1257 OPTIMAL RATE OF NASOGASTRIC (NG) CARBOHYDRATE IN PATIENTS WITH TYPE I GLYCOGEN STORAGE DISEASE (GSD I)

Abstract

Although noctural NG infusion (INF) of carbohydrate (CHO) is standard therapy in GSD I, the optimal rate of CHO has not been established. To determine if a CHO INF of ∼6 mg/kg·min (glucose flux in overnight fasted normal children) suppresses hepatic glucose (GLU) production and optimizes plasma lactate, ketone body (KB) and free fatty acid (FFA) concentrations, 6 GSD I children (4.2 ± 0.8 yrs) received NG INFs containing Vivonex (3 mg of CHO/kg·min) with [²H₂]Glucose plus varying GLU to achieve total INFs of 10.5, 8.6, 5.8 and 3.0 mg of CHO/kg·min. Each INF period was 3 hr with blood sampling during the last 30 min. GLU decreased (p < 0.05) from 106 ± 5 to 90 ± 5, 75 ± 5 and 52 ± 3 mg/dl during the 4 INFs whereas lactate (4.0 ± 0.5, 4.9 ± 0.6, 6.4 ± 0.7 and 8.8 ± 1.2 mM, respectively) and FFA (0.7 ± 0.1, 0.9 ± 0.1, 1.1 ± 0.2 and 1.6 ± 0.1 mM, respectively) increased. Lactate, pyruvate, KB and FFA were increased (p < 0.05) at CHO INFs of ∼6 mg/kg·min compared to the higher INFs. Total GLU flux decreased (p < 0.05) from 10.5 ± 0.3 to 9.1 ± 0.2, 7.2 ± 0.1, and 6.0 ± 0.4 mg/kg·min for the 4 INFs while estimates of endogenous GLU production rose (p < .05) from ∼0 to 0.5 ± 0.2, 1.4 ± 0.1 and 2.9 ± 0.3 mg/kg·min, respectively. In summary, NG INF of CHO at ∼6 mg/kg·min in GSD I fails to suppress endogenous glucose production and optimize organic acidemia. In conclusion, if organic acidemia has an adverse effect on growth in GSD I, NG INFs in excess of ∼ 6 mg/kg·min may be optimal.

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