Abstract:

Objective To explore the clinical efficacy of domino liver transplantation（DLT）in the treatment of liver diseases in children. Methods This study included 3 groups of 6 patients with DLT，including 2 living donors（LD）and 1 brain death donor，3 MSUD patients and 3 DLT recipients were enrolled. Each group ofDLT donors and recipients underwent surgery at the same time. The left lateral segment was obtained from theliving donor，and the whole liver was obtained from the donation after brain death（DBD）donor，angioplasty wasselectively performed according to the blood vessel conditions of the donor liver. All patients with MSUD had theirliver removed，two patients received living donor liver transplantation（LDLT）and 1 patient received DBD donorliver transplantation. The diseased liver of DLT recipients were removed and livers from MSUD patient’s wereimplanted. Results The liver function of 3 patients with MSUD returned to the normal range within 2 weeks after operation，and the blood flow of transplanted liver was normal. In 3 DLT recipients，bilirubin basically returned tothe normal range within 2 weeks after operation，and the blood flow of the transplanted liver was normal. One of MSUD patients had portal venous anastomotic stenosis after 1 month，it was improved after balloon dilation. A patient

with MSUD had acute cellular rejection after 2 weeks and was improved with steroid bolus therapy. One case ofDLT recipient experienced acute rejection after 1 week and was improved after steroid bolus therapy. One of DLTrecipient developed lung infection after 1 week，the patient stayed in intensive care unit（ICU）for a long time，and was improved with anti-infective treatment. During the follow-up period（8 ～ 16 months），all liver transplant patients and grafts survived. Conclusion It is technically feasible to apply livers of MSUD patients as DLT donors. Favorable outcome has been achieved and it will expand the source of organs to a certain degree.

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摘要：

目的 探讨多米诺肝移植（domino liver transplantation，DLT）治疗儿童肝脏疾病的临床疗效。方法 本研究纳入 3 组 6 例 DLT 患者，包括 2 例活体捐献者（living donor ，LD）及 1 例脑死亡捐献者、3 例 枫 糖 尿 病（maple syrup urine disease，MSUD） 患 者 及 3 例 胆 管 闭 锁（biliary atresia，BA）DLT 受 者。每组 DLT 供受者手术同期进行。活体供者获取左外叶，脑死亡器官捐献者（donation after brain death，DBD）获取全肝，根据供肝血管条件决定是否行血管成型。MSUD 患者均切除全部病肝，其中 2 例分别接受肝左外叶活体肝移植（living donor liver transplantation，LDLT），1 例接受 DBD 供肝移植。DLT 受者切除全部病肝，分别接受 MSUD 患者病肝全肝供肝肝移植。结果 3 例 MSUD 患者术后 2 周内肝功能均降至正常范围，移植肝血流无异常。3 例 DLT 受者术后 2 周内胆红素基本降至正常范围，移植肝血流均正常。其中 1 例 MSUD 患者术后 1 个月后出现门静脉吻合口狭窄，介入下行球囊扩张后好转。1 例 MSUD 患者术后 2 周余因出现急性细胞性排斥反应，激素冲击治疗后好转。1 例 DLT 受者术后 1 周余即出现急性排斥反应，应用激素后好转。1 例 DLT 受者术后 1 周出现肺感染，在重症监护病房（intensive care unit，ICU）停留时间较长，经抗感染治疗后好转。随访期间（8 ～ 16 个月）所有肝移植患者及移植物均存活。结论 应用 MSUD 患者的供肝行 DLT 在技术上是可行的，预后良好，在一定程度上扩大了肝移植供体的来源。

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