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Nutrition Management of Glycogen Storage Disease

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Nutrition Management of Inherited Metabolic Diseases

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References

  1. Berg JM, Tymoczko JL, Stryer L. Biochemistry. 7th ed. New York: W.H. Freeman; 2012. xxxii, 1054, 43, 41, 48 p.

    Google Scholar 

  2. Kishnani PS, Austin SL, Abdenur JE, Arn P, Bali DS, Boney A, et al. Diagnosis and management of glycogen storage disease type 1: a practical guideline of the American College of Medical Genetics and Genomics. Genet Med. 2014;16:1–29.

    Article  Google Scholar 

  3. Baumgartner MR, Saudubray J-M, Walter JH. Inborn metabolic diseases : diagnosis and treatment. Berlin/Heidelberg: Springer: Imprint: Springer; 2016.

    Google Scholar 

  4. Weinstein DA, Steuerwald U, De Souza CFM, Derks TGJ. Inborn errors of metabolism with hypoglycemia: glycogen storage diseases and inherited disorders of gluconeogenesis. Pediatr Clin N Am. 2018;65(2):247–65.

    Article  Google Scholar 

  5. Saavedra H, editor. Management of glycogen storage disease type I & IIIf. Vitaflo metabolic RD summit. San Diego; 2020.

    Google Scholar 

  6. Ross KM, Ferrecchia IA, Dahlberg KR, Dambska M, Ryan PT, Weinstein DA. Dietary management of the glycogen storage diseases: evolution of treatment and ongoing controversies. Adv Nutr. 2020;11(2):439–46.

    PubMed  Google Scholar 

  7. Institute of Medicine (U.S.). Panel on Macronutrients., Institute of Medicine (U.S.). Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein, and amino acids. Washington, DC: National Academies Press; 2005. xxv, 1331 p.

    Google Scholar 

  8. Shah KK, O’Dell SD. Effect of dietary interventions in the maintenance of normoglycaemia in glycogen storage disease type 1a: a systematic review and meta-analysis. J Hum Nutr Diet. 2013;26(4):329–39.

    Article  CAS  Google Scholar 

  9. Derks TG, Martens DH, Sentner CP, van Rijn M, de Boer F, Smit GP, et al. Dietary treatment of glycogen storage disease type Ia: uncooked cornstarch and/or continuous nocturnal gastric drip-feeding? Mol Genet Metab. 2013;109(1):1–2.

    Article  CAS  Google Scholar 

  10. Sidbury JB, Chen YT, Roe CR. The role of raw starches in the treatment of type I glycogenosis. Arch Intern Med. 1986;146(2):370–3.

    Article  CAS  Google Scholar 

  11. Saudubray JM, Van den Berghe G, Walter J. Inborn metabolic diseases: diagnosis and treatment. 5th ed. Berlin: Springer; 2012. xxv, 656 p.

    Book  Google Scholar 

  12. Blau N, Duran M, Gibson KM, Vici C D. Physician’s guide to the treatment and follow-up of metabolic diseases. New York: Springer; 2014.

    Google Scholar 

  13. Dahlberg KR, Ferrecchia IA, Dambska-Williams M, Resler TE, Ross KM, Butler GL, et al. Cornstarch requirements of the adult glycogen storage disease Ia population: a retrospective review. J Inherit Metab Dis. 2020;43(2):269–78.

    Article  CAS  Google Scholar 

  14. Nalin T, Venema K, Weinstein DA, de Souza CF, Perry ID, van Wandelen MT, et al. In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases. J Inherit Metab Dis. 2015;38(3):529–36.

    Article  CAS  Google Scholar 

  15. Vitaflo. A practical guide for overnight use of glycosade in hepatic glycogen storage disease. Bridgewater: Vitaflo USA, LLC; 2020.

    Google Scholar 

  16. Ross KM, Brown LM, Corrado MM, Chengsupanimit T, Curry LM, Ferrecchia IA, et al. Safety and efficacy of chronic extended release cornstarch therapy for glycogen storage disease type I. JIMD Rep. 2016;26:85–90.

    Article  Google Scholar 

  17. Kishnani PS, Austin SL, Arn P, Bali DS, Boney A, Case LE, et al. Glycogen storage disease type III diagnosis and management guidelines. Genet Med. 2010;12(7):446–63.

    Article  CAS  Google Scholar 

  18. Ross KM, Brown L, Corrado MM, Chengsupanimit T, Curry LM, Ferrecchia IA, et al. Safety and efficacy of long-term use of extended release cornstarch therapy for glycogen storage disease types 0, III, VI, and IX. J Nutr Ther. 2015;4(4):137–42.

    CAS  Google Scholar 

  19. Olgac A, Inci A, Okur I, Biberoglu G, Oguz D, Ezgu FS, et al. Beneficial effects of modified Atkins diet in glycogen storage disease type IIIa. Ann Nutr Metab. 2020;76(4):233–41.

    Article  CAS  Google Scholar 

  20. Francini-Pesenti F, Tresso S, Vitturi N. Modified Atkins ketogenic diet improves heart and skeletal muscle function in glycogen storage disease type III. Acta Myol. 2019;38(1):17–20.

    CAS  PubMed  PubMed Central  Google Scholar 

  21. Marusic T, Zerjav Tansek M, Sirca Campa A, Mezek A, Berden P, Battelino T, et al. Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa. Mol Genet Metab Rep. 2020;24:100628.

    Article  Google Scholar 

  22. Steunenberg TAH, Peeks F, Hoogeveen IJ, Mitchell JJ, Mundy H, de Boer F, et al. Safety issues associated with dietary management in patients with hepatic glycogen storage disease. Mol Genet Metab. 2018;125(1–2):79–85.

    Article  CAS  Google Scholar 

  23. Herbert M, Pendyal S, Rairikar M, Halaby C, Benjamin RW, Kishnani PS. Role of continuous glucose monitoring in the management of glycogen storage disorders. J Inherit Metab Dis. 2018;41(6):917–27.

    Article  CAS  Google Scholar 

  24. White FJ, Jones SA. The use of continuous glucose monitoring in the practical management of glycogen storage disorders. J Inherit Metab Dis. 2011;34(3):631–42.

    Article  CAS  Google Scholar 

  25. Daublin G, Schwahn B, Wendel U. Type I glycogen storage disease: favourable outcome on a strict management regimen avoiding increased lactate production during childhood and adolescence. Eur J Pediatr. 2002;161(Suppl 1):S40–5.

    Article  Google Scholar 

  26. Saunders AC, Feldman HA, Correia CE, Weinstein DA. Clinical evaluation of a portable lactate meter in type I glycogen storage disease. J Inherit Metab Dis. 2005;28(5):695–701.

    Article  CAS  Google Scholar 

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Acknowledgments

Thank you to Sandy van Calcar of Oregon Health & Science University for her contributions to this chapter’s content in the first Edition of Nutrition Management of Inherited Metabolic Diseases.

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Authors and Affiliations

  1. Division of Medical Genetics, CHOC Children’s, Orange, CA, USA

    Mary Sowa

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  1. Mary Sowa
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Correspondence to Mary Sowa .

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Editors and Affiliations

  1. Children’s Hospital Colorado, University of Colorado Hospital, Aurora, CO, USA

    Laurie E. Bernstein

  2. Met Ed Co, Boulder, CO, USA

    Fran Rohr

  3. Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, USA

    Sandy van Calcar

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Sowa, M. (2022). Nutrition Management of Glycogen Storage Disease. In: Bernstein, L.E., Rohr, F., van Calcar, S. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-030-94510-7_26

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  • DOI: https://doi.org/10.1007/978-3-030-94510-7_26

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-94509-1

  • Online ISBN: 978-3-030-94510-7

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