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OneLiner

Von Gierke disease is the most common form of glycogen storage disease. It is caused by a deficiency of glucose-6-phosphatase, which prevents glycogen breakdown into glucose and also prevents gluconeogenesis, causing hepatomegaly. Infants with Von Gierke disease typically have lab abnormalities that are not shown in this patient, including fasting hypoglycemia, elevated lactate, and elevated uric acid.