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

Kasahara M, Sakamoto S, Horikawa R, Koji U, Mizuta K, Shinkai M,Takahito Y, Taguchi T, Inomata Y, Uemoto S, Tatsuo K, Kato S.Living donor liver transplantation for pediatric patients with metabolicdisorders: The Japanese multicenter registry.Abstract: LDLT is indicated for a variety of metabolic disorders,primarily in Asian countries due to the absolute scarcity of deceaseddonor LT. We analyzed data for all pediatric LDLTs performedbetween November 1989 and December 2010, during which 2224pediatric 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 withmetabolic 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%, and79.3%, and 91.2%, 87.9%, 86.1%, and 74.4%, respectively. Wilson’sdisease and urea cycle deficiency were associated with better patientsurvival. The use of heterozygous donors demonstrated no negativeimpact on either the donors or recipients. With regard to X-linkedOTCD, symptomatic heterozygote maternal donors should not beconsidered potential donor candidates. Improving the understanding ofthe long-term suitability of this treatment modality will require theregistration and ongoing evaluation of all patients with inheritedmetabolic disease considered for LT.Mureo Kasahara