A young boy presented with severe hematemesis. On examination, there was no hepatomegaly, Mild splenomegaly is present. Endoscopy shows esophageal varices. The most probable diagnosis –
Correct Answer: Non cirrhotic portal fibrosis
Description: Splenomegaly, variceal bleeding in the absence of hepatomegaly suggest the diagnosis of non-cirrhotic portal fibrosis.
Non-Cirrhotic Portal Fibrosis
Its incidence is very high in India accounts for 15% to 18% of all patients with portal hypertension. Aetiology :
Infection → It was suggested that E.coli can cause NCPF
Exposure to chemicals → Chronic ingestion of Arsenic is incriminated in causing NCPE Vinyl Chloride toxicity can also cause NCPF
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 the spleen
Jaundice & Ascites uncommon and if they do occur it is for a short period of time. Signs
Splenomegaly (usually massive)
The liver usually normal in size
Sometimes mild ascites can be seen.
Endoscopy :
Esophageal varices are detected in over 90% of patients
Rectal varices in 70% of patients.
Category:
Pathology
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