PN 75001 RN 00168 AN 75125193 AU Wheatley-B. TI The fight against cystic fibrosis. SO AARN-News-Lett. 1975 Jan. 31(1). P 1. MJ CYSTIC-FIBROSIS: th. LONG-TERM-CARE. MN ADAPTATION-PSYCHOLOGICAL. ADOLESCENCE. ADULT. CHILD. FAMILY. HUMAN. RESEARCH. EX Cystic Fibrosis is a generalized hereditary disorder of children, adolescents, and young adults in which there is widespread dysfunction of mucus-secreting glands. The patient's condition may be characterized by one or all of the following: chronic pulmonary disease, pancreatic enzyme deficiency and abnormally high sweat electrolytes. Hence, each patient is treated on an individual basis. Parents tied to a rigid regime imposed by the disease must have help; volunteers are needed to support and assist them at home and at school. Cystic Fibrosis still needs a lot of help, and knowledge and understanding on the part of the nursing profession helps a great deal. PN 75002 RN 00169 AN 75221674 AU Holm-K. Kessing-S-V. TI Conjunctival goblet cells in patients with cystic fibrosis. SO Acta-Ophthalmol (Kbh). 1975 Mar. 53(2). P 167-72. MJ CONJUNCTIVA: pa. CYSTIC-FIBROSIS: pa. MN CHILD. CHILD-PRESCHOOL. EPITHELIUM. HUMAN. EPITHELIUM: cy AB In five patients with cystic fibrosis of the pancreas the mucous glandular system of the conjunctiva was studied, as changes, if any, in the conjunctival goblet cells might be applicable as a diagnostic test in questionable cases. A whole-mount technique was used, specially developed for studying conjunctival goblet cells. In all five cases the qualitative as well as quantitative goblet-cell findings were in accordance with a previously reported normal material. In particular, there were no signs of stagnated secretion. RF 001 BRUCE GM ARCH OPHTHALMOL 63 391 960 002 DANES BS AM J HUM GENET 23 297 971 003 GIBSON LE PEDIATRICS 23 545 959 004 KESSING SV ACTA OPTHAL SUPPL 95 968 005 KESSING SV ACTA OPTHAL 47 642 969 006 NIELSEN EL UGESKR LAEGER 133 1017 971 007 TYGSTRUP I UGESKR LAEG TRANS DAN MED 1 6 972 008 FLENSBORG EW UGESKR LAEG TRANS DAN MED 1 2 972 CT 1 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75003 RN 00170 AN 75221718 AU McWhirter-W-R. TI Plasma tocopherol in infants and children. SO Acta-Paediatr-Scand. 1975 May. 64(3). P 446-8. MJ CELIAC-DISEASE: bl. CYSTIC-FIBROSIS: bl. GIARDIASIS: bl. VITAMIN-E: bl. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. HUMAN. INFANT. AB A study has been made of plasma tocopherol concentrations in normal children and in children with intestinal abnormalities. A positive correlation between plasma tocopherol and age is shown between the ages of 4 months and 10 years. Most patients with cystic fibrosis or coeliac disease were found to have markedly reduced plasma tocopherol concentrations in comparison with the normal children of similar age. RF 001 FILER LJ JR PEDIATRICS 8 328 951 002 GORDON HH PEDIATRICS 21 673 958 003 HARRIES JT ARCH DIS CHILD 46 341 971 004 HARRIS PL AM J PUBLIC HEALTH 36 155 946 005 HASSAN H AM J CLIN NUTR 19 147 966 006 HORWITT MK ANN NY ACAD SCI 203 223 972 007 KERNER I AM J DIS CHILD 99 597 960 008 MARTINEK RG CLIN CHEM 10 1078 964 009 MULLER DPR BIOCHEM J 112 28P 969 010 MULLER DPR ARCH DIS CHILD 45 715 970 011 OSKI FA J PEDIATR 70 211 967 012 RITCHIE JH N ENGL J MED 279 1185 968 013 UNDERWOOD BA PEDIATR RES 6 26 972 014 WRIGHT SW PEDIATRICS 7 386 951 CT 1 FARRELL PM J CLIN INVEST 60 233 977 2 FARRELL PM AM J CLIN NUTR 31 1720 978 3 BOHLES H Z ERNAHRUNGSWISS 18 81 979 4 OGUNMEKAN AO AM J CLIN NUTR 32 2269 979 5 CORASH L N ENGL J MED 303 416 980 6 SOLOMONS NW NUTR REV 39 149 981 7 SOLOMONS NW REV INFECT DIS 4 859 982 8 BIERI JG N ENGL J MED 308 1063 983 9 ALVAREZ F J PEDIATR GASTROENTEROL NUTR 3 390 984 10 KEUSCH GT J ENVIRON PATHOL TOXICOL ONCO 5 165 985 11 OGUNMEKAN AO TROP GEOGR MED 37 175 985 12 HAFEZ M J PEDIATR 108 558 986 13 CYNAMON HA J PEDIATR GASTROENTEROL NUTR 6 46 987 PN 75004 RN 00171 AN 75105999 AU Hultberg-B. Sjoblad-S. Ockerman-P-A. TI Glycosidases in human skin fibroblast cultures. Alpha-fucosidase, alpha-galactosidase, alpha-glucosidase, beta-mannosidase, and N-acetyl-alpha-glucosaminidase. SO Acta-Paediatr-Scand. 1975 Jan. 64(1). P 123-31. MJ FIBROBLASTS: en. GLUCOSIDASES: me. SKIN: cy. MN ADOLESCENCE. CELLS-CULTURED. CHILD. CHROMATOGRAPHY-ION-EXCHANGE. CYSTIC-FIBROSIS: en. FEMALE. GALACTOSIDASES: an. GLUCOSAMINIDASE: an. GLUCOSIDASES: an. GLYCOGENOSIS: en. GLYCOSIDE-HYDROLASES: an. HUMAN. HYDROGEN-ION-CONCENTRATION. ISOELECTRIC-FOCUSING. MALE. MUCOPOLYSACCHARIDOSIS: en. MYOCARDIAL-DISEASES: en. SYNDROME. AB Five glycosidases, alpha-fucosidase, alpha-galactosidase, alpha- glucosidase, beta-mannosidase and N-acetyl-alpha-glucosaminidase were studied in human skin fibroblast cultures. The pH-dependency, kinetic properties of the enzymes and results of isoelectric focusing and ion exchange chromatography are presented. Techniques suitable for diagnosing inborn lysosomal diseases in skin fibroblast cultures are defined. RF 001 BARTHOLOMEW BA BIOCHIM BIOPHYS ACTA 315 123 973 002 BEUTLER E AM J HUM GENET 24 237 972 003 ANON J BIOL CHEM 247 7195 972 004 BOSMANN HB BIOCHIM BIOPHYS ACTA 258 265 972 005 BRADY RO N ENGL J MED 276 1163 967 006 CONCHIE J BIOCHEM J 87 354 963 007 DREYFUS JC BIOCHEM BIOPHYS RES COMMUN 48 914 972 008 DURAND P J PEDIATR 75 665 969 009 VON FIGURA K BIOCHEM BIOPHYS RES COMMUN 48 262 972 010 HAYFLICK L EXP CELL RES 37 614 965 011 HERS HG BIOCHEM J 86 11 963 012 HO MW AM J HUM GENET 24 256 972 013 HULTBERG B ACTA PAEDIATR SCAND 62 474 973 014 KINT JA SCIENCE 167 1268 970 015 KILLBERG R PROC EWGCF 1ST ANNU MTG 16 970 016 LANGLEY TJ ARCH BIOCHEM BIOPHYS 128 312 968 017 LEVVY GA BIOCHEM J 80 435 961 018 LOWRY OH J BIOL CHEM 193 265 951 019 MURAMATSU T J BIOCHEM (TOKYO) 62 700 967 020 OBRIEN JS PROC NAT ACAD SCI USA 69 1720 972 021 ROBINSON D CLIN CHIM ACTA 47 403 973 022 SALAFSKY IS J LAB CLIN MED 81 450 973 023 WEISMANN B BIOCHEMISTRY 6 207 967 024 WIEDERSCHAIN GY CLIN CHIM ACTA 46 305 973 025 BORJESON M NORD MED 81 567 969 CT 1 HULTBERG B BIOCHEM BIOPHYS RES COMMUN 67 1473 975 2 HULTBERG B BIOCHEM J 155 599 976 3 HULTBERG B CLIN CHIM ACTA 80 79 977 4 HULTBERG B HEREDITAS 86 103 977 5 HULTBERG B BIOCHIM BIOPHYS ACTA 481 573 977 6 ANDRIA G CLIN GENET 14 16 978 7 PILZ H FORTSCHR NEUROL PSYCHIAT 46 207 978 8 HULTBERG B HUM HERED 29 250 979 9 PILZ H HUM GENET 47 113 979 10 HULTBERG B CLIN CHIM ACTA 93 221 979 11 CHESTER MA BIOCHIM BIOPHYS ACTA 627 244 980 PN 75005 RN 00172 AN 75106013 AU Rask-Madsen-J. Schiotz-P-O. Bartels-U. Nielsen-M-D. Becher-Christensen-F. TI Electrical polarization of rectal mucosa and excretion of tetrahydroaldosterone in patients with cystic fibrosis of pancreas and in normal subjects. SO Acta-Paediatr-Scand. 1975 Jan. 64(1). P 81-86. MJ ALDOSTERONE: ur. CYSTIC-FIBROSIS: me. INTESTINAL-MUCOSA: me. RECTUM: me. MN ADOLESCENCE. ADULT. BIOLOGICAL-TRANSPORT. CHILD. CYSTIC-FIBROSIS: ur. ELECTROLYTES. FIBROBLASTS. HUMAN. INTESTINAL-ABSORPTION. MEMBRANE-POTENTIALS. SODIUM: ur. AB The electrical potential difference (PD) across the rectal wall was measured in 26 patients with cystic fibrosis of pancreas (CFP) and in 18 healthy subjects. The PDs obtained in normal children were identical to those previously obtained in normal adults. A significantly greater dispersion of the values was observed in CFP. When the patients were divided into groups according to metachromasia in fibroblast cultures, the mean PD was increased only in the ametachromatic group. True enough, this observation suggests a difference between various forms of CFP, distinguished by metachromasia, and thus is a further indication of the heterogeneity of the disease. The greater abnormalities in metachromasia negative patients may, however, be due solely to the fact that these patients are more severely affected by the disease. The urinary excretion of tetrahydroaldosterone in patients was within the ranges obtained in controls, which excludes the possibility of secondary hyperaldosteronism as the source of increased PD. No evidence was provided in favour of a basic defect in the intestinal transport of Na+ or Cl minus, but K+ concentrations in faecal fluids of patients were significantly lower than in controls. The equilibrium concentration of K+ could be accounted for by simple passive diffusion, suggesting that the epithelium behaved inertly with respect to this ion in CFP. RF 001 COFRE G J PHYSIOL (LOND) 188 177 967 002 CONOVER JH LANCET 1 1122 973 003 COOPERSTEIN IL J GEN PHYSIOL 42 461 959 004 DANES BS AM J HUM GENET 23 297 971 005 DARBY CW PROC EWGCF 1ST ANNU MTG 970 006 DEVROEDE GJ GASTROENTEROLOGY 56 101 969 007 EDMONDS CJ LANCET 2 624 970 008 GRADY GF GASTROENTEROLOGY 59 583 970 009 GRAND RJ J PEDIATR 70 346 967 010 GRAND RJ J PEDIATR 70 357 967 011 KAISER D LANCET 1 1003 970 013 MANGOS JA SCIENCE 158 135 967 014 MANGOS JA PEDIATR RES 1 436 967 015 NEW MI J CLIN INVEST 45 412 966 016 NIELSEN MD ACTA ENDOCRINOL 71 498 972 017 RASK MADSEN J SCAND J GASTROENTEROL 8 327 973 018 RASK MADSEN J ACTA MED SCAND 194 311 973 019 RASK MADSEN J SCAND J GASTROENTEROL 9 223 974 020 RASK MADSEN J SCAND J GASTROENTEROL 8 321 973 021 RASK MADSEN J SCAND J GASTROENTEROL 8 169 973 022 SCHALES O J BIOL CHEM 140 879 941 023 SIMOPOULOS AP PEDIATR RES 5 626 971 024 SOLE J ACTA ENDOCRINOL 70 533 972 CT 1 PATTON CJ PEDIATR RES 16 1035 982 2 WINGE P DAN MED BULL 29 358 982 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 BOUCHER RC LUNG 161 1 983 5 KNOWLES MR PEDIATR RES 19 676 985 PN 75006 RN 00173 AN 76036885 AU Darby-C-W. Edmonds-C-J. TI Letter: Measurement of electrical potential difference across colonic mucosa in cystic fibrosis patients. SO Acta-Paediatr-Scand. 1975 Sep. 64(5). P 770. MJ CYSTIC-FIBROSIS: pp. INTESTINAL-MUCOSA: pp. RECTUM. SKIN: pp. MN ACTION-POTENTIALS. CHILD. HUMAN. EX By measuring potential difference between rectal mucosa and perianal skin using a reference electrode placed on the forearm, we demonstrated an altered potential difference in cystic children compared with control adults and normal children. We suggested this might be due to an alteration in the rectal mucosa potential or altered skin potential, or both, in cystic fibrosis. We measured skin and rectal charges directly by connecting our reference electrode to the blood stream through an intravenous line. Using this technique we showed that the cystic fibrosis patients (C.F.) have an abnormal perianal skin potential difference compared with readings we obtained in normal children. There was little difference in the transmural rectal potential difference between our cystic patients and previously reported values in normal adults. Thus the recent paper confirms our findings of normal polarity of the rectal mucosa surface and the increased skin polarisation in cystic fibrosis. RF 001 ARCHAMPONG EQ GUT 13 559 972 002 DARBY CW PROC EWGCF 1ST ANNU MTG 970 003 DARBY CW CF RESEARCH WORKERS CONF MAN 970 004 RASK MADSEN J ACTA PAEDIATR SCAND 64 81 975 CT 1 EDMONDS CJ LANCET 1 937 983 PN 75007 RN 00174 AN 75161694 AU Feigelson-J. Pecau-Y. Cathelineau-L. Navarro-J. TI Additional data on hepatic function tests in cystic fibrosis. SO Acta-Paediatr-Scand. 1975 Mar. 64(2). P 337-44. MJ CYSTIC-FIBROSIS: pp. LIVER-FUNCTION-TESTS. LIVER: pp. MN ADOLESCENCE. ADULT. ALANINE-AMINOTRANSFERASE: bl. ALKALINE-PHOSPHATASE: bl. CHILD. CHILD-PRESCHOOL. CHOLINESTERASES: bl. CYSTIC-FIBROSIS: bl, en. FEMALE. GAMMA-GLUTAMYLTRANSFERASE: bl. HUMAN. IGA. IGG. IGM. INFANT. LEUCINE-AMINOPEPTIDASE: bl. LIVER-CIRRHOSIS: bl, en. MALE. ORNITHINE-CARBAMOYLTRANSFERASE: bl. PROTHROMBIN: me. SERUM-ALBUMIN: me. TRANSFERRIN: me. VITAMIN-B-12: bl. AB Fifty cystic fibrosis (CF) patients, of whom 9 had multilobular cirrhosis, were observed regularly for a period of 3 years and various liver function tests, indicating cytolysis, cholestasis and cellular insufficiency were performed. Immunoglobulin and prothrombin were assayed. In 9 patients with cirrhosis, the tests were generally abnormal. Two distinct biochemical patterns of cirrhosis were distinguished, one clearly cholestatic and the other of a more cellular type. The distinction was made on the basis of the IgA : Transferrin ratio and of gamma-glutamyl-transpeptidase levels. In the non-cirrhotic patients, a temporary increase of cytolysis and cholestasis was observed in 50% of the cases. RF 001 ARON E ANN GASTROENTEROL HEPATOL 9 1 973 002 BABSON AL CLIN CHEM 12 8 966 003 BENHAMOU JP PRESSE MED 79 185 971 004 BERG T ACTA UNIV UPSALIENSIS 969 005 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 006 CRAIG JM AM J DIS CHILD 93 357 957 007 DOMINICK H PROC EWGCF 4TH ANNU MTG 973 008 ELLMAN G BIOCHEM PHARMACOL 7 88 961 009 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 010 FEIGELSON J ACTA PAEDIATR SCAND 61 337 972 011 FEIGELSON J NOUV PRESSE MED 1 1899 972 012 GAUDIER B LILLE MED 15 1391 970 013 GEIGER H Z KINDERHEILK 109 22 970 014 HALLEN J SCAND J CLIN LAB INVEST SUPPL 124 97 972 015 KATTWINKEL J J PEDIATR 82 234 973 016 LELONG M ARCH FR PEDIATR 7 234 950 017 LOGRIPPO GA AM J GASTROENTEROL 54 357 971 019 NAGEL W KLIN WOCHENSCHR 42 447 964 020 REITMAN S AM J CLIN PATHOL 28 1 957 021 RITLAND S SCAND J GASTROENTEROL SUPPL 19 113 973 022 DI SANTAGNESE PA PEDIATRICS 18 387 956 023 SITZMANN FC ARCH KINDERHEILK 175 263 967 024 SNODGRASS PJ J LAB CLIN MED 73 263 969 025 SZASZ G CLIN CHEM 15 124 969 026 TURLA B RIV CLIN PEDIATR 78 485 966 027 TYSON KRT J PEDIATR SURG 3 271 968 028 WILROY RS JR J PEDIATR 68 67 966 CT 1 PECAU Y BIOL GASTROENTEROL 8 193 975 2 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 3 PECAU Y PATHOL BIOL (PARIS) 28 575 980 4 PECAU Y SEM HOP PARIS 57 1306 981 5 PARK RW GASTROENTEROLOGY 81 1143 981 6 CORRIGAN JJ J PEDIATR 99 254 981 7 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 8 MOWAT AP CLIN GASTROENTEROL 11 171 982 9 BERNARD O CLIN GASTROENTEROL 14 33 985 10 FEIGELSON J ANN PEDIATR (PARIS) 32 218 985 11 BAILEY DJ PEDIATRICS 79 281 987 PN 75008 RN 00175 AN 75221705 AU Kollberg-H. Hellsing-K. TI Screening for cystic fibrosis by analysis of albumin in meconium. SO Acta-Paediatr-Scand. 1975 May. 64(3). MJ ALBUMINS: an. CYSTIC-FIBROSIS: di. MECONIUM: an. MN COMPARATIVE-STUDY. GEL-DIFFUSION-TESTS. HUMAN. INFANT-NEWBORN. INFANT-PREMATURE. MASS-SCREENING. AB A clinical study of the albumin content in meconium was performed on two categories of newborn infants: a screening series of 8,830 infants and a high-risk group for Cystic Fibrosis (CF) of 70 infants. A single radial immunodiffusion technique and test strips were used. Three CF infants were detected in the screening series (1:3,000) and 16 in the high-risk group. The diagnostic accuracy for CF was fairly good. The specificity was 99.8% for the immunodiffusion technique and 99.2% for test strips. A high concentration of albumin in meconium was found not only in CF but also in preterm babies and infants with gastrointestinal disturbances, such as atresias, malaena neonatorum and malabsorption syndromes. The sensitivity was 90% for the immunodiffusion technique and 78% for the test strip. False-negative results were probably due to proteolytic activity and might be avoided if the samples are stored at a low temperature before analysis. CF screening of all meconiums by the use of test strips followed by analysis of positive tests by the immunodiffusion technique is suggested. RF 001 BENDER SW MONATSSCHR KINDERHEILKD 119 632 971 002 BERG T NORTHERN PEDIATR CONG 15 967 003 BRAY PT EWGCF SCREENING COMMITTEE 971 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 EGGERMONT E BIOL NEONATE 10 266 966 006 GEORGE L ARCH DIS CHILD 46 139 971 007 GREEN MN PEDIATRICS 41 989 968 008 HADORN B LANCET 1 812 969 009 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 010 HOBBS JR PROTIDES BIOL FLUIDS 17 517 969 011 HURWITT ES AM J DIS CHILD 64 443 942 013 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 014 KOLLBERG H ARCH DIS CHILD 47 836 972 015 LAWSON D ARCH DIS CHILD 47 1 972 016 OPITZ JM BIRTH DEF ORIG ART SER 2 144 969 017 RULE AH PEDIATRICS 45 847 970 018 SHWACHMAN H AM J DIS CHILD 96 6 958 019 SHWACHMAN H PEDIATRICS 46 335 970 020 SHWACHMAN H J PEDIATR 65 645 964 021 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 023 THORNER RM PUBLIC HEALTH MONOGRAPH NO 67 961 024 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 025 WILSON JMG PUBLIC HEALTH PAPERS 45 971 CT 1 PECAU Y BIOL GASTROENTEROL 8 193 975 2 ANON J PEDIATR 88 711 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 CROSSLEY JR LANCET 2 1093 977 5 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 6 CARAMELLO MTG MINERVA PEDIATR 30 989 978 7 ARVANITAKIS C GASTROENTEROLOGY 74 932 978 8 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 9 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 10 RYLEY HC ARCH DIS CHILD 54 92 979 11 DOMINICI R RIC CLIN LAB 10 511 980 12 MASTELLA G RIV ITAL PEDIATR 7 581 981 13 PARK RW GASTROENTEROLOGY 81 1143 981 14 DODGE JA ARCH DIS CHILD 57 774 982 15 HELLSING K ACTA PAEDIATR SCAND 71 827 982 16 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 17 EGGERMONT E ACTA PAEDIATR SCAND SUPPL 317 1985 16 985 18 KOLLBERG H J TROP PEDIATR 32 293 986 PN 75009 RN 00176 AN 76084672 AU Bergan-T. Hoiby-N. TI Epidemiological markers for Pseudomonas aeruginosa. 6. Relationship between concomitant non-mucoid and mucoid strains from the respiratory tract in cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [Suppl]. 1975 Dec. 83(6). P 553-60. MJ CYSTIC-FIBROSIS: mi. PSEUDOMONAS-AERUGINOSA: ip. RESPIRATORY-SYSTEM: mi. MN ADOLESCENCE. ADULT. BACTERIOPHAGE-TYPING. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: et. FEMALE. HUMAN. MALE. PHENOTYPE. PYOCINS. SEROTYPING. AB The simultaneously occurring mucoid (M) and non-mucoid (NM) variants of Pseudomonas aeruginosa frequently observed in cultures from the respiratory tract of chronically infected cystic fibrosis patients have been studied. M cultures in vitro were unstable and easily dissociated NM colonies. In a large proportion of the cases, M and NM variants occurring simultaneously in cultures from one and the same clinical specimen were of the same pyocine type, phage type, and serogroup. In some remaining cases there were small differences between the M and NM variants such that identity between the variants from one and the same specimen was possible, although not definite. The NM dissociants from M strains were of the same type as the M variant. The possible role of cross-infection and the interaction of bacteria and host response factors continuously tending to select the unstable M variant in vivo are discussed. RF 001 BARTELL PF INFECT IMMUN 2 543 970 002 BERGAN T ACTA PATH MICROBIOL SCAND (B) 80 177 972 003 BERGAN T ACTA PATH MICROBIOL SCAND (B) 81 70 973 004 BERGAN T ACTA PATH MICROBIOL SCAND (B) 80 91 973 005 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 1 975 007 BURNS MW BR MED J 3 382 973 008 CARLSON DM BIOCHEMISTRY 5 2817 966 009 DIAZ F J INFECT DIS 121 269 970 010 DOGGETT RG APPL MICROBIOL 18 936 969 011 DOGGETT RG J PEDIATR 68 215 966 012 DOGGETT RG INFECT IMMUN 6 628 972 013 GILLIES RR J PATHOL BACTERIOL 91 339 966 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 015 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 016 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 017 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 018 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 019 JESSEN O PSEUDOMONAS AERUGINOSA AND OT 965 020 KAGAYAMA M J GEN APPL MICROBIOL 16 531 970 021 MARTIN DR J MED MICROBIOL 6 111 973 022 SCHWARZMANN S INFECT IMMUN 3 762 971 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 HOIBY N SCAND J RESPIR DIS 58 65 977 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 6 MARKOWITZ SM INFECT IMMUN 22 530 978 7 SEALE TW J CLIN MICROBIOL 9 72 979 8 MAYBURY BA ANTIMICROB AGENTS CHEMOTHER 15 494 979 9 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 10 THOMASSEN MJ J INFECT DIS 140 873 979 11 ISKHAKOVA KI ZH MIKROBIO EPIDEMIO IMMUNOBI 1979 98 979 12 SAVITSKAYA KI ZH MIKROBIO EPIDEMIO IMMUNOBI 1979 84 979 13 DEMKO CA CURRENT MICROBIOL 4 69 980 14 MICHALSEN H CHEMOTHERAPY 26 135 980 15 WOODS DE INFECT IMMUN 30 694 980 16 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 17 MICHALSEN H SCAND J INFECT DIS 1981 92 981 18 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 19 MOLLER NE EUR J RESPIR DIS 63 130 982 20 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 21 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 22 ISKHAKOVA KI ZH MIKROBIO EPIDEMIO IMMUNOBI 1982 33 982 23 ZIMAKOFF J J HOSP INFECT 4 31 983 24 BRYAN LE J CLIN MICROBIOL 18 276 983 25 SANTINI G BOLL IST SIEROTER MILAN 62 242 983 26 PENKETH A AM REV RESPIR DIS 127 605 983 27 GOLD R PEDIATR INFECT DIS 4 172 985 28 PIER GB J CLIN MICROBIOL 24 189 986 29 ANASTASSIOU ED J CLIN MICROBIOL 25 656 987 30 OGLE JW J INFECT DIS 155 119 987 PN 75010 RN 00177 AN 76084671 AU Hoiby-N. TI Prevalence of mucoid strains of Pseudomonas aeruginosa in bacteriological specimens from patients with cystic fibrosis and patients with other diseases. SO Acta-Pathol-Microbiol-Scand [Suppl]. 1975 Dec. 83(6). P 549-52. MJ CYSTIC-FIBROSIS: mi. PSEUDOMONAS-AERUGINOSA: ip. MN ADOLESCENCE. ADULT. AGED. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. MIDDLE-AGE. PHENOTYPE. RESPIRATORY-SYSTEM: mi. AB The relative prevalence of mucoid strains compared with non-mucoid strains of Pseudomonas aeruginosa has been investigated in all routine bacteriological specimens received in a department of clinical microbiology during 1973. Pseudomonas aeruginosa was isolated from 1054 of the specimens (5.7 per cent) representing 53 patients with cystic fibrosis (551 isolates) and 169 patients with other diseases (503 isolates). The relative prevalence of mucoid strains was significantly higher in specimens from patients with cystic fibrosis (80 per cent) than in specimens from patients with other diseases (3 per cent). Considering patients without cystic fibrosis, the relative prevalence of mucoid strains was low in specimens from all anatomical regions, and no special preference of these strains for the respiratory tract could be demonstrated in these patients, in contrast to the situation in patients with cystic fibrosis. Alternation between mucoid strains and non-mucoid strains in subsequent specimens was observed in 33 of the patients. RF 001 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 002 BURNS MW BR MED J 3 382 973 003 DIAZ F J INFECT DIS 121 269 970 004 DOGGETT RG J PEDIATR 68 215 966 005 DOGGETT RG APPL MICROBIOL 18 936 969 006 DOGGETT RG INFECT IMMUN 6 628 972 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 010 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 011 JESSEN O THESIS 61 965 012 MARTIN DR J MED MICROBIOL 6 111 973 CT 1 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 HOIBY N SCAND J RESPIR DIS 58 65 977 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 6 MARKOWITZ SM INFECT IMMUN 22 530 978 7 SCHIOTZ PO HUM HERED 28 293 978 8 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 27 585 979 9 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 10 OHMAN DE INFECT IMMUN 33 142 981 11 HOIBY N SCAND J INFECT DIS 1981 27 981 12 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 14 DORING G INFECT IMMUN 42 197 983 15 ADLER KB JAMA 249 1615 983 16 WOODS DE J INFECT DIS 151 581 985 17 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 18 ADLER KB AM J PATHOL 125 501 986 PN 75011 RN 00178 AN 75221819 AU Hoiby-N. TI The serology of Pseudomonas aeruginosa analysed by means of quantitative immunoelectrophoretic methods. II. Comparison of the antibody response in man against thirteen O groups of Ps. aeruginosa. SO Acta-Pathol-Microbiol-Scand [B]. 1975 Aug. 83(4). P 328-34. MJ ANTIBODIES-BACTERIAL. ANTIGENS-BACTERIAL. IMMUNOELECTROPHORESIS. PSEUDOMONAS-AERUGINOSA: im. SEROTYPING. MN ANTIBODY-SPECIFICITY. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: im. HUMAN. SEROTYPING: mt. AB The occurrence of antibodies against antigens prepared from strains representing 13 O groups of Pseudomonas aeruginosa and against a polyvalent Ps. aeruginosa antigen (St-Ag) has been investigated in sera from 100 patients. By means of fused rocket immunoelectrophoresis with intermediate gel it was found that the humoral immune response against Ps. aeruginosa resulting in precipitating antibodies will be detected by St-Ag as well as by any other of the antigen samples investigated. Six of the sera contained group-specific antibodies which were revealed by only one of the antigen samples used and not by St-Ag. These six sera were further studied by means of various quantitative immunoelectrophoretic methods using St-Ag as well as antigens prepared from the infecting Ps. aeruginosa strain in the patient concerned. In all six sera, only one extra precipitin could be detected using antigens prepared from the homologous strain instead of St-Ag. This extra precipitin corresponded presumably to group-specific O-antigens not included in St-Ag. In sera from patients, these group-specific antibodies were always accompanied by antibodies against antigens common to all strains of Ps. aeruginosa. RF 001 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 71 973 002 AXELSEN NH SCAND J IMMUNOL SUPPL 2 1 101 973 003 BOCK E J IMMUNOL METH 2 75 972 004 BOCK E SCAND J IMMUNOL SUPPL 1 2 95 973 005 CROWDER JG J LAB CLIN MED 83 853 974 006 DOGGETT RG INFECT IMMUN 6 628 972 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 010 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 012 KROLL J SCAND J IMMUNOL SUPPL 79 973 013 MIKKELSEN OS ACTA PATH MICROBIOL SCAND 73 373 968 014 MIKKELSEN OS ACTA PATH MICROBIOL SCAND (B) 78 163 970 015 SVENDSEN PJ SCAND J IMMUNOL SUPPL 69 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 3 AXELSON NH SCAND J IMMUNOL 5 177 976 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 383 976 6 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 7 CLARKE CW CLIN ALLERGY 7 527 977 8 HOIBY N SCAND J RESPIR DIS 58 65 977 9 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75012 RN 00179 AN 75221805 AU Hoff-G-E. Hoiby-N. TI Staphylococcus aureus in cystic fibrosis: antibiotic sensitivity and phage types during the latest decade. Investigation of the occurrence of protein A and some other properties of recently isolated strains in relation to the occurrence of precipitating antibodies. SO Acta-Pathol-Microbiol-Scand [B]. 1975 Jun. 83(3). P 219-25. MJ CYSTIC-FIBROSIS: mi. STAPHYLOCOCCUS: ip. MN ANTIBIOTICS: pd, tu. ANTIBODIES-BACTERIAL: an. BACTERIAL-PROTEINS: me. BACTERIOPHAGE-TYPING. CYSTIC-FIBROSIS: dt. DRUG-RESISTANCE-MICROBIAL. HUMAN. LIPASE: me. MERCURY: pd. MICROBIAL-SENSITIVITY-TESTS. PRECIPITATION. STAPHYLOCOCCAL-PHAGES. STAPHYLOCOCCUS: im, de, me. AB During the recent decade, 1651 isolates of Staphylococcus aureus from 111 patients with cystic fibrosis have been tested for antibiotic sensitivity and half of the isolates have been phage typed. All the patients were followed in one clinic and the policy of antibiotic treatment was consistent during this period. The results show a dynamic situation where "epidemic" phage types during recent years have been gradually replaced by other types and, during the same period, the prevalence of strains resistant to more than one antibiotic decreased. Multiresistant strains including strains resistant to methicillin were infrequent in these patients. From 23 per cent of the patients, the same strains were repeatedly isolated for more than 1 year despite an apparently successful chemotherapy. Recently isolated strains were found to produce cellbound as well as extracellular protein A. Ninety-one per cent of the strains produced extracellular lipase and only 8 per cent were resistant to mercury chloride. Eighty-one per cent of the patients produced precipitating antibodies against S. aureus as judged by crossed immunoelectrophoresis. The investigated properties of S. aureus were not significantly correlated with the occurrence of precipitating antibodies against these bacteria. The possible significance of protein A in the pathology of the respiratory tract infection is discussed. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BLAIR JE BULL WHO 24 771 961 003 BROWN DFJ J CLIN PATHOL 27 420 974 004 BULOW P ANN NY ACAD SCI 182 21 971 005 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 006 DOSSETT JH J IMMUNOL 103 1405 969 007 EICHENWALD HF IN: MCINTOSH R 89 960 008 FORSGREN A INFECT IMMUN 2 672 970 009 FORSGREN A J IMMUNOL 112 1177 974 010 GUSTAFSON GT J IMMUNOL 98 1178 967 011 GUSTAFSON GT J IMMUNOL 100 530 968 012 HALBERT SP PEDIATRICS 26 792 960 013 HUANG NN J PEDIATR 59 512 961 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 015 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 016 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 019 IACOCCA VF AM J DIS CHILD 106 315 963 020 IACOCCA VF ARCH DIS CHILD 43 220 968 021 JENSEN K THESIS 959 022 JESSEN O N ENGL J MED 281 627 969 023 KRONVALL G INFECT IMMUN 3 10 971 024 LIND I ACTA PATH MICROBIOL SCAND (B) 80 702 972 025 MAY JR ARCH DIS CHILD 47 908 972 026 MEARNS MB ARCH DIS CHILD 47 902 972 027 PITTMAN FE PEDIATRICS 24 40 959 029 ROSENDAL K ACTA PATH MICROBIOL SCAND (B) 79 377 971 030 STALENHEIM G IMMUNOCHEMISTRY 10 501 973 031 WINBLAD S ACTA PATH MICROBIOL SCAND (B) 81 150 973 CT 1 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 U305 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 4 PIVETTA OH PEDIATR RES 11 1133 977 5 HOIBY N SCAND J RESPIR DIS 58 977 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 7 MAYANSKY AN ZH MIKROBIO EPIDEMIO IMMUNOBI 1978 96 978 8 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 9 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 10 CARRET G ZENTRALBL BAKT MIKROB HYG (A) 249 32 981 11 SZAFF M ACTA PAEDIATR SCAND 71 821 982 12 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 14 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 15 ESPERSEN F DAN MED BULL 34 59 987 PN 75013 RN 00180 AN 76133663 AU Winick-M. TI Effects of malnutrition on the maturing central nervous system. SO Adv-Neurol. 1975. 13. P 193-246. (REVIEW). MJ CENTRAL-NERVOUS-SYSTEM: gd. NUTRITION-DISORDERS: pp. MN AGE-FACTORS. AMINO-ACIDS: me. ANIMAL. BEHAVIOR-ANIMAL. BRAIN: gd, cy. CENTRAL-NERVOUS-SYSTEM: pp, me, pa. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. ENVIRONMENT. GLUCOSE: me. HUMAN. INFANT. INFANT-NEWBORN. LEARNING. LIPIDS: me. MENTAL-RETARDATION: et. MYELIN-SHEATH: me. NERVE-TISSUE-PROTEINS: me. NUTRITION-DISORDERS: pa. RATS. REVIEW. EX During recent years, more and more data have been accumulated implicating early malnutrition in subsequent small stature and behavioral abnormalities. These studies can be divided into two large categories: one dealing with cellular and biochemical changes at a tissue level and one dealing with functional changes within the nervous system as measured by changes in various modes of behavior. This chapter will attempt to review the studies in both of these broad categories, which have led to the present concept that malnutrition during the growing period will permanently retard growth, resulting in a stunted individual, and markedly retard psychologic development, resulting in an individual with permanently altered behaviors. We shall first consider the normal cellular and biochemical development of the central nervous system; second, we shall examine how malnutrition alters these cellular and biochemical processes; and finally, we shall review the evidence that implicates early malnutrition with alterations in later behavior and ability to learn. RF 001 MANDEL P IN: RICHTER D 964 002 FISH I PEDIATR RES 3 407 969 003 ALTMAN J J COMP NEUROL 128 431 974 004 ALTMAN J J COMP NEUROL 126 337 966 005 WINICK M FED PROC 29 1510 970 006 DAVISON AN IN: DAVISON AN 968 007 WINICK M PEDIATR RES 2 352 968 008 DOBBING J NATURE 226 639 970 009 HOWARD E BRAIN RES 14 697 969 010 DOBBING J ARCH DIS CHILD 48 757 973 011 BRASEL JA DEVELOP BIOL 23 424 970 012 WINICK M EXP NEUROL 26 393 970 013 DUCKETT S PROC INT CONG OF NEUR 5TH 738 966 014 DUCKETT S REV CAN BIOL 26 173 967 015 DUCKETT S J ANAT 102 183 968 016 DUCKETT S ANAT REC 163 59 969 017 NACHMANSOHN D IN: BARRON ESG 952 018 AUGUSTINSSON KB IN: SUMNER JB 1 950 019 IM HS FED PROC ABST 31 697 972 020 HOLSTEIN IJ J LIPID RES 7 364 966 021 KRITCHEVSKY D BIOCHEM BIOPHYS RES COMMUN 7 128 962 022 ADAMS CWM J NEUROCHEM 4 282 959 023 ROUSER G IN: LAJTHA A 1 969 024 SVENNERHOLM L J LIPID RES 9 570 968 025 WIEGARDT H J NEUROCHEM 14 671 967 027 LE BARON F IN: LAJTHA A 3 970 028 SPERRY WM IN: ELLIOTT KAC 962 029 BRANTE G ACTA PHYSIOL SCAND SUPPL 63 18 1 949 030 CUMINGS JN J NEUROCHEM 2 289 958 031 TINGEY AH J MENT SCI LOND 102 429 956 032 HOWARD E J NUTR 95 111 968 033 ALTMAN J IN: LAJTHA A 2 969 034 BUNGE RP PHYSIOL REV 48 197 968 035 ROSSO P AM J CLIN NUTR 23 1275 970 036 PORCELLATI G BIBL NUTR DIETA 17 16 972 037 MUZZO SJ IN: GARDNER LI 1 973 038 CULLEY WJ FED PROC ABST 30 459 971 039 ROBERTS E IN: KOREY SR 1 956 040 LAJTHA A J NEUROCHEM 3 322 959 041 MACHIYAMA Y BIOCHEM J 96 68 965 042 TSUKADA Y IN: ROBERTS E 960 044 NEAME DK J NEUROCHEM 11 655 964 045 TSUKADA Y J NEUROCHEM 10 241 963 046 GUROFF G J BIOL CHEM 236 1773 961 047 WURTMAN RJ FED MTGS REPORT 972 048 HASLAM RJ BIOCHEM J 88 566 963 049 STRECKER HJ IN: RICHTER D 957 050 CRAVIOTO RO PROC SOC EXP BIOL MED 78 856 951 051 DAWSON RMC BIOCHEM J 47 386 950 052 DE ROFF PS SCIENCE 133 1072 961 053 TALLAN HH J BIOL CHEM 219 257 956 054 CURATOLA A J NEUROCHEM 12 339 965 055 APRISON MH IN: LAJTHA A 3 970 056 BRIDGERS WF J BIOL CHEM 240 4591 965 057 SERENI F BIOL NEONATE 10 254 966 058 SHOEMAKER WJ SCIENCE 171 1017 971 059 ROBERTS S J NEUROCHEM 12 373 965 060 HARPER AE CITED IN: MUNRO HN 2 964 061 CLARK HE IN: ALBANESE AA 2 965 062 RICHTER D J BIOL CHEM 176 1199 948 063 LAJTHA A J NEUROCHEM 1 289 957 064 ROBERTS S IN: LAJTHA A 970 065 PORCELLATI G J NEUROCHEM 5 277 960 066 ANSELL GB BIOCHIM BIOPHYS ACTA 13 92 954 067 SCHAIN RJ SCIENCE 156 984 967 068 ABDEL-LATIF AA J NEUROCHEM 13 1189 966 069 OJA SS ANN ACAD SCI FENN 131 1 967 070 ORREGO F J BIOL CHEM 242 665 967 071 WUNNER WH BIOCHEM J 101 417 966 072 MILLER SA IN: MUNRO HN 969 073 WIDDOWSON EM PROC R SOC LOND BIOL 152 88 960 074 DICKERSON JWT PROC R SOC LOND BIOL 166 396 966 075 JACKSON CM J EXP ZOOL 30 97 920 076 PLATT BS PROC R SOC LOND BIOL 156 337 962 077 WINICK M J NUTR 89 300 966 078 WINICK M J NUTR 95 623 968 079 CULLEY WJ J NUTR 96 375 968 080 DAVISON AN BR MED BULL 22 40 966 081 CHASE HP PEDIATRICS 40 551 967 084 ALLEYNE GAO IN: WINICK M 1 972 085 WINICK M IN: WINICK M 1 972 086 SHOEMAKER WJ SCIENCE 171 1017 971 087 IM HS FED PROC ABST 31 697 972 088 FISH I EXP NEUROL 25 534 969 089 CULLEY WJ FED MTGS REPORT 971 090 PLATT BS IN: MUNRO HR 2 964 091 ZEMAN FJ FED MTGS REPORT 972 092 ZEMAN FJ FED MTGS REPORT 973 093 MANOCHA SL MALNUTRITION AND RETARDED HUM 972 094 WINICK M PEDIATR CLIN NORTH AM 17 69 970 095 WINICK M PEDIATR RES 3 181 969 097 CRAVIOTO J AM J ORTHOPSYCHIATRY 35 449 965 098 COWLEY JJ J GENET PSYCHOL 95 187 959 099 COWLEY JJ J GENET PSYCHOL 103 233 963 100 COWLEY JJ J GENET PSYCHOL 104 89 964 101 COWLEY JJ ANIM BEHAV 14 506 966 102 LEVITSKY DA IN: KALLEN DJ 970 103 LEVITSKY DA NATURE 225 468 970 104 GRIFFITHS WJ J COMP PHYSIOL PSYCHOL 47 41 954 105 BAIRD A BR J NUTR 25 391 971 106 BARNES RH J NUTR 89 399 966 107 COWLEY JJ PSYCHOL ANGLICANA 9 216 962 108 STROBEL DA PSYCHONOMIC SCI 24 19 971 109 KERR RR CITED IN: HARRIS RS 970 110 CALDWELL DF NEUROLOGY (MINN) 17 95 967 111 FRANKOVA S J NUTR 96 485 968 112 SIMONSON M J NUTR 100 685 970 113 BARNES RH J NUTR 100 149 970 114 SIMONSON M J NUTR 101 331 971 115 LAT J PROC R SOC LOND BIOL 153 347 961 116 ALTMAN J DEVELOP PSYCHOBIOL 4 97 971 117 FRANKOVA S J NUTR 96 477 968 118 GUTHRIE HA PHYSIOL BEHAV 3 619 968 119 BARNETT SA DEVELOP PSYCHOBIOL 4 1 971 120 ZIMMERMANN RR J ABNORM PSYCHOL 80 125 972 121 STROBEL DA PSYCHONOMIC SCI 24 19 971 122 STROBEL DA DEVELOP PSYCHOBIOL 5 291 972 123 AAKRE B PERCEPT MOT SKILLS 36 787 973 124 GEIST CR LAB ANIM SCI 22 369 972 125 WISE LA PERCEPT MOT SKILLS 36 674 973 126 PEREGOY PL PERCEPT MOT SKILLS 35 495 972 128 WISE LA BEHAV BIOL 9 77 973 129 ZIMMERMANN RR PROC ANN CONV AM PSYCHOL ASSO 187 970 130 LEVITSKY DA SCIENCE 176 68 972 131 BARNES RH IN: MOORE WM 973 132 CANOSA CA IN: MOORE WM 973 133 GIBSON EJ CITED IN: PRIN PERCEPTUAL LEA 967 134 STOLLNITZ F PSYCHOL REV 72 247 965 135 GUETZKOW HS CITED IN: MEN AND HUNGER 946 136 SMART JL BRAIN RES 28 85 971 137 SEITZ PFD AM J PSYCHIATRY 110 916 954 138 LEVINE S IN: AMBROSE A 969 139 DENENBERG VH SCIENCE 130 629 959 140 DENENBERG VH ENDOCRINOLOGY 81 1047 967 141 ADER R PHYSIOL BEHAV 3 327 968 142 ADER R PHYSIOL BEHAV 4 303 969 143 ADER R PHYSIOL BEHAV 5 837 970 144 DENENBERG VH PSYCHOL REV 71 335 964 145 KRECH D PHYSIOL BEHAV 1 99 966 146 TAPP JJ SCIENCE 140 486 963 147 LEVINE S CAN J PSYCHOL 12 103 958 148 SCHAPIRO S SCIENCE 167 292 970 149 ALTMAN J IN: SCRIMSHAW NS 968 150 ROSENZWEIG MR J COMP PHYSIOL PSYCHOL 55 429 962 151 ROSENZWEIG MR PHYSIOL BEHAV 3 819 968 152 ADER R PSYCHOSOM MED 30 277 968 153 WINICK M J NUTR 89 300 966 154 CULLEY WJ J NUTR 96 375 968 155 BENTON JW PEDIATRICS 38 801 966 156 DOBBING J BRAIN 88 357 965 157 GUTHRIE HA J NUTR 94 419 968 158 EAYRS JT ANAT REC 121 53 955 159 SERENI F BIOL NEONATE 10 254 966 160 ADLARD BPF BRAIN RES 28 97 971 161 ADLARD BPF BRAIN RES 30 198 971 162 IM HS THESIS 971 163 FRANKOVA S IN: SCRIMSHAW NS 968 164 LEVITSKY DA SCIENCE 176 68 972 165 CINES B SYMP ON NUTRITION AND REPORT 972 167 FRANKOVA S SYMP ON EARLY MALNUTRI REPORT 973 168 CABAK V ARCH DIS CHILD 40 532 965 169 CHASE HP PROC AM PEDIATR SOC ANNU MTG 969 170 GARROW JS LANCET 1 1 967 171 GRAHAM GG FED PROC 26 139 967 172 CRAVIOTO J BOL MED HOSP INFANTIL MEX 24 217 967 173 CRAVIOTO J PEDIATRICS PART 2 SUPPL 38 319 966 174 KUGELMASS IN AM J MED SCI 208 631 944 175 LIANG PH AM J CLIN NUTR 20 1290 967 176 CHAMPAKAM S AM J CLIN NUTR 21 844 968 177 BHATIA CM CITED IN: PERFORMANCE TESTS O 958 178 STOCH MB S AFR MED J 41 1027 967 179 STOCH MB ARCH DIS CHILD 38 546 963 180 HERTZIG ME PEDIATRICS 49 814 972 181 CRAVIOTO J J SPECIAL EDUCATION 2 75 967 182 LATHAM MC IN: SCRIMSHAW NS 968 183 STEIN Z SCIENCE 178 708 972 184 LLOYD-STILL JD PROC INT CONF ON NUTRI 9TH 972 185 MCKAY H PROC SYMP SWED NUTR FOUND 12 974 186 CANOSA CA PAN AM HEALTH ORG REPORT 968 188 RUSH D IN: WINICK M 2 974 189 DELICARDIE ER PROC SYMP SWED NUTR FOUND 12 974 190 ZIMMERMANN RR PROC SYMP SWED NUTR FOUND 12 115 974 CT 1 FIGLEWICZ DA EXP NEUROL 67 315 980 2 HAMBERGER A RES EXP MED 180 41 982 3 CONRADI NG ACTA NEUROPATH (BERL) 60 159 983 4 BAKER H J NEUROSCI RES 11 419 984 5 BHARGAVA P LIPIDS 19 179 984 6 PRENSKY AL N ENGL J MED 310 1527 984 PN 75014 RN 00181 AN 75125824 AU Berry-H-K. Kellogg-F-W. Hunt-M-M. Ingberg-R-L. Richter-L. Gutjahr-C. TI Dietary supplement and nutrition in children with cystic fibrosis. SO Am-J-Dis-Child. 1975 Feb. 129(2). P 165-71. MJ CHILD-NUTRITION. CYSTIC-FIBROSIS: dh. DIETARY-CARBOHYDRATES. DIETARY-FATS. DIETARY-PROTEINS. MN ADOLESCENCE. AMINO-ACIDS: bl. BLOOD-PROTEINS: an. BLOOD-UREA-NITROGEN. BODY-HEIGHT. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. CHOLESTEROL: bl. CLINICAL-TRIALS. FEMALE. GLUCOSE: tu. HEMOGLOBINS: an. HUMAN. INFANT. MALE. PROTEIN-HYDROLYSATES: tu. SERUM-ALBUMIN: an. TRANSFERRIN: an. TRIGLYCERIDES: tu. AB Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for a year. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement. RF 001 WEIHOFEN DM J AM DIET ASSOC 54 206 969 002 SHOHL AT J PEDIATR 23 267 943 003 WEST CD AM J DIS CHILD 72 251 946 004 GRACEY M ARCH DIS CHILD 44 401 969 005 ALLAN JD LANCET 1 785 970 006 ALLAN JD AM J DIS CHILD 126 22 973 007 KUMAR V PEDIATRICS 49 736 972 008 RANANATHAN MK INDIAN J MED RES 43 517 955 009 MCFARLANE H LANCET 1 392 969 010 SIMMONS WK BULL WHO 42 480 970 011 ANON RECOMMENDED DIETARY ALLOWANCE 968 012 SONTAG LW J PEDIATR 26 327 945 013 SHWACHMAN H AM J DIS CHILD 96 6 958 014 DOERSHUK CF J PEDIATR 65 677 964 015 ARROYAVE G AM J CLIN NUTR 23 703 970 016 GREULICH WW RADIOGRAPHIC ATLAS OF SKELETA 959 017 SPROUL A J PEDIATR 65 664 964 018 POLLACK JD CF CLUB ABST 13 46 972 CT 1 BERRY HK FED PROC 34 2134 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 MAASER R MONATSSCHR KINDERHEILKD 124 373 976 4 DAUM F PEDIATR RES 12 24 978 5 YASSA JG ARCH DIS CHILD 53 777 978 6 BRADLEY JA BR MED J 1 167 979 7 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 8 CHASE HP PEDIATRICS 64 207 979 9 WOOD RE SOUTH MED J 72 189 979 10 MOSS TJ J PEDIATR 94 32 979 11 CHASE HP J PEDIATR 95 337 979 12 SHEPHERD R J PEDIATR 97 351 980 13 KERZNER B PEDIATR RES 15 250 981 14 SOLOMONS NW AM J CLIN NUTR 34 462 981 15 BELL L J CAN DIET ASSOC 42 62 981 16 PARK RW GASTROENTEROLOGY 81 1143 981 17 BEDDOES V PRACTITIONER 225 557 981 18 PENCHARZ PB J PEDIATR GASTROENTEROL NUTR 2 400 983 19 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 20 DREHER ML CRC CRIT REV FOOD SCI NUTR 20 47 984 21 HODGES P J AM DIET ASSOC 84 664 984 22 BERTRAND JM J PEDIATR 104 41 984 23 MANSELL AL J PEDIATR 104 700 984 24 CANCIANI M J PEDIATR GASTROENTEROL NUTR 4 735 985 25 LEVY LD J PEDIATR 107 225 985 26 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 27 NEIJENS HJ ACTA PAEDIATR SCAND SUPPL 317 1985 38 985 28 LEVY L J PEDIATR GASTROENTEROL NUTR 5 97 986 29 KOHLER JA J CLIN HOSP PHARM 11 21 986 30 SOUTTER VL CLIN GASTROENTEROL 15 137 986 31 PUMARIEGA AJ J AM ACAD CHILD PSYCHIAT 25 269 986 32 MAHANEY MC ARCH ORAL BIOL 31 363 986 33 BUSTAMANTE SA AM J CLIN NUTR 43 891 986 34 OLOUGHLIN E AM J CLIN NUTR 43 732 986 35 MOORE MC AM J CLIN NUTR 44 33 986 36 DODGE JA J ROY SOC MED 79 27 986 PN 75015 RN 00182 AN 75125836 AU Wood-R-E. Herman-C-J. Johnson-K-W. di-SantAgnese-P-A. TI Pneumatosis coli in cystic fibrosis: clinical, radiological, and pathological features. SO Am-J-Dis-Child. 1975 Feb. 129(2). P 246-8. MJ CYSTIC-FIBROSIS: co. PNEUMATOSIS-CYSTOIDES-INTESTINALIS: et. MN ADOLESCENCE. BARIUM-SULFATE: du. CASE-REPORT. CYSTIC-FIBROSIS: pa. HUMAN. INTESTINE-LARGE: pa. MALE. PNEUMATOSIS-CYSTOIDES-INTESTINALIS: ra, pa. RECTAL-PROLAPSE: et. RESPIRATORY-FUNCTION-TESTS. AB A patient with cystic fibrosis was found to have pneumatosis coli associted with rectal prolapse. In cystic fibrosis there are several factors predisposing to pneumatosis, yet it is rarely reported. Symptoms are nonspecific, and the diagnosis should be considered in patients with cystic fibrosis or other chronic lung disease and vague abdominal complaints. RF 001 ECKER JA AM J GASTROENTEROL 56 125 971 002 KOSS LG ARCH PATHOL 53 523 952 003 DOUB HP JAMA 172 1238 960 004 KEYTING WS RADIOLOGY 76 733 961 005 CREEVEY K N ENGL J MED 259 912 958 006 MARSHAK RH AM J DIG DIS 1 99 956 007 SMITH WF GASTROENTEROLOGY 35 528 958 008 WHITE H RADIOL CLIN NORTH AM 1 539 963 009 KULCZYCKI LL N ENGL J MED 259 409 958 010 NELSON SW AM J ROENTG RAD THER NUCL MED 115 225 972 CT 1 OLMSTED WW GASTROINTEST RADIOL 1 177 976 2 EIMOTO T ACTA PATHOL JAP 28 481 978 3 KLEINMAN PK AM J DIS CHILD 134 1149 980 4 DORSTEN JF J AM OSTEOPATH ASSOC 81 123 981 5 WALL LL J PEDIATR 101 745 982 6 BALI A BR MED J 287 1011 983 7 KLIEGMAN RM N ENGL J MED 310 1093 984 8 HUBBARD VS SEM RESPIR MED 6 299 985 9 PIETERSE AS HUM PATHOL 16 683 985 10 AMMONS MA TRANSPLANTAT PROC 18 1868 986 11 HERNANZSCHULMAN M RADIOLOGY 160 497 986 12 YEAGER AM PEDIATR RADIOL 17 18 987 PN 75016 RN 00183 AN 75181297 AU Athreya-B-H. Borns-P. Rosenlund-M-L. TI Cystic fibrosis and hypertrophic osteoarthropathy in children. Report of three cases. SO Am-J-Dis-Child. 1975 May. 129(5). P 634-7. MJ CYSTIC-FIBROSIS: co. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: co. MN ADOLESCENCE. ASPIRIN: tu. CHILD. CHRONIC-DISEASE. FEMALE. HUMAN. JOINT-DISEASES: dt. MALE. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: ra. PERIOSTEUM: pa. SYNOVITIS: pa. AB Three patients with cystic fibrosis were noted to have swelling of knee and ankle joints during exacerbation of their lung disease. Synovial fluid was analyzed in one patient and the synovium underwent biopsy in another. These studies excluded other causes of arthritis but did not contribute any new information on the nature of secondary hypertrophic osteoarthropathy. Radiological examination of long bones confirmed the diagnosis of hypertrophic osteoarthropathy in all three patients. Since many patients with cystic fibrosis survive longer, more instances of hypertrophic osteoarthropathy are expected in the future. RF 001 CAVANAUGH JJA J PEDIATR 66 27 965 002 GROSSMAN H AM J DIS CHILD 107 1 964 003 VAN LEERSUM HG J BELGE RADIOL 49 65 966 004 BRUSILOW SW ANNU REV MED 21 99 970 005 TOMASHEFSKI JF CHEST 57 28 970 006 HOWELL DS IN: HOLLANDER JL 1120 966 007 MENDLOWITZ M MEDICINE 21 269 942 008 HOLLING HE JAMA 178 977 961 009 GREENFIELD GB AM J ROENTG RAD THER NUCL MED 101 927 967 010 GALL EA AM J PATHOL 27 349 951 011 ROPES MW SYNOVIAL FLUID CHANGES IN JOI 89 953 012 RACOCEANU SN ANN INTERN MED 75 933 971 013 GINSBURG J LANCET 2 1274 961 014 STEINER H LANCET 1 783 968 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 NATHANSON I RADIOLOGY 135 649 980 3 BELUFFI G EUR J PEDIATR 139 199 982 4 DELUMLEY L ARCH FR PEDIATR 40 723 983 5 LESTER LA SEM RESPIR MED 6 285 985 6 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 7 PITTSTUCKER TJ ARCH DIS CHILD 61 576 986 8 PHILLIPS BM J ROY SOC MED 79 44 986 9 COHEN AM AM J DIS CHILD 140 74 986 PN 75018 RN 00184 AN 76059172 AU Meyers-A. Dolan-T-F-Jr. Mueller-D. TI Compliance and self-medication in cystic fibrosis. SO Am-J-Dis-Child. 1975 Sep. 129(9). P 1011-3. MJ ANTIBIOTICS: ur. CYSTIC-FIBROSIS: dt. PATIENT-COMPLIANCE. SELF-MEDICATION. MN ADOLESCENCE. ADULT. ANTIBIOTICS: tu. BIOLOGICAL-ASSAY. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. MALE. AB Sixty-one patients with cystic fibrosis were studied to determine the relationship between degree of compliance with taking antibiotics and severity of the disease. The compliance rate was high in these patients, suggesting that perception of the severity of the disease and the potential consequences of discontinuing medication is a principal factor. RF 001 FRANCIS V N ENGL J MED 280 535 969 002 DAVIS MS AM J PUBLIC HEALTH 58 274 968 003 CHARNEY E PEDIATRICS 40 188 967 004 COLCHER IS JAMA 222 657 972 005 LEISTYNA JA AM J DIS CHILD 111 22 966 006 CHARNEY E PEDIATR CLIN NORTH AM 19 263 972 007 WATKINS JD AM J PUBLIC HEALTH 57 452 967 008 CARON H AM J MED SCI 261 61 971 009 GORDIS L J PEDIATR 75 957 969 010 DONABEDIAN A J CHRON DIS 17 847 964 011 BECKER MH J PEDIATR 81 843 972 012 GROVE DC ASSAY METH OF ANTIBIOTICS 955 013 MARKOWITZ M PEDIATRICS 41 151 968 014 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 STAPLETON FB N ENGL J MED 295 246 976 3 SHWACHMAN H MEDICINE 56 129 977 4 SUBLETT JL ANN ALLERGY 43 95 979 5 KUMAR A POSTGRAD MED 65 165 979 6 ASHBURN FS SURV OPHTHALMOL 24 237 980 7 LITT IF PEDIATR CLIN NORTH AM 27 3 980 8 KAUFFMAN RE PEDIATR PHARMACOL 1 231 981 9 PASSERO MA CLIN PEDIATR 20 264 981 10 GOYAN J WEST J MED 134 463 981 11 LITT IF AM J DIS CHILD 135 434 981 12 SLEATOR EK CLIN PEDIATR 21 474 982 13 MARKELLO JR PEDIATR INFECT DIS 4 579 985 14 WOOD PR ANN ALLERGY 54 400 985 15 FRIEDMAN IM PEDIATR CLIN NORTH AM 33 955 986 16 NOLAN T PEDIATRICS 77 229 986 PN 75019 RN 00185 AN 76059148 AU Lober-C-W. TI Letter: Nutritional supplementation in cystic fibrosis. SO Am-J-Dis-Child. 1975 Oct. 129(10). P 1239. MJ CYSTIC-FIBROSIS: dh. MN ADOLESCENCE. CHILD. HUMAN. EX Duke Medical Center and the National Institutes of Health have analyzed the maximum achieved heights and weights of 60 persons with CF over 20 years old. Both parameters were markedly skewed toward the lower percentile bands. No significant differences were found among patients whose condition was diagnosed early in life, in those with more severe pulmonary or gastrointestinal impairment, or among those in whom pancreatic enzyme and/or nutritional supplementation had been employed. Nutritional supplementation has yet to be proven a significant long-term influence on the morbidity or mortality of CF. - Lober reported data on 60 persons with cystic fibrosis (CF) over 20 years of age, while our population of 63 individuals contained only three who were over 15 years old. The median age of death in patients with CF is 12 years. It is not realistic to compare patients who have survived to adulthood with a younger group. He did not state the type of nutritional supplementation that was of no significance among his patients. Our experience with nutritional supplements other than the amino acid-glucose polymer mixture has been equally poor. Our data on growth rates were similar to those of Lober in that both heights and weights were skewed toward the lower end, and height was less affected than weight. Increased rates of linear growth were observed in eight of 13 children under 12 years of age at the time the dietary supplement was begun. Patients who continue to take the nutritional supplement have not shown further deterioration in pulmonary status. RF 001 BERRY HK AM J DIS CHILD 129 165 975 002 LOBER CW PROC AM COLL CHEST 40TH AN MT 974 003 VAUGHAN VC III IN: NELSON WE 15 969 004 MONTOYE HJ AM J CLIN NUTR 16 417 965 CT 1 YASSA JG ARCH DIS CHILD 53 777 978 2 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 PN 75020 RN 00186 AN 76059140 AU Perman-J. Breslow-L. Ingal-D. TI Nonoperative treatment of meconium ileus equivalent. SO Am-J-Dis-Child. 1975 Oct. 129(10). P 1210-1. MJ CYSTIC-FIBROSIS: co. FECES. INTESTINAL-OBSTRUCTION: et. MN ACETYLCYSTEINE. CASE-REPORT. CHILD. ENEMA. FEMALE. HUMAN. INTESTINAL-OBSTRUCTION: th. LIPASE: tu. MECONIUM. AB Intraluminal bowel obstruction secondary to inspissated feces is a known complication of cystic fibrosis. When seen in the older child, it is termed "meconium ileus equivalent." We studied a case in which nonsurgical resolution of the obstruction was obtained with N- acetylcysteine enemas and pancreatic replacement enzymes given orally and by enema. The pathogenesis of this disorder and the basis for the treatment are described. Recognition of this complication and familiarity with its medical management are important in caring for the older child with cystic fibrosis. RF 001 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 002 MULLINS F JAMA 192 741 965 003 JAFFE BF ARCH SURG 92 337 966 004 SHEFFNER AL ANN NY ACAD SCI 106 298 963 005 MEEKER IA SURGERY 56 419 964 006 SIMPSON TE MAYO CLIN PROC 43 725 968 007 SIGLER RM MAYO CLIN PROC 40 477 965 008 LILLIBRIDGE CB J PEDIATR 71 887 967 009 GRACEY M ARCH DIS CHILD 44 404 969 010 SHAW A J PEDIATR SURG 4 119 969 011 GRAND RJ CLIN PEDIATR 9 588 970 PN 75021 RN 00187 AN 76059130 AU Bardana-E-J-Jr. Sobti-K-L. Cianciulli-F-D. Noonan-M-J. TI Aspergillus antibody in patients with cystic fibrosis. SO Am-J-Dis-Child. 1975 Oct. 129(10). P 1164-7. MJ ANTIBODIES-FUNGAL: an. ASPERGILLUS-FUMIGATUS: im. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. ASPERGILLUS-FUMIGATUS: ip. BINDING-SITES-ANTIBODY. CHILD. CHILD-PRESCHOOL. CROSS-REACTIONS. HUMAN. IGE: an. INFANT. PRECIPITINS: an. RESPIRATORY-SYSTEM: mi. SUPPORT-U-S-GOVT-P-H-S. AB The respiratory flora of patients with cystic fibrosis (CF) frequently includes Aspergillus, and 30% of their serum samples have been observed to contain precipitating antibody to this fungus. Serum from 61 CF patients, 60 healthy persons, and three patients with CF and allergic bronchopulmonary aspergillosis was studied, using a quantitative assay for antibody to A fumigatus. Although CF patients had significantly higher levels of Aspergillus antibody, some antibody was found in all serum samples from normal individuals. Binding was immunologically specific for A fumigatus. Serum IgE levels and dermal reactivity to Aspergillus were similar in both CF and normal subjects. Increased levels of Aspergillus antibody in CF patients probably reflect pulmonary colonization, which only rarely causes infection or sensitization. RF 001 FEIGELSON J MOD PROBL PEDIATR 10 214 967 002 RIDDELL RW IN: LAWSON D PROC 5TH INT CF 189 969 003 HUGHES WT MYCOPATHOL MYCOL APPL 50 261 973 004 MEARNS MB LANCET 1 538 967 005 HALBERT SP MOD PROBL PEDIATR 10 144 967 006 SCHWARTZ RH AM J DIS CHILD 120 432 970 007 DOGGETT RG INFECT IMMUN 6 628 972 008 MEARNS MB THORAX 20 385 965 009 BATTEN JC MOD PROBL PEDIATR 10 227 967 010 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 011 BARDANA EJ JR J ALLERGY CLIN IMMUNOL 50 222 972 012 BARDANA EJ JR ANN NY ACAD SCI 221 64 974 013 BARDANA EJ JR J ALLERGY CLIN IMMUNOL 50 208 972 014 FARR RS J INFECT DIS 103 239 958 015 COOMBS RRA IN: GELL PGH 317 964 016 HEINER DC J ALLERGY 45 30 970 017 PATTERSON R AM J MED 54 16 973 018 BARDANA EJ JR AM REV RESPIR DIS 109 724 974 019 BAZARAL M J IMMUNOL 107 794 971 020 WALDMANN TA J IMMUNOL 109 304 972 021$ POLMAR SH J IMMUNOL 110 1253 970 022 YOUNG RC MEDICINE (BALTIMORE) 49 147 970 023 BOWMAN BH SCIENCE 164 325 969 024 BOXERBAUM B AM REV RESPIR DIS 108 777 973 025 CAUDILL M LANCET 2 307 973 026 MINDEN P INFECT IMMUN 6 574 972 027 MINDEN P J IMMUNOL 96 180 966 028 LINDBLAD JH J ALLERGY 32 392 961 029 SAFIRSTEIN BH AM REV RESPIR DIS 108 450 973 CT 1 BARDANA EJ AM REV RESPIR DIS 112 799 975 2 GALANT SP AM REV RESPIR DIS 114 325 976 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 4 OSVATH P MONATSSCHR KINDERHEILKD 125 220 977 5 SANDHU RS MYCOPATHOLOGIA 63 21 978 6 NELSON LA AM REV RESPIR DIS 120 863 979 7 TOBIN MJ THORAX 35 807 980 8 ANDERSON CJ J ALLERGY CLIN IMMUNOL 65 140 980 9 HARGIS JL AM J MED 68 389 980 10 MOSS RB J PEDIATR 99 215 981 11 PITCHERWILMOTT RW ARCH DIS CHILD 57 582 982 12 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 13 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 14 LAUFER P J ALLERGY CLIN IMMUNOL 73 44 984 15 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 PN 75022 RN 00188 AN 75162186 AU Pennington-J-E. Reynolds-H-Y. Wood-R-E. Robinson-R-A. Levine-A-S. TI Use of a Pseudomonas Aeruginosa vaccine in pateints with acute leukemia and cystic fibrosis. SO Am-J-Med. 1975 May. 58(5). P 629-36. MJ BACTERIAL-VACCINES. LEUKEMIA: im. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: pc. MN ADOLESCENCE. ADULT. BACTERIAL-VACCINES: ad, ae. CHILD. CLINICAL-TRIALS. CYSTIC-FIBROSIS: im. DRUG-ADMINISTRATION-SCHEDULE. DRUG-EVALUATION. FEMALE. HUMAN. INJECTIONS-INTRAMUSCULAR. MALE. MIDDLE-AGE. AB A heptavalent lipopolysaccharide Pseudomonas vaccine was evaluated in 22 patients with acute leukemia and 12 patients with cystic fibrosis during an 18 month interval at the Clinical Center of the National Institutes of Health. Of the 34 patients, 32 had an excellent serum hemagglutinating (HA) antibody response to immunization. In comparison to the patients with cystic fibrosis, the patients with leukemia had a smaller HA antibody response, which lasted a shorter period of time, and also}i experienced greater toxicity from the vaccine. The mixing of adrenal corticosteroids with vaccine greatly decreased side reactions among the patients with leukemia without significantly inhibiting antibody production. Previous antineoplastic chemotherapy had little influence on antibody response in patients with leukemia, with the exception of methortrexate. Vaccinated patients with leukemia had 1 Pseudomonas infection of 14 bacterial or fungal infections, whereas 2 pseudomonas infections of 5 bacterial or fungal infections occurred in a control group of 20 patients with acute leukemia. Of the 12 patients with cystic fibrosis, 4 had a Pseudomonas infection after vaccination. RF 001 MARKLEY K ANN SURG 145 175 957 002 RABIN ER N ENGL J MED 265 1225 961 003 DI SANTAGNESE PA N ENGL J MED 277 1399 967 004 ROSE HD AM REV RESPIR DIS 107 416 973 005 TILLOTSON JR ANN INTERN MED 68 295 968 006 LEVINE AS SEMIN HEMATOL 9 141 972 007 LEVINE AS SEMIN HEMATOL 11 141 974 008 SCHIMPFF SC ANN INTERN MED 77 707 972 009 PENNINGTON JE AM J MED 55 155 973 010 REYES MP MEDICINE (BALTIMORE) 52 173 973 011 MARKLEY K ANN INTERN MED 74 140 971 012 HANESSIAN S NATURE NEW BIOL 229 209 971 013 FISHER MW J BACTERIOL 98 835 969 014 ALEXANDER JW J TRAUMA 10 565 970 015 YOUNG LS ANN INTERN MED 79 518 973 016 HAGHBIN M CANCER 32 761 973 017 WOLFF SM J INFECT DIS 128 243 973 018 YOUNG LS J INFECT DIS 126 257 972 019 BJORNSON AB INFECT IMMUN 2 453 970 020 YOUNG LS INFECT IMMUN 2 495 970 021 SANTOS GW FED PROC 26 907 967 022 LEVENTHAL BG ISR J MED SCI 10 866 974 023 GUMP DW ARCH INTERN MED 132 847 973 024 BODEY GP AM J MED SCI 260 82 970 025 DALE DC J CLIN INVEST 54 664 974 026 UFFELMAN JA CLIN CHIM ACTA 28 185 970 CT 1 ANON LANCET 2 168 975 2 LEVINE AS CANCER 36 813 975 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 DISANTAGNESE PA N ENGL J MED 295 597 976 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 6 PENNINGTON JE CANCER 39 1345 977 7 KAZMIEROWSKI JA J INFECT DIS 135 438 977 8 HORTOBAGYI GN CANCER IMMUNOL IMMUNOTHER 4 201 978 9 MOODY MR INFECT IMMUN 21 905 978 10 GEDDES AM S AFR MED J 53 121 978 11 BERGERON MG SCAND J INFECT DIS 1978 189 978 12 PENNINGTON JE INFECT IMMUN 25 1029 979 13 POLLACK M J CLIN INVEST 63 276 979 14 WOOD RE SOUTH MED J 72 189 979 15 PENNINGTON JE J INFECT DIS 140 73 979 16 NETER E ZENTRALBL BAKT PARA INFEK HYG 243 349 979 17 LEPRAT R ANN MICROBIOL (PARIS) B131 209 980 18 PASKO M J MED 11 303 980 19 FICK RB J IMMUNOL METH 38 103 980 20 RAMSEY PG MEDICINE 59 206 980 21 BALTIMORE RS J INFECT DIS 141 238 980 22 PENNINGTON JE J INFECT DIS 142 191 980 23 FICK RB J CLIN INVEST 68 899 981 24 PENNINGTON JE J CLIN INVEST 68 1140 981 25 PIZZO PA J PEDIATR 98 524 981 26 SEID RC J BIOL CHEM 256 7305 981 27 BODEY GP SEM HEMATOL 19 193 982 28 OHMAN DE INFECT IMMUN 37 662 982 29 HOOKE AM INFECT IMMUN 38 136 982 30 PIER GB J CLIN INVEST 69 303 982 31 PIER GB J INFECT DIS 145 217 982 32 PINCHING AJ CLIN IMMUNOL ALLERGY 3 305 983 33 BODEY GP REV INFECT DIS 5 279 983 34 PENNINGTON JE REV INFECT DIS 5 S852 983 35 PIER GB REV INFECT DIS 5 S950 983 36 TEGTMEIER BR REV INFECT DIS 5 S963 983 37 BRYAN LE J CLIN MICROBIOL 18 276 983 38 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 39 CRYZ SJ INFECT IMMUN 39 1067 983 40 KLINGER JD INFECT IMMUN 39 1377 983 41 CRYZ SJ INFECT IMMUN 40 659 983 42 PIZZO PA CANCER TREAT REP 67 223 983 43 MORRISON AJ REV INFECT DIS 6 S627 984 44 YOUNG LS REV INFECT DIS 6 S769 984 45 SPEERT DP PEDIATR RES 18 431 984 46 CRYZ SJ INFECT IMMUN 44 508 984 47 TSAY GC INFECT IMMUN 45 217 984 48 GONGGRIJP R ANT V LEEUW J MICROBIOL 50 285 984 49 FICK RB J CLIN INVEST 74 236 984 50 YOUNG LS AM J MED 76 664 984 51 WHEELER WB J PEDIATR 104 695 984 52 OLSON B J INFECT DIS 150 808 984 53 GOULD IM LANCET 2 1224 985 54 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 55 MISCHLER EH SEM RESPIR MED 6 271 985 56 SHRYOCK TR CURRENT MICROBIOL 12 91 985 57 BANNISTER P J BIOL STANDARD 13 321 985 58 PODNOS SD WEST J MED 143 622 985 59 OLSON B J INFECT DIS 152 769 985 60 PENNINGTON JE REV INFECT DIS 8 S426 986 61 CLASENER HAL REV INFECT DIS 9 295 987 PN 75023 RN 00189 AN 76085352 AU Burnette-B-A. TI Family adjustment to cystic fibrosis. SO Am-J-Nurs. 1975 Nov. 75(11). P 1986-9. MJ ADAPTATION-PSYCHOLOGICAL. CYSTIC-FIBROSIS. FAMILY. MN CYSTIC-FIBROSIS: di. FEMALE. HUMAN. INFANT. LUNG-DISEASES: pc. SOCIAL-ADJUSTMENT. EX A nurse and mother describes the clinical effects of cystic fibrosis on patients and the long-term demands the disease places on her own and other children and families. Diagnosis is mainly accomplished with an analysis of sweat for sodium and chloride ions. CF is transmitted as a Mendelian recessive trait. It is a generalized dysfunction of the endocrine glands. The abnormalities, seemingly unrelated, appear as loss of electrolytes through the sweat and salivary glands and as unusually thick, sticky mucus secretions that obstruct and damage the patient's pancreas, lungs, gallbladder, intestines, and liver. The objective of pulmonary therapy are to decrease viscosity of the pulmonary secretions and to improve and maintain pulmonary hygiene. Financial cost is only one of many burdens on the family. RF 001 JEANS PC ESSENTIALS OF PEDIATRICS 714 954 002 DI SANTAGNESE PA TODAY'S HEALTH 47 38 969 003 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 PN 75024 RN 00190 AN 76016602 AU Changus-J-E. Quissell-D-O. Sukup-M-R. Pitot-H-C. TI Studies on the synthesis of plasma membrane proteins of fibroblasts from patients with cystic fibrosis. SO Am-J-Pathol. 1975 Aug. 80(2). P 317-28. MJ CELL-MEMBRANE: me. CYSTIC-FIBROSIS: me. PROTEINS: bi. MN BIOPSY. CARBON-RADIOISOTOPES. CELLS-CULTURED. CYSTIC-FIBROSIS: pa, fg. DENSITOMETRY. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FIBROBLASTS: me, ul. GENOTYPE. HUMAN. IN-VITRO. LEUCINE: me. SKIN: pa. TRITIUM. SUPPORT-U-S-GOVT-P-H-S. AB The characteristic increased salinity of sweat and other abnormalities of exocrine secretions in patients with cystic fibrosis (CF) suggest the possibility of a disturbed functioning of the plasma membrane in this disease. Several lines of evidence indicate that fibroblasts express the presence of the CF genotype. Therefore these cells were used in an in vitro study directed at determining whether the manifestations of CF might be related to an alteration of one or more of the protein components of the plasma membrane. In order to evaluate the synthesis of these components, growing fibrosblasts from patients with CF and normal subjects were briefly exposed to either 14C- or 3-H-leucine. Their plasma membranes were then isolated and subjected to analysis in a nondetergent acrylamide gel system. Coelectrophoresis of differentially labeled preparations revealed the absence of a detectable abnormality in the synthetic rates of any of the more than 30 resolved protein species. RF 001 FARBER S ARCH PATHOL 37 238 944 002 GIBSON LE PEDIATRICS 23 545 959 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 MCCOMBS ML TEX REP BIOL MED 31 615 973 005 POTTER JL ANN NY ACAD SCI 106 692 963 006 MANDEL ID AM J DIS CHILD 113 431 967 007 DISCHE Z PEDIATRICS 24 74 959 008 ROELFS RE AM J DIS CHILD 113 419 967 009 LOBECK CC IN: STANBURY JB 1605 972 010 BALFE JW SCIENCE 162 689 968 011 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 012 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 013 FITZPATRICK DF NATURE NEW BIOL 235 173 972 014 FEIG SA PEDIATR RES 8 594 974 015 DANES BS LANCET 1 1061 968 016 MATALON R LANCET 2 838 969 017 REED GB J PATHOL 101 251 970 018 DANES BS J EXP MED 137 1538 973 019 BERATIS NG PEDIATR RES 7 958 973 020 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 021 DANES BS J EXP MED 129 775 969 022 PALLAVICINI JC J PEDIATR 77 280 970 023 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 024 FLETCHER DS CLIN CHIM ACTA 44 5 973 025 BAIG MM J PEDIATR 86 72 975 027 BARLAND P J CELL BIOL 45 662 970 028 TERMAN SA PROC NAT ACAD SCI USA 65 985 970 029 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 030 DANES BS LANCET 2 765 973 031 ROBERTS RM BIOCHEMISTRY 13 4846 974 032 WARREN L J CELL BIOL 37 729 968 033 BERGSMA D BIRTH DEF ORIG ART SER 9 11 973 034 SHAW CR SCIENCE 149 936 965 035 CAPECCHI MR PROC NAT ACAD SCI USA 71 4732 974 036 DERN RJ J LAB CLIN MED 68 560 966 037 PITOT HC PROC SYMP MOLE BASIS HUM 14 972 038 BENKE PJ LANCET 1 182 972 039 MARSDEN JC BIOCHEM SOC TRANS 2 326 974 040 QUISSELL DO NATURE 247 115 974 CT 1 WARD JB TEX REP BIOL MED 34 11 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 SANTAGNESE PAD N ENGL J MED 295 481 976 4 YOKOYAMA M PEDIATR RES 11 765 977 5 QUISSELL DO ANAL BIOCHEM 79 240 977 6 OWEN E J MOL MED 3 203 978 7 PENA SDJ PEDIATR RES 12 887 978 8 PEARSON RD PEDIATR RES 13 834 979 9 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 10 HARRIS A CLIN CHIM ACTA 128 41 983 11 KARLSSON S J MED GENET 21 441 984 PN 75025 RN 00191 AN 75089105 AU Lieberman-J. TI Carboxypeptidase B-like activity and C3 in cystic fibrosis. SO Am-Rev-Respir-Dis. 1975 Jan. 111(1). P 100-2. MJ CARBOXYPEPTIDASES: bl. COMPLEMENT. CYSTIC-FIBROSIS: di. MN COMPLEMENT: an. CYSTIC-FIBROSIS: im, en. FEMALE. GEL-DIFFUSION-TESTS. HUMAN. MALE. SUPPORT-U-S-GOVT-NON-P-H-S. AB Assays of carboxypeptidase B-like activity and C3 in serum from patients with cystic fibrosis and appropriate control subjects failed to demonstrate a deficiency of carboxypeptidase B-like activity or a consistent increase in C3, as was suggested by Conover and associates. Differences between men and women with cystic fibrosis were apparent, in that women with cystic fibrosis had higher concentrations of both serum carboxypeptidase B-like activity and C3 than either men with cystic fibrosis or control subjects. RF 001 CONOVER JH LANCET 2 1501 973 002 CONOVER JH LIFE SCI 14 253 974 003 CUSHMAN DW BIOCHEM PHARMACOL 20 1637 971 004 NEURATH H IN: BOYER PD 4 11 960 005$ FOLK JE METHODS ENZYMOL 504 970 006 ERDOS EG BIOCHEM PHARMACOL 16 1287 967 CT 1 ALTLAND K HUM GENET 28 207 975 2 HARPER BL TEX REP BIOL MED 34 73 976 3 CORBIN NC ANAL BIOCHEM 73 41 976 4 ANON J PEDIATR 88 711 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 DISANTAGNESE PA N ENGL J MED 295 534 976 7 MARCHI AG HELV PAEDIATR ACTA 33 517 978 8 GOTZ M EUR J PEDIATR 127 133 978 9 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 10 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 11 BUTTERWORTH J CLIN CHIM ACTA 97 39 979 12 HODSON ME THORAX 35 801 980 13 PLUMMER TH ANAL BIOCHEM 108 348 980 14 MOSS RB AM REV RESPIR DIS 121 23 980 15 MCCORMICK JR AM J PATHOL 109 283 982 16 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 17 SCHWEISFURTH H DTSCH MED WSCHR 109 1254 984 18 CICARDI MGM INT J ARTIF ORGANS 8 135 985 19 MATHEWS KP PROG ALLERGY 39 344 986 20 BOGART BI J CHROMATOGR 381 29 986 PN 75026 RN 00192 AN 76086198 AU Corbet-A. Ross-J. Popkin-J. Beaudry-P. TI Relationship of arterial-alveolar nitrogen tension to alveolar-arterial oxygen tension, lung volume, flow measurements, and diffusing capacity in cystic fibrosis. SO Am-Rev-Respir-Dis. 1975 Oct. 112(4). P 513-9. MJ CYSTIC-FIBROSIS: pp. NITROGEN. OXYGEN. RESPIRATION. MN ADOLESCENCE. CARBON-DIOXIDE. CARBON-MONOXIDE. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. LUNG-VOLUME-MEASUREMENTS. MALE. NITROGEN: bl. OXYGEN: bl. PULMONARY-ALVEOLI. PULMONARY-ARTERY. PULMONARY-DIFFUSING-CAPACITY. VENTILATION-PERFUSION-RATIO. AB Pulmonary function in children with cystic fibrosis was assessed by the arterial-alveolar PN2 difference adjusted to sublingual temperature. The resulting values were compared with the alveolar- arterial PO2 difference, arterial PCO2, and standard measurements of lung volume, flow, and diffusing capacity. The arterial-alveolar PN2 difference was nearly one half of the PO2 difference, both early in the disease and at a more advanced stage. Analysis taking into account the O2 dissociation curve and the possibility that alveolar temperature is higher than sublingual temperature suggested that all of the PO2 difference could be explained in terms of ventilation- perfusion imbalance in gas-filled units of the lung. Reduction of fractional CO uptake with increasing PN2 difference suggested that the decrease in diffusing capacity in cystic fibrosis may be explained by ventilation-perfusion inequality. A significant relationship between arterial PCO2 and the PN2 difference supported the view that ventilation-perfusion inequality is the cause of CO2 retention when present. The PN2 and PO2 differences were abnormal before the standard tests of lung volume and flow, but in general, the correlation was excellent. Because the PN2 difference was not superior to the PO2 difference in detecting early disease, and because the technical problems in its measurement are considerable, it is not recommended as a routine measurement. RF 001 MELLINS RB PEDIATRICS 44 315 969 002 LANDAU LI AM REV RESPIR DIS 108 593 973 003 RILEY RL J APPL PHYSIOL 4 77 951 004 CANFIELD RE J APPL PHYSIOL 10 165 957 005 KABE J CAN J PHYSIOL PHARMACOL 46 795 968 006 FARHI LE J APPL PHYSIOL 18 97 963 007 KLOCKE FJ MEASURE TRACE AMOUNTS INERT G 75 967 008 CORBET A J APPL PHYSIOL 36 74 974 009 WINQUIST RA J PEDIATR 76 455 970 010 RAHN H GRAPH ANALYSIS RESPIR GAS EXC 955 011 BERNSTEIN L THORAX 7 255 952 012 LEUALLEN EC AM REV TUBERC PULM DIS 72 783 955 013 MENEELY GR J CLIN INVEST 28 129 949 014 BATES DV RESPIRATORY FUNCTION IN DISEA 971 015 BATES DV J PHYSIOL (LOND) 129 237 955 016 BEAUDRY PH IN: POLGAR G 87 971 017 ARMITAGE P STATISTICAL METHODS IN MEDICA 971 018 FEATHERBY EA AM REV RESPIR DIS 102 737 970 019 WARING WW AM REV RESPIR DIS 91 77 965 020 ABERNATHY JD PHYSIOLOGIST 9 128 966 021 CORBET A CAN J PHYSIOL PHARMACOL 52 63 975 022 LAMARRE A PEDIATRICS 50 291 972 023 DEMUTH GR AM J DIS CHILD 103 129 962 024 MARKELLO R ANESTHESIOLOGY 37 4 972 025 LENFANT C J APPL PHYSIOL 18 1090 963 026 EDWARDS AWT J APPL PHYSIOL 18 107 963 027 SEVERINGHAUS JW IN: FENN WO 2 1475 965 028 MOSS AJ PEDIATRICS 41 438 968 029 WAGNER PD J APPL PHYSIOL 33 62 972 030 WEST JB RESPIR PHYSIOL 7 88 969 031 MACKLEM PT J APPL PHYSIOL 22 395 967 032 MEAD J J APPL PHYSIOL 22 95 967 033 FOX WW PEDIATRICS 54 293 974 CT 1 MILLIS RM CHEST 71 508 977 2 MILLIS RM CHEST 72 217 977 PN 75027 RN 00193 AN 75163031 AU Stowe-S-M. Boat-T-F. Mendelsohn-H. Stern-R-C. Tucker-A-S. Doershuk-C-F. Matthews-L-W. TI Open thoracotomy for pneumothorax in cystic fibrosis. SO Am-Rev-Respir-Dis. 1975 May. 111(5). P 611-7. MJ CYSTIC-FIBROSIS: co. PLEURA: su. PNEUMOTHORAX: su. MN CYSTIC-FIBROSIS: mo. EVALUATION-STUDIES. HUMAN. LENGTH-OF-STAY. PNEUMOTHORAX: et, dt, pp, mo. POSTOPERATIVE-COMPLICATIONS. RECURRENCE. SCLEROSING-SOLUTIONS: tu. SUPPORT-U-S-GOVT-P-H-S. VITAL-CAPACITY. AB The results of open thoractomy and pleurectomy or pleural abrasion for 17 episodes of pneumothorax in patients with cystic fibrosis were compared with the results of observation, closed thoracostomy, and closed thoracostomy with sclerosing agents. Open thoracotomy had the advantage of insuring prompt resolution of the pneumothorax. A small incision minimized postoperative morbidity. The average hospital stay was 15 days after open thoracotomy and 19 days after closed thoracostomy, with or without sclerosing agents. Complications of all treatments were infrequent. A limited symptomatic recurrence was observed in 2 of 17 pneumothoraces successfully treated with open thoracotomy. Recurrence also occurred in 1 of 8 treated with observation, 3 of 9 treated with closed thoracostomy, and 1 of 14 treated with closed thoracostomy and sclerosing agents. A loss of vital capacity was usually noted 4 to 18 months after resolution, regardless of treatment. Forty per cent of patients in both operative and nonoperative groups survived 3 years. In our experience, open thoracotomy can be used safely for treatment of unresolved or recurrent pneumothorax and warrants further evaluation as a primary therapy for pneumothorax in patients with cystic fibrosis. RF 001 LIFSCHITZ MI AM J DIS CHILD 116 633 968 002 BOAT TF JAMA 209 1498 969 003 MITCHELL-HEGGS PF THORAX 25 165 970 004 HOLSCLAW DS CLIN PEDIATR 9 346 970 005 SMITH WG THORAX 17 342 962 006 ALDER RH ANN THORAC SURG 5 474 968 007 ANDERSEN I DIS CHEST 54 230 968 008 CATTANEO SM J THORAC CARDIOVASC SURG 66 467 973 009 KATTWINKEL J JAMA 226 557 973 010 ANON BR MED J 1 720 968 011 HORNE NW BR MED J 1 281 966 012 ANON AM REV RESPIR DIS 88 275 963 013 ROUSDELL HT JR ARCH SURG 87 161 963 014 YOUMANS CR AM J SURG 120 644 970 015 SEREMETIS MG CHEST 57 65 970 016 COLLINS HA AM SURGEON 29 844 963 017 INOUYE WY DIS CHEST 51 67 967 018 HICKOK DF SURG GYNECOL OBSTET 120 499 965 019 THOMAS PA J THORAC CARDIOVASC SURG 39 194 960 020 GOBBEL WG J THORAC CARDIOVASC SURG 46 331 963 021 CLARK TA AM J SURG 124 728 972 022 MATTHEWS LW J PEDIATR 65 558 964 023 MATTHEWS LW PEDIATRICS 39 176 967 024 BERNHARD WF DIS CHEST 42 403 962 025 KLASSEN KP JAMA 182 111 962 CT 1 STERN RC J PEDIATR 89 406 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 LUCK SR J THORAC CARDIOVASC SURG 74 834 977 4 RICH RH J PEDIATR SURG 13 237 978 5 SCHEELE J CHIRURG 49 236 978 6 FINK RJ CHEST 74 643 978 7 LARRIEU AJ ANN THORAC SURG 28 146 979 8 WOOD RE SOUTH MED J 72 189 979 9 STERN RC AM J DIS CHILD 134 267 980 10 MCLOUD TC RADIOLOGY 135 313 980 11 GELFAND ET CAN J SURG 24 458 981 12 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 13 KISTLER I HELV PAEDIATR ACTA 36 495 982 14 PENKETH ARL THORAX 37 850 982 15 MCLAUGHLIN FJ J PEDIATR 100 863 982 16 SCHUSTER SR J PEDIATR SURG 18 492 983 17 WEEDEN D THORAX 38 737 983 18 DAVIS PB CHEST 85 802 984 19 SEGERER H KLIN PAEDIATR 196 44 984 20 LESTER LA SEM RESPIR MED 6 285 985 21 TOMASHEFSKI JF HUM PATHOL 16 253 985 22 FERNALD GW SEM ROENTGENOL 22 87 987 PN 75028 RN 00194 AN 75181879 AU Landau-L-I. Taussig-L-M. Macklem-P-T. Beaudry-P-H. TI Contribution of inhomogeneity of lung units to the maximal expiratory flow-volume curve in children with asthma and cystic fibrosis. SO Am-Rev-Respir-Dis. 1975 Jun. 111(6). P 725-31. MJ ASTHMA: pp. CYSTIC-FIBROSIS: pp. RESPIRATION. MN ADOLESCENCE. ADULT. AGE-FACTORS. ANTHROPOMETRY. CHILD. HUMAN. MAXIMAL-EXPIRATORY-FLOW-VOLUME-CURVES. MAXIMAL-MIDEXPIRATORY-FLOW-RATE. VITAL-CAPACITY. AB Normal children as well as those with asthma and cystic fibrosis were studied to assess the contribution of lung zones emptying at different rates to the curvilinearity of the maximal expiratory flow- volume curve. Lung volumes, maximal expiratory flow-volume curves breathing air and then breathing a helium-oxygen mixture, and single- breath nitrogen washouts were measured. Forced expiratory maneuvers from lung volumes near functional residual capacity were performed to produce transients of flow exceeding maximal flow defined by the full flow-volume curve. Normal children and those with asthma and mild cystic fibrosis had small or no transients. Those with severe cystic fibrosis had large transients as well as increased phase I on the nitrogen washout curves. These large transients were associated with increased curvilinearity of the maximal expiratory flow-volume curve and smaller than normal flow response breathing helium. In severe cystic fibrosis, the large transients suggest sequential emptying of fast and slow spaces, which influences the shape of the maximal expiratory flow-volume curve. The fast space could be due to compression of an enlarged anatomic dead space. Any time constant inequality between parenchymal units present in asthma and mild cystic fibrosis does not appear to contribute significantly to the shape of the maximal expiratory flow-volume curve. RF 001 FRY DL AM J MED 16 80 954 002 HYATT RE J APPL PHYSIOL 13 331 958 003 FRY DL AM J MED 29 672 960 004 MEAD J J APPL PHYSIOL 22 95 967 005 MACKLEM PT J APPL PHYSIOL 25 159 968 006 TAKISHIMA TG SCAND J RESPIR DIS 48 384 967 007 DUBOIS AB J CLIN INVEST 35 322 956 008 MEAD J J APPL PHYSIOL 15 736 960 009 WENG TR AM REV RESPIR DIS 99 879 969 010 BEAUDRY PH AM REV RESPIR DIS 95 248 967 011 POLGAR G PULMONARY FUNCTION TESTING IN 971 012 ZAPLETAL A J APPL PHYSIOL 26 308 969 013 DESPAS PJ J CLIN INVEST 51 3235 972 014 FOX WW PEDIATRICS 54 293 974 015 ANTHONISEN NR RESPIR PHYSIOL 8 58 969 016 KNUDSON RJ J APPL PHYSIOL 36 653 974 017 ZUELZER WW PEDIATRICS 4 53 949 018 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 019 FRY DL COMPUT BIOMED RES 2 111 968 020 PRIDE NB J APPL PHYSIOL 23 646 967 021 HILL DJ AM REV RESPIR DIS 106 873 972 022 LANDAU LI AM REV RESPIR DIS 108 593 973 023 LANDAU LI AUST NZ J MED 3 557 973 024 MACKLEM PT PHYSIOL REV 51 368 971 025 FRASER RG BRONCHIAL DYNAMICS HEALTH OBS 76 970 026 FOWLER WS AM J PHYSIOL 154 405 948 027 ZAPLETAL A PEDIATRICS 48 64 971 028 MELLINS RB PEDIATRICS 41 560 968 029 MANSELL A AM REV RESPIR DIS 109 190 974 CT 1 ANCIC P REV MED CHIL 104 513 976 2 CERECEDA J REV MED CHIL 104 519 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 LIGAS JR J APPL PHYSIOL 42 607 977 5 PROCTOR DF AM REV RESPIR DIS 115 97 977 6 WEBSTER PM AM REV RESPIR DIS 115 805 977 7 KEENS TG AM REV RESPIR DIS 116 853 977 8 KRAEMER R HELV PAEDIATR ACTA 34 417 979 9 MELISSINOS CG J APPL PHYSIOL 47 1043 979 10 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 11 KATTAN M THORAX 35 531 980 12 JANSEN JM AM REV RESPIR DIS 122 221 980 13 MARAZZINI L RESPIRATION 42 88 981 14 HOLZER FJ ARCH DIS CHILD 56 455 981 15 LOUGHLIN GM CHEST 79 206 981 16 PESLIN R BULL EUR PHYSIOPATH RESP 18 679 982 17 LEMEN RJ J APPL PHYSIOL 53 977 982 18 REDDING GJ AM REV RESPIR DIS 126 31 982 19 VANPELT W BULL EUR PHYSIOPATH RESP 19 P83 983 20 TECULESCU DB BULL EUR PHYSIOPATH RESP 21 193 985 21 VANPELT W METH INF MED 24 91 985 22 PEDERSEN OF J APPL PHYSIOL 58 1305 985 23 PYSZCZYNSKI D J APPL PHYSIOL 59 28 985 24 ZACH MS AM REV RESPIR DIS 131 537 985 25 DARGA LL PEDIATR PULMONOL 2 82 986 26 OBERWALDNER B PEDIATR PULMONOL 2 358 986 27 ODONNELL CR J APPL PHYSIOL 61 2243 986 PN 75029 RN 00195 AN 75181880 AU Wood-R-E. Wanner-A. Hirsch-J. Farrell-P-M. TI Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. SO Am-Rev-Respir-Dis. 1975 Jun. 111(6). P 733-8. MJ CYSTIC-FIBROSIS: pp. MUCUS. TERBUTALINE: pd. TRACHEA: pp. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: dt. BRONCHOSCOPY. CILIA: de. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. MALE. MUCOUS-MEMBRANE: pp, de. STIMULATION-CHEMICAL. TERBUTALINE: tu. TRACHEA: de. AB Tracheal mucous velocity was measured by observing the motion of teflon discs across the tracheal mucosa through a fiberoptic bronchoscope. The average rate of movement in 14 adult patients with cystic fibrosis was 2.6 mm per min plus or minus 3.3 SD, compared with 20.1 mm per min plus or minus 6.3 in 20 normal subjects of the same age (P less than 0.001). This failure of mucociliary transport may play a role in the pathogenesis of the pulmonary disease in cystic fibrosis. Administration of a beta-adrenergic agent, terbutaline, increased the average mucous velocity in the patients with cystic fibrosis (to 5.5 mm per min plus or minus 3.6 SD, P less than 0.001) but not in control subjects. This observation has potential therapeutic significance. RF 001 SANCHIS J N ENGL J MED 288 651 973 002 NEWHOUSE M IN: MANGOS JA 319 973 003 SACKNER MA J APPL PHYSIOL 34 495 973 004 TAUSSIG LM J PEDIATR 82 380 973 005 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 006 SACKNER MA ANN INTERN MED 82 40 975 007 WARING WW N ENGL J MED 288 681 973 008 MORROW PE IN: BOUHUYS A 299 970 009 CAMNER P SCAND J RESPIR DIS 54 272 973 010 THOMSON ML N ENGL J MED 289 749 973 011 ASMUNDSSON T AM REV RESPIR DIS 102 388 970 012 HILDING AC AM J PHYSIOL 191 404 957 013 ADLER KB ARCH ENVIRON HEALTH 27 364 973 015$ EWERT G ANN OTOLARYNGOL 76 359 967 016 CHARMAN J BIORHEOLOGY 9 185 972 017 MAGID SL ARCH OTOLARYNGOL 86 212 967 018 SPOCK A PEDIATR RES 1 173 967 019 BOWMAN BH SCIENCE 164 325 969 020 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 021 SPENCER H PATHOLOGY OF THE LUNG 968 022 HILDING AC MED THORAC 22 329 965 023 LOURENCO RV J CLIN INVEST 50 1411 971 024 HINDS L ANESTHESIOLOGY 34 445 971 025 LEEGAARD J ARCH DIS CHILD 48 229 973 026 LAURENZI GA N ENGL J MED 279 333 968 027 ASMUNDSSON T IN: DULFANO MJ 107 973 028 BOYD EM RESPIRATORY TRACT FLUID 972 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 BOWMAN BH TEX REP BIOL MED 34 1 976 3 DULFANO MJ ANN ALLERGY 37 357 976 4 MOSSBERG B SCAND J RESPIR DIS 57 119 976 5 SACKNER MA CHEST 69 593 976 6 ANON J PEDIATR 88 711 976 7 DISANTAGNESE PA N ENGL J MED 295 597 976 8 CLARKE CW CLIN ALLERGY 7 527 977 9 SORENSEN RU INFECT IMMUN 18 735 977 10 GEUMEI AM CHEST 72 267 977 11 ROSSMAN CM J PEDIATR 90 579 977 12 FRIEDMAN M AM REV RESPIR DIS 115 67 977 13 SHIH CK AM REV RESPIR DIS 115 989 977 14 KNIGHT RK PRACTITIONER 218 286 977 15 GUERRIN F LILLE MED 23 368 978 16 MARIN MG J APPL PHYSIOL 44 900 978 17 KOLLBERG H SCAND J RESPIR DIS 59 297 978 18 SACKNER MA CHEST 73 958 978 19 GIORDANO A ANN OTOL RHINOL LARYNGOL 87 484 978 20 SACKNER MA ANN OTOL RHINOL LARYNGOL 87 474 978 21 WILSON GB J LAB CLIN MED 92 463 978 22 GOTZ M EUR J PEDIATR 127 133 978 23 NAGY EC PEDIATR RES 13 729 979 24 SACKNER MA BULL EUR PHYSIOPATH RESP 15 505 979 25 AFZELIUS BA INT REV EXP PATHOL 19 1 979 26 STURGESS JM PEDIATR CLIN NORTH AM 26 481 979 27 MOSSBERG B ACTA PHARMACOL TOXICOL 44 41 979 28 WANNER A AM J MED 67 477 979 29 WOOD RE SOUTH MED J 72 189 979 30 DAVIS B AM REV RESPIR DIS 120 547 979 31 CLARKE SW BR J CLIN PHARMACOL 9 537 980 32 PAVIA D CLIN RESP PHYSIOL 16 335 980 33 AFZELIUS BA THORAX 35 401 980 34 SVENONIUS E RESPIRATION 40 226 980 35 WELLER PH RESPIRATION 40 53 980 36 CAMNER P CLIN SCI 59 79 980 37 MOSSBERG B EUR J RESPIR DIS 61 51 980 38 DAVIS PB J LAB CLIN MED 96 75 980 39 SORENSEN RU PEDIATR RES 15 14 981 40 BATEMAN JRM THORAX 36 683 981 41 RUTLAND J THORAX 36 654 981 42 SADOUL P CHEST 80 885 981 43 SORENSEN RU AM REV RESPIR DIS 123 37 981 44 ANON LANCET 1 203 982 45 SCHLESINGER RB BIOSCIENCE 32 45 982 46 KANG SB ANESTH ANALG CLEVE 61 793 982 47 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 48 BATEMAN JRM BR J CLIN PHARMACOL 15 695 983 49 DAVIS PB J CHRON DIS 36 269 983 50 PAVIA D EUR J RESPIR DIS 64 304 983 51 DAVIS PB J PEDIATR 102 177 983 52 GAIL DB AM REV RESPIR DIS 127 366 983 53 RUTLAND J AM REV RESPIR DIS 128 1030 983 54 BOUCHER RC LUNG 161 1 983 55 OHASHI Y ACTA OTOLARYNGOL STOCKH SUPPL 397 49 983 56 BRIDGES MA ANN NY ACAD SCI 421 360 983 57 KATZ S CELL CALC 5 421 984 58 CAMNER P ATEMWEGS LUNGENKRANKH 10 237 984 59 WEISS T ATEMWEGS LUNGENKRANKH 10 383 984 60 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 61 REDDING GJ PEDIATR CLIN NORTH AM 31 891 984 62 STUART BO ENVIRON HEALTH PERSPECT 55 369 984 63 LURIE A REV MALAD RESPIR 2 117 985 64 DAVIS PB SEM RESPIR MED 6 261 985 65 MISCHLER EH SEM RESPIR MED 6 271 985 66 REYNOLDS HY DISEASE A MONTH 31 1 985 67 RONCORONI AJ MED (BUENOS AIRES) 45 183 985 68 GREENSTONE M BR J DIS CHEST 79 9 985 69 SOSULSKI R PEDIATR PULMONOL 2 269 986 70 VITTING C J VET MED (A) 33 706 986 71 WILSON R THORAX 41 453 986 72 VERBOON JML EUR J RESPIR DIS 69 169 986 73 SYKES DA THORAX 42 256 987 PN 75030 RN 00196 AN 76059953 AU Rossman-C-M. Dolovich-M. Dolovich-J. Wilson-W. Newhouse-M. TI Mucociliary clearance. SO Am-Rev-Respir-Dis. 1975 Nov. 112(5). P 744. MJ CYSTIC-FIBROSIS: pp. NASAL-MUCOSA: pp. MN COUGH. HUMAN. EX Mucociliary clearance data obtained by our group in relatively healthy cystic fibrosis subjects was not affected by cough. The majority of subjects mentioned in the original article did not cough during the study and indeed were chosen because they were relatively healthy at the time. It was possible to study them over fairly long periods without interference from cough and thus the relatively normal clearance times demonstrated were presumably due to mucociliary transport. Reference was made to nasal mucociliary clearance studies in patients with cystic fibrosis and normal subjects which indicated that clearance was similar in both groups until mild inflammation of the nasal mucosa was produced. We would like to point out that the work was performed in our Laboratory, since no reference was given in the discussion. RF 001 SANCHIS J N ENGL J MED 288 651 973 002 ROSSMAN CM PEDIATR RES 8 469 974 PN 75031 RN 00197 AN 75223179 AU Brown-B-R-Jr. Walson-P-D. Taussig-L-M. TI Congenital metabolic diseases of pediatric patients: anesthetic implications. SO Anesthesiology. 1975 Aug. 43(2). P 197-209. MJ ANESTHESIA. MN ACIDOSIS: pc. DYSAUTONOMIA-FAMILIAL. CHILD. CYSTIC-FIBROSIS. GLUCOSEPHOSPHATASE-DEFICIENCY. GLYCOGENOSIS-5. HOMOCYSTINURIA: me. HUMAN. HYPERINSULINISM. HYPOGLYCEMIA: pc. METABOLISM-INBORN-ERRORS. MUSCULAR-DISEASES: cn. MYOTONIA-ATROPHICA. MYOTONIA-CONGENITA. SUCCINYLCHOLINE: ae. SUPPORT-U-S-GOVT-P-H-S. EX The large number of congenital metabolic disorders makes a complete discussion of the biochemical problems, hazards, pharmacologic implications, and anesthetic recommendations for each impossible. This review is confined to discussion of certain aspects of several genetically acquired diseases of metabolism which the anaesthesiologist may be called upon to manage in the pediatric patient. Every genetic metabolic disease is the result of a specific enzyme defect (or defects) in function or in amount. In this review, diseases in which defects are known (e.g., homocystinuria), suspected (e.g., myotonia congenita), and unknown (e.g., cystic fibrosis) are discussed. Therapeutic approaches to genetic metabolic disease include: supplying the missing product; preventing substrate accumulation; preventing alternate product synthesis; enzyme induction; enzyme replacement; increasing deficient function with cofactor administration; and compensatory therapy. Obviously, the ideal way to treat the consequences of a genetic disease is to normalize the patient biochemically. For diseases where the pathophysiology is clear, this goal is approachable; for conditions where the pathophysiology is unknown, only compensatory therapy is possible. RF 001 SCHIMKE RN AMINO ACID METAB GENET VARIAT 297 967 002 CUSWORTH DC BR MED BULL 25 42 969 003 HARKER LA N ENGL J MED 291 537 974 004 MUDD SH J CLIN INVEST 49 1762 970 005 HOLLOWELL JG JR PROC SOC EXP BIOL MED 129 327 968 006 CARSON NAJ ARCH DIS CHILD 44 387 969 007 CHASE HP ARCH DIS CHILD 42 514 967 008 HAMBRAEUS L CLIN SCI 35 457 968 009 PERRY TL LANCET 2 474 968 010 WONG PWK PEDIATR RES 2 149 968 011 GAULL GE PEDIATR RES 5 265 971 012 WONG PWK PEDIATR RES 6 172 972 013 STURMAN JA BIOCHEM MED 5 404 971 014 CAREY MC IR J MED SCI 6 488 966 015 CAREY MC AM J MED 45 26 968 016 MUDD SH BIOCHEM BIOPHYS RES COMMUN 46 905 972 017 GOODMAN SI BIOCHEM MED 4 500 970 018 TALLAN HH BIOCHEM MED 9 90 974 019 STURMAN JA SCIENCE 169 74 970 020 FINKELSTEIN JD METABOLISM 23 387 974 021 HOLLOWELL JG JR LANCET 2 1428 969 022 RITCHIE JWK J OBSTET GYNAECOL BR COMMONW 80 664 973 023 HOLMGREN G UPSALA J MED SCI 78 215 973 024 RATNOFF OD SCIENCE 162 1007 968 025 CROOKE JW BR J ANAESTH 43 96 971 026 HARKER LA PROC AM SOC HEMATOL 15TH ANN 46 972 027 CROSS HE AM J OPHTHALMOL 75 405 973 028 SCHIMKE RN JAMA 193 711 965 029 BECKER PE ACTA NEUROL SCAND 49 480 973 030 BAXTER DW J CAN MED ASSOC 85 113 961 031 THRUSCH DC BRAIN 95 537 972 032 KIM C HAWAII MED J 33 15 974 033 WINTERS JL J BONE JOINT SURG 52 1345 970 034 CAUGHEY JE DYSTROPHIA MYOTONICA 963 035 HARVEY JC AM J MED 39 81 965 036 WELSH JD ARCH INTERN MED 114 669 964 037 FEARRINGTON EL AM HEART J 67 599 964 038 FISCH C N ENGL J MED 251 527 954 039 ROSENTHAL G NY STATE J MED 67 940 967 040 WATTERS GV ARCH NEUROL 17 137 967 041 KOHN NN N ENGL J MED 271 1179 964 042 BOURKE TD BR J ANAESTH 29 35 957 043 COBHAM IG ANESTH ANALG CLEVE 43 22 964 045 DI SANTAGNESE PA PEDIATRICS 18 387 956 046 SCHUSTER SR J THORAC CARDIOVASC SURG 48 750 964 047 HOLSCLAW DS MINN MED 52 1547 969 048 DOERSHUK CF ANESTH ANALG CLEVE 51 413 972 049 SALANITRE E ANESTHESIOLOGY 25 801 964 050 KATTWINKEL J J PEDIATR 82 234 973 051 BONDY PK DISORDERS CARBOHYD METAB DUNC 974 052 CORI GT J BIOL CHEM 199 661 952 053 FIELD JB J CLIN INVEST 44 1240 965 054 SCHWARTZ R PEDIATRICS 19 585 957 055 LOWE CU J CLIN INVEST 39 1007 960 056 HOWELL RR IN: STANBURY JB 149 972 057 COX JM ANESTHESIOLOGY 29 1221 968 058 JACKSON SH IN: KATZ J 973 059 ZUPPINGER K HELV MED ACTA 35 496 969 060 MCARDLE B CLIN SCI 10 13 951 061 MOMMAERTS WFHM PROC NAT ACAD SCI USA 45 791 959 062 SCHMID R J CLIN INVEST 38 2044 959 063 DANCIS J N ENGL J MED 274 207 966 064 FILLER J J PEDIATR 66 509 965 065 DANCIS J ANN NY ACAD SCI 151 876 968 066 HENKIN RI LIFE SCI 3 1319 964 067 SMITH AA N ENGL J MED 270 704 964 068 SMITH AA N ENGL J MED 268 705 963 069 GITTOW SE PEDIATRICS 46 513 970 070 WEINSHILBOUM RM N ENGL J MED 285 938 971 071 MASON DT AM J MED 41 898 966 072 KRICHMAN MM JAMA 170 529 959 073 MCCAUGHEY TJ CAN ANAESTH SOC J 12 558 965 074 MERIDY HW CAN ANAESTH SOC J 18 563 971 CT 1 CAMPBELL RL J ORAL MAXILLOFAC SURG 40 497 982 2 LYNN A CLIN ANAESTHESIOL 3 739 985 3 KEON TP INT ANESTHESIOL CLIN 23 87 985 PN 75032 RN 00198 AN 76060265 AU Lamm-L-U. Thorsen-I-L. Petersen-G-B. Jorgensen-J. Henningsen-K. Bech-B. Kissmeyer-Nielsen-F. TI Data on the HL-A linkage group. SO Ann-Hum-Genet. 1975 May. 38(4). P 383-90. MJ HISTOCOMPATIBILITY-ANTIGENS. HLA-ANTIGENS. LINKAGE-GENETICS. MN BLOOD-GROUPS. COMPUTERS. CYSTIC-FIBROSIS: fg. GENE-FREQUENCY. GENOTYPE. HUMAN. PHOSPHOGLUCOMUTASE: bi. RECOMBINATION-GENETIC. AB Lod scores from a study in 229 families of the linkage relations of HL-A-PGM3 to 19 marker loci and cystic fibrosis are reported. The data exclude that ADA belongs to this linkage group while they give weak support for the inclusion of P. There is weak evidence for linkage of cystic fibrosis to PGM3, but none for linkage to HL-A. No new suggestive linkages appeared. RF 001 AZEN EA BIOCHEM GENET 3 215 969 002 BACH FH AM J HUM GENET 25 208 973 003 CHEN SH SCIENCE 173 148 971 004 CREAGAN RP LANCET 2 1449 973 005 DANES BS J EXP MED 129 775 969 006 DANES BS AM J HUM GENET 23 297 971 007 DAUSSET J HISTOCOMPATIBILITY TESTING 972 008 EDWARDS JH CLIN GENET 3 371 972 009 EDWARDS JH HISTOCOMPATIBILITY TESTING 972 010 FELLOUS M EUR J IMMUNOL 3 543 973 011 JONGSMA A HUMANGENETIK 20 195 973 012 LAMM LU HUM HERED 24 273 974 013 LAMM LU HUM HERED 20 305 970 014 LAMM LU TISSUE ANTIGENS 2 205 972 015 LAMM LU NATURE NEW BIOL 231 109 971 016 MAYNARD-SMITH S MATH TABLES RES WORKERS IN HU 961 017 MIDDLETON J TISSUE ANTIGENS 4 366 974 018 RENWICK JH BR MED BULL 25 65 969 019 ROBSON EB ANN HUM GENET 36 393 973 021 SVEJGAARD A TISSUE ANTIGENS 1 81 971 CT 1 JONES EA SOMATIC CELL GENET 2 483 976 2 BIJNEN AB J IMMUNOGENET 3 171 976 3 WEITKAMP LR TISSUE ANTIGENS 7 273 976 4 GOODCHILD MC J MED GENET 13 417 976 5 BODMER WF CYTOGENET CELL GENET 16 24 976 6 LEWIS M CYTOGENET CELL GENET 16 317 976 7 SIMON M GUT 17 332 976 8 DAY NK VOX SANG 31 96 976 9 JERSILD C TRANSPLANTAT REV 32 43 976 10 RAUM D J CLIN INVEST 58 1240 976 11 ROTTHAUWE HW DTSCH MED WSCHR 101 849 976 12 MUNRO A NATURE 264 145 976 13 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 14 BOMFORD A LANCET 1 327 977 15 KOMPF J SCAND J IMMUNOL 6 453 977 16 FELLOUS M EUR J IMMUNOL 7 22 977 17 GIRALDO G CLIN IMMUNOL IMMUNOPATHOL 8 377 977 18 OHELA K TISSUE ANTIGENS 9 90 977 19 SAFWENBERG J TISSUE ANTIGENS 10 287 977 20 BREUNING MH HUM GENET 37 131 977 21 ROSENFELD SI J IMMUNOL 119 604 977 22 SIMON M N ENGL J MED 297 1017 977 23 HENNEQUET A TISSUE ANTIGENS 12 159 978 24 LEWIS M CYTOGENET CELL GENET 22 452 978 25 WEITKAMP L CYTOGENET CELL GENET 22 92 978 26 BODMER WF PROC R SOC LOND (B) 202 93 978 27 BODMER WF HARVEY LECTURES 1978 91 978 28 CUZNER ML MOL ASP MED 2 147 979 29 BERGER R CLIN GENET 15 245 979 30 HAUPTMANN G REV FR TRANSFUS IMMUNOHEMATOL 22 587 979 31 PEPPER B HUM HERED 29 279 979 32 BENDER K HUM GENET 49 159 979 33 RAUM D J CLIN INVEST 64 858 979 34 HERROD HG J PEDIATR 94 676 979 35 RUBINSTEIN P J IMMUNOL 122 2584 979 36 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 37 NAKAO Y CLIN EXP IMMUNOL 42 20 980 38 PEDERSEN L HUM GENET 54 371 980 39 LOPEZLARREA C HUM GENET 57 317 981 40 PLATZ P DIABETOLOGIA 23 16 982 41 NIELSEN OH ACTA PAEDIATR SCAND 71 339 982 42 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 43 MARDER HK KIDNEY INT 23 749 983 44 ROBSON EB ANN HUM GENET 48 347 984 45 REGUEIRO JR HUM GENET 67 437 984 46 EIBERG H CLIN GENET 27 206 985 47 EIBERG H CLIN GENET 28 265 985 48 PEDERSEN EB Q J MED 57 883 985 49 SCHMIEGELOW K CLIN GENET 29 374 986 50 WERDELIN L ACTA NEUROL SCAND 73 1 986 PN 75033 RN 00199 AN 75181965 AU Lieberman-J. Rodbard-S. TI Low blood pressure in young adults with cystic fibrosis: an effect of chronic salt loss in sweat?. SO Ann-Intern-Med. 1975 Jun. 82(6). P 806-8. MJ BLOOD-PRESSURE. CYSTIC-FIBROSIS: pp. SWEATING. WATER-ELECTROLYTE-BALANCE. MN ADOLESCENCE. ADULT. BLOOD-PRESSURE-DETERMINATION. CYSTIC-FIBROSIS: me. EXERTION. FEMALE. HUMAN. MALE. SEX-FACTORS. AB Young adults with cystic fibrosis have lower blood pressures than control subjects of similar age and sex. The low blood pressure may be related to the excessive loss of salt in the sweat of these patients. A beneficial effect of the cystic fibrosis gene may be protection against developing hypertension in both the homozygous and heterozygous states, especially if the heterozygote has borderline elevation of sweat electrolytes. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 JOHANSEN PG LANCET 1 455 968 003 RODBARD S AM HEART J 53 205 957 004 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 005 SHWACHMAN H PEDIATRICS 36 689 965 006 COATES EO JR DIS CHEST 49 195 966 007 TOMASHEFSKI JF CHEST 57 28 970 008 ROSENLUND ML ANN INTERN MED 78 959 973 009 MOSS AJ PROBLEMS OF BLOOD PRESSURE IN 962 010 HULL DH PRACTITIONER 210 195 973 011 QUINTERO-ATENCIO J N ENGL J MED 274 1224 966 012 LEVI J ISR J MED SCI 6 665 970 013 LIEBERMAN J AM J MED SCI 246 537 963 014 HOLMAN RL PEDIATRICS 24 34 959 015 HALLETT WY AM REV RESPIR DIS 92 714 965 CT 1 BEDROSSIAN CWM AM J CLIN PATHOL 70 244 978 2 LEMEN RJ AM REV RESPIR DIS 117 639 978 3 BARRY MM J AM DIET ASSOC 75 446 979 4 LAKE CR CLIN CHIM ACTA 92 141 979 5 STALCUP SA J CLIN INVEST 67 201 981 6 MOSS AJ PEDIATRICS 70 728 982 7 MELLINS RB AM J ROENTGENOL 138 999 982 8 DAVIS PB J CHRON DIS 36 269 983 9 MITCHELL EA AUST PAEDIATR J 21 127 985 10 DAVIS PB HORM METAB RES 18 217 986 PN 75034 RN 00200 AN 75181973 TI Pseudomonas aeruginosa infections: persisting problems and current research to find new therapies. SO Ann-Intern-Med. 1975 Jun. 82(6). P 819-31. MJ PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: th. MN ADULT. AGRANULOCYTOSIS: co, th. ANIMAL. ANTIBIOTICS. ANTIBODIES-BACTERIAL: bi. BLOOD-TRANSFUSION. BONE-MARROW: de. CHILD. CYSTIC-FIBROSIS: co. DOGS. GRANULOCYTES. HUMAN. IMMUNOSUPPRESSIVE-AGENTS: ae. IN-VITRO. LEUKEMIA: co. LYMPHOMA: co. PSEUDOMONAS-AERUGINOSA: ip. PSEUDOMONAS-INFECTIONS: mi, im. IMMUNOTHERAPY. BONE-MARROW: cy. AB Despite the availability of specific antibiotics, Pseudomonas aeruginosa bacteria still cause troublesome infections in patients with a variety of illnesses: extensive thermal injury, leukopenia from antineoplastic chemotherapy and other forms of immunosuppressive treatment, chronic pulmonary disease such as cystic fibrosis, or intravenous narcotic use. The use of antibiotics has improved the prognosis of pseudomonas infections considerably. However, patients with marginal or defective host immunity may need more extensive therapy to master the infection. By evaluating additional modalities of treatment such as granulocyte replacement, improved usage of antibiotics, and active (prophylaxis) or passive antibody administration, the optimal combination may be found. RF 001 LEVINE AS SEMIN HEMATOL 11 141 974 002 LEVINE AS SEMIN HEMATOL 9 141 972 003 BODEY GP ANN INTERN MED 64 328 966 004 LEVINE AS N ENGL J MED 288 477 973 005 BODEY GP EUR J CANCER 9 435 973 006 SCHIMPFF SC EUR J CANCER 9 445 973 007 ANDRIOLE VT J INFECT DIS 129 124 974 008 PENNINGTON JE J INFECT DIS SUPPL 130 159 974 010 DI SANTAGNESE PA N ENGL J MED 277 1287 967 011 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 012 BOXERBAUM B AM REV RESPIR DIS 108 777 973 013 DOGGETT RG APPL MICROBIOL 18 936 969 014$ SHIONOYA H JAPAN J EXP MED 37 359 972 015 NITO T TROP MED 14 1 972 016 PAVLATOU M ANN INST PASTEUR PARIS 102 300 962 017 FISHER MW J BACTERIOL 98 835 969 018 HOMMA JY JAPAN J EXP MED 41 89 971 019 HOMMA JY JAPAN J EXP MED 42 171 972 020 ZIERDT CH J BACTERIOL 87 1003 964 021 SONNENSCHEIN C ZENTRALBL BAKTERIOL 104 365 927 022 SCHIMPFF SW N ENGL J MED 284 1061 971 023 TATTERSALL MHN LANCET 1 162 972 024 STRUMIA MH AM J MED SCI 187 527 934 025 BOGGS DR N ENGL J MED 290 1055 974 026 GRAW RG JR TRANSFUSION 11 94 971 027 GRAW RG JR N ENGL J MED 287 367 972 028 MCCREDIE KB TRANSPLANT PROC 5 128 973 029 BENBUNAN M NOUV REV FR HEMATOL 13 469 973 030 DALE DC J CLIN INVEST 54 664 974 031 BUCKNER DR BLOOD 31 653 968 032 HERZIG GP BLOOD 39 554 972 033 TILLOTSON JR ANN INTERN MED 68 295 968 034 PENNINGTON JE AM J MED 55 155 973 035 HANESSIAN S NATURE NEW BIOL 229 209 971 036 YOUNG LS J INFECT DIS 126 257 972 037 ALEXANDER JW J TRAUMA 10 565 970 038 YOUNG LS ANN INTERN MED 79 518 973 039 HAGHBIN M CANCER 32 761 973 040 BJORNSON AB INFECT IMMUN 2 453 970 041 YOUNG LS INFECT IMMUN 2 495 970 042 ALEXANDER JW ARCH SURG 102 31 971 043 JONES CE J SURG RES 14 87 973 044 GREEN GM AM REV RESPIR DIS 102 691 970 045 REYNOLDS HY J IMMUNOL 111 358 973 046 REYNOLDS HY J LAB CLIN MED 84 559 974 047 REYNOLDS HY J IMMUNOL 111 358 973 048 REYNOLDS HY J CLIN INVEST 53 1351 974 049 HAND WL J CLIN INVEST 53 354 974 050 REYNOLDS HY CLIN RES 22 427 974 051 GOLDE DW N ENGL J MED 290 875 974 052 COHN EJ J AM CHEM SOC 72 465 950 053 BUCKLEY RH AM J DIS CHILD 124 376 972 CT 1 NEU HC CLIN HAEMATOL 5 449 976 2 DALE DC BLOOD 47 869 976 3 PAREDES L$ REV MED CHIL 104 615 976 4 PAREDESP L$ REV MED CHIL 104 538 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 REYNOLDS HY JAMA 236 2190 976 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 8 SENSAKOVIC JW INFECT IMMUN 18 304 977 9 LAVERDIERE M CURR THER RES CLIN EXP 21 464 977 10 HARANAKA K JAP J EXP MED 47 35 977 11 KAZMIEROWSKI JA J INFECT DIS 135 438 977 12 BALTCH AL AM J MED SCI 274 119 977 13 KROPINSKI AMB ANTIMICROB AGENTS CHEMOTHER 13 494 978 14 MARKOWITZ SM INFECT IMMUN 22 530 978 15 LIM DT ANN ALLERGY 41 30 978 16 PENNINGTON JE J INFECT DIS 137 764 978 17 KULCZYCKI LL JAMA 240 30 978 18 VANFURTH R NETH J MED 22 123 979 19 DIMITRACOPOULOS G INFECT IMMUN 23 87 979 20 PENNINGTON JE INFECT IMMUN 25 1029 979 21 HOLLOWAY BW MICROBIOL REV 43 73 979 22 LOENINGBAUCKE VA J PEDIATR 95 630 979 23 SOTER NA J PEDIATR 95 197 979 24 PENNINGTON JE J INFECT DIS 139 396 979 25 PENNINGTON JE J INFECT DIS 140 881 979 26 PENNINGTON JE J INFECT DIS 140 73 979 27 THOMASSEN MJ J INFECT DIS 140 873 979 28 MCCARTHY MM CLIN PEDIATR 19 746 980 29 KAMIMURA T INFECT IMMUN 29 13 980 30 FICK RB J IMMUNOL METH 38 103 980 31 RAMSEY PG MEDICINE 59 206 980 32 SIEBERT WT SOUTH MED J 73 75 980 33 CRYZ SJ PROC NAT ACAD SCI USA 77 7199 980 34 RAINES JM LARYNGOSCOPE 90 369 980 35 PENNINGTON JE J INFECT DIS 142 191 980 36 ROSENSTEIN BJ JOHNS HOPKINS MED J 147 188 980 37 FICK RB CLIN CHEST MED 2 91 981 38 CARLONE S MICROBIOLOGICA 4 215 981 39 LANDAU Z SCAND J INFECT DIS 13 227 981 40 BLACKWOOD LL INFECT IMMUN 32 443 981 41 CRYZ SJ INFECT IMMUN 32 759 981 42 KOEPP LH INFECT IMMUN 33 788 981 43 LONG GG AM J VET RES 42 2129 981 44 MASHIMO K JAP J EXP MED 51 271 981 45 PENNINGTON JE J CLIN INVEST 68 1140 981 46 MOSS RB J PEDIATR 99 215 981 47 MACONE AB N ENGL J MED 304 1445 981 48 FROLAND SS SCAND J INFECT DIS 1981 72 981 49 MOROZ AF ZH MIKROBIO EPIDEMIO IMMUNOBI 1981 6 981 50 JANDA JM MICROB ECOL 8 335 982 51 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 52 DAVIDSON S ISR J MED SCI 18 859 982 53 PENNINGTON JE ANTIMICROB AGENTS CHEMOTHER 22 406 982 54 SATO K ANTIMICROB AGENTS CHEMOTHER 22 548 982 55 OTANI T MICROBIOL IMMUNOL 26 67 982 56 OHMAN DE INFECT IMMUN 37 662 982 57 WEINER RN EXP MOL PATH 37 249 982 58 PIER GB J CLIN INVEST 69 303 982 59 LAMBRIS J J INFECT DIS 145 78 982 60 LONG GG J AM VET MED ASSOC 181 1343 982 61 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 62 CRYZ SJ REV INFECT DIS 5 S992 983 63 PATT LM INT J TISS REACT EXP CLIN ASP 5 135 983 64 PENNINGTON JE REV INFECT DIS 5 S852 983 65 NEU HC J ANTIMICROB CHEMOTHER 11 1 983 66 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 67 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 68 URBACH J ISR J MED SCI 19 992 983 69 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 31 199 983 70 BLACKWOOD LL INFECT IMMUN 39 198 983 71 MARKHAM RB INFECT IMMUN 41 232 983 72 LAL S J GEN MICROBIOL 129 93 983 73 PIER GB J INFECT DIS 148 206 983 74 CONKLIN JJ SURG GYNECOL OBSTET 156 809 983 75 ROSE HD JAMA 250 2027 983 76 MACKOWIAK PA REV INFECT DIS 6 649 984 77 PENNINGTON JE REV INFECT DIS 6 S657 984 78 KEREN G J INFECT 9 22 984 79 SPEERT DP PEDIATR RES 18 431 984 80 SCHIFF JB ANTIMICROB AGENTS CHEMOTHER 25 49 984 81 SCHIFF JB ANTIMICROB AGENTS CHEMOTHER 26 1 984 82 OTANI T MICROBIOL IMMUNOL 28 1077 984 83 CRYZ SJ INFECT IMMUN 43 795 984 84 SPEERT DP INFECT IMMUN 43 1006 984 85 PIER GB J IMMUNOL 133 734 984 86 RUSNAK MG J INFECT DIS 149 980 984 87 ELNIMA EI ZENTRALBL BAKT MIKROB HYG (A) 258 120 984 88 CRYZ SJ EUR J CLIN MICROBIOL 4 153 985 89 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 90 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 91 MARTY N MED MALAD INFECT 15 604 985 92 SUTTORP N MED WELT 36 1238 985 93 KOMIYAMA K ORAL SURG 59 590 985 94 SUTTORP N J CELL PHYSIOL 123 64 985 95 SAMYKINA TD ZH MIKROBIO EPIDEMIO IMMUNOBI 21 986 96 PENNINGTON JE REV INFECT DIS 8 S426 986 97 KRIEG DP J CLIN MICROBIOL 24 986 986 98 SALERNO RA MED (BUENOS AIRES) 46 377 986 99 SEEGER W INFECT IMMUN 52 846 986 100 PENNINGTON JE INFECT IMMUN 54 239 986 101 PIER GB J CLIN INVEST 77 491 986 102 FICK RB AM REV RESPIR DIS 133 418 986 103 SPEERT DP J HOSP INFECT 9 11 987 104 KHARAZMI A TRANS R SOC TROP MED HYG 81 49 987 105 PENNINGTON JE J INFECT DIS 155 973 987 PN 75035 RN 00201 AN 75203428 AU Adshead-P-C. Martinez-J-R. Kilburn-K-H. Hess-R-A. TI Ciliary inhibition and axonemal microtubule alterations in freshwater mussels. SO Ann-NY-Acad-Sci. 1975 Jun 30. 253. P 192-212. MJ CILIA: de. MICROTUBULES: de. MUSSELS: ul. MN ANIMAL. BLOOD. CILIA: ph. CYSTIC-FIBROSIS: bl. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MALE. MICROSCOPY-ELECTRON. MOVEMENT. MUSSELS: de, ph. RATS. RESERPINE: pd. SALIVA: de. EX A phenomenon associated with the genetic disease of cystic fibrosis (CF) is the presence of a serum factor that stops or discoordinates ciliary beating. This effect has been demonstrated by exposing freshwater mussel or oyster gill cilia, or rabbit tracheal cilia, to sera from homozygous or heterozygous cystic fibrosis subjects. Assay reproducibility is unsatisfactory, however, and the mechanism of the effect is not understood. Nor do we know why there may be considerable differences between the activity of tested heterozygotes and of homozygotes. Because the ciliary assay is capricious, there is a need to understand how ciliary beating is disturbed or stopped. Since microtubules are important in the mechanism of ciliary motion, they could conceivably show alterations during the response to inhibitory substances. There has been no ultrastructural study, however, of the effects of ciliary inhibition on the axonemal components. Therefore, this study was designed to examine ciliary structure during the different stages of ciliary inhibition. Rapid microtubular disappearance and reappearance occurs in the terminal cilia of the freshwater mussel Unio when exposure to CF serum causes a shock-stop reaction, and return to water allows resumption of normal ciliary beating. In contrast, a slow-stop reaction causes distortion of cilia, asynchronous beating, and ejection of cells from the distal gill filament, but ciliary axonemal structure is preserved. Slow-stopped cilia do not resume their beating in water. Saliva from reserpine-treated rats causes ciliary inhibition similar to that induced by CF serum. RF 001 SPOCK A PEDIATR RES 1 173 967 002 BOWMAN BH SCIENCE 164 325 969 003 BOWMAN BH SCIENCE 167 871 970 004 BESLEY GTN J MED GENET 6 278 969 005 CONOVER JH PEDIATR RES 7 220 973 006 CHERRY JD J PEDIATR 79 937 971 007 MARTINEZ JR PEDIATR RES 9 463 975 008 MARTINEZ JR PEDIATR RES 9 463 975 009 SATIR P J CELL BIOL 18 345 963 010 SATIR P J GEN PHYSIOL 50 241 967 011 SATIR P J CELL BIOL 39 77 968 012 AIELLO EL J CELL BIOL 54 493 972 013 WARNER FD J CELL SCI 12 313 973 014 SHIGENAKA Y J CELL SCI 8 127 971 015 GIBBONS IR J BIOPHYS BIOCHEM CYTOL 11 179 961 016$ MILLONIG G J APPL PHYS 32 1637 961 017 SATO T STAIN TECHNOL 48 223 973 018 REYNOLDS ES J CELL BIOL 17 208 963 019 ADSHEAD PC CF CLUB ABST 15 25 974 020 HOPKINS JM J CELL SCI 7 823 970 021 COSTELLO DP BIOL BULL 145 292 973 022 CONOVER JH LANCET 1 1194 973 023 BEHNKE O INT REV EXP PATH 9 1 970 024 WILSON L FED PROC 33 151 974 025 WILSON L FED PROC 33 158 974 026 WILSON L J CELL BIOL 58 709 973 027 GOSS RJ ADV CELL BIOL 1 233 970 028 BOVEE EC ARCH PROSTISTENK 104 503 960 029 BARDELE CF Z ZELLFORSCH MIKROSK ANAT 130 219 972 030 WITMAN GB J CELL BIOL 54 507 972 031 MARTINEZ JR CF CLUB ABST 15 3 974 032 VOLLET JJ J CELL BIOL 55 269A 972 CT 1 BAUR PS TEX REP BIOL MED 34 155 976 2 CZEGLEDYNAGY E LAB INVEST 35 588 976 3 MARTINEZ JR MO MED 73 173 976 4 WOOD DL PEDIATR RES 11 827 977 5 KILBURN KH J ULTRASTRUCT RES 60 34 977 6 BOGART BI PEDIATR RES 12 15 978 7 MARTINEZ JR PEDIATR RES 13 1156 979 8 NAGY EC PEDIATR RES 13 729 979 9 KENNEDY JR PEDIATR RES 14 1173 980 10 SILLERS PJ CAN J BIOCHEM 59 777 981 PN 75036 RN 00202 AN 75163543 AU McDougal-W-S. Izant-R-J-Jr. Zollinger-R-M-Jr. TI Primary peritonitis in infancy and childhood. SO Ann-Surg. 1975 Mar. 181(3). P 310-3. MJ PERITONITIS: di. MN ABDOMEN: ra. ABDOMEN-ACUTE: di. ADRENAL-HYPERPLASIA-CONGENITAL: co. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DIAGNOSIS-DIFFERENTIAL. FEMALE. BACTERIA: ip. BACTERIA: ip. HUMAN. HYALINE-MEMBRANE-DISEASE: co. HYDRONEPHROSIS: co. INFANT. INFANT-NEWBORN. MALE. NEPHROTIC-SYNDROME: co. PERITONITIS: et, mi. PNEUMONIA: co. SEX-FACTORS. TIME-FACTORS. URINARY-TRACT-INFECTIONS: co. AB Primary peritonitis, rarely diagnosed preoperatively, is an uncommon disease accounting for 2.1% of all pediatric abdominal emergencies. It is often associated with urinary or hepatic pathology, the former the source of the infecting organism in the majority of cases, and presents with characteristic symptoms depending upon whether it occurs in infancy or childhood. The symptoms and signs which allow for a positive prospective diagnosis are illustrated by comparing this disease to those entities with which it is most often confused, e.g. diffuse peritonitis of other etiologies, and include a short duration of symptoms, associated urinary tract infection and an absence of free air on abdominal roentgenograms. In the past, gram positive organisms were the most common infecting agent; however, in this series gram negative bacteria accounted for 69% or the organisms. Antibiotics with a gram negative spectrum and exploratory laparotomy with appendectomy are the hallmarks of therapy, the latter replaced by abdominal tap only in the patient who satisfies the criteria for primary peritonitis and in whom an associated disease makes the risk of surgery prohibitive. RF 001 EPSTEIN M N ENGL J MED 278 69 968 002 FOWLER R JR AUST NZ J SURG 26 204 957 003 FOWLER R JR AUST PAEDIATR J 7 73 971 004 FRIEDLAND JA AM J SURG 119 737 970 005 GOLDEN GT SURG GYNECOL OBSTET 135 513 972 006 LADD WE JAMA 113 1455 939 007 LADD WE ABDOMINAL SURGERY INFANCY CHI 188 941 008 SCHWEINBURG FB N ENGL J MED 242 747 959 CT 1 PRIGOGINE T ACTA CLIN BELG 33 143 978 2 MATTHEWS P BR MED J 2 903 979 3 TAYLOR L J PEDIATR 95 556 979 4 UDALL DA J UROL 125 750 981 5 KRENSKY AM AM J DIS CHILD 136 732 982 6 GORSKI J CLIN PEDIATR 22 183 983 7 HOFFMANN S DAN MED BULL 30 265 983 8 JENNY P Z KINDERCHIRURGIE 38 36 983 9 VANRIJSSEN H BR J SURG 70 446 983 10 MOORE JL J PEDIATR SURG 19 594 984 11 NOHR CW CAN J SURG 27 179 984 12 CLARK JH J PEDIATR 104 495 984 13 FREIJ BJ AM J DIS CHILD 138 1058 984 14 STEVENSON RJ SURG CLIN NORTH AM 65 1181 985 15 SERLO W ANN CHIR GYNAECOL 74 183 985 16 BASART MAG MED CLIN 84 207 985 PN 75037 RN 00203 AN 76110493 AU Childs-B. TI Genetic screening. SO Annu-Rev-Genet. 1975. 9. P 67-89. (REVIEW). MJ HEREDITARY-DISEASES: pc. INFANT-NEWBORN-DISEASES: di. MASS-SCREENING. PRENATAL-DIAGNOSIS. MN ADENOSINE-DEAMINASE: df. ADULT. LIPOIDOSIS: pc. AMNIOCENTESIS. CHILD. CHROMOSOME-ABNORMALITIES: pc. CYSTIC-FIBROSIS. DOWNS-SYNDROME: pc. HEMOGLOBINOPATHIES: pc. HUMAN. HYPERLIPIDEMIA: pc. INFANT-NEWBORN. METABOLISM-INBORN-ERRORS: pc. MUSCULAR-DYSTROPHY: pc. PHENYLKETONURIA: pc. POLYMORPHISM-GENETICS. REVIEW. THALASSEMIA: pc. SUPPORT-U-S-GOVT-P-H-S. EX The recent proliferation of genetic knowledge has been accompanied by speculation about its uses in the pursuit of both medical and social goals, as well as by fear and concern about possible misuses. These speculations have appeared in the proceedings of numerous symposia and in many books and papers. A review of this literature reveals that the nonmedical participants in this debate were preoccupied with those aims of genetics that might benefit (or threaten) society, while the physicians were concerned with the accomplishment of standard medical missions in which social aims figure only incidentally. These missions are diagnosis, management, and prevention of disease. The specific uses of genetic knowledge most frequently mentioned were advances in the taxonomy of disease that lead to improvements in diagnosis and treatment of individual patients and their relatives, genetic counseling, and genetic screening. The latter two, particularly screening, are preventive and represent the interface between medical genetics, especially population genetics, and society. This review focuses on developments in genetic screening, defined as the search in a population, generally for medical reasons, for individuals possessing particular genotypes. RF 001 MOTULSKY AG SCIENCE 185 653 974 002 CHILDS B YALE J BIOL MED 46 297 973 003 ANON GENETIC SCREENING PROG PRIN A 975 004 POWLES J SCI MED MAN 1 1 973 005 CARTER CO GREAT ORMOND ST J 11 65 968 006 ROBERTS DF ARCH DIS CHILD 45 33 970 007 CHILDS B IN: SUTTON HE 3 972 008 SCRIVER CR CAN MED ASSOC J 108 1111 973 009 DAY N AM J HUM GENET 25 237 973 010 LA DU BN IN: MCKUSICK VA 91 973 011 JICK H N ENGL J MED 291 824 974 012 STOCKINGER HE J OCCUP MED 15 564 973 013 THOMAS L BIOSCIENCE 24 99 974 014 WHITE K SCI AM 229 23 973 015 HARRIS H ANN HUM GENET 36 9 972 016 HUTCHINSON J PEDIGREE OF DISEASE 881 017 ROLLESTON H IDIOSYNCRACIES 927 018 GARROD AE INBORN FACTORS IN DISEASE 157 931 019 RIMOIN DL MED CLIN NORTH AM 55 807 971 020 BECKER MA ANNU REV MED 25 15 974 021 WYNGAARDEN JB AM J MED 56 651 974 022 MURPHY EA CIRC RES SUPPL 32 129 973 023 SWIFT M AM J HUM GENET 26 304 974 024 BESSMAN SP J PEDIATR 81 834 972 025 CHILDS B ANNU REV MED 23 374 972 026 SWIFT M SCIENCE 178 308 972 027 COHEN C MED CLIN NORTH AM 58 25 974 028 LEVINE BB CLIN IMMUNOL ALLERGY IN PAEDI 49 973 029 MARSH DG SCIENCE 179 691 973 030 MCDEVITT HO LANCET 1 1269 974 031 GUTHRIE R US DHEW CHILDRENS BUREAU PUBL 965 032 BLASKOVICS ME ARCH DIS CHILD 49 835 974 033 HOLTZMAN NA JAMA 229 667 974 034 HOLTZMAN NA J PEDIATR 85 175 974 035 HANSEN H AM J MENT DEFIC 75 22 970 036 HOWELL RR SOC BIOL SUPPL 19 18 29 971 037 PERRY TL N ENGL J MED 289 395 973 039 LEVY HL ADV HUM GENET 4 1 973 040 RAINE DN LANCET 2 966 974 041 ANON ACTA PAEDIATR SCAND 63 413 973 042 KOMROWER GM PEDIATRICS 53 182 974 043 LEVY HL N ENGL J MED 288 1299 973 044 KOMROWER GM LANCET 1 1047 974 045 ANON LANCET 1 710 974 046 POPKIN JS LANCET 1 721 974 047 LEVY HL N ENGL J MED 291 1214 974 048 BEUTLER E J LAB CLIN MED 68 137 966 049 ANON N ENGL J MED 291 1414 974 050 CLOW CL PROG MED GENET 9 159 973 051 GOLDSTEIN JL J CLIN INVEST 52 1533 973 052 GOLDSTEIN JL J CLIN INVEST 52 1544 973 053 GOLDSTEIN JL J LAB CLIN MED 85 15 975 054 KWITEROVICH PO JR PEDIATRICS 53 455 974 055 MOTULSKY AG IN: MILUNSKY A 306 975 057 MITTMAN C ARCH ENVIRON HEALTH 27 201 973 058 MITTMAN C ISR J MED SCI 9 1311 973 059 KUEPPERS F ANN INTERN MED 80 209 974 060 COOPER DM AM REV RESPIR DIS 110 708 974 061 MORSE JO N ENGL J MED 292 278 975 062 MORIN T LANCET 1 250 975 063 GIBSON LE CLIN PEDIATR 12 450 973 064 SCHUTT WH ARCH DIS CHILD 43 178 968 065 BRIMBLECOMBE FSW LANCET 2 1428 973 066 STEPHAN U PEDIATRICS 55 35 975 067 MEUWISSEN HJ J PEDIATR 86 169 975 068 MOORE EC J PEDIATR 85 802 974 069 ZELLWEGER H PEDIATRICS 55 30 975 070 MOTULSKY AG ISR J MED SCI 9 1341 973 071 JACKSON RE ARCH INTERN MED 133 533 974 072 WHITTEN CF N ENGL J MED 288 318 973 073 WHITTEN CF ARCH INTERN MED 133 681 973 074 YOUNG WI OHIO MED J 70 27 930 075 KELLON DB JAMA 227 71 974 076 HAMPTON ML AM J DIS CHILD 128 58 974 077 STAMATOYANNOPOULOS G IN: MOTULSKY AG 973 078 ANON S HEMOGLOBINOPATHIES: EVAL ST 973 079 PEARSON HA N ENGL J MED 288 351 973 080 KAZAZIAN HH JR ANN NY ACAD SCI 241 691 974 081 HOBBINS JC N ENGL J MED 290 1065 974 082 CHANG H N ENGL J MED 290 1067 974 083 KABACK MM PROG MED GENET 10 103 974 085 MILUNSKY A PRENATAL DIAGNOSIS OF HEREDIT 973 086 HARRIS H PRENATAL DIAG SELECTIVE ABORT 974 087 BLUMBERG B AM J HUM GENET 26 15A 974 088 CARTER CO J BIOSOCIAL SCI 1 71 969 089 MILUNSKY A J PEDIATR 84 889 974 090 BROCK DJH LANCET 2 923 973 091 ANON BR MED J 1 414 975 092 JACOBS PA ANN HUM GENET 37 359 974 093 HAMERTON JL CAN MED ASSOC J 106 776 972 094 LUBS HA IN: JACOBS PA 119 970 095 GERALD PS IN: JACOBS PA 143 970 096 MACHIN GA LANCET 1 549 974 097 GARVEY M ARCH DIS CHILD 48 937 973 098 LEONARD MF PEDIATRICS 54 208 974 099 ROBINSON AM LANCET 1 1343 974 100 BORGAONKAR DS PROG MED GENET 10 135 974 101 TENNES K AM J HUM GENET 27 71 975 102 MELLMAN WJ BIRTH DEF ORIG ART SER 10 123 974 103 BECK E N ENGL J MED 291 1166 974 105 KABACK MM ETHICAL SOCIAL LEGAL DIMEN SC 974 106 CHILDS B AM J HUM GENET 26 120 974 107 ROSENSTOCK IM MILBANK MEM FUND Q 44 94 966 CT 1 SCRIVER CR SCIENCE 200 946 978 2 ANON CAN MED ASSOC J 121 1193 979 3 KHOURY MJ AM J PUBLIC HEALTH 75 1204 985 PN 75038 RN 00204 AN 78143452 AU Goodchild-M-C. Banks-A-J. Drolc-Z. Anderson-C-M. TI Liver scans in cystic fibrosis. SO Arch-Dis-Child. 1975 Oct. 50(10). P 813-5. MJ CYSTIC-FIBROSIS: ri. LIVER: ri. MN ADOLESCENCE. CHILD. COMPARATIVE-STUDY. FEMALE. HUMAN. LIVER-FUNCTION-TESTS. MALE. AB Liver scans were performed on 16 cystic fibrosis patients. Most scans were considered abnormal. In most patients, the results of serological liver function tests were normal. Liver scanning in cystic fibrosis is unlikely to make a significant contribution to an assessment of prognosis. RF 001 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 002 COLOMBETTI LG J NUCL MED 10 597 969 003 DI SANTAGNESE PA PEDIATRICS 18 387 956 004 FEIGELSON J ACTA PAEDIATR SCAND 61 337 972 005 KATTWINKEL J J PEDIATR 82 234 973 006 LIEWENDAHL K ACTA MED SCAND 192 395 972 007 LUDBROOK J GASTROENTEROLOGY 62 1013 972 008 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 SMITH AL ARCH DIS CHILD 52 633 977 2 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 3 GIUNTA A RIV ITAL PEDIATR 9 171 983 PN 75039 RN 00205 AN 78143443 AU Goodchild-M-C. Murphy-G-M. Howell-A-M. Nutter-S-A. Anderson-C-M. TI Aspects of bile acid metabolism in cystic fibrosis. SO Arch-Dis-Child. 1975 Oct. 50(10). P 769-78. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: me. FECES: an. MN ADOLESCENCE. ADULT. BILE-ACIDS-AND-SALTS: bl. BODY-FLUIDS: me. CHILD. CHILD-PRESCHOOL. DUODENUM: me. FEMALE. HUMAN. INFANT. LIPIDS: me. MALE. PANCREATIC-DISEASES: me. AB Previous reports have indicated that cystic fibrosis (CF) patients with pancreatic enzyme insufficiency have a raised faecal bile acid output. In this study, 18 out of 29 CF patients and 2 out of the 4 non-CF patients with pancreatic enzyme insufficiency had raised faecal bile acid levels. In the CF patients no correlation was found between faecal bile acid and faecal fat excretion, but an inverse relation was shown between faecal bile acid values and age. Those CF patients with overt liver disease tended to have the lowest faecal bile acid values. Duodenal spiration in 5 CF patients and in one non-CF patient with pancreatic enzyme insufficiency (Shwachman-Diamond syndrome), produced very small fluid volumes. Duodenal fluid mean total bile acid concentrations were within normal limits. Estimation of serum bile acids in these 6 patients showed that 3 patients had raised serum bile acid values. It is suggested that excessive faecal bile acid loss may produce a contraction of the bile acid pool, and lead eventually to a reduction of intraduodenal bile acid concentrations. Measures which curtail faecal bile acid loss may have a particular significance in the management of CF. RF 001 ANDERSON CM LANCET 1 836 952 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 BURKE V BR MED J 2 1050 966 004 CAREY JB JR GASTROENTEROLOGY 56 1249 969 005 DI SANTAGNESE PA PEDIATRICS 18 387 956 006 DOWLING RH GASTROENTEROLOGY 62 122 972 007 DRASAR BS GASTROENTEROLOGY 56 71 969 008 GATZIMOS CD AM J DIS CHILD 89 182 955 009 GRACEY M AUSTRALAS ANN MED 18 91 969 010 HADORN B CAN MED ASSOC J 98 377 968 011 HADORN B J PEDIATR 73 39 968 012 HEATON KW BILE SALTS IN HEALTH AND DISE 972 013 KAPLOWITZ N GASTROENTEROLOGY 62 768 972 014 LAPEY A J PEDIATR 84 328 974 015 LEYLAND C ARCH DIS CHILD 45 714 970 016 MATTHEWS LW PEDIATRICS 27 351 961 017 MOORE RB J CLIN INVEST 47 1517 968 018 MURPHY GM ANN CLIN BIOCHEM 9 67 972 019 MURPHY GM J CLIN PATHOL 23 594 970 020 MURPHY GM CLIN CHIM ACTA 54 81 974 021 POLEY JR J LAB CLIN MED 63 838 964 022 RUDMAN D J CLIN INVEST 36 530 957 023 SCHAFFNER F LANCET 2 355 969 024 SHWACHMAN H J PEDIATR 65 645 964 025 TAYLOR WF AM J DIS CHILD 123 161 972 026 TURNBERG LA GUT 11 126 970 027 TYSON KRT J PEDIATR SURG 3 271 968 028 VALMAN HB ARCH DIS CHILD 46 805 971 029 VAN DEEST BW J CLIN INVEST 47 1314 968 030 VAN DE KAMER JH J BIOL CHEM 177 347 949 031 WEBER A PEDIATRICS 50 73 972 032 WEBER A N ENGL J MED 289 1001 973 033 WEBER AM CLIN CHIM ACTA 39 524 972 034 WEBER A CLIN RES 20 257 972 035 WEBSTER R ARCH DIS CHILD 28 343 953 036 WIGGINS HS GUT 8 415 967 CT 1 HUANG CTL AM J CLIN NUTR 29 1196 976 2 ROY CC CLIN GASTROENTEROL 6 377 977 3 ROLLER RJ GASTROENTEROLOGY 72 661 977 4 KERN F GASTROENTEROLOGY 73 631 977 5 ROY CC GASTROENTEROLOGY 73 631 977 6 WATKINS JB GASTROENTEROLOGY 73 1023 977 7 BALISTRERI WF J PEDIATR 90 21 977 8 ROY CC N ENGL J MED 297 1301 977 9 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 10 SMALLEY CA ARCH DIS CHILD 53 477 978 11 BENNION LJ N ENGL J MED 299 1221 978 12 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 13 FEIGELSON J NOUV PRESSE MED 8 3029 979 14 STARKEY BJ MONOGR PAEDIATR 10 12 979 15 HARRIES JT ARCH DIS CHILD 54 19 979 16 LLOYDSTILL JD AM J CLIN PATHOL 71 444 979 17 REGAN PT GASTROENTEROLOGY 77 285 979 18 CHASE HP J PEDIATR 95 337 979 19 ROY CC PROC SOC EXP BIOL MED 161 105 979 20 DAVIDSON GP J CLIN PATHOL 33 390 980 21 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 22 BOYLE BJ GASTROENTEROLOGY 78 950 980 23 BALISTRERI WF J PEDIATR 96 582 980 24 PARK RW GASTROENTEROLOGY 81 1143 981 25 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 26 COLOMBO C RIV ITAL PEDIATR 8 77 982 27 HILDEBRAND H GUT 23 243 982 28 ABDULKARIM FW GASTROENTEROLOGY 82 758 982 29 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 30 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 31 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 32 COLOMBO C DIG DIS SCI 28 306 983 33 DOHERTY DE SOUTH MED J 76 1580 983 34 BASS S GASTROENTEROLOGY 84 1592 983 35 MASOERO G AM J DIS CHILD 137 167 983 36 NIESSEN KH GUT 25 26 984 37 ZENTLERMUNRO PL GUT 25 500 984 38 ABRAMS CK J CLIN INVEST 73 374 984 39 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 40 ROBB TA GUT 26 1246 985 41 ZENTLERMUNRO PL GUT 26 892 985 42 SETCHELL KDR CLIN CHIM ACTA 151 101 985 43 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 44 WEIZMAN Z GUT 27 1043 986 45 LEROY C DIG DIS SCI 31 911 986 46 DUTTA SK GASTROENTEROLOGY 91 1243 986 PN 75040 RN 00206 AN 76110779 AU Jones-R-S. TI Letter: Bronchial lability in cystic fibrosis. SO Arch-Dis-Child. 1975 Nov. 50(11). P 909. MJ BRONCHI: pp. CYSTIC-FIBROSIS: pp. MN HUMAN. RESPIRATORY-FUNCTION-TESTS: mt. EX It has always seemed to me that the use of the Exercise Lability Index (ELI) distorts results and may be misleading because a falsely high value for lability is obtained when the PEF at rest is low. It is incorrect to compare percentage values from such patients with percentage values from those with figures at rest which are near to normal. The Jones Liability Index avoids this distortion. I do not suggest that a proportion of CF patients do not have abnormal lability but would maintain that the ELI introduces inaccuracy which may blur the distinction between CF and other respiratory conditions and may lead to the postulation of problems concerning lability in CF which do not necessarily exist. Failure to use a bronchodilator agent to reinforce brief exercise may also cause distortion but in the opposite direction. RF 001 COUNAHAN R ARCH DIS CHILD 50 477 975 002 DAY G ARCH DIS CHILD 48 355 973 003 JONES RS BR MED J 2 972 966 CT 1 SILVERMAN M ARCH DIS CHILD 53 873 978 PN 75041 RN 00207 AN 75145262 AU Elliott-R-B. Robinson-P-G. TI Unusual clinical course in a child with cystic fibrosis treated with fat emulsion. SO Arch-Dis-Child. 1975 Jan. 50(1). P 76-8. MJ CYSTIC-FIBROSIS. OILS: tu. MN CYSTIC-FIBROSIS: dt, di. EMULSIONS. FEMALE. HUMAN. ILEOSTOMY. INFANT. INFANT-NEWBORN. INFUSIONS-PARENTERAL. INTESTINAL-OBSTRUCTION: su. MECONIUM. PANCREATIC-EXTRACTS: tu. SOY-BEANS. SURGICAL-WOUND-INFECTION. SWEAT: an. AB A child diagnosed as having cystic fibrosis by customary criteria has been given regular parenteral soya oil emulsion from near birth. Sweat tests have improved, pancreatic achylia was relieved, and the child at present remains entirely well. Correction of the essential fatty acid deficiency found in cystic fibrosis may prevent some of the manifestations of the disease. RF 001 AHLUWALIA B J NUTR 92 205 967 002 CUTHBERT MF IN: CUTHBERT MF 973 003 HANSEN AE J NUTR 66 565 958 004 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 005 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 006 KUO PT J PEDIATR 60 394 962 007 MORRISON WR J LIPID RES 5 600 964 008 SCHWARTZ IL J CLIN INVEST 35 114 956 CT 1 DODGE JA BR MED J 2 192 975 2 MCEVOY FA LANCET 2 236 975 3 RIVERS JPW LANCET 2 642 975 4 ROBINSON PG LANCET 2 919 975 5 THOMPSON G LANCET 2 769 975 6 DODGE JA ARCH DIS CHILD 50 578 975 7 WARNER JO PEDIATRE 12 243 976 8 CAMPBELL IM PEDIATRICS 57 480 976 9 CHASE HP PEDIATRICS 57 441 976 10 ELLIOTT RB PEDIATRICS 57 474 976 11 HUBBARD VS LANCET 2 1302 977 12 CHAZALETTE JP PEDIATRE 13 303 977 13 DALE G ADV CLIN CHEM 19 207 977 14 GALABERT C LANCET 2 903 978 15 DAVIDSON GP AUST PAEDIATR J 14 80 978 16 PHELAN PD AUST PAEDIATR J 14 61 978 17 BERG U MONOGR PAEDIATR 10 1 979 18 BOHLES H Z ERNAHRUNGSWISS 18 81 979 19 LLOYDSTILL JD PEDIATRICS 64 50 979 20 ROGIERS V PEDIATR RES 14 1088 980 21 HUBBARD VS AM J CLIN NUTR 33 2281 980 22 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 23 SOLOMONS NW AM J CLIN NUTR 34 462 981 24 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 25 COLLINS JE ACTA PAEDIATR SCAND 74 423 985 26 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 27 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 PN 75042 RN 00208 AN 76038708 AU Dodge-J-A. Prosser-R. Yassa-J. TI Letter: Essential fatty acids in cystic fibrosis. SO Arch-Dis-Child. 1975 Jul. 50(7). P 578-9. MJ CYSTIC-FIBROSIS: dh. FATTY-ACIDS-ESSENTIAL: tu. MN FATTY-ACIDS-ESSENTIAL: ad. HUMAN. EX We have read with interest the report by Elliott and Robinson concerning the apparent beneficial effects of intravenous fat infusions in a child with cystic fibrosis. We question whether the increase in essential fatty acids brought about by soya oil can account for the favourable course of the disease in this patient. Our own observations confirm that essential fatty acids levels are often below normal in cystic fibrosis, but they do not correlate with the clinical condition of the patient. It seems unlikely that minor degrees of essential fatty acid deficiency would impair prostaglandin synthesis, or give rise to the usual clinical features of cystic fibrosis. - Our own more recent research in platelet aggregation, prostaglandin, and cyclic nucleotide production, makes us aware that simple EFA deficiency per se cannot be the origin of the symptoms of CF. The possibility that some other agent in the infusion mixture (which, for example, contains about 12% tocopherol) may be a contributing factor, is also under investigation. This does not, however, invalidate a possible therapeutic effect of EFA in the disease, and in particular, a partial correlation of a possible disorder of prostaglandin synthesis. RF 001 DODGE JA BR MED J 2 192 975 002 ELLIOTT RB ARCH DIS CHILD 50 76 975 003 FLENSBORG EW PROC EWGCF 1ST ANNU MTG 970 004 FORSTROM L PROSTAGLANDINS 7 459 974 005 GRIFFITHS AD ARCH DIS CHILD 47 132 972 006 HANSEN AE PEDIATRICS 31 171 963 PN 75043 RN 00209 AN 75204146 AU Kjellman-N-I. Larsson-Y. TI Insulin release in cystic fibrosis. SO Arch-Dis-Child. 1975 Mar. 50(3). P 205-9. MJ CYSTIC-FIBROSIS: pp. GLUCOSE-TOLERANCE-TEST. INSULIN: se. MN BLOOD-GLUCOSE: an. BODY-HEIGHT. BODY-WEIGHT. CATHETERIZATION. CHILD. CHILD-PRESCHOOL. COLORIMETRY. FEMALE. HUMAN. INFANT. MALE. RADIOIMMUNOASSAY. AB Early insulin response to rapid intravenous injection of glucose was studied in 7 cases of cystic fibrosis aged 8 months to 9 1/2 years. Plasma insulin was measured with a radioimmunological method. Blood glucose values were determined and the glucose disappearance rate (kG) calculated. In all children except the youngest one the early insulin response values were low compared with normal children. The kG-values were normal and correlated neither to the duration of clinical symptoms, nor to the patients' actual clinical condition measured by means of the Shwachman score. The explanation of the decreased insulin response is probably the progressive fibrosis of the pancreas. This may also explain the reported increased incidence of diabetes mellitus in cystic fibrosis. Comparison is made with the condition in pancreatic fibrosis in rabbits, produced through duct ligation. RF 001 BEARDMORE M BR MED J 2 1383 966 002 BROWN RE ARCH PATHOL 92 53 971 003 CARROLL JJ BIOCHEM MED 4 171 971 004 CERASI E ACTA ENDOCRINOL 55 278 967 005 CERASI E ACTA ENDOCRINOL 55 330 967 006 EDSTROM C UMED UNIV MED DISSERTATIONS 10 972 007 VON EULER U SCAND J CLIN LAB INVEST SUPPL 64 62 962 008 GRACEY M MED J AUST 1 212 971 009 HANDWERGER S N ENGL J MED 281 451 969 010 IKKOS D ACTA ENDOCRINOL 25 312 957 011 KAISER G HELV PAEDIATR ACTA 25 135 970 012 LARSSON Y ACTA PAEDIATR SCAND SUPPL 45 106 956 013 LARSSON Y PEDIATRICS 21 893 958 014 MEISSNER H BEITR PATHOL ANAT 114 192 954 015 MILNER AD ARCH DIS CHILD 44 351 969 016 MILUNSKY A AM J DIS CHILD 121 15 971 017 NOSSLIN B LAKARTIDNINGEN 69 5225 972 018 ROSAN RC AM J DIS CHILD 104 625 962 019 SHWACHMAN H AM J DIS CHILD 96 6 958 020 SHWACHMAN H PEDIATRICS 46 335 970 021 STERKY G ACTA PAEDIATR SCAND SUPPL 52 144 963 022 WALPOLE AL BR J PHARMACOL 1 174 946 023 WEBER B ARCH KINDERHEILK SUPPL 65 971 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 3 IANNUCCI A HUM PATHOL 15 278 984 4 GEFFNER ME AM J DIS CHILD 138 677 984 PN 75044 RN 00210 AN 75204201 AU Sarsfield-J-K. Davies-J-M. TI Negative sweat tests and cystic fibrosis. SO Arch-Dis-Child. 1975 Jun. 50(6). P 463-6. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN AMYLASES: an. BICARBONATES: an. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co, bl, ra. FEMALE. HUMAN. LUNG-DISEASES: co. MALE. PNEUMONIA: co. SPUTUM: mi. STAPHYLOCOCCUS. TRYPSIN: an. AB Two brothers are described with chronic suppurative pulmonary disease. One has classical cystic fibrosis with complete pancreatic involvement and abnormal sweat test. The other had incomplete pancreatic disease with repeatedly normal sweat tests. The implications of a negative sweat test in patients with cystic fibrosis are discussed. RF 001 COGSWELL JJ ARCH DIS CHILD 49 520 974 002 DI SANTAGNESE PA JAMA 172 2065 960 003 GIBSON LE J PEDIATR 81 193 972 004 HADORN B CAN MED ASSOC J 98 377 968 005 HADORN B J PEDIATR 73 39 968 006 SHWACHMAN H IN: KENDIG EL JR 1 524 972 CT 1 STERN RC JAMA 239 2676 978 2 HUFF DS J PEDIATR 94 237 979 3 DAVIS PB AM J MED 69 643 980 4 LITTLEWOOD JM PRACTITIONER 224 305 980 5 SCHWACHMAN H J PEDIATR 98 576 981 6 STERN RC LANCET 1 1401 982 7 WONG LTK GUT 23 744 982 8 LITTLEWOOD JM LANCET 1 183 983 9 TAL A CLIN PEDIATR 24 460 985 10 NIELSEN OH CURR THER RES CLIN EXP 41 367 987 PN 75045 RN 00211 AN 75204203 AU Counahan-R. Mearns-M-B. TI Prevalence of atopy and exercise-induced bronchial lability in relatives of patients with cystic fibrosis. SO Arch-Dis-Child. 1975 Jun. 50(6). P 477-81. MJ BRONCHIAL-SPASM: co. CYSTIC-FIBROSIS: fg. HYPERSENSITIVITY: co. MN ADOLESCENCE. ADULT. ALLERGENS. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. CYSTIC-FIBROSIS: co, pp. EXERCISE-TEST. FAMILY. FEMALE. HUMAN. MALE. MIDDLE-AGE. PARENTS. PEAK-EXPIRATORY-FLOW-RATE: mt. SKIN-TESTS. AB Skin tests and exercise tests were performed on children with cystic fibrosis (CF) and first-degree relatives of CF children. Positive skin tests were found in 56% of patients and 19% of relatives, but the prevalence of atopy in CF patients bore no relationship to its prevalence in their relatives. Increased 0ronchial lability, mainly due to bronchoconstriction, was present in 54% of patients and 27% of relatives. The results showed that increased prevalence of bronchial lability, but not atopy, is found in first-degree relatives of CF children, and that the abnormal lability and atopy found in CF children does not indicate a familial allergic background. RF 001 ANDERSON SD THORAX 26 396 971 002 CONNOLLY NM J ASTHMA RES 8 31 970 003 DANES BS LANCET 1 1061 968 004 DAY G ARCH DIS CHILD 48 355 973 005 GIMENO F THORAX 29 16 974 006 HEIMLICH EM J ALLERGY CLIN IMMUNOL 37 103 966 007 JONES RHT BR MED J 2 976 966 008 KONIG P ARCH DIS CHILD 48 513 973 009 KONIG P ARCH DIS CHILD 48 942 973 010 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 011 MEARNS MB LANCET 1 538 967 012 NAIRN JR ARCH DIS CHILD 36 253 961 013 SILVERMAN M ARCH DIS CHILD 47 882 972 014 SPOCK A PEDIATR RES 1 173 967 CT 1 JONES RS ARCH DIS CHILD 50 909 975 2 WARNER JO LANCET 1 990 976 3 TWAROG FJ J ALLERGY CLIN IMMUNOL 59 35 977 4 SILVERMAN M ARCH DIS CHILD 53 873 978 5 MELLIS CM PEDIATRICS 61 446 978 6 MITCHELL I J PEDIATR 93 744 978 7 ANON LANCET 1 708 979 8 BURDON JGW MED J AUST 2 77 980 9 VANASPEREN PP AUST PAEDIATR J 16 53 980 10 HODSON ME THORAX 35 801 980 11 ORMEROD LP THORAX 35 768 980 12 TOBIN MJ THORAX 35 807 980 13 HOLZER FJ ARCH DIS CHILD 56 455 981 14 CLARKE CW BR J DIS CHEST 75 15 981 15 GEORGITIS JW ANN ALLERGY 48 175 982 16 MURPHY MB THORAX 39 179 984 17 BIRX DL ANN ALLERGY 53 124 984 18 WONNE R CLIN ALLERGY 15 455 985 19 MITCHELL I ANN ALLERGY 54 233 985 PN 75046 RN 00212 AN 75204208 AU Barclay-R-P. Shannon-R-S. TI Trial of artificial diet in treatment of cystic fibrosis of pancreas. SO Arch-Dis-Child. 1975 Jun. 50(6). P 490-3. MJ CYSTIC-FIBROSIS: dh. MN BODY-HEIGHT. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. CYSTIC-FIBROSIS: co. FEMALE. GROWTH. HUMAN. INFANT. MALE. RESPIRATORY-TRACT-INFECTIONS: co. EX Twelve patients with cystic fibrosis were started on an artificial diet of Albumaid and Caloreen, together with a supplement of medium chain triglyceride oil, vitamins, and minerals. Growth velocity and general health were assessed over a period of a full year for each child. Patients who were only mildly affected by the disease showed improvement in stool frequency and growth rate when the diet represented the major part of their nutritional intake. Patients who were severely affected showed no improvement in growth, general clinical status, or frequency of respiratory infections. RF 001 ALLAN JD AM J DIS CHILD 126 22 973 002 DARBY CW ARCH DIS CHILD 46 866 971 003 MEARNS MB ARCH DIS CHILD 47 5 972 004 TANNER JM ARCH DIS CHILD 41 613 966 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 SOLOMONS NW AM J CLIN NUTR 34 462 981 3 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 4 OLOUGHLIN E AM J CLIN NUTR 43 732 986 PN 75047 RN 00213 AN 76111670 TI Letter: Management of children with cystic fibrosis. SO Aust-Paediatr-J. 1975 Jun. 11(2). P 89-92. MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mo. FEMALE. HUMAN. INFANT. PHYSICAL-THERAPY. EX Reports of children with cystic fibrosis living well whilst on intensive treatment can be covered by allegations that their disease must be basically mild. More revealing evidence comes from the longterm survival studies done by Professor Warren Warwick, of the University of Minnesota. The introduction of antibiotics have allowed the children to live loner, but 3/4 were dead by aged 10 years. Physiotherapy given after infections prolonged lives so that half were still alive at 10 years - but still faced a relentless death rate. Prophylactic - daily - physiotherapy produced a doubling of survival at 20 years from under 30% to over 60%. I do not deny for a moment that daily physiotherapy with inhalations is a great strain on the family, but it is not as great as the strain of watching the slow and relentless decline of the child which, with racking cough and persistent dyspnoea, is anything but peaceful. RF 001 PHELAN PD AUST PAEDIATR J 9 302 973 002 WARWICK WJ MINN MED 52 1476 969 PN 75048 RN 00214 AN 75183463 AU Bowman-B-H. Lankford-B-J. Fuller-G-M. Carson-S-D. Kurosky-A. Barnett-D-R. TI Cystic fibrosis: the ciliary inhibitor is a small polypeptide associated with immunoglobulin G. SO Biochem-Biophys-Res-Commun. 1975 Jun 16. 64(4). P 1310-5. MJ CILIA: pp. CYSTIC-FIBROSIS: bl. IGG: ph. MN ANIMAL. CYSTIC-FIBROSIS: pp. GILLS: ph. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MOLECULAR-WEIGHT. OYSTERS: ph. PEPTIDES: bl. SUPPORT-U-S-GOVT-P-H-S. AB A small polypeptide within the molecular weight range of 6,000 to 11,000 was found in immunoglobulin fractions from sera of cystic fibrosis homozygotes and heterozygotes. This factor appears to be responsible for interfering with ciliary activity in oyster gills. Since it can be dissociated from IgG without reductive cleavage it must be bound in a non-covalent manner. After its dissociation the IgG fractions from cystic fibrosis sera no longer inhibited ciliary activity. This finding explains the differences previously observed in the molecular weights of the ciliary inhibitor synthesized by cultured cells and that of sera from cystic fibrosis genotypes. 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 BARNETT DR TEX REP BIOL MED 31 709 973 005 HERZBERG V J CLIN INVEST 52 2732 973 006 DANES BS J EXP MED 136 1313 972 007 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 008 CONOVER JH PEDIATR RES 7 224 973 009 BERATIS NG PEDIATR RES 7 958 973 010 BARNETT DR TEX REP BIOL MED 31 703 973 011 BOWMAN BH SCIENCE 167 871 970 012 MCCOMBS ML CLIN GENET 1 171 970 013 PALACIOS R J BIOL CHEM 247 2316 972 014 SWANK RT ANAL BIOCHEM 39 462 971 CT 1 LEE TJ CLIN RES 23 A655 975 2 GILLARD BK PEDIATR RES 10 907 976 3 BOWMAN BH LIFE SCI 19 1289 976 4 BAUR PS TEX REP BIOL MED 34 113 976 5 BAUR PS TEX REP BIOL MED 34 155 976 6 BOWMAN BH TEX REP BIOL MED 34 1 976 7 CARSON SD TEX REP BIOL MED 34 209 976 8 HARPER BL TEX REP BIOL MED 34 73 976 9 WARD JB TEX REP BIOL MED 34 83 976 10 WOOD RE AM REV RESPIR DIS 113 833 976 11 DISANTAGNESE PA N ENGL J MED 295 534 976 12 CONOD EJ PEDIATR RES 11 45 977 13 THOMAS JM PEDIATR RES 11 1148 977 14 YOKOYAMA M PEDIATR RES 11 765 977 15 BOWMAN BH CLIN GENET 12 333 977 16 ROSSMAN CM J PEDIATR 90 579 977 17 CONOVER JH BIOCHEM BIOPHYS RES COMMUN 83 1595 978 18 GUY GJ CLIN CHIM ACTA 87 63 978 19 WILSON GB J LAB CLIN MED 92 463 978 20 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 21 NAGY EC PEDIATR RES 13 729 979 22 PIVETTA OH PEDIATR RES 13 1160 979 23 TUCKER RD PEDIATR RES 13 1371 979 24 STRAUSS RG HELV PAEDIATR ACTA 34 429 979 25 BOWMAN BH TEX REP BIOL MED 38 47 979 26 BOGART BI BIOCHEM BIOPHYS RES COMMUN 88 1398 979 27 KURLANDSKY LE PEDIATR RES 14 1263 980 28 BOWMAN BH FED PROC 39 3195 980 29 MARTINEZ JR PEDIATR RES 15 1439 981 30 SANDERSON MJ PEDIATR RES 15 219 981 31 FLEMING N EXPERIENTIA 37 139 981 32 TEGNER H ACTA PAEDIATR SCAND 70 629 981 33 CARSON SD BIOCHIM BIOPHYS ACTA 667 23 981 34 BLITZER MG PEDIATR RES 16 203 982 35 CARSON SD PEDIATR RES 16 13 982 36 MCNEELY MC PEDIATR RES 16 21 982 37 WOODS DE INFECT IMMUN 36 1223 982 38 BUCHANAN KD SCAND J GASTROENTEROL 18 155 983 39 HERMANS CO OCEANOGR MARINE BIOL 21 283 983 40 SHAPIRA E ANN NY ACAD SCI 421 352 983 41 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 42 BLITZER MG PEDIATR RES 18 540 984 PN 75049 RN 00215 AN 76000549 AU Wilson-J-D. Goldstein-J-L. TI Classification of hereditary disorders of sexual development. SO Birth-Defects. 1975. 11(4). P 1-16. (REVIEW). MJ SEX-DIFFERENTIATION-DISORDERS: cl. MN ANDROGENS: bi, me. CYSTIC-FIBROSIS. FEMALE. GENES. GENITALIA: em. HERMAPHRODITISM. HUMAN. MALE. OVARY: em. PHENOTYPE. PSEUDOHERMAPHRODITISM. REVIEW. SEX-DETERMINATION. SEX-DIFFERENTIATION. SEX-REVERSAL-GONADAL. TESTIS: em, ab. TESTOSTERONE: me. TURNERS-SYNDROME. SUPPORT-U-S-GOVT-P-H-S. UTERUS: ab. VAGINA: ab. EX We have recently proposed a classification of the disorders of sexual development based upon the presumed site of action of the abnormal gene during embryogenesis. These disorders, which may be either genetic or nongenetic in nature, are the result of disturbances in the physiology of sexual differentiation during embryogenesis. The purpose of this review is to summarize the current physiologic concepts of normal sexual differentiation, to review the known hereditary disorders of sexual development, and to consider briefly the implications that these mutations have for the understanding of normal sexual differentiation. The translation of genetic sex into phenotypic sex is a complex process. Since some 19 hereditary, single-gene determined disorders of sexual development are currently recognized that involve loci on both the autosomes and the X chromosome, it can be inferred that at a minimum 19 genes and hence 19 discrete biochemical steps are involved in this process. At least 10 mutations result in male pseudohermaphroditism, whereas only 2 mutations are known to cause female pseudohermaphroditism. The predominance of male type of mutation is the result of the fact that the formation of the male phenotype is actively induced by the secretions of the fetal testis whereas the formation of the female phenotype does not require secretion from the fetal ovary. The end result of these mutations that involve the formation and action of testosterone can be similar, and it requires anatomic, endocrinologic, and genetic study of individual patients to identify the mutant genes involved. Undoubtedly, many more as yet unidentified genes must participate in sexual differentiation. The identification of these genes will depend on the continued delineation of new simply inherited clinical syndromes. Any fundamental understanding of the programming of events in phenotypic differentiation will require not only the identification of the genes involved but the elucidation of the molecular mechanisms by which each of these genes is expressed. RF 001 GOLDSTEIN JL IN: MOTULSKY AG 165 974 002 JOST A ARCH ANAT MICROSC MORPHOL EXP 36 271 946 003 JOST A RECENT PROGR HORM RES 8 379 953 004 JOST A HARVEY LECTURES SER 55 201 959 005 JOST A JOHNS HOPKINS MED J 130 38 972 006 PATTEN BM HUMAN EMBRYOLOGY 953 007 BEATTY RA PHIL TRANS R SOC LOND BIOL 259 3 970 008 WILSON KM CONTRIBUTIONS EMBRYOL 18 25 926 009 GILLMAN J CONTRIBUTIONS EMBRYOL 32 83 948 010 FEDERMAN DD ABNORMAL SEXUAL DEVELOPMENT 967 011 JOSSO N J CLIN ENDOCRINOL METAB 32 404 971 012 JOSSO N J CLIN ENDOCRINOL METAB 34 265 972 013 JOSSO N BIOL NEONATE 20 368 972 014 WILSON JD ENDOCRINOLOGY 92 1182 973 015 SIITERI PK J CLIN ENDOCRINOL METAB 38 113 974 016 WILSON JD ENDOCRINOLOGY 89 659 971 017 WILSON JD ENDOCRINOLOGY 92 1192 973 018 SCHULTZ FM ENDOCRINOLOGY 94 979 974 019 CLAYTON GW J CLIN ENDOCRINOL METAB 18 1349 958 020 MILNER WA J UROL 79 1003 958 021 ROSENBERG HS J CLIN ENDOCRINOL METAB 23 203 963 022 MORI Y JAPAN J UROL 59 10 968 023 HAMERTON JL J REPROD FERTIL SUPPL 7 25 969 024 CATTANACH BM CYTOGENETICS 10 318 971 025 DE LA CHAPELLE A AM J HUM GENET 24 71 972 026 KASDAN R N ENGL J MED 288 539 973 027 BOCZKOWSKI K CLIN GENET 2 379 971 028 JOSSO N BULL SOC MED PARIS ANN PEDIAT 39 775 963 029 GUISTI G ACTA GENET MED GEMELLOL ROMA 15 51 966 030 SIMPSON JL OBSTET GYNECOL SURV 24 580 969 031 CHRISTAKOS AC AM J OBSTET GYNECOL 104 1027 969 032 SIMPSON JL BIRTH DEF ORIG ART SER 7 215 971 033 COHEN MM N ENGL J MED 272 1083 965 034 STERNBERG WH N ENGL J MED 278 695 968 035 CHEMKE J J MED GENET 7 105 970 036 ESPINER EA N ENGL J MED 283 6 970 037$ FEDERMAN DD IN: STEINBERG AG 9 215 973 038 HALL JG BIRTH DEF ORIG ART SER 11 115 975 039 PRADER A HELV PAEDIATR ACTA 10 397 955 040 PRADER A HELV PAEDIATR ACTA 12 569 957 041 CAMACHO AM J CLIN ENDOCRINOL METAB 28 153 968 042 BONGIOVANNI AM J CLIN ENDOCRINOL METAB 21 860 961 043 BONGIOVANNI AM J CLIN INVEST 41 2086 962 044 BONGIOVANNI AM RECENT PROGR HORM RES 23 375 967 045 KOGUT MD AM J DIS CHILD 110 562 965 046 JANNE O J CLIN ENDOCRINOL METAB 31 162 970 047 ZACHMANN M J CLIN ENDOCRINOL METAB 30 719 970 048 BONGIOVANNI AM IN: STANBURY JB 857 972 049 BIGLIERI EG J CLIN INVEST 45 1946 966 050 NEW MI J CLIN ENDOCRINOL METAB 27 300 967 051 MALLIN SR ANN INTERN MED 70 69 969 052 GOLDSMITH O N ENGL J MED 277 673 967 053 MIURA K J CLIN ENDOCRINOL METAB 28 1807 968 054 LINQUETTE M ANN ENDOCRINOL PARIS 32 574 971 055 MANTERO F SCHWEIZ MED WOCHENSCHR 101 38 971 056 NEW MI J CLIN INVEST 49 1930 970 057 BRICAIRE H J CLIN ENDOCRINOL METAB 35 67 972 058 ALVAREZ MN PEDIATR RES 7 325 973 059 ZACHMANN M ACTA ENDOCRINOL SUPPL 155 65 971 060 ZACHMANN M CLIN ENDOCRINOL TOKYO 1 369 972 061 SAEZ JM J CLIN ENDOCRINOL METAB 32 604 971 062 SAEZ JM J CLIN ENDOCRINOL METAB 34 598 972 063 GOEBELSMANN U J CLIN ENDOCRINOL METAB 36 867 973 064 MORRIS JM AM J OBSTET GYNECOL 65 1192 953 065 MORRIS JM AM J OBSTET GYNECOL 87 731 963 066 HAUSER GA IN: OVERZIER C 255 963 067 SOUTHREN AL IN: LEVINE R 2 227 965 068 WILKINS L DIAG TREATMENT ENDOCRINE DISO 278 957 069 SOUTHREN AL J CLIN ENDOCRINOL METAB 25 518 965 070 JUDD HL J CLIN ENDOCRINOL METAB 34 229 972 071 WILSON JD N ENGL J MED 290 1097 974 072 GOLDSTEIN JL J CLIN INVEST 51 1647 972 073 WINTERBORN MH ARCH DIS CHILD 45 811 970 074 PHILIP J AM J OBSTET GYNECOL 93 1076 965 075 KHOO SK AUST NZ J OBSTET GYNAECOL 12 1 972 076 ROSENFIELD RL J CLIN ENDOCRINOL METAB 32 625 971 077 LUBS HA J CLIN ENDOCRINOL METAB 19 1110 959 078 GILBERT-DREYFUS S ANN ENDOCRINOL PARIS 18 93 957 079 REIFENSTEIN EC CLIN RES 3 86 947 080 BOWEN P ANN INTERN MED 62 252 965 081 BOCZKOWSKI K AM J OBSTET GYNECOL 112 192 972 082 ROSEWATER S ANN INTERN MED 63 377 965 083 WALKER AC MED J AUST 1 156 970 084 RIMOIN DL GENET DISORDERS ENDOCRINE 279 971 085 SIMPSON JL BIRTH DEF ORIG ART SER 7 140 971 086 OPITZ JM CLIN GENET 3 1 972 087 IMPERATO-MCGINLEY J J CLIN INVEST 53 35A 974 089 NILSEN O ACTA CHIR SCAND 83 231 939 090 MORILLO-CUCCI G BIRTH DEF ORIG ART SER 7 229 971 091 DAVID L ACTA PAEDIATR SCAND 61 249 972 092 BROOK CGD BR MED J 1 771 973 093 GUELL-GONZALEZ JR REV ROUN ENDOCRINOL 7 343 970 094 BRYAN AL SURG GYNECOL OBSTET 88 79 949 095 WHARTON LR AM J OBSTET GYNECOL 53 37 947 096 BLOCH P GYNAECOLOGIA 151 113 961 097 HAUSER GA GYNAECOLOGIA 151 111 961 098 LEDUC B AM J OBSTET GYNECOL 100 512 968 099 HAUSER GA SCHWEIZ MED WOCHENSCHR 91 381 961 100 VAN CAMPENHOUT J LANCET 2 928 971 101 ANGER D BULL FED SOC GYNECOL OBSTET L 18 229 966 102 JONES HW JR AM J OBSTET GYNECOL 114 1100 972 103 HOLSCLAW DS J UROL 106 568 971 104 KAPLAN E N ENGL J MED 279 65 968 CT 1 SCHWEIKERT HU J CLIN ENDOCRINOL METAB 43 785 976 2 GRIFFIN JE J CLIN INVEST 57 1342 976 3 MOREIRAFILHO CA REV BRASIL PESQUISAS MED BIOL 10 345 977 4 GRIFFIN JE J CLIN ENDOCRINOL METAB 45 1137 977 5 SIMPSON JL AM J OBSTET GYNECOL 128 137 977 6 KOO GC SCIENCE 196 655 977 7 GRIFFIN JE CLIN OBSTET GYNAECOL 5 457 978 8 WILSON JD ANNU REV PHYSIOL 40 279 978 9 SIMPSON JL HUM GENET 44 1 978 10 WEISS EB PEDIATRICS 61 797 978 11 RAJFER J J UROL 119 525 978 12 BERNSTEIN R J MED GENET 17 291 980 13 GRIFFIN JE N ENGL J MED 302 198 980 14 WILSON JD SCIENCE 211 1278 981 15 GRIFFIN JE AM J PHYSIOL 243 E 81 982 16 WILSON JD AUST J BIOL SCI 36 101 983 17 PEREIRA RR HELV PAEDIATR ACTA 39 255 984 18 SAENGER P J PEDIATR 104 1 984 19 WILSON JD HARVEY LECTURES 79 145 985 20 GREENE RA HUM PATHOL 17 88 986 21 PAGE DC COLD SPRING HARBOR SYMP Q BIO 51 229 986 22 GYORKI S J STEROID BIOCHEM 26 7 987 PN 75050 RN 00216 AN 76000814 AU Zaidi-Z-H. TI Letter: Serum alpha-fetoprotein in cystic fibrosis. SO Br-Med-J. 1975 Aug 23. 3(5981). P 491. MJ CYSTIC-FIBROSIS: di. MN ALPHA-FETOPROTEINS: an. CARRIER-STATE: di. CYSTIC-FIBROSIS: fg. FEMALE. HETEROZYGOTE. HUMAN. MALE. EX Professor R. K. Chandra and others suggest that estimation of serum alpha-fetoprotein (AFP) might help in detecting carriers in families at risk for cystic fibrosis. Observers in the U.K. have been unable to confirm these findings, whereas Dr. J. A. Smith of New York supports them. Perhaps the crux of the matter lies in the technique of estimating serum AFP. if a small number of families is analysed the proportion of affected individuals may differ from the expected. By chance some families may have all normal children whereas some may have all affected children. For example, nowadays, since families tend to be small, the appearance of an autosomal recessive condition is often sporadic with only one affected person in the family. This could well apply to cystic fibrosis. RF 001 CHANDRA RK BR MED J 1 714 975 002 SMITH JA BR MED J 2 392 975 003 DANKS DM ANN HUM GENET 28 323 965 004 EMERY AEH IN: FORFAR JO 66 973 005 CARTER CO IN: HARRIS M 22 970 PN 75051 RN 00217 AN 76019895 AU Fitzsimmons-J-S. Smith-N. Hiller-E-J. Wynne-J. TI Letter: Serum alpha-fetoprotein in cystic fibrosis. SO Br-Med-J. 1975 Aug 30. 3(5982). P 544. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HUMAN. EX Professor R. K. Chandra and others found raised serum levels of alpha-fetoprotein (AFP) in a heterozygote carrier of the gene for cystic fibrosis and in patients with the disease and some of the siblings. Our results do not show any significant difference in AFP levels in patients, siblings, parents, or control children. Probably the discrepancy in the findings will be explained by more detailed examination of the technique used. RF 001 CHANDRA RK BR MED J 1 714 975 002 BROCK DJH BR MED J 2 392 975 003 ADINOLFI A J MED GENET 12 138 975 CT 1 BELANGER L BR MED J 4 759 975 2 BISWAS S CLIN CHIM ACTA 69 541 976 3 KNOPFLE G EUR J PEDIATR 122 241 976 4 KNOPFLE G KLIN PAEDIATR 189 207 977 5 ALHADEFF JA CLIN GENET 14 189 978 6 BROCK DJH CLIN CHIM ACTA 82 101 978 7 ADINOLFI M ADV HUM GENET 9 165 979 PN 75052 RN 00218 AN 76063497 AU Knopple-G. Rotthauwe-H-W. Lehmann-F-G. TI Letter: Serum alpha-fetoprotein in cystic fibrosis. SO Br-Med-J. 1975 Nov 22. 4(5994). P 459. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg. HUMAN. INFANT. EX Professor R. K. Chandra and others reported significantly increased levels of alpha-fetoprotein (AFP) in the serum of patients with cystic fibrosis (CF), in the parents of the patients, and in some of their siblings in Newfoundland, Canada. The serum AFP levels in our 38 patients with CF were not different from those in healthy adults. In our opinion, genetic differences cannot explain the different results concerning the serum AFP levels in patients with CF. RF 001 CHANDRA RK BR MED J 1 714 975 002 SMITH JA BR MED J 2 392 975 003 BROCK DJH BR MED J 2 392 975 004 NISHI S GANN MOGR CANCER RES 14 79 973 005 ISHII M GANN MOGR CANCER RES 14 89 973 006 LEHMANN FG CLIN CHIM ACTA 33 197 971 007 LEHMANN FG Z KLIN CHEM KLIN BIOCHEM 9 309 971 008 MANCINI G COLLOQ PROTIDES BIOL FLUIDS 11 370 965 009 LEHMANN FG Z KLIN CHEM KLIN BIOCHEM 11 399 973 010 SIZARET P J BIOL STANDARDS 3 201 975 CT 1 BISWAS S CLIN CHIM ACTA 69 541 976 2 KNOPFLE G EUR J PEDIATR 122 241 976 3 KNOPFLE G KLIN PAEDIATR 189 207 977 4 ALHADEFF JA CLIN GENET 14 189 978 PN 75053 RN 00219 AN 75165793 TI Letter: Serum alpha-fetoprotein in cystic fibrosis. SO Br-Med-J. 1975 May 17. 2(5967). P 392. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN CHILD. COUNSELING. CYSTIC-FIBROSIS: fg. HUMAN. RADIOIMMUNOASSAY. EX Over the past 12 months we have examined by both counterimmunoelectrophoresis and radioimmunoassay sera from (a) 37 patients with cystic fibrosis (age 6 months to 17 years), (b) 10 of their siblings, and (c) 24 of their parents for the presence of raised serum levels of AFP. By counterimmunoelectrophoresis we were unable to detect an increased serum concentration of AFP in any of the cystic fibrosis patients or their relatives. In addition, the results from the radioimmunoassay method showed that all of the cystic fibrosis patients and their relatives had normal concentrations of AFP in their serum. - Using a double antibody radioimmunoassay we found the mean serum AFP for seven children with cystic fibrosis to be little different from that of controls, while all seven values fell within the normal range for this series. - In seven patients considered to have cystic fibrosis on the basis of a sweat chloride level above 65 mmol/l the mean serum alpha-fetoprotein (AFP level was 1825 (range 130 - 2360) microg/l. The corresponding level in the patients was 433 (range 80 - 1055) microg/l. Drug therapy given to these patients may have a subtle hepatoxic influence, thereby causing a rise in AFP levels. The severity of the disease does not influence the degree of elevation. There is a corresponding rise in carcinoembryonic antigen levels also, but the latter is related to the degree of tissue damage and results from the sero-cross-reactivity between mucus-producing respiratory mucosa and fetal tissues. RF 001 CHANDRA RK BR MED J 1 714 975 002 BROCK DJH LANCET 1 767 974 003 RUOSLAHTI E NATURE 235 161 972 CT 1 FITZSIMMONS JS BR MED J 3 544 975 2 ZAIDI ZH BR MED J 3 491 975 3 BELANGER L BR MED J 4 759 975 4 BELANGER L BR MED J 4 759 975 5 KNOPFLE G BR MED J 4 459 975 6 KNOPFLE G BR MED J 4 459 975 7 GRENIER A CLIN CHEM 22 1001 976 8 BISWAS S CLIN CHIM ACTA 69 541 976 9 BISWAS S CLIN CHIM ACTA 69 541 976 10 KNOPFLE G EUR J PEDIATR 122 241 976 11 KNOPFLE G EUR J PEDIATR 122 241 976 12 DISANTAGNESE PA N ENGL J MED 295 597 976 13 DISANTAGNESE PA N ENGL J MED 295 597 976 14 RAMIREZ P N ENGL J MED 295 1381 976 15 CHANDRA RK INT ARCH ALLERGY APPL IMMUNOL 53 180 977 16 KNOPFLE G KLIN PAEDIATR 189 207 977 17 ALHADEFF JA CLIN GENET 14 189 978 18 ALHADEFF JA CLIN GENET 14 189 978 19 BROCK DJH CLIN CHIM ACTA 82 101 978 20 MIZEJEWSKI GJ PEDIATR RES 17 47 983 PN 75054 RN 00220 AN 75147772 AU Dodge-J-A. Salter-D-G. Yassa-J-G. TI Letter: Essential fatty acid deficiency due to artificial diet in cystic fibrosis. SO Br-Med-J. 1975 Apr 26. 2(5964). P 192-3. MJ CYSTIC-FIBROSIS: dh. FATTY-ACIDS-ESSENTIAL: df. INFANT-NUTRITION. MN CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. NUTRITIONAL-REQUIREMENTS. SKIN-MANIFESTATIONS. EX We wish to draw the attention of your readers to a possible hazard of feeding infants with an elemental diet. Considerable interest is currently being shown in the use of the artificial diet in cystic fibrosis. It may be used as a supplement to ordinary food or to supply all the nutritional needs of the child. In the latter event we believe that it is particularly important to emphasize to the parents the need for an additional source of dietary fat. Clinical evidence and animal experiments suggest that infants, because of their rapid growth rate, are particularly at risk and that in the absence of dietary E.F.A. they will develop deficiency symptoms in about two months. RF 001 ALLAN JD AM J DIS CHILD 126 22 973 002 HANSEN AE PEDIATRICS 31 171 963 003 WARWICK WJ AM J DIS CHILD 98 317 959 004 PAULSRUD JR AM J CLIN NUTR 25 897 972 005 CALDWELL MD J PEDIATR 81 894 972 006 COLLINS FD NUTR METAB 13 150 971 007 PRESS M BR MED J 2 247 974 008 ELLIOTT RB ARCH DIS CHILD 50 76 975 009 LOVE WC BIOCHEM SOC TRANS 1 141 973 CT 1 RIVERS JPW LANCET 2 642 975 2 DODGE JA ARCH DIS CHILD 50 578 975 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 OHALLORAN ET IR MED J 71 184 978 5 DODGE JA LANCET 2 1256 980 6 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 7 SOLOMONS NW AM J CLIN NUTR 34 462 981 8 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 9 PARSONS HG BIOCHIM BIOPHYS ACTA 860 420 986 PN 75055 RN 00221 AN 75147682 AU Chandra-R-K. Madhavankutty-K. Way-R-C. TI Serum alpha - fetoprotein levels in patients with cystic fibrosis and their parents and siblings. SO Br-Med-J. 1975 Mar 29. 1(5960). P 714-6. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN BRONCHIECTASIS: bl. CELIAC-DISEASE: bl. CHLORIDES: an. CYSTIC-FIBROSIS: fg, me. GEL-DIFFUSION-TESTS. GENETIC-COUNSELING. HETEROZYGOTE. HUMAN. IMMUNOELECTROPHORESIS. INFANT. LIVER: me. SWEAT: an. AB Patients with cystic fibrosis (C.F.) showed raised serum levels of alpha-fetoprotein (AFP). A moderate but significant increase in serum AFP was present in their parents and some siblings. There was no correlation between the clinical severity of the disease and serum AFP concentration. Samples from control groups with gluten-induced malabsorption and bronchiectasis had normal levels. Persistent synthesis of AFP may be an associated marker of C.F. genes, and estimation of serum AFP might help in detecting heterozygote carriers in families at risk. RF 001 ABELEV GI ADV CANCER RES 14 295 971 002 ALLAN LD LANCET 2 522 973 003 BESLEY GTN J MED GENET 6 278 969 004 BROCK DJH LANCET 2 197 972 005 BROCK DJH LANCET 2 923 973 006 BRUNECKY Z J MED GENET 9 33 972 007 CHANDRA RK ARCH DIS CHILD 48 157 973 008 CHANDRA RK MED CHIR DIG 3 63 974 010 CHERRY JD J PEDIATR 79 937 971 011 DANES BS LANCET 1 1061 968 012 DANES BS J EXP MED 129 775 969 013 DANKS DM ANN HUM GENET 28 323 965 014 GITLIN D J CLIN INVEST 45 1826 966 015 LEEK AE LANCET 2 385 973 016 LOCKHART LH SOUTH MED J 61 1356 968 017 MASOPUST J INT J CANCER 3 364 968 018 NAYAK NC LANCET 1 86 972 019 RENNERT OM CLIN PEDIATR 11 351 972 020 RUOSLAHTI E INT J CANCER 8 374 971 021 SPOCK A PEDIATR RES 1 173 967 022 WALDMANN TA LANCET 2 1112 972 023 WRIGHT SW HAWAII MED J 27 229 968 CT 1 BROCK DJH BR MED J 2 392 975 2 SMITH JA BR MED J 2 392 975 3 WALLWORK JC BR MED J 2 392 975 4 FITZSIMMONS JS BR MED J 3 544 975 5 ZAIDI ZH BR MED J 3 491 975 6 BELANGER L BR MED J 4 759 975 7 KNOPFLE G BR MED J 4 459 975 8 LEHMANN FG LEBER MAGEN DARM 5 144 975 9 ADINOLFI A J MED GENET 12 138 975 10 BROCK DJH CHILDS BRAIN 2 1 976 11 BERNAUDIN F NOUV PRESSE MED 5 643 976 12 GRENIER A CLIN CHEM 22 1001 976 13 LUCKING T KLIN WSCHR 54 391 976 14 BISWAS S CLIN CHIM ACTA 69 541 976 15 NORGAARDPEDERSEN B CLIN CHIM ACTA 71 343 976 16 KNOPFLE G EUR J PEDIATR 122 241 976 17 DISANTAGNESE PA N ENGL J MED 295 597 976 18 RAMIREZ P N ENGL J MED 295 1381 976 19 NORGAARDPEDERSEN B SCAND J IMMUNOL 1976 7 976 20 CRUIKSHANK DP SEM PERINATOL 1 135 977 21 BROCK DJH PROG MED GENET 2 1 977 22 SIZARET P DIGESTION 15 97 977 23 CHANDRA RK INT ARCH ALLERGY APPL IMMUNOL 53 180 977 24 OLSSON M J EXP MED 145 819 977 25 KNOPFLE G KLIN PAEDIATR 189 207 977 26 DELPRE G GASTROENTEROL CLIN BIOL 2 193 978 27 ALHADEFF JA CLIN GENET 14 189 978 28 BROCK DJH CLIN CHIM ACTA 82 101 978 29 ADINOLFI M ADV HUM GENET 9 165 979 30 BROCK DJH MOL ASP MED 3 431 980 31 BOYE NP EUR J RESPIR DIS 61 227 980 32 SCAMBLER P HUM GENET 69 250 985 PN 75056 RN 00222 AN 76115710 AU Belanger-L. Hamel-D. Aubin-G. Belanger-M. Bigonesse-P. Dorval-J. Sell-S. Stillman-D. Skelly-H. Scott-J. Harwood-I. Olmsted-N. TI Letter: Serum alpha-fetoprotein in cystic fibrosis. SO Br-Med-J. 1975 Dec 27. 4(5999). P 759. MJ ALPHA-FETOPROTEINS: an. CYSTIC-FIBROSIS: bl. FETAL-PROTEINS: an. MN HUMAN. EX Sera from 40 well-confirmed cystic fibrosis patients and 60 of their parents were obtained through two Canadian (Chicoutimi and Quebec) and one United States (San Diego) cystic fibrosis clinics. AFP was measured by two methods: a double antibody enzyme immunoassay and a sodium sulphate precipitation radioimmunoassay. Our results thus confirm that serum AFP levels are normal in cystic fibrosis. RF 001 CHANDRA RK BR MED J 1 714 975 002 WALLWORK JC BR MED J 2 392 975 003 BROCK DJH BR MED J 2 392 975 004 FITZSIMMONS JS BR MED J 3 544 975 005 SMITH JA BR MED J 2 392 975 007 SELL S IMMUNOCHEMISTRY 10 439 973 CT 1 GRENIER A CLIN CHEM 22 1001 976 2 KNOPFLE G EUR J PEDIATR 122 241 976 3 KNOPFLE G KLIN PAEDIATR 189 207 977 4 BROCK DJH CLIN CHIM ACTA 82 101 978 PN 75057 RN 00223 AN 75110215 AU McFarlane-H. Holzel-A. Brenchley-P. Allan-J-D. Wallwork-J-C. Singer-B-E. Worsley-B. TI Immune complexes in cystic fibrosis. SO Br-Med-J. 1975 Feb 22. 1(5955). P 423-8. MJ CYSTIC-FIBROSIS: im. MN ADSORPTION. ADULT. ANTIBODIES. ANTIGEN-ANTIBODY-COMPLEX: an. AUTORADIOGRAPHY. CHILD. CHILD-PRESCHOOL. COMPLEMENT: an. FEMALE. FLUORESCENT-ANTIBODY-TECHNIC. GASTROINTESTINAL-SYSTEM: im. CHROMATOGRAPHY-GEL. HEMOLYSINS: ip. HUMAN. IGA. IGG: ip. IGM. IMMUNOELECTROPHORESIS. INFANT. LUNG: im. MALE. PRECIPITIN-TESTS. RESPIRATORY-SYSTEM: im. SERUM-ALBUMIN-BOVINE: im. SPUTUM: im. STAPHYLOCOCCUS. AB Circulating immune complexes were detected in serum and sputum of patients with cystic fibrosis (C.F.). There were extensive deposits of immunoglobulins and complement immune complexes in several of the C.F. organs, especially the respiratory and gastrointestinal tracts, but not in the kidneys. Significant concentrations of IgG and of complement complexes could be eluted from the lungs of the C.F. patients but not from those of controls. Studies involving immunoabsorption, autoradiography, and molecular sieving through Sephadex G-200 columns identified both bovine serum albumin and staphylococcal alpha-haemolysin as two of the antigens present in the immune complexes. The sedimentation constant of the immune complexes was about 8S to 11S. The clinical significance of these immune complexes and the wide variety of antibodies detected in C.F. patients are discussed. RF 001 ALLISON AC LANCET 1 1232 969 002 BURNS MW LANCET 1 354 967 003 CRACCO G PROC EWGCF 5TH ANNU MTG 16 974 004 DIXON FJ HARVEY LECTURES 58 21 963 005 GRABAR P IN: GRABAR P 3 964 006 GULCHARD Y PROC EWGCF 5TH ANNU MTG 974 007 JOHANSSON SGO IMMUNOLOGY 10 265 968 008 KILBURN KH IN: MANGOS JA 129 973 009 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 010 MANCINI G IMMUNOCHEMISTRY 2 235 965 011 MERCOLA KE EXP MOL PATH 19 230 973 012 MURRAY MJ ANN INTERN MED 53 548 960 013 PEPYS J IN: SERAFINI U 136 971 014 SOOTHILL JF CLIN EXP IMMUNOL 2 83 967 015 SOOTHILL JF LANCET 2 929 967 016 STEIN AA J PEDIATR 65 495 964 017 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 018 WARREN CPW AM REV RESPIR DIS 109 672 974 CT 1 ALLAN JD CLIN ALLERGY 5 255 975 2 GIBBONS A BR MED J 1 120 976 3 MCFARLANE H LANCET 1 1241 976 4 WALLWORK JC CLIN ALLERGY 6 349 976 5 STEPHAN U INTERNIST 17 336 976 6 VISCONTI A ANN SCLAVO 18 308 976 7 WEEKE B DAN MED BULL 23 155 976 8 CLARKE CW THORAX 31 702 976 9 ANON NUTR REV 34 210 976 10 GALANT SP AM REV RESPIR DIS 114 325 976 11 DISANTAGNESE PA N ENGL J MED 295 597 976 12 GENIN C ARCH FR PEDIATR 34 717 977 13 STRUNK RC ARCH DIS CHILD 52 687 977 14 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 15 ZUBLER RH PROG ALLERGY 24 1 978 16 HILTON AM CLIN EXP IMMUNOL 31 237 978 17 SMITH WR CHEST 73 471 978 18 GOTZ M EUR J PEDIATR 127 133 978 19 HOIBY N MONOGR PAEDIATR 10 138 979 20 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 21 HOWIE AD SCOTT MED J 24 193 979 22 HOIBY N ACTA PAEDIATR SCAND 68 495 979 23 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 24 SOTER NA J PEDIATR 95 197 979 25 BERDISCHEWSKY M PEDIATR RES 14 830 980 26 HODSON ME THORAX 35 801 980 27 CARR RI CLIN EXP IMMUNOL 39 562 980 28 MIELIVERGANI G ARCH DIS CHILD 55 696 980 29 MOSS RB AM REV RESPIR DIS 121 23 980 30 MATTHEWS WJ N ENGL J MED 302 245 980 31 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 32 FICK RB CLIN CHEST MED 2 91 981 33 HOLLAND EJ J CLIN LAB IMMUNOL 6 137 981 34 PENNINGTON JE J CLIN INVEST 68 1140 981 35 CHURCH JA CHEST 80 405 981 36 MARKS MI J PEDIATR 98 173 981 37 MOSS RB J PEDIATR 99 215 981 38 DANIELE RP AM REV RESPIR DIS 124 738 981 39 FROLAND SS SCAND J INFECT DIS 1981 72 981 40 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 41 ABRAMOWSKY CR HUM PATHOL 13 934 982 42 MARGOLIES R PEDIATR RES 16 181 982 43 MOSS RB CLIN EXP IMMUNOL 47 301 982 44 PITCHERWILMOTT RW ARCH DIS CHILD 57 577 982 45 MANTHEI U AM REV RESPIR DIS 126 253 982 46 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 47 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 48 ANON LANCET 2 257 983 49 MARGOLIES R PEDIATR RES 17 931 983 50 BRYAN LE J CLIN MICROBIOL 18 276 983 51 KLINGER JD INFECT IMMUN 39 1377 983 52 WALLACE JM AM REV RESPIR DIS 128 1077 983 53 DORING G J INFECT DIS 147 744 983 54 DAVIS CA HUM PATHOL 15 244 984 55 BOSSO JA DRUG INTEL CLIN PHARM 18 825 984 56 SPEERT DP PEDIATR RES 18 431 984 57 HODSON ME POSTGRAD MED J 60 225 984 58 DORING G ACTA PATH MICROB IMMU SCA (C) 92 307 984 59 WHEELER WB J PEDIATR 104 695 984 60 MICHALSEN H PATHOL RES PRACT 178 261 984 61 AUERBACH HS LANCET 2 686 985 62 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 63 MISCHLER EH SEM RESPIR MED 6 271 985 64 HODSON ME CLIN ALLERGY 15 363 985 65 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 66 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 67 PIER GB J INFECT DIS 151 575 985 68 WOODS DE J INFECT DIS 151 581 985 69 DISIS ML PEDIATR RES 20 385 986 70 HOIBY N ANNU REV MICROBIOL 40 29 986 71 PHILLIPS BM J ROY SOC MED 79 44 986 72 MOSS RB AM REV RESPIR DIS 133 648 986 73 BOSSO JA PEDIATR INFECT DIS J 6 393 987 74 DASGUPTA MK J CLIN IMMUNOL 7 51 987 PN 75058 RN 00224 AN 75147969 AU Blenkarn-G-D. Lanning-C-F. Kylstra-J-A. TI Anaesthetic management of volume controlled unilateral lung lavage. SO Can-Anaesth-Soc-J. 1975 Mar. 22(2). P 154-63. MJ ANESTHESIA-GENERAL: mt. CYSTIC-FIBROSIS: th. DIAZEPAM. HALOTHANE. IRRIGATION: mt. PULMONARY-ALVEOLAR-PROTEINOSIS: th. MN ANESTHESIA-INTRATRACHEAL: mt. ANESTHESIA-INTRAVENOUS: mt. BLOOD-PRESSURE: de. CARBON-DIOXIDE: bl. COMPARATIVE-STUDY. DIAZEPAM: ad. HALOTHANE: ad. HEART-RATE: de. HUMAN. HYDROGEN-ION-CONCENTRATION. IRRIGATION: ae. KETAMINE: ad. OXYGEN-CONSUMPTION. OXYGEN: bl. THIOPENTAL: ad. AB Conventional means to facilitate clearance of inspissated material obstructing gas exchange in the alveoli and finer airways of patients with alveolar proteinosis, asthma, and mucoviscidosis, are often not very effective. The mechanical removal of such materials by volume controlled unilateral lung lavage with saline has been of benefit in selected patients refractory to conventional therapy (particularly those with alveolar proteinosis or accidentally inhaled radioactive particles). This report describes the anaesthetic and lavage techniques currently used in managing patients with alveolar proteinosis and mucoviscidosis and in whom lung lavage repeated at approximately six-month intervals has resulted in both subjective and objective improvements. In 14 lavages in four different patients (10 lavages in two patients with alveolar proteinosis and four lavages in two patients with mucoviscidosis) certain physiologic responses to lung lavage, particularly those related to oxygen transport, were measured quantitatively in an attempt to evaluate and to optimize anaesthetic and lavage techniques. The maintenance anaesthetic during lavage was selected randomly, so that each patient received light anaesthesia with ketamine/diazepam during one lavage and light halothane anaesthesia during lavage of the contralateral lung. RF 001 KYLSTRA JA AM REV RESPIR DIS 103 651 971 002 RAMIREZ RJ DIS CHEST 50 581 966 003 ROGERS RM MED CLIN NORTH AM 54 755 970 004 MCCLELLAN RO ANN REP FISSION PRODUCT INHAL 287 972 005 LYNCH CC ANESTHESIOLOGY 18 138 957 006$ KELMAN GR J APPL PHYSIOL 24 1375 966 007 WASSERMAN K AM J MED 44 611 968 008 SMITH JD ANESTHESIOLOGY 33 401 970 009 ROGERS RM N ENGL J MED 286 1230 972 010 PERMUTT S J APPL PHYSIOL 18 924 963 011 COPPEL D ANAESTHESIA 28 293 973 012 PRICE HL J APPL PHYSIOL 6 517 954 013 MEAD J J APPL PHYSIOL 22 95 967 CT 1 BRADFIELD HGC ANAESTHESIA 34 1032 979 2 ALFERY DD ANESTHESIOLOGY 55 376 981 3 ROTHMANN BF ANN OTOL RHINOL LARYNGOL 91 641 982 4 KARIMAN K LUNG 162 223 984 5 HUDES ET CAN ANAESTH SOC J 33 662 986 PN 75059 RN 00225 AN 76001068 AU Forstner-G. Crozier-D-N. Sturgess-J-M. TI Cystic fibrosis: present status and future prospects in detection of patients and carriers. SO Can-Med-Assoc-J. 1975 Sep 20. 113(6). P 550-6. MJ CARRIER-STATE: di. CYSTIC-FIBROSIS: di. MN BIOLOGICAL-ASSAY. BLOOD-PROTEINS: an. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg, bl. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MALE. MECONIUM: an. PREGNANCY. PRENATAL-DIAGNOSIS. SWEAT: an. AB Development of a sensitive, easily performed, reliable test would be an important advance in detecting cystic fibrosis, improving genetic counselling and providing early effective treatment. The sweat chloride test, which is reliable in diagnosis, is technically too difficult for a screening program, and only reliably detects homozygotes. In contrast, the meconium test for detecting homozygote newborns is simple, inexpensive, reasonably specific but its general application has yet to be evaluated. Detection of serum components is the basis of two new tests to distinguish patients with cystic fibrosis and carriers. The effect of these serum components on ciliary activity is the principle of one test, an extremely difficult procedure that is subjective and lacks sufficient specificity for routine use. The second test, in which serum components are separated by isoelectric focusing, may provide an objective biochemical means of detecting both homozygotes and heterozygotes. RF 001 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 002 SULTZ HA AM J PUBLIC HEALTH 56 1461 966 003 MERRITT AD J LAB CLIN MED 60 998 962 004 MILUNSKY A ANNU REV MED 23 57 972 005 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 006 SPROUL A J PEDIATR 69 759 966 007 DI SANTAGNESE PA N ENGL J MED 277 1287 967 008 STRACHAN PG ANN R COLL PHYS SURG CAN 3 23 970 009 SARLES H GUT 6 545 965 010 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 011 MACLEAN WC JR J PEDIATR 83 86 973 012 FLEISHER DS J PEDIATR 64 349 964 013 VINK CLJ IN: DE REUCK AVS 310 962 014 SEKELJ P AM REV RESPIR DIS 108 603 973 015 SCHWARZ V ARCH DIS CHILD 43 695 968 016 GIBSON LE PEDIATRICS 23 545 959 017 MAUER AM AM J DIS CHILD 92 160 956 018 BUCHANAN DJ PEDIATRICS 9 304 952 019 SCHACHTER H CAN J BIOCHEM 43 381 965 020 SCHUTT WH ARCH DIS CHILD 43 178 968 021 HELLSING K SCAND J CLIN LAB INVEST 34 333 974 022 STEPHAN U KLIN PAEDIATR 186 79 974 023 STEPHAN U CF CLUB ABST 15 974 024 DI SANTAGNESE PA PEDIATRICS 24 313 959 025 SPOCK A PEDIATR RES 1 173 967 026 BOWMAN BH SCIENCE 164 325 969 027 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 028 BARNETT DR TEX REP BIOL MED 31 703 973 029 DANES BS J EXP MED 137 1538 973 030 CONOVER JH LIFE SCI 14 253 974 031 BARNETT DR TEX REP BIOL MED 31 697 973 032 BARNETT DR TEX REP BIOL MED 31 691 973 033 BOWMAN BH CLIN GENET 4 461 973 034 CONOVER JH LANCET 1 1194 973 035 WOOD RE LANCET 2 1452 973 036 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 CZEGLEDYNAGY E LAB INVEST 35 588 976 2 FORSTNER G CLIN CHIM ACTA 70 459 976 3 GABRIDGE MG PEDIATR RES 13 31 979 4 BELL L J CAN DIET ASSOC 42 62 981 PN 75060 RN 00226 AN 76064099 AU Marcotte-A-A. TI Cystic fibrosis. SO Can-Nurse. 1975 Jul. 71(7). P 33-7. MJ CYSTIC-FIBROSIS. MN CASE-REPORT. CHILD. CYSTIC-FIBROSIS: th. FEMALE. HUMAN. PATIENT-CARE-PLANNING. PATIENT-CARE-TEAM. AB Cystic fibrosis, whether it is called fibrocystic disease of the pancreas, mucoviscidosis, of simply C.F., is a condition that currently afflicts one in every 2,500 children. A glimpse of this disease is gained through the experience of Amelia, one of its victims. RF 001 GARDNER LI ENDOCRINE GENET DIS CHILDH 991 969 002 BELMONTE MM CF MOST SERIOUS LUNG PROB CAN 1 003 BELMONTE MM FIBROSE KYSTIQUE UN MANUEL A 968 004 BELMONTE MM FIBROSE KYSTIQUE UN MANUEL A 968 005 STEINSCHNEIDER R SOINS ET OBSERVATIONS 18 25 973 006 STEINSCHNEIDER R SOINS ET OBSERVATIONS 18 25 973 007 BELMONTE MM FIBROSE KYSTIQUE UN MANUEL A 968 008 GILLY R ANN PEDIATR (PARIS) 20 5 973 009 TROPAUER A AM J DIS CHILD 119 424 970 010 TROPAUER A AM J DIS CHILD 119 424 970 011 MCCOLLUM AT J PEDIATR 77 571 970 012 BELMONTE MM UNION MED CAN 98 1944 969 PN 75061 RN 00227 AN 76002502 AU Lober-C-W. Saltzman-H-A. Kylstra-J-A. TI Volume-controlled lung lavage in a woman with cystic fibrosis. SO Chest. 1975 Sep. 68(3). P 382-3. MJ CYSTIC-FIBROSIS: th. IRRIGATION. LUNG. MN ADULT. CASE-REPORT. FEMALE. HUMAN. REMISSION-SPONTANEOUS. RESPIRATORY-FUNCTION-TESTS. AB A 22-year-old woman with cystic fibrosis was treated by volume- controlled lavage of each of her lungs on two occasions. Following the first lavages, the patient's vital capacity increased by 1.03 liters and her 1-sec forced expiratory volume increased by 0.70 liters/second. Similar improvements, although less pronounced, were noted after both lungs has been lavaged a second time. It is concluded that volume-controlled lung lavage can be of benefit as an adjunct in the treatment of patients with cystic fibrosis. RF 001 KYLSTRA JA AM REV RESPIR DIS 103 651 971 002 BRAUNSTEIN MS CHEST 66 96 974 003 ROGERS RM CHEST SUPPL 62 95 972 CT 1 MILLIS RM CHEST 71 508 977 2 EWING CW CHEST 73 750 978 3 ROTHMANN BF ANN OTOL RHINOL LARYNGOL 91 641 982 PN 75062 RN 00228 AN 85177317 AU Warren-C-P. Tai-E. Batten-J-C. Hutchcroft-B-J. Pepys-J. TI Cystic fibrosis--immunological reactions to A. fumigatus and common allergens. SO Clin-Allergy. 1975 Mar. 5(1). P 1-12. MJ ASPERGILLUS-FUMIGATUS: im. CYSTIC-FIBROSIS: im. MN ASPERGILLOSIS: im. ASTHMA: im. EOSINOPHILS: im. HAY-FEVER: im. HUMAN. HYPERSENSITIVITY: im. IGE: an. IMMUNOGLOBULINS: an. LEUKOCYTE-COUNT. PRECIPITIN-TESTS. SKIN-TESTS. AB Immunological studies of forty-three patients with cystic fibrosis showed that positive prick tests to at least one common allergen were obtained in 70%, to multiple allergens in 28% and to A. fumigatus in 50%. Specific IgE antibodies against these allergens were found in the appropriate subjects. In spite of this evidence of type 1, IgE, sensitization none of the patients had a history of infantile eczema. Intracutaneous tests with A. fumigatus extract gave types 1 and 3 reactions in sixteen patients (37%), only seven of whom were among the sixteen (37%) who gave positive precipitin tests. Raised levels of total serum IgG and IgA were found as compared with healthy controls and asthmatic subjects. No differences were found in total serum IgM and IgD levels. The high incidence of allergy to A. fumigatus in cystic fibrosis is confirmed. RF 001 BATTEN JC MOD PROBL PEDIATR 10 227 967 002 COCHRANE CG J EXP MED 134 75S 971 003 CURRAN WS ANN INTERN MED 55 777 961 004 DOLOVICH J J ALLERGY 46 127 970 005 DOLOVICH J J ALLERGY CLIN IMMUNOL 52 38 973 006 KATZ RM N ENGL J MED 288 233 973 007 LESKOWITZ S CLIN ALLERGY 2 237 972 008 LONGBOTTOM JL J PATHOL BACTERIOL 88 141 964 009 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 010 MCCARTHY DS CLIN ALLERGY 1 415 971 011 MEARNS MB THORAX 20 385 965 012 MEARNS MB LANCET 1 538 967 013 PEPYS J IN: GELL PGH 968 014 PEPYS J PROC INT CONG ALLERGOLOGY 6 221 968 015 PEPYS J CLIN ALLERGY SUPPL 3 491 973 016 PEPYS J CLIN ALLERGY 3 143 973 017 ROWE DS BULL WHO 40 613 969 018 SALVAGGIO JE CLIN ALLERGY 3 43 973 019 SCADDING JG SCAND J RESPIR DIS 48 372 967 020 SIMON G TEACH IN ON CF 970 021 TADA T J IMMUNOL 104 377 970 022 TAYLOR B LANCET 2 111 973 023 WIDE L LANCET 2 1105 967 CT 1 WARNER JO LANCET 1 990 976 2 MULLINS J CLIN ALLERGY 6 209 976 3 MITCHELLHEGGS P Q J MED 45 479 976 4 CLARKE CW CLIN ALLERGY 7 527 977 5 WARNER JO CLIN ALLERGY 7 385 977 6 SAKULA A BR J DIS CHEST 71 295 977 7 TURNER MW ARCH DIS CHILD 53 631 978 8 ZAMBIE MF ANN ALLERGY 42 290 979 9 HODSON ME THORAX 35 801 980 10 TOBIN MJ THORAX 35 807 980 11 PRIOR J BR J DIS CHEST 74 84 980 12 MATTHEWS WJ N ENGL J MED 302 245 980 13 CROMWELL O LANCET 2 164 981 14 REEN DJ CLIN ALLERGY 11 571 981 15 CLARKE CW BR J DIS CHEST 75 15 981 16 MOSS RB J PEDIATR 99 215 981 17 ZETTERSTROM O BR MED J 283 1215 981 18 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 19 ANON LANCET 2 257 983 20 BIRX DL ANN ALLERGY 53 124 984 21 HODSON ME POSTGRAD MED J 60 225 984 22 LAUFER P J ALLERGY CLIN IMMUNOL 73 44 984 23 AUERBACH HS LANCET 2 686 985 24 STARKE ID BR J DIS CHEST 79 295 985 25 GORDON IJ J ROY COLL PHYSICIANS LOND 20 206 986 26 PIEDRA P J PEDIATR 108 817 986 PN 75063 RN 00229 AN 76042250 AU Allan-J-D. Moss-A-D. Wallwork-J-C. McFarlane-H. TI Immediate hypersensitivity in patients with cystic fibrosis. SO Clin-Allergy. 1975 Sep. 5(3). P 255-61. MJ CYSTIC-FIBROSIS: im. HYPERSENSITIVITY-IMMEDIATE: im. MN ALLERGENS: ad. CHILD. CHILD-PRESCHOOL. GEL-DIFFUSION-TESTS. HUMAN. IGE: an. INFANT. PRECIPITIN-TESTS. SKIN-TESTS. AB Twenty-three out of thirty patients with cystic fibrosis gave strong immediate skin hypersensitivity reaction to a wide variety of allergens. Seventy-five per cent of these had a markedly elevated serum IgE concentration whereas those patients who had negative Type 1 immediate skin reactions also had normal levels of serum IgE. The sputum of those patients with immediate skin reactivity also had positive precipitins to a variety of antigens. RF 001 JOHANSSEN SGO IMMUNOLOGY 10 265 968 002 MANCINI G IMMUNOCHEMISTRY 2 235 965 003 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 004 MCFARLANE H BR MED J 1 423 975 005 MEARNS MB THORAX 20 385 965 006 MEARNS MB LANCET 1 538 967 007 SHWACHMAN H PEDIATRICS 30 389 962 008 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 009 YOHE RM ANN ALLERGY 30 627 972 CT 1 MCFARLANE H LANCET 1 1241 976 2 WARNER JO LANCET 1 990 976 3 SHAKIB F CLIN ALLERGY 6 237 976 4 WALLWORK JC CLIN ALLERGY 6 349 976 5 CLARKE CW CLIN ALLERGY 7 527 977 6 MCFARLANE H CLIN ALLERGY 7 279 977 7 WARNER JO CLIN ALLERGY 7 385 977 8 RAEBURN JA PROC NUTR SOC 36 77 977 9 SILVERMAN M ARCH DIS CHILD 53 873 978 10 TURNER MW ARCH DIS CHILD 53 631 978 11 GANIER M CLIN ALLERGY 9 125 979 12 PRICE JF CLIN ALLERGY 9 563 979 13 CARSWELL F MONOGR PAEDIATR 10 144 979 14 CARSWELL F CLIN EXP IMMUNOL 35 141 979 15 LARSEN GL AM REV RESPIR DIS 119 399 979 16 NELSON LA AM REV RESPIR DIS 120 863 979 17 THEOHARIDES TC LIFE SCI 27 703 980 18 HODSON ME THORAX 35 801 980 19 TOBIN MJ THORAX 35 807 980 20 MIELIVERGANI G ARCH DIS CHILD 55 696 980 21 MOSS RB AM REV RESPIR DIS 121 23 980 22 LARSEN GL AM J DIS CHILD 134 1143 980 23 HARPER TB LUNG 157 219 980 24 REEN DJ CLIN ALLERGY 11 571 981 25 HOLZER FJ ARCH DIS CHILD 56 455 981 26 CLARKE CW BR J DIS CHEST 75 15 981 27 MOSS RB J PEDIATR 99 215 981 28 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 29 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 30 ANON LANCET 2 257 983 31 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 32 WONNE R CLIN ALLERGY 15 455 985 33 LAUFER P CUTIS 35 557 985 34 LAUFER P CUTIS 36 245 985 PN 75064 RN 00230 AN 75207948 AU Lundgren-D-W. Farrell-P-M. Di-SantAgnese-P-A. TI Polyamine alterations in blood of male homozygotes and heterozygotes for cystic fibrosis. SO Clin-Chim-Acta. 1975 Jul 23. 62(2). P 357-62. MJ CYSTIC-FIBROSIS: bl. HOMOZYGOTE. SPERMIDINE: bl. SPERMINE: bl. MN FEMALE. HETEROZYGOTE. HUMAN. MALE. SEX-FACTORS. AB The polyamines, spermidine and spermine, have been measured in whole blood extracts from control volunteers, patients with cystic fibrosis, and obligate cystic fibrosis heterozygotes. Male homo- and heterozygotes for cystic fibrosis exhibit a consistent and significant decrease in blood spermine resulting in an elevated spermidine/spermine ratio when compared to control males. In contrast, control females exhibited reduced spermidine and spermine levels as compared to control males. Blood from female cystic fibrosis homo- and heterozygotes showed similar results. The sex difference is probably due to fluctuations of blood polyamines during the menstrual cycle. Abnormal polyamine levels are the first observation of an alteration of a low molecular weight metabolite characteristic of both male homo- and heterozygotes for cystic fibrosis. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 RENNERT OM IN: MANGOS JA 41 973 003 TAUSSIG LM J PEDIATR 82 380 973 004 TABOR H ANAL BIOCHEM 55 457 973 005 ROSENTHAL SM J PHARMACOL EXP THER 116 131 956 006 RUSSELL DH ENDOCRINOLOGY 88 1397 971 007 RUSSELL DH BIOCHEM J 128 1109 972 008 COHEN HI CF CLUB ABST 6 1 965 009 WARWICK WJ J ASTHMA RES 5 277 968 010 RUSSELL DH PROC NAT ACAD SCI USA 60 1420 968 011 RUSSELL DH CANCER RES 31 1555 971 013 BAKER AP FED PROC 33 1300 974 014 MUNRO GF J BIOL CHEM 247 1272 972 CT 1 RENNERT O LIFE SCI 19 257 976 2 TABOR CW ANNU REV BIOCHEM 45 285 976 3 COHEN LF BLOOD 48 469 976 4 CHUN PW BIOCHEM BIOPHYS RES COMMUN 69 1095 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 LUNDGREN DW PROC SOC EXP BIOL MED 152 81 976 7 SANTAGNESE PAD N ENGL J MED 295 481 976 8 KELLY JC BIOCHEM GENET 15 695 977 9 SULLIVAN JL BIOCHEM GENET 15 1125 977 10 BURDICK AB HUM HERED 27 366 977 11 LUNDGREN DW J PEDIATR 90 1034 977 12 ALHADEFF JA CLIN GENET 14 189 978 13 ROSENBLUM MG SCIENCE 200 1496 978 14 LUNDGREN DW AM J PHYSIOL 234 E451 978 15 SAVORY J LANCET 2 1136 979 16 RUSSELL DH PEDIATR RES 13 1137 979 17 JAKEL HP BIOL ZENTRALBL 98 55 979 18 BAYLIN SB PEDIATR RES 14 921 980 19 THEOHARIDES TC LIFE SCI 27 703 980 20 BENINATI S BIOMEDICINE EXPRESS 33 182 980 21 NAHAS N BIOCHEM BIOPHYS RES COMMUN 109 1035 982 22 CHENG PW J BIOL CHEM 257 6251 982 23 RUSSELL DH CRC CRIT REV CLIN LAB SCI 18 261 983 24 EKSTROM J ACTA PHYSIOL SCAND 119 287 983 25 CHAYEN R CELL BIOCHEM FUNCT 2 15 984 26 RAM BP CRC CRIT REV BIOCHEM 17 257 985 27 KAMOUN PP CLIN CHIM ACTA 154 219 986 PN 75065 RN 00231 AN 75129766 AU van-Stekelenburg-G-J. van-de-Laar-AJB. van-der-Laag-J. TI Copper analysis of nail clippings. An attempt to differentiate between normal children and patients suffering from cystic fibrosis. SO Clin-Chim-Acta. 1975 Mar 10. 59(2). P 233-40. MJ COPPER: me. CYSTIC-FIBROSIS: di. NAILS: me. MN CHILD. COPPER: an. CYSTIC-FIBROSIS: me. DIAGNOSIS-LABORATORY. FINGERS. HUMAN. METHODS. SPECTROPHOTOMETRY-ATOMIC-ABSORPTION. TOES. AB From 39 normal children and 36 patients, suffering from cystic fibrosis (C/F), the copper content of finger nail clippings and toe nail clippings were determined. From this study it can be concluded that, although the patients with cystic fibrosis (C/F) have a higher copper content, the determination of copper in nail samples cannot be used in order to differentiate between normal children and patients. Also higher mean copper concentrations were found in finger nail clippings in comparison with toe nails. This difference was significant for patients with cystic fibrosis. RF 001 DI SANTAGNESE PA PEDIATRICS 12 549 953 002 GIBSON LE PEDIATRICS 23 545 959 003 BENDER SW MUCOVISCIDOSE CYSTISCHE FIBR 970 004 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 005 WISER WC PEDIATRICS 33 115 964 006 GREEN MN PEDIATRICS 41 989 968 007 BABB AL TRANS AM NUCL SOC 9 591 966 008 FITE LE PROC SYMP BLED INT AT ENE 972 009 BARNETT WB ATOMIC ABSORP APPL STUDY 474 971 010 KOLLBERG H ACTA PAEDIATR SCAND 63 405 974 011 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 012 HARRISON GM CF CLUB ABST 11 26 970 013 MARTIN GM NATURE 202 903 964 014 KANABROCKI E J NUCL MED 9 478 968 015 HARRISON WW CLIN CHIM ACTA 31 63 971 016 BARNETT WB CLIN CHEM 18 923 972 017 DIXON WJ BIOMETRICS 9 74 953 CT 1 SOHLER A CLIN CHIM ACTA 70 391 976 2 SOLOMONS NW AM J CLIN NUTR 32 856 979 3 LANGMYHR FJ ANALYST 104 993 979 4 SOLOMONS NW AM J CLIN NUTR 34 462 981 5 LANGMYHR FJ PROG ANALYT ATOM SPECTR 8 193 985 PN 75066 RN 00232 AN 75129741 AU Kyaw-Myint-T-O. Howell-A-M. Murphy-G-M. Anderson-C-M. TI Alpha-1-antitrypsin in duodenal fluid and gallbladder bile. SO Clin-Chim-Acta. 1975 Feb 22. 59(1). P 51-4. MJ ALPHA-1-ANTITRYPSIN: an. DUODENUM: an. GALLBLADDER: an. MN BILE: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. LIVER-DISEASES: me. MALE. MEGACOLON: me. TRYPSIN-INHIBITORS: an. AB Using the immunoelectrophoretic method alpha-1-antitrypsin was detected in all but 6 of 26 samples of duodenal fluid obtained from 16 patients with various gastroenterological problems. The concentrations (mg/100 ml) of alpha-1-antitrypsin in duodenal aspirates from children with liver disease (7.32 plus or minus 6.1) were less than those from children with cystic fibrosis, Shwachman Diamond syndrome, or Hirschsprung's disease (16.7 plus or minus 11.9; p smaller than 0.02). Alpha-1-antitrypsin was detected in all but one of 6 samples of gallbladder bile, the exception being that from a patient with extrahepatic biliary atresia. No significant correlation was found between the alpha-1-antitrypsin concentration in the samples studied and the corresponding total antitrypsin activity. RF 001 BEARN AG IN: STANBURY JB 1637 972 002 TALAMO RC AM J DIS CHILD 125 845 973 003 TALAMO RC J PEDIATR 79 20 971 004 GLASGOW JFT AM J MED 54 181 973 005 AAGENAES O ACTA PAEDIATR SCAND 61 632 972 006 LEIBERMAN J SCIENCE 175 63 972 007 BELL OF NATURE 243 410 973 008 NELSON R GUT 14 427 973 009 WIGGINS HS GUT 8 415 967 010 HADORN B J PEDIATR 73 39 968 011 MURPHY GM J CLIN PATHOL 23 594 970 012 LAURELL CB SCAND J CLIN LAB INVEST 15 132 963 013 LASKOWSKI M IN: ANSON ML 9 203 954 CT 1 MORSE JO N ENGL J MED 299 1045 978 2 AROOR AR INDIAN J EXP BIOL 17 503 979 3 GRILL BB J PEDIATR GASTROENTEROL NUTR 2 95 983 4 MISZCZUKJAMSKA B HOPPE SEYLERS Z PHYSIOL CHEM 364 509 983 5 CALLEA F HISTOPATHOLOGY 9 99 985 6 THEAKER JM J CLIN PATHOL 39 58 986 PN 75067 RN 00233 AN 75207931 AU Robinson-P-G. Smith-P-A. Elliott-R-B. TI A simple method for the quantitative determination of stool trypsin and chymotrypsin. SO Clin-Chim-Acta. 1975 Jul 23. 62(2). P 225-9. MJ CHYMOTRYPSIN: an. FECES: en. TRYPSIN: an. MN CHILD. CHYMOTRYPSIN: me. CYSTIC-FIBROSIS: en. DUODENUM: en. HETEROZYGOTE. HUMAN. HYDROGEN-ION-CONCENTRATION. INFANT. METHODS. TRYPSIN: me. AB A simple method for the determination of the activity of trypsin and chymotrypsin in stool and duodenal aspirations is described. The method is based on that described by Barbero et al. (Am. J. Dis. Child., 112 (1966) 536) [1] but utilizes the time for a drop of 0.1 pH unit in the solution, instead of a constant pH titration, to determine enzyme activity. Results for a number of cystic fibrosis patients and normals compare favorably with the literature. RF 001 BARBERO GJ AM J DIS CHILD 112 536 966 002 SHWACHMAN H AM J DIS CHILD 66 418 943 003 SHWACHMAN H PEDIATRICS 4 222 949 004 JOHNSTONE DE PEDIATRICS 7 483 951 005 GORDON I BR MED J 1 463 952 006 HORSFIELD A J MED LAB TECH 10 18 952 007 JOHNSTONE DE AM J DIS CHILD 84 191 952 008 NEURATH H CHEM REV 46 69 950 009 RICHMOND RC PEDIATRICS 16 207 955 010 LUNDH G SCAND J CLIN LAB INVEST 9 229 957 011 GROSSMAN MI PROC SOC EXP BIOL MED 110 41 962 012 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 013 DYCK WP AM J DIG DIS 10 530 965 014 ROBINSON PG NZ MED J 79 1024 974 CT 1 ROBINSON PG ARCH DIS CHILD 51 301 976 2 CROSSLEY JR LANCET 2 1093 977 3 DURR HK DIGESTION 17 396 978 4 CONGDON PJ POSTGRAD MED J 57 453 981 5 VARIYAM EP GASTROENTEROLOGY 81 751 981 6 MITCHELL EA AUST PAEDIATR J 18 118 982 7 KASPAR P CLIN CHEM 30 1753 984 8 LAMI F AM J GASTROENTEROL 79 697 984 9 JUNGE W CLIN BIOCHEM 19 323 986 PN 75068 RN 00234 AN 76042342 AU Ryley-H-C. Neale-L-M. Brogan-T-D. Bray-P-T. TI Screening for cystic fibrosis by analysis of meconium for albumin and protease inhibitors. SO Clin-Chim-Acta. 1975 Oct 15. 64(2). P 117-25. MJ ALBUMINS: an. ALPHA-1-ANTITRYPSIN: an. CHYMOTRYPSIN: ai. CYSTIC-FIBROSIS: di. INFANT-NEWBORN-DISEASES: di. MECONIUM: an. MN ALBUMINS: im. COMPARATIVE-STUDY. FECES: an. HUMAN. IMMUNOELECTROPHORESIS. INFANT-NEWBORN. MASS-SCREENING. METHODS. AB A qualitative method of detecting elevated meconium protein concentration was compared with a method of determining meconium albumin concentration by electroimmunoassay since elevated meconium protein levels can indicate pancreatic insufficiency caused by cystic fibrosis. Between 5 and 10 per 1000 healthy infants passed meconium specimens that gave a false positive reaction with the Boehringer Mannheim test strip and contained a greater than expected concentration of albumin. It was possible to exclude pancreatic insufficiency in all of these children by determining the ratio, albumin : alpha1-antitrypsin in meconium and subsequent faecal specimens, since it was found that values of this ratio in excess of 2.0 suggested pancreatic insufficiency of the type associated with cystic fibrosis. Three of 14 neonates with subsequently proven cystic fibrosis yielded meconium specimens giving negative test strip results and low albumin concentrations. In two of these patients, the ratio, albumin : alpha1-antitrypsin in the meconium was within normal limits but, within two months of birth, the albumin : alpha1- antitrypsin ratio in the faeces of both children was greater than 3.0 suggesting that pancreatic insufficiency had developed. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 GEORGE L ARCH DIS CHILD 46 139 971 003 WARWICK WJ MINN MED 52 1567 969 004 DI SANTAGNESE PA N ENGL J MED 277 1344 967 005 DI SANTAGNESE PA N ENGL J MED 277 1399 967 006 BUCHANAN DJ PEDIATRICS 9 304 952 007 WISER WC PEDIATRICS 33 115 964 008 GREEN MN PEDIATRICS 21 635 958 009 ROULET DLA SCHWEIZ MED WOCHENSCHR 91 74 961 010 PRITCHARD JA OBSTET GYNECOL 25 289 965 011 HIGA Y AM J OBSTET GYNECOL 119 932 974 012 RYLEY HC ARCH DIS CHILD 49 901 974 013 DAVIDSON AG BR MED J 4 362 971 014 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 015 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 016 BULL FE ARCH DIS CHILD 49 602 974 017 RYLEY HC J CLIN PATHOL 26 852 973 018 CRAWFORD IP ARCH BIOCHEM BIOPHYS 156 215 973 019 ROSEBAUM T SCIENCE TOOLS APPL NOTE 87 973 020 AVRAMEAS S IMMUNOCHEMISTRY 6 53 969 021 FAGERHOL MK IN: MITTMAN C 145 972 022 SIEGAL S NONPARAMETRIC STATISTICS 956 023 PROSSER R ARCH DIS CHILD 49 597 974 CT 1 RYLEY HC LANCET 2 365 976 2 GRIFFITHS AD ARCH DIS CHILD 51 901 976 3 RYLEY HC ARCH DIS CHILD 51 641 976 4 ANON BR MED J 1 596 977 5 CROSSLEY JR BR MED J 1 428 977 6 CROSSLEY JR LANCET 2 1093 977 7 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 8 STARK JM J CLIN PATHOL 31 1177 978 9 EVANS TJ ARCH DIS CHILD 53 239 978 10 RYLEY HC MONOGR PAEDIATR 10 151 979 11 STEPHAN U MONOGR PAEDIATR 10 41 979 12 UXA F MINERVA PEDIATR 31 831 979 13 RYLEY HC ARCH DIS CHILD 54 92 979 14 DOYLE EE IR MED J 72 93 979 15 MASTELLA G RIV ITAL PEDIATR 7 581 981 16 MORRIS TJ CLIN ALLERGY 11 561 981 17 EVANS RT J CLIN PATHOL 34 911 981 18 RYLEY HC J CLIN PATHOL 34 179 981 19 THOMAS DW GASTROENTEROLOGY 80 776 981 20 PARK RW GASTROENTEROLOGY 81 1143 981 21 DODGE JA ARCH DIS CHILD 57 774 982 22 PEDERZINI F RIV ITAL PEDIATR 9 445 983 23 HEELEY AF CLIN CHEM 29 2011 983 24 RYLEY HC CLIN CHIM ACTA 135 49 983 25 NAYLOR EW SEM PERINATOL 9 232 985 PN 75069 RN 00235 AN 75167100 AU Danes-B-S. TI Cystic fibrosis heterozygote detection: a study on a normal population. SO Clin-Genet. 1975 Feb. 7(2). P 128-33. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. MN ADOLESCENCE. ADULT. BIOLOGICAL-ASSAY. CELLS-CULTURED. CILIA: me. CULTURE-MEDIA. CYSTIC-FIBROSIS: me. FEMALE. FIBROBLASTS. GENETICS-POPULATION. HUMAN. LEUKOCYTES. MALE. MIDDLE-AGE. OYSTERS. SKIN. AB Detection of the cystic fibrosis (CF) heterozygote by testing for cystic fibrosis factor activity (CFFA) in serum samples using bioassays had been reported to give unreliable results. The present study was undertaken to find whether the use of medium from short term white blood cell or skin fibroblast cultures, rather than serum, as the test sample would eliminate such inconsistencies. Short term white blood cell cultures were established from 107 normal, healthy individuals. Cultures from 97 had no CFFA, six (No. 1-6) showed variable results and four (No. 7-10) had activity in all four tests done on a single sample. When the assays were repeated 3 months later on new cultures from these four positive CFFA individuals, two showed activity (Nos. 7 & 9) and two did not (Nos. 8 & 10). All 10 individuals whose cultures showed CFFA has histories of allergies. The association of CFFA in the culture medium derived from individuals having allergies with the intermittent presence of this activity suggested that environmental factors induced a CFF-like activity in such white blood cell cultures. The oyster ciliary assay, using medium from white blood cell cultures, cannot be used for screening for the CF heterozygote since the substitution of culture medium for serum did not eliminate inconsistent results and CFFA was not specific by this assay for the CF gene. Of the fibroblast lines established from the four persons whose initial white blood cell cultures consistently showed CFFA, only one (No. 7) showed the CF culture phenotype (CFFA positive and metachromasia). RF 001 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 002 BOWMAN BH CLIN GENET 4 461 973 003 BOWMAN BH SCIENCE 164 325 969 004 BOWMAN BH SCIENCE 167 871 970 005 CONOVER JH PEDIATR RES 7 220 973 006 CONOVER JH PEDIATR RES 7 224 973 007 CONOVER JH LANCET 1 1194 973 008 CONOVER JH LANCET 2 1501 973 009$ DANES BS BIRTH DEF ORIG ART SER 7 141 971 010 DANES BS BIRTH DEF ORIG ART SER 8 114 972 011 DANES BS LANCET 2 765 973 013 DANES BS J EXP MED 123 1 966 014 DANES BS J EXP MED 129 775 969 016 DANES BS J EXP MED 137 1538 973 017 DANES BS J EXP MED 132 765 970 018 DI SANTAGNESE PA N ENGL J MED 277 1287 967 019 HIRSCHHORN K IN: MANGOS JA 11 973 020 SPOCK A PEDIATR RES 1 173 967 021 WOOD RE LANCET 2 1452 973 CT 1 DANES BS CLIN GENET 8 85 975 2 GILLARD BK PEDIATR RES 10 907 976 3 DANES BS TEX REP BIOL MED 34 135 976 4 DANES BS CLIN GENET 11 83 977 5 IMPERO JE PEDIATR RES 12 108 978 6 DANES BS CLIN GENET 13 327 978 7 WILSON GB J LAB CLIN MED 92 463 978 PN 75070 RN 00236 AN 75208046 AU Danes-B-S. Beck-B. Flensborg-E-W. TI Cystic fibrosis heterozygote detection: a family study. SO Clin-Genet. 1975 Jul. 8(1). P 85-91. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. MN CELLS-CULTURED. CHROMOSOME-MAPPING. FEMALE. FIBROBLASTS: cy. GENES. HOMOZYGOTE. HUMAN. LEUKOCYTES: cy. MALE. PEDIGREE. PHENOTYPE. AB Family studies have been done to determine if the phenotypes described for the cystic fibrosis (CF) cultured cell reliably detected the CF gene. Two culture phenotypes were studied in culture derived from consecutive generations in four CF families: (1) cystic fibrosis factor (CFF) in the medium of short-term white blood cell cultures assayed by a modified oyster ciliary test and (2) the three characteristics of cultured skin fibroblasts (metachromasia without alcianophilia at 0.3 M MgCl2, CFF in the culture medium and metabolic cooperation with normal fibroblasts). As CF is an autosomal recessive disorder, the family offered a test unit for determining the specificity of these cell phenotypes for the CF gene. On the basis of detecting the CF culture phenotypes in the homozygote and obligatory heterozygotes, the parents, and their segregation in consecutive generations, detection of the CF gene was not possible in the white blood cell cultures, but was possible in the skin fibroblast cultures. RF 001 BERATIS NG PEDIATR RES 7 958 973 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 003 BOWMAN BH SCIENCE 164 325 969 004 BOWMAN BH SCIENCE 167 871 970 005 CONOVER JH PEDIATR RES 7 220 973 006 CONOVER JH PEDIATR RES 7 224 973 007 CONOVER JH LANCET 1 1194 973 008$ DANES BS BIRTH DEF ORIG ART SER 7 141 971 009 DANES BS BIRTH DEF ORIG ART SER 8 114 972 010 DANES BS LANCET 2 765 973 011 DANES BS CLIN GENET 7 128 975 012 DANES BS J EXP MED 123 1 966 013 DANES BS J EXP MED 129 775 969 014 DANES BS AM J HUM GENET 23 297 971 015 DANES BS J EXP MED 137 1538 973 016 DANES BS J EXP MED 132 765 970 017 DI SANTAGNESE PA N ENGL J MED 277 1287 967 018 SPOCK A PEDIATR RES 1 173 967 019 WOOD RE LANCET 2 1452 973 CT 1 GILLARD BK PEDIATR RES 10 907 976 2 DANES BS TEX REP BIOL MED 34 135 976 3 WARD JB TEX REP BIOL MED 34 11 976 4 DANES BS CLIN GENET 11 83 977 5 IMPERO JE PEDIATR RES 12 108 978 6 NIELSEN HE ACTA PAEDIATR SCAND 67 443 978 7 PEDERSEN PS ACTA PAEDIATR SCAND 70 507 981 8 WINGE P DAN MED BULL 29 358 982 9 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75071 RN 00237 AN 75167122 AU Ho-Shin-Y. Glass-L. Evans-H-E. TI Pulmonary diseases in childhood. SO Clin-Notes-Respir-Dis. 1975 Summer. 14(1). P 3-12. MJ LUNG-DISEASES. MN ASTHMA. ATELECTASIS. BRONCHI. BRONCHIOLITIS-VIRAL. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS. FEMALE. FOREIGN-BODIES. HEMOPTYSIS. HUMAN. INFANT. INFANT-NEWBORN. MALE. MEDIASTINAL-EMPHYSEMA. PNEUMONIA. PNEUMOTHORAX. PREGNANCY. RESPIRATORY-DISTRESS-SYNDROME. TUBERCULOSIS-IN-CHILDHOOD. TUBERCULOSIS-PULMONARY. EX Many types of lung disease are seen in the pediatric age group. These range all the way from disorders of the neonate that are closely related to parturition, to acute infectious diseases seen in infancy and early childhood, to chronic disorders such as cystic fibrosis and bronchial asthma. Management of the conditions described involves cooperation between the primary care physician and the specialist. A variety of organizations offers aid to both physicians and patients' families. RF 001 MILLER HC J PEDIATR 72 628 968 002 GLUCK L PEDIATR RES 6 81 972 003 GLUCK L AM J OBSTET GYNECOL 109 440 971 004 CLEMENTS JA N ENGL J MED 286 1077 972 005 KATTWINKEL J PEDIATRICS 52 131 973 006 WILSON MG AM J DIS CHILD 99 489 960 007 ANDERSON WR ARCH PATHOL 91 506 971 008 ELDERKIN FM BR MED J 2 722 965 009 RAPKIN RH CLIN PEDIATR 10 312 971 010 GERBEAUX J AM J DIS CHILD 110 507 965 011 DURFEE ML AM REV RESPIR DIS 99 304 969 012 MARKS MB CLIN PEDIATR 13 225 974 013 FRANKLIN W N ENGL J MED 290 1469 974 014 GIBBS GE PAEDIATRICIAN 1 133 972 PN 75072 RN 00238 AN 76065031 AU Shwachman-H. Khaw-K-T. Kowalski-S-M. TI The management of cystic fibrosis. Observations on gentamycin when used with other antibiotics in pulmonary infections associated with cystic fibrosis. SO Clin-Pediatr (Phila). 1975 Dec. 14(12). P 1115-8. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: dt. GENTAMICINS: tu. LUNG-DISEASES: dt. MN CYSTIC-FIBROSIS: co. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. LUNG-DISEASES: et. MALE. PSEUDOMONAS-INFECTIONS: dt. EX From 1971 to 1973, we hospitalized 41 symptomatic patients with cystic fibrosis for intensive pulmonary therapy. The more severely ill patients were admitted more than once during this period. Our study included 100 courses of such intensive treatment. Garamycin pediatric injectable, containing 10 mg/ml of the active ingredient, was administered intravenously to all patients in divided doses of 3.0 to 5.0 mg/kg/day. The purpose of the study was to evaluate the tolerance, pharmacokinetics, and efficacy of garamycin when administered parenterally to cystic fibrosis patients with serious infections. During the 100 courses of treatment, clinical improvement was rated as follows: significant or definite improvement, 39; moderate improvement, 50; slight improvement, 9; and no change, 2. The use of garamycin pediatric injectable is safe and can be helpful in the management of the pulmonary infectious complications of cystic fibrosis when employed along with other well-established modalities of therapy. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 GREEN MN PEDIATRICS 41 989 968 003 STEPHAN U IN: MANGOS JA 281 973 004 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 005 SHWACHMAN H ANN NY ACAD SCI 93 600 962 006 SHWACHMAN H CF IN PEDIATRIC THERAPY 573 972 007 GINGELL JC J INFECT DIS 119 396 969 008 KULCZYCKI LL CLIN PEDIATR 9 390 970 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 SHWACHMAN H MEDICINE 56 129 977 3 SCHIOTZ PO HUM HERED 28 293 978 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 5 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 PN 75073 RN 00239 AN 76002085 AU Grossman-M-L. TI The psychosocial approach to the medical management of patients with cystic fibrosis. SO Clin-Pediatr (Phila). 1975 Sep. 14(9). P 830-3. MJ ADAPTATION-PSYCHOLOGICAL. CYSTIC-FIBROSIS: th. MN ADOLESCENCE. AMBULATORY-CARE. ANXIETY-SEPARATION. ATTITUDE-TO-DEATH. ATTITUDE-TO-HEALTH. CHILD-PSYCHIATRY. CHILD. CHILD-HOSPITALIZED. CHILD-PRESCHOOL. DEFENSE-MECHANISMS. HUMAN. IDENTITY-CRISIS. INFANT. INFANT-NEWBORN. LIFE-STYLE. PARENT-CHILD-RELATIONS. PROFESSIONAL-PATIENT-RELATIONS. REFERRAL-AND-CONSULTATION. STRESS-PSYCHOLOGICAL. EX It is most important to deal with the feelings of the child and his family in regard to cystic fibrosis. Genuine recognition of feelings by the pediatric staff can go a long way toward reassuring family members that their long-standing burden and suffering are being validated and understood. When a family manifests early signs of difficulty in coping with the emotional and physical stress imposed by cystic fibrosis, it is helpful to refer them to a child psychiatrist for consultation. It is primarily the families at high risk for breakdown which most require early prevention and intervention. The role of the pediatrician as the primary clinician is crucial. It is he who must provide the support and understanding that are integral parts of the primary prevention of emotional decompensation in this disorder. RF 001 BOWLBY J MATERNAL CARE AND MENTAL HEAL 952 002 BOWLBY J INT J PSYCHOANAL 41 89 960 003 TISZA VB J AM ACAD CHILD PSYCHIAT 9 515 970 004 SPITZ RA PSYCHOANALYT STUDY CHILD 2 313 946 005 BERGMANN T CHILDREN IN THE HOSPITAL 965 006 PRUGH DG AM J ORTHOPSYCHIATRY 23 70 953 007 PIAGET J SIX PSYCHOLOGICAL STUDIES 967 008 ERIKSON EH IDENTITY AND THE LIFE CYCLE 959 009 MCCOLLUM AT J PEDIATR 77 571 970 010 LEVY DM MATERNAL OVERPROTECTION 943 011 PRUGH DG IN: FREEDMAN AM 181 971 012 CAPLAN G PRINCIPLES OF PREVENTIVE PSYC 964 PN 75074 RN 00240 AN 76002091 AU Dolan-T-F-Jr. Meyers-A. TI Mild cystic fibrosis presenting as an asymptomatic distended appendiceal mass: A case report. SO Clin-Pediatr (Phila). 1975 Sep. 14(9). P 862-3. MJ APPENDICITIS: di. CYSTIC-FIBROSIS: di. MN APPENDECTOMY. APPENDIX: pa. CASE-REPORT. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. DIAGNOSIS-DIFFERENTIAL. FEMALE. HUMAN. PALPATION. AB A case of cystic fibrosis presenting as a right lower quadrant mass in a five-year-old white female is presented. At laparotomy, a mucoid, impacted appendix was found which microscopically suggested the diagnosis of cystic fibrosis. Cystic fibrosis must be added to conditions presenting with abdominal masses. RF 001 BECK AR GASTROENTEROLOGIA 106 84 966 002 HOLSCLAW DS PEDIATRICS 48 51 971 003 SHWACHMAN H N ENGL J MED 286 1300 972 004 SNYDER WH JR PEDIATRICS 34 72 964 CT 1 STERN RC J PEDIATR 89 412 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 LOBER CW JAMA 235 1140 976 4 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 5 MCCARTHY VP GASTROENTEROLOGY 86 564 984 PN 75075 RN 00241 AN 75130315 AU Monset-Couchard-M. Mason-C-V. Moss-A-J. TI Cor pulmonale in children. SO Curr-Probl-Pediatr. 1975 Feb. 5(4). P 3-31. (REVIEW). MJ PULMONARY-HEART-DISEASE. MN ACUTE-DISEASE. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co. DIGOXIN: tu. ELECTROCARDIOGRAPHY. FEMALE. HEART-CATHETERIZATION. HEART-ENLARGEMENT: co. HUMAN. HYPERTENSION-PULMONARY: co. INFANT. INFANT-NEWBORN. LUNG: bs. MALE. PULMONARY-HEART-DISEASE: pa, cl, pp, et, ra, co, dt. REVIEW. VECTORCARDIOGRAPHY. EX According to the World Health Organization, chronic cor pulmonale is defined as "hypertrophy of the right ventricle resulting from diseases affecting the function and/or the structure of the lung, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart or of congenital heart disease." Cor pulmonale has been extensively studied and reported in adults, but it has received relatively little attention in the pediatric age group. Although some parts of knowledge gained from adult studies can be used, chronic cor pulmonale in children and infants is quite a different entity from that usually seen in adults. This review is primarily an attempt to clarify some of the confusion surrounding this subject and to focus on the pediatric aspects. Pathophysiology, etiology, diagnosis, and treatment are discussed. RF 001 ANON CIRCULATION 27 594 963 002 YATER WM AM J MED SCI 191 474 936 003 NAEYE RL AM J PATHOL 38 561 961 004 YEPEZ CG AM J CARDIOL 10 30 962 005 MOUSA AH J EGYPT MED ASSOC 47 270 964 006 TURINO GM BULL NY ACAD MED 41 959 965 007 HOSONO K JAPAN HEART J 6 318 965 008 HICKAM JB BULL NY ACAD MED 41 994 965 009 GOUGH J BULL NY ACAD MED 41 927 965 010 FERRER MI BULL NY ACAD MED 41 942 965 011 DEXTER L BULL NY ACAD MED 41 981 965 012 FUKUDA M JAPAN CIRC J 30 693 966 013 OSTREA EM JR ACTA MED PHILIP 2 68 965 014 PARKER JO CIRCULATION 33 17 966 015 SASAHARA AA PROG CARDIOVASC DIS 9 259 966 016 TORRANCE DJ PROG CARDIOVASC DIS 9 275 966 017 DINES DE TUBERCULOLOGY 24 17 967 018 LEE J AM REV RESPIR DIS 96 1173 967 019 OAKLEY CM AM J CARDIOL 20 842 967 020 ABRAHAM AS CIRC RES 24 51 969 021 BUCHANAN WM J TROP MED HYG 73 218 970 022 MORET P ACTA CARDIOL BRUX 27 596 972 023 LUCAS RV MINN MED 52 1537 969 024 THOMAS AJ BR HEART J 34 653 972 025 BARNARD PJ AM J DIS CHILD 92 115 956 026 ROSENBERG HS PEDIATRICS 20 408 957 027 HUSSON GS PEDIATRICS 23 493 959 028 THILENIUS OG PEDIATRICS 36 75 965 029 NOONAN JA N ENGL J MED 269 70 963 030 SPERLING DR AM J DIS CHILD 107 308 964 031 FRIEDMAN S J PEDIATR 64 305 964 032 FAVARA BE JAMA 199 668 967 033 RAO PS J NEUROSURG 33 221 970 034 SINHA RP J S CAROLINA MED ASSOC 67 421 971 035 AL NAAMAN YD ANGIOLOGY 17 40 965 036 ANDERSON G PROC R SOC MED 65 824 972 037 CHESNEY CF AM J PATHOL 70 489 973 038 SIMMONS DH DIS CHEST 48 347 965 039 VON EULER US ACTA PHYSIOL SCAND 12 301 946 040 HARVEY RM AM J MED 10 719 951 041 FISHMAN AP HOSP PRACT 6 101 971 042 DOYLE JT CIRCULATION 5 263 952 043 FISHMAN AP CIRCULATION 22 204 960 044 ABRAHAM AS CARDIOVASC RES 5 95 971 045 JAMES WRL CARDIOVASC RES 2 278 968 046 HEATH D THORAX 28 24 973 047 SIME F AM J CARDIOL 11 143 963 048 PENALOZA D AM J CARDIOL 11 150 963 049 GROVER RF AM J CARDIOL 18 928 966 050 VOGEL JHK AM HEART J 72 50 966 051 KILBURN KH CIRCULATION 39 639 969 052 LILJESTRAND G ACTA PHYSIOL SCAND 44 216 958 053 BERGOFSKY EH J CLIN INVEST 41 1492 962 054 DOWNING SE AM J PHYSIOL 208 237 965 055 HERLES F CARDIOVASC RES 6 641 972 056 ENSON Y J CLIN INVEST 43 1146 964 057 LLOYD TC JR J APPL PHYSIOL 21 358 966 058 RUDOLPH AM J CLIN INVEST 45 399 966 059 HARVEY RM CIRCULATION 35 1019 967 060 NOONAN JA PEDIATR CLIN NORTH AM 18 1255 971 061 BERGOFSKY EH MEDICINE (BALTIMORE) 38 263 959 062 SYMCHYCH PS ARCH PATHOL 92 409 971 063 SWIGART RH CIRC RES 17 30 965 064 NAEYE RL AM J PATHOL 50 1027 967 065 VOGEL JHK CIRC RES 13 557 963 066 LAKS MM AM J PHYSIOL 222 578 972 067 MALAMANI V BULL PHYSIOPATH RESPIR NANCY 8 1397 972 068 BAROLDI G BULL PHYSIOPATH RESPIR NANCY 8 1406 972 069 WILLIAMS JF JR J CLIN INVEST 47 1143 968 070 FRANK MJ CIRCULATION 47 798 973 071 BAUM GL N ENGL J MED 285 361 971 072 LOCKHART A BULL PHYSIOPATH RESPIR NANCY 8 1448 972 073 RAO BS AM J MED 45 229 968 074 FISHMAN AP N ENGL J MED 285 402 971 075 HARRIS P BULL PHYSIOPATH RESPIR NANCY 8 1408 972 076 BERGLUND E BULL PHYSIOPATH RESPIR NANCY 8 1417 972 077 BLOUNT SG MOD CONCEPTS CARDIOVASC DIS 36 61 967 078 GROVER RF AM HEART J 66 1 963 079 FOWLER KT J APPL PHYSIOL 18 244 963 080 VOGEL JHK AM J DIS CHILD 113 576 967 081 MORGAN AD AM HEART J 73 550 967 082 WALCOTT G AM J MED 49 70 970 083 HOOD WB JR BR HEART J 30 336 968 084 THOMPSON P BR HEART J 32 758 970 085 WAGENVOORT CA CIRCULATION 42 1163 970 086 BLOUNT SG MOD CONCEPTS CARDIOVASC DIS 36 67 967 087 BURNELL RH AM J DIS CHILD 123 167 972 088 LEVINE OR J PEDIATR 83 964 973 089 MALLORY TB N ENGL J MED 217 1045 937 090 LE TAN VINH ARCH FR PEDIATR 31 187 974 091 EMERY JL ARCH DIS CHILD 37 591 962 092 HABER SL J PEDIATR 61 759 962 093 GOOTMAN N PEDIATRICS 34 861 964 094 SYMCHYCH PS AM J DIS CHILD 110 636 965 095 STREEPER RB DIS CHEST 46 61 964 096 EMERY JL SURGERY 50 309 961 097 MOSER KM AM J MED 22 561 957 098 OPPENHEIMER EH AM REV RESPIR DIS 103 858 971 099 PEARSON HA PEDIATRICS 48 629 971 100 MOTULSKY AG N ENGL J MED 288 31 973 101 PEARSON HA J PEDIATR 81 1201 972 102 DIGGS LW CLIN PEDIATR 10 697 971 103 RANNEY HM BLOOD 39 433 972 104 KENAWY MR AM HEART J 39 678 950 105 GIRGIS B AM HEART J 43 606 952 106 TURNER PP BR HEART J 26 821 964 107 ABDIN ZH J EGYPT MED ASSOC 55 622 972 108 ARIAS-STELLA J AM J PATHOL 41 55 962 109 PENALOZA D PEDIATRICS 34 568 964 110 VOGEL JHK J PEDIATR 64 315 964 111 CUDKOWICZ L BULL AM COLL CHEST PHYSICIANS 9 16 970 112 COX MA J PEDIATR 67 192 965 113 NOONAN JA CIRCULATION SUPPL 2 32 164 965 114 MENASHE VD J PEDIATR 67 198 965 115 LUKE MJ PEDIATRICS 37 762 966 116 LEVY AM N ENGL J MED 277 506 967 117 JOHNSON EA J MED SOC NJ 64 220 967 118 PILAPIL VR SURG FORUM 18 493 967 119 AINGER LE BR HEART J 30 356 968 120 GERALD B RADIOLOGY 90 679 968 121 SETLIFF RC LARYNGOSCOPE 78 845 968 122 BLAND JW JR AM J CARDIOL 23 830 969 123 CAYLER GG AM J DIS CHILD 118 708 969 124 JERESATY RM AM J DIS CHILD 117 710 969 125 MACARTNEY FJ ARCH DIS CHILD 44 585 969 126 MASSUMI RA DIS CHEST 55 110 969 127 GRESHAM EL CLIN PEDIATR 10 236 971 128 EDISON BD ARCH OTOLARYNGOL 98 205 973 129 CHAPMAN EM MEDICINE 18 167 939 130 STEINBORN KE ARCH INTERN MED 110 249 962 131 DOUGLAS FG J APPL PHYSIOL 33 559 972 132 JENAB M PEDIATRICS 24 23 959 133 CAYLER GG PEDIATRICS 27 237 961 134 MACGREGOR MI JOHNS HOPKINS MED J 126 279 970 135 DOLL E BEITR KLIN ERFORSCH TUBERK 141 88 969 136 SCHWARTZ BA REV NEUROL 116 677 967 137 SCHWARTZ BA REV EEG NEUROPHYSIOL 4 79 974 138 FISHMAN LS AM J DIS CHILD 110 155 965 139 FRUHMANN G BULL PHYSIOPATH RESPIR NANCY 8 1173 972 140 ROYCE SW PEDIATRICS 8 255 951 141 GOLDRING RM J PEDIATR 65 501 964 142 BOWDEN DH AM J MED 38 226 965 143 MOSS AJ J PEDIATR 67 797 965 144 KELMINSON LL PEDIATRICS 39 24 967 145 SIASSI B J PEDIATR 78 794 971 146 OPPENHEIMER EH JOHNS HOPKINS MED J 131 351 972 147 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 148 GERBEAUX J ANN PEDIATR (PARIS) 19 557 972 149 BAAR HS ARCH DIS CHILD 32 199 957 150 SWAYE P DIS CHEST 55 7 969 151 PERMUTT S CHEST SUPPL 63 25S 973 152 GRIFFIN JT PEDIATRICS 24 54 959 153 RICHARDS W AM J DIS CHILD 110 4 965 154 MIYAMOTO T CIRCULATION 22 90 960 155 QUIVERS WW AM J CARDIOL 14 616 964 156 OLIVA PB CHEST 58 167 970 157 LIEBERMAN J MED CLIN NORTH AM 57 691 973 158 SCOTT-DUNBAR J AM J ROENTG RAD THER NUCL MED 109 227 970 159 LAFOURCADE J REV PRAT 17 3479 967 160 OSHEA PA JOHNS HOPKINS MED J 126 320 970 161 GERBEAUX J ANN PEDIATR (PARIS) 19 565 972 162 CHASE HV MD STATE MED J 19 65 970 163 CHIN TDY MYCOPATHOL MYCOL APPL 41 35 970 164 MACKINNON JE MYCOPATHOL MYCOL APPL 41 187 970 165 KEPRON MW CAN MED ASSOC J 106 243 972 166 HODGMAN JE PEDIATRICS 44 179 969 167 KRAUSS AN J PEDIATR 77 27 970 168 GROSSMAN H AM J ROENTG RAD THER NUCL MED 114 293 972 169 BOMSEL F ANN RADIOL (PARIS) 17 305 974 170 NORTHWAY WH JR N ENGL J MED 276 357 967 171 SHEPARD FM N ENGL J MED 279 1063 968 172 DE VAN HEESE H J PEDIATR 76 183 970 173 BOMSEL F ANN RADIOL (PARIS) 16 70 973 175 NAEYE RL DIS CHEST 44 374 963 176 NORTON WL ANN INTERN MED 73 317 970 177 JORDAN JD AM J DIS CHILD 108 174 964 178 GROSS M AM REV RESPIR DIS 105 572 972 179 HEJTMANCIK MR AM HEART J 68 119 964 180 MEISLIN AG PEDIATRICS 42 37 968 181 KOERNER SK AM HEART J 88 115 974 183 PERLOFF JK PROG CARDIOVASC DIS 9 303 967 184 LIEBMAN J CIRCULATION 35 552 967 185 BURGESS JH CIRCULATION 49 541 974 186 KNUDSON RJ MED CLIN NORTH AM 47 681 973 187 BAUM GL MED CLIN NORTH AM 57 623 973 188 DOWNES JJ PEDIATR CLIN NORTH AM 19 423 972 189 MORGAN AD AM HEART J 73 700 967 190 RUTKOWSKI MM AM HEART J 86 270 973 191 HAYES CJ PEDIATRICS 52 561 973 192 NADAS AS PEDIATRIC CARDIOLOGY 286 972 193 MOSS AJ MOD PROBL PEDIATR 10 187 967 194 CROWELL JW AM J PHYSIOL 206 313 964 195 WILLIAMS JF JR AM J CARDIOL 16 534 965 196 HERNANDEZ A AM J DIS CHILD 115 576 968 PN 75076 RN 00242 AN 76021663 AU Lykkegaard-E. Pedersen-J-T. TI Classes of skin fibroblast metachromasia in cystic fibrosis correlated with lung function tests. SO Dan-Med-Bull. 1975 Jul. 22(5). P 175-7. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. BIOPSY. CELLS-CULTURED. CHILD. CYSTIC-FIBROSIS: pa. FEMALE. FIBROBLASTS. HUMAN. MALE. RESPIRATORY-FUNCTION-TESTS. SKIN: pa. STAINS-AND-STAINING. EX Lung function tests were conducted in 35 patients with cystic fibrosis and correlated to class of skin fibroblast metachromasia. Seven patients belonged to class I (vesticular metachromasia), 16 to class II (generalized metachromasia), and 12 to class III (no metachromasia). Class I patients had significantly lower vital capacity (VC) than class II patients. As regards the other lung function tests, no general trends were found in class I patients compared with the other two classes. For class III patients the lung function tests reflected more serious lung changes than those of class II patients; statistically significant differences were found as regards residual volume/total lung capacity, one-second forced expiratory volume (FEV1), and FEV1/VC. The differences were not due to varying intensity of treatment. The results are in accordance with the findings in a previous study of the clinical and radiological lung symptoms and serum protein values in CF when correlated to metachromatic classes. RF 001 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 002 DANES BS LANCET 1 1061 968 003 DANES BS J EXP MED 129 775 969 004 DANES BS AM J HUM GENET 23 297 971 005 HOYBYE G UGESKR LAEGER 132 2007 970 006 LYKKEGAARD E DAN MED BULL 22 169 975 007 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 008 POGUE RE MINN MED 52 1551 969 009 POLGAR G PULMONARY FUNCTION TESTING IN 971 CT 1 LYKKEGAARD E DAN MED BULL 22 177 975 2 LYKKEGAARD E DAN MED BULL 22 169 975 3 WEEKE B DAN MED BULL 23 155 976 4 DANES BS CLIN GENET 11 83 977 5 DANES BS CLIN GENET 13 327 978 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 WINGE P DAN MED BULL 29 358 982 8 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75077 RN 00243 AN 76021664 AU Lykkegaard-E. TI Classes of skin fibroblast metachromasia in cystic fibrosis correlated with growth parameters. SO Dan-Med-Bull. 1975 Jul. 22(5). P 177-80. MJ CYSTIC-FIBROSIS: pp. GROWTH. MN ADOLESCENCE. BIOPSY. BODY-HEIGHT. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. FEMALE. FIBROBLASTS. HUMAN. MALE. RETROSPECTIVE-STUDIES. SKIN: pa. STAINS-AND-STAINING. AB Metachromasia of cultured skin fibroblasts from patients with cystic fibrosis (CF), and the assignment of the fibroblasts to one of three classes after the morphological distribution of the metachromatic staining, have been described by Danes and Bearn (1968, 1969) and Danes (1969). In two previous studies from this hospital (Lykkegaard & Jacobsen 1975, Lykkegaard & Thuesen Pedersen 1975) the clinical, radiological, and functional severity of the lung disease and the serum protein pattern in CF patients were studied in relation to classes of skin fibroblast metachromasia. It was found that class III patients tended to be more severely affected than class II patients, and more than class I + II patients, and, possibly, class I patients were also more severely affected than class II patients. Sproul and Huang (1964), in a study of growth patterns in cystic fibrosis, found a significant correlation between severity of the respiratory disease and height and weight retardation. In the present study, height and weight development in 98 CF patients are correlated with class of skin fibroblast metachromasia. RF 001 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 002 DANES BS LANCET 1 1061 968 003 DANES BS J EXP MED 129 775 969 004 LYKKEGAARD E DAN MED BULL 22 169 975 005 LYKKEGAARD E DAN MED BULL 22 175 975 006 SPROUL A J PEDIATR 65 664 964 007 TANNER JM ARCH DIS CHILD 41 613 966 CT 1 LYKKEGAARD E DAN MED BULL 22 169 975 2 WEEKE B DAN MED BULL 23 155 976 3 DANES BS CLIN GENET 11 83 977 4 DANES BS CLIN GENET 13 327 978 5 JAKEL HP BIOL ZENTRALBL 98 55 979 6 WINGE P DAN MED BULL 29 358 982 7 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75078 RN 00244 AN 76021662 AU Lykkegaard-E. Jacobsen-L. TI Classes of skin fibroblast metachromasia in cystic fibrosis correlated with clinical parameters. SO Dan-Med-Bull. 1975 Jul. 22(5). P 169-74. MJ CYSTIC-FIBROSIS: pa. MN ADOLESCENCE. ADULT. AGE-FACTORS. BIOPSY. BLOOD-PROTEINS: an. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: ra, bl. FEMALE. FIBROBLASTS. HUMAN. MALE. RETROSPECTIVE-STUDIES. SEX-FACTORS. SKIN: pa. STAINS-AND-STAINING. EX A retrospective study of clinical and radiological lung symptoms and intensity of treatment of 98 patients with cystic fibrosis was conducted. The results were correlated to class of skin fibroblast metachromasia. Fourteen patients belonged to class I (vesicular metachromasia), 52 to class II (generalized metachromasia), and 32 to class III (no metachromasia). All patients above 16 years of age (six patients) were class II. Simple scoring systems for clinical lung symptoms and for treatment of lung disease were developed. When compared at the same ages, class I and III patients were more severely affected (judged by the clinical lung symptoms) than class II patients, and they had been more intensively treated. Comparing class I + II patients (metachromatic fibroblasts) with class III patients (ametachromatic fibroblasts), it was found that class III patients were more severely affected and had been more intensively treated than class I + II patients. As regards chest radiographs (judged by Norman's system) a similar pattern was seen, but no statistically significant differences were found. In 68 patients (9 of class I, 35 of class II, and 24 of class III) the serum protein electrophoretic fractions were studied. It was found that class III patients had more severe alterations than class II patients, and more than class I + II patients. The proportion of female patients in class III was greater than in the other two classes, but this could not explain the differences. Because of the small number of patients in class I, the differences between class I and class II patients are more doubtful than the differences between class III and class II patients, or between class III and class I + II patients. RF 001 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 002 BOWMAN BH SCIENCE 164 325 969 003 CONOVER JH LANCET 1 1122 973 004 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 005 DANES BS LANCET 1 1061 968 006 DANES BS J EXP MED 129 775 969 007 DANES BS LANCET 2 437 969 008 DANES BS J EXP MED 136 1313 972 009 DANES BS AM J HUM GENET 23 297 971 010 DANES BS NATURE 222 685 969 011 FLENSBORG EW ACTA PAEDIATR SCAND 61 383 972 012 LAURELL CB CLIN CHEM 2 99 956 013 LYKKEGAARD E PROC EWGCF 4TH ANNU MTG 973 014 LYKKEGAARD E DAN MED BULL 22 177 975 015 LYKKEGAARD E DAN MED BULL 22 175 975 016 LYKKEGAARD E DAN MED BULL 21 232 974 017 NORMAN AP PROC EWGCF 2ND ANNU MTG 971 018 POGUE RE MINN MED 52 1551 969 019 RASK MADSEN J PROC EWGCF 4TH ANNU MTG 973 CT 1 LYKKEGAARD E DAN MED BULL 22 177 975 2 LYKKEGAARD E DAN MED BULL 22 175 975 3 WEEKE B DAN MED BULL 23 155 976 4 DANES BS CLIN GENET 11 83 977 5 DANES BS CLIN GENET 13 327 978 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 WINGE P DAN MED BULL 29 358 982 8 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 75079 RN 00245 AN 75131670 AU Sorenson-R-L. Shank-R-D. Elde-R-P. TI Immunoperoxidase demonstration of human serum globulin binding to islet tissue. SO Diabetes. 1975 Feb. 24(2). P 230-7. MJ DIABETES-MELLITUS-INSULIN-DEPENDENT: me. IMMUNOGLOBULINS: me. ISLANDS-OF-LANGERHANS: me. PEROXIDASES. MN ADOLESCENCE. ADULT. ANIMAL. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. DIABETES-MELLITUS: ci. GOATS: im. HUMAN. IGG. INFANT. ISLANDS-OF-LANGERHANS: pa. PROTEIN-BINDING. RATS. STREPTOZOTOCIN. AB Sera from juvenile diabetic and nondiabetic controls were tested, using the immunoperoxidase method, for the presence of islet-binding immunoglobulins. All diabetic sera tested contained an islet-binding protein and on the average the sera were more positive than age- matched controls. Normal adult sera are undifferentiated from juvenile diabetic sera. Most sera from children less than two years of age did not bind to islet tissue and sera from cystic fibrosis patients had a markedly diminished ability to bind to islet tissue. The binding protein appears to be an immunoglobulin which selectively reacts with elements of the islet beta cell. RF 001 GEPTS W DIABETES 14 519 965 002 LECOMPTE PM ARCH PATHOL 66 450 958 003 MEYENBURG HV SCHWEIZ MED WOCHENSCHR 21 554 940 004 RENOLD AE CIBA FOUND COLLOQUIA ENDOCRIN 15 122 964 005 LECOMPTE PM DIABETES 15 586 966 006 GRODSKY GM DIABETES 14 396 965 007 KOREAKOVA L Z MIKROSK ANAT FORSCH 85 85 972 008 NERUP J DIABETES 20 424 971 009 UNGAR B LANCET 2 415 968 010 IRVINE WJ LANCET 2 163 970 011 PETTIT MD J CLIN ENDOCRINOL METAB 21 209 960 012 BLIZZARD RM CLIN EXP IMMUNOL 2 19 967 013 ANON LANCET 2 193 970 014 HUNG W MED ANN DC 39 487 970 015 KOGUT MD J PEDIATR 81 307 972 016 LANDING BH J CLIN ENDOCRINOL METAB 23 119 962 017$ DECKERT T ACTA MED SCAND 474 30 967 018 NERUP J ACTA ENDOCRINOL 72 279 973 019 PAV J LANCET 2 221 963 020 MANCINI AM LANCET 1 1189 965 021 CHETTY MP LANCET 1 67 965 022 NAKANE PK J HISTOCHEM CYTOCHEM 14 929 966 023 ELDE RP THESIS 974 024 SNEDECOR GW STATISTICAL METHODS 967 025 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 026 DESBUQUOIS B J CLIN ENDOCRINOL METAB 33 732 971 028 GOMORI G AM J CLIN PATHOL 20 665 950 029 GRIMELIUS L IN: BROLIN SE 964 030 HELLERSTROM C IN: BROLIN SE 964 CT 1 CHRISTY M CLIN ENDOCRINOL METAB 6 305 977 2 MONIER JC LYON MED 237 11 977 3 APPEL MC DIABETOLOGIA 15 327 978 4 LERNMARK A N ENGL J MED 299 375 978 5 KALDANY A DIABETES 28 102 979 6 BUSCHARD K DAN MED BULL 32 139 985 PN 75080 RN 00246 AN 75209073 AU Schoni-M. Kaiser-D. Drack-E. Axmacher-U. TI Excretion of trypsin-like activity, electrolytes and protein in mixed and parotid saliva of patients with cystic fibrosis of the pancreas. SO Eur-J-Clin-Invest. 1975 Apr. 5(2). P 153-8. MJ CYSTIC-FIBROSIS: me. SALIVA: me. TRYPSIN: me. MN CALCIUM: an. CHILD. CHILD-PRESCHOOL. HUMAN. HYDROGEN-ION-CONCENTRATION. PAROTID-GLAND: se. PROTEINS: an. SALIVA: an. AB The esterolytic activity of mixed and parotid saliva in cystic fibrosis (CF) patients and normal subjects was determined using BAEE (alpha-Benzoyl-1-arginine-ethylester) as the substrate. Using soybean- trypsin-inhibitor the trypsin-like activity (TLA) was measured and plotted as a function of parotid flow rate. In addition calcium, protein and pH were determined. Trypsin-like activity in mixed and parotid saliva showed large individual variations in CF and normal children. In parotid saliva we could not find any significant difference, whereas a reduction of TLA in mixed saliva of CF patients was observed. The fact that our normal values fell within the range of heterozygotes reported by Rao et al. (19), makes their hypothesis of a close relationship between reduced TLA and the genetic defect very doubtful. Protein, calcium and pH increased with augmented salivation and no difference between CF patients and normal age matched children could be found except for the pH at a flow rate above 0.75 ml/min per m2 body surface where significantly lower pH values resulted. The relevance of reduced TLA to the pathogenesis of cystic fibrosis is discussed. RF 001 BOWMAN BH SCIENCE 167 871 970 002 CONOVER JH PEDIATR RES 7 220 973 003 FREY EK KALLIKREIN KININ SYSTEM INH 968 004 GIBSON LE PEDIATRICS 48 695 971 005 GUGLER EC J PEDIATR 71 585 967 007 HEIDLAND A KLIN WOCHENSCHR 50 959 972 008 KAISER D PEDIATR RES 5 167 971 009 KAISER D IN: MANGOS JA 247 973 010 KAISER D PFLUEGERS ARCH 349 63 974 011 KASSELL B IN: PERTINAUM GE 19 853 970 012 KREUSSER W EUR J CLIN INVEST 2 398 972 013 LOCKHART LH SOUTH MED J 61 1356 968 014 LOURIE RS AM J DIS CHILD 65 455 943 015 LOWRY OH J BIOL CHEM 193 265 951 016 MANDEL ID AM J DIS CHILD 110 646 965 017 MANGOS JA PEDIATR RES 2 378 968 018 OUCHTERLONY O ACTA PATH MICROBIOL SCAND 26 507 949 019 RAO GJS J PEDIATR 80 573 972 020 RECTOR FC J CLIN INVEST 44 278 965 021 RICK W METHODS IN ENZYMATIC ANALYSIS 807 965 022 SCHNEYER LH PHYSIOL REV 52 3 972 023 SPOCK A PEDIATR RES 1 173 967 024 TRAUTSCHOLD I HOPPE SEYLERS Z PHYSIOL CHEM 325 48 961 025 WALLACH D EUR J BIOCHEM 21 433 971 026 WERLE E BIOCHEM Z 323 279 952 027 WERLE E ARZNEIMITTEL FORSCH 18 281 964 028 WERLE E ARCH INT PHARMACODYN THER 131 257 961 029 WERLE E BIOCHEM Z 290 129 937 030 WERLE E BIOCHEM Z 301 329 939 031 YOUNG JA PFLUEGERS ARCH 319 185 970 CT 1 GRIMMEL K ACTA HEPATOGASTROENTEROL 23 334 976 2 HAUBRICH J ARCH OTO RHINO LARYNGOL 213 1 976 3 MUNZEL M ARCH OTO RHINO LARYNGOL 213 209 976 4 CHILLA R ARCH OTO RHINO LARYNGOL 214 367 977 5 RAO GJS ENZYME 23 314 978 6 SLAVOTINEK AH AUST NZ J MED 11 91 981 7 KATZ S CELL CALC 5 421 984 8 KONARSKA L J CLIN CHEM CLIN BIOCHEM 23 337 985 PN 75081 RN 00247 AN 76043991 AU Berry-H-K. TI Screening for genetic disorders. SO Fed-Proc. 1975 Nov. 34(12). P 2134-9. (REVIEW). MJ MASS-SCREENING. METABOLISM-INBORN-ERRORS: di. MN LIPOIDOSIS: di. AMINO-ACID-METABOLISM-INBORN-ERRORS: di. BIOLOGICAL-ASSAY. CHILD. CHROMATOGRAPHY-THIN-LAYER. CYSTIC-FIBROSIS: di. DIAGNOSIS-LABORATORY. GALACTOSEMIA: di. HUMAN. HYPOTHYROIDISM: di. INFANT. INFANT-NEWBORN. JEWS. MAPLE-SYRUP-URINE-DISEASE: di. METABOLISM-INBORN-ERRORS: fg, me. MUSCULAR-DYSTROPHY: di. REVIEW. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. EX The past 50 years in developed countries have seen a steady decline in infant mortality from diseases associated with infections and malnutrition. During the period the contribution from genetic diseases and congenital malformations has risen significantly. Genetically oriented screening programs have a high potential to contribute to the overall improvement of health in both developed and underdeveloped countries. In this review only procedures for screening for hereditary biochemical disorders will be considered. Criteria for selection of genetic traits to be screened, the schedule for screening, specimens for screening, the screening of newborn infants, the testing of older infants, the testing of high-risk infants and children, the screening of families with genetic diseases, and screening for carriers in selected populations are discussed. RF 001 ALLAN JD AM J DIS CHILD 126 22 973 002 AMBROSE JA CLIN CHEM 15 15 969 003 AMBROSE JA APPLIC BASIC PRIN FLUOROMETRY 972 004 BERRY HK CLIN CHEM 5 603 959 006 BERRY HK AM J DIS CHILD 129 165 975 007 BERRY HK CLIN CHEM 14 1033 968 008 BEUTLER E J LAB CLIN MED 68 137 966 009 BREMER HJ MONATSSCHR KINDERHEILKD 117 32 969 010 CHILDS B IN: SUTTON HE 3 972 011 DUSSAULT JH UNION MED CAN 102 2062 973 012 MITTMAN C ISR J MED SCI 9 1311 973 013 ANON GENETIC SCREENING PROG PRIN A 975 014 GHADIMI H IN: NYHAN WL 265 975 015 GREEN MN PEDIATRICS 41 989 968 016 GUTHRIE R PEDIATRICS 32 338 963 017 GUTHRIE R BIRTH DEF ORIG ART SER 4 92 968 018 HILL JB CLIN CHEM 11 451 965 019 IRELAND JT ANN CLIN BIOCHEM 9 129 972 020 JELLUM E CLIN CHEM 18 800 972 021 LABERGE C AM J HUM GENET 21 36 969 022 LEVY HL N ENGL J MED 288 1299 973 023 LEVY HL PEDIATRICS 49 825 972 024 LEVY HL N ENGL J MED 291 1214 974 025 LIGHT IJ AM J DIS CHILD 112 229 966 026 LIGHT IJ AM J DIS CHILD 125 243 973 027 LOWDEN JA CLIN CHEM 19 1345 973 028 MCKUSICK VA IN: CARSON NAJ 179 971 029 MENKES JH PEDIATRICS 49 218 972 030 MILUNSKY A PRENATAL DIAGNOSIS OF HEREDIT 973 031 MOTULSKY AG SCIENCE 185 653 974 032 NITOWSKY HM AM J MENT DEFIC 77 538 973 033 PARTINGTON MW BIOL NEONATE 18 121 971 034 PASIEKA AE CLIN BIOCHEM 2 41 968 035 POPKIN JS LANCET 1 721 974 036 PROSSER R ARCH DIS CHILD 49 597 974 037 OBRIEN JS N ENGL J MED 283 15 970 038 RAINE DN LANCET 2 996 974 039 SCHMIDT L IN: NYHAN WL 675 975 040 SCRIVER CR PEDIATRICS 54 616 974 041 SCRIVER CR CLIN BIOCHEM 6 142 973 042 SCRIVER CR AMINO ACID METABOL DISORDERS 973 043 SCRIVER CR CAN MED ASSOC J 108 1111 973 044 SHIH VE LAB TECH FOR DETECTION HEREDI 973 045 STEPHAN U PEDIATRICS 55 35 975 046 THALHAMMER O ACTA UNIV CAROL MED MONOGR 56 79 973 047 THOMAS GH PEDIATR CLIN NORTH AM 20 105 973 048 TOCCI PM IN: NYHAN WL 461 967 049 ANON WHAT ARE FACTS GENET DISEASE 9 975 050 WILLIAMS KM AUST J BIOL SCI 26 831 973 051 ZELLWEGER H PEDIATRICS 55 30 975 CT 1 MEE JML BIOMED MASS SPECTROMET 4 178 977 2 NARAYANAN KR ARCH TOXICOL 38 61 977 3 PONGOR S ACTA BIOCHIM BIOPHYS 13 117 978 4 MACHILL G BIOL ZENTRALBL 100 449 981 PN 75082 RN 00248 AN 76189166 AU Hook-E-B. Bonenfant-R. TI Parathenar palmar pattern: a dermatoglyphic sign of possible clinical significance. SO Hum-Hered. 1975. 25(6). P 509-12. MJ CYSTIC-FIBROSIS: fg. DERMATOGLYPHICS. HEART-DEFECTS-CONGENITAL: fg. MN FEMALE. HUMAN. MALE. AB The frequency of a 'double proximal axial triradius' (DPAT), which results in a palmar parathenar pattern, has been reported to be increased in those with ventricular septal defect (BSD). We scored the presence of this pattern in 313 patients with congenital heart defects (including 86 with isolated VSD), 176 with cystic fibrosis (CF) or relatives of those with CF, and 333 with no known clinical disease who were unrelated to individuals with CF. The frequencies in these three groups were 1.6, 2.3, and 0.6%, respectively. None of those with isolated VSD defect had a DPAT. All five individuals in the heart defect group with a DPAT had pulmonic stenosis. Of the total of eleven individuals in our series with DPAT patterns, eight were affected only on the right hand. The frequency of DPAT in individuals with no known disease in this series was 4/406=1.0%, more than double the frequency in a control series, published previously by DAVID, 5/1,129=0.4%. If the 73 relatives of those with CF are excluded from our own 'control' group, the frequency of DPAT in this group is then 0.6%, closer to the results in DAVID's control series. RF 001 DAVID TJ J PEDIATR 79 343 971 002 CUMMINS H FINGER PRINTS PALMS AND SOLES 103 961 003 HOOK EB TERATOLOGY 9 233 974 CT 1 DENNIS RLH ANN HUM BIOL 5 269 978 2 DAVID TJ J MED GENET 18 344 981 PN 75083 RN 00249 AN 75210370 AU Altland-K. Schmidt-S-R. Kaiser-G. Knoche-W. TI Demonstration of a factor in the serum of homozygotes and heterozygotes for cystic fibrosis by a non-biological technique. SO Humangenetik. 1975 Jul 23. 28(3). P 207-16. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. MN BLOOD-PROTEIN-ELECTROPHORESIS: mt. COMPLEMENT-3. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IGG. ISOELECTRIC-POINT. MOLECULAR-WEIGHT. AB A factor has been isolated from serum of homozygotes and obligate heterozygotes for cystic fibrosis using isoelectric focusing and disc electrophoresis as analytical methods. The factor is focused within an IgG-fraction with an isoelectric point of pH 8 to 9 but differs from IgG in its lower molecular weight. It is thus similar to, if not identical with, the ciliary dyskinesia factor. RF 001 AWDEH ZL NATURE 219 66 968 002 BESLEY GTN J MED GENET 6 278 969 003 BOKISCH VA J CLIN INVEST 49 2427 970 004 BOWMAN BH LANCET 1 404 974 005 BOWMAN BH SCIENCE 164 325 969 006 BOWMAN BH SCIENCE 167 871 970 007 CONOVER JH PEDIATR RES 7 224 973 008 CONOVER JH PEDIATR RES 7 220 973 009 CONOVER JH LIFE SCI 14 253 974 010 CRAWFURD MDA IN: LAWSON D PROC 5TH INT CF 42 969 011 DANES BS J EXP MED 137 1538 973 012$ HJERTEN S ARCH BIOCHEM BIOPHYS SUPPL 1 147 962 013 LIEBERMAN J AM REV RESPIR DIS 109 399 974 014 LIEBERMAN J AM REV RESPIR DIS 111 100 975 015 MARCHI AG HELV PAEDIATR ACTA 28 427 973 016 REISFELD RA NATURE 195 281 962 017 RAO GJS PEDIATR RES 8 684 974 018 RAO GJS SCIENCE 177 610 972 019 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 020 SPOCK A PEDIATR RES 1 173 967 021 VALLOTA EH J EXP MED 137 1109 973 022 VESTERBERG O SCIENCE TOOLS 20 22 973 023 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 WILSON GB PEDIATR RES 10 1001 976 2 FORSTNER G CLIN CHIM ACTA 70 459 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 THOMAS JM PEDIATR RES 11 1148 977 5 THOMAS JM PEDIATR RES 11 138 977 6 WILSON GB PEDIATR RES 11 986 977 7 TULLY GW PEDIATR RES 13 1078 979 8 JAKEL HP BIOL ZENTRALBL 98 55 979 9 ALTLAND K ELECTROPHORESIS 2 49 981 10 NEVIN GB HUM GENET 56 387 981 11 HALLINAN FM CLIN CHIM ACTA 117 103 981 12 MCNEELY MC PEDIATR RES 16 21 982 13 ALTLAND K CLIN CHEM 28 1000 982 14 SHIBATA K HUM GENET 61 210 982 15 JAMIESON A HUM GENET 70 168 985 PN 75084 RN 00250 AN 76045098 AU Cunningham-D-G. Gatti-W-M. Eitenmiller-A-M. Van-Gorder-P-N. TI Cystic fibrosis: involvement of the ear, nose, and paranasal sinuses. SO Ill-Med-J. 1975 Oct. 148(4). P 470-1, 474. MJ CYSTIC-FIBROSIS: co. DEAFNESS: et. NASAL-POLYPS: et. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FRONTAL-SINUS: ab. HUMAN. INFANT. PSEUDOMONAS-AERUGINOSA: ip. SINUSITIS: mi. EX Twenty-six patients with cystic fibrosis all exhibited disease of the paranasal sinuses, consisting of opacification of the maxillary sinuses either alone or as part of a parasinusitis. Symptoms of sinusitis seldom occurred and treatment was not generally required. Agenesis or delayed development of frontal sinuses was also seen in the majority of patients. Nasal polyps occurred in 27% of the patients, all of whom were over five years of age. Excluding those patients under five years of age, none of whom had polyps, the incidence was 36%. In general, polyps were not observed in those patients with severe disease. Mild degrees of conductive hearing loss were noted in the younger age groups especially, but no instance of severe hearing loss or nerve conduction disturbance was encountered. Such mild degrees of hearing loss may merely reflect the application of standards too stringent for this age group. Throat and nose cultures on 26 patients revealed pseudomonas aeruginosa in eighteen cases, reflecting the flora of the tracheobronchial tree. Other organisms were encountered on an unpredictable basis with findings consistent with those anticipated in this age group of patients. RF 001 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 002 SHWACHMAN H PEDIATRICS 46 335 970 003 SHWACHMAN H AM J DIS CHILD 96 6 958 004 GIBSON LE PEDIATRICS 23 545 959 005 DOGGETT RG INFECT IMMUN 6 628 972 006 WILLIAMS RJ J MED MICROBIOL 6 409 972 007 CUNNINGHAM DG ILL MED J 145 493 974 008 JERGER J ARCH OTOLARYNGOL 93 111 971 009 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 010 PENNINGTON CL ARCH OTOLARYNGOL 63 576 956 011 SHWACHMAN H PEDIATRICS 30 389 962 012 KULCZYCKI LL ARCH OTOLARYNGOL 92 54 970 CT 1 FRIED MP LARYNGOSCOPE 94 192 984 2 DAVID TJ J ROY SOC MED 79 23 986 PN 75085 RN 00251 AN 76215175 AU Heinrich-H-C. TI Clinical aspects of iron absorption and turnover. pp. 34-61. SO In: Kief H, et.al., ed. Iron metabolism and its disorders. Amsterdam, Excerpta Medica, 1975. W3 EX89 no.366 1975. MJ INTESTINAL-ABSORPTION. IRON: me. MN ADMINISTRATION-ORAL. CYSTIC-FIBROSIS: me. DIGESTION. FECES: an. GASTRECTOMY. GASTRIC-MUCOSA: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INTESTINAL-MUCOSA: me. IRON: ad, df. MALABSORPTION-SYNDROMES: me. MONOGRAPH. THALASSEMIA: fg, me. AB The diagnostic 10 micro-mole (0.56 mg) 59Fe2 absorption whole body retention test (Fig. 1) has now been in use for 10 years for the detection of depleted iron stores in subjects with iron deficiency (Heinrich et al., 1966; Heinrich and Bartels, 1967). Further studies were necessary to investigate the clinical reliability of increased 59Fe2 absorption in patients with erythroblastic hyperplasia, which may control the regulation of iron absorption by iron stores. The relationship between iron absorption and iron stores was studied in pancreatic insufficiency to reinvestigate the effect of pancreatic secretion on iron absorption. Iron absorption in patients with gluten-sensitive enteropathy was investigated in order to correlate the impairment of iron absorption with the degree of villous atrophy and to study its recovery on a gluten-free diet. Dietary iron absorption was measured in patients with gastric mucosal atrophy and partial gastrectomy to investigate the extent to which iron malabsorption is responsible for the high incidence of iron deficiency in such patients. The combination of the diagnostic 59Fe2 absorption test and measurement of the whole body 59Fe2 elimination rate is a useful routine procedure for the detection and quantification of gastrointestinal or urogenital blood loss as a cause of iron deficiency. RF 001 BENDER-GOETZE C Z KINDERHEILK 118 283 975 002 CONRAD ME IN: HALLBERG L 87 970 003 HEINRICH HC IN: HALLBERG L 213 970 004 HEINRICH HC LANCET 2 1256 970 005 HEINRICH HC KLIN WOCHENSCHR 45 553 967 006 HEINRICH HC BIOCHEM MED 5 472 971 007 HEINRICH HC KLIN WOCHENSCHR 49 819 971 008 HEINRICH HC KLIN WOCHENSCHR 49 819 971 009 HEINRICH HC KLIN WOCHENSCHR 44 827 966 010 HEINRICH HC Z KINDERHEILK 115 1 973 011 KRAUSE U IN: HALLBERG L 505 970 012 BAIRD IM Q J MED 28 21 959 013 BAIRD IM Q J MED 28 21 959 014 NADLER SB SURGERY 51 224 962 015 SMITH MD CLIN SCI 16 23 957 016 TURNBULL A IN: JACOBS A 369 974 017 WEINFELD A IN: HALLBERG L 329 970 CT 1 COOK JD CLIN HAEMATOL 6 567 977 2 WORWOOD M SEM HEMATOL 14 3 977 3 HEINRICH HC KLIN WSCHR 55 1043 977 4 HEINRICH HC KLIN WSCHR 55 587 977 5 HEINRICH HC KLIN WSCHR 55 595 977 6 BARTELS U SCAND J GASTROENTEROL 13 649 978 7 HEINRICH HC KLIN WSCHR 56 1057 978 8 HEINRICH HC MED KLIN 73 1335 978 9 HEINRICH HC MED KLIN 73 1163 978 10 HEINRICH HC DTSCH MED WSCHR 103 753 978 11 GOLTNER E GYNAKOLOGE 12 52 979 12 HEINRICH HC MED WELT 30 89 979 13 HEINRICH HC KLIN WSCHR 57 187 979 14 HEINRICH HC ACTA HAEMATOL 62 56 979 15 HEINRICH HC MED KLIN 74 1225 979 16 HEILMANN E DTSCH MED WSCHR 104 1325 979 17 HEINRICH HC DTSCH MED WSCHR 104 1496 979 18 GOLTNER E MED KLIN 75 44 980 19 MCLAREN GD CRC CRIT REV CLIN LAB SCI 19 205 983 20 MANDELBAUM AY KLIN MED 61 121 983 21 DOMMISSE J S AFR MED J 64 1047 983 22 HUEBERS HA BLOOD 64 763 984 23 FINCH CA CLIN PHYSIOL BIOCHEM 4 5 986 24 HUEBERS HA PHYSIOL REV 67 520 987 PN 75086 RN 00252 AN 76045374 AU Chandra-R-K. TI Alpha-fetoprotein in serum and amniotic fluid in the diagnosis of neonatal hepatitis syndrome, Indian childhood cirrhosis, cystic fibrosis, ataxia telangiectasia and spina bifida. SO Indian-Pediatr. 1975 Jul. 12(7). P 545-8. MJ ALPHA-FETOPROTEINS: an. AMNIOTIC-FLUID: an. FETAL-PROTEINS: an. MN ATAXIA-TELANGIECTASIA: di. CHILD. CYSTIC-FIBROSIS: di. FEMALE. HEPATITIS: di. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: di. LIVER-CIRRHOSIS: di. PREGNANCY. PRENATAL-DIAGNOSIS. SPINA-BIFIDA: di. AB Alpha-fetoprotein (A.F.P.) was detected and quantitated by the methods of immunodiffusion in agarose, counterimmunoelectrophoresis and radioimmunoassay. Serum A.F.P. level was raised in patients and siblings of Indian childhood cirrhosis, cystic fibrosis homozygotes heterozygotes, neonatal hepatitis and ataxia telangiectasia. It was mildly elevated in biliary atresia. In women with previous history of a child with severe neural tube defect, raised amniotic fluid levels of A.F.P. were associated with anencephaly or spina bifida. The diagnostic utility of A.F.P. in pediatrics, especially in genetic counselling, is discussed. RF 001 GITLIN D J CLIN INVEST 45 1826 966 002 CHANDRA RK MED CHIR DIG 3 63 974 003 CHANDRA RK ARCH DIS CHILD 48 157 973 004 NAYAK NC LANCET 1 86 972 008 RAMALINGASWAMI V IN: POPPER H 3 222 970 009 ABELEV GI TRANSPLANTATION 1 174 963 010 TATARINOV YS VOPR MED KHIM 11 20 965 011 CHANDRA RK TECH REP SERIES NO 24 INDIAN 973 012 CHANDRA RK INDIAN J MED RES 61 466 973 014 BROCK DJH LANCET 2 197 972 015 ALLAN LD LANCET 2 522 973 CT 1 CHANDRA RK INT ARCH ALLERGY APPL IMMUNOL 53 180 977 2 ZELTZER PM J PEDIATR SURG 13 381 978 3 MIZEJEWSKI GJ CLIN CHEM 28 1207 982 4 MIZEJEWSKI GJ PEDIATR RES 17 47 983 PN 75087 RN 00253 AN 76024261 AU Baran-D. Dachy-A. Klastersky-J. TI Concentration of gentamicin in bronchial secretions of children with cystic fibrosis of tracheostomy. (Comparison between the intramuscular route, the endotracheal instillation and aerosolization). SO Int-J-Clin-Pharmacol-Biopharm. 1975 Oct. 12(3). P 336-41. MJ CYSTIC-FIBROSIS: me. GENTAMICINS: me. RESPIRATORY-TRACT-INFECTIONS: dt. SPUTUM: an. TRACHEOTOMY. MN ADOLESCENCE. AEROSOLS. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: mi, co. GENTAMICINS: ad, an, bl. HUMAN. INFANT. INJECTIONS-INTRAMUSCULAR. INTUBATION-INTRATRACHEAL. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: dt. AB The levels of gentamicin in blood and in secretions of the tracheobronchial tree were measured in 14 children (8 with cystic fibrosis and 6 with tracheostomy) in a cross over fashion after the administration of 40 mg of gentamicin by aerosol or by endotracheal injection. High levels of gentamicin (greater than 20 mug/ml) within the bronchial secretions were observed in 7 children after aerosolization and in 11 children after endotracheal instillation. Corresponding blood levels were low (less than 3 mug/ml) in all patients and no detectable levels were found in 10 children after aerosolization and in 6 children after endotracheal instillation. No significant differences were observed between children with cystic fibrosis and those with tracheostomy. Since intramuscular injection of gentamicin (a single dose of 1.5 mg/kg) resulted in low levels of gentamicin within the bronchial secretions (less than 2 mug/ml in 10 patients, among whom 4 had undetectable levels); it is concluded that the administration of an antibiotic such as gentamicin, directly to the trachea by endotracheal injection or by aerosolization might prove to be helpful when the infection is confined mainly to the tracheo-bronchial tree. RF 001 DAVIS WW APPL MICROBIOL 22 659 971 002 GATMAITAN BG AM J MED SCI 260 90 970 003 KLASTERSKY J J INFECT DIS 129 187 974 004 KLASTERSKY J CHEST 61 117 972 005 KLASTERSKY J CHEST 65 650 974 006 LOWBURY EJL J MED MICROBIOL 3 39 970 007 MARKS MI J PEDIATR 79 822 971 008 MEARNS MB ARCH DIS CHILD 47 902 972 009 PENNINGTON JE AM J MED 55 155 973 010 PENNINGTON JE J INFECT DIS 128 63 973 011 PINES A BR MED J 1 663 970 012 RAMIREZ JR CHEST 58 352 970 013 ROSE HD J CLIN PHARMACOL 10 274 970 014 SWIFT DL IN: LAWSON D PROC 5TH INT CF 969 CT 1 LI PK CLIN CHEM 26 1631 980 2 TABACHNIK E AM REV RESPIR DIS 122 97 980 3 LOURENCO RV ARCH INTERN MED 142 2299 982 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 5 MAXWELL D J ANTIMICROB CHEMOTHER 11 203 983 6 COURCOL RJ INT J CLIN PHARMACOL RES 5 87 985 7 MENDELMAN PM AM REV RESPIR DIS 132 761 985 8 AGUILERA D PATHOL BIOL (PARIS) 34 657 986 9 STOUT SA DRUG INTEL CLIN PHARM 21 322 987 PN 75088 RN 00254 AN 75114429 AU Arad-I. Lafair-J-S. Shapira-E. TI Application of the cilia dyskinesis test in the diagnosis of cystic fibrosis in an adolescent. SO Isr-J-Med-Sci. 1975 Jan. 11(1). P 10-4. MJ CILIA: ph. CYSTIC-FIBROSIS: di. MN ACID-BASE-EQUILIBRIUM. ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg, mi, bl. ELECTROLYTES: an. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IGA: an. IGG: an. IGM: an. MALE. PEDIGREE. RABBITS. RESPIRATORY-FUNCTION-TESTS. SERUM-GLOBULINS: du. SPUTUM: mi. SWEATING. TRACHEA: ph. AB In certain cases of obscure chronic lung disease, the possibility of a genetic disorder, such as cystic fibrosis of the pancreas, should be considered. When the genetic defect fails to cause pancreatic involvement, the partial expression of cystic fibrosis is encountered. Elevation of sweat electrolyte levels is not sufficient to confirm the diagnosis of cystic fibrosis in adults since normal levels vary widely in both adolescents and adults. Other tests should, therefore, be utilized. The cilia dyskinesis test is positive in both homozygous and heterozygous patients with cystic fibrosis. Utilization of this technique in a family study confirmed the diagnosis of partial expression of cystic fibrosis in an adolescent female. RF 001 DI SANTAGNESE PA MOD PROBL PEDIATR 10 10 967 002 DI SANTAGNESE PA IN: NELSON WE 771 964 003 ANDERSON CM MOD PROBL PEDIATR 10 344 967 004 DI SANTAGNESE PA MOD PROBL PEDIATR 10 135 967 005 SHWACHMAN H PEDIATRICS 36 689 965 006 SHWACHMAN H CF CLUB ABST 7 966 007 SPOCK A PEDIATR RES 1 173 967 008 SHAPIRA E J LAB CLIN MED 77 877 971 009 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 010 GRAND RJ J PEDIATR 70 346 967 011 GRAND RJ J PEDIATR 70 357 967 012 PEREIRA C J PEDIATR 78 1036 971 013 LOBECK CC J PEDIATR 62 393 963 014 TAUSSIG LM J PEDIATR 79 1034 971 015 MAY CD AM J DIS CHILD 109 2 965 016 CABANEL G MOD PROBL PEDIATR 10 284 967 017 CRAWFURD MDA IN: LAWSON D PROC 5TH INT CF 42 969 018 BESLEY GTN J MED GENET 6 278 969 019 IACOCCA VF J PEDIATR 79 508 971 020 BOWMAN BH SCIENCE 164 325 969 021 CHERRY JD J PEDIATR 79 937 971 022 LEVIN S ISR J MED SCI 2 333 966 023 MANCINI G IMMUNOCHEMISTRY 2 235 965 CT 1 WIEDERHOLD ML ANNU REV BIOPHYS BIOENG 5 39 976 2 GABRIDGE MG PEDIATR RES 13 31 979 PN 75089 RN 00255 AN 76068923 AU Parsons-R-L. Paddock-G-M. TI Absorption of two antibacterial drugs, cephalexin and co-trimoxazole, in malabsorption syndromes. SO J-Antimicrob-Chemother. 1975. 1(3 Suppl). P 59-67. MJ CEPHALEXIN: me. MALABSORPTION-SYNDROMES: me. SULFAMETHOXAZOLE: me. TRIMETHOPRIM: me. MN CELIAC-DISEASE: me. CHOLESTYRAMINE: ae. CYSTIC-FIBROSIS: me. DIVERTICULOSIS: me. DRUG-COMBINATIONS. CROHN-DISEASE: me. HUMAN. INTESTINAL-ABSORPTION. MALABSORPTION-SYNDROMES: ci. TIME-FACTORS. AB Plasma concentrations and urinary excretion of cephalexin, trimethoprim and sulphamethoxazole (co-trimoxazole) were measured in normal subjects and in patients with coeliac disease, small bowel diverticulosis, Crohn's disease, cystic fibrosis (cephalexin only) and after administration of cholestyramine. The absorption of cephalexin was improved in coeliac disease diverticulosis and cystic fibrosis. In Crohn's disease the peak plasma level was reduced and delayed. The absorption of both trimethoprim and sulphamethoxazole was increased in all three conditions. However there was a disproportionate increase in the plasma level of trimethoprim in coeliac disease and diverticulosis when compared with that of sulphamethoxazole. In Crohn's disease there was a threefold increase in the peak plasma level of sulphamethoxazole. After cholestyramine administration there was malabsorption of all three drugs. RF 001 BRODIE BB ABSORPTION DISTRIBUTION DRUGS 16 964 002 CHANARIN I BR MED J 1 651 972 003 DAVIES JA POSTGRAD MED J 46 16 970 004 DAVIS AE AUSTRALAS ANN MED 17 63 968 005 GROVE DC ASSAY METH OF ANTIBIOTICS 955 006 HASHIM SA PROC SOC EXP BIOL MED 106 173 961 007 MARGET W POSTGRAD MED J 47 54 971 008 MATILLA MJ ARZNEIMITTEL FORSCH 23 583 973 009 PARSONS RL PROC INT CONG CHEMOTHER 8TH 1 499 974 010 PARSONS RL PROC INT CONG CHEMOTHER 8TH 1 534 974 011 PARSONS RL BR J CLIN PHARMACOL 1 348 974 012 PARSONS RL J ANTIMICROB CHEMOTHER 1 39 975 013 RIEDER J CHEMOTHERAPY 17 1 972 014 SCHWARTZ DE CHEMOTHERAPY (SUPPL) 14 22 969 015 PARSONS RL BR J CLIN PHARMACOL 1 348 974 CT 1 SCHNEIDER RE BR MED J 2 794 976 2 PARSONS RL BR MED J 1 103 977 3 PARSONS RL CLIN PHARMACOKINET 2 45 977 4 AMOLO JGC E AFR MED J 54 565 977 5 KASANEN A ANN CLIN RES 10 5 978 6 COOKE DM CHEMOTHERAPY 25 356 979 7 WILSON K METH FIND EXP CLIN PHARMACOL 3 189 981 8 GIDUMAL R MT SINAI J MED 48 7 981 9 EVARD D GASTROENTEROL CLIN BIOL 7 398 983 10 GRIFFITH RS POSTGRAD MED J 59 16 983 11 WELLING PG CLIN PHARMACOKINET 9 404 984 12 WELLING PG J CLIN HOSP PHARM 9 163 984 PN 75090 RN 00256 AN 75114793 AU Roussel-P. Lamblin-G. Degand-P. TI Heterogeneity of the carbohydrate chains of sulfated bronchial glycoproteins isolated from a patient suffering from cystic fibrosis. SO J-Biol-Chem. 1975 Mar 25. 250(6). P 2114-22. MJ CARBOHYDRATES: an. CYSTIC-FIBROSIS: me. GLYCOPROTEINS: an. SPUTUM: an. SULFATES: an. MN ACETAMIDES: an. AMINO-ACIDS: an. BLOOD-GROUPS. BOROHYDRIDES. GALACTOSE: an. GLYCOPROTEINS: ip. HUMAN. NEURAMINIC-ACIDS: an. OLIGOSACCHARIDES: an. SUPPORT-U-S-GOVT-P-H-S. AB Sulfated glycoproteins having blood group H activity were isolated from the sputum of a child suffering from cystic fibrosis, by reduction of the fibrillar mucus, chromatography on ECTEOLA- cellulose, and gel filtration on Sepharose 4B. The sulfated glycoproteins were degraded with alkaline borohydride, and the degradation products were fractionated by chromatography on ion exchange resins and by gel filtration. The carbohydrate chains thus obtained have a wide heterogeneity with regard to acidity and molecular size. The neutral chains contain blood group H active oligosaccharides and incomplete chains as short as 1 residue of 2- acetamido-2-deoxy-D-galactose. The minimal size of the neuraminic acid-containing chains is less than that of the sulfated chains, which increases with the degree of sulfation. The sulfate groups are linked at C-6 at the D-galactose residues. RF 001 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 002 HAVEZ R CLIN CHIM ACTA 17 281 967 003 HAVEZ R EXP ANNU BIOCHIM MED 32 121 973 004 HAVEZ R CLIN CHIM ACTA 17 463 967 005 LAMB D J PATHOL 98 213 969 006 DEGAND P BIOCHIM BIOPHYS ACTA 320 318 973 007 DEGAND P CLIN CHIM ACTA 50 223 974 008 LAMBLIN G BIOCHIM BIOPHYS ACTA 322 372 973 009 ASSELAIN R COLLOQ INT PATHOL THORAC LILL 101 968 010 ROUSSEL P COLLOQ INT PATHOL THORAC LILL 155 968 011 LAMBLIN G CLIN CHIM ACTA 36 329 972 012 ROUSSEL P CLIN CHIM ACTA 36 315 972 013 REID L MOD PROBL PEDIATR 10 195 967 014 LEV R AM J PATHOL 46 23 965 015 LAMB D COLLOQ INT PATHOL THORAC LILL 143 968 016$ CRIMMIN WRC J CHEM SOC 2838 957 017 LLOYD AG BIOCHEM J 83 455 962 018 WALKER E CARBOHYD RES 35 270 974 019 HAVEZ R BULL SOC CHIM BIOL 51 245 969 020 DEMAILLE J BULL SOC CHIM FRANCE 1965 3506 965 021 DUBOIS M ANAL CHEM 28 350 956 022 AMINOFF D BIOCHEM J 81 384 961 023 LAGUNOFF D ARCH BIOCHEM BIOPHYS 99 396 962 024 YAPHE W ANAL CHEM 32 1327 960 025 SPENCER B BIOCHEM J 75 435 960 026 SAMPSON P PROC NAT ACAD SCI USA 68 2329 971 027 HORTON D IN: JEANLOZ RW 1A 92 969 028 REINHOLD VN METHODS ENZYMOL 25 244 972 029 WOODS KR BIOCHIM BIOPHYS ACTA 133 369 967 030 CROS C EUR J BIOCHEM 7 463 969 031 BOURRILLON R BULL SOC CHIM BIOL 41 267 959 032 MENZIES IS IN: SMITH I 1 310 969 033 PARTRIDGE SM BIOCHEM SOC SYMP 3 52 949 034 TREVELYAN WE NATURE 166 444 950 035 PARTRIDGE SM BIOCHEM J 42 238 948 036 POTTER JL ANN NY ACAD SCI 106 692 963 037 WATKINS WM IN: GOTTSCHALK A 830 972 038 CARLSON DM J BIOL CHEM 241 2984 966 039 BRAY BA J BIOL CHEM 242 3373 967 040 KABAT EA BIOCHEMISTRY 4 1632 965 041 ROVIS L BIOCHEMISTRY 12 1955 973 042 CARLSON DM J BIOL CHEM 243 616 968 043 BAIG MM J BIOL CHEM 247 6111 972 044 ROVIS L BIOCHEMISTRY 12 5340 973 CT 1 LAFITTE JJ LILLE MED 20 798 975 2 HOUDRET N BIOCHIMIE 57 603 975 3 BOERSMA A C R ACAD SCI (D)(PARIS) 281 1269 975 4 LAFITTE JJ C R ACAD SCI (D)(PARIS) 281 1901 975 5 WILSON GB SCAND J IMMUNOL 5 828 976 6 FORSTNER JF PEDIATR RES 10 609 976 7 WU JT PEDIATR RES 10 235 976 8 SIMPSON DL BIOCHEMISTRY 15 5449 976 9 LHERMITTE M BIOCHIMIE 58 367 976 10 WOOD RE AM REV RESPIR DIS 113 833 976 11 NEWMAN W ARCH BIOCHEM BIOPHYS 172 524 976 12 MAISONROUGEMCAULIFFE ARCH BIOCHEM BIOPHYS 175 81 976 13 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 14 SANTAGNESE PAD N ENGL J MED 295 481 976 15 LAMBLIN G BULL EUR PHYSIOPATH RESP 13 175 977 16 LAFITTE JJ CARBOHYD RES 56 383 977 17 LHERMITTE M BIOCHIMIE 59 611 977 18 LAMBLIN G CLIN CHIM ACTA 79 425 977 19 BARTON AD PROC SOC EXP BIOL MED 156 8 977 20 GALLAGHER JT TRENDS BIOCHEM SCI 3 38 978 21 FORSTNER JF DIGESTION 17 234 978 22 CLAMP JR BR MED BULL 34 25 978 23 PARTHASARATHY N J SCI INDUSTR RES 37 305 978 24 SUZUKI M CLIN CHIM ACTA 87 229 978 25 ROUSSEL P LUNG 154 241 978 26 SACHDEV GP BIOCHIM BIOPHYS ACTA 536 184 978 27 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 28 LAFITTE JJ BULL EUR PHYSIOPATH RESP 15 667 979 29 FELDHOFF PA BIOCHEMISTRY 18 2430 979 30 ROSE MC BIOCHEMISTRY 18 4030 979 31 LAMBLIN G BIOCHIMIE 61 23 979 32 KAIZU T COMP BIOCHEM PHYSIOL (B) 62 195 979 33 LIAO TH BIOCHIM BIOPHYS ACTA 577 442 979 34 SACHDEV GP BIOCHEM MED 24 82 980 35 ROUSSEL P LILLE MED 25 594 980 36 BOAT TF FED PROC 39 3067 980 37 LOPEZVIDRIERO MT J COMP PATHOL 90 415 980 38 LAMBLIN G J BIOL CHEM 255 4595 980 39 GALABERT C CLIN RESP PHYSIOL 17 197 981 40 LETREUT A BIOCHIMIE 63 425 981 41 LHERMITTE M CARBOHYD RES 92 333 981 42 BOERSMA A CARBOHYD RES 95 227 981 43 SHERMAN JM AM REV RESPIR DIS 124 476 981 44 BHASKAR KR J BIOL CHEM 256 7583 981 45 TABACHNIK NF J BIOL CHEM 256 7161 981 46 HOUDRET N BIOCHIM BIOPHYS ACTA 668 413 981 47 LAINE A BIOCHIM BIOPHYS ACTA 668 429 981 48 HEIFETZ A BIOCHEMISTRY 21 171 982 49 WOODWARD H BIOCHEMISTRY 21 694 982 50 MAZZUCA M J HISTOCHEM CYTOCHEM 30 956 982 51 TURCK D ARCH FR PEDIATR 39 405 982 52 BALDUYCK M BIOCHEM BIOPHYS RES COMMUN 109 1247 982 53 VANHALBEEK H EUR J BIOCHEM 127 7 982 54 GALLAGHER JT ADV EXP MED BIOL 144 335 982 55 REID L ADV EXP MED BIOL 144 369 982 56 SLOMIANY A BIOCHIM BIOPHYS ACTA 710 106 982 57 REID L EXP LUNG RES 4 157 983 58 SPICER SS EXP LUNG RES 4 137 983 59 WARREN L BIOMEMBRANES 11 53 983 60 FRATES RC PEDIATR RES 17 30 983 61 BLOOMFIELD VA BIOPOLYMERS 22 2141 983 62 BOERSMA A CARBOHYD RES 115 175 983 63 CHACE KV CLIN CHIM ACTA 132 143 983 64 LAMBLIN G BIOCHEM SOC TRANS 12 599 984 65 LLOYD C IN VITRO 20 416 984 66 STELANDER M EUR J RESPIR DIS 65 5 984 67 SLAYTER HS EUR J BIOCHEM 142 209 984 68 GUEANT JL CLIN CHIM ACTA 143 217 984 69 LAMBLIN G EUR J BIOCHEM 143 227 984 70 HARDING SE BIOCHEM J 219 1061 984 71 LAMBLIN G J BIOL CHEM 259 9051 984 72 BHASKAR KR EXP LUNG RES 9 289 985 73 CHACE KV BIOCHEMISTRY 24 7334 985 74 STOWELL CP CARBOHYD RES 151 279 986 75 HOUDRET N BIOCHIM BIOPHYS ACTA 880 54 986 76 MAWHINNEY TP J BIOL CHEM 262 2994 987 PN 75091 RN 00257 AN 75170286 AU Lazarides-E. TI Tropomyosin antibody: the specific localization of tropomyosin in nonmuscle cells. SO J-Cell-Biol. 1975 Jun. 65(3). P 549-61. MJ FIBROBLASTS: ul. TROPOMYOSIN: ip. MN ACTINS: im, ip. ANTIBODY-FORMATION. ANTIBODY-SPECIFICITY. CELL-LINE. CHICKENS: im. CHROMATOGRAPHY-DEAE-CELLULOSE. CYSTIC-FIBROSIS. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FLUORESCENT-ANTIBODY-TECHNIC. HUMAN. MICE-INBRED-STRAINS: em. MICROSCOPY-FLUORESCENCE. PERIODICITY. RABBITS: im. SKIN. TROPOMYOSIN: im. SUPPORT-U-S-GOVT-P-H-S. MICE. AB An antibody against purified chicken skeletal muscle tropomyosin is used in indirect immunofluorescence to visualize the localization of tropomyosin in a variety of nonmuscle cells. The antibody produces a fluorescent pattern which is very similar to that obtained with an actin-specific antibody. This pattern is composed of fluorescent fibers which are shown to be coincident with the fibers seen with phase-contrast optics. High resolution epifluorescent microscopy reveals that fibers stained with the actin antibody show a continuous fluorescence, while fibers reacted with the tropomyosin antibody show a periodic fluorescence. Measurements indicate that the lengths of the fluorescent segments are variable with an average of 1.2 mum while the spacing between segments is approximately 0.4 mum. RF 001 ALLERA A CYTOBIOLOGIE 6 261 972 002 ANDERSEN CW J VIROL 12 241 973 003 BEHNKE O J ULTRASTRUCT RES 37 351 971 004 BUCKLEY IK PROTOPLASMA 64 349 967 005 COHEN I J MOL BIOL 68 383 972 006 CUMMINS P BIOCHEM J 133 765 973 007 CUMMINS P BIOCHEM J 141 43 974 008 EBASHI S PROG BIOPHYS MOL BIOL 18 123 968 009 ENDO M J BIOCHEM (TOKYO) 60 605 966 010 FINE RE NATURE NEW BIOL 245 182 973 011 GOLDMAN RD J CELL BIOL 52 246 972 012 GOLDMAN RD COLD SPRING HARBOR SYMP QUANT 37 523 972 013 GOLDMAN RD EXP CELL RES 90 333 975 014 HUXLEY HE J MOL BIOL 7 281 963 015 HUXLEY HE HARVEY LECTURES 60 85 966 016 ISHIKAWA H J CELL BIOL 43 312 969 017 LAEMMLI UK NATURE 227 680 970 018 LAZARIDES E PROC NAT ACAD SCI USA 71 4742 974 019 LAZARIDES E PROC NAT ACAD SCI USA 71 2268 974 020 OHTSUKI I J BIOCHEM (TOKYO) 61 817 967 021 ORR TS NATURE 236 350 972 022 PAGE SG J CELL BIOL 19 369 963 023 PEPE FA J CELL BIOL 28 505 966 024 POLLARD TD IN: FASMAN GD 2 1 974 025 RASH JE NATURE NEW BIOL 237 160 972 026 SCHROEDER TE Z ZELLFORSCH MIKROSK ANAT 109 431 970 027 SPOONER BS J CELL BIOL 49 595 971 028 SPUDICH JA J BIOL CHEM 246 4866 971 029 SPUDICH JA COLD SPRING HARBOR SYMP QUANT 37 585 972 030 SPUDICH JA J MOL BIOL 72 619 972 031 WEBER K PROC NAT ACAD SCI USA 71 4561 974 032 WESSELLS NK SCIENCE 171 135 971 033 WOOLEY DE ARCH BIOCHEM BIOPHYS 150 519 972 034 YANG Y J BIOL CHEM 247 4503 972 CT 1 LAZARIDES E CELL 6 289 975 2 HOLTZER H Q REV BIOPHYS 8 523 975 3 AGOSTINI B Z NATURFORSCH (C) 30 793 975 4 WEBER K EXP CELL RES 95 111 975 5 GROSCHELSTEWART U CELL TISSUE RES 165 13 975 6 BRETSCHER MS NATURE 258 43 975 7 KORN ED TRENDS BIOCHEM SCI 1 55 976 8 GOLDMAN RD J SUPRAMOL STRUCT 5 155 976 9 LAZARIDES E J SUPRAMOL STRUCT 5 531 976 10 MILLER F CLIN IMMUNOL IMMUNOPATHOL 5 416 976 11 POLLARD TD J SUPRAMOL STRUCT 5 317 976 12 GASSNER G DIFFERENTIATION 7 39 976 13 BOOTH AG J CELL SCI 21 449 976 14 IZZARD CS J CELL SCI 21 129 976 15 SCHEINMAN JI LAB INVEST 34 150 976 16 GARANT PR J PERIODONTOL 47 380 976 17 LAZARIDES E J CELL BIOL 68 202 976 18 FUJIWARA K J CELL BIOL 71 848 976 19 WEBER K EXP CELL RES 102 285 976 20 BUCKLEY IK J MICROSC 107 129 976 21 LAGENAUR C J BACTERIOL 128 435 976 22 WOHLFARTHBOTTERMANN K CELL TISSUE RES 165 327 976 23 ISENBERG G CELL TISSUE RES 166 427 976 24 EATON BL SCIENCE 192 1337 976 25 NICOLSON GL N ENGL J MED 295 197 976 26 HYNES RO BIOCHIM BIOPHYS ACTA 458 73 976 27 WILLINGHAM MC CELL 10 375 977 28 STRAULI P EUR J CANCER 13 1 977 29 KEENAN TW CYTOBIOLOGIE 14 259 977 30 LOOR F PROG ALLERGY 23 1 977 31 MUSSINI JM PATHOL BIOL (PARIS) 25 477 977 32 RUTTER G J GEN VIROL 37 233 977 33 SILVERSTEIN SC ANNU REV BIOCHEM 46 669 977 34 VASILIEV JM INT REV CYTOL 50 159 977 35 BRAY D BIOCHIMIE 59 1 977 36 RUBENSTEIN PA PROC NAT ACAD SCI USA 74 120 977 37 ISHIURA M J BIOCHEM 82 105 977 38 CHAMLEY JH CELL TISSUE RES 177 445 977 39 CHAMLEYCAMPBELL J CELL TISSUE RES 183 153 977 40 YANG YZ J BIOL CHEM 252 3374 977 41 SANCHEZ G MORFOL NORMAL PATOL (A) 2 119 978 42 VARELA H MORFOL NORMAL PATOL (A) 2 215 978 43 TRIFARO JM NEUROSCIENCE 3 1 978 44 GORDON WE CELL 13 249 978 45 CARMON Y CELL 14 393 978 46 OSBORN M CELL 14 477 978 47 GRANGER BL CELL 15 1253 978 48 WANG E J HISTOCHEM CYTOCHEM 26 745 978 49 HEATH JP J CELL SCI 29 197 978 50 STOSSEL TP ANNU REV MED 29 427 978 51 JOCKUSCH BM HISTOCHEMISTRY 55 177 978 52 BAGBY RM HISTOCHEMISTRY 58 219 978 53 LLEWELLYNSMITH IJ DEVELOP BIOL 67 40 978 54 FUJIWARA K J CELL BIOL 77 182 978 55 KREIBICH G J CELL BIOL 77 464 978 56 BRETSCHER A J CELL BIOL 79 839 978 57 GERACE L J CELL BIOL 79 546 978 58 WANG E J CELL BIOL 79 708 978 59 CORCES VG EUR J BIOCHEM 83 529 978 60 ISHIMODATAKAGI T J BIOCHEM 83 1757 978 61 BRETSCHER A EXP CELL RES 116 397 978 62 GORDON WE EXP CELL RES 117 253 978 63 WEBSTER RE EXP CELL RES 117 47 978 64 WILLIAMSON DL CURRENT MICROBIOL 2 143 979 65 DIAZFLORES L MORFOL NORMAL PATOL (A) 3 63 979 66 AUNIS D NEUROSCI LETT 13 35 979 67 MCMILLAN SA CLIN IMMUNOL IMMUNOPATHOL 14 256 979 68 OTTO JJ CELL 17 285 979 69 DRIESSCHE TV ANNEE BIOL 18 417 979 70 RATHKE PC EUR J CELL BIOL 19 40 979 71 OSBORN M EUR J CELL BIOL 20 28 979 72 ELFVIN MJ J HISTOCHEM CYTOCHEM 27 1478 979 73 CAMPBELL GR J CELL SCI 37 303 979 74 WEHLAND J J CELL SCI 37 257 979 75 YOUNG RB J ANIMAL SCI 48 837 979 76 CHAMLEYCAMPBELL J PHYSIOL REV 59 1 979 77 GARRELS JI DEVELOP BIOL 73 134 979 78 FERAMISCO JR PROC NAT ACAD SCI USA 76 3967 979 79 GOLDMAN RD J CELL BIOL 80 759 979 80 WILLARD M J CELL BIOL 81 581 979 81 YOUNG RB J CELL BIOL 81 115 979 82 LESSARD JL ANAL BIOCHEM 94 140 979 83 HILLER G VIROLOGY 98 142 979 84 ISHIMODATAKAGI T EXP CELL RES 119 423 979 85 ZIGMOND SH EXP CELL RES 119 205 979 86 GORDON WE EXP CELL RES 120 335 979 87 WYLIE CC EXP CELL RES 121 315 979 88 KLEVE MG J EXP ZOOL 209 21 979 89 YAMAUCHI T J BIOL CHEM 254 6408 979 90 FEUCHTER FA UROL RES 8 139 980 91 KELLEY RO MECH AGEING DEV 13 127 980 92 GROSCHELSTEWART U EUR J CANCER 16 2 980 93 UMEDA PK BIOCHEMISTRY 19 1955 980 94 ALLEN RE EUR J CELL BIOL 21 247 980 95 WEHLAND J EUR J CELL BIOL 21 188 980 96 GODMAN G EUR J CELL BIOL 22 745 980 97 GODMAN G EUR J CELL BIOL 22 733 980 98 KREIS TE CELL 22 555 980 99 WYLIE CC BIOSCIENCE 30 27 980 100 LUTZ H CANCER RES 40 3642 980 101 LEMANSKI LF J EMBRYOL EXP MORPHOL 55 1 980 102 SMALL JV INT REV CYTOL 64 241 980 103 BAGBY RM HISTOCHEMISTRY 69 113 980 104 HODGE AJ J ULTRASTRUCT RES 70 220 980 105 BYERS HR PROC NAT ACAD SCI USA 77 6657 980 106 RACHUBINSKI RA J CELL BIOL 84 705 980 107 BRETSCHER A J CELL BIOL 86 335 980 108 SANGER JM J CELL BIOL 86 568 980 109 SCHLOSS JA J CELL BIOL 87 633 980 110 BRITCH M EXP CELL RES 125 221 980 111 RAMAEKERS FCS EXP CELL RES 127 309 980 112 WEHLAND J EXP CELL RES 127 397 980 113 OSBORN M EXP CELL RES 129 103 980 114 PAULIN D J MOL BIOL 144 95 980 115 MANJULA BN J EXP MED 151 695 980 116 ABERCROMBIE M PROC R SOC LOND (B) 207 129 980 117 FRANKO MC J NEUROIMMUNOL 1 391 981 118 MOSS M MOL CELL BIOL 1 289 981 119 GOMER RH CELL 23 524 981 120 DEBUS E EUR J CELL BIOL 24 45 981 121 KALNINS VI EUR J CELL BIOL 24 36 981 122 WEHLAND J EUR J CELL BIOL 24 176 981 123 BIRCHMEIER W COLD SPRING HARBOR SYMP Q BIO 46 755 981 124 BLOSE SH COLD SPRING HARBOR SYMP Q BIO 46 455 981 125 FRANK ED PROC NAT ACAD SCI USA 78 3020 981 126 HENDRICKS M PROC NAT ACAD SCI USA 78 5633 981 127 DHANARAJAN ZC DEVELOP BIOL 82 317 981 128 PENG HB DEVELOP BIOL 88 121 981 129 ALLEN RD J CELL BIOL 91 S148 981 130 MELYGOUBERT B J IMMUNOL 127 399 981 131 BRITCH M EXP CELL RES 131 161 981 132 KOENIG CS EXP CELL RES 131 319 981 133 NELSON GA EXP CELL RES 131 149 981 134 HILLER G EXP CELL RES 132 81 981 135 BRETSCHER A EXP CELL RES 135 213 981 136 COTE GP J BIOL CHEM 256 7257 981 137 GIOMETTI CS J BIOL CHEM 256 1840 981 138 KEEN JH J BIOL CHEM 256 2538 981 139 LETOURNEAU PC J NEUROSCI 2 806 982 140 DOMINGUEZ C MORFOL NORMAL PATOL (B) 6 157 982 141 LAZARIDES E METH CELL BIOL 24 313 982 142 OSBORN M METH CELL BIOL 24 97 982 143 HILLER G J VIROL 44 647 982 144 ALLORE RJ CAN J BIOCHEM 60 57 982 145 YAHARA I J CELL BIOL 92 69 982 146 BYERS HR J CELL BIOL 93 804 982 147 OHASHI K J CELL BIOL 95 85 982 148 WEBER K PATHOL RES PRACT 175 128 982 149 RABINOWITZ SS J BIOL CHEM 257 3291 982 150 GENY B BIOCHIM BIOPHYS ACTA 692 345 982 151 LEVINE AM CLIN ORTHOP 1982 240 982 152 DABROWSKA R J MUSCLE RES CELL MOTIL 4 83 983 153 FATTOUM A BIOCHEMISTRY 22 1187 983 154 OSBORN M EUR J CELL BIOL 29 179 983 155 SANGER JW EUR J CELL BIOL 31 197 983 156 ITO Y J VIROL 48 709 983 157 COTE GP MOL CELL BIOCHEM 57 127 983 158 GABBIANI G PROC NAT ACAD SCI USA 80 2361 983 159 KOBAYASHI R J BIOCHEM 94 171 983 160 SANGER JW J CELL BIOL 96 961 983 161 LEMANSKI LF DEVELOP BIOL 97 338 983 162 NAGELE RG EXP CELL RES 143 153 983 163 TALBOT K J MOL BIOL 164 159 983 164 MACLEOD AR J MOL BIOL 167 523 983 165 MATSUMURA F J BIOL CHEM 258 6636 983 166 LAZARIDES E CURR CONTENTS LIFE SCIENCES 1983 19 983 167 HENDRICKS M MOL CELL BIOL 4 1823 984 168 MASUDA H CELL MOTIL 4 315 984 169 KNOX P MOL ASP MED 7 177 984 170 PAYNE MR TRENDS BIOCHEM SCI 9 361 984 171 LANGANGER G J SUBMICROSC CYTOL 16 43 984 172 SHARPE AH COMPR VIROL 19 431 984 173 SCEBAT L ATHEROSCLEROSIS 51 269 984 174 OWADA MK PROC NAT ACAD SCI USA 81 3133 984 175 LIN JJC J CELL BIOL 98 116 984 176 LANGANGER G J CELL BIOL 99 1324 984 177 SANGER JW J CELL BIOL 99 918 984 178 GOLDMAN JE BRAIN RES 106 85 984 179 FOWLER VM J BIOL CHEM 259 5978 984 180 BENZEEV A BIOCHIM BIOPHYS ACTA 780 197 984 181 WAHRMAN MZ TUMOUR BIOL 6 41 985 182 WARREN RH EUR J CELL BIOL 38 245 985 183 LIM SS CAN J BIOCHEM CELL BIOL 63 470 985 184 BRETSCHER A J CELL BIOL 100 1656 985 185 MURAMATSU H DEVELOP BIOL 110 284 985 186 BURGOYNE RD FEBS LETTERS 179 25 985 187 KREISBERG JI AM J PHYSIOL 249 F227 985 188 YAMASHIROMATSUMURA S J BIOL CHEM 260 5087 985 189 BURGOYNE RD BRAIN RES 361 178 985 190 CANDE WZ CELL MOTIL CYTOSKELETON 6 640 986 191 KARLIK CC MOL CELL BIOL 6 1965 986 192 GUEST JF ELECTROPHORESIS 7 512 986 193 BRETT JG TISSUE CELL 18 175 986 194 DOBROWOLSKI Z EUR J CELL BIOL 41 65 986 195 JOST E BIOL CELL 57 111 986 196 SHIRAKATA M COMP BIOCHEM PHYSIOL (B) 85 71 986 197 AMATO PA J CELL BIOL 102 1074 986 198 ANTIN PB J CELL BIOL 102 1464 986 199 LANGANGER G J CELL BIOL 102 200 986 200 GRAIN J INT REV CYTOL 104 153 986 201 IIDA K EXP CELL RES 164 492 986 202 BROSCHAT KO J BIOL CHEM 261 3350 986 203 TAMM SL CELL MOTIL CYTOSKELETON 7 116 987 204 MIZUSHIMA Y BIOMED RES 8 73 987 205 GALLOWAY PG J NEUROPATHOL EXP NEUROL 46 185 987 206 MACLEOD AR J MOL BIOL 194 1 987 207 HALLAUER PL J BIOL CHEM 262 3590 987 PN 75092 RN 00258 AN 76095429 AU Warwick-W-J. Pogue-R-E. Gerber-H-U. Nesbitt-C-J. TI Survival patterns in cyctic fibrosis. SO J-Chronic-Dis. 1975 Dec. 28(11-12). P 609-22. MJ CYSTIC-FIBROSIS: mo. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, oc. DISEASES-IN-TWINS. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co. LIFE-EXPECTANCY. LONGITUDINAL-STUDIES. MALE. MECONIUM: me. RACIAL-STOCKS. REGISTRIES. SEX-FACTORS. TWINS-DIZYGOTIC. TWINS-MONOZYGOTIC. UNITED-STATES. AB This paper describes the Data Registry System of the Cystic Fibrosis Foundation. Through this system an annual up-dated and corrected data base has been prepared for cystic fibrosis patients seen at U.S.A. Centers from 1966 through 1972. From the data base, a large number of life table studies are calculated. A study is specified by its data base, the observation period, analysis age (age year or year since diagnosis), the Centers included, and the condition of patients at birth, and possible additional factors such as sex, age at diagnosis, or race. The annual rates of mortality are measured by a force of mortality technique. Two techniques have been used to appraise the mortality of particular groups, graphing the cumulative survival function for the group together with the cumulative survival function for a comparative group, and calculation of mortality ratios of actual to expected deaths (by an applicable standard mortality table). This latter approach lends itself readily to tests of significance. For the purpose of calculating mortality ratios, two standard tables have been prepared on the basis of the 1972 Data Base and the 1966-1972 observations. The first of these standard tables is for use with age year studies, and the second is for years since diagnosis studies. The paper comments on the number of mortality studies by calendar year, by sex, by condition at birth, by age at diagnosis, and of survival from diagnosis of patients diagnosed in 1966-1972. The calendar year studies indicate an improvement trend in the survival experience from 1966 to 1972 but a change may be occurring. The study by sex exhibits a higher female mortality. The 7% of patients who have meconium ileus at birth show a significantly higher first-year mortality, and a somewhat lower survival rate thereafter even if the first year of life experience is excluded. Mortality data for groups specified by age at diagnosis are given. For patients diagnosed during 1966-1972, there is some indication of better survival than for patients diagnosed prior to 1966. The continuation of these mortality studies on an annual basis should further our understanding of survival patterns for cystic fibrosis patients. RF 001 WARWICK WJ J ASTHMA RES 5 277 968 002 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 003 SHWACHMAN H PEDIATRICS 46 335 970 004 WECK FA REC AM INST ACTUARIES 36 23 947 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 BURDICK AB HUM HERED 27 366 977 3 KRAEMER R HELV PAEDIATR ACTA 32 107 977 4 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 271 977 5 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 6 LHEUREUX PR AM J ROENTGENOL 128 953 977 7 WARWICK WJ JAMA 238 2159 977 8 KRAEMER R ACTA PAEDIATR SCAND 67 33 978 9 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 10 SANTAGNESE PAD AM J MED 66 121 979 11 ANON CAN MED ASSOC J 121 1193 979 12 COHEN LF LANCET 2 842 980 13 JONES MB J CHRON DIS 33 697 980 14 HUBBARD VS AM J DIS CHILD 134 317 980 15 STERN RC AM J DIS CHILD 134 267 980 16 ELEJALDE BR REV BRASIL GENET 5 409 982 17 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 18 KANG SB ANESTH ANALG CLEVE 61 793 982 19 LIMACHER F SCHWEIZ MED WOCHENSCHR 112 264 982 20 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 21 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 PN 75093 RN 00259 AN 75151860 AU Murad-F. Moss-W-W. Johanson-J. Selden-R-F. TI Urinary excretion of adenosine 3',5'-monophosphate and guanosine 3',5'-monophosphate in normal children and those with cystic fibrosis. SO J-Clin-Endocrinol-Metab. 1975 Apr. 40(4). P 552-9. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: ur. CYSTIC-FIBROSIS: ur. GUANOSINE-CYCLIC-MONOPHOSPHATE: ur. MN ADOLESCENCE. AGE-FACTORS. BODY-SURFACE-AREA. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CREATININE: ur. DIURNAL-RHYTHM. FEMALE. HUMAN. MALE. SUPPORT-U-S-GOVT-P-H-S. AB The urinary excretion of adenosine 3',5'-monophosphate (cyclic AMP) and guanosine 3',5'-monophosphate (cyclic GMP) was examined in 98 normal children and 46 children with cystic fibrosis between the ages of 9 months and 18 yr. Diurnal variations in cyclic AMP and cyclic GMP excretion were observed in subjects from either group, and peak levels of cyclic nucleotide excretion were generally observed during the period of 0700 to 2100 h. Excretion rates (mumol/day) of cyclic AMP and cyclic GMP increased significantly with age. When cyclic AMP and cyclic GMP excretion rates were normalized for urinary creatine, or body weight, the values declined significantly with age in both groups of patients. Cyclic GMP excretion normalized for body surface area also decreased with age, while the value for cyclic AMP (2.86 plus or minus 0.08 mumol/day/m2, mean plus or minus SE) was constant with age in both normals and cystic fibrosis children. With some comparisons of age groups there were significant differences in cyclic nucleotide excretion between normal subjects and children with cystic fibrosis. The differences noted were dependent upon the methods used to normalize excretion rates (urinary creatine, body weight, surface area, and the ratio of cyclic AMP to cyclic GMP excreted). In general patients with cystic fibrosis excreted greater amounts of cyclic GMP than did normals. The most striking comparison was the ratio of cyclic AMP to cyclic GMP excreted which was 9.09 plus or minus 0.50 in all normal children and 4.41 plus or minus 0.32 in children with cystic fibrosis (P smaller than 0.001). RF 001 MURAD F ADV CYCLIC NUCLEO RES 3 355 973 002 CHASE LR J CLIN INVEST 48 1832 969 003 KAMINSKY NI J CLIN INVEST 49 2387 970 004 MURAD F N ENGL J MED 286 1382 972 005 AUGUST GP J CLIN ENDOCRINOL METAB 37 476 973 006 BERNSTEIN RA J LAB CLIN MED 80 772 972 007 LINARELLI LG PEDIATRICS 50 14 972 008 TSANG CP J CLIN ENDOCRINOL METAB 35 809 972 009 SIMOPOULOS AP PROC AM PEDIATR SOC ANNU MTG 201 971 010 GOLDBERG ND ADV CYCLIC NUCLEO RES 3 155 973 011 MOSS WW CLIN RES 45A 973 012 GILMAN AG PROC NAT ACAD SCI USA 67 305 970 013 MURAD F PROC NAT ACAD SCI USA 68 736 971 014 STEINER AL J BIOL CHEM 247 1114 972 015 BONSNES RW J BIOL CHEM 158 581 945 016 SAGEL J J CLIN ENDOCRINOL METAB 37 570 973 017 BROADUS AE J CLIN INVEST 49 2222 970 018 BALL JH J CLIN INVEST 51 2124 972 019 HARDMAN JG J BIOL CHEM 244 6354 969 CT 1 ROOF BS MECH AGEING DEV 5 289 976 2 CRISS WE CANCER RES 36 1714 976 3 VITEK V J CLIN ENDOCRINOL METAB 42 781 976 4 VITEK V CLIN CHIM ACTA 70 227 976 5 DISANTAGNESE PA N ENGL J MED 295 597 976 6 BROADUS AE ADV CYCLIC NUCLEO RES 8 509 977 7 GOLDBERG ND ANNU REV BIOCHEM 46 823 977 8 PERLOW MJ BRAIN RES 126 391 977 9 DAVIS PB CLIN CHIM ACTA 87 285 978 10 WAALER PE HORM RES 11 318 979 11 SACK J ISR J MED SCI 15 937 979 12 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 13 REINHARDT D INT J CLIN PHARMACOL THER TOX 18 399 980 14 MARKOVAC J BIOCHEM MED 26 299 981 15 KRUSE K J CLIN ENDOCRINOL METAB 53 1251 981 16 SATO T CLIN CHIM ACTA 110 215 981 17 MOUGIN C C R SOC BIOL (PARIS) 176 319 982 18 THODE J SCAND J CLIN LAB INVEST 45 185 985 PN 75094 RN 00260 AN 76025431 AU Zierdt-C-H. Williams-R-L. TI Serotyping of Pseudomonas aeruginosa isolates from patients with cystic fibrosis of the pancreas. SO J-Clin-Microbiol. 1975 Jun. 1(6). P 521-6. MJ CROSS-INFECTION: mi. CYSTIC-FIBROSIS: mi. CROSS-INFECTION: mi. PSEUDOMONAS-AERUGINOSA: cl. SEROTYPING: mt. MN AGGLUTINATION-TESTS. BIOLOGICAL-PRODUCTS: bi. PSEUDOMONAS-AERUGINOSA: me. SPECIES-SPECIFICITY. AB Pseudomonas aeruginosa isolates (173) from 144 patients with cystic fibrosis (CF) of the pancreas in seven hospitals were serotyped with the agglutination systems of Homma (1974) and Fisher et al. (1969). The two systems were complementary. Strains from CF patients were much less likely to furnish a stable type on repetitive typing tests than strains from other patients. This was related to the frequent occurrence of mucoid P. aeruginosa strains. The 173 strains were divided among 11 Homma serotypes. A single Homma type (type 8) capable of mucoid growth comprised 104 (60%) CF strains. Eight serotypes were detected in 77 strains from 48 CF patients in one hospital; three strains were detected in one hospital CF unit; and two strains were detected in each of five hospital CF units. The CF serotype comprised from 50 to 93% of CF strains in the seven hospitals. These P. aeruginosa strains dissociated in vivo as judged by mucoid and nonmucoid colonies on primary culture plates and continued to dissociate during subcultures. Both colony type were the same serotype. The tendency to regard colonial phenotypes (mucoid, nonmucoid, rough) as separate strains was erroneous. Repetitive typing with the two systems gave better results than a single system. The mucoid P. aeruginosa strain is probably spread from patient to patient, rather than acquiring its mucoid characteristic de novo in the CF patient. It is not known why the mucoid CF strain has a peculiar predilection for CF patients, nor why it generally loses the quality in culture but retains it indefinitely in the patient. RF 001 CETIN ET J BACTERIOL 89 1432 965 002 CHADWICK P CAN J MICROBIOL 18 1153 972 003 DOGGETT RG APPL MICROBIOL 18 936 969 004 ELSTON HR AM J CLIN PATHOL 48 519 967 005 FISHER MW J BACTERIOL 98 835 969 006 GOTO S JAPAN J MICROBIOL 15 317 971 007 HOMMA JY JAPAN J EXP MED 44 1 974 008 HOMMA JY JAPAN J EXP MED 42 171 972 009 HOMMA JY JAPAN J EXP MED 41 89 971 010 JONES LF APPL MICROBIOL 27 400 974 011 KAWAHARAJO K JAPAN J EXP MED 43 225 973 012 SHIONOYA H JAPAN J EXP MED 38 81 968 013 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 WALLACE R REV CAN BIOL 35 87 976 2 VERON M BULL INSTITUT PASTEUR 74 295 976 3 REYNOLDS HY JAMA 236 2190 976 4 DISANTAGNESE PA N ENGL J MED 295 597 976 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 6 HIRAO Y JAP J EXP MED 47 249 977 7 HOIBY N SCAND J RESPIR DIS 58 65 977 8 MARKOWITZ SM INFECT IMMUN 22 530 978 9 KULCZYCKI LL JAMA 240 30 978 10 SEALE TW J CLIN MICROBIOL 9 72 979 11 THOMASSEN MJ J INFECT DIS 140 873 979 12 WOODS DE INFECT IMMUN 30 694 980 13 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 14 BALTIMORE RS J INFECT DIS 141 238 980 15 REYNOLDS HY CLIN CHEST MED 2 103 981 16 FICK RB J CLIN INVEST 68 899 981 17 PENNINGTON JE J CLIN INVEST 68 1140 981 18 MACONE AB N ENGL J MED 304 1445 981 19 KELLY NM LANCET 2 688 982 20 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 21 SPEERT DP J PEDIATR 101 227 982 22 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 23 ANON LANCET 2 257 983 24 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 25 BOYD RL INFECT IMMUN 39 1403 983 26 HANCOCK REW INFECT IMMUN 42 170 983 27 JOHNSON SR OBSTET GYNECOL 61 S 2 983 28 BUCKMIRE FLA MICROBIOS 41 49 984 29 FICK RB J CLIN INVEST 74 236 984 30 FICK RB AM REV RESPIR DIS 129 76 984 31 HANCOCK REW J INFECT DIS 149 220 984 32 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 33 SPEERT DP J HOSP INFECT 9 11 987 34 ANASTASSIOU ED J CLIN MICROBIOL 25 656 987 35 KOMIYAMA K CAN J MICROBIOL 33 221 987 36 OGLE JW J INFECT DIS 155 119 987 PN 75095 RN 00261 AN 75212529 AU Green-M. Mascia-A-V. Behrendt-H. TI The source of Na+ and Cl- activities on the skin surface. Experiences with electrode measurements in children. SO J-Lab-Clin-Med. 1975 Sep. 86(3). P 494-8. MJ CHLORIDES: an. SKIN: an. SODIUM: an. SWEAT: an. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS. ELECTRODES. HUMAN. INFANT. INFANT-NEWBORN. MALE. AB We have used ion-specific electrodes in an attempt to map the sodium and chloride activities on the skin under differing surface conditions. The range of values and the standard deviations are so wide that a baseline for the untreated dry skin is precluded. In patients with cystic fibrosis, a wide range of values is also found. For this group, measurements on dry skin taken immediately after washing were in the same low range as for healthy subjects. The range of measurements on the dry skin of newborn infants was similar for full-term infants with reactive sweat glands and for premature infants who failed to show sweating responsiveness to natural reflex stimulation as well as local glandular stimulation with sudorific drugs. For only two conditions have measurements of sodium and chloride activities on the surface of the skin been of value: (1) on the sweat-covered skin for the detection of the high values encountered in cystic fibrosis, and (2) on the dry skin surface, deprived of autonomic innervation (spinal paraplegia), extremely low sodium and chloride activities reflect absent gland activity. RF 001 GOLDBLOOM RB N ENGL J MED 269 1349 963 002 SEKELJ P IN: EINSENMAN G 540 967 003 WARWICK WJ PEDIATRICS 36 261 965 004 HANSEN L AM J CLIN PATHOL 49 834 968 005 GREEN M CLIN CHEM 18 427 972 006 DAHMS H CLIN CHEM 14 859 968 007 SULZBERGER MB ARCH DERMATOL 81 235 960 008 KOPITO L PEDIATRICS 43 794 969 009 JONES JD MAYO CLIN PROC 45 768 970 010 KENNEY JF MATH OF STAT PART ONE 256 954 011 SECKENDORF R J APPL PHYSIOL 16 796 961 012 BEHRENDT H AM J DIS CHILD 117 299 969 013 BEHRENDT H N ENGL J MED 286 1376 972 CT 1 ANJO DM BR J DERMATOL 105 39 981 PN 75096 RN 00262 AN 75152611 AU Pacharee-P. TI Fibrocystic disease of the pancreas: a case report. SO J-Med-Assoc-Thai. 1975 Feb. 58(2). P 110-2. MJ CYSTIC-FIBROSIS: pa. MN HUMAN. INFANT. MALE. EX An autopsy case of cystic fibrosis of the pancreas in a 2-month-old Thai male infant is reported. The patient suffered from chronic repeated lung infection and died of lung disease. This case seems to be the first reported case in Thailand. RF 001 DI SANTAGNESE PA IN: NELSON WE 856 969 002 ANDERSEN DH AM J DIS CHILD 56 344 938 PN 75097 RN 00263 AN 75191915 AU Adinolfi-A. Adinolfi-M. Lessof. TI Alpha-feto-protein during development and in disease. SO J-Med-Genet. 1975 Jun. 12(2). P 138-51. MJ ALPHA-FETOPROTEINS. FETAL-PROTEINS. MN ALPHA-FETOPROTEINS: an, me, ph. AMNIOTIC-FLUID: an. ANENCEPHALY: me. ANIMAL. ANTIGEN-ANTIBODY-REACTIONS. CONCANAVALIN-A: pd. CYSTIC-FIBROSIS: me. DOWNS-SYNDROME: me. FEMALE. GASTROINTESTINAL-NEOPLASMS: me. GESTATIONAL-AGE. HEPATITIS: me. HEPATOMA: me. HUMAN. IMMUNOLOGIC-TECHNICS. INFANT-NEWBORN. LIVER: cy. METABOLISM-INBORN-ERRORS: me. NEOPLASM-METASTASIS. NEOPLASMS-EXPERIMENTAL: me. PREGNANCY. SPINA-BIFIDA: me. TERATOMA: me. AB An alpha-feto-protein (AFP) is present in many mammals, in birds, and in sharks during development. The AFP present in different species have similar physicochemical properties and often have common antigenic determinants. Their study, both in health and disease, has provided a useful model for the understanding of other phase-specific antigens and the activation of the genes which control their synthesis. In the human fetus, the level of AFP falls with increasing maturity. The more sensitive methods of detection have disclosed that this fetal protein persists in trace amounts throughout life and its level increases in maternal blood during pregnancy. The principal sites of synthesis are the fetal liver and in some mammals, the yolk sac splanchnopleur. In humans as well as in mice and cows, it is notable that the synthesis of AFP is increased in liver cancer cells and that high levels of this protein are present in serum. Elevated values of AFP have also been detected in human subjects with undifferentiated tumours of the testis and ovary. A fall to normal levels has been noted in cases of complete remission after surgery and a return to high levels in patients who develop metastases. In some patients with hepatitis a temporary rise in the level of AFP has also been observed. In recent years, the detection of high levels of AFP in amniotic fluid has proved to be of great value for the prenatal diagnosis of neural-tube defects. Abnormal levels have also been found in the amniotic fluid or in maternal serum in cases of spontaneous abortion. Such measurements are now being assessed as a method of monitoring abnormal pregnancy. RF 001 ABELEV GI PROG EXP TUMOR RES 7 104 965 002 ABELEV GI CANCER RES 28 1344 968 003 ABELEV GI ADV CANCER RES 14 295 971 004 ABELEV GI NEOPLASMA 20 563 973 005 ABELEV GI INT J CANCER 2 551 967 006 ABELEV GI IN: MASSEYEFF R 141 974 007 ABELEV GI TRANSPLANTATION 1 174 963 008 ADAMSON RH IN: ANDERSON NG 2 331 972 009 ADINOLFI A RICERCA 1 316 971 010 ADINOLFI A BIOCHIM BIOPHYS ACTA 251 197 971 011 ADINOLFI M IN: BENSON PF 224 971 013 ADINOLFI M DEVELOP MED CHILD NEUROL 9 609 967 014 ADINOLFI M ATTI ASSOC GENETICA ITALIANA 12 133 967 015 ADINOLFI M PROC INT SYMP IMMUN REPRO 2ND 558 973 016 AKEYAMA T N ENGL J MED 287 989 972 017 ALEXANDER P NATURE 235 137 972 018 ALLAN LD LANCET 2 522 973 019 ALPERT E LANCET 1 626 974 020 ALPERT E ANN NY ACAD SCI 209 387 973 021 ALPERT E GASTROENTEROLOGY 61 137 971 022 ALPERT E N ENGL J MED 278 984 968 023 ALPERT E LANCET 2 465 970 024 ALPERT ME CLIN RES 17 461 969 025 ALPERT ME AM J MED 46 794 969 026 ANDERSON NG IN: ANDERSON NG 1 7 971 027 ANDERSON NG IN: ANDERSON NG 2 361 972 028 ANDREOLI M J CLIN INVEST 41 1070 962 029 ANDRIEU J PRESSE MED 79 1595 971 030 ANSSEL C IN: MASSEYEFF R 17 974 031 BAGSHAWE A LANCET 2 268 970 032 BAKIROV RD BULL EXP BIOL MED 65 45 968 033 BALLAS M AM J CLIN PATHOL 57 511 972 034 BELANGER L PATHOL BIOL (PARIS) 21 457 973 035 BELANGER L PATHOL BIOL (PARIS) 21 449 973 036 BELANGER L IN: MASSEYEFF R 974 037 BELANGER L CLIN CHIM ACTA 48 15 973 038 BERGSTRAND CG SCAND J CLIN LAB INVEST 8 174 956 039 BERGSTRAND CG SCAND J CLIN LAB INVEST 9 277 957 040 BERGSTRAND CG ACTA PAEDIATR SCAND 61 128 972 041 BERNADES P PRESSE MED 79 1585 971 042 BODMAN J CLIN CHIM ACTA 4 103 959 043 BOURREILLE J PRESSE MED 78 1277 970 044 BRANCH WR INT J CANCER 10 451 972 045 BRANCH WR Z ZELLFORSCH 135 501 972 046 BROCK DJH LANCET 2 923 973 047 BROCK DJH LANCET 2 1252 972 048 BROCK DJH LANCET 1 569 974 049 BROCK DJH LANCET 2 197 972 050 BUFFE D GANN MOGR CANCER RES 14 117 973 051 BUFFE D BIOMEDICINE 19 172 973 052 BURTIN P BULL SOC CHIM BIOL 49 1389 967 053 CHANDRA RK ARCH DIS CHILD 48 157 973 054 CHANDRA RK BR MED J 1 714 975 055 ECONOMOPOULOS P LANCET 1 1337 970 056 ENDO Y KANZO 10 143 969 057 ENGELHARDT NV INT J CANCER 7 198 971 058 ENGELHARDT NV BULL EXP BIOL MED 65 62 969 059 FLORIN-CHRISTENSEN A CLIN EXP IMMUNOL 16 599 974 060 FOLI AK LANCET 2 1267 969 061 FOY H LANCET 2 663 970 062 FOY H LANCET 1 411 970 063 FOY H NATURE 225 952 970 064 FOY H LANCET 1 1336 970 065 VAN FURTH R NATURE 222 1296 969 066 GALDO A ARCH FR PEDIATR 16 954 959 067 GAROFF L J OBSTET GYNAECOL BR COMMONW 80 695 973 068 GEFFROY Y SEM HOP PARIS 47 1281 971 069 GEFFROY Y PRESSE MED 78 1107 970 070 GITLIN D IN: MASSEYEFF R 55 974 071 GITLIN D J CLIN INVEST 45 1826 966 072 GITLIN D J CLIN INVEST 46 1010 967 073 GITLIN D COMPAR BIOCHEM BIOL 21 327 967 074 GITLIN D BIOCHIM BIOPHYS ACTA 147 334 967 075 GITLIN D NATURE 228 995 970 076 GITLIN D CANCER RES 32 979 972 077 GOUSSEV AI INT J CANCER 7 207 970 078 HALBRECHT I NATURE 178 794 956 079 HARRIS R LANCET 1 429 974 080 HIRAI H GANN MOGR CANCER RES 14 19 973 081 HIRSZFELD L Z IMMUNITATS EXP THER 75 209 932 082 HULL EW J NATL CANCER INST 42 1035 969 083 HULL EW LANCET 1 779 970 084 HULL EW CLIN RES 17 403 969 085 HULL EW IN: ANDERSON NG 2 317 972 086 HYVARINEN M J PEDIATR 82 430 973 087 KARLSSON BW ACTA PAEDIATR SCAND 61 133 972 088 KARVOUNTZIS GG ANN INTERN MED 80 156 974 089 KEKOMAKI M INT J CANCER 8 250 971 090 KITHIER K IN: ANDERSON NG 2 351 972 091 KITHIER K NATURE 212 414 966 092 KITHIER K BIOCHIM BIOPHYS ACTA 160 135 968 093 KOHN J J CLIN PATHOL 23 733 970 094 KRESNO SB LANCET 1 1178 970 095 KRESNO SB PROTIDES BIOL FLUIDS 18 243 971 096 KROES R CANCER RES 32 1526 972 097 LARDINOIS R CLIN CHIM ACTA 37 81 972 098 LAURELL CB ANAL BIOCHEM 15 45 966 099 LIN TY SCAND J GASTROENTEROL SUPPL 6 223 970 100 MARKLEIN G Z KINDERHEILK 113 327 972 101 MARX JL SCIENCE 183 1279 974 102 MASOPUST J INT J CANCER 3 364 968 103 MASOPUST J ANN PAEDIATR 204 138 965 104 MASOPUST J PROTIDES BIOL FLUIDS 18 239 971 105 MASOPUST J PROTIDES BIOL FLUIDS 18 37 971 106 MASOPUST J PROTIDES BIOL FLUIDS 18 63 971 107 MASSEYEFF R PATHOL BIOL (PARIS) 20 703 972 108 MASSEYEFF R RICERCA 3 27 973 109 MASSEYEFF R IN: MASSEYEFF R 313 974 110 MASSEYEFF R BULL SOC MED AFRIQUE NOIRE 13 537 968 111 MAWAS C LANCET 1 1292 970 112 MAWAS C REV EUR ETUD CLIN BIOL 16 430 971 113 MAWAS C INT J CANCER 4 76 969 114 MURALT G DE HELV PAEDIATR ACTA 16 517 961 115 MURGITA RA J EXP MED 141 269 975 116 MURGITA RA J EXP MED 141 440 975 117 NECHAUD B DE INT J CANCER 8 71 971 118 NECHAUD B DE INT J CANCER 11 104 973 119 NECHAUD B DE PRESSE MED 77 1945 969 120 NEVIN NC LANCET 1 1383 973 121 NISHI S CANCER RES 30 2507 970 122 NISHI S PROTIDES BIOL FLUIDS 18 43 971 123 NISHI S GANN MOGR CANCER RES 14 79 973 124 NISHI S J IMMUNOL 109 957 972 125 NORGAARD-PEDERSEN B CLIN CHIM ACTA 48 345 973 126 OCONOR GT CANCER 25 1091 970 127 OKON E BR J CANCER 27 362 973 128 OLOVNIKOV AM BULL EXP BIOL MED 68 102 969 129 PEDERSEN K NATURE 154 575 944 130 PEROVA SD BULL EKSPRE BIOL MEDIT 71 45 971 131 PORTUGAL ML INT J CANCER 6 383 970 132 PREHN RT IN: TRENTIN JJ 105 967 133 PREHN RT IN: MIHICH E 265 967 134 PURVES LR CANCER 25 1261 970 135 PURVES LR S AFR MED J 44 590 970 136 PURVES LR LANCET 1 1007 973 137 PURVES LR CANCER 31 578 973 138 PURVES LR S AFR MED J 42 1138 968 139 ROBERTS JAF INTRO MEDICAL GENETICS 970 140 RUOSLAHTI E INT J CANCER 7 218 971 141 RUOSLAHTI E IN: ANDERSON NG 2 345 972 143 SELLER MJ LANCET 2 73 973 144 SELLER MJ LANCET 1 428 974 145 SEPPALA M INT J FERTIL 18 206 973 146 SEPPALA M OBSTET GYNECOL 42 613 973 148 SEPPALA M AM J OBSTET GYNECOL 112 208 972 149 SEPPALA M J PERINATAL MED 1 104 973 150 SEPPALA M AM J OBSTET GYNECOL 115 48 973 151 SEPPALA M ANN MED EXP BIOL FENNIAE 45 16 967 152 SIMONS MJ LANCET 1 1234 974 153 SMITH CJ BIOCHIM BIOPHYS ACTA 317 231 973 154 SMITH JB INT J CANCER 8 421 971 155 SWARTZ SK IN: MASSEYEFF R 97 974 156 TATARINOV YS VOPR MED KHIM 10 90 964 157 TATARINOV YS VOPR MED KHIM 10 584 964 158 URIEL J NATURE NEW BIOL 244 190 973 159 URIEL J BIOCHEM BIOPHYS RES COMMUN 46 1175 972 160 URIEL J C R ACAD SCI (D)(PARIS) 265 75 967 161 URIEL J PRESSE MED 76 1415 968 162 WALD NJ BR MED J 1 651 975 163 WALD NJ LANCET 1 765 974 164 WALDMANN TA LANCET 2 1112 972 165 WARD AM LANCET 2 345 974 166 WARNOCK ML CLIN CHIM ACTA 24 5 969 167 WATABE H CANCER RES 31 1192 971 168 ZELTZER PM LANCET 1 373 974 169 ZIMMERMAN EF BIOCHEM J 134 807 973 170 ZIZKOVSKY V IN: MASSEYEFF R 125 974 CT 1 SELL S J NATL CANCER INST 60 19 978 2 CZOKALO M EXP PATHOL (JENA) 17 176 979 3 SCHLAGER SI J NATL CANCER INST 70 753 983 4 ROSENTHAL SR BULL INSTITUT PASTEUR 81 55 983 5 SELL S DEVELOP BRAIN RES 22 49 985 6 FUHRMANN W HUM GENET 69 47 985 7 BARFORD DA AM J OBSTET GYNECOL 151 1038 985 8 WONG LT J CHROMATOGR 341 452 985 9 STAPLES J LANCET 2 1277 986 PN 75098 RN 00264 AN 76146441 AU Danes-B-S. TI The search for relevant cell culture research in cystic fibrosis: one researcher's opinion. SO J-Med-Genet. 1975 Dec. 12(4). P 405-7. MJ CELLS-CULTURED. CYSTIC-FIBROSIS: pa. RESEARCH. MN CELL-LINE. CYSTIC-FIBROSIS: fg. HUMAN. EX Recently there has been a plethora of research approaches using cell culture to study cystic fibrosis. Although these efforts can be lauded as attempts to understand one of the most common genetic disorders affecting the Caucasian population, such research should be relevant to the clinical entity known as cystic fibrosis. The ultimate objectives of this research should be to determine the, as yet unknown, basic defect and to develop an assay for heterozygote detection. RF 001 BARTMAN J J PEDIATR 76 430 970 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 003 BOWMAN BH SCIENCE 164 325 969 004 CAUDILL M LANCET 1 32 974 005 CONOVER JH PEDIATR RES 7 224 973 006 CONOVER JH PEDIATR RES 7 220 973 007 CONOVER JH LANCET 1 1122 973 008 CONOVER JH LANCET 2 1501 973 009 DANES BS BIRTH DEF ORIG ART SER 8 114 972 010 DANES BS LANCET 2 765 973 011 DANES BS BIOCHEM GENET 12 359 974 012 DANES BS LANCET 2 437 969 013 DANES BS J EXP MED 129 775 969 014 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 015 DANES BS J EXP MED 136 1313 972 016 FARBER S J MICH MED SOC 44 587 945 017 GIBSON LE LANCET 2 189 970 018 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 019 JOHANSEN PG LANCET 1 455 968 020 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 021 NADLER HL LANCET 2 84 969 022 PALLAVICINI JC J PEDIATR 77 280 970 023 RENNERT OM IN: MANGOS JA 41 973 024 ROBERTSON JA LANCET 1 1256 974 025 DI SANTAGNESE PA N ENGL J MED 277 1287 967 026 SPOCK A PEDIATR RES 1 173 967 027 WOOD RE LANCET 2 1452 973 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 75099 RN 00265 AN 76097649 AU Govan-J-R. TI Mucoid strains of Pseudomonas aeruginosa: the influence of culture medium on the stability of mucus production. SO J-Med-Microbiol. 1975 Nov. 8(4). P 513-22. MJ CULTURE-MEDIA. MUCUS: se. PSEUDOMONAS-AERUGINOSA: me. MN ACETYLCYSTEINE: pd. CETRIMONIUM-COMPOUNDS: pd. CITRATES: pd. CYSTIC-FIBROSIS: mi. DEOXYCHOLIC-ACID: pd. FERRIC-COMPOUNDS: pd. HUMAN. LACTOSE: pd. PHOSPHATIDYLCHOLINES: pd. NEUTRAL-RED: pd. POLYSORBATES: pd. PSEUDOMONAS-AERUGINOSA: gd, ip. SODIUM-DODECYL-SULFATE: pd. SPECIES-SPECIFICITY. SURFACE-ACTIVE-AGENTS: pd. THIOSULFATES: pd. AB Mucoid strains of Pseudomonas aeruginosa isolated from the respiratory tract of patients with cystic fibrosis (CF), those obtained from non-CF patients, and those obtained in vitro by the action of phage, were found to be stable in their mucoid colonial form when serially subcultured on deoxycholate-citrate agar. The ability of anionic, cationic and neutral surfactants to stabilise mucus production is described. The possible importance of dipalmitoyl lecithin as a stabilising agent for mucus production in vivo is considered, with particular reference to the role of mucoid P. aeruginosa in CF disease. RF 001 BROWN ES AM J PHYSIOL 207 402 964 002 BURNS MW LANCET 1 270 968 003 DIAZ F J INFECT DIS 121 269 970 004 DOGGETT RG APPL MICROBIOL 18 936 969 005 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 006 DOGGETT RG J PEDIATR 68 215 966 007 DOGGETT RG J BACTERIOL 87 427 964 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 009 IACOCCA VF AM J DIS CHILD 106 315 963 010 KELLY M J GEN MICROBIOL 27 305 962 011 KUENZIG MC J APPL PHYSIOL 20 779 965 012 MARTIN DR J MED MICROBIOL 6 111 973 013 SCHWARZMANN S INFECT IMMUN 3 762 971 014 STANISICH V GENETICS 61 327 969 015 STANISICH V GENET RES CAMB 13 57 969 016 SUTHERLAND IW J GEN MICROBIOL 39 373 965 017 THANNHAUSER SJ J BIOL CHEM 166 669 946 018 WILLIAMS RJ J MED MICROBIOL 6 409 973 019 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 VERON M BULL INSTITUT PASTEUR 74 295 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 BROWN MRW J ANTIMICROB CHEMOTHER 3 198 977 4 JONES SE J PHARMACY PHARMACOL 29 P 69 977 5 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 6 GOVAN JRW J ANTIMICROB CHEMOTHER 4 233 978 7 MARKOWITZ SM INFECT IMMUN 22 530 978 8 MIAN FA J BACTERIOL 134 418 978 9 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 27 585 979 10 HOLLOWAY BW MICROBIOL REV 43 73 979 11 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 12 GOVAN JRW J GEN MICROBIOL 110 229 979 13 MARRIE TJ J INFECT DIS 139 357 979 14 THOMASSEN MJ J INFECT DIS 140 873 979 15 BOYCE JR LANCET 2 268 980 16 CHENG KJ CAN J MICROBIOL 26 1104 980 17 LAM J INFECT IMMUN 28 546 980 18 RUHEN RW J CLIN PATHOL 33 1221 980 19 FYFE JAM J GEN MICROBIOL 119 443 980 20 COSTERTON JW CRC CRIT REV MICROBIOL 8 303 981 21 IRVIN RT ANTIMICROB AGENTS CHEMOTHER 19 1056 981 22 CHENG KJ CAN J MICROBIOL 27 461 981 23 OHMAN DE INFECT IMMUN 33 142 981 24 COSTERTON JW ANNU REV MICROBIOL 35 299 981 25 GOVAN JRW J GEN MICROBIOL 125 217 981 26 MACONE AB N ENGL J MED 304 1445 981 27 BAKER NR CURRENT MICROBIOL 7 35 982 28 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 29 BOYCE JR INFECT IMMUN 37 840 982 30 BOYCE JR INFECT IMMUN 37 695 982 31 CHAN R INFECT IMMUN 37 1170 982 32 OHMAN DE INFECT IMMUN 37 662 982 33 JARRELL KF J INVERTEBRATE PATHOL 39 395 982 34 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 35 COSTERTON JW REV INFECT DIS 5 S867 983 36 OMBAKA EA REV INFECT DIS 5 S880 983 37 COSTERTON JW PROG FOOD NUTR SCI 7 91 983 38 OLIVER AM J CLIN LAB IMMUNOL 10 221 983 39 HACKING AJ J GEN MICROBIOL 129 3473 983 40 COSTERTON JW REV INFECT DIS 6 S608 984 41 MORRISON AJ REV INFECT DIS 6 S627 984 42 KEREN G J INFECT 9 22 984 43 GRISTINA AG ORTHOP CLIN NORTH AM 15 517 984 44 CHAN R J CLIN MICROBIOL 19 8 984 45 MILLER RV J CLIN MICROBIOL 19 717 984 46 COSTERTON JW DEVELOP IND MICROBIOL 25 363 984 47 CHAN R CAN J MICROBIOL 30 451 984 48 LAMBE DW CAN J MICROBIOL 30 809 984 49 MESHULAM T CLIN IMMUNOL IMMUNOPATHOL 32 151 984 50 MAYBERRYCARSON KJ INFECT IMMUN 43 825 984 51 MILLS J INFECT IMMUN 43 359 984 52 HANSEN JB APPL ENVIRON MICROBIOL 47 704 984 53 CUTRI SS J APPL BACT 57 291 984 54 DARZINS A J BACTERIOL 159 9 984 55 LINKER A J BACTERIOL 159 958 984 56 MARRIE TJ J CLIN MICROBIOL 22 924 985 57 COSTERTON JW DEVELOP IND MICROBIOL 26 249 985 58 FERRIS FG BIOSCIENCE 35 172 985 59 MARCUS H INFECT IMMUN 47 723 985 60 TAJIMA M JAP J VET SCI 47 217 985 61 SHABTAI Y APPL ENVIRON MICROBIOL 49 192 985 62 LUZAR MA INFECT IMMUN 50 577 985 63 GRISTINA AG J BONE JOINT SURG 67A 264 985 64 DARZINS A J BACTERIOL 161 249 985 65 OHMAN DE J BACTERIOL 162 1068 985 66 GRISTINA AG SCIENCE 228 990 985 67 MACGEORGE J J MED MICROBIOL 21 331 986 68 KRIEG DP J CLIN MICROBIOL 24 986 986 69 MAYBERRYCARSON KJ MICROBIOS 48 189 986 70 SHABTAI Y APPL ENVIRON MICROBIOL 52 146 986 71 REED WP SURGERY 99 308 986 72 BANERJEE PC ARCH MICROBIOL 145 408 986 73 DERETIC V J BACTERIOL 165 510 986 74 DERETIC V BIO TECHNOL 5 469 987 75 ANASTASSIOU ED J CLIN MICROBIOL 25 656 987 76 DERETIC V J BACTERIOL 169 351 987 PN 75100 RN 00266 AN 75171532 AU Oppenheimer-E-H. Esterly-J-R. TI Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. SO J-Pediatr. 1975 May. 86(5). P 683-9. MJ CYSTIC-FIBROSIS: pa. LIVER-CIRRHOSIS-BILIARY: et. LIVER: pa. MN AGE-FACTORS. AUTOPSY. BILE-DUCTS-INTRAHEPATIC: pa. CHOLESTASIS: et, pa. CYSTIC-FIBROSIS: co. HUMAN. INFANT. INFANT-NEWBORN. LIVER-CIRRHOSIS-BILIARY: pa. MALE. MUCUS. AB Focal biliary cirrhosis is an uncommon finding in infants with cystic fibrosis, but it is present in more than a fifth of surviving children and adolescents. It was found at postmortem examination in only five of 47 infants with CF younger than 3 months, in five of 32 infants from 3 to 12 months, and in 18 of 67 children older than 1 year. In infants under 3 months, excessive mucus in intrahepatic bile ducts was seen in 11 necropsies; in 15 others there were only nonspecific periportal changes. Cholestasis was found in the livers of 18 of the 26 infants. Excessive mucus in the biliary tree was occasionally associated with periportal changes and cholestasis in older infants. The periportal changes, which are regarded as nonspecific, were never found in infants more than 1 year of age. RF 001 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 002 DI SANTAGNESE PA PEDIATRICS 18 387 956 003 CRAIG JM AM J DIS CHILD 93 357 957 004 GRAND RJ CLIN PEDIATR 9 588 970 005 KOPEL FB GASTROENTEROLOGY 62 483 972 006 BERNSTEIN JG AM J DIS CHILD 99 804 960 007 HOLSCLAW DS AM J DIS CHILD 109 101 965 008 GATZIMOS CD AM J DIS CHILD 89 182 955 009 TALAMO RC AM J DIS CHILD 115 74 968 010 VALMAN HB ARCH DIS CHILD 46 805 971 011 JEAN R ARCH FR PEDIATR 27 849 970 012 TAYLOR WF AM J DIS CHILD 123 161 972 013 TOULOUKIAN RJ ARCH SURG 106 58 973 014 RAGER R AM J PATHOL 74 6A 974 015 SHIER KJ CAN MED ASSOC J 89 645 963 016 MUECKE D DTSCH GESUNDHEITSW 27 1723 972 017 ROSCHLAU G DTSCH GESUNDHEITSW 27 1722 972 CT 1 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 2 WALKER WA PEDIATR CLIN NORTH AM 22 929 975 3 ISENBERG JN AM J GASTROENTEROL 65 134 976 4 JAVITT NB GASTROENTEROLOGY 70 1172 976 5 STERN RC GASTROENTEROLOGY 70 645 976 6 SCHUSTER SR J PEDIATR SURG 12 201 977 7 MATHIS RK J PEDIATR 90 864 977 8 ROSENSTEIN BJ J PEDIATR 91 1022 977 9 ROY CC N ENGL J MED 297 1301 977 10 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 11 BUTS JP J PEDIATR 92 729 978 12 VITULLO BB J PEDIATR 93 1060 978 13 STARKEY BJ MONOGR PAEDIATR 10 12 979 14 FINBERG L N ENGL J MED 300 420 979 15 CUNNINGHAM DG J COMPUT ASSIST TOMOGR 4 151 980 16 ARBORGH B SCAND J GASTROENTEROL 15 73 980 17 DAVIDSON GP J CLIN PATHOL 33 390 980 18 GORIUP U HELV PAEDIATR ACTA 35 177 980 19 MIELIVERGANI G ARCH DIS CHILD 55 696 980 20 BALISTRERI WF J PEDIATR 97 689 980 21 WILLI UV AM J ROENTGENOL 134 1005 980 22 PARK RW GASTROENTEROLOGY 81 1143 981 23 LLOYDSTILL JD J PEDIATR 99 580 981 24 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 25 MOWAT AP CLIN GASTROENTEROL 11 171 982 26 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 27 GASKIN KJ PEDIATR RES 16 554 982 28 CHOW CW EUR J PEDIATR 139 240 982 29 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 30 SPIVAK W J PEDIATR GASTROENTEROL NUTR 2 381 983 31 SCHWARTZ AM J COMPUT ASSIST TOMOGR 7 530 983 32 WATSON S CLIN PEDIATR 22 30 983 33 DOHERTY DE SOUTH MED J 76 1580 983 34 PERKINS WG CLIN PEDIATR 24 107 985 35 ROBB TA GUT 26 1246 985 36 FEIGELSON J ANN PEDIATR (PARIS) 32 218 985 37 HULTCRANTZ R HEPATOLOGY 6 881 986 38 KING A PEDIATR RES 20 536 986 39 TANNER MS J ROY SOC MED 79 38 986 40 MCHUGO JM BR J RADIOL 60 137 987 PN 75101 RN 00267 AN 75153056 AU Scanlin-T-F. Shapiro-R-S. Rosenlund-M-L. TI Letter: Vocal cord paralysis in cystic fibrosis. SO J-Pediatr. 1975 Jun. 86(6). P 984-5. MJ CYSTIC-FIBROSIS: co. VOCAL-CORD-PARALYSIS: et. MN ADOLESCENCE. COUGH: pp. CYSTIC-FIBROSIS: rh. HUMAN. HYPERTROPHY: ra. MALE. POLYTETRAFLUOROETHYLENE: tu. TOMOGRAPHY-X-RAY. SUPPORT-U-S-GOVT-NON-P-H-S. VOCAL-CORD-PARALYSIS: ra. VOCAL-CORDS: ra. EX Many conditions have been reported to cause vocal cord paralysis. To our knowledge cystic fibrosis has not been described as one of these conditions. A 15-year-old white male with cystic fibrosis, who has been treated at our hospital, reported hoarseness of two week's duration on a routine clinic visit in March, 1973. In March, 1974, mirror laryngoscopy revealed a paralysis of the left vocal cord in the paramedian position, consistent with paralysis of the left recurrent laryngeal nerve. It is felt that the vocal cord paralysis in this patient with cystic fibrosis is due to compression of the left recurrent laryngeal nerve by an enlarged pulmonary artery. RF 001 DEDO HH LARYNGOSCOPE 80 1455 970 002 DEDO HH ANN OTOL RHINOL LARYNGOL 82 661 973 PN 75102 RN 00268 AN 75213258 AU Desor-J-A. Maller-O. TI Taste correlates of disease states: cystic fibrosis. SO J-Pediatr. 1975 Jul. 87(1). P 93-6. MJ CYSTIC-FIBROSIS: co. TASTE-DISORDERS: et. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. HUMAN. MALE. PANCREAS: pa. SODIUM-CHLORIDE: du. TASTE-BUDS: pp. TASTE-DISORDERS: di. SUPPORT-U-S-GOVT-P-H-S. EX The purpose here was to test for a generalized abnormal sensitivity of the external chemoreceptive systems using a standardized method that separates sensory activity from biasing factors known to affect measurement of sensitivity. The frequency distributions of salt taste sensitivity for patients with cystic fibrosis are given. Normal siblings and control patients did not differ statistically, and they were combined into a single control group for analyses. The patients with cystic fibrosis did not differ as a group from the normal subjects in salt sensitivity. There were, however, two children with cystic fibrosis with taste sensitivities well below those observed among the normal children. No age or sex differences in sensitivity were observed. The taste sensitivity of the children with cystic fibrosis did not correlate with the Shwachman and Kulczycki rating of the severity of their disease. Of the seven children who had determinations of sweat electrolytes at the time of taste testing, three failed to excrete sufficient amounts for measurements. Patients with cystic fibrosis, as a group, were not found to have more sensitive taste acuity than normal. This corroborates the findings of Wotman and associates and strongly suggests that a general abnormality of the taste system is not routinely symptomatic of cystic fibrosis. Henkin and Powell hypothesized that extremely acute taste is related to elevations in the concentration of electrolytes in sweat. This seems unlikely since the patients given sweat tests in the present study all had the high levels of sodium and chloride characteristic of cystic fibrosis, yet all had taste sensitivities in the normal range. Wotman and associates suggested that perhaps the severity of the disease accounts for the appearance of hypersensitivity in the chemoreceptive systems. The clinical ratings of the severity of the disease did not correlate with taste sensitivity. RF 001 HENKIN RI SCIENCE 138 1107 962 002 WOTMAN S AM J DIS CHILD 108 372 964 003 MANLAPAS FC J PEDIATR 66 8 965 004 BLAKESLEE AF PROC NAT ACAD SCI USA 18 120 932 005 BARNICOT NA ANN EUGEN 16 119 951 006 COOMBS CH MATH PSYCHOL 165 970 007 SHWACHMAN H AM J DIS CHILD 96 6 958 CT 1 BARTOSHUK LM AM J CLIN NUTR 31 1068 978 2 BURGE JC KIDNEY INT 15 49 979 3 RUSSELL RM ANN INTERN MED 99 227 983 4 SCHIFFMAN SS N ENGL J MED 308 1275 983 5 WEIFFENBACH JM CHEM SENSES 9 193 984 PN 75103 RN 00269 AN 75078947 AU Baig-M-M. Cetorelli-J-J. Roberts-R-M. TI Plasma membrane components of skin fibroblasts from normal individuals and patients with cystic fibrosis. SO J-Pediatr. 1975 Jan. 86(1). P 72-6. MJ CELL-MEMBRANE: an. CYSTIC-FIBROSIS: pa. FIBROBLASTS: ul. GLYCOPROTEINS: an. PROTEINS: an. SKIN: cy. MN BIOPSY. CARBON-RADIOISOTOPES. CATTLE. CELL-LINE. CELL-MEMBRANE: ul, ip. CENTRIFUGATION. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FIBROBLASTS: gd. FUCOSE: me. GLUCOSAMINE: me. HETEROZYGOTE. HUMAN. LEUCINE: me. SKIN: pa. TRITIUM. SUPPORT-U-S-GOVT-P-H-S. AB Plasma membranes have been isolated without proteolytic modification from fibroblast lines derived from patients with CF, from heterozygous parents, and from normal children. The cells had been grown in the presence of 3H- OR 14C- labeled L-leucine, D- glucosamine, and L-fucose. Membranes were mixed in suitable combinations to allow comparisons to be made between the different cell types. No differences in the plasma membrane composition, as revealed by divergence in 3H- or 14C-profiles, could be detected after gel electrophoresis. Identical protein and glycoprotein components were present in approximately similar amounts in all groups of cells; RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 LOBECK CC IN: STANBURY JB 1605 972 003 GUGLER EC J PEDIATR 71 585 967 004 GIBSON LE PEDIATRICS 48 695 971 005 BARTMAN J J PEDIATR 76 430 970 006 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 007 DANES BS J EXP MED 129 775 969 008 DANES BS NATURE 222 685 969 009 FITZPATRICK DF NATURE NEW BIOL 235 173 972 010 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 011 NADLER HL LANCET 2 84 969 012 PALLAVICINI JC J PEDIATR 77 280 970 013 RENNERT OM IN: MANGOS JA 41 973 014 RENNERT OM CLIN PEDIATR 11 351 972 015 PITOT HC PROC SYMP MOLE BASIS HUM 14 972 016 BARLAND P J CELL BIOL 45 662 970 017 WEBER K J BIOL CHEM 244 4406 969 018 TURNER JC INT J APPL RADIAT ISOTOP 19 557 968 CT 1 CHANGUS JE AM J PATHOL 80 317 975 2 CHOU L PEDIATR RES 10 176 976 3 DANES BS TEX REP BIOL MED 34 135 976 4 WARD JB TEX REP BIOL MED 34 11 976 5 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 6 WOOD RE AM REV RESPIR DIS 113 833 976 7 SANTAGNESE PAD N ENGL J MED 295 481 976 8 YOKOYAMA M PEDIATR RES 11 765 977 9 QUISSELL DO ANAL BIOCHEM 79 240 977 10 OWEN E J MOL MED 3 203 978 11 PENA SDJ PEDIATR RES 12 887 978 12 PEARSON RD PEDIATR RES 13 834 979 13 JAKEL HP BIOL ZENTRALBL 98 55 979 14 BAIG MM CIRC RES 46 651 980 15 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 16 HARRIS A CLIN CHIM ACTA 128 41 983 17 KARLSSON S J MED GENET 21 441 984 PN 75104 RN 00270 AN 76047850 AU Bang-F-B. TI Comparative pathology of marine invertebrates and the study of human disease. SO J-Pediatr. 1975 Dec. 87(6 PT 2). P 1062-6. MJ MARINE-BIOLOGY. MODELS-BIOLOGICAL. MN ANIMAL. ARACHNIDA: mi. CLAMS: me. CRABS: im. CYSTIC-FIBROSIS: me. HUMAN. MUCUS: se. OYSTERS: me. STARFISH: im. EX Studies on an infection with gram-negative bacteria in Limulus led to the discovery of the extreme sensitivity of the amebocytes to a gram-negative endotoxin. This led to the development of a method for measuring very small amounts of endotoxin in biologic fluids and in human blood. Yet, had the original observation of Cantacuzene on the large common edible spider crab been followed, this remarkable sensitivity of marine arthropod blood to endotoxin would have been recognized much earlier. The original work of Metchnikoff on sea star larvae stimulated him to develop the major theory of phagocytosis but, until the adult animal of the same genus was re-examined half a century later, the presence of a powerful substance which selectively inhibits growth of lymphocytes following primary immunization remained unknown. Though mucus secretion is a fundamental characteristic of most invertebrate phyla and all vertebrates, little is known about the regulatory mechanism of the secretory process; studies of the responses of specialized invertebrate secretory cells to infection have now led to a new means of studying macromolecular control of mucus secretion in man. These three stories emphasize the continuing need to study disease as a biologic phenomenon. RF 001 ZEPP HD NATURE NEW BIOL 229 119 971 002 CONOVER JH CURR TOP MICROBIOL IMMUNOL 55 85 971 003 POCOCK RI ANN MAG NAT HIST 9 256 902 004 LOEB L J MED RES 7 145 902 005 LOEB L UNIV PENNSYLVANIA MED BULL 18 91 905 006 LEVIN J BULL JOHNS HOPKINS HOSP 115 337 964 007 HARTLINE HK HARVEY LECTURES 37 39 941 008 BANG FB BULL JOHNS HOPKINS HOSP 98 325 956 009 LEVIN J THROMB HAEMOST 19 186 968 010 YOUNG NS J CLIN INVEST 51 1790 972 011 SULLIVAN JD JR APPL MICROBIOL 28 1023 974 012 LEVIN J N ENGL J MED 283 1313 970 013 LEVIN J ANN INTERN MED 76 1 972 014 NACHUM R N ENGL J MED 289 931 973 016 COOPER JF J LAB CLIN MED 78 138 971 019 KLEIN KB LANCET 2 50 974 020 LOEB L J MED RES 10 407 903 021 PEARSON AD LANCET 1 1194 973 022 CANTACUZENE J C R SOC BIOL (PARIS) 84 1007 921 023 METCHNIKOFF E LECONS PATHOLOGIE COMPAREE DE 892 024 METCHNIKOFF O LIFE OF ELIE METCHNIKOFF 1845 921 025 BANG FB J INSECT PATHOL 4 401 962 026 REINISCH CL CELL IMMUNOL 2 496 971 027 REINISCH CL NATURE 250 349 974 029 PRENDERGAST RA NATURE 227 277 970 030 PRENDERGAST RA ANN NY ACAD SCI 234 7 974 031 WILLENBORG DO J EXP MED 139 820 974 033 ANON ANN INST PASTEUR 42 137 934 034 CANTACUZENE J C R SOC BIOL (PARIS) 92 1133 925 035 CANTACUZENE J ARCH ROUM PATHOL EXP 1 7 928 036 BANG FB CAHIERS BIOL MAR 3 363 962 037 BANG BG AM J PATHOL 68 407 972 039 BOWMAN BH SCIENCE 164 325 969 040 BANG BG LANCET 2 1292 974 PN 75105 RN 00271 AN 76007103 AU Seidmon-E-J. Mosovich-L-L. Neter-E. TI Colonization by Enterobacteriaceae of the respiratory tract of children with cystic fibrosis of the pancreas and their antibody response. SO J-Pediatr. 1975 Oct. 87(4). P 528-33. MJ ANTIBODY-FORMATION. CYSTIC-FIBROSIS: co. ENTEROBACTERIACEAE-INFECTIONS: co. RESPIRATORY-TRACT-INFECTIONS: co. MN ADOLESCENCE. ADULT. ANTIBODIES-BACTERIAL. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im. ENTEROBACTERIACEAE-INFECTIONS: im. ENTEROBACTERIACEAE: im. FEMALE. HUMAN. INFANT. MALE. RESPIRATORY-TRACT-INFECTIONS: im. SUPPORT-U-S-GOVT-P-H-S. AB Of 72 patients with fibrosis, 49 harbored Enterobacteriaceae in the respiratory tract, including Escherichia coli, Klebsiella, and Enterobacter. Colonization by two to four genera was documented in 29 subjects. Staphylococcus aureus was recovered from 44 of these 49 patients. The distribution of serogroups of E. coli was similar to that seen in patients with urinary tract infection. Antibody response against the O antigens of the patients' own Enterobacteriaceae was documented in 29 of these 49 children and encountered more often in patients with severe disease. Colonization by Enterobacteriaceae in the absence of Pseudomonas aeruginosa was seen more frequently in children with the mild form of the illness. RF 001 HUANG NN J PEDIATR 59 512 961 002 DOGGETT RG J BACTERIOL 87 427 964 003 DOGGETT RG J PEDIATR 68 215 966 004 IACOCCA VF AM J DIS CHILD 106 315 963 005 FEIGELSON J ARCH FR PEDIATR 24 1135 967 006 MEARNS MB ARCH DIS CHILD 47 902 972 007 MAY JR ARCH DIS CHILD 47 908 972 008 EDWARDS PR IDENTIFICATION OF ENTEROBACTE 972 009 NETER E ANN NY ACAD SCI 66 141 956 010 DIAZ F J INFECT DIS 121 269 970 011 HUANG NN J PEDIATR 78 338 971 012 NETER E N ENGL J MED 261 1162 959 013 ELSEA WR PUBLIC HEALTH REP 82 347 967 014 NETER E AM J PUBLIC HEALTH 52 61 962 015 NETER E IN: PRIER JE 37 974 016 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 017 DOGGETT RG INFECT IMMUN 6 628 972 018 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 CT 1 NETER E ZENTRALBL BAKT MIKROB HYG (A) 235 3 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 DOLBY JM J HYG LOND 78 235 977 4 OLLING S SCAND J INFECT DIS 1977 7 977 5 KLINGER JD J INFECT DIS 138 49 978 6 NETER E ZENTRALBL BAKT PARA INFEK HYG 243 349 979 7 BURDON MG CLIN GENET 17 249 980 8 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 9 FRIEND PA J INFECT 13 55 986 PN 75106 RN 00272 AN 76007111 AU Ellis-C-E. Hill-D-E. TI Growth, intelligence, and school performance in children with cystic fibrosis who have had an episode of malnutrition during infancy. SO J-Pediatr. 1975 Oct. 87(4). P 565-8. MJ CYSTIC-FIBROSIS: pp. GROWTH. INFANT-NUTRITION-DISORDERS: pp. INTELLIGENCE. MN ACHIEVEMENT. CHILD. CYSTIC-FIBROSIS: co. FEMALE. FOLLOW-UP-STUDIES. HUMAN. INFANT-NUTRITION-DISORDERS: et. MALE. SOCIAL-ADJUSTMENT. EX In this study, we have compared two groups of children with cystic fibrosis. One group of CF children had severe malnutrition in the first year of life, whereas the second group of CF children had not been malnourished. The catch-up growth experienced by both the control and malnourished groups was impressive and was generally complete in terms of height and weight for age. This finding is consistent with other reports regarding catch-up growth following malnutrition associated with celiac disease, anorexia nervosa, renal acidosis, Cushing syndrome, and hypothyroidism. Though the malnourished group tended to have lower IQ and school performance scores, the differences were not statistically significant except for backward digit span. That difference may indicate an organic impairment due to malnutrition or to any of several other factors. Our data fail to demonstrate any permanent or devastating effect of early malnutrition during a very rapid growth phase on the subsequent growth, IQ, and school performance in children with cystic fibrosis studied at age 7 to 10 years. RF 001 SCRIMSHAW NS MALNUTRITION LEARNING AND BEH 968 002 CRAVIOTO J PEDIATRICS PART 2 SUPPL 38 319 966 003 LATHAM MC PHYSIOL REV 54 541 974 004 KAPLAN BJ PSYCHOL BULL 78 321 972 005 DOBBING J PEDIATRICS 53 2 974 006 GRAHAM GG AM J CLIN NUTR 25 1184 972 007 GOMEZ F J TROP PEDIATR 2 77 956 008 SPROUL A J PEDIATR 65 664 964 009 KULCZYCKI LL CLIN PROC CHILD HOSP DC 25 320 969 010 LLOYD-STILL JD PEDIATRICS 54 306 974 CT 1 CHEEK DB AM J CLIN NUTR 29 1149 976 2 HILL DE POSTGRAD MED 62 103 977 3 EISENBERG L BR J PSYCHIATRY 131 225 977 4 BROZEK J ANNU REV PSYCHOL 29 157 978 5 CELEDON JM EARLY HUM DEV 3 267 979 6 CELEDON JM J MENT DEFIC RES 24 27 980 7 MACLEAN WC J PEDIATR 97 316 980 8 RICCIUTI HN J AM DIET ASSOC 79 115 981 9 GRAHAM GG AM J DIS CHILD 136 348 982 10 ANDRASSY RJ J PEDIATR SURG 18 431 983 11 ASHWORTH A NUTR REV 44 157 986 PN 75107 RN 00273 AN 75091389 AU Moss-A-J. Finkelstein-S. Crudup-C. Young-G-A. Dooley-R-R. Osher-A-B. TI Absorption of digoxin in children with cystic fibrosis. SO J-Pediatr. 1975 Feb. 86(2). P 295-7. MJ CYSTIC-FIBROSIS: me. DIGOXIN: me. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DIGOXIN: ad, tu. HUMAN. PULMONARY-HEART-DISEASE: dt, et. AB The absorption of digoxin in cystic fibrosis was evaluated in 16 subjects by assessing the relationship between dosage expressed in mug/kg/day and serum digoxin concentration. The results indicate that the same relationship exists between maintenance dosage and serum levels in these patients and in patients without cystic fibrosis. Thus, no evidence of impaired absorption was found. RF 001 ROYCE SW PEDIATRICS 8 255 951 002 WHITE WF J AM PHARMACOL ASSOC 41 42 952 003 GELLIS SS CURRENT PEDIATRIC THERAPY 137 973 004 MARCUS FI CIRCULATION 34 865 966 005 SMITH TW N ENGL J MED 281 1212 969 006 FOGELMAN AM LANCET 2 727 971 007 BROWN DD J LAB CLIN MED 82 201 973 008 HUFFMAN DH JAMA 222 957 972 009 ROGERS MC N ENGL J MED 287 1010 972 010 OMALLEY K ARCH DIS CHILD 48 55 973 011 HAYES CJ PEDIATRICS 52 561 973 012 HERNANDEZ A PEDIATRICS 44 418 969 013 BELLER GA N ENGL J MED 284 989 971 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 WETTRELL G CLIN PHARMACOKINET 2 17 977 3 KOUP JR DRUG INTEL CLIN PHARM 12 244 978 4 SCHNEIWEISS F DRUG INTEL CLIN PHARM 16 132 982 5 MOSS AJ PEDIATRICS 70 728 982 6 COATES AL CHEST 82 543 982 7 LESTER LA SEM RESPIR MED 6 285 985 8 WETTRELL G THER DRUG MONITOR 8 129 986 PN 75108 RN 00274 AN 76047933 AU Lukash-F. Zwiren-G-T. Andrews-H-G. TI Significance of absent vas deferens at hernia repair in infants and children. SO J-Pediatr-Surg. 1975 Oct. 10(5). P 765-9. MJ HERNIA-INGUINAL: su. VAS-DEFERENS: ab. MN CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. HUMAN. INFANT. KIDNEY: ab. MALE. VAS-DEFERENS: em. AB An absent vas deferens may be associated with unilateral renal agenesis or undiagnosed cystic fibrosis. Such patients should be further evaluated with a sweat chloride and intravenous pyelogram. The finding of absent vas deferens during exploration for an undescended testis does not provide absolute evidence for absence of the testis, since these structures have independent embryologic origins. RF 001 EMANUEL B AM J DIS CHILD 127 17 974 002 GRACEY M N ENGL J MED 280 276 969 003 HOLSCLAW DS J UROL 106 568 971 004 HOLSCLAW DS BR MED J 3 356 969 005 HOLSCLAW DS PEDIATRICS 48 442 971 006 KAPLAN E N ENGL J MED 279 65 968 007 LANDING BH ARCH PATHOL 88 569 969 008 OLSON JR J CLIN PATHOL 22 725 969 009 OPPENHEIMER EH J PEDIATR 75 806 969 010 SOLOWAY HM ANN SURG 109 267 939 011 WANG CI AM J DIS CHILD 119 236 970 012 AMELAR RD JAMA 213 12 970 013 CHARNY CW J UROL 93 399 965 014 COLLINS DC ANN SURG 95 715 932 015 DOROSHOW LW UROLOGICAL SURVEY 11 219 961 016 GUIZZETTI P CURR MED LIT 71 1867 918 017 HUTCH JA JAMA 219 1762 972 018 MICHELSON L J UROL 61 384 949 019 OCHSNER MG JAMA 222 1055 972 020 WHITEHORN C J UROL 72 685 954 021 YOUNG D BR J SURG 36 417 949 022 GILCHRIST FG SURVEY OF EMBRYOLOGY 385 968 023 VALMAN HB LANCET 2 566 969 024 EL-ITREBY AA INT J FERTIL 6 409 961 CT 1 PRIEBE CJ J PEDIATR SURG 14 834 979 2 PIROTH P UROLOGE (A) 18 276 979 3 MACMAHON RA J PEDIATR SURG 15 92 980 4 CHRISTENSON PJ J ANDROL 3 326 982 5 DEANE AM BR J UROL 54 298 982 6 SHAPIRO SR JAMA 247 517 982 7 FLACH A LANGENBECKS ARCH CHIR 361 343 983 PN 75109 RN 00275 AN 76097542 AU Brungard-K. Moser-G. Athreya-B-H. TI Size distribution of lymphocytes in juvenile rheumatoid arthritis. SO J-Rheumatol. 1975 Dec. 2(4). P 359-62. MJ ARTHRITIS-JUVENILE-RHEUMATOID: bl. LYMPHOCYTES: cy. MN ASTHMA: bl. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: bl. HUMAN. AB The diameters of circulating peripheral lymphocytes were measured in 28 children with juvenile rheumatoid arthritis. These were compared with lymphocyte measurements in 68 normal children, 19 children with asthma, and 12 children with cystic fibrosis. The average diameter of lymphocytes in normal children was found to be 11.2 microns. In contrast, the average diameter of lymphocytes in juvenile rheumatoid arthritis was 12.7 microns (P less than 0.0001). There were more lymphocytes with a diameter of 13 to 15 microns in children with juvenile rheumatoid arthritis than in normal children. RF 001 HEYN RM AM J DIS CHILD 125 789 973 002 ANON BULL RHEUM DIS 23 712 972 003 CARTWRIGHT GE DIAG LAB HEMATOL 141 968 004 WILLIAMS WJ HEMATOLOGY 19 972 005 CLEMENTS RJ ARTHRITIS RHEUM 17 347 974 006 WILLIAMS RC JR J CLIN INVEST 52 283 973 PN 75110 RN 00276 AN 76098876 AU Boon-W-H. TI Cystic fibrosis in a Chinese girl. SO J-Singapore-Paediatr-Soc. 1975 Oct. 17(2). P 96-102. MJ CYSTIC-FIBROSIS: di. MN BLACKS. CASE-REPORT. CHILD-PRESCHOOL. CHINA: eh. CYSTIC-FIBROSIS: fg, oc. FEMALE. HUMAN. INDIANS-NORTH-AMERICAN. INFANT. INFANT-NEWBORN. JAPAN: eh. KOREA: eh. LUNG-DISEASES: di. SINGAPORE. EX A four year old Chinese girl of parents who emigrated to Singapore from South China was found to be suffering from bronchiectasis as a result of repeated chest infections, and failure to thrive. Investigations confirmed the bronchiectasis, and, in the course of attempting to elucidate the course, sweat Cl and sweat Na were found to be raised. The patient occasionally passed soft bulky stools and the Vitamin A absorption test was abnormal. The pathogenesis, problems in diagnosis and the genetics are briefly reviewed. This is the first proved case of cystic fibrosis occurring in a child of pure Chinese stock, and the first to be described in South-East Asia. Cystic fibrosis occurring in non-Caucasians in reviewed. RF 001 AHARI H J TROP PEDIATR 11 14 965 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 BHAKOO ON INDIAN J PEDIATR 35 183 968 004 BRUNECKY Z J MED GENET 9 33 972 005 DANES BS BIRTH DEF ORIG ART SER 8 114 972 006 DANKS DM ANN HUM GENET 28 323 965 007 DI SANTAGNESE PA PEDIATRICS 22 507 958 008 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 009 GARROD AE Q J MED 6 242 913 010 HARRIS RL PEDIATRICS 41 733 968 011 IKAI K ACTA PAEDIATR JAP 7 23 965 012 KULCZYCKI LL CLIN PEDIATR 3 692 964 013 MARTINEZ JR PEDIATR RES 9 463 975 014 ROBERTS GBS ANN HUM GENET 24 127 960 015 SCHULZ IJ FED PROC 26 287 967 016 SPOCK A PEDIATR RES 1 173 967 017 WANG CI N ENGL J MED 279 1216 968 018 WRIGHT SW AM J HUM GENET 20 157 968 PN 75111 RN 00277 AN 76009445 AU Schwachman-H. Araki-H. Field-M. TI Letter: Detection of cystic fibrosis and heterozygotes. SO Lancet. 1975 Aug 23. 2(7930). P 372-3. MJ CYSTIC-FIBROSIS: fg. MN CYSTIC-FIBROSIS: di. HETEROZYGOTE. HUMAN. METHODS. EX During the past year, we have developed a new assay for cystic fibrosis by utilising the Ussing chamber technique and measuring the electrical properties of rat jejunum in the presence of the sera to be tested. The potential difference (P.D.) and resistance (R.) are measured with a sensitive electrometer at 5-minute intervals over 60 minutes. We were able to detect 90% of the heterozygotes. The possibility of detecting the remaining 10% is present, and can, we feel, be achieved by using a more concentrated specimen. RF 006 WOOD RE LANCET 2 1452 973 CT 1 BURDICK AB HUM HERED 27 366 977 PN 75112 RN 00278 AN 76029161 AU McEvoy-F-A. TI Letter: Essential fatty acids and cystic fibrosis. SO Lancet. 1975 Aug 2. 2(7927). P 236. MJ CYSTIC-FIBROSIS: bl. FATTY-ACIDS-ESSENTIAL: bl. MN ADULT. CELL-MEMBRANE: me. CHILD. ERYTHROCYTES: me. HUMAN. OXIDATION-REDUCTION. EX I have measured the fatty-acid profile of C.F. erythrocyte membranes in view of the fact that some variations in membrane activity have been observed with this system. The results show that there was a lowered content of linoleate (18:2) and arachidonate (20:4) in C.F. erythrocyte membranes, and the total essential-fatty-acid content was significantly lower than either control group. There was no significant difference in the concentrations of the other major fatty acids (palmitate, stearate, and oleate) and we could not observe any striking alteration in the concentration of any of the minor fatty-acid components of the membrane. It would thus appear that the overall fatty-acid pattern in C.F. erythrocyte membranes is altered only by a diminution in those fatty acids which are of dietary origin. The fact that there was considerable overlap between the C.F. and control data makes it rather unlikely that the fatty-acid profile closely reflects the biochemical lesion of the disease. When the essential-fatty-acid levels of the C.F. samples were compared with the Schwachman clinical scores of the patients, there was a surprisingly poor correlation (r = 0.49) which was not statistically significant. The correlation with the nutritional part of the clinical score was equally low. Diminished essential-fatty-acid content is often observed in many acute illnesses. In addition, the possibility exists that C.F. patients may be particularly susceptible to essential-fatty-acid depletion due to their pancreatic dysfunction. Further research on fatty-acid transport and metabolism in C.F. is clearly needed to establish the role of essential fatty acids in the disease. RF 002 ROSENLUND ML NATURE 251 719 974 003 ELLIOTT RB ARCH DIS CHILD 50 76 975 004 EMERY AEH LANCET 2 80 975 005 MCEVOY FA CLIN CHIM ACTA 54 195 974 006 GURR MI LIPID BIOCHEMISTRY 70 971 007 LOVE WC BR MED J 3 18 974 CT 1 WATTS R LANCET 2 983 975 2 HUBBARD VS LANCET 2 1302 977 3 DAVIS PB PEDIATR RES 12 703 978 4 KUTTY KM CLIN BIOCHEM 12 98 979 5 RIORDAN JR BIOCHIM BIOPHYS ACTA 574 39 979 6 HUBBARD VS CLIN CHIM ACTA 102 115 980 7 ROGIERS V CLIN CHIM ACTA 105 105 980 8 ROGIERS V PEDIATR RES 16 761 982 9 PARNHAM MJ AGENTS ACTIONS 14 223 984 10 ROGIERS V PEDIATR RES 18 704 984 PN 75113 RN 00279 AN 75099183 AU Conover-J-H. Conod-E-J. TI Letter: Complement in cystic fibrosis. SO Lancet. 1975 Jan 4. 1(7897). P 47-8. MJ COMPLEMENT. CYSTIC-FIBROSIS: im. MN CYSTIC-FIBROSIS: et. FEMALE. HISTAMINE-LIBERATION. HUMAN. IGG: an. IMMUNE-COMPLEX-DISEASE. MALE. SEX-RATIO. EX Lieberman reported that there was considerable overlap in quantitative C3 levels between all of the C.F. subjects tested, and the control group. His control group, however, was composed of subjects with bronchiectasis or chronic bronchitis, in whom C.F. had been ruled out. The use of a more proper control group by Lieberman, either in place of or in parallel with his "diseased" control groups, would have provided a more meaningful interpretation concerning the role of complement in cystic fibrosis. We agree that there is a tendency for C3 to be higher in C.F. females than in C.F. males. Although Lieberman was quick to dispel any notion of C3a being implicated in the aetiology of C.F., he overlooked the one facet of our initial study that still makes our hypothesis feasible - raised C3 levels in C.F. obligate heterozygotes. Hann et al. reported that serum-C5 was raised in both C.F. patients and their parents. Our initial data on C5 levels by radial immunodiffusion and haemolytic studies do not confirm this finding. We are supporting the hypothesis that C.F. is either a complement-caused or complement-mediated disorder, in which a C3a-like molecule (C3a, bradykinin) either alone or by complexing with IgG gives rise to the physiological and pathological state of C.F. RF 001 CONOVER JH LANCET 2 1501 973 002 LIEBERMAN J LANCET 1 1230 974 003 CONOVER JH PEDIATR RES 7 220 973 004 CONOVER JH LANCET 1 1194 973 005 HANN S LANCET 2 520 974 006 CONOVER JH LIFE SCI 14 253 974 007 DANES BS J EXP MED 137 1538 973 008 MOLENAAR J J IMMUNOL 112 1444 974 009 WILSON GB LIFE SCI 15 551 974 010 CONOVER JH CLIN RES 21 531 973 CT 1 WILSON GB PEDIATR RES 10 87 976 2 WEEKE B DAN MED BULL 23 155 976 3 LEDERBERG S AM J HUM GENET 28 597 976 4 WILSON GB TEX REP BIOL MED 34 51 976 5 GOTZ M EUR J PEDIATR 127 133 978 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 SANDERSON MJ PEDIATR RES 15 219 981 PN 75114 RN 00280 AN 75081159 AU Stephan-U. Brauning-C. Busch-E-W. TI Letter: Screening for cystic fibrosis. SO Lancet. 1975 Jan 18. 1(7899). P 167. MJ CYSTIC-FIBROSIS: di. MASS-SCREENING. MN ALBUMINS: an. GEL-DIFFUSION-TESTS. HUMAN. INFANT-NEWBORN. MECONIUM: an. SPECIMEN-HANDLING. EX We read with interest Dr Raine's paper but would like to stress that Hellsing and Kollberg used a single radial immunodiffusion method not radioimmunoassay. Prosser et al. demonstrated quite clearly that similar results were obtained in cystic-fibrosis (C.F.) screening with test-strips and with more elaborate methods of albumin detection in meconium. Using both radial immunodiffusion as well as test-strips, Kollberg's group noticed a decreased albumin content in C.F. meconium stored at room temperature and mailed for several days. Diminished albumin concentration might have been brought about by remaining proteolytic activity. Prosser et al. found no change in albumin content during refrigerator storage. Approximately 10% of C.F. cases have no raised albumin content in meconium and this problem has not yet been solved. RF 004 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 005 PROSSER R ARCH DIS CHILD 49 597 974 PN 75115 RN 00281 AN 75081158 AU Crawfurd-M-D. TI Letter: Frequency of cystic-fibrosis gene. SO Lancet. 1975 Jan 18. 1(7899). P 167. MJ CYSTIC-FIBROSIS: fg. GENES. MN CAUCASOID-RACE. COMPARATIVE-STUDY. HETEROZYGOTE. HOMOZYGOTE. HUMAN. EX Mr Stuart and Dr Burdon speculate on the possibility that the high frequency of the cystic-fibrosis gene among Caucasians may be due to the heterozygotes having some advantage in biological fitness over normal homozygotes. They suggest that this might be due to increased resistance to typhus, once endemic in Europe, on the basis of the well-known immunological cross-reactivity between the causative organism, Rickettsia prowazeki, and certain strains of the species Proteus vulgaris, which is agglutinated by sera from cystic-fibrosis patients and heterozygotes. However, there is no evidence that this agglutination is an immunological reaction. It appears microscopically to be associated with disorganisation and inhibition of the bacterial flagellae leading to clumping of the organisms. As such it is probably analogous to the effect on cilia of rabbit trachea and oyster or mussel gills. The cilia factor, although associated with IgG, is now known not to be IgG itself and may well be the C3a component of complement. These and other observations, such as those on salivary and sweat gland secretions and on sodium flux, suggest that all these phenomena are membrane rather than immunological effects. Although it is possible that a Proteus flagella antigen could also be the binding site for the cilia factor, this seems improbable. An alternative hypothesis is that heterozygotes for the cystic-fibrosis gene have increased resistance to tuberculosis. This would meet the requirement of a common disease recently endemic in Europe at least as well as typhus, and is suggested by indirect evidence from a family study demonstrating a significantly lower incidence of tuberculosis among the parents of cystic-fibrosis patients than among parents of controls. RF 006 COHEN FL J MED GENET 11 253 974 008 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 009 CONOVER JH LANCET 1 1194 973 010 CRAWFURD MDA HEREDITY 29 126 972 CT 1 SHIER WT MED HYPOTHESES 5 661 979 2 JAKEL HP BIOL ZENTRALBL 98 55 979 3 HOLLANDER DH MED HYPOTHESES 8 191 982 PN 75116 RN 00282 AN 75193605 TI Editorial: Pseudomonas vaccines. SO Lancet. 1975 Jul 26. 2(7926). P 168. MJ BACTERIAL-VACCINES. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: pc. MN ACUTE-DISEASE. BACTERIAL-VACCINES: tu. CYSTIC-FIBROSIS: co. HUMAN. IMMUNITY-ACTIVE. LEUKEMIA: co. PSEUDOMONAS-INFECTIONS: co. EX Few antibiotics have therapeutic value against Pseudomonas aeruginosa, and each of them has shortcomings. It is therefore important to try and prevent pseudomonas infection in patients with severe burns, immunodeficiency, cystic fibrosis, and other conditions or methods of treatment that make them particularly susceptible to invasion by these bacteria. RF 026 MILLICAN RC J INFECT DIS 107 389 960 027 JONES RJ BR J PLAST SURG 19 43 966 028 JONES CE SURG FORUM 21 238 970 029 FELLER I IN: WALLACE AB 470 966 030 ALEXANDER JW IN: URBASCHEK B 489 975 031 MARKLEY K J BACTERIOL 96 867 968 032 JONES RJ BR J EXP PATHOL 53 659 972 033 JONES RJ IMMUNOLOGY 23 889 972 034 ALEXANDER JW ARCH SURG 99 249 969 035 HANESSIAN S NATURE NEW BIOL 229 209 971 036 JONES RJ J HYG CAMB 70 343 972 038 YOUNG LS ANN INTERN MED 79 518 973 039 HAGHBIN M CANCER 32 761 973 040 PENNINGTON JE AM J MED 58 629 975 CT 1 MUNSTER AM AM J SURG 133 710 977 PN 75117 RN 00283 AN 75193529 AU Emery-A-E. Farrell-K. Keay-A-J. McCrae-W-M. TI Letter: Fatty-acid oxidation in cystic fibrosis. SO Lancet. 1975 Jul 12. 2(7924). P 80-1. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS: me. MN ADOLESCENCE. ADULT. CYSTIC-FIBROSIS: bl. FATTY-ACIDS: df. HUMAN. LEUKOCYTES: me. MIDDLE-AGE. OXIDATION-REDUCTION. EX The nature of the metabolic defect in cystic fibrosis (C.F.) is unknown. We therefore undertook to study fatty-acid oxidation (Beta-oxidation) in patients with C.F. There was no significant difference in the results obtained in controls and either affected individuals or heterozygous carriers. All the values in the affected individuals lay within the normal range. Unless for some special reason the defect is not expressed in leukocytes, these results indicate that the metabolic pathway involved in fatty-acid oxidation (Beta-oxidation) is intact in C.F. It therefore seems unlikely that the reported deficiency of certain essential fatty acids in C.F. is the result of a defect in fatty-acid metabolism. RF 001 ROSENLUND ML NATURE 251 719 974 002 KUO PT J CLIN INVEST 44 1924 965 003 HSIA DYY CLIN GENET 1 5 970 004 EMERY AEH J NEUROL SCI 14 463 971 005 KING B J NEUROL SCI 20 297 973 CT 1 HUBBARD VS LANCET 2 1302 977 PN 75118 RN 00284 AN 75173958 AU Scanlin-T-F-Jr. Norman-M-E. Rosenlund-M-L. TI Letter: C3 in cystic fibrosis. SO Lancet. 1975 Jun 21. 1(7921). P 1382. MJ COMPLEMENT-3: an. COMPLEMENT: an. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. EX We believe that serum-levels of individual complement components in children cannot be meaningfully interpreted unless they are compared with normal values obtained from age-matched controls. In a study of 163 healthy infants and children we found a statistically significant correlation of C3 levels with age, but not with sex or race, and we also found a wide range of values within any given age-group. In contrast to previous reports, we did not find raised C3 levels in the majority of our C.F. patients when the values were compared with projected group mean values for the appropriate age, and we did not find a significant elevation of the mean C3 level for the C.F. patients in any of the age-groups tested. RF 001 CONOVER JH LANCET 2 1501 973 002 HANN S LANCET 2 520 974 004 MANCINI G IMMUNOCHEMISTRY 2 235 965 CT 1 HOLZHAUER RJ AM J HUM GENET 28 602 976 2 ANON J PEDIATR 88 711 976 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 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 PN 75119 RN 00285 AN 75117984 AU Dodge-J-A. Burton-L. TI Letter: Heterozygote advantage in cystic fibrosis. SO Lancet. 1975 Mar 8. 1(7906). P 572-3. MJ CYSTIC-FIBROSIS. HETEROZYGOTE. PERSONALITY. MN ADAPTATION-PSYCHOLOGICAL. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. PERSONALITY-INVENTORY. EX In a recent study, we attempted to assess the personality characteristics of both mothers and fathers of children with cystic fibrosis. In all, more than 150 such parents were interviewed. During the course of the interview, the Taylor manifest anxiety scale, Fouldes' personality inventory, and the 16 PF test form A were used. It was apparent that these parents represented a wide range of personality types, and differed in no way from the general population. RF 001 GAIRDNER D LANCET 1 279 975 CT 1 SHIER WT MED HYPOTHESES 5 661 979 2 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 75120 RN 00286 AN 76074281 AU Watts-R. Taylor-S. Postuma-R. Smalley-C-A. TI Letter: Essential-fatty-acid metabolism in cystic fibrosis. SO Lancet. 1975 Nov 15. 2(7942). P 983. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: me. MN CHILD. CYSTIC-FIBROSIS: bl. FATTY-ACIDS-ESSENTIAL: bl, df. HUMAN. INFANT. INFANT-NEWBORN. EX 6 patients with proven cystic fibrosis and a wide variety of Schwachman clinical scores were studied and the controls were 10 healthy children undergoing minor elective surgical procedures. The results show that for children with cystic fibrosis there are not only decreased proportions of essential fatty acids but that 20:3 omega-9 is found in all cases. Our results suggest that there is no intrinsic defect of the desaturating system in cystic fibrosis and that the pattern of essential-fatty-acid deficiency is entirely similar to that found in dietary deficiency of essential fatty acids. A further significant finding is the occurance if 20:3 omega-9 in apparently healthy children, particularly the very young. This is probably diet-related and emphasises the young child's very large requirement for essential fatty acids for membrane synthesis. The need for adequate polyunsaturated fatty acids in childhood cannot be overstated, whether they be for normal membrane synthesis or lowering of serum-lipids, giving possible protection against cardiovascular disease. RF 001 SMITH S J LIPID RES 9 52 968 002 BENNETT MJ AM J CLIN NUTR 20 415 967 003 AAES-JORGENSEN E PHYSIOL REV 41 1 961 004 COLLINS FD NUTR METAB 13 150 971 005 PRESS M BR MED J 2 247 974 006 MCEVOY FA LANCET 2 236 975 007 LOVE WC BR MED J 3 18 974 008 PAULSRUD JR AM J CLIN NUTR 25 897 972 009 WOODRUFF CW AM J CLIN NUTR 14 83 964 010 GLUECK CJ METABOLISM 21 1181 972 011 MCBEAN LD PEDIATR RES 8 837 974 012 KINGSBURY KJ POSTGRAD MED J 50 425 974 CT 1 HUBBARD VS LANCET 2 1302 977 2 BOHLES H Z ERNAHRUNGSWISS 18 81 979 PN 75121 RN 00287 AN 76074205 AU Robinson-P-G. TI Letter: Essential fatty acids in cystic fibrosis. SO Lancet. 1975 Nov 8. 2(7941). P 919. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: me. MN CYSTIC-FIBROSIS: et. FATTY-ACIDS-ESSENTIAL: df. HUMAN. EX In three literature reports the linoleic acid (L.A.) levels for cystic fibrosis (C.F.) compared to controls are always more significantly lower than arachidonic acid (A.A.). Similarly the percentage lowering is also more for L.A. than A.A. There is, thus, no "overwhelming" evidence for a blocked or absent desaturase and the cause of the E.F.A. deficiency must be elsewhere. We have found up to 2.5% of the total fatty acids in both serum and red cells to be C20:3-omega-9. All of our C.F. patients have shown this acid at some stage and, for most, it is a permanent feature of their fatty acid profile. It has been reported that C20:3-omega-9 would probably act as an inhibitor of prostaglandin formation from A.A. and it is interesting to conjecture on whether this could account for some of the symptoms of C.F. C20:3-omega-9 would also be incorporated into P.I. and could then alter membrane properties. RF 001 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 002 KUO PT J CLIN INVEST 44 1924 965 003 ROSENLUND ML NATURE 251 719 974 004 BENNETT MJ AM J CLIN NUTR 20 415 967 006 ANON NUTR REV 32 19 974 007 ELLIOTT RB ARCH DIS CHILD 50 76 975 CT 1 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 PN 75122 RN 00288 AN 76074167 AU Press-M. TI Letter: Essential-fatty-acid metabolism in cystic fibrosis. SO Lancet. 1975 Nov 1. 2(7940). P 871-2. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: me. MN FATTY-ACIDS-ESSENTIAL: df. HUMAN. EX In an investigation of the effects of intestinal malabsorption on plasma-lipids, we compared the fatty-acid composition of the serum-lecithin of 23 patients with malabsorption from a variety of different causes and found similar changes irrespective of the underlying cause of the malabsorption. The only exception to this was that classical E.F.A. deficiency was found only in patients whose malabsorption was due to massive small-bowel resection. All other causes of malabsorption gave rise to changes identical to those found by other workers in C.F.; there is thus no reason to postulate an additional metabolic abnormality specific to C.F. The relatively normal amounts of arachidonic acid reported in some E.F.A. deficient patients may not be entirely due to arachidonic acid. RF 001 RIVERS JPW LANCET 2 642 975 002 CALDWELL MD J PEDIATR 81 894 972 003 PRESS M LANCET 1 597 974 004 SHIMOYAMA T GUT 14 716 973 005 PRESS M BR MED J 2 247 974 006 CLARK SB J LIPID RES 14 581 973 007 ACKMAN RG IN: HOLMAN RT 12 165 972 CT 1 HUBBARD VS LANCET 2 1302 977 PN 75123 RN 00289 AN 76074202 AU Barnes-G-L. Ekert-H. Dowling-S-V. TI Letter: Cystic fibrosis: detection of heterozygotes. SO Lancet. 1975 Nov 8. 2(7941). P 918. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. MN ADENOSINE-DIPHOSPHATE: pd. ADULT. CHILD. CYSTIC-FIBROSIS: bl. HUMAN. PLATELET-AGGREGATION: de. PROSTAGLANDINS-E: pd. EX Samuels and Elliott have described a new technique for detecting cystic fibrosis (C.F.) and the heterozygote for C.F. We have tested this technique in detail on a smaller number of patients. Although the number of samples tested in our study is small, results suggest that this technique is not likely to be successful in detection of heterozygotes for C.F. RF 001 SAMUELS CE LANCET 2 607 975 002 EKERT H J THORAC CARDIOVASC SURG 67 184 974 CT 1 HAMDI I MONOGR PAEDIATR 10 84 979 2 BOROVIKOVA TM TER ARKH 52 125 980 PN 75124 RN 00290 AN 76050225 AU Thompson-G. TI Letter: Defective essential-fatty-acid metabolism in cystic fibrosis. SO Lancet. 1975 Oct 18. 2(7938). P 769. MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: me. MN HUMAN. PHOSPHOLIPIDS: me. EX Mr Rivers and Mr Hassam propose that the subnormal levels of linoleic acid (18:2) found in the plasma-phospholipids of patients with cystic fibrosis are due to malabsorption. To substantiate their hypothesis they deduce that the remarkable remission observed in a cystic-fibrosis patient given intravenous 'Intralipid' was due not to the 18:2 content of the 20% triglyceride component of the fat emulsion but to the 20:4 content of its 1.2% phospholipid component (not 6% as they state). This explanation seems unlikely in view of the relatively small proportion of 20:4 in intralipid phospholipid, especially since intravenous administration of intralipid phospholipid to healthy subjects decreases both the 18:2 and 20:4 content of their plasma-phospholipids. Comparison of the effects of complete intralipid versus intralipid phospholipid on the level of 20:4 in the plasma-phospholipids of patients with cystic fibrosis would soon determine whether they lack the ability to desaturate 18:2 to 20:4. RF 001 RIVERS JPW LANCET 2 642 975 PN 75125 RN 00291 AN 78198472 AU Robinson-P-G. Elliott-R-B. TI Pancreatic supplementation for premature babies [letter]. SO Lancet. 1975 Oct 25. 2(7939). P 817-8. MJ INFANT-PREMATURE-DISEASES: th. PANCREATIC-EXTRACTS: tu. MN CYSTIC-FIBROSIS: dh, dt. FECES. HUMAN. INFANT-NEWBORN. INFANT-PREMATURE-DISEASES: dh, dt. TRYPSIN: an. EX For some time now we have been using a screening test for cystic fibrosis based on levels of stool trypsin and chymotrypsin on the fourth day of life. One interesting observation is the number of premature babies who have little or no pancreatic trypsin or chymotrypsin at this time. For example, of the babies tested from St. Helen's Maternity Hospital between June, 1974, and April, 1975, 84 were classified as premature in the birth-records book. Samples were received for only 65 of these babies. Of these 65 babies, 26 (40%) had stool-trypsin levels of <100 microg. per g. compared with only 2.5% of the other babies tested during this period. As may be expected, the lower-birth-weight babies tend to have lower stool-enzyme concentrations. We feel that our data indicate that at least some premature babies would benefit from pancreatic-enzyme supplementation or predigested foods in the first few days after birth. RF 001 ROBINSON PG NZ MED J 79 1024 974 002 HAVERBACK BJ GASTROENTEROLOGY 44 588 963 CT 1 MERRITT AD ADV HUM GENET 8 135 977 PN 75126 RN 00292 AN 76029171 AU Rivers-J-P. Hassam-A-G. TI Defective essential-fatty-acid metabolism in cystic fibrosis. SO Lancet. 1975 Oct 4. 2(7936). P 642-3. (REVIEW). MJ CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: me. MN ANIMAL. DIETARY-FATS: me. FATTY-ACID-DESATURASES: me. FATTY-ACIDS-ESSENTIAL: df. HUMAN. PARENTERAL-FEEDING. PHOSPHOLIPIDS: me. REVIEW. AB Cystic fibrosis (C.F.) is characterised by low serum levels of essential fatty acids (E.F.A.). However, the fatty-acid pattern does not totally resemble that of dietary E.F.A. deficiency. The differences suggest a reduction in the desaturation of E.F.S. It is not known whether this defect is the primary lesion in C.F. or is the result of tissue damage in the disease. It is proposed that C.F. patients might have increased linoleic-acid requirements, and possibly specific requirements for its desaturation products. RF 001 ROSENLUND ML NATURE 251 719 974 002 DODGE JA BR MED J 2 192 975 003 BENNETT MJ AM J CLIN NUTR 20 415 967 004 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 005 KUO PT J PEDIATR 60 394 962 006 KUO PT J CLIN INVEST 44 1924 965 007 COLLINS FD NUTR METAB 13 150 971 008 ELLIOTT RB ARCH DIS CHILD 50 76 975 009 HOLMAN RT PROG CHEM FATS OTHER LIPIDS 9 607 970 010 WENE JD J CLIN INVEST 56 127 975 013 BRENNER RR MOL CELL BIOCHEM 3 41 974 014 BRENNER RR LIPIDS 6 567 971 015 BRENNER RR AM J PHYSIOL 215 63 968 016 NINNO RE BIOCHIM BIOPHYS ACTA 360 124 974 017 HUBBLE DV CYSTIC FIBROSIS 55 964 CT 1 HUBBARD VS EUR J PEDIATR 141 68 983 2 FOGERTY AC AM J CLIN NUTR 39 201 984 3 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 4 FARRELL PM PEDIATR RES 19 104 985 5 MISCHLER EH PEDIATR RES 20 36 986 6 FRIEDMAN Z PROG LIPID RES 25 355 986 7 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 8 CARLSTEDTDUKE J PROC NAT ACAD SCI USA 83 9202 986 PN 75127 RN 00293 AN 76009694 AU Samuels-C-E. Elliott-R-B. TI Letter: Cystic fibrosis: detection of heterozygotes. SO Lancet. 1975 Sep 27. 2(7935). P 607-8. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. MN HUMAN. EX We have developed a new technique for detecting cystic fibrosis (C.F.) and the heterozygote of C.F. The assay can be done by any laboratory having an aggregometer for measuring platelet aggregation. Quantitation of the results is obtained by comparing the transmittance recorded when only A.D.P. is added to platelet-rich plasma (P.R.P.). In C.F. patients and known heterozygotes, this ratio is 4 - 9 times higher than in healthy subjects. We have tested P.R.P. from 40 healthy people, 25 patients with C.F., and 40 known heterozygotes. We could detect all C.F. patients and 95% of the known heterozygotes. CT 1 BURDICK AB HUM HERED 27 366 977 2 HAMDI I MONOGR PAEDIATR 10 84 979 3 MANCUSO G RIV ITAL PEDIATR 8 665 982 4 DAVIS PB AM J MED SCI 288 104 984 PN 75128 RN 00294 AN 76218737 AU Johns-M-K. TI Skin wrinkling in cystic fibrosis. SO Med-Biol-Illus. 1975 Nov. 25(4). P 205-10. MJ CYSTIC-FIBROSIS: di. SKIN-MANIFESTATIONS. MN CHILD-PRESCHOOL. HUMAN. INFANT-NEWBORN. PHOTOGRAPHY. EX It had been noticed that in cases of cystic fibrosis the palmar skin of the fingers and the plantar skin of the toes wrinkled very quickly when immersed in water. Almost without exception, the children with cystic fibrosis had noticeable wrinkling of the fingers within 2 min of immersion in hand-hot water, whereas normal children showed no (or minimal) wrinkling after 10 min. This wrinkling is due to an excess of salt in the skin, which increases water-binding capacity of keratin. The test would thus appear to provide a simple and cheap screening method for cystic fibrosis, but one that may not be absolutely reliable. RF 001 ELLIOTT RB LANCET 2 108 974 002 NORMAN AP LANCET 2 358 974 003 LUNNON RJ MEDICAL AND BIOLOGICAL ILLUST 9 150 959 004 MOYNAHAN EJ LANCET 2 399 974 PN 75129 RN 00295 AN 76009233 AU Vagenakis-A-G. Braverman-L-E. TI Adverse effects of iodides on thyroid function. SO Med-Clin-North-Am. 1975 Sep. 59(5). P 1075-88. (REVIEW). MJ IODIDES: ae. THYROID-DISEASES: ci. THYROID-GLAND: de. MN CYSTIC-FIBROSIS: co. DRUG-SYNERGISM. GOITER: ci. GOITER-EXOPHTHALMIC: co. HUMAN. HYPERTHYROIDISM: ci, dt. HYPOTHYROIDISM: ci, co. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: ci. IODINE: me, pd, tu. LACTATION. MATERNAL-FETAL-EXCHANGE. PREGNANCY. REVIEW. THYROID-DISEASES: di. THYROID-GLAND: me. THYROID-HORMONES: me. THYROIDITIS-LYMPHOMATOUS: co. SUPPORT-U-S-GOVT-P-H-S. AB The administration of pharmacologic quantities of iodine such as iodides for the treatment of pulmonary disease, organic iodine present in medications and x-ray contrast dyes, and the ingestion of iodine-rich natural foods, may result in goiter, hypothyroidism, or hyperthyroidism, especially in patients with underlying thyroid disease. Medications containing iodide may induce hypothroidism in euthyroid patients with Hashimoto's thyroiditis, 131I or surgically treated Graves' disease, or following hemithyroidectomy for nodules; and they may induce hyperthyroidism in patients with endemic iodine- deficient goiter, autonomous nodules or nontoxic nodular goiter, or in patients recently treated with antithyroid drugs for Graves' disease. Rarely, hypothyroidism or hyperthyroidism may develop in patients with completely normal thyroid function during administration of iodide. The etiology of iodide-induced goiter and hypothyroidism in patients with cystic fibrosis remains obscure. Iodide-induced myxedema may also occur in patients receiving drugs which alter thyroid function, such as lithium, phenazone, and sulfisoxazole. Finally, iodides do have a role in the treatment of hyperthyroidism but their use should probably be restricted to thyroid storm, preoperative preparation of the hyperthyroid patient, and following 131I treatment. RF 001 AHMED M J CLIN ENDOCRINOL METAB 38 574 974 002 ALEXANDER WD Q J MED 31 281 962 002 ALEXANDER WD LANCET 2 866 965 003 ANDERSON FB ACTA ENDOCRINOL 73 35 973 005 BEGG TB Q J MED 32 351 963 006 BELL CO TRANS AM GOITER ASSOC 28 952 007 BIERWALTER WH BULL ALL INDIA INST MED SCI 3 145 969 008 BOYLE JA J CLIN ENDOCRINOL METAB 25 1255 965 009 BLUM M N ENGL J MED 291 24 974 010 BRAVERMAN LE J CLIN INVEST 42 1216 963 011 BRAVERMAN LE J CLIN ENDOCRINOL METAB 32 515 971 012 BRAVERMAN LE TRANS ASSOC AM PHYSICIANS 84 130 971 013 BRAVERMAN LE N ENGL J MED 281 816 969 014 DEODHAR SD ENDOCRINOLOGY 85 629 969 014 CONNOLLY RJ LANCET 1 500 970 015 EMERSON CH J CLIN ENDOCRINOL METAB 40 33 975 016 ERMANS AM ACTA ENDOCRINOL 70 463 972 017 FEINBERG WD J CLIN ENDOCRINOL METAB 19 567 959 018 FISHER DA J CLIN ENDOCRINOL METAB 33 667 971 019 GALTON VA ENDOCRINOLOGY 64 835 959 019 HAGEN GA N ENGL J MED 277 559 967 020 HALL R CLIN EXP IMMUNOL 1 205 966 021 HAMILTON CR JR MEDICINE (BALTIMORE) 52 195 973 022 HURXTHAL LE LAHEY CLIN BULL 4 73 945 023 INGBAR SH ENDOCRINOLOGY 53 171 953 024 INGBAR SH IN: WILLIAMS RH 119 974 025 JACKSON AS BOSTON MED SURG J 193 1138 925 026 KOUTRAS DA J CLIN ENDOCRINOL METAB 24 867 964 027 MILNE K ENDOCRINOLOGY 71 580 962 028 MORTON ME J BIOL CHEM 154 381 944 029 NAGATAKI S ENDOCRINOLOGY 74 731 964 030 PASTERNAK DP ENDOCRINOLOGY 84 769 969 031 RABEN MS ENDOCRINOLOGY 42 107 948 033 SHOPSIN B AM J MED 55 695 973 034 STANLEY MD J CLIN ENDOCRINOL METAB 9 941 949 035 SUZUKI H ACTA ENDOCRINOL 50 161 965 036 SUZUKI H J CLIN ENDOCRINOL METAB 34 332 972 037 TIMMERMANS J CONGENITALE JODIDEKROP MOCAND 31 308 963 038 VAGENAKIS AG J CLIN INVEST 52 528 973 039 VAGENAKIS AG ENDOCRINOLOGY 94 1669 974 040 VAGENAKIS AG J CLIN INVEST 54 913 974 041 VAGENAKIS AG N ENGL J MED 287 523 972 042 VIDOR GI J CLIN ENDOCRINOL METAB 37 901 973 043 WARTOFSKY L J CLIN ENDOCRINOL METAB 33 488 971 044 WARTOFSKY L J CLIN INVEST 49 78 970 044 WARTOFSKY L JAMA 226 1083 973 045 WOLFF J AM J MED 47 101 969 046 WOLFF J J BIOL CHEM 174 555 948 CT 1 RODESCH F AM J OBSTET GYNECOL 126 723 976 2 COOPER DS CLIN ENDOCRINOL METAB 7 199 978 3 EMRICH D CLIN ENDOCRINOL 8 257 978 4 TORTOROLO G MINERVA PEDIATR 30 659 978 5 COBB WE AM J HOSP PHARM 35 51 978 6 RODESCH F ANN ENDOCRINOL PARIS 39 145 978 7 PEKONEN F ANN CHIR GYNAECOL 67 165 978 8 REINWEIN D LANGENBECKS ARCH CHIR 347 145 978 9 BRAVERMAN LE CLIN ENDOCRINOL METAB 8 621 979 10 BEKAERT J COEUR MED INTERNE 18 241 979 11 JACKSON R CANCER TREAT REP 63 1393 979 12 BREUEL HP MED KLIN 74 1492 979 13 THEODOROPOULOS T SCIENCE 205 502 979 14 BAJORUNAS DR CLIN ENDOCRINOL METAB 9 405 980 15 RABER JH DRUG INTEL CLIN PHARM 14 344 980 16 REINER RG DIG DIS SCI 25 379 980 17 BECROFT DMO ARCH DIS CHILD 55 213 980 18 DOSREMEDIOS LV ARCH INTERN MED 140 1045 980 19 STERNTHAL E N ENGL J MED 303 1083 980 20 VOLF V ATOMKERNENERG KERNTECH 37 50 981 21 OBER KP ARCH INTERN MED 141 1225 981 22 KLEINMANN RE CATHET CARDIOVASC DIAGN 8 261 982 23 EMANUELE R RIC CLIN LAB 12 589 982 24 WEMEAU JL THERAPIE 37 95 982 25 SAFRAN M OBSTET GYNECOL 60 35 982 26 COYLE PJ ANN R COLL SURG ENGL 64 334 982 27 PONT A WEST J MED 136 255 982 28 FRADKIN JE MEDICINE 62 1 983 29 CHEN BX CHIN MED J 96 235 983 30 DAHLBERG PA ACTA ENDOCRINOL 104 195 983 31 MCGOVERN B CLIN CARDIOL 7 131 984 32 ROBUSCHI G ACTA DIABETOL LAT 21 357 984 33 FABRE JL ANN CARDIOL ANGEIOL (PARIS) 33 175 984 34 MAZONSON PD AM J MED 77 751 984 35 RAJATANAVIN R AM J MED 77 378 984 36 BECKER DV JAMA 252 659 984 37 WILKIN JK CUTIS 36 335 985 38 JANSSON R J CLIN ENDOCRINOL METAB 60 168 985 39 MARIGOLD JH BR J SURG 72 45 985 40 HAWTHORNE GC ARCH INTERN MED 145 1016 985 41 DRALLE H LANGENBECKS ARCH CHIR 365 79 985 42 ABALOVICH MS MED (BUENOS AIRES) 46 9 986 43 MARTINO E J CLIN ENDOCRINOL METAB 63 1233 986 44 TAJIRI J J CLIN ENDOCRINOL METAB 63 412 986 45 SAFRAN M AM J MED SCI 292 136 986 46 ROBUSCHI GV J ENDOCRINOLOGICAL INVEST 10 183 987 47 MECHLIS S AM J CARDIOL 59 833 987 PN 75130 RN 00296 AN 75216046 TI Vitamin E. SO Med-Lett-Drugs-Ther. 1975 Aug 15. 17(17). P 69-70. MJ HEART-DISEASES: dt. VITAMIN-E-DEFICIENCY. VITAMIN-E: tu. MN ADULT. ANEMIA-HEMOLYTIC: et. CYSTIC-FIBROSIS: co. HUMAN. INFANT. LIVER-CIRRHOSIS-BILIARY: co. PORPHYRIA: dt. VITAMIN-E-DEFICIENCY: co. VITAMIN-E: ad, ae. EX Vitamin E has been claimed to prevent, ameliorate, or cure skin disease, warts, ulcers, strabismus, baldness, frostbite, arthritis, jaundice, muscular dystrophy, diabetes, liver dysfunction, thyroid disease, sexual impotence, and aging. The vitamin has recently been added to cosmetics, used as a deodorant, and alleged to be an effective antidote to air pollution. There is no evidence that large doses of vitamin E are effective for the prevention or treatment of heart disease or any other human disorder, except in premature infants and possibly in patients with diseases that impair fat absorption. Adverse effects, though rare, have resulted from ingestion of large doses of vitamin E. RF 001 HORWITT MK AM J CLIN NUTR 27 1182 974 002 BIERI JG NUTR REV 33 161 975 003 OSKI FA J PEDIATR 70 211 967 004 WILLIAMS ML N ENGL J MED 292 887 975 005 HORWITT MK AM J CLIN NUTR 12 99 963 006 VOGELSANG A NATURE 157 772 946 007 SHUTE WE VITAMIN E FOR AILING AND HEAL 970 008 BAILEY H VITAMIN E YOUR KEY TO A HEALT 966 009 LEVY H ANN INTERN MED 28 1117 948 010 RAVIN IS N ENGL J MED 240 331 949 011 MAKINSON DH LANCET 1 102 948 012 RINZLER SH CIRCULATION 1 288 950 013 ANDERSON TW CAN MED ASSOC J 110 401 974 014 HAEGER K AM J CLIN NUTR 27 1179 974 015 ANON BR MED J 4 251 971 016 SHARMAN IM BR J NUTR 26 265 971 017 LAWRENCE JD AM J CLIN NUTR 28 205 975 018 NAIR PP ARCH INTERN MED 128 411 971 019 WATSON CJ ARCH INTERN MED 131 698 973 020 ANDERSON TW CAN MED ASSOC J 110 401 974 021 CORRIGAN JJ JR JAMA 230 1300 974 CT 1 STEWART JA SOUTH MED J 73 1297 980 PN 75131 RN 00297 AN 75156136 AU Woodruff-C. Barbero-G-J. TI The challenges of cystic fibrosis. SO Mo-Med. 1975 Mar. 72(3). P 129-33. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, th. FEMALE. HOSPITALS. HUMAN. INFANT. INFANT-NEWBORN. MALE. MISSOURI. EX Cystic fibrosis (CF) is a generalized disorder of the exocrine glands inherited as a recessive genetic trait. The disease was more fully described as a specific entity in 1939 with a pathology of dilated ducts and fibrotic changes in the pancreas and copious secretory intraluminal obstruction of the bronchial tree. The symptoms predominately involve the gastrointestinal tract as more frequent, bulky, malformed and malodorous stools and the respiratory system as cough, wheezing and signs of chronic respiratory obstruction. In certain instances, only one of these two systems will have manifest symptoms and signs. An increased concentration of sodium and chloride in the sweat was discovered in 1953. The elevated salt concentration of the sweat has proven to be a reliable diagnostic tool to confirm the presence of the disease. Few diseases present in so many different forms, degrees of severity and at different ages as does CF. All present treatments for cystic fibrosis are essentially symptomatic. Since the study of families having a child with cystic fibrosis has shown an incidence of the disease in one fourth of their siblings, classification of the disease as an autosomal recessive genetic disorder suggests that the concept of an abnormal gene related to alteration in a single enzyme should apply. The diagnosis of cystic fibrosis in childhood is an overwhelming even to the patient and his family. RF 001 BLACKFAN KD J PEDIATR 13 627 938 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 DI SANTAGNESE PA AM J MED 15 777 953 004 GIBSON LE PEDIATRICS 23 545 959 005 SEKELJ P AM REV RESPIR DIS 108 603 973 006 BARBERO GJ AM J DIS CHILD 112 536 966 007 MANGOS JA PEDIATR RES 2 378 968 008 SPOCK A PEDIATR RES 1 173 967 009 BOWMAN BH SCIENCE 164 325 969 010 MANGOS JA FUNDAMENTAL PROB CF AND RELAT 973 011 DANES BS J EXP MED 136 1313 972 012 MARTINEZ JR CF CLUB ABST 974 PN 75132 RN 00298 AN 75176450 AU Kuzemko-J-A. TI Recurrent diarrhoea in children. SO Nurs-Mirror. 1975 Jul 10. 141(2). P 55-8. MJ DIARRHEA: et. DIARRHEA-INFANTILE: et. INTESTINAL-DISEASES: co. MN CELIAC-DISEASE: co. CHILD. CHILD-PRESCHOOL. COLITIS-ULCERATIVE: co. CYSTIC-FIBROSIS: co. ENTERITIS: co. FOOD-HYPERSENSITIVITY: co. GIARDIASIS: co. HUMAN. INFANT. INTESTINAL-DISEASES-PARASITIC. RECURRENCE. EX Diarrhoea can be defined as the passage of loose stools and increased bowel frequency above the accepted norm for the individual and resulting in excess loss of water and electrolytes in the faeces. It may or may not be associated with steatorrhea (fatty stools). History plays a vital part in the diagnosis of gastro-intestinal disease in children. Time spent here is often more rewarding than a battery of specialised investigations. One must be clear what the parent means by diarrhoea, as terminology among doctors, nurses, and parents is often confusing. When dealing with children one must always adopt a comprehensive approach and consider the whole child and his family. Common causes, mechanisms, and consequences are discussed. Some of the common causes of diarrhoea are cystic fibrosis, gluten-induced enteropathy (coeliac disease), Giardia lamblia, sugar intolerance, gastro-intestinal allergy, and irritable bowel syndrome. PN 75133 RN 00299 AN 75176353 AU Noakes-S. TI Nursing care study: Sally--a child with cystic fibrosis. SO Nurs-Times. 1975 Jun 5. 71(23). P 876-7. MJ CYSTIC-FIBROSIS: nu. PEDIATRIC-NURSING. MN CHILD-PRESCHOOL. FEMALE. HUMAN. EX Fibrocystic disease is a common disease of childhood and the pancreas is abnormal in 90% of these cases. There is fibrosis with atrophy of the exocrine parenchymal tissue, but islet cell tissue is rarely involved. Mucous glands throughout the body are grossly distended and they secrete an abnormal viscid mucus. Sweat glands appear normal, but they secrete sweat with an abnormally high concentration of electrolytes. A characteristic feature of the disease is the widespread chronic bronchiolitis. This is due to the Staphylococcus aureus which grow best in a salt-rich medium. PN 75134 RN 00300 AN 76031006 AU Craig-J-M. TI The pathology of infertility. SO Pathol-Annu. 1975. 10. P 299-328. (REVIEW). MJ INFERTILITY-FEMALE: pa. INFERTILITY-MALE: pa. MN ADNEXITIS: co. CHROMOSOME-ABNORMALITIES: pa. CRYPTORCHISM: pa. CYSTIC-FIBROSIS: co. DIABETES-MELLITUS: co. ENDOCRINE-DISEASES: co. ENDOMETRIOSIS: co. FEMALE. GENITAL-DISEASES-FEMALE: co. HUMAN. IATROGENIC-DISEASE. INFANT. INFANT-NEWBORN. KLINEFELTERS-SYNDROME: pa. MALE. METABOLISM-INBORN-ERRORS: co. MYOCLONUS: co. ORCHITIS: pa, et. PREGNANCY. REVIEW. SALPINGITIS: pa. SCLEROSIS. SEMINIFEROUS-TUBULES: pa. SPERM-MATURATION. INFERTILITY-FEMALE: et. INFERTILITY-MALE: et, fg. UTERINE-DISEASES: co. VARICOCELE: co, pa. EX In most series of infertility patients, approximately 40 to 60 percent have no discernible cause for infertility. No wonder that those working with this condition tend to seize upon any demonstrable tissue abnormality as related to the infertility. The use of fallible diagnostic tools may lead to misdiagnosis and inappropriate treatment. In studies of the accuracy of dye injections to demonstrate tubal patency by roentgenogram the large errors introduced by these methods were emphasized. Between 30 to 40 percent of tubes found blocked by using a single dye examination and using the criterion of "filling but not spilling" into the peritoneal cavity, or with apparent obstruction at the uterotubal junction, were subsequently found to be patent. Similar data were offered by others. Lesions with less obvious and demonstrable causal relationship to infertility, such as pelvic adhesions and endometriosis, are more difficult to judge as to their etiologic relation. These are assessed in discussions of male and female infertility. RF 001 ACOSTA AA OBSTET GYNECOL 42 19 973 002 ARRONET CH FERTIL STERIL 20 903 969 003 BAEYERTZ JD AUST NZ J OBSTET GYNAECOL 7 284 967 004 BOYD IA J OBSTET GYNAECOL BR COMMONW 80 142 973 005 BROGGEN A ACTA ENDOCRINOL 48 490 965 006 CARSWELL F POSTGRAD MED J 42 277 966 007 CHANDLEY AC CYTOGENETICS 10 295 971 008 CHANG MD FERTIL STERIL 15 97 964 009 CHAMY CW JAMA 204 1165 968 010 CLARKE BG JAMA 198 1121 966 011 COOK ID J OBSTET GYNAECOL BR COMMONW 79 697 972 012 COOPER HE AM J OBSTET GYNECOL 100 371 968 013 CRAIG JM AM J OBSTET GYNECOL 97 100 967 014 DEKRETSER DM J ENDOCRINOL 40 107 968 015 DEKRETSER DM J CLIN ENDOCRINOL METAB 35 392 972 016 DE LA BALZE FA J CLIN ENDOCRINOL METAB 22 1251 962 017 DE LA BALZE FA J CLIN ENDOCRINOL METAB 12 1426 952 018 DENNING CR PEDIATRICS 41 7 968 019 DI POALA GR INT J FERTIL 16 189 971 020 DORING GK GEBURTSHILFE FRAUENHEILKD 30 302 970 021 DUBIN L FERTIL STERIL 20 50 969 022 ETRIBY A FERTIL STERIL 18 666 967 023 FAIRLY KF LANCET 1 568 972 024 GALL EA AM J PATHOL 23 637 947 025 GRANT A INT J FERTIL STERIL 9 503 964 026 GRANT A AUST NZ J OBSTET GYNAECOL 9 224 969 027 HEDBERG E ACTA OBSTET GYNECOL SCAND 37 131 958 028 HECKER WC DTSCH MED WSCHR 97 1325 972 029 HORTLING H J CLIN ENDOCRINOL METAB 27 120 967 030 HOWARD JE J CLIN ENDOCRINOL METAB 10 121 950 031 JENSEN PA AM J OBSTET GYNECOL 113 150 972 032 KAPLAN E N ENGL J MED 279 65 968 033 KOZAK GP IN: MARBLE A 971 034 LANDING BH ARCH PATHOL 88 569 969 035 LEE PA AM J DIS CHILD 127 530 974 036 LILIEQUIST B ACTA OBSTET GYNECOL SCAND 43 240 964 037 MACKEY RA FERTIL STERIL 22 504 971 038 MACLEOD J FERTIL STERIL 20 545 969 039$ MACLEOD J OBSTET GYNECOL 26 335 971 040 MORAES-RUEHSEN M FERTIL STERIL 18 440 968 041 NEU RL FERTIL STERIL 24 811 973 042 NOYES RW IN: ENDERS AC 197 963 043 PAREKH M CLIN OBSTET GYNECOL 15 1 972 044 PARR EL J EXP MED 126 523 967 045 PETERSOHN L ACTA OBSTET GYNECOL SCAND 49 331 970 046 PHADKE AN FERTIL STERIL 24 802 973 047 PLONUM L ACTA OBSTET GYNECOL SCAND 39 143 960 048 PRINZ W GYNAECOLOGIA 167 23 968 049 QUAN A OBSTET GYNECOL 22 96 963 050 RANNEY B AM J OBSTET GYNECOL 107 743 970 051 RICHTER P CANCER 25 1026 970 052 RIMOIN DL AM J MED 44 225 968 053 ROGERS SF FERTIL STERIL 19 529 968 054 RUBIN IC SURG GYNECOL OBSTET 103 469 956 055 RUBIN IC TRANS NJ OBSTET SOC 2 77 957 056 RYAN G AM J OBSTET GYNECOL 90 715 964 057 SAEZ JM J CLIN ENDOCRINOL METAB 32 604 971 058 SARTO E CLIN OBSTET GYNECOL 15 183 972 059 SCHENKER JF SURG GYNECOL OBSTET 135 74 972 060 SIMPSON JL CLIN OBSTET GYNECOL 15 157 972 061 SKAKKEBAEK NE FERTIL STERIL 21 652 970 062 SKAKKEBAEK NE ACTA PATH MICROBIOL SCAND (A) 81 97 973 063 SKAKKEBAEK NE ACTA PATH MICROBIOL SCAND (A) 81 112 973 064 SOHVAL AR PHYSIOL REV 43 263 963 065 SOUTHAM AC FERTIL STERIL 8 25 957 066 VALMAN HB LANCET 2 566 969 067 VAN DEN BERGH H J CLIN ENDOCRINOL METAB 28 1370 968 068 VARE AM FERTIL STERIL 24 793 973 069 WARREN SL PATHOLOGY OF DIABETES 966 070 WILSON JD N ENGL J MED 290 1097 974 071 WONG TW ARCH PATHOL 95 151 973 072 WONG TW ARCH PATHOL 95 160 973 CT 1 HONORE LH FERTIL STERIL 29 164 978 2 NEWBOLD RR AM J PATHOL 117 333 984 3 NEWBOLD RR TERATOGEN CARCINOGEN MUTAGEN 5 473 985 4 PESCE CM ARCH ANDROL 15 193 985 5 WIEDEMANN R GEBURTSHILFE FRAUENHEILKD 47 96 987 PN 75135 RN 00301 AN 76076809 AU Shwachman-H. TI Gastrointestinal manifestations of cystic fibrosis. SO Pediatr-Clin-North-Am. 1975 Nov. 22(4). P 787-805. (REVIEW). MJ CYSTIC-FIBROSIS: co. INTESTINAL-DISEASES: co. MN ADOLESCENCE. ADULT. APPETITE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dh, di. DIABETES-MELLITUS: co. FEMALE. GROWTH-DISORDERS: co. HUMAN. HYPERTENSION-PORTAL: co. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co. INTUSSUSCEPTION: co. LACTOSE-INTOLERANCE: co. LIVER-CIRRHOSIS: co. MALE. MECONIUM. PANCREAS: pp. PANCREATITIS: co. RECTAL-PROLAPSE: co. REVIEW. EX From a historical view cystic fibrosis (CF) has been considered a disease of the pancreas which results in maldigestion and loss of fat, and nitrogen in the stool. A number of early synonyms included congenital steatorrhea, pancreatic infantilism, and pancreatic fibrosis. The concern of the pediatrician in the late 1930's and early 1940's was to distinguish nonfatal celiac disease from fatal cystic fibrosis. In 1954, Shwachman presented clinical and laboratory features that distinguish these two entities. Today one is not justified in making a diagnosis of celiac disease without a confirmatory small intestinal biopsy coupled with a normal sweat test. A number of gastrointestinal complications in cystic fibrosis are examined: meconium ileus, intestinal obstruction, pancreatic insufficiency, lactase deficiency, intestinal impaction, intussusception, rectal prolapse, growth retardation, excessive appetite, cirrhosis and portal hypertension, gallbladder problems, and diabetes. The article closes by recording the indications for the sweat test. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANTONOWICZ I PEDIATRICS 49 847 972 003 ANTONOWICZ I PEDIATRICS 42 492 968 004 BERK RN RADIOLOGY 106 377 973 006 COZZETTO FJ PEDIATRICS 32 228 963 007 DONNISON AB PEDIATRICS 37 833 966 008 GRAND RJ CLIN PEDIATR 9 588 970 009 GREEN MN PEDIATRICS 21 635 958 010 GREEN MN AM J DIS CHILD 100 365 960 011 GREEN MN PEDIATRICS 41 989 968 012 HADORN B J PEDIATR 73 39 968 013 HANDWERGER S N ENGL J MED 281 451 969 014 HOLSCLAW DS J PEDIATR SURG 9 867 974 015 HOLSCLAW DS PEDIATRICS 48 51 971 017 KOPEL FB GASTROENTEROLOGY 62 483 972 018 KOPITO L J PEDIATR 68 313 966 019 KULCZYCKI LL N ENGL J MED 259 409 958 020 LEBENTHAL E AM J CLIN NUTR 28 595 975 021 LEBENTHAL E GASTROENTEROLOGY 67 807 974 022 LLOYD-STILL JD PEDIATRICS 53 678 974 023 MADDOCK CL AM J DIS CHILD 66 370 943 024 NEUHAUSER EBD AM J ROENTG RAD THER 51 421 944 025 NOBLETT HR J PEDIATR SURG 4 190 969 026 OPPENHEIMER EH J PEDIATR 86 683 975 027 OPPENHEIMER EH PEDIATR RES 7 339 973 028 PARKINS RA LANCET 2 851 963 029 ROSAN RC AM J DIS CHILD 104 625 962 030 SHWACHMAN H PEDIATR CLIN NORTH AM 1 389 954 031 SHWACHMAN H IN: SLEISENGER MH 1206 973 032 SHWACHMAN H N ENGL J MED 286 1300 972 033 SHWACHMAN H PEDIATRICS 55 86 975 034 SHWACHMAN H PEDIATRICS 46 3 970 035 STEPHAN U PEDIATRE 10 63 974 036 TUCKER AS AM J ROENTG RAD THER NUCL MED 89 1048 963 037 WATKINS JB GASTROENTEROLOGY 68 1087 975 038 WEBER AM N ENGL J MED 289 1001 973 039 WILMSHURST EG PEDIATRICS 55 75 975 CT 1 ROY CC N ENGL J MED 297 1301 977 2 ANGERPOINTNER T Z KINDERCHIR GRENZGEB 24 99 978 3 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 4 LAMBRECHT W CHIRURG 49 410 978 5 ROSENSTEIN BJ AM J DIS CHILD 132 1043 978 6 BRADLEY JA BR MED J 1 167 979 7 BARRY MM J AM DIET ASSOC 75 446 979 8 CUNNINGHAM DG J COMPUT ASSIST TOMOGR 4 151 980 9 LEDESMAMEDINA J PEDIATR RADIOL 9 61 980 10 ARBORGH B SCAND J GASTROENTEROL 15 73 980 11 PSACHAROPOULOS HT LANCET 2 78 981 12 CONGDEN PJ ARCH DIS CHILD 56 708 981 13 MABOGUNJE OA SURGERY 90 114 981 14 CHURCHILL RJ JAMA 245 72 981 15 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 16 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 17 BASS S GASTROENTEROLOGY 84 1592 983 18 ROSENSTEIN BJ J PEDIATR 103 667 983 19 HODSON ME POSTGRAD MED J 60 225 984 20 GILLARD BK AM J DIS CHILD 138 577 984 21 PASCALI VL EUR J PEDIATR 143 133 984 22 TAL A CLIN PEDIATR 24 460 985 23 GRAHAM N CLIN RADIOL 36 199 985 24 NOUSIAARVANITAKIS S ARCH PATHOL LAB MED 109 722 985 25 VINOCUR CD AM J SURG 149 182 985 26 RUBINSTEIN S PEDIATRICS 78 473 986 27 DODGE JA J ROY SOC MED 79 27 986 28 ROSENSTEIN BJ AM J DIS CHILD 140 966 986 PN 75136 RN 00302 AN 76076877 AU Araki-H. Field-M. Shwachman-H. TI A new assay for cystic fibrosis factor: effects of sera from patients with cystic fibrosis in the in vitro electrical properties of rat jejunum. SO Pediatr-Res. 1975 Dec. 9(12). P 932-4. MJ CYSTIC-FIBROSIS: bl. JEJUNUM: de. MN ANIMAL. BIOLOGICAL-ASSAY. FRUCTOSE: pd. GLUCOSE: pd. HUMAN. IN-VITRO. JEJUNUM: ph. RATS. TEMPERATURE. AB The in vitro electrical properties of rat jejunum were utilized to assay a factor or factors in serum from patients with cystic fibrosis (CF). Sera from patients with CF were found to decrease short circuit current (SCC) and the SCC response to glucose, and to increase electrical resistance. These effects were present in all 24 patients with CF and 10 of 14 parents of CF patients and absent from the serum of 21 control subjects. This quantitative technique provides an alternative to the ciliostatic assays for the detection of the CF factor or factors. RF 001 ASANO T AM J PHYSIOL 207 415 964 002 BERATIS NG PEDIATR RES 7 958 973 003 BOWMAN BH SCIENCE 164 325 969 004 BOWMAN BH SCIENCE 167 871 970 005 BROWN GA LANCET 2 639 971 006 KAISER D PEDIATR RES 5 167 971 007 LOCKHART LH TEX REP BIOL MED 31 631 973 008 MANGOS JA SCIENCE 158 135 967 009 MORIN CL BIOMEDICINE EXPRESS 19 133 973 010 SCHULTZ SG J GEN PHYSIOL 47 567 964 011 SPOCK A PEDIATR RES 1 173 967 012 TAUSSIG LM LANCET 1 1367 972 013 USSING HH ACTA PHYSIOL SCAND 23 110 951 014 WOOD RE LANCET 2 1452 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 DISANTAGNESE PA N ENGL J MED 295 534 976 3 SHWACHMAN H MEDICINE 56 129 977 4 ARAKI H LANCET 1 793 978 5 IMPERO JE PEDIATR RES 12 108 978 6 GILMORE JP PROC SOC EXP BIOL MED 157 70 978 7 MORRISSEY SM MONOGR PAEDIATR 10 102 979 8 NAGY EC PEDIATR RES 13 729 979 9 TUCKER RD PEDIATR RES 13 1371 979 10 WILL PC PEDIATR RES 13 1129 979 11 TUCKER RD IEEE TRANS BIOMED ENG 26 607 979 12 SCANLIN TF CLIN CHEST MED 1 424 980 13 WILL PC PEDIATR RES 14 1245 980 14 BANCHINI G PEDIATR RES 15 1073 981 15 RUDIN DO BIOL PSYCHIAT 16 373 981 16 ALBAZZAZ FJ RESPIRATION 46 88 984 PN 75137 RN 00303 AN 76052122 AU Cole-C-H. Sella-G. TI Inhibition of ouabain-sensitive ATPase by the saliva of patients with cystic fibrosis of the pancreas. SO Pediatr-Res. 1975 Oct. 9(10). P 763-6. MJ ADENOSINE-TRIPHOSPHATASE: ai. CYSTIC-FIBROSIS: me. OUABAIN: pd. SALIVA: an. MN ADOLESCENCE. CELL-MEMBRANE: en. CHILD. COMPARATIVE-STUDY. ERYTHROCYTES: en. FEMALE. HUMAN. MAGNESIUM: pd. MALE. PHOSPHORUS: ip. POTASSIUM: pd. SODIUM: pd. AB A study has been made of the effect of saliva from children with cystic fibrosis of the pancreas (CFP) on various components of the ATP hydrolyzing enzyme system. The ouabain-sensitive ATPase activity of erythrocyte membranes prepared from intact erythrocytes preincubated with CFP saliva was 35 +/- 4 nmol Pi/mg fry wt membrane suspension/hr, compared with 48 +/- 7 nmol Pi/mg dry wt membrane suspension/hr when the erythrocytes were preincubated with control saliva. A calcium-activated component of ATPase was decreased from 202 +/- 30 nmol Pi/mg dry wt membrane suspension/hr in erythrocytes preincubated with control saliva, to 151 +/- 17 nmol Pi/mg dry wt membrane suspension/hr when the incubation was carried out with CFP saliva. In a second series of experiments, ultrafiltered saliva was added directly to an ATPase assay. The saliva from children with CFP brought about a mean decrease in ouabain-sensitive ATPase of 16% compared with control saliva. RF 001 BALFE JW SCIENCE 162 689 968 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BESLEY GTN IN: LAWSON D PROC 5TH INT CF 14 969 004 COLE CH PEDIATR RES 6 616 972 005 COLE CH CLIN SCI MOL MED 45 775 973 006 DUFFY MJ CLIN CHIM ACTA 50 97 974 007 FEIG SA PEDIATR RES 8 594 974 008 HANAHAN DJ BIOCHIM BIOPHYS ACTA 255 413 972 009 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 010 KAISER D PEDIATR RES 5 167 971 011 KATZ AI N ENGL J MED 278 253 968 012 KREUSSER W EUR J CLIN INVEST 2 398 972 013 LOWRY OH J BIOL CHEM 162 421 946 014 MANGOS JA PEDIATR RES 1 436 967 015 SCHATZMANN HJ J PHYSIOL (LOND) 201 369 969 016 SHWACHMAN H AM J DIS CHILD 96 6 958 017 SPOCK A PEDIATR RES 1 173 967 018 TAYLOR A PEDIATR RES 8 861 974 019 WIESMANN UN LANCET 2 510 972 CT 1 BOWMAN BH TEX REP BIOL MED 34 1 976 2 WARD JB TEX REP BIOL MED 34 11 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 JAKEL HP BIOL ZENTRALBL 98 55 979 5 BANCHINI G PEDIATR RES 15 1073 981 6 ALADJEM M NEPHRON 34 84 983 7 VONEULER AM LIFE SCI 37 2399 985 PN 75138 RN 00304 AN 75217264 AU Wilson-G-B. Fudenberg-H-H. TI Studies on cystic fibrosis using isoelectric focusing. I. An assay for detection of cystic fibrosis homozygotes and heterozygote carriers from serum. SO Pediatr-Res. 1975 Aug. 9(8). P 635-40. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. GELS. HUMAN. HYDROGEN-ION-CONCENTRATION. IGG: an. INFANT. ISOELECTRIC-FOCUSING. MALE. MIDDLE-AGE. AB We have developed a standardized biophysical assay for the rapid detection of individuals homozygous or heterozygous for cystic fibrosis (C/F). The assay employs isoelectric focusing in thin layer polyacrylamide gels to analyze microliter quantities of whole serum for the presence of a C/F factor protein and for deletions in a group of proteins called proteins B, C, and D (Fig, 1). A pH 5-10 gradient is used (Fig. 2) and each sample is screened using a serum volume which contains 300 g immunoglobulin G (IgG). Individuals homozygous or heterozygous for C/F are distinguished from normal unaffected individuals on the basis of the presence of a C/F factor protein band (Table 1). Heterozygous carriers for C/F are distinguished from C/F homozygotes 75 percent of the time, on the basis of a deletion in either band B, C, or D (Table 2). On the basis of screening 65 patients with cystic fibrosis, 61 heterozygous carriers for C/F, and 105 normal control subjects, it was concluded that no obvious correlation existed between either sex, age, or severity of the disease in the individual C/F patient, and the absolute presence or absence of the C/F factor. In addition, no correlation existed between sex or age and the presence of the C/F factor or deletions in proteins B, C, and D in the individual heterozygous carrier for C/F or normal control subjects. Analysis of serum samples from 68 patients with a variety of other diseases, many with clinical symptoms resembling those seen in the patient with cystic fibrosis (Table 3), indicated that the C/F factor protein described in this study appears to be diagnostic for C/F genotypes, with the possible exception of patients with certain types of leukemia. RF 001 BARNETT DR TEX REP BIOL MED 31 697 973 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BERATIS NG PEDIATR RES 7 958 973 004 BESLEY GTN J MED GENET 6 278 969 005 BOWMAN BH TEX REP BIOL MED 31 611 973 006 BOWMAN BH IN: MANGOS JA 29 973 007 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 008 BOWMAN BH SCIENCE 164 325 969 009 BOWMAN BH SCIENCE 167 871 970 010 CONNEALLY PM TEX REP BIOL MED 31 639 973 011 CONOVER JH PEDIATR RES 7 224 973 012 CONOVER JH CLIN RES 21 531 973 013 CONOVER JH PEDIATR RES 7 220 973 014 CONOVER JH LANCET 1 1194 973 015 CONOVER JH LANCET 2 1501 973 016 CONOVER JH LIFE SCI 14 253 974 017 DANES BS J EXP MED 137 1538 973 018 DANKS DM ANN HUM GENET 28 323 965 019 FAHEY JL J IMMUNOL 94 84 965 020 GIBSON LE PEDIATRICS 23 545 959 021 LOBECK CC IN: STANBURY JB 1605 972 022 MANCINI G IMMUNOCHEMISTRY 2 235 965 023 MANGOS JA PEDIATR RES 2 378 968 024 MCCOMBS ML TEX REP BIOL MED 31 615 973 025 MCCOMBS ML CLIN GENET 1 171 970 026 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 027 SHWACHMAN H IN: KENDIG EL JR 541 967 029 SPOCK A PEDIATR RES 1 173 967 030 WILLIAMSON AR IN: WEIR DM 973 032 WILSON GB DISSERT ABST INTERNAT 35 479 974 034 WILSON GB LIFE SCI 15 551 974 035 WILSON GB CLIN CHIM ACTA 49 79 973 CT 1 WILSON GB SCAND J IMMUNOL 5 828 976 2 WILSON GB PEDIATR RES 10 1001 976 3 WILSON GB PEDIATR RES 10 87 976 4 WILSON GB TEX REP BIOL MED 34 51 976 5 ANON J PEDIATR 88 711 976 6 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 7 WOOD RE AM REV RESPIR DIS 113 833 976 8 DISANTAGNESE PA N ENGL J MED 295 534 976 9 THOMAS JM PEDIATR RES 11 138 977 10 THOMAS JM PEDIATR RES 11 1148 977 11 WILSON GB PEDIATR RES 11 139 977 12 WILSON GB PEDIATR RES 11 317 977 13 WILSON GB PEDIATR RES 11 986 977 14 WILSON GB PEDIATR RES 11 143 977 15 ARNAUD P J IMMUNOL METH 16 221 977 16 BURDICK AB HUM HERED 27 366 977 17 WILSON GB NATURE 266 463 977 18 IMPERO JE PEDIATR RES 12 108 978 19 SCHOLEY J PEDIATR RES 12 800 978 20 WILSON GB PEDIATR RES 12 801 978 21 WILSON GB J LAB CLIN MED 92 463 978 22 ALLEN RC J CHROMATOGR 146 1 978 23 TULLY GW PEDIATR RES 13 1078 979 24 WILSON GB PEDIATR RES 13 1079 979 25 MANSON JC LANCET 1 330 980 26 WILSON GB LANCET 2 313 980 27 MAYO BJ CLIN GENET 18 379 980 28 WANG A ARCH DIS CHILD 55 130 980 29 HALLINAN F MED HYPOTHESES 7 793 981 30 NEVIN GB HUM GENET 56 387 981 31 HALLINAN FM CLIN CHIM ACTA 117 103 981 32 BOGART BI PEDIATR RES 16 223 982 33 MCNEELY MC PEDIATR RES 16 21 982 34 BULLOCK S CLIN GENET 21 336 982 35 BROCK DJH HUM GENET 60 30 982 36 HEELEY AF CLIN CHEM 29 2011 983 37 WILSON GB THYMUS 6 167 984 38 GRATAROLI R PEDIATR RES 18 130 984 39 WILSON GB CLIN GENET 26 331 984 40 GEDDES D THORAX 39 721 984 41 PASCALI VL EUR J PEDIATR 143 133 984 42 ANON LANCET 2 249 985 43 VANHEYNINGEN V CYTOGENET CELL GENET 40 767 985 44 SCAMBLER P HUM GENET 69 250 985 45 QURESHI AR J PEDIATR 106 913 985 46 VANHEYNINGEN V NATURE 315 513 985 47 HAYWARD C J IMMUNOL METH 91 117 986 PN 75139 RN 00305 AN 76076889 AU Rao-G-J. Nadler-H-L. TI Deficiency of arginine esterase in cystic fibrosis of the pancreas: demonstration of the proteolytic nature of the activity. SO Pediatr-Res. 1975 Sep. 9(9). P 739-41. MJ ARGININE: df. CYSTIC-FIBROSIS: me. ESTERASES: df. MN ADOLESCENCE. ARGININE: ai. CHILD. CHILD-PRESCHOOL. ENZYME-INHIBITORS: pd. ESTERASES: ai. HETEROZYGOTE. HUMAN. HYDROLYSIS. HYDROXYMERCURIBENZOATES: pd. LUNG-DISEASES: me. PANCREATIC-DISEASES: me. PEPTIDES: me. PROTAMINES. TOSYLLYSINE-CHLOROMETHYL-KETONE: pd. TRYPSIN-INHIBITORS: pd. AB Proteolytic activity, defined as the hydrolysis of peptide bonds involving the carboxyl group of L-arginine, in plasma of patients with cystic fibrosis, heterozygotes, and control subjects has been assayed using a fluorometric method with protamine as the substrate and fluorescamine as the reagent. The mean total proteolytic activity in plasma of patients with cystic fibrosis was approximately one-half the mean total activity in control subjects and heterozygotes. The mean proteolytic activity inhibited by soybean trypsin inhibitor in plasma of patients with cystic fibrosis was approximately one-third that of control subjects and heterozygotes. The relationship of arginine esterase activity to proteolytic activity was investigated. The pH optimum and action of reversible and irreversible inhibitors were similar for both activities, suggesting that the arginine esterase activity and proteolytic activity represent similar catalytic entities. These findings are consistent with our hypothesis that the basic defect in cystic fibrosis may reside in the deficiency of a proteolytic enzyme which results in the accumulation of the various cationic macromolecular "factors" described by other investigators in serum of patients with cystic fibrosis. The demonstration of a deficiency of proteolytic activity as assayed by the hydrolysis of protamine, a cationic polypeptide, could explain the presence of ciliotoxic cationic protein or polypeptide factors in serum of patients with cystic fibrosis and may, in some unknown manner, be related to the clinical manifestations of the disease. RF 001 BARNETT DR TEX REP BIOL MED 31 697 973 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BARRETT AJ BIOCHEM J 131 809 973 004 BOWMAN BH SCIENCE 167 871 970 005 BROWN F BIOCHEM BIOPHYS RES COMMUN 53 75 973 006 CHASE T JR BIOCHEMISTRY 8 2212 969 007 COLMAN RW J CLIN INVEST 48 23 969 008 CONOVER JH LIFE SCI 14 253 974 009 GLOVER G J BIOL CHEM 246 4594 971 010 GROSKOPF WR J BIOL CHEM 244 359 969 011 LIEBERMAN J PEDIATR RES 3 571 969 012 LOBECK CC IN: STANBURY JB 1605 972 013 RAO GJS J PEDIATR 80 573 972 014 RAO GJS PEDIATR RES 8 684 974 015 RAO GJS SCIENCE 177 610 972 016 SPOCK A PEDIATR RES 1 173 967 017 TALAMO RC PEDIATR RES 6 430 972 018 UDENFRIEND S SCIENCE 178 871 972 CT 1 SHAPIRA E PEDIATR RES 10 812 976 2 BOWMAN BH LIFE SCI 19 1289 976 3 BOWMAN BH TEX REP BIOL MED 34 1 976 4 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 5 CALLAHAN JW PEDIATR RES 11 1166 977 6 RAO GJS PEDIATR RES 11 981 977 7 BARRETT AJ ACTA BIOL MED GER 36 1959 977 8 COLMAN RW THROMB HAEMOST 38 751 977 9 GOLDSMITH GH J LAB CLIN MED 89 131 977 10 DANN LG LANCET 2 405 978 11 PLATT MW PEDIATR RES 12 874 978 12 SCHAAP T ISR J MED SCI 14 201 978 13 RAO GJS ENZYME 23 314 978 14 GUY GJ CLIN CHIM ACTA 87 63 978 15 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 16 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 17 DANN LG LANCET 2 907 979 18 WALSHPLATT M ENZYME 24 224 979 19 WALSH MMJ PEDIATR RES 14 353 980 20 LASZLO A ACTA PAEDIATR ACAD SCI HUNG 21 69 980 21 NADLER HL PEDIATRICS 66 690 980 22 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 23 BURY AF PEDIATR RES 16 613 982 24 SCHWARTZ M CLIN CHIM ACTA 124 213 982 25 TUMMLER B CLIN CHIM ACTA 125 219 982 26 BROCK DJH PRENAT DIAGN 3 1 983 27 BRANCHINI BR PEDIATR RES 17 850 983 28 HEELEY AF CLIN CHEM 29 2011 983 29 SLOMIANY A J BIOL CHEM 258 8535 983 30 BRIDGES MA ANN NY ACAD SCI 421 360 983 31 SEYMOUR CA BIOESSAYS 1 38 984 PN 75140 RN 00306 AN 76076886 AU Conod-E-J. Conover-J-H. Hirschhorn-K. TI Demonstration of human leukocyte degranulation induced by sera from homozygotes and heterozygotes for cystic fibrosis. SO Pediatr-Res. 1975 Sep. 9(9). P 724-9. MJ CYSTIC-FIBROSIS: bl. CYTOPLASMIC-GRANULES. GLUCURONIDASE: an. MYELOPEROXIDASE: an. NEUTROPHILS: en. PEROXIDASES: an. MN AMINOCAPROIC-ACIDS: pd. ANIMAL. CARRIER-STATE. CILIA: de. CYSTIC-FIBROSIS: en. CYTOCHALASIN-B: pd. CYTOPLASMIC-GRANULES: en, de. GLUCURONIDASE: ai. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IGG: me. NEUTROPHILS: de. TRACHEA: cy. AB The ability of epsilon-amino caproic acid (EACA)-treated normal serum and of cystic fibrosis (CF)-affected and carrier sera to promote the release of lysosomal enzymes from sensitized human polymorphonuclear leukocytes (PMN) was assessed through the measurement of beta- glucuronidase and myeloperoxidase activity after exposure of these cells to the various test sera. This study was initiated to extend the analogies between preciliary dyskinesia factor (pre-CDF), separated from the cell-free media of cultures derived from CF homozygous and heterozygous individuals, and C3a anaphylatoxin. The extent of lysosomal degranulation of human PMN exposed to fresh untreated sera of each of five controls, seven CF homozygotes, and eight heterozygotes, as expressed by the amount of beta-glucuronidase releases, was 7.84% (+/- 0.934) for control sera, 14.01% (+/- 1.79) for CF-affected sera, and 10.61% (+/- 1.43) for heterozygous sera. The difference between CF homozygotes and control subjects is significant (P less than 0.0001), as is the difference between CF- affected and carrier individuals (0.001 less than P less than 0.005) and between control subjects and carriers (0.001 less than P less than 0.005), when beta-glucuronidase. However, the differences between control subjects and CF heterozygous individuals are not significant. Treatment of these sera with 1 M EACA gave values for beta-glucuronidase and myeloperoxidase release which are slightly reduced when compared with those obtained with fresh, untreated samples. EACA apparently reduces the activity of beta-glucuronidase released from PMN. Amicon filtration studies of these serum samples demonstrated that degranulating ability and the presence of cilicary dyskinesia, as assessed by rabbit tracheal bioassay, are not always associated. Therefore, the relationship between pre-CDF and the degranulator activity in native CF-affected and carrier sera is unclear, in part because of the limitations inherent in the test systems employed. RF 001 BECKER EL J IMMUNOL 112 2047 974 002 BERATIS NG PEDIATR RES 7 958 973 003 BOWMAN BH SCIENCE 164 325 969 004 BRITTINGER G J CELL BIOL 37 394 968 005 CARTER SB NATURE 213 8261 967 006 COCHRANE CG J EXP MED 127 371 968 007 CONOVER JH PEDIATR RES 7 224 973 008 CONOVER JH PEDIATR RES 7 220 973 009 CONOVER JH LIFE SCI 14 253 974 010 DANES BS J EXP MED 136 1313 972 011 DAVIES P LAB INVEST 28 16 973 012 DAVIS AT PROC SOC EXP BIOL MED 137 161 971 013 ESTENSEN RD PROC NAT ACAD SCI USA 69 1430 972 014 GOLDSTEIN IM J IMMUNOL 111 33 973 015 GOLDSTEIN I PROC NAT ACAD SCI USA 70 2916 973 016 MULLER-EBERHARD HJ HARVEY LECTURES 66 75 971 017 MULLER-EBERHARD HJ IN: LEPOW IH 83 972 018 SPOCK A PEDIATR RES 1 173 967 019 VALLOTA EH J EXP MED 137 1109 973 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 BOWMAN BH TEX REP BIOL MED 34 1 976 3 HARPER BL TEX REP BIOL MED 34 73 976 4 WARD JB TEX REP BIOL MED 34 11 976 5 SCHNEYER CA CELL TISSUE RES 169 111 976 6 DISANTAGNESE PA N ENGL J MED 295 534 976 7 WILSON GB J LAB CLIN MED 92 463 978 8 PEARSON RD PEDIATR RES 13 834 979 9 JAKEL HP BIOL ZENTRALBL 98 55 979 10 SEALE TW PEDIATR RES 14 1398 980 11 HUGHES WT PEDIATR RES 16 874 982 12 ANON LANCET 2 249 985 13 VANHEYNINGEN V NATURE 315 513 985 14 KEMP T PEDIATR RES 20 520 986 PN 75141 RN 00307 AN 76076866 AU Shapiro-B-L. Smith-Q-T. Warick-W-J. TI Serum glutathione reductase and cystic fibrosis. SO Pediatr-Res. 1975 Dec. 9(12). P 885-8. MJ CYSTIC-FIBROSIS: en. GLUTATHIONE-REDUCTASE: bl. MN BLOOD-PROTEINS: an. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. FEMALE. HEPARIN: pd. HETEROZYGOTE. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: en. MALE. METHODS. TIME-FACTORS. AB Serum glutathione reductase (NADPH-GSSG oxidoreductase, EC. 1.6.4.2 (GR)) has been examined in cystic fibrosis subjects (CF), obligate CF heterozygotes, and control subjects. Serum protein concentration was similar in the three groups. Regardless of the units used to express activity (milligrams of protein or milliliters of serum) or whether or not samples were dialyzed against water or phosphate buffer, mean serum GR in CF was greater than in control subjects (P less than or equal to 0.002) in all series over several years. Under the above assay conditions no difference in serum GR between control subjects and carriers was detected. Calculated and assayed values of combined control and CF sera agreed as did expected and observed 50% activity in 1:2 sera dilutions in CF, control subjects, and carriers. Addition of FAD to incubation media did not effect enzyme activity in the three groups. Differences between CF and control subjects persisted after dialysis in membranes permitting passage of molecules of approximately 12,000 mol wt or less. These findings would tend to exclude the effect of extraneous serum factors in explaining the differences between CF and control subjects. The percentage of initial GR activity after four days storage (0-4 degrees) was significantly greater in CF than in control subjects (P less than 0.025). The effect of heparin on serum GR was recorded as the percentage of activity after incubation with heparin vs. activity in the standard assay for individual subjects. The effect of incubation with 5 mug/ml heparin on serum GR activity was greater in control subjects than in carriers (P less than 0.0005) and CF (P less than 0.0005). Mean serum GR activity in CF and carriers was unaffected by heparin, whereas mean activity in control subjects was decreased. In no control was the percentage of initial activity with heparin greater than the mean of CF and carrier groups. Only 3 of 20 CF and 4 of 20 carrier individuals had percentages lower than the control mean. The CF and carrier distributions were clearly different from the control distribution. Serum GR was determined in seven non-CF individuals with chronic obstructive pulmonary disease (COPD). Activity in the COPD was different from CF and no different from control subjects. In none of these controls or COPD was serum GR as great as the CF mean. Serum GR in no CF was as low as the mean of control subjects or COPD. It is concluded that serum GR activity is greater in CF than in control subjects, carriers, and non-CF COPD subjects; that the difference in activity is not attributable to an extraneous serum factor, that the activity difference is not secondary to chronic respiratory disease; that in comparison with control subjects, GR from CF serum behaves differently after storage; and that serum GR from CF and carriers behaves differently from control GR in the presence of heparin. RF 001 BEUTLER E SCIENCE 165 613 969 002 BREWER GJ IN: YUNIS JJ 139 969 003 DOGGETT RG NATURE NEW BIOL 243 250 973 004 HARRIS H PRIN OF HUMAN BIOCHEMICAL GEN 975 005 HORN HD IN: BERGMEYER HU 876 965 006 HORN HD BIOCHEM Z 331 58 958 007 CAUDILL M LANCET 2 307 973 008 LOBECK CC IN: STANBURY JB 1605 972 009 LOWRY OH J BIOL CHEM 193 265 951 010 MANGOS JA PEDIATR RES 2 378 968 011 SHAPIRO BL PROC IADR 47TH GENERAL MTG 503 969 012 SHAPIRO BL ANAT REC 166 87 970 013 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 014 SHAPIRO BL LANCET 2 1020 974 015 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 CT 1 STEPHAN U INTERNIST 17 336 976 2 WILL PC PEDIATR RES 13 1129 979 3 PRUSINER SB ANNU REV MED 32 521 981 PN 75142 RN 00308 AN 76052133 AU Hodes-M-E. Thomas-J. Morgan-S. Merritt-A-D. TI Do brine shrimp diagnose cystic fibrosis?. SO Pediatr-Res. 1975 Nov. 9(11). P 812-6. MJ CYSTIC-FIBROSIS: di. SHRIMP. MN ANIMAL. BIOLOGICAL-ASSAY: mt. CYSTIC-FIBROSIS: fg, bl. GENOTYPE. HUMAN. OXYGEN-CONSUMPTION. SALIVA. SHRIMP: me. SODIUM-CHLORIDE: pd. AB The nauplii of the brine shrimp Artemia salina are dependent upon the function of their salt gland to maintain osmotic pressure within narrow limits. A number of drugs interfere with this function and are lethal to the nauplii. Saliva and serum from normal persons, patients with cystic fibrosis, and obligate heterozygotes were tested for lethal effect against brine shrimp nauplii. At salt concentrations between 100 mM and 2.5 no difference was found among the phenotypes. At lower concentrations a difference was noted occasionally between some normal subjects and some individuals carrying one or two genes for cystic fibrosis. Data from an independent series of experiments indicate that the naupliar deaths result from distorted ratios of Na+/K+ and not from a specific gene product. No difference was noted in the O2 uptake of nauplii treated with saliva or serum obtained from normal subjects, patients with cystic fibrosis, or obligate heterozygotes. RF 001 BARGMAN GJ PEDIATR RES 8 464 974 002 CONNEALLY PM TEX REP BIOL MED 31 639 973 003 CONTE FP J COMP PHYSIOL 80 239 972 005 EWING RD J COMP PHYSIOL 80 247 972 006 HODES ME PEDIATR RES 8 212 974 007 HOOTMAN SR J COMP PHYSIOL 79 97 972 008 LOBECK CC IN: STANBURY JB 1605 972 009 MANGOS JA PEDIATR RES 1 436 967 010 SHWACHMAN H HOSP PRACT 9 143 974 CT 1 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 75143 RN 00309 AN 75176050 AU Martinez-J-R. Adelstein-E. Quissel-D. Barbero-G-J. TI The chronically reserpinized rat as a possible model for cystic fibrosis. I. Submaxillary gland morphology and ultrastructure. SO Pediatr-Res. 1975 May. 9(5). P 463-9. MJ CYSTIC-FIBROSIS: ci. DISEASE-MODELS-ANIMAL. RATS. RESERPINE. SUBMANDIBULAR-GLAND: ul. MN ANIMAL. BODY-WEIGHT. CELL-NUCLEUS: ul. CYTOPLASM: ul. ENDOPLASMIC-RETICULUM: ul. INJECTIONS-INTRAPERITONEAL. ISOPROTERENOL: pd. MALE. MICROSCOPY-ELECTRON. MUCINS: me. MUCOPROTEINS: me. NEURAL-TRANSMISSION: de. ORGAN-WEIGHT. PILOCARPINE: pd. RESERPINE: ad. STIMULATION-CHEMICAL. SUBMANDIBULAR-GLAND: me, pa. AB Rats treated for 7 days with reserpine develop structural changes in the submaxillary gland that resemble those that have been reported in cystic fibrosis. The salivary exocrinopathy is characterized by increased amounts of PAS-reactive mucoprotein in the acinar cells and by obstruction and dilatation of ducts by precipitated material with the same staining characteristics as those found in the acinar elements. RF 001 BARBERO GJ IN: DI SANTAGNESE PA 208 964 002 BENMILOUD M ACTA PHYSIOL SCAND 59 34 963 003 BLOMFIELD J GUT 14 558 973 004 CHERNICK WS ANN NY ACAD SCI 106 698 963 005 DI SANTAGNESE PA N ENGL J MED 277 1287 967 006 DI SANTAGNESE PA N ENGL J MED 277 1344 967 007 DI SANTAGNESE PA N ENGL J MED 277 1399 967 008 DOGGETT RG J CLIN PATHOL 24 270 971 009 JOHANSEN PG ANN NY ACAD SCI 106 755 963 010 LEESON CR HANDBOOK OF PHYSIOLOGY SECT 6 2 463 967 011 LEV R AM J PATHOL 46 23 965 012 MANGOS JA SCIENCE 158 135 967 013 PREECE A MANUAL FOR HISTOLIC TECHNIC 972 014 SCOTT BL AM J ANAT 104 115 959 015 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 016 SHACKLEFORD JM IN: DI SANTAGNESE PA 51 966 017 SPICER SS ANN NY ACAD SCI 106 379 963 018 SPICER SS IN: DI SANTAGNESE PA 26 966 019 TAMARIN A ANN NY ACAD SCI 106 609 963 020 TAYLOR PW JR J PHARMACOL EXP THER 156 483 967 021 WAGNER BM AM J DIS CHILD 99 61 960 CT 1 MARTINEZ JR J PHARMACOL EXP THER 194 384 975 2 THOMPSON FE PEDIATR RES 10 632 976 3 RENNERT OM TEX REP BIOL MED 34 187 976 4 MARTINEZ JR MO MED 73 173 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 DISANTAGNESE PA N ENGL J MED 295 597 976 7 WOOD DL PEDIATR RES 11 827 977 8 DAVIS PB PEDIATR RES 12 703 978 9 PERLMUTTER J PEDIATR RES 12 188 978 10 FORSTNER JF DIGESTION 17 234 978 11 SIMSON JAV LAB INVEST 39 157 978 12 SIMSON JAV J MICROSC 113 185 978 13 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 14 MARTINEZ JR PEDIATR RES 13 1156 979 15 MAWHINNEY TP PEDIATR RES 13 760 979 16 QUISSELL DO ARCH ORAL BIOL 24 639 979 17 SORDELLI DO LIFE SCI 24 2003 979 18 SETSER ME EXP MOL PATH 31 413 979 19 MARTINEZ JR EXPERIENTIA 35 1343 979 20 SETSER ME LAB INVEST 41 256 979 21 LEE JA ANN CLIN LAB SCI 10 227 980 22 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 23 MAWHINNEY TP PEDIATR RES 14 872 980 24 MORTON D PEDIATR RES 14 18 980 25 ROSCHER AA PEDIATR RES 14 261 980 26 BYLUND DB NATURE 285 229 980 27 MCCURDY RE PEDIATR RES 15 1308 981 28 GALANT SP J CLIN INVEST 68 253 981 29 ROSCHER AA J PHARMACOL EXP THER 216 419 981 30 FORSTNER J AM J PHYSIOL 241 G443 981 31 CUTLER LS VIRCHOWS ARCH PATHOL ANAT HIS 392 185 981 32 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 33 SHIFFMAN ML PEDIATR RES 16 104 982 34 FORSTNER G ADV EXP MED BIOL 144 199 982 35 JONES CJ J PHYSIOL (LOND) 332 P 65 982 36 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 37 MARTINEZ JR PEDIATR RES 17 523 983 38 SHIFFMAN ML PEDIATR RES 17 486 983 39 DAVIS PB J CHRON DIS 36 269 983 40 WERLIN SL EXP MOL PATH 39 24 983 41 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 42 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 43 BOYD RL PEDIATR RES 18 1028 984 44 MULLER RM EXP MOL PATH 40 391 984 45 MULLER RM EXP MOL PATH 41 363 984 46 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 47 WATSON E J DENT RES 63 82 984 48 SATO K J CLIN INVEST 73 1763 984 49 BRADY RC EXP CELL RES 150 141 984 50 ROOMANS GM ANN NY ACAD SCI 428 121 984 51 BARDON A BIOCHEM MED 33 99 985 52 MULLER RM EXP MOL PATH 43 97 985 53 MULLER RM ACTA PHYSIOL SCAND 123 383 985 54 BRADY RC AM J PHYSIOL 248 G 54 985 55 MULLER RM SCANN ELECTRON MICROSC 1985 1583 985 56 SMITH AC CLIN GASTROENTEROL 15 815 986 57 DAVIS PB HORM METAB RES 18 217 986 58 HAZLETT D PEDIATR RES 20 1236 986 59 KLEIN RM ARCH ORAL BIOL 31 133 986 60 ROOMANS GM SCANN ELECTRON MICROSC 1986 165 986 PN 75144 RN 00310 AN 75176052 AU Beratis-N-G. Turner-B-M. Weiss-R. Hirschhorn-K. TI Arylsulfatase B deficiency in Maroteaux-Lamy syndrome: Cellular studies and carrier identification. SO Pediatr-Res. 1975 May. 9(5). P 475-80. MJ ARYLSULFATASES: df. MUCOPOLYSACCHARIDOSIS-6: en. MUCOPOLYSACCHARIDOSIS: en. SULFATASES: df. MN AMNIOTIC-FLUID: en. ARYLSULFATASES: an. CELL-LINE. CELLS-CULTURED. CHILD-PRESCHOOL. CHONDROITIN. CYSTIC-FIBROSIS: en. FEMALE. FIBROBLASTS: en. GALACTOSEMIA: en. GAUCHERS-DISEASE: en. GLYCOGENOSIS-2: en. HETEROZYGOTE. HOMOZYGOTE. HUMAN. LEUKOCYTES: en. LEUKODYSTROPHY-METACHROMATIC: en. LIPOCHONDRODYSTROPHY: en. LYMPHOID-TISSUE: en. LYSOSOMES: en. MALE. METABOLISM-INBORN-ERRORS: en. MUCOPOLYSACCHARIDOSIS-6: fg. PREGNANCY. SKIN. AB Arylsulfatase B deficiency was demonstrated in peripheral leukocytes, cultured skin fibroblasts, and a lymphoid line derived from a patient with MLS. The patient's parents demonstrated levels of arylsulfatase B that were intermediate between those found in patient and those in control subjects. The activity (mean plus or minus SD) in leukocytes from normal subjects, the patient's parents, and the patient was 113.7 plus or minus 36.2, 31.0, and 5.2 nmol 4-nitrocatechol/mg protein/hr, respectively. In skin fibroblasts of the same subjects the activity was 145.2 plus or minus 41.6, 58.5, and 7.0, respectively. Nine other lysosomal enzymes were normal in skin fibroblasts of the patient. No arylsulfatase B activity was detected in a lymphoid line established from the patient with MLS. The arylsulfatase B activity in cultured amniotic fluid cells from 10 normal pregnancies was 203.2 plus or minus 49.9. RF 001 BARTON RW J PEDIATR 80 114 972 002 BAUM H CLIN CHIM ACTA 4 453 959 003 BERATIS NG ANN HUM GENET 38 485 975 004 BERATIS NG BIRTH DEF ORIG ART SER 9 247 973 005 BERATIS NG MAMM CHROM NEWSL 14 114 973 006 CHOI KW SCIENCE 170 89 970 007 CONOVER JH PEDIATR RES 7 224 973 008 FLEISHER LD PEDIATR RES 8 388 974 009 FLUHARTY AL BIOCHEM BIOPHYS RES COMMUN 59 455 974 010 GLASSER JH J LAB CLIN MED 82 969 973 011 HALL CW ARCH BIOCHEM BIOPHYS 158 817 973 012 HO MW AM J HUM GENET 24 37 972 013 KABACK MM N ENGL J MED 282 1336 970 014 KINT JA SCIENCE 167 1268 970 015 LOWRY OH J BIOL CHEM 193 265 951 016 MAROTEAUX P B PRESSE MED 71 1849 963 017 MCKUSICK VA HERITABLE DISORDERS OF CONNEC 972 018 NITOWSKY HM J LAB CLIN MED 69 472 967 019 OBRIEN J BIOCHEM BIOPHYS RES COMMUN 60 1170 974 020 PERCY AK SCIENCE 161 594 968 021 SPECTOR EB PEDIATR RES 7 700 973 022 SPRANGER JW HELV PAEDIATR ACTA 25 337 970 023 STUMPF DA TRANS AMER SOC NEUROCHEM 3 125 972 024 STUMPF DA AM J DIS CHILD 126 747 973 025 ZIELKE K J EXP MED 136 197 972 CT 1 BERATIS NG CYTOGENET CELL GENET 15 195 975 2 KIM HJ AM J HUM GENET 27 755 975 3 KLEIJER WJ LANCET 2 50 976 4 CORTNER JA PEDIATR RES 10 927 976 5 BERATIS NG CYTOGENET CELL GENET 17 236 976 6 CANTZ M HUM GENET 32 233 976 7 ROY AB AUST J EXP BIOL MED SCI 54 111 976 8 DORFMAN A PROC NAT ACAD SCI USA 73 630 976 9 DELVIN EE BIOCHEM J 157 353 976 10 DYGGVE HV NEUROPADIATRIE 8 429 977 11 BERATIS NG PEDIATR RES 11 862 977 12 ZUFFARDI O ANN GENET (PARIS) 20 191 977 13 MACPHEE GB J CLIN PATHOL 30 278 977 14 VANBIERVLIET JPGM ARCH FR PEDIATR 34 362 977 15 RASTOGI SC ACTA NEUROL SCAND 56 389 977 16 FAROOQUI AA CLIN CHIM ACTA 74 93 977 17 CHRISTOMANOU H CLIN CHIM ACTA 79 527 977 18 JEZYK PF SCIENCE 198 834 977 19 TURNER BM BIOCHIM BIOPHYS ACTA 480 442 977 20 BERATIS NG J CLIN INVEST 62 1264 978 21 MOFFITT KD ARCH BIOCHEM BIOPHYS 190 247 978 22 JOLLY RD AM J MED GENET 4 293 979 23 PILZ H ANN NEUROL 6 315 979 24 HULTBERG B J CLIN CHEM CLIN BIOCHEM 17 795 979 25 GONCERZEWICZ M GENET POLON 20 265 979 26 TALLAN HH BIOCHEM MED 21 129 979 27 KAIBARA N HUM GENET 53 37 979 28 HABUCHI H J BIOL CHEM 254 7570 979 29 BERATIS NG DEVELOP PHARM THER 1 305 980 30 LEVY LA AM J CLIN PATHOL 73 416 980 31 FAROOQUI AA CLIN CHIM ACTA 100 285 980 32 VANDYKE DL AM J MED GENET 8 235 981 33 MCGOVERN MM ENZYME 26 206 981 34 SHAFITZAGARDO B AM J HUM GENET 33 564 981 35 VINE DT AM J HUM GENET 33 916 981 36 DANESINO C HUM GENET 56 371 981 37 RAPRAEGER AC DEVELOP BIOL 88 269 981 38 PATTERSON DF ADV HUM GENET 12 263 982 39 GRABOWSKI GA METH CELL BIOL 26 95 982 40 GLEW RH AM J CLIN NUTR 35 236 982 41 GLEW RH COMP BIOCHEM PHYSIOL (B) 72 581 982 42 GRAVEL RA ANAL BIOCHEM 119 360 982 43 MCGOVERN MM J BIOL CHEM 257 2605 982 44 BERATIS NG ISOZYMES 11 25 983 45 BERATIS NG AM J HUM GENET 35 21 983 46 BERATIS NG CLIN CHIM ACTA 134 11 983 47 DANESINO C CLIN GENET 26 462 984 48 DANESINO C ANN GENET (PARIS) 27 162 984 49 KRIVIT W N ENGL J MED 311 1606 984 50 BETREMIEUX P ANN PEDIATR (PARIS) 32 639 985 51 MCGOVERN MM GENETICS 110 733 985 52 BERATIS NG CLIN GENET 29 129 986 PN 75145 RN 00311 AN 75176051 AU Martinez-J-R. Adshead-P-C. Quissell-D-O. Barbero-G-J. TI The chronically reserpinized rat as a possible model for cystic fibrosis. II. Comparison and cilioinhibitory effects of submaxillary saliva. SO Pediatr-Res. 1975 May. 9(5). P 470-5. MJ CYSTIC-FIBROSIS: ci. DISEASE-MODELS-ANIMAL. RATS. RESERPINE. SALIVA. SUBMANDIBULAR-GLAND: se. MN ANIMAL. BIOLOGICAL-ASSAY. CALCIUM: an. CARBOHYDRATES: an. CILIA: de. GUANETHIDINE: pd. INJECTIONS-INTRAPERITONEAL. ISOPROTERENOL: pd. MUSSELS. PILOCARPINE: pd. PROTEINS: an. SALIVA: se, an. SODIUM: an. STIMULATION-CHEMICAL. SUBMANDIBULAR-GLAND: me, de. AB Submaxillary saliva from reserpine-treated rats was found to have alterations in composition similar to those reported in the same secretion from patients with cystic fibrosis. Changes included elevated concentrations of Na+, Ca++, protein, and carbohydrate. In addition, this saliva was found to have ciliotoxic properties similar to those of CF serum. The similarities in cilioinhibitory effects salivary composition, and histologic appearance justify the use of the chronically reserpinized rat as a model for cystic fibrosis. RF 002 BENMILOUD M ACTA PHYSIOL SCAND 59 34 963 003 BESLEY GTN J MED GENET 6 278 969 004 BOWMAN BH SCIENCE 164 325 969 005 CARLSON A PHARMACOL REV 18 541 966 006 CHERNICK WS J PEDIATR 59 890 961 007 CHERNICK WS ANN NY ACAD SCI 106 698 963 008 CHERNICK WS MOD PROBL PEDIATR 10 125 967 009 DI SANTAGNESE PA N ENGL J MED 277 1287 967 010 DI SANTAGNESE PA N ENGL J MED 277 1344 967 011 DI SANTAGNESE PA N ENGL J MED 277 1399 967 012 DISCHE Z PEDIATRICS 24 74 959 013 DOGGETT RG NATURE NEW BIOL 243 251 973 014 DREISBACH RH PROC SOC EXP BIOL MED 126 279 967 015 DUBOIS M ANAL CHEM 28 350 956 016 EKFORS T LAB INVEST 24 197 971 017 GUGLER EC J PEDIATR 71 585 967 018 IWAYAMA T J PHARMACOL EXP THER 184 95 973 019 JOHANSEN PG ANN NY ACAD SCI 106 755 963 020 LOWRY OH J BIOL CHEM 193 265 951 021 MANDEL ID AM J DIS CHILD 113 431 967 022 MANGOS JA SCIENCE 158 135 967 023 MANGOS JA PEDIATR RES 1 436 967 024 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 026 MENAKER L LAB INVEST 24 197 971 027 SCHNEYER CA AM J PHYSIOL 203 232 962 028 SCHWARTZ A NATURE 188 948 960 029 SHORE PA PHARMACOL REV 22 209 972 030 SPICER SS IN: DI SANTAGNESE PA 26 966 031 SPOCK A PEDIATR RES 1 173 967 032 SPOCK A GAP CONF REP CILIARY INHIBITO 973 033 TAKAHAMA M J ULTRASTRUCT RES 17 452 967 034 WILCKEN DEL SCIENCE 157 1332 967 CT 1 MARTINEZ JR J PHARMACOL EXP THER 194 384 975 2 THOMPSON FE PEDIATR RES 10 632 976 3 RENNERT OM TEX REP BIOL MED 34 187 976 4 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 5 MARTINEZ JR MO MED 73 173 976 6 WOOD RE AM REV RESPIR DIS 113 833 976 7 MAYO JW ARCH BIOCHEM BIOPHYS 175 507 976 8 HAUBRICH J ARCH OTO RHINO LARYNGOL 213 1 976 9 DISANTAGNESE PA N ENGL J MED 295 597 976 10 WOOD DL PEDIATR RES 11 827 977 11 SCHNEYER CA PROC SOC EXP BIOL MED 156 132 977 12 DANN LG LANCET 2 405 978 13 DAVIS PB PEDIATR RES 12 703 978 14 PERLMUTTER J PEDIATR RES 12 188 978 15 SIMSON JAV LAB INVEST 39 157 978 16 SIMSON JAV J MICROSC 113 185 978 17 MARTINEZ JR PEDIATR RES 13 1156 979 18 MAWHINNEY TP PEDIATR RES 13 760 979 19 QUISSELL DO ARCH ORAL BIOL 24 639 979 20 SETSER ME EXP MOL PATH 31 413 979 21 MARTINEZ JR EXPERIENTIA 35 1343 979 22 SETSER ME LAB INVEST 41 256 979 23 JAKEL HP BIOL ZENTRALBL 98 55 979 24 POINTON SE BIOCHIM BIOPHYS ACTA 584 231 979 25 BLOMFIELD J MICRON 11 459 980 26 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 27 MAWHINNEY TP PEDIATR RES 14 872 980 28 ROSCHER AA PEDIATR RES 14 261 980 29 BYLUND DB NATURE 285 229 980 30 MCCURDY RE PEDIATR RES 15 1308 981 31 COMPTON J ARCH ORAL BIOL 26 555 981 32 ROSCHER AA J PHARMACOL EXP THER 216 419 981 33 BYLUND DB J PHARMACOL EXP THER 218 134 981 34 FORSTNER J AM J PHYSIOL 241 G443 981 35 CUTLER LS VIRCHOWS ARCH PATHOL ANAT HIS 392 185 981 36 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 37 SHIFFMAN ML PEDIATR RES 16 104 982 38 ABE K J DENT RES 61 52 982 39 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 40 SCHNEYER CA J AUTONOM NERV SYST 8 171 983 41 JIRAKULSOMCHOK D J ORAL PATHOL 12 491 983 42 MARTINEZ JR PEDIATR RES 17 523 983 43 SHIFFMAN ML PEDIATR RES 17 486 983 44 MARTINEZ JR ARCH ORAL BIOL 28 1101 983 45 BLOMFIELD J EXP MOL PATH 38 170 983 46 MARTINEZ JR J DENT RES 62 543 983 47 BODNER L COMP BIOCHEM PHYSIOL (A) 74 829 983 48 MARTINEZ JR AM J PHYSIOL 245 G711 983 49 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 50 KATZ S CELL CALC 5 421 984 51 BOYD RL PEDIATR RES 18 1028 984 52 JIRAKULSOMCHOK D ARCH ORAL BIOL 29 39 984 53 MULLER RM EXP MOL PATH 40 391 984 54 MULLER RM EXP MOL PATH 41 363 984 55 WATSON E J DENT RES 63 82 984 56 BRADY RC EXP CELL RES 150 141 984 57 ROOMANS GM ANN NY ACAD SCI 428 121 984 58 MARTINEZ JR PEDIATR RES 19 711 985 59 BARDON A BIOCHEM MED 33 99 985 60 MULLER RM EXP MOL PATH 43 97 985 61 MULLER RM ACTA PHYSIOL SCAND 123 383 985 62 BRADY RC AM J PHYSIOL 248 G 54 985 63 MULLER RM SCANN ELECTRON MICROSC 1985 1583 985 64 HAZLETT D PEDIATR RES 20 1236 986 65 ROOMANS GM SCANN ELECTRON MICROSC 1986 165 986 PN 75146 RN 00312 AN 76076880 AU Welch-D-W. Roberts-R-M. TI Complex saccharide metabolism in cystic fibrosis fibroblasts. SO Pediatr-Res. 1975 Sep. 9(9). P 698-702. MJ CYSTIC-FIBROSIS: me. FIBROBLASTS: me. MUCOPOLYSACCHARIDES: me. MN AUTORADIOGRAPHY. CELL-LINE. CHILD. CHILD-PRESCHOOL. CHONDROITIN-SULFATES: me. DERMATAN-SULFATE: me. FIBROBLASTS: an. GLUCOSAMINE: me. GLYCOSAMINOGLYCANS: ip. HEPARITIN-SULFATE: me. HUMAN. HYALURONIC-ACID: me. SKIN. SULFATES: me. TIME-FACTORS. SUPPORT-U-S-GOVT-P-H-S. AB There are several reports of secretory and other abnormalities present in cultured fibroblasts from patients with cystic fibrosis (CF). We have, therefore, investigated aspects of complex saccharide synthesis and secretion by such cells compared with fibroblasts derived from heterozygous (HZ) parents and from normal (N) children. The main glycosaminoglycans produced by skin fibroblasts during in vitro culture were hyaluronic acid, heparan sulfates, and dermatan sulfate-like materials. Using double-label experiments with D-[3H]- or [14c]glucosamine and analyzing the products by ion exchange chromatography, it was shown for five CF, two HZ, and four N lines that these polysaccharides were secreted into the medium in approximately similar proportions to each other. Moreover, experiments in which three CF, nine HZ, and three N lines were grown in log phase for up to 5 days in the presence of [35S]sulfate and [3H]glucosamine indicate that, during such a period, CF fibroblasts do not secrete complex carbohydrates at rates significantly different from N or HZ cells. Neither do such cells shown an abnormal intracellular accumulation of complex carbohydrates. The latter observation was further confirmed by preparing whole cell autoradiographs during growth of six CF, two HZ, and three N lines in the presence of D-[3H]glucosamine and, subsequently, after addition of unlabeled medium. RF 001 BADER JP BIOCHIM BIOPHYS ACTA 264 73 972 002 BARTMAN J J PEDIATR 76 430 970 003 DANES BS LANCET 1 1061 968 004 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 005 DANES BS J EXP MED 129 775 969 006 DANES BS NATURE 222 685 969 007 DI SANTAGNESE PA PEDIATRICS 12 549 953 008 DI SANTAGNESE PA N ENGL J MED 277 1344 967 009 FITZPATRICK DF NATURE NEW BIOL 235 173 972 010 GIBSON LE PEDIATRICS 48 695 971 011 GUGLER EC J PEDIATR 71 585 967 012 KRAEMER PM BIOCHEMISTRY 10 1437 971 013 KRAEMER PM BIOCHEMISTRY 10 1445 971 014 KRAEMER PM IN: MANSON LA 1 67 971 015 KONDO K BIOCHIM BIOPHYS ACTA 244 513 971 016 LOBECK CC IN: STANBURY JB 1605 972 017 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 018 MILUNSKY A N ENGL J MED 281 1128 969 019 NADLER HL LANCET 2 84 969 020 PALLAVICINI JC J PEDIATR 77 280 970 021 RENNERT OM CLIN PEDIATR 11 351 972 022 RENNERT OM IN: MANGOS JA 41 973 023 SATOH C PROC NAT ACAD SCI USA 70 54 973 024 SCHUBERT M J HISTOCHEM CYTOCHEM 4 159 956 025 SPIRO RG J BIOL CHEM 240 1603 965 026 TAYSI K N ENGL J MED 281 1108 969 027 TURNER JC INT J APPL RADIAT ISOTOP 19 557 968 028 WESSLER E ANAL BIOCHEM 26 439 968 029 WIESMANN UN J PEDIATR 77 685 970 CT 1 STEIN GS NATURE 258 639 975 2 WARD JB TEX REP BIOL MED 34 11 976 3 ANDERSON LC CLIN CHIM ACTA 73 63 976 4 DISANTAGNESE PA N ENGL J MED 295 534 976 5 SULLIVAN JL BIOCHEM GENET 15 1125 977 6 CONRAD GW J BIOL CHEM 252 6861 977 7 OWEN E J MOL MED 3 203 978 8 ALHADEFF JA CLIN GENET 14 189 978 9 JAKEL HP BIOL ZENTRALBL 98 55 979 10 SHAPIRO BL SCIENCE 203 1251 979 11 SPICER SS EXP MOL PATH 33 104 980 12 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 13 MAPLESON JL J CELL PHYSIOL 109 215 981 14 MASTERS RA J REPROD FERTIL 66 571 982 15 CEDER O SCANN ELECTRON MICROSC 1982 723 982 16 RUDICK VL J CELL PHYSIOL 115 143 983 17 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 PN 75147 RN 00313 AN 76076885 AU McEvoy-F-A. Hartley-C-B. TI Polyamines in cystic fibrosis. SO Pediatr-Res. 1975 Sep. 9(9). P 721-4. MJ CYSTIC-FIBROSIS: bl. POLYAMINES: bl. MN BILE: an. DUODENUM. HUMAN. INTESTINAL-SECRETIONS: an. POLYAMINES: an. SALIVA: an. SPERMIDINE: an. SPERMINE: an. SWEAT: an. AB Polyamine concentrations have been measured in blood samples from a series of cystic fibrosis (CF) patients and control subjects. Analyses were made by a fluorometric method, in which the amines were reacted with dansyl chloride and separated by thin layer chromatography on Kieselguhr. There was no significant difference between the polyamine concentrations of blood samples from CF patients and control subjects. Also, the spermidine-spermine ratio was similar in both groups. Polyamines were also analyzed in exocrine fluids, and although none could be detected in either CF or control sweat or saliva, significant amounts of spermine and spermidine were present in all samples of bile and duodenal fluids. It thus seems likely that the biliary route is a significant pathway in the human for excretion of polyamines. The observation that polyamines are excreted in considerable concentrations via the bile in children may be of interest in view of the reports of elevated urinary polyamine concentrations in neoplastic states. Since measurable amounts of spermine and spermidine were detected in all six bile preparations available for this study, analysis of these substances in bile may be of benefit in particular cases. RF 001 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 002 BACHRACH U BIOCHEM BIOPHYS RES COMMUN 55 1035 973 003 COHEN SS INTRODUCTION TO POLYAMINES 971 004 DAVENPORT HW PHYSIOLOGY OF THE DIGESTIVE T 966 005 DI SANTAGNESE PA PEDIATRICS 22 507 958 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 DREYFUS G CLIN CHIM ACTA 49 65 973 008 GIBSON LE PEDIATRICS 23 545 959 009 HAMMOND JE ANAL BIOCHEM 22 474 968 010 HARRIS R BR J HAEMATOL 18 229 968 011 HERBST EJ ANN NY ACAD SCI 171 3 970 012 JOHANSEN PG LANCET 1 455 968 013 RAINA AA SCAND J CLIN LAB INVEST 14 318 962 014 RAINA A ACTA PHYSIOL SCAND SUPPL 218 60 1 963 015 RENNERT OM IN: MANGOS JA 41 973 016 RUSSELL DH POLYAMINES IN NORMAL AND NEOP 973 017 RUSSELL DH NATURE 233 144 971 018 SEILER N METHODS BIOCHEM ANAL 18 259 970 019 STEVENS L BIOL REV 45 1 970 020 TABOR H PHARMACOL REV 16 245 964 CT 1 ANON J PEDIATR 88 711 976 2 DISANTAGNESE PA N ENGL J MED 295 534 976 3 KELLY JC BIOCHEM GENET 15 695 977 4 ALHADEFF JA CLIN GENET 14 189 978 5 SCHRODER CP EUR J PEDIATR 128 49 978 6 BAYLIN SB PEDIATR RES 14 921 980 PN 75148 RN 00314 AN 75083182 AU Shwachman-H. Lebenthal-E. Khaw-K-T. TI Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes. SO Pediatrics. 1975 Jan. 55(1). P 86-95. MJ AMYLASES. CHYMOTRYPSIN. CYSTIC-FIBROSIS: co. LIPASE. PANCREAS: en. PANCREATITIS: et. TRYPSIN. MN ACUTE-DISEASE. ADOLESCENCE. ADULT. AMYLASES: bl, ur, se. CHLORIDES: an. CHYMOTRYPSIN: an. CYSTIC-FIBROSIS: en, pa. FEMALE. GLUCOSE-TOLERANCE-TEST. HUMAN. INTUBATION-GASTROINTESTINAL. LIPASE: bl, se. MAGNESIUM: an. MALE. METHIONINE: du. PAIN. PANCREAS: pa. PANCREATITIS: en, pa. CHOLECYSTOKININ: du. POTASSIUM: an. RECURRENCE. SECRETIN: du. SELENIUM: du. SODIUM: an. TRYPSIN: an. SUPPORT-U-S-GOVT-P-H-S. SUPPORT-U-S-GOVT-NON-P-H-S. AB Ten adolescent and young adults with cystic fibrosis (CF) have had well-documented recurrent attacks of acute pancreatitis. The diagnosis of CF in each patient was delayed because they did not have pancreatic insufficiency. The diagnosis of CF was documented by the typical pulmonary involvement and elevated sweat sodium and chloride levels in all cases and a positive family history in six of the ten patients. Two patients were diagnosed as having acute pancreatitis before the diagnosis of CF was made, thus indicating that acute pancreatitis may be the presenting complaint in the young adult with CF. The diagnosis of acute pancreatitis was based on the presence of severe abdominal pain, usually with vomiting, tenderness in the mid- epigastrium, elevated serum and urinary amylase and serum lipase. Attacks were precipitated by fatty meals, alcohol ingestion; postcholecystectomy and tetracycline administration. In some patients no precipitating event could be elicited. Intravenous secretin- pancreozymin stimulation tests revealed a diminished bicarbonate secretion with little effect on the secretion of the zymogen enzymes. A mild attack of pancreatitis occurred after secretin-pancreozymin stimulation. The endocrine pancreatic function tested in four patients was normal as revealed by the glucose tolerance tests and determinations of serum insulin, growth hormone and free fatty acid. Transduodenal pancreatograms were performed in three patients; one showed a normal pancreatic duct, one showed duct obstruction and in the third patient a beady type of narrowing was found. The selenomethionine Se 75 uptake of the pancreas was noted only in the head of the pancreas. This suggests that loss of function occurs initially to a greater extent in the tail and body of the pancreas. Three patients died and showed characteristic lesions of CF. RF 001 FARBER S J MICH MED SOC 44 587 945 002 DI SANTAGNESE PA PEDIATRICS 15 683 955 003 SHWACHMAN H AM J DIS CHILD 92 347 956 004 RICK W MED WELT 42 2158 963 005 HADORN B J PEDIATR 73 39 968 006 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 007 SHWACHMAN H AM J DIS CHILD 96 6 958 008 FARBER S ARCH PATHOL 37 238 944 009 LAGERLOF HO ACTA MED SCAND SUPPL 128 1 942 010 HENRY RJ CLIN CHEM 6 434 960 011 NAGEL W PHYSIOL CHEM 340 1 965 012 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 013 COTTON PB LANCET 1 53 972 014 KASUGAI T GASTROENTEROLOGY 63 217 972 015 BACHRACH WH GASTROENTEROLOGY 63 890 972 016 DOLE VP J CLIN INVEST 35 150 956 017 SOELDNER JS DIABETES 14 771 965 018 SHWACHMAN H N ENGL J MED 286 1300 972 019 SNYDER WH JR PEDIATRICS 34 72 964 020 HOLSCLAW DS PEDIATRICS 48 51 971 021 SHWACHMAN H IN: KENDIG EL JR 1 524 972 022 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 023 SHWACHMAN H PEDIATRICS 30 389 962 024 KULCZYCKI LL N ENGL J MED 259 409 958 025 HOLSCLAW DS PEDIATRICS 48 442 971 026 KAPLAN E N ENGL J MED 279 65 968 027 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 028 POPPEL MH SEMIN ROENTGENOL 3 227 968 029 GEORGE PK GASTROENTEROLOGY 60 858 971 030 HANSSON K ACTA CLIN SCAND SUPPL 375 1 967 031 KALANT H GASTROENTEROLOGY 56 380 969 032 DWORKIN B PHYSIOLOGIST 8 158 965 033 OPPENHEIMER EH ARCH PATHOL 29 790 940 034 KORNBLITH BA AM J PATHOL 5 249 929 035 ANDERSEN DH AM J DIS CHILD 56 344 938 036 ZUELZER WW PEDIATRICS 4 53 949 037 BANKS PA GASTROENTEROLOGY 61 382 971 CT 1 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 2 LESEC G NOUV PRESSE MED 5 2470 976 3 HOLSCLAW DS J PEDIATR SURG 11 217 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 LEBENTHAL E CLIN GASTROENTEROL 6 397 977 6 ANDORSKY M AM J DIG DIS 22 56 977 7 SIBERT JR ARCH DIS CHILD 52 658 977 8 SHWACHMAN H MEDICINE 56 129 977 9 STERN RC ANN INTERN MED 87 188 977 10 SCHWACHMAN H N ENGL J MED 296 1519 977 11 BOURRY J GASTROENTEROL CLIN BIOL 2 139 978 12 DAVIDSON GP PEDIATR RES 12 967 978 13 BOURRY J AM J DIG DIS 23 423 978 14 HUBBARD VS J PEDIATR 92 685 978 15 STERN RC JAMA 239 2676 978 16 FEIGELSON J NOUV PRESSE MED 8 3029 979 17 SWAN HAP S AFR J SURG 17 51 979 18 SAHEL J DIG DIS SCI 24 897 979 19 SIBERT JR POSTGRAD MED J 55 171 979 20 NEZELOF C PEDIATRICS 63 361 979 21 SANTAGNESE PAD AM J MED 66 121 979 22 GILLARD BK PEDIATR RES 14 1168 980 23 BURLINA A SCAND J GASTROENTEROL 15 35 980 24 DURIE PR PEDIATR RES 15 1351 981 25 MENEELY RL PEDIATRICS 67 136 981 26 PARK RW GASTROENTEROLOGY 81 1143 981 27 STERN RC LANCET 1 1401 982 28 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 29 POCKNEE RC BR J OBSTET GYNAECOL 89 142 982 30 BARBERO GJ J PEDIATR 100 914 982 31 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 32 KARP MP J PEDIATR GASTROENTEROL NUTR 2 324 983 33 MASARYK TJ DIG DIS SCI 28 874 983 34 GHISHAN FK J PEDIATR 102 514 983 35 GILLARD BK AM J DIS CHILD 138 577 984 36 SCOTT HW ANN SURG 199 610 984 37 HUBBARD VS SEM RESPIR MED 6 299 985 38 ORENSTEIN DM SEM RESPIR MED 6 252 985 39 ISAACS JD J CLIN GASTROENTEROL 7 533 985 40 OCONNOR KW ARCH INTERN MED 145 153 985 41 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 42 BRAGANZA JM MED HYPOTHESES 20 233 986 43 DURIE PR PEDIATR RES 20 209 986 44 LIU P J CAN ASSOC RADIOL 37 279 986 45 SARNELLI R AM J MED 80 541 986 46 HUNSINGER RN CLIN CHIM ACTA 156 165 986 PN 75149 RN 00315 AN 75176126 AU David-S. TI Letter: Comment on outpatient intravenous therapy. SO Pediatrics. 1975 Jun. 55(6). P 896-7. MJ AMBULATORY-CARE. DRUG-THERAPY. INJECTIONS-INTRAVENOUS. PEDIATRICS. MN ADOLESCENCE. ANTIBIOTICS: ad, tu. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt. FOLLOW-UP-STUDIES. HEMOPHILIA: dt. HUMAN. INFANT. PATIENT-COMPLIANCE. PUBLIC-HEALTH-NURSING. RESPIRATORY-TRACT-INFECTIONS: dt. EX There has been a recent trend to treating more and more pediatric patients with chronic diseases on an ambulatory basis. This is true of both hemophilia and cystic fibrosis. Despite their success in keeping slightly more than two thirds of their patients out of the hospital, I think that they might be more successful in the future if the following points are considered. First, many of those initially selected for intravenous medications due to their nonresponsiveness to oral therapy and again later considered failures of intravenous treatment might actually be noncompliers. My second point concerns the lack of follow-up within the patient's home. It would seem to me that this would be an ideal place for a public health nurse to check on the patient's condition, ensure that the procedure was being properly followed under actual field conditions, and that the correct amount of medicine has been utilized. - Dr. David's letter regarding potential problems of noncompliance and home follow-up in the use of outpatient intravenous antibiotics points out the need for this type of therapy to be instituted only in those centers in which these facets can be well controlled. Public health nurses are utilized by some centers to supervise and assist in the home management of cystic fibrosis with good success. RF 001 RABINER SF N ENGL J MED 283 1011 970 002 STRAWCZYNSKI H PEDIATRICS 51 986 973 003 RUCKER RW PEDIATRICS 54 358 974 004 LASAGNA L N ENGL J MED 289 267 973 PN 75150 RN 00316 AN 75083181 AU Whitman-V. Stern-R-C. Bellet-P. Doershuk-C-F. Liebman-J. Boat-T-F. Borkat-G. Matthews-L-W. TI Studies on cor pulmonale in cystic fibrosis: I. Effects of diuresis. SO Pediatrics. 1975 Jan. 55(1). P 83-5. MJ CYSTIC-FIBROSIS: co. DIURESIS. ETHACRYNIC-ACID: tu. PULMONARY-HEART-DISEASE: et. MN ACID-BASE-EQUILIBRIUM. ADOLESCENCE. ADULT. BLOOD-PRESSURE. CARBON-DIOXIDE: bl. CARDIAC-OUTPUT. CYSTIC-FIBROSIS: pp. DRUG-EVALUATION. ETHACRYNIC-ACID: ad, ae. HEART-CATHETERIZATION. HEART: pp. HUMAN. HYDROGEN-ION-CONCENTRATION. OXYGEN: bl. PARTIAL-PRESSURE. PULMONARY-CIRCULATION. PULMONARY-HEART-DISEASE: dt, pp. SUPPORT-U-S-GOVT-NON-P-H-S. AB The effects of acute diuresis in patients with cor pulmonale secondary to cystic fibrosis were studied. Both hemodynamic parameters and arterial blood gas changes were investigated. The major effect noted was a significant reduction of systemic venous pressure. Pulmonary artery and pulmonary artery wedge pressure either remained constant or declined slightly. No consistent changes were noted in cardiac output or arterial blood gases. Acute diuresis of moderate degree appears to be a safe and effective manner in which to treat the systemic venous congestion of cor pulmonale in this situation. RF 001 FISHMAN AP HOSP PRACT 6 101 971 002 KINSMAN JM AM J PHYSIOL 89 322 929 003 MOSS AJ J PEDIATR 67 797 965 004 GOLDRING RM J PEDIATR 65 501 964 005 BERNE RM CARDIOVASCULAR PHYSIOLOGY 967 006 NOBLE MIM LANCET 2 257 966 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 ROSENTHAL A PEDIATRICS 59 919 977 3 TANG SC CHIN MED J 93 779 980 4 MOSS AJ PEDIATRICS 70 728 982 PN 75151 RN 00317 AN 75083179 AU Wilmshurst-E-G. Soeldner-J-S. Holsclaw-D-S. Kaufmann-R-L. Shwachman-H. Aoki-T-T. Gleason-R-E. TI Endogenous and exogenous insulin responses in patients with cystic fibrosis. SO Pediatrics. 1975 Jan. 55(1). P 75-82. MJ BLOOD-GLUCOSE. CARBOHYDRATES: me. CYSTIC-FIBROSIS: pp. INSULIN. MN ADMINISTRATION-ORAL. ADOLESCENCE. ADULT. BLOOD-GLUCOSE: an. FATTY-ACIDS-NONESTERIFIED: bl. GLUCOSE-TOLERANCE-TEST. GLUCOSE: ad. HUMAN. INJECTIONS-INTRAVENOUS. INSULIN: se, bl, du. MALE. NITROGEN: bl. TOLBUTAMIDE: du. SUPPORT-U-S-GOVT-NON-P-H-S. AB Eight male patients with cystic fibrosis, normal nutrition, normal physical activity, relatively mild pulmonary disease, no evidence of liver disease and no family history of diabetes mellitus underwent a series of carbohydrate tolerance tests in comparison with a group of 18 normal male subjects matched for age and body weight. Compared with the normal group, the patients with cystic fibrosis had significantly impaired glucose tolerance and significantly lower serum immunoreactive insulin levels during oral and intravenous glucose tolerance tests; serum insulin levels were also significantly lower after intravenous administration of tolbutamide in the patients with cystic fibrosis, but the reduction in blood glucose concentration in each group was not significantly different. During an intravenous insulin test, the decrease in blood glucose concentration was the same for both groups, in spite of significantly lower serum insulin levels in the patients with cystic fibrosis .The percentage fall in plasma free fatty acids was at least as great in the patients with cystic fibrosis as in normals during the test procedures; while a significant decrease in plasma alpha-amino nitrogen after intravenously administered insulin was seen only in the patients with cystic fibrosis. These studies suggest that the carbohydrate intolerance of cystic fibrosis is consequent upon an impaired insulin response to glucose, but that this insulin deficiency is partly compensated for by increased peripheral tissue sensitivity to insulin. RF 001 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 002 TALAMO RC CALIF MED 110 432 969 003 MCKUSICK VA MENDELIAN INHERIT IN MAN 167 966 004 ROSAN RC AM J DIS CHILD 104 625 962 005 WANG CI CF CLUB ABST 8 1 967 006 MILNER AD ARCH DIS CHILD 44 351 969 007 HOLSCLAW DS PROC AM PEDIATR SOC ANNU MTG 59 970 008 HANDWERGER S N ENGL J MED 281 451 969 009 SHWACHMAN H PEDIATRICS 36 689 965 010 HOFFMAN WS J BIOL CHEM 120 51 937 011 SOELDNER JS DIABETES 14 771 965 012 BODEN G DIABETOLOGICA 3 413 967 013 DOLE VP J BIOL CHEM 235 2595 960 014 RUSH RL ADV AUTOMATED ANALYSI 503 970 015 MOORE S J BIOL CHEM 211 907 954 016 KAHN CB N ENGL J MED 281 343 969 017 MILUNSKY A AM J DIS CHILD 121 15 971 018 KAISER G HELV PAEDIATR ACTA 25 135 970 019 PERLEY MJ DIABETES 15 867 966 020 SELTZER HS J CLIN INVEST 46 323 967 021 BIERMAN EL DIABETES 6 475 957 022 HALES CN LANCET 1 790 963 023 SHAFRIR E DIABETES 14 77 965 024 WILMSHURST EG DIABETES SUPPL 21 383 972 025 KAHN CB DIABETES SUPPL 21 365 972 CT 1 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 DISANTAGNESE PA N ENGL J MED 295 597 976 4 SHWACHMAN H MEDICINE 56 129 977 5 LIPPE BM J PEDIATR 90 751 977 6 GUYTON JR DIABETES 27 1027 978 7 WOOD RE SOUTH MED J 72 189 979 8 LIPPE BM PEDIATRICS 65 1018 980 9 ADRIAN TE GASTROENTEROLOGY 79 460 980 10 ROSS SA PEDIATRICS 67 252 981 11 BISTRITZER T ISR J MED SCI 19 600 983 12 HILLEMEIER C YALE J BIOL MED 56 195 983 13 PEDERSEN PS ACTA PAEDIATR SCAND 72 757 983 14 IANNUCCI A HUM PATHOL 15 278 984 15 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 16 GEFFNER ME AM J DIS CHILD 138 677 984 17 MOHAN V DIABETE METAB 11 376 985 18 KNOPFLE G KLIN PAEDIATR 197 13 985 19 RODMAN HM MEDICINE 65 389 986 20 DOLAN TF N ENGL J MED 314 991 986 21 WINTER WE N ENGL J MED 316 285 987 PN 75152 RN 00318 AN 75083169 AU Stephan-U. Busch-E-W. Kollberg-H. Hellsing-K. TI Cystic fibrosis detection by means of a test-strip. SO Pediatrics. 1975 Jan. 55(1). P 35-8. MJ ALBUMINS: an. CYSTIC-FIBROSIS: di. INDICATORS-AND-REAGENTS. INFANT-NEWBORN-DISEASES: di. MASS-SCREENING: mt. MECONIUM: an. REAGENT-STRIPS. MN EVALUATION-STUDIES. FALSE-NEGATIVE-REACTIONS. FALSE-POSITIVE-REACTIONS. GEL-DIFFUSION-TESTS. HUMAN. INFANT. INFANT-NEWBORN. IONTOPHORESIS. MASS-SCREENING: is. METHODS. PHENOLPHTHALEINS. PILOCARPINE. AB The effectiveness of meconium screening for albumin as an indication of cystic fibrosis is examined. BM-Test Meconium was applied to 69,000 investigations. In 60 positive tests, cystic fibrosis was confirmed later. No increased albumin content was observed in four cases of cystic fibrosis. RF 001 GEORGE L ARCH DIS CHILD 46 139 971 002 WARWICK WJ MINN MED 52 1567 969 003 SHWACHMAN H PEDIATRICS 46 335 970 004 KOCH G IN: VON WINDORFER A 30 968 005 WRIGHT SW AM J HUM GENET 20 157 968 006 KULCZYCKI LL AM J DIS CHILD 127 64 974 007 GLANZMANN E ANN PAEDIATR 175 33 950 008 BUCHANAN DJ PEDIATRICS 9 304 952 009 GREEN MN PEDIATRICS 21 635 958 010 WISER WC PEDIATRICS 33 115 964 011 GREEN MN PEDIATRICS 41 989 968 012 SCHUTT WH ARCH DIS CHILD 43 178 968 013 BRAY PT PROC EWGCF 3RD ANNU MTG 972 014 PROSSER R PROC EWGCF 4TH ANNU MTG 973 015 CAIN ARR ARCH DIS CHILD 47 131 972 016 GIBBS GE PAEDIATRICIAN 1 133 972 017 HOBBS JR PROTIDES BIOL FLUIDS 17 517 969 018 KOLLBERG H ARCH DIS CHILD 47 836 972 019 SHWACHMAN H IN: MANGOS JA 277 973 020 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 022 PROSSER R ARCH DIS CHILD 49 597 974 023 STEPHAN U IN: MANGOS JA 281 973 024 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 025 STEPHAN U PEDIATRE 10 63 974 026 STEPHAN U KLIN PAEDIATR 186 79 974 027 STEPHAN U CF CLUB ABST 15 974 028 KAISER D HELV PAEDIATR ACTA 29 51 974 030 THORNER RM PUBLIC HEALTH MONOGRAPH DHEW 10 967 031 PRITCHARD JA OBSTET GYNECOL 25 289 965 032 RULE AH PEDIATRICS 45 847 970 033 EGGERMONT E BIOL NEONATE 10 266 966 034 SHWACHMAN H HOSP PRACT 9 143 974 036 GOTTSCHALK B DTSCH GESUNDHEITSW 28 2049 973 037 LAWSON D ARCH DIS CHILD 47 1 972 CT 1 CHILDS B ANNU REV GENET 9 67 975 2 BERRY HK FED PROC 34 2134 975 3 ANTONOWICZ I LANCET 1 746 976 4 ANON LANCET 2 614 976 5 RYLEY HC LANCET 2 365 976 6 STEPHAN U INTERNIST 17 336 976 7 LANZA I MINERVA PEDIATR 28 1510 976 8 EGGERMONT E ARCH DIS CHILD 51 901 976 9 GRAND RJ GASTROENTEROLOGY 70 790 976 10 ANON J PEDIATR 88 711 976 11 WOOD RE AM REV RESPIR DIS 113 833 976 12 ANON BR MED J 1 596 977 13 TENKATE LP INT J EPIDEMIOL 6 23 977 14 CARTER CO J MED GENET 14 316 977 15 ANDORSKY M AM J DIG DIS 22 56 977 16 TENKATE LP ANN HUM GENET 40 287 977 17 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 18 BRUNS WT AM J DIS CHILD 131 71 977 19 KATZNELSON D ISR J MED SCI 14 204 978 20 FIFI AR MINERVA PEDIATR 30 597 978 21 ANTONOWICZ I BIOL NEONATE 34 225 978 22 WATTS RWE EXPERIENTIA 34 143 978 23 RETIEF AE S AFR MED J 54 849 978 24 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 25 GOTZ M EUR J PEDIATR 127 133 978 26 ROSENSTEIN BJ AM J DIS CHILD 132 167 978 27 SHWACHMAN H AM J DIS CHILD 132 1112 978 28 HOSLI P LANCET 2 543 979 29 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 30 RYLEY HC MONOGR PAEDIATR 10 151 979 31 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 32 RYLEY HC ARCH DIS CHILD 54 92 979 33 DOYLE EE IR MED J 72 93 979 34 WOOD RE SOUTH MED J 72 189 979 35 JAKEL HP BIOL ZENTRALBL 98 55 979 36 HOSLI P FEBS LETTERS 104 271 979 37 ANON CAN MED ASSOC J 121 1193 979 38 BERRY HK AM J DIS CHILD 134 930 980 39 MASTELLA G RIV ITAL PEDIATR 7 581 981 40 HSIEH MC CLIN CHEM 27 388 981 41 EVANS RT J CLIN PATHOL 34 911 981 42 FORREST DC ARCH DIS CHILD 56 151 981 43 SHWACHMAN H ARCH DIS CHILD 56 137 981 44 BORGSTROM A ACTA PAEDIATR SCAND 70 619 981 45 CAVELL B ACTA PAEDIATR SCAND 70 635 981 46 PARK RW GASTROENTEROLOGY 81 1143 981 47 MACHILL G BIOL ZENTRALBL 100 449 981 48 ANON LANCET 1 1000 982 49 KOLLBERG H ACTA PAEDIATR SCAND 71 197 982 50 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 51 ROSENSTEIN BJ JAMA 247 3313 982 52 MOHRENWEISER HW ISOZYMES 10 51 983 53 HEELEY AF CLIN CHEM 29 2011 983 54 POSSELT HG ATEMWEGS LUNGENKRANKH 10 345 984 55 HSIEH MC CLIN CHIM ACTA 138 221 984 56 NAYLOR EW SEM PERINATOL 9 232 985 57 PERINI JM BULL EUR PHYSIOPATH RESP 21 569 985 58 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 59 STOLLINGER O WIEN MED WOCHENSCHR 135 307 985 60 KNOPFLE G KLIN PAEDIATR 197 13 985 61 KNOPFLE G KLIN PAEDIATR 197 481 985 62 NAZER H TRANS R SOC TROP MED HYG 80 348 986 PN 75153 RN 00319 AN 76076929 AU Antonowicz-I. Ishida-S. Shwachman-H. TI Studies in meconium: disaccharidase activities in meconium from cystic fibrosis patients and controls. SO Pediatrics. 1975 Nov. 56(5). P 782-7. MJ CYSTIC-FIBROSIS: en. DISACCHARIDASES: an. MECONIUM: en. MN CYSTIC-FIBROSIS: di. HUMAN. INFANT-NEWBORN. SUPPORT-U-S-GOVT-P-H-S. AB Meconium ileus represents the earliest clinical manifestation of cystic fibrosis. The differences found in the composition and amount of protein, mucoprotein, mucopolysaccharides, and reducing sugars in meconium from newborns with cystic fibrosis might be of significance relative to the pathogenesis and early diagnosis of this disease. We studied the enzymatic activity of disaccharidases (lactase, sucrase, maltase, and palatinase) in meconium of infants with cystic fibrosis and controls. We found an increase in the specific activity of these enzymes in the meconium from infants with cystic fibrosis as compared to the specific activity in meconium from normal infants. The increase in the activities, expressed as micromols per gram of protein per minute was: lactase, 100 times higher; sucrase, 18 times; maltase, 4.8 times, and palatinase, 8.9 times. RF 001 BUCHANAN DJ PEDIATRICS 9 304 952 002 GREEN MN PEDIATRICS 21 635 958 003 SCHACHTER H CAN J BIOCHEM 43 381 965 004 GREEN MN PEDIATRICS 41 989 968 005 KNAUFF RE PROC SOC EXP BIOL MED 127 801 968 006 SCHUTT WH ARCH DIS CHILD 43 178 968 007 RULE AH PEDIATRICS 45 847 970 008 RULE AH PEDIATRICS 48 601 971 009 DONNISON AB PEDIATRICS 37 833 966 010 CAIN ARR ARCH DIS CHILD 47 131 972 011 STEPHAN U CF CLUB ABST 15 974 012 EGGERMONT E BIOL NEONATE 10 266 966 013 GRUNDIG CA ACTA BIOL MED GER 19 193 967 014 HERNANDEZ-JODRA M ENZYME 12 682 971 015 STEPHAN U CF CLUB ABST 15 974 016 SHWACHMAN H ANN NY ACAD SCI 93 600 962 017 DAHLQVIST A ANAL BIOCHEM 7 18 964 018 TOWNLEY RRW PEDIATRICS 36 911 965 019 LOWRY OH J BIOL CHEM 193 265 951 020 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 021 KOPITO L J PEDIATR 68 313 966 022 OPPENHEIMER EH ARCH PATHOL 96 149 973 023 CRANE RK IN: CODE CF 5 2535 968 024 HAEMMERLI UP IN: DOWLIN HF 7 966 025 AURICCHIO S PEDIATRICS 35 944 965 026 DAHLQVIST A CLIN SCI 30 517 966 027 SHEEHY TW AM J DIS CHILD 121 464 971 028 ANTONOWICZ I GASTROENTEROLOGY 67 51 974 029 KARLSON P INTRODUCTION TO MODERN BIOCHE 314 968 030 SIEGAL S NONPARAMETRIC STATISTICS 956 031 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 032 NAGEL W PHYSIOL CHEM 340 1 965 033 HADORN B LANCET 1 812 969 CT 1 ANTONOWICZ I LANCET 1 746 976 2 GRAND RJ GASTROENTEROLOGY 70 790 976 3 ANTONOWICZ I BIOL NEONATE 32 280 977 4 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 5 RYLEY HC J CHROMATOGR 143 411 977 6 ANTONOWICZ I BIOL NEONATE 34 225 978 7 HARRIES JT BR MED BULL 34 75 978 8 KARLSSON KA FEBS LETTERS 87 283 978 9 ANTONOWICZ I J PEDIATR 92 214 978 10 SHWACHMAN H AM J DIS CHILD 132 1112 978 11 RYLEY HC MONOGR PAEDIATR 10 151 979 12 MORIN PR CLIN GENET 18 217 980 13 BERRY HK AM J DIS CHILD 134 930 980 14 MASTELLA G RIV ITAL PEDIATR 7 581 981 15 LAMBOTTE C J GENET HUM 29 85 981 16 EVANS RT J CLIN PATHOL 34 911 981 17 RYLEY HC J CLIN PATHOL 34 179 981 18 LEBENTHAL E DIGESTION 23 39 982 19 POTIER M BIOL NEONATE 45 257 984 20 BROCK DJH BR J OBSTET GYNAECOL 91 449 984 21 KLEIJER WJ PRENAT DIAGN 5 135 985 22 SMITH T CELL TISSUE RES 242 197 985 PN 75154 RN 00320 AN 76076954 AU Jusko-W-J. Mosovich-L-L. Gerbracht-L-M. Mattar-M-E. Yaffe-S-J. TI Enhanced renal excretion of dicloxacillin in patients with cystic fibrosis. SO Pediatrics. 1975 Dec. 56(6). P 1038-44. MJ CYSTIC-FIBROSIS: dt. DICLOXACILLIN: ur. MN ADOLESCENCE. ADULT. BIOLOGICAL-AVAILABILITY. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: ur, bl. DICLOXACILLIN: tu, bl. FEMALE. HUMAN. KIDNEY: me. MALE. PROTEIN-BINDING. SUPPORT-U-S-GOVT-P-H-S. AB Single oral doses of 6.25 mg/kg of dicloxacillin suspension were given to ten cystic fibrosis (CF) patients and eight normal subjects. Peak serum concentrations and areas under the concentration versus time curves for dicloxacillin were variable and, on average, were 2 1/2 times lower in the CF patients. The time of occurrence of the peak serum concentration was similar in both groups and the total urinary recovery of dicloxacillin was normal or increased in the CF patients, suggesting that the intestinal absorption of the drug was unaffected by the disease. The low serum concentrations in the CF patients were caused by unusually high renal clearances of dicloxacillin which average 282 +/- 135 compared to 95 +/- 28 ml/min/1.73 sq m in the normal subjects. Creatinine clearances were also elevated in the CF patients by 55% on average, while urea clearances were normal. The serum protein binding of dicloxacillin was similar in both groups of subjects. Because the rapid excretion results in low and variable serum concentrations of the antibiotic, treatment of CF patients with dicloxacillin may warrant use of increased or more frequent doses and monitoring of serum antibiotic levels. RF 001 LOWE CU IN: BARNETT HL 413 972 002 HARRIS R ARCH DIS CHILD 30 424 955 003 DEREN JJ N ENGL J MED 288 949 973 004 WEBER AM N ENGL J MED 289 1001 973 005 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 970 006 BENNETT JV APPL MICROBIOL 14 170 966 007 HEINEGARD D CLIN CHIM ACTA 43 305 973 008 CROCKER CL AM J MED TECHNOLOGY 33 361 967 009 JUSKO WJ J PHARM SCI 58 57 969 010 BENNETT JV J LAB CLIN MED 66 721 965 011 STROBER W PEDIATRICS 43 416 969 012 WILSON GB CLIN CHIM ACTA 49 79 973 013 DOLUISIO JT ANTIMICROB AGENTS CHEMOTHER 9 49 969 014 DITTERT LW ANTIMICROB AGENTS CHEMOTHER 9 42 969 015 ROSENBLATT JE ARCH INTERN MED 121 345 968 016 JUSKO WJ J PHARM SCI 61 1270 972 017 AGUSTA VE J UROL 110 113 973 018 RENNICK BR AM J PHYSIOL 212 1131 967 019 MOGENSEN CE DIABETES 22 706 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 YAFFE SJ ANN NY ACAD SCI 281 90 976 3 YAFFE SJ J INFECT DIS 135 828 977 4 SOUICH PD CLIN PHARMACOKINET 3 257 978 5 GUGGENBICHLER JP MONOGR PAEDIATR 10 34 979 6 BERGAN T ARZNEIMITTEL FORSCH DRUG RES 29-2 1955 979 7 BRYSON YJ J PEDIATR 94 673 979 8 FINKELSTEIN E J PEDIATR 94 163 979 9 SOLDIN SJ THER DRUG MONITOR 2 417 980 10 PRINCE AS J PEDIATR 97 148 980 11 BAGGOT JD J AM VET MED ASSOC 176 1085 980 12 MICHALSEN H ANTIMICROB AGENTS CHEMOTHER 19 1029 981 13 STRANDVIK B LANCET 1 800 982 14 BINS JW BR J CLIN PHARMACOL 14 P611 982 15 SOLDIN SJ ADV CHROMATOGR 20 139 982 16 NEU HC MED CLIN NORTH AM 66 51 982 17 KEARNS GL J PEDIATR 100 312 982 18 EICHENWALD HF PEDIATR PHARMACOL 3 181 983 19 PADOAN R DRUGS UNDER EXP CLIN RES 9 661 983 20 HUANG NN J ANTIMICROB CHEMOTHER 11 205 983 21 KAMPF D J ANTIMICROB CHEMOTHER 11 25 983 22 ASSAEL BM J ANTIMICROB CHEMOTHER 12 341 983 23 KERCSMAR CM J ANTIMICROB CHEMOTHER 12 289 983 24 LEFF RD PEDIATR CLIN NORTH AM 30 93 983 25 ARVIDSSON A ACTA PAEDIATR SCAND 72 293 983 26 THIRUMOORTHI MC J PEDIATR 102 941 983 27 MACDONALD NE J PEDIATR 103 985 983 28 PADOAN R J PEDIATR 103 320 983 29 ISLES A AM REV RESPIR DIS 127 417 983 30 NAHATA MC DEVELOP PHARM THER 7 221 984 31 MARTINI N J CLIN HOSP PHARM 9 303 984 32 TURNER A J ANTIMICROB CHEMOTHER 14 521 984 33 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 34 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 35 BOSSO JA ANTIMICROB AGENTS CHEMOTHER 25 630 984 36 REED MD ANTIMICROB AGENTS CHEMOTHER 25 579 984 37 LEEDER JS CLIN PHARMACOL THER 36 355 984 38 REED MD J PEDIATR 104 303 984 39 SPINO M J PEDIATR 105 829 984 40 ANON LANCET 1 1020 985 41 KUHN RJ CLIN PHARMACY 4 555 985 42 WOOLF RA CLIN PHARMACY 4 664 985 43 HARRISON CJ PEDIATR PHARMACOL 5 7 985 44 PRANDOTA J DEVELOP PHARM THER 8 311 985 45 REED MD ANTIMICROB AGENTS CHEMOTHER 27 583 985 46 BLUMER JL AM J MED 79 37 985 47 HORREVORTS AM CHEST 88 260 985 48 JACOBS RF J PEDIATR 106 1001 985 49 WILSON CB J PEDIATR 106 1049 985 50 SPINO M J PEDIATR 107 64 985 51 REED MD PEDIATR PULMONOL 2 282 986 52 ASSAEL BM INT J PEDIATR NEPHROL 7 213 986 53 MICHALSEN H METH FIND EXP CLIN PHARMACOL 8 685 986 54 MICHALSEN H METH FIND EXP CLIN PHARMACOL 8 727 986 55 BENDER SW INFECTION 14 17 986 56 BRAGANZA JM MED HYPOTHESES 20 233 986 57 WRIGHT DB DRUG INTEL CLIN PHARM 20 845 986 58 GOLDFARB J J CLIN PHARMACOL 26 222 986 59 LEBEL M ANTIMICROB AGENTS CHEMOTHER 30 260 986 60 LITTLEWOOD JM J ROY SOC MED 79 55 986 61 BLUMER JL J PEDIATR 108 854 986 62 KEARNS GL J PEDIATR 108 847 986 PN 75155 RN 00321 AN 75083184 AU Dietzsch-H-J. Gottschalk-B. Heyne-K. Leupoid-W. Wunderlich-P. TI Cystic fibrosis: comparison of two mucolytic drugs for inhalation treatment (acetylcysteine and arginine hydrochloride). SO Pediatrics. 1975 Jan. 55(1). P 96-100. MJ ACETYLCYSTEINE: ad. ARGININE: ad. CYSTIC-FIBROSIS: dt. EXPECTORANTS: ad. MN ACETYLCYSTEINE: tu. AEROSOLS. ARGININE: tu. BRONCHOSCOPY. CARBON-DIOXIDE: bl. CHILD. CHILD-PRESCHOOL. COUGH. CYSTIC-FIBROSIS: pp. DRUG-EVALUATION. EXPECTORANTS: tu. FEMALE. HUMAN. MALE. MUCUS: se. OXYGEN: bl. RADIONUCLIDE-IMAGING. RESPIRATION. RESPIRATORY-FUNCTION-TESTS. SPIROMETRY. SPUTUM: mi. AB Clinical, bronchoscopic, spirographic, scintigraphic, and chemical analyses were done in 24 children with cystic fibrosis to assess the mucolytic effects of acetylcysteine inhalations versus L-arginine hydrochloride aerosols. The latter drug is less active than acetylcysteine and should not be used to treat children with cystic fibrosis. RF 001 WEBB WR POSTGRAD MED 36 449 964 002 DENTON R AM REV RESPIR DIS 95 643 967 003 DIETZSCH HJ BRONCHES 20 383 970 004 DIETZSCH HJ Z ERKR ATMUNGSORGANE 134 445 971 005 DIETZSCH HJ MEDICAMENTUM 13 299 972 006 GOTTSCHALK B DTSCH GESUNDHEITSW 25 700 970 007 GOTTSCHALK B DTSCH GESUNDHEITSW 27 1121 972 008 HUANG NN GUIDE TO DRUG THERAPY IN PATI 972 009 MILLER WF ARCH INTERN MED 131 148 973 010 REAS HW J PEDIATR 65 542 964 011 SHEFFNER AL BIOCHEM PHARMACOL 15 1523 966 012 WEBB WR J THORAC CARDIOVASC SURG 44 330 962 013 WEBB WR CLIN MED 71 1531 964 014 LEUPOLD W DTSCH GESUNDHEITSW 25 1400 970 015 MATTHEWS LW MINN MED 52 1483 969 016 DIETZSCH HJ DTSCH GESUNDHEITSW 28 842 973 017 SOLOMONS CC PEDIATRICS 47 384 971 018 DIETZSCH HJ PAEDIATR GRENZGEB 6 195 967 CT 1 DIETZSCH HJ PEDIATRICS 57 166 976 2 SOLOMONS C PEDIATRICS 57 166 976 3 RICHARDSON PS PHARMACOL THER (B) 3 441 978 4 TABACHNIK E AM REV RESPIR DIS 122 97 980 5 WANNER A AM REV RESPIR DIS 122 79 980 PN 75156 RN 00322 AN 75157451 AU Oppenheimer-E-H. Esterly-J-R. TI Pathology of cystic fibrosis review of the literature and comparison with 146 autopsied cases. SO Perspect-Pediatr-Pathol. 1975. 2. P 241-78. MJ CYSTIC-FIBROSIS: pa. MN AUTOPSY. CARDIOVASCULAR-SYSTEM: pa. CYSTIC-FIBROSIS: co. EAR: pa. GALLBLADDER: pa. GENITALIA-FEMALE: pa. GENITALIA-MALE: pa. HUMAN. INTESTINES: pa. KIDNEY: pa. LIVER: pa. LUNG: pa. MALABSORPTION-SYNDROMES: et, pa. NOSE: pa. PANCREAS: pa. PHARYNX: pa. SWEAT-GLANDS: pa. EX This review of pathologic findings in cystic fibrosis includes a survey of morphologic changes from the original descriptions of CF and the numerous subsequent reports, and an analysis of 146 cases autopsied at the Johns Hopkins Hospital and Billings Hospital. Achylia occurs in 85 to 90% of patients. Approximately 1 of 7 patients with CF presents with meconium ileus. In 1962 we described gallbladder abnormalities in 24 of 72 cases of CF (33%). In Anderson's original description of CF, hemosiderosis was present in nearly every liver, and fatty infiltration was a frequent finding. The respiratory tract is invariably involved in CF. The heart has never been considered a primary target organ in CF. Bodian described a higher incidence of nephrocalcinosis in CF than in routine pediatric autopsies (31% compared to 11%). With the increased longevity of patients with CF male sterility has become a recognized complication. Increased electrolyte concentrations in sweat are the most diagnostic manifestation of CF, although an occasional patient has escaped this defect. Nasal polyps are frequent and often recurrent problems in patients with CF. Malabsorption due to pancreatic exocrine deficiency was originally described as the main manifestation of CF. The autopsy diagnosis of CF is usually based on a combination of lesions rather than on the changes in a single organ. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 LANDSTEINER K ZENTRALBL ALLG PATHOL 16 903 905 003 FARBER S ARCH PATHOL 37 238 944 004 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 005 KESSLER WR PEDIATRICS 8 648 951 006 DI SANTAGNESE PA PEDIATRICS 12 549 953 007 DANKS DM ANN HUM GENET 28 323 965 008 HARRIS RL PEDIATRICS 41 733 968 009 OPPENHEIMER EH PEDIATRICS 42 547 968 010 REDDY CR J TROP MED HYG 73 59 970 011 ARTHAUD JB ARCH PATHOL 90 433 970 012 KULCZYCKI LL AM J DIS CHILD 127 64 974 013 DI SANTAGNESE PA N ENGL J MED 277 1287 967 014 SPOCK A PEDIATR RES 1 173 967 015 BOWMAN BH SCIENCE 164 325 969 016 DANES BS J EXP MED 129 775 969 017 REED GB J PATHOL 101 251 970 018 MANGOS JA PEDIATR RES 2 378 968 019 GIBSON LE PEDIATRICS 48 695 971 020 GRAND RJ CLIN PEDIATR 9 588 970 021 DI SANTAGNESE PA PEDIATRICS 15 683 955 022 OPPENHEIMER EH ARCH PATHOL 96 149 973 023 OPPENHEIMER EH IN: MCKUSICK VA 7 108 972 024 ALLEN RA AM J PATHOL 31 337 955 025 KORNBLITH BA AM J PATHOL 5 249 929 026 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 027 WANG CI CF CLUB ABST 968 028 OPPENHEIMER EH JOHNS HOPKINS MED J 131 351 972 029 LARSSON Y PEDIATRICS 21 893 958 030 BROWN RE ARCH PATHOL 92 53 971 031 HANDWERGER S N ENGL J MED 281 451 969 032 KOPEL FB GASTROENTEROLOGY 62 483 972 033 HOLSCLAW DS AM J DIS CHILD 109 101 965 034 SHWACHMAN H N ENGL J MED 286 1300 972 035 DONNISON AB PEDIATRICS 37 833 966 036 THOMAIDIS TS J PEDIATR 63 444 963 037 PARKINS RA LANCET 2 851 963 038 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 111 1 962 039 BERNSTEIN JG AM J DIS CHILD 99 804 960 040 LOUW JH LANCET 2 1065 955 041 SANTULLI TV ANN SURG 154 939 961 042 HOLSCLAW DS PEDIATRICS 48 51 971 043 KISSANE JM PATHOLOGY OF INFANCY AND CHIL 165 965 044 BERDON WE N ENGL J MED 277 585 967 045 SHWACHMAN H AM J DIS CHILD 91 223 956 046 RICKHAM PP AM J SURG 109 173 965 047 HUNTON DB GASTROENTEROLOGY 50 99 966 048 MULLINS F JAMA 192 741 965 049 LLOYD-STILL JD BR MED J 1 110 971 050 KULCZYCKI LL N ENGL J MED 259 409 958 051 BARBERO GJ PEDIATRICS 29 788 962 052 BOLANDE RP ARCH PATHOL 95 172 973 053 WARWICK WJ PEDIATRICS 34 621 964 054 SWENEY LR ARCH PATHOL 86 413 968 055 ALTERMAN K AM J DIS CHILD 101 210 961 056 MCPARTLIN JF ARCH DIS CHILD 47 207 972 057 ESTERLY JR BULL JOHNS HOPKINS HOSP 110 247 962 058 ROVSING H NORD MED 86 1136 971 059 VICHI GF RIV CLIN PEDIATR 82 239 969 063 CRAIG JM AM J DIS CHILD 93 357 957 064 LANDING B GAP CONF REP PATHOL OF CF 972 065 SHARP HL GAP CONF REP PATHOL OF CF 972 066 DI SANTAGNESE PA PEDIATRICS 18 387 956 067 ROBERTS WC AM J MED 32 324 962 069 TAYLOR WF AM J DIS CHILD 123 161 972 070 WENTWORTH P THORAX 23 582 968 071 ESTERLY JR THORAX 23 670 968 072 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 073 MELLINS RB PEDIATRICS 44 315 969 074 LEV R AM J PATHOL 46 23 965 075 ESTERLY JR J NATL CANCER INST 40 1 968 076 BOAT TF JAMA 209 1498 969 077 REID L IN: HUBBLE D 21 964 077A NADAS AS PEDIATRICS 10 319 952 077B GOLDRING RM J PEDIATR 65 501 964 077C BOWDEN DH AM J MED 38 226 965 078 MCGIVEN AR ARCH DIS CHILD 37 656 962 079 BARNES GL AUST PAEDIATR J 6 81 970 080 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 081 OPPENHEIMER EA AM J MED 48 637 970 083 MILUNSKY A PEDIATRICS 42 501 968 084 HOLMAN RL PEDIATRICS 24 34 959 085 OPPENHEIMER EH LAB INVEST 30 411 974 086 PAULSON DF J UROL 108 811 972 087 GHAZALI S ARCH DIS CHILD 48 291 973 088 KUHN JP RADIOLOGY 97 59 970 089 SPEAR GS NEPHRON 1 238 964 090 DENNING CR PEDIATRICS 41 7 968 091 KAPLAN E N ENGL J MED 279 65 968 092 OPPENHEIMER EH J PEDIATR 75 806 969 093 LANDING BH ARCH PATHOL 88 569 969 094 DI SANTAGNESE PA N ENGL J MED 279 103 968 095 VALMAN HB LANCET 2 566 969 096 JOCKIN H AM J PATHOL 62 22A 971 097 WANG CI AM J DIS CHILD 119 236 970 098 HOLSCLAW DS PEDIATRICS 48 442 971 099 RULE AH FERTIL STERIL 21 515 970 100 TAUSSIG LM LANCET 1 850 972 101 FEIGELSON J ARCH FR PEDIATR 26 937 969 102 FEIGELSON J LYON MED 226 839 971 103 TAUSSIG LM N ENGL J MED 287 586 972 104 BUMBALO TS PEDIATRICS 43 468 969 105 GRAND RJ JAMA 195 993 966 106 ROSENOW EC 3RD JAMA 203 227 968 107 OPPENHEIMER EA AM J OBSTET GYNECOL 108 673 970 108 OPPENHEIMER EH J PEDIATR 77 991 970 109 KOPITO LE FERTIL STERIL 24 512 973 110 WARD AM J CLIN PATHOL 25 119 972 111 GIBSON LE J PEDIATR 81 193 972 112 MACLEAN WC JR J PEDIATR 83 86 973 113 MUNGER BL J PEDIATR 59 497 961 114$ BARTMAN J J CLIN PATHOL 45 455 966 115 GIBBS GE J INVEST DERMATOL 51 200 968 116 ESTERLY NB AM J DIS CHILD 123 200 972 117 KOPITO L PEDIATR RES 6 427 972 118 KOPITO L N ENGL J MED 272 504 965 119 LEONARD PJ ARCH DIS CHILD 47 495 972 120 TAYLOR BW ARCH DIS CHILD 49 133 974 121 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 122 HENKIN RI SCIENCE 138 1107 962 123 PETERSEN RA AM J DIS CHILD 116 662 968 124 KEATING JP PEDIATRICS 46 41 970 125 BOTELHO SY J PEDIATR 83 601 973 126 FOLLIS RH JR PATHOLOGY OF NUTRITIONAL 163 948 127 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 128 BLANC WA PEDIATRICS 22 494 958 129 KERNER I AM J DIS CHILD 99 597 960 130 BOREL DM ARCH PATHOL 96 269 973 131 SIMOPOULOS AP CF CLUB ABST 13 971 132 PARK EA ARCH DIS CHILD 29 269 954 133 TORSTENSON OL PEDIATRICS 45 857 970 134 WALTERS TR AM J DIS CHILD 124 641 972 135 KOMP DM CHEST 58 501 970 136 ESTERLY JR J PEDIATR 69 3 966 137 OPPENHEIMER EH COMPAR PATHOL BULL 111 3 971 138 PENTSCHEW A AM J PATHOL 66 11A 972 139 OLSON C ADV VET SCI 13 101 969 CT 1 SANTAGNESE PAD N ENGL J MED 295 481 976 2 COHN D MED J AUST 1 101 977 3 TOS M J LARYNGOL OTOL 91 827 977 4 SCHWACHMAN H N ENGL J MED 296 1519 977 5 NEUTRA MR GASTROENTEROLOGY 75 701 978 6 VITULLO BB J PEDIATR 93 1060 978 7 VAUGHAN WJ SCIENCE 199 783 978 8 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 9 SWAN HAP S AFR J SURG 17 51 979 10 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 11 SANTAGNESE PAD AM J MED 66 121 979 12 LLOYDSTILL JD AM J CLIN PATHOL 71 444 979 13 IMRIE JR AM J PATHOL 95 697 979 14 MISCHLER EH AM J DIS CHILD 133 632 979 15 FINBERG L N ENGL J MED 300 420 979 16 HOLSCLAW DS CLIN CHEST MED 1 407 980 17 ARBORGH B SCAND J GASTROENTEROL 15 73 980 18 LOPEZCORELLA E PATOLOGIA (MEXICO CITY) 18 167 980 19 WILLI UV AM J ROENTGENOL 134 1005 980 20 BLUSTEIN PK PEDIATRICS 68 387 981 21 LAMBERT JR GASTROENTEROLOGY 80 169 981 22 PARK RW GASTROENTEROLOGY 81 1143 981 23 HULTBERG B CLIN CHIM ACTA 112 167 981 24 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 25 COLTEN HR N ENGL J MED 304 831 981 26 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 27 ABDULKARIM FW GASTROENTEROLOGY 82 758 982 28 CHOW CW EUR J PEDIATR 139 240 982 29 GUIDOTTI TL AM J MED SCI 283 157 982 30 SHAWKER TH J ULTRASOUND MED 2 439 983 31 MUKAI K PATHOL ANNU 18 87 983 32 JEFFREY I J CLIN PATHOL 36 1292 983 33 WERLIN SL EXP MOL PATH 39 24 983 34 GUIDOTTI TL RESPIRATION 44 351 983 35 BASS S GASTROENTEROLOGY 84 1592 983 36 HOLMES R J PEDIATR 103 491 983 37 DANEMAN A AM J ROENTGENOL 141 653 983 38 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 39 PHELAN P ARCH DIS CHILD 59 71 984 40 BRACHLOW M FORTSCHR GEB RONTG NUKL 140 145 984 41 MICHALSEN H PATHOL RES PRACT 178 261 984 42 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 43 HUBBARD VS SEM RESPIR MED 6 299 985 44 SWOBODNIK W J CLIN ULTRASOUND 13 469 985 45 ABRAMOWSKY C HUM PATHOL 16 1243 985 46 HIBON D ANN RADIOL (PARIS) 28 469 985 47 SZEIFERT GT CLIN GENET 28 561 985 48 AKIMOTO N HIROSHIMA J MED SCI 34 189 985 49 GRAHAM N CLIN RADIOL 36 199 985 50 EISENBURG J FORTSCHR MED 103 133 985 51 ROSENBLUM JL J PEDIATR 107 149 985 52 FIEDOREK SC CLIN PEDIATR 25 243 986 53 LIU P J CAN ASSOC RADIOL 37 279 986 54 RUBINSTEIN S PEDIATRICS 78 473 986 55 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 56 MCGLENNEN RC ARCH PATHOL LAB MED 110 879 986 57 ROSENSTEIN BJ AM J DIS CHILD 140 966 986 58 HERNANZSCHULMAN M RADIOLOGY 158 629 986 59 VARMA RR SEM LIVER DIS 7 59 987 60 MCHUGO JM BR J RADIOL 60 137 987 PN 75157 RN 00323 AN 76012241 AU Tecklin-J-S. Holsclaw-D-S. TI Evaluation of bronchial drainage in patients with cystic fibrosis. SO Phys-Ther. 1975 Oct. 55(10). P 1081-4. MJ BRONCHI. CYSTIC-FIBROSIS: su. DRAINAGE. MN ADOLESCENCE. ADULT. BRONCHIAL-SPASM: pp. CHILD. CYSTIC-FIBROSIS: pp. HUMAN. LUNG: pp. RESPIRATORY-FUNCTION-TESTS. SUPPORT-U-S-GOVT-P-H-S. AB Examined in this study are the results of six tests of pulmonary function immediately preceding and following bronchial drainage in twenty-six patients with cystic fibrosis. Highly significant increases averaging 5.67, 4.13, 13.47, and 6.98 percent occurred in peak expiratory flow rate, forced vital capacity, expiratory reserve volume, and inspiratory capacity respectively. Significant increases in peak expiratory flow rate, forced vital capacity, and inspiratory capacity were observed in a subgroup of six of the above patients who had evidence of bronchospasm. The authors conclude that bronchial drainage will produce significant increases in routine pulmonary function values. The results suggest that this treatment is most effective in clearing the larger, more proximal, airways and is of benefit even in the presence of clinical bronchospasm. RF 001 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 002 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 003 SPOCK A PEDIATR RES 1 173 967 004 DOYLE B PHYS THER REV 39 24 959 005 JACKSON CD AM J MED SCI 186 849 933 006 BUSHNELL GE AM REV TUBERC 2 259 918 007 KNIES PT PHYS THER REV 18 239 938 008 MAY E PHYS THER REV 32 121 952 009 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 973 010 THACKER EW POSTURAL DRAINAGE AND RESPIRA 971 011 ZAUSMER E PHYS THER 48 586 968 012 ANTHONISEN P ACTA MED SCAND 175 715 964 013 MARCH H ARCH PHYS MED REHABIL 52 528 971 014 KANG B J ALLERGY CLIN IMMUNOL 53 2 974 015 HUBER AL J ALLERGY CLIN IMMUNOL 53 109 974 016 LORIN MI AM J PHYS MED 50 215 971 017 MOTOYAMA EK IN: MANGOS JA 335 973 018 POLGAR G PULMONARY FUNCTION TESTING IN 971 019 BRYANT EC STATISTICAL ANALYSIS 966 020 MACKLEM PT ARCH ENVIRON HEALTH 14 5 967 021 REAS HW J PEDIATR 65 556 964 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 TECKLIN JS AM REV RESPIR DIS 114 1038 976 3 FRYDMAN MI J CHRON DIS 32 211 979 4 FELDMAN J AM REV RESPIR DIS 119 239 979 5 MURRAY JF N ENGL J MED 300 1155 979 6 HOLSCLAW DS CLIN CHEST MED 1 407 980 7 WELLER PH RESPIRATION 40 53 980 8 MACKENZIE CF ANESTH ANALG CLEVE 59 207 980 9 ROCHESTER DF AM REV RESPIR DIS 122 133 980 10 MALONEY FP ARCH PHYS MED REHABIL 62 452 981 11 HOLODY B AM REV RESPIR DIS 124 372 981 12 MALONEY FP ARCH PHYS MED REHABIL 63 423 982 13 SUTTON PP EUR J RESPIR DIS 63 188 982 14 REDDING GJ AM REV RESPIR DIS 126 31 982 15 DESMOND KJ J PEDIATR 103 538 983 16 FALK M EUR J RESPIR DIS 65 423 984 17 MARINI JJ AM REV RESPIR DIS 129 101 984 18 ANDERSON J BR J DIS CHEST 79 272 985 19 MOHSENIFAR Z CHEST 87 483 985 20 KIRILOFF LH CHEST 88 436 985 21 MAZZOCCO MC CHEST 88 360 985 22 VERBOON JML EUR J RESPIR DIS 69 169 986 23 CURRIE DC BR J DIS CHEST 80 249 986 24 WEBBER BA BR J DIS CHEST 80 353 986 PN 75158 RN 00324 AN 75218113 AU Hertz-J. Cain-W-S. Bartoshuk-L-M. Dolan-T-F-Jr. TI Olfactory and taste sensitivity in children with cystic fibrosis. SO Physiol-Behav. 1975 Jan. 14(1). P 89-94. MJ CYSTIC-FIBROSIS: pp. SMELL. TASTE. MN ADAPTATION-PHYSIOLOGICAL. ADOLESCENCE. ADULT. ALCOHOLS-BUTYL. CHILD. DIFFERENTIAL-THRESHOLD. FEMALE. HUMAN. MALE. SODIUM-CHLORIDE. SUPPORT-U-S-GOVT-P-H-S. AB Psychophysical experiments measured both olfactory sensitivity to 1- butanol and sodium chloride and taste sensitivity to sodium chloride in normal children and in children with cystic fibrosis. The sensitivity of the children with cystic fibrosis fell within the normal range. These results stand in contrast to those of Henkin and Powell, who found children with cystic fibrosis to be hypersensitive to both olfactory and taste stimuli. Apparent hypersensitivity to taste stimuli could have resulted from the adapting effects of the relatively high level of sodium in the saliva of children with cystic fibrosis. If the contaminating effects of saliva are removed, children with cystic fibrosis yield, on the average, a taste threshold identical to that of normal children. There appears to be no simple explanation for why Henkin and Powell found hypersensitivity to odorants. The present results indicate that, in fact, children with cystic fibrosis display a slight hyposensitivity to odorants. RF 001 ALTMAN PL BLOOD AND OTHER BODY FLUIDS 961 002 BARTOSHUK LM PERCEPT PSYCHOPHYS 3 69 968 003 BARTOSHUK LM J COMP PHYSIOL PSYCHOL 87 310 974 004 BARTOSHUK LM SCIENCE 143 967 964 005 CAIN WS PERCEPT PSYCHOPHYS 9 478 971 006 CAIN WS ANN NY ACAD SCI 237 427 974 007 DAVIES JT BIOL BULL 117 222 959 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 008 DI SANTAGNESE PA N ENGL J MED 277 1344 967 009 FRAWLEY TF IN: MOTE JR 115 951 010 GREEN DM SIGNAL DETECTION THEORY AND P 966 011 HENKIN RI SCIENCE 138 1107 962 012 HENKIN RI J CLIN INVEST 42 727 963 013 MCBURNEY DH J GEN PHYSIOL 48 1145 965 014 MONCRIEFF RW ANN NY ACAD SCI 58 73 954 016 PFAFFMAN C J EXP PSYCHOL 65 523 963 017 WOTMAN S AM J DIS CHILD 108 372 964 CT 1 BARTOSHUK LM AM J CLIN NUTR 31 1068 978 2 SHERMAN AH OTOLARYNGOL HEAD NECK SURG 87 717 979 3 HYDE RJ J DENT RES 60 1730 981 4 COWART BJ PSYCHOL BULL 90 43 981 5 POLLACK MS IMMUNOGENETICS 15 579 982 6 CAIN WS YALE J BIOL MED 55 515 982 7 WEIFFENBACH JM CHEM SENSES 8 151 983 8 ESKENAZI B NEUROPSYCHOLOGIA 21 365 983 9 RUSSELL RM ANN INTERN MED 99 227 983 10 SCHIFFMAN SS N ENGL J MED 308 1275 983 11 WEIFFENBACH JM CHEM SENSES 9 193 984 12 WYSOCKI CJ PROC NAT ACAD SCI USA 81 4899 984 13 LAWLESS H J AM DIET ASSOC 85 577 985 PN 75159 RN 00325 AN 76012347 AU Holzel-A. TI The quest for the basic defect in cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 238-9. MJ CYSTIC-FIBROSIS: et. MN CYSTIC-FIBROSIS: fg. HUMAN. METABOLISM-INBORN-ERRORS: co. EX The search for the basic defect in cystic fibrosis has so far been singularly disappointing. There is no doubt that it is a disease recessively inherited, ie that the parents carry the cystic fibrosis gene without showing any signs of ill health (heterozygous) but when transmitted from both of them to an offspring this child manifests the disease, having acquired the abnormal gene in double dose (homozygote). Cystic fibrosis is the most common of the genetically determined diseases. Two types of cultured cells, fibroblasts and white cells from affected individuals and heterozygous carriers for cystic fibrosis, showed characteristic changes of metachromasia that seemed to allow for a classification of patients and carriers to be distributed into separate classes. Serum from patients with cystic fibrosis of the pancreas disorganises the movement of cilia of the isolated rabbit tracheal mucosa. One of the early and also most constant observations is the 'viscosity' of secretions of the mucosal glands in the respiratory tract, in the alimentary apparatus, in the biliary system and in the pancreas. A striking increase in the levels of sodium and chloride, and to a lesser extent of potassium, is present in the sweat of practically all sufferers of cystic fibrosis. However frustrating and tantalising the outcome of research into the basic defect has been so far, the ultimate answer is likely to be astonishing in it simplicity. RF 001 BARBERO GJ IN: PORTER R 32 968 002 BEARN AG IN: MANGOS JA 21 973 003 DANES BS IN: LAWSON D PROC 5TH INT CF 67 969 004 DANES BS J EXP MED 123 1 966 005 DI SANTAGNESE PA PEDIATRICS 12 549 953 006 MANGOS JA PEDIATR RES 1 436 967 007 SPOCK A PEDIATR RES 1 173 967 PN 75160 RN 00326 AN 76012350 AU Mearns-M-B. TI Inhalation therapy in cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 245-6. MJ CYSTIC-FIBROSIS: dt. RESPIRATORY-THERAPY. MN ACETYLCYSTEINE: ad. ALBUTEROL: ad. ANTIBIOTICS: ad. HUMAN. PENICILLINS: ad. SODIUM-CHLORIDE: ad. EX Inhalation therapy can be divided into two types, intermittent aerosol therapy which is used to administer medications over brief periods and mist tent therapy which is used for prolonged periods of treatment. The present state of knowledge of the basic defect of cystic fibrosis makes it impossible to give hard and fast guide lines for the use of inhalation therapy. Much depends on the individual experience of the physician and the degree of pulmonary involvement of the individual patient. Response to bronchodilators can be assessed relatively easily, assessment of mucolytics and antibiotics is difficult. Antibiotics can be useful in helping to clear infection in those patients with early disease, as judged by clearing of pathogens from the cough swab or sputum. In patients with chronic cough and large volumes if purulent sputum with positive culture from gram negative bacteria, the cultures never become negative and only in the occasional patient can definite clinical benefit be claimed. A few severely ill patients have dramatically improved when this therapy has been introduced, and the patients are convinced of the benefit of this treatment. It is of course impossible in this disease, with such a varying clinical course, to prove scientifically that the inhalation therapy was the reason for this improvement. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH PROC R SOC MED 42 25 949 003 ANDERSEN DH J CHRON DIS 7 58 958 004 BAU SK PEDIATRICS 48 605 971 005 CHANG N AM REV RESPIR DIS 107 672 973 006 DENTON R DIS CHEST 28 123 955 007 DOERSHUK CF PEDIATRICS 36 675 965 008 DOERSHUK CF PEDIATRICS 41 723 968 009 FARBER S J MICH MED SOC 44 587 945 010 MOTOYAMA EK PEDIATRICS 50 299 972 011 NORMAN AP IN: LAWSON D PROC 5TH INT CF 155 969 012 SHWACHMAN H PEDIATRICS 36 689 965 013 WOLFSDORF J PEDIATRICS 43 799 969 CT 1 MITCHELL EA AUST PAEDIATR J 18 40 982 PN 75161 RN 00327 AN 76012353 AU Gaskell-D. TI Physiotherapy for adolescents and adults with cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 250-2. MJ CYSTIC-FIBROSIS: th. PHYSICAL-THERAPY. MN ADOLESCENCE. ADULT. AEROSOLS. BREATHING-EXERCISES. CYSTIC-FIBROSIS: co. DRAINAGE. HEMOPTYSIS: co. HUMAN. PNEUMOTHORAX: co. POSTURE. EX Physiotherapy for adolescents and adults with cystic fibrosis is continued along the same lines as for younger patients, but certain modifications may be necessary to fit in with their changing needs. When patients are transferred from the care of the paediatrician to the thoracic physician it may be helpful for them to be admitted to hospital for reassessment. During this admission, the opportunity can be taken to review the daily physiotherapy regimen with the patients and their parents. Postural drainage, the use of aerosols, breathing exercises, long-term follow-up, complications (haemoptysis and spontaneous pneumothorax), the use of intermittent positive pressure breathing, and terminal care are discussed. RF 001 MATTHEWS LW J PEDIATR 65 558 964 002 PLUMSTEAD AJ PHYS THER 52 178 972 PN 75162 RN 00328 AN 76012348 AU Carter-C-O. TI Genetics and incidence of cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 240-2. MJ CYSTIC-FIBROSIS: oc. MN CHILD. CYSTIC-FIBROSIS: fg. EUROPE. FEMALE. GENE-FREQUENCY. GENES-RECESSIVE. GENETIC-COUNSELING. HUMAN. MALE. MUTATION. EX Cystic fibrosis is a genetically determined disorder. The evidence that cystic fibrosis is inherited in this way comes from several sources. The birth frequency of cystic fibrosis is unexpectedly high in Britain for such a serious illness. It is easy to calculate the proportion of the population who are carriers from the birth frequency of the disease. The explanation of the unlikely high birth frequency of cystic fibrosis in Europe is not known. An attempt to reduce substantially the birth frequency of cystic fibrosis will become possible once it is practicable to detect reliably the carrier of the gene. RF 001 HALL BD J MED GENET 5 262 968 CT 1 WARWICK WJ HELV PAEDIATR ACTA 33 117 978 2 POSSELT HG ATEMWEGS LUNGENKRANKH 10 345 984 PN 75163 RN 00329 AN 76012352 AU Hodge-G-J. TI Physiotherapy for children with cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 248-9. MJ CYSTIC-FIBROSIS: th. PHYSICAL-THERAPY. MN CHILD. CHILD-PRESCHOOL. COUGH: et. DRAINAGE. HUMAN. INFANT. INFANT-NEWBORN. POSTURE. RESPIRATORY-TRACT-INFECTIONS: pc. EX The importance of regular efficient postural drainage cannot be emphasised too strongly. Most parents of pre-school and primary school children are young enough to be physically able to play a major role in the routine physiotherapy treatment. The parents will require a great deal of support and encouragement from the physiotherapist, who must be ready to organise professional physiotherapy at crisis times in the family as well as in the event of an exacerbation. PN 75164 RN 00330 AN 76012349 AU Norman-A-P. TI Medical management of cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 242-4. MJ CYSTIC-FIBROSIS: th. MN CHILD. CHILD-PRESCHOOL. COUGH. DIET. DRAINAGE. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-ABSORPTION. INTESTINAL-OBSTRUCTION: su. LIVER-CIRRHOSIS: et. MECONIUM. PSYCHOLOGY. RESPIRATORY-TRACT-INFECTIONS: et. EX The manifestations of cystic fibrosis make it a complex disease, in the management of which the respiratory physician, the microbiologist, the gastroenterologist and last but not least, the psychologist, all are or should be involved. About 1 in 10 babies with cystic fibrosis are born with acute intestinal obstruction due to plugging of the small intestine with very sticky meconium. Diagnosis is made by the test for excess salt in the sweat, and although possible very soon after birth, it is easier to carry out at about three weeks of age when the baby sweats more freely. The great majority of children, if not treated, tend to suffer progressively more frequent chest infections or chronic chest infections. The reason for the tendency of the lungs of children with cystic fibrosis to become colonised by harmful bacteria is not known. Most cystic fibrosis children absorb carbohydrate normally, but only about 50% (range 45% to 80%) of the protein and fats they eat. The only other major problem for which any active measures may be required is the occurrence of fibrosis of the liver in about 5% of these children. Children with cystic fibrosis should be brought up from the beginning to be like other children. PN 75165 RN 00331 AN 76012351 AU Batten-J. TI Cystic fibrosis in adolescents and adults. SO Physiotherapy. 1975 Aug 10. 61(8). P 247-8. MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. COUGH: et. FEMALE. HUMAN. MALE. PHYSICAL-THERAPY. PNEUMOTHORAX: co. SPUTUM: an. EX With improvement in the treatment of children suffering from cystic fibrosis and increase in the average survival to about 17 years of age, many more patients with this disease are now coming under the care of thoracic physicians. In nearly every patient, broncho-pulmonary disease is the major problem. Pneumothorax is encountered in about 15% of the older patients and some of these occur bilaterally. A few patients develop refractory airways obstruction and then a trial of steroids is justifiable. Most patients with cystic fibrosis also require treatment for their pancreatic insufficiency. Diabetes mellitus is more common in cystic fibrosis than in the normal population. Analysis of sweat is essential for diagnosis. The psychological stresses imposed upon patients and their families are very great. PN 75166 RN 00332 AN 76012354 AU McCrae-W-M. TI Emotional problems in cystic fibrosis. SO Physiotherapy. 1975 Aug 10. 61(8). P 252-4. MJ CYSTIC-FIBROSIS: co. STRESS-PSYCHOLOGICAL. MN ADULT. CHILD. CYSTIC-FIBROSIS: fg. FAMILY. FEMALE. GENETIC-COUNSELING. HUMAN. PARENT-CHILD-RELATIONS. PROFESSIONAL-PATIENT-RELATIONS. EX The emotional distress associated with cystic fibrosis is considerable. From the moment of diagnosis, delayed or not, the parents' problems multiply and for the most part these problems are at first only vaguely comprehended. Many parents embark on the long haul of caring for their child in almost total ignorance of the disease and its implications. The fact that the disease is inherited is of outstanding importance to the parents. The basis of genetic counseling in the disease is the explanation of recessive inheritance. Even when genetic counseling has been given effectively and the parents have decided to have no further children, the family planning problem is not yet solved. The physiotherapist is also necessarily concerned with the emotional state and behaviour of the child. Except in the latest stages of this disease the child's emotional distress is not directly related to the discomfort of his symptoms. Over-protection by parents is understandable but it is important to persuade them that it is undesirable. Perhaps the most difficult questions of protection arise in relation to the child's knowledge of his own disorder. Cystic fibrosis is a miserable disease and the cause of much distress, emotional and otherwise. RF 001 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 973 002 BATTEN JC MEDICINE 1974 14 877 974 003 MEARNS MB BR J HOSP MED 12 497 974 004 PORTER R CIBA FOUND STUDY GROUP 32 968 CT 1 GOLDBERG RT ARCH PHYS MED REHABIL 60 369 979 PN 75167 RN 00333 AN 75196313 AU Holzel-A. TI Cystic fibrosis. SO Practitioner. 1975 Jun. 214(1284). P 776-85. MJ CYSTIC-FIBROSIS. MN ADOLESCENCE. ADULT. ANIMAL. CARBENICILLIN: tu. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: et, di, th. DIAGNOSIS-DIFFERENTIAL. GENTAMICINS: tu. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: et. LIPASE: tu. PEPTIDE-HYDROLASES: tu. PHYSICAL-THERAPY. PROGNOSIS. RABBITS. RATS. RESPIRATORY-TRACT-DISEASES: et. CELIAC-DISEASE: et. EX Cystic Fibrosis (fibrocystic disease of the pancreas) is a common inherited disease which, to date, still has a fatal outcome. The diversity of clinical manifestations and pathological findings has added further to the mysterious nature of the disease, as have the tantalizingly conflicting results of biochemical, physical and experimental studies in many laboratories of the western world. One of the most constant clinical observations is the viscosity of the secretion of the mucosal glands in the respiratory and alimentary tracts, in the ducts of the biliary system and in the pancreas. In approximately 10 to 15 per cent of all patients with cystic fibrosis of the pancreas the earliest manifestation, meconium ileus, occurs in the neonatal period. In the remaining 85 to 90 per cent of affected infants the symptoms generally become evident within the course of the first year of life. The lung symptoms tend to dominate the clinical picture in the vast majority of untreated patients and will ultimately decide the outcome. The lower respiratory tract is not the only part involved: nasal polyps and the accumulation of viscous secretions in the paranasal sinuses with subsequent bacterial infection are not unusual. Pancreatic deficiency is present in the vast majority of patients with cystic fibrosis and consists of a gross reduction or complete absence of exocrine secretion. Although some histological damage has been seen in the neonatal period in infants suffering from meconium ileus, in general the full-blown picture of extensive cirrhosis, with portal hypertension, hepatosplenomegaly, and severe and sometimes fatal haemorrhages from oesophageal varices, is seen in the older child and adolescent. Screening of the newborn to enable the institution of protective measures before lung complications arise has been attempted with various techniques. Management includes diet and pancreatic enzyme replacement. The treatment of pulmonary disease is the most important aspect of care in patients with cystic fibrosis, and its prevention is priority number one in the over-all management. The outlook for the child born with cystic fibrosis depends to a large extent upon the avoidance, or delay until adult life, of the two complications that threaten his existence: cor pulmonale and cirrhosis of the liver. RF 001 KULCZYCKI LL JAMA 175 358 961 002 MANGOS JA SCIENCE 158 135 967 003 MCCARTHY DS CLIN ALLERGY 1 261 971 004 MEARNS MB BR J HOSP MED 12 497 974 005 MEARNS MB LANCET 1 538 967 006 RACHELEFSKY GS AM J DIS CHILD 128 355 974 007 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 CT 1 HARTUNG J SOC BIOL 24 192 977 PN 75168 RN 00334 AN 76052758 AU Chamberlain-J. TI Screening for the early detection of diseases in Great Britain. SO Prev-Med. 1975 Sep. 4(3). P 268-81. MJ DIAGNOSTIC-SERVICES. MN AGED. BREAST-NEOPLASMS: pc. BRONCHITIS: pc. CERVIX-NEOPLASMS: pc. CHILD. CORONARY-DISEASE: pc. CYSTIC-FIBROSIS: oc. FEMALE. GLAUCOMA: pc. GREAT-BRITAIN. HUMAN. HYPERTENSION: pc. CHILD-HEALTH-SERVICES. INFANT. INFANT-NEWBORN. LUNG-NEOPLASMS: pc. MENTAL-DISORDERS: pc. MULTIPHASIC-SCREENING. OCCUPATIONAL-HEALTH-SERVICES. PHENYLKETONURIA: bl. PREGNANCY. PRENATAL-CARE. SCHOOL-HEALTH-SERVICES. STATE-MEDICINE. TUBERCULOSIS-PULMONARY: pc. AB An account is given of screening for the early detection of disease in Great Britain today. The organisation of screening programmes provided nationally for the whole population is described. These include programmes for prenatal care, screening of infants, screening of schoolchildren, and screening of women for carcinoma in situ of the uterine cervix. Selective screening programmes for tuberculosis and for various industrial diseases are mentioned. Experience in provision of these services on a national scale has emphasised the importance of evaluating future screening programmes, before they are implemented, by research studies on their effectiveness, their possibly harmful side effects, and their cost. Evidence from research in screening for a number of diseases is very briefly summarized, and it is concluded that there may be some development of programmes for the early detection and treatment of hypertension and of breast cancer, but for other diseases and for multiphasic screening, evidence so far available is insufficient to warrant any immediate extension from research towards service. RF 001 ALLMAN ST HEALTH TRENDS 6 39 974 002 BEST L IN: MCLACHLAN G 2 339 973 003 BRETT GZ THORAX 23 414 968 004 BRIMBLECOMBE FSW LANCET 2 1428 973 005 BUTLER NR PERINATAL MORTALITY 963 007 CHAMBERLAIN J PROC R SOC MED 66 888 973 008$ COCHRANE AL IN: SCREENING IN MEDICAL CARE 81 968 009 DAVIE R FROM BIRTH TO SEVEN 972 010 ELWOOD PC BR MED J 3 254 970 011 GOLDBERG DP DETECTION OF PSYCHIATRIC ILLN 972 013 KEEN H PROC INT SYMP ON ATHE 2ND 435 970 014 ANON BR MED J 1 1317 966 015 MORRIS JN AM J MED 46 674 969 016 OSBORN GR LANCET 1 256 966 017 RAMCHARAN S PREV MED 2 207 973 018 ROSE GA TRANS SOC OCCUP MED 20 109 970 019 SHAPIRO S PROC NAT CANCER CONF 7TH 663 973 020 ANON INT J EPIDEMIOL 2 219 973 021 ANON RAPP 9 972 022 ANON HEALTH OF THE SCHOOL CHIL 972 023 TREVELYAN H PREV MED 2 278 973 024 ANON JAMA 213 1143 970 025 WILLIAMSON J LANCET 1 1117 964 026$ WILSON JMG IN: SCREENING IN MEDICAL CARE 15 968 CT 1 HARMAN D J AM GERIATR SOC 24 452 976 2 SCHWARTZ FW MED KLIN 71 1248 976 3 KRISTEIN MM SCIENCE 195 457 977 4 WERNER M CLIN CHEM 25 509 979 PN 75169 RN 00335 AN 75120418 AU Siwinska-Golebiowska-H. TI Studies on immunoglobulins in healthy children in various stages of development and in the chosen illnesses. SO Probl-Med-Wieku-Rozwoj. 1975. 4. P 207-14. MJ ATAXIA-TELANGIECTASIA: im. CYSTIC-FIBROSIS: im. DWARFISM-PITUITARY: im. IMMUNOGLOBULINS: bi. PHENYLKETONURIA: im. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. HUMAN. IGA: bi. IGG: bi. IGM: bi. IMMUNOELECTROPHORESIS. INFANT. INFANT-NEWBORN. AB The studies were carried out with view to evaluate development of immunoglobulins biosynthesis in children from birth through first year of life and up to age of 15 years as well as to establish the normal values of each immunoglobulin class in healthy children of different age in our conditions of investigations and in our geografical, social and economical situation. There was also under consideration the evaluation of body reaction on antigenic stimulation and administration of gammaglobulin preparations. In order to determine more broadly the development of these humoral immunity parameters in children qualitative and quantitative investigations of immunoglobulins were undertaken also in same chosen illnesses where changes in biosynthesis and metabolism of immunoglobulins may be connected with pathogenesis of illness or with type of treatment (cystic fibrosis) (C.F.), Ataxia-Telangiectasia syndrome (A-T), pituitary dwarfism, fenyloketonuria (PKU). In some groups of children investigations were systematically repeated to evaluate the dynamism of changes. RF 001 ANON CLIN EXP IMMUNOL 9 695 971 002 GOLEBIOWSKA H CLIN EXP IMMUNOL 2 275 967 003 GOLEBIOWSKA H ACTA HAEMATOL POL 1 363 970 PN 75170 RN 00336 AN 75217882 AU Farrell-P-M. Pallavicini-J-C. Ulane-M-M. TI Growth characteristics and protein content of tissue-cultured fibroblasts from cystic fibrosis patients. SO Proc-Soc-Exp-Biol-Med. 1975 Jun. 149(2). P 340-3. MJ CELL-DIVISION. CYSTIC-FIBROSIS: me. DNA: an. PROTEINS: an. MN BIOPSY. CELL-COUNT. CYSTIC-FIBROSIS: pa. FIBROBLASTS: ph, me, an. GROWTH. HUMAN. PROTEINS: bi. SKIN: me. TISSUE-CULTURE. AB Proliferation rates and cellular protein content have been measured in cultured fibroblasts derived from the skin of normal volunteers and cystic fibrosis patients. Three methods of measuring growth indicated that under our conditions, CF fibroblasts divide normally with a mean doubling time of 29 hr. During the logarithmic growth phase, however, lower cell protein/DNA ratios were observed consistently in CF cultures. This difference was not present in contact-inhibited, confluent fibroblasts. The finding of an apparent reduction in protein synthesis during rapid division, coupled with an observation by others that CF fibroblasts fail to normally induce collagen formation, suggests the possibility of a disturbance in the biochemical regulation of protein synthesis. RF 001 SEEGMILLER JE SCIENCE 155 1682 967 002 NEUFELD EF SCIENCE 169 141 970 003 DANES BS J EXP MED 129 775 969 004 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 005 PALLAVICINI JC J PEDIATR 77 280 970 006 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 007 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 008 HOUCK JC CF CLUB ABST 15 51 974 009 JACOBSON CB CF CLUB ABST 15 23 974 010 BURTON K BIOCHEM J 62 315 956 011 LOWRY OH J BIOL CHEM 193 265 951 012 TODARO GJ J CELL COMP PHYSIOL 62 257 963 013 RUBIN H IN: DEFENDI V 51 967 014 FLETCHER DS CLIN CHIM ACTA 44 5 973 015 MELLMAN WJ IN: ROTHBLAT GH 1 327 972 016 RAFF EC J CELL PHYSIOL 74 235 969 017 WIEBEL F J CELL PHYSIOL 74 191 969 018 TEMIN HM IN: ROTHBLAT GH 1 49 972 CT 1 WARD JB TEX REP BIOL MED 34 11 976 2 ANON J PEDIATR 88 711 976 3 DISANTAGNESE PA N ENGL J MED 295 534 976 4 LUNDGREN DW J PEDIATR 90 1034 977 5 OWEN E J MOL MED 3 49 978 6 HOSLI P ACTA PAEDIATR SCAND 67 617 978 7 SHAPIRO BL CLIN CHIM ACTA 82 125 978 8 FEIGAL RJ PEDIATR RES 13 764 979 9 SHAPIRO BL SCIENCE 203 1251 979 10 DAVIS PB PEDIATR RES 14 863 980 11 GAHL WA PEDIATR RES 14 118 980 12 LIMBOSCH S CLIN CHIM ACTA 102 11 980 13 JAKEL HP BIOL ZENTRALBL 100 273 981 14 LIEDTKE C N ENGL J MED 304 975 981 15 THOMPSON KVA GERONTOLOGY 29 97 983 PN 75171 RN 00337 AN 75196736 AU Shapiro-B-L. Pence-T-V. Warwick-W-J. Smith-Q-T. TI Insulin iontophoresis in cystic fibrosis. SO Proc-Soc-Exp-Biol-Med. 1975 Jul. 149(3). P 592-3. MJ CYSTIC-FIBROSIS: me. INSULIN: me. IONTOPHORESIS. MN CHLORIDES: an. CYSTIC-FIBROSIS: fg, pp. HETEROZYGOTE. HUMAN. INSULIN: ad. PILOCARPINE: me, ad. SWEAT: an. AB Insulin and its vehicle without insulin were administered separately by iontophoresis to patients with cystic fibrosis (CF), obligate heterozygotes, and healthy controls. The resultant sweat chloride concentration after treatment with both preparations was compared in each individual. No difference after the two treatments was found in the control sample. A decrease in sweat chloride concentration after insulin iontophoresis in comparison with the vehicle was observed in both the CF (P smaller than 0.005) and heterozygote (P smaller than 0.01) samples. These observations suggest an involvement of insulin in CF and a possible role of insulin in sweat gland function. RF 001 CAUDILL M LANCET 2 307 973 002 LOBECK CC IN: STANBURY JB 1605 972 003 KAISER D PEDIATR RES 5 167 971 004 MANGOS JA PEDIATR RES 2 378 968 005 MILNER AD ARCH DIS CHILD 44 351 969 006 HANDWERGER S N ENGL J MED 281 451 969 007 STAHL M J PEDIATR 84 821 974 008 GOODCHILD MC ARCH DIS CHILD 47 152 972 009 GIBSON LE PEDIATRICS 23 545 959 010 HANSEN L MINN MED 50 1191 967 011 SHAPIRO BL BIOCHEM BIOPHYS RES COMMUN 39 816 970 012 BROWN GA LANCET 2 639 971 013 ZIERLER KL AM J MED 40 735 966 014 KAISER D IN: MANGOS JA 247 973 015 SWEENEY EA VOX SANG 13 54 967 017 TOMIZAWA HH J BIOL CHEM 240 3191 965 018 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 019 SHAPIRO BL LANCET 2 1020 974 CT 1 BANK S AM J DIG DIS 23 178 978 2 GLASS JM INT J DERMATOL 19 519 980 3 SLOAN JB J AM ACAD DERMATOL 15 671 986 4 KARI B DIABETES 35 217 986 PN 75172 RN 00338 AN 76079679 AU Samuels-C-E. Robinson-P-G. Elliott-R-B. TI Decreased inhibition of platelet aggregation by PGE1 in children with cystic fibrosis and their parents. SO Prostaglandins. 1975 Oct. 10(4). P 617-21. MJ CYSTIC-FIBROSIS: bl. PLATELET-AGGREGATION: de. PROSTAGLANDINS-E: pd. MN ADENOSINE-DIPHOSPHATE: pd. ADULT. CARRIER-STATE: bl. CHILD. CLINICAL-TRIALS. CYSTIC-FIBROSIS: di. DEPRESSION-CHEMICAL. HUMAN. PROSTAGLANDINS-E: du. AB PGE1 inhibited ADP-induced platelet aggregation in children with cystic fibrosis and their parents to a much lesser extent than in normal controls. We suggest that this may be a reliable test for heterozygote carriers of cystic fibrosis. RF 001 KLOEZE JE PROC NOBEL SYMP 2ND 241 966 002 KINLOUGH-RATHBONE RL BR J HAEMATOL 19 559 970 003 SMITH JB J CLIN INVEST 52 965 973 004 SCHOENE NW PROSTAGLANDINS 5 387 974 005 CLAUSEN J LIPIDS 7 246 972 006 VAN DORP D ANN NY ACAD SCI 180 181 971 007 VINCENT JE PROSTAGLANDINS 5 369 974 008 KUO PT J PEDIATR 60 394 962 CT 1 ELLIOTT RB PEDIATRICS 57 474 976 2 ROSENLUND ML PEDIATRICS 59 428 977 3 MANCUSO G RIV ITAL PEDIATR 8 665 982 4 DAVIS PB J LAB CLIN MED 104 203 984 5 STEAD RJ PROSTAGLANDINS LEUKOTR MED 26 91 987 PN 75173 RN 00339 AN 76032664 AU Griscom-N-T. Capitanio-M-A. Wagoner-M-L. Culham-G. Morris-L. TI The visibly fatty liver. SO Radiology. 1975 Nov. 117(2). P 385-9. MJ FATTY-LIVER: ra. MN CASE-REPORT. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FATTY-LIVER: co. HUMAN. INFANT. LIVER-CIRRHOSIS: co. MALE. TOMOGRAPHY-X-RAY. AB Fatty infiltration of the liver has been identified roentgenographically in several young children. Awareness of this possibility will sometimes allow a radiologist to contribute to the understanding of the patient's metabolic and nutritional state. If a child's liver is shown to be abnormally radiolucent, cystic fibrosis should be considered. RF 001 AMBROSE J PROC R SOC MED 66 833 973 004 FLETCHER K AM J CLIN NUTR 19 170 966 005 ISSELBACHER KJ IN: SCHIFF L 672 969 007 LOMBARDI B LAB INVEST 15 1 966 008 MELHEM RE PROC EUR SOC PED RAD ANN MTG 975 009 NEW PFJ RADIOLOGY 110 109 974 010 STEINBACH HL RADIOLOGY 62 858 954 012 SWISCHUK LE AM J ROENTG RAD THER NUCL MED 122 159 974 014 TER-POGOSSIAN MM RADIOLOGY 113 515 974 015 WOLF BS IN: MARGULIS AR 233 967 CT 1 SWISCHUK LE J PEDIATR 88 452 976 2 GIBSON B PEDIATRICS 59 778 977 3 FAURE C ARCH FR PEDIATR 35 887 978 4 WORLEY L J PEDIATR 94 1005 979 5 YOUSEFZADEH DK RADIOLOGY 131 351 979 6 MINDELZUN R RADIOL CLIN NORTH AM 18 221 980 7 ZAMMIT AA BR J RADIOL 53 1018 980 8 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 9 SCATARIGE JC J ULTRASOUND MED 3 9 984 PN 75174 RN 00340 AN 75120914 AU Fellows-K-E. Stigol-L. Shuster-S. Khaw-K-T. Shwachman-H. TI Selective bronchial arteriography in patients with cystic fibrosis and massive hemoptysis. SO Radiology. 1975 Mar. 114(3). P 551-6. MJ ANGIOGRAPHY. BRONCHIAL-ARTERIES: ra. CYSTIC-FIBROSIS: ra. HEMOPTYSIS: ra. MN ADOLESCENCE. ADULT. BRONCHIAL-ARTERIES: su. BRONCHOSCOPY. CYSTIC-FIBROSIS: di, pa. FEMALE. HEMOPTYSIS: di, pa. HUMAN. MALE. PULMONARY-ARTERY: su. PULMONARY-CIRCULATION. SUPPORT-U-S-GOVT-P-H-S. AB Massive hemoptysis is a potentially fatal complication of long- standing cystic fibrosis. Lobectomy may prevent a hemorrhagic death if the hemorrhage source can be identified and if involvement of the remaining lung tissue is mild. Selective bronchial arteriography was performed in six patients with cystic fibrosis to localize a lobar source of bleeding. Arteriographic findings correlated with bronchoscopic observations. Bronchial arteriography may be helpful when bronchoscopy cannot be done because of continuous hemorrhage or because the severity of the lung disease precludes general anesthesia, but it is not an adequate substitute for bronchoscopy in most patients. RF 001 BOTENGA ASJ AM J ROENTG RAD THER NUCL MED 104 829 968 002 BOTENGA ASJ RADIOL CLIN 38 309 969 003 EARL GM JR AM REV RESPIR DIS 106 898 972 004 FEIGELSON HH RADIOLOGY 85 663 965 005 HAROUTUNIAN LM CHEST 62 587 972 006 HOLSCLAW DS J PEDIATR 76 829 970 007 LIEBOW AA AM J PATHOL 25 211 949 008 MACK JF BR J RADIOL 38 422 965 009 MOSS AJ PEDIATRICS 41 438 968 010 NEWTON TH RADIOLOGY 84 1043 965 011 NORTH LB AM J ROENTG RAD THER NUCL MED 107 328 969 012 SCHUSTER SR J THORAC CARDIOVASC SURG 48 750 964 013 SHWACHMAN H AM J DIS CHILD 96 6 958 015 WAGENVOORT CA PATHOLOGY OF PULMONARY VASCUL 964 016 WENTWORTH P THORAX 23 582 968 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 SCHUSTER SR J PEDIATR SURG 12 889 977 3 SHWACHMAN H MEDICINE 56 129 977 4 BOOKSTEIN JJ CHEST 72 658 977 5 REMY J RADIOLOGY 122 33 977 6 WOLFE JD WEST J MED 127 383 977 7 HARLEY JD AM J ROENTGENOL 128 302 977 8 WEBB WR AM J ROENTGENOL 129 233 977 9 SELECKY PA CHEST 73 741 978 10 BREDIN CP AM REV RESPIR DIS 117 969 978 11 STERN RC AM REV RESPIR DIS 117 825 978 12 JONES RSW CATHET CARDIOVASC DIAGN 5 145 979 13 BABO HV RADIOLOGE 19 506 979 14 LAMARQUE JL RADIOLOGE 19 514 979 15 CULHAM JAG PEDIATR CLIN NORTH AM 26 661 979 16 SWERSKY RB ANN THORAC SURG 27 262 979 17 ASPELIN P SCAND J RESPIR DIS 60 20 979 18 SANTAGNESE PAD AM J MED 66 121 979 19 WOOD RE SOUTH MED J 72 189 979 20 HARGROVE WC CHEST 76 234 979 21 FELLOWS KE J PEDIATR 95 959 979 22 SENAC JP ANN CHIR 34 542 980 23 FAIRFAX AJ BR J DIS CHEST 74 345 980 24 WISEMAN NE J PEDIATR SURG 16 457 981 25 REES S CLIN RADIOL 32 1 981 26 FELLOWS KE RADIOLOGY 140 249 981 27 MOSS AJ PEDIATRICS 70 728 982 28 KELLER FS CHEST 81 407 982 29 VUJIC I RADIOLOGY 143 19 982 30 GONG H RESPIRATION 44 225 983 31 PORTER DK J THORAC CARDIOVASC SURG 86 409 983 32 PORTER DK ARCH INTERN MED 143 287 983 33 SAPIRE DW CLIN CARDIOL 7 673 984 34 LESTER LA SEM RESPIR MED 6 285 985 35 IMGRUND SP CRIT CARE MED 13 438 985 36 STANKIEWICZ JA EAR NOSE THROAT J 64 180 985 37 OSADA H ANN THORAC SURG 41 440 986 PN 75175 RN 00341 AN 75121373 AU Haluszka-J. Scislicki-A. TI Bronchial lability in children suffering from some diseases of the bronchi. SO Respiration. 1975. 32(3). P 217-26. MJ BRONCHI: pp. BRONCHITIS: pp. CYSTIC-FIBROSIS: pp. RESPIRATION. MN AIRWAY-RESISTANCE: de. CHILD. COMPARATIVE-STUDY. HISTAMINE: du. HUMAN. PLETHYSMOGRAPHY-WHOLE-BODY. RESPIRATORY-FUNCTION-TESTS. AB The authors studied bronchial reactivity in children with cystic fibrosis and with deformative bronchitis. The reaction of the bronchi was studied both by inhalatory and exercise provocation. Inhalatory tests were monitored by means of a whole body plethysmograph. Free- range run was used for the exercise test, and the peak expiratory flow rate was measured. The described technique of inhalatory tests was accurate, safe, and not tiring for the patient. No distinct difference in bronchial reaction between the two groups of patients could be stated. The bronchial reaction to the inhalatory and exercise tests was stronger in both groups than in normals but not as strong as in asthmatics. The nature of bronchial hyperreactivity in those patients is still unknown. The inhalatory test seems to be more sensitive than the exercise test in detecting that hyperreactivity. RF 001 ANDERSON SD THORAX 26 396 971 002 CADE JF LANCET 2 186 971 003 CONNOLLY NM J ASTHMA RES 8 31 970 005 GAVALOV S PROC EWGCF 4TH ANNU MTG 61 973 006 HEIMLICH EM J ALLERGY CLIN IMMUNOL 37 103 966 007 JONES RS BR J DIS CHEST 56 78 962 008 KONIG P ARCH DIS CHILD 47 578 972 009 MASTELLA G PROC EWGCF 4TH ANNU MTG 51 973 010 SILVERMAN M ARCH DIS CHILD 47 882 972 011 WILLIAMS HE BR MED J 4 321 969 CT 1 VARPELA E CLIN ALLERGY 8 273 978 2 HALUSZKA J ARCH IMMUNOL THER EXP 26 731 978 3 MELLIS CM PEDIATRICS 61 446 978 4 LEUPOLD W MONOGR PAEDIATR 10 119 979 5 TOBIN MJ THORAX 35 807 980 6 DAVIS PB N ENGL J MED 302 1453 980 7 HOLZER FJ ARCH DIS CHILD 56 455 981 8 LEMANSKE RF AM REV RESPIR DIS 123 622 981 9 GEORGITIS JW ANN ALLERGY 48 175 982 10 GOTZ M WIEN KLIN WOCHENSCHR 94 3 982 11 DAVIS PB J CHRON DIS 36 269 983 12 DAVIS PB AM REV RESPIR DIS 129 911 984 13 DAVIS PB SEM RESPIR MED 6 261 985 14 HORDVIK NL AM REV RESPIR DIS 131 889 985 15 DARGA LL PEDIATR PULMONOL 2 82 986 16 DAVIS PB HORM METAB RES 18 217 986 17 BERDEL D KLIN PAEDIATR 198 71 986 PN 75176 RN 00342 AN 75219305 AU Super-M. TI Cystic fibrosis in the South West African Afrikaner. An example of population drift, possibly with heterozygote advantage. SO S-Afr-Med-J. 1975 May 10. 49(20). P 818-20. MJ CAUCASOID-RACE. CYSTIC-FIBROSIS: oc. GENE-FREQUENCY. HETEROZYGOTE. MN AFRICA-SOUTHERN. CHILD. CYSTIC-FIBROSIS: fg, di. HUMAN. INFANT. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: di. INTESTINAL-OBSTRUCTION: di. MALE. MASS-SCREENING. MECONIUM. AB An incidence of the genetic recessive disease cystic fibrosis (mucoviscidosis) far in excess of that reported recently from other countries, has been encountered in the South West African Afrikaner. This has probably resulted from the immigration of a segment of the South African Afrikaner population rich in the gene, into South West Africa, where, for religious reasons and reasons of geographical isolation, the gene has persisted and, perhaps, increased in frequency. Malaria, which killed many of the early settlers, might have selectively spared carriers of the gene, thus enriching its occurrence in the population. Details of patients, particularly with regard to the criteria of diagnosis, are given with the relevant population and birth figures, from which an estimate of the incidence of the disease and of its carrier rate has been made. Screening of the newborn for the condition and the compiling of a register of potential and obligatory carriers are also discussed. RF 001 GOODMAN HO AM J HUM GENET 4 59 952 002 STEINBERG AG AM J HUM GENET 12 416 960 003 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 004 HONEYMAN MS AM J HUM GENET 17 461 965 005 DANKS DM ANN HUM GENET 28 323 965 006 PUGH RJ ARCH DIS CHILD 42 544 967 007 HALL BD J MED GENET 5 262 968 008 SELANDER P ACTA PAEDIATR SCAND 51 65 962 009 CARTER CO ABC OF MEDICAL GENETICS 969 010 HSIA DYY INBORN ERRORS OF METABOLISM 959 011 GEORGE L ARCH DIS CHILD 46 139 971 012 DI SANTAGNESE PA AM J MED 15 777 953 013 TAUSSIG LM N ENGL J MED 287 586 972 014 SORSBY A TRANS R SOC MED HYG 57 15 963 015 DEAN G S AFR MED J 34 745 964 016 MYRIANTHOPOULOS NC AM J HUM GENET 24 341 972 017 VEDDER H SOUTH WEST AFRICA IN EARLY TI 420 966 018 CAIN ARR ARCH DIS CHILD 47 131 972 CT 1 BEIGHTON P S AFR MED J 50 1125 976 2 SUPER M S AFR MED J 50 238 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 SUPER M LANCET 2 1288 977 5 TENKATE LP INT J EPIDEMIOL 6 23 977 6 SUPER M S AFR MED J 52 390 977 7 SCHAAP T ISR J MED SCI 14 201 978 8 WARWICK WJ HELV PAEDIATR ACTA 33 117 978 9 RETIEF AE S AFR MED J 54 849 978 10 SUPER M S AFR MED J 54 18 978 11 SUPER M CLIN GENET 16 65 979 12 JAKEL HP BIOL ZENTRALBL 98 55 979 13 HOLLANDER DH MED HYPOTHESES 8 191 982 14 BOTHA MC S AFR MED J 64 609 983 15 KOESLAG JH S AFR MED J 66 87 984 16 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 17 RUBIO TT AM J MED 81 73 986 18 LIVINGSTONE FB HUM BIOL 59 59 987 PN 75177 RN 00343 AN 75219297 TI Editorial: Cystic fibrosis. SO S-Afr-Med-J. 1975 May 10. 49(20). P 800-1. MJ CYSTIC-FIBROSIS: fg. MN AFRICA-SOUTHERN. CYSTIC-FIBROSIS: oc. HUMAN. MALE. SELECTION-GENETICS. EX Cystic fibrosis, sometimes known as mucoviscidosis, is a generalised disorder of the exocrine glands which affects children, adolescents and young adults. No biochemical abnormality may be regarded as the primary cause, although at various times it has been suggested that the defect lies in ion transport, in glycoprotein structure or in membrane permeability. Cystic fibrosis is inherited as a Mendelian recessive, and is the commonest of all such genetic diseases in the Caucasoid populations of Western Europe and North America. Super has detected and documented a frequency of the disease among Caucasoids in South West Africa which is considerably higher than that found anywhere else in the world. An investigation of the ways in which the disease in South West Africa may differ, clinically or pathologically, from that found elsewhere, is necessary. RF 001 SUPER M S AFR MED J 49 818 975 PN 75178 RN 00344 AN 75159629 AU Skude-G. TI Electrophoretic separation, detection, and variation of amylase isoenzymes. SO Scand-J-Clin-Lab-Invest. 1975 Jan. 35(1). P 41-47. MJ AMYLASES: an. ISOENZYMES: an. MN ACUTE-DISEASE. AMYLASES: bl. CHRONIC-DISEASE. CYSTIC-FIBROSIS: en. ELECTROPHORESIS-AGAR-GEL. HUMAN. PANCREATITIS: en. SALIVA: en. STAINS-AND-STAINING. AB An electrophoretic technique for demonstrating amylase isoenzymes is described. After separation in an agarose gel containing a linear polyacrylamide polymer to reduce electroendosmotic flow, the amylase fractions are visualized by incubation with a commercially available dye-starch polymer (Phadebas Amylase Test). The technique detects amylase fractions with activities below 10 U/l. Some characteristic changes in such diseases as acute and chronic pancreatitis, cystic fibrosis of the pancreas, macroamylasemia and inherited variants as well as after maxillofacial surgery are mentioned. RF 001 AFONSE E CLIN CHIM ACTA 14 195 966 002 AW SE NATURE 209 298 966 003 AW SE GUT 8 402 967 004 BAKER RWR SCAND J CLIN LAB INVEST 6 94 954 005 DAVIES TJ J CLIN PATHOL 25 266 972 006 GOETZ H CLIN CHIM ACTA 19 235 968 007 GOT R CLIN CHIM ACTA 22 545 968 008 HILL C NATURE 235 162 972 009 HOBBS JR IN: DUBACK UC 281 968 010 JOHANSSON BG SCAND J CLIN LAB INVEST SUPPL 124 7 972 012 KAMARYT J HUMANGENETIK 1 579 965 013 MEITES S CLIN CHEM 14 1176 968 014 MEITES S CRC CRIT REV CLIN LAB SCI 2 103 971 015 MERRITT AD NATURE NEW BIOL 239 243 972 016 MUUS J NATURE 204 283 964 017 NORBY S EXP CELL RES 36 663 964 018 OGITA S MED J OSAKA UNIV 16 271 966 019 PIMSTONE NR CLIN CHEM 10 891 964 020 ROSALKI SB J CLIN PATHOL 23 373 970 021$ SCHIWARA HW ARZTL LAB ? 19 38 973 022 SCHIWARA HW Z KLIN CHEM KLIN BIOCHEM 11 319 973 023 SKUDE G HEREDITAS 65 277 970 025 SKUDE G SCAND J PLAST RECONSTR SURG 7 105 973 026 SOMOGYI M CLIN CHEM 6 23 960 027 SPIEKERMAN AM CLIN CHEM 20 324 974 028 WIEME RJ IN: DUBACK UC 293 968 029 WILDING P CLIN CHIM ACTA 12 97 965 030 WOLF RO NATURE 213 1128 967 CT 1 ODONNEL MD BIOL GASTROENTEROL 8 354 975 2 SKUDE G SCAND J GASTROENTEROL 10 577 975 3 SKUDE G SCAND J GASTROENTEROL 11 21 976 4 SKUDE G SCAND J GASTROENTEROL 11 17 976 5 SKUDE G SCAND J GASTROENTEROL 11 525 976 6 SKUDE G GUT 17 127 976 7 SKUDE G ACTA PAEDIATR SCAND 65 145 976 8 SKUDE G AM J OBSTET GYNECOL 126 652 976 9 HAUBRICH J ARCH OTO RHINO LARYNGOL 213 1 976 10 AMADOR E ACS SYMPOSIUM SERIES 1976 175 976 11 MERRITT AD ADV HUM GENET 8 135 977 12 SCHIOTZ PO CLIN GENET 11 43 977 13 DONALDSON LA SCAND J GASTROENTEROL 12 637 977 14 IHSE I SCAND J GASTROENTEROL 12 629 977 15 MAGID E SCAND J GASTROENTEROL 12 621 977 16 SKUDE G SCAND J GASTROENTEROL 12 53 977 17 SKUDE G SCAND J GASTROENTEROL 12 3 977 18 SKUDE G SCAND J GASTROENTEROL 12 673 977 19 TAKACS O ACTA PAEDIATR ACAD SCI HUNG 18 21 977 20 SKUDE G ACTA MED SCAND 201 53 977 21 BIRKHED D SCAND J DENT RES 86 248 978 22 JENSEN SL AM J PHYSIOL 235 E381 978 23 BORULF S SCAND J GASTROENTEROL 14 151 979 24 KANNO T J JAPAN SOC STARCH SCI 26 96 979 25 NAEIJE R ACTA OBSTET GYNECOL SCAND 58 531 979 26 JAKEL HP BIOL ZENTRALBL 98 55 979 27 MINAIRE Y LYON MED 241 213 979 28 MAGID E J CLIN CHEM CLIN BIOCHEM 18 669 980 29 SKUDE G J CLIN CHEM CLIN BIOCHEM 18 680 980 30 FAHRENKRUG J CLIN CHEM 26 1573 980 31 JACOBSON G SCAND J CLIN LAB INVEST 40 77 980 32 HUETHER G HISTOCHEM J 13 207 981 33 SORENSEN S SCAND J GASTROENTEROL 16 885 981 34 DERIJKE D J CLIN CHEM CLIN BIOCHEM 19 815 981 35 BOSSUYT PJ CLIN CHEM 27 451 981 36 FAHRENKRUG J CLIN CHEM 27 1655 981 37 JONES JB J PEDIATR GASTROENTEROL NUTR 1 43 982 38 RAMMELOO T CLIN CHEM 28 145 982 39 LINDBERG T PEDIATRICS 70 235 982 40 KOLLBERG H ACTA PAEDIATR SCAND 71 321 982 41 MAGID E SCAND J RHEUMATOL 12 129 983 42 RASMUSSEN SN SCAND J GASTROENTEROL 18 353 983 43 NASRALLAH SM GUT 24 161 983 44 VENTRUCCI M DIGESTION 28 114 983 45 DERIJKE D CLIN CHEM 29 1100 983 46 ZIMMERMAN HM AM J GASTROENTEROL 78 575 983 47 ZIMMERMAN HM GASTROENTEROLOGY 85 190 983 48 HEGARDT P J PEDIATR GASTROENTEROL NUTR 3 563 984 49 KJAERGAARD JJ DIABETE METAB 10 25 984 50 KOOP H CLIN GASTROENTEROL 13 739 984 51 PANTEGHINI M RIC CLIN LAB 14 449 984 52 PAVESI F RIC CLIN LAB 14 443 984 53 BORGSTROM A SCAND J GASTROENTEROL 19 220 984 54 MASSON P J CLIN CHEM CLIN BIOCHEM 22 427 984 55 PARVIAINEN MT J CLIN CHEM CLIN BIOCHEM 22 41 984 56 BECCIOLINI A ACTA RADIOL ONCO RAD PHYS BIO 23 9 984 57 NILSSON PE ACTA RADIOL DIAGN STOCKH 25 395 984 58 OKABE H CLIN CHEM 30 1219 984 59 ORBAEK P SCAND J WORK ENVIRON HEALTH 11 1 985 60 JENSEN AR SCAND J GASTROENTEROL 20 83 985 61 KJAERGAARD J SCAND J GASTROENTEROL 20 848 985 62 ZAKOWSKI JJ CRC CRIT REV CLIN LAB SCI 21 283 985 63 GERBER M J CLIN CHEM CLIN BIOCHEM 23 587 985 64 GERBER M CLIN CHEM 31 1331 985 65 KAMMERAAT C CLIN CHEM 31 1078 985 66 MASSEY TH CLIN CHEM 31 70 985 67 URDAL P CLIN CHEM 31 699 985 68 ECKFELDT JH ARCH PATHOL LAB MED 109 316 985 69 HARMOINEN A J CLIN CHEM CLIN BIOCHEM 24 903 986 70 GORUS F CLIN CHEM 32 398 986 PN 75179 RN 00345 AN 76033530 AU Skude-G. TI Sources of the serum isoamylases and their normal range of variation with age. SO Scand-J-Gastroenterol. 1975. 10(6). P 577-84. MJ GLYCOSIDE-HYDROLASES: bl. ISOAMYLASE: bl. MN ADOLESCENCE. ADULT. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. FEMALE. FETUS: en. GENITALIA-FEMALE: en. HUMAN. INFANT. INFANT-NEWBORN. ISOAMYLASE: ur. KIDNEY: en. LIVER: en. LUNG: en. MALE. PANCREAS: en. SUBMANDIBULAR-GLAND: en. AB The isoamylases in various human tissue homogenates and body fluids were separated by agarose gel electrophoresis. Nothing suggested any significant production of amylase in the liver. Minute amounts of amylase belonging to the pancreatic group of isoamylases might be produced by the glands of the proximal duodenum. The specific group of isoamylases produced in the female genital tract could not be demonstrated in serum or urine. The activity of amylase in serum was derived from two groups of isoenzymes, one group originating from the salivary glands, the other from the pancreatic gland. The contribution of each of these two sources to the total serum amylase was determined from early foetal life to adult age. A very low activity of the salivary isoamylases was regularly found in serum from 14-week-old foetuses. The activity increased steadily with age and reached the normal adult level, about 80 U/l, at the age of 5 years. The pancreatic group of isoamylases in serum developed later; the majority of children below 3 months had no demonstrable pancreatic isoamylase activity. The activity rose slowly to reach adult level, about 80 U/l, at the age of 10 to 15 years. The activity did not vary with sex, and the diurnal variation of the isoamylase was negligible. In children with cystic fibrosis of the pancreas the activity of pancreatic isoamylases in serum was low. RF 001 AMMAN RW ANN INTERN MED 78 521 973 002 AW SE GUT 8 402 967 003 BELFIORE F CLIN CHEM 18 1403 972 004 BELFIORE F CLIN CHEM 19 387 973 005 FARBER S J CLIN INVEST 22 827 943 006 GOT R CLIN CHIM ACTA 22 545 968 007 GREEN CL AM J OBSTET GYNECOL 73 402 957 008 JANOWITZ HD AM J MED 27 924 959 009 KAMARYT J Z KLIN CHEM KLIN BIOCHEM 8 564 970 010 KUTTNER M PROC SOC EXP BIOL MED 32 564 934 011 LAXOVA R J MED GENET 9 321 972 012 LEVITT MD ANN INTERN MED 71 919 969 013 LILLIBRIDGE CB J PEDIATR 82 279 973 014 MCGEACHIN RL PROC SOC EXP BIOL MED 99 130 958 015 MAYER WB BULL JOHNS HOPKINS HOSP 44 246 929 016 MEITES S CRC CRIT REV CLIN LAB SCI 2 103 971 017 NICORY C BIOCHEM J 6 387 922 018 ROSSITER MA ACTA PAEDIATR SCAND 63 389 974 019 SEARCY RL PEDIATRICS 39 294 967 020 SKUDE G SCAND J CLIN LAB INVEST 35 41 975 021 SOMOGYI M ARCH INTERN MED 67 665 941 022 TAKEUCHI T CLIN CHIM ACTA 54 137 974 023 VACIKOVA A J CHROMATOGR 69 349 972 CT 1 SKUDE G SCAND J GASTROENTEROL 11 21 976 2 SKUDE G SCAND J GASTROENTEROL 11 525 976 3 SKUDE G GUT 17 127 976 4 SKUDE G ACTA PAEDIATR SCAND 65 145 976 5 SKUDE G AM J OBSTET GYNECOL 126 652 976 6 WARSHAW AL LANCET 1 929 977 7 MERRITT AD ADV HUM GENET 8 135 977 8 DONALDSON LA SCAND J GASTROENTEROL 12 637 977 9 MAGID E SCAND J GASTROENTEROL 12 621 977 10 SKUDE G SCAND J GASTROENTEROL 12 53 977 11 SKUDE G SCAND J GASTROENTEROL 12 3 977 12 WARSHAW AL J SURG RES 22 362 977 13 ODONNELL MD CLIN CHEM 23 560 977 14 WARSHAW AL J LAB CLIN MED 90 1 977 15 DAVIDSON GP PEDIATR RES 12 967 978 16 GILLARD BK PEDIATR RES 12 868 978 17 KAMARYT J J CLIN CHEM CLIN BIOCHEM 16 539 978 18 BERK JE AM J GASTROENTEROL 69 417 978 19 BERK JE GASTROENTEROLOGY 74 1313 978 20 KENNY D CLIN CHIM ACTA 89 429 978 21 HUBBARD VS J PEDIATR 92 685 978 22 FORSTNER GG PEDIATR RES 13 1077 979 23 DONALDSON LA GUT 20 216 979 24 STICKLE JE AM J VET RES 41 506 980 25 AGGETT PJ ARCH DIS CHILD 55 236 980 26 ODONNELL MD CLIN CHIM ACTA 104 265 980 27 KRISTENSEN BO BR MED J 281 978 980 28 HAGGMARK A GASTROENTEROL CLIN BIOL 5 927 981 29 ZANNINO L RIV ITAL PEDIATR 7 247 981 30 BOSSUYT PJ CLIN CHEM 27 451 981 31 FAHRENKRUG J CLIN CHEM 27 1655 981 32 JACOBSON G SCAND J GASTROENTEROL 17 833 982 33 VONEYBEN FE J UROL 128 1195 982 34 MOLLERPETERSEN J ACTA MED SCAND 211 459 982 35 HICKS JM BULL MOL BIOL MED 8 105 983 36 GILLARD BK CLIN CHEM 29 1119 983 37 JACOBS E CLIN CHEM 29 887 983 38 LEBENTHAL E J PEDIATR 102 1 983 39 KOOP H CLIN GASTROENTEROL 13 739 984 40 OKABE H CLIN CHEM 30 1219 984 41 MASIAR PJ NEOPLASMA 31 351 984 42 GUERRA EG REV INVEST CLIN 36 109 984 43 MORGENSTERN T MONATSSCHR KINDERHEILKD 132 661 984 44 HUBBARD VS SEM RESPIR MED 6 299 985 45 GIBERTINI P ITAL J GASTROENTEROL 17 211 985 46 CHO JW ACTA CHIR SCAND 151 323 985 47 FOO AY ANN CLIN BIOCHEM 23 624 986 48 FUKAYAMA M DIFFERENTIATION 31 127 986 49 TSIANOS EB HEPATOGASTROENTEROLOGY 33 247 986 50 TSIANOS EB J CLIN PATHOL 39 464 986 51 BARROS FP AM J GASTROENTEROL 81 261 986 52 FJOSNE U ACTA MED SCAND 219 301 986 PN 75180 RN 00346 AN 75198186 AU Hoiby-N. Wiik-A. TI Antibacterial precipitins and autoantibodies in serum of patients with cystic fibrosis. SO Scand-J-Respir-Dis. 1975 May. 56(1). P 38-46. MJ ANTIBODIES-BACTERIAL: an. AUTOANTIBODIES: an. CYSTIC-FIBROSIS: im. PRECIPITINS: an. RESPIRATORY-TRACT-INFECTIONS: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. STREPTOCOCCUS-PNEUMONIAE: im. FEMALE. FOLLOW-UP-STUDIES. HAEMOPHILUS-INFLUENZAE: im. HUMAN. IGG: an. INFANT. MALE. PSEUDOMONAS-AERUGINOSA: im. RHEUMATOID-FACTOR: an. STAPHYLOCOCCUS: im. AB Sera from 84 patients with cystic fibrosis, 31 patients with other respiratory diseases and 21 control persons have been investigated for the occurrence of antibacterial precipitins and autoantibodies. Preciptins were studied by means of crossed immunoelectrophoresis, antinuclear factors by an indirect immunofluorescence technique and rheumatoid factors by the latex fixation slide test. A very heterogeneous antibacterial immune response was found in patients with cystic fibrosis, notably as regards Ps. aeruginosa. None of the other patients or controls had precipitins against this bacterium. The occurrence of precipitins against St. aureus and D. pneumoniae were more frequent in cystic fibrosis patients as compared with controls, but not as compared with other respiratory disease patients. No significant differences were found as regards precipitins against H. influenzae or the occurrence of rheumatoid factors. Antinuclear factors were more frequent in cystic fibrosis patients than in the other two groups investigated. A pronounced and heterogeneous humoral immune response against Ps. aeruginosa in cystic fibrosis patients chronically infected with mucoid strains of this bacterium was found to be correlated with poor prognosis and the reason for this is discussed. RF 001 BARTFELD H ANN NY ACAD SCI 168 30 969 002 BONOMO L AM J CLIN PATHOL 45 313 966 003 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 004 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 005 DOGGETT RG INFECT IMMUN 6 628 972 006 GORDON DS AM REV RESPIR DIS 108 127 973 007 HALBERT SP MOD PROBL PEDIATR 10 144 967 008 HOFF GE SCAND J INFECT DIS 6 333 974 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 010 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 012 HOIBY N ACTA PAEDIATR SCAND 63 843 974 014 JOHNSON KJ J CLIN INVEST 54 349 974 015 KOFFLER D J EXP MED 134 169 971 016 MASS B AM REV RESPIR DIS 106 384 972 017 MAY JR ARCH DIS CHILD 47 908 972 018 MEARNS MB ARCH DIS CHILD 47 902 972 019 MUNTHE E CLIN EXP IMMUNOL 8 249 971 020 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 021 TORRIGIANI G LANCET 1 14 970 022 TURNER-WARWICK M BR MED J 3 492 970 023 WALLER M J INFECT DIS 125 45 972 024 WIIK A IMMUNOLOGY 23 53 972 025 WIIK A ANN RHEUM DIS 33 515 974 026 WILLIAMS RC JR J CLIN INVEST 41 666 962 027 WINCHESTER RJ CLIN EXP IMMUNOL 6 689 970 CT 1 AXELSON NH SCAND J IMMUNOL 5 177 976 2 WEEKE B DAN MED BULL 23 155 976 3 ANDERSEN V CLIN EXP IMMUNOL 26 469 976 4 HOIBY N SCAND J RESPIR DIS 57 37 976 5 HOIBY N SCAND J RESPIR DIS 57 103 976 6 WOOD RE AM REV RESPIR DIS 113 833 976 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 8 CLARKE CW CLIN ALLERGY 7 527 977 9 SORENSEN RU INFECT IMMUN 18 735 977 10 HOIBY N SCAND J RESPIR DIS 58 65 977 11 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 12 NIELSEN HE ACTA PAEDIATR SCAND 67 443 978 13 HILTON AM BR J DIS CHEST 72 207 978 14 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 15 SORENSEN RU J PEDIATR 93 201 978 16 HOIBY N ACTA PAEDIATR SCAND 68 495 979 17 WOOD RE SOUTH MED J 72 189 979 18 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 345 979 19 HOLSCLAW DS CLIN CHEST MED 1 407 980 20 WIIK A ALLERGY 35 263 980 21 MIELIVERGANI G ARCH DIS CHILD 55 696 980 22 SAGRANSKY DM AM J DIS CHILD 134 319 980 23 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 24 FEIGELSON J NOUV PRESSE MED 10 955 981 25 SORENSEN RU PEDIATR RES 15 14 981 26 SORENSEN RU AM REV RESPIR DIS 123 37 981 27 MOSS RB CLIN EXP IMMUNOL 47 301 982 28 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 29 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 30 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 31 KLINGER JD INFECT IMMUN 39 1377 983 32 COLLINS MT J CLIN MICROBIOL 19 757 984 33 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 34 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 35 OLIVER AM CLIN EXP IMMUNOL 59 190 985 36 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 37 PEDERSEN M SCAND J INFECT DIS 18 245 986 38 PHILLIPS BM J ROY SOC MED 79 44 986 39 BRETT MM ARCH DIS CHILD 62 357 987 PN 75181 RN 00347 AN 75141326 AU Brogan-T-D. Ryley-H-C. Neale-L. Yassa-J. TI Soluble proteins of bronchopulmonary secretions from patients with cystic fibrosis, asthma, and bronchitis. SO Thorax. 1975 Feb. 30(1). P 72-9. MJ ASTHMA: me. BLOOD-PROTEINS: an. BRONCHITIS: me. CYSTIC-FIBROSIS: me. SPUTUM: an. MN ADULT. AGED. ALBUMINS: an. BETA-GLOBULINS: an. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. GLYCOPROTEINS: an. HAPTOGLOBINS: an. HUMAN. IGA: an. IGG: an. IMMUNOELECTROPHORESIS. LACTOFERRIN: an. MALE. MIDDLE-AGE. MURAMIDASE: an. TRANSFERRIN: an. AB The concentrations of nine plasma proteins were determined by quantitative immunoelectrophoresis in sputum specimens from 29 patients with cystic fibrosis (CF) and from 24 patients with severe asthma and chronic bronchitis. The results suggested that the population of CF patients could be divided into two groups in spite of an absence of difference in clinical status between the groups. Average concentrations of seven plasma proteins in sputum of group I CF patients were identical with those in sputum of patients with bronchitis, but the average concentrations of six of these proteins in sputum from group II CF patients were higher than those in specimens from the bronchitic patients and were similar to corresponding concentrations in sputum from patients with asthma, all of whom were examined while in status asthmaticus. The average concentrations of 14 secretory proteins were the same in all sputum specimens whether or not they were produced by patients with cystic fibrosis, asthma or bronchitis. It was concluded that the concentrations in the bronchopulmonary secretions of proteins associated with host defence were not diminished in patients with cystic fibrosis, and failure to produce adequate concentrations of proteins with antimicrobial activity was unlikely to be responsible for the above average susceptibility to chest infection in cystic fibrosis. It is suggested that there exists a group of CF patients in whom a pulmonary allergic reaction generates an inflammatory response as severe as that characterizing status asthmaticus and that this response could be detrimental. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BROGAN TD IMMUNOLOGY 7 626 964 003 BURNS MW LANCET 1 270 968 004 CLARKE HGM CLIN SCI 35 403 968 005 COOPERMAN EM CAN MED ASSOC J 105 580 971 006 HEINER DC AM J DIS CHILD 103 634 962 007 LOPEZ M IMMUNOCHEMISTRY 6 513 969 008 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 009 MAY JR IN: OSWALD NC 958 010 ANON LANCET 1 775 965 011 OLITZKI L BACT REV 12 149 948 012 RYLEY HC BIOCHIM BIOPHYS ACTA 271 300 972 013 RYLEY HC BR J EXP PATHOL 49 625 968 014 RYLEY HC J CLIN PATHOL 26 852 973 015 SPOCK A PEDIATR RES 1 173 967 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 2 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 3 WEEKE B DAN MED BULL 23 155 976 4 CLARKE CW THORAX 31 702 976 5 LAINE A CLIN CHIM ACTA 67 159 976 6 SANTAGNESE PAD N ENGL J MED 295 481 976 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 8 BROGAN TD J ANTIMICROB CHEMOTHER 3 247 977 9 PROCTOR DF AM REV RESPIR DIS 115 315 977 10 RUDNIK J ARCH IMMUNOL THER EXP 26 717 978 11 LOPEZVIDRIERO MT BR MED BULL 34 63 978 12 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 13 LOPEZVIDRIERO MT AM REV RESPIR DIS 117 465 978 14 ROUSSEL P LUNG 154 241 978 15 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 16 LOPEZVIDRIERO MT THORAX 34 512 979 17 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 18 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 19 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 345 979 20 BROGAN TD THORAX 35 624 980 21 HARBITZ O EUR J RESPIR DIS 61 84 980 22 KOTLAR HK EUR J RESPIR DIS 61 233 980 23 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 24 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 25 LOPEZVIDRIERO MT CHEST 80 799 981 26 KONSTAN MW AM REV RESPIR DIS 123 120 981 27 EMMETT M J IMMUNOL METH 50 R 65 982 28 MOLLER NE EUR J RESPIR DIS 63 130 982 29 PEATFIELD AC AM REV RESPIR DIS 125 210 982 30 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 31 JACQUOT J BULL EUR PHYSIOPATH RESP 19 453 983 32 WILLIAMS IP THORAX 38 682 983 33 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 34 STELANDER M EUR J RESPIR DIS 65 5 984 35 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 36 JACQUOT J INFECT IMMUN 47 555 985 37 PERSSON CGA LANCET 2 1126 986 38 MOSS RB MONOGR ALLERGY 19 202 986 39 MOSS RB CHEST 91 522 987 PN 75182 RN 00348 AN 75199129 AU Ryland-D. Reid-L. TI The pulmonary circulation in cystic fibrosis. SO Thorax. 1975 Jun. 30(3). P 285-92. MJ CYSTIC-FIBROSIS: pp. PULMONARY-CIRCULATION. PULMONARY-HEART-DISEASE: pa. MN ADOLESCENCE. ADULT. BODY-HEIGHT. BODY-WEIGHT. CHILD. CHILD-PRESCHOOL. CYANOSIS. ELECTROCARDIOGRAPHY. HEART-VENTRICLE: pp. HEART: ra. HEMOGLOBINS: an. HUMAN. INFANT. INFANT-NEWBORN. LUNG-VOLUME-MEASUREMENTS. LUNG: pa, ra. MYOCARDIUM: pa. ORGAN-WEIGHT. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC. PSEUDOMONAS-INFECTIONS. PULMONARY-ARTERY: pa. PULMONARY-HEART-DISEASE: di. PULMONARY-VEINS: pa. AB The hearts and lungs of 36 patients dying of cystic fibrosis have been studied to establish the relationship between right ventricular hypertrophy (RVH), pathological changes in the pulmonary circulation, and the clinical features. The presence and degree of RVH were measured by Fulton's technique of weighing the ventricles separately. Of the subjects who died after the age of 3 years, one in six had no RVH, although the mean age of this group was not significantly different from that of the remainder. There was no correlation between duration of cyanosis, haemoglobin levels, and RVH. Although not statistically significant, the cases without RVH tended to have had clubbing of the finger-nails for longer periods before death. The electrocardiogram was useful in the diagnosis of RVH. Right ventricular hypertrophy was common in children with lungs of large volume as judged by the radiographic centile of lung length. Cases with RVH show reduced background haze in the arteriogram and fewer arteries per unit area of lung. For the first time thickening of the medial muscle layer proportional to the degree of RVH has been demonstrated, only in the smaller arteries. Arterial subintimal fibrosis is more common in cases with RVH, as is thickening of the walls of small pulmonary veins. The implications of these findings are discussed. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH PEDIATRICS 3 406 949 003 BOWDEN DH CIRCULATION 28 693 963 004 CAPLAN AH J PEDIATR 73 540 968 005 DAVIES GM IN: LAWSON D PROC 5TH INT CF 350 969 006 DAVIES G THORAX 25 669 970 007 DUNNILL MS THORAX 17 329 962 008 ELLIOTT FM THESIS 964 009 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 010 FULTON RM BR HEART J 14 413 952 011 GOLDRING RM J PEDIATR 65 501 964 012 HISLOP A THESIS 971 013 HISLOP A THORAX 25 682 970 014 KELMINSON LL PEDIATRICS 39 24 967 015 LAWSON D IN: LAWSON D PROC 5TH INT CF 225 969 016 MAY CD J PEDIATR 34 663 949 017 MEARNS MB ARCH DIS CHILD 47 902 972 018 MILLARD FJC THESIS 965 019 RECAVARREN S BR HEART J 26 187 964 020 ROYCE SW PEDIATRICS 8 255 951 021 SHWACHMAN H AM J DIS CHILD 96 6 958 022 SHINEBOURNE E MEDICINE 16 999 973 023 SIASSI B J PEDIATR 78 794 971 024 SIMON G ARCH DIS CHILD 47 373 972 025 SYMCHYCH PS ARCH PATHOL 92 409 971 026 TANNER JM ARCH DIS CHILD 41 454 966 027 WEIBEL ER J APPL PHYSIOL 17 343 962 028 WIGLESWORTH FW AM J MED SCI 212 351 946 029 ANON WHO TECH REP SER NO 213 961 CT 1 GAULTIER C SEM HOP PARIS 52 447 976 2 HISLOP A BR J EXP PATHOL 57 542 976 3 FISHMAN AP AM REV RESPIR DIS 114 775 976 4 HAWORTH SG BR HEART J 39 80 977 5 SHELTON DM CHEST 71 303 977 6 REID L AM J ROENTGENOL 129 777 977 7 POUKKULA A SCAND J RESPIR DIS 1977 7 977 8 MEYRICK B LAB INVEST 38 188 978 9 GOLDSTEIN JD AM J CARDIOL 43 962 979 10 ALLEN HD CHEST 75 428 979 11 MEYRICK B AM J PATHOL 94 37 979 12 MEYRICK B AM J PATHOL 96 51 979 13 REID LM AM REV RESPIR DIS 119 531 979 14 MATTHAY RA BR HEART J 43 474 980 15 LESTER LA J PEDIATR 97 742 980 16 FRANCIS PWJ AM J DIS CHILD 134 734 980 17 MATTHAY RA MED CLIN NORTH AM 65 489 981 18 MIGALLY N J SUBMICROSC CYTOL 14 239 982 19 HAWORTH SG CARDIOVASC RES 16 293 982 20 RABINOVITCH M AM J CARDIOL 50 804 982 21 REID L ANN NY ACAD SCI 384 3 982 22 KUZEMKO JA LANCET 1 448 983 23 COTTON DJ RESPIR PHYSIOL 54 19 983 24 GAULTIER C EUR J RESPIR DIS 64 319 983 25 GAULTIER C AM REV RESPIR DIS 127 527 983 26 BRIGHAM KL CIRC RES 54 623 984 27 GAULTIER C EUR J RESPIR DIS 65 460 984 28 LESTER LA SEM RESPIR MED 6 285 985 29 GAULTIER C BULL EUR PHYSIOPATH RESP 21 55 985 30 GAULTIER C CHEST 87 168 985 31 MEYRICK B CHEST 88 S268 985 32 GEGGEL RL AM REV RESPIR DIS 131 531 985 33 HAWORTH SG INT J CARDIOL 13 207 986 34 THOMPSON BT HEART LUNG 15 457 986 35 MEYRICK B LAB INVEST 55 164 986 36 PERKETT EA J APPL PHYSIOL 61 1875 986 37 REID LM CHEST 89 279 986 38 HAWORTH SG WIEN KLIN WOCHENSCHR 98 732 986 39 BRIGHAM KL AM REV RESPIR DIS 133 913 986 40 GAULTIER C BULL EUR PHYSIOPATH RESP 23 87 987 41 LEBECQUE P CHEST 91 693 987 PN 75183 RN 00349 AN 77237076 AU Gocmen-A. TI The comparison of peak flow rate with other spirometric measurements in cyctic fibrosis patients. SO Turk-J-Pediatr. 1975 Jul-Aug. 17(3-4). P 92-104. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. COMPARATIVE-STUDY. FEMALE. HUMAN. MALE. RESPIRATORY-FUNCTION-TESTS. AB This study was done to determine the role of the Wright peak flowmeter for evaluation of pulmonary functions in children with cystic fibrosis. Peak expiratory flow rate was measured with the use of a Wright peak flowmeter and one second forced expiratory volume, maximal mid-expiratory flow rate and maximal voluntary ventilation were measured with a spirometer in a total of 55 children, 25 of whom had cystic fibrosis disease, with the remainder being normal. Measurements were repeated after administration of bronchodilating drugs to the cystic fibrosis patients. All the values of the four measurements were decreased significantly in the cystic fibrosis patients. Positive and significant correlation co-efficients were obtained by comparing the peak flow rate with other spirometric measurements. It was concluded that peak expiratory flow rate is a feasible and useful measurement of pulmonary functions in cystic fibrosis patients. No changes were observed after administration of bronchodilating drugs to these patients. RF 001 SHWACHMAN H PEDIATRICS 36 689 965 002 DI SANTAGNESE PA N ENGL J MED 277 1399 967 003 BEIER FR AM REV RESPIR DIS 94 430 966 004 WEST JR PEDIATRICS 13 155 954 005 COOK CD PEDIATRICS 24 181 959 006 ZELKOWITZ PS AM J DIS CHILD 117 543 969 007 HARRISON GM AM J DIS CHILD 100 530 960 008 DEMUTH GR AM J DIS CHILD 103 129 962 009 DUBOIS AB J CLIN INVEST 35 322 956 010 WRIGHT BM BR MED J 2 1041 959 011 NAIRN JR ARCH DIS CHILD 36 253 961 012 MURRAY AB J PEDIATR 62 186 963 013 HEAF PJD BR MED J 1 1595 962 014$ LOCKHART MB BR MED J 1 960 015 TINKER CM BR MED J 1 1365 961 016 JACKSON MJ BR MED J 1 1760 961 017 TINKER CM BR MED J 2 177 961 018 FLINT FJ BR MED J 2 1231 962 019 LEINER GC AM REV RESPIR DIS 88 644 963 020$ RILEY EA NY STATE J MED 40 2656 969 021 KAMAT SR AM REV RESPIR DIS 96 545 967 022 PRIME FJ BR MED J 1 423 960 023 ROSENBLATT G AM REV RESPIR DIS 87 589 963 024 RITCHIE B LANCET 2 271 962 025 KAZEMI H DIS CHEST 50 500 966 026 GANDEVIA B ARCH DIS CHILD 34 511 959 027 ROYCE SW ROSS PAEDIATR RES CONF 18TH 84 956 028 MATTHEWS LW MOD PROBL PEDIATR 10 297 967 029 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 030 KORY RC AM J MED 30 243 961 031 SNEDECOR GW STATISTICAL METHODS 967 032 CHERNIACK RM CAN MED ASSOC J 87 80 962 033$ STANG LB THORAX 14 39 962 034 ENGSTROM I ACTA PAEDIATR SCAND 45 277 956 035 HELLIESEN PJ PEDIATRICS 22 80 958 036 POLGAR G PULMONARY FUNCTION TESTING IN 971 037 WOOD DW PEDIATR CLIN NORTH AM 16 159 969 038 DENTRY JM MED THORAC 23 358 966 039 FAIRBAIRN AS THORAX 17 168 962 040 LEUALLEN EC AM REV TUBERC PULM DIS 72 783 955 041 COMROE JH JR LUNG CLINICAL PHYSIOLOGY AND 962 042 OLSEN HC BR MED J 1 1450 960 043 DOERSHUK CF IN: GREEN M 707 968 PN 75184 RN 00350 AN 77237069 AU Tinaztepe-B. Kural-N. Tinaztepe-K. Gocmen-A. TI Clinico-pathological evaluation of 37 cases of cystic fibrosis of the pancreas in infancy at necropsy. SO Turk-J-Pediatr. 1975 Jul-Aug. 17(3-4). P 108-18. MJ CYSTIC-FIBROSIS: pa. MN AUTOPSY. FEMALE. HUMAN. INFANT. MALE. EX The clinical and morphological findings of 37 cases of cystic fibrosis of the pancreas from 525 full, but non-consecutive necropsies are presented. All of these 37 cases were diagnosed at post-mortem examination. The autopsy incidence was 7% and none of the cases was beyond two years of age. Morphological findings revealed varying severities in organ involvement in all cases, with absence of fibrosis of the pancreas in some. In only two cases histopathological changes seemed to be confined to the pancreas. This study suggests that cystic fibrosis plays a considerable role in infant deaths in our country and in most if not all cases the diagnosis must necessarily be based on postmortem studies. RF 001 DI SANTAGNESE PA IN: NELSON WE 856 969 002 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 003 HALL BD IN: LAWSON D PROC 5TH INT CF 82 969 004 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 005 WARWICK WJ PROC INT CONG PEDIATR 13TH 101 971 006 GURSON CT HELV PAEDIATR ACTA 28 165 973 007$ HATEMI N MEDICINA ROCHE 2 972 008 OPPENHEIMER EH ARCH PATHOL 96 149 973 009 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 010 DI SANTAGNESE PA N ENGL J MED 277 1287 967 011 MORISON JE FOETAL AND NEONATAL PATHOL 305 963 012 SAY B TURK HALKINDA CESITLI KONGENI 27 971 013 DANKS DM ANN HUM GENET 28 323 965 014 SMOLLER M AM J DIS CHILD 98 277 959 015 SIASSI B J PEDIATR 78 794 971 016 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 017 THOMAIDIS TS J PEDIATR 63 444 963 018 HODSON WJ BR J SURG 56 472 969 019 SELIGER G DIGESTIVE DIS 27 10 972 020 SHWACHMAN H N ENGL J MED 286 1300 972 021 HOLSCLAW DS PEDIATRICS 48 442 971 022 TAYLOR WF AM J DIS CHILD 123 161 972 023 VALMAN HB ARCH DIS CHILD 46 805 971 024 RICKHAM PP IN: RICKHAM PP 366 969 025 MILUNSKY A PEDIATRICS 42 501 968 026 LEE PA JAMA 228 585 974 027 TINAZTEPE K TURK J PEDIATR 17 19 975 028 CASTLEMAN B N ENGL J MED 288 570 973 029 PORTA EA AM J CLIN PATHOL 42 451 964 030 ESTERLY NB AM J DIS CHILD 123 200 972 031 FALCHUK ZM PEDIATRICS 51 49 973 032 VALMAN HB LANCET 2 566 969 033 KAPLAN E N ENGL J MED 279 65 968 034 HOLSCLAW DS BR MED J 3 356 969 PN 75185 RN 00351 AN 75103651 AU Roth-S-S. Roth-R-A. TI Letter: Congenital absence of vasa. SO Urology. 1975 Jan. 5(1). P 158. MJ CYSTIC-FIBROSIS. INFERTILITY-MALE: et. VAS-DEFERENS: ab. MN ABNORMALITIES: co. CYSTIC-FIBROSIS: co. HUMAN. MALE. TESTICULAR-DISEASES: co. EX Although it is generally true that most male patients with cystic fibrosis are infertile due to congenital atresia or absence of the vasal structures, this finding is not invariable. Taussig et al. reported 2 well-studied patients with cystic fibrosis who had normal semen analysis, and at this writing both have fathered children. A third patient with normal semen analysis also is known to us. In addition there are many instances of presumed but undocumented paternity in these patients. Cystic fibrosis centers estimate male fertility at 2 to 3 per cent; although low, this figure warrants semen analysis before assuming sterility. In view of this, we feel that the diagnosis of cystic fibrosis should be considered when the vasa are absent, but it is incorrect to exclude the diagnosis on the basis of palpable vasa. RF 001 TAUSSIG LM N ENGL J MED 287 586 972 CT 1 AMELAR RD UROLOGY 5 430 975 PN 75186 RN 00352 AN 75103652 AU Kornitzer-G-D. Newton-R-A. TI Letter: Cystic fibrosis and absence of vas deferens. SO Urology. 1975 Jan. 5(1). P 158. MJ CYSTIC-FIBROSIS. INFERTILITY-MALE: et. VAS-DEFERENS: ab. MN ABNORMALITIES: co. CYSTIC-FIBROSIS: co. HUMAN. MALE. EX Infertility and abnormalities of the mesonephric derivatives are common in patients with cystic fibrosis, but it is not a uniform finding. Taussig et al. reported on 2 patients with cystic fibrosis who had proved fertility. Abnormalities of the vas could be documented in pathologic studies, but the vas was found to be present in some patients who died of cystic fibrosis. In many patients, absence could not be ruled out on physical examination. In healthy, young children the vas is not easily palpable; even when it is, the degree of certainty in its identification is less than in the post-pubertal male. Cystic fibrosis is a disease with protean manifestations, and it would be a mistake to rule out cystic fibrosis just because the vas can be palpated or to have victims of cystic fibrosis labeled as infertile. RF 001 TAUSSIG LM N ENGL J MED 287 586 972 002 HOLSCLAW DS J UROL 106 568 971 CT 1 AMELAR RD UROLOGY 5 430 975 PN 75187 RN 00353 AN 75199860 AU Dajani-A-M. TI Obscure bilateral calcified swellings of paratesticular tissues. SO Urology. 1975 Jul. 6(1). P 101-4. MJ CALCINOSIS: pa. SCROTUM: pa. MN CALCINOSIS: et. CASTRATION. CYSTIC-FIBROSIS: co. HUMAN. INFANT. MALE. MECONIUM. PERITONITIS: co. AB A case of calcified swellings of the paratesticular tissues discovered in an infant at different ages is described. The exact cause is obscure; and three pathologic conditions are suggested. Possibly it was due to an old reaction to meconium peritonitis through a patent processus vaginalis. RF 004 OERTEL HB JAMA 49 32 907 005 THANNHAUSER SJ PHYSIOL REV 26 275 946 006 WILLIAMS G BR J UROL 51 332 969 007 BERDON WE N ENGL J MED 277 585 967 008 OLNICK HM CITED IN: N ENGL J MED 277 585 967 009 THOMPSON RB J UROL 110 364 973 CT 1 FOROUHAR F AM J CLIN PATHOL 78 208 982 PN 75188 RN 00354 AN 75180144 AU Moss-A-J. Dooley-R-R. Mickey-M-R. TI Cystic fibrosis complicated by heart failure. SO West-J-Med. 1975 Jun. 122(6). P 471-3. MJ CYSTIC-FIBROSIS: co. HEART-FAILURE-CONGESTIVE: et. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mo. FEMALE. HEART-FAILURE-CONGESTIVE: mo. HUMAN. INFANT. MALE. PULMONARY-HEART-DISEASE: et, mo. TIME-FACTORS. AB Survival studies were done on 36 children with cystic fibrosis and heart failure. Thirty percent did not survive the first four weeks, and the median survival for the group was between two and three months. By the end of the first year from the onset of failure, 74 percent had died and at 30 months, 87 percent had died. RF 001 WIGLESWORTH FW AM J MED SCI 212 351 946 002 ROYCE SW PEDIATRICS 8 255 951 003 GOLDRING RM J PEDIATR 65 501 964 004 SHWACHMAN H AM J DIS CHILD 96 6 958 005 SIASSI B J PEDIATR 78 794 971 006 MOSS AJ MOD PROBL PEDIATR 10 187 967 007 CUTLER SJ J CHRON DIS 8 699 958 CT 1 MATTHAY RA BR HEART J 43 474 980 2 FRANCIS PWJ AM J DIS CHILD 134 734 980 3 STERN RC AM J DIS CHILD 134 267 980 4 MATTHAY RA MED CLIN NORTH AM 65 489 981 5 MOSS AJ PEDIATRICS 70 728 982 6 LESTER LA SEM RESPIR MED 6 285 985 PN 75189 RN 00355 AN 75180411 AU Dominick-H-C. Husen-N-V. Hosemann-R. Gerlach-U. TI Isoenzymes of alkaline phosphatase in the serum of patients with cystic fibrosis. SO Z-Kinderheilkd. 1975. 119(4). P 261-7. MJ ALKALINE-PHOSPHATASE: bl. CYSTIC-FIBROSIS: en. MN ADOLESCENCE. BILE-DUCTS: en. BILIARY-TRACT-DISEASES: et, di. BODY-WEIGHT. BONE-AND-BONES: en. CHILD. CHILD-PRESCHOOL. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. ELECTROPHORESIS-STARCH-GEL. ENZYME-TESTS. FEMALE. HUMAN. INTESTINES: en. ISOENZYMES: bl. LIVER-DISEASES: et, di. LIVER: en. MALE. AB In 36 children with cystic fibrosis (CF), the isoenzymes of alkaline phosphatase (AP) were determined microelectrophoretically in polyacrylamide- and starch-gel. The study was done to evaluate the clinical significance of these additional data for the diagnosis of liver involvement in CF. The results lead to the following conclusions. Serum activity of total AP is comparatively unsensitive "masking" alterations in the isoenzyme pattern contributing to the AP serum activity. In 17 children resp. 47% bile-duct phosphatase was increased indicating a secretostasis while other marker enzymes of cholestasis were normal in part. The activity of bone phosphatase in the serum showed a significant correlation to the degree of growth retardation in these patients. Intestinal phosphatase was present in the serum of only one child with cirrhosis of the liver being an indicator for liver insufficiency. Determination of AP isoenzymes in the serum may provide additional information about the organs involved for the physician in handling CF patients. RF 001 BAMBERGER CF ARCH BIOCHEM BIOPHYS 123 195 968 002 BATSAKIS JG ARCH SURG 95 138 967 003 BAUMFORD KF LANCET 1 530 965 004 BECKMANN R ACTA HEPATO SPLENOL 6 65 959 005 BODANSKY O J BIOL CHEM 118 341 937 006 DI SANTAGNESE PA PEDIATRICS 18 387 956 007 FANCONI G WIEN MED WOCHENSCHR 86 753 936 008 FEIGELSON J PROC EWGCF 4TH ANNU MTG 973 009 FISHMAN WH CLIN CHIM ACTA 12 298 965 010 GERLACH U MED WELT 21 1252 970 011 HERZ R ARCH BIOCHEM BIOPHYS 69 506 962 012 HORNE M J LAB CLIN MED 72 905 968 013 HOSENFELD D MED KLIN 67 1301 972 014 VAN HUSEN N MED WELT 24 1510 973 015 VAN HUSEN N CLIN CHIM ACTA 53 91 974 016 KAPLAN MM N ENGL J MED 286 200 972 017 KATTWINKEL J J PEDIATR 82 234 973 018 KLEIN UE GASTROENTEROLOGIA SUPPL 107 137 967 019 KLEIN UE CLIN CHIM ACTA 16 163 967 020 KLEIN UE LANCET 2 1396 968 021 KLEIN UE DTSCH MED WSCHR 94 526 969 022 KREISSL T Z KINDERHEILK 113 93 972 023 MOSSER RH AM J OBSTET GYNECOL 98 459 967 024 MUCKE D DTSCH GESUNDHEITSW 26 2418 971 025 POSEN S ANN INTERN MED 62 1234 965 026 RONCHI G BEITR PATHOL ANAT 149 213 973 027 SHWACHMAN H PEDIATRICS 46 335 970 028 SITZMANN FC MED KLIN 67 1183 972 029 SKILLEN AW CLIN CHIM ACTA 40 21 972 030 SUSSMANN HH J BIOL CHEM 243 160 968 031 SZASZ G IN: BERGMEYER HU 733 970 032 SZASZ G DTSCH MED WSCHR 94 1911 969 CT 1 ALHADEFF JA CLIN GENET 10 63 976 2 KRUSE K EUR J PEDIATR 126 53 977