Mucociliary clearance is an important primary innate defense mechanism that protects the
lungs from deleterious effects of inhaled pollutants, allergens, and pathogens. Mucociliary …

Chronic lung disease remains the major cause of morbidity and mortality of cystic fibrosis
(CF) patients. Cftr mutant mice developed severe intestinal obstruction, but did not exhibit …

Rationale: Studies demonstrating early structural lung damage in infants and preschool
children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to …

Rationale: Increased airway Na+ absorption mediated by epithelial Na+ channels (ENaC) is
a characteristic abnormality in the pathogenesis of cystic fibrosis (CF) lung disease …

Cystic fibrosis (CF) is a complex, potentially life-threatening disease that is most effectively
treated through the administration of antibiotics (eg, colistimethate sodium). Chronic …

HCO3− secretion in distal airways is critical for airway mucosal defense. HCO3−/H+
transport across the apical membrane of airway surface epithelial cells was studied by …

Background Noninvasive monitoring of early abnormalities and therapeutic intervention in
cystic fibrosis (CF) lung disease using MRI is important. Lung T1 mapping has shown …

Background: Cystic fibrosis (CF) lung disease starts in the first months of life often before the
onset of clinical symptoms. Multiple breath washout (MBW) detects abnormal lung function …

Recent evidence suggests that inadequate hydration of airway surfaces is a common
mechanism in the pathogenesis of airway mucus obstruction. Inhaled hypertonic saline (HS) …

Increased airway Na+ absorption mediated by the amiloride‐sensitive epithelial Na+
channel (ENaC) is a basic defect in cystic fibrosis (CF) lung disease. Cystic fibrosis is one of …