obsolete_glycogen storage disease I true Glycogen Storage Disease Type I http://www.orpha.net/ORDO/Orphanet_364 glycogen storage disease type I von Gierke's disease glycogenosis type I Glycogen storage disease, type I (disorder) true deficiency of glucose-6-phosphatase A glycogen storage disease that has_material_basis_in deficiency of the glucose-6-phosphatase enzyme which impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis and is characterized by accumulation of glycogen and fat in the liver and kidneys, resulting in hepatomegaly and renomegaly. von Gierke disease DOID:2749 duplicate. Use Orphanet_364 Glycogen storage disease due to glucose-6-phosphatase deficiency. 2.70