PN 74001 RN 00001 AN 75051687 AU Hoiby-N. Jacobsen-L. Jorgensen-B-A. Lykkegaard-E. Weeke-B. TI Pseudomonas aeruginosa infection in cystic fibrosis. Occurrence of precipitating antibodies against pseudomonas aeruginosa in relation to the concentration of sixteen serum proteins and the clinical and radiographical status of the lungs. SO Acta-Paediatr-Scand. 1974 Nov. 63(6). P 843-8. MJ CYSTIC-FIBROSIS: co. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: co. RESPIRATORY-TRACT-INFECTIONS: co. MN ADOLESCENCE. BLOOD-PROTEINS: me. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im, bl. FEMALE. HUMAN. IMMUNOELECTROPHORESIS. IMMUNOGLOBULINS: me. LUNG: ra. MALE. PRECIPITIN-TESTS. PRECIPITINS. PSEUDOMONAS-INFECTIONS: im, bl, ra. RESPIRATORY-TRACT-INFECTIONS: bl, im, ra. SERUM-ALBUMIN: me. AB The significance of Pseudomonas aeruginosa infection in the respiratory tract of 9 cystic fibrosis patients have been studied by means of immunoelectrophoretical analysis of patients' sera for the number of precipitins against Pseudomonas aeruginosa and the concentrations of 16 serum proteins. In addition, the clinical and radiographical status of the lungs have been evaluated using 2 scoring systems. Precipitins against Pseudomonas aeruginosa were demonstrated in all sera, the maximum number in one serum was 22. The concentrations of 12 of the serum proteins were significantly changed compared with matched control persons. Notably IgG and IgA were elevated and the "acute phase proteins" were changed, the latter suggesting active tissue damage. The concentrations of 3 of the acute phase proteins, notably haptoglobin, were correlated to the number of precipitins suggesting that the respiratory tract infection in patients with many precipitins is accompanied by more tissue damage than the infection in patients with few precipitins. The results indicate no protective value of the many precipitins on the tissue of the respiratory tract. RF 001 BELFRAGE S ACTA MED SCAND SUPPL 173 5 963 002 COOMBS RRA IN: GELL PGH 317 964 003 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 004 DOGGETT RG J PEDIATR 68 215 966 005 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 006 DOGGETT RG INFECT IMMUN 6 628 972 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 009 KOCH C ACTA PATH MICROBIOL SCAND (B) 81 787 973 010 LUNDH B ACTA UNIV LUND II 24 1 965 011$ LUNDH B ACTA UNIV LUND II 27 1 965 012 MINCHIN-CLARKE HG THORAX 25 423 970 013 MULLER HE DTSCH MED WSCHR 93 120 968 014 NORMAN AP PROC EWGCF 2ND ANNU MTG 971 015 NYMAN M SCAND J CLIN LAB INVEST SUPPL 39 5 959 016 WEEKE B ACTA MED SCAND 192 149 972 017 WEEKE B THESIS 1 973 018 WEEKE B SCAND J IMMUNOL SUPPL 1 2 37 973 019 WEEKE B SCAND J IMMUNOL SUPPL 2 1 48 973. CT 1 HOIBY N SCAND J RESPIR DIS 56 38 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 3 AXELSON NH SCAND J IMMUNOL 5 177 976 4 GARGANI GF ANN SCLAVO 18 179 976 5 WEEKE B DAN MED BULL 23 155 976 6 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 7 HOIBY N SCAND J RESPIR DIS 58 65 977 8 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 9 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 10 KLINGER JD J INFECT DIS 138 49 978 11 MCCARTHY MM CLIN PEDIATR 19 746 980 12 MOSS RB AM REV RESPIR DIS 121 23 980 13 MOSS RB J PEDIATR 99 215 981 14 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 15 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 16 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 17 MARHAUG G ACTA PAEDIATR SCAND 72 861 983 18 MCGLENNEN RC ARCH PATHOL LAB MED 110 879 986 19 EMMETT M PROC SOC EXP BIOL MED 184 74 987 PN 74002 RN 00002 AN 74260154 AU Rossiter-M-A. Barrowman-J-A. Dand-A. Wharton-B-A. TI Amylase content of mixed saliva in children. SO Acta-Paediatr-Scand. 1974 May. 63(3). P 389-92. MJ SALIVA: en. AMYLASES: me. NUTRITION-DISORDERS: en. CELIAC-DISEASE: en. MN INFANT. CHILD-PRESCHOOL. CHILD. ADOLESCENCE. HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: en. ENTERAL-FEEDING. AGE-FACTORS. FOLLOW-UP-STUDIES. INFANT-PREMATURE-DISEASES. ESOPHAGEAL-ATRESIA: en. AB Salivary amylase levels were determined in normal subjects from birth until adult life and in children with conditions sometimes associated with low pancreatic amylase such as malnutrition, coeliac disease and cystic fibrosis. Mixed saliva was collected under carefully standardised conditions and amylase was measured by the method of Dahlqvist. There was a wide scatter of values in the 84 normal subjects, but concentrations rose from very low levels at birth to reach adult levels by the age of 6 months to 1 year. Salivary amylase activity rose normally over ten weeks in one premature infant fed milk by gastrostomy. Thirteen children with coeliac disease and 9 children with cystic fibrosis mostly had normal salivary amylase concentrations. Six out of 12 malnourished children with jejunal villous atrophy of uncertain aetiology had low levels which rose to normal as recovery began. RF 001 ANDERSEN DH J PEDIATR 30 564 947 002 BARBEZAT GO S AFR MED J 41 84 967 003 CHERNICK WS J PEDIATR 65 694 964 004 DAHLQVIST A SCAND J CLIN LAB INVEST 14 145 962 005 DAWES C J PHYSIOL (LOND) 220 529 972 006 DELACHAUME-SALEM E BIOL GASTROENTEROL 2 135 970 007 DI SANTAGNESE PA ACTA PAEDIATR SCAND 46 51 957 008 DI SANTAGNESE PA PEDIATRICS 22 507 958 009 HADORN B J PEDIATR 73 39 968 010 KAMARYT J Z KLIN CHEM KLIN BIOCHEM 8 564 970 011 KERR AC PHYSIOLOGICAL REGULATION 28 961 012 ANON LANCET 2 302 970 013 MANDEL ID AM J DIS CHILD 113 431 967 014 MAYER WB BULL JOHNS HOPKINS HOSP 44 246 929 015 MCDOUGALL C PEDIATRICS 5 114 950 016 NICORY C BIOCHEM J 6 387 922 017 ZOPPI G ACTA PAEDIATR SCAND 61 506 972 CT 1 ALHADEFF JA CLIN GENET 10 63 976 2 LASZLO A ACTA PAEDIATR ACAD SCI HUNG 17 293 976 3 SKUDE G ACTA PAEDIATR SCAND 65 145 976 4 JONES JB J PEDIATR GASTROENTEROL NUTR 1 43 982 5 LINDBERG T PEDIATRICS 70 235 982 6 LEE PC J PEDIATR GASTROENTEROL NUTR 2 S227 983 7 HEITLINGER LA PEDIATR RES 17 15 983 8 HODGE C PEDIATR RES 17 998 983 9 LEBENTHAL E J PEDIATR 102 1 983 10 HEGARDT P J PEDIATR GASTROENTEROL NUTR 3 563 984 11 BENARYEH H ARCH ORAL BIOL 29 357 984 12 SEVENHUYSEN GP AM J CLIN NUTR 39 584 984 PN 74003 RN 00003 AN 74252221 AU Kollberg-H. Ekbohm-G. TI A clinical study of the diagnosis of cystic fibrosis by instrumental neutron activation analysis of sodium in nail clippings. SO Acta-Paediatr-Scand. 1974 May. 63(3). P 411-7. MJ CYSTIC-FIBROSIS: di. NAILS: an. SODIUM: an. MN INFANT-NEWBORN. INFANT. CHILD-PRESCHOOL. CHILD. ADOLESCENCE. ADULT. HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: me, fg. ACTIVATION-ANALYSIS. HETEROZYGOTE. AGE-FACTORS. INFANT-PREMATURE. BIRTH-WEIGHT. METHODS. AB This article reports on the possibility of using instrumental neutron activation analysis (INAA) of sodium in nail clippings for diagnosing cystic fibrosis (CF) in children and adults, for detecting heterozygotes and for screening in the neonatal period. Nail clippings from 1322 newborns, 22 CF patients (two of them newborns), 52 healthy controls and 22 heterozygotes were analyzed. The discrimination between CF patients and controls was found to be precise for individuals above one year of age and INAA of nail clippings should be accepted as a diagnostic test for CF after this age. Heterozygotes could not be detected by the method. During the first five days of life there is a big overlap between the values from normal newborns and those of CF children, which makes the method invaluable for early screening for CF. RF 001 BOWEN HJM ADV ACTIVATION ANALYS 1 101 969 002 BRAY PT PROC EWGCF 3RD ANNU MTG 972 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 FITE LE PROC INT CONF MOD TRENDS 147 968 005 GEORGE L ARCH DIS CHILD 46 139 971 006 HEINTZ PH THESIS 967 007 JILEK M ZASTOSAWANIA MATEMATYKI 5 155 960 008 JOHNSON GF PEDIATRICS 47 88 971 009 KOLLBERG H ACTA PAEDIATR SCAND 63 405 974 010 KOPITO L N ENGL J MED 272 504 965 011 SCHEFFE H ANALYSIS OF VARIANCE 959 012 SHWACHMAN H NAT CF RES FOUND 963 013 SHWACHMAN H PEDIATRICS 46 335 970 014 STAMM SJ TERMINAL REPORT TO WASHINGTON 971 015 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 016 WINGE-FLENSBORG E PROC EWGCF 1ST ANNU MTG 970 017 WOODRUFF L NUCL APPL 6 352 969 018 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 CT 1 KOLLBERG H SCAND J CLIN LAB INVEST 35 17 975 2 VANSTEKELENBURG GJ CLIN CHIM ACTA 59 233 975 3 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 4 TARNOKY AL CLIN CHIM ACTA 69 505 976 5 ANON J PEDIATR 88 711 976 6 HOSLI P ACTA PAEDIATR SCAND 67 617 978 7 ROOMANS GM ACTA PAEDIATR SCAND 67 89 978 8 HEELEY AF CLIN CHEM 29 2011 983 9 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 10 BARDON A ACTA PAEDIATR SCAND 73 263 984 11 ROOMANS GM ANN NY ACAD SCI 428 121 984 12 CHAPMAN AL EUR J RESPIR DIS 66 218 985 PN 74004 RN 00004 AN 74252220 AU Kollberg-H. Landstrom-O. TI A methodological study of the diagnosis of cystic fibrosis by instrumental neutron activation analysis of sodium in nail clippings. SO Acta-Paediatr-Scand. 1974 May. 63(3). P 405-10. MJ CYSTIC-FIBROSIS: di. NAILS: an. SODIUM: an. MN CHILD-PRESCHOOL. HUMAN. CYSTIC-FIBROSIS: me. ACTIVATION-ANALYSIS. SWEAT: an. METHODS. TIME-FACTORS. AB Instrumental neutron activation analysis (INAA) of nail clippings was used in the development of a diagnostic method for cystic fibrosis (CF). From CF patients and controls more than three years old, nail clippings were sampled for tests of different errors. As a result of this study a precise collection routine was outlined. Sodium is easily washed out from nail clippings. The risk of sodium contamination of the nails seems to be small. The sodium distribution in the nail is inhomogeneous, and the sodium content varies also from nail to nail in the same person. There is good evidence that the increased sodium in the nails of CF patients comes from the sweat. There seems to be a basic "intrinsic" sodium level, which is about the same in the nails of CF patients as in controls. The precision and accuracy of INAA for determining sodium in nails is considered satisfactory. It is concluded that INAA of the sodium concentration in nail clippings is a suitable method as an aid in the diagnosis of CF if a precise collection routine is used. RF 001 ANDERSEN GH TRANS AM NUCL SOC 10 57 967 002 ANTONELLI M ARCH DIS CHILD 44 218 969 003 BERG T NORTHERN PEDIATR CONG 15 967 004 BOWEN HJM PANEL ON ACTIVATION ANALYSIS 3 968 005 BOWEN HJM ADV ACTIVATION ANALYS 1 101 969 006 BRUNE D ANAL CHIM ACTA 34 447 966 007 DI SANTAGNESE PA IN: NELSON WE 771 964 008 FITE LE PROC INT CONF MOD TRENDS 147 968 009 GEORGE L ARCH DIS CHILD 46 139 971 010 GOLDBLUM RW J INVEST DERMATOL 20 13 953 011 GROSSE KP Z KINDERHEILK 100 87 967 012 HARRISON GM CF CLUB ABST 11 26 970 013 HEINTZ PH THESIS 967 014 JOHNSON GF PEDIATRICS 47 88 971 015 KOPITO L N ENGL J MED 272 504 965 016 NICOLAIDOU MA PROC INT CONG PEDIATR 13TH 971 017 SHWACHMAN H PEDIATRICS 46 335 970 018 STAMM SJ TERMINAL REPORT TO WASHINGTON 971 019 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 020 WOODRUFF L PROC INT CONF MOD TRENDS 156 968 021 WOODRUFF L NUCL APPL 6 352 969 CT 1 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 2 KOLLBERG H ACTA PAEDIATR SCAND 63 411 974 3 VANSTEKELENBURG GJ CLIN CHIM ACTA 59 233 975 4 LANZA I MINERVA PEDIATR 28 1510 976 5 TARNOKY AL CLIN CHIM ACTA 69 505 976 6 SOHLER A CLIN CHIM ACTA 70 391 976 7 ROOMANS GM ACTA PAEDIATR SCAND 67 89 978 8 FORSLIND B SCANN ELECTRON MICROSC 1982 1715 982 9 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 10 HEELEY AF CLIN CHEM 29 2011 983 11 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 12 ROOMANS GM ANN NY ACAD SCI 428 121 984 13 CHAPMAN AL EUR J RESPIR DIS 66 218 985 PN 74005 RN 00005 AN 75051680 AU Lindberg-T. TI Proteolytic activity in duodenal juice in infants, children, and adults. SO Acta-Paediatr-Scand. 1974 Nov. 63(6). P 805-8. MJ DUODENUM: en. INTESTINAL-SECRETIONS: en. PEPTIDE-HYDROLASES: me. TRYPSIN: me. MN ADULT. AGE-FACTORS. CASEINS: me. CELIAC-DISEASE: en. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. FEMALE. HUMAN. INFANT. INTESTINAL-SECRETIONS: me. MALABSORPTION-SYNDROMES: en. MALE. MILK-PROTEINS. PANCREATIC-DISEASES: en. PEPTIDES: me. TIME-FACTORS. AB The capacity of duodenal juice to hydrolyse casein to trichloroacetic acid-soluble peptides was investigated: in a control group (11 infants and children and 7 adults); in 5 children with pancreatic disease; in 10 children with enterogenic malabsorption (coeliac disease and cow's milk protein intolerance). The proteolytic activity per ml duodenal juice in fasting condition was as great in healthy infants and children as healthy adults (500-600 mg casein/ml/hour). There was a good correlation between trypsin content and proteolytic activity of duodenal juice. In pancreatic insufficiency, the proteolytic activity was extremely low (not measurable in 3 patients) as was the trypsin activity whereas in enterogenic malabsorption, it was within the ranges of the controls. RF 001 BERGSTROM K SCAND J GASTROENTEROL 5 533 970 002 BORGSTROM B J CLIN INVEST 36 1521 957 003 BORGSTROM B AM J DIS CHILD 101 454 961 004 CRANE CW BIOCHEM J 74 313 960 005 DENNEBERG T ACTA MED SCAND 195 465 974 006 ERLANGER BF ARCH BIOCHEM BIOPHYS 95 271 961 007 GOLDBERG DM PROC ASSOC CLIN BIOCHEM 4 202 967 008 GOLDBERG DM SCAND J GASTROENTEROL 3 193 968 009 HADORN B J PEDIATR 73 39 968 010 HARTLEY RC GASTROENTEROLOGY 48 312 965 011 HIRATA Y KOBE J MED SCI 11 103 965 012 LUNDH G ACTA CHIR SCAND SUPPL 231 958 013 LUNDH G GASTROENTEROLOGY 42 275 962 014 NIXON ES BR J NUTR 24 227 970 015 ROSENBERG R GASTROENTEROLOGY 50 191 966 CT 1 BROCK JH IMMUNOLOGY 32 207 977 2 SCHMITZ J GASTROENTEROL CLIN BIOL 2 929 978 3 BERG NO ACTA PAEDIATR SCAND 67 403 978 4 BROCK JH ARCH DIS CHILD 55 417 980 5 GLASGOW JFT IR J MED SCI 149 194 980 6 JAKOBSSON I J PEDIATR GASTROENTEROL NUTR 1 183 982 7 REITER B INT J TISS REACT EXP CLIN ASP 5 87 983 8 UDALL JN BIOL NEONATE 45 289 984 9 MCNEISH AS ANN ALLERGY 53 643 984 10 SARLES J ARCH FR PEDIATR 44 311 987 PN 74006 RN 00006 AN 75014083 AU Hoiby-N. Mathiesen-L. TI Pseudomonas aeruginosa infection in cystic fibrosis. Distribution of B and T lymphocytes in relation to the humoral immune response. SO Acta-Pathol-Microbiol-Scand [B]. 1974 Aug. 82(4). P 559-66. MJ B-LYMPHOCYTES: im. CYSTIC-FIBROSIS: im. IMMUNITY. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im. RESPIRATORY-TRACT-INFECTIONS: im. T-LYMPHOCYTES: im. MN ADOLESCENCE. ANTIBODIES-BACTERIAL. ANTIGENS-BACTERIAL. CHILD. COMPLEMENT. CYSTIC-FIBROSIS: mi, bl. FEMALE. HUMAN. IMMUNE-ADHERENCE-REACTION. IMMUNE-SERA. IMMUNOELECTROPHORESIS. IMMUNOGLOBULINS. MALE. NEUTROPHILS. PRECIPITINS. PSEUDOMONAS-AERUGINOSA: ip. RABBITS: im. RESPIRATORY-TRACT-INFECTIONS: mi. AB The influence of chronic Ps. aeruginosa infection on the occurrence of bone marrow-derived lymphocytes (B cells) and thymus-derived lymphocytes (T cells) in peripheral blood have been studied in 2 groups of patients with cystic fibrosis. One group (9 patients) suffered from chronic Ps. aeruginosa infection in the respiratory tract and produced multiple Ps. aeruginosa precipitins which were demonstrated by means of crossed immunoelectrophoresis. The other group (9 patients) had never harboured Ps. aeruginosa in the respiratory tract and presented no demonstrable precipitins against this bacteria. The lymphocytes were examined for the presence of 2 surface markers for B cells: receptor for C3 complement component (EAC rosette-formation) and surface immunoglobulins (immunofluorescent staining), and for the presence of 1 surface marker for T cells: spontaneous binding of sheep red blood cells (E rosette-formation). The B:T cell ratio was nearly identical in the 2 groups of patients whereas the total numbers of B and T cells were significant higher in patients with chronic Ps. aeruginosa infection. The number of Ps. aeruginosa precipitins per serum was significantly positively correlated to the total number of T cells, wheras the correlation with the total number of B cells was insignificant. RF 001 AIUTI F INFECT IMMUN 8 110 973 002 AIUTI F CLIN EXP IMMUNOL 13 171 973 003 BENTWICH Z CLIN IMMUNOL IMMUNOPATHOL 1 511 973 004 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 005 DOGGETT RG INFECT IMMUN 6 628 972 006 DWYER JM N ENGL J MED 288 1036 973 007 FLORESHEIM GL AGENTS ACTIONS 2 231 972 008 GAJL-PECZALSKA KJ N ENGL J MED 288 1033 973 009 GAJL-PECZALSKA KJ J CLIN INVEST 52 919 973 010 GIFFORD RM J S CAROLINA MED ASSOC 68 204 972 011 GORDON DS AM REV RESPIR DIS 108 127 973 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 015 JONDAL M J EXP MED 136 207 972 016 MELLSTEDT H CLIN EXP IMMUNOL 15 321 973 017 ROSS GD J CLIN INVEST 52 377 973 018 SHEVACH EM IN: MOLLER G 16 3 973 019 STJERNSWARD J LANCET 1 1352 972 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 2 VERBRUGGEN R CLIN CHEM 21 5 975 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 5 CLARKE CW CLIN ALLERGY 7 527 977 6 HOIBY N SCAND J RESPIR DIS 58 65 977 7 SORENSEN RU INFECT IMMUN 23 398 979 8 LIEBERMAN MM INFECT IMMUN 29 489 980 9 HODSON ME THORAX 35 801 980 10 WILSON GB J CLIN INVEST 66 1010 980 11 HARPER TB LUNG 157 219 980 12 VANGEFFEL R IMMUNOL LETTERS 5 155 982 13 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 14 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 15 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 16 MARESZBABCZYSZYN J ARCH IMMUNOL THER EXP 31 199 983 17 SORENSEN RU EUR J RESPIR DIS 64 524 983 18 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 19 KOMIYAMA K CAN J MICROBIOL 33 27 987 PN 74007 RN 00007 AN 75014084 AU Hoiby-N. TI Pseudomonas aeruginosa infection in cystic fibrosis. Relationship between mucoid strains of Pseudomonas aeruginosa and the humoral immune response. SO Acta-Pathol-Microbiol-Scand [B]. 1974 Aug. 82(4). P 551-8. MJ CYSTIC-FIBROSIS: im. IMMUNITY. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im. RESPIRATORY-TRACT-INFECTIONS: im. MN ANTIBODIES-BACTERIAL. ANTIGENS-BACTERIAL. CYSTIC-FIBROSIS: mi. HUMAN. IMMUNOELECTROPHORESIS. MUCOPROTEINS. PRECIPITINS. PSEUDOMONAS-AERUGINOSA: ip. RESPIRATORY-TRACT-INFECTIONS: mi. SEX-FACTORS. SPUTUM: mi. TIME-FACTORS. TRACHEA: mi. AB The occurrence of Pseudomonas aeruginosa in the respiratory tract of 70 cystic fibrosis patients and the occurrence of precipitins against Ps. aeruginosa in sera from the same patients have been investigated during one year by means of monthly bacteriological examinations of tracheal secretions and by means of crossed immunoelectrophoresis of a polyvalent Ps. aeruginosa antigen against sera from the patients. The one-year period prevalence rate of patients harbouring Ps. aeruginosa was 64 per cent. In newly colonized patients and in intermittently colonized patients non-mucoid strains were predominating, whereas mucoid strains were predominating in chronically colonized patients. The occurrence of mucoid strains, especially in chronically colonized patients, was associated with a significantly higher number of precipitins against Ps. aeruginosa than the occurrence of non-mucoid strains. Males chronically colonized with mucoid strains presented a significantly higher number of precipitins than females chronically colonized with mucoid strains and the number of precipitins was correlated with the duration of the chronic colonization with Ps. aeruginosa in males in contrast to females. The results are in accordance with the hypothesis that mucoid substance could be a virulence factor because it might inhibit the opsonizing effect of the precipitins on the mucoid cells and the complement dependent lysis of the cells hereby favouring mucoid strains at the expense on non-mucoid strains in the respiratory tract of cystic fibrosis patients. RF 001 BURNS MW LANCET 1 270 968 002 BURNS MW BR MED J 3 382 973 003 CARLSON DM BIOCHEMISTRY 5 2817 966 004 DIAZ F J INFECT DIS 121 269 970 005 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 006 DOGGETT RG J PEDIATR 68 215 966 007 DOGGETT RG SOUTH MED J 61 1347 968 008 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 009 DOGGETT RG APPL MICROBIOL 18 936 969 010 DOGGETT RG INFECT IMMUN 6 628 972 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 016 HUANG NN J PEDIATR 59 512 961 017 IACOCCA VF AM J DIS CHILD 106 315 963 018 MARTIN DR J MED MICROBIOL 6 111 973 019 SCHWARZMANN S INFECT IMMUN 3 762 971 020 SONNENSCHEIN C ZENTRALBL BAKTERIOL 104 365 927 021 THERKELSEN AJ MEDICINSK STATISTIK AKADEMISK 968 022 WEEKE B SCAND J IMMUNOL SUPPL 2 1 48 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 2 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 3 VERBRUGGEN R CLIN CHEM 21 5 975 4 HOIBY N SCAND J RESPIR DIS 56 38 975 5 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 6 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 8 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 321 975 9 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 433 975 10 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 328 975 11 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 12 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 195 975 13 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 144 975 14 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 15 HOIBY N SCAND J IMMUNOL 1975 197 975 16 HOIBY N SCAND J IMMUNOL 1975 187 975 17 AXELSON NH SCAND J IMMUNOL 5 177 976 18 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 383 976 19 HOIBY N ACTA PATH MICROBIOL SCAND (B) 84 395 976 20 HOIBY N ACTA PATH MICROBIOL SCAND (C) 84 372 976 21 WOOD RE AM REV RESPIR DIS 113 833 976 22 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 23 CLARKE CW CLIN ALLERGY 7 527 977 24 HOIBY N SCAND J RESPIR DIS 58 65 977 25 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 149 977 26 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 27 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 28 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 29 SCHIOTZ PO HUM HERED 28 293 978 30 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 31 KULCZYCKI LL JAMA 240 30 978 32 HAMPTON KD J CLIN MICROBIOL 9 632 979 33 SEALE TW J CLIN MICROBIOL 9 72 979 34 SCHIOTZ PO MONOGR PAEDIATR 10 131 979 35 FRIIS B SCAND J INFECT DIS 11 211 979 36 HOIBY N ACTA PAEDIATR SCAND 68 495 979 37 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 38 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 345 979 39 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 337 979 40 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 329 979 41 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 42 LOENINGBAUCKE VA J PEDIATR 95 630 979 43 MICHALSEN H CHEMOTHERAPY 26 135 980 44 LAM J INFECT IMMUN 28 546 980 45 LIEBERMAN MM INFECT IMMUN 29 489 980 46 MARTIN AJ ARCH DIS CHILD 55 604 980 47 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 48 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 49 BALTIMORE RS J INFECT DIS 141 238 980 50 COSTERTON JW CRC CRIT REV MICROBIOL 8 303 981 51 BLACKWOOD LL INFECT IMMUN 32 443 981 52 PENNINGTON JE J CLIN INVEST 68 1140 981 53 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 54 MACONE AB N ENGL J MED 304 1445 981 55 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 56 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 57 BOYCE JR INFECT IMMUN 37 840 982 58 OHMAN DE INFECT IMMUN 37 662 982 59 HOOKE AM INFECT IMMUN 38 136 982 60 RIVERA M AM REV RESPIR DIS 126 833 982 61 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 62 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 63 BALTIMORE RS PEDIATR RES 17 952 983 64 DORING G INFECT IMMUN 42 197 983 65 HANCOCK REW INFECT IMMUN 42 170 983 66 JOHNSON SR OBSTET GYNECOL 61 S 2 983 67 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 68 DORING G J INFECT DIS 147 744 983 69 GRISTINA AG ORTHOP CLIN NORTH AM 15 517 984 70 NACUCCHIO MC PEDIATR RES 18 295 984 71 CHAN R J CLIN MICROBIOL 19 8 984 72 MCCARTHY VP ARCH INTERN MED 144 408 984 73 HANCOCK REW J INFECT DIS 149 220 984 74 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 75 IRVIN RT CAN J MICROBIOL 31 268 985 76 STANISLAVSKY ES ACTA MICROBIOL HUNG 32 49 985 77 DORING G INFECT IMMUN 49 557 985 78 LUZAR MA INFECT IMMUN 50 577 985 79 GRISTINA AG J BONE JOINT SURG 67A 264 985 80 WOODS DE J INFECT DIS 151 581 985 81 BALTIMORE RS PEDIATR RES 20 1085 986 82 BRETT MM ARCH DIS CHILD 61 1114 986 83 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 84 DASGUPTA MK J CLIN IMMUNOL 7 51 987 85 KOMIYAMA K CAN J MICROBIOL 33 27 987 86 BRETT MM ARCH DIS CHILD 62 357 987 PN 74008 RN 00008 AN 75009735 AU Hoiby-N. TI Epidemiological investigations of the respiratory tract bacteriology in patients with cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [B]. 1974 Aug. 82(4). P 541-50. MJ CYSTIC-FIBROSIS: mi. BACTERIA: ip. BACTERIA: ip. RESPIRATORY-TRACT-INFECTIONS: mi. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: th, dt. STREPTOCOCCUS-PNEUMONIAE: ip. ESCHERICHIA-COLI: ip. FOLLOW-UP-STUDIES. HAEMOPHILUS-INFLUENZAE: ip. HUMAN. INFANT. RESPIRATORY-THERAPY. KLEBSIELLA: ip. PROTEUS: ip. PSEUDOMONAS-AERUGINOSA: ip. SPUTUM: mi. STAPHYLOCOCCUS: ip. STREPTOCOCCUS: ip. SULFONAMIDES: tu. TIME-FACTORS. TRACHEA: mi. AB Seventy patients with cystic fibrosis treated as out-patients in a cystic fibrosis clinic have been followed during one year by monthly bacteriological examinations of tracheal secretions. The daily impression obtained in the laboratory is expressed by the mean point prevalence rate of Pseudomonas aeruginosa: 44 per cent, Staphylococcus aureus: 39 per cent, Haemophilus influenzae: 17 per cent, Diplococcus pneumoniae: 8 per cent, and miscellaneous other bacteria, mainly Enterobacteriaceae: 8 per cent. The fluctuations of the bacteriology are described by additional epidemiological terms: At one or more examinations during the study (period prevalence rate), 90 per cent of the patients harboured St. aureus, 64 per cent Ps. aeruginosa (mainly mucoid strains), 64 per cent H. influenzae, 37 per cent D. pneumoniae, and 30 per cent miscellaneous other bacteria, mainly Enterobacteriaceae. This pattern was found in all age groups with minor age-dependent modifications, especially as regards Enterobacteriaceae. Ps. aeruginosa was predominating as regards chronic colonization which reflects the most difficult therapeutic problems, the period prevalence rate being 39 per cent in contrast to St. aureus: 10 per cent, and H. influenzae: 1 per cent. St. aureus was predominating as regards new colonization and recolonization which reflect the problems of prevention, the incidence rate of new colonization and recolonization per risk group being 84 per cent, followed by H. influenzae: 62 per cent, Ps. aeruginosa: 43 per cent, and D. pneumoniae: 30 per cent. The results show that the bacteriological problems in cystic fibrosis are still considerable as regards therapy as well as prevention. Although many species may colonize the respiratory tracts of these patients, the main clinical problems concern Ps. aeruginosa and St. aureus; the reason why is discussed. RF 001 BURNS MW LANCET 1 270 968 002 COWAN ST MANUAL FOR THE IDENTIFICATION 965 003 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 004 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 005 DOSSETT JH J IMMUNOL 103 1405 969 006 FEIGELSON J MOD PROBL PEDIATR 10 214 967 007 GIFFORD RM J S CAROLINA MED ASSOC 68 204 972 008 GUSTAFSON GT J IMMUNOL 98 1178 967 009 HALBERT SP PEDIATRICS 26 792 960 010 HALBERT SP MOD PROBL PEDIATR 10 144 967 012 HUANG NN J PEDIATR 59 512 961 013 HUMPHREY JH ADV IMMUNOL 11 75 969 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 016 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 018 IACOCCA VF AM J DIS CHILD 106 315 963 019 IACOCCA VF ARCH DIS CHILD 43 220 968 020 JENSEN K THESIS 959 022 KILBOURN JP LANCET 2 878 970 023 LAWSON D IN: WATT PJ 69 970 024 MACMAHON B EPIDEMIOLOGY PRINCIPLES AND M 970 025 MADDOCKS JL LANCET 1 793 969 026 MARTIN RR J IMMUNOL 102 437 969 027 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 028 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 029 MAY JR ARCH DIS CHILD 47 908 972 030 MEARNS MB ARCH DIS CHILD 47 902 972 031 SCHWARTZ RH AM J DIS CHILD 111 408 966 032 SJOQUIST J IN: KILLANDER J 341 967 033 STALENHEIM G IMMUNOCHEMISTRY 10 501 973 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 2 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 3 HOIBY N SCAND J RESPIR DIS 56 38 975 4 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 5 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 6 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 328 975 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 8 HOIBY N ACTA PATH MICROBIOL SCAND (B) 83 549 975 9 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 10 WEEKE B DAN MED BULL 23 155 976 11 BATTEN J POSTGRAD MED J 52 571 976 12 HOIBY N SCAND J RESPIR DIS 57 37 976 13 HOIBY N SCAND J RESPIR DIS 57 103 976 14 VERON M BULL INSTITUT PASTEUR 74 295 976 15 WOOD RE AM REV RESPIR DIS 113 833 976 16 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 17 CLARKE CW CLIN ALLERGY 7 527 977 18 PIVETTA OH PEDIATR RES 11 1133 977 19 HOIBY N SCAND J RESPIR DIS 58 65 977 20 LAU WK PEDIATRICS 60 372 977 21 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 22 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 23 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 24 PARRY MF J PEDIATR 90 144 977 25 OLLING S SCAND J INFECT DIS 1977 7 977 26 SCHIOTZ PO HUM HERED 28 293 978 27 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 28 HAMPTON KD J CLIN MICROBIOL 9 632 979 29 HOIBY N MONOGR PAEDIATR 10 138 979 30 PAPORISZ U MONOGR PAEDIATR 10 31 979 31 FRIIS B SCAND J INFECT DIS 11 211 979 32 MAYBURY BA ANTIMICROB AGENTS CHEMOTHER 15 494 979 33 KRAUSE PJ CURR THER RES CLIN EXP 25 609 979 34 HOIBY N ACTA PAEDIATR SCAND 68 495 979 35 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 36 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 345 979 37 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 337 979 38 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 39 PAPORISZ U EUR J PEDIATR 130 259 979 40 KILBOURN JP PEDIATR RES 14 259 980 41 WOODS DE INFECT IMMUN 30 694 980 42 SKOV PS ALLERGY 35 23 980 43 MARTIN AJ ARCH DIS CHILD 55 604 980 44 HOIBY N ACTA PATH MICROBIOL SCAND (B) 88 125 980 45 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 46 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 47 PETERSEN NT ACTA PAEDIATR SCAND 70 623 981 48 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 49 BOYCE JR INFECT IMMUN 37 695 982 50 KOCH C ALLERGY 37 191 982 51 PERMIN H ALLERGY 37 93 982 52 MOLLER NE EUR J RESPIR DIS 63 130 982 53 SZAFF M ACTA PAEDIATR SCAND 71 821 982 54 KRAEMER R EUR J PEDIATR 138 172 982 55 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 56 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 57 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 58 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 59 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 60 ZIMAKOFF J J HOSP INFECT 4 31 983 61 KERCSMAR CM J ANTIMICROB CHEMOTHER 12 289 983 62 KLINGER JD INFECT IMMUN 39 1377 983 63 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 64 DORING G J INFECT DIS 147 744 983 65 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 66 LIEBERMAN MM SURV SYNTH PATHOL RES 4 312 985 67 SCULLY BE REV INFECT DIS 7 S669 985 68 STANISLAVSKY ES ACTA MICROBIOL HUNG 32 49 985 69 JACQUOT J INFECT IMMUN 47 555 985 70 BLUMER JL AM J MED 79 37 985 71 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 72 TABLAN OC J PEDIATR 107 382 985 73 HILL DJS MED J AUST 143 230 985 74 KRIEG DP J CLIN MICROBIOL 24 986 986 75 RILEY TV FEMS MICROBIOL LETTERS 33 55 986 76 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 PN 74009 RN 00009 AN 74087894 AU Franklin-J-L. Asquith-P. Rosenberg-I-H. TI The occurrence of cystic fibrosis and celiac sprue within a single sibship. SO Am-J-Dig-Dis. 1974 Feb. 19(2). P 149-55. MJ CYSTIC-FIBROSIS: fg. CELIAC-DISEASE: fg. MN ADULT. BIOPSY. CELIAC-DISEASE: co, fg. CHILD. CYSTIC-FIBROSIS: co. DIET-THERAPY. DILATATION. FEMALE. FLOCCULATION. GLUTEN: me. HUMAN. INTESTINAL-MUCOSA: pa, ra. INTESTINE-SMALL: pa, ra. JEJUNUM: pa. MALE. PEDIGREE. CELIAC-DISEASE: co, th. AB A family is presented in which celiac sprue and cystic fibrosis occurred within the same sibship. A cousin of the index case was also discovered to have celiac sprue. The genetics and incidence of both conditions is reviewed. It is estimated that the likelihood of this association occurring on the basis of chance in this family is 1 in 50,000. RF 001 HIDE DW ARCH DIS CHILD 44 533 969 002 ANTONOWICZ I PEDIATRICS 42 492 968 003 CAREY JB GASTROENTEROLOGY 46 550 964 004 FINLAY JM ANN INTERN MED 61 411 964 005 VAN DE KAMER JH J BIOL CHEM 177 347 949 006 RASKIN HF GASTROENTEROLOGY 34 996 959 007 GIBSON LE PEDIATRICS 23 545 959 008 ANDERSON B BR J HAEMATOL 6 439 960 009 GODWIN HA J CLIN INVEST 49 35A 970 010 HERBERT V J CLIN PATHOL 19 12 966 011 MATTHEWS DM J CLIN PATHOL 20 683 967 012 QUINTON WE GASTROENTEROLOGY 42 281 962 013 COOKE WT IN: GLASS GBJ 277 968 014 MACDONALD WC N ENGL J MED 272 448 965 015 RUBIN CE GASTROENTEROLOGY 39 260 960 016 MACDONALD WC GASTROENTEROLOGY 47 573 964 017 MCCONNELL RB GUT 12 592 971 018 DANKS DM ANN HUM GENET 28 323 965 019 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 020 BLACK JA ACTA PAEDIATR SCAND 53 109 964 021 MCCRAE WM ANN MED GENET 6 129 969 CT 1 SAFWENBERG J TISSUE ANTIGENS 10 287 977 PN 74010 RN 00010 AN 74154352 AU Burnell-R-H. Robertson-E-F. TI Cystic fibrosis in a patient with Kartagener syndrome. SO Am-J-Dis-Child. 1974 May. 127(5). P 746-7. MJ CYSTIC-FIBROSIS: co. KARTAGENER-TRIAD: co. MN CASE-REPORT. CHLORIDES: an. HUMAN. INFANT. LUNG: ra. MALE. SITUS-INVERSUS: co, ra. SODIUM: an. SWEAT: an. AB A patient exhibited the features of both Kartagener syndrome and cystic fibrosis. At most, to the authors' knowledge, this represents the third such report of the combination. Cystic fibrosis should be excluded before a diagnosis of Kartagener syndrome is made. RF 001 KARTAGENER M BEITR KLIN TUBERK 83 489 933 002 SCHWARZ V ARCH DIS CHILD 43 695 968 003 MACE JW CLIN PEDIATR 10 285 971 004 ANDERSEN DH AM J DIS CHILD 56 344 938 005 DI SANTAGNESE PA IN: NELSON WE 856 969 006 MILLER RD CHEST 62 130 972 007 KARTAGENER M ARCH PEDIATR 79 193 962 008 KATSUHARA K CHEST 61 56 972 009 CHURCHILL ED J THORAC SURG 18 279 949 010 LOGAN WD DIS CHEST 48 613 965 011 BROWN NM BR MED J 2 725 959 012 CALICETI G OTORINOLARINGOL ITAL 30 18 961 CT 1 BOCHKOVA DN GENETIKA (SOVIET GENETICS) 11 154 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 MOSSBERG B MT SINAI J MED 44 837 977 4 ROTT HD HUM GENET 46 249 979 5 BOWMAN BH HUM HERED 31 248 981 6 AFZELIUS BA CRC CRIT REV BIOCHEM 19 63 985 PN 74011 RN 00011 AN 75008123 AU Rachelefsky-G-S. Osher-A. Dooley-R-E. Ank-B. Stiehm-E-R. TI Coexistent respiratory allergy and cystic fibrosis. SO Am-J-Dis-Child. 1974 Sep. 128(3). P 355-9. MJ CYSTIC-FIBROSIS: co. RESPIRATORY-HYPERSENSITIVITY: co. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: dt. ASTHMA: et. CALIFORNIA. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: im. EOSINOPHILS. EPINEPHRINE: tu. FEMALE. GEL-DIFFUSION-TESTS. HAY-FEVER: et. HUMAN. IGA: an. IGE: an. IGG: an. IGM: an. ISOPROTERENOL: tu. LEUKOCYTE-COUNT. MALE. NASAL-POLYPS: et. RADIOIMMUNOASSAY. RESPIRATORY-HYPERSENSITIVITY: oc, im. AB The prevalence of respiratory allergies and their effects on the course of cystic fibrosis (CF) were studied in 63 patients. Fifteen patients (24%) had respiratory allergies as defined by history, physical examination, positive allergy skin tests, nasal and peripheral eosinophilia, and reversible airway obstruction. Serum immunoglobulins on these 15 allergic CF and 22 nonallergic CF patients were elevated when compared with those in normal age-matched controls. The allergic CF patients had significantly higher IgE levels (mean 598 international units [IU] compared to 281 IU); IgG, IgM, and IgA levels were similar in the two CF groups. The clinical condition of the allergic CF patients, (Shwachman score) was better than the nonallergic, suggesting some modulating effect of respiratory allergy on the severity of the disease. RF 001 ABBOTT V CAN MED ASSOC J 64 419 951 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 004 KULCZYCKI LL JAMA 175 358 961 005 MANCINI G COLLOQ PROTIDES BIOL FLUIDS 11 370 965 006 STIEHM ER PEDIATRICS 37 715 966 007 PILOT ML AM J CLIN PATHOL 20 870 950 008 SHWACHMAN H AM J DIS CHILD 96 6 958 009 GREEN MN AM J DIS CHILD 100 365 960 010 COLLINS-WILLIAMS C ANN ALLERGY 25 177 967 011 SCHWARTZ RH AM J DIS CHILD 111 408 966 012 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 013 TADA T J IMMUNOL 104 377 970 014 NEWCOMB RW J IMMUNOL 105 85 970 015 ISHIZAKA K IN: GOOD RA 84 971 016 JOHANSSON SGO LANCET 1 1118 968 017 POLMAR SH J CLIN INVEST 51 326 972 018 STITES DP CLIN EXP IMMUNOL 10 391 972 019 SCHWARTZ DP N ENGL J MED 284 513 971 020 LEVY DA N ENGL J MED 283 541 970 CT 1 HOLZEL A PRACTITIONER 214 776 975 2 WARNER JO ARCH DIS CHILD 51 507 976 3 STERN RC J PEDIATR 89 406 976 4 GALANT SP AM REV RESPIR DIS 114 325 976 5 PARTINGTON MW ARCH DIS CHILD 52 386 977 6 LIN MS J PEDIATR 91 222 977 7 SILVERMAN M ARCH DIS CHILD 53 873 978 8 GANIER M CLIN ALLERGY 9 125 979 9 PRICE JF CLIN ALLERGY 9 563 979 10 ZAMBIE MF ANN ALLERGY 42 290 979 11 SANTAGNESE PAD AM J MED 66 121 979 12 NELSON LA AM REV RESPIR DIS 120 863 979 13 SLY PD AUST PAEDIATR J 16 205 980 14 VANASPEREN PP AUST PAEDIATR J 16 53 980 15 HODSON ME THORAX 35 801 980 16 ORMEROD LP THORAX 35 768 980 17 SKOV PS ALLERGY 35 23 980 18 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 19 TOBIN MJ THORAX 35 232 980 20 TOBIN MJ THORAX 35 807 980 21 MOSS RB AM REV RESPIR DIS 121 23 980 22 HARPER TB LUNG 157 219 980 23 MATTHEWS WJ N ENGL J MED 302 245 980 24 SHEN J INFECT IMMUN 32 967 981 25 MOSS RB J PEDIATR 99 215 981 26 PITCHERWILMOTT RW ARCH DIS CHILD 57 582 982 27 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 28 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 29 BIRX DL ANN ALLERGY 53 124 984 30 WONNE R CLIN ALLERGY 15 455 985 31 SIEGEL SC J ALLERGY CLIN IMMUNOL 76 1 985 32 DARGA LL PEDIATR PULMONOL 2 82 986 PN 74012 RN 00012 AN 74154356 AU Fluge-G. Aarskog-D. TI Letter: Silver-Russell dwarfism and cystic fibrosis. SO Am-J-Dis-Child. 1974 May. 127(5). P 760-1. MJ BONE-DISEASES-DEVELOPMENTAL: co. CYSTIC-FIBROSIS: co. DWARFISM: co. MN CASE-REPORT. CHILD. FEMALE. HUMAN. SYNDROME. EX Taussig et al reported a case of a 6-year-old boy with the Russell variant of the Silver-Russell syndrome concomitant with cystic fibrosis. We would like to describe another patient who had the same combination of diseases. The pattern of clinical findings in this patient are in accordance with that of the Silver-Russell syndrome. It is, of course, possible that the development of cystic fibrosis in this patient is entirely accidental, although such a coincidence would be a statistically rare event. The establishment of a possible relationship between these two conditions would depend on reports of other similar cases. RF 001 TAUSSIG LM AM J DIS CHILD 125 495 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 MARIETTI G MINERVA PEDIATR 31 41 979 3 ROBICHAUX V ARCH PATHOL LAB MED 105 157 981 PN 74013 RN 00013 AN 74074555 AU Kulczycki-L-L. Schauf-V. TI Cystic fibrosis in blacks in Washington, DC: incidence and characteristics. SO Am-J-Dis-Child. 1974 Jan. 127(1). P 64-7. MJ CYSTIC-FIBROSIS: oc. NEGROID-RACE. MN CYSTIC-FIBROSIS: co, di, fg. DISTRICT-OF-COLUMBIA. HUMAN. POPULATION-SURVEILLANCE. AB An intensive search for blacks with cystic fibrosis (CF) in Washington, DC, during the period 1962 to 1971 disclosed 16 black patients, eight of whom were born in the District of Columbia during this decade. The calculated incidence of CF is at least one in 17,033 black newborns. The clinical course and pathological changes in blacks with CF are similar to those in whites. Genetic considerations point to autosomal recessive inheritance in blacks, which is generally accepted for whites with this disease. RF 001 CARTER CO MOD PROBL PEDIATR 10 372 967 002 WRIGHT SW IN: LAWSON D PROC 5TH INT CF 91 969 003 KULCZYCKI LL CLIN PEDIATR 3 692 964 004 ZUELZER WW PEDIATRICS 4 53 949 005 SENECAL JJ BULL MED AFRI OCCID FRANC 11 95 954 006 MACDOUGALL LG LANCET 2 409 962 007 DI SANTAGNESE PA ANN INTERN MED 54 482 961 008 PILEGGI A DELAWARE MED J 34 97 962 009 GROVE SS S AFR J LAB CLIN MED 5 113 959 010 LEVIN SE S AFR MED J 41 482 967 011 OPPENHEIMER EH PEDIATRICS 42 547 968 012 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 013 ANON VITAL STATISTICS OF THE US 51 971 014 ROBERTS GBS ANN HUM GENET 24 127 960 015 FELLER W INTRODUCTION TO PROBABILIT 957 016 KULCZYCKI LL AM J DIS CHILD 100 174 960 017 SELANDER P ACTA PAEDIATR SCAND 51 65 962 018 DANKS DM ANN HUM GENET 28 323 965 PN 74014 RN 00014 AN 74103902 AU Burton-B-K. Gerbie-A-B. Nadler-H-L. TI Present status of intrauterine diagnosis of genetic defects. SO Am-J-Obstet-Gynecol. 1974 Mar 1. 118(5). P 718-46. (REVIEW). MJ HEREDITARY-DISEASES: di. PRENATAL-DIAGNOSIS. MN ACID-PHOSPHATASE: me. ADRENAL-HYPERPLASIA-CONGENITAL: di. AMINO-ACID-METABOLISM-INBORN-ERRORS: di. AMNIOCENTESIS. AMNIOSCOPY. AMNIOTIC-FLUID. BLOOD-CHEMICAL-ANALYSIS. CARBOHYDRATE-METABOLISM-INBORN-ERRORS: di. CHROMOSOME-ABNORMALITIES: di. CYSTIC-FIBROSIS: di. ENDOSCOPY. FEMALE. FETUS: pa, ra. HUMAN. LESCH-NYHAN-SYNDROME: di. LIPID-METABOLISM-INBORN-ERRORS: di. LYSOSOMES: me. METABOLISM-INBORN-ERRORS: di. MUCOPOLYSACCHARIDOSIS: di. PREGNANCY. REVIEW. ULTRASONICS: du. URINE: an. EX Prenatal diagnosis of genetic disorders has received increasing attention during the past several years. Rapid expansion of our knowledge of the basis of genetic disease and the development of reliable diagnostic methods have led to rapid advances in the intrauterine diagnosis of a large number of inherited disorders. Prenatal diagnosis was initially used to determine the presence of Down's syndrome and other chromosomal abnormalities and for the determination of fetal sex in pregnancies at risk for a number of severe X-linked recessive disorders. Shortly thereafter, it became clear that the same techniques could be applied to the prenatal diagnosis of a number of the inborn errors of metabolism. Most recently, a number of new techniques have been developed that may prove to be of great value in the prenatal diagnosis of a number of severe congenital malformations that before now could not be diagnosed prior to birth. The goal of this review is to define the past achievements, future goals, and present limitations of intrauterine diagnosis. The first section will deal with the general approaches used in prenatal diagnosis of genetic disorders. This will be followed by a discussion of the specific applications of the basic techniques to the intrauterine diagnosis of chromosomal abnormalities and of the inborn errors of metabolism. Approaches to prenatal diagnosis of genetic disorders include amniocentesis, direct and indirect visualization of the fetus, analysis of maternal blood and urine, and sampling of fetal tissues. Applications of prenatal genetic diagnosis include X-linked recessive disorders, chromosomal abnormalities, inborn errors of metabolism, disorders of lipid metabolism, disorders of carbohydrate metabolism, disorders of mucopolysaccharide metabolism, disorders of amino acid metabolism, and miscellaneous disorders such as cystic fibrosis. These techniques provide some parents with methods for having children without fear of having a child with a specific deformity. In addition to the development of approaches which will increase the number of disorders detectable in utero, the potential for treatment of genetic disorders is unlimited. RF 001 NADLER HL N ENGL J MED 282 596 970 002 GERBIE AB AM J OBSTET GYNECOL 109 765 971 004 FREDA VJ BULL NY ACAD MED 42 475 966 005 QUEENAN JT CLIN OBSTET GYNECOL 9 491 966 006 BURNETT RG J IOWA MED SOC 58 130 958 007 LILEY AW NZ MED J 59 581 960 008 CREASMAN WT JAMA 204 949 968 010 FUCHS F CLIN OBSTET GYNECOL 9 565 966 011 ANON NATIONAL AMNIOCENTESIS REG 012 GEYER VH Z KLIN CHEM KLIN BIOCHEM 8 145 970 013 TORNQVIST A ACTA OBSTET GYNECOL SCAND 50 79 971 014 SALAFSKY IS J PEDIATR 79 794 971 015 SALAFSKY IS AM J OBSTET GYNECOL 111 1046 971 016 SCHNECK L LANCET 1 582 970 017 FRIEDLAND J PROC SOC EXP BIOL MED 136 1297 971 018 NADLER HL LANCET 2 1277 969 019 NADLER HL LANCET 2 369 970 020 EMERY AEH LANCET 1 1307 970 021 SCOTT CR PEDIATR RES 6 659 972 022 EMERY AEH LANCET 1 970 973 023 JEFFCOATE TNA LANCET 2 553 965 024 JACOBSON CB AM J OBSTET GYNECOL 99 796 967 025 NICHOLS J LANCET 1 83 970 026 MERKATZ IR J PEDIATR 75 977 969 027 KAUFMAN S J PEDIATR 68 990 966 028 HOLLINGSWORTH DR J PEDIATR 79 923 971 029 MATALON R LANCET 1 83 970 031 MORROW G 3RD J PEDIATR 77 120 970 032 DESNICK RJ MIDWEST SOC PEDIAT RES PAPER 972 033 BROCK DJH LANCET 2 197 972 034 LEE TY AM J OBSTET GYNECOL 107 917 970 035 CASSADY G AM J OBSTET GYNECOL 97 395 967 036 NADLER HL J PEDIATR 74 132 969 037 RATTAZZI MC IN: DORFMAN A 207 972 038 HUG G LANCET 1 1002 970 039 UHLENDORF BW PROC TISS CULT ASS 19TH AN MT 158 968 040 GREGSON NM LANCET 1 84 970 041 VALENTI C J LAB CLIN MED 73 355 969 042 NELSON MM BR MED J 1 523 970 044 NADLER HL BIOCHEM GENET 2 119 968 046 RYAN CA EXP CELL RES 71 388 972 047 KABACK MM PEDIATR RES 5 366 971 048 UHLENDORF BW SCIENCE 160 1007 968 049 SHIH VE LANCET 2 45 970 050 SHIH VE CLIN CHIM ACTA 27 73 970 051 GERBIE AB AM J OBSTET GYNECOL 114 314 972 052 MELANCON S SCIENCE 173 627 971 053 BARNHISEL ML J CELL PHYSIOL 76 7 970 055 KABACK MM IN: DORFMAN A 81 972 056 RUSSELL JGB J OBSTET GYNAECOL BR COMMONW 76 345 969 057 NOONAN CD AM J OBSTET GYNECOL 101 929 968 058 OMENN GS AM J HUM GENET 24 31A 972 059 QUEENAN JT OBSTET GYNECOL 35 648 970 060 AGUERO O SURG GYNECOL OBSTET 130 649 970 061 AGUERO O AM J OBSTET GYNECOL 107 971 970 062 ERBSLOH J ARCH GYNAEKOL 173 160 942 063 WIESENHAAN PF AM J OBSTET GYNECOL 113 819 972 064 GARRETT WJ AUST NZ J OBSTET GYNAECOL 10 7 970 065 CAMPBELL S LANCET 2 1226 972 066 THIERY M LANCET 1 599 973 067 PAPP Z LANCET 1 729 973 068 HELLMAN LM AM J OBSTET GYNECOL 115 615 973 070 TETERIS NJ OBSTET GYNECOL 32 851 968 071 LEVKOFF AH AM J OBSTET GYNECOL 104 73 969 072 CATHRO DM LANCET 1 732 969 073 NICHOLS J LANCET 2 1068 967 074 WALKNOWSKA J LANCET 1 1119 969 075 TOWNER JW CLIN RES 18 208 970 076 MOHR J ACTA PATH MICROBIOL SCAND 73 73 968 077 VALENTI C AM J OBSTET GYNECOL 115 851 973 078 KAN YW N ENGL J MED 287 1 972 079 RIIS P LANCET 2 180 960 080 RIIS P IN: MOORE KL 966 081 CEDERQVIST LL CLIN OBSTET GYNECOL 13 159 970 082 CASPERSSON T CHROMOSOMA 30 215 970 083 VALENTI C LANCET 2 220 968 084 GERTNER M PROC AM PEDIATR SOC ANNU MTG 125 970 085 MILUNSKY A N ENGL J MED 283 1370 970 086 HSU LYF AM J DIS CHILD 125 290 973 087 MILUNSKY A J PEDIATR 79 303 971 088 KOHN G LANCET 2 778 970 089 MILUNSKY A LANCET 2 979 970 090 KOHN G PEDIATR RES 1 461 967 091 KLINGER HP J BIOPHYS BIOCHEM CYTOL 8 345 960 092 BOOK JA J MED GENET 5 224 968 093 SCHLEGEL RJ CYTOGENETICS 5 430 968 094 KARDON NB J PEDIATR 80 297 972 095 OBRIEN JS J PEDIATR 75 167 969 096 OBRIEN JS AM J DIS CHILD 109 338 965 097 OKADA S SCIENCE 160 1002 968 098 SINGER HS N ENGL J MED 282 571 970 099 SLOAN HR PEDIATR RES 3 532 969 100 THOMAS GH J LAB CLIN MED 74 725 969 101 OBRIEN JS LANCET 2 805 969 102 LOWDEN JA N ENGL J MED 288 225 973 103 KABACK MM J PEDIATR 82 1037 973 104 OBRIEN JS N ENGL J MED 284 893 971 105 WOLFE LS NEUROLOGY (MINN) 20 23 970 106 SINGER HS AM J HUM GENET 24 454 972 107 BOOTH CW PEDIATRICS 52 521 973 108 OKADA S SCIENCE 165 698 969 109 OBRIEN JS N ENGL J MED 283 15 970 110 OBRIEN JS SCIENCE 172 61 971 111 NAVON R AM J HUM GENET 25 287 973 112 SANDHOFF K LIFE SCI 7 283 968 113 OKADA S AM J HUM GENET 23 55 971 114 BRADY RO PROC NAT ACAD SCI USA 55 366 966 115 FREDRICKSON DS IN: STANBURY JB 783 972 116 EPSTEIN CJ AM J HUM GENET 23 533 971 117 BRADY RO BIOCHEM BIOPHYS RES COMMUN 18 221 965 118 BEUTLER E AM J HUM GENET 23 62 971 120 JATZKEWITZ H J NEUROCHEM 16 19 969 121 KABACK MM N ENGL J MED 282 1336 970 122 PORTER MT PROC NAT ACAD SCI USA 62 887 969 123 PERCY AK SCIENCE 161 594 968 124 AUSTIN J ARCH NEUROL 14 259 966 125 AUSTIN J ARCH NEUROL 13 593 965 126 MURPHY JV IN: BERNSOHN J 67 971 127 SUZUKI K PROC NAT ACAD SCI USA 66 302 970 128 SUZUKI K BIOCHEM BIOPHYS RES COMMUN 45 1363 971 129 BRADY RO N ENGL J MED 276 1163 967 130 BRADY RO SCIENCE 172 174 971 131 KINT JA SCIENCE 167 1268 970 132 BEUTLER E AM J HUM GENET 24 237 972 133 WOOD S AM J HUM GENET 24 250 972 134 HERNDON JH J CLIN INVEST 48 1017 969 135 HERNDON JH N ENGL J MED 281 1034 969 136 NADLER HL AM J OBSTET GYNECOL 103 710 969 137 DAWSON G SCIENCE 170 556 970 138 HERS HG BIOCHEM J 86 11 963 139 NITOWSKY HM J LAB CLIN MED 69 472 967 140 COX RP LANCET 2 893 970 141 STEINITZ K ISR J MED SCI 3 411 967 142 JUSTICE P BIOCHEM BIOPHYS RES COMMUN 39 301 970 143 HOWELL RR PROC AM PEDIATR SOC ANNU MTG 130 970 144 KROOTH RS J EXP MED 113 1155 961 145 NADLER HL PEDIATRICS 42 912 968 147 VAN HOOF F LANCET 1 1198 968 148 DANES BS SCIENCE 149 987 965 149 MILUNSKY A N ENGL J MED 281 1128 969 150 MATALON R LANCET 1 798 972 151 FRATANTONI JC PROC NAT ACAD SCI USA 60 699 968 152 FRATANTONI JC N ENGL J MED 280 686 969 153 FRATANTONI JC SCIENCE 162 570 968 154 SLY WS J PEDIATR 82 249 973 155 WIRTSCHAFTER ZT AM J OBSTET GYNECOL 76 1219 958 156 LEVY HL PEDIATR RES 3 113 969 157 COCKBURN F J OBSTET GYNAECOL BR COMMONW 80 10 973 158 SAIFER A CLIN CHEM 16 891 970 159 MAHONEY MJ IN: DORFMAN A 95 972 160 MAHONEY MJ PEDIATR RES 7 342 973 161 FRIMPTER GW AM J DIS CHILD 113 115 967 162 SCHULMAN JD J PEDIATR 77 468 970 163 SCHNEIDER JA PEDIATR RES 7 291 973 164 DANCIS J IN: DORFMAN A 123 972 165 GOODMAN SI CLIN RES 21 295 973 166 GOMPERTZ D LANCET 1 1009 973 167 SEEGMILLER JE SCIENCE 155 1682 967 168 ROSENBLOOM FM LANCET 2 305 967 169 FUJIMOTO WY LANCET 2 511 968 170 DEMARS R SCIENCE 164 1303 969 171 BOYLE JA SCIENCE 169 688 970 172 LEROY JG BIRTH DEF ORIG ART SER 5 174 969 173 GLASER JH PEDIATR RES 7 342 973 174 WARREN RJ PEDIATR RES 7 343 973 175 DANES BS LANCET 1 1061 968 176 NADLER HL LANCET 2 84 969 177 BOWMAN BH SCIENCE 164 325 969 178 SPOCK A PEDIATR RES 1 173 967 179 DANES BS J EXP MED 136 1313 972 180 BARNETT DR FED PROC 32 677 973 181 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 182 CONOVER JH CLIN RES 21 531 973 183 NADLER HL N ENGL J MED 282 302 970 184 ROMEO G BIOCHEM GENET 4 659 970 185 REGAN JD SCIENCE 174 147 971 186 MATALON R BIOCHEM BIOPHYS RES COMMUN 47 959 972 187 BACH G PROC NAT ACAD SCI USA 69 2048 972 188 BACH G FED PROC 32 483 973 189 MATALON R PEDIATR RES 7 384 973 190 OBRIEN JS PROC NAT ACAD SCI USA 69 1720 972 191 VON FIGURA K BIOCHEM BIOPHYS RES COMMUN 48 262 972 193 HSIA YR PROC AM PEDIATR SOC ANNU MTG 26 970 CT 1 HENRION R NOUV PRESSE MED 3 2651 974 2 KUSS E GYNAKOLOGE 7 124 974 3 ZELLWEGER H ACTA GENET MED GEMELLOL 23 103 974 4 LOWRY RB CAN MED ASSOC J 111 633 974 5 LEVINE MD AM J OBSTET GYNECOL 120 937 974 6 KLEIJER WJ HISTOCHEM J 7 496 975 7 PHILLIPS JM J REPROD MED 15 69 975 8 NIERMEIJER MF J GENET HUM 23 123 975 9 NUZZO F J GENET HUM 23 225 975 10 TABUCHI A HIROSHIMA J MED SCI 24 213 975 11 POTIER M BIOL NEONATE 27 141 975 12 NEUFELD EF ANNU REV BIOCHEM 44 357 975 13 GERBIE AB OBSTET GYNECOL 46 716 975 14 CHAUBE S AM J OBSTET GYNECOL 121 429 975 15 BICKEL H MONATSSCHR KINDERHEILKD 123 206 975 16 NIERMEIJER MF J MED GENET 13 182 976 17 POTIER M BIOMEDICINE EXPRESS 25 167 976 18 TANCREDI F MINERVA PEDIATR 28 1891 976 19 LAURENCE KM BR MED BULL 32 9 976 20 MACVICAR J BR MED BULL 32 4 976 21 KLEIJER WJ HUM GENET 33 299 976 22 CATERINI H OBSTET GYNECOL 47 373 976 23 ELIAS S OBSTET GYNECOL 47 S 75 976 24 GERBIE AB POSTGRAD MED 59 129 976 25 HULTBERG B BIOCHEM J 155 599 976 26 TOULOUKIAN RJ J PEDIATR SURG 12 397 977 27 SANDHOFF K ANGEWANDTE CHEMIE INTL ED 16 273 977 28 AUDY S ACTA CYTOL 21 330 977 29 ANTONOWICZ I BIOL NEONATE 32 280 977 30 NELSON LH OBSTET GYNECOL 50 65 977 31 LIEBAERS I J PEDIATR 90 423 977 32 GERBIE AB AM J OBSTET GYNECOL 127 158 977 33 BELISLE S AM J OBSTET GYNECOL 128 514 977 34 LEVINE SC CLIN GENET 14 133 978 35 MILES JH PEDIATR CLIN NORTH AM 25 593 978 36 VANDERVEER E HUM GENET 40 285 978 37 BURTON BK PEDIATRICS 61 398 978 38 GOLBUS MS CLIN PERINATOL 6 245 979 39 SIMPSON JL CLIN OBSTET GYNAECOL 6 259 979 40 FELIX JS CLIN GENET 15 215 979 41 TSVETKOVA IV VOPR MED KHIM 25 214 979 42 MARTIN AO CLIN OBSTET GYNAECOL 7 143 980 43 SANTOS M REV MED CHIL 109 36 981 44 CHEN WW J MED GENET 19 433 982 45 SHARMA A CURR SCI 52 704 983 PN 74015 RN 00015 AN 74129630 AU Dooley-R-R. Braunstein-H. Osher-A-B. TI Polypoid cervicitis in cystic fibrosis patients receiving oral contraceptives. SO Am-J-Obstet-Gynecol. 1974 Apr 1. 118(7). P 971-4. MJ CERVICITIS: ci. CERVIX-NEOPLASMS: ci. CONTRACEPTIVES-ORAL: ae. CYSTIC-FIBROSIS: co. POLYPS: ci. MN ADULT. BIOPSY. CERVICITIS: co. CERVIX-NEOPLASMS: co, pa. ETHINYL-ESTRADIOL: ae. FEMALE. HUMAN. MEDROXYPROGESTERONE: ae. NEUTROPHILS. NORETHINDRONE: ae. POLYPS: co, pa. RESPIRATORY-INSUFFICIENCY: co. AB Three of the 4 women in a cystic fibrosis clinic who used a combined form of oral contraceptive developed acute polypoid cervicitis. In one woman, the polyp recurred on reinstitution of the medication. There was resolution of the polyp in each instance on discontinuation of the drugs. In 2 of the 3 affected women, there was coincidental deterioration of respiratory status. Therefore, it is suggested that cystic fibrosis patients who are using oral contraception be observed closely regarding pulmonary and gynecologic status. RF 001 OPPENHEIMER EA AM J OBSTET GYNECOL 108 673 970 002 OPPENHEIMER EH J PEDIATR 77 991 970 003 TAYLOR HB JAMA 202 637 967 004 GOVAN ADT J CLIN PATHOL 22 84 969 005 GALL SA JAMA 207 2243 969 006 TALBERT JR AM J OBSTET GYNECOL 105 117 969 007 KYRIAKOS M CANCER 23 99 968 008 GRAHAM J OBSTET GYNECOL 31 190 968 009 MAQUEO M AM J OBSTET GYNECOL 96 994 966 010 BLAIR GWS BIOCHEM J 35 1039 941 011 KOPITO LE FERTIL STERIL 24 499 973 CT 1 GREENSTEIN JS CONTRACEPTION 11 105 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 ANASTASIADIS P GEBURTSHILFE FRAUENHEILKD 38 45 978 4 FITZPATRICK SB CHEST 86 863 984 5 STEAD RJ THORAX 42 59 987 PN 74016 RN 00016 AN 74107099 AU Ozonoff-M-B. TI Ataxia-telangiectasia: chronic pneumonia, sinusitis, and adenoidal hypoplasia. SO Am-J-Roentgenol-Radium-Ther-Nucl-Med. 1974 Feb. 120(2). P 297-9. MJ ADENOIDS: ra. ATAXIA-TELANGIECTASIA: co. PNEUMONIA: et. SINUSITIS: et. MN ATAXIA-TELANGIECTASIA: ra. CASE-REPORT. CHILD. CYSTIC-FIBROSIS: ra. DIAGNOSIS-DIFFERENTIAL. ETHMOID-SINUS: ra. FEMALE. FRONTAL-SINUS: ab, ra. HUMAN. MAXILLARY-SINUS: ra. PNEUMONIA: ra. EX The syndrome of ataxia-telangiectasia is characterized by ataxia, progressive neurologic degeneration, cutaneous and conjunctival telangiectasis, and immunologic deficiency. Roentgenologic evidence of chronic pulmonary and sinus infections and decreased lymphoid tissue will usually be present. As the sinopulmonary abnormalities resemble those of cystic fibrosis, a small adenoidal mass may be of differential aid. RF 001 BODER E PEDIATRICS 21 526 958 002 EISEN AH N ENGL J MED 272 18 965 003 KARPATI G AM J DIS CHILD 110 51 965 004 TADJOEDIN MK AM J DIS CHILD 110 64 965 CT 1 SHACKELFORD GD AM J ROENTGENOL 123 144 975 2 LALLEMAND D ANN RADIOL (PARIS) 24 67 981 PN 74017 RN 00017 AN 75144214 AU Schwartz-E-E. Holsclaw-D-S. TI Pulmonary involvement in adults with cystic fibrosis. SO Am-J-Roentgenol-Radium-Ther-Nucl-Med. 1974 Dec. 122(4). P 708-18. MJ CYSTIC-FIBROSIS: ra. LUNG: ra. MN ADOLESCENCE. ADULT. ATELECTASIS: ra. CYSTIC-FIBROSIS: co, pp. FEMALE. HEMOPTYSIS: ra. HUMAN. HYPERTENSION-PULMONARY: ra. MALE. PNEUMONIA: ra. PNEUMOTHORAX: ra. PULMONARY-HEART-DISEASE: ra. SPIROMETRY. EX Cystic fibrosis is no longer rare in adults. The lung is the main target organ and the basic lesion is bronchial obstruction leading to bronchiectasis. Interstitial infiltrates and cyst-like changes are characteristic on roentgenography. Complications include pulmonary hypertension, pneumonitis, hemorrhage, and pneumothorax. Little correlation exists between respiratory tract and pancreatic and gastrointestinal involvement, despite similarities in pathogenesis. Pulmonary cystic fibrosis in adults does not always resemble the condition in children, and must be differentiated from other chronic interstitial diseases. RF 002 CABANEL G J FRANC MED CHIR THORAC 21 245 967 003 COATES EO JR DIS CHEST 49 195 966 004 DI SANTAGNESE PA PEDIATRICS 12 178 953 005 DOERSHUK CF PEDIATRICS 36 675 965 006 ESTERLY JR THORAX 23 670 968 007 GYEPES MT AM J ROENTG RAD THER NUCL MED 106 567 969 008 HODSON CJ CLIN RADIOL 13 54 962 009 HOLSCLAW DS J PEDIATR 76 829 970 010 JONES JS BR J DIS CHEST 64 25 970 011 KEATS TE RADIOLOGY 65 223 955 012 KIRKPATRICK JA JR SEMIN ROENTGENOL 7 149 972 013 MCKUSICK VA MENDELIAN INHERIT IN MAN 167 966 014 MOSS AJ MOD PROBL PEDIATR 10 187 967 015 NOVY MJ OBSTET GYNECOL 30 530 967 016 REID L MOD PROBL PEDIATR 10 195 967 017 REILLY BJ RADIOLOGY 98 281 971 018 ROSENOW EC 3RD JAMA 203 227 968 019 SHWACHMAN H PEDIATRICS 36 689 965 020 SHWACHMAN H IN: KENDIG EL JR 1 524 972 021 SIMON G RADIOL CLIN NORTH AM 11 3 973 022 SINGLETON EB RADIOLOGIC ATLAS OF PULMONARY 136 971 023 SPENCER H PATHOLOGY OF THE LUNG 968 024 STUR O FORTSCHR GEB ROENTG NUKL 99 625 963 025 TAUSSIG LM RADIOLOGY 106 369 973 026 TOMASHEFSKI JF CHEST 57 28 970 027 ZAPLETAL A PEDIATRICS 48 64 971 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 LUCK SR J THORAC CARDIOVASC SURG 74 834 977 3 SANTAGNESE PAD AM J MED 66 121 979 4 DAVIS PB AM J MED 69 643 980 5 FICK RB CLIN CHEST MED 2 91 981 6 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 7 KISTLER I HELV PAEDIATR ACTA 36 495 982 8 LEWIS MI S AFR MED J 65 641 984 9 REINIG JW PEDIATR RADIOL 15 222 985 10 SEAMAN WB HOSP PRACT 20 123 985 11 TOMASHEFSKI JF AM REV RESPIR DIS 133 535 986 PN 74018 RN 00018 AN 74109137 AU Biggar-W-D. Holmes-B. Good-R-A. TI Letter: Selective inhibition of phagocytic activity of rabbit alveolar macrophages by cystic fibrosis serum. SO Am-Rev-Respir-Dis. 1974 Mar. 109(3). P 403. MJ CYSTIC-FIBROSIS: bl. MACROPHAGES: im. PHAGOCYTOSIS. PULMONARY-ALVEOLI: cy. MN CYSTIC-FIBROSIS: im. HUMAN. PSEUDOMONAS-AERUGINOSA. RABBITS. EX As far as rabbit alveolar macrophages are concerned, the concentration of homozygous CF serum used in our experiments was even greater than the fourfold concentration which Biggar and associates state was required for normal phagocytosis. Increasing dilutions of homozygous CF sera with either Hanks balanced salt solution or normal human serum, thus decreasing its concentration, increased the capacity of the rabbit alveolar macrophages to phagocytize and kill Pseudomonas aeruginosa. Increasing concentrations had just the opposite effect. Suspension of rabbit alveolar macrophages in human serum reduced their phagocytic activity, but activity was reduced more by human CF serum than by human normal serum. This is not unexpected because high human serum concentrations are likely to be toxic to rabbit alveolar macrophages. Because the inhibition of phagocytosis was absent when homozygous CF serum more than 24 hours old or previously heated or frozen was used and because of our observations that dilution increased macrophage phagocytic ability, we feel our data demonstrate the presence of a labile inhibitor rather than a deficiency. Since it is well known that CF patients have normal or elevated concentrations of immunoglobulins including IgA in their sera and pulmonary secretions and respond to infection, and IgA has not been shown to be essential for alveolar macrophage phagocytosis of Pseudomonas aeruginosa, we cannot agree that this inhibition is due to an IgA deficiency. We also note that some of the sera used in the experiments of Biggar and associates were stored at -70 degrees C. Perhaps this may account for the difference in results. RF 001 BOXERBAUM B AM REV RESPIR DIS 108 777 973 002 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 3 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 PN 74019 RN 00019 AN 75012012 AU Coburn-M-D. Coburn-L-A. Solomons-C-C. TI Letter: Plasma arginine esterase activity in cystic fibrosis. SO Am-Rev-Respir-Dis. 1974 Sep. 110(3). P 368-70. MJ CYSTIC-FIBROSIS: en. ESTERASES: bl. MN ADULT. ARGININE. CHILD. CYSTIC-FIBROSIS: bl. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. HUMAN. PROTEINS: me. EX We assayed the serum of a group of cystic fibrosis patients that were on oral arginine therapy and that of a group of cystic fibrosis patients that were not receiving this treatment for total arginine esterase activity. The untreated cystic fibrosis patients have significantly lower serum arginine esterase activity than the normal controls, the cystic fibrosis carriers, and the cystic fibrosis patients receiving oral arginine therapy. There is no significant difference between the arginine esterase activity of the normal controls and the cystic fibrosis carriers or the cystic fibrosis patients treated with arginine. - We have recently observed differences in the arginine esterases in plasma of controls and patients by electrofusing on polyacrylamide gels. Six activity bands could be detected in plasma of controls, whereas only five could be detected in plasma of patients. Our work on the deficiency of trypsin-like activity in mixed saliva of patients with cystic fibrosis has been reproduced by another laboratory. Coburn recently confirmed our work on deficiency of arginine esterase in serum of patients with cystic fibrosis. At present, the deficiency of arginine esterase in cystic fibrosis is a working hypothesis to understand the basic defect. - The variations of pH and sodium chloride content that I employed had been tested by me in 6 patients both with and without a diagnosis of cystic fibrosis and were found not to alter the level of arginine-esterase activity. The major differences between the two studies resulted from the control subjects. Dr. Rao neglects to mention the few other laboratories that were unable to confirm his findings. RF 001 LIEBERMAN J AM REV RESPIR DIS 109 399 974 002 RAO GJS SCIENCE 177 610 972 004 SOLOMONS CC PEDIATRICS 47 384 971 005 COTTON EK CF CLUB ABST 15 974 006 LIEBERMAN J AM REV RESPIR DIS 109 399 974 007 RAO GJS SCIENCE 177 610 972 008 COLMAN RW J CLIN INVEST 48 23 969 009 RAO GJS ACSI MTG ATLANTIC CITY 974 010 RAO GJS J PEDIATR 80 573 972 011 SCHONI M CF Q ANNOTATED REFERENCES 12 54 973 CT 1 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 2 DISANTAGNESE PA N ENGL J MED 295 597 976 3 GOLDSMITH GH J LAB CLIN MED 89 131 977 4 DANN LG LANCET 2 405 978 5 RAO GJS ENZYME 23 314 978 6 BURY AF PEDIATR RES 16 613 982 7 BRIDGES MA ANN NY ACAD SCI 421 360 983 PN 74020 RN 00020 AN 74146275 AU Cooper-D-M. Doron-I. Mansell-A-L. Bryan-A-C. Levison-H. TI The relative sensitivity of closing volume in children with asthma and cystic fibrosis. SO Am-Rev-Respir-Dis. 1974 May. 109(5). P 519-24. MJ ASTHMA: pp. CYSTIC-FIBROSIS: pp. LUNG: pp. RESPIRATION. MN ADOLESCENCE. ASTHMA: di. CHILD. HUMAN. OXYGEN: bl. PLETHYSMOGRAPHY-WHOLE-BODY. SPIROMETRY. AB The ability to detect abnormalities of lung function in children with asthma and cystic fibrosis by the measurement of closing volume was studied in 205 children. Flow volume characteristics, routine spirometry, body plethysmography, and arterialized oxygen tensions were studied for comparison with closing volume. Closing volume was less sensitive in detecting disease than were the residual volume to total lung capacity ratio, maximum expiratory flow volume curves, and arterialized blood gases. RF 001 MCCARTHY D AM REV RESPIR DIS 107 559 973 002 MCCARTHY DS AM J MED 52 747 972 003 MANSELL A J APPL PHYSIOL 33 711 972 004 LEBLANC P J APPL PHYSIOL 28 448 970 005 DUBOIS AB J CLIN INVEST 35 322 956 006 DUBOIS AB J CLIN INVEST 35 327 956 007 WENG TR AM REV RESPIR DIS 99 879 969 008 LEVISON H ACTA PAEDIATR SCAND 59 648 970 009 FEATHERBY EA AM REV RESPIR DIS 102 737 970 010 WENG TR AM REV RESPIR DIS 99 719 969 011 MANSELL A PHYSIOLOGIST 15 207 972 013 MANSELL A FED PROC 32 402 973 014 GOLD WM J APPL PHYSIOL 23 433 967 015 MANSELL A PEDIATR RES 7 435 973 016 ZAPLETAL A PEDIATRICS 48 64 971 017 HILL DJ ARCH DIS CHILD 47 874 972 018 ZAPLETAL A J APPL PHYSIOL 26 308 969 019 WENG TR ANN ALLERGY 27 565 969 CT 1 GOTZ M PAEDIATR PAEDOL 10 466 975 2 HALL DR BULL EUR PHYSIOPATH RESP 11 863 975 3 MELLIS CM AUST PAEDIATR J 12 77 976 4 HYDE JS JAMA 235 1125 976 5 COOPER DM CHEST 71 361 977 6 PROCTOR DF AM REV RESPIR DIS 115 97 977 7 KERREBIJN HF ACTA PAEDIATR SCAND SUPPL 261 1977 7 977 8 KERREBIJN KF BR MED J 1 886 978 9 WONNE R MONATSSCHR KINDERHEILKD 126 165 978 10 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 11 LANDAU LI THORAX 34 217 979 12 SANTAGNESE PAD AM J MED 66 121 979 13 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 14 LARSEN GL AM REV RESPIR DIS 119 399 979 15 KEENS TG PEDIATRICS 65 1013 980 16 LARSEN GL AM J DIS CHILD 134 1143 980 17 VANASPEREN P AM J DIS CHILD 135 815 981 18 MILNER AD BR J HOSP MED 28 89 982 19 KONIG P ANN ALLERGY 49 86 982 20 MOK JYQ EUR J RESPIR DIS 64 487 983 PN 74021 RN 00021 AN 75052992 AU Dolan-T-F-Jr. Meyers-A. TI Bronchial asthma and allergic rhinitis associated with inhalation of pancreatic extracts. SO Am-Rev-Respir-Dis. 1974 Dec. 110(6). P 812-3. MJ ALLERGENS. ASTHMA. CYSTIC-FIBROSIS: dt. DRUG-HYPERSENSITIVITY. HAY-FEVER. PANCREATIC-EXTRACTS: ae. MN ADULT. CHILD. CYSTIC-FIBROSIS: im. ENVIRONMENTAL-EXPOSURE. FOOD. HUMAN. MALE. PANCREATIC-EXTRACTS: tu. POWDERS. AB Five parents of children with cystic fibrosis developed allergic bronchospasm after inhaling pancreatic extracts sprinkled on their children's food. There was a positive history of allergy, but no past history of asthma in any of the 5 parents. Asthma responded to bronchodilators, and use of a face mask during preparation of meals prevented recurrences. To date, asthma precipitates by inhalation of pancreatic extracts has not been seen in any of the patients with cystic fibrosis. RF 001 MATTHEWS LW IN: MANGOS JA 303 973 002 DERBES VJ J ALLERGY 28 287 957 003 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 004 YOHE RM ANN ALLERGY 30 627 972 005 DAY G ARCH DIS CHILD 48 355 973 CT 1 BERGNER A PEDIATRICS 55 814 975 2 PILAT L AM REV RESPIR DIS 112 275 975 3 ABERNATHY RS PEDIATRICS 58 141 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 TWAROG FJ J ALLERGY CLIN IMMUNOL 59 35 977 6 SAKULA A BR J DIS CHEST 71 295 977 7 ROMEO G NATURE 274 909 978 8 GANIER M CLIN ALLERGY 9 125 979 9 ROMEO G PEDIATR RES 13 1030 979 10 ORMEROD LP THORAX 35 768 980 11 DAVIES RJ SEM RESPIR MED 5 229 984 12 LANKISCH PG CLIN GASTROENTEROL 13 985 984 13 BAUR X DTSCH MED WSCHR 109 257 984 14 WIESSMANN KJ EUR J RESPIR DIS 66 13 985 15 PAULI G BULL EUR PHYSIOPATH RESP 22 399 986 16 WARREN CPW AM J MED 81 939 986 17 MOIRA CY AM REV RESPIR DIS 133 686 986 PN 74022 RN 00022 AN 74122409 AU Doershuk-C-F. Fisher-B-J. Matthews-L-W. TI Specific airway resistance from the perinatal period into adulthood. Alterations in childhood pulmonary disease. SO Am-Rev-Respir-Dis. 1974 Apr. 109(4). P 542-7. MJ AGE-FACTORS. AIRWAY-RESISTANCE. CYSTIC-FIBROSIS: pp. LUNG-DISEASES: pp. PULMONARY-DIFFUSING-CAPACITY. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: ra. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. LUNG: ra. MALE. RESPIRATORY-FUNCTION-TESTS. SEX-FACTORS. VENTILATION-PERFUSION-RATIO. AB Airway resistance and lung volume were measured by a modification of the plethysmographic method of Briscoe and DuBois (1) to calculate specific airway resistance (SRaw) on 263 control subjects ranging in age from the newborn to 54 years. the mean SRaw was lowest in the newborn period and increased rapidly to adult levels during the first years of life. In each age group, the mean SRaw was lower for females than it was for males. No control subject had an SRaw greater than 8 sec cm H2O. A decrease in mean SRaw from 4.2 sec cm H2O for females 5 to 9 years of age to 3.5 sec cm H2O for those over 18 years of age paralleled sexual maturation, but similar changes were not observed in the males. A direct correlation of SRaw with clinical observations was found in young patients with various degrees of pulmonary involvement because of cystic fibrosis. RF 001 BRISCOE WA J CLIN INVEST 37 1279 958 002 LLOYD TC JR AM REV RESPIR DIS 87 529 963 003 PAYNE CB AM J MED 42 554 967 004 DOERSHUK CF PEDIATR RES 4 165 970 005 DUBOIS AB J CLIN INVEST 35 322 956 006 DUBOIS AB J CLIN INVEST 35 322 956 007 DRAPER NR APPLIED REGRESSION ANALYSIS 966 008 DOERSHUK CF J PEDIATR 65 677 964 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 POLGAR G J PEDIATR 67 557 965 011 KRIEGER I PEDIATRICS 33 45 964 012 WOHL MEB PEDIATRICS 43 495 969 013 PHELAN PD ARCH DIS CHILD 44 393 969 CT 1 HATCH DJ ARCH DIS CHILD 51 859 976 2 HIBBERT ME J APPL PHYSIOL 57 304 984 PN 74023 RN 00023 AN 75010757 AU Boat-T-F. Kleinerman-J-I. Carlson-D-M. Maloney-W-H. Matthews-L-W. TI Human respiratory tract secretions. 1. Mucous glycoproteins secreted by cultured nasal polyp epithelium from subjects with allergic rhinitis and with cystic fibrosis. SO Am-Rev-Respir-Dis. 1974 Oct. 110(4). P 428-41. MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: se. HAY-FEVER: me. MUCOPROTEINS: se. NASAL-POLYPS: me. MN ADULT. AMINO-ACIDS: an. AUTORADIOGRAPHY. CHILD. CHROMATOGRAPHY-DEAE-CELLULOSE. EPITHELIUM: se. CHROMATOGRAPHY-GEL. GLYCOPROTEINS: ip, an. HUMAN. MOLECULAR-WEIGHT. MUCOPROTEINS: ip, an. SULFUR-RADIOISOTOPES. TISSUE-CULTURE. TRITIUM. AB Ciliated nasal polyp epithelium from patients with allergic rhinitis and patients with cystic fibrosis was maintained in organ culture for 168 hours. Explanted epithelium retained its morphologic characteristics and incorporated 35SO4 and 3H into blood group specific glycoproteins that were secreted into the medium throughout the culture period. Labeled mucous glycoprotein was solubilized by reduction and alkylation, then purified by gel filtration chromatography. Purified glycoprotein was further separated on diethylaminoethyl (DEAE) cellulose columns into 3 blood group active fractions. These fractions had similar amino acid compositions but differed in N-acetyl neuraminic acid and fucose content and in relative incorporation of 35SO4 and 3H-glucosamine. Blood group glycoproteins from polyp epithelium of patients with allergic rhinitis and cystic fibrosis were indistinguishable in many respects. However, the 35SO4 per 3H ratio of all 3 glycoprotein fractions from patients with cystic fibrosis exceeded that of the corresponding fractions from secretions of patients with allergic rhinitis. RF 001 GREEN GM AM REV RESPIR DIS 102 691 970 002 PROCTOR DF IN: FENN WO 1 309 964 003 REMINGTON JS J CLIN INVEST 43 1613 964 004 ROSEN RD J IMMUNOL 97 369 966 005 ROSEN RD J CLIN INVEST 45 768 966 006 LORIN MI J LAB CLIN MED 80 275 972 007 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 008 BOAT TF PEDIATR RES 7 607 973 009 HUDSON BG J BIOL CHEM 247 4229 972 010 LEV R J HISTOCHEM CYTOCHEM 12 309 964 011 JOFTES DL J NUCL MED 4 143 963 012 LOWRY OH J BIOL CHEM 193 265 951 013 OSSERMAN EF J EXP MED 124 921 966 014 WARREN L J BIOL CHEM 234 1971 959 015 DISCHE Z ANAL BIOCHEM 21 119 967 016 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 017$ COTLOVE E J LAB CLIN MED 50 358 950 018 ANTONOPOULOS CA ACTA CHEM SCAND 16 1521 962 019 CLAMP JR METHODS BIOCHEM ANAL 19 229 971 020 RULON JT ARCH OTOLARYNGOL 78 192 963 021 JONES R HISTOCHEM J 5 19 973 022 ELLIS DB BIOCHEM J 136 837 973 023 HAVEZ R IN: PEETERS H 16 343 968 024 GOTTSCHALK A IN: GOTTSCHALK A 826 972 025 HILDING AC ANN OTOL RHINOL LARYNGOL 82 75 973 026 SPIRO RG N ENGL J MED 281 991 969 027 SLOMIANY BL J BIOL CHEM 247 5062 972 028 DI SANTAGNESE PA N ENGL J MED 277 1287 967 029 REID L IN: PORTER R 32 45 968 030 LEV R AM J PATHOL 46 23 965 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 ANON J PEDIATR 88 711 976 3 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 6 SANTAGNESE PAD N ENGL J MED 295 481 976 7 KHAN MA BIOCHIM BIOPHYS ACTA 444 369 976 8 BOAT TF PEDIATR RES 11 977 977 9 NEUTRA MR LAB INVEST 36 535 977 10 FORSTNER JF ANAL BIOCHEM 83 657 977 11 PROCTOR DF AM REV RESPIR DIS 115 97 977 12 ALHADEFF JA CLIN GENET 14 189 978 13 FORSTNER JF DIGESTION 17 234 978 14 CLAMP JR BR MED BULL 34 25 978 15 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 16 MAWHINNEY TP PEDIATR RES 13 760 979 17 PEARSON RD PEDIATR RES 13 834 979 18 SCANLIN TF CLIN CHEST MED 1 424 980 19 DAVIS PB PEDIATR RES 14 83 980 20 SMALL P ANN ALLERGY 46 317 981 21 ESTEP JA J APPL PHYSIOL 50 383 981 22 KONSTAN MW AM REV RESPIR DIS 123 120 981 23 SHERMAN JM AM REV RESPIR DIS 124 476 981 24 KLAASSEN ABM ANAT ANZ 149 51 981 25 MORIARTY CM J MED 13 257 982 26 MARIN MG J APPL PHYSIOL 52 198 982 27 ETCHISON JR CHEST 81 S 31 982 28 APPLEGARTH DA MED HYPOTHESES 11 277 983 29 FRATES RC PEDIATR RES 17 30 983 30 DAVIS PB J CHRON DIS 36 269 983 31 KUIJPERS W ACTA OTOLARYNGOL STOCKH 95 676 983 32 CHACE KV CLIN CHIM ACTA 132 143 983 33 KATZ S CELL CALC 5 421 984 34 SHELHAMER JH EXP LUNG RES 7 149 984 35 MULLER RM EXP MOL PATH 40 391 984 36 ALBAZZAZ FJ RESPIRATION 46 88 984 37 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 38 BASBAUM CB CIBA FOUND SYMP 109 4 984 39 BOAT TF CIBA FOUND SYMP 109 72 984 40 GUEANT JL CLIN CHIM ACTA 143 217 984 41 PERINI JM BULL EUR PHYSIOPATH RESP 21 569 985 42 VANSCOTT MR EXP LUNG RES 11 75 986 43 DAVID TJ J ROY SOC MED 79 23 986 44 ROOMANS GM SCANN ELECTRON MICROSC 1986 165 986 PN 74024 RN 00024 AN 74091941 AU Mansell-A. Dubrawsky-C. Levison-H. Bryan-A-C. Crozier-D-N. TI Lung elastic recoil in cystic fibrosis. SO Am-Rev-Respir-Dis. 1974 Feb. 109(2). P 190-7. MJ CYSTIC-FIBROSIS: pp. LUNG-COMPLIANCE. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: et, pp. CHILD. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. MALE. NITROGEN. OXYGEN: bl. RADIOISOTOPES. RESPIRATORY-FUNCTION-TESTS. SPIROMETRY. VITAL-CAPACITY. XENON. AB The pressure-volume behavior of the lung was studied in 24 patients with cystic fibrosis. Isovolume flow-pressure relationships were measured in 7 of these patients, and a modification of the flow-volume test was evaluated for detection of flow reduction in a separate group of 63 patients. Relative to results in a control group of 31 normal subjects, 13 of the 24 patients had grossly abnormal pressure-volume curves with excessive stiffness of the lung near maximum volume and loss of recoil at lower volume. Closing volumes in these patients were often high enough to cause significant disturbances in gas exchange and the elevated closing volumes were quantitatively explained by the low values for elastic recoil. The modified flow-volume test detected mild flow reduction in many patients who otherwise had normal pulmonary function tests. Loss of recoil per se did not account for the low maximum expiratory flows, since "upstream" resistances were high. However, pressure-flow curves were effort-dependent at high lung volumes even in patients with severe airway obstruction, showing that time constants of emptying varied widely in these patients. It was concluded that deterioration of lung function in cystic fibrosis involves more than airway obstruction and that the worsening gas trapping is explained by a progressive loss of lung elastic recoil. RF 001 COOK CD PEDIATRICS 24 181 959 002 FEATHERBY EA AM REV RESPIR DIS 102 737 970 003 HOGG JC N ENGL J MED 278 1355 968 004 ESTERLY JR THORAX 23 670 968 005 ZUELZER WW PEDIATRICS 4 53 949 006 TURNER JM J APPL PHYSIOL 25 664 968 007 HOLLAND J J CLIN INVEST 47 81 968 008 MEAD J J APPL PHYSIOL 22 95 967 009 COLEBATCH HJH J APPL PHYSIOL 34 143 973 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 DUBOIS AB J CLIN INVEST 35 322 956 012 MANSELL A J APPL PHYSIOL 33 711 972 013 DOLLFUSS RE RESPIR PHYSIOL 2 234 967 014 MILIC-EMILI J J APPL PHYSIOL 19 207 964 015 LEVISON H ACTA PAEDIATR SCAND 59 648 970 016 HYATT RE J APPL PHYSIOL 13 331 958 017 HYATT RE J APPL PHYSIOL 35 411 973 018 CRAIG DB J APPL PHYSIOL 31 717 971 019 LEBLANC P J APPL PHYSIOL 28 448 970 020 SALAZAR E J APPL PHYSIOL 19 97 964 021 MACKLEM PT AM REV RESPIR DIS 87 47 963 022 REID L IN: HUBBARD D 21 958 023 ZAPLETAL A PEDIATRICS 48 64 971 024 PRIDE NB J APPL PHYSIOL 23 646 967 025 MCFADDEN ER JR J APPL PHYSIOL 27 452 969 026 MELLINS RB PEDIATRICS 41 560 968 027 TAKISHIMA TG SCAND J RESPIR DIS 48 384 967 028 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 CT 1 LANDAU LI AM REV RESPIR DIS 111 725 975 2 ZAPLETAL A J APPL PHYSIOL 40 953 976 3 LAPP NL AM REV RESPIR DIS 113 155 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 ZAPLETAL A BULL EUR PHYSIOPATH RESP 14 265 978 6 WONNE R MONATSSCHR KINDERHEILKD 126 165 978 7 ZAPLETAL A BULL EUR PHYSIOPATH RESP 15 575 979 8 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 9 KRAEMER R SCHWEIZ MED WOCHENSCHR 109 39 979 10 LARSEN GL AM REV RESPIR DIS 119 399 979 11 COOPER DM AM REV RESPIR DIS 121 639 980 12 REDDING GJ AM REV RESPIR DIS 126 31 982 13 COTTON DJ CHEST 87 217 985 14 STOKES DC CHEST 87 785 985 15 DESMOND KJ PEDIATR PULMONOL 2 128 986 16 KRUHLAK RT WEST J MED 145 196 986 17 TEPPER RS AM REV RESPIR DIS 135 1075 987 PN 74025 RN 00025 AN 75071389 AU Gibson-L-E. TI Use of water vapor in the treatment of lower respiratory disease. SO Am-Rev-Respir-Dis. 1974 Dec. 110(6 Pt 2). P 100-3. MJ RESPIRATORY-THERAPY. RESPIRATORY-TRACT-DISEASES: th. WATER. MN AEROSOLS. AIR-MICROBIOLOGY. ANTISEPSIS. ASTHMA: th. BRONCHIAL-SPASM: et. BRONCHIOLITIS-VIRAL: th. CHILD. CYSTIC-FIBROSIS: th. HELIUM. HUMAN. RESPIRATORY-THERAPY: ae, is. PSEUDOMONAS-INFECTIONS: et. RESPIRATORY-FUNCTION-TESTS. SPIROMETRY. AB Use of bland aerosols, particularly mist tents, in the treatment of lower respiratory disease was reviewed and no convincing evidence of efficacy was found. This is not surprising because there is evidence that mist tents deposit little water in the lower respiratory tract. Mist tents may be detrimental, in some cases, because of bacterial contamination and because they can cause bronchospasm. RF 001 COLE P J LARYNGOL OTOL 67 449 953 002 SILVERMAN WZ PEDIATRICS 17 1 956 003 KELSCH RC AM J DIS CHILD 109 495 965 004 CHENEY FW JR ANESTHESIOLOGY 29 1099 968 005 CHENEY FW JR ANESTHESIOLOGY 32 456 970 006 ABERNATHY JD THORAX 23 421 968 007 PFLUG AE AM REV RESPIR DIS 101 710 970 008 WARWICK WJ MINN MED 50 1049 967 009 MATTHEWS LW PEDIATRICS 39 176 967 010 DOERSHUK CF PEDIATRICS 41 723 968 011 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 012 WOLFSDORF J PEDIATRICS 43 799 969 013 BAU SK PEDIATRICS 48 605 971 014 BEIER FR AM REV RESPIR DIS 94 430 966 015 FEATHERBY EA AM REV RESPIR DIS 102 737 970 016 ZAPLETAL A PEDIATRICS 48 64 971 017 MOTOYAMA EK PEDIATRICS 50 299 972 018 CHANG N AM REV RESPIR DIS 107 672 973 019 ANDERSEN AA J BACTERIOL 76 471 958 020 MOFFET HL AM J DIS CHILD 114 21 967 021 GRIEBLE HG N ENGL J MED 282 531 970 022 PIERCE AK N ENGL J MED 282 528 970 023 BOXERBAUM B J INFECT DIS SUPPL 122 59 970 024 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 CT 1 FEVRIER D SCHWEIZ MED WOCHENSCHR 105 903 975 2 HUGHES RL CHEST 69 500 976 3 SHIH CK AM REV RESPIR DIS 115 989 977 PN 74026 RN 00026 AN 74109135 AU Lieberman-J. TI Plasma arginine esterase activity in cystic fibrosis. SO Am-Rev-Respir-Dis. 1974 Mar. 109(3). P 399-401. MJ CYSTIC-FIBROSIS: en. ESTERASES: bl. MN ACIDS. ADOLESCENCE. ADULT. ARGININE. CHLOROFORM. ENZYME-ACTIVATION. HUMAN. MIDDLE-AGE. TRYPSIN-INHIBITORS. AB Plasma arginine esterase activity was measured in 13 young adult patients with cystic fibrosis and in 36 control subjects. The enzyme was activated by treating the plasma with chloroform and ellagic acid in the manner described by Rao and associates (1). No difference was found either between the arginine esterase activities of the patients with cystic fibrosis and the control subjects, or between the degrees of activity inhibited by soybean trypsin inhibitor. Thus, the findings of Rao and associates of a deficiency of arginine esterase activity in the plasma of patients with cystic fibrosis were not confirmed. RF 001 RAO GJS SCIENCE 177 610 972 002 CAUDILL M LANCET 2 307 973 003 RAO GJS J PEDIATR 80 573 972 004 LIEBERMAN J PEDIATR RES 3 571 969 005 CHERNICK WS J PEDIATR 65 694 964 CT 1 COBURN MD AM REV RESPIR DIS 110 368 974 2 ALTLAND K HUM GENET 28 207 975 3 ALHADEFF JA CLIN GENET 10 63 976 4 BOWMAN BH LIFE SCI 19 1289 976 5 BOWMAN BH TEX REP BIOL MED 34 1 976 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 295 597 976 9 CALLAHAN JW PEDIATR RES 11 1166 977 10 COLMAN RW THROMB HAEMOST 38 751 977 11 CHAN KYH CLIN CHIM ACTA 74 71 977 12 ROMEO G NATURE 274 909 978 13 ROMEO G PEDIATR RES 13 1030 979 14 JAKEL HP BIOL ZENTRALBL 98 55 979 15 PARSONS M CLIN CHIM ACTA 100 215 980 16 BURY AF PEDIATR RES 16 613 982 17 BRIDGES MA ANN NY ACAD SCI 421 360 983 PN 74027 RN 00027 AN 75071396 AU Mellins-R-B. TI Pulmonary physiotherapy in the pediatric age group. SO Am-Rev-Respir-Dis. 1974 Dec. 110(6 Pt 2). P 137-42. (REVIEW). MJ PHYSICAL-THERAPY. MN ACUTE-DISEASE. ASTHMA: th. BREATHING-EXERCISES. BRONCHITIS: th, pp. CHILD. CHRONIC-DISEASE. COUGH. CYSTIC-FIBROSIS: th. DRAINAGE. HUMAN. INFANT. LUNG-DISEASES-OBSTRUCTIVE: th. LUNG: pp. PERCUSSION. POSTURE. RADIONUCLIDE-IMAGING. RESPIRATION. RESPIRATORY-TRACT-DISEASES: th. REVIEW. SPIROMETRY. SPUTUM. THORAX. VIBRATION. AB In spite of the widespread clinical impression that pulmonary physical therapy, including breathing exercises, is helpful in the pediatric age group, objective demonstration of the value of the various maneuvers is for the most part lacking. There is evidence that the volume of sputum produced over short periods of time in patients with cystic fibrosis is increased when cough is accompanied by postural drainage and pulmonary physical therapy, but the long-term effect of the various maneuvers on the course of the chronic pulmonary disease remains unknown. RF 001 DOYLE B PHYS THER REV 39 24 959 002 ZAUSMER E PHYS THER 48 586 968 003 ANON AM REV RESPIR DIS 97 730 968 004 THACKER EW POSTURAL DRAINAGE AND RESPIRA 971 005 DUNN D PEDIATR CLIN NORTH AM 20 481 973 006 LOUGH MD PEDIATRIC RESPIRATORY THERAPY 974 007 TING P PEDIATR RES 7 398 973 008 MELLINS RB AM REV RESPIR DIS 105 461 972 009 MELLINS RB PEDIATRICS 41 560 968 010 FRASER RB AM J ROENTG RAD THER NUCL MED 93 821 965 011 SPOCK A PEDIATR RES 1 173 967 012 SANCHIS J N ENGL J MED 288 651 973 013 NEWHOUSE M IN: MANGOS JA 319 973 014 ROSSMAN CM PEDIATR RES 8 469 974 015 MATTHEWS LW J PEDIATR 65 558 964 016 DOERSHUK CF J PEDIATR 65 677 964 017 DOERSHUK CF PEDIATRICS 36 675 965 018 ANON AM REV RESPIR DIS 97 725 968 019 AVERY ME IN: MANGOS JA 291 973 020 TAUSSIG LM J PEDIATR 84 619 974 021 DENTON R AM REV RESPIR DIS 86 41 962 022 LORIN MI AM J PHYS MED 50 215 971 023 MOTOYAMA EK IN: MANGOS JA 335 973 024 PIEPSZ A J NUCL MED 14 326 973 025 ROBINSON AE RADIOLOGY 93 1123 969 026 WOOD RE PEDIATR RES 8 471 974 027 TREVES S J NUCL MED 15 582 974 028 AGOSTONI E IN: FENN WO 387 964 029 SACKNER MA J APPL PHYSIOL 34 495 973 030 GRASSINO AI FED PROC 33 323 974 CT 1 PANG LM ANESTHESIOLOGY 43 171 975 2 MELLINS RB J PEDIATR 87 1 975 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 NOLTE D PNEUMONOLOGIE 1976 61 976 5 WONG JW PEDIATRICS 60 146 977 6 FINER NN PEDIATRICS 61 282 978 7 ETCHES PC PEDIATRICS 62 713 978 8 NEWTH CJL PEDIATR CLIN NORTH AM 26 617 979 9 WOOD RE SOUTH MED J 72 189 979 10 WAGAMAN MJ J PEDIATR 94 787 979 11 MACKENZIE CF ANESTH ANALG CLEVE 59 207 980 12 ZMORA E AM J DIS CHILD 134 250 980 13 GILLIES J NZ MED J 94 61 981 14 HOLODY B AM REV RESPIR DIS 124 372 981 15 ZACH M MONATSSCHR KINDERHEILKD 129 633 981 16 CORADELLO H KLIN PAEDIATR 194 8 982 17 GROSMAIRE EK HEART LUNG 12 650 983 18 DESMOND KJ J PEDIATR 103 538 983 19 PLESS IB PEDIATR CLIN NORTH AM 31 259 984 20 MISCHLER EH SEM RESPIR MED 6 271 985 21 MACKENZIE CF CRIT CARE MED 13 483 985 22 WEBB MSC ARCH DIS CHILD 60 1078 985 23 DEAN E PHYS THER 65 613 985 PN 74028 RN 00028 AN 75053321 AU Savory-J. Leitner-S-S. TI Studies on neutron activation analysis for the measurement of sodium in nails as a screening test for cystic fibrosis. SO Ann-Clin-Lab-Sci. 1974 Nov-Dec. 4(6). P 413-9. MJ ACTIVATION-ANALYSIS. CYSTIC-FIBROSIS: di. NAILS: an. NEUTRON-ACTIVATION-ANALYSIS. SODIUM: an. MN ADULT. CHILD. HUMAN. NEUTRON-ACTIVATION-ANALYSIS: is, mt. AB The present study points out the simplicity and ease of using fingernails and toenails to measure electrolyte levels as a test for cystic fibrosis. Procedures and various effects on the ranges of values for different categories of healthy and diseased subjects are discussed and the limitations of the method are indicated. RF 001 ANTONELLI M ARCH DIS CHILD 44 218 969 002 BARNETT WB CLIN CHEM 18 923 972 003 BOCK H MOD PROBL PEDIATR 10 279 967 004 FITE LE PROC JAPAN CONF ON RADIOI 9TH 524 969 005 FITE LE PROC INT CONF MOD TRENDS 147 968 006 HARRISON WW CLIN CHIM ACTA 31 63 971 007 JOHNSON GF PEDIATRICS 47 88 971 008 KOPITO L N ENGL J MED 272 504 965 009 LEONARD PJ ARCH DIS CHILD 47 495 972 010 ANON JAMA 212 2039 970 011 WOODRUFF GL PROC INT CONF MOD TRENDS 156 968 PN 74029 RN 00029 AN 74124785 AU Taylor-B. Evans-J-N. Hope-G-A. TI Upper respiratory tract in cystic fibrosis. Ear-nose-throat survey of 50 children. SO Arch-Dis-Child. 1974 Feb. 49(2). P 133-6. MJ CYSTIC-FIBROSIS: co. RESPIRATORY-TRACT-DISEASES: co. MN AGE-FACTORS. AUDIOMETRY. CHILD. CHILD-PRESCHOOL. DEAFNESS: co. EAR-DISEASES: co. EAR-MIDDLE. EUSTACHIAN-TUBE. HUMAN. NASAL-POLYPS: co. OTITIS-MEDIA. AB An ear-nose-throat survey was carried out on 50 children aged 4 to 10 years with cystic fibrosis. 10% were shown to be transiently deaf, associated with eustachian tube dysfunction. There were no confirmed cases of secretory otitis media. 10 children (20%) were found to have nasal polyps or gave a history of polypectomy. Though a high incidence of middle ear problems in children with cystic fibrosis has been reported in the U.S.A., neither deafness nor secretory otitis media was found to be a problem in the group studied. RF 001 BROOKS DN INT AUDIOL 8 563 969 002 FORCUCCI RA ARCH OTOLARYNGOL 96 361 972 003 JERGER J ARCH OTOLARYNGOL 93 111 971 004 KULCZYCKI LL ARCH OTOLARYNGOL 92 54 970 005 KULCZYCKI LL CLIN PEDIATR 9 390 970 006 SHWACHMAN H PEDIATRICS 30 389 962 007 SIEGEL J ARCH OTOLARYNGOL 92 523 970 008 WATSON TJ PROC R SOC MED 62 455 969 CT 1 WARNER JO PEDIATRE 12 243 976 2 TOS M J LARYNGOL OTOL 91 827 977 3 PROCTOR DF AM REV RESPIR DIS 115 97 977 4 SWAN HAP S AFR J SURG 17 51 979 5 OPPENHEIMER EH LAB INVEST 40 445 979 6 FORMANFRANCO B ARCH OTOLARYNGOL 105 338 979 7 BAKPEDERSEN K ACTA OTOLARYNGOL STOCKH SUPPL 360 138 979 8 BATSAKIS JG HEAD NECK SURG 2 410 980 9 PRIMOSCH RE ORAL SURG 50 301 980 10 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 11 PEDERSEN M CLIN OTOLARYNGOL 7 373 982 12 STERN RC AM J DIS CHILD 136 1067 982 13 DAVID TJ J ROY SOC MED 79 23 986 PN 74030 RN 00030 AN 74154485 AU Levin-S. TI Letter: Muscular performance and vitamin E in cystic fibrosis. SO Arch-Dis-Child. 1974 Mar. 49(3). P 247. MJ CYSTIC-FIBROSIS: dt. MUSCLES: pp. MUSCULAR-DISEASES: dt. VITAMIN-E: tu. MN CLINICAL-TRIALS. CYSTIC-FIBROSIS: pp. HUMAN. MUSCULAR-DISEASES: pp. PLACEBOS. VITAMIN-E: bl. EX Patients with cystic fibrosis have tocopherol deficiency presumably as a result of steatorrhoea. Evidence for possible muscular involvement in cystic fibrosis patients is clinical (muscular weakness and wasting in many patients), biochemical (creatinuria on a creatine-poor diet, with reversal of this finding after ingestion of tocopherol esters, accompanied by a decrease in creatine in plasma and an increase in creatine in muscle), and pathological (focal lesions in skeletal muscle resembling nutritional muscular dystrophy, and ceroid pigment in smooth muscle). Muscle strength was measured with a bulb ergograph in 45 patients with cystic fibrosis attending the clinics at the Babies' Hospital, Columbia Presbyterian Medical Center, New York. In a double-blind study, in which half the cases received tocopherol orally, and the remainder placebo, no significant increase in muscle strength was found in those patients receiving tocopherol over a period of 6 months as compared to the placebo group. Blood tocopherol levels in the treated group returned to normal. RF 001 DARBY CW ARCH DIS CHILD 48 72 973 002 LEVIN S PEDIATRICS 27 578 961 PN 74031 RN 00031 AN 75034739 AU Rosenbluth-M. Chernick-V. TI Influence of mist tent therapy on sputum viscosity and water content in cystic fibrosis. SO Arch-Dis-Child. 1974 Aug. 49(8). P 606-10. MJ CYSTIC-FIBROSIS: th. HUMIDITY. SPUTUM: an. MN ADOLESCENCE. AEROSOLS. CHILD. DNA: an. HUMAN. SECRETORY-RATE. SPUTUM: se. ULTRASONICS. VISCOSITY. WATER: an. AB The hypothesis that mist tent therapy decreases the viscosity of sputum by direct liquefaction of the sputum in the lower respiratory tract was tested in 6 patients with cystic fibrosis (CF). The first night all patients slept without the mist tent and the first morning sputum was collected for analysis. The following 2 nights patients were randomly allocated to a tent supplied by either a jet or ultrasonic nebulizer. The early morning sputum was analysed for volume, viscosity, water content, and DNA content, an index of purulence. There was no relation between sputum viscosity and DNA content, water content, or volume. Furthermore, there was no consistent relation between sputum viscosity or volume expectorated and the presence or absence of an 8-hour stay in the tent with either method of water nebulization. These results therefore suggest that mist therapy does not consistently influence sputum viscosity or volume in patients with CF. Above a sputum water content of 90%, further increases in water content do not influence viscosity. RF 001 BARKER R J PEDIATR 80 396 972 002 BAU SK PEDIATRICS 48 605 971 003 CHANG N AM REV RESPIR DIS 107 672 973 004 DOERSHUK CF PEDIATRICS 41 723 968 005 FEATHER EA BR J DIS CHEST 64 192 970 006 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 007 MATTHEWS LW PEDIATRICS 39 176 967 008 MOTOYAMA EK PEDIATRICS 50 299 972 009 PHELAN PD ARCH DIS CHILD 44 393 969 010 SCHNEIDER WC METHODS ENZYMOL 3 680 957 011 STURGESS JM IN: LAWSON D PROC 5TH INT CF 368 969 012 WOLFSDORF J PEDIATRICS 43 799 969 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 PROCTOR DF AM REV RESPIR DIS 115 315 977 3 PICOT R THORAX 33 235 978 4 WANNER A AM REV RESPIR DIS 122 79 980 5 TAKISHIMA T TOHOKU J EXP MED 131 103 980 PN 74032 RN 00032 AN 74299357 AU Cogswell-J-J. Risdon-R-A. Taylor-B. TI Chronic suppurative lung disease in sisters mimicking cystic fibrosis. SO Arch-Dis-Child. 1974 Jul. 49(7). P 520-4. MJ CYSTIC-FIBROSIS: di. LUNG-DISEASES-OBSTRUCTIVE: di. MN CHILD. HUMAN. FEMALE. CYSTIC-FIBROSIS: fg, pp, pa. DIAGNOSIS-DIFFERENTIAL. LUNG-DISEASES-OBSTRUCTIVE: pa, fg. SUPPURATION. LUNG: pa. SUBLINGUAL-GLAND: pa. AUTOPSY. SWEAT: an. CHRONIC-DISEASE. AB Two sisters are described with fatal chronic obstructive lung disease of unknown origin. The lung condition was clinically and histologically indistinguishable from cystic fibrosis and the salivary glands showed pathological changes expected in cystic fibrosis. Neither child had abnormal sweat electrolytes, they had no bowel symptoms, and the pancreas was normal at necropsy. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 ANDERSEN DH ANN NY ACAD SCI 93 500 962 003 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 004 BECROFT DMO J CLIN PATHOL 24 72 971 005 BESLEY GTN J MED GENET 6 278 969 006 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 007 DI SANTAGNESE PA PEDIATRICS 12 549 953 008 DI SANTAGNESE PA JAMA 172 2065 960 009 SHWACHMAN H PEDIATRICS 30 167 962 010 SHWACHMAN H IN: KENDIG EL JR 1 524 972 011 SPOCK A PEDIATR RES 1 173 967 012 WILLIAMS HE ARCH DIS CHILD 47 423 972 CT 1 PERLMAN M ARCH DIS CHILD 50 727 975 2 SARSFIELD JK ARCH DIS CHILD 50 463 975 3 STERN RC JAMA 239 2676 978 4 HUFF DS J PEDIATR 94 237 979 5 DAVIS PB AM J MED 69 643 980 6 STERN RC LANCET 1 1401 982 7 BARBERO GJ J PEDIATR 100 914 982 PN 74033 RN 00033 AN 75071864 AU Ryley-H-C. Neale-L. Brogan-T-D. Bray-P-T. TI Plasma proteins in meconium from normal infants and from babies with cystic fibrosis. SO Arch-Dis-Child. 1974 Nov. 49(11). P 901-4. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: di. MECONIUM: an. MN ANTIGENS. BETA-GLOBULINS: an. CHYMOTRYPSIN: an. CYSTIC-FIBROSIS: me, en. GAMMA-GLOBULINS: an. HUMAN. IGA: an. IGG: an. IMMUNOELECTROPHORESIS. INFANT. SODIUM-CHLORIDE: an. SWEAT: an. TRANSFERRIN: an. TRYPSIN: an. EX Cellulose acetate strip electrophoresis was found to be not sufficiently reliable to differentiate between normal and CF meconium, but marked differences were obtained when quantitative immunoelectrophoretic methods were employed. Normal meconium was shown to contain alpha1-antitrypsin and alpha1-antichymotrypsin and most samples contained low concentrations of albumin, and measurable amounts of a variety of other plasma proteins. It is suggested that quantitative immunoelectrophoresis could be used as a means of confirming the diagnosis of CF if positive results are obtained when meconium specimens are screened with test strips. RF 001 CLARKE HGM CLIN SCI 35 403 968 002 GREEN MN PEDIATRICS 41 989 968 003 LOPEZ M IMMUNOCHEMISTRY 6 513 969 004 PRITCHARD JA OBSTET GYNECOL 25 289 965 005 RYLEY HC BIOCHIM BIOPHYS ACTA 271 300 972 006 RYLEY HC J CLIN PATHOL 26 852 973 CT 1 PECAU Y BIOL GASTROENTEROL 8 193 975 2 RYLEY HC CLIN CHIM ACTA 64 117 975 3 LANZA I MINERVA PEDIATR 28 1510 976 4 ROBINSON PG ARCH DIS CHILD 51 301 976 5 RYLEY HC ARCH DIS CHILD 51 641 976 6 RYLEY HC J CHROMATOGR 143 411 977 7 BORGO G MINERVA PEDIATR 30 1059 978 8 FIFI AR MINERVA PEDIATR 30 597 978 9 ANTONOWICZ I BIOL NEONATE 34 225 978 10 HARRIES JT BR MED BULL 34 75 978 11 PROSSER R MONOGR PAEDIATR 10 43 979 12 THOMAS DW GASTROENTEROLOGY 80 776 981 13 PARK RW GASTROENTEROLOGY 81 1143 981 14 DINARI G AM J DIS CHILD 138 971 984 15 SIVAN Y BIOL NEONATE 47 32 985 16 SHULMAN RJ J PEDIATR 107 287 985 17 BOUE A HUM GENET 74 288 986 PN 74034 RN 00034 AN 74303513 AU Davidson-D-C. Shannon-R-S. TI Letter: Cystic fibrosis and coeliac disease. SO Arch-Dis-Child. 1974 Jun. 49(6). P 501. MJ CYSTIC-FIBROSIS: co. CELIAC-DISEASE: co. MN CHILD-PRESCHOOL. HUMAN. FEMALE. JEJUNUM: pa. BIOPSY. IMMUNOGLOBULINS: an. ANTIBODIES: an. FECES: an. LIPIDS: an. EX The coexistence of cystic fibrosis and coeliac disease has recently been reported, and it has been suggested that cystic fibrosis may predispose to the later development of coeliac disease. The diagnosis of coeliac disease is largely dependent upon the demonstration of histological changes in the small intestine, but jejunal biopsy may be distressing to a small child. It is suggested that food protein antibody studies may be of value in the investigation of children with cystic fibrosis in whom gluten sensitivity is suspected. In the following case the decision to perform a jejunal biopsy was made when these studies were positive. Food protein antibody to cereal antigens, wheat, oatmeal, and gluten, and to ruminant antigens, cow's milk, and calf serum were persistently detected in the patient's blood. Jejunal biopsy showed partial villous atrophy with shortening, broadening, and branching of the villi. There was a satisfactory clinical improvement after gluten restriction. RF 001 GOODCHILD MC ARCH DIS CHILD 48 684 973 002 HIDE DW ARCH DIS CHILD 44 533 969 003 TAYLOR BW ARCH DIS CHILD 48 692 973 PN 74035 RN 00035 AN 75029324 AU Bull-F-E. Gladwin-D-E. Griffiths-A-D. TI Immunochemical method for detection of albumin in human meconium. SO Arch-Dis-Child. 1974 Aug. 49(8). P 602-5. MJ ALBUMINS: an. MECONIUM: an. MN COMPARATIVE-STUDY. CYSTIC-FIBROSIS: di. FEMALE. GEL-DIFFUSION-TESTS: mt. HUMAN. INFANT-NEWBORN. PRECIPITIN-TESTS. SALICYLIC-ACIDS: du. AB A simple immunochemical technique utilizing single radial immunodiffusion for the demonstration of albumin in human meconium is described. A comparison of this technique with the more widely used sulphosalicylic acid precipitation method is reported. We also have attempted to determine the normal levels of albumin in meconium. RF 001 BUCHANAN DJ PEDIATRICS 9 304 952 002 CAIN ARR ARCH DIS CHILD 47 131 972 003 GREEN MN PEDIATRICS 21 635 958 004 GREEN MN PEDIATRICS 41 989 968 005 KOLLBERG H ARCH DIS CHILD 47 836 972 006 SCHUTT WH ARCH DIS CHILD 43 178 968 007 WISER WC PEDIATRICS 33 115 964 CT 1 PROSSER R ARCH DIS CHILD 49 597 974 2 PECAU Y BIOL GASTROENTEROL 8 193 975 3 RYLEY HC CLIN CHIM ACTA 64 117 975 4 GRIFFITHS AD BIORHEOLOGY 13 225 976 5 GRIFFITHS AD ARCH DIS CHILD 51 321 976 6 PAPP Z J PEDIATR 88 151 976 7 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 8 UXA F MINERVA PEDIATR 31 831 979 9 MASTELLA G RIV ITAL PEDIATR 7 581 981 PN 74036 RN 00036 AN 75053488 AU McPherson-A-G. TI Proceedings: Neonatal peritonitis. SO Arch-Dis-Child. 1974 Oct. 49(10). P 825. MJ INFANT-NEWBORN-DISEASES: di. PERITONITIS: di. MN CYSTIC-FIBROSIS: co. HUMAN. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co. PERITONITIS: et, su. EX Neonatal peritonitis remains a dangerous condition. Factors complicating diagnosis and management include its low incidence, the diversity of aetiological factors found, and the severity of underlying or associated disease. 28 cases presenting since 1957 at Southmead General Hospital were reviewed. Thirteen aetiological factors relating to mother or baby were found. 13 infants were premature, 10 had severe respiratory problems at birth. Maternal hydramnios was present in 7. 6 babies had had exchange transfusion. The commonest findings were gangrenous volvulus and cystic fibrosis. Combined factors were often present. The usual presentation is with bilious vomiting, abdominal distension, and failure to pass normal meconium. 4 cases had distended abdomens at birth. Plain abdominal x-rays are of great diagnostic value. Pneumoperitoneum is diagnostic but absent from early films. Treatment is surgical. Close liaison with the paediatrician who sees the baby first is essential. In assessing results it is pointed out that many of these babies are small and/or suffering from serious conditions such as haemolytic disease, cystic fibrosis, or gangrenous volvulus. 8 cases were considered unfit for surgery. Of 20 submitted to surgery, 12 survived, mostly in the latter part of the series. PN 74037 RN 00037 AN 75053494 AU McWhirter-W-R. TI Proceedings: Experience with an ion-specific electrode to measure sweat chloride. SO Arch-Dis-Child. 1974 Oct. 49(10). P 828. MJ CHLORIDES: an. SWEAT: an. MN CHILD. CYSTIC-FIBROSIS: di. ELECTRODES. HUMAN. INFANT-NEWBORN. EX The Orion skin chloride measuring system had been evaluated in children of various ages. The method was found to give reproducible results in nearly all cases. Failure to obtain reproducibility was related to inadequate sweating by the subject and this was most likely to occur in the first week of life. There was generally a clear distinction between healthy children and patients with cystic fibrosis. The method was simple and reliable provided that the operator had had reasonable experience with the instrument. One distinct advantage of the method compared with the use of filter paper was the speed with which results were obtained. The design of the instrument made the method completely safe and only a few patients noticed slight tingling during iontophoresis. The mean sweat chloride in the 'normals' was 26.9 mEq/l., compared with 102.3 mEq/l. in the patients with cystic fibrosis. PN 74038 RN 00038 AN 75023276 AU Prosser-R. Owen-H. Bull-F. Parry-B. Smerkinich-J. Goodwin-H-A. Dathan-J. TI Screening for cystic fibrosis by examination of meconium. SO Arch-Dis-Child. 1974 Aug. 49(8). P 597-601. MJ ALBUMINS: an. CYSTIC-FIBROSIS: di. MECONIUM: an. MN COMPARATIVE-STUDY. COSTS-AND-COST-ANALYSIS. CYSTIC-FIBROSIS: oc. ELECTROPHORESIS. ENGLAND. FALSE-POSITIVE-REACTIONS. GEL-DIFFUSION-TESTS. HUMAN. INFANT-NEWBORN. MASS-SCREENING. METHODS. REAGENT-STRIPS. SALICYLIC-ACIDS: du. WALES. AB The value of detecting albumin in meconium as a screening procedure for cystic fibrosis (CF) has been assessed on 34,228 samples in South Wales and North Staffordshire over a 4-year period; simultaneously, four methods of detecting albumin were evaluated. 12 cases of CF were detected, detection rate being 60%. The incidence of the disease in the population screened was 1 in 1850, confirmed by clinical and other test procedures. Cases of CF without impairment of pancreatic function are likely to be missed by screening methods which depend on the presence of albumin in meconium. RF 001 BULL FE ARCH DIS CHILD 49 602 974 002 CAIN ARR ARCH DIS CHILD 47 131 972 003 GREEN MN PEDIATRICS 21 635 958 004 GREEN MN PEDIATRICS 41 989 968 005 HOBBS JR PROTIDES BIOL FLUIDS 17 517 969 006 KOLLBERG H ARCH DIS CHILD 47 836 972 007 PRITCHARD JA OBSTET GYNECOL 25 289 965 008 PRITCHARD JA OBSTET GYNECOL 28 606 966 009 SCHUTT WH ARCH DIS CHILD 43 178 968 010 WISER WC PEDIATRICS 33 115 964 CT 1 RAINE DN LANCET 2 996 974 2 STEPHAN U LANCET 1 167 975 3 PECAU Y BIOL GASTROENTEROL 8 193 975 4 PECAU Y ARCH FR PEDIATR 32 733 975 5 BERRY HK FED PROC 34 2134 975 6 FEIGELSON J SEM HOP PARIS 51 741 975 7 RYLEY HC CLIN CHIM ACTA 64 117 975 8 ANON LANCET 2 614 976 9 LANZA I MINERVA PEDIATR 28 1510 976 10 SCHAUB J HELV PAEDIATR ACTA 31 67 976 11 GRIFFITHS AD ARCH DIS CHILD 51 321 976 12 ROBINSON PG ARCH DIS CHILD 51 301 976 13 ANON J PEDIATR 88 711 976 14 PAPP Z J PEDIATR 88 151 976 15 ANON BR MED J 1 596 977 16 CROSSLEY JR LANCET 2 1093 977 17 KRAEMER R SCHWEIZ MED WOCHENSCHR 107 1105 977 18 BRUNS WT AM J DIS CHILD 131 71 977 19 ELLIOTT RB MED J AUST 2 95 978 20 FIFI AR MINERVA PEDIATR 30 597 978 21 WARWICK WJ HELV PAEDIATR ACTA 33 117 978 22 HARRIES JT BR MED BULL 34 75 978 23 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 24 PROSSER R MONOGR PAEDIATR 10 43 979 25 STAGG BH ANN CLIN BIOCHEM 16 147 979 26 SWAN HAP S AFR J SURG 17 51 979 27 UXA F MINERVA PEDIATR 31 831 979 28 RYLEY HC ARCH DIS CHILD 54 92 979 29 DOYLE EE IR MED J 72 93 979 30 MASTELLA G RIV ITAL PEDIATR 7 581 981 31 EVANS RT J CLIN PATHOL 34 911 981 32 RYLEY HC J CLIN PATHOL 34 906 981 33 RYLEY HC J CLIN PATHOL 34 179 981 34 CONGDON PJ POSTGRAD MED J 57 453 981 35 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 36 ANON LANCET 1 1000 982 37 SANDER J MONATSSCHR KINDERHEILKD 130 843 982 38 PEDERZINI F RIV ITAL PEDIATR 9 445 983 39 HEELEY AF CLIN CHEM 29 2011 983 40 LYON ICT NZ MED J 96 673 983 41 CASSIO A ACTA PAEDIATR SCAND 73 554 984 42 GRUTTNER R MONATSSCHR KINDERHEILKD 133 54 985 43 KNOPFLE G KLIN PAEDIATR 197 13 985 PN 74039 RN 00039 AN 75026014 AU Goodchild-M-C. Insley-J. Rushton-D-I. Gaze-H. TI Cystic fibrosis in 3 Pakistani children. SO Arch-Dis-Child. 1974 Sep. 49(9). P 739-41. MJ CYSTIC-FIBROSIS: fg. MN AUTOPSY. CYSTIC-FIBROSIS: pa, oc. ENGLAND. FEMALE. GENE-FREQUENCY. HUMAN. INFANT. INFANT-NEWBORN. MALE. PAKISTAN: eh. PANCREAS: pa. RACIAL-STOCKS. EX For Caucasians of European origin, cystic fibrosis (CF) remains the commonest recognized autosomal recessive disorder, but it appears to be a rarity among the coloured races. For a time, records may have suggested an association between CF and whiteness; in recent years, however, instance of CF children have been described whose racial origins are Negroid, Mongoloid, and non-European Caucasoid. The present case reports concern 3 Pakistani children born in Birmingham, in whom Cf has been confirmed. 2 of the 3 (Cases 1 and 2) were brother and sister. The present incidence of this disease among Asian immigrants in the West Midlands is approximately 1:10,000. RF 001 HARRIS RL PEDIATRICS 41 733 968 002 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 003 KULCZYCKI LL CLIN PEDIATR 3 692 964 004 MEHTA S INDIAN PEDIATR 5 185 968 005 REDDY CR J TROP MED HYG 73 59 970 006 WANG CI N ENGL J MED 279 1216 968 007 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 KLINGER KW SEM RESPIR MED 6 243 985 PN 74040 RN 00040 AN 75089862 AU Blomfield-J. Van-Lennep-E-W. Shorey-C-D. Malin-A-S. Dascalu-J. Brown-J-M. TI Ultrastructure of the in vitro formation of hydroxyapatite in submandibular saliva of children with cystic fibrosis. SO Arch-Oral-Biol. 1974 Dec. 19(12). P 1153-60. MJ CYSTIC-FIBROSIS: me. HYDROXYAPATITES: me. SALIVA: me. MN ADULT. CALCIUM-PHOSPHATES: me, an. CALCIUM: an. CHILD. CHILD-PRESCHOOL. CYTOPLASMIC-GRANULES: ul, an. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. ENZYME-PRECURSORS: an. HUMAN. HYDROXYAPATITES: an. MAGNESIUM: an. PHOSPHATES: an. PROTEINS: an. SALIVA: an. SPECTROPHOTOMETRY-ATOMIC-ABSORPTION. SUBMANDIBULAR-GLAND: me, ul. X-RAY-DIFFRACTION. AB Calcium phosphate, which precipitates in submandibular saliva of patients with cystic fibrosis because of elevated calcium concentrations, was shown by electron microscopy to transform, on standing in vitro, from amorphous calcium phosphate globules to stellar clusters of hydroxyapatite crystals. Amorphous globules appeared to form at regular nucleation points on a fine network of unknown nature. Hydroxyapatite crystal formation was confirmed by electron diffraction pattern and chemical solubility. Zymogen-granule spherules (inclusion bodies), which are present in greatly increased numbers in cystic fibrosis submandibular saliva, disintegrated on standing from a homogeneous appearance to beaded, hollow spheres. RF 001 BARKER LM JOHNS HOPKINS MED J 127 10 970 002 BLOMFIELD J GUT 14 558 973 003 BLOMFIELD J CF CLUB ABST 14 2 973 004 BLOMFIELD J ARCH DIS CHILD 48 267 973 005 BOAT TF FED PROC ABST 29 861 970 006 BRECEVIC L CALC TISS RES 10 82 972 007 CHEN PS ANAL CHEM 28 1756 956 008 CHERNICK WS J PEDIATR 59 890 961 009 CHERNICK WS J PEDIATR 65 694 964 010 EANES ED CALC TISS RES 12 143 973 011 EANES ED CLIN ORTHOP 53 223 967 012 EGGSTEIN M KLIN WOCHENSCHR 33 879 955 013 GOW BS J DENT RES 44 885 965 014 GOW BS J DENT RES 44 885 965 015 GRON P ARCH ORAL BIOL 12 829 967 016 GUGLER EC J PEDIATR 71 585 967 017 HAY DI ARCH ORAL BIOL 12 937 967 018 KARNOVSKY MJ J CELL BIOL 27 137A 965 019 LOWRY OH J BIOL CHEM 193 265 951 020 MANDEL ID CLIN PEDIATR 8 161 969 021 PALLAVICINI JC ATTACHMENT FOR THE BUCHLER AP 964 022 SCHROEDER HE HELV ODONT ACTA 9 73 965 023 SCHROEDER HE ARCH ORAL BIOL 11 1 966 024 TANDLER B AM J ANAT 135 419 972 025 WARTON KL BR MED J 3 570 971 026 WEST VC CALC TISS RES 7 212 971 027 WILLIS JB SPECTROCHIM ACTA 16 259 960 028 WILLIS JB SPECTROCHIM ACTA 16 273 960 029 WOTMAN S J PERIODONTOL 44 278 973 CT 1 MCGAUGHEY C J PERIODONTOL 46 681 975 2 ALLARS HM PEDIATR RES 10 584 976 3 ALLARS HM PEDIATR RES 10 578 976 4 GRON P ARCH ORAL BIOL 21 201 976 5 TANNENBAUM PJ J DENT RES 55 997 976 6 SEIFERT G ARCH OTO RHINO LARYNGOL 213 111 976 7 WOOD DL PEDIATR RES 11 827 977 8 MCMANUS WR AUST J AGRIC RES 30 635 979 9 LEGEROS RZ J DENT RES 58 2371 979 10 CUTLER LS VIRCHOWS ARCH PATHOL ANAT HIS 392 185 981 11 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 12 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 13 KATZ S CELL CALC 5 421 984 14 TANDLER B J DENT RES 66 398 987 PN 74041 RN 00041 AN 74152219 AU Symonds-D-A. TI Paneth cell metaplasia in diseases of the colon and rectum. SO Arch-Pathol. 1974 Jun. 97(6). P 343-7. MJ COLONIC-DISEASES: pa. COLONIC-NEOPLASMS: pa. INTESTINAL-MUCOSA: pa. RECTAL-DISEASES: pa. RECTAL-NEOPLASMS: pa. MN ADENOCARCINOMA: pa. ADENOMA: pa. AUTOPSY. CARCINOMA: pa. CARCINOMA-MUCINOUS: pa. CHILD. CHILD-PRESCHOOL. COLITIS-ULCERATIVE: pa. CYSTIC-FIBROSIS: pa. DIVERTICULITIS-COLONIC: pa. DYSENTERY-AMEBIC: pa. HUMAN. HYPERPLASIA. INFANT. INFANT-NEWBORN. POLYPS: pa. AB Paneth cell metaplasia is a variable feature of diverse diseases of the colon and rectum. Paneth cells are numerous in the atrophic mucosa of chronic ulcerative colitis, in some juvenile polyps, and around colloid (mucinous) carcinomas. Moderate numbers of Paneth cells are found in cystic fibrosis of the pancreas, ambeic colitis, and in villous adenomas. Paneth cells are sparse in diverticulitis, in adenomatous polyps, and around nonmucinous carcinomas. In this study, Paneth cell metaplasia is associated with mucus hypersecretion and mucin deposits. It is suggested that one function of the Paneth cell is to control gastrointestinal mucin viscosity. RF 001 RIECKEN EO GUT 7 86 966 002 TAYLOR JJ ARCH PATHOL 77 278 964 003 WATSON AJ J PATHOL BACTERIOL 80 309 960 004 PATERSON JC AM J PATHOL 38 243 961 005 HOLMES EJ CANCER 18 1416 965 006 GIBBS NB J CLIN PATHOL 20 826 967 007 HORRILLENO EG CANCER 10 1210 957 008 MOTTET NK HISTOPATHOLOGIC SPECTRUM OF R 143 971 009 GRECO V GUT 8 491 967 010 CREAMER B LANCET 1 314 967 011 BLACK CE ARCH PATHOL 46 107 948 012 TRIER JS GASTROENTEROLOGY 49 560 966 CT 1 SCHARFENBERG JC AM J CLIN PATHOL 64 204 975 2 RODNING CB LANCET 1 984 976 3 ERLANDSEN SL J HISTOCHEM CYTOCHEM 24 1085 976 4 MATSUBARA F ACTA PATHOL JAP 27 677 977 5 VESTFRID MA ACTA ANAT (BASEL) 97 347 977 6 SHOUSHA S HISTOPATHOLOGY 3 489 979 7 SANDOW MJ GUT 20 420 979 8 GONZALEZ AP MORFOL NORMAL PATOL (B) 4 465 980 9 REITAMO S ACTA PATH MICROBIOL SCAND (A) 89 165 981 10 GOLDMAN H HUM PATHOL 13 981 982 11 RODNING CB ANAT REC 204 33 982 12 HARTMAN PE ENVIRON MUTAGEN 5 139 983 13 GELLER SA ARCH PATHOL LAB MED 107 476 983 14 GOLDMAN H GASTROENTEROLOGY 86 199 984 15 BANSAL M AM J PATHOL 115 253 984 16 ARENDS JW BIOCHIM BIOPHYS ACTA 780 1 984 PN 74042 RN 00042 AN 74284190 AU Lox-C-D. Davis-J-R. Christian-C-D. Heine-M-W. TI Anosmic hypogonadotropic hypogonadism Kallman's syndrome--a case history complicated by cystic fibrosis. SO Ariz-Med. 1974 Jul. 31(7). P 508-9. MJ ANOSMIA: co. CYSTIC-FIBROSIS: co. HYPOGONADISM: co. MN ADULT. HUMAN. FEMALE. SYNDROME. AB The association of anomasia and hypogonadism has been reported with increasing frequency over the past several years. This case report of Kallman's Syndrome emphasizes the importance of testing for anomasia in those patients who manifest abnormalities in their sexual development. RF 001 KALLMAN FH AM J MENT DEFIC 48 203 944 002 DEMORSIER G ARCH SUISSES NERUROL PSYCHIAT 74 309 955 003 BOYAR RM ANN INTERN MED 71 1127 969 004 SOHVAL AR J CLIN ENDOCRINOL METAB 12 1229 952 005 MULLER P GYNAECOLOGIA 158 137 964 006 SCHROFFNER WG J CLIN ENDOCRINOL METAB 31 267 970 007 ODELL WD J LAB CLIN MED 70 973 967 008 COYOTUPA J ANAL LETT 5 329 972 009 ABRAHAM GE J CLIN ENDOCRINOL METAB 32 619 972 010 CHOPRA IJ J CLIN ENDOCRINOL METAB 34 938 972 011 SPARKES RS ARCH INTERN MED 121 534 968 012 WIEGELMANN W ACTA ENDOCRINOL SUPPL 152 63 971 013 TAYMOR ML AM J OBSTET GYNECOL 114 445 971 014 ABRAHAM GE J CLIN ENDOCRINOL METAB 33 42 971 015 GRAND RJ JAMA 195 117 966 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 PN 74043 RN 00043 AN 75028085 AU Allan-J-L. Townley-R-R. Phelan-P-D. TI Family response to cystic fibrosis. SO Aust-Paediatr-J. 1974 Jun. 10(3). P 136-46. MJ ATTITUDE-TO-HEALTH. CYSTIC-FIBROSIS. FAMILY. MN ADOLESCENCE. ADULT. AUSTRALIA. CHILD. CHILD-PRESCHOOL. EMOTIONS. FINANCING-PERSONAL. HUMAN. INFANT. INFANT-NEWBORN. SOCIOECONOMIC-FACTORS. VOLUNTARY-HEALTH-AGENCIES. AB The effects of cystic fibrosis on 50 Victorian families with one or more affected children were examined in a study carried out between August 1972 and February 1973. The social and emotional problems experienced by family members, particularly the patients and mothers, are reported on. At all ages, socially embarrassing symptoms and awareness of being different create major problems for the patient. The uncertainty of prognosis and doubts about the prospects of employment are specially relevant for the teenager. It is suggested that the patient be given ample opportunity, from early adolescence onward, to discuss all aspects of his disease, and that more appropriate provision should be made for the total care of adult patients. Many problems, such as depression, feelings of isolation and the physical demands of constant treatment, which the parents of children with cystic fibrosis experience, are probably similar to those confronting parents of other chronically ill and handicapped children. But special difficulties stem from the genetic basis of cystic fibrosis and from the knowledge of an eventually fatal outcome. The roles of professional members of the medical team are touched on, particularly in relation to genetic counseling and the provision of practical and emotional support to parents. Comments are made on the establishment of lay organisations. The financial strain associated with obtaining regular medical attention was looked at, and suggestions are made for minimizing the economic problems of certain vulnerable families. RF 001 LAWLER RH CAN MED ASSOC J 94 1043 966 002 MCCOLLUM AT AM J PUBLIC HEALTH 61 1335 971 003 MCCOLLUM AT J PEDIATR 77 571 970 004 MCCRAE WM LANCET 2 141 973 005 MEARNS MB ARCH DIS CHILD 45 605 970 006 TAYLOR K AM J DIS CHILD 119 209 970 007 TEICHER JD CALIF MED 110 371 969 008 TROPAUER A AM J DIS CHILD 119 424 970 CT 1 BEGLEITER ML SOC BIOL 23 260 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 FALKMAN C ACTA PAEDIATR SCAND SUPPL 264 1977 7 977 4 PHELAN PD MED J AUST 1 261 979 5 CAIRNS NU J PEDIATR 95 484 979 6 ALLAN JL MED J AUST 1 600 980 7 BYWATER EM ARCH DIS CHILD 56 538 981 8 MCKEEVER P AM J ORTHOPSYCHIATRY 53 209 983 9 ANON PEDIATRICS 72 741 983 10 DEWET B S AFR MED J 65 526 984 11 DEWET B S AFR MED J 67 292 985 12 PUMARIEGA AJ J AM ACAD CHILD PSYCHIAT 25 269 986 13 NOLAN T PEDIATRICS 77 229 986 14 MOLLERING M KLIN PAEDIATR 198 369 986 PN 74044 RN 00044 AN 74258256 AU Schmoyer-I-R. Baglia-F-A. TI Cystic fibrosis: effect of media from cultured cystic fibrosis fibroblasts on ATPase activity. SO Biochem-Biophys-Res-Commun. 1974 Jun 18. 58(4). P 1066-70. MJ CYSTIC-FIBROSIS: me. ADENOSINE-TRIPHOSPHATASE: bl. ERYTHROCYTES: en. MN HUMAN. COMPARATIVE-STUDY. ERYTHROCYTES: cy, de. FIBROBLASTS: me. CELL-MEMBRANE: en, de. CULTURE-MEDIA. CELL-LINE. DIALYSIS. SODIUM: pd. POTASSIUM: pd. CALCIUM: pd. MAGNESIUM: pd. OUABAIN: pd. AB Membrane associated ATPase activities of human red blood cells were examined following the addition of spent fibroblast culture media from normal and cystic fibrosis cell lines. In comparison to normal and unspent media, the cystic fibrosis media consistently depressed the ATPase activities. RF 001 BOWMAN BH SCIENCE 167 871 970 002 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 003 ANON GAP CONF REP CILIARY INHIBITO 973 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 006 BALFE JW SCIENCE 162 689 968 007 COLE CH CF CLUB ABST 12 971 008 POST RL J BIOL CHEM 235 1796 960 009 POST RL METHODS ENZYMOL 10 762 967 010 LOWRY OH J BIOL CHEM 193 265 951 011 BADER H GAP CONF REP CF FACTOR 971 CT 1 BOWMAN BH LIFE SCI 19 1289 976 2 BOWMAN BH TEX REP BIOL MED 34 1 976 3 DANES BS TEX REP BIOL MED 34 135 976 4 WARD JB TEX REP BIOL MED 34 83 976 5 WARD JB TEX REP BIOL MED 34 11 976 6 CAMPBELL IM PEDIATRICS 57 480 976 7 WOOD RE AM REV RESPIR DIS 113 833 976 8 RAO GJS PEDIATR RES 11 981 977 9 YOKOYAMA M PEDIATR RES 11 765 977 10 APPLEGARTH DA CLIN CHIM ACTA 74 183 977 11 JAKEL HP BIOL ZENTRALBL 98 55 979 12 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 PN 74045 RN 00045 AN 75164509 AU Danes-B-S. Backofen-J-E. Rottell-B-K. TI Cystic fibrosis: demonstration of an abnormality in mucopolysaccharides in cultured lymphoid lines. SO Biochem-Genet. 1974 Nov. 12(5). P 359-66. MJ CYSTIC-FIBROSIS: me. LYMPH: me. MN CELLS-CULTURED. HETEROZYGOTE. HOMOZYGOTE. HUMAN. LIPOCHONDRODYSTROPHY: me. MUCOPOLYSACCHARIDES. RETINITIS-PIGMENTOSA: me. SYNDROME. AB Cultured lymphoid cells of both homozygotes and heterozygotes for cystic fibrosis could be distinguished from those of normals by (1) growth pattern, gross clumping, and (2) a relative increase in dermatan sulfate, with a normal total mucopolysaccharide content. Lines derived from the genetic mucopolysaccharidoses also had these characteristics, but their total mucopolysaccharide content was markedly increased. These observations support the hypothesis that the cellular disturbance in cystic fibrosis resides in those cellular regions whose functions would be altered by mucopolysaccharide composition. RF 001 BITTER T ANAL BIOCHEM 4 330 962 002 CONOVER JH PEDIATR RES 7 224 973 003 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 004 DANES BS J EXP MED 129 775 969 005 DANES BS J EXP MED 136 644 972 006 DI FERRANTE N BIOCHEM J 124 549 971 007 DI FERRANTE N CONNECT TISSUE RES 1 93 972 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 009 GIBSON LE LANCET 2 189 970 010 HUTTEROTH TH CELL IMMUNOL 5 446 972 011 JOHANSEN PG LANCET 1 455 968 012 LAGUNOFF D ARCH BIOCHEM BIOPHYS 99 396 962 013 LIE SO PROC NAT ACAD SCI USA 69 2361 972 014 LOWE CU SCIENCE 153 1124 966 015 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 016 MATHEWS MB IN: WAGNER BM 304 967 017 MOORE GE CANCER 19 713 966 018 SCHILLER SS J BIOL CHEM 236 982 961 019 SCOTT JE METHODS BIOCHEM ANAL 8 145 960 020 WESSLER E ANAL BIOCHEM 26 439 968 021 WIESMANN UN J PEDIATR 77 685 970 CT 1 DANES BS J MED GENET 12 405 975 2 DANES BS TEX REP BIOL MED 34 135 976 3 ANDERSON LC CLIN CHIM ACTA 73 63 976 4 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 5 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 6 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 7 JAKEL HP BIOL ZENTRALBL 98 55 979 8 JAKEL HP BIOL ZENTRALBL 100 273 981 PN 74046 RN 00046 AN 75036436 AU Milne-M-D. TI Hereditary disorders of intestinal transport. SO Biomembranes. 1974. 4B(0). P 961-1013. MJ INTESTINAL-ABSORPTION. MALABSORPTION-SYNDROMES: me. MN ABETALIPOPROTEINEMIA: me. ACRODERMATITIS: me. AMINO-ACID-METABOLISM-INBORN-ERRORS: me. AMINO-ACIDS: me. AMYLASES: me. AMYLOIDOSIS: me. CARBOHYDRATE-METABOLISM-INBORN-ERRORS: me. COPPER: me. CYSTIC-FIBROSIS: me. CYSTINURIA: me. DIARRHEA: me. FANCONI-SYNDROME: me. FATS: me. FOLIC-ACID: me. FRUCTOSE-INTOLERANCE: me. GLUCOSIDASES: me. HARTNUP-DISEASE: me. HUMAN. LACTOSE-INTOLERANCE: me. MAGNESIUM: me. MALABSORPTION-SYNDROMES: fg. MONOSACCHARIDES: me. PANCREATITIS: me. PHENYLKETONURIA: me. RICKETS: me. VITAMIN-B-12: me. EX Hereditary abnormalities of intestinal transport are rare conditions but are of disproportionate importance because of the information they give regarding the mechanisms of intestinal absorption in man. The main disability is usually diarrhoea, but there may be abnormalities from lack of absorption of an essential nutrient or occasionally from absorption of compounds in excess due to failure of a normal control mechanism. These diseases are experiments of nature which cannot be exactly simulated in the laboratory animal, and thus may on occasions give information unobtainable from other sources. Most often, however, they confirm that the mechanisms of absorption studied in detail in smaller mammals are probably applicable to man. In some of these diseases there is an associated defect of transport in the proximal renal tubules as well as in the gut. They furnish evidence, therefore, that transport processes are closely related in the gut and in the kidney, or at least that an important step in transport is identical at the two sites. Such disorders include disorders of carbohydrate absorption, hereditary defects of amino acid absorption, hereditary defects in the absorption of fat, hereditary disorders of the intestinal absorption of electrolytes, and hereditary defects of vitamin B12 absorption. RF 001 BEAL VA J NUTR 50 223 953 002 GREAVES JP PROC NUTR SOC 23 136 964 003 LOWE CU AM J DIS CHILD 82 459 951 004 WEIJERS HA LANCET 2 296 960 005 EGGERMONT E PROG MED GENET 6 241 969 006 AURICCHIO S J PEDIATR 66 555 965 007 FREZAL JF IN: SHMERLING DH 113 968 008 SEMENZA S BIOCHIM BIOPHYS ACTA 105 386 965 009 REY J ARCH FR PEDIATR 24 65 967 010 HANSSON O ACTA DERM VENEREOL (STOCKH) 43 465 963 010 EGGERMONT E EUR J BIOCHEM 9 488 969 011 MOYNAHAN EJ PROC R SOC MED 56 300 963 011 REY J ARCH FR PEDIATR 20 381 963 012 PRADER A ANNU REV MED 16 345 965 013 LEVIN B ARCH DIS CHILD 45 173 970 014 HOLZEL A LANCET 1 1126 959 015 LAUNIALA K ACTA PAEDIATR SCAND 55 257 966 016 ZSCHIESCHE OM GASTROENTEROLOGY 54 1286 968 017 DURAND P MINERVA PEDIATR 10 706 958 018 HOLZEL A PROC R SOC MED 61 1095 968 019 HOLZEL A ARCH DIS CHILD 42 341 967 020 BERG NO ACTA PAEDIATR SCAND 58 525 969 021 LAPLANE R ARCH FR PEDIATR 19 895 962 022 LINDQUIST B ACTA PAEDIATR SCAND 51 674 962 023 MEEUWISSE GW ACTA PAEDIATR SCAND SUPPL 188 969 024 CAMPBELL PN BIOCHEM J 43 426 948 025 WILSON TH J BIOL CHEM 216 851 955 026 ANDERSON CM ARCH DIS CHILD 40 1 965 027 ALVARADO F BIOCHIM BIOPHYS ACTA 112 292 966 028 CSAKY TZ BIOCHIM BIOPHYS ACTA 82 215 964 029 LINNEWEH F KLIN WOCHENSCHR 43 405 965 030 ANNEGERS JH AM J PHYSIOL 206 1095 964 030 NEWEY H BR MED BULL 23 236 967 031 HOLDSWORTH CD CLIN SCI 27 371 964 032 MEEUWISSE GW ACTA PAEDIATR SCAND 57 273 968 033 ELSAS LJ J CLIN INVEST 49 576 970 034 GRAY GM GASTROENTEROLOGY 51 489 966 035 MEEUWISSE GW HELV PAEDIATR ACTA 25 13 970 036 ELSAS LJ J CLIN INVEST 48 1845 969 037 MEEUWISSE GW ACTA PAEDIATR SCAND 59 74 970 038 MEEUWISSE GW SCAND J CLIN LAB INVEST 25 145 970 040 BURKE V LANCET 1 1177 966 041 ZETTERSTROM R IN: SHMERLING DH 101 968 042 ROMMEL K KLIN WOCHENSCHR 45 559 967 043 CHAMBERS RA LANCET 2 340 956 044 FROESCH ER SCHWEIZ MED WOCHENSCHR 87 1168 957 045 HERS HG ENZYMOL BIOL CLIN 1 4 961 046 SACREZ R PEDIATRIE 17 875 962 047 CORNBLATH M N ENGL J MED 269 1271 963 048 CORNBLATH M ANNU REV MED 17 161 966 049 FINE RN J PEDIATR 75 632 969 050 FIELD JB J CLIN INVEST 44 1240 965 051 BARON DN LANCET 2 421 956 052 MILNE MD Q J MED 29 407 960 053 CUSWORTH DC BIOCHEM J 74 550 960 054 NAVAB F GUT 11 373 970 055 ASATOOR AM GUT 11 380 970 056 ASATOOR AM LANCET 1 126 963 057 SHAW KNF FED PROC 10 194 960 058 SHIH VE PEDIATR RES 3 487 969 059 TARLOW MJ CLIN SCI 39 18P 970 060 MILNE MD BIOCHEM J 111 3P 969 061 WONG PWK ARCH DIS CHILD 41 383 966 062 SCRIVER CR AM J DIS CHILD 117 4 969 064 NEWEY H J PHYSIOL (LOND) 152 367 960 065 NEWEY H J PHYSIOL (LOND) 164 527 962 066 CRAFT IL GUT 9 425 968 067 PETERS TJ GUT 10 1055 969 068 DENT CE Q J MED 20 205 951 069 MILNE MD GUT 2 323 961 070 ASATOOR AM CLIN SCI 23 285 962 071 BOSTROM H ACTA MED SCAND SUPPL 175 411 7 964 072 ARROW VK J PHYSIOL (LOND) 142 141 958 073 MCCARTHY CF J CLIN INVEST 43 1518 964 074 THIER SO J CLIN INVEST 44 442 965 075 LONDON DR CLIN SCI 29 129 965 076 MAWER GE CLIN SCI 36 463 969 077 ROSENBERG LE J CLIN INVEST 45 365 966 078 HARRIS H ANN EUGEN 18 125 953 079 HARRIS H ANN HUM GENET 19 196 955 080 ASATOOR AM CLIN SCI 41 23 971 081 GROSS JB MINN MED 41 78 958 082 GROSS JB AM J MED 33 358 962 083 GROSS JB J LAB CLIN MED 63 933 964 084 FLEMING WH PEDIATRICS 32 358 963 085 HURWITZ LJ BRAIN 90 799 967 086 HURWITZ LJ J NEUROL NEUROSURG PSYCHIAT 32 495 969 087 PERHEENTUPA J LANCET 2 813 965 088 KEKOMAKI M ACTA PAEDIATR SCAND 56 617 967 089 KEKOMAKI M ANN PEDIATR (PARIS) 14 18 968 090 MALMQUIST J LANCET 2 129 970 091 WHELAN DT PEDIATR RES 2 525 968 092 OYANAGI K J PEDIATR 77 259 970 093 SCRIVER CR J CLIN INVEST 47 823 968 094 MORIKAWA T TOHOKU J EXP MED 90 105 966 095 GOODMAN SI J PEDIATR 71 246 967 096 BARTSOCAS CS AM J DIS CHILD 117 93 969 097 ARMSTRONG MD ARCH BIOCHEM BIOPHYS 52 287 954 098 MACKENSIE DY BR MED J 1 90 959 099 BESSMAN SP METABOLISM 9 377 960 100 YARBRO MT J PEDIATR 68 895 966 101 LINNEWEH F KLIN WOCHENSCHR 41 253 963 102 HOOFT C LANCET 2 20 964 103 HOOFT C ANN PAEDIATR 205 73 965 104 SMITH AJ ARCH DIS CHILD 33 109 958 105 HOOFT C HELV PAEDIATR ACTA 23 334 968 106 DRUMMOND KN AM J MED 37 928 964 107 DANBOLT N ACTA DERM VENEREOL (STOCKH) 23 127 942 108 MARGILETH AM AM J DIS CHILD 105 285 963 109 BLOOM D NY STATE J MED 60 3609 960 112 THOMPSON MW AM J HUM GENET 3 159 951 113 MORRIS TSM PROC R SOC MED 59 1005 966 114 COOKE WT Q J MED 22 59 953 115 EBBS JH CAN MED ASSOC J 75 885 956 116 MACDONALD WC N ENGL J MED 272 448 965 117 CARTER C ANN HUM GENET 23 266 959 118 HOFFMAN HN GASTROENTEROLOGY 51 36 966 119 BASSEN FA BLOOD 5 381 950 120 WAYS PO J CLIN INVEST 46 35 967 121 VAN BUCHEM FSP AM J MED 40 794 966 122 ESTES JW AM J MED 42 868 967 123 LEVY RI J CLIN INVEST 45 532 966 124 DOBBINS WO GASTROENTEROLOGY 50 195 966 125 PARTIN JC J CLIN INVEST 49 72A 970 126 WAYS PO J CLIN INVEST 42 1248 963 127 MCBRIDE JA BR J HAEMATOL 18 383 970 128 SIMON ER J CLIN INVEST 43 1311 964 129 ANDERSON CM MED J AUST 2 617 961 130 LAMY M ARCH FR PEDIATR 24 1079 967 131 SILVERBERG M GASTROENTEROLOGY 54 1271 968 132 ANDRADE C BRAIN 75 408 952 133 HORTA JDS GAZETA MED PORT 9 678 956 134 DO ROSARIO R ACTA GASTROENTEROL BELG 24 391 961 135 ANDERSSON R ACTA MED SCAND 188 85 970 136 HOFMANN AF J CLIN INVEST 43 247 964 137 DI SANTAGNESE PA N ENGL J MED 277 1287 967 138 SEAKINS JWT GUT 11 600 970 139 THOMAIDIS TS J PEDIATR 63 444 963 140 MULLINS F JAMA 192 741 965 141 GRYBOSKI JD GASTROENTEROLOGY 45 633 963 142 COZZETTO FJ PEDIATRICS 32 228 963 143 SHMERLING DH HELV PAEDIATR ACTA 24 547 969 144 BURKE V ARCH DIS CHILD 42 147 967 145 SALEN S MT SINAI J MED 37 103 970 146 FREDRICKSON DS N ENGL J MED 276 34 967 147 ROBERTS WC ARCH PATHOL 90 46 970 148 SHELDON W ARCH DIS CHILD 39 268 964 149 REY J ARCH FR PEDIATR 23 5 966 150 TOWNES PL J PEDIATR 66 275 965 151 TOWNES PL J PEDIATR 71 220 967 152 MORRIS MD AM J DIS CHILD 114 203 967 153 HADORN B LANCET 1 812 969 154 TARLOW MJ ARCH DIS CHILD 45 651 970 155 POLONOVSKI C ACTA PAEDIATR SCAND 45 651 970 156 ROSS CAC LANCET 1 1087 955 157 CRABBE PA GASTROENTEROLOGY 51 305 966 158 DUBOIS RS J PEDIATR 76 377 970 159 TWOMEY JJ ANN INTERN MED 72 499 970 160 BRUTON OC AM J DIS CHILD 84 632 952 161 HITZIG WH SCHWEIZ MED WOCHENSCHR 91 1625 961 162 BUCKLEY RH PEDIATRICS 39 506 967 163 FULGINITI VA LANCET 2 5 966 164 CRABBE PA AM J MED 42 319 967 165 GUTMANN L ARCH NEUROL 8 318 963 166 SOUTH MA AM J MED 44 168 968 167 EIDELMAN S LANCET 1 884 968 168 STOBO JD J CLIN INVEST 46 1329 967 169 GAMBLE JL J PEDIATR 26 509 945 170 DARROW DC J PEDIATR 26 519 945 171 LAUNIALA K IN: SHMERLING DH 137 968 172 LAUNIALA K ACTA PAEDIATR SCAND 56 320 967 173 PASTERNACK A LANCET 2 1047 966 174 PASTERNACK A ACTA ENDOCRINOL 55 1 967 175 PEARSON AJG GUT 11 370 970 176 EVANSON JM GUT 6 29 965 177 WINTERS RW MEDICINE (BALTIMORE) 37 97 958 178 PRADER A HELV PAEDIATR ACTA 16 452 961 179 CONDON JR BR MED J 3 138 970 180 DENT CE J BONE JOINT SURG 50B 708 968 181 HAMILTON R PEDIATRICS 45 364 970 182 PAUNIER L PEDIATRICS 41 385 968 183 SALET J ARCH FR PEDIATR 23 749 966 184 FRIEDMAN M LANCET 1 703 967 185 SKYBERG D ACTA PAEDIATR SCAND SUPPL 177 26 967 186 VAINSEL M ARCH DIS CHILD 45 254 970 187 STROMME JH ACTA PAEDIATR SCAND 58 433 969 188 HEGGTVEIT HA AM J PATHOL 45 757 964 189 NAKAMURA M PROC SOC EXP BIOL MED 108 315 961 190 ROSS DB J PHYSIOL (LOND) 160 417 962 191 ALDOR TAM GASTROENTEROLOGY 59 745 970 192 BALCERZAK SP AM J MED 40 857 966 193 WILLIAMS R LANCET 1 1243 965 194 WILLIAMS R Q J MED 38 1 969 195 POWELL LW AUSTRALAS ANN MED 3 226 970 196 POWELL LW GUT 11 727 970 197 OLATUNBOSUN D J LAB CLIN MED 75 754 970 198 THOMSON ABR GASTROENTEROLOGY 58 1001 970 199 SCHADE SG PROC SOC EXP BIOL MED 134 741 970 200 ALTSTATT LB PROC SOC EXP BIOL MED 124 353 967 201 WILLIAMS R Q J MED 31 249 962 202 CUMINGS JN HEAVY METALS AND THE BRAIN 959 203 SCHEINBERG IH GASTROENTEROLOGY 37 550 959 204 BEARN AG J LAB CLIN MED 45 623 955 205 BUSH JA J CLIN INVEST 34 1766 955 206 MCINTYRE PA BULL JOHNS HOPKINS HOSP 204 309 959 207 ARBO JC ACTA GENET STATIST MED 8 105 958 208 BALCERZAK SP BLOOD 32 701 968 209 EDWARDS FC BR MED J 2 1409 966 210 CHANARIN I MEGALOBLASTIC ANAEMIAS 969 211 DONIACH D SEMIN HEMATOL 1 313 964 212 KUNIN AS AM J MED 34 856 963 213 WUEPPER KD CLIN EXP IMMUNOL 2 71 967 214 MCINTYRE OR N ENGL J MED 272 981 965 215 MILLER DR N ENGL J MED 275 978 966 216 IMERSLUND O ACTA PAEDIATR SCAND SUPPL 49 119 960 217 GRASBECK R ACTA MED SCAND 167 289 960 218 BEN-BASSAT I ISR J MED SCI 5 62 969 219 GRASBECK R SCAND J CLIN LAB INVEST SUPPL 95 38 967 220 VISAKORPI JK IN: SHMERLING DH 150 968 221 CHAVELET F NOUV REV FR HEMATOL 4 311 964 222 MOHAMED SD Q J MED 35 433 966 223 FOROOZAN P N ENGL J MED 277 553 967 224 COOPER BA J CLIN INVEST 39 199 960 225 HERBERT V FED PROC 19 884 960 226 HERBERT V GASTROENTEROLOGY 54 110 968 227 COLLE E BLOOD 18 48 961 228 HIPPE E ACTA PAEDIATR SCAND 55 510 966 229 BJORNSTAD P ACTA HAEMATOL (BASEL) 30 340 963 230 LUHBY AL AM J DIS CHILD 102 482 961 231 LUHBY AL PROC AM PEDIATR SOC ANNU MTG 967 232 LANZKOWSKY P BLOOD 34 452 969 233 LANZKOWSKY P AM J MED 48 580 970 CT 1 HEIZER WD ENVIRON HEALTH PERSPECT 33 101 979 PN 74047 RN 00047 AN 75165159 AU Mitchell-Heggs-P. Palfrey-A-J. Reid-L. TI The elasticity of sputum at low shear rates. SO Biorheology. 1974 Nov. 11(6). P 417-26. MJ RHEOLOGY. SPUTUM: ph. MN ASTHMA: pp. BRONCHIECTASIS: pp. BRONCHITIS: pp. CYSTIC-FIBROSIS: pp. ELASTICITY. HUMAN. VISCOSITY. AB Elasticity of sputum. Elasticity of sputum has been determined using the Weissenberg rheogoniometer, by oscillatory testing, over frequencies from 0.01 to 0.8 c/s. Sputum was from patients with either asthma, chronic bronchitis, cystic fibrosis, or bronchiectasis and, macroscopically, mucoid, mucopurulent or purulent. As in the viscosity plot over this shear rate range two Zones with a junctional region can be distinguished. In Zone 1, over the lowest shear rates, elasticity increases slowly, changes little over a "plateau" region and then, in Zone 2, increases sharply. In contrast to the viscosity plot, the plateau does not show nothing. By 0.8 c/s some samples show decreasing elasticity. Although variance between samples is wide, there is no level of elasticity characteristic of each disease or of one macroscopic appearance. Elasticity and viscosity are correlated, most significantly at the lowest shear rates. Asthmatic and bronchiectatic sputa resemble each other in that this correlation is still significant at higher shear rates, cystic fibrosis and chronic bronchitic sputum in that it is not. Since in these last two mucous gland hypertrophy is present, it may be that the sputum has a higher mucus component. RF 001 STURGESS J THESIS 970 002 DAVIS SS BIORHEOLOGY 6 11 969 003 VAN WAZER JR VISCOSITY AND FLOW MEASUREMEN 963 004 REID L BULL PHYSIOPATH RESPIR NANCY 9 15 973 005 STURGESS JM CLIN SCI 38 145 970 006 STURGESS J RHEOL ACTA 10 36 971 007 DAVIS SS BULL PHYSIOPATH RESPIR NANCY 9 47 973 008 MITCHELL-HEGGS PF THESIS 974 009 ANON LANCET 1 775 965 010 KESSLER WR PEDIATRICS 8 648 951 011 WEISSENBERG K TESTING OF MATERIALS BY MEANS 964 012$ HELDERS FE J AM CHEM SOC 60 1575 956 013 REID L LANCET 1 275 954 014 JACKSON ADM PROGNOSE CHRONISCHER 960 015 GODFREY S ARCH DIS CHILD 46 144 971 017 REID L THORAX 15 132 960 018 REID L IN: PORTER R 38 43 971 019 BLAKE J RESPIR PHYSIOL 17 394 973 020 PALMER KNV BR J DIS CHEST 64 185 970 021 DULFANO MJ AM REV RESPIR DIS 104 88 971 CT 1 MEYER FA BIORHEOLOGY 13 49 976 2 REID L POSTGRAD MED J 52 183 976 3 WANNER A AM REV RESPIR DIS 116 73 977 4 ANON LANCET 1 1189 978 5 RICHARDSON PS PHARMACOL THER (B) 3 441 978 6 GELMAN RA AM REV RESPIR DIS 120 553 979 7 MCINTIRE LV ANNU REV FLUID MECH 12 159 980 8 SHIMURA S BIORHEOLOGY 17 363 980 9 DAVIS SS EUR J RESPIR DIS 61 141 980 10 TAKISHIMA T TOHOKU J EXP MED 131 103 980 11 KIM CS BULL EUR PHYSIOPATH RESP 18 915 982 12 WANNER A SEM RESPIR MED 5 329 984 13 RONCORONI AJ MED (BUENOS AIRES) 45 183 985 PN 74048 RN 00048 AN 74302585 AU Bozkowa-K. Golebiowska-H. Lambert-I. Kunicka-A. Duros-H. Nowakowska-A. TI Immunological studies in children with cystic fibrosis (C.F.). SO Boll-Ist-Sieroter-Milan. 1974. 53(1). P suppl:266. MJ CYSTIC-FIBROSIS: im. MN INFANT. CHILD-PRESCHOOL. CHILD. ADOLESCENCE. HUMAN. IMMUNOGLOBULINS: an. IGM: an. IGG: an. AGE-FACTORS. ISOANTIBODIES: an. DIPHTHERIA-TOXOID. ANTIBODIES-BACTERIAL: an. STAPHYLOCOCCUS: im. LYMPHOCYTE-TRANSFORMATION: de. ANTIGENS-BACTERIAL: pd. LECTINS: pd. AB Concentrations of immunoglobulins were determined (using Mancini method and WHO standard) in 108 sera taken during long term treatment of 45 children with C.F. from 2 months to 16 years old. They were shown to depend markedly on the clinical state of the children particularly on the presence of pulmonary tract infectious processes. These infections caused a rise in immunoglobulin concentration with predominance of IgM in C.F. children aged up to 3 yrs, and with predominance of IgG in older children. Mean titer of isohemagglutinins in children with C.F. was higher than in healthy children. Low titers of diphtherial and stapholococcal antibodies were found. In the case of anti diphtheria antibodies, this can be attributed to the fact that these children are not regularly vaccinated owing to their poor clinical condition. As to the low titer of antistaphylococcal antibodies this is a well known finding in the course of chronic staphylococcal infections of appropriate bacteriological tests in our C.F. children. Furthermore their lymphocytes showed an increased blastic transformation when stimulated with staphylococcal filtrate. Transformation of PHA-stimulated lymphocytes was normal. CT 1 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 PN 74049 RN 00049 AN 75008207 AU Goodchild-M-C. Sagaro-E. Brown-G-A. Cruchley-P-M. Jukes-H-R. Anderson-C-M. TI Comparative trial of pancrex V forte and nutrizym in treatment of malabsorption in cystic fibrosis. SO Br-Med-J. 1974 Sep 21. 3(5933). P 712-4. MJ AMYLASES: tu. BROMELAINS: tu. CYSTIC-FIBROSIS: co. LIPASE: tu. MALABSORPTION-SYNDROMES: dt. PANCREATIC-EXTRACTS: tu. PEPTIDE-HYDROLASES: tu. TRYPSIN: tu. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. COMPARATIVE-STUDY. DIETARY-FATS: me. DIETARY-PROTEINS: me. DRUG-COMBINATIONS. FECES: an. FEMALE. HUMAN. INTESTINAL-ABSORPTION. LIPIDS: an. MALE. UREA: ur. AB A cross-over trial of pancreatic enzyme replacement therapy has been conducted on 12 children with cystic fibrosis using Pancrex V forte and Nutrizym tablets in equivalent dosage. No differences were found in the effectiveness of these products as measured by stool volume, number of bowel actions, faecal fat excretion, and urine urea excretion. Neither product eliminated the steatorrhoea. Though there was no laboratory evidence to support their choice nine patients expressed a preference for Nutrizym at the conclusion of the trial. This preference was based partly on the smaller number of tablets which are required. RF 001 ANDERSON CM LANCET 1 836 952 002 BONSNES RW J BIOL CHEM 158 581 945 003$ COLTINI EP J NUTR 103 581 945 004$ FAWCETT JK J CLIN PATHOL 13 156 960 005 HARRIS R ARCH DIS CHILD 30 424 955 006 KNILL-JONES RP BR MED J 4 21 970 007 LAPEY A J PEDIATR 84 328 974 008 MATTHEWS LW PEDIATRICS 27 351 961 009 ROSS CAC ARCH DIS CHILD 30 316 955 010 VAN DE KAMER JH J BIOL CHEM 177 347 949 CT 1 ALLEN WM J BIOL STANDARD 3 267 975 2 WIDHALM K WIEN KLIN WOCHENSCHR 88 557 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 WEISE J EUR J PEDIATR 122 107 976 5 ANON LANCET 2 73 977 6 REGAN PT LEBER MAGEN DARM 7 201 977 7 REGAN PT N ENGL J MED 297 854 977 8 REGAN PT MAYO CLIN PROC 53 79 978 9 REGAN PT MAYO CLIN PROC 54 267 979 10 DURIE PR GUT 21 778 980 11 GOW R LANCET 2 1071 981 12 CHO YW J CLIN PHARMACOL 21 224 981 13 CONGDEN PJ ARCH DIS CHILD 56 708 981 14 TOLCKMITT W KLIN PAEDIATR 193 365 981 15 MITCHELL EA AUST PAEDIATR J 18 114 982 PN 74050 RN 00050 AN 74278397 AU Braunstein-M-S. Fleegler-B. TI Failure of bronchopulmonary lavage in cystic fibrosis. SO Chest. 1974 Jul. 66(1). P 96-9. MJ CYSTIC-FIBROSIS: th. IRRIGATION: ae. SODIUM-CHLORIDE: ae. MN ADULT. HUMAN. MALE. CASE-REPORT. GENTAMICINS: tu. PSEUDOMONAS-INFECTIONS: et, dt. AIRWAY-OBSTRUCTION: et. AB In an attempt to retard deterioration of pulmonary function, a young man with cystic fibrosis underwent broncho-pulmonary lavage of an entire lung. In spite of theoretical advantages, the lavage produced an acute deterioration in the patient's clinical and physiologic status, from which he had not recovered one month later. We conclude that bronchopulmonary lavage in cystic fibrosis may be hazardous and should be reserved for those cases in which conventional methods of improving tracheobronchial clearance have failed. RF 001 HACKETT RR ANESTHESIOLOGY 26 248 965 002 CEZEAUX G JR JAMA 199 15 967 003 KYLSTRA JA AM REV RESPIR DIS 103 651 971 004 ROGERS RM CHEST SUPPL 62 95 972 005 WARING WW PEDIATRICS 39 166 967 006 DOGGETT RG J PEDIATR 68 215 966 007 DI SANTAGNESE PA N ENGL J MED 277 1399 967 008 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 009 RAMIREZ RJ ANN INTERN MED 63 819 965 010 ROGERS RM N ENGL J MED 286 1230 972 012 WINTERNITZ MC IN: CONTRIB MED BIOL RES 1255 919 CT 1 LOBER CW CHEST 68 382 975 2 MILLIS RM CHEST 71 508 977 3 WEINSTEIN HJ CHEST 72 583 977 4 EWING CW CHEST 73 750 978 5 HARNIK E ANESTH ANALG CLEVE 62 357 983 6 SHERMAN JM PEDIATR PULMONOL 2 244 986 PN 74051 RN 00051 AN 74277076 AU Singer-L. Crozier-D. Moscarello-M-A. TI The levels of galactosyltransferase activity in sera from normal children and patients with cystic fibrosis. SO Clin-Biochem. 1974 Jun. 7(2). P 146-9. MJ CYSTIC-FIBROSIS: en. HEXOSYLTRANSFERASES: bl. MN CHILD-PRESCHOOL. CHILD. ADOLESCENCE. ADULT. HUMAN. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: bl. GALACTOSE. GALACTOSAMINE. DRUG-STABILITY. EVALUATION-STUDIES. CARBON-RADIOISOTOPES. SPECTROPHOTOMETRY. GLUCOSAMINE. METHODS. TIME-FACTORS. AB The levels of galactosyltransferase have been studied in the sera obtained from 59 normal individuals varying in age from 5 - 19 years, and from 29 cystic fibrosis patients varying in age from 5 - 33 years. The enzyme level found in children was lower than that in adults. The levels in the sera of cystic fibrosis patients was found to be about half of that in the normal sera for the corresponding age group. In those cystic fibrosis patients over 20 years of age, normal levels were found. RF 001 HUDGIN RL CAN J BIOCHEM 49 838 971 002 MOOKERJEA S CAN J BIOCHEM 49 297 971 003 KIM YS J CLIN INVEST 51 2033 972 004 LOWRY OH J BIOL CHEM 193 265 951 005 MOOKERJEA S FEBS LETTERS 23 257 972 006 MOOKERJEA S CAN J BIOCHEM 50 738 972 007 WINZLER RJ IN: BALAZS EA 2A 399 955 008 MAUNIG M CAN J BIOCHEM 46 477 968 009 ARIAS IM J CLIN INVEST 37 875 958 CT 1 ALHADEFF JA CLIN GENET 10 63 976 2 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 3 SANTAGNESE PAD N ENGL J MED 295 481 976 4 HUNT LA J SUPRAMOL STRUCT 7 213 977 5 FRASER IH BIOCHEM J 164 541 977 6 ALHADEFF JA CLIN GENET 14 189 978 7 KESSEL D EUR J BIOCHEM 82 535 978 8 AGGETT PJ MONOGR PAEDIATR 10 8 979 9 NAGY EC PEDIATR RES 13 729 979 10 DELVES HT CLIN ENDOCRINOL METAB 14 725 985 PN 74052 RN 00052 AN 74301412 AU McEvoy-F-A. Davies-R-J. Goodchild-M-C. Anderson-C-M. TI Erythrocyte membrane properties in cystic fibrosis. SO Clin-Chim-Acta. 1974 Jul 31. 54(2). P 195-204. MJ ERYTHROCYTES: en. CELL-MEMBRANE: en. CYSTIC-FIBROSIS: en. MN CHILD. ADULT. HUMAN. MALE. FEMALE. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. CHROMATOGRAPHY-THIN-LAYER. ADENOSINE-TRIPHOSPHATASE: an. MAGNESIUM. CALCIUM. KINETICS. SODIUM. POTASSIUM. HETEROZYGOTE. AB The biochemical properties of the erythrocyte membrane have been studied in cystic fibrosis patients and in age-matched controls. No significant differences could be observed in either the concentration or composition of the principal membrane constituents. Contrary to previous reports, we found normal levels of the Mg2+-dependent and Ca2+ + Mg2+-dependent ATPase activities in CF preparations. However, a qualitative difference was observed in the Ca2+-dependent ATPase, since the enzyme had relatively greater activity at lower calcium concentrations in the cystic preparations. This effect on the membrane-bound Ca2+-ATPase is unlikely to represent a genetic defect in the enzyme itself but it may be connected with the mechanism of pathogenesis in cystic fibrosis. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 CONOVER JH PEDIATR RES 7 220 973 003 DANES BS J EXP MED 136 1313 972 004 BALFE JW SCIENCE 162 689 968 005 LAPEY A PEDIATR RES 5 446 971 006 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 007 COLE CH PEDIATR RES 6 616 972 008 LOWRY OH J BIOL CHEM 193 265 951 009 DUBOIS M ANAL CHEM 28 350 956 010 BARTLETT GR J BIOL CHEM 234 466 959 011 SACKETT V J BIOL CHEM 64 203 925 012 WEBER K J BIOL CHEM 244 4406 969 013 TAYLOR CB BIOCHIM BIOPHYS ACTA 60 437 962 014 FISKE CH J BIOL CHEM 66 375 925 015 FITZPATRICK DF NATURE NEW BIOL 235 173 972 016 WILKINSON JH BIOCHEM J 80 324 961 017 MARCHESI VT PROC NAT ACAD SCI USA 58 991 967 018 CHERNICK WS J PEDIATR 59 890 961 019 GUGLER EC J PEDIATR 71 585 967 020 BLOMFIELD J ARCH DIS CHILD 48 267 973 021 CHERNICK WS PEDIATRICS 24 739 959 022 KOPITO L NATURE 202 501 964 023 KOPITO L J PEDIATR 68 313 966 024 RULE AH FERTIL STERIL 21 515 970 025 GIBSON LE PEDIATRICS 48 695 971 026 BOAT TF PEDIATRICS 50 343 972 027 JOHANSEN PG LANCET 1 455 968 028 MARSDEN JC NATURE 223 214 969 029 SHWACHMAN H AM J DIS CHILD 96 6 958 030 DODGE JT ARCH BIOCHEM BIOPHYS 100 119 963 031 SCHATZMANN HJ BIOCHIM BIOPHYS ACTA 241 379 971 032 BOND GH BIOCHIM BIOPHYS ACTA 241 393 971 CT 1 MCEVOY FA LANCET 2 236 975 2 WARD JB TEX REP BIOL MED 34 11 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 DISANTAGNESE PA N ENGL J MED 295 597 976 5 KATZ S PEDIATR RES 12 1033 978 6 FODER B CLIN CHIM ACTA 104 187 980 7 ROGIERS V CLIN CHIM ACTA 105 105 980 8 STEGMAYR B ULTRASTRUCTURAL PATHOL 2 357 981 9 MICHELS RC ANN CLIN LAB SCI 11 158 981 10 SORSCHER EJ LANCET 1 368 982 11 KATZ S CELL CALC 5 421 984 12 BERGHOUT AGRV PEDIATR RES 18 1017 984 13 BOUCHER RC PEDIATR RES 18 1336 984 14 DEARBORN DG PEDIATR RES 18 890 984 15 WALLER RL LIFE SCI 35 775 984 16 CLOUGH DL CLIN CHIM ACTA 138 259 984 17 WALLER RL CELL CALC 6 245 985 18 HUNSINGER RN CLIN CHIM ACTA 156 165 986 19 PARSONS HG BIOCHIM BIOPHYS ACTA 860 420 986 PN 74053 RN 00053 AN 75056962 AU Butterworth-J. TI Properties of microsomal glycoprotein galactosyltransferase of cultured human fibroblasts in relation to cystic fibrosis. SO Clin-Chim-Acta. 1974 Oct 30. 56(2). P 159-68. MJ CYSTIC-FIBROSIS: en. FIBROBLASTS: en. HEXOSYLTRANSFERASES: me. MICROSOMES: en. MN CARBON-RADIOISOTOPES. CATTLE. CELLS-CULTURED. COMPARATIVE-STUDY. GALACTOSE. GLYCOPROTEINS. HEAT. HUMAN. HYDROGEN-ION-CONCENTRATION. KINETICS. MANGANESE: pd. METALS: pd. MUCINS: pd. NUCLEOTIDES: pd. SULFHYDRYL-REAGENTS: pd. TIME-FACTORS. POLYETHYLENE-GLYCOLS: pd. URIDINM-EIPHOSPHATE-SUGARS: pd. AB The levels and properties of microsomal glycoprotein galactosyltransferase of cultured skin fibroblasts from control and cystic fibrosis patients were investigated using bovine submaxillary gland mucin as the exogenous acceptor. Although the mean specific activity of this enzyme was higher tin cystic fibrosis fibroblasts, the difference was not significant. The properties of the enzyme as regards pH optimum, metal ion and Triton X-100 requirement, apparent Km, heat stability and nucleotide/sugar-nucleotide response were similar for control and cystic fibrosis fibroblasts. RF 001 DISCHE Z AM J DIS CHILD 102 733 961 002 KNAUFF RE CLIN CHIM ACTA 19 245 968 003 KRAUS I PEDIATRICS 47 1010 971 004 RUSSELL SB J MED GENET 8 441 971 005 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 006$ LOUISOT P C R ACAD SCI (PARIS) 277 533 973 007 LOUISOT P CLIN CHIM ACTA 48 373 973 008 MILLER GL ANAL CHEM 31 964 959 009 MOOKERJEA S CAN J BIOCHEM 50 1082 972 010 KO GKW BIOCHIM BIOPHYS ACTA 244 396 971 011 BARBER AJ BIOCHIM BIOPHYS ACTA 252 533 971 012 KIM YS J CLIN INVEST 51 2024 972 013 PRADAL MB Z NATURFORSCH 26 625 972 014 BOSMANN HB J NEUROCHEM 19 763 972 015 BOSMANN HB BIOCHIM BIOPHYS ACTA 252 369 971 016 RONZIO RA BIOCHIM BIOPHYS ACTA 313 286 973 017 SCHACHTER H J BIOL CHEM 245 1090 970 018 PORETZ RD EUR J BIOCHEM 25 455 972 CT 1 ISENBERG JN AM REV RESPIR DIS 113 567 976 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 SANTAGNESE PAD N ENGL J MED 295 481 976 4 STROUS GJAM BIOCHIM BIOPHYS ACTA 451 201 976 5 HUNT LA J SUPRAMOL STRUCT 7 213 977 6 RAO GJS PEDIATR RES 11 981 977 7 OWEN E J MOL MED 3 49 978 8 ALHADEFF JA CLIN GENET 14 189 978 9 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 10 AGGETT PJ MONOGR PAEDIATR 10 8 979 11 BUTTERWORTH J BIOCHEM SOC TRANS 13 220 985 PN 74054 RN 00054 AN 74120268 AU Duffy-M-J. Cohn-E-V. Schwarz-V. TI Ca2+ uptake and binding by isolated erythrocyte membranes from cystic fibrosis and control subjects. SO Clin-Chim-Acta. 1974 Jan 19. 50(1). P 97-101. MJ CALCIUM: me. CYSTIC-FIBROSIS: bl. ERYTHROCYTES: me. RECEPTORS-DRUG: de. MN ADENOSINE-TRIPHOSPHATASE: bl. ADENOSINE-TRIPHOSPHATE: pd. BINDING-SITES. BIOLOGICAL-TRANSPORT. CELL-MEMBRANE: de, me. COMPARATIVE-STUDY. ERYTHROCYTES: cy, de. HUMAN. PROTEIN-BINDING. AB Ca2+-ATPase activity, high affinity Ca2+ binding in the presence and absence of ATP and low affinity Ca2+ binding at added CaCl2 concentrations of 1 microM, 100 microM and 10 mM were measured in isolated erythrocyte membranes from cystic fibrosis and control subjects. No significant difference was detected between the two groups for any of these measurements. RF 001 GIBSON LE PEDIATRICS 48 695 971 002 MANGOS JA SCIENCE 158 135 967 003 BALFE JW SCIENCE 162 689 968 004 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 005 HARRISON D J PHYSIOL (LOND) 199 367 968 006 FITZPATRICK DF NATURE NEW BIOL 235 173 972 008 LOWRY OH J BIOL CHEM 193 265 951 009 CHA YN J GEN PHYSIOL 57 202 971 010 LONG C BIOCHEM J 132 559 973 011 SUTCLIFFE CH PROC R SOC MED 61 297 968 012 SPOCK A IN: TAKOUSKA S 70 970 013 MANGOS JA PEDIATR RES 1 436 967 014 KAISER D LANCET 1 1003 970 CT 1 COLE CH PEDIATR RES 9 763 975 2 WARD JB TEX REP BIOL MED 34 11 976 3 FODER B CLIN CHIM ACTA 104 187 980 4 KATZ S CELL CALC 5 421 984 5 CLOUGH DL CLIN CHIM ACTA 138 259 984 PN 74055 RN 00055 AN 76116806 AU Wallwork-J-C. Brenchley-P. McCarthy-J. Allan-J-D. Moss-D. Ward-A-M. Holzel-A. Williams-R-F. McFarlane-H. TI Some aspects of immunity in patients with cystic fibrosis. SO Clin-Exp-Immunol. 1974 Nov. 18(3). P 303-20. MJ CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. ALLERGENS. ANTIGEN-ANTIBODY-REACTIONS. CHILD. CHILD-PRESCHOOL. COMPLEMENT-3. FEMALE. FOOD-HYPERSENSITIVITY. GEL-DIFFUSION-TESTS. HUMAN. IGA: an. IGG: an. IGM: an. IMMUNOGLOBULINS-J-CHAIN. INFANT. INFANT-NEWBORN. INTESTINAL-ABSORPTION. LYMPHOCYTE-TRANSFORMATION. MALE. NUTRITION-DISORDERS. LECTINS. LECTINS. PREALBUMIN: an. PRECIPITIN-TESTS. PROTEINS: im. RETICULIN: im. SPUTUM: im. TRANSFERRIN: an. AB Various aspects of the immune status were examined in patients with cystic fibrosis (CF) and the following observations were made. Ten per cent of the CF patients had elevated or reduced serum IgG concentrations and there seems to be a transient low serum IgA concentration in the same number. Serum IgM concentrations were normal in CF patients. Normal levels of the three serum immunoglobulins (IgG, IgA and IgM) were detected in the siblings and heterozygotes. Serum IgE concentrations were elevated in 32% of patients with CF but normal concentrations were detected in heterozygotes and siblings. Precipitating antibodies to various antigens and allergens were detected in the sputum of most patients with CF, but their sera contained lower or undetectable titres of these antibodies. The precipitating antibodies in CF sputum to bacteria, to agar, to seminal fluid, to urine and to human serum may be related to a common antigenic determinant of the glycoproteins present in all these fluids. The total protein concentration in the sputum of CF patients seemed to reflect the extent of lung damage. The third component (C3) of serum complement concentration was normal, or moderately elevated in all CF patients tested. Serum transferrin concentrations were normal in CF patients but their prealbumin levels were depressed, suggesting marginal undernutrition. Peripheral blood lymphocyte transformations with PHA and PWM appear to be normal in CF patients. RF 001 AMMANN AJ LANCET 1 1264 971 002 ANDERSEN DH J PEDIATR 15 763 939 003 BAZARAL M J ALLERGY CLIN IMMUNOL 49 189 971 004 BERATIS NG PEDIATR RES 7 958 973 005 BESLEY GTN J MED GENET 6 278 969 006 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 007 BOWMAN BH SCIENCE 164 325 969 008 BRANDTZAEG P SCAND J HAEMATOL SUPPL 12 1 970 009 BURNS MW LANCET 1 354 967 010 CRIFO S PRACT OTORHINOLARYNGOL 33 394 971 011 DANES BS J EXP MED 137 1538 973 012 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 013 ESSEN R LANCET 1 1157 972 014 FLEISHER DS J PEDIATR 64 341 964 015$ GELL PGH J CLIN PATHOL 10 67 957 016 GRABAR P IN: GRABAR P 3 964 017 HEIDELBERGER M J AM CHEM SOC 77 3511 955 018 HOLSCLAW DS J UROL 106 568 971 019 HUMPHREY JH IMMUNOLOGY FOR STUDENTS OF ME 963 020 IZUMI K CARBOHYD RES 17 227 971 021 JOHANSSEN SGO IMMUNOLOGY 10 265 968 022 KOHN J J CLIN PATHOL 23 733 970 023 LAWSON D IN: WATT PJ 69 970 024 LOWRY OH J BIOL CHEM 193 265 951 025 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 026 MCFARLANE H W AFR MED J 7 149 968 027 MCFARLANE H LANCET 2 1146 971 028 MCFARLANE H SYMP INT TECHNICON 973 029 MCFARLANE H CLIN EXP IMMUNOL 13 153 973 030 MCFARLANE H BR MED J 4 268 970 031 MANCINI G IMMUNOCHEMISTRY 2 235 965 032 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 033 MURRAY MJ ANN INTERN MED 53 548 960 034 NAGASAWA T J IMMUNOL 103 736 969 035 NEVILLE DM J BIOL CHEM 246 6328 971 036 RICHIE RF COLLOQ TECHNICON INT CONG 972 037 ROWE WS BULL WHO 43 609 970 038 SEAH PP LANCET 1 834 971 039 SEAH PP LANCET 2 681 971 040 SEAH PP LANCET 1 611 972 041 SIRISHINHA S LANCET 1 1016 973 042 SMITH FR J LAB CLIN MED 80 423 972 043 SMYTHE PM LANCET 2 939 971 044 SOOTHILL JF J LAB CLIN MED 59 859 962 045 SOUTH MA J PEDIATR 71 645 967 046 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 047 SPOCK A PEDIATR RES 1 173 967 048 STEIN AA J PEDIATR 65 495 964 049 TAYLOR B LANCET 2 111 973 050 TOMASI TB JR N ENGL J MED 287 500 972 051 WAGSTAFF BW PROC AM PEDIATR SOC ANNU MTG 966 052 WHISTLER RL POLYSACCHARIDE CHEMISTRY 227 953 CT 1 MCFARLANE H BR MED J 1 423 975 2 ALLAN JD CLIN ALLERGY 5 255 975 3 WALLWORK JC CLIN CHEM 21 984 975 4 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 5 GIBBONS A BR MED J 1 120 976 6 MCFARLANE H LANCET 1 1241 976 7 WALLWORK JC CLIN ALLERGY 6 349 976 8 WEEKE B DAN MED BULL 23 155 976 9 CLARKE CW THORAX 31 702 976 10 ANON NUTR REV 34 210 976 11 MCFARLANE H PROC NUTR SOC 35 263 976 12 MITCHELLHEGGS P Q J MED 45 479 976 13 WARNER JO ARCH DIS CHILD 51 507 976 14 GALANT SP AM REV RESPIR DIS 114 325 976 15 DISANTAGNESE PA N ENGL J MED 295 597 976 16 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 17 NOVEY HS LANCET 2 249 977 18 GENIN C ARCH FR PEDIATR 34 717 977 19 RAEBURN JA PROC NUTR SOC 36 77 977 20 STRUNK RC ARCH DIS CHILD 52 687 977 21 ROSSMAN CM J PEDIATR 90 579 977 22 ANON LANCET 2 715 978 23 TURNER MW ARCH DIS CHILD 53 631 978 24 HILTON AM BR J DIS CHEST 72 207 978 25 SORENSEN RU J PEDIATR 93 201 978 26 HOIBY N MONOGR PAEDIATR 10 138 979 27 SORENSEN RU INFECT IMMUN 23 398 979 28 CARSWELL F CLIN EXP IMMUNOL 35 141 979 29 HOIBY N ACTA PAEDIATR SCAND 68 495 979 30 HODSON ME THORAX 35 801 980 31 TACIEREUGSTER H HELV PAEDIATR ACTA 35 31 980 32 TOBIN MJ THORAX 35 807 980 33 FICK RB J IMMUNOL METH 38 103 980 34 MIELIVERGANI G ARCH DIS CHILD 55 696 980 35 WILSON GB J CLIN INVEST 66 1010 980 36 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 37 MOSS RB AM REV RESPIR DIS 121 23 980 38 BALTIMORE RS J INFECT DIS 141 238 980 39 HARPER TB LUNG 157 219 980 40 MATTHEWS WJ N ENGL J MED 302 245 980 41 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 42 HODSON ME CLIN ALLERGY 11 565 981 43 LEVINSKY RJ J TROP PEDIATR 27 1 981 44 FICK RB J CLIN INVEST 68 899 981 45 PENNINGTON JE J CLIN INVEST 68 1140 981 46 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 47 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 48 VANGEFFEL R IMMUNOL LETTERS 5 155 982 49 MOSS RB CLIN EXP IMMUNOL 47 301 982 50 GOTZ M CLIN EXP IMMUNOL 50 178 982 51 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 52 LEVINSKY RJ CLIN IMMUNOL ALLERGY 3 441 983 53 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 54 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 91 187 983 55 HODSON ME POSTGRAD MED J 60 225 984 56 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 57 MOSS RB MONOGR ALLERGY 19 202 986 58 DISIS ML PEDIATR RES 20 385 986 59 FERGUSON A HUM NUTR CLIN NUTR 40C 255 986 60 MOSS RB CHEST 91 522 987 PN 74057 RN 00056 AN 74110421 AU Marks-M-B. TI Differential diagnosis of wheezing in children. Remarks based on a postgraduate lecture. SO Clin-Pediatr (Phila). 1974 Mar. 13(3). P 225-8. MJ ASTHMA: di. RESPIRATORY-TRACT-DISEASES: di. MN AUSCULTATION. BRONCHIOLITIS-VIRAL: di. BRONCHITIS: di. CHILD. COUGH. CYSTIC-FIBROSIS: di. DEGLUTITION-DISORDERS: di. DIAGNOSIS-DIFFERENTIAL. FEMALE. FOREIGN-BODIES: di. HABITS. HUMAN. LARYNGITIS: di. MALE. MIDDLE-LOBE-SYNDROME: di. RESPIRATION. RESPIRATORY-HYPERSENSITIVITY: di. EX In order to differentiate asthma from other disorders resembling it, one should attempt to define clearly what we mean by asthma and wheezing. A most simplistic statement would define asthma as a recurring form of dyspnea marked by wheezing breath sounds. The incompleteness of this definition is apparent: asthma is really a symptom complex of labored wheezing breathing, characterized by hyperreactivity of the bronchi to various stimuli, resulting in generalized narrowing of the airways, variable in scope, and often reversible by sympathomimetics, theophylline derivatives, and corticosteroids. A variety of conditions must be considered when studying the wheezing child. These include asthmatic bronchitis, acute bronchitis, spasmodic croup, allergic cough, habit cough, foreign body, cystic fibrosis (mucoviscidosis), right middle lobe syndrome, and upper and lower respiratory tract conditions simulating asthma. RF 001 DEES SC IN: KENDIG EL JR 967 002 EISEN AH PEDIATRICS 31 859 963 003 GLASER J ALLERGY IN CHILDHOOD 956 004 HOWARD WA IN: SPEER F 963 005 LEVIN SJ ANN ALLERGY 22 20 964 006 MARKS MB CLIN PEDIATR 10 479 971 007 PESHKIN MM J ASTHMA RES 6 5 968 008 SALZBERG AM IN: KENDIG EL JR 967 009 TUFT L ALLERGY IN CHILDREN 970 010 WITTIG HJ J ALLERGY 30 19 959 CT 1 KNOOP U MONATSSCHR KINDERHEILKD 125 818 977 PN 74058 RN 00057 AN 74071483 AU Peden-V-H. Powell-K-R. Rejent-A-J. TI Glucose intolerance in a three-year-old child with cystic fibrosis. SO Clin-Pediatr (Phila). 1974 Jan. 13(1). P 10-1 passim. MJ CYSTIC-FIBROSIS: me. GLUCOSE: me. MN ANTIBIOTICS: tu. BLOOD-GLUCOSE: an. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, dt. DIABETES-MELLITUS-INSULIN-DEPENDENT: co, dt. GLUCOSE-TOLERANCE-TEST. GLYCOSURIA: co, dt. HUMAN. INSULIN: bl, tu. MALE. PNEUMONIA: co. TOLBUTAMIDE: tu. AB Glucose intolerance preceding pulmonary disease in a child with cystic fibrosis is described. After glucose intolerance was controlled with tolbutamide, the pulmonary infection resolved. Early recognition and treatment of "diabetes" in children with cystic fibrosis is emphasized. RF 001 ROSAN RC AM J DIS CHILD 104 625 962 002 HANDWERGER S N ENGL J MED 281 451 969 003 KAISER G HELV PAEDIATR ACTA 25 135 970 004 SHWACHMAN H PEDIATRICS 46 335 970 005 MILNER AD ARCH DIS CHILD 44 351 969 006 MORGAN CR DIABETES 12 115 963 007 BERSON SA AM J MED 31 847 961 008 BOSHELL BR METABOLISM 12 108 963 009 MARBLE A JOSLINS DIABETES MELLITUS ED 12 971 010 MILUNSKY A AM J DIS CHILD 121 15 971 011 JOFFE BI LANCET 2 890 968 012 MEGYESI C LANCET 2 1051 967 CT 1 IANNUCCI A HUM PATHOL 15 278 984 PN 74059 RN 00058 AN 75029111 AU Jaffe-B-F. TI Chronic sinusitis in children. Comments on pathogenesis and management. SO Clin-Pediatr (Phila). 1974 Nov. 13(11). P 944-8. MJ SINUSITIS. MN ACUTE-DISEASE. ADENOIDS. ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CLEFT-PALATE: co. CYSTIC-FIBROSIS: co. HAY-FEVER: co. HUMAN. INFANT. INFANT-NEWBORN. KARTAGENER-TRIAD. LYMPHATIC-DISEASES: co. NASAL-POLYPS: et. PARANASAL-SINUSES: ra. PHYSICAL-EXAMINATION. PRESSURE: ae. SINUSITIS: et, co, di, dt, su. VASOCONSTRICTOR-AGENTS-NASAL: tu. EX Chronic sinusitis in young children does not often invoke "sinus" complaints of fullness, discomfort, or pain over the affected sinus, for it is only the older child who verbalizes these complaints. Rather, it is the secondary signs and symptoms that lead the physician to suspect this entity in children. The most common of these are chronic nasal discharge and chronic nasal obstruction. A careful nasal examination is essential for the recognition of chronic sinusitis, the most common predecessor of chronic sinusitis. Allergic rhinitis is probably the second most common background of acute and then chronic sinusitis. Chronic sinusitis in children with cystic fibrosis is a characteristic finding. Sinusitis is common in cleft lip and cleft palate children. Infants and children with clinical signs of chronic infection and radiographic changes should have a nose culture and sensitivity studies to plan appropriate antibiotic therapy. Surgical therapy is indicated when the symptoms, such as chronic pain, chronic otitis media, chronic bronchitis, orbital infections, or intracranial complications, occur. RF 001 BIRRELL JF EAR NOSE AND THROAT DISEA 960 002 BENNER M ARCH PATHOL 29 455 940 003 SCHAEFFER JP NOSE PARANASAL SINUSES NA 920 004 WASSON WW ARCH OTOLARYNGOL 17 197 933 005 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 006 GHARIB R AM J DIS CHILD 108 499 964 007 SHWACHMAN H PEDIATRICS 30 389 962 008 JAFFE BF ARCH OTOLARYNGOL 93 479 971 009 VAN ALYEA OE NASAL SINUSES 207 951 010 GRAHAM WP 3RD CLEFT PALATE J 10 176 973 011 ASCHAN G ACTA OTOLARYNGOL STOCKH 61 371 966 012 KARTAGENER M BEITR KLIN TUBERK 83 489 933 013 OLSEN AM AM REV TUBERC 47 435 943 014 WEISSMAN B LARYNGOSCOPE 82 2160 972 CT 1 JAZBI B ADV OTORHINOLARYNGOL 23 73 978 2 RACHELEFSKY GS J ALLERGY CLIN IMMUNOL 61 310 978 3 KUFTINEC MM CHRONOBIOLOGIA 11 383 984 PN 74060 RN 00059 AN 74250037 AU Boat-T-F. Doershuk-C-F. Stern-R-C. Matthews-L-W. TI Serum alkaline phosphatase in cystic fibrosis. Interpretation of elevated values based on electrophoretic isoenzyme analyses. SO Clin-Pediatr (Phila). 1974 Jun. 13(6). P 505-12. MJ CYSTIC-FIBROSIS: en. ALKALINE-PHOSPHATASE: bl. MN HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: di. AGE-FACTORS. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. ISOENZYMES: an. INTESTINES: en. BONE-AND-BONES: en. LIVER: en. HEPATITIS-TOXIC: en. CHOLESTASIS: en. LIVER-CIRRHOSIS: en, di. ENZYME-TESTS. HEART-FAILURE-CONGESTIVE: co. FINGERS: ab. ABNORMALITIES: co. DIAGNOSIS-DIFFERENTIAL. SEX-FACTORS. AB Sera from 31 of 146 patients with cystic fibrosis (CF) contained levels of serum alkaline phosphatase (AP) which were elevated by age-related criteria. Prominent liver isoenzyme bands were present after polyacrylamide gel electrophoresis of all 31 abnormal sera. Larger than normal amounts of bone and intestinal AP were not present in these sera. These findings indicate that liver abnormalities consistently are responsible for elevations of total AP in sera of patients with CF. Several causes of high liver AP levels in CF patients were identified: chronic hepatic congestion, drug hepatotoxicity, overt biliary cirrhosis, and most commonly subclinical focal biliary cirrhosis. RF 001 KAPLAN MM GASTROENTEROLOGY 62 452 972 002 KATTWINKEL J J PEDIATR 82 234 973 003 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 004 DI SANTAGNESE PA PEDIATRICS 18 387 956 005 CRAIG JM AM J DIS CHILD 93 357 957 006 KOPEL FB GASTROENTEROLOGY 62 483 972 007 SIMOPOULOS AP PEDIATR RES 6 355 972 008 WINKELMAN J AM J CLIN PATHOL 57 625 972 009 BODANSKY AJ J BIOL CHEM 101 93 933 010 CLARK LC JR J PEDIATR 36 335 950 011 BESSEY OA J BIOL CHEM 164 321 946 012 KAPLAN MM LANCET 2 1029 969 013 MATTHEWS LW J PEDIATR 65 558 964 014 RICHMAN SM AM J MED 30 211 961 CT 1 ALHADEFF JA CLIN GENET 10 63 976 2 STERN RC GASTROENTEROLOGY 70 645 976 3 WOOD RE AM REV RESPIR DIS 113 833 976 4 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 5 HOWIE AD SCOTT MED J 24 193 979 6 MIELIVERGANI G ARCH DIS CHILD 55 696 980 7 PARK RW GASTROENTEROLOGY 81 1143 981 8 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 9 BASS S GASTROENTEROLOGY 84 1592 983 10 STEAD RJ THORAX 42 59 987 PN 74061 RN 00060 AN 74149626 AU Richards-W. TI Differential diagnosis of childhood asthma. SO Curr-Probl-Pediatr. 1974 Mar. 4(5). P 1-36. (REVIEW). MJ ASTHMA: di. MN AGE-FACTORS. ASTHMA: co, dt, pp, ra, th. CHILD. CHILD-HEALTH-SERVICES. CHILD-INSTITUTIONALIZED. CYSTIC-FIBROSIS: di. DEFICIENCY-DISEASES: di. DIAGNOSIS-DIFFERENTIAL. EPHEDRINE: tu. FOREIGN-BODIES: co. HEART-DEFECTS-CONGENITAL: co. HUMAN. NEOPLASMS: co. PSYCHOTHERAPY. RESPIRATORY-HYPERSENSITIVITY: di. RESPIRATORY-SYSTEM: ab. RESPIRATORY-TRACT-INFECTIONS: di. RESPIRATORY-TRACT-NEOPLASMS: di. REVIEW. THEOPHYLLINE: tu. EX "Asthma," according to the United States National Tuberculosis Association, "is a disease characterized by an increased responsiveness of the trachea and bronchi to various stimuli, and made manifest by difficulty of breathing due to generalized narrowing of the airways. This narrowing is dynamic and changes in degree, either spontaneously or because of therapy. The basic defect appears to be an altered state of the host." The maxim, "All that wheezes is not asthma," justifiably reinforces the importance of differential diagnosis; indeed, the characteristic features of asthma may be found to some degree in a wide variety of chest conditions or general systemic diseases. The purposes of this monograph are to review those conditions that may be confused with asthma and to focus attention on the specific points of importance in the history, physical examination and laboratory test results that might provide clues to the final diagnosis. RF 001 ANON CHRONIC OBSTRUCTIVE PULMONARY 967 002 KENDIG EL JR PULMONARY DISORDERS 972 003 CHANG N PEDIATRICS 41 739 968 004 LEVIN SJ ANN ALLERGY 22 20 964 005 GUPTA TCM AM J DIS CHILD 115 88 968 006 BROWDER JA AM J DIS CHILD 119 322 970 007 WANDERER AA PEDIATRICS 44 709 969 008 DEPAREDES CG J PEDIATR SURG 5 136 970 009 GLASGOW JFT AM J MED 54 181 973 010 TALAMO RC J PEDIATR 79 20 971 011 LENCZNER M CAN MED ASSOC J 91 421 964 012 GELL PGH CLINICAL ASPECTS OF IMMUNOLOG 968 013 MCCOMBS RP N ENGL J MED 286 1186 972 014 SLAVIN RG J PEDIATR 76 416 970 015 GORDON DS AM REV RESPIR DIS 108 127 973 016 SAHN SA CHEST 63 286 973 017 KATZ RM N ENGL J MED 288 233 973 018 OCONNELL EJ AM J DIS CHILD 108 302 964 019 STIEHM ER PEDIATRICS 39 904 967 020 BANASZEK EF N ENGL J MED 283 271 970 021 NICHOLSON DP AM J MED 53 131 972 022 BRETTNER A RADIOLOGY 96 31 970 023 WEISEL W PEDIATRICS 28 394 961 024 REPETTO G PEDIATRICS 40 24 967 025 BRYAN MH PEDIATRICS 52 169 973 026 CAYLER GG AM J DIS CHILD 118 708 969 027 STANGER P PEDIATRICS 43 760 969 028 ERAKLIS AJ N ENGL J MED 281 1150 969 029 LEONIDAS JC J PEDIATR 83 628 973 030 MATSANIOTIS N ARCH DIS CHILD 42 308 967 031 RILEY CM PEDIATRICS 37 435 966 032 KAPPELMAN MM AM J DIS CHILD 118 568 969 033 WEITZMAN JJ J PEDIATR 73 329 968 034 MCNAMARA JJ PEDIATRICS 43 527 969 035 SHELDON WH J PEDIATR 61 780 962 036 SCHNEIDER RM N ENGL J MED 277 1056 967 037 ONEILL RP ANN INTERN MED 67 957 967 038 DANIGELIS JA AM J DIS CHILD 118 871 969 039 HODGMAN JE PEDIATRICS 44 179 969 040 KRAVITZ H CLIN PEDIATR 8 580 969 041 COMROE JH JR N ENGL J MED 282 1249 970 042 PENDARVIS BC AM J ROENTG RAD THER NUCL MED 107 313 969 CT 1 KNOOP U MONATSSCHR KINDERHEILKD 125 818 977 2 GERSHEL JC N ENGL J MED 309 336 983 PN 74062 RN 00061 AN 74250648 AU Hawley-H-B. Lewis-R-M. Swartz-D-R. Gump-D-W. TI Tobramycin therapy of pulmonary infections in patients with cystic fibrosis. SO Curr-Ther-Res. 1974 May. 16(5). P 414-23. MJ ANTIBIOTICS: tu. LUNG-DISEASES: dt. BACTERIAL-INFECTIONS: dt. CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: dt. STAPHYLOCOCCAL-INFECTIONS: dt. MN HUMAN. ANTIBIOTICS: bl, pd, an. AMINOGLYCOSIDES: tu, bl, pd, an. LUNG-DISEASES: et, mi. SPUTUM: mi, an. PSEUDOMONAS: ip, de. STAPHYLOCOCCUS: ip, de. AB Six patients having exacerbations of chronic pulmonary infections in association with cystic fibrosis were treated with intramuscular tobramycin 5 mg./kg./day in 3 divided doses. The eight treatment courses lasted 9 to 30 days. Mean serum levels were 2.5 microg./ml. at 1 to 3 hours and 0.2 microg./ml. at 7 1/2 to 8 hours after a dose of tobramycin. The mean tobramycin concentration in sputum was 0.3 microg./ml. Eighty-two percent of the 66 pathogenic bacterial isolates from sputum were either Pseudomonas sp. (64%) or Staphylococcus aureus (18%). Ninety-seven percent of Pseudomonas sp. and 83 percent of Staphylococcus aureus were inhibited by 3.1 microg. tobramycin per ml. No organism that was isolated before therapy was observed to become resistant to tobramycin, although on 3 occasions colonization with new resistant gram-negative bacilli occurred. In 6 out of 8 courses of therapy patients showed clinical improvement. Elimination of bacterial pathogens from the sputum was rarely achieved and was not necessary to produce clinical improvement. Colonization with resistant flora was an uncommon finding. Tobramycin's side effects were limited to local discomfort at the injection site, nausea and vomiting. RF 001 POGUE RE MINN MED 52 1551 969 002 SHWACHMAN H MINN MED 52 1521 969 003 HUANG NN J PEDIATR 59 512 961 004 IACOCCA VF AM J DIS CHILD 106 315 963 005 KOCH KF IN: HOBBY GL 309 971 006 BLACK HR ANTIMICROB AGENTS CHEMOTHER 11 314 971 007 RAWLINS GA LANCET 2 538 953 008 WARREN E ANTIMICROB AGENTS CHEMOTHER 1 46 972 009 FISHER MW J BACTERIOL 98 835 969 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 SHADOMY S ANTIMICROB AGENTS CHEMOTHER 1 412 972 012 DIENSTAG J ANTIMICROB AGENTS CHEMOTHER 1 41 972 013 KIRBY WMM J CLIN INVEST 24 165 945 014 MARKS MI J PEDIATR 79 822 971 015 HUANG NN J PEDIATR 78 338 971 CT 1 BROGDEN RN DRUGS 12 166 976 2 MCCRAE WM SCOTT MED J 21 68 976 3 MCCRACKEN GH J PEDIATR 88 315 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 BENDUSH CL J INFECT DIS 134 S219 976 6 MCCRAE WM J INFECT DIS 134 S191 976 7 NEU HC J INFECT DIS 134 S 3 976 8 PARRY MF J INFECT DIS 134 S194 976 9 LAU WK PEDIATRICS 60 372 977 10 ATTERHOLM I SCAND J INFECT DIS 10 229 978 11 PAPORISZ U MONOGR PAEDIATR 10 31 979 12 FRIIS B SCAND J INFECT DIS 11 211 979 13 KRAUSE PJ CURR THER RES CLIN EXP 25 609 979 14 PAPORISZ U EUR J PEDIATR 130 259 979 15 MARTIN AJ ARCH DIS CHILD 55 604 980 16 WIENTZEN R AM J DIS CHILD 134 1134 980 17 MOMBELLI G ANTIMICROB AGENTS CHEMOTHER 19 72 981 18 HYATT AC J PEDIATR 99 307 981 19 MALMBORG AS SCAND J INFECT DIS 1981 64 981 20 SANDE MA ARCH INTERN MED 142 2033 982 21 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 22 BERGOGNEBEREZIN E PRESSE MED 12 287 983 23 GUAY DRP DRUG INTEL CLIN PHARM 17 83 983 24 SZAFF M ACTA PAEDIATR SCAND 72 651 983 25 LEVY J J PEDIATR 105 117 984 26 RODRIGUEZ JR ANTIMICROB AGENTS CHEMOTHER 27 246 985 27 RODRIGUEZ JR AM J MED 78 42 985 28 MENDELMAN PM AM REV RESPIR DIS 132 761 985 29 MARLIN GE AM REV RESPIR DIS 134 1209 986 30 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 74063 RN 00062 AN 75038260 AU Lykkegaard-E. Jacobsen-L. TI Serum protein paper electrophoresis in cystic fibrosis correlated with bacteriological lung findings. SO Dan-Med-Bull. 1974 Oct. 21(6). P 241-5. MJ BLOOD-PROTEINS: me. CYSTIC-FIBROSIS: bl. LUNG: mi. MN ADOLESCENCE. BLOOD-PROTEIN-ELECTROPHORESIS. CHILD. CYSTIC-FIBROSIS: mi. ELECTROPHORESIS-PAPER. HUMAN. PSEUDOMONAS-AERUGINOSA: ip. STAPHYLOCOCCUS: ip. AB Serum proteins have been studied by means of paper electrophoresis in 88 patients with cystic fibrosis (CF), and correlated with bacteriological lung findings. An increase of all serum globulin fractions in CF patients was found, whereas albumin and albumin/globulin ratio were decreased. The changes in all serum protein fractions were significantly greater in patients with constant Pseudomonas aeruginosa infection in the lungs than in patients without this constant infection (p < 0.005), whereas patients with intermittent pseudomonas infection did not have more severe changes than patients without pseudomonas infection. The changes in gamma-globulin and total globulin were significantly greater in patients with frequent Staphylococcus aureus infections than in patients with rare Staphylococcus aureus infections (p < 0.005 and p < 0.05, respectively). As regards the other serum protein fractions, no significant differences between patients with frequent and rare staphylococcus infections were found. Judged by simple scoring systems, the lung disease of patients with constant pseudomonas infection was significantly more progressed clinically and radiologically than that of patients without constant pseudomonas infection (p < 0.00005), whereas no significant differences were found between the other patient groups. RF 001 BURNS MW LANCET 1 270 968 002 CROSATO M MINERVA PEDIATR 20 2326 968 003 DENNING CR AM J DIS CHILD 94 538 957 004 DIAZ F J INFECT DIS 121 269 970 005 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 006 DOGGETT RG INFECT IMMUN 6 628 972 007 GREEN MN AM J DIS CHILD 100 365 960 008 GREEN MN J LAB CLIN MED 63 416 964 009 HABBOUSHE C PEDIATRICS 48 973 971 010 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 012 IACOCCA VF AM J DIS CHILD 106 315 963 013 LAURELL CB CLIN CHEM 2 99 956 014 LYKKEGAARD E DAN MED BULL 21 232 974 015 MAY JR ARCH DIS CHILD 47 908 972 016 MEARNS MB ARCH DIS CHILD 47 902 972 017 OWEN JA ADV CLIN CHEM 1 237 958 018 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 019 PITTMAN FE AM J DIS CHILD 108 360 964 020 STROBER W PEDIATRICS 43 416 969 CT 1 LYKKEGAARD E DAN MED BULL 21 232 974 2 WEEKE B DAN MED BULL 23 155 976 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 4 GOSCINIAK G ARCH IMMUNOL THER EXP 28 619 980 5 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 74064 RN 00063 AN 75038259 AU Lykkegaard-E. Jacobsen-L. Flensborg-E-W. TI Serum protein paper electrophoresis in cystic fibrosis correlated with clinical and radiological findings. SO Dan-Med-Bull. 1974 Oct. 21(6). P 232-40. MJ BLOOD-PROTEINS: me. CYSTIC-FIBROSIS: bl. MN ADOLESCENCE. ADULT. BLOOD-PROTEIN-ELECTROPHORESIS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: ra. ELECTROPHORESIS-PAPER. FOLLOW-UP-STUDIES. HUMAN. INFANT. EX Few studies of serum proteins by means of paper electrophoresis in patients with cystic fibrosis (CF) have been published. In these studies alterations of the serum protein fractions were found, some of which were significantly correlated with the clinical and radiological severity of the disease. In the present study, serum proteins determined by paper electrophoresis in 95 patients with CF have been studied and correlated with clinical and radiological findings. The serum proteins and electrophoretic patterns if 95 patients with CF were followed for 1 to 6 years in each patient. For CF patients as a whole, total serum protein was unchanged, whereas albumin and albumin/globulin ratio were decreased and all globulin fractions increased. Total serum protein was increased in patients with severe pulmonary disease. The changes in total serum protein and in all protein fractions were significantly correlated with clinical and radiological severity of the lung disease. All terminal patients had severe changes. Patients with complicating liver cirrhosis had electrophoretic patterns that did not differ from those of other CF patients. It is concluded that the main cause for serum protein alterations in CF is the lung affection, whereas liver involvement and malabsorption probably are of much less importance. RF 001 CRAIG JM AM J DIS CHILD 90 299 955 002 CROSATO M MINERVA PEDIATR 20 2326 968 003 DENNING CR AM J DIS CHILD 94 538 957 004 DI SANTAGNESE PA PEDIATRICS 18 387 956 005 DOERSHUK CF IN: GREEN M 707 968 006 DOGGETT RG INFECT IMMUN 6 628 972 007 DOLAN TF JR CLIN PEDIATR 9 295 970 008 FLEISHER DS J PEDIATR 64 341 964 009 GRASSMANN W HOPPE SEYLERS Z PHYSIOL CHEM 290 1 952 010 GREEN MN AM J DIS CHILD 100 365 960 011 GREEN MN J LAB CLIN MED 63 416 964 012 GUGLER EC J PEDIATR 73 548 968 013 HALBERT SP MOD PROBL PEDIATR 10 144 967 014 LAURELL CB CLIN CHEM 2 99 956 015 LYKKEGAARD E DAN MED BULL 21 241 974 016 NEBERT DW CALIF MED 104 57 966 017 NORMAN AP PROC EWGCF 2ND ANNU MTG 971 018 OWEN PA ADV CLIN CHEM 1 237 958 019 PITTMAN FE AM J DIS CHILD 108 360 964 020 RICHTERICH R KLINISCHE CHEMIE 965 021 ROBERTS WC AM J MED 32 324 962 022 SCHWARTZ RH AM J DIS CHILD 111 408 966 023 SHWACHMAN H IN: KENDIG EL JR 541 967 024 SHWACHMAN H AM J DIS CHILD 96 6 958 026 STROBER W PEDIATRICS 43 416 969 027 WEBSTER R ARCH DIS CHILD 28 343 953 028 WILROY RS JR J PEDIATR 68 67 966 CT 1 LYKKEGAARD E DAN MED BULL 21 241 974 2 LYKKEGAARD E DAN MED BULL 22 169 975 3 WEEKE B DAN MED BULL 23 155 976 4 KAMPER J ANN RADIOL (PARIS) 20 95 977 5 KAMPER J ACTA PAEDIATR SCAND 67 53 978 6 DOMINICK HC LANCET 2 1229 981 7 BRANCHINI BR LANCET 1 618 982 8 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 9 BRANCHINI BR PEDIATR RES 17 850 983 10 WILSONSHARP RC ARCH DIS CHILD 59 923 984 PN 74065 RN 00064 AN 74166363 AU Pedersen-J-T. Lykkegaard-E. Faero-O. Jacobsen-L. TI Lung function in children and young adults with cystic fibrosis. SO Dan-Med-Bull. 1974 Mar. 21(1). P 12-9. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. BODY-HEIGHT. BODY-WEIGHT. BONE-DEVELOPMENT. CHILD. FEMALE. HUMAN. RESPIRATORY-THERAPY. MALE. PLETHYSMOGRAPHY-WHOLE-BODY. RESPIRATORY-FUNCTION-TESTS. VITAL-CAPACITY. AB The main characteristic of patients with cystic fibrosis is lung involvement; the fatal outcome of the disease is most often due to pulmonary complications. Since the paper by West et al. (1954) on pulmonary function in cystic fibrosis, the importance of lung function studies in this disorder has been stressed in several publications. We have studied lung function in a number of children and young adults with cystic fibrosis. The results have been correlated with clinical, radiological, and bacteriological findings. RF 001 BEIER FR AM REV RESPIR DIS 94 430 966 002 COOK CD PEDIATRICS 24 181 959 003 DEMUTH GR AM J DIS CHILD 103 129 962 004 ESTERLY JR THORAX 23 670 968 005 FAIRBAIRN AS THORAX 17 168 962 006 FEATHERBY EA AM REV RESPIR DIS 102 737 970 007 GANDEVIA B ARCH DIS CHILD 34 511 959 009 GOLDRING RM J PEDIATR 65 501 964 010 GREULICH WW RADIOGRAPHIC ATLAS OF SKELETA 959 011 HARRISON GM AM J DIS CHILD 100 530 960 012 HOYBYE G UGESKR LAEGER 132 2007 970 013 LIFSCHITZ MI AM REV RESPIR DIS 99 399 969 014 MATTHEWS LW MOD PROBL PEDIATR 10 237 967 015 MEARNS MB ARCH DIS CHILD 43 528 968 016 MELLINS RB PEDIATRICS 41 560 968 017 NORMAN AP PROC EWGCF 2ND ANNU MTG 971 018 POLGAR G AM REV RESPIR DIS 85 319 962 019 POLGAR G PULMONARY FUNCTION TESTING IN 971 020 REILLY BJ RADIOLOGY 98 281 971 021 SHWACHMAN H PEDIATRICS 36 689 965 022 SPROUL A J PEDIATR 65 664 964 023 TANNER JM ARCH DIS CHILD 41 613 966 024 TOMASHEFSKI JF CHEST 57 28 970 025 WARWICK WJ J ASTHMA RES 5 277 968 026 WEST JR PEDIATRICS 13 155 954 027 ZAPLETAL A PEDIATRICS 48 64 971 028 ZELKOWITZ PS AM J DIS CHILD 117 543 969 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) 83 459 975 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 PROCTOR DF AM REV RESPIR DIS 115 97 977 4 GOSCINIAK G ARCH IMMUNOL THER EXP 28 619 980 5 MOLLER NE EUR J RESPIR DIS 63 130 982 6 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 7 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 PN 74066 RN 00065 AN 75038262 AU Steinrud-J. Winkel-S. Flensborg-E-W. TI Screening for cystic fibrosis with chloride electrode. An investigation of sweat chloride with chloride electrode orion 417 in normal persons and in patients with cystic fibrosis. SO Dan-Med-Bull. 1974 Oct. 21(6). P 251-5. MJ CHLORIDES: an. CYSTIC-FIBROSIS: me. SWEAT: an. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. ELECTRIC-STIMULATION. FEMALE. HUMAN. INFANT. IONTOPHORESIS: is. MALE. MASS-SCREENING. AB An investigation of sweat chloride with chloride electrode Orion 417 in normal persons and in patients with cystic fibrosis. Sweat chloride values examined with Orion 417 direct-reading skin electrode were collected in a group of 811 normal persons and a group of 91 patients with cystic fibrosis and were evaluated for differences attributable to age and sex. In the normal group no significant difference was found between the two sexes. In the normal group the values among newborns are found to be considerably higher than in the rest of childhood. During the first month of life the values decrease to a steady level, which is maintained from the age of 1 month to 15 years. After the age of 15 a steady increase takes place. With data gathered from the normal group we propose the following upper limits of "normal" for the sweat chloride test: 1 month - 15 years: Chloride 40 meq/l. 16 - 25 years: Chloride 55 meq/l. Upon examination of the Cystic Fibrosis group, the sweat chloride values in all age groups were found to be above the upper limits of normal. The method is found valuable for large scale screening purposes for Cystic Fibrosis. RF 001 ANDERSON CM ARCH DIS CHILD 35 581 960 002 DOERSHUK CF J PEDIATR 65 677 964 003 FLENSBORG EW PROC EWGCF 1ST ANNU MTG 970 004 GIBSON LE PEDIATRICS 23 545 959 005 GRONBAEK P UGESKR LAEGER 122 1109 960 006 HANSEN L AM J CLIN PATHOL 49 834 968 007 KOPITO L PEDIATRICS 43 794 969 008 KULCZYCKI LL AM J DIS CHILD 100 174 960 009 NIELSEN EL UGESKR LAEGER 133 1017 971 010 SHWACHMAN H PEDIATRICS 46 335 970 011 SPROUL A J PEDIATR 69 759 966 012 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 013 STEPHAN U IN: MANGOS JA 281 973 CT 1 KOLLBERG H SCAND J CLIN LAB INVEST 35 17 975 2 WARWICK WJ CLIN CHEM 24 2050 978 3 DANES BS MED HYPOTHESES 5 289 979 4 NIELSEN OH J PEDIATR GASTROENTEROL NUTR 1 355 982 5 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 74067 RN 00066 AN 75029287 AU Kaiser-D. Drack-E. TI Diminished excretion of bicarbonate from the single sweat gland of patients with cystic fibrosis of the pancreas. SO Eur-J-Clin-Invest. 1974 Aug. 4(4). P 261-5. MJ BICARBONATES: se. CYSTIC-FIBROSIS: me. SWEAT-GLANDS: se. MN ADULT. BIOLOGICAL-TRANSPORT. BODY-WATER: me. CELL-MEMBRANE-PERMEABILITY. CHILD. CHILD-PRESCHOOL. EPITHELIUM. FEMALE. HUMAN. HYDROGEN-ION-CONCENTRATION. MALE. MICROELECTRODES. PANCREATIC-DISEASES: me. POTASSIUM: me. SODIUM: me. SWEAT: an. WATER-ELECTROLYTE-BALANCE. EPITHELIUM: cy. AB Using pH-microelectrodes the excretion of hydrogen-ions and bicarbonate in relation to flow rate was investigated in a single sweat gland of cystic fibrosis children. The results show decreased excretion of hydrogen-ions and an increased excretion of bicarbonate. Evidence is presented that due to reduced secretion of hydrogen-ions in the duct both the reabsorption of sodium and the secretion of potassium may be impaired. Reduced flux of protons across the luminal membrane of epithelial cells is proposed as a general feature of exocrine gland defects. It is then possible to describe both the cationic transport defect of the sweat gland duct and the anionic bicarbonate-secretory defect in one term. RF 001 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 002 BOWMAN BH SCIENCE 167 871 970 003 BRAUN-FALCO O ARCH KLIN EXP DERM 201 73 955 003A BRUSILOW SW AM J PHYSIOL 214 513 968 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 DREILING DA GASTROENTEROLOGY 29 262 955 006 EMRICH HM KLIN WOCHENSCHR 48 966 970 007 HADORN B IN: LAWSON D PROC 5TH INT CF 56 969 008 KAISER D PEDIATR RES 5 167 971 009 KAISER D HELV PAEDIATR ACTA 26 551 971 011 KAISER D IN: MANGOS JA 247 973 012 MALNIC G AM J PHYSIOL 211 529 966 013 MALNIC G KIDNEY INT 1 280 972 014 MANGOS JA PEDIATR RES 2 378 968 015 RAO GJS J PEDIATR 80 573 972 016 RAO GJS SCIENCE 177 610 972 017 RAWLS JA AM J PHYSIOL 205 651 963 018 SATO K J CLIN INVEST 52 2166 973 019 SCHULZ IJ PFLUEGERS ARCH 284 360 965 020 SCHULZ IJ IN: MUKOVISZIDOSE 12 968 021 SCHULZ IJ PROC INT CONG PEDIATR 13TH 2 523 971 022 SLEGERS JFG DERMATOLOGICA 127 242 963 023 SPOCK A PEDIATR RES 1 173 967 024 UHLICH E PFLUEGERS ARCH 303 31 968 025 YOUNG JA PFLUEGERS ARCH 295 157 967 CT 1 SATO K REV PHYSIOL BIOCHEM PHARMACOL 79 51 977 2 QUINTON PM PEDIATR RES 16 533 982 3 SHIFFMAN ML PEDIATR RES 17 486 983 4 BRIGGMAN JV AM J PATHOL 112 250 983 5 KESTEL FG KLIN PAEDIATR 195 330 983 6 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 7 BIJMAN J PEDIATR RES 21 79 987 PN 74068 RN 00067 AN 77050936 AU Bearn-A-G. TI The expression of inherited metabolic disease in cultured cells. SO Harvey-Lect. 1974-75. 70 Series. P 75-91. MJ CELLS-CULTURED. CYSTIC-FIBROSIS: me. METABOLISM-INBORN-ERRORS: me. MN HUMAN. LIPOCHONDRODYSTROPHY: me. SUPPORT-U-S-GOVT-NON-P-H-S. EX Since 1900 improvements in general hygiene, and the development of antibiotic therapy have enabled many of the epidemic infectious diseases, particularly those of infancy and early childhood, increasingly to be controlled. In contrast, the morbidity and mortality of diseases of genetic origin have been little affected. The rational use of cell culture as an investigative technique in inherited metabolic disease is rooted in the simple biologic truth that, since a full complement of chromosomes is present in all somatic cells, it should be possible to detect the specific biochemical consequences of gene activity in cultured fibroblasts unless, during the process of differentiation, genes are irreversibly inactivated. The usefulness of tissue culture in investigating inherited metabolic disease does not rest with the biochemical identification of the primary defect. The cells in culture provide a model system of the disease, which can be used to test the possible effectiveness of therapeutic agents. Tissue culture techniques indicated that the cells derived from patients with cystic fibrosis showed metachromasia. Although the isolation and characterization of cystic fibrosis from cystic fibrosis serum has not been accomplished, a number of properties have been described. RF 001 BATESON W ESSAYS AND ADDRESSES 93 928 002 BEARN AG N ENGL J MED 286 764 972 003 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 004 BERGGARD I AM J MED 39 221 965 005 BOWMAN BH TEX REP BIOL MED 31 611 973 006 BOWMAN BH SCIENCE 164 325 969 007 BOWMAN BH SCIENCE 167 871 970 008 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 009 BRANTE G SCAND J CLIN LAB INVEST 4 43 952 010 CARREL A IN: NOBEL LECTURES PHYSIOLOGY 444 964 011 CHILDS B IN: SUTTON HE 3 972 012 CONOVER JH PEDIATR RES 7 220 973 013 CONOVER JH LANCET 2 1501 973 014 DANES BS J EXP MED 123 1 966 015 DANES BS J EXP MED 124 1181 966 016 DANES BS J EXP MED 126 509 967 017 DANES BS J EXP MED 129 775 969 018 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 019 DANES BS J EXP MED 132 765 970 020 DANES BS J EXP MED 137 1538 973 022 DARLINGTON GJ SCIENCE 185 859 974 023 DI SANTAGNESE PA PEDIATRICS 12 549 953 024 DORFMAN A PROC NAT ACAD SCI USA 43 443 957 025 FRATANTONI JC PROC NAT ACAD SCI USA 60 699 968 026 GARROD AE LANCET 2 1 908 027$ GARROD AE BR MED J 747 924 028 GARROD AE Q J MED 6 242 913 029 HARRISON RG HARVEY LECTURES 3 199 907 030 HERZBERG V J CLIN INVEST 52 2732 973 031 KESSLER WR PEDIATRICS 8 648 951 032 KEYNES G LIFE OF WILLIAM HARVEY 368 966 033 LOCKHART LH TEX REP BIOL MED 31 631 973 034 LYON MF AM J HUM GENET 14 135 962 035 MCKUSICK VA HERITABLE DISORDERS OF CONNEC 972 036 MCKUSICK VA MENDELIAN INHERIT IN MAN 975 037 MCKUSICK VA LANCET 1 993 972 038 MANGOS JA SCIENCE 158 135 967 039 MEYER K PROC SOC EXP BIOL MED 97 275 958 040 NEUFELD EF IN: MCKUSICK VA 141 973 041$ NEUFELD EF IN: STEINBERG AG 10 81 974 042 POLLEY MJ J MED GENET 11 249 974 044 SPOCK A PEDIATR RES 1 173 967 045 WHITTERIDGE G WILLIAM HARVEY AND THE CIRCUL 971 PN 74069 RN 00068 AN 75059993 AU Mavromichalis-J. Zannos-Mariolea-L. Nicolaidou-M. Georgatou-P. Dentaki-Svolaki-K-DENTAKIAASVOLAKI-K. Matsaniotis-N. TI Hematological findings in Greek children with cystic fibrosis. SO Helv-Paediatr-Acta. 1974 Jun. 29(2). P 151-5. MJ CYSTIC-FIBROSIS: bl. ERYTHROCYTES. HEMOGLOBINS. IRON: bl. MN BONE-MARROW: me. CHYMOTRYPSIN: tu. CYSTIC-FIBROSIS: dt. GREECE. HEMATOCRIT. HEMOGLOBINS: an. HEMOSIDERIN: an. HUMAN. INFANT. PANCREATOPEPTIDASE: tu. PROTEIN-BINDING. TRYPSIN: tu. AB 43 children with cystic fibrosis, of whom 16 had not received pancreatic extracts, were investigated hematologically. No difference was observed in mean hemoglobin and serum iron levels and bone marrow non hemoglobin iron between patients receiving and those not receiving pancreatic enzymes. A significant proportion of children in both groups were found to have mild iron deficiency. These findings are in agreement with the view that pancreatic insufficiency does not increase iron absorption. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BIGGS JC LANCET 2 814 963 003 CRAIG JM AM J DIS CHILD 93 357 957 004 DACIE V PRACTICAL HAEMATOLOGY 966 005 DAVIS AE LANCET 2 749 961 006 DAVIS AE LANCET 2 6 962 007 DAVIS AE AUSTRALAS ANN MED 13 201 964 008 DELLER DJ AM J DIG DIS 10 249 965 010 KATTAMIS C ARCH HELLIN PAEDIAT HETAIR 36 209 973 011 KAVIN H GUT 8 556 967 012 KINNEY TD AM J PATHOL 26 746 950 013 KINNEY TD J EXP MED 102 151 955 014 LONGNECKER DS ARCH PATHOL 80 148 965 015 MATSANIOTIS N PAIDIATRIKI 1 63 972 016 METAXOTOU-MAVROMMA A THESIS 971 017 MURRAY MJ GASTROENTEROLOGY 53 38 967 018 MURRAY MJ GASTROENTEROLOGY 51 694 966 019 SCHADE AL PROC SOC EXP BIOL MED 87 443 954 020 SMITH NJ PEDIATRICS 16 166 955 021 STROHMEYER G DTSCH MED WSCHR 95 2067 970 022 TAYLOR J J PATHOL BACTERIOL 34 793 931 023 TAYLOR J J PATHOL BACTERIOL 41 397 935 CT 1 WARWICK WJ AM J MED TECHNOLOGY 48 539 982 2 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 PN 74070 RN 00069 AN 74285151 AU Cunningham-D-G. TI Clinical variations of cystic fibrosis. SO Ill-Med-J. 1974 Jun. 145(6). P 493-6. MJ CYSTIC-FIBROSIS: di. SWEATING. MN CHILD. ADOLESCENCE. HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: co, fg. DIAGNOSIS-DIFFERENTIAL. MASS-SCREENING. GASTROINTESTINAL-DISEASES: co. LIVER-DISEASES: co. RESPIRATORY-TRACT-DISEASES: co. NASAL-POLYPS: co. AB It is estimated that cystic fibrosis affects approximately one of every 2,000 white children born in the United States, and is now generally regarded as the commonest serious metabolic disorder of white children with the exception of diabetes mellitus. Its occurrence in children of Afro-Americans is quite rare and exceedingly so in those of Oriental extraction. Since cystic fibrosis is genetically determined as an autosomal recessive trait, and since the obligate heterozygote parent is entirely asymptomatic, the disease on its first occurrence in a given family can only be suspected by its manifestations in the patient himself. At present there is no test available for the detection of cystic fibrosis which can be applied as a simple, practical screening test, such as is available for phenylketonuria. Diagnostic tests are, therefore, used to confirm or refute the diagnosis of cystic fibrosis when suggestive symptoms arise or when a sibling or relative is known to have the disease. CT 1 PEARSON RD PEDIATR RES 13 834 979 2 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 74071 RN 00070 AN 77072255 AU Seakins-J-W. Ersser-R-S. TI Use of a semi-synthetic amino acid, 3-methoxyphenyl-L-alanine to measure amino acid absorption. pp. 304-6. SO In: Bickel H, Stern J, ed. Inborn errors of calcium and bone metabolism. Baltimore, Univ Park Press, 1976. WE 250 S678i 1974. MJ AMINO-ACIDS: me. PHENYLALANINE: aa. MN ANIMAL. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. HUMAN. MONOGRAPH. PHENYLALANINE: cs, me. RATS. AB This paper describes investigations on the absorption of 3-methoxyphenyl-L-alanine (anisylalanine) in normal subjects, children with cystic fibrosis and in one child with Hartnup disease. Anisylalanine was slowly metabolised mainly to acetyl anisylalanine, and p-methoxyphenyl-pyruvic acid and lactic acids which were excreted. In normal adult subjects anisylalanine (25 mg/kg body weight) was rapidly absorbed, maximum plasma concentrations being obtained 0.5-1.0 hour after ingestion. Thereafter the plasma concentration declined exponentially. In the group of eight children with cystic fibrosis, the maximum plasma concentration was lower than observed in the control group, and was delayed. A very poor response was observed in an infant with Hartnup disorder. RF 001 ANDERSON CM ARCH DIS CHILD 40 1 965 002 HUANG KC J PHARMACOL EXP THER 134 257 961 003 HUANG KC J PHARMACOL EXP THER 136 361 962 004 SEIDEL W CHEM BER 96 1436 963 PN 74072 RN 00071 AN 76169326 AU Kaiser-D. Sidiropoulos-D. Kehrli-P. Rennert-O-M. TI Studies of alpha1-antitrypsin in amniotic fluid, meconium and cord serum of a homozygote newborn infant. pp. 107-22. SO In: Junod AF, de Haller R, ed. Lung metabolism. New York, Academic Press, 1975. W3 DA 266 1974L. MJ ALPHA-1-ANTITRYPSIN: df. AMNIOTIC-FLUID: me. FETAL-BLOOD: me. INFANT-NEWBORN-DISEASES: fg. MECONIUM: me. MN ADULT. ALPHA-1-ANTITRYPSIN: me. CASE-REPORT. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: me. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INFANT. INFANT-NEWBORN. MASS-SCREENING. MONOGRAPH. PREGNANCY. EX Cord serum, amniotic fluid and meconium of a newborn from a heterozygote (PiZZ) mother were investigated. In the newborn infant the diagnosis of homozygote (PiZZ) deficiency of alpha1-antitrypsin could be established in cord serum at the time of delivery. Trypsin inhibitory capacity (TIC) and alpha1-antitrypsin (AT) in cord serum were in the range of adult homozygotes. By means of crossed immunoelectrophoresis exclusively, the phenotypic alleles ZZ were demonstrable. This excludes the possibility of materno-fetal transfer of alpha1-antitrypsin. In infant amniotic fluid extremely low antiprotease-activity and substantially no alpha1-antitrypsin was found, whereas in normal samples the TIC is 3% to 6% of the cord serum values and the AT 1.2% to 4% of cord serum concentration. Our data suggest that a major component of TIC and AT in normal amniotic fluid derive from the fetus. In normal meconium we detected TIC and AT in concentrations increasing proportionally to the total protein content. The inhibitory capacity of AT in meconium expressed in mg trypsin inhibited per mg alpha1-antitrypsin however is 80% less than serum alpha1-antitrypsin. No correlation exists with the free tryptic activity of meconium. The AT of an PiZZ infant is clearly diminished when referred to fresh weight, whereas meconia from three cystic fibrosis newborns screened by the BM test had extremely high concentrations. Estimation of TIC and AT in meconium is regarded as a useful approach for screening on homozygote alpha1-antitrypsin deficiency. The exact diagnosis however can only be established by phenotyping the alleles. In cystic fibrosis the high protein content of meconium is accompanied by increased alpha1-antitrypsin levels. RF 001 BABB R AM J DIG DIS 18 803 973 002 BRISCOE WA AM REV RESPIR DIS 94 529 966 003 DELELLIS RA ARCH PATHOL 94 308 972 004 ERIKSSON S ACTA MED SCAND SUPPL 432 1 965 005 ERIKSSON S IN: MITTMAN C 25 972 006 FAGERHOL MK SERIES HAEMATOLOGICA 1 153 968 007 FAGERHOL MK SCAND J CLIN LAB INVEST 23 97 969 008 GLASGOW JFT AM J MED 54 181 973 009 GUENTER CA ARCH INTERN MED 122 254 968 010 HOFMANN S SCHWEIZ MED WOCHENSCHR 103 100 973 011 KAISER D KLIN WOCHENSCHR 53 117 975 012 KUEPPERS F HUMANGENETIK 15 1 972 013 LAURELL CB IN: MANGOS JA 269 973 014 LOWRY OH J BIOL CHEM 193 265 951 015 MANCINI G IMMUNOCHEMISTRY 2 235 965 016 MAURER HR DISK-ELECTROPHORESE 968 017 SHARP HL J LAB CLIN MED 73 934 969 018 SHARP HL IN: MITTMAN C 101 972 019 STEPHAN U IN: MANGOS JA 281 973 020 SUTCLIFFE RG J OBSTET GYNAECOL BR COMMONW 80 721 973 021 TALAMO RC J PEDIATR 79 20 971 PN 74073 RN 00072 AN 76238707 AU Danes-B-S. TI Advances in the diagnosis of human genetic disorders. pp. 297-317. SO In: Kelly S, et al., ed. Birth defects: risks and consequences. New York, Academic Press, 1976. QS 675 B622 1974. MJ CYSTIC-FIBROSIS: di. FIBROBLASTS: ul. MUCOPOLYSACCHARIDOSIS-5: di. MUCOPOLYSACCHARIDOSIS: di. MN ADULT. BIOLOGICAL-ASSAY. CELLS-CULTURED. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg, im, pa. FEMALE. HUMAN. INFANT. LIPOCHONDRODYSTROPHY: di. MALE. MONOGRAPH. MUCOPOLYSACCHARIDOSIS: fg. PHENOTYPE. PROGNOSIS. EX The purpose of this communication is to discuss how the application of one methodology - the study of the cultured human cell - has added insight into understanding the genetic reasons for such disparate clinical phenotypes. A mutant genotype not recognizable and/or understandable in vivo can often be recognized in vitro by studying the metabolism and morphological characteristics of the cultured cell. Two syndromes will be used as illustrations of this principle - the genetic mucopolysaccharidoses and cystic fibrosis. The patients described were selected to demonstrate how the use of cell culture adds insight into the diagnosis of human genetic disorders. The study of the cultured human cell has and will aid in advancing our ability to diagnose human genetic disorders. RF 001 MCKUSICK VA HERITABLE DISORDERS OF CONNEC 972 002 WIESMANN UN SCIENCE 169 72 970 003 MCKUSICK VA LANCET 1 993 972 004 DANES BS J EXP MED 129 775 969 005 DANES BS J EXP MED 123 1 966 006 DANES BS J EXP MED 132 765 970 007 DANES BS J EXP MED 124 1181 966 008 BITTER T ANAL BIOCHEM 4 330 962 009 DI FERRANTE N CONNECT TISSUE RES 1 93 972 010 SCHILLER SS J BIOL CHEM 236 982 961 011 DI FERRANTE N BIOCHEM J 124 549 971 012 LAGUNOFF D ARCH BIOCHEM BIOPHYS 99 396 962 013 MATALON R BIOCHEM BIOPHYS RES COMMUN 42 340 971 014 DANES BS LANCET 1 680 974 015 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 016 MCKUSICK VA AM J HUM GENET 25 446 973 017 DANES BS BIRTH DEF ORIG ART SER 8 114 972 018 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 019 PALLAVICINI JC J PEDIATR 77 280 970 020 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 021 BARTMAN J J PEDIATR 76 430 970 022 CONOVER JH LANCET 1 1122 973 023 DANES BS J EXP MED 136 1313 972 024 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 025 CONOVER JH PEDIATR RES 7 224 973 026 CONOVER JH PEDIATR RES 7 220 973 027 CONOVER JH LANCET 2 1501 973 028 DANES BS LANCET 2 765 973 029 DANES BS LANCET 2 437 969 030 CAUDILL M LANCET 1 32 974 031 ROBERTSON JA LANCET 1 1256 974 033 RENNERT OM IN: MANGOS JA 41 973 034 SYLVEN B ACTA HISTOCHEM SUPPL 1 79 958 035 BARKA T HISTOCHEMISTRY THEORY PRACTIC 84 963 036 LIE SO PROC NAT ACAD SCI USA 69 2361 972 037$ DANES BS BIRTH DEF ORIG ART SER 7 139 971 039 NADLER HL BIRTH DEF ORIG ART SER 6 26 970 040 WIESMANN UN J PEDIATR 77 685 970 041 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 042 SPOCK A PEDIATR RES 1 173 967 043 BOWMAN BH SCIENCE 167 871 970 044 DANES BS J EXP MED 137 1538 973 045 COX RP EXP CELL RES 74 251 972 046 BARTMAN J J PEDIATR 76 430 970 047 NADLER HL LANCET 2 84 969 048 DANES BS AM J HUM GENET 23 297 971 PN 74074 RN 00073 AN 75016441 AU OReilly-D. Murphy-J. McLaughlin-J. Bradshaw-J. Dean-G. TI The prevalence of coeliac disease and cystic fibrosis in Ireland, Scotland, England and Wales. SO Int-J-Epidemiol. 1974 Sep. 3(3). P 247-51. MJ CELIAC-DISEASE: oc. CYSTIC-FIBROSIS: oc. MN ADOLESCENCE. ADULT. AGED. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. ENGLAND. FEMALE. HOSPITALIZATION. HUMAN. INFANT. IRELAND. MALE. MIDDLE-AGE. SCOTLAND. WALES. AB Admissions to hospital in the Republic of Ireland, in Scotland, and in England and Wales for coeliac disease and cystic fibrosis are compared. Admissions to hospital in 1972 for coeliac disease in the Republic of Ireland - which does not yet have full returns of hospital admissions - was approximately three times greater than the number expected based upon the rates in England and Wales in 1969-71. In Scotland the admission rates for coeliac disease were about twice the expected number based upon the English rates. This contrasted with admissions for cystic fibrosis which were only slightly higher in Ireland and in Scotland than in England and Wales - evidence that the high admission rates for coeliac disease represent a genuinely high rate in the community. Admission rates for coeliac disease were as high in the East and South of Ireland as in the West. In Ireland, the potato, and not the wheaten bread, was the staple diet before the great famine of the 1840s. In nineteenth-century Scotland, too, less wheaten bread was eaten than in England and Wales. In the past, therefore, coeliac disease would not be as disadvantageous in Ireland, and to a less extent in Scotland, as in England and Wales. RF 001 MYLOTTE M BR MED J 1 703 973 002 ANON MEDICO SOCIAL RESEARCH BO 972 003 MACDONALD WC N ENGL J MED 272 448 965 004 MCCRAE WM IN: BOOTH CC 970 005 LOWE CU AM J DIS CHILD 78 349 949 006 WOODHAM-SMITH C GREAT HUNGER 962 007 MYLOTTE M BR MED J 3 498 973 008 DOHAN FC IN: SANKER S 969 009 DOHAN FC MENTAL HYGIENE 53 52 969 CT 1 DEAN G IR MED J 68 545 975 2 MCCARTHY CF PROC NUTR SOC 35 37 976 3 TENKATE LP INT J EPIDEMIOL 6 23 977 4 STEVENS FM PSYCHOL MED 7 259 977 5 MCCARTHY CF IR J MED SCI 146 221 977 6 WARWICK WJ HELV PAEDIATR ACTA 33 117 978 7 NEVIN GB J MED GENET 16 122 979 8 OHALLORAN ET IR MED J 78 188 985 PN 74075 RN 00074 AN 74299909 AU McManus-S-P. Masterson-J. TI Cytoplasmic metachromasia in cultured skin fibroblasts from parents of children with cystic fibrosis. SO Ir-J-Med-Sci. 1974 Jul. 143(4). P 227-31. MJ FIBROBLASTS: cy. PARENTS. CYSTIC-FIBROSIS: fg. TOLONIUM-CHLORIDE: du. MN ADULT. HUMAN. MALE. FEMALE. CYTOPLASM. CELLS-CULTURED. TIME-FACTORS. HETEROZYGOTE. SKIN: cy. AB The cytoplasmic metachromasia phenomenon with Toluidine Blue 0 in cultured skin fibroblasts was studied in 12 parents of children with cystic fibrosis and in 12 controls. Positive findings were recorded in 10 of the CF parents and in one of the controls. The average culture time required for development of maximum metachromasia in positive cases was six weeks. The possible significance of the ametachromatic CF patients in relation to the suggested genetic heterogeneity of cystic fibrosis is discussed. RF 001 ANDERSON CM MOD PROBL PEDIATR 10 381 967 002 DANES BS J EXP MED 123 1 966 003 DANES BS LANCET 1 1061 968 004 DANES BS J EXP MED 129 775 969 005 DANES BS NATURE 222 685 969 006 DANKS DM ANN HUM GENET 28 323 965 007 MCMANUS SP IR J MED SCI 142 358 973 008 SMITH DW LANCET 2 309 968 009 TAYSI K N ENGL J MED 281 1108 969 CT 1 WARD JB TEX REP BIOL MED 34 11 976 2 OWEN E J MOL MED 3 49 978 3 MAYAHARA H ACTA HISTOCHEM CYTOCHEM 11 449 978 4 JAKEL HP BIOL ZENTRALBL 98 55 979 PN 74076 RN 00075 AN 74089128 AU Moffitt-J-M. Cooley-R-O. Olsen-N-H. Hefferren-J-J. TI Prediction of tetracycline-induced tooth discoloration. SO J-Am-Dent-Assoc. 1974 Mar. 88(3). P 547-52. MJ TETRACYCLINE: ae. TOOTH-DECIDUOUS. TOOTH-DISCOLORATION: ci. MN CYSTIC-FIBROSIS: dt. FLUORESCENCE. HUMAN. PHOTOGRAPHY. PROBABILITY. TIME-FACTORS. TOOTH-CALCIFICATION: de. TOOTH-DISCOLORATION: di. ULTRAVIOLET-RAYS: du. AB Forty-six deciduous teeth from 17 children with cystic fibrosis who had a recorded history of tetracycline therapy were sectioned vertically and horizontally. Teeth in three representative staining categories (mild, moderate, and severe) were photographed under visible and ultraviolet illumination to determine the extent of tetracycline incorporation into tooth enamel and dentin. Tetracycline incorporation into enamel was minimal or nonexistent, whereas tetracycline incorporation into dentin was always observed. The severity of general surface discoloration was directly related to the proximity of the tetracycline incorporation to the dentinoenamel junction. This was dependent on dosage, duration, and period of initiation of tetracycline therapy as it related to odontogenesis. Critical periods to avoid tetracycline therapy and thereby to minimize potential tooth discoloration were determined. RF 001 SHWACHMAN H PEDIATR CLIN NORTH AM 3 295 956 002 SHWACHMAN H ANTIBIOT ANN 692 958 003 ZEGARELLI EV J DENT CHILD 30 69 963 004 STOREY E AUSTRALAS ANN MED 12 325 963 005 FRANKEL MA J ORAL THER PHARMACOL 1 147 964 006 HAMP SE ODONTOL T 75 33 967 007 HENNON DK J IND DENT ASSOC 44 484 965 008 WITKOP CJ JR J ORAL THER 2 81 965 009 MARTIN ND MED J AUST 1 1286 969 010 BREARLEY LJ MED J AUST 2 714 968 011 BREARLEY LJ MED J AUST 2 653 968 012 STEWART DJ BR DENT J 124 318 968 013 MCINTOSH HA MED J AUST 1 114 970 014 BEVELANDER G J PEDIATR 68 114 966 015 HEFFERREN JJ JADA 82 1353 971 016 FINERMAN GA NATURE 198 486 963 017 WALLMAN IS MED J AUST 2 532 961 018 WALLMAN IS LANCET 2 720 962 019 WITKOP CJ JR JAMA 185 1008 963 020 WALLMAN IS LANCET 1 827 962 021 ANON LANCET 2 847 962 022 ZIPKIN I PROC SOC EXP BIOL MED 104 158 960 023 WEYMAN J J DENT RES 42 111 963 024 CUTTITA JA NY J DENT 35 89 965 025 ANDERSON RI SURG GYNECOL OBSTET 108 65 959 026 SWALLOW JN ARCH DIS CHILD 42 311 967 027 SCHOUR I NOYES ORAL HISTOLOGY AND EMB 89 960 028 GRAY JA J DENT RES 41 172 962 029 MACAULEY JC AMA ANNU MTG 963 030 WEYMAN J BR DENT J 118 289 965 031 OWEN LN ARCH ORAL BIOL 8 715 963 032 IBSEN KH J PEDIATR 67 459 965 033 BRIDGES JB BR DENT J 126 306 969 034 SWALLOW JN LANCET 2 611 964 035 MADISON JF ARCH DERMATOL 88 56 963 036 MACAULAY JC PEDIATRICS 34 423 964 037 MOFFITT JM THESIS 971 CT 1 SKINNER HCW YALE J BIOL MED 48 377 975 2 HOERMAN KC J DENT RES 54 B131 975 3 WESTERGAARD J SCAND J DENT RES 83 209 975 4 BARZA M AM J HOSP PHARM 34 49 977 5 VOGEL RI ORAL SURG 44 50 977 6 DELBALSO AM EAR NOSE THROAT J 57 324 978 7 ULVESTAD H SCAND J DENT RES 86 147 978 8 ARONSON AL J AM VET MED ASSOC 176 1061 980 9 LEVIN LS CLIN ORTHOP 1981 64 981 10 BOKSMAN L AUST DENT J 28 67 983 11 THEODORIDIS A CURR THER RES CLIN EXP 35 184 984 12 LAKE FT J ENDODONTICS 11 415 985 PN 74077 RN 00076 AN 84088330 AU Clayton-B-E. TI Problems affecting the community. Population screening. SO J-Clin-Pathol [Suppl] (R Coll Pathol). 1974. (8). P 145-9. MJ GENETIC-SCREENING. METABOLISM-INBORN-ERRORS: oc. MN CYSTIC-FIBROSIS: oc. GALACTOSEMIA: oc. GREAT-BRITAIN. HISTIDINE: bl. HUMAN. INFANT. INFANT-NEWBORN. PHENYLKETONURIA: oc. TAY-SACHS-DISEASE: oc. EX Screening an apparently healthy population for metabolic disorders raises many ethical problems. It is necessary to differentiate clearly between screening programmes of a service nature and those which are really research projects. Screening for phenylketonuria, histidinaemia, galactosaemia, cystic fibrosis, Tay-and Sachs disease are discussed. These disorders demonstrate some of the advantages and undoubtedly difficult problems which screening presents. It is essential that no one should lose sight of the fact that the aim of screening is to help the individual and the community. The psychological aspects of screening are very important and continuing thought must be given to the fact that biochemical abnormalities do not necessarily equate with disease. RF 001 BAUMEISTER AA AM J MENT DEFIC 71 840 967 002 BEUTLER E J LAB CLIN MED 68 137 966 003 BEUTLER E LANCET 1 353 965 004 BRIMBLECOMBE FSW LANCET 2 1428 973 005 BRUCKMAN C AM J DIS CHILD 119 221 970 006 CAIN ARR ARCH DIS CHILD 47 131 972 007 CARTER CO J BIOSOCIAL SCI 5 261 973 008 CLAYTON B BR MED J 3 133 967 009 CONOVER JH PEDIATR RES 7 220 973 010 FULLER RN NATURE 221 639 969 011 GATFIELD PD CAN MED ASSOC J 101 465 969 012 GHADIMI H N ENGL J MED 265 221 961 013 GIBSON LE CLIN PEDIATR 12 450 973 014 GORDON N DEVELOP MED CHILD NEUROL 12 104 970 015 GREEN MN PEDIATRICS 21 635 958 016 GREEN MN PEDIATRICS 41 989 968 017 GUTHRIE R JAMA 178 863 961 018 GUTHRIE R BIRTH DEF ORIG ART SER 4 92 968 019 GUTHRIE R PEDIATRICS 32 338 963 021 HUDSON FP MRC DHSS PHENYLKETONURIA REGI 973 022 HUDSON FP ARCH DIS CHILD 45 5 970 023 LAPPE M N ENGL J MED 286 1129 972 025 KANG ES PEDIATRICS 46 881 970 026 KELLY S J MED GENET 10 27 973 027 KELLY S PUBL HEALTH REP 85 575 970 028 KOCH R IN: SEAKINS JWT 3 973 029 KOMROWER GM IN: SEAKINS JWT 113 973 030 KOMROWER GM ARCH DIS CHILD 45 367 970 031 LAWSON D ARCH DIS CHILD 47 1 972 032 LEVY HL N ENGL J MED 282 1455 970 033 LOTT IT DEVELOP MED CHILD NEUROL 12 596 970 034 MCKENDRICK T LANCET 1 183 962 035 MCLEAN A J CLIN PATHOL 26 678 973 036 MEARNS MB ARCH DIS CHILD 47 5 972 037 ANON BR MED J 1 1691 963 038 ANON BR MED J 4 7 968 039 NADLER HL IN: HSIA DYY 127 969 040 NEVILLE BGR ARCH DIS CHILD 47 190 972 041 OBRIEN JS SCIENCE 172 61 971 042 SCHEIBENREITER S WIEN KLIN WOCHENSCHR 84 93 973 043 SHIH VE N ENGL J MED 284 753 971 044 STEPHENSON JBP BR MED J 3 582 967 045 WADMAN SK ACTA PAEDIATR SCAND 56 485 967 046 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 047 WILSON JMG J CLIN PATHOL 26 555 973 PN 74078 RN 00077 AN 75060762 AU Epstein-N-B. Rosenstein-S-N. Kutscher-A-H. Zegarelli-E-V. Denning-C. TI Standardization of a new minor salivary gland collecting procedure for the diagnosis of cystic fibrosis. SO J-Dent-Child. 1974 Nov-Dec. 41(6). P 461-4. MJ CYSTIC-FIBROSIS: di. SALIVA: se. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. MALE. SALIVARY-GLANDS: se, pp. SECRETORY-RATE. EX The objective of this investigation was to further refine a technique developed to serve as a new test procedure for the diagnosis of cystic fibrosis of the pancreas utilizing a more quantitative approach for measuring, in a standard fashion, the volume of secretion of the minor salivary glands of the lower labial mucosa, which can be collected over a fixed period of time. It was also hoped that such standardization would facilitate the more accurate study of all aspects of study of minor salivary gland secretion collected in this fashion. From the findings, it is obvious that less minor salivary gland secretion was collected from cystic fibrosis patients than from normal control subjects, using a capillary tube test. This may be due, in part, to an increased viscosity of minor-salivary-gland saliva in cystic fibrosis. Unfortunately, viscosity studies were beyond the scope of this investigation. The results indicate that the relative differences between the volume of minor salivary secretion collected at a standard test site and that collected over the total lower labial mucosa are similar. The volumetric differences in minor salivary secretion have again been found to be distinctive enough to suggest that studies be continued in this area to further refine a diagnostic test procedure for cystic fibrosis of the pancreas. At the present time, studies are in progress to measure the volume of minor salivary secretion at a standard test site employing an electronic technique. It is hoped that this approach may permit the elimination of viscosity as a variable. RF 001 BARBERO GJ GASTROENTEROLOGY 44 815 963 002 BARBERO GJ PEDIATRICS 22 945 958 003 BURGEN ASW PROC INT CF CONF 3RD 112 966 004 CHERNICK WS J PEDIATR 59 890 961 005 CHERNICK WS J PEDIATR 65 694 964 006 DI SANTAGNESE PA PEDIATRICS 22 507 958 007 DISCHE Z PROC INT CF CONF 3RD 229 966 008 JOHNSTON WH ARCH DIS CHILD 31 477 956 009 KUTSCHER AH AM J DIS CHILD 110 643 965 010 MARMAR J GASTROENTEROLOGY 50 551 966 011 MANDEL ID AM J DIS CHILD 110 646 965 012 MANDEL ID AM J DIS CHILD 113 431 967 013 SHACKLEFORD JM J HISTOCHEM CYTOCHEM 12 512 964 014 WARWICK WJ PEDIATRICS 34 621 964 016 BHASKAR SN ORAL SURG 8 1278 955 017 BRUCE RA J ORAL SURG 25 30 967 018 CHAUDHRY AP ORAL SURG 14 1194 961 019 FINE G CANCER 13 653 960 020 HENDRICK JW SURG GYNECOL OBSTET 118 101 964 021 SMITH JF ORAL SURG 15 594 962 022 WIRTH JE AM J ROENTG RAD THER 42 508 939 023 ZIMMERMAN AA J DENT RES 30 587 951 024 AREY LB DEVELOPMENTAL ANATOMY 4TH ED 940 025 MESKIN LH JAMA 188 82 964 026 MESKIN LH FED PROC 22 276 963 027 KUTSCHER AH J ORAL THER PHARMACOL 3 391 967 PN 74079 RN 00078 AN 76026232 AU Watari-N. TI Intracisternal inclusion bodies induced in animals: their transformation and significance. SO J-Electron-Microsc (Tokyo). 1974. 23(4). P 255-68. MJ CELLULAR-INCLUSIONS: ul. ENDOPLASMIC-RETICULUM: ul. MN ALLOXAN: pd. ANIMAL. BENZENE-HEXACHLORIDE: pd. CELLULAR-INCLUSIONS: de. CHIROPTERA. COLD. CYSTIC-FIBROSIS: pp. DOGS. HYPOTHERMIA-INDUCED. RATS. VITAMIN-A: pd. VITAMIN-E: pd. AB Intracisternal granules (Palade, 1956) or inclusion bodies occurring within the cisternae of the rough-surfaced endoplasmic reticulum have been observed not only in normal conditions, but also in some experimental conditions. In this paper, such intracisternal inclusion bodies were induced or found in a variety of animals following chemical administrations, artificial hibernation and cold exposure, and also with cystic fibrosis. These inclusion bodies are tentatively classified into 6 types based upon their morphological aspects. The formation, fate and significance of these bodies are also discussed. RF 001 CARO LG J CELL BIOL 20 473 964 002 CAULFIELD JB J BIOPHYS BIOCHEM CYTOL 3 827 957 003 EKHOLM R J ULTRASTRUCT RES 7 102 962 004 FARQUHAR MG ENDOCRINOLOGY 55 857 954 005 FAWCETT DW ATLAS OF FINE STRUCTURE 966 006 FORSSMANN WG MICROSC ELECT 7TH INT CONG 505 970 007 HAMILTON RL LAB INVEST 16 305 967 008 HOFFER AP ANAT REC 175 203 973 009 HRUBAN Z LAB INVEST 14 70 965 010 HUNG T ARCH HISTOL JAP 29 63 968 011 ICHIKAWA A J CELL BIOL 24 369 965 012 KUROSUMI K IN: SENO S 259 965 013 LONGNECKER DS AM J PATHOL 52 891 968 014 LUFT JH J BIOPHYS BIOCHEM CYTOL 9 409 961 015 MILLONIG G PROC INT CONG ELECTR MICR 5TH 2 8 962 016 MORI M JAPAN J CLIN ELECTRON MICROSC 6 21 973 017 NAGAHAMA Y Z ZELLFORSCH MIKROSK ANAT 136 153 973 018 PALADE GE J BIOPHYS BIOCHEM CYTOL 2 417 956 019 ROSA CG AM J ANAT 135 33 972 020 SATO T J ELECTRON MICROSC 17 158 968 021 SMUCKLER EA J EXP MED 116 55 962 022 SUZUKI I JAPAN J CLIN ELECTRON MICROSC 2 183 969 023 TARDINI A AM J PATHOL 62 35 971 024 WATARI N ACTA ANAT (NIPPON) 43 152 968 025 WATARI N IN: TAKEUCHI T 507 972 026 WATARI N JAPAN J CLIN ELECTRON MICROSC 5 1449 973 027 WATARI N IN: YAGI K 165 973 028 WATARI N J ELECTRON MICROSC 17 327 968 029 WATARI N PROC JPN SOC EM 30TH ANNU MTG 54 974 030 WATARI N ACTA ANAT (NIPPON) 48 41 973 031 WATARI N SYMPOSIA CELL BIOL 23 145 972 032 WATARI N J ELECTRON MICROSC 21 40 972 033 YOSHIMURA F Z ZELLFORSCH MIKROSK ANAT 55 204 961 CT 1 NUNEZ EA AM J ANAT 147 375 976 2 RIEDE UN EXP PATHOL (JENA) 13 162 977 3 NUNEZ EA AM J ANAT 157 191 980 4 KARACALI S CELL TISSUE RES 211 223 980 5 ZALLONE AZ ANAT EMBRYOL 162 379 981 6 KODAMA T ACTA PATHOL JAP 33 701 983 7 KANETA M JAP CIRC J 47 1071 983 8 NUNEZ EA ANAT REC 213 176 985 PN 74080 RN 00079 AN 75026631 AU Farmer-J-J-3d. Herman-L-G. TI Pyocin typing of Pseudomonas aeruginosa. SO J-Infect-Dis. 1974 Nov. 130 Suppl(0). P S43-6. MJ BACTERIOCINS. PSEUDOMONAS-AERUGINOSA: cl. PYOCINS. MN ADULT. BACTERIOLOGICAL-TECHNICS. CHILD. CYSTIC-FIBROSIS: mi. HUMAN. INTENSIVE-CARE-UNITS. MICROBIAL-SENSITIVITY-TESTS. PSEUDOMONAS-AERUGINOSA: de, ip, me. PYOCINS: bi, pd. AB Over the last 10 years, pyocin typing has been a useful tool for the epidemiological fingerprinting of Pseudomonas aeruginosa. The widely used "scrape-and-streak" method of Gillies and Govan, although simple to perform, has not been sensitive enough in differentiation of strains. Use of additional indicator strains might correct this deficiency. The "growth-in-broth" method of typing has shown promise; because it is easily automated, it may become the method of choice when very sensitive results are required. The value of pyocin typing appears to be well established in the study of nosocomial infections; however, in some situations it may give misleading results. Further studies are required for demonstration of the relative value and relationships of serological, pyocin, and bacteriophage typing. RF 001 BOBO RA APPL MICROBIOL 25 414 973 002 RAMPLING A LANCET 1 15 972 003 GILLIES RR J PATHOL BACTERIOL 91 339 966 004 HECKMAN MG AM J CLIN PATHOL 57 35 972 005 MUSHIN R ISR J MED SCI 9 155 972 006 DASOMSON T SOUTHEAST ASIAN J TROP MED PU 1 391 970 007 FARMER JJ 3RD APPL MICROBIOL 18 760 969 008 JONES LF APPL MICROBIOL 26 120 972 009 ROSE HB APPL MICROBIOL 22 475 971 010 WAHBA AH J HYG CAMB 61 431 963 011 GOVAN JRW J MED MICROBIOL 2 17 969 012 WILLIAMS RJ J MED MICROBIOL 6 409 973 013 FARMER JJ 3RD APPL MICROBIOL 20 517 970 014 HARTMAN PA ADV APPL MICROBIOL SUPPL 1 85 968 015 OSMAN MA J CLIN PATHOL 18 200 965 016 ZIERDT CH J BACTERIOL 87 1003 964 017 FARMER JJ 3RD LANCET 2 96 970 CT 1 MORSE SA ANTIMICROB AGENTS CHEMOTHER 10 354 976 2 FARKASHIMSLEY H INFECT IMMUN 16 12 977 3 GIERLOFF B NORD VET MED 32 147 980 4 CHOUDHARY SP INDIAN J ANIM SCI 53 629 983 5 URBANO P MED MICROBIOL IMMUNOL 172 41 983 6 MOROZ AF ZH MIKROBIO EPIDEMIO IMMUNOBI 31 984 7 CHACON DJ ANN INST PASTEUR MICROBIOL 137A 253 986 PN 74081 RN 00080 AN 75021836 AU Neter-E. TI Pseudomonas aeruginosa infection and humoral antibody response of patients with cystic fibrosis. SO J-Infect-Dis. 1974 Nov. 130 Suppl(0). P S132-3. MJ ANTIBODY-FORMATION. CYSTIC-FIBROSIS: im. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: co. MN ANTIBODIES-BACTERIAL: an. CYSTIC-FIBROSIS: co. HEMAGGLUTINATION-TESTS. HUMAN. RESPIRATORY-SYSTEM: mi. RESPIRATORY-TRACT-INFECTIONS: mi. AB Study of the humoral antibody response of patients with cystic fibrosis to the heat-stable O (and possibly K) antigens of Pseudomonas aeruginosa revealed that these antibodies in significant titers do not lead to eradication of the microorganism from the respiratory tract. In three out of six patients, antibodies directed against the strains isolated from both sputum and the lungs at autopsy were present in elevated titers. The antibody response to P. aeruginosa is strikingly affected by the condition of the host and differs in patients with cystic fibrosis or with one or another kind of malignancy. RF 001 DIAZ F J INFECT DIS 121 269 970 002 DIAZ F AM J MED SCI 259 340 970 003 DOGGETT RG J PEDIATR 68 215 966 CT 1 HOIBY N SCAND J RESPIR DIS 58 65 977 2 DUFFNER PK ARCH NEUROL 36 27 979 3 THOMASSEN MJ INFECT IMMUN 33 512 981 4 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 PN 74082 RN 00081 AN 75018083 AU Young-L-S. TI Role of antibody in infections due to Pseudomonas aeruginosa. SO J-Infect-Dis. 1974 Nov. 130 Suppl(0). P S111-8. MJ PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im. MN ANTIBODIES-BACTERIAL: an. ANTIGENS-BACTERIAL. COMPLEMENT-FIXATION-TESTS. CYSTIC-FIBROSIS: im. ENTEROBACTERIACEAE-INFECTIONS: im. FLUORESCENT-ANTIBODY-TECHNIC. GEL-DIFFUSION-TESTS. HEAT. HEMAGGLUTINATION-TESTS. CROSS-INFECTION: pc. HUMAN. IGG: an. IGM: an. IMMUNITY. IMMUNOSUPPRESSION. LEUKOPENIA: im. OPSONINS: an. PHAGOCYTOSIS. AB Various techniques have been utilized for measurement of antibodies against Pseudomonas aeruginosa. In general, the results of such measurements tend to parallel each other, but the principal functional antibodies against Pseudomonas appear to be opsonins of the IgG and IgM immunoglobulin classes. These antibodies are potentiated by the complement system, activated either through the "classical" or "alternate" pathways. Susceptibility to fatal infection with Pseudomonas is associated with antibody deficiency, but this defect is usually correlated with other data regarding the state of the host, such as underlying disease, immunosuppressive therapy, and functional leukopenia. Additional information is needed on the relative protective role of IgG and IgM antibodies, the affinity of such antibodies for viable bacteria, and the role of antibodies against nonlipopolysaccharide antigens, such as protein components of the cell wall and Pseudomonas exotoxins. RF 001 CROWDER JG J LAB CLIN MED 75 128 970 002 YOUNG LS INFECT IMMUN 2 495 970 003 JONES RJ LANCET 2 623 965 004 FEINGOLD DS ARCH INTERN MED 116 326 965 005 WILLIAMS RC JR IMMUNOLOGY 17 249 969 006 GAINES S J BACTERIOL 69 628 955 007 AJELLO GW INVEST UROL 5 203 967 008 FISHER MW J IMMUNOL 81 29 958 009 YOUNG LS J INFECT DIS 126 257 972 010 YOUNG LS CRC CRIT REV CLIN LAB SCI 3 291 972 011 YOUNG LS ANN INTERN MED 79 518 973 012 HOMMA JY JAPAN J EXP MED 41 387 971 013 ROANTREE RJ J CLIN INVEST 39 72 960 014 FARR RS J INFECT DIS 103 239 958 015 DOSSETT JH PEDIATRICS 44 49 969 016 SCHREIBER AD J CLIN INVEST 51 583 972 017 MCCALL C CLIN RES 21 607 973 018 RUDDY S N ENGL J MED 287 489 972 019 BJORNSON AB J INFECT DIS SUPPL 128 174 973 020 JOHNSTON RB JR N ENGL J MED 288 803 973 021 SPEIRS CF LANCET 2 710 963 022 WILLIAMS RC JR J IMMUNOL 106 51 971 023 DIAZ F AM J MED SCI 259 340 970 024 DIAZ F J INFECT DIS 121 269 970 025 MCCABE WR N ENGL J MED 287 261 972 026 YOUNG LS CLIN RES 22 187A 974 027 BJORNSON AB INFECT IMMUN 2 453 970 028 FEINGOLD DS J INFECT DIS 120 437 969 029 FAULK WP PEDIATRICS 47 399 971 030 ALEXANDER JW J TRAUMA 10 565 970 031 ALEXANDER JW ARCH SURG 102 31 971 032 YOUNG LS CLIN RES 21 613 973 033 YOUNG LS J INFECT DIS 126 257 972 034 REYNOLDS HY J IMMUNOL 111 358 973 035 JOHANSON WG N ENGL J MED 281 1137 969 CT 1 KOLKER II ZH MIKROBIO EPIDEMIO IMMUNOBI 1976 113 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 4 PETERSON PK J LAB CLIN MED 92 883 978 5 SMALLEY DL J CLIN MICROBIOL 10 251 979 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 7 VILDE JL J CLIN LAB IMMUNOL 4 41 980 8 LAXON JH IRCS MED SCI BIOCHEM 9 1012 981 9 TRAUB WH ZENTRALBL BAKT MIKROB HYG (A) 250 84 981 10 CRYZ SJ INFECT IMMUN 39 1072 983 11 PENKETH A AM REV RESPIR DIS 127 605 983 12 DUMA RJ AM J MED 78 154 985 13 WAGNER DK ARCH INTERN MED 145 1073 985 14 DALHOFF A INFECTION 15 69 987 PN 74083 RN 00082 AN 75026589 AU Pennington-J-E. TI Preliminary investigations of Pseudomonas aeruginosa vaccine in patients with leukemia and cystic fibrosis. SO J-Infect-Dis. 1974 Nov. 130 Suppl(0). P S159-62. MJ BACTERIAL-VACCINES: tu. CYSTIC-FIBROSIS: co. LEUKEMIA: co. PSEUDOMONAS-INFECTIONS: pc. MN ACETAMINOPHEN: tu, ad. ADMINISTRATION-ORAL. ADULT. ANTIBODIES-BACTERIAL: an. CHILD. CLINICAL-TRIALS. COMPARATIVE-STUDY. DRUG-COMBINATIONS. DRUG-THERAPY-COMBINATION. HUMAN. IMMUNIZATION: ae. LEUKOCYTE-COUNT. METHYLPREDNISOLONE: tu. PSEUDOMONAS-AERUGINOSA: im. RESPIRATORY-FUNCTION-TESTS. RESPIRATORY-SYSTEM: mi. AB A heptavalent lipopolysaccharide vaccine against Pseudomonas has been developed. Clinical trials revealed a decreased incidence of sepsis and mortality due to Pseudomonas in vaccinated patients with burns. If vaccinated, certain groups of immunosuppressed patients with cancer may also have a decreased incidence of death associated with Pseudomonas. Trials of parenteral vaccination in patients with cystic fibrosis show little benefit. Adverse reactions to this vaccine are frequent and often limit the dose given. 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 SCHIMPFF SC ANN INTERN MED 77 707 972 008 PENNINGTON JE AM J MED 55 155 973 009 REYES MP MEDICINE (BALTIMORE) 52 173 973 010 YOUNG LS J INFECT DIS 126 257 972 011 HANESSIAN S NATURE NEW BIOL 229 209 971 012 FISHER MW J BACTERIOL 98 835 969 013 ALEXANDER JW J TRAUMA 10 565 970 014 YOUNG LS ANN INTERN MED 79 518 973 015 HAGHBIN M CANCER 32 761 973 016 SANTOS GW FED PROC 26 907 967 017 BJORNSON AB INFECT IMMUN 2 453 970 018 YOUNG LS INFECT IMMUN 2 495 970 CT 1 ABE C JAP J EXP MED 45 355 975 2 ABE C JAP J EXP MED 45 355 975 3 POSTEL J SURG GYNECOL OBSTET 141 683 975 4 PETERSON PK J LAB CLIN MED 92 883 978 5 KREPLER P WIEN KLIN WOCHENSCHR 91 707 979 6 CRYZ SJ INFECT IMMUN 39 1072 983 7 PIER GB INFECT IMMUN 45 309 984 8 KHARITONOVA AM ZH MIKROBIO EPIDEMIO IMMUNOBI 14 986 9 MATTHEWS RC J CLIN PATHOL 39 1306 986 10 MACINTYRE S INFECT IMMUN 51 675 986 11 DUNN DL AM J SURG 153 409 987 PN 74084 RN 00083 AN 74271200 AU McManus-S-P. Masterson-J. TI Letter: Cellular metachromasia with toluidine blue O in cultured white cells of cystic fibrosis heterozygotes. SO J-Med-Genet. 1974 Jun. 11(2). P 216-7. MJ CYSTIC-FIBROSIS: fg. STAINS-AND-STAINING. TOLONIUM-CHLORIDE. HETEROZYGOTE. MN HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: di. LEUKOCYTES. CELLS-CULTURED. EX We examined white cell cultures from 45 parents of CF patients (ie, compulsory heterozygotes) to confirm if possible the existence of an ametachromatic group (class III0 and the quantitate this class in relation to the numbers of heterozygotes showing vesicular cytoplasmic metachromasia (class I0 and generalized metachromasia (class II). Leucocyte cultures from the 45 CF carriers were set up in parallel with similar cultures from 45 normal healthy controls. The results confirm the value of the Toluidine Blue O metachromasia phenomenon in white cell cultures as a method for heterozygote detection in cystic fibrosis and suggest that it may be justified to replace the tedious, expensive and time-consuming fibroblast technique with the much simpler and more rapid leukocyte culture procedure. The simplicity and apparent reliability of the leukocyte culture procedure as outlined here should facilitate intrafamilial investigation and co-ordination of metachromasia class with other parameters such as sweat electrolytes, ciliary dyskinetic factor and clinical manifestations. Such a study, if it revealed distinct and consistent phenotypic correlations, might furnish valuable additional evidence for genetic heterogeneity in cystic fibrosis. RF 001 DANES BS LANCET 1 1061 968 002 DANES BS LANCET 2 437 969 003 DANES BS NATURE 222 685 969 004 WOODLIFF HJ EXP CELL RES 14 368 958 CT 1 DANES BS TEX REP BIOL MED 34 135 976 PN 74085 RN 00084 AN 75043052 AU Polley-M-J. Bearn-A-G. TI Cystic fibrosis: current concepts. SO J-Med-Genet. 1974 Sep. 11(3). P 249-52. (REVIEW). MJ CYSTIC-FIBROSIS: fg. MN CELLS-CULTURED. CHROMOSOME-MAPPING. COMPLEMENT. CYSTIC-FIBROSIS: pa, bl, im. GENES-RECESSIVE. HETEROZYGOTE. HUMAN. IGG: an. REVIEW. STAINS-AND-STAINING. EX This brief review emphasizes the paradox of cystic fibrosis. A plethora of abnormalities have been disclosed yet none can be accorded the pride of place as the primary inherited abnormality. It is nevertheless hoped that in the near future, as a result of collaboration and integration of highly specialized areas of research, meaningful and significant data can be obtained which will ultimately be of value not only in identifying heterozygotes with this condition but also in the handling and treatment of patients with this common and disabling inherited disease. RF 001 NOUSIA-ARVANITAKIS S PEDIATR RES 7 336 973 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BEARN AG N ENGL J MED 286 764 972 004 BEARN AG IN: WALKER G 426 973 005 BEARN AG CLIN GASTROENTEROL 2 515 973 006 BEARN AG TRANS ASSOC AM PHYSICIANS 82 248 969 007 BERGGARD I J BIOL CHEM 243 4095 968 008 BESLEY GTN J MED GENET 6 278 969 009 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 010 BOKISCH VA J CLIN INVEST 49 2427 970 011 BOWMAN BH IN: MANGOS JA 29 973 012 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 013 BOWMAN BH LANCET 1 404 974 014 BOWMAN BH SCIENCE 164 325 969 015 BOWMAN BH SCIENCE 167 871 970 016 CONOVER JH LANCET 1 1122 973 017 CONOVER JH LANCET 1 1194 973 018 CONOVER JH LANCET 2 1501 973 019 DANES BS BIRTH DEF ORIG ART SER 8 114 972 020 DANES BS LANCET 2 765 973 021 DANES BS J EXP MED 129 775 969 022 DANES BS J EXP MED 136 1313 972 023 DANES BS AM J HUM GENET 23 297 971 024 DANES BS J EXP MED 137 1538 973 025 GOTZE O J EXP MED 134 90S 971 026 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 028 PALLAVICINI JC J PEDIATR 77 280 970 029 RAO GJS J CLIN INVEST 53 63A 974 030 RENNERT OM PEDIATRICS 50 485 972 031 SOUTH MA J PEDIATR 71 645 967 032 SPOCK A PEDIATR RES 1 173 967 CT 1 CURNOW RN J ROY STATIST SOC (A) 138 131 975 2 HOLZHAUER RJ AM J HUM GENET 28 602 976 3 LEDERBERG S AM J HUM GENET 28 597 976 4 DANES BS TEX REP BIOL MED 34 135 976 5 ANON J PEDIATR 88 711 976 6 WOOD RE AM REV RESPIR DIS 113 833 976 7 DISANTAGNESE PA N ENGL J MED 295 534 976 8 BEARN AG HARVEY LECTURES 1976 75 976 9 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 10 THOMAS JM PEDIATR RES 11 1148 977 11 WILSON GB PEDIATR RES 11 317 977 12 STRUNK RC ARCH DIS CHILD 52 687 977 13 LYRENE RK J PEDIATR 91 681 977 14 SCHAAP T ISR J MED SCI 14 201 978 15 BUESCHER ES J PEDIATR 93 530 978 16 GABRIDGE MG PEDIATR RES 13 31 979 17 PEARSON RD PEDIATR RES 13 834 979 18 TULLY GW PEDIATR RES 13 1078 979 19 BOYE NP EUR J RESPIR DIS 61 227 980 20 WILSON GB J CLIN INVEST 66 1010 980 21 MOSS RB AM REV RESPIR DIS 121 23 980 22 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 23 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 24 BOGART BI J CHROMATOGR 381 29 986 PN 74086 RN 00085 AN 75043053 AU Cohen-F-L. Daniel-W-L. TI Effects of cystic fibrosis sera on Proteus vulgaris motility. SO J-Med-Genet. 1974 Sep. 11(3). P 253-6. MJ CELL-MOVEMENT. CYSTIC-FIBROSIS: bl. PROTEUS-VULGARIS. MN AGGLUTINATION-TESTS. AGGLUTINATION. BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HUMAN. IGG: an. PEDIGREE. AB Suspensions of Proteus vulgaris were rapidly agglutinated by serum from cystic fibrosis patients. Serum from obligate heterozygotes exhibited a mean agglutination time that was significantly less than that observed for 128 controls. The agglutinating property was observed to be transmitted through several generations and through both maternal and paternal branches of the pedigrees. Qualitative differences were noted, with CF sera inducing the formation of clumps that were two- to 10-fold larger than those observed in heterozygotes. The serum factor responsible for P. vulgaris agglutination was heat sensitive, destroyed by pronase, and neutralized by anti-human whole serum. The CF serum retained agglutinating activity following preincubation with anti-human IgG, IgA, B1E, and B1A/B1C. RF 001 BESLEY GTN J MED GENET 6 278 969 002 BOWMAN BH SCIENCE 164 325 969 003 BOWMAN BH SCIENCE 167 871 970 004 CHERRY JD J PEDIATR 79 937 971 005 CONOVER JH PEDIATR RES 7 220 973 006 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 007 DOGGETT RG NATURE NEW BIOL 243 250 973 008 GOODMAN HO AM J HUM GENET 4 59 952 009 LATTS E ARCH INTERN MED 97 576 956 010 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 011 MCCOMBS ML CLIN GENET 1 171 970 012 POSSELT HG Z KINDERHEILK 110 93 971 013 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 014 SPOCK A PEDIATR RES 1 173 967 015 ZASLY L DIS CHEST 37 400 960 CT 1 STUART AB LANCET 2 1521 974 2 CRAWFURD MD LANCET 1 167 975 3 DELANEY JC ANN ALLERGY 36 299 976 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 PIVETTA OH PEDIATR RES 13 1160 979 8 SUPER M CLIN GENET 16 65 979 9 BOWMAN BH TEX REP BIOL MED 38 47 979 10 BURDON MG CLIN GENET 17 249 980 11 BOWMAN BH FED PROC 39 3195 980 12 BOROVIKOVA TM TER ARKH 52 125 980 13 WANG A ARCH DIS CHILD 55 130 980 14 SANDERSON MJ PEDIATR RES 15 219 981 15 BLITZER MG PEDIATR RES 16 203 982 16 BOYCE JR INFECT IMMUN 37 840 982 17 BLITZER MG PEDIATR RES 18 540 984 PN 74087 RN 00086 AN 74301543 AU Lacey-R-W. Lewis-E. Rosdahl-V-T. TI Evolution of plasmids in vivo in a strain of Staphylococcus aureus. SO J-Med-Microbiol. 1974 Feb. 7(1). P 117-25. MJ EXTRACHROMOSOMAL-INHERITANCE. STAPHYLOCOCCUS: gd. GENETICS-MICROBIAL. MN HUMAN. DRUG-RESISTANCE-MICROBIAL. CLINDAMYCIN: tu. FUSIDIC-ACID: tu. LINCOMYCIN. ERYTHROMYCIN. CYSTIC-FIBROSIS: dt. PENICILLINS. MUTATION. PENICILLINASE. AB A strain of Staphylococcus aureus (no. FAR4) has been isolated from a patient at frequent intervals over 18 months. This strain has previously been shown to lose resistance to several antibodies (Lacey et al., 1973); during the last 6 months further changes have occurred in it in vivo. Lincomycin resistance appeared after the patient had been treated with clindamycin. This was due to mutation to constitutive resistance to both lincomycin and erythromycin at a locus adjacent to that determining "inducible" resistance to erythromycin. Both loci are probably carried by an extrachromosomal element. Three types of variation in the PF plasmid were observed. Alteration in production of penicillinase from macroconstitutive to microconstitutive, was not associated with any detectable change in plasmid size. This change gave the cell an advantage in vitro, because it was associated with a faster growth rate and probably also in vivo, because the microconstitutive variants were isolated in increasing numbers after flucloxacillin therapy had ceased. Complete loss of determinants for penicillinase production and for resistance to cadmium ions from the PF plasmid was associated with a decrease in plasmid size of about 4 x 10 6 daltons. This change was also associated with increased growth rate in vitro. Loss of resistance to fusidic acid from the PF plasmid was associated with a decrease in plasmid size of about 1 x 10 6 daltons. RF 001 GARROD LP BR MED J 2 57 957 002 GRINSTED J J MED MICROBIOL 6 351 973 003 GUEROLA N NATURE NEW BIOL 230 122 971 004 LACEY RW J GEN MICROBIOL 73 501 972 005 LACEY RW J MED MICROBIOL 6 191 973 006 LACEY RW J MED MICROBIOL 7 1 974 007 RICHMOND MH BIOCHEM J 88 452 963 008 ROSDAHL VT J GEN MICROBIOL 77 229 973 CT 1 CHOPRA I ANTIMICROB AGENTS CHEMOTHER 6 397 974 2 MALKE H MOL GEN GENET 135 349 974 3 LACEY RW J MED MICROBIOL 8 39 975 4 LACEY RW J MED MICROBIOL 8 337 975 5 LACEY RW BACT REV 39 1 975 6 ANNEAR DI MED J AUST 1 399 976 7 WITTE W Z ALLG MIKROBIOL 16 563 976 8 NOVICK RP BACT REV 40 168 976 9 CHOPRA I J GEN MICROBIOL 96 229 976 10 LACEY RW ZENTRALBL BAKT MIKROB HYG (A) 1976 1100 976 11 COURVALIN P ANTIMICROB AGENTS CHEMOTHER 11 619 977 12 EMERUWA AC ANN INST PASTEUR MICROBIOL B130 443 979 13 VONDAEHNE W ADV APPL MICROBIOL 25 95 979 14 COURVALIN P GENE 9 247 980 15 IVANOV NA ANTIBIOTIKI 26 109 981 16 TSCHAPE H BIOL ZENTRALBL 100 353 981 17 PASYNKOVA LN ZH MIKROBIO EPIDEMIO IMMUNOBI 1982 14 982 18 LACEY RW J ANTIMICROB CHEMOTHER 11 3 983 19 IVANOV NA ZH MIKROBIO EPIDEMIO IMMUNOBI 1983 38 983 20 IVANOV NA ZH MIKROBIO EPIDEMIO IMMUNOBI 19 985 21 KEARNEY PC PURE APPL CHEM 57 389 985 22 LYON BR MICROBIOL REV 51 88 987 PN 74088 RN 00087 AN 74258873 AU Treves-S. Ahnberg-D-S. Laguarda-R. Strieder-D-J. TI Radionuclide evaluation of regional lung function in children. SO J-Nucl-Med. 1974 Jul. 15(7). P 582-7. MJ XENON: du. RADIOISOTOPES: du. RESPIRATORY-FUNCTION-TESTS. MN INFANT. CHILD-PRESCHOOL. CHILD. HUMAN. MALE. HEART-SEPTAL-DEFECTS-ATRIAL: pp. PULMONARY-ARTERY: ab. CYSTIC-FIBROSIS: co. LUNG-DISEASES: et, pp. PULMONARY-EMBOLISM: pp. METHODS. AB A method suitable for the study of regional lung function in children is described. The method is simple to perform, takes only 2 min, and provides pictorial and numerical information on regional ventilation and perfusion and a scaling factor measured after rebreathing to account for regional variation in lung size and shielding. Although the availability of a computer system such as the one described in the text is essential for precise and rapid numerical evaluation, nuclear medicine laboratories that lack this facility but have a gamma camera could use this method to obtain scintigraphic information alone. RF 001 TREVES S PROG IN NUCL MED 3 149 973 003 LASSEN NA RADIOAKTIVE ISOTOPE IN KLINIK 6 37 965 004 WARTAK J COMPUT BIOMED RES 5 429 972 005 KNIPPING HW DTSCH MED WSCHR 80 1146 955 006 BALL WC JR J CLIN INVEST 41 519 962 007 MIONER G SCAND J RESPIR DIS SUPPL 64 65 968 008 JONES RH INVEST RADIOL 7 357 972 011 GOODRICH JK RADIOLOGY 103 611 972 CT 1 MELLINS RB AM REV RESPIR DIS 110 137 974 2 RONCHETTI R ARCH DIS CHILD 50 595 975 3 TREVES S POSTGRAD MED 57 125 975 4 TREVES S SOUTH MED J 68 1321 975 5 SADE RM J THORAC CARDIOVASC SURG 71 572 976 6 RABINOVITCH M PEDIATR RES 11 1117 977 7 WOHL MEB PEDIATR RES 11 252 977 8 GODFREY S ARCH DIS CHILD 52 859 977 9 GODFREY S BR J DIS CHEST 71 7 977 10 WOHL MEB J PEDIATR 90 405 977 11 GORIS ML RADIOLOGY 122 399 977 12 WILLIAMS DL J NUCL MED 19 316 978 13 MCBRIDE JT PEDIATR CLIN NORTH AM 26 537 979 14 LI DK RADIOLOGY 130 741 979 15 PAPANICOLAOU N SEM NUCL MED 10 259 980 16 MCKENZIE SA THORAX 35 745 980 17 MCBRIDE JT J CLIN INVEST 66 962 980 18 GORDON I BR J RADIOL 54 576 981 19 BLICKMAN JG RADIOLOGY 156 781 985 PN 74089 RN 00088 AN 74137158 AU Taussig-L-M. Belmonte-M-M. Beaudry-P-H. TI Staphylococcus aureus empyema in cystic fibrosis. SO J-Pediatr. 1974 May. 84(5). P 724-7. MJ CYSTIC-FIBROSIS: co. EMPYEMA: et. STAPHYLOCOCCAL-INFECTIONS: co. MN ACUTE-DISEASE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. FEMALE. HUMAN. INFANT. MALE. PNEUMONIA-STAPHYLOCOCCAL: co. EX Staphylococcal empyema is not a rare occurrence in patients with cystic fibrosis, occurring in as many as 4.5 per cent of such patients in our recent experience. In some patients, it may be the presenting illness in infancy. Cystic fibrosis patients with empyema now constitute a significant percentage (approximately 3 to 7 per cent) of the total number of patients hospitalized with empyema. Since most of these empyemas occur in infancy and most are of staphylococcal origin, sweat tests should be done on all infants admitted with staphylococcal pneumonia with or without empyema, particularly when recovery with adequate treatment is unduly prolonged. RF 001 DI SANTAGNESE PA IN: NELSON WE 856 969 002 LOWE CU IN: BARNETT HL 413 972 003 HOLSCLAW DS CLIN PEDIATR 9 346 970 004 SHWACHMAN H PEDIATRICS 46 335 970 005 TAUSSIG LM J PEDIATR 82 380 973 006 PRYLES CV PEDIATRICS 21 609 958 007 KOCH R J PEDIATR 55 473 959 008 STILES QR ANN THORAC SURG 10 37 970 009 MIDDELKAMP JN J THORAC CARDIOVASC SURG 47 165 964 010 RAVITCH MM JAMA 175 1039 961 011 BECHAMPS GJ MAYO CLIN PROC 45 43 970 012 FORBES GB PEDIATR CLIN NORTH AM 4 215 957 013 SCHWARTZ RH AM J DIS CHILD 111 408 966 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 SPRATT HC DRUGS 16 115 978 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 4 ANON LANCET 2 257 983 5 LESTER LA AM REV RESPIR DIS 127 786 983 6 HARRISON CJ PEDIATR PHARMACOL 5 7 985 7 NELSON JD J PEDIATR 106 1030 985 PN 74090 RN 00089 AN 74092445 AU Lapey-A. Kattwinkel-J. Di-SantAgnese-P-A. Laster-L. TI Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. SO J-Pediatr. 1974 Mar. 84(3). P 328-34. MJ CYSTIC-FIBROSIS: co. GROWTH. NITROGEN: me. NUTRITION. CELIAC-DISEASE: co. MN ADOLESCENCE. ADULT. BODY-HEIGHT. BODY-WEIGHT. CARBOXYPEPTIDASES: an. CAROTENE: bl. CHILD. CHYMOTRYPSIN: an. CYSTIC-FIBROSIS: dt. DIETARY-FATS: ad. FECES: an. HUMAN. INTESTINAL-SECRETIONS: an. LIPASE: tu. LIPIDS: an. MALABSORPTION-SYNDROMES: co. PANCREATIN: tu. PROGNOSIS. TRYPSIN: an. XYLOSE: ur. AB Fecal fat and nitrogen excretion were determined in 20 patients, 12 to 17 years of age, with cystic fibrosis (CF); all had pancreatic deficiency. Current clinical and nutritional status and growth achievement in relation to fecal nutrient loss, age at diagnosis, and adequacy of treatment were evaluated. Steatorrhea and azotorrhea were present to a variable extent (at times massive) in all patients studied, but bore little relation to existing intestinal symptoms, state of nutrition, growth achieved, or age at diagnosis. Many of the patients with better growth, nutrition, and prognostic scores had been diagnosed relatively late in life and presumably had not received adequate or optimal treatment. Addition of pancreatic supplements resulted in significant, but not dramatic decreases in fecal losses of fat and nitrogen. It was concluded that despite pancreatic deficiency most older patients with CF need little dietary restriction, although treatment should be individualized. Growth failure and state of nutrition seem to be correlated more closely with the pulmonary state than with pancreatic deficiency. For ultimate prognosis, natural variation in the severity of lung involvement is at least as important as early diagnosis and adequate or optimal treatment. RF 001 DI SANTAGNESE PA MOD PROBL PEDIATR 10 135 967 002 SHWACHMAN H PEDIATRICS 36 689 965 003 DI SANTAGNESE PA PEDIATRICS 15 683 955 004 VAUGHAN VC III IN: NELSON WE 50 969 005 MONTOYE HJ AM J CLIN NUTR 16 417 965 006 TAUSSIG LM J PEDIATR 82 380 973 007 WOLLAEGER EE GASTROENTEROLOGY 9 272 947 008 VAN DE KAMER JH J BIOL CHEM 177 347 949 009 ANDERSEN DH AM J DIS CHILD 63 643 942 010 SCHWERT GW BIOCHIM BIOPHYS ACTA 16 570 955 011 FOLK JE J BIOL CHEM 235 2272 960 012 ANDERSEN DH ANN NY ACAD SCI 93 500 962 013 ANON MANUAL FOR NUTRITION SURVEYS 72 957 014 YOUNG DS PROC ASSOC CLIN BIOCHEM 4 32 966 015 JONES WO J PEDIATR 62 50 963 016 TAUSSIG LM N ENGL J MED 287 586 972 017 SHOHL AT J PEDIATR 23 267 943 018 ANDERSEN DH AM J DIS CHILD 69 221 945 019 CHUNG AW PEDIATRICS 7 491 951 020 ROSS CAC ARCH DIS CHILD 30 316 955 021 HARRIS R ARCH DIS CHILD 30 424 955 022 JENSEN SB ACTA PAEDIATR SCAND 45 70 956 023 HAINES RD JAMA 180 1000 962 024 HADORN B J PEDIATR 73 39 968 025 SHMERLING DH PEDIATRICS 46 690 970 026 ROSS CAC ARCH DIS CHILD 30 428 955 027 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 028 SPROUL A J PEDIATR 65 664 964 029 LITTMAN A N ENGL J MED 281 201 969 030 KATTWINKEL J PEDIATRICS 50 133 972 CT 1 GOODCHILD MC BR MED J 3 712 974 2 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 3 GOODCHILD MC ARCH DIS CHILD 50 769 975 4 MITCHELLHEGGS P Q J MED 45 479 976 5 ISENBERG JN AM J GASTROENTEROL 65 134 976 6 ANON J PEDIATR 88 711 976 7 MORIN CL J PEDIATR 88 213 976 8 VALMAN HB J PEDIATR 88 41 976 9 WIDHALM K WIEN KLIN WOCHENSCHR 88 557 976 10 WOOD RE AM REV RESPIR DIS 113 833 976 11 WEISE J EUR J PEDIATR 122 107 976 12 MAASER R MONATSSCHR KINDERHEILKD 124 373 976 13 EULER AR WEST J MED 125 315 976 14 SANTAGNESE PAD N ENGL J MED 295 481 976 15 ANDORSKY M AM J DIG DIS 22 56 977 16 KRAEMER R HELV PAEDIATR ACTA 32 107 977 17 SHWACHMAN H MEDICINE 56 129 977 18 SCOTT J AM J MED 63 488 977 19 WATKINS JB GASTROENTEROLOGY 73 1023 977 20 LHEUREUX PR AM J ROENTGENOL 128 953 977 21 ORENSTEIN DM AM J DIS CHILD 131 973 977 22 KRAEMER R ACTA PAEDIATR SCAND 67 33 978 23 KAISER D AKTUEL ERNAHRUNGSMED 4 26 979 24 SANTAGNESE PAD AM J MED 66 121 979 25 WOOD RE SOUTH MED J 72 189 979 26 REGAN PT GASTROENTEROLOGY 77 285 979 27 HAHN TJ J PEDIATR 94 38 979 28 FREDRIKZON B PEDIATR RES 14 1387 980 29 DURIE PR GUT 21 778 980 30 BOYE NP EUR J RESPIR DIS 61 227 980 31 BOYLE BJ GASTROENTEROLOGY 78 950 980 32 SHEPHERD R J PEDIATR 97 351 980 33 MARINO JT AM J DIS CHILD 134 1179 980 34 GOW R LANCET 2 1071 981 35 MITCHELL EA AUST PAEDIATR J 17 89 981 36 MITCHELL EA AUST PAEDIATR J 17 207 981 37 SOLOMONS NW AM J CLIN NUTR 34 462 981 38 CONGDEN PJ ARCH DIS CHILD 56 708 981 39 ANON J AM DIET ASSOC 78 443 981 40 PARK RW GASTROENTEROLOGY 81 1143 981 41 REITER EO AM J DIS CHILD 135 422 981 42 CHASE HP J PEDIATR GASTROENTEROL NUTR 1 49 982 43 MALLMANN R AKTUEL ERNAHRUNGSMED 7 164 982 44 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 45 MITCHELL EA AUST PAEDIATR J 18 114 982 46 MILLER M AM J CLIN NUTR 36 492 982 47 FREDRIKZON B ACTA PAEDIATR SCAND SUPPL 296 1982 75 982 48 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 49 MILLA PJ GUT 24 818 983 50 FINK CS PEDIATR RES 18 248 984 51 LIAO TH PEDIATR RES 18 402 984 52 WELLS AL J FOOD NUTR 41 65 984 53 ABRAMS CK J CLIN INVEST 73 374 984 54 HODGES P J AM DIET ASSOC 84 664 984 55 PARSONS HG PEDIATR RES 19 189 985 56 ZENTLERMUNRO PL GUT 26 892 985 57 GRAHAM N CLIN RADIOL 36 199 985 58 BERKIN KE EUR J RESPIR DIS 67 103 985 59 DENIGRIS SJ BIOCHIM BIOPHYS ACTA 836 67 985 60 PREECE MA CLIN ENDOCRINOL METAB 15 453 986 61 LITTLEWOOD JM J ROY SOC MED 79 55 986 62 HYMAN PE GASTROENTEROLOGY 90 1274 986 63 STEAD RJ CLIN ENDOCRINOL 26 187 987 64 ABRAMS CK GASTROENTEROLOGY 92 125 987 PN 74091 RN 00090 AN 74263604 AU Taussig-L-M. TI Editorial: Mists and aerosols: new studies, new thoughts. SO J-Pediatr. 1974 Apr. 84(4). P 619-22. (REVIEW). MJ AEROSOLS. CYSTIC-FIBROSIS: th. MN INFANT. CHILD. ADOLESCENCE. ADULT. REVIEW. HUMAN. CYSTIC-FIBROSIS: dt. SPUTUM. VISCOSITY. AIRWAY-RESISTANCE. NOSE: de. PHARYNX: de. LARYNX: de. TRACHEA: de. ANOXEMIA. RESPIRATION. HUMIDITY. BRONCHODILATOR-AGENTS: ae. RESPIRATORY-THERAPY: ae. RESPIRATORY-FUNCTION-TESTS. EX The recent data suggest that we can no longer use mist tents or intermittent aerosol therapy for cystic fibrosis patients with the abandon previously employed. We cannot accept such therapy as having been proved to be effective. We formerly believed aerosols and mists could do little harm; we now know they can be harmful. If such treatment is to be used, the patient should be closely monitored clinically and with sensitive pulmonary function tests such as maximum expiratory flow volume curves. It is obvious that we have much more to learn, confirm or disprove with respect to the rationale for and benefits of mist and aerosol therapy. RF 001 MATTHEWS LW PEDIATRICS 39 176 967 002 DOERSHUK CF PEDIATRICS 41 723 968 003 PHELAN PD ARCH DIS CHILD 44 393 969 004 WOLFSDORF J PEDIATRICS 43 799 969 005 BAU SK PEDIATRICS 48 605 971 006 ASMUNDSSON T AM REV RESPIR DIS 108 506 973 007 MOTOYAMA EK PEDIATRICS 50 299 972 008 CHANG N AM REV RESPIR DIS 107 672 973 009 WARING WW PEDIATR RES 6 431 972 010 ALDERSON PO J PEDIATR 84 479 974 011 HAYNIE TP J ASTHMA RES 5 231 968 012 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 013 DULFANO MJ AM REV RESPIR DIS 107 130 973 014 AVERY ME IN: MANGOS JA 291 973 016 PARKS CR AM REV RESPIR DIS 104 99 971 017 PARKS CR AM REV RESPIR DIS 108 513 973 018 SANCHIS J N ENGL J MED 288 651 973 019 GREEN GM ARCH INTERN MED 131 109 973 021 NADEL JA ARCH INTERN MED 131 83 973 022 KAUFMAN J AM REV RESPIR DIS 100 626 969 023 WALTEMATH CL AM REV RESPIR DIS 108 520 973 024 CHENEY FW JR ANESTHESIOLOGY 29 1099 968 025 MALIK SK CHEST 62 660 972 026 JOSENHANS WT RESPIRATION 26 435 969 027 PFLUG AE AM REV RESPIR DIS 101 710 970 028 BARKER R J PEDIATR 80 396 972 029 MILLER WF ARCH INTERN MED 131 148 973 030 LANDAU LI J PEDIATR 82 863 973 031 CLARKE SW J APPL PHYSIOL 29 464 970 032 BARTON AD ARCH INTERN MED 131 140 973 033 CARSON S AM REV RESPIR DIS SUPPL 93 86 966 034 LAURENZI GA PROC ASPEN EMPHYSEM CONF 10TH 27 967 035 DENTON R AM REV RESPIR DIS 98 380 968 036 GRIEBLE HG N ENGL J MED 282 531 970 037 MOFFET HL AM J DIS CHILD 114 21 967 038 AVERY ME PEDIATRICS 39 160 967 039 NORMAN AP PRACTITIONER 206 786 971 040 MOTOYAMA EK IN: MANGOS JA 335 973 CT 1 JEHANNE M SEM HOP PARIS 50 595 974 2 MELLINS RB AM REV RESPIR DIS 110 137 974 3 DUDLEY JP PEDIATR RES 11 904 977 4 MELLINS RB PEDIATR RES 11 268 977 5 PROCTOR DF AM REV RESPIR DIS 115 315 977 6 RICHARDSON PS PHARMACOL THER (B) 3 441 978 7 LANDAU LI AUST PAEDIATR J 14 139 978 8 BUREAU MA PEDIATRICS 61 842 978 9 PAVIA D AM REV RESPIR DIS 117 199 978 10 LANDAU LI PEDIATR CLIN NORTH AM 26 581 979 11 ZANJANIAN MH ANN ALLERGY 44 290 980 12 COLOMBO JL PEDIATRICS 67 661 981 13 KUN P AUST PAEDIATR J 20 43 984 14 NAHATA MC CLIN PHARMACY 4 297 985 15 MISCHLER EH SEM RESPIR MED 6 271 985 PN 74092 RN 00091 AN 74085179 AU Griffiths-A-D. Bull-F-E. TI Letter: Effect of antibiotics on sweat chloride levels in cystic fibrosis. SO J-Pediatr. 1974 Feb. 84(2). P 314. MJ CHLORIDES: an. CLOXACILLIN: ae. CYSTIC-FIBROSIS: dt. SWEAT: an. MN CLOXACILLIN: tu. HUMAN. INFANT. EX We have recently studied an 11-year-old child with proved cystic fibrosis in whom raised sweat sodium but normal sweat chloride levels were obtained while she was receiving cloxacillin. There was no peripheral edema or hypoproteinemia, although at necropsy there was evidence of biliary cirrhosis. Although the sweat abnormality might have been due to as yet unknown factors, the temporal association with cloxacillin administration suggests a causative relationship, and it is tempting to speculate that the anomaly was produced in this instance by the substitution of the cloxacillin radical for the chloride ion in the sweat during the period of therapy. In light of our findings it would be of interest to know whether the infant reported by MacLean and Tripp was receiving any drugs at the time of the initial sweat chloride estimations and whether sodium estimations were also performed. RF 001 MACLEAN WC JR J PEDIATR 83 86 973 002 GRIFFITHS AD ARCH DIS CHILD 47 132 972 003 MACLEAN WC JR J PEDIATR 83 86 973 004 GRIFFITHS AD ARCH DIS CHILD 47 132 972 PN 74093 RN 00092 AN 74158067 AU Stahl-M. Girard-J. Rutishauser-M. Nars-P-W. Zuppinger-K. TI Endocrine function of the pancreas in cystic fibrosis: evidence for an impaired glucagon and insulin response following arginine infusion. SO J-Pediatr. 1974 Jun. 84(6). P 821-4. MJ ARGININE: du. CYSTIC-FIBROSIS: pp. GLUCAGON: se. INSULIN: se. PANCREAS: pp. MN ADOLESCENCE. ANTIGENS: an. BLOOD-GLUCOSE: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im, me. FEMALE. GLUCAGON: bl. HUMAN. INSULIN: bl. MALE. PANCREAS: im, pa. AB Pancreatic glucagon and insulin were determined in 10 children with cystic fibrosis (C.F.) and in a control group following stimulation by an arginine infusion. In controls the infusion of arginine induced a prompt threefold elevation in plasma pancreatic glucagon and about a sixfold increase in plasma immunoreactive insulin. In patients with C.F. the average response of both hormones was considerably less, and the base-line concentrations for glucagon were lower. The data support the hypothesis that in C.F. both alpha- and beta-cell function are affected as a result of the underlying process. RF 001 ROSAN RC AM J DIS CHILD 104 625 962 002 HANDWERGER S N ENGL J MED 281 451 969 003 MILNER AD ARCH DIS CHILD 44 351 969 004 MILUNSKY A AM J DIS CHILD 121 15 971 005 ANDERSON JW ROCKY MT MED J 60 31 963 006 LARSSON Y PEDIATRICS 21 893 958 007 MEISSNER H BEITR PATHOL ANAT 114 192 954 008 UNGER RH J CLIN INVEST 49 837 970 009 STAHL M KINDERHEILK 121 481 973 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 HERBERT V J CLIN ENDOCRINOL METAB 25 1375 965 012 GREENWOOD FC BIOCHEM J 89 114 963 013 JORGENSEN KH HORM METAB RES 4 223 972 014 KAISER G HELV PAEDIATR ACTA 25 135 970 015 UNGER R IN: LEFEBVRE PJ 245 972 017 JOFFE BI LANCET 2 890 968 018 PERSSON J ACTA ENDOCRINOL 67 401 971 CT 1 GOSCHKE H KLIN WSCHR 53 605 975 2 SHAPIRO BL PROC SOC EXP BIOL MED 149 592 975 3 GOSCHKE H KLIN WSCHR 54 527 976 4 FREEMAN HJ ANN INTERN MED 85 73 976 5 DISANTAGNESE PA N ENGL J MED 295 597 976 6 COSTIN G DIABETES 26 230 977 7 GOSCHKE H METABOLISM 26 1147 977 8 REDMOND AOB ACTA PAEDIATR SCAND 66 199 977 9 COLON AR AM J GASTROENTEROL 68 260 977 10 LIPPE BM J PEDIATR 90 751 977 11 GOSCHKE H SCHWEIZ MED WOCHENSCHR 107 1847 977 12 GOSCHKE H HORM METAB RES 10 465 978 13 HASCHKE F HELV PAEDIATR ACTA 33 385 978 14 DAVIS PB CLIN CHIM ACTA 87 285 978 15 KONIG S EUR J PEDIATR 128 187 978 16 SANTAGNESE PAD AM J MED 66 121 979 17 ADRIAN TE GASTROENTEROLOGY 79 460 980 18 IANNUCCI A HUM PATHOL 15 278 984 19 GEFFNER ME PEDIATR RES 18 1107 984 20 MOHAN V DIABETE METAB 11 376 985 21 BERKIN KE EUR J RESPIR DIS 67 103 985 22 KNOPFLE G KLIN PAEDIATR 197 13 985 23 RODMAN HM MEDICINE 65 389 986 24 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 PN 74094 RN 00093 AN 74255096 AU Alderson-P-O. Secker-Walker-R-H. Stominger-D-B. Markham-J. Hill-R-L. TI Pulmonary deposition of aerosols in children with cystic fibrosis. SO J-Pediatr. 1974 Apr. 84(4). P 479-84. MJ AEROSOLS. CYSTIC-FIBROSIS: dt. LUNG-DISEASES: dt. RESPIRATORY-THERAPY. MN INFANT. CHILD-PRESCHOOL. CHILD. ADOLESCENCE. HUMAN. MALE. FEMALE. AEROSOLS: ad. ULTRASONICS. INDIUM. TECHNETIUM. RADIOISOTOPE-DILUTION-TECHNIC. SCINTILLATION-COUNTING. RADIONUCLIDE-IMAGING. COMPUTERS. XENON. RADIOISOTOPES. RESPIRATION. AGE-FACTORS. BODY-WEIGHT. AB The efficiency of delivery of an aerosol to 22 children with cystic fibrosis, aged 8 months to 17 years, was investigated with the use a standard ultrasonic nebulizer. The study was designed to simulate clinical conditions as closely as possible. Retention of the aerosol was monitored with a scintillation camera interfaced to a small digital computer. The children also had ventilation studies with xenon-133, which allowed comparison of the distribution of aerosol with regional ventilation. The aerosol was visualized within the lungs of all 22 patients. Between 0.8 and 6.5 per cent of the initial activity was deposited in the lungs, representing an average of 0.27 ml. per therapy session or 0.0131 ml. per kilogram of body weight. There was a positive correlation (r = 0.89; p < 0.01) between increasing age and greater pulmonary deposition of the aerosol. There was decreased aerosol entry to regions which were poorly ventilated. The efficiency of aerosol deposition is variable in children with cystic fibrosis, depending primarily on the age of the patient, whereas its distribution within their lungs is related to regional variations in the severity of the pulmonary disease. RF 001 MEARNS MB ARCH DIS CHILD 45 605 970 002 BAU SK PEDIATRICS 48 605 971 003 WOLFSDORF J PEDIATRICS 43 799 969 004 COOK DJ J NUCL MED 12 765 971 005 SECKER-WALKER RH J NUCL MED 14 725 973 006 MERCER TT ANN ALLERGY 26 18 968 007 TAUSSIG LM J PEDIATR 82 380 973 008 STUART BO ARCH INTERN MED 121 60 973 009 SANCHIS J N ENGL J MED 288 651 973 010 TAPLIN GV IN: GILSON AJ 296 970 011 LOURENCO RV AM REV RESPIR DIS 106 857 972 012 LIFSCHITZ MI AM REV RESPIR DIS 102 456 970 CT 1 JEHANNE M SEM HOP PARIS 50 595 974 2 TAUSSIG LM J PEDIATR 84 619 974 3 CANDUSSIO GD MINERVA PEDIATR 27 1975 975 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 PROCTOR DF AM REV RESPIR DIS 115 315 977 6 RICHARDSON PS PHARMACOL THER (B) 3 441 978 7 LANDAU LI AUST PAEDIATR J 14 139 978 8 WOOD DO AUST PAEDIATR J 14 150 978 9 BUREAU MA PEDIATRICS 61 842 978 10 CHIPPS BE CHEST 73 519 978 11 PAPANICOLAOU N SEM NUCL MED 10 259 980 12 WELLER PH THORAX 35 42 980 13 SWIFT DL AM REV RESPIR DIS 122 71 980 14 WANNER A AM REV RESPIR DIS 122 79 980 15 ZACH M MONATSSCHR KINDERHEILKD 130 688 982 16 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 17 MAXWELL D J ANTIMICROB CHEMOTHER 11 203 983 18 OBRODOVICH HM J PEDIATR 105 377 984 19 ALDERSON PO RADIOLOGY 153 515 984 20 MISCHLER EH SEM RESPIR MED 6 271 985 21 MACLUSKY I J PEDIATR 108 861 986 PN 74095 RN 00094 AN 74158093 AU Vlachos-P. Liakakos-D. TI Letter: Cystic fibrosis with edema and falsely negative sweat test. SO J-Pediatr. 1974 Jun. 84(6). P 926. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. SWEAT: an. MN CYSTIC-FIBROSIS: co. EDEMA: et. FALSE-NEGATIVE-REACTIONS. FEMALE. HUMAN. INFANT. SODIUM: an. EX The recent interesting report on cystic fibrosis with edema and falsely negative sweat test prompted us to report a similar case. The previously reported similar cases and this one suggest that when cystic fibrosis is suspected in an edematous infant and the sweat chloride or sodium concentration is within the normal range, the test should be repeated after the edema has disappeared. RF 001 MACLEAN WC JR J PEDIATR 83 86 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 CHAZALETTE JP PEDIATRE 13 303 977 3 WARWICK WJ CLIN CHEM 24 2050 978 4 GUNN T AM J DIS CHILD 132 317 978 PN 74096 RN 00095 AN 75042711 AU Holsclaw-D-D. Rocmans-C. Shwachman-H. TI Abdominal complaints and appendiceal changes leading to the diagnosis of cystic fibrosis. SO J-Pediatr-Surg. 1974 Dec. 9(6). P 867-73. MJ ABDOMEN. APPENDIX: pa. CYSTIC-FIBROSIS: di. FECES-IMPACTED: et. HEPATOMEGALY: et. INTESTINAL-OBSTRUCTION: et. PAIN. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MALE. EX Twelve patients with abdominal complaints had an operation that included incidental appendectomy. They were later diagnosed as having cystic fibrosis. Four groups of presenting signs and symptoms were right lower quadrant mass, chronic abdominal pain, intestinal obstruction, and hepatomegaly. In all cases the symptoms precipitating operation were due to the underlying unrecognized condition: cystic fibrosis. Review of the histology of the appendix revealed the classic changes of hypersecretion of the mucous glands to be present. The appendix shares in the generalized intestinal mucous gland involvement peculiar to cystic fibrosis. Awareness of this fact has not been widespread, perhaps because of the usual emphasis on the pulmonary and pancreatic aspects of the disease. Greater appreciation of the clinical symptoms of cystic fibrosis and more liberal use of the sweat test could have diminished the number of exploratory celiotomies. Recognition of the histoloc changes in the removed appendices can also suggest the proper diagnosis and lead to earlier institution of treatment with an improved prognosis. RF 001 THOMAIDIS TS J PEDIATR 63 444 963 002 CORDONNIER JK SURGERY 54 667 963 003 MULLINS F JAMA 192 741 965 004 JAFFE BF ARCH SURG 92 337 966 005 BECK AR GASTROENTEROLOGIA 106 84 966 006 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 007 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 008 ANDERSEN DH ANN NY ACAD SCI 93 500 962 009 SHWACHMAN H N ENGL J MED 286 1300 972 010 SHWACHMAN H AM J DIS CHILD 96 6 958 011 GRAND RJ CLIN PEDIATR 9 588 970 012 KOPEL FB GASTROENTEROLOGY 62 483 972 013 DI SANTAGNESE PA PEDIATRICS 18 387 956 014 SHIER KJ CAN MED ASSOC J 89 645 963 015 HOLSCLAW DS PEDIATRICS 48 51 971 016 WARWICK WJ PEDIATRICS 34 621 964 017 PARKINS RA LANCET 2 851 963 018 MURTHY MSN OHIO STATE MED J 69 768 973 CT 1 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 2 HOLSCLAW DS J PEDIATR SURG 11 217 976 3 LOBER CW JAMA 235 1140 976 4 SUPER M S AFR MED J 52 991 977 5 SHWACHMAN H MEDICINE 56 129 977 6 ANGERPOINTNER T Z KINDERCHIR GRENZGEB 24 99 978 7 PARK RW GASTROENTEROLOGY 81 1143 981 8 ROSENSTEIN BJ J PEDIATR GASTROENTEROL NUTR 2 299 983 9 ROSENBERG E KLIN PAEDIATR 195 323 983 10 GEORGE DH HUM PATHOL 18 75 987 PN 74097 RN 00096 AN 75042703 AU Dolan-T-F-Jr. Touloukian-R-J. TI Familial meconium ileus not associated with cystic fibrosis. SO J-Pediatr-Surg. 1974 Dec. 9(6). P 821-24. MJ INTESTINAL-OBSTRUCTION: fg. MECONIUM. MN CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. HUMAN. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: et, ra. MALE. EX With rare exceptions, patients with meconium ileus have cystic fibrosis of the pancreas with pancreatic achylia, elevated salt content of sweat, and characteristic pulmonary manifestations. We wish to report the occurrence of meconium ileus in two full-term siblings with normal pancreatic function and no evidence of cystic fibrosis by history of physical findings, and with normal sweat electrolytes. To our knowledge this is the first report of familial meconium ileus without associated cystic fibrosis of the pancreas or pancreatic insufficiency. RF 001 RICKHAM PP AM J SURG 109 173 965 002 GELLIS SS YEARBOOK OF PEDIATRICS 204 966 003 MACLAURIN C AUST NZ J SURG 34 196 964 004 EMERY JL ARCH DIS CHILD 32 17 957 005 MEEKER IA SURG CLIN NORTH AM 44 1483 969 006 BRETON A SEM HOP PARIS 35 1101 959 007 KORNBLITH BA AM J PATHOL 5 249 929 008 HURWITT ES AM J DIS CHILD 64 443 942 009 AUBURN RP SURGERY 65 689 969 010 CHING NPH ARCH SURG 90 65 965 011 GILLIS DA CAN MED ASSOC J 92 225 965 CT 1 SHIGEMOTO H J PEDIATR SURG 13 475 978 2 ROSENSTEIN BJ AM J DIS CHILD 132 167 978 3 YOSHIKAWA Y ACTA PATHOL JAP 31 845 981 4 PARK RW GASTROENTEROLOGY 81 1143 981 5 OLSEN MM J PEDIATR SURG 17 479 982 6 HILLEMEIER AC PEDIATRICS 69 325 982 7 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 8 TAL A CLIN PEDIATR 24 460 985 9 LAMBERTY JM ANAESTHESIA 40 448 985 10 SAMUEL N J ULTRASOUND MED 5 425 986 PN 74098 RN 00097 AN 74132411 AU Lee-P-A. Roloff-D-W. Howatt-W-F. TI Hypoproteinemia and anemia in infants with cystic fibrosis. A presenting symptom complex often misdiagnosed. SO JAMA. 1974 Apr 29. 228(5). P 585-8. MJ ANEMIA: co. CYSTIC-FIBROSIS: co. HYPOPROTEINEMIA: co. INFANT-NUTRITION. MN AGE-FACTORS. BODY-WEIGHT. BREAST-FEEDING. DIARRHEA-INFANTILE: di. DIETARY-PROTEINS: ad. EDEMA: di. FEMALE. HUMAN. INFANT. MALE. MILK. MILK-HUMAN. NUTRITIONAL-REQUIREMENTS. PLANT-EXTRACTS: ad. RETROSPECTIVE-STUDIES. SERUM-ALBUMIN: an. SOY-BEANS. AB Infants with cystic fibrosis occasionally may present with a symptom complex of anemia, hypoproteinemia, and edema. Case reports of seven infants between the ages of 1 to 4 months who developed these abnormalities indicate that in several instances the children's condition was misdiagnosed as milk-sensitivity or milk-allergy. Furthermore, their case histories verify that their hypoalbuminemia and anemia is nutritional and is corrected when a readily absorbed diet with pancreatic-enzyme supplement is given. A retrospective review of feeding histories of 100 infants with cystic fibrosis, as well as these case reports, indicate that affected children who are breast-fed or fed with soy-based formulas are more likely to develop the syndrome. RF 001 FLEISHER DS J PEDIATR 64 341 964 002 DURAND P MINERVA PEDIATR 11 717 959 003 RODRIQUEZ-VIGIL L REV ESP PEDIATR 20 157 964 004 LIBAN E ISR J MED SCI 2 513 966 005 LEVIN SE S AFR MED J 41 482 967 006 DUKES RE ILL MED J 134 147 968 007 OLIVE BADOSA A AN MEDICINA 54 20 968 008 STRALA-DELIDZAKOVA M GOD ZBORN MED FAK SKOPJE 14 313 968 009 DOLAN TF JR CLIN PEDIATR 9 295 970 010 STROBER W PEDIATRICS 43 416 969 011 FLEISHER DS J PEDIATR 64 349 964 012 SHAHIDI NT J PEDIATR 59 533 961 013 GOLDMAN AS J PEDIATR 59 301 961 014 MACLEAN WC JR J PEDIATR 83 86 973 015 DI SANTAGNESE PA N ENGL J MED 277 1344 967 CT 1 LARSON PH HUM PATHOL 5 629 974 2 DOLAN TF CLIN PEDIATR 15 597 976 3 SWISCHUK LE J PEDIATR 88 452 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 SUNSHINE P CLIN GASTROENTEROL 6 445 977 6 BASS HN PEDIATRICS 59 126 977 7 GUNN T AM J DIS CHILD 132 317 978 8 MARIANELLI L MINERVA PEDIATR 31 1787 979 9 CHU JY NUTR REV 37 351 979 10 WORLEY L J PEDIATR 94 1005 979 11 MAYA PP TROP GEOGR MED 32 45 980 12 BLUMENTHAL I ARCH DIS CHILD 55 812 980 13 SHEPHERD R J PEDIATR 97 351 980 14 PARK RW GASTROENTEROLOGY 81 1143 981 15 NIELSEN OH J PEDIATR GASTROENTEROL NUTR 1 355 982 16 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 17 MCCARTHY VP J PEDIATR GASTROENTEROL NUTR 4 320 985 18 DODGE JA J ROY SOC MED 79 27 986 PN 74099 RN 00098 AN 75023824 AU Paxson-C-L-Jr. Lock-J. TI Letter: Idiopathic hypoproteinemia in twins. SO JAMA. 1974 Dec 2. 230(9). P 1257-8. MJ CYSTIC-FIBROSIS: fg. DISEASES-IN-TWINS. HYPOPROTEINEMIA: fg. MN ANEMIA: fg. CYSTIC-FIBROSIS: co. EDEMA: fg. HUMAN. HYPOPROTEINEMIA: et. INFANT. INFANT-NEWBORN. MALE. SYNDROME. EX Since the report of an edema-anemia-hypoproteinemia symptom complex in infants with cystic fibrosis, several articles have been published. We have also participated in the care of identical male twins with this interesting symptom complex. These twins offer two more examples of the edema-anemia-hypoproteinemia symptom complex of infant cystic fibrosis. In the presence of transsupplementation and the absence of hemolysis, the normal serum iron and increased peripheral and marrow iron values suggest that the anemia results from a defect at the level of hemoglobin synthesis, and that iron transport remains sufficient in the face of protein depletion. Possibly, a relative iron deficiency occurs after increased nutritional intake due to increased utilization. Lee suggested that the hypoproteinemia is due to loss of protein in stools, but these twins suggest that the problem lies not with losses through urine or stool, but rather with failure in protein synthesis. The possibility of hypoproteinemia secondary to liver dysfunction is attractive. Although all liver function tests and biopsy findings were normal, this is often the case in patients with cystic fibrosis. The anemia is associated with increased serum and marrow iron values, and is most likely due to a decrease in hemoglobin synthesis. Routine cystic fibrosis therapy appears to be sufficient for clinical recovery for all components of the symptom complex. RF 001 BILLE BSV ACTA PAEDIATR SCAND 44 435 955 002 LEE PA JAMA 228 585 974 003 SHAHIDI NT J PEDIATR 59 533 961 004 KOPEL FB GASTROENTEROLOGY 62 483 972 CT 1 GUNN T AM J DIS CHILD 132 317 978 2 BLUMENTHAL I ARCH DIS CHILD 55 812 980 PN 74100 RN 00099 AN 75008520 AU Cooper-H-S. Oppenheimer-E-H. TI Cystic fibrosis manifested as focal biliary cirrhosis in a newborn infant with congenital heart disease. SO Johns-Hopkins-Med-J. 1974 Oct. 135(4). P 268-73. MJ CYSTIC-FIBROSIS: di. HEART-DEFECTS-CONGENITAL: co. LIVER-CIRRHOSIS-BILIARY: di. MN AUTOPSY. CYSTIC-FIBROSIS: co, cn, pa. HUMAN. INFANT-NEWBORN. INTESTINES: pa. LIVER-CIRRHOSIS-BILIARY: co, cn, pa. LIVER: pa. MALE. PANCREAS: pa. AB A 3-day-old infant, meconium stained at birth, presented with respiratory distress. Hypoplastic left heart syndrome was diagnosed clinically and documented at autopsy. Unsuspected cystic fibrosis was demonstrated by the histologic presence of severe focal biliary cirrhosis with spreading fibrosis in the liver; minimal lesions in the pancreas and intestines were compatible with a diagnosis of cystic fibrosis. The abundant hepatic fibrosis is unusual in an infant of this age indicating its in utero origin. This case is also unusual because of the fortuitous association of cystic fibrosis and a congenital cardiac malformation. RF 001 FARBER S ARCH PATHOL 37 238 944 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 CRAIG JM AM J DIS CHILD 93 357 957 004 BERNSTEIN JG AM J DIS CHILD 99 804 960 005 DI SANTAGNESE PA PEDIATRICS 18 387 956 006 OPPENHEIMER EH ARCH PATHOL 96 149 973 007 VALMAN HB LANCET 2 566 969 008 JOCKIN H AM J PATHOL 62 22A 971 009 KOPITO L PEDIATRICS 49 620 972 010 SHIER KJ CAN MED ASSOC J 89 645 963 011 OPPENHEIMER EA AM J MED 48 637 970 012 ANDERSEN DH AM J DIS CHILD 56 344 938 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 SCHWARZ HP HELV PAEDIATR ACTA 33 351 978 3 PARK RW GASTROENTEROLOGY 81 1143 981 PN 74101 RN 00100 AN 74148799 AU Oppenheimer-E-H. Esterly-J-R. TI Medical mucoid lesions of the pulmonary artery in cystic fibrosis, pulmonary hypertension, and other disorders. SO Lab-Invest. 1974 Apr. 30(4). P 411-6. MJ CYSTIC-FIBROSIS: pa. HYPERTENSION-PULMONARY: pa. MUCOPOLYSACCHARIDES: an. PULMONARY-ARTERY: pa. PULMONARY-HEART-DISEASE: pa. MN ADOLESCENCE. ADULT. AUTOPSY. CHILD. CHILD-PRESCHOOL. CONNECTIVE-TISSUE: pa. FEMALE. HEART-DEFECTS-CONGENITAL: pa. HEART-ENLARGEMENT: pa. HISTOCYTOCHEMISTRY. HUMAN. MALE. MICROSCOPY. MIDDLE-AGE. AB Alterations in the pulmonary vascular bed were reviewed in the autopsy material from 35 patients with cystic fibrosis of the pancreas and cor pulmonale. Hypertrophy of the media or intimal proliferation of small muscular arteries and arterioles was noted in eight patients; intimal proliferation of the larger caliber arteries was seen in seven patients. The unexpected change, however, was the presence of interstitial hyaline material in the media of the pulmonary artery or its major branches in one-third of the specimens. The elastic fibers were distorted, but not disrupted, and the finding was unrelated to the presence or severity of other vascular changes. Intimal deposits of the same material were also present in seven of the patients with intimal proliferation in major pulmonary arteries. Identical medial lesions were found in the pulmonary arteries of seven of 35 children with pulmonary hypertension and in seven patients with other disorders. Similar lesions in the media of the aorta were found in each group of patients. The material was not Schiff-reactive, but had the histochemical characteristics of a connective tissue acid mucopolysaccharide. RF 001 GOLDRING RM J PEDIATR 65 501 964 002 HOLMAN RL PEDIATRICS 24 34 959 003 LIEBOW AA IN: GOULD SE 940 960 004 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 005 SMITH HL AM HEART J 4 79 928 006 SPICER SS J HISTOCHEM CYTOCHEM 8 18 960 007 SYMCHYCH PS ARCH PATHOL 92 409 971 008 TIGHE JR J PATHOL BACTERIOL 86 141 963 009 WAGENVOORT CA PATHOLOGY OF PULMONARY VASCUL 964 010 WAGENVOORT CA AM J CARDIOL 9 446 962 CT 1 MUKHERJI RN ARCH DIS CHILD 51 563 976 2 CHIOSSI FM GASLINI 10 86 978 PN 74102 RN 00101 AN 74303978 AU Gupte-S. TI Letter: Treatment of cystic fibrosis. SO Lancet. 1974 Aug 31. 2(879). P 521. MJ CYSTIC-FIBROSIS: th. PROTEIN-HYDROLYSATES: ae. DIET-THERAPY. MN CHILD-PRESCHOOL. CHILD. HUMAN. CYSTIC-FIBROSIS: dt. PROTEIN-HYDROLYSATES: tu. NAUSEA: ci. VOMITING: ci. EX This letter is prompted by recent reports of favourable results after the use of an artificial diet consisting of a protein hydrolysate, a glucose polymer, and medium-chain glycerides in the treatment of cystic fibrosis of the pancreas. It has been our observation that children often find these preparations unpleasant and unpalatable. Loose motions, nausea, and vomiting are quite common. I have comparable experience with their use in other malabsorption states and advanced protein-calorie malnutrition. Whether the untoward effects result from excessive flooding of gut and/or exceedingly high blood-amino acid levels is a matter of speculation. RF 001 ALLAN JD LANCET 1 785 970 002 ALLAN JD AM J DIS CHILD 126 22 973 003 GUPTE S INDIAN J MED RES 56 1663 968 004 GUPTE S PEDIATR CLIN INDIA 4 263 969 005 MEHTA S J ASSOC PHYICIANS INDIA 6 16 968 006 MEHTA S PEDIATR CLIN INDIA 4 259 969 PN 74103 RN 00102 AN 74303487 AU Hann-S. Holsclaw-D-S. Shin-H-S. TI Letter: Complement components in cystic fibrosis. SO Lancet. 1974 Aug 31. 2(879). P 520-1. MJ CYSTIC-FIBROSIS: im. COMPLEMENT: an. MN CHILD-PRESCHOOL. CHILD. ADOLESCENCE. ADULT. HUMAN. MALE. FEMALE. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. EX Dr. Conover and his colleagues reported no difference in the haemolytic activity of serum complement components C1-C9 of cystic fibrosis (C.F.) patients, obligate heterozygotes, and normal controls. They also measured individual complement components by radial immunodiffusion and found a significant rise of C3 but no significant difference in C5 levels between these groups. Using immunodiffusion kits from the same manufacturer and freshly obtained sera, we have observed quite different findings. C5 was mildly elevated in 20 and more highly elevated in 25 of 56 C.F. patients, 2 to 29 years of age. C5 was also elevated in 24 of 51 parents of C.F. patients, 15 showing mild and 9 higher elevations, while 4 of 24 controls showed only mild elevation. No correlation was observed between serum-C5 levels, and the age, sex, clinical severity of disease (judged by Shwachman-Kulczyki scores), sputum bacterial colonisation, and mode of treatment of the C.F. patients. The elevation of C3 and C5 in a majority of patients with C.F. and obligate heterozygotes is very interesting and is suggestive of certain genetic defect(s) in the complement activation system in this autosomal recessive disorder. RF 001 CONOVER JH LANCET 2 1501 973 002 LIEBERMAN J LANCET 1 1230 974 CT 1 SCANLIN TF LANCET 1 1382 975 2 WEEKE B DAN MED BULL 23 155 976 3 HOLZHAUER RJ AM J HUM GENET 28 602 976 4 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 5 GENIN C ARCH FR PEDIATR 34 717 977 6 STRUNK RC ARCH DIS CHILD 52 687 977 7 LYRENE RK J PEDIATR 91 681 977 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 HODSON ME THORAX 35 801 980 12 MOSS RB AM REV RESPIR DIS 121 23 980 13 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 14 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 74104 RN 00103 AN 74284429 AU Norman-A-P. Mall-M-L. Johns-M-K. TI Letter: Skin wrinkling in cystic fibrosis. SO Lancet. 1974 Aug 10. 2(876). P 358-9. MJ CYSTIC-FIBROSIS. SKIN-MANIFESTATIONS. MN HUMAN. IMMERSION. WATER. HEAT. EX The letter from Professor Elliott appears to be the first record of skin wrinkling in cystic fibrosis and may be an observation as important as that of di Sant' Agnese when he noted the high salt concentration of the sweat. It seems that parents are well aware of this response, and repeating Professor Elliott's observation by soaking the hands of six infants with cystic fibrosis in hot water showed that very marked wrinkling was present by five minutes and often by two minutes. This did not occur in control children of similar nutritional state except in a case of twins - one with cystic fibrosis, one without - where the unaffected child's fingers began to wrinkle slightly by five minutes. The wrinkling does not appear to be associated with wasting due to malnutrition since it occurs equally in plump and in thin infants with cystic fibrosis, nor is it present in children with severe wasting and respiratory disease not due to cystic fibrosis. RF 001 ELLIOTT RB LANCET 2 108 974 CT 1 SHELLEY WB ACTA DERM VENEREOL SUPPL 108 1983 1 983 PN 74105 RN 00104 AN 75063155 AU Stuart-A-B. Burdon-M-G. TI Letter: Frequency of cystic-fibrosis gene. SO Lancet. 1974 Dec 21. 2(7895). P 1521. MJ CYSTIC-FIBROSIS: fg. GENE-FREQUENCY. MN CAUCASOID-RACE. HETEROZYGOTE. HUMAN. POLYMORPHISM-GENETICS. EX An unsolved problem in human population genetics is the high frequency of the gene for cystic fibrosis (C.F.) in Caucasians. Whereas the gene frequency is probably low enough in non-Caucasians to be maintained by recurring mutation, this is not the case in Caucasians, where the carrier frequency is approximately 1 in 25. In order to establish a stable genetic polymorphism for a gene where the homozygous state is lethal, some degree of heterozygote advantage must exist, and it has been suggested that C.F. heterozygotes have a higher reproductive fitness than normal individuals. It is not possible, of course, to know exactly how stable the C.F. polymorphism is, and one cannot exclude the possibility that some form of heterozygote advantage has existed at some time in the past and no longer exists today. We should like to suggest a possible explanation which has much in common with the generally accepted explanation for the very high frequency of the sickle-cell gene in certain populations. In the latter case, heterozygotes have an increased resistance to a particular type of malaria. Patients with C.F. and heterozygous carriers have been found to have, in their serum, a substance (probably a protein) that agglutinates Proteus vulgaris. Immunological cross-reactivity between certain strains of Proteus (e.g., OX 19) and Rickettsia prowazeki has long provided the basis of a serological test for typhus (Weil-Felix reaction). Thus it is conceivable that the C.F. heterozygote might have an increased innate ability to counter infection by R. prowazeki. Although typhus is known in many parts of the world, it seems to have been predominantly endemic and epidemic in the temperate regions inhabited by Caucasian peoples. Some areas where it is now endemic, such as South America, first experienced the disease after it had been introduced by Europeans. This would seem to be compatible with the observed higher C.F. gene frequency in Caucasians than in other groups. RF 004 KNUDSON AG JR AM J HUM GENET 19 388 967 005 COHEN FL J MED GENET 11 253 974 CT 1 SAUGSTAD LF J MED GENET 14 20 977 2 SCHAAP T ISR J MED SCI 14 201 978 3 SHIER WT MED HYPOTHESES 5 661 979 4 HOLLANDER DH MED HYPOTHESES 8 191 982 PN 74106 RN 00105 AN 76124835 AU Elliott-R-B. TI Letter: Skin wrinkling in cystic fibrosis. SO Lancet. 1974 Dec 7. 2(7893). P 1383. MJ CYSTIC-FIBROSIS. SKIN. MN HUMAN. IMMERSION. TIME-FACTORS. EX Dr. Economou-Mavrou and his colleagues appear to have misunderstood my previous note concerning skin wrinkling in cystic fibrosis. The range of normality varies in different climatic conditions, and in Aukland wrinkling does not appear in cool tap-water (18-22 C) under 3 minutes in normals. Skin wrinkling on immersion appears to be age-related, but is difficult to detect reliably in the newborn. Prior washing of the hands, or immersion in sea-water (even the day before), accelerates skin wrinkling, as does warm weather. I would suggest that "normality" needs redefining in Athens if 6 of 17 "normals" were "abnormal". RF 001 ECONOMOU-MAVROU C LANCET 2 953 974 PN 74107 RN 00106 AN 74066757 AU Caudill-M. Schafer-I. Stjernholm-R. TI Letter: Sulphate incorporation of leucocytes from patients with cystic fibrosis. SO Lancet. 1974 Jan 5. 1(845). P 32. MJ CYSTIC-FIBROSIS: bl. LEUKOCYTES: me. SULFATES: bl. MN CELLS-CULTURED. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HUMAN. SODIUM: bl. EX In preliminary studies of short-term leukocyte cultures using Na2-35SO4 incorporation into cetyl-pyridinium-chloride (C.P.C.)-precipitable material (presumably glycosaminoglycans, G.A.G.), we have found significant variation among C.F. patients and their parents (obligate heterozygotes). This biochemical heterogeneity is comparable to what has been reported in fibroblast cultures and serum. The amount of Na2-35SO4 incorporated into G.A.G. material found in both the incubation medium and the cells from C.F. patients is significantly increased over normals. A similar analysis between normals and heterozygotes shows no significant difference in Na2-35SO4 incorporation into G.A.G. material of the cells. However, 4 out of 6 heterozygotes appeared to show a pattern of increased incorporation into material found in the incubation medium (indicated by a higher mean than the normal donors). These results suggest yet another system to study the effects of the C.F. gene. The incorporation of Na2-35SO4 into G.A.G. material of C.F. leukocyte cultures gives additional evidence for the genetic heterogeneity in this disorder. RF 001 DANES BS J EXP MED 129 775 969 002 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 003 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 004 CONOVER JH LANCET 1 1122 973 005 STJERNHOLM RL J RETICULOENDOTHEL SOC 6 194 969 006 OLSSON I BIOCHIM BIOPHYS ACTA 141 348 967 CT 1 ROBERTSON JA LANCET 1 1256 974 2 DANES BS LANCET 2 655 974 3 DANES BS J MED GENET 12 405 975 4 DANES BS TEX REP BIOL MED 34 135 976 PN 74108 RN 00107 AN 74259959 AU Lieberman-J. TI Letter: Complement components in cystic fibrosis. SO Lancet. 1974 Jun 15. 1(868). P 1230. MJ CYSTIC-FIBROSIS: im. COMPLEMENT: ip. MN ADOLESCENCE. ADULT. HUMAN. MALE. FEMALE. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. GEL-DIFFUSION-TESTS. SEX-FACTORS. HETEROZYGOTE. EX Freshly obtained sera were assayed from 14 C.F. young adult patients (age 18 - 33, 7 men and 7 women) and from 15 control patients with bronchiectasis or chronic bronchitis in whom C.F. had been ruled out. In addition, the blood from 2 mothers (heterozygotes) of C.F. patients was assayed. The mean C3 level in the C.F. men was slightly lower than that in the male controls, whereas the C.F. women had a significantly higher mean level of C3 than did the female controls. The C3 level in the C.F. women was also significantly higher than that of the C.F. men (p < 0.01, t = 3.855 by the Student's t test). The female controls had slightly higher C3 levels than did the male controls, but this difference was not significant. Comparison of C3 levels from all of the C.F. subjects to the controls shows considerable overlap in values, especially in the men. These studies suggest that an assay of serum-C3 does not effectively distinguish C.F. subjects from patients with other types of chronic lung disease. However, the differences between C.F. women and C.F. men is interesting and deserves further attention. Our data from C.F. young adults do not fully confirm those reported by Conover et al., but it is possible that the experimental effects of carboxypeptidase B observed by Conover might result from action of the enzyme on the C.F. factor of serum, although its identity most likely is not C3a. RF 001 CONOVER JH LANCET 2 1501 973 CT 1 CONOVER JH LANCET 1 47 975 2 WILSON GB PEDIATR RES 10 87 976 3 WEEKE B DAN MED BULL 23 155 976 4 LEDERBERG S AM J HUM GENET 28 597 976 5 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 6 JAKEL HP BIOL ZENTRALBL 98 55 979 7 SANDERSON MJ PEDIATR RES 15 219 981 PN 74109 RN 00108 AN 74251010 AU Robertson-J-A. Chernick-B. Segal-D-J. McCoy-E-E. TI Increased uptake of 35S-heparin by lymphocytes from patients with cystic fibrosis. SO Lancet. 1974 Jun 22. 1(869). P 1256-7. MJ CYSTIC-FIBROSIS: bl. LYMPHOCYTES: me. HEPARIN: me. MN HUMAN. CYSTIC-FIBROSIS: fg. SULFUR-RADIOISOTOPES. HOMOZYGOTE. HETEROZYGOTE. BLOOD-SPECIMEN-COLLECTION. CELLS-CULTURED. AB Previous studies on cystic fibrosis (C.F.) have shown increased mucopolysaccharide synthesis in C.F. cells. The uptake of 35S-heparin sulphonate into purified lymphocytes was examined after 3 days' culture in medium 199 supplemented with 20% autochthonous plasma. Fourteen C.F. homozygotes, seven siblings, and nine parents all showed significantly greater uptake (p < 0.001) of 35S-heparin than cultures from twenty-three normal controls. There was partial overlap in heparin-uptake values between C.F. patients and their families. This procedure thus differentiates C.F. families from the normal population, but does not differentiate C.F. homozygotes from heterozygotes. It is hoped that either variations in culture methods or a combination of this procedure with other measurements of C.F., such as ciliary dyskinesis or cellular metachromasia, will permit unequivocal identification of C.F. carriers. RF 001 STEELE MW PEDIATR RES 5 393 971 002 DANES BS LANCET 2 437 969 003 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 004 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 005 DOGGETT RG NATURE NEW BIOL 243 250 973 006 CAUDILL M LANCET 1 32 974 007 CAUDILL M LANCET 2 307 973 008 DANES BS J EXP MED 129 775 969 009 STEELE MW CF CLUB ABST 974 010 DANES BS LANCET 2 765 973 011 BOWMAN BH LANCET 1 404 974 012 GOLD RJM ANN HUM GENET 37 315 974 CT 1 DANES BS LANCET 2 655 974 2 DANES BS J MED GENET 12 405 975 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 PN 74110 RN 00109 AN 74304018 AU Elliott-R-B. TI Letter: Wrinkling of skin in cystic fibrosis. SO Lancet. 1974 Jul 13. 2(872). P 108. MJ CYSTIC-FIBROSIS: di. MN INFANT. CHILD-PRESCHOOL. CHILD. HUMAN. SKIN. WATER. IMMERSION. EX I have observed that the skin of children with cystic fibrosis wrinkles excessively early when soaked in tap water. This physical sign is highly reliable irrespective of the degree of nutrition of the patient. It is sometimes seen in infants with marasmus. Normal skin wrinkles eventually to exposure to water (except that from the Dead Sea). It is possible that the disorder of salt flux in cystic fibrosis is connected with the apparent excessive hypertonic reactivity of the skin. Three minutes and a bowl of water might provide a cheap screening test for this condition. CT 1 BULL C BR MED J 1 551 977 2 BRAHAM J ARCH NEUROL 36 113 979 PN 74111 RN 00110 AN 74107646 AU Kilbourn-J-P. TI Letter: Cystic fibrosis. SO Lancet. 1974 Mar 9. 1(854). P 405. MJ CYSTIC-FIBROSIS: mi. MN CANDIDA: ip. ENTEROBACTERIACEAE: ip. HAEMOPHILUS-INFLUENZAE: ip. HUMAN. PSEUDOMONAS: ip. SPUTUM: mi. STAPHYLOCOCCUS: ip. STREPTOCOCCUS: ip. EX In 1972 May, Herrick, and Thompson questioned the classical hypothesis of cystic fibrosis (C.F.) and suggested that C.F. children are in fact abnormally susceptible fo any respiratory pathogen from birth. It is perhaps a bit premature to change the classical hypothesis for the bacteriology of C.F. Since the predominant flora of hospital nurseries is not now Staph. aureus but rather gram-negative bacteria, it may be that the initial exposure of C.F. patients is to an organism other than Staph. aureus. In addition, other organisms are becoming as resistant to antibodies as Ps. aeruginosa, so other organisms could perhaps be present in terminal sputum flora - which agrees with our data. RF 001 MAY JR ARCH DIS CHILD 47 908 972 002 BURNS MW LANCET 1 270 968 003 KILBOURN JP AM REV RESPIR DIS 98 810 968 CT 1 KILBOURN JP LANCET 1 334 978 2 KILBOURN JP PEDIATR RES 14 259 980 PN 74112 RN 00111 AN 74168792 AU Evans-T-J. McCrae-W-M. Innes-E-M. TI Letter: Nitroblue-tetrazolium test in cystic fibrosis. SO Lancet. 1974 May 18. 1(864). P 992. MJ CYSTIC-FIBROSIS: di. TETRAZOLIUM-SALTS: du. MN CHILD. CHILD-HEALTH-SERVICES. HUMAN. EX It has been claimed that the nitroblue tetrazolium (N.B.T.) test is of value in the surveillance of children with cystic fibrosis, a positive test giving evidence of active bacterial infection which may not be apparent clinically and not made evident by such investigations as the white-blood-cell count, sputum culture, and chest X-ray. The test was therefore included in the routine assessment of patients attending this clinic. Of the total 269 N.B.T. tests, 110 were positive. In only 57 of these cases was there other evidence of infection; these 57 were treated with antibiotics. The remaining 53 were left untreated. Of these, 41 remained completely free of infection by standard criteria at subsequent review. In 12 cases, however, the patient developed obvious infection before the next clinic visit. It could be argued that in those cases the N.B.T. test had given early evidence of infection not otherwise apparent. This is by no means certain, since the onset of infection could have been at any time during the four-week interval. In our experience, the N.B.T. has been of little help in the management of cystic fibrosis. There were in this series 27% false-negatives and at least 37% false-positives, some of which had very high counts. It is possible, however, that in 12 (i.e., 4%) of the tests carried out, the N.B.T. gave early indication of infection not otherwise suspected. RF 003 SULLIVAN JF AM J DIS CHILD 125 702 973 004 PARK BH LANCET 2 532 968 CT 1 BERRY DH ANN ALLERGY 38 316 977 2 BRAITO A MINERVA PEDIATR 30 1601 978 PN 74113 RN 00112 AN 74107645 AU Bowman-B-H. Hirschhorn-K. Bearn-A-G. TI Letter: Bioassays of cystic-fibrosis factor. SO Lancet. 1974 Mar 9. 1(854). P 404-5. MJ CYSTIC-FIBROSIS: di. MN BIOLOGICAL-ASSAY. BLOOD-SPECIMEN-COLLECTION. CILIA. CYSTIC-FIBROSIS: bl. HUMAN. REFRIGERATION. EX We wish to respond to the letter of Dr. Woods and Dr. di Sant'Agnese regarding their study on the reliability of the ciliary assays in cystic-fibrosis screening. These investigators concluded that results from their study ruled out the possibility that reliable conclusions could be obtained from ciliary assays. We feel that this conclusion is far from accurate, although it has never been the intention of any of our research groups to utilise ciliary preparations for massive heterozygote screening or prenatal diagnosis. Ciliary assays have provided valuable scientific progress in cystic fibrosis research. The existing assays are not suitable for massive screening for cystic fibrosis or for the clinical detection of cystic-fibrosis carriers; nevertheless, these techniques offer the most promising method to study, purify, and characterise a molecule or molecules directly or indirectly related to the pathogenesis of cystic fibrosis. RF 000 WOOD RE LANCET 2 1452 973 001 DANES BS J EXP MED 129 775 969 002 CONOVER JH LANCET 1 1122 973 005 BESLEY GTN J MED GENET 6 278 969 006 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 CONOVER JH PEDIATR RES 7 224 973 011 CONOVER JH PEDIATR RES 7 220 973 012 BERATIS NG PEDIATR RES 7 958 973 013 DANES BS J EXP MED 136 1313 972 014 DANES BS J EXP MED 137 1538 973 015 SCHMOYER IR BIOCHEM BIOPHYS RES COMMUN 46 1923 972 016 SPOCK A PEDIATR RES 1 173 967 CT 1 ROBERTSON JA LANCET 1 1256 974 2 POLLEY MJ J MED GENET 11 249 974 3 ALTLAND K HUM GENET 28 207 975 4 BENDER SW MONATSSCHR KINDERHEILKD 123 216 975 5 BOWMAN BH TEX REP BIOL MED 34 1 976 6 WARD JB TEX REP BIOL MED 34 11 976 7 VISSER GHA EUR J OBSTE GYNECOL REPR BIOL 7 109 977 8 THOMAS JM PEDIATR RES 11 1148 977 9 WILSON GB PEDIATR RES 11 143 977 10 BOWMAN BH FED PROC 39 3195 980 11 HEELEY AF CLIN CHEM 29 2011 983 PN 74114 RN 00113 AN 74116631 AU Valman-H-B. TI Intelligence after malnutrition caused by neonatal resection of ileum. SO Lancet. 1974 Mar 16. 1(855). P 425-7. MJ ILEUM: su. INFANT-NEWBORN-DISEASES: su. INFANT-NUTRITION-DISORDERS: co. INTELLIGENCE. MENTAL-RETARDATION: et. MN ADOLESCENCE. BIRTH-ORDER. BIRTH-WEIGHT. CHILD. CHILD-DEVELOPMENT. CHILD-HOSPITALIZED. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. INTELLIGENCE-TESTS. MALE. MATERNAL-AGE. MATERNAL-DEPRIVATION. MENTAL-RETARDATION: di. SOCIAL-CLASS. AB Assessment of intelligence by the "draw-a-man" test and school reports has shown that, despite severe early malnutrition in children who had neonatal resection of ileum, the frequency of mental retardation is no higher than in the normal population. The scores in patients with cystic fibrosis were also similar to the control group. RF 001 CRAVIOTO J AM J ORTHOPSYCHIATRY 35 449 965 002 STOCH MB S AFR MED J 41 1027 967 003 MONCKEBERG F IN: SCRIMSHAW NS 269 968 004 DOBBING J IN: DAVISON AN 287 968 005 WINICK M PEDIATRICS 47 969 971 007 HARRIS DB CHILDRENS DRAWINGS AS MEASURE 90 963 008 BURT C MENTAL AND SCHOLASTIC TESTS 251 962 009 ANON REGISTRAR GENERALS DECENNIAL 954 010 SWAIN VAJ ARCH DIS CHILD 38 103 963 011 YOUNG WF ARCH DIS CHILD 44 465 969 012 YAKTIN US J MENT DEFIC RES 14 25 970 013 BIRCH HG PEDIATR RES 5 579 971 014 MCKEOWN T BR MED J 3 63 970 015 DAVE R FROM BIRTH TO SEVEN 176 972 CT 1 VALMAN HB J PEDIATR 88 41 976 2 HALLERT C J NEUROL NEUROSURG PSYCHIAT 46 87 983 3 STAUFFER UG MONATSSCHR KINDERHEILKD 132 411 984 4 STEIN Z PSYCHOL MED 15 717 985 PN 74115 RN 00114 AN 75044438 AU Bang-B-G. Bang-F-B. TI Invertebrate model for study of macromolecular regulators of mucus secretion. SO Lancet. 1974 Nov 30. 2(7892). P 1292-4. MJ ANNELIDA. MACROMOLECULAR-SYSTEMS. MODELS-BIOLOGICAL. MUCUS: se. MN ADULT. AMYLASES: pd. ANIMAL. ANNELIDA: cy. BLOOD: an. CELLS-CULTURED. CHILD. CYSTIC-FIBROSIS: pp. HEAT. HUMAN. MUCUS: cy, de. PRONASE: pd. SALIVA: an. TEARS: an. TRYPSIN: pd. ULTRACENTRIFUGATION. ULTRAFILTRATION. URINE: an. AB Large molecules which consistently stimulate hypersecretion in individual mucus-secretory sells in an animal system (the marine invertebrate Sipunculus) have been found in human serum and urine. This activity is heat-evoked in serum, heat-labile in urine. Heat-stable and heat-evoked factors were also found respectively in saliva and tears. The serum and urine factors withstood freeze-thawing. Activity of both factors was destroyed by pronase, and activity of the urine factor was destroyed by trypsin. After incubation with alphaamylase the factor in urine induced production of a very aberrant type of secretion. RF 001 BANG BG CAHIERS BIOL MAR 6 257 965 002 BANG BG AM J PATHOL 68 407 972 003 SPOCK A PEDIATR RES 1 173 967 004 BESLEY GTN J MED GENET 6 278 969 005 BOWMAN BH SCIENCE 164 325 969 006 CHERRY JD J PEDIATR 79 937 971 007 BARNETT DR TEX REP BIOL MED 31 703 973 008 DOGGETT RG TEX REP BIOL MED 31 685 973 009 SADE J ACTA OTOLARYNGOL STOCKH 70 351 970 010 BANG BG J EXP MED 130 105 969 011 TAMM I PROC SOC EXP BIOL MED 74 8 950 012 MAXFIELD M J CLIN INVEST 41 455 962 013 SCHWARTZ RH J LAB CLIN MED 70 725 967 014 MCLEAN IW J CELL BIOL 59 209 973 CT 1 BANG FB J PEDIATR 87 1062 975 2 BANG BG NATURE 253 634 975 3 BANG BG CAHIERS BIOL MAR 17 423 976 4 BANG BG JOHNS HOPKINS MED J 145 209 979 5 NICOSIA SV SCIENCE 206 698 979 6 KURLANDSKY LE PEDIATR RES 14 1263 980 7 FRANKLIN RM INVEST OPHTHALMOL VIS SCI 19 430 980 8 DYBAS LK BIOL BULL 161 104 981 9 BANG BG EUR J PEDIATR 140 22 983 PN 74116 RN 00115 AN 75012105 AU Weinstein-M. Ryan-J. Rogers-P. TI Letter: Skin wrinkling and nerve function. SO Lancet. 1974 Oct 12. 2(7885). P 907. MJ CYSTIC-FIBROSIS: pp. SKIN. MN CHILD. HAND: ir. HUMAN. PERIPHERAL-NERVES: pp. SKIN: ir. EX We were interested in Professor Elliott's description of early skin wrinkling in children with cystic fibrosis. Although this phenomenon may well be related to osmotic differences between skin and water, it has been observed that skin wrinkling (as seen on the finger pulp) is fully dependent on an intact peripheral nerve-supply. This is a useful sign in peripheral nerve lesions and helpful in following recovery when the wrinkling returns. Furthermore, it was our impression that in normal individuals wrinkling was more apparent on the left hand than on the right-handed individuals. However, in a preliminary study using fingerprint densities measured by photometry, no significant differences were found between the sides. RF 001 BUNCKE J SURG CLIN NORTH AM 52 1267 972 002 ORIAIN S BR MED J 3 615 973 PN 74117 RN 00116 AN 75024076 AU Economou-Mavrou-C. Antonakakis-C. Matsaniotis-N. TI Letter: Skin wrinkling in cystic fibrosis. SO Lancet. 1974 Oct 19. 2(7886). P 953. MJ CYSTIC-FIBROSIS: di. SKIN. MN ADULT. CHILD. CYSTIC-FIBROSIS: pp. DIAGNOSIS-DIFFERENTIAL. HUMAN. IMMERSION. SKIN-MANIFESTATIONS. WATER. EX Professor Elliott and Dr Norman and his colleagues suggested that excessive wrinkling of the skin after soaking in tap water might provide an easy, cheap, and probably reliable screening test for cystic fibrosis. Our results show that this not true. Marked wrinkling after immersion of the fingers in warm tap water for 5 minutes was observed in randomly selected children with a wide variety of conditions, including not only cystic fibrosis but also malnutrition and renal, heart, endocrine, collagen, and other diseases. It was elicited even in apparently healthy individuals, both young and adult. RF 000 NORMAN AP LANCET 2 358 974 000 ELLIOTT RB LANCET 2 108 974 PN 74118 RN 00117 AN 75027484 AU Laurence-K-M. TI Fetal malformations and abnormalities. SO Lancet. 1974 Oct 19. 2(7886). P 939-42. MJ PRENATAL-DIAGNOSIS. MN ABORTION-INDUCED. ADULT. ALPHA-GLOBULINS: an. LIPOIDOSIS: di. AMNIOCENTESIS. AMNIOTIC-FLUID: cy, an. ANENCEPHALY: di. CHROMOSOME-ABNORMALITIES: di. COSTS-AND-COST-ANALYSIS. CYSTIC-FIBROSIS: di. FEMALE. FETAL-PROTEINS: an. GESTATIONAL-AGE. HUMAN. KARYOTYPING. MATERNAL-AGE. METABOLISM-INBORN-ERRORS: di. DOWNS-SYNDROME: fg. PREGNANCY. RADIOIMMUNOASSAY. SEX-CHROMOSOME-ABNORMALITIES: di. SPINA-BIFIDA: di. STAINS-AND-STAINING. ULTRASONICS: du. AB The prevention of fetal malformations and abnormalities has long been the hope of obstetricians, paediatricians, and geneticists, but since the cause of most human malformations and of spontaneous gene and chromosome mutations is unknown, this must remain a dream for the present. However, with the introduction of mid-trimester amniocentesis it is now possible to detect some abnormalities early enough for selective abortion of abnormal fetuses, though this should be considered a temporary expedient only. The abnormalities which are amenable to this approach are the chromosome aberrations such as Down's syndrome, the neural-tube malformations including spina bifida cystica, and some recessively inherited and X-linked metabolic disorders. RF 001 STEELE MW LANCET 1 383 966 003 HUGHES DT LANCET 2 1319 971 004 LITTLEFIELD JW IN: MOTULSKY AG 221 974 005 MILUNSKY A PRENATAL DIAGNOSIS OF HEREDIT 973 006 NELSON MM IN: EMERY AEH 69 973 007 TURNBULL AC PROC R SOC MED 66 1115 973 008 BROCK DJH LANCET 2 197 972 009 WARD AM LANCET 2 345 974 010 WISNIEWSKI L BR MED J 3 742 974 011 MACRI JN LANCET 1 14 974 012 MACRI JN LANCET 1 1109 974 013 HARRIS R LANCET 1 429 974 014 ALLAN LD LANCET 2 522 973 015 SELLER MJ LANCET 2 73 973 017 LAURENCE KM LANCET 2 860 973 018 LAURENCE KM LANCET 1 301 974 020 NEVIN NC LANCET 1 1383 973 021 CARTER CO J MED GENET 5 81 968 022 CARTER CO LANCET 2 924 973 023 NADLER HL PEDIATRICS 42 912 968 024 ANON BIRTH DEFECTS GENETIC SERVICE 974 025 HARPER PS J MED GENET 11 123 974 026 FUJIMOTO WY LANCET 2 511 968 027 CAMPBELL S IN: MOTULSKY AG 249 974 028 GERBIE AB AM J OBSTET GYNECOL 109 765 971 029 WHITBY LG LANCET 2 819 974 030 PENROSE LS DOWNS ANOMALY 966 031 STEIN Z LANCET 1 305 973 032 KABACK MM IN: MOTULSKY AG 248 974 033 LORBER J LANCET 1 1187 973 034 BROCK DJH LANCET 2 923 973 035 BROCK DJH LANCET 1 767 974 036 SELLER MJ LANCET 1 428 974 037 WALD NJ LANCET 1 765 974 038 WALD NJ LANCET 1 907 974 CT 1 HOLLAND WW LANCET 2 1494 974 2 CANKI N ZDRAVSTVENI VESTNIK 54 269 985 PN 74119 RN 00118 AN 75024206 AU Shapiro-B-L. Smith-Q-T. Martinez-A. TI Letter: White-cell glutathione reductase in cystic fibrosis. SO Lancet. 1974 Oct 26. 2(7887). P 1020-1. MJ CYSTIC-FIBROSIS: bl. GLUTATHIONE-REDUCTASE: bl. LEUKOCYTES: en. MN BLOOD-PRESERVATION. BLOOD-SPECIMEN-COLLECTION. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en, fg. HETEROZYGOTE. HUMAN. TIME-FACTORS. EX We have reported that red-cell glutathione reductase (G.R.) activity is increased in cystic-fibrosis (C.F.) patients in comparison with age and sex matched controls. We have also demonstrated that serum-G.R. activity is significantly greater in C.F. than in controls, obligate heterozygotes, and patients with a variety of respiratory and immunological diseases. Moreover, serum-G.R. from C.F. patients and carriers responds differently from controls after storage or incubation with heparin. We have now examined G.R. in white blood-cells (W.B.C.) of C.F. patients. The day after collection of samples mean W.B.C.-G.R. activity was significantly less in the C.F. group than in carriers and controls. No differences in G.R. activity between the groups was apparent after 4 and 6 days. White-cell G.R. activity apparently is lower in C.F. patients than in controls. We cannot rationalise this decrease with the increased activity found in C.F. red cells and serum. Nevertheless, the differences between C.F. and controls are of greater interest than the apparent directional contradiction. The basis for quantatitive enzyme differences is very difficult to determine. The simplest explanation is that the initial white-cell G.R. differences between C.F. patients on the one hand and controls and carriers on the other are secondary to some aspect of the disease or its treatment; but there is no evidence to support such a conclusion. These results, which reflect altered behaviour of G.R. from C.F. patients, suggest to us that G.R. is involved in C.F. RF 001 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 002 SHAPIRO BL PEDIATR RES 9 885 975 003 ICEN A SCAND J CLIN LAB INVEST SUPPL 96 967 004 LOWRY OH J BIOL CHEM 193 265 951 CT 1 SHAPIRO BL PEDIATR RES 9 885 975 2 SHAPIRO BL PROC SOC EXP BIOL MED 149 592 975 3 DISANTAGNESE PA N ENGL J MED 295 534 976 4 CHANG JC ANN CLIN LAB SCI 8 23 977 5 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 PN 74120 RN 00119 AN 75012106 AU Moynahan-E-J. TI Letter: Skin wrinkling in cystic fibrosis. SO Lancet. 1974 Oct 12. 2(7885). P 907. MJ CYSTIC-FIBROSIS. SKIN. MN CYSTIC-FIBROSIS: me. HAND. HUMAN. HYDROGEN-ION-CONCENTRATION. IMMERSION. KERATIN. PROTEIN-BINDING. SKIN: me. SODIUM-CHLORIDE: an. SWEAT-GLANDS. SWEAT: an. EX The water-binding capacity of a protein is minimal at its isoelectric point (pH 3.5 - 5 for the keratins making up the horny layers) and increases with increasing divergence from the isoelectric point. It is for this reason that normal palmar skin wrinkles after moderate immersion in warm water, especially if it is soapy (pH 8 or more). However, in patients with cystic fibrosis it is the increased salt content of the sweat rather than the pH difference which increases the water-binding capacity of the keratin, and is responsible for the wrinkling in these patients. RF 000 ELLIOTT RB LANCET 2 108 974 CT 1 BULL C BR MED J 1 551 977 2 BRAHAM J ARCH NEUROL 36 113 979 3 CLARK CV DIABETES CARE 7 224 984 4 BRANDRUP F CONTACT DERMATITIS 10 62 984 PN 74121 RN 00120 AN 75032210 AU Raine-D-N. TI Inherited metabolic disease. SO Lancet. 1974 Oct 26. 2(7887). P 996-8. MJ MASS-SCREENING. METABOLISM-INBORN-ERRORS: di. MN ALBUMINS: an. AMINO-ACID-METABOLISM-INBORN-ERRORS: di. CHROMATOGRAPHY: mt. COMPARATIVE-STUDY. COSTS-AND-COST-ANALYSIS. CYSTIC-FIBROSIS: di, oc. DIAGNOSIS-LABORATORY: mt. EVALUATION-STUDIES. IRON: du. GALACTOSEMIA: di. GENETIC-COUNSELING. HUMAN. HYPERLIPIDEMIA: di. INFANT. INFANT-NEWBORN. MECONIUM: an. PHENYLALANINE: ur. PHENYLKETONURIA: di, oc, ur, bl. EX At present it is extremely doubtful if mass-population screening for any inherited metabolic disease other than phenylketonuria should be established beyond the few pilot studies at present in progress. For many diseases, although early detection is clearly desirable or the technical means of screening are available, even the establishment of pilot surveys is not yet indicated. Instead, methods of screening more limited populations at risk for particular disorders within a nationally coordinated administrative structure should be established. Those with experience of mass screening for more than one inherited metabolic disease must surely be convinced that in new programmes launched prematurely the harm caused by incomplete detection of cases or recognition of disorders whose management is uncertain or unavailable can far outweigh any benefits. RF 001 ANON BR MED J 4 7 968 002 WOOLF LI PHENYLKETONURIA AND ALLIED ME 50 967 003 GUTHRIE R PEDIATRICS 32 338 963 004 KOMROWER GM PROC R SOC MED 61 296 968 005 RAINE DN BR MED J 3 7 972 006 MCKEOWN T SCREENING IN MEDICAL CARE 968 007 WHITBY LG LANCET 2 819 974 008 RAINE DN ANN CLIN BIOCHEM 6 29 969 009 PAPPWORTH MH HUMAN GUINEA PIGS 48 969 010 FRANKLIN AW BR MED J 2 402 973 011 BESSMAN SP J PEDIATR 69 334 966 012 SMITH I LANCET 2 540 974 013 RAINE DN TREATMENT OF INHERITED ME 974 014 GUTHRIE R BIRTH DEF ORIG ART SER 4 92 968 016 SHWACHMAN H PEDIATRICS 32 85 963 017 HEROLD W MONATSSCHR KINDERHEILKD 117 626 969 018 GOLDBLOOM RB N ENGL J MED 269 1349 963 019 WARWICK WJ PEDIATRICS 36 261 965 020 PROSSER R ARCH DIS CHILD 49 597 974 021 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 022 GOLDSTEIN JL J CLIN INVEST 52 1533 973 023 WESTWOOD A IN: SENKINS WT 63 973 024 CARTER CO LANCET 1 281 971 025 KELLER DF G-6-PD DEFICIENCY 47 971 026 RAINE DN BR MED J 2 329 972 027 KABACK MM N ENGL J MED 289 1090 973 028 WHITTEN CF N ENGL J MED 288 318 973 CT 1 HOLLAND WW LANCET 2 1494 974 2 BENKE PJ PERSPECT BIOL MED 19 118 975 3 CRAMPTON RF BR MED BULL 31 209 975 4 BERRY HK FED PROC 34 2134 975 5 MELLMAN WJ ANN NY ACAD SCI 265 134 976 6 CHALMERS RA ANN CLIN BIOCHEM 14 149 977 PN 74122 RN 00121 AN 75012228 AU Danes-B-S. TI Letter: Uptake of 35S-heparin by lymphocytes from cystic-fibrosis patients. SO Lancet. 1974 Sep 14. 2(7881). P 655. MJ CYSTIC-FIBROSIS: me. HEPARIN: me. LYMPHOCYTES: me. MN CELLS-CULTURED. CYSTIC-FIBROSIS: bl. HEPARIN: bl. HUMAN. LEUKOCYTES: me. MUCOPOLYSACCHARIDES: bl, me. SULFUR-RADIOISOTOPES. EX In 1969 Danes and Bearn proposed that the metachromasia noted in short-term white-blood-cell cultures from both cystic fibrosis (C.F.) homozygotes and heterozygotes probably reflected uptake of heparin from the culture-medium by pinocytosis and not, as in the genetic mucopolysaccharidoses, stored mucopolysaccharides. This hypothesis was recently confirmed by Robertson et al. Using 35S-heparin they demonstrated an increased uptake of heparin in short-term C.F. white-blood-cell cultures which corresponded to the observations made on the basis of staining. However, as mentioned in 1971 by Bafalluy et al., any white blood-cell in culture can be overloaded with heparin. As the processes involved in endocytosis and exocytosis probably are inconstant within one cell and certainly in different cell types, standardisation of cytoplasmic retention of heparin may not be possible in order to eliminate the partial overlap between normals, C.F. homozygotes and obligatory heterozygotes observed by Robertson et al. This report on 35S-heparin again raised the question of the significance of cellular mucopolysaccharides in cystic fibrosis. The relevance of mucopolysaccharides in the C.F. cultured cell is still unclear. Between 1968 and 1970 the mucopolysaccharide content of the cultured fibroblast was reported as normal and variable. RF 001 DANES BS LANCET 2 437 969 002 ROBERTSON JA LANCET 1 1256 974 003 BAFALLUY E LANCET 1 973 971 004 WIESMANN UN J PEDIATR 77 685 970 005 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 006 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 007 CAUDILL M LANCET 1 32 974 CT 1 DANES BS TEX REP BIOL MED 34 135 976 PN 74123 RN 00122 AN 74102576 AU Conover-J-H. Conod-E-J. Hirschhorn-K. TI Studies on ciliary dyskinesia factor in cystic fibrosis. IV. Its possible identification as anaphylatoxin (C3a)-IgG complex. SO Life-Sci. 1974 Jan 16. 14(2). P 253-66. MJ COMPLEMENT. CYSTIC-FIBROSIS: me. IGG: me. MN AMINOCAPROIC-ACIDS. ANIMAL. BIOLOGICAL-ASSAY. BLOOD-COAGULATION-FACTORS. CARBOXYPEPTIDASES. CELL-LINE. DIPEPTIDES. FIBROBLASTS: me. HUMAN. ISOFLUROPHATE. LYMPHOCYTES: me. MACROMOLECULAR-SYSTEMS. OLIGOPEPTIDES. PANCREAS: en. PROTEIN-BINDING. RABBITS: im. SKIN: me. SWINE. TRACHEA. TRYPSIN. AB Presumptive evidence indicates that the substance responsible for the pathophysiologic conditions of cystic fibrosis (CF) is a complement component, C3a, also known as anaphylatoxin. The known biochemical and physiological properties of C3a, together with the behavior of this purified substance when associated with IgG in our tracheal bioassay, compare favorably with those of molecular species which we have separated from sera and cell cultures of CF homozygotes and heterozygotes. Sera from normal healthy subjects, previously inactive by bioassay for ciliary dyskinesia factor (CDF), were converted to a CDF positive state by incubation with epsilon-amino-caproic-acid (EACA). EACA is a known inhibitor of the carboxypeptidase-B-like anaphylatoxin inactivator, and is the method of choice for accumulating anaphylatoxins in normal blood. Incubation of EACA-treated normal sera and two fresh CDF-positive CF sera with carboxypeptidase-B produced reversion in all instances to a CDF negative, normal state. It is proposed that anaphylatoxin inactivator, a carboxypeptidase-B-like enzyme, is defective or deficientin cystic fibrosis and that this deficiency is the primary gene defect. RF 002 CONOVER JH CLIN RES 21 531 973 003 SPOCK A PEDIATR RES 1 173 967 004 CONOVER JH PEDIATR RES 7 220 973 005 CONOVER JH CF CLUB ABST 14 22 973 006 CONOVER JH PEDIATR RES 7 224 973 008 MCCOMBS ML CLIN GENET 1 171 970 009 CONOVER JH LANCET 1 1194 973 011 MULLER-EBERHARD HJ HARVEY LECTURES 66 75 971 012 BOKISCH VA J CLIN INVEST 49 2427 970 013 BOKISCH VA EXP MED 129 1109 969 014 STECHER V J IMMUNOL 99 660 967 015 VALLOTA EH J EXP MED 137 1109 973 017 GOLDSTEIN IM PROC AMER SOC CLIN INV 65 34A 973 018 COCHRANE CG J EXP MED 127 371 968 019 WARTON KL BR MED J 3 570 971 020 GOEBELL H GUT 13 477 972 021 LANDING BH ARCH PATHOL 88 569 969 022 MEYRICK B THORAX 24 729 969 023 DI SANTAGNESE PA PEDIATRICS 12 549 953 024 WARD PA J IMMUNOL 110 1003 973 CT 1 BERATIS NG PEDIATR RES 8 687 974 2 WILSON GB LIFE SCI 15 551 974 3 CONOVER JH LANCET 1 47 975 4 CONOD EJ PEDIATR RES 9 724 975 5 RAO GJS PEDIATR RES 9 739 975 6 WILSON GB PEDIATR RES 9 635 975 7 ALTLAND K HUM GENET 28 207 975 8 LIEBERMANN J AM REV RESPIR DIS 111 100 975 9 FORSTNER G CAN MED ASSOC J 113 550 975 10 LESEC G NOUV PRESSE MED 5 2470 976 11 WILSON GB PEDIATR RES 10 87 976 12 WEEKE B DAN MED BULL 23 155 976 13 HOLZHAUER RJ AM J HUM GENET 28 602 976 14 LEDERBERG S AM J HUM GENET 28 597 976 15 CONOVER JH TEX REP BIOL MED 34 45 976 16 DANES BS TEX REP BIOL MED 34 135 976 17 HARPER BL TEX REP BIOL MED 34 73 976 18 WILSON GB TEX REP BIOL MED 34 51 976 19 CZEGLEDYNAGY E LAB INVEST 35 588 976 20 CORBIN NC ANAL BIOCHEM 73 41 976 21 ANON J PEDIATR 88 711 976 22 WOOD RE AM REV RESPIR DIS 113 833 976 23 DISANTAGNESE PA N ENGL J MED 295 597 976 24 CALLAHAN JW PEDIATR RES 11 1166 977 25 CONOD EJ PEDIATR RES 11 45 977 26 WILSON GB PEDIATR RES 11 317 977 27 WILSON GB PEDIATR RES 11 143 977 28 SPITZER RE PEDIATR CLIN NORTH AM 24 341 977 29 BAKER AP AM REV RESPIR DIS 115 811 977 30 WILSON GB NATURE 266 463 977 31 ANON LANCET 2 1032 978 32 MARCHI AG HELV PAEDIATR ACTA 33 517 978 33 WILSON GB J LAB CLIN MED 92 463 978 34 GOTZ M EUR J PEDIATR 127 133 978 35 NEZELOF C PEDIATRICS 63 361 979 36 BUTTERWORTH J CLIN CHIM ACTA 97 39 979 37 JAKEL HP BIOL ZENTRALBL 98 55 979 38 BURDON MG CLIN GENET 17 249 980 39 HODSON ME THORAX 35 801 980 40 BOWMAN BH FED PROC 39 3195 980 41 WILSON GB J CLIN INVEST 66 1010 980 42 PLUMMER TH ANAL BIOCHEM 108 348 980 43 MOSS RB AM REV RESPIR DIS 121 23 980 44 SANDERSON MJ PEDIATR RES 15 219 981 45 DANDONA P THORAX 36 60 981 46 SCHWEISFURTH H DTSCH MED WSCHR 109 166 984 47 SCHWEISFURTH H DTSCH MED WSCHR 109 1254 984 48 BUYS CHCM CLIN CHIM ACTA 136 229 984 49 SCHWEISFURTH H CLIN BIOCHEM 18 242 985 50 SCHWEISFURTH H CLIN BIOCHEM 20 43 987 PN 74124 RN 00123 AN 74138418 AU Rawal-B-D. McKay-G. Blackhall-M-I. TI Inhibition of Pseudomonas aeruginosa by ascorbic acid acting singly and in combination with antimicrobials: in-vitro and in-vivo studies. SO Med-J-Aust. 1974 Feb 9. 1(6). P 169-74. MJ ANTIBIOTICS: pd. ASCORBIC-ACID: pd. PSEUDOMONAS-AERUGINOSA: de. MN ADOLESCENCE. ANIMAL. ASCORBIC-ACID: tu. CHILD. CYSTIC-FIBROSIS: co. DRUG-COMBINATIONS. DRUG-SYNERGISM. DRUG-THERAPY-COMBINATION. ERYTHROMYCIN: tu. FEMALE. HUMAN. MICE. MICROBIAL-SENSITIVITY-TESTS. PSEUDOMONAS-INFECTIONS: co, dt. SULFAMETHOXAZOLE: tu. TRIMETHOPRIM: tu. AB Ascorbic acid, acting on its own, is found to exert a bacteriostatic action in vitro on 16 strains of Pseudomonas aeruginosa tested. This action, attributable to the oxidation of ascorbic acid, is dependent on individual strains and the inoculum size. In combination with sulphamethoxazole, trimethoprim, Septrin, ampicillin, erythromycin, chloramphenicol or colistin sulphate, ascorbic acid acts synergistically on Pseudomonas aeruginosa in vitro. Experiments in mice with erythromycin-ascorbic acid combination show that the curvative dose 50 of ascorbic acid acting alone is 11.22 milligrammes per kilogram body weight. This was significantly lowered (P =< 0.001) to 6.91 mg/kg if administered in combination with erythromycin (10 mg/kg). In human patients with cystic fibrosis, by the combined administration of ascorbic acid sulphamethoxazole and trimethoprim (Bactrim, Roche) the pseudomonas infection could be controlled, as the viable number of organisms significantly diminished during the therapy. RF 001 BURNS JJ ANN NY ACAD SCI 151 959 968 002 CHABBERT Y QUOTED IN: HOCKENHULL DJD 180 961 003 EDDY BP BACT REV 17 93 953 004 ISHERWOOD FA BIOCHEM J 56 1 954 005 JESSEN O PSEUDOMONAS AERUGINOSA AND OT 965 006 LACEY RW IN: COWAN ST 247 958 007 LWOFF A QUOTED IN: BACT REV 17 93 953 008 MACCACARO GA IN: HOCKENHULL DJD 180 961 009 RAWAL BD SOUTHEAST ASIAN J TROP MED PU 3 225 972 010 REED LJ AM J HYG 27 493 938 011 SLADE HD J BACTERIOL 60 301 950 CT 1 WILSON CWM J CLIN PHARMACOL 15 570 975 2 WILSON CWM ANN NY ACAD SCI 258 355 975 3 WILSON CWM LANCET 1 586 976 4 DESTRO RL CLIN PEDIATR 16 936 977 5 HUMPHRIES AL AM SURGEON 43 259 977 6 RAWAL BD MICROBIOS LETTERS 7 127 978 7 RAWAL BD CHEMOTHERAPY 24 166 978 8 LEIBOVITZ B INT J VIT NUTR RES 48 159 978 9 HETEY SK AM J HOSP PHARM 37 235 980 10 CLARKE CW J ANTIMICROB CHEMOTHER 7 311 981 11 HANCOCK REW ANTIMICROB AGENTS CHEMOTHER 26 48 984 12 HANCOCK REW ANNU REV MICROBIOL 38 237 984 13 DRYBURGH DR J MANIP PHYSIOL THER 8 95 985 14 FAHIM MS ARCH ANDROL 14 81 985 15 HANCOCK REW J ANTIMICROB CHEMOTHER 18 653 986 PN 74125 RN 00124 AN 74064913 AU Quissell-D-O. Pitot-H-C. TI Number of ouabain-binding sites in fibroblasts from normal subjects and patients with cystic fibrosis. SO Nature. 1974 Jan 11. 247(436). P 115-6. MJ CYSTIC-FIBROSIS: me. FIBROBLASTS: me. OUABAIN: me. MN ADENOSINE-TRIPHOSPHATASE: me. BINDING-SITES. CELLS-CULTURED. CYSTIC-FIBROSIS: en. FIBROBLASTS: en. HUMAN. KINETICS. POTASSIUM: me. SKIN. SODIUM: me. TRITIUM. EØ Onå oæ thå characteristicó oæ cystiã fibrosis¬ aî autosomal recessive disease, is a defect in the net sodium transport in the eccrine glands resulting in an increased concentration of sodium in the sweat. Because of possible complications in the erythrocytes from CF patients, and since the activity observed in a cell homogenate may not express the situation in the intact cell, we have looked for differences in the binding of 3H-ouabain in cultured normal and CF human diploid fibroblasts. We observed no appreciable differences in the number of ouabain-binding sites between normal and CF cell strains. We were also unable to observe any differences in the kinetics of ouabain-binding between normal and CF fibroblasts in K+-free phosphate-buffered saline solution, and the results were essentially the same as those seen in HeLa cells. We observed no alterations in the kinetics of binding of ouabain nor any alteration in the total number of binding sites. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1344 967 002 MANGOS JA SCIENCE 158 135 967 003 MANGOS JA PEDIATR RES 1 436 967 004 SPOCK A PEDIATR RES 1 173 967 005 BOWMAN BH SCIENCE 167 871 970 006 BALFE JW SCIENCE 162 689 968 007 LAPEY A PEDIATR RES 4 478 970 008 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 009 FITZPATRICK DF NATURE NEW BIOL 235 173 972 010 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 011 BAKER PF BIOCHIM BIOPHYS ACTA 183 646 969 012 HOFFMAN JF GEN PHYSIOL 54 343 969 013 ELLORY JC NATURE 221 776 969 014 LAMB JF J PHYSIOL (LOND) 213 57P 971 015 BAKER PF J PHYSIOL (LOND) 227 855 972 016 BOARDMAN LJ J PHYSIOL (LOND) 225 619 972 017 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 018 FLETCHER DS CLIN CHIM ACTA 44 5 973 CT 1 SANN L LYON MED 232 709 974 2 QUISSELL DO NATURE 249 95 974 3 CHANGUS JE AM J PATHOL 80 317 975 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 DISANTAGNESE PA N ENGL J MED 295 534 976 8 YOKOYAMA M PEDIATR RES 11 765 977 9 EPSTEIN J PROC NAT ACAD SCI USA 74 1676 977 10 BRESLOW JL EXP CELL RES 110 399 977 11 KATZ S RES COMMUN CHEM PATH PHARM 19 491 978 12 JAKEL HP BIOL ZENTRALBL 98 55 979 13 SPICER SS EXP MOL PATH 33 104 980 14 KATZ S CLIN CHIM ACTA 100 245 980 15 BANCHINI G PEDIATR RES 15 1073 981 16 REZNIK VM PROC NAT ACAD SCI USA 78 7143 981 17 VONEULER AM LIFE SCI 37 2399 985 PN 74126 RN 00125 AN 74168901 AU Duffy-M-J. TI Letter: Na+ transport defect in cystic fibrosis. SO Nature. 1974 May 3. 249(452). P 94-5. MJ CYSTIC-FIBROSIS: me. SODIUM: me. MN ADENOSINE-TRIPHOSPHATASE: me. BIOLOGICAL-TRANSPORT: de. CYSTIC-FIBROSIS: en. ERYTHROCYTES: me. ETHACRYNIC-ACID: pd. FIBROBLASTS: me. HUMAN. OUABAIN. TRITIUM. EX Using the technique of 3H-ouabain binding to isolate cells Quissell and Pitot concluded that the Na+-K+ ATPase functions normally in fibroblasts from cystic fibrosis patients. However this finding may not be very relevant to the Na+ transport defect occurring in this genetic disease. The Na+ transport abnormality in cystic fibrosis seems to be confined to exocrine glands. It is unknown at present whether the defect resides in an active or passive transport step of the cation. At least with isolated sweat gland tissue and erythrocytes from cystic fibrosis patients, Na+-K+ ATPase activity has been found to be normal. However a decrease in the ethacrynic acid-sensitive efflux, a Na+ exchange mechanism, has been reported in cystic fibrosis erythrocytes. These findings suggest that there is no fundamental abnormality in the Na+-K+ ATPase enzyme in the disease, but there may be a defect in an ethacrynic acid-sensitive transport step. Therefore in order to obtain a better understanding of the cation transport defect one should measure the binding of labelled ethacrynic-acid rather than ouabain. RF 001 QUISSELL DO NATURE 247 115 974 002 DI SANTAGNESE PA N ENGL J MED 277 1344 967 003 GIBBS GE PEDIATR RES 1 24 967 004 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 005 DUNN MJ J CLIN INVEST 49 1815 970 006 BALFE JW SCIENCE 162 689 968 007 LAPEY A PEDIATR RES 5 446 971 008 DUFFY MJ LANCET 2 136 972 009 DUFFY MJ CLIN CHIM ACTA 49 397 973 010 ORLOFF J AM J MED 42 757 967 011 WEISMAN UN LANCET 1 510 972 012 WIESMANN UN J PEDIATR 76 444 970 013 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 014 EPSTEIN RW BIOCHIM BIOPHYS ACTA 274 119 972 015 SATO K PFLUEGERS ARCH 341 233 973 016 EPSTEIN RW BIOCHIM BIOPHYS ACTA 274 119 972 017 SIMOPOULOS AP PEDIATR RES 5 626 971 CT 1 BOUCHER RC PEDIATR RES 18 1336 984 PN 74127 RN 00126 AN 75045312 AU Rosenlund-M-L. Kim-H-K. Kritchevsky-D. TI Essential fatty acids in cystic fibrosis. SO Nature. 1974 Oct 25. 251(5477). P 719. MJ CYSTIC-FIBROSIS: bl. FATTY-ACIDS-ESSENTIAL: bl. MN CHILD. CHILD-PRESCHOOL. CHROMATOGRAPHY-THIN-LAYER. CYSTIC-FIBROSIS: me. FATTY-ACIDS-ESSENTIAL: df. HUMAN. EX The nature of the metabolic defect in cystic fibrosis (CF) has, to date, not been elucidated. Elliott reported that intravenous administration of soybean oil and lecithin to children suffering from CF caused their sweat sodium concentration to decrease towards normal values. This prompted us to investigate the fatty acid spectra of the various serum lipid classes of children with CF. We have found that affected children are deficient in essential fatty acids. The data suggest another approach to the study of the metabolic defect in cystic fibrosis. There is an apparent deficiency in essential fatty acids, and a marked reduction in serum vitamin E levels has also been reported in CF. These conditions might be partially corrected by intravenous injection, or possibly feeding of, lipids containing essential fatty acids with concomitant administration of vitamin E. The observed deficiency in essential fatty acids may be produced by a reduced ability to absorb these acids from the diet and this, in turn, may result in defects in membrane structure or stability. Reduced levels of vitamin E in CF may reflect a reduced requirement for this vitamin because of the lower levels of essential fatty acids. It is also possible that the observed deficiency in serum levels of essential fatty acids may lead to less than normal production of prostaglandins. RF 001 ELLIOTT RB AUST PAEDIATR J 8 217 972 002 FOLCH J J BIOL CHEM 226 497 957 003 KRITCHEVSKY D CLIN CHIM ACTA 46 63 973 004 KRITCHEVSKY D ARCH BIOCHEM BIOPHYS 35 346 952 005 KUO PT J CLIN INVEST 44 1924 965 006 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 007 BENNETT MJ AM J CLIN NUTR 20 415 967 CT 1 EMERY AEH LANCET 2 80 975 2 MCEVOY FA LANCET 2 236 975 3 RIVERS JPW LANCET 2 642 975 4 ROBINSON PG LANCET 2 919 975 5 LIN SN J CHROMATOGR 112 465 975 6 LIN SN J CHROMATOGR 112 483 975 7 CAMPBELL IM PEDIATRICS 57 480 976 8 WIDHALM K WIEN KLIN WOCHENSCHR 88 557 976 9 WOOD RE AM REV RESPIR DIS 113 833 976 10 SANJURJO P LANCET 1 752 977 11 HUBBARD VS LANCET 2 1302 977 12 MALMENDIER CL HELV PAEDIATR ACTA 32 115 977 13 ROSENLUND ML PEDIATRICS 59 428 977 14 SAHU S AM REV RESPIR DIS 115 233 977 15 MEADE CJ ADV LIPID RES 16 127 978 16 VAUGHAN WJ SCIENCE 199 783 978 17 BERG U MONOGR PAEDIATR 10 1 979 18 GALEY WR PEDIATR RES 14 1269 980 19 ROGIERS V PEDIATR RES 14 1088 980 20 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 21 COATES AL ACTA PAEDIATR SCAND 69 353 980 22 HOLMAN RT AM J CLIN NUTR 34 1534 981 23 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 24 SOLOMONS NW AM J CLIN NUTR 34 462 981 25 RIVERS JPW BR MED BULL 37 59 981 26 HOLMAN RT CHEM IND 1981 704 981 27 HARPER TB AM REV RESPIR DIS 126 540 982 28 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 29 ROGIERS V EUR J PEDIATR 141 39 983 30 PARNHAM MJ AGENTS ACTIONS 14 223 984 31 HOLMAN RT J AM COLL NUTR 5 183 986 32 MISCHLER EH PEDIATR RES 20 36 986 33 DUHAMEL JF ARCH FR PEDIATR 43 229 986 34 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 PN 74128 RN 00127 AN 75006446 AU Rodgers-B. Ferholt-J. Cooper-C-L. TI A screening tool to detect psychosocial adjustment of children with cystic fibrosis. SO Nurs-Res. 1974 Sep-Oct. 23(5). P 420-5. MJ CHILD-BEHAVIOR-DISORDERS: di. CYSTIC-FIBROSIS: pp. SOCIAL-ADJUSTMENT. MN ADAPTATION-PSYCHOLOGICAL. CHILD-BEHAVIOR-DISORDERS: et. CHILD. CYSTIC-FIBROSIS: co. DEMOGRAPHY. HUMAN. INTERVIEW-PSYCHOLOGICAL. NURSE-PRACTITIONERS: ut. PARENTS. PEDIATRIC-NURSING. QUESTIONNAIRES. SCHOOL-HEALTH-SERVICES. SOCIAL-WORK. SOCIOECONOMIC-FACTORS. TEACHING. AB A tool to assess psychosocial adjustment of school-age children with cystic fibrosis was developed using three instruments: a standardized open-ended parent interview, a self-administered teacher questionnaire, and a self-administered parent demographic data form. The three instruments enabled the pediatric nurse practitioner to make a clinical assessment of the child's psychosocial adjustment. This assessment was then checked for validity by means of a social worker interview. Implications for further research are discussed. RF 001 ALLEN CM PEDIATR CLIN NORTH AM 18 169 971 002 GLIDEWELL JC HUM ORGANIZATION 18 123 959 003 GRAHAM P BR J PSYCHIATRY 114 581 968 004 KAHN RL DYNAMICS OF INTERVIEWING 957 005 LEVI L STRESS SOURCES MANAGEMENT AND 967 006 MCCOLLUM AT J PEDIATR 77 571 970 007 MEYERS A PEDIATRICS 51 22 973 008 MUTTER AZ PSYCHOSOM MED 28 333 966 009 PLESS IB J PEDIATR 79 351 971 010 PRUGH DG IN: SOLNIT AJ 246 963 011 ROMANO J JAMA 143 409 950 012 RUTTER M EDUCATION HEALTH BEHAVIOR 970 013 TEICHER JD CALIF MED 110 371 969 014 TROPAUER A AM J DIS CHILD 119 424 970 015 YARROW LJ IN: MUSSEN PH 561 960 CT 1 FALKMAN C ACTA PAEDIATR SCAND SUPPL 264 1977 7 977 PN 74129 RN 00128 AN 74259671 AU Pinney-M. TI Home care for CF patients. SO Nursing (Jenkintown). 1974 Jun. 4(6). P 70-1. MN CYSTIC-FIBROSIS: nu. HOME-NURSING. EX Nearly all agree that home therapy, which permits cystic fibrosis children to lead nearly normal lives, is desirable. Between hospital visits, it's also essential for warding off the primary threats of CF: pulmonary insufficiency and cor pulmonale. Treatment is palliative, focusing on supplying enzymes that the body can't secrete and, when the lungs are involved, clearing airway obstructions. The first step in therapy is starting the child on supplemental enzymes, which will enable him to absorb fat and protein. Usually we encourage a normal diet. If X-rays, pulmonary function tests, or clinical observation shows lung involvement, we initiate pulmonary therapy and teach parents how to perform it at home. Postural drainage is continued indefinitely, even if the lungs clear. If the child shows clinical signs of infection, we instruct the parents to increase the number of active mist and postural drainage treatments and to administer oral antibiotics in high doses. Above all, we instruct the parents of CF children to treat them as normally as possible. PN 74130 RN 00129 AN 74284675 TI Bile acid malabsorption in cystic fibrosis. SO Nutr-Rev. 1974 Aug. 32(8). P 232-4. (REVIEW). MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: co. MALABSORPTION-SYNDROMES: co. MN REVIEW. INFANT. CHILD-PRESCHOOL. CHILD. HUMAN. CYSTIC-FIBROSIS: me. MALABSORPTION-SYNDROMES: et. LIPIDS: me. FECES: an. CELIAC-DISEASE: me, co. PANCREATIC-DISEASES: co. CHRONIC-DISEASE. CELIAC-DISEASE: co. INTESTINAL-MUCOSA: pp. ILEUM: pp. AB Malabsorption of bile acids occurs in cystic fibrosis but not in celiac disease and is correlated with fecal excretion. Bile acid malabsorption may occur secondary to exocrine pancreatic deficiency. RF 001 BERGSTROM S FED PROC SUPPL 21 11 28 962 002 BORGSTROM B J CLIN INVEST 36 1521 957 003 TYOR MP AM J MED 51 614 971 004 MCLEOD GM LANCET 1 873 968 005 HOFMANN AF GASTROENTEROLOGY 52 752 967 006 WEBER AM CLIN CHIM ACTA 39 524 972 007 WEBER AM N ENGL J MED 289 1001 973 008 DAVIDSON M J PEDIATR 69 1027 966 009 THOMAIDIS TS J PEDIATR 63 444 963 010 FREYE HB J PEDIATR 64 575 964 011 WILSON FA GASTROENTEROLOGY 61 911 971 012 BERNSTEIN L AM J CLIN NUTR 26 340 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 FONDACARO JD PEDIATR RES 16 494 982 3 FONDACARO JD LIFE SCI 32 1449 983 PN 74131 RN 00130 AN 75064554 AU Heffer-E-T. TI Cystic fibrosis in black population. SO NY-State-J-Med. 1974 Dec. 74(13). P 2355-7. MJ CYSTIC-FIBROSIS: oc. BLACKS. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. INFANT. MALE. NEW-YORK-CITY. EX In this study, we will report on a high number of black cystic fibrosis patients in Brooklyn, New York, with a description of these patients and the population of the area in which they reside. A total of 70 cases of cystic fibrosis were followed at the Long Island College Hospital. Ten, 14.2 per cent, of the 70 cases of cystic fibrosis are black. These are equally divided into 5 females and 5 males. RF 001 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 002 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 003 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 005 KULCZYCKI LL CLIN PEDIATR 3 692 964 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 ANON STATISTICAL ABST OF THE US 23 972 008 ANON VITAL STATISTICS OF THE US 51 971 CT 1 LOBER CW JAMA 235 1140 976 2 KULCZYCKI LL J PEDIATR 92 855 978 3 PORTER RC J PEDIATR 94 239 979 PN 74132 RN 00131 AN 74306577 AU Robinson-P-G. Elliott-R-B. TI Rapid semiquantitative tests for trypsin and chymotrypsin application to screening for cystic fibrosis, enterokinase deficiency and pancreatic hypoplasia in neonates. SO NZ-Med-J. 1974 Jun 12. 79(517). P 1024-6. MJ TRYPSIN: an. CHYMOTRYPSIN: an. ENZYME-TESTS. CYSTIC-FIBROSIS: di. METABOLISM-INBORN-ERRORS: di. PEPTIDE-PEPTIDOHYDROLASES: me. PANCREAS: ab. MN INFANT-NEWBORN. HUMAN. FECES: en. INDICATORS-AND-REAGENTS. COSTS-AND-COST-ANALYSIS. MASS-SCREENING. FALSE-NEGATIVE-REACTIONS. INFANT-PREMATURE. AB Methods for the rapid semiquantitative analyses of trypsin and chymotrypsin in faeces samples have been developed. The faecal sample is shaken with pH 8.2 buffer and solutions containing enzyme substrate, buffer and indicator are added. The sample is incubated at 37 degrees C for a period dependent on the amount of faecal material present and the colour noted. A change in colour from violet to yellow (trypsin) or green to rose (chymotrypsin) indicates the presence of the enzyme. A screening trial using these tests is being carried out at St. Helen's Maternity Hospital in Auckland. RF 001 BARBERO GJ AM J DIS CHILD 112 536 966 002 ELLIOTT RB AUST PAEDIATR J 8 217 972 003 KEMPE CH CURRENT PEDIATRIC DIAGNOSIS A 429 972 004 KOPITO L PEDIATRICS 43 794 969 005 SHWACHMAN H PEDIATRICS 4 222 949 006 SHWACHMAN H PEDIATRICS 46 335 970 CT 1 ROBINSON PG LANCET 2 817 975 2 ROBINSON PG CLIN CHIM ACTA 62 225 975 3 ROBINSON PG ARCH DIS CHILD 51 301 976 4 BARNES GL NZ MED J 83 349 976 5 ROBINSON PG NZ MED J 83 268 976 6 CROSSLEY JR LANCET 2 1093 977 PN 74133 RN 00132 AN 74127338 TI Cystic fibrosis. SO NZ-Nurs-J. 1974 Jan. 67(1). P 12-4. MN CYSTIC-FIBROSIS: nu. PATIENTS: ed. SPECIAL-LIST-NURSING. PATIENT-EDUCATION. EX Cystic fibrosis is an inborn disease which affects chiefly the lungs and the digestive system. Today cystic fibrosis is known to be one of the commonest genetic disorders affecting children in Britain. The children who actually develop cystic fibrosis have inherited the disease from both parents, who are carriers but are unaffected by the disease themselves. Being thick and sticky, instead of thin and slimy as it normally is, the mucus of the cystic fibrosis case tends to block the passages instead of clearing them. The main problem is in the lungs. Symptoms, diagnosis, care of nutrition, prevention of infection, respiratory education, and future research are discussed. PN 74134 RN 00133 AN 75138680 AU Gracey-M. TI The use of medium-chain triglyceride in children with intestinal malabsorption. SO Paediatr-Indones. 1974 Jul-Aug. 14(7-8). P 113-7. (REVIEW). MJ INTESTINAL-ABSORPTION: de. MALABSORPTION-SYNDROMES: dh. TRIGLYCERIDES: tu. MN ABNORMALITIES: dh. BILE-DUCTS: ab. CHILD. CYSTIC-FIBROSIS: dh. FECES: an. HEPATITIS: co. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: dh. INTESTINE-SMALL: me. LIPIDS: an. LYMPHATIC-DISEASES: dh. MALABSORPTION-SYNDROMES: et. REVIEW. TRIGLYCERIDES: pd, me. EX Medium-chain triglycerides (MCT) are fats in which the chain length is six to twelve carbon atoms. This is in contrast to the more usual dietary sources of fats, long-chain triglycerides (LCT), which contain more than 14 carbon atoms in their atomic chain. The differences in chemical structure between LCT and MCT are important because they are related to major differences in the characteristics of absorption and metabolism of these two classes of lipids. These physiological differences between LCT and MCT have led to the use of MCT in various disorders where malabsorption of LCT occurs but where MCT can be absorbed because they bypass several of the steps necessary for the absorption of long chain fats. These include pancreatic disease, chronic liver disease, disorders of the biliary tract and diseases of the small intestinal epithelium and lymphatic vessels. The comments which follow are a brief summary of the therapeutic place of MCT in children with malabsorption. This will be followed by some remarks about the potential value of MCT in countries like Indonesia where the commonest cause of chronic diarrhoea and malabsorption in children is malnutrition and chronic and repeated gastrointestinal infections and infestations and where MCT has not been used widely. RF 001 BLOOM B AM J PHYSIOL 166 451 951 002 BURKE V BR MED J 2 1050 966 003$ BURKE V AUST PAEDIATR J 2 114 967 004 GRACEY M ARCH DIS CHILD 44 401 969 005 GRACEY M ARCH DIS CHILD 45 445 970 006 GRACEY M AUST PAEDIATR J 4 66 968 008 GRAHAM GG AM J DIS CHILD 126 330 973 009 HOLT PR GASTROENTEROLOGY 53 961 967 010 ISSELBACHER KJ GASTROENTEROLOGY 50 78 966 011 SCHEIG R IN: SENIOR JR 39 968 012 SENIOR JR AM J MED SCI 257 75 969 013 SUTEDJO R PAEDIATR INDONES 14 73 974 014 TAMIR I ARCH DIS CHILD 43 302 968 PN 74135 RN 00134 AN 75046081 AU Gall-D-G. Hamilton-J-R. TI Chronic diarrhea in childhood: a new look at an old problem. SO Pediatr-Clin-North-Am. 1974 Nov. 21(4). P 1001-17. (REVIEW). MJ DIARRHEA. MN CELIAC-DISEASE: co. CHLORIDES: me. CHRONIC-DISEASE. CYSTIC-FIBROSIS: co. DIARRHEA: et, pp, su, dt, th, di. DIET-THERAPY. FOOD-HYPERSENSITIVITY: co. GLUCOSE: me. HUMAN. IMMUNOSUPPRESSION. INFECTION: co. INFLAMMATION: co. INTESTINAL-DISEASES: co, dt. INTESTINAL-OBSTRUCTION: co. INTESTINE-SMALL: su. LACTOSE-INTOLERANCE: co. METABOLISM-INBORN-ERRORS: co. NEOPLASMS: co. PANCREATIC-DISEASES: co. POSTOPERATIVE-COMPLICATIONS. PREDNISONE: tu. REVIEW. SALICYLAZOSULFAPYRIDINE: tu. SODIUM: me. STEROIDS: tu. AB Diarrhea can be defined as excess loss of water and electrolytes in feces, but what is an excess? To the physician, fecal losses may be excessive when they cause the patient to be ill; to a parent a stool pattern that differs from that of a neighbor's infant or even a single watery stool may be too much. New knowledge of normal gut function and the pathologic processes that may afflict the child's alimentary tract has improved one capability to diagnose and manage certain specific diseases that cause diarrhea. Although of great interest to academicians, many of these diseases are extraordinarily rare and in most practices there is a substantial and perplexing pool of poopers in whom no specific disease can be found. Fortunately, patients in this latter group are rarely seriously ill. This article attempts to help the practitioner understand, diagnose and treat children with specific diseases known to cause chronic diarrhea. We will speculate also on the mechanisms and management of idiopathic chronic diarrhea. RF 001 AMENT ME J PEDIATR 81 685 972 002 AMENT ME J PEDIATR 81 867 972 003 AMENT ME GASTROENTEROLOGY 62 216 972 004 BISHOP RF LANCET 2 1281 973 005 BOOK LS PEDIATR RES ABST 8 379 974 006 BORTOLUSSI R PEDIATR RES ABST 8 379 974 007 BRANDBORG LL GASTROENTEROLOGY 52 143 967 008 BROBERGER O ADV PEDIATR 14 9 966 009 BROOKE BN CLIN GASTROENTEROL 1 261 972 010 BUTLER DG J CLIN INVEST 53 1335 974 011 DAS KM GUT 14 631 973 012 DELORME J CAN MED ASSOC J 110 281 974 013 DEVROEDE GJ N ENGL J MED 285 17 971 014 DISSANAYAKE AS GUT 14 923 973 015 DONALDSON RM JR FED PROC 26 1426 967 016 DONALDSON RM JR IN: CODE CF 5 2807 968 017 EDWARDS FC GUT 4 299 963 018 FUBARA ES J IMMUNOL 111 395 973 019 GELFAND EW PEDIATR CLIN NORTH AM 21 745 974 020 GOLDMAN AS PEDIATRICS 32 425 963 021 GORBACH SL N ENGL J MED 287 791 972 022 GRACEY M ARCH DIS CHILD 48 331 973 023 GRAY GM GASTROENTEROLOGY 58 96 970 024 GUTMAN LT N ENGL J MED 288 1372 973 025 HADORN B CLIN GASTROENTEROL 1 125 972 026 HAMILTON JR IN: SLEISENGER MH 280 973 027 HAMILTON JR Q J MED 38 135 969 028 HAMILTON JR J PEDIATR 81 885 972 029 HAMILTON JR GASTROENTEROLOGY 56 124 969 030 HEIRD WC J PEDIATR 80 351 972 031 HENSON D ARCH PATHOL 93 477 972 032 HOSKINS LC GASTROENTEROLOGY 53 265 967 033 HUANG SS N ENGL J MED 276 1283 967 034 JEFFRIES GH GASTROENTEROLOGY 56 777 969 035 KAMATH KR GASTROENTEROLOGY 66 16 974 036 LAFLEUR L UNION MED CAN 101 2407 972 037 LENNARD-JONES JE LANCET 1 185 965 038 MCCOMBS ML TEX REP BIOL MED 31 615 973 039 MILLER RA ARCH DIS CHILD 16 22 941 040 MOORE GT N ENGL J MED 281 402 969 041 ORMISTON G BR MED J 2 151 942 042 PHILLIPS SF GASTROENTEROLOGY 63 495 972 043 POLEY JR SOUTH MED J 66 1035 973 044 POLEY JR SOUTH MED J 66 1133 973 045 RYAN JA N ENGL J MED 290 757 974 046 SAUNDERS SJ GASTROENTEROLOGY 50 586 966 047 SCHULTZ SG GASTROENTEROLOGY 63 161 972 048 SHWACHMAN H IN: SLEISENGER MH 1206 973 049 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 050 TRUELOVE SC BR MED J 2 1072 958 051 WEI P CAN MED ASSOC J 106 969 972 052 WILMORE DW J PEDIATR 80 88 972 CT 1 BRUETON MJ ARCH DIS CHILD 51 989 976 2 GALL DG CLIN GASTROENTEROL 6 431 977 3 BONZANIGO C MONATSSCHR KINDERHEILKD 127 125 979 4 LO CW PEDIATRICS 72 786 983 5 SHARMA RK INDIAN J MED RES 78 713 983 6 GEORGE DE AM FAM PHYSICIAN 29 280 984 7 HAMILTON JR PEDIATR CLIN NORTH AM 32 419 985 PN 74136 RN 00135 AN 75046094 AU Crozier-D-N. TI Cystic fibrosis: a not-so-fatal disease. SO Pediatr-Clin-North-Am. 1974 Nov. 21(4). P 935-50. (REVIEW). MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, mo, co. DIET-THERAPY. DIETARY-FATS: ad. DRUG-THERAPY-COMBINATION. FEMALE. GENTAMICINS: tu. HUMAN. INFANT. INFANT-NEWBORN. INFECTION: dt, et. INTESTINAL-ATRESIA: co. INTESTINAL-OBSTRUCTION: co. LUNG-DISEASES: dt, et. MALE. NASAL-POLYPS: co. NUTRITION. PANCREATIC-DISEASES: co. PENICILLINS: tu. PROGNOSIS. RECTAL-PROLAPSE: pc. REVIEW. SINUSITIS: co. INFERTILITY-MALE: et. VITAMINS. EX Cystic fibrosis is an intricate, vexatious disease involving the whole patient. As yet we are only able to treat the signs and symptoms, and this should be done as completely as possible. Treatment is geared at eradicating the Staphylococcus aureus from the lung and preventing reinfection. Gram-negative organisms, particularly Pseudomonas aeruginosa, are treated when they become pathogenic. Other conditions such as atopia of the respiratory tract or gastrointestinal system may be detrimental to the patient's cystic fibrosis condition and must be controlled. Pancreatic insufficiency requires adequate enzyme replacement therapy to prevent steatorrhea and azotorrhea. If fatty acids play a role in membrane oxygen transport then their abnormal composition in cystic fibrosis may require specific changes in the type of fat intake. Salt depletion should be looked for and corrected. A screening test for cystic fibrosis is needed, preferably one done on a small blood specimen. RF 001 ANDRIOLE VT J INFECT DIS 129 124 974 002 BELMONTE MM MOD PROBL PEDIATR 10 388 967 003 BOWMAN BH SCIENCE 167 871 970 005 CHANG N AM REV RESPIR DIS 107 672 973 006 CONOVER JH LANCET 1 1194 973 007 DENNING CR PEDIATRICS 41 7 968 008 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 009 DI SANTAGNESE PA N ENGL J MED 277 1287 967 010 DI SANTAGNESE PA ANN INTERN MED 50 1321 959 011 DOGGETT RG NATURE NEW BIOL 243 251 973 012 DYCK WP AM J DIG DIS 12 310 967 013 HOLMAN RL PEDIATRICS 24 34 959 014 KUO PT J PEDIATR 60 394 962 015 LAPEY A J PEDIATR 84 328 974 016 LAWSON D ARCH DIS CHILD 47 1 972 017 LAWSON D IN: LAWSON D PROC 5TH INT CF 225 969 018 LAWSON D BR MED J 1 317 965 019 MANGOS JA SCIENCE 158 135 967 020 MATTHEWS LW IN: MANGOS JA 303 973 021 MAUER AM AM J DIS CHILD 92 160 956 022 MCCLURE PD LANCET 2 1307 970 023 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 024 RENNERT OM IN: MANGOS JA 41 973 025 SHWACHMAN H PEDIATRICS 30 389 962 026 SPOCK A PEDIATR RES 1 173 967 027 SPROUL A J PEDIATR 65 664 964 028 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 029 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 030 WARWICK WJ CANADIAN CF FOUND REPORT 973 CT 1 FORSTNER G CAN MED ASSOC J 113 550 975 2 MUNZENBERGER PJ J AM PHARMACEUT ASSOC 16 560 976 3 HAWKINS E AM J CLIN PATHOL 66 710 976 4 STERN RC J PEDIATR 89 406 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 COREY M AM REV RESPIR DIS 114 1085 976 7 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 8 SCOTT J AM J MED 63 488 977 9 STERN RC ANN INTERN MED 87 188 977 10 BUREAU MA PEDIATRICS 61 842 978 11 FLOWER KA BR MED J 2 630 979 12 ADAM D INFECTION 7 577 979 13 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 14 SHEPHERD R J PEDIATR 97 351 980 15 DUFFY MJ CLIN CHIM ACTA 103 233 980 16 PSACHAROPOULOS HT LANCET 2 78 981 17 SOLOMONS NW AM J CLIN NUTR 34 462 981 18 BELL L J CAN DIET ASSOC 42 62 981 19 DENTON JR CLIN ORTHOP 1981 71 981 20 HENRY RL AUST PAEDIATR J 18 43 982 21 MITCHELL EA AUST PAEDIATR J 18 114 982 22 PENCHARZ PB J PEDIATR GASTROENTEROL NUTR 2 400 983 23 CAMPBELL IM J CLIN MICROBIOL 18 408 983 24 WELLS AL J FOOD NUTR 41 65 984 25 HODGES P J AM DIET ASSOC 84 664 984 26 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 27 CAMPBELL IM EUR J CLIN MICROBIOL 5 622 986 28 SOUTTER VL CLIN GASTROENTEROL 15 137 986 29 LEVY L J PEDIATR 108 1038 986 30 DANIELS L J PEDIATR GASTROENTEROL NUTR 6 381 987 PN 74137 RN 00136 AN 75046084 AU Wolfish-M-G. McLean-J-A. TI Chronic illness in adolescents. SO Pediatr-Clin-North-Am. 1974 Nov. 21(4). P 1043-9. MJ CHRONIC-DISEASE. MN ADAPTATION-PSYCHOLOGICAL. ADOLESCENCE. AFFECTIVE-SYMPTOMS: et. ASTHMA: co. CHRONIC-DISEASE: co. COLITIS-ULCERATIVE: co. CYSTIC-FIBROSIS: co. DIABETES-MELLITUS: co. EDUCATION-SPECIAL. STRESS-PSYCHOLOGICAL. HEART-DISEASES: co. HOSPITALIZATION. HUMAN. KIDNEY-DISEASES: co. PARENT-CHILD-RELATIONS. PATIENTS: ed. SELF-CONCEPT. SIBLING-RELATIONS. PATIENT-EDUCATION. EX Chronic illness at any age is unfortunate. It taxes the strengths and resources of the individual and those associated with him. The chronically ill teenager faces difficulties different from those of the child or adult. Illness itself is a burden to the adolescent; chronic illness is an even greater burden. The tasks of maturation which must be successfully completed in adolescence are made more difficult by chronic ill health. Impact upon the patient, educational needs, sexuality needs, life expectancy, impact upon the family, and impact upon society are examined. Specific illnesses, such as diabetes mellitus and fibrocystic disease, are discussed. RF 001 ANON PUBLIC HEALTH SERV PUB 1000 968 002 DUBO S AM J ORTHOPSYCHIATRY 20 520 950 003 ANON BR MED J 1 134 969 004 HOLDAWAY D CLIN PEDIATR 11 63 972 005 KOOP CE PEDIATR CLIN NORTH AM 16 555 969 006 MADDISON D MED J AUST 2 1265 971 007 MATTSSON A PEDIATRICS 50 801 972 008 PLESS IB J PEDIATR 79 351 971 009 SHULMAN JL MANAGEMENT OF EMOTIONAL DISOR 967 010 WRIGHT L POSTGRAD MED 47 173 970 CT 1 LI FP ANN INTERN MED 84 551 976 2 MARKY I ACTA PAEDIATR SCAND SUPPL 1982 1 982 PN 74138 RN 00137 AN 75083116 AU Taylor-A. Mayo-J-W. Boat-T-F. Matthews-L-W. TI Standardized assay for the sodium reabsorption inhibitory effect and studies of its salivary gland distribution in patients with cystic fibrosis. SO Pediatr-Res. 1974 Nov. 8(11). P 861-5. MJ CYSTIC-FIBROSIS: me. SALIVARY-GLANDS: me. SODIUM: me. MN ADOLESCENCE. ADULT. ANIMAL. BIOLOGICAL-ASSAY: mt. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. HUMAN. PAROTID-GLAND: me. PERFUSION. PROTEINS: an. RATS. SALIVA: an. SODIUM: ai. SUBLINGUAL-GLAND: me. SUBMANDIBULAR-GLAND: me. AB The retrograde perfusion assay of Magos for the sodium reabsorption inhibitory effect in sweat and mixed saliva from patients with cystic fibrosis (CF) was modified by (1) using the second parotid gland of the rat as a control, (2) carefully controlling the quantity of saliva perfused into the rat parotid gland so that the ratio of milliliters perfused to gram dry gland weight fell between 1.8 and 2.8, and (3) reporting the inhibitory effect as percentage of inhibition of the rate of sodium reabsorption (RNa) in the perfused gland calculated from the difference between the RNa's in the two glands. Using this modified assay we were able to confirm that significant differences exist in the percentage of inhibition of RNa caused by CF and normal mixed saliva (47.0 plus or minus 24.8 and 18.0 plus or minus 8.3, mean plus or minus SD). In addition, similar significant differences in the percentage of inhibition of RNa by submandibular gland secretions (39.7 plus or minus 13.5 and 16.0 plus or minus 11.8), sublingual gland secretions (39.5 plus or minus 6.0 and 19.7 plus or minus 14.3), and submucosal gland secretions (34.0 plus or minus 11.7 and 21.7 plus or minus 12.7) between CF and normal subjects were observed. However, parotid gland secretions from CF subjects showed no increased inhibitory effect (12.5 plus or minus 7.9 and 12.5 plus or minus 6.6). These findings demonstrate that the sodium reabsorption inhibitory effect is produced by the secretions from some but not all salivary glands of patients with CF. RF 001 BLOOM W TEXTBOOK OF HISTOLOGY 521 968 002 DI SANTAGNESE PA PEDIATRICS 12 549 953 003 DOERSHUK CF J PEDIATR 65 677 964 004 JOHANSEN PG LANCET 1 455 968 005 KAISER D PEDIATR RES 5 167 971 006 LASHLEY KS PSYCHOL RES 5 167 971 007 LOWRY OH J BIOL CHEM 193 265 951 008 MANDEL ID AM J DIS CHILD 113 431 967 009 MANDEL ID AM J DIS CHILD 110 646 965 010 MANGOS JA SCIENCE 158 135 967 011 MANGOS JA PEDIATR RES 2 378 968 012 MANGOS JA PEDIATR RES 1 436 967 013 SCHNEYER LH J DENT RES 33 683 954 014 WEISMAN UN LANCET 1 510 972 CT 1 COLE CH PEDIATR RES 9 763 975 2 FARRELL PM PEDIATR RES 10 127 976 3 ANON J PEDIATR 88 711 976 4 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 DISANTAGNESE PA N ENGL J MED 295 534 976 7 IMPERO JE PEDIATR RES 12 108 978 8 SCHRODER CP EUR J PEDIATR 128 49 978 9 MARTINEZ JR PEDIATR RES 13 1156 979 10 WILL PC PEDIATR RES 13 1129 979 11 JAKEL HP BIOL ZENTRALBL 98 55 979 12 MUENZER J J BIOL CHEM 254 5623 979 13 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 14 SEALE TW PEDIATR RES 14 1398 980 15 WILL PC PEDIATR RES 14 1245 980 16 BRESLOW JL N ENGL J MED 304 1 981 17 SORSCHER EJ LANCET 1 368 982 18 BIJMAN J PEDIATR RES 18 1292 984 PN 74139 RN 00138 AN 74115763 AU Hodes-M-E. Merritt-A-D. Glier-J-T. TI An evaluation of differential methylation of ribonucleic acid in cystic fibrosis. SO Pediatr-Res. 1974 Mar. 8(3). P 212-4. MJ CYSTIC-FIBROSIS: me. RNA: me. MN CELLS-CULTURED. CENTRIFUGATION-DENSITY-GRADIENT. CYSTIC-FIBROSIS: fg. DNA: me. EVALUATION-STUDIES. FIBROBLASTS: me. HETEROZYGOTE. HUMAN. LYMPHOCYTES: me. METHYLATION. RNA-RIBOSOMAL: me. RNA-TRANSFER: me. SKIN: cy. AB No differences were found in the methylation of total nucleic acids, total RNA, DNA, tRNA, or rRNA from lymphocytes or fibroblasts obtained from normal subjects, obligate heterozygotes, or individuals with cystic fibrosis. Similar results were obtained using four methods for isolation of the nucleic acids. The reasons for the failure of two laboratories to discern the reported variations in methylation of RNA in the normal, carrier, or cystic fibrosis state may be obscure differences in culture conditions or conditions of extraction. RF 001 DRASHER ML SCIENCE 118 181 953 002 DUNN A ANAL BIOCHEM 41 460 967 003 EWTON D VIROLOGY 33 77 967 004 KLAGSBRUN M J BIOL CHEM 247 7443 972 005 KLAGSBRUN M IN: MANGOS JA 53 973 006 RENNERT OM CLIN PEDIATR 11 351 972 007 RENNERT OM PEDIATRICS 50 485 972 008 SCHMIDT G J BIOL CHEM 161 83 945 009 SEIN KT ANAL BIOCHEM 28 65 969 CT 1 HODES ME PEDIATR RES 9 812 975 2 BOLTON WE TEX REP BIOL MED 34 97 976 3 WARD JB TEX REP BIOL MED 34 11 976 4 ANON J PEDIATR 88 711 976 5 DISANTAGNESE PA N ENGL J MED 295 534 976 6 SULLIVAN JL BIOCHEM GENET 15 1125 977 7 SPICER SS EXP MOL PATH 33 104 980 PN 74140 RN 00139 AN 74153997 AU Boat-T-F. Wiesman-U-N. Pallavicini-J-C. TI Purification and properties of the calcium-precipitable protein in submaxillary saliva of normal and cystic fibrosis subjects. SO Pediatr-Res. 1974 May. 8(5). P 531-9. MJ CYSTIC-FIBROSIS: me. PHOSPHOPROTEINS: ip. SALIVA: an. SUBMANDIBULAR-GLAND: se. MN ADOLESCENCE. ADULT. AMINO-ACIDS: an. CALCIUM. COMPARATIVE-STUDY. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. FEMALE. CHROMATOGRAPHY-GEL. HUMAN. HYDROGEN-ION-CONCENTRATION. ISOELECTRIC-FOCUSING. MALE. MOLECULAR-WEIGHT. PHOSPHOPROTEINS: an. PHOSPHORUS: an. PRECIPITATION. PROTEIN-BINDING. SALIVARY-GLANDS: se. AB A calcium-precipitable protein (CaPP), previously shown to cause turbidity of submaxilliary saliva (SMS) in patients with cystic fibrosis (CF), was purified from normal and CF-SMS by (NH4)2SO4 precipitation, gel filtration, and preparative polyacrylamide gel electrophoresis. This protein has a molecular weight of 12,000. It contains large numbers of serine, glycine, and glutamic acid residues, 0.85% phosphorus as phosphate monoester, and small amounts of carbohydrate. Precipitation (aggregation) of CaPP occurs at basic pH values in the presence of ionic calcium and is enhanced by increasing calcium concentration, CaPP concentration, or pH. Aggregated CaPP binds calcium in molar quantities equal to the number of phosphate prosthetic groups. Alkaline phosphatase removes all phosphate from CaPP and eliminates calcium-induced precipitation. Studies of CaPP from CF saliva have demonstrated no electrophoretic or compositional differences from normal CaPP. Higher than normal pH values or elevated CaPP concentrations are not present in SMS of patients with CF and do not contribute to the formation of turbid SMS. Aggregation and precipitation of CaPP in saliva of patients with CF seems to be induced primarily by elevated levels of calcium in saliva. This precipitation may be entirely, or in part, responsible for focal obstruction of submaxillary gland ducts in patients with CF. However, little or no CaPP is secreted in the lower gastrointestinal or tracheobronchial tracts. Therefore, CaPP does not play a more general role in the obstructive processes which characterize CF. RF 001 ANTONOPOULOS CA ACTA CHEM SCAND 16 1521 962 002 BETTELHEIM FA BIOCHIM BIOPHYS ACTA 236 702 971 003 CHERNICK WS MOD PROBL PEDIATR 10 125 967 004 CHERNICK WS J PEDIATR 59 890 961 005 DISCHE Z J BIOL CHEM 175 595 948 006 EDELHOCH H BIOCHEMISTRY 6 1948 967 007 FARBER S ARCH PATHOL 37 238 944 008$ FISCHER EH IN: BOYER PD 4 343 960 009 FISKE CH J BIOL CHEM 66 375 925 010 GUGLER EC J PEDIATR 71 585 967 011 GUGLER EC J PEDIATR 73 548 968 012 HAY DI ARCH ORAL BIOL 12 937 967 013 KUNITZ M J GEN PHYSIOL 24 15 940 014 LOWRY OH J BIOL CHEM 193 265 951 015 MANDEL ID CLIN PEDIATR 8 161 969 016 MARMAR J GASTROENTEROLOGY 50 551 966 018 MECKEL AH ARCH ORAL BIOL 10 585 965 019 MCKENZIE HA ADV PROT CHEM 22 55 967 020 OSSERMAN EF J EXP MED 124 921 966 021 PALLAVICINI JC PROTIDES BIOL FLUIDS 15 541 967 022 PERLMANN GE ADV PROT CHEM 10 1 955 023 REISFELD RA NATURE 195 281 962 024 SCHNEYER LH J DENT RES 33 683 954 025 SHANNON IL US AIR FORCE SCHOOL OF AVIATI 60 960 026 SPIRO RG N ENGL J MED 281 991 969 027 SVENNERHOLM L BIOCHIM BIOPHYS ACTA 24 604 957 028 VAN LOON EJ AM J CLIN PATHOL 22 1134 952 029 WADSWORTH C INT ARCH ALLERGY APPL IMMUNOL 21 131 962 030 WEBER K J BIOL CHEM 175 595 969 031 WARTON KL BR MED J 3 683 971 032 WOTMAN S J PERIODONTOL 44 278 973 033 WRIGLEY C SCIENCE TOOLS 15 968 CT 1 BENNICK A BIOCHEM J 145 557 975 2 FORSTNER JF BIOCHIM BIOPHYS ACTA 386 283 975 3 WILSON GB SCAND J IMMUNOL 5 828 976 4 ALLARS HM PEDIATR RES 10 584 976 5 ALLARS HM PEDIATR RES 10 578 976 6 FORSTNER JF PEDIATR RES 10 609 976 7 WU JT PEDIATR RES 10 235 976 8 WOOD RE AM REV RESPIR DIS 113 833 976 9 BENNICK A BIOCHEM J 155 163 976 10 MAYO JW ARCH BIOCHEM BIOPHYS 175 507 976 11 SIEGEL IA AM J PHYSIOL 231 974 976 12 SANTAGNESE PAD N ENGL J MED 295 481 976 13 WOOD DL PEDIATR RES 11 827 977 14 NIEUWAMERONGEN AV BIOCHIM BIOPHYS ACTA 495 324 977 15 OWEN E J MOL MED 3 49 978 16 KATZ S PEDIATR RES 12 1033 978 17 ALHADEFF JA CLIN GENET 14 189 978 18 KATZ S RES COMMUN CHEM PATH PHARM 19 491 978 19 BOAT TF ACS SYMPOSIUM SERIES 1978 108 978 20 LEACH SA J DENT 7 149 979 21 WILKES PD J DENT 7 213 979 22 MARRIOTT C BIORHEOLOGY 16 331 979 23 MANDEL ID CRC CRIT REV CLIN LAB SCI 12 321 980 24 HALLINAN F MED HYPOTHESES 7 793 981 25 HAY DI CALC TISS INT 34 531 982 26 BAUM BJ J GERONTOL 37 392 982 27 SHOMERS JP J DENT RES 61 764 982 28 EGGEN KH SCAND J DENT RES 90 182 982 29 EGGEN KH SCAND J DENT RES 91 439 983 30 NORDBO H SCAND J DENT RES 91 182 983 31 KATZ S CELL CALC 5 421 984 32 EGGEN KH SCAND J DENT RES 93 426 985 33 DAVIS PB J DENT RES 66 667 987 PN 74141 RN 00140 AN 74154004 AU Feig-S-A. Segel-G-B. Kern-K-A. Osher-A-B. Schwartz-R-H. TI Erythrocyte transport function in cystic fibrosis. SO Pediatr-Res. 1974 May. 8(5). P 594-7. MJ CELL-MEMBRANE-PERMEABILITY. CYSTIC-FIBROSIS: bl. ERYTHROCYTES: me. MN ADENOSINE-TRIPHOSPHATASE: me. BLOOD-PROTEINS. CYSTIC-FIBROSIS: en. ERYTHROCYTES: en. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MAGNESIUM: me. OUABAIN: pd. POTASSIUM: me. POTASSIUM-ISOTOPES. RADIOISOTOPES. SODIUM: me. SODIUM-ISOTOPES. AB ATPase activity and monovalent cation transport were examined in erythrocytes (RBC) of subjects with cystic fibrosis (CF). The RBC membrane (Ca2+)ATPase activity of homozygous patients and obligate heterozygotes was indistinguishable from that of normal control subjects. This was true similarly for total, ouabain-sensitive, and ouabain-insensitive (Na+-K+)ATPase. Total, ouabain-sensitive, and ouabain-insensitive 24Na+ transport were normal in CF cells. Ouabain-induced Na+ accumulation was similar in CF and normal RBC. Measurements of K+ transport in CF RBC were, likewise, normal. We conclude that intrinsic ATPase and monovalent transport functions are normal in CF RBC. We were unable to confirm several abnormalities in these cells reported previously. The CF RBC is not helpful in defining a transport abnormality in that entity. These studies document normal intrinsic transport function in RBC of patients with CF. There have been reports of an abnormal serum factor in patients with CF. This factor might be involved in the production of a transport defect in CF. Further studies are indicated to determine whether CF plasma contains a factor capable of altering membrane transport functions of normal RBC. RF 001 AMES BN J BIOL CHEM 235 769 960 002 BALFE JW SCIENCE 162 689 968 003 COLE CH PEDIATR RES 6 616 972 005 FEIG SA J CLIN INVEST 51 1547 972 006 HADDEN JW PROC SOC EXP BIOL MED 142 577 973 007 HOFFMAN JF AM J MED 41 666 966 008 HORTON CR BIOCHEM BIOPHYS RES COMMUN 40 505 970 009 LAPEY A PEDIATR RES 4 478 970 010 LOWRY OH J BIOL CHEM 193 265 951 011 NAKAO K LIFE SCI 6 595 967 012 OGAWA Y J BIOCHEM (TOKYO) 64 255 968 013 POST RL J BIOL CHEM 235 1796 960 014 SPOCK A PEDIATR RES 1 173 967 CT 1 COLE CH PEDIATR RES 9 763 975 2 CHANGUS JE AM J PATHOL 80 317 975 3 WARD JB TEX REP BIOL MED 34 11 976 4 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 5 WOOD RE AM REV RESPIR DIS 113 833 976 6 EMRICH HM EUR J PEDIATR 122 293 976 7 SIEGEL IA AM J PHYSIOL 231 974 976 8 ARAKI H LANCET 1 793 978 9 KATZ S PEDIATR RES 12 1033 978 10 GALEY WR PEDIATR RES 14 1269 980 11 FODER B CLIN CHIM ACTA 104 187 980 12 STEGMAYR B ULTRASTRUCTURAL PATHOL 2 357 981 13 BANCHINI G PEDIATR RES 15 1073 981 14 REINILA M ANAL BIOCHEM 124 19 982 15 ALADJEM M NEPHRON 34 84 983 16 KATZ S CELL CALC 5 421 984 17 BERGHOUT AGRV PEDIATR RES 18 1017 984 18 DEARBORN DG PEDIATR RES 18 890 984 19 CLOUGH DL CLIN CHIM ACTA 138 259 984 20 WALLER RL CELL CALC 6 245 985 21 HUNSINGER RN CLIN CHIM ACTA 156 165 986 PN 74142 RN 00141 AN 74251247 AU Rao-G-J. Nadler-H-L. TI Arginine esterase in cystic fibrosis of the pancreas. SO Pediatr-Res. 1974 Jun. 8(6). P 684-6. MJ ESTERASES: bl. CYSTIC-FIBROSIS: en. MN HUMAN. ARGININE. CYSTIC-FIBROSIS: bl, fg. CHROMATOGRAPHY-DEAE-CELLULOSE. CHROMATOGRAPHY-ION-EXCHANGE. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. TRYPSIN-INHIBITORS: pd. HYDROGEN-ION-CONCENTRATION. BLOOD. AB Arginine esterase activity in chloroform-ellagic acid-treated plasma from 11 patients with cystic fibrosis(CF) and 12 age-matched control subjects has been resolved into its component fractions by ion exchange chromatography on DEAE-Sephadex and electrofocusing on polyacrylamide gels. The activity can be resolved into two fractions by chromatography, one of which is inhibited by soybean trypsin inhibitor (STI) and the other of which is not inhibited by STI. In plasma of CF patients, the fraction of activity inhibited by STI is reduced to approximately 30% of the corresponding fraction in control plasma. In contrast, the fraction of activity resistant to inhibition by STI did not show any significant quantitative differences between control and CF plasma samples. Analysis of plasma samples by electrofocusing on 5% polyacrylamide gels in the range of pH 5.0-8.0 and subsequent staining for arginine esterase activity showed qualitative differences between control and CF plasma samples. Six activity bands could be detected in control samples, whereas five bands were detected in CF samples. Eight samples had one type of band missing, two had another type of band missing, and one had yet another type of band missing. All of these bands were restricted to a narrow pH zone in the center of the gel. These data are consistent with and extend our earlier reports of differences in arginine esterase activity between plasma samples of CF patients and control subjects. The demonstration of the absence of a single band of arginine esterase activity is consistent with the absence of a specific arginine esterase isoenzyme in patients with CF. The deficiency of arginine esterase in patients with cystic fibrosis may account for the presence of "factors" in saliva, plasma, and fibroblasts of these patients and consequently for the clinical manifestations of the disease. RF 001 BOWMAN BH SCIENCE 167 871 970 002 COLMAN RW J CLIN INVEST 48 23 969 003 CONOVER JH LANCET 1 1122 973 004 DANES BS J EXP MED 129 775 969 005 CAUDILL M LANCET 2 307 973 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 DOGGETT RG NATURE NEW BIOL 243 251 973 008 FUJIMOTO Y J BIOCHEM (TOKYO) 71 751 972 009 HAGLUND H METHODS BIOCHEM ANAL 19 1 971 011 MANGOS JA PEDIATR RES 2 378 968 012 RAO GJS J PEDIATR 80 573 972 013 RAO GJS SCIENCE 177 610 972 014 SPOCK A PEDIATR RES 1 173 967 CT 1 RAO GJS PEDIATR RES 9 739 975 2 ALTLAND K HUM GENET 28 207 975 3 ALHADEFF JA CLIN GENET 10 63 976 4 SHAPIRA E PEDIATR RES 10 812 976 5 WILSON GB PEDIATR RES 10 87 976 6 LEDERBERG S AM J HUM GENET 28 597 976 7 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 8 ANON J PEDIATR 88 711 976 9 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 10 WOOD RE AM REV RESPIR DIS 113 833 976 11 DISANTAGNESE PA N ENGL J MED 295 534 976 12 CALLAHAN JW PEDIATR RES 11 1166 977 13 RAO GJS PEDIATR RES 11 981 977 14 COLMAN RW THROMB HAEMOST 38 751 977 15 CHAN KYH CLIN CHIM ACTA 74 71 977 16 GOLDSMITH GH J LAB CLIN MED 89 131 977 17 DANN LG LANCET 2 405 978 18 PLATT MW PEDIATR RES 12 874 978 19 RAO GJS ENZYME 23 314 978 20 ROMEO G NATURE 274 909 978 21 RAO GJS BIOCHIM BIOPHYS ACTA 541 435 978 22 ROMEO G PEDIATR RES 13 1030 979 23 WALSHPLATT M ENZYME 24 224 979 24 JAKEL HP BIOL ZENTRALBL 98 55 979 25 WALSH MMJ PEDIATR RES 14 353 980 26 NADLER HL PEDIATRICS 66 690 980 27 DUFFY MJ CLIN CHIM ACTA 103 233 980 28 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 29 MARTINEZ JR PEDIATR RES 15 1439 981 30 DANDONA P THORAX 36 60 981 31 SEALE TW ANN CLIN LAB SCI 12 415 982 32 SCHWARTZ M CLIN CHIM ACTA 124 213 982 33 GREEN JR EUR J PEDIATR 139 35 982 34 BROCK DJH PRENAT DIAGN 3 1 983 35 BRANCHINI BR PEDIATR RES 17 850 983 36 HEELEY AF CLIN CHEM 29 2011 983 PN 74143 RN 00142 AN 74251248 AU Barnett-D-R. Bowman-B-H. TI Letter: Cystic fibrosis ciliary inhibitor. SO Pediatr-Res. 1974 Jun. 8(6). P 687. MJ CYSTIC-FIBROSIS: me. CILIA: de. PROTEINS: ip. MN ANIMAL. HUMAN. CYSTIC-FIBROSIS: im. FIBROBLASTS: me. SKIN. MOLECULAR-WEIGHT. CHROMATOGRAPHY-DEAE-CELLULOSE. COMPLEMENT: an. MOLLUSCA. EX The fact that amniotic cells from fetuses at high risk for the CF gene, as well as cells derived from heterozygous individuals, produce the ciliary inhibitor suggests a close relation to the genetic defect and necessitates further identification and characterization of the responsible molecule. RF 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BERATIS NG PEDIATR RES 7 958 973 004 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 005 BOWMAN BH CLIN GENET 4 461 973 006 BOWMAN BH AM J HUM GENET 25 16A 973 007 CONOVER JH PEDIATR RES 7 224 973 008 CONOVER JH AM J HUM GENET 25 21A 973 009 DANES BS J EXP MED 136 1313 972 010 DANES BS J EXP MED 137 1538 973 CT 1 WILSON GB PEDIATR RES 10 87 976 2 DANES BS TEX REP BIOL MED 34 135 976 3 HARPER BL TEX REP BIOL MED 34 73 976 4 WILSON GB TEX REP BIOL MED 34 51 976 5 WILSON GB PEDIATR RES 11 317 977 6 WILSON GB NATURE 266 463 977 PN 74144 RN 00143 AN 74115762 AU Klagsbrun-M. Farrell-P-M. TI The methylation of transfer and ribosomal ribonucleic acid in human fibroblasts: normal methylation of ribonucleic acid in cystic fibrosis. SO Pediatr-Res. 1974 Mar. 8(3). P 205-11. MJ CYSTIC-FIBROSIS: me. RNA-RIBOSOMAL: me. RNA-TRANSFER: me. MN ADOLESCENCE. ADULT. AUTORADIOGRAPHY. CARBON-RADIOISOTOPES. CELLS-CULTURED. CHILD. CHROMATOGRAPHY. CYSTIC-FIBROSIS: di. FIBROBLASTS: me. HUMAN. METHIONINE: me. METHYLATION. RIBONUCLEOSIDES: me. SKIN: cy. TRITIUM. URIDINE: me. AB A comprehensive study of tRNA and 18 S and 28 S rRNA methylation in human fibroblasts is presented. Cells from normal volunteers and patients with cystic fibrosis (CF) were examined during exponential growth by incubation with L-(methyl-14C)methionine and (3H)uridine. The ratio of 14C to 3H counts per minute in isolated RNA serves as a measure of the extent of methylation and was found to be similar in normal and CF fibroblasts for tRNA, 18 S rRNA, and 28 S rRNA. Hydrolyzed samples of (14C)-methyl-labeled RNA species were analyzed by two dimensional chromatography and autoradiography. The methylated base and nucleoside compositions of tRNA and rRNA agree well with data for HeLa cells and other eurkaryote cells such as those from yeast, mouse, and chicken. RNA methylation patterns in CF fibroblasts were qualitatively and quantitatively similar to those observed in normal cells for 4 S, 18 S, and 28 S RNA. There is no evidence that RNA species from patients with cystic fibrosis are deficient in any methylated components. Methylation of RNA by tissue-cultured cells from cystic fibrosis is qualitatively and quantitatively normal and will not prove to be a useful marker for identification of carriers of cystic fibrosis. RF 001 BALTIMORE D J MOL BIOL 18 421 966 002 BROWN GM BIOCHEM BIOPHYS RES COMMUN 20 298 965 003 FURANO AV ANAL BIOCHEM 43 639 971 004 HATTMAN J J VIROL 7 690 971 006 KLAGSBRUN M VIROLOGY 44 153 971 007 KLAGSBRUN M J BIOL CHEM 247 7443 972 008 KLAGSBRUN M J BIOL CHEM 248 2612 973 009 KLAGSBRUN M IN: MANGOS JA 53 973 010 MELLMAN WJ IN: ROTHBLAT GH 1 327 972 012 RAZIN A J MOL BIOL 53 251 970 013 RENNERT OM CLIN PEDIATR 11 351 972 014 RENNERT OM PEDIATRICS 50 485 972 015 WAGNER EK J MOL BIOL 129 371 967 016 WRIGHT SW AM J HUM GENET 20 157 968 CT 1 BOLTON WE TEX REP BIOL MED 34 97 976 2 DANES BS TEX REP BIOL MED 34 135 976 3 WARD JB TEX REP BIOL MED 34 11 976 4 DISANTAGNESE PA N ENGL J MED 295 534 976 5 SULLIVAN JL BIOCHEM GENET 15 1125 977 PN 74145 RN 00144 AN 75010573 AU Fox-W-W. Bureau-M-A. Taussig-L-A. Martin-R-R. Beaudry-P-H. TI Helium flow-volume curves in the detection of early small airway disease. SO Pediatrics. 1974 Sep. 54(3). P 293-9. MJ AIRWAY-OBSTRUCTION: di. CYSTIC-FIBROSIS: co. HELIUM: du. RESPIRATORY-FUNCTION-TESTS. MN ADOLESCENCE. AIRWAY-OBSTRUCTION: co, pp. CARBON-DIOXIDE: bl. CHILD. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. LUNG: ra, pp. MALE. OXYGEN: bl. PARTIAL-PRESSURE. RESPIRATION. SPIROMETRY. VITAL-CAPACITY. AB To assess small airway disease in cystic fibrosis (CF) patients with minimal pulmonary involvement, maximal expiratory flow volume (MEFV) curves were obtained while the patients were breathing first air and then an 80% helium-20% oxygen gas mixture. Fifteen CF patients and 24 controls were studied. Flow rates at 50% and 25% vital capacity (VC) were calculated from the air and helium-mixture MEFV curves and were compared to give flow ratios at these lung volumes. At 50% VC, the helium/air flow ratios were similar in CF patients and normal subjects. At 25% VC, the flow ratio was significantly lower (p < 0.05) in the CF patients. The air and helium MEFV curves were superimposed and the point where the curves crossed (point of identical flow, PIF) was determined and expressed in % VC. For the CF patients, the mean PIF was 18% and for the controls, 5% (p < .001). Nine of the 15 CF patients had PIF values greater than 2 standard deviations (SD) from the normal mean. None of the following tests were abnormal in more than three patients: FEV1/FVC, MMEF, RV/TLC, Vmax 50% TLC (TLC/sec), and Vmax 25% VC (TLC/sec). Arterial oxygen tensions were below 86 mm Hg in four patients. Closing volumes by the 100% helium bolus technique were normal in all patients. Determination of the PIF appears to be a simple, noninvasive, and sensitive test for the detection of early small airway involvement. RF 001 MELLINS RB PEDIATRICS 44 315 969 002 ZAPLETAL A PEDIATRICS 48 64 971 003 LANDAU LI AM REV RESPIR DIS 108 593 973 004 LAMARRE A PEDIATRICS 50 291 972 005 HOGG JC N ENGL J MED 278 1355 968 006 HOGG JC N ENGL J MED 282 1283 970 007 MEAD J N ENGL J MED 282 1318 970 008 MCCARTHY DS AM J MED 52 747 972 009 DESPAS PJ J CLIN INVEST 51 3235 972 010 MEAD J J APPL PHYSIOL 22 95 967 011 MACKLEM PT ARCH ENVIRON HEALTH 14 5 967 012 DI SANTAGNESE PA IN: NELSON WE 856 969 013 TAUSSIG LM J PEDIATR 82 380 973 014 BEAUDRY PH AM REV RESPIR DIS 95 248 967 015 WENG TR AM REV RESPIR DIS 99 879 969 016 POLGAR G PULMONARY FUNCTION TESTING IN 971 017 ZAPLETAL A J APPL PHYSIOL 26 308 969 018 GREEN M J APPL PHYSIOL 33 827 972 019 SIGGAARD ANDERSEN O SCAND J CLIN LAB INVEST 12 172 960 020 LEVISON H AM REV RESPIR DIS 101 972 970 021 FEATHERBY EA AM REV RESPIR DIS 102 737 970 022 MANSELL A J APPL PHYSIOL 33 711 972 023 REILLY BJ RADIOLOGY 98 281 971 024 HILL DJ AM REV RESPIR DIS 106 873 972 025 SEELY JE SCIENCE 172 741 971 CT 1 LANDAU LI AM REV RESPIR DIS 111 725 975 2 CORBET A AM REV RESPIR DIS 112 513 975 3 RONCORONI AJ MED (BUENOS AIRES) 36 347 976 4 WOOD RE AM REV RESPIR DIS 113 833 976 5 WAYNE KS AM REV RESPIR DIS 114 15 976 6 TAUSSIG LM PEDIATR RES 11 261 977 7 TAUSSIG LM CLIN PEDIATR 16 57 977 8 CHERNICK V PEDIATRICS 59 783 977 9 VANCE JC PEDIATRICS 60 263 977 10 COATES AL J PEDIATR 90 611 977 11 WOHL MEB J PEDIATR 90 405 977 12 LOREN ML J PEDIATR 91 688 977 13 WALSON PD J PEDIATR 91 321 977 14 PROCTOR DF AM REV RESPIR DIS 115 97 977 15 ZAPLETAL A BULL EUR PHYSIOPATH RESP 14 265 978 16 BUREAU MA PEDIATRICS 61 842 978 17 GURWITZ D PEDIATRICS 62 789 978 18 COATES AL J PEDIATR 92 247 978 19 ANCIC P REV MED CHIL 106 709 978 20 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 21 MCBRIDE JT PEDIATR CLIN NORTH AM 26 537 979 22 LANDAU LI THORAX 34 217 979 23 DULL WL RESPIRATION 38 18 979 24 SANTAGNESE PAD AM J MED 66 121 979 25 LOUGHLIN GM J PEDIATR 94 365 979 26 WIESEMANN H RESPIRATION 41 181 981 27 ZUSKIN E INT ARCH OCCUP ENVIRON HEALTH 49 41 981 28 COATES AL ARCH DIS CHILD 56 106 981 29 CLOUTIER MM PEDIATRICS 67 6 981 30 LOUGHLIN GM CHEST 79 206 981 31 TAUSSIG LM AM REV RESPIR DIS 123 640 981 32 TECULESCU DB LUNG 159 127 981 33 TECULESCU DB CLIN PHYSIOL 2 379 982 34 KERREBIJN KF EUR J RESPIR DIS 63 35 982 35 BEGIN R AM J MED 72 743 982 36 REDDING GJ AM REV RESPIR DIS 126 31 982 37 PAGTAKHAN RD J APPL PHYSIOL 56 1204 984 38 WOLFSON MR J PEDIATR 104 752 984 39 TAL A EUR J PEDIATR 142 117 984 40 TECULESCU DB BULL EUR PHYSIOPATH RESP 21 193 985 41 DESMOND KJ PEDIATR PULMONOL 2 128 986 42 ALLEN JL CLIN PEDIATR 25 541 986 PN 74146 RN 00145 AN 75011199 AU Lloyd-Still-J-D. Hurwitz-I. Wolff-P-H. Shwachman-H. TI Intellectual development after severe malnutrition in infancy. SO Pediatrics. 1974 Sep. 54(3). P 306-11. MJ CHILD-DEVELOPMENT. NUTRITION-DISORDERS. MN ADAPTATION-PSYCHOLOGICAL. ADOLESCENCE. ADULT. BIRTH-WEIGHT. BLOOD-PROTEINS: an. BODY-HEIGHT. CEPHALOMETRY. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, me. FECES: an. FEMALE. GESTATIONAL-AGE. HUMAN. HYPOPROTEINEMIA: bl. ILEUM: ab. INFANT. INTELLIGENCE-TESTS. INTELLIGENCE. INTESTINAL-DISEASES: cn. MALE. MOTOR-ACTIVITY. NUTRITION-DISORDERS: et. SERUM-ALBUMIN: an. TRYPSIN: me. VITAMIN-K-DEFICIENCY: bl. AB Intellectual performance, sensory motor abilities and social adaptation were studied in 41 subjects (2 to 21 years of age) who had severe malnutrition in infancy. A control group consisted of 41 siblings. The mean IQ of 31 parents ws 108 (S.D. plus or minus 11.3). Socioeconomic deprivation was not present. The results of the Merrill-Palmer test for the malnourished group and the controls revealed significant differences in favor of the controls. No differences were found in the older population for whom the WISC and WAIS were used. The Lincoln-Oseretsky test of motor development and the Vineland scale of social maturity showed no significant differences. These results are consistent with the hypothesis that malnutrition in infancy can affect intellectual development in the first five years of life. Beyond this age, given adequate socioeconomic support, no significant differences were observed. RF 001 STOCH MB ARCH DIS CHILD 38 546 963 002 CRAVIOTO J AM J ORTHOPSYCHIATRY 35 449 965 003 CABAK V ARCH DIS CHILD 40 532 965 004 CRAVIOTO J PEDIATRICS PART 2 SUPPL 38 319 966 005 STOCH MB S AFR MED J 41 1027 967 006 LIANG PH AM J CLIN NUTR 20 1290 967 007 MONCKEBERG F IN: SCRIMSHAW NS 269 968 008 CHAMPAKAM S AM J CLIN NUTR 21 844 968 009 CRAVIOTO J AM J DIS CHILD 120 404 970 010 BIRCH HG PEDIATR RES 5 579 971 011 HERTZIG ME PEDIATRICS 49 814 972 012 DOBBING J AM J DIS CHILD 120 411 970 013 BROWN RE DEVELOP MED CHILD NEUROL 8 512 966 014 WINICK M PEDIATR RES 3 181 969 015 KUGELMASS IN AM J MED SCI 208 631 944 016 CHASE HP N ENGL J MED 282 933 970 017 STEIN Z SCIENCE 178 708 972 018 HANSEN JDL PEDIATRICS 47 299 971 019 LLOYD-STILL JD PROC INT CONF ON NUTRI 9TH 972 020 ANON LANCET 2 302 970 021 GOMEZ F J TROP PEDIATR 2 77 956 022 SHWACHMAN H IN: KENDIG EL JR 1 524 972 023 HOLLINGSHEAD AB IN: BONJEAN CM 441 967 024 SHWACHMAN H PEDIATRICS 7 153 951 025 DOBBING J ARCH DIS CHILD 48 757 973 026 OSOFSKY HJ AM J OBSTET GYNECOL 105 1150 969 027 NAEYE RL PEDIATR RES 5 17 971 028 LUBCHENCO LO PEDIATR CLIN NORTH AM 17 125 970 029 WIENER G J PEDIATR 76 694 970 030 DRILLIEN CM J OBSTET GYNAECOL BR COMMONW 66 721 959 031 AMIEL-TISON C BIOL NEONATE 14 234 969 032 LUBCHENCO LO J PEDIATR 80 501 972 033 SCRIMSHAW NS JAMA 212 1685 970 034 DAVISON AN BR MED BULL 22 40 966 035 BOWLBY J INT J PSYCHOANAL 41 89 960 036 GRAHAM GG AM J CLIN NUTR 25 1184 972 037 BOYER PH PEDIATRICS 16 778 955 038 ANON AM J CLIN NUTR 23 807 970 039 WATERLOW JC BR MED J 3 566 972 040 KAGAN J CHILD DEVELOPMENT 30 325 959 041 RICHARDSON SA IN: SCRIMSHAW NS 346 968 042 RASOF B CHILD DEVELOPMENT 38 1043 967 CT 1 ELLIS CE J PEDIATR 87 565 975 2 KLEIN PS J PEDIATR 87 8 975 3 CHEEK DB AM J CLIN NUTR 29 1149 976 4 HALL RT ARCH DIS CHILD 51 929 976 5 JONES DG SCI PROG 63 483 976 6 LLOYDSTILL JD J PEDIATR 88 702 976 7 FIEHRING C PAEDIATR PAEDOL 12 283 977 8 BALLI F MINERVA PEDIATR 29 2255 977 9 BALLI F MINERVA PEDIATR 29 1621 977 10 ELIAS MF AM J CLIN NUTR 30 355 977 11 WOOD RE JADA 95 596 977 12 EISENBERG L BR J PSYCHIATRY 131 225 977 13 CELEDON JM EARLY HUM DEV 3 267 979 14 OLIVI O MINERVA PEDIATR 31 1459 979 15 CELEDON JM J MENT DEFIC RES 24 27 980 16 MACLEAN WC J PEDIATR 97 316 980 17 CRNIC LC PHYSIOL BEHAV 26 695 981 18 BARRETT DE AM J CLIN NUTR 35 1222 982 19 BEARDSLEE WR AM J CLIN NUTR 35 1437 982 20 BURGESS DB PEDIATRICS 70 624 982 21 GRAHAM GG AM J DIS CHILD 136 348 982 22 ABRAHAMIAN FP J PEDIATR GASTROENTEROL NUTR 3 460 984 23 HAMMOND PB NEUROTOXICOL 5 53 984 24 PRENSKY AL N ENGL J MED 310 1527 984 25 ULTMANN MH DEVELOP MED CHILD NEUROL 27 563 985 26 LAZARUS T S AFR MED J 68 479 985 27 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 PN 74147 RN 00146 AN 75013285 AU Rucker-R-W. Harrison-G-M. TI Outpatient intravenous medications in the management of cystic fibrosis. SO Pediatrics. 1974 Sep. 54(3). P 358-60. MJ ANTIBIOTICS: ad. CYSTIC-FIBROSIS: co. INFUSIONS-PARENTERAL: mt. RESPIRATORY-TRACT-INFECTIONS: dt. MN ADMINISTRATION-ORAL. ADOLESCENCE. ADULT. ANTIBIOTICS: tu. CHILD. COLISTIN: aa, tu, ad. GENTAMICINS: tu, ad. OUTPATIENT-CLINICS-HOSPITAL. HUMAN. INFUSIONS-PARENTERAL: ae. PSEUDOMONAS-AERUGINOSA: ip. RESPIRATORY-FUNCTION-TESTS. RESPIRATORY-TRACT-INFECTIONS: co, di, mi. STAPHYLOCOCCUS: ip. EX The management of cystic fibrosis continues to present a constant therapeutic problem to both pediatrician and internist. In these hospitalized patients, the use of a butterfly scalp vein needle attached to a plastic tubing sealed by a rubber cap ("Heparin Lock") offers many advantages. This report relates our experience with this method. The use of an indwelling intravenous infusion set for the home administration of potent antibiotics has been quite successful in the therapy of the chronic bronchopulmonary infection associated with cystic fibrosis. RF 001 STERN RC CLIN PEDIATR 11 521 972 002 SHWACHMAN H AM J DIS CHILD 96 6 958 003 HUANG NN AM J DIS CHILD 120 289 970 004 GELLIS SS YEARBOOK OF PEDIATRICS 169 973 005 GINGELL JC BR MED J 2 19 968 006 STRATFORD BC LANCET 1 378 974 CT 1 DAVID S PEDIATRICS 55 896 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 LAU WK PEDIATRICS 60 372 977 4 STEIN REK J PEDIATR 92 495 978 5 KRAUSE PJ CURR THER RES CLIN EXP 25 609 979 6 WOOD RE SOUTH MED J 72 189 979 7 KIND AC ARCH INTERN MED 139 413 979 8 MARKS MI J PEDIATR 98 173 981 9 BERGER LR AM J DIS CHILD 135 812 981 10 STIVER HG CAN MED ASSOC J 127 207 982 11 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 12 GLECKMAN R PHARMACOTHERAPY 3 239 983 13 REHM SJ ANN INTERN MED 99 388 983 14 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 15 GALT MA AM J HOSP PHARM 41 2355 984 16 REED MD DRUG INTEL CLIN PHARM 19 288 985 17 SMEGO RA AM J HOSP PHARM 42 2185 985 18 REHM SJ CLEVE CLIN Q 52 333 985 19 HARRIS WL AM J HOSP PHARM 43 699 986 PN 74148 RN 00147 AN 74146030 AU Gibson-L-E. TI Letter: Rates and calcium concentrations of sweat in relation to cystic fibrosis. SO Pediatrics. 1974 Apr. 53(4). P 584-5. MJ CALCIUM: an. CYSTIC-FIBROSIS: me. SWEAT: an. MN HUMAN. SECRETORY-RATE. SWEAT: se. EX The authors of the recent article which failed to show a significant difference between sweat calcium concentrations of CF patients and control subjects, suggest that the findings refute our hyperpermeability hypothesis. They found sweat Ca of 0.32 to 1.67 mEq/liter in 26 control subjects and 0.42 to 2.03 mEq/liter in 16 CF patients. With these tremendous ranges many more subjects would be required to prove or disprove a difference. A difference undetectable in a moderate number of subjects is probably not of pathophysiologic significance if the Ca really varies unpredictably. Sweat Ca, however, is generally believed to vary with the rate of sweating, being much higher at lower rates, and measurements made at comparable rates might well show significant differences. The authors did attempt to control rate, but in a very inappropriate manner. They measured no constant rate, but took the average rate during the 30 minutes immediately after iontophoresis. This gives little information concerning the rate at which the sweat was actually produced. RF 001 PAUNIER L PEDIATRICS 52 446 973 002 GIBSON LE PEDIATRICS 48 695 971 003 MITCHELL HH J BIOL CHEM 178 345 949 004 EMRICH HM PEDIATR RES 2 464 968 005 GIBSON LE PEDIATRICS 23 545 959 006 KUNO Y HUMAN PERSPIRATION 956 007 GIBBS GE TRANS INT RES CONF CF 145 959 008 HUELEY HJ J INVEST DERMATOL 39 329 962 PN 74149 RN 00148 AN 74158300 AU Lloyd-Still-J-D. Khaw-K-T. Shwachman-H. TI Severe respiratory disease in infants with cystic fibrosis. SO Pediatrics. 1974 May. 53(5). P 678-82. MJ CYSTIC-FIBROSIS: co. RESPIRATORY-INSUFFICIENCY: et. RESPIRATORY-TRACT-INFECTIONS: et. MN ACIDOSIS: et. ACIDOSIS-RESPIRATORY: et. ANOXEMIA: di. ANTIBIOTICS: tu. ATELECTASIS: et, ra. AUTOPSY. CYSTIC-FIBROSIS: dt, ra. ESCHERICHIA-COLI-INFECTIONS: dt. FEMALE. FOLLOW-UP-STUDIES. HUMAN. HUMIDITY. HYDROCORTISONE: tu. INFANT. KLEBSIELLA-INFECTIONS: dt. MALE. OXYGEN: bl. OXYGEN-INHALATION-THERAPY. PREDNISONE: tu. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-INSUFFICIENCY: di, dt, ra. RESPIRATORY-TRACT-INFECTIONS: di, ra. STAPHYLOCOCCAL-INFECTIONS: dt. AB The diagnosis, management and prognosis of 17 infants with cystic fibrosis (CF) and severe respiratory disease were reviewed for the period 1968 to 1972. The clinical course of these infants was characterized by a bronchiolitis-like syndrome with failure to thrive and malnutrition. Arterial blood gases demonstrated marked hypoxemia (mean PaO2, 36.5 mm Hg). Tracheal cultures showed a predominance of gram-negative microorganisms with Pseudomonas aeruginosa, Klebsiella pneumoniae and Escherichia coli predominating. Corticosteroids were used in all patients. Despite vigorous therapy including antibiotics there was a 60% mortality. A delay in the diagnosis of CF from the onset if respiratory symptoms with a mean of six weeks was considered an important factor affecting survival. This data supports the need for developing a reliable screen test for CF at birth. RF 001 SHWACHMAN H PEDIATRICS 46 335 970 002 SHWACHMAN H MINN MED 52 1521 969 003 HOLSCLAW DS J PEDIATR 76 829 970 004 HANDWERGER S N ENGL J MED 281 451 969 005 KAPLAN E N ENGL J MED 279 65 968 006 MCCOLLUM AT J PEDIATR 77 571 970 007 NOBLETT HR J PEDIATR SURG 4 190 969 008 DI SANTAGNESE PA PEDIATRICS 12 178 953 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 DONNISON AB PEDIATRICS 37 833 966 011 SHWACHMAN H ADV PEDIATR 7 249 955 012 DENNY FW PEDIATR RES 3 463 969 013 PARROTT RH J PEDIATR 61 205 962 014 SIMON G J PEDIATR 70 533 967 015 ROONEY JC J PEDIATR 79 744 971 016 MCGIVEN AR ARCH DIS CHILD 37 656 962 017 REYNOLDS EOR BR MED J 1 1192 963 018 HUANG NN GUIDE TO DRUG THERAPY IN PATI 974 019 SHWACHMAN H IN: KENDIG EL JR 1 524 972 020 DABBOUS IA PEDIATRICS 37 477 966 021 ZOUMBOULAKIS D ARCH DIS CHILD 48 51 973 CT 1 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 2 WOOD RE AM REV RESPIR DIS 113 833 976 3 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 4 STERN RC AM REV RESPIR DIS 118 821 978 5 SHWACHMAN H AM J DIS CHILD 132 1112 978 6 LLOYDSTILL JD JAMA 240 739 978 7 KISTLER I HELV PAEDIATR ACTA 36 495 982 8 STEIN MT WEST J MED 136 505 982 9 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 10 ABMAN SH J PEDIATR 107 933 985 11 SHERMAN JM PEDIATR PULMONOL 2 244 986 12 ABMAN SH J PEDIATR GASTROENTEROL NUTR 5 393 986 PN 74150 RN 00149 AN 74158303 AU Rosenfield-N. Treves-S. TI Liver-spleen scanning in pediatrics. SO Pediatrics. 1974 May. 53(5). P 692-701. MJ BILIARY-TRACT-DISEASES: di. HEPATOMA: di. KIDNEY-NEOPLASMS: di. LIVER-DISEASES: di. LIVER-NEOPLASMS: di. NEPHROBLASTOMA: di. RADIONUCLIDE-IMAGING. SPLENIC-DISEASES: di. MN ANEMIA-SICKLE-CELL: di. CHILD. CYSTIC-FIBROSIS: di. CYSTS: di. DIAGNOSTIC-ERRORS. EVALUATION-STUDIES. HEPATOMEGALY: et. HUMAN. IODINE-RADIOISOTOPES: du. IODINE-RADIOISOTOPES. CHOLESTASIS: di. LIVER-CIRRHOSIS: di. SPLENIC-RUPTURE: di. SPLENOMEGALY: et. TECHNETIUM. AB Liver-spleen scans on 254 children were reviewed retrospectively with regard to accuracy and yield of examination. The scan predicted abnormality correctly 95% of the time and normality correctly 86% of the time. Pitfalls in interpretation include the nonspecificity of abnormalities present on the scan, confusion of extrinsic with intrinsic defects, and normal anatomical variations with pathology. The technetium-99m-sulfur colloid scan was found to be most helpful in diagnosing splenic abnormalities, in working up abdominal masses, and in evaluating tumor patients with a baseline scan. It was found least useful in patients with fever of unknown origin, abdominal pain, diffuse liver disease, and most inflammatory conditions. Liver abscesses were not found in febrile, otherwise healthy children. The iodine-131-rose bengal liver scan was found to be useful to differentiate potentially curable lesions (e.g., choledochal cysts) from those not surgically treatable. RF 001 TEFFT M AM J ROENTG RAD THER NUCL MED 101 570 967 002 TREVES S SEMIN NUCL MED 3 55 973 003 NAGLER W GASTROENTEROLOGY 44 36 963 004 DRUM DE J NUCL MED 13 908 973 005 FELLOWS KE AM J ROENTG RAD THER NUCL MED 104 678 968 006 CONN HO GASTROENTEROLOGY 54 135 968 007 LUSTED LB N ENGL J MED 284 416 971 008 TEFFT M AM J ROENTG RAD THER NUCL MED 111 165 971 009 FELLOWS KE AM J ROENTG RAD THER NUCL MED 103 422 968 010 TEFFT M AM J ROENTG RAD THER NUCL MED 108 365 970 011 FREEDOM RM RADIOLOGY 107 381 973 012 GRISCOM NT AM J DIS CHILD 109 224 965 013 BLANK E PEDIATRICS 51 75 973 014 PEARSON HA J PEDIATR 77 216 970 015 PEARSON HA N ENGL J MED 283 334 970 016 PEARSON HA J NUCL MED 11 348 970 017 OMARA RE J PEDIATR 77 211 970 018 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 019 WHITE WE PEDIATRICS 32 239 963 020 BRENT RL AM J DIS CHILD 98 720 959 021 MAKSOUD JG PEDIATRICS 48 966 971 022 SILVERBERG M SEMIN NUCL MED 3 69 973 023 THALER MM AM J DIS CHILD 116 257 968 024 WILLIAMS LE N ENGL J MED 283 85 970 025 WAGNER HN IN: WAGNER HN 601 968 026 VIDRIH VE CAN J SURG 14 273 971 027 COVINGTON E AM J ROENTG RAD THER NUCL MED 109 742 970 028 LUDBROOK J GASTROENTEROLOGY 62 1013 972 029 MCAFEE JG ARCH INTERN MED 116 95 965 030 CONN HO GASTROENTEROLOGY 62 1085 972 031 SEYMOUR EQ AM J ROENTG RAD THER NUCL MED 107 54 969 032 ODONELL TA AM J ROENTG RAD THER NUCL MED 96 1063 963 033 FREEMAN LM BR J RADIOL 42 651 969 CT 1 WALKER WA PEDIATR CLIN NORTH AM 22 929 975 2 WHITE PR PEDIATR CLIN NORTH AM 22 851 975 3 TREVES S POSTGRAD MED 57 125 975 4 ROSENFIELD N RADIOLOGY 114 113 975 5 GATES GF AM J ROENTGENOL 128 773 977 6 GATES GF JAMA 239 2667 978 7 RACHEDMOHASSEL MA ANN CHIR 33 123 979 8 ROSEN PR CLIN PEDIATR 21 180 982 PN 74151 RN 00150 AN 74290775 AU Taussig-L-M. Wolf-R-O. Woods-R-E. Deckelbaum-R-J. TI Use of serum amylase isoenzymes in evaluation of pancreatic function. SO Pediatrics. 1974 Aug. 54(2). P 229-35. MJ AMYLASES: bl. CYSTIC-FIBROSIS: en. ISOENZYMES: bl. PANCREAS: en. MN ADOLESCENCE. ADULT. AMYLASES: ur. CHILD. CHILD-PRESCHOOL. DUODENUM: se. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-SECRETIONS: en. ISOENZYMES: ur. MALE. PANCREATIN: du. SALIVA: en. AB Amylase isoenzyme patterns were evaluated in serum, urine, and duodenal fluid from 19 patients with cystic fibrosis (CF) and normal subjects. Two thirds of the CF patients with absent pancreatic enzymes lacked a serum pancreatic isoamylase band, while the remainder had a markedly diminished pancreatic band when compared to the salivary isoamylase. In normal sera the pancreatic band is equal to or greater than the salivary band. In all patients with absent enzymes the diagnosis of abnormal pancreatic function could have been made by evaluation of serum isoamylase patterns thereby avoiding duodenal intubation or collection of 72-hour stools for proteolytic enzyme activities. The patients with normal pancreatic enzyme activities had normal serum zymograms. No CF patient had a low total serum amylase concentration. Total duodenal fluid amylase levels may be misleading in the evaluation of pancreatic function since the amylase may be of salivary origin. The advantages of this isoamylase method include (1) simplicity and noninvasiveness, requiring only a small blood sample; (2) specificity for pancreatic function; and (3) independence of pancreatic enzyme medication. RF 001 DI SANTAGNESE PA IN: NELSON WE 856 969 002 KATTWINKEL J PEDIATRICS 51 55 973 003 SHWACHMAN H J PEDIATR 65 645 964 004 BARBERO GJ AM J DIS CHILD 112 536 966 005 BANWELL JG GUT 6 143 965 006 DYCK WP AM J DIG DIS 12 310 967 007 BONIN A J PEDIATR 83 594 973 008 SILVERMAN A PEDIATR CLIN GASTROENTEROL 971 009 HADORN B J PEDIATR 73 39 968 010 MEITES S CRC CRIT REV CLIN LAB SCI 2 103 971 011 WOLF RO OBSTET GYNECOL 41 337 973 012 AW SE GUT 8 402 967 013 TAUSSIG LM J PEDIATR 82 380 973 014 NORTHROP JH J GEN PHYSIOL 5 353 923 015 SCHWERT GW BIOCHIM BIOPHYS ACTA 16 570 955 016 FOLK JE J BIOL CHEM 235 2272 960 017 SHAPIRA E J LAB CLIN MED 77 877 971 018 WOLF RO AM J CLIN PATHOL 49 871 968 019 SOMOGYI M J BIOL CHEM 125 319 938 020 NORBY S EXP CELL RES 36 663 964 021 KAMARYT J HUMANGENETIK 1 579 965 022 SHWACHMAN H AM J DIS CHILD 92 347 956 023 GOLDBERG DM GUT 11 859 970 024 DUANE WC J CLIN INVEST 50 156 971 025 DUANE WC J CLIN INVEST 51 1504 972 026 LILLIBRIDGE CB J PEDIATR 82 279 973 027 LOWE CU AM J DIS CHILD 82 459 951 028 TOWNES PL J PEDIATR 75 221 969 029 TOWNES PL J PEDIATR 66 275 965 CT 1 ROSS ME ANN INTERN MED 81 566 974 2 ODONNEL MD BIOL GASTROENTEROL 8 354 975 3 GOLDBERG DM DIGESTION 13 56 975 4 MACGREGOR IL AUST NZ J MED 6 551 976 5 ALHADEFF JA CLIN GENET 10 63 976 6 SKUDE G SCAND J GASTROENTEROL 11 525 976 7 LEGAZ ME CLIN CHEM 22 57 976 8 OTSUKI M CLIN CHEM 22 439 976 9 SKUDE G ACTA PAEDIATR SCAND 65 145 976 10 WOLF RO AM J CLIN PATHOL 65 1022 976 11 LEHRNER LM AM J CLIN PATHOL 66 576 976 12 WOLF RO J LAB CLIN MED 87 164 976 13 WOOD RE AM REV RESPIR DIS 113 833 976 14 HUBBARD VS LANCET 2 1302 977 15 LEBENTHAL E CLIN GASTROENTEROL 6 397 977 16 MERRITT AD ADV HUM GENET 8 135 977 17 MAGID E SCAND J GASTROENTEROL 12 621 977 18 SKUDE G SCAND J GASTROENTEROL 12 3 977 19 SKUDE G SCAND J GASTROENTEROL 12 53 977 20 SKUDE G SCAND J GASTROENTEROL 12 673 977 21 GOWENLOCK AH ANN CLIN BIOCHEM 14 61 977 22 TAKACS O ACTA PAEDIATR ACAD SCI HUNG 18 21 977 23 ANDORSKY M AM J DIG DIS 22 56 977 24 ODONNELL MD CLIN CHEM 23 560 977 25 TAUSSIG LM AM J HUM GENET 29 408 977 26 TOWNES PL AM J HUM GENET 29 408 977 27 LEVITT MD J LAB CLIN MED 90 141 977 28 OTSUKI M EUR J PEDIATR 125 175 977 29 FINK R CLIN ENDOCRINOL METAB 7 297 978 30 DAVIDSON GP PEDIATR RES 12 967 978 31 JOHNSON SG AM J DIG DIS 23 914 978 32 STERN RC JAMA 239 2676 978 33 DONALDSON LA GUT 20 216 979 34 BERK JE DIG DIS SCI 24 6 979 35 SANTAGNESE PAD AM J MED 66 121 979 36 DERUGIN N AM J GASTROENTEROL 72 416 979 37 WOOD RE SOUTH MED J 72 189 979 38 GILLARD BK PEDIATR RES 14 1168 980 39 FOO Y J CLIN PATHOL 33 1102 980 40 DAVIS PB AM J MED 69 643 980 41 DAVIS PB J LAB CLIN MED 96 75 980 42 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 43 PARK RW GASTROENTEROLOGY 81 1143 981 44 GOFF JS WEST J MED 135 368 981 45 TOBIN MJ IR J MED SCI 150 325 981 46 MITCHELL EA AUST PAEDIATR J 18 118 982 47 GILLARD BK CLIN CHEM 29 1119 983 48 JUNGLEE D CLIN CHEM 29 2003 983 49 KOOP H CLIN GASTROENTEROL 13 739 984 50 GILLARD BK AM J DIS CHILD 138 577 984 51 WINTER WE J PEDIATR 109 465 986 PN 74152 RN 00151 AN 74176950 AU Lillibridge-C-B. Brown-M-R. Hall-J-G. TI The electron microscopic appearance of presecreted gastric mucus in cystic fibrosis. SO Pediatrics. 1974 Jun. 53(6). P 913-9. MJ CYSTIC-FIBROSIS: pp. GASTRIC-MUCOSA: se. MUCUS. MN ANALYSIS-OF-VARIANCE. BIOPSY. CHILD. CYSTIC-FIBROSIS: di. GASTRIC-MUCOSA: pp. HUMAN. MICROSCOPY-ELECTRON. VISCOSITY. AB Gastric mucosal biopsies from eight new untreated patients with cystic fibrosis (CF) and two normal subjects were studied by quantitative techniques of electron microscopy (EM) to determine whether presecreted gastric mucus was more compact (viscid) than normal. Analysis of variance techniques show no differences between means of all groups studied. These observations suggest that mucus may not be viscid prior to secretion. Electron microscopy of gastric mucus does not appear to be of diagnostic value in CF. RF 001 MANDEL ID AM J DIS CHILD 113 431 967 002 CHERNICK WS J PEDIATR 65 694 964 003 HADORN B CAN MED ASSOC J 98 377 968 004 MATTHEWS LW AM REV RESPIR DIS 88 199 963 005 FIELDING JF LANCET 2 423 970 006 GIBSON LE PEDIATRICS 48 695 971 007 GUGLER EC J PEDIATR 71 585 967 008 LILLIBRIDGE CB GASTROENTEROLOGY 47 269 964 009 LILLIBRIDGE CB IN: GLASS GBJ 22 968 010 BRANDBORG LL GASTROENTEROLOGY 37 1 959 011 BENNETT HS J BIOPHYS BIOCHEM CYTOL 6 113 959 012 LUFT JH J BIOPHYS BIOCHEM CYTOL 9 409 961 013 RICHARDSON KC STAIN TECHNOL 35 313 960 014 REYNOLDS ES J CELL BIOL 17 208 963 015 WETHERILL BG ELEMENTARY STATISTICAL ME 276 967 016 DENTON R PEDIATRICS 25 611 960 017 BAUER U ANN PAEDIATR 194 236 960 018 MANDEL ID AM J DIS CHILD 110 646 965 019 POTTER JL J PEDIATR 61 297 962 020 CHERNICK WS ANN NY ACAD SCI 106 698 963 021 DISCHE Z PEDIATRICS 24 74 959 022 DISCHE Z AM J DIS CHILD 102 733 961 023 JOHANSEN PG ANN NY ACAD SCI 106 755 963 024 ROELFS RE AM J DIS CHILD 113 419 967 025 DOGGETT RG J CLIN PATHOL 24 270 971 026 JOHANSEN PG J PATHOL 99 299 969 027 BLOMFIELD J GUT 14 558 973 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 SCHMIDT KD EUR J PEDIATR 136 193 981 3 BRIDGES MA ANN NY ACAD SCI 421 360 983 4 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 5 SEYMOUR CA BIOESSAYS 1 38 984 6 GOSDEN CM LANCET 2 541 984 7 MORRIS GP VIRCHOWS ARCH CELL PATHOL 46 239 984 8 QURESHI AR J PEDIATR 106 913 985 9 RUBINSTEIN S PEDIATRICS 78 473 986 PN 74153 RN 00152 AN 75176642 AU McAllister-T-A. TI Gentamicin in paediatrics. SO Postgrad-Med-J. 1974 Nov. 50 Suppl 7. P 45-52. MJ GENTAMICINS: tu. MN AGE-FACTORS. BACTERIA: de. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: dt, mi. ENTEROBACTERIACEAE-INFECTIONS: dt. FEMALE. GENTAMICINS: ae, ad. HUMAN. INFANT. INFANT-NEWBORN. LEUKEMIA: co. MALE. SEPTICEMIA: dt, mi. URINARY-TRACT-INFECTIONS: mi. AB Gentamicin is a broad-spectrum, bactericidal aminoglycoside antibiotic in its tenth year of use in the U.K. League tables of antibiotics, ranked by disc sensitivity tests against urinary tract pathogens and Staph. aureus confirm its remarkable effectiveness in 1973. Bacteriodes sp. and some streptococci are the only major omissions from its spectrum. MIC of sensitive strains is usually less than or equal to 2 microg/ml although Pseudomonas may require greater than or equal to 4 microg/ml. The therapeutic range aimed for is 2-12 microg/ml in serum and peak levels are measured 1 hr after intramuscular and 20 min after intravenous injection. Most workers regard a level of 12 microg/ml as potentially toxic and try to avoid exceeding it. RF 001 ABSTON S J INFECT DIS SUPPL 124 275 971 002 BARBER M BR MED J 1 203 966 003 BLOCKEY NJ J BONE JOINT SURG 54B 299 972 004 BLOOM SR POSTGRAD MED J 45 761 969 005 BULGER RJ AM J MED SCI 246 717 963 006 BULGER RJ ANN INTERN MED 59 593 963 007 BUSUTTIL AA LANCET 1 264 973 008 CLANCY MT J IR MED ASSOC 65 514 972 009 DARRELL JH BR MED J 4 427 972 010 EYKYN S LANCET 1 545 971 011 FILLASTRE JP BR MED J 2 396 973 012 GINGELL JC BR MED J 2 19 968 013 HOLT RJ ARCH DIS CHILD 43 329 968 014 ILLINGWORTH RS PRESCRIBERS' JOURNAL 13 124 973 015 JACKSON GG J INFECT DIS SUPPL 124 65 971 016 JAO RL ANTIMICROB AGENTS CHEMOTHER 3 148 963 017 KABINS SA J INFECT DIS SUPPL 124 65 971 018 KLASTERSKY J LANCET 1 653 971 019 KLEIN JO J INFECT DIS SUPPL 124 224 971 020 ANON LANCET 1 191 973 021 LEVISON ME LANCET 2 45 971 022 LEWIS JE NATURE NEW BIOL 239 214 972 023 LIGHTBOWN JB IN: WATT PJ 5 970 024 LYNN B LANCET 1 654 971 025 MCALLISTER TA LANCET 1 1520 973 026 MCALLISTER TA IN: FORFAR JO 1795 973 027 MCALLISTER TA POSTGRAD MED J SEPT SUPPL 47 7 971 029 MCHENRY MC J INFECT DIS SUPPL 124 164 971 030 MCLAUGHLIN JE LANCET 1 261 971 031 MACMILLAN BG J INFECT DIS SUPPL 124 278 971 033 MILNER RDG POSTGRAD MED J SUPPL 7 50 40 974 034 MILNER RDG ARCH DIS CHILD 47 927 972 035 NEWMAN RL MR MED J 1 539 967 036 NOONE P BR MED J 1 477 974 037 NOONE P LANCET 2 1194 972 038 NOONE P LANCET 1 316 973 039 RIFF LJ J INFECT DIS SUPPL 124 98 971 040 RIFF LJ LANCET 1 592 971 041 SMITH DH LANCET 1 252 967 042 SMITH DH N ENGL J MED 286 583 972 043 SONNE M APPL MICROBIOL 17 893 969 044 STRATFORD BC LANCET 1 378 974 045 WEINSTEIN MJ ANTIMICROB AGENTS CHEMOTHER 3 1 963 CT 1 MCALLISTER TA SCOTT MED J 20 97 975 2 RAINE PAM SCOTT MED J 21 66 976 3 TAYLOR M ARCH DIS CHILD 51 369 976 4 RAINE PAM J INFECT DIS 134 S165 976 PN 74154 RN 00153 AN 75019344 AU Francis-D-E. TI Therapeutic special diets. SO Practitioner. 1974 Apr. 212(1270 Spec No). P 545-51. MJ DIET-THERAPY. MN CARBOHYDRATE-METABOLISM-INBORN-ERRORS: dh. CELIAC-DISEASE: dh. CYSTIC-FIBROSIS: dh. DIABETES-MELLITUS: dh. GALACTOSEMIA: dh. HUMAN. NUTRITIONAL-REQUIREMENTS. OBESITY: dh. PHENYLKETONURIA: dh. EX The aim of a therapeutic diet is to provide a nutritionally adequate diet from a variety of natural and/or specially prepared artificial food substitutes for the treatment of specific clinical disorders. The role of the dietitian is to work as a member of the medical team. Paediatric dietetics require rather more detailed advice and help for the parents, particularly when the diet involves calculation or restriction of specific nutrients. Dietary considerations in obesity, diabetes mellitus, coeliac disease, cystic fibrosis, carbohydrate intolerance, galactosaemia, and phenylketonuria are discussed. RF 001 ANDERSON CM ARCH DIS CHILD 40 1 965 002 ANDERSON CM ARCH DIS CHILD 41 571 966 003 ANDERSON CM ARCH DIS CHILD 47 292 972 004 COURT JM YOUR CHILD HAS DIABETES 972 005 EID EE BR MED J 2 74 970 006 FISCH RO AM J DIS CHILD 112 3 966 008 HAEMMERLI UP AM J MED 38 7 965 009 KOMROWER GM ARCH DIS CHILD 45 367 970 010 ANON BR MED J 2 64 970 011 LIFSHITZ F J PEDIATR 77 595 970 012 MANN TP ARCH DIS CHILD 40 364 965 013$ ANON MIMS 014 SHELDON W BR MED J 2 401 969 015 SUTHERLAND BS JAMA 211 270 970 016 TAITZ LS NUTRITION 26 339 972 PN 74155 RN 00154 AN 75065745 AU Houck-J-C. Cheng-F-L. TI Defects in the cell cycle of fibroblasts obtained from patients with cystic fibrosis. SO Proc-Soc-Exp-Biol-Med. 1974 Oct. 147(1). P 167-70. MJ CYSTIC-FIBROSIS: pa. FIBROBLASTS: pa. MN AUTORADIOGRAPHY. CELL-DIVISION. CELL-LINE. CELLS-CULTURED. CULTURE-MEDIA. HUMAN. LUNG. THYMIDINE: me. TRITIUM. EX This paper analyzes the cell cycle of cystic fibrosis-derived fibroblasts and statistically demonstrates their failure to support a normal population doubling time in vitro and indicates that this is due largely to a failure of the cystic fibrosis-derived cells to enter the cell cycle in normal numbers. We have no explanation for the significant difference in the behavior of these cells from patients with cystic fibrosis vis-a-vis either normal or disease control fibroblasts, save to conclude that it proceeds from an intrinsic property of the cells themselves. RF 001 DANES BS J EXP MED 129 775 969 002 RAFF EC J CELL PHYSIOL 74 235 969 003 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 004 DI SANTAGNESE PA N ENGL J MED 277 1287 967 005 MAK S EXP CELL RES 39 286 965 006 STANNERS CP BIOCHIM BIOPHYS ACTA 37 406 960 007 HOUCK JC PROC SOC EXP BIOL MED 134 22 970 CT 1 GOSPODAROWICZ D J CELL BIOL 66 451 975 2 BOLTON WE TEX REP BIOL MED 34 97 976 3 DANES BS TEX REP BIOL MED 34 135 976 4 WARD JB TEX REP BIOL MED 34 11 976 5 ANON J PEDIATR 88 711 976 6 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 7 DISANTAGNESE PA N ENGL J MED 295 534 976 8 KONYUKHOV BV IZVES AKAD NAUK SSSR SER BIOL 1977 747 977 9 NEUTRA MR GASTROENTEROLOGY 75 701 978 10 SHAPIRO BL CLIN CHIM ACTA 82 125 978 11 JAKEL HP BIOL ZENTRALBL 98 55 979 12 SHAPIRO BL SCIENCE 203 1251 979 13 VRACKO R IN VITRO 19 504 983 PN 74156 RN 00155 AN 74116849 AU Alderson-P-O. Secker-Walker-R-H. Strominger-D-B. McAlister-W-H. Hill-R-L. Markham-J. TI Quantitative assessment of regional ventilation and perfusion in children with cystic fibrosis. SO Radiology. 1974 Apr. 111(1). P 151-5. MJ CYSTIC-FIBROSIS: pp. RADIONUCLIDE-IMAGING. VENTILATION-PERFUSION-RATIO. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. COMPUTERS. CYSTIC-FIBROSIS: ra. EVALUATION-STUDIES. FEMALE. HUMAN. INFANT. MALE. RESPIRATORY-FUNCTION-TESTS. TECHNETIUM. XENON. AB Twenty-five children aged 8 months to 17 years were studied to determine the usefulness of computer-assisted ventilation-perfusion (V-P) scanning in analysis of regional pulmonary function in cystic fibrosis. Ventilation and perfusion scintiphotographs were compared with the patients' radiographs. Regional fractional exchanges of air and relative regional V-P ratios were obtained. Whole-lung air-exchange was significantly correlated with standard pulmonary function tests and radiograph scores. Ventilation-perfusion ratio gradients were reversed in pats with predominantly upper zone disease, and regional V-P ratios were uneven in distribution. Quantitative assessment of regional ventilation and perfusion is a useful technique for assessing severity of pulmonary disease in children with cystic fibrosis. RF 001 LAMARRE A PEDIATRICS 50 291 972 002 HOGG JC N ENGL J MED 282 1283 970 003 HOGG JC N ENGL J MED 278 1355 968 004 GYEPES MT AM J ROENTG RAD THER NUCL MED 106 567 969 005 SECKER-WALKER RH PROC CONF COMPUTER APPLIC 3RD 972 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 TAUSSIG LM J PEDIATR 82 380 973 008 AVERY ME LUNG AND ITS DISORDER 40 964 009 KAMEL M SCAND J RESPIR DIS 50 125 969 010 MANSELL A J APPL PHYSIOL 33 711 972 011 MOSS AJ PEDIATRICS 41 438 968 012 KNIGHT L CLIN RES 21 674 973 013 JAMES AE AM J ROENTG RAD THER NUCL MED 111 492 971 014 REILLY BJ RADIOLOGY 98 281 971 CT 1 SECKERWALKER RH RESPIRATION 32 265 975 2 ALDERSON PO CIRCULATION 53 332 976 3 DZIUK E RADIOL DIAGN 18 533 977 4 GODFREY S ARCH DIS CHILD 52 859 977 5 SECKERWALKER RH J NUCL MED 19 961 978 6 CHIPPS BE CHEST 73 519 978 7 HOLSCLAW DS CLIN CHEST MED 1 407 980 8 PIEPSZ A J NUCL MED 21 909 980 9 CUNNINGHAM DA BR J RADIOL 54 110 981 10 GORDON I BR J RADIOL 54 576 981 11 COATES AL ARCH DIS CHILD 56 106 981 12 SECKERWALKER RH RESPIRATION 43 8 982 13 GUIDOTTI TL RESPIRATION 44 351 983 14 LOEUILLE GA SEM HOP PARIS 59 2043 983 15 GOODING CA J PEDIATR 105 384 984 16 DESMOND KJ PEDIATR PULMONOL 2 128 986 17 EVANS I EUR J NUCL MED 12 620 987 PN 74157 RN 00156 AN 75032798 AU Robbins-A-H. Messian-R-A. Widrich-W-C. Paul-R-E-Jr. Norton-R-A. Schimmel-E-M. Ogoshi-K. TI Endoscopic pancreatography: an analysis of the radiologic findings in pancreatitis. SO Radiology. 1974 Nov. 113(2). P 293-6. MJ CHOLANGIOGRAPHY. ENDOSCOPY. PANCREAS: ra. PANCREATITIS: ra. MN ADULT. CHOLANGIOGRAPHY: mt. CYSTIC-FIBROSIS: ra, co. DUODENUM. HUMAN. MALE. MIDDLE-AGE. PANCREATIC-DUCTS: ra. PANCREATITIS: di, et. AB The endoscopic pancreaticographic findings in 46 proved cases of pancreatitis are analyzed. Pathologic ductograms were demonstrated in approximately 60%. Significant information was obtained in many cases, allowing both proper diagnosis of the primary disease process and complications thereof. Because of the latter, the procedure has proved to be quite helpful in isolating those patients who could benefit from surgery. RF 001 BLUMGART LH LANCET 2 1269 972 002 COTTON PB LANCET 1 53 972 003 DICKINSON PB JAMA 225 944 973 004 KASUGAI T GASTROENTEROLOGY 63 217 972 005 KASUGAI T GASTROENTEROLOGY 63 217 972 006 KOZOWER M ANN SURG 178 197 973 007 MUJAHED Z RADIOL CLIN NORTH AM 4 535 966 008 OGOSHI K GASTROENTEROLOGY 64 210 973 009 ROBBINS AH AM J ROENTG RAD THER NUCL MED 117 432 973 010 STRACK PR N ENGL J MED 285 1225 971 011 WISE RE INTRAVENOUS CHOLANGIOGRAPHY 21 962 CT 1 KASUGAI T DIGESTION 13 76 975 2 PAUL RE SEM ROENTGENOL 11 223 976 3 FERRUCCI JT RADIOL CLIN NORTH AM 14 543 976 4 JABBARI M CAN J SURG 19 192 976 5 CREMER M ACTA GASTROENTEROL BELG 39 522 976 6 ENGELHOLM L ACTA GASTROENTEROL BELG 39 405 976 7 WARSHAW AL GASTROENTEROLOGY 70 562 976 8 BRUHLMANN W FORTSCHR GEB RONTG NUKL 124 542 976 9 FEINBERG SB J CLIN ULTRASOUND 5 96 977 10 COTTON PB GUT 18 316 977 11 CLASSEN M MED KLIN 72 684 977 12 CLARKE AC NZ MED J 86 514 977 13 DIMAGNO EP N ENGL J MED 297 737 977 14 HACKEN JB GASTROINTEST RADIOL 3 173 978 15 STANDERTSKJOLDNORDENS ANN CLIN RES 10 30 978 16 ROLNY P SCAND J GASTROENTEROL 13 777 978 17 MULLENS JE CAN J SURG 21 60 978 18 TAGUCHI K CAN J SURG 21 313 978 19 GO VLW MED CLIN NORTH AM 62 129 978 20 MULLENS JE SURGERY 84 308 978 21 KLINGER J REV MED CHIL 106 277 978 22 NELSON EW AM J SURG 136 740 978 23 SIEGEL JH AM J GASTROENTEROL 72 259 979 24 WEINSTEIN DP RADIOLOGY 130 729 979 25 FISHMAN A AM J ROENTGENOL 133 225 979 26 ROLNY P SCAND J GASTROENTEROL 15 1 980 27 SIMEONE JF INVEST RADIOL 15 6 980 28 EPSTEIN RJ GASTROINTEST ENDOSC 26 98 980 29 SWENSEN T BR J RADIOL 53 760 980 30 WEINSTEIN BJ RADIOLOGY 134 185 980 31 GOLD RP GASTROINTEST RADIOL 6 35 981 32 VALENTINI M ENDOSCOPY 13 64 981 33 LUKES PJ ACTA RADIOL DIAGN STOCKH 22 145 981 34 CREAGHE SB AM SURGEON 47 243 981 35 CALETTI G BR J SURG 69 507 982 36 MASARYK TJ DIG DIS SCI 28 874 983 37 SWOBODNIK W KLIN WSCHR 61 291 983 38 KARASAWA E RADIOLOGY 148 489 983 39 DARNIS E ANAT CLIN 6 109 984 40 CLASSEN M CLIN GASTROENTEROL 13 819 984 41 TRILLER J FORTSCHR GEB RONTG NUKL 141 483 984 42 AXON ATR CLIN GASTROENTEROL 15 279 986 PN 74158 RN 00157 AN 74137595 AU Melhorn-D-K. TI Vitamin E: who needs it?. SO RI-Med-J. 1974 Mar. 57(3). P 100-3 passim. MJ MALABSORPTION-SYNDROMES: co. NUTRITION-DISORDERS: co. VITAMIN-E-DEFICIENCY: co. MN CHILD. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. INFANT. LIPID-METABOLISM-INBORN-ERRORS: co. VITAMIN-E: me. VITAMIN-E-DEFICIENCY: et. EX This section considers the significance of vitamin E (tocopherol) deficiency in a variety of disease states in which the effects of the vitamin lack are unclear. Causes of vitamin E deficiency include dietary lack and intestinal malabsorption. A case history involving cystic fibrosis is examined. RF 001 MELHORN DK OHIO STATE MED J 69 899 973 002 MAJAJ AS AM J CLIN NUTR 18 362 966 003 HORWITT MK ANN NY ACAD SCI 203 223 972 004 FILER LJ JR PEDIATRICS 8 328 951 005 QUAIFE ML J BIOL CHEM 180 1229 949 006 LUDWIG MI PHYSIOLOGICAL ACTIVITY OF D A 665 959 007 GORDON HH AM J DIS CHILD 90 669 955 008 STOCKS J BR J HAEMATOL 20 95 971 009 BIERI JG INT J VITAM RES 40 344 970 010 KAYDEN HJ ANN NY ACAD SCI 203 127 972 011 BINDER HJ N ENGL J MED 273 1289 965 012 SCHNITZER B AM J CLIN PATHOL 50 443 968 013 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 014 WITTING LA ANN NY ACAD SCI 203 192 972 PN 74159 RN 00158 AN 74298265 AU Hellsing-K. Kollberg-H. TI Analysis of albumin in meconium for early detection of cystic fibrosis. A methodological study. SO Scand-J-Clin-Lab-Invest. 1974 Jun. 33(4). P 333-40. MJ MECONIUM: an. ALBUMINS: an. CYSTIC-FIBROSIS: di. MN INFANT-NEWBORN. HUMAN. CYSTIC-FIBROSIS: me. GEL-DIFFUSION-TESTS. POLYSACCHARIDES. TEMPERATURE. METHODS. EVALUATION-STUDIES. SPECIMEN-HANDLING. AB Albumin in meconium was quantitated by means of a single radial immunodiffusion technique, which is described in detail. The method shows good precision and accuracy for this material, when certain conditions associated with collection, preparation, and analysis are observed. The method can discriminate between healthy newborns and newborns with cystic fibrosis. RF 001 ANDERSEN GH TRANS AM NUCL SOC 10 57 967 002 BENDER SW MONATSSCHR KINDERHEILKD 119 632 971 003 CAIN ARR ARCH DIS CHILD 47 131 972 004 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 GLANZMANN E ANN PAEDIATR 175 33 950 008 GOLDBLOOM RB N ENGL J MED 269 1349 963 009 GREEN MN PEDIATRICS 21 635 958 010 GREEN MN PEDIATRICS 41 989 968 011 GRUNDIG CA ACTA BIOL MED GER 19 193 967 012 HARBOE N SCAND J IMMUNOL SUPPL 1 2 161 973 013 HARRISON GM CF CLUB ABST 11 26 970 014 HELLSING K ACTA CHEM SCAND 20 1251 966 015 HEROLD W MONATSSCHR KINDERHEILKD 117 626 969 016 HOBBS JR PROTIDES BIOL FLUIDS 17 517 969 017 KOLLBERG H ARCH DIS CHILD 47 836 972 019 KOLLBERG H ACTA PAEDIATR SCAND 63 405 974 020 LAURELL CB SCAND J CLIN LAB INVEST SUPPL 124 21 972 021 LAWSON D ARCH DIS CHILD 42 689 967 022 MANCINI G IMMUNOCHEMISTRY 2 235 965 023 RAPOPORT S SCIENCE 112 150 950 024 SCHACHTER H CAN J BIOCHEM 43 381 965 025 SCHUTT WH ARCH DIS CHILD 43 178 968 026 SHWACHMAN H PEDIATRICS 32 85 963 027 SHWACHMAN H PEDIATRICS 46 335 970 028 STAMM SJ TERMINAL REPORT TO WASHINGTON 971 029 STEPHAN U PADIATRISCHE PRAXIS 12 487 973 030 WARWICK WJ PEDIATRICS 36 261 965 031 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 032 WISER WC PEDIATRICS 33 115 964 033 YOUNG DM PROC SOC EXP BIOL MED 99 673 958 CT 1 RAINE DN LANCET 2 996 974 2 STEPHAN U LANCET 1 167 975 3 PECAU Y BIOL GASTROENTEROL 8 193 975 4 PECAU Y ARCH FR PEDIATR 32 733 975 5 FEIGELSON J SEM HOP PARIS 51 741 975 6 STEPHAN U PEDIATRICS 55 35 975 7 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 8 RYLEY HC CLIN CHIM ACTA 64 117 975 9 LANZA I MINERVA PEDIATR 28 1510 976 10 ROMAGNOLI C MINERVA PEDIATR 28 13 976 11 ANON J PEDIATR 88 711 976 12 ROMAGNOLI C MINERVA PEDIATR 29 809 977 13 BRUNS WT AM J DIS CHILD 131 71 977 14 FIFI AR MINERVA PEDIATR 30 597 978 15 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 16 BERRY HK AM J DIS CHILD 134 930 980 17 CRISTOL P SEM HOP PARIS 58 449 982 18 HELLSING K ACTA PAEDIATR SCAND 71 827 982 PN 74160 RN 00159 AN 75102542 AU Hoff-G-E. Schiotz-P-O. Paulsen-J. TI Tobramycin treatment of pseudomonas aeruginosa infections in cystic fibrosis. SO Scand-J-Infect-Dis. 1974. 6(4). P 333-7. MJ AMINOGLYCOSIDES: tu. ANTIBIOTICS: tu. CYSTIC-FIBROSIS: dt. PSEUDOMONAS-AERUGINOSA: de. MN ABSORPTION. ADOLESCENCE. AMINOGLYCOSIDES: bl, ad, ae. ANTIBIOTICS: bl, ad, ae. CHILD. CHRONIC-DISEASE. FEMALE. HUMAN. RESPIRATORY-THERAPY. INJECTIONS-INTRAMUSCULAR. MALE. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-TRACT-INFECTIONS: dt. SPUTUM: mi. TIME-FACTORS. AB Tobramycin treatment of chronic pulmonary infections caused by Pseudomonas aeruginosa in 13 children with cystic fibrosis was evaluated. Initially the patients received the recommended dose of 125 mg/m2/24 hours; the dose was then increased to 250 mg/m2/24 hours (approximately 10 mg/kg/24 hours). This higher dosage was administered intramuscularly to 9 patients. Eight of these received additional therapy with an aerosol containing tobramycin. With this therapy it was possible to eradicate Ps. aeruginosa from the respiratory tract in 5 of the patients. After discontinuing therapy Ps. aeruginosa re-occurred in all patients within one month. All patients showed a clinical improvement in relation to therapy. No toxic or allergic side effects were observed. RF 001 BLACK HR ANTIMICROB AGENTS CHEMOTHER 11 314 971 002 BRUMMET RE ARCH OTOLARYNGOL 94 59 971 003 BRUSCH JL ANTIMICROB AGENTS CHEMOTHER 1 280 972 004 DIAZ F J INFECT DIS 121 269 970 005 DIENSTAG J ANTIMICROB AGENTS CHEMOTHER 1 41 972 006 GIBSON LE PEDIATRICS 23 545 959 007 LAURELL CB CLIN CHEM 2 99 956 008 MEYER RD APPL MICROBIOL 22 1147 971 009 PRESTON DA ANTIMICROB AGENTS CHEMOTHER 11 322 971 010 ROSDAHL VT DAN MED BULL 16 133 969 011 WELLES JS TOXICOL APPL PHARMACOL 22 332 972 012 WRIGHT BM BR MED J 2 1041 959 CT 1 HOIBY N SCAND J RESPIR DIS 56 38 975 2 BROGDEN RN DRUGS 12 166 976 3 BENDUSH CL J INFECT DIS 134 S219 976 4 NEU HC J INFECT DIS 134 S 3 976 5 PERKINS RL AM J MED SCI 271 297 976 6 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 7 HOIBY N SCAND J RESPIR DIS 58 65 977 8 LAU WK PEDIATRICS 60 372 977 9 ATTERHOLM I SCAND J INFECT DIS 10 229 978 10 SCHIOTZ PO HUM HERED 28 293 978 11 THOMSEN J J ANTIMICROB CHEMOTHER 5 257 979 12 PAPORISZ U MONOGR PAEDIATR 10 31 979 13 FRIIS B SCAND J INFECT DIS 11 211 979 14 PAPORISZ U EUR J PEDIATR 130 259 979 15 MARKOWITZ SM J ANTIMICROB CHEMOTHER 6 251 980 16 MARTIN AJ ARCH DIS CHILD 55 604 980 17 WIENTZEN R AM J DIS CHILD 134 1134 980 18 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 19 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 20 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 21 HODSON ME BR J DIS CHEST 77 71 983 22 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 23 KUHN RJ CLIN PHARMACY 4 555 985 24 CONWAY SP ACTA PAEDIATR SCAND 74 107 985 25 MENDELMAN PM AM REV RESPIR DIS 132 761 985 26 WIJNANDS WJA J ANTIMICROB CHEMOTHER 18 719 986 27 STOUT SA DRUG INTEL CLIN PHARM 21 322 987 28 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 74161 RN 00160 AN 74270254 AU McCrae-W-M. Raeburn-J-A. TI The patterns of infection in cystic fibrosis. SO Scott-Med-J. 1974 Jul. 19(4). P 187-90. MJ CYSTIC-FIBROSIS: co. RESPIRATORY-TRACT-INFECTIONS: et. MN INFANT. CHILD-PRESCHOOL. CHILD. ADOLESCENCE. HUMAN. MALE. FEMALE. RESPIRATORY-TRACT-INFECTIONS: im. IGG: an. IGA: an. IGM: an. GEL-DIFFUSION-TESTS. HAEMOPHILUS-INFLUENZAE: im. HAEMOPHILUS-INFECTIONS: im, et. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: im, et. STREPTOCOCCUS: im. STREPTOCOCCAL-INFECTIONS: im, et. AB Thirty-five patients with cystic fibrosis (aged 11 months to 13.5 years) have been investigated with regard to respiratory symptoms, respiratory tract pathogens, serum immunoglobulins and serum precipitins. Haemophilus influenzae was the commonest pathogen being supplanted in advanced disease by Pseudomonas aeruginosa. The patients who had no respiratory infection had levels of IgG significantly lower than normal children of comparable ages. In children with severe respiratory infection the immunoglobulins were raised and precipitins to the predominant respiratory organism were present. These findings differ from the prevalent view that patients with cystic fibrosis have a normal response to infection and that the common infecting organism is Staphylococcus aureus. RF 001 ALLANSMITH M J PEDIATR 72 276 968 002 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 003 BURNS MW LANCET 1 270 968 004 FAHEY JL J IMMUNOL 94 84 965 005 GREEN MN AM J DIS CHILD 100 365 960 006 HUANG NN J PEDIATR 59 512 961 007 IACOCCA VF AM J DIS CHILD 106 315 963 008 LAWSON D IN: WATT PJ 69 970 009 LAWSON D ARCH DIS CHILD 47 1 972 010 MAY JR ARCH DIS CHILD 47 908 972 011 MEARNS MB ARCH DIS CHILD 47 5 972 012 RAEBURN JA SCAND J INFECT DIS 5 135 973 013 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 014 SCHWARTZ RH AM J DIS CHILD 111 408 966 CT 1 MITCHELLHEGGS P Q J MED 45 479 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 RAEBURN JA PROC NUTR SOC 36 77 977 4 LAU WK PEDIATRICS 60 372 977 5 BOYE NP EUR J RESPIR DIS 61 227 980 6 MATTHEWS WJ N ENGL J MED 302 245 980 7 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 PN 74162 RN 00161 AN 74258320 AU Anderson-E-L. TI Susceptibility of mucoid Pseudomonas aeruginosa to gentamicin disks. SO South-Med-J. 1974 Jul. 67(7). P 771-2. MJ PSEUDOMONAS-AERUGINOSA: de. GENTAMICINS: pd. PHARYNX: mi. SPUTUM: mi. MN HUMAN. PSEUDOMONAS-AERUGINOSA: ip. MICROBIAL-SENSITIVITY-TESTS. CYSTIC-FIBROSIS: mi. AB To compare the in vitro susceptibility of mucoid and nonmucoid Pseudomonas aeruginosa to gentamicin, 144 isolates of both mucoid and nonmucoid P aeruginosa were cultured from throat swab and sputum specimens from 29 patients with diagnosed cystic fibrosis. Mucoid forms of P aeruginosa from throat swab cultures were all susceptible to gentamicin, whereas only 85.5% of the mucoid strains from sputum cultures were susceptible. Nonmucoid strains from throat swab and sputum cultures were 85.1% and 77.8% susceptible, respectively. Since the differences in susceptibility between the four groups of organisms were not significant (P < 0.05), it was concluded that these data show no real differences in susceptibility to gentamicin between the mucoid and nonmucoid strains. RF 001 COX CE MED CLIN NORTH AM 54 1305 970 002 GATMAITAN BG AM J MED SCI 260 90 970 003 BAUER AW AM J CLIN PATHOL 45 493 966 004 GILARDI GL APPL MICROBIOL 16 1497 968 005 KIRBY WMM J INFECT DIS 119 361 969 006 TRAUB WH APPL MICROBIOL 20 98 970 007 DOGGETT RG APPL MICROBIOL 18 936 969 008 DOGGETT RG J BACTERIOL 87 427 964 009 HUANG NN J PEDIATR 78 338 971 010 SCHWARZMANN S INFECT IMMUN 3 762 971 CT 1 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 2 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 PN 74163 RN 00162 AN 74162052 AU Reiderman-M-I. TI Some materials on the genetics of mucoviscidosis. SO Sov-Genet. 1974 Mar 1. 7(12). P 1608-12. MJ CHROMOSOMES. CYSTIC-FIBROSIS: fg. MN ADULT. CHILD. CONSANGUINITY. FEMALE. GENES-RECESSIVE. HETEROZYGOTE. HUMAN. MALE. PEDIGREE. POLYMORPHISM-GENETICS. PROBABILITY. SELECTION-GENETICS. VARIATION-GENETICS. EX The purpose of the present work was to study the type of inheritance involved in mucoviscidosis. Analysis of the genetic relations in mucoviscidosis performed on 26 families bears witness to a monomeric autosomal mechanism of transmission of the character. In contrast to other recessive diseases, parental consanguinity plays a secondary role in mucoviscidosis, which apparently is explained by the wide distribution of the mucoviscidosis gene among the population. The observations described may be utilized in medicogenetic consultations given to members of those families where there are children suffering from mucoviscidosis. RF 001 FANCONI G WIEN MED WOCHENSCHR 86 753 936 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 DI SANTAGNESE PA JAMA 160 846 956 004 WARWICK WJ JAMA 198 177 966 005 WINDORFER A MUCOVISCIDOSE CYSTISCHE FIBR 968 006 BAUMANN TH MUKOVISCIDOSIS ALS RECESS 958 007 BERNHEIM M PEDIATRIE 16 17 961 008 NETAKHATA ZH N PEDIATRIYA 5 45 965 009 REZNIK B YA VOPR OKHR MATERIN DET 76 970 010 WALD J IN: BOCHKOV NP 130 970 011 STERN C PRINCIPLES OF HUMAN GENETIC 960 012 SAPELKINA LV VOPR OKHR MATERIN DET 74 963 013 ANDERSEN DH AM J DIS CHILD 72 62 946 014 PETER G LANCET 1 978 968 015 BLANC WA LANCET 1 1152 968 016 DANKS DM ANN HUM GENET 28 323 965 017 STEINBERG AG AM J HUM GENET 12 416 960 018 HOUSTEK J CESK PEDIATR 17 445 962 019 REIDERMAN MI GENETIKA (SOV GENETICS) 7 148 971 020 CHILDS B ROSS PAEDIATR RES CONF 18TH 87 956 021 STEINBERG AG IN: GOLDSCHMIDT E 133 963 022 DANES BS LANCET 1 1061 968 023 MERRELL DJ J HERED 60 180 969 PN 74164 RN 00163 AN 75141827 AU Azizi-F. Bentley-D. Vagenakis-A. Portnay-G. Bush-J-E. Shwachman-H. Ingbar-S-H. Braverman-L-E. TI Abnormal thyroid function and response to iodides in patients with cystic fibrosis. SO Trans-Assoc-Am-Physicians. 1974. 87. P 111-9. MJ CYSTIC-FIBROSIS: dt. IODIDES: tu. THYROID-GLAND: pp. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: bl, co. FEMALE. GOITER: et. HUMAN. HYPOTHYROIDISM: et. MALE. RESPIRATORY-TRACT-INFECTIONS: pc. SULFISOXAZOLE: tu. THYROTROPIN: bl. THYROXINE: bl. TRIIODOTHYRONINE: bl. EX It has been suggested that the development of goiter and hypothyroidism in patients receiving chronic iodide therapy indicates that they have underlying thyroid disease. In 1971, Dolan and Gibson, in a retrospective study, reviewed the records of children with cystic fibrosis receiving chronic expectorant therapy with iodide and reported that approximately 85 percent had goiter and 15 percent suggestive clinical findings of hypothyroidism. This study, together with the fact that the thyroid, like lung and pancreas, is of endodermal origin raised the possibility that patients with cystic fibrosis might have an underlying defect in thyroid function. As thyroid function in patients with cystic fibrosis has not been evaluated in a systematic fashion, the present study was undertaken. In it, we have evaluated basal thyroid function in a large group of patients with cystic fibrosis and have, in addition, observed the effects of chronic iodide administration. The present study strongly suggests that there often occurs in patients with cystic fibrosis some derangement in thyroid hormone economy. The frequency with which iodides increased serum thyrotropin, decreased serum thyroxine concentration, and induced goiter, with or without hypothyroidism, in patients with cystic fibrosis, points strongly to the presence of an intrinsic abnormality of the thyroid in this disease. The nature of such abnormality remains unclear. RF 001 BRAVERMAN LE N ENGL J MED 281 816 969 002 BRAVERMAN LE TRANS ASSOC AM PHYSICIANS 84 130 971 003 BRAVERMAN LE J CLIN ENDOCRINOL METAB 32 515 971 004 DOLAN TF JR J PEDIATR 79 684 971 005 BRAVERMAN LE J CLIN ENDOCRINOL METAB 32 497 971 006 MITSUMA T BIOCHEM BIOPHYS RES COMMUN 46 2107 972 007 ODELL WD RECENT PROGR HORM RES 23 47 967 008 AZIZI F ANN INTERN MED 80 194 974 009 BIRD T J CLIN PATHOL 26 623 973 010 SNEDECOR GW STATISTICAL METHODS APPLIED T 956 011 MILNE K ENDOCRINOLOGY 71 580 962 012 BRAVERMAN LE J CLIN INVEST 49 855 970 013 SURKS MI J CLIN INVEST 52 805 973 014 SULLIVAN PRC J CLIN INVEST 52 83A 973 015 BERMUDEZ F CLIN RES 22 335A 974 016 CHOPRA IJ CLIN RES 22 337A 974 017 NORMURA S J CLIN INVEST 53 57A 974 018 PORTNAY G J CLIN INVEST 52 61A 974 019 FALLIERS CJ J ALLERGY 38 183 966 020 BOREL DM ARCH PATHOL 96 269 973 021 SMITH FR J LAB CLIN MED 80 423 972 CT 1 OSATHANONDH R J CLIN ENDOCRINOL METAB 42 98 976 2 AZIZI F N ENGL J MED 295 1381 976 3 DAVIS PB N ENGL J MED 295 1381 976 4 MILLER LJ GASTROENTEROLOGY 75 901 978 5 ROSENSTEIN BJ J PEDIATR 93 261 978 6 BARAN D MONOGR PAEDIATR 10 114 979 7 BARSANO CP ENVIRON HEALTH PERSPECT 38 71 981 8 SEGALLBLANK M J PEDIATR 98 218 981 9 CUKOR G J MED VIROL 9 161 982 10 DELUCA F EUR J PEDIATR 138 327 982 11 CUKOR G J CLIN MICROBIOL 18 457 983 12 SACK J ISR J MED SCI 19 17 983 13 ROBUSCHI G ACTA DIABETOL LAT 21 357 984 14 KNOPFLE G KLIN PAEDIATR 197 481 985 15 WIERSINGA WM BR MED J 293 106 986 PN 74165 RN 00164 AN 74141045 AU Waisbren-B-A. Martins-R-R. Bruns-W-T. Kurzynski-T-A. TI Whole cell heat-killed gram-negative bacilli vaccine. SO Wis-Med-J. 1974 Apr. 73(4). P S42-5. MJ BACTERIAL-INFECTIONS: pc. BACTERIAL-VACCINES: tu. ESCHERICHIA-COLI: im. PSEUDOMONAS-AERUGINOSA: im. VACCINATION. MN ANTIBODY-FORMATION. BURNS: im. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im. FEMALE. HEART-SURGERY. HEAT. HUMAN. MALE. EX This preliminary clinical study is of the ability of a multivalent heat-killed whole cell gram-negative bacilli vaccine to be tolerated and to evoke an antibody response in groups of patients who have a manifest need for increased resistance to gram-negative bacilli. Three groups of patients were studied: 35 burn patients; seven small children with cystic fibrosis; and 35 patients who were about to undergo coronary vein grafts. The results of vaccination were different in each of the three groups of patients that were vaccinated. RF 001 FINLAND M JAMA 170 2188 959 002 MYEROWITZ RL J INFECT DIS 124 239 971 003 SMITH DT ARCH INTERN MED 125 344 970 004 WRIGHT AE BR MED J 1 256 897 005 FISHER MW J BACTERIOL 98 835 969 006 KUNIN CM PROC SOC EXP BIOL MED 111 160 962 007 ALEXANDER JW ARCH SURG 99 249 969 008 WAISBREN BA CF CLUB ABST 12 971 009 WAISBREN BA J IMMUNOL 97 431 966 010 GORZYNSKI EA INFECT IMMUN 5 625 972 011 CROWDER JG J LAB CLIN MED 79 47 972 012 WAISBREN BA J INFECT DIS 119 518 969 013 SPRACKLEN FHN BR MED J 1 89 968 014 STEINBERG AD ANN INTERN MED 76 619 972 015 ALEXANDER JW J TRAUMA 6 780 966 016 WEISBERGER AS JAMA 209 97 969 017 BORTIN MM TRANSPLANTATION 12 573 971 018 WAISBREN BA PROC AM BURN ASS 4TH ANN MTG 972 CT 1 STANISLAVSKY ES ZH MIKROBIO EPIDEMIO IMMUNOBI 1983 22 983 PN 74166 RN 00165 AN 75123760 AU Underwood-B-A. TI The determination of vitamin A and some aspects of its distribution, mobilization and transport in health and disease. SO World-Rev-Nutr-Diet. 1974. 19. P 123-72. (REVIEW). MJ VITAMIN-A. MN ADOLESCENCE. ADULT. BIOLOGICAL-TRANSPORT. CARRIER-PROTEINS. CHILD. CYSTIC-FIBROSIS: me. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. LIVER: me. LYMPH: me. MALE. MIDDLE-AGE. PREGNANCY. VITAMIN-A: me. REVIEW. SPECTROMETRY-FLUORESCENCE. SPECTROPHOTOMETRY. SPECTROPHOTOMETRY-ULTRAVIOLET. VITAMIN-A: an, me. EX The author has attempted to evaluate some of the methodology currently in use or suggested for the routine determination of vitamin A in blood and tissues. Special consideration is given to extraneous factors which can cause erroneous results in fluorometric analyses. A review of the older and current literature on the distribution of vitamin A in tissues is presented. Special consideration is given to the uptake of vitamin A by the liver and its storage in hepatocytes and Kupffer's cell and in subcellular organelles. Data on the concentration of vitamin A in the liver of victims of accidental death and the effect of diseases of several etiologies are presented. The mechanism by which liver stores of retinyl esters are mobilized and released to the plasma remains obscure. Considerable information has been gained recently on the mechanism by which retinol is transported in blood. Studies leading to the isolation and characterization of retinol transport proteins are summarized. The metabolism of retinol binding protein in health and in disease, especially in cystic fibrosis, is discussed. Finally, some factors of importance to the interpretation of plasma levels in terms of vitamin A are reviewed. Special attention is given to the effect on plasma levels of retinol and the retinol transport proteins of periods of rapid growth (correlations with age) and the influence of dietary adequacy on the distribution of plasma levels of the vitamin. RF 001 ALVSAKER JO BIOCHEM J 102 362 967 002 ANDERSEN DH J PEDIATR 15 763 939 003 ANDREWS JS J BIOL CHEM 239 4073 964 004 ANON NATURE 228 309 970 005 ARROYAVE G AM J CLIN NUTR 22 1119 969 006 ARROYAVE G AM J CLIN NUTR 9 180 961 007 AWDEH ZL ANAL BIOCHEM 10 156 965 008 BEAL VA J NUTR 60 335 956 009 DI BENEDETTO RJ JAMA 201 700 967 010 BENNETT MJ AM J CLIN NUTR 20 415 967 011 BERGEN SS JR AM J CLIN NUTR 16 265 965 012 BESSEY OA J BIOL CHEM 166 177 946 013 BIERI JG IN: MARINETTI GV 2 459 969 014 BLACKFAN KD J PEDIATR 13 627 938 015 BONATI B ACTA VITAMIN 10 262 956 016 BRENNER S J NUTR 23 459 942 017 BRENNER S ARCH INTERN MED 71 474 943 018 BROWN HZ METABOLISM 1 349 952 019 BUDOWSKI P ANALYST 82 751 957 020 BUNNELL RH LIPIDS 6 245 971 021 CARR FH BIOCHEM J 20 497 926 022 CARROLL KK AM OIL CHEM SOC J 41 473 964 023 CASTER WO AM J CLIN NUTR 3 409 955 024 ANON TEN STATE NUTRITION SURVEY IN 971 026 DAGADU JM BR J NUTR 21 453 967 027 DAGADU JM LANCET 1 531 963 028 DAVIES AW BIOCHEM J 29 147 935 029 DELUCA L ARCH INTERN MED 127 853 971 030 DELUCA L J BIOL CHEM 245 4551 970 031 DELUCA HF FAT SOLUBLE VITAMINS 970 032 DELUCA L ARCH BIOCHEM BIOPHYS 123 1 968 033 DELUCA HF IN: MARINETTI GV 2 345 969 034 DEMOVSKY R PROC SOC EXP BIOL MED 118 158 965 035 DINGLE JT BIOCHEM J 79 509 961 036 DRUJAN BD ANAL BIOCHEM 23 44 968 037 DOWLING JE PROC NAT ACAD SCI USA 44 648 958 038 DUNAGIN PE JR ANAL CHEM 36 756 964 039 DZIALOSZYNSKI LM BIOCHEM J 39 63 945 040 DZIALOSZYNSKI LM EDINB MED J 54 252 947 041 ELLISON JB BIOCHEM J 31 165 937 042 ERLANSON C BIOCHIM BIOPHYS ACTA 167 629 968 043 ANON WHO TECH REP SER NO 362 967 044 FIDGE NH J LIPID RES 9 103 968 045 FOLCH J J BIOL CHEM 226 497 957 046 ANON SUMMARY PROC WRKSHOP ON BIOCH 971 047 FOX SH AM PHARM ASSOC J 39 621 950 048 FREDRICKSON DS N ENGL J MED 276 34 967 049 FRIEND CJ BR J NUTR 15 231 961 050 FUTTERMAN S FED PROC 25 521 966 051 FUTTERMAN S J BIOL CHEM 239 4077 964 052 FUTTERMAN S J BIOL CHEM 239 81 964 053 GAGNON M PROC SOC EXP BIOL MED 127 99 968 054 GAL I PROC NUTR SOC 28 9A 968 055 GANGULY J ARCH BIOCHEM BIOPHYS 52 186 954 056 GANGULY J VITAM HORM 18 387 960 057 GANGULY J J SCI INDUSTR RES 26 110 967 058 GANGULY J BIOCHEM J 54 12 953 059 GANGULY J ARCH BIOCHEM BIOPHYS 38 275 952 060 GANGULY J BIOCHEM J 71 756 959 061 GARBERS CF NATURE 182 1018 958 062 GARBERS CF BIOCHEM J 75 124 960 063 GARRY PJ CLIN CHEM 16 766 970 064 GLOVER J BIOCHEM J 41 97 947 065 GLOVER J EXP EYE RES 3 374 964 066 GOODMAN DE WS AM J CLIN NUTR 22 911 969 067 GOODMAN DE WS IN: DELUCA HF 212 970 068 GOODMAN DE WS J CLIN INVEST 45 1615 966 069 GOODMAN DE WS J LIPID RES 6 390 965 070 HACK MH PROC SOC EXP BIOL MED 91 92 956 071 HANSEN L AM J CLIN PATHOL 50 525 968 072 HARASHIMA K VITAMINOL 7 150 961 073 HARRIS AD BR MED J 1 553 947 074 HAYES KC NUTR REV 29 3 971 075 HAYES KC LAB INVEST 22 81 970 076 HENLEY TH AM J DIS CHILD 68 257 944 077 HEPNER R NUTR REV 29 219 971 078 HIGH EG ARCH BIOCHEM BIOPHYS 49 19 954 079 HIGH EG AM J CLIN NUTR 22 1129 969 080 HIGH EG ARCH BIOCHEM BIOPHYS 62 163 956 081 HOCH H BR J EXP PATHOL 27 316 946 082 HODGES RE IN: SUMMARY PROC WORKSHOP 10 971 083 HOPPNER K CAN MED ASSOC J 101 736 969 084 HOPPNER K CAN MED ASSOC J 99 983 968 085 HUANG HS J BIOL CHEM 240 2839 965 086 HUME EM MED RES COUNCIL SPEC REP SER 949 087 ANON MANUAL FOR NUTRITION SURVEYS 124 969 088 JACOBS AL AM J CLIN NUTR 2 155 954 089 JOHANNESSEN S FEBS LETTERS 2 146 969 090 KAGAN BM J LAB CLIN MED 40 12 952 091 KAGAN BM J NUTR 57 277 955 092 KAGAN BM J CLIN INVEST 29 141 950 093 KAHAN J SCAND J CLIN LAB INVEST 18 679 966 094 KALBE VI INT Z VITAMINFORSCH 36 16 966 095 KANAI MA J CLIN INVEST 47 2025 968 096 KARPACHEVA VA BIOKHIMIYA 28 209 963 097 KARRER P VITAM HORM 18 291 960 098 KIMBLE MS J LAB CLIN MED 24 1055 939 099 KNUDSON AG JR AM J DIS CHILD 85 316 953 100 KOTHARI LK AM J CLIN NUTR 24 510 971 101 KRAUSE RF J NUTR 38 535 949 102 KRAUSE RF AM J CLIN NUTR 16 455 965 103 KRAUSE RF J NUTR 33 633 947 104 KRAUSE RF AM J CLIN NUTR 4 68 956 105 KRINSKY NI ARCH BIOCHEM BIOPHYS 73 233 958 106 KRINSKY NI J BIOL CHEM 202 227 953 107 KRISHNAMURTHY S NATURE 177 575 956 108 KRISHNAMURTHY S J BIOL CHEM 233 32 958 109 KRISHNAMURTHY SM ARCH BIOCHEM BIOPHYS 75 6 958 110 LAUGHLAND DH ARCH BIOCHEM BIOPHYS 73 95 958 111 LAWRENCE CW J LIPID RES 7 226 966 112 LEONARD PJ E AFR MED J 41 133 964 113 LEWIS JM AM J DIS CHILD 62 1129 941 114 LINDER MC J BIOL CHEM 246 5538 971 115 LUCY JA AM J CLIN NUTR 22 1033 969 116 MAHADEVAN S J BIOL CHEM 241 57 966 117 MAHADEVAN S BIOCHEM J 93 499 964 118 MAHADEVAN S BIOCHEM J 81 53 961 119 MAHADEVAN S INDIAN J MED RES 47 199 959 120 MAHADEVAN S WORLD REV NUTR DIET 5 209 965 121 MAHADEVAN S BIOCHEM J 88 531 963 122 MAHADEVAN S BIOCHEM J 88 534 963 123 MAY CD AM J DIS CHILD 59 1167 940 124 MCFARLANE AS NATURE 149 439 942 125 MCGUGAN WA ARCH BIOCHEM BIOPHYS 35 428 952 126 MCLAREN DS METHODS BIOCHEM ANAL 15 1 967 127 MCLAREN DS AM J CLIN NUTR 17 117 965 128 MEYER KA PROC SOC EXP BIOL MED 49 589 942 129 MILLS DM AM J PATHOL 54 147 969 130 MOORE T BIOCHEM J 31 155 937 131 MOORE T VITAMIN A 957 132 MORGAN FJ BIOCHIM BIOPHYS ACTA 236 798 971 133 MORTON RA ANALYST 71 348 946 134 MURRAY TK CAN J BIOCHEM 39 1103 961 135 MUTO Y J BIOL CHEM 247 2542 972 136 NAKANE PK J HISTOCHEM CYTOCHEM 13 640 965 137 NEELD JB J NUTR 79 454 963 138 NEWMAN RG TRANS NY ACAD SCI 33 316 971 139 NIR I BR J NUTR 21 557 967 140 NYQUIST SE SCIENCE 173 939 971 141 OLIVER TK JR AM J DIS CHILD 95 57 958 142 OLSON JA PHARMACOL REV 19 559 967 143 OLSON JA VITAM HORM 26 1 968 144 ONCLEY JL J AM CHEM SOC 72 458 950 145 ONEAL RM PEDIATR RES 4 103 970 146 OWEN EC WORLD REV NUTR DIET 5 132 965 147 OWEN GM AM J CLIN NUTR 22 1444 969 148 OWEN GM J PEDIATR 78 1042 971 149 PALMER LS J BIOL CHEM 17 223 914 150 PATWARDHAN VN AM J CLIN NUTR 22 1106 969 151 PEASE CN JAMA 182 980 962 152 PETERSEN RA AM J DIS CHILD 116 662 968 153 PETERSON PA J BIOL CHEM 246 34 971 154 PETERSON PA J BIOL CHEM 246 44 971 155 PETERSON PA J BIOL CHEM 246 25 971 156 PETT LB J BIOL CHEM 132 585 940 157 POPPER H PROC SOC EXP BIOL MED 43 133 940 158 POPPER H ARCH PATHOL 31 766 941 159 POPPER H J NUTR 23 335 942 160 POPPER H JAMA 123 1108 943 161 POPPER H J CLIN INVEST 22 775 943 162 POWERLL LT ARCH BIOCHEM BIOPHYS 44 102 953 163 RAICA N JR AM J CLIN NUTR 25 291 972 164 RALLI EP ARCH INTERN MED 68 102 941 165 RAZ A J BIOL CHEM 244 3230 969 166 RAZ A J BIOL CHEM 245 1903 970 167 REDGRAVE TG AUST J EXP BIOL MED SCI 49 209 971 168 ROELS OA AM J CLIN NUTR 22 1020 969 169 RUBIN E AM J DIS CHILD 119 132 970 170 SABOTKA H J AM CHEM SOC 65 1959 943 171 SABOTKA H J BIOL CHEM 152 635 944 172 SASTRY PS INDIAN J MED RES 45 263 957 173 SAUBERLICH HE PROC WORKSHOP ON BIOCHEM AND 32 971 174 SEBRELL WH JR ARCH LATINO AMER NUTR 22 1 972 175 SEBRELL WH AM J CLIN NUTR 7 538 959 176 SELVARAJ RJ CLIN CHIM ACTA 27 165 970 177 SEWELL HB VITAMINFORSCH 37 301 967 178 SHERMAN BS INT J VITAM RES 39 111 969 179 SMITH BM BR J NUTR 16 213 962 180 SMITH FR J CLIN INVEST 49 90A 970 181 SMITH FR J CLIN INVEST 50 2426 971 182 SMITH FR J CLIN INVEST 49 1754 970 183 SMITH FR J LAB CLIN MED 80 423 972 184 STIMSON WH N ENGL J MED 265 369 961 185 VON TARJAN R INT Z VITAMINFORSCH 34 326 964 186 THOMPSON JN BIOCHEM MED 5 67 971 187 THOMPSON JN PROC R SOC LOND BIOL 159 510 964 188$ UDENFRIEND S IN: BIOLOGY AND MEDICINE 231 962 189 UNDERWOOD BA BULL NY ACAD MED 47 34 971 190 UNDERWOOD BA PEDIATR RES 6 26 972 191 UNDERWOOD BA AM J CLIN NUTR 23 1314 970 192 UNDERWOOD BA AM J CLIN NUTR 23 1037 970 193 UTLEY MH J NUTR 66 205 958 194 VAHLQUIST A EUR J BIOCHEM 20 160 971 195 VEEN MJ CAN J PHYSIOL PHARMACOL 44 521 966 196 WALD G VITAM HORM 18 417 960 197 WENDT H KLIN WOCHENSCHR 16 1253 937 198 WILSON JG POULTRY SCI 45 980 966 199 WOEBER KA J CLIN INVEST 47 1710 968 200 WOLFF LK LANCET 2 617 932 201 WONG YC LAB INVEST 24 55 971 202 WOO TT CHIN J PHYSIOL 15 83 940 203 YEUNG DL AM J CLIN NUTR 24 172 971 CT 1 NATANSON AO VOPR MED KHIM 22 388 976 2 LECHAT MF ANN SOC BELG MED TROP 56 333 976 3 RAMALINGASWAMI V ANN NY ACAD SCI 300 210 977 4 CORREIADEARAUJO CR CLIN CHEM 24 386 978 5 PALIN D AM J CLIN NUTR 32 1253 979 6 HUQUE T PROC NUTR SOC 38 A 41 979 7 LOERCH JD J NUTR 109 778 979 8 UNDERWOOD BA J NUTR 109 796 979 9 HELLER J ARCH BIOCHEM BIOPHYS 198 562 979 10 ROGERS EL AM J CLIN NUTR 33 1208 980 11 CARNEY EA J NUTR 110 552 980 12 MOBARHAN S AM J CLIN NUTR 34 2264 981 13 PITT GAJ PROC NUTR SOC 40 173 981 14 ROLTSCH IA NUTR RES 2 499 982 15 MORITA A J NUTR 112 789 982 16 BAUERNFEIND JC WORLD REV NUTR DIET 41 110 983 17 HUQUE T INT J VIT NUTR RES 53 123 983 18 MAWSON AR LANCET 1 1181 984 19 MEJIA LA AM J CLIN NUTR 39 62 984 20 FLORES H AM J CLIN NUTR 40 146 984 21 FLORES H AM J CLIN NUTR 40 1281 984 22 SHAH RS AM J CLIN NUTR 40 794 984 23 HUSTEAD VA J PEDIATR 105 610 984 24 RASMUSSEN M ACTA MED SCAND 216 403 984 25 MAWSON AR MED HYPOTHESES 18 387 985 26 HENNIG A ARCH TIERERNAHR-ARCH ANI NUTR 35 19 985 27 RASMUSSEN M J PEDIATR GASTROENTEROL NUTR 5 397 986 28 PEREIRA GR CLIN PERINATOL 13 175 986 29 SCHONE F MONATSH VETERINARMED 41 401 986 30 SPEAR PA CAN J ZOOL 64 204 986 PN 74167 RN 00166 AN 74117029 AU Cichocki-T. Litwin-J-A. Szotowa-W. Hanicka-M. Gutkowski-P. Zebrak-J. Czeczotko-E. TI Histochemical observations on the pulmonary macrophages in cystic fibrosis. SO Z-Kinderheilkd. 1974 Jan 17. 116(2). P 127-36. MJ CYSTIC-FIBROSIS: pa. MACROPHAGES: cy. MN ACID-PHOSPHATASE: an. ADOLESCENCE. ASTHMA: pa. BRONCHIECTASIS: pa. BRONCHITIS: pa. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. GLUCOSAMINIDASE: an. GLUCURONIDASE: an. HUMAN. MACROPHAGES: en, an. MUCOPOLYSACCHARIDES: an. AB The pulmonary macrophages collected with bronchial mucus from patients with cystic fibrosis and other chronic lung diseases were investigated histochemically for acid phosphatase, beta-glucuronidase and beta-glucosaminidase activity, and also for mucopolysaccharide content. In macrophages from patients with cystic fibrosis decreased beta-glucuronidase activity was observed, together with large cytoplasmic vacuoles suggesting storage of partially digested material. It thus seems very likely that apart from the increased synthesis of mucopolysaccharides described in cystic fibrosis by several authors, the degradation of mucosubstances produced can be impaired. RF 001 BALLANTYNE B J PHYSIOL (LOND) 191 89P 967 002 BARKA T J HISTOCHEM CYTOCHEM 10 741 962 003 BROWN GA LANCET 2 639 971 004 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 DESJEUX JF EUROPEAN SOC PEDIAT RES MTG 970 008 DI SANTAGNESE PA N ENGL J MED 277 1287 967 009 GIBBS GE SCIENCE 167 993 970 010 GIBSON LE PEDIATRICS 48 695 971 011 GILLY R ANN PEDIATR (PARIS) 20 5 973 012 GUGLER EC J PEDIATR 73 548 968 013 HAYASHI M J HISTOCHEM CYTOCHEM 13 26 965 014 HAYASHI M J HISTOCHEM CYTOCHEM 12 293 964 015 LINKER A J BIOL CHEM 213 237 955 016 MANGOS JA PEDIATR RES 1 436 967 017 MOWRY RW LAB INVEST 7 566 958 018 RAO GJS SCIENCE 177 610 972 019 SLY WS J PEDIATR 82 249 973 020 SPOCK A POSTGRAD MED J 50 92 971 021 WARTON KL BR MED J 3 570 971 CT 1 SOMAYAJULU RSN FOLIA HISTOCHEM CYTOCHEM 16 3 978 2 CASSINO RJJ PEDIATR RES 14 1212 980 PN 74168 RN 00167 AN 75010829 AU Gotz-M. Ludwig-H. Polymenidis-Z. TI HL-A antigens in cystic fibrosis. SO Z-Kinderheilkd. 1974. 117(3). P 183-6. MJ CYSTIC-FIBROSIS: im. HISTOCOMPATIBILITY-ANTIGENS: an. MN ABO-BLOOD-GROUP-SYSTEM: an. ADOLESCENCE. BLOOD-GROUPS. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. AB HL-A frequencies of 28 patients with cystic fibrosis and of 240 unrelated controls were compared. No statistically significant difference of HL-A frequencies could be observed for both groups. A slightly increased HL-A 2 frequency is considered a chance phenomenon. In accordance with a previously demonstrated lacking association of the ABH blood group substances no correlation with certain HL-A antigens was demonstrable. RF 001 ASQUITH P LANCET 1 113 974 002 BREWERTON DA LANCET 1 904 973 003? COLOMBANI J REV EUR ETUD CLIN BIOL 17 551 972 004 DI SANTAGNESE PA IN: MANGOS JA XI 973 005 FORBES JF J CLIN INVEST 51 1156 972 006 JONGSMA A HUMANGENETIK 20 195 973 007 KLAGSBRUN M IN: MANGOS JA 53 973 008 LIES RB ARTHRITIS RHEUM 15 524 972 009 LOUISOT P CLIN CHIM ACTA 48 373 973 010 LUDWIG H J IMMUNOGENET 1 109 974 011 MACKAY IR LANCET 2 793 972 012 MARTZ E TISSUE ANTIGENS 3 30 973 013 RAO GJS SCIENCE 177 610 972 014 RENNERT OM PEDIATRICS 50 485 972 015 STEINBERG AG AM J HUM GENET 8 177 956 016 THORSBY E TISSUE ANTIGENS 1 147 971 017 VIRTANEN S J PEDIATR 68 139 966 CT 1 WOOD RE AM REV RESPIR DIS 113 833 976 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 3 977 3 SAFWENBERG J TISSUE ANTIGENS 10 287 977 4 KAISER GI ACTA PAEDIATR ACAD SCI HUNG 18 27 977 5 BOWMAN BH HUM HERED 31 248 981 6 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 7 KNOPFLE G KLIN PAEDIATR 197 13 985 8 MOSS RB MONOGR ALLERGY 19 202 986