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Pediatrics General 02a8a7a8

A 8 yr old boy presents with upper GI bleeding. On examination, he is found to have splenomegaly; there are no signs of ascites, or hepatomegaly; esophageal varices are found on upper GI endoscopy. Most likely diagnosis is:

A
Budd chiari syndrome
B
Cirrhosis
C
Veno-occlusive disease
D
Non cirrhotic poal fibrosis
High-Yield Explanation
The diagnostic criteria of NCPF includes splenomegaly, normal liver function test, esophageal varices (90-95% cases), patent hepatic and poal veins and no evidence of cirrhosis on biopsy. Based on this criteria, the most probable diagnosis in this patient is Non cirrhotic poal fibrosis. Ref: Arun J. Sanyal, Vijay H. Shah (2005), Chapter 25, "Noncirrhotic Poal Hypeension and Poal Vein Thrombosis", In the book, "Poal Hypeension: Pathobiology, Evaluation and Treatment", USA, Pages 411-12 ; Harrison's Internal Medicine, 15th Edition, Page 1759

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