Fragile X Syndrome: Medical Considerations and Stomatological Aspects for Dental Treatment

C. Amaral, Nilzete Santana Silva, Alexsander Guedes, Mariana Olívia Ferreira Do Amaral, Eliane-Cristina-Gava Pizi, F. G. Straioto
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Abstract

The objective of this study was to carry out a literature review  in order to  elucidate general and oral physical aspects, behavioral disorders, medical considerations  and discuss   considerations  about the dental approach in individuals with Fragile X Syndrome (FXS) as well as to provide  surgeons dentists with   greater care clarification towards these patients. A literature review has been  carried out in the following databases: Virtual Health Library (VHL) –MEDLINE– PubMed , SciELO and in the  Capes database  Journals. The descriptors used were: Fragile X Syndrome, Oral Health, Systemic Condition, Behavioral Disorders. The main phenotypic characteristics found in the literature were: prominent ears, elongated face, strabismus, hyperextensible joints , macrorchidism , mitral valve prolapse, seizures. Fragile X Syndrome  oral characteristics  are: mandibular prognathism, atresic and deep palate, enamel hypoplasia, malocclusion, presence of biofilm due to unestablished hygiene habits, caries, calculus and gingivitis. Cognitive deficit, autism spectrum disorder, anxiety disorder and hyperactivity are behaviors found in FXS  which bring   difficulties for these patients to undergo  a dental approach. The dental surgeon must be up-to-date and aware of the medical and behavioral conditions of FXS individuals and, thus, offer an adequate and safe dental approach. Systemic, behavioral and oral abnormalities require an initial  dental treatment planning.  
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脆性X综合征:牙科治疗的医学考虑和口腔方面
本研究的目的是进行文献综述,以阐明一般和口腔身体方面,行为障碍,医学注意事项,并讨论脆性X综合征(FXS)患者牙科治疗的注意事项,并为外科医生牙医对这些患者提供更大的护理说明。在以下数据库中进行了文献综述:虚拟健康图书馆(VHL) - medline - PubMed, SciELO和Capes数据库期刊。使用的描述词是:脆性X综合征、口腔健康、全身状况、行为障碍。文献中发现的主要表型特征为:耳朵突出、面部拉长、斜视、关节过伸、大睾丸、二尖瓣脱垂、癫痫发作。脆性X综合征的口腔特征是:下颌前突、闭锁和深腭、牙釉质发育不全、错牙合、由于未建立的卫生习惯而出现的生物膜、龋齿、牙石和牙龈炎。认知缺陷、自闭症谱系障碍、焦虑障碍和多动是FXS患者的行为,这些行为给这些患者接受牙科治疗带来了困难。牙科医生必须了解FXS个人的最新医疗和行为状况,从而提供适当和安全的牙科治疗方法。全身、行为和口腔异常需要初步的牙科治疗计划。
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