894 DIFFERING FOOD COMPOSITION FOR NOCTURNAL INTRAGASTRIC THERAPY IN TYPES I AND III GLYCOGEN STORAGE DISEASE (GSD)

Abstract

GSD-I and GSD-III, although clinically similar, are metabolically quite different and so may demand different nutritional therapeutic approaches. To test this, some of the metabolic responses of patients with GSD-I and GSD-III were measured in response to meals of glucose, beef and glucose plus beef. Glucose ingestion in GSD-I led to a fall in plasma glucagon, lactate, alanine and valine, whereas in GSD-III, although glucagon fell, a discordant rise in lactate, alanine and valine took place. Beef ingestion in GSD-I led to a rapid fall in glucose and insulin and a sharp rise in glucagon, alanine and valine, while in GSD-III, there was a considerable rise in glucose, insulin and glucagon and only a modest and transient rise in alanine and valine. These results suggest that enhanced gluconeogenesis is detrimental in GSD-I, whereas in GSD-III, it is beneficial and essential. This motivated us to treat GSD-I with nocturnal intragastric therapy (NIG) of high carbohydrate content and GSD-III with NIG therapy of high protein content. Both types of patient showed marked clinical and metabolic response to their respective therapies.