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Von Gierke disease

• Overview
• Symptoms
• Treatment
• Prevention

Alternative Names:

Type I glycogen storage disease

Treatment:

The goal of treatment is to avoid low blood sugar. Frequent feedings are needed during the day, especially with foods that contain carbohydrates (starches). Older children and adults may take cornstarch by mouth.

Overnight, a feeding tube is placed through the nose into the stomach to provide a supply of sugars or uncooked cornstarch. The tube can be put in at bedtime and taken out each morning.

A medication called allopurinol, which can lower blood uric acid and decrease the risk for gout, may also be prescribed.

Persons with von Gierke disease cannot properly break down fruit or milk sugar, so such products are best avoided.

Support Groups:

Association for Glycogen Storage Disease -- www.agsdus.org

Expectations (prognosis):

Since development of the treatments listed above, growth, puberty, and quality of life have markedly improved for those with von Gierke disease. Those who are identified and carefully treated at a young age can live into adulthood.

Early treatment also decreases the rate of severe problems such as liver tumors, kidney failure, gout and life-threatening low blood sugar.

Complications:

• Seizures, lethargy, confusion due to low blood sugar
• Gout
• Kidney failure
• Liver tumors
• Short adult stature
• Underdeveloped secondary sexual characteristics
• Osteoporosis
• Ulcers of the mouth or bowel
• Frequent infection

Calling your health care provider:

Call your health care provider if there is a family history of glycogen storage disease or early infant death due to low blood sugar.

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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