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日本特发性门脉高压症(IPH)研究委员会为该病作出定义如下:本病是一原因不明,有脾肿大、贫血、门脉高压,无肝硬化或肝外门脉梗阻的疾病,并已排除血吸虫病、血液病、门脉与肝静脉血栓形成以及先天性肝纤维化.本病在印度称非肝硬化性门脉纤维化(noncirrhotic portal fibrosis,NCPE),在美国称肝门脉硬化(hepato-portal sclerosis).这类病在我国亦并非罕见,但由于国内对门脉高压患者不常规作门脉造影、肝静脉造影以及门脉压、肝静脉嵌入压测定,以致术前很少确诊.腹外科医师仍有遇见这种非肝硬化性门脉高压的可能,如果重视这方面的检查,肯定会找出一定数目的病例.病因学本病的病因尚未完全明了.近年来日本学者Kohno(1982)制作了特发性门脉高压的动物模型,他以非致病性大肠杆菌混悬液及家兔抗血清反复注入被大肠杆菌致敏家兔的门静脉,获得一种与人类IPH相似的肝脏病理学
Japan’s idiopathic portal hypertension (IPH) research committee to define the disease as follows: The disease is an unknown cause, splenomegaly, anemia, portal hypertension, no cirrhosis or extrahepatic portal obstruction disease, And has ruled out schistosomiasis, blood diseases, portal and hepatic venous thrombosis and congenital liver fibrosis.The disease in India called noncirrhotic portal fibrosis (NCPE), in the United States called the portal vein (Hepato-portal sclerosis) .This kind of disease is not uncommon in our country, but due to the domestic routine portal venography, hepatic venography and portal vein pressure, hepatic vein embedding pressure measurement, so that before surgery is very Less diagnosis .Palvage surgeons still have the possibility of meeting this non-cirrhotic portal hypertension, if you pay attention to this inspection, will certainly find a certain number of cases .EtiologyThe cause of the disease is not yet fully understood .In recent years, Japan Kohno (1982) produced an animal model of idiopathic portal hypertension. He repeatedly injected non-pathogenic E. coli suspension and rabbit antiserum into the portal vein of rabbits sensitized to E. coli, IPH similar Dirty pathology