Interesting Case of the Month - (IGHP, ICOM) - July
ugie
Clinical History-
8-year-old boy known HBsAg positive, presented with hematemesis. UGIE showed features of esophageal varices. LFT Bilirubin 0.8mg/dl, SGOT/ SGPT-70/60 U/l, SAP/ GGT -75/ 40 U/L, Serum Albumin-3.5 mg/ dl. USG abdomen showed coarse liver echotexture? CLD. Liver biopsy was performed for evaluation. From the images below identify the likely disease?
Upper GI Endoscopy Image-
Microscopic Images-
Special Stains-
MT stain showed presence of broad fibrous septae. Orcein stain highlighted HBsAg positive antigen with hepatocytes.
A. Liver Cirrhosis with HBsAg-
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B. Chronic Hepatitis with HBsAg-
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C. Congenital Hepatic Fibrosis with Cirrhosis
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D. Congenital Hepatic Fibrosis with HBsAg-
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Congenital Hepatic Fibrosis is a type of Ductal Plate Malformation. The disease usually presents with features of portal hypertension. Liver synthetic function remains normal unless complicated by features of cholangitis. The disease mimics cirrhosis on histology.