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1 型强直性肌营养不良症和杜氏肌营养不良症患者的开颌畸形和口颌功能障碍。

Open Bite Malocclusion and Orofacial Dysfunction in Patients with Myotonic Dystrophy Type 1 and Duchenne Muscular Dystrophy.

机构信息

Department of Orthodontics, Institute of Odontology, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

Mun-H-Center, Orofacial Resource Centre for Rare Diseases, Public Dental Service, Gothenburg, Sweden.

出版信息

J Neuromuscul Dis. 2023;10(5):885-896. doi: 10.3233/JND-230025.

Abstract

UNLABELLED

Open bite (OB) is a common malocclusion in individuals with orofacial dysfunction and syndromes, especially in neuromuscular diseases.

OBJECTIVES

The objectives were to explore the prevalence of OB in myotonic dystrophy type 1 (DM1) and Duchenne muscular dystrophy (DMD) and to create and compare orofacial dysfunction profiles.

METHODS

In this database study, 143 individuals with DM1 and 99 with DMD were included. The Mun-H-Center questionnaire and observation chart were used together with the Nordic Orofacial Test -Screening (NOT-S) to create orofacial dysfunction profiles. OB was categorised as: lateral (LOB); anterior (AOB); severe anterior (AOBS); or both types of anterior OB (AOBTot). Descriptive and multivariate statistics were used to compare the OB prevalence and to study associations with orofacial variables, respectively.

RESULTS

There was a statistically significant difference in OB prevalence between the DM1 (37%) and DMD (49%) groups (p = 0.048). LOB was seen in < 1% of DM1 and 18% of DMD. LOB was associated with macroglossia and closed mouth posture, AOB with hypotonic lips, and open mouth posture and AOBS with hypotonic jaw muscles. The orofacial dysfunction profiles showed similar patterns, although the mean NOT-S total scores for DM1 and DMD were 4.2±2.8 (median 4.0, min-max 1-8) and 2.3±2.0 (median 2.0, min-max 0-8), respectively.

LIMITATIONS

The two groups were not age- or gender-matched.

CONCLUSION

OB malocclusion is common in patients with DM1 and DMD and is associated with different types of orofacial dysfunction. This study highlights the need for multi-disciplinary assessments to support tailored treatment strategies that improve or sustain orofacial functions.

摘要

未加标签

开颌(OB)是口腔功能障碍和综合征患者,特别是神经肌肉疾病患者中常见的错颌畸形。

目的

本研究旨在探讨 1 型肌强直性营养不良(DM1)和杜氏肌营养不良症(DMD)患者中开颌的患病率,并建立和比较口腔功能障碍特征。

方法

在这项数据库研究中,纳入了 143 名 DM1 患者和 99 名 DMD 患者。使用 Mun-H-Center 问卷和观察表以及北欧口腔测试-筛查(NOT-S)来建立口腔功能障碍特征。将开颌分为:侧方开颌(LOB);前方开颌(AOB);严重前方开颌(AOBS);或两种类型的前方开颌(AOBTot)。采用描述性和多变量统计方法比较开颌的患病率,并分别研究与口腔变量的关联。

结果

DM1(37%)和 DMD(49%)组之间的开颌患病率存在统计学显著差异(p=0.048)。LOB 在 DM1 中占比<1%,在 DMD 中占比 18%。LOB 与巨舌和闭口姿势有关,AOB 与唇肌无力和张口姿势有关,AOBS 与下颌肌无力有关。口腔功能障碍特征显示出相似的模式,尽管 DM1 和 DMD 的平均 NOT-S 总分分别为 4.2±2.8(中位数 4.0,最小-最大值 1-8)和 2.3±2.0(中位数 2.0,最小-最大值 0-8)。

局限性

两组未进行年龄或性别匹配。

结论

DM1 和 DMD 患者中开颌畸形较为常见,与不同类型的口腔功能障碍有关。本研究强调需要进行多学科评估,以支持制定针对特定患者的治疗策略,改善或维持口腔功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7f73/10578289/1ac8da25f28f/jnd-10-jnd230025-g001.jpg

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