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Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease

โœ Scribed by Y. Shigematsu; K. Kikuchi; T. Momoi; M. Sudo; Y. Kikawa; K. Nosaka; M. Kuriyama; S. Haruki; K. Sanada; N. Hamano; Y. Suzuki


Publisher
Springer
Year
1983
Tongue
English
Weight
849 KB
Volume
6
Category
Article
ISSN
0141-8955

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## Abstract Individuals with maple syrup urine disease (MSUD) have an inherited metabolic disorder resulting in a deficiency in the branchedโ€chain ketoโ€acid dehydrogenase complex. As a result, these individuals have elevated concentrations of the branchedโ€chain amino acids valine, isoluecine, alloโ€

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