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  • Title: Proton magnetic resonance spectroscopy reflects metabolic decompensation in maple syrup urine disease.
    Author: Heindel W, Kugel H, Wendel U, Roth B, Benz-Bohm G.
    Journal: Pediatr Radiol; 1995; 25(4):296-9. PubMed ID: 7567243.
    Abstract:
    Using localized proton magnetic resonance spectroscopy (1H-MRS), accumulation of branched-chain amino acids (BCAA) and their corresponding 2-oxo acids (BCOA) could be non-invasively demonstrated in the brain of a 9-year-old girl suffering from classical maple syrup urine disease. During acute metabolic decompensation, the compounds caused a signal at a chemical shift of 0.9 ppm which was assigned by in vitro experiments. The brain tissue concentration of the sum of BCAA and BCOA could be estimated as 0.9 mmol/l. Localized 1H-MRS of the brain appears to be suitable for examining patients suffering from maple syrup urine disease in different metabolic states.
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