Glucose production and utilization in children with glycogen storage disease type I

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Abstract
Glucose turnover rates were estimated with [2H2]glucose in 5 children with glycogen storage disease type I (GSD I) during sequential withdrawal of an infusion of glucose to determine whether their hypoglycemia was the result of decreased glucose production or increased rates of glucose utilization. Plasma glucose concentrations were 78 .+-. 5 mg/dl during infusion of glucose at 6 mg/kg per min and decreased to 53 .+-. 3 and 45 .+-. 7 mg/dl during the 3 and 1.5 mg/kg per min glucose infusions, respectively. Total glucose flux during the 6 mg/kg per min glucose infusion was 7.3 .+-. 0.7 mg/kg per min and decreased with reduction in the rate of glucose infused. Endogenous glucose production increased with each decrease in the rate of exogenous glucose infused. Following discontinuation of glucose infusion, endogenous glucose production was 3.9 .+-. 0.3 mg/kg per min a value that was lower (P < 0.05) than that observed in overnight-fasted normal children (6.3 .+-. 0.3 mg/kg per min) but not statistically different from the Ra [glucose appearance] of normal children fasted for 30 h (4.1 .+-. 0.5 mg/kg per min). Children with GSD have glucose production rates that are 40% lower than those of normal children fasted overnight but equal to those of normal children fasted 30 h. Rates of glucose utilization during the 6 mg/kg per min glucose infusion were higher (0.05 < P < 0.1) than those of overnight-fasted normal children, despite lower plasma glucose concentrations when expressed on the basis of estimated brain weight. The hypoglycemia in children with GSD type I is the result of impaired glucose production.