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先天性面肌无力罕见病相关的口腔健康生活质量。

Oral Health-Related Quality of Life in Rare Disorders of Congenital Facial Weakness.

机构信息

Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA.

Department of Orofacial Sciences, School of Dentistry, University of California, San Francisco, CA 94143, USA.

出版信息

Int J Environ Res Public Health. 2024 May 13;21(5):615. doi: 10.3390/ijerph21050615.

Abstract

Congenital facial weakness (CFW) encompasses a heterogenous set of rare disorders presenting with decreased facial movement from birth, secondary to impaired function of the facial musculature. The aim of the present study is to provide an analysis of subject-reported oral health-related quality of life (OHRQoL) in congenital facial weakness (CFW) disorders. Forty-four subjects with CFW and age- and sex- matched controls were enrolled in an Institutional Review Board (IRB)-approved study. Demographic data, medical and surgical history, comprehensive oral examination, and the Oral Health Impact Profile (OHIP-14) were obtained. Compared to unaffected controls, subjects with CFW had higher OHIP-14 scores overall (mean ± SD: 13.11 ± 8.11 vs. 4.46 ± 4.98, < 0.0001) and within five of seven oral health domains, indicating decreased OHRQoL. Although subjects with Moebius syndrome (MBS) were noted to have higher OHIP-14 scores than those with Hereditary Congenital Facial Paresis (HCFP), there was no significant correlation in OHIP-14 score to age, sex, or specific diagnosis. An increase in OHIP-14 scores in subjects was detected in those who had undergone reanimation surgery. In conclusion, subjects with CFW had poorer OHRQoL compared to controls, and subjects with MBS had poorer OHRQoL than subjects with HCFP. This study provides better understanding of oral health care needs and quality of life in a CFW cohort and suggests that guidelines for dental treatment are required.

摘要

先天性面肌无力(CFW)包含一组异质性罕见疾病,表现为出生后面部运动减弱,继发于面部肌肉功能障碍。本研究旨在分析先天性面肌无力(CFW)患者的主观报告口腔健康相关生活质量(OHRQoL)。44 例 CFW 患者和年龄、性别匹配的对照组被纳入机构审查委员会(IRB)批准的研究。获取人口统计学数据、医疗和手术史、全面口腔检查和口腔健康影响简表(OHIP-14)。与无影响的对照组相比,CFW 患者的 OHIP-14 评分总体较高(平均值±标准差:13.11±8.11 比 4.46±4.98,<0.0001),且在七个口腔健康领域中的五个领域内评分较高,表明 OHRQoL 下降。尽管 Moebius 综合征(MBS)患者的 OHIP-14 评分高于遗传性先天性面肌无力(HCFP)患者,但 OHIP-14 评分与年龄、性别或特定诊断均无显著相关性。在接受再神经支配手术的患者中,OHIP-14 评分增加。结论:与对照组相比,CFW 患者的 OHRQoL 较差,MBS 患者的 OHRQoL 较 HCFP 患者差。本研究更好地了解了 CFW 患者群体的口腔保健需求和生活质量,并提示需要制定牙科治疗指南。

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