Congenital hepatic fibrosis. A disease with diverse manifestations

Fayez K Ghishan, M. K. Younoszai

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

The authors 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
JournalAmerican Journal of Gastroenterology
Volume75
Issue number4
StatePublished - 1981
Externally publishedYes

Fingerprint

Portal Hypertension
Autosomal Dominant Polycystic Kidney
Monozygotic Twins
Esophageal and Gastric Varices
Ascites
Age Groups
Congenital Hepatic Fibrosis
Newborn Infant
Pediatrics

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Congenital hepatic fibrosis. A disease with diverse manifestations. / Ghishan, Fayez K; Younoszai, M. K.

In: American Journal of Gastroenterology, Vol. 75, No. 4, 1981, p. 317-320.

Research output: Contribution to journalArticle

@article{0eaec010fdce48529b2165620cb336c3,
title = "Congenital hepatic fibrosis. A disease with diverse manifestations",
abstract = "The authors 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.",
author = "Ghishan, {Fayez K} and Younoszai, {M. K.}",
year = "1981",
language = "English (US)",
volume = "75",
pages = "317--320",
journal = "American Journal of Gastroenterology",
issn = "0002-9270",
publisher = "Nature Publishing Group",
number = "4",

}

TY - JOUR

T1 - Congenital hepatic fibrosis. A disease with diverse manifestations

AU - Ghishan, Fayez K

AU - Younoszai, M. K.

PY - 1981

Y1 - 1981

N2 - The authors 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.

AB - The authors 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.

UR - http://www.scopus.com/inward/record.url?scp=0019459159&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0019459159&partnerID=8YFLogxK

M3 - Article

C2 - 7196150

AN - SCOPUS:0019459159

VL - 75

SP - 317

EP - 320

JO - American Journal of Gastroenterology

JF - American Journal of Gastroenterology

SN - 0002-9270

IS - 4

ER -