Since the detection of the underlying gene defect, our knowledge of how the genetic
mutations in cystic fibrosis cause lung disease has increased substantially, but we still lack a …

Despite significant advances in treatment strategies targeting the underlying defect in cystic
fibrosis (CF), airway infection remains an important cause of lung disease. In this two-part …

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

Airway infections are a key component of cystic fibrosis (CF) lung disease. Whereas the
approach to common pathogens such as Pseudomonas aeruginosa is guided by a significant …

Background Pulmonary exacerbations are associated with significant lung function decline
from baseline in cystic fibrosis (CF) and it is not well understood why some patients do not …

The lung clearance index (LCI) measured by the multiple breath washout (MBW) test is
sensitive to early lung disease in children with cystic fibrosis. While LCI worsens during the …

Newborn infants, in contrast to adults, dynamically maintain end-expiratory lung volume (EEV)
above relaxation volume. The purpose of this study was to determine at what age children …

Inflammation leads to lung destruction and loss of pulmonary function in patients with cystic
fibrosis (CF). Drugs that modulate the cystic fibrosis transmembrane conductance regulator (…

Background The multiple breath washout (MBW) test may be most useful in tracking disease
progression over time to inform treatment decisions. In the clinical setting, alternative …

Rationale: The extent of the genetic relatedness among Pseudomonas aeruginosa isolates
and its impact on clinical outcomes in the cystic fibrosis (CF) population is poorly understood. …