| ORIGINAL ARTICLE |
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| Year : 2008 | Volume
: 26
| Issue : 5 | Page : 11-13 |
Lesch-Nyhan syndrome: A case report
A Kale1, K Shah2, S Hallikerimath2
1 Department of Oral and Maxillofacial Pathology, KLE Dental College and Hospital, Belgaum, Karnataka, India
2 Teaching Faculty, KLE Dental College and Hospital, Belgaum, Karnataka, India
Correspondence Address:
A Kale
KLE Dental College and Hospital, Belgaum, Karnataka
India
PMID: 18974538
Lesch-Nyhan syndrome, a rare inborn error of metabolism, is characterized by mental retardation and self-destructive behavior resulting in self-mutilation through biting and scratching. It is a rare anomaly consisting of deficiency in the production of hypoxanthine phosphoribosyltransferase that leads to the overproduction of purine and accumulation of uric acid. No medical treatment exists to alleviate the symptoms of self-mutilation where direct dental intervention is the only way these behavior can be affected. A unique case of this type affecting a 6-year-old male child is reported.
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