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: Pediatrics
Coarse facies, hepatosplenomegaly and tall QRS on ECG are characteristic feature of- 
Select and explain. 
A. Glycogen storage disease type II, B. Hurler's disease, C. Hunters disease, D. Hemochromatosis
B: Hurler's disease
Answer: B: Hurler's disease 
Explanation: Ans. is 'b' i.e., Glycogen storage disease type II o Hepatosplenomegaly, characteristics E.C.G. findings and coarse facies are seen in Glycogen storage Type II or pompe's ds. o Death of infant before 2 yrs of age. o Pompe's disease is caused due to absent or reduced level of acid alpha glucosidase (acid maltase) activity. o Hurler's and Hunter syndrome are mucopolysaccharidosis that presents with hepatomegaly and coarse facies, but characteristic ECG pattern of Pompe's disease clinches the diagnosis in our of Pompe's disease.