A young boy presented with severe hematemesis. 0/E there was no hepatomegaly, Mild splenomegaly is present. Endoscopy shows esophageal varices. The most probable diagnosis -

Ans. is `d' i.e., Non Cirrhotic poal fibrosisSplenomegaly, variceal bleeding in the absence of hepatomegaly suggest the diagnosis of non cirrhotic poal fibrosis. Non Cirrhotic Poal Fibrosis o Its incidence is very high in India accounts for 15% to 18% of all patients with poal hypeension. Aetiology :(i) Infection ---> It was suggested that E.coli can cause NCPF(ii) Exposure to chemicals --> Chronic ingestion ofArsenic is incriminated in causing NCPF.Vinyl Chloride toxicity can also cause NCPFo Clinical features :Symptoms :Patients are usually young in 3rd or 4th decade of life.Gastrointestinal haemorrhage is the most common presenting symptom seen in 60-90% of patients.Lump in the left side of Abdomen d/t enlargement of spleenJaundice & Ascites uncommon and if they do occur it is for a sho period of time.SignsSplenomegaly (usually massive)Liver usually normal in sizeSometimes mild ascites can be seen.Endoscopy :Oesophageal varices are detected in over 90% of patientsRectal varices in 70% of patients.