This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Disorders of Amino acid Metabolism, Subject name: Pediatrics
[{'content': 'A child who had derangement of milestones and was hyperactive later on developed self mutilating behavior and hair pulling behavior. The child died a few years later. Which of the following enzyme is most likely deficient in the child? \nSelect and explain. \nA. Phenylalanine hydroxylase, B. HGP, C. Adenosine deaminase, D. Hexosaminidase A', 'role': 'user'}, {'content': 'Answer: B \nExplanation: Delayed development, hyperactivity and self-mutilating behavior in a child who died later is suggestive of Lesch- Nyhan disease due to HGP deficiency. Phenylalanine hydroxylase deficiency- intellectual disability, hypopigmentation, musty body odour Adenosine deaminase deficiency- early vasculopathy, stroke, immunodeficiency Hexosaminidase A deficiency- Tay sachs disease: macular cherry red spots', 'role': 'assistant'}]