Abstract
In maple sirup urine disease febrile infections endanger life due to the associated catabolic state which raise leucine etc. concentrations to toxic levels. The ratio of glucagon/insulin which is closely related to gluconeogenesis (i.e. protein breakdown) in the catabolic state can be depressed by administration of glucose in sufficient amounts. The catabolic state of fever can be favourably influenced by this simple therapy.
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Wick, H., Girard, J. & Stahl, M. Prevention of catabolic state during febrile infection in maple sirup urine disease. Pediatr Res 8, 136 (1974). https://doi.org/10.1203/00006450-197402000-00058
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DOI: https://doi.org/10.1203/00006450-197402000-00058