Rationale: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal
airways secretions, chronic endobronchial infection, and progressive airway obstruction …

Stenotrophomonas maltophilia is an emerging multidrug-resistant global opportunistic
pathogen. The increasing incidence of nosocomial and community-acquired S. maltophilia …

Background Reducing treatment burden is a priority for people with cystic fibrosis, whose
health has benefited from using new modulators that substantially increase CFTR protein …

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 …

Abbreviations: ADA Americans with Disabilities Act; BMD bone mineral density; BMI body
mass index; CBAVD congenital bilateral absence of the vas deferens; CF cystic fibrosis; CFF …

1. Cough can (a) be an important defense mechanism to help clear excessive secretions
and foreign material from airways;(b) be an important factor in the spread of infection;(c) …

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 …

Rationale: Cystic fibrosis is a recessive genetic disease characterized by dehydration of the
airway surface liquid and impaired mucociliary clearance. As a result, individuals with the …

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 …