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KMID : 1144020180140020097
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Journal of Korean Association for Disability and Oral Health
2018 Volume.14 No. 2 p.97 ~ p.101
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Management of Lesch-Nyhan Syndrome patients with self-mutilation behavior using their teeth : Case reports
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Lee Ji-Mi
Lee Sang-Ho
Lee Nan-Young
Jih Myeong-Kwan
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Abstract
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Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.
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KEYWORD
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Lesch-Nyhan syndrome, Self-mutilation behavior, Soft mouthguard, Soft tissue defect, Extraction
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