Nutrition Therapy for Hepatic Glycogen Storage Diseases

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There are many different types of glycogen storage diseases. The first type of glycogen storage disease occurs when the body is missing the enzyme glucose-6-phosphatase which is the enzyme that converts glucose-6-phosphatase to glucose. People with disease can encounter many different side effects including hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. The main nutritional intervention to treat this disease focuses on controlling hypoglycemia. Increasing blood glucose levels will enable the body to function more efficiently. Infants up to one year of age receive and overnight continuous feeding of a high carbohydrate via a nasogastric tube. During the day they consume formula, instant rice and oatmeal cereals every three hours. At eight months to a year infants receive an uncooked cornstarch therapy. Throughout their life time individuals diagnosed with this disease will continue to receive overnight continuous feedings and are instructed to consume a diet that consists of high complex carbohydrates such as oatmeal, barley, rice, pasta, and legumes; as well as a diet that is low in fat. Nutrition therapy has been proven to help with physical growth, metabolic control, glucose homeostasis, lactic acidosis, cholesterol, and hyperlipidemia. 
KM

 Goldberg, T., & Slonim, A. E. (1993). Nutrition therapy for hepatic glycogen storage diseases. Journal of the American Dietetic Association, 93(12), 1423-1430.

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