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August 4, 1956


JAMA. 1956;161(14):1388. doi:10.1001/jama.1956.02970140044012

Cystic fibrosis is a hereditary disease seen in children in which many or all of the exocrine glands are affected.1 It is rare in Negroes and never seen in Mongolians but occurs in about one in 600 live births in the white population. In most but not all infants with this disease, pancreatic deficiency is present from birth, as shown by a marked decrease or absence of amylase, lipase, and protease in the duodenal fluid. This is due to a plugging of the pancreatic ducts by amorphous eosinophilic concretions assumed to be highly concentrated normal secretions. As a result ingested food passes through the digestive tract largely undigested. The patient is usually hungry but, despite an adequate food intake, is undernourished. The stools are copious, greasy, and foul-smelling but not watery. Dammedback secretions appear as cystic distentions in the pancreatic ducts, and the parenchyma becomes atrophic and fibrotic.