Oral self-mutilation in the Lesch-Nyhan syndrome.
Lesch-Nyhan Syndrome is a disorder caused by congenital absence of a purine metabolic enzyme, hypoxanthine-guanine phosphoribosyl transferase (HPRT). This syndrome is characterized clinically by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilation. This report is of two children, who are cousins, both of whom have Lesch-Nyhan syndrome and presented with severe self-mutilation wounds on their lip(s). Vital pulpotomy and coronal resection was done as a more conservative approach than extracting all offending teeth. By maintaining the root portion of the teeth in the bone, it is expected that preservation of the alveolar bone will be achieved.
Lee
Jae-Ho
JH
Department of Pediatric Dentistry, Yonsei University College of Dentistry, Seoul, Korea.
Berkowitz
Robert J
RJ
Choi
Byung-Jai
BJ
D001641:Bicuspid / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000209:etiology; D003481:Cuspid / Q000601:surgery; D005500:Follow-Up Studies; D005899:Glass Ionomer Cements; D006801:Humans; D007180:Incisor / Q000601:surgery; D007926:Lesch-Nyhan Syndrome / Q000150:complications*; D008046:Lip / Q000293:injuries*; D008297:Male; D011672:Pulpotomy; D012008:Recurrence; D012652:Self Mutilation / Q000209:etiology / Q000628:therapy*; D019228:Tooth Crown / Q000601:surgery; D014094:Tooth, Deciduous / Q000601:surgery; D014945:Wound Healing
D005899:Glass Ionomer Cements