An overview of the pathogenesis of cystic fibrosis lung disease.

摘要: The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview the physiologic mechanisms that regulate mucus transport. A major emphasis placed active transport systems airway surface liquid (ASL) volume and, particularly, periciliary (PCL) layer. sequence developed for CF whereby there a depletion PCL reflects combined dysfunctions accelerated Na(+)-dependent absorption and failure to secrete Cl(-). Both are direct consequence missing transmembrane conductance regulator (CFTR) at apical membrane epithelial cells. leads transport, which associated with persistent mucin secretion formation adherent plaques plugs. These plugs become nidus bacterial infections ultimately lead markedly anaerobic luminal environment.