Posts Tagged ‘neurocognitive outcomes’

October 1, 2011

Liver Transplantation for Classical Maple Syrup Urine Disease: Long-Term Follow-Up in 37 Patients and Comparative United Network for Organ Sharing Experience


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Maple syrup urine disease (MSUD; OMIM 248600) is an autosomal recessive disorder characterized by impaired activity of the branched-chain alpha-keto acid dehydrogenase complex.  The resulting accumulated branched-chain L-amino acids (BCAAs) and alpha-keto acids exert neurotoxic effects.  The clinical course is typically punctuated by episodes of ketoacidosis, each potentially life-threatening.   Medical therapy is limited to dietary […]

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