOL 85 611 975 018 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 019 BOURNE HR SCIENCE 181 952 973 020 D ARMIENTO M PROC NAT ACAD SCI USA 69 459 972 021 MAGANIELLO V PROC NAT ACAD SCI USA 69 269 972 022 THOMPSON EB METABOLISM 23 159 974 023 MANKOVITZ R CELL 3 221 974 024 LEVER JE J CELL PHYSIOL 88 343 976 025 BAXTER JD SCIENCE 171 189 971 026 SIBLEY CH CELL 2 221 974 027 YAMAMOTO KR IN: GREEP RO 32 3 976 028 BOURGEOIS S CELL 11 423 977 029 BOURNE HR SCIENCE 187 750 975 030$ GRANNER DK NATURE 259 572 974 031 SCHAFFER LD BIOCHIM BIOPHYS ACTA 192 304 969 032 MILLER TB J BIOL CHEM 246 3672 971 033 AXTON JH J BIOL CHEM 247 3579 972 034 STELLWAGEN RH BIOCHEM BIOPHYS RES COMMUN 47 1144 972 035$ VAN RIJN H J BIOL CHEM 60 181 974 036 LEMAIRE I CELL 11 149 977 037 MOMMAERTS WFHM PROC NAT ACAD SCI USA 45 791 959 038 HUG G J CLIN INVEST 48 704 969 039 HUIJING F AM J HUM GENET 21 275 969 CT 1 KURZ JB SCIENCE 206 1317 979 2 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 3 DAVIS PB PEDIATR RES 14 83 980 4 DAVIS PB PEDIATR RES 14 863 980 5 BRESLOW JL SCIENCE 207 1007 980 6 GNEGY ME BIOCHEM MED 26 294 981 7 MARKOVAC J BIOCHEM MED 26 299 981 8 BRESLOW JL N ENGL J MED 304 1 981 9 UNSICKER K CELL TISSUE RES 223 73 982 10 LANGHOFF E PEDIATR RES 18 488 984 11 PINSKY L ADV HUM GENET 16 299 987 PN 78194 RN 00974 AN 78204429 AU Carpentieri-U. Gustavson-L-P. Haggard-M-E. TI Misdiagnosis of neglect in a child with bleeding disorder and cystic fibrosis. SO South-Med-J. 1978 Jul. 71(7). P 854-5. MJ CHILD-ABUSE. CYSTIC-FIBROSIS: co. DISSEMINATED-INTRAVASCULAR-COAGULATION: et. VITAMIN-K-DEFICIENCY: co. MN CASE-REPORT. CYSTIC-FIBROSIS: di. DIAGNOSTIC-ERRORS. HUMAN. INFANT. MALE. AB This report describes a child in whom a bleeding disorder secondary to vitamin K deficiency led to a mistaken diagnosis of child neglect, which was eventually corrected to that of cystic fibrosis. RF 001 WALTERS TR AM J DIS CHILD 124 641 972 002 TORSTENSON OL PEDIATRICS 45 857 970 003 OPPENHEIMER EH J PEDIATR 88 1079 976 CT 1 OHARE AE ARCH DIS CHILD 59 860 984 2 PAYNE NR PEDIATRICS 73 712 984 3 LANE PA J PEDIATR 106 351 985 PN 78195 RN 00975 AN 79077519 AU Lewis-R-W. TI The biochemical basis of cystic fibrosis: an hypothesis based upon the polyelectrolytes of mucus. SO Tex-Rep-Biol-Med. 1978. 36. P 33-8. MJ CYSTIC-FIBROSIS: me. ELECTROLYTES: me. MUCUS: me. MN CYSTIC-FIBROSIS: pp. HUMAN. MEMBRANE-PROTEINS: me. MEMBRANES: me. MUCUS: se. VISCOSITY. AB The evidence shows that the pathology of cystic fibrosis (CF) is related to mucoid secretions. Consequently, the question of what governs the viscosity of mucus is examined in detail. It is generally agreed that the mucin gel consists of polyanionic glycoproteins and a hitherto unidentified poly cationic cross-linking protein. It is proposed that larger amounts of polycations increase viscosity and, in excess, cause mucus to become a polycation. As these compounds are known to inhibit the ductal reabsorption of sodium, the two principal aspects of the pathology of CF can be related to the same mechanism. The mucus globule membrane (MGM) has been proposed as the cross- linking protein. RF 001 BARBERO GJ PEDIATRICS 22 945 958 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 BOTELHO SY J PEDIATR 83 601 973 004 CARLBORG L ACTA ENDOCRINOL 62 721 969 005 COOK GMW SURFACE CARBOHYDRATES OF THE 58 973 006 DI SANTAGNESE PA PEDIATRICS 12 549 953 007 DISCHE Z FED PROC 19 904 960 008 DISCHE Z PEDIATRICS 24 74 959 009 ELSTEIN M J OBSTET GYNAECOL BR COMMONW 77 443 970 010 FARBER S J MICH MED SOC 44 587 945 011 GIBBONS RA BIOCHEM J 73 209 959 012 GIBBONS RA PROTIDES BIOL FLUIDS 16 299 968 013 IACOBELLI S FERTIL STERIL 22 727 971 014 JIRKA M CLIN CHIM ACTA 2 292 957 015 JOHNSTON WH ARCH DIS CHILD 31 477 956 016 KAISER D PEDIATR RES 5 167 971 017 KIM SK J ULTRASTRUCT RES 38 371 972 018 KORNGUTH SE EXP CELL RES 24 484 961 020 LEWIS RW LIPIDS 8 321 973 021 MANDEL ID AM J DIS CHILD 110 646 965 022 MANGOS JA PEDIATR RES 2 378 968 023 MAMELAK M J MEMBR BIOL 1 144 969 024 MASSON PL IN: ELSTEIN M 82 973 025 NEUTRA M J CELL BIOL 30 119 966 026 PALLAVICINI JC ANN NY ACAD SCI 106 330 963 027 POTTER JL ANN NY ACAD SCI 106 692 963 028 SCHUMACHER GFB PROTIDES BIOL FLUIDS 16 525 968 029 SHWACHMAN H PEDIATRICS 7 153 951 030 ROELFS RE AM J DIS CHILD 113 419 967 031 TANDLER B J MORPHOL 127 383 969 CT 1 PUCHELLE E EUR J CLIN INVEST 15 389 985 PN 78196 RN 00976 AN 78204754 AU Picot-R. Das-I. Reid-L. TI Pus, deoxyribonucleic acid, and sputum viscosity. SO Thorax. 1978 Apr. 33(2). P 235-42. MJ DNA: an. SPUTUM: an. MN ADOLESCENCE. ADULT. AGED. ASTHMA: me. BRONCHIECTASIS: me. BRONCHITIS: me. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: me. FEMALE. HUMAN. HYDROGEN-ION-CONCENTRATION. MALE. MIDDLE-AGE. SPUTUM: ph. SUPPURATION. VISCOSITY. AB On 100 sputum specimens selected from patients suffering from chronic bronchitis, bronchiectasis, asthma, and cystic fibrosis total deoxyribonucleic acid (DNA) content has been related to macroscopic type, to total dry weight yield, and to the apparent viscosity of the secretion at 1350 s-1: since DNA may be present, either as fibres or within cells, in one-third of the specimens the contribution of each form to the apparent viscosity was assessed. The effect on sputum viscosity of the addition of DNA in vitro has also been studied. Whereas between mucoid, mucopurulent, and purulent macroscopic types a significant difference in total DNA and dry weight yield has been found, viscosity was not significantly correlated with purulence. Similarly, the concentration of either cells or fibres correlated significantly with total DNA but not with viscosity. The in vitro addition of DNA to sputum caused a significant increase in its viscosity, and reasons for the differences between the iv vivo and in vitro effect are discussed. Certain constituents of purulent sputum tend to increase viscosity and others to reduce it, and the influence of these varies in the several diseases studied. RF 001 ADLER K AM REV RESPIR DIS 106 86 972 002 BRUCE RA BR MED J 1 282 962 003 BURGI H MED THORAC 21 156 964 004 BURGI H MED HYGIENE 23 1169 965 005 BURTON K BIOCHEM J 62 315 956 006 CHARMAN J BIORHEOLOGY 9 185 972 007 CONWAY BE J POLYM SCI 12 199 954 008 CROFT DN BIOCHEM J 95 612 965 009 DAVIS SS BULL PHYSIOPATH RESPIR NANCY 9 47 973 010 DAVIS SS BIORHEOLOGY 6 11 969 011 FEATHER EA BR J DIS CHEST 64 192 970 012 FEULGEN R QUOTED IN: DRURY RAB 158 967 013 GUERRIN F IN: POINSOT R 249 969 014 KEAL EE POSTGRAD MED J 47 171 971 015 LEACH SA NATURE 199 486 963 016 LIEBERMAN J J LAB CLIN MED 70 595 967 017 LIEBERMAN J PEDIATRICS 31 1028 963 018 LIEBERMAN J LAB INVEST 14 249 965 019 LITT M BIORHEOLOGY 11 111 974 020 LITT M BIORHEOLOGY 13 37 976 021 LOPEZ-VIDRIERO MT THORAX 28 401 973 022 LOPEZ-VIDRIERO MT THESIS 976 023$ LOPEZ-VIDRIERO MT THORAX 734 977 024 MILLER DL AM REV RESPIR DIS 88 473 963 025 MOLINA C J FRANC MED CHIR THORAC 23 734 969 026 PALMER KNV BR J DIS CHEST 64 185 970 027 PUCHELLE E BULL PHYSIOPATH RESPIR NANCY 9 237 973 028 ROSENBLUTH M ARCH DIS CHILD 49 606 974 029 STURGESS J RHEOL ACTA 10 36 971 CT 1 SHIMURA S BIORHEOLOGY 17 363 980 2 KING M FED PROC 39 3080 980 3 MIRRAKHIMOV MM TER ARKH 52 22 980 4 TAKISHIMA T TOHOKU J EXP MED 131 103 980 5 KING M PEDIATR RES 15 120 981 6 MIRRAKHIMOV MM TER ARKH 53 110 981 7 SNYDER CE CARBOHYD RES 105 87 982 8 PUCHELLE E EUR J CLIN INVEST 15 389 985 PN 78197 RN 00977 AN 79077659 AU Hennequet-A. Jehanne-M. Betuel-H. Gilly-R. Schmid-M. Hors-J. TI Cystic fibrosis and HLA. SO Tissue-Antigens. 1978 Sep. 12(3). P 159-62. MJ CYSTIC-FIBROSIS: fg. HLA-ANTIGENS. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FEMALE. GENE-FREQUENCY. HUMAN. HLA-ANTIGENS: an. INFANT. MALE. PEDIGREE. AB In 94 children suffering from cystic fibrosis, no abnormal frequencies of HLA markers of the A and B locus were observed in comparison with the distribution of these antigens in control series. Furthermore, the HLA genotypes of seven pairs of diseased sibs are incompatible with the hypothesis of a closed linkage between CF--an autosomal recessive transmitted disease--and HLA. RF 001 BRIMBLECOMBE FSW LANCET 2 1428 973 002 CONOVER JH LANCET 1 1122 973 003 DANES BS J EXP MED 129 775 969 005 ANON TRANSPLANT REV 22 975 006 KAISER GI HLA AND DISEASE 252 976 007 LAMM LU ANN HUM GENET 38 383 975 008 LOWE CU AM J DIS CHILD 78 349 949 009 MITTAL KK TRANSPLANTATION 6 913 968 010 MORTON NE AM J HUM GENET 7 277 955 011 POLYMENIDIS Z LANCET 2 1452 973 012 RYDER LP HUMANGENETIK 25 251 974 CT 1 SCHWARZ HP J PEDIATR 104 799 984 2 KNOPFLE G KLIN PAEDIATR 197 13 985 PN 78198 RN 00978 AN 78229466 AU Hanawa-M. Takebe-T. Takahashi-S. Koizumi-M. Endo-K. TI The significance of the sweat test in chronic pancreatitis. SO Tohoku-J-Exp-Med. 1978 May. 125(1). P 59-69. MJ PANCREATITIS: me. SODIUM-CHLORIDE: an. SWEAT: an. MN ADOLESCENCE. ADULT. AGE-FACTORS. AGED. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: me. EATING. FEMALE. HUMAN. MALE. METHODS. MIDDLE-AGE. PILOCARPINE: pd. SEX-FACTORS. AB In order to study the disposition which is thought to be latent in chronic pancreatitis, we investigated the sweat chloride concentration of 95 normal subjects, 43 cases of chronic pancreatitis, 12 cases of cholelithiasis, 15 cases of peptic ulcers, 16 cases of hepatic diseases and 23 cases of diabetes mellitus with the sweat test, using the method of pilocarpine iontophoresis. We obtained the following results. (1) In normal subjects, the sweat chloride concentration was inclined to rise gradually with age from childhood to adulthood; the mean value of sweat chloride concentration was 30.0 mEq/liter in adults from 20 years old, and the upper limit was about 60 mEq/liter. (2) The mean value of sweat chloride concentration was 60.0 mEq/liter in chronic calcifying pancreatitis; this value was markedly higher than that of control subjects of the same age (p is less than 0.001). (3) The mean value of sweat chloride concentration in cholelithiasis, peptic ulcer and hepatic diseases did not differ significantly from control subjects. The mean value of sweat chloride concentration in diabetes mellitus was significantly higher than that of control subjects (p is less than 0.01), but was significantly lower than that in chronic pancreatitis (p is less than 0.01). (4) It was supposed that some cases of chronic pancreatitis have a congenital disposition toward abnormal secretion of sweat glands and epithelium in the pancreatic duct, resembling cystic fibrosis, and this disposition leads easily to pancreatic disorders when the individual is exposed to various external factors. RF 001 CAREY MC GUT 9 700 968 002 GIBSON LE PEDIATRICS 23 545 959 003 GROSS JB IN: DE REUCK AVS 278 962 004 KLATSKIN G AM J MED 12 3 952 005 KOCH E IN: HEINKEL K 261 964 006 NOVIS MH LANCET 1 748 975 007 DI SANTAGNESE PA N ENGL J MED 295 481 976 010 SARLES H BIBL GASTROENT BASEL 7 75 965 011 TAKEBE T SAISHIN IGAKU 27 1757 972 012 VINK CLJ IN: DE REUCK AVS 310 962 CT 1 FIGARELLA C EUR J CLIN INVEST 12 145 982 2 QUINTON PM ANNU REV MED 34 429 983 PN 78199 RN 00979 AN 80082663 AU Gogus-S. Caglar-M. Yilgor-E. Altay-C. TI Coexistence of sickle cell trait and cystic fibrosis. SO Turk-J-Pediatr. 1978 Jan-Apr. 20(1-2). P 58-62. MJ ANEMIA-SICKLE-CELL: co. CYSTIC-FIBROSIS: co. SICKLE-CELL-TRAIT: co. MN CASE-REPORT. CONSANGUINITY. CYSTIC-FIBROSIS: pa. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. SICKLE-CELL-TRAIT: pa. EX The aim of this paper is to report in detail the clinical and pathological observations in a case of cystic fibrosis showing widely disseminated intravascular sickling. To the best of our knowledge no case of cystic fibrosis associated with sickle cell trait has been reported in the literature. A six month old infant who was diagnosed to have cystic fibrosis and sickle cell trait was presented and the literature was reviewed for this coexistence. RF 001 MYERSON RM AM J MED 26 543 959 002 ALTAY C HUM HERED 28 56 978 003 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 004 GURSON CT HELV PAEDIATR ACTA 28 165 973 005 OBER WB N ENGL J MED 263 947 960 006 JONES SR N ENGL J MED 282 323 970 007 MCCORMICK WF AM J MED SCI 241 329 961 008 MULLICK FG ARCH ENVIRON HEALTH 26 221 973 PN 78200 RN 00980 AN 79161280 AU Foltinova-J. Mikulikova-K. Soltes-L. TI Mucopolysaccharides in bronchial secretion of children. SO Z-Mikrosk-Anat-Forsch. 1978. 92(2). P 409-15. MJ ASTHMA: me. BRONCHI: se. BRONCHITIS: me. CYSTIC-FIBROSIS: me. MUCOPOLYSACCHARIDES: an. MUCUS: an. MN CHILD. HUMAN. INFANT. MUCUS: cy, se. AB The diseases of respiratory system are nowadays one of the important medical, economical and social problems of modern civilization. A common symptom of these diseases regardless of their etiology, character, klinical manifestation and morphological findings is hypersecretion of bronchial sputum. Tracheobronchial mucus is produced by mucous bronchial glands and goblet cells. This mucus represents at physiological condition about 5 micrometer thick cover on mucous of nasopharynx, larynx, trachea, bronchi up to terminal bronchioles. Protective function of mucus cover resposes in mechanical and humoral barrier and also in removing the inhaled particles and their transport to the upper part of the respiratory system. In spite of the fact, that bronchial secretion has an important role in protective mechanism and in thermal and water exchange of respiratory tract, this secretion is not sufficiently scrutinized mainly of children yet. RF 001 CHLAP Z ACTA MED POL 10 209 959 002 GERTH B Z ERKR ATMUNGSORGANE 132 223 970 003 JONES R HISTOCHEM J 5 19 973 004 KISZELI G MIKROTECHNISCHE UND HISTOCHEM 964 005 PEARSE AGE HISTOCHEM THEORET AND APPLIED 974 006 POGODIN BIV VOPR CASNOJ PAT ANAT VORNA 81 973 007 RAVETTO C J HISTOCHEM CYTOCHEM 12 44 964 008 LAMB D HISTOCHEM J 4 91 972 009 SALVATO G THORAX 23 168 968 010 SCOTT DE HISTOCHEMIE 5 221 965 011 SPICER SS J HISTOCHEM CYTOCHEM 8 18 960 012 SPICER SS J HISTOCHEM CYTOCHEM 13 211 965 013 SPICER SS TECH BULL REG MED TECHNOL 30 53 960 014 SORVARI TE J HISTOCHEM CYTOCHEM 17 291 969 CT 1 FOLTINOVA J BIOLOGIA 36 703 981