title: Lesch-Nyhan syndrome: successful prevention of lower lip ulceration caused by self-mutilation by use of mouth guard.
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.
authors.affiliation: Second Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Osaka University, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Sugahara
authors.forename: K
authors.initials: K
authors.lastname: Mishima
authors.forename: Y
authors.initials: Y
authors.lastname: Mori
mesh_terms: D006801:Humans; D007223:Infant; D007926:Lesch-Nyhan Syndrome / Q000628:therapy*; D008046:Lip / Q000293:injuries*; D008297:Male; D009064:Mouth Protectors; D012652:Self Mutilation / Q000517:prevention & control*