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What is A10-year-old boy rapidly develops hypoglycemia after moderate activity. Blood examination reveals raised ketone bodies, lactic acid, and triglycerides. On examination, the liver & kidneys were enlarged. Histopathology of the liver shows deposits of glycogen in excess amount. What is the diagnosis? and explain in detail?
Most common glycogen storage disease presenting with hypoglycemia and normal glycogen structure is Von Gierke disease, Von Gierke disease is due to deficiency of glucose-6-phosphatase. affects only liver Clinical features: severe hypoglycemia ketosis massive hepatomegaly enlarged kidneys hyperiglyceridemia lactic acidosis hyperuricemia Spleen not enlarged