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Portal Hypertension in Cystic Fibrosis

Gordon K. Danielson, MD; George Clayton Kyle, MD; Robert Denton, MD
JAMA. 1966;195(3):217-220. doi:10.1001/jama.1966.03100030111036.
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CYSTIC FIBROSIS (CF) is a hereditary disease in which there is a generalized abnormality of the function of the exocrine glands.1 It is now recognized that in addition to the pancreas, there may be involvement of the respiratory tract, salivary glands, sweat glands, and liver. The commonly noted histological changes in the liver of focal biliary cirrhosis are usually of little importance,1,2 but the less frequent multilobular biliary cirrhosis (MBC) may be associated with portal hypertension and any combination of hypersplenism, gastrointestinal bleeding, and ascites.2 This report describes two patients with portal hypertension and bleeding esophageal varices who were successfully treated by portacaval shunting.

Report of Cases 

Case 1.—  A 13-year-old white boy was admitted to the hospital in November 1964 with the chief complaint of having vomited more than a cup of blood several hours before. Though there was a history of poor weight gain, frequent


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