title: Living donor liver transplantation for inborn errors of metabolism - An underutilized resource in the United States.
abstract: Inborn metabolic diseases of the liver can be life-threatening disorders that cause debilitating and permanent neurological damage. Symptoms may manifest as early as the neonatal period. Liver transplant replaces the enzymatically deficient liver, allowing for metabolism of toxic metabolites. LDLT for metabolic disorders is rarely performed in the United States as compared to countries such as Japan, where they report >2000 cases performed within the past two decades. Patient and graft survival is comparable to that of the United States, where most of the studies are based on deceased donors. No living donor complications were observed, suggesting that LDLT is as safe and effective as deceased donor transplants performed in the USA. Increased utilization of living donors in the USA will allow for early transplantation to prevent permanent neurological damage in those with severe disease. Pediatric transplant centers should consider utilizing living donors when feasible for children with metabolic disorders of the liver.
authors.affiliation: Department of Surgery, Stanford University, Palo Alto, CA, USA.
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Pham
authors.affiliation: Department of Pediatrics, Stanford University, Palo Alto, CA, USA.
authors.forename: Gregory M
authors.initials: GM
authors.lastname: Enns
authors.affiliation: Department of Surgery, Stanford University, Palo Alto, CA, USA.
authors.forename: Carlos O
authors.initials: CO
authors.lastname: Esquivel
mesh_terms: D002648:Child; D006801:Humans; D007564:Japan; D016031:Liver Transplantation / Q000706:statistics & numerical data; D019520:Living Donors; D008661:Metabolism, Inborn Errors / Q000601:surgery*; D010372:Pediatrics; D014481:United States
keywords: indications; living donor; metabolic disorders; pediatric transplantation