Inhaled nebulized hypertonic saline (HTS) is a commonly prescribed mutation-agnostic
therapy used in the treatment of CF-related lung disease. It is currently well-understood that …

Inhaled hypertonic saline (HTS) treatment is used to improve lung health in patients with
cystic fibrosis (CF). The current consensus is that the treatment generates an osmotic …

Mucus clearance, a primary innate defense mechanism of airways, is defective in patients
with cystic fibrosis (CF) and CF animals. In previous work, the combination of a low dose of …

Respiratory mucociliary clearance (MCC) is a key defense mechanism that functions to
entrap and transport inhaled pollutants, particulates, and pathogens away from the lungs …

One mechanism believed to link CF genotype to phenotype is abnormalities in properties of
the airway surface liquid (ASL). Understanding how important ASL is to airway hydration …

Dehydration of airway surfaces and reduced mucus clearance due to ENaC-mediated
increased airway Na+ absorption plays an important role in the pathogenesis of cystic …

In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR) anion
channel activity causes airway surface liquid (ASL) pH to become acidic, which impairs …

Background Mucus dehydration and impaired mucus clearance are common features of
cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD). In CF, inhaled …

Inhalation of hypertonic saline (HS) acutely enhances mucociliary clearance (MC) in both
health and disease. In patients with cystic fibrosis (CF), repeated use of HS causes a …

Our current understanding of the pathogenesis of cystic fibrosis (CF) lung disease stresses
the importance of the physical and chemical properties of the airway surface liquid (ASL). In …