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Treatment of Glycogen storage disease type I
The main goal of treatment is to correct hypoglycemia and maintain a healthy blood glucose level. We have three management lines as follows:
Diet therapy
Your child’s doctor asks you to feed your young infant through a nasogastric tube. Old infant or child eats cornstarch in his diet and continues to use nasogastric during sleep. Also, the diet should include restrictions of:
- Lipid restriction
- Galactose and fructose that increase lactic acid
Medical therapy
- Allopurinol to decrease the uric acid level
- Oral iron for iron deficiency anemia
Surgery:
Surgery aims to remove liver adenomas before they become malignant. The patient may need liver transplantation.
Prognosis of Glycogen storage disease type I
The patient lives a healthy life with adequate treatment. Without proper treatment, the patient will die or survive with poor health.
