This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Biochemistry
[{'content': 'A child  rapidly develops hypoglycemia after moderate activity. On physical examination, kidney and liver are found to be enlarged. Histopathology of liver shows deposits of glycogen in an excess amount. Blood examination reveals raised ketone bodies, lactic acid and triglycerides. Which enzyme is deficient in this patient? \nSelect and explain. \nA. Glycogen synthase, B. Endoplasmic reticulum glucose-6-phosphate transporter, C. Glucose-6-phosphatase, D. Liver phosphorylase kinase', 'role': 'user'}, {'content': "Answer: C \nExplanation: Type Ia GSD-Von Gierke’s Disease:\n\nMost\xa0common\xa0Glycogen\xa0Storage\xa0Disorder (GSD) in childhood.\nAutosomal recessive.\nGlucose 6-phosphatase is deficient.\n\nThe biochemical hallmarks are:\n\nHypoglycemia\nLactic acidosis\nHyperlipidemia\nHyperuricemia\n\nClinical Presentation:\nMost commonly present at 3–4 months of age with:\n\nDoll like facies with fat cheeks\nRelatively thin extremities\nShort stature, protuberant abdomen\nMassive hepatomegaly\nKidneys are also enlarged\nNo splenomegaly\nPlasma may be milky due to associated hypertriglyceridemia.\n\nKey Concept:\xa0\nGlucose 6-phosphatase\xa0is deficient in Von Gierke's disease. It is the most\xa0common glycogen storage disorder\xa0in childhood.\nReference- Harper’s illustrated biochemistry. 30th edition page no: 179", 'role': 'assistant'}]