Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …

Persistent neutrophilic inflammation is a central feature in both the pathogenesis and
progression of bronchiectasis. Neutrophils release neutrophil serine proteases (NSPs), such …

Background We recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves
the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic …

Background Cystic fibrosis (CF) is a genetic disorder causing poor mucociliary clearance in
the airways and subsequent respiratory infection. The recently approved triple therapy …

Rationale: Pulmonary exacerbations (PEx) remain the most common cause of morbidity,
recurrent hospitalization, and diminished survival in people with cystic fibrosis (PWCF) and …

Rationale: Pharmacological improvement of CFTR function with elexacaftor/tezacaftor/
ivacaftor (ETI) provides unprecedented improvements in lung function and other clinical …

Extract In people with cystic fibrosis (CF), CF transmembrane conductance regulator (CFTR)
protein dysfunction in the lower airways results in dehydrated airway secretions and …

Chronic airway inflammation is a central feature in the pathogenesis of bronchiectasis (BE),
which can be caused by cystic fibrosis (CFBE; hereafter referred to as CF lung disease) and …

Bronchiectasis, originating from the Greek words bronkhia (“airway”) and ektasis
(“distortion”), is a permanent, irreversible airway dilatation described in 1819 by René …

Rationale Pulmonary exacerbations are clinically impactful events that accelerate cystic
fibrosis (CF) lung disease progression. The pathophysiological mechanisms underlying an …