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Von Gierke disease

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Definition:

Von Gierke disease is a disorder of metabolism in which the body cannot break down glycogen for energy. Gycogen is stored in the liver and muscles and is normally broken down into glucose when you do not eat.

Von Gierke disease is also called Type I glycogen storage disease (GSD I).

Alternative Names:

Type I glycogen storage disease

Causes, incidence, and risk factors:

Von Gierke disease occurs when the body lacks the specific protein (enzyme) that releases glucose from glycogen. This causes abnormal amounts of glycogen to build up in various tissues, leading to low blood sugar.

Von Gierke disease is inherited, which means it is passed down through families. If both parents carry the defective gene related to this condition, each of their children has a 25% chance of developing the disease.

References:

Behrman RE. Nelson Textbook of Pediatrics . 17th ed. Philadelphia, Pa: WB Saunders; 2004.

• Review Date: 5/2/2007
• Reviewed By: Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.

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