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Domino liver transplantation in maple syrup urine disease†
Article first published online: 20 APR 2006
Copyright © 2006 American Association for the Study of Liver Diseases
Volume 12, Issue 5, pages 876–882, May 2006
How to Cite
Khanna, A., Hart, M., Nyhan, W. L., Hassanein, T., Panyard-Davis, J. and Barshop, B. A. (2006), Domino liver transplantation in maple syrup urine disease. Liver Transpl, 12: 876–882. doi: 10.1002/lt.20744
There was no role of the study sponsor in study design, collection, analysis, or interpretation of data, in the writing of the report, or in the decision to submit the paper for publication. None of the authors have any financial or personal relationships that could be construed as conflicts of interest.
- Issue published online: 20 APR 2006
- Article first published online: 20 APR 2006
- Manuscript Accepted: 18 JAN 2006
- Manuscript Received: 2 AUG 2005
- UCSD General Clinical Research Center. Grant Number: M01 RR00827
Liver transplantation has been reported in a few cases of maple syrup urine disease (MSUD), but is controversial. Many patients with approved indications for liver transplantation die before grafts are available. A 25-yr-old man with MSUD underwent liver transplantation, and his liver was used as a domino graft for a 53-yr-old man with hepatocellular carcinoma who had low priority on the liver transplant waiting list and was unlikely to survive until routine organ procurement. Both transplants were performed as “piggy back” procedures, reconstructing the domino graft with caval segments from the cadaveric donor. Neither required veno-venous bypass. Whole body leucine oxidation was estimated by 13CO2 in breath after oral boluses of L-[1-13C]-leucine, before and after transplantation in both patients and a control subject. The surgical outcome was successful. The patient with MSUD had marked decreases in plasma branched-chain amino acids (BCAAs) and alloisoleucine (from 255 ± 66 to 16 ± 7 μmol/L), despite advancement of dietary protein from 6 to >40 gm/day. The domino recipient maintained near-normal levels of plasma amino acids with no detectable alloisoleucine on unrestricted diet. Leucine oxidation increased in the patient with MSUD (from 2.2 to 5.6% recovered in 4 hours) and decreased in the recipient (from 9.7 to 6.2%). Neither patient demonstrated any apparent symptoms of MSUD over more than 7 months. In conclusion, liver transplantation substantially corrects whole body BCAA metabolism in MSUD and greatly attenuates the disease. Livers from patients with MSUD may be considered as domino grafts for patients who might otherwise not survive until transplantation. Liver Transpl 12:876–882, 2006. © 2006 AASLD.