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Von Gierke disease

  • Alternative Names

    Type I glycogen storage disease


    Treatment

    The goal of treatment is to avoid low blood sugar. Eat frequently during the day, especially 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 sugars or uncooked cornstarch. The tube can be put in at bedtime and taken out each morning.

    A medication called allopurinol can lower blood uric acid and decrease the risk for gout. Other medications may include those for kidney disease, high lipids, and to increase the cells that fight infection.

    Persons with von Gierke disease cannot properly break down fruit or milk sugar. It is best to avoid these products.


    Support Groups

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


    Expectations (prognosis)

    Since treatments have been developed, growth, puberty, and quality of life have improved for people 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:

    • Gout
    • Kidney failure
    • Life-threatening low blood sugar
    • Liver tumors

    Complications
    • Frequent infection
    • Gout
    • Kidney failure
    • Liver tumors
    • Osteoporosis
    • Seizures, lethargy, confusion due to low blood sugar
    • Short height
    • Underdeveloped secondary sexual characteristics (breasts, pubic hair)
    • Ulcers of the mouth or bowel

    Calling your health care provider

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