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: Unknown
[{'content': 'Acid maltase deficiencyoccurs in : \nSelect and explain. \nA. Pompe’s disease, B. Von-gerke’s disease, C. Mc-ardle’s disease, D. Neimen pick disease', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Pompe Disease\nAcid maltase (acid α-glucosidase) deficiency, also called Pompedisease, is a glycogen and lysosomal storage disease with autosomal recessive inheritance.\nThe classic severe infantile form presents with hypotonia, myocardiopathy, and hepatosplenomegaly.\nThis variant is rapidly progressive and generally results in death in the first year of life. However, as with other lysosomal diseases, there are early- and late-onset forms of this disorder.\nThe late-onset patients typically present with a slowly progressive myopathy that may resemble a limb-girdle muscular dystrophy. Respiratory insufficiency may develop with advancing disease.\nIn late stages of the disease, patients may require mechanical ventilation, report swallowing difficulties, and experience loss of bowel and bladder control.\nMyocardiopathy is not usually seen in late-onset variants of Pompe disease.', 'role': 'assistant'}]