大量腹水以及纯合子患儿中来自杂合子α1抗胰蛋白酶（αAT）活体亲属供肝的情况。
Massive ascites and the heterozygous alpha 1 antitrypsin (α AT) living related donor liver in the homozygous child.
作者信息
Khorsandi Shirin E, Thompson Richard, Vilca-Melendez Hector, Dhawan Anil, Heaton Nigel
机构信息
Institute of Liver Studies, King's College Hospital, London, UK.
出版信息
Pediatr Transplant. 2018 Feb;22(1). doi: 10.1111/petr.13075. Epub 2017 Oct 15.
PMID:29034613
Abstract
The following is a short report on the use of a heterozygous (PiMZ) alpha 1 antitrypsin (α1AT) living related donor liver in a homozygous (PiZ) child that was complicated by massive ascites early after transplant. This clinical report is then followed by a brief summary of present knowledge on the α AT protein and management of massive ascites in the pediatric liver transplant recipient.
摘要
以下是一份关于在一名纯合子（PiZ）儿童中使用杂合子（PiMZ）α1抗胰蛋白酶（α1AT）活体亲属供肝的简短报告，该患儿在移植后早期出现大量腹水并发症。本临床报告之后是关于α1抗胰蛋白酶蛋白的现有知识以及小儿肝移植受者大量腹水管理的简要总结。
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