This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Genetic and genetic disorders, Subject name: Pediatrics
[{'content': "Enzyme replacement therapy is most commonly used for \nSelect and explain. \nA. Gaucher's disease, B. Fabry's disease, C. Niemenn pick's disease, D. Pompe disease", 'role': 'user'}, {'content': 'Answer: A \nExplanation: Gaucher disease is a multisystemuc lipidosis characterized by hematological abnormalities,organomegaly and,skeletal involvement.It has 3 clinical subtypes . It occurs due to the deficient activity of acid beta glucosidase . Thus glucosylceramide accumulate in reticule end I the loam cells. Treatment is mainly enzyme replacement therapy with recombinant acid beta glucosidase.Most extra skeletal symptoms are reversed by 60 IU/kg enzyme iv infusion every other week. Monthly maintenance improves skeletal manifestations. Reference: Nelsons TB of pediatrics 19th edit,pg 487', 'role': 'assistant'}]