European Journal of Pediatrics

, Volume 149, Issue 9, pp 630–633 | Cite as

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

  • M. Hayde
  • K. Widhalm
Metabolic Diseases


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.

Key words

Glycogen storage disease type I Dietary management Uncooked cornstarch 



Glycogen storage disease type I


very low density lipoprotein


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Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • M. Hayde
    • 1
  • K. Widhalm
    • 1
  1. 1.Department of Paediatrics, Division Metabolic DiseasesUniversity of ViennaViennaAustria

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