Journal
CURRENT OPINION IN PULMONARY MEDICINE
Volume 9, Issue 6, Pages 486-491Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/00063198-200311000-00007
Keywords
cystic fibrosis; pathogenesis; airway surface liquid depletion; mucus stasis
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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.
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