接受维持性康复治疗的遗传性运动神经和感觉神经病以及肌营养不良症患者在呼吸功能、抑郁症状和生活质量方面的差异。

IF 1.5 4区 医学 Q3 REHABILITATION International Journal of Rehabilitation Research Pub Date : 2024-03-01 Epub Date: 2023-12-14 DOI:10.1097/MRR.0000000000000608
Nika Lajlar, Gaj Vidmar, Metka Moharić
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引用次数: 0

摘要

遗传性运动与感觉神经病(HMSN)和肌营养不良症(MD)是一种慢性神经肌肉疾病,会导致进行性肌肉损伤并影响患者的生活质量。现有文献中的研究结果相互矛盾,这突出表明有必要对这些患者的具体健康状况进行重点研究。本研究旨在探讨接受维持性康复治疗的 HMSN 和 MD 患者在呼吸功能、抑郁症状和生活质量方面的差异。我们的前瞻性观察研究包括 62 名接受维持性康复治疗的 HMSN 患者(中位年龄 53.5 岁,年龄范围 19-79 岁;38 名女性)和 50 名接受维持性康复治疗的 MD 患者(中位年龄 54.0 岁,年龄范围 18-77 岁;34 名女性)。他们进行了呼吸功能测试(生命容量、强迫生命容量、强迫呼气量和呼气流量峰值)和呼吸肌力测试(最大吸气压力、最大呼气压力和嗅鼻吸力)。流行病学研究中心抑郁量表用于评估抑郁情况,遗传性神经肌肉疾病生活质量问卷用于评估与健康相关的生活质量。在考虑年龄、症状持续时间和婚姻状况的情况下,我们使用逻辑回归法研究了存在抑郁症状的组间差异。在对年龄、性别和症状持续时间进行调整后,我们使用多元线性回归评估了生活质量得分的差异。在所有呼吸测量指标上,HMSN 组的结果在统计学上明显优于 MD 组(呼吸功能测量指标的绝对差异约为 17%,呼吸肌强度测量指标的相对差异平均为 30%)。两组均表现出较高程度的抑郁症状(HMSN 24%,MD 44%;估计调整后几率 MD vs. HMSN 1.9,95% CI 0.8-4.5,P = 0.127)。我们没有发现两组患者在生活质量方面存在统计学意义上的显著差异,但可以观察到 HMSN 患者的生活质量有提高的趋势。对未来实践的启示是,MD 患者可能会从有针对性的呼吸康复干预中获益最多,而两组患者都可能从有针对性的心理健康干预中获益。
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Differences in respiratory function, depressive symptoms and quality of life between patients with hereditary motor and sensory neuropathy and myotonic dystrophy undergoing maintenance rehabilitation.

Hereditary motor and sensory neuropathy (HMSN) and myotonic dystrophy (MD) are chronic neuromuscular diseases that cause progressive muscular impairment and impact patient's quality of life. Conflicting findings in existing literature underscore the need for focussed research on specific health aspects in these patients. The aim of the study was to explore the differences in respiratory function, depressive symptoms and quality of life between patients with HMSN and MD undergoing maintenance rehabilitation. Our prospective observational study included 62 HMSN patients (median age 53.5, range 19-79 years; 38 women) and 50 MD patients (median age 54.0, range 18-77 years; 34 women) undergoing maintenance rehabilitation. They performed respiratory function tests (vital capacity, forced vital capacity, forced expiratory volume and peak expiratory flow) and respiratory muscle strength tests (maximum inspiratory pressure, maximum expiratory pressure and sniff nasal inspiratory force). The Center for Epidemiological Studies-Depression Scale was used to evaluate depression, and their health-related quality of life was assessed using the Quality of Life in Genetic Neuromuscular Disease Questionnaire. Using logistic regression, we examined group-difference in presence of depressive symptoms while accounting for age, symptoms duration, and marital status. Multiple linear regression was used to assess the difference in quality-of-life scores, adjusting for age, gender, and symptoms' duration. The HMSN group achieved statistically significantly better results than the MD group on all respiratory measures (about 17% absolute difference in the respiratory function measures and 30% relative difference in the respiratory muscle strength measures on average). Both groups exhibited a high level of depression symptoms (HMSN 24%, MD 44%; estimated adjusted odds-ratio MD vs. HMSN 1.9, 95% CI 0.8-4.5, P  = 0.127). We did not find a statistically significant difference between the groups regarding quality-of-life domains, though a trend towards better quality-of-life among the HMSN patients could be observed. The implication for future practice is that the MD patients would potentially benefit the most from targeted respiratory-rehabilitation interventions, and both groups could benefit from focussed mental-health interventions.

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来源期刊
CiteScore
3.10
自引率
0.00%
发文量
88
审稿时长
6-12 weeks
期刊介绍: International Journal of Rehabilitation Research is a quarterly, peer-reviewed, interdisciplinary forum for the publication of research into functioning, disability and contextual factors experienced by persons of all ages in both developed and developing societies. The wealth of information offered makes the journal a valuable resource for researchers, practitioners, and administrators in such fields as rehabilitation medicine, outcome measurement nursing, social and vocational rehabilitation/case management, return to work, special education, social policy, social work and social welfare, sociology, psychology, psychiatry assistive technology and environmental factors/disability. Areas of interest include functioning and disablement throughout the life cycle; rehabilitation programmes for persons with physical, sensory, mental and developmental disabilities; measurement of functioning and disability; special education and vocational rehabilitation; equipment access and transportation; information technology; independent living; consumer, legal, economic and sociopolitical aspects of functioning, disability and contextual factors.
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