| DENTAL MANAGEMENT OF PATIENT WITH HUNTER SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE II) : A CASE REPORT |
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Min-Jeong Lee, Jae-Gon Kim, Yeon-Mi Yang, Byeong-Ju Baik, Hee-Jeong Song |
| Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University |
| Hunter 증후군 환아의 치과적 관리: 증례보고 |
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이민정, 김재곤, 양연미, 백병주, 송희정 |
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전북대학교 치의학전문대학원 소아치과학교실 및 구강생체과학연구소 |
Correspondence:
Jae-Gon Kim, Tel: 063-250-2128, Email: pedodent@chonbuk.ac.kr
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Received: 28 August 2012 • Accepted: 31 October 2012 |
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| Abstract |
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Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications. |
| Key Words:
Hunter syndrome, Mucopolysaccharoidosis (MPS) type II, Dental complications, Dental management |
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