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Lesch-Nyhan 증후군 환아의 자해에 의한 혀의 외상

Other Titles
 TONGUE INJURIES BY SELF MUTILATION IN LESCH-NYHAN SYNDROME PATIENT: A CASE REPORT 
Authors
 김지희  ;  최병재  ;  김성오  ;  최형준  ;  손흥규  ;  이제호 
Citation
 Journal of the Korean Academy of Pediatric Dentistry (대한소아치과학회지), Vol.35(3) : 532-538, 2008 
Journal Title
 Journal of the Korean Academy of Pediatric Dentistry (대한소아치과학회지) 
ISSN
 1226-8496 
Issue Date
2008
Keywords
Lesch-Nyhan syndrome ; Self mutilation ; Tongue wound ; Mouth guard ; Extraction of teeth
Abstract
Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence or decrease in activity of hypoxanthine guanine phosphoribosyl transferase(HPRT), an enzyme involved in purine metabolism. This enzyme deficiency gives rise to nephropathy symptoms, such as hyperuricosuria and hyperuricemia by excessive uric acid production and neuropathy symptoms, such as mental retardation, choreoathetosis and self mutilation behavior. Patients with Lesch-Nyhan syndrome have tendency to bite their lip, tongue and finger. In severe cases, partial or even total amputation of tongue or finger occur. Self-inflicted bites are often complicated by secondary infection to the injured site as well as pain. Furthermore tissue loss by biting results in esthetic problems. The dental management of self mutilation includes treatment with appliances such as soft mouth guard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a case of a 13 year-old boy with Lesch-Nyhan syndrome, who severely injured himself on his tongue. At first, conservative treatment using soft mouth guard was considered, but it could not prevent trauma on his tongue. Therefore, extraction of the lower anterior and posterior teeth was carried out.
Files in This Item:
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Appears in Collections:
2. College of Dentistry (치과대학) > Dept. of Pediatric Dentistry (소아치과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Seong Oh(김성오) ORCID logo https://orcid.org/0000-0002-8620-1377
Son, Heung Kyu(손흥규)
Lee, Jae Ho(이제호) ORCID logo https://orcid.org/0000-0002-1556-3485
Choi, Byung Jai(최병재)
Choi, Hyung Jun(최형준) ORCID logo https://orcid.org/0000-0002-3315-6912
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/107081
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