Objectives Study of currently approved drugs and exploration of future clinical development
pipeline therapeutics for cystic fibrosis, and possible limitations in their use. Methods …

This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

Background Inhaled hypertonic saline acutely increases mucociliary clearance and, in short-
term trials, improves lung function in people with cystic fibrosis. We tested the safety and …

Background Abnormal homeostasis of the volume of airway surface liquid in patients with
cystic fibrosis is thought to produce defects in mucus clearance and airway defense …

The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and
cloning of the CFTR gene more than 21 years ago led to the identification of the structure …

Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic
bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the …

Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

Background Hypertonic saline enhances mucociliary clearance and may lessen the
destructive inflammatory process in the airways. This is an update of a previously published …

Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens.
Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of …

Airway mucus hypersecretion is a feature of a number of severe respiratory diseases,
including asthma, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (CF) …