Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …

Rationale: The mechanisms underlying cystic fibrosis (CF) lung disease pathogenesis are
unknown. Objectives: To establish mechanisms linking anion transport with the functional …

In cystic fibrosis (CF), whether cystic fibrosis transmembrane conductance regulator (CFTR)
dysfunction leads to decreased mucociliary clearance and mucus hypersecretion, before …

The mechanisms underlying the development and natural progression of the airway mucus
defect in cystic fibrosis (CF) remain largely unclear. New animal models of CF, coupled with …

Most cystic fibrosis (CF) patients die of lung failure, due to the combined effects of bacterial
infection, neutrophil-mediated inflammation, and airway obstruction by hyperviscous mucus …

Defective expression and function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway …

The aims of this article are to summarize existing knowledge regarding the pathophysiology
of small airways disease in cystic fibrosis (CF), to speculate about additional mechanisms …

Exposed to a diverse array of potentially noxious agents, the respiratory tract is protected by
a highly developed innate defense system. Physiologically regulated epithelial ion and …

Airway mucus obstruction is a key feature of cystic fibrosis (CF) and chronic obstructive
pulmonary disease (COPD). The thin layer of mucus that covers healthy airway surfaces has …

The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely
involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow …