<prompt> A male child with coarse facies, macroglossia, thick lips with hepatosplenomegaly presents with copious mucus discharge from nose. Probable underlying diagnosis is:Hurler diseaseBeckwith-Wiedemann syndromeProteus syndromeHypothyroidism  <response>: a. Hurler diseaseRef: Nelsons text book of Pediatrics, 20th edp 747-743, Ghai 8e p 659-661Hurler disease deficiency of L-iduronidaseDiagnosis is usually made between 6-24 months of age with hepatosplenomegaly, coarse facial features, corneal clouding, large tongue, prominent forehead, joint stiffness, short stature and skeletal dysplasiaMost patients have recurrent upper respiratory tract infections, noisy breathing and copious nasal dischargeValvular heart disease, obstructive airway disease, respiratory infection, and cardiac complications are common. 