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

이민정; KIM JAE GON; Yang Yeon Mi; Byeong Ju Baik; 송희정

doi:10.5933/JKAPD.2012.39.4.412

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Journal of the Korean academy of pediatric dentistry 2021 KCI Impact Factor : 0.3

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

Journal of the Korean academy of pediatric dentistry
Abbr : J Korean Acad Pediatr Dent
2012, 39(4), pp.412-417
DOI : 10.5933/JKAPD.2012.39.4.412

Publisher : The Korean Academy Of Pediatric Dentistry
Research Area : Dentistry

이민정 ¹, KIM JAE GON ¹, Yang Yeon Mi ¹, Byeong Ju Baik ¹, 송희정 ¹

¹전북대학교

ABSTRACT
열림/닫힘 버튼

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
열림/닫힘 버튼

Hunter syndrome, Mucopolysaccharoidosis (MPS) type II, Dental complications, Dental management

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* 2021년 이후 발행 논문의 참고문헌은 현재 구축 중입니다.

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