Dental Findings in Kabuki Syndrome: A Systematic Review for Dentistry
Abstract: Kabuki syndrome consists in a series of congenital anomalies involving intellectual disability, hypotonia, and facial and body dysmorphism. In addition to these cardinal signs, craniofacial and dental changes are frequently observed, although they are not fully understood. Thus, the aim of this article is to present the dental findings in patients with Kabuki syndrome. This is a systematic review developed according to the PRISMA protocol. A search strategy with descriptors ("kabuki syndrome" AND "dentistry") was applied to six databases. As inclusion criteria, we selected clinical case reports that addressed dental findings in one or more patients with Kabuki syndrome between 1999 and 2019, without restrictions regarding gender, age or language. Data collection in all databases was performed by a calibrated evaluator and titles, abstracts and full-text studies were systematically analyzed. Eleven case reports that fit the proposed inclusion criteria were found. After the qualitative synthesis, teeth absence or agenesis were the most reported findings, being observed by nine authors. Less frequent dental findings in patients with this syndrome are taurodontism, microdontia, fusion, gemination and conical or screwdriver-shaped teeth. In fact, when any syndrome or genetic condition is related to manifestations of dental interest, the dental surgeon should be able to investigate them, especially teeth agenesis or absence. For this reason, the dentist should be aware of the manifestations of Kabuki syndrome, contributing to patients' understanding and management.
| Main Authors: | , , , , |
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| Format: | Digital revista |
| Language: | English |
| Published: |
Facultad de Odontología. Universidad de Costa Rica
2020
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| Online Access: | http://www.scielo.sa.cr/scielo.php?script=sci_arttext&pid=S2215-34112020000200062 |
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| Summary: | Abstract: Kabuki syndrome consists in a series of congenital anomalies involving intellectual disability, hypotonia, and facial and body dysmorphism. In addition to these cardinal signs, craniofacial and dental changes are frequently observed, although they are not fully understood. Thus, the aim of this article is to present the dental findings in patients with Kabuki syndrome. This is a systematic review developed according to the PRISMA protocol. A search strategy with descriptors ("kabuki syndrome" AND "dentistry") was applied to six databases. As inclusion criteria, we selected clinical case reports that addressed dental findings in one or more patients with Kabuki syndrome between 1999 and 2019, without restrictions regarding gender, age or language. Data collection in all databases was performed by a calibrated evaluator and titles, abstracts and full-text studies were systematically analyzed. Eleven case reports that fit the proposed inclusion criteria were found. After the qualitative synthesis, teeth absence or agenesis were the most reported findings, being observed by nine authors. Less frequent dental findings in patients with this syndrome are taurodontism, microdontia, fusion, gemination and conical or screwdriver-shaped teeth. In fact, when any syndrome or genetic condition is related to manifestations of dental interest, the dental surgeon should be able to investigate them, especially teeth agenesis or absence. For this reason, the dentist should be aware of the manifestations of Kabuki syndrome, contributing to patients' understanding and management. |
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