Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators,
Kalydeco®(ivacaftor), Orkambi®(lumacaftor/ivacaftor) and Symkevi®(tezacaftor/ivacaftor) …

Background: Airway clearance techniques (ACTs) are integral to cystic fibrosis (CF)
management. However, there is no consensus as to which outcome measures (OMs) are …

The availability of highly effective CFTR modulators is revolutionizing the treatment of cystic
fibrosis (CF) and drastically improving outcomes. MRI-based imaging modalities are now …

Purpose To explore the feasibility of measuring ventilation defect percentage (VDP) using
19F MRI during free‐breathing wash‐in of fluorinated gas mixture with postacquisition …

Rationale The clinical significance of Aspergillus fumigatus (Af) detection in the absence of
allergic bronchopulmonary aspergillosis in cystic fibrosis (CF) airways remains unclear. Yet …

Background Children with cystic fibrosis (CF) pulmonary exacerbations receive IV
tobramycin therapy, with dosing guided by either log-linear regression (LLR) or Bayesian …

Background People with cystic fibrosis (PWCF) suffer from acute unpredictable reductions in
pulmonary function associated with a pulmonary exacerbation (PEx) that may require …

Background Pulmonary exacerbations (PExs) are clinically important in people with cystic
fibrosis (CF). Multiple definitions have been used for PEx, and this scoping review aimed to …

Pulmonary exacerbations in people with cystic fibrosis are associated with significant
morbidity and reduced quality of life. Pulmonary exacerbation treatment guidelines …

Background Cystic fibrosis (CF) is a rare, inherited, life-limiting condition predominantly
affecting the lungs, for which there is no cure. The disease is characterized by recurrent …