Medical Dictionary

von Gierke disease

noun von Gier·ke disease \ vän-ˈgir-kə- \
variants: or von Gierke's disease play \-kəz-\

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

Biographical Note for von gierke disease

  • Gierke, Edgar Otto Konrad von (1877–1945), German pathologist. Von Gierke's chief academic position was a professorship of bacteriology at Karlsruhe's Technical University. During World War I he served first as a field doctor and then as a military pathologist. In 1911 he published a textbook of pathological anatomy. He described the type of glycogen storage disease identified with his name in 1929. Other areas of his research included thyroid gland structure, bone tumors, and metabolic diseases.

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