Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
Defective expression and function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway
mucus hypersecretion, inflammation and infection that begin early in life and lead, at an
advanced stage of the disease, to severe airway obstruction with hyperviscous and
adhesive airway mucus. Whether the abnormalities of airway mucus are already present at
birth before infection is debatable. In CF, the impaired Cl− and HCO3− secretion associated …