Two consecutive partial liver transplants in a patient with Classic Maple Syrup Urine Disease

Two consecutive partial liver transplants in a patient with Classic Maple Syrup Urine Disease

Maple syrup urine disease is caused by a deficiency in the branched chain ketoacid dehydrogenase (BCKAD) complex. This results in the accumulation of branched chain amino acids (BCAA) and branched chain ketoacids in the body. Even when aggressively treated with dietary restriction of BCAA, patients...

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Bibliographic Details
Main Authors: H.L. Chin, M.M. Aw, S.H. Quak, J. Huang, C.E. Hart, K. Prabhakaran, D.L. Goh
Format: Article
Language:English
Published: Elsevier 2015-09-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Maple syrup urine disease
Liver transplant
Metabolic control
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426915300173

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http://www.sciencedirect.com/science/article/pii/S2214426915300173

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