Pediatric glycogen storage disease type Ⅰ

Glycogen storage disease type Ⅰ is also called VonGeirk disease, glucose-6-phosphatase deficiency, mucopolysaccharidosis type Ⅰ, and lipomucopolysaccharidosis type Ⅰ. Mucopolysaccharidosis patients have excessive mucopolysaccharides stored in bones, cartilage and other tissues or organs, which affects the normal development of these tissues or organs, and excess mucopolysaccharides are excreted from the urine. The disease was first described by Spmnger in 1968. It is more common in infants and young children, and is also seen in older children.