Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Comparison of Long-term Prognosis in Siblings with Dentinogenesis Imperfecta depending on the Timing of the Treatment Intervention : Case Reports

상아질형성부전증 남매의 치료개입 시기에 따른 상이한 장기 예후 : 증례 보고

  • Kim, Gimin (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Lee, Jaesik (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University)
  • 김기민 (경북대학교 치의학대학원 소아치과학교실) ;
  • 이제식 (경북대학교 치의학대학원 소아치과학교실)
  • Received : 2020.10.19
  • Accepted : 2020.11.24
  • Published : 2021.05.31
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Abstract

Dentinogenesis imperfecta (DI) is a hereditary disorder of dentinal defect. It is generally inherited as a single autosomal dominant trait. DI usually affects both the primary and permanent dentition. Affected teeth have various types of discolorations, rapid destruction of the dentin, and severe attrition. In radiologic view, the affected teeth have bulbous crowns, short roots and narrow or closed pulp chambers. The treatment objective is to prevent additional attrition and recover the vertical dimension of occlusion. The aim of this report was to present the long-term prognosis in 15 years in a pair of siblings. Both the patients had DI with tooth attrition and discoloration. Different treatment procedures were used, depending on the difference in the timing of intervention. The first patient saved most of his teeth. The second patient had all of her teeth extracted. This report could be helpful for early diagnosis and overall treatment of DI.

상아질형성부전증은 상아질 형성 이상이 초래되는 유전성 질환으로 상염색체 우성 형질을 따른다. 상아질형성부전증은 유치와 영구치 모두 이환되며, 다양한 치아 변색, 상아질 및 법랑질 파괴, 심한 치아마모 등의 임상소견을 보인다. 방사선학적으로 치아는 가느다란 치근과 둥그런 치과의 형태를 보이고 치수강은 적거나 폐쇄되었다. 상아질형성부전증의 치료는 추가적인 마모를 방지하고 수직고경 회복을 목표로 한다. 본 증례는 치아의 마모와 변색을 주소로 내원한 상아질형성부전증을 지닌 남매의 상이한 15년간의 장기 예후를 보고하고자 한다. 두 환자에서 치료개입 시기의 차이에 따라 상이한 치료과정을 볼 수 있다. 첫 번째 환아는 대부분의 본인 치아를 살렸으나, 두 번째 환아는 모든 치아를 발치하였다. 이는 상아질형성부전증의 조기 진단 및 치료방법 결정에 도움이 될 수 있다.

Keywords

References

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