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Glycogen Storage Diseases

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Nutrition Management of Inherited Metabolic Diseases

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Abstract

  • In glycogen storage diseases, there is insufficient use of glycogen resulting in glycogen buildup, or insufficient synthesis of glycogen.

  • There are multiple types of glycogen storage diseases, which can be classified in hepatic and myopathic forms. Types I, III, IV, VI, and IX affect liver primarily.

  • Glycogen storage diseases that also affect gluconeogenetics cause severe lactic acidosis on fasting.

  • Treatment of glycogen storage disease type I should not only focus on management of hypoglycemia but also on prevention of long term complications.

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Author information

Authors and Affiliations

  1. Clinical Genetics and Metabolism, Children’s Hospital Colorado, University of Colorado Denver – Anschutz Medical Campus, 13123 East 17th Avenue, Mailstop 8400, Aurora, CO, 80045, USA

    Johan L. K. Van Hove MD, PhD, MBA

Authors
  1. Johan L. K. Van Hove MD, PhD, MBA
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Corresponding author

Correspondence to Johan L. K. Van Hove MD, PhD, MBA .

Editor information

Editors and Affiliations

  1. Clinical Genetics and Metabolism, Children’s Hospital Colorado, University of Colorado Denver – Anschutz Medical Campus, Aurora, Colorado, USA

    Laurie E. Bernstein

  2. Division of Genetics and Genomics, Boston Children’s Hospital, Boston, Massachusetts, USA

    Fran Rohr

  3. Clinical Genetics and Metabolism, University of Colorado Denver, Children’s Hospital Colorado, Aurora, Colorado, USA

    Joanna R. Helm

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© 2015 Springer International Publishing Switzerland

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Van Hove, J.L.K. (2015). Glycogen Storage Diseases. In: Bernstein, L., Rohr, F., Helm, J. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-14621-8_26

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  • DOI: https://doi.org/10.1007/978-3-319-14621-8_26

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14620-1

  • Online ISBN: 978-3-319-14621-8

  • eBook Packages: MedicineMedicine (R0)

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