The airway surface liquid (ASL) is a thin sheet of fluid that covers the luminal aspect of the
airway epithelium. The ASL is a site of several first-line host defenses, and its composition is …

Tremendous progress has been made toward achieving pharmacologic restoration of cystic
fibrosis transmembrane conductance regulator (CFTR) function in people with CF (pwCF) …

Defective CFTR biogenesis and activity in cystic fibrosis airways leads to airway dehydration
and impaired mucociliary clearance, resulting in chronic airway infection and inflammation …

The implementation of cystic fibrosis (CF) transmembrane conductance regulator (CFTR)
modulator drugs into clinical practice has been attaining remarkable therapeutic outcomes …

Cystic fibrosis (CF) is a monogenic syndrome determined by over 2000 mutations in the CF
Transmembrane Conductance Regulator (CFTR) gene harbored on chromosome 7. In …

Cystic fibrosis (CF) is an inherited disorder caused by biallelic mutations of the CF
transmembrane conductance regulator (CFTR) gene. Converging evidence suggests that …

ETI treatment reduces inflammatory markers and positive bacterial cultures on BAL in PwCF.
These findings suggest that ETI has a greater impact on chronic infection and inflammation …

Introduction Inflammation appears early in cystic fibrosis (CF) pathogenesis, with specific
elevated inflammatory markers in bronchoalveolar lavage fluid (BALF) correlating with …

The correction of the genetic defects of the cystic fibrosis (CF) transmembrane conductance
regulator (CFTR) protein by the novel generation drugs termed" modulators" is a promising …

Background Cystic fibrosis (CF) is associated with dysregulated immune responses,
exaggerated inflammation and chronic infection. CF transmembrane conductance regulator …