Living donor liver transplantation for pediatric patients with metabolic disorders: The Japanese multicenter registry
Living donor liver transplantation for pediatric patients with metabolic disorders: The Japanese multicenter registry
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DOI：
10.1111/petr.12196
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发表时间：
2014-02-01
影响因子：
1.3
通讯作者：
Kato, Shunichi
中科院分区：
文献类型：
--
作者：
Kasahara, Mureo;Sakamoto, Seisuke;Kato, Shunichi
LDLT主要用于多种代谢性疾病，主要在亚洲国家，因为死亡供体lt的绝对稀缺。我们分析了1989年11月至2010年12月期间进行的所有儿童LDLT的数据，在此期间，日本有2224名儿童患者接受了LDLT。在这些患者中，194例（8.7%）接受了LDLT治疗代谢紊乱。威尔逊病（n=59, 30.4%）是代谢性疾病患者中最常见的适应症，其次是OTCD （n=40, 20.6%）、MMA （n=20, 10.3%）和GSD （n=15, 7.7%）。1年、5年、10年和15年患者和移植物存活率分别为91.2%、87.9%、87.0%和79.3%,91.2%、87.9%、86.1%和74.4%。威尔逊氏病和尿素循环缺乏与更好的患者生存相关。使用杂合子供体对供体和受体均无负面影响。关于x连锁OTCD，有症状的杂合子母体供体不应被视为潜在的供体候选人。为了提高对这种治疗方式的长期适用性的理解，需要对所有被认为是肝移植的遗传性代谢性疾病患者进行登记和持续评估。
LDLT is indicated for a variety of metabolic disorders, primarily in Asian countries due to the absolute scarcity of deceased donor LT. We analyzed data for all pediatric LDLTs performed between November 1989 and December 2010, during which 2224 pediatric patients underwent LDLT in Japan. Of these patients, 194 (8.7%) underwent LDLT for metabolic disorders. Wilson's disease (n=59; 30.4%) was the most common indication in the patients with metabolic disorders, followed by OTCD (n=40; 20.6%), MMA (n=20; 10.3%), and GSD (n=15; 7.7%). The one-, five-, 10-, and 15-yr patient and graft survival rates were 91.2%, 87.9%, 87.0%, and 79.3%, and 91.2%, 87.9%, 86.1%, and 74.4%, respectively. Wilson's disease and urea cycle deficiency were associated with better patient survival. The use of heterozygous donors demonstrated no negative impact on either the donors or recipients. With regard to X-linked OTCD, symptomatic heterozygote maternal donors should not be considered potential donor candidates. Improving the understanding of the long-term suitability of this treatment modality will require the registration and ongoing evaluation of all patients with inherited metabolic disease considered for LT.