Neutrophil elastase (NE) is a major inflammatory protease released by neutrophils and is
present in the airways of patients with cystic fibrosis (CF), chronic obstructive pulmonary …

Bronchiectasis refers to abnormal dilatation of the bronchi. Airway dilatation can lead to
failure of mucus clearance and increased risk of infection. Pathophysiological mechanisms …

Background Recent studies demonstrated that the triple combination cystic fibrosis
transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor …

Abstract α1-Antitrypsin deficiency (A1ATD) is an inherited disorder caused by mutations in
SERPINA1, leading to liver and lung disease. It is not a rare disorder but frequently goes …

Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that
regulates the transport of ions and the movement of water across the epithelial barrier …

The host inflammatory response in cystic fibrosis (CF) lung disease has long been
recognized as a central pathological feature and an important therapeutic target. Indeed …

Rationale: Chronic obstructive pulmonary disease (COPD) exacerbations are associated
with virus (mostly rhinovirus) and bacterial infections, but it is not known whether rhinovirus …

Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

The immune system is continuously challenged with large quantities of exogenous antigens
at the barriers between the external environment and internal human tissues. Antimicrobial …

Introduction Cystic fibrosis (CF) is a life-threatening inherited disease caused by mutations
in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, an …