Document Detail

Lesch-Nyhan syndrome. A case report.
MedLine Citation:
PMID:  7970591     Owner:  NLM     Status:  MEDLINE    
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.
B M Smith; B J Cutilli; M Fedele
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Oral surgery, oral medicine, and oral pathology     Volume:  78     ISSN:  0030-4220     ISO Abbreviation:  Oral Surg. Oral Med. Oral Pathol.     Publication Date:  1994 Sep 
Date Detail:
Created Date:  1994-12-20     Completed Date:  1994-12-20     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0376406     Medline TA:  Oral Surg Oral Med Oral Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  317-8     Citation Subset:  D; IM    
Department of Oral and Maxillofacial Surgery, Temple University School of Dentistry, Philadelphia, Pa.
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MeSH Terms
Diseases in Twins*
Lesch-Nyhan Syndrome / physiopathology*
Lip / injuries
Self Mutilation / surgery*
Tongue / injuries
Tooth Extraction*

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