대한소아치과학회지 (Journal of the korean academy of Pediatric Dentistry)

  • 제35권4호
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  • Pages.713-717
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  • 2008
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  • 1226-8496(pISSN)
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  • 2288-3819(eISSN)
대한소아치과학회 (Korean Academy of Pediatric Dentistry)
권호 표지

전신성 백색증(Oculocutaneous albinism) 환아의 증례 보고

OCULOCUTANEOUS ALBINISM : A CASE REPORT

  • 최지은 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 최남기 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 김선미 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 양규호 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21)
  • Choi, Ji-Eun (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 21) ;
  • Choi, Nam-Ki (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 21) ;
  • Kim, Seon-Mi (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 21) ;
  • Yang, Kyu-Ho (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 21)
  • 발행 : 2008.11.30
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초록

백색증(albinism)이란 신체 내 melanin 합성의 부분적, 혹은 완전한 결핍에 의해 일어나는 질환이다. Melanin은 신체 내에서 머리카락, 눈, 피부 등에 분포하기 때문에 백색증 환자는 밝은 금발이나 갈색의 머리카락과 하얀 피부, 붉거나 갈색의 홍채를 갖는 특징적인 외모를 지닌다. 백색증 환자는 변이된 유전자 및 임상적 특징에 따라 크게 전신성 백색증, 안성 백색증, 부분적 백색증으로 나뉘며 그 밖에 많은 하위군이 존재하고 그에 따른 치료법이나 치과적 주의사항도 달라진다. 본 증례는 치아의 발육지연 여부 평가를 주소로 내원한 정신 지체가 동반된 전신성 백색증 환아로서 치아 발거 등의 관혈적 시술 및 보존수복, 공간 유지장치 등의 치료를 통해 양호한 결과를 보였기에 이를 보고하는 바이다.

Albinism is a disease caused by partial or complete failure of melanin production in the skin, hair and eyes despite the presence of normal number, structure and distribution of melanocytes. Typical aspects are white-colored skin, blonde-brown hair, blue-brown irides and a prominent red reflex. Three main categories of albinism are oculocutaneous, ocular and localized albinism, and also they are divided into many subgroups. Therefore, appropriate treatment plan and dental direction would be differentiated according to them. This case report was about oral conditions and treatment of the oculocutaneous albinism patient with mental retardation who was refered due to developmental delay of teeth and treated with teeth extraction, restoration and space maintenance etc.

키워드

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