Glycogen storage disease: a basic understanding and guide to nursing care.

Glycogen storage disease (GSD) is a group of genetic metabolic disorders resulting from a defect in the synthesis or degradation of glycogen. GSDs are classified according to the type of enzymatic defect and primary organs involved and are usually diagnosed in infancy or early childhood. Little is generally known about GSD and nurses may be unfamiliar with care requirements of the GSD patient. Nine distinct types of GSD are presented with the primary focus on type 1, the most common. Current treatment methods, roles of the nurse, and a sample nursing care plan are included.