Hepatol Res. 2017 Apr;47(5):373-386. doi: 10.1111/hepr.12862. Epub 2017 Feb 17.
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.
特发性门静脉高压症（IPH）可导致门静脉血流动力学异常，是一种病因不明且尚无既定治疗方案的疾病。日本特发性门静脉高压症研究小组由厚生劳动省于1975年设立。此后，肝外门静脉阻塞和布加综合征（BCS）也被纳入该小组的研究对象。该研究小组的目标是准确评估这三种疾病在日本的现状，阐明其病因和发病机制，并开发新的治疗方法。由于日本研究小组的长期努力，从流行病学和病理学研究到分子生物学分析等多个方面对门静脉血流动力学异常进行了研究。结果表明，肝脏中存在特发性门静脉高压症特有的异常基因。此外，对布加综合征的病理结果进行了国际比较，明确了日本与欧洲（或北美）在病理结果上的差异。此外，还发现日本布加综合征患者肝细胞癌的并发症发生率较高。基于这些研究，2001年出版了《门静脉血流动力学异常的诊断与治疗（2001）》，其中包括门静脉血流动力学异常的诊断标准。2013年，根据现状纳入诊断和治疗内容后，修订为《门静脉血流动力学异常的诊断与治疗指南（2013）》。
