Background: Obstructive airway disease is nonuniformly distributed throughout the bronchial
tree, although the extent to which this occurs can vary among conditions. The multiple …

The cystic fibrosis (CF) drug development pipeline promises many exciting new treatments
for patients with CF, all which will require clinical studies to prove their benefits on CF lung …

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: Implementation of intervention strategies to prevent lung damage in early cystic
fibrosis (CF) requires objective outcome measures that capture and track lung disease …

Background The CFTR modulator tezacaftor/ivacaftor was efficacious and generally safe
and well tolerated in Phase 3 studies in participants≥ 12 years of age with cystic fibrosis …

Hyperpolarised 3He ventilation-MRI, anatomical lung MRI, lung clearance index (LCI), low-
dose CT and spirometry were performed on 19 children (6–16 years) with clinically stable …

Pulmonary exacerbations (PExs) are significant life events in people with cystic fibrosis (CF),
associated with declining lung function, reduced quality of life, hospitalizations, and …

Background Little is known about the role of small airway dysfunction (SAD) and its complex
relation with asthma control and physical activity (PA). Objective To investigate the …

Background Antibiotic treatment for pulmonary symptoms in preschool children with cystic
fibrosis (CF) varies among clinicians. The lung clearance index (LCI) is sensitive to early CF …

Background Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis
(CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI …