BACKGROUND Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder whose oral manifestations and dental phenotypes have not been well-characterized. The aim of this study was to explore the influence of oral manifestations on oral health-related quality of life (OHRQoL) in LDS patients. MATERIAL AND METHODS LDS subjects were assessed by the craniofacial team at the National Institutes of Health Clinical Center Dental Clinic between June 2015 and January 2018. Oral Health Impact Profile (OHIP-14) questionnaire, oral health self-care behavior questionnaire and a comprehensive dental examination were completed for each subject. OHRQoL was assessed using the OHIP-14 questionnaire with higher scores corresponding to worse OHRQoL. Regression models were used to determine the relationship between each oral manifestation and the OHIP-14 scores using a level of significance of p ≤ 0.05. RESULTS A total of 33 LDS subjects (51.5% female) aged 3-57 years (19.6 ± 15.1 years) were included in the study. The OHIP-14 scores (n = 33) were significantly higher in LDS subjects (6.30 [SD 6.37]) when compared to unaffected family member subjects (1.50 [SD 2.28], p < 0.01), and higher than the previously reported scores of the general U.S. population (2.81 [SD 0.12]). Regarding oral health self-care behavior (n = 32), the majority of LDS subjects reported receiving regular dental care (81%) and maintaining good-to-excellent daily oral hygiene (75%). Using a crude regression model, worse OHRQoL was found to be associated with dental hypersensitivity (β = 5.24; p < 0.05), temporomandibular joints (TMJ) abnormalities (β = 5.92; p < 0.01), self-reported poor-to-fair oral health status (β = 6.77; p < 0.01), and cumulation of four or more oral manifestations (β = 7.23; p < 0.001). Finally, using a parsimonious model, self-reported poor-to-fair oral health status (β = 5.87; p < 0.01) and TMJ abnormalities (β = 4.95; p < 0.01) remained significant. CONCLUSIONS The dental hypersensitivity, TMJ abnormalities, self-reported poor-to-fair oral health status and cumulation of four-or-more oral manifestations had significant influence on worse OHRQoL. Specific dental treatment guidelines are necessary to ensure optimal quality of life in patients diagnosed with LDS.

中文翻译：

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Loeys-Dietz综合征（一种罕见的结缔组织疾病）的口腔健康相关生活质量：一项观察性队列研究。

背景技术Loeys-Dietz综合征（LDS）是一种罕见的结缔组织疾病，其口腔表现和牙齿表型尚未得到很好的表征。这项研究的目的是探讨对LDS患者口腔表现对口腔健康相关生活质量（OHRQoL）的影响。材料与方法在2015年6月至2018年1月之间，美国国立卫生研究院临床中心牙科诊所的颅面小组对LDS受试者进行了评估。口腔健康影响概况（OHIP-14）问卷，口腔健康自我护理行为问卷和综合牙科每个科目的考试均已完成。使用OHIP-14问卷对OHRQoL进行评估，分数较高则对应于较差的OHRQoL。回归模型用于确定每种口腔表现与OHIP-14得分之间的关​​系，其显着性水平为p≤0.05。结果共有33名LDS受试者（女性为51.5％）的年龄为3-57岁（19.6±15.1岁）。与未受影响的家庭成员受试者（1.50 [SD 2.28]，p <0.01）相比，LDS受试者的OHIP-14得分（n = 33）显着更高（6.30 [SD 6.37]），并且高于先前报道的OHIP-14得分（n = 33）。美国总人口（2.81 [SD 0.12]）。关于口腔健康自我保健行为（n = 32），大多数LDS受试者报告接受定期牙齿保健（81％）并保持良好至卓越的日常口腔卫生（75％）。使用粗略的回归模型，发现较差的OHRQoL与牙齿过敏有关（β= 5.24; p <0.05），颞下颌关节（TMJ）异常（β= 5.92; p <0.01），自我报告的口腔健康状况不佳（β= 6.77; p <0.01），以及四个或四个以上口腔表现的累积量（β= 7.23; p <0.001）。最后，使用简约模型，自我报告的口腔健康状况差至正常（β= 5.87; p <0.01）和TMJ异常（β= 4.95; p <0.01）仍然很显着。结论牙齿过敏，TMJ异常，自我报告的不良至公平的口腔健康状况以及四或更多口腔表现的累积均对较差的OHRQoL产生了显着影响。特定的牙科治疗指南对于确保诊断为LDS的患者的最佳生活质量必不可少。并累积了四个或更多的口腔表现（β= 7.23； p <0.001）。最后，使用简约模型，自我报告的口腔健康状况差至正常（β= 5.87; p <0.01）和TMJ异常（β= 4.95; p <0.01）仍然很显着。结论牙齿过敏，TMJ异常，自我报告的不良至公平的口腔健康状况以及四或更多口腔表现的累积均对较差的OHRQoL产生了显着影响。特定的牙科治疗指南对于确保诊断为LDS的患者的最佳生活质量必不可少。并累积了四个或更多的口腔表现（β= 7.23； p <0.001）。最后，使用简约模型，自我报告的口腔健康状况差至正常（β= 5.87; p <0.01）和TMJ异常（β= 4.95; p <0.01）仍然很显着。结论牙齿过敏，TMJ异常，自我报告的不良至一般口腔健康状况以及四或更多口腔表现的累积对不良OHRQoL有重要影响。特定的牙科治疗指南对于确保诊断为LDS的患者的最佳生活质量必不可少。TMJ异常，自我报告的口腔健康状况不佳以及四或更多口腔表现的累积对较差的OHRQoL有重要影响。特定的牙科治疗指南对于确保诊断为LDS的患者的最佳生活质量必不可少。TMJ异常，自我报告的口腔健康状况不佳以及四或更多口腔表现的累积对较差的OHRQoL有重要影响。特定的牙科治疗指南对于确保诊断为LDS的患者的最佳生活质量必不可少。