| ENCYCLOPEDIA INDEX |
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Injury Disease Nutrition Poison Symptoms Surgery Test |
| A B C D E F G H I J K L M N O P Q R S T U V W X Y Z |
Von Gierke disease |
| Overview Symptoms Treatment Prevention |
| Alternative Names: |
| Type I glycogen storage disease |
| Treatment: |
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Treatment for this disease concentrates on avoiding low blood sugar. Frequent feedings are needed during the day, especially foods that contain carbohydrates (starches). Neither fruit nor milk sugar can be adequately broken down in this disease, and are best avoided. Overnight fasting is avoided by continuous feedings (through a tube that goes through the nose into the stomach) 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, is often used. |
| Support Groups: |
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Association for Glycogen Storage Disease |
| Expectations (prognosis): |
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Since development of the treatments listed above, patients have improved growth, development and health. 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 that can occur without treatment. |
| Complications: |
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| 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. Genetic counseling is recommended if there is a family history of glycogen storage disease. |
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