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Congenital Hepatic Fibrosis

Arch Surg. 1965;91(6):884-886. doi:10.1001/archsurg.1965.01320180018005.
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CONGENITAL hepatic fibrosis is a rare cause of intrahepatic portal hypertension in children or young adults. Hepatic function is well preserved, as in extrahepatic obstruction, making these patients acceptable candidates for surgical decompression of their portal hypertension. Two such siblings, each having congenital hepatic fibrosis, a boy age 4 and a girl age 7 are presented.

Report of Cases 

Case 1.  —A 7-year-old white girl was admitted on March 15, 1962. Her first episode of hematemesis occurred in 1958. Since then bleeding occurred at about monthly intervals. A total of 30 units of whole blood had been given as replacement. Ten months prior to admission a splenectomy had been done without influencing the recurrence of bleeding. There was no history of hepatitis or jaundice. Prenatal course and delivery had been normal. There was no history of a similar bleeding tendency or of renal disease in the families of either parent.


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