Glucose-6-phosphatase hepatorenal glycogenosis

Glucose-6-phosphatase hepatorenal glycogenosis --> type 1 glycogenosis

glycogenosis due to glucose-6-phosphatase deficiency, resulting in accumulation of excessive amounts of glycogen of normal chemical structure, particularly in liver and kidney.

Synonym: gierkes disease, glucose-6-phosphatase hepatorenal glycogenosis, von gierkes disease.

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