A rare case of severe Hunter's Syndrome

Abstract

Hunter's Syndrome or mucopolysaccharidosis Type II is a rare metabolic disorder caused by the deficiency of lysosomal enzyme iduronate-2-sulfatase. It results in progressive accumulation of deramatan and heparan sulfates in tissues leading to a wide clinical spectrum of systemic manifestations. This is a case report of a 6-year-old boy with classical features of Hunter's Syndrome who presented with a chief complaint of multiple decayed teeth. The purpose of this case report is to highlight the role of a pediatric dentist in the management of such special children with advanced dental care.

Keywords

• Dental care
• Hunter's syndrome
• mucopolysaccharidosis
• severe form