The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL MANAGEMENT ASSOCIATED WITH ERUPTION DISORDERS IN A PATIENT WITH EHLERS-DANLOS SYNDROME : A CASE REPORT

Ehlers-Danlos syndrome 환아의 맹출장애 : 증례보고

  • Jin, Dallae (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Chong-Chul (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Lee, Sang-Hoon (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Jung-Wook (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Young-Jae (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Hyun, Hong-Keun (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo-Jeon (Department of Pediatric dentistry, School of Dentistry, Seoul National University) ;
  • Jang, Ki-Taeg (Department of Pediatric dentistry, School of Dentistry, Seoul National University)
  • 진달래 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김종철 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2012.11.14
  • Accepted : 2012.12.03
  • Published : 2012.12.31
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Abstract

Ehlers-Danlos syndrome (EDS), an inherited connective tissue disorder, is caused by mutations in genes encoding different types of collagen or collagen-processing enzymes. EDS most typically affects the joints, ligaments, skin, and blood vessels. Oral health may be severely compromised in EDS as a result of specific alterations of collagen in orofacial structures. Dental hard tissue defects, root dilaceration, pulp stones, ectopic or delayed eruption, impaction, and periodontal disease could be observed. Therefore, a number of tissue responses related to collagen and precautions should be anticipated when considering dental treatment in EDS. Long-term and comprehensive dental management is required. The purpose of this report is to describe a clinical case of eruption disorders in a patient with EDS.

EDS는 관절의 과운동성, 피부의 과신장, 조직 약화 등을 임상적 특징으로 하는 유전 질환이다. 피부, 골격, 근육, 인대, 점막, 혈관 등을 구성하는 교원질의 합성 과정과 관련된 유전자의 결함으로 결합조직이 비정상적으로 변하므로 신체 전반에 걸쳐 그 증상이 나타난다. 구강 영역에서도 교원 질과 관련된 조직에 영향을 받게 된다. 구강 점막, 치주 조직, 측두하악관절 및 치아 경조직에 문제가 발생할 수 있으므로, 총체적이고 정기적인 구강관리가 이루어져야 한다. 이번 증례와 같이 EDS에 의해 치아의 맹출 장애가 발생하고, 치은비대, 유착, 매복, 선천적 결손치, 부정교합 등이 관찰되는 경우에도 구강 위생 관리와 함께 장기적인 계획 하에 맹출 관리 및 치료가 요구된다. EDS 환아의 치과 치료시에는 질환의 원인과 전신적 특징에 대한 이해를 바탕으로, 비정상적 교원질 대사에 의해 약화된 점막과 치주조직, 지혈 관리, 반흔 형성 및 측두하악관절에 대한 종합적인 고려와 지속적인 관리가 필요하다.

Keywords

References

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