“Cystic fibrosis (CF) is an autosomal recessive, multisystem disease characterized by altered function of the exocrine glands involving primarily the lungs, pancreas, and sweat glands.” (Connolly, 2004)

Pathophysiology

CF results from a mutation in a certain gene. The genetic defect is the abnormal regulation of the chloride channel activity, which alters the ionic transport of sodium and chloride across the epithelial surfaces of the dermis. A decreased in chloride reabsorption results in a high concentration of sodium and chloride in the sweat of an individual. With CF, there is a blockage in the exocrine gland ducts, which results from thick, viscous secretions. The glands farthest from the ducts eventually undergo fibrosis. (Connolly, 2004)