Cystic Fibrosis: Pathophysiology, Pharmacotherapy, CFTR Modulators and Emerging Therapeutic Strategies—A Contemporary Review
Cystic fibrosis (CF) is an autosomal recessive multisystem disorder caused by pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Defective CFTR-mediated chloride and bicarbonate transport produces dehydrated epithelial secretions, impaired mucociliary clearance, chronic airway i...