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  • Title: Reversal of debrancher deficiency myopathy by the use of high-protein nutrition.
    Author: Slonim AE, Weisberg C, Benke P, Evans OB, Burr IM.
    Journal: Ann Neurol; 1982 Apr; 11(4):420-2. PubMed ID: 7049057.
    Abstract:
    A child with debrancher deficiency presented with myopathy, recurrent hypoglycemia, and growth failure. Evidence for enhanced gluconeogenesis was demonstrated by low postabsorptive gluconeogenic plasma amino acids, a marked fall in alanine during fasting, and a substantial rise in plasma glucose following protein ingestion. The patient was treated with high-protein nocturnal intragastric therapy, which resulted in marked improvement in exercise tolerance, muscle strength and mass, electromyographic findings, and growth.
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