Research in Cystic Fibrosis

Abstract

(First of Three Parts)CYSTIC fibrosis is the most common lethal or semi-lethal genetic disease of peoples of Caucasian descent and is transmitted as an autosomal recessive trait. It affects primarily the exocrine glands, but although mucus-secreting ones are involved pathologically, the serous glands (e.g., eccrine sweat and parotid glands) have no histologic changes, even though their secretions may be abnormal chemically.Clinically, cystic fibrosis is characterized by the triad of chronic pulmonary disease, pancreatic insufficiency and elevated sweat electrolytes. Many other clinical manifestations may be present and complicate the clinical picture. The homozygotes have all, or substantially all, manifestations . . .