Congenital Hepatic Fibrosis associated with polycystic kidney disease

Authors

  • Jaimini Natvarlal Patel Santokba Durlabhji Memorial Hospital
  • Shubha Gupta Santokba Durlabhji Memorial Hospital
  • Mansi Faujdar Santokba Durlabhji Memorial Hospital
  • Gajendra Gupta Santokba Durlabhji Memorial Hospital
  • Shweta Chaturvedi Santokba Durlabhji Memorial Hospital

Keywords:

Congenital Hepatic Fibrosis, Portal hypertension, Celiac Disease, Polycystic Kidney Disease,

Abstract

Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder and occurs as a result of ductal plate malformation. Clinically it is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. The exact incidence and prevalence of CHF are not known, but it is a rare disease. This disorder is diagnosed in most patients during childhood or young adulthood. We present the case of 8 year old female with hepatosplenomegaly, hematemesis, melena, bilateral polycystic kidney disease and a histopathological diagnosis of congenital hepatic fibrosis. She had a history of celiac disease. Congenital hepatic fibrosis belongs to the so-called fibropolycystic diseases. Celiac disease is an immune-mediated enteropathy. We describe its association with congenital hepatic fibrosis.

Author Biographies

Jaimini Natvarlal Patel, Santokba Durlabhji Memorial Hospital

DNB Pathology

Shubha Gupta, Santokba Durlabhji Memorial Hospital

MD Pathology

Mansi Faujdar, Santokba Durlabhji Memorial Hospital

MD Pathology

Gajendra Gupta, Santokba Durlabhji Memorial Hospital

MD Pathology

Shweta Chaturvedi, Santokba Durlabhji Memorial Hospital

DNB Pathology

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Published

21-07-2015

How to Cite

1.
Patel JN, Gupta S, Faujdar M, Gupta G, Chaturvedi S. Congenital Hepatic Fibrosis associated with polycystic kidney disease. Ann of Pathol and Lab Med [Internet]. 2015 Jul. 21 [cited 2025 Jan. 15];2(3):C187-191. Available from: https://pacificejournals.com/journal/index.php/apalm/article/view/apalm233

Issue

Vol. 2 No. 3 (2015)

Section

Case Report

License

Copyright (c) 2015 Jaimini Natvarlal Patel, Shubha Gupta, Mansi Faujdar, Gajendra Gupta, Shweta Chaturvedi

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