使用杂合子供体进行α1抗胰蛋白酶缺乏症（A1ATD）的肝移植：结果及文献综述。
Liver transplantation for alpha 1 antitrypsin deficiency (A1ATD) using a heterozygous donor: Outcomes and review of the literature.
作者信息
Sood Vikrant, Lee Eliza J, Raghu Vikram, Reyes-Mugica Miguel, Salgado Claudia M, Squires James, Mazariegos George
机构信息
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Dehli, India.
Department of Surgery, Pediatric Transplant Center, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts, USA.
出版信息
Pediatr Transplant. 2023 Jun;27(4):e14488. doi: 10.1111/petr.14488. Epub 2023 Feb 19.
INTRODUCTION
Alpha 1 antitrypsin deficiency (A1ATD) accounts for 21% of all pediatric liver transplants due to metabolic disease in the western world. Donor heterozygosity has been evaluated in adults but not to a recipient with A1ATD.
METHODS
The data of patient were retrospectively analyzed and a literature review performed.
RESULTS
We present a unique case of living related donation from a A1ATD heterozygote female to a child for decompensated cirrhosis due to A1ATD. In the immediate postoperative period, the child had low-alpha 1 antitrypsin levels, but these normalized by 3 months posttransplant. He is currently 19 months post-transplant with no evidence of recurrent disease.
CONCLUSION
Our case provides initial evidence that A1ATD heterozygote donors may be safely used for pediatric patients with A1ATD, thus expanding the donor pool.
引言
在西方世界，α1抗胰蛋白酶缺乏症（A1ATD）占所有因代谢性疾病进行儿科肝移植病例的21%。供体杂合性已在成人中进行评估，但尚未针对患有A1ATD的受者进行评估。
方法
对患者数据进行回顾性分析并进行文献综述。
结果
我们呈现了一个独特的病例，一名A1ATD杂合子女性作为活体亲属供体为一名因A1ATD导致失代偿性肝硬化的儿童进行肝移植。术后即刻，患儿的α1抗胰蛋白酶水平较低，但在移植后3个月恢复正常。目前，他移植后已19个月，无疾病复发迹象。
结论
我们的病例提供了初步证据，表明A1ATD杂合子供体可安全用于患有A1ATD的儿科患者，从而扩大了供体库。
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