Progressive obstructive lung disease secondary to chronic airway infection, coupled with
impaired host immunity, is the leading cause of morbidity and mortality in cystic fibrosis (CF) …

Pseudomonas aeruginosa opportunistically infects the airways of patients with cystic fibrosis
and causes significant morbidity and mortality. Initial infection can often be eradicated …

The advent of Cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a
critical event in the history of cystic fibrosis (CF) treatment. Unlike traditional therapies that …

Rationale: Ivacaftor can greatly improve clinical outcomes in people with cystic fibrosis (CF)
and has been shown to have in vitro antibacterial properties, yet the long-term …

Chronic airway colonisation by Pseudomonas aeruginosa, a hallmark of cystic fibrosis (CF)
lung disease, is associated with increased morbidity and mortality and despite aggressive …

The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …

Highlights•Drug combinations improve efficacy and permit dose reductions of each
drug.•Lowering the dose of combined drugs prevents concentration-associated …

Lumacaftor-ivacaftor is a combination of two small molecule therapies targeting the basic
defect in cystic fibrosis (CF) at a cellular level. It is a precision medicine and its effects are …

Characterized by impaired mucus transport and subsequent enhanced colonization of
bacteria, pulmonary infection causes major morbidity and mortality in patients with cystic …

Major historical advances in cystic fibrosis (CF) respiratory clinical care, including
mechanical airway clearance and inhaled medications, have aimed to address the …