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

Rationale: The combination of the CFTR (cystic fibrosis transmembrane conductance regulator)
corrector lumacaftor with the potentiator ivacaftor has been approved for the treatment of …

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

Neutrophil elastase (NE) is a key risk factor for severity of cystic fibrosis (CF) lung disease.
Recent studies identified increased NE activity on the surface of airway neutrophils from CF-…

Purpose Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome
coronavirus type 2 (SARS-CoV-2) is currently the major threat for immunocompromised …

Airway dysbiosis has been associated with lung disease severity in patients with cystic fibrosis
(CF). However, the relationship between dysbiosis, airway inflammation and lung function …

… Sabine Wege … Sabine Wege … Wielpütz MO, Eichinger M, Biederer J, Wege S, Stahl M,
Sommerburg O, et al. Imaging of cystic fibrosis lung disease and clinical interpretation. Rofo 2016;…

Airway inflammation and microbiome dysbiosis are hallmarks of cystic fibrosis (CF) lung
disease. However, longitudinal studies are needed to decipher which factors contribute to the …

Background Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical
outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study …

Background Chronic inflammation and remodeling of the airways remain a hallmark of cystic
fibrosis (CF). However, knowledge of the associated mucosal micro-anatomical changes is …