THE EFFECTS OF GLUCAGON IN THREE FORMS OF GLYCOGEN STORAGE DISEASE*

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Abstract
The response of glucagon of patients with different forms of glycogen storage disease (glucose-6-phosphatase deficiency, limit dextrinosis, and combined limit dextrinosis and glucose-6-phosphatase deficiency) was related in each instance to the nature of the underlying enzyme anomaly. In the patients with limit dextrinosis fasting blood lactic acid levels were normal and glucagon induced neither hyperglycemia nor increased blood lactic acid levels. In the patients with glucose-6-phosphatase deficiency increased blood lactic acid levels were observed and a modest increase in blood glucose and a more marked increase in blood lactic acid occurred in response to glucagon. Treatment of 2 patients with proven or presumed glucose-6-phosphatase deficiency, 1 patient with limit dextrinosis and 1 patient with the combined defect of limit dextrinosis and glucose-6-phosphatase deficiency failed to give lasting clinical benefit. This failure may in part be the result of the development of some form of tolerance to glucagon.