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Dietary management in glycogen storage disease type III: what is the evidence?

  • Glycogenoses
  • Published:
Journal of Inherited Metabolic Disease

Abstract

In childhood, GSD type III causes relatively severe fasting intolerance, classically associated with ketotic hypoglycaemia. During follow up, history of (documented) hypoglycaemia, clinical parameters (growth, liver size, motor development, neuromuscular parameters), laboratory parameters (glucose, lactate, ALAT, cholesterol, triglycerides, creatine kinase and ketones) and cardiac parameters all need to be integrated in order to titrate dietary management, for which age-dependent requirements need to be taken into account. Evidence from case studies and small cohort studies in both children and adults with GSD III demonstrate that prevention of hypoglycaemia and maintenance of euglycemia is not sufficient to prevent complications. Moreover, over-treatment with carbohydrates may even be harmful. The ageing cohort of GSD III patients, including the non-traditional clinical presentations in adulthood, raises ‬‬‬new questions.

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Abbreviations

EGP:

endogenous glucose production

GSD III:

glycogen storage disease type III

HCM:

hypertrophic cardiomyopathy

IEM:

inborn error of metabolism

UCCS:

uncooked cornstarch

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Acknowledgments

Taking care of GSD-patients is teamwork and the discussions with the colleagues in our department have been very stimulating while preparing the presentation and this subsequent manuscript. Therefore the authors are thankful to Rixt van der Ende, Cynthia van Amerongen, Irene Hoogeveen, Chris Peter Sentner, Esther van Dam, Foekje de Boer, Francjan van Spronsen and Margreet van Rijn, the latter who critically read an early version of the manuscript.

Compliance with Ethics Guidelines

Conflict of interest

The authors received speaker’s fees from Recordati Rare Diseases for this study, which was presented orally at “The changing spectrum of IMD: surviving longer and growing old with IMDs” Recordati Rare Diseases Academy Symposium in London, United Kingdom, 8 May 2014. In addition to that, in the last 5 years, Terry GJ Derks had received speaker’s fees from Danone Nutricia and Vitaflo, research fees from Sigma Tau and Vitaflo, and training support from Sigma Tau and Genzyme. G Peter A Smit declares that he has no conflict of interest.

This article does not contain any studies with human or animal subjects performed by the any of the authors.

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Correspondence to Terry G. J. Derks.

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Presented at “The changing spectrum of IMD: surviving longer and growing old with IMDs” Recordati Rare Diseases Academy Symposium in London, United Kingdom, 8 May 2014.

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Derks, T.G.J., Smit, G.P.A. Dietary management in glycogen storage disease type III: what is the evidence?. J Inherit Metab Dis 38, 545–550 (2015). https://doi.org/10.1007/s10545-014-9756-x

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  • DOI: https://doi.org/10.1007/s10545-014-9756-x

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