Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Mediated Residual Chloride Secretion Does Not Protect against Early Chronic Pseudomonas aeruginosa Infection in F508del Homozygous Cystic Fibrosis Patients

Pediatric Research

Volumn 55, Issue 1, 2004, Pages 69-75

  Derichs, Nico ^a   Mekus, Frauke ^a   Bronsveld, Inez ^b   Bijman, Jan ^c   Veeze, Henk J ^b   Von Der Hardt, Horst ^a   Tümmler, Burkhard ^a   Ballmann, Manfred ^a  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b SOPHIA CHILDREN S HOSPITAL   (Netherlands)

^c ERASMUS UNIVERSITY ROTTERDAM   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE; CHLORIDE CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ADOLESCENT; ARTICLE; BACTERIAL COLONIZATION; CHILD; CHLORIDE TRANSPORT; CLINICAL ARTICLE; CYSTIC FIBROSIS; DISEASE SEVERITY; FEMALE; GENE MUTATION; GENOTYPE; GRAM NEGATIVE INFECTION; HUMAN; MALE; PHENOTYPE; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA;

ADOLESCENT; ADULT; AGE OF ONSET; CHILD; CHILD, PRESCHOOL; CHLORIDES; CHRONIC DISEASE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ELECTROPHYSIOLOGY; EPITHELIAL CELLS; FEMALE; HOMOZYGOTE; HUMANS; MALE; MUTATION; PHENOTYPE; PSEUDOMONAS INFECTIONS; RESPIRATORY FUNCTION TESTS;

EID: 0346362331     PISSN: 00313998     EISSN: None     Source Type: Journal    
DOI: 10.1203/01.PDR.0000100758.66805.CE     Document Type: Article

References (49)

1. Santis G 1995 Basic molecular genetics. In: Hodson M, Geddes D (eds) Cystic Fibrosis. Chapman & Hall, London, pp 15-39
2. Veeze HJ, Halley DJJ, Bijman J, de Jongste JC, de Jonge HR, Sinaasappel M 1994 Determinants of mild clinical symptoms in cystic fibrosis patients-residual chloride secretion measured in rectal biopsies in relation to the genotype. J Clin Invest 93:461-466
3. Gan KH, Veeze HJ, van der Ouweland AMW, Halley DJJ, Scheffer H, van der Hout A, Overbeek SE, de Jongste JC, Bakker W, Heijerman HGM 1995 A cystic fibrosis mutation associated with mild lung disease. N Engl J Med 333:95-99
4. Walker LC, Venglarik CJ, Aubin G, Weatherly MR, McCarty NA, Lesnick B, Ruiz F, Clancy JP, Sorscher EJ 1997 Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR. Am J Respir Crit Care Med 155:1684-1689
5. Lester LA, Kraut J, Lloyd-Still J, Karrison T, Mott C, Billstrand C, Lemke A, Ober C 1994 ΔF508 genotype does not predict disease severity in an ethnically diverse cystic fibrosis population. Pediatrics 93:114-118
6. Johansen HK, Nir M, Høiby N, Koch C, Schwartz M 1991 Severity of cystic fibrosis in patients homozygous and heterozygous for ΔF508 mutation. Lancet 337:631-634
7. Mekus F, Ballmann M, Bronsveld I, Bijman J, Veeze HJ, Tümmler B 2000 Categories of ΔF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics. Twin Res 3:277-293
8. Bronsveld I, Mekus F, Bijman J, Ballmann M, Greipel J, Hundrieser J, Halley DJJ, Laabs U, Busche R, de Jonge HR, Tümmler B, Veeze HJ, The European Twin and Sibling Study Consortium 2000 Residual chloride secretion in intestinal tissue of ΔF508 homozygous twins and siblings with cystic fibrosis. Gastroenterology 119:32-40
9. Bronsveld I, Mekus F, Bijman J, Ballmann M, de Jonge HR, Laabs U, Halley DJ, Ellemunter H, Mastella G, Thomas S, Veeze HJ, Tümmler B 2001 Chloride conductance and genetic background modulate the cystic fibrosis phenotype of ΔF508 homozygous twins and siblings. J Clin Invest 108:1705-1715
10. Høiby N, Koch C 1990 Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax 45:881-884
11. Demko C, Byard P, Davis P 1995 Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection. J Clin Epidemiol 48:1041-1049
12. 1 and treatment intensity during Pseudomonas aeruginosa colonisation in patients with cystic fibrosis. Thorax 53:732-737
13. Zar H, Saiman L, Quittell L, Prince A 1995 Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator. J Pediatr 126:230-233
14. Kubesch P, Dörk T, Wulbrand U, Kälin N, Neumann T, Wulf B, Geerlings H, Weißbrodt H, von der Hardt H, Tümmler B 1993 Genetic determinants of airways' colonisation with Pseudomonas aeruginosa in cystic fibrosis. Lancet 341:189-193
15. Santis G, Osborne L, Knight RA, Hodson ME 1990 Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosis. Lancet 336:1081-1084
16. Pier GB, Grout M, Zaidi TS 1997 Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung. Proc Natl Acad Sci U S A 94:12088-12093
17. Pier GB 2002 CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection. Curr Opin Microbiol 5:81-86
18. Schroeder TH, Lee MM, Yacono PW, Cannon CL, Gerceker AA, Golan DE, Pier GB 2002 CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation. Proc Natl Acad Sci U S A 99:6907-6912
19. Poschet JF, Boucher JC, Tatterson L, Skidmore J, Van Dyke RW, Deretic V 2001 Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung. Proc Natl Acad Sci U S A 98:13972-13977
20. Taylor CJ, Baxter PS, Hardcastle J. Hardcastle PT 1988 Failure to induce secretion in jejunal biopsies from children with cystic fibrosis. Gut 29:957-962
21. Dray-Charier N, Paul A, Scoazec J, Veissière D, Mergey M, Capeau J, Soubrane O, Housset C 1999 Expression of ΔF508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients. Hepatology 29:1624-1634
22. Knowles M, Gatzy J, Boacher R 1981 Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 305:1489-1495
23. Ho LP, Samways JM, Porteous DJ, Dorin JR, Carothers A, Greening AP, Innes JA 1997 Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis. Eur Respir J 10:2018-2022
24. Thomas SR, Jaffe A, Geddes DM, Hodson ME, Alton EWFW 1999 Pulmonary disease severity in men with ΔF508 cystic fibrosis and residual chloride secretion. Lancet 353:984-985
25. Alton EWFW 1995 Applied cell biology. In: Hodson M, Geddes D (eds) Cystic Fibrosis. Chapman & Hall. London, pp 41-73
26. Schwiebert EM, Flotte T, Cutting GR, Guggino WB 1994 Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents. Am J Physiol 266:C1464-C1477
27. Anderson MP, Sheppard DN, Berger HA, Welsh MJ 1992 Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am J Physiol 263:L1-L14
28. - channel proteins. Genomics 54:200-214
29. Gruber AD, Schreur KD, Ji H, Fuller CM, Pauli BU 1999 Molecular cloning and transmembrane structure of hCLCA2 from human lung trachea and mammary gland. Am J Physiol 276:C1261-C1270
30. Pauli BU, Abdel-Ghany M, Cheng HC, Gruber AD, Archibald HA, Elble RC 2000 Molecular characteristics and functional diversity of CLCA family members. Clin Exp Pharmacol Physiol 27:901-905
31. - channels in planar lipid bilayers. Am J Physiol 256:C902-C912
32. Schwiebert EM, Morales MM, Devidas S, Egan ME, Guggino WB 1998 Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci U S A 95:2674-2679
33. Stern M, Sens B, Wiedemann B, Busse O 2002 Bericht Qualitä tssicherung Mukoviszidose 2001. Wissenschaftlicher Beirat "Qualitä tssicherung Mukoviszidose" and Zentrum für Qualitätsmanagement im Gesundheitswesen in cooperation with Mukoviszidose e.V. Germany, Hannover, pp 45
34. Veeze HJ, Sinaasappel M, Bijman J, Bouquet J, de Jonge HR 1991 Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology 101:398-403
35. Dharmsathaphom K, Pandol SJ 1986 Mechanism of chloride secretion induced by carbachol in a colonic epithelial cell line. J Clin Invest 77:348-354
36. Keely SJ, Stack WA, O'Donoghue DP, Baird AW 1995 Regulation of ion transport by histamine in human colon. Eur J Pharmacol 279:203-209
37. Goldstein JL, Shapiro AB, Rao MC, Layden TJ 1991 In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa. Gastroenterology 101:1012-1019
38. Bijman J, Kansen M, Hoogeveen AM, Scholte BJ, van der Kamp AWM, de Jonge HR 1988 Electrolyte transport in normal and CF epithelia. In: Young JA, Wong PY (eds) Exocrine Secretion. University Press, Hong Kong, pp 17-19
39. Derichs N, Bronsveld I, Mekus F, Bijman J, Veeze HJ, Tümmler B, Ballmann M 2000 Intestinal current measurement (ICM) for the diagnosis of atypical cystic fibrosis. Pediatr Pulmonol S20:91
40. Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B 1983 Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis 127:725-734
41. Navarro J, Rainisio M, Harms HK, Hodson ME, Koch C, Mastella G, Strandvik B, McKenzie SG 2001 Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF. European Epidemiologic Registry of Cystic Fibrosis. Eur Respir J 18:298-305
42. Kromeyer-Hauschild K, Wabitsch M, Kunze D, Geller F, Geiß HC, Hesse V, von Hippel A, Jaeger U, Johnsen D, Korte W, Menner K, Müller G, Müller JM, Niemann-Pilatus A, Remer T, Schaefer F, Wittchen HU, Zabransky S, Zellner K, Ziegler A, Hebebrand J 2001 Percentiles of body mass index in children and adolescents evaluated from different regional German studies. Monatsschr Kinderheilkd 149:807-818
43. Cole TJ 1990 The LMS method for constructing normalized growth standards. Eur J Clin Nutr 44:45-60
44. Kälin N, Claaß A, Sommer M, Puchelle E, Tümmler B 1999 ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis. J Clin Invest 103:1379-1389
45. Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, O'Riordan CR, Smith AE 1990 Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63:827-834
46. Pressler T, Jensen ET, Espersen F, Pedersen SS, Høiby N, Koch C 1994 Correlation between specific IgG subclass antibodies to Pseudomonas aeruginosa and opsonic activity in serum from patients with cystic fibrosis. Pediatr Pulmonol 17:31-40
47. Döring G, Krogh-Johansen H, Weidinger S, Høiby N 1994 Allotypes of alpha-1-antitrypsin in patients with cystic fibrosis, homozygous and heterozygous for ΔF508. Pediatr Pulmonol 18:3-7
48. Garred P, Pressler T, Madsen HO, Frederiksen B, Svejgaard A, Høiby N, Schwartz M, Koch C 1999 Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis. J Clin Invest 104:431-437
49. Gabolde M, Guilloud-Bataille M, Feingold J, Besmond C 1999 Association of variant alleles of mannose-binding lectin with severity of pulmonary disease in cystic fibrosis: cohort study. BMJ 319:1166-1167