肝移植治疗儿童急性肝衰竭:单中心8年治疗经验

Liver transplantation for acute liver failure in children: 8-year experience in a single center

目的 探讨肝移植治疗儿童急性肝衰竭(ALF)的疗效。方法 收集行肝移植的15例ALF患儿的临床资料进行回顾性分析,统计同期儿童肝移植受者中ALF所占比例,分析ALF患儿肝移植手术特点、术后并发症及治疗转归情况。结果 同期儿童肝移植受者中ALF占2.0%(15/743)。15例ALF患儿均急性起病,多数伴有发热、腹泻、进行性皮肤及巩膜黄染加重。13例患儿术前合并肝性脑病(其中6例为4期脑病),2例患儿术前合并骨髓抑制、粒细胞减少。10例患儿行亲属供肝的活体肝移植,4例行心脏死亡器官捐献(DCD)供肝肝移植,1例行多米诺供肝辅助性肝移植。15例患儿中,12例供、受者血型相同,1例血型相容,2例跨血型。15例患儿中,有10例术后出现并发症。术后并发脑水肿5例,其中4例死于弥漫性脑水肿,另1例为持续植物状态(睁眼昏迷)。术后发生巨细胞病毒(CMV)感染5例,2例术后出现再生障碍性贫血行骨髓移植治疗后生存,1例死于CMV性肝炎、病毒性脑炎,2例死于弥漫性脑水肿。1例患儿术后出现移植物抗宿主病(GVHD),行骨髓移植后死于感染性休克。存活的9例患儿随访期间肝功能良好。结论 肝移植是治疗儿童ALF的有效手段,可以提高患儿的生存率。脑水肿是ALF患儿肝移植术后死亡的主要原因,应积极行降颅压、改善脑代谢及血液净化等治疗。在ALF患儿出现不可逆性神经系统损害前尽早行肝移植手术,有望延长患儿的生存期和改善其长期预后。

Objective To evaluate the efficacy of liver transplantation for acute liver failure(ALF) in children.Methods Clinical data of 15 children with ALF who underwent liver transplantation were collected and retrospectively analyzed. The proportion of ALF among children undergoing liver transplantation during the same period was calculated.The characteristics, postoperative complications and clinical prognosis of ALF children receiving liver transplantation were analyzed. Results In the same period, the proportion of ALF was 2.0%(15/743) among pediatric recipients undergoing liver transplantation. All 15 children had acute onset of ALF, and most of them were accompanied by fever, diarrhea and progressive yellowing of skin and sclera. Thirteen children were complicated with hepatic encephalopathy before operation(6 cases of stage Ⅳ hepatic encephalopathy), and two children were complicated with myelosuppression and granulocytopenia before liver transplantation. Ten children underwent living donor liver transplantation with relative donor liver, 4 received liver transplantation from donation after cardiac death(DCD), and 1 underwent Domino donor-auxiliary liver transplantation. Of 15 children, 12 recipients had the same blood type with their donors, 1 recipient had compatible blood type with the donor and 2 cases had different blood type with their donors. Among 15 children, 10 cases developed postoperative complications. Postoperative cerebral edema occurred in 5 cases, of whom 4 cases died of diffuse cerebral edema, and the remaining case was in a persistent vegetative state(eyes-open coma). Postoperative cytomegalovirus(CMV) infection was seen in 5 cases. Two children presented with aplastic anemia and survived after bone marrow transplantation, 1 case died of CMV hepatitis and viral encephalitis, and 2 cases died of diffuse brain edema. One child developed graft-versus-host disease(GVHD)after liver transplantation, and died of septic shock after bone marrow transplantation. Nine children survived and obtained favorable liver function during postoperative follow-up. Conclusions Liver transplantation is an efficacious treatment for ALF in children, which may enhance the survival rate. Brain edema is the main cause of death in ALF children following liver transplantation, and treatment such as lowering intracranial pressure, improving brain metabolism and blood purification should be actively performed. Liver transplantation should be promptly performed prior to the incidence of irreversible neurological damage in ALF children, which might prolong the survival and enhance long-term prognosis.