Executive summary The past six decades have seen remarkable improvements in health
outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young …

Airway mucus obstruction is a hallmark of many chronic lung diseases including rare genetic
disorders such as cystic fibrosis (CF) and primary ciliary dyskinesia, as well as common lung …

Rationale: The CFTR (cystic fibrosis transmembrane conductance regulator) modulator
combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to improve clinical …

Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis
transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor …

Rationale: The triple-combination regimen elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA)
was shown to be safe and efficacious in children aged 6 through 11 years with cystic fibrosis …

Pulmonary MRI provides structural and quantitative functional images of the lungs without
ionizing radiation, but it has had limited clinical use due to low signal intensity from the lung …

Purpose In this feasibility study, a phase‐resolved functional lung imaging postprocessing
method for extraction of dynamic perfusion (Q) and ventilation (V) parameters using a …

Abstract Background Therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) was recently
approved for adult cystic fibrosis (CF) patients with at least one F508del mutation. However …

Rationale: Previous studies showed that lumacaftor–ivacaftor therapy results in partial
rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a …

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