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- W2172131043 abstract "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 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.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." @default.
- W2172131043 created "2016-06-24" @default.
- W2172131043 creator A5001166056 @default.
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- W2172131043 date "2003-11-01" @default.
- W2172131043 modified "2023-10-17" @default.
- W2172131043 title "Update on pathogenesis of cystic fibrosis lung disease" @default.
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- W2172131043 doi "https://doi.org/10.1097/00063198-200311000-00007" @default.
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