A 20 Yr old male presented with repeated episodes of hemetemesis. There is no history of jaundice or liver decompensation. On examination the significant findings include splenomegaly (8cms below costal margin), and presence of esophageal varices. There is no ascites or peptic ulceration. The liver function tests are normal. The most likely diagnosis is
Correct Answer: Non-cirrhotic poal fibrosis
Description: Non cirrhotic poal fibrosis Condition of liver characterized by widespread fibrosis of liver(mainly poal, subcapsular and rarely perisinusoidal) causing wide variation in normal architecture There is no true cirrhosis Etiology Chronic ingestion of arsenic, copper, vinyl chloride Clinical features Youny age patient with features of poal hypeension with conspicuous absence of liver cell failure Patient are usually on 2nd or 3rd decade Onset of symptoms is gradual Most common presenting symptom is GI bleed (90% cases) Splenomegaly Jaundice, Hepatomegaly, ascites and stigmata of liver cell failure are uncommon Diagnosis Site of block is smaller branches (3rd or 4th order branches) Ultrasound shows normal splenopoal axis. Withered tree appearance and peripoal fibrosis is seen in NCPF Ref: Blumga 5th edition Pgno : 1099-1105
Category:
Anatomy
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