Successful experience of liver transplantation in maple syrup urine disease (MSUD)

Introduction: MSUD is an autosomal recessive metabolic disorder characterized by impaired activity of the branched-chain acid dehydrogenase complex. If left untreated, MSUD can result in mental illness and even death. Liver transplantation (LT) has been effective in patients with MSUD. Liver from patients with MSUD can be used for domino LT (DLT).