Non-cirrhotic @entity101 (NCPH) comprises diseases having an increase in portal pressure (PP) due to @entity75 , in the absence of @entity82 . The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of diseases included in the category of NCPH are well-characterized disease entities where @entity101 ( @entity101 ) is a late manifestation and, hence, these are not discussed. Two diseases that present only with features of @entity101 and are common in developing countries are non-cirrhotic portal @entity82 (NCPF) and extrahepatic @entity6315 (EHPVO). Non-cirrhotic portal @entity82 is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to @entity101 , massive @entity381 and well-tolerated episodes of @entity548 in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called @entity28406 ( @entity28406 ) or ' @entity1463 '. Because 85-95% of @entity1 with NCPF and EHPVO present with @entity548 , treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of @entity1 . Gastric varices are another common cause of @entity360 in these @entity1 and these can be managed with @entity3080 glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic @entity6118 . The prognosis of @entity1 with NCPF is good and 5 years survival in @entity1 in whom @entity548 can be controlled has been reported to be approximately 95-100%.

Non-cirrhotic portal XXXX : current concepts and management.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis', 'cirrhosis'] @entity101 :: ('MESH:D006973', 'Disease') :: ['PHT', 'portal hypertension'] @entity548 :: ('MESH:D006470', 'Disease') :: ['variceal bleeding'] @entity75 :: ('MESH:D001927', 'Disease') :: ['intraheptic or prehepatic lesions'] @entity3080 :: ('MESH:D003487', 'Chemical') :: ['cyanoacrylate'] @entity28406 :: ('MESH:C537903', 'Disease') :: ['idiopathic portal hypertension', 'IPH'] @entity1463 :: ('MESH:D012598', 'Disease') :: ['hepatoportal sclerosis'] @entity381 :: ('MESH:D013163', 'Disease') :: ['splenomegaly'] @entity6118 :: ('MESH:D006971', 'Disease') :: ['hypersplenism'] @entity360 :: ('MESH:D006471', 'Disease') :: ['upper gastrointestinal bleeding'] @entity6315 :: ('MESH:C563407', 'Disease') :: ['portal vein obstruction']
@entity82 :: (MESH:D005355,Disease) :: ['fibrosis']
