TY  - JOUR
PY  - 2006//
TI  - A preventive approach to oral self-mutilation in Lesch-Nyhan syndrome: a case report
JO  - Pediatric dentistry
A1  - Jeong, Tae-Sung
A1  - Lee, Jung-Hua
A1  - Kim, Sungyop
A1  - Kim, Joo-Heon
A1  - Tootla, Ruwaida G. H.
SP  - 341
EP  - 344
VL  - 28
IS  - 4
N2  - 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>
LA  - en
SN  - 0164-1263
UR  - http://dx.doi.org/
ID  - ref1
ER  -