HYPERLIPIDEMIA IN TYPE III GLYCOGENOSIS

SUMMARY During 4 years of observation, a patient suffering from type III glycogenosis developed considerable hyperlipidemia with hyperlipoproteinemia and hypercholesterolemia. The boy repeatedly suffered from hypoglycaemia especially during infections. Our diagnosis was made on the basis of markedly increased quantities of glycogen in the liver, skeletal muscle and the erythrocytes. After a 12-hour fast, glucagon admininstration resulted in an elevation of free fatty acids and pyruvate, no change in lactate, and no increase in glucose as measured in both the hepatic and peripheral veins. These observations can be explained by the influence of glucagon on lipolysis.