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Orofacial aspects in Noonan syndrome: 2 case report.

作者信息

Ortega Adriana de Oliveira Lira, Guaré Renata de Oliveira, Kawaji Nilce Samecima, Ciamponi Ana Lídia

机构信息

Federal University of São Paulo, Brazil.

出版信息

J Dent Child (Chic). 2008 Jan-Apr;75(1):85-90.

Abstract

Noonan syndrome (NS) is an autosomal dominant disorder characterized by dysmorphic facial features in association with short stature and heart disease. A webbed neck, chest deformities, mental retardation, and bleeding disorders are also frequently associated with this pathology. NS is relatively common, with an estimated incidence of 1 per 1,000 to 1 per 2,500 live births. Many cases have been reported in the dental literature, yet only a few of them describe some details of the oral features. The aim of this paper is to describe 2 cases of Noonan syndrome, emphasizing oral and facial aspects and particularities of the dental treatment in subjects affected by this genetic disease.

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