Dental evaluation of Kabuki syndrome patients.

Autor: Teixeira CS; Genetics Unit, Instituto da Criança, University of São Paulo, 05403-900 São Paulo-SP, Brazil. ca_steixeira@yahoo.com, Silva CR, Honjo RS, Bertola DR, Albano LM, Kim CA
Jazyk: angličtina
Zdroj: The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association [Cleft Palate Craniofac J] 2009 Nov; Vol. 46 (6), pp. 668-73. Date of Electronic Publication: 2009 May 17.
DOI: 10.1597/08-077.1
Abstrakt: Kabuki syndrome is a genetic disorder of unknown etiology characterized by mental retardation, growth deficiency, and peculiar face (i.e., long palpebral fissures, eversion of the lateral third of the lower eyelids, prominent ears, and broad and depressed nasal tip). Oral manifestations commonly observed in Kabuki syndrome may comprise cleft lip/palate, bifid tongue and uvula, malocclusion, and dental abnormalities. We evaluated the dental findings of eight patients with Kabuki syndrome. One presented cleft palate; three presented caries; and seven had missing teeth, with the upper lateral incisors and inferior central incisors being the most commonly absent. All missing teeth were permanent, and there was no alteration of dental chronology or morphology. Because most patients had mixed dentition, the presence or absence of primary teeth was assessed through the parents' reports. One patient presented an absent upper canine, which had not been reported previously in the literature. Dental findings may be helpful for clinical diagnosis, or they may be an additional finding to substantiate the diagnosis of Kabuki syndrome in children with mild phenotype.
Databáze: MEDLINE