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What is A child presents with massive hemetemesis and systemic hypotension. He has no fever or other significant history. Examination reveal massive splenomegaly but no hepatomegaly. Likely diagnosis is and explain in detail?
Answer is D (Non cirrhotic poal fibrosis): NCPF is suspected in a patient presenting with symptoms of poal hypeension with: YOUNG AGE Moderate/Large Splenomegaly NO FEATURES SUGGESTIVE OF LIVER CELL FAILURE -No Jaundice (uncommon) - No Ascitis (uncommon) - No Hepatomegaly (uncommon) - No Stigmata of liver cell failure (uncommon) Non cirrhotic poal fibrosis is a condition of liver characterized by widespread fibrosis of the 1 ver (mainly poal, subcapsular and rarely perisinusuoidal) causing wide variation in the normal architecture but there is no true cirrhosis. NCPF is the most impoant causes of intrahepatic non cirrhotic poal hypeension in India