Studies of Glycogen Metabolism in Liver Glycogen Disease (von Gierke's Disease): Six Cases with Similar Metabolic Abnormalities and Responses to Glucagon.


Glycogen disease of the liver (von Gierke's disease) is a rare and incompletely understood disorder of childhood. It is characterized by defective breakdown of liver glycogen to glucose. In most cases, excessive liver glycogen deposition occurs, reaching levels as high as 14 to 17 per cent of the wet liver weight (1, 2). Major clinical manifestations… (More)

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