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Dental evaluation of Kabuki syndrome patients.
Cleft Palate Craniofac J. 2009 Nov; 46(6):668-73.CP

Abstract

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.

Authors+Show Affiliations

Genetics Unit, Instituto da Criança, University of São Paulo, 05403-900 São Paulo-SP, Brazil. ca_steixeira@yahoo.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19860501

Citation

Teixeira, Camila Santos, et al. "Dental Evaluation of Kabuki Syndrome Patients." The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association, vol. 46, no. 6, 2009, pp. 668-73.
Teixeira CS, Silva CR, Honjo RS, et al. Dental evaluation of Kabuki syndrome patients. Cleft Palate Craniofac J. 2009;46(6):668-73.
Teixeira, C. S., Silva, C. R., Honjo, R. S., Bertola, D. R., Albano, L. M., & Kim, C. A. (2009). Dental evaluation of Kabuki syndrome patients. The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association, 46(6), 668-73. https://doi.org/10.1597/08-077.1
Teixeira CS, et al. Dental Evaluation of Kabuki Syndrome Patients. Cleft Palate Craniofac J. 2009;46(6):668-73. PubMed PMID: 19860501.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Dental evaluation of Kabuki syndrome patients. AU - Teixeira,Camila Santos, AU - Silva,Claudia Renata Leite, AU - Honjo,Rachel Sayuri, AU - Bertola,Débora Romeo, AU - Albano,Lílian Maria José, AU - Kim,Chong Ae, Y1 - 2009/05/17/ PY - 2009/10/29/entrez PY - 2009/10/29/pubmed PY - 2010/2/27/medline SP - 668 EP - 73 JF - The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association JO - Cleft Palate Craniofac. J. VL - 46 IS - 6 N2 - 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. SN - 1055-6656 UR - https://www.unboundmedicine.com/medline/citation/19860501/Dental_evaluation_of_Kabuki_syndrome_patients_ L2 - http://journals.sagepub.com/doi/full/10.1597/08-077.1?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -