title: A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome: a case report.
abstract: Lesch-Nyhan syndrome is a rare X-linked recessive disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. Persons affected with this incurable disease are developmentally and physically delayed, and suffer from self-injurious behavior. The most typical feature results in partial or total destruction of perioral tissues. The purpose of this paper was to discuss a case of Lesch-Nyhan syndrome with self-mutilative behavior and lip injuries, including some suggestions of a preventive approach avoiding the extraction of teeth. A soft mouthguard fabricated to prevent the destruction of perioral soft tissues and combined psychiatric pharmacologic therapy proved to have satisfactory results.
authors.affiliation: Department of Pediatric Dentistry, College of Dentistry, Pusan National University, Busan, Korea. tsjeong@pusan.ac.kr
authors.forename: Tae-Sung
authors.initials: TS
authors.lastname: Jeong
authors.forename: Ji-Hyun
authors.initials: JH
authors.lastname: Lee
authors.forename: Shin
authors.initials: S
authors.lastname: Kim
authors.forename: Ji-Hoon
authors.initials: JH
authors.lastname: Kim
authors.forename: Ruwaida G H
authors.initials: RG
authors.lastname: Tootla
mesh_terms: D002675:Child, Preschool; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000150:complications*; D008046:Lip / Q000293:injuries*; D008297:Male; D009064:Mouth Protectors; D018967:Risperidone / Q000627:therapeutic use; D012652:Self Mutilation / Q000209:etiology* / Q000517:prevention & control*; D018490:Serotonin Agents / Q000627:therapeutic use; D020280:Sertraline / Q000627:therapeutic use
chemical_list: D018490:Serotonin Agents; D018967:Risperidone; D020280:Sertraline