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Effects of cornstarch treatment in very young children with type I glycogen storage disease

  • Metabolic Diseases
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Abstract

Three children aged 1–2 years with glycogenosis type I were treated with 2 g/kg bodyweight oral cornstarch per meal (4–5 times a day) for a period up to 16 months. In comparison to the previous dietary regimen (day and nocturnal feedings every 3 h) the cornstarch diet stabilised serum glucose profiles and dramatically improved secondary hyperlipoproteinaemia. Mean total triglycerides decreased up to one half, consistent with a fall of very low density lipoprotein-triglycerides up to two thirds. Metabolic acidosis and hyperuricaemia did not occur and normal growth rates (0.7–1 cm/month) were achieved. We conclude that the cornstarch regimen even in the age group up to 2 years can be considered as an efficient alternative in the treatment of glycogenosis type I patients with less frequent feedings and without nocturnal infusion.

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Abbreviations

GSD-I:

Glycogen storage disease type I

VLDL:

very low density lipoprotein

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Authors and Affiliations

  1. Department of Paediatrics, Division Metabolic Diseases, University of Vienna, Währinger Gürtel 18-20, A-1090, Vienna, Austria

    M. Hayde & K. Widhalm

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  1. M. Hayde
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  2. K. Widhalm
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Hayde, M., Widhalm, K. Effects of cornstarch treatment in very young children with type I glycogen storage disease. Eur J Pediatr 149, 630–633 (1990). https://doi.org/10.1007/BF02034750

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  • DOI: https://doi.org/10.1007/BF02034750

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