Lesch-Nyhan syndrome. A case report.
Lesch-Nyhan syndrome is a rare anomaly consisting of a deficiency in the production of hypoxanthine phosphoribosyltransferase that leads to the overproduction of purine and the accumulation of uric acid. Major manifestations include mental retardation and self-destructive behavior resulting in self-mutilation through biting and scratching. Because no medical treatment exists to alleviate the symptoms of self-mutilation, direct dental intervention is the only way these behaviors can be affected. A unique case of this type involving two male identical twins is reported.
Department of Oral and Maxillofacial Surgery, Temple University School of Dentistry, Philadelphia, Pa.
B M
BM
Smith
B J
BJ
Cutilli
M
M
Fedele
D002648:Child; D004200:Diseases in Twins; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000503:physiopathology*; D008046:Lip / Q000293:injuries; D008297:Male; D012652:Self Mutilation / Q000601:surgery*; D014059:Tongue / Q000293:injuries; D014081:Tooth Extraction