Update on pathogenesis of cystic fibrosis lung disease.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Donaldson
Scott H
SH
Department of Medicine, University of North Carolina at Chapel Hill, 27599, USA. scott_donaldson@med.unc.edu
Boucher
Richard C
RC
D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000502:physiology*; D006801:Humans; D017136:Ion Transport / Q000502:physiology*; D009093:Mucus / Q000502:physiology; D020545:Respiratory Mucosa / Q000503:physiopathology
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator