The<unk>  management of glycogen storage disease type 1 (GSD type 1) is divided into nutritional therapy and pharmacologic management of systemic complications. The primary concern in infants and young children with GSD type 1 is hypoglycemia. Small frequent feeds high in complex carbohydrates (preferably those high in fiber) are administered at regular intervals throughout 24 hours for the prevention of hypoglycemia. Sucrose (fructose and glucose) and lactose (galactose and glucose) may be limited or avoided. Solid food is introduced at the time of 4 - 6 months. Infant cereals are started followed by vegetables and then by meat.The preferred treatment for young child is cornstarch (CS); which may be used alone or by mixing it with sucrose-free, fructose-free, lactose-free infant formula, sugar-free soy milk, sugar-free drinks, and/or water. Other treatment strategies are directed towards management of hypocitraturia, hypercalcemia, proteinuria, platelet dysfunction, and neutropenia.
Glycogen storage disease type I medical therapy
Glycogen storage disease type II medical therapy
Glycogen storage disease type III medical therapy
Glycogen storage disease type I secondary prevention
Glycogen storage disease type I primary prevention
Glycogen storage disease type II secondary prevention
Glycogen storage disease type III secondary prevention
Glycogen storage disease type II primary prevention