@article{ref1,
title="A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome: a case report",
journal="Pediatric dentistry",
year="2006",
author="Jeong, Tae-Sung and Lee, Jung-Hua and Kim, Sungyop and Kim, Joo-Heon and Tootla, Ruwaida G. H.",
volume="28",
number="4",
pages="341-344",
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.<p /> <p>Language: en</p>",
language="en",
issn="0164-1263",
doi="",
url="http://dx.doi.org/"
}