title: Domino liver transplantation in maple syrup urine disease: a case report and review of the literature.
abstract: BACKGROUND
  Improved outcomes have expanded the indications for liver transplantation, thus aggravating the already limited supply of donor organs. Domino liver transplantation (DLT) has been one strategy to augment the supply of donor organs in cases of inborn errors of metabolism. One such disease is maple syrup urine disease (MSUD), an inherited disorder of branched-chain amino acid (BCAA) metabolism.
  
  
  METHODS
  We report on the transplantation of a deceased donor liver into a patient with MSUD, and the sequential transplantation of the explanted liver into a patient with hemophilia A, HIV, hepatitis C, and a low priority on the transplant waiting list.
  
  
  RESULTS
  At 30 months, the MSUD recipient has had significant correction of BCAA metabolism on a protein-unrestricted diet and no progression of neuropsychiatric symptoms. The DLT recipient has been cured of hemophilia and has normal BCAA homeostasis. This case provides further evidence that elective orthotopic liver transplantation for MSUD attenuates the disease with restoration of BCAA metabolism, and that DLT in this setting can achieve excellent results in ESLD patients.
  
  
  CONCLUSIONS
  It is possible that domino grafts from patients with MSUD could be used in more conventional recipients, but additional studies and longer-term outcomes are needed to determine the validity of DLT in MSUD.
authors.affiliation: Emory Transplant Center, Emory University, Atlanta, Georgia 30322, USA.
authors.forename: I R
authors.initials: IR
authors.lastname: Badell
authors.forename: S I
authors.initials: SI
authors.lastname: Hanish
authors.forename: C B
authors.initials: CB
authors.lastname: Hughes
authors.forename: W R
authors.initials: WR
authors.lastname: Hewitt
authors.forename: R T
authors.initials: RT
authors.lastname: Chung
authors.forename: J R
authors.initials: JR
authors.lastname: Spivey
authors.forename: S J
authors.initials: SJ
authors.lastname: Knechtle
mesh_terms: D000998:Antiviral Agents / Q000627:therapeutic use; D003029:Coagulants / Q000009:adverse effects; D046148:Donor Selection; D004340:Drug Contamination; D005260:Female; D015658:HIV Infections / Q000188:drug therapy / Q000635:transmission; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006526:Hepatitis C / Q000188:drug therapy / Q000635:transmission; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors / Q000600:supply & distribution; D008297:Male; D008375:Maple Syrup Urine Disease / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D008875:Middle Aged; D013997:Time Factors; D014019:Tissue Donors / Q000600:supply & distribution*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000998:Antiviral Agents; D003029:Coagulants