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.
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