Diagnosis and treatment guidelines for aberrant portal hemodynamics

Abstract

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.

The clinical impact of macrophage polarity after Kasai portoenterostomy in biliary atresia

“…Postoperative cholangitis was defined as illness with a fever not attributed to other sources and treated with antibiotics. Portal hypertension and thrombocytopenia were defined according to the diagnosis and treatment guidelines for aberrant portal hemodynamics by a Japanese study group ( 18 ). To analyse the correlation between the M2 ratio and postoperative clinical events, we generated ROCs with AUCs of 0.7 or greater as moderate accuracy.…”

Post-allogeneic Hematopoietic Stem Cell Transplantation Portal Hypertension Not Associated with Liver Cirrhosis, Veno-occlusive Disease, or Graft-versus-host Disease

“…IPH causes portal vein pressure elevation due to obstruction of peripheral intrahepatic portal vein branches, which in advanced stages leads to gastrointestinal varices, portal hypertensive gastropathy, ascites, hepatic encephalopathy, and hypersplenism ( 24 ). In contrast, IPH has a relatively good prognosis because it does not lead to cirrhosis and is unlikely to predispose patients to the development of hepatocellular carcinoma ( 13 ). As IPH is often associated with no or only mild liver dysfunction, the absence of hyperbilirubinemia in this case is consistent with IPH and provides strong evidence to exclude VOD or GVHD of the liver.…”

“…No analytical epidemiological studies have examined the etiology of IPH, and the risk factors for its development remain unknown ( 13 ). However, a survey of patients with IPH found myeloproliferative disease in 18% of cases ( 28 ), suggesting that associated coagulation abnormalities may be involved in the development of IPH.…”

“…Hepatic venography did not show “willow-like” findings, which are characteristic in IPH ( Fig. 2H ) ( 13 ).…”