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The Pathological Changes in the Liver in Cystic Fibrosis of the Pancreas

AMA Am J Dis Child. 1957;93(4):357-369. doi:10.1001/archpedi.1957.02060040359002.
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Once cystic fibrosis of the pancreas had been recognized as a clinical and pathological entity, histological alteration of the liver was found to be a common feature of the total process1-3 with qualitative and quantitative differences. The basic liver lesions described have included liver-cell atrophy, fatty metamorphosis, periportal fibrosis, and proliferation of the bile ducts, often distended by obstructing bile plugs.1-7 In certain more advanced cases, a focal biliary cirrhosis was found, and this has been considered by most to be due to the focal obstructive lesions resulting from the altered and inspissated bile-containing mucus plugs in the dilated terminal radicles.1-3,5,7-13*

This report is a review of the clinical records and postmortem material of 198 autopsied cases of cystic fibrosis of the pancreas. An attempt is made to define the liver changes, to indicate the pathogenesis, and to relate their changes noted to the total clinical picture


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