Intractable Rare Dis Res. 2013;2(3):94-97. (DOI: 10.5582/irdr.2013.v2.3.94)

The current clinical aspects of idiopathic portal hypertension.

Tanaka T, Sugawara Y, Kokudo N


Idiopathic portal hypertension (IPH) comprises disorders developing increased portal pressure in the absence of cirrhosis: the clear mechanisms to explain this disease are still not well recognized. IPH usually suggests a benign prognosis, but sometimes is complicated with severe hemorrhage due to ruptured esophageal varices, or massive splenomegaly. Conventional treatments for those complications for patients with cirrhosis usually works when diverted to patients with IPH, although some of those patients might require liver transplantation if the treatment fails. However, there are few consistent treatment strategies for IPH itself, its complications or the indications for liver transplantation. In this mini review, we summarize the clinical manifestations and several potential theories to explain the etiology, as well as the current treatment options for IPH.

KEYWORDS: Idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis, nodular regenerative hyperplasia (NRH), Idiopathic noncirrhotic portal hypertension

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