Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
Division of Gastroenterology, Hepatology and Nutrition.
J Pediatr Gastroenterol Nutr. 2021 Jan 1;72(1):11-17. doi: 10.1097/MPG.0000000000002952.
Metabolic liver diseases (MLDs) are a heterogeneous group of inherited conditions for which liver transplantation can provide definitive treatment. The limited availability of deceased donor organs means some who could benefit from transplant do not have this option. Living related liver transplant (LrLT) using relatives as donors has emerged as one solution to this problem. This technique is established worldwide, especially in Asian countries, with shorter waiting times and patient and graft survival rates equivalent to deceased donor liver transplantation. However, living donors are underutilized for MLDs in many western countries, possibly due to the fear of limited efficacy using heterozygous donors. We have reviewed the published literature and shown that the use of heterozygous donors for liver transplantation is safe for the majority of MLDs with excellent metabolic correction. The use of LrLT should be encouraged to complement deceased donor liver transplantation (DDLT) for treatment of MLDs.
代谢性肝病（MLD）是一组异质性的遗传性疾病，肝移植可以为其提供明确的治疗方法。由于可供使用的已故捐赠者器官数量有限，一些可能受益于移植的患者无法获得这种选择。使用亲属作为供体的活体亲属肝移植（LrLT）已成为解决这一问题的方法之一。这种技术在世界范围内得到了确立，尤其是在亚洲国家，其等待时间更短，患者和移植物的存活率与已故供体肝移植相当。然而，在许多西方国家，活体供体在 MLD 中的利用率较低，这可能是由于担心使用杂合子供体的疗效有限。我们已经回顾了已发表的文献，并表明，对于大多数 MLD 来说，使用杂合子供体进行肝移植是安全的，并且具有出色的代谢纠正效果。应该鼓励使用 LrLT 来补充已故供体肝移植（DDLT）治疗 MLD。
