Format

Send to

Choose Destination
Curr Opin Pulm Med. 2003 Nov;9(6):486-91.

Update on pathogenesis of cystic fibrosis lung disease.

Author information

1
Department of Medicine, University of North Carolina at Chapel Hill, 27599, USA. scott_donaldson@med.unc.edu

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.

PMID:
14534400
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wolters Kluwer
Loading ...
Support Center