Background Studies have described illness associated with cystic fibrosis (CF) early in life,
but there is no comprehensive accounting of the prevalence and ages of disease …

Mucus plugging constitutes a nutrient‐rich nidus for a bacterial infection that has long been
recognized as a potent stimulus for neutrophilic airway inflammation driving progressive …

Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

Rationale: Early onset and progression of lung disease in children with cystic fibrosis (CF)
indicates that sensitive noninvasive outcome measures are needed for diagnostic …

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

Background The efficacy, safety, and tolerability of lumacaftor and ivacaftor are established
in patients aged 6 years and older with cystic fibrosis, homozygous for the F508del-CFTR …

Rationale: Cystic fibrosis (CF) lung disease starts in early infancy, suggesting that preventive
treatment may be most beneficial. Lung clearance index (LCI) and chest magnetic …

Rationale: Previous cross-sectional studies have demonstrated that chest magnetic
resonance imaging (MRI) is sensitive to detect early lung disease in infants and preschool …

Background Inhaled hypertonic saline enhances mucociliary clearance, improves lung
function, and reduces pulmonary exacerbations in people with cystic fibrosis older than age …

Summary Background In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7%
hypertonic saline improved the lung clearance index in children aged 3–6 years with cystic …