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桥接肝移植治疗婴儿肠衰竭相关肝病——桥太远了?

Bridging Liver Transplantation in the Treatment of Intestinal Failure Associated Liver Disease in Infants-A Bridge Too Far?

作者信息

Sharif Abubakar, Sharif Khalid, Mirza Darius F, Gupte Girish L

机构信息

Liver Unit (Including Small Bowel Transplantation), Birmingham Women & Children's Hospital, Birmingham B4 6NH, UK.

Liver Transplant Unit, Queen Elizabeth University Hospitals Birmingham, Birmingham B15 2TH, UK.

出版信息

Children (Basel). 2022 May 10;9(5):699. doi: 10.3390/children9050699.

Abstract

Infants with intestinal failure associated liver disease (IFALD) requiring liver and bowel transplant have a high mortality on the transplant waiting list due to the scarcity of the size-matched donor organs. Bridging liver transplantation has been used to allow the children to grow to a reasonable size so that a combined liver and small bowel transplant could be performed in the future. We report on two children with irreversible intestinal failure (ultra-short bowel syndrome secondary to gastroschisis and microvillous inclusion disease) with IFALD who underwent bridging liver transplantation at our institution. Both patients made a good recovery from their initial surgery. One patient died 6 months following surgery from generalized sepsis, and the other patient survived in good condition to undergo a combined liver and small bowel transplant but died a few days post-transplant. In the current era of scarcity of donor organs, this raises an ethical dilemma for the team involved regarding appropriate utilisation of a scarce resource.

摘要

患有肠衰竭相关肝病(IFALD)且需要肝肠移植的婴儿,由于缺乏尺寸匹配的供体器官,在移植等待名单上的死亡率很高。过渡性肝移植已被用于让儿童成长到合适的大小,以便将来能够进行肝小肠联合移植。我们报告了两名患有不可逆肠衰竭(继发于腹裂和微绒毛包涵体病的超短肠综合征)及IFALD的儿童,他们在我们机构接受了过渡性肝移植。两名患者均从初次手术中顺利康复。一名患者术后6个月死于全身性败血症,另一名患者状况良好地存活下来接受了肝小肠联合移植,但在移植后几天死亡。在当前供体器官稀缺的时代,这给相关团队带来了关于稀缺资源合理利用的伦理困境。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bfed/9139533/0fe977777abb/children-09-00699-g001.jpg

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