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Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically ificant PHT with preserved liver functions.
Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy.
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Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance.
Download PDF Download. Author links open overlay panel Rajeev Khanna 1 Shiv K. Sarin 2.
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CHF congenital hepatic fibrosis. EHPVO extrahepatic portal venous obstruction. ERCP endoscopic retrograde cholangiopancreatography.
EST endoscopic sclerotherapy. EVL endoscopic variceal ligation. HIV human immunodeficiency virus. HPS hepatopulmonary syndrome. HVPG hepatic venous pressure gradient. INCPH idiopathic non-cirrhotic portal hypertension. IPH idiopathic portal hypertension. LTx liver transplantation. MHE minimal hepatic encephalopathy.
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MRCP magnetic resonance cholangiopancreatography. NCPF non-cirrhotic portal fibrosis. NCPH non-cirrhotic portal hypertension. NRH nodular regenerative hyperplasia.
PHT portal hypertension. PV portal vein.
PVT portal vein thrombosis. QoL quality of life. UVC umbilical vein catheterization.
Keywords Portal hypertension. Recommended articles Citing articles 0. Published by Elsevier B.