편친 이염색체성 엔젤만 증후군(Angelman syndrome) 환자의 치과치료
DENTAL TREATMENT IN A PATIENT WITH ANGELMAN SYNDROME DUE TO UNIPARENTAL DISOMY
박성희, 신터전, 현홍근, 김영재, 김정욱, 이상훈, 김종철, 장기택,
소속 상세정보
박성희 ( Park Sung-Hee ) - 서울대학교 치의학대학원 소아치과학교실
신터전 ( Shin Teo-Jeon ) - 서울대학교 치의학대학원 소아치과학교실
현홍근 ( Hyun Hong-Keun ) - 서울대학교 치의학대학원 소아치과학교실
김영재 ( Kim Young-Jae ) - 서울대학교 치의학대학원 소아치과학교실
김정욱 ( Kim Jung-Wook ) - 서울대학교 치의학대학원 소아치과학교실
이상훈 ( Lee Sang-Hoon ) - 서울대학교 치의학대학원 소아치과학교실
김종철 ( Kim Chong-Chul ) - 서울대학교 치의학대학원 소아치과학교실
장기택 ( Jang Ki-Taeg ) - 서울대학교 치의학대학원 소아치과학교실
KMID : 1144020160120010011
Abstract
Angelman syndrome is characterized by a partial deficit of paired autosomal chromosome 15, which contains a subunit of the GABA (Gamma-Amino Butyric Acid) receptor. The main clinical characteristics of this syndrome are delayed neuropsychological development, intellectual disability, and speech impairment. The syndrome has oral manifestations such as diastemas, tongue thrusting, sucking swallowing disorder, and excessive chewing behavior. A 3-year-6-month old girl with Angelman syndrome was scheduled for dental treatment. She had multiple caries, but she was poorly cooperative for treatment due to developmental delay and movement disorder, so general anesthesia was considered.
The patient with Angelman syndrome was successfully treated under general anesthesia. There were no postoperative complications related to anesthesia and dental treatment. When treating children with this syndrome, the dentist needs to manage their uncooperative behavior and medical problem.
키워드
Angelman syndrome; Uniparental disomy; Dental treatment; General anesthesia
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