Capron J P
Service d'hépato-gastroentérologie, CHU Nord, Amiens.
Rev Prat. 1990 Jun 1;40(16):1473-8.
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.
导致非肝硬化性肝内门静脉高压症的疾病发生率因患者的地理来源而异。这种情况在欧洲较为罕见。这些疾病可分为两大类，有时相互交织：1）由慢性活动性肝炎或慢性胆汁淤积性疾病（原发性胆汁性肝硬化、原发性硬化性胆管炎、结节病、先天性肝纤维化）引起的纤维化疾病；2）阻塞门静脉（血吸虫病、特发性门静脉高压症、再生结节性增生）、肝内静脉（酒精中毒、药物、辐射、有毒物质）或肝血窦（肝紫癜、淀粉样变性、维生素A过多症、血液病）的血管疾病。这些疾病（某些致病或相关疾病除外）的预后优于肝硬化，因为肝细胞功能在很长一段时间内正常或仅有轻微改变。然而，在大多数情况下，非肝硬化性肝内门静脉高压症的治疗与肝硬化门静脉高压症的治疗相似。
