Kim Jun Suk, Kim Kyung Mo, Oh Seak Hee, Kim Hyun Jin, Cho Jin Min, Yoo Han-Wook, Namgoong Jung-Man, Kim Dae Yeon, Kim Ki-Hun, Hwang Shin, Lee Sung-Gyu
Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
Pediatr Gastroenterol Hepatol Nutr. 2015 Mar;18(1):48-54. doi: 10.5223/pghn.2015.18.1.48. Epub 2015 Mar 30.
Metabolic liver disease (MLD) often progresses to life-threatening conditions. This study intends to describe the outcomes of liver transplantation (LTx) for MLD at a living donor-dominant transplantation center where potentially heterozygous carrier grafts are employed.
We retrospectively evaluated the medical records of 54 patients with MLD who underwent LTx between November 1995 and February 2012 at Asan Medical Center in Seoul, Korea. The cumulative graft and patient survival rates were analyzed according to patient age, and living or deceased donor LTx. Recurrence of the original disease was also investigated.
The post-transplant cumulative patient survival rates at one, five, and 10 years were 90.7%, 87.5% and 87.5%, and the graft survival rates were 88.8%, 85.5%, and 85.5%, respectively. There were no differences in the patient survival rates according to the recipient age, human leukocyte antigen matching, and living or deceased donor LTx. There were also no differences in the patient survival rates between the MLD and the non-MLD groups for children. Recurrence of the original metabolic disease was not observed in any patient during the follow-up period.
Our results suggest that the living donor-dominant transplantation program is well-tolerated in MLD without recurrence of the original MLD using all types of transplantation.
代谢性肝病（MLD）常进展为危及生命的疾病。本研究旨在描述在一个以活体供体为主的移植中心，采用可能为杂合子携带者移植物进行肝移植（LTx）治疗MLD的结果。
我们回顾性评估了1995年11月至2012年2月期间在韩国首尔峨山医学中心接受LTx的54例MLD患者的病历。根据患者年龄、活体或尸体供体LTx分析累积移植物和患者生存率。还对原发病的复发情况进行了调查。
移植后1年、5年和10年的累积患者生存率分别为90.7%、87.5%和87.5%，移植物生存率分别为88.8%、85.5%和85.5%。根据受者年龄、人类白细胞抗原匹配情况以及活体或尸体供体LTx，患者生存率无差异。儿童MLD组和非MLD组的患者生存率也无差异。在随访期间，未观察到任何患者出现原代谢疾病复发。
我们的结果表明，在MLD中，以活体供体为主的移植方案耐受性良好，使用所有类型的移植均未出现原MLD复发。
