Living related versus deceased donor liver transplantation for maple syrup urine disease.

Abstract

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain ketoacid (BCKA) oxidation associated with episodic and chronic brain disease. Transplantation of liver from an unrelated deceased donor restores 9-13% whole-body BCKA oxidation capacity and stabilizes MSUD. Recent reports document encouraging short-term outcomes for MSUD patients… (More)
DOI: 10.1016/j.ymgme.2016.01.005

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