Cystic Fibrosis (CF): Pathophysiology, Clinical Manifestations, Treatment Advances, and Prognosis
Cystic fibrosis (CF) is a life-limiting, autosomal recessive genetic disorder caused primarily by pathogenic variants in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. CFTR functions as an epithelial chloride/bicarbonate channel that regulates ion and water transport across airway, pancreatic, intestinal, hepatobiliary, and reproductive tissues. Defective CFTR results in decreased chloride secretion and impaired bicarbonate… Read More »