Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Familial Occurrence of Dentin Dysplasia Type I: Case Report

가족 내에 발생한 제 1형 상아질 이형성증: 증례보고

  • Kim, Sohyun (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Kim, Youngjin (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Kim, Hyunjung (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Nam, Soonhyeun (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University)
  • 김소현 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김영진 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김현정 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 남순현 (경북대학교 치의학전문대학원 소아치과학교실)
  • Received : 2013.10.07
  • Accepted : 2013.10.22
  • Published : 2014.02.28
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Abstract

Dentin dysplasia is a rare hereditary disturbance characterized by a dental anomaly of the dentin layer. The etiology is unclear, and this rare hereditary disturbance affects approximately one person in every 100,000. Dentin dysplasia is classified into two types, radicular dentin dysplasia as type I and coronal dentin dysplasia as type II. The characteristic clinical findings of dentin dysplasia type I are normal appearance of the crown and hypermobility of teeth. The radiographic findings are obliteration of all pulp canals, short, blunted and malformed or absent roots. Dentin dysplasia type II as coronal dentin dysplasia shows similar clinical features with dentinogensis imperfecta. This report shows a case of dentin dysplasia type I affecting one family except the father. The clinical, radiographic and histopathologic findings of this family are presented. Dentin dysplasia type I is difficult to diagnose unless dentist performs radiographic examination. If the affecting patient does not get regular dental care, dental abscesses or cysts may form spontaneously without caries. In this regard, early diagnosis is important to prevent premature loss of dentition.

상아질 이형성증은 매우 드물게 발생하는 상염색체 우성 유전 질환이다. 발생학적 원인에 대해서는 아직 명확하게 알려지지 않았으며 인구 100,000당 1명의 발병률을 나타낸다. 상아질 이형성증은 두 가지 형으로 분류가 되며, 치근부위 상아질 형성이상을 보이는 치근형의 제 1형과 치관부위 상아질 형성이상을 보이는 치관형인 제 2형으로 나눠진다. 제 1형 상아질 이형성증은 임상적으로 정상적인 치관형태을 보이고 있으나 동요도를 가지며 방사선학적으로 치수강과 치근관의 폐쇄, 짧은 원추형의 치근형태 및 자발적으로 형성된 치근단 병소가 특징적으로 관찰된다. 본 논문은 한 가족 내에서 아버지를 제외한 4명의 가족 구성원들에게 발생한 제 1형 상아질 이형성증을 관찰한 바, 이에 대해 이환된 가족 구성원들의 임상, 방사선학 및 조직병리학적 소견을 보고하고자 한다. 제 1형 상아질 이형성증은 방사선학적 검사를 시행하지 않고서는 임상적으로 인지하기 어려우며 조기 진단을 통해 정기적인 구강 위생 관리 및 예방치료를 시행할 경우 이환된 치아의 조기 탈락을 예방하거나 지연시킬 수 있을 것이다.

Keywords

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