• Acta Paediatr Jpn · Apr 1991

    1H-NMR studies of urine in propionic acidemia and methylmalonic acidemia.

    • S Kodama, M Sugiura, H Nakao, K Kobayashi, M Miyoshi, K Yoshii, M Komatsu, and T Sakurai.
    • Department of Pediatrics, Himeji Red Cross Hospital, Japan.
    • Acta Paediatr Jpn. 1991 Apr 1;33(2):139-45.

    AbstractThe present study deals with the usefulness of 1H-NMR spectroscopy in the chemical diagnosis of propionic acidemia and methylmalonic acidemia. A Varian VXR-500 spectrometer was used. 1H-NMR spectra of urine from a patient with propionic acidemia show peaks of 3-hydroxy-n-valerate, methylcitrate, propionyl glycine, 3-hydroxy-n-butyrate, lactate, 3-hydroxypropionate, tiglylglycine and hippurate. The assignment was made possible by the use of 2D COSY. The spectra from a patient with methylmalonic acidemia show methylmalonate and glycine. 1H-NMR spectroscopy has the advantages of requiring a short time for analysis, relatively small amounts of urine and no preliminary extraction.

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