title: Orofacial aspects in Noonan syndrome: 2 case report.
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.
authors.affiliation: Federal University of São Paulo, Brazil. aliraort@uol.com.br
authors.forename: Adriana de Oliveira Lira
authors.initials: Ade O
authors.lastname: Ortega
authors.forename: Renata de Oliveira
authors.initials: Rde O
authors.lastname: Guaré
authors.forename: Nilce Samecima
authors.initials: NS
authors.lastname: Kawaji
authors.forename: Ana Lídia
authors.initials: AL
authors.lastname: Ciamponi
mesh_terms: D000293:Adolescent; D002508:Cephalometry; D003680:Deglutition Disorders / Q000209:etiology; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000150:complications; D003773:Dental Plaque / Q000150:complications; D019066:Facies; D005891:Gingivitis / Q000150:complications; D006801:Humans; D008297:Male; D008312:Malocclusion, Angle Class II / Q000209:etiology*; D009634:Noonan Syndrome / Q000150:complications*; D011378:Prognathism / Q000209:etiology*; D063173:Retrognathia / Q000209:etiology*