PN 77001 RN 00583 AN 78099755 AU Kaiser-G-I. Laszlo-A. Gyurkovits-K. TI Cystic fibrosis: a HLA associated hereditary disease?. SO Acta-Paediatr-Acad-Sci-Hung. 1977. 18(1). P 27-9. MJ CYSTIC-FIBROSIS: fg. HLA-ANTIGENS. MN CYSTIC-FIBROSIS: im. HETEROZYGOTE. HOMOZYGOTE. HUMAN. AB Twelve homozygote patients and thirty-two heterozygote gene carriers from families with cystic fibrosis were HLA-typed. Diagnostic criteria were sweat electrolyte concentration, pancreatic enzyme levels from duodenal juice and stool, Szczepanski's bromide test in the group of homozygotes, and the latter only in the cases of heterozygotes. In comparison with 130 healthy blood donors typed for 29 HLA antigens. B18 proved to be more frequent in the group of patients and gene carriers, with 50 and 31%, respectively, and 14% in the normal population. The association seems to be stronger in the homozygotes than in the heterozygotes (p less than 0.02, respectively). RF 001 DAUSSET J PRESSE MED 76 1397 968 002 DAUSSET J NOUV REV FR HEMATOL 7 161 967 003 DAUSSET J CLIN IMMUNOL IMMUNOPATHOL 3 127 974 004 GOTZ M Z KINDERHEILK 117 183 974 005 POLYMENIDIS Z LANCET 2 1452 973 006 VAN ROOD JJ TRANSPLANT REV 22 75 975 007 SNELL GD FOLIA BIOL 14 335 968 008 SVEJGAARD A TRANSPLANT REV 22 3 975 009 SZCZEPANSKI Z Z KINDERHEILK 113 297 972 010 WOODROW JC PROC R SOC MED 68 802 975 011 ANON EUR J IMMUNOL 5 889 975 CT 1 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 PN 77002 RN 00584 AN 78099754 AU Takacs-O. Sohar-I. Laszlo-A. Penzes-P. Gyurkovits-K. TI Distribution of serum amylase isoenzymes in cystic fibrosis homozygotes and heterozygotes. SO Acta-Paediatr-Acad-Sci-Hung. 1977. 18(1). P 21-6. MJ AMYLASES: bl. CYSTIC-FIBROSIS: fg. ISOENZYMES: bl. MN ADULT. CHILD. CYSTIC-FIBROSIS: di, en. HETEROZYGOTE. HOMOZYGOTE. HUMAN. AB A simple method has been elaborated for the routine separation and quantitative determination of amylase isoenzymes. The ratio P/S, the quotient of the activity values obtained by densitometric evaluation of the pancreatic and salivary isoenzymes, is used to characterize their distribution. In healthy adults and children the value for P/S is above 1 in 80% of the cases, with a mean of 1.87 +/- 0.23. In 90% of heterozygote CF gene-carriers, the P/S is below 1 with a mean of 0.68 +/- 0.13. In addition to the higher total amylase activity, in MV homozygote patients P/S is less than 0.1, and even 0.001. The phenomenon is explained by a compensatory enhancement of salivary activity. The method is a suitable diagnostic test of the exocrine function of the pancreas and for evaluation of the serum amylase isoenzymes. The P/S value allows to differentiate heterozygote CF gene-carriers from homozygotes and healthy individuals. RF 001 DAVIES TJ J CLIN PATHOL 25 266 972 002 DOGGETT RG NATURE NEW BIOL 243 251 973 003 GYURKOVITS K ACTA PAEDIATR ACAD SCI HUNG 15 49 974 005 MERRITT AD AM J HUM GENET 25 510 973 006 NIKOLAJEK WP EUR J PEDIATR 122 289 976 007 OJALA K SCAND J CLIN LAB INVEST 35 163 975 008 OTSUKI M CLIN CHIM ACTA 22 439 976 009 SKUDE G SCAND J CLIN LAB INVEST 35 41 975 010 TAKEUCHI T CLIN CHIM ACTA 54 137 974 011 TAUSSIG LM PEDIATRICS 54 229 974 012 WARD JC AM J HUM GENET 23 403 971 013 WOLF RO AM J CLIN PATHOL 49 871 968 014$ WOLF RO NATURE 213 439 976 PN 77003 RN 00585 AN 77131159 AU Redmond-A-O. Buchanan-K-D. Trimble-E-R. TI Insulin and glucagon response to arginine infusion in cystic fibrosis. SO Acta-Paediatr-Scand. 1977 Mar. 66(2). P 199-204. MJ ARGININE: du. CYSTIC-FIBROSIS: pp. GLUCAGON: se. INSULIN: se. MN ARGININE: ad. BLOOD-GLUCOSE: an. BODY-HEIGHT. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FASTING. FEMALE. HUMAN. INJECTIONS-INTRAVENOUS. MALE. AB The glucagon and insulin responses to intravenous arginine were studied in 17 children with cystic fibrosis and in 9 control children. It was found that the overall secretion of insulin was diminished; however, four of the CF children did have a normal output. The glucagon responses did not parallel those of insulin. The glucagon output varied in the CF children--seven had normal, four excessively high and six low output. Three of the four children with extremely high output had more severe disease and were below the third centile on the weight chart. These four had a fasting hypoglycaemia and also a very low glucose and insulin response. We have confirmed diminished insulin secretion in cystic fibrosis, but diminished glucagon secretion was only noted when some insulin secretion was preserved. The high levels of glucagon seen in the most insulin deficient subjects may be derived from extrapancreatic sources, or may be associated with 'stress' reaction in these patients who also had most severe pulmonary involvement. The data would be consistent with diminished glucagon and insulin secretion from the pancreas but as the disease progresses an excessive secretion of extra pancreatic glucagon results. RF 001 BARBERO GJ CF CLUB ABST 4 967 002 BILBREY GL J CLIN INVEST 53 841 974 003 BUCHANAN KD IN: KIRKHAM KE 136 971 004 BUCHANAN KD LANCET 1 1394 972 005 BUCHANAN KD THESIS 47 973 006 CHARLES RN J CHRON DIS 14 381 961 007 CONLON JM DIABETOLOGICA ABST 39 NO 4 11 336 975 008 FLANAGAN RWJ DIABETOLOGICA 10 365 974 009 HANDWERGER S N ENGL J MED 281 451 969 010 HEDING LG DIABETES 23 257 971 011 JORGENSEN KH HORM METAB RES 4 223 972 012 KALK WJ DIABETES 23 257 974 013 KALK WJ DIABETES 24 851 975 014 MARCO J N ENGL J MED 289 1107 973 015 MARLISS EB J CLIN INVEST 49 2256 970 016 MATSOYAMA T PROC SOC EXP BIOL MED 147 197 974 017 MEGUID MM LANCET 2 1145 972 018 MILNER AD ARCH DIS CHILD 44 351 969 019 MILUNSKY A AM J DIS CHILD 121 15 971 020 PORTE D J CLIN INVEST 45 228 966 021 ROSAN RC AM J DIS CHILD 104 625 962 022 SCHMIDT FH IN: PFEIFFER EF 2 938 971 023 STAHL M J PEDIATR 84 821 974 024 UNGER RH J CLIN INVEST 49 837 970 025 VRANIC M DIABETES 23 905 974 026 WANG CI CF CLUB ABST 8 1 967 027 WILMORE DW LANCET 1 73 974 PN 77004 RN 00586 AN 78183831 AU Falkman-C. TI Cystic fibrosis--a psychological study of 52 children and their families. SO Acta-Paediatr-Scand [Suppl]. 1977. (269). P 1-93. MJ CYSTIC-FIBROSIS: px. MN ADOLESCENCE. ADULT. CASE-REPORT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, fg. ENURESIS: et. FEMALE. HUMAN. INTERVIEW-PSYCHOLOGICAL. MALE. PARENT-CHILD-RELATIONS. PARENTS. PSYCHOLOGICAL-TESTS. QUESTIONNAIRES. SLEEP-DISORDERS: et. SOCIOECONOMIC-FACTORS. SWEDEN. EX The primary goal of this investigation has been to disclose special characteristics among children with Cystic Fibrosis and their families and to focus on important consequences of the illness. The main issues have been: (1) to explore the quality and the magnitude of psychological problems that CF imposes on the child and on its parents; (2) to investigate how the CF child experiences and reacts to its illness; (3) to assess if the CF child differs emotionally, intellectually or in visual-motor functioning from peers; (4) to see how the parents adapt to their child's condition and cope with the problems; (5) to assess the need of help and how it can be given. RF 001 AGLE DP ARCH INTERN MED 114 76 964 002 AGLE DP MODERN TREATMENT 5 111 968 003 AGLE DP BIBL HAEMAT 34 89 970 004 ALLAN JL AUST PAEDIATR J 10 136 974 005 ALLEN F MED PSYCH 8 212 928 006 ANNELL AL PERTUSSIS IN INFANCY AS A CAU 953 007 ARTHUR G ARTHUR ADAPT LEITER INT PERF 970 008 BECK L DYSLEXIA IN PATIENTS WITH CYS 969 009 BENDER L INSTRUCT USE VISU MOT GESTALT 946 010 BLAESING S NURS CLIN NORTH AM 7 597 972 011 BLUMENTHAL MD ARCH GEN PSYCHIAT 21 160 969 012 BOYLE IR J PEDIATR 88 318 976 013 BRAITHWAITE JV PRACTITIONER 165 273 950 014 BROWNE JW AM J ORTHOPSYCHIATRY 30 730 960 015 BURTON L PROC EWGCF 3RD ANNU MTG 972 016 BURTON L PROC INVALID CHLD 84TH ANN MT 972 017 BYRD E IN: MURSTEIN BJ 968 018 CARNE S PROC R SOC MED 68 277 975 019 CARR P LANCET 2 1075 971 020 CHODOFF P AM J PSYCHIATRY 120 743 964 021 CLAWSON A J PROJ TECHN 23 198 959 022 CULLBERG J PSYKISKA TRAUMAT KRIST KRISP 973 023 CYTRYN L IN: ANTHONY EJ 37 973 024 DENNING CR IN: MANGOS JA 127 976 025 DUBO S AM J ORTHOPSYCHIATRY 20 520 950 026 EMERY AEH BR MED J 1 724 973 027 EYSENCK HJ MANUAL OF THE EYSENCK PERSONA 964 028 FEIGELSON J ARCH FR PEDIATR 26 937 969 029 FRANKLIN AW LANCET 1 256 958 030 FRIEDMAN SB PEDIATRICS 32 610 963 031 FALLSTROM K ACTA PAEDIATR SCAND SUPPL 251 974 032 GAYTON WF PEDIATR CLIN NORTH AM 22 161 975 033 GIBSON LE PEDIATRICS 23 545 959 034 GLASER HH PEDIATRICS 33 367 964 035 GLASER HH AM J DIS CHILD 102 344 961 036 GOLDY FB CHILDREN 10 189 963 037 GOODENOUGH F MEASUREMENT OF INTELLIGENCE B 926 038 GREEN M CHILDREN 12 185 965 039 GREEN MN PEDIATRICS 29 438 962 040 GREEN MN PEDIATRICS 34 58 964 041 HALLGREN B ACTA NEUROL SCAND 31 379 956 042 HAMBURG DA ARCH GEN PSYCHIAT 17 277 967 043 HARRIS DB IN: BUROS OK 401 972 044 HECHT F N ENGL J MED 287 464 972 045A HEISLER VA HANDICAPPED CHILD IN THE FAMI 972 045B HILL R IN: PARAD HJ 32 965 046 HUANG NN GUIDE TO DRUG THERAPY IN PATI 972 047 HUANG NN J PEDIATR 59 512 961 048 HUTT M HUTT ADAPTATION OF THE BE 969 049 JORDAN TE MERRILL PALMER QUART 8 243 962 050 KAHN RL DYNAMICS OF INTERVIEWING 957 051 KAHN AU AM J PSYCHIATRY 127 1194 971 052 KALES A N ENGL J MED 290 487 974 053 KANOF A PEDIATRICS 29 37 962 054 KAUFMAN RV PEDIATRICS 48 123 971 055 KELLOGG R WHAT CHILDREN SCRIBBLE AND WH 959 056 KITAY PM IN: BUROS OK 394 972 057 KLACKENBERG G ACTA PAEDIATR SCAND SUPPL 224 971 058 KOLLBERG H CYSTISK FIBROS SYNPUNKT DIAG 969 059 KOLLBERG H MEDLEMSBLAD FUR DE NORDISKA C 969 060 KOLLBERG H FYSIKALISK TERAPI VID CYSTISK 970 061 KOLLBERG H CYSTISK FIBROS MUCOVISCIDOS 970 062 KOLLBERG H CYSTISK FIBROS MUCOVISCIDOS 970 063 KOLLBERG H THESIS 974 064 KOLLBERG H LAKARTIDNINGEN 73 847 976 065 KOLLBERG H CYSTISK FIBROS FORSLAG TILL V 976 066 KOPPITZ EM BENDER GESTALT TEST FOR CHILD 964 067 KOPPITZ EM PSYCHOLOGICAL EVALUATION OF C 968 068 KORSCH BC J PEDIATR 44 703 954 069 KULCZYCKI LL CLIN PROC CHILD HOSP DC 25 320 969 070 LAGERSTROM L SOCIAL SECURITY IN SWEDEN 977 071 LAWLER RH CAN MED ASSOC J 94 1043 966 072 LAWSON D ARCH DIS CHILD 47 1 972 073 LEIBMAN R AM J PSYCHIATRY 131 535 974 074 LINDBERG T LAKARTIDNINGEN 73 2989 976 075 LINDEMANN E AM J PSYCHIATRY 101 141 944 076 LINDER R MED CHILD NEUROL 12 202 970 077 LIPOWSKI ZJ PSYCHIAT MED 2 91 970 078 MACHOVER K PERSONALITY PROJECTION IN THE 949 079 MAIER HW THREE THEORIES OF CHILD DEVEL 969 080 MANDELBAUM A SOC CASEWK 41 360 960 081 MATTSSON A PEDIATRICS 50 801 972 082 MATTSSON A PEDIATR CLIN NORTH AM 22 77 975 083 MATTSSON A J AM ACAD CHILD PSYCHIAT 11 558 972 084 MATTSSON A AM J PSYCHIATRY 122 1349 966 085 MATTSSON A J PEDIATR 68 952 966 086 MCCOLLUM AT J PEDIATR 77 571 970 087 MECHANIC D PEDIATRICS 33 444 964 088 MEYER H SCHWEIZ MED WOCHENSCHR 99 1738 969 089 MEYEROWITZ JH SOC SCI MED 1 249 967 090 NEWMAN J IN: CRUICKSHANK 218 962 091 OPPEL WC PEDIATRICS 42 614 968 092 OPPENHEIMER EH J PEDIATR 77 991 970 093$ PACELLA M J CLIN PSYCHOL 6 18 962 094 PASCAL G BENDER GESTALT TEST 951 095 PEARN JH J MED GENET 10 129 973 096 PECKHAM CS PROC R SOC MED 66 701 973 097 POZANSKI EO REHAB LIT 34 322 973 098 PRUGH DG WIDENING WORLD OF CHILDHOOD 962 099 PRUGH DG IN: SOLNIT AJ 246 963 100 RICHMOND JB AM J DIS CHILD 89 42 955 101 ROBINSON LH J PSYCHOTHERAPY 28 397 974 102 RODGERS B NURS RES 23 420 974 103 DI SANTAGNESE PA N ENGL J MED 277 1287 967 104 SCHMIDT FF ARBETSLAGSTIFTNING- 975 105 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 106 SILVERSTEIN AB J CONSULT CLIN PSYCHOL 25 146 961 107 SPOCK A NC MED J 27 426 966 108 ANON DOCUMENTATIONS ON SWEDEN 977 109 TAUSSIG LM N ENGL J MED 287 586 972 110 TAYLOR K AM J DIS CHILD 119 209 970 111 TEICHER JD CALIF MED 110 371 969 112 TIPS RL BIRTH DEF ORIG ART SER 4 110 968 113 TROPAUER A AM J DIS CHILD 119 424 970 114 TURK J PEDIATRICS 34 67 964 115 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 117 ANON WISC MANUAL WECHSLER INTELLIG 970 118 WERRY JS CAN MED ASSOC J 97 319 967 119 WILLIAMS JI SOC SCI MED 5 219 971 120 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 CT 1 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 2 DEWET B S AFR MED J 65 526 984 3 DEWET B S AFR MED J 67 292 985 4 DEWET B S AFR MED J 67 370 985 5 PUMARIEGA AJ J AM ACAD CHILD PSYCHIAT 25 269 986 6 MOLLERING M KLIN PAEDIATR 198 369 986 PN 77005 RN 00587 AN 78036889 AU Ryssing-E. TI Assessment of cor pulmonale in cystic fibrosis by echocardiography. SO Acta-Paediatr-Scand. 1977 Nov. 66(6). P 753-6. MJ CYSTIC-FIBROSIS: co. ECHOCARDIOGRAPHY. PULMONARY-HEART-DISEASE: di. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FEMALE. HEART-ENLARGEMENT. HUMAN. MALE. PEAK-EXPIRATORY-FLOW-RATE. PULMONARY-HEART-DISEASE: co. VITAL-CAPACITY. AB Thirty-one patients with cystic fibrosis of varying severity were examined by echocardiography. Right ventricular dimension (RVD) was above upper normal limit in 14 patients and right ventricular dimension index (RVD index) was higher than the upper normal limit in 11 patients. Furthermore, there was a significant relationship between increasing RVD index and 1) decreasing forced vital capacity (FVC) both actual test results and average 6 months values; and 2) decreasing peak-expiratory flow rate (PEFR) both actual test results and average 6 months values. This observation suggests a persistent heart involvement. Five patients had either heart failure and/or electrocardiographic evidence of right ventricular abnormality. These patients had increased RVD index and one patient with the highest RVD index died 8 weeks after the examination. The present study has shown the usefulness of echocardiographic measurement of right ventricular dimension and of septal motion in assessing cor pulmonale, before development of electrocardiographic abnormalities and right heart failure. RF 001 DIAMOND MA CIRCULATION 43 129 971 002 FEIGENBAUM H ECHOCARDIOGRAPHY 972 003 GOLDRING RM J PEDIATR 65 501 964 004 GOODMAN DJ AM J CARDIOL 33 438 974 005 HOYBYE G UGESKR LAEGER 132 2007 970 006 LAURENCEAU JL CHEST 69 388 976 007 LUNDSTROM NR ACTA PAEDIATR SCAND SUPPL 243 974 008 LUNDSTROM NR ACTA PAEDIATR SCAND 63 257 974 009 LYONS HA AM J DIS CHILD 100 196 960 010 MCCANN WD JAMA 221 1243 972 011 MOSS AJ J PEDIATR 67 797 965 012 PADMAVATI S BR HEART J 34 658 972 013 POPP RL AM J CARDIOL 24 523 969 014 ROSENTHAL A PEDIATR CLIN NORTH AM 23 327 976 015 SIASSI B J PEDIATR 78 794 971 016 TAJIK AJ CIRCULATION 46 36 972 017 WEYMAN AE CIRCULATION 54 179 976 CT 1 SANTAGNESE PAD AM J MED 66 121 979 2 HIRSCHFELD SS CHEST 75 351 979 3 MATTHAY RA BR HEART J 43 474 980 4 LESTER LA J PEDIATR 97 742 980 5 MATTHAY RA MED CLIN NORTH AM 65 489 981 6 JACOBSTEIN MD CHEST 80 399 981 7 MOSS AJ PEDIATRICS 70 728 982 8 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 9 JOHNSON SR OBSTET GYNECOL 61 S 2 983 10 LESTER LA SEM RESPIR MED 6 285 985 PN 77006 RN 00588 AN 77131198 AU Schiotz-P-O. Hoiby-N. Juhl-F. Permin-H. Nielsen-H. Svehag-S-E. TI Immune complexes in cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [C]. 1977 Feb. 85(1). P 57-64. MJ ANTIGEN-ANTIBODY-COMPLEX. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. ANTINUCLEAR-FACTORS: an. CHILD. CHRONIC-DISEASE. COMPLEMENT: an. CYSTIC-FIBROSIS: co. FEMALE. HAPTOGLOBINS: an. HUMAN. IMMUNOGLOBULINS: an. MALE. PRECIPITINS: an. PSEUDOMONAS-INFECTIONS: co, im. RESPIRATORY-TRACT-INFECTIONS: co, im. RHEUMATOID-FACTOR: an. SKIN: im. AB Eleven patients with cystic fibrosis (CF) chronically infected with mucoid P. aeruginosa and ten patients without P. aeruginosa infection were examined for occurrence of circulating immune complexes, for immune complex deposits in the dermo-epidermal junction of the skin and for precipitins against P. aeruginosa, S. aureus, H. influenzae and D. pneumoniae antigens. The serum concentrations of haptoglobin, orosomucoid, immunoglobulins, C1q, C3, C4 and total haemolytic complement, antinuclear and rheumatoid factor activities as well as white blood cell counts and erythrocyte sedimentation rates were determined also. The results indicated that 6 patients from the chronically P. aeruginosa infected group, exhibiting a spectrum of serum precipitins against P. aeruginosa antigens, also had immune complexes in the serum, while only one patient (suffering from selective IgA deficiency) in the group without P. aeruginosa infection was positive for soluble immune complexes. Granular deposits of IgM was found in the skin of 10 of the chronically P. aeruginosa infected patients and in 7 of the patients without P. aeruginosa infection. A few pactients in both groups had dermo- epidermal deposits of C1q, C3 or fibrinogen as well. Eight of the patients in the chronically infected group and five in the group without P. aeruginosa infection had organ non-specific antinuclear factors. The haptoglobin levels appeared to be elevated in the chronically infected patients (p less than 0.05). None of the other parameters showed any significant difference between the two groups. RF 001 BAART DE LA FAILLE-E THESIS 969 002 BONOMO L AM J CLIN PATHOL 45 313 966 003 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 004 GORDON DS AM REV RESPIR DIS 108 127 973 005 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 006 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 009 HOIBY N ACTA PAEDIATR SCAND 63 843 974 010 HOIBY N SCAND J RESPIR DIS 56 38 975 011 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 012 JOHNSON KJ J CLIN INVEST 54 349 974 013 JOHNSON U ACTA PATH MICROBIOL SCAND (C) 83 285 975 014 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 144 975 015 KOFFLER D J EXP MED 126 607 967 016 LANDRY M J CLIN INVEST 52 1861 973 017 LAURELL CB SCAND J CLIN LAB INVEST SUPPL 124 21 972 018 LEBER PD IN: ZWEIFACH BW 3 401 974 019 MASS B AM REV RESPIR DIS 106 384 972 020 MAYER MM IN: KABAT EA 133 961 021 MCFARLANE H BR MED J 1 423 975 022 MCINTOSH RM Q J MED 44 286 975 023 NIELSEN H ACTA PATH MICROBIOL SCAND (C) 84 261 976 024 NIELSEN H EUROPEAN IMMUNOLOGY MTG ABST 975 025 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 026 TAN EM POSTGRAD MED 54 143 973 027 TAN EM J CLIN INVEST 45 1732 966 028 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 029 TUFFANELLI DL ARCH DERMATOL 99 652 969 030 TURNER-WARWICK M BR MED J 3 492 970 031 WIIK A IMMUNOLOGY 23 53 972 032 WIIK A ANN RHEUM DIS 33 515 974 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 FARRELL C SCAND J IMMUNOL 6 1233 977 3 PERMIN H SCAND J RHEUMATOL 6 105 977 4 ZUBLER RH PROG ALLERGY 24 1 978 5 NIELSEN HE ACTA PAEDIATR SCAND 67 443 978 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 7 GUPTA RK CANCER IMMUNOL IMMUNOTHER 6 211 979 8 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 9 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 10 PERMIN H ACTA MED SCAND 205 333 979 11 BERDISCHEWSKY M PEDIATR RES 14 830 980 12 HODSON ME THORAX 35 801 980 13 SKOV PS ALLERGY 35 23 980 14 MOSS RB AM REV RESPIR DIS 121 23 980 15 MATTHEWS WJ N ENGL J MED 302 245 980 16 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 17 EGESKJOLD EM ALLERGY 36 573 981 18 PENNINGTON JE J CLIN INVEST 68 1140 981 19 CHURCH JA CHEST 80 405 981 20 MOSS RB J PEDIATR 99 215 981 21 SORENSEN RU AM REV RESPIR DIS 123 37 981 22 DANIELE RP AM REV RESPIR DIS 124 738 981 23 FROLAND SS SCAND J INFECT DIS 1981 72 981 24 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 25 PERMIN H ALLERGY 37 93 982 26 MOSS RB CLIN EXP IMMUNOL 47 301 982 27 PITCHERWILMOTT RW ARCH DIS CHILD 57 577 982 28 MANTHEI U AM REV RESPIR DIS 126 253 982 29 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 30 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 31 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 32 ANON LANCET 2 257 983 33 KLINGER JD INFECT IMMUN 39 1377 983 34 SORENSEN RU EUR J RESPIR DIS 64 524 983 35 WALLACE JM AM REV RESPIR DIS 128 1077 983 36 DORING G J INFECT DIS 147 744 983 37 DAVIS CA HUM PATHOL 15 244 984 38 DORING G ACTA PATH MICROB IMMU SCA (C) 92 307 984 39 HODSON ME CLIN ALLERGY 15 363 985 40 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 41 KHARAZMI A EUR J CLIN INVEST 16 143 986 42 HOIBY N ANNU REV MICROBIOL 40 29 986 43 PHILLIPS BM J ROY SOC MED 79 44 986 44 MOSS RB AM REV RESPIR DIS 133 648 986 45 BOGART BI J CHROMATOGR 381 29 986 46 DASGUPTA MK J CLIN IMMUNOL 7 51 987 PN 77007 RN 00589 AN 77178547 AU Holby-N. Olling-S. TI Pseudomonas aeruginosa infection in cystic fibrosis. Bactericidal effect of serum from normal individuals and patients with cystic fibrosis on P. aeruginosa strains from patients with cystic fibrosis or other diseases. SO Acta-Pathol-Microbiol-Scand [C]. 1977 Apr. 85(2). P 107-14. MJ BLOOD-BACTERICIDAL-ACTIVITY. CYSTIC-FIBROSIS: im. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: co. MN ADOLESCENCE. ANTIBODIES-BACTERIAL: an. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: co. HUMAN. PSEUDOMONAS-INFECTIONS: im. AB P. aeruginosa strains originating from the respiratory tract of patients with cystic fibrosis (CF) and patients with other diseases (non-CF) were analysed with regard to their sensitivity to the bactericidal activity of human serum. P. aeruginosa strains isolated from CF patients were more sensitive than strains from non-CF patients to the bactericidal activity of normal human serum. The bactericidal activity was heatlabile. As regards the sensitivity to normal human serum, mucoid and non-mucoid variants were not found to differ. Strains originating from chronically infected CF patients with many precipitins against these bacteria did not differ with respect to serum sensitivity from strains originating from intermittently colonized CF patients without P. aeruginosa precipitins. Compared with normal sera, CF sera showed similar or higher bactericidal activity against a panel of P. aeruginosa strains. In this respect, any difference between CF sera with precipitins and CF sera without precipitins against P. aeurginosa was not found. Sera from three CF patients chronically infected with P. aeruginosa, and with many precipitins against these bacteria, showed a selective inability in bactericidal activity against the patients' own P. aeruginosa isolate possibly reflecting the presence of "bactericidal blocking" antibodies. RF 001 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 002 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 003 BJORNSON AB INFECT IMMUN 2 453 970 004 BJORNSON AB INFECT IMMUN 4 462 971 005 BJORNSON AB INFECT IMMUN 5 775 972 006 BJORNSON AB J INFECT DIS SUPPL 130 119 974 007 BJORNSON AB J INFECT DIS SUPPL 130 127 974 008 BOXERBAUM B AM REV RESPIR DIS 108 777 973 009 CROWDER JG J LAB CLIN MED 83 853 974 010 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 011 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 012 GOLDMAN JN INFECT DIS 129 444 974 013 GOVAN JRW J MED MICROBIOL 8 513 975 014 GRIFFISS JM J IMMUNOL 114 1779 975 015 GUTTMAN RM CLIN EXP IMMUNOL 19 121 975 016 HOWARD CJ IMMUNOLOGY 20 767 971 017 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 018 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 019 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 020 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 021 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 144 975 022 LINDBERG U ACTA PAEDIATR SCAND 64 432 975 023 MARTIN DR J MED MICROBIOL 6 111 973 024 MOREAU SC SCIENCE 190 278 975 025 MUSCHEL LH J IMMUNOL 76 1 956 026 OLLING S INFECTION 1 24 973 027 REED WP IMMUNOLOGY 26 205 974 028 ROWLEY D IN: KASS E 162 973 029 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 030 SIMBERKOFF MS J LAB CLIN MED 87 207 976 031 TAYLOR PW CLIN SCI 43 705 972 032 TAYLOR PW J GEN MICROBIOL 89 57 975 033 TAUB WH INFECT IMMUN 13 1343 976 034 WAISBREN BA J IMMUNOL 97 431 966 035 YOUNG LS J INFECT DIS 126 257 972 036 YOUNG LS J INFECT DIS 126 257 972 037 YOUNG LS J INFECT DIS SUPPL 130 111 974 038 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 LARSSON P MED MICROBIOL IMMUNOL 163 77 977 3 LIDINJANSON G MED MICROBIOL IMMUNOL 164 247 978 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 5 HODSON ME THORAX 35 801 980 6 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 7 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 8 KOEPP LH INFECT IMMUN 33 788 981 9 THOMASSEN MJ INFECT IMMUN 33 512 981 10 FICK RB J CLIN INVEST 68 899 981 11 MARKS MI J PEDIATR 98 173 981 12 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 14 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 15 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 16 OFFREDOHEMMER C ANN MICROBIOL (PARIS) A134 281 983 17 BOROWSKI RS CURRENT MICROBIOL 9 25 983 18 PENKETH ARL J MED MICROBIOL 16 401 983 19 JAGGER KN J CLIN MICROBIOL 17 55 983 20 SCHILLER NL PEDIATR RES 17 747 983 21 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 22 CRYZ SJ INFECT IMMUN 39 1072 983 23 TAYLOR PW MICROBIOL REV 47 46 983 24 PENKETH A AM REV RESPIR DIS 127 605 983 25 SCHILLER NL CURRENT MICROBIOL 10 185 984 26 MESHULAM T CLIN IMMUNOL IMMUNOPATHOL 32 151 984 27 KHARAZMI A INFECT IMMUN 43 161 984 28 SCHILLER NL INFECT IMMUN 45 748 984 29 THOMASSEN MJ INFECT IMMUN 45 741 984 30 FICK RB J CLIN INVEST 74 236 984 31 FALKENHAGEN U Z UROL NEPHROL 77 629 984 32 PIER GB J INFECT DIS 150 223 984 33 NICAS TI EUR J CLIN MICROBIOL 4 175 985 34 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 35 PAJDAK E ARCH IMMUNOL THER EXP 33 499 985 36 OJENIYI B ACTA PATH MICROB IMMU SCA (B) 93 7 985 37 SAWADA S J INFECT DIS 152 1290 985 38 SCHILLER NL INFECT IMMUN 54 689 986 39 FALKENHAGEN U Z UROL NEPHROL 79 619 986 40 FICK RB AM REV RESPIR DIS 133 418 986 41 SPEERT DP J HOSP INFECT 9 11 987 PN 77008 RN 00590 AN 78036942 AU Hoiby-N. TI Pseudomonas aeruginosa infection in cystic fibrosis. Diagnostic and prognostic significance of pseudomonas aeruginosa precipitins determined by means of crossed immunoelectrophoresis. A survey. SO Acta-Pathol-Microbiol-Scand [Suppl]. 1977. (262). P 1-96. (REVIEW). MJ CYSTIC-FIBROSIS: im. PRECIPITINS. PSEUDOMONAS-INFECTIONS: im. MN ANTIBODIES-BACTERIAL: an. ANTIGENS-BACTERIAL: an. CROSS-REACTIONS. CYSTIC-FIBROSIS: co. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. PRECIPITINS: an. PROGNOSIS. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: co. REVIEW. EX The purpose of the first part of the present work is to review the literature concerning the lower respiratory tract infections and the antimicrobial resistance mechanisms in cystic fibrosis patients. The second part of the work deals with the most important therapeutical problem in cystic fibrosis today, viz. the Pseudomonas aeruginosa lung infection. A survey is made of the investigations of the present author and his colleagues into the immune response against Pseudomonas aeruginosa infection and the literature concerning these subjects. Furthermore, the possible mechanisms of tissue damage in the respiratory tract of cystic fibrosis patients during these infections are discussed. RF 001 ADLER JL ARCH INTERN MED 127 760 971 002 ALLEN JC INFECT IMMUN 12 318 975 003$ ALLISON AC TRANSPLANT REV 19 3 975 004 ANDERSEN B IN: PEDERSEN J 120 971 005 ANDERSEN B NORD MED 39 1575 948 006 ANDERSEN DH AM J DIS CHILD 56 344 938 007 ANDERSEN DH PEDIATRICS 3 406 949 008 ANDERSEN DH J CHRON DIS 7 58 958 009 ANDERSEN V CLIN EXP IMMUNOL 26 469 976 010 ANDERSON EL SOUTH MED J 67 771 974 011 ASAY LD N ENGL J MED 262 1062 960 012 AXELSEN NH INFECT IMMUN 4 525 971 013 AXELSEN NH PROTIDES BIOL FLUIDS 19 561 971 014 AXELSEN NH J IMMUNOL METH 1 109 972 015 AXELSEN NH INFECT IMMUN 7 949 973 016 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 71 973 017 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 91 973 018 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 101 973 019 AXELSEN NH J IMMUNOL METH 2 393 973 020 AXELSEN NH J IMMUNOL METH 2 245 973 021 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 973 022 AXELSEN NH CLIN EXP IMMUNOL 17 385 974 023 AXELSEN NH INFECT IMMUN 9 952 974 024 AXELSEN NH SCAND J IMMUNOL SUPPL 2 4 1 975 025 AXELSEN NH SCAND J IMMUNOL SUPPL 2 4 217 975 026 AXELSEN NH SCAND J IMMUNOL 5 177 976 027 BAIRD IM ANTIMICROB AGENTS CHEMOTHER 10 626 976 028 BARDANA EJ JR AM J DIS CHILD 129 1164 975 029 BARSON AJ ARCH DIS CHILD 46 55 971 030 BARTON AD J LAB CLIN MED 88 423 976 031 BEDROSSIAN CWM HUM PATHOL 7 195 976 032 BERGAN T IN: BROWN MRW 189 975 033 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 034 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 035 BIGGAR WD AM REV RESPIR DIS 109 403 974 036 BJERRUM OJ SCAND J IMMUNOL SUPPL 1 2 133 973 037 BJERRUM OJ SCAND J IMMUNOL SUPPL 2 4 89 975 038 BJORNSON AB J INFECT DIS SUPPL 130 119 974 039 BJORNSON AB J INFECT DIS SUPPL 130 127 974 040 BLACKFAN KD J PEDIATR 13 627 938 041 BOCK E J NEUROCHEM 18 2435 971 042 BOCK E J IMMUNOL METH 2 75 972 043 BOCK E SCAND J IMMUNOL SUPPL 1 2 95 973 044 BODE FR MEDICINE (BALTIMORE) 53 255 974 045 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 046 BOG-HANSEN TC ANAL BIOCHEM 56 480 973 047 BOHME B HELV PAEDIATR ACTA 27 607 972 048 BOXERBAUM B AM REV RESPIR DIS 108 777 973 049 BOXERBAUM B AM REV RESPIR DIS 109 403 974 050 BRADLEY DE J HYG LOND 74 419 975 051 BRADSHAW MW LANCET 1 1095 971 052 BRENTJENS JR J EXP MED 140 105 974 053 BROGAN TD IMMUNOLOGY 7 626 964 054 BROGAN TD THORAX 30 72 975 055 BROGREN CH SCAND J IMMUNOL SUPPL 2 4 37 975 056 BRYANT DH J ALLERGY CLIN IMMUNOL 56 417 975 057 BUCKLEY RH J CLIN INVEST 55 157 975 058 BURNS MW AUSTRALAS ANN MED 17 289 968 059 BURNS MW LANCET 1 270 968 060 BURNS MW BR MED J 3 382 973 061 CALDWELL HD J IMMUNOL 115 963 975 062 CALDWELL HD J IMMUNOL 115 969 975 063 CAMPBELL PA BACT REV 40 284 976 064 CARLSON DM BIOCHEMISTRY 5 2817 966 065 CETIN ET J BACTERIOL 89 1432 965 066 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 067 CLARKE CW POSTGRAD MED J 51 147 975 068 CLARKE HGM PROTIDES BIOL FLUIDS 14 503 966 069 CLOSS O SCAND J IMMUNOL SUPPL 2 4 173 975 070 COCHRANE CG IN: ZWEIFACH BW 3 85 974 071 COOMBS RRA IN: GELL PGH 761 975 072 COOPER RG MED J AUST 1 527 967 073 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 074 CURTIN JA ANN INTERN MED 54 1077 961 075 DAHR P DTSCH MED WSCHR 61 1879 935 076 DANES BS LANCET 1 1061 968 077 DANES BS AM J HUM GENET 23 297 971 078 DANES BS CLIN GENET 11 83 977 079 DEFOREST A CF CLUB ABST 976 080 DIAZ F J INFECT DIS 121 269 970 081 DICKEY LB DIS CHEST 17 151 950 082 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 083 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 084 DI SANTAGNESE PA PEDIATRICS 12 178 953 085 DI SANTAGNESE PA DIS CHEST 27 654 955 086 DI SANTAGNESE PA AM J MED 21 406 956 087 DI SANTAGNESE PA N ENGL J MED 277 1287 967 088 DI SANTAGNESE PA N ENGL J MED 295 481 976 089 DOGGETT RG J BACTERIOL 87 427 964 090 DOGGETT RG J BACTERIOL 89 476 965 091 DOGGETT RG J PEDIATR 68 215 966 092 DOGGETT RG APPL MICROBIOL 18 936 969 093 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 094 DOGGETT RG INFECT IMMUN 6 628 972 095 DOGGETT RG CF CLUB ABST 976 097 ANON NUTR REV 34 210 976 098 EICHENWALD HF IN: MCINTOSH R 89 960 099 ELSTON HR AM J CLIN PATHOL 48 519 967 100 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 101 ESTERLY JR IN: MANGOS JA 115 976 102 EVANS LR J BACTERIOL 116 915 973 103 FANCONI G WIEN MED WOCHENSCHR 86 753 936 104 FARBER S ARCH PATHOL 37 238 944 105 FEIGELSON J MOD PROBL PEDIATR 10 214 967 106 FEIGHERY C BR MED J 1 524 976 107 FEIGIN RD J PEDIATR 87 677 975 108 FLENSBORG EW NORD MED 39 1574 948 109 FLENSBORG EW MDSKR PRAKT LAEGEG 50 269 972 110 FOLEY FD TEX MED 65 36 969 111 FORKNER CE PSEUDOMONAS AERUGINOSA INFECT 69 960 112 FORSGREN A INFECT IMMUN 10 402 974 113 FRIIS B PROC INT CF CONG 7TH 976 114 FAERO O UGESKR LAEGER 138 2551 976 115 GALANT SP AM REV RESPIR DIS 114 325 976 116 GALLIN JI IN: KIRKPATRICK CH 161 976 117 ANON GAP CONF REP PSEUDOMON IN CF 974 118 GARRARD SD PEDIATRICS 8 482 951 119 GELFAND EW PEDIATR CLIN NORTH AM 21 745 974 120 GIBBONS A BR MED J 1 120 976 121 GIFFORD RM J S CAROLINA MED ASSOC 68 204 972 122 GORDON DS AM REV RESPIR DIS 108 127 973 123 GOVAN JRW J MED MICROBIOL 8 513 975 124 GOVAN JRW J ANTIMICROB CHEMOTHER 2 215 976 125 GRABAR P BIOCHIM BIOPHYS ACTA 10 193 953 126 GRANT JA J IMMUNOL 114 1101 975 127 GREEN GM MED CLIN NORTH AM 57 547 973 128 GRIFFISS JM J IMMUNOL 114 1779 975 129 GROV A ACTA PATH MICROBIOL SCAND (C) 84 71 976 130 GRUBB A SCAND J CLIN LAB INVEST SUPPL 124 59 972 131 GUGLER EC J PEDIATR 73 548 968 132 GUTTMAN RM CLIN EXP IMMUNOL 19 121 975 133 GOTZ M Z KINDERHEILK 117 183 974 134 HABBOUSHE C PEDIATRICS 48 973 971 135 HALBERT SP PEDIATRICS 26 792 960 136 HALBERT SP MOD PROBL PEDIATR 10 144 967 137 HANN S INFECT IMMUN 14 114 976 138 HARBOE N SCAND J IMMUNOL SUPPL 1 2 161 973 139 HARRISON GM CF Q ANNOTATED REFERENCES 13 30 975 140 HENRIKSEN SD ACTA PATH MICROBIOL SCAND 25 485 948 141 HERTZ JB ACTA PATH MICROBIOL SCAND (B) 84 386 976 142 HILL HR J PEDIATR 84 55 974 143 HIRSCHHORN R IN: ZWEIFACH BW 1 259 974 144 HOBBS G J APPL BACT 27 83 964 145 HOFF GE SCAND J INFECT DIS 6 333 974 146 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 147 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 148 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 149 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 150 HOIBY N ACTA PAEDIATR SCAND 63 843 974 151 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 152 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 153 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 328 975 154 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 155 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 156 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 157 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 158 HOIBY N SCAND J RESPIR DIS 56 38 975 159 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 160 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 161 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 162 HOIBY N SCAND J RESPIR DIS 57 103 976 163 HOIBY N SCAND J RESPIR DIS 57 37 976 164 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 165 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 166 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 167 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 168 HOIBY N SCAND J RESPIR DIS 58 65 977 169 HOLMBERG K THESIS 1 975 170 HOLZHAUER RJ CF CLUB ABST 17 22 976 171 HOMMA JY JAPAN J EXP MED 42 583 972 172 HOMMA JY JAPAN J EXP MED 44 1 974 173 HOOK WA J IMMUNOL 114 1185 975 174 HUANG NN J PEDIATR 59 512 961 175 HUANG NN AM J DIS CHILD 120 289 970 176 HUANG NN CF CLUB ABST 43 976 177 HUANG NN CF CLUB ABST 976 178 HUGHES DE IN: NORRIS JR 5B 1 971 179 IACOCCA VF AM J DIS CHILD 106 315 963 180 IACOCCA VF ARCH DIS CHILD 43 220 968 181 JACKSON AE J LAB CLIN MED 69 833 967 182 JESSEN O THESIS 61 965 183 JOHANSEN KS UGESKR LAEGER 138 2772 976 184 JOHNSON KJ J CLIN INVEST 54 349 974 185 KAIJSER B INT ARCH ALLERGY 48 72 975 186 KALTREIDER HB AM REV RESPIR DIS 113 347 976 187 KALTREIDER HB IN: KIRKPATRICK CH 73 976 188 KILBOURN JP J PEDIATR 73 408 968 189 KIRKPATRICK CH IMMUNOL INFECT REACT LUNG 1 976 190 KIRKPATRICK CH IN: KIRKPATRICK CH 211 976 191 KLEINMAIER H ZENTRALBL BAKTERIOL ORIG A 172 54 958 192 KLINEBERGER C MUNCH MED WOCHENSCHR 54 1330 907 193 KLINEBERGER C Z IMMUN FORSCH 2 686 909 194 KOCH C ACTA PATH MICROBIOL SCAND (B) 81 787 973 195 KOCH C ACTA PATH MICROBIOL SCAND (B) 82 439 974 196 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 144 975 197 KOLLBERG H THESIS 974 198 KONTOMICHALOU P ANTIMICROB AGENTS CHEMOTHER 9 866 976 199 KRONVALL G INFECT IMMUN 13 1132 976 200 LAMM LU ANN HUM GENET 38 383 975 201 LANYI B ACTA MICROBIOL ACAD SCI HUNG 19 259 972 202 LANYI B ACTA MICROBIOL ACAD SCI HUNG 20 249 973 203 LANYI B ACTA MICROBIOL ACAD SCI HUNG 20 337 973 204 LARAYA-CUASAY LR J PEDIATR 89 23 976 205 LAURELL CB SCAND J CLIN LAB INVEST SUPPL 124 21 972 206 LAWSON D IN: WATT PJ 69 970 207 LEBER PD IN: ZWEIFACH BW 3 401 974 208 LIND I ACTA PATH MICROBIOL SCAND 73 637 968 209 LINKER A J BIOL CHEM 241 3845 966 210 LIU PV J INFECT DIS SUPPL 130 94 974 211 LLOYD-STILL JD PEDIATRICS 53 678 974 212 LOPEZ M ANNU REV MED 27 453 976 213 LOWBURY EJL J MED MICROBIOL 3 39 970 214 LYKKEGAARD E DAN MED BULL 21 241 974 215 MARGARETTEN W N ENGL J MED 265 773 961 216 MARTIN DR J MED MICROBIOL 6 111 973 217 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 218 MATTHEWS LW J PEDIATR 65 558 964 219 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 220 MAY JR ARCH DIS CHILD 47 908 972 221 MAY JR J MED MICROBIOL 6 77 973 222 MAY JR POSTGRAD MED J 51 144 975 223 MCCOMBS ML TEX REP BIOL MED 31 615 973 224 MCCOMBS RP N ENGL J MED 286 1186 972 225 MCCRAE WM SCOTT MED J 19 187 974 226 MCFARLANE H BR MED J 1 423 975 227 MCFARLANE H BR MED J 1 524 976 228 MEARNS MB LANCET 1 538 967 229 MEARNS MB ARCH DIS CHILD 47 5 972 230 MEARNS MB ARCH DIS CHILD 47 902 972 231 MITCHELL-HEGGS PF Q J MED 45 479 976 232 MURAKAWA T JAPAN J MICROBIOL 17 273 973 233 MURAKAWA T JAPAN J MICROBIOL 17 513 973 234 MYEROWITZ RL LANCET 2 250 972 235 NELSON RA JR IN: ZWEIFACH BW 3 37 974 236 NETER E J INFECT DIS SUPPL 130 132 974 237 NEWHOUSE M N ENGL J MED 295 990 976 238 NIELSEN EL UGESKR LAEGER 133 1017 971 239 NIELSEN H ACTA PATH MICROBIOL SCAND (C) 84 261 976 240 OGILVIE BM BR MED BULL 32 177 976 241 OLITZKI AL BOLL IST SIEROTER MILAN 42 213 963 242 ORES CN CF Q ANNOTATED REFERENCES 13 31 975 243 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 244 PENNINGTON JE AM J MED 55 155 973 245 PENNINGTON JE J INFECT DIS SUPPL 130 159 974 246 PENNINGTON JE AM J MED 58 629 975 247 PITTMAN FE PEDIATRICS 24 40 959 248 POLLEY MJ J MED GENET 11 249 974 249 REYNOLDS HY J IMMUNOL 111 358 973 250 REYNOLDS HY J IMMUNOL 111 358 973 251 REYNOLDS HY J INFECT DIS SUPPL 130 134 974 252 REYNOLDS HY J CLIN INVEST 53 1351 974 253 REYNOLDS HY ANN INTERN MED 82 819 975 254 REYNOLDS HY J CLIN INVEST 56 376 975 255 REYNOLDS HY J IMMUNOL 114 1813 975 256 REYNOLDS HY IN: KIRKPATRICK CH 143 976 257 REYNOLDS HY IN: KIRKPATRICK CH 3 976 258 ROBINSON MJ ARCH DIS CHILD 50 962 975 259 ROE EA BR J EXP PATHOL 55 336 974 260 ROSENLUND ML CRC CRIT REV CLIN LAB SCI 3 257 972 261 SADOFF JC J INFECT DIS SUPPL 130 61 974 262 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 263 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 264 SCHWAB JH BACT REV 39 121 975 265 SCHWARTZ RH AM J DIS CHILD 111 408 966 266 SCHWARTZ RH AM J DIS CHILD 120 432 970 267 SCHWARZMANN S INFECT IMMUN 3 762 971 268 SCHWARZ LH J BACTERIOL 54 30 947 269 SEIDMON EJ J PEDIATR 87 528 975 270 SHWACHMAN H CLIN PEDIATR 14 1115 975 271 SHIRAGANIAN RP J IMMUNOL 116 639 976 272 SLAVIN RG POSTGRAD MED 59 137 976 273 SMYTH CJ INFECT IMMUN 13 1273 976 275 SOLBERG CO LANCET 2 727 972 276 SONNENSCHEIN C ZENTRALBL BAKTERIOL 104 365 927 277 SOUTAR CA THORAX 31 158 976 278 SOUTH MA J PEDIATR 71 645 967 279 SOUTHERN PM J LAB CLIN MED 76 548 970 280 SOUTHERN PM APPL MICROBIOL 21 377 971 281 SPOCK A PEDIATR RES 1 173 967 282 STERN RC J PEDIATR 89 406 976 283 STOSSEL TP N ENGL J MED 290 717 974 284 SULLIVAN JF AM J DIS CHILD 125 702 973 285 SUTHERLAND IW IN: WEIR DM 1 1 973 286 SWEET GH J IMMUNOL 111 554 973 287 SVENDSEN PJ J IMMUNOL METH 1 169 972 288 TALAMO RC IN: MANGOS JA 195 976 289 TAUSSIG LM J PEDIATR 84 724 974 290 TAUSSIG LM CF CLUB ABST 17 21 976 291 TAYLOR PW CLIN SCI 43 705 972 292 THERKELSEN AJ MEDICINSK STATISTIK AKADEMISK 968 293 THIRKILL CE J IMMUNOL 114 1107 975 294 TUNSTALL AM J APPL BACT 38 159 975 295 TURK JL BOLL IST SIEROTER MILAN SUPPL 53 208 974 296 TURNER-WARWICK M THORAX 30 601 975 297 URIEL J IN: GRABAR P 30 964 298 VAWTER GF GAP CONF REP PATHOL OF CF 9 972 299 VERBRUGGEN R CLIN CHEM 21 5 975 300 VESTERGAARD BJ ACTA PATH MICROBIOL SCAND (B) 81 808 973 301 WAISBREN BA J IMMUNOL 97 431 966 302 WALDMAN RH J INFECT DIS 130 419 974 303 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 304 WARNER JO ARCH DIS CHILD 51 507 976 305 WARREN CPW CLIN ALLERGY 5 1 975 306 WARREN SL ANN ALLERGY 36 337 976 307 WARWICK WJ J ASTHMA RES 5 277 968 308 WARWICK WJ J CHRON DIS 28 609 975 309 WEEKE B SCAND J CLIN LAB INVEST 21 351 968 310 WEEKE B PROTIDES BIOL FLUIDS 19 547 971 311 WEEKE B SCAND J IMMUNOL SUPPL 1 2 37 973 312 WEEKE B SCAND J IMMUNOL SUPPL 1 3 47 973 313 WEEKE B SCAND J IMMUNOL SUPPL 1 2 149 973 314 WEEKE B DAN MED BULL 23 155 976 315 WEINSTEIN RJ J CLIN INVEST 58 190 976 316 WIGLESWORTH FW AM J MED SCI 212 351 946 317 WILLIAMS RJ J MED MICROBIOL 6 409 973 318 WILSON GS TOPLEY WILSONS PRINCIPLES OF 1 368 975 319 WISSLER H HELV PAEDIATR ACTA SUPPL 1 1 3 945 320 WOOD RE AM REV RESPIR DIS 113 833 976 321 WRETLIND B J MED MICROBIOL 6 91 973 322 WRIGHT GL JR IMMUNOL COMMUN 3 35 974 323 WRIGHT PF J INFECT DIS 134 144 976 324 WULFF HR RATIONEL KLINIK 973 325 YOUNG LS J INFECT DIS 126 257 972 326 YOUNG LS J INFECT DIS 126 257 972 327 YOUNG LS CRC CRIT REV CLIN LAB SCI 3 291 972 328 YOUNG LS J INFECT DIS SUPPL 130 111 974 329 ZAVALA DC J ALLERGY CLIN IMMUNOL 56 450 975 330 ZEGERS BJM N ENGL J MED 294 1026 976 331 ZIERDT CH J BACTERIOL 87 1003 964 332 ZIERDT CH J CLIN MICROBIOL 1 521 975 333 ZUELZER WW PEDIATRICS 4 53 949 CT 1 THOMASSEN MJ PEDIATR RES 13 1085 979 2 THOMASSEN MJ J INFECT DIS 140 873 979 3 BERDISCHEWSKY M PEDIATR RES 14 830 980 4 THOMASSEN MJ PEDIATR RES 14 715 980 5 GOSCINIAK G ARCH IMMUNOL THER EXP 28 619 980 6 HODSON ME THORAX 35 801 980 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) 88 149 980 8 BEAUDRY PH J PEDIATR 97 144 980 9 FICK RB CLIN CHEST MED 2 91 981 10 PETERSEN NT ACTA PAEDIATR SCAND 70 623 981 11 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 12 MARKS MI J PEDIATR 98 173 981 13 MOSS RB J PEDIATR 99 215 981 14 MOLLER NE SCAND J INFECT DIS 1981 87 981 15 MOROZ AF ZH MIKROBIO EPIDEMIO IMMUNOBI 1981 6 981 16 SEALE TW ANN CLIN LAB SCI 12 415 982 17 BREMMELGAARD A SCAND J INFECT DIS 14 271 982 18 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 19 BAKER NR IN VITRO 18 369 982 20 SORDELLI DO CLIN IMMUNOL IMMUNOPATHOL 22 153 982 21 BAKER NR CAN J MICROBIOL 28 248 982 22 PERMIN H ALLERGY 37 93 982 23 MOLLER NE EUR J RESPIR DIS 63 130 982 24 SZAFF M ACTA PAEDIATR SCAND 71 821 982 25 ZIELINSKI CC N ENGL J MED 306 486 982 26 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 27 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 28 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 29 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 30 MICHALSEN H ACTA PAEDIATR SCAND SUPPL 301 1982 101 982 31 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 32 ZIMAKOFF J J HOSP INFECT 4 31 983 33 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 34 PERMIN H J ANTIMICROB CHEMOTHER 12 313 983 35 HEILESEN AM SCAND J INFECT DIS 15 271 983 36 SCHILLER NL PEDIATR RES 17 747 983 37 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 31 199 983 38 DORING G INFECT IMMUN 42 197 983 39 LAM JS INFECT IMMUN 42 88 983 40 BROOK I EUR J RESPIR DIS 64 51 983 41 DORING G J INFECT DIS 147 744 983 42 CAPLAN DB REV INFECT DIS 6 S705 984 43 SCHILLER NL CURRENT MICROBIOL 10 185 984 44 ANWAR H FEMS MICROBIOL LETTERS 24 235 984 45 EFTHIMIOU J THORAX 39 150 984 46 MCNEILL WF AM J CLIN PATHOL 81 742 984 47 DORING G ACTA PATH MICROB IMMU SCA (C) 92 307 984 48 ISLES A J PEDIATR 104 206 984 49 THOMASSEN MJ J PEDIATR 104 352 984 50 LYNN AR J BACTERIOL 158 1161 984 51 KLINGER KW EUR J CLIN MICROBIOL 4 201 985 52 ROE EA BURNS 11 252 985 53 BRAUNER A SCAND J INFECT DIS 17 63 985 54 BAEK L J CLIN MICROBIOL 22 229 985 55 WARREN RL ANTIMICROB AGENTS CHEMOTHER 27 468 985 56 REYNOLDS HY DISEASE A MONTH 31 1 985 57 LUZAR MA INFECT IMMUN 50 572 985 58 LUZAR MA INFECT IMMUN 50 577 985 59 WINKLER U KLIN WSCHR 63 490 985 60 OJENIYI B ACTA PATH MICROB IMMU SCA (B) 93 7 985 61 THOMASSEN MJ AM REV RESPIR DIS 131 791 985 62 FICK RB J INFECT DIS 151 589 985 63 ILYUKHIN VI ZH MIKROBIO EPIDEMIO IMMUNOBI 88 986 64 PADGETT PJ CURRENT MICROBIOL 14 187 986 65 PEDERSEN SS SCAND J INFECT DIS 18 133 986 66 FICK RB PEDIATR RES 20 1258 986 67 BRETT MM J CLIN PATHOL 39 1124 986 68 BRETT MM ARCH DIS CHILD 61 1114 986 69 MOSS RB AM REV RESPIR DIS 133 648 986 70 WANG EEL PEDIATR INFECT DIS J 6 256 987 71 DASGUPTA MK J CLIN IMMUNOL 7 51 987 72 HINDERSSON P J GEN MICROBIOL 133 587 987 PN 77009 RN 00591 AN 77178552 AU Holby-N. TI Pseudomonas aeruginosa infection in cystic fibrosis. Relative prevalence of antibodies in serum against cathodic and anodic migrating P. aeruginosa antigens determined by immunoelectrophoretic methods. SO Acta-Pathol-Microbiol-Scand [C]. 1977 Apr. 85(2). P 149-52. MJ CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: co. MN ADOLESCENCE. ADULT. ANTIBODIES-BACTERIAL: an. ANTIGENIC-DETERMINANTS. ANTIGENS-BACTERIAL: an. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. IMMUNOELECTROPHORESIS. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. INFANT. MALE. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im. AB Sera from 119 patients with cystic fibrosis were examined for precipitating antibodies against anodic migrating P. aeruginosa antigens by means of crossed immunoelectrophoresis. The presence of precipitins against cathodic migrating P. aeruginosa antigens was investigated by means of classical immunoelectrophoresis. Thirty-six per cent of the sera contained precipitins against both anodic and cathodic antigens, 20% contained only precipitins against anodic antigens, none contained only precipitins against cathodic antigens, and 44% had no demonstrable P. aeurginosa precipitins. The amount of extra information obtained by combining the results of classical immunoelectrophoresis with the results of crossed immunoelectrophoresis was small. RF 001 AXELSEN NH SCAND J IMMUNOL 5 177 976 002 BURNS MW LANCET 1 270 968 003 BURNS MW MED J AUST 2 697 972 004 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 005 DOGGETT RG INFECT IMMUN 6 628 972 006 GRABAR P BIOCHIM BIOPHYS ACTA 10 193 953 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 010 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 011 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 013 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 014 MAY JR ARCH DIS CHILD 47 908 972 015 WEEKE B SCAND J IMMUNOL SUPPL 1 2 15 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 NEGASSI K CLIN EXP IMMUNOL 38 135 979 3 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 329 979 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 337 979 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 6 EMMETT M J IMMUNOL METH 50 R 65 982 7 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 77010 RN 00592 AN 78057956 AU Reid-L. TI Treatment of mucus hypersecretion in human disease. SO Adv-Exp-Med-Biol. 1977. 89. P 469-76. MJ BRONCHIAL-DISEASES: th. MUCUS: se. MN AIRWAY-OBSTRUCTION: me. ATROPINE: tu. BRONCHIAL-DISEASES: me. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: th. EXUDATES-AND-TRANSUDATES. GLYCOPROTEINS: an. HUMAN. SPUTUM: an. SUPPURATION. VISCOSITY. EX Treatment of mucus hypersecretion in human disease should first be directed at prevention. The hypersecretion of mucus, even in the absence of pus in the sputum or of other signs of infection, is of itself associated with significantly poor lung function. Infection is probably the important stimulus in cystic fibrosis. Certain anti-inflammatory agents are now known to cause retention of mucus within the secretory cells although the rate of synthesis is not raised. It may be that this offers a new way of control. On the other hand, the cells become larger and this may be undesirable. I would prefer to see a study of the effect of drugs on the cellular stages of synthesis and discharge rather than concentration on mucolytic agents. RF 001 BURGI H MED THORAC 21 156 964 002 CHARMAN J BIORHEOLOGY 10 295 973 003 GREGG I PROC ASPEN EMPHYSEM CONF 9TH 235 968 004 HILLIS BR LANCET 1 1230 952 005 JONES R BR J EXP PATHOL 54 229 973 006 KEAL EE POSTGRAD MED J 47 171 971 007 LOPEZ-VIDRIERO MT BULL PHYSIOPATH RESPIR NANCY 9 339 973 008 LOPEZ-VIDRIERO MT THORAX 30 624 975 009 LOPEZ-VIDRIERO MT THORAX 30 543 975 010 MAY JR IN: TAVERNER D 6 968 011 MILLER DL AM REV RESPIR DIS 88 473 963 012 REID L BULL PHYSIOPATH RESPIR NANCY 9 15 973 013 ROBERTS GP ARCH BIOCHEM BIOPHYS 173 528 976 014 STURGESS JM CLIN SCI 43 533 972 015 WHITE JC J PATHOL BACTERIOL 67 105 954 PN 77011 RN 00593 AN 78057939 AU Mitchell-Heggs-P. TI Physical properties of bronchial secretion. SO Adv-Exp-Med-Biol. 1977. 89. P 203-15. MJ BRONCHI: se. SPUTUM. MN ASTHMA: pa. BRONCHIECTASIS: pa. BRONCHITIS: pa. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pa. ELASTICITY. HUMAN. RHEOLOGY. VISCOSITY. EX The Weissenberg rheogoniometer is designed to estimate viscosity at a single applied shear. By varying this shear, the associated detailed variation of viscosity may be studied. The level of viscosity depends more on the macroscopic appearance of the sample than the disease with which it may be associated. This has particular significance in relation to cystic fibrosis. In cystic fibrosis, the sputum viscosity is not abnormally high. It is not higher than that of macroscopically similar sputum from chronic bronchitis or asthma. Whatever the feature differentiating cystic fibrosis from other diseases, it is not the viscosity of the sputum. RF 001 AIACHE JM POUMON COEUR 26 35 970 002 BAMBERG H MED WELT 37 1997 968 003 BLANSHARD G ARCH MIDDX HOSP 5 222 955 004 BRUCE RA BR J CLIN PRACT 22 289 968 005 CHARMAN J BIORHEOLOGY 9 185 972 006 DAVIS SS BIORHEOLOGY 6 11 969 007 FEATHER EA BR J DIS CHEST 64 192 970 008 GOLDFARB H CLIN RES 12 291 964 009 KEAL EE POUMON COEUR 26 51 970 010 KINGHORN FC PHYS MED BIOL 13 455 968 011 LIEBERMAN J AM REV RESPIR DIS 97 654 968 012 MITCHELL-HEGGS PF BULL PHYSIOPATH RESPIR NANCY 9 163 973 013 MITCHELL-HEGGS PF THESIS 974 014 MITCHELL-HEGGS PF PROC EUROP CONG RHEUM 7TH ABS 971 015 MITCHELL-HEGGS PF BIORHEOLOGY 11 417 974 016 PALMER KNV BR J DIS CHEST 64 185 970 017 REID L PROC ROY INST GR BR 43 438 970 018 SHEFFNER AL AM REV RESPIR DIS 90 721 964 019 STRESEMANN E KLIN WOCHENSCHR 42 1165 964 020 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 021 STURGESS JM CLIN SCI 38 145 970 CT 1 MARRIOTT C THORAX 33 545 978 PN 77012 RN 00594 AN 78100225 AU Lheureux-P-R. Isenberg-J-N. Sharp-H-L. Warwick-W-J. TI Gallbladder disease in cystic fibrosis. SO AJR. 1977 Jun. 128(6). P 953-6. MJ CYSTIC-FIBROSIS: co. GALLBLADDER-DISEASES: et. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CHOLELITHIASIS: et, ra. FEMALE. GALLBLADDER-DISEASES: ra. HUMAN. INFANT. MALE. AB In 84 consecutive patients with cystic fibrosis, oral cholecystography was abnormal in 39 (46.4%). The incidence of abnormal cholecystograms increased with patient age. The 26 patients with a nonvisualized gallbladder following double-dosage oral cholecystography were evaluated with intravenous cholangiography; 19 (70.3%) of these were abnormal. Ten patients were found to have calculi, an incidence of 11.9%. Awareness of the high incidence of cholecystographic abnormalities and calculi should be helpful in the evaluation of patients with cystic fibrosis, particularly since symptoms of abdominal pain are frequent in such patients and may lead to radiographic investigations. RF 001 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 002 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 003 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 004 SLOTH K ACTA PAEDIATR SCAND 61 380 972 005 VILLA-VENZANO G RAD MED FAK ZAGREBU 47 747 961 006 MACARINI N CONG NAZIONALE DI RADIOLOGIA 968 007 FEIGELSON J JOURNEES PARISIENNES PEDIATR 77 969 008 VICHI GF FRACASTORO (VERONA) 63 366 970 009 ROVSING H ACTA RADIOL DIAGN STOCKH 14 588 973 010 FEIGELSON J MED CHIR DIG 4 121 975 011 JONES MD J PEDIATR 53 172 958 012 WOOD RE AM REV RESPIR DIS 113 833 976 013 WARWICK WJ J CHRON DIS 28 609 975 014 POGUE RE MINN MED 52 279 969 015 NEWMAN DE PEDIATR RADIOL 1 100 973 016 ZIMMERMAN HJ IN: BECKER FF 5 225 974 017 WEBER AM N ENGL J MED 289 1001 973 018 GOODCHILD MC PROC EWGCF 5TH ANNU MTG 974 019 BENNION LJ N ENGL J MED 294 189 976 020 BAKER HL JR RADIOLOGY 74 239 960 021 MUJAHED Z RADIOLOGY 112 1 974 022 WISE RE RADIOL CLIN NORTH AM 4 521 966 023 BARTURM RJ JAMA 236 1147 976 024 LAPEY A J PEDIATR 84 328 974 025 ISENBERG JN AM J GASTROENTEROL 65 134 976 CT 1 PARK RW GASTROENTEROLOGY 81 1143 981 2 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 3 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 4 ISDALE JM S AFR MED J 62 160 982 5 ABDULKARIM FW GASTROENTEROLOGY 82 758 982 6 HENSCHKE CI J ULTRASOUND MED 2 481 983 7 KRAMER NR J CAN ASSOC RADIOL 34 271 983 8 BASS S GASTROENTEROLOGY 84 1592 983 9 FITZPATRICK SB CHEST 86 863 984 10 HUBBARD VS SEM RESPIR MED 6 299 985 11 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 PN 77013 RN 00595 AN 78142570 AU Patredis-K. TI An evaluation of the sensory integrative development of young children with cystic fibrosis. SO Ala-J-Med-Sci. 1977 Oct. 14(4). P 437. MJ CHILD-DEVELOPMENT. CYSTIC-FIBROSIS: px. MN CHILD. CHILD-PRESCHOOL. HUMAN. SENSATION. EX Studies have shown that children with chronic illnesses are more prone to develop secondary handicaps in the areas of educational achievement, behavior, and psychological maladjustment than are 'normal' children. Besides his physical symptoms, the child with a chronic illness must endure prolonged hospitalization, psychological stresses, decreased activity, and is often denied opportunity for skill practice and peer group activities. All these factors result in an overall decrease in sensory stimulation which could impair perceptual-motor functioning as well as the child's ability to successfully adapt to his disease. Therefore, the purpose of this research was to obtain a general idea of the perceptual-motor or sensory integrative development of children with CF, to determine if there is, in fact, a need for sensory integrative treatment in the care of these children. PN 77014 RN 00596 AN 77109030 AU Andorsky-M. Finley-A. Davidson-M. TI Pediatric gastroenterology 1/1/69-12/31/75: a review. Part I. Hollow viscera and the pancreas. SO Am-J-Dig-Dis. 1977 Jan. 22(1). P 56-68. (REVIEW). MJ GASTROINTESTINAL-DISEASES. MN ABDOMINAL-WALL: ab. ADOLESCENCE. ADULT. ANIMAL. ANUS: ab. CELIAC-DISEASE: di. CHILD. CHILD-PRESCHOOL. COLONIC-DISEASES: co. CONSTIPATION. CYSTIC-FIBROSIS: di. DIARRHEA-INFANTILE: mi. ENTEROCOLITIS-PSEUDOMEMBRANOUS. ESOPHAGEAL-ATRESIA: su. FOOD-HYPERSENSITIVITY: di. HEMORRHAGE-GASTROINTESTINAL: et. HERNIA-HIATAL: su. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-POLYPS. LYMPHANGIECTASIS-INTESTINAL. MALABSORPTION-SYNDROMES. MEGACOLON: et. PANCREATITIS: et. PARENTERAL-HYPERALIMENTATION: ae. PEPTIC-ULCER. PYLORIC-STENOSIS: su. RECTUM: ab. REVIEW. EX Cumulative survival in cystic fibrosis (CF) for three successive 5-year periods between 1952 and 1967 is 35%, 64%, and 77%, respectively. Operative mortality in meconium ileus is not reduced to 25% from 70%. N-Acetylcysteine effectively cleanses the bowel of tenacious meconium plugs when directly injected during laparotomy. However, Mucomist enema alone generally does not suffice. Selected cases have been treated successfully with hyperosmolar Gastrograffin enema. Several screening tests, based on the increased albumin concentrations present in the meconium, are available for CF. The Boehringer-Mannheim test strip is favored, and yielded no false positives in 69,000 tests of meconium. However, 4 of 60 neonates who subsequently proved to have CF had false negative tests. A pathognomonic elevation of sodium and chloride concentrations in the sweat collected by pilocarpine iontophoresis remains the definitive diagnostic test. Bile acid malabsorption occurs in CF but not in celiac disease. The authors postulate that the presence of maldigested materials or the absence of pancreatic enzymes interferes with bile acid absorption. The defect disappears with pancreatic extracts. Substitution of medium-chain triglycerides for dietary long-chain fatty acids reduces steatorrhea in CF. However, the positive effect of MCT on weight gain is transitory. Growth retardation correlates better with the degree of pulmonary disease than with pancreatic insufficiency. RF 001 SILVERBERG M GASTROENTEROLOGY 58 229 970 002 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 002 ROY CC PEDIATR CLIN GASTROENTEROL 975 003 ANDERSON CM PEDIATRIC GASTROENTEROLOGY 975 004 GRYBOSKI J MAJ PROB CLIN PEDIATR 13 450 975 005 MICHENER WM PEDIATR CLIN NORTH AM 14 159 967 006 GRAND RJ PEDIATR CLIN NORTH AM 22 975 007 GRAND RJ GASTROENTEROLOGY 70 790 976 008 HENDREN WH N ENGL J MED 289 456 973 009 LOUW JH LANCET 2 1065 955 010 FAVARA BE AM J DIS CHILD 122 501 971 011 STEVENSON RE J PEDIATR 81 1123 972 012 KOOP CE PEDIATRICS 54 558 974 013 HOFMANN S J PEDIATR SURG 11 257 976 014 MAHOUR GH J PEDIATR SURG 9 373 974 015 PIERETTI R J PEDIATR SURG 9 355 974 016 GROTTE G ACTA PAEDIATR SCAND 60 59 970 017 LOUHIMO I J PEDIATR SURG 4 663 969 018 SCHARLI A J PEDIATR SURG 4 108 969 019$ HUGUENARD JR J PEDIATR 8 45 972 020 SHIM WKT J PEDIATR 76 89 970 021 STEPHENS FD SURG CLIN NORTH AM 50 919 970 022 SANTULLI TV J PEDIATR SURG 5 281 970 023 TAYLOR I J PEDIATR SURG 8 497 973 024 ARHAN P J PEDIATR SURG 11 157 976 025 KERREMANS RPJ AM J DIS CHILD 128 811 974 026 HOLLABAUGH RS J PEDIATR SURG 8 263 973 027 WESSELHOEFT CW PEDIATRICS 44 101 969 028 FRIEDLAND GW AM J ROENTG RAD THER NUCL MED 120 305 974 029 DARLING DB PEDIATRICS 54 450 974 030 CARCASSONNE M J PEDIATR SURG 8 575 973 031 CARRE IJ ARCH DIS CHILD 34 344 959 032 CARRE IJ ACTA PAEDIATR SCAND 61 492 972 033 PRINSEN JE J PEDIATR SURG 10 97 975 034 RANDOLPH JG ANN SURG 180 479 974 035 JEWETT TC JR J PEDIATR SURG 10 757 975 036 WOODWARD ER ANN SURG 173 782 971 037 SEAGRAM CGF J PEDIATR SURG 8 407 973 039 RAVITCH MM ANN SURG 171 641 970 040 TSUCHIDA Y J PEDIATR SURG 9 499 974 041 SPENCER R SURGERY 55 718 964 042 COLLINS REC ARCH DIS CHILD 46 110 971 043 GLEASON WA JR J PEDIATR 85 810 974 044 AMENT ME GASTROENTEROLOGY 68 858 975 045 ROSENTHALL L RADIOLOGY 105 371 972 046 LEONIDAS JC ARCH DIS CHILD 49 21 974 047 JAROS R J PEDIATR 82 42 973 048 SILVERBERG SG AM J DIG DIS 15 617 970 049 SACHATELLO CR GASTROENTEROLOGY 58 699 970 050 RUYMANN FB GASTROENTEROLOGY 57 431 969 051 BUSSEY HJR GUT 11 970 970 052 COLI RD AM J DIG DIS 15 551 970 053 DONALDSON MH PEDIATRICS 48 307 971 054 LOEHR WJ ANN SURG 170 232 969 055 SWENSON O J PEDIATR SURG 8 587 973 056 EARLAM RJ AM J DIG DIS 17 255 972 057 NIXON HH ARCH DIS CHILD 41 147 966 058 TOULOUKIAN RJ J PEDIATR SURG 8 191 973 059 SUSTER G AM J DIS CHILD 119 494 970 060 EHRENPREIS T AM J DIG DIS 16 1032 971 061 SHERRY SN J PEDIATR 46 158 955 062 CAMPBELL PE J PEDIATR SURG 4 410 969 063 USTACH TJ ARCH DIS CHILD 44 694 969 064 VERDER H ACTA PAEDIATR SCAND 62 59 973 065 HOLSCHNEIDER AM J PEDIATR SURG 11 151 976 066 WILCOX MW J PEDIATR SURG 7 168 972 067 APLEY J CHILD WITH ABDOMINAL PAIN 975 068 APLEY J BR MED J 3 7 973 069 OSTER J PEDIATRICS 50 429 972 070 BAYLESS TM PEDIATRICS 47 1029 971 071 DOUGLAS EF J PEDIATR 78 59 971 072 BAKWIN H AM J DIS CHILD 121 179 971 073 LEVINE MD PEDIATRICS 56 412 975 074 OLATAWURA MO ACTA PAEDIATR SCAND 62 358 973 075 DAVIDSON M J PEDIATR 62 261 963 076 BELLMAN M ACTA PAEDIATR SCAND SUPPL 170 1 966 077 BACKLOW NR ANN INTERN MED 76 993 972 078 BISHOP RF LANCET 2 1281 973 079 FLEWETT TH LANCET 2 61 974 080 SCHREIBER DS N ENGL J MED 288 1318 973 081 BISHOP RF LANCET 1 149 974 082 SOUTH MA J PEDIATR 79 1 971 083 GRADY GF N ENGL J MED 285 891 971 084 DUPONT HL N ENGL J MED 285 1 971 085 MERSON MH N ENGL J MED 294 1299 976 086 SMITH HW J MED MICROBIOL 3 403 970 087 MCCRACKEN GH JR J PEDIATR 75 923 969 088 CARPENTER CCJ J PEDIATR 80 874 972 089 GOLDMAN AS J PEDIATR 82 1082 973 090 GINDRAT JJ ACTA PAEDIATR SCAND 61 587 972 091 GRADY GF N ENGL J MED 285 831 971 092 SMITH ER CAN MED ASSOC J 104 1004 971 093 KAZEMI M J PEDIATR 83 646 973 094 BERGSTRAND CG ACTA PAEDIATR SCAND 63 875 974 095 BRADFORD WD ARCH PATHOL 98 17 974 096 EGAN-MITCHELL B ARCH DIS CHILD 47 238 972 097 HAMILTON JR Q J MED 38 135 969 098 MYLOTTE M Q J MED 43 359 974 099 YOUNG WF ARCH DIS CHILD 46 421 971 100 VISAKORPI JK ACTA PAEDIATR SCAND 59 481 970 101 WALKER WA J PEDIATR 83 711 973 102 HOBBS JR LANCET 2 649 969 103 SHINER M GUT 14 1 973 104 JULIUS R J PEDIATR 83 1007 973 105 MOYNAHAN EJ LANCET 2 399 974 106 KAYDEN HJ J PEDIATR 83 993 973 107 ROBERTSON AF PEDIATRICS 55 783 975 108 PITTMAN FE PEDIATRICS 54 81 974 109 VARDY PA PEDIATRICS 55 842 975 110 AMIRHAKIMI GH AM J DIS CHILD 117 178 969 111 GRACEY M ARCH DIS CHILD 45 445 970 112 GERRARD JW ACTA PAEDIATR SCAND SUPPL 234 1 973 113 SHINER M LANCET 1 136 975 114 KUITUNEN P ACTA PAEDIATR SCAND 62 585 973 115 FREIER S J PEDIATR 75 623 969 116 GOLDMAN AS PEDIATRICS 32 425 963 117 KUITUNEN P ARCH DIS CHILD 50 351 975 118 FREIER S LANCET 1 913 973 119 DAVIDSON AG PEDIATRICS 46 632 970 120 SOEPARTO P ARCH DIS CHILD 47 56 972 121 NEWCOMER AD GASTROENTEROLOGY 50 340 966 122 KEIFFER JM PEDIATRICS 56 718 975 123 METZ G LANCET 1 1155 975 124 RODRIGUIZ DE CURET H GASTROENTEROLOGY 59 396 970 125 LIFSHITZ F J PEDIATR 79 760 971 126 RANSOME-KUTI O GASTROENTEROLOGY 68 431 975 127 SAHI T ACTA PAEDIATR SCAND 61 11 972 128 HABTE D ACTA PAEDIATR SCAND 62 649 973 129 MEEUWISSE GW ACTA PAEDIATR SCAND SUPPL 188 969 130 TORRES-PINEDO R J CLIN INVEST 45 1916 966 131 LIFSHITZ F J PEDIATR 77 595 970 132 HIRSCHHORN N J PEDIATR 83 562 973 133 HUANG NN AM J DIS CHILD 120 289 970 134 GEORGE L ARCH DIS CHILD 46 139 971 135 SHAW A J PEDIATR SURG 4 119 969 136 NOBLETT HR J PEDIATR SURG 4 190 969 137 STEPHAN U PEDIATRICS 55 35 975 138 ANON J PEDIATR 88 711 976 139 WEBER AM N ENGL J MED 289 1001 973 140 GRACEY M ARCH DIS CHILD 44 401 969 141 LAPEY A J PEDIATR 84 328 974 142 SIBERT JR ARCH DIS CHILD 50 443 975 143 MALONE JI J PEDIATR 85 825 974 144 RIEMENSCHNEIDER TA PEDIATRICS 41 428 968 145 PENA SDJ J PEDIATR 83 1026 973 146 ATTARD J BR J SURG 56 235 969 147 BONGIOVI JJ J PEDIATR SURG 4 220 969 148 KATTWINKEL J PEDIATRICS 51 55 973 149 MCELROY R AM J MED 52 228 972 150 FARMER RG AM J MED 54 161 973 151 SHWACHMAN H PEDIATRICS 55 86 975 152 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 153 DAVIDSON I CLIN DIAG LAB METHODS 870 974 154 TAUSSIG LM PEDIATRICS 54 229 974 155 FILLER RM N ENGL J MED 281 589 969 156 WILMORE DW J PEDIATR SURG 4 181 969 157 WILMORE DW J PEDIATR 80 88 972 158 BELL MJ J PEDIATR SURG 8 197 973 159 WINICK M J PEDIATR 74 667 969 160 HEIRD WC J PEDIATR 86 2 975 161 PEDEN VH J PEDIATR 81 137 972 162 AVERY GB PEDIATRICS 41 712 968 163 LLOYD-STILL JD AM J DIS CHILD 125 358 973 164 FISCHER JE AM J SURG 125 165 973 165 HEIRD WC J PEDIATR 80 351 972 166 GHADIMI H PEDIATRICS 48 955 971 167 HEIRD WC J PEDIATR 81 162 972 168 COHEN MI IN: WINTERS RW 293 975 169 ROGER R J PEDIATR 86 264 975 170 TOULOUKIAN RJ J PEDIATR 86 270 975 171 ASCH MJ J PEDIATR SURG 7 213 972 172 CORAN AG J PEDIATR SURG 8 801 973 173 GUSTAFSON A ACTA PAEDIATR SCAND 63 177 974 174 WHITE HB J PEDIATR 83 490 973 175 PEDEN VH J PEDIATR 83 305 973 176 FRANTZ ID 3RD J PEDIATR 86 259 975 177 SANTULLI TV PEDIATRICS 55 376 975 178 HOPKINS GB AM J DIS CHILD 120 229 970 179 BOOK LS PEDIATR RES ABST 8 379 974 180 TORMA MJ AM J SURG 126 758 973 181 JOSHI W AM J DIS CHILD 126 113 973 182 STEIN H BR MED J 2 616 972 183 PITT J PEDIATR RES 8 384 974 184 ENGEL RR PEDIATR RES 7 292 973 185 BELL MJ J PEDIATR SURG 8 601 973 186 KRASNA IH J PEDIATR SURG 8 615 973 187 KRASNA IH J PEDIATR SURG 5 200 970 188 EHRENPREIS TH ACTA PAEDIATR SCAND 60 209 971 189 AMENT ME J PEDIATR 86 322 975 190 BINDER V SCAND J GASTROENTEROL 8 161 973 191 MILLER RC AM J DIS CHILD 122 301 971 192 LINDSLEY CB J PEDIATR 84 16 974 193 DEVROEDE GJ N ENGL J MED 285 17 971 194 TRUELOVE SC N ENGL J MED 285 50 971 195 WEEDON DD N ENGL J MED 289 1099 973 196 MCCAFFERY TD PEDIATRICS 45 386 970 197 GOTLIN RW GUT 14 191 973 198 MCCAFFERY TD PEDIATRICS 45 386 970 199 SADEGHI NEJAD A PEDIATRICS 43 870 969 200 DAVIDSON M IN: KIRSNER JB 312 975 201 GUTTMAN FM J PEDIATR SURG 9 115 974 202 HARRIS BH J PEDIATR SURG 9 301 974 203 DE DOMBAL FT GUT 12 519 971 204 STAHLGREN LH JAMA 175 986 961 205 LEVINE AM J PEDIATR SURG 9 715 974 PN 77015 RN 00597 AN 77131731 AU Gewitz-M. Eshaghpour-E. Holsclaw-D-S. Miller-H-A. Kawai-N. TI Echocardiography in cystic fibrosis. SO Am-J-Dis-Child. 1977 Mar. 131(3). P 275-80. MJ CYSTIC-FIBROSIS: co. ECHOCARDIOGRAPHY. PULMONARY-HEART-DISEASE: di. MN ADOLESCENCE. ADULT. CHILD. COMPARATIVE-STUDY. ELECTROCARDIOGRAPHY. HUMAN. PULMONARY-HEART-DISEASE: et. RESPIRATORY-FUNCTION-TESTS. VECTORCARDIOGRAPHY. AB Echocardiograms were obtained for 25 patients with cystic fibrosis (CF) and 20 controls to evaluate this technique as a means of assessment of right ventricular changes in patients with CF. Right ventricular anterior wall thickness per square meter of body surface (RVAW/sq m) and right ventricular internal dimension per square meter of body surface (RVID/sq m) were compared with other techniques for detection of cor pulmonale. Significant correlations existed between both RVAW/sq m and RVID/sq m and the forced vital capacity, forced expiratory volume in one second, midmaximal expiratory flow rate, clinical score of severity of disease, and roentgenographic score of pulmonary involvement. The RVAW/sq m was slightly more sensitive than RVID/sq m; RVAW/sq m thickness on echocardiogram in vivo compared well with actual measurements at autopsy in five patients. No correlations were found between echocardiography and electrocardiograms, vectorcardiograms, thoracic index, or cardiothoracic ratio. RF 001 ROYCE SW PEDIATRICS 8 255 951 002 GOLDRING RM J PEDIATR 65 501 964 003 MOSS AJ MOD PROBL PEDIATR 10 187 967 004 SIASSI B J PEDIATR 78 794 971 005 LIEBMAN J CIRCULATION 35 552 967 006 LIEBMAN J CHEST 63 218 973 007 POPP RL AM J CARDIOL 24 523 969 008 DIXON WJ INTRO STATISTICAL ANALYSIS 204 969 009 MOSS AJ PRACT PEDIATRIC ELECTROCARDIO 58 973 010 SIMON G ARCH DIS CHILD 47 373 972 011 WARING WW THORACIC DEPTH WIDTH CHARTS 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 EPSTEIN ML CIRCULATION 51 1124 975 014 MEYER RA PROG CARDIOVASC DIS 15 341 973 015 HAGAN AD CIRCULATION 48 1221 973 016 GRAMIAK R RADIOLOGY 92 939 969 017 LUNDSTROM NR ACTA PAEDIATR SCAND SUPPL 243 974 018 MEYER RA AM J CARDIOL 30 349 972 019 TAJIK AJ CIRCULATION 46 36 972 020 MEYER RA PEDIATRICS 54 266 974 021 MASON DT PROG CARDIOVASC DIS 12 507 970 CT 1 NUSSBAUM E J PEDIATR 93 931 978 2 NEWTH CJL THORAX 34 428 979 3 ALLEN HD CHEST 75 428 979 4 HIRSCHFELD SS CHEST 75 351 979 5 CHIPPS BE J PEDIATR 95 379 979 6 TSUDA T BR HEART J 44 55 980 7 MORIN DP PEDIATRICS 65 44 980 8 LESTER LA J PEDIATR 97 742 980 9 FOWLER RS J ELECTROCARDIOL 14 319 981 10 JACOBSTEIN MD CHEST 80 399 981 11 NEWTH CJL AM REV RESPIR DIS 124 463 981 12 MOSS AJ PEDIATRICS 70 728 982 13 COATES AL CHEST 82 543 982 14 CHERON G ACTA PAEDIATR SCAND 73 697 984 15 MOSKOWITZ WB PEDIATR PHARMACOL 5 139 985 16 LESTER LA SEM RESPIR MED 6 285 985 17 PANIDIS IP J AM COLL CARDIOL 6 701 985 PN 77016 RN 00598 AN 77239487 AU Ristow-S-C. Condemi-J-J. Stuard-I-D. Schwartz-R-H. Bryson-M-F. TI Systemic amyloidosis in cystic fibrosis. SO Am-J-Dis-Child. 1977 Aug. 131(8). P 886-8. MJ AMYLOIDOSIS: co. CYSTIC-FIBROSIS: co. MN ADULT. AMYLOIDOSIS: fg. CASE-REPORT. CYSTIC-FIBROSIS: fg. FEMALE. HUMAN. MALE. SUPPORT-U-S-GOVT-P-H-S. AB We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis. RF 001 BRIGGS GW ANN INTERN MED 55 953 961 002 COHEN AS N ENGL J MED 277 522 967 003 BRANDT K AM J MED 44 955 968 004 BROWNSTEIN MH SOUTH MED J 64 491 971 005 KYLE RA MEDICINE (BALTIMORE) 54 271 975 006 MISSMAHL HP FORTSCHR MED 85 621 967 007 EHRLICH JC AM J PATHOL 38 49 961 008 COOPER JH J CLIN PATHOL 22 410 969 009 AUERBACH O ARCH INTERN MED 74 244 944 010 WALD MH ANN INTERN MED 43 383 955 011 WIMAN L SCAND J RESPIR DIS 55 5 974 012 AREAN VM J CLIN PATHOL 36 341 961 013 SHAPIRO ST ARCH OTOLARYNGOL 93 203 971 014 JAMES PD J CLIN PATHOL 25 683 972 015 KENNEDY JS Q J MED 43 127 974 016 LANDING BH ANN NY ACAD SCI 93 518 962 CT 1 PRIOR J BR J DIS CHEST 74 84 980 2 KYLE RA INT J DERMATOL 20 75 981 3 VILASECA J AM J DIS CHILD 135 667 981 4 MARHAUG G ACTA PAEDIATR SCAND 72 861 983 5 BIBERSTEIN M AM J CLIN PATHOL 80 752 983 6 MICHALSEN H EUR J RESPIR DIS 66 306 985 7 CANCIANI M ACTA PAEDIATR SCAND 74 613 985 8 CASTILE R AM J DIS CHILD 139 728 985 9 KIRSCHNER BS J PEDIATR GASTROENTEROL NUTR 5 816 986 10 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 11 TRAVIS WD AM J CLIN PATHOL 85 419 986 12 MCGLENNEN RC ARCH PATHOL LAB MED 110 879 986 PN 77017 RN 00599 AN 77264053 AU Orenstein-D-M. Boat-T-F. Stern-R-C. Tucker-A-S. Charnock-E-L. Matthews-L-W. Doershuk-C-F. TI The effect of early diagnosis and treatment in cystic fibrosis: a seven-year study of 16 sibling pairs. SO Am-J-Dis-Child. 1977 Sep. 131(9). P 973-5. MJ CYSTIC-FIBROSIS: di, th. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg. EXPIRATORY-RESERVE-VOLUME. FEMALE. HUMAN. INFANT. LUNG: pp. MALE. PROGNOSIS. RESIDUAL-VOLUME. THORACIC-RADIOGRAPHY. TOTAL-LUNG-CAPACITY. SUPPORT-U-S-GOVT-P-H-S. AB Data on 16 sibling pairs with cystic fibrosis were analyzed to test the hypothesis that early treatment of this condition improves prognosis. Younger siblings' conditions were diagnosed before 1 year of age, usually before the onset of pulmonary disease. Older siblings' conditions were diagnosed after 1 year of age and after the onset of pulmonary disease. Although the sibling pairs received similar treatment, comparison at 7 years of age showed that the younger siblings had significantly better chest roentgenogram scores, total clinical scores, residual lung volumes, and ratios of residual volume to total lung volume. Younger siblings also required fewer hospital admissions to control their lung disease. The results suggest that, in general, early initiation of therapy is beneficial for patients with cystic fibrosis. RF 001 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 002 GEORGE L ARCH DIS CHILD 46 139 971 003 HUANG NN AM J DIS CHILD 120 289 970 004 SHWACHMAN H PEDIATRICS 46 335 970 005 DOERSHUK CF PEDIATRICS 36 675 965 006 GELLIS SS YEARBOOK OF PEDIATRICS 164 976 007 MATTHEWS LW J PEDIATR 65 558 964 008 MATTHEWS LW IN: MANGOS JA 303 973 009 DOERSHUK CF J PEDIATR 65 677 964 010 HELLIESEN PJ PEDIATRICS 22 80 958 011 DUBOIS AB J CLIN INVEST 35 322 956 012 LEVISON H IN: MANGOS JA 3 976 013 DI SANTAGNESE PA N ENGL J MED 277 1287 967 014 LAPEY A J PEDIATR 84 328 974 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 PHELAN PD MED J AUST 1 261 979 3 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 4 RYLEY HC ARCH DIS CHILD 54 92 979 5 SANTAGNESE PAD AM J MED 66 121 979 6 WOOD RE SOUTH MED J 72 189 979 7 JAKEL HP BIOL ZENTRALBL 98 55 979 8 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 9 TRAVERT G J GENET HUM 28 141 980 10 WARWICK WJ J CHRON DIS 33 685 980 11 LITTLEWOOD JM PRACTITIONER 224 305 980 12 ROMEO G RIV ITAL PEDIATR 7 201 981 13 TRAVERT G NOUV PRESSE MED 10 2093 981 14 EVANS RT J CLIN PATHOL 34 911 981 15 CONGDEN PJ ARCH DIS CHILD 56 708 981 16 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 17 CROSSLEY JR CLIN CHIM ACTA 113 111 981 18 GITZELMANN R HELV PAEDIATR ACTA 36 493 982 19 PHELAN PD ARCH DIS CHILD 57 779 982 20 HELLSING K ACTA PAEDIATR SCAND 71 827 982 21 STERN RC GASTROENTEROLOGY 82 707 982 22 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 23 FRATES RC AM J DIS CHILD 136 279 982 24 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 25 ORENSTEIN DM JAMA 248 2113 982 26 ROSENSTEIN BJ JAMA 248 2113 982 27 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 28 PEDERZINI F RIV ITAL PEDIATR 9 445 983 29 TRAVERT G ARCH FR PEDIATR 40 295 983 30 ANON PEDIATRICS 72 741 983 31 WILCKEN B J PEDIATR 102 383 983 32 GROBE H MONATSSCHR KINDERHEILKD 131 806 983 33 POSSELT HG ATEMWEGS LUNGENKRANKH 10 345 984 34 BLYTHE SA CLIN BIOCHEM 17 277 984 35 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 36 LEWIS MI S AFR MED J 65 641 984 37 CASSIO A ACTA PAEDIATR SCAND 73 554 984 38 FARRELL PM PEDIATRICS 73 115 984 39 REARDON MC J PEDIATR 105 271 984 40 ORENSTEIN DM SEM RESPIR MED 6 252 985 41 NAYLOR EW SEM PERINATOL 9 232 985 42 BERKIN KE EUR J RESPIR DIS 67 103 985 43 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 44 ABMAN SH J PEDIATR GASTROENTEROL NUTR 5 393 986 45 MAHANEY MC ARCH ORAL BIOL 31 363 986 46 DUHAMEL JF ARCH FR PEDIATR 43 229 986 47 DANKERTROELSE JE ACTA PAEDIATR SCAND 76 209 987 PN 77018 RN 00600 AN 77108964 AU Bruns-W-T. Connell-T-R. Lacey-J-A. Whisler-K-E. TI Test strip meconium screening for cystic fibrosis. SO Am-J-Dis-Child. 1977 Jan. 131(1). P 71-3. MJ CYSTIC-FIBROSIS: di. INDICATORS-AND-REAGENTS. MASS-SCREENING. MECONIUM: an. PHENOLPHTHALEINS: du. REAGENT-STRIPS. MN ALBUMINS: an. FALSE-NEGATIVE-REACTIONS. FALSE-POSITIVE-REACTIONS. FEMALE. HUMAN. INFANT-NEWBORN. SWEAT: an. WISCONSIN. AB The Boehringer-Mannheim Corporation (BMC) strip test is extremely reliable in indicating an albumin content above 20 mg/gm of dried meconium. All infants born during one year in 14 Milwaukee area hospitals were tested. Of 16,224 newborns, two were diagnosed correctly as suffering from cystic fibrosis and two were missed. False-positive tests were obtained in 0.9% of infants (prematurity, melena, gastroschisis, and intrauterine infection). The strip test is, at present, the best available but not the perfect screening method for cystic fibrosis. RF 001 GREEN MN PEDIATRICS 41 989 968 002 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 003 PROSSER R ARCH DIS CHILD 49 597 974 004 STEPHAN U PEDIATRICS 55 35 975 005 KAISER D HELV PAEDIATR ACTA 29 51 974 006 KOLLBERG H ARCH DIS CHILD 47 836 972 007 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 008 FRANKENBURG WK PEDIATRICS 54 612 974 009 NORTH AF JR PEDIATRICS 54 631 974 010 DI SANTAGNESE PA PEDIATRICS 15 683 955 011 HOLTZMAN NA J PUBLIC HEALTH 64 775 974 012 HOLTZMAN NA JAMA 229 667 974 CT 1 BRUNS WT AM J DIS CHILD 131 853 977 2 RYLEY HC MONOGR PAEDIATR 10 151 979 3 TRAVERT G J GENET HUM 28 141 980 4 TRAVERT G NOUV PRESSE MED 10 2093 981 5 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 PN 77019 RN 00601 AN 78078190 AU Colon-A-R. TI Hepatic steatosis in children. SO Am-J-Gastroenterol. 1977. 68(3). P 260-9. (REVIEW). MJ FATTY-LIVER. MN ADOLESCENCE. AFLATOXINS: po. ALPHA-1-ANTITRYPSIN: df. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FATTY-LIVER: et, me, th. FATTY-LIVER-ALCOHOLIC. GANGLIOSIDOSIS: co. GLYCOGENOSIS: co. HISTIOCYTOSIS-X: co. HUMAN. HYPERLIPIDEMIA: co. INFANT. INFANT-NEWBORN. INFLAMMATION: co. NUTRITION-DISORDERS: co. REVIEW. TOXINS: po. TRIGLYCERIDES: me. EX Childhood hepatic steatosis is a commonly and generally benign reaction that occurs in many pathophysiologic states. Steatosis can result from the following mechanism(s): impaired TGC clearance; increased hepatic lipogenesis; decreased FA oxidation; and increased lipolysis. Cystic fibrosis and syndromes producing pancreatic insufficiency (pancreatitis) result in a variable degree of steatosis most probably dependent on the nutritional and hormonal state of the patient. Patients with cystic fibrosis have impaired glucagon and insulin release which alters their fat metabolism. The treatment of hepatic steatosis is aimed at the primary cause. RF 001 HARTFORD WS IN: GALL EA 113 973 002 HOYUMPA AM AM J DIG DIS 20 1142 975 003 GOLDRICK RB IN: JOHNSON AR 501 971 004 HAVEL RJ FED PROC 34 2250 975 005 GLUECK CJ N ENGL J MED 292 1347 975 006 LIEBER CS HOSP PRACT 12 73 977 007 LIEBER CS N ENGL J MED 288 356 973 008 JUDAH JD AM J MED 49 609 970 009 WAKIL SJ IN: WAKIL SJ 1 970 010 WOGAN GN ANN NY ACAD SCI 174 623 970 011 REES KR GUT 7 205 966 012 BUTLER WH BR J CANCER 18 756 964 013 BOURGEOIS CH LAB INVEST 24 206 971 014 AMLA C AM J CLIN NUTR 24 609 971 015 COLON AR AM J DIG DIS 19 1091 974 016 LAMPE K PLANT TOXICITY AND DERMAT 131 973 017 ANON NAT COMM MARIJUANA DRUG ABUSE 973 018 RYBACK RS N ENGL J MED 293 719 975 019 HASSAL CH WEST INDIAN MED J 4 83 955 020 CORREDOR CK PROC NAT ACAD SCI USA 58 2299 967 021 RUBIN E AM J DIS CHILD 119 132 970 022 COMBES B PROG LIVER DIS 4 589 972 023 KERN WH AM J CLIN PATHOL 61 763 974 024 SOPER RT J PEDIATR SURG 10 51 975 025 BUCHWALD H CURR PROB SURG 12 35 975 026 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 027 SHINGLETON WW MALABSORPTION SYNDROMES 79 968 028 MORENS DM AM J DIS CHILD 128 401 974 029 STAHL M J PEDIATR 84 821 974 030 OLEFSKY JM AM J MED 57 551 974 031 CAHILL G N ENGL J MED 282 668 970 032 ALPERS DH IN: SCHIFF L 824 975 033 SEGAL S IN: STANBURY JB 174 972 034 HOWELL RR IN: STANBURY JB 149 972 035 FORGET P PEDIATR RES 8 114 974 036 LEES RS N ENGL J MED 284 186 971 037 BRICKER LA MED CLIN NORTH AM 55 403 971 038 FROESCH ER IN: STANBURY JB 131 972 039 WADLINGTON WB PEDIATRICS 51 192 973 040 SATRAN L J PEDIATR 75 39 969 041 PEREMAN J J PEDIATR 69 1108 966 042 PARR J PEDIATRICS 35 770 965 043 GAUTIER E HELV MED ACTA 35 423 970 044 MORROW G IN: NYHAN WL 61 974 045 STREETEN D JAMA 232 944 975 046 BUIST NRM IN: NYHAN WL 160 974 047 STERNLEID I ANN INTERN MED 76 59 972 048 EADE MN ANN INTERN MED 72 475 970 049 STROLE WE N ENGL J MED 289 964 973 050 MOROZ SP J PEDIATR 88 19 976 051 DEHNER LP PEDIATRIC SURGICAL PATHOLOGY 332 975 052 SILVERSTEIN MN N ENGL J MED 282 1 970 053 FRANKEN FH IN: SCHNAFFNER F 117 974 054 COLON AR PEDIATR PORTFOLIO 3 8 975 055 PARTIN JC N ENGL J MED 285 1339 971 057 FAVARA BE MED COM 973 058 COHEN MI IN: WINTERS RW 293 975 059 BROWN RE N ENGL J MED 286 787 972 060 MASSARRAT S ACTA HEPATOGASTROENTEROL 21 176 974 CT 1 DONZELLI F RIV ITAL PEDIATR 7 171 981 PN 77020 RN 00602 AN 77219194 AU Taussig-L-M. TI Isoamylase abnormalities in cystic fibrosis [letter]. SO Am-J-Hum-Genet. 1977 Jul. 29(4). P 408-9. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: me. ISOAMYLASE: me. MN HUMAN. POLYMORPHISM-GENETICS. EX The article by Townes et al. provides confirmation of our initial studies with reference to the isoamylase patterns of serum, duodenal fluid, and urine in patients with cystic fibrosis (CF). However, the impression given by Townes et al. in their article is that their data are the first demonstration of isoamylase abnormalities in cystic fibrosis. I wish to point out that out previous work published 2 years earlier and not referenced by Townes et al. demonstrated that CF patients had decreased amounts of pancreatic isoamylase in their serum and urine and that the duodenal aspirates in CF patients contained both salivary and pancreatic amylase. We concluded, as eluded to by Townes et al., that salivary amylase in the duodenal fluid was probably secondary to the intubation procedure. RF 001 TOWNES PL AM J HUM GENET 28 378 976 002 TAUSSIG LM PEDIATRICS 54 229 974 003 TAUSSIG LM PEDIATRICS 54 229 974 004 TOWNES PL AM J HUM GENET 28 378 976 PN 77021 RN 00603 AN 77264147 AU Scott-J. Elias-E. Moult-P-J. Barnes-S. Wills-M-R. TI Rickets in adult cystic fibrosis with myopathy, pancreatic insufficiency and proximal renal tubular dysfunction. SO Am-J-Med. 1977 Sep. 63(3). P 488-92. MJ CYSTIC-FIBROSIS: co. KIDNEY-TUBULES-PROXIMAL: pp. MUSCULAR-DISEASES: et. PANCREATIC-DISEASES: et. RICKETS: et. MN ADULT. CASE-REPORT. HUMAN. HYPERPARATHYROIDISM-SECONDARY: et. KIDNEY-DISEASES: et. LIVER-CIRRHOSIS-BILIARY: et. MALE. MUSCULAR-DISEASES: dt. RICKETS: dt. VITAMIN-D: me, tu. AB Rickets is reported in a 19 year old white man with cystic fibrosis in whom pancreatic and hepatic involvement was advanced. There was evidence of secondary hyperparathyroidism with proximal renal tubular acidosis, aminoaciduria, phosphaturia and hypophosphatemia. Treatment with oral pancreatic and parenteral vitamin D supplements led to full recovery of the rachitic syndrome and the proximal renal tubular dysfunction. RF 001 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 002 LAPEY A J PEDIATR 84 328 974 003 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 004 COATES EO JR DIS CHEST 49 195 966 005 HUNTON DB GASTROENTEROLOGY 50 99 966 006 TYSON KRT J PEDIATR SURG 3 271 968 007 HARRIES JT ARCH DIS CHILD 46 341 971 008 UNDERWOOD BA PEDIATR RES 6 26 972 009 JAMES O GUT 14 582 973 010 SPROUL A J PEDIATR 65 664 964 011 SIMOPOULOS AP CF CLUB ABST 13 971 012 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 013 SIWE SA DTSCH ARCH KLIN MED 173 339 932 014 SHWACHMAN H AM J DIS CHILD 92 347 956 015 CLARK ML IN: MCCHOLL I 209 975 016 WEBER AM GUT 17 295 976 017 STERN RC GASTROENTEROLOGY 70 645 976 018 MULDOWNEY FP Q J MED 37 517 968 019 MULDOWNEY FP Q J MED 40 487 971 020 SCHOTT GD LANCET 1 626 976 021 PATTEN BM ANN INTERN MED 80 182 974 022 SUNG JH EXP NEUROL 23 567 963 023 MALAMUD N ANN NY ACAD SCI 52 135 949 CT 1 SITRIN M ARCH INTERN MED 138 886 978 2 SANTAGNESE PAD AM J MED 66 121 979 3 CHASE HP J PEDIATR 95 337 979 4 MISCHLER EH AM J DIS CHILD 133 632 979 5 MEREDITH SC CLIN ENDOCRINOL METAB 9 131 980 6 BOROVIKOVA TM TER ARKH 52 125 980 7 ELIAS E LANCET 2 1319 981 8 DUNCOMBE VM CLIN GASTROENTEROL 10 653 981 9 PITT MJ RADIOL CLIN NORTH AM 19 581 981 10 PIERIDES AM DRUGS 21 241 981 11 CONGDEN PJ ARCH DIS CHILD 56 708 981 12 PARK RW GASTROENTEROLOGY 81 1143 981 13 PALING MR SKELETAL RADIOL 8 63 982 14 ABRAMOWSKY CR HUM PATHOL 13 934 982 15 DIBBLE JB Q J MED 53 119 984 16 HANLY JG Q J MED 56 377 985 17 MINGUELLA JF MED CLIN 84 700 985 18 FRIEDMAN HZ GASTROENTEROLOGY 88 808 985 PN 77022 RN 00604 AN 77132666 AU Sahu-S. Lynn-W-S. TI Lipid composition of airway secretions from patients with asthma and patients with cystic fibrosis. SO Am-Rev-Respir-Dis. 1977 Feb. 115(2). P 233-9. MJ ASTHMA: me. CYSTIC-FIBROSIS: me. LIPIDS: se. LUNG: se. MN ADOLESCENCE. ADULT. CERAMIDES: se. CHILD. CHOLESTEROL: se. DIGLYCERIDES: se. GLYCOLIPIDS: an, se. HUMAN. IRRIGATION. LUNG: an. PHOSPHATIDYLCHOLINES: se. PHOSPHATIDYLETHANOLAMINES: se. PHOSPHOLIPIDS: an, se. TRIGLYCERIDES: se. SUPPORT-U-S-GOVT-P-H-S. AB Lipids from the particulate material obtained from pulmonary lavage of patients with asthma and cystic fibrosis were isolated and characterized. In both cases, lipids constituted 30 to 40 per cent of the dry insoluble material and phosphatidylcholine was the predominant lipid. Significant amounts of phosphatidylethanolamine and phosphatidyglycerol were present. Hexosyl ceramides, spingomyelin, phosphatidylinositol, phosphatidylserine, and lyso(bis)phosphatidic acid were present as minor lipid components. Appreciable quantities of neutral lipids were also found. Significant amounts of lysophosphatides and free fatty acids were present only in the lavage sediment from patients with asthma, and not in patients with cystic fibrosis. Phosphatidylcholine, lysophosphatidycholine, sphingomyelin, and phosphatidylglycerol of asthmatic lavage, but not cystic lavage, were highly saturated, containing mostly palmitic acid. RF 001 KYLSTRA JA AM REV RESPIR DIS 103 651 971 002 KING RJ AM J PHYSIOL 223 715 972 003 BAXTER CF LIPIDS 4 243 969 004 ROONEY SA BIOCHIM BIOPHYS ACTA 360 56 974 005 CHERNICK WS PEDIATRICS 24 739 959 006 MATTHEWS LW AM REV RESPIR DIS 88 199 963 007 ROSENLUND ML NATURE 251 719 974 008 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 009 RAMIREZ RJ AM J MED 45 502 968 010 LEWIS RW LIPIDS 6 859 971 011 GALLAI-HATCHARD JJ BIOCHIM BIOPHYS ACTA 116 532 966 012 ADAMS EP CHEM PHYS LIPIDS 1 368 967 013 SAHU S AM REV RESPIR DIS 114 177 976 014 FOLCH J J BIOL CHEM 226 497 957 015 ROUSER G METHODS ENZYMOL 14 272 969 016 ESSELMAN WJ METHODS ENZYMOL 28 140 972 017 VIOQUE E J AM OIL CHEM SOC 39 63 962 018 SKIPSKI VP METHODS ENZYMOL 14 530 969 019 ROONEY SA J LIPID RES 16 418 975 020 TARLOV AR J BIOL CHEM 240 49 965 021 DITMER JC METHODS ENZYMOL 14 482 969 022 BARTLETT GR J BIOL CHEM 234 466 959 023 FARKAS J J LIPID RES 14 344 973 024 VANHANDEL E J LAB CLIN MED 50 152 957 025 RUDEL LL J LIPID RES 14 364 973 026 LAUTER CJ J LIPID RES 3 136 962 027 TAO RVP J LIPID RES 14 16 973 028 HARLAN WR AM J PHYSIOL 211 855 966 029 MIKOLASEV V LIPIDS 9 827 974 030 GLUCK L PEDIATR RES 1 237 967 031 MORGAN TE BIOCHIM BIOPHYS ACTA 106 403 965 032 GRAY GM BIOCHIM BIOPHYS ACTA 144 519 967 033 HENDERSON RF LIPIDS 7 492 972 034 KING RJ AM J PHYSIOL 224 788 973 035 GODINEZ RI BIOCHEMISTRY 14 830 975 036 HALLMAN M J LIPID RES 17 257 976 037 MASON RJ J CLIN INVEST 51 2399 972 CT 1 CREETH JM BR MED BULL 34 17 978 2 LOPEZVIDRIERO MT BR MED BULL 34 63 978 3 ROUSSEL P LUNG 154 241 978 4 SAHU S BIOCHEM J 173 565 978 5 MAWHINNEY TP PEDIATR RES 13 760 979 6 LYNN WS BULL EUR PHYSIOPATH RESP 15 P 18 979 7 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 8 SAHU SC INFLAMMATION 4 107 980 9 CASSINO RJJ PEDIATR RES 14 1212 980 10 ABBRUZZESE A ITAL J BIOCHEM 29 450 980 11 BOAT TF FED PROC 39 3067 980 12 LOPEZVIDRIERO MT CHEST 80 799 981 13 MACONE AB N ENGL J MED 304 1445 981 14 ROGIERS V PEDIATR RES 16 761 982 15 WOODWARD H BIOCHEMISTRY 21 694 982 16 BERTHOLON JF BIOMED PHARMACOTHER 36 189 982 17 JOUANEL P ANN BIOL CLIN 40 255 982 18 SLOMIANY BL J DENT RES 61 1163 982 19 SLOMIANY A BIOCHIM BIOPHYS ACTA 710 106 982 20 WITAS H CARBOHYD RES 120 67 983 21 SLOMIANY A J BIOL CHEM 258 8535 983 22 SLOMIANY A BIOCHIM BIOPHYS ACTA 750 253 983 23 ROGIERS V PEDIATR RES 18 704 984 24 HENDERSON RF ENVIRON HEALTH PERSPECT 56 115 984 25 STELANDER M EUR J RESPIR DIS 65 5 984 26 COLES SJ CIBA FOUND SYMP 109 40 984 27 JOZWIAK Z BIOCHIM BIOPHYS ACTA 802 282 984 28 BHASKAR KR EXP LUNG RES 9 289 985 29 RAMI J BULL EUR PHYSIOPATH RESP 21 331 985 30 RAO GA TOXICOL LETT 29 207 985 31 WIDDICOMBE JG EUR J RESPIR DIS 67 1 985 32 MILLER FJ TOXICOL APPL PHARMACOL 79 11 985 33 GILLJAM H SCAND J CLIN LAB INVEST 46 511 986 PN 77023 RN 00605 AN 77219997 AU Wanner-A. TI Clinical aspects of mucociliary transport. SO Am-Rev-Respir-Dis. 1977 Jul. 116(1). P 73-125. (REVIEW). MJ CILIA: ph. LUNG: ph. MUCUS: ph. MN AIR-POLLUTANTS-ENVIRONMENTAL: ae. ANESTHETICS: pd. ANIMAL. ASTHMA: pp. BRONCHITIS: pp. CHRONIC-DISEASE. CILIA: cy, de. CYSTIC-FIBROSIS: pp. HUMAN. LUNG: cy, de, pp. MUCUS: cy, de. OXYGEN. RESPIRATORY-TRACT-INFECTIONS: pp. REVIEW. RHEOLOGY. SMOKING: pp. SYMPATHOMIMETICS: pd. WEATHER. EX It has long been believed that the mucociliary apparatus of the airways serves to remove inhaled particulate matter from the tracheobronchial mucosa, thereby contributing to pulmonary host defense. This review deals mainly with factors affecting mucociliary transport that may be of clinical importance. The information available at present strongly suggests that mucociliary transport, at least in central airways, is impaired in most patients with cystic fibrosis; however, it is still unclear whether this disorder is a nonspecific feature of the chronic inflammatory airway disease or represents an essential factor in the pathogenesis of the pulmonary abnormalities. RF 001 SLEIGH MA BIOLOGY OF CILIA AND FLAG 962 002 GRAY C CILIARY MOVEMENT 928 003 RIVERA JA CILIA CILIATED EPITHELIUM AND 962 004 KINOSITA H PHYSIOL REV 47 53 967 005 KILBURN KH AM REV RESPIR DIS 93 184 966 006 HILDING AC MINN MED 50 916 967 007 KILBURN KH YALE J BIOL MED 40 339 968 008 KILBURN KH AM REV RESPIR DIS 98 449 968 009 OKESON GC MAYO CLIN PROC 45 361 970 010 ASMUNDSSON T IN: DULFANO MJ 107 973 011 DELAHUNTY JE J LARYNGOL OTOL 83 803 969 012 LEESON TS J ANAT 98 183 964 013 VON HAYCK H HUMAN LUNG 960 014 GILLESPIE JR AM REV RESPIR DIS 95 477 967 015 RHODIN J ANN OTOL RHINOL LARYNGOL 68 964 959 016 ALEXANDER I THORAX 30 171 975 017 RHODIN JAG AM REV RESPIR DIS SUPPL 3 93 1 966 018 YONEDA K AM REV RESPIR DIS 114 837 976 019 SERAFINI SM BULL PHYSIOPATH RESPIR NANCY 12 415 976 021 VAN AS A ENVIRON RES 7 1 974 022 VANAS A S AFR MED J 46 347 972 023 HOLMA B ARCH ENVIRON HEALTH 18 330 969 024 RHODIN J Z ZELLFORSCH MIKROSK ANAT 44 415 956 025 FAWCETT DW J MORPHOL 94 221 954 026 BARBER VC Z ZELLFORSCH MIKROSK ANAT 84 269 968 027 GIBBONS IR J BIOPHYS BIOCHEM CYTOL 7 697 960 028 GIBBONS IR J BIOPHYS BIOCHEM CYTOL 11 179 961 029 ROTH LE J CELL BIOL 20 249 964 030 DULFANO MJ IN: DULFANO MJ 3 973 031 SPICER SS IN: DULFANO MJ 22 973 032 ELFTMAN AG AM J ANAT 72 1 943 033 LARSELL O AM J ANAT 52 125 933 034 EBERT RV AM REV RESPIR DIS 114 567 976 035 KILBURN KH IN: DULFANO MJ 69 973 036 LUCAS AM ARCH OTOLARYNGOL 20 518 934 037 NOWELL JA AM REV RESPIR DIS 103 313 971 038 SADE J AM REV RESPIR DIS 102 48 970 039 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 040 BARCLAY AE J PHYSIOL (LOND) 90 482 937 041 HILDING AC AM J PHYSIOL 191 404 957 042 LIGHTOWLER NM BR J EXP PATHOL 50 139 969 043 PROCTOR DF AM REV RESPIR DIS 115 97 977 044 WILHELM DL J PATHOL BACTERIOL 67 361 954 045 BATTISTA SP TOXICOL APPL PHARMACOL 22 56 972 046 BANG FB AM REV RESPIR DIS 93 142 966 047 HILDING AC ACTA OTOLARYNGOL STOCKH 57 352 964 048 LUCAS AM IN: KOWDRY EV 409 932 049 WILSON GB TRANS AM MICROSC SOC 94 43 975 050 SATIR P IN: SLEIGH MA 131 974 051 PITELKA DR IN: SLEIGH MA 437 974 052 GIBBONS IR J CELL BIOL 26 707 965 053 SAAVEDRA S EXP CELL RES 90 439 975 054 USUKI I SCI REP RES INST TOHOKU UNIV 26 65 959 055 ALEXANDROV VY DOKL AKAD NAUK SSSR 110 457 956 056$ BORHAUS EF SCIENCE 92 553 953 057 GOSSELIN RE AM REV RESPIR DIS 93 41 966 058 GRAY J PROC R SOC LOND BIOL 93 104 922 059 SLEIGH MA AM REV RESPIR DIS 93 16 966 060 KNIGHT-JONES EW Q J MICR SCI 95 503 954 061 BROKAW CJ AM REV RESPIR DIS 93 32 966 062 IRAVANI J PFLUEGERS ARCH 305 199 969 063 BAUER V Z ALLG PHYSIOL 10 230 910 064 AIELLO EL SCIENCE 146 1692 964 065 KINOSITA H J FAC SCI UNIV TOKYO 7 1 954 066 KINOSITA H J FAC SCI UNIV TOKYO 10 304 963 067 KAMADA T J FAC SCI UNIV TOKYO 2 285 931 068 UEDA K ANNOT ZOOL JAPON 34 99 961 069 MURAKAMI A NATURE 257 48 975 070 PEHLE K ARCH EXP PATHOL PHARMAKOL 159 452 931 071 MCDONALD JF AM J PHYSIOL 85 395 928 072 RAZANSKY N RUSS FIZIOL Z 9 556 926 073 BRODY JS CLIN RES 29 507 971 074 REID L ARCH ENVIRON HEALTH 10 265 965 075 REID L BULL PHYSIOPATH RESPIR NANCY 9 15 973 076 TOREMALM NG ACTA OTOLARYNGOL STOCKH SUPPL 158 43 960 077 GROTE JJ ACTA OTOLARYNGOL STOCKH 79 124 975 078 BOAT TF IN: DULFANO MJ 243 973 079 BARTON AD ARCH INTERN MED 131 140 973 080 NEGUS VE ACTA OTOLARYNGOL STOCKH 56 204 963 081 LITT M ARCH INTERN MED 126 417 970 082 RICHARDSON JB CLIN RES 23 647A 975 083 OLVER RE AM REV RESPIR DIS 112 811 975 084 JENSSEN AO SCAND J RESPIR DIS 54 290 973 085 SHARPEY W EDINB MED SURG J 34 113 830 086 LOMMEL F DTSCH ARCH KLIN MED 94 265 908 087 STEWART WC AM J PHYSIOL 152 1 948 088 HILDING AC ANN OTOL RHINOL LARYNGOL 54 725 945 089 HILDING AC N ENGL J MED 256 34 957 090 IRAVANI J BULL PHYSIOPATH RESPIR NANCY 9 397 975 092 HIRSCH JA ARCH ENVIRON HEALTH 30 249 975 093 SACKNER MA ANN INTERN MED 82 40 975 094 CAMNER P ARCH ENVIRON HEALTH 24 82 972 095 CAMNER P AM REV RESPIR DIS 112 807 975 097 KING MA AM REV RESPIR DIS 110 740 974 098 BARNETT B ANN NY ACAD SCI 130 891 966 099 DULFANO MJ AM REV RESPIR DIS 112 341 975 100 PUCHELLE E BULL PHYSIOPATH RESPIR NANCY 12 771 976 101 ADLER K J LAB CLIN MED 88 22 976 102 SLEIGH MA NATURE 191 931 961 103 MILLER CE J EXP BIOL 49 617 968 104 IRAVANI J ARCH PHARMAKON 32 248 975 105 IRAVANI J J PATHOL 106 81 972 106 KINOSITA H ANNOT ZOOL JAPON 25 8 952 108 FRIEDMAN M AM REV RESPIR DIS 115 67 977 109 GOLDSTEIN SF IN: SLEIGH MA 111 974 110 RIVERA JA CILIA CILIATED EPITHELIUM AND 962 112 CORSSEN HL TEX REP BIOL MED 16 194 958 113 BALLENGER JJ AM REV RESPIR DIS 93 61 966 114 BALLENGER JJ ANN OTOL RHINOL LARYNGOL 72 31 963 115 BLECKER JD ACTA OTOLARYNGOL STOCKH 71 726 971 116 BERNFELD P AM REV RESPIR DIS SUPPL 93 74 966 117 RYLANDER R AM REV RESPIR DIS 93 67 966 118 MERCKE U ACTA OTOLARYNGOL STOCKH 78 444 974 119 PROETZ AW ARCH OTOLARYNGOL 19 608 934 120 MERCKE U ACTA OTOLARYNGOL STOCKH 78 118 974 121 DALHAMN T ACTA PHYSIOL SCAND SUPPL 36 123 956 122 ADLER KB ARCH ENVIRON HEALTH 27 364 973 123 DAVIS SS BULL PHYSIOPATH RESPIR NANCY 9 47 973 124 AIACHE JM BIORHEOLOGY 11 375 974 125 OBRECHT B BIORHEOLOGY 12 211 975 126 DAVIS SS IN: GABELNICK HL 158 973 127 MCCUTCHEN CW BIORHEOLOGY 11 265 974 128 BLECKER JD ANN OTOL RHINOL LARYNGOL 82 248 973 129 WARDELL JR JR AM REV RESPIR DIS 191 741 970 130 LUTZ RJ IN: GABELNICK HL 119 973 131 ADAMS GK J APPL PHYSIOL 40 247 976 132 POWELL RL J APPL PHYSIOL 37 447 974 133 GILBOA A BIORHEOLOGY 13 67 976 134 MICHAELSON ED PHYSIOLOGIST 19 295 976 135 PHILIPPOFF W BIORHEOLOGY 7 55 970 136 BARNETT B AM REV RESPIR DIS 101 773 970 137 MORROW PE AM REV RESPIR DIS 96 1209 967 138 TAPLIN GV RADIOLOGY 85 365 965 139 ALBERT RE ARCH ENVIRON HEALTH 14 10 967 140 ALBERT RE ARCH INTERN MED 131 115 973 141 CAMNER P ARCH ENVIRON HEALTH 26 294 973 142 MULLER M KLIN WOCHENSCHR 53 815 975 143 SANCHIS J J APPL PHYSIOL 33 757 972 144 ALBERT RE ARCH ENVIRON HEALTH 18 738 969 145 YEATES DB J APPL PHYSIOL 39 487 975 146 WOOD PB CAN ANAESTH SOC J 20 192 973 147 GIORDANO AM JR ARCH ENVIRON HEALTH 25 443 972 148 BERKE HL AM IND HYG ASSOC J 32 174 971 149 IRAVANI J PNEUMONOLOGIE 144 93 971 150 GOLDHAMER RE METHOD FOR STUDYING MUCUS F 964 151 LAURENZI GA PROC ASPEN EMPHYSEM CONF 10TH 27 967 152 GIORDANO A ANN OTOL RHINOL LARYNGOL 85 631 976 153 SAKAKURA Y PROC SOC EXP BIOL MED 140 870 972 154 MARIN MG J APPL PHYSIOL 27 385 969 155 WANNER A ARCH ENVIRON HEALTH 27 370 973 156 LANDA JF J APPL PHYSIOL 38 946 975 158 LIERLE DM ARCH OTOLARYNGOL 19 55 934 159 SCUDI JV J PHARMACOL EXP THER 102 132 951 160 BOYD EM ARCH OTOLARYNGOL 33 909 941 161 HILL JR J PHYSIOL (LOND) 139 157 957 162 HILL L LANCET 2 802 928 163 KENSLER CJ AM REV RESPIR DIS SUPPL 93 93 966 164 CARSON S AM REV RESPIR DIS SUPPL 93 86 966 165 LAURENZI GA N ENGL J MED 279 333 968 166 BATTISTA SP ARCH ENVIRON HEALTH 20 326 970 167 IRAVANI J PFLUEGERS ARCH 297 221 967 168 BAETJER AM AM REV RESPIR DIS 93 79 966 169 BAETJER AM J APPL PHYSIOL 23 498 967 170 PROCTOR DF ARCH INTERN MED 131 132 973 171 QUINLAN MF AM REV RESPIR DIS 99 13 969 172 EWERT G ACTA OTOLARYNGOL STOCKH SUPPL 200 32 965 173 SACKNER MA J APPL PHYSIOL 34 495 973 174 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 175 WOOD RE AM REV RESPIR DIS 113 833 976 176 ANDERSEN IB AM REV RESPIR DIS 106 438 972 177 PARKS CR AM REV RESPIR DIS 104 99 971 178 PARKS CR AM REV RESPIR DIS 108 513 973 179 BOHNING DE J APPL PHYSIOL 41 920 976 180 BAETJER AM ARCH ENVIRON HEALTH 26 61 973 181 BANG BG PROC SOC EXP BIOL MED 106 516 961 182 PROETZ AW ANN OTOL RHINOL LARYNGOL 42 778 933 183 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 184 TOREMALM NG ACTA OTOLARYNGOL STOCKH 53 442 961 185 HIRSCH JA J APPL PHYSIOL 39 242 975 186 FORBES AR BR J ANAESTH 45 874 973 187 PALMER KNV LANCET 1 91 960 188 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 189 MATTHEWS LW PEDIATRICS 39 176 967 190 HIRSCH SR IN: STEIN M 351 975 191 CRAGG J ARCH INTERN MED 107 81 967 192 BURTON JDK LANCET 1 235 962 193 TOREMALM NG ACTA OTOLARYNGOL STOCKH 52 461 960 194 MERCKE U ACTA OTOLARYNGOL STOCKH 78 253 974 195 IRAVANI J PFLUEGERS ARCH 297 221 967 196 IRAVANI J PFLUEGERS ARCH 297 221 967 197 TANAKA AT J OTOLARYNGOL JAP 70 2091 967 198 DALHAMN T ACTA PHYSIOL SCAND 49 242 960 199 MERCKE U ACTA OTOLARYNGOL STOCKH 79 133 975 201 OLIPHANT JF PHYSIOL ZOOL 11 19 938 202 JENNINGS HS AM J PHYSIOL 2 311 899 203 PARKER GH AM J PHYSIOL 13 1 905 204 MERTON H BIOL ZENTRALBL 55 268 935 205 KAMADA T PROC IMP ACAD TOKYO 16 125 940 206 HEE J BULL PHYSIOPATH RESPIR NANCY 9 377 973 207 KRUEGER AP PROC SOC EXP BIOL MED 94 807 957 208 KRUEGER AP PROC SOC EXP BIOL MED 102 355 959 209 KRUEGER AD J GEN PHYSIOL 44 269 960 210 GUILLERM R C R ACAD SCI (D)(PARIS) 266 528 968 211 GUILLERM R C R ACAD SCI (D)(PARIS) 262 669 966 212 BADRE R ANN PHARM FR 24 469 966 213 KENSLER CJ AM REV RESPIR DIS SUPPL 93 93 966 214 BALDETORP L ACTA RADIOL THER STOCKH 15 225 976 215 BOUSHY SF AM J ROENTG RAD THER NUCL MED 108 287 970 216 MENDENHALL WL J PHARMACOL EXP THER 60 111 937 217 MENDENHALL WL J PHARMACOL EXP THER 69 295 940 218 HILDING AC N ENGL J MED 254 1155 956 219 DALHAMN T ARCH OTOLARYNGOL 70 166 959 220 DALHAMN T AM REV RESPIR DIS SUPPL 93 108 966 221 KENSLER CJ N ENGL J MED 269 1161 963 222 FALK HL J NATL CANCER INST 23 999 959 223 GUILLERM R BULL ACAD NATL MED 145 416 961 224 BATTISTA SP FED PROC 21 701 962 225 AUERBACH O CANCER 20 2055 967 226 LEUCHTENBERGER C CANCER 11 490 958 227 RYLANDER R ARCH ENVIRON HEALTH 29 329 974 228 FRASCA JM EXP MOL PATH 21 300 974 229 REGLAND B SCAND J RESPIR DIS 57 171 976 230 NIEWOEHNER DE N ENGL J MED 291 755 974 231 MATSUBA K AM REV RESPIR DIS 104 516 971 232 PROETZ AW ANN OTOL RHINOL LARYNGOL 48 176 939 233 WEISS W ARCH ENVIRON HEALTH 9 50 964 234 WANG H NATURE 197 946 963 235 BALLENGER JJ N ENGL J MED 263 832 960 235 JAKAB GJ AM REV RESPIR DIS 115 33 977 236 GUILLERM R REV TUBERC PNEUMOL 36 187 972 237 DALHAMN T ARCH ENVIRON HEALTH 21 633 970 238 GRAY JP ARCH ENVIRON HEALTH 28 283 974 239 KENNEDY JR SCIENCE 168 1097 970 240 ALBERT RE ARCH ENVIRON HEALTH 29 96 974 241 CARSON S AM REV RESPIR DIS SUPPL 93 86 966 242 RAKIETEN N ARCH OTOLARYNGOL 56 495 952 243 TSUCHIYA M FED PROC 18 453 959 244 DALHAMN T ARCH OTOLARYNGOL 84 325 966 245 BERNFELD P TOXICOL APPL PHARMACOL 6 103 964 246 DALHAMN T ARCH OTOLARYNGOL 87 162 968 247 WYNDER E CANCER 16 1222 963 248 IRAVANI J RESPIRATION 29 480 972 249 DALHAMN T AM REV RESPIR DIS 89 870 964 250 HOLMA B ARCH ENVIRON HEALTH 18 171 969 251 DALHAMN T AM REV RESPIR DIS 99 447 969 252 KAMIMSKI EJ ARCH ENVIRON HEALTH 16 188 968 253 ALBERT RE ARCH ENVIRON HEALTH 18 30 969 254 LA BELLE CW ARCH ENVIRON HEALTH 12 588 966 255 BAIR WJ IN: DAVIES CN 2 251 967 256 DALHAMN T ARCH ENVIRON HEALTH 17 746 968 257 FRANCES R ARCH ENVIRON HEALTH 21 25 970 258$ RYLAND R ARCH ENVIRON HEALTH 23 321 961 259 DALHAMN T AM REV RESPIR DIS 103 855 971 260 CAMNER P ARCH ENVIRON HEALTH 23 421 971 261 ALBERT RE ARCH ENVIRON HEALTH 30 361 975 262 YERGIN B AM REV RESPIR DIS SUPPL 115 183 977 263 IRAVANI J RESPIRATION 31 358 974 264 PAVIA D NATURE 226 1228 970 265 THOMSON ML ARCH ENVIRON HEALTH 26 86 973 266 LOURENCO RV J CLIN INVEST 50 1411 971 267 CAMNER P ARCH ENVIRON HEALTH 26 90 973 268 CAMNER P ARCH ENVIRON HEALTH 25 60 972 269 BOHNING DE ARCH ENVIRON HEALTH 30 457 975 270 ALBERT RE ARCH ENVIRON HEALTH 22 12 971 271 DALHAMN T AM REV RESPIR DIS SUPPL 93 108 966 272 HILDING AC ANN OTOL RHINOL LARYNGOL 41 52 932 273 CRALLEY LV J IND HYG TOXICOL 24 193 942 274 CAMNER P ARCH ENVIRON HEALTH 27 81 973 275 BALCHUM OJ J APPL PHYSIOL 15 62 960 276 NADEL JA ARCH ENVIRON HEALTH 10 175 965 277 GOLDRING IP ARCH ENVIRON HEALTH 15 167 967 278 SPICER SS AM REV RESPIR DIS 110 13 974 279 CHAKRIN LW LAB INVEST 30 145 974 280 MARTIN SW ARCH ENVIRON HEALTH 25 158 972 281 REID L ARCH INTERN MED 126 428 970 282 QUEVAUVILLER A C R SOC BIOL (PARIS) 160 1845 961 283 DALHAMN T AM REV RESPIR DIS 83 566 961 284 SPIEGELMAN JR ARCH ENVIRON HEALTH 17 321 968 285 ANDERSEN I ARCH ENVIRON HEALTH 28 31 974 286 WOLFF RK ARCH ENVIRON HEALTH 30 521 975 287 AMDUR MO ARCH ENVIRON HEALTH 16 460 968 288 DALHAMN T ARCH ENVIRON HEALTH 16 821 968 289 FAIRCHILD GA ARCH ENVIRON HEALTH 30 538 975 290 FAIRCHILD GA ARCH ENVIRON HEALTH 25 174 972 291 ANON AIR QUALITY CRITERIA FOR SULF 969 292 FRANK NR ARCH ENVIRON HEALTH 18 315 969 293 HAAGEN-SMIT AJ ARCH INDUST HEALTH 20 399 959 294 LEWIS TR ARCH ENVIRON HEALTH 18 596 969 295 STRESEMANN E STAUB REINHALT LUFT 30 33 970 296 FREEMAN G AM REV RESPIR DIS 106 563 972 297 DAVIDSON JT AM REV RESPIR DIS 95 790 967 298 BLAIR WH ARCH ENVIRON HEALTH 18 186 969 299 BUCKLEY RD ARCH ENVIRON HEALTH 18 588 969 300 FREEMAN G ARCH ENVIRON HEALTH 18 609 969 301 FREEMAN G ARCH ENVIRON HEALTH 17 181 968 302 WAGNER WD ARCH ENVIRON HEALTH 10 455 965 303 EHRLICH R ARCH ENVIRON HEALTH 17 860 968 304 ANON AIR QUALITY CRITERIA FOR NIT 971 305 STOKINGER HE ANNU REV PHARMACOL 12 407 972 306 BATES DV J APPL PHYSIOL 32 176 972 307 LINN WS AM REV RESPIR DIS 114 477 976 308 MOORMAN WJ ARCH ENVIRON HEALTH 26 153 973 309 TREMER HM 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Krastins-I-R. Wannamaker-E-M. Levison-H. Crozier-D-N. Bryan-A-C. TI Ventilatory muscle endurance training in normal subjects and patients with cystic fibrosis. SO Am-Rev-Respir-Dis. 1977 Nov. 116(5). P 853-60. MJ CYSTIC-FIBROSIS: pp. MUSCLES: pp. MN ADOLESCENCE. ADULT. EXERTION. HUMAN. PHYSICAL-EDUCATION-AND-TRAINING. PHYSICAL-ENDURANCE. RESPIRATION. RESPIRATORY-FUNCTION-TESTS: is, mt. TIME-FACTORS. AB Ventilatory muscles can become fatigued, and this can contribute to respiratory failure. Patients with chronic obstructive lung disease may benefit from improving their ventilatory muscle endurance to improve resistance to fatigue. Ventilatory muscle endurance was measured in 30 normal subjects and 55 patients with cystic fibrosis by finding the highest level of normocapnic hyperpnea that could be sustained for 15 min. Subjects with cystic fibrosis had 36 per cent higher ventilatory muscle endurance than normal subjects, reflecting the chronic training stress of breathing against increased respiratory loads. Four normal subjects and 4 subjects with cystic fibrosis participated in a specific ventilatory muscle endurance training program consisting of 25 min per day of maximal normocapnic hyperpnea 5 days per week for 4 weeks. The cystic fibrosis patients who trained improved their ventilatory muscle endurance by 51.6 per cent, whereas the normal subjects who trained showed a 22.1 per cent increase in ventilatory muscle endurance. Seven subjects with cystic fibrosis participated in a 4-week physical activity training program consisting of at least 1.5 hours per day of intensive swimming and canoeing at summer camp. They increased their ventilatory muscle endurance by 56.7 per cent. There were no other pulmonary function changes. Ventilatory muscle endurance can be readily improved in cystic fibrosis equally well by specific ventilatory muscle endurance exercise. RF 001 ROUSSOS CS PHYSIOLOGIST 19 345 976 002 LEITH DE J APPL PHYSIOL 41 508 976 003 ZOCCHE GP J APPL PHYSIOL 15 1073 960 004 TENNEY SM RESPIR PHYSIOL 5 187 968 005 FREEDMAN S RESPIR PHYSIOL 8 230 970 006 LEVISON H J APPL PHYSIOL 25 21 968 007 CLARK TJH CLIN SCI 36 307 969 008 POTTER WA J CLIN INVEST 50 910 971 009 ASTRAND PO J APPL PHYSIOL 7 218 954 010 SNEDECOR GW STATISTICAL METHODS 974 011 LANDAU LI AM REV RESPIR DIS 111 725 975 012 LIEBERMAN DA AM J PHYSIOL 222 556 972 013 SANTAMBROGIO A RESPIR PHYSIOL 10 236 970 014 KEENS TG FED PROC 36 614 977 015 PAEZ PN AM REV RESPIR DIS 95 944 967 CT 1 DERENNE JP AM REV RESPIR DIS 118 119 978 2 PERESS L BULL EUR PHYSIOPATH RESP 15 91 979 3 ROCHESTER DF BULL EUR PHYSIOPATH RESP 15 951 979 4 SMIDT U BULL EUR PHYSIOPATH RESP 15 95 979 5 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 6 KEENS TG PEDIATR CLIN NORTH AM 26 517 979 7 ANDERSEN JB SCAND J RESPIR DIS 60 151 979 8 MACKLEM PT AM REV RESPIR DIS 119 93 979 9 GROSS D AM J MED 68 27 980 10 COATES AL ACTA PAEDIATR SCAND 69 353 980 11 BELMAN MJ AM REV RESPIR DIS 121 273 980 12 CAMPBELL JA AM REV RESPIR DIS 122 679 980 13 ROCHESTER DF AM REV RESPIR DIS 122 133 980 14 HUBBARD VS AM J DIS CHILD 134 317 980 15 MOSER KM ARCH INTERN MED 140 1596 980 16 ZACH MS LANCET 2 1201 981 17 BELMAN MJ EUR J RESPIR DIS 62 391 981 18 ORENSTEIN DM CHEST 80 392 981 19 LISBOA C REV MED CHIL 109 476 981 20 MORENO R REV MED CHIL 109 393 981 21 PARDY RL AM REV RESPIR DIS 123 426 981 22 JARDIM J AM REV RESPIR DIS 124 274 981 23 MARTIN BJ INT J SPORTS MED 3 100 982 24 MILES DS ERGONOMICS 25 239 982 25 MILLER M AM J CLIN NUTR 36 492 982 26 GIMENEZ M EUR J APP PHYSI OCCUP PHYSIOL 49 379 982 27 NICKERSON BG J APPL PHYSIOL 52 768 982 28 ROBINSON EP J APPL PHYSIOL 52 1400 982 29 ZACH M ARCH DIS CHILD 57 587 982 30 COHEN CA AM J MED 73 308 982 31 BELMAN MJ CHEST 81 440 982 32 SONNE LJ CHEST 81 436 982 33 BELMAN MJ CHEST 82 761 982 34 ALDRICH TK AM REV RESPIR DIS 126 195 982 35 ARORA NS AM REV RESPIR DIS 126 5 982 36 ASHER MI AM REV RESPIR DIS 126 855 982 37 DELGADO HR AM REV RESPIR DIS 126 200 982 38 ROUSSOS C N ENGL J MED 307 786 982 39 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 40 SHARP JT CLIN CHEST MED 4 421 983 41 GROSS D ISR J MED SCI 19 383 983 42 FANTA CH J APPL PHYSIOL 54 1618 983 43 ROCHESTER DF MED CLIN NORTH AM 67 573 983 44 NICKERSON BG PEDIATRICS 71 147 983 45 MORENO R REV MED CHIL 111 647 983 46 WEISMAN IM AM REV RESPIR DIS 127 641 983 47 ONEILL S AM REV RESPIR DIS 128 1051 983 48 PARDY RL CLIN CHEST MED 5 35 984 49 CARRIERI VK HEART LUNG 13 436 984 50 KIM MJ HEART LUNG 13 333 984 51 KOTTKE TE PREV MED 13 47 984 52 MOXHAM J BULL EUR PHYSIOPATH RESP 20 437 984 53 HODSON ME POSTGRAD MED J 60 225 984 54 FARKAS GA J CLIN INVEST 74 1214 984 55 HODGES P J AM DIET ASSOC 84 664 984 56 JEDERLINIC P CHEST 86 870 984 57 SONNE LJ CHEST 86 939 984 58 WAGENER JS AM REV RESPIR DIS 129 873 984 59 DIMARCO AF MUSCLE NERVE 8 284 985 60 GREEN M CLIN SCI 68 1 985 61 SHARP JT CHEST 88 S118 985 62 RENZI G UNION MED CAN 114 897 985 63 BELMAN MJ AM REV RESPIR DIS 131 226 985 64 CHEN HI AM REV RESPIR DIS 131 251 985 65 ZACH M MONATSSCHR KINDERHEILKD 133 868 985 66 ALDRICH TK LUNG 163 15 985 67 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 68 HADDAD GG CLIN CHEST MED 7 79 986 69 KELSEN SG CLIN CHEST MED 7 101 986 70 MCKEON JL AUST NZ J MED 16 648 986 71 MARTIN AJ DEVELOP MED CHILD NEUROL 28 314 986 72 ESTRUP C RESPIRATION 50 36 986 73 MACINTYRE NR CHEST 89 677 986 74 BELMAN MJ CHEST 90 662 986 75 RIES AL CHEST 90 285 986 76 MOCELLIN R WIEN KLIN WOCHENSCHR 98 735 986 77 LEVINE S AM REV RESPIR DIS 133 400 986 78 EDLUND LD AM J DIS CHILD 140 80 986 79 CLANTON TL J APPL PHYSIOL 62 39 987 80 CROPP A AM REV RESPIR DIS 135 1056 987 PN 77025 RN 00607 AN 77219983 AU Boat-T-F. Petty-T-L. TI Chronic bronchitis and cystic fibrosis: two chronic obstructive lung diseases of adults [editorial]. SO Am-Rev-Respir-Dis. 1977 Jul. 116(1). P 1-2. MJ BRONCHITIS. CYSTIC-FIBROSIS. MN ADULT. BRONCHITIS: di, th. CHRONIC-DISEASE. CYSTIC-FIBROSIS: di, th. HUMAN. PROGNOSIS. EX As patients with cystic fibrosis survive longer, the number of adults with this chronic obstructive lung disease is increasing rapidly. Chronic bronchitis and cystic fibrosis are similar in a number of respects. The treatment of cystic fibrosis and chronic bronchitis has some similarities. But chronic bronchitis and cystic fibrosis also differ in many respects. The pathogenesis of chronic bronchitis is more clearly related to exogonous factors such as smoking and air pollution than is cystic fibrosis, which is genetically determined. Clinical differences also exist. Major differences in treatment between cystic fibrosis and chronic bronchitis are currently in vogue. Because of the special problems of adulthood, it is appropriate that many adult patients with cystic fibrosis be cared for by internists who have previously focused their attention on chronic bronchitis and emphysema. Increased communication between internists and pediatricians on the issue of cystic fibrosis is badly needed today. CT 1 HOWIE AD SCOTT MED J 24 193 979 2 SANTAGNESE PAD AM J MED 66 121 979 3 HENRY RL AUST PAEDIATR J 18 43 982 4 GOTZ M ATEMWEGS LUNGENKRANKH 10 594 984 5 KAUFFMANN F BULL EUR PHYSIOPATH RESP 20 163 984 6 DAVIS PB CHEST 85 802 984 7 GOTZ M MED KLIN 80 657 985 PN 77026 RN 00608 AN 78058877 AU Lieberman-J. Kaneshiro-W. TI Abnormal response of cultured lymphocytes to phytohemagglutinin and autologous serum in cystic fibrosis. SO Am-Rev-Respir-Dis. 1977 Dec. 116(6). P 1047-55. MJ CYSTIC-FIBROSIS: im. LYMPHOCYTE-TRANSFORMATION. LYMPHOCYTES: im. MN ADOLESCENCE. ADULT. BLOOD-TRANSFUSION-AUTOLOGOUS. CELLS-CULTURED. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. FEMALE. GLUCURONIDASE: an. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IN-VITRO. LYMPHOCYTES: an. MALE. LECTINS. PROTEINS: an. SUPPORT-U-S-GOVT-P-H-S. AB Lymphocytes from adults homozygous or heterozygous for cystic fibrosis show biochemical abnormalities when cultured for 48 hours in the presence of phytohemagglutinin and autologous serum. In contrast to the 45 per cent increase in total protein and beta-glucuronidase concentrations seen in healthy control subjects when measured per 10(10) cells, both concentrations decreased by 1 per cent in adults heterozygous for cystic fibrosis and by 18 per cent in adults homozygous for cystic fibrosis. The abnormal response of the lymphocytes from persons with cystic fibrosis was due to a serum factor and not to any intrinsic abnormality of the lymphocytes. An abnormal response to hytohemagglutinin occurred in only 14 per cent of 44 healthy control subjects, but in 100 per cent of 14 adults homozygous for cystic fibrosis and in 85 per cent of 26 adults presumed to be heterozygous for cystic fibrosis. As a result of this phenomenon, lymphocytic beta-glucuronidase concentrations were significantly lower than normal in patients with cystic fibrosis when the cells were cultured with phytohemagglutinin and autologous serum. The demonstration of this phenomenon in both homozygotes and presumed heterozygotes (parents) suggests a relationship to the genetic defect in cystic fibrosis. RF 001 LIEBERMAN J CHEST 70 433 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 DANES BS NATURE 222 685 969 005 DANES BS LANCET 2 437 969 006 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 007 ANTONOWICZ I PEDIATR RES 6 803 972 008 RENNERT OM CLIN PEDIATR 11 351 972 009 MARCHI AG HELV PAEDIATR ACTA 28 427 973 010 CONOVER JH PEDIATR RES 7 224 973 011 STEELE WM PEDIATR RES 7 394 973 012 BOZKOWA K BOLL IST SIEROTER MILAN 53 266 974 013 DANES BS BIOCHEM GENET 12 359 974 014 SHAPIRO BL LANCET 2 1020 974 015 OGRADY LR CF CLUB ABST 16 975 016 BRAUNSTEIN M CF CLUB ABST 28 975 017 BEUTLER E J EXP MED 143 975 976 018 THURMAN GB CLIN EXP IMMUNOL 15 289 973 019 HIRSCHHORN K SCIENCE 166 1632 969 CT 1 LIEBERMAN J LANCET 1 985 978 2 MARCHI AG HELV PAEDIATR ACTA 33 517 978 3 SORENSEN RU INFECT IMMUN 23 398 979 4 LIEBERMAN J CHEST 75 220 979 5 HODSON ME THORAX 35 801 980 6 WILSON GB J CLIN INVEST 66 1010 980 7 SORENSEN RU PEDIATR RES 15 14 981 8 SORENSEN RU AM REV RESPIR DIS 123 37 981 9 SEALE TW ANN CLIN LAB SCI 12 415 982 10 DISTELHORST CW CELL IMMUNOL 75 188 983 11 LIEBERMAN J AM J MED 77 678 984 12 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 77027 RN 00609 AN 77179912 AU Berry-D-H. Brewster-M-A. TI Granulocyte NADH oxidase in cystic fibrosis. SO Ann-Allergy. 1977 May. 38(5). P 316-9. MJ BACTERIAL-INFECTIONS: di. CYSTIC-FIBROSIS: en. GRANULOCYTES: en. LEUKOCYTES: en. NADH-NADPH-OXIDOREDUCTASES: bl. MN BACTERIAL-INFECTIONS: co. CYSTIC-FIBROSIS: bl, co. HUMAN. NITROBLUE-TETRAZOLIUM: du. AB The nitro blue tetrazolium dye test was evaluated for its usefulness in detecting superimposed bacterial infection in 50 children with cystic fibrosis. No correlation of percentage of positive neutrophils to superimposed infection, clinical status or to bacterial organisms could be determined. Cystic fibrosis granulocytes generally have greater NADH oxidase activity than do controls. RF 001 KARNOVSKY ML SEMIN HEMATOL 5 156 968 002 NATHAN DG J CLIN INVEST 48 85 969 003 BAEHNER RL N ENGL J MED 278 971 968 004 PARK BH LANCET 2 532 968 005 FEIGIN RD J PEDIATR 78 230 971 006 MATULA G N ENGL J MED 285 311 971 007 EVANS TJ LANCET 1 992 974 008 FEIGIN RD CLIN MED 81 16 974 009 STEIGBIGEL RT N ENGL J MED 290 235 974 010 FINEBERG H N ENGL J MED 290 1144 974 011 SULLIVAN JF AM J DIS CHILD 125 702 973 PN 77028 RN 00610 AN 77155815 AU Ten-Kate-L-P. TI A method for analysing fertility of heterozygotes for autosomal recessive disorders, with special reference to cystic fibrosis, Tay-Sachs disease and phenylketonuria. SO Ann-Hum-Genet. 1977 Jan. 40(3). P 287-97. MJ FERTILITY. GENES-RECESSIVE. HEREDITARY-DISEASES: fg. HETEROZYGOTE. MN LIPOIDOSIS: fg. CYSTIC-FIBROSIS: fg. GENETICS-POPULATION. HUMAN. PHENYLKETONURIA: fg. STATISTICS. AB Increased fertility of heterozygotes with respect to decreased foetal loss among offspring of heterozygotes has been proposed by several authors as a possible explanation for the high gene frequency of CF, TSD and PKU in certain populations. Studies comparing reproductive outcome of heterozygotes with reproductive performance in the general population or in special control groups have been done on several occasions. These studies, however, are known to be heavily biased, on the one side by the fact that ascertainment of heterozygotes through affected offspinrg will tend to underestimate the relative frequency of smaller families, and on the other side because of the inadequacy of census data for comparison and the biases inherent in selection of control families. Careful analysis of the biases involved provides suggestions for proper corrections. From this a method has been developed which offers a better approach to the study of heterozygote fertility in those autosomal recessive conditions which lack a test for direct heterozygote detection. RF 001 BARRAI I AM J HUM GENET 17 221 965 002 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 003 BRUNECKY Z PAEDIATR GRENZGEB 8 99 969 004 CHASE GA AM J HUM GENET 24 339 972 005 ANON HUMANGENETIK 30 273 975 006 CONNEALLY PM TEX REP BIOL MED 31 639 973 007 DANKS DM ANN HUM GENET 28 323 965 008 ELANDT-JOHNSON RC PROB MODELS AND STAT METH IN 971 009 GERY G J GENET HUM 19 275 971 010 GILLY R ARCH FR PEDIATR 28 49 971 011 GOLD RJM ANN HUM GENET 37 315 974 012 GOODMAN HO AM J HUM GENET 4 59 952 013 HIRSCHHORN K IN: MANGOS JA 11 973 014 KAARIAINEN R HEREDITAS 75 241 973 015 KAARIAINEN R HEREDITAS 75 109 973 016 KNUDSON AG JR AM J HUM GENET 19 388 967 017 LENZ W ACTA GENETICA 9 169 959 018 MASTERSON J PROC EWGCF 6TH ANNU MTG 975 019 MAYO O ANN HUM GENET 33 307 970 020 MYRIANTHOPOULOS NC IN: ARONSON SM 962 021 MYRIANTHOPOULOS NC AM J HUM GENET 18 313 966 022 OAKES MW ANN HUM GENET 32 173 968 023 OBRIEN JS N ENGL J MED 283 15 970 024 RAO CR IN: PATIL GP 965 025 RAO DC AM J HUM GENET 25 594 973 026 RECORD RG BR J PREV SOC MED 29 267 975 027 SAUGSTAD LE CLIN GENET 4 105 973 028 SIMANKOVA N CESK PEDIATR 25 77 970 029 SINISCALCO M MAANDSCHRIFT VOOR KINDERGENEE 37 134 969 030 ANON STATISTISCHES JAHRBUCH DER SC 955 031 STEINBERG AG AM J HUM GENET 12 416 960 032 STEPHAN U PEDIATRICS 55 35 975 033 WAGENER DK AM J HUM GENET 27 348 975 034 WESTWOOD A J MED GENET 12 327 975 035 WOOLF LI ANN HUM GENET 38 461 975 036 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 037 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 MAYO O HUM HERED 28 270 978 2 TENKATE LP ANN HUM GENET 41 463 978 3 TENKATE LP ANN HUM GENET 41 397 978 4 MACCLUER JW ANN HUM GENET 42 59 978 5 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 77029 RN 00611 AN 77240385 AU Stern-R-C. Boat-T-F. Doershuk-C-F. Tucker-A-S. Miller-R-B. Matthews-L-W. TI Cystic fibrosis diagnosed after age 13. Twenty-five teenage and adult patients including three asymptomatic men. SO Ann-Intern-Med. 1977 Aug. 87(2). P 188-91. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. AGE-FACTORS. CYSTIC-FIBROSIS: co, mi. FEMALE. HUMAN. LUNG-DISEASES: et. MALE. PSEUDOMONAS-INFECTIONS: et. INFERTILITY-MALE: et. SUPPORT-U-S-GOVT-P-H-S. AB Cystic fibrosis was diagnosed after age 13 in 25 patients. All had an elevated sweat chloride and either a sibling with cystic fibrosis or typical pulmonary infection or digestive symptoms caused by exocrine pancreatic deficiency. Fourteen had long-standing pulmonary or digestive symptoms. In contrast, four of eight patients whose symptoms began after age 13 presented with biliary cirrhosis. Three male patients were asymptomatic at diagnosis. Opacification of all paranasal sinuses was found in all patients examined radiologically. At diagnosis, pulmonary-function testing showed obstructive changes in 19 patients and sputum cultures showed Pseudomonas aeruginosa in 15 patients. Delayed menarche in five of seven female patients and infertility in the asymptomatic male patient (two of whom were found to have aspermia) could have led to earlier diagnosis. Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis. RF 001 SHWACHMAN H PROC INT CF CONG 7TH 90 976 002 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 003 STERN RC J PEDIATR 89 406 976 004 MITCHELL-HEGGS PF Q J MED 45 479 976 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 ADDINGTON WW CHEST 59 306 971 007 LOBER CW CHEST 66 332 974 008 COATES EO JR DIS CHEST 49 195 966 009 TOMASHEFSKI JF CHEST 57 28 970 010 NOLAN AJ CAN MED ASSOC J 114 142 976 011 STERN RC GASTROENTEROLOGY 70 645 976 012 KAPLAN E N ENGL J MED 279 65 968 013 DENNING CR PEDIATRICS 41 7 968 014 COMROE JH JR LUNG CLINICAL PHYSIOLOGY AND 962 015 WILLMON TL AM J PHYSIOL 153 138 948 016 DUBOIS AB J CLIN INVEST 35 322 956 017 DOERSHUK CF J PEDIATR 65 677 964 018 GHARIB R AM J DIS CHILD 108 499 964 019 LOBECK CC PEDIATRICS 30 172 962 020 MURTHY MSN OHIO STATE MED J 69 768 973 021 SHWACHMAN H PEDIATRICS 55 86 975 022 MEARNS MB ARCH DIS CHILD 47 902 972 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 FINK RJ CHEST 74 643 978 3 ANDREWS C ANN INTERN MED 88 128 978 4 HOWIE AD SCOTT MED J 24 193 979 5 SANTAGNESE PAD AM J MED 66 121 979 6 HOLSCLAW DS CLIN CHEST MED 1 407 980 7 ALLAN JL AUST PAEDIATR J 16 270 980 8 SANDERS JS CHEST 77 226 980 9 REYNOLDS HY CLIN CHEST MED 2 103 981 10 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 11 COLTEN HR N ENGL J MED 304 831 981 12 STERN RC LANCET 1 1401 982 13 MASARYK TJ DIG DIS SCI 28 874 983 14 VANSCOY RE MED CLIN NORTH AM 67 173 983 15 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 16 LEWIS MI S AFR MED J 65 641 984 17 ROSENSTEIN BJ SOUTH MED J 77 1383 984 18 HANDELSMAN DJ N ENGL J MED 310 3 984 19 DAVIS PB SEM RESPIR MED 6 314 985 20 ORENSTEIN DM SEM RESPIR MED 6 252 985 21 REINIG JW PEDIATR RADIOL 15 222 985 22 REYNOLDS HY DISEASE A MONTH 31 1 985 23 SCOGGIN CH POSTGRAD MED 77 243 985 24 ISPIZUA AU MED CLIN 85 628 985 25 HILL DJS MED J AUST 143 230 985 26 OCONNOR KW ARCH INTERN MED 145 153 985 27 ISPIZUA AU REV CLIN ESP 176 417 985 28 BOUE A HUM GENET 74 288 986 29 ROSENSTEIN BJ SOUTH MED J 79 319 986 30 SARNELLI R AM J MED 80 541 986 31 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 PN 77030 RN 00612 AN 78079393 AU Schwarz-V. Simpson-N-I. Ahuja-A-S. TI Limitations of diagnostic value of the sweat test. SO Arch-Dis-Child. 1977 Nov. 52(11). P 870-4. MJ CYSTIC-FIBROSIS: di. SODIUM: an. SWEAT: an. MN ADOLESCENCE. ASTHMA: di. CHILD. CHILD-PRESCHOOL. DIAGNOSIS-DIFFERENTIAL. HUMAN. SWEAT: se. TIME-FACTORS. AB The sweat test, even if carried out by an experienced technician, sometimes lacks reproducibility owing presumably to physiological variations (patient's diet, temperature, and other factors at present unrecognized). Some patients are particularly prone to exhibit this variability and in them a single sweat test is almost valueless. The aldosterone status is believed to be responsible for a reciprocal relationship between sweat sodium and potassium concentrations: tests done on 8 patients show that a high sweat potassium is associated with a correspondingly lower sodium--a circumstance which must be borne in mind when interpreting a patient's sweat sodium. Of 30 patients presenting with a variety of symptoms compatible with a diagnosis of cystic fibrosis and with sweat sodium ranging from 50 to 75 mEq/1 (50-75 mmol/1), only 4 have proved to have cystic fibrosis after several years of observations; 13 have later been diagnosed as having asthma. The problem of the 'grey area' of uncertainty is aggravated by the heterozygous state which is also associated with a sweat sodium in this range. Repeated sweat tests are indicated if the sweat sodium lies within the 'grey area', and the diagnostic importance accorded the test should diminish as the sodium value approaches this area. The diagnosis of cystic fibrosis must remain in doubt unless there is strong supportive clinical evidence. RF 001 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 002 ANON J PEDIATR 88 711 976 003 DARLING RC AM J MED SCI 225 67 953 004 DI SANTAGNESE PA J PEDIATR 81 196 972 005 GIBSON LE J PEDIATR 81 193 972 006 GIBSON LE PEDIATRICS 23 545 959 007 MCKENDRICK T LANCET 1 183 962 008 SCHWARZ V IN: PORTER R 32 968 009 SCHWARZ V ARCH DIS CHILD 43 695 968 010 SUTCLIFFE CH PROC R SOC MED 61 297 968 CT 1 CHAZALETTE JP PEDIATRE 13 303 977 2 ANON LANCET 2 1032 978 3 SMALLEY CA LANCET 2 415 978 4 BARNES GL AUST PAEDIATR J 14 78 978 5 SMALLEY CA ARCH DIS CHILD 53 351 978 6 TARNOKY AL J ROY SOC MED 73 73 980 7 LITTLEWOOD JM PRACTITIONER 224 305 980 8 DAVID TJ LANCET 2 1204 982 9 HEELEY AF CLIN CHEM 29 2011 983 PN 77031 RN 00613 AN 77200637 AU Partington-M-W. Ferguson-A-C. TI Serotonin metabolism in cystic fibrosis. SO Arch-Dis-Child. 1977 May. 52(5). P 386-90. MJ CYSTIC-FIBROSIS: bl. SEROTONIN: bl. MN ADOLESCENCE. BLOOD-CELL-COUNT. BLOOD-PLATELETS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. HYDROXYINDOLEACETIC-ACID: ur. IGE: an. INFANT. MALE. AB The average blood serotonin level of 67 children with cystic fibrosis was found to be about twice that of age-matched normal children. There was no corresponding increase in the urinary excretion of 5- hydroxyindoleacetic acid (5-HIAA). Children with cystic fibrosis were well able to metabolize serotonin taken by mouth. No significant correlations were found between the blood serotonin level and the platelet count, height, weight, skinfold thickness, and pulmonary function test, 5 out of 44 patients had raised serum IgE levels, and their mean blood serotonin was higher than in those with normal IgE levels. No explanation for this emerged. Comparable findings (raised blood serotonin normal platelet count, normal urinary 5-HIAA) have been reported only in severe mental retardation. Further study of this phenomenon is warranted because (a) a raised blood serotonin level is sufficiently characteristic of cystic fibrosis to explore its use in diagnosis, and (b) it may help to explain the pathogenesis of cystic fibrosis and (c) the metabolism and function of serotonin. RF 001 ASHCROFT GW CLIN CHIM ACTA 9 364 964 002$ BELLANTI JA IMMUNOLOGY 226 972 003 BERMAN JL J PEDIATR 67 603 965 004 CHALLACOMBE DN ARCH DIS CHILD 47 442 972 005 DICKMAN ML AM REV RESPIR DIS 104 680 971 006 FISHMAN AP N ENGL J MED 291 884 974 007 GRAHAME-SMITH DG CARCINOID SYNDROME 972 008 HAVERBACK BJ GASTROENTEROLOGY 35 570 958 009 KJELLMAN NIM CLIN ALLERGY 6 51 976 011 KORF J CLIN CHIM ACTA 26 301 969 012 LOTT IT PEDIATR RES 6 730 972 013 MCCOY EE N ENGL J MED 291 950 974 014 PARE CMB J NEUROL NEUROSURG PSYCHIAT 23 341 960 015 PARTINGTON MW DEVELOP MED CHILD NEUROL 15 616 973 016 PIMPARKAR BD GASTROENTEROLOGY 40 504 961 017 RACHELEFSKY GS AM J DIS CHILD 128 355 974 018 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 019 TANNER JM BR MED J 1 446 962 020 TANNER JM ARCH DIS CHILD 41 454 966 021 TEOTIA M AM J CLIN NUTR 28 1284 975 022 TU JB DEVELOP MED CHILD NEUROL 14 457 972 023 UDENFRIEND S ARCH BIOCHEM BIOPHYS 85 487 959 024 WAALKES TP SCIENCE 127 648 958 CT 1 CUSACK D HORM METAB RES 11 448 979 2 SCHONI MH PEDIATR RES 19 47 985 PN 77032 RN 00614 AN 78038980 AU Strunk-R-C. Sieber-O-F. Taussig-L-M. Gall-E-P. TI Serum complement depression during viral lower respiratory tract illness in cystic fibrosis. SO Arch-Dis-Child. 1977 Sep. 52(9). P 687-90. MJ COMPLEMENT: an. CYSTIC-FIBROSIS: co. RESPIRATORY-TRACT-INFECTIONS: im. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. COMPLEMENT-3: an. COMPLEMENT-4: an. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. INFANT. MALE. RESPIRATORY-TRACT-INFECTIONS: co. SUPPORT-U-S-GOVT-P-H-S. VIRUS-DISEASES: im. AB Cystic fibrosis (CF) patients with viral lower respiratory tract illnesses (LRI) had depressed levels of the third and fourth components of complement, which returned to normal after recovery. There was no clinical evidence of immune complex disease. CF patients with LRI and no virus isolates, CF patients in stable status, and non- CF patients with LRI did not have complement depression. It is postulated that antigen-antibody complex activation of complement may occur in CF patients with viral LRI. RF 001 CONOVER JH LANCET 2 1501 973 002 DEFOREST A PEDIATR RES 6 388 972 003 GAITHER TA J IMMUNOL 113 574 974 004 HANN S LANCET 2 520 974 005 LENNETTE EH DIAGN PROCEDURES FOR VIRAL AN 969 006 MCFARLANE H BR MED J 1 423 975 007 MANCINI G IMMUNOCHEMISTRY 2 235 965 008 MAYER MM IN: KABAT EA 133 961 009 NADLER HL IN: STANBURY JB 1683 978 010 OLDSTONE MBA PROG MED VIROL 19 84 975 011 POLLEY MJ J MED GENET 11 249 974 012 STEIN AA J PEDIATR 65 495 964 013 TAUSSIG LM J PEDIATR 82 380 973 014 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 CT 1 HOWIE AD SCOTT MED J 24 193 979 2 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 3 MOSS RB AM REV RESPIR DIS 121 23 980 4 PETERSEN NT ACTA PAEDIATR SCAND 70 623 981 5 CHURCH JA CHEST 80 405 981 6 MANTHEI U AM REV RESPIR DIS 126 253 982 7 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 8 GUPTA AK BR J OPHTHALMOL 68 350 984 9 SEN DK BR J OPHTHALMOL 69 707 985 10 FRIEND PA J INFECT 13 55 986 PN 77033 RN 00615 AN 77110778 AU Hide-D-W. Martlew-R. TI Cystic fibrosis and myocardial fibrosis [letter]. SO Arch-Dis-Child. 1977 Feb. 52(2). P 163. MJ CYSTIC-FIBROSIS: co. ENDOMYOCARDIAL-FIBROSIS: co. MN CASE-REPORT. CHILD-PRESCHOOL. HUMAN. MALE. EX We were interested in the case reported by Mukherji et al., which suggested that cystic fibrosis might be an acid mucopolysaccharidosis. We have seen a similar child. Aged 5 years he was admitted to hospital with pneumonia. In spite of conventional treatment with cloxacillin and colistin he collapsed and died 48 hours after admission. The heart was not enlarged, but the myocardium showed a grossly abnormal appearance with variations in thickness and extensive areas of firm pale tissue. Histology of the heart muscle showed very dramatic changes with severe replacement fibrosis distributed patchily through the myocardium. This similar mixture of neutral and acid mucopolysaccharides was shown in both the pancreas and the salivary glands. There have now been a number of reports of myocardial fibrosis complicating cystic fibrosis. The case reported by Mukherji et al. and this case are the first in which mucopolysaccharide deposits have been found in the myocardium. The mixture of neutral and acid mucopolysaccharides in our case does not support the hypothesis that cystic fibrosis is simply a genetic acid mucopolysaccharidosis. RF 001 BARNES GL AUST PAEDIATR J 6 81 970 002 HIDE DW ARCH DIS CHILD 44 533 969 003 MUKHERJI RN ARCH DIS CHILD 51 563 976 004 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 CT 1 TORRIANI R MONOGR PAEDIATR 10 155 979 2 ZIMMERMANN A HELV PAEDIATR ACTA 37 183 982 3 VANVLEET JF AM J PATHOL 124 95 986 PN 77034 RN 00616 AN 78079397 AU McCollum-J-P. Muller-D-P. Harries-J-T. TI Test meal for assessing intraluminal phase of absorption in childhood. SO Arch-Dis-Child. 1977 Nov. 52(11). P 887-9. MJ BILE-ACIDS-AND-SALTS: an. DIET. MALABSORPTION-SYNDROMES: di. PANCREAS: en. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. ESTERASES: me. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. LIPASE: me. MALABSORPTION-SYNDROMES: en. MALE. TRYPSIN: me. AB A test meal for assessing the intraluminal phase of absorption in childhood has been validated. 132 test meals were administered to 110 patients aged 2 weeks to 18 years (mean age 4.3 years). 10 children with suspected malabsorption, who were proven to be normal after extensive investigation, constituted the control group. The activities of pancreatic enzymes, and the total and individual bile salt concentrations are presented for the control subjects, and pancreatic enzyme levels in this group are compared with those seen in children with pancreatic insufficiency (cystic fibrosis). The test meal has been designed so that it can be administered to children with suspected gluten, cows' milk, or disaccharide intolerance. The control data provided a basis for the interpretation of information obtained from the application of such a test meal to the clinical investigation of children with suspected malabsorption. RF 001 CESKA M CLIN CHIM ACTA 26 437 969 002 ERLANSON C SCAND J GASTROENTEROL 5 333 970 003 ERLANSON C SCAND J GASTROENTEROL 5 293 970 004 HADORN B CLIN GASTROENTEROL 1 125 972 005 HOFMANN AF IN: JAMES AT 362 964 006 IWATA T J BIOCHEM (TOKYO) 56 424 964 007 LUNDH G GASTROENTEROLOGY 42 275 962 008 MAKINO I ANAL LETT 5 341 972 009 WIGGINS HS GUT 8 415 967 010 ZOPPI G PEDIATR RES 6 880 972 CT 1 HARRIES JT ARCH DIS CHILD 54 19 979 2 WALLER SL AUST NZ J MED 10 351 980 3 FREDRIKZON B PEDIATR RES 14 1387 980 4 AGGETT PJ ARCH DIS CHILD 55 331 980 5 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 6 GLASGOW JFT IR J MED SCI 149 346 980 7 GLASGOW JFT IR J MED SCI 149 194 980 8 BANCHINI G PEDIATR RES 15 1073 981 9 MATHIAS PM J LIPID RES 22 829 981 10 MATHIAS PM J LIPID RES 22 177 981 11 MULLER DPR CLIN GASTROENTEROL 11 119 982 12 MULLER DPR ANN CLIN BIOCHEM 19 89 982 13 MILLA PJ GUT 24 818 983 14 BALI A BR MED J 287 1011 983 15 SAVAGE MO J PEDIATR GASTROENTEROL NUTR 4 187 985 16 SARLES J ARCH FR PEDIATR 44 311 987 PN 77035 RN 00617 AN 77200629 AU Edwards-J-H. TI Heterozygote advantage. SO Arch-Dis-Child. 1977 May. 52(5). P 343-4. MJ HEREDITARY-DISEASES. HETEROZYGOTE. MN CYSTIC-FIBROSIS: fg. GENE-FREQUENCY. HUMAN. SELECTION-GENETICS. EX When a genic disorder is only manifest if there are offending alleles at both loci, as in fibrocystic disease, there is a loss of two abnormal alleles with each affected birth since the homozygotes rarely reproduce. How is this loss compensated? How could some 50,000 alleles, which can only survive to convey themselves in effective gametes if they have a normal allelic partner, become established in the British Isles - and how have some half a million or more got into the world currency? First, migrations and settlements involve the proportion of defective alleles at all loci being conserved, but their variety being increased. Secondly, there could be a general tendency due to some advantage in health or vitality, which could be manifest by disease resistance, cold resistance, and by any number of physical or psychological mechanisms. Thirdly, there may be 'compensation' - that is the death of an infant may lead to replacement as a result of which the replacing sib, if surviving, would have a 2/3 chance of being a carrier. Fourthly, the disease may be maintained by mutation. Of the four possibilities, three are unrealistic, so that substantial selective advantage of heterozygotes over the last few hundred years must be postulated. RF 001 SPOCK A PEDIATR RES 1 173 967 002 BOWMAN BH SCIENCE 164 325 969 003 WEISENFELD SL SCIENCE 157 1134 967 004 GIBBON E DECLINE AND FALL OF THE ROMAN 783 005 DANKS DM ANN HUM GENET 28 323 965 CT 1 SUPER M CLIN GENET 16 65 979 2 BITTLES AH J SCI INDUSTR RES 39 768 980 3 HOLLANDER DH MED HYPOTHESES 8 191 982 4 LYEN KR MED HYPOTHESES 12 77 983 PN 77036 RN 00618 AN 77220556 AU Lipson-A. Meikle-H. TI Porcine pancreatin as a source of salmonella infection in children with cystic fibrosis. SO Arch-Dis-Child. 1977 Jul. 52(7). P 569-72. MJ DRUG-CONTAMINATION. PANCREATIN: ae. SALMONELLA-INFECTIONS: et. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt. DRUG-CONTAMINATION: pc. FEMALE. HUMAN. INFANT. MALE. PANCREATIN: st, tu. AB Contamination of a powdered preparation of pancreatin with Salmonella schwarzengrund and S. eimsbuettel resulted in the infection of at least 31% of one group of paediatric patients with cystic fibrosis. The pancreatin contained very small numbers of Salmonellae, the infecting dose in at least one child being less than 44 organisms. More stringent bacteriological standards are needed for pharmaceuticals and foods used by paediatric patients. RF 001 ARMSTRONG RW AM J EPIDEMIOL 91 300 970 002 BOHNHOFF M J INFECT DIS 111 117 962 003 BOWMER EJ AM J MED SCI 247 467 964 004 ANON BRITISH PHARMACOPOEIA NO 340 973 005 ANON COMMUNICABLE DIS REPORT 1975 975 006 ANON COMMUNICABLE DIS REPORT 1976 976 007 CHRISTIE AB INFECT DIS 20 974 008 CRAVEN PC LANCET 1 788 975 009 DRACHMAN RH PEDIATR CLIN NORTH AM 21 711 974 010 ANON CODE OF HYGENIC PRACTICE FOR 976 011 GLENCROSS EJG BR MED J 2 376 972 012 GORDON JE MED CLIN NORTH AM 54 1495 970 013 GUINEE PAM ZENTRALBL BAKTERIOL 231 97 975 014 HOOK EW BULL NY ACAD MED 37 499 961 015 HORNICK RB N ENGL J MED 283 686 970 016 KALLINGS LO ACTA PHARMACEUTICA SUEDICA 3 219 966 017 KRUGMAN S INFECT DIS CHILDREN AND ADULT 261 973 018 ANON LANCET 1 830 972 019 LANG DJ N ENGL J MED 276 829 967 020 MCCULLOUGH NB J INFECT DIS 88 276 951 021 MCCULLOUGH NB J INFECT DIS 89 209 951 022 MCCULLOUGH NB J INFECT DIS 89 259 951 023 PROST E ANNU REV MICROBIOL 21 495 967 024 ROWE B BR MED J 4 51 975 CT 1 TURNBULL PCB CLIN GASTROENTEROL 8 663 979 2 PARK RW GASTROENTEROLOGY 81 1143 981 3 CANTEY JR J INFECT DIS 143 440 981 4 CALVERT CA J AM ANIM HOSP ASSOC 21 499 985 PN 77037 RN 00619 AN 77201257 AU Elliott-R-B. Eyre-J. Robinson-P-G. TI Salivary amylase in the parents of children with cystic fibrosis. SO Aust-Paediatr-J. 1977 Mar. 13(1). P 29-32. MJ AMYLASES: an. CYSTIC-FIBROSIS: fg. PARENTS. SALIVA: en. MN FEMALE. HETEROZYGOTE. HUMAN. MALE. SALIVATION. AB Mixed submaxillary/sublingual saliva from obligate heterozygotes for cystic fibrosis shows significantly elevated concentration of amylase at high or low salivation rates compared with normals. Sixty per cent of known carriers had values outside the range of apparently normal controls. RF 001 BABSON SR CLIN CHIM ACTA 44 193 973 002 BLOMFIELD J AUST PAEDIATR J 10 75 974 003 BLOMFIELD J SCI PROC AUSTRALIAN PAEDIA 975 004 BLOMFIELD J GUT 14 558 973 005 BRUNS WT AM J DIS CHILD 126 685 973 006 CHERNICK WS J PEDIATR 59 890 961 007 CHERNICK WS J PEDIATR 65 694 964 008 MANDEL ID AM J DIS CHILD 113 431 967 009 WOTMAN S J PERIODONTOL 44 278 973 CT 1 BARDON A CLIN CHIM ACTA 101 17 980 PN 77038 RN 00620 AN 77266353 AU Himmelhoch-A. TI The effects of chronic illness on children of 0-3 years and their families. SO Australas-Nurses-J. 1977 Jun. 6(11). P 14-6. MJ ADAPTATION-PSYCHOLOGICAL. CHRONIC-DISEASE. PARENT-CHILD-RELATIONS. MN AFFECTIVE-SYMPTOMS. CHILD-PRESCHOOL. CYSTIC-FIBROSIS. HUMAN. INFANT. INFANT-NEWBORN. MENTAL-RETARDATION. NEOPLASMS. EX I have taken as examples of chronic illness in infancy four different types of disability: emotional deprivation, infants with sub-normal development, children suffering from mucodiscoidosis, and children who have neoplastic disease. RF 001 KLAUS MH PEDIATRICS 46 187 970 002$ KLAUS MH IN: GAIRDNER D 133 976 003 BUDIN P NURSLING 907 004 MONCRIEFF J MENTAL SUBNORMALITY IN LONDON 966 005 HEWETT S FAMILY AND HANDICAPPED CHILD 970 006 KAPLAN DM MATERNAL REACTION PREMATURE B 965 PN 77039 RN 00621 AN 78080307 AU Christian-S-T. Monti-J-A. Finley-W-H. TI Membrane fluidity in normal and cystic fibrosis fibroblasts. SO Biochem-Biophys-Res-Commun. 1977 Dec 7. 79(3). P 966-72. MJ CELL-MEMBRANE: ul. CYSTIC-FIBROSIS: pp. MN CELLS-CULTURED. FIBROBLASTS: ph. HUMAN. MALE. PHOSPHOLIPIDS. SKIN: ph, pp. SPECTROMETRY-FLUORESCENCE. TEMPERATURE. SUPPORT-U-S-GOVT-NON-P-H-S. AB We have observed distinct differences in the polarization of fluorescence and temperature dependent emission intensity of the highly fluoescent phospholipid derivative (1-acyl-2-(N-4-nitrobenzo-2-oxa-1,3-diazole)--aminocaproyl phosphatidylcholine (NBD-PC), when incorporated in the plasma membranes of normal and cystic fibrosis fibroblasts. Fluorescence polarization measurements indicate that the fluorochrome has a much higher degree of rotational mobility in cystic fibrosis fibroblasts as compared with normal cells. Temperature dependent transitions in the emission intensity of NBD-PC incorporated in normal fibroblasts are indicated at 17.7 and 21.2 degrees C while the abnormal cell membranes apparently undergo transitions at 8.7 and 13.5 degrees C. These differences might be due to changes in plasma membrane composition and/or organization, in the case of the cystic fibrosis cells. RF 001 SINGER SJ SCIENCE 175 720 972 002 GITLER C ANNU REV BIOPHYS BIOENG 1 51 972 003 SHINITZKY M FEBS LETTERS 24 247 973 004 INBAR M J MOL BIOL 81 245 973 005 RUDY B BIOCHIM BIOPHYS ACTA 288 231 972 006 COGAN U BIOCHEMISTRY 12 521 973 007 MONTI JA PROC AM SOC NEUROCHEM 8 75 977 008 CHEN RF SCIENCE 147 729 965 009 SKLAR LA J SUPRAMOL STRUCT 4 449 976 010 BUTTERFIELD DA PROC NAT ACAD SCI USA 71 909 974 011 BUTTERFIELD DA BIOCHEMISTRY 13 5078 974 012 BUTTERFIELD DA NATURE 263 159 976 013 BUTTERFIELD DA ARCH BIOCHEM BIOPHYS 177 226 976 014 BUTTERFIELD DA NATURE 267 453 977 CT 1 SHINITZKY M BIOCHIM BIOPHYS ACTA 515 367 978 2 MONTI JA ARCH BIOCHEM BIOPHYS 193 496 979 3 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 4 RIMON G BIOCHEMISTRY 19 4451 980 5 CHASE HP METABOLISM 29 365 980 6 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 7 HARRIS A CLIN CHIM ACTA 128 41 983 PN 77040 RN 00622 AN 78080277 AU Hosli-P. Vogt-E. TI Cystic fibrosis: leakage of lysosomal enzymes and of alkaline phosphatase into the extracellular space. SO Biochem-Biophys-Res-Commun. 1977 Dec 7. 79(3). P 741-8. MJ ALKALINE-PHOSPHATASE: me. CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: me. LYSOSOMES: en. MN CELLS-CULTURED. FIBROBLASTS: en. HUMAN. MUCOLIPIDOSIS: en. SKIN: en. AB Nine lysomal enzymes and alkaline phosphatase have been assayed with two different ultramicro techniques in the intra- and extracellular space of fibroblast cultures derived from the skin of cystic fibrosis patients, cystic fibrosis carriers, and normals controls, respectively. Evidence has been obtained for a multiple leakage of lysomal enzymes and of alkaline phosphatase into the medium of fibroblast cultures from cystic fibrosis patients and carriers. The situation is comparable to a certain extent, to that observed in I-cell-disease (mucolipidosis II). This multiple leakage results in the decrease of intracellular activity of several lysosomal enzymes in cultures from cystic fibrosis patients and carriers and due to the coordinate regulationof the synthesis of the "leaky enzymes" in an overshooting of the intracellular alkaline phosphatase activity in cultures from cystic fibrosis patients. It also explains the retarded catabolism of certain molecules, such as the Tamm-Horsfall glycoprotein, in cystic fibrosis cells. It is speculated that the basic defect in cystic fibrosis leads to abnormal recognition sites on lysosomal enzymes and on alkaline phosphatase, and in consequence to the leakage of those enzymes into the extracellular space. The present findings allow one to develop methods for the pre- and postnatal diagnosis of cystic fibrosis with cell cultures, and for the detection of cystic fibrosis carriers with the peripheral blood. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 LIGHTBODY J LANCET 1 451 971 003 HOSLI P ADV EXP MED BIOL 68 1 976 004 HOSLI P TISSUE CULTIVATION ON PLASTIC 972 005 HOSLI P IN: MOTULSKY AG 226 974 006 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 007 HOSLI P ADV EXP MED BIOL 76A 591 977 008 HOSLI P CLIN CHEM 23 1476 977 009 HICKMAN S BIOCHEM BIOPHYS RES COMMUN 49 992 972 CT 1 ALHADEFF JA CLIN GENET 14 189 978 2 HOSLI P ACTA PAEDIATR SCAND 67 617 978 3 CAREY WF MED J AUST 2 604 979 4 HOSLI P LANCET 2 543 979 5 HOSLI P MONOGR PAEDIATR 10 90 979 6 VLADUTIU GD LIFE SCI 24 2369 979 7 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 8 ALHADEFF JA BIOCHEM BIOPHYS RES COMMUN 86 787 979 9 SCANLIN TF CLIN CHIM ACTA 91 197 979 10 WILLCOX P CLIN CHIM ACTA 91 81 979 11 OKADA S CLIN CHIM ACTA 96 281 979 12 JAKEL HP BIOL ZENTRALBL 98 55 979 13 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 14 HOSLI P FEBS LETTERS 104 271 979 15 ROSSI E SCHWEIZ MED WOCHENSCHR 109 33 979 16 WIJCIK L BIOCHIM BIOPHYS ACTA 585 374 979 17 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 18 DAVIS PB PEDIATR RES 14 83 980 19 AITKEN DA J MED GENET 17 187 980 20 FASTH A ACTA PAEDIATR SCAND 69 189 980 21 ALHADEFF JA CLIN CHIM ACTA 105 131 980 22 MALER T FEBS LETTERS 121 153 980 23 JESSUP W BIOCHEM J 190 847 980 24 VLADUTIU GD BIOCHEM J 192 813 980 25 KRAKAUER KA PROG DIS SKIN 1 63 981 26 HALLINAN F MED HYPOTHESES 7 793 981 27 KATZNELSON D ISR J MED SCI 17 687 981 28 HARRIS A CLIN GENET 20 315 981 29 JAKEL HP BIOL ZENTRALBL 100 273 981 30 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 31 HULTBERG B CLIN CHIM ACTA 112 167 981 32 ALHADEFF JA CLIN CHIM ACTA 117 227 981 33 MALER T J BIOL CHEM 256 1420 981 34 FIGARELLA C EUR J CLIN INVEST 12 145 982 35 HUGHES WT PEDIATR RES 16 874 982 36 JESSUP W BIOCHEM BIOPHYS RES COMMUN 105 922 982 37 ANDERSON PJ BIOCHEM BIOPHYS RES COMMUN 108 182 982 38 JESSUP W CLIN CHIM ACTA 126 265 982 39 CEDER O SCANN ELECTRON MICROSC 1982 723 982 40 MARGOLIES R PEDIATR RES 17 931 983 41 CEDER O CLIN GENET 23 298 983 42 HEELEY AF CLIN CHEM 29 2011 983 43 CEDER O ACTA PAEDIATR SCAND 72 291 983 44 BOURASSA C CLIN CHIM ACTA 129 263 983 45 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 46 OGLESBEE LH CLIN CHIM ACTA 143 135 984 47 ROOMANS GM ANN NY ACAD SCI 428 121 984 48 BOUE A PRESSE MED 14 575 985 49 ALHADEFF JA PEDIATR RES 19 171 985 50 BRAGANZA JM MED HYPOTHESES 20 233 986 51 BOZON D ARCH BIOCHEM BIOPHYS 249 546 986 52 HERMELIN B CELL MOL BIOL 33 83 987 PN 77041 RN 00623 AN 78080294 AU Scanlin-T-F. Matacic-S-S. Pace-M. Santer-U-V. Glick-M-C. TI Abnormal distribution of alpha-L-fucosidase in cystic fibrosis: increased activity in skin fibroblasts. SO Biochem-Biophys-Res-Commun. 1977 Dec 7. 79(3). P 869-76. MJ CYSTIC-FIBROSIS: en. FUCOSIDASE: me. SKIN: en. MN ACETYLGLUCOSAMINIDASE: me. ACID-PHOSPHATASE: me. ADOLESCENCE. ADULT. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FIBROBLASTS: en. GLUCURONIDASE: me. GLYCOSIDE-HYDROLASES: me. HUMAN. INFANT. REFERENCE-VALUES. SUPPORT-U-S-GOVT-P-H-S. AB Alpha-L-Fucosidase activity is elevated in skin fibroblasts from cystic fibrosis patients when compared to controls. The activities of nine other acid hydrolases including neuraminidase are similar in cystic fibrosis and control fibroblasts. The relationship of these results to the recent finding of a decreased activity of alpha-L-fucosidase in the serum of cystic fibrosis patients is discussed. It is proposed that an abnormal distribution of alpha-L-fucosidase is involved in the pathogenesis of this disease. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 SHWACHMAN H AM J DIS CHILD 96 6 958 005 LOWRY OH J BIOL CHEM 193 265 951 006 ANTONOWICZ I PEDIATR RES 6 803 972 008 NISHIGAKI M J BIOCHEM (TOKYO) 75 509 974 009 ALHADEFF JA J BIOL CHEM 250 7106 975 010 DISCHE Z PEDIATRICS 24 74 959 011 NOVAK RA TEX REP BIOL MED 34 199 976 012 PEARSON RD PEDIATR RES 11 462 977 013 ZIELKE K J EXP MED 136 197 972 014 BERMAN ER CLIN CHIM ACTA 52 115 974 015 BERATIS NG PEDIATR RES 11 862 977 016 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 017 DI MATTEO G BIOCHIM BIOPHYS ACTA 429 527 976 018 TURNER BM AM J HUM GENET 27 651 975 019 ASHWELL G ADV ENZYM MOLEC BIOL 41 99 974 020 NEUFELD EF ANNU REV BIOCHEM 44 357 975 021 KAPLAN A PROC NAT ACAD SCI USA 74 2026 977 CT 1 ALHADEFF JA CLIN GENET 14 189 978 2 ALHADEFF JA CLIN CHIM ACTA 89 469 978 3 SANTER UV BIOCHIM BIOPHYS ACTA 523 435 978 4 ALHADEFF JA BIOCHEM BIOPHYS RES COMMUN 86 787 979 5 SCANLIN TF CLIN CHIM ACTA 91 197 979 6 WILLCOX P CLIN CHIM ACTA 91 81 979 7 BUTTERWORTH J CLIN CHIM ACTA 92 109 979 8 SCANLIN TF CLIN CHEST MED 1 424 980 9 SANTER UV BIOCHEM BIOPHYS RES COMMUN 96 219 980 10 ALHADEFF JA CLIN CHIM ACTA 105 131 980 11 MALER T FEBS LETTERS 121 153 980 12 JAKEL HP BIOL ZENTRALBL 100 273 981 13 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 14 SCANLIN TF CLIN CHIM ACTA 114 269 981 15 ALHADEFF JA CLIN CHIM ACTA 117 227 981 16 MALER T J BIOL CHEM 256 1420 981 17 SCANLIN TF BIOCHEMISTRY 21 491 982 18 FRATES RC PEDIATR RES 17 30 983 19 MARGOLIES R PEDIATR RES 17 931 983 20 ALHADEFF JA CLIN CHIM ACTA 134 1 983 21 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 22 SCANLIN TF PEDIATR RES 19 368 985 23 PORTER WH CLIN CHEM 32 652 986 24 BOZON D ARCH BIOCHEM BIOPHYS 249 546 986 25 HERMELIN B CELL MOL BIOL 33 83 987 PN 77042 RN 00624 AN 78039817 AU Kelly-J-C. DeBusk-A-G. TI Membrane function in cystic fibrosis. I. Putrescine transport in normal and cystic fibrosis fibroblasts. SO Biochem-Genet. 1977 Aug. 15(7-8). P 695-705. MJ CYSTIC-FIBROSIS: me. PUTRESCINE: me. MN BIOLOGICAL-TRANSPORT. CELL-LINE. FIBROBLASTS: me. HUMAN. SKIN. SUPPORT-U-S-GOVT-P-H-S. AB Putrescine transport was examined in normal and cystic fibrosis fibroblasts. No differences were observed in accumulation pattern, kinetics of uptake, or efflux between CF and normal cells. In both growing and growth-arrested CF and normal fibroblasts, exogenously supplied putrescine remained unchanged for at least 60 min. Some differences were observed in the response of CF and normal cells to environmental (media) changes. RF 001 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 002 BAKER AP FED PROC 33 1300 974 003 BENKE PJ LANCET 1 182 972 004 BOSE SK PROC NAT ACAD SCI USA 70 2374 973 005 BROWN GA LANCET 2 639 971 006 COHEN LF PEDIATR RES 9 312 975 007 FLETCHER DS CLIN CHIM ACTA 44 5 973 008 JOHNSON HG ESCHERICHIA COLI ARCH BIOCHEM 128 113 968 009 KANO K J BIOL CHEM 251 2795 976 010 KLETZIEN RF J BIOL CHEM 249 3366 974 011 LOWRY OH J BIOL CHEM 193 265 951 012 LUNDGREN DW CLIN CHIM ACTA 62 357 975 013 MCEVOY FA PEDIATR RES 9 721 975 014 MORTON HJ IN VITRO 6 89 970 015 MUNRO GF J BIOL CHEM 247 1272 972 016 MUNRO GF J BACTERIOL 118 952 974 017 POHJANPELTO P J CELL BIOL 68 512 976 018 RENNERT OM IN: MANGOS JA 41 973 019 ROSENTHAL SM J PHARMACOL EXP THER 116 131 956 020 SCHNEIDER EL EXP CELL RES 84 311 974 022 TABOR CW J BIOL CHEM 241 3714 966 023 TABOR H PHARMACOL REV 16 245 964 024 TABOR H ADV ENZYMOL 36 203 972 025 TAUSSIG LM LANCET 1 1367 972 026 WILLIAMS-ASHMAN HG ANN NY ACAD SCI 171 882 970 CT 1 SULLIVAN JL BIOCHEM GENET 15 1125 977 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 LIMBOSCH S CLIN CHIM ACTA 102 11 980 PN 77043 RN 00625 AN 78103121 AU Sullivan-J-L. Kelly-J-C. Roess-W-B. DeBusk-A-G. TI Membrane function in cystic fibrosis. II. Methionine transport in normal and cystic fibrosis fibroblasts. SO Biochem-Genet. 1977 Dec. 15(11-12). P 1125-32. MJ CYSTIC-FIBROSIS: me. METHIONINE: me. MN BIOLOGICAL-TRANSPORT-ACTIVE. CELL-LINE. CELLS-CULTURED. FIBROBLASTS: me. HUMAN. KINETICS. SKIN: cy. SUPPORT-U-S-GOVT-P-H-S. AB Initial rate kinetics of methionine transport, time course of accumulation of methionine, and efflux of accumulated methionine were studied in three normal and four CF human diploid fibroblast strains. The range of apparent Km's was 12.7-32.1 micrometer for the CF strains and 18.3-39.2 micrometer for the normal strains. The range of apparent Vmax's was 6.69-9.22 nmole mg-1 min-1 for the CF strains and 5.59-7.87 nmole mg-1 min-1 for the normal strains. The patterns of accumulation and efflux are quite similar in all the strains studied except for WI-38, which showed somewhat higher efflux and lower accumulation than for others. There was no significant difference in the kinetic parameters of methionine transport between CF and normal skin fibroblasts, and methionine transport will not serve as a marker for cystic fibrosis in cultured fibroblasts. RF 001 BENKE PJ LANCET 1 182 972 002 BERATIS NG PEDIATR RES 7 958 973 003 BOSE SK PROC NAT ACAD SCI USA 70 2374 973 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 006 DANES BS LANCET 1 1061 968 007 DANES BS J EXP MED 129 775 969 008 FLETCHER DS CLIN CHIM ACTA 44 5 973 009 HODES ME PEDIATR RES 8 212 974 010 KELLY JC BIOCHEM GENET 15 695 977 011 KLAGSBRUN M PEDIATR RES 8 205 974 012 KLETZIEN RF J BIOL CHEM 249 3366 974 013 LOBECK CC IN: STANBURY JB 1605 972 014 LOWRY OH J BIOL CHEM 193 265 951 015 LUNDGREN DW CLIN CHIM ACTA 62 357 975 016 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 017 MCCOMBS ML TEX REP BIOL MED 31 615 973 018 PLATTER H PROC SOC EXP BIOL MED 123 140 966 019 RENNERT OM CLIN PEDIATR 11 351 972 020 RENNERT OM PEDIATRICS 50 485 972 021 RENNERT OM IN: MANGOS JA 41 973 022 SPOCK A PEDIATR RES 1 173 967 023 WELCH DW PEDIATR RES 9 698 975 024 WILSON RG CLIN CHIM ACTA 36 189 972 PN 77044 RN 00626 AN 78060657 AU Sakula-A. TI Bronchial asthma due to allergy to pancreatic extract: a hazard in the treatment of cystic fibrosis. SO Br-J-Dis-Chest. 1977 Oct. 71(4). P 295-9. MJ ASTHMA: ci. DRUG-HYPERSENSITIVITY: et. PANCREATIN: ae. MN ADULT. CASE-REPORT. CYSTIC-FIBROSIS: dt. FEMALE. HUMAN. PANCREATIN: ad, tu. RESPIRATORY-FUNCTION-TESTS. AB The mother of two children suffering from cystic fibrosis developed marked allergic symptoms affecting the eyes, nose and bronchi and severe bronchial asthma ensued. The cause of this allergy proved to be the powder released on opening the capsules of pancreatic extract which she provided for her children at each mealtime. The risk of pancreatic extract to those handling it, and possibly also to patients suffering from cystic fibrosis, is emphasized. RF 001 BERGNER A PEDIATRICS 55 814 975 002 CHIGNELL R PROC R SOC MED 65 679 972 003 COOMBES RRA IN: GELL PGH 575 968 004 DARBY CW BR MED J 2 299 970 005 DAY G ARCH DIS CHILD 48 355 973 006 DERBES VJ J ALLERGY 28 287 957 007 DOLAN TF JR AM REV RESPIR DIS 110 812 974 008 FLINDT MLH LANCET 1 1177 969 009 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 010 ANON MARTINDALE EXTRA PHARMACOPOE 684 972 011 MITCHELL-HEGGS PF Q J MED 45 479 976 012 NAKAMURA S JAPAN J ALLERGY 20 361 971 013 NAKAMURA S J ASTHMA RES 10 37 972 014 PEPYS J CLIN EXP IMMUNOL 1 377 966 015 PILAT L REV FR ALLERGIE 7 153 967 016 ANON BR MED J 2 161 970 017 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 018 WARREN CPW CLIN ALLERGY 5 1 975 019 WOOD RE AM REV RESPIR DIS 113 833 976 020 YOHE RM ANN ALLERGY 30 627 972 CT 1 FLINDT MLH LANCET 1 430 978 2 ORMEROD LP THORAX 35 768 980 3 HARTMANN AL SCHWEIZ MED WOCHENSCHR 114 916 984 4 LIPKIN GW LANCET 1 392 987 PN 77045 RN 00627 AN 77135753 TI Screening for cystic fibrosis [editorial]. SO Br-Med-J. 1977 Mar 5. 1(6061). P 596-7. MJ CYSTIC-FIBROSIS: pc. MASS-SCREENING. MN HUMAN. INFANT-NEWBORN. EX Cystic fibrosis (mucoviscidosis) is the most common potentially lethal autosomal recessive disease in Caucasian peoples. Despite much research and several promising leads there is no completely reliable test for detecting heterozygotes, nor a test for the identification of the affected homozygous fetus in utero. Attention is being focused on the value and the means of screening newborn infants. Pilot studies carried out in the past six years have indicated two main possibilities. Firstly, the examination of meconium for excess albumin, refined to include immunoelectrophoretic estimation of the albumin alpha-1 antitrypsin ratio, disaccharidases, and lysosomal enzymes. A second approach is based on the work with a chloride-sensitive electrode, which has been modified recently to give greater precision in measurement of neonatal sweat electrolytes. While the tests available are reasonably accurate, collaborative trials and respiratory could improve their efficiency still further. Nevertheless, any screening test is presumptive only, and there must be an adequate back-up service with a specific validated diagnostic test. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 DI SANTAGNESE PA N ENGL J MED 295 597 976 003 BOWMAN BH TEX REP BIOL MED 34 1 976 004 WARD JB JR TEX REP BIOL MED 34 11 976 005 PROSSER R ARCH DIS CHILD 49 597 974 006 STEPHAN U PEDIATRICS 55 35 975 007 RYLEY HC CLIN CHIM ACTA 64 117 975 008 KOPITO L PEDIATRICS 43 794 969 009 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 011 LAWSON D ARCH DIS CHILD 47 1 972 012 GIBSON LE PEDIATRICS 23 545 959 013 COCHRANE AL BR MED BULL 27 3 968 PN 77046 RN 00628 AN 77203082 AU Scott-J-K. TI Cystic fibrosis - Camp Couchiching. four summers. SO Can-Nurse. 1977 Jun. 73(6). P 14-9. MJ CAMPING. CYSTIC-FIBROSIS: rh. MN ADOLESCENCE. CHILD. CYSTIC-FIBROSIS: dt. FEMALE. HUMAN. MALE. ONTARIO. PATIENTS: ed. SPORTS. PATIENT-EDUCATION. EX The camp on Lake Couchiching for children with CF was started in 1973. Initially, certain changes were necessary to make the camp suitable for CF campers. Most of the activities normally available to campers at Camp Couchiching are continued as usual for our CF campers during the month of August. Staff members spend a great deal of time teaching the campers about the need for pancreatic enzymes. Each cystic child requires two or three physiotherapy treatments every day after the inhalation of prescribed bronchodilators and antibiotics. RF 001 CAMPBELL IM LIPIDS 9 916 974 002 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 003 DI SANTAGNESE PA N ENGL J MED 277 1287 967 004 FRIEDMAN M LANCET 1 310 975 005 ANON J PEDIATR 88 711 976 006 STERN RC J PEDIATR 89 412 976 007 STERN RC J PEDIATR 89 406 976 CT 1 STANGHELLE JK ACTA PAEDIATR SCAND 72 935 983 PN 77047 RN 00629 AN 77161496 AU Millis-R-M. Young-R-C-Jr. Kulczycki-L-L. TI Validation of therapeutic bronchoscopic bronchial washing in cystic fibrosis. SO Chest. 1977 Apr. 71(4). P 508-13. MJ BRONCHOSCOPY. CYSTIC-FIBROSIS: th. IRRIGATION. MN ACETYLCYSTEINE: tu. ADOLESCENCE. ADULT. AGE-FACTORS. AIRWAY-RESISTANCE: de. BRONCHI. CARBENICILLIN: tu. CYSTIC-FIBROSIS: dt. GENTAMICINS: tu. HUMAN. MAXIMAL-EXPIRATORY-FLOW-VOLUME-CURVES. MIDDLE-AGE. PANCREATIC-EXTRACTS: tu. SUCTION. SUPPORT-U-S-GOVT-P-H-S. AB Validation of rigid-tube bronchoscopy with small-volume (5-ml increments not to exceed 300 ml) bronchial washing as a therapeutic adjunct was performed on six patients with cystic fibrosis, using serial tests of pulmonary function as a yardstick for assessment of efficacy. Two patients did not undergo the procedure and served as control subjects. All patients were characterized as having varying severity of pulmonary involvement. Large central airways were severely obstructed, and older patients had more trapped gas in their lungs. Hypoxemia and large alveolar-arterial oxygen pressure differences [P(A-a)O2] were due to inhomogeneity of alveolar ventilation. Results indicated that up to ten days to two weeks, bronchoscopic bronchial washing may in some instances improve maximal expiratory flow-volume curves and specific airway conductance and decrease P(A-a)O2 towards normal. Distribution of alveolar gas P(A- a)O2 towards normal. Distribution of alveolar gas became more homogeneous. We conclude that bronchoscopic bronchial washing may be effective in the management of patients with cystic fibrosis, by augmentation of their inadequate cleansing function of the conducting airways. RF 001 WARING WW PEDIATRICS 39 166 967 002 DI SANTAGNESE PA N ENGL J MED 277 1399 967 003 ALTMAN RP J PEDIATR SURG 8 809 973 004 BEIER FR AM REV RESPIR DIS 94 430 966 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 COMROE JH JR AM J MED 10 408 951 007 ROGERS RM CHEST SUPPL 62 95 972 008 RAMIREZ RJ ANN INTERN MED 63 819 965 009 KYLSTRA JA AM REV RESPIR DIS 103 651 971 010 THOMPSON HT THORAX 21 557 966 011 KENSLER CJ AM REV RESPIR DIS SUPPL 93 93 966 012 LOBER CW CHEST 68 382 975 013 BRAUNSTEIN MS CHEST 66 96 974 014 MACKLEM PT J APPL PHYSIOL 22 395 967 015 LAMARRE A PEDIATRICS 50 291 972 016 CORBET A AM REV RESPIR DIS 112 513 975 CT 1 EWING CW CHEST 73 750 978 2 KULCZYCKI LL JAMA 240 30 978 3 EMSLANDER HP INTERNIST 21 11 980 4 ROTHMANN BF ANN OTOL RHINOL LARYNGOL 91 641 982 5 GEMBITSKAYA TE SOV MED 107 984 6 MOAZAM F J PEDIATR SURG 20 398 985 7 SHERMAN JM PEDIATR PULMONOL 2 244 986 PN 77048 RN 00630 AN 78023435 AU Dahm-L-S. Ewing-C-W. Harrison-G-M. Rucker-R-W. TI Comparison of three techniques of lung lavage in patients with cystic fibrosis. SO Chest. 1977 Nov. 72(5). P 593-6. MJ CYSTIC-FIBROSIS: th. IRRIGATION: mt. MN ADOLESCENCE. ADULT. ANESTHESIA-GENERAL. ANESTHESIA-LOCAL. BLOOD-GAS-ANALYSIS. BRONCHOSCOPY: ae, mt. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. HUMAN. IRRIGATION: ae. MALE. VITAL-CAPACITY. AB One hundred fifty-three lung lavage procedures were performed on 81 cystic fibrosis patients during the years 1963 to 1976. Local analgesia was used in 24 lavages done via a rigid bronchoscope and in a group of 73 lavages directed by a fiberoptic bronchoscope. No abnormalities in cardiac rate or rhythm were observed with the lavages directed by fiberoptic bronchoscope. Fifty-six lavages were performed under general anesthesia with 20 cardiac abnormalities being noted among this group. Subjective improvement was noted in 96% of the 56 patients who had lavage under general anesthesia. Objective improvement was apparent in 45% of the 24 patients who had lavage with the rigid bronchoscope and 64% of the 36 patients who had lavage by fiberoptic bronchoscopy. It is concluded that patients with cystic fibrosis of varying stages of disability can more safety (and with good improvement) have bronchial washouts with the fiber-optic bronchoscope than with either of the other two techniques. RF 001 CEZEAUX G JR JAMA 199 15 967 002 HACKETT RR ANESTHESIOLOGY 26 248 965 003 RAMIREZ RJ ANN INTERN MED 63 819 965 004 THOMPSON HT LANCET 2 8 964 005 SACKNER MA AM REV RESPIR DIS 111 62 975 006 MURRAY JF LUNG DISEASE STATE OF THE ART 976 007 ANON RELATIVE VALUE GDE AM SOC ANE 975 CT 1 LUCK JC CHEST 74 139 978 2 LENZINI L RESPIRATION 40 81 980 3 ROTHMANN BF ANN OTOL RHINOL LARYNGOL 91 641 982 4 ROSEGGER H KLIN PAEDIATR 194 381 982 5 HARNIK E ANESTH ANALG CLEVE 62 357 983 PN 77049 RN 00631 AN 78002389 AU McFarlane-H. Allan-J-D. van-der-Zeil-P. TI Passive cutaneous anaphylaxis (PCA) and specific IgE in cystic fibrosis and their heterozygotes. SO Clin-Allergy. 1977 May. 7(3). P 279-84. MJ ANTIBODY-SPECIFICITY. CYSTIC-FIBROSIS: im. HETEROZYGOTE. IGE: an. PASSIVE-CUTANEOUS-ANAPHYLAXIS. MN ADOLESCENCE. ADULT. ALLERGENS: ad. ASTHMA: im. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg. DERMATITIS-ATOPIC: im. FEMALE. HAY-FEVER: im. HUMAN. HYPERSENSITIVITY-IMMEDIATE: im. INTRADERMAL-TESTS. MALE. SALIVA: im. SPUTUM: im. AB The serum from 75% of the patients with cystic fibrosis (C.F.) who had a positive prick test in their skin to at least one or more antigens, together with elevated concentrations of total serum IgE, also gave strong immediate PCA reactions in the baboon skin to Aspergillus fumigatus, bovine serum albumin and egg albumin. Of the C.F. patients, 37% also had elevated serum specific IgE to A. fumigatus whereas only 8-10% had either raised specific IgE or PCA reaction to Dermatophagoides pteronyssinus. Abolition of the PCA activity by incubating the C.F. sera or sputum at 56 degrees C suggested that the reaginic antibody was IgE rather than IgG4. PCA reactions to a number of allergens could be detected in both the C.F. sputum and saliva. Several of the C.F. heterozygotes had a strongly positive history of allergy and a significant number of these heterozygotes had an elevated serum total IgE as well as positive PCA to Timothy grass pollen or to D. pteronyssinus similar to the patients with asthma or hay fever. Three C.F. patients who died gave strong prick test reactions to several allergens and their sera also had raised serum IgE and positive PCA to at least three different allergens, suggesting that immediate hypersensitivity is of some significance in patients with C.F. RF 001 ABBOTT V CAN MED ASSOC J 64 419 951 002 ALLAN JD CLIN ALLERGY 5 255 975 003 BRENCHLEY PEC PROC EUR CONG ALLER IMMUN 9TH 974 004 DERBES VJ J ALLERGY 28 287 957 005 HSIA DYY AM J DIS CHILD 96 685 958 006 JOHANSSON SGO IMMUNOLOGY 10 265 968 007 KLETTER B CLIN ALLERGY 1 249 971 008 LAYTON JL INT ARCH ALLERGY APPL IMMUNOL 23 87 963 009 LURIE MH ANN OTOL RHINOL LARYNGOL 68 478 959 010 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 011 MCFARLANE H LANCET 1 1241 976 012 ROSE NR J ALLERGY 35 520 964 013 SHWACHMAN H PEDIATRICS 30 389 962 014 SHAKIB F CLIN ALLERGY 6 237 976 015 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 016 WARNER JO LANCET 1 990 976 017 WARREN CPW CLIN ALLERGY 5 1 975 CT 1 CARSWELL F MONOGR PAEDIATR 10 144 979 2 CARSWELL F CLIN EXP IMMUNOL 35 141 979 3 ZAMBIE MF ANN ALLERGY 42 290 979 4 VANASPEREN PP AUST PAEDIATR J 16 53 980 5 HODSON ME THORAX 35 801 980 6 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 7 TOBIN MJ THORAX 35 807 980 8 VANASPEREN P AM J DIS CHILD 135 815 981 9 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 10 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 PN 77050 RN 00632 AN 78062991 AU Warner-J-O. TI The variability of skin test hypersensitivity reactions in cystic fibrosis and asthma. SO Clin-Allergy. 1977 Jul. 7(4). P 385-9. MJ ASTHMA: co. CYSTIC-FIBROSIS: co. HYPERSENSITIVITY-IMMEDIATE: di. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. HYPERSENSITIVITY-IMMEDIATE: co. MALE. SKIN-TESTS. AB Cystic fibrosis (CF) children had a greater variability of skin prick test response compared with asthmatic children, when the tests were performed on two occasions. This suggests that there is a different cause for the allergy in the two groups, perhaps because the asthmatics have a transient period of susceptibility to sensitization in infancy and the CF children a persistent vulnerability. Amongst the asthmatic children, clinical improvement was associated with loss of some skin prick test reactions, and clinical deterioration with an increase in the number of positive reactions. Thus continuing allergic reactions may maintain a mucosal defect, resulting in a persistence of susceptibility to allergen sensitization, and control of the reactions may re-establish normal mucosal defence mechanisms. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 LEVINE BB SCIENCE 178 1201 972 003 SOOTHILL JF CLIN ALLERGY 3 511 973 004 TAYLOR B LANCET 2 111 973 005 WARNER JO ARCH DIS CHILD 51 507 976 006 WARNER JO ARCH DIS CHILD 51 507 976 007 WARREN CPW CLIN ALLERGY 5 1 975 CT 1 TOBIN MJ THORAX 35 807 980 2 HOLZER FJ ARCH DIS CHILD 56 455 981 3 CLARKE CW CLIN ALLERGY 12 1 982 PN 77051 RN 00633 AN 78002395 AU Guha-A-K. Kutty-K-M. Chandra-R-K. Way-R-C. TI A study of the salivary glycoprotein in cystic fibrosis patients and controls: fucose incorporation and protein pattern. SO Clin-Biochem. 1977 Aug. 10(4). P 153-5. MJ CYSTIC-FIBROSIS: me. FUCOSE: me. GLYCOPROTEINS: bi. SALIVA: me. MN COMPARATIVE-STUDY. GUANOSINE-DIPHOSPHATE-FUCOSE: me. HUMAN. ISOELECTRIC-FOCUSING. REFERENCE-VALUES. AB The incorporation of fucose to glycoprotein acceptors prepared from the saliva of Cystic Fibrosis (CF) patients was compared with the incorporation into acceptors from controls. The CF acceptor glycoprotein incorporated significantly more fucose in the presence of either patients' or control plasma. The fucosyl transferase activity in the patients' plasma was not significantly different from controls. Fucosidase activity was similar also for both groups. The protein bands of the acceptor glycoproteins from the patients' saliva differed from those of the control in number and electrophoretic mobility. On the basis of these studies of fucose incorporation we propose that glycoprotein in the salivary secretion of E&Apatients are qualitatively different from normal. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 LOBECK CC IN: STANBURY JB 1605 972 003 SHETTLES LB J BIOL CHEM 192 589 951 004 WERNER I ACTA SOC MED UPPSAL 58 1 953 005 DISCHE Z ANN NY ACAD SCI 93 526 962 006 VAZE D PROC INT CF CONG 7TH 976 007 DI SANTAGNESE PA PEDIATRICS 19 252 957 008 BELLA A JR ARCH BIOCHEM BIOPHYS 147 753 971 009 SCHENKEL-BRUNNER H EUR J BIOCHEM 30 269 972 010 RIGHETTI PG J CHROMATOGR 98 271 974 011 GORDON BA CLIN BIOCHEM 3 193 970 012 NORUM KR FEBS SYMPOSIUM 19 333 969 013 BLOMFIELD J AUST PAEDIATR J 10 75 974 CT 1 ALHADEFF JA CLIN GENET 13 417 978 2 ALHADEFF JA CLIN GENET 14 189 978 3 BENYOSEPH Y PEDIATR RES 15 839 981 4 BENYOSEPH Y BIOCHIM BIOPHYS ACTA 718 172 982 5 DURAND P RIV ITAL PEDIATR 9 300 983 6 WESLEY A PEDIATR RES 17 65 983 7 ALHADEFF JA CLIN CHIM ACTA 134 1 983 PN 77052 RN 00634 AN 77203751 AU Hosli-P. TI Quantitative assays of enzyme activity in single cells: early prenatal diagnosis of genetic disorders. SO Clin-Chem. 1977 Aug. 23(8). P 1476-84. MJ ALKALINE-PHOSPHATASE: bi. METABOLISM-INBORN-ERRORS: di. PRENATAL-DIAGNOSIS. MN AMNIOTIC-FLUID: cy. CHROMOSOME-ABNORMALITIES: di. CYSTIC-FIBROSIS: di. ENZYME-INDUCTION. FEMALE. HISTOCYTOCHEMISTRY: is. HUMAN. ISOENZYMES: an. LYSOSOMES: en. PREGNANCY. AB The combined use of special cell culture techniques and biochemical ultramicromethods permits one to handle very small amounts of materials, to reduce the costs of chemicals, and more accurately to assess gene dosage effects by expressing enzyme activities per cell instead of per total cell protein. An alkaline phosphatase induction test has been developed which allows one to screen small numbers of fibroblasts for lysosomal storage diseases, cystic fibrosis, and chromosomal disorders. A successful attempt has been made to automate the microtechniques. Combining the alkaline phosphatase induction with the ultramicro automatization should eventually permit one to screen all pregnancies for major possible fetal genetic defects. Automated ultramicro enzyme assays should contribute to the general development of clinical chemistry. RF 001 LEVY HL ADV HUM GENET 4 1 973 002 MILUNSKY A PRENATAL DIAGNOSIS OF HEREDIT 973 003 HOSLI P ANAT ANZ 120 583 967 004 HOSLI P TISSUE CULTIVATION ON PLASTIC 972 005 HOSLI P BULL EUR SOC HUM GENET 32 972 006 HOSLI P IN: SPERLING O 811 974 007 HOSLI P PRENATAL DIAGNOSIS NEWSLETTER 1 10 972 008 HOSLI P IN: MOTULSKY AG 226 974 009 HOSLI P PROC INT CONG ASSOC SCI 3RD 354 974 010 HOSLI P IN: VOLK BW 1 976 011 LOWRY OH FLEXIBLE SYSTEM OF ENZYMATIC 972 012 ROTMAN B PROC NAT ACAD SCI USA 47 1981 961 013 WUDL L SCIENCE 184 992 974 014 DE BRUYN CHMM BIOCHEM BIOPHYS RES COMMUN 68 483 976 015 NADLER HL IN: BERGSMA D 26 970 016 BROCK DJH LANCET 2 923 973 017 PAUL J CELL AND TISSUE CULTURE 970 018 UITENDAAL MP ADV EXP MED BIOL 76A 597 977 019 GABRIEL A FEBS LETTERS 39 307 974 020 SAIFERT A CLIN CHEM 20 538 974 021 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 023 SCHRODER J J MED GENET 12 230 975 CT 1 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 2 UITENDAAL MP BIOCHEM GENET 16 1187 978 3 HOSLI P CLIN CHEM 24 1325 978 4 HOSLI P HUM GENET 41 169 978 5 HOSLI P ACTA PAEDIATR SCAND 67 617 978 6 BAHR GF ANAL QUANT CYTOL 1 1 979 7 CAREY WF MED J AUST 2 604 979 8 JOLLY RD AM J MED GENET 4 293 979 9 HOSLI P MONOGR PAEDIATR 10 90 979 10 HOSLI P EUR SURG RES 11 205 979 11 KAISER E CLIN BIOCHEM 12 208 979 12 OKADA S ACTA HISTOCHEM CYTOCHEM 12 193 979 13 SCOTT IV CLIN BIOCHEM 12 275 979 14 HOSLI P CLIN GENET 15 487 979 15 UITENDAAL MP IN VITRO 15 103 979 16 SANDHOFF K HUM GENET 50 107 979 17 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 18 GRAVEL RA PROC NAT ACAD SCI USA 76 6520 979 19 OKADA S CLIN CHIM ACTA 96 281 979 20 BENZIE RJ CAN MED ASSOC J 120 685 979 21 AVRAMEAS S J IMMUNOL 122 648 979 22 MALINBERDEL J CYTOMETRY 1 222 980 23 GALJAARD H TRENDS BIOCHEM SCI 5 201 980 24 AITKEN DA J MED GENET 17 187 980 25 BUCHANAN PD CLIN GENET 18 177 980 26 SCHOONDERWALDT HC CLIN GENET 18 348 980 27 VANLAARHOVEN JPRM J IMMUNOL METH 39 47 980 28 GAUSSET P CLIN EXP IMMUNOL 42 294 980 29 MARKOVIC O PERIOD BIOL 82 491 980 30 GLICK D HISTOCHEM J 13 227 981 31 GARDNER HA OTOL CLIN NORTH AM 14 47 981 32 KATO T ACTA HISTOCHEM CYTOCHEM 14 343 981 33 HARRIS A CLIN GENET 20 315 981 34 JAKEL HP BIOL ZENTRALBL 100 273 981 35 DEBERSAQUES J BR J DERMATOL 104 147 981 36 HOSLI P EUR J PEDIATR 137 116 981 37 STEPHENSON SR AM J OBSTET GYNECOL 141 319 981 38 FIGARELLA C EUR J CLIN INVEST 12 145 982 39 GALJAARD H METH CELL BIOL 26 241 982 40 GRAVEL RA ANAL BIOCHEM 119 360 982 41 CEDER O SCANN ELECTRON MICROSC 1982 723 982 42 DEBRUYN CHMM CLIN BIOCHEM 16 38 983 43 BLUTHMANN H DIFFERENTIATION 24 65 983 44 JAKEL HP BIOMED BIOCHIM ACTA 42 1123 983 45 GRAVEL RA HUM GENET 65 112 983 46 CEDER O ACTA PAEDIATR SCAND 72 291 983 47 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 48 LEOTARD B CLIN CHEM 32 1779 986 PN 77053 RN 00635 AN 77114515 AU ODonnell-M-D. FitzGerald-O. McGeeney-K-F. TI Differential serum amylase determination by use of an inhibitor, and design of a routine procedure. SO Clin-Chem. 1977 Mar. 23(3). P 560-6. MJ AMYLASES: bl. PLANT-PROTEINS: pd. MN ADOLESCENCE. ADULT. AMYLASES: ai. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. ISOENZYMES: bl. MALE. MIDDLE-AGE. MUMPS: bl. PANCREAS: en. PANCREATIC-DISEASES: bl. SALIVARY-GLANDS: en. WHEAT. AB We describe a new method for measuring pancreatic and salivary-type amylases in serum that requires no electrophoresis or chromatography. An inhibitor protein (from wheat) with a 100-fold greater specificity for human salivary than for human pancreatic amylase was used to analyze mixtures of the two enzymes. The concentration of pancreatic and salivary amyalase was determined in 141 normal sera (72 men and 69 women). Statistically significant differences were found for serum pancreatic amylase between mean and women, higher values being shown in women. No sex-related difference was found for the salivary component of serum amylase. With this method, the increase in serum amylase activity in pancreatitis was shown to be attributable to the pancreatic component. In mumps, the increase is attributable to the salivary component. In pancreatic insufficiency, serum pancreatic amylase activities were significantly lower than in the controls. Our method is simple and rapid; our results agree well with those of other authors who used chromatographic or electrophoretic methods. RF 001 BERNFELD P HELV CHIM ACTA 31 2165 948 002 BERNFELD P HELV CHIM ACTA 33 1964 950 003 STIEFEL DJ BIOCHIM BIOPHYS ACTA 302 345 973 004 NORBY S EXP CELL RES 36 663 964 005 TAKEUCHI T CLIN CHIM ACTA 54 137 974 006 BERK JE AM J DIG DIS 11 695 966 007 TAKEUCHI T CLIN CHIM ACTA 60 207 975 008 BERK JE AM J GASTROENTEROL 63 457 975 009 ODONNELL MD BIOCHIM BIOPHYS ACTA 422 159 976 010 HALL FF AM J DIG DIS 15 1013 970 011 STIEFEL DJ CLIN CHEM 21 343 975 012 ODONNELL MD ENZYME 18 348 974 013 ROBYT JF IN: RADLEY JA 431 968 014 LEGAZ ME CLIN CHEM 22 57 976 015 AW SE GUT 8 402 967 016 OTSUKI M CLIN CHIM ACTA 22 439 976 017 MCGEACHIN RL ANN NY ACAD SCI 151 208 968 018 MESSER M BIOCHEM J 151 17 975 019 TAKEUCHI T BIOCHIM BIOPHYS ACTA 403 122 975 020 FRIDHANDLER L CLIN CHEM 18 1493 972 021 JANOWITZ HD AM J MED 27 924 959 022 ADLERCREUTZ H BR MED J 3 529 972 023 FITZGERALD O BR MED J 1 349 955 024 KAISER R AM J OBSTET GYNECOL 122 283 975 025 SKUDE G SCAND J GASTROENTEROL 10 577 975 026 AMMAN RW ANN INTERN MED 78 521 973 027 TAUSSIG LM PEDIATRICS 54 229 974 028 TYE JG J MED GENET 13 96 976 CT 1 HEGARTY JE GUT 19 350 978 2 KEOGH JB GUT 19 1125 978 3 BRAGANZA JM CANCER 41 1522 978 4 BERK JE AM J GASTROENTEROL 69 417 978 5 BERK JE ANN INTERN MED 88 838 978 6 KENNY D CLIN CHIM ACTA 89 429 978 7 BERK JE CLIN BIOCHEM 12 264 979 8 GILLARD BK CLIN CHEM 25 1919 979 9 TAKEUCHI T CLIN CHEM 25 1406 979 10 STEPAN J CLIN CHIM ACTA 91 263 979 11 VINICOR F ANN INTERN MED 91 200 979 12 CAILLENS H NOUV PRESSE MED 9 3079 980 13 WALLER SL AUST NZ J MED 10 351 980 14 GILLARD BK PEDIATR RES 14 1168 980 15 MCGEENEY KF PEDIATR RES 14 967 980 16 WOLF RO PEDIATR RES 14 968 980 17 BIRATH K J CLIN CHEM CLIN BIOCHEM 18 680 980 18 HOLMBERG H J CLIN CHEM CLIN BIOCHEM 18 681 980 19 SOFRANKOVA A PHYSIOL BOHEMOSLOV 29 89 980 20 EDERY M ANN BIOL CLIN 38 243 980 21 JACOBSON G SCAND J CLIN LAB INVEST 40 77 980 22 FRIDHANDLER L CLIN CHIM ACTA 101 135 980 23 ODONNELL MD CLIN CHIM ACTA 104 265 980 24 LANKISCH PG DTSCH MED WSCHR 105 1636 980 25 LANKISCH PG MONATSSCHR KINDERHEILKD 128 739 980 26 KLONOFF DC WEST J MED 133 392 980 27 GROARKE JF IR J MED SCI 149 102 980 28 BIRATH K J CLIN CHEM CLIN BIOCHEM 19 616 981 29 DERIJKE D J CLIN CHEM CLIN BIOCHEM 19 815 981 30 JAFFRAY P J CLIN CHEM CLIN BIOCHEM 19 711 981 31 MCGEENEY KF J CLIN CHEM CLIN BIOCHEM 19 231 981 32 ODONNELL MD J CLIN CHEM CLIN BIOCHEM 19 786 981 33 VADER HL J CLIN CHEM CLIN BIOCHEM 19 241 981 34 BOSSUYT PJ CLIN CHEM 27 451 981 35 LANKISCH PG HEPATOGASTROENTEROLOGY 28 333 981 36 BENOIT MO PATHOL BIOL (PARIS) 29 223 981 37 BERK JE AM J GASTROENTEROL 75 128 981 38 TOBIN MJ IR J MED SCI 150 325 981 39 RUDDELL WSJ BR MED J 283 1429 981 40 OCONNOR CM BIOCHIM BIOPHYS ACTA 635 397 981 41 TSIANOS EB LANCET 1 856 982 42 HARDY R CLIN BIOCHEM 15 95 982 43 AMMANN RW SCAND J GASTROENTEROL 17 997 982 44 LANKISCH PG GUT 23 777 982 45 LANKISCH PG DIGESTION 25 211 982 46 ABRAMSON SB DIG DIS SCI 27 889 982 47 ELLIS C DIG DIS SCI 27 897 982 48 HUANG WY CLIN CHEM 28 1525 982 49 RAMMELOO T CLIN CHEM 28 145 982 50 MIWA I CHEM PHARMACEUT BULL 30 362 982 51 BROWN RC J CLIN PATHOL 35 547 982 52 DUCHASSAING D ANN BIOL CLIN 40 448 982 53 MAEDA K AGRIC BIOL CHEM 46 2873 982 54 TSIANOS EB CLIN CHIM ACTA 124 13 982 55 KAMEYA A CLIN CHIM ACTA 125 77 982 56 MOLLERPETERSEN J ACTA MED SCAND 211 459 982 57 BARRETT A BR MED J 285 170 982 58 ROBISON JC N ENGL J MED 307 899 982 59 BRIVET M GASTROENTEROL CLIN BIOL 7 940 983 60 KELLEHER J SCAND J GASTROENTEROL 18 791 983 61 NASRALLAH SM GUT 24 161 983 62 ANDRIULLI A DIGESTION 26 67 983 63 WARCHALEWSKI JR NAHRUNG 27 103 983 64 DERIJKE D CLIN CHEM 29 1100 983 65 HOEK FJ CLIN CHEM 29 995 983 66 LECLERC P CLIN CHEM 29 1020 983 67 ODONNELL MD CLIN CHEM 29 510 983 68 SKRHA J CLIN CHEM 29 407 983 69 SOBIECH KA ENZYME 30 66 983 70 MURAO S AGRIC BIOL CHEM 47 453 983 71 SATZ N KLIN WSCHR 61 91 983 72 OMICHI K J BIOCHEM 94 1797 983 73 DURR GHK DTSCH MED WSCHR 108 1876 983 74 SOYAMA K CLIN CHIM ACTA 131 149 983 75 KIRBY J IR J MED SCI 152 394 983 76 TSIANOS EB CLIN EXP RHEUMATOL 2 235 984 77 PELLETIER G GASTROENTEROL CLIN BIOL 8 792 984 78 SIMPSON JW VET RES COMMUN 8 303 984 79 WILCOX ER J FOOD BIOCHEM 8 189 984 80 KOOP H CLIN GASTROENTEROL 13 739 984 81 PAVESI F RIC CLIN LAB 14 443 984 82 TAIT AD AUST NZ J MED 14 600 984 83 PARVIAINEN MT J CLIN CHEM CLIN BIOCHEM 22 41 984 84 BANKS PA DIG DIS SCI 29 297 984 85 GORDON S CLIN CHEM 30 339 984 86 OKABE H CLIN CHEM 30 1219 984 87 ROYSE VL CLIN CHEM 30 387 984 88 TIETZ NW CLIN CHEM 30 1227 984 89 ZAKOWSKI JJ CLIN CHEM 30 62 984 90 MASIAR PJ NEOPLASMA 31 351 984 91 LANG C THER UMSCH 41 606 984 92 MORGENSTERN T MONATSSCHR KINDERHEILKD 132 661 984 93 HAFKENSCHEID JCM CLIN CHIM ACTA 136 235 984 94 GILLARD BK AM J DIS CHILD 138 577 984 95 OMICHI K CLIN CHIM ACTA 138 197 984 96 GIVEN F IR J MED SCI 153 203 984 97 MOOSSA AR N ENGL J MED 311 639 984 98 MCMAHON LF J CLIN GASTROENTEROL 7 17 985 99 NAGARAJ RH J BIOSCI 7 257 985 100 LAFERLA G IRCS MED SCI BIOCHEM 13 1034 985 101 GIBERTINI P ITAL J GASTROENTEROL 17 211 985 102 LESI C CLIN BIOCHEM 18 317 985 103 ZAKOWSKI JJ CRC CRIT REV CLIN LAB SCI 21 283 985 104 TSIANOS EB CLIN TRIALS J 22 239 985 105 COURTOIS P J CLIN CHEM CLIN BIOCHEM 23 733 985 106 STEINBERG WM DIG DIS SCI 30 1209 985 107 HAFKENSCHEID JCM CLIN CHEM 31 162 985 108 MIFFLIN TE CLIN CHEM 31 1283 985 109 BRAULT D PATHOL BIOL (PARIS) 33 195 985 110 HAFKENSCHEID JCM ENZYME 33 128 985 111 ARAI M AGRIC BIOL CHEM 49 987 985 112 BRAULT D SEM HOP PARIS 61 2847 985 113 MALFERTHEINER P KLIN WSCHR 63 43 985 114 KAMEYA A AM J GASTROENTEROL 80 54 985 115 NIEDERAU C GASTROENTEROLOGY 88 1973 985 116 ITO K J BIOCHEM 97 1357 985 117 STEINBERG WM ANN INTERN MED 102 576 985 118 STRNAD G J ELECTROAN CHEM INTERF ELECT 194 123 985 119 MCCORMICK PA BR MED J 290 1472 985 120 SCHNEIDER MU LANGENBECKS ARCH CHIR 363 149 985 121 MAEDA K BIOCHIM BIOPHYS ACTA 828 213 985 122 KAMEYA S J CLIN GASTROENTEROL 8 438 986 123 MAILLIE AJ SCAND J GASTROENTEROL 21 941 986 124 HARMOINEN A J CLIN CHEM CLIN BIOCHEM 24 903 986 125 LANKISCH PG DIG DIS SCI 31 1299 986 126 GORUS F CLIN CHEM 32 398 986 127 JIMENEZ A CLIN CHEM 32 1577 986 128 JUNGLEE D CLIN CHEM 32 609 986 129 LACHER DA CLIN CHEM 32 1539 986 130 TIETZ NW CLIN CHEM 32 301 986 131 WOLF RO CLIN CHEM 32 296 986 132 TSIANOS EB HEPATOGASTROENTEROLOGY 33 247 986 133 BUCHLER M KLIN WSCHR 64 1186 986 134 BARROS FP AM J GASTROENTEROL 81 261 986 135 KAMEYA S AM J GASTROENTEROL 81 358 986 136 LANKISCH PG AM J GASTROENTEROL 81 365 986 137 NAGARAJ RH INDIAN J MED RES 84 89 986 138 OMICHI K J BIOCHEM 100 1353 986 139 ADACHI K CLIN CHIM ACTA 154 103 986 140 MOLLERPETERSEN J CLIN CHIM ACTA 157 151 986 141 HIROISHI S CLIN CHIM ACTA 159 89 986 PN 77054 RN 00636 AN 77089890 AU Applegarth-D-A. Davidson-A-G. Haworth-E-M. Quist-E-E. Zuk-R. Roufogalis-B-D. Clark-D-G. TI Cystic fibrosis: the effect of medium from cultured cystic fibrosis fibroblasts on ATPase activity. SO Clin-Chim-Acta. 1977 Jan 17. 74(2). P 183-5. MJ ADENOSINE-TRIPHOSPHATASE: me. CYSTIC-FIBROSIS: pa. MN ADENOSINE-TRIPHOSPHATASE: ai. CELL-LINE. CELLS-CULTURED. CULTURE-MEDIA: pd. ERYTHROCYTE-MEMBRANE: en. FIBROBLASTS: pa. HUMAN. AB Culture medium from fibroblasts of cystic fibrosis patients and controls was examined for the ability to inhibit (calcium plus magnesium)-activated ATPase and (sodium plus potassium)-activated ATPase. The ATPase systems used were both a solubilised preparation from dog-fish and a membrane associated preparation from human erythrocyes. Contrary to other reports the medium from cystic fibrosis fibroblasts did not inhibit ATPase activity. RF 001 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 002 QUIST EE ARCH BIOCHEM BIOPHYS 168 240 975 003$ HOKIN LE J BIOL CHEM 248 2593 975 004 SEE YP ANAL BIOCHEM 49 430 972 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 77055 RN 00637 AN 78022900 AU Bray-P-T. Clark-G-C. Moody-G-J. Thomas-J-D. TI Sweat testing for cystic fibrosis: errors associated with the in-situ sweat test using chloride ion selective electrodes. SO Clin-Chim-Acta. 1977 Oct 15. 80(2). P 333-8. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. SWEAT: an. MN ELECTRODES. HUMAN. HYDROGEN-ION-CONCENTRATION. METHODS. SKIN-ABSORPTION. TEMPERATURE. AB The in-situ sweat test is prone to errors from various sources. This paper examines errors due to evaporation, absorption of water into the skin, and pressure of the electrode on the skin. Only evaporation caused serious errors. The accuracy and precision when measuring small chloride/sweat samples on the skin are not significantly worse than when measuring bulk solutions. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 003 ANON J PEDIATR 88 711 976 004 DI SANTAGNESE PA GAP CONF REP PROB SWEAT TESTI 6 975 005 WARWICK WJ JAMA 198 59 966 006 WARWICK WJ PEDIATRICS 36 261 965 007 HANSEN L AM J CLIN PATHOL 49 834 968 008 GURSON CT HELV PAEDIATR ACTA 28 165 973 009 BRAY PT PROC EWGCF 5TH ANNU MTG 974 010 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 011 TARNOKY AL CLIN CHIM ACTA 69 505 976 012 SZABO L CLIN CHEM 19 727 973 013 BRAY PT CLIN CHIM ACTA 77 69 977 014 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 015 BLANK IH J INVEST DERMATOL 18 433 952 016 SPOCK A GAP CONF REP PROB SWEAT TESTI 12 975 CT 1 BARNES GL AUST PAEDIATR J 14 78 978 2 WARWICK WJ CLIN CHEM 24 2050 978 3 BUCK RP ANAL CHEM 50 R 17 978 4 BRAY PT ARCH DIS CHILD 53 483 978 5 ROSENSTEIN BJ JAMA 240 1987 978 6 STAGG BH ANN CLIN BIOCHEM 16 147 979 7 SHIRAMIZU B ANAL CHIM ACTA 108 161 979 8 BENSON RG CLIN CHEM 28 2447 982 9 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 PN 77056 RN 00638 AN 77203773 AU Bray-P-T. Clark-G-C. Moody-G-J. Thomas-J-D. TI Sweat testing for cystic fibrosis: characteristics of a combination chloride ion-selective electrode. SO Clin-Chim-Acta. 1977 May 16. 77(1). P 69-76. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHEMISTRY-CLINICAL: is. CHLORIDES: an. DRUG-STABILITY. ELECTRODES. HUMAN. METHODS. TEMPERATURE. AB Filling solution leakage, reproducibility, stability, interferences and temperature effects have been studied in relation to the use of the Orion 96-17-01 combination chloride ion-selective electrode in sweat testing for cystic fibrosis. While stability and reproducibility is good, certain antiseptic systems and especially Hibitaine (chlorhexidine gluconate) interfere with the electrode and therefore need to be absent during sweat testing. Temperature conditions during calibration and sweat testing need to be controlled in view of the extent of response variations for even modest temperature differences between sensor-skin interface and electrode stem. RF 001 GIBSON LE PEDIATRICS 23 545 959 002 BRAY PT PERSPECTIVE OF SODIUM AND CHL 975 003 GIBSON LE GAP CONF REP PROB SWEAT TESTI 975 004 ANON MODEL 417 SKIN CHLORIDE MEASU 971 CT 1 BRAY PT CLIN CHIM ACTA 80 333 977 2 WARWICK WJ CLIN CHEM 24 2050 978 3 WATTS RWE EXPERIENTIA 34 143 978 4 BRAY PT ARCH DIS CHILD 53 483 978 5 LAWRENCE CA BR J HOSP MED 26 625 981 6 SOLSKY RL CRC CRIT REV ANALYT CHEM 14 1 982 PN 77057 RN 00639 AN 77089874 AU Chan-K-Y. Applegarth-D-A. Davidson-A-G. TI Plasma arginine esterase activity in cystic fibrosis of the pancreas. SO Clin-Chim-Acta. 1977 Jan 3. 74(1). P 71-5. MJ ARGINASE: df. CYSTIC-FIBROSIS: en. PANCREAS: pp. MN ADOLESCENCE. ADULT. ARGINASE: bl. CHILD. CHILD-PRESCHOOL. CHLOROFORM: pd. CYSTIC-FIBROSIS: pp. ELLAGIC-ACID: pd. HUMAN. INFANT. KINETICS. METHODS. MICROCHEMISTRY. MIDDLE-AGE. TRYPSIN-INHIBITOR-KUNITZ-SOYBEAN: pd. AB Using a micro-method for the determination of plasma arginine esterase activity, we have investigated the values for soybean trypsin inhibitor (STI)-inhibited arginine esterase activity in patients with cystic fibrosis, obligate heterozygotes and age matched control individuals. The mean of STI-inhibited activity is lowest for cystic fibrosis patients while the mean for normal controls is the highest. The mean of STI-inhibited activity for the heterozygotes is midway between the values of the patients and the normal individuals. The deficiency of arginine esterase activity was statistically significant for both cystic fibrosis patients and heterozygotes. RF 001 RAO GJS SCIENCE 177 610 972 002 RAO GJS PEDIATR RES 8 684 974 003 LIEBERMAN J AM REV RESPIR DIS 109 399 974 004 WILSON GB PEDIATR RES 10 87 976 005 HARPEL PC J EXP MED 138 508 973 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 SIEGELMAN AM ARCH BIOCHEM BIOPHYS 97 159 962 PN 77058 RN 00640 AN 77224156 AU Shapira-E. Ben-Yoseph-Y. Nadler-H-L. TI Abnormal breakdown of alpha2-macroglobulin-trypsin complex in cystic fibrosis. SO Clin-Chim-Acta. 1977 Aug 1. 78(3). P 359-63. MJ ALPHA-MACROGLOBULINS: me. CYSTIC-FIBROSIS: me. TRYPSIN: me. MN COMPARATIVE-STUDY. HUMAN. PROTEIN-BINDING. TRYPSIN-INHIBITOR-KUNITZ-SOYBEAN: pd. SUPPORT-U-S-GOVT-P-H-S. AB The complex of trypsin with purified alpha2-macroglobulin from normals and patients with cystic fibrosis was studied. The formed complex failed to reveal any proteolytic activity toward a high molecular weight substrate whereas the esterolytic activity towards a low molecular weight substrate was retained. This esterolytic activity was resistant to inhibition by a high molecular weight inhibitor. During iincubation at 38 degrees C the complex with normal alpha2-macroglobulin was slowly inhibited by the high molecular weight inhibitor and regained activity with the high molecular weight substrate. This phenomenon was not obtained when the alpha2- macroglobulin from cystic fibrosis was examined. These data suggest that the gradual conversion of normal alpha2-macroglobulin-trypsin complex into an alpha2-macroglobulin fragment-trypsin complex is deficient in patients with cystic fibrosis. RF 001 LAURELL CB IN: PUTNAM FW 246 975 002 HARPEL PC J EXP MED 138 508 973 003 BARRETT AJ BIOCHEM J 133 709 973 004 BAUMSTARK JS BIOCHIM BIOPHYS ACTA 207 318 970 005 WILSON GB PEDIATR RES 10 87 976 006 SHAPIRA E PEDIATR RES 10 812 976 007 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 008 KUNITZ M J GEN PHYSIOL 30 291 947 009 IWAMOTO M BIOCHIM BIOPHYS ACTA 214 402 970 010 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 CT 1 SHAPIRA E J BIOL CHEM 252 7923 977 2 RAO GJS ENZYME 23 314 978 3 CHOY H BIOCHEM BIOPHYS RES COMMUN 82 1325 978 4 VANLEUVEN F PEDIATR RES 13 1384 979 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 7 COMINGS DE AM J HUM GENET 32 273 980 8 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 9 ROMEO G J LAB CLIN MED 95 116 980 10 BURDON MG CLIN CHIM ACTA 100 225 980 11 SCHIDLOW DV AM REV RESPIR DIS 121 31 980 12 COPPENHAVER D HUM GENET 57 399 981 13 RICHMAN JBY CAN J BIOCHEM 59 519 981 14 BLITZER MG J PEDIATR GASTROENTEROL NUTR 1 289 982 15 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 16 WILSON GB MED HYPOTHESES 8 527 982 17 BURY AF IRCS MED SCI BIOCHEM 10 255 982 18 BLITZER MG PEDIATR RES 16 203 982 19 ROBERTS RC PEDIATR RES 16 416 982 20 CRISTOL P SEM HOP PARIS 58 449 982 21 BRIDGES MA CLIN CHIM ACTA 118 33 982 22 TUMMLER B CLIN CHIM ACTA 125 219 982 23 BRIDGES MA ANN NY ACAD SCI 421 360 983 24 SHAPIRA E ANN NY ACAD SCI 421 352 983 25 EAGER KB PEDIATR RES 18 999 984 26 MARYNEN P BIOCHIM BIOPHYS ACTA 799 187 984 27 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 PN 77059 RN 00641 AN 77224220 AU Sunshine-P. Sinatra-F-R. Mitchell-C-H. TI Intractable diarrhoea of infancy. SO Clin-Gastroenterol. 1977 May. 6(2). P 445-61. MJ DIARRHEA-INFANTILE. MN CARBOHYDRATE-METABOLISM-INBORN-ERRORS: co. COLITIS: co. CYSTIC-FIBROSIS: co. DIARRHEA-INFANTILE: di, et, pp. GALACTOSE: me. GLUCOSE: me. HUMAN. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: co. INFANT. INTESTINES: su. MEGACOLON: co. NEOPLASMS: co. NEOPLASMS-EMBRYONAL-AND-MIXED: co. POSTOPERATIVE-COMPLICATIONS. SUPPORT-U-S-GOVT-P-H-S. AB The intractable diarrhoea syndrome of infancy continues to be a major diagnostic and therapeutic challenge to the paediatrician and paediatric gastroenterologist. A carefully organized, staged approach to diagnosis will provide the best method of identifying those infants in whom a specific aetiology exists and for whom specific therapy is often available. Regardless of aetiology, however, the early use of appropriate nutritional support will not only reduce morbidity and mortality in these infants, but will prevent the development of many of the secondary consequences of malnutrition. The physician must compulsively pay attention to the details of daily management and provide an organized approach to diagnosis and treatment in order to improve the outcome of infants with intractable diarrhoea. RF 001 ALVARADO J AM J CLIN NUTR 26 595 973 002 AMENT ME PEDIATR CLIN NORTH AM 22 807 975 003 AMENT ME GASTROENTEROLOGY 62 216 972 004 AVERY GB PEDIATRICS 41 712 968 005 BARBEZAT GO PEDIATRICS 42 77 968 006 BARNES GL ARCH DIS CHILD 48 343 973 007 BLOOM SR LANCET 2 14 973 008 BOWIE MD J PEDIATR 66 1083 965 009 BURKE V LANCET 1 1177 966 010 CHRISTIE DL J PEDIATR 87 657 975 011 CHRISTIE DL J PEDIATR 87 705 975 012 FEINBERG SB RADIOLOGY 80 212 963 013 FELDMAN EJ GASTROENTEROLOGY 70 712 976 014 GHADIMI H PEDIATR RES 7 161 973 015 GRACEY M ARCH DIS CHILD 48 331 973 016 GREENE HL J PEDIATR 87 695 975 017 GRIFFIN JW J PEDIATR 59 394 961 018 HAMILTON JR AM J MED 44 453 968 019 HEIRD WC J PEDIATR 86 2 975 020 HEIRD WC GASTROENTEROLOGY 66 A55 974 021 HERSKOVIC T AM J CLIN NUTR 22 300 969 022 HOFMANN AF GASTROENTEROLOGY 62 918 972 023 HYMAN CJ J PEDIATR 78 17 971 024 JAMES WPT LANCET 1 333 968 025 JAMES WPT ARCH DIS CHILD 46 218 971 026 KEATING JP AM J DIS CHILD 128 369 974 027 LEE PA JAMA 228 585 974 028 LEENDERS E SURG CLIN NORTH AM 50 907 970 029 LIFSHITZ F J PEDIATR 77 595 970 030 LLOYD-STILL JD AM J DIS CHILD 125 358 973 031 MATA LJ AM J CLIN NUTR 25 1118 972 032 MITCHELL C CLIN RES 24 169 976 033 NYLANDER G ACTA CHIR SCAND 133 131 967 034 PHILLIPS SF GASTROENTEROLOGY 63 495 972 035 PHILLIPS SF AM J DIG DIS 18 1017 973 036 PRESS M LANCET 1 597 974 037 PULLAN JM PROC R SOC MED 52 31 959 038 SAID SI N ENGL J MED 293 155 975 039 SCHNEIDER RE AM J CLIN NUTR 25 1092 972 040 SCHNEIDER RE AM J CLIN NUTR 27 777 974 041 SHWACHMAN H AM J DIS CHILD 125 365 973 042 SINATRA F J PEDIATR 88 304 976 043 STRAUS E GASTROENTEROLOGY 66 175 974 044 SUSKIND RM PEDIATR CLIN NORTH AM 22 873 975 045 SWENSON O J PEDIATR SURG 8 587 973 046 VEGHELYI PV AM J DIS CHILD 79 658 950 047 VITERI FE MED CLIN NORTH AM 58 1487 974 048 VITERI FE AM J DIG DIS 18 201 973 049 WILMORE DW J PEDIATR 80 88 972 050 WINICK M PEDIATR RES 3 181 969 051 WRIGHT HK IN: RAVITCH M 971 CT 1 NETZ H KLIN PAEDIATR 190 603 978 2 DESOUSA JS ARCH DIS CHILD 55 937 980 3 ROSSI TM PEDIATRICS 66 730 980 4 FISHER SE DIG DIS SCI 26 181 981 5 ANON J AM DIET ASSOC 78 443 981 6 THOMAS DW GASTROENTEROLOGY 80 776 981 7 STERN M MONATSSCHR KINDERHEILKD 129 51 981 8 ELLIS D AM J DIS CHILD 136 323 982 9 LO CW PEDIATRICS 72 786 983 10 LEBENTHAL E POSTGRAD MED 74 153 983 11 HARMS HK MONATSSCHR KINDERHEILKD 131 428 983 12 ROHDE JE REV INFECT DIS 6 840 984 13 MACFARLANE PI ARCH DIS CHILD 59 260 984 14 AURICCHIO S RIV ITAL PEDIATR 11 383 985 15 PENNY ME BR MED J 292 1223 986 PN 77060 RN 00642 AN 77244733 AU Watkins-J-B. Perman-J-A. TI Bile acid metabolism in infants and children. SO Clin-Gastroenterol. 1977 Jan. 6(1). P 201-18. (REVIEW). MJ BILE-ACIDS-AND-SALTS: me. GASTROINTESTINAL-SYSTEM: me. LIVER: me. MN BILE-ACIDS-AND-SALTS: bi. CHENODEOXYCHOLIC-ACID: me. CHOLESTASIS: me. CYSTIC-FIBROSIS: me. DIARRHEA: me. ENTEROHEPATIC-CIRCULATION. FEMALE. FETUS: me. GALLBLADDER: me. GASTROINTESTINAL-DISEASES: me. HUMAN. INFANT. INFANT-NEWBORN. PREGNANCY. REVIEW. TAURINE: me. SUPPORT-U-S-GOVT-P-H-S. AB It is apparent that bile acid metabolism in the young child must be evaluated within the context of a maturing organism. Accordingly information has been presented so that the physician and the investigator will be able to integrate new data concerning mechanisms of bile salt action or pose new questions concerning previously existing hypotheses of hepatic and intestinal function in children. RF 001 ALAGILLE D J PEDIATR 86 63 975 002 BACK P HOPPE SEYLERS Z PHYSIOL CHEM 354 83 973 003 BISSELL PM GASTROENTEROLOGY 69 809 975 004 BLOOMER JR ANN INTERN MED 82 310 975 005 BONGIOVANNI AM J CLIN ENDOCRINOL METAB 25 678 965 006 BOOK LD PEDIATRICS 57 201 976 007 BURKE V AUST PAEDIATR J 2 219 966 008 CAREY MC AM J MED 49 590 970 009 CHALLACOMBE DN ARCH DIS CHILD 50 837 975 010 CHALLACOMBE DN ARCH DIS CHILD 49 264 974 011 CHALLACOMBE DN ARCH DIS CHILD 49 270 974 012 CLAYTON RJ AM J DIS CHILD 117 112 969 013 COELLO-RAMIREZ P PEDIATRICS 49 233 972 014 DANIELSSON H BIOCHEMISTRY 7 346 968 015 DEBELLE RC GASTROENTEROLOGY 69 815 975 016 DIETSCHY JM J LIPID RES 9 297 968 017 EYSSEN H BIOCHIM BIOPHYS ACTA 273 212 972 018 GARNICA AD PEDIATR RES 10 354 976 019 GRACEY M LANCET 2 384 969 020 GRACEY M SCAND J GASTROENTEROL 6 273 971 021 GRACEY M BIOCHIM BIOPHYS ACTA 225 308 971 022 GRACEY M GUT 12 683 971 023 GRAND RJ GASTROENTEROLOGY 70 790 976 024 HANSON RR J CLIN INVEST 56 577 975 025 HAYES KC SCIENCE 188 949 975 026 HEPNER GW BIOCHIM BIOPHYS ACTA 291 237 973 027 HEPNER GW J CLIN INVEST 51 1889 972 028 HEPNER GW J CLIN INVEST 52 433 972 029 HILL MJ GUT 9 22 968 030 HOFMANN AF J CLIN INVEST 43 247 964 031 INGELMAN-SUNDBERG M BIOCHEMISTRY 14 429 975 032 JACKSON BT J CLIN INVEST 50 1286 971 033 JACOBSEN JG PHYSIOL REV 48 424 968 034 JAVITT NB AM J MED 51 637 971 035 JAVITT NB GASTROENTEROLOGY 70 1172 976 036 JAVITT NB PEDIATR RES 7 119 973 037 KATZ L J PEDIATR 85 608 974 038 KRISHNA GSG PEDIATR RES 9 306 975 039 LAPEY A J PEDIATR 84 328 974 040 LARUSSO NF N ENGL J MED 292 1209 975 041 LAVY U PEDIATR RES 9 306 975 042 LESTER R PEDIATR RES 6 375 972 043 LEWIS R ARCH INTERN MED 130 545 972 044 LINARELLI LG J PEDIATR 83 291 973 045 LINARELLI LG J PEDIATR 81 484 972 046 LITTLE M GASTROENTEROLOGY 69 1315 975 047 LOW-BEER TS BR MED J 1 338 973 048 MAKINO I FEBS LETTERS 15 161 971 049 MATA LJ AM J CLIN NUTR 25 1118 972 050 MORRISSEY KP SURGERY 74 116 973 051 NORMAN A ACTA PAEDIATR SCAND 62 253 973 052 NORMAN A ACTA PAEDIATR SCAND 62 161 973 053 NORMAN A ACTA PAEDIATR SCAND 61 571 972 054 OMAILLE ERL J PHYSIOL (LOND) 180 67 965 055 PALMER RH ARCH INTERN MED 130 606 972 056 POLEY JR J LAB CLIN MED 63 838 964 057 RAIHA NCR PEDIATRICS 53 147 974 058 RICOUR C BIOL GASTROENTEROL 5 545 972 059 RICOUR C REV EUR ETUD CLIN BIOL 17 172 972 060 ROY CC J PEDIATR 86 446 975 061 SAMUEL P PEDIATRICS 54 222 974 062 SANDBERG DH PEDIATR RES 4 262 970 063 SCHNEIDER RE AM J CLIN NUTR 27 777 974 064 SCHNEIDER RE AM J CLIN NUTR 27 777 974 065 SHARP HL J PEDIATR 81 116 972 066 SHARP HL J PEDIATR 71 723 967 067 SHARP HL PEDIATR RES 5 274 971 068 SINGER E ARCH DIS CHILD 49 174 974 069 SMALL DM ARCH INTERN MED 130 552 972 070 SMALLWOOD RA CLIN SCI MOL MED 45 403 974 071 SMALLWOOD RA J CLIN INVEST 51 1388 972 072 STIEHL A N ENGL J MED 286 858 972 073 TAMER MA PEDIATRICS 53 217 974 074 VON BERGMANN J CLIN CHIM ACTA 64 241 975 075 WATKINS JB GASTROENTEROLOGY 70 996 976 076 WATKINS JB PEDIATRICS 53 511 974 077 WATKINS JB N ENGL J MED 288 431 973 078 WATKINS JB GASTROENTEROLOGY 69 706 975 079 WATKINS JB PEDIATR RES 7 341 973 080 WATKINS JB GASTROENTEROLOGY 68 1087 975 081 WEBER AM CLIN CHIM ACTA 39 524 972 082 WEBER AM N ENGL J MED 289 1001 973 083 WILLIAMS CN J PEDIATR 81 493 972 084 WILLIAMS RC GASTROENTEROLOGY 69 483 975 085 WINGATE DL J CLIN INVEST 52 1230 972 086 ZOPPI G PEDIATR RES 6 880 972 CT 1 COWEN AE AUST NZ J MED 7 579 977 2 WATKINS JB AM J CLIN NUTR 31 S148 978 3 HEIKURA S ACTA PAEDIATR SCAND 69 659 980 4 MATHIS RK GASTROENTEROLOGY 79 1311 980 5 SEWELL RB AM J PHYSIOL 239 G354 980 6 HARDY KJ J PHYSIOL (LOND) 309 1 980 7 STRANGE RC PEDIATR RES 15 1425 981 8 BARNES S J CLIN INVEST 68 775 981 9 SUCHY FJ GASTROENTEROLOGY 80 1037 981 10 FINNI K ACTA PAEDIATR SCAND 71 763 982 11 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 S207 983 12 LESTER R J PEDIATR GASTROENTEROL NUTR 2 355 983 13 WATKINS JB J PEDIATR GASTROENTEROL NUTR 2 365 983 14 FINNI K ACTA PAEDIATR SCAND 72 215 983 15 WATKINS JB GASTROENTEROLOGY 85 793 983 16 REICHEN J INT REV EXP PATHOL 26 231 984 17 TAVOLONI N J PEDIATR GASTROENTEROL NUTR 4 256 985 18 BECKETT GJ CLIN CHEM 31 1168 985 19 EGESTAD B SCAND J CLIN LAB INVEST 45 443 985 20 WATKINS JB PEDIATRICS 75 151 985 21 WATKINS JB PEDIATRICS 75 151 985 22 PATTON S J PEDIATR GASTROENTEROL NUTR 5 262 986 23 GUSTAFSSON J J LIPID RES 27 801 986 24 COLOMBO C PEDIATR RES 21 197 987 PN 77061 RN 00643 AN 77224217 AU Lebenthal-E. Shwachman-H. TI The pancreas--development, adaptation and malfunction in infancy and childhood. SO Clin-Gastroenterol. 1977 May. 6(2). P 397-413. MJ PANCREATIC-DISEASES. PANCREATITIS: et. MN AMYLASES: df. CHILD. CYSTIC-FIBROSIS: di, th. ENTEROPEPTIDASE: df. HUMAN. INFANT. METHODS. NEUTROPENIA: di. PANCREAS: ab, em. PANCREATIC-CYST: cn. PANCREATIC-DISEASES: di. PANCREATIC-NEOPLASMS. PEPTIDE-HYDROLASES: df. SYNDROME. TRYPSINOGEN: df. EX Pancreatic development begins during the fourth week of gestation. In annular pancreas, the ventral pancreas fails to migrate to the right of the duodenum. Duplication of the pancreatic ducts is a rare entity, probably arising from accidental misplacement of mucosal cells from the duodenum or stomach during the development of the pancreatic pouches. Congenital pancreatic cysts may be single, multiple, unilocular or multilocular and can be associated with polycystic disease of the kidney, liver or spleen. Ectopic pancreas occurs before or during the rotation of the ventral pancreas and its fusion with the dorsal pancreas. Few tumours of the pancreas have been reported in children less than 15 years of age. The evaluation of patients via different pancreatic function tests is significant only when the results are interpreted in the light of clinical manifestations. Tests of exocrine pancreatic function in childhood include stool examination, the triolein test, blood examination, analysis of duodenal contents, the secretin-pancreozymin test, pancreatic scanning with selenomethionine, transduodenal pancreatogram, abdominal ultrasound and arteriography, and upper gastrointestinal x-ray. The most prevalent disorder of the pancreas in infancy and childhood is exocrine pancreatic insufficiency. Well over 96 per cent of infants and children with pancreatic insufficiency have cystic fibrosis. Shwachman syndrome is the second most common cause of pancreatic insufficiency and is associated with neutropenia, metaphyseal dystosis, sever stunting of growth, eczema, and susceptibility to infections. Patients with cystic fibrosis who have partial pancreatic insufficiency sometimes show dissociation of different enzyme activities. Although pancreatitis is comparatively rare in infancy and childhood, we suspect that it is often overlooked. RF 001 ABRUZZO JL ANN SURG 147 921 958 002 ADAMS JT SURGERY 63 877 968 003 AKERS DR SURGERY 71 817 972 004 APPEL MF ARCH SURG 108 63 974 005$ AREY LB IN: TEXTBOOK LAB MANU EMBRYOL 965 006 BACHRACH WH GASTROENTEROLOGY 63 890 972 007 BALZER E Z GASTROENTEROL 5 239 967 008 BANKS PA GASTROENTEROLOGY 61 382 971 009 BARBERO GJ AM J DIS CHILD 112 536 966 010 BARBEZAT GO PEDIATRICS 42 77 968 011 BECKER WF ANN SURG 163 892 966 012 BERK JE JAMA 199 98 967 013 BISHOP RP AM J GASTROENTEROL 49 112 968 014 BLAINEY JD CLIN SCI 32 377 967 015 BONIN A J PEDIATR 83 594 973 016 BROOKS FP N ENGL J MED 286 300 972 017 BUNNELL CE J PEDIATR 80 465 972 018 CAREY MC GUT 9 700 968 019 CASTLEMAN B N ENGL J MED 286 1353 972 020 COMFORT MW GASTROENTEROLOGY 21 54 952 021 CORNET E J CHIR (PARIS) 84 527 962 022 DI SANTAGNESE PA PEDIATRICS 15 683 955 023 DOLAN RV ARCH SURG 109 762 974 024 DRUMMEY GD N ENGL J MED 264 85 961 025 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 026 FELDMAN M JAMA 148 893 952 027 FRABLE WJ CANCER 27 667 971 028 FREDRICKSON DS N ENGL J MED 276 34 967 029 FRINGLE EM PROC R SOC MED 61 776 968 030 GEOKAS MC CALIF MED 117 1 972 031 GIEDION A FORTSCHR GEB ROENTG NUKL 108 51 968 032 GO VLW J CLIN INVEST 49 1558 970 033 GRACES L PEDIATRICS 41 789 968 034 GREENBERGER NJ MEDICINE (BALTIMORE) 45 161 966 035 GROSFELD J ARCH SURG 101 370 970 036 GROSS JB AM J MED 33 358 962 037 GRYBOSKI J MAJ PROB CLIN PEDIATR 13 450 975 038 GUNDERSEN AE J PEDIATR SURG 4 478 969 039 HADORN B IN: ANDERSON CM 289 975 040 HADORN B CAN MED ASSOC J 98 377 968 041 HADORN B LANCET 1 812 969 042 HOWARD JM ANN SURG 165 293 967 043 JACKSON CE AM J MED 43 727 967 044 KATTWINKEL J PEDIATRICS 51 55 973 045 KILMAN JW SURGERY 55 455 964 046 KLEIN B CLIN CHEM 16 32 970 047 KLEITSCH WP ARCH SURG 71 795 955 048 LAGERLOF HO ACTA MED SCAND SUPPL 128 1 942 049 LEBENTHAL E PEDIATRICS 56 585 975 050 LEBENTHAL E GASTROENTEROLOGY 70 508 976 051 LIECHTY RD JAMA 230 1538 974 052 LILLIBRIDGE CB J PEDIATR 82 279 973 053 LIFTON LJ JAMA 229 47 974 054 LOWE CU AM J DIS CHILD 82 459 951 055 LUNDH G GASTROENTEROLOGY 42 275 962 056 MARCHIS-MOUREN G ARCH BIOCHEM BIOPHYS 83 309 959 057 MARTIN DU PAN R INT Z VITAMINFORSCH 1 67 971 058 MARTINEZ W ANN SURG 147 1 958 059 MATSUMOTO Y SURGERY 76 827 974 060 MCELROY R AM J MED 52 228 972 061 MOORE JG AM J DIG DIS 16 97 971 062 MOYNAN RW J PEDIATR 65 711 964 063 NAGEL W PHYSIOL CHEM 340 1 965 064 NORTHRUP WF SURGERY 71 27 972 065 PATTEN BM HUMAN EMBRYOLOGY 968 066 REETSMA K SURGERY 42 22 957 067 ROBINSON MJ PEDIATRICS 48 232 971 068 SCHMIDT H CLIN ORTHOP 69 135 970 069 SHELDON W ARCH DIS CHILD 39 268 964 070 SHMERLING DH HELV PAEDIATR ACTA 24 547 969 071 SHWACHMAN H IN: SLEISENGER MH 1206 973 072 SHWACHMAN H BIRTH DEF ORIG ART SER 8 46 972 073 SHWACHMAN H ADV PEDIATR 7 249 955 074 SHWACHMAN H PEDIATRICS 55 86 975 075 SHWACHMAN H J PEDIATR 63 835 963 076 SHWACHMAN H J PEDIATR 65 645 964 077 SIBERT JR ARCH DIS CHILD 50 443 975 078 SKUDE G ACTA PAEDIATR SCAND 65 145 976 079 STOVER SL J PEDIATR 73 235 968 080 TAUSSIG LM PEDIATRICS 54 229 974 081 TOWNES PL J PEDIATR 66 275 965 082 URSING B BR MED J 3 524 973 083 WEITZMAN JJ SURGERY 57 309 965 084 WELCH KJ IN: BENSON CD 1 607 962 085$ WITTE CL JAMA 120 132 968 086 WOODLEY JF GUT 13 900 972 087 WORNING H SCAND J GASTROENTEROL 1 268 966 088 WRIGHT E SURGERY 69 389 971 089 ZOPPI G PEDIATR RES 6 880 972 CT 1 THONG YH AUST PAEDIATR J 14 34 978 2 BERG NO ACTA PAEDIATR SCAND 67 403 978 3 LEBENTHAL E AM J DIS CHILD 132 850 978 4 BRANSKI D DIG DIS SCI 24 865 979 5 CASELITZ J VIRCHOWS ARCH PATHOL ANAT HIS 385 109 979 6 FORBES DA AUST PAEDIATR J 16 126 980 7 LEBENTHAL E J PEDIATR 97 389 980 8 SPEER C MONATSSCHR KINDERHEILKD 128 785 980 9 LEBENTHAL E AM J DIS CHILD 134 834 980 10 LEBENTHAL E AM J GASTROENTEROL 75 436 981 11 COLEMAN BG RADIOLOGY 146 145 983 12 ADDA G ANAT CLIN 5 275 984 13 ISAACS JD J CLIN GASTROENTEROL 7 533 985 14 LEBENTHAL E CLIN PERINATOL 13 37 986 PN 77063 RN 00644 AN 77224237 AU Papp-Z. Ember-I. Juhasz-E. Tasnady-Z. Karsai-T. Elodi-P. TI Acid-soluble glycoproteins in amniotic fluid and cystic fibrosis of the foetus. SO Clin-Genet. 1977 Jun. 11(6). P 431-2. MJ AMNIOTIC-FLUID: an. CYSTIC-FIBROSIS: di. GLYCOPROTEINS. PRENATAL-DIAGNOSIS. MN AMNIOCENTESIS. CYSTIC-FIBROSIS: me. FEMALE. GLYCOPROTEINS: an. HETEROZYGOTE. HOMOZYGOTE. HUMAN. PREGNANCY. EX We suggest that the intrauterine detection of cystic fibrosis may be based on the investigation of the amniotic fluid. It is known that the protein content of the meconium of homozygotic newborn infants is significantly increased, whereas that of heterozygotic infants is only moderately increased. As a result of intrauterine intestinal activity, the bowel content may be excreted into the amniotic fluid; thus a detectable deviation in the protein content of amniotic fluid may be expected. We decided to determine the total protein content and the amount of acid-soluble glycoproteins in 86 amniotic fluid samples, both by polography and by measuring the hexose content. In the homozygous case a slight increase was observed in total protein content, whereas in the heterozygous cases no changes were observed. RF 001 CHODOS DD PROC SOC EXP BIOL MED 99 775 958 002 DISCHE Z PEDIATRICS 24 74 959 003 GREEN MN PEDIATRICS 41 989 968 004 KNAUFF RE CLIN CHIM ACTA 19 245 968 005 PAPP Z J PEDIATR 88 151 976 006 DI SANTAGNESE PA PEDIATRICS 19 252 957 CT 1 ALHADEFF JA CLIN GENET 14 189 978 2 HOSLI P LANCET 2 543 979 3 HOSLI P FEBS LETTERS 104 271 979 4 DANN LG PRENAT DIAGN 3 161 983 5 HUHLE D Z KLIN MED 40 1199 985 PN 77064 RN 00645 AN 77067477 AU Schiotz-P-O. Magid-E. TI Serum pancreatic isoamylases in the diagnosis of cystic fibrosis heterozygotes: A non-valuable test. SO Clin-Genet. 1977 Jan. 11(1). P 43-5. MJ CYSTIC-FIBROSIS: fg. GLYCOSIDE-HYDROLASES: bl. ISOAMYLASE: bl. MN ADULT. AGED. CYSTIC-FIBROSIS: di, en. FEMALE. HETEROZYGOTE. HUMAN. MALE. MIDDLE-AGE. PANCREAS: en. SALIVA: en. AB A group of 102 obligate heterozygotes for the cystic fibrosis gene were examined for genetic type of pancreatic isoamylases as well as enzyme activity of pancreatic iso-amylases, salivary isoamylases and total amylase in serum. The analysis was performed using electrophoretic separation for detection of the various types of isoamylases, and their relative activity was determined by means of densitometry. The activities of salivary isoamylases and total amylase in serum showed no significant differences between the cystic fibrosis heterozygotes and a control group. The frequency of the variant gene for pancreatic isoamylase was also the same as in the controls. The mean value for pancreatic isoamylase was slightly higher in the cystic fibrosis heterozygotes than in the control group (0.05 greater than P greater than 0.02). RF 001 CESKA M CLIN CHIM ACTA 26 445 969 002 DANES BS LANCET 1 1061 968 003 DANES BS J EXP MED 129 775 969 005 GIBSON LE PEDIATRICS 23 545 959 006 KAMARYT J HUMANGENETIK 3 41 966 007 KAMARYT J HUMANGENETIK 11 213 971 008 SKUDE G SCAND J CLIN LAB INVEST 35 41 975 009 SKUDE G ACTA PAEDIATR SCAND 65 145 976 CT 1 HUBBARD VS J PEDIATR 92 685 978 2 GILLARD BK PEDIATR RES 14 1168 980 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 77065 RN 00646 AN 78063212 AU Bowman-B-H. Lankford-B-J. McNeely-M-C. Carson-S-D. Barnett-D-R. Berg-K. TI Cystic fibrosis: studies with the oyster ciliary assay. SO Clin-Genet. 1977 Dec. 12(6). P 333-43. MJ BIOLOGICAL-ASSAY: mt. CILIA. CYSTIC-FIBROSIS: bl. OYSTERS. MN CELL-LINE. CULTURE-MEDIA. CYSTIC-FIBROSIS: fg. FIBROBLASTS. GENOTYPE. HUMAN. TISSUE-CULTURE. SUPPORT-U-S-GOVT-P-H-S. AB Bioassays using ciliary systems have detected a factor or factors in cystic fibrosis (CF) sera and tissue culture medium derived from CF cells. The typical shortcomings of an assay measuring biological activity have been studied, and the means to overcome the weaknesses of the oyster gill cilia assay have been established. The presence of the cystic fibrosis mucociliary inhibitor (CFMI) in experimental fractions may be determined by accepting data from only those assays in which authentic CF and normal (non-CF) fractions give defined reactions, by measuring the reaction of each sample at least three times, and by examining each experimental sample at a protein concentration greater than the minimum established in this study. The relative concentrations of the CFMI present in the first steps of purification of serum and medium have been calculated in terms of units of inhibition. Generally, the units of inhibition present in serum and medium fractions from heterozygotes are close to one-half of that in fractions from homozygous sources. Analogous fractions concentrated from a normal (non-CF) source never inhibited mucociliary activity, even when tested at nearly 100 times the CF concentration. Ciliary assays utilizing oyster gills are essential for monitoring fractionation procedures aimed at purifying the CFMI, and have been shown to be capable and reliable enough to do so. RF 001 BARNETT DR TEX REP BIOL MED 31 703 973 002 BARNETT DR TEX REP BIOL MED 31 697 973 003 BAUR PS TEX REP BIOL MED 34 155 976 004 BERATIS NG PEDIATR RES 7 958 973 005 BESLEY GTN J MED GENET 6 278 969 006 BOLTON WE AM J HUM GENET 27 394 975 007 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 008 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 009 BOWMAN BH SCIENCE 164 325 969 010 BOWMAN BH SCIENCE 167 871 970 011 CHERRY JD J PEDIATR 79 937 971 012 CONNEALLY PM TEX REP BIOL MED 31 639 973 013 CONOVER JH PEDIATR RES 7 220 973 014 DANES BS J EXP MED 137 1538 973 015 GALSTOFF PS FISHERY BULL 64 1 964 016 LOCKHART LH TEX REP BIOL MED 31 631 973 017 LOWRY OH J BIOL CHEM 193 265 951 019 SCHMOYER IR LIFE SCI PART 2 11 1037 972 020 SPOCK A PEDIATR RES 1 173 967 021 WILSON GB PEDIATR RES 11 143 977 022 WOOD RE LANCET 2 1452 973 CT 1 DANN LG LANCET 2 405 978 2 BOWMAN BH TEX REP BIOL MED 38 47 979 3 KURLANDSKY LE PEDIATR RES 14 1263 980 4 MAYO BJ CLIN GENET 18 379 980 5 BOWMAN BH FED PROC 39 3195 980 6 SANDERSON MJ PEDIATR RES 15 219 981 7 CARSON SD PEDIATR RES 16 13 982 8 MCNEELY MC PEDIATR RES 16 21 982 9 DULFANO MJ THORAX 37 646 982 10 SANDERSON MJ CELL MOTIL 4 103 984 PN 77066 RN 00647 AN 77114561 AU Danes-B-S. Hodson-M-E. Batten-J. TI Cystic fibrosis: Evidence for a genetic compound from a family study in cell culture. SO Clin-Genet. 1977 Feb. 11(2). P 83-90. MJ CYSTIC-FIBROSIS: fg. MN ADULT. CASE-REPORT. CELL-LINE. CELLS-CULTURED. FEMALE. FIBROBLASTS. HUMAN. PEDIGREE. PHENOTYPE. AB Although the majority of patients with cystic fibrosis (CF) show a typical clinical course, a minority with the same clinical phenotype at the time of initial diagnosis have an atypical (mild) course. Skin fibroblast cultures were established from 49 members of the family of one such atypical CF adult patient, previously identified (Danes et al. 1976) as CF Class II (ametachromatic and no metabolic cooperation with CF Class I fibroblasts), the offspring of Class I (metachromatic, metabolic cooperation with normal fibroblasts)/Class II mating. The culture phenotype for Class I was traced on the maternal side and for Class II on the paternal side through consecutive generations and the culture phenotype of each class segregated. This family study added experimental evidence to support the hypothesis that the atypical (mild) clinical features and course of this adult CF patient were due to two different CF genes combining to produce a genetic compound expressing a mild form of CF. RF 001 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 002 DANES BS BIRTH DEF ORIG ART SER 8 114 972 003 DANES BS LANCET 2 765 973 004 DANES BS CLIN GENET 7 128 975 005 DANES BS J EXP MED 129 775 969 006 DANES BS CLIN GENET 8 85 975 007 DANES BS AM J HUM GENET 23 297 971 008 DANES BS CLIN GENET 9 527 976 009 HALDANE JBS ANN EUGEN 8 263 938 011 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 MCKUSICK VA HERITABLE DISORDERS OF CONNEC 972 015 MCKUSICK VA AM J HUM GENET 25 446 973 016 MATALON R LANCET 2 838 969 017 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 018 WOOD RE LANCET 2 1452 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 WILSON GB PEDIATR RES 12 801 978 3 TULLY GW PEDIATR RES 13 1078 979 4 JAKEL HP BIOL ZENTRALBL 98 55 979 5 TARNOKY AL J ROY SOC MED 73 73 980 6 BULLOCK S CLIN GENET 21 336 982 7 WINGE P DAN MED BULL 29 358 982 8 ROSENSTEIN BJ SOUTH MED J 79 319 986 PN 77067 RN 00648 AN 77067550 AU Kapp-J-P. Arora-B. Sibinga-M-S. TI Depression of erythropoiesis, physical growth, and hair growth by chloramphenicol in a three-year-old child with cystic fibrosis. SO Clin-Pediatr (Phila). 1977 Jan. 16(1). P 64-6. MJ CHLORAMPHENICOL: ae. CYSTIC-FIBROSIS: dt. ERYTHROPOIESIS: de. GROWTH: de. HAIR: gd. MN ANOREXIA: ci. CHILD-PRESCHOOL. CHLORAMPHENICOL: tu. CYSTIC-FIBROSIS: pp. DEPRESSION-CHEMICAL. FEMALE. HUMAN. SUPPORT-U-S-GOVT-P-H-S. EX In patients with cystic fibrosis (CF) of the pancreas, optic neuritis and peripheral neuritis have been reported to follow treatment with chloramphenicol. This toxic sequel appears to be related to the dosage and duration of therapy. In patients with other diseases who are treated with chloramphenicol, the risk of devastating bone marrow toxicity has been estimated to range from 1:40,000 to 1:100,000. We have found only two reports of patients with CF who had hematologic complications after treatment with chloramphenicol. We here report a 3-year-old girl who had selective depression of the erythroid bone marrow, anorexia, weight loss, temporary hair loss and retardation of growth after prolonged administration of chloramphenicol. RF 001 DENNING CR J PEDIATR 63 878 963 002 HUANG NN ANTIMICROB AGENTS CHEMOTHER 3 79 963 003 HARLEY RD TRANS AM ACAD OPHTHALMOL OTOL 74 1011 970 004 BEST WR JAMA 201 181 967 005 LLOYD AVC JAMA 184 1001 963 006 SHWACHMAN H PEDIATRICS 46 335 970 007 YUNIS AA SEMIN HEMATOL 10 225 973 008 MCCURDY PR JAMA 176 588 961 009 WEISBERGER AS JAMA 209 97 969 010 AMBROSE CT J EXP MED 117 1075 963 011 DANIEL TM N ENGL J MED 273 367 965 012 SAIDI P J LAB CLIN MED 57 247 961 PN 77068 RN 00649 AN 77114688 AU Goldschmidt-Z. Cohen-H. Isaacsohn-M. Schiller-M. TI Meconium ileus equivalent in an infant, aggravated by soy bean formula. SO Clin-Pediatr (Phila). 1977 Mar. 16(3). P 284-6. MJ CYSTIC-FIBROSIS: co. INFANT-NUTRITION. INTESTINAL-OBSTRUCTION: co. MN CASE-REPORT. HUMAN. INFANT. INTESTINE-SMALL. MALE. SOY-BEANS. EX Meconium ileus in the neonate is the presenting symptom in 10 to 20 per cent of instances of cystic fibrosis of the pancreas. Similar intestinal obstruction occurring later, in the post-neonatal period, is a rare occurrence, and was called "Meconium Ileus Equivalent" by Jensen. Of 40 cases affected by this syndrome reported in medical literature, only 13 were infants. We report a 7-week-old patient who died 3 days after surgery for small bowel obstruction. The clinical suspicion of cystic fibrosis was confirmed at autopsy. RF 001 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 002 KALAYOGLU M J PEDIATR SURG 6 290 971 003 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 004 JAFFE BF ARCH SURG 92 337 966 005 DONNISON AB PEDIATRICS 37 833 966 006 SHAW A J PEDIATR SURG 4 119 969 007 THOMAIDIS TS J PEDIATR 63 444 963 008 TUCKER AS AM J ROENTG RAD THER NUCL MED 112 135 971 009 HUNTON DB GASTROENTEROLOGY 50 99 966 010 BROWN PM N ENGL J MED 263 544 960 011 MULLINS F JAMA 192 741 965 012 CORDONNIER JK SURGERY 54 667 963 013 SIGLER RM MAYO CLIN PROC 40 477 965 014 SNYDER WH JR PEDIATRICS 34 72 964 015 MOSS MH AM J DIS CHILD 117 540 969 016 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 017 OPPENHEIMER EH ARCH PATHOL 96 149 973 CT 1 ROSENBERG E KLIN PAEDIATR 195 323 983 2 SAMUEL N J ULTRASOUND MED 5 425 986 PN 77069 RN 00650 AN 77067548 AU Patterson-P-R. TI Minocycline in the antibiotic regimen of cystic fibrosis patients: weight gain and clinical improvement. SO Clin-Pediatr (Phila). 1977 Jan. 16(1). P 60-3. MJ BODY-WEIGHT: de. CYSTIC-FIBROSIS: dt. MINOCYCLINE: tu. TETRACYCLINES: tu. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. MALE. MINOCYCLINE: pd. SEX-FACTORS. SPUTUM: de. STIMULATION-CHEMICAL. AB Minocycline hydrochloride was given to 100 patients with cystic fibrosis of the pancreas for periods of 3 months over a broad- spectrum antibiotic drug rotation which lasted 2 years. Increased weight gain and some clinical improvement was seen during treatment with minocycline. Those patients with severe disease gained more weight than those with mild or moderate disease, and males gained more than females. When minocycline hydrochloride was not the drug in use, patients lost weight and their health declined. RF 001 SHWACHMAN H AM J DIS CHILD 96 6 958 002 HUANG NN AM J DIS CHILD 120 289 970 003 STUR O WIEN MED WOCHENSCHR 119 395 969 PN 77070 RN 00651 AN 78042532 AU Harrisson-S-P. TI Dysfunctional aspects of a supportive group. SO Community-Health (Bristol). 1977 Aug. 9(1). P 17-9. MJ STRESS-PSYCHOLOGICAL. VOLUNTARY-HEALTH-AGENCIES. MN CHILD. CYSTIC-FIBROSIS: px. HUMAN. AB A number of voluntary organizations exist for the support of patients and their relatives suffering from specific medical conditions. It is usually assumed that membership will provide positive emotional and social support but membership may provide dysfunctional responses. A small-scale study of parents of fibrocystic children provides some evidence of dysfunctional aspects of membership of one specific organization. Further research into the effects of formal and informal reference group activity and the types of support most acceptable to patients and their families is suggested. RF 001 BURTON L PRACTITIONER 210 247 973 002 BURTON L FAMILY LIFE OF SICK CHILDREN 975 003 HARRISSON SP THESIS 975 004 HEWETT S FAMILY AND HANDICAPPED CHILD 970 005 MCCOLLUM AT J PEDIATR 77 571 970 006 MERTON RK SOCIAL THEORY AND SOCIAL STRU 343 968 PN 77071 RN 00652 AN 78084292 AU Longnecker-D-S. TI Environmental factors and diseases of the pancreas. SO Environ-Health-Perspect. 1977 Oct. 20. P 105-12. MJ ENVIRONMENTAL-POLLUTANTS: ae. PANCREATIC-DISEASES: ci. MN AGING. CYSTIC-FIBROSIS: fg. DIABETES-MELLITUS: et. HUMAN. PANCREAS: ab. PANCREATIC-DISEASES: co, pc. PANCREATIC-NEOPLASMS: ci. PANCREATITIS: ci. AB The five major diseases of the pancreas together make a significant contribution to morbidity and mortality among the people of the United States. These diseases are diabetes, cystic fibrosis, acute and chronic pancreatitis, and carcinoma of the exocrine pancreas. Four of these diseases can be modeled in laboratory animals by acute or chronic administration of chemical poisons or carcinogens. Human pancreatic diseases attributed to the effect of chemical agents including alcohol and drugs include many cases of chronic pancreatitis and some cases of acute pancreatitis. The cause is not known in many cases of human pancreatitis, including interstitial, acute, and chronic clinical forms. Epidemiologic studies suggest that the increasing incidence of carcinoma of the exocrine pancreas in the United States may reflect chemical carcinogenesis. On the basis of experimental observations, we know that pancreatic islet cells can be damaged directly by toxic chemicals, and that islet cell tumors can be chemically induced. Thus, there is adequate background data to conclude that several pancreatic diseases of obscure etiology may be due in part to hitherto unidentified toxic effects of chemical agents encountered in personal or general environments. RF 001 SARLES H DIGESTION 9 389 973 002 TOKUHATA GK J CHRON DIS 28 23 975 003 TRAPNELL JE BR MED J 2 179 975 004 OSULLIVAN JN GASTROENTEROLOGY 62 373 972 005 SARLES H MED CLIN NORTH AM 58 1333 974 006 SARLES H ANN NY ACAD SCI 252 171 975 007 DREILING DA ANN NY ACAD SCI 252 187 975 008 ISHII K DIGESTION 9 429 973 009 DIMAGNO EP ANN NY ACAD SCI 252 200 975 010 BOWMAN BH N ENGL J MED 294 937 976 011 CONNEALLY PM TEX REP BIOL MED 31 639 973 012 HADORN B MED CLIN NORTH AM 58 1319 974 013 KRAIN LS J CHRON DIS 23 685 971 014 BATES RR J SURG ONCOL 7 143 975 015 LI FP J NATL CANCER INST 43 1159 969 016 WYNDER EL J NATL CANCER INST 50 645 973 017 FLAKS B EUR J CANCER 5 231 969 018 FLAKS B CHEM BIOL INTERACT 6 91 973 019 RODRIGUEZ TG J ULTRASTRUCT RES 19 116 967 020 RAO MS VIRCHOWS ARCH CELL PATHOL 17 149 974 021 HRUBAN Z CANCER RES 25 708 965 022 VEGHELYI PV AM J DIS CHILD 80 390 950 023 FEDORKO ME LAB INVEST 18 27 968 024 CORNISH AL N ENGL J MED 265 673 961 025 KERN HF VERH ANAT GES 64 115 970 026 STUMPF HH LAB INVEST 5 224 956 027 CHENARD J AM J PATHOL 52 825 968 028 HRUBAN Z LAB INVEST 14 1652 965 029 SARLES H GUT 12 377 971 030 FARBER E PROC SOC EXP BIOL MED 74 838 950 031 LOMBARDI B AM J PATHOL 79 465 975 032 WENK ML J NATL CANCER INST 52 533 974 033 BOQUIST L ACTA PATH MICROBIOL SCAND 76 91 969 034 GOLDBERG RC PROC SOC EXP BIOL MED 74 869 950 035 MARTIN BF J ANAT 104 93 969 036 HRUBAN Z LAB INVEST 14 70 965 037 REDDY JK LAB INVEST 32 98 975 038 KONISHI Y J NATL CANCER INST 52 917 974 039 BOQUIST L LAB INVEST 21 96 969 040 KAUFMAN N ARCH PATHOL 70 331 960 041 MORGAN WS J CELL BIOL 36 261 968 042 WEISS JM J EXP MED 101 213 955 043 LONGNECKER DS LAB INVEST 16 321 967 044 LONGNECKER DS ARCH PATHOL 99 5 975 045 HRUBAN Z J ULTRASTRUCT RES 7 273 962 046 NEVALAINEN TJ VIRCHOWS ARCH CELL PATHOL 18 119 975 047 BREUER RI LAB MED 8 7 977 048 JONES MF N ENGL J MED 267 1029 962 049 JOHNSTON DH JAMA 170 2054 959 050 JONES PE BR MED J 1 133 975 051 WILSON AE LANCET 1 105 967 052 NOGUEIRA JR GASTROENTEROLOGY 62 1040 972 053 DAVIDOFF F N ENGL J MED 289 552 973 054 LONGNECKER DS CANCER RES 35 2249 975 055 POUR P CANCER RES 35 2259 975 056 MORRIS HP J NATL CANCER INST 29 977 962 057 SCHOENTAL R BR J CANCER 31 264 975 058 HAYASHI Y GANN 62 329 971 059 KONISHI Y GANN 67 919 976 060 REDDY JK CANCER RES 35 2269 975 061 RAKIETEN N PROC SOC EXP BIOL MED 137 280 971 062 OAKLEY WG DIABETES AND ITS MANAGEMENT 975 063 ZONANA J N ENGL J MED 295 603 976 064 DUNN JS LANCET 1 484 943 065 FISCHER LJ CRC CRIT REV TOXICOL 3 231 975 066 HRUBAN Z LAB INVEST 26 270 972 067 WOLD JS TOXICOL APPL PHARMACOL 19 188 971 068 RAKIETEN N CANCER CHEMOTHER REPTS 29 91 963 069 WILANDER E ACTA PATH MICROBIOL SCAND (A) 83 206 975 070 BERNE C LANCET 1 173 974 071 ANON HEW PUB NO NIH76-1018 TO 76-1 976 072 DI SANTAGNESE PA N ENGL J MED 295 597 976 CT 1 BALLDIN G J CLIN INVEST 66 159 980 2 DANNI O RES COMMUN SUBST ABUSE 5 67 984 3 VENA JE BR J IND MED 42 85 985 4 PANG VF VET PATHOL 23 310 986 5 ZELINSKYPAPEZ K IN VITRO CELL DEVEL BIOL 23 118 987 PN 77072 RN 00653 AN 77162158 AU Brohet-C-R. Neal-W-A. Warwick-W-J. Tuna-N. TI Reassessment of the diagnostic value of the vectorcardiogram in cystic fibrosis (correlation with clinical score, pulmonary function tests and echocardiogram). SO Eur-J-Cardiol. 1977 May. 5(3). P 221-38. MJ CYSTIC-FIBROSIS: di. ECHOCARDIOGRAPHY. RESPIRATORY-FUNCTION-TESTS. VECTORCARDIOGRAPHY. MN ADOLESCENCE. ADULT. CHILD. FEMALE. FORCED-EXPIRATORY-VOLUME. HUMAN. MALE. PEAK-EXPIRATORY-FLOW-RATE. VITAL-CAPACITY. AB In 21 children with cystic fibrosis, the vectorcardiogram (VCG) was correlated with various indices of the severity of the disease. The best correlation was found between (1) the azimuth angle of the spatial QRS loop area, representing the rightward and posterior shift of the main electrical forces, and pulmonary function tests (PFT): FEV1 % predicted (r = -0.671, P less than 0.005) and VC% predicted (r = -0.607, P less than 0.005), and (2) the right ventricular anterior wall index measured echocardiographically (r = 0.472, P less than 0.05). More VCG parameters correlated significantly with PFT than with the echocardiogram. This shows the major influence of pulmonary changes on the VCG of those patients. In several subgroups with different clinical scores, the VCG were analyzed and compared to normal limits of age- and sex-matched controls. In this small series, the VCG was a specific but not very sensitive method for predicting the degree of severity of the disease. Echocardiography and vectorcardiography can be considered as two complementary techniques in the evaluation of cystic fibrosis. RF 001 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 002 ROYCE SW PEDIATRICS 8 255 951 003 GOLDRING RM J PEDIATR 65 501 964 004 SIASSI B J PEDIATR 78 794 971 005 KEANE JF PEDIATR RES 8 355 974 006 LIEBMAN J CIRCULATION 35 552 967 007 LIEBMAN J CHEST 63 218 973 008 JOHNSON DH PEDIATR RES 8 350 974 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 SCHMITT OH ARCH INTERN MED 96 574 955 011 BROHET CR J ELECTROCARDIOL 8 1 975 012 BROHET CR J ELECTROCARDIOL 8 103 975 013 DOWNS TD IEEE TRANS BIOMED ENG 16 87 969 014 CHOU TC CLINICAL VECTORCARDIOGRAP 103 974 015 KILCOYNE MM CIRCULATION 42 903 970 016 MOSS AJ J PEDIATR 67 797 965 017 FEIGENBAUM H ECHOCARDIOGRAPHY 972 018 QUIVERS WW AM J CARDIOL 14 616 964 019 FLAHERTY JT AM J CARDIOL 20 29 967 020 FOWLER RS PEDIATR RES 8 349 974 CT 1 FOWLER RS J ELECTROCARDIOL 14 319 981 PN 77073 RN 00654 AN 78023997 AU Doering-K-M. Arglebe-C. Lubahn-H. Chilla-R. TI "Fast isoamylases" in the parotid saliva of children with cystic fibrosis and heterozygous carriers. SO Eur-J-Pediatr. 1977 Oct 12. 126(3). P 185-8. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: an. ISOAMYLASE: an. SALIVA: en. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HETEROZYGOTE. HUMAN. MALE. MIDDLE-AGE. PAROTID-GLAND: se. AB On electrophoresis, parotid saliva always exhibits a basic pattern of 6 isoamylases. Additional faster migrating isoamylases occur in varying numbers. These "fast isoamylases" are generated, at least in part, by deamidation. Compared with juvenile and adult controls, a significantly greater number of "fast isoamylases" was found in the parotid saliva of children with cystic fibrosis and their healthy heterozygous parents. A shift in the equilibrium between amidation and deamidation is discussed in terms of its possible connection with the metabolic defect responsible for cystic fibrosis. RF 001 ARGLEBE C LARYNGOL RHINOL OTOL 54 542 975 002 ARGLEBE C CLIN OTOLARYNGOLOGY 1 249 976 003 GREENBERG DM METABOLIC PATHWAYS 3 969 004 IMMICH H MEDIZINISCHE STATISTIK 974 005 KAUFFMAN DL ARCH BIOCHEM BIOPHYS 137 325 970 006 KELLER PJ BIOCHEMISTRY 10 4867 971 007 KULCZYCKI LL AM J DIS CHILD 127 64 974 008 LOWRY OH J BIOL CHEM 193 265 951 009 MANN HB ANN MATH STAT 18 50 947 010 DI SANTAGNESE PA N ENGL J MED 295 481 976 011 STEPHAN U INTERNIST (BERL) 17 336 976 012 STREET HV CLIN CHIM ACTA 1 256 956 013 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 SHAPIRO BL SCIENCE 203 1251 979 2 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 3 TARNOKY AL J ROY SOC MED 73 73 980 4 BARDON A CLIN CHIM ACTA 101 17 980 5 HEELEY AF CLIN CHEM 29 2011 983 PN 77074 RN 00655 AN 78064391 AU Breslow-J-L. Epstein-J. Vayo-M-M. Fontaine-J-H. TI Decreased ouabain binding in cystic fibrosis fibroblasts in potassium-free medium. SO Exp-Cell-Res. 1977 Dec. 110(2). P 399-407. MJ CYSTIC-FIBROSIS: me. OUABAIN: me. MN BIOLOGICAL-TRANSPORT. CULTURE-MEDIA. FIBROBLASTS: me. GLUCOSE: pd. HUMAN. POTASSIUM: pd. RECEPTORS-DRUG: me. RUBIDIUM: me. SUPPORT-U-S-GOVT-P-H-S. AB We have previously demonstrated that cystic fibrosis (CF) cells show increased survival compared to normal cells when exposed to ouabain in medium lacking potassium. In this report, we show that the CF cells bind significantly less ouabain than the normal cells in potassium-deficient medium. Using age-matched normal and CF skin fibroblast strains, we show that (1) at ouabain concentrations < 20x10-9 M binding for both normal and CF cells is linear with time for 1 h and reaches equilibrium after 4 h; (2) at ouabain concentrations between 2 and 20x10-9 M, the initial rate of binding for CF cells is approx. 70% of the normal cells; (3) under equilibrium-binding conditions, Scatchard analysis reveals that three different CF strains have 12, 16, and 44% fewer ouabain-binding sites than their matched normal controls. In addition, studies in potassium-free medium of the inhibition of 86Rb flux (a K-plus analogue) into the cell by ouabain show no differences between CF and normal cells. We have also previously shown that in a low glucose, potassium-deficient medium the CF cells survived ouabain exposure no better than normal cells. In this report, equilibrium-binding studies with [3H]ouabain clearly show that CF cells bind less ouabain under these conditions than normal cells. These results indicate that ouabain resistance in CF cells is not solely a function of differences in ouabain binding. Furthermore, the differential ouabain killing may not be due to ion transport differences, but rather to as yet unknown mechanisms. CF cells thus appear to be unlike previously characterized ouabain-resistant mutants. RF 001 ANON J PEDIATR 88 711 976 002 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 003 BAKER RM CELL 1 9 974 004 LEVER JE J CELL PHYSIOL 88 343 976 005 ROSENBERG HM J CELL PHYSIOL 85 135 975 006 BAKER RM IN: COOK JS 93 976 007 MAYHEW E J CELL PHYSIOL 79 441 972 008 MANKOVITZ R CELL 3 221 974 009 CORSARO CM EXP CELL RES 95 47 975 010 LOWRY OH J BIOL CHEM 193 265 951 011 FREUND JE MODERN ELEMENTARY STATISTICS 967 012 SCATCHARD G ANN NY ACAD SCI 51 660 949 013 QUISSELL DO NATURE 247 115 974 014 AVERDUNK R EXP CELL RES 93 331 975 015 VAUGHAN GL PROC NAT ACAD SCI USA 69 2627 972 016 BAKER PF J PHYSIOL (LOND) 224 441 972 CT 1 EPSTEIN J SOMATIC CELL GENET 4 451 978 2 BRESLOW JL CELL 13 663 978 3 BRESLOW JL SCIENCE 201 180 978 4 REZNIK VM PROC NAT ACAD SCI USA 78 7143 981 5 BRESLOW JL N ENGL J MED 304 1 981 6 LANGHOFF E PEDIATR RES 18 488 984 PN 77075 RN 00656 AN 77185358 AU Lefebvre-D. Ratelle-S. Chartrand-L. Roy-C-C. TI Reduced microbial transformation of bile acids in cystic fibrosis. SO Experientia. 1977 May 15. 33(5). P 616-8. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: mi. MN ADOLESCENCE. CHENODEOXYCHOLIC-ACID: me. CHILD. CHILD-PRESCHOOL. CHOLIC-ACIDS: me. CYSTIC-FIBROSIS: me. FECES: mi. GLYCINE: me. GLYCOCHOLIC-ACID: me. HUMAN. TAURINE: me. TAUROCHOLIC-ACID: me. AB The microbial transformation of bile acids by incubates of stool homogenates from children with cystic fibrosis is decreased. RF 002 GORBACH SL GASTROENTEROLOGY 60 1110 971 003 NEAL G AM J CLIN NUTR 25 1409 972 004 DRASAR BS GASTROENTEROLOGY 56 71 969 005 GRIFFEN WO JR SOUTH MED J 64 1056 971 006 DACK GM J INFECT DIS 54 204 934 007 GOLDSWORTHY NE BR J EXP PATHOL 11 192 930 008 BROWN WR GASTROENTEROLOGY 62 1143 972 009 BRYANT MR AM J CLIN NUTR 25 1485 972 010 FLOCH MH GASTROENTEROLOGY 61 228 971 011 WILLIAMS RC GASTROENTEROLOGY 69 483 975 012 MALLORY A GASTROENTEROLOGY 64 26 973 013 WEBER AM N ENGL J MED 289 1001 973 014 WEBER AM GUT 17 295 976 015 SAMUEL P CIRC RES 33 393 973 016 MIDTVEDT T AM J CLIN NUTR 27 1341 974 017 SOONG CS GASTROENTEROLOGY 63 748 972 018 BINDER HJ AM J CLIN NUTR 28 119 975 019 PERCY-ROBB IW BR MED J 3 813 972 020 KRAG E J CLIN INVEST 53 1686 974 021 HOFMANN AF IN: NAIR PP 2 144 973 CT 1 ROY CC AM J CLIN NUTR 32 2404 979 2 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 3 PARK RW GASTROENTEROLOGY 81 1143 981 4 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 PN 77076 RN 00657 AN 77116784 AU Roller-R-J. Kern-F. TI Minimal bile acid malabsorption and normal bile acid breath tests in cystic fibrosis and acquired pancreatic insufficiency. SO Gastroenterology. 1977 Apr. 72(4 Pt 1). P 661-5. MJ BILE-ACIDS-AND-SALTS: me. BREATH-TESTS. CYSTIC-FIBROSIS: me. MALABSORPTION-SYNDROMES: me. PANCREATIC-DISEASES: me. MN ADULT. AGED. CARBON-DIOXIDE. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: dt. DIARRHEA: me. FECES: an. FEMALE. GLYCOCHOLIC-ACID: me. HUMAN. ILEUM: su. LIPASE: me. LIPIDS: me. MALE. MIDDLE-AGE. PANCREATIC-DISEASES: co, dt. PANCREATIN: me. CELIAC-DISEASE: et. SUPPORT-U-S-GOVT-P-H-S. AB This study was undertaken because of reports of a marked increase in fecal bile acid excretion by children with cystic fibrosis. We attempted to confirm this finding by performing [1-14C]cholylglycine breath tests and by measuring fecal bile acid and fat excretion in patients with cystic fibrosis and acquired pancreatic insufficiency. Studies were done when patients were taking pancreatic enzymes (Cotazym) and also without medication. 14CO2 excretion in breath was normal in patients with acquired pancreatic insufficiency and even lower in cystic fibrosis, both with and without Cotazym therapy. Fecal bile acid excretion was slightly elevated in both groups without Cotazym and became normal with Cotazym in patients with acquired pancreatic insufficiency. Steatorrhea was present in both patient groups and improved during Cotazym therapy. Bile acid malabsorption in cystic fibrosis and acquired pancreatic insufficiency is minimal and probably not clinically important. RF 001 ROLLER RJ GASTROENTEROLOGY 70 930 976 002 SMALL DM ARCH INTERN MED 130 552 972 003 HOFMANN AF GASTROENTEROLOGY 52 752 967 004 KELLY TR ARCH SURG 105 352 972 005 WEBER AM N ENGL J MED 289 1001 973 006 WATKINS JB GASTROENTEROLOGY 68 1087 975 007 WOODBURY JF J CLIN INVEST 50 2531 971 008 SHERR HP N ENGL J MED 285 656 971 009 FROMM H LANCET 2 621 971 010 FROMM H GASTROENTEROLOGY 64 1077 973 011 ABT AF BULL JOHNS HOPKINS HOSP 119 316 966 012 VAN DE KAMER JH J BIOL CHEM 177 347 949 013 ANDERSON CM LANCET 1 836 952 014 WEBER AM CLIN CHIM ACTA 39 524 972 015 ALI SS CAN J BIOCHEM 44 957 966 016 MCGREGOR M J CLIN INVEST 40 971 961 017 LEWIS R ARCH INTERN MED 130 545 972 018 ROSS CAC ARCH DIS CHILD 30 316 955 019 KALSER MH N ENGL J MED 279 570 968 020 JORDAN PH GASTROENTEROLOGY 36 447 959 021 MIETTINEN TA J LIPID RES 6 411 965 022 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 023 GOODCHILD MC ARCH DIS CHILD 50 769 975 CT 1 KERN F GASTROENTEROLOGY 73 631 977 2 ROY CC GASTROENTEROLOGY 73 631 977 3 WATKINS JB GASTROENTEROLOGY 73 1023 977 4 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 5 SMALLEY CA ARCH DIS CHILD 53 477 978 6 BARR RG PEDIATRICS 62 393 978 7 GILAT T GASTROENTEROLOGY 74 332 978 8 STARKEY BJ MONOGR PAEDIATR 10 12 979 9 PELED Y DIGESTION 19 267 979 10 LOESCHKE K INTERNIST 20 369 979 11 FARIVAR S DIG DIS SCI 24 33 979 12 HARRIES JT ARCH DIS CHILD 54 19 979 13 BARRY MM J AM DIET ASSOC 75 446 979 14 REGAN PT GASTROENTEROLOGY 77 285 979 15 PASANEN AVO SCAND J GASTROENTEROL 15 503 980 16 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 17 BOYLE BJ GASTROENTEROLOGY 78 950 980 18 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 19 COLOMBO C DIG DIS SCI 28 306 983 20 ZENTLERMUNRO PL GUT 25 500 984 21 DUTTA SK GASTROENTEROLOGY 91 1243 986 PN 77077 RN 00658 AN 77247480 AU Roy-C-C. Weber-A-M. TI Minimal bile acid malabsorption [letter]. SO Gastroenterology. 1977 Sep. 73(3). P 631-2. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: me. MALABSORPTION-SYNDROMES: me. MN CHILD. CYSTIC-FIBROSIS: co. HUMAN. MALABSORPTION-SYNDROMES: et. EX Roller and Kern report that bile acid malabsorption in patients with cystic fibrosis (CF) and acquired pancreatic insufficiency is minimal. These findings are at variance with other studies and deserve comment. In the absence of fecal bile acid values in controls, the authors cannot state that levels in CF are only twice normal values, especially since the papers cited on normal bile acid excretion refer to measurements obtained by a different technique. We also question the validity of the breath test as an index of bile acid malabsorption in CF. Our recent report shows that in 8 older CF (mean age 12.1 years) the magnitude of bile acid loss in the stools was comparable to that of the younger age group (mean age 3.9 years) and that there was no correlation between the age and the degree of bile acid loss. On the other hand, we must take issue with Roller and Kern when they suggest that bile acid malabsorption in CF is probably not clinically important. Our findings not only bear relevance to the intestinal manifestations of CF and to the increased incidence of cholelithiasis but perhaps also to the early deposition within the intrahepatic ducts of eosinophilic mucus concretions proposed as the cause of biliary cirrhosis in this disease. RF 001 ROLLER RJ GASTROENTEROLOGY 72 661 977 001 ROLLER RJ GASTROENTEROLOGY 72 661 977 002 WEBER AM N ENGL J MED 289 1001 973 002 GOODCHILD MC ARCH DIS CHILD 50 769 975 003 WEBER AM N ENGL J MED 289 1001 973 003 WATKINS JB GASTROENTEROLOGY 67 835 974 004 GOODCHILD MC ARCH DIS CHILD 50 769 975 004 WEBER AM GUT 17 295 976 005 WOODBURY JF J CLIN INVEST 50 2531 971 005 WEBER AM GUT 17 295 976 006 FROMM H GASTROENTEROLOGY 64 268 973 009 ROY CC GASTROENTEROLOGY 72 1123 977 PN 77078 RN 00659 AN 77116798 AU Matsehe-J-W. Go-V-L. DiMagno-E-P. TI Meconium ileus equivalent complicating cystic fibrosis in postneonatal children and young adults. Report of 12 cases. SO Gastroenterology. 1977 Apr. 72(4 Pt 1). P 732-6. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: et. MECONIUM. MN ADOLESCENCE. ADULT. BARIUM-SULFATE: du. CHILD. CHILD-PRESCHOOL. ENEMA. FEMALE. HUMAN. INFANT. INTESTINAL-OBSTRUCTION: oc, th. MALE. DIATRIZOATE-MEGLUMINE: du, tu. MINNESOTA. PAIN: et. AB Twelve patients with meconium ileus equivalent complicating cystic fibrosis in the postneonatal period were seen at the Mayo Clinic in the years 1950 through 1975. In a child or young adult with known or suspected cystic fibrosis, the triad of recurrent colicky abdominal pain, a mass in the right lower quadrant, and mechanical intestinal obstruction provides a clue to diagnosis of meconium ileus equivalent. The clinical suspicion of meconium ileus equivalent may be confirmed by meglumine diatrizoate (Gastrografin) enema, which in most uncomplicated cases also serves as treatment. RF 001 MULLINS F JAMA 192 741 965 002 RASOR R ROCKY MT MED J 38 218 941 003 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 004 FISHER OD ARCH DIS CHILD 29 262 954 005 NIXON HH GR ORMOND STR J 43 955 006 BIRSE EL BR MED J 2 286 956 007 PORCARO F AM J SURG 100 113 960 008 FANCONI A HELV PAEDIATR ACTA 15 566 960 009 CORDONNIER JK SURGERY 54 667 963 010 SNYDER WH JR PEDIATRICS 34 72 964 011 SIGLER RM MAYO CLIN PROC 40 477 965 012 GARNHAM JR GUT 5 256 964 013 HUNTON DB GASTROENTEROLOGY 50 99 966 014 IBACH EG MED J AUST 1 268 968 015 LYNCH G J IR MED ASSOC 61 209 968 016 SELIGER G AM J DIG DIS 17 934 972 017 BROWN PM N ENGL J MED 263 544 960 018 JAFFE BF ARCH SURG 92 337 966 019 HOLSCLAW DS AM J DIS CHILD 109 101 965 020 SHWACHMAN H PEDIATRICS 46 335 970 021 HOLSCLAW DS PEDIATRICS 48 51 971 022 SHWACHMAN H N ENGL J MED 286 1300 972 023 QUINLAN MF AUSTRALAS ANN MED 16 84 967 024 DONNISON AB PEDIATRICS 37 833 966 025 THOMAIDIS TS J PEDIATR 63 444 963 026 COHEN D AUST NZ J SURG 34 47 964 027 BROWN PM N ENGL J MED 263 544 960 028 NEUHAUSER EBD RADIOLOGY 46 319 946 029 HARRIS GBC POSTGRAD MED 34 251 963 030 WEINSTEIN LD GASTROENTEROLOGY 54 1282 968 031 BARTRAM CI BR J RADIOL 44 195 971 032 BOWRING AC J PEDIATR SURG 5 338 970 033 LILLIBRIDGE CB J PEDIATR 71 887 967 034 SHWACHMAN H JAMA 149 1101 952 035 LLOYD AVC JAMA 184 1001 963 PN 77079 RN 00660 AN 78004283 AU Watkins-J-B. Tercyak-A-M. Szczepanik-P. Klein-P-D. TI Bile salt kinetics in cystic fibrosis: influence of pancreatic enzyme replacement. SO Gastroenterology. 1977 Nov. 73(5). P 1023-8. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: dt. PANCREATIN: tu. MN BILE-ACIDS-AND-SALTS: bi. CHENODEOXYCHOLIC-ACID: me. CHILD-PRESCHOOL. CHOLIC-ACIDS: me. CLINICAL-TRIALS. CYSTIC-FIBROSIS: me. DIETARY-FATS: me. HUMAN. INFANT. KINETICS. SUPPORT-U-S-GOVT-P-H-S. AB Bile acid kinetics was investigated by stable isotope dilution technique in 6 children (ages 3 1/2 months to 4 1/2 years) with previously untreated cystic fibrosis. All of the patients had clinical and laboratory evidence of malabsorption, normal intestinal mucosal function, as judged by glucose absorption, intestinal histology, disaccharidase levels, and normally functioning gallbladders. The children were maintained on a constant diet throughout the study period; fat intake averaged 4.2 g per kg per day. Before administration of pancreatic enzyme replacement, fat excretion equalled 50 plus or minus 4% (mean plus or minus SE) of intake and was reduced to 20 plus or minus 1.0% of intake after therapy. Total bile acid pool size nearly doubled during enzyme replacement from 379 plus or minus 32 micromoles per kg to 620 plus or minus 36 micromoles per kg with secondary bile acids comprising 57% of the total pool before therapy and 40% after therapy. Total bile acid synthesis changed little with therapy, equalling 139 plus or minus 17 micromoles per kg per day (total 602 plus or minus 109 mg per day) without enzyme replacement and 125 plus or minus 19 micromoles per kg per day (total 470 plus or minus 86 milligrams per day) with enzyme replacement. This was confirmed by the fecal excretion of bile acids: 545 plus or minus 166 milligrams of bile acid per day were excreted without enzymes as compared to 513 plus or minus 146 milligrams per day with enzymes. After therapy, the fractional turnover rate of the bile acid pool was reduced from 0.602 plus or minus 0.05 per day to 0.233 plus or minus .03 per day for cholic acidand from 0.696 plus or minus 0.09 per day to 0.39 plus or minus 0.04 per day for chenodeoxycholic acid. These data indicate that both primary and secondary bile acids are conserved within the enterohepatic circulation during enzyme therapy, and that the mechanism for the regulation of hepatic bile acid synthesis is intact in cystic fibrosis. However, the demonstration that large amounts of bile acid continue to be excreted during therapy suggests that interruption of the enterohepatic circulation continues and that deficiencies of the intraluminal phase may persist during enzyme therapy in this disease. RF 001 WATKINS JB GASTROENTEROLOGY 67 835 974 002 LAPEY A J PEDIATR 84 328 974 003 DIMAGNO EP GASTROENTEROLOGY 70 878 976 004 WEBER AM GUT 17 295 976 005 WEBER AM N ENGL J MED 289 1001 973 006 GOODCHILD MC ARCH DIS CHILD 50 769 975 007 ROLLER RJ GASTROENTEROLOGY 72 661 977 008 DI SANTAGNESE PA PEDIATRICS 18 387 956 009 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 010 VAN DE KAMER JH J BIOL CHEM 177 347 949 011 WATKINS JB N ENGL J MED 288 431 973 012 HOFMANN AF J LIPID RES 9 707 968 013 KLEIN PD CLIN CHEM 17 735 971 014 FOLCH J J BIOL CHEM 226 497 957 015 ROOVERS J CLIN CHIM ACTA 19 449 968 016 KLEIN PD ANAL CHEM 44 490 972 017 ADMIRAND WH J CLIN INVEST 47 1043 968 018 LINDSTEDT S ACTA PHYSIOL SCAND 40 1 957 019 POMARE EW CLIN CHIM ACTA 57 239 974 020 DOWLING RH J CLIN INVEST 50 1917 971 021 SMALL DM ARCH INTERN MED 130 552 972 022 POLEY JR GASTROENTEROLOGY 71 38 976 023 MOTSON RW GASTROENTEROLOGY 71 922 976 024 GRUNDY SM GASTROENTEROLOGY 62 1200 972 025 GO VLW GASTROENTEROLOGY 58 321 970 026 MALAGELADA JR J CLIN INVEST 58 493 976 027 LARUSSO NF GASTROENTEROLOGY 69 1301 975 028 BRUNNER H MAYO CLIN PROC 49 851 974 029 GO VLW J CLIN INVEST 49 1558 970 030 ROY CC PEDIATR RES 11 449 977 031? RICOUR C REV EUR ETUD CLIN BIOL 17 172 972 032 CAREY MC AM J MED 49 590 970 033 BENDER S GUT 16 927 975 034 HOFFMAN NE AM J PHYSIOL 279 298 975 035 LOW-BEER TS GASTROENTEROLOGY 64 764 973 036 MEKHJIAN HS J CLIN INVEST 50 1569 971 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 STARKEY BJ MONOGR PAEDIATR 10 12 979 3 LOESCHKE K INTERNIST 20 369 979 4 HARRIES JT ARCH DIS CHILD 54 19 979 5 REGAN PT GASTROENTEROLOGY 77 285 979 6 COX KL J PEDIATR 94 488 979 7 ROY CC PROC SOC EXP BIOL MED 161 105 979 8 HEUBI JE PEDIATR RES 14 943 980 9 ELLIOTT WH LIPIDS 15 764 980 10 HUBBARD VS AM J CLIN NUTR 33 2281 980 11 GORIUP U HELV PAEDIATR ACTA 35 177 980 12 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 13 BOYLE BJ GASTROENTEROLOGY 78 950 980 14 BALISTRERI WF J PEDIATR 96 582 980 15 ELIAS E LANCET 2 1319 981 16 WORMSLEY KG ITAL J GASTROENTEROL 13 144 981 17 MITCHELL EA AUST PAEDIATR J 17 207 981 18 MITCHELL EA AUST PAEDIATR J 17 89 981 19 PARK RW GASTROENTEROLOGY 81 1143 981 20 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 21 MULLER DPR CLIN GASTROENTEROL 11 119 982 22 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 23 FONDACARO JD PEDIATR RES 16 494 982 24 ROY CC GASTROENTEROLOGY 83 1156 982 25 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 26 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 27 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 28 BASS S GASTROENTEROLOGY 84 1592 983 29 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 30 NORMAN EJ BIOMED MASS SPECTROMET 11 269 984 31 ZENTLERMUNRO PL GUT 25 500 984 32 ABRAMS CK J CLIN INVEST 73 374 984 33 KLEIN PD J PEDIATR GASTROENTEROL NUTR 4 9 985 34 DARLING PB PEDIATR RES 19 578 985 35 ROBB TA GUT 26 1246 985 36 ZENTLERMUNRO PL GUT 26 892 985 37 SETCHELL KDR CLIN CHIM ACTA 151 101 985 38 KNOPFLE G KLIN PAEDIATR 197 481 985 39 DEROOIJ FWM ACTA PAEDIATR SCAND SUPPL 317 1985 28 985 40 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 41 JONAS A J PEDIATR GASTROENTEROL NUTR 5 143 986 42 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 43 KOHLER JA J CLIN HOSP PHARM 11 21 986 44 STEAD RJ GUT 27 714 986 45 WEIZMAN Z GUT 27 1043 986 46 LEROY C DIG DIS SCI 31 911 986 47 DUTTA SK GASTROENTEROLOGY 91 1243 986 PN 77080 RN 00661 AN 79129993 AU Kraemer-R. Hadorn-B. Rossi-E. TI Classification at time of diagnosis and subsequent survival in children with cystic fibrosis. SO Helv-Paediatr-Acta. 1977 Jul. 32(2). P 107-14. MJ CYSTIC-FIBROSIS: mo. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, fg. GASTROINTESTINAL-DISEASES: mo. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: mo. LUNG-DISEASES: mo. MECONIUM. SWITZERLAND. AB The survival rates in 204 patients suffering from cystic fibrosis observed between January 1956 and June 1976 were recorded. The patients were divided into five groups according to the symptoms present at the time of diagnosis and survival rates were recorded separately for each group. Survival was less good in the group of patients presenting initially with pulmonary symptoms compared to the group presenting with gastrointestinal symptoms. In the group presenting with meconium ileus survival was poor initially, but later in the course of the disease it became similar to that observed in the other groups. The differences in survival between the different categories occurred during the first two years after diagnosis. The study shows that, on clinical grounds, a severe form of the disease which is characterized by early manifestation of pulmonary symptoms and poor survival can be distinguished from a more protracted form with a better survival. Because of the great inter-individual variability large numbers of patients will have to be evaluated in order to achieve statistically significant results in studies which attempt to compare different therapeutic approaches. If such numbers cannot be reached, it may be necessary to compare only patients who belong to the same symptomatic category. RF 001 CUTLER SJ J CHRON DIS 8 699 958 002 DOERSHUK CF J PEDIATR 65 677 964 003 GEORGE L ARCH DIS CHILD 46 139 971 004 HADORN B HANDBUCH DER INNEREN MEDIZIN 889 976 005 HOGGER GP HELV PAEDIATR ACTA 30 151 975 006 HUANG NN AM J DIS CHILD 120 289 970 007 KERREBIJN KF PROC INT CF CONG 7TH 976 008 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 271 977 009 LAPEY A J PEDIATR 84 328 974 010 ROBINSON MJ ARCH DIS CHILD 50 962 975 011 SHWACHMAN H AM J DIS CHILD 96 6 958 012 SHWACHMAN H PEDIATRICS 46 335 970 013 WARWICK WJ J CHRON DIS 28 609 975 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 KRAEMER R ACTA PAEDIATR SCAND 67 33 978 3 KRAEMER R MONOGR PAEDIATR 10 61 979 4 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 5 CROSSLEY JR CLIN CHIM ACTA 113 111 981 6 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 7 CHOW CW EUR J PEDIATR 139 240 982 8 ANON PEDIATRICS 72 741 983 9 WILCKEN B J PEDIATR 102 383 983 10 GROBE H MONATSSCHR KINDERHEILKD 131 806 983 11 CASSIO A ACTA PAEDIATR SCAND 73 554 984 12 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 13 KATZ JN J PEDIATR 108 352 986 14 DANKERTROELSE JE ACTA PAEDIATR SCAND 76 209 987 PN 77081 RN 00662 AN 79129994 AU Malmendier-C-L. Van-Den-Bergen-C-J. Baran-D. TI Influence of lyophilized total pancreas on plasma lipids and on fat absorption in cystic fibrosis. SO Helv-Paediatr-Acta. 1977 Jul. 32(2). P 115-28. MJ CYSTIC-FIBROSIS: th. LIPIDS: me. PANCREATIC-EXTRACTS: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CHOLESTEROL-ESTERS: bl. CYSTIC-FIBROSIS: bl, me. FATTY-ACIDS-NONESTERIFIED: bl. HUMAN. INFANT. INTESTINAL-ABSORPTION. LIPIDS: bl. PHOSPHOLIPIDS: bl. TRIGLYCERIDES: bl. AB 31 children with cystic fibrosis (CF) subdivided into three groups of age were studied before and after treatment with lyophilized pancreas and compared to 27 controls. CF was characterized by lower phospholipid, cholesterol and polyunsaturated fatty acid plasma concentrations and reduced coefficient of fat absorption. Treatment tended towards normalization of these values. With age polyunsaturated fatty acids increased in normals but not in CF. The significant correlations existing between fat absorption coefficient and polyunsaturated fatty acid percentage in neutral lipids of CF children suggests the use of the latter percentage as index of the stage of malabsorption. RF 001 BARAN D ACTA PAEDIATR BELG 29 117 976 002 BLOMSTRAND R ACTA CHEM SCAND 10 1019 964 003 BORGSTROM B ACTA PHYSIOL SCAND 25 101 952 004 BOY J ANN BIOL CLIN 18 669 960 005 BRAGDON JH J BIOL CHEM 190 513 951 006 CAREN R J ATHEROSCLER RES 4 444 964 007 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 008 CARLSON LA CLIN CHIM ACTA 4 197 959 009 CASH R J PEDIATR 74 717 969 010 CHIN HP CLIN CHIM ACTA 20 305 968 011 DEMETRIOU JA IN: HENRY RJ 914 974 012 FOLCH J J BIOL CHEM 226 497 957 013 KATES M IN: WORK TS 393 972 014 KUO PT J CLIN INVEST 44 1924 965 015 LUBIN AH CF CLUB ABST 14 4 973 016 MALMENDIER CL BIOMEDICINE 20 398 974 017 MCCOMBS ML TEX REP BIOL MED 31 615 973 018 NIESSEN KH KLIN PAEDIATR 185 271 973 019 NORMAN AP BR MED J 2 621 968 020 NORUM KR SCAND J CLIN LAB INVEST 20 231 967 021 REEMTSMA K PEDIATRICS 22 525 958 022 ROSENBERG A J BIOL CHEM 232 1031 958 023 ROSENLUND ML NATURE 251 719 974 024 SHIMOYAMA T GUT 14 716 973 025 SNEDECOR GW STATISTICAL METHODS APPLIED T 966 026 STEWART CP BIOCHEM J 29 1683 935 027 WEIJERS HA ACTA PAEDIATR SCAND 42 24 953 CT 1 ROGIERS V PEDIATR RES 14 1088 980 2 HAMILTON RM LIPIDS 16 374 981 3 ROGIERS V EUR J PEDIATR 141 39 983 PN 77082 RN 00663 AN 77186767 AU Kendig-E-L-Jr. TI Chronic lung disease in children. SO Hosp-Pract. 1977 May. 12(5). P 87-93, 95-8. MJ LUNG-DISEASES. MN BRONCHIAL-DISEASES: di. CHILD. CYSTIC-FIBROSIS: di, th. DIAGNOSIS-DIFFERENTIAL. HISTOPLASMOSIS: di, dt. HUMAN. LUNG-DISEASES-FUNGAL: di, dt. MYCOBACTERIUM-INFECTIONS-ATYPICAL: di, dt. PULMONARY-FIBROSIS: di, dt. SARCOIDOSIS: di, dt. TUBERCULIN-TEST. TUBERCULOSIS-IN-CHILDHOOD. TUBERCULOSIS-PULMONARY: di, dt. AB Since tuberculosis is still a significant threat, the family physician needs to be familiar with methods of its diagnosis and treatment. Among other entities discussed are atypical mycobacteriosis sarcoidosis, and cystic fibrosis. RF 001 STEIGMAN AJ PEDIATRICS 56 160 975 002 KENDIG EL JR N ENGL J MED 281 250 969 003 LINCOLN EM AM REV RESPIR DIS 105 683 972 004 KENDIG EL JR N ENGL J MED 268 1001 963 005 JAMES DG ANN NY ACAD SCI 278 321 976 006 NIITU Y ANN NY ACAD SCI 278 532 976 007 KENDIG EL JR PEDIATRICS 54 289 974 008 CHRISTIE A IN: KENDIG EL JR 1 608 972 009 SHWACHMAN H PEDIATRICS 46 335 970 010 DOERSHUK CF PEDIATRICS 36 675 965 011 LIEBOW AA IN: SIMON M 968 012 STOCKS J PEDIATRICS 57 352 976 013 NORTHWAY WH JR N ENGL J MED 276 357 967 PN 77083 RN 00664 AN 78004768 AU Tchen-P. Bois-E. Feingold-J. Feingold-N. Kaplan-J. TI Inbreeding in recessive diseases. SO Hum-Genet. 1977 Sep 22. 38(2). P 163-7. MJ CONSANGUINITY. GENES-RECESSIVE. MN ALBINISM: fg. COLOR-BLINDNESS: fg. CYSTIC-FIBROSIS: fg. CYSTINOSIS: fg. FRANCE. HUMAN. KIDNEY-DISEASES: fg. MUSCULAR-ATROPHY: fg. AB The consanguinity of parents (born in France) of individuals who have a recessive disease has been studied. The frequency of first cousin marriages is less than 0.2% in the general French population. Among the parents of affected individuals the following frequencies of first cousin matings were observed: cystic fibrosis: 1.4% cystinosis: 7.1% nephronophtisis: 5.6% spinal muscular atrophy: 4.5% albinism: 5.0% achromatopsia: 12.5% (Albinism and spinal muscular atrophy are heterogeneous conditions). The increase in the frequency of first cousin marriage relative to that of the general population is much greater, as expected, in cystinosis, which is a rare disease, than in cystic fibrosis, which is the most frequent recessive disorder in France. Inbreeding in cystinosis and cystic fibrosis was also studied by computing the distance between parental birth places. This distance is smaller in cystinosis than in cystic fibrosis. RF 001 DE ARAUJO AM SOCIAL BIOLOGY 21 249 974 002 AZEVEDO E AM J HUM GENET 21 1 969 003 BARRAI I ANN HUM GENET 25 347 962 004 BICKEL H ACTA PAEDIATR SCAND SUPPL 90 22 952 005 BOIS E J MED GENET 13 434 976 006 BROBERGER O ACTA PAEDIATR SCAND 49 470 960 007 CAVALLI-SFORZA LL GENETICS 54 37 966 008 DANKS DM ANN HUM GENET 28 323 965 009 EMERY AEH PROC INT CONG MUSCLE D 3RD 557 975 010 FEINGOLD J ANN GENET (PARIS) 17 257 974 011 GIRARD A CHOIX DU CONJOINT 974 012 GORDILLO G BOL MED HOSP INFANTIL MEX 24 533 967 013 LIRENMAN DS BIRTH DEF ORIG ART SER 10 32 974 014 MANGOS JA PEDIATRICS 34 337 964 015 MORTON NE AM J HUM GENET 25 347 973 016 NEEL JV AM J HUM GENET 1 156 949 017 PEARN JH BRAIN 96 463 973 018 SUTTER J POPULATION 13 683 958 019 SUTTER J POPULATION 17 683 962 020 VON SYDOW G ACTA PAEDIATR SCAND 51 561 962 021 WITKOP CJ JR ADV HUM GENET 2 971 CT 1 BITTLES AH J SCI INDUSTR RES 39 768 980 2 ROMEO G RIV ITAL PEDIATR 7 201 981 3 BOIS E ARCH FR PEDIATR 42 175 985 PN 77084 RN 00665 AN 78004791 AU Burdick-A-B. TI Frequency of the gene for cystic fibrosis with a view of replacement and recognition effects and reproduction by homozygotes. SO Hum-Hered. 1977. 27(5). P 366-71. MJ CYSTIC-FIBROSIS: fg. GENE-FREQUENCY. MN FEMALE. HOMOZYGOTE. HUMAN. MALE. MUTATION. REPRODUCTION. AB The apparent frequency of the recessive autosomal allele for cystic fibrosis (cf) is too high to be satisfactorily explained by mutation equilibrium. However, higher reproductive fitness by heterozygotes (expressed by t greater than 0) or higher gametic viability of the cf allele (expressed by a greater than 0.5) can provide reasonable explanations of the present frequency. Furthermore, birth replacement in families with CF children (RPE) can account for the estimated historical values of t. Today, however, t is probably at or approaching zero as a result of discontinuation of RPE. In the future we can look for an imperceptibly slow decrease in the incidence of CF. Reproduction by CF females will not cause an increase in incidence; it will only slightly slow the rate of decrease. RF 001 HALDANE JBS PROC CAMB PHIL SOC 23 838 927 002 WARWICK WJ J CHRON DIS 28 609 975 003 GRAND RJ JAMA 195 993 966 004 LI CC FIRST COURSE IN POPULATION GE 976 005 KNUDSON AG JR AM J HUM GENET 19 388 967 006 WRIGHT SW AM J HUM GENET 20 157 968 007 CONNEALLY PM TEX REP BIOL MED 31 639 973 008 GLASS B AM J HUM GENET 2 269 950 009 RECORD RG BR J PREV SOC MED 29 267 975 010 LARSEN JW OBSTET GYNECOL 39 880 972 011 LUNDGREN DW CLIN CHIM ACTA 62 357 975 012 SAMUELS CE LANCET 2 607 975 013 SHWACHMAN H LANCET 2 372 975 014 WILSON GB PEDIATR RES 9 635 975 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 2 AFZELIUS BA AM J HUM GENET 33 852 981 PN 77085 RN 00666 AN 77139738 AU Wyatt-P-R. Cox-D-M. TI Utilization of electron microscopy in the prenatal diagnosis of genetic disease. SO Hum-Hered. 1977. 27(1). P 22-37. MJ HEREDITARY-DISEASES: di. PRENATAL-DIAGNOSIS: mt. MN LIPOIDOSIS: di. AMNIOTIC-FLUID: cy. ANGIOKERATOMA-CORPORIS-DIFFUSUM: di. CELL-LINE. CYSTIC-FIBROSIS: di. CYSTINOSIS: di. EVALUATION-STUDIES. FEMALE. FIBROBLASTS: ul. GLYCOGENOSIS-2: di. HUMAN. INFANT-NEWBORN. LESCH-NYHAN-SYNDROME: di. LEUKODYSTROPHY-METACHROMATIC: di. MAPLE-SYRUP-URINE-DISEASE: di. MICROSCOPY-ELECTRON. MUCOLIPIDOSIS: di. MUSCULAR-DYSTROPHY: di. PREGNANCY. SKIN: ul. AB The use of electron microscopy as a further method of diagnosis of disease in cultured skin fibroblasts and cultured amniotic fluid fibroblasts is presented. It was demonstrated that Tay-Sachs disease, Fabry's disease, and metachromatic leukodystrophy had distinctive abnormalities in both cultured skin fibroblasts and cultured amniotic fluid fibroblasts. It was shown that control of culturing conditions made it possible to distinguish normal cell lines from certain cell lines carrying known genetic diseases. RF 001 AULA P J PEDIATR 87 221 975 002 BARTMAN J J PEDIATR 76 430 970 003 BARTMAN J OBSTET GYNECOL 38 838 971 004 BUTTERWORTH J AM J OBSTET GYNECOL 119 821 974 005 COMINGS DE EXP CELL RES 61 295 970 006 CONRAD GW PEDIATR RES 6 563 972 007 EPSTEIN CJ ADV INTERN MED 20 325 975 008 FELIX JS PEDIATR RES 8 870 974 009 FUCHS F IN: FAIRWEATHER DVI 262 973 010 GERBIE AB AM J OBSTET GYNECOL 114 314 972 011 GORDON GB J CELL BIOL SUPPL 25 41 965 012 HANAI J AM J DIS CHILD 122 34 971 013 HOEHN H PEDIATR RES 8 746 974 014 HOYES AD J OBSTET GYNAECOL BR COMMONW 75 164 968 015 HSU LYF LIFE SCI 14 2311 974 016 HUG G BIRTH DEF ORIG ART SER 9 160 973 017 HUISJES HJ IN: FAIRWEATHER DVI 95 973 018 KABACK MM PEDIATR RES 5 366 971 019 KAMENSKY E AM J PATHOL 73 59 973 020 KOHN G PEDIATR RES 9 314 975 021 LIE SO PEDIATR RES 7 13 973 022 LIPETZ J J ULTRASTRUCT RES 39 43 972 023 LUCKY AW EXP CELL RES 92 383 975 024 LYON G J NEUROL SCI 19 235 973 025 MACIEIRA-COELHO A PROC SOC EXP BIOL MED 138 712 971 026 MCLEAN J J MED GENET 11 133 974 027 MCLEAN J J MED GENET 11 257 974 028 MELLMAN WJ ADV HUM GENET 2 259 971 029 MELLMAN WJ IN: ROTHBLAT GH 1 327 972 030 NADLER HL N ENGL J MED 282 596 970 031 NADLER HL IN: FAIRWEATHER DVI 223 973 032 NIELAND ML AM J OBSTET GYNECOL 108 1030 970 033 PRIEST JH HUMAN CELL CULTURE IN DIAGNOS 972 034 ROBBINS E J EXP MED 131 1211 970 035 ROOK A NATURE 230 53 971 036 RYAN CA EXP CELL RES 71 388 972 037 RUTSAERT J LAB INVEST 29 527 973 038 STEIN H ISR J MED SCI 10 463 974 039 TENCONI R AM J DIS CHILD 124 296 972 040 TONDEUR M ACTA PAEDIATR BELG 24 355 970 041 UHLENDORF BW IN: HARRIS M 149 970 042 WHALSTROM J HUMANGENETIK 22 335 974 043 WOOD S EXP CELL RES 96 317 975 CT 1 WYATT PR PEDIATR RES 12 310 978 2 MARTIN JJ J NEUROL NEUROSURG PSYCHIAT 41 232 978 3 BURTON BK SEM PERINATOL 4 179 980 4 BURTON BK CLIN OBSTET GYNAECOL 7 27 980 5 STEPHENSON SR AM J OBSTET GYNECOL 141 319 981 6 WANDALL A ULTRASTRUCTURAL PATHOL 3 51 982 7 PATRICK AD BR MED BULL 39 378 983 8 ARNON J PRENAT DIAGN 6 351 986 9 JAFFRAY JY PATHOL BIOL (PARIS) 34 91 986 10 HASHOLT L HUM GENET 72 72 986 PN 77086 RN 00667 AN 77139699 AU Lack-E-E. TI Carotid body hypertrophy in patients with cystic fibrosis and cyanotic congenital heart disease. SO Hum-Pathol. 1977 Jan. 8(1). P 39-51. MJ CAROTID-BODY: pa. CYSTIC-FIBROSIS: pa. HEART-DEFECTS-CONGENITAL: pa. MN ADOLESCENCE. ADULT. ANOXEMIA: co. AUTOPSY. CAROTID-BODY: ah. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FEMALE. HEART-DEFECTS-CONGENITAL: co. HUMAN. HYPERTROPHY. INFANT. INFANT-NEWBORN. MALE. ORGAN-WEIGHT. AB The carotid bodies from 71 patients ranging in age from 28 weeks' gestation to 30 years were obtained at autopsy. Patients were divided into two groups based on the presence or absence of chronic hypoxemia. There was a high correlation between the weight of individual carotid bodies in each case. Among the 12 patients with chronic hypoxemia, eight patients had carotid bodies heavier than predicted by statistical analysis. Of these eight patients, six had cystic fibrosis and two had cyanotic heart disease. Morphometric and cell population analyses of the carotid bodies from these eight patients and from those of the control population indicated that enlargement of the carotid bodies during normal or abnormal growth results from proportionate increases in lobule parenchyma and stroma. There was also an increase in the width and length of the lobules without an increase in the diameter of the cell cords or a change in the size or proportion of the chief cells. Growth and development of the carotid bodies were studied in a control group of 59 patients without chronic hypoxemia. There were no sex related differences in carotid body weights. The combined weight of the carotid bodies correlated most strongly with body weight, although there was some correlation with age and body length. A regression equation reflecting the data relating to body weight (BW) is: Combined weight of carotid bodies (in mg.) = 0.29 BW (in kg.) +3.0. Leukemic infiltrates were present in two patients with acute lymphocytic leukemia, and diffuse lymphocytic infiltration with nodule formation was present in one patient with mental retardation. Metaplastic cartilage was present in a carotid body of one patient. RF 001 HEATH D THORAX 25 129 970 002 EDWARDS C J PATHOL 104 1 971 003 ARIAS-STELLA J AM J PATHOL 55 150A 969 004 SALDANA MJ HUM PATHOL 4 251 973 005 HEYMANS C PHARMACOL REV 7 119 955 006 JOELS N BR MED BULL 19 21 963 007 SOLCIA E HISTOCHEMIE 20 116 969 008 GRIMLEY PM CANCER 20 1473 967 009 PRYSE-DAVIES J CANCER 17 185 964 010 WINSON M ARCH PATHOL 96 58 973 011 LUGLIANI R N ENGL J MED 285 1105 971 012 DAVIDSON JT N ENGL J MED 290 819 974 013 COOK CD J PEDIATR 59 710 961 014 LATTES R CANCER 3 667 950 015 OBERMAN HA CANCER 21 838 968 016 LACK EE ARCH PATHOL 99 215 975 017 VALDES-DAPENA MA N ENGL J MED 289 1195 973 018 SALK L N ENGL J MED 291 219 974 019 ARIAS-STELLA J HUM PATHOL 7 361 976 020 NAEYE RL SCIENCE 191 567 976 CT 1 LACK EE AM J PATHOL 91 497 978 2 GALLIVAN MVE AM J SURG PATHOL 3 85 979 3 LACK EE HUM PATHOL 10 191 979 4 LACK EE CANCER 43 269 979 5 VERNA A INT REV CYTOL 60 271 979 6 VANZWIETEN MJ J PATHOL 128 99 979 7 DUNCAN AW RADIOLOGY 132 99 979 8 LACK EE AM J SURG PATHOL 4 109 980 9 MCDONALD DM FED PROC 39 2627 980 10 ROBERTSON DI CANCER 46 2623 980 11 GALLIVAN MVE ARCH PATHOL LAB MED 104 46 980 12 MERINO MJ CANCER 47 1403 981 13 WETMORE RF CANCER 48 2717 981 14 DELACROIX R J CHIR (PARIS) 118 29 981 15 HABECK JO ANAT ANZ 150 374 981 16 BOCKELMAN HW CANCER 50 2513 982 17 BORGES LF J NEUROSURG 59 867 983 18 GRONBLAD M MED BIOL 61 229 983 19 PERRIN DG LANCET 2 535 984 20 PERRIN DG PEDIATRICS 73 646 984 21 HABECK JO ANAT ANZ 157 351 984 22 DELAMONTE SM AM J PEDIATR HEMATOL ONCOL 7 109 985 23 HABECK JO EXP PATHOL 27 79 985 24 LACK EE AM J PATHOL 119 301 985 25 HABECK JO ANAT ANZ 162 17 986 PN 77087 RN 00668 AN 78065932 AU Sorensen-R-U. Stern-R-C. Polmar-S-H. TI Cellular immunity to bacteria: impairment of in vitro lymphocyte responses to Pseudomonas aeruginosa in cystic fibrosis patients. SO Infect-Immun. 1977 Dec. 18(3). P 735-40. MJ ANTIGENS-BACTERIAL. CYSTIC-FIBROSIS: im. LYMPHOCYTE-TRANSFORMATION. PSEUDOMONAS-AERUGINOSA: im. MN ANTIBODIES-BACTERIAL. CONCANAVALIN-A. HAEMOPHILUS-INFLUENZAE: im. HUMAN. LECTINS. STAPHYLOCOCCUS-AUREUS: im. STREPTOCOCCUS-PYOGENES: im. SUPPORT-U-S-GOVT-P-H-S. AB Lymphocyte responses to the mitogens phytohemagglutinin and concanavalin A and to Streptococcus pyogenes, Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa were evaluated in patients with cystic fibrosis and in normal individuals. Lymphocyte proliferation in vitro was stimulated by gentamicin-killed whole bacteria, and the proliferative response was measured by [3H]thymidine incorporation. The in vitro lymphocyte responses to antibiotic-killed bacterial reached maximum thymidine incorporation after 5 days in culture and followed a unimodal dose-response curve for each of the bacteria studied. A significant specific incapacity to respond to P. aeruginosa was detected in cystic fibrosis patients with advanced clinical disease. RF 001 BEKIERKUNST A INFECT IMMUN 14 28 976 002 BURNS MW LANCET 1 270 968 003 DOERSHUK CF J PEDIATR 65 677 964 004 EIJSVOOGEL VP SEMIN HEMATOL 11 305 974 005 FOWLES RE J EXP MED 138 952 973 006 GODAL T CLIN EXP IMMUNOL 8 625 971 007 HANN S INFECT IMMUN 14 114 976 008 HOIBY N SCAND J RESPIR DIS 56 38 975 009 HOMMA JY JAPAN J EXP MED 41 89 971 010 KRAHENBUHL JL INFECT IMMUN 4 337 971 011 MACKANESS GB J EXP MED 129 973 969 012 MOONEY JJ J IMMUNOL 105 1138 970 013 ORDAL J J IMMUNOL 116 1182 976 014 SHWACHMAN H AM J DIS CHILD 96 6 958 015 SCHWARTZ RH AM J DIS CHILD 111 408 966 016 SIMON GB CELL IMMUNOL 4 163 972 017 SISKIND GW FED PROC 33 1886 974 018 SOUTH MA J PEDIATR 71 645 967 019 THORSBY E HISTOCOMPATIBILITY TESTING 655 970 020 WOOD RE AM REV RESPIR DIS 111 733 975 021 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 CHO YJ JAP J EXP MED 48 491 978 2 SORENSEN RU J PEDIATR 93 201 978 3 TOURAINE JL REV FR MAL RESP 7 41 979 4 THOMASSEN MJ PEDIATR RES 13 1085 979 5 WILSON GB J CLIN INVEST 66 1010 980 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 7 RUBIN HR CELL IMMUNOL 57 307 981 8 HEYNE K EUR J PEDIATR 137 116 981 9 SEALE TW ANN CLIN LAB SCI 12 415 982 10 BODEY GP REV INFECT DIS 5 279 983 11 PETIT JC BIOMED PHARMACOTHER 37 422 983 12 RUBIN HR INFECT IMMUN 39 630 983 13 PORWOLL JM INFECT IMMUN 40 670 983 14 PARMELY MJ J EXP MED 160 1338 984 15 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 16 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 17 HEGGIE AD PROC SOC EXP BIOL MED 181 586 986 PN 77088 RN 00669 AN 79108764 AU Sahu-S. Lynn-W-S. TI Enzymes of phospholipid metabolism in airway secretions of patients with asthma, cystic fibrosis, and alveolar proteinosis. SO Inflammation. 1977 Jun. 2(2). P 93-104. MJ ASTHMA: en. CYSTIC-FIBROSIS: en. PHOSPHOLIPIDS: me. PULMONARY-ALVEOLAR-PROTEINOSIS: en. RESPIRATORY-SYSTEM: en. MN ADOLESCENCE. ADULT. ANIMAL. CHILD. CHILD-PRESCHOOL. HUMAN. IN-VITRO. INFANT. PHOSPHOLIPASES: me. LYSOLECITHIN-ACYLTRANSFERASE: me. LYSOPHOSPHATIDYLCHOLINES. PHOSPHOLIPASES: ip, me. RABBITS. RESPIRATORY-SYSTEM: se. SUPPORT-U-S-GOVT-NON-P-H-S. SUPPORT-U-S-GOVT-P-H-S. AB Phospholipase A2, lysolecithinase, and lysolecithin-lysolecithin acyltransferase are present in the airway secretions of patients with asthma, cystic fibrosis (CF), and alveolar proteinosis. Assays of these enzymes under the same conditions in extracts of human polymorphonuclear leukocytes, alveolar macrophages, and pig tracheal mucosa indicated that these extracellular airway enzymes were probably not derived from these cell types. Although the above three enzymes were present in secretions of all patients with these three diseases, large amounts of palmitoyl lysolecithin, free fatty acids, and dipalmitoyl lecithin were found only in patients having alveolar proteinosis and asthma. The amount of free fatty acids in these secretions was sufficient to inhibit both the lysolecithinase and the lysolecithin-lysolecithin acyltransferase, but not the phospholipase A2. These findings suggest that the large amount of dipalmitoyl lecithin, free fatty acids, and lysolecithin found in these secretions results from extracellular remodelling by these enzymes. Because the phospholipase A2 was present in large amounts, it was possible after delipidation to purify it to homogeneity. It is a stable enzyme with an apparent molecular weight of 75,000 as estimated by SDS-mercaptoethanol gel electrophoresis. RF 001 SAHU S AM REV RESPIR DIS 114 177 976 002 SAHU S AM REV RESPIR DIS 115 233 977 003 LEWIS RW LIPIDS 6 859 971 004 ROBERTSON AF BIOCHEMISTRY 1 804 962 005 GALLAI-HATCHARD JJ BIOCHIM BIOPHYS ACTA 98 128 965 006 GARCIA A BIOCHEM BIOPHYS RES COMMUN 64 128 975 007 OLDENBERG V BIOCHIM BIOPHYS ACTA 441 433 976 008 KYLSTRA JA AM REV RESPIR DIS 103 651 971 009 TURNER S J EXP MED 141 1437 975 010 MYRVIK QN J IMMUNOL 86 128 961 011 MAGEE WL METHODS ENZYMOL 14 170 969 012 NIEUWENHUIZEN W METHODS ENZYMOL 32 147 974 013 BARTLETT GR J BIOL CHEM 234 466 959 014 LONG C BIOCHEM J 65 382 957 015 MOORE JH BIOCHIM BIOPHYS ACTA 70 348 963 016 HANAHAN DJ J BIOL CHEM 235 1917 960 017 TAGUCHI R BIOCHIM BIOPHYS ACTA 409 75 975 018 BHATTACHARYYA SN BIOCHIM BIOPHYS ACTA 427 91 976 019 ERBLAND JF BIOCHIM BIOPHYS ACTA 106 128 965 020 ERBLAND JF BIOCHIM BIOPHYS ACTA 106 139 965 021 ELSBACH P BIOCHIM BIOPHYS ACTA 125 510 966 022 OTTOLENGHI A LIPIDS 5 531 970 023 VAN DEENEN LLM IN: HOLMAN RT 8 1 965 024 AKINO T BIOCHIM BIOPHYS ACTA 248 274 971 025 VEREKEN JM BIOCHIM BIOPHYS ACTA 260 70 972 026 GATT S ANNU REV BIOCHEM 42 61 973 027 DE HAAS GH BIOCHIM BIOPHYS ACTA 159 103 968 028 SCOW RO BIOCHIM BIOPHYS ACTA 431 538 976 CT 1 SMITH FB NY STATE J MED 80 1372 980 2 RAO RH EXP LUNG RES 2 9 981 3 GALABERT C CLIN RESP PHYSIOL 17 197 981 4 WIGHTMAN PD BIOCHEM J 200 441 981 5 LANNI C BIOCHIM BIOPHYS ACTA 658 54 981 PN 77089 RN 00670 AN 77164475 AU Zegers-J-M. Stoop-J-W. TI Deficiency of kappa- or lambda-type immunoglobulins. SO Int-J-Dermatol. 1977 Apr. 16(3). P 191-3. MJ DYSGAMMAGLOBULINEMIA: di. IMMUNOGLOBULINS-KAPPA-CHAIN. IMMUNOGLOBULINS-LAMBDA-CHAIN. IMMUNOGLOBULINS-LIGHT-CHAIN. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: di. MN ADULT. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DIABETES-MELLITUS: co. DYSGAMMAGLOBULINEMIA: co. FEMALE. HUMAN. IGA. INTRINSIC-FACTOR: df. MALABSORPTION-SYNDROMES: co. MALE. EX It is widely recognized that immunoglobulins, as the carriers of antibody activity, play an important role in the defense of the individual against infections. The known cases of kappa or lambda light chain type immunoglobulin deficiencies are heterogenous with regard to clinical and immunological findings. There are also a number of similarities which may suggest common underlying factors. The accurate investigation of additional patients, if available, should provide more insight into the basis of kappa or lambda type immunoglobulin deficiencies. RF 001 BERNIER GM BLOOD 40 795 972 002 YOUNT WJ J CLIN INVEST 49 1957 970 003 SELIGMANN M REV FR ETUD CLIN BIOL 12 604 967 004 BARANDUN S BLOOD 47 79 976 005 ZEGERS BJM N ENGL J MED 294 1026 976 PN 77090 RN 00671 AN 77248610 AU Ten-Kate-L-P. TI Cystic fibrosis in the Netherlands. SO Int-J-Epidemiol. 1977 Mar. 6(1). P 23-34. MJ CYSTIC-FIBROSIS: oc. MN ADOLESCENCE. AGE-FACTORS. CAUCASOID-RACE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mo. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: oc. NETHERLANDS. RETROSPECTIVE-STUDIES. SAMPLING-STUDIES. SEASONS. SEX-FACTORS. AB In the Netherlands a retrospective study of the prevalence of cystic fibrosis (CF) at birth has been performed by means of an inquiry set up among 815 medical specialists (paediatricians, lung specialists and pathologists) and by analysis of hospital admission data, death certificates and data of the national CF foundation. The study was confined to the years of birth 1961-1965. On a total of 1,239,566 live births 342 infants and children were found to have CF (1/3600). A list of all studies on the frequency of CF in Caucasian populations is presented as an appendix. A seasonal trend in the expression of the CF gene at birth (either with or without meconium ileus) was noted. RF 001 KULCZYCKI LL AM J DIS CHILD 127 64 974 002 WRIGHT SW AM J HUM GENET 20 157 968 003 ANON HUMANGENETIK 30 273 975 004 ANON J PEDIATR 88 711 976 005 STEPHAN U PEDIATRICS 55 35 975 006 SHWACHMAN H N ENGL J MED 255 999 956 007 GIBSON LE PEDIATRICS 23 545 959 008 EDWARDS JH ANN HUM GENET 25 83 961 009 CUTLER SJ J CHRON DIS 8 699 958 010 WARWICK WJ J ASTHMA RES 5 277 968 011 HOLSCLAW DS AM J DIS CHILD 109 101 965 012 DONNISON AB PEDIATRICS 37 833 966 013 GRACEY M CF CLUB ABST 969 014 GEORGE L ARCH DIS CHILD 46 139 971 015 MCPARTLIN JF ARCH DIS CHILD 47 207 972 016 KESSLER WR PEDIATRICS 8 648 951 017 DI SANTAGNESE PA PEDIATRICS 12 549 953 018 STEINBERG AG AM J HUM GENET 12 416 960 019 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 020 DANKS DM ANN HUM GENET 28 323 965 021 TEN KATE LP THESIS 975 022 SHWACHMAN H PEDIATRICS 36 689 965 023 PUGH RJ ARCH DIS CHILD 42 544 967 024 BRUNECKY Z J MED GENET 9 33 972 025 OREILLY D INT J EPIDEMIOL 3 247 974 026 ROBERTS GBS ANN HUM GENET 24 127 960 027 HALL BD J MED GENET 5 262 968 028 CARTER CO IN: BODIAN M 49 952 029 FEINGOLD J ANN GENET (PARIS) 17 257 974 030 BERNHEIM M PEDIATRIE 16 17 961 031 GILLY R ARCH FR PEDIATR 28 49 971 032 SELANDER P ACTA PAEDIATR SCAND 51 65 962 033 KOLLBERG H PROC EWGCF 1ST ANNU MTG 970 034 NIELSEN EL ACTA PAEDIATR SCAND 61 377 972 035 KOCH G IN: VON WINDORFER A 30 968 036 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 037 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 038 NEVANLINNA HR HEREDITAS 71 195 972 039 ZYCHOWICZ C PEDIATR POLSKA 40 289 965 040 HOUSTEK J CESK PEDIATR 17 445 962 041 SIMANKOVA N ANN GENET (PARIS) 7 84 964 042 HOUSTEK J REV MED LIEGE 22 421 967 043 BRUNECKY Z PAEDIATR GRENZGEB 8 99 969 044 REIDERMAN MI GENETIKA (SOV GENETICS) 7 148 971 045 BOCHKOVA DN PEDIATRIIA 12 24 974 046 GURSON CT HELV PAEDIATR ACTA 28 165 973 047 LEVIN S IN: GOLDSCHMIDT E 294 963 048 SUPER M S AFR MED J 49 818 975 049 BECROFT DMO NZ MED J 68 113 968 050 TRIMBLE BK ANN HUM GENET 38 199 974 051 GOODMAN HO AM J HUM GENET 4 59 952 052 MERRITT AD J LAB CLIN MED 60 998 962 053 MERRITT AD JAMA 184 152 963 054 HANNA BL IN: US PUB HEAL SERV PUB1163 7 963 055 CONNEALLY PM TEX REP BIOL MED 31 639 973 056 STEINBERG AG IN: GOLDSCHMIDT E 133 963 057 LOWE CU AM J DIS CHILD 78 349 949 058 KULCZYCKI LL AM J DIS CHILD 100 174 960 059 HONEYMAN MS AM J HUM GENET 17 461 965 060 ANDERSEN DH AM J DIS CHILD 72 62 946 061 SULTZ HA AM J PUBLIC HEALTH 56 1461 966 062 GLUCKSON MM CF CLUB ABST 974 063 CLARKE CA IN: ROBERTS DF 969 064 ANON UNO DEMOGRAPHIC YEARBOOK 13 961 065 CARTER CO MOD PROBL PEDIATR 10 372 967 066 ANDERSON CM MOD PROBL PEDIATR 10 381 967 067 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 069 DI SANTAGNESE PA N ENGL J MED 277 1287 967 CT 1 BOIS E CLIN GENET 14 73 978 2 WARWICK WJ HELV PAEDIATR ACTA 33 117 978 3 BRACKENRIDGE CJ ANN HUM GENET 42 197 978 4 NEVIN GB J MED GENET 16 122 979 5 DEMENAIS F REV EPIDEMIOL SANTE PUBLIQUE 27 5 979 6 BRACKENRIDGE CJ AM J MED GENET 5 295 980 7 BRACKENRIDGE CJ AM J MED GENET 5 303 980 8 ROMEO G RIV ITAL PEDIATR 7 201 981 9 DAVID TJ J MED GENET 18 299 981 10 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 11 PASSARGE E EUR J PEDIATR 143 54 984 12 STURGESS JM AM J MED GENET 22 383 985 13 ROMEO G AM J HUM GENET 37 338 985 14 ALLAN JL ACTA PAEDIATR SCAND 74 286 985 15 DANKERTROELSE JE LANCET 1 802 986 16 KOLLBERG H J TROP PEDIATR 32 293 986 17 DANKERTROELSE JE ACTA PAEDIATR SCAND 76 209 987 PN 77091 RN 00672 AN 77094418 AU Twarog-F-J. Weinstein-S-F. Khaw-K-T. Strieder-D-J. Colten-H-R. TI Hypersensitivity to pancreatic extracts in parents of patients with cystic fibrosis. SO J-Allergy-Clin-Immunol. 1977 Jan. 59(13). P 35-40. MJ CYSTIC-FIBROSIS: im. HYPERSENSITIVITY-IMMEDIATE: ci. PANCREATIC-EXTRACTS: pd. MN ADULT. CHILD. CHILD-PRESCHOOL. HISTAMINE-LIBERATION. HUMAN. IMMUNIZATION-PASSIVE. RESPIRATORY-FUNCTION-TESTS. SKIN-TESTS. SUPPORT-U-S-GOVT-P-H-S. AB Because immediate hypersensitivity reactions can occur in individuals exposed to powdered pancreatic extracts, 36 patients with cystic fibrosis and 51 patents of such patients wwer studied for evidence of sensitization. Sensitivity to the extracts as evidence by history and skin testing was infrequent in the children with cystic fibrosis. However, skin testing for immediate hypersensitivity with either crude pancreatic extracts or inactivated trypsin correlated well in their patents with a history of clinical symptoms. IgE mediation of these reactions in sensitized individuals was demonstrated by antigen- induced histamine release from leukocytes, passive transfer studies, and immediate response to inhalation challenge. RF 001 FRANZ T J ALLERGY CLIN IMMUNOL 47 170 971 002 DOLOVICH J J ALLERGY CLIN IMMUNOL 49 43 972 003 WATT A CLIN ALLERGY 3 133 973 004 PEPYS J CLIN ALLERGY 3 143 973 005 ZETTERSTROM O CLIN ALLERGY 4 273 974 006 COLTEN HR N ENGL J MED 292 1050 975 007 DOLAN TF JR AM REV RESPIR DIS 110 812 974 008 BERGNER A PEDIATRICS 55 814 975 009 HILL D MED J AUST 2 553 975 010 MAY CD J ALLERGY 46 12 970 011 AXEN R EUR J BIOCHEM 18 351 971 012 COOK CD J PEDIATR 59 820 961 013 MURRAY AB J PEDIATR 62 186 963 014 KULCZYCKI LL JAMA 175 358 961 015 RACHELEFSKY GS AM J DIS CHILD 128 355 974 016 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 017 WARREN CPW CLIN ALLERGY 5 1 975 018 COUNAHAN R ARCH DIS CHILD 50 477 975 019 CHASE MW PROC SOC EXP BIOL MED 61 257 946 020 CHASE MW IN: SHAFFER JH 507 959 021 DAVID MF J ALLERGY CLIN IMMUNOL 55 135 975 022 LITTMAN A N ENGL J MED 281 201 969 023 FREEDMAN S J APPL PHYSIOL 38 974 975 024 PEPYS J CLIN ALLERGY SUPPL 3 491 973 025 PEPYS J AM REV RESPIR DIS 112 829 975 026 DOLOVICH J J ALLERGY CLIN IMMUNOL 52 38 973 027 ROBERTSON DG J ALLERGY CLIN IMMUNOL 54 244 974 CT 1 FORSBECK M LANCET 2 524 978 2 KARR RM J ALLERGY CLIN IMMUNOL 61 54 978 3 ROMEO G NATURE 274 909 978 4 BAUR X CLIN ALLERGY 9 75 979 5 GANIER M CLIN ALLERGY 9 125 979 6 ROMEO G PEDIATR RES 13 1030 979 7 PARK RW GASTROENTEROLOGY 81 1143 981 8 SJOGREN B SCAND J WORK ENVIRON HEALTH 9 385 983 9 BAUR X DTSCH MED WSCHR 109 257 984 10 PERRY RS CLIN PHARMACY 4 161 985 11 WIESSMANN KJ EUR J RESPIR DIS 66 13 985 PN 77092 RN 00673 AN 77228433 AU Blacharsh-C. TI Dental aspects of patients with cystic fibrosis: a preliminary clinical study. SO J-Am-Dent-Assoc. 1977 Jul. 95(1). P 106-10. MJ CYSTIC-FIBROSIS: co. TOOTH-DISEASES: et. MN COMPARATIVE-STUDY. DENTAL-PLAQUE: et. FOOD-HABITS. GINGIVITIS: et. HUMAN. MOUTH-BREATHING: et. TOOTH-DISCOLORATION: et. TOOTH-MOBILITY: et. AB The oral conditions of 42 patients being treated for cystic fibrosis were evaluated. The patients were grouped by age and, in some aspects, were compared with a small control group of their siblings. The patient group had a lowered incidence of plaque and less gingival disease than did the control group in which every person had some amount of plaque or gingival disease. Calculus formation was minimal. The reasons for the finding of minimal plaque in the patient group could be related to several factors, including the life-long use of various antibiotic agents, the chewing of digestive enzyme supplements, the effect of medical management on tooth hardness, and the effect of stained teeth (possible tetracycline deposition) on the plaque microorganisms. It appears that the therapy for cystic fibrosis was beneficial to the periodontal health of these patients. Much further study is needed to understand the interrelationship between an altered oral environment (salivary changes in cystic fibrosis), altered microbial flora (by antibiotics, enzymes) and even altered tooth surfaces (possible tetracycline deposition). Most patients were found to have one or more oral habits. Tooth mobility was associated with tension habits. Patients who had clubbed fingers (indicating pulmonary compromise) and possibly a severe disease process, did not appear to have either stained teeth or the severity of the gingivitis associated with this. The relationship of tetracycline to tooth staining could not be pinpointed. RF 002 CHERNICK WS J PEDIATR 59 890 961 003 MANDEL ID AM J DIS CHILD 113 431 967 004 WOTMAN S J PERIODONTOL 44 278 973 005 ZEGARELLI EV PEDIATRICS 26 1050 960 006 APPLEBAUM E ORAL SURG 17 366 964 008 REITMAN AA JADA 71 1436 965 009 GREENE JC JADA 68 25 964 010 ZEGARELLI EV ORAL SURG 15 929 962 011 JENSEN AL JADA 59 923 959 012 ENNEVER J J PERIODONTOL 32 331 961 013 KEYES PH ARCH ORAL BIOL 9 377 964 014 KEYES PH IADR PROGRAM ABST 67 965 CT 1 PRIMOSCH RE ORAL SURG 50 301 980 2 SING CF AM J MED GENET 13 179 982 3 MAHANEY MC ARCH ORAL BIOL 31 363 986 PN 77093 RN 00674 AN 77187640 AU Ligas-J-R. Primiano-F-P-Jr. Saidel-G-M. Doershuk-C-F. TI Comparison of measures of forced expiration. SO J-Appl-Physiol. 1977 Apr. 42(4). P 607-13. MJ CYSTIC-FIBROSIS: pp. LUNG: ph. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FORCED-EXPIRATORY-FLOW-RATES. HUMAN. METHODS. MODELS-BIOLOGICAL. SUPPORT-U-S-GOVT-P-H-S. VITAL-CAPACITY. AB Theoretical relationships among a number of parameters were derived for idealized timed vital capacity (TVC) and maximal expiratory flow- volume (MEFV) curves to determine a minimal set of independent parameters. Normal pediatric subjects and those with cystic fibrosis were studied to verify these relationships experimentally. The average flow over the middle half (FEF25-75%) of the forced vital capacity (FVC) and flows at various exhaled percentages of the FVC (FEF50%, FEF75%), as well as moments of the TVC and MEFV curves were computed. From the TVC moments, a mean transit time (MTT) and an index of dispersion (ID) were also calculated. The minimum information needed to detect pulmonary mechanical changes associated with obstructive lung disease requires at least two reproducible measures: one related to the mean slope (e.g., FEF25-75%/FVC or MTT) and the other to the shape (e.g;, ID) of the effort-independent portion of the MEFV curve. RF 001 ALLEN GW AM REV RESPIR DIS 104 61 971 002 BLACK LF AM REV RESPIR DIS 110 282 974 003 BOUHUYS A BREATHING 974 004 BOUHUYS A J CLIN INVEST 48 1159 969 005 CLEMENT J RESPIR PHYSIOL 15 70 972 006 CLEMENT J J APPL PHYSIOL 31 55 971 007 DOERSHUK CF J PEDIATR 65 677 964 008 FISH J AM REV RESPIR DIS 109 700 974 009 FRY DL AM J MED 29 672 960 010 GREEN M J APPL PHYSIOL 37 793 974 011 GREEN M J APPL PHYSIOL 37 67 974 012 HALD A STATISTICAL THEORY WITH ENGIN 952 013 HOEL PG INTRODUCTION TO MATHEMATICAL 971 014 HOGG JC N ENGL J MED 278 1355 968 015 HYATT RE AM REV RESPIR DIS 107 191 973 016 KNUDSON RJ AM REV RESPIR DIS 113 587 976 017 LANDAU LI AM REV RESPIR DIS 108 593 973 018 LANDAU LI AM REV RESPIR DIS 111 725 975 019 LEUALLEN EC AM REV TUBERC PULM DIS 72 783 955 020 MCCALL CB J APPL PHYSIOL 10 215 957 021 MCCARTHY DS AM REV RESPIR DIS 112 407 975 022 MEAD J J APPL PHYSIOL 22 95 967 023 NEUBURGER N J APPL PHYSIOL 40 329 976 024 PRIDE NB J APPL PHYSIOL 23 646 967 025 SAIDEL GM J APPL PHYSIOL 38 328 975 026 SOBOL BJ AM REV RESPIR DIS 107 753 973 027 TAKISHIMA TG SCAND J RESPIR DIS 48 384 967 028 WENG TR AM REV RESPIR DIS 99 879 969 029 ZAMEL N AM REV RESPIR DIS 107 861 973 030 ZAPLETAL A PEDIATRICS 48 64 971 CT 1 TSAI MJ IEEE TRANS BIOMED ENG 26 293 979 2 PIMMEL RL AM REV RESPIR DIS 120 1245 979 3 PREFAUT C BULL EUR PHYSIOPATH RESP 16 25 980 4 MENKES H ANN BIOMED ENG 9 501 981 5 PIMMEL RL J APPL PHYSIOL 51 1581 981 6 PESLIN R BULL EUR PHYSIOPATH RESP 18 679 982 7 GILBERT R ARCH INTERN MED 145 1635 985 PN 77094 RN 00675 AN 78026551 AU Shapira-E. Martin-C-L. Nadler-H-L. TI Comparison between purified alpha2-macroglobulin preparations from normal controls and patients with cystic fibrosis. Kinetic and structural properties of the complex with bovine trypsin. SO J-Biol-Chem. 1977 Nov 25. 252(22). P 7923-9. MJ ALPHA-MACROGLOBULINS. CYSTIC-FIBROSIS: bl. MN ALPHA-MACROGLOBULINS: im, ip. CHROMATOGRAPHY-GEL. COMPARATIVE-STUDY. HUMAN. IMMUNOELECTROPHORESIS. KINETICS. PROTEIN-BINDING. TRYPSIN. SUPPORT-U-S-GOVT-P-H-S. AB Alpha-2-macroglobulin was purified from plasma obtained from three healthy controls and three patients with cystic fibrosis. The normal and patient preparations were indistinguishable antigenically and in their UV absorption spectra. Competitive inhibition by these preparations of the hydrolysis of benzoyl-L-arginine ethyl ester by bovine trypsin was observed. The Ki value for normal alpha-2-macroglobulin was 5.3 x 10-8 M as compared to 1.8 x 10-7 M for cystic fibrosis patients preparations. The km values of the alpha-2-macroglobulin-trypsin complex were 6.3 x 10-4 M for the normal protein and 1.5 x 10-4 M with the alpha-2-macroglobulin from the cystic fibrosis patients. Gel chromatography of normal alpha-2-macroglobulin, preincubated in molar ratios with bovine trypsin, revealed the formation of alpha-2-macroglobulin fragments. These fragments were not obtained with the preparations from the cystic fibrosis patients. Both the normal and cystic fibrosis alpha-2-macroglobulins were shown by gel chromatography in sodium dodecyl sulfate to be tetramers, noncovalently bound. After reduction and alkylation the monomers were found to be a single polypeptide chain. The fragments obtained by preincubating normal alpha-2-macroglobulin with trypsin had approximately half the molecular weight of the intact monomer. Cystic fibrosis alpha-2-macroglobulin preincubated with trypsin in sodium dodecyl sulfate a single major component identical to the intact monomers, with no formation of alpha-2-macroglobulin fragments. RF 001 JONES JM BIOCHEM J 127 187 972 002 BARRETT AJ BIOCHEM J 133 709 973 003 ROBERTS RC IN: FRITZ H 63 974 004 DUNN JT J BIOL CHEM 242 5549 967 005 HARPEL PC J EXP MED 138 508 973 006 BAUMSTARK JS BIOCHIM BIOPHYS ACTA 207 318 970 007 SAUNDERS R J CLIN INVEST 50 2376 971 008 SUGIHAR H J BIOCHEM (TOKYO) 70 649 971 009 BIETH J FEBS LETTERS 8 319 970 010 DOLOVICH J J LAB CLIN MED 77 951 971 011 SCHREIBER AD J CLIN INVEST 52 1394 973 012 OHLSSON K SCAND J CLIN LAB INVEST 34 349 974 013 BERTHILLIER G BIOCHIM BIOPHYS ACTA 170 140 968 014 MCCONNELL DJ PROC SOC EXP BIOL MED 147 891 974 015 SHAPIRA E PEDIATR RES 10 812 976 016 WILSON GB PEDIATR RES 10 87 976 017 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 018 SHAPIRA E J LAB CLIN MED 77 877 971 019 HUNTER WM NATURE 194 495 962 020 TRAVIS J J BIOL CHEM 240 1962 965 021 DIXON M BIOCHEM J 55 170 953 022 HANES CS BIOCHEM J 26 1406 932 023 DIXON M ENZYMES 316 964 024 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 025 IWAMOTO M BIOCHIM BIOPHYS ACTA 214 402 970 026 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 027 SHAPIRA E CLIN CHIM ACTA 78 359 977 028 LENAD J BIOCHEM BIOPHYS RES COMMUN 45 662 971 029 RINDERKNECHT H IMMUNOCHEMISTRY 12 1 975 030 ARNON R IN: SELA M 88 973 031 SHAPIRA E BIOCHEMISTRY 6 3951 967 032 CITRI N ADV ENZYMOL 37 397 973 033 BECHET JJ BIOCHIM BIOPHYS ACTA 178 561 969 034 BARRETT AJ IN: FRITZ H 72 974 035 GAUTHIER F C R SOC BIOL (PARIS) 168 437 974 CT 1 ALHADEFF JA CLIN GENET 14 189 978 2 TRAVIS J BIOCHEMISTRY 17 5651 978 3 WILSON GB J LAB CLIN MED 92 463 978 4 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 5 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 6 VANLEUVEN F PEDIATR RES 13 1384 979 7 BENYOSEPH Y CLIN CHIM ACTA 99 31 979 8 KURECKI T ANAL BIOCHEM 99 415 979 9 BARRETT AJ BIOCHEM J 181 401 979 10 SCANLIN TF CLIN CHEST MED 1 424 980 11 DAVIS PB PEDIATR RES 14 83 980 12 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 13 ROMEO G J LAB CLIN MED 95 116 980 14 PARSONS M CLIN CHIM ACTA 100 215 980 15 KRESS LF BIOCHIM BIOPHYS ACTA 613 469 980 16 NELLES LP BIOCHIM BIOPHYS ACTA 623 46 980 17 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 18 HALLINAN F MED HYPOTHESES 7 793 981 19 BENYOSEPH Y PEDIATR RES 15 839 981 20 RICHMAN JBY CAN J BIOCHEM 59 519 981 21 VANLEUVEN F J BIOL CHEM 256 9016 981 22 LAINE A BIOCHIM BIOPHYS ACTA 668 429 981 23 BLITZER MG J PEDIATR GASTROENTEROL NUTR 1 289 982 24 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 25 BURY AF IRCS MED SCI BIOCHEM 10 255 982 26 BLITZER MG PEDIATR RES 16 203 982 27 ROBERTS RC PEDIATR RES 16 416 982 28 CRISTOL P SEM HOP PARIS 58 449 982 29 BRIDGES MA CLIN CHIM ACTA 118 21 982 30 BRIDGES MA CLIN CHIM ACTA 118 33 982 31 TUMMLER B CLIN CHIM ACTA 125 219 982 32 BACK SA BIOCHEM MED 30 34 983 33 SLOMIANY A J BIOL CHEM 258 8535 983 34 BRIDGES MA ANN NY ACAD SCI 421 360 983 35 SHAPIRA E ANN NY ACAD SCI 421 352 983 36 EAGER KB PEDIATR RES 18 999 984 37 MARYNEN P BIOCHIM BIOPHYS ACTA 799 187 984 38 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 PN 77095 RN 00676 AN 77207468 AU Farrell-P-M. Bieri-J-G. Fratantoni-J-F. Wood-R-E. di-SantAgnese-P-A. TI The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis. SO J-Clin-Invest. 1977 Jul. 60(1). P 233-41. MJ CYSTIC-FIBROSIS: co. VITAMIN-E-DEFICIENCY: co. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. FEMALE. HEMOLYSIS. HUMAN. INFANT. INTESTINAL-ABSORPTION. MALABSORPTION-SYNDROMES: co. MALE. PANCREATIC-DISEASES: co. TRIGLYCERIDES: bl. VITAMIN-E-DEFICIENCY: dt. VITAMIN-E: me, tu. AB The role of vitamin E in human nutrition was studied by investigation of patients with cystic fibrosis (CF) and associated pancreatic insufficiency. Vitamin E status was assessed by measurement of the plasma concentration of the principal circulating isomer, alpha-tocopherol. Results of such determinations in 52 CF patients with pancreatogenic steatorrhea revealed that all were deficient in the vitamin. The extent of decreased plasma tocopherol varied markedly but correlated with indices of intestinal malabsorption, such as the serum carotene concentration and percentage of dietary fat absorbed. Supplementation with 5-10 times the recommended daily allowance of vitamin E in a water-miscible form increased the plasma alpha-tocopherol concentrations to normal in all 19 CF patients so evaluated. Studies on the effects of vitamin E deficiency focused on possible hematologic alterations. An improved technique was developed to measure erythrocyte hemolysis in vitro in the presence of hydrogen peroxide. While erythrocyte suspensions from control subjects demonstrated resistance to hemolysis during a 3-h incubation, all samples from tocopherol-deficient CF patients showed abnormal oxidant susceptibility, evidenced by greater than 5% hemoglobin release. The degree of peroxide-induced hemolysis was related to the plasma alpha-tocopherol concentrationin an inverse, sigmoidal manner. The possibility of in vivo hemolysis was assessed by measuring the survival of 51Cr-labeled erythrocytes in 19 vitamin-E deficient patients. A moderate but statistically significant decrease in the mean 51Cr erythrocyte half-life value was found in this group. Measurement of erythrocyte survival before and after supplementation of 6 patients with vitamin E demonstrated that the shortened erythrocyte lifespan could be corrected to normal with this treatment. Other hematologic indices in deficient subjects, however, were normal and did not change upon supplementation with vitamin E. It is concluded that CF is invariably associated with vitamin E deficiency, provided that the patient in question has pancreatic achylia and is not taking supplementary doses of tocopherol. Concomitant hematologic effects consistent with mild hemolysis, but not anemia, occur and may be reversed with vitamin E therapy. Patients with CF should be given daily doses of a water-miscible form of vitamin E to correct the deficiency. RF 001 BINDER HJ N ENGL J MED 273 1289 965 002 FILER LJ JR PEDIATRICS 8 328 951 003 NITOWSKY HM VITAM HORM 20 559 962 004 GORDON HH AM J DIS CHILD 62 328 941 005 BIERI JG NUTR REV 33 161 975 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 NITOWSKY HM BULL JOHNS HOPKINS HOSP 98 361 956 008 GOLDBLOOM RB CAN MED ASSOC J 82 1114 960 009 MCWHIRTER WR ACTA PAEDIATR SCAND 64 446 975 010 HORWITT MK ANN NY ACAD SCI 203 223 972 011 BIERI JG PROC SOC EXP BIOL MED 120 554 965 012 BIERI JG IN: MARINETTI GV 2 459 969 013 BIERI JG INT J VITAM RES 40 344 970 014 HORWITT MK AM J CLIN NUTR 4 408 956 015 ANON BLOOD 38 378 971 016 VAN DE KAMER JH J BIOL CHEM 177 347 949 017 KESSLER G IN: SKEGGS LT 341 965 018 DE LA HUERGA J AM J CLIN PATHOL 23 1163 953 019 BIERI JG J NUTR 100 557 970 020 HARRIS PL PROC SOC EXP BIOL MED 107 381 961 021 BIERI JG PROC SOC EXP BIOL MED 117 131 964 022 GORDON HH AM J DIS CHILD 90 669 955 023 MULLER DPR GUT 15 966 974 024 UNDERWOOD BA PEDIATR RES 6 26 972 025 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 026 MACMAHON MT CLIN SCI 38 197 970 027 LOSOWSKY MS ANN NY ACAD SCI 203 212 972 028 NITOWSKY HM AM J DIS CHILD 92 164 956 029 TSEN CC CAN J BIOCHEM PHYSIOL 38 957 960 030 OSKI FA J PEDIATR 70 211 967 031 PANOS TC AM J CLIN NUTR 21 15 968 032 LEONARD PJ AM J CLIN NUTR 24 388 971 033 BIERI JG AM J CLIN NUTR 28 717 975 CT 1 ANON LANCET 2 1268 977 2 ROCKERBIE RA CLIN CHEM 25 1411 979 3 TOMASI LG NEUROLOGY 29 1182 979 4 CHOW CK AM J CLIN NUTR 32 1066 979 5 RACHMILEWITZ EA AM J CLIN NUTR 32 1850 979 6 WOOD RE SOUTH MED J 72 189 979 7 SPIELBERG SP ANN INTERN MED 90 53 979 8 CHIU D J LAB CLIN MED 94 542 979 9 CHASE HP J PEDIATR 95 337 979 10 FARRELL PM J PEDIATR 95 869 979 11 WEISMAN Y J PEDIATR 95 416 979 12 DRAKE JR AM J CLIN NUTR 33 2386 980 13 HORWITT MK NUTR REV 38 105 980 14 SUNG JH J NEUROPATHOL EXP NEUROL 39 584 980 15 RACHMILEWITZ EA BR J HAEMATOL 46 1 980 16 SCHULMAN JD ANN INTERN MED 93 330 980 17 HUBBARD VS CLIN CHIM ACTA 102 115 980 18 ROGIERS V CLIN CHIM ACTA 105 105 980 19 SIMON C EUR J PEDIATR 133 273 980 20 CORASH L N ENGL J MED 303 416 980 21 ANDREWS WS J PEDIATR SURG 16 284 981 22 CHO YW J CLIN PHARMACOL 21 224 981 23 CAVALIER SJ NEUROLOGY 31 714 981 24 CONGDEN PJ ARCH DIS CHILD 56 708 981 25 ANON S AFR MED J 59 618 981 26 PARK RW GASTROENTEROLOGY 81 1143 981 27 HAGA P EUR J PEDIATR 136 143 981 28 ROSENBLUM JL N ENGL J MED 304 503 981 29 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 30 ROGIERS V PEDIATR RES 16 761 982 31 ALBRIGHT RK KIDNEY INT 21 161 982 32 HAGA P AM J CLIN NUTR 36 1200 982 33 HOWARD L AM J CLIN NUTR 36 1243 982 34 VANVLEET JF AM J VET RES 43 1049 982 35 DUTTA SK ANN INTERN MED 97 549 982 36 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 37 CHIU D ANN NY ACAD SCI 393 323 982 38 CORASH LM ANN NY ACAD SCI 393 348 982 39 KITABCHI AE ANN NY ACAD SCI 393 300 982 40 RACHMILEWITZ EA ANN NY ACAD SCI 393 336 982 41 STUART MJ ANN NY ACAD SCI 393 277 982 42 KITABCHI AE BIOCHIM BIOPHYS ACTA 684 200 982 43 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 44 NEVILLE HE NEUROLOGY 33 483 983 45 FARRELL PM J DENT CHILD 50 385 983 46 KON K ACTA HAEMATOL 69 111 983 47 GUGGENHEIM MA J PEDIATR 102 577 983 48 BURTON GW ARCH BIOCHEM BIOPHYS 221 281 983 49 BIERI JG N ENGL J MED 308 1063 983 50 DEARBORN DG PEDIATR RES 18 890 984 51 ROGIERS V PEDIATR RES 18 704 984 52 PRENSKY AL DEVELOP MED CHILD NEUROL 26 669 984 53 OVESEN L DRUGS 27 148 984 54 BERTONI JM NEUROLOGY 34 1046 984 55 GUTCHER GR AM J CLIN NUTR 40 1078 984 56 BOUGLE D ARCH FR PEDIATR 41 63 984 57 DAVIS PB J LAB CLIN MED 104 203 984 58 ANON JAMA 252 U316 984 59 SOKOL RJ N ENGL J MED 310 1209 984 60 STEAD RJ LANCET 2 862 985 61 GUTCHER GR J PEDIATR GASTROENTEROL NUTR 4 604 985 62 CARPENTER D SEM NEUROL 5 283 985 63 HUBBARD VS SEM RESPIR MED 6 308 985 64 RACHMILEWITZ EA CLIN HAEMATOL 14 163 985 65 FARRELL PM PEDIATR RES 19 104 985 66 COSTAGLIOLA C METABOLISM 34 712 985 67 HADDAD E AM J CLIN NUTR 41 599 985 68 MINO M AM J CLIN NUTR 41 631 985 69 BYE AME ARCH DIS CHILD 60 162 985 70 CLARK JH CLIN CHIM ACTA 153 117 985 71 HARDING AE N ENGL J MED 313 32 985 72 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 73 KALVARIA I INT J PANCREATOL 1 119 986 74 HANDELMAN GJ ADV FREE RAD BIOL MED 2 1 986 75 SOKOL RJ HEPATOLOGY 6 1263 986 76 BOUGLE D DEVELOP PHARM THER 9 310 986 77 MISCHLER EH PEDIATR RES 20 36 986 78 STEAD RJ GUT 27 714 986 79 ARANDA JV PEDIATR CLIN NORTH AM 33 583 986 80 COSTAGLIOLA C EXP EYE RES 43 905 986 81 ANON NUTR REV 44 373 986 82 DAVIDAI G ARCH DIS CHILD 61 901 986 83 KAY MMB PROC NAT ACAD SCI USA 83 2463 986 84 ASAYAMA K J LAB CLIN MED 107 459 986 85 HAFEZ M J PEDIATR 108 558 986 86 HUANG ML J CHROMATOGR 380 331 986 87 SOKOL RJ J PEDIATR GASTROENTEROL NUTR 6 10 987 88 REED JD AM J HEMATOL 24 441 987 89 SITRIN MD ANN INTERN MED 107 51 987 PN 77096 RN 00677 AN 78195444 AU Chakravarty-S-C. Bhattacharya-S-N. Sarkar-D-K. TI Bronchiectasis and mucoviscidosis. SO J-Indian-Med-Assoc. 1977 Dec 16. 69(12). P 273-5. MJ BRONCHIECTASIS: co. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. AGED. BRONCHIECTASIS: me. CASE-REPORT. CHLORIDES: an. HUMAN. MALE. MIDDLE-AGE. SWEAT: an. AB Sweat chloride of 25 healthy adults was tested by iontophoresis method as a control. The mean value was 27.4 plus or minus 15.4 mEq./l. Thirty-six adult patients of bronchiectasis proved by bronchography was investigated for mucoviscidosis. If a patient had sweat chloride above 60 mEq./l., it was considered to be diagnostic of mucoviscidosis. Five (13.9 per cent) of 36 adult bronchiectasis patients had mucoviscidosis. The mean value of sweat chloride in 31 adult bronchiectasis patients was 32.9 plus or minus 11.7 mEq./l. The mean value of sweat chloride in 36 adult bronchiectasis patients (including 5 mucoviscidosis patients) was 42.3 plus or minus 26.8 mEq./l. Of 36 patients 18 had cylindrical type and 18 had saccular type of bronchiectasis. Three (16.6 per cent) out of 18 cylindrical type of bronchiectasis patients and 2 (11.1 per cent) of 18 saccular type of bronchiectasis patients had mucoviscidosis. Five (13.9 per cent) patients had emphysema and 6 (16.6 per cent) patients had emphysema with cor pulmonale. One patient of each group had mucoviscidosis. ECG showed P-pulmonale in 3 (8.3 per cent) patients one of whom had mucoviscidosis. Only one mucoviscidosis patient had coagulase positive Staph. aureus in sputum. RF 001 AGREN G ACTA MED SCAND 90 1 936 002 ANDERSEN DH AM J DIS CHILD 63 643 942 003 CHAHRAVARTY SC J INDIAN MED ASSOC 53 554 969 004$ CHILDS B TRANS INT CONF ON CF 195 960 005 COHEN JS ABSTRACT OF WORLD MEDICINE 42 867 968 006 DI SANTAGNESE PA IN: CECIL RL 917 959 007 DI SANTAGNESE PA JAMA 172 135 960 008 GIBSON LE PEDIATRICS 23 545 959 009 JAGGI OP INDIAN J CHEST DIS 6 134 964 010 KARLISH AJ PROC R SOC MED 54 980 961 011 LAGERLOF HO PANCREATIC FUNCTION AND PANCR 942 012 LIEBERMAN J AM J MED SCI 246 537 963 013$ PETERSON EM JAMA 176 1 959 014 SARKAR DK THESIS 965 015 SHWACHMAN H ANN NY ACAD SCI 93 600 962 016 SUN DCH GASTROENTEROLOGY 38 570 960 PN 77097 RN 00678 AN 78110292 AU Kim-H-W. Arrobio-J-O. Brandt-C-D. Chanock-R-M. Parrott-R-H. TI Safety and antigenicity of inactivated influenza virus vaccines in children: trials with monovalent and bivalent A/New Jersey/76 (HswN1) and A/Victoria/75 virus vaccines in Washington, D.C. SO J-Infect-Dis. 1977 Dec. 136 Suppl. P S588-91. MJ INFLUENZA-VACCINE: pd. ORTHOMYXOVIRUS-TYPE-A-HUMAN: im. MN ADOLESCENCE. ANTIBODIES-VIRAL: bi. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im. DISTRICT-OF-COLUMBIA. DOSE-RESPONSE-RELATIONSHIP-IMMUNOLOGIC. ENGLAND. HEMAGGLUTINATION-INHIBITION-TESTS. HUMAN. INFLUENZA-VACCINE: ae. NEW-JERSEY. PATIENT-CARE-TEAM. SUPPORT-U-S-GOVT-P-H-S. AB Safety and antigenicity of monovalent and bivalent A/New Jersey/NJ)/76 (HssN1) and A/Victoria/75 inactivated influenza virus vaccines were studied in 125 children aged three to 18 years. In recruitment, families who knew the study team, who were professionally involved, and/or who were under close continuing care were more likely to volunteer for such studies than those who were unfamiliar with the team or institution. Antibody responses and systemic reactions occurred more often after administration of inactivated whole-virus vaccine than after split-virus vaccine. Significant titers (greater than or equal to 1:40) of hemagglutination-inhibiting antibody to A/NJ/76 virus occurred in 95% of normal children three to 18 years of age who received two doses of the same vaccine (whole or split). However, insufficient numbers of children achieved a reasonable antibody titer (greater than or equal to 1:40) after one dose of vaccine. CT 1 VASILYEVA RI ZH MIKROBIO EPIDEMIO IMMUNOBI 1982 96 982 PN 77098 RN 00679 AN 78110300 AU Modlin-J-F. Smith-D-H. Harding-L. TI Clinical trials of bivalent A/New Jersey/76-A/Victoria/75 influenza vaccines in high-risk children. SO J-Infect-Dis. 1977 Dec. 136 Suppl. P S626-31. MJ INFLUENZA-VACCINE: pd. ORTHOMYXOVIRUS-TYPE-A-HUMAN: im. MN ADOLESCENCE. ANTIBODIES-VIRAL: bi. ASTHMA: im. CHILD. CHRONIC-DISEASE. CYSTIC-FIBROSIS: im. DIABETES-MELLITUS-INSULIN-DEPENDENT: im. ENDOCRINE-DISEASES: im. FEMALE. HEART-DEFECTS-CONGENITAL: im. HUMAN. INFLUENZA-VACCINE: ae. LUNG-DISEASES: im. MALE. RISK. SUPPORT-U-S-GOVT-P-H-S. AB Various doses of two whole-virus and one split-product bivalent influenza A/New Jersey/76-A/Victoria/75 vaccines were administered to 253 children aged six to 18 years. There were no statistically significant differences in either reactivity or humoral antibody response among the 167 children in seven chronic disease categories and 86 healthy children. The whole-virus vaccines were associated with unacceptably high rates of reaction when given in sufficiently antigenic initial doses but were relatively nonreactive when used for booster immunization. Split-product vaccines were no more reactive than placebo. All vaccine preparations induced adequate seroconversion rates and protective titers of antibody to A/Victoria virus after one dose and to A/New Jersey virus after two doses. RF 001 EICKHOFF TC JAMA 176 776 961 002 WEIL WB IN: BARNETT HL 354 972 003 SCHRIENER GE IN: STRAUSS MB 606 971 004 ANON AM ACAD PEDIATRICS 139 974 005 LERMAN SJ J INFECT DIS SUPPL 136 563 977 006 PABICO RC ANN INTERN MED 81 171 974 007 PHILLIPS CF PEDIATRICS 52 416 973 008 KILBOURNE ED J INFECT DIS 129 750 974 009 NICHOLAS RV PEDIATRICS 3 208 949 010 HIGGONS RA AM J DIS CHILD 75 887 948 011 GRANT HB J PEDIATR 29 485 946 012 REIMER CB J VIROL 1 1207 967 013 MARINE WM J PEDIATR 88 26 976 014 KILBOURNE ED J INFECT DIS 127 220 973 PN 77099 RN 00680 AN 77166681 AU Yaffe-S-J. Gerbracht-L-M. Mosovich-L-L. Mattar-M-E. Danish-M. Jusko-W-J. TI Pharmacokinetics of methicillin in patients with cystic fibrosis. SO J-Infect-Dis. 1977 May. 135(5). P 828-31. MJ CYSTIC-FIBROSIS: me. METHICILLIN: me. MN ADOLESCENCE. ADULT. BACILLUS-SUBTILIS: de. CREATININE: ur. FEMALE. HUMAN. MALE. METHICILLIN: ur. SUPPORT-U-S-GOVT-P-H-S. AB The disposition of methicillin in normal subjects and in subjects with cystic fibrosis (CF) was studied after administration of single intravenous doses of 15 mg/kg. The area under the serum concentration vs. time curve for CF patients was, on the average, only 75% of that found for normal subjects. The low concentrations in serum were caused by more rapid urinary excretion of the antibiotic, with rates of renal clearance averaging 425 ml/min per 1.73 m2 in the patients with CF and 362 ml/min per 1.73 m2 in the normal subjects. No differences were found in volumes of distribution and metabolic clearance rates of methicillin or in rates of creatinine clearance between the two groups of subjects. These data support previous findings with dicloxacillin which show that patients with CF exhibit unusually rapid, active tubular secretion of certain penicillins that may necessitate use of larger doses of these drugs in treatment of infections. RF 001 LOWE CU IN: BARNETT HL 413 972 002 JUSKO WJ PEDIATRICS 56 1038 975 003 DITTERT LW ANTIMICROB AGENTS CHEMOTHER 9 42 969 004 BENNETT JV APPL MICROBIOL 14 170 966 005 HEINEGARD D CLIN CHIM ACTA 43 305 973 006 METZLER CM TECHNICAL REPORT UPJOHN COMPA 969 007 JUSKO WJ J PHARM SCI 61 1270 972 008 LOO JCK J PHARM SCI 59 53 970 009 HIRSCH GH J PHARMACOL EXP THER 174 152 970 CT 1 SOUICH PD CLIN PHARMACOKINET 3 257 978 2 MARKS MI ANTIMICROB AGENTS CHEMOTHER 13 753 978 3 BERGAN T ARZNEIMITTEL FORSCH DRUG RES 29-2 1955 979 4 FINKELSTEIN E J PEDIATR 94 163 979 5 TOGNONI G CLIN PHARMACOKINET 5 105 980 6 BENNETT SW AM J HOSP PHARM 37 523 980 7 BEAUDRY PH J PEDIATR 97 144 980 8 BAGGOT JD J AM VET MED ASSOC 176 1085 980 9 MICHALSEN H ANTIMICROB AGENTS CHEMOTHER 19 1029 981 10 MARKS MI J PEDIATR 98 173 981 11 STRANDVIK B LANCET 1 800 982 12 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 13 BINS JW BR J CLIN PHARMACOL 14 P611 982 14 NEU HC MED CLIN NORTH AM 66 51 982 15 KEARNS GL J PEDIATR 100 312 982 16 KELLY HB J PEDIATR 100 318 982 17 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 18 PADOAN R DRUGS UNDER EXP CLIN RES 9 661 983 19 HUANG NN J ANTIMICROB CHEMOTHER 11 205 983 20 KERCSMAR CM J ANTIMICROB CHEMOTHER 12 289 983 21 LEFF RD PEDIATR CLIN NORTH AM 30 93 983 22 ARVIDSSON A ACTA PAEDIATR SCAND 72 293 983 23 WESLEY AW NZ MED J 96 651 983 24 MACDONALD NE J PEDIATR 103 985 983 25 PADOAN R J PEDIATR 103 320 983 26 ISLES A AM REV RESPIR DIS 127 417 983 27 NAHATA MC DEVELOP PHARM THER 7 221 984 28 TURNER A J ANTIMICROB CHEMOTHER 14 521 984 29 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 30 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 31 REED MD ANTIMICROB AGENTS CHEMOTHER 25 579 984 32 ZIEMNIAK JA EUR J CLIN PHARMACOL 26 183 984 33 LEEDER JS CLIN PHARMACOL THER 36 355 984 34 REED MD J PEDIATR 104 303 984 35 SPINO M J PEDIATR 105 829 984 36 ANON LANCET 1 1020 985 37 KUHN RJ CLIN PHARMACY 4 555 985 38 WOOLF RA CLIN PHARMACY 4 664 985 39 PRANDOTA J DEVELOP PHARM THER 8 311 985 40 REED MD ANTIMICROB AGENTS CHEMOTHER 27 583 985 41 BLUMER JL AM J MED 79 37 985 42 HORREVORTS AM CHEST 88 260 985 43 JACOBS RF J PEDIATR 106 1001 985 44 WILSON CB J PEDIATR 106 1049 985 45 SPINO M J PEDIATR 107 64 985 46 ASSAEL BM INT J PEDIATR NEPHROL 7 213 986 47 MICHALSEN H METH FIND EXP CLIN PHARMACOL 8 727 986 48 MICHALSEN H METH FIND EXP CLIN PHARMACOL 8 685 986 49 BENDER SW INFECTION 14 17 986 50 WRIGHT DB DRUG INTEL CLIN PHARM 20 845 986 51 GOLDFARB J J CLIN PHARMACOL 26 222 986 52 RUBIO TT AM J MED 81 73 986 53 REED MD ANTIMICROB AGENTS CHEMOTHER 31 698 987 PN 77100 RN 00681 AN 77072953 AU Goldsmith-G-H. Stern-R-C. Saito-H. Ratnoff-O-D. TI Normal plasma arginine esterase and the Hageman factor (factor XII) -prekallikrein-kininogen system in cystic fibrosis. SO J-Lab-Clin-Med. 1977 Jan. 89(1). P 131-4. MJ CYSTIC-FIBROSIS: bl. ESTERASES: bl. FACTOR-XII. KALLIKREIN. KININOGENS: bl. PREKALLIKREIN. MN ADOLESCENCE. ADULT. ARGININE. CHILD. HUMAN. TOSYLARGININE-METHYL-ESTER: me. SUPPORT-U-S-GOVT-P-H-S. AB Previous investigators have suggested that the biological activity of plasma prekallikrein is defective in cystic fibrosis. In contrast, no such difference was demonstrable between normal and cystic fibrosis plasma. Esterolytic activity for the synthetic substrate p-toluene sulfonyl-arginine methyl ester (TAMe) evolved normally in cystic fibrosis plasma treated with chloroform and ellagic acid, a measure of generation of plasma kallikrein. Additionally, plasma prekallikrein (Fletcher factor) and high molecular weight kininogen (Fitzgerald factor), a substrate of plasma kallikrein, were normal. Thus, the concept that cystic fibrosis is associated with abnormalities in the plasma kallikrein-kinin system could not be supported. RF 001 MANGOS JA PEDIATR RES 2 378 968 002 RAO GJS J PEDIATR 80 573 972 003 SPOCK A PEDIATR RES 1 173 967 004 BOWMAN BH SCIENCE 164 325 969 005 RAO GJS SCIENCE 177 610 972 006 RAO GJS PEDIATR RES 8 684 974 007 LIEBERMAN J AM REV RESPIR DIS 109 399 974 008 SIEGELMAN AM ARCH BIOCHEM BIOPHYS 97 159 962 010 ZIMMERMAN TS J CLIN INVEST 50 244 971 011 SAITO H J CLIN INVEST 55 1082 975 012 COLMAN RW J CLIN INVEST 48 23 969 013 DONALDSON VH CIRC RES 34 652 974 014 OH-ISHI S BIOCHEM PHARMACOL 24 591 975 015 COBURN MD AM REV RESPIR DIS 110 368 974 016 TALAMO RC PEDIATR RES 6 430 972 017 RAO GJS PEDIATR RES 9 739 975 018 WILSON GB PEDIATR RES 10 87 976 PN 77101 RN 00682 AN 77120193 AU Berman-J-M. Colman-B-H. TI Nasal aspects of cystic fibrosis in children. SO J-Laryngol-Otol. 1977 Feb. 91(2). P 133-9. MJ CYSTIC-FIBROSIS: co. NOSE-DISEASES: et. MN CASE-REPORT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. MALE. NASAL-POLYPS: et, th. NOSE-DISEASES: th. AB Despite the extraordinary prevalence of upper respiratory allergy and infection in children nasal polyposis is an extremely rare condition. Seven cases of cystic fibrosis are presented in order to demonstrate the nasal aspects of the disease. All but one had nasal polyposis. The literature has been reviewed with particular reference to clinical, radiological and histo-pathological features. Conservative treatment is stressed. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BLUMSTEIN GI ARCH OTOLARYNGOL 83 266 966 003 COHEN SR ANN OTOL RHINOL LARYNGOL 71 1080 962 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 GHARIB R AM J DIS CHILD 108 499 964 006 KULCZYCKI LL JAMA 175 358 961 007 LURIE MH ANN OTOL RHINOL LARYNGOL 68 478 959 008 MENDELSOHN RS ARCH OTOLARYNGOL 79 312 964 009 NEELY JG LARYNGOSCOPE 82 910 972 010 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 011 SHWACHMAN H PEDIATRICS 30 389 962 012 TOMA GA J LARYNGOL OTOL 82 265 968 013 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 CT 1 LEE ABD INT J PEDIATR OTORHINOLARYNGO 4 209 982 2 MILLS SE ARCH OTOLARYNGOL 108 530 982 3 STERN RC AM J DIS CHILD 136 1067 982 4 BENJAMIN B INT J PEDIATR OTORHINOLARYNGO 5 281 983 PN 77102 RN 00683 AN 78047117 AU Tos-M. Mogensen-C. Thomsen-J. TI Nasal polyps in cystic fibrosis. SO J-Laryngol-Otol. 1977 Oct. 91(10). P 827-35. MJ CYSTIC-FIBROSIS: co. NASAL-POLYPS: co. MN CHILD. CHILD-PRESCHOOL. EPITHELIUM: pa. HUMAN. NASAL-MUCOSA: pa. NASAL-POLYPS: pa. AB In II polyps from patients with cystic fibrosis of the pancreas, the density, shape, and architecture of mucous glands were studied by the whole-mount method and compared with 102 non-cystic-fibrosis polyps. There were no differences in density which was in most polyps less than 0.5 gland/mm.2. The shape and architecture as well as other histological appearances of the polyp were also alike in both groups of polyps which could not be distinguished. The pathogenesis of nasal polyps in cystic fibrosis is discussed. Apparently, it is the same as that of non-cystic-fibrosis polyps. RF 001 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 002 MAGID SL ARCH OTOLARYNGOL 86 106 967 003 MENDELSOHN RS ARCH OTOLARYNGOL 79 312 964 004 MOGENSEN C INT RHINOLOGY 15 39 977 005 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 006 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 007 PENNINGTON CL ARCH OTOLARYNGOL 63 576 956 008 SHWACHMAN H PEDIATRICS 30 389 962 009 SORENSEN H PROC INT SYMP INFECT ALLE 233 977 010 TAYLOR BW ARCH DIS CHILD 49 133 974 011 TOS M ACTA PATH MICROBIOL SCAND S185 966 012 TOS M ANAT ANZ 126 146 970 013 TOS M ARCH OTOLARYNGOL 103 407 977 014 TOS M ARCH OTO RHINO LARYNGOL 215 101 977 015 TOS M INT RHINOLOGY 15 87 977 016 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 NAKASHIMA T ANN OTOL RHINOL LARYNGOL 88 210 979 2 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 3 STERN RC AM J DIS CHILD 136 1067 982 4 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 5 BUNNAG C ANN ALLERGY 50 126 983 6 BENDE M J LARYNGOL OTOL 99 167 985 7 DAVID TJ J ROY SOC MED 79 23 986 8 HIRAIDE F ACTA OTOLARYNGOL STOCKH SUPPL 430 5 986 PN 77103 RN 00684 AN 77120676 AU Cohen-L-F. di-SantAgnese-P-A. Taylor-A. Gill-J-R-Jr. TI The syndrome of inappropriate antidiuretic hormone secretion as a cause of hyponatremia in cystic fibrosis. SO J-Pediatr. 1977 Apr. 90(4). P 574-8. MJ CYSTIC-FIBROSIS: me. HYPONATREMIA: et. VASOPRESSINS: se. MN ADULT. CASE-REPORT. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. MALE. AB The syndrome of inappropriate secretion of antidiuretic hormone was observed in two patients with cystic fibrosis during acute exacerbation of chronic pulmonary disease. It was diagnosed by the accepted clinical and laboratory criteria and confirmed in one case by values for immunoreactive vasopressin that were inappropriately high for plasma osmolality. The severe hyponatremia was corrected by fluid restriction, alone or combined with intravenous treatment with diuretic and hypertonic saline solution. In addition, there was simultaneous therapy of the pulmonary disease. SIADH thus must be added to salt loss as a cause of hyponatremia in CF, and may be more common than realized in patients with CF and severe pulmonary disease. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 SCHWARTZ WB AM J MED 23 529 957 003 BARTTER FC AM J MED 42 790 967 004 KLENK EL IN: WINTERS RW 415 973 005 ROBERTSON GL J CLIN INVEST 52 2340 973 006 MILLER M ANN INTERN MED 77 715 972 007 MOR J AM J DIS CHILD 129 133 975 008 BARTTER FC DISEASE A MONTH 973 009 TAUSSIG LM J PEDIATR 82 380 973 010 HABER E J CLIN ENDOCRINOL METAB 29 134 969 011 ITO T J CLIN ENDOCRINOL METAB 34 106 972 012 ROLLINS DE CLIN RES 24 238A 976 013 DEWARDNER HE KIDNEY 53 967 014 DERUBERTIS FR AM J MED 51 41 971 015 MILLER M J CLIN ENDOCRINOL METAB 34 537 972 016 ROBERTSON GL ANNU REV MED 25 315 974 017 STROBER W PEDIATRICS 43 416 969 018 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 019 TAUSSIG LM J INVEST DERMATOL 60 197 973 020 ROBSON AM J PEDIATR 79 42 971 021 BOWER BF N ENGL J MED 271 934 964 022 GOLDRING RM J PEDIATR 65 501 964 023 SIASSI B J PEDIATR 78 794 971 024 BENSON H J CLIN INVEST 49 791 970 025 SCHRIER RW N ENGL J MED 292 81 975 026 GOETZ KL PHYSIOL REV 55 157 975 027 HANTMAN D ANN INTERN MED 78 870 973 CT 1 SCHIFFER M J PEDIATR 91 850 977 2 POMAREDE R ARCH FR PEDIATR 35 75 978 3 SANTAGNESE PAD AM J MED 66 121 979 4 ZERBE R ANNU REV MED 31 315 980 5 KREMEN AF MINN MED 63 385 980 6 RIVERS RPA ARCH DIS CHILD 56 358 981 PN 77104 RN 00685 AN 77120677 AU Rossman-C. Dodlovich-J. Dodlovich-M. Wilson-W. Newhouse-M. TI Cystic fibrosis--related inhibition of mucociliary clearance in vivo in man. SO J-Pediatr. 1977 Apr. 90(4). P 579-84. MJ CILIA: ph. CYSTIC-FIBROSIS: pp. MUCUS: ph. NASAL-MUCOSA: pp. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: bl. HUMAN. IGE. AB Studies were performed to demonstrate possibly cystic fibrosis- related inhibition of mucociliary clearance in man. Topical application of normal serum or of CF serum did not inhibit in vivo nasal MCC. Induction of local inflammation by topical anti-IgE- reduced nasal MCC in CF subjects, but increased MCC in normal individuals. Furthermore, nasal MCC was inhibited in normal patients by CF serum but not normal serum, applied to the anti-IgE-treated nasal mucosa. These observations are consistent with the hypothesis that CF serum inhibits MCC in vivo in the inflamed mucosa. RF 001 SPOCK A PEDIATR RES 1 173 967 002 BOWMAN BH SCIENCE 164 325 969 003 BESLEY GTN J MED GENET 6 278 969 004 CRAWFURD MDA IN: LAWSON D PROC 5TH INT CF 42 969 005 WOOD RE LANCET 2 1452 973 006 CHRISTENSEN J IN: LAWSON D PROC 5TH INT CF 49 969 007 CHERRY JD J PEDIATR 79 937 971 008 SANCHIS J N ENGL J MED 288 651 973 009 YEATES DB ARCH DIS CHILD 51 28 976 010 THOMSON ML N ENGL J MED 289 749 973 011 WOOD RE AM REV RESPIR DIS 111 733 975 012 PROCTOR DF IN: DAVIES CN 2 25 967 013 SANCHIS J J APPL PHYSIOL 33 757 972 014 ISHIZAKA K J IMMUNOL 100 554 968 015 PROCTOR DF ARCH INTERN MED 131 132 973 016 ANDERSEN I ARCH ENVIRON HEALTH 29 290 974 017 ANDERSEN I ARCH ENVIRON HEALTH 23 408 971 018 BANG BG JOHNS HOPKINS MED J 121 38 967 019 BOXERBAUM B AM REV RESPIR DIS 108 777 973 020 DANES BS J EXP MED 136 1313 972 021 DANES BS LANCET 2 765 973 022 MANGOS JA PEDIATR RES 1 436 967 023 MANGOS JA SCIENCE 158 135 967 024 SPOCK A POSTGRAD MED J 50 92 971 025 BOWMAN BH SCIENCE 167 871 970 026 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 027 DANES BS J EXP MED 137 1538 973 028 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 029 SHWACHMAN H AM J DIS CHILD 96 6 958 030 WANNER A CHEST 61 287 972 CT 1 GOTZ M EUR J PEDIATR 127 133 978 2 AFZELIUS BA INT REV EXP PATHOL 19 1 979 3 PAVIA D CLIN RESP PHYSIOL 16 335 980 4 ROSSMAN CM AM REV RESPIR DIS 121 1011 980 5 RUTLAND J THORAX 36 654 981 6 BERGAN T SCAND J INFECT DIS 1981 7 981 7 NEWHOUSE MT EUR J RESPIR DIS 64 151 983 8 ROSSMAN CM EUR J RESPIR DIS 64 64 983 9 RUTLAND J AM REV RESPIR DIS 128 1030 983 10 ROSSMAN CM AM REV RESPIR DIS 129 161 984 11 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 12 TODISCO T EUR J RESPIR DIS 66 136 985 13 KOHLER D ATEMWEGS LUNGENKRANKH 12 358 986 14 MATTHYS H EUR J RESPIR DIS 69 311 986 PN 77105 RN 00686 AN 77209374 AU Vogelstein-B. Kowarski-A. Lietman-P-S. TI The pharmacokinetics of amikacin in children. SO J-Pediatr. 1977 Aug. 91(2). P 333-9. MJ AMIKACIN: bl. KANAMYCIN: aa. METABOLIC-CLEARANCE-RATE. MN ADNEXITIS: bl. ADOLESCENCE. AMIKACIN: ad. ANEMIA-APLASTIC: bl. APPENDICITIS: bl. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. HALF-LIFE. HUMAN. KIDNEY: me. LEUKEMIA: bl. MALE. NEOPLASMS: bl. NEUTROPENIA: bl. SUPPORT-U-S-GOVT-P-H-S. AB The pharmacokinetic variables of amikacin were measured in 20 children and adolescents, four to 16 years of age. The mean plasma clearance of amikacin was 120 ml/minute/1.73 m2. The major route of elimination was renal (82%). Simultaneously measured inulin clearance showed that a majority (64%) of the drug filtered by the kidneys was excreted in the urine. The mean volume of distribution of amikacin was 32% of body weight; half-life during the final (pseudoequilibrium) phase averaged 1.6 hours. No significant accumulation of the drug was seen when four doses were given intravenously at 420 mg/m2 every 8 hours; the plasma concentration just before injection of the fourth dose averaged 2.4 plus or minus 1.1 microgram/ml. The mean plasma concentration at 60 to 75 minutes after injection of the drug was 28.7 microg/ml with little interpatient variation (SD 3.5 microg/ml). When the dose was expressed in terms of milligrams per kilogram, it was found that children often needed 50 to 100% more of the drug, compared to adults, to achieve equivalent plasma concentrations; this larger requirement was primarily due to a higher clearance of amikacin in proportion to body weight. Dosages based on surface area, however, resulted in uniform requirements and predictable plasma concentrations in all patients studied. RF 001 WOLF SM N ENGL J MED 291 733 974 003 UMEZAWA A J ANTIBIOT (TOKYO) 24 274 971 004 SMITH CR N ENGL J MED 296 349 977 005 SHARP PM ANTIMICROB AGENTS CHEMOTHER 5 435 974 006 TALLY FP ANN INTERN MED 83 484 975 007 MEYER RD ANN INTERN MED 83 790 975 008 VALDIVIESCO M AM J MED SCI 270 453 975 009 PRICE KE ANTIMICROB AGENTS CHEMOTHER 5 143 974 010 RIES K ANTIMICROB AGENTS CHEMOTHER 3 552 973 011 HOLMES SW PROC ICAAC 12TH 7 972 012 PRICE KE J ANTIBIOT (TOKYO) 25 709 972 013 CLARKE JT CLIN PHARMACOL THER 15 610 974 014 CABANA BE ANTIMICROB AGENTS CHEMOTHER 3 478 973 015 BODEY GP ANTIMICROB AGENTS CHEMOTHER 5 508 974 016 HOWARD JB ANTIMICROB AGENTS CHEMOTHER 8 86 975 017 SIBER GR J INFECT DIS 132 637 975 018 NUNNERY AW J INFECT DIS 119 402 969 019 NAGASHIMA R J PHARM SCI 57 1888 968 020 GIBALDI M J PHARM SCI 58 193 969 021 KOWARSKI A J CLIN ENDOCRINOL METAB 32 356 971 023 SMITH HW PRINCIPLES OF RENAL PHYSIOLOG 196 956 024 COLE BR N ENGL J MED 287 1109 972 025 HAAS MJ ANTIMICROB AGENTS CHEMOTHER 4 497 973 026 BOJENSEN E ACTA MED SCAND SUPPL 266 275 952 027 DEAN RFA J PHYSIOL (LOND) 109 81 947 028 AGUSTA VE J UROL 110 113 973 029 GOMBOS EA ANTIMICROB AGENTS CHEMOTHER 4 373 964 030 PRESTON FW ANTIBIOT ANN 7 857 960 031 WYAN JB ARCH SURG 98 381 969 032 GRYSELYNCK AM J INFECT DIS SUPPL 124 70 971 033 JUSKO WJ J PHARM SCI 61 1270 972 034 LAUGHMAN PM J PEDIATR 88 869 976 035 SCHMIDT-NIELSEN K FED PROC 29 1524 970 036 SHIRKEY HC IN: SHIRKEY HC 22 975 CT 1 MCCRACKEN GH J PEDIATR 91 358 977 2 RODIERE M NOUV PRESSE MED 8 3479 979 3 KOWARSKI CR DRUG INTEL CLIN PHARM 13 782 979 4 KOWARSKI CR DRUG INTEL CLIN PHARM 13 409 979 5 CLEARY TG ANTIMICROB AGENTS CHEMOTHER 16 829 979 6 KRAMER WG CLIN PHARMACOL THER 26 635 979 7 KAFETZIS DA ACTA PAEDIATR SCAND 68 419 979 8 FINKELSTEIN E J PEDIATR 94 163 979 9 CHRYMKO M AM J HOSP PHARM 37 713 980 10 LANAO JM KIDNEY INT 20 115 981 11 KAUFFMAN RE PEDIATR CLIN NORTH AM 28 35 981 12 PIEN FD AM J HOSP PHARM 38 981 981 13 PHILIPS JB SEM PERINATOL 6 166 982 14 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 15 LANAO JM EUR J CLIN PHARMACOL 23 155 982 16 MEISTAS MT DIABETES 31 449 982 17 KELLY HB J PEDIATR 100 318 982 18 KUMOR KM DEVELOP PHARM THER 7 368 984 19 NAHATA MC DEVELOP PHARM THER 7 221 984 20 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 21 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 22 LEEDER JS CLIN PHARMACOL THER 36 355 984 23 COLANGELO PM AM J HOSP PHARM 41 2650 984 24 MISCHLER EH SEM RESPIR MED 6 271 985 25 HORREVORTS AM CHEST 88 260 985 26 HALL JW INT J PEDIATR OTORHINOLARYNGO 12 187 986 27 AUTRET E EUR J CLIN PHARMACOL 31 79 986 28 KEARNS GL J PEDIATR 108 847 986 29 HORNER GW DRUG INTEL CLIN PHARM 21 276 987 PN 77106 RN 00687 AN 77209359 AU Herrod-H-G. Spock-A. TI Mother and daughter with cystic fibrosis. SO J-Pediatr. 1977 Aug. 91(2). P 276-7. MJ CYSTIC-FIBROSIS: fg. MN ADULT. CASE-REPORT. CHLORIDES: an. CYSTIC-FIBROSIS: di. FEMALE. FOLLOW-UP-STUDIES. HUMAN. INFANT. INFANT-NEWBORN. PREGNANCY. RESPIRATORY-FUNCTION-TESTS. SWEAT: an. EX With improved therapy and recognition of patients with mild disease, an increasing number of children with cystic fibrosis are reaching the reproductive years. Although virtually all males with cystic fibrosis are sterile, an unknown percentage of females with cystic fibrosis will be fertile. We know of only one case in which a mother with cystic fibrosis produced a child with the same disease. The purpose of this communication is to report the birth of a child with cystic fibrosis whose mother also has the disease. RF 001 TAUSSIG LM N ENGL J MED 287 586 972 002 HILLMAN B GAP CONF REP PROB REPRO PHYSI 2 975 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 005 SIEGEL B OBSTET GYNECOL 16 438 960 006 GRAND RJ JAMA 195 993 966 007 LARSEN JW OBSTET GYNECOL 39 880 972 008 BOYLE IR J PEDIATR 88 318 976 CT 1 OPITZ JM POSTGRAD MED 66 129 979 2 SANTAGNESE PAD AM J MED 66 121 979 3 WOOD RE SOUTH MED J 72 189 979 4 HERROD HG J PEDIATR 94 676 979 5 COHEN LF LANCET 2 842 980 6 JACOBS WH AM J GASTROENTEROL 76 342 981 7 LIMACHER F SCHWEIZ MED WOCHENSCHR 112 264 982 8 JOHNSON SR OBSTET GYNECOL 61 S 2 983 9 PALMER J ANN INTERN MED 99 596 983 PN 77107 RN 00688 AN 77073636 AU Nouisa-Arvanitakis-S. Stapleton-F-B. Linshaw-M-A. Kennedy-J. TI Therapeutic approach to pancreatic extract-induced hyperuricosuria in cystic fibrosis. SO J-Pediatr. 1977 Feb. 90(2). P 302-5. MJ CYSTIC-FIBROSIS: th. PANCREATIC-EXTRACTS: tu. URIC-ACID: ur. MN BODY-WEIGHT. CHILD. CYSTIC-FIBROSIS: dt. DIET-THERAPY. DIETARY-CARBOHYDRATES. DIETARY-FATS. DIETARY-PROTEINS. DOSE-RESPONSE-RELATIONSHIP-DRUG. HUMAN. KIDNEY-DISEASES: ci. NUTRITIONAL-REQUIREMENTS. TRIGLYCERIDES: tu. AB The relationship between the dosage of pancreatic extract and the excretion of uric acid was investigated in 29 patients with cystic fibrosis and exocrine pancreatic insufficiency. Urinary excretion of uric acid was normal in patients receiving small doses of pancreatic extracts and abnormally high in those receiving large amounts. In the latter group, normouricosuria was achieved by reducing the dose of pancreatic extract. Normal stool patterns and adequate weight gains were preserved by a diet modification that was well accepted by the patients. To eliminate the potential renal consequences of hyperuricosuria, it seems appropriate to control the need for increasing amounts of pancreatic enzymes by limiting the dietary intake of fat and maintaining a positive caloric and nitrogen balance with high intake of protein and carbohydrates and supplementation with medium-chain triglycerides. RF 001 LITTMAN A N ENGL J MED 281 201 969 002 SHWACHMAN H IN: SHIRKEY HC 651 975 003 HARRIS R ARCH DIS CHILD 30 424 955 004 STAPLETON FB N ENGL J MED 295 246 976 005 EMMERSON B KIDNEY INT 8 65 975 006 TAUSSIG LM J PEDIATR 82 380 973 007 HENRY RJ CLINICAL CHEMISTRY PRINCIPLES 283 964 008 SEEGMILLER JE IN: COHEN AS 216 967 009 MICHENER WM AM J DIS CHILD 113 195 967 010 GUTMAN AB TRANS ASSOC AM PHYSICIANS 74 353 961 011 HOLMES EW KIDNEY INT 2 115 972 012 ROBSON AM J PEDIATR 79 42 971 013 FAREBROTHER DA CLIN NEPHROL 4 243 975 014 SPENCER HW KIDNEY INT 9 489 976 015 GRACEY M ARCH DIS CHILD 45 445 970 CT 1 GAHL WA N ENGL J MED 297 1349 977 2 GAHL WA N ENGL J MED 297 1349 977 3 DAVIDSON GP J PEDIATR 93 976 978 4 SANTAGNESE PAD AM J MED 66 121 979 5 GAHL WA J PEDIATR 95 330 979 6 SACK J ISR J MED SCI 16 417 980 7 NIESSEN KH MONATSSCHR KINDERHEILKD 128 301 980 8 JACOBS WH AM J GASTROENTEROL 76 342 981 9 PARK RW GASTROENTEROLOGY 81 1143 981 10 NASSIF EG J PEDIATR 98 320 981 11 NIESSEN KH J PEDIATR GASTROENTEROL NUTR 1 349 982 12 HUBBARD VS J AM DIET ASSOC 80 127 982 13 SORKIN EM DRUG INTEL CLIN PHARM 17 110 983 14 NIESSEN KH EUR J PEDIATR 141 23 983 15 LANKISCH PG CLIN GASTROENTEROL 13 985 984 16 PERRY RS CLIN PHARMACY 4 161 985 17 ZENTLERMUNRO PL GUT 26 892 985 18 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 19 PHILLIPS BM J ROY SOC MED 79 44 986 PN 77108 RN 00689 AN 78007533 AU Lyrene-R-K. Polhill-R-B-Jr. Guthrie-L-A. Tiller-R-E. TI Alternative complement pathway activity in cystic fibrosis [letter]. SO J-Pediatr. 1977 Oct. 91(4). P 681-2. MJ COMPLEMENT: me. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. MALE. SUPPORT-U-S-GOVT-P-H-S. EX The possibility has been raised that complement plays a role in the pathogenesis of cystic fibrosis (CF). This impairment would be consistent with the thesis that the alternative pathway had been activated in vivo and, therefore, depleted. The purpose of this work is to explore further the function of the alternative pathway in cystic fibrosis. Examination of the data using Student's t test and analysis of variance showed no significant difference in the mean hemolytic activity of sera of CF patients as compared to the control group. The data presented do not support the work of Polley and Bearn who demonstrated an abnormality in CF patients using conversion of C3 by inulin as assay. The reason for this discrepancy is not clear. Our results do not preclude in vivo activation of the alternative pathway, since a compensatory increase in synthesis of components could offset component consumption. Since the net result is a completely normal level of activity, however, it does not seem likely that significant consumption has occurred. RF 001 ANON J PEDIATR 88 711 976 002 HOLZHAUER RJ PEDIATR RES 10 365 976 003 HANN S LANCET 2 520 974 004 POLLEY MJ J MED GENET 11 249 974 006 SCHREIBER RD J EXP MED 144 1062 976 CT 1 BUESCHER ES J PEDIATR 93 530 978 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 PAEDIATR SCAND SUPPL 301 1982 55 982 6 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 7 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 PN 77109 RN 00690 AN 78047782 AU Rosenstein-B-J. Oppenheimer-E-H. TI Prolonged obstructive jaundice and giant cell hepatitis in an infant with cystic fibrosis [letter]. SO J-Pediatr. 1977 Dec. 91(6). P 1022-3. MJ CYSTIC-FIBROSIS: co. HEPATITIS: co. CHOLESTASIS: et. MN AUTOPSY. CASE-REPORT. CYSTIC-FIBROSIS: pa. HEPATITIS: pa. HUMAN. INFANT. CHOLESTASIS: pa. MALE. EX Histologic evidence of liver involvement, consisting of focal biliary cirrhosis and/or intrahepatic mucus, is a frequent autopsy finding in infants with cystic fibrosis (CF). We wish to report an infant with CF in which liver biopsy showed features typical of neonatal giant cell hepatitis. In our patient, none of the pathologic features usually described in CF were present in either of the biopsies. Rather, the changes were those described in neonatal hepatitis and, to our knowledge, have not been reported in association with CF. It is likely that the giant cell hepatitis was a coincidental finding, unrelated to CF. It emphasizes, however, that one cannot assume a uniform etiology for all instances of obstructive jaundice seen in infants with CF. RF 001 OPPENHEIMER EH J PEDIATR 86 683 975 002 VALMAN HB ARCH DIS CHILD 46 805 971 003 OPPENHEIMER EH LANCET 2 1031 973 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 3 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 PN 77110 RN 00691 AN 77167592 AU Lungren-D-W. Hankins-J-M. Ulane-M-M. Willison-J-W. TI Putrescine uptake in cystic fibrosis fibroblasts [letter]. SO J-Pediatr. 1977 Jun. 90(6). P 1034-5. MJ CYSTIC-FIBROSIS: me. FIBROBLASTS: me. PUTRESCINE: me. MN CELL-LINE. CELLS-CULTURED. CYSTIC-FIBROSIS: pa. HUMAN. KINETICS. EX Our laboratory has reported abnormal polyamine levels in extracted whole blood from individuals with cystic fibrosis (CF). Since an abnormality in putrescine uptake in CF fibroblasts would imply a direct relationship to the genetic lesion, we initiated studies to confirm and possible extend this observation. There is no significant increase in the Vmax of CF fibroblasts as compared to values obtained for control cells. We conclude that with our growth and assay conditions, in which we attempted to duplicate those described in the original investigation, there is no consistent or significant increase in the Vmax of putrescine uptake in CF fibroblasts. RF 001 DEBUSK AG PROC INT CF CONG 7TH 12 976 002 DEBUSK AG GAP CONF REP POLYAMINES 13 976 003 LUNDGREN DW CLIN CHIM ACTA 62 357 975 004 COHEN LF BLOOD 48 469 976 005 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 CT 1 GAHL WA IN VITRO 15 252 979 PN 77111 RN 00692 AN 77073645 AU Heffer-E-T. TI Cystic fibrosis in black children. SO J-Pediatr. 1977 Feb. 90(2). P 324-5. MJ BLACKS. CYSTIC-FIBROSIS: oc. MN HUMAN. MALE. NEW-YORK-CITY. EX We feel that cystic fibrosis in the black population occurs more frequently than 1/17,000 as reported by Kulcycki and Schauf. Our experience with black patients does not indicate that their course and prognosis is different from that in the white population with cystic fibrosis. 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 CT 1 KULCZYCKI LL J PEDIATR 92 855 978 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 KARJOO M J TROP PEDIATR 30 195 984 PN 77112 RN 00693 AN 77073637 AU Finberg-L. TI Pancreatic extracts in the management of cystic fibrosis. SO J-Pediatr. 1977 Feb. 90(2). P 306. MJ CYSTIC-FIBROSIS: dt. PANCREATIC-EXTRACTS: ad. MN HUMAN. KIDNEY-DISEASES: ci. PANCREATIC-EXTRACTS: ae. URIC-ACID: ur. EX The University of Kansas group have established that pancreatic extracts, in the doses currently used in the management of patients with cystic fibrosis, cause hyperuricosuria which may occasionally be symptomatic. They correctly worry about long-term ill effects of such therapy. In treated CF patients the uricosuria results primarily from the increased dietary purine load, though the abnormal transport physiology in CF may contribute. Even though final evidence on risk is not at hand, prudence would suggest holding the purine load in CF patients to the minimum needed to ensure digestion of sufficient foodstuffs without necessarily permitting a "normal" diet sans steatorrhea. Alternatively, purified enzyme preparations free of the remaining sweetbread could eliminate the excess nucleic acid intake and be administered in a dosage permitting the luxury of redundant safety. RF 001 STAPLETON FB N ENGL J MED 295 246 976 PN 77113 RN 00694 AN 78007587 AU Schiffer-M. Lynch-R. TI Inappropriate secretion of ADH as a cause of hyponatremia in cystic fibrosis [letter]. SO J-Pediatr. 1977 Nov. 91(5). P 850-1. MJ CYSTIC-FIBROSIS: co. HYPONATREMIA: et. VASOPRESSINS: se. MN BLOOD-VOLUME. BODY-WEIGHT. HUMAN. HYPONATREMIA: pp. EX After reading "The syndrome of inappropriate antidiuretic hormone [ADH] secretion [SIADH] as a cause of hyponatremia in cystic fibrosis [CF]," we are puzzled by certain unusual phenomena in the two cases presented. We agree that it would be important to recognize the occurrence of ADH mediated hyponatremia in CF. We would, though, appreciate the authors' comments on the weight losses and the fluid and electrolyte balances early in these patients's courses. Also helpful would be information on furosemide dosage and any other drugs, such as acetaminophen, which may have been given so we can further understand the pathophysiology of these two complex patients. - We do not think there was hypovolemia in either case. Patient 1 was given only one dose of 600 mg of acetaminophen on the fourth day of hospitalization during recovery. Patient 2 received only one dose of acetaminophen (650 mg) at 2 AM on the twenty-first day of admission after recovery. Both patients were receiving antibiotics and Patient 2 was receiving digitalis as well. The reason for production of ADH remains obscure as it is in most cases of SIADH, although it was mentioned in the discussion that the transient nature of SIADH in these patients with cystic fibrosis would argue against the production of this hormone in the lung. RF 001 COHEN LF J PEDIATR 90 574 977 002 NOLPH KD AM J MED 49 534 970 003 STROBER W PEDIATRICS 43 416 969 004 NUSYNOWITZ ML AM J MED SCI 252 429 966 CT 1 ZERBE R ANNU REV MED 31 315 980 PN 77114 RN 00695 AN 77167531 AU Lippe-B-M. Sperling-M-A. Dooley-R-R. TI Pancreatic alpha and beta cell functions in cystic fibrosis. SO J-Pediatr. 1977 May. 90(5). P 751-5. MJ CYSTIC-FIBROSIS: pp. ISLANDS-OF-LANGERHANS: pp. MN ADOLESCENCE. ADULT. ARGININE: du. CHILD. CYSTIC-FIBROSIS: co. FEMALE. GLUCAGON: se. GLUCOSE-TOLERANCE-TEST. HUMAN. INSULIN: se. MALE. PREDIABETIC-STATE: co. SUPPORT-U-S-GOVT-P-H-S. AB Insulin and glucagon secretions were studied during oral glucose tolerance testing and arginine infusion in 13 patients with cystic fibrosis. Two groups of patients were identified; Group I (N=6) whose OGTT was entirely normal and Group II (N=7) who had some abnormality in glucose during OGTT. In each group basal glucagon concentrations were normal and supressed appropriately (p less than 0.05) after glucose; insulin responses were attenuated and the peak responses delayed. During arginine stimulation, insulin secretion was impaired in each group. However, glucagon secretion was diminished only in Group II. Thus, insulinopenia was found in both groups and hyperglucagonemia was not found as a contributory factor to the hyperglycemia in Group II. RF 001 HANDWERGER S N ENGL J MED 281 451 969 002 ROSAN RC AM J DIS CHILD 104 625 962 003 WILMSHURST EG PEDIATRICS 55 75 975 004 CHAZAN BI J PEDIATR 77 86 970 005 UNGER RH N ENGL J MED 285 443 971 006 MULLER WA AM J MED 54 52 973 007 GERICH JE N ENGL J MED 292 985 975 008 RASKIN P J CLIN INVEST 56 1132 975 009 SHERWIN RS N ENGL J MED 294 455 976 010 LEVINE R N ENGL J MED 294 494 976 011 STAHL M J PEDIATR 84 821 974 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 MORGAN CR DIABETES 12 115 963 014 SPERLING MA CLIN CHEM 20 566 974 015 SELTZER HS IN: FAJANS SS 101 971 016 SAVAGE PJ DIABETES 24 362 975 017 RUSHFORTH NB DIABETES 24 538 975 018 DRASH A PEDIATR RES 2 94 968 019 PERLEY MJ J CLIN INVEST 46 1954 967 020 PERLEY MJ DIABETES 15 867 966 021 KALK WJ DIABETES 23 257 974 022 KALK WJ HORM METAB RES 6 95 974 023 VINIK AI LANCET 1 485 974 024 ARONOFF SL DIABETES 25 404 976 025 BARNES AJ LANCET 1 219 976 CT 1 ROSS SA DIABETES 27 501 978 2 HASCHKE F HELV PAEDIATR ACTA 33 385 978 3 NELSON RL J CLIN ENDOCRINOL METAB 49 412 979 4 SANTAGNESE PAD AM J MED 66 121 979 5 WOOD RE SOUTH MED J 72 189 979 6 LIPPE BM PEDIATRICS 65 1018 980 7 ADRIAN TE GASTROENTEROLOGY 79 460 980 8 ROSS SA PEDIATRICS 67 252 981 9 BERG U ACTA PAEDIATR SCAND 71 833 982 10 BISTRITZER T ISR J MED SCI 19 600 983 11 DANDONA P J CLIN PATHOL 36 790 983 12 IANNUCCI A HUM PATHOL 15 278 984 13 GEFFNER ME PEDIATR RES 18 1107 984 14 GEFFNER ME AM J DIS CHILD 138 677 984 15 MOHAN V DIABETE METAB 11 376 985 16 DESANCTIS V POSTGRAD MED J 61 963 985 17 KNOPFLE G KLIN PAEDIATR 197 13 985 18 DAVIS TME EUR J CLIN INVEST 17 12 987 PN 77115 RN 00696 AN 77073569 AU Parry-M-F. Neu-H-C. Merlino-M. Gaerlan-P-F. Ores-C-N. Denning-C-R. TI Treatment of pulmonary infections in patients with cystic fibrosis: a comparative study of ticarcillin and gentamicin. SO J-Pediatr. 1977 Jan. 90(1). P 144-8. MJ CYSTIC-FIBROSIS: co. GENTAMICINS: tu. PENICILLINS: tu. PNEUMONIA: dt. PSEUDOMONAS-INFECTIONS: dt. TICARCILLIN: tu. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. COMPARATIVE-STUDY. FEMALE. GENTAMICINS: ae. HUMAN. MALE. PNEUMONIA: co. PSEUDOMONAS-AERUGINOSA: de. PSEUDOMONAS-INFECTIONS: co. TICARCILLIN: ae. AB The effectiveness of ticarcillin against Pseudomonas aeruginosa in acute exacerbations of pulmonary infection in patients with cystic fibrosis was evaluated. Seventy-one percent of patients treated with ticarcillin alone responded favorably. The response rate was similar in patients treated with a combination of ticarcillin plus gentamicin or with gentamicin alone. Severity of the underlying disease was the most important determinant of response to treatment. Ticarcillin- resistant organisms were recovered during treatment in 50% of patients who received this drug; recovery of them was not prevented by the inclusion of gentamicin in the therapeutic regimen nor did they interfere with clinical improvement. The ticarcillin-resistant strains persisted at follow-up, two to six months after completion of therapy, in only one of ten patients. No serious toxicity to ticarcillin was noted during the study period. RF 001 MEARNS MB ARCH DIS CHILD 47 902 972 002 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 003 MAY JR ARCH DIS CHILD 47 908 972 004 ACRED P NATURE 215 25 967 005 HUANG NN J PEDIATR 78 338 971 006 PHAIR JP AM J DIS CHILD 120 22 970 007 MARKS MI J PEDIATR 79 822 971 008 BOXERBAUM B J INFECT DIS SUPPL 122 59 970 009 SWARZ H ADV MANAG PSEUDOMON PROT INF 27 970 010 NEU HC ANTIMICROB AGENTS CHEMOTHER 10 385 970 011 HUGH R IN: BLAIR JE 175 970 012 EDWARDS PR IDENTIFICATION OF ENTEROBACTE 972 013 BAUER AW AM J CLIN PATHOL 45 493 966 014 NEU HC APPL MICROBIOL 21 66 971 015 NEU HC J GEN MICROBIOL 58 301 969 016 SHWACHMAN H AM J DIS CHILD 96 6 958 017 JACK GW J GEN MICROBIOL 61 43 970 018 LOWBURY EJL LANCET 2 448 969 CT 1 PETERSON CD DRUG INTEL CLIN PHARM 11 482 977 2 PICKERING LK SOUTH MED J 70 1215 977 3 FEDER HM J PEDIATR 91 354 977 4 PARRY MF AM J MED 64 961 978 5 APPEL GB ANN INTERN MED 89 528 978 6 CHOW M CONN MED 43 25 979 7 NEU HC INFECTION 8 S 62 980 8 BROGDEN RN DRUGS 20 325 980 9 MARTIN AJ ARCH DIS CHILD 55 604 980 10 BEAUDRY PH J PEDIATR 97 144 980 11 MARKS MI J PEDIATR 98 173 981 12 HYATT AC J PEDIATR 99 307 981 13 MALMBORG AS SCAND J INFECT DIS 1981 64 981 14 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 15 SCRIBNER RK ANTIMICROB AGENTS CHEMOTHER 21 939 982 16 BROWN LA J ALLERGY CLIN IMMUNOL 69 51 982 17 REDDING GJ AM REV RESPIR DIS 126 31 982 18 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 19 BODEY GP REV INFECT DIS 5 279 983 20 MCLAUGHLIN FJ J ANTIMICROB CHEMOTHER 11 195 983 21 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 22 SHLAES DM PEDIATR CLIN NORTH AM 30 121 983 23 PENKETH ARL BR J DIS CHEST 77 179 983 24 MCLAUGHLIN FJ J INFECT DIS 147 559 983 25 ARAVIND MK J LIQUID CHROMATOGR 7 2887 984 26 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 27 NICHOLS L CHEMIOTERAPIA 4 102 985 28 ACAR JF REV INFECT DIS 7 S513 985 29 LAFERRIERE C CHEMOTHERAPY 31 255 985 30 JACOBS RF J PEDIATR 106 1001 985 31 NELSON JD J PEDIATR 106 1030 985 32 WILSON CB J PEDIATR 106 1049 985 33 TABLAN OC J PEDIATR 107 382 985 34 SCHAAD UB ACTA PAEDIATR SCAND 75 128 986 35 PITT TL J ROY SOC MED 79 13 986 36 SMITH AL J PEDIATR 108 866 986 37 CHANDRASEKAR PH J ANTIMICROB CHEMOTHER 19 321 987 PN 77116 RN 00697 AN 77251783 AU Desai-N. Nousia-Arvanitakis-S. TI False negative meconium test results in screening for cystic fibrosis. SO J-Pediatr. 1977 Sep. 91(3). P 447-8. MJ CYSTIC-FIBROSIS: di. MASS-SCREENING: st. MECONIUM. MN CASE-REPORT. FALSE-NEGATIVE-REACTIONS. HUMAN. INFANT. INFANT-NEWBORN. MALE. MECONIUM: me. PANCREAS: en. PROTEINS: me. EX The value of the Boehringer-Mannheim (B-M) meconium test for routine screening for cystic fibrosis has been questioned recently because of the high proportion of false negative results. This report presents our observations on the validity of the B-M meconium test. Our findings show that the B-M meconium test may contribute to false negative results even when the exocrine pancreatic function of a newborn infant with CF is abnormal. Our observation supports the doubts expressed recently in regard to the validity of the B-M meconium test in screening neonates for cystic fibrosis. RF 001 ANON J PEDIATR 88 711 976 002 IMONDI AR ANAL BIOCHEM 54 199 973 003 PROSSER R ARCH DIS CHILD 49 597 974 004 ROY CC PEDIATR CLIN GASTROENTEROL 975 CT 1 ARVANITAKIS C GASTROENTEROLOGY 74 932 978 2 UXA F MINERVA PEDIATR 31 831 979 3 BARRY MM J AM DIET ASSOC 75 446 979 4 PARK RW GASTROENTEROLOGY 81 1143 981 5 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 PN 77117 RN 00698 AN 78068479 AU Schuster-S-R. Fellows-K-E. TI Management of major hemoptysis in patients with cystic fibrosis. SO J-Pediatr-Surg. 1977 Dec. 12(6). P 889-96. MJ CYSTIC-FIBROSIS: co. EMBOLIZATION-THERAPEUTIC. HEMOPTYSIS: th. MN ADOLESCENCE. ADULT. BRONCHIAL-ARTERIES: ra, su. CHILD. CYSTIC-FIBROSIS: ra. FEMALE. GELATIN-SPONGE-ABSORBABLE: tu. HEMOPTYSIS: di, et. HEMORRHAGE: di. HUMAN. MALE. AB Although bronchoscopy remains the best definitive method for localization of the site of hemorrhage in patients who have massive hemopytsis, angiography remains an important adjunct to this localization. When combined with embolization of the bleeding bronchial artery, it is an effective method of therapy for the management of massive hemoptysis in patients with cystic fibrosis. RF 001 HOLSCLAW DS J PEDIATR 76 829 970 002 FELLOWS KE RADIOLOGY 114 551 975 003 NEWTON TH RADIOLOGY 84 1043 965 004 NORTH LB AM J ROENTG RAD THER NUCL MED 107 328 969 005 FEIGELSON HH RADIOLOGY 85 663 965 006 LIEBOW AA AM J PATHOL 25 211 949 007 MARCHAND P THORAX 5 207 950 008 WOOD DA J THORAC SURG 7 649 938 009 BOTENGA ASJ AM J ROENTG RAD THER NUCL MED 104 829 968 010 WENTWORTH P THORAX 23 582 968 011 LIEBOW AA YALE J BIOL MED 22 637 950 012 ROOSENBURG JG DIS CHEST 26 664 954 013 REMY J RADIOLOGY 122 33 977 CT 1 FILSTON HC J PEDIATR SURG 14 276 979 2 FUCHS WA RADIOLOGE 19 505 979 3 MACERLEAN DP BR J RADIOL 52 558 979 4 FELLOWS KE J PEDIATR 95 959 979 5 FAIRFAX AJ BR J DIS CHEST 74 345 980 6 TOM LWC ANN OTOL RHINOL LARYNGOL 89 419 980 7 JANIK JS PEDIATRICS 67 671 981 8 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 9 MARMON L J PEDIATR SURG 18 811 983 10 STEINHERZ PG MED PEDIATR ONCOL 12 28 984 11 HOFFER FA PEDIATR CLIN NORTH AM 32 1461 985 12 TRENTO A ANN THORAC SURG 39 254 985 13 STANKIEWICZ JA EAR NOSE THROAT J 64 180 985 PN 77118 RN 00699 AN 77143411 AU McKenzie-S-A. Godfrey-S. Singh-M-P. TI A new method for investigating regional lung function in children with localized lung disease. SO J-Pediatr-Surg. 1977 Apr. 12(2). P 177-81. MJ LUNG-DISEASES: di. RESPIRATORY-FUNCTION-TESTS: mt. MN ASTHMA: di. BRONCHIECTASIS: di. CASE-REPORT. CHILD. CYSTIC-FIBROSIS: di. CYSTS: di. FOREIGN-BODIES: di. HUMAN. INFANT. INFANT-NEWBORN. MALE. NITROGEN-RADIOISOTOPES: du. PNEUMONIA: di. PULMONARY-EMPHYSEMA: di. AB Lung function studies using 13Nitrogen have been used to determine regional and total lung function in 16 children suspected of having localized lung disease amenable to surgery. This method is simple, requires no active co-operation on the part of the child and gives a radiation dosage of about one-third of that for a bronchogram. The studies provided information about the severity and localization of the disease, and in addition, information about the remaining lung areas. RF 001 BALL WC JR J CLIN INVEST 41 519 962 002 RONCHETTI R ARCH DIS CHILD 50 595 975 CT 1 HENRIKSEN O J NUCL MED 21 333 980 PN 77119 RN 00700 AN 77143415 AU Schuster-S-R. Shwachman-H. Toyama-W-M. Rubino-A. Taik-Khaw-K. TI The management of portal hypertension in cystic fibrosis. SO J-Pediatr-Surg. 1977 Apr. 12(2). P 201-6. MJ CYSTIC-FIBROSIS: co. HYPERTENSION-PORTAL: su. MN CHILD. CYSTIC-FIBROSIS: mo. HUMAN. POSTOPERATIVE-CARE. POSTOPERATIVE-COMPLICATIONS: mo. PREOPERATIVE-CARE. AB We have found that with proper selection and preoperative preparation, a major portosystemic shunt can be done with considerable safety in a majority of cystic fibrosis patients and thus provide them with significant palliation and improved quality of life. RF 001 SHWACHMAN H HOSP PRACT 9 143 974 002 CLATWORTHY HW IN: MUSTARD WT 777 969 003 DI SANTAGNESE PA PEDIATRICS 18 387 956 004 TYSON KRT J PEDIATR SURG 3 271 968 005 TANK ES ARCH SURG 98 451 969 006 RAFFENSBERGER JG ARCH SURG 105 249 972 007 VOORHEES AB SURGERY 58 540 965 008 DANIELSON GK JAMA 195 217 966 009 DI SANTAGNESE PA PEDIATRICS 24 313 959 010 WILLIS SDV PROC R SOC MED 53 320 960 011 TUCKER AS AM J ROENTG RAD THER NUCL MED 89 1048 963 012 SHWACHMAN H AM J DIS CHILD 96 6 958 013 OPPENHEIMER EH J PEDIATR 86 683 975 014 WEBSTER R ARCH DIS CHILD 28 343 953 015 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 CT 1 STARKEY BJ MONOGR PAEDIATR 10 12 979 2 REDMOND AOB PRACTITIONER 224 295 980 3 PSACHAROPOULOS HT LANCET 2 78 981 4 PARK RW GASTROENTEROLOGY 81 1143 981 5 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 6 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 7 MOWAT AP CLIN GASTROENTEROL 11 171 982 8 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 9 RICHARDSON VF ACTA PAEDIATR SCAND 73 75 984 10 HUBBARD VS SEM RESPIR MED 6 299 985 11 VALAYER J WORLD J SURG 9 258 985 12 BERNARD O CLIN GASTROENTEROL 14 33 985 13 BERNARD O ARCH FR PEDIATR 42 249 985 14 SCHRODER R SCHWEIZ MED WOCHENSCHR 115 828 985 15 PRICE JF J ROY SOC MED 79 10 986 16 TANNER MS J ROY SOC MED 79 38 986 PN 77120 RN 00701 AN 77230292 AU Goldberg-R-T. TI Rehabilitation research on disability: New horizons. SO J-Rehabil. 1977 Jul-Aug. 43(3). P 14-8. MJ CYSTIC-FIBROSIS: rh. FACIAL-INJURIES: rh. HANDICAPPED. LARYNGEAL-NEOPLASMS: rh. REHABILITATION. SPINAL-CORD-INJURIES: rh. MN ADOLESCENCE. ADULT. CHILD. HUMAN. RESEARCH. UNITED-STATES. AB This paper presents an overview of research on disability, and integrates rehabilitation research with the mainstream of scientific activity. Research on disability is an applied science which uses the general tools of scientific method in solving specific problems. Research on disability embraces a wide variety of physical, intellectual, and emotional conditions. The psychological aspects of physical disabilities are especially stressed in the adaptation to lost functions. Examples of research are given from spinal cord injury, end-stage renal disease, facial disfigurement, and cancer of the larynx. RF 001 BAER JA OPTICAL TO TACTILE IMAGE CONV 972 002 BOBO EC IN: STAUFFER ES 969 003 BORS E UROLOGICAL SURVEY 57 246 960 004 COLE T PARAPLEGIA 11 111 973 005 CONANT JB ARCH OTOLARYNGOL 88 80 968 006 DALRYMPLE GF DEVELOPMENT AND DEMONSTRATI 973 007 GOLDBERG RT J COUNSELING PSYCH 21 428 974 008 GOLDBERG RT SCAND J REHAB MED 6 65 974 009 GOLDBERG RT REHAB LIT 35 354 974 010 GOLDBERG RT SCAND J REHAB MED 7 1 975 011 GUTTMANN L PROC SYMP ROYAL COLL SURG EDI 963 012 HAMILTON LS SPINAL CORD INJURY IN FLORIDA 974 013 HOLZEL A PHYSIOTHERAPY 61 238 975 014 JACKSON RW PARAPLEGIA 10 50 972 015 KRAUS JF J CHRON DIS 28 471 975 016 MOONEY T SEXUAL OPTIONS FOR PARAPLEGIC 975 017 PEARMAN JW PARAPLEGIA 9 95 971 018 POPPER KR LOGIC OF SCIENTIFIC DISCOVERY 959 019 ROGERS BO FACIAL DISFIGUREMENT A REHABI 963 020 SAVELLE M SEEDS OF LIBERTY THE GENESIS 948 021 SCHEIN JD DEAF POPULATION OF THE US 974 022 SHARMAN GJ SPINAL CORD INJURY A REPORT T 970 023 TRUAX CB COUNSELING AND PSYCHOTHERAPY 966 024 TSUKERBERG JS COMP SERV NEEDS STUDY HEW REP 975 025 WILCOX NE STATE WIDE CENSUS SPINAL CORD 972 PN 77121 RN 00702 AN 78090696 AU Hunt-L-A. Summers-D-F. TI Glycosylation of VSV glycoprotein is similar in cystic fibrosis, heterozygous carrier, and normal human fibroblasts. SO J-Supramol-Struct. 1977. 7(2). P 213-21. MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: me. HEXOSYLTRANSFERASES: me. MN CELL-LINE. FIBROBLASTS: me. HETEROZYGOTE. HUMAN. MEMBRANE-PROTEINS: me. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. VESICULAR-STOMATITIS-VIRUS: me. VIRAL-PROTEINS: me. AB The single envelope glycoprotein of vesicular stomatitis virus was used as a specific probe of glycosyltransferase activities in fibroblasts from two cystic fibrosis patients, an obligate heterozygous carrier and a normal individual. Gel filtration of pronase-digested glycopeptides from both purified virions and infected cell-associated VSV glycoprotein which had been labeled with[3H] glucosamine did not reveal any significant differences in the glycosylation patterns between the different cell cultures. All 4 cell lines were apparently able to synthesize the mannose- and glucosamine- containing core structure and branch chains terminating in sialic acid which are characteristic of asparagine-linked carbohydrate side chains in cellular glycoproteins. Analysis of tryptic glycopeptides by anion-exchange chromotography indicated that the same 2 major sites on the virus polypeptide were recognized and glycosylated in all 4 VSV-infected cell cultures. These studies suggest that the basic biochemical defect(s) in cystic fibrosis is not an absence or deficiency in enzymes responsible for the biosynthesis of complex carbohydrate side chains. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 LOUISOT P CLIN CHIM ACTA 48 373 973 004 SINGER L CLIN BIOCHEM 7 146 974 005 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 006 HOWATSON AF VIROLOGY 16 466 962 007 ZEE YC J GEN VIROL 7 95 970 008 WAGNER RR J VIROL 10 1228 972 009 KLENK HD J VIROL 7 416 971 010 ATKINSON PH J MOL BIOL 102 613 976 011 HUNT LA J VIROL 20 637 976 012 HUNT LA J VIROL 20 646 976 013 ETCHISON JR VIROLOGY 60 217 974 014 ETCHISON JR PROC NAT ACAD SCI USA 71 4011 974 015 MOYER SA J VIROL 18 167 976 016 ROBERTSON JR J VIROL 19 871 976 017 ETCHISON JR VIROLOGY 78 375 977 018 MUDD JA VIROLOGY 42 328 970 019 GRUBMAN MJ J VIROL 12 265 973 020 BONNER WM EUR J BIOCHEM 46 83 974 021 DRAEMER PM J CELL PHYSIOL 69 199 967 022 SCHLESINGER S J VIROL 17 239 976 023 GOTTLIEB C J BIOL CHEM 251 7761 976 024 SLY WS PROC NAT ACAD SCI USA 73 2443 976 CT 1 HUNT LA PROC NAT ACAD SCI USA 75 754 978 2 HUNT LA J VIROL 35 362 980 3 MARGOLIES R PEDIATR RES 16 181 982 4 MARGOLIES R PEDIATR RES 17 931 983 PN 77122 RN 00703 AN 78049611 AU Luck-S-R. Raffensperger-J-G. Sullivan-H-J. Gibson-L-E. TI Management of pneumothorax in children with chronic pulmonary disease. SO J-Thorac-Cardiovasc-Surg. 1977 Dec. 74(6). P 834-9. MJ CYSTIC-FIBROSIS: co. PNEUMOTHORAX: th. MN ADOLESCENCE. ADULT. CASE-REPORT. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. FEMALE. HISTIOCYTOSIS-X: co. HUMAN. INFANT. INTUBATION. LUNG: su. MALE. PNEUMOTHORAX: et, ra. RECURRENCE. SCLEROSING-SOLUTIONS: tu. AB Cystic fibrosis is the most common form of chronic pulmonary disease in the pediatric age group. As these children survive longer with improved therapy, the incidence of pneumothorax has increased. In our patients who were more than 10 years of age, the incidence of pneumothorax was 12.5 percent. We have reviewed the records of 22 patients who have had 44 episodes of pneumothorax complicating cystic fibrosis. In addition, our experience includes one child with histiocytosis in whom pneumothorax was a serious complication. One other pneumothorax occurred in a teen-age girl with apical bullous disease. A child who presents with even a minimal pneumothorax complicating cystic fibrosis is at considerable risk of developing an increasing lung collapse. Even if the pneumothorax resolves, there is a high risk of recurrence. In children treated with only a chest tube there was a 50 percent recurrence. Consequently, we recommend a chest tube for any episode of pneumothorax in a child with cystic fibrosis, with installation of a sclerosing agent when the lung expands. Three of our patients so treated had no recurrence. Children who have a persistent air leak are confined to bed, retain secretions, and pursue a rapid downhill course. A more aggressive approach to these children, consisting of thoracotomy, suture of the air leak, pteural symphysis, and pulmonary lavage is feasible and the mortality rate is low. A pneumothorax in a child with no evidence of cystic fibrosis is a rare event. A persistent air leak is an indication for thoracotomy, suture of the air leak, and lung biopsy. Histiocytosis X is one rare cause for this complication in children. RF 001 STERN RC J PEDIATR 89 406 976 002 DI SANTAGNESE PA N ENGL J MED 277 1287 967 003 BODROSSIAN CWM HUM PATHOL 1 633 968 004 DI SANTAGNESE PA JAMA 172 2065 960 005 HOLSCLAW DS CLIN PEDIATR 9 346 970 006 SCHWARTZ EE AM J ROENTG RAD THER NUCL MED 122 708 974 007 LIFSCHITZ MI AM J DIS CHILD 116 633 968 008 BOAT TF JAMA 209 1498 969 009 STOWE SM AM REV RESPIR DIS 111 611 975 010 MITCHELL-HEGGS PF THORAX 25 165 970 011 KATTWINKEL J JAMA 226 557 973 012 WALLACH HW CHEST 68 510 975 013 CUNNINGHAM GJ THORAX 5 43 950 014 HEPPLESTON AG THORAX 11 77 956 015 ROLAND AS N ENGL J MED 270 73 964 016 GELFAND ET CAN MED ASSOC J 110 937 974 017 LAHEY ME J PEDIATR 87 179 975 018 LUCAYA J AM J DIS CHILD 121 289 971 CT 1 LARRIEU AJ ANN THORAC SURG 28 146 979 2 SANTAGNESE PAD AM J MED 66 121 979 3 DEVRIES WC SURG CLIN NORTH AM 60 851 980 4 OFOEGBU RO AM J SURG 140 679 980 5 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 6 PENKETH ARL THORAX 37 850 982 7 MCLAUGHLIN FJ J PEDIATR 100 863 982 8 SCHUSTER SR J PEDIATR SURG 18 492 983 9 SEGERER H KLIN PAEDIATR 196 44 984 10 JENKINSON SG CLIN CHEST MED 6 153 985 11 LESTER LA SEM RESPIR MED 6 285 985 12 TRIBBLE CG ANN SURG 204 677 986 PN 77123 RN 00704 AN 77210123 AU Hassani-M-A. TI Cystic fibrosis in Iraqi children. SO J-Trop-Pediatr. 1977 Jun. 23(3). P 136-7. MJ CYSTIC-FIBROSIS: oc. MN CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. IRAQ. MALE. EX Cystic fibrosis (C.F.) was firmly established as a single disease by Anderson in 1938 although previously case reports had appeared in the European literature. Subsequently C.F. has been described in many parts of the world. This is the first report of cystic fibrosis in Arab-Iraqi children. Seventeen cases of cystic fibrosis were diagnosed at Medical City Teaching College Hospital, University of Baghdad, between 1970 and 1976. In this study the sex-ratio was 2:1. This compared with the roughly equal ratio usually quoted. A possible explanation for this discrepancy is the preference given to boys in Iraqi families and medical attention is more likely to be sought on their behalf. RF 001 BENGOA JM WHO CHRONICLE 28 1 974 002 HARFOUCHE JK GROWTH AND ILLNESS PATTERNS O 966 003 JELLIFFE DB ASSESSMENT NUTRITIONAL STATUS 966 004 JELLIFFE DB WHO CHRONICLE 21 127 967 005 MCLAREN DS LANCET 2 146 972 006 MORLEY D BR MED J 4 85 974 007 PUYET JH AM J CLIN NUTR 13 147 963 008 SHAKIR A LANCET 2 143 972 009 WATERLOW JC BR MED J 4 88 974 010 WRAY JD J TROP PEDIATR 15 76 969 011 ANDERSEN DH AM J DIS CHILD 56 344 938 012 DANKS DM ANN HUM GENET 28 323 965 013 FANCONI G WIEN MED WOCHENSCHR 86 753 936 014 GIBSON LE PEDIATRICS 23 545 954 015 JOHN O TEXTBOOK OF PAEDIATRICS 1989 973 016 MARIA SALEM H DRISS LEB MED J 15 1 962 017 SALAM MZ ANN PEDIATR (PARIS) 190 252 958 018 NELSON WE TEXTBOOK OF PEDIATRICS 850 964 CT 1 KARJOO M J TROP PEDIATR 30 195 984 PN 77124 RN 00705 AN 78029248 AU Warwick-W-J. Pogue-R-E. TI Cystic fibrosis. An expanding challenge for internal medicine. SO JAMA. 1977 Nov 14. 238(20). P 2159-62. MJ CYSTIC-FIBROSIS: oc. MN ADULT. AGE-FACTORS. AGED. CYSTIC-FIBROSIS: mo. HUMAN. LIFE-EXPECTANCY. MIDDLE-AGE. MODELS-THEORETICAL. PROGNOSIS. UNITED-STATES. AB The number of adults with cystic fibrosis (CF) receiving care from the US Cystic Fibrosis Centers has been increasing at an average rate of about 200 patients each year. A model based on the continuation of this rate of increase predicts that almost 4,000 adults will be receiving care at the centers by the end of 1985. Four other models developed are based on continuation of a steady state with two variations: a 50% reduction in mortality and universal newborn screening. These models show the boundaries of an equilibrium population to be between 24,000 and 64,000 patients, with 7,000 to 18,000 adults. RF 001 POGUE RE MINN MED 52 1551 969 002 WARWICK WJ J CHRON DIS 28 609 975 003 WARWICK WJ J ASTHMA RES 5 277 968 004 SHWACHMAN H PROC INT CF CONG 7TH 90 976 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 HOWIE AD SCOTT MED J 24 193 979 3 STRAUSS GD AM J DIS CHILD 133 301 979 4 ALLAN JL MED J AUST 1 600 980 5 HOLSCLAW DS CLIN CHEST MED 1 407 980 6 SANDERS JS CHEST 77 226 980 7 WILLI UV AM J ROENTGENOL 134 1005 980 8 BELL L J CAN DIET ASSOC 42 62 981 9 JACOBS WH AM J GASTROENTEROL 76 342 981 10 MOSS RB J PEDIATR 99 215 981 11 EVENSEN SA ACTA MED SCAND 209 141 981 12 PETTY TL SEM RESPIR MED 3 263 982 13 JOHNSON SR OBSTET GYNECOL 61 S 2 983 14 MACPHERSON AIS SCOTT MED J 29 6 984 15 COWEN L PSYCHOSOM MED 46 363 984 16 COLE BNL J PARENT ENTERAL NUTR 11 205 987 PN 77125 RN 00706 AN 78048779 AU Hirao-Y. Homma-J-Y. Zierdt-C-H. TI Serotyping of Pseudomonas aeruginosa from patients with cystic fibrosis of the pancreas. SO Jpn-J-Exp-Med. 1977 Aug. 47(4). P 249-54. MJ CYSTIC-FIBROSIS: mi. PSEUDOMONAS-AERUGINOSA: im. MN AGGLUTINATION-TESTS: mt. COMPARATIVE-STUDY. HUMAN. SEROTYPING: mt. AB Serotyping of 30 mucoid strains isolated from cystic fibrosis patients was carried out by slide agglutination tests with both live and heat-killed cells and by tube agglutination test with heat-killed cells. Comparison of the results obtained by these 2 methods revealed that tube agglutination with heat-killed cells was the superior method. More than half the strains were found to be Homma's serotype 15 (group M in the new schema [2]). Slide agglutination with live cells did not give clear results: some strains showed occasionally positive or negative agglutinations against the same serotype serum. Changes in serotypes (groups in the new schema [2]) were found in some strains, although the number was very small. RF 001 HOMMA JY JAPAN J EXP MED 44 1 974 002 HOMMA JY JAPAN J EXP MED 46 329 976 003 YABUUCHI E JAPAN J EXP MED 46 393 976 004 ZIERDT CH J CLIN MICROBIOL 1 521 975 005 HOMMA JY JAPAN J EXP MED 40 347 970 006 SHIONOYA H JAPAN J EXP MED 47 185 977 007 KODAMA H JAPAN J EXP MED 46 383 976 008 KONO M JAPAN J EXP MED 47 1 977 009 HOMMA JY JAPAN J EXP MED 42 171 972 010 KAWAHARAJO K JAPAN J EXP MED 43 225 973 CT 1 HOMMA JY JAP J EXP MED 49 89 979 2 JAGGER KN J CLIN MICROBIOL 17 55 983 3 OGLE JW J INFECT DIS 155 119 987 PN 77126 RN 00707 AN 77145455 AU Emrich-H-M. Dahlheim-H. TI Renin-like (angiotensinogenase) activity in sweat of patients with cystic fibrosis and controls. SO Klin-Wochenschr. 1977 Mar 15. 55(6). P 291-2. MJ CYSTIC-FIBROSIS: en. RENIN: an. SWEAT: en. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. HUMAN. SWEAT-GLANDS: en. AB Using bioassay method (rat blood pressure technique) as well as the radioimmunoassay, renin-like activity (RLA) was measured in eccrine sweat of patients with cystic fibrosis of the pancreas (CF) and of controls. Sweat-formation was induced by pilocarpine-iontophoresis or by local injection of carbamylcholine (Doryl). RLA-values between O (not measurable) and 460 ng/ml.h were measured. With increasing sweat flow-rate a tendency to lower RLA-values was detected. No significant difference was observed between CF and controls. From the observation that RLA of sweat is up to 30 times higher than that of plasma, it is concluded that RLA is probably released not from plasma but from the sweat glands themselves, where it is stored or synthesized. RF 001 OCHWADT B PROC INT CONG NEPHROL STO 4TH 969 002 STEVEN K KIDNEY INT 6 73 974 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 EMRICH HM PEDIATR RES 2 464 968 005 LOBECK CC IN: STANBURY JB 1300 966 006 GIBSON LE PEDIATRICS 23 545 959 007 EMRICH HM PFLUEGERS ARCH 290 298 966 008 DAHLHEIM H PFLUEGERS ARCH 312 303 970 009 SCHUCHARD J VERH DTSCH GES INN MED BD 80 974 010 OPARIL S N ENGL J MED 291 389 974 CT 1 LIAPPIS N ARCH DERMATOL RES 261 281 978 2 KAMOUN P IRCS MED SCI BIOCHEM 11 605 983 3 QUINTON PM ANNU REV MED 34 429 983 PN 77127 RN 00708 AN 77231257 AU Heinrich-H-C. Gabbe-E-E. Bartels-H. Oppitz-K-H. Bender-Gotze-C. Pfau-A-A. TI Bioavailability of food iron-(59Fe), vitamin B12-(60Co) and protein bound selenomethionine-(75Se) in pancreatic exocrine insufficiency due to cystic fibrosis. SO Klin-Wochenschr. 1977 Jun 15. 55(12). P 595-601. MJ CYSTIC-FIBROSIS: me. IRON: me. SELENIUM: me. SELENOMETHIONINE: me. VITAMIN-B-12: me. MN BIOLOGICAL-AVAILABILITY. CHILD. CHILD-PRESCHOOL. COBALT-RADIOISOTOPES. DIETARY-PROTEINS: me. FOOD. HUMAN. INTESTINAL-ABSORPTION. INTRINSIC-FACTOR: pd. IRON-RADIOISOTOPES. LIVER. MEAT. PANCREATIN: pd. PROTEIN-BINDING. RADIOISOTOPES. VITAMIN-B-12-DEFICIENCY: et. WHOLE-BODY-COUNTING. AB The absorption of biosynthetically 59Fe-labeled hemiglobin, pork and hog liver, 60 Co-vitamin B12 and 75Se-selenomethionin labeled pork was measured in children with cystic fibrosis by whole body counting of the absorbed radionuclides within the 4-pi geometry of a large volume radioactivity detector with liquid organic scintillator. RF 001 AWWAD HK J BIOL CHEM 242 492 967 002 BEN PORATH M J NUCL MED 9 168 968 003 DEREN JJ N ENGL J MED 288 949 973 004 EVANS WB AM J DIG DIS 11 594 966 005 HEINRICH HC PROC UNIV NEW MEXICO CONF ORG 312 960 006 HEINRICH HC SEMIN HEMATOL 1 199 964 007 HEINRICH HC IN: HALLBERG L 213 970 008 HEINRICH HC PROC WORKSHOP CONF HOECHS 3RD 34 975 009 HEINRICH HC KLIN WOCHENSCHR 55 587 977 010 HEINRICH HC ATOMPRAXIX 10 477 964 011 HEINRICH HC EUR J CLIN INVEST 1 321 971 012 HEINRICH HC KLIN WOCHENSCHR 49 819 971 013 HEINRICH HC KLIN WOCHENSCHR 44 827 966 015 HENDERSON JT LANCET 2 241 972 016 LE BAUER E ARCH INTERN MED 122 423 968 017 MCINTYRE PA ARCH INTERN MED 98 541 956 018 NIEWEG HO IN: HEINRICH HC 610 962 019 TOSKES PP J CLIN INVEST 52 1660 973 020 TOSKES PP N ENGL J MED 284 627 971 021 VEEGER W N ENGL J MED 267 1341 962 022 SEPHTON-SMITH R BR MED J 1 608 964 023 STRAHM HW ARCH KINDERHEILK 175 270 967 CT 1 HEINRICH HC KLIN WSCHR 55 587 977 2 LLOYDSTILL JD PEDIATRICS 65 1010 980 3 SOLOMONS NW AM J CLIN NUTR 34 462 981 4 YOUNG VR AM J CLIN NUTR 35 1076 982 5 SWANSON CA AM J CLIN NUTR 38 169 983 6 LEVANDER OA FED PROC 42 1721 983 7 CHRISTENSEN MJ BR J NUTR 50 43 983 8 ROTTOLI A RIV ITAL PEDIATR 10 337 984 9 LEVANDER OA BULL NY ACAD MED 60 144 984 10 WARD KP EUR J PEDIATR 142 21 984 11 SOLOMONS NW TRACE ELEMENTS MED 2 59 985 12 WATSON RD J PARENT ENTERAL NUTR 9 58 985 13 SOLOMONS NW J PEDIATR GASTROENTEROL NUTR 5 122 986 14 DWORKIN B J PARENT ENTERAL NUTR 11 38 987 PN 77128 RN 00709 AN 77231256 AU Heinrich-H-C. Bender-Gotze-C. Gabbe-E-E. Bartels-H. Oppitz-K-H. TI Absorption of inorganic iron- (59Fe2+) in relation to iron stores in pancreatic exocrine insufficiency due to cystic fibrosis. SO Klin-Wochenschr. 1977 Jun 15. 55(12). P 587-93. MJ CYSTIC-FIBROSIS: me. IRON: me. MN CHILD. CHILD-PRESCHOOL. CYTOPLASM: me. HUMAN. INFANT. INTESTINAL-ABSORPTION. IRON-RADIOISOTOPES. IRON: df. PANCREATIN: pd. PANCREATITIS: me. WHOLE-BODY-COUNTING. AB The absorption of 59Fe from a diagnostic 0.56 mg 59Fe2+ dose was measured by 4pi-geometry whole body counting and related to the amount of stainable diffuse cytoplasmatic non heme storage iron in the bone marrow macrophages of children with cystic fibrosis. When the storage iron was within the normal range (+/2+) children with cystic fibrosis absorbed 10-38% (Xa+/-S.D.=21+/-9.3) of the oral 59Fe2+ dose which is identical with the 59Fe-absorption in normal children with normal iron stores (9-45:23+/-8.7%). Depleted iron stores caused an increase of 59Fe-absorption to 43-95% (Xa+/- S.D.=62+/-19) in children with cystic fibrosis and to 45-100% (Xa+/- S.D.=73+/-18) in control children. The interruption or continuation of pancreatin maintenance therapy and the simultaneous administration of 1-1.5 g pancreatin did not influence 59Fe2+ absorption in cystic fibrosis. There is no evidence for a pancreatic factor required for or inhibiting inorganic and food iron absorption in human beings. Iron absorption is controlled also in cystic fibrosis chiefly by the amounts of available storage iron. It is therefore not justified to apprehend the development of hemosiderosis in children with cystic fibrosis who are not or not sufficiently treated with pancreatin. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BALCERZAK SP GASTROENTEROLOGY 53 257 967 003 BIGGS JC LANCET 2 814 963 004 BIGGS JC AUSTRALAS ANN MED 15 36 966 005 DAVIS AE LANCET 2 6 962 006 DELLER DJ AM J DIG DIS 10 249 965 007 GILLY R MOD PROBL PEDIATR 10 263 967 008 GOTZE CH MONATSSCHR KINDERHEILKD 118 210 970 009 GOTZE CH MONATSSCHR KINDERHEILKD 119 13 971 010 HAUSMANN K ACTA HAEMATOL (BASEL) 42 193 969 011 HEINRICH HC IN: HALLBERG L 213 970 012 HEINRICH HC ARZNEIMITTEL FORSCH 25 420 975 013 HEINRICH HC PROC WORKSHOP CONF HOECHS 3RD 34 975 014 HEINRICH HC KLIN WOCHENSCHR 45 553 967 015 HEINRICH HC KLIN WOCHENSCHR 47 984 969 016 HEINRICH HC KLIN WOCHENSCHR 55 587 977 017 HEINRICH HC KLIN WOCHENSCHR 49 819 971 018 HEINRICH HC KLIN WOCHENSCHR 44 827 966 019 HEINRICH HC Z KINDERHEILK 120 251 975 020 KAVIN H GUT 8 556 967 021 KINNEY TD AM J PATHOL 26 746 950 022 KINNEY TD J EXP MED 102 151 955 023 LONGNECKER DS ARCH PATHOL 80 148 965 024 MURRAY MJ GASTROENTEROLOGY 51 694 966 025 SAUNDERS SJ LANCET 1 510 962 026 TAYLOR J J PATHOL BACTERIOL 34 793 931 028 TONZ O LANCET 2 1096 965 029 WEISS S ARCH KINDERHEILK 174 254 966 CT 1 HEINRICH HC KLIN WSCHR 55 595 977 2 CHASE HP J PEDIATR 95 337 979 3 SOLOMONS NW AM J CLIN NUTR 34 462 981 4 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 5 ATER JL PEDIATRICS 71 810 983 6 HODGES P J AM DIET ASSOC 84 664 984 7 WONGMONGKOLRIT T ACTA NEUROPATH (BERL) 65 265 985 PN 77129 RN 00710 AN 77191056 AU Neutra-M-R. Grand-R-J. Trier-J-S. TI Glycoprotein synthesis, transport, and secretion by epithelial cells of human rectal mucosa: normal and cystic fibrosis. SO Lab-Invest. 1977 May. 36(5). P 535-46. MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: bi. INTESTINAL-MUCOSA: me. RECTUM: me. MN ADOLESCENCE. ADULT. AMINO-SUGARS: me. BIOPSY. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pa. EPITHELIUM: cy, me. FUCOSE: me. GLUCOSAMINE: me. GLYCOPROTEINS: me, se. HUMAN. INTESTINAL-MUCOSA: cy. ORGAN-CULTURE. RECTUM: cy, pa. SULFATES: me. SUPPORT-U-S-GOVT-P-H-S. EPITHELIUM: cy. AB The synthesis, transport, and secretion of glycoprotein by human rectal epithelium from normal volunteers, patients with cystic fibrosis, and their disease-free siblings were studied by autoradiography of rectal biopsies pulse-labeled with 3H-glucosamine and maintained in organ culture for various intervals of up to 24 hours. Human rectal goblet and columnar cells transported 3H- glucosamine-labeled secretory products at a substantially slower rate than do comparable colonic cells in smaller mammals. Within any one biopsy sample, the movement of labeled mucus in goblet cells varied widely among cells. Even with individual cells, labeled mucous granules often did not move in concert toward the apical cell surface. Average transport time in the cells of six cystic fibrosis patients and six sibling controls did not differ significantly from those of four adult controls. The carbohydrate composition of glycoprotein secretions of rectal epithelial cells was investigated by comparing autoradiographs of 3H-glucosamine-labeled biopsies with those labeled with 3H-fucose, 3H-N-acetylmannosamine, and 35S- sulfate. The patterns of incorporation of these four precrusors into normal goblet and columnar cells suggested that both cell types may alter the quantity and composition of newly synthesized glycoproteins as they migrate, mature, and senesce. Incorporation patterns in cystic fibrosis biopsies were indistinguishable from those of sibling or adult controls. With the techniques used, no abnormalities of epithelial glycoprotein production were detected in cystic fibrosis rectal mucosa. RF 001 ADELSTEIN RS NATURE 255 106 975 002 ALPERS DH J CLIN INVEST 51 2621 972 003 BARBERO GJ IN: MANGOS JA 83 976 004 BAUDUIN H J CELL BIOL 66 165 975 005 BENNETT G J CELL BIOL 46 409 970 006 BENNETT G J CELL BIOL 60 258 974 007 BENNETT GC ANAT REC 184 357 976 008 BOAT TF IN: MANGOS JA 165 976 009 BOAT TF AM REV RESPIR DIS 110 428 974 010 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 011 BROWNING TH J CLIN INVEST 48 1423 969 012 COFFEY JW J BIOL CHEM 239 4011 964 013 DALTON AJ ANAT REC 121 281 955 014 DI SANTAGNESE PA N ENGL J MED 295 481 976 015 DI SANTAGNESE PA N ENGL J MED 277 1287 967 016 DISCHE Z PEDIATRICS 24 74 959 017 DONNISON AB PEDIATRICS 37 833 966 018 EASTWOOD GL GASTROENTEROLOGY 64 375 973 019 EYLAR E GAP CONF REP CELL BIOCHEM 969 020 FILIPE MI GUT 10 577 969 021 FLOREY HW GASTROENTEROLOGY 43 326 962 022 FORSTNER GG J BIOL CHEM 245 3584 970 023 FORSTNER JF PEDIATR RES 10 609 976 024 FREEMAN JA ANAT REC 154 121 966 025 FREYE HB J PEDIATR 64 575 964 026 GUGLER EC J PEDIATR 71 585 967 027 HOSKINS LC ANN NY ACAD SCI 106 767 963 028 ITO S FED PROC 28 12 969 029 JABBAL I CAN J BIOCHEM 54 707 976 030 JABRO IH J LANCET 86 385 966 031 JOHANSEN PG LANCET 1 455 968 032 JOHANSEN PG J PATHOL 99 299 969 033 KIM YS J BIOL CHEM 246 5466 971 034 LAMB D BR J DIS CHEST 66 239 972 035 LEV R AM J PATHOL 46 23 965 036 MACDERMOTT RP J CLIN INVEST 54 545 974 037 MICHAELS JE J MICROSC BIOL CELL 25 243 976 038 MONACO F J BIOL CHEM 248 2072 973 040 NEUTRA M J CELL BIOL 30 119 966 041 NEUTRA M J CELL BIOL 30 137 966 042 PARKINS RA LANCET 2 851 963 043 RAVETTO C ARCH ITAL MAL APP DIG 37 49 973 044 REDMAN CM J CELL BIOL 66 42 975 045 ROELFS RE AM J DIS CHILD 113 419 967 046 SHANTZ A STAIN TECHNOL 40 279 965 047 SHWACHMAN H N ENGL J MED 286 1300 972 048 SKY-PECK HH ANN NY ACAD SCI 130 951 966 049 SOERGEL KH GASTROENTEROLOGY 47 610 964 050 TRIER JS IN: CODE CF 3 1125 968 051 UGOLEV AM BIOCHIM BIOPHYS ACTA 300 105 973 052 WEISER MM J BIOL CHEM 248 2536 973 053 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 NEUTRA MR J CELL BIOL 74 983 977 2 ALHADEFF JA CLIN GENET 14 189 978 3 DERMER GB IN VITRO 14 804 978 4 JONES R BR MED BULL 34 9 978 5 NEUTRA MR GASTROENTEROLOGY 75 701 978 6 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 7 FILIPE MI INVEST CELL PATHOL 2 195 979 8 BERTELOOT A IN VITRO 15 294 979 9 QURESHI R J CLIN INVEST 64 1149 979 10 QUARONI A BIOCHEM J 182 213 979 11 NEUTRA MR ANAT REC 193 367 979 12 DAWSON PA HISTOCHEM J 12 23 980 13 DAVIS PB PEDIATR RES 14 83 980 14 NEUTRA MR J ULTRASTRUCT RES 70 186 980 15 SPECIAN RD J CELL BIOL 85 626 980 16 LAMONT JT BIOCHIM BIOPHYS ACTA 629 553 980 17 STALEY TE AM J VET RES 42 912 981 18 BENNETT G J CELL BIOL 88 16 981 19 SPECIAN RD AM J ANAT 160 461 981 20 COLES SJ CELL TISSUE RES 214 107 981 21 SAKATA T CELL TISSUE RES 219 371 981 22 FORSTNER JF AM J PHYSIOL 240 G 10 981 23 FORSTNER J AM J PHYSIOL 241 G443 981 24 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 25 EHSANULLAH M J CLIN PATHOL 35 26 982 26 MARIN MG J APPL PHYSIOL 52 198 982 27 BOAT TF CHEST 81 S 29 982 28 NEUTRA MR CHEST 81 S 14 982 29 SENIOR PV J ANAT 134 459 982 30 FORSTNER G ADV EXP MED BIOL 144 199 982 31 DOUGLAS WHJ ANAT REC 202 285 982 32 NEUTRA MR AM J PHYSIOL 242 G380 982 33 SPECIAN RD AM J PHYSIOL 242 G370 982 34 APPLEGARTH DA MED HYPOTHESES 11 277 983 35 DAWSON PA HISTOCHEM J 15 3 983 36 FRATES RC PEDIATR RES 17 30 983 37 DAVIS PB J CHRON DIS 36 269 983 38 HOWDLE PD CLIN SCI 65 105 983 39 PODOLSKY DK J CLIN INVEST 72 142 983 40 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 41 SENIOR PV BR J CANCER 49 281 984 42 TRIER JS LAB INVEST 50 673 984 43 SZABO S LAB INVEST 51 121 984 44 SPECIAN RD GASTROENTEROLOGY 87 1313 984 45 NEUTRA MR CIBA FOUND SYMP 109 20 984 46 BRADY RC EXP CELL RES 150 141 984 47 PERDUE MH AM J PHYSIOL 247 G632 984 48 ROOMI N AM J PHYSIOL 247 G140 984 49 BASBAUM C GASTROENTEROL CLIN BIOL 9 45 985 50 SANDOZ D BIOL CELL 54 79 985 51 BASBAUM CB CLIN CHEST MED 7 231 986 52 SMITH AC CLIN GASTROENTEROL 15 815 986 53 PODOLSKY DK J CLIN INVEST 77 1263 986 54 RUBINSTEIN S PEDIATRICS 78 473 986 55 DEITCHER DL J CELL BIOL 102 911 986 56 COAPMAN RA ARCH PATHOL LAB MED 110 124 986 57 COLONY PC GASTROENTEROLOGY 92 1116 987 58 COOPER HS ARCH PATHOL LAB MED 111 270 987 PN 77130 RN 00711 AN 77145963 AU Sanjurjo-P. Allue-X. Rodriguez-Soriano-J. TI Fatty-acid composition of lecithin fraction of mucus in cystic fibrosis [letter]. SO Lancet. 1977 Apr 2. 1(8014). P 752. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: an. MUCUS: an. PHOSPHATIDYLCHOLINES: an. MN BRONCHI. CHILD. FATTY-ACIDS-ESSENTIAL: df. HUMAN. EX We have studied seven patients with C.F. and ten normal children and have confirmed the low levels of E.F.A. both in plasma and in red-cell membrane in the C.F. group. We would like to report here preliminary results on the fatty-acid pattern of the lecithin fraction of bronchial mucus in the same group of patients and controls. Content of palmitic acid was lower and that of oleic acid was higher in the C.F. patients than in normal children. This abnormality in the fatty-acid composition of the lecithin of the mucus could be an important finding in relation to the respiratory symptoms in C.F. Some experimental work supports this hypothesis. RF 001 KUO PT J CLIN INVEST 44 1924 965 002 ROSENLUND ML NATURE 251 719 974 003 RIVERS JPW LANCET 2 642 975 004 CHASE HP PEDIATRICS 57 441 976 005 ELLIOTT RB PEDIATRICS 57 474 976 006 KYRIAKIDES EC BIOCHIM BIOPHYS ACTA 431 399 976 007 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 CT 1 ALLUE X PEDIATRICS 61 924 978 2 FRIEDMAN Z PEDIATRICS 63 855 979 3 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 4 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 5 GALABERT C CLIN RESP PHYSIOL 17 197 981 6 ROGIERS V PEDIATR RES 16 761 982 7 HARPER TB AM REV RESPIR DIS 126 540 982 8 ROGIERS V PEDIATR RES 18 704 984 9 CAMPBELL IM EUR J CLIN MICROBIOL 5 622 986 10 GILLJAM H SCAND J CLIN LAB INVEST 46 511 986 PN 77131 RN 00712 AN 77253838 AU Berger-H-M. Reynolds-S-J. Lee-K-H. TI False-positive meconium screen [letter]. SO Lancet. 1977 Aug 27. 2(8035). P 458. MJ CYSTIC-FIBROSIS: di. INFANT-NEWBORN-DISEASES: di. MECONIUM. MN FALSE-POSITIVE-REACTIONS. GLYCERIN: du. HUMAN. INFANT-NEWBORN. MECONIUM: me. SUPPOSITORIES. EX The BM meconium test is a useful screening test for cystic fibrosis. However, we have had false-positive results in two babies caused by the use of rectal glycerin suppositories. The babies presented with signs suggestive of intestinal obstruction possibly due to meconium ileus, and glycerin suppositories were inserted to speed the passage of meconium so that the BM test could be done to help confirm the diagnosis. The tests were positive but the babies were subsequently shown not to have cystic fibrosis, and we found that the glycerin suppositories themselves produce the typical blue colour on the test strip. PN 77132 RN 00713 AN 78070727 AU Super-M. TI Heterozygote disadvantage in cystic fibrosis [letter]. SO Lancet. 1977 Dec 17. 2(8051). P 1288. MJ CYSTIC-FIBROSIS: fg. MN CAUCASOID-RACE. HETEROZYGOTE. HUMAN. SOUTH-AFRICA. TRANSIENTS-AND-MIGRANTS. TROPICAL-CLIMATE. EX I have found that cystic fibrosis (C.F.), which is very common in South West Africa, occurs almost exclusively in white Afrikaners, probably as a result of inbreeding, the drift from the Northern Cape Colony, and religious and geographical separation. However, I have not found C.F. in the descendants of the Angola Boers, a small proportion (6 - 7%) of the White population who belong to a small branch of the Dutch Reformed Church. The absence of C.F. may stem from their limited numbers (7000) but I suggest that it may have at least partially a genetic basis. Homozygotes with C.F. are subject to salt-losing crises which can be fatal in hot weather and heterozygotes often have an increased concentration of sodium and chlorine ions in their sweat. The influence of environmental temperature and drought may explain why C.F. occurs among Blacks in temperate zones of the United States but not in Africa, and is less frequent in the hot southern regions of Europe than in the colder, more northern areas. Negative selection of this type may have operated only in hot, arid environments and the possibility that other environments offered advantages to C.F. heterozygotes cannot be excluded. RF 001 SUPER M S AFR MED J 49 818 975 003 GOLDBLATT I HIST SW AFRICA FROM BEGINNING 971 004 KESSLER WR PEDIATRICS 8 648 951 005 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 006 VEDDER H SOUTH WEST AFRICA IN EARLY TI 420 966 007 TRUMPELMAN GPJ IN: BOER IN SUID WEST AFRI 948 009 KULCZYCKI LL AM J DIS CHILD 127 64 974 010 DI SANTAGNESE PA N ENGL J MED 295 481 976 011 LEVIN S IN: GOLDSCHMIDT E 294 963 CT 1 SUPER M S AFR MED J 54 18 978 2 SUPER M MONOGR PAEDIATR 10 106 979 PN 77133 RN 00714 AN 78070728 AU Whitelaw-A. Butterfield-A. TI High breast-milk sodium in cystic fibrosis [letter]. SO Lancet. 1977 Dec 17. 2(8051). P 1288. MJ CYSTIC-FIBROSIS: me. MILK-HUMAN: an. SODIUM: an. MN FEMALE. HUMAN. PREGNANCY. EX Increased sodium concentration in sweat is a characteristic of cystic fibrosis. However, we have read nothing on the sodium content of breast milk in this condition. A patient with sweat-test-proved cystic fibrosis with mild pulmonary disease had her first baby by normal delivery at term. Samples of breast milk were obtained on the sixth and seventh days post partum when the mother was not breast feeding. The two sodium concentrations were 132 and 280 mmol/l, concentrations much higher than in any other milk sample tested at this hospital in a large survey and higher than any we have seen in the literature. Milk of such high sodium content would be unsafe for a baby, and we suggest that, if mothers with cystic fibrosis were to breast feed, the sodium of the mild should be measured first. The breast, like so many other exocrine glands, seems to be affected by the secretory abnormalities of cystic fibrosis. CT 1 DANN LG LANCET 2 405 978 2 BERLIN CM OBSTET GYNECOL 58 S 17 981 3 WELCH MJ PEDIATRICS 67 664 981 4 WALPOLE IR J PEDIATR 98 333 981 5 MCKIERNAN J PEDIATR RES 16 60 982 6 GRUSKIN AB PEDIATR CLIN NORTH AM 29 907 982 7 ALPERT SE J PEDIATR 102 77 983 8 SEALE TW ANN CLIN LAB SCI 15 152 985 9 STEAD RJ ARCH DIS CHILD 62 433 987 PN 77134 RN 00715 AN 78091185 AU Hubbard-V-S. Dunn-G-D. di-SantAgnese-P-A. TI Abnormal fatty-acid composition of plasma-lipids in cystic fibrosis. A primary or a secondary defect?. SO Lancet. 1977 Dec 24-31. 2(8052-8053). P 1302-4. MJ CYSTIC-FIBROSIS: bl. FATTY-ACIDS: bl. LIPIDS: bl. MN ADOLESCENCE. ADULT. CYSTIC-FIBROSIS: fg, pp. FEMALE. HETEROZYGOTE. HUMAN. MALABSORPTION-SYNDROMES: bl, et. MALE. MIDDLE-AGE. OLEIC-ACIDS: bl. PALMITIC-ACIDS: bl. PANCREAS: pp. PHOSPHOLIPIDS: bl. TRIGLYCERIDES: bl. AB The percentage fatty-acid composition of the various plasma-lipid fractions from cystic-fibrosis patients with and without pancreatic insufficiency, obligate heterozygotes, and normal subjects was determined. Only the cystic-fibrosis patients with pancreatic insufficiency had significantly abnormal fatty-acid composition. This general observation did not correlate with vitamin-E deficiency, age, sex, or severity of the disease. Thus the altered fatty-acid composition of the different plasma-lipid fractions appears to be a secondary consequence of the pancreatic insufficiency commonly associated with cystic fibrosis and not a direct metabolic defect related to the cystic-fibrosis gene itself. RF 001 BENNETT MJ AM J CLIN NUTR 20 415 967 002 KUO PT J CLIN INVEST 44 1924 965 003 KUO PT J PEDIATR 60 394 962 004 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 005 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 006 ELLIOTT RB ARCH DIS CHILD 50 76 975 007 RIVERS JPW LANCET 2 642 975 008 ROSENLUND ML NATURE 251 719 974 009 BEVERIDGE J PEDIATRICS 58 465 976 010 ELLIOTT RB PEDIATRICS 57 474 976 011 TAUSSIG LM PEDIATRICS 54 229 974 012 DANON A BIOCHIM BIOPHYS ACTA 388 318 975 013 SOBEL AE J BIOL CHEM 171 617 947 014 QUAIFE ML J BIOL CHEM 180 1229 949 015 DODGE JA BR MED J 2 192 975 016 EMERY AEH LANCET 2 80 975 017 MCEVOY FA LANCET 2 236 975 018 THOMPSON G LANCET 2 719 975 019 PRESS M LANCET 2 871 975 020 WATTS R LANCET 2 983 975 021 CAMPBELL IM PEDIATRICS 57 480 976 022 CHASE HP PEDIATRICS 57 441 976 023 COLLINS FD NUTR METAB 13 150 971 024 PRESS M BR MED J 2 247 974 025 WOLFRAM G NUTR METAB (SUPPL 1) 21 127 977 026 ALFIN-SLATER RB PHYSIOL REV 48 758 968 027 TAUSSIG LM J PEDIATR 82 380 973 CT 1 CHASE HP LANCET 2 236 978 2 GALABERT C LANCET 2 903 978 3 FRIEDMAN Z SEM PERINATOL 3 341 979 4 ANON NUTR REV 37 247 979 5 LLOYDSTILL JD PEDIATRICS 64 50 979 6 SANTAGNESE PAD AM J MED 66 121 979 7 HUBBARD VS AM J CLIN NUTR 33 2281 980 8 COATES AL ACTA PAEDIATR SCAND 69 353 980 9 DAVIS PB J LAB CLIN MED 96 75 980 10 HUBBARD VS CLIN CHIM ACTA 102 115 980 11 ROGIERS V CLIN CHIM ACTA 105 105 980 12 FRIEDMAN Z AM J DIS CHILD 134 397 980 13 PARK RW GASTROENTEROLOGY 81 1143 981 14 LLOYDSTILL JD J PEDIATR 99 580 981 15 CHASE HP J PEDIATR GASTROENTEROL NUTR 1 49 982 16 ROGIERS V PEDIATR RES 16 761 982 17 GASKIN K J PEDIATR 100 857 982 18 HARPER TB AM REV RESPIR DIS 126 540 982 19 FARRELL PM ANN NY ACAD SCI 393 96 982 20 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 21 FARRELL PM J DENT CHILD 50 385 983 22 HANSEN RC ARCH DERMATOL 119 51 983 23 HUBBARD VS EUR J PEDIATR 141 68 983 24 ROGIERS V EUR J PEDIATR 141 39 983 25 ROGIERS V PEDIATR RES 18 704 984 26 DAVIS PB J LAB CLIN MED 104 203 984 27 ROGIERS V EUR J PEDIATR 142 305 984 28 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 29 FARRELL PM PEDIATR RES 19 104 985 30 ZENTLERMUNRO PL GUT 26 892 985 31 CHRISTOPHE A ANN NUTR METAB 29 239 985 32 MOILANEN T AM J CLIN NUTR 42 708 985 33 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 34 LESTER LA J PARENT ENTERAL NUTR 10 289 986 35 MISCHLER EH PEDIATR RES 20 36 986 36 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 37 MOORE MC AM J CLIN NUTR 44 33 986 38 PARSONS HG BIOCHIM BIOPHYS ACTA 860 420 986 PN 77135 RN 00716 AN 77098975 AU Gyurkovits-K. Markus-V. Bittera-I. TI Cystic-fibrosis heterozygosity in childhood bronchial asthma [letter]. SO Lancet. 1977 Jan 22. 1(8004). P 203. MJ ASTHMA: co. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. MN ASTHMA: et. BROMIDES: du. CHILD. CHLORIDES: an. HUMAN. SODIUM: an. SWEAT: an. EX We found that the Szczepanski bromide test, which we simplified with the aid of ion-sensitive electrode, is suitable for the detection of C.F. heterozygosity in most cases. We examined the sweat of 74 randomly selected children with respiratory asthma during the past 2 years, the sodium and chloride concentrations and bromide index being determined. Sodium and chloride concentrations in the sweat of the asthma children were slightly but significantly higher (P < 0.05) than those of the controls. The difference in bromide index was even more significant (P < 0.01). This suggests that C.F. gene carriers are unusually prevalent among children with bronchial asthma. One possibility is that enhanced ion loss in various diseases with allergic pathological mechanisms is secondary in nature. However, this seems unlikely since in our material high sweat-electrolyte concentrations were observed in only a proportion of control children whose illness was similar in severity to that of the asthmatic children, and normal sweat values were obtained even when the child's condition was very serious. RF 001 WARNER JO LANCET 1 990 976 002 MCFARLANE H LANCET 1 1241 976 003 SZCZEPANSKI Z Z KINDERHEILK 113 297 972 004 GYURKOVITS K ACTA PAEDIATR ACAD SCI HUNG 15 49 974 PN 77136 RN 00717 AN 78049968 AU Crossley-J-R. Berryman-C-C. Elliott-R-B. TI Cystic-fibrosis screening in the newborn. SO Lancet. 1977 Nov 26. 2(8048). P 1093-5. MJ CYSTIC-FIBROSIS: di. MN BENZOYLARGININE-NITROANILIDE: du. COLORIMETRY: mt. CYSTIC-FIBROSIS: en. EVALUATION-STUDIES. FALSE-POSITIVE-REACTIONS. FECES: en. HUMAN. HYDROLYSIS. INFANT-NEWBORN. TRYPSIN: an. AB In a new method of testing stool samples from newborn babies for cystic fibrosis (C.F.), a colourless substrate, benzoyl-arginine-p- nitroanilide (B.A.P.N.A.), releases yellow p-nitroaniline when hydrolysed by trypsin. Samples from infants with C.F., who lack trypsin, give negligible colour. 2 infants with C.F. were detected among 2500 consecutive newborn babies tested. The incidence of false- positive results was 1.2% after the first specimen and 0.05% after the second specimen. A further refinement has reduced the positive rate to 0.1% after the first specimen (2000 samples). Tests on samples from 5 other older patients with untreated C.F. have yielded no evidence for false-negative results. RF 001 ROBINSON PG NZ MED J 83 268 976 002 SHWACHMAN H PEDIATRICS 46 335 970 003 ELLIOTT RB PEDIATRICS 57 474 976 004 VERDONK G PROC INT CONF ON NUTRI 9TH 1 278 972 005 PROSSER R ARCH DIS CHILD 49 597 974 006 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 007 ROBINSON PG NZ MED J 79 1024 974 008 ROBINSON PG ARCH DIS CHILD 51 301 976 009 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 010 ROBINSON PG CLIN CHIM ACTA 62 225 975 011 RYLEY HC CLIN CHIM ACTA 64 117 975 012 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 013 ERLANGER BF ARCH BIOCHEM BIOPHYS 115 206 966 CT 1 ELLIOTT RB MED J AUST 2 95 978 2 HOLTZMAN NA PEDIATR CLIN NORTH AM 25 411 978 3 CROSSLEY JR LANCET 1 472 979 4 STEPHAN U MONOGR PAEDIATR 10 41 979 5 STAGG BH ANN CLIN BIOCHEM 16 147 979 6 WARWICK WJ J CHRON DIS 33 685 980 7 TARNOKY AL J ROY SOC MED 73 73 980 8 LAMBOTTE C J GENET HUM 29 85 981 9 CARREL O HELV PAEDIATR ACTA 36 405 981 10 FORREST DC ARCH DIS CHILD 56 151 981 11 BORGSTROM A ACTA PAEDIATR SCAND 70 619 981 12 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 13 CROSSLEY JR CLIN CHIM ACTA 113 111 981 14 DODGE JA ARCH DIS CHILD 57 774 982 15 HEELEY AF CLIN CHEM 29 2011 983 16 LUTHI M HELV PAEDIATR ACTA 38 149 983 17 LYON ICT NZ MED J 96 673 983 18 WILCKEN B J PEDIATR 102 383 983 19 WILCKEN B J PEDIATR 103 668 983 20 ROGIERS V EUR J PEDIATR 141 39 983 21 BORGSTROM A PEDIATR RES 18 957 984 22 NAYLOR EW SEM PERINATOL 9 232 985 PN 77137 RN 00718 AN 78049949 AU George-R-H. Healing-D-E. TI Thymidine-requiring Haemophilus influenzae and Staphylococcus aureus [letter]. SO Lancet. 1977 Nov 19. 2(8047). P 1081. MJ HAEMOPHILUS-INFLUENZAE: gd. STAPHYLOCOCCUS-AUREUS: gd. MN CHILD. CULTURE-MEDIA. CYSTIC-FIBROSIS: mi. HAEMOPHILUS-INFLUENZAE: ip. HUMAN. SPUTUM: mi. STAPHYLOCOCCUS-AUREUS: ip. THYMIDINE: me. EX In the past year we have isolated thy- Staphylococcus aureus from the sputum of four children with cystic fibrosis who were receiving co-trimoxazole, and lately we have isolated a thy- strain of Haemophilus influenzae from a patient with cystic fibrosis also receiving co-trimoxazole. In view of the exacting growth requirements of H. influenzae we feel that thy- strains might easily be overlooked or assumed to have died when they fail to grow on sensitivity-test media. RF 001 LACEY RW BR MED J 4 165 973 002 MASKELL R LANCET 1 834 976 003 SEDGWICK B J BACTERIOL 123 1208 975 CT 1 SPARHAM PD J CLIN PATHOL 31 913 978 2 DAVIES J ANNU REV MICROBIOL 32 469 978 3 DAVIES J REV INFECT DIS 1 23 979 4 WILLIAMS RF CURR MED RES OPIN 6 43 979 5 WORMSER GP ANN INTERN MED 91 420 979 6 STURM AW ANT V LEEUW J MICROBIOL SEROL 47 92 981 7 NEU HC REV INFECT DIS 4 S288 982 8 LACEY RW J MED MICROBIOL 15 403 982 9 BROGDEN RN DRUGS 23 405 982 10 GRIECO MH MED CLIN NORTH AM 66 25 982 11 KING CH J CLIN MICROBIOL 18 79 983 PN 77138 RN 00719 AN 77169610 AU Sleisenger-M-H. Brandborg-L-L. TI Malabsorption. SO Major-Probl-Intern-Med. 1977. 13. P v-x, 1-261. MJ MALABSORPTION-SYNDROMES: di. MN BIOPSY. CELIAC-DISEASE: di. CHILD. CYSTIC-FIBROSIS: di. GASTROENTERITIS: di. HUMAN. INTESTINAL-ABSORPTION. INTESTINAL-NEOPLASMS: co. INTESTINE-SMALL: im, mi, pa, pp. LIPODYSTROPHY-INTESTINAL: di. LYMPHOMA: co. MALABSORPTION-SYNDROMES: co, pa, pp, th. CELIAC-DISEASE: di. EX Laboratory diagnosis of malabsorption syndromes in children is more difficult for obvious reasons involving the collection of specimens. The early diagnosis of cystic fibrosis is extremely urgent, since the disease is treatable. The standard test is the sweat test. Over 99 percent of patients with this disease will have elevation of sodium and chloride in the sweat. Three tests are used to assess pancreatic function. First is the measurement of stool trypsin and chymotrypsin. Second, stimulation with hormones - the standard secretin and CCK tests. A third procedure tests carbohydrate tolerance, an increasingly important test since an ever-growing number of patients in late childhood and early adulthood with cystic fibrosis are being found to have diabetes mellitus. The commonest and most important hereditary disease that affects pancreatic function is cystic fibrosis. The commonest form of pancreatic insufficiency in childhood is mucoviscidosis or cystic fibrosis of the pancreas. The incidence of this disease is approximately one per 1000 live births. It occurs in all races, and the defect is transmitted as an autosomal recessive. The classic features are frequent bowel movements with the characteristic appearance of steatorrheic stools, and a failure to grow and develop normally. RF 001 SHWACHMAN H IN: SLEISENGER MH 1206 973 001 HAMILTON JR IN: SLEISENGER MH 280 973 002 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 002 WORMSLEY KG IN: HOWAT HT 1 972 003 MARKS JF J PEDIATR 69 225 966 004 WINAWER SJ IN: GLASS GBJ 1 968 004 NAVAB F GUT 11 373 970 005 MORIN CL J CLIN INVEST 50 1961 971 005 SCRIBNER BH JAMA 212 457 970 006 KEKOMAKI M ACTA PAEDIATR SCAND 56 617 967 006 KRONE CL MEDICINE (BALTIMORE) 47 89 968 007 GOODMAN SI J PEDIATR 71 246 967 007 PRIZONT R GASTROENTEROLOGY 69 1254 975 008 BANTSOCAS CS AM J DIS CHILD 117 93 969 008 KAHN IJ N ENGL J MED 274 1339 966 009 HOOFT C ANN PAEDIATR 205 73 965 009 PEARSON AJ AM J DIG DIS 14 200 969 010 DRUMMOND KN AM J MED 37 928 964 011 CASH R J PEDIATR 74 717 969 012 HADORN B CLIN GASTROENTEROL 1 125 972 013 LAURIALA K IN: SCHMERLING DH 968 014 HALL SD AM J CLIN NUTR 22 448 969 015 SCRIVER CR PEDIATRICS 45 361 970 016 PAUNIER L PEDIATRICS 41 385 968 017 LILLIBRIDGE CB GASTROENTEROLOGY 52 792 967 018 MOHAMED SD Q J MED 35 433 966 019 LANZKOWSKY P AM J MED 48 580 970 CT 1 RASMUSSEN P J DENT CHILD 47 42 980 2 LO CW PEDIATRICS 72 786 983 PN 77139 RN 00720 AN 77146553 AU Cohn-D. TI Multilobular biliary cirrhosis of the liver associated with cystic fibrosis (mucoviscidosis). SO Med-J-Aust. 1977 Jan 22. 1(4). P 101-4. MJ CYSTIC-FIBROSIS: co. LIVER-CIRRHOSIS-BILIARY: et. MN ADULT. AUTOPSY. CASE-REPORT. HUMAN. MALE. AB A case (including autopsy findings) is reported of multilobular biliary cirrhosis with a strictly nodular liver developing in a 20- year-old male with cystic fibrosis (mucoviscidosis). The pathogenesis of biliary cirrhosis in cystic fibrosis is briefly discussed. RF 001 DI SANTAGNESE PA PEDIATRICS 18 387 956 002 SORRELL VF AUST NZ J SURG 37 217 968 003 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 004 CRAIG JM AM J DIS CHILD 93 357 957 005 TYSON KRT J PEDIATR SURG 3 271 968 006 GRAND RJ CLIN PEDIATR 9 588 970 007 CAMERON R BILIARY CIRRHOSIS 962 008 SHIER KJ CAN MED ASSOC J 89 645 963 009 MONTGOMERY BK AM J CLIN PATHOL 26 630 956 010 WEBSTER R ARCH DIS CHILD 28 343 953 011 KOPEL FB GASTROENTEROLOGY 62 483 972 012 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 CT 1 FINBERG L N ENGL J MED 300 420 979 2 CUNNINGHAM DG J COMPUT ASSIST TOMOGR 4 151 980 3 BERNARD O CLIN GASTROENTEROL 14 33 985 PN 77140 RN 00721 AN 77232262 AU Webster-L. Lochlin-H. TI Cystic fibrosis screening by sweat analysis: a critical review of techniques. SO Med-J-Aust. 1977 Jun 18. 1(25). P 923-7. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. ELECTRIC-CONDUCTIVITY. HUMAN. INFANT. INFANT-NEWBORN. OSMOLAR-CONCENTRATION. POTASSIUM: an. SODIUM: an. SPECIMEN-HANDLING: mt. TEMPERATURE. AB Currently employed methods for the assay of electrolytes in sweat, relevant to the diagnosis of cystic fibrosis, are critically evaluated with particular reference to the measurement of electrical conductivity. Attention is drawn to the factors responsible for potentially large errors. On the basis of experimentation, supported by laboratory practice, recommendations are made for the reduction of these errors to clinically acceptable levels. Data concerning sodium and potassium content, electrical conductivity and osmolality of undiluted sweat collections are compared. The feasibility and comparative advantages of measuring sweat osmolality with a vapour pressure osmometer are discussed. RF 001 SHWACHMAN H PEDIATRICS 32 85 963 002 PHILLIPS WR PEDIATRICS 32 89 963 003 WEINER JS NATURE 164 351 949 CT 1 SHIRAMIZU B ANAL CHIM ACTA 108 161 979 2 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 3 KIRK JM ANN CLIN BIOCHEM 20 369 983 4 FRANCKX J HELV PAEDIATR ACTA 39 347 984 5 SCHONI MH J PEDIATR 104 691 984 6 ITANO M RESPIRATION 47 220 985 7 GEETHA H BR MED J 294 156 987 PN 77141 RN 00722 AN 77146406 AU Shwachman-H. Kowalski-M. Khaw-K-T. TI Cystic fibrosis: a new outlook. 70 patients above 25 years of age. SO Medicine (Baltimore). 1977 Mar. 56(2). P 129-49. MJ CYSTIC-FIBROSIS: di. MN ADULT. ANTIBIOTICS: tu. CHILD. CLUBFOOT: et. CYSTIC-FIBROSIS: co, th. EDUCATIONAL-STATUS. FAMILY-CHARACTERISTICS. FEMALE. FOLLOW-UP-STUDIES. HOSPITALIZATION. HUMAN. MALE. MARRIAGE. NASAL-POLYPS: et. OCCUPATIONS. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: et. PATIENT-COMPLIANCE. PREGNANCY. QUALITY-OF-LIFE. RESPIRATORY-FUNCTION-TESTS. SOCIOECONOMIC-FACTORS. SPUTUM: mi. SWEAT. AB We have presented a group of 70 patients over 25 years of age as of January 1, 1975, who were diagnosed as having cystic fibrosis (CF) at various ages from birth to 28 years. Some patients were diagnosed late in life in spite of classical symptoms and a characteristic history of the disease. Patients with CF present with a broad spectrum of symptoms which vary considerably in severity. This older age group represents approximately 10% of our current roster of patients with CF. This disease is no longer confined to early life and will continue to be seen with increasing frequency in older patients. Our experience with this disease indicates that a number of affected individuals can attain adulthood and find a useful place in society. The quality of life provided to these adults is illustrated in their intellectual, socioeconomic achievements and marital life. Our aim has been to recognize the disease as early in life as possible and to provide a comprehensive medical program which includes all aspects of health care. RF 001 ADDINGTON WW CHEST 59 306 971 002 ANDERSON EG BR J DIS CHEST 59 173 965 003 ANDERSON EL J S CAROLINA MED ASSOC 68 275 972 004 ANTONOWICZ I PEDIATRICS 42 492 968 005 ARAKI H PEDIATR RES 9 932 975 007 BATTEN J RESPIRATION SUPPL 27 163 970 008 BAUMGARTNER U SCHWEIZ MED WOCHENSCHR 89 130 959 009 BIANCO S GAZZ INT MED CHIR 72 2747 967 010 BOHLE E AERZTL PRAXIX 21 907 969 011 BOHN H DTSCH MED WSCHR 86 1384 961 012 BOUCHER H PRESSE MED 69 2123 961 013 BOUR H REV PRAT 17 3347 967 014 BRUN J POUMON COEUR 22 961 966 015 BRUSILOW SW ANNU REV MED 21 99 970 016 BURNARD ED NZ MED J 52 395 953 017 CABANEL G MOD PROBL PEDIATR 10 284 967 018 CABANEL G SEM HOP PARIS 44 1203 968 019 CALDWELL DM CALIF MED 89 280 958 020 CARBONI M EPATOLOGIA 16 465 970 021 CECE JD JAMA 181 31 962 022 CHEBAT J J FRANC MED CHIR THORAC 24 771 970 023 CHEBAT J IMMEX (PARIS) 871 963 024 CHEBAT J POUMON COEUR 27 3 971 025 COATES EO JR DIS CHEST 49 195 966 026 DALFINO G MED CLIN SPER 12 403 962 027 DE HALLER R REV MED SUISSE ROM 82 42 962 028 DE KALBERMATTEN JP POUMON COEUR 24 459 968 029 DESMOND FB NZ MED J 62 380 963 030 DI SANTAGNESE PA N ENGL J MED 279 103 968 031 DI SANTAGNESE PA MOD PROBL PEDIATR 10 135 967 032 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 033 DOYLE B PHYS THER REV 39 24 959 034 DUBACH UC SCHWEIZ MED WOCHENSCHR 92 783 962 035 ANON LANCET 1 963 960 036 ANON JAMA 150 1407 952 037 FELLOWS KE RADIOLOGY 114 551 975 038 GALLET M LYON MED 225 845 971 039 GALLET M MED CHIR DIG 1 249 972 040 GEORGE L BR MED J 3 718 969 041 GIBSON LE PEDIATRICS 23 545 959 042 GRAND RJ JAMA 195 993 966 043 GRANDCHAMP A SCHWEIZ MED WOCHENSCHR 98 1388 968 044 HOLSCLAW DS J PEDIATR SURG 9 867 974 045 HOLSCLAW DS PEDIATRICS 48 442 971 046 HOLSCLAW DS MINN MED 52 1547 969 047 HOLSCLAW DS J PEDIATR 76 829 970 048 HOLSCLAW DS PEDIATRICS 48 51 971 049 HOLSCLAW DS J UROL 106 568 971 050 JONES JS BR J DIS CHEST 64 25 970 051 KAPLAN E N ENGL J MED 279 65 968 052 KARLISH AJ LANCET 2 514 960 053 KOCH E DTSCH ARCH KLIN MED 206 470 960 054$ KOCH E MED WSCHR 40 2033 961 055 KOHL HW ARIZ MED 5 47 948 056 KULCZYCKI LL JAMA 175 358 961 057 KULCZYCKI LL N ENGL J MED 257 203 957 058 KULCZYCKI LL N ENGL J MED 259 409 958 059 LANDING BH ARCH PATHOL 88 569 969 060 LAPEY A J PEDIATR 84 328 974 061 LEPORE MJ GASTROENTEROLOGY 44 696 963 062 LIFSCHLITZ NA KLIN MED 51 117 973 063 MARKS BL LANCET 1 365 960 064 MEYERS A AM J DIS CHILD 129 1011 975 065 MURTHY MSN OHIO STATE MED J 69 768 973 066 NOWAK S POL MED J 7 114 968 067 ONEAL R THESIS 948 068 PARKINS RA LANCET 2 851 963 069 PETERSON EM JAMA 171 87 959 070 POLGAR G AM REV RESPIR DIS 85 319 962 071 RECANT L AM J MED 27 483 959 072 ROSAN RC AM J DIS CHILD 104 625 962 073 SCHANDEVYL W ACTA TUBERC PNEUMONOL BELG 62 478 971 074 SELIGER G AM J DIG DIS 17 934 972 075 SHWACHMAN H AM J DIS CHILD 96 6 958 076 SHWACHMAN H PEDIATRICS 36 689 965 077 SHWACHMAN H ANN NY ACAD SCI 93 600 962 078 SHWACHMAN H PEDIATRICS 7 153 951 079 SHWACHMAN H AM J DIS CHILD 92 347 956 080 SHWACHMAN H N ENGL J MED 286 1300 972 082 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 083 SHWACHMAN H MINN MED 52 1521 969 084 SHWACHMAN H PEDIATRICS 30 389 962 085 SHWACHMAN H IN: SLEISENGER MH 1206 973 086 SHWACHMAN H PEDIATRICS 55 86 975 087 SHWACHMAN H AM J DIS CHILD 91 223 956 088 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 089$ SHWACHMAN H IN: SHIRKEY HC 964 090 SHWACHMAN H CLIN PEDIATR 14 1115 975 091 SHWACHMAN H IN: LEVINE SZ 249 955 092 SHWACHMAN H PEDIATRICS 46 335 970 093 SIEGEL B OBSTET GYNECOL 16 438 960 094 SIEGENTHALER P SCHWEIZ MED WOCHENSCHR 43 1528 963 095 SIEGENTHALER P HELV MED ACTA 965 096 STEJFA M BRATISL LEK LISTY 51 523 969 097 TAUSSIG LM RADIOLOGY 106 369 973 098 TOMASHEFSKI JF CHEST 56 270 969 099 TOMASHEFSKI JF CHEST 57 28 970 100 TOW A AM J DIS CHILD 87 192 954 101 TREVER RW ARCH INTERN MED 106 253 960 102 TYSON KRT J PEDIATR SURG 3 271 968 103 WANG CI CF CLUB ABST 51 972 104 WARING WW AM REV RESPIR DIS 104 166 971 105 WARWICK WJ MOD PROBL PEDIATR 10 353 967 106 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 107 WARWICK WJ J ASTHMA RES 5 277 968 108 WILMSHURST EG PEDIATRICS 55 75 975 CT 1 SCHWACHMAN H N ENGL J MED 296 1519 977 2 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 3 FINK RJ CHEST 74 643 978 4 ANDREWS C ANN INTERN MED 88 128 978 5 GORDEUK V N ENGL J MED 299 1137 978 6 ANON BR MED J 2 626 979 7 SEALE TW J CLIN MICROBIOL 9 72 979 8 HOWIE AD SCOTT MED J 24 193 979 9 SHWACHMAN H CLIN CHEM 25 158 979 10 GOLDBERG RT ARCH PHYS MED REHABIL 60 369 979 11 SANTAGNESE PAD AM J MED 66 121 979 12 HAHN TJ J PEDIATR 94 38 979 13 NEWMAN AJ J PEDIATR 94 594 979 14 PENNINGTON JE J INFECT DIS 139 396 979 15 ALLAN JL MED J AUST 1 600 980 16 HOLSCLAW DS CLIN CHEST MED 1 407 980 17 PARIENTE EA GASTROENTEROL CLIN BIOL 4 275 980 18 WARWICK WJ J CHRON DIS 33 685 980 19 HODSON ME THORAX 35 801 980 20 DAVIS PB AM J MED 69 643 980 21 SANDERS JS CHEST 77 226 980 22 WILLI UV AM J ROENTGENOL 134 1005 980 23 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 24 FICK RB CLIN CHEST MED 2 91 981 25 PASSERO MA CLIN PEDIATR 20 264 981 26 CAVALIER SJ NEUROLOGY 31 714 981 27 BLACKWOOD LL INFECT IMMUN 32 443 981 28 MULLARKEY MF MED CLIN NORTH AM 65 977 981 29 WELCH MJ PEDIATRICS 67 664 981 30 JACOBS WH AM J GASTROENTEROL 76 342 981 31 LAMBERT JR GASTROENTEROLOGY 80 169 981 32 PARK RW GASTROENTEROLOGY 81 1143 981 33 VILASECA J AM J DIS CHILD 135 667 981 34 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 35 HODSON ME EUR J PEDIATR 137 117 981 36 EVENSEN SA ACTA MED SCAND 209 141 981 37 MALER T J BIOL CHEM 256 1420 981 38 COLTEN HR N ENGL J MED 304 831 981 39 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 40 KISTLER I HELV PAEDIATR ACTA 36 495 982 41 OHMAN DE INFECT IMMUN 37 662 982 42 SEVENS C ANN BIOL CLIN 40 527 982 43 RIVERA M AM REV RESPIR DIS 126 833 982 44 MCGUIRE S IR J MED SCI 151 253 982 45 WIESEMANN HG ARCH OTO RHINO LARYNGOL 235 329 982 46 MASARYK TJ DIG DIS SCI 28 874 983 47 PHELAN MS CLIN RADIOL 34 573 983 48 JEFFREY I J CLIN PATHOL 36 1292 983 49 NEVILLE E THORAX 38 929 983 50 DELUMLEY L ARCH FR PEDIATR 40 723 983 51 GUIDOTTI TL RESPIRATION 44 351 983 52 JOHNSON SR OBSTET GYNECOL 61 S 2 983 53 DAVIS PB AM REV RESPIR DIS 128 34 983 54 ROSENBERG E KLIN PAEDIATR 195 323 983 55 HANLY JG BR MED J 286 1411 983 56 HODSON ME BR MED J 286 1381 983 57 IANNUCCI A HUM PATHOL 15 278 984 58 RABKIN CS ANN NEUROL 15 608 984 59 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 60 COWEN L PSYCHOSOM MED 46 363 984 61 LEWIS MI S AFR MED J 65 641 984 62 HODGES P J AM DIET ASSOC 84 664 984 63 FITZPATRICK SB CHEST 86 863 984 64 GUGGER M SCHWEIZ MED WOCHENSCHR 114 620 984 65 RALPH DD AM REV RESPIR DIS 129 641 984 66 HANDELSMAN DJ N ENGL J MED 310 3 984 67 HANDELSMAN DJ N ENGL J MED 310 1670 984 68 SPRINCE NL N ENGL J MED 310 375 984 69 CRAWFORD AM RHEUMATOL INT 5 283 985 70 LESTER LA SEM RESPIR MED 6 285 985 71 AHMAD HR ATEMWEGS LUNGENKRANKH 11 366 985 72 SWOBODNIK W J CLIN ULTRASOUND 13 469 985 73 SEAMAN WB HOSP PRACT 20 123 985 74 HUNT B THORAX 40 23 985 75 HANLY JG Q J MED 56 377 985 76 GOLDBERG RT ARCH PHYS MED REHABIL 66 492 985 77 ROTH RM SOUTH MED J 78 573 985 78 ISPIZUA AU MED CLIN 85 628 985 79 FRIEDMAN HZ GASTROENTEROLOGY 88 808 985 80 REITER EO J PEDIATR 106 21 985 81 STOLLINGER O WIEN MED WOCHENSCHR 135 307 985 82 OCONNOR KW ARCH INTERN MED 145 153 985 83 SCHANKER HMJ ARCH INTERN MED 145 2201 985 84 ISPIZUA AU REV CLIN ESP 176 417 985 85 KOHLER JA J CLIN HOSP PHARM 11 21 986 86 SOUTTER VL CLIN GASTROENTEROL 15 137 986 87 FIEDOREK SC CLIN PEDIATR 25 243 986 88 SUMMERS GD BR J RHEUMATOL 25 393 986 89 OJEDA VJ DIS COLON RECTUM 29 567 986 90 BIGGS BG CANCER 57 2441 986 91 RUBINSTEIN S PEDIATRICS 78 473 986 92 BROWN RF SOUTH MED J 79 1430 986 93 PHILLIPS BM J ROY SOC MED 79 44 986 94 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 95 TRIBBLE CG ANN SURG 204 677 986 PN 77142 RN 00723 AN 77147066 AU Alpers-D-H. Seetharam-B. TI Pathophysiology of diseases involving intestinal brush-border proteins. SO N-Engl-J-Med. 1977 May 5. 296(18). P 1047-50. (REVIEW). MJ INTESTINAL-MUCOSA: me. MALABSORPTION-SYNDROMES: me. MEMBRANE-PROTEINS: me. MN ADULT. ANIMAL. CONTRACTILE-PROTEINS: me. CYSTIC-FIBROSIS: me. DIABETES-MELLITUS: me. ENTEROPEPTIDASE: df. EPITHELIUM: en, me. GLYCOLYSIS. GLYCOPROTEINS: me. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-MUCOSA: en, ul. MALABSORPTION-SYNDROMES: en. MEMBRANE-PROTEINS: bi. ORGANOIDS: me. PANCREATITIS: me. RATS. REVIEW. SUCRASE: df. SUPPORT-U-S-GOVT-P-H-S. EPITHELIUM: cy. AB The intestinal brush border is a complex organelle that contains hydrolytic enzymes, receptors, other proteins involved in transport and contractile proteins. In this article, we review the molecular organization of brush-border proteins within the whole membrane, and discuss their synthesis and turnover. This knowledge provides a framework for understanding the mechanisms by which certain diseases are associated with changes in brush-border proteins. RF 001 MILLER D BIOCHIM BIOPHYS ACTA 52 293 961 002 MAESTRACCI D BIOCHIM BIOPHYS ACTA 433 469 976 003 LOUVARD D J MOL BIOL 106 1023 976 004 ALPERS DH GASTROENTEROLOGY 64 471 973 005 ALPERS DH BIOCHIM BIOPHYS ACTA 401 28 975 006 SEETHARAM B LIFE SCI 18 89 976 007 HOSKINS LC J CLIN INVEST 57 63 976 008 GRAY GM N ENGL J MED 294 750 976 009 HADORN B LANCET 1 812 969 010 FREIBURGHAUS AU N ENGL J MED 294 1030 976 011 SEETHARAM B GASTROENTEROLOGY 70 A78 976 012 GIANNELLA RA GASTROENTEROLOGY 67 965 974 013 ARVANITAKIS C AM J DIG DIS 19 417 974 CT 1 STRYKER JA INT J RADIAT ONCOL BIOL PHYS 4 859 978 2 BERENSON MM CLIN GASTROENTEROL 8 141 979 3 PHILLIPS AD GUT 21 44 980 4 CHENEY CP INFECT IMMUN 28 1019 980 5 KAUFMAN MA J CLIN INVEST 65 1174 980 6 LUK GD J CLIN INVEST 66 66 980 7 KINSELLA TJ SURG GYNECOL OBSTET 151 273 980 8 CARONE FA AM J PHYSIOL 238 F151 980 9 PHILLIPS AD SCAND J GASTROENTEROL 16 65 981 10 LUK GD CANCER RES 41 2334 981 11 GUTSCHMIDT S HISTOCHEMISTRY 71 451 981 12 YOUNG GP BIOCHIM BIOPHYS ACTA 640 131 981 13 HARRIES JT CLIN GASTROENTEROL 11 17 982 14 CATALA J DIGESTION 24 234 982 15 BERGOZ R ANN NUTR METAB 26 291 982 16 CARONE FA J LAB CLIN MED 100 1 982 17 WEISS RG RADIOLOGY 142 507 982 18 GUTSCHMIDT S ARCH DERMATOL RES 273 85 982 19 WILDGRUBE HJ Z GASTROENTEROL 21 628 983 20 ARIMA T CELL TISSUE RES 233 549 983 21 SINCLAIR TS HISTOPATHOLOGY 8 739 984 22 LEVINE JS J CELL BIOL 98 1111 984 23 PRUITT AW PEDIATRICS 76 635 985 24 LINDBERG R J COMP PATHOL 95 65 985 25 STRYKER JA INT J RADIAT ONCOL BIOL PHYS 12 789 986 26 LEBENTHAL E CLIN PERINATOL 13 37 986 27 CASPARY WF CLIN GASTROENTEROL 15 631 986 28 ORENSTEIN SR J PEDIATR 109 277 986 29 TIRUPPATHI C ANAL BIOCHEM 153 330 986 30 SASAKI T ANAT REC 214 353 986 PN 77143 RN 00724 AN 77192224 TI Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 26-1977. SO N-Engl-J-Med. 1977 Jun 30. 296(26). P 1519-26. MJ CYSTIC-FIBROSIS: pa. MN AGE-FACTORS. CASE-REPORT. CYSTIC-FIBROSIS: di. DIAGNOSIS-DIFFERENTIAL. EPIDIDYMIS: pa. HUMAN. INTESTINES: pa. LIVER: pa. LUNG-ABSCESS: pa. LUNG: pa. MALE. MIDDLE-AGE. PANCREAS: pa. RESPIRATORY-INSUFFICIENCY: di. SALIVARY-GLANDS: pa. SEMINAL-VESICLES: pa. SWEAT: an. EX A 46-year-old office worker was admitted to the hospital because of dyspnea. Anatomical diagnoses were cystic fibrosis, involving lungs, salivary glands, pancreas, liver, intestine, epididymis, vas deferens, and seminal vesicles, and marked bronchiectasis with bronchopneumonia, multiple pulmonary abscesses, and bilateral empyema. RF 001 MURTHY MSN OHIO STATE MED J 69 768 973 002 MARKS BL LANCET 1 365 960 003 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 004 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 005 ANDERSON CM ARCH DIS CHILD 35 581 960 006 LANDING BH ARCH PATHOL 88 569 969 007 SHWACHMAN H PEDIATRICS 55 86 975 008 ANDERSEN DH AM J DIS CHILD 56 344 938 009 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 010 PARKINS RA LANCET 2 851 963 011 SHWACHMAN H N ENGL J MED 286 1300 972 012 ESTERLY NB AM J DIS CHILD 123 200 972 013 KAPLAN E N ENGL J MED 279 65 968 014 CZEGLEDY-NAGY E LAB INVEST 35 588 976 015 KOPITO LE PEDIATR RES 10 742 976 CT 1 GORDEUK V N ENGL J MED 299 1137 978 2 EVENSEN SA ACTA MED SCAND 209 141 981 3 SING CF AM J MED GENET 13 179 982 4 GOLDBERG RT ARCH PHYS MED REHABIL 66 492 985 5 OCONNOR KW ARCH INTERN MED 145 153 985 PN 77144 RN 00725 AN 78031151 AU Roy-C-C. Weber-A-M. Morin-C-L. Combes-J-C. Nussle-D. Megevand-A. Lasalle-R. TI Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes. SO N-Engl-J-Med. 1977 Dec 15. 297(24). P 1301-5. MJ BILE: me. CHOLELITHIASIS: me. CYSTIC-FIBROSIS: me. LIPIDS: me. PANCREATIC-EXTRACTS: tu. MN BILE-ACIDS-AND-SALTS: me. BILE: an. CHILD. CHOLESTEROL: me. CHOLIC-ACIDS: an. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: dt. FEMALE. GLYCINE: an. HUMAN. MALE. PHOSPHOLIPIDS: me. SEX-FACTORS. TAURINE: an. AB Because of the increased incidence of gallstones in cystic fibrosis we compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls. Eighteen of the cystic fibrosis group had cholecystograms, and only one had gallstones. In 14 patients with cystic fibrosis who had stopped taking pancreatic enzymes for one week molar percentage of lipid composition accounted for by cholesterol (mean +/- S.E., 16.3 +/- 2.9) and saturation index (2.0 +/- 0.3) were comparable to values of the cholelithiasis group and higher (P less than 0.01) than those of controls. In 12 patients with cystic fibrosis taking pancreatic enzymes, molar percentage of cholesterol (8.6 +/- 1.7) and saturation index (1.0 +/- 0.1) did not differ from those of controls; in cystic fibrosis there was a preponderance of cholic over chenodeoxycholic acid both off (1.7 +/- 0.2) and on (1.9 +/- 0.3) therapy as compared to the cholelithiasis (0.7 +/- 0.1) and control (0.8 +/- 0.0) groups. The glycine/taurine ratio of conjugated bile acids were lower in enzyme-treated patients with cystic fibrosis (3.7 +/- 0.6) than in patients off treatment (6.4 +/- 1.0), but was higher (P less than 0.01) than in controls (1.8 +/- 0.2). Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes. RF 001 CALABRESE C SURGERY 70 413 971 002 VON BERGMANN J CLIN CHIM ACTA 64 241 975 003 ANDRASSY RJ AM J SURG 132 19 976 004 WEBER AM N ENGL J MED 289 1001 973 005 GOODCHILD MC ARCH DIS CHILD 50 769 975 006 WATKINS JB GASTROENTEROLOGY 67 835 974 007 ROVSING H ACTA RADIOL DIAGN STOCKH 14 588 973 008 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 009 COURCHAINE AJ CLIN CHEM 5 609 959 010 BARTLETT GR J BIOL CHEM 234 466 959 011 WEBER AM CLIN CHIM ACTA 39 524 972 012 HOFMANN AF J LIPID RES 3 127 962 013 RUDMAN D J CLIN INVEST 36 530 957 014 ENEROTH P IN: NAIR PP 121 971 015 EVRARD E J LIPID RES 9 226 968 016 ROOVERS J CLIN CHIM ACTA 19 449 968 017 ADMIRAND WH J CLIN INVEST 47 1043 968 018 THOMAS PJ GASTROENTEROLOGY 65 698 973 019 SCHEFFE H BIOMETRIKA 40 87 953 020 WEBER AM GUT 17 295 976 021 HARRIES JT PROC INT CF CONG 7TH 976 022 PALMER RH AM J DIG DIS 21 795 976 023 DOWLING RH GUT 13 415 972 024 HEATON KW BR MED J 3 494 969 025 GARBUTT JT GASTROENTEROLOGY 56 711 969 026 GRUNDY SM GASTROENTEROLOGY 62 1200 972 027 SHAFFER EA N ENGL J MED 287 1317 972 028$ SHAFFER EA J CLIN INVEST 49 828 977 029 REDINGER RN GASTROENTEROLOGY 71 470 976 030 HEPNER GW GASTROENTEROLOGY 66 556 974 031 ISENBERG JN GASTROENTEROLOGY 66 887 974 032 DANZINGER RG N ENGL J MED 286 1 972 033 ADLER RD GASTROENTEROLOGY 68 326 975 034 COYNE MJ J LAB CLIN MED 87 281 976 035 THISTLE JL N ENGL J MED 289 655 973 036 OPPENHEIMER EH J PEDIATR 86 683 975 037 STERN RC GASTROENTEROLOGY 70 645 976 038 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 CT 1 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 2 ROY CC N ENGL J MED 298 352 978 3 BENNION LJ N ENGL J MED 299 1221 978 4 FEIGELSON J NOUV PRESSE MED 8 3029 979 5 PEARLMAN BJ CLIN GASTROENTEROL 8 123 979 6 ROY CC AM J CLIN NUTR 32 2404 979 7 HARRIES JT ARCH DIS CHILD 54 19 979 8 REDINGER RN POSTGRAD MED 65 56 979 9 BOLCK F NATURWISSENSCHAFTEN 66 35 979 10 SANTAGNESE PAD AM J MED 66 121 979 11 COX KL J PEDIATR 94 488 979 12 STREMMEL W MUNCH MED WOCHENSCHR 121 1721 979 13 ROY CC PROC SOC EXP BIOL MED 161 105 979 14 CAPRON JP GASTROENTEROL CLIN BIOL 4 63 980 15 SHMERLING DH NOUV PRESSE MED 9 1034 980 16 DURIE PR GUT 21 778 980 17 SHAFFER EA CAN J SURG 23 517 980 18 GORIUP U HELV PAEDIATR ACTA 35 177 980 19 BALISTRERI WF J PEDIATR 96 582 980 20 GAREL L PEDIATR RADIOL 11 75 981 21 MITCHELL EA AUST PAEDIATR J 17 207 981 22 MITCHELL EA AUST PAEDIATR J 17 89 981 23 JACOBS WH AM J GASTROENTEROL 76 342 981 24 LAMBERT JR GASTROENTEROLOGY 80 169 981 25 PARK RW GASTROENTEROLOGY 81 1143 981 26 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 27 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 28 ABDULKARIM FW GASTROENTEROLOGY 82 758 982 29 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 30 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 31 GRUNDY SM SEM LIVER DIS 3 97 983 32 BOUCHIER IAD CLIN GASTROENTEROL 12 25 983 33 DORVIL NP AM J CLIN NUTR 37 221 983 34 SHAFER AD AM SURGEON 49 314 983 35 TAZAWA Y ARCH DIS CHILD 58 819 983 36 DOHERTY DE SOUTH MED J 76 1580 983 37 BASS S GASTROENTEROLOGY 84 1592 983 38 TAZAWA Y J PEDIATR GASTROENTEROL NUTR 3 378 984 39 ABRAMS CK J CLIN INVEST 73 374 984 40 DAVIS PB CHEST 85 802 984 41 HUBBARD VS SEM RESPIR MED 6 299 985 42 DARLING PB PEDIATR RES 19 578 985 43 ROBB TA GUT 26 1246 985 44 SETCHELL KDR CLIN CHIM ACTA 151 101 985 45 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 46 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 47 LUSSIERCACAN S CLIN INVEST MED 9 94 986 48 WEIZMAN Z GUT 27 1043 986 49 LEROY C DIG DIS SCI 31 911 986 50 BRAGGION C ARCH DIS CHILD 62 349 987 PN 77145 RN 00726 AN 77171258 AU Wilson-C-B. Fudenberg-H-H. TI Ciliary dyskinesia factors in cystic fibrosis and asthma. SO Nature. 1977 Mar 31. 266(5601). P 463-4. MJ ASTHMA: bl. BLOOD-PROTEINS: ip. CILIA: de. CYSTIC-FIBROSIS: bl. MN ANTIGENIC-DETERMINANTS. BIOLOGICAL-ASSAY. BLOOD-PROTEINS: im, pd. COMPARATIVE-STUDY. COMPLEMENT-3. EPITHELIUM: de. HUMAN. MOVEMENT: de. TRACHEA: de. SUPPORT-U-S-GOVT-NON-P-H-S. EPITHELIUM: cy. EX Using a modified rabbit tracheal bioassay, we have confirmed that serum from individuals with bronchial asthma and serum from homozygotes and heterozygotes for CF cause ciliary dyskinesia, whereas normal healthy control sera do not. To evaluate the specificity of the ciliary dyskinesia activity (CDA) detected, we bioassayed serum from eight patients with bronchial asthma. We found that all eight asthmatic sera caused a ciliary dyskinesia reaction; however, sera from asthmatics generally caused a subsequent ciliostasis (cessation of ciliary beating). Concurrent evaluation of all sera for CDA by bioassay and for cystic fibrosis protein (CFP) by electrofocusing showed that all CDA-positive sera except seven of the eight asthmatic sera were also positive for CFP (our specific marker for the CF gene). These two finding indicated that the activity detected in patients with asthma and other respiratory diseases could be a substance different from a CF-specific CDF. To test our hypothesis, we set out to purify the CDAs in asthmatic, CF homozygote and OHCF sera. Representative results clearly indicate that the asthmatic CDA is found in DEAE-II and Sephadex G-200 Fraction II, whereas the CDA found in CF and OHCF sera elutes in DEAE-I and Sephadex G-200 Fractions III and IV (weak CDA). We conclude that the CDAs in asthma and CF are distinct substances. Our findings may facilitate purification of what we feel is a CF-specific CDF from CF and OHCF serum. Purification is a prerequisite to elucidating the exact nature of this substance and its role in the pathophysiology of CF. RF 001 SPOCK A PEDIATR RES 1 173 967 002 CONOVER JH PEDIATR RES 7 220 973 003 WILSON GB CLIN RES 24 295 976 004 WILSON GB PEDIATR RES 11 143 977 005 CONOVER JH LANCET 1 1194 973 006 CONOVER JH LIFE SCI 14 253 974 007 CONOVER JH LANCET 2 1501 973 008 CHERRY JD J PEDIATR 79 937 971 009 CHRISTENSEN J IN: LAWSON D PROC 5TH INT CF 49 969 010 POSSELT HG Z KINDERHEILK 110 93 971 011 WILSON GB PEDIATR RES 9 635 975 012 WILSON GB TEX REP BIOL MED 34 51 976 013 WILSON GB PEDIATR RES 10 1001 976 014 WILSON GB PEDIATR RES 11 139 977 016 WILSON GB SCAND J IMMUNOL 5 829 976 017 DI SANTAGNESE PA N ENGL J MED 295 534 976 020 BOWMAN BH SCIENCE 164 325 969 021 BARNETT DR PEDIATR RES 8 687 974 022 LOCKHART LH TEX REP BIOL MED 31 631 973 023$ BERATIS NG PEDIATR RES 8 687 974 024 WILSON GB PEDIATR RES 10 87 976 025 WILSON GB CLIN RES 25 34 977 027 SHAPIRA E PEDIATR RES 10 812 976 CT 1 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 2 WILSON GB J LAB CLIN MED 92 463 978 3 WILL PC PEDIATR RES 13 1129 979 4 WILSON GB PEDIATR RES 13 1079 979 5 AFZELIUS BA INT REV EXP PATHOL 19 1 979 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 LEWISTON NJ CHEST 80 389 981 8 MCNEELY MC PEDIATR RES 16 21 982 9 DULFANO MJ THORAX 37 646 982 10 DULFANO MJ CLIN SCI 63 393 982 11 GREENSTONE M EUR J RESPIR DIS 64 480 983 12 SMALLMAN LA THORAX 39 663 984 13 SCAMBLER P HUM GENET 69 250 985 14 GREENSTONE M BR J DIS CHEST 79 9 985 PN 77146 RN 00727 AN 77147208 AU Peterson-J-A-Jr. Harry-R-D. TI Intussusception in patients with cystic fibrosis. SO Nebr-Med-J. 1977 Apr. 62(4). P 93-5. MJ CYSTIC-FIBROSIS: co. INTUSSUSCEPTION: et. MN ADOLESCENCE. CASE-REPORT. HUMAN. MALE. AB A 16-year-old boy with cystic fibrosis had intermittent episodes of abdominal pain probably due to recurrent intussusceptions. This type of bowel obstruction is probably more frequent in patients with fibrocystic disease. Thick, vascid, inspissated feces appears to be the cause of the intussusceptions. Prevention of the inspissation may reduce the incidence of these obstructions. The treatment for this problem is the same as in other (noncystic) patients. RF 001 HOLSCLAW DS PEDIATRICS 48 51 971 002 SELIGER G AM J DIG DIS 17 934 972 003 KOPEL FB GASTROENTEROLOGY 62 483 972 004 PONKA JL SURG GYNECOL OBSTET 124 99 967 005 MCLAUGHLIN CW JR ARCH SURG 56 48 948 006 ELLIS H IN: MAINGOT R 1525 969 PN 77147 RN 00728 AN 77100825 AU Geller-A. Gilles-F. Shwachman-H. TI Degeneration of fasciculus gracilis in cystic fibrosis. SO Neurology (Minneap). 1977 Feb. 27(2). P 185-7. MJ CYSTIC-FIBROSIS: pa. NERVE-DEGENERATION. SPINAL-CORD: pa. MN ADOLESCENCE. AUTOPSY. CHILD-PRESCHOOL. CHLORAMPHENICOL: ae. CYSTIC-FIBROSIS: co, dt. HUMAN. OPTIC-NEURITIS: et. SUPPORT-U-S-GOVT-P-H-S. AB The spinal cords of 19 percent of patients dying with cystic fibrosis after 5 years of age showed posterior column degeneration. The risk did not appear to increase with advancing age. None of these patients had findings of pernicious anemia or spinocerebellar degeneration. The lesions had been undetected clinically. It is possible that nutritional, toxic, or hereditary factors may play a role in producing this lesion. RF 001 SPENCE AM NEUROLOGY (MINN) 21 386 971 002 GILLES FH J NEUROPATH EXP NEUROL 25 138 966 003 HARLEY RD TRANS AM ACAD OPHTHALMOL OTOL 74 1011 970 004 SUNG JH J NEUROPATH EXP NEUROL 23 567 964 005 EINARSON L EFFECT OF CHRONIC VITAMIN E D 938 006 PENTSCHEW A ACTA NEUROPATH (BERL) 1 313 962 007 SWAIMAN KF NEUROLOGY (MINN) 23 474 973 008 SWAIMAN KF NEUROLOGY (MINN) 25 1084 975 009 CAMPBELL AW BRAIN 20 488 897 CT 1 TOMASI LG NEUROLOGY 29 1182 979 2 FISCHER EG J PEDIATR 95 385 979 3 SHAPIRA Y ANN NEUROL 10 266 981 4 CAVALIER SJ NEUROLOGY 31 714 981 5 NELSON JS J NEUROPATHOL EXP NEUROL 40 166 981 6 ROSENBLUM JL N ENGL J MED 304 503 981 7 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 8 HARDING AE ANN NEUROL 12 419 982 9 HOWARD L AM J CLIN NUTR 36 1243 982 10 SAITO K ACTA NEUROPATH (BERL) 58 187 982 11 GUGGENHEIM MA J PEDIATR 100 51 982 12 GUGGENHEIM MA ANN NY ACAD SCI 393 84 982 13 GUGGENHEIM MA J PEDIATR 102 577 983 14 BIERI JG N ENGL J MED 308 1063 983 15 MESSENHEIMER JA ANN NEUROL 15 499 984 16 BERTONI JM NEUROLOGY 34 1046 984 17 CARPENTER D SEM NEUROL 5 283 985 18 WILLISON HJ J NEUROL NEUROSURG PSYCHIAT 48 1097 985 19 HARDING AE N ENGL J MED 313 32 985 20 KRENDEL DA NEUROLOGY 37 538 987 PN 77148 RN 00729 AN 78052421 AU Liberi-P-T. TI Cystic fibrosis. SO Nurs-Care. 1977 Dec. 10(12). P 22-5, 30. MJ CYSTIC-FIBROSIS. MN CASE-REPORT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: nu. HOME-CARE-SERVICES. HUMAN. MALE. PROFESSIONAL-FAMILY-RELATIONS. EX The nurse is an indispensable member of the team of health professionals who provide the comprehensive treatment needed by patients with the complex, emotionally, and physically demanding disease, cystic fibrosis. Comprehensive medical management by a health care team composed of the physician, nurse, social worker, physical therapist, nutritionist, and psychologist is essential for patients with CF and other forms of obstructive pulmonary disease. PN 77149 RN 00730 AN 78031677 AU Clegg-K-M. TI Nitrogen balance trials with cystic fibrosis patients. SO Nutr-Metab. 1977. 21 Suppl 1. P 77-9. MJ CYSTIC-FIBROSIS: me. NITROGEN: me. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. DIETARY-PROTEINS: ad. FEMALE. HUMAN. HYDROLYSIS. MALE. PANCREATIC-EXTRACTS: ad. EX Preliminary nitrogen balance trials have been carried out with six patients known to have pancreatic insufficiency to investigate the utilisation of protein from a general diet; protein from a general diet plus exogenous pancreatic extract; and pre-digested protein, comprising 85% of dietary nitrogen intake, in the absence of a pancreatic supplement. In all cases, the addition of pancreatic supplement to the general diet improved protein digestibility and utilisation. Substituting 85% of the dietary protein with the enzymic casein hydrolysate resulted in further improvement in nitrogen balance (but not digestibility) for five of the patients. Urinary nitrogen levels of the casein hydrolysate diet tended to be lower, which accounts for the finding above. With the acid-hydrolysed protein, urinary nitrogen was higher when compared with the excretion from the partial casein hydrolysate and provided smaller positive nitrogen balances. These preliminary findings indicate that, for some patients with pancreatic insufficiency, partial hydrolysates have greater potential for nitrogen retention than the administration of exogenous pancreatic extracts with intact protein. It is suggested that a daily supplement of pre-digested protein might be advantageous for CH children to ensure that minimum protein requirements are met. RF 001 CLEGG KM J FD TECHNOL 9 425 974 PN 77150 RN 00731 AN 77233274 AU Buganski-R. TI Regional pediatric pulmonary centers in Ohio. SO Ohio-State-Med-J. 1977 Jun. 73(6). P 363-5,369. MJ CYSTIC-FIBROSIS: th. HOSPITALS-SPECIAL. RESPIRATORY-TRACT-DISEASES: th. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. OHIO. REGIONAL-MEDICAL-PROGRAMS. AB Acute and chronic respiratory disorders play a large role in the practitioner's daily care of infants, children, and adolescents. To assist with the more sophisticated problems in these diseases, the Ohio Committee on Pulmonary Standards has established pediatric pulmonary care centers distributed over the State of Ohio. Referral to these centers may be requested by the practicing physician. To illustrate the validity of this concept, this article reviews the care given to patients with cystic fibrosis (CF), the improved prognosis for this disease entity, and the advantages of expertise and of centralized care. RF 001$ KENDIG EL JR IN: DOERSHUK CF 971 002 BLACKFAN KD J PEDIATR 13 627 938 003 ANDERSEN DH AM J DIS CHILD 56 344 938 004 MATTHEWS LW J PEDIATR 65 558 964 005 DI SANTAGNESE PA AM J MED 15 777 953 006 DOERSHUK CF IN: GREEN M 707 965 007 GIBSON LE PEDIATRICS 23 545 959 008 STEPHAN U PEDIATRICS 55 474 976 009 ELLIOTT RB PEDIATRICS 57 474 976 010 MATTHEWS LW PEDIATRICS 39 176 967 011 BAU SK PEDIATRICS 48 605 971 012 WARWICK WJ STATISTICAL ANALYSIS OF NATIO 974 PN 77151 RN 00732 AN 77172193 AU Jaffe-B-F. Strome-M. Khaw-K-T. Shwachman-H. TI Nasal polypectomy and sinus surgery for cystic fibrosis--a 10 year review. SO Otolaryngol-Clin-North-Am. 1977 Feb. 10(1). P 81-90. MJ CYSTIC-FIBROSIS. NASAL-POLYPS: su. SINUSITIS: su. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg. ETHMOID-BONE: su. HUMAN. MAXILLARY-SINUS: su. NASAL-BONE: su. NASAL-POLYPS: fg. NASAL-SEPTUM: su. NEOPLASM-RECURRENCE-LOCAL. OROANTRAL-FISTULA: su. PREOPERATIVE-CARE. EX Patients with cystic fibrosis are likely to develop nasal polyps and pansinusitis. This presentation concerns the results of various surgical procedures used to treat nasal polyposis in cystic fibrosis. At present recurrent nasal polyposis is still the major problem following surgery. The literature does not deal specifically with comparisons of different surgical procedures aimed at controlling nasal obstruction or with the surgical management of sinus disease in cystic fibrosis patients. Perhaps this lack reflects the unsatisfactory results that would have to be reported, since a high recurrence rate is expected. Also the prolonged survival in these children means that more of them are reaching adolescence and adulthood with an increasing risk for nasal obstruction and nasal polyposis. RF 001 BAKER DC JR OTOL CLIN NORTH AM 3 257 970 002 GHARIB R AM J DIS CHILD 108 499 964 003 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 004 SHWACHMAN H PEDIATRICS 30 389 962 005 SINGH K J LARYNGOL OTOL 85 185 971 006 WALTERS TR AM J DIS CHILD 124 641 972 CT 1 SCHRAMM VL LARYNGOSCOPE 90 1488 980 2 JACOBS RL J ALLERGY CLIN IMMUNOL 67 253 981 3 BRADLEY PJ CLIN OTOLARYNGOL 7 87 982 4 JOHNSON JT POSTGRAD MED 72 87 982 5 FAIRBANKS DNF OTOLARYNGOL HEAD NECK SURG 90 527 982 6 STERN RC AM J DIS CHILD 136 1067 982 7 SHAPIRO ED J INFECT DIS 146 589 982 8 SHWACHMAN H J PEDIATR SURG 18 570 983 9 FAIRBANKS DNF PEDIATR INFECT DIS 4 S 75 985 10 DAVIS PB SEM RESPIR MED 6 319 985 11 REILLY JS LARYNGOSCOPE 95 1491 985 12 DAVID TJ J ROY SOC MED 79 23 986 13 MYERS EN LARYNGOSCOPE 96 911 986 PN 77152 RN 00733 AN 77172178 AU Kramer-R-I. TI Otorhinolaryngologic complications of cystic fibrosis. SO Otolaryngol-Clin-North-Am. 1977 Feb. 10(1). P 205-8. MJ CYSTIC-FIBROSIS: co. OTORHINOLARYNGOLOGIC-DISEASES: et. MN ADOLESCENCE. CELL-MEMBRANE-PERMEABILITY. CHILD. CYSTIC-FIBROSIS: et, pp. HUMAN. LARYNGEAL-NEOPLASMS: et, su. NASAL-POLYPS: et, su. OTITIS-MEDIA: dt, et. SALIVARY-GLANDS: se. AB Inasmuch as cystic fibrosis is a generalized exocrinopathy and affects all the mucous glands in the body, the complications of this disease involving the ear, nose, and throat are obviously protean. This presentation includes a discussion of chronic ear infections relative to the diagnosis of cystic fibrosis, a discussion of nasal polyps and their treatment, salivary gland involvement in cystic fibrosis, and the results of sialography. The effects of respiratory therapy upon the upper respiratory tract and the problems of general anesthesia in patients who undergo surgical procedures are also discussed. CT 1 MYER CM PEDIATRICS 72 766 983 2 TODD NW INT J PEDIATR OTORHINOLARYNGO 10 27 985 3 DAVID TJ J ROY SOC MED 79 23 986 PN 77153 RN 00734 AN 77256116 AU Holsclaw-D-S. Tecklin-J-S. TI A critical evaluation of bronchial hygiene in pediatric pulmonary disease. SO Pediatr-Ann. 1977 Aug. 6(8). P 550-6. MJ BRONCHI. CYSTIC-FIBROSIS: th. MN AEROSOLS. ANTIBIOTICS: tu. BRONCHODILATOR-AGENTS: tu. CHILD. CYSTIC-FIBROSIS: dt, pp, su. DRAINAGE. EXPECTORANTS: tu. HUMAN. RESPIRATORY-THERAPY. RESPIRATION. AB The variables inherent in analyzing the effectiveness of modalities used to achieve bronchial hygiene are numerous. Treatments may be given in a variety of settings, including intensive-care units, pediatric wards, respiratiory therapy departments, physical therapy departments, chronic-care hospitals, special education centers, and patient homes. Treatments are administered by a variety of medical professionals, allied health personnel, and lay volunteers. Motivation of the care providers as well as of the patients may vary greatly. Also, efforts are needed to study patients at comparable stages in their growth and development and in the progression of their particular diseases. Rather than perpetuating the unquestioning use of these various treatments, a series of carfully constructed and controlled studies done in collaborative fashion need to be performed. Until that time, the recommendations we have outlined dealing with each of the various segments of bronchial hygiene may serve as useful guidelines. RF 001 DENTON R AM REV RESPIR DIS 86 41 962 002 LORIN MI AM J PHYS MED 50 215 971 003 WONG JW AM REV RESPIR DIS 113 97 976 004 MOTOYAMA EK IN: MANGOS JA 335 973 005 TECKLIN JS PHYS THER 55 1081 975 006 FELDMAN J AM REV RESPIR DIS 113 272 976 007 LEVISON H IN: MANGOS JA 3 976 008 WEBB WR J THORAC CARDIOVASC SURG 44 330 962 009 REAS HW J PEDIATR 62 31 963 010 REAS HW SOUTH MED J 56 1271 963 011 HUANG NN CF CLUB ABST 5 12 964 012 STAMM SJ DIS CHEST 47 414 965 013 DENTON R AM REV RESPIR DIS 95 643 967 014 LIEBERMAN J JAMA 205 312 968 015 ADHIKARI PK CF CLUB ABST 9 29 968 016 LIFSCHITZ MI CF CLUB ABST 9 48 968 017 SOLOMONS CC PEDIATRICS 47 384 971 018 BUSCH-PETERSEN D Z ERKR ATMUNGSORGANE 143 140 975 019 DIETZSCH HJ PEDIATRICS 55 96 975 020 GANDEVIA B ARCH DIS CHILD 34 511 959 021 LIFSCHITZ MI AM REV RESPIR DIS 99 399 969 022 FEATHERBY EA CAN MED ASSOC J 102 835 970 023 ZAPLETAL A PEDIATRICS 48 64 971 024 LANDAU LI J PEDIATR 82 863 973 025 CHANG N AM REV RESPIR DIS 106 867 972 026 SHAPIRO GG PEDIATRICS 58 740 976 027 GIBBS GE ANTIBIOT MED 2 332 956 028 GIBBS GE AM J DIS CHILD 93 65 957 029 CAMPBELL RA CF CLUB ABST 10 31 969 030 CAMPBELL RA CF CLUB ABST 12 30 971 031 LIFSCHITZ MI CLIN PHARMACOL THER 12 91 971 032 BARAN D INT J CLIN PHARMACOL 12 336 975 033 OLSON DL RESPIR CARE 21 333 976 034 TECKLIN JS PHYS THER 56 999 976 035 ANON AM REV RESPIR DIS 110 169 974 036 KEENS TG PEDIATR RES ABST 11 573 977 PN 77154 RN 00735 AN 77124776 AU Selekman-J. TI Cystic fibrosis: what is involved in the home treatment program for these children, adolescents and young adults?. SO Pediatr-Nurs. 1977 Mar-Apr. 3(2). P 32-5. MJ CYSTIC-FIBROSIS: th. HEALTH-EDUCATION. HOME-NURSING. MN ADOLESCENCE. ADULT. BREATHING-EXERCISES. CHILD. CYSTIC-FIBROSIS: dh. HUMAN. EX Cystic fibrosis is one of the most frequent of the serious chronic diseases of childhood and adolescence and is the most common genetic disease among Caucasian children. Medical research and home treatment programs have greatly improved the outlook for these children. The nursing profession has played a significant role in this progress at home, the hospital, the school, and the community. Our goal is to teach the treatments that may help maintain the health of these children for as long as possible and, in the face of this, to help them and their families to lead as normal a life as possible. PN 77155 RN 00736 AN 84192886 AU Matthew-D-J. Warner-J-O. Chrispin-A-R. Norman-A-P. TI The relationship between chest radiographic scores and respiratory function tests in children with cystic fibrosis. SO Pediatr-Radiol. 1977. 5(4). P 198-200. MJ CYSTIC-FIBROSIS: ra. RESPIRATORY-FUNCTION-TESTS. THORACIC-RADIOGRAPHY. MN ADOLESCENCE. CHILD. CYSTIC-FIBROSIS: pp. FORCED-EXPIRATORY-VOLUME. FUNCTIONAL-RESIDUAL-CAPACITY. HUMAN. VITAL-CAPACITY. AB Chest radiographic scores and respiratory function on 80 sets of results from 50 patients with Cystic Fibrosis were analyzed. Chest radiographic scores were assessed independently using the method of Chrispin and Norman. Respiratory function tests were found to correlate well with the chest radiographic score, the best correlation being with the forced expiratory volume in 0.75 sec. to forced vital capacity ratio F.E.V. 0.75/ F.V.C. (r = -0.674 n = 80 p less than 0.001). RF 001 BEIER FR AM REV RESPIR DIS 94 430 966 002 COGSWELL JJ BR J DIS CHEST 69 177 975 003 COGSWELL JJ BR J DIS CHEST 69 177 975 004 COGSWELL JJ BR J DIS CHEST 69 177 975 005 COOPERMAN EM CAN MED ASSOC J 105 580 971 006 CHRISPIN AR PEDIATR RADIOL 2 101 974 007 DOERSHUK CF J PEDIATR 65 677 964 008 DUBOIS AB J CLIN INVEST 35 322 956 009 FEATHERBY EA AM REV RESPIR DIS 102 737 970 010 GODFREY S ARCH DIS CHILD 46 144 971 011 KARLSBERG P ACTA PAEDIATR SCAND 49 345 960 012 MEARNS MB ARCH DIS CHILD 43 528 968 013 REILLY BJ RADIOLOGY 98 281 971 014 SHWACHMAN H AM J DIS CHILD 96 6 958 015 TAUSSIG LM J PEDIATR 82 380 973 016 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 KRAEMER R HELV PAEDIATR ACTA 34 417 979 2 BRASFIELD D PEDIATRICS 63 24 979 3 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 4 BRASFIELD D AM J ROENTGENOL 134 1195 980 5 VANDERPUT JM PEDIATR RADIOL 12 57 982 6 KRAEMER R EUR J PEDIATR 138 172 982 7 GOODING CA J PEDIATR 105 384 984 PN 77156 RN 00737 AN 78011299 AU Rao-G-J. Spells-G. Nadler-H-L. TI Enhanced UDP-galactose:glycoprotein galactosyl transferase activity in cultivated skin fibroblasts from patients with cystic fibrosis and its possible relationship to the pathogenesis of the disease. SO Pediatr-Res. 1977 Sep. 11(9 Pt 1). P 981-5. MJ CYSTIC-FIBROSIS: en. GALACTOSYLTRANSFERASES: me. SKIN: en. MN CELLS-CULTURED. CULTURE-MEDIA. CYSTIC-FIBROSIS: fg. ENZYME-ACTIVATION. FIBROBLASTS. HETEROZYGOTE. HOMOZYGOTE. HUMAN. OVALBUMIN. PEPTIDES: pd. SUPPORT-U-S-GOVT-P-H-S. URIDINE-DIPHOSPHATE-GALACTOSE. AB Homogenates of cultivated skin fibroblasts derived from patients with cystic fibrosis had a higher level of UDP-galactose:ovalbumin galactosyl transferase activity compared to fibroblasts derived from control subjects. The activity in control subjects was 1.82 +/- 0.43 nmol galactose transferred/hr/mg protein, whereas the activity in fibroblasts of patients was 2.95 +/- 0.77. The difference was significant at P less than 0.01. Activity in the fibroblasts of obligate heterozygotes was 2.15 +/- 0.60. The difference between the activities in fibroblasts of heterozygotes and patients was significant at P less than 0.05. The activity in control fibroblasts could be enhanced by basic polypeptides like polylysine, polyarginine, histone, and protamine but not by neutral or acidic polypeptides. Fibroblasts from patients released significantly higher amounts of a soluble form of the enzyme activity into the culture medium than control fibroblasts. RF 001 ARMITAGE P STATISTICAL METHODS IN MEDICA 971 002 BAKER AP ARCH BIOCHEM BIOPHYS 165 597 974 003 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 004 BOWMAN BH SCIENCE 167 871 970 005 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 006 CONOD EJ PEDIATR RES 9 724 975 007 CONOVER JH PEDIATR RES 7 220 973 008 DANES BS LANCET 1 1061 968 009 DEPPERT W PROC NAT ACAD SCI USA 71 3068 974 010 FORSTNER JF PEDIATR RES 10 609 976 011 FRASER IH BIOCHEM J 156 347 976 012 FREILICH LS BIOCHEM J 146 741 975 013 GUGLER EC J PEDIATR 71 585 967 014 LANGER GA SCIENCE 193 1013 976 015 LOBECK CC IN: STANBURY JB 1605 972 016 LOUISOT P CLIN CHIM ACTA 48 373 973 017 MANGOS JA SCIENCE 158 135 967 018 MANGOS JA PEDIATR RES 1 436 967 019 MATALON R FED PROC 35 1639 976 020 MICHALISZYN GA J BIOL CHEM 251 2531 976 021 NADLER HL NATURE 213 1261 967 022 RAO GJS J PEDIATR 80 573 972 023 RAO GJS PEDIATR RES 8 684 974 024 RAO GJS PEDIATR RES 9 739 975 025 RAO GJS SCIENCE 177 610 972 026 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 027 SCHRAMM M IN: MANGOS JA 215 973 028 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 029 SHAPIRA E PEDIATR RES 10 812 976 030 SPOCK A PEDIATR RES 1 173 967 031 WILSON GB PEDIATR RES 10 87 976 032 WORK TS LAB TECH IN BIOCHEM AND MOLEC 1 34 969 CT 1 BERGER EG EUR J BIOCHEM 90 213 978 2 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 3 AGGETT PJ MONOGR PAEDIATR 10 8 979 4 NAGY EC PEDIATR RES 13 729 979 5 PEARSON RD PEDIATR RES 13 834 979 6 WILSON GB PEDIATR RES 13 1079 979 7 CAMPBELL IM CAN J BIOCHEM 57 1099 979 8 KOTTGEN E KLIN WSCHR 57 199 979 9 JAKEL HP BIOL ZENTRALBL 98 55 979 10 WALSH MMJ PEDIATR RES 14 353 980 11 BENYOSEPH Y BIOCHIM BIOPHYS ACTA 718 172 982 12 FRATES RC PEDIATR RES 17 30 983 13 RUDICK VL J CELL PHYSIOL 115 143 983 14 BUTTERWORTH J BIOCHEM SOC TRANS 13 220 985 15 BRAGANZA JM MED HYPOTHESES 20 233 986 16 BOZON D ARCH BIOCHEM BIOPHYS 249 546 986 PN 77157 RN 00738 AN 77124792 AU Wilson-G-B. Monsher-M-T. Fudenberg-H-H. TI Studies on cystic fibrosis using isoelectric focusing. III. Correlation between cystic fibrosis protein and ciliary dyskinesia activity in serum shown by a modified rabbit tracheal bioassay. SO Pediatr-Res. 1977 Feb. 11(2). P 143-6. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ISOELECTRIC-FOCUSING. MN ANIMAL. ASTHMA: pp. BIOLOGICAL-ASSAY. CILIA: de. CYSTIC-FIBROSIS: pp. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IGG: an. IN-VITRO. METHODS. MUCOUS-MEMBRANE: se. RABBITS. TISSUE-CULTURE. TRACHEA: de. AB We have developed a modified rabbit tracheal bioassay for use in investigating a possible correlation between cystic fibrosis protein (CFP) and ciliary dyskinesia factor (CDF) in human serum. The bioassay requires high standards of tissue selection, and all epithelial tissue must be free of underlying connective tissue. When serum samples were collected and processed carefully and warmed to 37 degrees before assay, CDF could be reliably detected in 31 of 31 sera from cystic fibrosis (CF) homozygotes or obligate heterozygotes in 35 min or less without prior fractionation or concentration of sera, whereas 13 of 14 normal control sera were nonreactive. CDF-positive serum reacts in three consecutive phases: (1) initial increase in ciliary beat frequency, (2) ciliary dyskinesia, and (3) tissue destruction with extrusion of single ciliated cells, mucus, and debris. Our results confirm the association of CDF with cystic fibrosis. The bioassay is not specific for CF, however, when whole sera are bioassayed, since serum from several patients with bronchial asthma also caused ciliary dyskinesia. However, this finding need not preclude using rabbit tracheal ciliated epithelial tissue as an assay for following the purification of CDF. Isoelectric focusing showed that the presence or absence of CFP corresponded with that of dyskinesia activity in all sera tested except for the active samples from seven asthma patients, which were negative for CFP. The results indicate that CFP and CDF may be identical or closely related markers for the CF gene, and suggest that the activity detected by the rabbit tracheal bioassay in sera from patients with asthma and other diseases probably is caused by a substance different from a CF- specific CDF. RF 001 BARNETT DR TEX REP BIOL MED 31 703 973 002 BOWMAN BH IN: MANGOS JA 29 973 003 BOWMAN BH LANCET 1 404 974 004 CHERRY JD J PEDIATR 79 937 971 005 CHEUNG ATW PEDIATR RES 10 144 976 006 CHEUNG ATW PEDIATR RES 10 144 976 007 CHRISTENSEN J IN: LAWSON D PROC 5TH INT CF 49 969 008 CONOVER JH PEDIATR RES 7 220 973 009 CONOVER JH LANCET 1 1194 973 010 CONOVER JH LIFE SCI 14 253 974 011 LOCKHART LH TEX REP BIOL MED 31 631 973 012 MCCOMBS ML TEX REP BIOL MED 31 615 973 013 POSSELT HG Z KINDERHEILK 110 93 971 014 SHWACHMAN H IN: KENDIG EL JR 541 967 015 SPOCK A MOD PROBL PEDIATR 10 200 967 016 SPOCK A PEDIATR RES 1 173 967 022 WILSON GB PEDIATR RES 9 635 975 023 WILSON GB CLIN CHIM ACTA 49 79 973 024 WILSON GB CLIN RES 24 295 976 025 WILSON GB CF CLUB ABST 17 18 976 CT 1 WILSON GB PEDIATR RES 11 986 977 2 WILSON GB PEDIATR RES 11 139 977 3 BOWMAN BH CLIN GENET 12 333 977 4 WILSON GB NATURE 266 463 977 5 CONOVER JH BIOCHEM BIOPHYS RES COMMUN 83 1595 978 6 WILSON GB J LAB CLIN MED 92 463 978 7 VERDUGO P PEDIATR RES 13 131 979 8 WILSON GB J CLIN INVEST 66 1010 980 9 WILSON GB PROC SOC EXP BIOL MED 164 105 980 10 HALLINAN FM CLIN CHIM ACTA 117 103 981 11 BLITZER MG PEDIATR RES 16 203 982 12 RUTLAND J AM REV RESPIR DIS 128 1030 983 PN 77158 RN 00739 AN 77124790 AU Wilson-G-B. Monsher-M-T. Fudenberg-H-H. TI Additional notes on the use of analytic isoelectric focusing for the detection of cystic fibrosis protein in serum [letter]. SO Pediatr-Res. 1977 Feb. 11(2). P 139-41. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ISOELECTRIC-FOCUSING. MN BLOOD-SPECIMEN-COLLECTION. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. HETEROZYGOTE. HUMAN. METHODS. EX In a previous communication, written in response to a letter by Smith et al., we suggested several possible reasons for the inability of Thomas et al. and other investigators to detect cystic fibrosis protein (CFP) using electrofocusing techniques. Thomas and coworkers communicated with us for the purpose of determining possible reasons for their inability to detect CFP by electrofocusing. Key points made during these conversations are reiterated below in a firm attempt to alleviate future problems by inexperienced investigators in their attempts to use analytic electrofocusing to detect CFP. In our communications with Thomas and coworkers we learned that, despite our suggestions, they had not analyzed the samples they screened by either using the equipment of Awdeh et al. or employing our standardized procedure. It is unknown whether or not they exercised the requirements for serum sample collection, processing, and storage. We feel the above reasons are sufficient to explain the reported failure of Thomas et al. to reproduce our results and to detect consistently a CFP in sera from individuals with the CF gene. RF 001 AWDEH ZL NATURE 219 66 968 002 HOWARD A IN: PEETERS H 17 449 969 003 SMITH QT PEDIATR RES 10 999 976 004 THOMAS JM CF CLUB ABST 17 19 976 005 THOMAS JM PEDIATR RES 11 138 977 006 WILLIAMSON AR IN: WEIR DM 973 007 WILSON GB PEDIATR RES 10 1001 976 009 WILSON GB TEX REP BIOL MED 34 51 976 010 WILSON GB PEDIATR RES 10 87 976 011 WILSON GB PEDIATR RES 9 635 975 012 WILSON GB CLIN CHIM ACTA 49 79 973 013 WILSON GB PEDIATR RES 11 143 977 CT 1 THOMAS JM PEDIATR RES 11 1148 977 2 WILSON GB PEDIATR RES 11 986 977 3 WILSON GB NATURE 266 463 977 4 SCHOLEY J PEDIATR RES 12 800 978 5 WILSON GB PEDIATR RES 12 801 978 6 WILSON GB J LAB CLIN MED 92 463 978 7 WILSON GB PEDIATR RES 13 1079 979 8 BROCK DJH HUM GENET 60 30 982 9 SUPER M AM J MED GENET 18 449 984 10 WILSON GB CLIN GENET 26 331 984 PN 77159 RN 00740 AN 77124789 AU Thomas-J-M. Merritt-A-D. Hodes-M-E. TI Electrophoretic analysis of serum proteins in cystic fibrosis [letter]. SO Pediatr-Res. 1977 Feb. 11(2). P 138-9. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. ISOELECTRIC-FOCUSING. MN GAMMA-GLOBULINS: an. HETEROZYGOTE. HUMAN. EX We have attempted to reproduce the isoelectric focusing in thin layer polyacrylamide gels (IEFAG) method specified by Wilson et al. in our laboratory. Since the resolution obtained with their procedure appeared to be inadequate for identifying single protein bands in the relevant region, we have incorporated several methodologic improvements into the isoelectric focusing technique. These improvements have significantly enhanced the resolution and have thus enabled us to demonstrate a greater degree of heterogeneity of serum gamma-globulins than is visible in the paper of Wilson et al. Despite the significant increase in the number of bands resolved by our method, a difference among CF, heterozygote, and control sera in the pH 8.4 - 8.5 region could not be detected. In addition, the two-step IEFAG/disc electrophoresis technique of Altland et al. has been tested in our laboratory. We have also modified this analytic procedure, enabling the fractionation of several small proteins from a portion of the IgG fraction. Again, despite an improvement in resolution which reveals more heterogeneity, no unique protein has been observed consistently in the CF and heterozygote area. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 ANON J PEDIATR 88 711 976 005 WILSON GB PEDIATR RES 9 635 975 006 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 THOMAS JM PEDIATR RES 11 1148 977 2 WILSON GB PEDIATR RES 11 986 977 3 WILSON GB PEDIATR RES 11 139 977 4 WILSON GB PEDIATR RES 12 801 978 PN 77160 RN 00741 AN 77124787 AU Bogart-B-I. TI The biologic activities of cystic fibrosis serum. I. The effects of cystic fibrosis sera and calcium ionophore A 23187 on rabbit tracheal explants. SO Pediatr-Res. 1977 Feb. 11(2). P 131-4. MJ A-23187: pd. ANTIBIOTICS: pd. CALCIUM: pd. CYSTIC-FIBROSIS: bl. TRACHEA: de. MN ANIMAL. CELL-MEMBRANE-PERMEABILITY: de. CILIA: de. CYSTIC-FIBROSIS: pp. EGTA: pd. IN-VITRO. MUCOUS-MEMBRANE: se. OSMOLAR-CONCENTRATION. RABBITS. TISSUE-CULTURE. TRACHEA: me. AB An ionophore A23187-induced increase in membrane permeability to calcium ions in culture medium produced a rabbit tracheal mucociliary response indistinguishable from that caused by cystic fibrosis (CF) sera on three different occasions. Specific chelation of calcium ions with EGTA in the basal medium Eagle (BME) media with no additive or in native CF sera abolished the mucociliary disturbances in all cases. Increased membrane permeability to calcium may be important in the production of the mucociliary response by CF serum factor(s) in the tracheal assay system. RF 001 BLOMFIELD J ARCH DIS CHILD 48 267 973 002 CHERNICK WS J PEDIATR 59 890 961 003 COCHRANE DE PROC NAT ACAD SCI USA 71 408 974 004 CONOVER JH PEDIATR RES 7 220 973 005 DI SANTAGNESE PA PEDIATRICS 12 40 953 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 EIMERL S J BIOL CHEM 249 3991 974 008 FOREMAN JC NATURE 245 249 973 009 GUGLER EC J PEDIATR 71 585 967 010 KAGAYAMA M J CELL BIOL 62 519 974 011 LOBECK CC IN: STANBURY JB 1605 972 012 MANGOS JA SCIENCE 158 135 967 013 MANGOS JA PEDIATR RES 2 378 968 014 MANGOS JA PEDIATR RES 1 436 967 015 PRESSMAN BC FED PROC 32 1698 973 016 REED PW J BIOL CHEM 247 6970 972 017 SATIR P SCIENCE 190 586 975 018 SELINGER Z PROC NAT ACAD SCI USA 71 128 974 019 SPOCK A PEDIATR RES 1 173 967 020 WILLIAMS JA BIOCHEM BIOPHYS RES COMMUN 60 542 974 021 WONG DT ARCH BIOCHEM BIOPHYS 156 578 973 022 WOTMAN S ARCH ORAL BIOL 16 663 971 CT 1 MARIN MG J APPL PHYSIOL 44 900 978 2 CONOVER JH BIOCHEM BIOPHYS RES COMMUN 83 1595 978 3 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 4 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 5 FEIGAL RJ PEDIATR RES 13 764 979 6 VERDUGO P PEDIATR RES 13 131 979 7 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 8 KENNEDY JR PEDIATR RES 14 1173 980 9 ROSCHER AA PEDIATR RES 14 261 980 10 SATIR P ENVIRON HEALTH PERSPECT 35 77 980 11 SANDERSON MJ PEDIATR RES 15 219 981 12 CAMPBELL AK EXPERIENTIA 37 1110 981 13 SORSCHER EJ LANCET 1 368 982 14 MORIARTY CM J MED 13 257 982 15 ANDO T ACTA HISTOCHEM CYTOCHEM 15 812 982 16 BOAT TF PEDIATR RES 16 792 982 17 BOGART BI PEDIATR RES 16 223 982 18 MARIN MG J APPL PHYSIOL 52 198 982 19 CEDER O SCANN ELECTRON MICROSC 1982 723 982 20 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 21 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 22 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 23 MCPHERSON MA CLIN CHIM ACTA 135 181 983 24 KATZ S CELL CALC 5 421 984 25 BARBIERI EJ BR J PHARMACOL 82 199 984 26 RUSSI EW CHEST 86 475 984 27 VONEULER AM LIFE SCI 37 2399 985 28 VONEULER AM EXP MOL PATH 43 142 985 29 AHMED T CHEST 88 S142 985 30 MORRISSEY SM DIGESTION 34 28 986 31 MARIN MG PHARMACOL REV 38 273 986 32 LOFDAHL CG ACTA PHARMACOL TOXICOL 58 91 986 PN 77161 RN 00742 AN 77213827 AU Wood-D-L. Martinez-R. TI The chronically reserpinized rat as a possible model for cystic fibrosis. VI. Synergistic effects of isoproterenol on Ca++ and protein in the submaxillary gland. SO Pediatr-Res. 1977 Jul. 11(7). P 827-32. MJ CALCIUM: me. CYSTIC-FIBROSIS: me. ISOPROTERENOL: pd. PROTEINS: me. RESERPINE: pd. SUBMANDIBULAR-GLAND: me. MN ANIMAL. DISEASE-MODELS-ANIMAL. DRUG-SYNERGISM. ISOPROTERENOL: ad. MALE. RATS. RESERPINE: ad. AB Elevated calcium and protein concentration are a consistent abnormality in submaxillary saliva from patients with cystic fibrosis (CF) and from experimental animal models developed by the chronic administration of either isoproterenol (IPR) or reserpine. The possibility that the effects of the two drugs may be additive was investigated by assessing their combined effects on glandular and salivary C++ and protein in the rat submaxillary gland. Individually, their effects were also assessed in relation to the dose used. Results indicate that: (1) treatment for 7 days with 0.05, 0.50, and 5.0 mg/kg daily dose of reserpine caused, respectively, a 45%, 95%, and 120% increase in glandular Ca++ and a 9.3%, 16.5%, and 37.4% increase in glandular protein; (2) treatment for 7 days with a 5.0 mg/kg daily dose of isoproterenol caused a 138% increase in gland Ca++ and a 12.38% increase in gland protein. Treatment with a 5.0 mg/rat daily dose of this drug caused increases of 166% and 10.3% in gland Ca++ and protein; (3) in experiments involving a combination of the two drugs, isoproterenol was administered in a 5.0 mg/kg daily dose from days 1-7 and reserpine in a 0.5 mg/kg daily dose from days 4-10 of the treatment schedule. This procedure resulted in (a) a 217% increase in gland Ca++ and a 25.7% increase in gland protein; (b) a marked accumulation of a granular, basophilic material in acinar cells and the development of intraductal precipitates; (c) the secretion of turbid saliva with high Ca++ and protein concentrations after a secretory dose of isoproterenol (this type of stimulation also reduced the gland Ca++ (50%) and protein (20%) contents and produced vacuolization in the acinar cells); (d) the secretion of saliva with elevated Ca++ and protein in response to pilocarpine (these elevated concentrations were, however, one-fifth of those obtained after isoproterenol stimulation); (e) the secretion of smaller volume of saliva after both types of stimulation. These findings indicate that both IPR and reserpine have a dose-related and significant effect on submaxillary gland Ca++ and protein and that their individual effects are synergistic. The implications of this synergism for the physiologic state of the submaxillary gland and for the secretory abnormality of cystic fibrosis are discussed. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 BLOMFIELD J ARCH ORAL BIOL 19 1153 974 003 BLOMFIELD J ARCH DIS CHILD 48 267 973 004 BOAT TF PEDIATR RES 8 531 974 005 BOWMAN BH SCIENCE 164 325 969 006 CHERNICK WS ANN NY ACAD SCI 106 698 963 007 CONOVER JH CF CLUB ABST 17 9 976 008 DI SANTAGNESE PA N ENGL J MED 277 1344 967 009 DREISBACH RH PROC SOC EXP BIOL MED 126 279 967 010 EKFORS T LAB INVEST 24 197 971 011 FORSTNER JF PEDIATR RES 10 609 976 012 GUGLER EC J PEDIATR 71 585 967 013 LOWRY OH J BIOL CHEM 193 265 951 014 MANDEL ID AM J DIS CHILD 113 431 967 015 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 016 MARTINEZ JR PEDIATR RES 9 463 975 017 MARTINEZ JR PEDIATR RES 9 470 975 018 MARTINEZ JR J PHARMACOL EXP THER 194 384 975 019 SCHNEYER CA AM J PHYSIOL 203 232 962 020 SCHNEYER LH AM J PHYSIOL 226 821 974 021 TAYLOR PW JR FED PROC ABST 27 709 968 022 TAYLOR PW JR J PHARMACOL EXP THER 156 483 967 CT 1 PERLMUTTER J PEDIATR RES 12 188 978 2 MARTINEZ JR PEDIATR RES 13 1156 979 3 JAKEL HP BIOL ZENTRALBL 98 55 979 4 FORSTNER J AM J PHYSIOL 241 G443 981 5 SHIFFMAN ML PEDIATR RES 16 104 982 6 MULLER RM J ULTRASTRUCT RES 81 377 982 7 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 8 MODRZAKOWSKI MC CAN J MICROBIOL 29 1339 983 9 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 10 BOYD RL PEDIATR RES 18 1028 984 11 JIRAKULSOMCHOK D ARCH ORAL BIOL 29 39 984 12 MULLER RM EXP MOL PATH 40 391 984 13 MULLER RM EXP MOL PATH 41 363 984 14 KUIJPERS GAJ ACTA PHYSIOL SCAND 121 85 984 15 ROOMANS GM ANN NY ACAD SCI 428 121 984 16 SCHNEYER CA J AUTONOM NERV SYST 14 191 985 17 MULLER RM EXP MOL PATH 43 97 985 18 MULLER RM ACTA PHYSIOL SCAND 123 383 985 19 SCHNEYER CA PROC SOC EXP BIOL MED 179 143 985 20 MULLER RM SCANN ELECTRON MICROSC 1985 1583 985 21 HAZLETT D PEDIATR RES 20 1236 986 PN 77162 RN 00743 AN 77124804 AU Buehler-B. Wright-R. Schott-S. Darby-B. Rennert-O-M. TI Ornithine decarboxylase and S-adenosyl methionine decarboxylase in skin fibroblasts of normal and cystic fibrosis patients. SO Pediatr-Res. 1977 Mar. 11(3 Pt 1). P 186-90. MJ CARBOXY-LYASES: me. CYSTIC-FIBROSIS: en. ORNITHINE-DECARBOXYLASE: me. S-ADENOSYLMETHIONINE-DECARBOXYLASE: me. SKIN: en. MN ADULT. CELLS-CULTURED. CHILD-PRESCHOOL. FIBROBLASTS: en. HUMAN. INFANT. MALE. ORNITHINE-AMINOTRANSFERASE: me. PUTRESCINE: pd. AB The key enzymes in the synthesis of the naturally occurring polyamines, ornithine decarboxylase (ODC) and S-adenosyl methionine (SAM) decarboxylase, were investigated during cell growth and aging in fibroblast cultures from normal patients and patients with cystic fibrosis. A linear correlation between increased S-adenosyl methionine activity and putrescine concentration was apparent in all cell lines. A putrescine concentration of 0.8 mM was optimal for enhancement of SAM decarboxylase activity. The passage number of the cell line correlated inversely with maximal putrescine-stimulated SAM decarboxylase activity, earlier passage numbers having the highest specific activity (Fig. 1). No significant differences in basal or putrescine-stimulated SAM decarboxylase activity were noted between normal fibroblast cultures and cells from patients with cystic fibrosis (Fig. 2). SAM decarboxylase activity increased as the cell lines approached confluence. Activity was lowest during exponential growth (Fig. 3). ODC activity was increased during early exponential growth and fell as cells reached confluence (Fig. 4). No differences in ODC activity and putrescine inhibition between the normal and cystic fibrosis cell cultures at equivalent points of exponential growth were noted. RF 001 BACHRACH U FUNCTION OF NATURALLY OCCURRI 973 002 CLARK JL BIOCHEMISTRY 14 4403 975 003 HANNONEN P ACTA CHEM SCAND (A) 29 295 975 004 HOGAN BLM BIOCHEM BIOPHYS RES COMMUN 45 301 971 005 JANNE J BIOCHEM BIOPHYS RES COMMUN 61 399 974 006 LOWRY OH J BIOL CHEM 193 265 951 007 RENNERT OM IN: MANGOS JA 41 973 008 RENNERT OM IN: PREISIG R 298 976 009 RENNERT OM BLOOD 47 695 976 010 RICHARDS GM ANAL BIOCHEM 57 369 974 011 RUSSELL DH POLYAMINES IN NORMAL AND NEOP 973 012 RUSSELL DH POLYAMINES IN NORMAL AND NEOP 973 013 RUSSELL DH BIOCHIM BIOPHYS ACTA 259 247 972 CT 1 WRIGHT RK PEDIATR RES 12 830 978 2 BRADDON SA BIOCHEM BIOPHYS RES COMMUN 80 75 978 3 JAKEL HP BIOL ZENTRALBL 98 55 979 4 BUEHLER BA ANN CLIN LAB SCI 10 195 980 5 GAHL WA PEDIATR RES 14 118 980 6 THEOHARIDES TC LIFE SCI 27 703 980 7 BRADDON SA ADV EXP MED BIOL 143 255 982 8 KAMOUN PP CLIN CHIM ACTA 154 219 986 PN 77163 RN 00744 AN 78011300 AU Wilson-G-B. Arnaud-P. Fudenberg-H-H. TI Improved method for detection of cystic fibrosis protein in serum using the LKB multiphor electrofocusing apparatus. SO Pediatr-Res. 1977 Sep. 11(9 Pt 1). P 986-9. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. MN ASTHMA: bl. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. ISOELECTRIC-FOCUSING: is. SUPPORT-U-S-GOVT-P-H-S. AB We have developed an improved method for the detection of cystic fibrosis protein (CFP). The method employs the LKB Multiphor to electrofocus whole serum, instead of the apparatus used in previous studies. Two basic modifications were necessary: (1) a pH 2.5--10.0 gradient instead of a pH 5--10 gradient, and (2) constant power for focusing the serum proteins instead of constant voltage. The first modification ensured adequate dissolution of the CFP-IgG complexes (or other precursor complexes which may liberate CFP). The second modification ensured a linear gradient (between pH 3.8 and pH 9.2), excellent resolution in the pH 8--9 region, and the separation of CFP within 2 hr without overheating of the gel. Electrofocusing with the LKB Multiphor permits the detection of CFP in as many as 24 serum samples per gel. Results obtained from the analysis of 31 cystic fibrosis, 28 obligate heterozygote carrier, and 28 normal control sera indicate that CFP can be reproducibly and accurately detected in sera using the LKB Multiphor. RF 002 ALTLAND K HUMANGENETIK 28 207 975 003 AWDEH ZL NATURE 219 66 968 004 DI SANTAGNESE PA N ENGL J MED 295 534 976 005 SMITH QT PEDIATR RES 10 999 976 006 THOMAS JM PEDIATR RES 11 138 977 007 WILLIAMSON AR IN: WEIR DM 973 008 WILSON GB PEDIATR RES 10 1001 976 009 WILSON GB SCAND J IMMUNOL 5 829 976 010 WILSON GB TEX REP BIOL MED 34 51 976 011 WILSON GB PEDIATR RES 9 635 975 012 WILSON GB CLIN CHIM ACTA 49 79 973 013 WILSON GB PEDIATR RES 11 139 977 014 WILSON GB PEDIATR RES 11 143 977 016 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 SCHOLEY J PEDIATR RES 12 800 978 2 WILSON GB PEDIATR RES 12 801 978 3 NAGY EC PEDIATR RES 13 729 979 4 TULLY GW PEDIATR RES 13 1078 979 5 WILSON GB PEDIATR RES 13 1079 979 6 MANSON JC LANCET 1 330 980 7 TARNOKY AL J ROY SOC MED 73 73 980 8 ACKERMAN SJ J IMMUNOL 125 2118 980 9 HALLINAN FM CLIN CHIM ACTA 117 103 981 10 VICKERS MF ANN CLIN BIOCHEM 19 442 982 11 BULLOCK S CLIN GENET 21 336 982 12 CAREY WF MED J AUST 2 528 983 13 HEELEY AF CLIN CHEM 29 2011 983 14 WILSON GB THYMUS 6 167 984 15 GRATAROLI R PEDIATR RES 18 130 984 16 ROBINSON PA ANN CLIN BIOCHEM 21 70 984 17 WILSON GB CLIN GENET 26 331 984 18 QURESHI AR J PEDIATR 106 913 985 PN 77164 RN 00745 AN 78032149 AU Pivetta-O-H. Sordelli-D-O. Labal-M-L. TI Pulmonary clearance of Staphylococcus aureus in mutant mice with some hereditary alterations resembling cystic fibrosis. SO Pediatr-Res. 1977 Nov. 11(11). P 1133-6. MJ CYSTIC-FIBROSIS: fg. DISEASE-MODELS-ANIMAL. LUNG: im. MICE-INBRED-STRAINS: im. PNEUMONIA-STAPHYLOCOCCAL: im. STAPHYLOCOCCUS-AUREUS: py. MN ANIMAL. CYSTIC-FIBROSIS: im. HUMAN. LUNG: mi. MALE. MICE. MICE-INBRED-BALB-C. MICE-INBRED-C57BL. MICE-INBRED-DBA. MUTATION. PHAGOCYTOSIS. AB Pulmonary clearance for Staphylococcus aureus has been examined in two inbred strains of mice with some hereditary alterations resembling cystic fibrosis (CF). These mice were mice with abnormal electrolyte metabolism (DBA/@J-cri), mice with spontaneous pneumonitis (C57BL/6J-bg), and mice without any CF-like alterations (BALB/c). The methods used to produce pulmonary infection were essentially those of Laurenzi and associates adapted as needed for mutant mice. The animals mentioned above were exposed to a finely divided aerosol suspension of a coagulase-positive strain of S. aureus in phosphate buffer. Immediately after the exposure, half of the animals were killed, and the remaining half were killed 4 hr later. In each experimental unit the mice killed immediately after the exposure were as similar as possible to the mice killed 4 hr later with respect to genotype, age, and sex; some were siblings. The uncleared bacteria (UBR) in 4 hr were 0.20 for the mice without any CF-like alterations (BALB/c) and the C57BL/6J-bg, bg/bg genotypes; 0.28 for C57BL/6J-bg, +/? genotypes; 0.50 for the DBA/2J-cri, +/? genotypes; and 0.56 for cri/cri mice. The number of viable staphylococci found immediately after the aerosol exposure (Co) in the C57BL/6J-bg strain is significantly lower than the Co of the BALB/c (P < 0.05) and DBA/2J-cri strain (P < 0.01). The latter two did not differ from each other. There was no sex difference with respect to the UBR and Co data. The DBA/2J-cri strain of mice, where the cri mutation first appeared, has a decreased capacity for clearance of S. aureus by the lung. Together with the other CF-like alterations of the cri mutation, namely failure for the reabsorption of Na+ by the parotid duct, high level of Na+ in the fur, and CF abnormal serum factor activity in the serum of the cri/cri mice, we suggest that the cribriform degeneration mouse mutant may provide a potential animal model for studying CF. The finding of a mouse mutation with electrolyte alterations and CF-like abnormal serum activity, simialr to the activity found in CF children, in an inbred strain of mice with a decreased bacterial clearance by the lungs, makes these mice very useful for CF studies. RF 001 ANDERSEN AA J BACTERIOL 76 471 958 002 BARTLETT MS BIOMETRICS 3 39 947 003 DI SANTAGNESE PA GAP CONF REP LUNG ORGAN DEF 972 004 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 005 DIXON WJ ANN MATH STAT 21 488 950 006 FEIGIN RD J PEDIATR 87 677 975 007 FINNEY DJ PROBIT ANALYSIS 971 008 GREEN GM J EXP MED 119 167 964 009 GREEN GM J CLIN INVEST 43 769 964 010 GREEN GM BR J EXP PATHOL 46 360 965 011 GREEN MC SCIENCE 176 800 972 012 HARROW EM AM REV RESPIR DIS 112 7 975 013 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 015 HUANG NN J PEDIATR 59 512 961 016 IACOCCA VF AM J DIS CHILD 106 315 963 017 KAISER D LIFE SCI 15 803 974 018 KASS EH BACT REV 30 488 966 019 LANE PW GENETICS 72 451 972 020 LAURENZI GA J CLIN INVEST 43 759 964 021 LAURENZI GA AM REV RESPIR DIS SUPPL 3 93 134 966 022 LAWSON D GAP CONF REP PSEUDOMON IN CF 974 023 LURIE MB AM REV TUBERC 61 765 950 024 MIDDLEBROOK G PROC SOC EXP BIOL MED 80 105 952 025 MYRVIK QN GAP CONF REP LUNG ORGAN DEF 972 027 RUPPERT D J LAB CLIN MED 87 544 976 028 SHWACHMAN H HOSP PRACT 9 143 974 CT 1 PIVETTA OH PEDIATR RES 13 1160 979 2 SORDELLI DO LIFE SCI 24 2003 979 3 SORDELLI DO MED (BUENOS AIRES) 39 457 979 4 DAVIS PB PEDIATR RES 14 83 980 5 PIVETTA OH LIFE SCI 26 1349 980 6 REHM SR J CLIN INVEST 66 194 980 7 PIVETTA OH LIFE SCI 28 2207 981 8 CERQUETTI MC IMMUNOL COMMUN 12 375 983 9 NOURI LA PEDIATR RES 17 657 983 10 CATANZARO OL LIFE SCI 32 825 983 11 SORDELLI DO INFECT IMMUN 39 1275 983 PN 77165 RN 00746 AN 78032153 AU Thomas-J-M. Merritt-A-D. Hodes-M-E. TI Electrophoretic analysis of serum proteins in cystic fibrosis. SO Pediatr-Res. 1977 Nov. 11(11). P 1148-54. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. MN BLOOD-PROTEIN-ELECTROPHORESIS. BLOOD-PROTEINS: ge. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. ELECTROPHORESIS-DISC. HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYDROGEN-ION-CONCENTRATION. IGG: an. ISOELECTRIC-FOCUSING. AB This study utilized isoelectric focusing and electrophoresis in an attempt to detect a cystic fibrosis (CF) serum factor(s), for which there is considerable indirect evidence. The method of isoelectric focusing in polyacrylamide gels (IEFAG) specified by Wilson et al. (53), as reproduced in our laboratory, did not enable the detection of a CF factor protein reported to focus near pH 8.4-8.5. Consequently, we employed our modified IEFAG techniques, which enabled us to demonstrate significantly enhanced resolution and striking heterogeneity in serum gamm-globulins. Despite the significant increase in the number of bands resolved by our methods, neither a difference at pH 8.4-8.5 nor other differences throughout the alkaline pH range could be detected consistently in the CF and heterozygous sera. The two-step IEFAG/disc electrophoresis technique outline by Altland et al. (2), as reproduced in our laboratory, indicated that at least one small, cationic protein could be fractioned from all serum samples. Improvements in the method of disc electrophoresis resulted in the observation of numerous bands from some samples and of differences among the samples, but no protein band unique to the CF genotypes was observed. Our approach, employing different electrophoresis techniques and varying the conditions of sample analysis, should have increased the likelihood of detecting a protein or proteins specific for the CF genotypes. Despite the many variations in our approach, no consistently unique protein was observed in the CF or heterozygous sera. A CF serum factor cannot be readily demonstrated by the electrophoretic techniques described. The value of the "biophysical assay" and the "nonbiologic technique" reported in the literature is suspect, and the promise and applicability of these techniques as diagnostic tests for the CF gene should be carefully evaluated. RF 002 ALTLAND K HUMANGENETIK 28 207 975 003 AWDEH ZL NATURE 219 66 968 004 BARNETT DR TEX REP BIOL MED 31 697 973 005 BARNETT DR TEX REP BIOL MED 31 703 973 006 BARNETT DR TEX REP BIOL MED 31 709 973 007 BERATIS NG PEDIATR RES 7 958 973 008 BOWMAN BH TEX REP BIOL MED 31 611 973 009 BOWMAN BH LANCET 1 404 974 010 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 011 BOWMAN BH N ENGL J MED 294 937 976 012 BULL HB ARCH BIOCHEM BIOPHYS 104 297 964 013 ANON J PEDIATR 88 711 976 014 CONNEALLY PM TEX REP BIOL MED 31 639 973 015 CONOVER JH PEDIATR RES 7 224 973 016 CROW JF IN: NEEL JV 23 965 017 DANES BS J EXP MED 137 1538 973 018 DANKS DM ANN HUM GENET 28 323 965 019 DI SANTAGNESE PA N ENGL J MED 295 481 976 020 GIBSON LE PEDIATRICS 23 545 959 021 HAGLUND H METHODS BIOCHEM ANAL 19 1 971 022 HOWARD A IN: PEETERS H 17 449 969 023 JOSEPHSEN RV ANAL BIOCHEM 40 476 971 024 KULCZYCKI LL CLIN PEDIATR 3 692 964 025 LAGOW J BIOCHEMISTRY 11 4520 972 026 LOBECK CC IN: STANBURY JB 1605 972 027 MCCOMBS ML TEX REP BIOL MED 31 615 973 028 NAKHLEH ET ANAL BIOCHEM 49 218 972 029 OPPENHEIMER EH PEDIATRICS 42 547 968 030 POLLEY MJ J MED GENET 11 249 974 031 RADOLA BJ IN: PEETERS H 18 487 970 032 RADOLA BJ BIOCHIM BIOPHYS ACTA 295 412 973 033 RADOLA BJ ANN NY ACAD SCI 209 127 973 034 RADOLA BJ BIOCHIM BIOPHYS ACTA 386 181 974 035 SALAMAN MR BIOCHEM J 122 93 971 036 SCHMOYER IR LIFE SCI PART 2 11 1037 972 037 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 038 SHWACHMAN H HOSP PRACT 9 143 974 039 SMITH QT PEDIATR RES 10 999 976 040$ SMYTH CJ J GEN MICROBIOL 7 41 974 041 THOMAS JM CF CLUB ABST 17 19 976 042 THOMAS JM PEDIATR RES 11 138 977 044 UI N BIOCHIM BIOPHYS ACTA 229 567 971 045 UI N ANN NY ACAD SCI 209 198 973 046 VALMET E SCIENCE TOOLS 15 8 968 047 VESTERBERG O BIOCHIM BIOPHYS ACTA 257 11 972 048 VESTERBERG O IN: ARBUTHNOTT JP 975 049 WANG CI N ENGL J MED 279 1216 968 050 WILLIAMSON AR IN: WEIR DM 973 052 WILSON GB PEDIATR RES 10 1001 976 053 WILSON GB PEDIATR RES 9 635 975 054 WILSON GB CLIN CHIM ACTA 49 79 973 055 WILSON GB PEDIATR RES 11 139 977 056 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 SCHOLEY J PEDIATR RES 12 800 978 2 WILSON GB PEDIATR RES 12 801 978 3 SCHAAP T ISR J MED SCI 14 201 978 4 THOMAS JM ANAL BIOCHEM 90 596 978 5 TULLY GW PEDIATR RES 13 1078 979 6 WILSON GB PEDIATR RES 13 1079 979 7 MANSON JC LANCET 1 330 980 8 NEVIN GB HUM GENET 56 387 981 9 THOMAS JM CLIN CHIM ACTA 111 199 981 10 BULLOCK S CLIN GENET 21 336 982 11 BROCK DJH HUM GENET 60 30 982 12 BURY AF CLIN CHIM ACTA 118 45 982 13 GRATAROLI R PEDIATR RES 18 130 984 14 ANON LANCET 2 249 985 15 KLINGER KW SEM RESPIR MED 6 243 985 16 JAMIESON A HUM GENET 70 168 985 17 GETLIFFE KA J INHERIT METAB DIS 9 348 986 PN 77166 RN 00747 AN 78032158 AU Callahan-J-W. Leung-A. TI Arginine esterase and lysosomal hydrolases in liver from cystic fibrosis subjects. SO Pediatr-Res. 1977 Nov. 11(11). P 1166-9. MJ CYSTIC-FIBROSIS: en. ESTERASES: me. HYDROLASES: me. LIVER: en. MN ADOLESCENCE. ADULT. ARGININE: me. CATHEPSINS: me. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. HYDROGEN-ION-CONCENTRATION. ISOELECTRIC-FOCUSING. LYSOSOMES: en. MALE. AB The total activity and isoelectric focusing patterns of arginine esterase, cathepsin B1, and several lysosomal hydrolases were normal in liver from two patients with cystic fibrosis. No abnormalities were observed in values for pH optimum, Km, and Vmax for arginine esterase and cathepsin B1 in liver from cystic fibrosis patients compared to those values for liver from the control subject. Soybean trypsin inhibitor at concentrations up to 100 microgram/ml had no effect on liver arginine esterase or cathepsin B1. RF 001 ALHADEFF JA CLIN GENET 10 63 976 002 BARRETT AJ ANAL BIOCHEM 7 290 972 003 BERATIS NG PEDIATR RES 7 958 973 004 CALLAHAN JW BIOCHIM BIOPHYS ACTA 391 141 975 005 CALLAHAN JW PEDIATR RES 9 908 975 006 CALLAHAN JW CLIN RES 24 679A 976 007 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 008 CONOVER JH PEDIATR RES 7 220 973 009 CONOVER JH PEDIATR RES 7 224 973 010 CONOVER JH LIFE SCI 14 253 974 011 IODICE AA ARCH BIOCHEM BIOPHYS 117 477 966 012 LIEBERMAN J AM REV RESPIR DIS 109 399 974 013 LOWRY OH J BIOL CHEM 193 265 951 014 RAO GJS J PEDIATR 80 573 972 015 RAO GJS SCIENCE 177 610 972 016 RAO GJS PEDIATR RES 8 684 974 017 RAO GJS PEDIATR RES 9 739 975 018 SCHACHTER H PHYSIOL REV 49 509 969 019 SCHNEIDER PB J LIPID RES 8 202 967 020 SIEGELMAN AM ARCH BIOCHEM BIOPHYS 97 159 962 021 THOMAS GH PEDIATR RES 7 751 973 022 VAN HOOF F EUR J BIOCHEM 7 34 968 CT 1 PLATT MW PEDIATR RES 12 874 978 2 ALHADEFF JA CLIN CHIM ACTA 89 469 978 3 ALHADEFF JA CLIN CHIM ACTA 105 131 980 4 ALHADEFF JA CLIN CHIM ACTA 117 227 981 PN 77167 RN 00748 AN 77078176 AU Conod-E-J. Conover-J-H. Gaerlan-P. TI Separation of serum ciliary dyskinesia substances from cystic fibrosis subjects. SO Pediatr-Res. 1977 Jan. 11(1 Pt 1). P 45-7. MJ CYSTIC-FIBROSIS: bl. IGG: ip. MN BIOLOGICAL-ASSAY. CILIA. CYSTIC-FIBROSIS: et. HUMAN. TRACHEA. ULTRAFILTRATION. AB Purified serum immunoglobulin G (IgG), derived from eight cystic fibrosis (CF) and five carrier subjects, has been shown to be responsible for the mucociliary disturbances noted in the rabbit tracheal bioassay. A small molecular substance of less than 10,000 but greater than 1,000 daltons (by Amicon filtration) was found associated with gamma-globulin fractions isolated from sera of these same cystic patients and their parents. Once separated by PM-10 ultrafiltration, this small substance was unable to promote the ciliary dyskinesia response in eight of eight CF and five of five CF carrier individuals unless pooled purified human IgG was added. In addition, the IgG-rich fraction retained by PM-10 ultrafiltration was still able to promote the ciliary dyskinesia response in the bioassay, an event noted in our earlier work with whole serum. The size of the small serum substance and its association with IgG closely corresponds to that described for the oyster test system, as well as to that produced by cultured cells derived from homozygotes and heteroxygotes for this genetic disorder. The persistence of the ability to promote mucociliary disturbances by the IgG-rich retentate PM-10 fractions may be indicative of the ineffective molecular separation by the Amicon ultransfiltration apparatus or may represent another CF-related, IgG-associated substance not influenced by ultrafiltration. Speculation The genetic disturbance of CF can be explained by the presence of a molecule(s) which has an affinity for IgG, which in turn gives rise to the various physiologic facets of this disorder. This molecule(s) is present in CF only because of a deficiency of an enzyme which normally controls its level by inactivation. RF 001 BERATIS NG PEDIATR RES 7 958 973 002 BESLEY GTN J MED GENET 6 278 969 003 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 004 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 005 BOWMAN BH SCIENCE 164 325 969 006 CONOVER JH PEDIATR RES 7 224 973 007 CONOVER JH PEDIATR RES 7 220 973 008 CONOVER JH LIFE SCI 14 253 974 009 CONOVER JH LANCET 1 1194 973 010 CONOVER JH PEDIATR RES ABST 10 385 976 011 DANES BS J EXP MED 136 1313 972 012 SPOCK A PEDIATR RES 1 173 967 013 WILLIAMS CA METHODS IMMUNOL AND IMM 1 307 967 CT 1 CONOVER JH BIOCHEM BIOPHYS RES COMMUN 83 1595 978 2 WILSON GB J LAB CLIN MED 92 463 978 3 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 4 WILL PC PEDIATR RES 13 1129 979 5 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 6 BARDON A CLIN CHIM ACTA 101 17 980 7 SANDERSON MJ PEDIATR RES 15 219 981 8 FLEMING N EXPERIENTIA 37 139 981 9 CARSON SD PEDIATR RES 16 13 982 10 MCNEELY MC PEDIATR RES 16 21 982 11 BOGART BI J CHROMATOGR 381 29 986 PN 77168 RN 00749 AN 77148152 AU Wilson-G-B. Fudenberg-H-H. TI Studies on cystic fibrosis using isoelectric focusing. IV. Distinction between ciliary dyskinesia activity in cystic fibrosis and asthmatic sera and association of cystic fibrosis protein with the activity in cystic fibrosis serum. SO Pediatr-Res. 1977 Apr. 11(4). P 317-24. MJ ASTHMA: bl. BLOOD-PROTEINS: ip. CYSTIC-FIBROSIS: bl. MN ASTHMA: im. CILIA: pp. COMPARATIVE-STUDY. COMPLEMENT-3: ip. CYSTIC-FIBROSIS: im. HUMAN. IGG: ip. ISOELECTRIC-FOCUSING. MOLECULAR-WEIGHT. SUPPORT-U-S-GOVT-P-H-S. AB The cystic fibrosis protein (CFP) and ciliary dyskinesia activities (CDA) in sera from CF homozygotes, heterozygote carriers, and individuals with bronchial asthma have been partially purified. Concurrently, the CDA's in sera from patients with cystic fibrosis (CF) or bronchial asthma were shown to be different substances by ion- exchange or gel permeation chromatographic procedures. Sephadex G-200 chromatography indicated that the CF-CDA eluted with a protein fraction of molecular weight (MW) 68,000-150,000 and that the asthma CDA was found in a protein fraction of MV greater than 150,000. The two activities could also be separated by DEAE-cellulose chromatography. Prior acidification of whole normal, CF homozygote, obligate heterozygote, or asthmatic sera to pH 3.7 using EDTA, followed by fractionation of Sephadex G-200 removed all the CDA's from fractions of highermolecular weight and shifted the activities to a protein fraction of MW 1,100-13,700. This procedure afforded a 200-fold purification of the CDA's in sera from patients with asthma or CF. EDTA treatment, however, also generated a CDA in previously nonreactive normal sera. Subsequent fractionation of the various active G-200 fractions on Bio-Gel P10 allowed for the separation of three separate activities (Bio-Gel Fractions I, II-IV, and V). Fraction I was shown to represent the activity in sera from patients with asthma and was determined to be C3a (MW 9,000). Fraction I was also found in normal, CF, and carrier sera and therefore is not a specific CDA. Fraction II-IV is thought to represent a CF-specific CDA (MW 5,000) since it could not be demonstrated in either normal or asthmatic sera but was found in sera of obligate heterozygotes. Fraction III-IV also did not react with antisera to human C3a. Fraction V was generated from all serum types upon acidification of the serum with EDTA and is thought to be a nonspecific CDA. Bio-Gel P10 filtration of Sephadex G-200 fractions provided 823-fold and 650- fold purification of the asthmatic and CF CDA's, respectively. Concurrent analysis of column fractions for CDA's by bioassay and for CFP by electrofocusing showed CFP only in fractions that contained the CF-CDA. Combined analyses employing acid disc gel electrophoresis, isoelectric focusing, and EDTA treatment of active CF-immunoglobulin (Ig) G and Sephadex G-200 column fractions, followed by Bio-Gel P10 chromatography, provided evidence that CFP was associated with the CF-CDA. It is unknown as yet whether CFP itself is responsible for the CF-CDA activity. RF 001 BARNETT DR PEDIATR RES 8 687 974 002 BOWMAN BH IN: MANGOS JA 277 976 003 BOWMAN BH SCIENCE 164 325 969 004 CONOVER JH PEDIATR RES 7 220 973 005 CONOVER JH LANCET 1 1194 973 006 CONOVER JH LIFE SCI 14 253 974 007 DI SANTAGNESE PA N ENGL J MED 295 534 976 008 EDER J J IMMUNOL METH 2 67 972 009 GEWURTZ H IN: INGRAM DG 56 972 010 HUGLI TE J BIOL CHEM 250 1472 975 011 JAMES K CLIN EXP IMMUNOL 19 237 975 012 LOCKHART LH TEX REP BIOL MED 31 631 973 013 LOWRY OH J BIOL CHEM 193 265 951 014 MOSHER DF J EXP MED 143 462 976 015 OUCHTERLONY O IN: WEIR DM 191 973 016 POLLEY MJ J MED GENET 11 249 974 017 REISFELD RA NATURE 195 281 962 018 SHAPIRA E PEDIATR RES 10 812 976 019 SPOCK A PEDIATR RES 1 173 967 021 WILSON GB PEDIATR RES 10 1001 976 022 WILSON GB PEDIATR RES 10 87 976 023 WILSON GB TEX REP BIOL MED 34 51 976 024 WILSON GB ADV POLYMINE RES 2 281 977 027 WILSON GB PEDIATR RES 9 635 975 028 WILSON GB CF CLUB ABST 59 973 029 WILSON GB LIFE SCI 15 551 974 030 WILSON GB CLIN CHIM ACTA 49 79 973 031 WILSON GB CLIN RES 24 295 976 034 WILSON GB CLIN RES 25 34 977 CT 1 WILSON GB J LAB CLIN MED 92 463 978 2 WILL PC PEDIATR RES 13 1129 979 3 WILSON GB J CLIN INVEST 66 1010 980 4 SORENSEN RU PEDIATR RES 15 14 981 5 BLITZER MG PEDIATR RES 16 938 982 6 BLITZER MG PEDIATR RES 18 540 984 PN 77169 RN 00750 AN 77193146 AU Yokoyama-M. Chang-J-P. Hom-D. Wilson-S-L. TI An electron microscopic cytochemical study on concanavalin A binding sites and their mobility in normal and cystic fibrosis fibroblasts in vitro. SO Pediatr-Res. 1977 Jun. 11(6). P 765-9. MJ CONCANAVALIN-A: me. CYSTIC-FIBROSIS: me. MN BINDING-SITES. CONCANAVALIN-A: pd. CYSTIC-FIBROSIS: pa. FIBROBLASTS: de, me, ul. HETEROZYGOTE. HISTOCYTOCHEMISTRY. HOMOZYGOTE. HUMAN. IN-VITRO. TEMPERATURE. SUPPORT-U-S-GOVT-P-H-S. AB Concanavalin A (Con A) binding sites were visualized ultrastructurally in the cultured fibroblasts from cystic fibrosis patients, obligatory heterozygotes, and normal individuals by peroxidase labeling technique. Con A binding sites were localized as a continuous layer on the external side of the plasma membrane in fixed fibroblasts of the three genotypes. In living cells, Con A induces both lateral and vertical movements of binding sites as expressed by cap formation and internalization of the plasma membrane. Fibroblasts of the three genotypes responded similarly to Con A treatment and failed to show significant detectable differences. RF 001 ANTOINE JC J CELL BIOL 63 12 974 002 BAIG MM J PEDIATR 86 72 975 003 BARRANCO SC J CELL PHYSIOL 88 33 976 004 BAUR PS TEX REP BIOL MED 34 113 976 005 BENKE PJ LANCET 1 182 972 006 BERLIN RD N ENGL J MED 292 515 975 007 BERNARD W EXP CELL RES 64 232 971 008 BESLEY GTN J MED GENET 6 278 969 009 BOLTON WE AM J HUM GENET 27 394 975 010 BORAT N EXP CELL RES 76 451 973 011 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 012 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 013 BOWMAN BH SCIENCE 164 325 969 014 BOWMAN BH N ENGL J MED 294 937 976 015 CHANG JP J ULTRASTRUCT RES 37 370 971 016 CHANGUS JE AM J PATHOL 80 317 975 017 COLLARD JG EXP CELL RES 86 81 974 018 EDELSON PJ J EXP MED 140 1364 974 019 FLETCHER DS CLIN CHIM ACTA 44 5 973 020 GOLDSTEIN IJ BIOCHEMISTRY 4 876 965 021 GONATAS NK EXP CELL RES 94 426 975 022 GRAHAM RC J HISTOCHEM CYTOCHEM 14 291 966 023 HIRANO H PROC NAT ACAD SCI USA 69 2945 972 024 HUET C INT J CANCER 13 227 974 025 MANGOS JA SCIENCE 158 135 967 026 MANGOS JA PEDIATR RES 2 378 968 027 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 028 NICOLSON GL INT REV CYTOL 39 89 974 029 NICOLSON GL CANCER RES 35 144 975 030 QUISSELL DO NATURE 247 115 974 031 ROSENBLITH JZ PROC NAT ACAD SCI USA 70 1625 973 032 ROWLATT C INT J CANCER 11 314 973 033 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 58 1066 974 034 SINGER SJ SCIENCE 175 720 972 035 SPURR AR J ULTRASTRUCT RES 26 31 969 036 WOOD JG J CELL BIOL 63 541 974 038 YOKOYAMA M PROC CONG OF ANATOMY 10TH 501 975 CT 1 MOLLER PC HISTOCHEMISTRY 62 289 979 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 YOKOYAMA M ACTA HISTOCHEM CYTOCHEM 13 139 980 4 YOKOYAMA M J HISTOCHEM CYTOCHEM 28 543 980 5 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 PN 77170 RN 00751 AN 77148186 AU Rosenthal-A. Button-L-N. Khaw-K-T. TI Blood volume changes in patients with cystic fibrosis. SO Pediatrics. 1977 Apr. 59(4). P 588-94. MJ BLOOD-VOLUME. CYSTIC-FIBROSIS: pp. MN ADOLESCENCE. ADULT. ANOXEMIA: co. CHILD. CYSTIC-FIBROSIS: co. ERYTHROCYTES. FEMALE. HEMATOCRIT. HUMAN. MALE. OXYGEN: bl. PULMONARY-HEART-DISEASE: co, pp. RESPIRATORY-FUNCTION-TESTS. SUPPORT-U-S-GOVT-P-H-S. AB Simultaneous red blood cell (RBC) and plasma volume determinations were obtained in 16 patients with cystic fibrosis (CF) and moderately severe pulmonary involvement. Hypervolemia with an increase in both RBC and plasma volumes was observed. Changes in blood volume were marked when values were indexed by weight but less significant when indexed by height. Decreasing systemic arterial oxygen saturation was associated with a progressive increase in RBC mass, hematocrit value, and hemoglobin level and a decrease in mean corpuscular hemoglobin concentration. RBC and total blood volumes were highest in patients with cor pulmonale and congestive heart failure. However, the compensatory polycythemic response in patients with CF was inadequate when compared with the response to hypoxemia in patients with cyanotic congenital heart disease. The insufficient oxygen-carrying capacity may compromise tissue oxygen delivery and necessitate treatment. RF 001 ROSENTHAL A AM J CARDIOL 27 162 971 002 VANIER T N ENGL J MED 269 169 963 003 CAPLAN AH J PEDIATR 73 540 968 004 SCHRIEBER SS IN: FRIEDBERG CK 29 962 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 BUTTON LN TRANSFUSION 5 143 965 007 KEANE JF PROG CARDIOVASC DIS 18 57 975 008 TONZ O LANCET 2 1096 965 009 WENNESLAND R J CLIN INVEST 38 1065 959 010 RUSSELL SJM ARCH DIS CHILD 24 88 949 011 WEIL JV J CLIN INVEST 47 7 968 012 ROSENTHAL A PEDIATRICS 47 537 971 013 ORZALESI MM AM REV RESPIR DIS 107 928 973 014$ BORDEN M CF CLUB ABST 15 975 CT 1 ROSENTHAL A PEDIATRICS 59 919 977 2 LEMEN RJ AM REV RESPIR DIS 117 639 978 3 SANTAGNESE PAD AM J MED 66 121 979 4 LAKE CR CLIN CHIM ACTA 92 141 979 5 FISCHER EG J PEDIATR 95 385 979 6 PEDERSEN PS ACTA PAEDIATR SCAND 70 507 981 7 RIFF L SEM PERINATOL 6 155 982 8 MOSS AJ PEDIATRICS 70 728 982 9 BERG U ACTA PAEDIATR SCAND 71 833 982 10 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 11 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 12 WAGENER JS PEDIATRICS 74 168 984 13 LESTER LA SEM RESPIR MED 6 285 985 PN 77171 RN 00752 AN 77172346 AU Gibson-B. Levin-D. Cubrarino-G. Weinberg-A. TI Roentgenographically visible fatty liver in cystic fibrosis. SO Pediatrics. 1977 May. 59(5). P 778-80. MJ CYSTIC-FIBROSIS: ra. FATTY-LIVER: ra. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. MALE. EX The initial roentgenograms of 178 consecutive newly diagnosed patients with cystic fibrosis seen during the past five years were reviewed to determine the presence of fatty liver. Seven cases of visibly fatty liver were identified. The incidence of visibly fatty liver on the initial roentgenograms in newly diagnosed cases of cystic fibrosis was 4%. The cause of fatty infiltration of the liver in cystic fibrosis is not clear; however, the basis is most likely a nutritional deficiency in protein. RF 001 GRISCOM NT RADIOLOGY 117 385 975 002 SWISCHUK LE AM J ROENTG RAD THER NUCL MED 122 159 974 003 SWISCHUK LE J PEDIATR 88 452 976 004 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 005 CRAIG JM AM J DIS CHILD 93 357 957 006 MELHEM RE PEDIATR RADIOL 4 153 976 CT 1 YOUSEFZADEH DK RADIOLOGY 131 351 979 2 ARBORGH B SCAND J GASTROENTEROL 15 73 980 3 PARK RW GASTROENTEROLOGY 81 1143 981 PN 77172 RN 00753 AN 77256215 AU Lau-W-K. Young-L-S. Osher-A-B. Dooley-R-R. TI Amikacin therapy of exacerbations of Pseudomonas aeruginosa infections in patients with cystic fibrosis. SO Pediatrics. 1977 Sep. 60(3). P 372-7. MJ AMIKACIN: tu. CYSTIC-FIBROSIS: co. KANAMYCIN: aa. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-TRACT-INFECTIONS: dt. MN ACUTE-DISEASE. ADOLESCENCE. ADULT. AMIKACIN: ad. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. MALE. PSEUDOMONAS-AERUGINOSA. RESPIRATORY-TRACT-INFECTIONS: et. SUPPORT-U-S-GOVT-P-H-S. AB Amikacin, a new semisynthetic aminoglycoside antibiotic with activity against Pseudomonas aeruginosa, was used to treat 22 acute exacerbations of chronic pulmonary infections in 18 patients with cystic fibrosis. Patients ranged from 5 to 32 years of age and had mucoid P. aeruginosa isolated from sputum. The amikacin dose was usually 7.5 mg/kg every eight hours but was increased to 10 mg/kg and/or carbenicillin was added in selected cases depending on clinical course. Although P. aeruginosa was not eliminated from our patients' sputum except in two cases, there was a good clinical response in 19 of 22 courses. Significant improvement in chest x-ray films, spirometry, or arterial oxygen tension was documented in 11 of 17 courses. One instance of serum creatinine level elevation could not be attributed to this antibiotic. Two patients showed minimal (15 dB) unilateral high-frequency hearing loss on serial audiograms. Activity against many gentamicin-resistant strains and high blood levels are among the attractive properties of amikacin. Amikacin is clinically effective in treating Pseudomonas-associated pulmonary infections complicating cystic fibrosis. RF 001 SHWACHMAN H AM J DIS CHILD 96 6 958 002 LAWSON D IN: WATT PJ 69 970 003 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 004 RUCKER RW PEDIATRICS 54 358 974 005 MEARNS MB ARCH DIS CHILD 47 902 972 006 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 007 MAY JR ARCH DIS CHILD 47 908 972 008 HUANG NN J PEDIATR 59 512 961 009 MCCRAE WM SCOTT MED J 19 187 974 010 MEARNS MB ARCH DIS CHILD 47 5 972 011 ROLINSON GN IN: LAWSON D PROC 5TH INT CF 214 969 012 HAWLEY HB CURR THER RES 16 414 974 013 HOFF GE SCAND J INFECT DIS 6 333 974 014 BOXERBAUM B J INFECT DIS SUPPL 124 293 971 015 BOXERBAUM B J INFECT DIS SUPPL 122 59 970 016 HUANG NN J PEDIATR 78 338 971 017 PARRY MF PROC ICAAC 15TH ABST 335 975 018 BODEY GP ANTIMICROB AGENTS CHEMOTHER 4 186 973 019 YOUNG LS ANTIMICROB AGENTS CHEMOTHER 4 617 973 020 YU PKW ANTIMICROB AGENTS CHEMOTHER 4 133 973 021 PRICE KE J ANTIBIOT (TOKYO) 25 709 972 022 MEYER RD ANN INTERN MED 83 790 975 023 CLARKE JT CLIN PHARMACOL THER 15 610 974 024 BODEY GP ANTIMICROB AGENTS CHEMOTHER 5 508 974 025 STEVENS P J LAB CLIN MED 86 349 975 026 STEVENS P ANTIMICROB AGENTS CHEMOTHER 7 374 975 027 BLACK RE ANTIMICROB AGENTS CHEMOTHER 9 956 976 CT 1 KRAUSE PJ CURR THER RES CLIN EXP 25 609 979 2 BEAUDRY PH J PEDIATR 97 144 980 3 PIEN FD AM J HOSP PHARM 38 981 981 4 MEYER RD ANN INTERN MED 95 328 981 5 MARKS MI J PEDIATR 98 173 981 6 HYATT AC J PEDIATR 99 307 981 7 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 8 KELLY HB J PEDIATR 100 318 982 9 FRASER GL J PEDIATR 101 792 982 10 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 11 GUAY DRP DRUG INTEL CLIN PHARM 17 83 983 12 SZAFF M ACTA PAEDIATR SCAND 72 651 983 13 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 14 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 15 HOLM SE REV INFECT DIS 8 S305 986 16 AUTRET E EUR J CLIN PHARMACOL 31 79 986 17 PITT TL J ROY SOC MED 79 13 986 18 CHADWICK EG AM J MED 80 166 986 PN 77173 RN 00754 AN 77233615 AU Wong-J-W. Keens-T-G. Wannamaker-E-M. Douglas-P-T. Crozier-N. Levison-H. Aspin-N. TI Effects of gravity on tracheal mucus transport rates in normal subjects and in patients with cystic fibrosis. SO Pediatrics. 1977 Aug. 60(2). P 146-52. MJ CYSTIC-FIBROSIS: th. GRAVITATION. MUCUS: ph. TRACHEA: ph. MN ADOLESCENCE. ADULT. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. MALE. POSTURE. RADIONUCLIDE-IMAGING: mt. RESPIRATORY-TRACT-INFECTIONS: th. AB A noninvasive, radionuclide imaging technique for measuring the rate of mucus clearance in the trachea (RT), was used to study gravitational effects on mucus clearance in 13 patients with cystic fibrosis (CF), average age 17 years; 7 normal, nonsmoking adults, average age 26 years; and a normal subject who was recovering from an acute upper respiratory tract infection (URTI). In the upright position, nine of the CF patients and the subject with URTI demonstrated abnormal tracheal mucus clearance which approached normal when they were placed in 25 degrees headdown position. The normal subjects and two of the CF patients showed no significant difference in the RT measured in the two positions. The results of the study indicate that the force of gravity can be a major influence on tracheal mucus clearance in CF and URTI subjects. This conclusion supports the use of postural drainage as an effective form of therapy in patients with cystic fibrosis. RF 001 ZUELZER WW PEDIATRICS 4 53 949 002 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 003 JONES NL AM REV RESPIR DIS 110 132 974 004 MELLINS RB AM REV RESPIR DIS SUPPL 110 137 974 005 YEATES DB J APPL PHYSIOL 39 487 975 006 WONG JW THESIS 977 007 YEATES DB ARCH DIS CHILD 51 28 976 008 SNEDECOR GW STATISTICAL METHODS 967 009 BLAKE J J BIOMECH 8 179 975 010 WOOD RE PEDIATR RES 8 471 974 012 IRAVANI J J PATHOL 106 81 972 013 HERS JFP AM REV RESPIR DIS SUPPL 3 93 162 966 CT 1 BASSETT PG COMPUT BIOL MED 9 97 979 2 OLDENBURG FA AM REV RESPIR DIS 120 739 979 3 STURGESS JM N ENGL J MED 300 53 979 4 CLARKE SW BR J CLIN PHARMACOL 9 537 980 5 PAVIA D CLIN RESP PHYSIOL 16 335 980 6 PAVIA D EUR J RESPIR DIS 61 157 980 7 KATZ SM AM J CLIN PATHOL 73 682 980 8 DARROW G AM REV RESPIR DIS 122 155 980 9 MENKES H AM REV RESPIR DIS 122 127 980 10 ROCHESTER DF AM REV RESPIR DIS 122 133 980 11 STURGESS JM N ENGL J MED 303 318 980 12 YEATES DB ANN BIOMED ENG 9 577 981 13 BATEMAN JRM THORAX 36 683 981 14 TURNER JAP PEDIATRICS 67 805 981 15 MILNER AD BR J HOSP MED 28 89 982 16 SUTTON PP EUR J RESPIR DIS 63 188 982 17 ROSSMAN CM AM REV RESPIR DIS 126 131 982 18 ZAPLETAL A EUR J RESPIR DIS 64 426 983 19 SUTTON PP SEM RESPIR MED 5 353 984 20 WANNER A AM REV RESPIR DIS 130 701 984 21 BROENNLE AM INT ANESTHESIOL CLIN 23 1 985 22 WOLLMER P EUR J RESPIR DIS 66 233 985 23 KIRILOFF LH CHEST 88 436 985 24 FALING LJ CLIN CHEST MED 7 599 986 25 VANDERSCHANS CP THORAX 41 448 986 26 MATTHYS H EUR J RESPIR DIS 69 311 986 27 VERBOON JML EUR J RESPIR DIS 69 169 986 28 PIEDRA P J PEDIATR 108 817 986 29 WOODHEAD M BR MED J 294 921 987 PN 77174 RN 00755 AN 77213860 AU Morin-C-L. van-Caillie-M. Roy-C-C. Lasalle-R. TI Mucosal enterokinase activity in pancreatic insufficiency and celiac disease. SO Pediatrics. 1977 Jul. 60(1). P 114-6. MJ CELIAC-DISEASE: en. ENTEROPEPTIDASE: me. INTESTINAL-MUCOSA: en. PANCREATIC-DISEASES: en. PEPTIDE-PEPTIDOHYDROLASES: me. MN CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en. HUMAN. INFANT. SUCRASE: me. EX Jejunal biopsy specimens were obtained from children with cystic fibrosis (CF), celiac disease (CD), or congenital exocrine pancreatic hypoplasia (CEPH) and from control subjects. Sections were examined histologically and homogenates were used for measurement of enterokinase and sucrase activities. This work was undertaken because enterokinase is considered a brush border enzyme, the release of which into the intestinal lumen is influenced by pancreatic enzymes, and also because we have recently shown a small bowel mucosal dysfunction in patients with CF. As expected, sucrase activity was reduce (p < .05) in children with CD as compared to the other three groups. On the other hand, enterokinase activity in children with pancreatic insufficiency (CF and CEPH) was higher (p < .05) than that of control subjects. The present study has shown that in patients with CF and CEPH, mucosal enterokinase activity was increased as compared to control subjects. It seems that the pancreatic enzymes play a large role in the turnover of enzymes in the brush border membrane and that the observed increase of mucosal enterokinase activity is probably explained by a reduced degradation in patients with exocrine pancreatic insufficiency. RF 001 HADORN B LANCET 1 812 969 002 NORDSTROM C BIOCHIM BIOPHYS ACTA 242 209 971 003 SCHMITZ J BIOCHIM BIOPHYS ACTA 343 435 974 004 NORDSTROM C BIOCHIM BIOPHYS ACTA 268 711 972 005 NORDSTROM C BIOCHIM BIOPHYS ACTA 289 367 972 006 MORIN CL J PEDIATR 88 213 976 007 NEWCOMER AD GASTROENTEROLOGY 53 881 967 008 LOUVARD D BIOCHIM BIOPHYS ACTA 309 127 973 009 LEBENTHAL E GASTROENTEROLOGY 70 508 976 010 ALPERS DH BIOCHIM BIOPHYS ACTA 401 28 975 011 CASPARY WF GUT 16 89 975 012 SEETHARAM B LIFE SCI 18 89 976 013 WOODLEY JF GUT 13 900 972 014 RUTGEERSTS L ACTA GASTROENTEROL BELG 36 449 973 015 LEBENTHAL E PEDIATRICS 56 585 975 CT 1 MORIN CL DIGESTION 18 402 978 2 SCHMIDT E CLIN BIOCHEM 12 247 979 3 SCHMIDT E J CLIN CHEM CLIN BIOCHEM 17 693 979 4 LENTZE MJ LANCET 2 504 982 5 LEBENTHAL E DIGESTION 23 39 982 6 LENTZE MJ J PEDIATR GASTROENTEROL NUTR 2 S252 983 7 KAI H J PEDIATR GASTROENTEROL NUTR 3 328 984 PN 77175 RN 00756 AN 77124835 AU Bass-H-N. Miller-A-A. TI Cystic fibrosis presenting with anemia and hypoproteinemia in identical twins. SO Pediatrics. 1977 Jan. 59(1). P 126-7. MJ ANEMIA: fg. CYSTIC-FIBROSIS: fg. DISEASES-IN-TWINS. HYPOPROTEINEMIA: fg. MN ANEMIA: co. CASE-REPORT. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. HYPOPROTEINEMIA: co. INFANT-NUTRITION. INFANT. MILK-PROTEINS. TWINS-MONOZYGOTIC. EX Cystic fibrosis of the pancreas is a recessively inherited disorder generally considered to result in chronic pulmonary or digestive symptoms. Therefore, it may not be readily appreciated that this disease can also present with anemia, edema, and hypoproteinemia, especially in infants taking breast milk or soy-based formulas. The following example involving monozygotic twins given cow's milk formula since birth will serve to illustrate this association. RF 001 LEE PA JAMA 228 585 974 002 DOLAN TF JR CLIN PEDIATR 9 295 970 003 FLEISHER DS J PEDIATR 64 341 964 004 LEE PA YEARBOOK OF PEDIATRICS 210 975 005 STROBER W PEDIATRICS 43 416 969 006 SHAHIDI NT J PEDIATR 59 533 961 CT 1 GUNN T AM J DIS CHILD 132 317 978 2 MARIANELLI L MINERVA PEDIATR 31 1787 979 3 CHU JY NUTR REV 37 351 979 4 BLUMENTHAL I ARCH DIS CHILD 55 812 980 PN 77176 RN 00757 AN 77193185 AU Rosenthal-A. Khaw-K-T. Shwachman-H. TI Hemoglobin-oxygen equilibrium in cystic fibrosis. SO Pediatrics. 1977 Jun. 59(6). P 919-26. MJ CYSTIC-FIBROSIS: bl. HEMOGLOBINS. OXYGEN: bl. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: pp. DIPHOSPHOGLYCERIC-ACIDS: bl. ERYTHROCYTES: an. ERYTHROPOIESIS. FEMALE. HEMODYNAMICS. HUMAN. MALE. RESPIRATION. SUPPORT-U-S-GOVT-P-H-S. AB A study of 35 patients with cystic fibrosis demonstrated that increasing severity of pulmonary involvement was associated with a mild but definite increase in erythrocyte 2,3-diphosphoglycerate (2,3- DPG) and a decrease in hemoglobin affinity for oxygen. The predominant regulators of 2,3-DPG were blood pH, cardiac output, and systemic oxygen transport. No significant relationship was observed between erythrocyte 2,3-DPG content and arterial oxygen tension. Hypophosphatemia may have prevented a greater increase in erythrocyte 2,3-DPG content. The inadequate increase in 2,3-DPG and consequent insufficient change in hemoglobin-oxygen affinity, coupled with an insufficient compensatory erythrocytic response, may adversely affect tissue oxygenation in patients with severe cystic fibrosis. RF 001 EDWARDS MJ CLIN RES 16 153 968 002 ASTE-SALAZAR H AM J PHYSIOL 142 733 944 003 LENFANT C RESPIR PHYSIOL 7 7 969 004 ROSENTHAL A AM J CARDIOL 27 162 971 005 TORRANCE JD S AFR J MED SCI 39 33 974 006 ROSENTHAL A PEDIATRICS 47 537 971 007 ROSENTHAL A PEDIATRICS 59 588 977 008 ORZALESI MM AM REV RESPIR DIS 107 928 973 009 FAIRWEATHER LJ CLIN SCI MOL MED 47 577 974 010 EDWARDS MJ CHEST 61 255 972 011 SIASSI B J PEDIATR 78 794 971 012 MOSS AJ J PEDIATR 67 797 965 013 LIEBMAN J IN: MANGOS JA 41 976 014 SHWACHMAN H AM J DIS CHILD 96 6 958 015$ ROSE ZB J BIOL CHEM 256 3232 970 016 ROSE AB ANAL BIOCHEM 35 117 970 017 DUVELLEROY MA J APPL PHYSIOL 28 227 970 018 SEVERINGHAUS JW J APPL PHYSIOL 21 1108 966 019 GIBSON DG BR HEART J 35 128 973 020 KEANE JF PROG CARDIOVASC DIS 18 57 975 021 OSKI FA N ENGL J MED 280 1165 969 022 LENFANT C J APPL PHYSIOL 30 625 971 023 ROSENTHAL A PEDIATR RES 7 301 973 024 WOODSON RD J CLIN INVEST 49 1349 970 025 CAPLAN AH J PEDIATR 73 540 968 026 LICHTMAN MA ANN INTERN MED 74 563 971 027 TRAVIS SF N ENGL J MED 285 763 971 028 DESFORGES JF BLOOD 40 740 972 029 BELLINGHAM AJ J CLIN INVEST 50 700 971 030 LENFANT C IN: RORTH M 736 972 031 BELLINGHAM AJ TRANS ASSOC AM PHYSICIANS 83 113 970 032 LICHTMAN MA N ENGL J MED 280 240 969 033 CHEN GSH DISSERTATION 974 034 WAYBURNE S IN: MCCANCE RA 967 035 KEITT AS J LAB CLIN MED 84 275 974 036 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 037 METCALF J IN: RORTH M 613 972 038 ROSENTHAL A PEDIATR CLIN NORTH AM 23 327 976 039 TUCKER CR PEDIATR RES 8 355 974 040 WHITMAN V PEDIATRICS 55 83 975 041 FILLER RM N ENGL J MED 281 589 969 CT 1 MULLER NL AM REV RESPIR DIS 121 463 980 2 FRANCIS PWJ AM J DIS CHILD 134 734 980 3 WARWICK WJ AM J MED TECHNOLOGY 48 539 982 4 ROSENTHAL A N ENGL J MED 306 538 982 5 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 6 VICHINSKY EP J PEDIATR 105 15 984 PN 77177 RN 00758 AN 77124877 AU Rosenlund-M-L. Selekman-J-A. Kim-H-K. Kritchevsky-D. TI Dietary essential fatty acids in cystic fibrosis. SO Pediatrics. 1977 Mar. 59(3). P 428-32. MJ CYSTIC-FIBROSIS: dh. DIETARY-FATS: tu. FATTY-ACIDS-ESSENTIAL: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. FATTY-ACIDS: bl. FEMALE. HUMAN. MALE. SODIUM: an. SWEAT: an. THYROID-HORMONES: bl. SUPPORT-U-S-GOVT-P-H-S. AB Analyses of serum lipids of children with cystic fibrosis (CF) have indicated a deficiency in essential fatty acids (EFA). In view of a report that intravenous administration of soybean oil emulsions normalized sweat sodium values in CF children, we studied the effects of orally administered essential fatty acids (as corn oil) for one year. Some improvement was noted in all patients, but no one consistent factor predominated. However, arachidonic acid, which was found only in trace amounts or was absent in sera of all children with CF before the clinical trial, was increased significantly in the sera after the oral administration of EFA for one year. Other findings included a significant decrease in sweat sodium and T3 in most patients. RF 001 ROSENLUND ML NATURE 251 719 974 002 ELLIOTT RB AUST PAEDIATR J 8 217 972 003 ELLIOTT RB PEDIATRICS 57 474 976 004 SHWACHMAN H AM J DIS CHILD 96 6 958 005 GIBSON LE PEDIATRICS 23 545 959 006 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 007 RIVERS JPW LANCET 2 642 975 008 ROBINSON PG PROC INT CF CONG 7TH 25 976 009 DODGE JA PROC INT CF CONG 7TH 68 976 010 SAMUELS CE PROSTAGLANDINS 10 617 975 011 DODGE JA PROC INT CF CONG 7TH 69 976 CT 1 VAUGHAN WJ SCIENCE 199 783 978 2 HAMDI I MONOGR PAEDIATR 10 84 979 3 CHASE HP PEDIATRICS 64 207 979 4 LLOYDSTILL JD PEDIATRICS 64 50 979 5 WOOD RE SOUTH MED J 72 189 979 6 BARRY MM J AM DIET ASSOC 75 446 979 7 CHASE HP J PEDIATR 95 337 979 8 HAAN GJ J CHROMATOGR 162 261 979 9 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 10 ROGIERS V PEDIATR RES 14 1088 980 11 HUBBARD VS AM J CLIN NUTR 33 2281 980 12 SHEPHERD R J PEDIATR 97 351 980 13 HUBBARD VS CLIN CHIM ACTA 102 115 980 14 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 15 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 16 RIVERS JPW BR MED BULL 37 59 981 17 LEVI N RIV ITAL PEDIATR 8 695 982 18 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 19 HARPER TB AM REV RESPIR DIS 126 540 982 20 FARRELL PM J DENT CHILD 50 385 983 21 ROGIERS V EUR J PEDIATR 141 39 983 22 HODGES P J AM DIET ASSOC 84 664 984 23 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 24 FARRELL PM PEDIATR RES 19 104 985 25 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 26 LESTER LA J PARENT ENTERAL NUTR 10 289 986 27 MISCHLER EH PEDIATR RES 20 36 986 28 STEAD RJ PROG LIPID RES 25 305 986 29 DUHAMEL JF ARCH FR PEDIATR 43 229 986 30 CARLSTEDTDUKE J PROC NAT ACAD SCI USA 83 9202 986 PN 77178 RN 00759 AN 77124890 AU Davis-J-A. TI Orchitis and cystic fibrosis [letter]. SO Pediatrics. 1977 Mar. 59(3). P 481-2. MJ CYSTIC-FIBROSIS: co. EPIDIDYMITIS: co. ORCHITIS: co. MN ADOLESCENCE. CASE-REPORT. HUMAN. MALE. EX I wish to report the case of J. J., a 17-1/2-year-old white boy known to have cystic fibrosis since early infancy, who developed an acute left orchitis and epididymitis in May 1975. In my review of the past literature and in my current readings, I have been unable to find any reported cases of acute orchitis and/or epididymitis in a patient with cystic fibrosis. I offer this case as reported and invite comments. RF 001 TAUSSIG LM N ENGL J MED 287 586 972 002 BUMBALO TS PEDIATRICS 43 468 969 003 KAPLAN E N ENGL J MED 279 65 968 004 DI SANTAGNESE PA N ENGL J MED 279 103 968 005 LANDING BH ARCH PATHOL 88 569 969 006 HOLSCLAW DS J UROL 106 568 971 PN 77179 RN 00760 AN 77193178 AU Gayton-W-F. Friedman-S-B. Tavormina-J-F. Tucker-F. TI Children with cystic fibrosis: I. Psychological test findings of patients, siblings, and parents. SO Pediatrics. 1977 Jun. 59(6). P 888-94. MJ CYSTIC-FIBROSIS. FAMILY. PERSONALITY. MN ADAPTATION-PSYCHOLOGICAL. ADOLESCENCE. ADULT. AFFECTIVE-SYMPTOMS: et. CHILD. FEMALE. HUMAN. INTERPERSONAL-RELATIONS. MALE. MIDDLE-AGE. MMPI. PARENTS. PERSONAL-SATISFACTION. PERSONALITY-TESTS. STRESS-PSYCHOLOGICAL. AB Psychological evaluation of 43 families, each with a child with cystic fibrosis, is reported. Personality testing of the parents showed that 32% of the fathers and 22% of the mothers obtained scores in the range suggestive of emotional disturbance. The Family-Concept O Sort assessment of family interaction indicated that the primary effect of having a child with cystic fibrosis was in terms of decreased family satisfaction and family adjustment. The results do not support previous estimates of an increased incidence of emotional disturbance in children with cystic fibrosis. Evidence for negative psychological impact of chronic illness on sibling development was also lacking. Future research would benefit from a focus on the strengths and resilience of children with cystic fibrosis and their families. RF 001 GAYTON WF AM J DIS CHILD 126 856 973 002 LAWLER RH CAN MED ASSOC J 94 1043 966 003 MCCOLLUM AT J PEDIATR 77 571 970 004 TROPAUER A AM J DIS CHILD 119 424 970 005 TURK J PEDIATRICS 34 67 964 006 MEYEROWITZ JH SOC SCI MED 1 249 967 007 NOVAK AL FAM PROC 9 157 970 008 DAHLSTROM WG MMPI HANDBOOK 960 009 PIERS EV MANUAL PIERS HARRIS CHILDRENS 969 010 SINES JO MANUAL FOR THE MISSOURI CHILD 971 011 HOLTZMAN WH INKBLOT PERCEPTION AND PERSON 961 012 FITTS WH MANUAL FOR THE TENN SELF-CONC 965 013 ROTTER JB PSYCHIATR MONO 80 966 014 ANDERSON LM J CONSULT CLIN PSYCHOL 33 575 969 015 LIVERANT S J CONSULT CLIN PSYCHOL 23 256 959 016 WOLKING WD J CLIN PSYCHOL 22 39 966 017 MCCOLLUM AT AM J PUBLIC HEALTH 61 1335 971 018 PIERS EV J CONSULT CLIN PSYCHOL 38 428 972 019 TAVORMINA JB J ABNORM CHILD PSYCHOL 4 99 976 020 PLESS IB CHRONIC CHILDHOOD DISORDER 975 021 HAMBURG DA PSYCHOTHER PSYCHOSOM 23 13 974 CT 1 BOLL TJ J PSYCHOSOM RES 22 209 978 2 BARRY MM J AM DIET ASSOC 75 446 979 3 CAIRNS NU J PEDIATR 95 484 979 4 HODGMAN CH J PEDIATR 95 309 979 5 VANCE JC PEDIATRICS 65 948 980 6 DROTAR D PEDIATRICS 67 338 981 7 LEWIS BL J PEDIATR 101 636 982 8 BRESLAU N AM J DIS CHILD 136 682 982 9 STEINHAUSEN HC J AM ACAD CHILD PSYCHIAT 22 559 983 10 MCKEEVER P AM J ORTHOPSYCHIATRY 53 209 983 11 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 12 SABBETH B PEDIATR CLIN NORTH AM 31 47 984 13 DEWET B S AFR MED J 65 526 984 14 ORR DP J PEDIATR 104 152 984 15 LEWISTON NJ SEM RESPIR MED 6 321 985 16 BRESLAU N J AM ACAD CHILD PSYCHIAT 24 87 985 17 JOHNSTON IDA BR MED J 290 1636 985 18 ALLEN L J AM ACAD CHILD PSYCHIAT 25 708 986 19 COWEN L PEDIATRICS 77 745 986 20 MEARNS MB J ROY SOC MED 79 51 986 21 NOLAN T J PEDIATR 109 201 986 22 MOLLERING M KLIN PAEDIATR 198 369 986 23 WALKER LS PEDIATRICS 79 239 987 PN 77180 RN 00761 AN 78073115 AU Heycock-J-B. TI Pulmonary diseases in children. SO Practitioner. 1977 Nov. 219(1313). P 670-80. MJ RESPIRATORY-TRACT-DISEASES. MN ASTHMA: dt. BRONCHIECTASIS. BRONCHITIS: di, th. BRONCHOPNEUMONIA: co. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS. HUMAN. INFANT. INFANT-NEWBORN. PNEUMONIA: th. RESPIRATORY-TRACT-DISEASES: co. RESPIRATORY-TRACT-INFECTIONS: th. TUBERCULOSIS-PULMONARY: co, di, dt. EX Respiratory infections in infants and children cause much ill-health and also an appreciable mortality. They are found at times in most school-children and in infants, especially those in large families and those living in poor circumstances. In all respiratory infections the whole respiratory tract is involved. For descriptive purposes it is useful to divide these infections into upper respiratory-tract infections, in which the infection is predominantly at the larynx or above; and lower respiratory-tract infections, in which the predominant infection is below the larynx. Acute upper respiratory-tract infections are usually viral and the poly-virus infection of coryza is the commonest. Acute lower respiratory-tract infections are discussed in two groups: in infants under two years of age and in children over two (including cystic fibrosis). RF 001 GARDNER PS BR MED J 2 932 967 002 HEYCOCK JB BR J CLIN PRACT 10 457 972 PN 77181 RN 00762 AN 77148663 AU Swann-I. TI Anorexia nervosa--a difficult diagnosis in boys. Illustrated by three cases. SO Practitioner. 1977 Mar. 218(1305). P 424-7. MJ ANOREXIA-NERVOSA: di. MN ADOLESCENCE. ANOREXIA-NERVOSA: et. CASE-REPORT. CEREBRAL-VENTRICLE-NEOPLASMS: di. CHILD. CYSTIC-FIBROSIS: di. DIAGNOSIS-DIFFERENTIAL. HUMAN. MALE. SEX-FACTORS. EX The three patients described here have all been seen recently at the Royal Hospital for Sick Children in Edinburgh, and each has presented a diagnostic problem. In the three cases presented it has been difficult to establish the true diagnosis. In the first case, symptoms compatible with the diagnostic criteria of Beumont and his colleagues were directly the result of a slow-growing cerebral neoplasm which was elusive to diagnose. The second patient, a boy with cystic fibrosis, showed symptoms strongly suggestive of anorexia nervosa and responded to dietary measures without pancreatic enzyme replacement. We have been unable to demonstrate any organic disease in the third case and, hence, a presumptive diagnosis of anorexia nervosa has been made. It remains to be said that this condition is extremely rare in boys and that anorexia with weight loss warrants extremely thorough investigation. RF 001 BELL ET ACTA ENDOCRINOL 51 140 966 002 BEUMONT PJV PSYCHOL MED 2 216 972 003 ANON BR MED J 4 686 972 004 COBB S BORDERLANDS OF PSYCHIATRY 943 005 GARFINKEL PE ARCH GEN PSYCHIAT 32 739 975 006 GULL WW TRANS R SOC LOND 7 22 874 007 KIDD CB POSTGRAD MED J 42 443 966 008 NEMIAH JC MEDICINE 29 225 950 009 RUSSELL GFM J PSYCHOSOM RES 9 79 965 010 SEAVER RL ADV PSYCHOSOMATIC MED 7 257 972 011 SELVINI P IN: MEYER JE 965 012 TAIPALE V PSYCHOSOMATICS 13 236 972 CT 1 ANON S AFR MED J 52 2 977 2 ANON BR MED J 1 5 978 3 HAY GG LANCET 2 574 979 4 ANDERSEN AE PSYCHOSOMATICS 24 1066 983 PN 77182 RN 00763 AN 78053411 AU Warren-Lober-C. TI Manifestations of cystic fibrosis. SO Primary-Care. 1977 Dec. 4(4). P 705-20. MJ CYSTIC-FIBROSIS: di. MN BILIARY-TRACT-DISEASES: pa. CYSTIC-FIBROSIS: co, pa. DIAGNOSIS-DIFFERENTIAL. FEMALE. GASTROINTESTINAL-DISEASES: et. GENITAL-DISEASES-FEMALE: pa. HUMAN. LIVER-DISEASES: pa. LUNG: pa. MALE. SWEAT-GLANDS: pa. SWEATING. WATER-ELECTROLYTE-IMBALANCE: di. AB The diagnosis of cystic fibrosis is usually established by the presence of chronic obstructive lung disease and/or gastrointestinal compromise, together with elevated sweat electrolytes or pathologic changes in tissue. Because of its highly variable genetic penetrance, the disease is characterized by varying degrees of involvement of different exocrine systems. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH PEDIATRICS 3 406 949 003 ANDERSON EL J S CAROLINA MED ASSOC 68 275 972 004 BARTON AD J LAB CLIN MED 88 423 976 005 BARTRAM CI BR J RADIOL 44 195 971 006 BEDROSSIAN CWM HUM PATHOL 7 195 976 007 BEIER FR AM REV RESPIR DIS 94 430 966 008 BIGGS JC LANCET 2 814 963 009 BLACKFAN KD J PEDIATR 13 627 938 010 BLANCK RR JAMA 235 1364 976 011 BOOT T JAMA 209 1498 967 012 COOK CD PEDIATRICS 24 181 959 013 SHWACHMAN H PEDIATRICS 30 389 962 014 ANON CF FND 1974 REP SURVI STUD PA 976 015 DENTON R PEDIATRICS 25 611 960 016 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 017 DI SANTAGNESE PA PEDIATRICS 15 683 955 018 DI SANTAGNESE PA IN: DOWNEY JA 25 974 019 DI SANTAGNESE PA N ENGL J MED 295 481 976 020 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 021 DI SANTAGNESE PA PEDIATRICS 12 549 953 022 DOLAN TF JR CLIN PEDIATR 14 862 975 023 ESTERLY JR THORAX 23 670 968 024 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 025 FANCONI G WIEN MED WOCHENSCHR 86 753 936 026 FOX WW PEDIATRICS 54 293 974 027 GARROD AE Q J MED 6 242 913 028 GHARIB R AM J DIS CHILD 108 499 964 029 GIBSON LE PROC FOR QUANTITATIVE ION 975 030 GRAND RJ CLIN PEDIATR 9 588 970 031 GRAND RJ JAMA 195 993 966 032 HANDWERGER S N ENGL J MED 281 451 969 033 HARPER MH ARCH DIS CHILD 13 45 938 034 HOLSCLAW DS J PEDIATR SURG 9 867 974 035 HOLSCLAW DS PEDIATRICS 48 51 971 036 HUANG NN CF CLUB ABST 43 976 037 HUANG NN J PEDIATR 59 512 961 038 ISENBERG JN AM J GASTROENTEROL 65 134 976 039 KOPITO LE FERTIL STERIL 24 512 973 040 KATTWINKEL J J PEDIATR 82 234 973 041 KESSLER WR PEDIATRICS 8 648 951 042 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 043 LANDAU LI AM REV RESPIR DIS 108 593 973 044 LAPEY A J PEDIATR 84 328 974 045 LOBECK CC IN: STANBURY JB 1605 972 046 LOBER CW AM J DIS CHILD 129 10 975 047 LOBER CW CHEST 66 3 974 048 LIEBERMAN J ANN INTERN MED 82 806 975 049 MAY JR ARCH DIS CHILD 47 908 972 050 MEARNS MB ARCH DIS CHILD 47 902 972 051 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 052 OPPENHEIMER EH J PEDIATR 86 683 975 053 OPPENHEIMER EH J PEDIATR 75 806 969 054 OPPENHEIMER EH J PEDIATR 77 991 970 055 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 056 PONKA JL SURG GYNECOL OBSTET 124 99 967 057 REID L MOD PROBL PEDIATR 10 195 967 058 ANON J PEDIATR 88 711 976 059 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 060 SHWACHMAN H CF CLUB ABST 16 19 975 061 SHWACHMAN H PEDIATRICS 30 389 962 062 SHWACHMAN H PEDIATRICS 55 86 975 063 STRANG R BR J SURG 46 484 958 064 TAUSSIG LM N ENGL J MED 287 586 972 065 TAUSSIG LM RADIOLOGY 106 369 973 066 TAYLOR BW ARCH DIS CHILD 49 133 974 067 THOMAIDIS TS J PEDIATR 63 444 963 068 TOMASHEFSKI JF CHEST 57 28 970 069 TONZ O MOD PROBL PEDIATR 10 259 967 070 WARING WW ADV PEDIATR 23 401 976 071 WARING WW PEDIATRICS 39 166 967 072 WARWICK WJ MOD PROBL PEDIATR 10 353 967 073 WARWICK WJ PEDIATRICS 34 621 964 074 WARWICK WJ J CHRON DIS 28 609 975 075 WILMSHURST EG PEDIATRICS 55 75 975 076 WENTWORTH P THORAX 23 582 968 077 WOOD RE AM REV RESPIR DIS 113 833 976 PN 77183 RN 00764 AN 78053410 AU Honicky-R-E. TI Cystic fibrosis. SO Primary-Care. 1977 Dec. 4(4). P 693-703. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. ANTIBIOTICS: tu. CHILD. CYSTIC-FIBROSIS: co, dt. FEMALE. GASTROINTESTINAL-DISEASES: di, dt. HUMAN. MALE. SWEATING. AB Cystic fibrosis has been regarded as a disease of infants. Yet, with improved management, a high percentage of patients how survive into young adulthood. In addition, mild cases may not be detected until long after infance. The older patient often has minimal symptoms, and the majority are socially active and productive. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 GIBSON LE PEDIATRICS 23 545 959 004 SHWACHMAN H ADV PEDIATR 7 249 955 005 WARING WW ADV PEDIATR 23 401 976 006 WOOD RE AM REV RESPIR DIS 113 833 976 PN 77184 RN 00765 AN 78094465 AU Epstein-J. Breslow-J-L. Davidson-R-L. TI Increased dexamethasone resistance of cystic fibrosis fibroblasts. SO Proc-Natl-Acad-Sci-USA. 1977 Dec. 74(12). P 5642-6. MJ CYSTIC-FIBROSIS: me. DEXAMETHASONE: pd. OUABAIN: pd. MN AMINOPTERIN: pd. BIOLOGICAL-TRANSPORT: de. CELL-SURVIVAL: de. CELLS-CULTURED. COLCHICINE: pd. ETHACRYNIC-ACID: pd. HUMAN. STROPHANTHIDIN: pd. STRUCTURE-ACTIVITY-RELATIONSHIP. SUPPORT-U-S-GOVT-P-H-S. AB We demonstrate previously that fibroblasts from cystic fibrosis (CF) patients are significantly more resistant to the toxic effects of ouabain than normal human fibroblasts are. Ouabain is generally assumed to act primarily at the level of the cell membrane and to cause killing of cells by inhibiting ion transport. We have therefore examined the ability of normal and CF cells to survive exposure to ethacrynic acid, another inhibitor of ion transport, and to survive colchicine and aminopterin, resistance to which has been associated with membrane alterations in other cells. The effect of dexamethasone, which has a sterol nucleus similar to that of ouabain but is thought to have a different site of cellular action, was also tested. Exposure of the cells to ethacrynic acid, colchicine, or aminopterin did not reveal any differences in survival between normal and CF fibroblasts. However, CF cells survived exposure to dexamethasone significantly better than normal cells did. These results suggest that normal and CF cells do not differ in terms of a generalized resistance to ion transport inhibitors or to drugs that must pass through the membrane to be active. Instead, the results raise the possibility that CF cells have an enhanced resistance to drugs that have the sterol nucleus found in ouabain and dexamethasone. RF 001 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 002 SCHATZMANN JJ HELV PHYSIOL ACTA 11 346 953 003 ERLY D J PHYSIOL (LOND) 214 327 971 004 CARLSEN SA BIOCHIM BIOPHYS ACTA 455 900 976 005 FLINTOFF WF SOMATIC CELL GENET 2 245 976 006 WILSON WE MOL PHARMACOL 6 449 970 007 SIBLEY CH CELL 2 221 974 009 YAMAMOTO KR PROC NAT ACAD SCI USA 71 3901 974 010 DUGGAN DE ARCH BIOCHEM BIOPHYS 109 388 965 CT 1 EPSTEIN J SOMATIC CELL GENET 4 451 978 2 BRESLOW JL CELL 13 663 978 3 BRESLOW JL SCIENCE 201 180 978 4 HARRIS A DEVELOP MED CHILD NEUROL 21 675 979 5 SHWACHMAN H J PEDIATR 95 661 979 6 BRESLOW JL J CELL PHYSIOL 99 343 979 7 KURZ JB SCIENCE 206 1317 979 8 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 9 WEICHSELBAUM RR J CELL PHYSIOL 103 429 980 10 BRESLOW JL SCIENCE 207 1007 980 11 DANIEL A HUM GENET 57 96 981 12 BRESLOW JL N ENGL J MED 304 1 981 13 SEALE TW ANN CLIN LAB SCI 12 415 982 14 KHALID BAK CLIN ENDOCRINOL 18 407 983 15 LANGHOFF E PEDIATR RES 18 488 984 16 PINSKY L ADV HUM GENET 16 299 987 PN 77185 RN 00766 AN 77172959 AU Epstein-J. Breslow-J-L. TI Increased resistance of cystic fibrosis fibroblasts to ouabain toxicity. SO Proc-Natl-Acad-Sci-USA. 1977 Apr. 74(4). P 1676-9. MJ CYSTIC-FIBROSIS: pp. OUABAIN: pd. SKIN: de, pp. MN ADOLESCENCE. ADULT. BIOLOGICAL-TRANSPORT: de. CELL-SURVIVAL: de. DOSE-RESPONSE-RELATIONSHIP-DRUG. DRUG-RESISTANCE. FEMALE. FIBROBLASTS: de, ph. HUMAN. INFANT-NEWBORN. MALE. POTASSIUM: pd. SKIN: ph. SUPPORT-U-S-GOVT-P-H-S. AB Diploid skin fibroblasts derived from several unrelated patients with cystic fibrosis (CF) were tested for resistance to the cytotoxic effects of ouabain in comparison with cells from normal individuals. Cells from CF and normal individuals were plated at low density and exposed to ouabain at different concentrations for 24 hr. Dose- response curves showed that CF cells survived significantly better than did normal cells at all ouabain concentrations from 0.1 nM to 1 micronM in potassium-deficient medium. In medium containing the usual amount of potassium (6.25 mM), survival of the CF cells exposed to ouabain was decreased to that of the normal cells. If the glucose concentration of the potassium-deficient medium was decreased to 10% of the usual amount, the resistance of the CF cells to ouabain disappeared and their survival was identical to that of the normal cells. These findings may be useful in understanding the biochemical basis of CG and its clinical manifestations. RF 001 ANON J PEDIATR 88 711 976 002 ANON CF FND 1973 REP SURVI STUD PA 975 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 MANGOS JA PEDIATR RES 1 436 967 005 TAUSSIG LM LANCET 1 1367 972 006 SCHATZMANN JJ HELV PHYSIOL ACTA 11 346 953 007 SHANK BB J CELL PHYSIOL 87 377 975 008 BAKER RM CELL 1 9 974 009 LEVER JE J CELL PHYSIOL 88 343 976 010 CORSARO CM EXP CELL RES 95 47 975 011 MANKOVITZ R CELL 3 221 974 012 QUISSELL DO NATURE 247 115 974 013 BARRANCO SC J CELL PHYSIOL 88 33 976 014 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 CT 1 EPSTEIN J PROC NAT ACAD SCI USA 74 5642 977 2 BRESLOW JL EXP CELL RES 110 399 977 3 EPSTEIN J SOMATIC CELL GENET 4 451 978 4 BRESLOW JL CELL 13 663 978 5 BRESLOW JL SCIENCE 201 180 978 6 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 7 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 8 EPSTEIN J PROC NAT ACAD SCI USA 76 6396 979 9 BRESLOW JL J CELL PHYSIOL 99 343 979 10 KURZ JB SCIENCE 206 1317 979 11 DAVIS PB PEDIATR RES 14 83 980 12 BRESLOW JL SCIENCE 207 1007 980 13 REZNIK VM PROC NAT ACAD SCI USA 78 7143 981 14 BRESLOW JL N ENGL J MED 304 1 981 15 SEALE TW ANN CLIN LAB SCI 12 415 982 PN 77186 RN 00767 AN 77257159 AU Raeburn-J-A. TI Nutrition and immunity in cystic fibrosis. SO Proc-Nutr-Soc. 1977 May. 36(1). P 77-83. (REVIEW). MJ CYSTIC-FIBROSIS: im. INFECTION: co. NUTRITION-DISORDERS: im. MN ADULT. ANTIBODY-FORMATION. CHILD. CYSTIC-FIBROSIS: co, et. HUMAN. HYPERSENSITIVITY-IMMEDIATE: co. IGA-SECRETORY. IMMUNITY-CELLULAR. IMMUNOGLOBULINS: me. INFANT. KWASHIORKOR: im. LYMPHOCYTES: ph. MUCOUS-MEMBRANE: pp. NUTRITION-DISORDERS: co. NUTRITION. PHAGOCYTOSIS. RESPIRATORY-TRACT-INFECTIONS: co. REVIEW. EX Has too much attention been focused on antibiotic therapy in cystic fibrosis, thereby overshadowing other measures which have led to the improved prognosis? This may well be true and there is no doubt that regular intensive physiotherapy, performed daily by the patients' relatives, plays the major part in improving airway obstruction. More skilful nutritional management including adequate pancreatic replacement therapy and high-energy, high-protein diets will also have had beneficial effects on the respiratory defects. The infected CF patient may not require high doses of antibiotics; he too might benefit more from simple nutritional alterations. It is not new to suggest that nutritional factors are important influences on the resistance to infection of the CF patient. What we can now do is measure the different aspects of the immune response in such patients and those with malnutrition, so that the simplest and most direct therapy can be chosen. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 BEARN AG IN: WALKER G 426 973 003 BEARN AG CLIN GASTROENTEROL 2 515 973 004 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 006 BRYCESON A LANCET 2 685 976 007 CHANDRA RK BR MED J 3 608 974 009 DOUGLAS SD CLIN IMMUNOL IMMUNOPATHOL 5 1 976 010 FAULK WP IN: BRENT L 4 355 974 011 FORBES IJ AUST NZ J MED 2 160 971 012 GEORGE L ARCH DIS CHILD 46 139 971 013 GIBBONS A BR MED J 1 120 976 014 GREEN MN AM J DIS CHILD 100 365 960 015 HARLAND PS LANCET 2 719 965 016 HEYWORTH B IN: BRENT L 4 343 974 017 HILL HR J PEDIATR 84 55 974 019 ANON BR MED J 4 64 973 020 MCCRAE WM IN: APLEY J 4 157 974 021 MCCRAE WM SCOTT MED J 19 187 974 022 MEARNS MB ARCH DIS CHILD 47 5 972 023 MITCHELL-HEGGS PF Q J MED 45 479 976 024 NEWHOUSE M IN: MANGOS JA 319 973 025 RAEBURN JA SCOTT MED J 19 91 974 026 RAEBURN JA IRCS J MED SCI 3 266 975 027 RAEBURN JA IN: EMERY AEH 2 975 028 RAEBURN JA J ANTIMICROB CHEMOTHER 2 107 976 029 RAEBURN JA IN: DAIKOS GK 2 730 974 031 RAEBURN JA CHEMOTHERAPY 4 17 976 032 REDDY V ARCH DIS CHILD 51 871 976 033 REID L IN: MANGOS JA 195 973 034 SCHWARTZ RH AM J DIS CHILD 111 408 966 035 SMYTHE PM LANCET 2 939 971 036 STEPHAN U IN: MANGOS JA 281 973 037 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 038 TAYLOR B LANCET 2 111 973 039 VON FURTH R IN: MACPHEE T 47 972 040 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 041 WALLWORK JC CLIN ALLERGY 6 349 976 042 WARNER JO LANCET 1 990 976 043 WRIGHT WC JR CLIN RES 21 308 973 CT 1 WOOD RE SOUTH MED J 72 189 979 2 ASKANAZI J CRIT CARE MED 10 163 982 3 RISTER M KLIN PAEDIATR 195 334 983 4 IRWIN MM AM J NURS 85 274 985 PN 77187 RN 00768 AN 78094602 AU Sweet-E-M. TI Causes of 'delayed' respiratory distress in infancy. SO Proc-R-Soc-Med. 1977 Dec. 70(12). P 863-6. MJ RESPIRATORY-DISTRESS-SYNDROME: et. RESPIRATORY-INSUFFICIENCY: et. MN BONE-DISEASES-DEVELOPMENTAL: co. CASE-REPORT. CHYLOTHORAX: co. CYSTIC-FIBROSIS: co. DIAGNOSIS-DIFFERENTIAL. HEART-DEFECTS-CONGENITAL: co. HISTIOCYTOSIS-X: co. HUMAN. INFANT. LYMPHANGIECTASIS: cn. PULMONARY-EMPHYSEMA: cn. THORACIC-DISEASES: co. EX There are many causes of respiratory distress staring more than one week after birth in infants who have had no, or only transient and minor, respiratory problems immediately after birth. They may be divided into two broad groups: those with primary pulmonary pathology and those with extrapulmonary pathology with secondary effects on lung expansion. The former category includes Wilson-Mikity syndrome, chylothorax and congenital pulmonary lymphangiectasia, congenital lobar emphysema, cystic fibrosis, hystiocytosis X, and acute infections. The second group includes congenital heart disease, foregut duplication, tracheo-oesophageal fistula, diaphragmatic hernia, and asphyxiating thoracic dysplasia. RF 001 BAKER DH RADIOL CLIN NORTH AM 1 519 963 002 BORNHURST RA RADIOLOGY 83 476 964 003 CAFFEY J PEDIATRIC X-RAY DIAGNOSIS 594 973 004 CAMPBELL AGM BR MED J 1 1322 977 005 CHATRATH RR ARCH DIS CHILD 46 633 971 006 FLOYD FW PEDIATRICS 31 87 963 007 FROSTIN MH AM J DIS CHILD 114 330 967 008 GROSSMAN H RADIOLOGY 85 409 965 009 HODGMAN JE PEDIATRICS 44 179 969 010 JEUNE M ARCH FR PEDIATR 12 886 955 011 JEUNE M PEDIATRIE 9 390 954 012 KIRKPATRICK JA JR PROG IN PEDIATR RADIOL 1 294 967 013 LEAPE LL PEDIATRICS 34 246 964 014 LUCAS RV PEDIATR CLIN NORTH AM 10 781 963 015 NEIMANN N PEDIATRIE 18 387 963 016 NORTHWAY WH JR RADIOLOGY 91 49 968 017 RICE RP RADIOLOGY 87 1021 966 018 ROBINSON MJ ARCH DIS CHILD 50 962 975 019 RUDHE U ANN RADIOL (PARIS) 12 289 967 020 SAUVEGRAIN J PROG IN PEDIATR RADIOL 2 39 969 021 SIEBER WK PEDIATRICS 18 935 956 022 TALNER NS PEDIATRICS 56 562 975 023 TUDOR J CLIN RADIOL 27 65 976 024 WILSON MG AM J DIS CHILD 99 489 960 CT 1 STORM W KLIN PAEDIATR 197 310 985 PN 77188 RN 00769 AN 77125703 AU Griffiths-E-K. Yoonessi-S. Neter-E. TI Antibody response to enterobacterial lipoprotein of patients with varied infections due to Enterobacteriaceae. SO Proc-Soc-Exp-Biol-Med. 1977 Feb. 154(2). P 246-9. MJ ANTIBODIES-BACTERIAL: an. BACTERIAL-PROTEINS: im. ENTEROBACTERIACEAE-INFECTIONS: im. LIPOPROTEINS: im. MN ADOLESCENCE. ADULT. AGED. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DYSENTERY-BACILLARY: im. ENTEROBACTERIACEAE: im. HUMAN. INFANT. MIDDLE-AGE. PERITONITIS: im. RESPIRATORY-TRACT-INFECTIONS: et, im. SALMONELLA-INFECTIONS: im. SEPTICEMIA: im. SUPPORT-U-S-GOVT-P-H-S. EX The present study was initiated to determine the antibody response to lipoprotein of patients with various enterobacterial infections. This investigation was also considered important because patients with these infections respond differently to another antigen common to enterobacteriaceae, the common enterobacterial antigen. Included were 33 patients with cystic fibrosis, complicated by enterobacterial respiratory tract infection. The lipoprotein (LP) antibody levels of healthy subjects and of patients with varied enterobacterial infections were determined. None of the healthy children and only one of the healthy adults had antibodies against LP. In contrast, 18% of the children with peritonitis complicating appendicitis, 24% of patients with enterobacterial infection of the respiratory tract complicating cystic fibrosis, and 42% of adult subjects with bacteremia complicating malignancy, had LP antibodies in the serum. The reason for these striking differences in the immune response to this common antigen remains to be elucidated. RF 001 WEIDEL W ADV ENZYMOL 26 193 964 002 BRAUN V PROC NAT ACAD SCI USA 69 970 972 003 BRAUN V IN: PEETERS H 221 972 004 BRAUN V EUR J BIOCHEM 13 336 970 005 BRAUN V BIOCHEMISTRY 9 5041 970 006 BRAUN V EUR J BIOCHEM 10 426 969 007 BRAUN V J INFECT DIS SUPPL 128 9 973 008 NETER E INFECTION 1 12 973 009 NETER E ANN NY ACAD SCI 66 141 956 010 NETER E N ENGL J MED 261 1162 959 011 RIESEN W KLIN WOCHENSCHR 53 353 975 CT 1 MAYER H CURR TOP MICROBIOL IMMUNOL 1979 99 979 2 MAYER H CURR TOP MICROBIOL IMMUNOL 1979 99 979 3 BLAKE D EXPERIENTIA 36 254 980 4 GRIFFITHS E INFECT IMMUN 47 808 985 PN 77189 RN 00770 AN 77195135 AU Haller-J-O. Heffer-E-T. Kassner-E-G. Pinck-R-L. TI Unusual radiographic manifestations of cystic fibrosis. SO Rev-Interam-Radiol. 1977 Jan. 2(1). P 41-2. MJ CYSTIC-FIBROSIS: ra. MN ACIDOSIS-DIABETIC: ra. ADOLESCENCE. ADULT. CASE-REPORT. DIABETES-MELLITUS: ra. DIAGNOSIS-DIFFERENTIAL. ESCHERICHIA-COLI-INFECTIONS: ra. FEMALE. HUMAN. PNEUMATOSIS-CYSTOIDES-INTESTINALIS: ra. AB The authors report 2 radiographic findings that have not previously been described in patients with cystic fibrosis: (1) the linear form of pneumatosis coli (2) large pancreatic concretions which resembled the pancreatic calcificaitons of hereditary pancreatitis. The patient with pancreatic calcification also had labile diabetes and episodes of ketoacidosis, unusual complications of cystic fibrosis. RF 001 ELLIOTT GB AM J ROENTG RAD THER NUCL MED 89 720 963 002 HUNT CE PEDIATR RES 7 292 973 003 KEYTING WS RADIOLOGY 76 733 961 004 NELSON SW AM J ROENTG RAD THER NUCL MED 115 225 972 005 RING EJ AM J ROENTG RAD THER NUCL MED 117 446 973 006 ROSAN RC AM J DIS CHILD 104 625 962 007 SMITH BH AM J CLIN PATHOL 48 455 967 008 WHITE H RADIOL CLIN NORTH AM 1 539 963 009 WOOD RE AM J DIS CHILD 129 246 975 PN 77190 RN 00771 AN 78013568 AU Sorensen-H. Mygind-N. Tygstrup-I. Winge-Flensborg-E. TI Histology of nasal polyps of different etiology. SO Rhinology. 1977 Sep. 15(3). P 121-8. MJ NASAL-MUCOSA: pa. NASAL-POLYPS: pa. MN ADOLESCENCE. ADULT. AGED. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, pa. EOSINOPHILIA: pa. FEMALE. HUMAN. MALE. MIDDLE-AGE. NASAL-POLYPS: et. AB The aim of the present study is to elucidate the correlation between etiology and histology in nasal polyps with special regard to cystic fibrosis (CF). Nasal polyps from 15 children with CF and a control group of non-CF polyps from 15 adult patients were examined by light- microscopy. The histological evaluation was carried out on a blind basis in order to avoid bias. Among the parameters used, the tissue eosinophilia proved to be the most valuable factor in the differentiation between CF and non-CF polyps, as only few eosinophils were found in the CF-polyps. The polyp glands were few and generally pathological. Some characteristics abnormalities in the grandular morphology are apparently more common in CF polyps. It is concluded, that the histological examination of nasal polyps is of importance for the correct classification of the patient, but the diagnosis of CF cannot be made based on microscopy of polyps. Further studies including blinded histological examination of nasal polypous tissue might contribute to a more differentiated diagnose of nasal polyposis. RF 001$ CAUNA N OTOL RHINOL LARYNG ST LOUIS 81 41 972 002 MAGID SL ARCH OTOLARYNGOL 86 212 967 004 MYGIND N ISIAN PROC TOKYO 120 977 005 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 006 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 007 PAULSEN JW PROC EUR WRK CONF CF WARS 3RD 973 008 SHWACHMAN H AM J DIS CHILD 102 768 961 009 TAYLOR BW ARCH DIS CHILD 49 133 974 011 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 MYGIND N CLIN OTOLARYNGOL 3 325 978 2 MYGIND N CLIN OTOLARYNGOL 7 343 982 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 BENDE M J LARYNGOL OTOL 99 167 985 5 HIRAIDE F ACTA OTOLARYNGOL STOCKH SUPPL 430 5 986 PN 77191 RN 00772 AN 77259376 AU Edge-W-E. Nuss-D. Loening-W-E. TI Late-onset intestinal obstruction in cystic fibrosis--meconium ileus equivalent. SO S-Afr-Med-J. 1977 Aug 6. 52(7). P 271-4. MN ADOLESCENCE. CASE-REPORT. CECAL-DISEASES: et. FEMALE. HUMAN. ILEUM. INFANT. INTESTINAL-OBSTRUCTION: th. JEJUNUM. AB Four patients who suffered from cystic fibrosis and late-onset bowel obstruction (meconium ileus equivalent), and who were treated by surgery, are discussed. The importance of early correct diagnosis is stressed, since surgery may be prevented if bowel washouts and oral medications are given before the condition becomes complicated. RF 001 NOBLETT HR J PEDIATR SURG 4 190 969 002 PERMAN J AM J DIS CHILD 129 1210 975 003 SANTULLI TV IN: MUSTARD WT 2 856 969 004 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 005 DONNISON AB PEDIATRICS 37 833 966 006 MACDONALD JA CAN MED ASSOC J 83 881 960 007 OLIM CB ANN SURG 140 736 954 008 LILLIBRIDGE CB J PEDIATR 71 887 967 009 WAGGET J J PEDIATR 77 407 970 010 RICKHAM PP IN: RICKHAM PP 366 969 011 ROWE MI AM J SURG 125 185 973 PN 77192 RN 00773 AN 77259275 AU Super-M. TI Collecting sweat in patients with cystic fibrosis [letter]. SO S-Afr-Med-J. 1977 Aug 27. 52(10). P 390. MJ CYSTIC-FIBROSIS: di. SPECIMEN-HANDLING: mt. SWEAT. MN CHILD. HUMAN. EX Dr. Soldin describes a method of collecting sweat for the diagnosis of cystic fibrosis which involves placing the patient on an electric blanket and wrapping him warmly for 2 - 5 hours until profuse sweating has occurred for 30 minutes. One important omission from the title and the abstract is any mention of safety. Children with cystic fibrosis may develop heat prostration when exposed to high temperatures, which may cause massive sodium and chloride losses via the sweat. This could occur with the method described. The apparatus described by Gibson and Cooke in the classic pilocarpine iontophoresis method of collecting sweat is neither overly sophisticated nor expensive. It remains the method of choice, although attention to detail is important. RF 001 SOLDIN P S AFR MED J 51 935 977 002 DI SANTAGNESE PA PEDIATRICS 12 549 953 003 SUPER M S AFR MED J 49 818 975 004 GIBSON LE PEDIATRICS 23 545 959 005 ANON J PEDIATR 88 711 976 PN 77193 RN 00774 AN 77216327 AU Jenkins-T. TI The role of screening in the prevention of inherited disease in South Africa. SO S-Afr-Med-J. 1977 Jun 4. 51(23). P 832-7. MJ HEREDITARY-DISEASES: pc. MASS-SCREENING. MN ADULT. LIPOIDOSIS: pc. AMINO-ACID-METABOLISM-INBORN-ERRORS: oc. CYSTIC-FIBROSIS: oc. FEMALE. HEMOGLOBINOPATHIES: oc. HUMAN. HYPERLIPIDEMIA: oc. INFANT. INFANT-NEWBORN. JEWS. MALE. PHENYLKETONURIA: oc. PREGNANCY. PRENATAL-DIAGNOSIS. PSEUDOCHOLINESTERASE: df. SOUTH-AFRICA. HYPERLIPIDEMIA: fg. AB It is now possible to screen for the presence of the carrier state of a number of inherited diseases. Such screening can alert one to the need for antenatal diagnosis where feasible, it can ensure that early treatment is given to affected infants, and it can assist carriers in reaching a decision about having children. Facilities for certain types of screening already exist in some centres in South Africa, and new techniques will be introduced in the future. RF 001 LEVY HL ADV HUM GENET 4 1 973 002 GOLDSTEIN JL J CLIN INVEST 52 1544 973 003 MOTULSKY AG IN: MILUNSKY A 306 975 004 SUPER M S AFR MED J 49 818 975 005 DEAN G PORPHYRIAS: A STORY OF IN 963 006 BENATAR SR S AFR MED J 44 999 970 007 PANNALL PR S AFR MED J 50 304 976 008 JENKINS T THESIS 972 009 JENKINS T S AFR J SCI 71 44 975 010 MORROW A SCIENCE 159 202 962 011 ZAIL S S AFR J MED SCI 27 95 962 012 PATZ IM S AFR MED J 37 377 963 013 BERNSTEIN RE S AFR MED J 37 447 963 014 ZELLWEGER H PEDIATRICS 55 30 975 015 JENKINS T S AFR MED J 51 95 977 016 LANE AB S AFR MED J 50 1553 976 017 BROCK DJH LANCET 2 197 972 PN 77194 RN 00775 AN 78139020 AU Chappell-J-S. TI Management of meconium ileus by resection and end-to-end anastomosis. SO S-Afr-Med-J. 1977 Dec 24. 52(27). P 1093-4. MJ INFANT-NEWBORN-DISEASES: su. INTESTINAL-OBSTRUCTION: su. MN CYSTIC-FIBROSIS: co. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: et. INTESTINAL-OBSTRUCTION: et. MECONIUM. AB Six patients with meconium ileus, in whom bowel resection and end-to- end anastomosis were performed, are presented. Meconium ileus may be simple or complicated. The simple form may be treated by Gastrografin enema. Surgery is indicated if conservative treatment fails and for patients with a complicated obstruction. Provided the bowel is disobstructed and carefully resected, end-to-end anastomosis is preferable. Confirmation of the diagnosis by careful histological examination of the resected bowel is urged. RF 001 HIATT RB SURG GYNECOL OBSTET 87 317 948 002 MCPARTLIN JF ARCH DIS CHILD 47 207 972 003 NOBLETT HR J PEDIATR SURG 4 190 969 004 GROSS RE SURGERY OF INFANCY AND CHILDH 953 005 BISHOP HC ANN SURG 145 410 957 006 SANTULLI TV ANN SURG 154 939 961 007 SWENSON P PEDIATRIC SURGERY 319 958 CT 1 ANON LANCET 1 1000 982 2 MABOGUNJE OA ARCH SURG 117 37 982 PN 77195 RN 00776 AN 78096837 AU Super-M. TI Cystic fibrosis. SO SA-Nurs-J. 1977 Nov. 44(11). P 42-3. MJ CYSTIC-FIBROSIS. MN CYSTIC-FIBROSIS: di, nu. HUMAN. INFANT. INFANT-NEWBORN. AB Cystic fibrosis (C.F.) is a serious genetic disorder occurring mainly among the Whites and starting in early childhood or even at birth. The name is a shortening of the old term Cystic Fibrosis (or Fibrocystic disease) of the pancreas. This comes from the historical appearance at post-mortem first described by Landsteiner in 1905. It was only in 1938 that Dorothee Anderson and Fanconi realised that the disease was a separate entity and not simply a manifestation of other illnesses. Another name is Mucoviscidosis which gives a clue as to one of the most important features of the disease. Mucus formed is thicker and stickier (more viscid) than normal and tends to clog up the bronchi and ducts, particularly the pancreatic ducts. Chronic bronchitis with recurrent severe infection and malabsorption are the main symptoms of the disease. The other important abnormality is an increased amount of sodium chloride (salt) in the sweat. This fact, used in the diagnosis, was first realised during a heat wave in New York in 1953 when it was noticed that most of the children with heat prostration suffered from C.F. Little is known of the causes of the abnormalities of sweat and mucus. Some generalised cell abnormality (? enzymatic) seems possible. There is no doubt that the condition is genetically transmitted and most evidence favours a recessive mode of inheritance. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 FANCONI G SEPARATABDRUCK AUS DER WIENER 27 936 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 SUPER M S AFR MED J 49 818 975 PN 77196 RN 00777 AN 77258751 AU Skude-G. TI On human amylase isoenzymes. SO Scand-J-Gastroenterol [Suppl]. 1977. 12(44). P 1-37. MJ AMYLASES. ISOENZYMES. MN AMYLASES: an, bl. CHRONIC-DISEASE. CYSTIC-FIBROSIS: en. HUMAN. ISOENZYMES: an, bl. REFERENCE-VALUES. PANCREATIC-DISEASES: en. PANCREATIC-JUICE: en. PANCREATIC-NEOPLASMS: en. PANCREATITIS: en. SALIVA: en. SALIVARY-GLAND-DISEASES: en. EX The purpose of the investigation was to elucidate the variation of the isoenzymes of human amylase in health and disease as well as physico-chemical differences between individual isoamylases. In cystic fibrosis determination of serum isoamylase is of value, although absence of pancreatic serum isoamylase is a normal finding during the first year of life. Demonstrable pancreatic serum isoamylase in such early life argues against the diagnosis. After one year of age the test has a positive diagnostic value. RF 001 SKUDE G HEREDITAS 65 277 970 002 SKUDE G SCAND J PLAST RECONSTR SURG 7 105 973 003 SKUDE G SCAND J CLIN LAB INVEST 35 41 975 004 SKUDE G SCAND J GASTROENTEROL 10 577 975 006 SKUDE G ACTA PAEDIATR SCAND 65 145 976 007 SKUDE G SCAND J GASTROENTEROL 11 525 976 008 SKUDE G SCAND J GASTROENTEROL 12 53 977 010 AFONSO E CLIN CHIM ACTA 14 195 966 011 ALPER CA VOX SANG 17 445 969 012 AMMAN RW ANN INTERN MED 78 521 973 013 AW SE NATURE 209 298 966 014 AW SE BIOCHEM J 99 16P 966 015 AW SE GUT 8 402 967 016 BABSON AL CLIN CHEM 14 802 968 017 BAKER RWR SCAND J CLIN LAB INVEST 6 94 954 018 BENJAMIN D AM J CLIN PATHOL 62 752 974 019 CARLIER A CLIN CHIM ACTA 47 249 973 020 CESKA M EXPERIENTIA 25 555 969 021 COHNHEIM J VIRCHOWS ARCH PATHOL PHYSIOL 28 267 972 022 DAVIES TJ J CLIN PATHOL 25 266 972 023 DREILING DA ANN INTERN MED 58 235 963 024 FRIDHANDLER L CLIN CHEM 18 1493 972 025 GOT R CLIN CHIM ACTA 22 545 968 026 HAMMERTON JL IN: BERGSMA D 6 976 027 HOBBS JR IN: DUBACK UC 308 968 028 HOEKE JOO CLIN CHIM ACTA 16 171 967 029 KAMARYT J HUMANGENETIK 1 579 965 030 KAMARYT J HUMANGENETIK 3 41 966 031 KAUFFMAN DL ARCH BIOCHEM BIOPHYS 137 325 970 032 KELLER PJ BIOCHEMISTRY 10 4867 971 033$ KELLEY ML JAMA 164 406 967 034 KLEIN B ANAL BIOCHEM 31 412 969 035 LAURELL CB ANAL BIOCHEM 10 358 965 036 LEGAZ ME CLIN CHEM 22 57 976 037 LEVITT MD CLIN RES 23 394A 975 038 LEVINE RI N ENGL J MED 292 329 975 039 LUNDH G GASTROENTEROLOGY 42 275 962 040 MAGENDIE M C R ACAD SCI (D)(PARIS) 23 189 846 042 MARKERT CL PROC NAT ACAD SCI USA 45 753 959 043 MAYO JW ARCH BIOCHEM BIOPHYS 163 498 974 044 MCGEACHIN RL J BIOL CHEM 234 795 959 045 MEITES S CRC CRIT REV CLIN LAB SCI 2 103 971 046 MUUS J CR TRAV LAB CARLSBERG SER CHI 28 317 953 047 MUUS J NATURE 204 283 964 048 NORBY S EXP CELL RES 36 663 964 049 OGER A CLIN CHIM ACTA 13 670 966 050 OJALA K SCAND J CLIN LAB INVEST 35 163 975 051 PAZUR JH IN: WHISTLER RL 1 962 052 PIMSTONE NR CLIN CHEM 10 891 964 053 RINDERKNECHT H EXPERIENTIA 23 805 967 054 ROBYT JF ARCH BIOCHEM BIOPHYS 122 8 967 055 ROSALKI SB J CLIN PATHOL 23 373 970 056 SAX SM CLIN CHEM 17 311 971 057 SCHIWARA HW Z KLIN CHEM KLIN BIOCHEM 11 319 973 058 SKUDE G HUMANGENETIK 12 255 971 059 SKUDE G SCAND J CLIN LAB INVEST 36 399 976 061 SKUDE G SCAND J GASTROENTEROL 11 17 976 062 SKUDE G AM J OBSTET GYNECOL 126 652 976 063 SKUDE G ACTA MED SCAND 201 53 977 064 SKUDE G GUT 17 127 976 065 SOMOGYI M CLIN CHEM 6 23 960 066 SPIEKERMAN AM CLIN CHEM 20 324 974 067 STEINORTH AM POSTGRAD MED 55 103 974 068 STIEFEL DJ BIOCHIM BIOPHYS ACTA 302 345 973 069 STIEFEL DJ CLIN CHEM 21 343 975 070 SUDO K CLIN CHIM ACTA 73 1 976 071 TAKEUCHI T CLIN CHIM ACTA 60 207 975 072 TAKEUCHI T CLIN CHIM ACTA 54 137 974 073 TAUSSIG LM PEDIATRICS 54 229 974 074 VACIKOVA A J CHROMATOGR 69 349 972 075 WARSHAW AL N ENGL J MED 292 325 975 076 WESTROM L AM J OBSTET GYNECOL 126 657 976 077 WILDING P CLIN CHIM ACTA 8 918 963 078 WILDING P CLIN CHIM ACTA 12 97 965 079 WILDING P ANN INTERN MED 60 1053 964 080 WOHLGEMUTH J BERL KLIN WSCHR 47 92 910 081 WOLF RO AM J CLIN PATHOL 65 1022 976 082 WOLF RO NATURE 213 1128 967 CT 1 SOYZA KD HUM GENET 45 189 978 2 STEPAN J CLIN CHIM ACTA 91 263 979 3 VINICOR F ANN INTERN MED 91 200 979 4 FRIDHANDLER L CLIN CHIM ACTA 101 135 980 5 SKRHA J DIABETOLOGIA 20 129 981 6 COLLINS REC BR J SURG 69 373 982 7 VENTRUCCI M DIGESTION 28 114 983 8 COLLINS REC SURGERY 93 845 983 9 ANDRIULLI A J CLIN GASTROENTEROL 6 239 984 10 PUGAEV AV SOV MED 53 985 11 ZAKOWSKI JJ CRC CRIT REV CLIN LAB SCI 21 283 985 12 HEINE W Z KLIN MED 40 181 985 13 PACE BW AM J GASTROENTEROL 80 898 985 PN 77197 RN 00778 AN 77174552 AU Hoiby-N. Flensborg-E-W. Beck-B. Friis-B. Jacobsen-S-V. Jacobsen-L. TI Pseudomonas aeruginosa infection in cystic fibrosis. Diagnostic and prognostic significance of Pseudomonas aeruginosa precipitins determined by means of crossed immunoelectrophoresis. SO Scand-J-Respir-Dis. 1977 Apr. 58(2). P 65-79. MJ CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: co. RESPIRATORY-TRACT-INFECTIONS: co. MN ANTIBODIES-BACTERIAL: an. CHRONIC-DISEASE. CYSTIC-FIBROSIS: mo. FEMALE. FOLLOW-UP-STUDIES. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. PRECIPITIN-TESTS. PROGNOSIS. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: di. RESPIRATORY-FUNCTION-TESTS. RESPIRATORY-TRACT-INFECTIONS: di. SPUTUM: mi. AB A total of 133 patients with cystic fibrosis have been followed for up to 5 years with monthly examinations including bacteriological examinations of sputum. Sera from the patients were examined by means of crossed immunoelectrophoresis for the occurence and number of precipitating antibody specificites against Pseudomonas aeruginosa. Poor prognosis in cystic fibrosis was associated with chronic colonization (9 months - more than 5 years) of the respiratory tract with mucoid Pseudomonas aeruginosa, and with an onset of the chronic colonization before puberty. Among the patients with chronic Pseudomonas aeruginosa colonization, poor prognosis was associated with high numbers of precipitins against antigens from these bacteria (up to 61). The number of Pseudomonas aeruginosa precipitins increased on an average with five per year in chronically colonized patients. Rapidly increasing number of precipitins was associated with poor prognosis. Patients with any degree of impairment of the ventilatory function and any changes on the chest radiographs could contract chronic Pseudomonas aeruginosa colonization. Poor ventilatory function and severe changes on the chest radiographs was associated with high numbers of Pseudomonas aeruginosa precipitins and with poor prognosis. Although many O groups of Pseudomonas aeruginosa were found in the chronically colonized group of patients, 53% of the patients harboured strains belonging to O group 3 or 3/9, and the highest numbers of precipitins were found in serum from these patients. RF 001 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 CHRISPIN AR PEDIATR RADIOL 2 101 974 004 DIAZ F J INFECT DIS 121 269 970 005 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 006 DI SANTAGNESE PA N ENGL J MED 277 1399 967 007 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 008 DOGGETT RG INFECT IMMUN 6 628 972 009 HABS I Z HYG 144 218 957 010 HOFF GE SCAND J INFECT DIS 6 333 974 011 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 012 HOMMA JY JAPAN J EXP MED 44 1 974 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 014 HOIBY N ACTA PAEDIATR SCAND 63 843 974 015 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 016 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 017 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 018 HOIBY N SCAND J RESPIR DIS 56 38 975 019 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 020 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 021 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 022 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 023 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 024 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 025 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 026 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 027 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 028 HOIBY N SCAND J RESPIR DIS 57 37 976 029 HOIBY N SCAND J RESPIR DIS 57 103 976 030 HOYBYE G UGESKR LAEGER 132 2007 970 031 LAWSON D IN: WATT PJ 69 970 032 MAY JR ARCH DIS CHILD 47 908 972 033 MEARNS MB ARCH DIS CHILD 47 902 972 034 MIKKELSEN OS ACTA PATH MICROBIOL SCAND (B) 78 163 970 035 NETER E J INFECT DIS SUPPL 130 132 974 036 POLGAR G PULMONARY FUNCTION TESTING IN 971 037 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 039 THERKELSEN AJ MEDICINSK STATISTIK AKADEMISK 968 040 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 041 TURK JL BOLL IST SIEROTER MILAN SUPPL 53 208 974 042 WULFF HR RATIONEL KLINIK 973 043 ZAVALA DC J ALLERGY CLIN IMMUNOL 56 450 975 044 ZIERDT CH J CLIN MICROBIOL 1 521 975 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 OLLING S SCAND J INFECT DIS 1977 7 977 3 SCHIOTZ PO HUM HERED 28 293 978 4 NIELSEN HE ACTA PAEDIATR SCAND 67 443 978 5 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 6 PAPORISZ U MONOGR PAEDIATR 10 31 979 7 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 8 FRIIS B SCAND J INFECT DIS 11 211 979 9 MAYBURY BA ANTIMICROB AGENTS CHEMOTHER 15 494 979 10 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 11 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 12 PAPORISZ U EUR J PEDIATR 130 259 979 13 GOSCINIAK G ARCH IMMUNOL THER EXP 28 619 980 14 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 15 HOIBY N ACTA PATH MICROBIOL SCAND (C) 88 149 980 16 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 17 MOSS RB AM REV RESPIR DIS 121 23 980 18 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 19 PETERSEN NT ACTA PAEDIATR SCAND 70 623 981 20 LEWISTON NJ CHEST 80 389 981 21 ESPERSEN F ACTA PATH MICROBIOL SCAND (B) 89 261 981 22 MOSS RB J PEDIATR 99 215 981 23 FROLAND SS SCAND J INFECT DIS 1981 72 981 24 MALMBORG AS SCAND J INFECT DIS 1981 64 981 25 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 26 GOTZ M CLIN EXP IMMUNOL 50 178 982 27 PITCHERWILMOTT RW ARCH DIS CHILD 57 577 982 28 SZAFF M ACTA PAEDIATR SCAND 71 821 982 29 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 30 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 31 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 32 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 33 ANON LANCET 2 257 983 34 JACQUOT J BULL EUR PHYSIOPATH RESP 19 453 983 35 DORING G INFECT IMMUN 42 197 983 36 MARHAUG G ACTA PAEDIATR SCAND 72 861 983 37 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 38 SZAFF M ACTA PAEDIATR SCAND 72 651 983 39 DORING G J INFECT DIS 147 744 983 40 BOSSO JA DRUG INTEL CLIN PHARM 18 825 984 41 SPEERT DP PEDIATR RES 18 431 984 42 COLLINS MT J CLIN MICROBIOL 19 757 984 43 GRANSTROM M ACTA PAEDIATR SCAND 73 772 984 44 MCCARTHY VP ARCH INTERN MED 144 408 984 45 LITTLEWOOD JM LANCET 1 865 985 46 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 47 SMITH SR J CLIN HOSP PHARM 10 243 985 48 DORING G INFECT IMMUN 49 557 985 49 CONWAY SP ACTA PAEDIATR SCAND 74 107 985 50 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 51 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 52 WILMOTT RW AM J DIS CHILD 139 669 985 53 PEDERSEN SS J ANTIMICROB CHEMOTHER 17 505 986 54 HOIBY N ANNU REV MICROBIOL 40 29 986 55 BRETT MM ARCH DIS CHILD 61 1114 986 56 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 57 KURLAND G PEDIATRICS 78 1097 986 58 LITTLEWOOD JM J ROY SOC MED 79 55 986 59 PHILLIPS BM J ROY SOC MED 79 44 986 60 SUTER S J INFECT DIS 153 902 986 61 BRETT MM ARCH DIS CHILD 62 357 987 62 MOSS RB CHEST 91 522 987 PN 77198 RN 00779 AN 78097268 AU Colman-R-W. Wong-P-Y. TI Participation of Hageman factor dependent pathways in human disease states. SO Thromb-Haemostas. 1977 Dec 15. 38(4). P 751-75. (REVIEW). MJ FACTOR-XII: ph. HEREDITARY-DISEASES: pp. METABOLIC-DISEASES: pp. MN ANGIONEUROTIC-EDEMA: pp. ARTHRITIS: me. BLOOD-COAGULATION-DISORDERS: pp. BLOOD-TRANSFUSION: ae. CORONARY-DISEASE: pp. CYSTIC-FIBROSIS: me. DENGUE: bl. DISSEMINATED-INTRAVASCULAR-COAGULATION: pp. FACTOR-XII-DEFICIENCY: pp. GRAFT-REJECTION. HUMAN. HYPERLIPIDEMIA: pp. HYPERSENSITIVITY: me. KIDNEY: tr. KININOGENS: df. LIVER-CIRRHOSIS: bl. MALIGNANT-CARCINOID-SYNDROME: me, pp. MOLECULAR-WEIGHT. NEPHROTIC-SYNDROME: bl. POLYCYTHEMIA-VERA: pp. POSTGASTRECTOMY-SYNDROMES: me. PREKALLIKREIN. REVIEW. SHOCK-SEPTIC: bl. TRANSPLANTATION-HOMOLOGOUS. TYPHOID: bl. HYPERLIPIDEMIA: fg. AB Abnormalities of Hageman factor dependent pathways have been described in a wide variety of human disease states. Congenital deficiencies of factor XII (Hageman trait) prekallikrein (Fletcher trait) and high molecular weight kininogen (Williams, Fitzgerald and Flaujeac traits) although resulting in profound in vitro changes, do not cause in vivo difficulties. In contrast, deficiency of C1 esterase inhibitor (hereditary angioedema) results in significant morbidity and mortality. Acquired diseases may exhibit decreased synthesis of these three proteins in cirrhosis and dengue fever. In vivo activation of factor XII initiated pathways occur in septic shock, disseminated or localized intravascular coagulation, typhoid fever, polycythemia vera, hyperbetalipoproteinemia, coronary artery disease, nephrotic syndrome, transfusion reactions, hemodialysis and extracorporeal bypass. Activation of both the intrinsic system and tissue mediators contribute to the vasomotor phenomena in carcinoid syndrome and postgastrectomy dumping. Roles for factor XII, prekallikrein and kininogen have been suggested in gouty arthritis, allergic disorders and cystic fibrosis but the evidence is not yet convincing in these disorders. RF 001 AUSTEN KF N ENGL J MED 272 649 965 002 BAGDASARIAN A J BIOL CHEM 248 3456 973 003 BAGDASARIAN A J BIOL CHEM 248 7742 973 004 BAGDASARIAN A J CLIN INVEST 54 1444 974 005 BELL G BR J SURG 52 300 965 006 BLUMEL G BEDEUTUNG DER PLASMAKININ 2751 967 007 BOKISCH VA N ENGL J MED 289 996 973 008 BOUMA BN THROMB HAEMOST 38 136 977 009 BRISELD K ACTA ALLERGOL 31 297 976 010 BROCKLEHURST WE J PHYSIOL (LOND) 191 417 967 011 BROCKWAY WJ J BIOL CHEM 246 4641 971 012 BURROWES CE PROC SOC EXP BIOL MED 138 959 971 013 BUSCH GJ AM J PATHOL 80 1 975 014 BUSCH GJ AM J PATHOL 79 31 975 015 CARVALHEIRA AF HISTOCHEMIE 14 33 968 016 CARVALHO AC N ENGL J MED 290 434 974 017 CARVALHO AC CIRCULATION 50 570 974 018 CARVALHO AC BLOOD 47 669 976 019 CARVALHO AC THROMB RES 8 843 976 020 CARVALHO AC CIRCULATION 56 114 977 021 CASTANIA A BR J PHARMACOL 50 375 974 022 COCHRANE CG J EXP MED 138 1564 973 023 COCHRANE CG J EXP MED 134 986 971 024 COHEN SH J PEDIATR 68 448 966 025 COLLIER HOJ IN: ERDOS EG 305 966 026 COLMAN RW BIOCHEM BIOPHYS RES COMMUN 35 273 969 027 COLMAN RW ANN INTERN MED 85 399 976 028 COLMAN RW J CLIN INVEST 56 1650 975 029 COLMAN RW N ENGL J MED 281 685 969 030 COLMAN RW IN: THOMAS CC 87 972 031 COLMAN RW ANN INTERN MED 71 763 969 032 COLMAN RW J CLIN INVEST 48 23 969 034 COLMAN RW IN: PISANO JJ 487 976 035 CUEVAS P GASTROENTEROLOGY 64 285 973 036 CURRIMBHOY A AM J CLIN PATHOL 65 970 976 037 CUSCHIERI A BR MED J 3 565 971 038 DELBIANCO PL ADV EXP MED BIOL 21 453 972 039 DOEGLAS HMG ARCH DERMATOL 110 382 974 040 DOEGLAS HMG ARCH DERMATOL 111 979 975 041 DONALDSON VH J EXP MED 127 411 968 042 DONALDSON VH AM J MED 35 37 963 043 DONALDSON VH J LAB CLIN MED 89 327 976 044 DONALDSON VH J CLIN INVEST 48 642 969 045 DONALDSON VH J CLIN INVEST 43 2204 964 046 DONALDSON VH CLIN RES 25 520A 977 047 EDELMAN R J LAB CLIN MED 86 410 975 048 ERDOS EG IN: MOVAT VASEL HZ 233 969 049 ERDOS EG FED PROC 27 92 968 050 FANCIULLACCI M ADV EXP MED BIOL 70 201 975 051 FORSSMANN WG J CELL BIOL 40 692 969 052 FRANK MM N ENGL J MED 286 808 972 053 GADEK JE CLIN RES 25 357A 977 054 GALLETTI R LO SPERIMENTALE 118 253 968 055 GIGLI I J IMMUNOL 104 574 970 056 GIREY GJD J LAB CLIN MED 80 465 972 057 GJONNAESS H THROMB HAEMOST 28 155 972 058 GJONNAESS H THROMB HAEMOST 28 182 972 059 GLUECK HI ANN INTERN MED 64 390 966 060 GOLDSMITH GH J LAB CLIN MED 89 131 977 061 GREEN J AM J PHYSIOL 226 784 974 062 GRIFFIN JH PROC NAT ACAD SCI USA 73 2554 976 063 HABAL FM BIOCHEM PHARMACOL 23 2291 974 064 HARPEL PC J CLIN INVEST 49 568 970 065 HASIMOTO K ADV EXP MED BIOL 70 245 975 066 HATHAWAY WE BLOOD 26 521 965 067 HATTERSLEY PG BR J HAEMATOL 18 411 970 068 HERMAN CM J LAB CLIN MED 84 731 974 069 HERZHEIMER H J PHYSIOL (LOND) 158 38P 961 070 HIRSCH EF J SURG RES 17 147 974 071 HOAK JC LANCET 2 884 966 072 HOLLENBERG NK TRANSFUSION 6 59 968 073 HONIG GR J PEDIATR 78 633 971 074 IZAKA K TRANSFUSION 15 46 975 075 IZAKA K TRANSFUSION 14 242 974 076 JOHNSON LP SURG FORUM 12 316 961 077 JONASSON O J EXP MED 123 509 966 078 KALLEN RJ PEDIATR RES 9 705 975 079 KAPLAN AP J IMMUNOL 105 802 970 080 KAPLAN AP J EXP MED 133 696 971 081 KAPLAN AP J EXP MED 136 1378 972 082 KATORI M IN: PISANO JJ 553 976 083 KELLERMEYER RW IN: GUTMAN AB 741 964 084 KELLERMEYER RW J LAB CLIN MED 65 307 965 085 KELLERMEYER RW N ENGL J MED 279 859 968 086 KELLERMEYER RW ARTHRITIS RHEUM (SUPPL) 18 765 975 087 KLEMPERER MR J CLIN INVEST 47 604 968 088 LAAKE K THROMB RES 4 285 974 089 LACOMBE MJ BLOOD 46 761 975 090 LACOMBE MJ C R ACAD SCI (D)(PARIS) 280 1039 975 091 LAHIRI B ARCH BIOCHEM BIOPHYS 175 737 976 092 LANDERMAN NS J ALLERGY 33 316 962 093 LANDERMAN NS J ALLERGY 33 330 962 094 LANGE LG AM J MED 56 565 974 095 LIEBERMAN J AM REV RESPIR DIS 109 399 974 096 LIU CY J CLIN INVEST 60 7 977 097 LOPAS H AM J PHYSIOL 225 372 973 098 LUKIAN H ALLERGIC AND IMMUNOLOGIC BAND 18 25 972 099 LUTCHER CL CLIN RES 24 47 976 100 MACDONALD JM AM J SURG 117 204 969 101 MACKAY ME BR J HAEMATOL 11 563 965 102 MANDLE R PROC NAT ACAD SCI USA 73 4179 976 104 MASON DT CIRC RES 17 106 965 105 MASON JW THROMB HAEMOST 26 325 971 106 MASON JW ANN INTERN MED 73 545 970 107 MATHESON RT J CLIN INVEST 58 1395 976 108 MCGRATH JM J EXP MED 129 833 969 109 MEIER HK J CLIN INVEST 60 18 977 110 MEIER JL ANN NY ACAD SCI 283 93 977 111 MELMON KL CLIN CHIM ACTA 12 292 965 112 MELMON KL ARTHRITIS RHEUM 10 13 967 113 MILLER RL J INFECT DIS SUPPL 125 144 973 114 MINNA JD DISSEMINATED INTRAVASCULAR CO 974 115 MOVAT HZ FED PROC 25 682 966 116 NIES AS CIRC RES 22 155 968 117 OATES JA LANCET 1 514 964 118 OATES JA J CLIN INVEST 45 173 966 119 ODONNELL TF SURG GYNECOL OBSTET 143 539 976 120 PASKHINA TS VOPR MED KHIM 20 660 974 121 PENCE HL J ALLERGY CLIN IMMUNOL 53 298 974 122 PETERSON DW J NUTR 101 347 971 123 PHELPS P J EXP MED 124 115 966 124 PHELPS P J LAB CLIN MED 68 433 966 125 PICKERING RJ LANCET 1 326 969 126$ PIERCE JV FOGARTY INT CENT PROC 121 977 127 PITT B TRANS ASSOC AM PHYSICIANS 82 98 969 129 RAO GJS J PEDIATR 80 573 972 130 RAO GJS PEDIATR RES 8 684 974 131 RAO GJS PEDIATR RES 9 739 975 132 RAO GJS SCIENCE 177 610 972 133 RAPAPORT SA J CLIN INVEST 34 9 955 134 RATNOFF OD IN: BROWN EB 5 204 966 135 RATNOFF OD N ENGL J MED 279 760 968 136 RATNOFF OD J CLIN INVEST 34 601 955 137 RATNOFF OD J CLIN INVEST 40 803 961 138 RATNOFF OD J EXP MED 129 315 969 139$ REVAK SD FED PROC 32 845 973 140 ROBINSON JA AM J MED 59 61 975 141 ROCHA E SILVA M MEDICAL EXPERIMENTIA (BASEL) 3 371 960 142 ROCHA E SILVA M AM J PHYSIOL 156 261 949 143 RUDDY S N ENGL J MED 287 545 972 144 SAITO H BLOOD 48 941 976 145 SAITO H NATURE 248 597 974 146 SAITO H J CLIN INVEST 55 1082 975 147 SARDESAI VM J TRAUMA 14 945 974 148 SCHIFFMAN S BR J HAEMATOL 27 101 974 149 SCHIFFMAN S J CLIN INVEST 56 1082 975 150 SEKI T BIOCHEM PHARMACOL 21 1227 972 151 SHERRY S THROMB HAEMOST 16 18 966 152 SICUTERI F ADV EXP MED BIOL 21 445 972 153 SICUTERI F ADV EXP MED BIOL 9 315 970 154 SNYDER A INT ARCH ALLERGY 47 400 974 155 SOLTAY MJ PROC SOC EXP BIOL MED 138 952 971 156 SOTER NA J IMMUNOL 114 928 975 157 STEWART D J CLIN PATHOL 25 410 972 158 TALAMO RC J LAB CLIN MED 74 816 969 159 THUNE P ACTA DERM VENEREOL (STOCKH) 56 217 976 160 VAN ARMAN CG IN: PISANO JJ 471 976 161 VASSALIO G Z ZELLFORSCH MIKROSK ANAT 98 333 969 162 VELTKAMP JM THROMB HAEMOST 17 181 965 163 VENNEROD AM THROMB RES 8 519 976 164 WARDLE EN J CLIN PATHOL 25 1045 972 165 WEBSTER ME IN: SICUTERI F 976 166$ WEBSTER ME FED PROC 32 845 973 167 WERLE E IN: SCHMID E 788 963 168 WIEGERSHAUSEN B ADV EXP MED BIOL 8 221 970 169 WONG PY ANN INTERN MED 77 205 972 170 WONG PY ANN INTERN MED 80 577 974 171 WONG PY J CLIN INVEST 55 691 975 172 WONG PY IN: PISANO JJ 415 976 174 WUEPPER KD IN: LEPOW IH 93 972 175 WUEPPER KD J EXP MED 138 1345 973 176 ZEITLIN IJ IN: SICUTERI F 329 970 177 ZEITLIN IJ IN: PISANO JJ 483 976 178 ZEITLIN IJ ARCH PHARMACOLOGY 293 159 976 179 ZEITLIN IJ LANCET 1 986 966 CT 1 MORRISON DC AM J PATHOL 93 526 978 2 KOVALAINEN S LANCET 1 1035 979 3 GRIFFIN JH SEM THROMB HEMOSTAS 5 254 979 4 WRIGHT IG GEN PHARMAC 10 319 979 5 PRESTON FE BR J HOSP MED 21 232 979 6 DEUTSCH E MONATSSCHR KINDERHEILKD 127 231 979 7 ULEVITCH RJ INFLAMMATION 4 9 980 8 KUWAHARA SS TRANSFUSION 20 433 980 9 CARRETERO OA AM J PHYSIOL 238 F247 980 10 ABE K CLIN ENDOCRINOL METAB 10 577 981 11 BORGES DR BIOCHEM PHARMACOL 30 1065 981 12 SAITO H AM J MED 70 531 981 13 KATO H METH ENZYMOL 80 172 981 14 KAZAL LA CLIN BIOCHEM CONTEM THEO TECH 2 73 982 15 ADDONIZIO VP BIOMATERIALS 3 9 982 16 RAO AK PATHOBIOL ANNU 12 35 982 17 BERGER J TRANSPLANTAT PROC 14 472 982 18 HATHAWAY WE THROMB RES 25 267 982 19 COCHRANE CG ADV IMMUNOL 33 241 982 20 THOMPSON AR THROMB HAEMOST 48 27 982 21 FISHER CA BLOOD 59 963 982 22 GRAHAM JB ADV HUM GENET 13 1 983 23 HEDNER U HAEMOSTASIS 13 219 983 24 AASEN AO ARCH SURG 118 343 983 25 LASSON A SCAND J GASTROENTEROL 19 1 984 26 LASSON A THROMB RES 35 27 984 27 COLMAN RW J CLIN INVEST 73 1249 984 28 HANCOCK W SEM NEPHROL 5 69 985 29 SCHMAIER AH SEM HEMATOL 22 187 985 30 DOTSENKO VL VOPR MED KHIM 31 116 985 31 FULLER PJ KIDNEY INT 29 953 986 32 WIGGINS RC CIRC RES 58 595 986 33 KIESEWETTER H KLIN WSCHR 64 653 986 34 SCHMAIER AH BLOOD 67 119 986 35 LANG M SCHWEIZ MED WOCHENSCHR 116 1681 986 36 SAITO H SEM THROMB HEMOSTAS 13 36 987 37 DELACADENA RA J LAB CLIN MED 109 601 987 PN 77199 RN 00780 AN 78034723 AU Safwenberg-J. Kollberg-H. Lindblom-J-B. TI HLA frequencies in patients with cystic fibrosis. SO Tissue-Antigens. 1977 Oct. 10(4). P 287-90. MJ CYSTIC-FIBROSIS: im. HLA-ANTIGENS. MN ADOLESCENCE. ADULT. CELIAC-DISEASE: im. CHILD. CHILD-PRESCHOOL. FEMALE. GENE-FREQUENCY. HUMAN. HLA-ANTIGENS: an. INFANT. MALE. CELIAC-DISEASE: im. AB Thirty-three patients with Cystic Fibrosis were HLA typed. Relative risk values and phenotype frequencies were calculated. No statistically significant differences in the HLA antigen distribution were found among the patients with Cystic Fibrosis. RF 001 ALBERT ED HISTOCOMPATIBILITY TESTING 221 970 002 BOYUM A SCAND J CLIN LAB INVEST SUPPL 97 77 968 003 FANCONI G WIEN MED WOCHENSCHR 86 753 936 004 FRANKLIN JP AM J DIG DIS 19 149 974 005 GIBSON LE PEDIATRICS 23 545 959 006 GOODCHILD MC J MED GENET 13 417 976 007 GOODCHILD MC ARCH DIS CHILD 48 684 973 008 GOTZ M Z KINDERHEILK 117 183 974 009 HIDE DW ARCH DIS CHILD 44 533 969 010 JONGSMA A HUMANGENETIK 20 195 973 011 KAISER GI HLA AND DISEASE 252 976 012 KATZ AJ PEDIATRICS 57 715 976 013 KEUNING JJ LANCET 1 506 976 014 KISSMEYER-NIELSEN F HISTOCOMPATIBILITY TESTING 381 976 015 LAMM LU ANN HUM GENET 38 383 975 016 LUDVIGSSON J DIABETOLOGICA 13 13 977 017 NORDIO S MINERVA MED 33 967 018 POLYMENIDIS Z LANCET 2 1452 973 019 DI SANTAGNESE PA N ENGL J MED 277 1287 967 020 SHWACHMAN H CF CLUB ABST 4 23 963 021 SVEJGAARD A TISSUE ANTIGENS 4 95 974 022 TAYLOR BW ARCH DIS CHILD 48 692 973 023 THORSBY E VOX SANG 13 194 967 024 WOOLF B ANN HUM GENET 19 251 955 025 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 CT 1 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 2 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 PN 77200 RN 00781 AN 77177039 AU Sheth-K-J. Heimier-R. TI Cystic fibrosis in an infant presenting with metabolic alkalosis. SO Wis-Med-J. 1977 Apr. 76(4). P S47-8. MJ ALKALOSIS: di. CYSTIC-FIBROSIS: co. MN ALKALOSIS: et. CASE-REPORT. HUMAN. INFANT. MALE. EX We recently had an opportunity to study a four-month-old boy with failure to thrive, hypochloremic metabolic alkalosis, high serum aldosterone values, and who was found to have cystic fibrosis. Children with cystic fibrosis have moderately elevated plasma renin and secondarily elevated serum aldosterone value. This is due to chronic sodium chloride loss through sweat. Our patient had high serum aldosterone values associated with only slightly elevated serum cortisol value. High serum aldosterone value increased further despite correcting conditions which stimulate aldosterone secretion. This suggests that long-standing salt depletion is not the only cause for abnormal renin-aldosterone system. RF 001 WINTERS RW IN: WINTERS RW 402 973 002 WINTERS RW IN: WINTERS RW 46 973 003 NEW MI J CLIN INVEST 45 412 966 004 BARTTER FC AM J MED 33 811 962 005 GOTTLIEB RP J PEDIATR 79 930 971 006 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 007 DI SANTAGNESE PA PEDIATRICS 12 549 953 008 KESSLER WR PEDIATRICS 8 648 951 009 DI SANTAGNESE PA JAMA 172 2014 960 010 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 011 KASSIER JP N ENGL J MED 291 773 974 012 SIMOPOULOS AP PEDIATR RES 5 626 971