Non-cirrhotic intrahepatic portal hypertension

The frequency of diseases responsible for non-cirrhotic intrahepatic portal hypertension varies according to the patients' geographical origin. The condition is rare in Europe. These diseases may be classified into two main groups, sometimes intricated: 1) fibrosing diseases due to chronic active hepatitis or to a chronic cholestatic disease (primary biliary cirrhosis, primary sclerosing cholangitis, sarcoidosis, congenital hepatic fibrosis); 2) vascular diseases occluding the portal vein (schistosomiasis, idiopathic portal hypertension, regenerative nodular hyperplasia), the intrahepatic veins (alcoholism, drugs, radiations, toxic substances) or the sinusoids (peliosis, amyloidosis, hypervitaminosis A, blood diseases). The prognosis of these diseases, (apart from that of certain responsible or associated diseases), is better than the prognosis of cirrhosis, since the liver cell functions are normal or little altered for a long time. However, in most cases the treatment of non-cirrhotic intrahepatic portal hypertension is similar to that of cirrhotic portal hypertension.