LESCH-NYHAN SYNDROME: A CASE REPORT

Kim, Myoung-Gook; Yang, Kyu-Ho; Choi, Nam-Ki; Kim, Seon-Mi

Journal of the Korean Academy of Pediatric Dentistry 2011;38(3):284-289.
Published online August 31, 2011.

LESCH-NYHAN SYNDROME: A CASE REPORT

Myoung-Gook Kim, Kyu-Ho Yang, Nam-Ki Choi, Seon-Mi Kim

Department of Pediatric Dentistry, School of Dentistry, Chonnam National University

Lesch-Nyhan syndrome: 증례보고

김명국, 양규호, 최남기, 김선미

전남대학교 치과대학 소아치과학교실

Correspondence: Myoung-Gook Kim, Tel: 010-2861-2467, Email: audrnrdl@hanmail.net

Received: 26 April 2011 • Accepted: 20 July 2011

Abstract

esch-Nyhan syndrome is a disease caused by metabolic disorder of purine. General muscle stiffness and hyposomia are shown from infancy and symptoms can include involuntary or irregular movements of arms and legs, mental retardation, and compulsive self-mutilating behaviors. Self-mutilating behaviors begin at approximately the first year or sometimes at late teens. The patients bite their lips, especially lower lip, tongue, buccal mucosa, hands and fingers. Tongue and lips can be injured or mutilated in severe cases. As the patient gets older, self-mutilating behaviors become more serious and extensive and secondary infection of injured areas is possible. Periodic soft tissue damage due to self-mutilating may evolve to cancer. Medical treatment, appliance treatment, extraction of tooth and surgical operation was attempted to control self-mutilaing behaviors. We hereby report the case of child Lesch-Nyhan syndrome patient who has self-inflicted labial damage as chief complaint. When patient was treated with conservate therapy, such as removable or fixed appliance, the frequency of labial damage could be subdued and yielded favorable results.

Key Words: Lesch-Nyhan syndrome, Compulsive self-mutilation, Lip bumper