PN 79001 RN 00981 AN 79120857 AU Jensen-O. TI 'Rusters'. The corrosive action of palmar sweat: I. Sodium chloride in sweat. SO Acta-Derm-Venereol (Stockh). 1979. 59(2). P 135-8. MJ CORROSION. HYPERHIDROSIS. METALLURGY. OCCUPATIONAL-MEDICINE. SODIUM-CHLORIDE. SWEAT: an. MN ADMINISTRATION-TOPICAL. ADOLESCENCE. ADULT. ALUMINUM: tu. CHLORIDES: tu. CYSTIC-FIBROSIS: co. DERMATOLOGIC-AGENTS: tu. FEMALE. HAND. HUMAN. HYPERHIDROSIS: dt. IONTOPHORESIS. MALE. MIDDLE-AGE. PILOCARPINE. SODIUM-CHLORIDE: an. AB Sweat from 8 'rusters' and 8 control persons was examined for its sodium concentration. Sweating was induced by iontophoresis of pilocarpine on the distal forearm. In no case did the concentrations exceed the normal upper limit for this procedure, and no difference was found between the two groups. In this way the study failed to confirm earlier reports of elevated sodium chloride concentrations in sweat from 'rusters'. Proposals of a relationship between 'rusters' and patients with pancreatic cystic fibrosis were not substantiated. Hyperhidrosis of the palms and volar surfaces of the fingers was present in all 'rusters'. This seemed to be the main cause of the corrosive tendency, and patients referred for palmar hyperhidrosis were found to produce corrosion similar to the 'rusters'. Topical application of aluminium chloride hexahydrate in a 25% solution in absolute ethyl alcohol proved effective against both hyperhidrosis and the corrosive tendency. RF 001 ALLEN JA BR J DERMATOL 90 277 974 002 BANG PEDERSEN N BR J DERMATOL 96 332 977 003 BUCKLEY WR J OCCUP MED 2 23 960 004 BURTON JL BR J DERMATOL 95 417 976 005 DARLING RC ARCH PHYS MED 29 150 948 006 EISLER SJ CORROSION 10 237 954 007 EVANS UR INTRODUCTION TO METAL CORR 28 963 008 FISHER AA CONTACT DERMATITIS 94 973 009 FLENSBORG EW IONTROPHORESIS APPARATUS 971 010 GIBSON LE PEDIATRICS 23 545 959 011 HUFF WJ CHEM MET ENG 25 865 921 012 JOHNSON BB J INVEST DERMATOL 53 116 969 013 LOBITZ WC ARCH DERMATOL SYPHIL 56 462 947 014 ROBINSON S PHYSIOL REV 34 202 954 015 SATO K J INVEST DERMATOL 55 433 970 016 SHELLY WB ACTA DERM VENEREOL (STOCKH) 55 241 975 CT 1 JENSEN O ACTA DERM VENEREOL (STOCKH) 59 139 979 2 TAYLOR JS J AM ACAD DERMATOL 11 1007 984 PN 79002 RN 00982 AN 79205962 AU Bak-Pedersen-K. Larsen-P-K. TI Inflammatory middle ear diseases in patients with cystic fibrosis. SO Acta-Otolaryngol [Suppl] (Stockh). 1979. (360). P 138-40. MJ CYSTIC-FIBROSIS: co. OTITIS-MEDIA: et. MN ACOUSTIC-IMPEDANCE-TESTS. ADOLESCENCE. ADULT. AUDIOMETRY. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. FEMALE. HEARING-LOSS-PARTIAL: et. HUMAN. INFANT. MALE. NASAL-POLYPS: et. OTITIS-MEDIA: co, pp. PROSPECTIVE-STUDIES. AB 111 patients with cystic fibrosis (CF), aged 7 months to 29 years, were examined to detect inflammatory middle ear diseases. 35% had a history of one or more attacks of acute otitis media, an incidence at the same level as in non-CF patients. None had chronic suppurative otitis. Only 4 out of the 88 patients able to cooperate in audiometry had a hearing impairment. In 2, the hearing loss was perceptive, in 3 very mild, and in the fourth case, moderately severe. The middle ear pressure was measured in 108 patients, in 86 of whom it proved normal. In 15 patients the middle ear pressure was lower than--100 mmH2O, indicating tubal occlusion, and in 7 patients the impedance minimum was lacking, indicating an accumulation of mucus. These 7 patients (6%) had secretory otitis media. Nasal polyps were or had been present in 32%. There was no correlation between nasal polyps and tubal occlusion or secretory otitis. The incidence of inflammatory middle ear diseases in CF patients was in the same range as in non-CF patients, a finding at variance with some previous investigations showing middle ear pathology in 25--48%. RF 001 BAK-PEDERSEN K THESIS 977 002 FORCUCCI RA ARCH OTOLARYNGOL 96 361 972 003 FRITZE W Z KINDERHEILK 114 111 973 004 JERGER J ARCH OTOLARYNGOL 93 111 971 005 JOHANSEN PG LANCET 1 455 968 006 KULCZYCKI LL CLIN PEDIATR 9 390 970 007 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 008 PENNINGTON CL ARCH OTOLARYNGOL 63 576 956 009 SIEGEL J ARCH OTOLARYNGOL 92 523 970 010 TAYLOR BW ARCH DIS CHILD 49 133 974 011 TYGSTRUP I UGESKR LAEG TRANS DAN MED 1 6 972 CT 1 PEDERSEN M CLIN OTOLARYNGOL 7 373 982 2 VANCAUWENBERGE PB ACTA OTOLARYNGOL STOCKH SUPPL 414 147 984 3 DAVID TJ J ROY SOC MED 79 23 986 PN 79003 RN 00983 AN 79228230 AU Hoiby-N. Hertz-J-B. TI Precipitating antibodies against Escherichia coli, Bacteroides fragilis ss. thetaiotaomicron and Pseudomonas aeruginosa in serum from normal persons and cystic fibrosis patients, determined by means of crossed immunoelectrophoresis. SO Acta-Paediatr-Scand. 1979 Jul. 68(4). P 495-500. MJ ANTIBODIES-BACTERIAL. BACTEROIDES-FRAGILIS: im. CYSTIC-FIBROSIS: im. ESCHERICHIA-COLI: im. PSEUDOMONAS-AERUGINOSA: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mi. FEMALE. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. INFANT. MALE. AB Serum from normal persons and from 133 cystic fibrosis patients was examined for precipitins against faecal bacteria (E. coli and B. fragilis) and against P. aeruginosa by means of crossed immunoelectrophoresis. 27% of the normal sera contained 1-2 precipitins against E. coli, 16% contained one precipitin against B. fragilis and 6% contained one precipitin against P. aeruginosa. In sera from cystic fibrosis patients, there was a significantly increased prevalence of precipitins against E. coli (76%), B. fragilis (38%) and P. aeruginosa (63%), and the mean number of precipitins against each of these bacteria was 3 (range: 1-12), 1.4 (range: 1-4), and 16 (range: 1-60), respectively. Increased numbers of precipitins against P. aeruginosa were correlated with chronic lung infection caused by this species and with poor prognosis, whereas such associations were not revealed as regards precipitins against the two other bacterial species. Increased numbers of precipitins against E. coli and B. fragilis were significantly associated with increased numbers of precipitins against P. aeruginosa. It is suggested that this association is due to immune reactions in the intestinal mucosa, resulting in increased absorption of antigens from the gut. RF 001 BJORNEBOE M LANCET 1 58 972 002 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 003 FINEGOLD SM ANAEROBIC BACTERIA HUMAN DIS 977 004 GIBBONS A BR MED J 1 120 976 005 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 006 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 008 HOIBY N SCAND J RESPIR DIS 56 38 975 009 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 010 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 011 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 012 MCFARLANE H BR MED J 1 423 975 013 SASAKI M JAPAN J EXP MED 45 335 975 014 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 015 SCHWAB JH BACT REV 39 121 975 016 SKIDMORE BJ J IMMUNOL 114 770 975 017 THERKELSEN AJ MEDICINSK STATISTIK AKADEMISK 968 018 TOLO K IMMUNOLOGY 33 733 977 019 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 020 WALLWORK JC CLIN ALLERGY 6 349 976 021 WEEKE B SCAND J IMMUNOL SUPPL 1 2 15 973 022 WEEKE B SCAND J IMMUNOL SUPPL 1 2 47 973 CT 1 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 3 KOCH C ALLERGY 37 191 982 4 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 6 BERG RD EOS RIV IMMUNOL IMMUNOFARMACO 5 161 985 7 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 PN 79004 RN 00984 AN 79121155 AU Berg-N-O. Dahlqvist-A. Lindberg-T. TI Exocrine pancreatic insufficiency, small intestinal dysfunction and protein intolerance. A chance occurrence or a connection?. SO Acta-Paediatr-Scand. 1979 Mar. 68(2). P 275-6. MJ CELIAC-DISEASE: co. CYSTIC-FIBROSIS: co. MN CASE-REPORT. CHILD. DIPEPTIDASES: me. DISACCHARIDASES: me. FEMALE. GLUTEN: du. HUMAN. INTESTINAL-MUCOSA: en, pa. INTESTINE-SMALL: en, pa. MALE. AB The coexistence of cystic fibrosis (CF) and coeliac disease (CD) is reported in eight children, the odds against this occurring in the same child being 1:2 million to 1:5.9 million. In Sweden, it would be around 1:3 million (incidence of CF 1:3000 and of CD 1:1000). The coexistence of these two diseases is thought to be chance, although CF might predispose to the development of CD. In a 10-year period (with 33405 live births), we observed three children with exocrine pancreatic insufficiency and small intestine dysfunction. RF 001 ANTONOWICZ I PEDIATRICS 42 492 968 003 DAHLQVIST A ACTA PAEDIATR SCAND 59 621 970 004 GOODCHILD MC ARCH DIS CHILD 48 684 973 005 KATZ AJ PEDIATRICS 57 715 976 006 KOLLBERG H ACTA UNIV UPSALIENSIS 192 974 007 LEBENTHAL E GASTROENTEROLOGY 70 508 976 008 MORIN CL J PEDIATR 88 213 976 009 SHMERLING DH HELV PAEDIATR ACTA 24 547 969 010 SHWACHMAN H J PEDIATR 65 645 964 CT 1 SJOSTROM H J BIOL CHEM 255 1332 980 PN 79005 RN 00985 AN 79228250 AU Taussig-L-M. TI Increased intracranial pressure in infants with cystic fibrosis [letter]. SO Acta-Paediatr-Scand. 1979 Jul. 68(4). P 615-6. MJ CYSTIC-FIBROSIS: pp. INTRACRANIAL-PRESSURE. MN HUMAN. INFANT. EX Although the mechanism proposed by Dr Katznelson for the increased intracranial pressure observed in three infants with cystic fibrosis cannot be discounted, another explanation is possible. It has been reported previously that a deficiency of vitamin A may predispose to development of increased intracranial pressure and bulging fontanelles in cystic fibrosis. Finally, it is somewhat difficult to understand how such marked airway obstruction which produced increased intracranial pressure could occur with preservation of arterial blood gases. - Vitamin A and carotene blood levels were not determined in our cases and therefore obviously this possibility cannot be excluded, at least in two of the cases. Dr Taussig's suggestion that the improvement in the intracranial pressure in our cases was really the result of the "unknowing" associated administration of vitamin A and not to the improvements in the pulmonary status, as I proposed, fails to note the clinical observation that there had been a "rise and fall" in the bulge of the fontanel concomitantly with the increase and decrease of respiratory obstruction signs. As to the question of the severity of airway obstruction, the clinical reversible picture of severe tachypnea with poor air entry, clear lung fields on X-ray with overdistended lungs and small heart appear most suggestive of airway obstruction in C.F. RF 001 KATZNELSON D ACTA PAEDIATR SCAND 67 607 978 002 KEATING JP PEDIATRICS 46 41 970 PN 79006 RN 00986 AN 80150170 AU Schiotz-P-O. Hoiby-N. TI Precipitating antibodies against Haemophilus influenzae and Staphylococcus aureus in sputum and serum from patients with cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [B]. 1979 Dec. 87(6). P 345-51. MJ ANTIBODIES-BACTERIAL: an. CYSTIC-FIBROSIS: mi. HAEMOPHILUS-INFLUENZAE: im. PRECIPITINS: an. SPUTUM: mi. STAPHYLOCOCCUS-AUREUS: im. MN ADOLESCENCE. ADULT. CHILD. CROSS-REACTIONS. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. AB Serum and sputum sol phase from 23 patients with cystic fibrosis (CF) were examined for occurrence and titres of precipitins against Haemophilus influenzae and Staphylococcus aureus by means of crossed immunoelectrophoresis with intermediate gel. The patients had from four to nine H. influenzae precipitins in serum and in most cases fewer precipitins in sputum, but, on an average, there was no difference between the titres of the antibodies in serum and sputum. Most of the antibodies were cross-reactive with other species, notably those of the Haemophilus genus. S. aureus precipitins were generally found in higher numbers in serum than in sputum, but, on an average, the titre of the precipitins in sputum was higher than in serum. Three of the precipitins were detectable only in sputum and not in serum, and one of these is a S. aureus-specific precipitin. Most of the antibodies were cross-reactive with other species, and these antibodies were often present in sputum in much higher titres than in the corresponding sera. Antibodies against teichoic acid of the S. aureus cell wall could not be demonstrated in sputum, while they were present in 22 sera. The possible role of the local pulmonary humoral immune response in protective immunity and in the pathology of the lung disease in CF is discussed. RF 001 BROGAN TD THORAX 30 72 975 002 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 003 GIBSON LE PEDIATRICS 23 545 959 004 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 005 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 006 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 007 HOIBY N SCAND J RESPIR DIS 56 38 975 008 MAY JR ARCH DIS CHILD 47 908 972 009 MEARNS MB ARCH DIS CHILD 47 902 972 010 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 011 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 012 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 337 979 013 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (B) 87 329 979 CT 1 ESPERSEN F ACTA PATH MICROBIOL SCAND (B) 88 237 980 2 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 5 PRESSLER T ACTA PAEDIATR SCAND 73 541 984 PN 79007 RN 00987 AN 80016770 AU Schiotz-P-O. Hoiby-N. Permin-H. Wiik-A. TI IgA and IgG antibodies against surface antigens of Pseudomonas aeruginosa in sputum and serum from patients with cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [C]. 1979 Jun. 87C(3). P 229-33. MJ CYSTIC-FIBROSIS: im. IGA. IGG. PSEUDOMONAS-AERUGINOSA: im. SPUTUM: im. MN ADOLESCENCE. ADULT. ANTIBODIES-BACTERIAL. ANTIGENS-BACTERIAL. ANTIGENS-SURFACE. CHILD. CHRONIC-DISEASE. FEMALE. FLUORESCENT-ANTIBODY-TECHNIC. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. AB Eleven cystic fibrosis (CF) patients chronically infected in the lungs with mucoid Pseudomonas aeruginosa and presenting multiple precipitins in serum against this bacterium (CF + P) and 10 CF patients without P. aeruginosa infection (CF-P) had their serum and sputum sol phase specimens examined for antibodies of the IgA and IgG classes against surface antigens of P. aeruginosa by means of an indirect immunofluorescence technique. Both the IgA and IgG antibody titres demonstrated in serum and sputum of the CF + P patients were significantly higher than in those of the CF-P patients (p less than 0.01). The titre of IgA antibodies in the sputum was higher than in serum in 3 cases indicating local pulmonary production of specific IgA antibodies. The role of the demonstrated antibodies in the local pulmonary immune defense mechanisms and the possible patogenesis of the pulmonary tissue damage in CF patients is discussed. RF 001 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 002 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 003 BJORNSON AB J INFECT DIS SUPPL 130 119 974 004 BJORNSON AB J INFECT DIS SUPPL 130 127 974 005 BOXERBAUM B AM REV RESPIR DIS 108 777 973 006 BROGAN TD THORAX 30 72 975 007 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 008 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 009 GIBSON LE PEDIATRICS 23 545 959 010 GOVAN JRW J MED MICROBIOL 8 513 975 011 HANN S INFECT IMMUN 14 114 976 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 015 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 016 HOIBY N SCAND J RESPIR DIS 58 65 977 017 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 018 KOCH C ACTA PATH MICROBIOL SCAND (C) 83 144 975 019 MCFARLANE H BR MED J 1 423 975 020 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 021 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 022 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 023 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 1 979 024 WIIK A IMMUNOLOGY 23 53 972 025 YOUNG LS J INFECT DIS SUPPL 130 111 974 CT 1 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 5 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 6 SCHILLER NL PEDIATR RES 17 747 983 7 DORING G J INFECT DIS 147 744 983 8 KOHLER B Z KLIN MED 40 1773 985 9 BALTIMORE RS PEDIATR RES 20 1085 986 PN 79008 RN 00988 AN 79162009 AU Schiotz-P-O. Sorensen-H. Hoiby-M. TI Activated complement in the sputum from patients with cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [C]. 1979 Feb. 87C(1). P 1-5. MJ COMPLEMENT-ACTIVATION. COMPLEMENT. CYSTIC-FIBROSIS: im. SPUTUM: im. MN ADOLESCENCE. ADULT. ALBUMINS: an. CHILD. CHRONIC-DISEASE. COMPLEMENT-3: an. COMPLEMENT-4: an. COMPLEMENT-5: an. COMPLEMENT: an. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. PNEUMONIA: et, im. PSEUDOMONAS-INFECTIONS: et, im. SERUM-ALBUMIN: an. AB 14 cystic fibrosis (CF) patients chronically infected with mucoid P. aeruginosa and presenting multiple precipitins in serum against this bacterium (CF + P) and 13 CF patients without P. aeruginosa infection (CF-P) had their plasma and sputum sol phase examined for albumin, Clq. C3/C3c, C4 and C5 by means of electroimmunoassays. Their sputum sol phase was examined also for factor B by rocketimmunoelectrophoresis. C3c was demonstrated in the sputum sol phase but significantly more frequent (p less than 0.01) among the CF + P patients than among the CF-P patients. Factor B was also demonstrated in the sputum sol phase, but no significant difference in frequency could be demonstrated between the CF + P and the CF-P patients. None of the results indicated that a local pulmonary production of complement factors took place. Complement activation was significantly (p less than 0.01) associated with inflammation expressed as increased (formula: see text). The results show the importance of complement mediated inflammation in the pathogenesis of pulmonary tissue damage in patients with CF and support the concept of chronic P. aeruginosa lung infection as an immune complex disease in CF patients. RF 001 BROGAN TD THORAX 30 72 975 002 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 003 GEWURTZ H J EXP MED 128 1049 968 004 GIBSON LE PEDIATRICS 23 545 959 005 GREENBLATT J INFECT IMMUN 19 296 978 006 HOFF GE ACTA PATH MICROBIOL SCAND (B) 83 219 975 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 009 HOIBY N SCAND J RESPIR DIS 58 65 977 010 LAURELL CB SCAND J CLIN LAB INVEST SUPPL 124 21 972 011 NELSON RA JR IN: ZWEIFACH BW 3 37 974 012 REYNOLDS HY J LAB CLIN MED 84 559 974 013 ROBERTSON J J IMMUNOL 117 900 976 014 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 015 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 016 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 017 STALENHEIM G IMMUNOCHEMISTRY 10 501 973 018 TEISBERG P CLIN CHIM ACTA 62 35 975 019 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 CT 1 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 87 229 979 2 ETIEVANT M ANN IMMUNOL (PARIS) D131 13 980 3 SKOV PS ALLERGY 35 23 980 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 88 275 980 5 SMITH TF INFECT IMMUN 33 43 981 6 MOSS RB CLIN EXP IMMUNOL 47 301 982 7 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 8 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 9 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 10 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 11 PIER GB J INFECT DIS 150 223 984 12 SORDELLI DO EUR J RESPIR DIS 67 118 985 13 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 14 KHARAZMI A EUR J CLIN INVEST 16 143 986 15 HOIBY N ANNU REV MICROBIOL 40 29 986 16 EDWARDS KM ARCH VIROL 88 49 986 17 MOSS RB AM REV RESPIR DIS 133 648 986 PN 79009 RN 00989 AN 80084554 AU Schiotz-P-O. Egeskjold-E-M. Hoiby-N. Permin-H. TI Autoantibodies in serum and sputum from patients with cystic fibrosis. SO Acta-Pathol-Microbiol-Scand [C]. 1979 Oct. 87(5). P 319-24. MJ AUTOANTIBODIES: an. CYSTIC-FIBROSIS: im. SPUTUM: im. MN ADOLESCENCE. ADULT. ANTINUCLEAR-FACTORS: an. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. DNA: im. FEMALE. FLUORESCENT-ANTIBODY-TECHNIC. HUMAN. IGA: an. IGG: an. IGM: an. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. LATEX-FIXATION-TESTS. MALE. PSEUDOMONAS-AERUGINOSA. PSEUDOMONAS-INFECTIONS: co, im. RHEUMATOID-FACTOR: an. AB Sera from 89 patients with cystic fibrosis (CF) and 88 control persons were examined for the occurrence of rheumatoid factors (RF) of the IgG, IgA and IgM classes by an indirect immunofluorescence method and by the latex fixation slide test. The prevalence of RF-IgG was significantly higher (88%) (p less than 0.0005) among the CF patients than among the control persons (7%), while no difference was found between the two groups with regard to RF of the IgA or IgM classes. Fifty-five of the CF patients had chronic Pseudomonas aeruginosa infection in their lungs and two or more precipitins against these bacteria in their sera determined by crossed immunoelectrophoresis. These CF patients did not differ from the 34 CF patients without chronic P. aeruginosa infection, neither with regard to prevalence nor titer of RFs, but there was a positive correlation between the number of P. aeruginosa precipitins in the 55 chronically infected CF patients and their titers of IgG-RF. Nineteen CF patients were examined also for RFs, antinuclear antibodies (ANA) and anti-DNA antibodies in their sputum sol phase and corresponding sera. RFs were demonstrated in the sputum sol phase from 6 of the patients by the latex fixation test, whereas their sera were negative in this test, possibly indicating a local production of RF. Positive reactions for ANA and anti-DNA antibodies were found in 7 and 10 of the sputa respectively, and in higher titers than in the corresponding sera, also suggesting a local production. Titers of autoantibodies in sputum were low and no difference was found between patients with chronic P. aeruginosa infection and patients without P. aeruginosa infection. The possible role of autoantibodies in the patogenesis of pulmonary tissue damage in CF patients is discussed. RF 001 BARTFELD H ANN NY ACAD SCI 168 30 969 002 BONOMO L AM J CLIN PATHOL 45 313 966 003 BROGAN TD THORAX 30 72 975 004 CARSON DA CLIN EXP IMMUNOL 31 100 978 005 DEHORATIUS RJ ARCH INTERN MED 129 441 972 006 DEHORATIUS RJ ARTHRITIS RHEUM 15 293 972 007 DIEM K DOCUMENTA GEIGY SCIENTIFIC TA 962 008 ESTES D ARTHRITIS RHEUM 16 59 973 009 GIBSON LE PEDIATRICS 23 545 959 010 HODSON ME BR J DIS CHEST 70 83 976 011 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 012 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 013 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 014 HOIBY N SCAND J RESPIR DIS 56 38 975 015 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 107 977 016 HOIBY N SCAND J RESPIR DIS 58 65 977 017 KALLERUP HE SCAND J RHEUMATOL 8 1 979 018 KOFFLER D J EXP MED 126 607 967 019 KOFFLER D J EXP MED 134 169 971 020 LIEBERMAN J LAB INVEST 14 249 965 021 LIND I SCAND J IMMUNOL 3 689 974 022 MCCORMICK JN IN: DUMONDE D 68 976 023 MCFARLANE H BR MED J 1 423 975 024 MUNTHE E CLIN EXP IMMUNOL 8 249 971 025 PERMIN H ACTA MED SCAND 203 61 978 026 RHEINS MS J LAB CLIN MED 50 113 957 027 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 028 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 029 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 030 TAN EM J CLIN INVEST 45 1732 966 032 THUESEN-PEDERSEN J DAN MED BULL 21 12 974 033 TURNER-WARWICK M BR MED J 3 492 970 034 WALLER M J INFECT DIS 125 45 972 035 WIIK A IMMUNOLOGY 23 53 972 036 WIIK A ANN RHEUM DIS 33 515 974 037 WINCHESTER RJ CLIN EXP IMMUNOL 6 689 970 CT 1 EGESKJOLD EM ALLERGY 36 573 981 2 HOIBY N ACTA PATH MICROBIOL SCAND (C) 89 185 981 3 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 5 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 6 EGESKJOLD EM SCAND J RHEUMATOL 14 51 985 7 DISIS ML PEDIATR RES 20 385 986 8 LIMSCHOU EME DAN MED BULL 33 249 986 9 HOIBY N ANNU REV MICROBIOL 40 29 986 PN 79010 RN 00990 AN 80172132 AU Taussig-L-M. Lemen-R-J. TI Chronic obstructive lung disease. SO Adv-Pediatr. 1979. 26. P 343-416. (REVIEW). MJ LUNG-DISEASES-OBSTRUCTIVE. MN AIRWAY-OBSTRUCTION: di. ASTHMA: di. ATELECTASIS: di. BRONCHIAL-DISEASES: di. CYSTIC-FIBROSIS: di. HUMAN. HYPOVENTILATION: et. IMMUNOLOGIC-DEFICIENCY-SYNDROMES: di. LUNG-DISEASES-OBSTRUCTIVE: di, et, th. MEDICAL-HISTORY-TAKING. PHYSICAL-EXAMINATION. PNEUMONIA-ASPIRATION: di. REVIEW. TRACHEAL-DISEASES: di. EX This chapter deals with the problems of chronic obstructive lung disease in children by first considering an approach to the evaluation, diagnosis and management of various forms of chronic obstructive lung disease with emphasis on history, physical and laboratory data. A brief overview of therapeutic principles that can be applied to most forms of chronic obstructive lung disease are included. The second part of this chapter consists of reviews of some of the more common diseases producing chronic airway obstruction in infancy and childhood. Patient evaluation topics discussed include history and physical examination and laboratory studies. Therapeutic principles in the management of chronic obstructive lung disease, pediatric precursors of chronic obstructive lung disease, and chronic obstructive lung disorders (asthma, cystic fibrosis, bronchopulmonary dysplasia, aspiration pneumonitis, atelectasis, bronchomalacia and tracheomalacia, immunodeficiency, bronchiectasis, chronic bronchitis, and chronic upper airway obstruction with hypoventilation) are examined. RF 001 TAUSSIG LM ARIZ MED 33 205 976 002 HOLSCLAW DS PEDIATR ANN 6 438 977 003 ANON US NATL HEALTH SURVEY 975 004 WARING WW RESPIR CARE 20 1138 975 005 HOGG JC IN: KENDIG EL JR 977 006 DUNNILL MS THORAX 17 329 962 007 WILLIAMS HE RESPIRATORY ILLNESS IN CHILDR 975 008 TAUSSIG LM AM REV RESPIR DIS 116 233 977 009 WARING WW IN: KENDIG EL JR 105 977 010 SMITH CB RADIOL CLIN NORTH AM 11 261 973 011 FLETCHER BD IN: KENDIG EL JR 977 012 LEMEN RG IN: KENDIG EL JR 166 977 013 POLGAR G PULMONARY FUNCTION TESTING IN 971 014 ANON GAP CONF REP PROB SWEAT TESTI 975 015 ANON J PEDIATR 88 711 976 016 GODFREY S EXERCISE TESTING IN CHILDREN 974 017 WARING WW DIAGNOSTIC PROCEDURES PRACTIC 975 018 EULER AR PEDIATRICS 60 65 977 019 CHRISTIE DL J PEDIATR 93 23 978 020 JONES FL JR IN: JOHNSTON RF 973 021 RICKARD K PEDIATR CLIN NORTH AM 24 157 977 022 SUSKIND RM MALNUTRITION AND THE IMMUNE R 7 977 023 HAMBRAEUS L PEDIATR CLIN NORTH AM 24 17 977 024 FELDMAN J AM REV RESPIR DIS 119 239 979 025 HARTSELL M AM REV RESPIR DIS 117 204 978 026 WARING WW IN: KENDIG EL JR 77 977 027 TAUSSIG LM J PEDIATR 84 619 974 028 KEENS TG AM REV RESPIR DIS 116 853 977 029 MACKOWIAK PA N ENGL J MED 298 21 978 030 AMMANN AJ N ENGL J MED 297 897 977 031 AUSTRIAN R N ENGL J MED 297 938 977 032 MONSET-COUCHARD M CURR PROB PEDIATR NO 4 5 975 033 LUCAS RV MINN MED 52 1537 969 034 HEINEMANN H AM J MED 64 367 978 035 BERGLUND E AM J CARDIOL 11 477 963 036 BLAND R J CLIN INVEST 62 601 978 037 TAUSSIG LM PEDIATR RES 11 216 977 038 KERREBIJN KF ACTA PAEDIATR SCAND SUPPL 261 3 977 039 LANG WR BR MED J 1 73 969 040 GOLD R J CAN ASSOC RADIOL 20 218 969 041 BECROFT DMO J CLIN PATHOL 24 72 971 042 COLLEY JRT BR MED J 2 213 970 043 COLLEY JRT BR MED BULL 27 9 971 044 HOLLAND WW BR MED J 2 205 969 045 DOUGLAS JWB BR J PREV SOC MED 20 1 966 046 ZAPLETAL A AM REV RESPIR DIS 107 400 973 047 HARLAP S LANCET 1 529 974 048 COLLEY JRT LANCET 2 1031 974 049 COLLEY JRT BR MED J 2 201 974 050 BLAND M ARCH DIS CHILD 53 100 978 051 LEBOWITZ MD CHEST 69 48 976 052 SCHILLING RSF AM J EPIDEMIOL 106 274 977 053 SEELY JE SCIENCE 172 741 971 054 HAYNES WF JR AM REV RESPIR DIS SUPPL 93 730 966 055 COLLEY JRT BR MED J 3 195 973 056 KIERNAN KE RESPIRATION 33 236 976 057 BURROWS B AM REV RESPIR DIS 115 751 977 058 LOUGHLIN GM J PEDIATR 94 365 979 059 BECKERMAN RC PEDIATR RES 12 559 978 060 KATTAN M PEDIATRICS 59 683 977 061 SIMS DG BR MED J 1 11 978 062 KONIG P ARCH DIS CHILD 47 578 972 063 ROONEY JC J PEDIATR 79 744 971 064 ZWEIMAN B J ALLERGY 37 48 966 065 HILL DJ AM REV RESPIR DIS 106 873 972 066 MCFADDEN ER JR J ALLERGY CLIN IMMUNOL 56 18 975 067 PALMER KNV BR MED J 1 485 975 068 BLAIR H ARCH DIS CHILD 52 613 977 069 BURANAKUL B AM J DIS CHILD 128 343 974 070 DEES SC ADV ASTHMA ALLERGY 1 12 974 071 LEVISON H PEDIATR CLIN NORTH AM 21 951 974 072 GOLD WM J APPL PHYSIOL 33 496 972 073 OLIVE JT AM REV RESPIR DIS 106 366 972 074 TSUCHIYA Y J ALLERGY 36 514 965 075 TURIAF J REV TUBERC 26 238 962 076 INNES IR IN: GOODMAN LS 447 975 077 SCHAER H PFLUEGERS ARCH 347 297 974 078 STALCUP SA N ENGL J MED 297 592 977 079 GALANT SP PEDIATRICS 61 46 978 080 MCFADDEN ER JR N ENGL J MED 288 221 973 081 EGGLESTON PA PEDIATRICS 54 442 974 082 WEISS EB CIBA FOUND SYMP 27 71 975 083 WEINBERGER M POSTGRAD MED J 61 85 977 084 CHAI H ADV ASTHMA ALLERGY 2 1 975 085 BIERMAN CW PEDIATRICS 54 245 974 086 WALSON PD J PEDIATR 91 321 977 087 KOCH-WEBER J N ENGL J MED 297 476 977 088 INGRAM RH JR AM REV RESPIR DIS 101 364 970 089 WOOD DW ANN ALLERGY 31 607 973 090 PIERSON WE PEDIATRICS 54 282 974 091 BERMAN SZ J ALLERGY CLIN IMMUNOL 56 206 975 092 MCINTOSH K J PEDIATR 82 578 973 093 SHAPIRO GG PEDIATRICS 53 867 974 094 MINOR TE JAMA 227 292 974 095 TAUSSIG LM IN: KELLEY VC 4 1 976 096 NADLER HL IN: STANBURY JB 1683 978 097 WOOD RE AM REV RESPIR DIS 113 833 976 098 DI SANTAGNESE PA N ENGL J MED 295 481 976 099 ZUELZER WW PEDIATRICS 4 53 949 100 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 101 BEDROSSIAN CWM HUM PATHOL 7 195 976 102 LAMARRE A PEDIATRICS 50 291 972 103 LANDAU LI AM REV RESPIR DIS 108 593 973 104 ZAPLETAL A PEDIATRICS 48 64 971 105 FOX WW PEDIATRICS 54 293 974 106 LANDAU LI AM REV RESPIR DIS 111 725 975 107 TAUSSIG LM J PEDIATR 84 724 974 108 LLOYD-STILL JD PEDIATRICS 53 678 974 109 FELLOWS KE RADIOLOGY 114 551 975 110 HOLSCLAW DS J PEDIATR 76 829 970 111 STRUNK RC ARCH DIS CHILD 52 687 977 112 MCFARLANE H BR MED J 1 423 975 113 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 85 57 977 114 SCHWARTZ RH AM J DIS CHILD 120 432 970 115 WARREN CPW CLIN ALLERGY 5 1 975 116 BARDANA EJ JR AM J DIS CHILD 129 1164 975 117 GALANT SP AM REV RESPIR DIS 114 325 976 118 LANDAU LI J PEDIATR 82 863 973 119 SHAPIRO GG PEDIATRICS 58 740 976 120 WARNER JO ARCH DIS CHILD 51 507 976 121 TALAMO RC IN: MANGOS JA 195 976 122 SCHWARTZ RH AM J DIS CHILD 111 408 966 123 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 124 PENNINGTON JE AM J MED 58 629 975 125 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 126 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 127 BOXERBAUM B AM REV RESPIR DIS 108 777 973 128 STEIN AA J PEDIATR 65 495 964 129 HOIBY N SCAND J RESPIR DIS 56 38 975 130 HODSON ME BR J DIS CHEST 70 83 976 131 ORENSTEIN DM AM J DIS CHILD 131 973 977 132 WARWICK WJ J CHRON DIS 28 609 975 133 WARING WW ADV PEDIATR 23 401 976 134 THOMPSON T J PERINATAL MED 5 149 977 135 NORTHWAY WH JR N ENGL J MED 276 357 967 136 BENOIST MR BULL EUR PHYSIOPATH RESP 12 703 976 137 JOHNSON JD J PEDIATR 84 272 974 138 RHODES PG PEDIATRICS 55 788 975 139 HARROD JR J PEDIATR 84 277 974 140 SPOELSTRA AJG BIOL NEONATE 17 321 971 141 SHEPARD FM N ENGL J MED 279 1063 968 142 BOSS JA PEDIATRICS 29 890 962 143 TAGHIZADEH A AM J PATHOL 82 241 976 144 ANDERSON WR ARCH PATHOL 91 506 971 145 LALANDE J ANN RADIOL (PARIS) 15 R74 973 146 STAHLMAN M PEDIATR CLIN NORTH AM 20 433 973 147 BRYAN MH PEDIATRICS 52 169 973 148 OUTERBRIDGE EW AM J DIS CHILD 123 99 972 149 LAMARRE A AM REV RESPIR DIS 108 56 973 150 WESTGATE HD DIS CHEST 55 465 969 151 WATTS JL PEDIATRICS 60 273 977 152 STOCKS J PEDIATRICS 57 352 976 153 BARNES ND LANCET 2 1096 969 154 COATES AL J PEDIATR 90 611 977 155 EDWARDS DK PEDIATRICS 59 839 977 156 DELEMOS R ANESTHESIOLOGY 30 609 969 157 LEE CJ J THORAC CARDIOVASC SURG 53 759 967 158$ NASH G ARCH PATHOL 21 234 971 159 BERG TJ PEDIATRICS 55 51 975 160 FLETCHER BD ANN RADIOL (PARIS) 16 R78 973 161 PHILIP AGS PEDIATRICS 55 44 975 162 REYNOLDS EOR ARCH DIS CHILD 49 50 974 163 LILAND AE J SURG RES 20 85 976 164 RASCHE RFH PEDIATRICS 50 632 972 165 SAHEBJAMI H AM REV RESPIR DIS 110 566 974 166 BADEN M PEDIATRICS 50 526 972 167 PINNEY MA PEDIATRICS 58 856 976 168 MCNAMARA JJ PEDIATRICS 43 527 969 169 DAVIS MV ANN THORAC SURG 3 99 967 170 HERBST JJ J PEDIATR 92 73 978 171 LEAPE LL PEDIATRICS 60 924 977 172 WILLIAMS HE RESPIRATORY ILLNESS IN CHILDR 975 173 JOHNSON DG PEDIATRICS 59 62 977 174 JAMES V Q J MED 25 121 956 175 WARING WW PEDIATRICS 39 166 967 176 NEMIR RL IN: KENDIG EL JR 553 977 177 STANGER P PEDIATRICS 43 760 969 178 WILLIAMS H ARCH DIS CHILD 35 182 960 179 WILLIAMS HE ARCH DIS CHILD 47 423 972 180 GUPTA TCM AM J DIS CHILD 115 88 968 182 AGOSTI E ACTA PAEDIATR SCAND 63 616 974 183 WAYNE KS AM REV RESPIR DIS 114 15 976 184 LANDING BH PERSPECT PEDIATR PATHOL 1 973 185 EIGEN H AM REV RESPIR DIS 113 823 976 186 JONES JF ARIZ MED 33 359 976 187 POLMAR SH IN: KIRKPATRICK CH 191 976 188 STIEHM ER IMMUNOL DISORD INFANT CHILD 145 973 189 AMMANN AJ CURR PROB PEDIATR 5 975 190 BACHMANN R SCAND J CLIN LAB INVEST 17 316 965 191 HUGHES WT N ENGL J MED 297 1381 977 192 FIELD CE ARCH DIS CHILD 44 551 969 193 WIGLESWORTH FW MCGILL MED J 24 189 955 194 ELIASSON R N ENGL J MED 297 1 977 195 GLAUSER EM ACTA PAEDIATR SCAND SUPPL 165 966 196 LANDAU LI THORAX 29 304 974 197 MOORE JR SURG GYNECOL OBSTET 89 146 949 198 STRANG LB ARCH DIS CHILD 35 224 960 199 AVERY ME BULL JOHNS HOPKINS HOSP 109 20 961 200 CHERNIACK RM RESPIRATION IN HEALTH AND DIS 331 972 201 MCNAMARA MJ AM REV RESPIR DIS 100 19 969 202 GUMP DW AM REV RESPIR DIS 113 465 976 203 MCFADDEN ER JR N ENGL J MED 292 555 975 204 HIRSCH SR CHEST 63 9 973 205 BURROWS B ANN INTERN MED 77 993 972 206 NOONAN JA CIRCULATION SUPPL 2 32 164 965 207 MENASHE VD J PEDIATR 67 198 965 208 LUKE MJ PEDIATRICS 37 762 966 209 AINGER LE BR HEART J 30 356 968 210 DJALILIAN M MAYO CLIN PROC 50 11 975 211 GUILLEMINAULT C PEDIATRICS 58 23 976 212 PERLMAN M ARCH DIS CHILD 50 727 975 213 COGSWELL JJ ARCH DIS CHILD 49 520 974 CT 1 POLGAR G WIEN KLIN WOCHENSCHR 95 14 983 2 COLOMBO JL PEDIATR PULMONOL 3 86 987 PN 79011 RN 00991 AN 80172130 AU Antonowicz-I. Shwachman-H. TI Meconium in health and in disease. SO Adv-Pediatr. 1979. 26. P 275-310. (REVIEW). MJ INFANT-NEWBORN-DISEASES: pa. INTESTINAL-OBSTRUCTION: pa. MECONIUM: an. MN CYSTIC-FIBROSIS: di, en, pa. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: di. INTESTINAL-OBSTRUCTION: di. MASS-SCREENING. MECONIUM: en. REVIEW. EX Meconium is the first gastrointestinal excretion normally expelled by the neonate. This review discusses the composition of meconium: proteins, lipids, steroid and sterols, bile pigments and bile acids, glycoproteins, mineral composition, and enzymes. The major complications relating to meconium are serious: they include aspiration pneumonia, which may prove fatal and is not related to cystic fibrosis, the "meconium plug syndrome" and meconium ileus. Screening for cystic fibrosis is also examined. RF 001 EMERY JL ARCH DIS CHILD 32 17 957 002 WINDLE WF PHYSIOLOGY OF THE FETUS 940 003 POLLIFRONI GC LATTANTE 23 331 952 004 JIRASEK JE ACTA HISTOCHEM 22 33 965 005 SCHAFFER AJ DISEASES OF THE NEWBORN 267 971 006 SHERRY SN J PEDIATR 46 158 955 007 ZACHARY RB PROG PEDIATR SURG 2 57 971 008 GRULEE CG NEWBORN PHYSIOLOGY AND CA 926 009 DAVY J MEDICO CHIRURGICAL TRANS 27 189 844 010 RAPOPORT S SCIENCE 112 150 950 011$ FELDMAN WM PRINCIPLES OF ANE NATAL 191 920 013 YOSIDA KI Z GESAMTE EXP MED 63 331 928 014$ OKAJIMA S JAPAN J MED SCI VII SOC MED H 3 54 940 015 BUCHANAN DJ PROC SOC EXP BIOL MED 77 114 951 016 BUCHANAN DJ J BIOL CHEM 192 251 951 017 PUTKONEN T ACTA MED FENN DUODENUM (A) 930 018 ODIN L ACTA CHEM SCAND 9 862 955 019 SCHACHTER H CAN J BIOCHEM 43 381 965 020 KIMURA A SEIKAGAKU 36 454 964 021 CURTAIN CC AUST J EXP BIOL MED SCI 31 349 953 022 BUCHANAN DJ PEDIATRICS 9 304 952 023 GREEN MN PEDIATRICS 21 635 958 024 SCHUTT WH ARCH DIS CHILD 43 178 968 025 KNAUFF RE PROC SOC EXP BIOL MED 127 801 968 026 BERNHARD K HELV CHIM ACTA 39 1443 956 027 KINSELLA RA JR J CLIN ENDOCRINOL METAB 32 801 971 028$ ENEROTH P FEBS 3-4 129 969 029 GUSTAFSSON JA PROC R SOC LOND BIOL 180 179 972 030 BACK P HOPPE SEYLERS Z PHYSIOL CHEM 354 83 973 031 LAVY U J LIPID RES 18 232 977 032 BROWN AK IN: SMITH CA 323 976 033$ WATKINS JB IN: PAUMGARTNER G 3582 976 034 DELEZE G EXP J CLIN INVEST 8 41 978 035 SHARP HL PEDIATR RES 5 274 971 036 FRASER D CLIN CHIM ACTA 59 301 975 037 SCHRAGER J ARCH ORAL BIOL 16 287 971 038 BOUCHIER IAD CLIN CHIM ACTA 35 219 971 039 BROGAN TD BIOCHEM J 71 125 959 040 BROGAN TD BR J EXP PATHOL 41 288 960 041 OATES MDG BIOCHEM J 97 697 965 042 HOUGH L CARBOHYD RES 23 1 972 043 DISCHE Z ARCH BIOCHEM BIOPHYS 135 1 969 044 LIEBERMAN J GASTROENTEROLOGY 50 183 966 046 EMERY JL ARCH DIS CHILD 27 257 952 047 SHIVELY JA AM J CLIN PATHOL 22 1220 952 048 DE FILIPPI F PEDIATRICS 15 114 954 049 MACCHIA A RIV PEDIAT SICILIANA 12 285 967 050 EGGERMONT E BIOL NEONATE 10 266 966 051 ANTONOWICZ I GASTROENTEROLOGY 72 1299 977 052 EGGERMONT E ACTA GASTROENTEROL BELG 34 655 971 053 FOMINA LS VOPR MED KHIM 2 956 054 ZWEIFEL ARCH J GYNAEK 7 474 875 055 MULLER F Z BIOL 20 327 884 056 WIDDOWSON EM LANCET 2 373 962 057 KOPITO L J PEDIATR 68 313 966 058 DESROCHES A J GYN OBSTET BIOL REPR 3 321 974 059 SCHWARZ E ANN PAEDIATR 181 306 953 060 LINDBERG T CLIN SCI 30 505 966 061 GRUNDIG CA ACTA BIOL MED GER 19 193 967 062 POTTEVIN MH C R SOC BIOL (PARIS) 11 589 900 063 GALERNE D J GYN OBSTET BIOL REPR 2 969 973 064 GEYER VH Z KLIN CHEM KLIN BIOCHEM 8 145 970 065 DAHLQVIST A CLIN SCI 30 517 966 066 ANTONOWICZ I PEDIATRICS 56 782 975 067 AURICCHIO S PEDIATRICS 35 944 965 068 SHEEHY TW AM J DIS CHILD 121 464 971 069 ANTONOWICZ I GASTROENTEROLOGY 67 51 974 070 ANTONOWICZ I BIOL NEONATE 32 280 977 071 ANDERSEN H ACTA PATH MICROBIOL SCAND 61 377 964 072 WATZKA M VERH ANAT GES 66 971 073 BIERRING F ACTA PATH MICROBIOL SCAND 61 365 964 074 LEV R HISTOCHEMIE 29 103 972 076 TAKIMOTO M BIOL NEONATE 18 66 971 077 ELIAKIS E MED LEG DOMM CORP 4 163 971 078 ASENSIO C MEDICAMENTA MARK 15 51 956 079 HERNANDEZ-JODRA M ENZYME 12 682 971 081 CLATWORTHY HW SURGERY 39 131 956 082 ROSENSTEIN BJ AM J DIS CHILD 132 167 978 083 EMERY JL ARCH DIS CHILD 29 34 954 084 GRIFFITHS AD BIORHEOLOGY 13 225 976 085 YOUNG DM PROC SOC EXP BIOL MED 99 673 958 086 FARBER S J PEDIATR 24 387 944 087 NOBLETT HR J PEDIATR SURG 4 190 969 088 GLANZMANN E ANN PAEDIATR 175 33 950 089 LANDSTEINER K ZENTRALBL ALLG PATHOL 16 903 905 090 NEUHAUSER EBD RADIOLOGY 46 319 946 091 DONNISON AB PEDIATRICS 37 833 966 092 HOLSCLAW DS AM J DIS CHILD 109 101 965 093 ANON CF FND 1976 REP SURVI STUD PA 3 973 094 BERNSTEIN JG AM J DIS CHILD 99 804 960 095 GROSS RE SURGERY OF INFANCY AND CHILDH 953 096 SANTULLI TV IN: BENSON CD 953 097 BISHOP HC ANN SURG 145 410 957 098 SHWACHMAN H IN: KENDIG EL JR 1 524 972 099 ELIAN E N ENGL J MED 264 13 961 100 GREEN MN PEDIATRICS 21 635 958 101 WISER WC PEDIATRICS 33 115 964 102 CHODOS DD PROC SOC EXP BIOL MED 99 775 958 103 RULE AH PEDIATRICS 45 847 970 104 RULE AH PEDIATRICS 48 601 971 105 RYLEY HC ARCH DIS CHILD 49 901 974 106 PECAU Y BIOL GASTROENTEROL 8 193 975 107 ZUELZER WW PEDIATRICS 4 53 949 108 OPPENHEIMER EH ARCH PATHOL 96 149 973 109 THOMAIDIS TS J PEDIATR 63 444 963 110 HINDEN E ARCH DIS CHILD 25 99 950 111 STEPHAN U IN: MANGOS JA 281 973 112 GOTTSCHALK B DTSCH GESUNDHEITSW 32 2439 977 113 HOLSCLAW DS PEDIATR ANN 7 29 968 114 STEPHAN U PEDIATRICS 55 35 975 116 ALPERS DH BIOCHIM BIOPHYS ACTA 401 28 975 117 SEETHARAM B LIFE SCI 18 89 976 118 ANTONOWICZ I LANCET 1 746 976 119 SHWACHMAN H AM J DIS CHILD 132 112 978 120 SHWACHMAN H IN: MANGOS JA 277 973 CT 1 PARK RW GASTROENTEROLOGY 81 1143 981 2 KNIGHT PJ SURGERY 89 332 981 3 SHWACHMAN H J PEDIATR SURG 18 570 983 4 KOPELMAN H N ENGL J MED 312 329 985 5 EGGERMONT E ACTA PAEDIATR SCAND SUPPL 317 1985 16 985 6 DEHNER LP HUM PATHOL 17 807 986 7 KING A PEDIATR RES 20 536 986 8 HUNSINGER RN CLIN CHIM ACTA 156 165 986 PN 79012 RN 00992 AN 79184947 AU Palin-D. Underwood-B-A. Denning-C-R. TI The effect of oral zinc supplementation on plasma levels of vitamin A and retinol-binding protein in cystic fibrosis. SO Am-J-Clin-Nutr. 1979 Jun. 32(6). P 1253-9. MJ CYSTIC-FIBROSIS: me. RETINOL-BINDING-PROTEINS: me. VITAMIN-A: bl. ZINC: pd. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: dt. DIET. GROWTH: de. HAIR: me. HUMAN. PLACEBOS. TASTE: de. TIME-FACTORS. ZINC: bl, tu. AB Oral zinc supplementation with 100 mg zinc sulfate over an 8-week period in 36 patients with cystic fibrosis and 17 siblings did not affect the vitamin A transport system, as evidenced by an absence of consistent and significant quantitative changes in plasma levels of vitamin A, retinol-binding protein, and zinc, nor did the exogenous zinc appear to stimulate growth or exert any clinical benefit to the cystic fibrosis patients. The lack of response to zinc supplementation might be explained by the fact that the group of cystic fibrosis subjects studied was fairly healthy in regard to clinical status and certain blood parameters, including zinc. RF 001 MAY CD J CLIN INVEST 27 226 948 002 BENNETT MJ AM J CLIN NUTR 20 415 967 003 UNDERWOOD BA BULL NY ACAD MED 47 34 971 004 ANDERSEN DH J PEDIATR 15 763 939 005 SMITH FR J LAB CLIN MED 80 423 972 006 NAVAB M J BIOL CHEM 252 5107 977 007 STEVENSON J J ANIMAL SCI 15 1036 956 008 SMITH JC JR SCIENCE 181 954 973 009 SMITH JC JR J NUTR 106 569 976 010 CARNEY SM J NUTR 106 1773 976 011 HALSTED JA LANCET 1 322 970 012 DISCHE Z PEDIATRICS 24 74 959 013 CAGGIANO V AM J MED SCI 257 305 969 014 UNDERWOOD BA PEDIATR RES 6 26 972 015 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 016 ANON NUTRITIVE VALUE OF FOODS HOME 971 017 PRASAD AS ZINC METABOLISM 966 018 LARKIN D FOOD RES 19 211 954 019 SCHROEDER HA J CHRON DIS 20 179 967 020 GORMICAN A J AM DIET ASSOC 56 397 970 021 MURPHY EW J AM DIET ASSOC 66 345 975 022 HENKIN RI J CLIN INVEST 42 727 963 023 NEELD JB J NUTR 79 454 963 024 MANCINI G IMMUNOCHEMISTRY 2 235 965 025 HACKLEY B CLIN CHEM 14 1 968 026 ANON MANUAL FOR NUTRITION SURVEYS 133 963 027 HENRY R AM J CLIN PATHOL 34 381 960 028 KALOW W CAN J BIOCHEM PHYSIOL 33 568 955 029 RICHTERICH R CLINICAL CHEMISTRY THEORY AND 969 030 DIXON R J CLIN PATHOL 27 341 974 031 KLEVAY LM AM J CLIN NUTR 23 284 970 032 STEEL RGD PRIN PROC OF STATISTICS 960 033 NIE NH STAT PACKAGE FOR THE SOCIAL S 975 034 BAKER H AM J CLIN NUTR 20 850 967 035 SMITH FR J CLIN INVEST 50 2426 971 036 HARPER HA REVIEW PHYSIOLOGICAL CHEMI 201 969 037 ANON RECOMMENDED DIETARY ALLOWANCE 974 038 JACOB RA AM J CLIN NUTR 31 638 978 039 PETERSEN RA AM J DIS CHILD 116 662 968 040 BLACKFAN KD J PEDIATR 13 627 938 041 UNDERWOOD BA WORLD REV NUTR DIET 19 123 974 042 KOPITO L NATURE 202 501 964 043 KOPITO L AM J CLIN NUTR 21 533 968 044 LOBECK CC IN: STANBURY JB 1605 972 045 MCCOLLUM AT J PEDIATR 77 571 970 CT 1 NAVARRO J AM J CLIN NUTR 34 1439 981 2 SOLOMONS NW AM J CLIN NUTR 34 462 981 3 GORDON EF J PEDIATR 99 341 981 4 JOHNSON MA AM J CLIN NUTR 35 1332 982 5 SOLOMONS NW NUTR REV 41 33 983 6 RUSSELL RM ANN INTERN MED 99 227 983 7 GAETANI S NUTR REP INT 30 111 984 8 BALY DL AM J CLIN NUTR 40 199 984 9 HUBBARD VS SEM RESPIR MED 6 308 985 10 BAER MT AM J CLIN NUTR 41 1220 985 11 DUHAMEL JF ARCH FR PEDIATR 43 229 986 PN 79013 RN 00993 AN 80062331 AU Roy-C-C. Delage-G. Fontaine-A. Robitaille-L. Chartrand-L. Weber-A. Morin-C-L. TI The fecal microflora and bile acids in children with cystic fibrosis. SO Am-J-Clin-Nutr. 1979 Dec. 32(12). P 2404-9. MJ ANTIBIOTICS: tu. BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: me, mi. FECES: an, mi. MN CARBENICILLIN: tu. CHILD. CHILD-PRESCHOOL. CLOXACILLIN: tu. CYSTIC-FIBROSIS: dt. DRUG-THERAPY-COMBINATION. GENTAMICINS: tu. GRAM-NEGATIVE-AEROBIC-BACTERIA. HUMAN. INFANT. AB Fecal fat, bile acids, and the rectal microflora were studied in seven control children and in three groups of patients with cystic fibrosis (CF). Seven were off antibiotics, ten on oral cloxacillin, and six on i.v. triple therapy (cloxacillin, gentamycin, and carbenicillin) for at least 2 weeks. Controls had lower concentrations (milligrams per gram dry stool) of both fat (P < 0.005) and vile acids (P < 0.025) than CF children off antibiotics. Antibiotics had little or no effect on fat but led to a striking decrease (P < 0.01) of bile acids in the triple therapy group. Concomitantly, there was reduced deconjugation of bile acids (P < 0.01) and formation of secondary bile acids (P < 0.005). This was associated with a 5-fold increase in the percentage of bile acids found in the aqueous phase of stools and with a marked reduction (P < 0.001) of the anaerobic flora (log counts per gram wet stool) in children on triple therapy (4.3 plus or minus 1.9) when compared to controls (9.8 plus or minus 0.1), CF off antibiotics (9.1 plus or minus 0.1) and CF on cloxacillin (9.6 plus or minus 0.2). The close relationship between the anaerobic flora, the extent of bile acid microbial transformation, and fecal bile acid concentrations suggest that reduction of the anaerobic flora decreases adsorption of bile acids to dietary residues and microbes, favors bile acid absorption, and leads to a decreased fecal loss of bile acids in CF. RF 001 ROY CC CLIN GASTROENTEROL 6 377 977 002 ROY CC N ENGL J MED 297 1301 977 003 WEBER AM GUT 17 295 976 004 WEBER AM N ENGL J MED 289 1001 973 005 LEFEBVRE D EXPERIENTIA 33 616 977 006 HOLDEMAN LV ANAEROBE LABORATORY MANUAL 975 007 BEST WR AM J CLIN NUTR 23 1609 970 008 JEEJEEBHOY KN CLIN BIOCHEM 3 157 970 009 LEVIN SJ ANAL CHEM 33 1407 961 010 WEBER AM CLIN CHIM ACTA 39 524 972 011 BANCROFT TA TOPICS IN INTERMED STAT METH 1 100 968 012 HOLDEMAN LV APPL ENVIRON MICROBIOL 31 539 976 013 MOORE WEC APPL MICROBIOL 27 961 974 014 ATTEBERY HR AM J CLIN NUTR 25 1931 972 015 MATA LJ AM J CLIN NUTR 25 1380 972 016 FLOCH MH AM J CLIN NUTR 25 1418 972 017 SUTTER VL J INFECT DIS 131 417 975 018 SUTTER VL ANTIMICROB AGENTS CHEMOTHER 10 736 976 019 MIDTVEDT T AM J CLIN NUTR 27 1341 974 020 GARBUTT JT GASTROENTEROLOGY 56 711 969 021 KRAG E J CLIN INVEST 53 1686 974 022 GUSTAFSSON BE ACTA PATH MICROBIOL SCAND 72 433 968 023 EASTWOOD MA BIOCHIM BIOPHYS ACTA 152 165 968 024 GUSTAFSSON BE SCAND J GASTROENTEROL 3 125 968 025 SCHIFF ER J CLIN INVEST 51 1351 972 026 EASTWOOD M AM J CLIN NUTR 29 1461 976 CT 1 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 2 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 3 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 4 WESLEY A PEDIATR RES 17 65 983 5 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 6 TAZAWA Y J PEDIATR GASTROENTEROL NUTR 3 378 984 7 GRYBOSKI JD AM J GASTROENTEROL 80 871 985 8 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 9 LEROY C DIG DIS SCI 31 911 986 10 LONNQVIST B J INFECT DIS 153 175 986 PN 79014 RN 00994 AN 79184974 AU Lloyd-Still-J-D. Demers-L-M. TI Serum glycine-conjugated bile acids in pediatric hepatobiliary disorders. SO Am-J-Clin-Pathol. 1979 Apr. 71(4). P 444-51. MJ BILE-ACIDS-AND-SALTS: bl. CYSTIC-FIBROSIS: bl. INFANT-NEWBORN-DISEASES: bl. INTESTINAL-DISEASES: bl. LIVER-DISEASES: bl. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CHOLESTASIS: bl. DIARRHEA-INFANTILE: bl. GLYCOCHENODEOXYCHOLIC-ACID: bl. GLYCOCHOLIC-ACID: bl. GLYCODEOXYCHOLIC-ACID: bl. HEPATITIS: bl. HUMAN. INFANT. INFANT-NEWBORN. REYES-SYNDROME: bl. AB Measurements of serum bile acids (glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and lithocholic acids) by radioimmunoassay in a variety of pediatric hepatobiliary disorders showed elevations in neonatal hepatitis syndromes, cholestasis, and hepatitis of extrahepatic or intrahepatic origin. Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. In one patient with cholestasis, the increased levels of bile acids observed returned to normal following therapy with cholestyramine and phenobarbital. In chronic active hepatitis the serum bile acid values correlated well with the bilirubin and SGOT in response to therapy with corticosteroids. These data confirm suggestions that serum cholylglycine and chenodeoxycholylglycine levels are a sensitive indicator of disturbed hepatic function and can be used in monitoring the course, activity, and therapeutic response in various hepatitis syndromes. In Reye's syndrome and protracted diarrhea of infancy, elevations in serum bile acids were detected without associated hyperbilirubinemia and provided additional evidence of disturbed hepatic function. RF 001 BALISTRERI WF IN: POLLACK JD 269 975 002 BALISTRERI WF PEDIATR RES 8 378 974 003 BOVE KE GASTROENTEROLOGY 69 685 975 004 BURKE V AUST PAEDIATR J 2 219 966 005 DASHER CA CLIN RES 24 11A 976 006 DEMERS LM CLIN CHEM 22 602 976 007 DI SANTAGNESE PA GASTROENTEROLOGY 40 64 961 008 GOODCHILD MC ARCH DIS CHILD 50 769 975 009 GRACEY M GUT 12 683 971 010 JAVITT NB POSTGRAD MED J 50 354 974 011 JAVITT NB GASTROENTEROLOGY 70 1172 976 012 JAVITT NB J CLIN INVEST 47 1002 968 013 JAVITT NB J PEDIATR 90 786 977 014 KAPLOWITZ N JAMA 225 292 973 015 KORMAN MG N ENGL J MED 290 1399 974 016 LANDING BH PROG PEDIATR SURG 6 113 974 017 LLOYD-STILL JD PEDIATRICS 59 626 977 018 LLOYD-STILL JD AM J DIS CHILD 125 358 973 019 MURPHY GM GUT 15 151 974 020 NORMAN A ACTA PAEDIATR SCAND 62 253 973 021 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 022 POLEY JR J LAB CLIN MED 63 838 964 023 SANDBERG DH PEDIATR RES 4 262 970 024 SCHEUER P LIVER BIOPSY INTERPRETATION 22 973 025 TOULOUKIAN RJ ARCH SURG 106 58 973 026 UTILI R GASTROENTEROLOGY 70 248 976 027 WATKINS JB PEDIATR CLIN NORTH AM 21 501 974 028 WATKINS JB PEDIATR RES 7 341 973 029 WATKINS JB GASTROENTEROLOGY 67 835 974 030 WEBER AM GUT 17 295 976 031 WEBER AM N ENGL J MED 289 1001 973 CT 1 FINNI K ANN CLIN RES 12 168 980 2 MATSUI A J CLIN PATHOL 35 1011 982 3 FINNI K EUR J PEDIATR 138 53 982 4 STREET JM J LIPID RES 24 491 983 5 STRANDVIK B SCAND J GASTROENTEROL 20 381 985 PN 79015 RN 00995 AN 79252922 AU Warwick-W-J. Hansen-L. Viela-I. Matheson-J. TI Comparison of the chloride electrode and gravimetric chloride titration sweat tests. SO Am-J-Clin-Pathol. 1979 Aug. 72(2). P 142-5. MJ CHLORIDES. CYSTIC-FIBROSIS: di. SWEATING. MN COMPARATIVE-STUDY. COMPUTERS. ELECTRODES. HUMAN. MODELS-BIOLOGICAL. PROBABILITY. AB The chloride electrode sweat test and gravimetric chloride titration technic are compared. The two technics gave comparable mean values, ogives, equivalent points, and overlap for patients with cystic fibrosis and healthy subjects. As a single test with either technic has an unacceptable risk for false-positive or false-negative results, tests should always be done in pairs. A statistical interpretation technic based on prevalence and technical variability of the two tests will decrease the number of undiagnosed subjects and will define probability of cystic fibrosis in the undiagnosed group. RF 001 GIBSON LE CLIN PEDIATR 12 450 973 002 GIBSON LE PEDIATRICS 23 545 959 003 HANSEN L MINN MED 50 1191 967 004 HANSEN L AM J CLIN PATHOL 49 834 968 005 KOPITO L PEDIATRICS 43 794 969 006 ANON J PEDIATR 88 711 976 007 WARWICK WJ CF CLUB ABST 5 975 008 WARWICK WJ PEDIATRICS 36 261 965 009 WARWICK WJ CLIN CHEM 24 381 978 CT 1 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 PN 79016 RN 00996 AN 79252934 AU Warwick-W-J. Viela-I. Hansen-L-G. TI Comparison of the errors due to the use of gauze and the use of filter paper in the gravimetric chloride titration sweat test. SO Am-J-Clin-Pathol. 1979 Aug. 72(2). P 211-5. MJ CHLORIDES. CYSTIC-FIBROSIS: di. SWEATING. MN COMPARATIVE-STUDY. HUMAN. MATHEMATICS. AB Potential sources of error in the Gibson and Cooke method of sweat testing using gauze and filter paper as collection media as described. The absolute chloride content of gauze was higher than that of filter paper; however, the variability of chloride contents between the two was insignificant. Complete chloride elution from filter paper requires more time than elution from gauze. A method of determining probability of a clinically acceptable measurement based on sweat sample size, chloride determination, and variability of chloride in collection media is also described. RF 001 GIBSON LE PEDIATRICS 23 545 959 002 HANSEN L MINN MED 50 1191 967 003 SCHALES O J BIOL CHEM 140 879 941 004 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 CT 1 SCHWACHMAN H J PEDIATR 98 576 981 PN 79017 RN 00997 AN 79253016 AU Nussbaum-E. Boat-T-F. Wood-R-E. Doershuk-C-F. TI Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy. SO Am-J-Dis-Child. 1979 Sep. 133(9). P 965-6. MJ ALKALOSIS: et. WATER-ELECTROLYTE-IMBALANCE: et. MN ALKALOSIS: th. CASE-REPORT. COLD. CYSTIC-FIBROSIS: co. FEMALE. FLUID-THERAPY. HUMAN. INFANT. MALE. SEASONS. SWEATING. SUPPORT-U-S-GOVT-P-H-S. WATER-ELECTROLYTE-IMBALANCE: th. EX We describe two infants with previously undiagnosed cystic fibrosis who had electrolyte depletion, metabolic alkalosis, and dehydration in the winter and three infants known to have CF in whom similar manifestations developed. We confirm previous reports that salt depletion and metabolic alkalosis do occur throughout the year in the northern United States and England. When metabolic alkalosis and hypoelectrolytemia in infancy are not explained on the basis of other clinical conditions, CF should be suspected. Pediatricians should measure serum electrolyte values promptly and consider early parenteral fluid and electrolyte therapy as a preventive measure for infants with CF with persistent vomiting and/or diarrhea. RF 001 KESSLER WR PEDIATRICS 8 648 951 002 GIBSON LE PEDIATRICS 23 545 959 003 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 004 DI SANTAGNESE PA JAMA 172 2014 960 005 GOTTLIEB RP J PEDIATR 79 930 971 006 DI SANTAGNESE PA PEDIATRICS 12 549 953 007 STOLL E HELV PAEDIATR ACTA 25 234 970 008 BECKERMAN RC PEDIATRICS 63 580 979 009 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 010 HOCHMAN HI ARCH DIS CHILD 51 390 976 CT 1 LAUGHLIN JJ PEDIATRICS 68 203 981 2 RAPAPORT R J PEDIATR 98 768 981 3 SORSCHER EJ LANCET 1 368 982 4 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 5 ORENSTEIN DM PEDIATR RES 17 267 983 6 ORENSTEIN DM J APPL PHYSIOL 57 408 984 PN 79018 RN 00998 AN 79185026 AU Mischler-E-H. Chesney-P-J. Chesney-R-W. Mazess-R-B. TI Demineralization in cystic fibrosis detected by direct photon absorptiometry. SO Am-J-Dis-Child. 1979 Jun. 133(6). P 632-5. MJ BONE-AND-BONES: me. BONE-DISEASES: et. CYSTIC-FIBROSIS: co. GROWTH-DISORDERS: et. MINERALS: me. MN ADOLESCENCE. ADULT. ALKALINE-PHOSPHATASE: bl. BONE-DEVELOPMENT. BONE-DISEASES: di, me. CALCIUM: bl, me. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: me. DENSITOMETRY. FEMALE. GROWTH-DISORDERS: me. HUMAN. MALE. METHODS. PHOSPHORUS: bl. AB Bone mineral content, bone width, and their ratio were measured in patients with cystic fibrosis (CF) using monoenergetic photon absorptiometry. Serial measurements of the radius and ulna were made in 27 patients with CF and were compared with 968 age-matched controls. Demineralization was found in 37% of the boys and 63% of the girls. Patients under age 10 years had normal bone mineral content and nine of 15 patients aged 13 or older were demineralized (P less than .01). Demineralization correlated with the extent of weight reduction in patients (P less than .001). Patients most likely to be demineralized were adolescent girls. To our knowledge, this is the first report of bone mineral status of children with CF, and the results indicate that a sizable proportion of these patients may be demineralized without overt rickets. RF 001 NADLER HL IN: STANBURY JB 1683 978 002 SCOTT J AM J MED 63 488 977 003 AVIOLI LV METABOLISM 22 507 973 004 CHESNEY RW PEDIATRICS 60 864 977 005 CHESNEY RW AM J DIS CHILD 132 768 978 006 LEVIN ME N ENGL J MED 294 241 976 007 CHRISTIANSEN C AM J ROENTGENOL 126 1302 976 008 CAMERON JR INVEST RADIOL 3 141 968 009 MAZESS RB INVEST RADIOL 6 52 971 010 CHRISTIANSEN C SCAND J CLIN LAB INVEST 35 425 975 011 MAZESS RB GROWTH 36 77 972 012 MAZESS RB AM J ANTHROPOL 35 399 971 013 MAZESS RB IN: MAZESS RB 228 974 014 TAUSSIG LM J PEDIATR 82 380 973 015 SPROUL A J PEDIATR 65 664 964 016 ANON MONTHLY VITAL STAT REPORT USD 25 76 976 017 LACHMAN E AM J ROENTG RAD THER NUCL MED 74 712 955 018 SIWE SA DTSCH ARCH KLIN MED 173 339 932 019 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 020 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 021 PARK EA ARCH DIS CHILD 29 269 954 022 BYERS PD IN: AVIOLI LV 184 977 023 COOKE RE J PEDIATR 57 326 960 024 SIMOPOULOS AP PEDIATR RES 6 95 972 025 WEBER AM GUT 17 295 976 026 FARRELL PM PEDIATR RES 11 113 977 027 GALLAGHER JC N ENGL J MED 298 193 978 CT 1 PITT MJ RADIOL CLIN NORTH AM 19 581 981 2 SOLOMONS NW AM J CLIN NUTR 34 462 981 3 CHESNEY RW AM J DIS CHILD 136 578 982 4 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 5 FARRELL PM J DENT CHILD 50 385 983 6 LANDIN LA ACTA ORTHOP SCAND 54 1 983 7 HUBBARD VS SEM RESPIR MED 6 308 985 8 HELIN I ACTA PAEDIATR SCAND 74 264 985 9 REITER EO J PEDIATR 106 21 985 10 STEAD RJ CLIN ENDOCRINOL 26 187 987 PN 79019 RN 00999 AN 79142382 AU Strauss-G-D. Pedersen-S. Dudovitz-D. TI Psychosocial support for adults with cystic fibrosis: a group approach. SO Am-J-Dis-Child. 1979 Mar. 133(3). P 301-5. MJ CYSTIC-FIBROSIS: px. SELF-HELP-GROUPS. MN ADAPTATION-PSYCHOLOGICAL. ADULT. ATTITUDE-TO-HEALTH. FEMALE. HUMAN. MALE. NURSING-STAFF-HOSPITAL. PSYCHIATRY. ROLE. SOCIAL-WORK. AB This article describes the first time-limited psychosocial support group for adults with cystic fibrosis (CF).The chronology of the group is traced, focusing on issues of importance for health professionals who work with patients with CF. Some of the issues raised by patient members include doubts about the competence of nonpediatricians to adequately treat CF; resentment towards "normals" (those without CF), including health professionals; and the desire to control as many areas of their lives as possible. Covert (ie, unrecognized) issues, the role of staff, risks, and outcome are detailed as a guide for others who may wish to conduct such groups. The literature on groups for medical patients and a rationale for the beneficial effects on the group are given. RF 001 BOWMAN BH N ENGL J MED 294 937 976 002 WARWICK WJ JAMA 238 2159 977 003 ROSENLUND ML ANN INTERN MED 78 959 973 004 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 005 BOYLE IR J PEDIATR 88 318 976 006 DENNING CR IN: MANGOS JA 127 976 007 PRATT JH JAMA 49 755 907 008 CUNNINGHAM J COMPR PSYCHIATRY 19 135 978 009 REID J GERIATRICS 17 823 962 010 DUDLEY DL AM REV RESPIR DIS 100 575 969 011 FORTIN JN INT J GROUP PSYCHOTHER 6 838 956 012 GUY W ARCH DERMATOL SYPHIL 70 767 954 013 ADSETT CA CAN MED ASSOC J 99 577 968 014 LONG RT J NERV MENT DIS 119 366 954 015 TEITELBAUM SH GROUP PSYCHOTHER 17 49 964 016 PARSELL S AM J NURS 74 650 974 017 SCHOENBERG B J CHRON DIS 19 649 966 018 COOKE M CF SOCIALWORK HIGHLIGHTS 1 5 977 CT 1 STRAUSS GD J CHRON DIS 34 141 981 PN 79020 RN 01000 AN 79121969 AU di-Santagnese-P-A. Davis-P-B. TI Cystic fibrosis in adults. 75 cases and a review of 232 cases in the literature. SO Am-J-Med. 1979 Jan. 66(1). P 121-32. MJ CYSTIC-FIBROSIS: co. INTESTINAL-OBSTRUCTION: et. LUNG-DISEASES-OBSTRUCTIVE: et. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: mi, ra. FEMALE. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: di. LUNG: ra. MALE. RESPIRATORY-FUNCTION-TESTS. SPUTUM: mi. AB Cystic fibrosis is now the most common cause of chronic obstructive pulmonary disease (COPD) and of pancreatic insufficiency in the first three decades of life in the United States. In this report we describe 75 patients with cystic fibrosis aged 18 to 47 years and review another 232 cases reported in the literature. All of these 307 patients had elevated sweat chloride and sodium levels, which proved excellent discriminants for cystic fibrosis even in patients in the older age group. COPD, present in 97 per cent, was the major cause of morbidity and mortality, and differed from COPD of other etiologies. The progressive downhill course in these patients was punctuated by recurrent symptomatic exacerbations of chronic bacterial bronchitis caused by Pseudomonas aeruginosa and Staphyloccocus aureus, and terminated in pulmonary insufficiency, cor pulmonale and death. COPD was complicated by minor hemoptysis in 60 per cent, massive hemoptysis in 7 per cent and pneumothorax in 16 per cent, problems rare in children. Sinusitis was present in all those examined roentgenographically, and 48 per cent had nasal polyposis. Pancreatic insufficiency was present in 95 per cent of the patients, but in contrast to younger patients it was seldom symptomatic although steatorrhea and azotorrhea were still massive. Intussusception and meconium ileus equivalent (fecal accumulation) are frequent in adults (21 per cent) but rare in children, and they require immediate diagnostic and therapeutic intervention with enemas of diatrizoate sodium. Glycosuria, biliary cirrhosis, cholelithiasis and aspermia were among other complications. Height and weight were usually within the lower limits of normal, but 17 per cent of the men were above 180 cm in height and 7 per cent were overweight. Therefore, a high index of suspicion is needed to make the diagnosis, because older patients with cystic fibrosis may look quite well. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 MANGOS JA TEX REP BIOL MED 31 651 973 003 KAISER D PEDIATR RES 5 167 971 004 SCHULZ IJ J CLIN INVEST 48 1470 969 005 ANON CF FND 1974 REP SURVI STUD PA 976 006 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 007 MITCHELL-HEGGS PF Q J MED 45 479 976 008 GRACEY M AUSTRALAS ANN MED 18 91 969 009 STERN RC ANN INTERN MED 87 188 977 010 SCHWARTZ EE AM J ROENTG RAD THER NUCL MED 122 708 974 011 HODSON ME BR MED J 2 790 976 012 FRIEDMAN PJ AM REV RESPIR DIS 113 159 976 013 GIBSON LE PEDIATRICS 23 545 959 014 SCHWERT GW BIOCHIM BIOPHYS ACTA 16 570 955 015 ANDERSEN DH AM J DIS CHILD 63 643 942 016 FOLK JE J BIOL CHEM 235 2272 960 017 VAN LOON EJ AM J CLIN PATHOL 22 1134 952 018 SUN DCH GASTROENTEROLOGY 44 602 963 019 TAUSSIG LM PEDIATRICS 54 229 974 020 VAN DE KAMER JH J BIOL CHEM 177 347 949 021 WOLLAEGER EE GASTROENTEROLOGY 9 272 947 022 TANNER JM ARCH DIS CHILD 41 454 966 023 NICKLAUS TM AM REV RESPIR DIS 93 889 966 024 TAUSSIG LM J PEDIATR 82 380 973 025 JENSEN KG ACTA PAEDIATR SCAND 51 344 962 026 DI SANTAGNESE PA IN: DOWNEY JA 25 974 027 WARWICK WJ J CHRON DIS 28 609 975 028 STERN RC J PEDIATR 89 406 976 029 KRAEMER R ACTA PAEDIATR SCAND 67 33 978 030 ORENSTEIN DM AM J DIS CHILD 131 973 977 031 WOOD RE AM REV RESPIR DIS 113 833 976 032 ANON J PEDIATR 88 711 976 033 DI SANTAGNESE PA PEDIATRICS 12 178 953 034 ELIASSON R N ENGL J MED 297 1 977 035 COREY M AM REV RESPIR DIS 114 1085 976 036 LANDAU LI AM REV RESPIR DIS 108 593 973 037 ZAPLETAL A PEDIATRICS 48 64 971 038 FOX WW PEDIATRICS 54 293 974 039 COOPER DM AM REV RESPIR DIS 109 519 974 040 REYNOLDS HY JAMA 236 2190 976 041 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 042 BEDROSSIAN CWM HUM PATHOL 7 195 976 043 MILLIS RM CHEST 71 508 977 044 DAVIS PB JAMA 239 1851 978 045 BOAT TF AM REV RESPIR DIS 116 1 977 046 TAGER I N ENGL J MED 292 563 975 047 ROSENTHAL A PEDIATRICS 59 588 977 048 SIASSI B J PEDIATR 78 794 971 049 ROSENTHAL A PEDIATRICS 59 588 977 050 RYSSING E ACTA PAEDIATR SCAND 66 753 977 051 LEVITSKY S JAMA 213 125 970 052 FELLOWS KE RADIOLOGY 114 551 975 053 SCHUSTER SR J PEDIATR SURG 12 889 977 054 LUCK SR J THORAC CARDIOVASC SURG 74 834 977 055 KATTWINKEL J JAMA 226 557 973 056 TAUSSIG LM J PEDIATR 90 574 977 057 COHEN LF J PEDIATR 90 574 977 058 SHWACHMAN H PEDIATRICS 30 389 962 059 CAPLIN I ANN ALLERGY 29 631 971 060 KULCZYCKI LL JAMA 175 358 961 061 RACHELEFSKY GS AM J DIS CHILD 128 355 974 062 WARNER JO ARCH DIS CHILD 51 507 976 063 KOPITO LE PEDIATR RES 10 742 976 064 DI SANTAGNESE PA IN: YOUNG DS 976 065 SHWACHMAN H PEDIATRICS 55 86 975 066 LAPEY A J PEDIATR 84 328 974 067 ROY CC N ENGL J MED 297 1301 977 068 NOUSIA-ARVANITAKIS S J PEDIATR 90 302 977 069 UNDERWOOD BA PEDIATR RES 6 26 972 070 SCOTT J AM J MED 63 488 977 071 HUBBARD VS LANCET 2 1302 977 072 MULLINS F JAMA 192 741 965 073 MATSESHE JW GASTROENTEROLOGY 72 732 977 074 MCPARTLIN JF BR J SURG 60 707 973 075 KATTWINKEL J J PEDIATR 82 234 973 076 DI SANTAGNESE PA PEDIATRICS 18 387 956 077 CRAIG JM AM J DIS CHILD 93 357 957 078 STERN RC GASTROENTEROLOGY 70 645 976 079 HUBBARD VS PEDIATR RES 12 437 978 080 ISENBERG JN AM J GASTROENTEROL 65 134 976 081 WEBER AM N ENGL J MED 289 1001 973 082 HANDWERGER S N ENGL J MED 281 451 969 083 LIPPE BM J PEDIATR 90 751 977 084 STAHL M J PEDIATR 84 821 974 085 MULLER WA AM J MED 54 52 973 086 DI SANTAGNESE PA PEDIATRICS 12 549 953 087 TAUSSIG LM N ENGL J MED 287 586 972 088 HOLSCLAW DS J UROL 106 568 971 089 KAPLAN E N ENGL J MED 279 65 968 090 OPPENHEIMER EH J PEDIATR 75 806 969 091 SHWACHMAN H AM J DIS CHILD 96 6 958 092 HERROD HG J PEDIATR 91 276 977 093 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 094 KOPITO LE FERTIL STERIL 24 512 973 095 OPPENHEIMER EH J PEDIATR 77 991 970 CT 1 ANON BR MED J 2 626 979 2 GURWITZ D PEDIATR CLIN NORTH AM 26 603 979 3 HOLSCLAW DS CLIN CHEST MED 1 407 980 4 COHEN LF LANCET 2 842 980 5 LELANNOU D ANN UROLOGIE 14 94 980 6 HODSON ME THORAX 35 801 980 7 MATTHAY RA BR HEART J 43 474 980 8 DAVIS PB AM J MED 69 643 980 9 FAIRFAX AJ BR J DIS CHEST 74 345 980 10 SANDERS JS CHEST 77 226 980 11 HUBBARD VS AM J DIS CHILD 134 317 980 12 WILLI UV AM J ROENTGENOL 134 1005 980 13 HODSON ME PRACTITIONER 224 301 980 14 PASSERO MA CLIN PEDIATR 20 264 981 15 BLACKWOOD LL INFECT IMMUN 32 443 981 16 EVANS RT J CLIN PATHOL 34 911 981 17 RUTLAND J THORAX 36 654 981 18 LELANNOU D SEM HOP PARIS 57 608 981 19 MATTHAY RA MED CLIN NORTH AM 65 489 981 20 WELCH MJ PEDIATRICS 67 664 981 21 PENNINGTON JE J CLIN INVEST 68 1140 981 22 JACOBS WH AM J GASTROENTEROL 76 342 981 23 LAMBERT JR GASTROENTEROLOGY 80 169 981 24 FRIEDMAN PJ AM J ROENTGENOL 136 1131 981 25 HODSON ME EUR J PEDIATR 137 117 981 26 CORKEY CWB AM J OBSTET GYNECOL 140 737 981 27 OBOYLE CP IR J MED SCI 150 286 981 28 OGRADY JG IR J MED SCI 150 220 981 29 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 30 MACONE AB N ENGL J MED 304 1445 981 31 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 32 HOOGENRAAD TU NEUROOPHTHALMOL 2 267 982 33 JAGGER KS J CLIN MICROBIOL 15 1054 982 34 MCKIERNAN J PEDIATR RES 16 60 982 35 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 36 HARVEY J BR J HOSP MED 27 278 982 37 KISTLER I HELV PAEDIATR ACTA 36 495 982 38 OHMAN DE INFECT IMMUN 37 662 982 39 PENKETH ARL THORAX 37 850 982 40 HUBBARD VS J AM DIET ASSOC 80 127 982 41 NOLAN G J PEDIATR 101 626 982 42 RIVERA M AM REV RESPIR DIS 126 833 982 43 STERN RC AM J DIS CHILD 136 1067 982 44 TSCHOLAKOFF D FORTSCHR GEB RONTG NUKL 137 18 982 45 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 46 GUIDOTTI TL AM J MED SCI 283 157 982 47 BONFORTE RJ LANCET 1 71 983 48 SHAWKER TH J ULTRASOUND MED 2 439 983 49 WAGENER JS AM J PEDIATR HEMATOL ONCOL 5 153 983 50 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 51 BRECHER DB AM FAM PHYSICIAN 28 187 983 52 KRAMER NR J CAN ASSOC RADIOL 34 271 983 53 DAVIS PB J CHRON DIS 36 269 983 54 JEFFREY I J CLIN PATHOL 36 1292 983 55 DORING G INFECT IMMUN 42 197 983 56 GUIDOTTI TL RESPIRATION 44 351 983 57 JACOBS RL ANN ALLERGY 51 500 983 58 GEMBITSKAYA TE KLIN MED 61 9 983 59 JOHNSON SR OBSTET GYNECOL 61 S 2 983 60 DOHERTY DE SOUTH MED J 76 1580 983 61 ALLEN JM GASTROENTEROLOGY 85 1379 983 62 PORTER DK J THORAC CARDIOVASC SURG 86 409 983 63 ANON ANN INTERN MED 98 1033 983 64 DAVIS PB AM REV RESPIR DIS 128 34 983 65 PORTER DK ARCH INTERN MED 143 287 983 66 BOUCHER RC LUNG 161 1 983 67 ROSENBERG E KLIN PAEDIATR 195 323 983 68 HANLY JG BR MED J 286 1411 983 69 HODSON ME BR MED J 286 1381 983 70 ZENTLERMUNRO PL GUT 25 500 984 71 MCCLURE HM ADV VET SCI COMP MED 28 267 984 72 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 73 PENKETH A THORAX 39 299 984 74 COWEN L PSYCHOSOM MED 46 363 984 75 WILSONSHARP RC ARCH DIS CHILD 59 923 984 76 LEWIS MI S AFR MED J 65 641 984 77 ABRAMS CK J CLIN INVEST 73 374 984 78 SLAVIN RG J ALLERGY CLIN IMMUNOL 73 712 984 79 JACOBS RL J ALLERGY CLIN IMMUNOL 74 61 984 80 HODGES P J AM DIET ASSOC 84 664 984 81 DAVIS PB CHEST 85 802 984 82 MCCARTHY VP GASTROENTEROLOGY 86 564 984 83 SPINO M J PEDIATR 105 829 984 84 GUGGER M SCHWEIZ MED WOCHENSCHR 114 620 984 85 SIGG C SCHWEIZ MED WOCHENSCHR 114 1942 984 86 GEFFNER ME AM J DIS CHILD 138 677 984 87 HANDELSMAN DJ N ENGL J MED 310 1670 984 88 SPRINCE NL N ENGL J MED 310 375 984 89 PINKERTON P LANCET 2 761 985 90 CRAWFORD AM RHEUMATOL INT 5 283 985 91 DAVIS PB SEM RESPIR MED 6 261 985 92 DAVIS PB SEM RESPIR MED 6 314 985 93 DAVIS PB SEM RESPIR MED 6 319 985 94 HUBBARD VS SEM RESPIR MED 6 299 985 95 LESTER LA SEM RESPIR MED 6 285 985 96 SHAW LMA J OBSTET GYNAECOL BR COMMONW 6 1 985 97 SWOBODNIK W J CLIN ULTRASOUND 13 469 985 98 REINIG JW PEDIATR RADIOL 15 222 985 99 SEAMAN WB HOSP PRACT 20 123 985 100 KAHAN A PHARMACOL THER 28 163 985 101 GRAHAM N CLIN RADIOL 36 199 985 102 TRENTO A ANN THORAC SURG 39 254 985 103 DAVIS PB THORAX 40 376 985 104 HUNT B THORAX 40 23 985 105 AMES P INFECT IMMUN 49 281 985 106 HANLY JG Q J MED 56 377 985 107 MEARNS MB ARCH DIS CHILD 60 272 985 108 BERKIN KE EUR J RESPIR DIS 67 103 985 109 ROTH RM SOUTH MED J 78 573 985 110 ISPIZUA AU MED CLIN 85 628 985 111 REITER EO J PEDIATR 106 21 985 112 MELLIS CM MED J AUST 143 227 985 113 OCONNOR KW ARCH INTERN MED 145 153 985 114 OHMAN DE J BACTERIOL 162 1068 985 115 ISPIZUA AU REV CLIN ESP 176 417 985 116 KNOPFLE G KLIN PAEDIATR 197 481 985 117 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 118 OHMAN DE EUR J CLIN MICROBIOL 5 6 986 119 PENNINGTON JE REV INFECT DIS 8 S426 986 120 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 121 KRIEG DP J CLIN MICROBIOL 24 986 986 122 PIER GB J CLIN MICROBIOL 24 189 986 123 OJEDA VJ DIS COLON RECTUM 29 567 986 124 ROBERTS JA SCOTT MED J 31 109 986 125 RODMAN HM MEDICINE 65 389 986 126 PIER GB J CLIN INVEST 77 491 986 127 DINWIDDIE R J ROY SOC MED 79 6 986 128 ROSENSTEIN BJ SOUTH MED J 79 319 986 129 WELLER PH J ROY SOC MED 79 36 986 130 TRAVIS WD AM J CLIN PATHOL 85 419 986 131 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 132 MCGLENNEN RC ARCH PATHOL LAB MED 110 879 986 133 COHEN AM AM J DIS CHILD 140 74 986 134 ROSENSTEIN BJ AM J DIS CHILD 140 966 986 135 KRUHLAK RT WEST J MED 145 196 986 136 STEINKAMP G EUR J PEDIATR 145 526 986 137 KUMARISUBAIYA S J PEDIATR GASTROENTEROL NUTR 6 71 987 138 STEAD RJ CLIN ENDOCRINOL 26 187 987 139 STEAD RJ THORAX 42 59 987 140 MCHUGO JM BR J RADIOL 60 137 987 141 DAVIS PB J DENT RES 66 667 987 142 BAILEY DJ PEDIATRICS 79 281 987 PN 79021 RN 01001 AN 80062584 AU Fisher-N-L. TI Genetic counseling and prenatal diagnosis of cystic fibrosis [letter]. SO Am-J-Obstet-Gynecol. 1979 Nov 1. 135(5). P 699. MJ CYSTIC-FIBROSIS: fg. GENETIC-COUNSELING. PRENATAL-DIAGNOSIS. MN CYSTIC-FIBROSIS: di. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. PREGNANCY. EX Turner and associates cited cystic fibrosis as an example of the application of the judicious use of genetic counseling and prenatal diagnosis. The reader who is unfamiliar with genetic disorders, prenatal diagnosis, and genetic counseling may have taken the "cystic fibrosis example" as fact rather than a model, as intended. Cystic fibrosis is a recessive disorder for which prenatal diagnosis and heterozygote detection are not yet possible. Currently, because of the present inability to detect the heterozygous state, genetic counseling of individuals with a family history of cystic fibrosis frequently means a statistical prediction is given, rather than a precise recurrence risk. RF 000 TURNER JH AM J OBSTET GYNECOL 134 83 979 PN 79022 RN 01002 AN 79206917 AU Imrie-J-R. Fagan-D-G. Sturgess-J-M. TI Quantitative evaluation of the development of the exocrine pancreas in cystic fibrosis and control infants. SO Am-J-Pathol. 1979 Jun. 95(3). P 697-707. MJ CYSTIC-FIBROSIS: pa. PANCREAS: pa. MN COMPARATIVE-STUDY. CONNECTIVE-TISSUE: pa. FEMALE. GESTATIONAL-AGE. HUMAN. INFANT-NEWBORN. MALE. MECONIUM. PREGNANCY. AB The development of the exocrine pancreas has been determined quantitatively in 31 infants with cystic fibrosis (CF) both with and without meconium ileus and in 29 control infants. In the normal pancreas, the ratio of acinar to connective tissue volume is 0.5 at 32 weeks postconceptional age (PCA) and increases linearly to 2.0 at 52 weeks PGA. In cystic fibrosis infants, with or without meconium ileus, the ration is 0.5 at 35 weeks PCA anddecreases linearly to 0.3 at 52 weeks PCA. The volume of acinar and duct lumens is greater in CF than control infants but is independent of age or acinar volume. The development of the exocrine pancreas in infants with CF with and without meconium ileus diverges from the normal pattern: There is consistent lack of exocrine tissue before or a full-term birth, which persists throghout the age range of this study. CF infants above 42 weeks PCA can be discriminated from controls on the basis of the quantitative assessment of acinar volume. RF 001 FARBER S J PEDIATR 24 387 944 002 ANDERSEN DH AM J DIS CHILD 56 344 938 003 KORNBLITH BA AM J PATHOL 5 249 929 004 HURWITT ES AM J DIS CHILD 64 443 942 005 GLANZMANN E ANN PAEDIATR 166 289 946 006 THOMAIDIS TS J PEDIATR 63 444 963 007 WEIBEL ER MORPHOMETRY OF THE HUMAN LUNG 963 008 OPPENHEIMER EH ARCH PATHOL 96 149 973 009 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 010 DONNISON AB PEDIATRICS 37 833 966 011 KOPITO LE PEDIATR RES 10 742 976 012 RICKHAM PP PROG PEDIATR SURG 2 73 971 CT 1 GILLARD BK PEDIATR RES 14 1168 980 2 CARREL O HELV PAEDIATR ACTA 36 405 981 3 PARK RW GASTROENTEROLOGY 81 1143 981 4 STURGESS J AM J PATHOL 106 303 982 5 REID L INT REV EXP PATHOL 24 335 983 6 MASOERO G AM J DIS CHILD 137 167 983 7 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 8 GOTZ M ATEMWEGS LUNGENKRANKH 10 594 984 9 LANGLEY FA CLIN OBSTET GYNAECOL 11 79 984 10 MULLER F PRENAT DIAGN 5 109 985 11 HUBBARD VS SEM RESPIR MED 6 299 985 12 GOTZ M MED KLIN 80 657 985 13 FRIEND PA J INFECT 13 55 986 14 DODGE JA J ROY SOC MED 79 27 986 15 HUNSINGER RN CLIN CHIM ACTA 156 165 986 PN 79023 RN 01003 AN 79185761 AU Larsen-G-L. Barron-R-J. Cotton-E-K. Brooks-J-G. TI A comparative study of inhaled atropine sulfate and isoproterenol hydrochloride in cystic fibrosis. SO Am-Rev-Respir-Dis. 1979 Mar. 119(3). P 399-407. MJ ATROPINE: du. CYSTIC-FIBROSIS: pp. ISOPROTERENOL: du. RESPIRATION: de. MN ADOLESCENCE. AEROSOLS. AIRWAY-RESISTANCE: de. ATROPINE: ad, pd. CHILD. COMPARATIVE-STUDY. DOSE-RESPONSE-RELATIONSHIP-DRUG. FEMALE. HUMAN. ISOPROTERENOL: ad, pd. MALE. MAXIMAL-EXPIRATORY-FLOW-RATE. MAXIMAL-EXPIRATORY-FLOW-VOLUME-CURVES. RESIDUAL-VOLUME. TOTAL-LUNG-CAPACITY. VITAL-CAPACITY. AB To investigate the contribution of vagal tone to the obstructive airway disease in cystic fibrosis, we evaluated the pulmonary function response to administration of 0.1 mg of atropine sulfate per kg of body weight, 0.05 mg of isoproterenol hydrochloride per kg of body weight, or isotonic saline (control solution) administered by nebulization on 3 different days to each of 10 patients with cystic fibrosis in a single blind study. The doses of atropine sulfate and isoproterenol hydrochloride were based on published dose-response characteristics of the drugs. Each day, thoracic gas volume and airway resistance were measured in a volume displacement body plethysmograph, and maximal expiratory flow-volume curves were performed before and at 15, 30, and 60 min after the inhalation. After inhalation of saline, group mean values for all pulmonary functions indicated greater airway obstruction at 15, 30, and 60 min when compared to pretreatment values. After inhalation of isoproterenol and atropine, significant improvement (P < 0.05) was noted in maximal expiratory flows at 60 per cent of total lung capacity, specific airway conductance, thoracic gas volume, and residual volume. Inhalation of atropine also significantly improved maximal expiratory flow at 25 per cent of vital capacity. Isoproterenol increased the mean flows by 8 to 46 per cent above baseline, and decreased thoracic gas volume and residual volume by 11 to 16 per cent with the maximal response at 15 or 30 min. Atropine increased mean flows by 15 to 77 per cent and decreased thoracic gas volume and residual volume by 16 to 25 per cent with the maximal effect at 30 to 60 min. The mean response to atropine, expressed as a percentage of baseline, was always greater than or equal to that for isoproterenol. We conclude that vagal tone, which may reflect increased irritant receptor activity, contributes to the obstructive pulmonary function in cystic fibrosis. Inhalation of atropine sulfate will decrease the pulmonary function abnormality, but side effects prohibit its clinical usefulness. RF 001 WEST JR PEDIATRICS 13 155 954 002 COOK CD PEDIATRICS 24 181 959 003 BEIER FR AM REV RESPIR DIS 94 430 966 004 GANDEVIA B ARCH DIS CHILD 34 511 959 005 POLGAR G AM J DIS CHILD 100 733 960 006 MELLINS RB PEDIATRICS 41 560 968 007 MEARNS MB ARCH DIS CHILD 43 528 968 008 LIFSCHITZ MI AM REV RESPIR DIS 99 399 969 009 PHELAN PD ARCH DIS CHILD 44 393 969 010 FEATHERBY EA CAN MED ASSOC J 102 835 970 011 ZAPLETAL A PEDIATRICS 48 64 971 012 CHANG N AM REV RESPIR DIS 106 867 972 013 LANDAU LI J PEDIATR 82 863 973 014 SHAPIRO GG PEDIATRICS 58 740 976 015 DAY G ARCH DIS CHILD 48 355 973 016 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 017 EMPEY DW AM REV RESPIR DIS 113 131 976 018 KLOCK LE AM REV RESPIR DIS 112 371 975 019 CROPP GJA AM REV RESPIR DIS 112 599 975 020 TAUSSIG LM J PEDIATR 82 380 973 021 BARRON R AM REV RESPIR DIS SUPPL 115 275 977 022 CAVANAUGH MJ AM REV RESPIR DIS 114 517 976 023 TINKELMAN D ANN ALLERGY 38 298 977 024 MERCER TT ANN ALLERGY 23 314 965 025 MEAD J J APPL PHYSIOL 15 736 960 026 DUBOIS AB J CLIN INVEST 35 322 956 027 DUBOIS AB J CLIN INVEST 35 322 956 028 POLGAR G PULMONARY FUNCTION TESTING IN 971 029 WENG TR AM REV RESPIR DIS 99 879 969 030 DEMUTH GR PEDIATRICS 35 200 965 031 MATTHYS H RESPIRATION 27 447 970 032 ZAPLETAL A J APPL PHYSIOL 26 308 969 033 STEEL RGD PRIN PROC OF STATISTICS 960 034 HAHN GJ BIOMETRIKA 58 323 971 035 LANDAU LI AM REV RESPIR DIS 108 593 973 036 MANSELL A AM REV RESPIR DIS 109 190 974 037 COOPER DM AM REV RESPIR DIS 109 519 974 038 INGRAM RH J CLIN INVEST 59 696 977 039 ALLAN JD CLIN ALLERGY 5 255 975 040 WARNER JO ARCH DIS CHILD 51 507 976 041 MELLIS CM PEDIATRICS 61 446 978 042 WANNER A AM REV RESPIR DIS 116 73 977 CT 1 BOHADANA AB CLIN RESP PHYSIOL 16 769 980 2 KATTAN M THORAX 35 531 980 3 BERGMAN KR CHEST 78 891 980 4 BOUSHEY HA AM REV RESPIR DIS 121 389 980 5 LARSEN GL AM J DIS CHILD 134 1143 980 6 KRAEMER R PEDIATR RES 15 1433 981 7 KRADJAN WA AM REV RESPIR DIS 123 471 981 8 VANASPEREN P AM J DIS CHILD 135 815 981 9 GRIFFIN MP J APPL PHYSIOL 53 1576 982 10 PAK CCF AM REV RESPIR DIS 125 331 982 11 DAVIS PB J CHRON DIS 36 269 983 12 FRANCIS PB MED CLIN NORTH AM 67 657 983 13 DAVIS PB CHEST 85 802 984 14 HEIDEPRIEM CC DRUG INTEL CLIN PHARM 19 732 985 15 SCHONI MH PEDIATR RES 19 47 985 16 KONIG P ANN ALLERGY 55 95 985 17 HORDVIK NL AM REV RESPIR DIS 131 889 985 18 DARGA LL PEDIATR PULMONOL 2 82 986 19 KNOWLES MR CLIN CHEST MED 7 285 986 20 BROWN SE CHEST 89 7 986 21 BOUCHER RC TOXICOL APPL PHARMACOL 87 264 987 PN 79024 RN 01004 AN 79185775 AU Reid-L-M. TI The pulmonary circulation: remodeling in growth and disease. The 1978 J. Burns Amberson lecture. SO Am-Rev-Respir-Dis. 1979 Apr. 119(4). P 531-46. MJ PULMONARY-ARTERY: pa. MN ADULT. ANIMAL. ANOXIA: pa. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pa. HEART-DEFECTS-CONGENITAL: pa. HEART-SEPTAL-DEFECTS-VENTRICULAR: pa. HEART-VENTRICLE: pa. HUMAN. HYPERTENSION-PULMONARY: et, pa. INFANT. INFANT-NEWBORN. LUNG: bs, em, gd. PULMONARY-ARTERY: ah, gd. PULMONARY-HEART-DISEASE: pa. RATS. SWINE. EX This account draws on studies of young and mature lung, on normal and morbid anatomic features, on normal adaptations as in growth, and remodeling by disease. Qualitative analysis of the vascular bed, development of the pulmonary circulation, right ventricular hypertrophy or cor pulmonale, remodeling in disease, and experimental studies are discussed. Pulmonary hypertension offers us a complex pattern of structural and functional interaction; we are just beginning to realize how complex it is. Information on normal structure, its modification and remodeling by growth and disease, and appropriate techniques are available that will enable us to formulate our questions with due regard for anatomic precision and functional implication. And so research will lead to understanding and, it can be expected, to control of the undesirable aspects of pulmonary hypertension. RF 001 ELLIOTT FM THESIS 964 002 ONEAL RM ARCH PATHOL 60 267 955 003 WEIBEL ER J APPL PHYSIOL 17 343 962 004 DUNNILL MS THORAX 17 329 962 005 HAYWARD J THORAX 7 89 952 006 BUCHER U THORAX 16 207 961 007 BRIGHT R GUYS HOSP REP 1 338 836 008 SHORT DS J FACULTY RADIOL 8 118 956 009 HISLOP A THORAX 25 682 970 010 ELLIOTT FJ CLIN RADIOL 16 193 965 011$ REID L IN: SCIENTIFIC BASIS OF M 289 968 012 HISLOP A J ANAT 113 35 972 013 HISLOP A THORAX 28 313 973 014 HISLOP A THORAX 28 129 973 015 HISLOP A IN: DAVIS JA 214 974 016 REID L AM J ROENTGENOL 129 777 977 017 HISLOP A IN: HODSON WA 6 37 977 018 BOYDEN EA AM J ANAT 121 749 967 019 DAVIES G THORAX 25 669 970 020 ANON WHO TECH REP SER NO 213 961 021 FULTON RM BR HEART J 14 413 952 022 HISLOP A J CLIN PATHOL 25 534 972 023 WEEKS JR PROC SOC EXP BIOL MED 104 646 960 024 HERGET J ARCH INT PHARMACODYN THER 198 107 972 025 RABINOVITCH M CIRCULATION SUPPL 2 56 275 977 026 RENDAS A J APPL PHYSIOL 45 806 978 027 DAVIES GM IN: LAWSON D PROC 5TH INT CF 350 969 028 RYLAND D THORAX 30 285 975 029 ANDERSON EG J PATHOL 110 273 973 030 MEYRICK B BR J DIS CHEST 68 11 974 031 HISLOP A BR HEART J 35 1178 973 032 HISLOP A BR HEART J 37 1014 975 033 HAWORTH SG AM J CARDIOL 40 781 977 034 HAWORTH SG THORAX 32 129 977 035 HAWORTH SG THORAX 32 121 977 036 HAWORTH SG BR HEART J 39 80 977 037 GROVER RF AM HEART J 66 1 963 038 ANDERSON G CLIN RADIOL 24 113 973 039 HAWORTH SG BR HEART J 40 826 978 040 RABINOVITCH M CIRCULATION SUPPL 2 56 193 977 041 RABINOVITCH M CIRCULATION 58 1107 978 042 HEATH D CIRCULATION 18 533 958 043 MEYRICK B COLLOQ INSERM ECHANG GAZ PUL 51 145 975 044 HISLOP A BR J EXP PATHOL 57 542 976 045 MEYRICK B ANAT REC 193 71 979 046 MEYRICK B J ANAT 125 209 978 047 MEYRICK B LAB INVEST 38 188 978 048 HISLOP A BR J EXP PATHOL 58 653 977 050 HISLOP A BR J EXP PATHOL 55 153 974 051 MEYRICK B AM J PATHOL 94 37 979 052 RENDAS A J THORAC CARDIOVASC SURG 77 109 979 053 CHEUNG DLC THESIS 977 054 UNGER M J APPL PHYSIOL 43 662 977 CT 1 REID L J PEDIATR 95 836 979 2 RENDAS A AM REV RESPIR DIS 121 873 980 3 SCHNEEBERGER EE CIRC RES 49 1102 981 4 JANNEY CG AM J CLIN PATHOL 76 722 981 5 INSELMAN LS J PEDIATR 98 1 981 6 GEWITZ MH PEDIATR PHARMACOL 2 57 982 7 DAVIES P HUM PATHOL 13 911 982 8 BERGOFSKY EH BULL EUR PHYSIOPATH RESP 18 63 982 9 DAVIES P J APPL PHYSIOL 53 859 982 10 VANDEVENNE A SEM HOP PARIS 58 397 982 11 REID L ANN NY ACAD SCI 384 3 982 12 MEYRICK B CLIN CHEST MED 4 199 983 13 SOIFER SJ J DEV PHYSIOL 5 237 983 14 GAULIN P CAN J SURG 26 250 983 15 ZHU YJ J APPL PHYSIOL 54 680 983 16 PITT BR J APPL PHYSIOL 55 383 983 17 SOBIN SS J APPL PHYSIOL 55 1445 983 18 WILCKEN B J PEDIATR 102 383 983 19 GAULTIER C AM REV RESPIR DIS 127 527 983 20 HALES CA AM REV RESPIR DIS 128 747 983 21 KAY JM AM REV RESPIR DIS 128 S 53 983 22 LISTER G PEDIATR PHARMACOL 4 85 984 23 SCHALL SA INT J CARDIOL 5 103 984 24 HEYMANN MA J DEV PHYSIOL 6 281 984 25 LUCAS CL CRC CRIT REV BIOMED ENG 10 317 984 26 LYRENE RK CLIN PERINATOL 11 551 984 27 SOIFER SJ CLIN PERINATOL 11 745 984 28 BRIGHAM KL CIRC RES 54 623 984 29 PITT BR J APPL PHYSIOL 57 1158 984 30 CASSIO A ACTA PAEDIATR SCAND 73 554 984 31 REID LM BR J DIS CHEST 78 113 984 32 PERKIN RM J PEDIATR 105 511 984 33 HALES CA SEM RESPIR MED 7 136 985 34 KINSELLA JP PEDIATR RADIOL 15 340 985 35 GAULTIER C BULL EUR PHYSIOPATH RESP 21 55 985 36 BURROWS FA CAN ANAESTH SOC J 32 364 985 37 MEYRICK B J APPL PHYSIOL 59 443 985 38 ABMAN SH PEDIATRICS 75 80 985 39 COTTON DJ CHEST 87 217 985 40 MICHAEL JR LUNG 163 65 985 41 HUREWITZ AN HEART LUNG 15 327 986 42 THOMPSON BT HEART LUNG 15 457 986 43 BENITZ WE PEDIATR RES 20 966 986 44 HEYMANN MA DRUGS 32 164 986 45 CUSTER JR J APPL PHYSIOL 60 32 986 46 OU LC RESPIR PHYSIOL 64 81 986 47 REID LM CHEST 89 279 986 PN 79025 RN 01005 AN 80063276 AU Nelson-L-A. Callerame-M-L. Schwartz-R-H. TI Aspergillosis and atopy in cystic fibrosis. SO Am-Rev-Respir-Dis. 1979 Oct. 120(4). P 863-73. MJ ASPERGILLOSIS-ALLERGIC-BRONCHOPULMONARY: et. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. ASPERGILLOSIS-ALLERGIC-BRONCHOPULMONARY: di, im. ASPERGILLUS: ip. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: im, mi. EOSINOPHILS. FOLLOW-UP-STUDIES. HUMAN. IGE: an. PRECIPITINS: an. SKIN-TESTS. SPUTUM: cy, mi. SUPPORT-U-S-GOVT-P-H-S. AB Forty-six patients with cystic fibrosis (CF) were studied for colonization and sensitization by Aspergillus organisms. The fungus was cultured from 21 of 37 (57%) patients who produced sputum. The non-CF sputum isolation rate was 6.5%. Sputum hyphae were seen in 95% of patients with positive cultures. Sputum eosinophilia accompanied colonization by Aspergillus. Aspergillus precipitins were found in 37% of CF patients; positive immediate skin tests were found in 39%. "Atopy" defined by skin test criteria was found in 46%. All of these findings were more common in patients with more severe disease (p less than 0.05). CF patients had predominant mold sensitivity in contrast to predominant pollen sensitivity in a control group of asthmatic subjects. Serum IgE concentrations were increased in 22% of CF patients. CF patients who had increased serum concentrations of IgE were more likely to have a family history of atopy. Wheezing was more common in those who were atopic, especially the group with predominant mold sensitivity who had more severe disease. Four patients fulfilled the diagnostic criteria for allergic bronchopulmonary aspergillosis (ABPA) during the time of study and follow-up. A fifth patient had ABPA diagnosed before the study. The incidence of ABPA during a 2-yr period in our CF population was 11%. RF 001 MEARNS MB LANCET 1 538 967 002 SCHWARTZ RH AM J DIS CHILD 120 432 970 003 WARREN CPW CLIN ALLERGY 5 1 975 004 BARDANA EJ JR AM J DIS CHILD 129 1164 975 005 GALANT SP AM REV RESPIR DIS 114 325 976 006 WELLS ID J ALLERGY CLIN IMMUNOL 57 234 976 007 YOUNG RC MEDICINE (BALTIMORE) 49 147 970 008 MEARNS MB THORAX 20 385 965 009 BATTEN JC MOD PROBL PEDIATR 10 227 967 010 ORES CN CF CLUB ABST 16 48 975 011 TAUSSIG LM J PEDIATR 82 380 973 012 SLAVIN RG J ALLERGY 46 150 970 013 SAFIRSTEIN BH AM REV RESPIR DIS 108 450 973 014 ROSENBERG M ANN INTERN MED 86 405 977 015 SLAVIN RG IN: MIDDLETON E 843 978 016 ESTERLY JR JOHNS HOPKINS MED J 122 94 968 017 NEMIR RL IN: KENDIG EL JR 446 977 018 FRASER RG DIAGNOSIS OF DISEASES OF CHES 970 019 COMSTOCK GW MYCOPATHOL MYCOL APPL 54 55 974 020 AUSTWICK PKC IN: LENNETTE EH 974 021 SCHNEIERSON SS ATLAS OF DIAGNOSTIC MICROBIOL 974 022 KJELLMAN NIM THESIS 976 023 BLYTH W SABOURAUDIA 9 273 971 024 BLYTH W SABOURAUDIA 9 283 971 025 SLAVIN RG J ALLERGY CLIN IMMUNOL 62 7 978 026 HENDERSON AH THORAX 23 501 968 027 IRANI FA AM REV RESPIR DIS 103 552 971 028 ROSENBERG M J PEDIATR 91 194 977 029 RACHELEFSKY GS AM J DIS CHILD 128 355 974 030 ALLAN JD CLIN ALLERGY 5 255 975 031 BARRON R CF CLUB ABST 23 977 032 BUCKLEY RH N ENGL J MED 281 465 969 033 TOWNSEND RW IN: FEINGOLD BF 973 034 MORROW MB ANN ALLERGY 22 575 964 035 CAMPBELL MJ AM REV RESPIR DIS 89 186 964 036 SCHWARTZ HJ J ALLERGY CLIN IMMUNOL 62 9 978 037 PEPYS J CLIN ALLERGY SUPPL 3 491 973 038 NOVEY HS WEST J MED 180 1 979 039 MELLIS CM PEDIATRICS 61 446 978 040 SMITH JM IN: MIDDLETON E 633 978 041 PATTERSON R INT ARCH ALLERGY 46 150 974 042 MARX JJ J ALLERGY CLIN IMMUNOL 57 328 976 043 GEHA RS J ALLERGY CLIN IMMUNOL 60 357 977 044 WARNER JO ARCH DIS CHILD 51 507 976 045 LEWISTON NJ CF CLUB ABST 47 973 046 IMBEAU SA J ALLERGY CLIN IMMUNOL 52 91 978 047 SHADOMY S IN: LENNETTE EH 569 974 CT 1 SLY PD AUST PAEDIATR J 16 205 980 2 HODSON ME THORAX 35 801 980 3 LARSEN GL AM J DIS CHILD 134 1143 980 4 SCHWARTZ RH J ALLERGY CLIN IMMUNOL 68 290 981 5 CLARKE CW BR J DIS CHEST 75 15 981 6 GLIMP RA CHEST 80 85 981 7 LEWISTON NJ CHEST 80 389 981 8 MOSS RB J PEDIATR 99 215 981 9 HENRY RL AUST PAEDIATR J 18 110 982 10 PITCHERWILMOTT RW ARCH DIS CHILD 57 582 982 11 VOSS MJ J ALLERGY CLIN IMMUNOL 69 536 982 12 MANTHEI U AM REV RESPIR DIS 126 253 982 13 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 14 KATZ SM JAMA 248 2284 982 15 GUIDOTTI TL AM J MED SCI 283 157 982 16 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 17 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 18 SCHOLER HJ MYKOSEN 26 173 983 19 LAUFER P CUTIS 31 665 983 20 BACULARD A ARCH FR PEDIATR 40 109 983 21 EFTHIMIOU J THORAX 39 150 984 22 SMITH MJ THORAX 39 369 984 23 BIRX DL ANN ALLERGY 53 124 984 24 LAUFER P J ALLERGY CLIN IMMUNOL 73 44 984 25 ROHATGI PK SOUTH MED J 77 1291 984 26 SLAVIN RG CLIN REV ALLERGY 3 167 985 27 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 28 LESTER LA SEM RESPIR MED 6 285 985 29 LAUFER P J ASTHMA 22 253 985 30 LAUFER P CUTIS 35 557 985 31 LAUFER P CUTIS 36 245 985 32 DUPONT B ARCH FR PEDIATR 42 943 985 33 BERKIN KE EUR J RESPIR DIS 67 103 985 34 ISPIZUA AU MED CLIN 85 628 985 35 FINK JN CHEST 87 S 81 985 36 SCHONHEYDER H ACTA PATH MICROB IMMU SCA (B) 93 105 985 37 WILMOTT RW AM J DIS CHILD 139 669 985 38 GORDON IJ J ROY COLL PHYSICIANS LOND 20 206 986 39 PATY E ARCH FR PEDIATR 43 243 986 40 SLAVIN RG ARCH INTERN MED 146 1799 986 PN 79026 RN 01006 AN 79163856 AU Feldman-J. Traver-G-A. Taussig-L-M. TI Maximal expiratory flows after postural drainage. SO Am-Rev-Respir-Dis. 1979 Feb. 119(2). P 239-45. MJ BRONCHITIS: th. CYSTIC-FIBROSIS: th. RESPIRATION. MN ADOLESCENCE. ADULT. AGED. BRONCHITIS: pp. CHILD. CYSTIC-FIBROSIS: pp. FEMALE. FORCED-EXPIRATORY-VOLUME. HUMAN. MALE. MAXIMAL-EXPIRATORY-FLOW-VOLUME-CURVES. MIDDLE-AGE. PEAK-EXPIRATORY-FLOW-RATE. PHYSICAL-THERAPY. POSTURE. TOTAL-LUNG-CAPACITY. SUPPORT-U-S-GOVT-P-H-S. VITAL-CAPACITY. AB Flows measured from maximal expiratory flow-volume (MEFV) curves were used to evaluate the efficacy of postural drainage in improving ventilatory function acutely. Maximal expiratory flow-volume curves were obtained for 9 cystic fibrosis subjects and 10 subjects with chronic bronchitis before and 5, 15, and 45 min after a 30-min session of postural drainage with percussion, vibration, and coughing. Forced vital capacity (FVC) was significantly increased 45 min after drainage for the combining group. Flows at high lung volumes were different for the 2 subgroups. Subjects with cystic fibrosis demonstrated a significant increase in peak expiratory flow rates 45 min after drainage and an increase in forced expiratory volume in one sec at all time intervals. The subjects with chronic bronchitis had a decreased peak expiratory flow rate 5 min after drainage, but by 45 min, it had returned to baseline. There was no significant change in one-sec forced expiratory volume at any time interval for the chronic bronchitis subgroup. Changes in flows at low lung volumes were similar for the 2 subgroups. Forty-five min after drainage there was an increase in flow rates near 50 per cent of FVC. Flows near 25 per cent of FVC were increased 15 and 45 min after drainage. This study demonstrated that postural drainage with coughing resulted in significant improvement in flows at low lung volumes. Changes in flows at high lung volumes were less consistent. RF 001 THACKER EW POSTURAL DRAINAGE AND RESPIRA 971 002 PETTY TL INTENSIVE AND REHABILITATIVE 974 003 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 973 004 JONES NL AM REV RESPIR DIS 110 132 974 005 HUBER AL J ALLERGY CLIN IMMUNOL 53 109 974 006 KANG B J ALLERGY CLIN IMMUNOL 53 109 974 007 MARCH H ARCH PHYS MED REHABIL 52 528 971 008 PETERSEN SE ACTA MED SCAND 182 293 967 009 TECKLIN JS PHYS THER 55 1081 975 010 MOTOYAMA EK IN: MANGOS JA 335 973 011 HYATT RE AM REV RESPIR DIS 107 191 973 012 LANDAU LI AM REV RESPIR DIS 108 593 973 013 GELB AF N ENGL J MED 288 395 973 014 TAUSSIG LM J PEDIATR 82 380 973 015 WALKER HM STATISTICAL INFERENCE 953 016 DUNN OJ APPLIED STATISTICS ANALYSIS O 974 017 POLGAR G PULMONARY FUNCTION TESTING IN 971 018 BATES DV RESPIRATORY FUNCTION IN DISEA 971 019 ANTHONISEN P ACTA MED SCAND 175 715 964 020 MEAD J J APPL PHYSIOL 22 95 967 021 MEAD J J APPL PHYSIOL 44 156 978 CT 1 NEWTH CJL PEDIATR CLIN NORTH AM 26 617 979 2 CONNORS AF CHEST 78 559 980 3 DARROW G AM REV RESPIR DIS 122 155 980 4 ROCHESTER DF AM REV RESPIR DIS 122 133 980 5 PASSERO MA CLIN PEDIATR 20 264 981 6 HUDSON LD MED CLIN NORTH AM 65 629 981 7 PETTY TL SEM RESPIR MED 3 263 982 8 KERREBIJN KF EUR J RESPIR DIS 63 35 982 9 MALONEY FP ARCH PHYS MED REHABIL 63 423 982 10 SUTTON PP EUR J RESPIR DIS 63 188 982 11 REDDING GJ AM REV RESPIR DIS 126 31 982 12 SUTTON PP EUR J RESPIR DIS 64 62 983 13 ZAPLETAL A EUR J RESPIR DIS 64 426 983 14 DESMOND KJ J PEDIATR 103 538 983 15 DEBOECK C AM REV RESPIR DIS 129 182 984 16 WOLLMER P EUR J RESPIR DIS 66 233 985 17 KIRILOFF LH CHEST 88 436 985 18 FALING LJ CLIN CHEST MED 7 599 986 19 VANDERSCHANS CP THORAX 41 448 986 20 VERBOON JML EUR J RESPIR DIS 69 169 986 21 WEBBER BA BR J DIS CHEST 80 353 986 PN 79027 RN 01007 AN 79207490 AU Zambie-M-F. Gupta-S. Lemen-R-J. Hilman-B. Waring-W-W. Sly-R-M. TI Relationship between response to exercise and allergy in patients with cystic fibrosis. SO Ann-Allergy. 1979 May. 42(5). P 290-4. MJ CYSTIC-FIBROSIS: co. EXERTION. HYPERSENSITIVITY: co. MN ADOLESCENCE. ADULT. BODY-HEIGHT. CHILD. FEMALE. FORCED-EXPIRATORY-VOLUME. HUMAN. MALE. PEAK-EXPIRATORY-FLOW-RATE. VITAL-CAPACITY. AB Responses to treadmill exercise of 36 patients with cystic fibrosis were studied to evaluate their usefulness in identifying those with atopy. Ten of 36 patients with cystic fibrosis (28%) had an abnormal response to exercise but had no more frequent atopic characteristics than those who had normal exercise responses. Response to treadmill exercise alone cannot identify patients with atopic disease among those with cystic fibrosis. RF 001 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 002 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 003 KULCZYCKI LL JAMA 175 358 961 004 RACHELEFSKY GS AM J DIS CHILD 128 355 974 005 WARNER JO ARCH DIS CHILD 51 507 976 006 WARNER JO LANCET 1 990 976 007 FEATHERBY EA CAN MED ASSOC J 102 835 970 008 WARREN CPW CLIN ALLERGY 5 1 975 009 MCFARLANE H CLIN ALLERGY 7 279 977 010 SPITZ E J ALLERGY CLIN IMMUNOL 49 337 972 011 GODFREY S EXERCISE TESTING IN CHILDREN 974 012 CROPP GJA PEDIATR CLIN NORTH AM 22 63 975 013 SLY RM IN: WEISS EB 976 014 BIERMAN CW PEDIATRICS 56 847 975 015 HANSEL FK CLIN ALLERGY 953 016 KJELLMAN NIM CLIN ALLERGY 6 51 976 017 DERBES VJ J ALLERGY 28 287 957 018 MELLIS CM PEDIATRICS 61 446 978 019 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 020 HEIMLICH EM J ALLERGY CLIN IMMUNOL 37 103 966 021 DAY G ARCH DIS CHILD 48 355 973 022 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 023 GIMENO F THORAX 29 16 974 024 BARBEE RA ANN INTERN MED 84 129 976 025 SLY RM ANN ALLERGY 28 1 970 026 CROPP GJA PEDIATRICS SUPPL 56 868 975 027 KATTAN M J PEDIATR 92 718 978 CT 1 SLY PD AUST PAEDIATR J 16 205 980 PN 79028 RN 01008 AN 80086311 AU Stagg-B-H. Wood-T-P. TI Radioimmunoassay of trypsin. A new aid in the assessment of pancreatic function. SO Ann-Clin-Biochem. 1979 May. 16(3). P 147-51. MJ PANCREAS: en. PANCREATIC-DISEASES: di. TRYPSIN: an. MN CYSTIC-FIBROSIS: di. DIABETES-MELLITUS-INSULIN-DEPENDENT: di. HUMAN. PANCREATIC-DISEASES: en. PANCREATIC-NEOPLASMS: di. PANCREATITIS: di. RADIOIMMUNOASSAY: mt. AB This review describes the development and application of a novel test to determine levels of human immunoreactive trypsin, an enzyme produced solely by the pancreas, in biological fluids. Being organ- specific, the assay of immunoreactive trypsin should be an ideal marker of pancreatic function, and this is supported by the results of a number of clinical and research investigations. Use of this assay in studies of chronic pancreatitis, juvenile-onset diabetes, and cystic fibrosis has yielded much valuable data, and it is expected that further research will lead to an improved understanding of these and other conditions associated with the pancreas in health and disease. RF 001 ADRIAN TE GUT 19 A960 978 002 ADRIAN TE CLIN SCI MOL MED 54 13P 978 004 BAMBULE-DICK J IR J MED SCI SUPPL 1 146 3 978 005 BRAY PT CLIN CHIM ACTA 80 333 977 006 CROSSLEY JR LANCET 2 1093 977 007 CROSSLEY JR LANCET 1 472 979 008 DANDONA P BR MED J 2 1125 978 009 DOMSCHKE S AM J DIG DIS 20 309 975 010 ELIAS E LANCET 2 66 977 011 FEDAIL SS GUT 19 A445 978 012 FRIER BM GUT 17 685 976 013 GOWENLOCK AH ANN CLIN BIOCHEM 14 61 977 014 GREENE LJ IN: FRITZ H 188 974 016 KOOP H PROC EUROP PANCREAS 11TH SYM 978 017 LAKE-BAKAAR G GUT 19 A446 978 018 LAKE-BAKAAR G GUT "CLEARANCE OF" 19 A960 978 019 LAKE-BAKAAR G GUT 19 A445 978 020 LAKE-BAKAAR G GUT "URINE TRYPSIN" 19 A960 978 021 LIEBOW C SCIENCE 189 472 975 023 PROSSER R ARCH DIS CHILD 49 597 974 024 SMALLEY CA LANCET 2 415 978 025 TEMLER RS BIOCHIM BIOPHYS ACTA 236 72 971 026 TEMLER RS BIOCHIM BIOPHYS ACTA 445 720 976 027 TEMLER RS ENZYME 22 249 977 028 WOOD TP LANCET 1 781 979 CT 1 BURLINA A SCAND J GASTROENTEROL 15 35 980 2 FAHRENKRUG J CLIN CHEM 26 1573 980 3 ODONNELL MD CLIN CHIM ACTA 104 265 980 4 BUTTERWORTH J ANAL BIOCHEM 106 156 980 5 FIEDLER F HOPPE SEYLERS Z PHYSIOL CHEM 361 1661 980 6 FAHRENKRUG J CLIN CHEM 27 1655 981 7 KOEHN HD CLIN CHEM 27 502 981 8 OCONNOR CM CLIN CHIM ACTA 114 29 981 9 LAWRENCE CH BIOCHIM BIOPHYS ACTA 657 13 981 10 BECKER H LANGENBECKS ARCH CHIR S 47 983 11 HAFKENSCHEID JCM J CLIN CHEM CLIN BIOCHEM 21 167 983 12 BOHNER J J CLIN CHEM CLIN BIOCHEM 22 943 984 13 LEUNG FY CLIN CHEM 30 1361 984 14 ASH KO J MED TECHNOL 2 427 985 15 LESI C CLIN BIOCHEM 18 317 985 16 LESI C DIG DIS SCI 30 552 985 PN 79029 RN 01009 AN 79208273 AU Swersky-R-B. Chang-J-B. Wisoff-B-G. Gorvoy-J. TI Endobronchial balloon tamponade of hemoptysis in patients with cystic fibrosis. SO Ann-Thorac-Surg. 1979 Mar. 27(3). P 262-4. MJ CATHETERIZATION: mt. CYSTIC-FIBROSIS: co. HEMOPTYSIS: th. HEMOSTATIC-TECHNICS. TAMPONS. MN ADOLESCENCE. ADULT. BRONCHOSCOPY: is. CATHETERIZATION: is. CYSTIC-FIBROSIS: ra. FEMALE. FIBER-OPTICS. HEMOPTYSIS: et, ra. HEMOSTATIC-TECHNICS: is. HUMAN. MALE. METHODS. RECURRENCE. AB The flexible bronchoscope with Fogarty balloon and irrigating catheters is used effectively to manage and treat hemoptysis in a series of patients with cystic fibrosis. This procedure is well suited to such patients, who are prone to recurrent hemoptysis, and may also be indicated in any inoperable patient with poor cardiopulmonary reserve. RF 001 CROCCO JA ARCH INTERN MED 121 495 968 002 FELLOWS KE RADIOLOGY 114 551 975 003 FOGARTY T SURG GYNECOL OBSTET 116 241 963 004 GOTTLIEB LS CHEST 67 482 975 005 GOURIN A CHEST 68 120 975 006 HIEBERT CA CHEST 66 397 974 007 HOLSCLAW DS J PEDIATR 76 829 970 008 MEARNS MB ARCH DIS CHILD 47 499 972 009 ORES CN AM REV RESPIR DIS 99 790 969 010 REMY J RADIOLOGY 122 33 977 011 SAW EC CHEST 70 589 976 012 SCHUSTER SR J THORAC CARDIOVASC SURG 48 750 964 013 SMIDDY JF CHEST 64 158 973 014 WHOLEY MH JAMA 236 2501 976 CT 1 DAROSA JFT ANN THORAC SURG 30 409 980 2 FERMON C NY STATE J MED 81 1223 981 3 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 4 MARMON L J PEDIATR SURG 18 811 983 5 PORTER DK ARCH INTERN MED 143 287 983 6 HOWARD WJ POSTGRAD MED 77 53 985 PN 79030 RN 01010 AN 79230232 AU Maybury-B-A. Blessing-Moore-J. deWit-S-A. Lewiston-N-J. Yeager-A-S. TI Antimicrobial susceptibilities of rough, smooth, and mucoid colony types of Pseudomonas isolated from cystic fibrosis patients. SO Antimicrob-Agents-Chemother. 1979 Mar. 15(3). P 494-6. MJ ANTIBIOTICS: pd. CYSTIC-FIBROSIS: mi. PSEUDOMONAS: de. MN HUMAN. PSEUDOMONAS: cy. SPUTUM: mi. AB Minimal inhibitory concentrations of carbenicillin, chloramphenicol, tetracycline, gentamicin, tobramycin, and trimethoprim- sulfamethoxazole were determined for rough, smooth, and mucoid Pseudomonas sp. isolates from sputa collected from 40 cystic fibrosis patients. Ninety-four percent of the minimal inhibitory concentrations obtained by using mixed inocula of colonies of different morphological types fell within one serial dilution of the most resistant minimal inhibitory concentration obtained when each colony type was tested separately. RF 001 BARRY AL ANTIMICROBIC SUSCEPTIBILI 976 002 BARRY AL ANTIMICROB AGENTS CHEMOTHER 13 61 978 003 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 004 DOGGETT RG INFECT IMMUN 6 628 972 005 GAVAN TL IN: BALOWS A 88 974 006 GAVAN TL AM J CLIN PATHOL 53 880 970 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 008 HOIBY N SCAND J RESPIR DIS 58 65 977 009 WASHINGTON JA II IN: LENNETTE EH 410 974 010 ZIERDT CH J BACTERIOL 87 1003 964 CT 1 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 PN 79031 RN 01011 AN 80264180 AU Greven-Brauns-G. Mulkens-E. TI Results of 10 years of screening of newborn infants for inherited metabolic diseases in the province of Limburg (Belgium). SO Arch-Belg-Med-Soc. 1979 Oct. 37(8). P 516-9. MJ METABOLISM-INBORN-ERRORS: oc. MN AMINO-ACID-METABOLISM-INBORN-ERRORS: oc. BELGIUM. CYSTIC-FIBROSIS: oc. GALACTOSEMIA: oc. HUMAN. INFANT-NEWBORN. PHENYLKETONURIA: oc. AB In the province of Limburg, all newborn babies are screened for metabolic anomalies: for amino acidopathies since 1968, for galactosemia since 1974 and for mucoviscidosis since 1978. On 127,676 newborns, there were 12 cases of phenylketonuria and 3 other inherited anomalies. A meaningful policy of the government on neonatal screening necessarily requires a good organization, to put together the screening data, diagnosis, treatment and follow-up. RF 001 BEUTLER E J LAB CLIN MED 68 137 966 002 CLOW C AM J DIS CHILD 117 48 969 003 EFRON ML N ENGL J MED 270 1378 964 004 GREVEN-BRAUNS G BELG ARCH SOC GENEESK HYG ARB 28 538 970 005 GREVEN-BRAUNS G BELG ARCH SOC GENEESK HYG ARB 33 253 975 006 GUTHRIE R PEDIATRICS 32 338 963 007 LEVY HL CLIN BIOCHEM 1 200 968 008 SCRIVER CR LANCET 2 230 964 PN 79032 RN 01012 AN 80018835 AU Maxwell-M. Redmond-A. TI Comparative trial of manual and mechanical percussion technique with gravity-assisted bronchial drainage in patients with cystic fibrosis. SO Arch-Dis-Child. 1979 Jul. 54(7). P 542-4. MJ CYSTIC-FIBROSIS: th. DRAINAGE. PHYSICAL-THERAPY: mt. MN ADOLESCENCE. ADULT. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: pp. FORCED-EXPIRATORY-VOLUME. GRAVITATION. HUMAN. PHYSICAL-THERAPY: is. RANDOM-ALLOCATION. SPUTUM. VITAL-CAPACITY. CLINICAL-TRIALS. AB Chest physiotherapy still remains one of the most important aspects in the treatment of chest complications of cystic fibrosis. A mechanical device that allows the patient with cystic fibrosis to do his own chest physiotherapy will be of great benefit if it is as effective as manual percussion. 14 patients with cystic fibrosis using mechanical and manual percussion physiotherapy were studied by measuring sputum volumes, and FEV and FVC. Results with mechanical percussor were as good as with the manual percussor and, therefore, it would be reasonable for the older patient to use the former on his own. RF 001 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 002 DENTON R AM REV RESPIR DIS 86 41 962 003 KENDIG EL JR IN: WARING W 114 977 CT 1 ANON LANCET 2 729 979 2 DARROW G AM REV RESPIR DIS 122 155 980 3 SUTTON PP EUR J RESPIR DIS 63 188 982 4 SUTTON PP SEM RESPIR MED 5 353 984 5 WANNER A AM REV RESPIR DIS 130 701 984 6 SUTTON PP EUR J RESPIR DIS 66 147 985 PN 79033 RN 01013 AN 79164522 TI Paediatric Research Society. Plymouth, 15 and 16 September 1978: Abstracts. SO Arch-Dis-Child. 1979 Feb. 54(2). P 160-5. MJ ASTHMA: th. CYSTIC-FIBROSIS: me. PEDIATRICS. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CONGRESSES. FEMALE. GREAT-BRITAIN. HUMAN. INFANT. INFANT-NEWBORN. MIDDLE-AGE. RESEARCH. SOCIETIES-MEDICAL. EX Abstracts are given for articles on abnormalities of the complement system in children with liver disease, hepatic collagen synthesis and its modification by colchicine is a rat model of cirrhosis of the liver, postprandal total serum bile acid concentrations in cystic fibrosis, release of endogenous secretin in children with cystic fibrosis, bronchial allergy in children with cystic fibrosis, and other topics. RF 001 MCNEISH AS LANCET 2 946 975 001 POPA VT J ALLERGY CLIN IMMUNOL 59 54 977 001 CHARLESWORTH JA CLIN EXP IMMUNOL 28 496 977 001 HOLMES H LANCET 1 755 977 001 MASHIGE F CLIN CHIM ACTA 70 79 976 002 OFEK I NATURE 265 623 977 002 SOOTHILL JF CLIN EXP IMMUNOL 27 30 977 003 WURTMAN RJ N ENGL J MED 296 1383 977 CT 1 MCKIERNAN J IR MED J 74 89 981 2 MCKIERNAN J IR J MED SCI 150 66 981 PN 79034 RN 01014 AN 79164528 AU Ryley-H-C. Neale-L-M. Brogan-T-D. Bray-P-T. TI Screening for cystic fibrosis in the newborn by meconium analysis. SO Arch-Dis-Child. 1979 Feb. 54(2). P 92-7. MJ CYSTIC-FIBROSIS: pc. MASS-SCREENING: mt. MECONIUM: an. MN ALBUMINS: an. ALPHA-1-ANTITRYPSIN: an. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: di. FALSE-NEGATIVE-REACTIONS. FECES: an. HUMAN. INFANT. INFANT-NEWBORN. PROTEASE-INHIBITORS: an. TIME-FACTORS. AB During a 4-year routine screening programme for cystic fibrosis (CF) 15 464 specimens were examined for raised meconium albumin levels by a test strip method and by electroimmunoassay. The incidence of false- positive results was about 5 per 1000 specimens in either test. This could be reduced by 90% by determining the ratio of albumin : alpha-1- trypsin inhibitor (a ratio below 2.0 being considered as a negative result), and it could be reduced to zero by determining the ratio in subsequent faecal specimens. Three of 12 meconium specimens from infants with proved CF gave false-negative results in all 3 tests. The other 9 specimens had greater than 100 mg albumin/g dry weight and albumin: alpha-1-trypsin inhibitor ratios of greater than 3.0; in subsequent faecal specimens the ratios were over 4.0. 176 meconium specimens from elsewhere in the UK were examined and these included 23 from infants who were subsequently proved to have CF. Six of these 23 CF specimens gave false-negative results, the other 17 being strongly positive. The origins of meconium serum protein suggest that infants with CF in whom meconium gives false-negative results have normal pancreatic functions at birth. The specificity of current meconium tests therefore cannot be improved as they depend on pancreatic dysfunction. RF 001 ANON J PEDIATR 88 711 976 002 GITLIN D AM J OBSTET GYNECOL 113 632 972 003 GRIFFITHS AD ARCH DIS CHILD 51 321 976 004 HUANG NN AM J DIS CHILD 120 289 970 005 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 006 MAKAREM A IN: HENRY RJ 1123 974 007 ORENSTEIN DM AM J DIS CHILD 131 973 977 008 PROSSER R ARCH DIS CHILD 49 597 974 009 RULE AH PEDIATRICS 45 847 970 010 RYLEY HC CLIN CHIM ACTA 64 117 975 011 RYLEY HC ARCH DIS CHILD 51 641 976 012 SCHUTT WH ARCH DIS CHILD 43 178 968 013 SCHUTTRINGER G CLIN CHIM ACTA 83 109 978 014 SHWACHMAN H PEDIATRICS 46 335 970 015 STEPHAN U PEDIATRICS 55 35 975 016 STERN RC J PEDIATR 89 406 976 017 WARWICK WJ MINN MED 52 1567 969 018 WISER WC PEDIATRICS 33 115 964 CT 1 TARNOKY AL J ROY SOC MED 73 73 980 2 LITTLEWOOD JM PRACTITIONER 224 305 980 3 MASTELLA G RIV ITAL PEDIATR 7 581 981 4 LAMBOTTE C J GENET HUM 29 85 981 5 EVANS RT J CLIN PATHOL 34 911 981 6 RYLEY HC J CLIN PATHOL 34 906 981 7 RYLEY HC J CLIN PATHOL 34 179 981 8 PEDERZINI F RIV ITAL PEDIATR 9 445 983 9 HEELEY AF CLIN CHEM 29 2011 983 10 CASSIO A ACTA PAEDIATR SCAND 73 554 984 11 ORENSTEIN DM SEM RESPIR MED 6 252 985 PN 79035 RN 01015 AN 80018840 AU Jenner-B-M. Landau-L-I. Phelan-P-D. TI Pulmonary candidiasis in cystic fibrosis. SO Arch-Dis-Child. 1979 Jul. 54(7). P 555-6. MJ CYSTIC-FIBROSIS: co. LUNG-DISEASES-FUNGAL: et. CANDIDIASIS: et. MN ASTHMA: co. CASE-REPORT. CHILD. FEMALE. HUMAN. AB A child with cystic fibrosis and asthma developed pulmonary candidiasis. Predisposing factors in this patient were prolonged antibiotic therapy, high-dose corticosteroids, and intravenous catherisation. A diagnosis was made by lung puncture and confirmed by rapid response to 5-fluorocytosine. RF 001 BANDT PD JAMA 220 1578 972 002 GOLDSTEIN E J INFECT DIS 125 190 972 003 HURLEY DL J INFECT DIS 132 393 975 004 KLEIN JO PEDIATRICS 44 486 969 005 KOHLSCHUTTER A ARCH DIS CHILD 49 154 974 006 MIRSKY HS CANCER 30 348 972 007 MONTGOMERIE JZ J INFECT DIS 132 82 975 008 WINNER HI PROC SYMP ON CANDIDA INFECTIO 87 966 CT 1 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 2 LESTER LA SEM RESPIR MED 6 285 985 3 MALFROOT A PEDIATR INFECT DIS 5 376 986 4 RUBIO TT AM J MED 81 73 986 PN 79036 RN 01016 AN 79122941 AU Harries-J-T. Muller-D-P. McCollum-J-P. Lipson-A. Roma-E. Norman-A-P. TI Intestinal bile salts in cystic fibrosis: studies in the patient and experimental animal. SO Arch-Dis-Child. 1979 Jan. 54(1). P 19-24. MJ BILE-ACIDS-AND-SALTS: me. CYSTIC-FIBROSIS: me. DUODENUM: me. INTESTINAL-SECRETIONS: me. MN ADOLESCENCE. ANIMAL. CHILD. CHILD-PRESCHOOL. ENTEROHEPATIC-CIRCULATION. FEMALE. HUMAN. ILEUM: me. INFANT. INTESTINAL-ABSORPTION: de. MALE. RATS. RICINOLEIC-ACIDS: pd. SERUM-ALBUMIN-BOVINE: pd. TAUROCHOLIC-ACID: me. TRIOLEIN: pd. AB The quantitative and qualitative distribution of bile salts in the duodenal juice of 13 patients with cystic fibrosis (CF) was studied after a test meal. The effects of triolein (TO), bovine serum albumin (BSA), and ricinoleic acid (RA) on the absorption of taurocholate (TCA) in the distal ileum of the rat in vivo was also studied. The mean (and ranges) of total bile salt concentrations, glycine: taurine conjugate ratios, and percentage of dihydroxy bile salts in the patients with CF and pancreatic insufficiency were 3.5 (1.3--6.6) mmol/l, 8.6 (greater than 10-3.1), and 37 (10--60) compared with control values of 7.4 (3.0--16.0) mmol/l, 3.0 (1.3--4.5), and 61 (52-- 70) respectively. The differences between the control and CF values were statistically significant (P less than 0.01--P less than 0.001). Three of the 13 CF patients had total bile salt concentrations less than 2 mmol/l, 8 had much higher glycine: taurine ratios, and 8 had a reduced percentage of dihydroxy bile salts. In 2 patients with normal pancreatic enzyme activities, duodenal bile salts were both quantitatively and qualitatively normal. TO (10 and 30 mmol/l), BSA (3%), and RA (5 mmol/l) had no inhibitory effect on the ileal absorption of TCA. These results show pronounced abnormalities of duodenal juice bile salts in CF with pancreatic insufficiency consistent with a broken enterohepatic circulation (EHC); such abnormalities may contribute to defective lipid absorption in CF. The data in the experimental animal do not support the suggestion that unhydrolysed dietary substrates play a role in the pathophysiology of the broken EHC. RF 001 ANTONOWICZ I PEDIATRICS 42 492 968 002 DAWSON AM PROC SOC EXP BIOL MED 142 906 973 003 DEREN JJ N ENGL J MED 288 949 973 004 DOWLING RH GASTROENTEROLOGY 62 122 972 005 FREYE HB J PEDIATR 64 575 964 006 GIBBONS ISE IN: LAWSON D PROC 5TH INT CF 279 969 007 GOODCHILD MC ARCH DIS CHILD 50 769 975 008 HARRIES JT GUT 13 596 972 009 HEATON KW BILE SALTS IN HEALTH AND DISE 972 010 HOFMANN AF IN: JAMES AT 362 964 011 IWATA T J BIOCHEM (TOKYO) 56 424 964 012 MCCOLLUM JPK ARCH DIS CHILD 52 887 977 013 MAKINO I ANAL LETT 5 341 972 014 MATTHEWS DM GUT 6 411 965 015 MORIN CL J PEDIATR 88 213 976 016 POLEY JR J LAB CLIN MED 63 838 964 017 ROLLER RJ GASTROENTEROLOGY 72 661 977 018 ROY CC N ENGL J MED 297 1301 977 019 SLADEN GE BIOCHIM BIOPHYS ACTA 288 443 972 020 THOMAIDIS TS J PEDIATR 63 444 963 021 TOSKES PP J CLIN INVEST 52 1660 973 022 WATKINS JB GASTROENTEROLOGY 67 835 974 023 WATKINS JB GASTROENTEROLOGY 73 1023 977 024 WEBER AM N ENGL J MED 289 1001 973 025 WEBER AM GUT 17 295 976 CT 1 FREDRIKZON B PEDIATR RES 14 1387 980 2 GORIUP U HELV PAEDIATR ACTA 35 177 980 3 BALISTRERI WF J PEDIATR 96 582 980 4 ELIAS E LANCET 2 1319 981 5 FONDACARO JD PROC SOC EXP BIOL MED 168 276 981 6 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 7 MULLER DPR CLIN GASTROENTEROL 11 119 982 8 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 9 HEUBI JE GASTROENTEROLOGY 83 804 982 10 FREDRIKZON B ACTA PAEDIATR SCAND SUPPL 296 1982 75 982 11 BALISTRERI WF J PEDIATR GASTROENTEROL NUTR 2 105 983 12 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 13 ROY CC J PEDIATR GASTROENTEROL NUTR 2 152 983 14 FONDACARO JD PROC SOC EXP BIOL MED 173 118 983 15 COLOMBO C J PEDIATR GASTROENTEROL NUTR 3 556 984 16 TAZAWA Y J PEDIATR GASTROENTEROL NUTR 3 378 984 17 ZENTLERMUNRO PL GUT 25 500 984 18 ABRAMS CK J CLIN INVEST 73 374 984 19 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 20 DARLING PB PEDIATR RES 19 578 985 21 ROBB TA GUT 26 1246 985 22 SETCHELL KDR CLIN CHIM ACTA 151 101 985 23 WEBER AM ACTA PAEDIATR SCAND SUPPL 317 1985 9 985 24 WEIZMAN Z GUT 27 1043 986 25 LEROY C DIG DIS SCI 31 911 986 26 DUTTA SK GASTROENTEROLOGY 91 1243 986 PN 79037 RN 01017 AN 79254866 AU Hubbard-V-S. Davis-P-B. di-SantAgnese-P-A. TI Possible hazard of timed-release aspirin in a patient with pancreatic insufficiency. SO Arch-Intern-Med. 1979 Sep. 139(9). P 1054-5. MJ ASPIRIN: ae. CYSTIC-FIBROSIS: co. HEMORRHAGE-GASTROINTESTINAL: ci. MN ADULT. ASPIRIN: ad. CASE-REPORT. DELAYED-ACTION-PREPARATIONS. HUMAN. INTESTINE-SMALL: de. MALE. OSTEOARTHROPATHY-SECONDARY-HYPERTROPHIC: dt, et. AB An episode of severe small intestinal hemorrhage occurred in a cystic fibrosis patient having pancreatic insufficiency and receiving timed- release aspirin therapy for disabling hypertrophic pulmonary osteoarthropathy. The increased acidity of small intestinal contents due to decreased bicarbonate secretion observed in patients with pancreatic insufficiency may alter the luminal environment and result in mucosal erosions and/or ulcerations in association with the presence of aspirin. Thus, physicians should be aware of the possibility that timed-release aspirin causes small intestinal hemorrhage in such patients. RF 001 LANGMAN MJS GUT 11 627 970 002 LOEBL DH JAMA 237 976 977 003 GARTNER AH JADA 93 111 976 004 TREADWELL BLJ NZ MED J 78 435 973 005 ROSSOUW JE RHEUMATOL REHAB 15 31 976 006 HOON JR JAMA 229 841 974 007 TAUSSIG LM RADIOLOGY 106 369 973 008 HADORN B J PEDIATR 73 39 968 009 THJODLEIFSSON B SCAND J GASTROENTEROL 11 273 976 010 THJODLEIFSSON B SCAND J GASTROENTEROL 11 505 976 011 IVEY KJ GASTROENTEROLOGY 76 50 979 CT 1 PARIENTE EA GASTROENTEROL CLIN BIOL 6 16 982 2 HUBBARD VS SEM RESPIR MED 6 299 985 3 PHILLIPS BM J ROY SOC MED 79 44 986 PN 79038 RN 01018 AN 79123379 AU Duffner-P-K. Cohen-M-E. TI Cystic fibrosis with brain abscess. SO Arch-Neurol. 1979 Jan. 36(1). P 27-8. MJ BRAIN-ABSCESS: co. CYSTIC-FIBROSIS: co. STREPTOCOCCAL-INFECTIONS: co. MN ADULT. BRAIN-ABSCESS: ra. BRAIN: ra. CASE-REPORT. HUMAN. MALE. STREPTOCOCCAL-INFECTIONS: ra. TOMOGRAPHY-X-RAY-COMPUTED. AB A 21-year-old patient with cystic fibrosis developed bilateral brain abscesses due to anaerobic Streptococcus. This rare association presents an interesting etiologic study. RF 001 GATES EM MEDICINE 29 71 950 002 BREWER NS ANN INTERN MED 82 571 975 003 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 004 SCHWARTZ RH AM J DIS CHILD 111 408 966 005 NETER E J INFECT DIS SUPPL 130 132 974 006 DIAZ F J INFECT DIS 121 269 970 007 HALBERT SP PEDIATRICS 26 792 960 CT 1 FISCHER EG J PEDIATR 95 385 979 2 MCCARTHY MM CLIN PEDIATR 19 746 980 3 AYRES J BR J DIS CHEST 76 99 982 4 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 5 RABKIN CS ANN NEUROL 15 608 984 6 KLINE MW PEDIATR INFECT DIS 4 72 985 7 CANNY GJ THORAX 41 221 986 8 KURLAND G PEDIATRICS 78 1097 986 PN 79039 RN 01019 AN 79208794 AU Forman-Franco-B. Abramson-A-L. Gorvoy-J-D. Stein-T. TI Cystic fibrosis and hearing loss. SO Arch-Otolaryngol. 1979 Jun. 105(6). P 338-42. MJ CYSTIC-FIBROSIS: co. HEARING-LOSS-CONDUCTIVE: et. HEARING-LOSS-PARTIAL: et. HEARING-LOSS-SENSORINEURAL: et. MN ACOUSTIC-IMPEDANCE-TESTS. ADOLESCENCE. ADULT. AUDIOMETRY. AUDITORY-THRESHOLD. CHILD. EAR-OSSICLES: pp. EAR-MIDDLE: pp. FEMALE. GENTAMICINS: ae. HUMAN. MALE. PRESSURE. STAPES: pp. TOBRAMYCIN: ae. TYMPANIC-MEMBRANE: pp. AB The mucosal epithelium of the middle ear and Eustachian tube is in direct continuity with the upper respiratory tract. Since the otolaryngological aspects of cystic fibrosis (CF) are dominated by involvement of the paranasal sinuses, it might be assumed that children with CF would be expected to have a higher than usual incidence of middle ear disease. Eighty patients who were afflicted with CF had audiological evaluations, which consisted of hearing threshold levels (250 to 8,000 Hz) and speech and impedance audiometry. We found no greater incidence of a conductive or sensorineural hearing loss in patients with CF when compared with a normal age-adjusted population. RF 001 KULCZYCKI LL CLIN PEDIATR 9 390 970 002 ANON TRANS AM ACAD OPHTHALMOL OTOL 63 236 959 003 JERGER J ARCH OTOLARYNGOL 93 111 971 004 TAYLOR BW ARCH DIS CHILD 49 133 974 005 GOODMAN A ASHA 7 262 965 006 RULON JT ARCH OTOLARYNGOL 78 192 963 007 CRIFO S PRACT OTORHINOLARYNGOL 33 394 971 008 KULCZYCKI LL ARCH OTOLARYNGOL 92 54 970 009 ROBINSON CG CAN MED ASSOC J 97 1199 967 010 EAGLES E LARYNGOSCOPE 73 12 963 011 SIEGEL J ARCH OTOLARYNGOL 92 523 970 012 FORCUCCI RA ARCH OTOLARYNGOL 96 361 972 013 FRITZE W Z KINDERHEILK 114 111 973 014 WATSON TJ PROC R SOC MED 62 455 969 015 BROOKS DN INT AUDIOL 8 563 969 016 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 CT 1 CRIFO S INT J PEDIATR OTORHINOLARYNGO 2 251 980 2 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 3 DAVID TJ J ROY SOC MED 79 23 986 PN 79040 RN 01020 AN 79230729 AU Goldberg-R-T. Isralsky-M. Shwachman-H. TI Vocational development and adjustment of adolescents with cystic fibrosis. SO Arch-Phys-Med-Rehabil. 1979 Aug. 60(8). P 369-74. MJ ATTITUDE. CAREER-CHOICE. CYSTIC-FIBROSIS: rh. DECISION-MAKING. GOALS. MN ADOLESCENCE. CHILD-DEVELOPMENT. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: px. EDUCATION. HUMAN. SOCIAL-VALUES. VOCATIONAL-GUIDANCE. AB Adolescents with cystic fibrosis were compared with normal adolescents of the same age and educational grade on several measures of vocational development and adjustment. The cystic fibrosis group in ages 12--16 and in grades 7, 8, and 9 scored lower than their normal counterparts in measures of vocational and educational plans, and were less realistic than normals in considering their limitations and financial constraints. In contrast, the cystic fibrosis group at nearly all age and grade levels scored significantly higher on strength of commitment to vocational choice, work values, and awareness of occupational information. Compared to 4 additional handicapped groups already studied, adolescents with cystic fibrosis scored favorably. They not only plan to work but also hold strong work values compared to normals. Rehabilitation programs should emphasize preparation for a career and the development of educational and vocational plans while the adolescent is still in high school. RF 001 BOYLE IR J PEDIATR 88 318 976 002 TROPAUER A AM J DIS CHILD 119 424 970 003 CYTRYN L IN: ANTHONY EJ 37 973 004 GAYTON WF AM J DIS CHILD 126 856 973 005 ROSENLUND ML ANN INTERN MED 78 959 973 006 SHWACHMAN H PEDIATRICS 46 335 970 007 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 008 SHWACHMAN H N ENGL J MED 296 1519 977 009 TURK J PEDIATRICS 34 67 964 010 MCCOLLUM AT J PEDIATR 77 571 970 011 MCCRAE WM PHYSIOTHERAPY 61 252 975 012 MATTHEWS LW VOCATIONAL AND LIFE ADJUSTMEN 969 013 GOLDBERG RT SCAND J REHAB MED 9 85 977 014 GOLDBERG RT J COUNSELING PSYCH 21 428 974 015 GOLDBERG RT REHAB COUNS BULL 19 140 975 016 GOLDBERG RT SCAND J REHAB MED 10 215 978 017 GOLDBERG RT VOCATIONAL AND SOCIAL REHABIL 972 018 KULCZYCKI LL IN: PATTERSON PR 117 973 CT 1 PINKERTON P LANCET 2 761 985 2 SIMMONS RJ PSYCHOSOM MED 47 111 985 3 GOLDBERG RT ARCH PHYS MED REHABIL 66 492 985 PN 79041 RN 01021 AN 80175020 AU Bergan-T. Michalsen-H. TI Pharmacokinetics of azlocillin in children with cystic fibrosis. SO Arzneimittelforschung. 1979. 29(12a). P 1955-7. MJ CYSTIC-FIBROSIS: me. PENICILLINS: me. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. DOSE-RESPONSE-RELATIONSHIP-DRUG. FEMALE. HALF-LIFE. HUMAN. KINETICS. MALE. PENICILLINS: bl, ur. TIME-FACTORS. AB 6-E1(R)-2-(oxo-imidazolidine-1-carboxamido)-2-phenyl-acetamido]- penicillanic acid sodium salt (azlocillin, Securopen) was given in doses of 100 and 200 mg/kg body weight to children with cystic fibrosis. After intravenous bolus infections, the serum half-life was 0.82 +/- 0.12 h after the lower dose and 0.98 +/- 0.18 h after the higher dose. This was consequent to a dose limited elimination kinetics due to limitation in both renal and non-renal processes of elimination. Upon doubling of the dose from 100 to 200 mg/kg, the total body clearance dropped from 13.93 to 5.10 1/h. Evaluation of data presented in other publications indicate that a dose limited elimination kinetics is the normal situation for azlocillin. Besides, the serum concentrations of patients with cystic fibrosis were considerably lower than seen in the healthy state. The reason is faster elimination by the renal route in cystic fibrosis. RF 001 BERGAN T ANTIMICROB AGENTS CHEMOTHER 13 801 978 002 BERGAN T ACTA PATH MICROBIOL SCAND (B) 80 101 972 003 EVANS MAL J ANTIMICROB CHEMOTHER 4 255 978 004 GIAMARELLOU H INT SYMPOS ACYLUREIDOPENICILL 68 978 005 JUSKO WJ PEDIATRICS 56 1038 975 006 KONIG HB INFECTION 5 170 977 007 KRASEMANN C INT SYMPOS ACYLUREIDOPENICILL 84 978 008 LODE H INFECTION 5 163 977 009 REEVES DS INT SYMPOS ACYLUREIDOPENICILL 18 978 010 WAGNER JG FUND CLIN PHARMACOKINETICS 975 011 WIRTH K INFECTION 4 25 976 012 YAFFE SJ J INFECT DIS 135 828 977 CT 1 MICHALSEN H ANTIMICROB AGENTS CHEMOTHER 19 1029 981 2 BERGAN T SCAND J INFECT DIS 1981 33 981 3 MALMBORG AS SCAND J INFECT DIS 1981 64 981 4 MICHALSEN H SCAND J INFECT DIS 1981 92 981 5 LEVY J J ANTIMICROB CHEMOTHER 10 235 982 6 ELIOPOULOS GM ANN INTERN MED 97 755 982 7 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 8 MICHALSEN H ACTA PAEDIATR SCAND SUPPL 301 1982 101 982 9 AGOSTINI M DRUGS UNDER EXP CLIN RES 9 671 983 10 BERGAN T J ANTIMICROB CHEMOTHER 11 101 983 11 MASTELLA G J ANTIMICROB CHEMOTHER 12 297 983 12 ISLES A AM REV RESPIR DIS 127 417 983 13 MARTINI N J CLIN HOSP PHARM 9 303 984 14 SINGLAS E PRESSE MED 13 788 984 15 LEEDER JS CLIN PHARMACOL THER 36 355 984 16 PENKETH A THORAX 39 299 984 17 MICHALSEN H PATHOL RES PRACT 178 261 984 18 ANON LANCET 1 1020 985 19 WOOLF RA CLIN PHARMACY 4 664 985 20 PRANDOTA J DEVELOP PHARM THER 8 311 985 21 HORREVORTS AM CHEST 88 260 985 22 JACOBS RF J PEDIATR 106 1001 985 23 WILSON CB J PEDIATR 106 1049 985 24 SORGEL F EUR J PEDIATR 143 249 985 25 MICHALSEN H METH FIND EXP CLIN PHARMACOL 8 727 986 26 BENDER SW INFECTION 14 17 986 27 SCHAAD UB ACTA PAEDIATR SCAND 75 128 986 PN 79042 RN 01022 AN 80129795 AU Chapman-J-A. Goodall-J. TI Dying children need help too. SO Aust-Fam-Physician. 1979 Dec. 8(12). P 1236-7. MJ CHILD-HOSPITALIZED. CYSTIC-FIBROSIS: th. TERMINAL-CARE. MN ATTITUDE-TO-DEATH. CASE-REPORT. FAMILY. FEMALE. GRIEF. HUMAN. INFANT. PAIN. EX The control of symptoms in dying children is often sadly neglected. This neglect is reflected in a review of the case notes of two children who died of cystic fibrosis within three years of each other. The girls were under the care of the same consultant, who in the interval between the two cases was introduced to the concern of proper terminal care for children. RF 001 BRETT EM DEVELOP MED CHILD NEUROL 12 655 970 002 SHAFFER D IN: RUTTER M 977 003 TWYCROSS RG IN: SAUNDERS CM 978 PN 79043 RN 01023 AN 80129794 AU Phelan-P-D. TI Cystic fibrosis: is it relevant to family practitioners?. SO Aust-Fam-Physician. 1979 Dec. 8(12). P 1232-4. MJ CYSTIC-FIBROSIS: di. FAMILY-PRACTICE. MN ADOLESCENCE. ADULT. AUSTRALIA. CHILD. CYSTIC-FIBROSIS: oc, th. HUMAN. PATIENT-CARE-TEAM. AB There has been a major change in the outlook for children with cystic fibrosis during the last 20 years. Provided the diagnosis is made before serious irreversible lung disease has occurred, most patients can expect to reach adult life without very serious disability. PN 79044 RN 01024 AN 80019967 AU Carey-W-F. Hosli-P. TI Inhibition of Tamm-Horsfall glycoprotein induction of alkaline phosphatase in cystic fibrosis fibroblasts by medium conditioned by normal cells. SO Aust-J-Exp-Biol-Med-Sci. 1979 Apr. 57(2). P 225-30. MJ ALKALINE-PHOSPHATASE: me. CELLS-CULTURED: me. CYSTIC-FIBROSIS: me. FIBROBLASTS: me. GLYCOPROTEINS: pd. MN COMPARATIVE-STUDY. CULTURE-MEDIA. CYSTIC-FIBROSIS: en. HUMAN. TIME-FACTORS. AB It is confirmed that the level of alkaline phosphatase in fibroblasts derived from cystic fibrosis patients can be induced many-fold by growing the cells in the presence of Tamm-Horsfall glycoprotein. It is further shown that normal fibroblasts produce a "CF corrective factor" which markedly inhibits this phenomenon. These observations support a previous hypothesis on the nature of the metabolic defect in cystic fibrosis. RF 001 BACH G PROC NAT ACAD SCI USA 69 2048 972 002 FRATANTONI JC PROC NAT ACAD SCI USA 64 360 969 003 HOSLI P ADV EXP MED BIOL 68 1 976 004 HOSLI P CLIN CHEM 23 1476 977 005 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 006 HOSLI P HUM GENET 41 169 978 007 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 008 HOSLI P PROC INT CF CONG 7TH 278 976 009 HOSLI P ACTA PAEDIATR SCAND 67 617 978 CT 1 CAREY WF MED J AUST 2 604 979 2 LYNN KL BIOCHEM J 194 561 981 3 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 4 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 5 BUYS CHCM CLIN CHIM ACTA 136 229 984 PN 79045 RN 01025 AN 80087357 AU Feery-B-J. Phelan-P-D. Gallichio-H-A. Hampson-A-W. TI Antibody responses to influenza virus vaccine in patients with cystic fibrosis. SO Aust-Paediatr-J. 1979 Sep. 15(3). P 181-2. MJ ANTIBODIES-VIRAL: im. CYSTIC-FIBROSIS: im. INFLUENZA-VACCINE: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. HEMAGGLUTINATION-INHIBITION-TESTS. HUMAN. INFLUENZA-VACCINE: ad. ORTHOMYXOVIRUSES-TYPE-A: im. AB Patients with cystic fibrosis were immunized with subunit influenza vaccine and their haemagglutination-inhibiting (HI) antibody responses were compared with responses in young healthy adults. There was no significant difference in the antibody levels reached after immunization and no evidence of any impairment of responses in the patient group. RF 001 DAVENPORT FM J EXP MED 98 641 953 002 ANON MED J AUST 1 1310 970 003 PALMER DF ADVANCED LABORATORY TECHNIQUE 975 PN 79046 RN 01026 AN 79255569 AU Bogart-B-I. Conod-E-J. Gaerlan-P-F. Denning-C-R. Conover-J. TI Biological activities of cystic fibrosis serum. III. CF serum induced uptake of 45Ca++ by rabbit tracheal explants. SO Biochem-Biophys-Res-Commun. 1979 Jun 27. 88(4). P 1398-404. MJ CALCIUM: me. CYSTIC-FIBROSIS: bl. MN ANIMAL. CELL-MEMBRANE-PERMEABILITY: de. CILIA: pp. CYSTIC-FIBROSIS: me, pp. GALLOPAMIL: pd. HETEROZYGOTE. HUMAN. IGG: ph. RABBITS. TRACHEA: me. AB Cystic Fibrosis (CF) serum and its isolated component IgG fraction produce an increased uptake of 45Ca++ in rabbit tracheal explants when compared to control serum and its isolated IgG fraction. Heterozygote serum also produced an increased uptake of 45Ca++ but not to the same extent as CF serum. The calcium channel blocker D600 inhibited the CF serum induced uptake of 45Ca++ indicating that CF serum may be acting on the plasma membrane to produce changes in calcium permeability in rabbit tracheal explants. RF 001 SPOCK A PEDIATR RES 1 173 967 002 CONOVER JH PEDIATR RES 7 220 973 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 BOWMAN BH SCIENCE 164 325 969 005 BOGART BI PEDIATR RES 11 131 977 006 BOGART BI PEDIATR RES 12 15 978 007 PUTNEY JW J PHARMACOL EXP THER 199 526 976 008 CONOD EJ PEDIATR RES 11 45 977 009 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 010 CONOVER JH BIOCHEM BIOPHYS RES COMMUN 83 1595 978 011 FLECKENSTEIN A IN: CARAFOLI E 555 975 012 BANSCHBACH MW BIOCHEM BIOPHYS RES COMMUN 84 922 978 CT 1 BOWMAN BH FED PROC 39 3195 980 2 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 3 WILSON GB MED HYPOTHESES 8 527 982 4 BOGART BI PEDIATR RES 16 223 982 5 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 6 KATZ S CELL CALC 5 421 984 PN 79047 RN 01027 AN 79144816 AU Alhadeff-J-A. Watkins-P. TI Differential concanavalin A binding of cystic fibrosis and normal liver alpha-L-fucosidase. SO Biochem-Biophys-Res-Commun. 1979 Feb 14. 86(3). P 787-92. MJ CONCANAVALIN-A: me. CYSTIC-FIBROSIS: en. FUCOSIDASE: me. LIVER: en. MN COMPARATIVE-STUDY. HUMAN. KINETICS. PROTEIN-BINDING. SUPPORT-U-S-GOVT-P-H-S. AB A novel technique has been employed to demonstrate that alpha-L- fucosidase purified from cystic fibrosis and control livers exhibits differential binding to the lectin Concanavalin A. The concentration of alpha-CH3-mannoside necessary to prevent 50% binding of alpha-L-fucosidase to Concanavalin A is considerably lower for the cystic fibrosis enzyme (13.5 vs. 33.3 mM). Comparable results were found when binding studies were done on crude supernatant alpha-L-fucosidase from 8 cystic fibrosis and 8 control livers (5.6 plus or minus 0.4 mM and 13.2 plus or minus 3.4 respectively), without any overlap of values between the cystic fibrosis and control livers. These results suggest that comparative lectin binding studies on cystic fibrosis and normal glycoproteins from readily available tissues might result in an assay for detecting the cystic fibrosis genotype. RF 001 MCCOMBS ML TEX REP BIOL MED 31 615 973 002 NADLER HL IN: STANBURY JB 1683 978 003 WRIGHT SW AM J HUM GENET 20 157 968 005 ALHADEFF JA CLIN GENET 13 417 978 006 ALHADEFF JA J BIOL CHEM 250 7106 975 007 ALHADEFF JA MOL CELL BIOCHEM 18 33 977 008 ALHADEFF JA CLIN GENET 10 63 976 009 ALHADEFF JA IN: GLEW RH 247 977 010 BISHAYEE S BIOCHIM BIOPHYS ACTA 334 378 974 011 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 012 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 013 SCANLIN TF JR PEDIATR RES ABST 549 11 463 977 014 LIS H IN: SELA M 4 429 977 CT 1 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 2 ALHADEFF JA CLIN CHIM ACTA 105 131 980 3 HALLINAN F MED HYPOTHESES 7 793 981 4 ALHADEFF JA CLIN CHIM ACTA 117 227 981 5 MALER T J BIOL CHEM 256 1420 981 6 SCANLIN TF BIOCHEMISTRY 21 491 982 7 BOURASSA C CLIN CHIM ACTA 129 263 983 8 HERMELIN B CELL MOL BIOL 33 83 987 PN 79048 RN 01028 AN 80069763 AU Adams-T-H. Ramsay-G-M. Wisdom-G-B. TI Enzyme immunoassay of albumin as an aid to the diagnosis of cystic fibrosis in the newborn human [proceedings]. SO Biochem-Soc-Trans. 1979 Oct. 7(5). P 1018-9. MJ CYSTIC-FIBROSIS: di. SERUM-ALBUMIN: an. MN CYSTIC-FIBROSIS: bl. HUMAN. IMMUNOENZYME-TECHNICS. INFANT-NEWBORN. MECONIUM: an. EX One of the symptoms of cystic fibrosis (mucoviscidosis) is pancreatic insufficiency. The reduced proteolytic activity in the gut of children with the disease results in an elevated concentration of protein, especially serum albumin, in the meconium. This feature is used in the diagnosis of the disease in the newborn, and we decided to examine the utility of enzyme immunoassay for the measurement of albumin in meconium samples from normal and cystic fibrosis infants. Normal samples were found to have apparent albumin concentrations in the range 0.05-0.26 mg/g dry wt., whereas cystic fibrosis samples had concentrations in the range 60-230 mg/g dry wt. The assay provides a cheap and relatively rapid method of differentiating cystic fibrosis positive and negative meconium samples. RF 001 HELLSING K SCAND J CLIN LAB INVEST 33 333 974 002 MAIOLINI R J IMMUNOL METH 8 223 975 003 NAKANE PK J HISTOCHEM CYTOCHEM 22 1084 974 004 RYLEY HC CLIN CHIM ACTA 64 117 975 CT 1 NAYLOR EW SEM PERINATOL 9 232 985 PN 79049 RN 01029 AN 80021202 AU Wijcik-L. Buchwald-M. Riordan-J-R. TI Induction of alkaline phosphatase in cultured human fibroblasts. Comparison of normal cells and those from patients with cystic fibrosis. SO Biochim-Biophys-Acta. 1979 Jul 4. 585(3). P 374-82. MJ ALKALINE-PHOSPHATASE: bi. CYSTIC-FIBROSIS: en. FIBROBLASTS: en. MN CELL-DIVISION. CELLS-CULTURED. COMPARATIVE-STUDY. DNA: bi. ENZYME-INDUCTION: de. HUMAN. TIME-FACTORS. AB The membrane glycoprotein enzyme, alkaline phosphatase was induced in cultured human fibroblasts by dibutyryl cyclic AMP, sodium butyrate, the serum glycoprotein fetuin, the Tamm-Horsfall urinary glycoprotein, and by a number of inhibitors of DNA synthesis. The uninduced basal enzyme activity increased at later stages of growth when the cells became confluent. Induction by dibutyryl cyclic AMP or fetuin was most effective when the agents were added after the cells had reached stationary phase and was maximal after at least two days of exposure. The levels of induction resulting from the addition of pairs of the agents, dibutyryl cyclic AMP, n-butyrate and fetuin were additive indicating that these have different modes of action. The inhibitors of DNA synthesis, cytosine arabinoside, hydroxyurea, and methothrexate were less effective inducers. Bromodeoxyuridine which also has non-DNA mediated effects induced to the same extent as dibutyryl cyclic AMP. Similar experiments with sex- and age-matched cell strains derived from patients with cystic fibrosis failed to detect differences in the levels of induction from those observed in normal cells. In addition, the combined inductive effects of Tamm- Horsfall glycoprotein, isoproterenol and theophylline, were similar with normal and cystic fibrosis cells. RF 001 STEPAN J EXPERIENTIA 29 948 973 002 FISHMAN WH AM J MED 56 617 974 003 LIN CW J HISTOCHEM CYTOCHEM 24 659 976 004 RIORDAN JR IN: JULIONO RL 2 146 978 005 KOYAMA H BIOCHEM BIOPHYS RES COMMUN 46 305 972 006 MAZIERE JC BIOCHIMIE 59 221 977 007 NOSE K JAPAN J EXP MED 46 347 976 008 COX RJ BIOCHEM J 105 155 967 009 COX RP J MOL BIOL 58 197 971 010 GRIFFIN MJ ARCH BIOCHEM BIOPHYS 164 619 974 011 MIEDEMA E EXP CELL RES 53 488 968 012 VANNEUVILLE FJ BIOCHEM SOC TRANS 5 1117 977 013 LUNDGREN E EXP CELL RES 110 25 977 014 HOSLI P ADV EXP MED BIOL 68 1 974 015 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 016 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 017 RIORDAN JR J SUPRAMOL STRUCT SUPPL 2 126 978 018 TAMM I J EXP MED 95 71 952 019 BUCHWALD M MOD PROBL PEDIATR 19 165 977 020 STANNERS CP NATURE NEW BIOL 230 52 971 021 BOHLEN P ARCH BIOCHEM BIOPHYS 155 213 973 022 DEUTSCH SI BIOCHIM BIOPHYS ACTA 499 382 977 023 GHOSH NK NATURE 267 435 977 024 HENNEBERRY RC NATURE 268 252 977 025 TALLMAN JF PROC NAT ACAD SCI USA 74 873 977 026 MERIMEE TJ J CLIN ENDOCRINOL METAB 43 1190 976 027 FISHMAN PH BIOCHEM BIOPHYS RES COMMUN 59 292 974 028 GHOSH NK NATURE 267 435 977 029 WRIGHT JA EXP CELL RES 78 456 973 030 GINSBURG E PROC NAT ACAD SCI USA 70 2457 973 031 HENNEBERRY RC CELL 5 1 975 032 VIDALI G PROC NAT ACAD SCI USA 75 2239 978 033 HORVAT A J CELL PHYSIOL 83 59 974 034 MAZIERE C BIOCHIMIE 59 225 977 CT 1 CAREY WF MED J AUST 2 604 979 2 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 3 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 4 CROFTON PM CRC CRIT REV CLIN LAB SCI 16 161 982 5 BUCHWALD M CLIN BIOCHEM 17 143 984 6 ZGURSKII AA BIOCHEMISTRY USSR 49 1380 984 7 ZGURSKY AA TSITOLOGIYA 27 775 985 8 YANG AH IN VITRO CELL DEVEL BIOL 23 34 987 9 WHYTE MP CALC TISS INT 40 244 987 PN 79050 RN 01030 AN 80000602 AU Riordan-J-R. Alon-N. Buchwald-M. TI Plasma membrane lipids of human diploid fibroblasts from normal individuals and patients with cystic fibrosis. SO Biochim-Biophys-Acta. 1979 Jul 27. 574(1). P 39-47. MJ CYSTIC-FIBROSIS: me. MEMBRANE-LIPIDS: an. MN CELLS-CULTURED. CHOLESTEROL: an. FATTY-ACIDS: an. FIBROBLASTS. HUMAN. MEMBRANE-FLUIDITY. PHOSPHOLIPIDS: an. SKIN: an. AB The lipid composition of isolated plasma membranes of human skin fibroblasts is described for the first time. Plasma membranes from a number of strains of fibroblasts from patients with cystic fibrosis and matched normals were isolated by a recently described procedure and analysed for major phospholipid classes, cholesterol and fatty acids. No differences in the quantities of these compounds were detected between cells of the two different origins. The fetal calf serum used to supplement the growth medium contained relatively more palmitoleate and oleate but less stearate than the membranes. There were also no consistent differences between cystic fibrosis and normal membranes in terms of the fatty acid compositions of their individual phospholipid classes. Consistent with this lack of chemical change in the lipids of membranes of cystic fibrosis cells, the degree of fluorescence polarization of diphenylhexatriene, an index of fluidity, was also unchanged. RF 001 KARTNER N J MEMBR BIOL 36 191 977 002 KUO PT J PEDIATR 60 394 962 003 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 004 SANJURJO P LANCET 1 752 977 005 ROSENLUND ML PEDIATRICS 59 428 977 006 RIVERS JPW LANCET 2 642 975 007 ELLIOTT RB PEDIATRICS 57 474 976 008 DI SANTAGNESE PA N ENGL J MED 295 534 976 009 FARIAS RN BIOCHIM BIOPHYS ACTA 415 231 975 010 LUCY JA FEBS LETTERS SUPP 1 40 5105 974 011 CHRISTIAN ST BIOCHEM BIOPHYS RES COMMUN 79 966 977 012 LOWRY OH J BIOL CHEM 193 265 951 013 FOLCH J J BIOL CHEM 226 497 957 014 BARTLETT GR J BIOL CHEM 234 466 959 015 ROUSER G IN: MARINETTI GV 1 99 967 016 VAN HOEVEN RP J MEMBR BIOL 9 105 972 017 SHINITZKY M BIOCHIM BIOPHYS ACTA 433 133 976 018 MALKIEWICZ-WASOWICZ B BIOCHIM BIOPHYS ACTA 482 358 977 019 BENEDETTI EL IN: DALTON AJ 33 968 020 PATTON S J THEOR BIOL 29 489 970 021 PFLEGER RC BIOCHEMISTRY 7 2826 968 022 WOOD RE ARCH BIOCHEM BIOPHYS 141 174 970 023 HENNING R HOPPE SEYLERS Z PHYSIOL CHEM 351 1191 970 024 HOROWITZ AF PROC NAT ACAD SCI USA 71 3115 974 025 CAMPBELL IM PEDIATRICS 57 480 976 026 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 027 SCHROEDER F J BIOL CHEM 251 5015 976 028 SAHU S AM REV RESPIR DIS 115 233 977 029 MCEVOY FA LANCET 2 236 975 030 DALE RE J BIOL CHEM 252 7500 977 CT 1 KEELEY FW CAN J BIOCHEM 57 1273 979 2 PHILLIPS MC ATHEROSCLEROSIS 36 409 980 3 RIORDAN JR CAN J BIOCHEM 58 928 980 4 MALER T BIOCHIM BIOPHYS ACTA 598 1 980 5 KOIZUMI K BIOCHIM BIOPHYS ACTA 619 344 980 6 ROGIERS V PEDIATR RES 16 761 982 7 FIELDING CJ J BIOL CHEM 257 955 982 8 ROTHBLAT GH J BIOL CHEM 257 4775 982 9 HARRIS A CLIN CHIM ACTA 128 41 983 10 HUBBARD VS EUR J PEDIATR 141 68 983 11 SLOTTE JP BIOCHEM J 220 605 984 12 SLOTTE JP BIOCHIM BIOPHYS ACTA 793 423 984 13 RIORDAN JR PHARMACOL THER 28 51 985 14 LUNDBERG B ATHEROSCLEROSIS 56 345 985 15 BRAUNAGEL SC BIOCHIM BIOPHYS ACTA 813 183 985 16 GOLDBERG DM CLIN PHYSIOL BIOCHEM 4 305 986 17 LUNDBERG B ACTA CHEM SCAND (A) 40 315 986 PN 79051 RN 01031 AN 79232883 AU Lappe-M. TI 2. Theories of genetic causation in human disease. SO Birth-Defects. 1979. 15(2). P 33-45. MJ GENETICS-MEDICAL. HEREDITARY-DISEASES. MN ALCOHOLISM: fg. CYSTIC-FIBROSIS: fg. GENETICS-BEHAVIORAL. GENOTYPE. HUMAN. LESCH-NYHAN-SYNDROME: fg. PHENOTYPE. PHENYLKETONURIA: fg. VARIATION-GENETICS. XYY-KARYOTYPE. EX The central problem of the mid-20th century genetic counselor has been to determine the place of the genes in the origin of complex human characteristics - such as neural tube defects, cleft palate, diabetes, and congenital heart disease - in short, the major malformation and polygenic syndromes which make up the bulk of what we call birth defects. Of comparable importance in terms of genetic counseling is understanding the origin and incidence of the newly recognized genetic heterogeneity lying behind what had previously been regarded as simple, single-gene determined diseases. Topics include dealing with genetic heterogeneity, phenotypes and genotypes, where genetic causation defies simple analysis, and sources of variation. Behavior disorders with presumptive single-gene causation (Lesch-Nyhan disease), presumptive sex-linked causation (aggressivity), and presumptive polygenic causation (alcoholism) are also discussed. RF 000 JOHANSSEN W IN: PETERS JA 959 001 KAUFMAN S N ENGL J MED 293 785 975 002 BLANCK RR JAMA 235 1364 976 003 DARLINGTON CD GENETICS AND MAN 102 969 004 NEWMAN HH EVOLUTION GENETICS AND EUGENI 485 969 005 WRIGHT S GENETICS 19 537 934 006 BECK F IN: ROBSON JM 1 965 007 FRASER FC PROC INT CONF CONGENITA 1ST 179 961 008 KALTER H TERATOLOGY 13 1 976 009 BARLOW SM TERATOLOGY 12 97 975 010 ERWAY L SCIENCE 152 1766 966 011 ANON COLUMBIA JOURNALISM REVIEW MA 975 012 LENZ W J MED GENET 10 34 973 013 DEGENHARDT KH IN: RASPE G 6 971 014 ROSENTHAL MR LANCET 2 250 976 015 VAN DE PUTTE I LANCET 2 251 976 016 CAVALLI-SFORZA LL AM J HUM GENET 25 618 973 017 ANDERSON LT PSYCHOLOGICAL SPECTATOR 10 4 975 018 SELMANOFF MK NATURE 253 529 975 019 SELMANOFF MK BEHAV GENET 6 53 976 020 LAGERSPETZ KMJ IN: VAN ABEELEN 321 974 021 RANDT CT DEVELOP PSYCHOBIOL 8 275 975 023 OMENN GS ANN NY ACAD SCI 197 16 972 024 FULLER JL COMPARATIVE PHYSIOL PSYCHOL 57 85 965 025 RANDALL CL SCIENCE 189 149 975 026 HIRSCH J IN: HIRSCH J 416 967 PN 79052 RN 01032 AN 79233368 AU MacErlean-D-P. Gray-B-J. FitzGerald-M-X. TI Bronchial artery embolization in the control of massive haemoptysis. SO Br-J-Radiol. 1979 Jul. 52(619). P 558-61. MJ BRONCHIAL-ARTERIES. EMBOLIZATION-THERAPEUTIC. HEMOPTYSIS: th. MN ADOLESCENCE. ADULT. ASPERGILLOSIS: co. BRONCHIAL-ARTERIES: ra. CASE-REPORT. CYSTIC-FIBROSIS: co. FEMALE. HEMOPTYSIS: et. HUMAN. LUNG-DISEASES-FUNGAL: co. MALE. AB Bronchial artery embolization is a useful palliative treatment for life-threatening haemoptyses where surgery is contra-indicated. Patients presenting with haemoptyses complicating cystic fibrosis and aspergilloma have been embolized. Control of haemoptysis was achieved in the former, but late recurrence of haemoptysis in the latter led to asphyxiation. RF 001 BOOKSTEIN JJ CHEST 72 658 977 002 BREDIN CP CHEST 72 258 977 003 KARDJIEV V RADIOLOGY 112 81 974 004 MACERLEAN DP BR J RADIOL 51 414 978 005 REMY J RADIOLOGY 122 33 977 006 SCHUSTER SR J PEDIATR SURG 12 889 977 007 STONE RM SURG FORUM 17 109 966 008 WHOLEY MH JAMA 236 2501 976 CT 1 COLLINS JV BR J HOSP MED 23 179 980 2 FAIRFAX AJ BR J DIS CHEST 74 345 980 3 MAGILLIGAN DJ ANN THORAC SURG 32 392 981 4 FERRIS EJ CHEST 80 710 981 5 KUCERA V THORAC CARDIOVASC SURG 30 152 982 6 GRENIER P AM J ROENTGENOL 140 467 983 7 NAAR CA AM J ROENTGENOL 140 271 983 8 PORTER DK ARCH INTERN MED 143 287 983 9 UFLACKER R RADIOLOGY 146 627 983 10 TAFRESHI M NY STATE J MED 85 38 985 11 KLAMUT M FORTSCHR GEB RONTG NUKL 143 645 985 12 NOLAN MT IR J MED SCI 154 336 985 PN 79053 RN 01033 AN 80044035 TI Cystic fibrosis in adults [editorial]. SO Br-Med-J. 1979 Sep 15. 2(6191). P 626. MJ CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. BRONCHIAL-DISEASES: et. CYSTIC-FIBROSIS: oc. FEMALE. HUMAN. LUNG-DISEASES: et. MALE. PANCREATIC-DISEASES: et. EX Cystic fibrosis is the commonest semilethal genetic disorder in Caucasians. The biochemical sequence that leads to widespread dysfunction of exocrine glands has yet to be determined. Cystic fibrosis in adults has been the subject of extensive review. We still do not know the cause of the changes in the lungs. Pancreatic insufficiency was recorded in 95% of the patients, but the older ones needed little dietary restriction and some seemed to thrive without pancreatic replacement. Ninety-five per cent of males have aspermia due to failure of development of the vas deferens, epididymis, and seminal vesicles. Despite the obvious handicap and emotional and social strains in all four series, adolescent and adult patients with cystic fibrosis not only did well at school and at work but managed to fit in well in their social framework. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 DI SANTAGNESE PA AM J MED 66 121 979 003 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 004 MITCHELL-HEGGS PF Q J MED 45 479 976 005 GRACEY M AUSTRALAS ANN MED 18 91 969 PN 79054 RN 01034 AN 80044037 AU Flower-K-A. Eden-R-I. Lomax-L. Mann-N-M. Burgess-J. TI New mechanical aid to physiotherapy in cystic fibrosis. SO Br-Med-J. 1979 Sep 15. 2(6191). P 630-1. MJ CYSTIC-FIBROSIS: th. PERCUSSION: is. PHYSICAL-THERAPY: is. MN ADOLESCENCE. ADULT. AGED. HUMAN. MIDDLE-AGE. PRESSURE. AB The force of impact and frequency of percussion by physiotherapists and parents of children with cystic fibrosis were analysed on a special test rig and incorporated in a prototype percussor. In adult volunteers and cadavers a maximum intrathoracic pressure could be achieved by a critical frequency of mechanical percussion which was higher than that reached by physiotherapists and parents. Consequently the percussor was redesigned to operate at this optimum frequency. It was then discovered that if the percussor was pressed firmly enough against the chest, this maximum intrathoracic pressure could be indicated by quivering of the voice. In a continuing study of intrathoracic pressures obtained mechanically and manually the Salford percussor's produced higher pressures than the physiotherapists' and maintained them constantly, while the physiotherapists' efforts and results varied from one to another. Hospital and domiciliary use of the percussor have shown it to help in the first stage of the physiotherapy routine for patients with cystic fibrosis. The percussor should enable adolescents and adults to treat themselves and encourage twice-daily and more effective chest treatments. It is easy to apply and its speed and efficiency should enable parents to improve the quality of their chest therapy for younger children at home. Its long-term benefits are difficult to assess because of the nature of the disease. RF 001 SHWACHMAN H CF CLUB ABST 13 974 002 ROBINSON MJ ARCH DIS CHILD 50 962 975 003 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 004 HODSON ME BR MED J 1 971 978 005 LOUGH MD PEDIATRIC RESPIRATORY THERAPY 974 CT 1 ANON LANCET 2 729 979 2 FREEDMAN BJ EUR J RESPIR DIS 61 254 980 3 HOLODY B AM REV RESPIR DIS 124 372 981 4 KERREBIJN KF EUR J RESPIR DIS 63 35 982 5 BATTISTINI A RIV ITAL PEDIATR 10 220 984 PN 79055 RN 01035 AN 80021861 AU Pryor-J-A. Webber-B-A. Hodson-M-E. Batten-J-C. TI Evaluation of the forced expiration technique as an adjunct to postural drainage in treatment of cystic fibrosis. SO Br-Med-J. 1979 Aug 18. 2(6187). P 417-8. MJ BREATHING-EXERCISES. CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. COMPARATIVE-STUDY. FEMALE. HUMAN. MALE. PHYSICAL-THERAPY: mt. POSTURE. SPUTUM. AB Sixteen patients with cystic fibrosis were treated with conventional physiotherapy aided by an assistant. The results were compared with those produced by physiotherapy using the forced expiration technique cleared more sputum in less time than conventional physiotherapy. A sputum in less time than conventional physiotherapy. A second study showed that an assistant did not further improve the results obtained by the patient performing the forced expiration technique himself. These findings mean that patients with cystic fibrosis who have had to rely on the help of others for their home treatment may now perform more effective treatment without help. The forced expiration technique might also be helpful for patients with chronic bronchitis, asthma, or bronchiectasis. RF 001 COCHRANE GM BR MED J 2 1181 977 002 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 977 003 THOMPSON BJ NZ J PHYSIOTHERAPY 4 4 973 004 MEAD J J APPL PHYSIOL 22 95 967 005 DAWSON SV J APPL PHYSIOL 43 498 977 006 DE KOCK MA DYNAMIC BRONCHOSCOPY 977 CT 1 NICKERSON BG AM REV RESPIR DIS 122 859 980 2 HODSON ME PRACTITIONER 224 301 980 3 SUTTON PP EUR J RESPIR DIS 63 188 982 4 HARRISON AC NZ MED J 95 270 982 5 ROSSMAN CM AM REV RESPIR DIS 126 131 982 6 SUTTON PP EUR J RESPIR DIS 64 62 983 7 HODSON ME PRACTITIONER 227 1723 983 8 SUTTON PP SEM RESPIR MED 5 353 984 9 HODSON ME POSTGRAD MED J 60 225 984 10 FALK M EUR J RESPIR DIS 65 423 984 11 DEBOECK C AM REV RESPIR DIS 129 182 984 12 MACKENZIE CF CRIT CARE MED 13 483 985 13 MEARNS MB ARCH DIS CHILD 60 272 985 14 SUTTON PP EUR J RESPIR DIS 66 147 985 15 KIRILOFF LH CHEST 88 436 985 16 MAZZOCCO MC CHEST 88 360 985 17 COCHRANE GM BR MED J 290 1026 985 18 OBERWALDNER B PEDIATR PULMONOL 2 358 986 19 FALING LJ CLIN CHEST MED 7 599 986 20 HOFMEYR JL THORAX 41 951 986 21 KIM CS J APPL PHYSIOL 60 908 986 22 BLOMQUIST M ARCH DIS CHILD 61 362 986 23 TYRRELL JC ARCH DIS CHILD 61 598 986 24 DINWIDDIE R J ROY SOC MED 79 6 986 25 CURRIE DC BR J DIS CHEST 80 249 986 26 WEBBER BA BR J DIS CHEST 80 353 986 27 SUTTON PP THORAX 42 159 987 28 KIM CS J APPL PHYSIOL 62 959 987 29 ANDREASSON B ACTA PAEDIATR SCAND 76 70 987 PN 79056 RN 01036 AN 79146187 AU Chapman-J-A. Goodall-J. TI Dying children need help too. SO Br-Med-J. 1979 Mar 3. 1(6163). P 593-4. MJ CHILD-CARE. TERMINAL-CARE. MN CASE-REPORT. CHILD-CARE: mt, st. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: th. FEMALE. HUMAN. TERMINAL-CARE: mt, st. EX The control of symptoms in dying children is often sadly neglected. This neglect is reflected in a review of the case notes of two children who died of cystic fibrosis within three years of each other. The girls were under the care of the same consultant, who in the interval between the two cases was introduced to the concept of proper terminal care for children. These two cases show that illness can cause physical, emotional, and spiritual pain to family and staff. In retrospect these areas of distress were not clearly defined in either case and opportunities to alleviate suffering were missed. The principles of controlling symptoms need to be remembered as much during distressing treatment as during the distress of dying. RF 001 BRETT EM DEVELOP MED CHILD NEUROL 12 655 970 002 SHAFFER D IN: RUTTER M 977 003 TWYCROSS RG IN: SAUNDERS CM 978 PN 79057 RN 01037 AN 79125271 AU Bradley-J-A. Axon-A-T. Hill-G-L. TI Nocturnal elemental diet for retarded growth in a patient with cystic fibrosis. SO Br-Med-J. 1979 Jan 20. 1(6157). P 167. MJ CYSTIC-FIBROSIS: co. FOOD-FORMULATED. NUTRITION-DISORDERS: dh. MN ADOLESCENCE. BODY-WEIGHT. CASE-REPORT. FEMALE. HUMAN. INTUBATION-GASTROINTESTINAL. NUTRITION-DISORDERS: et. POTASSIUM: me. EX The combination of chronic pulmonary sepsis and pancreatic insufficiency in children with cystic fibrosis may lead to malnutrition. We describe our experience of the long-term use of continuous nocturnal infusion of elemental diet in a patient with cystic fibrosis. Our patient tolerated the long-term nocturnal elemental diet well, and it enabled her to continue normal everyday activity and eat normal meals. The periods of nutritional support coincided with periods of increased body weight and total body potassium, indicating that the lean body mass was increased. We think that these results show a need for further studies of home treatment in children with cystic fibrosis. RF 001 WEIHOFEN DM J AM DIET ASSOC 54 206 969 002 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 003 COUCH R FED PROC 19 13 960 004 ANDRASSY RJ SURGERY 82 205 977 005 BERRY HK AM J DIS CHILD 129 165 975 CT 1 POULIN E CAN J SURG 25 584 982 2 WORTHEN DB DRUG INTEL CLIN PHARM 18 794 984 3 BERTRAND JM J PEDIATR 104 41 984 4 SOUTTER VL CLIN GASTROENTEROL 15 137 986 5 MOORE MC AM J CLIN NUTR 44 33 986 PN 79058 RN 01038 AN 80043560 AU Zapletal-A. Houstek-J. Samanek-M. Vavrova-V. Srajer-J. TI Lung function abnormalities in cystic fibrosis and changes during growth. SO Bull-Eur-Physiopathol-Respir. 1979 Jul-Aug. 15(4). P 575-92. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. AIRWAY-RESISTANCE. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. FEMALE. FOLLOW-UP-STUDIES. HUMAN. LUNG-COMPLIANCE. LUNG-VOLUME-MEASUREMENTS. LUNG: gd. MALE. AB Static lung volumes (VC, TLC, FRC, RV), elastic recoil pressure of the lungs (Pst(1) at 100, 90, and 60% of TLC), static lung compliance [Cst(1)], specific airway conductance at FRC level (Gaw/TGVex), forced expiratory volume in the first second (FEV1), maximal expiratory flows (Vmax.) at 25 and 50% of VC and at 60% of TLC, and "upstream" airway conductance (Gus) at 60% of TLC were studied in 28 patients with cystic fibrosis, 5 to 25 years old, over a period of 1 to 5 years. The data were compared individually with normal values, related to body height in the form of regression equations and expressed in percentage of predicted values. From the indices assessing airway function, Vmax. at low lung volumes, Gus at 60% TLC, RV and RV/TLC were the functional parameters most consistently abnormal. Among the indices characterizing lung elasticity, Pst(1) at 60% TLC was the most abnormal. Generally, the values of the majority of lung function indices [VC, TLC, FRC, Pst(1)] declined during growth. Vmax. at all lung volumes and Gus at 60% TLC did not deteriorate with growth probably due to the great abnormality of these parameters already in young patients. It was also observed that lung function did not change significantly over a period of 1 to 5 years corresponding to a 10 cm increase in body height. However, over that period, lung function improved in some of the subjects, did not change in over 50% of the cases and deteriorated in the others. RF 001 BEIER FR AM REV RESPIR DIS 94 430 966 002 COOK CD PEDIATRICS 24 181 959 003 COREY M AM REV RESPIR DIS 114 1085 976 004 DEMUTH GR AM J DIS CHILD 103 129 962 005 FEATHERBY EA AM REV RESPIR DIS 102 737 970 006 GOLDRING RM J PEDIATR 65 501 964 007 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 008 HOUSTEK J PRAKT LEKAR 49 216 969 009 HOUSTEK J VNITR LEK 16 449 970 010 LOUGH MD PEDIATRIC RESPIRATORY THERAPY 974 011 MANSELL A AM REV RESPIR DIS 109 190 974 012 MEAD J J APPL PHYSIOL 22 95 967 013 MELLINS RB PEDIATRICS 41 560 968 014 NEUBURGER N AM REV RESPIR DIS 114 753 976 015 SAMANEK M ACTA PAEDIATR SCAND 60 149 971 016 STERN RC J PEDIATR 89 406 976 017 VAVROVA V CESK PEDIATR 28 528 973 018 WILLIAMS HE RESPIRATORY ILLNESS IN CHILDR 975 019 ZAPLETAL A PEDIATRICS 48 64 971 020 ZAPLETAL A CESK PEDIATR 31 532 976 021 ZAPLETAL A J APPL PHYSIOL 40 953 976 022 ZAPLETAL A Z ERKR ATMUNGSORGANE 149 343 977 023 ZAPLETAL A CAS LEK CES 113 1225 974 024 ZAPLETAL A CESK PEDIATR 32 513 977 CT 1 GOTZ M EUR J RESPIR DIS 61 122 980 2 SOLYMAR L EUR J RESPIR DIS 61 275 980 3 DESWINIARSKI R BULL EUR PHYSIOPATH RESP 18 39 982 4 ZAPLETAL A EUR J RESPIR DIS 64 426 983 5 LEUPOLD W Z KLIN MED 40 661 985 6 ZACH MS AM REV RESPIR DIS 131 537 985 7 WIESEMANN HG MONATSSCHR KINDERHEILKD 133 726 985 8 OBERWALDNER B PEDIATR PULMONOL 2 358 986 9 ANDREASSON B ACTA PAEDIATR SCAND 76 70 987 PN 79059 RN 01039 AN 80021639 AU Coates-A-L. Boyce-P. Muller-D. Mearns-M. Godfrey-S. TI The role of nutritional status, airway obstruction, hypoxia and abnormalities in serum lipid composition in limiting exercise tolerance in children with cystic fibrosis. SO Bull-Eur-Physiopathol-Respir. 1979 Mar-Apr. 15(2). P 341-2. MJ AIRWAY-OBSTRUCTION: bl. ANOXIA: bl. CYSTIC-FIBROSIS: bl. EXERTION. LIPIDS: bl. NUTRITION-DISORDERS: bl. MN CARBON-DIOXIDE: bl. CHILD. HEART-RATE. HUMAN. LUNG-VOLUME-MEASUREMENTS. OXYGEN: bl. EX This study investigated the roles of hypoxia, pulmonary mechanics, nutritional status and serum fatty acid composition in limiting exercise tolerance in cystic fibrosis. Twenty children with CF of carrying severity performed two progressive exercise tests on a cycle ergometer, once while breathing air, once while breathing O2. The children with the lower maximal mid-expiratory flow rates tended to have very little respiratory reserve at the final work load. We conclude that nutritional status and airway obstruction are closely correlated with exercise tolerance in CF and that, unlike the adult with chronic obstructive pulmonary disease, exercise limiting dyspnoea occurs in the presence of normal end-tidal CO2. CT 1 LEUPOLD W Z KLIN MED 40 661 985 PN 79060 RN 01040 AN 79234522 AU Sams-C-A. TI One breath at a time: a family teaching program for children with cystic fibrosis. SO Can-Nurse. 1979 Sep. 75(8). P 20-3. MJ CYSTIC-FIBROSIS: rh. PARENTS: ed. PATIENTS: ed. PATIENT-EDUCATION. MN ADOLESCENCE. ADULT. BREATHING-EXERCISES. CASE-REPORT. CHILD. CHILD-PRESCHOOL. FAMILY. FEMALE. HUMAN. MALE. RESPIRATORY-THERAPY. EX We have found patients who have cystic fibrosis to be very special people; they have to overcome apparently insurmountable odds. Our teaching program comes from a basic belief that with support, encouragement and affection, information and a focus on the positive aspects of life, a person with CF can live well, even if only one breath at a time. RF 001 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 001 LEFEBVRE A DISSERTATION 974 002 BURNETTE BA AM J NURS 75 1986 975 002 STEINHAUER PD PEDIATR CLIN NORTH AM 21 825 974 003 MCCOLLUM AT COPING WITH PROLONG HLTH IMP 975 004 FAKKEM L AM J NURS 59 1269 959 005 LEONARD CO N ENGL J MED 287 433 972 PN 79061 RN 01041 AN 80022554 AU Berger-H-J. Zaret-B-L. TI Noninvasive radionuclide assessment of right ventricular performance in man. SO Cardiovasc-Clin. 1979. 10(2). P 91-104. MJ HEART-VENTRICLE: ri. RADIONUCLIDE-IMAGING: mt. MN ADULT. CYSTIC-FIBROSIS: pp, ri. DIAGNOSIS-DIFFERENTIAL. HEART-VENTRICLE: pp. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: pp, ri. MYOCARDIAL-INFARCTION: pp, ri. RESPIRATION. STROKE-VOLUME. SUPPORT-U-S-GOVT-P-H-S. EX This chapter focuses on the application of radionuclide angiocardiography to assessment of right ventricular ejection fraction and the relationship between right and left heart performance demonstrated by this technique. Prior to discussion of the clinical applications, the functional geometry of the two ventricles will be reviewed. In addition, the first-pass radiotracer techniques will be compared to other radionuclide imaging approaches of the right heart. First-pass radionuclide angiocardiographic techniques are now suitable for clinical application. They have been validated and standardized and can be relied upon for accurate, reproducible noninvasive assessment of right and left ventricular performance. They already have been applied to the study of patients with cardiopulmonary disease; future directions will include earlier evaluation of myocardial infarction, application to children and adults with a variety of congenital cardiac abnormalities (primarily those affecting the right heart), and assessment of the effects of physiologic stress upon right ventricular function. With this background valuable clinical and investigative insights should become available during the coming years. RF 001 STARR I AM HEART J 26 291 943 002 BURROWS B N ENGL J MED 286 912 972 003 PONTOPPIDAN H N ENGL J MED 287 690 972 004 COHN J AM J CARDIOL 33 209 974 005 BERGER HJ AM J CARDIOL 41 897 978 006 ELLIS J CHEST SUPPL 71 281 977 007 FISHER EA AM J CARDIOL 36 67 975 008 ARCILLA R CHEST 60 446 971 009 GRAHAM TP JR CIRCULATION 47 144 973 010 GRAHAM TP JR CIRCULATION 51 881 975 011 GENTZLER R CIRCULATION 50 324 974 012 MARSHALL RC CIRCULATION 56 820 977 013 RUSHMER RF CARDIOVASCULAR DYNAMICS 89 976 014 FERLINZ J CIRCULATION 52 608 975 015 JAFFE C CURR PROB RADIOL 4 1 974 016 BLUMGART HL J CLIN INVEST 4 399 927 017 PRINZMETAL M SCIENCE 108 340 948 018 VAN DYKE D J NUCL MED 13 585 972 019 WEBER P J NUCL MED 13 815 972 020 SHELBERT H CIRCULATION 51 902 975 021 STEELE P AM J CARDIOL 34 179 974 022 STEELE P AM J CARDIOL 37 388 976 023 ELLIS J CHEST 69 575 976 024 STEELE P CHEST 70 51 976 025 TOBINICK E CIRCULATION SUPPL 56 140 977 026 MARSHALL RC AM J CARDIOL 41 531 978 027 BERGER HJ CLIN RES 25 209A 977 028 BORKOWSKI H CIRCULATION SUPPL 56 198 977 029 STEELE P AM J MED 59 21 975 030 KLINE L CHEST 72 5 977 031 MATTHAY R AM HEART J 92 730 976 032 MATTHAY R CHEST 72 407 977 033 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 034 YATER WM AM HEART J 36 683 948 035 ERHARDT L ACTA MED SCAND SUPPL 560 1 974 036 SHARPE N CIRCULATION SUPPL 2 54 76 976 037 BUSEMAN SOKOLE E CIRCULATION SUPPL 3 56 62 977 038 REDUTO L ANN INTERN MED 89 441 978 039 RIGO P CIRCULATION 52 268 975 CT 1 ARVAN S VASC SURG 18 72 984 2 DETRANO R J AM COLL CARDIOL 5 1377 985 3 DEC GW N ENGL J MED 312 885 985 PN 79062 RN 01042 AN 79126800 AU Hoogkamp-Korstanje-J-A. Westerdaal-N-A. TI In vitro susceptibility of pseudomonas to four beta-lactamantibiotics (ampicillin, cephalothin, carbenicillin, piperacillin), to four aminoglycosides (kanamycin, amikacin, gentamicin, tobramycin) and to colimycin. SO Chemotherapy. 1979. 25(1). P 48-53. MJ AMINOGLYCOSIDES: pd. CEPHALOTHIN: pd. COLISTIN: pd. PENICILLINS: pd. PSEUDOMONAS: de. MN AMIKACIN: pd. AMPICILLIN: pd. CARBENICILLIN: pd. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: mi. DRUG-RESISTANCE-MICROBIAL. GENTAMICINS: pd. HUMAN. KANAMYCIN: pd. MICROBIAL-SENSITIVITY-TESTS. SPUTUM: mi. TOBRAMYCIN: pd. AB Of 97 well-defined strains of Pseudomonas, isolated from sputum of patients with cystic fibrosis (CF), the minimum inhibitory concentration (MIC) of several antibiotics was determined with a broth dilution method. The majority of the strains were resistant to ampicillin and cephalothin, moderately susceptible to carbenicillin (70% to 100 microgram/ml) and highly susceptible to piperacillin (100% to 25 microgram/ml, 88% to 6.25 microgram and 60% to 3.12 microgram/ml). If the pharmacological properties of piperacillin are comparable with those of carbenicillin, it can be expected that the sputum level of this drug will be adequate to treat Pseudomonas pulmonary infections. At the lowest concentration tested (0.78 microgram/ml) 3% of the strains were susceptible to kanamycin, 85,5% to amikacin, 95% to gentamicin, 98% of tobramycin, and 80% to colimycin. With regard to clinically attainable concentrations, 98.9% of the strains were susceptible to gentamicin and tobramycin 97.9% to amikacin, 96.9% to colimycin, 88.6% to piperacillin, 38% to carbenicillin, 25.7% to kanamycin, 12.3% to ampicillin, and 1% to cephalothin. RF 001 BLAIR JE MANUAL OF CLINICAL MICROBIOLO 970 002 BOXERBAUM B J INFECT DIS SUPPL 122 59 970 003 BOXERBAUM B J INFECT DIS SUPPL 124 293 971 004 COWAN ST MANUAL FOR THE IDENTIFICATION 973 005 ERICSSON HM ACTA PATH MICROBIOL SCAND S217 971 006 MARKS MI J PEDIATR 79 822 971 007 MONROE PW APPL MICROBIOL 18 214 969 008 PENNINGTON JE ANTIMICROB AGENTS CHEMOTHER 4 299 973 009 PHAIR JP AM J DIS CHILD 120 22 970 010 REYNOLDS AV BR MED J 3 778 974 011 SAGGERS BA ARCH DIS CHILD 43 404 968 012 WOOD RE PSEUDOMONAS INFECTION IN CF P 975 013 ZIERDT CH J CLIN MICROBIOL 1 521 975 CT 1 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 8 101 981 2 HOOGKAMPKORSTANJE JAA INFECTION 10 S257 982 3 HOOGKAMPKORSTANJE JAA J ANTIMICROB CHEMOTHER 12 175 983 4 SEGNI G CURR THER RES CLIN EXP 33 265 983 PN 79063 RN 01043 AN 79190706 AU Allen-H-D. Taussig-L-M. Gaines-J-A. Sahn-D-J. Goldberg-S-J. TI Echocardiographic profiles of the long-term cardiac changes in cystic fibrosis. SO Chest. 1979 Apr. 75(4). P 428-33. MJ CYSTIC-FIBROSIS: co. ECHOCARDIOGRAPHY. HEART-DISEASES: di. MN ADOLESCENCE. ADULT. AORTIC-DISEASES: di, et. CHILD. CHILD-PRESCHOOL. FEMALE. HEART-DISEASES: et. HUMAN. INFANT. MALE. PROGNOSIS. SUPPORT-U-S-GOVT-P-H-S. AB In this echocardiographic study, assessment of the heart in children with cystic fibrosis has shown that changes occur in not only the right ventricle but also in the left ventricle and aorta, as compared with normal. Echocardiograms could be successfully performed in 34 of 37 patients. The thickness of the right ventricular anterior wall and the dimension of the right ventricular cavity were abnormal, even in patients with mild disease (National Institutes of Health [NIH] score for severity of disease of 85 or greater). Larger, older children with lower NIH score had disproportionately larger right ventricular anterior walls and cavities. The thickness of the septal wall, the thickness of the left ventricular posterior wall, and the aortic dimensions were increased in those with severe disease. The dimensions of the left ventricular cavity for the population were slightly but significantly smaller than normal in systole and in diastole throughout the course of the disease. This study demonstrates that echocardiography is an effective noninvasive means of assessing the long-term changes in children with cystic fibrosis. These changes occur in both sides of the heart and appear to worsen as the disease progresses. RF 001 ANON CIRCULATION 27 594 963 002 LIEBMAN J CHEST 63 218 973 003 RYLAND D THORAX 30 285 975 004 SIASSI B J PEDIATR 78 794 971 005 MOSS AJ J PEDIATR 67 797 965 006 SHWACHMAN H AM J DIS CHILD 96 6 958 007 WARSHAWSKY JK IN: KULCZYCKI LL 16 974 008 GEWITZ M AM J DIS CHILD 131 275 977 009 ROSENTHAL A PEDIATR CLIN NORTH AM 23 327 976 010 TAUSSIG LM J PEDIATR 82 380 973 011 GOLDBERG SJ PEDIATRIC AND ADOLESCENT ECHO 975 012 ALLEN HD WORKBOOK IN PEDIATRIC ECHOCAR 978 013 EPSTEIN ML CIRCULATION 51 1124 975 014 GRIFFITH JM IEEE TRANS BIOMED ENG 22 337 975 015 ROYCE SW PEDIATRICS 8 255 951 016 LARTER WE CIRCULATION 53 19 976 017 WEYMAN AE CIRCULATION 54 179 976 018 STEELE P AM J MED 59 21 975 019 MITCHELL RS AM REV RESPIR DIS 114 147 976 020 WILLIAMS JF JR J CLIN INVEST 47 1143 968 021 KOUNTZ WB AM HEART J 11 163 936 022 SCOTT RW AM HEART J 22 56 941 023 MICHELSON N DIS CHEST 38 435 960 024 FLUCK DC BR HEART J 28 92 966 025 RAO BS AM J MED 45 229 968 026 BAUM GL N ENGL J MED 285 361 971 027 FULTON RM BR HEART J 14 413 952 CT 1 LESTER LA J PEDIATR 97 742 980 2 FRANCIS PWJ AM J DIS CHILD 134 734 980 3 JACOBSTEIN MD CHEST 80 399 981 4 MOSS AJ PEDIATRICS 70 728 982 5 TEPPER RS CHEST 84 388 983 6 CHERON G ACTA PAEDIATR SCAND 73 697 984 7 MICHAEL JR AM REV RESPIR DIS 130 516 984 8 LESTER LA SEM RESPIR MED 6 285 985 PN 79064 RN 01044 AN 79168494 AU Lieberman-J. Kaneshiro-W. TI Lymphocytic acid-hydrolases and response to mitogens in cystic fibrosis. SO Chest. 1979 Feb. 75(2 Suppl). P 220-3. MJ ACID-PHOSPHATASE: bl. CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: bl. LYMPHOCYTES: en. MITOGENS: pd. MN ACETYLGLUCOSAMINIDASE: bl. AMYLASES: bl. BETA-GALACTOSIDASES: bl. BLOOD-PROTEINS: an. CELLS-CULTURED. COMPARATIVE-STUDY. CULTURE-MEDIA. CYSTIC-FIBROSIS: fg. FUCOSIDASE: bl. GLUCOSIDASES: bl. GLUCURONIDASE: bl. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MANNOSIDASES: bl. STIMULATION-CHEMICAL. SUPPORT-U-S-GOVT-NON-P-H-S. SUPPORT-U-S-GOVT-P-H-S. XYLOSIDASES: bl. EX In the present study, we compared the levels of a series of cultured lymphocytic acid-hydrolase enzymes in cystic fibrosis homozygotes, heterozygotes and controls; compared the effects of three different mitogens on these levels; and studied the effect of CF vs control serum in the culture medium. Our observations provide an entirely new interpretation for the apparent changes occurring in lymphocytic enzymes during culture of lymphocytes with mitogens, and suggest that a serum factor is present in cystic fibrosis serum that mimics mitogens in this regard. Lymphocytic acid-hydrolases and total protein are present in higher concentrations in lymphocytes cultured for 48 hours without mitogen when CF homozygous or heterozygous serum is present in the culture media. This appears to result from a CF serum factor that has mitogen-like activity and prevents loss of cell protein during culture. Responsiveness of the cellular proteins to mitogens is blunted when CF serum is present in the culture media, either because the cell proteins are already maximally stimulated by the CF serum factor, or the factor blocks or modifies the action of mitogens on lymphocyte protein content. This phenomenon is useful for detection of CF heterozygosity, and appears to reflect the genetic abnormality causing cystic fibrosis. RF 001 MARCHI AG HELV PAEDIATR ACTA 28 427 973 002 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 003 ANTONOWICZ I PEDIATR RES 6 803 972 CT 1 SEALE TW ANN CLIN LAB SCI 12 415 982 PN 79065 RN 01045 AN 79190766 AU Weng-T-R. Spence-J-A. Polgar-G. Nyboer-J. TI Measurement of regional lung function by tetrapolar electrical impedance plethysmography. SO Chest. 1979 Jul. 76(1). P 64-9. MJ PLETHYSMOGRAPHY-IMPEDANCE: mt. PULMONARY-CIRCULATION. RESPIRATORY-FUNCTION-TESTS: mt. MN ADOLESCENCE. CASE-REPORT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. ELECTRODES. FEMALE. HEART-DEFECTS-CONGENITAL: pp. HUMAN. MALE. PLETHYSMOGRAPHY-IMPEDANCE: is. PULMONARY-VEINS: ab. RESPIRATORY-FUNCTION-TESTS: is. RESPIRATORY-INSUFFICIENCY: pp. VENTILATION-PERFUSION-RATIO. AB This study demonstrates the clinical feasibility of atraumatically measuring dynamic regional lung function with tetrapolar electrical impedance plethysmography (EIP) in the pediatric age group. A tetrapolar electrode system was used because of its ability to detect local baseline and pulsatile impedance changes, minimal electrode- tissue impedance interference, and uniform current distribution. Studies performed on 4 children 4 to 15 years old with various pulmonary diseases are presented. The information obtained on their regional ventilation and pulsatile perfusion by EIP was found to agree closely with pulmonary ventilation and perfusion scans. In one case, EIP detected pulsatile perfusion abnormalities which were not apparent in the non-pulsatile perfusion scan but were demonstrated by cineangiography. It is concluded that tetrapolar EIP can provide safe, reliable regional information about ventilation and perfusion in diseased lungs, and because of its atraumatic approach, is technically most suitable for use in children. RF 001 OLSSON T ACTA PAEDIATR SCAND SUPPL 20 390 970 002 VAN DE WATER JM CHEST 64 598 973 003 POMERANTZ M ANN SURG 171 686 970 004 LABABIDI Z PEDIATRICS 47 870 971 005 HOON RS BR HEART J 39 61 977 006 DENNISTON JC J APPL PHYSIOL 19 166 964 007 ALLISON RD J APPL PHYSIOL 19 166 964 008 NYBOER J PROC INT CONG OF BIOL 2ND 216 976 009 SPENCE JA PEDIATR RES ABST 11 579 977 010 POLGAR G CF CLUB ABST 4 978 011 NYBOER J PROC INT CONG OF BIOL 2ND 5 976 012 NYBOER J ELECTRICAL IMPEDANCE PLETHYSM 390 970 013 NYBOER J PROC J SOC CLIN INVEST 19 963 940 014 NYBOER J ANN NY ACAD SCI 170 421 970 015 KAUFMAN SS NAT RES COUNC COMM AVIAT 1 944 016 GOLDENSOHN ES J APPL PHYSIOL 14 463 959 017 GEDDES LA PRINCIPLES OF APPL BIOMEDICAL 616 975 018 KUBICEK WG AEROSP MED 37 1208 966 019 NYBOER J HARPER HOSP BULL 22 232 964 020 NYBOER J AM J ROENTG RAD THER NUCL MED 98 497 966 021 KIRA S JAPAN HEART J 11 149 970 022 KAWAKAMI K MED BIOL ENG 11 460 973 023 KAWAKAMI K J RADIOL ELECTROL MED NUCL 54 135 973 024 WELTMAN G ANN BIOMED ENG 1 69 972 025 SCHRAIBMAN IG BR J SURG 63 413 976 CT 1 PENNEY BC CRC CRIT REV BIOMED ENG 13 227 986 2 WENG TR AVIAT SPACE ENVIRON MED 57 449 986 PN 79066 RN 01046 AN 79190761 AU Keens-T-G. Mansell-A. Krastins-I-R. Levison-H. Bryan-A-C. Hyland-R-H. Zamel-N. TI Evaluation of the single-breath diffusing capacity in asthma and cystic fibrosis. SO Chest. 1979 Jul. 76(1). P 41-4. MJ ASTHMA: di. CARBON-MONOXIDE: du. CYSTIC-FIBROSIS: di. PULMONARY-DIFFUSING-CAPACITY. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: pp. ASTHMA: pp. BRONCHODILATOR-AGENTS: du. CHILD. CYSTIC-FIBROSIS: pp. HUMAN. MICROCIRCULATION. PULMONARY-CIRCULATION. AB To explain why the single-breath carbon monoxide diffusing capacity (Dsb) was, on the average, elevated in 163 asthmatic subjects and 175 patients with cystic fibrosis, we simulated this elevation in ten normal subjects by having them perform the test for Dsb through an inspiratory obstruction. This resulted in an 18 percent increase in Dsb corrected for pulmonary volume. Inhalation of a bronchodilator drug was associated with relief of obstruction and a fall in the corrected Dsb in 31 asthmatic subjects but did not change either the obstruction or the corrected Dsb in 17 patients with cystic fibrosis. We suggest that elevated Dsb in asthma and cystic fibrosis is partly due to maximal inspiration against obstructed airways. This requires abnormally negative intrathoracic pressures, increasing the pulmonary capillary blood volume, and, thereby, increasing the Dsb. RF 001 WENG TR AM REV RESPIR DIS 99 719 969 002 OGILVIE CM BR MED J 1 768 968 003 EVANS CC LANCET 1 891 968 004 COTES JE J APPL PHYSIOL 15 372 960 005 STEINER SH J CLIN INVEST 44 1623 965 006 SMITH TC J APPL PHYSIOL 27 826 969 007 OGILVIE CM J CLIN INVEST 36 1 957 008 ROUGHTON FJM J APPL PHYSIOL 11 290 957 009 CAREL RS ISR J MED SCI 9 1535 973 010 PECORA LJ J ALLERGY 37 204 966 011 WAGNER PD J APPL PHYSIOL 31 847 971 012 HYATT RE J APPL PHYSIOL 21 477 966 CT 1 WEINBERGER SE CHEST 78 483 980 2 DAVIES NJH BR J DIS CHEST 76 105 982 3 RUSSELL NJ BR J DIS CHEST 76 35 982 4 OBRODOVICH HM AM REV RESPIR DIS 125 670 982 5 OGILVIE C THORAX 38 5 983 6 COTTON DJ RESPIR PHYSIOL 54 19 983 7 GRAHAM BL AM REV RESPIR DIS 129 403 984 8 COTTON DJ CHEST 87 217 985 9 PAOLETTI P AM REV RESPIR DIS 132 806 985 10 LEBECQUE P CHEST 91 693 987 PN 79067 RN 01047 AN 79126767 AU Hirschfeld-S-S. Fleming-D-G. Doershuk-C. Liebman-J. TI Echocardiographic abnormalities in patients with cystic fibrosis. SO Chest. 1979 Mar. 75(3). P 351-5. MJ CYSTIC-FIBROSIS: pp. ECHOCARDIOGRAPHY. HEART: pp. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FEMALE. HEART-FAILURE-CONGESTIVE: di, et, pp. HEART-VENTRICLE: pp. HUMAN. INFANT. MALE. MYOCARDIAL-CONTRACTION. RESIDUAL-VOLUME. SYSTOLE. VITAL-CAPACITY. AB An echographic study was undertaken to evaluate left (LV) and right ventricular (RV) function in 30 patients with cystic fibrosis. Echographic recording of the pulmonary and aortic valve echogram permitted measurement of the phases of right and left ventricular systole. The ratio of the LV preejection period/LV ejection time (LPEP/LVET) and shortening of the LV internal dimension %SID was employed to reflect LV function, while RV preejection period/RV ejection time (RPEP/RVET) has excellent correlation with pulmonary artery diastolic pressure. RPEP/RVET and two other echographic measurements, right ventricular wall (RVW) and internal dimension (RVD) were compared with pulmonary function tests and clinical scores. RPEP/RVET correlated well with percent vital capacity(%VC), r = -0.73, percent residual volume (%RVol) r = +0.72, and clinical score, r = -0.77. Multilinear regression of RPEP/RVET, RVD, and RVW improved correlation for %VC (r = -0.80), %RVol, r = +0.82, and clinical score, r = -0.84. Patients in overt right heart failure exhibited elevated RPEP/RVET (mean = 0.48) when compared to patients not in right heart failure (mean = .33). Marked diminution of LV function was present in two patients. A variety of cardiovascular abnormalities were demonstrated echographically and were valuable in assessing the degree of cardiac involvement in patients with cystic fibrosis. RF 001 LIEBMAN J IN: MANGOS JA 41 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 SIASSI B J PEDIATR 78 794 971 004 GOLDRING RM J PEDIATR 65 501 964 005 THOMAS AJ BR HEART J 34 653 972 006 LIEBMAN J CIRCULATION 35 552 967 007 ROSENTHAL A PEDIATR CLIN NORTH AM 23 327 976 008 RYSSING E ACTA PAEDIATR SCAND 66 753 977 009 GEWITZ M AM J DIS CHILD 131 275 977 010 HIRSCHFELD S CIRCULATION 51 304 975 011 HIRSCHFELD S CIRCULATION 52 641 975 012 RIGGS T CIRCULATION 57 939 978 013 WEISSLER AM IN: YU PN 1 155 972 014 FORTUIN NJ CIRCULATION 46 26 972 015 HELLIESEN PJ PEDIATRICS 22 80 958 016 DUBOIS AB J CLIN INVEST 35 322 956 017 SHWACHMAN H AM J DIS CHILD 96 6 958 018 GALANT SP PEDIATRICS 61 46 978 019 MEYER RA ECHOCARDIOGRAPHY 257 977 020 GUTGESELL HP CIRCULATION 56 457 977 CT 1 MATTHAY RA BR HEART J 43 474 980 2 LESTER LA J PEDIATR 97 742 980 3 FRANCIS PWJ AM J DIS CHILD 134 734 980 4 BERGER HJ AM J CARDIOL 47 950 981 5 JACOBSTEIN MD CHEST 80 399 981 6 NEWTH CJL AM REV RESPIR DIS 124 463 981 7 MOSS AJ PEDIATRICS 70 728 982 8 HIRSCHFELD SS CHEST 82 524 982 9 MATTHAY RA CLIN CHEST MED 4 269 983 10 MATTHAY RA CRIT CARE MED 11 329 983 11 MACDONALD NE J PEDIATR 103 985 983 12 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 13 BENSON LN AM REV RESPIR DIS 130 987 984 14 MOSKOWITZ WB PEDIATR PHARMACOL 5 139 985 15 LESTER LA SEM RESPIR MED 6 285 985 16 PANIDIS IP J AM COLL CARDIOL 6 701 985 17 PIEPSZ A PEDIATR PULMONOL 3 24 987 PN 79068 RN 01048 AN 80090384 AU Price-J-F. Weller-P-H. Harper-S-A. Matthew-D-J. TI Response to bronchial provocation and exercise in children with cystic fibrosis. SO Clin-Allergy. 1979 Nov. 9(6). P 563-70. MJ ASTHMA: et. BRONCHIAL-PROVOCATION-TESTS. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ASPERGILLUS-FUMIGATUS: im. ASTHMA: di. CHILD. EXERTION. FEMALE. HUMAN. MALE. SKIN-TESTS. AB Ten of fifteen cystic fibrosis children with positive skin prick tests to common antigens gave an immediate bronchial reaction to the antigen inhaled, five of them also gave a late reaction; however only one gave a history of asthma. The antigen most commonly eliciting a positive skin reaction in cystic fibrosis patients is Aspergillus fumigatus. In six children tested to this antigen the bronchial response varied, two were negative, one gave an immediate reaction and three gave a dual (immediate and late) reaction. None of the children showed the characteristic pattern of response to exercise seen in asthmatic patients, an initial rise in Peak Expiratory Flow Rate followed by a fall of greater than 14% below the resting level. Two patients showed an abnormal rise in Peak Expiratory Flow Rate during exercise, a pattern described previously in cystic fibrosis. The results suggest that bronchial allergy, immediate or late does completely explain susceptibility to asthma, and that other factors including perhaps the type of bronchial reactivity shown by bronchoconstriction after exercise may be required. However the majority of the children tested had bronchial allergy and anti- allergy therapy such as inhaled sodium cromoglycate may have a place in the management of selected patients with cystic fibrosis. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 ANDERSON SD THESIS 972 003 CHRISPIN AR PEDIATR RADIOL 2 101 974 004 COGSWELL JJ BR J DIS CHEST 69 177 975 005 COGSWELL JJ BR J DIS CHEST 69 177 975 006 DAY G ARCH DIS CHILD 48 355 973 007 GODFREY RC CLIN ALLERGY 6 79 976 008 GODFREY S EXERCISE TESTING IN CHILDREN 974 009 KONIG P ARCH DIS CHILD 48 513 973 010 LANDAU LI J PEDIATR 82 863 973 011 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 012 MEARNS MB LANCET 1 538 967 013 MEARNS MB THORAX 20 385 965 014 ORES CN CF CLUB ABST 16 48 975 015 PRICE JF PROC INT CF CONG 7TH 253 976 016 RACHELEFSKY GS AM J DIS CHILD 128 355 974 017 SILVERMAN M ARCH DIS CHILD 53 873 978 018 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 019 WARNER JO ARCH DIS CHILD 51 507 976 020 WARNER JO ARCH DIS CHILD 51 507 976 021 WARNER JO LANCET 1 990 976 022 WARREN CPW CLIN ALLERGY 5 1 975 CT 1 WONNE R CLIN ALLERGY 15 455 985 PN 79069 RN 01049 AN 79190129 AU Ganier-M. Lieberman-P. TI IgE mediated hypersensitivity to pancreatic extract (PE) in parents of cystic fibrosis (CF) children. SO Clin-Allergy. 1979 Mar. 9(2). P 125-32. MJ CYSTIC-FIBROSIS: co. DRUG-HYPERSENSITIVITY: im. HYPERSENSITIVITY-IMMEDIATE: ci. PANCREATIC-EXTRACTS: ae. PARENTS. MN ADULT. ANTIGENIC-DETERMINANTS. CASE-REPORT. CHILD. CHILD-PRESCHOOL. DRUG-HYPERSENSITIVITY: co. FEMALE. HISTAMINE-LIBERATION. HUMAN. HYPERSENSITIVITY-IMMEDIATE: co. MIDDLE-AGE. IMMUNIZATION-PASSIVE. RADIOALLERGOSORBENT-TEST. SKIN-TESTS. AB On exposure to pancreatic extract (PE), four parents of cystic fibrosis (CF) children developed immediate hypersensitivity-like symptoms: i.e. rhinoconjunctivitis, asthma, and/or anaphylaxis. IgE to PE was demonstrated in the subjects by skin testing, leucocyte histamine release and radioallergosorbent test (RAST). No serum precipitating antibodies were found. Bronchial challenge caused an immediate asthmatic response. No delayed asthmatic response or hypersensitivity pneumonitis-like reaction occurred. The responsible antigen for PE induced asthma is unknown; trypsin failed to inhibit PE-RAST and is therefore not responsible in these subjects. Caution should be exercised in using PE for skin testing and bronchial challenge in subjects with suspected hypersensitivity to PE. Certain measures were found useful in preventing the occurrence of symptoms in the four subjects. RF 001 ABERNATHY RS PEDIATRICS 56 141 975 002 ALLAN JD CLIN ALLERGY 5 255 975 003 BATES DV J CANADA 18 211 962 004 BERGNER A PEDIATRICS 55 814 975 005 BERNSTEIN IL J ALLERGY CLIN IMMUNOL 57 141 976 006 BOOTH BH III IN: PATTERSON R 74 972 007 COLTEN HR N ENGL J MED 292 1050 975 008 DOLAN TF JR AM REV RESPIR DIS 110 812 974 009 HILL D MED J AUST 2 553 975 010 KOPEL FB GASTROENTEROLOGY 62 483 972 011 KULCZYCKI LL JAMA 175 358 961 012 MURPHY RLH JR IN: WEISS EB 517 976 013 NAKAMURA S J ASTHMA RES 10 37 972 014 OUCHTERLONY O HANDBOOK OF IMMUNODIFFUSION A 968 015 PEPYS J CLIN ALLERGY 2 225 972 016 PRAUSNITZ C ZENTRALBL BAKTERIOL 86 160 921 017 PRUZANSKY JJ J ALLERGY 38 315 966 018 RACHELEFSKY GS AM J DIS CHILD 128 355 974 019 ROBERTSON DG J ALLERGY CLIN IMMUNOL 54 244 974 020 SAKALA A LANCET 1 193 977 021 SCHLEUTER D IN: STOLLERMAN G 153 972 022 SHORE PA J PHARMACOL EXP THER 127 182 959 023 TWAROG FJ J ALLERGY CLIN IMMUNOL 59 35 977 024 WIDE L LANCET 2 1105 967 025 WOOD RE AM REV RESPIR DIS 113 833 976 CT 1 MATHEWS KP AFR J CLIN EXP IMMUNOL 3 69 982 2 BAUR X DTSCH MED WSCHR 109 257 984 3 WIESSMANN KJ EUR J RESPIR DIS 66 13 985 4 WARREN CPW AM J MED 81 939 986 5 DAVIS PB THORAX 42 120 987 PN 79070 RN 01050 AN 79211680 AU Kutty-K-M. Guha-A-K. Chandra-R-K. Vaze-D. TI A study of erythrocyte fatty acids, adenosinetriphosphatase and acetylcholinesterase in cystic fibrosis. SO Clin-Biochem. 1979 Jun. 12(3). P 98-9. MJ ACETYLCHOLINESTERASE: bl. ADENOSINE-TRIPHOSPHATASE: bl. CYSTIC-FIBROSIS: bl. ERYTHROCYTES: me. FATTY-ACIDS-UNSATURATED: bl. MN ARACHIDONIC-ACIDS: bl. HUMAN. LINOLEIC-ACIDS: bl. AB 1. The proportions of both saturated and polyunsaturated fatty acids were measured in the erythrocytes in Cystic Fibrosis (CF) patients along with two membrane bound enzymes ATPase and acetylcholinesterase. Linoleic acid was found to be significantly decreased and arachidonic acid increased in CF patients. The proportion of saturated fatty acids were not significantly different from the controls. Only adenosinetriphosphatase activity was found to be reduced and not acetylcholinesterase in CF patients. RF 001 LEHINGER AL MOLECULAR BASIS OF CELL 660 975 002 ROTHFIELD LI STRUCTURE AND FUNCTION OF BIO 196 971 003 LEE JB PERSPECTIVES ON THE PROSTAGLA 8 973 004 BONELLI EJ LIPID ANALYSIS BY GAS CHROMAT 968 005 DUNHAM ET J PHYSIOL (LOND) 156 274 961 007 MCEVOY FA LANCET 2 236 975 008 BALFE JW SCIENCE 162 689 968 CT 1 HUBBARD VS CLIN CHIM ACTA 102 115 980 2 WARWICK WJ AM J MED TECHNOLOGY 48 539 982 PN 79071 RN 01051 AN 80002169 AU Chalmers-R-A. Valman-H-B. Liberman-M-M. TI Measurement of 4-hydroxyphenylacetic aciduria as a screening test for small-bowel disease. SO Clin-Chem. 1979 Oct. 25(10). P 1791-4. MJ INTESTINAL-DISEASES: di. MANDELIC-ACIDS: ur. MN ADOLESCENCE. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: ur. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-DISEASES: ur. MASS-SCREENING. REFERENCE-VALUES. AB We evaluted measurement of urinary 4-hydroxyphenyl acetic acid as a potential screening method for small-bowel disease and bacterial overgrowth syndromes in 360 unselected acutely ill infants and children. Control data were obtained on 120 healthy children, ages 1.5 to 15 years, from a general medical practice, 48 healthy infants, ages one to five years, from local day nurseries, and 150 healthy babies, ages less than one to eight days. Comparative data were from 300 acutely ill hospitalized babies and children, ranging in age from less than one day to 15 years and without clinical evidence for small- bowel disease and bacterial overgrowth syndrome. No false-negative results and only 2% false-positive results were observed. Among the 10 patients whose urinary excretion of the analyte was considered to be abnormal were patients with Giardia lamblia infestation, ileal resection with blind loop, and other diseases of the small intestine associated with bacterial overgrowth. We conclude that measurement of 4-hydroxyphenylacetic acid excretion is useful in screening for such diseases. RF 001 GARRETTSON LK AM J CLIN PATHOL 55 318 971 002 GIBBONS ISE LANCET 1 877 967 003 GJESSING LR LANCET 2 47 967 004 VAN DER HEIDEN C CLIN CHIM ACTA 31 133 971 005 VAN DER HEIDEN C CLIN CHIM ACTA 34 289 971 006 CHALMERS RA ANN CLIN BIOCHEM 14 149 977 007 CHALMERS RA CLIN CHEM 22 1292 976 008 CHALMERS RA ANALYST 97 958 972 009 HEALY MJR J CHROMATOGR 87 365 973 010 LAWSON AM CLIN CHEM 22 1283 976 011 FELLMAN JH CLIN BIOCHEM 10 171 977 012 BORUD O ACTA PHARMACOL TOXICOL 30 185 971 013 AMENT ME PEDIATR CLIN NORTH AM 22 807 975 014 BURKE JA AM J DIS CHILD 129 1304 975 015 CHALLACOMBE DN AM J DIS CHILD 128 719 974 016 GORBACH S IN: DIETSCHY JM 137 976 017 AARBAKKE J SCAND J GASTROENTEROL 11 409 976 CT 1 WATTS RWE J MENT DEFIC RES 24 257 980 2 WILLIAMS DA J SMALL ANIMAL PRACT 22 263 981 3 GRACEY M J PEDIATR GASTROENTEROL NUTR 1 13 982 4 BURROWS CF J AM VET MED ASSOC 183 318 983 5 GRACEY M NAHRUNG 28 659 984 6 BYRD DJ KLIN PAEDIATR 196 298 984 7 HAAN E EUR J PEDIATR 144 63 985 PN 79072 RN 01052 AN 79105836 AU Shwachman-H. Mohmoodian-A. TI Quality of sweat test performance in the diagnosis of cystic fibrosis. SO Clin-Chem. 1979 Jan. 25(1). P 158-61. MJ CYSTIC-FIBROSIS: di. SWEATING. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. HUMAN. INFANT. PILOCARPINE: du. SWEAT: ph. AB The sweat test, correctly performed, appears to be reliable, but results were found to be unreliable about half the time in 84 small community hospitals assessed. The reasons are inexperienced and untrained technicians, the infrequency of test performance, the use of unstandardized equipment, and the lack of appropriate standards. It behooves all clinical laboratory directors to re-examine and standardize their procedures and use the recommendations of the Cystic Fibrosis Foundation. Physicians should be made aware of the current situation and send their patients only to laboratories that perform the test reliably. RF 001 GIBSON LE PEDIATRICS 23 545 959 002 ANON GUIDE TO DIAGNOSIS AND MANAGE 963 003 SCHALES O J BIOL CHEM 140 879 941 004 SHWACHMAN H N ENGL J MED 255 999 956 005 SHWACHMAN H PEDIATRICS 30 167 962 006 KOPITO L N ENGL J MED 272 504 965 007 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 008 WEBSTER L MED J AUST 1 923 977 CT 1 SHWACHMAN H CLIN CHEM 25 1008 979 2 SHWACHMAN H J PEDIATR 95 661 979 3 SEALE TW PEDIATR RES 14 1398 980 4 DENNING CR PEDIATRICS 66 752 980 5 WONG LTK GUT 23 744 982 6 BOWLING SA AM J MED TECHNOLOGY 48 25 982 7 RANDOLPH VS AM J MED TECHNOLOGY 48 15 982 8 KIRK JM ANN CLIN BIOCHEM 20 369 983 9 HEELEY AF CLIN CHEM 29 2011 983 10 GREEN A ANN CLIN BIOCHEM 22 171 985 11 ITANO M RESPIRATION 47 220 985 12 WARWICK WJ CLIN CHEM 32 850 986 13 ROSENSTEIN BJ CLIN PEDIATR 26 78 987 PN 79073 RN 01053 AN 79105888 AU Willcox-P. TI Secretion of beta-N-acetylglucosaminidase isoenzymes by cultured cystic fibrosis fibroblasts. SO Clin-Chim-Acta. 1979 Jan 1. 91(1). P 81-9. MJ ACETYLGLUCOSAMINIDASE: se. CYSTIC-FIBROSIS: en. GLUCOSAMINIDASE: se. ISOENZYMES: se. MN CELLS-CULTURED. FIBROBLASTS: en. HUMAN. AB Secretion of the lysosomal enzyme beta-N-acetylglucosaminidase (EC 3.2.1.30) by normal and cystic fibrosis fibroblasts has been compared. In contrast to an earlier report, no differences were found in either the rate of secretion, or in the molecular forms of the enzyme secreted by normal fibroblast cultures and cultures initiated from patients with cystic fibrosis. The B isoenzyme of beta-N- acetylglucosaminidase from cystic fibrosis fibroblasts was converted to a more negatively charged form during preincubation for 3 h at pH 5.0 and 37 degrees C. The enzyme from normal fibroblasts was unaffected by this treatment. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 WILLCOX P EUR J BIOCHEM 73 579 977 003 WILLCOX P BIOCHEM J 173 433 978 004 WARREN L J BIOL CHEM 234 1971 959 005 LOWRY OH J BIOL CHEM 193 265 951 006 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 007 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 008 DI SANTAGNESE PA N ENGL J MED 295 534 976 009 ALHADEFF JA CLIN GENET 10 63 976 CT 1 WILLCOX P BIOCHIM BIOPHYS ACTA 586 442 979 2 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 3 FIGARELLA C EUR J CLIN INVEST 12 145 982 PN 79074 RN 01054 AN 79211819 AU Casola-L. Di-Matteo-G. Romano-M. Rutigliano-B. Mastella-G. TI Glycosidases in serum of cystic fibrosis patients. SO Clin-Chim-Acta. 1979 May 16. 94(1). P 83-8. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: bl. MN ACETYLGLUCOSAMINIDASE: bl. ADULT. ARYLSULFATASES: bl. BETA-GALACTOSIDASES: bl. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. FUCOSIDASE: bl. GLUCURONIDASE: bl. HUMAN. MANNOSIDASES: bl. REFERENCE-VALUES. AB In a study of eight glycosidases in serum samples from 72 cystic fibrosis patients, 85 cystic fibrosis parents and 34 healthy and diseased controls, significant elevations of mean alpha-glucosidase levels were found in cystic fibrosis patients. All other glycosidases did not show any significant change. Mean alpha-glucosidase levels in obligate heterozygotes were the same as in control individuals. Moreover, alpha-glucosidase levels in cystic fibrosis patients correlated with the degree of clinical impairment as measured by the Schwachman score. RF 001 BARTMAN J J PEDIATR 76 430 970 002 DANES BS LANCET 1 1061 968 003 DANES BS J EXP MED 129 775 969 004 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 005 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 006 KRAUS I PEDIATRICS 47 1010 971 007 ANTONOWICZ I PEDIATR RES 6 803 972 008 BUTTERWORTH J CLIN CHIM ACTA 41 367 972 009 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 010 WILSON RG CLIN CHIM ACTA 36 189 972 011 MARCHI AG HELV PAEDIATR ACTA 28 427 973 012 GIBBS GE SCIENCE 167 993 970 013 GRIFFIN GD PROC SOC EXP BIOL MED 137 438 971 014 GIBBS GE J INVEST DERMATOL 51 200 968 015 EVANS BW AM J DIS CHILD 127 660 974 016 BENKE PJ N ENGL J MED 284 731 971 017 FILLIAT M PATHOL BIOL (PARIS) 21 13 973 018 RUSSELL SB J MED GENET 8 441 971 019 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 020 ALHADEFF JA CLIN GENET 10 63 976 021 STAHL P ARCH BIOCHEM BIOPHYS 170 53 975 022 MORELL AG J BIOL CHEM 246 1461 971 023 SHWACHMAN H AM J DIS CHILD 96 6 958 024 DAHLQVIST A ANAL BIOCHEM 7 18 964 025 SMITH BW J BIOL CHEM 227 357 957 026 ANNUNZIATA P CLIN CHIM ACTA 90 101 978 027 JEFFREY PL BIOCHEMISTRY 9 1416 970 028 NIKOLAJEK WP EUR J PEDIATR 122 289 976 029 TOWNES PL AM J HUM GENET 28 378 976 030 STANBURY JB METABOLIC BASIS OF INHERITED 972 031 KAWASAKI T J BIOL CHEM 251 1296 976 CT 1 ALHADEFF JA CLIN CHIM ACTA 105 131 980 2 MALER T FEBS LETTERS 121 153 980 3 BARLOW JJ J NATL CANCER INST 67 1005 981 4 ALHADEFF JA CLIN CHIM ACTA 117 227 981 5 CALVO P CLIN CHIM ACTA 119 15 982 6 CABRINI G RIV ITAL PEDIATR 10 405 984 7 ALHADEFF JA PEDIATR RES 19 171 985 8 PORTER WH CLIN CHEM 32 652 986 PN 79075 RN 01055 AN 80045420 AU Butterworth-J. Duncan-J-J. TI Carboxypeptidase B activity of cultured skin fibroblasts and relationship to cystic fibrosis. SO Clin-Chim-Acta. 1979 Sep 15. 97(1). P 39-43. MJ CARBOXYPEPTIDASES: me. CYSTIC-FIBROSIS: en. MN ARGININE: aa, me. CELLS-CULTURED. DITHIOTHREITOL: pd. FIBROBLASTS: en. HIPPURATES: me. HUMAN. HYDROGEN-ION-CONCENTRATION. SUBCELLULAR-FRACTIONS: en. AB Enzyme activity against hippuryl-L-arginine was studied in cultured skin fibroblasts from controls and cystic fibrosis patients. The enzyme had a lysosomal distribution and an acid optimum of pH 4.5 with little or no activity present above pH 7.0. Dithiothreitol was required for full activity and the kinetics of thiol activation were different for the control and cystic fibrosis enzyme. The properties and lysosomal distribution of the enzyme indicated that it was a carboxypeptidase B. Substrate affinity, thermolability, pH stability, the fall and rise in activity with subculture, the cyclical pattern of activity through serial passage and the level of activity were similar for the control and cystic fibrosis enzyme. RF 001 CONOVER JH LIFE SCI 14 253 974 002 BUTTERWORTH J CLIN CHIM ACTA 44 295 973 003 GUY GJ CLIN CHIM ACTA 87 63 978 004 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 005 LOWRY OH J BIOL CHEM 193 265 951 006 DAWSON RMC DATA FOR BIOCHEMICAL RESEARCH 972 007$ MARINKOVIC DV BIOCHEM J 163 265 951 008 ERDOS EG BIOCHEM PHARMACOL 11 585 962 009 OSHIMA G BIOCHIM BIOPHYS ACTA 365 344 974 010 PLUMMER TH J BIOL CHEM 253 3907 978 011 LIEBERMAN J AM REV RESPIR DIS 111 100 975 012 KOHEIL A BIOCHIM BIOPHYS ACTA 524 156 978 013 GREENBAUM LM J BIOL CHEM 237 1082 962 014 OTTO K BIOCHIM BIOPHYS ACTA 379 462 975 015 NINJOOR V BIOCHIM BIOPHYS ACTA 370 308 974 016 TAYLOR SL BIOCHIM BIOPHYS ACTA 341 99 974 017 MCDONALD JK IN: BARRETT AJ 311 977 018 SHAPIRO BL PROC SOC EXP BIOL MED 144 181 973 CT 1 BUTTERWORTH J ANAL BIOCHEM 106 156 980 2 BUTTERWORTH J CLIN CHIM ACTA 108 143 980 3 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 4 BUTTERWORTH J CLIN CHIM ACTA 122 51 982 5 BUYS CHCM CLIN CHIM ACTA 136 229 984 PN 79076 RN 01056 AN 80045463 AU Ben-Yoseph-Y. Defranco-C-L. Nadler-H-L. TI Decreased sialic acid and altered binding to lectins of purified alpha 2-macroglobulin from patients with cystic fibrosis. SO Clin-Chim-Acta. 1979 Nov 15. 99(1). P 31-5. MJ ALPHA-MACROGLOBULINS. CYSTIC-FIBROSIS: bl. LECTINS. SIALIC-ACIDS: an. MN ALPHA-MACROGLOBULINS: ip. CONCANAVALIN-A. CYSTIC-FIBROSIS: fg. HETEROZYGOTE-DETECTION. HEXOSES: an. HUMAN. SUPPORT-U-S-GOVT-P-H-S. AB Purified preparations of plasma alpha 2-macroglobulin from patients with cystic fibrosis are shown to have normal amounts of total hexose but as much as 40% decrease in their sialic acid content. The binding of these preparations to concanavalin A and wheat-germ agglutinin was markedly reduced as compared to normal values in controls. Intermediate values were found in obligate heterozygotes. These results suggest a possible alteration in the carbohydrate moiety of alpha 2-macroglobulin in cystic fibrosis, presumably due to a defective posttranslational process. RF 001 ALHADEFF JA CLIN GENET 14 189 978 002 NADLER HL IN: STANBURY JB 1683 978 003 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 004 HICKMAN S BIOCHEM BIOPHYS RES COMMUN 57 55 974 005 ALHADEFF JA CLIN GENET 13 417 978 006 SCHULTZE HE MOLECULAR BIOL OF HUMAN PROTE 1 966 007 DUNN JT J BIOL CHEM 242 5549 967 008 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 009 SHAPIRA E CLIN CHIM ACTA 78 359 977 010 SHAPIRA E J BIOL CHEM 252 7923 977 011 PORATH J NATURE 215 1401 967 012 WEBER K J BIOL CHEM 244 4406 969 013 SPIRO RG IN: NEUFELD EF 8 3 966 014 WARREN L J BIOL CHEM 234 1971 959 015 GOLDSTEIN IJ ADV CARBOHYDR CHEM BIOCHEM 35 127 978 016 GREENAWAY PJ NATURE NEW BIOL 241 191 973 017 ROUSSON R BIOCHEM J 180 501 979 CT 1 COMINGS DE AM J HUM GENET 32 273 980 2 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 3 BENYOSEPH Y BIOCHEM J 189 9 980 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 5 HALLINAN F MED HYPOTHESES 7 793 981 6 BENYOSEPH Y PEDIATR RES 15 839 981 7 MALER T J BIOL CHEM 256 1420 981 8 OWENSWILLIAMS L J PEDIATR GASTROENTEROL NUTR 1 567 982 9 BURY AF IRCS MED SCI BIOCHEM 10 255 982 10 BLITZER MG PEDIATR RES 16 203 982 11 ROBERTS RC PEDIATR RES 16 416 982 12 BRIDGES MA CLIN CHIM ACTA 118 33 982 13 BURY AF CLIN CHIM ACTA 118 45 982 14 TUMMLER B CLIN CHIM ACTA 125 219 982 15 MARGOLIES R PEDIATR RES 17 931 983 16 BACK SA BIOCHEM MED 30 34 983 17 SLOMIANY A J BIOL CHEM 258 8535 983 18 BRIDGES MA ANN NY ACAD SCI 421 360 983 19 SHAPIRA E ANN NY ACAD SCI 421 352 983 20 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 21 EAGER KB PEDIATR RES 18 999 984 22 KARLSSON S J MED GENET 21 441 984 23 GUEANT JL CLIN CHIM ACTA 143 217 984 24 PARK CM PEDIATR RES 19 344 985 PN 79077 RN 01057 AN 79084606 AU Scanlin-T-F. Matacic-S-S. Glick-M-C. TI Abnormal distribution of alpha-L-fucosidase in cystic fibrosis: decreased activity in serum. SO Clin-Chim-Acta. 1979 Jan 15. 91(2). P 197-202. MJ CYSTIC-FIBROSIS: bl. FUCOSIDASE: bl. MN ACETYLGLUCOSAMINIDASE: bl. ACID-PHOSPHATASE: bl. ADULT. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en. FIBROBLASTS: en. FUCOSIDASE: an. HUMAN. INFANT-NEWBORN. SKIN: en. SUPPORT-U-S-GOVT-P-H-S. AB The activity of alpha-L-fucosidase is decreased in the serum of cystic fibrosis patients when compared to age-matched controls. This result, combined with the elevated activity in skin fibroblasts, supports the concept of an abnormal intracellular and extracellular distribution of alpha-L-fucosidase in cystic fibrosis. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 004 ZIELKE K J LAB CLIN MED 79 164 972 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 SCANLIN TF JR PROC INT CF CONG 7TH 44 976 007 SCANLIN TF JR PEDIATR RES ABST 549 11 463 977 008 NG WG AM J HUM GENET 28 42 976 009 RAMAGE P BIOCHIM BIOPHYS ACTA 403 473 975 010 TURNER BM AM J HUM GENET 27 651 975 011 DI MATTEO G BIOCHIM BIOPHYS ACTA 429 538 976 012 CHESTER MA BIOCHIM BIOPHYS ACTA 485 147 977 013 ALHADEFF JA CLIN GENET 10 63 976 014 SCANLIN TF JR PEDIATR RES 12 457 978 015 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 016 NEUFELD EF ANNU REV BIOCHEM 44 357 975 017 KAPLAN A PROC NAT ACAD SCI USA 74 2026 977 018 HIEBER V BIOCHEM BIOPHYS RES COMMUN 73 710 976 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 TARNOKY AL J ROY SOC MED 73 73 980 3 ALHADEFF JA CLIN CHIM ACTA 105 131 980 4 BARLOW JJ J NATL CANCER INST 67 1005 981 5 JAKEL HP BIOL ZENTRALBL 100 273 981 6 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 7 SCANLIN TF CLIN CHIM ACTA 114 269 981 8 ALHADEFF JA CLIN CHIM ACTA 117 227 981 9 MALER T J BIOL CHEM 256 1420 981 10 SCANLIN TF BIOCHEMISTRY 21 491 982 11 SHEPHERD VL J RETICULOENDOTHEL SOC 32 423 982 12 BOURASSA C CLIN CHIM ACTA 129 263 983 13 SCANLIN TF PEDIATR RES 19 368 985 PN 79078 RN 01058 AN 80023017 AU Lake-C-R. Davis-P-B. Ziegler-M. Kopin-I-J. TI Electrolytes and norepinephrine levels in blood of patients with cystic fibrosis. SO Clin-Chim-Acta. 1979 Mar 1. 92(2). P 141-6. MJ CYSTIC-FIBROSIS: bl. ELECTROLYTES: bl. NOREPINEPHRINE: bl. MN ADOLESCENCE. ADULT. HUMAN. BLOOD-PRESSURE. DOPAMINE-BETA-HYDROXYLASE: bl. EXERTION. FEMALE. MALE. POSTURE. SEASONS. AB Young adults with cystic fibrosis in good to excellent condition have reduced plasma sodium and chloride and elevated plasma potassium compared to a group of healthy young adult control subjects. Blood pressure was also lower in the patients with cystic fibrosis. However, plasma norepinephrine and dopamine-beta-hydroxylase and their response to standing and isometric hand grip were normal in the cystic fibrosis patients. RF 001 DAVIS PB PEDIATR RES 12 703 978 002 BARBERO GJ CF CLUB ABST 4 967 003 BONGIOVANNI AM LAB INVEST 10 956 961 004 HENRY DP LIFE SCI 16 375 975 005 LAKE CR LIFE SCI 18 1315 976 006 WEINSHILBOUM RM SCIENCE 174 1349 971 007 TAUSSIG LM J PEDIATR 82 380 973 008 WEINSHILBOUM RM CIRC RES 28 307 971 009 BOLANDE RP ARCH PATHOL 95 172 973 010 LAKE CR CIRCULATION 57 774 978 011 LAKE CR AM SOC NEPHROL ABST 50A 979 012 OGIHARA T J LAB CLIN MED 85 566 975 013 LIEBERMAN J ANN INTERN MED 82 806 975 014 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 015 QUINTERO-ATENCIO J N ENGL J MED 274 1224 966 016 LEVI J ISR J MED SCI 6 665 970 017 MCCANE RA LANCET 1 823 936 018 MCCANE RA PROC R SOC LOND BIOL 119 245 936 019 SIMOPOULOS AP PEDIATR RES 5 626 971 020 ROBSON AM J PEDIATR 79 42 971 021 GRAND RJ J PEDIATR 70 357 967 022 MONTALVO JM J CLIN ENDOCRINOL METAB 28 582 968 023 ROSENTHAL A PEDIATRICS 59 588 977 024 STROBER W PEDIATRICS 43 416 969 CT 1 DAVIS PB IRCS MED SCI BIOCHEM 8 413 980 2 LEMANSKE RF AM REV RESPIR DIS 123 622 981 3 SEALE TW ANN CLIN LAB SCI 12 415 982 4 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 5 DAVIS PB J CHRON DIS 36 269 983 6 DAVIS PB J CLIN INVEST 71 1787 983 7 DAVIS PB SEM RESPIR MED 6 319 985 8 SCHONI MH PEDIATR RES 19 47 985 9 MITCHELL EA AUST PAEDIATR J 21 127 985 10 DAVIS PB HORM METAB RES 18 217 986 11 SCHONI MH EUR J PEDIATR 145 80 986 PN 79079 RN 01059 AN 80023013 AU Butterworth-J. Guy-G-J. TI Primary amniotic fluid cell, skin fibroblast and liver alpha-L-fucosidase and its relation to cystic fibrosis. SO Clin-Chim-Acta. 1979 Mar 1. 92(2). P 109-16. MJ CYSTIC-FIBROSIS: en. FUCOSIDASE: me. MN AMNIOTIC-FLUID: en. CELLS-CULTURED. CHROMATOGRAPHY-DEAE-CELLULOSE. CHROMATOGRAPHY-GEL. FUCOSIDASE: an. HUMAN. HYDROGEN-ION-CONCENTRATION. ISOELECTRIC-FOCUSING. LIVER: en. SKIN: en. AB Cultured skin fibroblast and primary amniotic fluid cell alpha-L- fucosidase had a double optimum of pH 5.0 and 6.0. Alpha-L-fucosidase was largely bound as a single peak to DEAE-cellulose at pH 6.6. Sucrose density isoelectric focusing revealed up to seven components with pI values of 4.9, 5.2, 5.4, 5.8, 6.1, 6.5 and 7.1 with their apparent KM values (77--500 mumol/l) being higher than that (57 mumol/l) of the unfocused enzyme. Liver, skin fibroblast and amniotic fluid cell alpha-L-fucosidase was separated into two peaks by gel filtration. Peak one was more active and stable at low pH and more thermostable at 50 degrees C than peak two, while both peaks had an apparent KM of 52 mumol/l. Apart from the different proportions of the peaks separated by gel filtration, the results for the three tissues were similar. The properties of alpha-L-fucosidase studied were similar for control and cystic fibrosis liver or skin fibroblasts. RF 001 VAN HOOF F LANCET 1 1198 968 002 ZIELKE K J EXP MED 136 197 972 003 POENARU L CLIN GENET 10 260 976 004 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 005 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 006 ALHADEFF JA CLIN GENET 10 63 976 007 BUTTERWORTH J CLIN CHIM ACTA 78 335 977 008 DAWSON RMC DATA FOR BIOCHEMICAL RESEARCH 972 009 BUTTERWORTH J CLIN GENET 12 297 977 010 BUTTERWORTH J CLIN GENET 9 505 976 011 LOWRY OH J BIOL CHEM 193 265 951 012 GODSON NG ANAL BIOCHEM 35 66 970 013 BERATIS NG PEDIATR RES 11 862 977 014 ROBINSON D CLIN CHIM ACTA 55 65 974 015 BERATIS NG J PEDIATR 87 1193 975 016 ELLIS RB CLIN SCI MOL MED 49 543 973 017 WILLCOX P EUR J BIOCHEM 73 579 977 018 ROBINSON D CLIN CHIM ACTA 47 403 973 019 ALHADEFF JA CLIN CHIM ACTA 57 307 974 020 THORPE R FEBS LETTERS 54 89 975 021 DI MATTEO G BIOCHIM BIOPHYS ACTA 429 527 976 022 TURNER BM AM J HUM GENET 27 651 975 023 WIEDERSCHAIN GY CLIN CHIM ACTA 46 305 973 024 WOOD S J LAB CLIN MED 88 469 976 025 CHESTER MA BIOCHIM BIOPHYS ACTA 485 147 977 026 CARLSEN RB J BIOL CHEM 247 23 972 027 ALHADEFF JA J BIOL CHEM 250 7106 975 028 ANDREWS P BIOCHEM J 96 595 965 CT 1 ALHADEFF JA CLIN CHIM ACTA 105 131 980 2 BUTTERWORTH J ANAL BIOCHEM 106 156 980 3 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 4 BUTTERWORTH J CLIN CHIM ACTA 108 143 980 5 MALER T FEBS LETTERS 121 153 980 6 JAKEL HP BIOL ZENTRALBL 100 273 981 7 BUTTERWORTH J CLIN CHIM ACTA 110 319 981 8 HARRIS A CLIN CHIM ACTA 116 171 981 9 MALER T J BIOL CHEM 256 1420 981 10 COHENFORD MA COMP BIOCHEM PHYSIOL (B) 72 695 982 11 BURY AF CLIN CHIM ACTA 118 45 982 12 BUTTERWORTH J CLIN CHIM ACTA 122 51 982 13 BEYER EM CLIN CHIM ACTA 123 251 982 14 BOURASSA C CLIN CHIM ACTA 129 263 983 15 WAUTERS J ARCH INT PHYSIOL BIOCHIM 92 B115 984 16 HERMELIN B CELL MOL BIOL 33 83 987 PN 79080 RN 01060 AN 79147574 AU Carswell-F. Oliver-J. Silverman-M. TI Allergy in cystic fibrosis. SO Clin-Exp-Immunol. 1979 Jan. 35(1). P 141-6. MJ CYSTIC-FIBROSIS: im. HYPERSENSITIVITY-IMMEDIATE: et. MN ADULT. ANTIGENS: im. ASPERGILLUS-FUMIGATUS: im. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, fg. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYPERSENSITIVITY-IMMEDIATE: fg, im. IGE: an. IGG: an. MALE. RESPIRATORY-FUNCTION-TESTS. SKIN-TESTS. AB Forty-four patients with cystic fibrosis, sixty-seven parents, and thirty-nine controls were compared with regard to their immediate cutaneous hypersensitivity, total and specific serum IgE and serum IgG4 concentrations. The patients had increased prick test reactions and specific IgE to Aspergillus fumigatus. The development of reactions to A. fumigatus correlated with the severity of the disease. There were no other significant differences when serum total IgE, specific IgE and IgG4 concentrations or skin test reactivity were compared in the patients, parents and controls. RF 001 ALLAN JD CLIN ALLERGY 5 255 975 002 BATTEN JC MOD PROBL PEDIATR 10 227 967 003 BUTLER U PROC BPA RESPIRATORY GROUP MT 977 004 COGSWELL JJ BR J DIS CHEST 69 177 975 005 CHRISPIN AR PEDIATR RADIOL 2 101 974 006 DAVIS JB CLIN ALLERGY 6 329 976 007 GODFREY RC CLIN ALLERGY 6 79 976 008 GODFREY S BR J DIS CHEST 64 15 970 009 JOHANSSON SGO IN: VYAS GN 225 975 010 KJELLMAN NIM ACTA PAEDIATR SCAND 65 465 976 011 KJELLMAN NIM CLIN ALLERGY 6 51 976 012 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 013 MCFARLANE H LANCET 1 1241 976 014 MCFARLANE H CLIN ALLERGY 7 279 977 015 MALO JL THORAX 32 269 977 016 SARSFIELD JK ARCH DIS CHILD 49 711 974 017 SHAKIB F J IMMUNOL METH 8 17 975 018 SHAKIB F CLIN ALLERGY 6 237 976 019 STANWORTH DR CLIN ALLERGY 3 37 973 020 VIJAY HM INT ARCH ALLERGY APPL IMMUNOL 53 78 977 021 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 022 WARNER JO LANCET 1 990 976 023 WARNER JO ARCH DIS CHILD 51 507 976 024 WARNOCK DW J CLIN PATHOL 30 388 977 025 WARREN CPW CLIN ALLERGY 5 1 975 CT 1 REEN DJ CLIN ALLERGY 11 571 981 2 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 3 WONNE R CLIN ALLERGY 15 455 985 4 MOSS RB MONOGR ALLERGY 19 202 986 5 MOSS RB CHEST 91 522 987 PN 79081 RN 01061 AN 80002327 AU Super-M. van-Schalkwyk-D-J. TI Heterozygote advantage in cystic fibrosis: mosquito tests. SO Clin-Genet. 1979 Aug. 16(2). P 65-8. MJ CYSTIC-FIBROSIS: fg. INSECT-BITES-AND-STINGS. MOSQUITOES. MN HETEROZYGOTE. HUMAN. MALARIA: mo, oc. AB Tests to demonstrate a preference by mosquitoes for stinging controls as opposed to obligate heterozygotes for Cystic Fibrosis proved negative. If a heterozygote advantage caused a lower malarial incidence in carriers in South West Africa, it must have worked through the malarial parasite being adversely affected by a serum factor. This remains to be tested. RF 001 ANDERSON CM MOD PROBL PEDIATR 10 381 967 003 COHEN FL J MED GENET 11 253 974 004 CONNEALLY PM TEX REP BIOL MED 31 639 973 005 CRAWFURD MDA HEREDITY 29 126 972 006 DANKS DM ANN HUM GENET 28 323 965 007 EDWARDS JH ARCH DIS CHILD 52 343 977 009 GAIRDNER D LANCET 1 279 975 010 GIBSON LE PEDIATRICS 23 545 959 012 KNUDSON AG JR AM J HUM GENET 19 388 967 013 MASTERSON J PROC EWGCF 6TH ANNU MTG 975 014 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 015 SPOCK A PEDIATR RES 1 173 967 016 STUART AB LANCET 2 1521 974 017 SUPER M S AFR MED J 49 818 975 018 SUPER M THESIS 978 PN 79082 RN 01062 AN 80090635 AU Koheil-A. Corey-M. Forstner-G. TI Deficiency of serum carboxypeptidase B-like (anaphylatoxin inactivator, carboxypeptidase N) activity in sera from patients with cystic fibrosis. SO Clin-Invest-Med. 1979. 2(2-3). P 99-103. MJ CARBOXYPEPTIDASES: df. CYSTIC-FIBROSIS: en. ARGININE-CARBOXYPEPTIDASE: df. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. ARGININE-CARBOXYPEPTIDASE: bl. MALE. AB Serum from 95 patients with cystic fibrosis and 47 control subjects without cystic fibrosis was analyzed for carboxypeptidase B-like activity in the presence of optimal concentrations of Co2+ ion, hippuryl-L-lysine and serum. Fourteen of the cystic fibrosis patients had no steatorrhea and therefore minimal pancreatic dysfunction. Patients with cystic fibrosis and controls were of comparable age. Serum carboxypeptidase B-like activity was significantly decreased in cystic fibrosis patients including cystic fibrosis patients without steatorrhea. Significantly low values were found in both male and female cystic fibrosis patients when compared with controls of similar sex. Although values were unaffected by age in the control population, values in older patients with cystic fibrosis were significantly lower than values in younger patients. No correlation was obtained between serum carboxypeptidase B-like activity and pulmonary or hepatic function. Serum carboxypeptidase B-like activity therefore falls with progression of the disease and may limit the ability to degrade circulating peptides in older patients with cystic fibrosis. Deficient serum carboxypeptidase B-like activity is less likely, however, to be a factor in the development of the early manifestations of the disease. RF 001 OSHIMA G BIOCHIM BIOPHYS ACTA 365 344 974 002 BOKISCH VA J CLIN INVEST 49 2427 970 003 ERDOS EG BIOCHEM PHARMACOL 13 893 964 004 ERDOS EG BIOCHEM PHARMACOL 11 585 962 005 ERDOS EG BIOCHEM PHARMACOL 8 112 961 006 CORBIN NC ANAL BIOCHEM 73 41 976 007 ERDOS EG BIOCHEM PHARMACOL 16 1287 967 008 ERDOS EG CLIN CHIM ACTA 11 39 965 009 CONOVER JH LANCET 2 1501 973 010 CONOVER JH LIFE SCI 14 253 974 011 LIEBERMAN J AM REV RESPIR DIS 111 100 975 012 CUSHMAN DW BIOCHEM PHARMACOL 20 1637 971 013 COREY M AM REV RESPIR DIS 114 1085 976 014 NIE NH STAT PACKAGE FOR THE SOCIAL S 975 015 KOHEIL A BIOCHIM BIOPHYS ACTA 524 156 978 CT 1 SCHWEISFURTH H DTSCH MED WSCHR 109 166 984 2 SCHWEISFURTH H DTSCH MED WSCHR 109 1254 984 3 SCHWEISFURTH H CLIN BIOCHEM 18 242 985 4 SCHWEISFURTH H CLIN BIOCHEM 20 43 987 PN 79083 RN 01063 AN 79235200 AU Crass-J-R. LHeureux-P. Loken-M. TI False-positive 111In-labeled leukocyte scan in cystic fibrosis. SO Clin-Nucl-Med. 1979 Jul. 4(7). P 291-3. MJ CYSTIC-FIBROSIS: ri. INDIUM: du. RADIOISOTOPES: du. MN ABDOMEN: ri. CASE-REPORT. CHILD. DIAGNOSIS-DIFFERENTIAL. FALSE-POSITIVE-REACTIONS. FEMALE. HUMAN. INFLAMMATION: ri. ISOTOPE-LABELING. LEUKOCYTES. LUNG-DISEASES: ri. PELVIS: ri. AB A false-positive 111In-labeled leukocyte scan was obtained in a girl with cystic fibrosis and infection. There was focal accumulation of 111In activity in the abdomen and marked pulmonary uptake. Surgical exploration and postmortem examination revealed no abdominal abnormality. The abnormal activity was apparently due to swallowed sputum which contained labeled leukocytes. Broader clinical implications are discussed. RF 001 THAKUR ML J NUCL MED 18 1014 977 002 THAKUR ML J LAB CLIN MED 89 217 977 CT 1 GOODWIN DA CLIN NUCL MED 8 36 983 2 KIPPER MS CLIN NUCL MED 8 449 983 3 KRIEVES DA CLIN NUCL MED 8 243 983 4 GAINEY MA CLIN NUCL MED 9 71 984 5 LOKEN MK CLIN NUCL MED 10 902 985 6 LOMENA F MED CLIN 84 58 985 7 MCAFEE JG RADIOLOGY 155 221 985 8 FLOYD JL J NUCL MED 27 495 986 9 DATZ FL RADIOLOGY 160 635 986 10 WILLIAMSON SL CLIN NUCL MED 12 27 987 PN 79084 RN 01064 AN 80090738 AU Clamp-J-R. Gough-M. TI Study of the oligosaccharide units from mucus glycoproteins of meconium from normal infants and from cases of cystic fibrosis with meconium ileus. SO Clin-Sci. 1979 Nov. 57(5). P 445-51. MJ CYSTIC-FIBROSIS: me. MECONIUM: an. MUCOPROTEINS. OLIGOSACCHARIDES: an. MN CARBOHYDRATES: me. COMPARATIVE-STUDY. FUCOSE: me. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: me. INTESTINAL-OBSTRUCTION: me. MECONIUM: me. MONOSACCHARIDES: an. AB 1. The mucus glycoproteins in meconium from normal infants and from infants having cystic fibrosis with meconium ileus have been studied. 2. Whereas normal meconium contained about 50% protein-bound carbohydrate, the meconium from cystic fibrosis contained only about 10%. 3. Glycopolypeptides were prepared from the mucus of glycoproteins. The oligosaccharide units from this material were released and fractionated. The fractions ranged widely in size and composition. 4. The fractions from cystic fibrosis specimens had a significantly higher content of fucose than those from normal specimens. RF 001 ALHADEFF JA BIOCHEM BIOPHYS RES COMMUN 86 787 979 002 ALLEN A BR MED BULL 34 28 978 003 BOORMAN KE INTRO BLOOD GROUP SEROL 72 970 004 BUCHANAN DJ PROC SOC EXP BIOL MED 77 114 951 005 CARLSON DM J BIOL CHEM 243 616 968 006 CLAMP JR BIOCHEM SOC TRANS 5 1693 977 007 DISCHE Z ANN NY ACAD SCI 106 259 963 008 DISCHE Z PEDIATRICS 24 74 959 009 DUBOIS M ANAL CHEM 28 350 956 010 DURAND P J PEDIATR 75 665 969 011 FRASER D THESIS 122 974 012 FRASER D CLIN CHIM ACTA 59 301 975 013 GIBBONS RA BR MED BULL 34 34 978 014 HARRIES JT BR MED BULL 34 75 978 015 LIST SJ BIOCHEM J 175 565 978 016 ROELFS RE AM J DIS CHILD 113 419 967 017 SCANLIN TF JR BIOCHEM BIOPHYS RES COMMUN 79 869 977 018 SCANLIN TF JR CLIN CHIM ACTA 91 197 979 CT 1 MALER T J BIOL CHEM 256 1420 981 2 SCANLIN TF BIOCHEMISTRY 21 491 982 3 WESLEY AW ADV EXP MED BIOL 144 145 982 4 WESLEY A PEDIATR RES 17 65 983 5 JACOBS LR DIG DIS SCI 28 422 983 6 RUDICK VL J CELL PHYSIOL 115 143 983 7 HARRIS A CLIN CHIM ACTA 128 41 983 8 ALHADEFF JA CLIN CHIM ACTA 134 1 983 9 RYLEY HC CLIN CHIM ACTA 135 49 983 10 SLOMIANY A BIOCHIM BIOPHYS ACTA 750 253 983 11 CROWTHER RS J PHARMACY PHARMACOL 36 21 984 12 ALBAZZAZ FJ RESPIRATION 46 88 984 13 SCANLIN TF PEDIATR RES 19 368 985 14 EGGERMONT E ACTA PAEDIATR SCAND SUPPL 317 1985 16 985 15 SMITH AC CLIN GASTROENTEROL 15 815 986 16 SCHACHTER M J HISTOCHEM CYTOCHEM 34 927 986 PN 79085 RN 01065 AN 79212183 AU Pennington-J-E. TI Pseudomonas aeruginosa infection: pathogenesis and therapy. SO Compr-Ther. 1979 May. 5(5). P 14-22. MJ PSEUDOMONAS-INFECTIONS: th. MN BACTERIAL-VACCINES: tu. BURNS: co. CARBENICILLIN: tu. CROSS-INFECTION: et. CYSTIC-FIBROSIS: co. GENTAMICINS: tu. HUMAN. IMMUNOSUPPRESSION: ae. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: di, et. TICARCILLIN: tu. TOBRAMYCIN: tu. WOUND-INFECTION: et. EX Infection with the gram-negative bacillus, Pseudomonas aeruginosa, is less common than infections with Escherichia coli, Klebsiella pneumoniae, and Proteus sp; however, the mortality associated with pseudomonas sepsis exceeds that found with the more commonly isolated gram-negative bacilli by a considerable margin. While explanations exist for this increased virulence of pseudomonas, including secretions of toxic exoproducts and a high degree of antibiotic resistance, the precise reasons for such unfavorable clinical experience with this organism are uncertain. The current interest in pseudomonas infection is partly due to the ever-increasing numbers of immunosuppressed and granulocytopenic patients seen in clinical practice. The organism, pathogenesis, clinical presentations (burn patients, nosocomial infections, immunosuppressed patients, cystic fibrosis), and therapy are discussed. RF 001 REYNOLDS HY ANN INTERN MED 82 819 975 002 LIU PV J INFECT DIS SUPPL 130 94 974 003 YOUNG LS J INFECT DIS SUPPL 130 111 974 004 TAPPER ML J INFECT DIS SUPPL 130 14 974 005 STEVENS RM ARCH INTERN MED 134 106 974 006 IANNINI PB JAMA 230 558 974 007 PENNINGTON JE AM J MED 55 155 973 008 SCHIMPFF SC ANN INTERN MED 77 707 972 009 PENNINGTON JE J INFECT DIS 131 158 975 010 NOONE P BR MED J 1 477 974 011 ANDRIOLE VT J INFECT DIS 129 124 974 012 PARRY MF AM J MED 64 961 978 013 KLASTERSKY J CHEST 61 117 972 014 FEELEY TW N ENGL J MED 293 471 975 015 PENNINGTON JE J INFECT DIS SUPPL 130 159 974 CT 1 HEIMBACH DM REV INFECT DIS 6 S744 984 2 KNOLLE P BURNS 11 54 984 3 HOLDER IA CAN J MICROBIOL 31 393 985 4 SCHENTAG JJ AM J MED 78 34 985 PN 79086 RN 01066 AN 80112075 AU Rosenlund-M-L. Walsh-E-P. Scott-D-A. Kritchevsky-D. TI Lipids in cystic fibrosis. SO Curr-Concepts-Nutr. 1979. 8. P 219-27. (REVIEW). MJ CYSTIC-FIBROSIS: dh. DIETARY-FATS. MN ADOLESCENCE. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, me. FATTY-ACIDS: bl, me. FATTY-ACIDS-ESSENTIAL: df. FEMALE. FIBROBLASTS: me. HUMAN. MALE. OILS. REVIEW. SUPPORT-U-S-GOVT-P-H-S. EX There are certain aspects of the symptomatology of cystic fibrosis that bear a resemblance to those of essential fatty acid deficiency. We fed a group of patients essential fatty acids orally since intravenous administration is not a practical method for long term treatment. Results of the study were varied. Although some response was noted in all patients, there were no consistent clinical changes seen in every patient. The oral EFA therapy effected no change in sputum cultures. After one year of EFA, the sweat sodium concentration was lower in all but one patient. The clinical aspect of our study is small, but promising. The results suggest a role for EFAs in the care of CF. Whether EFA is a factor in the etiology of CF or merely a manifestation of malabsorption requires further investigation. RF 001 WARWICK WJ JAMA 238 2159 977 002 BURR GO J BIOL CHEM 86 587 930 003 KRAMAR J J NUTR 50 149 953 004 HANSEN AE TEX REP BIOL MED 9 491 951 005 HANSEN AE PEDIATRICS 31 171 963 006 ELLIOTT RB PEDIATR RES 7 427 973 007 ELLIOTT RB PEDIATRICS 57 474 976 008 SHWACHMAN H AM J DIS CHILD 96 6 958 009 GIBSON LE PEDIATRICS 23 545 959 010 KUO PT J CLIN INVEST 44 1924 965 011 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 012 WIESE HF AM J CLIN NUTR 18 155 966 013 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 014 BENNETT MJ AM J CLIN NUTR 20 415 967 015 ROSENLUND ML NATURE 251 719 974 016 ROSENLUND ML PEDIATRICS 59 428 977 017 MOHRHAUER H J LIPID RES 4 151 963 018 PELUFFO RO BIOCHIM BIOPHYS ACTA 441 25 976 019 SPRECHER H IN: KUNAU WH 1 977 020 HOWARD BV BIOCHIM BIOPHYS ACTA 187 293 969 CT 1 PARNHAM MJ AGENTS ACTIONS 14 223 984 PN 79087 RN 01067 AN 80112266 AU Houstek-J. Zapletal-A. Samanek-M. Vavrova-V. TI Obstruction of the respiratory pathways, its evaluation by methods of functional examination of the lungs and development in patients with cystic fibrosis. SO Czech-Med. 1979. 2(1-2). P 1-10. MJ CYSTIC-FIBROSIS: pp. LUNG: pp. MN ADOLESCENCE. ADULT. AIRWAY-OBSTRUCTION: et. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co. FORCED-EXPIRATORY-FLOW-RATES. FUNCTIONAL-RESIDUAL-CAPACITY. HUMAN. RESIDUAL-VOLUME. RESPIRATORY-FUNCTION-TESTS. TOTAL-LUNG-CAPACITY. VITAL-CAPACITY. AB In 28 patients with cystic fibrosis aged 5--24 years the values of maximum expiratory flow rates (Vmax) at lower volume levels were assessed, as well as the "specific" conductance of the respiratory pathways (Gaw/TGV), the vital capacity (VC), total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC), RV/TLC and FRC/TLC ratios and the one-second forced expiration of the vital capacity (FEV1) to evaluate obstruction of the respiratory pathways. Most markedly and most frequently abnormal were the values of Vmax, RV and of the RV/TLC ratio. These findings revealed that in almost all patients with CF there was already during the initial examination an obstruction of the peripheral respiratory pathways and hyperinflation of the lungs. During repeated measurement of the above values of lung function in 15 patients with cystic fibrosis during a period of 1--5 years when the patients increased in height by 10 cm on average, the above values did not deteriorate on average. There was, however, an individual variability of the investigated values during this period. The comprehensive treatment provided in our country prevented in patients with CF a deterioration of obstruction of the respiratory pathways during the period of investigation. RF 001 COOK CD PEDIATRICS 24 181 959 002 COREY M AM REV RESPIR DIS 114 1085 976 003 HOUSTEK J Z ERKR ATMUNGSORGANE 139 7 974 004 HOUSTEK J PRAKT LEKAR 49 216 969 005 VAVROVA V CESK PEDIATR 28 528 973 006 ZAPLETAL A PEDIATRICS 48 64 971 007 ZAPLETAL A CESK PEDIATR 31 532 976 008 ZAPLETAL A CESK PEDIATR 32 513 977 009 ZAPLETAL A Z ERKR ATMUNGSORGANE 149 343 977 PN 79088 RN 01068 AN 80070028 AU Harris-A. TI Prenatal diagnosis of cystic fibrosis [letter]. SO Dev-Med-Child-Neurol. 1979 Oct. 21(5). P 675-6. MJ CYSTIC-FIBROSIS: di. MN FEMALE. HUMAN. PREGNANCY. PRENATAL-DIAGNOSIS: mt. EX As far as I know, no test for the prenatal diagnosis of cystic fibrosis has yet been shown to be reliable, but several experimental studies on prenatal diagnosis of cystic fibrosis are being carried out. Deficient protease activity, assayed by 4-methylumbelliferyl guanidinobenzoate, an active site titrant of serine proteases, has been reported in plasma and cultivated skin fibroblasts from patients with cystic fibrosis. Another prenatal diagnostic test for cystic fibrosis involves the observed resistance of patients' skin fibroblasts to dexamethasone (a synthetic glucocorticoid) toxicity, in comparison to normal fibroblasts. Specific induction of alkaline phosphatase with Tamm Horsfall glycoprotein, isoproterenol and theophylline has been recorded in skin-derived fibroblasts from affected patients. RF 001 WALSH-PLATT M AM J HUM GENET 29 111A 977 002 RAO GJS ENZYME 23 314 978 003 WALSH-PLATT M PEDIATR RES 12 874 978 004 WALSH-PLATT M LANCET 1 622 979 005 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 006 BRESLOW JL CELL 13 663 978 007 BRESLOW JL SCIENCE 201 180 978 008 HOSLI P HUM GENET 41 169 978 PN 79089 RN 01069 AN 79192319 AU MacLeod-S-M. TI Clinical pharmacology in a paediatric hospital. SO Dimens-Health-Serv. 1979 May. 56(5). P 33-4. MJ DRUG-THERAPY: st. HOSPITALS-PEDIATRIC. HOSPITALS-SPECIAL. PEDIATRICS. PHARMACOLOGY. MN ADOLESCENCE. CANADA. CHILD. CHILD-PRESCHOOL. COMMUNICABLE-DISEASES: dt. CYSTIC-FIBROSIS: dt. SUBSTANCE-ABUSE. DRUG-EVALUATION. DRUG-INFORMATION-SERVICES. HUMAN. INFANT. INFANT-NEWBORN. NERVOUS-SYSTEM-DISEASES: dt. PHARMACOLOGY: ed, td. PHARMACY-SERVICE-HOSPITAL. RESEARCH. EX Despite the critical role played by a clinical pharmacologist, there are only three in Canada who specialize in paediatrics. The role of paediatric clinical pharmacologists in the promotion of safer and more effective drug use, in research, in teaching, and in service are discussed, as well as contributions from clinical pharmacology. PN 79090 RN 01070 AN 80024183 AU Walsh-Platt-M. Rao-G-J. Nadler-H-L. TI Protease deficiency in plasma of patients with cystic fibrosis. Reduced reaction of 4-methylumbelliferylguanidinobenzoate with plasma of patients with cystic fibrosis. SO Enzyme. 1979. 24(4). P 224-9. MJ CYSTIC-FIBROSIS: en. HYMECROMONE: bl. PEPTIDE-HYDROLASES: df. UMBELLIFERONES: bl. MN BENZAMIDINES: pd. CHILD. GUANIDINES: bl, me. HUMAN. HYMECROMONE: aa, me. PEPTIDE-HYDROLASES: bl. TRYPSIN-INHIBITORS: me. SUPPORT-U-S-GOVT-P-H-S. AB Protease activity in plasma is assayed using 4- methylumbelliferylguanidinobenzoate. The assay is modified by carrying out the reaction in the presence and absence of benzamidine, a competitive inhibitor of trypsin-like proteases. The parameters of the assay are described in detail. Using this assay, our earlier demonstration of a deficiency of protease activity in plasma of patients with cystic fibrosis is confirmed. The activity, corrected for the nonspecific hydrolysis of 4- methylumbelliferylguanidinobenzoate by benzamidine, is expressed as nanomoles of 4-methylumbelliferone released per milliliter plasma. Under standard conditions, the activity in plasma activated with chloroform-ellagic acid was 127.2 +/- 23.1 in 7 controls, 70.4 +/- 11.7 in 11 obligate heterozygotes, and 48.7 +/- 16.6 in 12 patients with cystic fibrosis. Identical results were obtained when unactivated plasma was used. These data demonstrate that the judicious use of specific inhibitors such as benzamidine might be useful in assaying low levels of protease activity in crude systems. RF 001 ARMITAGE P STATISTICAL METHODS IN MEDICA 971 002 BENDER ML J AM CHEM SOC 88 5890 966 003 CHASE T JR BIOCHEMISTRY 8 2212 969 004 JAMESON GW BIOCHEM J 131 107 973 005 RAO GJS J PEDIATR 80 573 972 006 RAO GJS PEDIATR RES 8 684 974 007 RAO GJS PEDIATR RES 9 739 975 008 RAO GJS SCIENCE 177 610 972 009 RAO GJS ENZYME 23 314 978 010 WALSH-PLATT M AM J HUM GENET 29 111A 977 011 WALSH-PLATT M PEDIATR RES 12 874 978 012 WALSH-PLATT M LANCET 1 622 979 013 WALSH-PLATT M PEDIATR RES 12 874 978 CT 1 NADLER HL LANCET 2 96 980 2 NADLER HL PEDIATRICS 66 690 980 3 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 5 SCHWARTZ M LANCET 2 1226 981 6 BRANCHINI BR LANCET 1 618 982 7 CAREY WF MED J AUST 2 314 982 8 BURY AF PEDIATR RES 16 613 982 9 SCHWARTZ M CLIN CHIM ACTA 124 213 982 10 TUMMLER B CLIN CHIM ACTA 125 219 982 11 GREEN JR EUR J PEDIATR 139 35 982 12 CAREY WF MED J AUST 2 528 983 13 BROCK DJH PRENAT DIAGN 3 1 983 14 POENARU L PRENAT DIAGN 3 169 983 15 BRANCHINI BR PEDIATR RES 17 850 983 16 HEELEY AF CLIN CHEM 29 2011 983 17 BRIDGES MA ANN NY ACAD SCI 421 360 983 18 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 19 ANON LANCET 2 249 985 20 QURESHI AR J PEDIATR 106 913 985 PN 79091 RN 01071 AN 79169319 AU Paporisz-U. Posselt-H-G. Wonne-R. Ristow-W. Roser-D. Knothe-H. Bender-S-W. TI Evaluation of long term tobramycin therapy in patients with cystic fibrosis and advanced pulmonary disease. SO Eur-J-Pediatr. 1979 Apr 3. 130(4). P 259-69. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: dt. LUNG-DISEASES: dt. TOBRAMYCIN: tu. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: ra. FEMALE. HUMAN. LUNG-DISEASES: ra. MALE. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-FUNCTION-TESTS. TIME-FACTORS. TOBRAMYCIN: ad, ae. AB To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but one patient. Objective parameters (chest X-ray, pulmonary function tests) changed to a lesser extent. In only one patient was Pseudomonas eradicated from the sputum but reappeared after discontinuation of therapy. In the rest of the patients Pseudomonas was significantly suppressed or replaced by other pathogens. Four patients showed rises of antibody titres to Candida and two to Aspergillus fumigatus. No nephrotoxic side effects were observed, but vestibular function was reversibly impaired in one patient without corresponding clinical symptoms. No bacterial resistance to tobramycin was observed during therapy. RF 001 BOXERBAUM B J INFECT DIS SUPPL 124 293 971 002 DOGGETT RG SOUTH MED J 61 1347 968 003 DOGGETT RG J BACTERIOL 87 427 964 004 DOGGETT RG SOUTH MED J 57 1476 964 005 DOGGETT RG SOUTH MED J 57 1470 964 006 DOGGETT RG SOUTH MED J 61 1346 968 007 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 008 DOGGETT RG BACT PROC 69 87 969 009 DOGGETT RG INFECT IMMUN 6 628 972 010 HAWLEY HB CURR THER RES 16 414 974 011 HARRISON GM PAEDIATRICIAN 1 180 972 012 HOFF GE SCAND J INFECT DIS 6 333 974 013 HOIBY N SCAND J RESPIR DIS 58 65 977 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 015 HUMPHREY JH ADV IMMUNOL 11 75 969 016 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 017 MEARNS MB ARCH DIS CHILD 47 902 972 018 WAITZ JA ANTIMICROB AGENTS CHEMOTHER 2 431 972 PN 79092 RN 01072 AN 80004150 AU Hosli-P. Vogt-E. TI Cystic fibrosis: decreased thermostability of alpha-mannosidase in crude extracellular fluids of patients and carriers. SO FEBS-Lett. 1979 Aug 15. 104(2). P 271-4. MJ CYSTIC-FIBROSIS: en. EXTRACELLULAR-SPACE: en. MANNOSIDASES: me. SKIN: en. MN CELLS-CULTURED. DRUG-STABILITY. FIBROBLASTS: en. HEAT. HETEROZYGOTE. HUMAN. KINETICS. EX We wish to demonstrate that an abnormal thermostability has been found in crude extracellular fluids for alpha-mannosidase. More extensive studies involving several of the 'leaky' hydrolases are under way and they indicate that the observed thermolabilities can be exploited to develop the urgently needed simple diagnostic tools for cystic fibrosis. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 GIBSON LE PEDIATRICS 23 545 959 004 SCHWARZ V ARCH DIS CHILD 43 695 968 005 STEPHAN U PEDIATRICS 55 35 975 006 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 007 PAPP Z CLIN GENET 11 431 977 008 BRESLOW JL SCIENCE 201 180 978 009 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 010 HOSLI P PROC INT CF CONG 7TH 278 976 011 HOSLI P MONOGR PAEDIATR 10 90 979 012 NEUFELD EF J SUPRAMOL STRUCT 6 95 977 013 KAPLAN A PROC NAT ACAD SCI USA 74 2026 977 CT 1 HIRANI S LANCET 2 906 979 2 HOSLI P FEBS LETTERS 106 U421 979 3 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 4 CAREY WF MED J AUST 2 314 982 5 CRISTOL P SEM HOP PARIS 58 449 982 6 CEDER O CLIN GENET 23 298 983 7 CEDER O ACTA PAEDIATR SCAND 72 291 983 8 KOHLSCHUTTER A FEBS LETTERS 155 223 983 9 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 10 BOZON D ARCH BIOCHEM BIOPHYS 249 546 986 PN 79093 RN 01073 AN 80114944 AU Kraemer-R. Rudeberg-A. Klay-M. Rossi-E. TI Relationship between clinical conditions, radiographic findings and pulmonary functions in patients with cystic fibrosis. SO Helv-Paediatr-Acta. 1979. 34(5). P 417-28. MJ CYSTIC-FIBROSIS: pp. LUNG-VOLUME-MEASUREMENTS. MN ADOLESCENCE. ADULT. BLOOD-GAS-ANALYSIS. CHILD. CYSTIC-FIBROSIS: di, ra. FEMALE. FORCED-EXPIRATORY-VOLUME. FUNCTIONAL-RESIDUAL-CAPACITY. HUMAN. MALE. PARTIAL-PRESSURE. TOTAL-LUNG-CAPACITY. AB Clinical and radiological findings, quantified by special scores, in 36 patients with cystic fibrosis are compared with lung function measurements, including the ratio of RV/TLC and FEV/VC and the arterial blood gases at rest and during exercise. Although respiratory function tests were found to correlate well with both the pulmonary finding score and the chest radiographic score, distinction of three severity groups was possible only by a vector calculation of the blood gases in PaO2-PaCO2-diagram, at rest and during exercise. Thus, measurements of pO2 and pCO2 at rest and on exercise appear to be a helpful adjuvant to routine spirometry for the individual appraisal of the degree of lung involvement, performance and care level. RF 001 BACHOFEN H SCHWEIZ MED WOCHENSCHR 102 1061 972 002 BACHOFEN H J APPL PHYSIOL 34 137 973 003 BACHOFEN H PNEUMONOLOGY 153 160 976 004 BATSCHELET E STATISTICAL METHODS FOR THE A 965 005 BEIER FR AM REV RESPIR DIS 94 430 966 006 CHRISPIN AR PEDIATR RADIOL 2 101 974 007 COREY M AM REV RESPIR DIS 114 1085 976 008 DEMUTH GR AM J DIS CHILD 103 129 962 009 DOERSHUK CF J PEDIATR 65 677 964 010 DOERSHUK CF PEDIATRICS 36 675 965 011 FEATHERBY EA AM REV RESPIR DIS 102 737 970 012 GODFREY S ARCH DIS CHILD 45 534 970 013 GODFREY S ARCH DIS CHILD 46 144 971 014 GODFREY S ARCH DIS CHILD 53 83 978 015 GREENWOOD JA ANN MATH STAT 26 233 955 016 JOHNSON RL JR J APPL PHYSIOL 25 1 968 017 KNOKE JD PEDIATR RES 12 676 978 018 KRAEMER R MONOGR PAEDIATR 10 61 979 019 KRAEMER R PADIAT FORTBILDK PRAXIS 48 7 979 020 LAMARRE A PEDIATRICS 50 291 972 021 LANDAU LI AM REV RESPIR DIS 108 593 973 022 LANDAU LI AM REV RESPIR DIS 111 725 975 023 MARDIA KV STATISTICS OF DIRECTION DATA 972 024 MATTHEW DJ PEDIATR RADIOL 5 198 977 025 MEARNS MB ARCH DIS CHILD 43 528 968 026 MELLINS RB PEDIATRICS 44 315 969 027 PHELAN PD ARCH DIS CHILD 44 393 969 028 STRUTT JW NATURE 72 318 905 029 SCHERRER M SCHWEIZ MED WOCHENSCHR 107 1072 977 030 SPEARMAN C AM J PSYCHOL 15 72 904 031 WATSON GS BIOMETRIKA 43 344 956 032 WEST JB N ENGL J MED 284 1232 971 033 WEST JB J APPL PHYSIOL 30 479 971 034 WHEELER S BIOMETRIKA 51 256 964 035 ZAPLETAL A PEDIATRICS 48 64 971 CT 1 KRAEMER R METH INF MED 19 50 980 2 KRAEMER R EUR J RESPIR DIS 61 118 980 3 KRAEMER R EUR J PEDIATR 138 172 982 4 HARMS HK KLIN PAEDIATR 195 24 983 5 SUTER S J INFECT DIS 149 523 984 6 SUTER S PEDIATR RES 19 346 985 7 SCHAAD UB ACTA PAEDIATR SCAND 75 128 986 PN 79094 RN 01074 AN 80114945 AU Strauss-R-G. TI Complement in cystic fibrosis. SO Helv-Paediatr-Acta. 1979. 34(5). P 429-35. MJ COMPLEMENT-3: an. CYSTIC-FIBROSIS: im. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. COMPLEMENT-PATHWAY-ALTERNATIVE. COMPLEMENT-PATHWAY-CLASSICAL. COMPLEMENT-3B-INACTIVATORS: an. HUMAN. INULIN: du. AB Quantitative and functional assessments were made of both the classical and alternative pathways of complement activation in sera from 23 patients with cystic fibrosis. The classical pathway functioned similarly in patients and controls as measured by CH50 titre. Alternative pathway function, initiated in patient sera by incubation with inulin, was equal to that of controls as determined by cleavage of Factor B and C3, and by the consumption of terminal components. Factor B, however, was more readily activated in patient than in control sera. This rapid alteration of Factor B did not lead to accelerated or more extensive activation of the terminal complement components via the alternative pathway when assessed by C3 cleavage and the consumption of terminal components. Thus, a complement deficiency was not found. The importance of the easily activated Factor B is undefined. RF 001 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 002 BUESCHER ES J PEDIATR 93 530 978 003 CONOVER JH LANCET 2 1501 973 004 CONOVER JH TEX REP BIOL MED 34 45 976 005 FORRISTAL J CLIN EXP IMMUNOL 23 61 977 006 GOTZ M EUR J PEDIATR 127 133 978 007 GOTZE O J EXP MED 134 90S 971 008 HOLZHAUER RJ PEDIATR RES 10 365 976 009 HANN S LANCET 2 520 974 010 KABAT EA EXPERIMENTAL IMMUNOCHEMISTRY 961 011 LIEBERMAN J AM REV RESPIR DIS 111 100 975 012 LYRENE RK J PEDIATR 91 681 977 013 POLLEY MJ J MED GENET 11 249 974 014 SCANLIN TF JR LANCET 1 1382 974 015 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 016 STRAUSS RG PEDIATR RES 11 285 977 017 STRUNK RC ARCH DIS CHILD 52 687 977 018 WEST CD J IMMUNOL 96 650 966 019 WILSON GB J LAB CLIN MED 92 463 978 CT 1 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 PN 79095 RN 01075 AN 80005196 AU Cusack-D. Cannon-D. Skrabanek-P. Powell-D. TI Substance P plasma levels in pregnancy and in various clinical disorders. SO Horm-Metab-Res. 1979 Jul. 11(7). P 448-51. MJ PREGNANCY. SUBSTANCE-P: bl. MN CYSTIC-FIBROSIS: bl. FEMALE. HUMAN. INTESTINAL-DISEASES: bl. NEOPLASMS: bl. RADIOIMMUNOASSAY. THYROID-DISEASES: bl. AB Using radioimmunoassay, immunoreactive SP (iSP) has been measured in the plasma of 162 hospital patients with various disorders and in the plasma of 67 pregnant women. In pregnancy, iSP was undetectable in 40% women as compared to 12% in other hospital patients. The mean iSP plasma level in pregnancy was 37.8 +/- 4.6 (SEM) as compared to 77.1 +/- 4.9 (SEM) pg/ml in other hospital patients. The results support earlier observations based on bioassay, suggesting that the blood of pregnant women contains higher concentrations of a SP-inactivating factor. Of the hospital patients, elevated levels of iSP were found in patients with chronic leukaemia, in one patient with a basaloid carcinoma of the anus, and in one patient with toxic liver damage and pancreatic insufficiency. No correlation was found between thyroid function and iSP plasma levels. ISP plasma levels in various gastrointestinal disorders were similar to those found in normal subjects. RF 001 ALUMETS J HISTOCHEMISTRY 52 217 977 002 CANNON D BIOCHEM SOC TRANS 5 1776 977 003 EHRENPREIS T ACTA PHYSIOL SCAND 27 380 953 004$ HARKINS J BRAIN RES 147 405 978 005 KOCIC-METROVIC D PROC SCI SOC BOSNIA HERZEGOVI 1 113 961 006 NAKANO T JAPAN J PHARMACOL 25 43P 975 007 OCONNELL R IR J MED SCI 145 392 976 008 PARTINGTON MW ARCH DIS CHILD 52 386 977 009$ PEARSE AGE HISTOCHEMISTRY 41 373 975 010 PERNOW B PROC SCI SOC BOSNIA HERZEGOVI 1 57 961 011 POLAK JM IN: FUJITA T 103 976 013 SKRABANEK PD IR J MED SCI 145 399 976 014 SKRABANEK PD IR J MED SCI 147 47 978 015 SKRABANEK PD SUBSTANCE P 978 016 STERN PI PROC SCI SOC BOSNIA HERZEGOVI 1 139 961 CT 1 SKRABANEK P EUR J OBSTE GYNECOL REPR BIOL 11 157 980 2 KAIYA H PSYCHIATRY RES 5 11 981 3 POLLOCK M NEUROLOGY 32 1311 982 4 VERMA PS EUR J PHARMACOL 86 275 982 5 SKRABANEK P OBSTET GYNECOL 61 641 983 6 SKRABANEK P IR J MED SCI 153 47 984 7 ZHANG CL ACTA ZOOL SINICA 31 106 985 PN 79096 RN 01076 AN 80070842 AU Tucker-R-D. Hudrlik-T-R. Gibbs-G-E. Christensen-M-B. TI The effect of serum from patients with pancreatic disease on the short circuit current of rat jejunum. SO IEEE-Trans-Biomed-Eng. 1979 Nov. 26(11). P 607-13. MJ BIOLOGICAL-ASSAY: mt. JEJUNUM: ph. PANCREATIC-DISEASES: bl. MN ANIMAL. BIOLOGICAL-ASSAY: is. CYSTIC-FIBROSIS: bl. DIABETES-MELLITUS: bl. ELECTRIC-CONDUCTIVITY. HUMAN. MEMBRANE-POTENTIALS. PANCREATITIS: bl. RATS. AB The short circuit current rat jejunum bioassay, first employed in an attempt to discern the cystic fibrosis (CF) serum "factor," was evaluated using sera from patients with pancreatic disease. The data suggest other pancreatic diseases, specifically genetic diabetes and alcoholic pancreatitis, also present with a serum "factor" (or "factors") which reduce the short circuit current of rat jejunum, an effect very similar to that of the CF serum "factor." A large number of sera from presumed normal subjects also exhibited a significant reduction in short circuit current; these (false positives) represent a yet to be defined mechanism, however, they do decrease the likelihood that the observed effect is merely due to pancreatic destruction. Detailed procedure and equipment specifications for the bioassay system are included. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 KOPEL FB GASTROENTEROLOGY 62 483 972 004 CRAIG JM AM J DIS CHILD 93 357 957 005 DI SANTAGNESE PA PEDIATRICS 18 387 956 006 DI SANTAGNESE PA N ENGL J MED 295 481 976 007 SPOCK A PEDIATR RES 1 173 967 008 BOWMAN BH SCIENCE 164 325 969 009 BESLEY GTN J MED GENET 6 278 969 010 CHERRY JD J PEDIATR 79 937 971 011 POSSELT HG Z KINDERHEILK 110 93 971 012 ARAKI H PEDIATR RES 9 932 975 013 ARAKI H PROC INT CF CONG 7TH 28 976 014 GILMORE JP PROC SOC EXP BIOL MED 157 70 978 016 CONOVER JH LANCET 1 1194 973 CT 1 TUCKER RD COMPUT BIOL MED 11 153 981 PN 79097 RN 01077 AN 79129529 AU Sorensen-R-U. Stern-R-C. Chase-P. Polmar-S-H. TI Defective cellular immunity to gram-negative bacteria in cystic fibrosis patients. SO Infect-Immun. 1979 Feb. 23(2). P 398-402. MJ ANTIGENS-BACTERIAL: im. CYSTIC-FIBROSIS: im. ENTEROBACTERIACEAE: im. LYMPHOCYTE-TRANSFORMATION. PSEUDOMONAS-AERUGINOSA: im. MN HUMAN. KLEBSIELLA-PNEUMONIAE: im. PROTEUS-MIRABILIS: im. SERRATIA-MARCESCENS: im. SUPPORT-U-S-GOVT-P-H-S. AB In vitro lymphocyte responses to Pseudomonas aeruginosa have been found to be impaired in cystic fibrosis patients with advanced clinical disease. The responses to Klebsiella pneumoniae, Serratia marcescens, and Proteus mirabilis were studied in a similar group of cystic fibrosis patients and normal individuals. Cystic fibrosis patients found to be unresponsive to pseudomonas were also unresponsive to klebsiella, serratia, and proteus. Responsiveness to Staphylococcus aureus was not impaired in cystic fibrosis patients. We postulate that in vitro lymphocyte responses to several gram- negative bacteria require the function of a lymphocyte subpopulation which may be impaired in some cystic fibrosis patients. RF 001 CHANDRA RK PEDIATRICS 59 423 977 002 DOERSHUK CF J PEDIATR 65 677 964 003 FERGUSON AC J PEDIATR 85 717 974 004 GREEN GM AM REV RESPIR DIS 102 691 970 005 GREEN GM J EXP MED 119 167 964 006 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 559 974 007 KALTREIDER HB AM REV RESPIR DIS 113 347 976 008 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 009 MOORE VL RES J RETICULOENDOTHEL SOC 21 131 977 010 NEUMANN CG AM J CLIN NUTR 28 89 975 011 PRESSMAN D IN: SAUNTER M 11 971 012 SELLMEYER E ARCH DIS CHILD 47 429 972 013 SHWACHMAN H AM J DIS CHILD 96 6 958 014 SORENSEN RU INFECT IMMUN 18 735 977 015 SORENSEN RU J PEDIATR 93 201 978 016 THORSBY E HISTOCOMPATIBILITY TESTING 655 970 017 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 018 WARR GA AM REV RESPIR DIS 108 371 973 019 WEIGLE WO IMMUNOLOGY 7 239 964 020 WEIGLE WO J EXP MED 121 289 965 021 WEIGLE WO J IMMUNOL 94 117 965 CT 1 RUUSKANEN O J CLIN LAB IMMUNOL 4 111 980 2 RUUSKANEN O J IMMUNOL 125 411 980 3 MATTHEWS WJ N ENGL J MED 302 245 980 4 SORENSEN RU PEDIATR RES 15 14 981 5 JOHNSON DL RES COMMUN CHEM PATH PHARM 32 377 981 6 RUBIN HR CELL IMMUNOL 57 307 981 7 SORENSEN RU AM REV RESPIR DIS 123 37 981 8 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 9 WILSON GB MED HYPOTHESES 8 527 982 10 SEALE TW ANN CLIN LAB SCI 12 415 982 11 LILLIE MA J CLIN MICROBIOL 16 111 982 12 WOLFE JHN ARCH SURG 117 1266 982 13 MCIRVINE AJ ANN SURG 196 297 982 14 GUIDOTTI TL AM J MED SCI 283 157 982 15 RUBIN HR INFECT IMMUN 39 630 983 16 PORWOLL JM INFECT IMMUN 40 670 983 17 SORENSEN RU INFECT IMMUN 41 321 983 18 MCIRVINE AJ SURG CLIN NORTH AM 63 245 983 19 SORENSEN RU EUR J RESPIR DIS 64 524 983 20 MILLER KM CLIN EXP IMMUNOL 56 415 984 21 AUERBACH HS LANCET 2 686 985 22 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 23 ZAPATASIRVENT RI SURGERY 97 721 985 24 ZAPATASIRVENT RL SURGERY 99 53 986 25 HANSBROUGH JF AM J SURG 151 249 986 26 KEANE RM IR J MED SCI 155 143 986 PN 79098 RN 01078 AN 81093097 AU Thomsen-J. Friis-B. TI High dosage tobramycin treatment of children with cystic fibrosis. Bacteriological effect and clinical ototoxicity. SO Int-J-Pediatr-Otorhinolaryngol. 1979 Jul. 1(1). P 33-40. MJ ANTIBIOTICS: ad. CYSTIC-FIBROSIS: co. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-TRACT-INFECTIONS: dt. TOBRAMYCIN: ad. MN CARBENICILLIN: ad. CHILD. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. MALE. PSEUDOMONAS-INFECTIONS: co, mi. RESPIRATORY-TRACT-INFECTIONS: co, mi. TOBRAMYCIN: ae. AB The bacteriological effect of chemotherapy against mucoid strains of Pseudominas aeruginosa in the lower respiratory tract of patients with cystic fibrosis is reported. A comparison of the effect of high doses of tobramycin (10 mg/kg/24 h) given alone or in combination with carbenicillin showed a significant difference in favour of the combination therapy. In 74.5% of the initially successful courses the patients were recolonized within one month. Fifty-three patients were examined by audiometric and vestibular tests. Only in one patient was it possible to register a transient, high tone hearing impairment at 8000 Hz bilaterally, that might be attributed to the tobramycin treatment. It is concluded that high dose tobramycin treatment in children and adolescents with normal kidney function implies only a minimal clinical risk of adverse ototoxic effects, even after repeated courses of treatment. No nephrotoxic side-effects were found in this material. RF 001 BRUMMET RE ARCH OTOLARYNGOL 101 540 975 002 BRUMMET RE ARCH OTOLARYNGOL 96 505 972 004 HOFF GE SCAND J INFECT DIS 6 333 974 005 LOGAN TB ARCH OTOLARYNGOL 99 190 974 006 MCCRAE WM SCOTT MED J 21 68 976 007 NEU HC J INFECT DIS SUPPL 134 206 976 008 STUPP HF ACTA OTOLARYNGOL STOCKH SUPPL 262 1 970 009 WILSON P BR MED J 1 259 977 CT 1 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 2 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 3 MILLER JJ LANCET 2 272 985 4 CHRYSTYN H J CLIN HOSP PHARM 10 219 985 5 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 PN 79099 RN 01079 AN 79215540 AU Afzelius-B-A. TI The immotile-cilia syndrome and other ciliary diseases. SO Int-Rev-Exp-Pathol. 1979. 19. P 1-43. (REVIEW). MJ CILIA: pp. MN ADULT. ANIMAL. BRAIN: pp. CILIA: ph, ul. CYSTIC-FIBROSIS: pp. DOGS. DYNEIN: ph. EAR: pp. FEMALE. HUMAN. KARTAGENER-TRIAD: pp. MALE. MOVEMENT. RESPIRATORY-TRACT-INFECTIONS: pp. REVIEW. SPERM-MOTILITY. SYNDROME. TUBULIN: ph. UROGENITAL-SYSTEM: pp. EX It is the purpose of this review to present evidence for a disorder which is believed to be caused by defective cilia and to review some data on the occurrence of abnormal cilia, evidently formed in response to environmental insults. The cilium, human ciliated structures, the immotile-cilia syndrome and other inborn ciliary diseases, cystic fibrosis, and acquired disorders of ciliated structures are discussed. Ciliary activity is not vital, but its loss will cause a great deal of discomfort. The greatest problems relate to the lower respiratory tract, which is also a site of most of the cilia in the body. It is striking that a certain degree of clearance can be maintained in the lungs in the absence of motile cilia by coughing, hawking, and other mechanisms. RF 001 AARONS GH POSTGRAD MED J 45 736 959 002 ADAMS R J THORAC SURG 7 206 937 003 AFZELIUS BA J BIOPHYS BIOCHEM CYTOL 5 269 959 004 AFZELIUS BA IN: BACCETTI B 565 970 005 AFZELIUS BA SCIENCE 193 317 976 006 AFZELIUS BA J CELL BIOL 66 225 975 007 AFZELIUS BA FERTIL STERIL 29 72 978 008 AILSBY RL J PATHOL 109 75 973 009 ALLEN RA J ULTRASTRUCT RES 12 730 965 010 AMJAD H JAMA 227 1420 974 011 ANDERSEN I ARCH ENVIRON HEALTH 29 290 974 012 ANDERSON H ACTA OTOLARYNGOL STOCKH SUPPL 247 1 969 013 ARGE E LANCET 1 412 960 014 AUERBACH O N ENGL J MED 256 97 957 015 BACCETTI B J SUBMICROSC CYTOL 7 349 975 016 BALDETORP L CELL TISSUE RES 180 421 977 017 BARNES BG J ULTRASTRUCT RES 5 453 961 018 BERGSTROM WH PEDIATRICS 6 573 950 019 BISSON JP J GYN OBSTET BIOL REPR SUPP 1 4 37 975 020 BLAKE J J BIOMECH 8 179 975 021 BLOM E NATURE 209 739 966 022 BOQUIST L Z ZELLFORSCH MIKROSK ANAT 89 519 968 023 BORELL U ACTA OBSTET GYNECOL SCAND 36 22 957 024 BOWMAN BH LIFE SCI 19 1289 976 025 BREDBERG G ACTA OTOLARYNGOL STOCKH SUPPL 301 972 026 BROKAW CJ IN: INOUE S 165 975 027 BRUSIS T Z LARYNGOL RHINOL OTOL 52 883 973 028 BRYAN JHD CELL TISSUE RES 180 187 977 029 BURIAN K ACTA OTOLARYNGOL STOCKH 56 376 963 030 CAMNER P ENVIRON PHYSIOL 1 137 971 031 CAMNER P AM REV RESPIR DIS 112 807 975 032 CARRIG GB J AM VETER MED 164 1127 974 033$ CATHCART RC J NEUROPATH EXP NEUROL 23 60 964 034 CHANG KH J THORAC CARDIOVASC SURG 43 27 962 035 CHATTERJEE K J INDIAN MED ASSOC 52 389 969 036 CHERNIAK NS AM J MED 41 562 966 037 CHEVANCE LG ACTA OTOLARYNGOL STOCKH 72 121 971 038 CHUNG JW CELL TISSUE RES 175 421 976 039 CHURCHILL ED J THORAC SURG 18 279 949 040 CLYMAN MJ FERTIL STERIL 17 281 966 041 COCKAYNE EA Q J MED 7 479 938 042 COHEN S J MED SOC NJ 47 557 950 043 COLE DB J THORAC SURG 9 689 940 044 COLLIER AM INFECT IMMUN 3 694 971 045 CONOVER JH LANCET 1 1194 973 046 COOK CD AM J HUM GENET 14 290 962 047 DAHLGREN SE VIRCHOWS ARCH CELL PATHOL 11 211 972 048 DAROCZY J ZENTRALBL ALLG PATHOL 118 314 974 049 DAVID G J GYN OBSTET BIOL REPR SUPP 1 4 17 975 050 DE DUVE C SCIENCE 189 186 975 051 DELP MH J KANSAS MED SOC 47 93 946 052 DEMPSEY LC J NEUROSURG 45 53 976 053 DI MAURO S J NEUROL SCI 27 217 976 054 DOCHEZ C TIJDSCHR GASTROENTEROL 18 263 975 055 DOUEK E PROC R SOC MED 69 467 975 056 DOURMASHKIN RR J GEN VIROL 9 77 970 057 ELIASSON R N ENGL J MED 297 1 977 058 ENGSTROM H ACTA OTOLARYNGOL STOCKH SUPPL 301 75 972 059 ERNSTSON S ACTA OTOLARYNGOL STOCKH 67 521 969 060 EWERT G ACTA OTOLARYNGOL STOCKH SUPPL 200 1 965 061 FADEL HE FERTIL STERIL 27 1176 976 062 FAWCETT DW J MORPHOL 94 221 954 063 FINKLER E SCHWEIZ MED WOCHENSCHR 86 631 956 064 FLOCK A J CELL BIOL 25 1 965 065 FLOCK A ACTA OTOLARYNGOL STOCKH 83 85 977 066 FLOOD PR CELL TISSUE RES 183 281 977 067 FONTE VG J CELL BIOL 49 226 971 068 FORNATTO EJ LARYNGOSCOPE 66 1202 956 069 FOX MH ANAT REC 124 189 956 070 FRASCA JM EXP MOL PATH 9 380 968 071 FREDRICSSON B Z ZELLFORSCH MIKROSK ANAT 58 387 962 072 FRIEDMANN I LARYNGOSCOPE 81 1852 971 073 GADDUM-ROSSE P BIOL REPROD 14 605 976 074 GADDUM-ROSSE P AM J ANAT 138 26 973 075 GIBBONS IR NATURE 190 1128 961 076 GIBBONS IR J BIOPHYS BIOCHEM CYTOL 11 179 961 077 GIBBONS IR IN: INOUE S 207 975 078 GLAUM K MEITR KLIN TUBERK 91 421 938 079 GOODENOUGH UW J CELL BIOL 66 480 975 080 GUDE HE JAMA 171 1825 959 081 GUILLON G COLLOQ NATL SOC FERTIL STERIL 391 972 082 HALBERT SP GYNECOL INVEST 7 306 976 083 HANCOCK JL VET REC 67 825 955 084 HARADA Y ACTA OTOLARYNGOL STOCKH 83 284 977 085 HERS HG BIOCHEM J 86 11 963 086 HERS HG LYSOSOMES AND STORAGE DISEASE 973 087 HILDING AC ANN INTERN MED 6 227 932 088 HILDING AC ANN OTOL RHINOL LARYNGOL 52 5 943 089 HILDING AC N ENGL J MED 256 634 957 090 HILDING AC ANN OTOL RHINOL LARYNGOL 80 306 971 091 HILDING AC ANN OTOL RHINOL LARYNGOL 75 281 966 092 HOLMES LB AM J MED SCI 255 13 968 093 HOLSTEIN AF ZWANGLOSE ABH GEB NORM PATHOL 20 1 969 094 HOLSTEIN AF VIRCHOWS ARCH PATHOL ANAT 367 93 975 095 HOORN B PROG MED VIROL 11 408 969 096 HUBERMAN D ACTA PHYSIOL SCAND 99 42 977 097 HUMMEL KP J HERED 50 9 959 098 JAHNKE V Z LARYNGOL RHINOL OTOL 51 30 972 099 JAHNKE V Z LARYNGOL RHINOL OTOL 55 637 976 100 JEFFERY PK IN: BRAIN JD 5 193 977 101 JEFFERY PK IN: AHARONSON EF 253 976 102 JONES R BR MED J 2 142 972 103 KAPA E ACTA ANAT (BASEL) 95 444 976 104 KARANI S BR MED J 2 74 952 105 KARTAGENER M BEITR KLIN TUBERK 83 489 933 106 KARTAGENER M BEITR KLIN TUBERK 87 331 935 107 KATSUHARA K CHEST 61 56 972 108 KATZ M N ENGL J MED 248 730 953 109 KAWABATA I ACTA OTOLARYNGOL STOCKH 67 511 969 110 KENNEDY JR J CELL BIOL 70 80A 976 111 KIEFER BI IN: RUDDLE FH 47 973 112 KILKUCHI K J FRANC OTORHINOLARYNGOL 23 697 974 113 KILBURN KH J ULTRASTRUCT RES 60 34 977 114 KONRADOVA V FOLIA MORPHOL (PRAGUE) 16 398 968 115 KONRADOVA V FOLIA MORPHOL (PRAGUE) 23 293 975 116 KUBOTA K AM J ANAT 144 119 975 117 LATTA H J BIOPHYS BIOCHEM CYTOL 11 248 961 118 LAYTON WM J HERED 67 336 976 119 LEONARD SL ANAT REC 104 89 949 120 LINDBERG LA CELL TISSUE RES 179 121 977 121 LINDGREN E ACTA RADIOL 35 277 951 122 LOFBERG J ZOON 2 3 974 123 LOGAN WD DIS CHEST 48 613 965 124 LUDWIG H ARCH CYNAEKOL 212 380 972 125 LUFT R J CLIN INVEST 41 1776 962 126 MANN T PROC SOC STUDY FERT 9 3 958 127 MATULIONIS DH AM J ANAT 145 79 976 128 MCDOWELL EM ARCH PATHOL LAB MED 100 429 976 129 MENCO BPM MEDEDEL LANDBOUWHOGESCH WAGEN 1 77 977 130 MEYER CH FRANKF Z PATHOL 76 21 966 131 MOHRI H IN: PERRY SV 336 976 132 MORE IAR J REPROD FERTIL 47 19 976 133 MORITA I ARCH HISTOL JAP 26 341 966 134 MOSSBERG B SCAND J RESPIR DIS 59 55 978 135 MYGIND N ACTA ALLERGOL 28 9 973 136 MYKLEBUST R ANAT EMBRYOL 151 127 977 137 NAGY L BEITR KLIN TUBERK 95 26 940 138 NAKAYAMA Y J NEUROCYTOL 3 449 974 139 NIELSEN SL LAB INVEST 30 618 974 140 NICHAMIN SJ JAMA 161 966 956 141 NILSSON O UPSALA J MED SCI 77 3 972 142 NUNN JF J CELL SCI 15 537 974 143 OKUDA M ACTA OTOLARYNGOL STOCKH 76 283 973 144 OLSEN AM AM REV TUBERC 47 435 943 145 PAVELKA M ACTA ANAT (BASEL) 94 262 976 146 PEDERSEN H Z ZELLFORSCH MIKROSK ANAT 123 305 972 147 PEDERSEN H NATURE 262 494 976 148 PEDERSEN H BIOL REPROD 12 541 975 149 PEDERSEN H FERTIL STERIL 22 156 971 150 PFALLER W CELL TISSUE RES 166 91 976 151 PROCTOR DF IN: FENN WO 1 309 964 152 PROCTOR DF AM REV RESPIR DIS 115 97 977 153 RANDALL JT J PROTOZOOL SUPPL 6 30 959 154 RANDALL J IN: BEATTY RA 13 972 155 RASH JE J ULTRASTRUCT RES 29 470 969 156 REED SE INFECT IMMUN 6 68 972 157 REESE TS J CELL BIOL 25 209 965 158 RHODIN JAG AM REV RESPIR DIS SUPPL 3 93 1 966 159 ROGERS L LARYNGOSCOPE 72 456 962 160 ROMRELL LJ J ULTRASTRUCT RES 38 578 972 161 ROSS SM THESIS 971 162 ROSSMAN CM J PEDIATR 90 579 977 163 RUNGGER-BRANDLE E EXP CELL RES 107 313 977 164 RUSSAKOFF AH N ENGL J MED 235 253 946 165 SADE J ARCH OTOLARYNGOL 86 128 967 166 SAFIAN LS J FL MED ASSOC 45 1143 959 167 SANCHIS J N ENGL J MED 288 651 973 168 SANDOZ D J CELL BIOL 71 449 976 169 SANTA CRUZ R AM REV RESPIR DIS 109 458 974 170 SATIR P J CELL BIOL 26 805 965 171 SCHERFT JP J ULTRASTRUCT RES 19 546 967 172 SCHOEMPERLEN CB AM REV RESPIR DIS 88 698 968 173 SEINSCH W ACTA ANAT (BASEL) 95 537 976 174 SETHI BR J LARYNGOL OTOL 89 183 975 175 SEVTENKO G FOLIA MORPHOL (PRAGUE) 21 261 973 176 SHACK W BULL MATH BIOPHYS 34 342 972 177 SHENEFELT RE TERATOLOGY 5 103 972 178 SIELICKA-ZUBER L PRZEGL DERMATOL 61 171 974 179 SPOCK A PEDIATR RES 1 173 967 180 STEPHENS RE BIOL BULL 139 438 970 181 STOWATER JL J AM VET RADIOL SOC 17 174 976 182 SUMMERS K BIOCHIM BIOPHYS ACTA 416 153 975 183 TAIANA JA J THORAC SURG 30 34 955 184 TAMM SL PROC R SOC LOND BIOL 175 219 970 185 TENDLER H Z ERKR ATMUNGSORGANE 137 161 972 186 THOMSON ML N ENGL J MED 289 749 973 187 TIHEN JA J HERED 39 29 948 188 TOREMALM NG RHINOLOGY 13 113 975 189 TORGERSEN J ACTA RADIOL 28 17 947 190 TORGERSEN J SCHWEIZ MED WOCHENSCHR 82 770 952 191 TORIKATA C VIRCHOWS ARCH PATHOL NAT HIS 371 121 976 192 VARANO NR INT COLL SURG 33 131 960 193 VICKERS TH ARCH ENTWICKLUNGSMECH ORGAN 153 255 961 194 WALKER TR SCIENCE 153 1248 966 195 WARNER FD J CELL BIOL 63 35 974 196 WENT HA EXP CELL RES 108 63 977 197 WENT HA J THEOR BIOL 68 95 977 198 WHEATLEY DN J ANAT 101 223 967 199 WHEATLEY DN EXPERIENTIA 24 1157 968 200 WILHELM DL J PATHOL BACTERIOL 67 361 954 201 WILKINSON RF J ULTRASTRUCT RES 48 242 974 202 WILSON GB NATURE 266 463 977 203 WILSON JG AM J ANAT 85 113 949 204 WILSON JG AM J ANAT 92 153 953 205 WITMAN GB COLD SPRING HARBOR SYMP 41 969 977 206 WOLINSKY JS LAB INVEST 37 229 977 207 WONG YC LAB INVEST 24 55 971 208 WOOD RE AM REV RESPIR DIS 111 733 975 209 WOODRUFF KH AM J PATHOL 72 91 973 210 WORTHINGTON RC SCIENCE 139 221 963 211 YEATES DB ARCH DIS CHILD 51 28 976 212 YONEDA K AM REV RESPIR DIS 114 837 976 CT 1 AFZELIUS B BR MED J 2 674 979 2 AFZELIUS BA J ULTRASTRUCT RES 69 43 979 3 BACCETTI B ANDROLOGIA 12 525 980 4 NORWOOD JT BIOL REPROD 23 788 980 5 AFZELIUS BA THORAX 35 401 980 6 ROSSMAN CM CHEST 78 580 980 7 AFZELIUS BA ACTA MED SCAND 208 145 980 8 DIRKSEN ER CELL MOTIL 1 247 981 9 VANDEDONK HJM PHARM INT 2 157 981 10 FRIDAY GA PEDIATR CLIN NORTH AM 28 807 981 11 AFZELIUS BA AM J HUM GENET 33 852 981 12 ANTONELLI M ACTA PAEDIATR SCAND 70 571 981 13 TEGNER H ACTA PAEDIATR SCAND 70 629 981 14 ROSSMAN CM CHEST 80 860 981 15 KAPELLEROVA A SB LEK 83 316 981 16 PABST HF J PEDIATR 98 223 981 17 AFZELIUS BA AM REV RESPIR DIS 124 107 981 18 SVEDBERGH B ALB V GRAEF ARCH KLIN EX OPHT 215 265 981 19 DALEN H CELL TISSUE RES 220 685 981 20 HAKANSSON CH SCANN ELECTRON MICROSC 1981 93 981 21 JOHANNESSEN JV DIAGN HISTOPATHOL 5 113 982 22 VANDEDONK HJM INT J PHARMACEUT 12 57 982 23 AUGUST JR J AM ANIM HOSP ASSOC 18 822 982 24 FINKELSTEIN D BIRTH DEF ORIG ART SER 18 197 982 25 KONRADOVA V EUR J RESPIR DIS 63 516 982 26 DUDLEY JP LARYNGOSCOPE 92 297 982 27 HYBBINETTE JC ACTA OTOLARYNGOL STOCKH 93 151 982 28 HYBBINETTE JC ACTA OTOLARYNGOL STOCKH 94 121 982 29 DORUP J ANAT EMBRYOL 164 19 982 30 WILSMAN NJ AM J ANAT 164 343 982 31 WEN GY ANAT EMBRYOL 165 315 982 32 JONSSON MS N ENGL J MED 307 1131 982 33 AFZELIUS BA J SUBMICROSC CYTOL 15 111 983 34 HARD R TISSUE CELL 15 217 983 35 HARD R TISSUE CELL 15 227 983 36 MELTZER EO PEDIATR CLIN NORTH AM 30 847 983 37 NEVILLE E THORAX 38 929 983 38 AFZELIUS BA EUR J RESPIR DIS 64 280 983 39 NIELSEN MH EUR J RESPIR DIS 64 19 983 40 PEDERSEN M EUR J RESPIR DIS 64 78 983 41 ROOMANS GM EUR J RESPIR DIS 64 416 983 42 SLEIGH MA EUR J RESPIR DIS 64 157 983 43 MURPHY S PEDIATRICS 72 1 983 44 KENNEDY HG J ROY SOC MED 76 1065 983 45 LEWIS FH CHEST 83 487 983 46 MYGIND N ACTA OTOLARYNGOL STOCKH 95 688 983 47 RUNGGERBRANDLE E AM J PATHOL 110 361 983 48 EDWARDS DF J AM VET MED ASSOC 183 667 983 49 BRYAN JHD VIRCHOWS ARCH PATHOL ANAT HIS 399 265 983 50 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 51 NEWHOUSE MT SEM RESPIR MED 5 341 984 52 KATZ SM ULTRASTRUCTURAL PATHOL 6 285 984 53 LUNGARELLA G ULTRASTRUCTURAL PATHOL 6 1 984 54 CANET E PRESSE MED 13 1607 984 55 HOLLEY MC TISSUE CELL 16 287 984 56 ISAWA T J NUCL MED 25 447 984 57 RANDOLPH JF J SMALL ANIMAL PRACT 25 679 984 58 BECKER B RESPIRATION 46 180 984 59 WELCH MJ ANN ALLERGY 52 32 984 60 GREENSTONE M ARCH DIS CHILD 59 704 984 61 GREENSTONE MA ARCH DIS CHILD 59 481 984 62 WASSERMAN SJ J ALLERGY CLIN IMMUNOL 73 17 984 63 HANDEL MA J HERED 75 498 984 64 LIDOW MS J ULTRASTRUCT RES 86 18 984 65 ELOFSSON R J ULTRASTRUCT RES 87 212 984 66 ERNSTSON S ACTA OTOLARYNGOL STOCKH 97 83 984 67 FISCHER L SCHWEIZ MED WOCHENSCHR 114 610 984 68 ROSSMAN CM AM REV RESPIR DIS 129 161 984 69 WELCH MJ WEST J MED 141 509 984 70 CORNILLIE FJ PATHOL RES PRACT 178 595 984 71 AFZELIUS BA J AM VET MED ASSOC 184 560 984 72 HANDELSMAN DJ N ENGL J MED 310 3 984 73 CHARPIN D REV MALAD RESPIR 2 151 985 74 GONZALEZ S ULTRASTRUCTURAL PATHOL 8 345 985 75 AFZELIUS BA CRC CRIT REV BIOCHEM 19 63 985 76 SATO T EXP MOL PATH 43 13 985 77 AFZELIUS BA EUR J RESPIR DIS 66 173 985 78 LUNGARELLA G EUR J RESPIR DIS 66 165 985 79 BABIN RW SOUTH MED J 78 737 985 80 GREENSTONE M BR J DIS CHEST 79 9 985 81 GREENSTONE M J LARYNGOL OTOL 99 985 985 82 WU R J CELL PHYSIOL 125 167 985 83 ODOR DL AM J ANAT 174 437 985 84 GONZALEZ S PATHOL RES PRACT 180 511 985 85 CARSON JL N ENGL J MED 312 463 985 86 WANNER A CLIN CHEST MED 7 247 986 87 HOLLEY MC TISSUE CELL 18 521 986 88 AFZELIUS BA HOSP PRACT 21 73 986 89 TEICHTAHL H MED BIOL ENG COMPUT 24 193 986 90 WOODS RP ARCH NEUROL 43 1083 986 91 HO KL ACTA NEUROPATH (BERL) 70 30 986 92 RAMET J CHEST 90 138 986 93 EAVEY RD ARCH OTOLARYNG HEAD NECK SURG 112 646 986 94 SOIFER D ANN NY ACAD SCI 466 1 986 95 KAISER D ATEMWEGS LUNGENKRANKH 13 101 987 96 DELAROCHA SR PEDIATR RADIOL 17 97 987 PN 79100 RN 01080 AN 80007295 AU Barry-M-M. TI Cystic fibrosis. SO J-Am-Diet-Assoc. 1979 Oct. 75(4). P 446-9. MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, dh, di. FEMALE. GROWTH-DISORDERS: co. HUMAN. INFANT. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co. MALE. MECONIUM. PANCREATIC-DISEASES: co. RESPIRATORY-TRACT-INFECTIONS: co. SWEATING. EX Cystic fibrosis is an hereditary disorder that affects infants, children, adolescents, and young adults. Its genetic influence, signs and symptoms, diagnostic tests, nutritional management and diet therapy, respiratory infection, and growth retardation are discussed. RF 001 ROSENLUND ML PEDIATRICS 59 428 977 002 FRANCIS DEM DIETS FOR SICK CHILDREN 974 003 ANON GUIDE TO DIAGNOSIS AND MANAGE 971 004 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 005 GAYTON WF PEDIATRICS 59 888 977 006 MEARNS MB BR J HOSP MED 12 497 974 007 MORRIS W AMERICAN HERITAGE DICTIONARY 973 008 HODSON ME BR MED J 2 790 976 009 PIKE R DIGESTION AND ABSORPTION- 975 010 ROLLER RJ GASTROENTEROLOGY 72 661 977 011 BARBERO GJ IN: MANGOS JA 83 976 012 ANON CYSTIC FIBROSIS 975 013 ANON TEACHERS' GUIDE TO CF 014 BRUNS WT AM J DIS CHILD 131 71 977 015 DESAI N J PEDIATR 91 447 977 016 KASTRUP E GASTROINTESTINAL DRUGS FACTS 308B 979 017 MITCHELL H COOPERS NUTRITION IN HEALTH 380 968 018 GOODHART RS MODERN NUTRITION IN HEALTH AN 826 974 019 BERRY HK AM J DIS CHILD 129 165 975 020 HUANG NN GUIDE TO DRUG THERAPY IN PATI 979 021 FOMON SJ IN: FOMON SJ 472 974 022 WEIHOFEN DM J AM DIET ASSOC 54 206 969 023 KEATING JP PEDIATRICS 46 41 970 024 FILER LJ JR PEDIATRICS 48 483 971 025 MATTHEWS LW J PEDIATR 65 558 964 026 WILMSHURST EG PEDIATRICS 55 75 975 027 RUDOLPH AM IN: RUDOLPH AM 1563 977 028 ANON GENETICS OF CF 029 LIEBERMAN J ANN INTERN MED 82 806 975 030 NEWBURGER EH CLIN PEDIATR 12 456 973 031 WATKINS JB GASTROENTEROLOGY 73 1023 977 CT 1 BELL L J CAN DIET ASSOC 42 62 981 2 HODGES P J AM DIET ASSOC 84 664 984 PN 79101 RN 01081 AN 80006346 AU Thomsen-J. Friis-B. Jensen-K. Bak-Pedersen-K. Larsen-P-K. TI Tobramycin ototoxicity. Repeated courses of high dosage treatment in children with cystic fibrosis. SO J-Antimicrob-Chemother. 1979 May. 5(3). P 257-60. MJ ANTIBIOTICS: ae. CYSTIC-FIBROSIS: co. EAR-DISEASES: ci. PSEUDOMONAS-INFECTIONS: dt. RESPIRATORY-TRACT-INFECTIONS: dt. TOBRAMYCIN: ae. MN CHILD. DRUG-ADMINISTRATION-SCHEDULE. FEMALE. HEARING-DISORDERS: ci. HUMAN. MALE. PSEUDOMONAS-INFECTIONS: co. RESPIRATORY-TRACT-INFECTIONS: co. TOBRAMYCIN: ad, tu. AB Fifty-three patients with cystic fibrosis were treated with large doses of tobramycin sulphate, 10 mg/kg/24 h. Patients received an average of 3.2 courses, each lasting 10 to 14 days. The patients were examined with audiometric and vestibular tests, and in one patient, a 10-year-old girl, we found a transient high tone loss at 8000 Hz bilaterally that might be attributed to the tobramycin treatment. All patients had normal kidney function. High dose tobramycin treatment in children with normal kidney function gives minimal clinical risks of adverse ototoxic effects, even after repeated courses of treatment. RF 001 BODEY GP ANTIMICROB AGENTS CHEMOTHER 2 109 972 002 BREZEZINSKA M BIOCHEMISTRY NY 2 671 972 003 BRUMMET RE ARCH OTOLARYNGOL 94 59 971 004 BRUMMET RE ARCH OTOLARYNGOL 96 505 972 005 BRUMMET RE ARCH OTOLARYNGOL 101 540 975 006 DRASAR FA BR MED J 2 1284 976 007 FEDERSPIL P PROC TOBRAMYCIN SEL 8TH INT C 38 973 009 HIGGINS CE ANTIMICROB AGENTS CHEMOTHER 7 332 967 010 HOFF GE SCAND J INFECT DIS 6 333 974 011 LOGAN TB ARCH OTOLARYNGOL 99 190 974 012 MCCRAE WM SCOTT MED J 21 68 976 013 NEU HC J INFECT DIS SUPPL 134 206 976 014 PRAZMA J LARYNGOSCOPE 86 259 976 015 STUPP HF ACTA OTOLARYNGOL STOCKH SUPPL 262 1 970 016 WILSON P BR MED J 1 259 977 CT 1 RIFF L SEM PERINATOL 6 155 982 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 3 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 4 BEREZHINSKAYA VV ANTIBIOTIKI 28 218 983 5 RYBAK LP AM J OTOL 6 482 985 6 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 7 HUY PTB J CLIN INVEST 77 1492 986 8 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 79102 RN 01082 AN 80137261 AU Hughes-D-T. Pines-A. Percival-A. Stille-W. TI Round table discussion--treatment of lower respiratory tract infections with co-trimoxazole. SO J-Antimicrob-Chemother. 1979 Nov. 5(B). P 207-19. MJ RESPIRATORY-TRACT-INFECTIONS: dt. SULFAMETHOXAZOLE: tu. TRIMETHOPRIM: tu. MN BRONCHIECTASIS: dt. BRONCHITIS: co, pc. CHRONIC-DISEASE. CYSTIC-FIBROSIS: dt. DRUG-COMBINATIONS. HUMAN. LUNG-ABSCESS: dt. PNEUMONIA: co. RESPIRATORY-TRACT-INFECTIONS: co. AB Over one thousand patients with acute and chronic chest infections have been treated with a combination of trimethoprim and sulphamethoxazole. The patients may be divided into four groups and the results are summarised in this presentation. Much of the work is published elsewhere. RF 001 HUGHES DTD BR MED J 4 470 969 002 HUGHES DTD ADV IN ANTIMICROBIAL ANTINEO 1 1105 972 003 HUGHES DTD J ANTIMICROB CHEMOTHER 2 320 976 004 HUGHES DTD J ANTIMICROB CHEMOTHER 1 55 975 005 PERCIVAL A IN: BAKER FJ 154 972 006 BUSHBY SRM BR MED J 3 50 973 007 HOWARD AJ BR MED J 1 1657 978 008 LAMBERT HP SCAND J INFECT DIS SUPPL 14 262 978 009 MAY JR BR MED J 3 376 972 010 PERCIVAL A IN: BAKER FJ 154 972 011 WONG GA AM J MED 59 219 975 012 BAGG R J ANTIMICROB CHEMOTHER 4 297 978 013 PHILLIPS I IN: PHILLIPS I 37 974 014 THORNSBERRY C CURRENT MICROBIOL 1 51 978 PN 79103 RN 01083 AN 79151301 AU Frydman-M-I. TI Epidemiology of cystic fibrosis: a review. SO J-Chronic-Dis. 1979. 32(3). P 211-9. (REVIEW). MJ CYSTIC-FIBROSIS: oc. MN ADULT. AUSTRALIA. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: fg, pp. EUROPE. FEMALE. FERTILITY. HUMAN. MALE. REVIEW. UNITED-STATES. AB Three main reasons have been advanced for the degree of ignorance and confusion surrounding epidemiologic measures of the incidence and prevalence of Cystic Fibrosis. (CF) Relatively recent recognition of CF as a specific disease entity. Wide range of clinical manifestations, presentation as a multisystem disease, and great natural variation in the severity of involvement of various organs. Past difficulty in establishing a diagnosis based on sound laboratory evidence, this being largely due to the lack of a screening test that had gained wide acceptance among diagnosticians, and inaccuracies in the performance and interpretation of the sweat test. In addition to these factors, there are a number of methodological deficiencies and questionable assumptions in several of the papers that have here been reviewed. It may be noteworthy that the two sets of authors whose research design most closely matched being aimed at providing multiple ascertainment of cases and cross-checks of validity, have arrived at nearly the same estimated incidence of this particular disorder (1/3700 and 1/3800 respectively). The issue of selection and diagnostic criteria is one that has important implications for epidemiologic studies of cystic fibrosis. Particularly in a disease like CF, where the spectrum of clinical manifestations and severity of involvement is so broad, estimates of incidence will vary according to the criteria by which cases are ascertained and included. A greater degree of uniformity in standards than has been employed in previous studies is necessary before comparisons can meaningfully be made. There are good grounds for regarding most estimates of CF incidence to be minimum confirmed figures, and this is particularly so where the research design ensures that multiple ascertainments of the same case will be identifiable. Higher estimates are made where several sources of error (respondent bias, generalization of hospital data to the wider population, multiple ascertainments of the same patient) are not recognised. RF 001 ANON CF GUIDELINES FOR HEALTH PERS 976 002 DI SANTAGNESE PA IN: DOWNEY JA 25 974 003 FANCONI G WIEN MED WOCHENSCHR 86 753 936 004 ANDERSEN DH AM J DIS CHILD 56 344 938 005 BLACKFAN KD J PEDIATR 13 627 938 006 ANDERSEN DH CHILDREN 7 11 960 007 WARWICK WJ MINN MED 52 1567 969 008 SHWACHMAN H PEDIATRICS 36 689 965 009 SHWACHMAN H AM J DIS CHILD 96 6 958 010 LIFSCHITZ M ARCH FOUND THANATOLOGY 1 23 969 011 POGUE RE MINN MED 52 1551 969 012 BURTON L HEALTH VISITOR 456 186 973 013 LAWLER RH CAN MED ASSOC J 94 1043 966 014 SHWACHMAN H IN: GELLIS SS 237 970 015 SHWACHMAN H AM J DIS CHILD 92 347 956 016 LAPEY A IN: GELLIS SS 221 976 017 MAY CD J PEDIATR 34 663 949 018 MATTHEWS LW MINN MED 52 1506 969 019 CHILDRESS J IN: PATTERSON PR 973 020 ANON GAP CONF REP CHRON BRONC CF 1 977 021 TAUSSIG LM J PEDIATR 82 380 973 022 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 023 ANDERSEN DH J CHRON DIS 7 58 958 024 DI SANTAGNESE PA JAMA 172 2065 960 025 MCPARTLIN JF ARCH DIS CHILD 47 207 972 026 HIATT RB SURG GYNECOL OBSTET 87 317 948 027 KULCZYCKI LL N ENGL J MED 257 203 957 028 DI SANTAGNESE PA AM J MED 21 406 956 029 DI SANTAGNESE PA PEDIATRICS 24 313 959 030 ROSENLUND ML ANN INTERN MED 78 959 973 031 LANDAU LI AM REV RESPIR DIS 108 593 973 032 STERN RC J PEDIATR 89 406 976 033 TECKLIN JS PHYS THER 55 1081 975 034 HOLSCLAW DS GAP CONF REP CHRON BRONC CF 13 977 035 DENNING CR PEDIATRICS 41 7 968 036 KAPLAN E N ENGL J MED 279 65 968 037 HOLSCLAW DS J UROL 106 568 971 038 ANON GAP CONF REP PROB REPRO PHYSI 9 975 039 DI SANTAGNESE PA N ENGL J MED 279 103 968 040 KOCH E INTERNIST (BERL) 1 35 960 041 FEIGELSON J ARCH FR PEDIATR 26 937 969 042 TAUSSIG LM N ENGL J MED 287 586 972 043 MEYEROWITZ JH IN: PATTERSON PR 40 973 044 HOLSCLAW DS GAP CONF REP PROB REPRO PHYSI 1 975 045 DENNING CR GAP CONF REP PROB REPRO PHYSI 2 975 046 GRAND RJ JAMA 195 993 966 047 NOVY MJ OBSTET GYNECOL 30 530 967 048 SIEGEL B OBSTET GYNECOL 16 438 960 049 POLGAR G AM REV RESPIR DIS 85 319 962 050 BALL RE N ENGL J MED 265 31 961 051 ROSENOW EC 3RD JAMA 203 227 968 052 SALHANICK H GAP CONF REP PROB REPRO PHYSI 4 975 053 VANDE WIELE R GAP CONF REP PROB REPRO PHYSI 5 975 054 LOWE CU AM J DIS CHILD 78 349 949 055 DANKS DM ANN HUM GENET 28 323 965 056 GOODMAN HO AM J HUM GENET 4 59 952 057 DI SANTAGNESE PA N ENGL J MED 277 1287 967 058 STEINBERG AG AM J HUM GENET 12 416 960 059 SELANDER P ACTA PAEDIATR SCAND 51 65 962 060 BAUMANN T HELV PAEDIATR ACTA SUPPL 8 13 1 958 061 ROBERTS GBS ANN HUM GENET 24 127 960 062 FANCONI G SCHWEIZ MED WOCHENSCHR 74 85 944 063 BRODY H NY STATE J MED 41 1256 941 065 KULCZYCKI LL CLIN PEDIATR 3 692 964 066 KULCZYCKI LL IN: PATTERSON PR 117 973 067 WANG CI N ENGL J MED 279 1216 968 068 HARRIS RL PEDIATRICS 41 733 968 069 HAMAMOTO E NIPON SHONIKAGAKKAI ZASSHI 65 502 961 070 YAMAMOTO T SHONIKA RINSHO 10 1003 957 071 LEVIN S IN: GOLDSCHMIDT E 294 963 072 KANDAR HR ANTISEPTIC 58 863 961 073 SALAM M LEB MED J 15 61 962 074 WRIGHT SW AM J HUM GENET 20 157 968 075 MACMAHON B EPIDEMIOLOGY PRINCIPLES AND M 970 076 ANDERSEN DH AM J DIS CHILD 72 62 946 077 HARPER MH MED J AUST 1 137 949 078 SUDNOW D PASSING ON THE SOCIAL ORGANIS 76 967 079 EVANS AE N ENGL J MED 278 138 968 080 MERRITT AD J LAB CLIN MED 60 998 962 081 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 082 KRAMM ER PEDIATRICS 28 128 961 083 GREGG RH PEDIATRICS 36 700 965 084 GIBSON LE CLIN PEDIATR 12 450 973 CT 1 SING CF AM J MED GENET 13 179 982 2 LONGNECKER DS AM J PATHOL 107 103 982 3 KUMMER F ATEMWEGS LUNGENKRANKH 10 567 984 4 GORTMAKER SL PEDIATR CLIN NORTH AM 31 3 984 PN 79104 RN 01084 AN 79151302 AU Kerner-J. Harvey-B. Lewiston-N. TI The impact of grief: a retrospective study of family function following loss of a child with cystic fibrosis. SO J-Chronic-Dis. 1979. 32(3). P 221-5. MJ CYSTIC-FIBROSIS: px. DEATH. FAMILY. GRIEF. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. FEMALE. GENETIC-COUNSELING. HUMAN. INFANT. LIFE-CHANGE-EVENTS. MALE. PARENTS. RELIGION. RETROSPECTIVE-STUDIES. AB The impact of the death of a cystic fibrosis child on subsequent family function was evaluated in a retrospective study of 16 families. In findings similar to that reported for another model of fatal childhood illness, leukemia, there was a high incidence of emotional and medical problems in the parents. There was an unexpectedly high incidence (7/16) of incomplete mourning, i.e., maintenance of the child's room as a shrine or weekly grave visit for more than six months. Siblings had less difficulty in adjusting to the loss than that reported for the siblings of leukemic children. It is suggested that the prolonged course of a disease with a hopeless prognosis prepares the family for the child's death but also retards the mourning process. The family may need counseling to ensure that mourning is completed. RF 001 GOLDBERG IK IN: PATTERSON PR 168 973 002 TURK J PEDIATRICS 34 67 964 003 LAWLER RH CAN MED ASSOC J 94 1043 966 004 MCCOLLUM AT J PEDIATR 77 571 970 005 TROPAUER A AM J DIS CHILD 119 424 970 006 GAYTON WF AM J DIS CHILD 126 856 973 007 FARKAS A IN: PATTERSON PR 202 973 008 BINGER CM N ENGL J MED 280 414 969 009 MEYEROWITZ JH SOC SCI MED 1 249 967 010 HILGARD JR INTERN PSYCHIATRY CLIN 6 197 969 011 KOLB LC MODERN CLINICAL PSYCHIATRY 56 973 012 FISCHHOFF J J PEDIATR 88 140 976 013 MCCRAE WM LANCET 2 141 973 CT 1 SPINETTA JJ CANCER 53 2330 984 2 DEWET B S AFR MED J 65 526 984 3 LEWISTON NJ SEM RESPIR MED 6 321 985 4 MILES MS NURS RES 34 76 985 5 WILSON AL AM J DIS CHILD 139 1235 985 PN 79105 RN 01085 AN 79194554 AU Spicer-K-M. Allen-R-C. Hallett-D. Buse-M-G. TI Synthesis of hemoglobin Aic and related minor hemoglobin by erythrocytes. In vitro study of regulation. SO J-Clin-Invest. 1979 Jul. 64(1). P 40-8. MJ ERYTHROCYTES: me. HEMOGLOBIN-A: bi. HEMOGLOBINS: me. MN ADULT. CHILD. CYSTIC-FIBROSIS: bl. DIABETES-MELLITUS: bl. FETAL-HEMOGLOBIN: me. GLUCOSE: pd. HEMODIALYSIS. HEMOGLOBIN-C: me. HEMOGLOBIN-SICKLE: me. HEXOSES: bl. HUMAN. HYDROGEN-ION-CONCENTRATION. IN-VITRO. INSULIN: pd. MALE. PHOSPHORYLATION. TEMPERATURE. TIME-FACTORS. SUPPORT-U-S-GOVT-P-H-S. AB Factors that influence hemoglobin (Hb)Aic synthesis by intact erythrocytes were studied in vitro. After incubation cells were lysed, and hemoglobins were separated by isoelectric focusing on polyacrylamide slab gels and quantitated by microdensitometry. HbAic increased with time, glucose concentrations (5-500 mM) and incubation temperature (4 degrees-37 degrees C). Low temperatures allowed prolonged incubations with minimal hemolysis. At 4 degrees C HbAic increased linearly with time for 6 wk; after incubation at the highest glucose concentration, HbAic comprised 50% of total hemoglobin. Insulin (1 and 0.1 mU/ml) did not affect HbAic synthesis in vitro. In addition to glucose, galactose and mannose, but not fructose, served as precursors to HbAic. A good substrate for hexokinase (2-deoxy-glucose) and a poor hexokinase substrate (3-O-methyl-glucose), were better precursors for HbAic synthesis than glucose, suggesting that enzymatic phosphorylation of glucose is not required for HbAic synthesis. Autoradiography after erythrocyte incubation with 32P-phosphate showed incorporation of radioactivity into HbAia1 and HbAia2, but not HbAib, Aic, or A. Acetylated HbA, generated during incubation with acetylsalicylate, migrated anodal to HbAic and clearly separated from it. Erythrocytes from patients with insulinopenic diabetes mellitus synthesized HbAic at the same rate as controls when incubated with identical glucose concentrations. Likewise, the rate of HbAic synthesis by erythrocytes from patients with cystic fibrosis and congenital spherocytosis paralleled controls. When erythrocytes from cord blood and from HbC and sickle cell anemia patients were incubated with elevated concentrations of glucose, fetal Hb, HbC, and sickle Hb decreased whereas hemoglobins focusing at isoelectric points near those expected for the corresponding glycosylated derivatives appeared in proportionately increased amounts. RF 001 BUNN HF J CLIN INVEST 57 1652 976 002 KOENIG RJ DIABETES 25 230 976 003 KOENIG RJ N ENGL J MED 295 417 976 004 GABBAY KH J CLIN ENDOCRINOL METAB 44 859 977 005 GONEN BA LANCET 1 734 977 006 STEVENS VJ J BIOL CHEM 252 2998 977 007 HANEY DN PROC NAT ACAD SCI USA 73 3534 976 008 BUNN HF SCIENCE 200 21 978 009 DIXON HBF BIOCHEM J 129 203 972 010 BUNN HF BIOCHEM BIOPHYS RES COMMUN 67 103 975 011 KOENIG RJ J BIOL CHEM 252 2992 977 012 SPICER KM DIABETES 27 384 978 013 BEUTLER E IN: GREENWALT TJ 189 974 014 BEUTLER E J LAB CLIN MED 80 723 972 015 BRIDGES KR J CLIN INVEST 56 201 975 016 TRIVELLI LA N ENGL J MED 284 353 971 017 SCHNEK AG J AM CHEM SOC 83 1472 961 018 DRABKIN DL J BIOL CHEM 164 703 946 019 STEVENS VJ PROC NAT ACAD SCI USA 75 2918 978 020 PAULSEN EP DIABETES 25 890 976 021 MORGAN HE CURR TOP MEMBRANES TRANSP 4 255 973 022 MCDONALD MJ J BIOL CHEM 253 2327 978 023 BUNN HF HUMAN HEMOGLOBIN 8 977 024 BASSETT PY BLOOD 51 971 978 025 ROSE IA J BIOL CHEM 239 12 964 026 BEUTLER E RED CELL METABOLISM 123 975 027 KRISHNAMOORTHY R FEBS LETTERS 77 99 977 028 DOLHOFER R FEBS LETTERS 85 86 978 029 LEFEVRE PG PHARMACOL REV 13 39 961 030 CANTOR SM J AM CHEM SOC 62 2113 940 031 LOS JM J AM CHEM SOC 78 1564 956 032 FLUCKIGER R FEBS LETTERS 71 356 976 033 ZIPPER H BIOCHIM BIOPHYS ACTA 282 311 972 034 GAMBHIR KK DIABETES 27 701 978 035 GRAF R DIABETES SUPPL 26 368 977 036 ABDELLA PM BIOCHIM BIOPHYS ACTA 490 462 977 037 BUNN HF J CLIN INVEST 49 1088 970 CT 1 FITZGERALD MD AM J HEMATOL 9 311 980 2 SVENDSEN PA DIABETOLOGIA 19 130 980 3 BERNSTEIN RE CLIN CHEM 26 174 980 4 MENARD L CLIN CHEM 26 1598 980 5 ROMEN W KLIN WSCHR 58 1013 980 6 BERGER W SCHWEIZ MED WOCHENSCHR 110 485 980 7 BARTOSZ G MECH AGEING DEV 17 237 981 8 MONNIER LH DIABETOLOGIA 20 12 981 9 BOUCHER BJ DIABETOLOGIA 21 34 981 10 DEAN PDG DIABETOLOGIA 21 579 981 11 MORTENSEN HB SCAND J CLIN LAB INVEST 41 451 981 12 FADEL HE AM J OBSTET GYNECOL 139 397 981 13 FADEL HE AM J OBSTET GYNECOL 141 704 981 14 GOEBEL FD RES EXP MED 179 133 981 15 PEREJDA AJ COLLAGEN RELAT RES 2 83 982 16 ESCHWEGE E DIABETE METAB 8 137 982 17 LEUTENEGGER M DIABETE METAB 8 249 982 18 UITTO J CONNECT TISS RES 10 287 982 19 POWELL HC CLIN BIOCHEM 15 133 982 20 PEER G ISR J MED SCI 18 960 982 21 BARTOSZ G MECH AGEING DEV 20 223 982 22 VIALETTES B DIABETOLOGIA 22 264 982 23 HOWARD CF DIABETES 31 1105 982 24 ARNQVIST H SCAND J CLIN LAB INVEST 42 265 982 25 PANZER S BLOOD 59 1348 982 26 BACHEM MG KLIN WSCHR 60 497 982 27 SMITH RJ J CLIN INVEST 69 1164 982 28 BASSET P J CHROMATOGR 227 267 982 29 MORTENSEN HB BIOCHIM BIOPHYS ACTA 707 154 982 30 MEHL TD DIABETES CARE 6 34 983 31 ZELLER WP PEDIATR RES 17 200 983 32 COHEN MP EXP GERONTOL 18 461 983 33 WETTRE S DIABETOLOGIA 24 148 983 34 CZECH A J CHRON DIS 36 803 983 35 SANDHU RS CLIN BIOCHEM CONTEM THEO TECH 3 215 984 36 MIEDEMA K ANN CLIN BIOCHEM 21 2 984 37 OLESEN HA SCAND J CLIN LAB INVEST 44 329 984 38 DAVIDOFF P REV MED CHIL 112 3 984 39 COSSU G J CHROMATOGR 307 103 984 40 KUMAR A BIOCHEM MED 34 112 985 41 ABRAHAM EC HEMOGLOBIN 10 173 986 PN 79106 RN 01086 AN 79151454 AU Seale-T-W. Thirkill-H. Tarpay-M. Flux-M. Rennert-O-M. TI Serotypes and antibiotic susceptibilities of Pseudomonas aeruginosa isolates from single sputa of cystic fibrosis patients. SO J-Clin-Microbiol. 1979 Jan. 9(1). P 72-8. MJ CYSTIC-FIBROSIS: mi. PSEUDOMONAS-AERUGINOSA: cl. SPUTUM: mi. MN ADOLESCENCE. ADULT. ANTIBIOTICS: pd. CHILD. CHILD-PRESCHOOL. DRUG-RESISTANCE-MICROBIAL. HAEMOPHILUS-INFLUENZAE: ip. HUMAN. PSEUDOMONAS-AERUGINOSA: de. SEROTYPING. STAPHYLOCOCCUS-AUREUS: ip. SUPPORT-U-S-GOVT-P-H-S. AB A phenotypic characterization of Pseudomonas aeruginosa from single sputum samples of 21 typical cystic fibrosis patients indicated a high frequency of heterogeneity among isolates on the basis of differences in antibiotic resistance, colony morphology, pigmentation, and serotype. Two or more isolates with different but stable susceptibilities to carnecillin, gentamycin, streptomycin, tetracycline, chloramphenicol, and sulfamethoxazole plus trimethoprim were detected in 38% of the sputa. Differences generally were independent of the mucoid state of the strain. O- antigen group determination with the Difco typing set showed that two or more serologically distinct strains were present in 10/21 sputum specimens. Nonmucoid derivatives of mucoid isolates almost always retained both the antibiotic susceptibilities and serotype of their parent strain. These data suggest that cystic fibrosis patients may be cocolonized/coinfected by difference strains of P. aeruginosa more frequently than generally believed. Alternatively, phenotypically distinct strains from a single patient might arise as phenotypic dissociants from a single infecting strain. Because of the frequency and multiplicity of phenotypically distinct P. aeruginosa isolates which we obtained from our cystic fibrosis patients, it is important to select multiple isolates from sputum cultures for antimicrobial susceptibility testing so as to assess adequately the susceptibility of this organism to antibiotic therapy in cystic fibrosis. We recommend that several colonies of each distinguishable colony type of P. aeruginosa be pooled for the antibiogram. RF 001 BAUER AW AM J CLIN PATHOL 45 493 966 002 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 003 BOWMAN BH IN: MANGOS JA 277 976 004 BOXERBAUM B AM REV RESPIR DIS 108 777 973 005 DIAZ F J INFECT DIS 121 269 970 006 DI SANTAGNESE PA N ENGL J MED 295 481 976 007 DOGGETT RG J PEDIATR 68 215 966 008 GRIEBLE HG N ENGL J MED 282 531 970 009 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 010 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 011 LEVISON H IN: MANGOS JA 3 976 012 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 013 SCHWARZMANN S INFECT IMMUN 3 762 971 014 TALAMO RC IN: MANGOS JA 195 976 015 WILLIAMS RJ J MED MICROBIOL 6 409 973 016 ZIERDT CH J BACTERIOL 87 1003 964 017 ZIERDT CH J CLIN MICROBIOL 1 521 975 CT 1 DEMKO CA CURRENT MICROBIOL 4 69 980 2 MARKOWITZ SM J ANTIMICROB CHEMOTHER 6 251 980 3 GODFREY AJ ANTIMICROB AGENTS CHEMOTHER 19 705 981 4 IRVIN RT ANTIMICROB AGENTS CHEMOTHER 19 1056 981 5 WILSON LA AM J OPHTHALMOL 92 546 981 6 MARKS MI J PEDIATR 98 173 981 7 SEALE TW ANN CLIN LAB SCI 12 415 982 8 JANDA JM J CLIN MICROBIOL 15 178 982 9 SHEEHAN DJ J CLIN MICROBIOL 15 926 982 10 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 11 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 12 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 13 ANON LANCET 2 257 983 14 GOVAN JRW REV INFECT DIS 5 S874 983 15 JAGGER KN J CLIN MICROBIOL 17 55 983 16 FLOURNOY DJ METH FIND EXP CLIN PHARMACOL 6 487 984 17 CHAN R J CLIN MICROBIOL 19 8 984 18 MCNEILL WF AM J CLIN PATHOL 81 742 984 19 GOLD R PEDIATR INFECT DIS 4 172 985 20 LUZAR MA INFECT IMMUN 50 577 985 21 KOMIYAMA K ORAL SURG 59 590 985 22 BERKIN KE EUR J RESPIR DIS 67 103 985 23 PIER GB J INFECT DIS 151 575 985 24 PACIFICO L J INFECT DIS 152 852 985 25 MAYO MS J CLIN MICROBIOL 24 372 986 26 PIER GB J CLIN MICROBIOL 24 189 986 27 KOMIYAMA K CAN J MICROBIOL 33 221 987 28 OGLE JW J INFECT DIS 155 119 987 PN 79107 RN 01087 AN 79174043 AU Lloyd-J-K. TI Dietary problems associated with the care of chronically sick children. SO J-Hum-Nutr. 1979 Apr. 33(2). P 135-9. MJ CHRONIC-DISEASE: dh. DIET. MN CELIAC-DISEASE: dh. CHILD. CYSTIC-FIBROSIS: dh. DIABETES-MELLITUS-INSULIN-DEPENDENT: dh. DIABETIC-DIET. HEART-DEFECTS-CONGENITAL: dh. HUMAN. KIDNEY-FAILURE-CHRONIC: dh. MENINGOMYELOCELE: dh. MENTAL-RETARDATION: dh. PHENYLKETONURIA: dh. AB The dietary problems associated with the care of chronically sick children can be considered under two broad headings. First, there are the problems in those children for whom diet is an essential part of the treatment of their chronic disorder; and second there are nutritional complications which may occur in children with chronic diseases in which diet does not have a specific role in treatment, and is thus often neglected or considered only at a later stage of management. Many of the problems are of course common to both situations. This paper will review briefly some of the difficulties, illustrating these with specific examples; no attempt will be made to describe or discuss the treatment of any particular disorder. RF 001 ALLAN JD AM J DIS CHILD 126 22 973 002 BIRBECK JA ARCH DIS CHILD 51 467 976 003 COURT JM J HUM NUTR 32 285 978 004 HAYES-ALLEN MC BR J PREV SOC MED 27 192 973 005 LINDE LM J PEDIATR 70 413 967 006 PACKER SM ARCH DIS CHILD 53 449 978 007 RICKARD K PEDIATR CLIN NORTH AM 24 157 977 008 ROBINSON MJ ARCH DIS CHILD 50 962 975 009 SMITH I IN: MCLAREN DS 263 976 010 SMITH I BR MED J 2 723 978 CT 1 FISCHER M J MENT DEFIC RES 29 373 985 PN 79108 RN 01088 AN 79174045 AU Francis-D-E. TI Inborn errors of metabolism: the need for sugar. SO J-Hum-Nutr. 1979 Apr. 33(2). P 146-54. MJ DIETARY-CARBOHYDRATES. METABOLISM-INBORN-ERRORS: dh. MN ADOLESCENCE. AMINO-ACID-METABOLISM-INBORN-ERRORS: dh. CARBOHYDRATE-METABOLISM-INBORN-ERRORS: dh. CHILD. CYSTIC-FIBROSIS: dh. DENTAL-CARIES: et. DIETARY-FATS. HUMAN. LIPID-METABOLISM-INBORN-ERRORS: dh. MAPLE-SYRUP-URINE-DISEASE: dh. METABOLISM-INBORN-ERRORS: co. PHENYLKETONURIA: dh. EX Inborn errors of metabolism are conditions in which the body fails to metabolise certain chemicals due to an inherited enzyme deficiency. The inborn errors of metabolism considered in this paper are those which affect the metabolism of dietary nutrients and which require manipulation of the carbohydrate intake. In these cases the need for sugar - either as a source of energy when other energy-supplying nutrients have to be suppressed, or for the control of hypoglycaemia - is vitally important. Inborn errors of protein metabolism include phenylketonuria, maple-syrup urine disease, organic acidaemias, and hyperammonaemia. Inborn errors of fat metabolism include abetalipoprotinaemia, type I lipoprotein-lipase deficiency, and lymphangiectasia. Inborn errors of carbohydrate metabolism include diabetes, galactosaemia, fructosaemia, and glucose-6-phosphate deficiency (type I glycogen storage disease). Inborn errors of multiple nutrients include cystic fibrosis. Dietary carbohydrate, psychological considerations, and dental caries are examined. RF 001 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 002 CLAYTON BE IN: DICKERSON JWT 29 978 003 COURT JM YOUR CHILD HAS DIABETES 972 004 CORNBLATH M DISORDERS CARBOHYD METAB INFA 115 966 005 CRAIG O CHILDHOOD DIABETES AND ITS MA 977 006 CRIGLER JF CIBA FOUND SYMP 55 331 978 007 FRANCIS DEM DIETS FOR SICK CHILDREN 974 008 GREENE HL N ENGL J MED 294 1125 976 009 HENNON DK JADA 95 965 977 010 LEVINE RS BR DENT J 137 429 974 011 LLOYD JK IN: MCLAREN DS 254 976 012 LLOYD JK IN: PEETERS H 331 972 013 PARTIN JC PEDIATR RES 8 384 974 014 SMALLEY CA ARCH DIS CHILD 53 477 978 015 SMITH I BR MED J 2 723 978 016 SMITH I IN: MCLAREN DS 263 976 017 STEPHEN KW BR DENT J 144 202 978 018 WINTER GB CARIES RES 8 256 974 019 YASSA JG ARCH DIS CHILD 53 777 978 PN 79109 RN 01089 AN 80095419 AU Vymola-F. Trojanova-M. Pillich-J. Lochmann-O. TI Therapeutic possibilities in Pseudomonas infections. SO J-Hyg-Epidemiol-Microbiol-Immunol (Praha). 1979. 23(1). P 114-7. MJ PSEUDOMONAS-INFECTIONS: th. MN ACUTE-DISEASE. ANTIBIOTICS: tu. BACTERIOPHAGES. CYSTIC-FIBROSIS: th. GAMMA-GLOBULINS: ad, tu. HUMAN. IMMUNIZATION-PASSIVE. PSEUDOMONAS-INFECTIONS: dt. TOXOIDS: ad, tu. IMMUNOTHERAPY. AB Acute pseudomonas infections require treatment with antibiotics producing a bactericidal effect. The most useful are gentamicin, tobramycin, sisomicin and polymyxin B. In resistant strains, amikacin is indicated in addition. Carbenicillin, ticarcillin, carfenicillin or azocillin should never be given alone but in combination with some of the above preparations. Other drugs, such as chloramphenicol, tetracycline or streptomycin, though effective in vitro, should be avoided. Chemotherapy may be complemented by passive immunization either with hyperimmune specific gama globulin or hyperimmune plasma. A programmatic item of combined treatment is active immunization, especially with toxoid vaccine. Chronic processes are not, perhaps with the exception of urinary infections, suitable for antibiotic therapy. For this reason effective polyvalent vaccines should be developed from appropriate strains. It is now certain that in infections caused by mucous strains (most frequently encountered in cystic fibrosis) the vaccine should be prepared from these strains, since they have distinct functional and antigenic characteristics. PN 79110 RN 01090 AN 80161290 AU Thomassen-M-J. Demko-C-A. Boxerbaum-B. Stern-R-C. Kuchenbrod-P-J. TI Multiple of isolates of Pseudomonas aeruginosa with differing antimicrobial susceptibility patterns from patients with cystic fibrosis. SO J-Infect-Dis. 1979 Dec. 140(6). P 873-80. MJ CYSTIC-FIBROSIS: dt. PSEUDOMONAS-AERUGINOSA: ip. MN ADOLESCENCE. ADULT. CARBENICILLIN: tu. CHILD. CHILD-PRESCHOOL. GENTAMICINS: tu. HUMAN. INFANT. KANAMYCIN: tu. MICROBIAL-SENSITIVITY-TESTS. TETRACYCLINE: tu. TICARCILLIN: tu. TIME-FACTORS. TOBRAMYCIN: tu. SUPPORT-U-S-GOVT-P-H-S. AB Clinical isolates of Pseudomonas aeruginosa from patients with cystic fibrosis were studied in an effort to determine the unique characteristics of the infecting strains and to elucidate the pattern of colonization. Of 413 patients studied, 81% were chronically infected with P. aeruginosa. Patients from whom P. aeruginosa was never or only occasionally isolated were in better clinical condition than the chronically infected patients. Isolates were classified into six morphologic varieties: classic, rough, mucoid, gelatinous, dwarf, and enterobacter. Most patients had two or more of these varieties. Such multiple varieties from the same individual were of the same serotype but often differed in antibiotic susceptibility as determined by both the disk and the minimal inhibitory concentration methods. These differences were apparent when mucoid strains were compared with nonmucoid strains and when nonmucoid strains were compared with one another. Studies of antibiotic susceptibility should be performed on each morphologically different type of P. aeruginosa obtained from patients with cystic fibrosis. RF 001 DOERSHUK CF IN: GREEN M 707 968 002 DOGGETT RG APPL MICROBIOL 18 936 969 003 MARTIN DR J MED MICROBIOL 6 111 973 004 ZIERDT CH J BACTERIOL 87 1003 964 005 GOVAN JRW J MED MICROBIOL 8 513 975 006 DIAZ F J INFECT DIS 121 269 970 007 WILLIAMS RJ J MED MICROBIOL 6 409 973 008 BERGAN T ACTA PATH MICROBIOL SCAND (B) 83 553 975 009 DOERSHUK CF PEDIATRICS 36 675 965 010 SHWACHMAN H PEDIATRICS 36 689 965 011 WAHBA AH J GEN MICROBIOL 38 329 965 012 BAUER AW AM J CLIN PATHOL 45 493 966 013 BAILEY WR DIAGNOSTIC MICROBIOLOGY 313 974 014 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 015 REYNOLDS HY ANN INTERN MED 82 819 975 016 GOVAN JRW J ANTIMICROB CHEMOTHER 4 233 978 017 ZIERDT CH J CLIN MICROBIOL 1 521 975 CT 1 DEMKO CA CURRENT MICROBIOL 4 69 980 2 JANDA JM J CLIN MICROBIOL 12 626 980 3 IRVIN RT ANTIMICROB AGENTS CHEMOTHER 19 1056 981 4 BLACKWOOD LL INFECT IMMUN 32 443 981 5 THOMASSEN MJ INFECT IMMUN 33 512 981 6 PENNINGTON JE J CLIN INVEST 68 1140 981 7 MARKS MI J PEDIATR 98 173 981 8 MACONE AB N ENGL J MED 304 1445 981 9 JANDA JM J CLIN MICROBIOL 15 178 982 10 SHEEHAN DJ J CLIN MICROBIOL 15 926 982 11 PUGASHETTI BK J CLIN MICROBIOL 16 686 982 12 OHMAN DE INFECT IMMUN 37 662 982 13 THOMASSEN MJ INFECT IMMUN 38 802 982 14 WINNIE GB INFECT IMMUN 38 1088 982 15 SPEERT DP J PEDIATR 101 227 982 16 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 17 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 18 COSTERTON JW REV INFECT DIS 5 S867 983 19 GOVAN JRW REV INFECT DIS 5 S874 983 20 BOROWSKI RS CURRENT MICROBIOL 9 25 983 21 NINANE G INFECTION 11 S 16 983 22 JAGGER KN J CLIN MICROBIOL 17 55 983 23 BASSETTI D CURR THER RES CLIN EXP 33 874 983 24 JOHNSON SR OBSTET GYNECOL 61 S 2 983 25 FLOURNOY DJ METH FIND EXP CLIN PHARMACOL 6 487 984 26 CHAN R J CLIN MICROBIOL 19 8 984 27 MCNEILL WF AM J CLIN PATHOL 81 742 984 28 THOMASSEN MJ J PEDIATR 104 352 984 29 GOLD R PEDIATR INFECT DIS 4 172 985 30 KOMIYAMA K ORAL SURG 59 590 985 31 THOMASSEN MJ AM REV RESPIR DIS 131 791 985 32 PACIFICO L J INFECT DIS 152 852 985 33 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 34 GILLJAM H THORAX 41 641 986 35 PITT TL J ROY SOC MED 79 13 986 36 LEVY J J PEDIATR 108 841 986 37 REED MD PEDIATR PULMONOL 3 101 987 38 DUNNE WM DIAGN MICROBIOL INFECT DIS 6 165 987 39 SPEERT DP J HOSP INFECT 9 11 987 40 KOMIYAMA K CAN J MICROBIOL 33 221 987 41 OGLE JW J INFECT DIS 155 119 987 PN 79111 RN 01091 AN 80007721 AU Pennington-J-E. Wolff-S-M. Puziss-M. TI Summary of a workshop on infections in patients with cystic fibrosis. SO J-Infect-Dis. 1979 Aug. 140(2). P 252-6. MJ BACTERIAL-INFECTIONS: co. CYSTIC-FIBROSIS: et. MN ANTIBODIES-BACTERIAL: an. CONGRESSES. HUMAN. PSEUDOMONAS-AERUGINOSA: im, ip. SPUTUM: mi. NATIONAL-INSTITUTES-OF-HEALTH-U-S. EX Cystic fibrosis is currently the most common fatal hereditary disease of the Caucasian race. The lack of effective oral antibiotics against Pseudomonas aeruginosa precludes either systemic treatment or suppression, in an outpatient setting, of the chronic pseudomonas bronchitis typically seen in CF patients. Because of the central role of infection, and particularly pseudomonas infection, in the sequence of events that leads to respiratory failure and death in CF, currently available information regarding the pathogenesis of infections caused by P. aeruginosa was emphasized and discussed at a workshop on infections in patients with CF, sponsored by the National Institute of Allergy and Infectious Diseases in Bethesda, Md., on January 9 and 10, 1979. CT 1 CASSINO RJJ PEDIATR RES 14 1212 980 2 FICK RB J IMMUNOL METH 38 103 980 3 BANNATYNE RM ANTIMICROB AGENTS CHEMOTHER 20 493 981 4 MARKS MI J PEDIATR 98 173 981 5 OBOYLE CP IR J MED SCI 150 286 981 6 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 7 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 8 SUTER S J INFECT DIS 149 523 984 9 JEWETT CV J PEDIATR 106 669 985 10 SUTER S J INFECT DIS 153 902 986 PN 79112 RN 01092 AN 79152690 AU Dreisin-R-B. Mostow-S-R. TI Sulfhydryl-mediated depression of ciliary activity: an adverse effect of acetylcysteine. SO J-Lab-Clin-Med. 1979 Apr. 93(4). P 674-8. MJ ACETYLCYSTEINE: ae. CILIA: de. SULFHYDRYL-COMPOUNDS: pd. MN CYSTIC-FIBROSIS: th. DEPRESSION-CHEMICAL. SUPPORT-U-S-GOVT-P-H-S. AB Although N-acetyl-L-cysteine (Mucomyst) is an effective mycolytic, numerous investigations have failed to demonstrate a consistent improvement in pulmonary mechanics following its use. In order to determine whether its beneficial mucolytic activity might be counterbalanced by a deleterious direct effect on cilia, we studied its effect on the activity of ciliated epithelium in the ferret tracheal organ culture system. N-acetyl-L-cysteine consistently caused progressive time-dependent ciliostasis at concentrations clinically employed, with complete ciliary paralysis within 8 hr. The effect was only partially reversed by the removal of the drug. Control preparations retained full ciliary activity for 3 to 12 weeks. In order to determine the active site on the N-acetyl-L- cysteine molecule, we investigated the ciliostatic effects of five of its chemical analogs. Isomolar N-acetyl-L-alanine was not ciliostatic, indicating the necessity of a sulfhydryl group for activity. Ciliostasis was independent of isomeric structure, acetylation, and chain length, as evidenced by the similar ciliostatic effects of L-cysteine, D-cysteine, 2-mercaptoethylamine, and N-acetyl-L-homocysteine. We conclude that N-acetyl-L-cysteine induces partially reversible ciliostasis of tracheal epithelium via its sulfhydryl group; prolonged use of this drug may impair mucociliary clearance. RF 001 HURST GA AM REV RESPIR DIS 96 962 967 002 HIRSCH SR J LAB CLIN MED 74 346 969 003 SHEFFNER AL TUBERCULOLOGY 23 31 966 004 CARSON S AM REV RESPIR DIS SUPPL 93 86 966 005 STAMM SJ DIS CHEST 47 414 965 006 MOSER KM DIS CHEST 49 370 966 007 PULLE DF CURR THER RES 12 485 970 008 HIRSCH SJ J ALLERGY 39 265 967 009 WALTEMATH CL AM REV RESPIR DIS 108 520 973 010 TECKLIN JS PHYS THER 56 999 976 011 SILVERS GW AM REV RESPIR DIS 110 170 974 012 MOSTOW SR ARCH GESAMTE VIRUSFORSCH 43 385 973 013 WILCOXON F SOME RAPID APPROXIMATE STATIS 964 014 WANNER A AM REV RESPIR DIS 116 73 977 015 SHEFFNER AL ANN NY ACAD SCI 106 298 963 CT 1 YANAURA S JAP J PHARMACOL 31 957 981 2 ROOMANS GM EUR J RESPIR DIS 64 416 983 3 NIEDERMAN MS AM REV RESPIR DIS 127 85 983 4 VENTURELLI J CRIT CARE MED 12 926 984 5 FREDERICK KA LAB ANIM SCI 35 298 985 6 LOW PMP ANN ALLERGY 54 273 985 7 STAFANGER G EUR J RESPIR DIS 70 157 987 PN 79113 RN 01093 AN 80050997 AU Bria-W-F. Kenny-W-R. TI Adult cystic fibrosis: a new therapeutic challenge for the primary care physician. SO J-Med-Assoc-Ga. 1979 Oct. 68(10). P 912-4. MJ CYSTIC-FIBROSIS: th. MN ANTIBIOTICS. CYSTIC-FIBROSIS: fg, pp. DIET-THERAPY. HUMAN. LUNG: pp. POSTURE. EX In this article, we will examine the reasons for the prolonged survival of individuals with cystic fibrosis as well as discuss the pathogenesis, diagnosis and treatment of this multisystemic disease. The reasons for the improved prognosis in CF are multiple: improved antibiotic therapy, early diagnosis, specialized care centers, and aggressive treatment regimens. This presents the primary care physician with a new and challenging patient population, requiring the coordinated effort of the health care team, including specialists, nurses, therapists, etc., to achieve effective therapy for this truly multisystemic disease. RF 001 ROSENLUND ML ANN INTERN MED 78 959 973 002 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 003 STERN RC ANN INTERN MED 87 188 977 004 DI SANTAGNESE PA AM FAM PHYSICIAN 7 102 973 005 STERN RC J PEDIATR 89 406 976 006 DOERSHUK CF J PEDIATR 65 677 964 007 HANDWERGER S N ENGL J MED 281 451 969 008 STERN RC GASTROENTEROLOGY 70 645 976 009 LOBER CW CHEST 66 332 974 010 KULCZYCKI LL JAMA 240 30 978 011 EWING C CHEST 73 750 978 012 HODSON ME BR MED J 2 790 976 PN 79114 RN 01094 AN 79218787 AU Nevin-G-B. Nevin-N-C. Redmond-A-O. TI Cystic fibrosis in Northern Ireland. SO J-Med-Genet. 1979 Apr. 16(2). P 122-4. MJ CYSTIC-FIBROSIS: oc. MN HUMAN. INFANT-NEWBORN. NORTHERN-IRELAND. RETROSPECTIVE-STUDIES. AB In a retrospective study of cystic fibrosis in Northern Ireland for 1961 to 1971, the incidence was 1 in 1857 livebirths, which is comparable to figures from Great Britain and the Republic of Ireland. RF 001 CARTER CO J MED GENET 14 316 977 002 DANKS DM ANN HUM GENET 28 323 965 003 GOODMAN HO AM J HUM GENET 4 59 952 004 HALL BD J MED GENET 5 262 968 005 HONEYMAN MS AM J HUM GENET 17 461 965 006 KRAMM ER AM J PUBLIC HEALTH 52 2041 962 007 NEVIN NC INT J EPIDEMIOL 7 319 978 008 OREILLY D INT J EPIDEMIOL 3 247 974 009 PUGH RJ ARCH DIS CHILD 42 544 967 010 SELANDER P ACTA PAEDIATR SCAND 51 65 962 011 STEVENSON AC RADIAT RES SUPPL 1 306 959 012 TEN KATE LP INT J EPIDEMIOL 6 23 977 CT 1 NEVIN GB HUM GENET 56 387 981 2 GRATAROLI R PEDIATR RES 18 130 984 3 STURGESS JM AM J MED GENET 22 383 985 4 JAMIESON A HUM GENET 70 168 985 PN 79115 RN 01095 AN 79196306 AU Soter-N-A. Mihm-M-C-Jr. Colten-H-R. TI Cutaneous necrotizing venulitis in patients with cystic fibrosis. SO J-Pediatr. 1979 Aug. 95(2). P 197-201. MJ CYSTIC-FIBROSIS: co. PHLEBITIS: pa. PURPURA: pa. SKIN: bs. MN ADOLESCENCE. ADULT. BIOPSY. CASE-REPORT. HUMAN. MALE. PHLEBITIS: et. PURPURA: et. SUPPORT-U-S-GOVT-P-H-S. VENULES: pa. AB Palpable purpura was noted to occur late in the course of some patients with cystic fibrosis. Skin biopsy specimens showed necrotizing venulitis characterized by a perivenular infiltrate composed of neutrophilic leukocytes, fibrin, hypogranulated mast cells, and endothelial cell necrosis. Circulating immune complexes were detected. Recurrent pulmonary infections and the chronic administration of therapeutic agents provide sources of potential antigens. RF 001 SOTER NA IN: SAMTER M 993 978 002 PARISH WE IN: BEUTNER EH 153 974 003 MOSCHELLA SL ARCH DERMATOL 95 565 967 004 QUISMORIO FP JR CLIN IMMUNOL IMMUNOPATHOL 9 184 978 005 LEVO Y TRANS ASSOC AM PHYSICIANS 90 167 977 006 DIENSTAG JL ANN INTERN MED 89 34 978 007 SOTER NA J INVEST DERMATOL 63 219 974 008 SAMS WM JR BR J DERMATOL 80 555 968 009 THEOFILOPOULOS AN J CLIN INVEST 57 169 976 010 KAMMER GM CLIN RES 25 361A 977 011 REYNOLDS HY ANN INTERN MED 82 819 975 012 WOOD RE AM REV RESPIR DIS 113 833 976 013 DVORAK HF J EXP MED 135 235 972 014 MIHM MC JR J INVEST DERMATOL 67 305 976 015 NYDEGGER UE J CLIN INVEST 54 297 974 016 SOTER NA J ALLERGY CLIN IMMUNOL 59 294 977 017 BEAVEN MA CLIN CHIM ACTA 37 91 972 018 NIELSEN HE ACTA PAEDIATR SCAND 67 443 978 019 SCHROETER AL ARCH DERMATOL 104 254 971 020 BRAVERMAN IM J INVEST DERMATOL 64 105 975 021 FEIG PU JAMA 236 2065 976 022 KAMMER GM CLIN IMMUNOL IMMUNOPATHOL 10 202 978 023 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 024 MCFARLANE H BR MED J 1 423 975 CT 1 JOHN EG J PEDIATR 97 505 980 2 MATTHEWS WJ N ENGL J MED 302 245 980 3 SORENSEN RU PEDIATR RES 15 14 981 4 MOSS RB J PEDIATR 99 215 981 5 SAVEL P ANN DERMATOL VENEREOL 109 503 982 6 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 7 GIGLI I J AM ACAD DERMATOL 11 948 984 8 SCHIDLOW DV ARCH DIS CHILD 59 377 984 9 AUERBACH HS LANCET 2 686 985 10 RESNICK AH INT J DERMATOL 24 139 985 11 SANCHEZ NP ARCH DERMATOL 121 220 985 12 WISNIESKI JJ AM REV RESPIR DIS 132 770 985 13 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 14 SUMMERS GD BR J RHEUMATOL 25 393 986 15 HOIBY N ANNU REV MICROBIOL 40 29 986 16 PHILLIPS BM J ROY SOC MED 79 44 986 17 MOSS RB AM REV RESPIR DIS 133 648 986 PN 79116 RN 01096 AN 79153525 AU Herrod-H-G. Amos-D-B. Spock-A. TI HLA determinants in families with more than one member with cystic fibrosis [letter]. SO J-Pediatr. 1979 Apr. 94(4). P 676. MJ ANTIGENIC-DETERMINANTS. CYSTIC-FIBROSIS: fg. HLA-ANTIGENS. MN CYSTIC-FIBROSIS: im. HUMAN. EX Attempts to identify the carrier state for cystic fibrosis (CF) or to make a prenatal diagnosis of CF have been unsuccessful. One approach to this problem is to look for associations between known genetic markers and the locus for CF. In general these studies have looked at the frequency of HLA types in patients with CF and have failed to demonstrate a strong association. We attempted to look for linkage between HLA haplotypes and CF in families with more than one affected member. The segregation of HLA in these affected sibpairs vary little from the expected. Although the sample size is small, it is unlikely that a close linkage exists between the HLA loci and the CF locus. RF 001 LAMM LU ANN HUM GENET 38 383 975 002 GOODCHILD MC J MED GENET 13 417 976 003 AMOS DB IN: ROSE NR 797 976 004 HERROD HG J PEDIATR 91 276 977 CT 1 ANON LANCET 2 249 985 2 MOSS RB MONOGR ALLERGY 19 202 986 PN 79117 RN 01097 AN 79153494 AU Newman-A-J. Ansell-B-M. TI Episodic arthritis in children with cystic fibrosis. SO J-Pediatr. 1979 Apr. 94(4). P 594-6. MJ ARTHRITIS: co. CYSTIC-FIBROSIS: co. MN CHILD. CHILD-PRESCHOOL. FEMALE. HUMAN. MALE. EX Five children 2 to 10 years of age with cystic fibrosis developed transient episodic arthritis. Although polyarticular in distribution, only one joint might be involved during each attack. No laboratory or radiologic evidence of juvenile chronic arthritis was present and no permanent joint limitation was noted. RF 001 LINDSLEY CB J PEDIATR 84 16 974 002 PETTY RE ARTHRITIS RHEUM 19 902 976 003 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 004 GIBSON TJ J RHEUMATOL 2 7 975 005 MULLIN GT ANN INTERN MED 68 75 968 006 LUCAS PF GUT 3 146 962 007 TANNENBAUM H J RHEUMATOL 2 14 975 008 SMITH BS BR MED J 1 1403 966 009 HASLOCK I RHEUMATOL REHAB 13 51 974 010 STAPLETON FB N ENGL J MED 295 246 976 CT 1 VAZE D J PEDIATR 96 346 980 2 SAGRANSKY DM AM J DIS CHILD 134 319 980 3 BYWATERS EGL N ENGL J MED 302 1467 980 4 MOSS RB J PEDIATR 99 215 981 5 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 63 982 6 DELUMLEY L ARCH FR PEDIATR 40 723 983 7 SCHIDLOW DV ARCH DIS CHILD 59 377 984 8 BRAUDE S BR MED J 288 822 984 9 CRAWFORD AM RHEUMATOL INT 5 283 985 10 LESTER LA SEM RESPIR MED 6 285 985 11 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 12 SUMMERS GD BR J RHEUMATOL 25 393 986 13 CASSIDY JT PEDIATR CLIN NORTH AM 33 1033 986 14 HOIBY N ANNU REV MICROBIOL 40 29 986 15 PHILLIPS BM J ROY SOC MED 79 44 986 16 COHEN AM AM J DIS CHILD 140 74 986 PN 79118 RN 01098 AN 79196242 AU Worley-L. Poole-C. Valdes-Depena-M. TI Failure to thrive, anemia, and anasarca. SO J-Pediatr. 1979 Jun. 94(6). P 1005-9. MJ CYSTIC-FIBROSIS: co. FOOD-HYPERSENSITIVITY: co. MILK: ae. PROTEIN-LOSING-ENTEROPATHIES: et. MN ANEMIA: et. CASE-REPORT. FEMALE. HUMAN. INFANT. EX A 5-month-old white girl was admitted to an Infant Intensive Care Unit of Jackson Memorial Hospital. According to her mother, the baby had been in "her usual state of health" until one week before admission, when she began to "spit up" after feedings and her lower extremities became puffy. Vomiting increased in frequency until it occurred after every meal; eventually the baby refused all feedings; there was no diarrhea. Ultimately, the family's physician, when consulted, recommended that she be admitted to the hospital. On the fourth day she developed renal failure with anuria and rising creatinine concentration despite adequate fluid intake; peritoneal dialysis was begun. However, she soon became hypothermic and developed pulmonary edema, and she died a few hours later. A discussion of the case is presented. RF 001 MACLEAN WC JR J PEDIATR 83 86 973 002 LEE PA JAMA 228 585 974 003 COLON AR SOUTH MED J 66 641 973 004 SWISCHUK LE J PEDIATR 88 452 976 005 GRISCOM NT RADIOLOGY 117 385 975 006 MELHEM RE PEDIATR RADIOL 4 153 976 PN 79119 RN 01099 AN 79196248 AU Mihalas-L-S. Mitchell-I. TI Bronchial hyperreactivity in cystic fibrosis [letter]. SO J-Pediatr. 1979 Jun. 94(6). P 1013-4. MJ BRONCHIAL-SPASM: co. CYSTIC-FIBROSIS: co. MN HUMAN. EX Mitchell and co-workers conclude that bronchial hyperreactivity is common in cystic fibrosis (CF) but is different from that in asthma. Their data were interesting, but I do not agree with their conclusion. One can interpret these data as suggesting a common reversible component in both patient groups, and perhaps a common denominator in mechanism. Because of certain problems with baseline measurements and withholding of medication, it seems that the conclusion of Mitchell et al may not be well supported. - We felt justified in studying those patients with a low baseline FEV (forced expiratory volume in one second), since a subject's bronchial response to methacholine remains stable even if these is moderate fluctuation both in initial specific airway conductance and in the severity of the disease. We feel that withholding the drug for a longer period would not have affected the comparisons. Although there might be a common underlying mechanism in bronchial hyperreactivity in asthma and cystic fibrosis, our data showed a number of differences between the two groups of patients. Although these differences would not exclude a common mechanism in bronchial hyperreactivity in the two diseases, the existence of the differences should lead to caution in concluding that there is a common underlying mechanism in the bronchial hyperreactivity in the two diseases. RF 001 MITCHELL I J PEDIATR 93 744 978 002 SPECTOR SL MED CLIN NORTH AM 58 71 974 003 CHAI H J ALLERGY CLIN IMMUNOL 56 323 975 004 RUBINFELD AR AM REV RESPIR DIS 115 381 977 PN 79120 RN 01100 AN 79196281 AU Malis-F. Fric-P. Kasafirek-E. Jodl-J. Vavrova-V. Slaby-J. TI A peroral test of pancreatic insufficiency with 4-(N-acetyl-L-tyrosyl)aminobenzoic acid in children with cystic fibrosis. SO J-Pediatr. 1979 Jun. 94(6). P 942-4. MJ P-AMINOBENZOIC-ACID: du. AMINOBENZOIC-ACIDS: du. CYSTIC-FIBROSIS: di. MN P-AMINOBENZOIC-ACID: aa. ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. MALE. PANCREAS: pp. TYROSINE: aa, du. EX We report on the examination of stimulated pancreatic secretion using 4-(N-acetyl-L-tyrosyl)aminobenzoic acid in children with cystic fibrosis. In controls the urinary excretion of 4-aminobenzoic acid amounted to 54.8 +/- 11.7% with 19.6% as the lower limit of normal during six hours, and 74.7 +/- 10.8% and 40.5% during eight hours. In children with cystic fibrosis the mean values were 5.6% in the six-hour test and 13.7% in the eight-hour test. Both values are significantly different in comparison with controls. It may be assumed that both 4-(N-acetyl-L-tyrosyl)aminobenzoic acid and 4-(N-benzoyl-L-tyrosyl)aminobenzoic acid are suitable for indirect determination of exocrine pancreatic secretion in children. RF 001 IMONDI AR GUT 13 726 972 002 GYR K GUT 17 27 976 003 ARVANITAKIS C LANCET 1 663 976 004 BORNSCHEIN W CLIN CHIM ACTA 67 21 976 005 NOUSIA-ARVANITAKIS S J PEDIATR 92 734 978 006 MALIS F J CLIN CHEM CLIN BIOCHEM 14 499 976 007 MALIS F ACTA HEPATOGASTROENTEROL 25 233 978 008 SMITH HW J CLIN INVEST 24 388 945 009 ZASSENHAUS PH ANAL BIOCHEM 76 321 976 010 WOLGEMUTH RL AM J DIG DIS 21 821 976 CT 1 FRIC P HEPATOGASTROENTEROLOGY 27 220 980 2 NIESSEN KH MONATSSCHR KINDERHEILKD 128 746 980 3 SCHONBERGER W MONATSSCHR KINDERHEILKD 128 195 980 4 LANKISCH PG HEPATOGASTROENTEROLOGY 28 333 981 5 PARK RW GASTROENTEROLOGY 81 1143 981 6 NEIS P MONATSSCHR KINDERHEILKD 129 347 981 7 LANKISCH PG GUT 23 777 982 8 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 9 LANKISCH PG CLIN GASTROENTEROL 13 717 984 10 SCOTTA MS CLIN BIOCHEM 18 233 985 11 RIEU D ANN PEDIATR (PARIS) 32 205 985 12 CUMMING JGR ARCH DIS CHILD 61 573 986 PN 79121 RN 01101 AN 79131916 AU Cox-K-L. Isenberg-J-N. Osher-A-B. Dooley-R-R. TI The effect of cimetidine on maldigestion in cystic fibrosis. SO J-Pediatr. 1979 Mar. 94(3). P 488-92. MJ CIMETIDINE: tu. CYSTIC-FIBROSIS: co. GUANIDINES: tu. MALABSORPTION-SYNDROMES: dt. MN ADOLESCENCE. ADULT. CHILD. CIMETIDINE: bl. CYSTIC-FIBROSIS: bl. DRUG-THERAPY-COMBINATION. FEMALE. HUMAN. MALABSORPTION-SYNDROMES: bl, et. MALE. PANCREATIC-EXTRACTS: tu. PANCREATIN: tu. CELIAC-DISEASE: dt, et. SUPPORT-U-S-GOVT-P-H-S. AB Ten patients (6 to 27 years of age) who had severe pancreatic exocrine insufficiency due to cystic fibrosis were studied to determine whether cimetidine would improve dietary fat and nitrogen absorption. When a constant diet was consumed and oral pancreatic enzymes were administered, the addition of cimetidine (150 or 200 mg taken orally one-half hour before meals) signficantly reduced fecal fat excretion from 25.3 +/- 2.9 to 17.3 +/- 2.1 gm/24 hours and fecal nitrogen excretion from 4.5 +/- 0.6 to 3.4 +/- 0.5 gm/24 hours (P less than 0.05). Lower doses of cimetidine resulted in less significant reductions of steatorrhea and azotorrhea. Cimetidine may be a useful adjunct to oral pancreatic enzyme therapy in patients with cystic fibrosis who continue to have steatorrhea and azotorrhea with enzyme therapy alone. RF 001 HEIZER WD BULL JOHNS HOPKINS HOSP 116 261 965 002 GRAHAM DY N ENGL J MED 296 1314 977 003 DIMAGNO EP N ENGL J MED 296 1318 977 004 ROY CC PEDIATR CLIN GASTROENTEROL 975 005 HARO EN CLIN RES 12 207 964 006 VEEGER W N ENGL J MED 267 1341 962 007 REGAN PT N ENGL J MED 297 854 977 008 RICHARDSON CT GASTROENTEROLOGY 74 366 978 009 SAUNDERS JHB BR MED J 1 418 977 010 WIGGINS HS GUT 8 415 967 011 TIETZ NW CLIN CHIM ACTA 13 352 966 012 WORMSLEY KG GASTROENTEROLOGY 54 197 968 013 VAN DE KAMER JH J BIOL CHEM 177 347 949 014 HILLER A J BIOL CHEM 177 1401 948 015 FREYE HB J PEDIATR 64 575 964 016 WALKENSTEIN SS GASTROENTEROLOGY 74 360 978 017 POUNDER RE GUT 17 161 976 018 ANTONOWICZ I J PEDIATR 92 214 978 019 MORIN CL J PEDIATR 88 213 976 020 REEMTSMA K PEDIATRICS 22 525 958 021 WATKINS JB GASTROENTEROLOGY 73 1023 977 022 ROY CC N ENGL J MED 297 1301 977 023 UFBERG MH GASTROENTEROLOGY 73 635 977 024 MCGREGOR CGA LANCET 1 122 977 025 AVELLA J LANCET 1 624 978 026 BRIMBLECOMBE RW IN: BURLAND WL 977 CT 1 MARTIN DW WEST J MED 131 417 979 2 GILLARD BK PEDIATR RES 14 1168 980 3 DURIE PR GUT 21 778 980 4 HUBBARD VS AM J CLIN NUTR 33 2281 980 5 LAVARENNE J THERAPIE 35 83 980 6 BOYLE BJ GASTROENTEROLOGY 78 950 980 7 BLAKER F MONATSSCHR KINDERHEILKD 128 626 980 8 GOW R LANCET 2 1071 981 9 BRADBEAR RA BR J CLIN PHARMACOL 12 248 981 10 MITCHELL EA AUST PAEDIATR J 17 89 981 11 MITCHELL EA AUST PAEDIATR J 17 207 981 12 SCHONI M HELV PAEDIATR ACTA 36 359 981 13 DEBIEVILLE F ACTA PAEDIATR SCAND 70 33 981 14 PARK RW GASTROENTEROLOGY 81 1143 981 15 HALL KW CAN MED ASSOC J 124 1579 981 16 STAUB JL N ENGL J MED 304 1364 981 17 CHASE HP J PEDIATR GASTROENTEROL NUTR 1 49 982 18 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 19 SALOM IL SCAND J GASTROENTEROL 17 129 982 20 MITCHELL EA AUST PAEDIATR J 18 114 982 21 DIMAGNO EP DIG DIS SCI 27 481 982 22 GRAHAM DY DIG DIS SCI 27 485 982 23 HUBBARD VS J AM DIET ASSOC 80 127 982 24 BRADBEAR RA J PEDIATR 100 325 982 25 ISENBERG JN J PEDIATR GASTROENTEROL NUTR 2 447 983 26 SOMOGYI A CLIN PHARMACOKINET 8 463 983 27 GRENDELL JH CLIN GASTROENTEROL 12 551 983 28 SORKIN EM DRUG INTEL CLIN PHARM 17 110 983 29 LANKISCH PG CLIN GASTROENTEROL 13 985 984 30 SCHONI M PEDIATR RES 18 66 984 31 ZIEMNIAK JA EUR J CLIN PHARMACOL 26 183 984 32 ABRAMS CK J CLIN INVEST 73 374 984 33 HODGES P J AM DIET ASSOC 84 664 984 34 GILLARD BK AM J DIS CHILD 138 577 984 35 HYMAN PE J PEDIATR GASTROENTEROL NUTR 4 316 985 36 PERRY RS CLIN PHARMACY 4 161 985 37 ZENTLERMUNRO PL GUT 26 892 985 38 SCHNEIDER MU HEPATOGASTROENTEROLOGY 32 97 985 39 CHALMERS DM ACTA PAEDIATR SCAND 74 114 985 40 SOMOGYI A EUR J PEDIATR 144 72 985 41 SINAASAPPEL M ACTA PAEDIATR SCAND SUPPL 317 1985 22 985 42 RUBINSTEIN S PEDIATRICS 78 473 986 43 LAMERS CBHW BR MED J 293 994 986 44 BRAGGION C ARCH DIS CHILD 62 349 987 PN 79122 RN 01102 AN 79196324 AU Church-J-A. Keens-T-G. Wang-C-I. ONeal-M. Richards-W. TI Normal neutrophil and monocyte chemotaxis in patients with cystic fibrosis. SO J-Pediatr. 1979 Aug. 95(2). P 2724. MJ CHEMOTAXIS-LEUKOCYTE. CYSTIC-FIBROSIS: bl. MN ADULT. HUMAN. MONOCYTES: im. NEUTROPHILS: im. AB The prominent role of infection in the clinical course of cystic fibrosis has led to considerable investigation of possible immune deficiency in these patients. Leukotaxis of circulating phagocytic cells and the ability to generate chemotactic factors are important in host defenses against bacterial invasion. These properties were investigated in patients with clinically stable cystic fibrosis to determine if they possess dysfunctional inflammatory responses which would predispose them to severe infection. RF 001 TALAMO RC IN: MANGOS JA 195 976 002 GALLIN JI IN: BELLANTI JA 227 975 003 LETT-BROWN MA J IMMUNOL 117 246 976 004 KLEIN RB PEDIATRICS 60 467 977 005$ ORR W J IMMUNOL METH 20 95 978 006 SORENSEN RU J PEDIATR 93 201 978 007 CONOVER JH LANCET 2 1501 973 008 HILL HR J PEDIATR 84 55 974 PN 79123 RN 01103 AN 79196343 TI Hyperuricemia in cystic fibrosis [letter]. SO J-Pediatr. 1979 Aug. 95(2). P 330-1. MJ CYSTIC-FIBROSIS: bl. URIC-ACID: bl. MN CYSTIC-FIBROSIS: dt. HUMAN. PANCREATIC-EXTRACTS: ae, tu. EX Davidson et al report hyperuricemia in cystic fibrosis patients ingesting large quantities of pancreatic extracts. This article and others emphasize the considerable purine content of these preparations as causal to the increased serum and urine uric acid levels in patients who consume them. This may find clinical application, since uric acid production from RNA might be decreased by an inhibitor of ribonuclease. While itself a purine chain, polyadenylic acid added to pancreatic extracts may prevent the hydrolysis of mixed-base ribonucleic acid to uric acid precursors, while allowing the beneficial effects of the other pancreatic enzymes to prevail. - A number of weaknesses to the proposal bears stressing before we begin giving polyadenylic acid to our patients. No one knows whether nucleic acids are malabsorbed in cystic fibrosis. If polyadenylic acid produces only 70% inhibition of pancreatic ribonucleases at a one-to-one ratio of substrate and inhibitor, a relatively enormous amount of polyadenylic acid would have to be fed with the pancreatic extracts. If there were an intestinal enzyme capable of cleaving polyadenylic acid, the treatment would add to, not subtract from, the purine load. Finally, Stapleton et al's results suggest that more than 75% of the purine load in the pancreatic extracts would probably be absorbed anyway. RF 001 DAVIDSON GP J PEDIATR 93 976 978 002 STAPLETON FB N ENGL J MED 295 246 976 003 NOUSIA-ARVANITAKIS S J PEDIATR 90 302 977 004 REDDI KK BIOCHEM BIOPHYS RES COMMUN 67 110 975 005 SCHMUKLER M J BIOL CHEM 250 2206 975 006 GAHL WA N ENGL J MED 297 1349 977 007 ZIMMERMAN SB ANAL BIOCHEM 10 444 965 008 ZAN-KOWALCZEWSKA M BIOCHIM BIOPHYS ACTA 341 138 974 PN 79124 RN 01104 AN 80051503 AU Fellows-K-E. Khaw-K-T. Schuster-S. Shwachman-H. TI Bronchial artery embolization in cystic fibrosis; technique and long-term results. SO J-Pediatr. 1979 Dec. 95(6). P 959-63. MJ BRONCHIAL-ARTERIES. CYSTIC-FIBROSIS: co. EMBOLIZATION-THERAPEUTIC. HEMOPTYSIS: th. MN ADOLESCENCE. ADULT. ANGIOGRAPHY. BRONCHIAL-DISEASES: ra, th. BRONCHOSCOPY. CHILD. FEMALE. FOLLOW-UP-STUDIES. HEMOPTYSIS: et. HUMAN. MALE. RECURRENCE. AB Severe bronchial hemorrhage in 13 patients with cystic fibrosis was treated by catheter embolization of bronchial arteries. Indications were either excessive bleeding persisting for several days, or bleeding serious enough to interfere with pulmonary drainage and recurring over weeks or months. In follow-up ranging from one to 30 months, cessation of major bleeding was achieved in 12 of 13 patients (93%), although 5 of 13 patients (40%) did have recurrence of minor hemoptysis. No neurologic or other major complications were encountered. However, there are potential risks and this approach at present should be limited to patients with life-threatening bleeding and carried out only by experienced angiographers. RF 001 HOLSCLAW DS J PEDIATR 76 829 970 002$ STERN RC AM REV RESPIR DIS 117 825 978 003 SCHUSTER SR J PEDIATR SURG 12 889 977 004 WHOLEY MH JAMA 236 2501 976 005 HARLEY JD AM J ROENTGENOL 128 302 977 006 BOOKSTEIN JJ CHEST 72 658 977 007 REMY J RADIOLOGY 122 33 977 008 SHWACHMAN H AM J DIS CHILD 96 6 958 009 FELLOWS KE RADIOLOGY 114 551 975 010 FEIGELSON HH RADIOLOGY 85 663 965 011 KARDJIEV V RADIOLOGY 112 81 974 012 REUTER S RADIOLOGY 84 87 965 013 DICHIRO G RADIOLOGY 112 231 974 014 DICHIRO G J NEUROSURG 39 1 973 015 TVETEN L ACTA RADIOL 17 1 976 016 TVETEN L ACTA RADIOL 17 257 976 017 BOTENGA ASJ SELECTIVE BRONCHIAL AND INTER 970 018 HELENON CH NOUV PRESSE MED 6 4209 977 019 BARTH KH INVEST RADIOL 12 273 977 020 KERBER CW AM J ROENTGENOL 130 1193 978 CT 1 HOLSCLAW DS CLIN CHEST MED 1 407 980 2 ATHANASOULIS CA N ENGL J MED 302 1117 980 3 FELLOWS KE RADIOLOGY 140 249 981 4 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 5 SILVERMAN NA CHEST 82 195 982 6 HEASTON DK SEM RESPIR MED 5 58 983 7 MARMON L J PEDIATR SURG 18 811 983 8 PORTER DK J THORAC CARDIOVASC SURG 86 409 983 9 PORTER DK ARCH INTERN MED 143 287 983 10 UFLACKER R RADIOLOGY 146 627 983 11 WHITE RI ANN RADIOL (PARIS) 27 310 984 12 BARTH KH MED CLIN NORTH AM 68 1647 984 13 DAVIS PB CHEST 85 802 984 14 WHITE RI AM J ROENTGENOL 142 27 984 15 LESTER LA SEM RESPIR MED 6 285 985 16 HOFFER FA PEDIATR CLIN NORTH AM 32 1461 985 17 TRENTO A ANN THORAC SURG 39 254 985 18 STANKIEWICZ JA EAR NOSE THROAT J 64 180 985 19 NOLAN MT IR J MED SCI 154 336 985 20 LOCK JE J AM COLL CARDIOL 7 1420 986 21 KAUFMAN SL AM J CARDIOL 58 1130 986 22 MOORE LB RADIOLOGY 161 173 986 23 FERNALD GW SEM ROENTGENOL 22 87 987 24 LOCK JE CIRCULATION 75 593 987 PN 79125 RN 01105 AN 80008528 AU Schwachman-H. TI Reliability of sweat test in cystic fibrosis [letter]. SO J-Pediatr. 1979 Oct. 95(4). P 661-2. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN CHLORIDES: an. FALSE-POSITIVE-REACTIONS. HUMAN. SODIUM: an. EX The report by Huff, Huang, and Arey again calls attention to the heterogeneity of cystic fibrosis and to the fact that a properly performed sweat test in the normal or borderline range does not rule out cystic fibrosis. This may occur in approximately 1% of patients we classify as having cystic fibrosis and confirms our original impression that the sweat test has a reliability of between 98% and 99%. One must pay close attention to the clinical picture, the family history, and especially the pulmonary or gastrointestinal findings, and provide rational treatment until more acceptable evidence of the disease can be documented. In the same issue is a report on a false positive sweat test in a Canadian patient with the Mauriac syndrome. The authors list a variety of conditions that have been reported as yielding elevated sweat electrolytes. The manner of the collections can greatly influence the result. - In patients with transient abnormal sweat electrolytes, sodium values were consistently higher than chloride values. Perhaps the finding of sodium values in excess of chloride in the abnormal range suggests a false positive test. RF 001 HUFF DS J PEDIATR 94 237 979 002 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 003 BRESLOW JL SCIENCE 201 180 978 004 ROSENFELD R J PEDIATR 94 240 979 005 SHWACHMAN H CLIN CHEM 25 158 979 CT 1 RUDDY RM CLIN PEDIATR 26 83 987 PN 79126 RN 01106 AN 79069499 AU Hubbard-V-S. Farrell-P-M. di-SantAgnese-P-A. TI 25-Hydroxycholecalciferol levels in patients with cystic fibrosis. SO J-Pediatr. 1979 Jan. 94(1). P 84-6. MJ CYSTIC-FIBROSIS: bl. HYDROXYCHOLECALCIFEROLS: bl. MN HUMAN. VITAMIN-D: ad. EX Assessment of the status of circulating vitamin D in cystic fibrosis patients was investigated by determining serum levels of 25-hydroxycholecalciferol. The majority of the patients with CF and pancreatic insufficiency had levels of 25-OH-D within the normal range. Clinical symptoms or pathologic signs resulting from deficiencies of vitamins A and E in patients with CF have been repeatedly documented. In contrast, a paradoxical lack of vitamin D deficiency signs has been observed in CF patients. This is especially puzzling since rickets had been commonly associated with most other forms of steatorrhea. We can only speculate why rickets was formerly rarely reported in association with CF. Perhaps because of voracious appetites and the selective absorption of vitamin D via the lymphatics without requiring esterification, these individuals were spared clinical rickets. RF 001 DI SANTAGNESE PA AM J MED 21 406 956 002 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 003 ANDERSEN DH J PEDIATR 15 763 939 004 PARSONS LG ARCH DIS CHILD 2 198 927 005 WEBER A PEDIATRICS 50 73 972 006 TAUSSIG LM J PEDIATR 82 380 973 007 BELSEY RE J CLIN ENDOCRINOL METAB 38 1046 974 008 FARRELL PM PEDIATR RES 11 443 977 009 ARNAUD SB PEDIATRICS 57 221 976 010 HADDAD JG JR NATURE 244 515 973 011 THOMPSON GR J CLIN INVEST 45 94 966 012 BLOMSTRAND R ACTA CHEM SCAND 21 1662 967 CT 1 ANON NUTR REV 37 247 979 2 CHASE HP J PEDIATR 95 337 979 3 WEISMAN Y J PEDIATR 95 416 979 4 MEREDITH SC CLIN ENDOCRINOL METAB 9 131 980 5 SOLOMONS NW AM J CLIN NUTR 34 462 981 6 CONGDEN PJ ARCH DIS CHILD 56 708 981 7 COPPENHAVER D HUM GENET 57 399 981 8 PARK RW GASTROENTEROLOGY 81 1143 981 9 LOGVINOFF MM CLIN PEDIATR 23 389 984 10 HUBBARD VS SEM RESPIR MED 6 308 985 11 HANLY JG Q J MED 56 377 985 12 REITER EO J PEDIATR 106 21 985 13 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 14 DUHAMEL JF ARCH FR PEDIATR 43 229 986 PN 79127 RN 01107 AN 79069484 AU Hahn-T-J. Squires-A-E. Halstead-L-R. Strominger-D-B. TI Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. SO J-Pediatr. 1979 Jan. 94(1). P 38-42. MJ CALCIUM: bl. CYSTIC-FIBROSIS: bl. HYDROXYCHOLECALCIFEROLS: bl. MN ADOLESCENCE. ADULT. BONE-AND-BONES: me. CALCIUM: ur. CHILD. CYSTIC-FIBROSIS: me. FEMALE. HUMAN. KIDNEY: me. MALE. PARATHYROID-HORMONES: bl. SUPPORT-U-S-GOVT-P-H-S. AB Vitamin D and mineral metabolism were studied in 21 adolescents and young adults with cystic fibrosis and the results were compared to those in 21 matched controls. All CF patients had been maintained on standard multivitamin supplements in combination with pancreatic enzyme replacement. Despite this supplementation, relative to control subjects the CF patients had a 36% reduction in serum 25- hydroxyvitamin D concentration, a slight but significant reduction in serum calcium concentration, evidence of calcium malabsorption with secondary hyperparathyroidism, and a 14% decrease in bone mass measured by the photon absorption technique. Currently accepted modes of pancreatic enzyme replacement and vitamin D supplementation are often inadequate to maintain normal mineral homeostasis in CF patients; additional measurements may be required to reduce the risk of clinically significant osteopenia concomitant with prolonged survival in CF. RF 001 BENNETT MJ AM J CLIN NUTR 20 415 967 002 TORSTENSON OL PEDIATRICS 45 857 970 003 GUGLER EC MOD PROBL PEDIATR 10 95 967 004 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 005 DI SANTAGNESE PA N ENGL J MED 295 597 976 006 HAHN TJ N ENGL J MED 292 550 974 007 TAUSSIG LM J PEDIATR 82 380 973 008 HAHN TJ J CLIN ENDOCRINOL METAB 39 274 974 009 ANON MEDICAL LABORATORY TECHNOLOGY 169 969 010 SLATOPOLSKY E J CLIN INVEST 50 492 971 011 HADDAD JG JR J LAB CLIN MED 81 22 973 012 KERMACK KA BIOMETRIKA 37 30 950 013 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 014 LAPEY A J PEDIATR 84 328 974 015 PARSONS JA CLIN ENDOCRINOL METAB 1 33 972 016 HAHN TJ DRUGS 12 201 976 017 REEMTSMA K PEDIATRICS 22 525 958 018 ARNAUD SB PROC SOC EXP BIOL MED 149 570 975 019 THOMPSON GR J CLIN INVEST 45 94 966 020 GERTNER JM BR MED J 1 1310 977 021 DELUCA HF J LAB CLIN MED 87 7 976 022 DI SANTAGNESE PA PEDIATRICS 18 387 956 023 TYSON KRT J PEDIATR SURG 3 271 968 024 GARABEDIAN M PROC NAT ACAD SCI USA 69 1673 972 025 SCHNEIDER LE SCIENCE 196 1452 977 026 JUBIZ W J CLIN ENDOCRINOL METAB 44 617 977 027 LACHMAN E AM J ROENTG RAD THER NUCL MED 74 712 955 028 WILSON CR NORLAND CAMERON BONE MINERAL 972 029 LEVIN ME N ENGL J MED 294 241 976 CT 1 AVIOLI LV CLIN ENDOCRINOL METAB 8 547 979 2 ANON NUTR REV 37 247 979 3 CHASE HP J PEDIATR 95 337 979 4 WEISMAN Y J PEDIATR 95 416 979 5 MEREDITH SC CLIN ENDOCRINOL METAB 9 131 980 6 LOVINGER RD PEDIATRICS 66 359 980 7 MITCHELL EA J PEDIATR 97 789 980 8 PITT MJ RADIOL CLIN NORTH AM 19 581 981 9 SOLOMONS NW AM J CLIN NUTR 34 462 981 10 CONGDEN PJ ARCH DIS CHILD 56 708 981 11 COPPENHAVER D HUM GENET 57 399 981 12 PARK RW GASTROENTEROLOGY 81 1143 981 13 LLOYDSTILL JD J PEDIATR 99 580 981 14 LEVI N RIV ITAL PEDIATR 8 695 982 15 AGUS ZS KIDNEY INT 24 113 983 16 KATZ S CELL CALC 5 421 984 17 GOTZ M ATEMWEGS LUNGENKRANKH 10 594 984 18 LOGVINOFF MM CLIN PEDIATR 23 389 984 19 HODGES P J AM DIET ASSOC 84 664 984 20 HUBBARD VS SEM RESPIR MED 6 308 985 21 LO CW AM J CLIN NUTR 42 644 985 22 HANLY JG Q J MED 56 377 985 23 FRIEDMAN HZ GASTROENTEROLOGY 88 808 985 24 REITER EO J PEDIATR 106 21 985 25 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 26 DUHAMEL JF ARCH FR PEDIATR 43 229 986 PN 79128 RN 01108 AN 79241418 AU Weisman-Y. Reiter-E. Stern-R-C. Root-A. TI Serum concentrations of 25-hydroxyvitamin D and 24,25-dihydroxyvitamin D in patients with cystic fibrosis. SO J-Pediatr. 1979 Sep. 95(3). P 416-8. MJ CYSTIC-FIBROSIS: bl. DIHYDROXYCHOLECALCIFEROLS: bl. HYDROXYCHOLECALCIFEROLS: bl. MN HUMAN. SUPPORT-U-S-GOVT-P-H-S. EX Steatorrhea is a prevalent gastrointestinal manifestation of cystic fibrosis. Although deficiencies of vitamins A, E, and K have been described in patients with this disease, vitamin D deficiency and rickets have rarely been reported. The major form of vitamin D, cholecalciferol (vitamin D3), is produced by ultraviolet irradiation of 7-dehydrocholesterol in the skin. Ergocalciferol (vitamin D2) is a product of plant ergosterol. We studied the vitamin D status of patients with cystic fibrosis by measurement of the serum concentrations of the major circulating metabolites of vitamin D: 25-hydroxyvitamin D and 24,25-dihydroxyvitamin D. The mean serum concentrations of total 25OHD, 25OHD3, and 24,25(OH)2D and 25OHD3/25OHD ratios in patients with cystic fibrosis were similar to values recorded in control subjects. The present study demonstrates that the serum concentrations of the major metabolites of vitamin D in patients with cystic fibrosis are similar to those in healthy subjects. RF 001 DI SANTAGNESE PA N ENGL J MED 295 597 976 002 KOPEL FB GASTROENTEROLOGY 62 483 972 003 FARRELL PM J CLIN INVEST 60 233 977 004 TORSTENSON OL PEDIATRICS 45 857 970 005 KEATING JP PEDIATRICS 46 41 970 006 WEISMAN Y J PEDIATR 91 904 977 007 STERN RC J PEDIATR 89 406 976 008 PREECE MA Q J MED 44 575 975 009 HADDAD JG JR NATURE 244 515 973 010 HAHN TJ J PEDIATR 94 38 979 011 HUBBARD VS J PEDIATR 94 84 979 CT 1 PITT MJ RADIOL CLIN NORTH AM 19 581 981 2 PARK RW GASTROENTEROLOGY 81 1143 981 3 SHANY S AM J CLIN NUTR 40 1290 984 4 HANLY JG Q J MED 56 377 985 5 REITER EO J PEDIATR 106 21 985 6 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 7 DUHAMEL JF ARCH FR PEDIATR 43 229 986 PN 79129 RN 01109 AN 79111345 AU Huff-D-S. Huang-N-N. Arey-J-B. TI Atypical cystic fibrosis of the pancreas with normal levels of sweat chloride and minimal pancreatic lesions. SO J-Pediatr. 1979 Feb. 94(2). P 237-9. MJ CHLORIDES: an. CYSTIC-FIBROSIS: di. SWEAT: an. MN CASE-REPORT. CHILD. CYSTIC-FIBROSIS: fg, pa. HUMAN. INFANT. MALE. PANCREAS: pa. EX This paper presents additional evidence of the variability and independence of the involvement of the sweat glands in cystic fibrosis. A case report of an infant girl, who died at age 9 years of progressive pulmonary disease, is presented. The case indicates that fatal cystic fibrosis, or a variant thereof, can exist with normal levels of sweat chloride and that the pulmonary disease develops independently of the functional abnormality of the sweat glands. RF 001 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 002 THOMAIDIS TS J PEDIATR 63 444 963 003 LANDING BH ANN NY ACAD SCI 93 518 962 004 DI SANTAGNESE PA PEDIATRICS 12 549 953 005 DI SANTAGNESE PA JAMA 172 2065 960 006 COGSWELL JJ ARCH DIS CHILD 49 520 974 007 SARSFIELD JK ARCH DIS CHILD 50 463 975 CT 1 SHWACHMAN H J PEDIATR 95 661 979 2 DAVIS PB AM J MED 69 643 980 3 STERN RC LANCET 1 1401 982 4 WONG LTK GUT 23 744 982 5 BARBERO GJ J PEDIATR 100 914 982 6 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 7 YEUNG WH CLIN PEDIATR 23 603 984 8 MCCARTHY VP PEDIATR INFECT DIS 5 256 986 9 ROSENSTEIN BJ CLIN PEDIATR 26 78 987 PN 79130 RN 01110 AN 79241407 AU Chipps-B-E. Alderson-P-O. Roland-J-M. Yang-S. van-Aswegen-A. Martinez-C-R. Rosenstein-B-J. TI Noninvasive evaluation of ventricular function in cystic fibrosis. SO J-Pediatr. 1979 Sep. 95(3). P 379-84. MJ CYSTIC-FIBROSIS: pp. HEART-VENTRICLE: pp. MN ADOLESCENCE. ADULT. ANGIOGRAPHY. CARBON-DIOXIDE: bl. CHILD. CHILD-PRESCHOOL. ECHOCARDIOGRAPHY. ELECTROCARDIOGRAPHY. FEMALE. HEART-VENTRICLE: ra, ri. HUMAN. MALE. OXYGEN: bl. RESPIRATORY-FUNCTION-TESTS. SUPPORT-U-S-GOVT-P-H-S. AB The cardiac function of 21 patients with cystic fibrosis was studied using radionuclides and M-mode echocardiography. The patients (mean age 13.2 years, range 4 to 27) had a wide range of clinical and pulmonary function abnormalities (mean Shwachman-Kulczycki score 62.1). Decreased right ventricular ejection fraction was found in 13 of 18 patients (72%); an additional four patients had abnormal septal motion on ECHO. Left ventricular ejection fraction was abnormal at rest in four patients (19%); an additional four patients had borderline low LVEF. The LV pre-ejection period to ejection time ratio increased significantly (i.e., worsening LV performance) with deterioration of S-K score, chest radiograph score, and forced expiratory volume in 1 second. Three of eight patients with normal LVEF at rest had an abnormal response to supine bicycle exercise: LVEF fell in two patients and was unchanged in one. Thus evidence of LV dysfunction was observed in seven of 21 (33%) of the patients; four at rest and in three only during exercise. RF 001 LIEBMAN J IN: MANGOS JA 41 976 002 SIASSI B J PEDIATR 78 794 971 003 ROSENTHAL A PEDIATR CLIN NORTH AM 23 327 976 004 GEWITZ M AM J DIS CHILD 131 275 977 005 FLEMING DG PEDIATR RES 12 560 978 006 MARSHALL RC CIRCULATION 56 820 977 007 KACHEL RG CHEST 74 286 978 008 WILLIAMS JF JR J CLIN INVEST 47 1143 968 009 FRANK MJ CIRCULATION 47 798 973 010 STEELE P AM J MED 59 21 975 011 BAUM GL N ENGL J MED 285 361 971 012 MICHELSON N DIS CHEST 38 435 960 013 BARNES GL AUST PAEDIATR J 6 81 970 014 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 015 STRAUSS HW AM J CARDIOL 28 575 971 016 BUROW RD CIRCULATION 56 1024 977 017 QURESHI S J NUCL MED 19 135 978 018 PITT B N ENGL J MED 296 1097 977 019 SHWACHMAN H AM J DIS CHILD 96 6 958 020 BRASFIELD D PEDIATRICS 63 24 979 021 GENTZLER R CIRCULATION 50 324 974 022 DOUGLASS KH J NUCL MED 19 670 978 023 CANTOR A CIRCULATION 58 1123 978 024 POLGAR G PULMONARY FUNCTION TESTING IN 971 025 HOOPER RG CIRCULATION 50 1205 974 026 BOWDEN DH AM J MED 38 226 965 027 ZINMAN R CF CENT DIRECTO 17TH MTG ABST 977 029 ROBOTHAM JL J APPL PHYSIOL 44 703 978 030 ROBOTHAM JL J APPL PHYSIOL 46 411 979 031 BERGER HJ AM J CARDIOL 41 897 978 032 WEISSLER AM MOD CONCEPTS CARDIOVASC DIS 40 5 971 033 BORER JS N ENGL J MED 296 839 977 CT 1 LESTER LA J PEDIATR 97 742 980 2 BOVE AA PROG CARDIOVASC DIS 23 365 981 3 BERGER HJ AM J CARDIOL 47 950 981 4 MATTHAY RA MED CLIN NORTH AM 65 489 981 5 JACOBSTEIN MD CHEST 80 399 981 6 NEWTH CJL AM REV RESPIR DIS 124 463 981 7 MOSS AJ PEDIATRICS 70 728 982 8 COATES AL CHEST 82 543 982 9 HIRSCHFELD SS CHEST 82 524 982 10 ROBOTHAM JL CLIN CHEST MED 4 161 983 11 DENNIS JB SEM NUCL MED 13 292 983 12 HESS OM AM HEART J 106 1377 983 13 BADKE FR AM J CARDIOL 53 1187 984 14 CHERON G ACTA PAEDIATR SCAND 73 697 984 15 PARRISH M J PEDIATR 104 165 984 16 GOODING CA J PEDIATR 105 384 984 17 BENSON LN AM REV RESPIR DIS 130 987 984 18 MICHAEL JR AM REV RESPIR DIS 130 516 984 19 MOSKOWITZ WB PEDIATR PHARMACOL 5 139 985 20 LESTER LA SEM RESPIR MED 6 285 985 21 PANIDIS IP J AM COLL CARDIOL 6 701 985 22 PIEPSZ A PEDIATR PULMONOL 3 24 987 PN 79131 RN 01111 AN 79069472 AU Finkelstein-E. Hall-K. TI Aminoglycoside clearance in patients with cystic fibrosis [letter]. SO J-Pediatr. 1979 Jan. 94(1). P 163-4. MJ AMIKACIN: me. CYSTIC-FIBROSIS: me. KANAMYCIN: aa. KIDNEY: me. MN CHILD. HUMAN. EX We have been utilizing a patient's individual pharmacokinetic profile to plan and monitor aminoglycoside therapy, and we have often noted what appeared to be an extremely rapid elimination rate of gentamicin in our patients with cystic fibrosis. The patients with cystic fibrosis had significantly greater renal and total clearances than those without cystic fibrosis. If this observation could be extended to other drugs (such as the aminoglycosides), which are not normally subject to renal secretion, it would prove intriguing evidence for a possible primary renal defect in these patients. RF 001 VOGELSTEIN B J PEDIATR 91 333 977 002 CLARKE JT CLIN PHARMACOL THER 15 610 974 003 HEWITT WL AM J MED US AMIKACIN SYMP 62 863 977 004 JUSKO WJ PEDIATRICS 56 1038 975 005 YAFFE SJ J INFECT DIS 135 828 977 CT 1 MICHALSEN H ANTIMICROB AGENTS CHEMOTHER 19 1029 981 2 LEVY J J ANTIMICROB CHEMOTHER 10 227 982 3 KRAEMER R EUR J PEDIATR 138 172 982 4 THIRUMOORTHI MC J PEDIATR 102 941 983 5 MACDONALD NE J PEDIATR 103 985 983 6 PADOAN R J PEDIATR 103 320 983 7 ISLES A AM REV RESPIR DIS 127 417 983 8 NAHATA MC DEVELOP PHARM THER 7 221 984 9 MARTINI N J CLIN HOSP PHARM 9 303 984 10 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 11 SCHOFIELD DH DRUG INTEL CLIN PHARM 18 153 984 12 LEEDER JS CLIN PHARMACOL THER 36 355 984 13 LEVY J J PEDIATR 105 117 984 14 SPINO M J PEDIATR 105 829 984 15 COURCOL RJ INT J CLIN PHARMACOL RES 5 87 985 16 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 17 HORREVORTS AM CHEST 88 260 985 18 SPINO M J PEDIATR 107 64 985 19 GOLDFARB J J CLIN PHARMACOL 26 222 986 20 AUTRET E EUR J CLIN PHARMACOL 31 79 986 21 RUBIO TT AM J MED 81 73 986 22 KEARNS GL J PEDIATR 108 847 986 23 PEDERSEN SS ANTIMICROB AGENTS CHEMOTHER 31 594 987 PN 79132 RN 01112 AN 80008520 AU Loening-Baucke-V-A. Mischler-E. Myers-M-G. TI A placebo-controlled trial of cephalexin therapy in the ambulatory management of patients with cystic fibrosis. SO J-Pediatr. 1979 Oct. 95(4). P 630-7. MJ AMBULATORY-CARE. CEPHALEXIN: tu. CYSTIC-FIBROSIS: co. MN ADMINISTRATION-ORAL. ADOLESCENCE. ADULT. BACTERIAL-INFECTIONS: dt, et. CEPHALEXIN: ad, ae. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. CYSTIC-FIBROSIS: pp. FEMALE. HUMAN. INFANT. MALE. PLACEBOS. RESPIRATORY-TRACT-INFECTIONS: dt. SPUTUM: mi. AB The effects of oral administration of cephalexin were evaluated in a double-blind, placebo-controlled, crossover study in 17 patients with mild to moderate pulmonary disease due to cystic fibrosis. For two years, four-month periods with cephalexin were alternated with four-month placebo periods. Thus, patients served as their own control subjects. Fungal vulvovaginitis occurred in two patients during cephalexin therapy. During the 2 years of study, the rate of colonization with mucoid strains of Pseudomonas aeruginosa increased and disease severity deteriorated in patients initially colonized with P. aeruginosa. Short-term courses of sputum culture-specific antibiotics improved the course of some patients with mild to moderate pulmonary disease due to cystic fibrosis. Treatment with cephalexin decreased the frequency of respiratory illnesses, respiratory illnesses requiring antibiotics, and hospitalizations for respiratory illnesses in patients initially colonized with Staphylococcus aureus and/or Haemophilus influenzae, and also reduced colonization with these organisms. Improved weight gain in 16 of 17 patients was associated with periods of cephalexin therapy. Pulmonary function tests remained stable or improved in 10 or 14 patients. Disease severity improved in patients not colonized with P. aeruginosa. RF 001 SHWACHMAN H N ENGL J MED 241 185 949 002 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 003 LAWSON D IN: LAWSON D PROC 5TH INT CF 225 969 004 SHWACHMAN H AM J DIS CHILD 96 6 958 005 SHWACHMAN H PEDIATRICS 46 335 970 006 MATTHEWS LW IN: MANGOS JA 303 973 007 GIBSON LE PEDIATRICS 23 545 959 008 MONROE PW APPL MICROBIOL 18 214 969 009 HUANG NN J PEDIATR 59 512 961 010 LENNETTE EH MANUAL OF CLINICAL MICROBIOLO 974 011 CATLIN BW ANTIMICROB AGENTS CHEMOTHER 7 265 975 012 HUNTBERGER DV STATISTICAL INFERENCE IN BIOM 970 013 SNEDECOR GW STATISTICAL METHODS 967 014 LOURIA DB JAMA 182 1082 962 015 LORIAN V APPL MICROBIOL 15 564 967 016 BARTLETT JG AM REV RESPIR DIS 117 1019 978 017 STECHENBERG BW PEDIATRICS 58 532 976 018 BURNS MW LANCET 1 270 968 019 MAY JR LANCET 2 534 953 020$ MAY JR J CLIN MICROBIOL 17 254 964 021 REYNOLDS HY ANN INTERN MED 82 819 975 022 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 023 DOGGETT RG J PEDIATR 68 215 966 024 DOGGETT RG INFECT IMMUN 6 628 972 025 KULCZYCKI LL JAMA 240 30 978 026 MAY JR ARCH DIS CHILD 47 908 972 027 MEARNS MB ARCH DIS CHILD 47 902 972 CT 1 WIENTZEN R AM J DIS CHILD 134 1134 980 2 MARKS MI J PEDIATR 98 173 981 3 NOLAN G J PEDIATR 101 626 982 4 KRAEMER R EUR J PEDIATR 138 172 982 5 DAVIS PB J PEDIATR 102 177 983 6 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 7 DAVIS PB CHEST 85 802 984 8 HARRISON CJ PEDIATR PHARMACOL 5 7 985 9 DAVIS PB SEM RESPIR MED 6 261 985 10 MISCHLER EH SEM RESPIR MED 6 271 985 11 SMITH SR J CLIN HOSP PHARM 10 243 985 12 NELSON JD J PEDIATR 106 1030 985 13 GOTZ M MONATSSCHR KINDERHEILKD 133 718 985 14 FRIEND PA J INFECT 13 55 986 15 LITTLEWOOD JM J ROY SOC MED 79 55 986 16 FICK RB AM REV RESPIR DIS 133 418 986 PN 79133 RN 01113 AN 79111347 AU Rosenfeld-R. Spigelblatt-L. Chicoine-R. TI False positive sweat test, malnutrition, and the Mauriac syndrome. SO J-Pediatr. 1979 Feb. 94(2). P 240-2. MJ CHLORIDES: an. DWARFISM: co. HEPATOMEGALY: co. NUTRITION-DISORDERS: co. SWEAT: an. MN CASE-REPORT. CHILD. CYSTIC-FIBROSIS: di. DIABETES-MELLITUS-INSULIN-DEPENDENT: co. FALSE-POSITIVE-REACTIONS. FEMALE. HUMAN. SYNDROME. EX Mauriac syndrome is characterised by hepatomegaly, glycogen infiltration of the liver, retarded sexual maturation, and short stature in diabetic children. We report the first example of Mauriac syndrome, in a patient clinically malnourished and edematous, who had a transient false positive sweat test. Malnourished children who have a positive sweat test should have repeated sweat tests once the malnourished state has been reversed. This would eliminate error in the diagnosis of cystic fibrosis. RF 001 CONN JW ARCH INTERN MED 83 416 949 002 HARRIS RC PEDIATRICS 31 1044 963 003 LOBECK CC J PEDIATR 62 868 963 004 ROBINSON GC PEDIATRICS 30 797 962 005 MORSE WI N ENGL J MED 264 1021 961 006 MACE JW CLIN PEDIATR 10 285 971 007 DURAND P RECENT PROG MED 44 279 968 008 DURAND P J PEDIATR 75 665 969 009 MARDOFF L J PEDIATR 73 244 968 010 GIBSON LE PEDIATRICS 23 545 959 011 SCHALES O J BIOL CHEM 140 879 941 CT 1 SHWACHMAN H J PEDIATR 95 661 979 2 PARTINGTON MW AM J MED GENET 10 65 981 3 BEER SI ISR J MED SCI 17 1181 981 4 SCHWACHMAN H J PEDIATR 98 576 981 5 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 6 QUINTON PM ANNU REV MED 34 429 983 7 LUTHI M HELV PAEDIATR ACTA 38 149 983 8 DEMAY G ARCH FR PEDIATR 41 370 984 9 MOR A MINERAL ELECTROL METAB 11 155 985 10 CHRISTOFFEL KS J PEDIATR 107 231 985 11 BECK R ACTA PAEDIATR SCAND 75 639 986 12 RUDDY RM CLIN PEDIATR 26 83 987 PN 79134 RN 01114 AN 79111346 AU Porter-R-C. Cloutier-M-M. Brasfield-D-M. Mangos-J-A. Miale-T. Tiller-R-E. TI Cystic fibrosis in two black children with sickle cell anemia. SO J-Pediatr. 1979 Feb. 94(2). P 239-40. MJ ANEMIA-SICKLE-CELL: co. CYSTIC-FIBROSIS: co. MN CASE-REPORT. CHILD. FEMALE. HUMAN. INFANT. MALE. SUPPORT-U-S-GOVT-P-H-S. AB Sickle cell anemia and cystic fibrosis are the most frequent lethal inherited diseases in the black and white population, respectively. The occurrence of both diseases in the same individual has not been reported in the literature. This report presents two such patients and poses questions concerning the possible clinical implications of the simultaneous occurrence of both diseases. RF 001 GIBSON LE PEDIATRICS 23 545 959 002 STEINBERG AG AM J HUM GENET 12 416 960 003 KULCZYCKI LL CLIN PEDIATR 3 692 964 004 KULCZYCKI LL AM J DIS CHILD 127 64 974 005 HEFFER ET NY STATE J MED 74 2355 974 006 SULTZ HA AM J PUBLIC HEALTH 58 491 968 007 WINTROBE MM CLINICAL HEMATOLOGY 823 974 PN 79135 RN 01115 AN 79241401 AU Chase-H-P. Long-M-A. Lavin-M-H. TI Cystic fibrosis and malnutrition. SO J-Pediatr. 1979 Sep. 95(3). P 337-47. (REVIEW). MJ CYSTIC-FIBROSIS: co. NUTRITION-DISORDERS: et. MN CYSTIC-FIBROSIS: me. DIETARY-PROTEINS: me. ENERGY-METABOLISM. FATTY-ACIDS-ESSENTIAL: df. HUMAN. INFANT. IRON: df. LINOLEIC-ACIDS: bl. REVIEW. SELENIUM: df. TRACE-ELEMENTS: df. SUPPORT-U-S-GOVT-P-H-S. VITAMIN-A-DEFICIENCY: et. VITAMIN-B-12-DEFICIENCY: et. VITAMIN-D-DEFICIENCY: et. VITAMIN-E-DEFICIENCY: et. VITAMIN-K-DEFICIENCY: et. ZINC: df. AB Cystic fibrosis as a specific disease entity has been known to be associated with malnutrition for almost half a century. The importance of the malnutrition in the disease process remains unknown, as does much information about specific nutritional deficiencies in CF. Supplements for children with CF should include extra energy as fat or carbohydrate, a form of linoleic acid that can be absorbed, hydrolyzed protein, fat-soluble vitamins with vitamins A and E in a water emulsion, vitamin B12, probably B vitamins and vitamin C, and trace minerals. Routine measurements of nutritional status, particularly in children with growth failure, should be made at regular intervals and should include a three-day diet record and a simultaneous 72-hour stool fat determination. If fat malabsorption is not controlled by pancreatic enzymes, the use of antacids or cimetidine should be considered. The true role of nutrition in patients with CF will not be known until the appropriate studies are completed. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 REGAN PT N ENGL J MED 297 854 977 004 KATTWINKEL J PEDIATRICS 50 133 972 005 COX KL CYSTIC FIBROSIS 17 7 978 006 COX KL CYSTIC FIBROSIS 17 7 978 007 DIMAGNO EP N ENGL J MED 296 1318 977 009 KHAW K-T CF CLUB ABST 58 978 010 GRACEY M ARCH DIS CHILD 44 401 969 011 YASSA JG ARCH DIS CHILD 53 777 978 012 CHASE HP LANCET 2 236 978 013 GOODCHILD MC ARCH DIS CHILD 50 769 975 014 HANSEN AE PEDIATRICS 31 171 963 015 DUPONT J FED PROC 37 445 978 016 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 017 FRIEDMAN Z PEDIATRICS 58 650 976 018 ELLIOTT RB PEDIATRICS 57 474 976 019 ROSENLUND ML PEDIATRICS 59 428 977 020 FLEISHER DS J PEDIATR 64 341 964 021 DOLAN TF JR CLIN PEDIATR 9 295 970 022 STROBER W PEDIATRICS 43 416 969 023 WEST CD AM J DIS CHILD 72 251 946 024 ANFANGER H AM J DIS CHILD 77 425 949 025 FRISANCHO AR AM J CLIN NUTR 27 1052 974 026 VAUGHAN WJ SCIENCE 199 783 978 027 BERRY HK AM J DIS CHILD 129 165 975 028 UNDERWOOD BA PEDIATR RES 6 26 972 029 ABERNATHY RS AM J DIS CHILD 130 1360 976 030 SMITH FR J LAB CLIN MED 80 423 972 031 COUSINS RJ J NUTR 97 409 969 032 OWEN GM J PEDIATR 78 1042 971 033 WARWICK WJ CLIN PEDIATR 15 807 976 034 SCOTT J AM J MED 63 488 977 035 HAHN TJ J PEDIATR 94 38 979 036 HUBBARD VS J PEDIATR 94 84 979 037 LUBIN AH CF CLUB ABST 71 978 038 FARRELL PM J CLIN INVEST 60 233 977 039 OSKI FA J PEDIATR 70 211 967 040 DOLAN TF JR CLIN PEDIATR 15 597 976 041 GREEN J NUTR ABSTR REV 39 321 969 042 HOPE WC PROSTAGLANDINS 10 557 975 043 SCOTT ML IN: DELUCA HF 357 970 044 KAPLAN E N ENGL J MED 279 65 968 045 OPPENHEIMER EH BULL JOHNS HOPKINS HOSP 98 353 956 046 DARBY CW ARCH DIS CHILD 48 72 973 047 NITOWSKY HM AM J CLIN NUTR 10 368 962 048 TENGERDY RP INT ARCH ALLERGY 44 221 973 049 HARRIES JT ARCH DIS CHILD 46 341 971 050 WALTERS TR AM J DIS CHILD 124 641 972 051 KOMP DM CHEST 58 501 970 052 ANSELL JE JAMA 238 40 977 053 FILER LJ JR PEDIATRICS 48 483 971 054 MOSS MH AM J DIS CHILD 117 540 969 055 SUTHERLAND JM AM J DIS CHILD 113 524 967 056 VEEGER W N ENGL J MED 267 1341 962 057 DEREN JJ N ENGL J MED 288 949 973 058 RUCKER RW N ENGL J MED 289 329 973 059 WEBER AM N ENGL J MED 289 1001 973 060 SHWACHMAN H PEDIATRICS 25 155 960 061 DAVIDSON M IN: RUDOLPH AM 994 977 062 HALSTED JA LANCET 1 322 970 063 PALIN HD PEDIATR RES ABST 343 10 358 976 064 JACOB RA AM J CLIN NUTR 31 638 978 065 HAMBRIDGE KM PEDIATR CLIN NORTH AM 24 95 977 066 SMITH JC JR SCIENCE 181 954 973 067 SMITH JE J LAB CLIN MED 84 692 974 068 PEKAREK RS PROC SOC EXP BIOL MED 138 728 971 069 HEINRICH HC KLIN WOCHENSCHR 55 587 977 070 FISCHER WC J NUTR 107 1493 977 071 MISCHLER EH CF CLUB ABST 80 978 072 HELLER RM J PEDIATR 92 947 978 073 CHASE HP JAMA 230 1535 974 074 HANSEN L AM J CLIN PATHOL 46 133 966 CT 1 CHU JY NUTR REV 37 351 979 2 KARP RJ CLIN CHEST MED 1 375 980 3 LEE JA ANN CLIN LAB SCI 10 227 980 4 HUBBARD VS J PEDIATR 96 421 980 5 SHEPHERD R J PEDIATR 97 351 980 6 GOW R LANCET 2 1071 981 7 HAMILTON RM LIPIDS 16 374 981 8 PASSERO MA CLIN PEDIATR 20 264 981 9 SCHONI M HELV PAEDIATR ACTA 36 359 981 10 BELL L J CAN DIET ASSOC 42 62 981 11 CONGDEN PJ ARCH DIS CHILD 56 708 981 12 ANON J AM DIET ASSOC 78 443 981 13 PARK RW GASTROENTEROLOGY 81 1143 981 14 CASTILLO R J PEDIATR 99 583 981 15 CORRIGAN JJ J PEDIATR 99 254 981 16 GORDON EF J PEDIATR 99 341 981 17 ROSENFELD RG J PEDIATR 99 252 981 18 REED MD AM J DIS CHILD 135 829 981 19 BRANCHINI BR LANCET 1 618 982 20 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 21 LEVI N RIV ITAL PEDIATR 8 695 982 22 STAFFORD RJ CLIN GASTROENTEROL 11 141 982 23 MILLER M AM J CLIN NUTR 36 492 982 24 HUBBARD VS J AM DIET ASSOC 80 127 982 25 PENCHARZ PB J PEDIATR GASTROENTEROL NUTR 2 400 983 26 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 27 MERRITT RJ ADV NUTR RES 5 77 983 28 BRANCHINI BR PEDIATR RES 17 850 983 29 FARRELL PM J DENT CHILD 50 385 983 30 ATER JL PEDIATRICS 71 810 983 31 NEVE J ACTA PAEDIATR SCAND 72 437 983 32 SMITH AE J PEDIATR 103 820 983 33 HANSEN RC ARCH DERMATOL 119 51 983 34 HUBBARD VS EUR J PEDIATR 141 68 983 35 HOLT TL IRCS MED SCI BIOCHEM 12 370 984 36 SCHONI M PEDIATR RES 18 66 984 37 ZENTLERMUNRO PL GUT 25 500 984 38 ABRAMS CK J CLIN INVEST 73 374 984 39 HODGES P J AM DIET ASSOC 84 664 984 40 PARSONS HG J PEDIATR 105 958 984 41 VICHINSKY EP J PEDIATR 105 15 984 42 KUHN RJ CLIN PHARMACY 4 555 985 43 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 44 HUBBARD VS SEM RESPIR MED 6 308 985 45 ZENTLERMUNRO PL GUT 26 892 985 46 MAAK B Z KLIN MED 40 1115 985 47 HOLT TL AM J CLIN NUTR 41 1061 985 48 DODGE JA ACTA PAEDIATR SCAND SUPPL 317 1985 31 985 49 FERNANDES J ACTA PAEDIATR SCAND SUPPL 317 1985 5 985 50 HUGHES RL ACTA PAEDIATR SCAND SUPPL 317 1985 42 985 51 NEIJENS HJ ACTA PAEDIATR SCAND SUPPL 317 1985 38 985 52 ANON LANCET 1 249 986 53 ABMAN SH J PEDIATR GASTROENTEROL NUTR 5 393 986 54 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 55 LESTER LA J PARENT ENTERAL NUTR 10 289 986 56 KOHLER JA J CLIN HOSP PHARM 11 21 986 57 SOUTTER VL CLIN GASTROENTEROL 15 137 986 58 MISCHLER EH PEDIATR RES 20 36 986 59 DUHAMEL JF ARCH FR PEDIATR 43 229 986 60 CRAIGSCHMIDT MC AM J CLIN NUTR 44 816 986 61 MOORE MC AM J CLIN NUTR 44 33 986 62 GOODCHILD MC J ROY SOC MED 79 32 986 63 SHEPHERD RW J PEDIATR 109 788 986 64 DANIELS L J PEDIATR GASTROENTEROL NUTR 6 381 987 65 HUBBARD VS LIPIDS 22 424 987 66 ZAKRZEWSKI JT BR J CLIN PHARMACOL 23 19 987 67 YOUNGBERG CA DIG DIS SCI 32 472 987 PN 79136 RN 01116 AN 79069483 AU Moss-T-J. Austin-G-E. Moss-A-J. TI Preatherosclerotic aortic lesions in cystic fibrosis. SO J-Pediatr. 1979 Jan. 94(1). P 32-7. MJ AORTA: pa. ARTERIOSCLEROSIS: pa. ATHEROSCLEROSIS: pa. CYSTIC-FIBROSIS: pa. MN ADOLESCENCE. ADULT. ATHEROSCLEROSIS: co, et. CHILD. CYSTIC-FIBROSIS: co. ATHEROSCLEROSIS: pa. FEMALE. HUMAN. MALE. AB Patients with cystic fibrosis have fat malabsorption, providing an experimental model for evaluation of the hypothesis that a low-fat intake may prevent atherosclerosis. We studied the frequency and extent of aortic precursor lesions (fatty streaks, early fibromusculoelastic lesions, late fibromusculoelastic lesions) found at autopsy in this disease as well as in other patients with debilitating disorders but with no apparent impairment of fat absorption. Fatty streaks were less common in the cystic fibrosis group, as were the late fibromusculoelastic lesions. There was no significant difference in the frequency, length, or thickness of the early fibromusculoelastic lesions. The findings suggest that fat may be responsible for progression but not initiation of the fibromusculoelastic precursor lesions, and support the concept that early restriction of dietary fat may prevent, delay, or otherwise modify atherosclerosis in the adult. RF 001$ ANON ARTERIOSCLEROSIS DHEW PUB NO? 2 971 002 STRONG JP J ATHEROSCLER RES 9 251 969 003 HENNEKENS CH PEDIATRICS 58 211 976 004 GLAGOV S IN: WISSLER RW 9 972 005 MITCHELL S PEDIATRICS 49 165 972 006 SHOHL AT J PEDIATR 23 267 943 007 ANDERSEN DH AM J DIS CHILD 69 221 945 008 ROSS CAC ARCH DIS CHILD 30 316 955 009 BERRY HK AM J DIS CHILD 129 165 975 010 HOLMAN RL PEDIATRICS 24 34 959 011 ROSS R N ENGL J MED 295 369 976 012 WILENS SL AM J PATHOL 27 825 951 013 MCGILL HC JR LAB INVEST 18 560 968 014 SCHWARTZ CJ ARCH PATHOL 83 325 967 015 HARDIN NJ AM J PATHOL 73 301 973 016 GORE I ARCH PATHOL 84 49 967 017 STRONG JP AM J PATHOL 34 731 958 018 FRIEDMAN M ARCH PATHOL 79 345 965 CT 1 MOSS TJ J MENT DEFIC RES 24 137 980 2 MOSS AJ PEDIATRICS 70 728 982 PN 79137 RN 01117 AN 79069466 AU Sacher-M. Shmerling-D-H. TI PABA-test for diagnosis of exocrine pancreatic insufficiency [letter]. SO J-Pediatr. 1979 Jan. 94(1). P 159. MJ P-AMINOBENZOIC-ACID: du. AMINOBENZOIC-ACIDS: du. CYSTIC-FIBROSIS: di. MN P-AMINOBENZOIC-ACID: ur. CYSTIC-FIBROSIS: ur. HUMAN. INFANT. INFANT-NEWBORN. EX We have also found a significant difference between the 6-hour urinary PABA recovery in healthy controls and CF patients, with no overlap of the ranges, and a good correlation with fecal chymotrypsin activity. We can stress the usefulness of the test in this age group. In studying a group of healthy newborn infants, however, the results were quite different: The PABA recovery was smaller and showed greater variations and overlap with results in CF patients occurred. It is clear that the test is not applicable in newborn infants when performed in the described manner. One possible explanation is the physiologically limited glomerular filtration rate in the newborn infant, which could have delayed the PABA excretion. RF 001 NOUSIA-ARVANITAKIS S J PEDIATR 92 734 978 002 SACHER M ARCH DIS CHILD 53 639 978 PN 79138 RN 01118 AN 79241408 AU Fischer-E-G. Shwachman-H. Wepsic-J-G. TI Brain abscess and cystic fibrosis. SO J-Pediatr. 1979 Sep. 95(3). P 385-8. MJ BRAIN-ABSCESS: et. CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. BRAIN-ABSCESS: mi. CASE-REPORT. FEMALE. HUMAN. MALE. PROTEUS-INFECTIONS: et. PROTEUS-MIRABILIS. PSEUDOMONAS-INFECTIONS: et. STAPHYLOCOCCAL-INFECTIONS: et. AB Brain abscess has only recently been considered a complication of cystic fibrosis. Three patients are reported here and a fourth cited from the literature. All of our patients were young adults with advanced pulmonary disease. The bacteria involved were mouth organisms and were found in the sputum culture in only one of the patients. Resistance was present to previously given antibiotics. As patients with cystic fibrosis survive into adulthood, the risk of developing a brain abscess appears to increase. RF 001 SHWACHMAN H CURR PROB PEDIATR NO 10 8 978 002 DUFFNER PK ARCH NEUROL 36 27 979 003 EVERETT ED MEDICINE (BALTIMORE) 56 61 977 004 JAGELS AE J DENT RES 55 991 976 005 MOLINARI GF NEUROLOGY (MINN) 23 1205 973 006 PRUITT AA MEDICINE (BALTIMORE) 57 329 978 007 MATSON DD PEDIATRICS 27 772 961 008 FISCHBEIN CA AM J CARDIOL 34 97 974 009 NEWHOUSE M N ENGL J MED 295 1045 976 010 WOOD WG JR J EXP MED 94 521 951 011 GELLER A NEUROLOGY (MINN) 27 185 977 012 LEROUX BT BR HEART J 32 571 970 013 HARRIS P HUMAN PULMONARY CIRCULATI 962 014 WAGENVOORT CA PATHOLOGY OF PULMONARY HYPERT 977 015 COMROE JH JR LUNG CLINICAL PHYSIOLOGY AND 962 016 ROSENTHAL A AM J CARDIOL 27 162 971 017 ROSENTHAL A PEDIATRICS 59 588 977 CT 1 MCCARTHY MM CLIN PEDIATR 19 746 980 2 SHERMAN JM J PEDIATR 96 952 980 3 FISCHER EG AM J DIS CHILD 135 746 981 4 SANGER RG ORAL SURG 53 131 982 5 AYRES J BR J DIS CHEST 76 99 982 6 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 7 SHWACHMAN H J PEDIATR SURG 18 570 983 8 RABKIN CS ANN NEUROL 15 608 984 9 KLINE MW PEDIATR INFECT DIS 4 72 985 10 CANNY GJ THORAX 41 221 986 11 KURLAND G PEDIATRICS 78 1097 986 PN 79139 RN 01119 AN 81009005 AU Venugopal-S. Shandling-B. TI Meconium ileus: laparotomy without resection, anastomosis, or enterostomy. SO J-Pediatr-Surg. 1979 Dec. 14(6). P 715-8. MJ INFANT-NEWBORN-DISEASES: su. INTESTINAL-OBSTRUCTION: su. MECONIUM. MN CYSTIC-FIBROSIS: co. FEMALE. HUMAN. ILEOSTOMY. INFANT-NEWBORN. INTESTINAL-OBSTRUCTION: co, et. LAPAROTOMY. MALE. METHODS. AB During the 14 yr from 1965 through 1978, 49 infants presented shortly after birth with intestinal obstruction due to impacted meconium. Three of these patients did not have fibrocystic disease. Eight patients were cured by a Gastrografin enema. There were 18 patients who had complications that included associated atresia, volvulus, and/or peritonitis. Various operations were done including resection with either primary anastomosis or enterostomy or varieties of the foregoing. Twenty-three babies had the simple uncomplicated form of meconium ileus. Eleven of these underwent resection and six patients died. Twelve patients were treated by laparotomy, ileotomy through a purse-string suture and prolonged irrigations using acetylcysteine. Of this group only one succumbed. This latter course of management is recommended for patients with simple uncomplicated meconium ileus as it involves no resection, no enterostomy, nor any primary anastomosis. RF 001 DONNISON AB PEDIATRICS 37 833 966 002 KALAYOGLU M J PEDIATR SURG 6 290 971 003 ONEILL JA JR AM J SURG 119 99 970 004 NOBLETT HR J PEDIATR SURG 4 190 969 005 MCPARTLIN JF ARCH DIS CHILD 47 207 972 CT 1 GROSS K J PEDIATR SURG 20 431 985 2 GHORY MJ SURG CLIN NORTH AM 65 1083 985 PN 79140 RN 01120 AN 80008799 AU Morrissey-S-M. Tymvios-M-C. TI The effect of histamine, metiamide and cystic fibrosis factor on the electrophysiological properties of the frog's gastric mucosa [proceedings]. SO J-Physiol (Lond). 1979 Jun. 291. P 25P-26P. MJ CYSTIC-FIBROSIS: bl. GASTRIC-MUCOSA: de. HISTAMINE: pd. METIAMIDE: pd. THIOUREA: aa. MN ANIMAL. BIOLOGICAL-TRANSPORT-ACTIVE: de. CHLORIDES: me. ANURA. GASTRIC-MUCOSA: ph. HUMAN. HYDROGEN-ION-CONCENTRATION. IN-VITRO. MEMBRANE-POTENTIALS: de. AB The total active transport of Cl ions across the frog's gastric mucosa can be considered as: net Cl- flux (serosa to mucosa) = H+ + SSC (short-circuit current), all terms expressed as micromole cm-2 hr-1. The net chloride flux (Hogben, 1955; Durbin & Heinz, 1958) may be divided into two fractions: an acidic one which is equivalent to the acid secreted and an electromotive one which accounts for the gastric electromotive force. The changes in SCC can accordingly be used as a partial index to the activity of the chloride pump. Experiments were designed to investigate the effects of histamine, metiamide (H2 receptor antagonist), cystic fibrosis (CF) and non-CF plasma on the above parameters using the frog's isolated gastric mucosa. The SCC and the resistance of the mucosal preparation is measured by means of an automatic SCC apparatus (de Jesus, 1977). The H ion secretion is measured by pH meter. Results obtained (expressed as mean change (delta) plus or minus S.E. of mean) for histamine (10-4 M) (n = 10) show an initial increase of the SCC by 36.94 plus or minus 2.95 microA cm-2 and a parallel small increase in H+ output by 0.045 plus or minus 0.003 micromole cm-2 hr-1. This initial increase in SCC is followed by a drop to a steady-state level of 23.62 plus or minus 2.23 microA cm-2 and also by a large increase in H+ secretion 2.26 plus or minus 0.22 micromole cm-2 hr-1. These results support the work of Rehm (1961) in that histamine has an effect first on the Cl- pump and then on the H+ secretion. Metiamide (10-4 M) (n = 12) caused a drop in the SCC by 20.16 plus or minus 2.32 microA cm-2 and also reduced the acid secretion by 2.21 plus or minus 0.14 micromole cm-2 hr-1, thus suggesting an effect on the Cl- pump as well as on the H+ secretion. Significant differences are shown (P < 0.001) between CF and non-CF plasma results. The CF plasma (n = 8) increased the SCC by 17.25 plus or minus 2.94 microA cm-2 and H+ secretion by 2.38 plus or minus 0.43 micromole cm-2 hr-1. Non-CF plasma caused a slight increase in SCC by 5.17 plus or minus 1.1 microA cm-2 and had little effect on the H+ secretion, 0.34 plus or minus 0.01 micromole cm-2 hr-1 (n = 8). The effect of the CF plasma on the Cl- and H+ mechanism is of interest since in cystic fibrosis there is an abnormal increase of Cl- and Na+ in the sweat of patients (di Sant Agnese, Darling, Perera & She, 1953). RF 001 DURBIN RP J GEN PHYSIOL 41 1035 958 002 HOGBEN CAM AM J PHYSIOL 180 641 955 003 DE JESUS HC J PHYSIOL (LOND) 268 353 977 004 REHM WS AM J PHYSIOL 203 63 961 005 DI SANTAGNESE PA AM J DIS CHILD 86 618 953 PN 79141 RN 01121 AN 79218491 AU Bond-C-E. Fraser-P-A. Smaje-L-H. TI The chronically ligated submandibular salivary duct of the rabbit as a possible model for cystic fibrosis [proceedings]. SO J-Physiol (Lond). 1979 Apr. 289. P 25P. MJ CYSTIC-FIBROSIS: pp. SUBMANDIBULAR-GLAND: pp. MN ANIMAL. BIOLOGICAL-TRANSPORT. EPITHELIUM: cy, pp. LIGATION. RABBITS. SODIUM: ph. AB Schulz (1969) showed that the raised Na concentration in the sweat of children suffering from cystic fibrosis was due to a defect in Na reabsorption in the duct epithelium, but somewhat surprisingly the transepithelial potential difference (p.d.) is the same as that in normal controls. Na transport is also reduced in the chronically ligated salivary duct of the rabbit submandibular gland (Fraser & Smaje, 1978), and because p.d. is unaffected, the ligated gland may be a useful model of this aspect of cystic fibrosis. Here we would like to report a further similarity to cystic fibrosis; duct ligation leads to goblet cell hyperplasia which also occurs in the salivary glands in cystic fibrosis (Lev & Spicer, 1965). Sections of the salivary duct 6 microm thick were taken at 60 microm intervals from ducts which had been ligated for between 1 and 5 weeks and from their contralateral controls following fixation by immersion. Ligation resulted in an increase in luminal diameter by distension of the previously convoluted epithelium, but there was no significant change in epithelial surface area. The number of cell layers appeared to have increased however and there was a proliferation of goblet cells. The mucus in these cells was stained by alcian blue at pH 1 indicating the presence of sulphomucin. The mean goblet cell density rose from 7.62 plus or minus 4.73 mm-2 (mean plus or minus S.E. of the mean, n = 6) in controls to 114 plus or minus 49.6 in ligated ducts. The transepithelial Na flux in the same ducts was reduced from 10.1 plus or minus 0.79 n-mole cm-2 sec-1 to 1.6 plus or minus 0.84 while p.d. remained at -22 mV, similar to those results previously reported. There was no significant correlation between the degree of diminution of Na transport and the density of goblet cells but there was a positive correlation (r = 0.90) between the number of goblet cells in the control and contralateral ligated ducts. RF 001 FRASER PA J PHYSIOL (LOND) 285 24P 978 002 LEV R AM J PATHOL 46 23 965 003 SCHULZ IJ J CLIN INVEST 48 1470 969 PN 79142 RN 01122 AN 79175557 AU Hicks-J-M. TI Sweat tests [letter]. SO JAMA. 1979 May 25. 241(21). P 2264-5. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN HUMAN. EX I agree that sweat tests are frequently not performed reliably, but I wonder why the clinical chemist has been singled out as being responsible when most hospital laboratories are still directed by pathologists and not by professional clinical chemists. In addition to our laboratory, I know of many other hospital clinical chemistry laboratories in which the test is being performed properly. Furthermore, the Pediatric Committee of the American Association for Clinical Chemistry is working on a review article for the journal, Clinical Chemistry, regarding the correct way the sweat test should be run. Clearly, clinical chemists do not "lack interest." RF 001 SHWACHMAN H JAMA 240 2731 978 002 GIBSON LE PROC FOR QUANTITATIVE ION 975 CT 1 PRIEST JB JAMA 242 1970 979 PN 79143 RN 01123 AN 80009690 AU Priest-J-B. TI The sweat test for cystic fibrosis [letter]. SO JAMA. 1979 Nov 2. 242(18). P 1970. MJ CYSTIC-FIBROSIS: di. SWEAT: an. MN HUMAN. EX The major issue is not the "lack of interest of clinical chemists," but the actual performance of the test. Neither the pathologist nor the clinical chemist, in all likelihood, actually performs the test. Rather, it is the medical technologist or technician who carries out the actual procedure. It is a major part of our roles as educators and laboratory administrators to ensure that these people are adequately trained for the task and that the test meets high enough performance standards to provide a clinically relevant answer. RF 000 HICKS J JAMA 241 2264 979 000 SHWACHMAN H JAMA 240 2731 978 001 ROSENSTEIN BJ JAMA 240 1987 978 PN 79144 RN 01124 AN 80098014 AU Heinrich-H-C. Gabbe-E-E. Icagic-F. TI Immunoreactive serum trypsin in diseases of the pancreas. SO Klin-Wochenschr. 1979 Nov 15. 57(22). P 1237-8. MJ PANCREATIC-DISEASES: en. TRYPSIN: bl. MN ACUTE-DISEASE. CHRONIC-DISEASE. CYSTIC-FIBROSIS: en. HUMAN. PANCREATECTOMY. PANCREATITIS: en. RADIOIMMUNOASSAY. AB Immunoreactive serum trypsin was measured with a double antibody radioimmunoassay in normal subjects and patients with various diseases of the pancreas. The normal range is 115-350 ng/ml with a geometric mean of 212 ng/ml. No trypsin was found in serum after total duodenopancreatectomy, in about 75% of patients with cystic fibrosis and in a few patients with pancreas carcinoma or chronic pancreatitis. Reduced serum trypsin levels between 10 and 100 ng/ml were measured in the remaining 25% of cystic fibrosis and in one third of the patients with chronic pancreatitis. Serum trypsin was increased to 700-17,000 ng/ml in all patients with acute pancreatitis or during the acute phase of chronic pancreatitis. Absent or reduced serum trypsin is a reliable indicator of total or partial exocrine pancreatic insufficiency whereas considerably increased serum trypsin concentration do indicate acute pancreatitis. RF 001 DANDONA P BR MED J 2 1125 978 002 ELIAS E LANCET 2 66 977 003 GEOKAS MC J LAB CLIN MED 84 574 974 004 GOBELET CH SCHWEIZ MED WOCHENSCHR 108 1902 978 006 TEMLER RS BIOCHIM BIOPHYS ACTA 445 720 976 007 TEMLER RS ENZYME 22 249 977 CT 1 HEINRICH HC KLIN WSCHR 57 1295 979 2 FELBER JP RIC CLIN LAB 10 55 980 3 KOEHN HD CLIN CHEM 27 502 981 4 LANKISCH PG HEPATOGASTROENTEROLOGY 28 333 981 5 CARREL O HELV PAEDIATR ACTA 36 405 981 6 LANKISCH PG GUT 23 777 982 7 AMMANN RW KLIN WSCHR 60 243 982 8 MOLLERPETERSEN J CLIN CHIM ACTA 124 31 982 9 HEER M SCHWEIZ MED WOCHENSCHR 113 1950 983 10 KOOP H CLIN GASTROENTEROL 13 739 984 11 BORGSTROM A SCAND J GASTROENTEROL 19 220 984 12 VEZZADINI P AM J GASTROENTEROL 79 213 984 13 VEZZADINI P SURG GYNECOL OBSTET 158 319 984 14 DELFAVERO G RIC CLIN LAB 15 343 985 15 GERSMANN A MED WELT 36 23 985 16 DELFAVERO G KLIN WSCHR 63 603 985 17 NIEDERAU C GASTROENTEROLOGY 88 1973 985 18 NIEDERAU C GASTROENTEROLOGY 89 1451 985 PN 79145 RN 01125 AN 79155182 AU Oppenheimer-E-H. Rosenstein-B-J. TI Differential pathology of nasal polyps in cystic fibrosis and atopy. SO Lab-Invest. 1979 Apr. 40(4). P 445-9. MJ CYSTIC-FIBROSIS: co. HYPERSENSITIVITY-IMMEDIATE: co. NASAL-POLYPS: pa. MN ADOLESCENCE. ADULT. BASEMENT-MEMBRANE: pa. CHILD. CHILD-PRESCHOOL. EXOCRINE-GLANDS: pa. FEMALE. HUMAN. MALE. METAPLASIA. MUCOUS-MEMBRANE: pa. MUCUS. NASAL-POLYPS: co. SUPPORT-U-S-GOVT-P-H-S. AB Nineteen nasal polyps from 13 patients were examined histologically. Nine polyps from seven cystic fibrosis (CF) patients could be positively identified by a triad of observations: delicate, barely visible basement membrane of surface epithelium without submucosal hyalinization, lack of extensive infiltration of eosinophils (Giemsa stains), and a preponderance of acid mucin in glands and cysts of the polyp and in its surface mucous blanket (Alcian Blue-periodic acid- Schiff stains). Two polyps from two patients with CF and atopy showed the characteristic findings of CF without modification. Eight polyps from four atopic patients without CF were identified by the reverse triad of changes: extensive thickening of the epithelial basement membrane and its extension into the submucosa as an irregular hyaline membrane, high stromal eosinophil count, and mainly neutral mucin in mucous glands, cysts, and mucous blanket. Hyperplastic mucous glands, mucous cysts of variable sizes, focal metaplasia of surface epithelium, and acid mucin in goblet cells were considered nonspecific lesions. RF 001 BLUMSTEIN GI ARCH OTOLARYNGOL 83 266 966 003 KULCZYCKI LL JAMA 175 358 961 004 LAMB D J PATHOL 98 213 969 005 LAMB D BR J DIS CHEST 66 239 972 006 NEELY JG TRANS AM ACAD OPHTHALMOL OTOL 76 313 972 007 RULON JT ARCH OTOLARYNGOL 78 192 963 008 SHWACHMAN H PEDIATRICS 30 389 962 009 TAYLOR BW ARCH DIS CHILD 49 133 974 010 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 31 141 960 011 VAN METRE TE JR J ALLERGY CLIN IMMUNOL 49 131 972 CT 1 BATSAKIS JG HEAD NECK SURG 2 410 980 2 OPPENHEIMER EH HUM PATHOL 12 36 981 3 THAETE LG AM J ANAT 162 243 981 4 COLTEN HR N ENGL J MED 304 831 981 5 MILLS SE ARCH OTOLARYNGOL 108 530 982 6 STERN RC AM J DIS CHILD 136 1067 982 7 BUNNAG C ANN ALLERGY 50 126 983 8 KNOWLES MR SCIENCE 221 1067 983 9 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 10 STUTTS MJ PEDIATR RES 20 1316 986 11 DAVID TJ J ROY SOC MED 79 23 986 PN 79146 RN 01126 AN 80076794 AU King-D-N. Heeley-A-F. Walsh-M-P. Kuzemko-J-A. TI Sensitive trypsin assay for dried-blood specimens as a screening procedure for early detection of cystic fibrosis. SO Lancet. 1979 Dec 8. 2(8154). P 1217-9. MJ CYSTIC-FIBROSIS: di. TRYPSIN: bl. MN ADOLESCENCE. BLOOD-SPECIMEN-COLLECTION. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. HUMAN. INFANT. INFANT-NEWBORN. PREGNANCY. RADIOIMMUNOASSAY: mt. TRYPSIN: im. FEMALE. AB An immunoreactive-trypsin assay uses small dried-blood spots (diameter 1.25 mm) and is therefore suitable for incorporation in established neonatal screening schemes. Blood specimens from neonates with cystic fibrosis had trypsin levels greater than those in control subjects, thus confirming earlier findings. Trypsin levels were below normal in several older patients with cystic fibrosis. RF 001 CROSSLEY JR LANCET 1 472 979 002 DANDONA P LANCET 1 1032 979 CT 1 DOMINICI R RIC CLIN LAB 10 511 980 2 BONORA G SCAND J GASTROENTEROL 15 11 980 3 BURLINA A SCAND J GASTROENTEROL 15 35 980 4 TRAVERT G J GENET HUM 28 141 980 5 TRAVERT G NOUV PRESSE MED 10 2093 981 6 DURIE PR PEDIATR RES 15 1351 981 7 KIRBY LT CLIN CHEM 27 678 981 8 EVANS RT J CLIN PATHOL 34 911 981 9 BORGSTROM A ACTA PAEDIATR SCAND 70 619 981 10 CROSSLEY JR CLIN CHIM ACTA 113 111 981 11 TOBIN MJ IR J MED SCI 150 325 981 12 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 13 DURIE PR J PEDIATR GASTROENTEROL NUTR 1 337 982 14 BURLINA A IRCS MED SCI BIOCHEM 10 951 982 15 WONG LTK GUT 23 744 982 16 ZUCCHELLI GC J NUCL MED ALLIED SCI 26 35 982 17 BROWN RC J CLIN PATHOL 35 547 982 18 HEELEY AF ARCH DIS CHILD 57 18 982 19 BORGSTROM A ACTA PAEDIATR SCAND 71 621 982 20 BORGSTROM A ACTA PAEDIATR SCAND 71 849 982 21 HEELEY AF CLIN CHEM 29 2011 983 22 TRAVERT G ARCH FR PEDIATR 40 295 983 23 ANON PEDIATRICS 72 741 983 24 WILCKEN B J PEDIATR 102 383 983 25 MASOERO G AM J DIS CHILD 137 167 983 26 HALLINAN FM IR J MED SCI 152 409 983 27 HAMMOND KB LANCET 1 42 984 28 GOLDBERG DM CLIN PHYSIOL BIOCHEM 2 249 984 29 KOOP H CLIN GASTROENTEROL 13 739 984 30 BLYTHE SA CLIN BIOCHEM 17 277 984 31 HJELM M CLIN BIOCHEM 17 284 984 32 CASSIO A ACTA PAEDIATR SCAND 73 554 984 33 REARDON MC J PEDIATR 105 271 984 34 NAYLOR EW SEM PERINATOL 9 232 985 35 PLUMBE RM ANN CLIN BIOCHEM 22 408 985 36 DURIE PR PEDIATR RES 20 209 986 37 KUZEMKO JA J ROY SOC MED 79 2 986 38 BOURGUIGNON JP EUR J PEDIATR 144 547 986 39 BOWLING FG LANCET 1 826 987 PN 79147 RN 01127 AN 80076881 AU Harris-A. Benson-P-F. Fensom-A-H. TI Detection of cystic fibrosis with plasma hydrolases [letter]. SO Lancet. 1979 Dec 22-29. 2(8156-8157). P 1371. MJ ACID-PHOSPHATASE: bl. CYSTIC-FIBROSIS: di. MANNOSIDASES: bl. MN COMPARATIVE-STUDY. HEAT. HETEROZYGOTE-DETECTION. HUMAN. OILS: du. PARAFFIN: du. EX Dr Hosli and Mrs Hosli reported that differential thermolability of plasma alpha-mannosidase and plasma acid phosphatase allowed discrimination between normal people, cystic fibrosis carriers, and patients. We have repeated these experiments, paying special attention to the technical details given in the Hoslis's paper. In a separate series of experiments we used larger volumes of plasma for each assay, which was performed either with or without non-fluorescent paraffin oil. In both cases the blood samples were processed exactly as outlined in Hosli and Vogt's paper. There were no significant differences in the heat stability of plasma alpha-mannosidase and acid phosphatase between the three groups. RF 000 PATRICK AD LANCET 2 1015 979 000 HOSLI P LANCET 2 543 979 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 3 HALLINAN F MED HYPOTHESES 7 793 981 4 KATZNELSON D ISR J MED SCI 17 687 981 5 HULTBERG B CLIN CHIM ACTA 112 167 981 6 SCANLIN TF CLIN CHIM ACTA 114 269 981 7 HEELEY AF CLIN CHEM 29 2011 983 8 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 9 OGLESBEE LH CLIN CHIM ACTA 143 135 984 PN 79148 RN 01128 AN 80076806 AU Applegarth-D-A. Davidson-A-G. Kirby-L-T. Bridges-M. Sorensen-P. Wong-L-T. Hardwick-D-F. TI Dried blood spot screening for cystic fibrosis [letter]. SO Lancet. 1979 Dec 8. 2(8154). P 1236-7. MJ CYSTIC-FIBROSIS: di. TRYPSIN: bl. MN AGE-FACTORS. BLOOD-SPECIMEN-COLLECTION. ENZYME-TESTS: mt. HUMAN. INFANT. INFANT-NEWBORN. EX We have measured immunoreactive trypsin IRT activity in dried blood spot samples and have found abnormally high levels in infants with cystic fibrosis (CF). We agree with Crossley et al. that this test deserves further investigation as a screen for CF in the newborn. The low value for the 94 week infant is consistent with their finding that, in children with low pancreatic enzyme output, the increased IRT results are restricted to the first few months of life. It remains to be proved whether older CF patients with residual pancreatic enzyme secretion do indeed continue to have increased IRT levels. If they do, the IRT test would also deserve further investigation as a tool, to be used in conjunction with stool pancreatic enzyme activity, in smaller centres who have had problems with inaccurate sweat tests. RF 001 CROSSLEY JR LANCET 1 472 979 002 STARKEY PM IN: BARRETT AJ 663 977 003 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 CT 1 DOMINICI R RIC CLIN LAB 10 511 980 2 BURLINA A SCAND J GASTROENTEROL 15 35 980 3 DURIE PR PEDIATR RES 15 1351 981 4 KIRBY LT CLIN CHEM 27 678 981 5 TOBIN MJ IR J MED SCI 150 325 981 6 WONG LTK GUT 23 744 982 7 ZUCCHELLI GC J NUCL MED ALLIED SCI 26 35 982 8 HEELEY AF ARCH DIS CHILD 57 18 982 9 MASOERO G AM J DIS CHILD 137 167 983 10 HALLINAN FM IR J MED SCI 152 409 983 11 NAYLOR EW SEM PERINATOL 9 232 985 PN 79149 RN 01129 AN 79243319 AU Lieberman-J. TI Age dependence of serum angiotensin-converting enzyme activity [letter]. SO Lancet. 1979 Jul 28. 2(8135). P 196. MJ KININASE-II: bl. MN ADOLESCENCE. AGE-FACTORS. CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en. GRANULOMATOUS-DISEASE-CHRONIC: en. HUMAN. EX We examined the age dependence of serum angiotensin-converting enzyme (SACE) activity in healthy controls. While we found no age dependence in controls older than 20 years, those under 20 had much increased SACE values. We feel that it is inappropriate to evaluate SACE activity in patients under 20 years old with diagnostic criteria determined in adults. - Dr Romer and colleagues reported high activities of SACE in 3 patients out of 10 with chronic granulomatous disease (CGD) and concluded that "CGD is associated with increased levels of SACE". Unfortunately, Romer et al. did not study an adequate paedeatric age control group, and their mean values are compared primarily with that in 5 children with cystic fibrosis. Our most recent studies confirm our earlier observation that children under the age 19 years definitely have higher mean SACE values than do adults. Cystic-fibrosis patients should not be used for comparison since they tend to have lower SACE levels than comparable aged controls. In my opinion patients with CGD, according to the data published by Romer et al., show no increase in SACE levels for their age. RF 000 ROMER FK LANCET 1 1237 979 001 LIEBERMAN J AM J MED 59 365 975 002 ROHRBACH MS AM REV RESPIR DIS 119 761 979 003 LIEBERMAN J AM J MED 59 365 975 CT 1 SANTONASTASO CL ITAL J GASTROENTEROL 16 9 984 PN 79150 RN 01130 AN 79198307 AU Brock-D-J. Hayward-C. TI Methylumbelliferyl-guanidinobenzoate reactive proteases and prenatal diagnosis of cystic fibrosis [letter]. SO Lancet. 1979 Jun 9. 1(8128). P 1245-6. MJ AMNIOTIC-FLUID: en. CYSTIC-FIBROSIS: di. PEPTIDE-HYDROLASES: an. MN CYSTIC-FIBROSIS: en. FEMALE. HUMAN. HYMECROMONE. PREGNANCY. EX For several reasons the supernatant fraction of amniotic fluid is not often used in the prenatal detection of inborn errors of metabolism. In early pregnancy the bulk of the protein in amniotic fluid is derived from the maternal circulation, and this can obscure the more critical contribution from the fetus needed for prenatal diagnosis. 10 - 30% of amniotic fluids are significantly contaminated with maternal blood, and if the enzyme in question is present in this blood (as 4-methylumbelliferyl guanidinobenzoate [MUGB] protease is), this can further distort diagnostic conclusions. For this reason we have examined the variation of MUGB protease in a heterogenous collection of amniotic fluids. No significant differences in titre between individual weeks in the second trimester were observed. A further 104 second-trimester amniotic fluids from pregnancies with abnormal outcome were also investigated. The means of the four groups were not significantly different from that of the normals and the ranges were very similar. We have not yet had the opportunity to examine an amniotic fluid from a known case of cystic fibrosis. Diagnosis of cystic fibrosis could probably be further refined by coupling amniotic-fluid protease titration and isoelectric focusing on polyacrylamide gel. RF 001 WALSH-PLATT M PEDIATR RES 12 874 978 002 RAO GJS ENZYME 23 314 978 003 JAMESON GW BIOCHEM J 131 107 973 004 SUTCLIFFE RG J OBSTET GYNAECOL BR COMMONW 80 721 973 005 DANN LG LANCET 2 405 978 CT 1 DANN LG LANCET 2 907 979 2 BROCK DJH LANCET 1 619 982 3 CRISTOL P SEM HOP PARIS 58 449 982 4 GREEN JR EUR J PEDIATR 139 35 982 5 BROCK DJH PRENAT DIAGN 3 1 983 6 HODSON ME POSTGRAD MED J 60 225 984 7 SHAW LMA J OBSTET GYNAECOL BR COMMONW 6 1 985 PN 79151 RN 01131 AN 79198291 AU Phillips-B. Davis-J-A. Goode-M. TI Screening for cystic fibrosis [letter]. SO Lancet. 1979 Jun 9. 1(8128). P 1238-9. MJ CYSTIC-FIBROSIS: en. PEPTIDE-HYDROLASES: bl. MN CHILD. HUMAN. TRYPSIN-INHIBITORS: bl. TRYPSIN: bl. EX Our cystic-fibrosis children usually either have severe lung disease with mild steatorrhea or severe maldigestion with lesser pulmonary problems. We therefore measured serum-proteases in cystic-fibrosis patients and controls to see if there was any support for the following hypothesis: since lack of antitrypsin is associated with lung damage, plasma-trypsin in excess of antitrypsin might be responsible for the lung damage in cystic fibrosis. General protease and trypsin levels were measured spectrophotometrically using a modification of the method of Erlanger, Kojowsky and Cohen. Our result showed no significant difference between protease and trypsin levels in cystic-fibrosis patients and controls. The difference between our results and those of Crossley et al. may be explained by the differing measuring technique used. Our trypsin levels were measured spectrophotometrically and theirs by radioimmunoassay. RF 000 CROSSLEY JR LANCET 1 472 979 PN 79152 RN 01132 AN 79198289 AU Romer-F-K. Faber-V. Koch-C. Pedersen-F-K. Friis-B. Johansen-K-S. Taudorff-E. TI Serum-angiotensin-converting-enzyme in chronic granulomatous disease [letter]. SO Lancet. 1979 Jun 9. 1(8128). P 1237. MJ GRANULOMATOUS-DISEASE-CHRONIC: en. KININASE-II: bl. MN ADOLESCENCE. ADULT. AGED. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: en. FEMALE. GRANULOMATOUS-DISEASE-CHRONIC: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. INFANT. KININASE-II: ge. MALE. MIDDLE-AGE. EX We have studied serum-angiotensin-converting-enzyme (SACE) in sarcoidosis and other granulomatous disorders. We have measured SACE in 10 randomly chosen, consecutive patients with chronic granulomatous disease (CGD) and in some of their family members and in 5 children with cystic fibrosis and severe chronic pulmonary involvement. SACE levels in the patient with CGD differed significantly from those of normal controls and those of family members and patients with cystic fibrosis. Our preliminary studies show that CGD is associated with increased levels of SACE. RF 001 CUSHMAN DW BIOCHEM PHARMACOL 20 1637 971 002 LIEBERMAN J AM J MED 59 365 975 004 STUDDY P LANCET 2 1331 978 005 LIEBERMAN J CLIN RES 25 145A 977 006 GEE JBL LUNG 155 243 978 007 SILVERSTEIN E AM J CLIN PATHOL 69 467 978 008 PERKS WH THORAX 33 101 978 CT 1 ROMER FK LANCET 2 860 979 2 ROMER FK ACTA MED SCAND 1 984 3 FARGE D AM J NEPHROL 6 21 986 PN 79153 RN 01133 AN 79133794 AU Crossley-J-R. Elliott-R-B. Smith-P-A. TI Dried-blood spot screening for cystic fibrosis in the newborn. SO Lancet. 1979 Mar 3. 1(8114). P 472-4. MJ BLOOD-STAINS. CYSTIC-FIBROSIS: di. TRYPSIN: bl. MN ANTIGENS. BLOOD-SPECIMEN-COLLECTION. CYSTIC-FIBROSIS: en. HUMAN. INFANT. INFANT-NEWBORN. RADIOIMMUNOASSAY. TRYPSIN: im. AB Serum-immunoreactive-trypsin (I.R.T.) was measured in children with cystic fibrosis (C.F.) and a variety of controls. In the first few months of life all C.F. children had a raised serum-I.R.T. A dried blood-spot assay for I.R.T. was established and has potential as a screening test for C.F. in the newborn. RF 001 CROSSLEY JR LANCET 2 1093 977 002 FRIER BM GUT 17 685 976 003 ELIAS E LANCET 2 66 977 004 DANDONA P BR MED J 2 1125 978 CT 1 KING DN LANCET 2 1217 979 2 STAGG BH ANN CLIN BIOCHEM 16 147 979 3 BURLINA A SCAND J GASTROENTEROL 15 35 980 4 TARNOKY AL J ROY SOC MED 73 73 980 5 ROMEO G J LAB CLIN MED 95 116 980 6 MASTELLA G RIV ITAL PEDIATR 7 581 981 7 BURLINA A IRCS MED SCI BIOCHEM 10 951 982 8 MASOERO G DIG DIS SCI 27 1089 982 9 SANDER J MONATSSCHR KINDERHEILKD 130 843 982 10 PITT D MED J AUST 1 333 983 11 HEELEY AF CLIN BIOCHEM 16 369 983 12 REY F CLIN BIOCHEM 16 23 983 13 BUCHANAN KD SCAND J GASTROENTEROL 18 155 983 14 HEELEY AF CLIN CHEM 29 2011 983 15 KIRBY LT CLIN CHEM 29 1559 983 16 LUTHI M HELV PAEDIATR ACTA 38 149 983 17 TRAVERT G ARCH FR PEDIATR 40 295 983 18 WILCKEN B ARCH DIS CHILD 58 863 983 19 CARRERE J J IMMUNOL METH 60 235 983 20 ANON PEDIATRICS 72 741 983 21 LYON ICT NZ MED J 96 673 983 22 WILCKEN B J PEDIATR 102 383 983 23 MASOERO G AM J DIS CHILD 137 167 983 24 HALLINAN FM IR J MED SCI 152 409 983 25 JUNGLEE D BR MED J 286 1693 983 26 GOLDBERG DM CLIN PHYSIOL BIOCHEM 2 249 984 27 RYLEY HC PRENAT DIAGN 4 303 984 28 ANDRIULLI A J CLIN GASTROENTEROL 6 239 984 29 KOOP H CLIN GASTROENTEROL 13 739 984 30 FIGARELLA C RIC CLIN LAB 14 349 984 31 BLYTHE SA CLIN BIOCHEM 17 277 984 32 HJELM M CLIN BIOCHEM 17 284 984 33 BORGSTROM A PEDIATR RES 18 957 984 34 PLESS IB PEDIATR CLIN NORTH AM 31 259 984 35 HODSON ME POSTGRAD MED J 60 225 984 36 BROCK DJH BR J OBSTET GYNAECOL 91 449 984 37 SCHONI MH J PEDIATR 104 691 984 38 REARDON MC J PEDIATR 105 271 984 39 SANDER J KLIN PAEDIATR 196 224 984 40 SHEPS SB JAMA 252 2418 984 41 NAYLOR EW SEM PERINATOL 9 232 985 42 ROSALKI SB CLIN CHEM 31 779 985 43 SZIBOR R Z KLIN MED 40 313 985 44 REDDY S AUST J EXP BIOL MED SCI 63 667 985 45 DURIE PR J PEDIATR 106 233 985 46 CLEGHORN G J PEDIATR 107 377 985 47 STOLLINGER O WIEN MED WOCHENSCHR 135 307 985 48 MELLIS CM MED J AUST 143 227 985 49 BOLLBACH R EUR J PEDIATR 144 167 985 50 LAROCHE D J PEDIATR GASTROENTEROL NUTR 5 404 986 51 MOORE DJ CLIN BIOCHEM 19 303 986 52 DURIE PR PEDIATR RES 20 209 986 53 CARRERE J BIOL NEONATE 49 113 986 54 RAFFLES AKM ACTA PAEDIATR SCAND 75 502 986 55 DODGE JA J ROY SOC MED 79 27 986 56 KUZEMKO JA J ROY SOC MED 79 2 986 57 GARCIAPUGES AM GASTROENTEROLOGY 91 17 986 58 BOURGUIGNON JP EUR J PEDIATR 144 547 986 59 ROBERTS DD ARCH BIOCHEM BIOPHYS 245 292 986 60 CARRERE J BIOCHIM BIOPHYS ACTA 883 46 986 61 BLANDIN C J BIOPHYS BIOMECAN 11 5 987 62 SARLES J ARCH FR PEDIATR 44 311 987 PN 79154 RN 01134 AN 79155633 TI Allergy and bronchial hyperreactivity in cystic fibrosis [editorial]. SO Lancet. 1979 Mar 31. 1(8118). P 708. MJ ASTHMA: et. BRONCHI: im. CYSTIC-FIBROSIS: im. MN ALLERGENS: im. CHILD. HUMAN. EX A little-known feature of cystic fibrosis is the frequent occurrence of positive skin-prick tests to environmental allergies. In the normal population only about half those with positive prick tests actually have clinical allergic disease; so, are the positive skin-tests of cystic fibrosis clinically important? In particular, do they mean a high prevalence of asthma, overt or hidden, amongst these patients? A general clinical impression is that asthma is no more frequent amongst cystic fibrosis than in the normal population. The often positive skin tests, although of considerable immunological interest, do not necessarily imply additional clinical disease. RF 001 WARNER JO ARCH DIS CHILD 51 507 976 002 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 003 GODFREY RC CLIN ALLERGY 6 79 976 004 COUNAHAN R ARCH DIS CHILD 50 477 975 005 MITCHELL I J PEDIATR 93 744 978 006 CHAN H J ALLERGY CLIN IMMUNOL 56 323 975 007 SKORECKI K ACTA PAEDIATR SCAND 65 39 976 008 DAY G ARCH DIS CHILD 48 355 973 009 EMPEY DW AM REV RESPIR DIS 113 131 976 CT 1 YANKAUER A AM J PUBLIC HEALTH 69 762 979 2 ORMEROD LP THORAX 35 768 980 3 VANASPEREN P AM J DIS CHILD 135 815 981 4 CONNOLLY J BR J HOSP MED 33 24 985 PN 79155 RN 01135 AN 79177182 AU Dandona-P. Hodson-M. Bell-J. Ramdial-L. Batten-J-C. TI Serum-immunoreactive-trypsin concentrations in cystic fibrosis [letter]. SO Lancet. 1979 May 12. 1(8124). P 1032. MJ CYSTIC-FIBROSIS: di. TRYPSIN: bl. MN ADOLESCENCE. ADULT. AGE-FACTORS. CHILD. CHILD-PRESCHOOL. HETEROZYGOTE-DETECTION. HUMAN. INFANT-NEWBORN. TRYPSIN: im. EX We measured immunoreactive trypsin (IRT) concentrations in 32 patients with established cystic fibrosis and in 16 parents (heterozygotes) of patients with CF. 29 patients (91%) and 6 parents (38%) had abnormal trypsin concentrations. We found that in only 3 out of the 29 patients with abnormal IRT was the concentration supranormal. Our data suggest that low IRT concentrations are an excellent index of diminished pancreatic reserve in CF and that serum-IRT concentration would be a useful diagnostic test. The fact that CF patients at birth invariably have very high IRT concentrations which fall with age and the progress of the disease, allied with the fact that heterozygotes have high concentrations, supports the previous suggestion that an uncontrolled hypersecretion of zymogen granules is the basic cellular defect in CF. RF 000 CROSSLEY JR LANCET 1 472 979 CT 1 KING DN LANCET 2 1217 979 2 DOMINICI R RIC CLIN LAB 10 511 980 3 FELBER JP RIC CLIN LAB 10 55 980 4 BURLINA A SCAND J GASTROENTEROL 15 35 980 5 VEZZADINI P SCAND J GASTROENTEROL 15 29 980 6 FOO Y J CLIN PATHOL 33 1102 980 7 DUFFY MJ CLIN CHIM ACTA 103 233 980 8 ODONNELL MD CLIN CHIM ACTA 104 265 980 9 DANDONA P LANCET 1 380 981 10 DURIE PR PEDIATR RES 15 1351 981 11 DURIE PR J PEDIATR GASTROENTEROL NUTR 1 337 982 12 HEELEY AF CLIN CHEM 29 2011 983 13 MASOERO G AM J DIS CHILD 137 167 983 14 ANDRIULLI A J CLIN GASTROENTEROL 6 239 984 15 KOOP H CLIN GASTROENTEROL 13 739 984 16 HJELM M CLIN BIOCHEM 17 284 984 17 HODSON ME POSTGRAD MED J 60 225 984 18 DURIE PR PEDIATR RES 20 209 986 PN 79156 RN 01136 AN 79133965 AU Walsh-M-M. Nadler-H-L. TI Methylumbelliferyl-guanidinobenzoate reactive proteases in amniotic fluid: possible marker for cystic fibrosis [letter]. SO Lancet. 1979 Mar 17. 1(8116). P 622. MJ AMNIOCENTESIS. AMNIOTIC-FLUID: en. CYSTIC-FIBROSIS: di. HYMECROMONE: du. PEPTIDE-HYDROLASES: an. UMBELLIFERONES: du. MN CYSTIC-FIBROSIS: en. FEMALE. HUMAN. PREGNANCY-TRIMESTER-SECOND. PREGNANCY. EX Previous studies in our laboratory have demonstrated significant differences in the extent of protease reactivity with MUGB (4-methylumbelliferyl guanidinobenzoate, a sensitive active site titrant of many serine proteases) when plasma and cultivated skin fibroblasts from patients with cystic fibrosis (CF), obligate heterozygotes, and controls were compared. Because of these observations, and the potential value of amniotic fluid for the prenatal detection of cystic fibrosis, these studies were extended to human amniotic fluid obtained by amniocentesis done for the intrauterine detection of genetic and other fetal abnormalities. An amniotic-fluid sample obtained from a woman who was subsequently delivered of a baby with CF showed a much reduced level of MUGB reactivity similar to the relative deficiency observed in plasma and cultivated skin fibroblasts from patients with CF. These observations suggest that amniotic-fluid protease activity may be a useful marker for the prenatal detection of CF. RF 001 JAMESON GW BIOCHEM J 131 107 973 002 WALSH-PLATT M AM J HUM GENET 29 111A 977 003 RAO GJS ENZYME 23 314 978 004 WALSH-PLATT M PEDIATR RES 12 129 978 005 WALSH-PLATT M PEDIATR RES 12 874 978 CT 1 DANN LG LANCET 2 907 979 2 MAHONEY MJ CLIN PERINATOL 6 255 979 3 WALSH MMJ PEDIATR RES 14 353 980 4 FREZAL J ARCH FR PEDIATR 38 217 981 5 BRANCHINI BR PEDIATR RES 17 850 983 6 BRIDGES MA ANN NY ACAD SCI 421 360 983 7 KUZEMKO JA J ROY SOC MED 79 2 986 PN 79157 RN 01137 AN 80053578 AU Patrick-A-D. Ellis-R-B. TI Plasma hydrolases in cystic fibrosis [letter]. SO Lancet. 1979 Nov 10. 2(8150). P 1015-6. MJ CYSTIC-FIBROSIS: en. HYDROLASES: bl. MN HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYDROLASES: ge. EX Dr Hosli and Esther Vogt reported that the alpha-mannosidase and acid phosphatase activities of cystic fibrosis plasma assayed at pH 5.4 are abnormally thermolabile at 41 degrees and 36.5 degrees C, respectively, and that the levels of residual activity after heat inactivation clearly distinguish between CF patients, CF carriers, and normal subjects. We have repeated these experiments under identical conditions and found no differences between the three groups. RF 000 HOSLI P LANCET 2 543 979 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 SACK J LANCET 2 268 980 3 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 4 KATZNELSON D ISR J MED SCI 17 687 981 5 HULTBERG B CLIN CHIM ACTA 112 167 981 6 SCANLIN TF CLIN CHIM ACTA 114 269 981 7 HEELEY AF CLIN CHEM 29 2011 983 8 OGLESBEE LH CLIN CHIM ACTA 143 135 984 9 KUZEMKO JA J ROY SOC MED 79 2 986 PN 79158 RN 01138 AN 80031544 TI Mechanical chest physiotherapy [editorial]. SO Lancet. 1979 Oct 6. 2(8145). P 729. MJ CYSTIC-FIBROSIS: th. PERCUSSION: is. PHYSICAL-THERAPY: mt. SPUTUM. MN ADOLESCENCE. ADULT. CHILD. DRAINAGE. HUMAN. EX Despite some critical debate lately about the usefulness of physiotherapy in certain chest conditions, the fact remains that many patients voluntarily undertake regular postural bronchial drainage to help their sputum. A substantial number also find rapid chest percussion over affected lung segments useful in loosening the sputum. Chest percussion by a mechanical device controlled by the patient himself seems worthy to trial. Maxwell and Redmond have compared a commercial device with manual percussion during postural bronchial drainage. The two techniques resulted in expectoration of similar quantities of sputum, although all but one patient felt subjectively that the machine delivered better-quality percussion. Before parents and others are advised to buy percussing machines, we need a formal study of postural drainage with and without chest percussion. RF 001 NEWTON DAG LANCET 2 228 978 002 GRAHAM WGB N ENGL J MED 299 624 978 003 FLOWER KA BR MED J 2 630 979 004 MAXWELL M ARCH DIS CHILD 54 542 979 CT 1 MACHIN GA AM J MED GENET 9 341 981 2 TURKEL SB CLIN PERINATOL 9 613 982 3 CARSWELL F EUR J RESPIR DIS 65 53 984 PN 79159 RN 01139 AN 80031735 AU Hirani-S. Winchester-B-G. TI Detection of cystic fibrosis homozygotes and heterozygotes with plasma [letter]. SO Lancet. 1979 Oct 27. 2(8148). P 906-7. MJ CYSTIC-FIBROSIS: di. GENETIC-TECHNICS. MANNOSIDASES: bl. MN CHILD. CYSTIC-FIBROSIS: fg. HETEROZYGOTE-DETECTION. HOMOZYGOTE. HUMAN. HYDROGEN-ION-CONCENTRATION. EX Dr Hosli and Esther Vogt base their diagnosis of heterozygotes and homozygotes for cystic fibrosis on the thermal stability in plasma or extracellular fluids of alpha-mannosidase and/or phosphatase activity assayed at pH 5.4. The activity of alpha-mannosidase in plasma and this pH is a composite one: both acidic alpha-mannosidase, which has a pH optimum of 4.25 and intermediate alpha-mannosidase, which has a pH optimum of 5.6 - 6.0, will contribute to the activity measured at pH 5.4. These two forms of alpha-mannosidase are structurally and genetically distinct and have different kinetics and thermostabilities. Therefore the total alpha-mannosidase activity at pH 5.4 and the physicochemical properties of this activity will depend upon the relative proportions of the two types of alpha-mannosidase in the sample. The proportions of the two forms vary widely in normal plasma, especially in plasma from children. Although the data of Hosli and Vogt suggest that the total alpha-mannosidase at pH 5.4 does not differ significantly between controls, CF heterozygotes and CF homozygotes, they do not show whether the proportions of acidic and intermediate alpha-mannosidase have changed. An increase in the proportion of the thermolabile intermediate alpha-mannosidase in CF would be manifested as a decreased thermostability of alpha-mannosidase at pH 5.4. Alternatively a mutation in the normally stable acidic alpha-mannosidase leading to a less stable form would also be reflected as a decreased stability in the activity at pH 5.4, especially if the proportion of acidic alpha-mannosidase was increased in CF. These situations should also be considered as possible explanations of the observations, in addition to the one favoured by Hosli and Vogt. RF 000 HOSLI P LANCET 2 543 979 001 HIRANI S CLIN CHIM ACTA 81 135 977 002 HIRANI S BIOCHEM J 179 583 979 003 HOSLI P FEBS LETTERS 104 271 979 004 HULTBERG B BIOCHIM BIOPHYS ACTA 481 573 977 CT 1 BUTTERWORTH J CLIN CHIM ACTA 108 347 980 2 HALLINAN F MED HYPOTHESES 7 793 981 3 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 PN 79160 RN 01140 AN 80031736 AU Dann-L-G. Blau-K. TI Arginine esterase in amniotic fluid: possible marker for cystic fibrosis [letter]. SO Lancet. 1979 Oct 27. 2(8148). P 907. MJ AMNIOTIC-FLUID: en. CYSTIC-FIBROSIS: di. ESTERASES: an. MN ARGININE. BENZOATES: du. COMPARATIVE-STUDY. COUMARINS: du. CYSTIC-FIBROSIS: en, fg. FEMALE. GENETIC-MARKER. HUMAN. METHODS. PREGNANCY. EX Following Rao and Nadlers' report of decrease arginine activity in the blood of patients with cystic fibrosis we have been trying to apply their findings to the prenatal diagnosis of cystic fibrosis. We were therefore most interested in the reports on the use of 4-methylumbelliferyl-rho-guanidinobenzoate (MUGB) for assaying arginine esterase activity in amniotic fluid. Our results with MUGB are similar to those of Brock and Haywood but are substantially higher than those of Walsh and Nedler. A sample of amniotic fluid that had been incubated with MUGB and then assayed with pro-phe-arg-4-methylcoumarinyl-7-amide (PPA-MCA) showed less than 20% of the activity of a similarly treated control sample that had not been incubated with MUGB. The activity of arginine esterase towards PPA-MCA both in amniotic fluid and in cultures amniotic fluid cells is much greater than towards CBZ-Arg-AMC. These levels of activity are high enough to warrant further investigation for use in an antenatal diagnosis. RF 005 RAO GJS PEDIATR RES 9 739 975 006 WALSH-PLATT M LANCET 1 622 979 007 BROCK DJH LANCET 1 1245 979 008 JAMESON GW BIOCHEM J 131 107 973 009 DANN LG LANCET 2 405 978 010 BLAU K J INHER METAB DIS 1 85 978 CT 1 NADLER HL PEDIATRICS 66 690 980 2 WALSH MMJ AM J OBSTET GYNECOL 137 978 980 3 NADLER HL AM J OBSTET GYNECOL 141 885 981 4 DANN LG LANCET 1 619 982 5 GREEN JR EUR J PEDIATR 139 35 982 6 SEYMOUR CA BIOESSAYS 1 38 984 PN 79161 RN 01141 AN 79243573 AU Hosli-P. Vogt-E. TI Detection of cystic fibrosis homozygotes and heterozygotes with plasma. SO Lancet. 1979 Sep 15. 2(8142). P 543-5. MJ ACID-PHOSPHATASE: bl. CYSTIC-FIBROSIS: di. MANNOSIDASES: bl. MN CYSTIC-FIBROSIS: en, fg. HEAT. HETEROZYGOTE-DETECTION. HOMOZYGOTE. HUMAN. AB Evidence is accumulating which indicates that the basic defect in cystic fibrosis interferes with the formation of recognition markers on hydrolytic enzymes. As a result these hydrolases leak into the extracellular space and some of them display abnormal thermolabilities. By heat inactivation of plasma alpha-mannosidase at 41 degrees C and plasma phosphatase at 36.5 degrees C, both enzymes being assayed at pH 5.4, it is possible to differentiate normal people from cystic fibrosis carriers and patients. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 STEINBERG AG AM J HUM GENET 12 416 960 003 DI SANTAGNESE PA N ENGL J MED 295 481 976 004 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 005 GIBSON LE PEDIATRICS 23 545 959 006 SCHWARZ V ARCH DIS CHILD 43 695 968 007 STEPHAN U PEDIATRICS 55 35 975 008 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 009 PAPP Z CLIN GENET 11 431 977 010 BRESLOW JL SCIENCE 201 180 978 011 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 012 HOSLI P PROC INT CF CONG 7TH 278 976 013 HOSLI P MONOGR PAEDIATR 10 90 979 014 NEUFELD EF J SUPRAMOL STRUCT 6 95 977 015 KAPLAN A PROC NAT ACAD SCI USA 74 2026 977 016 PAZUR JH BIOCHEM BIOPHYS RES COMMUN 40 110 970 017 HIRANI S CLIN CHIM ACTA 81 135 977 018 KRESS BC BIOCHEM BIOPHYS RES COMMUN 81 756 978 CT 1 KOLLBERG H ACTA PAEDIATR SCAND SUPPL 301 1982 15 982 2 CAREY WF MED J AUST 2 528 983 3 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 4 GUEANT JL CLIN CHIM ACTA 143 217 984 5 OGLESBEE LH CLIN CHIM ACTA 143 135 984 6 PERINI JM BULL EUR PHYSIOPATH RESP 21 569 985 7 MORSE ML LANCET 1 625 986 8 KUZEMKO JA J ROY SOC MED 79 2 986 PN 79162 RN 01142 AN 80010695 AU Lam-L-F. Shapiro-B-L. TI Differential incorporation of 3H-thymidine into DNA in cultured skin fibroblasts derived from patients with cystic fibrosis and controls. SO Life-Sci. 1979 Jun 25. 24(26). P 2483-9. MJ CYSTIC-FIBROSIS: me. DNA: me. MN ADOLESCENCE. ADULT. CELL-DIVISION. CHILD. COMPARATIVE-STUDY. FEMALE. FIBROBLASTS: me. HUMAN. MALE. THYMIDINE: me. TIME-FACTORS. AB Tritiated thymidine (3H-TdR) incorporation into DNA of the fibroblasts derived from subjects with cystic fibrosis (CF) and their controls was studied with scintillation counting and autoradiography. 3H-TdR incorporation at 24 hours postseeding was significantly less (p < 0.005) in CF strains in comparison with cells from controls. The percentage of labeled fibroblasts was not significantly difference between the two strains (p > 0.1). The cell cycle time and the duration of each phase were studied by a mitotic selection and scintillation counting technique. There was no difference in cell cycle time between CF and control fibroblasts, however, the duration of the synthetic phase was significantly (p < 0.005) longer in CF subjects. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 DANKS DM ANN HUM GENET 28 323 965 004 NADLER HL IN: STANBURY JB 1683 978 005 SHAPIRO BL CLIN CHIM ACTA 82 125 978 007 BARRANCO SC CELL TISSUE KINET 10 335 977 008 BARRANCO SC J CELL PHYSIOL 88 33 976 009 BOLTON WE AM J HUM GENET 27 394 975 010 SHAPIRO BL GAP CONF REP TISS CULT APPR 977 011 SHAPIRO BL SCIENCE 203 1251 979 CT 1 SHAPIRO BL AM J HUM GENET 34 846 982 2 SHAPIRO BL SCIENCE 216 417 982 3 AVIOLI LV AM J NEPHROL 6 151 986 PN 79163 RN 01143 AN 79220419 AU Sordelli-D-O. Cassino-R-J. Pivetta-O-H. TI Animal model for cystic fibrosis: pulmonary clearance of Staphylococcus aureus in mice treated with reserpine. SO Life-Sci. 1979 May 21. 24(21). P 2003-10. MJ CYSTIC-FIBROSIS: pp. LUNG: mi. RESERPINE: pd. STAPHYLOCOCCUS-AUREUS. MN ANIMAL. DISEASE-MODELS-ANIMAL. FEMALE. LUNG: de. MALE. MICE. MICE-INBRED-BALB-C. AB Since it has been demonstrated that chronic administration of reserpine to rats produces CF-like alterations, we have studied the lung resistance to bacterial infections in animals treated with reserpine. Mice of the BALB/c inbred strain were injected subcutaneously with different doses of reserpine for 7 days; after, they were submitted to an infective aerosol containing Staphylococcus aureus in a nebulization chamber. With each pair of exposed animals, an individual value of the uncleared bacteria ratio (UBR) at 4 hours was obtained. Reserpinized animals showed a significant increase in UBR values when compared to control mice (p < 0.05 and p < 0.01 depending on the doses), suggesting an impairment of the lung antibacterial defenses. Although pharmacological doses of reserpine produce catecholamine depletion, we can not conclude that this action upon the nervous system is the only cause of the CF-like response we detected. In conclusion, the observed UBR decrease lends support to the concept of a reserpine- induced CF animal model. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 WOOD RE AM REV RESPIR DIS 113 833 976 003 OPPENHEIMER EH COMPAR PATHOL BULL 111 3 971 004 MARTINEZ JR PEDIATR RES 9 463 975 005 PIVETTA OH PEDIATR RES 11 1133 977 006 THOMPSON FE PEDIATR RES 10 632 976 007 SNEDECOR GW STATISTICAL METHODS 967 008 FINNEY DJ PROBIT ANALYSIS 971 009 TAYLOR PW JR J PHARMACOL EXP THER 156 483 967 010 GREEN GM J CLIN INVEST 43 769 964 011 LEE JA FED PROC 35 262 976 012 STITZEL RE PHARMACOL REV 28 179 977 013 MARTINEZ JR PROC WORKSHOP ON MODEL SYS CF 978 014 MENOM AL J PHARM PHARMACOL 28 827 976 015 GREEN GM BR J EXP PATHOL 46 360 965 CT 1 HAZLETT D PEDIATR RES 20 1236 986 PN 79164 RN 01144 AN 80032233 AU Shier-W-T. TI Increased resistance to influenza as a possible source of heterozygote advantage in cystic fibrosis. SO Med-Hypotheses. 1979 Jun. 5(6). P 661-7. MJ CYSTIC-FIBROSIS: fg. HETEROZYGOTE. ORTHOMYXOVIRUS-INFECTIONS: im. PARAMYXOVIRUS-INFECTIONS: im. MN HUMAN. IMMUNITY-NATURAL. INFLUENZA: im. MEMBRANE-PROTEINS: an. SIALIC-ACIDS: an, me. SIALOGLYCOPROTEINS: an. AB Cystic fibrosis is the most common lethal or semi-lethal genetic disease in Caucasians of Central European origin, among whom it is inherited as an autosomal recessive trait at a frequency approximately 10 times that expected from recurrent mutation alone. A decreased sialic acid content has been observed in cell surface glycoproteins on cystic fibrosis fibroblasts and in numerous soluble glycoprotein preparations from cystic fibrosis homozygotes. Sialic acid residues on cell surface glycoconjugates play an essential role in the binding and infectivity of myxoviruses and paramyxoviruses, including those causing pandemic influenza. It is suggested that increased resistance to these viruses conferred by similar but quantitatively smaller alterations in sialoglycoconjugate structure in cystic fibrosis heterozygotes may have provided a selective advantage to maintain the high frequency of the cystic fibrosis gene in Caucasian populations. RF 001 DI SANTAGNESE PA N ENGL J MED 295 481 976 002 DI SANTAGNESE PA N ENGL J MED 277 1287 967 003 CONNEALLY PM TEX REP BIOL MED 31 639 973 004 WRIGHT SW AM J HUM GENET 20 157 968 005 HIRSCHHORN K IN: MANGOS JA 275 976 006 ALLISON AC BR MED J 1 290 954 007 HALDANE JBS HEREDITAS SUPPL 267 949 008 STEINBERG AG AM J HUM GENET 12 416 960 009 GAIRDNER D LANCET 1 279 975 010 CRAWFURD MDA HEREDITY 29 126 972 011 STUART AB LANCET 2 1521 974 012 DODGE JA LANCET 1 572 975 013 CRAWFURD MDA LANCET 1 167 975 014 ANDERSON CM MOD PROBL PEDIATR 10 381 967 015 DISCHE Z PEDIATRICS 24 74 959 016 ALHADEFF JA CLIN GENET 14 189 978 017 ROELFS RE AM J DIS CHILD 113 419 967 018 MANDEL ID AM J DIS CHILD 113 431 967 019 PEARSON RD PEDIATR RES 11 462 977 020 LAMB D J PATHOL 98 213 969 021 REID L IN: PORTER R 32 45 968 022 POTTER JL ANN NY ACAD SCI 106 692 963 023 ALHADEFF JA CLIN GENET 13 417 978 024 SCANLIN TF JR PEDIATR RES ABST 549 11 463 977 025 SPIRO RG ADV PROT CHEM 27 349 973 026 GRIMES WJ BIOCHEMISTRY 12 990 973 027 LURIA SE GENERAL VIROLOGY 307 967 028 HIRST GK J EXP MED 78 99 943 029 GOTTSCHALK A YALE J BIOL MED 26 352 954 030 LEICHTENSTERN O INFLUENZE UND DENGUE 896 031 STUART-HARRIS CH INFLUENZA THE VIRUSES AND DIS 976 CT 1 BURNET M PERSPECT BIOL MED 24 511 981 PN 79165 RN 01145 AN 79220977 AU Danes-B-S. Sutanto-E. TI Pathogenesis of cystic fibrosis: possible importance of alteration in epithelial surface topography. SO Med-Hypotheses. 1979 Feb. 5(2). P 289-95. MJ CYSTIC-FIBROSIS: et. LUNG: ul. MN CELL-MEMBRANE: ul. EPITHELIUM: ul. HUMAN. MICROSCOPY-ELECTRON-SCANNING. MUCOUS-MEMBRANE: ph. MUCUS: ph. AB As the pathogenesis in cystic fibrosis (CF) has been considered to lie in a disturbance in the behavior of secretions, particularly mucins, from exocrine glands, it has been proposed that an alteration in surface topography (form, number or distribution of microvilli or blebs) of epithelial cells lining ducts could impair distribution leading to retention of mucus leading to the clinical phenotype. Scanning electronmicroscopy of human epithelial suspension cultures now offers an opportunity to evaluate the role of plasmalemma topography in CF. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 ANDERSEN DH ANN NY ACAD SCI 93 500 962 003 DI SANTAGNESE PA ANN NY ACAD SCI 93 555 962 004 DANES BS BIRTH DEF ORIG ART SER 8 114 972 006 FELIX JS AM J HUM GENET 29 41A 977 007 ANDREWS PM AM J ANAT 139 399 974 008 ANDREWS PM J CELL BIOL 67 11A 975 009 MISHIMA Y ACTA DERM VENEREOL SUPPL 73 53 83 973 010 DAWBER RPR BR J DERMATOL 86 272 972 011 ORFANOS CE ARCH DERMATOL 107 38 973 012 STEINRUD J DAN MED BULL 21 251 974 013 DANKS DM ANN HUM GENET 28 323 965 014 FARBER S J MICH MED SOC 44 587 945 015 JOHANSEN PG LANCET 1 455 968 016 GIBSON LE LANCET 2 189 970 017 TILLMAN B CELL TISSUE RES 185 279 977 018 BAUR PS TEX REP BIOL MED 34 113 976 019 PORTER KR J CELL BIOL 57 815 973 020 ANDREWS PM AM J ANAT 140 81 974 021 SYLVEN B ACTA HISTOCHEM SUPPL 1 79 958 022 DANES BS J EXP MED 123 1 966 023 DANES BS BIOCHEM BIOPHYS RES COMMUN 36 919 969 024 SPOCK A PEDIATR RES 1 173 967 025 DANES BS J EXP MED 136 1313 972 CT 1 THEOHARIDES TC LIFE SCI 27 703 980 2 MICHALSEN H PATHOL RES PRACT 178 261 984 PN 79166 RN 01146 AN 80099211 AU Hahnel-R. Hosli-P. TI Alkaline phosphatase induction in cystic fibrosis fibroblasts [letter]. SO Med-J-Aust. 1979 Nov 3. 2(9). P 490. MJ ALKALINE-PHOSPHATASE: du. CYSTIC-FIBROSIS: di. MN ALKALINE-PHOSPHATASE: me. FIBROBLASTS: en. HUMAN. EX Lysosomal acid hydrolases and alkaline phosphatase cooperate in the intracellular digestion of natural polymers. A stimulus for the induction of these enzymes is the retention of any kind of polymer inside the cell. This stimulus is long-lasting and strong if polymers are intracellularly retained because of a lack of digestive enzymes. In previous papers it has been demonstrated that the alkaline phosphatase (AIP) induction with Tamm Horsfall glycoprotein discriminates between fibroblasts of CF homozygotes on the one hand and normals and CF carriers on the other hand, and a hypothesis has been proposed to explain these observations. As predicted, more efficient inducing mixtures have since been developed; these have been used in the present study. The first writer familiarized himself with ultramicrotechniques and then carried out double blind studies on 20 fibroblast cultures from French patients. These findings demonstrate the reproducibility of the AIP induction and of the methods used, and they particularly underline the importance of employing the present ultramicrotechniques for a successful AIP induction. It is likely that amniotic fluid cells behave like skin fibroblasts in this test; if this proves to be so, then the induction of alkaline phosphatase should provide a useful technique for the prenatal diagnosis of cystic fibrosis. RF 001 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 002 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 003 HOSLI P MONOGR PAEDIATR 10 90 979 004 HOSLI P CLIN CHEM 23 1476 977 PN 79167 RN 01147 AN 79199129 AU Phelan-P-D. Allan-J-L. Landau-L-I. Barnes-G-L. TI Improved survival of patients with cystic fibrosis. SO Med-J-Aust. 1979 Apr 7. 1(7). P 261-3. MJ CYSTIC-FIBROSIS: mo. MN ADOLESCENCE. AGE-FACTORS. ANTIBIOTICS: tu. CHILD. CYSTIC-FIBROSIS: dt, th. HUMAN. AB The survival of 320 patients, who were born in and after 1958 with cystic fibrosis and managed by the Departments of Thoracic Medicine and Gastroenterology, Royal Children's Hospital, Melbourne, is reviewed. Eighty per cent of patients survived to 11 years of age, and 64% to 18 years. Of patients managed between 1973 and 1977, 91% survived to 12 years of age, and 80% to 17 years of age. In the same period, 79% of patients survived for 16 years after diagnosis. Forty- four per cent of the 240 patients currently being managed have no significant permanent lung disease, and only 9% have advanced disease. RF 001 ANDERSON CM MOD PROBL PEDIATR 10 344 967 002 WARWICK WJ IN: LAWSON D PROC 5TH INT CF 320 969 004 ROBINSON MJ ARCH DIS CHILD 50 962 975 005 CROFTON J RESPIRATORY DISEASES 638 975 006 GIBSON LE PEDIATRICS 23 545 959 007 ARMITAGE P STATISTICAL METHODS IN MEDICA 971 008 REILLY BJ RADIOLOGY 98 281 971 009 ORENSTEIN DM AM J DIS CHILD 131 973 977 010 WILLIAMS HE RESPIRATORY ILLNESS IN CHILDR 975 011 WARING WW ADV PEDIATR 23 401 977 012 ALLAN JL AUST PAEDIATR J 10 136 974 CT 1 ALLAN JL MED J AUST 1 600 980 2 ROBINSON MJ MED J AUST 1 580 980 3 SHEPHERD R J PEDIATR 97 351 980 4 SINCLAIR MI MED J AUST 2 283 981 5 FRYDMAN MI AUST PAEDIATR J 17 35 981 6 MILLER M AM J CLIN NUTR 36 492 982 7 WARWICK WJ ACTA PAEDIATR SCAND SUPPL 301 1982 27 982 8 SHEPHERD RW J PEDIATR GASTROENTEROL NUTR 2 439 983 9 WILMOTT RW ARCH DIS CHILD 58 835 983 10 WESLEY AW NZ MED J 96 651 983 11 ANON LANCET 1 663 984 12 WELLS AL J FOOD NUTR 41 65 984 13 HODGES P J AM DIET ASSOC 84 664 984 14 WESLEY AW NZ MED J 97 773 984 15 HOLT TL AM J CLIN NUTR 41 1061 985 16 BERKIN KE EUR J RESPIR DIS 67 103 985 17 HILL DJS MED J AUST 143 230 985 18 PEDERSEN SS ACTA PAEDIATR SCAND 75 840 986 19 LITTLEWOOD JM J ROY SOC MED 79 55 986 PN 79168 RN 01148 AN 80120196 AU Carey-W-F. Pollard-A-C. TI Alkaline phosphatase induction in cystic fibrosis fibroblasts [letter]. SO Med-J-Aust. 1979 Dec 1. 2(11). P 604-5. MJ ALKALINE-PHOSPHATASE: bi. CYSTIC-FIBROSIS: en. MN CELLS-CULTURED. ENZYME-INDUCTION. FIBROBLASTS: en. HUMAN. EX Hosli has published a hypothesis relating to the primary cause of cystic fibrosis (CF) based on his observations that the level of alkaline phosphatase (ALP) in CF-derived skin fibroblasts, but not fibroblasts derived from normal individuals, could be increased several-fold by growing the cells in the presence of the urinary glycoprotein, Tamm-Horsfall protein (THP). However, we have not been able to show, in Adelaide, any change or induction in the level of ALP activity in CF-derived skin fibroblasts when grown in the presence of locally prepared THP under what appear to be identical cell culture conditions to those used by Hosli. It thus appears that under the conditions currently employed in our laboratory, there is no diagnostic discrimination between fibroblasts of CF homozygotes and normal individuals. RF 001 HOSLI P MONOGR PAEDIATR 10 90 978 002 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 003 CAREY WF AUST J EXP BIOL MED SCI 57 225 979 004 HOSLI P CLIN CHEM 23 1476 977 005 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 006 CAREY WF PAEDIATR RES SOC AUST 979 007 CAREY WF HUMAN GENET SOC AUST 979 008 WIJCIK L BIOCHIM BIOPHYS ACTA 585 374 979 CT 1 CAREY WF MED J AUST 2 314 982 2 CAREY WF MED J AUST 2 528 983 PN 79169 RN 01149 AN 79221582 AU Weller-P-H. Bush-E. Preece-M-A. Norman-A-P. Matthew-D-J. TI The short-term effects of chest physiotherapy on lung function tests in children with cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 58-9. MJ CYSTIC-FIBROSIS: th. LUNG: pp. PHYSICAL-THERAPY. MN CHILD. CYSTIC-FIBROSIS: pp. HUMAN. RESPIRATORY-FUNCTION-TESTS. THORAX. TIME-FACTORS. AB Chest physiotherapy is an important part of the treatment program of children with cystic fibrosis, but controlled studies of its effects are few. 27 children with cystic fibrosis, 20 being chronic sputum producers and 7 non-sputum producers, were studied in hospital with serial lung function tests on 3 consecutive days. On the 1st day physiotherapy was performed twice; on the 2nd day no physiotherapy was performed; on the 3rd day the two periods of physiotherapy were preceded by an inhaled bronchodilator (Rimiterol). Lung function tests included peak flow, forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1.0) and residual volume to total lung capacity ratio, and were measured at the beginning of each day and at similar intervals throughout the 3 days. Sputum volume and cough frequency were noted on day 1 and day 3. Physiotherapy resulted in significant improvement of peak flow in the sputum producers. However, the FVC and FEV1.0, while improving after physiotherapy, also improved on the control day when no physiotherapy was performed. This suggests that physiotherapy may be effective in removing sputum from central large airways with resultant improvement in peak flow, but has little added effect on the more peripheral airways, whose airflow improves through the day with normal activity. No significant effect was shown in the non- sputum producers although there was a trend towards improvement in peak flow. A bronchodilator before physiotherapy may be beneficial in those patients who demonstrate significant reduction in airflow obstruction at formal testing of bronchodilator response, but may lessen the beneficial effects of physiotherapy in those without a positive response. The study illustrates the importance of examining difference methods of chest physiotherapy, to find ways of improving sputum clearance. CT 1 GOTZ M EUR J RESPIR DIS 61 122 980 PN 79170 RN 01150 AN 79221583 AU Kerrebijn-K-F. Veentjer-M-A. Water-E-B. TI Effect of four physiotherapy schedules on pulmonary function in children with cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 60. MJ CYSTIC-FIBROSIS: th. LUNG: pp. PHYSICAL-THERAPY. MN CHILD. CYSTIC-FIBROSIS: pp. HUMAN. RESPIRATORY-FUNCTION-TESTS. AB In 17 children (group A) with cystic fibrosis (CF) the effect of the following treatment schedules on pulmonary function has been studied in random order on 4 consecutive days: (1) clapping and postural drainage (CP); (2) CP after inhaling an aerosol of 5 ml 20% N-acetylcysteine by a Monaghan ultrasonic device; (3) as in 2 but with the addition of 0.5 mg isoprenaline to the aerosol; (4) as in 3 but with an IPPB device added to the nebulizer. Treatment was started at 9.30 a.m. and lasted for about 1 h. Pulmonary function was measured prior to and 30 min after treatment (9.00 and 11.00 a.m., respectively). In 8 children (group B) with CF (severity comparable to the previous group) pulmonary function was measured at 9.00 and 11.00 a.m. but without physiotherapy in between. The following pulmonary function measurements have been included in the evaluation of the results: VC, FVC, FEV1, TLC (body box), TLC (helium dilution), TLCBb-TLCHe, RVBb, RVHe, maximal expiratory flows at 60% TLBBb, 25% FVC, maximal midexpiratory flow, airway conductance. The technique of the determinations was carefully controlled and initial values were reproducible in most children. In group B, mean values of pulmonary function were equal at 9.00 and 11.00 a.m. In group A none of the treatment schedules caused a significant improvement of mean pulmonary function values. In some of the children, however, pulmonary function improved, but in others it deteriorated. The significance of these findings was discussed. PN 79171 RN 01151 AN 79221584 AU Kraemer-R. Rudeberg-A. Rossi-E. TI Relationship between clinical conditions, chest radiographic findings and respiratory function tests in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 61-5. MJ CYSTIC-FIBROSIS: di. MN CHILD. CYSTIC-FIBROSIS: pp, ra. HUMAN. LUNG: pp. RESPIRATORY-FUNCTION-TESTS. THORACIC-RADIOGRAPHY. EX We explored 44 children suffering from cystic fibrosis with predominant pulmonary involvement and followed in particular the course of illness of 30 of these patients during an average period of 4 years. We were impressed by the enormous variability of the pulmonary symptomatology in these children. The question arose whether this clinical impression is supported by the finding of the thorax radiograph and the results of the lung functions tests and the blood gas measures at rest and on exercise. From the clinical point of view, there is a possibility to make a classification of severity in pulmonary aspects of this disease. Because of the large ranges of the score values a clear distinction of the severity could only be possible if large groups of patients were to be compared. For the interindividual comparison the cumulative evaluation of a clinical score, the chest radiograph and the lung function tests are indispensable. Only the continuous follow-up of these examinations allows one to notice, in time, a progressive lung involvement with its degenerative changes. RF 001 KRAEMER R PROC EWGCF 7TH ANNU MTG 31 977 002 KRAEMER R HELV PAEDIATR ACTA 32 107 977 003 SHWACHMAN H AM J DIS CHILD 96 6 958 004 HUANG NN AM J DIS CHILD 120 289 970 005 GEORGE L ARCH DIS CHILD 46 139 971 006 ROBINSON MJ ARCH DIS CHILD 50 962 975 007 KRAEMER R TAG SCHWEIZ GES PNEUMOLOGIE L 978 008 KRAEMER R PADIAT FORTBILDK PRAXIS 48 7 979 009 CHRISPIN AR PEDIATR RADIOL 2 101 974 010 SCHERRER M SCHWEIZ MED WOCHENSCHR 107 1072 977 CT 1 KRAEMER R HELV PAEDIATR ACTA 34 417 979 2 KRAEMER R METH INF MED 19 50 980 PN 79172 RN 01152 AN 79221581 AU Flower-K-A. Mann-N-M. Eden-R-I. TI A new percussion aid for cystic fibrosis patients. SO Monogr-Paediatr. 1979. 10. P 54-7. MJ CYSTIC-FIBROSIS: th. PERCUSSION. MN ADOLESCENCE. ADULT. HUMAN. PHYSICAL-THERAPY. SPUTUM. THORAX. EX This paper is a report on the first part of a comprehensive research programme. The aim is the development of a mechanical aid which will enable patients with cystic fibrosis to give themselves satisfactory physiotherapy. The percussor, which can be used by patients, parents or relatives, is portable and lightweight. The domiciliary trial showed that effective percussion could be self-administered to the front of the chest and to a lesser extent to the sides of the chest. The percussor was found to be very acceptable to adolescent patients and especially girls with early breast development. Further research work has now been completed and as a result a system has been developed which enables patients to treat themselves with satisfactory chest percussion in the five preferred positions of postural drainage. RF 001 HODSON ME BR MED J 1 971 978 002 FLOWER KA PROC EWGCF 8TH ANNU MTG 978 003 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 973 PN 79173 RN 01153 AN 79221580 AU Dab-I. Alexander-F. TI The mechanism of autogenic drainage studied with flow volume curves. SO Monogr-Paediatr. 1979. 10. P 50-3. MJ CYSTIC-FIBROSIS: co. LUNG-DISEASES-OBSTRUCTIVE: th. SPUTUM. MN BRONCHI. CHILD. CYSTIC-FIBROSIS: th. HUMAN. LUNG-DISEASES-OBSTRUCTIVE: et. POSTURE. EX In 1977, we demonstrated the efficiency of autogenic drainage with two bronchorrheic children, who needed bronchography for diagnostic purposes. This demonstration was made possible thanks to a radiocinematographic study after bronchography. The examination also showed the inefficacy of coughing. The object of the present study is to analyze the physiologic mechanism of autogenic drainage. For children older than five, this method is very simple and efficient and as the effort or onset is not excessive and as expiration does not need to be complete, autogenic drainage is not exhausting and can be performed for a long time, which is indispensable since the progression of secretions is slow. RF 001 DAB I IN: BARAN D 185 977 002 DAB I PROC EWGCF 7TH ANNU MTG 977 CT 1 EICHLER I DTSCH MED WSCHR 111 896 986 PN 79174 RN 01154 AN 79221579 AU Bakken-A-F. Motzfeldt-C. TI Intestinal lactase deficiency in newborns with cystic fibrosis--dietary consequences. SO Monogr-Paediatr. 1979. 10. P 5-7. MJ CYSTIC-FIBROSIS: co. INTESTINES: en. LACTOSE-INTOLERANCE: dh. MN BETA-GALACTOSIDASES: me. CYSTIC-FIBROSIS: dh, en. HUMAN. INFANT. INFANT-NEWBORN. LACTOSE-INTOLERANCE: et. EX The problem of proper diet to children and infants with cystic fibrosis is continuously discussed. This paper deals with the diet for CF infants younger than one year. The basic view is that the diet should be as close to the 'normal baby' diet as possible, partly to ensure normal growth and development, and partly to avoid extra work for the families with CF infants. Infants suffering from CF were given lactose-free diet until they reached the age of one year. The weight increased more rapidly than in CF patients given the traditional CF diet with low fat and high protein. Furthermore, it was not necessary to give increased amounts of calories to the infants given lactose-free diet, nor did they receive additional fat-soluble vitamins. PN 79175 RN 01155 AN 79221578 AU Konradova-V. Vavrova-V. Tomanek-A. Houstek-J. TI Ultrastructure of the bronchial epithelium in children with cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 46-9. MJ BRONCHI: ul. CYSTIC-FIBROSIS: pa. MN BRONCHI: pa. CHILD. CHILD-PRESCHOOL. EPITHELIUM: pa, ul. FEMALE. HUMAN. MALE. EX The ultrastructure of the bronchial epithelium in 3 children with cystic fibrosis was described. In 2 of them a developed squamous metaplasia with many signs of pathological alteration of the epithelial cells was observed. In the third patient severely injured pseudostratified epithelium with remnants of ciliary border is preserved. RF 001 KONRADOVA V FOLIA MORPHOL (PRAGUE) 24 296 976 002 KONRADOVA V Z ERKR ATMUNGSORGANE 270 977 CT 1 ROBERTSON B EUR J RESPIR DIS 63 496 982 2 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 PN 79176 RN 01156 AN 79221585 AU di-SantAgnese-P-A. Davis-P-B. TI Recent research in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 66-76. MJ CYSTIC-FIBROSIS. RESEARCH. MN ANIMAL. AUTONOMIC-NERVOUS-SYSTEM. BONE-MARROW. CYSTIC-FIBROSIS: et, fg, pp. FEMALE. HUMAN. INFANT. INFANT-NEWBORN. MICE. ANTHROPOIDEA. PREGNANCY. RABBITS. TISSUE-CULTURE. EX This review emphasizes some of the positive accomplishments of cystic fibrosis research, some of the puzzles and some of the recent developments that have promise. One of the major unresolved questions is the maintenance of this lethal gene at such a high frequency, perhaps because of a heterozygote advantage in the past, no longer operative at present. Nothing is known of the chromosomal localization of the trait; except that chromosomes are morphologically normal, that it is autosomal, not sex linked and that there is no correlation with blood groups or major HLA loci. One of the important achievements of the past many years is that we understand relatively well the pathogenesis of most clinical manifestations of CF. The as yet unknown inherited basic defect or metabolic error of CF gives rise to two main anomalies: the electrolyte defect in exocrine sweat glands and the 'mucous secretion' abnormality. While the results of tissue culture studies often have been conflicting and disparate, it does seem to be the consensus that the fibroblasts are affected by the basic metabolic error in some manner, and that they respond abnormally under different experimental circumstances. Virtually all investigators have found some anomalies in biologic fluids of CF, although using different approaches and methods. An abnormality in the function of the autonomic nervous system, which is so closely associated with the exocrine glands, has always been an attractive explanation for the generalized exocrinopathy of CF. The search for a genetic animal model for CF has led to interesting findings. RF 001 BARTMAN J J PEDIATR 76 430 970 002 DANES BS J EXP MED 129 775 969 003 DAVIS PB PEDIATR RES 12 703 978 004 DI SANTAGNESE PA N ENGL J MED 295 481 976 005 DI SANTAGNESE PA N ENGL J MED 295 481 976 006 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 007 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 009 GUGLER EC J PEDIATR 71 585 967 010 HOSLI P MONOGR PAEDIATR 10 90 979 011 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 012 KAISER D LIFE SCI 15 803 974 013 MANGOS JA PEDIATR RES 2 378 968 014 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 015 MARTINEZ JR PEDIATR RES 9 463 975 016 SHAPIRA E J BIOL CHEM 252 7923 977 018 ANON PROC WORKSHOP ON MODEL SYS CF 978 019 WRIGHT SW AM J HUM GENET 20 157 968 020 HOSLI P ACTA PAEDIATR SCAND 67 617 978 CT 1 DAVIS PB PEDIATR RES 14 83 980 2 HULTBERG B CLIN CHIM ACTA 112 167 981 3 MORIARTY CM J MED 13 257 982 4 KAMOUN P BIOCHEM MED 31 104 984 5 NEAL JL EVOLUTIONARY THEORY 7 153 985 6 RUPP GM MED HYPOTHESES 20 245 986 PN 79177 RN 01157 AN 79221586 AU Roscher-A. Wiesmann-U-N. Hadorn-B. TI Relevance of second messengers in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 77-83. MJ ADENOSINE-CYCLIC-MONOPHOSPHATE: me. CYSTIC-FIBROSIS: me. ISOPROTERENOL: pd. RECEPTORS-ADRENERGIC. RECEPTORS-ADRENERGIC-BETA. MN ADRENERGIC-BETA-RECEPTOR-BLOCKADERS: pd. CYSTIC-FIBROSIS: pp. FIBROBLASTS: me. HUMAN. NEUROREGULATORS: ph. PROSTAGLANDINS-E: ph. EX Water, electrolyte and macromolecular secretion, which is affected in cystic fibrosis, is regulated by neurotransmitters of the autonomic nervous system stimulating the cells by binding to alpha-adrenergic, beta-adrenergic and cholinergic receptors. Therefore, a genetic disturbance in cyclic 3'5'-adenosine monophosphate (c-AMP) response could eventually explain some of the manifold expressions of labile biochemical changes observed in CF, specially in tissue culture. We further characterized beta-receptors in cultured fibroblasts of CF patients and normal controls and investigated isoproterenol and prostaglandin E1-mediated c-AMP response using defined culture conditions. No significant difference was found between the two phenotypes on basal c-AMP values. With the exception of one cell line, CF strains had 25 to 180% increased c-AMP content. This difference is highly significant (p < 0.0025). This statistically significant difference in response can neither be attributed to differences in dose- or time-response curves nor to differential c-AMP escape into culture medium. Since CF cells synthesize more c-AMP than controls only in response to isoproterenol but not to prostaglandin E1, the alteration which might be responsible for the observed phenomenon is probably located proximal of the adenylate cyclase at the cells border between intra- and extracellular space. RF 001 BERRIDGE MJ ADV CYCLIC NUCLEO RES 6 1 975 002 RAMWELL PW IN: RABIN BR 207 971 003 BUTCHER FR BIOCHIM BIOPHYS ACTA 392 82 975 004 SCHRAMM M J CYCLIC NUCLEOTIDES RES 1 181 975 005 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 006$ SHAPIRO BL CF CONF REP 2 978 007 RECHLER MM J CLIN ENDOCRINOL METAB 39 512 974 008 MANGANIELLO VC BIOCHIM BIOPHYS ACTA 362 509 974 009 GILMAN AG PROC NAT ACAD SCI USA 67 305 970 010 KELLY LA J BIOL CHEM 249 3098 974 011 DUNLOP D BR J PHARMAC CHEMOTHER 32 201 968 012 BARBERO GJ IN: DI SANTAGNESE PA 208 964 013 LANDS AM NATURE 214 597 967 014 FRANKLIN TJ MOL PHARMACOL 11 485 975 015 HO RJ J BIOL CHEM 246 6822 971 016 NEWCOMBE DS PROC NAT ACAD SCI USA 72 3124 975 CT 1 BECKER M EUR J PEDIATR 134 217 980 PN 79178 RN 01158 AN 79221587 AU Aggett-P-J. Thorn-J-M. Delves-H-T. Harries-J-T. Clayton-B-E. TI Trace element malabsorption in exocrine pancreatic insufficiency. SO Monogr-Paediatr. 1979. 10. P 8-11. MJ CYSTIC-FIBROSIS: me. IRON: me. MALABSORPTION-SYNDROMES: et. MANGANESE: me. PANCREATIC-DISEASES: me. ZINC: me. MN CHILD. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: co. FEMALE. HUMAN. MALE. EX This paper presents preliminary results from metabolic balance studies of iron, zinc and manganese absorption and retention in children with cystic fibrosis and Shwachman's syndrome. Malabsorption of zinc, iron and manganese in exocrine pancreatic insufficiency has been demonstrated by these early studies. A possible relationship between this and the associated features of CF has been briefly discussed, and indicates areas for further research. If such relationships are established then some form of trace element supplementation should be considered. RF 001 ALEXANDER FW Q J MED 43 89 974 002 BUTTERWORTH J CLIN CHIM ACTA 56 159 974 003 CAPLAN AH J PEDIATR 73 540 968 004 DAVIS AE LANCET 2 6 962 005 DODGE JA BR MED J 1 411 978 006 EVANS GW IN: KIRCHGESSNER M 98 978 007 HALSTED JA LANCET 1 322 970 008 RAO GJS PEDIATR RES 11 981 977 009 RHEINHOLD JG CLIN CHEM 21 476 975 010 SINGER L CLIN BIOCHEM 7 146 974 011 UNDERWOOD EJ TRACE ELEMENTS IN HUMAN AND A 977 CT 1 CLAYTON BE ADV CLIN CHEM 21 147 980 2 AGGETT PJ PROC NUTR SOC 39 241 980 3 SOLOMONS NW AM J CLIN NUTR 34 462 981 4 HURRY VJ BIOL TRACE ELEMENT RES 4 157 982 5 DELVES HT ANN CLIN BIOCHEM 19 302 982 6 SOLOMONS NW AM J CLIN NUTR 35 1048 982 7 VANCAILLIEBERTRAND M ACTA PAEDIATR SCAND 71 203 982 8 SOLOMONS NW J AM DIET ASSOC 80 115 982 9 VANDERHOOF JA DIG DIS SCI 28 300 983 10 RUSSELL RM ANN INTERN MED 99 227 983 11 PATRICK J CRC CRIT REV CLIN LAB SCI 20 95 984 12 MCCLAIN CJ J AM COLL NUTR 4 49 985 13 DELVES HT CLIN ENDOCRINOL METAB 14 725 985 PN 79179 RN 01159 AN 79221588 AU Hamdi-I. Bloom-A-L. Goodchild-M-C. Dodge-J-A. TI Prostaglandins and cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 84-9. MJ CYSTIC-FIBROSIS: me. PROSTAGLANDINS-E: me. SWEAT: me. MN ADENOSINE-CYCLIC-MONOPHOSPHATE: bi. ADULT. CHILD. CYSTIC-FIBROSIS: bl. HUMAN. PLATELET-AGGREGATION. EX We have investigated two aspects of prostaglandin function in cystic fibrosis: the response of platelets to PGE1, and the concentrations of PGE2 and PGE-alpha in the sweat. Low concentrations of PGE1 have less effect on platelet agglutination in CF patients and heterozygotes than in normals, but no difference is observed when high PG concentrations are used. Sweat PGE2 and PGE-alpha concentrations are similar in normals, CF patients and heterozygotes, but sweat PGE2 concentrations are proportional to Na/Cl levels in CF, but not in control children or heterozygotes. RF 001 BARNES GL LANCET 2 918 975 002 BUCHWALD M AM J HUM GENET 27 22A 975 003 CHASE HP PEDIATRICS 57 441 976 004 FORSTROM L PROSTAGLANDINS 7 459 974 005 FREWIN DB AUST J EXP BIOL MED SCI 51 701 973 006 GALABERT C PROC INT CF CONG 7TH 100 976 007 ROBINSON PG PROC INT CF CONG 7TH 107 976 008 ROSENLUND ML PEDIATRICS 59 428 977 009 SAMUELS CE LANCET 2 607 975 010 YASSA JG PROC INT CF CONG 7TH 323 976 CT 1 ANDERSON CM J PEDIATR GASTROENTEROL NUTR 3 15 984 2 DAVIS PB J LAB CLIN MED 104 203 984 3 KHULLAR AK CLIN SCI 68 433 985 4 STEAD RJ PROSTAGLANDINS LEUKOTR MED 26 91 987 PN 79180 RN 01160 AN 79221589 AU Hosli-P. Kollberg-H. Vogt-E. TI Operational definition of cystic fibrosis. I. SO Monogr-Paediatr. 1979. 10. P 90-5. MJ CYSTIC-FIBROSIS: di. MN ANIMAL. CYSTIC-FIBROSIS: en, fg, me. FIBROBLASTS: me. HETEROZYGOTE. HOMOZYGOTE. HUMAN. HYDROLASES: me. LYSOSOMES: en. EX Recently, one of us suggested a new hypothesis concerning the 'basic defect' of cystic fibrosis. The purpose of the present paper is to review this model and to critically discuss its eventual diagnostic applications. With extensive studies, we collected experimental data which contribute five major arguments in favour of the proposition that CF may be an enzyme recognition site mutant. With fibroblast cultures the three genotypes can be separated in the following way: CF homozygote, leaky for alkaline phosphatase (A1P) and lysosomal hydrolases/A1P is Tamm-Horsfall glycoprotein (THP) inducible; CF heterozygote, leaky for A1P and lysosomal hydrolases/A1P is not THP inducible; normal control, not leaky for A1P and lysosomal enzymes/A1P is not THP inducible. With the exception of a second double-blind study, the THP induction as well as the leakage analysis seem to be statistically unequivocal: they strongly support our hypothesis concerning the basic defect in CF. RF 001 HOSLI P PROC INT CF CONG 7TH 278 976 002 HOSLI P BIOCHEM BIOPHYS RES COMMUN 79 741 977 003 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 004 HOSLI P HUM HERED 27 185 977 005 WARD JB JR TEX REP BIOL MED 34 11 976 006 RENNERT OM GAP CONF REP TISS CULT APPR 1 977 007 HOSLI P IN: MOTULSKY AG 226 974 010 HOSLI P ADV EXP MED BIOL 68 1 976 011 HOSLI P CLIN CHEM 23 1476 977 012 BOAT TF IN: MANGOS JA 165 976 013 HOSLI P HUM GENET 41 169 978 015 HICKMAN S BIOCHEM BIOPHYS RES COMMUN 57 55 974 016 WIESMANN UN N ENGL J MED 284 109 971 017 SCANLIN TF JR PROC INT CF CONG 7TH 44 976 020 MANGOS JA SCIENCE 158 135 967 021 PALADE GE INT SYMP ON LYSOSOMES ABST 36 972 022 PALADE GE CF Q ANNOTATED REFERENCES 14 29 975 023 HOSLI P TISSUE CULTIVATION ON PLASTIC 972 CT 1 HOSLI P LANCET 2 543 979 2 DISANTAGNESE PA MONOGR PAEDIATR 10 66 979 3 HOSLI P FEBS LETTERS 104 271 979 4 TEGNER H ACTA PAEDIATR SCAND 70 629 981 5 MALER T J BIOL CHEM 256 1420 981 6 CAREY WF MED J AUST 2 528 983 7 HEELEY AF CLIN CHEM 29 2011 983 8 CEDER O ACTA PAEDIATR SCAND 72 291 983 9 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 10 BUYS CHCM CLIN CHIM ACTA 136 229 984 PN 79181 RN 01161 AN 79221590 AU Casola-L. Di-Matteo-G. Mastella-G. Rutigliano-B. Romano-M. TI Alpha-glucosidase in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 96-101. MJ CYSTIC-FIBROSIS: en. GLUCOSIDASES: bl. MN ADULT. CHILD. CYSTIC-FIBROSIS: fg. HETEROZYGOTE. HOMOZYGOTE. HUMAN. EX Studies are presented on the serum level of several acid glycosidases in normal and cystic fibrosis subjects. Levels of alpha-glucosidase found in serum from CF patients were significantly higher than from normals and heterozygotes. The increase in alpha-glucosidase was directly related to the clinical gravity of the disease. Levels of all other glycosidases studied in CF patients were in the normal range. RF 001 ALHADEFF JA CLIN GENET 10 63 976 003 ANTONOWICZ I PEDIATR RES 6 803 972 004 BARTMAN J J PEDIATR 76 430 970 005 DANES BS J EXP MED 129 775 969 006 DI MATTEO G MONOGR PAEDIATR 10 19 979 007 DI SANTAGNESE PA N ENGL J MED 295 534 976 008 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 009 MATALON R BIOCHEM BIOPHYS RES COMMUN 33 954 968 010 SHWACHMAN H AM J DIS CHILD 96 615 958 CT 1 DIMATTEO G MONOGR PAEDIATR 10 19 979 2 HULTBERG B CLIN CHIM ACTA 112 167 981 PN 79182 RN 01162 AN 79221562 AU Starkey-B-J. Goodchild-M-C. TI Postprandial total serum bile acid concentrations in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 12-8. MJ BILE-ACIDS-AND-SALTS: bl. CYSTIC-FIBROSIS: bl. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. EATING. HUMAN. MIDDLE-AGE. TIME-FACTORS. EX In recent years, several workers have reported that a single 2-hour postprandial total serum bile acid (TSBA) concentration, in a variety of liver disease states, is a more sensitive index of deranged liver function than are the 'conventional' serum liver function tests. This observation seemed to us to provide a challenging situation for the assessment of TSBA concentrations in cystic fibrosis. The distribution of results for the CF patients (mean TSBA concentration 27.0 micromol/l) and for the age-matched controls (mean concentration 19.9 micromol/l) shows a wider range for the CF group. Statistical analysis, however, of these two groups shows no significant difference between them. Many factors affect both the size and distribution of the bile acid pool in CF, and a consideration of these factors may go some way towards explaining the rather unspectacular TSBA results. RF 001 BARNES S J CLIN PATHOL 28 506 975 002 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 003 DI SANTAGNESE PA PEDIATRICS 18 387 956 004 FAUSA O SCAND J GASTROENTEROL 11 537 976 005 FEIGELSON J ACTA PAEDIATR SCAND 59 539 970 006 GOODCHILD MC ARCH DIS CHILD 50 769 975 007 HARVEY IC CLIN RADIOL 28 635 977 008 HEATON KW BILE SALTS IN HEALTH AND DISE 972 009 KAPLOWITZ N JAMA 225 292 973 010 KATTWINKEL J J PEDIATR 82 234 973 011 MASHIGE F CLIN CHIM ACTA 70 79 976 012 MATHIS RK J PEDIATR 90 864 977 013 OPPENHEIMER EH J PEDIATR 86 683 975 014 ROLLER RJ GASTROENTEROLOGY 72 661 977 015 SANDBERG DH PEDIATR RES 4 262 970 016 SCHUSTER SR J PEDIATR SURG 12 201 977 017 SMALLEY CA ARCH DIS CHILD 53 477 978 018 TYSON KRT J PEDIATR SURG 3 271 968 019 WATKINS JB GASTROENTEROLOGY 67 835 974 020 WATKINS JB GASTROENTEROLOGY 73 1023 977 021 WEBER AM GUT 17 295 976 022 WEBER AM GASTROENTEROLOGY 68 1066 975 023 WEBER AM N ENGL J MED 289 1001 973 024 WEBSTER R ARCH DIS CHILD 28 343 953 CT 1 EKLUND A SCAND J CLIN LAB INVEST 40 595 980 2 STRANDVIK B SCAND J GASTROENTEROL 20 381 985 PN 79183 RN 01163 AN 79221568 AU Carswell-F. Oliver-J. Silverman-M. TI The cystic fibrosis gene, IgE and IgG4. SO Monogr-Paediatr. 1979. 10. P 144-7. MJ CYSTIC-FIBROSIS: fg. GENES. IGE: an. IGG: an. MN ADOLESCENCE. ADULT. CHILD. CYSTIC-FIBROSIS: im. FEMALE. HUMAN. MALE. EX We decided to survey a geographically defined population of cystic fibrosis patients and parents living in Avon County, England. By examining the obligate heterozygotes (the parents), we would be able to assess the influence of the CF gene without the presence of the disease. There is an increase in immediate hypersensitivity to A. fumigatus in CF patients but not their parents. This increased sensitivity is probably IgE-mediated and a secondary effect of lung damage. There is no evidence that the CF gene produces a generalised increase in skin reactivity (atopy), serum IgE or serum IgG4 in the heterozygotes. They are not therefore likely to develop asthma. RF 001 WARREN CPW CLIN ALLERGY 5 1 975 002 WARNER JO LANCET 1 990 976 003 MCFARLANE H LANCET 1 1241 976 004 MCFARLANE H CLIN ALLERGY 7 279 977 005 SHAKIB F CLIN ALLERGY 6 237 976 006 VIJAY HM INT ARCH ALLERGY APPL IMMUNOL 53 78 977 007 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 008 ALLAN JD CLIN ALLERGY 5 255 975 009 WARNER JO ARCH DIS CHILD 51 507 976 010 GODFREY RC CLIN ALLERGY 6 79 976 011 DAVIS JB CLIN ALLERGY 6 329 976 PN 79184 RN 01164 AN 79221561 AU Leupold-W. Dietzsch-H-J. TI Bronchial reactivity in children with cystic fibrosis and other recurrent or chronic bronchopulmonary diseases. SO Monogr-Paediatr. 1979. 10. P 119-22. MJ ASTHMA: et. BRONCHITIS: et. CYSTIC-FIBROSIS: co. RESPIRATORY-HYPERSENSITIVITY: et. MN CHILD. CHRONIC-DISEASE. HUMAN. RECURRENCE. EX In our efforts to standardize the evaluation of bronchial reactivity we performed investigations into the threshold dosage of acetylcholine-induced bronchospasm in patients with various recurrent and chronic bronchopulmonary diseases. A significantly decreased threshold value of acetylcholine and thus an increased bronchial reactivity could be proven in patients with cystic fibrosis in comparison with healthy children. The cause of bronchial hyperreactivity in patients with CF may be due to proteases deriving from leucocytes of the purulent sputum. Furthermore, we investigated the influence of terbutaline on the bronchial obstruction induced by acetylcholine in patients with bronchial asthma and cystic fibrosis. The groups were comparable, especially with regard to the degree of the initial bronchial obstruction. RF 001 EMPEY DW AM REV RESPIR DIS 113 131 976 002 HALUSZKA J RESPIRATION 32 217 975 003 MASTELLA G PROC EWGCF 4TH ANNU MTG 51 973 004 MCCARTHY DS IN: LAWSON D PROC 5TH INT CF 194 969 005 MCCHESNEY-YOHE R ANN ALLERGY 30 627 972 006 MELLIS CM PEDIATRICS 61 446 978 007$ OUELLETTE JJ J ALLERGY 36 558 975 008 ROTHSTEIN RJ J ALLERGY CLIN IMMUNOL 53 100 974 009 THAL W ASTHMA BRONCHIALE IM KINDESAL 977 010 ULMER WT DTSCH MED WSCHR 96 1759 971 011 WARNER JO ARCH DIS CHILD 51 507 976 CT 1 DARGA LL PEDIATR PULMONOL 2 82 986 PN 79185 RN 01165 AN 79221560 AU Baran-D. Wolter-R. Bourdoux-P. Ermans-A-M. TI Increased serum TSH response to TRH in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 114-8. MJ CYSTIC-FIBROSIS: pp. THYROID-GLAND: pp. THYROTROPIN-RELEASING-HORMONE: du. THYROTROPIN: bl. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. HUMAN. INFANT. INJECTIONS-INTRAVENOUS. THYROTROPIN-RELEASING-HORMONE: ad. EX The aim of the present study is to reassess the peripheral pattern of thyroid function in subjects with cystic fibrosis with special reference to their responsiveness to thyrotropin-releasing hormone (TRH) stimulation. In all the CF patients the basal levels of serum T4, T3, and TBG were within the normal range. Mean values in CF patients and in control children were not significantly different. In CF patients there is no correlation between the TSH response area and the basal level of serum T4, or with grading according to Shwachman and Kulczycki. We found no relation with therapy. In basal conditions, our data show a completely normal pattern of the serum concentration of T4, T3, TBG and TSH in subjects with CF in absence of iodide therapy. Thyroid status should be examined in patients with CF specially when they receive iodide therapy or other drugs which can enhance the impairment of thyroid function. RF 001 AZIZI F TRANS ASSOC AM PHYSICIANS 87 111 974 002 DOLAN TF JR J PEDIATR 79 684 971 CT 1 SEGALLBLANK M J PEDIATR 98 218 981 2 BECKER DJ ANNU REV NUTRITION 3 187 983 3 SACK J ISR J MED SCI 19 17 983 4 KNOPFLE G KLIN PAEDIATR 197 481 985 PN 79186 RN 01166 AN 79221559 AU Super-M. TI Factors influencing the frequency of cystic fibrosis in South West Africa. SO Monogr-Paediatr. 1979. 10. P 106-13. MJ CYSTIC-FIBROSIS: oc. MN CHILD. CONSANGUINITY. CYSTIC-FIBROSIS: fg. FEMALE. GENE-FREQUENCY. HUMAN. MALE. NAMIBIA. PEDIGREE. EX Drift, chance effect in a small community and consanguinity have resulted in South West Africa's high cystic fibrosis incidence. Isolated communities generations ago in Europe, e.g. Bad Gastein, where this congress was held, surrounded by its Alpine peaks, could have resulted in gene enrichment, which together with the increased fertility of the heterozygote could still be responsible for most of the CF seen wherever Europeans live today. RF 001 SUPER M S AFR MED J 49 818 975 002 SUPER M S AFR MED J 54 18 978 003 FALCONER DS INTRO TO QUANTITAT GENET 100 960 004 GOLDBLATT I HIST SW AFRICA FROM BEGINNING 971 005 SUPER M LANCET 2 1288 977 006 KULCZYCKI LL AM J DIS CHILD 127 64 974 007 DANKS DM ANN HUM GENET 28 323 965 CT 1 KLINGER KW HUM GENET 65 94 983 2 KLINGER KW SEM RESPIR MED 6 243 985 PN 79187 RN 01167 AN 79221558 AU Morrissey-S-M. Tymvios-M-C. TI The effect of cystic fibrosis and non-cystic fibrosis plasma on electrophysiological responses in isolated frog gastric mucosa. SO Monogr-Paediatr. 1979. 10. P 102-5. MJ CYSTIC-FIBROSIS: bl. GASTRIC-MUCOSA: ph. MN ANIMAL. CYSTIC-FIBROSIS: pp. ELECTROPHYSIOLOGY. ANURA. HUMAN. EX An alternative method for assaying the cystic fibrosis factor could be on the electrophysiological responses of isolated living tissues. The ability of the frog's gastric mucosa to secrete hydrochloric acid and maintain a negative potential difference is well known. This potential difference across the secretory (mucosa) to the nutrient (serosa) side coupled with its property of transporting chloride and hydrogen ions against an electrochemical gradient could therefore serve as a useful model for investigating any induced changes in the presence of CF and non-CF plasma. From the results obtained the short circuit current and H+ secretion responses in the presence of CF (whole) plasma show significant increases compared to the control. These findings show that in the presence of CF plasma, whole or plasma fraction B, there is a marked increase in the actively transported Cl- and H+. The effect of CF plasma in increasing net Cl- flux is important considering the elevation of this electrolyte in the sweat of CF patients and deserved further investigation. RF 001 ARAKI H PEDIATR RES 9 932 975 002 DURBIN RP J GEN PHYSIOL 41 101 957 003 HENRIQUES DE JESUS C THESIS 974 004 HOGBEN CAM AM J PHYSIOL 180 641 955 005 MANGOS JA PEDIATR RES 1 436 967 006 SPOCK A PEDIATR RES 1 173 967 CT 1 COX KL J PEDIATR GASTROENTEROL NUTR 1 559 982 2 ALBAZZAZ FJ RESPIRATION 46 88 984 3 MINA M J PHYSIOL (LOND) 348 P 4 984 PN 79188 RN 01168 AN 79221557 AU Berg-U. Kallner-A. Kusoffsky-E. Strandvik-B. TI Fatty acid supplementation in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 1-4. MJ CYSTIC-FIBROSIS: dt. FAT-EMULSIONS-INTRAVENOUS: tu. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FAT-EMULSIONS-INTRAVENOUS: ae. FATTY-ACIDS: bl. HUMAN. EX Elliott's works, together with the previous works of several authors reporting low essential fatty acid concentrations in the serum lipids of patients with cystic fibrosis, stimulated us to make a pilot study treating older CF patients with Intralipid for 1 year. We found no side effects in treating older CF patients with Intralipid for 1 year. On the other hand the objective benefit was very limited and today confusing, i.e., only a slight but significant improvement in the renal sodium excretion after an oral sodium load was found. The relevance of this is speculative. RF 001 ELLIOTT RB ARCH DIS CHILD 50 76 975 002 ELLIOTT RB PEDIATRICS 57 474 976 003 KUO PT J PEDIATR 60 394 962 004 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 005 ROSENLUND ML NATURE 251 719 974 006 GIBSON LE PEDIATRICS 23 545 959 007 BERG U ACTA PAEDIATR SCAND 62 505 973 CT 1 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 PN 79189 RN 01169 AN 79221563 AU Price-J-F. Weller-P-H. Harper-S-A. Matthew-D-J. TI Bronchial provocation tests in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 123-4. MJ ASTHMA: di. CYSTIC-FIBROSIS: co. RESPIRATORY-HYPERSENSITIVITY: di. MN ADOLESCENCE. ASTHMA: im. CHILD. CYSTIC-FIBROSIS: im. HUMAN. PEAK-EXPIRATORY-FLOW-RATE. RESPIRATORY-HYPERSENSITIVITY: im. SKIN-TESTS. EX Patients with cystic fibrosis have a higher incidence of positive skin prick tests than normal; this may be the result of defective antigen handling at mucosal surfaces. Our aim was to investigate bronchial allergy in a group of children with CF and positive skin tests. We studied 15 children aged 7 to 16 years with mild to moderate respiratory disease. Only 1 child gave a history of wheezing not associated with chest infections and this study supports the hypothesis that clinical asthma is a product of bronchial allergy and broncholability. The responses to bronchial provocation with Aspergillus suggest that allergy to this antigen may play a part in the pathogenesis of lung disease in some patients with CF. CT 1 DAVIS PB SEM RESPIR MED 6 261 985 2 DARGA LL PEDIATR PULMONOL 2 82 986 PN 79190 RN 01170 AN 79221564 AU Pryjma-J. Rudnik-J. Zebrak-J. Herman-T. Gawe-J. TI The local immune status in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 125-30. MJ BRONCHI: im. CYSTIC-FIBROSIS: im. MN ANTIBODIES: an. CHILD. CHILD-PRESCHOOL. HUMAN. IMMUNITY-CELLULAR. IMMUNOGLOBULINS: an. INFANT. LUNG-DISEASES: im. SPUTUM: im. EX We focused our study on concentration of immunoglobulins, lysozyme, lactoferrin, some antiproteolytic fractions and acute phase proteins in bronchial secretion of children with various pulmonary diseases, especially with cystic fibrosis. Secretory IgA is decreased in all studied groups of patients having chronic or recurrent infections of the respiratory tract (when compared to the group of healthy children). Groups which were characterized by the infection of the lower part of the respiratory tract had increased lysozyme level. The results obtained in the group of CF children are similar to those obtained in groups with bronchiectasis and chronic bronchitis, which suggests that the observed differences are characteristic for chronic infection rather than for CF. Although in CF as well as in bronchiectasis massive bacterial infection results in increased concentration of immunoglobulins, antiproteolytic fractions and acute phase proteins, the results seen in both groups are somewhat different. These differences may indicate some difference in the reactivity to infection of the respiratory tract in CF in comparison to patients with bronchiectasis. It seems conceivable that among children with CF who have very low S-IgA, chronic respiratory tract infections occur earlier and IgG response to permanent exposition to variety of antigens is less vigorous. The presented results raise also a possibility that the bacterial infection in CF does not stimulate defence mechanisms to the same extent as in bronchiectasis. RF 001 BROGAN TD THORAX 30 72 975 003 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 004 WALDMAN RH IN: NETER E 334 975 005 WALLWORK JC CLIN ALLERGY 6 349 976 PN 79191 RN 01171 AN 79221565 AU Schiotz-P-O. Sorensen-H. Hoiby-N. TI Complement activation and pulmonary inflammation in CF patients. SO Monogr-Paediatr. 1979. 10. P 131-4. MJ COMPLEMENT-3: an. CYSTIC-FIBROSIS: im. LUNG: im. PNEUMONIA: im. MN HUMAN. INFLAMMATION. LUNG: pa. EX This study examined whether biologic activation of C3 with subsequent pulmonary inflammation takes place in the lungs of patients with cystic fibrosis. Plasma complement C3 levels were all normal. Eleven of the patients with the C3 split product C3c were from the group of 14 patients suffering from chronic infection with mucoid strains of Pseudomonas aeruginosa and 2 were from the group of 14 CF patients without P. aeruginosa infection. This difference was significant (p < 0.002). A comparison of albumin ratios between the 13 patients with the C3 split product C3c and the remaining other 15 patients showed a significant higher ratio in the former group (p < 0.01) on an average 4.1 versus 1.4 per cent. The significantly higher albumin ratio demonstrated in the patients with C3c in their sputum sol phase supports the concept of complement-induced inflammation with subsequent transudation of plasma proteins in these patients. This study thus shows that complement-mediated inflammatory reactions may play an important role in the pathogenesis of pulmonary tissue damage in patients with CF, even when the plasma C3 levels are normal, and it supports the concept of chronic P. aeruginosa lung infection as an immune complex disease in CF patients. RF 001 BROGAN TD THORAX 30 72 975 002 CONOVER JH LANCET 2 1501 973 003 GEWURTZ H J EXP MED 128 1049 968 004 GIBSON LE PEDIATRICS 23 545 959 005 GREENBLATT J INFECT IMMUN 19 296 978 006 GOTZ M EUR J PEDIATR 127 133 978 007 HANN S LANCET 2 520 974 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 009 HOIBY N SCAND J RESPIR DIS 58 65 977 010 LAURELL CB SCAND J CLIN LAB INVEST SUPPL 124 21 972 011 LIEBERMAN J AM REV RESPIR DIS 111 100 975 012 MCFARLANE H BR MED J 1 423 975 013 NELSON RA JR IN: ZWEIFACH BW 3 37 974 014 SCANLIN TF JR LANCET 1 1382 974 015 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 83 469 975 016 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) 86 37 978 017 TEISBERG P CLIN CHIM ACTA 62 35 975 018 WEEKE B DAN MED BULL 23 155 976 CT 1 HODSON ME THORAX 35 801 980 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 79192 RN 01172 AN 79221566 AU Hein-J. David-A. Grimm-J. Gottschalk-B. TI Protein, IgA, secretory IgA, IgG and electrolytes as parameters of parotid gland function in children with cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 135-7. MJ CYSTIC-FIBROSIS: pp. ELECTROLYTES: me. IGA: an. IGA-SECRETORY: an. IGG: an. PAROTID-GLAND: pp. PROTEINS: an. SALIVA: an. MN CHILD. CYSTIC-FIBROSIS: im, me. HUMAN. EX To study the parotid gland function in children with cystic fibrosis we investigated parotid saliva specimens of 18 such patients. We compared the results with a control group of 12 healthy children of the same age and sex. Furthermore, children with bronchial asthma and chronic bronchitis were examined. The flow rate of the parotid and the protein output without stimulation were nearly the same for the four groups. In patients with CF the protein output after stimulation was significantly lower. An increased level of sodium was observed in the stimulated secretion and the unstimulated saliva as well. In CF children we found a significant higher output of sodium in both stimulated parotid saliva and unstimulated parotid saliva. The IgG output of the gland after stimulation was found to be slightly reduced in patients with CF in comparison to healthy children. The output of the gland for IgA after stimulation was observed to be strongly reduced for the group with CF and for the group with bronchial asthma. The decreased output of the gland for secretory IgA after stimulation was demonstrated again in CF and in bronchial asthma in comparison with healthy persons. The increased sodium concentration in parotid saliva may be caused in a similar way as the electrolyte abnormality of sweat in CF patients, namely by a decreased net reabsorption of sodium. Due to the result of an increased electrolyte output and decreased immunoglobulin output of parotid gland we can suppose that the molecular magnitude plays its role in this disturbance. RF 002 LAKNER V ALLERGIE IMMUNOL 23 147 977 003 MANGOS JA MOD PROBL PEDIATR 10 107 967 004 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 CT 1 KATZ S CELL CALC 5 421 984 PN 79193 RN 01173 AN 79221567 AU Hoiby-N. Hertz-J-B. TI Precipitating antibodies against E. coli, B. fragilis, and P. aeruginosa in CF patients and normal persons determined by means of crossed immunoelectrophoresis. SO Monogr-Paediatr. 1979. 10. P 138-43. MJ ANTIBODIES-BACTERIAL: an. BACTEROIDES-FRAGILIS: im. CYSTIC-FIBROSIS: im. ESCHERICHIA-COLI: im. PSEUDOMONAS-AERUGINOSA: im. MN ADOLESCENCE. ADULT. CHILD. FEMALE. HUMAN. IMMUNOELECTROPHORESIS-TWO-DIMENSIONAL. MALE. EX Wallwork and co-workers have proposed that increased absorption of antigens takes place across the mucosal membranes of the gut and respiratory tract possibly due to a transient defect in the production of secretory IgA. As a consequence of this hypothesis, one would expect cystic fibrosis patients to produce a pronounced antibody response against the intestinal bacterial flora. The present work was therefore carried out to investigate the antibody response in CF patients and in normal persons against Escherichia coli and Bacteroides fragilis which are present in the gut in large numbers and against the respiratory pathogen P. aeruginosa. A significantly higher prevalence of precipitins against each of the 3 bacterial species was found in CF patients compared to normal persons, and the prevalence of precipitins against each of these bacteria increased significantly with age. As regards P. aeruginosa precipitins, the prevalence and number was significantly higher in CF patients who have subsequently succumbed compared to CF patients who are still alive. Such a correlation between the antibody response and the prognosis of the patients was not found as regards E. coli or B. fragilis. Considering the hypothesis of Wallwork and co-workers concerning increased absorption of antigens from the gut, it seems more attractive to suppose that increased absorption of antigens from the gut flora occurs predominantly in CF patients with P. aeruginosa lung infection with a pronounced antibody response against P. aeruginosa. RF 001 FINEGOLD SM ANAEROBIC BACTERIA HUMAN DIS 977 002 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 003 HOIBY N SCAND J IMMUNOL SUPPL 2 4 187 975 004 HOIBY N ACTA PATH MICROBIOL SCAND (C) 85 142 977 005 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 006 MCFARLANE H BR MED J 1 423 975 007 SASAKI M JAPAN J EXP MED 45 335 975 008 SCHWAB JH BACT REV 39 121 975 009 TOLO K IMMUNOLOGY 33 733 977 010 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 011 WALLWORK JC CLIN ALLERGY 6 349 976 CT 1 HODSON ME THORAX 35 801 980 2 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 PN 79194 RN 01174 AN 79221577 AU Prosser-R. TI Screening for cystic fibrosis in the newborn. SO Monogr-Paediatr. 1979. 10. P 43-5. MJ CYSTIC-FIBROSIS: pc. MN CYSTIC-FIBROSIS: di. ENGLAND. HUMAN. INFANT-NEWBORN. EX In an 8-year period, a programme of screening for cystic fibrosis has been conducted over an extensive part of Wales. The screening method used is based on the detection of a high level of albumen (greater than 20 mg%) in the meconium. During the course of the investigations several different tests have been used. Because of the high incidence of false-negative results (34%) the detection of protein in meconium is not an ideal screening test, but is the most satisfactory presently available, in the newborn period. Because of its relative simplicity, low cost, absence of direct involvement of the parent or patient, and a high yield of positive cases, its use on a wider scale may be justified. RF 001 GIBSON LE PEDIATRICS 23 545 959 002 HARDY JD ARCH DIS CHILD 48 316 973 003 KOLLBERG H PROC INT CF CONG 7TH 32 976 004 PROSSER R ARCH DIS CHILD 49 597 974 005 ROBINSON MJ ARCH DIS CHILD 50 962 975 006 RYLEY HC ARCH DIS CHILD 49 901 974 CT 1 TRAVERT G J GENET HUM 28 141 980 2 TRAVERT G NOUV PRESSE MED 10 2093 981 PN 79195 RN 01175 AN 79221569 AU Michalsen-H. Rolla-G. Melsen-B. Sonju-T. TI Sulphated glycoproteins in the pancreas. SO Monogr-Paediatr. 1979. 10. P 148-50. MJ CYSTIC-FIBROSIS: me. GLYCOPROTEINS: me. PANCREAS: me. SULFATES: me. MN ANIMAL. CYSTIC-FIBROSIS: et. ANTHROPOIDEA. EX Several reports have indicated the presence of abnormally high amounts of sulphate in the mucous glands of patients with cystic fibrosis. Bearing these facts in mind, we suspected that abnormal, over-sulphated, and strongly anionic, glycoproteins might be involved in the pathogenesis of CF. The present study was carried out to determine whether sulphated blood-group substances occur in normal pancreatic tissue. The distribution of sulphate over the exocrine part of pancreas does coincide with the distribution of blood-group substance previously observed by fluorescent microscopy. We therefore find it very likely that the sulphate and blood-group substance are associated with the same glycoprotein molecule. Since blood-group substance has been demonstrated in many secretions in man, and since high amounts of sulphate have been found in the mucous glands in CF, such a mechanism is possible in many locations and may thus offer an explanation for the multiorgan involvement in CF. RF 001 HAUG A ACTA CHEM SCAND 24 843 970 002 LAMB D BR J DIS CHEST 66 239 972 003 LUDWIG H SCAND J IMMUNOL 6 724 977 004 ROLLA G ACTA PATH MICROBIOL SCAND 86 83 978 005 SMIDSROD O CHEM SOC FARADAY DISC 57 263 974 006 SZULMAN AE J EXP MED 111 785 960 007 SONJU T ARCH ORAL BIOL 19 897 974 008 SONJU T ACTA PATH MICROBIOL SCAND 83 215 975 009 SONJU T ARCH ORAL BIOL 19 897 974 010 WATKINS WM IN: GOTTSCHALK A 830 972 011 WOOD RE AM REV RESPIR DIS 113 833 976 012 YOSIZAWA Z IN: GOTTSCHALK A 5 1000 972 CT 1 ENDO M CARBOHYD RES 151 409 986 PN 79196 RN 01176 AN 79221570 AU Ryley-H-C. TI An evaluation of the meconium 'lactase test' for the detection of cystic fibrosis in the newborn. SO Monogr-Paediatr. 1979. 10. P 151-4. MJ BETA-GALACTOSIDASES: an. CYSTIC-FIBROSIS: di. GALACTOSIDASES: an. MECONIUM: an. MN ALBUMINS: an. HUMAN. INFANT-NEWBORN. EX Recently, Antonowicz et al. proposed a simple test for cystic fibrosis that could be used to verify the Boehringer-Mannheim meconium test result and thus reduce the false-positive incidence. The test is based on the detection of lactase activity in meconium. The 'lactase test' may be of some value as a confirmatory test when used with the BM-mec test although its specificity in this study was not as good as previously reported. RF 001 ANTONOWICZ I PEDIATRICS 56 782 975 002 ANTONOWICZ I LANCET 1 746 976 003 BRUNS WT AM J DIS CHILD 131 71 977 004 RYLEY HC CLIN CHIM ACTA 64 117 975 006 STEPHAN U PEDIATRICS 55 35 975 CT 1 MASTELLA G RIV ITAL PEDIATR 7 581 981 2 PEDERZINI F RIV ITAL PEDIATR 9 445 983 PN 79197 RN 01177 AN 79221571 AU Torriani-R. Chassot-J. Stocker-F. Weber-J-W. TI Two children with cystic fibrosis and myocardial fibrosis. SO Monogr-Paediatr. 1979. 10. P 155-7. MJ CYSTIC-FIBROSIS: co. MYOCARDIAL-DISEASES: et. MN CASE-REPORT. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di. HUMAN. INFANT. MYOCARDIAL-DISEASES: di. EX Usually cardiac affection in cystic fibrosis is secondary to progressive affection of the lungs (cor pulmonale). In rare cases, babies and young children show a primary and severe cardiac affection caused by myocardial fibrosis. We could recently observe two of these cases in our clinic. The cases are presented and discussed. In babies and young children cardiac insufficiency caused by cardiomyopathy might be the first presenting symptom in CF. Therefore, in all children with cardiomyopathy of unknown origin, CF has to be suspected and the sweat test has to be performed. Rapidly progressive cardiac insufficiency responding poorly to treatment and rhythm disturbances are typical. Correct early diagnosis is important for early genetic counseling. RF 001 COUSIN J J SCI MED LILLE 5 161 975 002 DESCHAMPS JP ARCH FR PEDIATR 28 417 971 003 MCGIVEN AR ARCH DIS CHILD 37 656 962 004 HIDE DW ARCH DIS CHILD 52 163 977 005 NEZELOF C ARCH FR PEDIATR 16 1035 959 006 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 007 NIZNIKOWSKA J HELV PAEDIATR ACTA 26 56 971 CT 1 SCHONI M SCHWEIZ MED WOCHENSCHR 111 658 981 PN 79198 RN 01178 AN 79221572 AU Di-Matteo-G. Romano-M. Mastella-G. Castellani-E. Rutigliano-B. Casola-L. TI Chemical comparison of normal and cystic fibrosis meconium: a quantitative and qualitative analysis of carbohydrate splitting enzymes. SO Monogr-Paediatr. 1979. 10. P 19-26. MJ CYSTIC-FIBROSIS: en. GLYCOSIDE-HYDROLASES: me. MECONIUM: en. MN ABO-BLOOD-GROUP-SYSTEM. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: bl. HUMAN. INFANT-NEWBORN. EX Meconium specimens from more than 200 normal and premature and from 5 cystic fibrosis newborns were analyzed for their level of several glycosidases. Considerable scatter of activities were found in all three groups studied and no evidence for any relation of such scatter with the blood group-specific A, B, AB, H substances was found. Alpha-glucosidase, an acid glycosidase found elevated in CF serum and fibroblasts, was elevated in all CF meconium extracts including that which had a very low albumin content. RF 001 ANTONOWICZ I PEDIATR RES 9 301 975 002 CASOLA L MONOGR PAEDIATR 10 96 979 003 HOSLI P BIOCHEM BIOPHYS RES COMMUN 73 209 976 CT 1 CASOLA L MONOGR PAEDIATR 10 96 979 2 ALHADEFF JA CLIN CHIM ACTA 117 227 981 3 RYLEY HC CLIN CHIM ACTA 135 49 983 4 SLOMIANY A J BIOL CHEM 258 8535 983 5 CABRINI G RIV ITAL PEDIATR 10 405 984 6 ALHADEFF JA PEDIATR RES 19 171 985 PN 79199 RN 01179 AN 79221573 AU Kulczycki-L-L. Wientzen-R. TI The use and abuse of antibiotics, including in management of pulmonary manifestations in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 27-30. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: dt. LUNG-DISEASES: dt. MN ANTIBIOTICS: ad. CYSTIC-FIBROSIS: co. SUBSTANCE-ABUSE. HUMAN. LUNG-DISEASES: et. TOBRAMYCIN: tu. EX Individually and collectively opinion is expressed that antibiotics are among the most frequently prescribed drugs in United States, probably also in many other countries. While it is adopted by many that the increased longevity of cystic fibrosis patients is strongly related to antibiotic usage, this has never been documented adequately. The principles which we try to follow while using antibiotics in CF patients are outlined. RF 001 ROBERTS AW AM J HOSP PHARM 29 828 972 002 SCHECKLER WE JAMA 213 264 970 003 ACHONG MR CAN MED ASSOC J 116 256 977 004 CASTLE M JAMA 237 2819 977 005 KUNIN CM ANN INTERN MED 79 555 973 006 FINLAND M J INFECT DIS 122 419 970 007 MCGOWAN JE JR J INFECT DIS 130 165 974 008 OSOL A UNITED STATES DISPENSATORY 973 009 LARAYA-CUASAY LR J PEDIATR 89 23 976 010 DAVIS BD MICROBIOLOGY 783 973 011 DI SANTAGNESE PA AM J DIS CHILD 72 17 946 012 HUANG NN J PEDIATR 59 512 961 013 BURNS MW LANCET 1 270 968 014 DOERSHUK CF IN: MANGOS JA 976 015 KULCZYCKI LL JAMA 240 30 978 PN 79200 RN 01180 AN 79221574 AU Paporisz-U. Posselt-H-G. Wonne-R. Ristow-W. Roser-D. Knothe-H. Bender-S-W. TI Long-term tobramycin therapy in patients with cystic fibrosis and advanced pulmonary disease. SO Monogr-Paediatr. 1979. 10. P 31-3. MJ ANTIBIOTICS: ad. CYSTIC-FIBROSIS: dt. LUNG-DISEASES: dt. TOBRAMYCIN: ad. MN CYSTIC-FIBROSIS: co. HUMAN. INFANT. LUNG-DISEASES: et. PSEUDOMONAS-INFECTIONS: dt, et. TIME-FACTORS. TOBRAMYCIN: tu. EX The aim of our clinical study was to find if prolonged tobramycin therapy can prevent further rapid deterioration in cystic fibrosis patients. Eleven courses of treatment were given to the nine patients. Eight of these markedly improved their physical activity and gained weight. However, only in four of these patients was clinical improvement correlated with an improved radiogram. Pseudomonas was eradicated from the sputum of only four patients during therapy but reappeared in three of them before the end of therapy. Clinical improvement and negligible side effects suggest treatment of exacerbating Pyocyaneus bronchopneumonia with prolonged tobramycin therapy. Dosage should be higher than 5 mg/kg/day with strict control of tobramycin blood level and awareness of side effects. RF 001 BOXERBAUM B J INFECT DIS SUPPL 124 293 971 002 DOGGETT RG SOUTH MED J 61 1347 968 003 DOGGETT RG J BACTERIOL 87 427 964 004 DOGGETT RG SOUTH MED J 57 1476 964 005 DOGGETT RG SOUTH MED J 57 1470 964 006 DOGGETT RG SOUTH MED J 61 1346 968 007 DOGGETT RG IN: LAWSON D PROC 5TH INT CF 175 969 008 DOGGETT RG BACT PROC 69 87 969 009 DOGGETT RG INFECT IMMUN 6 628 972 010 HAWLEY HB CURR THER RES 16 414 974 011 HARRISON GM PAEDIATRICIAN 1 180 972 012 HOFF GE SCAND J INFECT DIS 6 333 974 013 HOIBY N SCAND J RESPIR DIS 58 65 977 014 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 015 MAY JR CHEMOTHERAPY OF CHRONIC BRONC 968 016 MEARNS MB ARCH DIS CHILD 47 902 972 017 WAITZ JA ANTIMICROB AGENTS CHEMOTHER 2 431 972 018 HUMPHREY JH ADV IMMUNOL 11 75 969 PN 79201 RN 01181 AN 79221575 AU Guggenbichler-J-P. Kienel-G. TI Bioavailability of oral antibiotics in cystic fibrosis. SO Monogr-Paediatr. 1979. 10. P 34-40. MJ AMPICILLIN: aa. CEPHALEXIN: me. CYSTIC-FIBROSIS: dt. EPICILLIN: me. MN ADMINISTRATION-ORAL. BIOLOGICAL-AVAILABILITY. CEPHALEXIN: ad, bl, tu. CHILD. CYSTIC-FIBROSIS: bl, me. EPICILLIN: ad, bl, tu. HUMAN. LUNG-DISEASES: dt. EX The present investigation was designed to assess the absorption and disposition in cystic fibrosis patients of two commonly used antibiotics: an aminopenicillin, epicillin, and a cephalexin, Ospexin. The pharmacokinetic parameters of epicillin in patients with CF and their normal controls differ in a number of aspects. The difference found in the maximal plasma concentrations could be accounted for by a manifold increase in the renal clearance of the drug. Our data support the idea that patients with CF exhibit an unusually large and rapid renal clearance of certain penicillins. RF 001 SHWACHMAN H PEDIATR CLIN NORTH AM 22 787 975 002 DEREN JJ N ENGL J MED 288 949 973 003 WEBER AM N ENGL J MED 289 1001 973 005 JUSKO WJ PEDIATRICS 56 1038 975 006 JUSKO WJ J PHARM SCI 61 1270 972 007 YAFFE SJ J INFECT DIS 135 828 977 008 PARSONS RL CLIN PHARMAKOKINET 2 45 977 CT 1 MICHALSEN H ANTIMICROB AGENTS CHEMOTHER 19 1029 981 2 LEFF RD PEDIATR CLIN NORTH AM 30 93 983 3 NAHATA MC DEVELOP PHARM THER 7 221 984 4 SPINO M J PEDIATR 105 829 984 5 HARRISON CJ PEDIATR PHARMACOL 5 7 985 6 SPINO M J PEDIATR 107 64 985 7 SORGEL F EUR J PEDIATR 143 249 985 8 SMITH MJ ANTIMICROB AGENTS CHEMOTHER 30 614 986 PN 79202 RN 01182 AN 79221576 AU Stephan-U. TI Progress in screening. SO Monogr-Paediatr. 1979. 10. P 41-2. MJ CYSTIC-FIBROSIS: pc. MN CYSTIC-FIBROSIS: di. HUMAN. INFANT-NEWBORN. MASS-SCREENING. EX Since the Dresden Meeting in 1977 there have only been a few new developments of interest in special field of screening for cystic fibrosis. There is no ideal screening test for CF available today. The BM Test Meconium is the test most widely used now but many technical, psychological and methodological problems remain to be solved before the test can be endorsed without reservation. RF 001 ANTONOWICZ I LANCET 1 746 976 002 CROSSLEY JR LANCET 2 1093 977 003 ROOMANS GM ACTA PAEDIATR SCAND 67 89 978 004 RYLEY HC CLIN CHIM ACTA 64 117 975 005 HOLSCLAW DS PEDIATR ANN 7 15 978 PN 79203 RN 01183 AN 79156725 AU Davidson-A-G. Applegarth-D-A. Wong-L-T. TI Diagnosing cystic fibrosis in adults [letter]. SO N-Engl-J-Med. 1979 May 17. 300(20). P 1164. MJ CYSTIC-FIBROSIS: di. MN ADULT. AGE-FACTORS. ELECTROLYTES: an. HUMAN. SWEAT: an. EX Gordeuk et al. state that cystic fibrosis may be first diagnosed in adulthood, but we feel that a condition with such grave genetic and prognostic implications must be diagnosed with special caution in this age group. In particular, the sweat test, which is the main diagnostic tool for use with children, must be interpreted in adults in the light of possible age-related increases in normal sweat-electrolyte concentration and other factors that may affect the assay. Although it is generally accepted that "normal" sweat sodium and chloride concentrations are higher in adults than in children, there is no clear agreement about the upper limits of the normal range in adults. The volume of sweat produced during the test may also have a substantial effect on electrolyte values. For the diagnosis of cystic fibrosis in adults, an attempt should thus be made to base the diagnosis not only on "elevated" sweat-electrolyte values and clinical impression but also on pancreatic-function data. RF 001 GORDEUK V N ENGL J MED 299 1137 978 002 ANON J PEDIATR 88 711 976 003 LOBECK CC PEDIATRICS 30 172 962 004 DOBSON RL MOD PROBL PEDIATR 10 23 967 005 GUP AM ARCH INTERN MED 112 699 963 006 HADORN B CAN MED ASSOC J 98 377 968 CT 1 WONG LTK GUT 23 744 982 PN 79204 RN 01184 AN 79093917 TI Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 8-1979. SO N-Engl-J-Med. 1979 Feb 22. 300(8). P 420-7. MJ CYSTIC-FIBROSIS: pa. ESOPHAGEAL-AND-GASTRIC-VARICES: pa. LIVER-CIRRHOSIS: pa. MN BRAIN-DISEASES: di, pa. BRAIN: pa. CASE-REPORT. CHILD. CYSTIC-FIBROSIS: co, di. DIAGNOSIS-DIFFERENTIAL. ESOPHAGEAL-AND-GASTRIC-VARICES: di. FEMALE. HEPATIC-ENCEPHALOPATHY: di, et, pa. HUMAN. LIVER-CIRRHOSIS: di. LIVER: pa. EX The case of a nine-year-old girl admitted to the hospital because of coma is presented. She was found shortly after birth to have meconium ileus and a high concentration of sodium in her sweat indicative of cystic fibrosis. Clinical diagnoses were cystic fibrosis of pancreas, cirrhosis of liver, and congestive heart failure secondary to electrolyte imbalance. Anatomical diagnoses were cystic fibrosis of pancreas with cirrhosis of liver and esophageal varices, brain necrosis, metabolic encephalopathy, bronchiectasis, acute bronchopneumonia, and acute renal tubular necrosis. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 REYE RDK LANCET 2 249 963 003 WEBSTER R ARCH DIS CHILD 28 343 953 004 DI SANTAGNESE PA PEDIATRICS 18 387 956 005 CRAIG JM AM J DIS CHILD 93 357 957 006 COHN D MED J AUST 1 101 977 007 OPPENHEIMER EH J PEDIATR 86 683 975 008 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 009 BOVE KE IN: POLLACK JD 93 975 CT 1 ALLMAN FD N ENGL J MED 304 1111 981 PN 79205 RN 01185 AN 79135047 AU Feigal-R-J. Shapiro-B-L. TI Mitochondrial calcium uptake and oxygen consumption in cystic fibrosis. SO Nature. 1979 Mar 15. 278(5701). P 276-7. MJ CALCIUM: me. CYSTIC-FIBROSIS: me. MITOCHONDRIA: me. OXYGEN-CONSUMPTION. MN BIOLOGICAL-TRANSPORT-ACTIVE. CELLS-CULTURED. ELECTRON-TRANSPORT. HUMAN. KINETICS. SKIN: ul. SUPPORT-U-S-GOVT-P-H-S. EX Reports of exocrine gland secretory anomalies in cystic fibrosis and increased calcium ion concentrations in some CF secretions and the importance of cellular Ca to stimulus-secretion coupling led us to study cellular Ca in CF. We report data on Ca uptake studies with mitochondrial and microsomal fractions isolated from skin fibroblasts of subjects with CF and age-, sex- and passage number-matched controls in which mitochondria from CF cells accumulated more Ca than those from control cells. We also describe subsequent studies which show increased mitochondrial electron transport activity, measured as oxygen uptake by whole cells, in cells from subjects with CF and obligate heterozygotes. One can hypothesise a mechanism to link the altered mitochondrial Ca in CF fibroblasts and the major signs associated with the disease. Data suggest that the electron transport system in CF and obligate heterozygote fibroblasts is more active than that in respective controls, in the conditions of this study. The apparent central role of Ca in several pathogenetic pathways in CF and the data reported here in which both mitochondrial Ca capacity and oxygen consumption are increased in CF suggest that this organelle is a suitable focus for CF investigations. RF 001 LOBECK CC IN: STANBURY JB 1605 972 002 BOTELHO SY J PEDIATR 83 601 973 003 WOTMAN S ARCH ORAL BIOL 16 663 971 004 ALLARS HM PEDIATR RES 10 578 976 005 BLOMFIELD J PEDIATR RES 10 574 976 006 DOUGLAS WW BR J PHARMACOL 34 451 968 008 PENNINGTON RJ BIOCHEM J 80 649 961 009 REED KC ANAL BIOCHEM 67 44 975 010 MOORE L J CELL PHYSIOL 91 289 977 011 KIMURA S J BIOL CHEM 252 1217 977 012 SHAPIRO BL CLIN CHIM ACTA 82 125 978 013 ASH GR FEBS LETTERS 78 166 977 014 ROSE B SCIENCE 190 1204 975 015 GIBSON LE PEDIATRICS 48 695 971 016 FORSTNER JF MOD PROBL PEDIATR 19 54 977 017 WALLACH D EUR J BIOCHEM 21 433 971 018 CARAFOLI E EXP BIOL SYMP 30 89 976 019 BYGRAVE FL BIOL REV 53 43 978 CT 1 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 2 ANSAH TA CELL CALC 1 195 980 3 DAVIS PB PEDIATR RES 14 83 980 4 KATZ S CLIN CHIM ACTA 100 245 980 5 CROMWELL O LANCET 2 164 981 6 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 7 HALLINAN F MED HYPOTHESES 7 793 981 8 ROOMANS GM J SUBMICROSC CYTOL 13 445 981 9 SORSCHER EJ LANCET 1 368 982 10 SANGUINETTIBRICENO NR CLIN GENET 22 308 982 11 FEIGAL RJ LIFE SCI 30 93 982 12 SHAPIRO BL AM J HUM GENET 34 846 982 13 MATKOVICS B CLIN CHIM ACTA 125 59 982 14 SHAPIRO BL SCIENCE 216 417 982 15 FONDACARO JD LIFE SCI 32 1449 983 16 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 17 SEYMOUR CA BIOESSAYS 1 38 984 18 KATZ S CELL CALC 5 421 984 19 CABRINI G RIV ITAL PEDIATR 10 405 984 20 DEARBORN DG PEDIATR RES 18 890 984 21 VONRUECKER AA PEDIATR RES 18 594 984 22 WALLER RL LIFE SCI 35 775 984 23 RUSSI EW CHEST 86 475 984 24 GRINSTEIN S BIOCHIM BIOPHYS ACTA 769 270 984 25 CABRINI G LIFE SCI 36 1561 985 26 PINO JA ANAL CHEM 57 295 985 27 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 28 STUTTS MJ PEDIATR RES 20 1316 986 29 MORRISSEY SM DIGESTION 34 28 986 30 BATTINO M FEBS LETTERS 199 155 986 31 FEIGAL RJ ANN NY ACAD SCI 488 82 986 PN 79206 RN 01186 AN 79178685 AU Columbo-J-L. Gibbs-G-E. TI Cystic fibrosis and erroneous sweat tests. SO Nebr-Med-J. 1979 Jun. 64(6). P 164-5. MJ CYSTIC-FIBROSIS: di. SWEATING. MN CASE-REPORT. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: pp. HUMAN. MALE. EX Making the diagnosis of cystic fibrosis requires clinical suspicion and sweat tests. Physicians today are aware of the main clinical features of pulmonary and pancreatic involvement and are suspecting the diagnosis earlier than in past years. The accuracy of the sweat test is the most crucial factor in confirming the diagnosis. The present case illustrates this point. The main purpose of this paper is to serve as a reminder that the sweat test is often less than accurate as frequently performed. The principal pitfall of sweat testing at present seems to be the use of a chloride electrode applied to the skin without assurance that an adequate amount of sweat is present. RF 001 GIBSON LE CLIN PEDIATR 12 450 973 002 ANON J PEDIATR 88 711 976 003 WOOD RE AM REV RESPIR DIS 113 833 976 004 COLTMAN CA JR AM REV RESPIR DIS 93 63 966 005 ANON GAP CONF REP PROB SWEAT TESTI 975 006 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 007 GEORGE L ARCH DIS CHILD 46 139 971 CT 1 WEBSTER HL CRC CRIT REV CLIN LAB SCI 18 313 983 PN 79207 RN 01187 AN 79201313 AU Iveson-Iveson-J. TI Cystic fibrosis: a disease of unknown causes. SO Nurs-Mirror. 1979 May 24. 148(21). P 40. MJ CYSTIC-FIBROSIS: nu. MN CHILD. COMMUNITY-HEALTH-NURSING. HUMAN. EX The author looks at the disease which affects two out of every 2000 newborn children - a disease which causes most of the childhood chronic pulmonary disease in the Western world. The author tells how the Cystic Fibrosis Research Trust can help parents of affected children. PN 79208 RN 01188 AN 80034503 AU Clement-M. Jankowski-L-W. Beaudry-P-H. TI Prone immersion physical exercise therapy in three children with cystic fibrosis: a pilot study. SO Nurs-Res. 1979 Nov-Dec. 28(6). P 325-9. MJ CYSTIC-FIBROSIS: th. EXERCISE-THERAPY: mt. IMMERSION. PHYSICAL-THERAPY: mt. MN ADOLESCENCE. BRADYCARDIA. CHILD. CHILD-PRESCHOOL. COMPARATIVE-STUDY. EVALUATION-STUDIES. FEMALE. HUMAN. MALE. PILOT-PROJECTS. POSTURE. AB Prone immersion physical exercise therapy has been used successfully in the physical rehabilitation of middle-aged adults with moderate to severe chronic obstructive pulmonary disease. The purpose of this pilot project was to evaluate the effects of PIPE therapy in children with cystic fibrosis. Three children with CF, two boys aged 6 and 14 years and one girl aged 15 years, voluntarily participated in the experimental training program. The patients performed individualized group exercise three times a week on alternate days for 28 consecutive weeks at an intensity of approximately 75 percent aerobic capacity. Duration of exercise gradually increased from 15 (3 x 5 minutes) to 60 (3 x 20 minutes) minutes while the rest period after each exercise was constant at two minutes. PIPE training resulted in increased physical work capacity and maximal oxygen consumption in all three subjects. Curiously, these changes were not accompanied by training bradycardia. RF 001 ANON GUIDELINES GRADED EXERCISE TE 975 002 ASTRAND PO J APPL PHYSIOL 7 218 954 003 BARACH AL BULL NY ACAD MED 28 175 973 004 BOYLE IR J PEDIATR 88 318 976 005 CHARD MA IN: SCIPIEN GM 975 006 ANON J PEDIATR 88 711 976 007 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 008 DI SANTAGNESE PA N ENGL J MED 295 597 976 009 DOUGLAS WW AM REV RESPIR DIS 115 559 977 010 GROSSMAN ML CLIN PEDIATR 14 830 975 011 HAAS A MED CLIN NORTH AM 53 593 969 012 JANKOWSKI LW MOREHOUSE C 978 013 JANKOWSKI LW CAN J APPL SPORTS SCI 1 277 976 014 JANKOWSKI LW SCAND J REHAB MED 8 135 976 015 KANEKO K J APPL PHYSIOL 21 767 966 016 KEENS TG AM REV RESPIR DIS 116 853 977 017 LEFCOE NM AM J MED 54 343 973 018 LORIN MI AM J PHYS MED 50 215 971 019 MATTHEWS LW J PEDIATR 65 558 964 020 MILLER WF SOUTH MED J 55 1216 962 021 PAEZ PN AM REV RESPIR DIS 95 944 967 022 PARKS CR J PEDIATR 76 305 970 023 PATTON RG IN: WAECHTER EH 976 024 PIERCE AK ARCH INTERN MED 113 28 964 025 SCHOLANDER PF J BIOL CHEM 167 235 947 026 VYAS GN AM REV RESPIR DIS 103 390 971 027 WOOD RE AM REV RESPIR DIS 113 833 976 PN 79209 RN 01189 AN 80121338 AU Chu-J-Y. TI Anemia, hypoproteinemia and edema in an infant with cystic fibrosis. SO Nutr-Rev. 1979 Nov. 37(11). P 351-2. MJ CYSTIC-FIBROSIS: co. INFANT-NUTRITION-DISORDERS: et. MN ANEMIA: co. CASE-REPORT. EDEMA: co. HUMAN. HYPOPROTEINEMIA: co. INFANT. MALE. EX A patient with cystic fibrosis was encountered who presented with anemia, hypoproteinemia, edema and failure to thrive. A blood transfusion resulted in some improvement, but the symptoms recurred in a few weeks. Only after the correct diagnosis was made and appropriate therapy instituted did the patient show signs of improvement. The account of this case illustrates the importance of considering the diagnosis of cystic fibrosis in any child with malnutrition. RF 001 SHAHIDI NT J PEDIATR 59 533 961 002 FLEISHER DS J PEDIATR 64 341 964 003 DOLAN TF JR CLIN PEDIATR 9 295 970 004 LEE PA JAMA 228 585 974 005 BASS HN PEDIATRICS 59 126 977 006 GOLDMAN AS J PEDIATR 59 301 961 007 CHASE HP J PEDIATR 95 337 979 CT 1 NIELSEN OH J PEDIATR GASTROENTEROL NUTR 1 355 982 PN 79210 RN 01190 AN 80033423 TI Serum vitamin D and related mineral metabolism in cystic fibrosis. SO Nutr-Rev. 1979 Aug. 37(8). P 247-9. MJ CYSTIC-FIBROSIS: me. MINERALS: me. VITAMIN-D: bl. MN HUMAN. AB Two groups of investigators have independently explored the role of vitamin D and related mineral metabolism in patients with cystic fibrosis, and found relatively minor changes. RF 001 DI SANTAGNESE PA IN: VAUGHAN VC III 903 975 002 HAHN TJ J PEDIATR 94 38 979 003 HUBBARD VS J PEDIATR 94 84 979 004 ARNAUD SB PEDIATRICS 57 221 976 005 HUBBARD VS LANCET 2 1302 977 006 HAHN TJ J CLIN ENDOCRINOL METAB 39 274 974 007 ANON NUTR REV 26 200 968 008 REEMTSMA K PEDIATRICS 22 525 958 009 WILKINS L IN: THOMAS CC 965 010 ANON NUTR REV 33 61 975 011 HATHCOCK JN NUTR REV 34 65 976 012 ANDERSEN DH J PEDIATR 15 763 939 013 BLOMSTRAND R ACTA CHEM SCAND 21 1662 967 CT 1 LO CW AM J CLIN NUTR 42 644 985 PN 79211 RN 01191 AN 80121798 AU Sherman-A-H. Amoore-J-E. Weigel-V. TI The pyridine scale for clinical measurement of olfactory threshold: a quantitative reevaluation. SO Otolaryngol-Head-Neck-Surg. 1979 Nov-Dec. 87(6). P 717-33. MJ PYRIDINES: du. SMELL: ph. MN CYSTIC-FIBROSIS: co. EVALUATION-STUDIES. HUMAN. INDICATOR-DILUTION-TECHNICS. PERCEPTUAL-DISORDERS: di, et. REFERENCE-VALUES. SENSORY-THRESHOLDS. AB Serial dilutions of pyridine in water are employed for measuring the olfactory detection thresholds of patients. Experimental precautions are described that improve the precision and accuracy of the method. Existing data on the sensitivities of hyposmic patients are confirmed, but the sensitivities of normal subjects and of hyperosmic patients are believed to have been substantially overestimated in some earlier publications. Routine clinical applications of the revised pyridine odor threshold test are discussed. In the author's experience, the average cystic fibrosis patient is slightly hyposmic, and some cases of pituitary tumor are accompanied by a 100,000-fold hyperosmia. RF 001 SCHNEIDER RA N ENGL J MED 277 299 967 002 HENKIN RI IN: HAYASHI T 967 003 HENKIN RI IN: BOSMA JF 970 004 HENKIN RI LIFE SCI 5 331 966 005 AMOORE JE J CHEM ECOL 1 299 975 006 MITCHELL MJ Q J EXP PSYCHOL 22 301 970 007 AMOORE JE CHEM SENSES FLAVOR 3 57 978 008 AMOORE JE PERCEPT MOT SKILLS 26 143 968 009 VENSTROM D J FOOD SCI 33 264 968 010 CHEESMAN GH Q J EXP PSYCHOL 5 22 953 011 PATTERSON PM J HERED 39 295 948 012 HENKIN RI J CLIN INVEST 45 1631 966 013 HENKIN RI SCIENCE 138 1107 962 014 HERTZ J PHYSIOL BEHAV 14 89 975 015 LEMAGNEN J ARCH SCI PHYSIOL 6 125 952 016 AMOORE JE J CHEM ECOL 1 291 975 017 CURTIS RF NATURE 235 223 972 018 GUADAGNI DG J FOOD SCI 43 1347 978 019 HENKIN RI LANCET 1 823 971 020 HENKIN RI PERFUM FLAVORIST 1 19 976 021 STONE H PERCEPT PSYCHOPHYS 2 516 967 022 PARKER GH SMELL TASTE AND ALLIED SENSES 922 023 HENKIN RI LIFE SCI 5 1031 966 024 HENKIN RI JAMA 217 434 971 025 AMOORE JE CHEM SENSES FLAVOR 2 267 977 026 AMOORE JE IN: DENTON DA 975 027 ALTER M PATIENT CARE 8 76 974 CT 1 WYSOCKI CJ PROC NAT ACAD SCI USA 81 4899 984 2 MOOREGILLON V J ROY SOC MED 78 435 985 3 DOTY RL CHEM SENSES 11 259 986 4 AMOORE JE J AM WATER WORKS ASSOC 78 70 986 PN 79212 RN 01192 AN 80211288 AU Vawter-G-F. Shwachman-H. TI Cystic fibrosis in adults: an autopsy study. SO Pathol-Annu. 1979. 14 Pt 2. P 357-82. MJ CYSTIC-FIBROSIS: pa. MN ADULT. ARTERIOVENOUS-ANASTOMOSIS: pa. AUTOPSY. BILIARY-TRACT: pa. BRONCHIAL-ARTERIES: pa. BRONCHIECTASIS: pa. ENDOCRINE-GLANDS: pa. EXOCRINE-GLANDS: pa. FEMALE. HOARSENESS: pa. HUMAN. LIVER-CIRRHOSIS-BILIARY: pa. LUNG: pa. MALE. NASAL-POLYPS: pa. PANCREAS: pa. TRACHEITIS: pa. UROGENITAL-SYSTEM: pa. AB An annotated catalog of clinical and pathologic observations in an adult population with CF has been presented. The spectrum of disease is broad, and the diagnosis usually requires the demonstration of abnormalities in multiple systems. Tissues of each germ layer have been found involved in CF. Correlation of clinical and pathologic observations suggests that manifestations are essentially episodic and focal. In this population, the most characteristic abnormalities recognized by the pathologist are focal biliary cirrhosis, distinctive obstructive lesions of the male genital tract, prolonged staphylococcal or pseudomonas colonization of respiratory secretions, and obstructive bronchopulmonary disease. Some form of pancreatic atrophy is usually present. RF 001 DANKS DM ANN HUM GENET 28 323 965 002 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 003 SHWACHMAN H PEDIATRICS 30 389 962 004 GELLER A NEUROLOGY (MINN) 27 185 977 006 MELLINS RB PEDIATRICS 44 315 969 007 WARING WW PEDIATRICS 39 166 967 008 FIELD WEH BR J DIS CHEST 60 66 966 009 LIEBOW AA AM J PATHOL 25 211 949 010 LACK EE HUM PATHOL 8 39 977 012 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 013 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 014 FARBER S ARCH PATHOL 37 238 944 015 DI SANTAGNESE PA PEDIATRICS 18 387 956 016 ROBERTS WC AM J MED 32 324 962 017 BLANC WA PEDIATRICS 22 494 958 018 ESTERLY NB AM J DIS CHILD 123 200 972 019 KOPITO L PROC AM PEDIATR SOC ANNU MTG 972 020 DUNNILL MS J PATHOL BACTERIOL 77 299 959 021 DI SANTAGNESE PA N ENGL J MED 295 481 976 022 DOLAN TF JR J PEDIATR 79 684 971 024 HOLSCLAW DS J UROL 106 568 971 025 GILLES FH J NEUROPATH EX NEUROL 25 138 966 026 SUNG JH J NEUROPATH EXP NEUROL 23 567 964 027 WALLWORK JC CLIN EXP IMMUNOL 18 303 974 028 KOPITO LE PEDIATR RES 10 742 976 029 SHWACHMAN H PEDIATRICS 55 86 975 CT 1 HOLSCLAW DS CLIN CHEST MED 1 407 980 2 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 3 ABDULKARIM FW GASTROENTEROLOGY 82 758 982 4 IANNUCCI A HUM PATHOL 15 278 984 5 MCCARTHY VP GASTROENTEROLOGY 86 564 984 6 DAVIS PB SEM RESPIR MED 6 314 985 7 CASTILE R AM J DIS CHILD 139 728 985 8 STERN RC J PEDIATR GASTROENTEROL NUTR 5 35 986 9 DISIS ML PEDIATR RES 20 385 986 10 TRAVIS WD AM J CLIN PATHOL 85 419 986 11 ABDULKARIM FW ARCH PATHOL LAB MED 110 602 986 12 MCGLENNEN RC ARCH PATHOL LAB MED 110 879 986 13 HERNANZSCHULMAN M RADIOLOGY 158 629 986 14 GRISCOM NT AM J ROENTGENOL 148 691 987 PN 79213 RN 01193 AN 79157663 AU Ghadially-F-N. TI Invited review. The technique and scope of electron-probe X-ray analysis in pathology. SO Pathology. 1979 Jan. 11(1). P 95-110. (REVIEW). MJ ELECTRON-PROBE-MICROANALYSIS: mt. PATHOLOGY: is. MN ACID-PHOSPHATASE: an. ANTIGENS: me. BISMUTH: an. CALCIUM: an. CELL-NUCLEUS: an. COPPER: an. CYSTIC-FIBROSIS: di. ELECTRON-PROBE-MICROANALYSIS: is. FOREIGN-BODIES: pa. GOLD: an. HEMOSIDERIN: an. HEPATOLENTICULAR-DEGENERATION: pa. HUMAN. LUNG: an. METALS: an. MINERALS: an. MITOCHONDRIA: an. NAILS: an. PROSTHESIS. REVIEW. STAINS-AND-STAINING. EX The purpose of this article is to review briefly both the techniques and applications of electron-probe x-ray analysis, but only studies of interest to pathologists will be dealt with. The principles of x-ray fluorescence spectrometry, the capabilities and limitations of energy dispersive x-ray analysis, equipment, specimen preparation, and the method of electron-probe x-ray analysis are discussed. Applications of electron-probe x-ray analysis include mineral deposits in lungs, intranuclear bismuth inclusions, gold deposits in tissues, the nature of haemosiderin, copper in Wilson's disease, calcium in mitochondria, surgical implants, foreign bodies in eyes and wounds, the specificity of v. Kossa method for calcium, cytochemical localization of acid phosphatase, chromaffin and argentaffin reactions, the transfer of antigenic material from macrophages to lymphocytes, and the diagnosis of cystic fibrosis by electron-probe x-ray analysis of nails. RF 001 BEAVER DL ARCH PATHOL 76 89 963 002 BEEUWKES R 3RD KIDNEY INT 12 435 977 003 BERRY JP AM J PATHOL 83 427 976 004 BORLAND RM DEVELOP BIOL 50 201 976 005 BUJA LM J HISTOCHEM CYTOCHEM 24 508 976 006 BURR RE TOXICOL APPL PHARMACOL 7 588 965 007 CHANDLER JA X-RAY MICROANALYSIS ELECTRON 977 008 CHISHOLM IA CAN J OPHTHALMOL 12 316 977 009 CHONG AP AM J OBSTET GYNECOL 128 209 977 010 CLARKE JA NATURE 227 69 971 011 ECHLIN P NATURE 270 102 977 012 ELEY BM HISTOCHEM J 8 647 976 013 ESSNER E IN: HAYAT MA 1 973 014 FOWLER BA J HISTOCHEM CYTOCHEM 23 722 975 015 GARDNER DL J PATHOL 98 105 969 016 GHADIALLY FN ULTRASTRUCTURAL PATHOL CELL 975 017 GHADIALLY FN J PATHOL 120 201 976 018 GHADIALLY FN J PATHOL 124 77 978 019 GHADIALLY FN VIRCHOWS ARCH CELL PATHOL 22 135 976 021 GHADIALLY FN VIRCHOWS ARCH CELL PATHOL 16 43 974 022 GHADIALLY FN ANN RHEUM DIS 35 67 976 023 GHADIALLY FN J PATHOL 123 181 977 024 GHADIALLY FN VIRCHOWS ARCH CELL PATHOL 21 45 976 025 GOLDFISCHER S AM J PATHOL 53 883 968 026 GOLDFISCHER S J HISTOCHEM CYTOCHEM 12 72 964 027 HARRISON JD J PATHOL 121 83 977 028 HESS H KLIN WOCHENSCHR 50 776 972 029 LALONDE JMA VIRCHOWS ARCH CELL PATHOL 25 221 977 031 LANGHANS Z CHIR 22 575 885 032 LECHENE CP AM J PHYSIOL 232 F391 977 033 LEVER JD J HISTOCHEM CYTOCHEM 25 275 977 034 MAUGH TH SCIENCE 197 356 977 035 NORTON WL ARTHRITIS RHEUM 11 436 968 036 ORYSCHAK AF VIRCHOWS ARCH CELL PATHOL 17 159 974 037 ORYSCHAK AF VIRCHOWS ARCH CELL PATHOL 20 29 976 038 ORYSCHAK AF J PATHOL 119 183 976 039 PAPPENHEIMER AM AM J PATHOL 10 577 934 040 REED GB ARCH PATHOL 93 249 972 041 RICHTER GW J EXP MED 106 203 957 042 ROOMANS GM ACTA PAEDIATR SCAND 67 89 978 043 RUSS JC PROC SCANNING ELECTRO 5TH ANN 972 044 RYDER TA J MICROSC 101 143 974 045 SALSBURY AJ PROC R SOC MED 65 826 972 046 SELZER S ORAL SURG 35 828 973 047 SHUMAN H PROC NAT ACAD SCI USA 73 1193 976 048 SIPPONEN P ARCH PATHOL LAB MED 100 664 976 049 STUMP IG CLIN BIOCHEM 10 127 977 050 STURGEON P IN: WOLMAN M 969 051 THOMAS I VIRCHOWS ARCH CELL PATHOL 26 105 977 052 WACHSTEIN M AM J CLIN PATHOL 19 608 949 053 WACHSTEIN M AM J PATHOL 22 603 946 054 WOOD JG ACTA HISTOCHEM 52 143 975 055 YAROM R VIRCHOWS ARCH CELL PATHOL 15 11 973 056 YAROM R J HISTOCHEM CYTOCHEM 24 453 976 057 ZS-NAGY I J ULTRASTRUCT RES 58 22 977 CT 1 SHAW CF INORG PERSP BIOL MED 2 287 979 2 GHADIALLY FN CRC CRIT REV TOXICOL 6 303 979 3 GHADIALLY FN J SUBMICROSC CYTOL 11 503 979 4 HALL TA J MICROSC 117 145 979 5 BACHMANN S CELL TISSUE RES 213 109 980 6 DODSON RF ULTRASTRUCTURAL PATHOL 2 365 981 7 GHADIALLY FN J SUBMICROSC CYTOL 13 455 981 8 MORETON RB BIOL REV 56 409 981 9 DINSDALE D EXP MOL PATH 36 396 982 10 GHADIALLY FN VIRCHOWS ARCH CELL PATHOL 39 21 982 11 DAVID H BIOL ZENTRALBL 101 715 982 12 WEINER JM MED J AUST 1 272 983 13 WANG NS HUM PATHOL 14 888 983 14 BRAIN TJ PATHOLOGY 15 336 983 15 GOLIGORSKY MS NEPHRON 35 89 983 16 MUKHERJEE TM SCANN ELECTRON MICROSC 1983 663 983 17 GHADIALLY FN J SUBMICROSC CYTOL 16 773 984 18 WOZNIEWICZ B FOLIA HISTOCHEM CYTOBIOL 22 162 984 19 FUNAHASHI A CHEST 85 506 984 20 HOOK GR J AM COLL NUTR 4 599 985 21 GOLIGORSKY MS MINERAL ELECTROL METAB 11 301 985 22 TERZAKIS JA ORAL SURG 59 399 985 23 WARLEY A J CELL SCI 75 401 985 24 BAKER D SCANN ELECTRON MICROSC 1985 659 985 25 HALL TA MICRON MICROSC ACTA 17 91 986 26 HARTMAN LC J ORAL MAXILLOFAC SURG 44 628 986 PN 79214 RN 01194 AN 80033905 AU Cox-D-W. Talamo-R-C. TI Genetic aspects of pediatric lung disease. SO Pediatr-Clin-North-Am. 1979 Aug. 26(3). P 467-80. MJ LUNG-DISEASES: fg. MN ALPHA-1-ANTITRYPSIN: df. ASTHMA: fg. CHILD. CILIA: pa. CYSTIC-FIBROSIS: fg, pp. DEFICIENCY-DISEASES: pp. HUMAN. KARTAGENER-TRIAD: fg, pp. LUNG-DISEASES: im. AB Several genetic diseases are described which affect the lung, usually or at least in some cases, in the pediatric age group. The four disorders discussed have been selected wither for their high frequency or for their demonstration of the variety of ways in which genetic factors can influence disease: cystic fibrosis, one of the most common of the metabolic disorders inherited as an autosomal recessive trait; immotile cilia syndrome, including Kartagener's syndrome, single gene disorders involving structural abnormalities of the cilia; asthma, a common disorder in children with a genetic component; and alpha1-antitrypsin deficiency, an inherited protein deficiency commonly causing obstructive lung disease in adults and occasionally affecting children. RF 001 AFZELIUS BA SCIENCE 193 317 976 002 BIAS WB IN: AUSTEN KF 973 003 BOIS E CLIN GENET 14 73 978 004 COX DW AM REV RESPIR DIS 113 601 976 006 DANKS DM ANN HUM GENET 28 323 965 007 DI SANTAGNESE PA N ENGL J MED 295 481 976 008 ELIASSON R N ENGL J MED 297 1 977 009 FAGERHOL MK CLIN CHIM ACTA 16 199 967 010 FALLIERS CJ J ALLERGY 47 207 971 011 FISCHER TJ PEDIATRICS 62 443 978 012 GARROD AE Q J MED 6 242 913 013 GORDIS L EPIDEMIOL CHRONIC LUNG DIS IN 973 014 GRUNDBACHER FJ J ALLERGY CLIN IMMUNOL 56 104 975 015 HOLMES LB AM J MED SCI 255 13 968 016 HOUSTEK J CHEST 64 773 973 017 KARTAGENER M BEITR KLIN TUBERK 83 489 933 018 KARTAGENER M ARCH PEDIATR 79 193 962 019 KONIG P J ALLERGY CLIN IMMUNOL 54 280 974 020 KUEPPERS F IN: LENFANT C 13 978 021 KUEPPERS F AM REV RESPIR DIS 110 176 974 022 LAURELL CB SCAND J CLIN LAB INVEST 15 132 963 023 LOGAN WD DIS CHEST 48 613 965 024 LUCK D PROC NAT ACAD SCI USA 74 3456 977 025 MARSH DG SCIENCE 179 691 973 026 MOROZ SP J PEDIATR 88 19 976 027 MOSSBERG B SCAND J RESPIR DIS 59 55 978 028 MOSSBERG B MT SINAI J MED 44 837 977 029 NADLER HL IN: STANBURY JB 1683 978 030 PIERCE JA ANAL BIOCHEM 74 227 976 031 SCHAAP T IN: MANGOS JA 291 976 032 SCHWARTZ M ACTA ALLERGOL SUPPL II 5 952 033 SHARP HL J LAB CLIN MED 73 934 969 034 STURGESS JM N ENGL J MED 300 53 979 035 SVEGER T N ENGL J MED 294 1316 976 036 TALAMO RC J PEDIATR 79 20 971 037 TORGERSEN J ACTA RADIOL 28 17 947 038 TORGERSEN J AM J HUM GENET 2 361 950 CT 1 AFZELIUS BA AM J HUM GENET 33 852 981 2 TURNER JAP PEDIATRICS 67 805 981 3 HURWITZ RC CLIN GENET 22 7 982 4 YARNAL JR POSTGRAD MED 71 195 982 5 RIMPELA A ACTA PAEDIATR SCAND SUPPL 1982 1 982 6 LEVISON H EUR J RESPIR DIS 64 102 983 7 CANET E PRESSE MED 13 1607 984 8 NADEL HR RADIOLOGY 154 651 985 9 STURGESS JM AM J MED GENET 25 149 986 PN 79215 RN 01195 AN 80033914 AU Gurwitz-D. Corey-M. Francis-P-W. Crozier-D. Levison-H. TI Perspectives in cystic fibrosis. SO Pediatr-Clin-North-Am. 1979 Aug. 26(3). P 603-15. MJ CYSTIC-FIBROSIS: th. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: di, fg, ra. GROWTH. HUMAN. LUNG: pp. SEX-FACTORS. VENTILATION-PERFUSION-RATIO. EX It would seem that early diagnosis of cystic fibrosis and the institution of an aggressive treatment program before widespread irreversible lung damage has occurred may greatly prolong survival. Individual prediction at the time of diagnosis is impossible, but there are a number of factors which, when considered together, enable the clinician to place patients in a number of broadly defined risk groups. It is the definition and characteristics of these groups which would help the physician in assessment, counseling, and follow-up of patients with cystic fibrosis which are addressed in this article. The heterogeneity of cystic fibrosis, sex differences, presentation and age at diagnosis, pulmonary function, radiologic assessment, and projections for the future are discussed. RF 001 BEDROSSIAN CWM HUM PATHOL 7 195 976 002 CHALON J JAMA 218 1928 971 003 COOPER DM AM REV RESPIR DIS 109 519 974 004 COREY M AM REV RESPIR DIS 114 1085 976 005 COREY M CF CLUB ABST 978 006 CROZIER DN PEDIATR CLIN NORTH AM 21 935 974 007 ANON CF FND 1976 REP SURVI STUD PA 978 008 DANES BS J EXP MED 129 775 969 009 DI SANTAGNESE PA AM J MED 66 121 979 010 FEATHERBY EA AM REV RESPIR DIS 102 737 970 011 FOX WW PEDIATRICS 54 293 974 013 GRACEY M AUSTRALAS ANN MED 18 91 969 014 HAYASHI M AM REV RESPIR DIS 115 595 977 015 HUTCHEON M AM REV RESPIR DIS 110 458 974 017 KEENS TG AM REV RESPIR DIS 116 853 977 018 KOLLBERG H ACTA PAEDIATR SCAND 64 477 975 019 KOPEL FB GASTROENTEROLOGY 62 483 972 020 LAMARRE A PEDIATRICS 50 291 972 021 LAPIERRE JG CAN PAED SOC ANN MTG ABST 61 975 022 LEVISON H IN: MANGOS JA 3 976 023 HADORN B CAN MED ASSOC J 98 377 968 024 MANSELL A AM REV RESPIR DIS 109 190 974 025 MITCHELL-HEGGS PF Q J MED 45 479 976 026 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 027 ORENSTEIN DM AM J DIS CHILD 131 973 977 028 REID L MOD PROBL PEDIATR 10 195 967 029 REILLY BJ RADIOLOGY 98 281 971 030 SHWACHMAN H PEDIATRICS 36 689 965 031 SHWACHMAN H BIRTH DEF ORIG ART SER 8 102 972 032 SHWACHMAN H CURR PROB PEDIATR NO 10 8 978 033 SHWACHMAN H PEDIATRICS 46 335 970 034 STEPHAN U PEDIATRICS 55 35 975 035 STERN RC JAMA 239 2676 978 036 STERN RC J PEDIATR 89 406 976 037 WARWICK WJ J CHRON DIS 28 609 975 038 WOOD RE AM REV RESPIR DIS 113 833 976 039 YEAGER H JR PROC SOC EXP BIOL MED 155 115 977 CT 1 HOLSCLAW DS CLIN CHEST MED 1 407 980 2 SVENONIUS E RESPIRATION 40 226 980 3 BELL L J CAN DIET ASSOC 42 62 981 4 CORKEY CWB AM REV RESPIR DIS 124 544 981 5 CORKEY CWB AM J OBSTET GYNECOL 140 737 981 6 GASKIN K J PEDIATR 100 857 982 7 MCLAUGHLIN FJ J PEDIATR 100 863 982 8 NOLAN G J PEDIATR 101 626 982 9 PARSONS HG J PEDIATR GASTROENTEROL NUTR 2 44 983 10 PENCHARZ PB J PEDIATR GASTROENTEROL NUTR 2 400 983 11 SINNEMA G ACTA PAEDIATR SCAND 72 427 983 12 BIBERSTEIN M AM J CLIN PATHOL 80 752 983 13 CHACE KV CLIN CHIM ACTA 132 143 983 14 BLYTHE SA CLIN BIOCHEM 17 277 984 15 WELLS AL J FOOD NUTR 41 65 984 16 COREY M J INFECT DIS 149 283 984 17 WANG EEL N ENGL J MED 311 1653 984 18 CANCIANI M J PEDIATR GASTROENTEROL NUTR 4 735 985 19 SCULLY BE REV INFECT DIS 7 S669 985 20 CHACE KV BIOCHEMISTRY 24 7334 985 21 LAMBERTY JM ANAESTHESIA 40 448 985 22 SIMMONS RJ PSYCHOSOM MED 47 111 985 23 LEVY LD J PEDIATR 107 225 985 24 TABLAN OC J PEDIATR 107 382 985 25 WIESEMANN HG MONATSSCHR KINDERHEILKD 133 726 985 26 MELLIS CM MED J AUST 143 227 985 27 LEVY L J PEDIATR GASTROENTEROL NUTR 5 97 986 28 RUSH PJ SEM ARTHRITIS RHEUM 15 213 986 29 SOUTTER VL CLIN GASTROENTEROL 15 137 986 30 OLOUGHLIN E AM J CLIN NUTR 43 732 986 31 TSUI LC COLD SPRING HARBOR SYMP Q BIO 51 325 986 32 MARCOTTE JE CHEST 90 375 986 33 LEVY L J PEDIATR 108 1038 986 34 SHEPHERD RW J PEDIATR 109 788 986 35 WANG EEL PEDIATR INFECT DIS J 6 256 987 PN 79216 RN 01196 AN 80012551 AU Pearson-R-D. Lubin-A-H. TI Increased heparin binding in cystic fibrosis: a reflection of altered glycoprotein biosynthesis?. SO Pediatr-Res. 1979 Jul. 13(7). P 834-40. MJ BLOOD-PROTEINS: me. CYSTIC-FIBROSIS: bl. GLYCOPROTEINS: bi. HEPARIN: bl. MN ADOLESCENCE. ADULT. CARBOHYDRATES: bl. CYSTIC-FIBROSIS: me. FEMALE. GLYCOPROTEINS: bl. HUMAN. MALE. PROTEIN-BINDING. AB Some of the serum proteins which bind to heparin and contribute to the pH 5.57 "heparin binding capacity" of human serum are glycoproteins; those from cystic fibrosis serum were found to be 27% higher in fucose (methylpentose) content, 27% lower in sialic acid content, and 31% lower in hexose content when compared to heparin- precipitated serum glycoproteins from normal control subjects. Hexosamine content of the heparin-precipitated serum glycoproteins was the same. Results of this preliminary investigation indicate that altered carbohydrate composition in serum glycoproteins may affect significantly their heparin binding capacity. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 AYALA W J CLIN INVEST 30 781 951 003 BAIG MM J PEDIATR 86 72 975 004 BARTMAN J J PEDIATR 76 430 970 005 BENNETT G J CELL BIOL 60 258 974 006 BITTER T ANAL BIOCHEM 4 330 962 007 BOAT TF AM REV RESPIR DIS 110 428 974 008 BOWMAN BH TEX REP BIOL MED 31 611 973 009 BUTTERWORTH J CLIN CHIM ACTA 40 139 972 010 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 011 CHANGUS JE AM J PATHOL 80 317 975 012 COBURN AF TRANS ASSOC AM PHYSICIANS 66 308 953 013 COBURN AF J EXP MED 99 1 954 014 COBURN AF ARCH INTERN MED 92 185 953 015 CONOD EJ PEDIATR RES 9 724 975 016 CONNEALLY PM TEX REP BIOL MED 31 639 973 017 CUNNINGHAM DG ILL MED J 145 493 974 018 CURRAN RC BIOCHEM SOC SYMP 20 24 961 019 DANES BS LANCET 2 765 973 020 DANES BS J EXP MED 129 775 969 021 DI SANTAGNESE PA N ENGL J MED 295 481 976 022 DI SANTAGNESE PA PEDIATRICS 19 252 957 023 DI SANTAGNESE PA N ENGL J MED 277 1287 967 024 DISCHE Z J BIOL CHEM 167 189 947 025 DISCHE Z PEDIATRICS 24 74 959 026 DISCHE Z ANN NY ACAD SCI 93 526 962 027 DISCHE Z J BIOL CHEM 175 595 948 028 DOGGETT RG NATURE NEW BIOL 243 251 973 029 DOGGETT RG TEX REP BIOL MED 31 685 973 030 DOGGETT RG NATURE NEW BIOL 243 250 973 031 EHRLICH J J PHARM SCI 62 517 973 032 GUHA AK CLIN BIOCHEM 10 153 977 033 GOLDBERG AL ANNU REV BIOCHEM 45 835 974 034 GOLDBERG AL ANNU REV BIOCHEM 45 747 976 035 GIBSON LE PEDIATRICS 48 695 971 036 JOHANSEN PG LANCET 1 455 968 037 KRAUS I PEDIATRICS 47 1010 971 038 LOUISOT P CLIN CHIM ACTA 48 373 973 039 LOWRY OH J BIOL CHEM 193 265 951 040 LUSTIG B BIOCHEM Z 242 320 931 041 MANGOS JA PEDIATR RES 2 378 968 042 MARTIN F DIGESTION 1 165 968 043 MATALON R LANCET 2 838 969 044 MCCOMBS ML TEX REP BIOL MED 31 615 973 045 MCMANUS SP IR J MED SCI 143 227 974 046 MOLNAR J MOL CELL BIOCHEM 6 3 975 047 NOVAK RA TEX REP BIOL MED 34 199 976 048 PADDAY JF IN: BALAZS EA 2 970 049 PEARSON RD HEALTH LAB SCI 15 32 978 050 POLLEY MJ J MED GENET 11 249 974 051 RAO GJS PEDIATR RES 11 981 977 052 RIMINGTON C BIOCHEM J 34 931 940 053 SHAPIRA E PEDIATR RES 10 812 976 054 SLACK J J LAB CLIN MED 59 302 962 055 SPIRO RG N ENGL J MED 281 991 969 056 SPOCK A PEDIATR RES 1 173 967 057 STURGESS JM BIOCHIM BIOPHYS ACTA 320 123 973 058 WEIMER HR AM REV TUBERC 68 594 952 059 WIESMANN UN J PEDIATR 77 685 970 060 WILSON GB PEDIATR RES 10 87 976 061 WILSON GB CLIN CHIM ACTA 49 79 973 062 WINZLER RJ METHODS BIOCHEM ANAL 2 279 955 063 WINZLER RJ IN: JEANLOZ RW 2A 965 064 YOUNG MD BIOCHIM BIOPHYS ACTA 141 374 967 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 3 BLITZER MG PEDIATR RES 16 203 982 4 BLITZER MG PEDIATR RES 16 938 982 5 MARGOLIES R PEDIATR RES 16 181 982 6 SCANLIN TF BIOCHEMISTRY 21 491 982 7 MARGOLIES R PEDIATR RES 17 931 983 8 HARRIS A CLIN CHIM ACTA 128 41 983 9 ALHADEFF JA CLIN CHIM ACTA 134 1 983 10 SLOMIANY A BIOCHIM BIOPHYS ACTA 750 253 983 11 GUEANT JL CLIN CHIM ACTA 143 217 984 12 SCANLIN TF PEDIATR RES 19 368 985 13 HUHLE D Z KLIN MED 40 1199 985 PN 79217 RN 01197 AN 80012535 AU Mawhinney-T-P. Feather-M-S. Martinez-J-R. Barbero-G-J. TI The chronically reserpinized rat as an animal model for cystic fibrosis: I. Acute effect of isoproterenol and pilocarpine upon pulmonary lavage fluid. SO Pediatr-Res. 1979 Jun. 13(6). P 760-3. MJ CYSTIC-FIBROSIS: ci. ISOPROTERENOL: pd. LUNG: de. PILOCARPINE: pd. RESERPINE: pd. MN ANIMAL. CARBOHYDRATES: me. CHOLESTEROL: me. CYSTIC-FIBROSIS: me. IRRIGATION. LIPIDS: me. LUNG: me. MALE. PHOSPHATIDYLCHOLINES: me. PHOSPHOLIPIDS: me. PROTEINS: me. RATS. AB Lung lavage samples from rats treated in a chronic fashion with reserpine had mean increases of 133, 170, and 120% in the total protein, lipid, and carbohydrate contents, respectively, when compared with those of untreated control animals, when these values were expressed in terms of body weight. An acute single ip injection of the beta-adrenergic agent isoproterenol (10 mg) increased the glycoprotein content of pulmonary lavage fluid from control rats approximately 15-19%, but only 9-10% in those from reserpine treated rats. By contrast, pilocarpine (10 mg), administered in the same manner, caused a 15% increase in the total protein, carbohydrate, and lipid content of lavage samples from control rats and increased these same constituents in the lavage samples of reserpine treated rats 98, 102, and 80%, respectively. The increased total carbohydrate content in the lavage samples of the treated animals was not associated with changes in the percent distribution of neutral sugars, amino sugars, or sialic acid. The ratio of these various sugar components did not change in the lavage samples of control or reserpine treated rats upon stimulation with either pilocarpine or isoproterenol. The increased total lipid content fround in the lavage samples from the treated rats probably results from an increase in phospholipids. A decrease in phospholipid content occurred in the lavage samples of reserpine treated rats upon stimulation, while the opposite was observed in those of control animals. Chronic treatment of rats with reserpine, thus, appears to induce an enhanced production of glycoproteins in the airways and to interfere with phospholipid metabolism in the lung. In addition, the drug treatment enhances the secretory response to pilocarpine in comparison with the responses of control animals. The enhanced response or hypersecretion of glycoproteins is a quantitative one and does not seem to involve alterations in the ratios or distribution of the various sugar components. This disturbance in the secretory function of the respiratory tract, a target organ which is prominently involved in cystic fibrosis (CF) together with alterations in other exocrine glands which resemble those of CF patients, makes the reserpine treated rat a useful model for further study of possible pathogenetic mechanisms in CF. The experimental animal model for CF developed by the chronic administration of reserpine to rats has been found to have changes in the protein content of lung lavage samples, in addition to morphologic and secretory alterations in the salivary glands and the pancreas. Further analysis of the organic composition of lung lavage samples shows that the protein, carbohydrate, and lipid contents are significantly increased after chronic reserpine administration and that the response to stimulation with pilocarpine is enhanced in the treated animals when compared to that of untreated control rats. It therefore appears that chronic reserpine administration causes a hypersecretion of glycoproteins and changes in lipid metabolism in the respiratory tract of the rat and that, in conjunction with previous findings in other exocrine glands, these effects on lung function make the reserpine treated rat a useful tool for the study of the pathologic disturbance seen in the major exocrine glands affected in CF. RF 001 BOAS NF J BIOL CHEM 204 553 953 002 BOAT TF ARCH BIOCHEM BIOPHYS 177 95 976 003 BOAT TF AM REV RESPIR DIS 110 428 974 004 BARBERO GJ IN: DI SANTAGNESE PA 208 964 005 CHAKRIN LW AM REV RESPIR DIS 108 69 973 006 CHERNICK WS ANN NY ACAD SCI 106 698 963 007 DI SANTAGNESE PA N ENGL J MED 277 1287 967 008 DUBOIS M ANAL CHEM 28 350 956 009 FLEMING WW ERGEBNISSE PHYSIOLOGIE 68 55 973 010 HUANG TC ANAL CHEM 33 1405 961 011 JANATUINEN M EXP PATHOL 265 112 969 012 LAMB D BR J DIS CHEST 66 239 972 013 LEV R AM J PATHOL 46 23 965 014 LOWRY OH J BIOL CHEM 193 265 951 015 LOWRY RR LIPIDS 9 491 974 016 MARTINEZ JR J PHARMACOL EXP THER 194 384 975 017 MARTINEZ JR J PHARMACOL EXP THER 201 206 977 018 MARTINEZ JR PEDIATR RES 9 463 975 019 MARTINEZ JR PEDIATR RES 9 470 975 020 PERLMUTTER J PEDIATR RES 12 188 978 021 POORTHUIS JHM J LIPID RES 17 433 976 022 POTTER JL ANN NY ACAD SCI 106 692 963 023 ROTH RH BIOCHEM PHARMACOL 17 1581 968 024 ROUSER G IN: MARINETTI GV 1 99 967 025 SAHU S AM REV RESPIR DIS 115 233 977 026 STURGESS JM BR J EXP PATHOL 54 388 973 027 SUN SC J PHARMACOL EXP THER 161 210 968 028 THOMPSON FE PEDIATR RES 10 632 976 029 TUCKLEY B LIPIDS 9 493 974 030 VAN GOLD LMG AM REV RESPIR DIS 114 977 976 031 VARMA R J CHROMATOGR 77 222 973 032 WARREN L J BIOL CHEM 234 1971 959 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 MAWHINNEY TP PEDIATR RES 14 872 980 3 MCCURDY RE PEDIATR RES 15 1308 981 4 FORSTNER J AM J PHYSIOL 241 G443 981 5 FORSTNER G ADV EXP MED BIOL 144 199 982 6 MARTINEZ JR PEDIATR RES 17 523 983 7 MODRZAKOWSKI MC CAN J MICROBIOL 29 1339 983 8 BOYD RL PEDIATR RES 18 1028 984 9 SPICER SS ENVIRON HEALTH PERSPECT 55 193 984 10 BRADY RC EXP CELL RES 150 141 984 11 MARTINEZ JR PEDIATR RES 19 711 985 12 BRADY RC AM J PHYSIOL 248 G 54 985 13 HAZLETT D PEDIATR RES 20 1236 986 PN 79218 RN 01198 AN 80055643 AU Russell-D-H. Rosenblum-M-G. Beckerman-R-C. Durie-B-G. Taussig-L-M. Barnett-D-R. TI Altered polyamine metabolism in cystic fibrosis. SO Pediatr-Res. 1979 Oct. 13(10). P 1137-40. MJ CYSTIC-FIBROSIS: ur. POLYAMINES: ur. MN ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl. FEMALE. HETEROZYGOTE. HUMAN. MALE. PUTRESCINE: ur. SPERMIDINE: ad, bl, ur. SPERMINE: ur. AB Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, but not levels significantly different from levels in normal controls. Patients with cystic fibrosis who were administered a tracer amount of [14C]spermidine excreted 11--13% of the radiolabel within 72 hr whereas normal controls excreted 60--76% of the radiolabel within 72 hr. Spermine excretion was positively correlated with increased pathology as assessed by the National Institutes of Health (NIH) clinical score, whereas urinary putrescine and spermidine levels were negatively correlated with increased pathology. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BOWMAN BH SCIENCE 164 325 969 003 COHEN LF BLOOD 48 469 976 004 DI SANTAGNESE PA PEDIATRICS 12 549 953 005 DI SANTAGNESE PA N ENGL J MED 295 481 976 006 DI SANTAGNESE PA N ENGL J MED 277 1287 967 007 DURIE BGM CANCER RES 37 214 977 008 LUNDGREN DW CLIN CHIM ACTA 62 357 975 009 PROCTOR MS J INVEST DERMATOL 65 409 975 010 RENNERT OM IN: MANGOS JA 41 973 011 ROSENBLUM MG SCIENCE 200 1496 978 012 ROSENBLUM MG CANCER RES 38 3161 978 013 RUSSELL DH IN: RUSSELL DH 1 973 014 RUSSELL DH CLIN CHEM 23 22 977 015 RUSSELL DH POLYAMINES AS BIOCHEM MARKERS 978 016 RUSSELL DH LANCET 2 797 975 017 RUSSELL DH CLIN CHEM 21 860 975 018 SPOCK A PEDIATR RES 1 173 967 019 TAUSSIG LM J PEDIATR 82 380 973 020 TAUSSIG LM IN: KELLEY VC 4 1 976 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 THEOHARIDES TC LIFE SCI 27 703 980 3 KAMINSKA AM ANN NEUROL 9 605 981 4 PINEL JPJ PHYSIOL BEHAV 27 819 981 5 RUSSELL DH NEUROLOGY 31 80 981 6 RUSSELL DH CRC CRIT REV CLIN LAB SCI 18 261 983 7 NORDLANDER JE J ORGANIC CHEM 49 133 984 8 KAMOUN PP CLIN CHIM ACTA 154 219 986 PN 79219 RN 01199 AN 80055647 AU Martinez-J-R. Martinez-A-M. Garrett-L. Korman-P. TI Chronically reserpinized rat as a model for cystic fibrosis: Na+ transport inhibitory effect in submaxillary saliva. SO Pediatr-Res. 1979 Oct. 13(10). P 1156-9. MJ CYSTIC-FIBROSIS: me. RESERPINE: pd. SALIVA: me. SODIUM: me. MN ANIMAL. CYSTIC-FIBROSIS: ci, pp. FREEZING. HEAT. PAROTID-GLAND: se. RATS. SECRETORY-RATE. SUBMANDIBULAR-GLAND: se. AB The retrograde perfusion assay in the rat parotid gland was used to investigate the effects of salivary secretions from control and reserpine-treated rats on Na+ reabsorption. Results demonstrated that submaxillary saliva from the treated animals caused a 69% inhibition of Na+ reabsorption, accompanied by a 28% reduction in the volume of saliva secreted, and a 22% reduction in flow rate. By contrast, submaxillary saliva from control rats caused a 6% inhibition of Na+ reabsorption, a 6% reduction in volume, and a 5% reduction in flow rate. Parotid saliva from reserpine-treated rats also inhibited Na+ reabsorption to the extent of 39% and caused a 38% reduction in volume and a 33% reduction in flow. Parotid saliva from control rats only inhibited Na+ reabsorption to the extent of 2.7% and caused a 4-- 6% reduction in salivary volumes and flow rates. The inhibition of Na+ reabsorption and the reduction in salivary volume and flow rates caused by submaxillary saliva of reserpine-treated rats were either abolished or significantly reduced when the saliva was previously heated to 100 degrees C, frozen, and then thawed or kept in glass tubes at 4 degrees C for 24 hr. These results indicate that saliva from reserpine-treated rats have comparable effects in this assay system to those of saliva from cystic fibrosis (CF) patients and further support its use as an animal model for this disease. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 DI SANTAGNESE PA PEDIATRICS 12 549 953 003 MANGOS JA PFLUEGERS ARCH 291 99 966 004 MANGOS JA PEDIATR RES 1 436 967 005 MARTINEZ JR PEDIATR RES 9 463 975 006 MARTINEZ JR PEDIATR RES 9 470 975 007 PETERSEN OH ACTA PHYSIOL SCAND SUPPL 381 1 972 008 PERLMUTTER J PEDIATR RES 12 188 978 009 TAYLOR A PEDIATR RES 8 861 974 010 THOMPSON FE PEDIATR RES 10 632 976 011 YOUNG JA PROC AUST PHYSIOL PHARMACO 4 101 973 012 WEISMAN UN LANCET 1 510 972 013 WOOD DL PEDIATR RES 11 827 977 CT 1 MARTINEZ JR PEDIATR RES 15 1439 981 2 ROSCHER AA J PHARMACOL EXP THER 216 419 981 3 FORSTNER J AM J PHYSIOL 241 G443 981 4 ROOMANS GM ULTRASTRUCTURAL PATHOL 3 285 982 5 BAUM BJ J GERONTOL 37 392 982 6 ROOMANS GM SCANN ELECTRON MICROSC 1982 229 982 7 SHIFFMAN ML PEDIATR RES 17 486 983 8 VO CP ARCH ORAL BIOL 28 259 983 9 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 10 MULLER RM EXP MOL PATH 40 391 984 11 WATSON E J DENT RES 63 82 984 12 SATO K J CLIN INVEST 73 1763 984 13 MORTON AJ BR J PHARMACOL 86 287 985 14 BRADY RC AM J PHYSIOL 248 G 54 985 PN 79220 RN 01200 AN 80055641 AU Will-P-C. Boat-T-F. Hopfer-U. TI Evidence against a specific effect of serum from patients with cystic fibrosis on sodium-dependent glucose transport in the rat jejunum. SO Pediatr-Res. 1979 Oct. 13(10). P 1129-33. MJ CYSTIC-FIBROSIS: bl. GLUCOSE: me. JEJUNUM: me. SODIUM: pd. MN ANIMAL. HUMAN. IN-VITRO. MALE. MICROVILLI: me. PHLORHIZIN: pd. RATS. AB Sera from patients with cystic fibrosis of the pancreas (CF) and normal human sera were assayed for the ability to inhibit sodium- dependent glucose transport in rat brush-border membrane vesicles. Fresh CF and age- and sex-matched control sera were both inhibitory when compared to physiologic saline. The inhibition by CF serum was 44 +/- 13% (mean +/- SD) at a final serum concentration of 6.7%, 67 +/- 34% at 10% serum, and 68 +/- 28% at 20% serum. The ratio of the inhibition of CF sera compared to that of control sera was 1.00, 0.78, and 0.93 at 6.7, 10, and 20% serum concentrations, respectively. Although a slightly greater inhibition by CF serum was observed at a concentration of 10%, this is probably not significant because no difference could be detected at a concentration of 20% serum. Glucose transport in the presence of serum was sensitive to phlorizin indicating that the residual glucose transport was proceeding by the sodium-dependent glucose transport system. These findings suggest that CF serum does not specifically inhibit the sodium-dependent glucose transport system. The intravesicular space accessible to glucose was reduced in the presence of CF or control serum. Fresh CF serum was 1.4 times more effective than fresh control serum (P less than 0.01). The presence of substantial vesicle- shrinking activity in control serum indicates that this activity cannot be considered specific for CF. RF 001 ARAKI H PEDIATR RES 9 932 975 002 ARAKI H PROC INT CF CONG 7TH 28 976 003 ARVANITAKIS S TEX REP BIOL MED 34 175 976 004 BARNETT DR TEX REP BIOL MED 31 709 973 005 BENKE PJ PROC SOC EXP BIOL MED 137 1283 971 006 BENSADOUN A ANAL BIOCHEM 70 241 976 007 BROWN GA LANCET 2 639 971 008 CONOD EJ PEDIATR RES 11 45 977 009 DESJEUX JF EUROPEAN SOC PEDIAT RES MTG 970 010 DESJEUX JF CF CLUB ABST 15 974 011 DI SANTAGNESE PA N ENGL J MED 295 481 976 012 DOERSHUK CF J PEDIATR 65 677 964 013 DOGGETT RG TEX REP BIOL MED 31 685 973 014 FRIZZELL RA J MEMBR BIOL 27 297 976 015 FROMTER E NATURE NEW BIOL 235 9 972 016 GILMORE JP PROC SOC EXP BIOL MED 157 70 978 017 HOPFER U PROC NAT ACAD SCI USA 72 2027 975 018 HOPFER U J SUPRAMOL STRUCT 7 1 977 019 HOPFER U J BIOL CHEM 248 25 973 020 HOPFER U ANN NY ACAD SCI 264 414 975 021 KAISER D PEDIATR RES 5 167 971 022 LOWRY OH J BIOL CHEM 193 265 951 023 MANGOS JA TEX REP BIOL MED 31 651 973 024 MANGOS JA SCIENCE 158 135 967 025 MANGOS JA PEDIATR RES 1 436 967 026 MENG K PFLUEGERS ARCH 357 91 975 027 MORIN CL BIOMEDICINE EXPRESS 19 133 973 028 MURER H PROC NAT ACAD SCI USA 71 484 974 029 SCHMITZ J BIOCHIM BIOPHYS ACTA 323 98 973 030 SCHNEYER LH AM J PHYSIOL 219 1050 970 031 SCHNEYER LH PHYSIOL REV 52 720 972 032 SCHULTZ SG PHYSIOL REV 50 637 970 033 SHAPIRO BL PEDIATR RES 9 885 975 034 SIGRIST-NELSON K J BIOL CHEM 250 5674 975 035 TANNENBAUM C FED PROC 36 360 977 036 TAUSSIG LM LANCET 1 1367 972 037 TAYLOR A PEDIATR RES 8 861 974 038 WILSON GB NATURE 266 463 977 039 WILSON GB PEDIATR RES 11 317 977 040 ZAR JH BIOSTATISTICAL ANALYSIS 974 CT 1 WILL PC PEDIATR RES 14 1245 980 2 BANCHINI G PEDIATR RES 15 1073 981 3 ALBAZZAZ FJ RESPIRATION 46 88 984 PN 79221 RN 01201 AN 80012536 AU Feigal-R-J. Shapiro-B-L. TI Altered intracellular calcium in fibroblasts from patients with cystic fibrosis and heterozygotes. SO Pediatr-Res. 1979 Jun. 13(6). P 764-8. MJ BODY-FLUIDS: me. CALCIUM: me. CYSTIC-FIBROSIS: me. HETEROZYGOTE. INTRACELLULAR-FLUID: me. MN CELLS-CULTURED. CYSTIC-FIBROSIS: fg. FIBROBLASTS: me. HUMAN. AB The importance of intracellular calcium (Ca) in secretion and transmembrane ion movement led us to study Ca in cells from patients with cystic fibrosis (CF) which is a lethal genetic exocrinopathy. Skin fibroblasts from patients with CF, obligate heterozygotes (HZ), and age- and sex-matched controls (C) were used in matched pair experiments measuring 45Ca exchange into and efflux from the cells over time. CF cell lines and HZ cell lines exhibit increased 45Ca exchange when compared with their respective controls (P less than 0.005). The magnitude of this difference (approximately 30%) is not reduced when cells are washed with lanthanum chloride after the exchange period. This difference is likely attributable to an altered capacity of one or more of the intracellular Ca sequestering organelles. Further evidence for this explanation was seen in 45Ca efflux experiments in which CF cells retained a higher percent of their initial 0-time 45Ca than did C cells late in the efflux period (P less than 0.05). The finding of an altered Ca pool size in both CF and particularly HZ cells suggests that altered Ca metabolism is related to the basic gene defect in CF. RF 001 BAUR PS TEX REP BIOL MED 34 113 976 002 BLOMFIELD J ARCH DIS CHILD 48 267 973 003 BOGART BI PEDIATR RES 11 131 977 004 BOLTON WE TEX REP BIOL MED 34 97 976 005 BORLE AB METHODS ENZYMOL 39 513 975 006 BORLE AB TISSUE RES SUPPL 21 21 976 007 BOTELHO SY J PEDIATR 83 601 973 008 CARAFOLI E SOC SYMP 39 89 974 009 DI SANTAGNESE PA N ENGL J MED 295 481 976 010 DI SANTAGNESE PA N ENGL J MED 277 1287 967 011 DOUGLAS WW IN: CASE RM 976 012 DUNN OJ PRIMER FOR THE BIOMED SCI 964 013 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 014 FLETCHER DS CLIN CHIM ACTA 44 5 973 015 FOSTER DO J BIOL CHEM 244 2675 969 016 GIBSON LE PEDIATRICS 48 695 971 017 LEHMANN H IN: STANBURY JB 972 018 LEHNINGER AL PROC NAT ACAD SCI USA 71 1520 974 019 LEHNINGER AL ADV IMMUNOL 29 259 967 020 LOBECK CC IN: STANBURY JB 1605 972 021 MANGOS JA IN: ANDREOLI TE 978 022 MOORE L J CELL PHYSIOL 91 289 977 023 OYAMA VI PROC SOC EXP BIOL MED 91 305 956 024 RASMUSSEN H CRIT REV BIOCHEM 1 95 972 025 ROBBLEE LS BIOCHIM BIOPHYS ACTA 463 448 976 026 SHAPIRO BL CLIN CHIM ACTA 82 125 978 027 VANBREEMAN C CIRC RES 33 44 972 028 WOTMAN S ARCH ORAL BIOL 16 663 971 CT 1 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 2 ANSAH TA CELL CALC 1 195 980 3 SCANLIN TF CLIN CHEST MED 1 424 980 4 KATZ S CLIN CHIM ACTA 100 245 980 5 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 6 ROOMANS GM J SUBMICROSC CYTOL 13 445 981 7 REZNIK VM PROC NAT ACAD SCI USA 78 7143 981 8 SORSCHER EJ LANCET 1 368 982 9 SEALE TW ANN CLIN LAB SCI 12 415 982 10 BOGART BI PEDIATR RES 16 223 982 11 FEIGAL RJ LIFE SCI 30 93 982 12 SHAPIRO BL AM J HUM GENET 34 846 982 13 SHAPIRO BL SCIENCE 216 417 982 14 FONDACARO JD LIFE SCI 32 1449 983 15 MINER C J PHYSIOL (LOND) 338 P 63 983 16 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 17 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 18 SEYMOUR CA BIOESSAYS 1 38 984 19 KATZ S CELL CALC 5 421 984 20 CABRINI G RIV ITAL PEDIATR 10 405 984 21 KAMOUN P BIOCHEM MED 31 104 984 22 RUSSI EW CHEST 86 475 984 23 GRINSTEIN S BIOCHIM BIOPHYS ACTA 769 270 984 24 WALLER RL CELL CALC 6 245 985 25 KIRKPATRICK C PEDIATR RES 19 1341 985 26 SUTER S PEDIATR RES 19 346 985 27 MUALLEM S BIOCHIM BIOPHYS ACTA 819 143 985 28 RUPP GM MED HYPOTHESES 20 245 986 29 MORRISSEY SM DIGESTION 34 28 986 30 FEIGAL RJ ANN NY ACAD SCI 488 82 986 31 ROOMANS GM SCANN ELECTRON MICROSC 1986 165 986 PN 79222 RN 01202 AN 80100643 AU Van-Leuven-F. Cassiman-J-J. Van-Den-Berghe-H. TI Anomalous alpha 2-macroglobulin-protease complexes in cystic fibrosis: decreased uptake of the complexes by fibroblasts in culture. SO Pediatr-Res. 1979 Dec. 13(12). P 1384-6. MJ ALPHA-MACROGLOBULINS: me. CYSTIC-FIBROSIS: bl, en. PEPTIDE-HYDROLASES: bl. MN FIBROBLASTS: en. HUMAN. MACROMOLECULAR-SYSTEMS. TRYPSIN: bl. AB Immunochemical and functional properties of control and Cystic Fibrosis (CF) alpha 2-Macroglobulin (alpha 2M) are compared. Crossed immunoelectrophoresis and Ouchterlony double diffusion revealed no qualitative differences between the two alpha 2-M preparations. Trypsin-esterase activity assayed with BAPNA as a substrate, in the presence of an excess STI, gave similar ratios between total and active alpha 2M. These alpha 2M-trypsin complexes were equally stable under various experimental conditions and maintained a constant STI non-inhibited esterase activity. Normal and CF-alpha 2M-trypsin complexes were taken up by normal human fibroblasts to a similar extent during a four hour period. The only significant difference was observed when the uptake of alpha 2M from untreated sera was examined. The uptake of alpha 2M from CF sera was always lower than from pooled control sera despite large variation. Mixing of control and CF serum did not affect the normal uptake and other serum components were taken up to the normal extent. Intracellular degradation of CF alpha 2M had a half life of 2.0 to 2.8 hours, which compares well to the normal half life of 2.2 hours. More work needs to be done on the nature of the interaction between alpha 2M and proteases before a reasonable explanation for the molecular nature of the abnormal behavior can be sought. RF 001 GANROT PO CLIN CHIM ACTA 14 493 966 002 ROMEO G NATURE 274 909 978 003 SHAPIRA E CLIN CHIM ACTA 78 359 977 004 SHAPIRA E J BIOL CHEM 252 7923 977 005 SHAPIRA E PEDIATR RES 10 812 976 006 STARKEY PM IN: BARRETT AJ 663 977 007 VAN LEUVEN F EXP CELL RES 117 273 978 008 VAN LEUVEN F J BIOL CHEM 254 5160 979 009 VAN LEUVEN F EXP CELL RES 109 468 977 010 WILSON GB PEDIATR RES 10 87 976 CT 1 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 93 50 980 2 HUBBARD WJ J IMMUNOL 126 292 981 3 VANLEUVEN F J BIOL CHEM 256 9016 981 4 BLITZER MG J PEDIATR GASTROENTEROL NUTR 1 289 982 5 WILSON GB MED HYPOTHESES 8 527 982 6 BACK SA BIOCHEM MED 30 34 983 7 BRIDGES MA ANN NY ACAD SCI 421 360 983 8 MARYNEN P ANN NY ACAD SCI 421 401 983 9 SHAPIRA E ANN NY ACAD SCI 421 352 983 10 VANLEUVEN F ANN NY ACAD SCI 421 434 983 11 MARYNEN P BIOCHIM BIOPHYS ACTA 799 187 984 12 VANLEUVEN F J IMMUNOL METH 90 125 986 13 VANLEUVEN F J BIOL CHEM 261 1369 986 PN 79223 RN 01203 AN 80055675 AU Thomassen-M-J. Boxerbaum-B. Demko-C-A. Kuchenbrod-P-J. Dearborn-D-G. Wood-R-E. TI Inhibitory effect of cystic fibrosis serum on pseudomonas phagocytosis by rabbit and human alveolar macrophages. SO Pediatr-Res. 1979 Sep. 13(9). P 1085-8. MJ CYSTIC-FIBROSIS: bl. MACROPHAGES: ph. PHAGOCYTOSIS. PSEUDOMONAS-AERUGINOSA: cy. MN ADOLESCENCE. ADULT. AGGLUTINATION. ANIMAL. ANTIBODIES-BACTERIAL: an. CHILD. CHILD-PRESCHOOL. HUMAN. INFANT. LUNG: cy, mi. RABBITS. SUPPORT-U-S-GOVT-P-H-S. AB This report presents experimental observations indicating the presence of an inhibitory activity in cystic fibrosis (CF) serum which impairs phagocytosis of Pseudomonas aeruginosa by rabbit as well as human alveolar macrophages. Of the 49 patient serum samples studied, 40 consistently showed greater than or equal to 60% inhibition, 3 showed no inhibition and 6 were in the range of 20-60% inhibition of Pseudomonas phagocytosis. In parallel studies, the phagocytosis of S. aureus and S. marcescens was found not to be inhibited by CF serum. Mixing of CF serum with normal serum could not overcome the inhibitory effect, indicating the presence of an inhibitory factor rather than the lack of a necessary component. The inhibitory activity is not lost upon exposure of serum to glass, upon freezing the serum once, or upon heating at 56 degrees C for 30 minutes. RF 001 BIGGAR WD PROC NAT ACAD SCI USA 68 1716 971 002 BOXERBAUM B AM REV RESPIR DIS 108 777 973 003 BRAIN JD J APPL PHYSIOL 34 75 973 004 DI SANTAGNESE PA N ENGL J MED 295 534 976 005 DI SANTAGNESE PA N ENGL J MED 295 597 976 006 DOERSHUK CF IN: GREEN M 707 968 007 DOERSHUK CF PEDIATRICS 36 675 965 008 GREEN GM J EXP MED 119 167 964 009 HENNEY CS SCIENCE 169 696 970 010 HOIBY N ACTA PATH MICROBIOL SCAND (C) S262 1 977 011 KOSKI IR IN: BLOOM BR 976 012 MAY JR ARCH DIS CHILD 47 908 972 013 MYRVIK QN J IMMUNOL 86 128 961 014 NETER E J INFECT DIS SUPPL 130 132 974 015 REYNOLDS HY J CLIN INVEST 59 165 977 016 REYNOLDS HY J CLIN INVEST 56 376 975 017 SHWACHMAN H PEDIATRICS 36 689 965 018 SORENSEN RU INFECT IMMUN 18 735 977 019 SORENSEN RU J PEDIATR 93 201 978 020 WALDMAN RH J EXP MED 134 482 971 CT 1 DEMKO CA CURRENT MICROBIOL 4 69 980 2 CASSINO RJJ PEDIATR RES 14 1212 980 3 SEALE TW PEDIATR RES 14 1398 980 4 THOMASSEN MJ PEDIATR RES 14 715 980 5 FICK RB J IMMUNOL METH 38 103 980 6 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 7 FICK RB CLIN CHEST MED 2 91 981 8 SORENSEN RU PEDIATR RES 15 14 981 9 THOMASSEN MJ INFECT IMMUN 33 512 981 10 FICK RB J CLIN INVEST 68 899 981 11 LEWISTON NJ CHEST 80 389 981 12 MARKS MI J PEDIATR 98 173 981 13 MOSS RB J PEDIATR 99 215 981 14 KONSTAN MW AM REV RESPIR DIS 123 120 981 15 SORENSEN RU AM REV RESPIR DIS 123 37 981 16 VANGEFFEL R ANN IMMUNOL (PARIS) D133 293 982 17 SORDELLI DO CLIN IMMUNOL IMMUNOPATHOL 22 153 982 18 WOODS DE INFECT IMMUN 36 1223 982 19 HOOKE AM INFECT IMMUN 38 136 982 20 THOMASSEN MJ INFECT IMMUN 38 802 982 21 WINNIE GB INFECT IMMUN 38 1088 982 22 HARPER TB AM REV RESPIR DIS 126 540 982 23 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 33 982 24 SCHIOTZ PO ACTA PAEDIATR SCAND SUPPL 301 1982 55 982 25 WINNIE GB CURRENT MICROBIOL 9 63 983 26 GOLD R J ANTIMICROB CHEMOTHER 12 331 983 27 SCHILLER NL PEDIATR RES 17 747 983 28 FICK RB BULL EUR PHYSIOPATH RESP 19 151 983 29 THOMASSEN MJ J LEUKOCYTE BIOL 35 345 984 30 THOMASSEN MJ INFECT IMMUN 45 741 984 31 PIER GB J INFECT DIS 150 223 984 32 SPEERT DP SURV SYNTH PATHOL RES 4 14 985 33 SHRYOCK TR CURRENT MICROBIOL 12 91 985 34 KLEIN TW MICROBIOL IMMUNOL 29 671 985 35 REYNOLDS HY DISEASE A MONTH 31 1 985 36 SHERMAN JM J PEDIATR 106 126 985 37 PIER GB J INFECT DIS 151 575 985 38 MOSS RB MONOGR ALLERGY 19 202 986 39 MOSS RB PEDIATR RES 20 453 986 40 SHRYOCK TR J CLIN MICROBIOL 23 513 986 41 FICK RB AM REV RESPIR DIS 133 418 986 42 SHRYOCK TR CURRENT MICROBIOL 14 251 987 PN 79224 RN 01204 AN 80055648 AU Pivetta-O-H. Labal-M-L. Sordelli-D-O. TI Serum ciliotoxic activity in mutant mice with some hereditary alterations resembling cystic fibrosis. SO Pediatr-Res. 1979 Oct. 13(10). P 1160-2. MJ CILIA: ph. CYSTIC-FIBROSIS: bl. SPERM-TAIL: ph. SPERMATOZOA: ph. MN ANIMAL. CYSTIC-FIBROSIS: fg. FEMALE. HETEROZYGOTE. HOMOZYGOTE. HUMAN. MALE. MICE. MICE-INBRED-BALB-C. MICE-INBRED-C57BL. MICE-INBRED-DBA. AB Serum ciliotoxic activity (SCA), which had been described in cystic fibrosis (CF), was studied in mutant mice with some hereditary alterations resembling this inherited disease. A test was developed to detect SCA in biologic fluids by means of a natural vibratile system; it consisted in the incubation of mouse sperm with different CF serum dilutions under controlled conditions, in order to determine the sperm translation cessation time (STCT) and the sperm activity cessation time (SACT). As the findings of this study were similar to those obtained by other authors who have been employing alternative systems, it was decided to apply this method on mice serum. Sera were obtained from male and female 3- to 4-wk-old mice of DBA/2J-cri, C57BL/6J-bgJ, and BALB/c inbred strains; SCA was investigated in affected homozygotes and their +/? siblings were used as controls. Male 8- to 16-wk-old BALB/c mice were employed as sperm donors. Significant differences were found between cri/cri and +/? mice of the DBA/2J-cri strain in both STCT and SACT determinations at 1/10 serum dilution. It is concluded that mouse sperm is a system sensitive enough to detect SCA in biologic fluids from human beings. Likewise, the test of the mouse sperm allowed us to detect SCA in cri/cri mice, turning this mutation into a possible animal model for CF. RF 001 BESLEY GTN J MED GENET 6 278 969 002 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 003 BOWMAN BH SCIENCE 164 325 969 004 BOWMAN BH SCIENCE 167 871 970 005 COHEN FL J MED GENET 11 253 974 006 CONOVER JH LANCET 1 1194 973 007 DANES BS J EXP MED 136 1313 972 008 DANKS DM ANN HUM GENET 28 323 965 009 DI SANTAGNESE PA N ENGL J MED 295 481 976 010 DIXON WJ BIOMETRICS 9 74 953 011 DOGGETT RG TEX REP BIOL MED 31 685 973 012 GREEN MC SCIENCE 176 800 972 013 KAISER D LIFE SCI 15 803 974 014 LANE PW GENETICS 72 451 972 015 PIVETTA OH MEDICINA 37 379 977 016 PIVETTA OH EFECTO DEL SUERO DE MUTACIONE 975 017 PIVETTA OH PEDIATR RES 11 1133 977 018 SPOCK A PEDIATR RES 1 173 967 019 WILSON GB LIFE SCI 15 551 974 020 WOOD RE AM REV RESPIR DIS 113 833 976 021 WOOD RE LANCET 2 1452 973 CT 1 PIVETTA OH LIFE SCI 26 1349 980 2 PIVETTA OH LIFE SCI 28 2207 981 3 CERQUETTI MC IMMUNOL COMMUN 12 375 983 4 CATANZARO OL LIFE SCI 32 825 983 PN 79225 RN 01205 AN 80055663 AU Romeo-G. Parsons-M. Bossen-A. Blessing-Moore-J. Cavalli-Sforza-L-L. TI Binding of 125I-labeled proteinases to plasma proteins in cystic fibrosis. SO Pediatr-Res. 1979 Sep. 13(9). P 1030-6. MJ BLOOD-PROTEINS: me. CYSTIC-FIBROSIS: bl. PEPTIDE-PEPTIDOHYDROLASES: bl. TRYPSIN: bl. MN ALPHA-MACROGLOBULINS: me. ARGININE. ELECTROPHORESIS-POLYACRYLAMIDE-GEL. ESTERASES: bl. HUMAN. IGG: me. IMMUNOELECTROPHORESIS. IODINE-RADIOISOTOPES. PROTEIN-BINDING. SUPPORT-U-S-GOVT-P-H-S. AB Samples of plasma or serum from 53 cystic fibrosis (CF) patients, 90 relatives of CF patients , and 159 controls have been incubated with porcine or bovine 125I-trypsin, electrophoresed on polyacrylamide gel, and autoradiographed. In these individuals, the main binding protein for 125I-trypsin has been shown to be alpha 2-macroglobulin (alpha 2M). Using this method of analysis, no difference in electrophoretic migration of 125I-trypsin-alpha 2M complexes has been observed between CF and control individuals. However, trypsin binding to IgG has been observed in 80% of CF patients, 30% of their mothers, 3% of controls, and in two patients affected with pancreatitis. These trypsin binding immunoglobulins are called TbIg, and specifically, Tb1gG when referring to the G class. Experimental evidence indicates that binding of trypsin to IgG occurs through the Fab portion of the molecule. Tb1gG must be antibodies most probably induced by the exogenous trypsin ingested daily by most CF patients (and by patients affected with chronic pancreatitis). Antibodies against porcine pancreatic elastase have been observed using the same analysis, but not as frequently as Tb1g. RF 001 AARON R IMMUNOCHEMISTRY 3 451 966 002 BORGSTROM A SCAND J CLIN LAB INVEST 36 809 976 003 CHAN KYH CLIN CHIM ACTA 74 71 977 004 DAIGER S PROC NAT ACAD SCI USA 72 2076 975 005 DANES BS J EXP MED 137 1538 973 006 DEBANNE MT BR J EXP PATHOL 54 571 973 007 DOERSHUK CF J PEDIATR 65 677 964 008 DOLAN TF JR AM REV RESPIR DIS 110 812 974 009 ELIAS E LANCET 2 66 977 010 ERICKSON RP IMMUNOCHEMISTRY 11 41 974 012 GIBBONS RA BIOCHIM BIOPHYS ACTA 444 1 976 013 GOLDSMITH GH J LAB CLIN MED 89 131 977 014 HUNTER WM NATURE 194 495 962 015 KEIL B IN: BOYER PD 3 249 971 016 LAURELL CB ANAL BIOCHEM 10 358 965 017 LIEBERMAN J AM REV RESPIR DIS 109 399 974 018 MURRAY MJ ANN INTERN MED 53 548 960 019 OHLSSON K ACTA PHYSIOL SCAND 81 269 971 020 OUCHTERLONY O IN: WEIR DM 978 021 OWEN JA NATURE 182 1373 958 022 RAO GJS PEDIATR RES 8 684 974 023 ROMEO G AM J HUM GENET 29 92A 977 024 SCHROEDER DD J BIOL CHEM 243 294 968 025 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 026 SHAPIRA E PEDIATR RES 10 812 976 027 STANWORTH DR IN: WEIR DM 6 1 978 028 STEIN AA J PEDIATR 65 495 964 029 TALAMO RC PEDIATR RES 6 430 972 030 TWAROG FJ J ALLERGY CLIN IMMUNOL 59 35 977 031 WALSH-PLATT M AM J HUM GENET 29 111A 977 032 WILSON GB PEDIATR RES 10 87 976 033 YUNG BY BIOCHEM BIOPHYS RES COMMUN 65 927 975 CT 1 DENARO M PEDIATR RES 14 1086 980 2 ROMEO G J LAB CLIN MED 95 116 980 3 PARSONS M CLIN CHIM ACTA 100 215 980 4 THOMAS JM CLIN CHIM ACTA 111 199 981 5 COX KL J PEDIATR GASTROENTEROL NUTR 1 345 982 6 ROBERTS RC PEDIATR RES 16 416 982 7 BRIDGES MA ANN NY ACAD SCI 421 360 983 8 SHAPIRA E ANN NY ACAD SCI 421 352 983 PN 79226 RN 01206 AN 80055671 AU Wolf-R-O. Taussig-L. TI Serum salivary amylase in cystic fibrosis [letter]. SO Pediatr-Res. 1979 Sep. 13(9). P 1076-7. MJ AMYLASES: an. CYSTIC-FIBROSIS: en. ISOENZYMES: an. SALIVA: en. MN AMYLASES: bl. HUMAN. ISOENZYMES: bl. EX We found that cystic fibrosis (CF) patients with pancreatic insufficiency (PI) had a statistically lower total serum amylase level than normal subjects, but all values were still within the normal range. Although we found that the absolute amount of salivary amylase, in the serum of patients with CF and PI, was the same as that for normal subjects, the percent of contribution was considerably higher. We found that the pancreas contributed approximately 23% of the total serum amylase in CF patients with PI, a value very similar to the 34% amount found by Kamaryt et al. We also found, as did Davidson et al., that CF patients without PI had higher total serum amylase values than did normal subjects. Thus, we feel that Davidson et al. have misinterpreted the results of our previous study and, in fact, we feel that Davidson et al. have essentially confirmed our previous work by suggesting that CF patients with pancreatic insufficiency have an increased salivary amylase for reasons yet unknown. Our experience has shown that, indeed, the pancreatic portion is first increased and then decreases as the patient with CF and apparent normal pancreatic function progresses, with time, toward pancreatic insufficiency. However, the changes in serum amylase values in patients with CF are on the average due to changes in the pancreatic contribution to the serum and as expressed as a mean was highly significantly different for both normal and abnormal pancreatic function. The second important point of our paper was that this mean variation occurred in serum samples from patients with CF that had total amylase values within the normal test range. The serum salivary isoamylase in both normals and patients with CF and PI had a higher serum salivary percent. RF 001 SKUDE G SCAND J GASTROENTEROL 10 577 975 001 DAVIDSON GP PEDIATR RES 12 967 978 002 WOLF RO J LAB CLIN MED 87 164 976 002 HUBBARD VS J PEDIATR 92 685 978 003 KAMARYT J CESK PEDIATR 25 78 970 004 WOLF RO J LAB CLIN MED 87 164 976 CT 1 MCGEENEY KF PEDIATR RES 14 967 980 PN 79227 RN 01207 AN 79157701 AU Gabridge-M-G. Bright-M-J. Agee-C-C. Nickerson-J-M. Henderson-N-S. TI Development of an improved tracheal explant bioassay for the detection of the ciliary dyskinesia factor in cystic fibrosis serum. SO Pediatr-Res. 1979 Jan. 13(1). P 31-5. MJ CILIA: de. CYSTIC-FIBROSIS: bl. CYTOTOXINS: pd. TRACHEA: de. MN ANIMAL. CILIA: ph. COMPARATIVE-STUDY. CULTURE-MEDIA. FEMALE. GUINEA-PIGS. HAMSTERS. HUMAN. MALE. MOVEMENT. MUCUS: de. ORGAN-CULTURE: mt. RABBITS. RATS. TRACHEA: ul. SUPPORT-U-S-GOVT-P-H-S. AB A tracheal ring explant system, when used with 25% cystic fibrosis (CF) serum, displayed obvious ciliostasis. Hamster, rabbit, and guinea pig explants all had measurable decreases in ciliary activity after 24 hr of incubation in the serum. The differential response to CF serum (relative to normal serum) was greatly increased by using explants which were maintained 24-72 hr in minimal essential medium (MEM) with 10% horse serum and which were selected on the basis of optimal ciliary activity and vigor. With such a bioassay system of guinea pig tracheal explants, incubation with 25% normal serum would produce essentially no change in relative ciliary activity (score of 242 of a possible 300), whereas CF serum resulted in an 86% decrease (score of 33). Scanning electron microscopic observation indicated that the explants displaying the CF-ciliostatic effect had significant accumulations of mucous over the ciliated epithelial surface. A biochemical viability assay (dehydrogenase activity) showed no cytonecrosis when CF serum-treated tissues were compared to standard explants (10% horse serum in MEM) or control explants (25% normal human serum). RF 001 ARAD I ISR J MED SCI 11 10 975 002 BARGMAN GJ TEX REP BIOL MED 34 37 976 003 BAUR PS TEX REP BIOL MED 34 155 976 004 BERATIS NG PEDIATR RES 7 958 973 005 BOWMAN BH LIFE SCI 19 1289 976 006 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 007 CHANGUS JE ARCH PATHOL LAB MED 100 7 976 008 CHERRY JD ANN NY ACAD SCI 225 290 973 009 CHEUNG ATW PEDIATR RES 10 144 976 010 CHEUNG ATW PEDIATR RES 10 144 976 011 CONOVER JH PEDIATR RES 7 224 973 012 CONOVER JH PEDIATR RES 7 220 973 013 DI SANTAGNESE PA N ENGL J MED 295 481 976 014 FORSTNER GG CAN MED ASSOC J 113 550 975 015 GABRIDGE MG IN: EVANS V 1 976 016 GABRIDGE MG J CLIN MICROBIOL 3 560 976 017 GABRIDGE MG INFECT IMMUN 10 1127 974 018 GABRIDGE MG IN VITRO 13 510 977 019 GABRIDGE MG INFECT IMMUN 13 84 976 020 POLLEY MJ J MED GENET 11 249 974 021 SPOCK A PEDIATR RES 1 173 967 022 WOOD RE AM REV RESPIR DIS 113 833 976 023 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 KENNEDY JR PEDIATR RES 14 1173 980 2 GABRIDGE MG IN VITRO 17 847 981 3 TEGNER H ACTA PAEDIATR SCAND 70 629 981 4 GABRIDGE MG ENVIRON HEALTH PERSPECT 44 189 982 5 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 6 MA DI LIFE SCI 32 2849 983 7 RUTLAND J AM REV RESPIR DIS 128 1030 983 8 ROOMANS GM SCANN ELECTRON MICROSC 1983 697 983 9 SANDERSON MJ CELL MOTIL 4 103 984 10 RUDICK VL J CELL PHYSIOL 118 67 984 11 WOOTEN MW J CELL PHYSIOL 121 490 984 12 WOOTEN MW IN VITRO CELL DEVEL BIOL 21 207 985 13 HINGLEY ST INFECT IMMUN 51 254 986 PN 79228 RN 01208 AN 79157717 AU Verdugo-P. Hinds-T-R. Vincenzi-F-F. TI Laser light-scattering spectroscopy: preliminary results on bioassay of cystic fibrosis factor(s). SO Pediatr-Res. 1979 Feb. 13(2). P 131-5. MJ BIOLOGICAL-ASSAY: mt. CILIA: ph. CYSTIC-FIBROSIS: bl. LASERS. SPECTRUM-ANALYSIS: mt. MN CHILD. EPITHELIUM. HUMAN. MOVEMENT. SCATTERING-RADIATION. TRACHEA. SUPPORT-U-S-GOVT-P-H-S. AB The effect of 7 cystic fibrosis sera and 4 normal sera was investigated in 37 cultures of ciliated epithelium of the rabbit trachea. Serum was introduced in the Rose culture chambers in a concentration of 10% by volume. The frequency of ciliary beat was monitored by laser light-scattering spectroscopy. The results show that the response of cilia to sera can be well characterized by laser light scattering. Our preliminary evidence suggests that an early increase in the frequency of ciliary beat, followed by rapid arrest of ciliary activity, is a sensitive and characteristic response produced by cystic fibrosis serum, and not by normal serum. RF 001 BERGE P C R ACAD SCI (D)(PARIS) 256 889 967 002 BESLEY GTN J MED GENET 6 278 969 003 BOGART BI PEDIATR RES 11 131 977 004 BOWMAN BH IN: MANGOS JA 277 976 005 BOWMAN BH SCIENCE 164 325 969 006 CHEUNG ATW PEDIATR RES 10 144 976 007 CONOVER JH PEDIATR RES 7 220 973 008 CONOVER JH LANCET 2 1362 976 009 DOGGETT RG NATURE NEW BIOL 243 250 973 010 DUBIN SB PROC NAT ACAD SCI USA 57 1164 967 011 ECKERT R J PHYSIOL (LOND) 226 699 972 012 LEE WI BIOPHYS J 16 1109 976 013 LEE WI ANN BIOMED ENG 5 248 977 014 LEE WI BIOPHYS J 16 120 976 015 NOSSAL R J PHYSIQUE COLL CL 33 171 972 016 RUMERY RE IN: DANIEL JC JR 970 017 SPOCK A PEDIATR RES 1 173 967 018 VERDUGO P J CELL BIOL 75 293 977 019 WILSON GB PEDIATR RES 11 143 977 CT 1 KENNEDY JR PEDIATR RES 14 1173 980 2 VERDUGO P FERTIL STERIL 33 193 980 3 VERDUGO P J APPL PHYSIOL 48 868 980 4 WILSON GB J CLIN INVEST 66 1010 980 5 SANDERSON MJ PEDIATR RES 15 219 981 6 BOGART BI PEDIATR RES 16 223 982 7 MCNEELY MC PEDIATR RES 16 21 982 8 DULFANO MJ THORAX 37 646 982 9 HARD R TISSUE CELL 15 217 983 10 RUTLAND J AM REV RESPIR DIS 128 1030 983 11 KATZ S CELL CALC 5 421 984 12 SANDERSON MJ CELL MOTIL 5 267 985 13 BRAGA PC J PHARMACOL METHODS 16 161 986 14 HINGLEY ST INFECT IMMUN 51 254 986 PN 79229 RN 01209 AN 80100639 AU Tucker-R-D. Gibbs-G-E. Christensen-M-B. TI Cystic fibrosis serum effect on the short circuit current of rat jejunum. SO Pediatr-Res. 1979 Dec. 13(12). P 1371-4. MJ CYSTIC-FIBROSIS: bl. MN ANIMAL. CELL-MEMBRANE: me. COMPARATIVE-STUDY. CYSTIC-FIBROSIS: fg. GENOTYPE. HUMAN. JEJUNUM: bs. MALE. MEMBRANE-POTENTIALS. RATS. AB Sera from 100 subjects were tested for the presence of the cystic fibrosis (CF) "factor" utilizing the short circuit current rat jejunum bioassay. Comparing the mean percent decrease in short circuit current, the presumed normal group differs from the homozygous CF group at P = 0.001 and from the heterozygous group at P = 0.05. The two CF genotypes are statistically different at P = 0.004. There is, however, a large overlap among the three groups, which limits the assay's utility as a guide for factor purification as well as clinical use. RF 001 ARAKI H PEDIATR RES 9 932 975 002 ARAKI H PROC INT CF CONG 7TH 28 976 003 BESLEY GTN J MED GENET 6 278 969 004 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 005 BOWMAN BH SCIENCE 164 325 969 006 CHERRY JD J PEDIATR 79 937 971 007 CONOVER JH LANCET 1 1194 973 008 GILMORE JP PROC SOC EXP BIOL MED 157 70 978 009 MANGOS JA PEDIATR RES 1 436 967 010 POSSELT HG Z KINDERHEILK 110 93 971 011 SPOCK A PEDIATR RES 1 173 967 012 USSING HH ACTA PHYSIOL SCAND 61 484 964 CT 1 TUCKER RD COMPUT BIOL MED 11 153 981 2 ALBAZZAZ FJ RESPIRATION 46 88 984 3 VONEULER AM EXP MOL PATH 43 142 985 PN 79230 RN 01210 AN 80055672 AU Tully-G-W. Nevin-G-B. Young-I-R. Nevin-N-C. TI Detection of cystic fibrosis protein by isoelectric focusing of serum [letter]. SO Pediatr-Res. 1979 Sep. 13(9). P 1078. MJ BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. ISOELECTRIC-FOCUSING. MN HUMAN. EX Several cystic fibrosis research groups have independently attempted to reproduce the isoelectric focusing work of Wilson et al. The protocol that we employed with the LKB Mulitphor electrofocusing apparatus was that of Wilson et al., although fixing and staining of the polyacrylamide gels was performed in a boiling water bath for 30 min. In 10 of 11 sera from patients with CF, the cystic fibrosis protein band was directly identified by each of two independent observers and in the sera from nine obligate heterozygote individuals, the CFP band was noted in seven and eight instances by each observer, respectively. In sera from 26 normal control individuals, the CFP band was observed in two instances. Our results would support the view that the CFP band is indeed directly identifiable in both CT heterozygote and homozygote sera without recourse to photographic image enhancement and that its frequency of occurrence is similar to that reported previously. RF 001 ALTLAND K HUMANGENETIK 28 207 975 002 BEARN AG N ENGL J MED 286 764 972 003 DANES BS CLIN GENET 11 83 977 004 POLLEY MJ J MED GENET 11 249 974 005 SCHOLEY J PEDIATR RES 12 800 978 006 SMITH QT PEDIATR RES 10 999 976 007 THOMAS JM PEDIATR RES 11 1148 977 009 WILSON GB PEDIATR RES 11 986 977 010 WILSON GB PEDIATR RES 12 801 978 011 WILSON GB PEDIATR RES 9 635 975 CT 1 TARNOKY AL J ROY SOC MED 73 73 980 2 NEVIN GB HUM GENET 56 387 981 3 HEELEY AF CLIN CHEM 29 2011 983 4 JAMIESON A HUM GENET 70 168 985 5 GETLIFFE KA J INHERIT METAB DIS 9 348 986 PN 79231 RN 01211 AN 80055673 AU Wilson-G-B. TI Cystic fibrosis protein, a confirmed diagnostic marker for detecting heterozygote carriers: significance in relation to future screening and to a proposed primary defect in alpha 2-macroglobulin. SO Pediatr-Res. 1979 Sep. 13(9). P 1079-81. MJ ALPHA-MACROGLOBULINS: me. BLOOD-PROTEINS: an. CYSTIC-FIBROSIS: bl. HETEROZYGOTE-DETECTION. MN CYSTIC-FIBROSIS: di, fg. GENETIC-SCREENING. HUMAN. ISOELECTRIC-FOCUSING. SUPPORT-U-S-GOVT-P-H-S. EX The fact that alpha2-macroglobulin and cystic fibrosis CDA are made by fibroblasts and monocytes points to the fact that these markers and the CF protein will be of value for the detection of the CF gene antenatally. Screening for CFP and CF-CDA by isoelectric focusing or radioimmunoassay in conjunction with quantitation of the functional capacity of A2M could distinguish a fetus homozygous from one heterozygous for the CF gene. RF 001 ASHWELL G ADV ENZYM MOLEC BIOL 41 99 974 002 DANES BS TEX REP BIOL MED 34 135 976 003 DANN LG LANCET 2 405 978 004 HOVY M J EXP MED 145 1580 977 005 ANON J PEDIATR 88 711 976 006 LOUISOT P CLIN CHIM ACTA 48 373 973 007 MOSHER DF J EXP MED 143 462 976 008 NADLER HL IN: STANBURY JB 1683 978 009 OHLSSON K CLIN SCI MOL MED 51 87 976 010 RAO GJS PEDIATR RES 11 981 977 011 SCHOLEY J PEDIATR RES 12 800 978 012 SHAPIRA E BIOCHEM BIOPHYS RES COMMUN 71 864 976 013 SHAPIRA E PEDIATR RES 10 812 976 014 SMITH QT PEDIATR RES 10 999 976 015 THOMAS JM CF CLUB ABST 17 19 976 016 THOMAS JM PEDIATR RES 11 1148 977 017 TULLY GW PEDIATR RES 13 1078 979 019 WILSON GB PEDIATR RES 11 986 977 021 WILSON GB TEX REP BIOL MED 34 51 976 022 WILSON GB PEDIATR RES 10 87 976 023 WILSON GB NATURE 266 463 977 024 WILSON GB PEDIATR RES 12 801 978 025 WILSON GB J LAB CLIN MED 92 463 978 026 WILSON GB PEDIATR RES 9 635 975 027 WILSON GB PEDIATR RES 11 139 977 028 WOLFF J ENDOCRINOLOGY 101 1767 977 029 ANON GENETIC SCREENING PROG PRIN A 975 030 ANON GAP CONF REP PROB REPRO PHYSI 975 CT 1 MANSON JC LANCET 1 330 980 2 WILSON GB LANCET 2 313 980 3 WILSON GB J CLIN INVEST 66 1010 980 4 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 5 FREZAL J ARCH FR PEDIATR 38 217 981 6 WILSON GB J CLIN INVEST 68 171 981 7 HALLINAN FM CLIN CHIM ACTA 117 103 981 8 HUBBARD WJ J IMMUNOL 126 292 981 9 WILSON GB MED HYPOTHESES 8 527 982 10 BLITZER MG PEDIATR RES 16 203 982 11 BULLOCK S CLIN GENET 21 336 982 12 BROCK DJH HUM GENET 60 30 982 13 BURY AF CLIN CHIM ACTA 118 45 982 14 HEELEY AF CLIN CHEM 29 2011 983 15 BRIDGES MA ANN NY ACAD SCI 421 360 983 16 GRATAROLI R PEDIATR RES 18 130 984 17 ANON LANCET 2 249 985 18 LAROCHE D J PEDIATR GASTROENTEROL NUTR 5 404 986 PN 79232 RN 01212 AN 80012528 AU Nagy-E-C. Khan-S. Sturgess-J-M. TI Serum factor in cystic fibrosis: correlation with clinical parameters. SO Pediatr-Res. 1979 Jun. 13(6). P 729-32. MJ CYSTIC-FIBROSIS: bl. PEPTIDES: bl. MN ADOLESCENCE. ADULT. ANIMAL. BIOLOGICAL-ASSAY. CARBON-DIOXIDE: bl. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: en. FEMALE. GALACTOSYLTRANSFERASES: bl. HUMAN. INFANT. MALE. OXYGEN: bl. RABBITS. RESPIRATORY-FUNCTION-TESTS. TRACHEA. AB The relationship between the activity of the cystic fibrosis serum ciliary dyskinesia factor, detected by the rabbit tracheal bioassay, and clinical status of the patient has been investigated in children, 1-24 years old, with cystic fibrosis. No significant correlation was found between the amount of serum factor activity and age, clinical status assessed by the Schwachman score, pulmonary function (vital capacity, functional residual capacity, total lung capacity, residual volume, maximum breathing capacity, maximal midinspiratory flow, and maximal midexpiratory flow) or blood gas levels (pCO2, pO2). The activity showed no significant relationship to serum galactosyltransferase activity in children with cystic fibrosis. RF 001 ADSHEAD PC ANN NY ACAD SCI 253 192 975 002 ARAKI H PEDIATR RES 9 932 975 003 BAKER AP IN: FALKNER F 19 977 004 BAUR PS TEX REP BIOL MED 34 155 976 005 BERATIS NG PEDIATR RES 7 958 973 006 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 007 BOWMAN BH SCIENCE 164 325 969 008 CONOVER JH PEDIATR RES 7 224 973 009 CZEGLEDY-NAGY E LAB INVEST 35 588 976 010 DOGGETT RG NATURE NEW BIOL 243 251 973 011 LOUISOT P CLIN CHIM ACTA 48 373 973 012 MANGOS JA PEDIATR RES 2 378 968 013 RAO GJS PEDIATR RES 11 981 977 014 SANCHIS J N ENGL J MED 289 749 974 015 SINGER L CLIN BIOCHEM 7 146 974 016 SPOCK A PEDIATR RES 1 173 967 017 STURGESS JM IN: FALKNER F 19 977 018 WARWICK WJ CF CLUB ABST 18 91 977 019 WILSON GB PEDIATR RES 11 986 977 020 WOOD RE AM REV RESPIR DIS 113 833 976 021 WOOD RE AM REV RESPIR DIS 111 733 975 022 YEATES DB ARCH DIS CHILD 51 28 976 CT 1 SORENSEN RU PEDIATR RES 15 14 981 2 RUTLAND J AM REV RESPIR DIS 128 1030 983 3 WOOTEN MW J CELL PHYSIOL 121 490 984 4 WOOTEN MW IN VITRO CELL DEVEL BIOL 21 207 985 PN 79233 RN 01213 AN 79246210 AU Chase-H-P. Cotton-E-K. Elliott-R-B. TI Intravenous linoleic acid supplementation in children with cystic fibrosis. SO Pediatrics. 1979 Aug. 64(2). P 207-13. MJ CYSTIC-FIBROSIS: dh. LINOLEIC-ACIDS: tu. MN ARACHIDONIC-ACIDS: bl. BODY-HEIGHT. BODY-WEIGHT. CALORIC-INTAKE. CHILD. CHILD-PRESCHOOL. CLINICAL-TRIALS. CYSTIC-FIBROSIS: pa, pp. HUMAN. FAT-EMULSIONS-INTRAVENOUS: tu. LINOLEIC-ACIDS: bl. PLACEBOS. RESPIRATORY-FUNCTION-TESTS. SKINFOLD-THICKNESS. SODIUM: me. SWEAT: me. SUPPORT-U-S-GOVT-P-H-S. AB Ten children with CF in matched pairs were infused with either Intralipid or with 10% glucose on a double blind basis every other week for one year. Although statistically there was significantly greater gain in height and weight in the study year compared to the previous year only for the test group, both groups improved more than expected. Cumulative data analysis showed greater improvement for the Intralipid group (23 of a possible 45 points) compared to the glucose group (-2 points; P less than .02). This study indicates the need to better define the role of nutrition in the pathophysiology of CF. Meanwhile, it is recommended that all children with CF have plasma linoleic acid levels measured at least once yearly, and if levels are low, appropriate supplements should be given. RF 001 KUO PT J PEDIATR 60 394 962 002 CAREN R J CLIN ENDOCRINOL METAB 26 470 966 003 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 004 RIVERS JPW LANCET 2 642 975 005 ELLIOTT RB PEDIATRICS 57 474 976 006 ROSENLUND ML PEDIATRICS 59 428 977 007 BERRY HK AM J DIS CHILD 129 165 975 008 JELLIFFE DB ASSESSMENT NUTRITIONAL STATUS 966 009 FRISANCHO AR AM J CLIN NUTR 27 1052 974 010 CHASE HP LANCET 2 236 978 011 OBRIEN D LAB MANUAL PEDIAT MICROBIOC 968 012 MOORE RB J ALLERGY CLIN IMMUNOL 49 137 972 013 HOLLANDER M NONPARAMET STAT METHODS 973 014 VERGROESEN AJ NUTR REV 35 1 977 015 CAMPBELL IM PEDIATRICS 57 480 976 016 HANSEN AE PEDIATRICS 31 171 963 017 HOPKINS DT PROC SOC EXP BIOL MED 114 82 963 018 DAVIDSON M IN: RUDOLPH AM 994 977 019 WEIHOFEN DM J AM DIET ASSOC 54 206 969 020 KHAW K-T CF CLUB ABST 58 978 CT 1 KARP RJ CLIN CHEST MED 1 375 980 2 PARSONS HG PEDIATRICS 66 812 980 3 HUBBARD VS J PEDIATR 96 421 980 4 SHEPHERD R J PEDIATR 97 351 980 5 HUBBARD VS CLIN CHIM ACTA 102 115 980 6 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 7 HARPER TB AM REV RESPIR DIS 126 540 982 8 KUSOFFSKY E J PEDIATR GASTROENTEROL NUTR 2 434 983 9 ROGIERS V EUR J PEDIATR 141 39 983 10 BERTRAND JM J PEDIATR 104 41 984 11 DAVIS PB J LAB CLIN MED 104 203 984 12 MCKENNA MC J PEDIATR GASTROENTEROL NUTR 4 45 985 13 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 14 LESTER LA J PARENT ENTERAL NUTR 10 289 986 15 MISCHLER EH PEDIATR RES 20 36 986 16 DUHAMEL JF ARCH FR PEDIATR 43 229 986 17 CARLSTEDTDUKE J PROC NAT ACAD SCI USA 83 9202 986 PN 79234 RN 01214 AN 79179506 AU Brasfield-D. Hicks-G. Soong-S. Tiller-R-E. TI The chest roentgenogram in cystic fibrosis: a new scoring system. SO Pediatrics. 1979 Jan. 63(1). P 24-9. MJ CYSTIC-FIBROSIS: ra. LUNG: ra. MN CHILD. CYSTIC-FIBROSIS: pp. EVALUATION-STUDIES. HUMAN. PROGNOSIS. RESPIRATORY-FUNCTION-TESTS. AB A roentgenogram scoring system is presented that is useful in the evaluation and follow-up of patients with cystic fibrosis. The system has been shown to be reproducible by and between observers and to correlate significantly with results of pulmonary function tests, Shwachman-Kulczycki scores, and, in a short-term evaluation, morbidity. RF 001 HODSON CJ CLIN RADIOL 13 54 962 002 SHWACHMAN H AM J DIS CHILD 96 6 958 003 REILLY BJ RADIOLOGY 98 281 971 004 CHRISPIN AR PEDIATR RADIOL 2 101 974 005 MATTHEW DJ PEDIATR RADIOL 5 198 977 006 DOERSHUK CF J PEDIATR 65 677 964 007 COOPERMAN EM CAN MED ASSOC J 105 580 971 008 TAUSSIG LM J PEDIATR 82 380 973 009 WOOD RE AM REV RESPIR DIS 113 833 976 010 DOERSHUK CF PEDIATRICS 36 675 965 011 KNUDSON RJ AM REV RESPIR DIS 113 587 976 012 ZAPLETAL A J APPL PHYSIOL 26 308 969 013 DRAPER NR APPLIED REGRESSION ANALYSIS 966 CT 1 CHIPPS BE J PEDIATR 95 379 979 2 LESTER LA J PEDIATR 97 742 980 3 BRASFIELD D AM J ROENTGENOL 134 1195 980 4 FEIGELSON J ARCH FR PEDIATR 38 617 981 5 HEN J J PEDIATR 99 585 981 6 HYATT AC J PEDIATR 99 307 981 7 NOLAN G J PEDIATR 101 626 982 8 DANTZKER DR AM REV RESPIR DIS 125 400 982 9 TUMMLER B MONATSSCHR KINDERHEILKD 130 157 982 10 KATZ SM JAMA 248 2284 982 11 PALMER J ANN INTERN MED 99 596 983 12 HARMS HK KLIN PAEDIATR 195 24 983 13 MCCARTHY VP GASTROENTEROLOGY 86 564 984 14 BERTRAND JM J PEDIATR 104 41 984 15 WHEELER WB J PEDIATR 104 695 984 16 MCCARTHY VP ARCH INTERN MED 144 408 984 17 WANG EEL N ENGL J MED 311 1653 984 18 AUERBACH HS LANCET 2 686 985 19 PANIDIS IP J AM COLL CARDIOL 6 701 985 20 TEMEERMAN GJ PEDIATR RADIOL 15 98 985 21 FARRELL PM PEDIATR RES 19 104 985 22 BOSSO JA ANTIMICROB AGENTS CHEMOTHER 28 829 985 23 JEWETT CV J PEDIATR 106 669 985 24 ZACH MS AM REV RESPIR DIS 131 537 985 25 SMITH TF AM J DIS CHILD 139 1039 985 26 BOLAND MP LANCET 1 232 986 27 OBERWALDNER B PEDIATR PULMONOL 2 358 986 28 RUBIO TT J ANTIMICROB CHEMOTHER 18 147 986 29 JACOBSEN LE J CAN ASSOC RADIOL 37 17 986 30 KATZ JN J PEDIATR 108 352 986 31 HARDY KA J PEDIATR 109 51 986 32 WATTS WJ AM REV RESPIR DIS 134 699 986 33 ANDREASSON B ACTA PAEDIATR SCAND 76 70 987 34 FIEL SB CHEST 91 181 987 PN 79235 RN 01215 AN 79200786 AU Lloyd-Still-J-D. Simon-S-H. Wessel-H-U. Gibson-L-E. TI Negative effects of oral fatty acid supplementation on sweat chloride in cystic fibrosis. SO Pediatrics. 1979 Jul. 64(1). P 50-2. MJ CYSTIC-FIBROSIS: me. OILS: pd. SAFFLOWER-OIL: pd. SODIUM-CHLORIDE: an. SWEAT: an. MN ADOLESCENCE. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, dt. FEMALE. HUMAN. INFANT. MALE. PROSTAGLANDINS-E: bl. PROSTAGLANDINS-F: bl. SWEAT: de. AB Essential fatty acid supplementation with oral safflower oil (1 gm/kg/day) to 11 cystic fibrosis patients (aged 6 months to 14 years) for one year produced no significant change in sweat chloride concentration (mEq/liter) or sweat rate (gm/min/m2), Addition of vitamin E (10 mg/kg/day) to the safflower oil had no effect on sweat chloride concentration or rate compared to placebo. No clinical improvement could be detected compared to a control group. These results do not support previous reports of the effects of fatty acid supplementation on sweat electrolyte concentrations in cystic fibrosis. RF 001 ELLIOTT RB ARCH DIS CHILD 50 76 975 002 ELLIOTT RB PEDIATRICS 57 474 976 003 BEVERIDGE J PEDIATRICS 58 465 976 004 ROSENLUND ML PEDIATRICS 59 428 977 005 SHWACHMAN H AM J DIS CHILD 96 6 958 006 GIBSON LE PEDIATRICS 23 545 959 007 SCHWARTZ IL J CLIN INVEST 35 114 956 008 SHWACHMAN H MOD PROBL PEDIATR 10 158 967 009 HOLMAN RT PROC WEST HEM NUTR CONG 5TH 977 010 HUBBARD VS LANCET 2 1302 977 011 DUBOIS RS GASTROENTEROLOGY 72 1052 977 012 LEMEN RJ AM REV RESPIR DIS 117 639 978 013 CHASE HP PEDIATR RES 12 431 978 014 HYMAN AL AM REV RESPIR DIS 117 111 978 CT 1 ROGIERS V PEDIATR RES 14 1088 980 2 LLOYDSTILL JD AM J CLIN NUTR 34 1 981 3 HARPER TB AM REV RESPIR DIS 126 540 982 4 ROGIERS V EUR J PEDIATR 141 39 983 5 ANON J AM COLL TOXICOL 4 171 985 6 GIBSON RA J PEDIATR GASTROENTEROL NUTR 5 408 986 7 MISCHLER EH PEDIATR RES 20 36 986 PN 79236 RN 01216 AN 79179608 AU Beckerman-R-C. Taussig-L-M. TI Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. SO Pediatrics. 1979 Apr. 63(4). P 580-3. MJ ALKALOSIS: et. CYSTIC-FIBROSIS: co. WATER-ELECTROLYTE-IMBALANCE: et. MN CASE-REPORT. CHLORIDES: bl. FEMALE. HUMAN. HYPOKALEMIA: et. HYPONATREMIA: et. INFANT. MALE. AB The records of all children in the Tucson area diagnosed as having cystic fibrosis (CF) before the age of 12 months were reviewed to ascertain the prevalence of metabolic alkalosis as a major presenting manifestation of CF. Five of eleven infants (46%) in whom CF had been diagnosed between 1 and 12 months of age initially were seen with hypokalemia, hypochloremia, and metabolic alkalosis unassociated with marked dehydration, hyperpyrexia, or major pulmonary and/or gastrointestinal symptoms. Two infants had repeated episodes of metabolic alkalosis; for one of these infants, both episodes of metabolic alkalosis occurred before the diagnosis of CF. It is postulated that chronic loss of sweat electrolytes together with mild gastrointestinal or respiratory illness may predispose the infant with cystic fibrosis to a severe electrolyte and acid-base disturbance. The lack of shock and hyperpyrexia together with the apparent chronicity of electrolyte losses differentiates metabolic alkalosis from the heat prostration syndrome, a more acute complication of cystic fibrosis. Quantitative sweat testing should be part of the evaluation of any infant with unexplained metabolic alkalosis. Serum electrolytes should be assessed regularly in infants with cystic fibrosis during hot weather months. RF 001 GOTTLIEB RP J PEDIATR 79 930 971 002 DI SANTAGNESE PA JAMA 172 2014 960 003 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 004 HOCHMAN HI ARCH DIS CHILD 51 390 976 005 SHETH KJ WIS MED J 76/4 S47 977 006 RENDLE-SHORT J ARCH DIS CHILD 31 28 956 007 KESSLER WR PEDIATRICS 8 648 951 008 SIMOPOULOS AP PEDIATR RES 5 626 971 009 MONTALVO JM J CLIN ENDOCRINOL METAB 28 582 968 CT 1 BECKERMAN RC PEDIATRICS 64 389 979 2 NUSSBAUM E AM J DIS CHILD 133 965 979 3 SIMOPOULOS AP NUTR REV 38 201 980 4 GROSSMAN H PEDIATRICS 66 366 980 5 LEBOWITZ MD ENVIRON RES 25 225 981 6 GONZALEZ J PEDIATRICS 67 820 981 7 LAUGHLIN JJ PEDIATRICS 68 203 981 8 RAPAPORT R J PEDIATR 98 768 981 9 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 10 SORSCHER EJ LANCET 1 368 982 11 RUDDY R CLIN PEDIATR 21 367 982 12 FORSYTH JS SCOTT MED J 27 333 982 13 ROSENSTEIN BJ JOHNS HOPKINS MED J 150 113 982 14 ZAMMARCHI E RIV ITAL PEDIATR 9 505 983 15 ORENSTEIN DM PEDIATR RES 17 267 983 16 HILL ID ARCH DIS CHILD 58 224 983 17 CARLILE JR AM J DIS CHILD 137 702 983 18 ORENSTEIN DM J APPL PHYSIOL 57 408 984 19 DAVIS PB SEM RESPIR MED 6 319 985 20 ORENSTEIN DM SEM RESPIR MED 6 252 985 PN 79237 RN 01217 AN 79179561 AU Nezelof-C. LeSec-G. TI Multifocal myocardial necrosis and fibrosis in pancreatic diseases of children. SO Pediatrics. 1979 Mar. 63(3). P 361-8. MJ ENDOMYOCARDIAL-FIBROSIS: pa. MYOCARDIUM: pa. PANCREATIC-DISEASES: pa. MN ACUTE-DISEASE. CYSTIC-FIBROSIS: pa. FEMALE. HEART-FAILURE-CONGESTIVE: pa. HUMAN. INFANT. INFANT-NEWBORN. INTESTINE-SMALL: su. LIPOMATOSIS: pa. MALE. MYOCARDITIS: pa. NECROSIS. PANCREAS: pa. PANCREATIC-NEOPLASMS: pa. PANCREATITIS: pa. POSTOPERATIVE-COMPLICATIONS: pa. AB From a review of 2,000 autopsies of children, 16 cases of extensive necrosis and scarring fibrosis of the myocardium were found. These lesions involved mainly the left ventricle and spared the endocardium, the pericardium, and the coronary vessels. These necrotic of fibrotic heart lesions were found to be closely associated with various pancreatic diseases: cystic fibrosis (11 cases), pancreatic lipomatosis (2 cases), extensive small bowel resection (3 cases, 2 of which were associated with acute interstitial pancreatitis). To explain these unexpected associations, two hypotheses can be put forth: (1) The lack of absorption of some presently undetermined substances indispensable for the correct trophicity of the myocardium, and (2) the release in the blood of proteolytic enzymes with consecutive activation of phlogistic substances such as kinins. RF 001 WISSLER H HELV PAEDIATR ACTA SUPPL 1 1 3 945 002 ESTAGER EM LES LESIONS DE NECROSE MYOCAR 958 003 HERMIER M PEDIATRIE 22 931 967 004 KINTZEN W INT Z VITAMINFORSCH 22 273 950 005 LINHARTOVA A ZENTRALBL ALLG PATHOL 103 405 962 006 MOUY A ARCH FR PEDIATR 25 687 968 007 FARBER S ARCH PATHOL 37 238 944 008 NEZELOF C ARCH FR PEDIATR 16 1035 959 009 OPPENHEIMER EH JOHNS HOPKINS MED J 133 252 973 010 SHWACHMAN H N ENGL J MED 281 500 969 012 RICOUR C ARCH FR PEDIATR 30 469 973 013 RICOUR C ARCH FR PEDIATR 34 154 977 014 GALLOIS B THESIS 973 015 MCGIVEN AR ARCH DIS CHILD 37 656 962 016 OPPERMANN A ARCH FR PEDIATR 17 1339 960 017 CLAVER M PATHOL EUR 7 69 974 018 BURDZINSKA J KARDIOL POL 7 233 964 019 KUTOR J GYOGYSZERESZET 17 286 966 020 DEGARCIA DADONI CR ARCH ARGENT PEDIATR 63 188 965 021 POWELL LW JR VA MED 84 178 957 022 GIRON B MED INFANT 7 747 976 023 OPPENHEIMER EH CYSTIC FIBROSIS 15 21 975 024 PICANDET B MED INFANT 7 757 976 025 NEZELOF C ARCH FR PEDIATR 18 1135 961 026 BODIAN M ACTA PAEDIATR SCAND 53 282 964 027 SACREZ R ANN PEDIATR (PARIS) 16 43 969 028 SHWACHMAN H J PEDIATR 65 645 964 029 ARGOSTI B MED INFANT 8 822 976 030 MOZZICONACCI P IN: FLAMMARION M 966 031 GODEAU P SEM HOP PARIS 51 2383 975 032 LEVY A PRESSE MED 73 1345 966 033 BLUMENSTOCK DA PEDIATRICS 19 1002 957 034 HENDREN WH ARCH DIS CHILD 40 132 965 035 RIEMENSCHNEIDER TA PEDIATRICS 41 428 968 036 STRICKLER GB AM J DIS CHILD 95 206 958 037 COHEN MI PEDIATR RES 7 106 973 038 GUPTA MC GUT 14 438 973 039 DESCHAMPS JP MED INFANT 1 27 973 040 CLAIREAUX AE ARCH DIS CHILD 31 22 956 041 BLANC WA PEDIATRICS 22 494 958 042 DESJEUX JF ANN MED INTERNE PARIS 127 353 976 043 FEER W SCHWEIZ MED WOCHENSCHR 82 101 952 044 HARIDAS G ARCH DIS CHILD 27 23 947 045 HEGGTVEIT HA AM J PATHOL 45 757 964 046 WARTER J PRESSE MED 70 255 962 047 WERLE E MUNCH MED WOCHENSCHR 115 225 973 048 DOS REIS L ARCH SURG 87 604 963 049 SPOCK A PEDIATR RES 1 173 967 050 CONOVER JH LIFE SCI 14 253 974 051 LESEC G NOUV PRESSE MED 5 2470 976 052 SHWACHMAN H PEDIATRICS 55 86 975 CT 1 BACHMAN DS PEDIATRICS 64 388 979 2 ZIMMERMANN A HELV PAEDIATR ACTA 37 183 982 3 GUESNU M ARCH FR PEDIATR 39 127 982 4 MOSS AJ PEDIATRICS 70 728 982 5 ROSSI E MONATSSCHR KINDERHEILKD 130 314 982 6 CHERON G ACTA PAEDIATR SCAND 73 697 984 7 SAVILAHTI E ACTA PAEDIATR SCAND 73 642 984 8 MICHAEL JR AM REV RESPIR DIS 130 516 984 9 SULLIVAN MM CHEST 90 239 986 PN 79238 RN 01218 AN 79200748 AU Phelps-D-L. TI Vitamin E: where do we stand?. SO Pediatrics. 1979 Jun. 63(6). P 933-5. MJ CHILD-NUTRITION. INFANT-NUTRITION-DISORDERS: dt. VITAMIN-E-DEFICIENCY: dt. VITAMIN-E: tu. MN ADMINISTRATION-ORAL. ANEMIA-HEMOLYTIC: dt. BILIARY-TRACT: ab. BRONCHI: ab. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: bl, co. HUMAN. INFANT. INFANT-PREMATURE-DISEASES: dt. LUNG: ab. MALABSORPTION-SYNDROMES: dt. RETROLENTAL-FIBROPLASIA: dt. VITAMIN-E-DEFICIENCY: et. VITAMIN-E: ad, ae, ph. EX I propose that clinicians should have a general understanding of what little physiology is known about tocopherol, be able to recognize the circumstances that cause low tocopherol levels, understand when to provide physiologic replacement therapy, and accept the responsibility for protecting their patients from uncontrolled experimental use of tocopherols. Tocopherol physiology, tocopherol pharmacology, tocopherol deficiency, therapy in deficiency states, and pharmacological doses of tocopherol are discussed. Patients with prolonged fat malabsorption and some premature infants receiving iron must be given vitamin E. Use of tocopherol therapy in bronchopulmonary dysplasia and retrolental fibroplasia, while promising, is unproven and should be used only in a controlled study. Research on the role of vitamin E in human physiology is entering a rapid-growth phase. RF 001 MELHORN DK OHIO STATE MED J 69 899 973 002 OSKI FA HOSP PRACT 12 79 977 003 BIERI JG VITAM HORM 34 31 976 005 DORMANDY TL LANCET 1 647 978 007 HORWITT MK AM J CLIN NUTR 4 408 956 008 OSKI FA J PEDIATR 70 211 967 009 RITCHIE JH N ENGL J MED 279 1185 968 010 ROSENBLUM JL PEDIATR RES 12 555 978 011 HILLMAN RW AM J CLIN NUTR 5 597 957 012 CORRIGAN JJ JR JAMA 230 1300 974 014 EHRLICH HP ANN SURG 175 235 972 015 LEVY L INFLAMMATION 1 333 976 016 EHRENKRANZ RA N ENGL J MED 299 564 978 017 NORTHWAY WH JR N ENGL J MED 299 599 978 018 PHELPS DL PEDIATRICS 59 998 977 019 JOHNSON L AM J CLIN NUTR 27 1158 974 CT 1 HIGASHI A PEDIATR PHARMACOL 1 129 980 2 LEBOULANGER J REV PRAT 31 69 981 3 BELL EF AM J CLIN NUTR 34 414 981 4 HAGA P EUR J PEDIATR 136 143 981 5 LLOYD BW NEUROPEDIATRICS 13 155 982 6 ANDERSON SA AM J CLIN NUTR 35 381 982 7 GUGGENHEIM MA J PEDIATR 100 51 982 8 WILLE L KLIN PAEDIATR 194 1 982 9 ROY CC J PEDIATR GASTROENTEROL NUTR 2 S282 983 10 GREGORY JG SCIENTOMETRICS 6 307 984 11 VAISMAN N NUTR RES 5 931 985 12 BOHLES H KLIN PAEDIATR 197 386 985 13 CONWAY SP BR J NUTR 56 105 986 PN 79239 RN 01219 AN 80012602 AU Beckerman-R-C. TI Metabolic alkalosis in infants with cystic fibrosis [letter]. SO Pediatrics. 1979 Sep. 64(3). P 389. MJ ALKALOSIS: et. CYSTIC-FIBROSIS: co. MN CARBON-DIOXIDE: bl. CHLORIDES: an. CYSTIC-FIBROSIS: di. ELECTROLYTES: bl. FEMALE. HUMAN. INFANT. MALE. SWEAT: ah. EX I have diagnosed six cystic fibrosis infants under 1 year of age, four of whom had documented episodes of hypoelectrolytemia and severe metabolic alkalosis. These additional data support our previous hypothesis that chronic sweat electrolyte depletion, more frequently than previously recognized, can cause severe metabolic derangements in infants with cystic fibrosis, especially those living in both humid and dry, hot climates. RF 001 BECKERMAN RC PEDIATRICS 63 580 979 002 DI SANTAGNESE PA JAMA 172 2014 960 003 GOTTLIEB RP J PEDIATR 79 930 971 004 NOUSIA-ARVANITAKIS S J PEDIATR 82 535 973 005 SHETH KJ WIS MED J 76/4 S47 977 CT 1 RAPAPORT R J PEDIATR 98 768 981 2 FORSYTH JS SCOTT MED J 27 333 982 PN 79240 RN 01220 AN 80146088 AU Pryor-J-A. Webber-B-A. TI An evaluation of the forced expiration technique as an adjunct to postural drainage. SO Physiotherapy. 1979 Oct. 65(10). P 304-7. MJ BRONCHIAL-SPASM: th. CYSTIC-FIBROSIS: th. RESPIRATORY-THERAPY: mt. MN ADOLESCENCE. ADULT. FEMALE. HUMAN. MALE. EX A criticism often levelled at the forced expiration technique (FET) is that it increases and may even produce bronchospasm. It was our impression that the use of forced expiration when combined with periods of relaxed diaphragmatic breathing did not aggravate bronchospasm and shortened the postural drainage session. Two groups of patients were studied: 24 with cystic fibrosis to evaluate the use of the FET as an independent means of postural drainage; and 20 asthmatic patients to assess the effect of the FET on bronchospasm. The studies indicate that the forced expiration technique increases the efficiency of postural drainage, but does not increase airway obstruction. This technique can be applied to both medical and surgical conditions when postural drainage, or a modified form of postural drainage, is indicated. RF 001 ANON THORAX 14 286 959 002 COCHRANE GM BR MED J 2 1181 977 003 COTES JE LUNG FUNCTION 975 004 GASKELL DV BROMPTON HOSP GUIDE TO CHEST 977 006 MEAD J J APPL PHYSIOL 22 95 967 007 PRYOR JA BR MED J 2 417 979 009 THOMPSON BJ NZ J PHYSIOTHERAPY 4 4 973 010 THOMPSON BJ ASTHMA AND YOUR CHILD 978 CT 1 VANDERMEER JWM REV INFECT DIS 6 107 984 2 BATTISTINI A RIV ITAL PEDIATR 10 220 984 3 RIVINGTONLAW BA CHEST 85 378 984 4 OBERWALDNER B PEDIATR PULMONOL 2 358 986 5 HOFMEYR JL THORAX 41 951 986 6 VERBOON JML EUR J RESPIR DIS 69 169 986 7 WEBBER BA BR J DIS CHEST 80 353 986 PN 79241 RN 01221 AN 79180081 AU Martodam-R-R. Baugh-R-J. Twumasi-D-Y. Liener-I-E. TI A rapid procedure for the large scale purification of elastase and cathepsin G from human sputum. SO Prep-Biochem. 1979. 9(1). P 15-31. MJ CATHEPSINS: ip. CYSTIC-FIBROSIS: en. PANCREATOPEPTIDASE: ip. SPUTUM: en. MN AMINO-ACID-SEQUENCE. BINDING-SITES. CHROMATOGRAPHY-AFFINITY: mt. CHROMATOGRAPHY-GEL: mt. DEOXYRIBONUCLEASES. ELECTROPHORESIS-DISC. HUMAN. LEUKOCYTES: en. SUPPURATION. SUPPORT-U-S-GOVT-P-H-S. AB A procedure is described which permits the rapid isolation of large amounts of elastase and cathepsin G from purulent sputum. This procedure involves: (1) digestion of sputum with DNase, (2) extraction of the insoluble residue that remains with 1 M NaCl, pH 8, (3) affinity chromatography on Sepharose-bound Trasylol, and (4) separation of the two enzymes by chromatogrphy on CM-Sephadex. Starting with 500 g of sputum it was possible to isolate 175 mg of each of these two enzymes within 7 to 10 days. Active site titration indicated both enzymes to be at least 97% pure. Disc gel electrophoresis in the presence and absence of SDS and amino acid sequence of the N-terminal region support the conclusion that the elastase and cathepsin G isolated from sputum are identical to the same enzymes isolated directly from the leukocytes of human blood. RF 001 JANOFF A AM J PATHOL 68 579 972 002 LAZARUS GS AM J PATHOL 68 565 972 003 STARKEY PM IN: BARRETT AJ 57 977 004 TWUMASI DY J BIOL CHEM 252 1917 977 005 BAUGH RJ BIOCHEMISTRY 15 836 976 006 BIETH J BIOCHEM MED 11 350 974 007 VISSER L BIOCHIM BIOPHYS ACTA 268 257 972 008 POWERS JC BIOCHEM BIOPHYS RES COMMUN 67 639 975 010 CHASE T JR BIOCHEM BIOPHYS RES COMMUN 29 508 967 011 BREWER JM J CHEM EDUC 46 41 969 012 WEBER K J BIOL CHEM 244 4406 969 013 ANON BECKMAN INSTRUCTION MANUAL 974 014 SMITHIES O BIOCHEMISTRY 10 4912 971 015$ WARBURG O BIOCHEM Z 310 384 942 016 JANOFF A LAB INVEST 29 458 973 017 SCHMIDT W PHYSIOL CHEM 355 1077 974 018 OHLSSON K EUR J BIOCHEM 42 519 974 019 TAYLOR JC ARCH BIOCHEM BIOPHYS 169 91 975 020 DRUZE D BIOCHIM BIOPHYS ACTA 438 505 976 021 FEINSTEIN G BIOCHIM BIOPHYS ACTA 403 477 975 022 RINDLER-LUDWIG R BIOCHIM BIOPHYS ACTA 379 606 975 023 STARKEY PM BIOCHEM J 155 255 976 024 STARKEY PM BIOCHEM J 155 265 976 025 STARKEY PM BIOCHEM J 155 273 976 CT 1 SENIOR RM J CLIN INVEST 66 859 980 2 MCDONALD JA J BIOL CHEM 255 8848 980 3 BOUDIER C ARCH BIOCHEM BIOPHYS 210 790 981 4 BOUDIER C J BIOL CHEM 256 256 981 5 MARTODAM RR BIOCHIM BIOPHYS ACTA 667 328 981 6 BAICI A SCAND J IMMUNOL 16 487 982 7 BAICI A MOL IMMUNOL 19 719 982 8 BOUDIER C BIOCHEM MED 28 41 982 9 CAMPBELL EJ J CLIN INVEST 70 845 982 10 CAMPBELL EJ PROC NAT ACAD SCI USA 79 6941 982 11 LAINE A BIOCHEM BIOPHYS RES COMMUN 105 186 982 12 LAINE A BIOCHEM BIOPHYS RES COMMUN 107 337 982 13 BROWER MS J BIOL CHEM 257 9849 982 14 VISCARELLO BR PREPAR BIOCHEM 13 57 983 15 STERRENBERG L THROMB RES 31 719 983 16 ANDREWS JL CHEMBIOL INTERACT 47 157 983 17 STONE PJ CHEST 83 S 65 983 18 CAMPBELL EJ J LAB CLIN MED 101 527 983 19 CAMPBELL EJ AM REV RESPIR DIS 127 631 983 20 MCGOWAN SE AM REV RESPIR DIS 128 688 983 21 STONE PJ ANN NY ACAD SCI 421 398 983 22 SNIDER GL AM REV RESPIR DIS 129 155 984 23 MCGOWAN SE AM REV RESPIR DIS 130 734 984 24 SENIOR RM J IMMUNOL 132 2547 984 25 BOUDIER C ADV EXP MED BIOL 167 313 984 26 MIKUNITAKAGAKI Y J BIOL CHEM 259 6739 984 27 BREUER R EXP LUNG RES 9 167 985 28 LENTINI A BIOCHEM INT 10 221 985 29 STERRENBERG L HAEMOSTASIS 15 126 985 30 STEIN RL ANNU REP MED CHEM 20 237 985 31 BINDER R RESPIRATION 47 267 985 32 JACQUOT J INFECT IMMUN 47 555 985 33 DORING G INFECT IMMUN 49 557 985 34 BREUER R J LAB CLIN MED 105 635 985 35 LAURENT P BIOCHEM BIOPHYS RES COMMUN 126 275 985 36 BRUCE MC AM REV RESPIR DIS 132 529 985 37 LUCEY EC AM REV RESPIR DIS 132 362 985 38 SNIDER GL AM REV RESPIR DIS 132 1155 985 39 TOURNIER JM AM REV RESPIR DIS 132 524 985 40 SMITH CE BIOCHEM J 225 463 985 41 BALDUYCK M BIOL CHEM HOPPE SEYLER 366 9 985 42 BALDUYCK M BIOL CHEM HOPPE SEYLER 366 9 985 43 VERCAIGNEMARKO D BIOL CHEM HOPPE SEYLER 366 655 985 44 DORING G CLIN EXP IMMUNOL 64 597 986 45 WELGUS HG J CLIN INVEST 77 1675 986 46 NILES RM J LAB CLIN MED 108 489 986 47 GOLDSTEIN W AM REV RESPIR DIS 134 49 986 48 LUCEY EC AM REV RESPIR DIS 134 471 986 49 HECK LW ANAL BIOCHEM 158 217 986 50 SKINNER M PROC SOC EXP BIOL MED 181 211 986 51 BRUCH M BIOCHEM J 238 269 986 52 BERMAN G J BIOL CHEM 261 4095 986 53 JOHNSON DA J BIOL CHEM 261 4748 986 54 LAURENT P BIOCHEM PHARMACOL 36 765 987 55 PETERSON MW J APPL PHYSIOL 62 1521 987 56 BROWER MS BLOOD 69 813 987 57 MORRISON HM CLIN SCI 72 151 987 58 BOUDIER C ARCH BIOCHEM BIOPHYS 253 439 987 59 SMITH CE ARCH BIOCHEM BIOPHYS 253 146 987 60 SELLOUM L BIOL CHEM HOPPE SEYLER 368 47 987 PN 79242 RN 01222 AN 79223914 AU Shapiro-B-L. Feigal-R-J. Lam-L-F. TI Mitrochondrial NADH dehydrogenase in cystic fibrosis. SO Proc-Natl-Acad-Sci-USA. 1979 Jun. 76(6). P 2979-83. MJ CYSTIC-FIBROSIS: en. MITOCHONDRIA: en. NADH-NADPH-OXIDOREDUCTASES: me. SKIN: en. MN CELLS-CULTURED. FIBROBLASTS: en. HUMAN. HYDROGEN-ION-CONCENTRATION. KINETICS. OXYGEN-CONSUMPTION. AB We have shown that skin fibroblast from patients with cystic fibrosis (CF) and from carriers for CF [heterozygotes (HZ)] consume more O2 than do their controls. When the mitochondrial electron transport inhibitor rotenone was added to the cells, the relative inhibition of O2 consumption was CF greater than HZ greater than controls (P less than 0.005 in both comparisons). Because rotenone specifically inhibits NADH dehydrogenase, [NADH: (acceptor) oxidoreductase, EC 1.6.99.3], which is the enzyme of energy-conserving site 1 of the mitochondrial electron transport system, activity and kinetics of this enzyme system were studied in fibroblast homogenates. NADH dehydrogenase activity was equal in cells from the three genotypes. At pH 8.0, affinity of the enzyme for its substrate was CF greater than HZ = controls; at pH 8.6, affinity was CF greater than HZ = controls (P less than 0.005 for the differences). pH optima for the genotypes were without exception 8.6 (CF), 8.3 (HZ), and 8.0 (control). HZ and control lines were distinguished unequivocally in a blind test on the basis of differences in pH optima. Purified mitochondrial preparations revealed pH optima identical to those found in whole cell homogenates. These data suggest that the mutant gene responsible for CF is expressed in the complex mitochondrial NADH dehydrogenase system. RF 001 NADLER HL IN: STANBURY JB 1683 978 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 DI SANTAGNESE PA N ENGL J MED 295 534 976 004 DI SANTAGNESE PA N ENGL J MED 295 597 976 005 PALADE GE SCIENCE 189 347 975 006 SHAPIRO BL GAP CONF REP TISS CULT APPR 977 007 SHAPIRO BL SCIENCE 203 1251 979 008 SHAPIRO BL CLIN CHIM ACTA 82 125 978 009 FEIGAL RJ PEDIATR RES 13 764 979 010 FEIGAL RJ NATURE 278 276 979 011 MITCHELL P BIOL REV 41 445 966 012 BYGRAVE FL BIOL REV 53 43 978 013 FEIGAL RJ DISSERTATION 979 014 SLATER EC METHODS ENZYMOL 10 48 967 015 LOWRY OH J BIOL CHEM 193 265 951 016 KING TE METHODS ENZYMOL 10 275 967 017 STANBURY JB METABOLIC BASIS OF INHERITED 978 018 SINGER TP METHODS ENZYMOL 53 397 978 019 HATEFI Y IN: BOYER PD 13 175 976 020 RAGAN CI BIOCHIM BIOPHYS ACTA 456 249 976 021 HATEFI Y METHODS ENZYMOL 53 11 976 022 FORSTNER JF PEDIATR RES 10 609 976 023 CARAFOLI E ANN NY ACAD SCI 307 269 978 024 LEHNINGER AL ANN NY ACAD SCI 307 160 978 025 WALLACH D EUR J BIOCHEM 21 433 971 026 ARGENT BE J PHYSIOL (LOND) 230 575 973 027 MANGOS JA IN: ANDREOLI TE 978 CT 1 DAVIS PB PEDIATR RES 14 83 980 2 SEALE TW ANN CLIN LAB SCI 12 415 982 3 SANGUINETTIBRICENO NR CLIN GENET 22 308 982 4 FEIGAL RJ LIFE SCI 30 93 982 5 SHAPIRO BL AM J HUM GENET 34 846 982 6 BOYCE JR INFECT IMMUN 37 695 982 7 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 8 SEYMOUR CA BIOESSAYS 1 38 984 9 REGELMANN WE PEDIATR RES 20 619 986 10 STUTTS MJ PEDIATR RES 20 1316 986 11 KUZEMKO JA J ROY SOC MED 79 2 986 12 BATTINO M FEBS LETTERS 199 155 986 13 FEIGAL RJ ANN NY ACAD SCI 488 82 986 PN 79243 RN 01223 AN 80122681 AU Mangos-J-A. TI Cystic fibrosis: potential management through manipulation of the secretory process in affected exocrine glands. SO Prog-Clin-Biol-Res. 1979. 34. P 111-7. MJ CYSTIC-FIBROSIS: th. EXOCRINE-GLANDS: se. MN ANIMAL. CATS. CYSTIC-FIBROSIS: pp. EXOCRINE-GLANDS: de. FERRETS. ISOPROTERENOL: pd. MACROMOLECULAR-SYSTEMS. MUCUS: se. PAROTID-GLAND: de. RATS. SALIVA: se. SODIUM: se. TRACHEA: de. WATER-ELECTROLYTE-BALANCE. EX Ten years ago, we demonstrated that chronic administration of isoproterenol to rats results is structural and functional changes of their salivary glands similar to those seen in exocrine glands of patients with cystic fibrosis. Recently, we began to study the cellular pathophysiology of these glands and managed to achieve pharmacological control of this abnormality. We demonstrated that administration of a beta-adrenergic antagonist in these animals resulted in the correction of the electrolyte abnormality which is very similar to that seen in the sweat glands of patients with cystic fibrosis. These two examples of manipulation of the secretory processes in exocrine glands of experimental animals demonstrate the feasibility of this approach in the therapy of patients with cystic fibrosis. RF 001 MANGOS JA IN: ANDREOLI TE 978 002 MANGOS JA J DENT RES 57 989 978 004 MANGOS JA CF PROJECTIONS INTO THE FUTUR 976 007 MANGOS JA AM J PHYSIOL 225 683 973 008 MANGOS JA AM J PHYSIOL 229 553 975 009 MANGOS JA AM J PHYSIOL 229 560 975 010 MANGOS JA AM J PHYSIOL 229 566 975 011 MANGOS JA IN: LAWSON D PROC 5TH INT CF 25 969 012 NADEL JA CIBA FOUND SYMP 54 978 013 STURGESS JM BR J EXP PATHOL 54 388 973 PN 79244 RN 01224 AN 80122682 AU Gluckson-M-M. Denning-C-R. TI Changing patterns of genetic counseling in a cystic fibrosis center. SO Prog-Clin-Biol-Res. 1979. 34. P 119-32. MJ CYSTIC-FIBROSIS: fg. GENETIC-COUNSELING. MN ABORTION-LEGAL: px. ADULT. ATTITUDE-TO-HEALTH. CYSTIC-FIBROSIS: px. FAMILY-PLANNING. FEMALE. HETEROZYGOTE-DETECTION. HUMAN. MALE. PRENATAL-DIAGNOSIS. RELIGION. RISK. EX The change of life expectancy of cystic fibrosis patients has stimulated our interest in assessing the understanding and utilization of genetic information by adult CF patients and how this may affect their coping mechanism(s). The present study was designed to obtain information in these areas with the expectation that more effective genetic counseling might be developed for this enlarging group of patients. Knowledge of genetics, attitudes concerning reproduction, attitudes toward prenatal diagnosis, and attitudes toward a carrier identification study are reported for 25 males and 15 females with proven cystic fibrosis. RF 001 BOYLE IR J PEDIATR 88 318 976 002 CARTER CO LANCET 1 281 971 003 ANON GAP CONF REP PROB REPRO PHYSI 975 004 DANKS DM ANN HUM GENET 28 323 965 005 DENNING CR IN: MANGOS JA 127 976 006 DENNING CR PEDIATRICS 41 7 968 007 DODGE JA PATIENT COUNS HEALTH EDUC 1 8 978 008 EMERY AEH BR MED J 1 724 973 009 GLUCKSON MM CF CLUB ABST 18 977 010 KABACK MM PROG MED GENET 10 103 974 011 LEONARD CO N ENGL J MED 287 433 972 012 MERRITT AD J LAB CLIN MED 60 998 962 013 MOHR I CF CLUB ABST 16 975 014 SHAW MW IN: LUBS HA 977 015 SIBINGA MS PEDIATR RES 6 331 972 016 STEINHAUER PD PEDIATR CLIN NORTH AM 21 825 974 CT 1 PASSARGE E EUR J PEDIATR 143 54 984 PN 79245 RN 01225 AN 80080576 TI New test to detect carriers of gene for cystic fibrosis [news]. SO Public-Health-Rep. 1979 Nov-Dec. 94(6). P 577. MJ CYSTIC-FIBROSIS: di. HETEROZYGOTE-DETECTION: mt. MN CYSTIC-FIBROSIS: fg. HUMAN. EX Development of a new test to detect carriers of the gene for cystic fibrosis was announced. The test involves the interaction between red blood cells from a mouse and a small amount of a person's blood serum. The gene for cystic fibrosis is associated with a lectin, a factor in the person's blood serum that acts to agglutinate (or cluster) the red blood cells of the mouse. PN 79246 RN 01226 AN 81015715 AU Swan-H-A. Chappell-J-S. TI Cystic fibrosis--its surgical presentation. SO S-Afr-J-Surg. 1979 Jun. 17(2). P 51-7. MJ CYSTIC-FIBROSIS: co. MN ADOLESCENCE. ADULT. BILIARY-TRACT-DISEASES: su. CHILD-PRESCHOOL. GASTROINTESTINAL-DISEASES: su. HUMAN. LIVER-DISEASES: su. MALE. PANCREATIC-DISEASES: su. RESPIRATORY-TRACT-DISEASES: su. AB In a series of 62 cases of cystic fibrosis presenting at the Transvaal Memorial Hospital for Children, Johannesburg, during the 10-year period 1968-1978, 15 patients required surgical treatment for 17 complications of the disease. Meconium ileus, rectal prolapse, intussusception and ethmoiditis were the commonest surgical presentations, and portal hypertension, bronchial obstruction and nasal polyposis were also experienced. As the basic defect is unknown, treatment today is still directed towards these usually unpredictable secondary consequences. RF 001 DI SANTAGNESE PA N ENGL J MED 277 1287 967 002 PROSSER R ARCH DIS CHILD 49 597 974 003 DANKS DM ANN HUM GENET 28 323 965 004 MCPARTLIN JF ARCH DIS CHILD 47 207 972 005 ANDERSON CM CF A MANUAL OF DIAGNOSIS AND 976 006 KOPEL FB GASTROENTEROLOGY 62 483 972 007 BERNSTEIN JG AM J DIS CHILD 99 804 960 008 GRACEY M IN: ANDERSON CM 329 359 975 009 OPPENHEIMER EH ARCH PATHOL 96 149 973 010 LLOYD-STILL JD BR MED J 1 110 971 011 ALTERMAN K AM J DIS CHILD 101 210 961 012 BOAT TF JAMA 209 1498 969 013 TAYLOR BW ARCH DIS CHILD 49 133 974 014 WANG CI AM J DIS CHILD 119 236 970 015 OPPENHEIMER EH PERSPECT PEDIATR PATHOL 2 241 975 016 KISSANE JM PATHOLOGY OF INFANCY AND CHIL 967 017 GRAND RJ CLIN PEDIATR 9 588 970 018 SHWACHMAN H PEDIATRICS 55 86 975 019 KOMP DM CHEST 58 501 970 020 ESTERLY JR J PEDIATR 69 3 966 021 CRAIG JM AM J DIS CHILD 93 357 957 022 TAYLOR WF AM J DIS CHILD 123 161 972 023 HUANG NN AM J DIS CHILD 120 289 970 024 DI SANTAGNESE PA N ENGL J MED 279 103 968 PN 79247 RN 01227 AN 80103180 AU Friis-B. TI Chemotherapy of chronic infections with mucoid Pseudomonas aeruginosa in lower airways of patients with cystic fibrosis. SO Scand-J-Infect-Dis. 1979. 11(3). P 211-7. MJ ANTIBIOTICS: tu. CYSTIC-FIBROSIS: co. PNEUMONIA: dt. PSEUDOMONAS-INFECTIONS: dt. MN ADOLESCENCE. ADULT. CARBENICILLIN: tu. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. COLISTIN: tu. DRUG-EVALUATION. DRUG-THERAPY-COMBINATION. FEMALE. GENTAMICINS: tu. HUMAN. INFANT. MALE. PNEUMONIA: co. PRECIPITINS: an. PSEUDOMONAS-AERUGINOSA: im. PSEUDOMONAS-INFECTIONS: co. TOBRAMYCIN: tu. AB The bacteriological effect of chemotherapy against Pseudomonas aeruginosa (Ps.ae.) in lungs of patients with cystic fibrosis is reviewed. During a 5-year period 49 children and adults were treated with 190 courses of different antibiotics. The mucoid strains of Ps.ae. disappeared in 72.0% of the courses in which a combination of tobramycin and carbenicillin was employed. Tobramycin given alone had only bacteriological effect in 26.6% of the courses. Colimycin alone or in combination with carbenicillin had no effect. In 18 patients who received subsequent courses of tobramycin and combination of tobramycin and carbenicillin a significant difference in favour of the combination therapy was found, also in cases with many precipitins against Ps.ae. in serum. In 74.5% of the initially successful courses the patients were recolonized with Ps.ae. within 1 month. No nephrotoxic or ototoxic side effects were demonstrated in spite of the high doses of tobramycin (10 mg/kg/24 h) emmployed and the repeated courses. RF 001 ANDERSON EL ANTIMICROB AGENTS CHEMOTHER 8 300 975 002 DAVIES J J INFECT DIS SUPPL 124 7 971 003 GIBSON LE PEDIATRICS 23 545 959 004 HAWLEY HB CURR THER RES 16 414 974 005 HOFF GE SCAND J INFECT DIS 6 333 974 006 HUANG NN J PEDIATR 78 338 971 007 HOIBY N ACTA PATH MICROBIOL SCAND (B) 81 298 973 008 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 541 974 009 HOIBY N ACTA PATH MICROBIOL SCAND (B) 82 551 974 010 HOIBY N SCAND J RESPIR DIS 58 65 977 011 KLASTERSKY J AM J MED SCI 266 13 973 012 KLUGE RM ANN INTERN MED 81 584 974 013 KLUGE RM ANTIMICROB AGENTS CHEMOTHER 6 442 974 014 MARKS MI J PEDIATR 79 822 971 015 MCCRAE WM SCOTT MED J 21 68 976 016 MEYER RD APPL MICROBIOL 22 1147 971 017 MEYERS BR J CLIN PHARMACOL 12 321 972 CT 1 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 2 FEIGELSON J NOUV PRESSE MED 10 955 981 3 MALMBORG AS SCAND J INFECT DIS 1981 64 981 4 MOLLER NE SCAND J INFECT DIS 1981 87 981 5 DAVIDSON S ISR J MED SCI 18 859 982 6 KELLY HW J PEDIATR 101 793 982 7 BECK B ACTA PAEDIATR SCAND SUPPL 301 1982 125 982 8 HOIBY N ACTA PAEDIATR SCAND SUPPL 301 1982 75 982 9 PERMIN H J ANTIMICROB CHEMOTHER 12 313 983 10 HEILESEN AM SCAND J INFECT DIS 15 271 983 11 DORING G INFECT IMMUN 42 197 983 12 SCHIOTZ PO ACTA PAEDIATR SCAND 72 283 983 13 SZAFF M ACTA PAEDIATR SCAND 72 651 983 14 DRUSANO GL REV INFECT DIS 6 13 984 15 KELLY HW DRUG INTEL CLIN PHARM 18 772 984 16 FRASER GL DRUG INTEL CLIN PHARM 19 757 985 17 STACK BHR THORAX 40 358 985 18 STOUT SA DRUG INTEL CLIN PHARM 21 322 987 PN 79248 RN 01228 AN 79181690 AU Gawel-J. Rudnik-J. Pryjma-J. Zebrak-J. Hauszka-J. Rudnik-I. Majewska-Zalewska-H. TI Proteins in bronchial secretion of children with chronic pulmonary diseases. I. Relation to clinical diagnosis. SO Scand-J-Respir-Dis. 1979 Apr. 60(2). P 63-8. MJ BRONCHI: se. IGA: se. IGA-SECRETORY. IGG: se. LACTOFERRIN: se. LACTOGLOBULINS: se. MURAMIDASE: se. RESPIRATORY-TRACT-DISEASES: di. MN ADOLESCENCE. BRONCHI: im. BRONCHIECTASIS: di, im. BRONCHITIS: di, im. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CYSTIC-FIBROSIS: di, im. HUMAN. IGA: an. IGA-SECRETORY: an. IGG: an. LACTOFERRIN: an. MURAMIDASE: an. RECURRENCE. RESPIRATORY-TRACT-DISEASES: im. RESPIRATORY-TRACT-INFECTIONS: di, im. AB Bronchial secretions from 207 children suffering from various pulmonary diseases and from 15 healthy controls were tested concentration of IgA, IgG, lactoferrin and lysozyme. The results obtained suggest that in many cases of chronic lung diseases in children the levels of lactoferrin and immunoglobulins, especially secretory IgA, are very low. In severe infections (cystic fibrosis, bronchiectases) significant increase of IgG concentration was observed. RF 001$ ADINOLFI M IMMUNOLOGY 10 517 966 002 ALFORD RH J IMMUNOL 101 984 968 003 BISERTE G EXP ANNU BIOCHIM MED 24 85 963 004 BRUZDZINSKI H MED DOSW MIKROBIOL 28 377 976 005 BULLEN GJ BR J HAEMATOL 23 289 972 006 DEUSCHL H CLIN EXP IMMUNOL 16 401 973 007 FALK GA AM REV RESPIR DIS 105 14 972 008 FLEMMING A PROC R SOC LOND BIOL 93 306 922 009 HEISE ER J RETICULOENDOTHEL SOC 4 510 967 010 HILL IR IMMUNOLOGY 26 1239 974 011 KALTREIDER HB J IMMUNOL 116 423 976 012 KOBAYASHI K IMMUNOCHEMISTRY 8 785 971 013 LICHT W PNEUMONOLOGY 144 139 971 014 LOWRY OH J BIOL CHEM 193 265 951 015 MANCINI G IMMUNOCHEMISTRY 2 235 965 016 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 017 MASSON PL IN: DULFANO MJ 973 018 MCINTOSH K J ALLERGY CLIN IMMUNOL 57 595 976 019 MEDICI TC AM REV RESPIR DIS 103 784 971 020 MESTECKY J SCIENCE 171 1163 971 021 NEWHOUSE M N ENGL J MED 295 1045 976 022 SIEGLER DIM THORAX 29 313 974 023 SOOTHILL JF IN: BRENT L 5 974 024 SOUTAR CA THORAX 30 239 975 025 SZOTOWA W CZYNNE PORADNICTWO W OPIECE Z 976 026 THACORE H AM REV RESPIR DIS 93 786 966 027 TISCHENDORF FW IN: ENGELHARDT A 1 974 028 WALDMAN RH IN: NETER E 334 975 029 ZEBRAK J SCAND J RESPIR DIS 60 69 979 CT 1 ZEBRAK J SCAND J RESPIR DIS 60 69 979 2 SCHIOTZ PO ACTA PATH MICROBIOL SCAND (C) S276 1 981 3 GOLDSTEIN E REV INFECT DIS 5 1078 983 4 JACQUOT J BULL EUR PHYSIOPATH RESP 19 453 983 5 MOSS RB MONOGR ALLERGY 19 202 986 6 DVORETSKY LI TER ARKH 58 72 986 7 DVORETSKY LI KLIN MED 65 79 987 8 MOSS RB CHEST 91 522 987 PN 79249 RN 01229 AN 79181691 AU Zebrak-J. Herman-T. Werys-R. Pryjma-J. Gawel-J. TI Proteins in bronchial secretion of children with chronic pulmonary diseases. II. Relation to bronchoscopic and bronchographic examination. SO Scand-J-Respir-Dis. 1979 Apr. 60(2). P 69-75. MJ BRONCHI: se. IGA: se. IGA-SECRETORY. IGG: se. LACTOFERRIN: se. LACTOGLOBULINS: se. MURAMIDASE: se. RESPIRATORY-TRACT-DISEASES: di. MN ADOLESCENCE. BRONCHI: im. BRONCHIECTASIS: di, im. BRONCHITIS: di, im. BRONCHOGRAPHY. BRONCHOSCOPY. CHILD. CHILD-PRESCHOOL. CHRONIC-DISEASE. CYSTIC-FIBROSIS: di, im. HUMAN. IGA: an. IGA-SECRETORY: an. IGG: an. LACTOFERRIN: an. MURAMIDASE: an. RESPIRATORY-TRACT-DISEASES: im. AB The concentration of immunoglobulins, lactoferrin and lysozyme we compared in bronchial secretions obtained from children with various chronic lung diseases. The IgG, lactoferrin and lysozyme, but not secretory IgA, concentrations were shown to be increased during chronic inflammatory response. RF 001 ALFORD RH J IMMUNOL 101 984 968 002 DEUSCHL H CLIN EXP IMMUNOL 16 401 973 003 FALK GA AM REV RESPIR DIS 105 14 972 004 GAWEL J SCAND J RESPIR DIS 60 63 979 005 HAND WL J CLIN INVEST 53 354 974 006 KALTREIDER HB J CLIN INVEST 54 263 974 007 MARTINEZ-TELLO FJ J IMMUNOL 101 989 968 008 MASSON PL IN: DULFANO MJ 973 009 MASSON PL PROTIDES BIOL FLUIDS 16 633 968 010 MASSON PL J EXP MED 130 643 969 011 MEDICI TC AM REV RESPIR DIS 103 784 971 012 NEWHOUSE M N ENGL J MED 295 1045 976 013 SIEGLER DIM THORAX 29 313 974 014 SOUTAR CA THORAX 30 239 975 015 TOURVILLE DR J EXP MED 129 411 969 CT 1 GAWEL J SCAND J RESPIR DIS 60 63 979 2 AMBRUSO DR J CLIN INVEST 67 352 981 3 LEE GR SEM HEMATOL 20 61 983 4 KIJLSTRA A BR J OPHTHALMOL 67 199 983 5 STELANDER M EUR J RESPIR DIS 65 5 984 6 FICK RB J CLIN INVEST 74 236 984 7 PRYJMA J ANN ALLERGY 54 60 985 PN 79250 RN 01230 AN 80081508 AU Kurz-J-B. Perkins-J-P. Buchwald-M. TI Killing of fibroblasts by dexamethasone or dibutyryl adenosine 3',5'-monophosphate is not a valid test for cystic fibrosis. SO Science. 1979 Dec 14. 206(4424). P 1317-9. MJ CYSTIC-FIBROSIS: di. DEXAMETHASONE: pd. DIBUTYRYL-CYCLIC-AMP: pd. FIBROBLASTS: de. MN ADOLESCENCE. ADULT. CELL-DIVISION: de. CELL-SURVIVAL: de. CELLS-CULTURED. CHILD. CHILD-PRESCHOOL. DOSE-RESPONSE-RELATIONSHIP-DRUG. FEMALE. HUMAN. OUABAIN: pd. SKIN: cy. AB Assays based on the counting of total cells and of colony-forming cells were used to demonstrate that neither dexamethasone nor dibutyryl adenosine 3',5'-monophosphate (cyclic AMP) kills human fibroblasts under a variety of conditions. These results contradict those of previous studies showing that dexamethasone and dibutyryl cyclic AMP kill a higher percentage of fibroblasts from normal humans than from individuals with cystic fibrosis. RF 001 EPSTEIN JL PROC NAT ACAD SCI USA 74 5642 977 002 BRESLOW JL SCIENCE 201 180 978 003 BRESLOW JL CELL 13 663 978 004 EPSTEIN J SOMATIC CELL GENET 4 451 978 005 BUCHWALD M PROC NAT ACAD SCI USA 73 2899 976 006 ELMORE E IN VITRO 13 837 977 009 SIBLEY CH CELL 2 213 974 010 SIBLEY CH CELL 2 221 974 011 MANKOVITZ R CELL 3 221 974 012 EPSTEIN JL PROC NAT ACAD SCI USA 74 1676 977 016 HAM RG IN VITRO 14 11 978 018 PRATT WB J INVEST DERMATOL 71 23 978 019 CASTOR CW J LAB CLIN MED 65 490 965 020 THRASH CR NATURE 242 399 973 021 KAMELY D EXP CELL RES 97 120 976 022 GOSPODAROWICZ D J CELL BIOL 66 541 975 023 HARVEY W ANN RHEUM DIS 33 427 974 024 MILO GF IN VITRO 12 23 976 025 HAYASHI I IN VITRO 14 23 978 026 MACIEIRA-COELHO A EXPERIENTIA 22 390 966 027 MACIEIRA-COELHO A NATURE 251 67 974 028 CRISTOFALO VJ IN VITRO 6 396 971 029 GROVE GL CELL BIOL INT REP 1 147 977 CT 1 SCANLIN TF CLIN CHEST MED 1 424 980 2 BUCHWALD M ADV CYCLIC NUCLEO RES 12 243 980 3 EPSTEIN J PEDIATR RES 14 964 980 4 WEICHSELBAUM RR J CELL PHYSIOL 103 429 980 5 BRESLOW JL SCIENCE 207 1007 980 6 LITTLEFIELD JW N ENGL J MED 304 44 981 7 SHAPIRO BL N ENGL J MED 304 975 981 8 SEALE TW ANN CLIN LAB SCI 12 415 982 9 UNSICKER K CELL TISSUE RES 223 73 982 10 GRANAT M FERTIL STERIL 39 180 983 11 LANGHOFF E PEDIATR RES 18 488 984 12 JANSEN RPS AM J OBSTET GYNECOL 153 363 985 13 PINSKY L ADV HUM GENET 16 299 987 PN 79251 RN 01231 AN 79138718 AU Shapiro-B-L. Lam-L-F. Fast-L-H. TI Premature senescence in cultured skin fibroblasts from subjects with cystic fibrosis. SO Science. 1979 Mar 23. 203(4386). P 1251-3. MJ CELL-SURVIVAL. CYSTIC-FIBROSIS: pa. MN ADOLESCENCE. ADULT. AGING. CELL-DIVISION. CELLS-CULTURED. CHILD. DNA: bi. FEMALE. HUMAN. MALE. SKIN: pa. AB Cultured skin fibroblasts from subjects with cystic fibrosis exhibited normal population doubling times in early passages. After about 13 cumulative population doublings, cystic fibrosis lines doubled more slowly than controls and ceased doubling after about 19 weekly passages. Control lines continued doubling for 27 passages. The premature senescence noted in cells from subjects with cystic fibrosis reconciles controversial observations of cell doubling reported in the literature. Data presented here demonstrate that experiments with cystic fibrosis cells in late passage may generate misleading results since differences from control lines may be ascribed to generalized senile changes rather than to specific results of the cystic fibrosis genotype. RF 001 ANON GAP CONF REP TISS CULT APPR 977 002 RAFF EC J CELL PHYSIOL 74 235 969 003 HOUCK JC PROC SOC EXP BIOL MED 135 369 970 004 HOUCK JC PROC SOC EXP BIOL MED 147 167 974 005 FLETCHER DS CLIN CHIM ACTA 44 5 973 006 WELCH DW PEDIATR RES 9 698 975 007 FARRELL PM PROC SOC EXP BIOL MED 149 340 975 008 BOLTON WE AM J HUM GENET 27 394 975 009 BARRANCO SC J CELL PHYSIOL 88 33 976 010 SHAPIRO BL CLIN CHIM ACTA 82 125 978 011 HAYFLICK L EXP CELL RES 25 585 961 012 HAYFLICK L EXP CELL RES 37 614 965 013 SCHNEIDER EL PROC SOC EXP BIOL MED 141 1092 972 014 GOLDSTEIN S SCIENCE 199 781 978 015 NADLER HL IN: STANBURY JB 1683 978 016 LOBECK CC IN: STANBURY JB 1605 972 017 DOERING KM EUR J PEDIATR 126 185 977 018 KELLER PJ BIOCHEMISTRY 10 4867 971 019 ROBINSON AB PROC NAT ACAD SCI USA 66 753 970 019 MARTIN GM IN: BERGSMA D 978 020 ARGLEBE C CLIN OTOLARYNGOLOGY 1 249 976 021 CHILLA R ARCH OTO RHINO LARYNGOL 214 267 977 022 SWENEY LR PEDIATRICS 40 421 967 023 SWENEY LR ARCH PATHOL 86 413 968 024 STREHLER BL TIME CELLS AND AGING 977 CT 1 LAM LFH LIFE SCI 24 2483 979 2 SHAPIRO BL PROC NAT ACAD SCI USA 76 2979 979 3 HOEHN H AM J MED GENET 7 141 980 4 DAVIS PB PEDIATR RES 14 83 980 5 CHASE HP METABOLISM 29 365 980 6 TARNOKY AL J ROY SOC MED 73 73 980 7 VRACKO R EXP CELL RES 129 345 980 8 ROOMANS GM ULTRASTRUCTURAL PATHOL 2 53 981 9 KURZ JB PEDIATR RES 15 1328 981 10 SHAPIRO BL AM J HUM GENET 34 846 982 11 ANDERSON PJ BIOCHEM BIOPHYS RES COMMUN 108 182 982 12 SHAPIRO BL SCIENCE 216 417 982 13 CEDER O SCANN ELECTRON MICROSC 1982 723 982 14 CARMAGNOL F PEDIATR RES 17 181 983 15 VRACKO R IN VITRO 19 504 983 16 THOMPSON KVA GERONTOLOGY 29 97 983 17 CEDER O ACTA PAEDIATR SCAND SUPPL 309 1983 1 983 18 KATZ S CELL CALC 5 421 984 19 MATTHEWS LW PEDIATR CLIN NORTH AM 31 133 984 20 GRINSTEIN S BIOCHIM BIOPHYS ACTA 769 270 984 21 RAVIA Y HUM GENET 71 294 985 22 AVIOLI LV AM J NEPHROL 6 151 986 23 MATTES PM PROC NAT ACAD SCI USA 84 3009 987 PN 79252 RN 01232 AN 80036401 AU Howie-A-D. Stack-B-H. TI Cystic fibrosis in adolescents and young adults. SO Scott-Med-J. 1979 Jul. 24(3). P 193-8. MJ CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. CHILD. CHILD-PRESCHOOL. CYSTIC-FIBROSIS: co, th. FEMALE. GASTROINTESTINAL-DISEASES: co. HUMAN. INFANT. INFANT-NEWBORN. JOINT-DISEASES: co. LUNG-DISEASES: co. LUNG: ra. MALE. RESPIRATORY-FUNCTION-TESTS. SINUSITIS: co. AB With the early diagnosis of cystic fibrosis and the better management of these patients in childhood increasing numbers are surviving to adult life. The main problem requiring continuous medical care in the older cystic fibrosis patient is persisting and often progressive chest disease. For this reason the cystic fibrosis clinic for adolescents and young adults was started in the Western Infirmary in 1975. This paper presents our observations on 13 patients who had attended between 1975 and 1977. Eleven patients are alive and well and have shown no evidence of deterioration over this period. Ten are at school or in regular employment. With optimum medical supervision it is possible to give young adults with cystic fibrosis a worthwhile existence. RF 001 BOAT TF AM REV RESPIR DIS 116 1 977 002 BOAT TF CLIN PEDIATR 13 505 974 003 BODIAN M FIBROCYSTIC DIS OF THE PANCRE 952 004 COOPERMAN EM CAN MED ASSOC J 105 580 971 005 GAYTON WF AM J DIS CHILD 126 856 973 006 GRACEY M AUSTRALAS ANN MED 18 91 969 007 HODSON ME BR MED J 2 790 976 008 LAWSON D IN: LAWSON D PROC 5TH INT CF 225 969 009 MCCRAE WM SCOTT MED J 21 68 976 010 MCFARLANE H BR MED J 1 423 975 011 MATHIEU J-P BR J DIS CHEST 72 57 978 012 MILNER AD ARCH DIS CHILD 44 351 969 013 MITCHELL-HEGGS PF Q J MED 45 479 976 014 ROSAN RC AM J DIS CHILD 104 625 962 015 SHWACHMAN H MEDICINE (BALTIMORE) 56 129 977 016 STERN RC ANN INTERN MED 87 188 977 017 STRUNK RC ARCH DIS CHILD 52 687 977 018 WARWICK WJ JAMA 238 2159 977 CT 1 JACOBS WH AM J GASTROENTEROL 76 342 981 2 MACPHERSON AIS SCOTT MED J 29 6 984 3 PHILLIPS BM J ROY SOC MED 79 44 986 PN 79253 RN 01233 AN 79181843 AU Frydman-M-I. TI Implications of cystic fibrosis for health services and the afflicted. SO Soc-Sci-Med. 1979 Mar. 13A(2). P 147-50. (REVIEW). MJ CYSTIC-FIBROSIS: px. MN ADAPTATION-PSYCHOLOGICAL. ADULT. CHILD. CYSTIC-FIBROSIS: co, th. FAMILY. HUMAN. MENTAL-DISORDERS: co. PARENT-CHILD-RELATIONS. REVIEW. AB "Psychosocial" literature on cystic fibrosis (CF) is critically reviewed. Major methodological shortcomings include the anecdotal nature of findings, overemphasis on negative consequences of the disease, and the lack of a suitable theoretical framework. Avenues for future research are suggested. RF 001 KRAMM ER PEDIATRICS 28 135 961 002 POGUE RE MINN MED 52 1551 969 003 SHWACHMAN H AM J DIS CHILD 96 6 958 004 SHWACHMAN H PEDIATRICS 36 689 965 005 ANON GAP CONF REP CHRON BRONC CF 977 006 DI SANTAGNESE PA N ENGL J MED 295 481 976 007 WOOD RE AM REV RESPIR DIS 113 833 976 008 LAPEY A IN: GELLIS SS 221 976 009 LIFSCHITZ M ARCH FOUND THANATOLOGY 1 23 969 010 TOWNES BD J PSYCHOSOM RES 18 9 974 011 TURK J PEDIATRICS 34 67 964 012 TROPAUER A AM J DIS CHILD 119 424 970 013 TEICHER JD CALIF MED 110 371 969 014 BLOM G PEDIATRICS 22 593 958 015 MATTSSON A PEDIATRICS 50 801 972 016 FRIEDMAN SB PEDIATRICS 32 610 963 017 LAWLER RH CAN MED ASSOC J 94 1043 966 018 GAYTON WF AM J DIS CHILD 126 856 973 019 GOLDBERG DP DETECTION OF PSYCHIATRIC ILLN 972 020 KULCZYCKI LL CLIN PROC CHILD HOSP DC 25 320 969 021 ELLIS CE J PEDIATR 87 565 975 022 BURTON L FAMILY LIFE OF SICK CHILDREN 975 023 SPOCK A NC MED J 27 426 966 024 GREEN MN PEDIATRICS 34 58 964 025 MCCOLLUM AT J PEDIATR 77 571 970 026 GORDON NB J HLTH HUM BEHAV 6 190 965 027 BLUMENTHAL MD ARCH GEN PSYCHIAT 21 160 969 028 ORBACH CE CANCER 8 20 955 029 LINDEMANN E AM J PSYCHIATRY 101 141 944 030 GLASER BG AWARENESS OF DYING 965 031 NAJMAN JM HOSP HLTH ADMIN 5 8 973 032 PINKERTON P ARIZ MED 26 348 969 033 ROSENLUND ML ANN INTERN MED 78 959 973 034 MCCRAE WM LANCET 2 141 973 035 BURTON L PRACTITIONER 210 247 973 036 MEYEROWITZ JH SOC SCI MED 1 249 967 037 PLESS IB CHRONIC CHILDHOOD DISORDER 975 038 ALLAN JL AUST PAEDIATR J 10 136 974 039 HALLORAN KH J PEDIATR 88 1054 976 040 KUPST MJ PEDIATRICS 59 266 977 041 FRASER FC AM J HUM GENET 26 636 974 042 TAVORMINA JB J ABNORM CHILD PSYCHOL 4 99 976 043 GAYTON WF PEDIATRICS 59 888 977 044 BEDELL JR J PSYCHOSOM RES 21 237 977 045 CHODOFF P AM J PSYCHIATRY 120 743 964 046 GOFFMAN E ASYLUMS 961 047 SZASZ TS IDEOLOGY AND INSANITY 973 048 DENZIN NK IN: SPITZER SP 968 049 BOZEMAN MF CANCER 8 1 955 050 PARSONS T J SOC ISSUES 8 31 952 051 GORDON NB J HLTH HUM BEHAV 6 190 965 052 PRATT L FAMILY STRUCTURE EFFECTIVE HE 976 053 FREIDSON E PROF DOMINANCE THE SOCIAL STR 970 054 STRAUSS AL CHRONIC ILLNESS AND THE QUALI 975 055 ANDERSEN DH CHILDREN 7 11 960 056 KULCZYCKI LL PUBL HEALTH REP 76 88 961 057 STEINBERG AG AM J HUM GENET 12 416 960 058 WRIGHT SW AM J HUM GENET 20 157 968 059 ANON GAP CONF REP PSYCHOSOCIAL ASP 974 060 GOLODETZ A MED CARE 7 385 969 061 PATTERSON PR PSYCHOSOCIAL ASPECTS OF CF 973 062 MCCRAE WM PHYSIOTHERAPY 61 252 975 063 HAMBURG DA PSYCHOTHER PSYCHOSOM 23 13 974 064 HAGGERTY RJ CHILD HEALTH AND THE COMMUNIT 975 065 PLESS IB PEDIATR CLIN NORTH AM 21 223 974 CT 1 PLESS IB PEDIATR CLIN NORTH AM 31 33 984 PN 79254 RN 01234 AN 79138906 AU Wood-R-E. TI Cystic fibrosis: diagnosis, treatment, and prognosis. SO South-Med-J. 1979 Feb. 72(2). P 189-202. (REVIEW). MJ CYSTIC-FIBROSIS: th. MN AEROSOLS. ANTIBIOTICS: tu. COUGH: et. CYSTIC-FIBROSIS: co, di, fg, mo, pp, px. DIET. HUMAN. INFANT-NEWBORN. INFANT-NEWBORN-DISEASES: di. LUNG-DISEASES: et. METHODS. PANCREAS: pp. PHYSICAL-THERAPY. PROGNOSIS. REVIEW. INFERTILITY: et. SWEAT: an. SUPPORT-U-S-GOVT-P-H-S. UNITED-STATES. EX This review is intended to present a brief summary of the important areas and concepts of cystic fibrosis relevant to clinical practice, indicating those that remain controversial and offering opinions based on both relevant literature and the clinical experience of the Cleveland Cystic Fibrosis Center. The sweat test, tests of pancreatic function, tests of pulmonary function, genetics and family history in diagnosis, infertility as a diagnostic factor, the role of circulating factors in diagnosis, heterozygote detection and prenatal diagnosis, miscellaneous factors leading to diagnosis, neonatal screening, and general observations on the diagnosis of CF are examined. A philosophy of treatment, physical measures, antimicrobial therapy, aerosol therapy, the role of hospitalization in a therapeutic program, other drug therapy, the treatment of cor pulmonale, respiratory failure, and specific pulmonary complications are discussed. Enzyme replacement and obstructive phenomena, glucose intolerance, the treatment of the sweat defect, psychosocial therapy, and prognosis are also considered. RF 001 WOOD RE AM REV RESPIR DIS 113 833 976 002 DI SANTAGNESE PA N ENGL J MED 295 481 976 003 DI SANTAGNESE PA PEDIATRICS 12 549 953 004 GIBSON LE PEDIATRICS 23 545 959 005 GIBSON LE PROC FOR QUANTITATIVE ION 975 006 HADORN B J PEDIATR 73 39 968 007 LOBER CW CHEST 66 332 974 008 BARBERO GJ AM J DIS CHILD 112 536 966 009 LAPEY A J PEDIATR 84 328 974 010 TAUSSIG LM PEDIATRICS 54 229 974 011 WOLF RO J LAB CLIN MED 87 164 976 012 ANON CF FND 1975 REP SURVI STUD PA 977 013 ORENSTEIN DM AM J DIS CHILD 131 973 977 014 KAPLAN E N ENGL J MED 279 65 968 015 COHEN LF GAP CONF REP PROB REPRO PHYSI 6 975 016 HERROD HG J PEDIATR 91 276 977 017 OPPENHEIMER EH J PEDIATR 75 806 969 018 OPPENHEIMER EH J PEDIATR 77 991 970 019 KOPITO LE FERTIL STERIL 24 512 973 020 DI SANTAGNESE PA N ENGL J MED 277 1287 967 021 SPOCK A PEDIATR RES 1 173 967 022 BOWMAN BH SCIENCE 164 325 969 023 KAISER D PEDIATR RES 5 167 971 024 MANGOS JA PEDIATR RES 1 436 967 025 ANON GAP CONF REP CILIARY INHIBITO 973 026 WOOD RE HOSP PRACT 11 91 976 027 GREEN MN PEDIATRICS 21 635 958 028 STEPHAN U PEDIATRICS 55 35 975 029 HOLSCLAW DS CF CLUB ABST 977 031 WOOD RE AM REV RESPIR DIS 111 733 975 032 COMROE JH JR PHYSIOLOGY OF RESPIRATION 965 033 MELLINS RB AM REV RESPIR DIS SUPPL 110 137 974 034 WOOD RE IN: BARAN D 977 035 SCHWARTZ RH AM J DIS CHILD 120 432 970 036 PENNINGTON JE J INFECT DIS 128 63 973 037 MARKS MI J PEDIATR 79 822 971 038 STERN RC CLIN PEDIATR 11 521 972 039 RUCKER RW PEDIATRICS 54 358 974 040 PENNINGTON JE AM J MED 58 629 975 041 LANDAU LI J PEDIATR 82 863 973 042 MATTHEWS LW J PEDIATR 65 558 964 043 DOERSHUK CF PEDIATRICS 41 723 968 044 MOTOYAMA EK PEDIATRICS 50 299 972 045 CHANG N AM REV RESPIR DIS 107 672 973 046 WOLFSDORF J PEDIATRICS 43 799 969 047 BAU SK PEDIATRICS 48 605 971 048 WOOD RE PROC INT CF CONG 7TH 976 049 WOOD RE AM REV RESPIR DIS 113 833 976 050 HUANG NN GUIDE TO DRUG THERAPY IN PATI 972 051 YEATES DB AM REV RESPIR DIS 107 602 973 052 STREIDER DJ PROC INT CF CONG 7TH 976 053 HOIBY N SCAND J RESPIR DIS 56 38 975 054 BOWDEN DH AM J MED 38 226 965 055 BLOCK AJ AM REV RESPIR DIS SUPPL 110 71 974 056 KELMINSON LL PEDIATRICS 39 24 967 057 DAVIS PB JAMA 239 1851 978 058 STOWE SM AM REV RESPIR DIS 111 611 975 059 HOLSCLAW DS J PEDIATR 76 829 970 060 STERN RC AM REV RESPIR DIS 117 825 978 061 FELLOWS KE RADIOLOGY 114 551 975 062 BROWN CH 3RD N ENGL J MED 291 265 974 063 KYLSTRA JA AM REV RESPIR DIS 103 651 971 064 REGAN PT N ENGL J MED 297 854 977 065 FARRELL PM J CLIN INVEST 60 233 977 066 GRAHAM DY N ENGL J MED 296 1314 977 067 DIMAGNO EP N ENGL J MED 296 1318 977 068 ELLIOTT RB PEDIATRICS 57 474 976 069 CAMPBELL IM PEDIATRICS 57 480 976 070 STAPLETON FB N ENGL J MED 295 246 976 071 ROSENLUND ML PEDIATRICS 59 428 977 072 HUANG NN IN: SENIOR JR 207 968 073 ALLAN JD AM J DIS CHILD 126 22 973 074 BERRY HK AM J DIS CHILD 129 165 975 075 RAEBURN JA PROC NUTR SOC 36 77 977 076 WAGGET J J PEDIATR 77 407 970 077 DONNISON AB PEDIATRICS 37 833 966 078 STERN RC GASTROENTEROLOGY 70 645 976 079 HANDWERGER S N ENGL J MED 281 451 969 080 LIPPE BM J PEDIATR 90 751 977 081 WILMSHURST EG PEDIATRICS 55 75 975 082 CHAZAN BI J PEDIATR 77 86 970 083 ANON CF FND 1976 REP SURVI STUD PA 978 084 TAUSSIG LM J PEDIATR 82 380 973 085 DOERSHUK CF PEDIATRICS 36 675 965 CT 1 HOLSCLAW DS CLIN CHEST MED 1 407 980 2 REED MD PEDIATR RES 15 1234 981 3 CONGDEN PJ ARCH DIS CHILD 56 708 981 4 KANG SB ANESTH ANALG CLEVE 61 793 982 5 IANNUCCI A HUM PATHOL 15 278 984 6 LEEDER JS CLIN PHARMACOL THER 36 355 984 7 NAYLOR EW SEM PERINATOL 9 232 985 8 GILLIGAN PH J CLIN MICROBIOL 22 5 985 9 REED MD ANTIMICROB AGENTS CHEMOTHER 27 583 985 10 KATZ JN J PEDIATR 108 352 986 PN 79255 RN 01235 AN 80014930 AU van-Vroonhoven-T-J. Molenaar-J-C. TI Distal splenorenal shunt for decompression of portal hypertension in children with cystic fibrosis. SO Surg-Gynecol-Obstet. 1979 Oct. 149(4). P 559-60. MJ CYSTIC-FIBROSIS: co. HYPERTENSION-PORTAL: su. RENAL-VEINS: su. SPLENIC-VEIN: su. MN ADOLESCENCE. CHILD. FEMALE. HUMAN. HYPERTENSION-PORTAL: co. MALE. METHODS. AB Apart from the sound physiologic basis for the distal splenorenal shunt as compared with the portacaval shunt and the conventional central splenorenal shunt, there are two important reasons why we think the use of this type of shunt is especially advantageous in children with portal hypertension secondary to cystic fibrosis. Firstly, the thick, fibrotic retroperitoneal area in the porta hepatis, where a portacaval shunt has to be constructed, can be avoided, which makes the distal splenorenal shunt the easier operation. Secondly, notwithstanding the relatively small-sized vessels, a wide anastomosis can be constructed with a high flow rate and, therefore, a minimal chance of shunt thrombosis. RF 001 GALAMBOS JT N ENGL J MED 295 1089 976 002 MAILLARD JN ETUDE HEMODYNAMIQUE DE LANAST 978 003 MAKSOUD JG J PEDIATR SURG 13 335 978 004 SCHUSTER SR J PEDIATR SURG 12 201 977 005 STERN RC GASTROENTEROLOGY 70 645 976 006 WARREN WD ANN SURG 166 437 967 CT 1 TABACHNIK NF SURG GYNECOL OBSTET 152 837 981 2 ROY CC J PEDIATR GASTROENTEROL NUTR 1 469 982 3 HENDERSON JM SEM LIVER DIS 3 251 983 4 HUBBARD VS SEM RESPIR MED 6 299 985 5 BERNARD O CLIN GASTROENTEROL 14 33 985 6 SCHRODER R SCHWEIZ MED WOCHENSCHR 115 828 985 PN 79256 RN 01236 AN 80124640 AU Bowman-B-H. TI Cystic fibrosis: review of investigations of the mucociliary inhibitor. SO Tex-Rep-Biol-Med. 1979. 38. P 47-53. (REVIEW). MJ BLOOD-PROTEINS: ph. CYSTIC-FIBROSIS: pp. MN BIOLOGICAL-ASSAY. BODY-FLUIDS. CILIA: de. CYSTIC-FIBROSIS: fg, me. GENES-RECESSIVE. GILLS. HETEROZYGOTE. HOMOZYGOTE. HUMAN. OYSTERS. PROTEINS: an. REVIEW. EX Recently, we found the cystic fibrosis mucociliary inhibitor in urine from homozygotes and heterozygotes, but not from normals. In recent studies we have attempted to compare the relative concentrations of CFMI in material from homozygotes and heterozygotes. The unites of inhibition which reflect the concentration of the CFMI in serum and tissue culture medium fractions from heterozygotes is close to one-half of that in fractions from homozygous sources. With purification of the CFMI and the generation of a monospecific antibody preparation, answers will become available to the questions of whether or not there is a normal counterpart to the CFMI and whether or not quantitation of the CFMI will be useful in heterozygote detection and prenatal diagnosis of cystic fibrosis. RF 001 ANDERSEN DH AM J DIS CHILD 56 344 938 002 BARNETT DR TEX REP BIOL MED 31 703 973 003 BAUR PS TEX REP BIOL MED 34 155 976 004 BERATIS NG PEDIATR RES 7 958 973 005 BOWMAN BH PROC NAT ACAD SCI USA 70 548 973 006 BOWMAN BH BIOCHEM BIOPHYS RES COMMUN 64 1310 975 007 BOWMAN BH SCIENCE 164 325 969 008 BOWMAN BH CLIN GENET 4 461 973 009 BOWMAN BH CLIN GENET 12 333 977 011 CARSON SD DISSERTATION 978 012 COHEN FL J MED GENET 11 253 974 013 CONOVER JH PEDIATR RES 7 220 973 014 DANES BS J EXP MED 136 1313 972 015 FANCONI G WIEN MED WOCHENSCHR 86 753 936 016 HIRSCHHORN K IN: MANGOS JA 305 976 017 LITT M BIOCHEM BIOPHYS RES COMMUN 43 919 971 018 MANGOS JA SCIENCE 158 135 967 019 MAYO BJ TEX REP BIOL MED 34 107 976 020 MCNEELY MC DISSERTATION 978 021 SPOCK A PEDIATR RES 1 173 967 022 WILSON GB CLIN RES 24 295 976 CT 1 HALLINAN F MED HYPOTHESES 7 793 981 2 THOMAS JM CLIN CHIM ACTA 111 199 981 3 BLITZER MG PEDIATR RES 16 203 982 4 CEDER O SCANN ELECTRON MICROSC 1982 723 982 5 CEDER O ULTRASTRUCTURAL PATHOL 4 305 983 6 VONEULER AM ULTRASTRUCTURAL PATHOL 5 37 983 PN 79257 RN 01237 AN 80015007 AU Landau-L-I. Mellis-C-M. Phelan-P-D. Bristowe-B. McLennan-L. TI "Small airways disease" in children: no test is best. SO Thorax. 1979 Apr. 34(2). P 217-23. MJ ASTHMA: di. CYSTIC-FIBROSIS: di. MN ADOLESCENCE. ADULT. ASTHMA: pp. CHILD. CYSTIC-FIBROSIS: pp. FEMALE. HELIUM: du. HUMAN. LUNG: ph. MALE. NITROGEN: du. RESPIRATORY-FUNCTION-TESTS. AB Tests claimed to detect small airway disease were evaluated in children. Fifty-two subjects who were normal, 30 with cystic fibrosis (CF), and 35 with asthma were selected on the basis of normal spirometry, informed consent being obtained from them and their parents. Tests undertaken were measurement of maximum expiratory flow volume curves breathing air and a helium-oxygen mixture, single breath nitrogen washout curves, lung volumes, and, except in the normal subjects, measurement of arterial oxygen tension. Closing volumes were normal in most children with CF and asthma, whereas the slope of the alveolar plateau was abnormal in 83%. Maximum expiratory flow rate at 50% of total lung capacity was reduced in 65% and maximum mid-expiratory flow rate in 57%; residual volume increased in 51%, and results of other tests were abnormal in less than 50% of the children. There was evidence suggesting that lung disease was more patchy in children with mild CF than in those with interval phase asthma. These tests do not detect specific pathological processes in small airways but reflect the consequences of small airway abnormalities, which may vary in the same disease as well as in different diseases. No one test alone appears most useful. Together they contribute to understanding the functional abnormalities present in each individual. RF 001 ANTHONISEN NR CLIN SCI 35 495 968 002 ANTHONISEN NR RESPIR PHYSIOL 8 58 969 003 ALDERSON PO RADIOLOGY 111 151 974 004 BODE FR AM J MED 59 43 975 005 BUIST AS AM REV RESPIR DIS 108 1078 973 006 BURROWS B AM REV RESPIR DIS 115 751 977 007 COLLEY JRT BR MED J 3 195 973 008 COOPER DM AM REV RESPIR DIS 109 519 974 009 DEMUTH GR AM J DIS CHILD 103 129 962 010 DESPAS PJ J CLIN INVEST 51 3235 972 011 DUBOIS AB J CLIN INVEST 35 322 956 012 FOX WW PEDIATRICS 54 293 974 013 GELB AF AM REV RESPIR DIS 112 401 975 014 HILL DJ AM REV RESPIR DIS 106 873 972 015 KNUDSON RJ AM REV RESPIR DIS 115 423 977 016 LAMARRE A PEDIATRICS 50 291 972 017 LANDAU LI AM REV RESPIR DIS 108 593 973 018 MCCARTHY DS AM J MED 52 747 972 019 MANSELL A J APPL PHYSIOL 33 711 972 020 MATSUBA K AM REV RESPIR DIS 107 552 973 021 MELLINS RB PEDIATRICS 44 315 969 022 MELLIS CM AUST PAEDIATR J 12 77 976 023 STANESCU D J APPL PHYSIOL 42 859 977 024 WEST JB VENTILATION BLOOD FLOW AND GA 107 965 025 WOOLCOCK AJ J CLIN INVEST 48 1097 969 026 ZAPLETAL A J APPL PHYSIOL 26 308 969 CT 1 MANSMANN HC CLIN CHEST MED 1 339 980 2 THOMPSON JE MED J AUST 1 668 980 3 MARTIN AJ AM REV RESPIR DIS 122 609 980 4 KERREBIJN KF EUR J RESPIR DIS 63 5 982 5 WOOLCOCK AJ EUR J RESPIR DIS 65 1 984 6 FELDMAN M AVIAT SPACE ENVIRON MED 57 706 986 PN 79258 RN 01238 AN 80237415 AU Lieberman-J. Costea-N-V. Jakulis-V-J. Kaneshiro-W. TI Detection of a lectin in the blood of cystic fibrosis homozygotes and heterozygotes. SO Trans-Assoc-Am-Physicians. 1979. 92. P 121-9. MJ CYSTIC-FIBROSIS: bl. LECTINS: an. MN ANIMAL. CYSTIC-FIBROSIS: fg. DEXTRANS: pd. DOGS. GUINEA-PIGS. HAMSTERS. HEMAGGLUTINATION-INHIBITION-TESTS: mt. HEMAGGLUTINATION-TESTS: mt. HEMAGGLUTINATION: de. HETEROZYGOTE. HOMOZYGOTE. HUMAN. IGM: im. LYMPHOCYTES: de, en. MICE. PEDIGREE. PHYTOHEMAGGLUTININS: pd. RABBITS. RATS. SUPPORT-U-S-GOVT-NON-P-H-S. SUPPORT-U-S-GOVT-P-H-S. EX We theorize that cystic fibrosis may be a disease in which a plasma factor with lectin-like activity stimulates mucus secretion, causes precipitation of mucus glycoproteins, and incidentally affects lymphocytes in culture. To confirm our theory, we planned to search for additional lectin-like activities in CF serum. Our experiments suggest that the lectin-like substance in CF blood requires IgM for its red-blood-cell-agglutination activity. Thus, no difference in agglutination titer is observed between CF homozygotes and heterozygotes, because IgM is the limiting factor, and IgM concentrations do not differ appreciably in such subjects. We suspect that CF homozygotes have a larger pool of free lectin in their blood than do heterozygotes, so that quantitation of this free lectin pool may distinguish between the two phenotypes. Excess free lectin may account for the signs and symptoms of CF, whereas lesser amounts may theoretically be advantageous to the heterozygote. RF 001 MARCHI AG HELV PAEDIATR ACTA 28 427 973 002 LIEBERMAN J AM REV RESPIR DIS 116 1047 977 003$ LIEBERMAN J CHEST 75S 220S 979 004 FREED DLJ LANCET 1 585 978 005 SHARON N SCIENCE 177 949 972 006 BROOKS DE IN: MOHN JF 27 977 CT 1 LIEBERMAN J J LAB CLIN MED 97 646 981 2 SEALE TW ANN CLIN LAB SCI 12 415 982 3 LIEBERMAN J AM J MED 77 678 984 4 QURESHI AR J PEDIATR 106 913 985 PN 79259 RN 01239 AN 80105142 AU Bohles-H. Heid-H. Stehr-K. Fekl-W. TI Deficiencies of essential fatty acids and vitamin E in cystic fibrosis. SO Z-Ernahrungswiss. 1979 Jul. 18(2). P 81-7. MJ CYSTIC-FIBROSIS: pp. FATTY-ACIDS-ESSENTIAL: df. VITAMIN-E-DEFICIENCY: pp. MN ADOLESCENCE. BODY-HEIGHT. CHILD. CHILD-PRESCHOOL. CHOLESTEROL-ESTERS: bl. CYSTIC-FIBROSIS: co. FATTY-ACIDS: bl. FEMALE. HUMAN. INFANT. MALE. PHOSPHOLIPIDS: bl. TRIGLYCERIDES: bl. VITAMIN-E-DEFICIENCY: co. VITAMIN-E: bl. AB In 25 children (13 male; 12 female) with cystic fibrosis aged 6 months to 16 years and 24 matched controls total serum vitamin E levels and fatty acid patterns of serum cholesterol esters, phospholipids and triglycerides are demonstrated. Compared to controls (1.02 +/- 0.24 mg/dl) the total serum vitamin E levels are significantly decreased in patients with cystic fibrosis (0.30 +/- 0.26 mg/dl) (p less than 0.01). There is no significant difference comparing the fatty acid patterns of the serum ester fractions of both groups. Differences can be seen best in the cholesterol ester fraction. In this fraction linoleic acid shows a trend to be decreased in the cystic fibrosis patients compared to the control group. A possible influence of height velocity on the levels of essential fatty acids is discussed. RF 001 ANON NUTR REV 17 13 959 002 BLANC WA PEDIATRICS 22 494 958 003 ELLIOTT RB ARCH DIS CHILD 50 76 975 004 FOLCH J J BIOL CHEM 226 497 957 005 GORDON HH TRANS AM CLIN CLIMAT ASSOC 68 155 956 006 GORDON HH AM J CLIN NUTR 4 391 956 007 HARRIS R ARCH DIS CHILD 30 424 955 008 HARRIES JT ARCH DIS CHILD 46 341 971 009 HOLMAN RT J NUTR 70 405 960 010 JENSEN SB ACTA PAEDIATR SCAND 45 70 956 011 MCWHIRTER WR ACTA PAEDIATR SCAND 64 446 975 012 MULLER DPR GUT 15 966 974 013 NITOWSKY HM AM J DIS CHILD 92 164 956 014 NITOWSKY HM AM J CLIN NUTR 4 397 956 015 NITOWSKY HM BULL JOHNS HOPKINS HOSP 98 361 956 016 PAULSRUD JR AM J CLIN NUTR 25 897 972 017 QUAIFE ML J BIOL CHEM 180 1229 949 018 ROBINSON GR LANCET 2 919 975 019 ROSS CAC ARCH DIS CHILD 30 316 955 020 RIVERS JPW LANCET 2 642 975 021 SHMERLING DH PEDIATRICS 46 690 970 022 UNDERWOOD BA ANN NY ACAD SCI 203 237 972 023 UNDERWOOD BA PEDIATR RES 6 26 972 024 WATTS R LANCET 2 983 975 025 WITTING LA AM J CLIN NUTR 27 952 974 026 ZOLLNER N Z GESAMTE EXP MED 146 89 968 CT 1 CHO YW J CLIN PHARMACOL 21 224 981 2 BOHLES H KLIN PAEDIATR 197 386 985