Lesch–Nyhan syndrome: a case report
Journal of the Korean Association of Oral and Maxillofacial Surgeons
; : 228-232, 2023.
Article
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| WPRIM
| ID: wpr-1001620
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WPRO
ABSTRACT
Lesch–Nyhan syndrome (LNS) is a rare X-linked recessive disorder caused by a mutation in the hypoxanthine phosphoribosyltransferase 1 (HPRT1) gene. This syndrome is characterized by excessive production of uric acid, mental retardation, self-mutilation, choreoathetosis, and spasticity. The most distinctive symptom is compulsive self-mutilation. For patients with LNS, different methods have been tried to reduce self-biting behaviors including restraints, behavioral treatment, medications, deep brain stimulation, tooth extraction and botulinum toxin A injection. In this report, we present a case of LNS undergoing cheiloplasty due to self-mutilation and tooth extraction of the left deciduous maxillary canine.
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WPRIM
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Revista:
Journal of the Korean Association of Oral and Maxillofacial Surgeons
Año:
2023
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Article