Congenital Hepatic Fibrosis: A Disease with Divers Manifestations

FAYEZ K. GHISHAN, M. KABIR YOUNOSZAI

Research output: Contribution to journalArticle

17 Scopus citations

Abstract

We present four pediatric patients with histologically proven congenital hepatic fibrosis. The patients had diverse manifestations. The first two patients were seven‐year old identical twins who presented with hepatosplenomegaly and were found to have portal hypertension with esophageal varices. The third patient was a newborn who had intractable ascites, secondary to portal hypertension. The fourth patient was a seven‐year old with adult type polycystic disease of the kidney but no evidence of portal hypertension. Contrary to what has been reported in the literature of the appearance of portal hypertension in the late childhood period, congenital hepatic fibrosis may present in any age group with portal hypertension.

Original languageEnglish (US)
Pages (from-to)317-320
Number of pages4
JournalThe American Journal of Gastroenterology
Volume75
Issue number4
DOIs
StatePublished - Apr 1981
Externally publishedYes

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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