Update on pathogenesis of cystic fibrosis lung disease.

Abstract:

PURPOSE OF REVIEW:It has been an ongoing challenge to translate knowledge pertaining to the molecular basis of cystic fibrosis (CF) into a clear understanding of the development of CF lung disease. Various hypotheses have attempted to explain the apparent breach of innate defenses in CF, although a definitive explanation has been elusive. RECENT FINDINGS:Recent data suggest that altered ion transport functions--namely sodium hyperabsorption and reduced chloride secretion--lead to a depletion of airway surface liquid. As a result, the overlying mucus layer may encroach upon cell surfaces and become adherent, thus interfering with cilia-dependent and cough clearance. These static, and ultimately anaerobic, niches provide a favorable environment for the development of bacterial biofilms and persistent infection with Pseudomonas aeruginosa. SUMMARY:With a better understanding of pathogenic steps leading to CF lung disease, we may now be able to direct the development of therapies that will substantially improve disease outcomes.

journal_name

Curr Opin Pulm Med

authors

Donaldson SH,Boucher RC

doi

10.1097/00063198-200311000-00007

keywords:

subject

Has Abstract

pub_date

2003-11-01 00:00:00

pages

486-91

issue

6

eissn

1070-5287

issn

1531-6971

journal_volume

9

pub_type

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