Rare metabolic liver disease Glycogen storage disease due to glucose-6-phosphatase deficiency glycogen storage disease type I ICD10:E74.0 DOID:2749 Hepatorenal glycogenosis Glycogen storage disease due to G6P deficiency Glycogen storage disease type 1 von Gierke disease orphanet glycogenosis type I MESH:D005953 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 (see these terms), and characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver. Glycogen storage disease, type I (disorder) GSD due to G6P deficiency von Gierke's disease GSD type 1 G6P deficiency Glycogen Storage Disease Type I UMLS:C0017920 Glycogenosis type 1 MedDRA:10018464 Von Gierke disease deficiency of glucose-6-phosphatase OMIM:232200 glycogen storage disease I OMIM:232220 OMIM:232240 Disorder of carbohydrate metabolism Rare genetic renal disease