title: Liver transplantation for metabolic liver disease: experience at a living donor dominant liver transplantation center.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Jun Suk
authors: JS
authors: Kim
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Kyung Mo
authors: KM
authors: Kim
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Seak Hee
authors: SH
authors: Oh
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Hyun Jin
authors: HJ
authors: Kim
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Jin Min
authors: JM
authors: Cho
authors: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Han-Wook
authors: HW
authors: Yoo
authors: Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Jung-Man
authors: JM
authors: Namgoong
authors: Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Dae Yeon
authors: DY
authors: Kim
authors: Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Korea.
authors: Ki-Hun
authors: KH
authors: Kim
authors: Division of Hepato-Biliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors: Shin
authors: S
authors: Hwang
authors: Division of Hepato-Biliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors: Sung-Gyu
authors: SG
authors: Lee
keywords: Adult; Child; Liver transplantation; Metabolic diseases