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LESCH-NYHAN SYNDROME: A CASE REPORT
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 Title & Authors
LESCH-NYHAN SYNDROME: A CASE REPORT
Kim, Myoung-Gook; Yang, Kyu-Ho; Choi, Nam-Ki; Kim, Seon-Mi;
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 Abstract
Lesch-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.
 Keywords
Lesch-Nyhan syndrome;Compulsive self-mutilation;Lip bumper;
 Language
Korean
 Cited by
1.
Self-inflicted Tongue Ulceration in a Patient with Tourette Syndrome: A Case Report, THE JOURNAL OF THE KOREAN ACADEMY OF PEDTATRIC DENTISTRY, 2016, 43, 3, 327  crossref(new windwow)
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