von Gierke’s disease /fôn gir″kəz/ [Edgar von Gierke, German pathologist, 1877–1945] , a form of glycogen storage disease in which abnormally large amounts of glycogen are deposited in the liver and kidneys. The disorder is characterized by hypoglycemia, metabolic acidosis, dyslipidemia, and hepatomegaly. Biopsy of the affected organs reveals the absence of glucose-6-phosphatase, an enzyme necessary for glycogen metabolism. There is no effective treatment for the disorder. Medical efforts are directed at preventing hypoglycemia and acidosis. Also called glycogen storage disease, type Ia. See also glycogen storage disease.
