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Maple syrup urine disease-therapeutic use of insulin in catabolic states

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Abstract

High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and α-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.

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Authors and Affiliations

  1. University Children's Hospital C, University of Düsseldorf, Federal Republic of Germany

    U. Wendel & H. J. Bremer

  2. Institute of Human Genetics, University of Göttingen, Federal Republic of Germany

    U. Langenbeck & Ingrid Lombeck

Authors
  1. U. Wendel
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  2. U. Langenbeck
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  3. Ingrid Lombeck
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  4. H. J. Bremer
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Additional information

With support of the Landesamt für Forschung des Ministeriums für Wissenschaft und Forschung des Landes Nordrhein-Westfalen. U.L. was supported by Deutsche Forschungsgemeinschaft, Bad Godesberg, G.F.R. (SFB 33 and La 217)

Presented in part on the 19th Annual Symposium of the Society for the Study of Inborn Errors of Metabolism, Southampton 9–11. Sept. 1981 and in a short communication in J. Inher. Metab. Dis.

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Wendel, U., Langenbeck, U., Lombeck, I. et al. Maple syrup urine disease-therapeutic use of insulin in catabolic states. Eur J Pediatr 139, 172–175 (1982). https://doi.org/10.1007/BF01377350

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  • DOI: https://doi.org/10.1007/BF01377350

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