Living donor liver transplantation for pediatric patients with metabolic disorders: The Japanese multicenter registry

Mureo Kasahara, Seisuke Sakamoto, Reiko Horikawa, Umeshita Koji, Koichi Mizuta, Masato Shinkai, Yagi Takahito, Tomoaki Taguchi, Yukihiro Inomata, Shinji Uemoto, Kuroda Tatsuo, Shunichi Kato

Research output: Contribution to journalArticlepeer-review

74 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)6-15
Number of pages10
JournalPediatric Transplantation
Issue number1
Publication statusPublished - 2014 Feb
Externally publishedYes


  • liver transplantation
  • living donor liver transplantation
  • long-term results
  • metabolic disease
  • pediatric liver transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation


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