A female infant appeared normal at bih but developed signs of liver disease and muscular weakness at 3 months. She had periods of hypoglycemia, paicularly on awakening. Examination revealed hepatomegaly. Laboratory analyses following fasting revealed ketoacidosis, blood pH 7.25, and elevations in both alanine transaminase (ALT) and aspaate transaminase (AST). Administration of glucagon following a carbohydrate meal elicited a normal rise in blood glucose, but glucose levels did not rise when glucagon was administered following an overnight fast. Liver biopsy revealed an increase in the glycogen content. To prevent the frequent episodes of hypoglycemia, which of the following dietary supplements would be most appropriate for this patient?
Because fasting hypoglycemia results from an inability to break down glycogen past the limit dextrin of phosphorylase, a patient with type III glycogen storage disease should be given frequent meals high in carbohydrates. Uncooked cornstarch is an effective supplement because it is slowly digested, and therefore the glucose is released slowly into the bloodstream, helping to maintain blood glucose concentrations. Ref: Powers A.C., D'Alessio D. (2011). Chapter 43. Endocrine Pancreas and Pharmacotherapy of Diabetes Mellitus and Hypoglycemia. In L.L. Brunton, B.A. Chabner, B.C. Knollmann (Eds), Goodman & Gilman's The Pharmacological Basis of Therapeutics, 12e.
Casein (milk protein)
Uncooked cornstarch
Fructose
Lactose