P. A. di Sant'Agnese, M.D.
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Cystic fibrosis of the pancreas is a generalized disorder in which there is a widespread dysfunction of exocrine glands. Abnormalities include such varied findings as an abnormal physicochemical behavior and chemical structure of mucopolysaccharide secretions and an electrolyte abnormality in the composition of eccrine sweat and saliva. The unknown basic defect is genetically transmitted, probably as a recessive trait.
In the homozygotic form, cystic fibrosis with its chronic pulmonary involvement, pancreatic achylia, and, at times, cirrhosis of the liver with portal hypertension is one of the most common and serious of the chronic diseases of children and adolescents. As a
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di Sant'Agnese PA. Cystic Fibrosis of the Pancreas: The Possible Relation of Some of the Clinical Entities of the Adult to the Generalized Disease of Children.. Ann Intern Med. 1962;56:679-680. doi: 10.7326/0003-4819-56-4-679_2
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Published: Ann Intern Med. 1962;56(4):679-680.
Gastroenterology/Hepatology, Pancreatic Disease, Pulmonary/Critical Care.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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