Cystic fibrosis

Alternative names:

mucoviscidosis

Definition:

An inherited disease that affects the respiratory and digestive systems.

Causes, incidence, and risk factors:

Cystic fibrosis affects the exocrine (mucus and sweat) glands of the body and is caused by a defective gene. Thick mucus is formed in the bronchial tree which predisposes the person to chronic lung infections. Many pancreatic enzymes involved in the breakdown and absorption of fats in the intestine are absent causing malabsorption (inadequate absorption of nutrients from the intestinal tract) and malnutrition. About one in 2500 Caucasians is affected and one in 25 is a carrier of the cystic fibrosis gene. It is the most common cause of chronic lung disease in children and young adults, and the most common fatal hereditary disorder affecting Caucasians in the US. Risk factors include a family history of cystic fibrosis or unexplained infant death. The incidence in adults is 2 out of 10,000 people.