title: Non-cirrhotic portal hypertension.
abstract: Non-cirrhotic portal hypertension (NCPH) encompasses a wide range of disorders, primarily vascular in origin, presenting with portal hypertension (PHT), but with preserved liver synthetic functions and near normal hepatic venous pressure gradient (HVPG). Non-cirrhotic portal fibrosis/Idiopathic PHT (NCPF/IPH) and extrahepatic portal venous obstruction (EHPVO) are two prototype disorders in the category. Etiopathogenesis in both of them centers on infections and prothrombotic states. Presentation and management strategies focus on repeated well tolerated episodes of variceal bleed and moderate to massive splenomegaly and other features of PHT. While the long-term prognosis is generally good in NCPF, portal biliopathy and parenchymal extinction after prolonged PHT makes outcome somewhat less favorable in EHPVO. While hepatic schistosomiasis, congenital hepatic fibrosis and nodular regenerative hyperplasia have their distinctive features, they often present with NCPH.
authors.affiliation: Department of Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India. Electronic address: shivsarin@gmail.com.
authors.forename: Shiv K
authors.initials: SK
authors.lastname: Sarin
authors.affiliation: Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India.
authors.forename: Rajeev
authors.initials: R
authors.lastname: Khanna
mesh_terms: D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D007239:Infections / Q000150:complications; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology / Q000503:physiopathology; D010198:Pancytopenia / Q000209:etiology / Q000503:physiopathology; D017082:Portal Pressure; D011379:Prognosis; D013163:Splenomegaly / Q000209:etiology / Q000503:physiopathology; D020246:Venous Thrombosis / Q000209:etiology; D000094724:Idiopathic Noncirrhotic Portal Hypertension
keywords: Endotherapy; Extrahepatic portal venous obstruction; Non-cirrhotic portal fibrosis; Portal biliopathy; Portal hypertension; Shunt surgery