대한소아치과학회지 (Journal of the korean academy of Pediatric Dentistry)

  • 제35권3호
  • /
  • Pages.562-570
  • /
  • 2008
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  • 1226-8496(pISSN)
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  • 2288-3819(eISSN)
대한소아치과학회 (Korean Academy of Pediatric Dentistry)
권호 표지

법랑질 형성부전증 환아의 치험례

AMELOGENESIS IMPERFECTA: A CASE REPORT

  • 박희숙 (경북대학교 치과대학 소아치과학교실) ;
  • 김태완 (경북대학교 치과대학 소아치과학교실) ;
  • 김영진 (경북대학교 치과대학 소아치과학교실) ;
  • 김현정 (경북대학교 치과대학 소아치과학교실) ;
  • 남순현 (경북대학교 치과대학 소아치과학교실)
  • Park, Hee-Suk (Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University) ;
  • Kim, Tae-Wan (Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University) ;
  • Kim, Young-Jin (Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University) ;
  • Kim, Hyun-Jung (Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University) ;
  • Nam, Soon-Hyeun (Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University)
  • 발행 : 2008.08.29
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초록

법랑질 형성부전증(amelogenesis imperfecta)은 전신질환과 관련없이 법랑질에 결함을 나타내는 유전질환이다. 법랑질 형성부전증은 발현되는 증상과 유전 양상에 따라 14가지의 아류형으로 분류된다(Witkop, 1989). 임상적으로는 크게 형성부 전형(hypoplastic type), 저석회화형(hypocalcified type), 성숙부전형(hypomaturation type)으로 나누어진다. 하지만 이들은 때로 혼재된 양상으로 나타난다. 법랑질 형성부전증은 법랑질이 질적으로나 양적으로 부족하며 치수 석회화, 치근 형태 이상, 맹출 장애와 영구치의 매복, 점진적인 치근의 흡수, 선천적 치아 결손, 그리고 전치부나 구치부의 개교합 등의 문제점을 나타낸다. 첫 번째 증례는 만 1세 4개월때 유전치부의 치아색 이상을 주소로 내원한 환자로 그 후 모든 유치 및 영구치에서 법랑질 형성부전증이 관찰되어 수복 술식, 교정적 치료 및 최근 보철 치료까지 완료하였다. 두 번째 증례는 만 9세 3개월된 환아로 유치 및 영구치에서 법랑질 형성부전증이 관찰되었다. 수복 치료가 시행되었고 보철 치료는 성장 완료 후 시행하기로 계획하였다. 본 증례들을 통하여 법랑질 형성부전증은 유치 및 영구치 모두에 발생할 수 있으며 장기간의 치료 및 관리가 필요하다는 것을 알 수 있었다.

Amelogenesis imperfecta is a group of hereditary defects of enamel, unassociated with any other generalized defects. It is classified into 14 subtypes according to different clinical and genetic features. According to its clinical features, it is classified into hypoplastic type, hypocalcified type and hypomaturation type. However, these features tend to co-exist often. Dental features associated with amelogenesis imperfecta include quantitative and qualitative enamel deficiencies, pulpal calcification, root malformations, abnormal eruption, impaction of permanent teeth, progressive resorption of root and crown, congenital missing teeth and anterior and posterior open bite occlusions. The first case patient is a 16 month-old child with discoloration of deciduous teeth. All of her deciduous and permanent dentition has shown amelogenesis imperfecta. The restorational, orthodontic and recent prosthodontic treatments have been completed. Another patient is a 9 year and 3 month-old child with amelogenesis imperfecta in both deciduous and permanent dentition. The restoration has been done and the prosthodontic treatment is planned after the completion of growth. Above cases indicate that amelogenesis imperfecta occurs both in deciduous and permanent dentition, and it requires the long term treatment and care.

키워드

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