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Case Reports
. 1994 Feb;23(1):37-8.
doi: 10.1016/s0901-5027(05)80324-8.

Lesch-Nyhan syndrome: successful prevention of lower lip ulceration caused by self-mutilation by use of mouth guard

T Sugahara  1 , K Mishima, Y Mori
Affiliations
Case Reports

Lesch-Nyhan syndrome: successful prevention of lower lip ulceration caused by self-mutilation by use of mouth guard

T Sugahara et al. Int J Oral Maxillofac Surg. 1994 Feb.

Abstract

Lesch-Nyhan syndrome (LNS) is a disorder caused by congenital absence of a purine metabolic enzyme, hypoxanthine guanine phosphoribosyl-transferase (HGPRT). This syndrome is characterized clinically by hyperuricemia and neurologic features including choreoathetoid spasticity, self-mutilation, and mental retardation. We report on a patient in whom self-mutilation of the lower lip was suppressed with the help of a mouth guard made from soft resin.

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