All the following are features of Von Gierke disease except: (A) Hypoglycemia, (B) Lactic acidosis, (C) Hyperlipidemia, (D) Muscle hypotonia
Answer is D. Type name Enzyme Deficiency Clinical Features Ia Von Gierke disease Glucose-6-phosphatase Glycogen accumulation in liver and renal tubule cells Hypoglycemia Lactic acidemia Ketosis Hyperlipidemia Reference: Harper; 30th edition; Table: 18-2; Page no: 179