대한소아치과학회지 (Journal of the korean academy of Pediatric Dentistry)

  • 제38권1호
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  • Pages.68-74
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  • 2011
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  • 1226-8496(pISSN)
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  • 2288-3819(eISSN)
대한소아치과학회 (Korean Academy of Pediatric Dentistry)
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Rett syndrome 환자의 증례보고

RETT SYNDROME : CASE REPORT

  • 공은경 (이화여자대학교 목동병원 소아치과) ;
  • 홍소이 (이화여자대학교 목동병원 소아치과) ;
  • 마연주 (이화여자대학교 목동병원 소아치과) ;
  • 안병덕 (이화여자대학교 목동병원 소아치과) ;
  • 정영정 (이화여자대학교 목동병원 소아치과)
  • Kong, Eun-Kyung (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Hong, So-Yi (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Mah, Yon-Joo (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Ahn, Byung-Duk (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Jung, Young-Jung (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
  • 투고 : 2010.10.15
  • 심사 : 2010.12.18
  • 발행 : 2011.02.28
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초록

레트 증후군은 X-염색체 연관 신경발달 장애로 주로 여아에서 발병하며 특히 언어 표현, 손의 사용 발달의 퇴행 및 심각한 지적 장애를 동반한다. 구강내 증상은 경련, 구강 위생 불량, 보행 장애, 구강 습관과 관련이 있고, 특히 이갈이가 가장 흔한 증상이다. 본 증례에서 레트 증후군 환아는 치아우식증을 주소로 내원하여 전신마취 하에 치료를 받았으며, 손의 상동증(stereotypic hand movement), 이갈이, 손가락 빨기 등의 전형적인 증상을 보였다.

Rett syndrome is a X-linked genetic neurological disorder characterized by developmental regression, particularly in relation to expressive language and use of the hands, together with profound mental retardation, that almost exclusively affects females. Oral manifestations of the disease are commonly associated with the clinical conditions such as convulsion activity, difficulties for oral hygiene behavior, walking problems and oral/digitalmanual habits. Bruxism is the most frequently observed oral habit in patients with Rett syndrome. Two cases with the Rett syndrome were reported. Both patients had the typical manifestations like stereotypic hand movement, bruxism and digit-hand sucking. Caries control for the patients was perfomed under general anesthesia.

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참고문헌

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