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Glycogen Storage Disease, Type 1

Glycogen storage disease (GSD) refers to an inherited group of glycogen metabolism disorders marked by a deficiency in one of the enzymes responsible for glycogen metabolism As a result, abnormal levels of glycogen buildup in the body. GSD type one, also known as von Gierke's disease, is the most common of these disorders and is caused by a deficiency of the enzyme 6-phosphate dehydrogenase, which helps to metabolize glycogen.

Excessive amounts of glycogen in the liver and kidney lead to an enlarged liver, retarded growth, low blood sugar (hypoglycemia) that occurs within four to six hours after eating, lactic acidosis and hyperlipidemia. Treatment includes preventing hypoglycemia with frequent meals and glucose monitoring.