Williams 증후군 환아의 치의학적 소견에 대한 증례 보고
WILLIAMS SYNDROME : TWO CASES
김지희, 최병재, 최형준, 송제선, 이제호,
소속 상세정보
김지희 ( Kim Ji-Hee ) - 연세대학교 치과대학 소아치과학교실
최병재 ( Choi Byung-Jai ) - 연세대학교 치과대학 소아치과학교실
최형준 ( Choi Hyung-Jun ) - 연세대학교 치과대학 소아치과학교실
송제선 ( Song Je-Seon ) - 연세대학교 치과대학 소아치과학교실
이제호 ( Lee Jae-Ho ) - 연세대학교 치과대학 소아치과학교실
KMID : 1144020080040010012
Abstract
Williams syndrome is a rare genetic disorder with a frequency of one per 20,000~50,000 live births. It is caused by a deletion of one elastin allele located within chromosome subunit 7q11.23(long arm). This syndrome is frequently accompanied by disorders such as congenital heart disease, facial anomalies, mental retardation, and so on. The characteristic facial appearance includes full lips, rounded cheeks, broad forehead, periorbital fullness, flattened bridge of nose, small nose with anteverted nostril, long filtrum and low-set ears. In oral features, hypodontia, high prevalence of dental caries, microdontia, enamel hypoplasia, delayed eruption, and malocclusions have been found. Most adult patients with Williams syndrome lack social adaptability and lead seclusive lives, however, young patients are rather very friendly and talkative, and seem smarter than their actual intellectual quotients. They also tend to favor staying with grown-ups rather than mixing with their peers, and tend to present problematic temper tantrum during dental treatment.
키워드
Williams syndrome
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