cystic fibrosis (CF)

Inherited metabolic disorder characterized by production of thick, sticky mucus. It is recessive (see recessiveness) and the most common inherited disorder (about 1 per 2,000 live births) in those of European ancestry. Concentrated mucous secretions in the lungs plug the bronchi, making breathing difficult, promoting infections, and producing chronic cough, recurrent pneumonia, and progressive loss of lung function, the usual cause of death. The secretions interfere with digestive enzymes and block nutrient absorption. Abnormally salty sweat is the basis for diagnosis of cystic fibrosis. Treatment includes enzyme supplements, a diet high in calories, protein, and fat, vigorous physical therapy, and antibiotics. Persons with cystic fibrosis once seldom survived beyond childhood; now more than half reach adulthood, though males are usually sterile.