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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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 ORIGINAL ARTICLE
Year : 2008  |  Volume : 26  |  Issue : 5  |  Page : 11-13

Lesch-Nyhan syndrome: A case report


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
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Source of Support: None, Conflict of Interest: None


PMID: 18974538

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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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  2005 - Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow 
Online since 1st May '05