title: Living donor liver transplantation for pediatric patients with metabolic disorders: the Japanese multicenter registry.
abstract: 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.
authors.affiliation: National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Reiko
authors.initials: R
authors.lastname: Horikawa
authors.forename: Umeshita
authors.initials: U
authors.lastname: Koji
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuta
authors.forename: Masato
authors.initials: M
authors.lastname: Shinkai
authors.forename: Yagi
authors.initials: Y
authors.lastname: Takahito
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Inomata
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
authors.forename: Kuroda
authors.initials: K
authors.lastname: Tatsuo
authors.forename: Shunichi
authors.initials: S
authors.lastname: Kato
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006527:Hepatolenticular Degeneration / Q000628:therapy; D006579:Heterozygote; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007564:Japan; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D008659:Metabolic Diseases / Q000401:mortality / Q000628:therapy*; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D012042:Registries; D015996:Survival Rate; D016896:Treatment Outcome; D056806:Urea Cycle Disorders, Inborn / Q000628:therapy; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: liver transplantation; living donor liver transplantation; long-term results; metabolic disease; pediatric liver transplantation