Mutations in the CFTR gene A
Impaired Cl- secretion and increased
Na+ absorption Man
Decreased Cl- diffusion into peri-ciliary
fluid
Decreased water composition of the
peri-ciliary fluid (dehydrated state)
Production thick, sticky mucus
Impairs clearance of mucociliary
secretions
Accumulation of secretions may obstruct
secretory passages in the airways
Pseudomonas aerugino
colonizes the respirator
environ
Airway irritation Bacterial proliferation
Stimulation of mechano- and Release of pro-inflammatory cytokines