Lesch–Nyhan syndrome: a case report
Lesch–Nyhan syndrome: a case report
- 대한구강악안면외과학회
- 대한구강악안면외과학회지
- 제49권 제4호
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2023.08228 - 232 (5 pages)
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DOI : 10.5125/jkaoms.2023.49.4.228
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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.
I. Introduction
II. Case Report
III. Discussion
ORCID
Authors’ Contributions
Funding
Consent for Publishing Photographs
Conflict of Interest
References
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