Farmer Douglas G, Venick Robert S, McDiarmid Sue V, Duffy John P, Kattan Omar, Hong Johnny C, Vargas Jorge, Yersiz Hasan, Busuttil Ronald W
Departments of Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Ann Surg. 2009 Sep;250(3):484-93. doi: 10.1097/SLA.0b013e3181b480ad.
OBJECTIVE(S): Death occurs in half of all children with fulminant hepatic failure (FHF). Although liver transplantation (LT) is potentially life-saving, there are only a few published series with limited experience. The aim was to examine predictors of survival after LT for FHF.
Between 1984 and 2008, all LT for FHF performed in recipients less than or equal to 18 years of age were analyzed from a prospectively maintained database using 35 demographic, laboratory, and operative variables. Unique calculated variables included creatinine clearance (cCrCl) and Pediatric End-Stage Liver Disease score (PELD). Study end-points were patient and death censored graft survival. Median follow-up was 98 months. Statistical analysis involved the log-rank test and Cox proportional hazards model.
A total of 122 children underwent 159 LTx. Cryptogenic was the primary etiology (70%) and the median age was 53 months. The significant (P < 0.05) univariate predictors of worse graft survival were: recipient age <24 months, cCrCl <60 mL/min/1.73m, PELD >25 points, and warm ischemia time >60 minutes. The significant (P < 0.05) univariate predictors of worse patient survival were: recipient African-American and Asian race, recipient age <24 months, cCrCl <60 mL/min/1.73m, and time from onset jaundice to encephalopathy <7 days. On multivariate analysis, survival was significantly impacted by 4 variables: cCrCl <60 mL/min/1.73m (GRAFT and PATIENT), PELD >25 points (GRAFT), recipient age <24 months (GRAFT), and time from onset jaundice to encephalopathy <7 days (PATIENT). While overall 5- and 10-year survival was 73% and 72% (GRAFT) and 77% and 73% (PATIENT), these were significantly worse when a combination of multivariate risk-factors were present.
This data from a large, single-center experience demonstrates that LT is the treatment of choice for FHF and results in durable survival. Analysis revealed 4 novel outcome predictors. Young children with rapid onset acute liver failure are a high-risk subpopulation. Unique to this study, cCrCl and PELD accurately predicted the end-points. This analysis identifies patient subpopulations requiring early aggressive intervention with LT.
暴发性肝衰竭（FHF）患儿中有半数会死亡。尽管肝移植（LT）可能挽救生命，但已发表的系列报道较少，经验有限。本研究旨在探讨FHF肝移植术后生存的预测因素。
1984年至2008年间，对前瞻性维护数据库中所有接受LT的18岁及以下FHF患者进行分析，纳入35个人口统计学、实验室和手术变量。计算得出的独特变量包括肌酐清除率（cCrCl）和儿童终末期肝病评分（PELD）。研究终点为患者生存及死亡截尾的移植物存活情况。中位随访时间为98个月。统计分析采用对数秩检验和Cox比例风险模型。
共有122例儿童接受了159次肝移植。隐源性是主要病因（70%），中位年龄为53个月。移植物存活情况较差的显著（P<0.05）单因素预测因素为：受者年龄<24个月、cCrCl<60 mL/min/1.73m²、PELD>25分以及热缺血时间>60分钟。患者生存情况较差的显著（P<0.05）单因素预测因素为：受者为非裔美国人和亚洲人种、受者年龄<24个月、cCrCl<60 mL/min/1.73m²以及从黄疸发作到出现脑病的时间<7天。多因素分析显示，4个变量对生存有显著影响：cCrCl<60 mL/min/1.73m²（移植物和患者）、PELD>25分（移植物）、受者年龄<24个月（移植物）以及从黄疸发作到出现脑病的时间<7天（患者）。虽然总体5年和10年生存率分别为移植物73%和72%、患者77%和73%，但当存在多种多因素风险因素时，生存率显著降低。
这项来自大型单中心经验的数据表明，LT是FHF的首选治疗方法，可带来持久生存。分析揭示了4个新的预后预测因素。急性肝衰竭发病迅速的幼儿是高危亚群。本研究特有的是，cCrCl和PELD能准确预测终点。该分析确定了需要早期积极进行肝移植干预的患者亚群。
