Error creating thumbnail: This article needs additional citations for verification. Glycogen storage disease type IClassification and external resourcesICD-10 E74.0ICD-9 271.0OMIM 232200DiseasesDB 5284MedlinePlus 000338eMedicine ped/2416 Glycogen storage disease type I (GSD I) or von Gierke's disease, is the most common of the glycogen storage diseases. This genetic disease results from deficiency of the enzyme glucose-6-phosphatase. This deficiency impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis. Since these are the two principal metabolic mechanisms by which the liver supplies glucose to the rest of the body during periods of fasting, it causes severe hypoglycemia...