An Unusual Case of Glycogen Storage Disease

  • S. W. Moses
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 41A)


Glycogen Storage Disease Type I (G.S.D.I) is characterised by a block in the final common pathway of glucose liberation from the liver leading to abnormal deposition of liver glycogen. The pertinent metabolic features include fasting hypoglycemia, lactic acidemia and increased free fatty acids. A severe tendency towards acidosis, hyperuricemia, hypertriglyceridemia with an abnormal pre- β band are frequently observed. Glucagon administration does not provoke hyperglycemic response, but causes a further rise in blood lactic acid. No hyperglycemic response can be elicited after galactose, fructose or glycerol administration.


Uric Acid Glycogen Storage Disease Glycogen Storage Disease Type Glucagon Stimulation Test Blood Lactic Acid 
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  1. 1.
    J. Schrub et al: Ped. Research 50; 66, 1973.Google Scholar
  2. 2.
    B. Senior and L. Loridan: Studies of Liver Glycogenosis with particular reference to the metabolism of intravenously administered glucose. New Engl Jnl Med 958; 279, 1968.Google Scholar

Copyright information

© Plenum Press, New York 1974

Authors and Affiliations

  • S. W. Moses
    • 1
  1. 1.Department of Pediatrics B and Pediatric Research LaboratorySoroka Medical Center and the University of the NegevIsrael

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