<prompt> Coarse facies, hepatosplenomegaly and tall QRS on ECG are characteristic feature of-Glycogen storage disease type IIHurlers diseaseHunters diseaseHemochromatosis  <response>: 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 pompes ds. o Death of infant before 2 yrs of age. o Pompes disease is caused due to absent or reduced level of acid alpha glucosidase acid maltase activity. o Hurlers and Hunter syndrome are mucopolysaccharidosis that presents with hepatomegaly and coarse facies, but characteristic ECG pattern of Pompes disease clinches the diagnosis in our of Pompes disease. 