Non-cirrhotic portal hypertension. 2014

Shiv K Sarin, and Rajeev Khanna
Department of Hepatology, Institute of Liver and Biliary Sciences, D-1 Vasant Kunj, New Delhi 110070, India. Electronic address: shivsarin@gmail.com.

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.

UI MeSH Term Description Entries
D006975 Hypertension, Portal Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN. Cruveilhier-Baumgarten Disease,Cruveilhier-Baumgarten Syndrome,Cruveilhier Baumgarten Disease,Cruveilhier Baumgarten Syndrome,Disease, Cruveilhier-Baumgarten,Portal Hypertension,Portal Hypertensions,Syndrome, Cruveilhier-Baumgarten
D007239 Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Infection,Infection and Infestation,Infections and Infestations,Infestation and Infection,Infestations and Infections
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008103 Liver Cirrhosis Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules. Cirrhosis, Liver,Fibrosis, Liver,Hepatic Cirrhosis,Liver Fibrosis,Cirrhosis, Hepatic
D010198 Pancytopenia Deficiency of all three cell elements of the blood, erythrocytes, leukocytes and platelets. Pancytopenias
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D006439 Hemodynamics The movement and the forces involved in the movement of the blood through the CARDIOVASCULAR SYSTEM. Hemodynamic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000094724 Idiopathic Noncirrhotic Portal Hypertension Portal hypertension without known risk factors for hypertension, e.g., HEPATIC CIRRHOSIS and SCHISTOSOMIASIS. Idiopathic Noncirrhotic Portal Hypertension is most often associated with pathology in the PORTAL SYSTEM vasculature. Banti's Disease,Banti's Syndrome,INCPH,Idiopathic Congestive Splenomegaly,Idiopathic Non-Cirrhotic Portal Hypertension,Idiopathic Portal Hypertension,Noncirrhotic Portal Fibrosis,Porto-Sinusoidal Vascular Diseases,Banti Disease,Banti Syndrome,Congestive Splenomegaly, Idiopathic,Disease, Banti's,Disease, Porto-Sinusoidal Vascular,Diseases, Porto-Sinusoidal Vascular,Fibrosis, Noncirrhotic Portal,Hypertension, Idiopathic Portal,Idiopathic Congestive Splenomegalies,Idiopathic Non Cirrhotic Portal Hypertension,Idiopathic Portal Hypertensions,Noncirrhotic Portal Fibroses,Portal Fibrosis, Noncirrhotic,Portal Hypertension, Idiopathic,Porto Sinusoidal Vascular Diseases,Porto-Sinusoidal Vascular Disease,Splenomegaly, Idiopathic Congestive,Syndrome, Banti's,Vascular Disease, Porto-Sinusoidal
D013163 Splenomegaly Enlargement of the spleen. Enlarged Spleen,Spleen, Enlarged

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