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HYPERLIPIDEMIA IN TYPE III GLYCOGENOSIS
Author(s) -
VÍTEK BOHUMIL,
ŠRÁČKOVÁ DANUŠE,
TOMAN MIROSLAV,
KRÁTKÝ JAROSLAV,
VOGNÁREK JIŘÍ
Publication year - 1970
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1970.tb17709.x
Subject(s) - medicine , lipolysis , hyperlipidemia , glucagon , endocrinology , glycogen , glycogen storage disease , clofibrate , diabetes mellitus , insulin , adipose tissue
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.