Idiopathic non-cirrhotic portal hypertension.
Hepatology. 2011 May 13;
Authors: Jnl S, Jc GP, Dc V, Hla J
Idiopathic non-cirrhotic portal hypertension (INCPH) is characterized by an increased portal venous pressure gradient in the absence of a known cause of liver disease and portal vein thrombosis. In contrast to the high prevalence of this disorder in India, INCPH is a rare disease in the Western world. The etiology of INCPH can be divided in five categories: chronic infections, exposure to medication or toxins, thrombophilia, immunological disorders and genetic disorders. Multifactorial etiology can also be encountered. Chronic abdominal infection is incriminated as the most important etiological factor in Eastern patients and thrombophilia in the Western patients. The majority of INCPH patients initially present with signs or complications of portal hypertension (mainly variceal bleeding and splenomegaly). These patients usually have preserved liver function. Liver function impairment occurs mainly in the context of intercurrent conditions. INCPH patients are often clinically and radiologically misdiagnosed as liver cirrhosis so that a liver biopsy is indispensable to discriminate cirrhosis from INCPH. The histopathological characteristics of INCPH are heterogeneous, demonstrating overlap between several pathological entities (hepatoportal sclerosis, nodular regenerative hyperplasia and incomplete septal cirrhosis). Even though hemodynamical changes in INCPH patients are not comparable to those in cirrhotics, prophylaxis and treatment of variceal bleeding is recommended to be similar. Anticoagulation therapy must be considered only in patients who develop portal vein thrombosis. INCPH has been considered a disorder with a relatively benign disease course. However, liver failure, hepatic encephalopathy and hepatopulmonary syndrome can occur and are considered indications for liver transplantation. (HEPATOLOGY 2011.).
PMID: 21574171 [PubMed - as supplied by publisher]