REVIEW Acute liver failure in Japan: definition, classification, and prediction of the outcome

Abstract Acute liver failure is a clinical syndrome characterized by hepatic encephalopathy and a bleeding tendency due to severe impairment of liver function caused by massive or submassive liver necrosis. Viral hepatitis is the most important and frequent cause of acute liver failure in Japan. The diagnostic criteria for fulminant hepatitis, including that caused by viral infections, autoimmune hepatitis, and drug allergy induced-liver damage, were first established in 1981. Considering the discrepancies between the definition of fulminant hepatitis in Japan and the defi-nitions of acute liver failure in the United States and Eur-ope, the Intractable Hepato-Biliary Disease Study Group established the diagnostic criteria for ‘‘acute liver failure’’ for Japan in 2011, and performed a nationwide survey of patients seen in 2010 to clarify the demographic and clin-ical features and outcomes of these patients. According to the survey, the survival rates of patients receiving medical treatment alone were low, especially in those with hepatic encephalopathy, despite artificial liver support, consisting of plasma exchange and hemodiafiltration, being provided to almost all patients in Japan. Thus, liver transplantation is inevitable to rescue most patients with hepatic encepha-lopathy. The indications for liver transplantation had, until recently, been determined according to the guideline pub-lished by the Acute Liver Failure Study Group in 1996. Recently, however, the Intractable Hepato-Biliary Disease Study Group established a scoring system to predict the outcomes of acute liver failure patients. Algorithms for outcome prediction have also been developed based on data-mining analyses. These novel guidelines need further evaluation to determine their usefulness