medical Definition of von Gierke disease
: a glycogen storage disease that is caused by a deficiency of glucose-6-phosphate and has a clinical onset at birth or during infancy, that is characterized especially by enlargement of the liver and kidney, hypoglycemia, hyperlipidemia, hyperuricemia, acidosis, adiposity, xanthomas, and nosebleeds, and is inherited as an autosomal recessive trait
