J Korean Acad Pediatr Dent. 2012;39(4): 412-417.
DENTAL MANAGEMENT OF PATIENT WITH HUNTER SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE II) : A CASE REPORT
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
Corresponding Author: Jae-Gon Kim ,Tel: 063-250-2128, Email: pedodent@chonbuk.ac.kr
Received: August 28, 2012;  Accepted: October 31, 2012.
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ABSTRACT
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.
Keywords: Hunter syndrome | Mucopolysaccharoidosis (MPS) type II | Dental complications | Dental management
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