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- Title
Craniofacial and Dental Features in Kabuki Syndrome Patients.
- Authors
do Prado Sobral, Samantha; Ferreira Leite, André; Sousa Figueiredo, Paulo Tadeu; Ferrari, Íris; Neto Safatle, Heloísa Pires; Santos Córdoba, Mara; Ribeiro Versiani, Beatriz; Acevedo, Ana Carolina; Dantas Mestrinho, Heliana
- Abstract
Objective: To describe oral manifestations in Brazilian individuals with Kabuki syndrome, a multiple congenital anomaly/mental retardation syndrome. Study Design: A total of 16 Kabuki syndrome individuals, aged between B to 24 years and of both sexes, were referred by the Department of Clinical Genetics for oral treatment and follow- up to the Oral Care Center for Inherited Diseases, University Hospital of Brasilia, Brasilia, Brazil. Each individual underwent complete physical examination, as well as intraoral and radiographic examinations. Results: Craniofacial and dental alterations were observed in all Kabuki syndrome patients examined. In addition, atypical shape of the molars' crowns, occlusal convergence of the premolars' crowns, and root dilaceration were also observed. Enamel diffuse opacities were observed in permanent dentition (n = 10). Conclusion: A great clinical heterogeneity was observed in Kabuki syndrome individuals in line with previous studies in the literature. Further clinical and molecular studies are necessary in order to better understand the presence of dental anomalies in this syndrome.
- Subjects
BRAZIL; ACADEMIC medical centers; GENES; GENETIC mutation; PHYSICAL diagnosis; QUESTIONNAIRES; TEETH abnormalities; PHENOTYPES; EARLY medical intervention; DATA analysis software; CRANIOFACIAL abnormalities; DESCRIPTIVE statistics; KABUKI syndrome; DIAGNOSIS
- Publication
Cleft Palate Craniofacial Journal, 2013, Vol 50, Issue 4, p440
- ISSN
1055-6656
- Publication type
Article
- DOI
10.1597/11-052