Type I glycogen storage disease

Parents/patients should be educated regarding preparation of cornstarch and dietary restrictions. Uncooked cornstarch is given as a slurry using water or artificially sweetened fluid, or in formula for infants, at 3- to 5-hour intervals during the day and 4- to 6-hour intervals overnight. Patients should be advised to consume an adequate quantity of proteins and fats. Dietary cholesterol should be restricted to <300 mg/day. Milk products and fruit may be used sparingly. An emergency letter can be created for patients with glycogen storage diseases to enhance interconnectivity and help prevent metabolic emergencies. Metabolic Emergency Protocol: ​emergency letter generator Opens in new window​