Abstract Abstract 192

Uncooked cornstarch is widely used for the treatment of patients with GSD-Ia and may maintain normoglycaemia for up to 6 hours. The aim of this study was to see if the duration of normoglycaemia could be extended using a single dose of pancreatin combined with the cornstarch. We studied the metabolic response and the glucose oxidation rate (using the natural enrichment of 13C in cornstarch) with and without pancreatin in 5 controls and 5 patients with GSD Ia. Methods: Before and following a cornstarch load (1g/kg) blood samples were taken to measure blood glucose, lactate, insulin and free fatty acids at hourly intervals up to 8 hours or until the blood glucose concentration fell below 2.5 mmol/l. Breath samples were collected at 30 min intervals throughout. Results: In neither the controls nor the patients was there any statistically significant difference in the blood glucose concentrations or the glucose oxidation rates with and without pancreatin although glucose oxidation was significantly higher in the patients compared to the controls. However there was wide individual variation in the responses, and in the two patients who had the lowest glucose oxidation rate pancreatin increased the duration of normoglycaemia by 23 and 29%. Conclusions: Pancreatin may prolong the duration of normoglycaemia in some patients with GSD1a but those patients who would benefit and the most effective dose needs to be determined. The financial support of the AGSD (UK) is gratefully acknowledged