Airway irritation
Stimulation of mechano- and
chemoreceptors due to respiratory
discomfort
Frequent Cough with mucoid sputum
Mutations in the CFTR gene
Production thick, sticky mucus
17 y/o girl with cystic fibrosis
Impairs clearance of mucociliary
secretions
Decreased water composition of the
peri-ciliary fluid (dehydrated state)
Impaired Cl- secretion and increased
Na+ absorption
Decreased Cl- diffusion into peri-ciliary
fluid
Accumulation of secretions may obstruct
secretory passages in the airways
Bacterial proliferation
Neutrophil recruitment
Elastase enzyme release
Further tissue damage and
airway irritation
Release of pro-inflammatory cytokines
(IL-8, TNF-ą)
Legends
Associated Risk Factors
Pathogenesis
Manifested signs/symptoms
Pseudomonas aeruginosa which adheres and
colonizes the respiratory epithelium favors the
environment
