Noncirrhotic portal hypertension (noncirrhotic portal hypertension[1].pdf)
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Clin Liver Dis 10 (2006) 627–651 Noncirrhotic Portal Hypertension Shiv Kumar Sarin, MD, DM*, Ashish Kumar, MD, DM Department of Gastroenterology, G B Pant Hospital, Room 201, Academic Block, New Delhi 110 002, India Portal hypertension is characterized by an increase in portal pressure (O10 mm Hg), and could be a result of cirrhosis of the liver or noncirrhotic diseases. Noncirrhotic portal hypertension (NCPH), as it generally is termed, is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow as ‘‘prehepatic,’’ ‘‘hepatic,’’ and ‘‘posthepatic.’’ The ‘‘hepatic’’ causes of NCPH can be subdivided into ‘‘presinusoidal,’’ ‘‘sinusoidal,’’ and ‘‘postsinusoidal’’ (Box 1). Portal pressure is difficult to measure because direct puncture of the deepseated portal vein is impractical. By wedging a catheter into the hepatic vein, the hepatic sinusoidal pressure can be measured. The difference between the wedged hepatic venous pressure (WHVP) and the free hepatic venous pressure gives the hepatic venous pressure gradient (HVPG), which reflects portal pressure in cirrhotic patients (see the article by Groszmann and colleagues elsewhere in this issue). In NCPH, HVPG usually is near normal or is elevated only mildly, and is significantly lower than the portal vein pressure [1–3]. In most of the diseases that are grouped under the category of NCPH (see Box 1), portal hypertension is a late manifestation of the disease. Noncirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO) are two diseases that are common in developing countries and most often present only with features of portal hypertension and not of parenchymal dysfunction (Fig. 1). These will be described here in detail.
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