title: Effects of cornstarch treatment in very young children with type I glycogen storage disease.
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.
authors.affiliation: Department of Paediatrics, University of Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Hayde
authors.forename: K
authors.initials: K
authors.lastname: Widhalm
mesh_terms: D001786:Blood Glucose / Q000032:analysis; D002675:Child, Preschool; D004040:Dietary Carbohydrates / Q000008:administration & dosage*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy*; D006801:Humans; D007223:Infant; D008055:Lipids / Q000097:blood; D008074:Lipoproteins / Q000097:blood; D008297:Male; D003313:Zea mays
chemical_list: D001786:Blood Glucose; D004040:Dietary Carbohydrates; D008055:Lipids; D008074:Lipoproteins