Abstract
Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture inherited as autosomal recessive trait. We report a 19 year-old-female admitted to Bangabandhu Sheikh Mujib Medical University with the complaints of lump in upper abdomen since last 13 years and episodes of fever and abdominal pain for same duration. She was diagnosed with hepatic TB on hepatic histology. Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood. Prognosis of congenital hepatic fibrosis is good. Life threatening events in these patients are related with variceal bleeding and episodes of cholangitis. Owing to relatively good liver function these patients tolerate portosystemic shunt surgeries quite well.Though rare, congenital hepatic fibrosis should be included in the differential diagnosis of portal hypertension in early life.
Highlights
Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture, often occurring in association with cystic lesions of the kidney
Though frequently confused with liver cirrhosis[2], congenital hepatic fibrosis do not present with feature of hepatocellular insufficiency.[1]
Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood
Summary
Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture, often occurring in association with cystic lesions of the kidney. Though on occasions the fever was associated with chills and rigors, she did not ever have jaundice or itching, dysuria or hematuria She was diagnosed as a case of chronic liver disease since the age of 5 yrs on the basis of firm hepatomegaly and presence of oesophageal varices grade 2 and she was put on propranolol therapy since . No KF ring was noted in slit lamp examination of cornea and copper studies were normal Her Anti nuclear antibody was positive but with titre of 18.5 U/ ml (normal
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