• Scott H Donaldson and Richard C Boucher.
• Department of Medicine, University of North Carolina at Chapel Hill, 27599, USA. scott_donaldson@med.unc.edu
• Curr Opin Pulm Med. 2003 Nov 1; 9 (6): 486-91.

Purpose Of ReviewIt 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 FindingsRecent 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.SummaryWith 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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