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DENTAL TREATMENT OF CHILDREN WITH ANGELMAN SYNDROME : CASE REPORTS

Angelman syndrome 환자의 치과치료 : 증례보고

  • Bak, So-Yeon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Chong-Chul (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Lee, Sang-Hoon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Jang, Ki-Taeg (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Jung-Wook (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Young-Jae (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo-Jeon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Hyun, Hong-Keu (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 박소연 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김종철 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2011.09.30
  • Accepted : 2011.12.10
  • Published : 2011.12.30

Abstract

Angelman syndrome(AS) is a rare genetic neurological disorder. The main clinical characteristics of this syndrome are delayed neuropsychological development, intellectual disability, speech impairment, jerky movements especially hand-lapping, frequent laughter or smiling. AS is a classic example of genetic imprinting in that it is usually caused by deletion or inactivation of genes on the maternally inherited chromosome 15. The syndrome has oral manifestations such as diastemas, tongue thrusting, sucking/swallowing disorder, mandibular prognathism, frequent drooling, and excessive chewing behavior. The purpose of this paper is to describe the interesting aspects of the dental treatment of a childe with AS.

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