Sinha R, Chapman A R, Reid G T, Hayes P C
R Sinha, Scottish Liver Transplant Unit, Royal Infirmary of Edinburgh, Little France, Edinburgh EH16 4SA, UK. Email
J R Coll Physicians Edinb. 2015;45(2):136-40. doi: 10.4997/JRCPE.2015.210.
Fulminant hepatic failure is liver disease that causes encephalopathy within 8 weeks of onset of symptoms or within 2 weeks of onset of jaundice in a patient without prior evidence of liver disease. Autoimmune polyendocrine syndrome type-1 is an autoimmune autosomal-recessive condition causing parathyroid and adrenal insufficiency, alopecia, chronic mucocutaneous candidiasis, ectodermal dystrophy and, rarely, hepatitis. Although the liver can be affected as a consequence of the autoimmune process, the spectrum of disease activity is varied. Autoimmune hepatitis develops in 10-20% of patients and successful liver transplantation has been reported in pediatric patients who failed immunosuppressive treatment. We report fulminant hepatic failure in an adult patient with autoimmune polyendocrine syndrome type-1 who responded to medical treatment and did not require liver transplantation. We highlight the diagnostic scoring system for autoimmune hepatitis and the referral criteria for liver transplantation in fulminant hepatic failure.
暴发性肝衰竭是一种肝脏疾病，在无先前肝脏疾病证据的患者中，症状出现后8周内或黄疸出现后2周内导致肝性脑病。1型自身免疫性多内分泌综合征是一种自身免疫性常染色体隐性疾病，可导致甲状旁腺和肾上腺功能不全、脱发、慢性黏膜皮肤念珠菌病、外胚层营养不良，以及罕见的肝炎。虽然肝脏可能因自身免疫过程而受到影响，但疾病活动范围各不相同。10%-20%的患者会发展为自身免疫性肝炎，对于免疫抑制治疗无效的儿科患者，已有成功进行肝移植的报道。我们报告了一名患有1型自身免疫性多内分泌综合征的成年患者发生暴发性肝衰竭，该患者对药物治疗有反应，无需进行肝移植。我们重点介绍了自身免疫性肝炎的诊断评分系统以及暴发性肝衰竭肝移植的转诊标准。
