Glycogen storage disease type I secondary prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Secondary Prevention

Effective measures for secondary prevention of GSD type 1 include:

Gastrointestinal /Nutritional recommendations
A metabolic dietician is an important member of the team. If not available, one should be consulted. Maintaining blood glucose levels ≥70 mg/dl is important to achieve good metabolic control. Levels should e kept consistent to avoid hypoglycemia and fluctuations blood glucose levels. In infants and children: Avoid fasting for more than 3-4 hours. Offer small, frequent feedings; avoid or limit sucrose, fructose, and galactose (a soy formula such as Prosobee may be used overnight). Access via NG or G-tube placement is recommended for emergencies and/or for OGFs; caution with surgical G-tube placement should be taken in GSD 1b. Monitor blood glucose before feeds. Raw, uncooked cornstarch may be introduced between 6 and 12 months of age/ Continuous gastric feedings may be used overnight. In adolescent and adults: Avoid fasting for more than 5-6 hours with the use of raw, uncooked cornstarch and/or OGFs; it is important to not change the brand of cornstarch. If changed, then monitoring of BG levels after the change is necessary. Plan for small, frequent meals (nutrient distribution:60-70% carbohydrates, 10-15% protein, <30% fat); avoid or limit sucrose, fructose, and galactose. Regular blood glucose monitoring is needed, especially during periods of growth. Multivitamins, calcium, and vitamin D are necessary because of the restricted nature of the diet. Both overtreatment and undertreatment are harmful. Overtreatment can result in insulin resistance. Good glucose control improves several of the metabolic sequelae of GSD 1.
Adopted from Genetics in medicine