Browse > Article
http://dx.doi.org/10.5933/JKAPD.2012.39.4.412

DENTAL MANAGEMENT OF PATIENT WITH HUNTER SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE II) : A CASE REPORT  

Lee, Min-Jeong (Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University)
Kim, Jae-Gon (Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University)
Yang, Yeon-Mi (Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University)
Baik, Byeong-Ju (Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University)
Song, Hee-Jeong (Department of Pediatric Dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University)
Publication Information
Journal of the korean academy of Pediatric Dentistry / v.39, no.4, 2012 , pp. 412-417 More about this Journal
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;
Citations & Related Records
Times Cited By KSCI : 2  (Citation Analysis)
연도 인용수 순위
1 Liu KL : The oral signs of Hurler-Hunter syndrome : report of four cases. ASDC J Dent, 47:122-127, 1980.
2 Wraith JE, Scarpa M, Beck M, et al. : Mucopolysaccharidosis type II (Hunter syndrome) : a clinical review and recommendations for treatment in the era of enzyme replacement therapy. Eur J Pediatr, 167:267-277, 2008.   DOI
3 Ahn SY, Lee Y, Jeon SY, et al. : A Case of Hunter Syndrome. Kor J Dermatol, 46:928-932, 2008.   과학기술학회마을
4 Lin SP, Chang JH, Lee-Chen GJ, et al. : Detection of Hunter syndrome (Mucopolysaccharidosis type II) in Taiwanese: biochemical and linkage studies of the iduronate-2-sulfatase gene defects in MPS II patients and carriers. Clin Chim Acta, 369:29-34, 2006.   DOI
5 Faragher MW, Stark RJ : Hunter syndrome complicated by hydrocephalus and an endogenous anticoagulant. J Clin Neurosci, 4:252-255, 1997.   DOI
6 Downs AT, Crisp T, Ferretti G : Hunter's syndrome and oral manifestations : a review. Pediatr Dent, 17:98-100, 1995.
7 Lee SM, Kim JS, Yang MK, et al. : Clinical Experience of the Anesthetic Management of Mucopolysaccharidosis. Korean J Anesthesiol, 45:672-676, 2003.   과학기술학회마을   DOI
8 Park JS, Kim JC, Han SH : Dental features of a child with hurler syndrome : a case report. J Korean Acad Pediatr Dent, 22:625-630, 1995.
9 Song JH, Jang CH, Kim YJ, et al. : Gingival hyperplasia in a Mucopolysaccharidosis'patient : a case report. J Korean Acad Pediatr Dent, 34:150-155, 2007.