Effect of Cornstarch Formula in an Infant with Type I Glycogen Storage Disease

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Abstract
In order to cope with profound hypoglycemia in an infant with type I glycogen storage disease receiving nocturnal intragastric infusion, we administered cornstarch formula every four hours at 14 months of age. Following the cornstarch therapy, fasting blood glucose in creased from 39.3±4.0 to 83.5±4.0 mg/dl (p<0.01), σ insulin during oral glucose tolerance test (2.5 g/kg) increased from 76 to 191 µU/ml, 24‐hour urinary C‐peptide excretion increas ed from 31.0±4.7 to 47.4±55.2 µg/g/‐creatinine(p<0.05), plasma somatomedin‐C increased from 0.13 to 0.43 U/ml, and length increased from the 3rd percentile to the range between the 10th and the 25th percentile.The results indicate that cornstarch formula may prevent the precipitous hypoglycemia that occurs during the day in an infant with type I glycogen storage disease on nocturnal in tragastric infusion. Increased insulin and/or somatomedin‐C secretion appears to be responsible for the improvement in linear growth of the infant.