This study was undertaken to test the glycemic response of five infants with
glycogen storage disease type 1, aged 0.7 to 1.5 years, to uncooked
cornstarch under various dietary conditions, and to evaluate the long-term effects of a dietary regimen consisting of uncooked
cornstarch in milk every 4 hours, in addition to three meals daily, on biochemical values and physical growth. The results were compared with previous experience in treating six infants with continuous overnight
glucose infusion via
gastrostomy plus multiple daily feedings containing an adequate source of
glucose. A test dose of
cornstarch (1.6 to 1.8 gm/kg) providing four times the calculated hourly
glucose production rate, when given in water 15 to 30 minutes after a continuous overnight intragastric
glucose infusion was stopped, did not maintain normoglycemia. When the same dose was given in 2% cow milk 4 hours later, mean
blood glucose levels remained greater than 68 mg/dl (3.8 mmol/L) for up to 4 hours. A regimen of uncooked
cornstarch in 2% cow milk at 4-hour intervals in addition to three meals daily prevented
hypoglycemia, and maintained blood
lactate at nearly normal levels and serum
uric acid and
cholesterol within the normal range;
triglyceride levels were increased only modestly. Overnight
blood glucose levels were comparable to those achieved with continuous intragastric
glucose infusion. With this regimen the five infants have maintained linear growth rates normal for their age and genetic potential; the mean percentage of ideal body weight for length percentile did not change significantly, although two of the five patients were
overweight (123% and 124% of ideal body weight respectively) after 3 years of treatment. We conclude that a trial of uncooked
cornstarch in feedings of milk every 4 hours should be attempted as soon as a more frequent feeding schedule with
dextrose-containing formulas proves ineffective, because the former has the potential to provide the continuous
glucose required by infants with
glycogen storage disease type 1 in a safer and less invasive fashion than continuous intragastric
glucose infusion.