대한소아치과학회지 (Journal of the korean academy of Pediatric Dentistry)

  • 제42권2호
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  • Pages.180-187
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  • 2015
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  • 1226-8496(pISSN)
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  • 2288-3819(eISSN)
대한소아치과학회 (Korean Academy of Pediatric Dentistry)
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다수의 치아결손을 동반한 색소실조증 환아의 치험례

Incontinentia Pigmenti with Multiple Missing Teeth : Case Reports

  • 최시내 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김영진 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 남순현 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김현정 (경북대학교 치의학전문대학원 소아치과학교실)
  • Choi, Shinae (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University) ;
  • Kim, Youngjin (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University) ;
  • Nam, Soonhyeun (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University) ;
  • Kim, Hyunjung (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University)
  • 투고 : 2014.10.13
  • 심사 : 2014.12.02
  • 발행 : 2015.05.31
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초록

색소실조증은 외배엽 이형성증과 같이 다수의 치아결손을 동반하는 선천성 질환으로 Bloch-Sulzberger syndrome이라고 불리며 X 연관 우성 유전질환이므로 주로 여아에서 많이 발병한다. 외배엽 및 중배엽 기원 조직인 피부, 눈, 머리카락, 치아 및 중추신경계에 증상이 나타나며 Xq28 염색체의 NEMO 유전자의 돌연변이에 의해 발병된다. 본 증례는 유치의 선천적 결손을 주소로 내원한 3명의 환아의 증례를 다루고 있으며 이들은 다수의 유치와 영구치의 선천적 결손, 전치부 원뿔형 치관, 구치부 과잉 교두 등을 보이고 있었다. 치아결손부에 공간 유지 장치를 장착하여 저작기능을 회복해 주고 심미적인 문제를 개선해주었으며 계속적인 관찰이 필요하다.

Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

키워드

참고문헌

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