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Factors affecting airway host immune responses. Defective cystic fibrosis transmembrane conductance regulator (CFTR) results in dehydration of the airway surface liquid (ASL), which impairs mucociliary clearance (MCC) and host immune responses, thereby permitting a vicious cycle of airway infection (e.g., Pseudomonas aeruginosa, Pa), lung damage and a hyperinflammatory state in people with cystic fibrosis (PWCF).
