ALF是少见的疾病。在美国,每年大约发生2000例,尽管如此,它在所有肝病相关死亡中占比高达6%。[7]Hoofnagle JH, Carithers RL Jr, Shapiro C, et al. Fulminant hepatic failure: summary of a workshop. Hepatology. 1995;21:240-252.http://www.ncbi.nlm.nih.gov/pubmed/7806160?tool=bestpractice.com报告显示,因为ALF而行肝脏移植的患者占美国肝移植总数的6%。[8]2007 Annual Report of the U.S. Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients (OPTN/SRTR): Transplant Data 1997-2006. Rockville, MD: Health Resources and Services Administration, Healthcare Systems Bureau, Division of Transplantation; 2007.由于其低发生率和急性表现,在美国肝功能衰竭研究组(ALFSG)成立前,ALF并没有得到很好的前瞻性研究。近 20 年中,美国 30 多个中心的 2000 多名成年患者已经入组了 ALFSG 数据库。[5]Lee WM, Squires RH Jr, Nyberg SL, et al. Acute liver failure: summary of a workshop. Hepatology. 2008;47:1401-1415.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3381946/http://www.ncbi.nlm.nih.gov/pubmed/18318440?tool=bestpractice.com[9]Chung RT, Stravitz RT, Fontana RJ, et al. Pathogenesis of liver injury in acute liver failure. Gastroenterology. 2012;143:e1-e7.http://www.gastrojournal.org/article/S0016-5085%2812%2900959-6/fulltexthttp://www.ncbi.nlm.nih.gov/pubmed/22796239?tool=bestpractice.com[10]Reuben A, Tillman H, Fontana RJ, et al. Outcomes in adults with acute liver failure between 1998 and 2013: an observational cohort study. Ann Intern Med. 2016;164:724-732.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5526039/http://www.ncbi.nlm.nih.gov/pubmed/27043883?tool=bestpractice.com一个单独用于儿童ALF病例的数据库也已经在美国19个中心,加拿大1个中心和英国2个中心建立。成人数据集的分析表明,大多数病例为女性(67%)并且平均年龄为38岁(范围17-79岁)。未行肝移植患者自发性恢复率为45%;25%的患者接受了肝脏移植手术;并且整体病死率为30%。[5]Lee WM, Squires RH Jr, Nyberg SL, et al. Acute liver failure: summary of a workshop. Hepatology. 2008;47:1401-1415.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3381946/http://www.ncbi.nlm.nih.gov/pubmed/18318440?tool=bestpractice.com与过去十年的描述相比,这些报告的结果有所改善,[7]Hoofnagle JH, Carithers RL Jr, Shapiro C, et al. Fulminant hepatic failure: summary of a workshop. Hepatology. 1995;21:240-252.http://www.ncbi.nlm.nih.gov/pubmed/7806160?tool=bestpractice.com部分归因于ALF病因学的变化。另有数据显示,特别是过去十年中,总体短期生存率、无移植生存率和移植后生存率有所改善,这可能是早期发现及重症监护管理中某些改变的结果。[10]Reuben A, Tillman H, Fontana RJ, et al. Outcomes in adults with acute liver failure between 1998 and 2013: an observational cohort study. Ann Intern Med. 2016;164:724-732.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5526039/http://www.ncbi.nlm.nih.gov/pubmed/27043883?tool=bestpractice.com