背景:多米诺肝移植(DLT)代表了另一种类型的肝脏供体,以扩大供体库。最近关于在患有枫糖浆尿病(MSUD)的儿童中成功进行DLT的报道显示了有希望的长期结果。
方法:这是一项回顾性研究。所有患有MSUD的儿童都与患有终末期肝病(ESLD)或非MSUD代谢疾病的接受者配对。每一对都接受了同时肝移植(LT),其中MSUD受体接受来自活体相关供体的移植物,并将从MSUD供体移植的肝脏移植到各自配对的多米诺骨牌受体.我们在我们的中心报告我们关于DLT技术和结果的经验。
结果:登记了11名MSUD儿童和12名DLT接受者,其中之一是多米诺骨牌肝分裂移植。DLT收件人包括七个ESLD,二丙酸血症(PA),一种糖原贮积病(GSD)1型,一种GSD3型和一种瓜氨酸血症。LT后ICU和住院时间相当(p>0.05)。在平均随访13.5个月和15个月时,MSUD组和DLT接受者的患者和移植物存活率分别为100%和66.6%。与DLT组相比,MSUD组没有死亡。在患有MSUD的儿童中,LT后氨基酸水平迅速恢复正常,并且他们耐受正常的不受限制的饮食。没有血管,胆道,或在移植后期间观察到与移植物相关的并发症。在DLT接受者中未发现MSUD。
结论:DLT具有优异的术后疗效。DLT应该被全世界的移植计划强烈考虑和采用,以避免器官短缺。
Domino liver transplant (DLT) represents another type of liver donor to expand the donor pool. Recent
reports of successful DLT in children with maple syrup urine disease (MSUD) show promising long-term outcomes.
It was a retrospective study. All children with MSUD were paired with either recipients with end-stage liver disease (ESLD) or non-MSUD metabolic disease. Each pair underwent simultaneous liver transplant (LT), where the MSUD recipient received the graft from a living-related donor and the liver explanted from the MSUD donor was transplanted to the respective paired domino recipient. We report our experience regarding the techniques and outcomes of DLT at our center.
Eleven children with MSUD and 12 respective DLT recipients were enrolled, one of which was domino split-liver transplantation. DLT recipients included seven ESLD, two propionic acidemia (PA), one glycogen storage disease(GSD) type-1, one GSD type-3, and one Citrullinemia. Post-LT ICU and hospital stays were comparable (p > .05). Patient and graft survival was 100% and 66.6% in the MSUD group and DLT recipients at a mean follow-up of 13.5 and 15 months. There was no death in the MSUD group as compared to four in the DLT group. The amino acid levels rapidly normalized after the LT in the children with MSUD and they tolerated the normal unrestricted diet. No vascular, biliary, or graft-related complications were seen in the post-transplant period. No occurrence of MSUD was noted in DLT recipients.
DLTs have excellent post-surgical outcomes. DLT should be strongly considered and adopted by transplant programs worldwide to circumvent organ shortage.