Cystic fibrosis: a disease of vulnerability to airway surface dehydration.
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the vulnerability of airway surfaces to dehydration and collapse of mucus clearance. This predisposition is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in (i) the absence of CFTR-mediated Cl- secretion and regulation of epithelial Na+ channel (ENaC) function; and (ii) the sole dependence on extracellular ATP to rebalance these ion transport processes through P2 purinoceptor signaling. Recent clinical studies indicate that inhalation of hypertonic saline osmotically draws sufficient water onto CF airway surfaces to provide clinical benefit.
Boucher
Richard C
RC
Cystic Fibrosis Pulmonary Research and Treatment Center and the UNC Virtual Lung Group, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. rboucher@med.unc.edu
D000042:Absorption; D000818:Animals; D001826:Body Fluids; D002712:Chlorides / Q000378:metabolism; D003550:Cystic Fibrosis / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D003681:Dehydration / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology; D009079:Mucociliary Clearance; D009154:Mutation / Q000235:genetics; D012137:Respiratory System / Q000473:pathology* / Q000503:physiopathology; D012964:Sodium / Q000378:metabolism
D002712:Chlorides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D012964:Sodium