Congenital Hepatic Fibrosis associated with polycystic kidney disease

  • 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,


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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Case Report

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