618 EFFECTS OF DIETARY THERAPY ON ETHANOL ELIMINATION IN TYPE I GLYCOGEN STORAGE DISEASE

Type I Glycogen Storage Disease (GSD-I) is associated with a wide variety of metabolic abnormalities including an extremely rapid elimination of ethanol. As dietary therapy aimed at maintaining normoglycemia corrects many of the metabolic abnormalities, the effect of this therapy on ethanol elimination was studied. We measured the half-life (T½min) and clearance (Cl ml/min) of 16ml/m2 of intravenous ethanol in 3 groups of patients with GSD-I. Group I consisted of 3 patients who were untreated; Group II consisted of 3 patients who were treated; Group III consisted of 4 patients who were partially treated and were developing metabolic imbalances. Results: Group I showed T½ 11.79±0.8 and Cl 1059±240; Group II showed T½ 28.3±1.5 and Cl 489±105; Group III showed T½ 20.6±2.3 and Cl 770±170; and the Control showed T½ 28.5±6.4. The T½ and Cl of ethanol was significantly shortened in Group I when compared with Group II patients (p < 0.05). The T½ and Cl in Group III patients was intermediate between group I and Group II patients.