Cystic fibrosis

Cystic fibrosis is an autosomal recessive genetic disorder that affects ion transport in exocrine glands. Inadequate ion transport causes dehydration and the production of thick secretions in organs such as the lungs, sinuses, pancreas, intestines, hepatobiliary tree, and vas deferens. Although cystic fibrosis usually is diagnosed through a sweat chloride test, medical imaging is used to monitor pathologic changes caused by the disease. This article discusses the respiratory and nonrespiratory manifestations of cystic fibrosis and describes the diagnosis and treatment of the disease. Because patients with cystic fibrosis tend to be young children, the article also covers ways to minimize radiation exposure from repeated imaging examinations.