disease disrupts disease causes disruption of disease has feature glucose-6-phosphatase activity Hepatomegaly glycogen storage disease glycogen storage disease I DOID:2749 G6P deficiency GSD due to G6P deficiency GSD type 1 GSD type I GSD1 Glycogenosis due to glucose-6-phosphatase (G6P) deficiency or glycogen storage disease, (GSD), type 1, is a group of inherited metabolic diseases, including types a and b, and characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver. ICD10:E74.0 ICD10:E74.01 MESH:D005953 MONDO:0002413 MONDO:0018220 MedDRA:10018464 NCIT:C84733 Orphanet:364 SCTID:7265005 deficiency of glucose-6-phosphatase glycogen storage disease due to G6P deficiency glycogen storage disease due to glucose-6-phosphatase deficiency glycogen storage disease type 1 glycogen storage disease type I glycogen storage disease, type I glycogenosis type 1 glycogenosis type I hepatorenal glycogenosis von Gierke disease von Gierke's disease nephropathy secondary to a storage or other metabolic disease disease of catalytic activity