The clinical face of cystic fibrosis (CF) has significantly changed over the 25 years since its
basic genetic defect was discovered (1). Increasing numbers of patients are diagnosed at …

The predominant feature of cystic fibrosis (CF) is muco-obstructive lung disease. Loss of or
deficiency of cystic fibrosis transmembrane receptor (CFTR) activity results in decreased …

Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …

Historically, airway inflammation has been recognized to play a key role in the pathogenesis
of pulmonary disease progression in cystic fibrosis (CF). Inflammatory markers are elevated …

In cystic fibrosis (CF), absent or dysfunctional CF transmembrane conductance regulator
(CFTR) on the surface of airway epithelial cells causes abnormal mucociliary clearance …

Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a
description of CF lung disease pathophysiology unifying superficial epithelial and …

Cystic fibrosis (CF) airway disease results from the complex interactions of 3 pathologic
processes:(1) primary airway epithelial cell deficiency in CF transmembrane conductance …

Cystic fibrosis (CF) is a recessive genetic disease that is characterised by airway mucus
plugging and reduced mucus clearance. There are currently alternative hypotheses that …

Background The cystic fibrosis transmembrane conductance regulator (CFTR) modulator
elexacaftor/tezacaftor/ivacaftor (E/T/I) is highly effective clinically for those with at least one …