Journal of pediatric gastroenterology and nutrition
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J. Pediatr. Gastroenterol. Nutr. · Aug 1999
Effect of continuous glucose therapy begun in infancy on the long-term clinical course of patients with type I glycogen storage disease.
To evaluate the effects of continuous glucose therapy on metabolic control, occurrence of severe hypoglycemia, physical growth and development, and complications of glycogen storage disease type I (GSD-I). ⋯ Long-term continuous glucose therapy with cornstarch, begun in infancy, resulted in mean height 0.7 SDS less than target height. Optimal biochemical control of GSD-I requires meticulous adherence to an individualized dietary regimen that is based on the results of periodic metabolic evaluation and home blood glucose monitoring. Renal glomerular dysfunction and formation of hepatic adenomata remain serious long-term complications.