Effects of cornstarch treatment in very young children with type I glycogen storage disease
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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 wordsGlycogen storage disease type I Dietary management Uncooked cornstarch
Glycogen storage disease type I
very low density lipoprotein
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