레쉬니한 증후군(Lesch-Nyhan syndrome) 환자의 치아와 연관된 자해행동의 관리: 증례보고
Management of Lesch-Nyhan Syndrome patients with self-mutilation behavior using their teeth : Case reports
이지미, 이상호, 이난영, 지명관,
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이지미 ( Lee Ji-Mi ) - 조선대학교 치의학전문대학원 소아치과학교실
이상호 ( Lee Sang-Ho ) - 조선대학교 치의학전문대학원 소아치과학교실
이난영 ( Lee Nan-Young ) - 조선대학교 치의학전문대학원 소아치과학교실
지명관 ( Jih Myeong-Kwan ) - 조선대학교 치의학전문대학원 소아치과학교실
Abstract
Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.
키워드
Lesch-Nyhan syndrome; Self-mutilation behavior; Soft mouthguard; Soft tissue defect; Extraction
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