Nelson Textbook of Pediatrics 22nd Ed β Section 107.1. AGL gene mutations β deficiency of glycogen debranching enzyme (amylo-1,6-glucosidase). Hepatomegaly, fasting hypoglycemia, muscle weakness, cardiomyopathy. Limit dextrin accumulation. Types IIIa (muscle + liver) and IIIb (liver only). Treatment: high-protein diet, cornstarch.
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