探讨活体肝移植治疗儿童急性肝功能衰竭(ALF)的疗效，并比较其与活体肝移植治疗慢性终末期肝病的疗效差异。

回顾性分析2016年10月至2020年6月复旦大学附属华山医院行活体肝移植治疗的100例儿童受者临床资料，根据原发病将其分为ALF组(17例)和慢性终末期肝病组(83例)。收集受者性别、年龄、体质量、儿童终末期肝病模型(PELD)评分、移植物受者体重比率和血型匹配等一般资料。观察两组受者围手术期并发症和术后远期并发症发生情况以及受者和移植物术后1、3年生存情况。对符合正态分布的计量资料采用成组t检验比较；非正态分布计量资料采用Mann-Whitney U检验比较；计数资料采用χ²检验比较。采用Kaplan-Meier法绘制两组受者生存曲线，并采用log-rank检验比较生存率。P<0.05为差异有统计学意义。

ALF组和慢性终末期肝病组受者PELD评分分别为(33±10)和(17±12)分，差异有统计学意义(t＝5.138，P<0.05)。ALF组受者术后发生围手术期并发症10例(11例次)，包括手术相关外科并发症5例次，感染4例次，急性排斥反应1例次和胆道并发症1例次；术后远期并发症2例。慢性终末期肝病组受者术后发生围手术期并发症41例(41例次)，包括手术相关外科并发症10例次，感染10例次，急性排斥反应10例次，胆道并发症6例次和血管并发症5例次；术后远期并发症7例，其中腔静脉吻合口狭窄2例，门静脉狭窄2例，肠梗阻1例，胆道狭窄1例，EB病毒感染相关移植后淋巴增殖性疾病1例。随访期间，ALF组受者死亡3例，慢性终末期肝病组死亡5例。ALF组受者术后1、3年累积生存率分别为82.4%和82.4%，移植物存活率分别为82.4%和82.4%；慢性终末期肝病组受者术后1、3年累积生存率分别为94.0%和94.0%，移植物存活率分别为94.0%和94.0%。两组受者术后3年累积生存率及术后3年移植物存活率差异均无统计学意义(χ²＝1.251和1.251，P均>0.05)。

活体肝移植能有效改善ALF患儿预后，可取得与肝移植治疗儿童慢性终末期肝病相似的预后。

To explore the effect of living liver transplantation in the treatment of pediatric acute liver failure (ALF), and to compare with the effect of children with chronic end-stage liver disease also treated with living liver transplantation.

The clinical data of 100 pediatric liver transplantation recipients in Huashan Hospital of Fudan University from October 2016 to June 2020 were retrospectively analyzed, and the recipients were divided into ALF group (n=17) and chronic end-stage liver disease group (n=83) according to the primary disease. General data were collected, including sex, age, body mass index, pediatric end-stage liver disease model (PELD) score, graft and recipient weight ratio and blood type matching. The incidence of perioperative complications, postoperative long-term complications, and the survival of the recipients and grafts at 1 and 3 years after transplantation were observed in the two groups. The measurement data conforming to normal distribution were compared by group t test. Non-normal distribution measurement data were compared by Mann-Whitney U test. The counting data were compared with Chi-square test. Kaplan-Meier method was used to draw the survival curve of the two groups, and log-rank test was used to compare the survival rate. A P<0.05 was considered statistically significant.

PELD scores of ALF group and chornic end-stage liver disease group were (33±10) and (17±12), respectively, and the difference was statistically significant (t=5.138, P<0.05). There were 10 recipients (11 cases) with perioperative complications in ALF group, including 5 cases of surgery-related surgical complications, 4 cases of infection, 1 case of acute rejection, and 1 case of biliary tract complication. Long-term complications were reported in 2 recipients. There were 41 recipients (41 cases) with perioperative complications in chronic end-stage liver disease group, including 10 cases of surgery-related surgical complications, 10 cases of infection, 10 cases of acute rejection, 6 cases of biliary tract complications, and 5 cases of vascular complications. There were 7 recipients with postoperative long-term complications, including anastomotic stenosis of vena cava in 2 recipients, portal vein stenosis in 2 recipients, intestinal obstruction in 1 recipient, biliary tract stenosis in 1 recipient, and epstein-barr virus infection secondary post transplant lymphoproliferative disorder after transplantation in 1 recipient. During the follow-up, 3 recipients in the ALF group and 5 recipients in the chronic end-stage liver disease group died. In the ALF group, the 1-year and 3-year cumulative survival rates of recipients were 82.4% and 82.4%, respectively, and the grafts survival rates were 82.4% and 82.4%, respectively. The 1-year and 3-year cumulative survival rates of recipients in the chronic end-stage liver disease group were 94.0% and 94.0%, respectively, and the grafts survival rates were 94.0% and 94.0%, respectively. There was no significant difference in the 3-year cumulative survival rates of recipients and grafts between the two groups (χ²=1.251 and 1.251, all P>0.05).

Living liver transplantation is effective in the treatment of ALF in children and can achieve the same prognosis as chronic end-stage liver disease in children.