Pediatric Research Laboratory, University of Pittsburgh, Department of Occupational Therapy, Pennsylvania, PA, United States.
University of Pittsburgh, Department of Occupational Therapy, Pennsylvania, PA, United States.
Eur J Paediatr Neurol. 2020 May;26:20-28. doi: 10.1016/j.ejpn.2020.02.012. Epub 2020 Feb 28.
Sleep health in rare disease is often overlooked due to the complex nature of the disease. For males with Duchenne muscular dystrophy, sleep assessment is typically focused on pulmonary function and identification of sleep disordered breathing. Unfortunately for young boys with Duchenne muscular dystrophy, sleep assessment is often neglected, resulting in a dearth of knowledge on sleep health in this population. This study describes sleep quantity and quality in both younger (4-9 years) and older (10-17 years) males with Duchenne muscular dystrophy (n = 19) and compares these characteristics with sleep characteristics of unaffected peers (n = 17).
This study was a longitudinal, observational study. Sleep measures were collected using the parent-proxy Children's Sleep Habits Questionnaire-Abbreviated version and objective sleep measures from actigraphy (sleep efficiency, awakenings, and awakening duration) over 30 days for all participants. Means and standard deviations were examined, and effect sizes were computed to quantify the magnitude of difference between the Duchenne muscular dystrophy and unaffected groups.
Overall, boys with Duchenne muscular dystrophy were found to experience worse sleep than their unaffected peers as measured by parent report and actigraphy. Effect sizes of both measures demonstrated moderate to large magnitudes of difference in many of the sleep variables. Parents of boys with Duchenne muscular dystrophy reported higher scores (indicating worse sleep) in all subsections and total score of the Children's Sleep Habits Questionnaire - Abbreviated version. Actigraphy data indicated that the Duchenne muscular dystrophy group had lower percent sleep efficiency, more night awakenings and longer duration of night awakenings than their unaffected peers.
Our findings offer a novel look into sleep in young boys with Duchenne muscular dystrophy. Both parent-report and actigraphy data indicate poor sleep health in this population compared with age-matched unaffected peers. Actigraphy was found to align with parent-report of sleep in this population, supporting the use of these two different ways to measure sleep in Duchenne muscular dystrophy. Results from this study should encourage clinicians and researchers alike to further explore sleep and its impact on disease in young boys with Duchenne muscular dystrophy.
由于疾病的复杂性，罕见病患者的睡眠健康往往被忽视。对于患有杜氏肌营养不良症的男性，睡眠评估通常侧重于肺功能和睡眠呼吸障碍的识别。不幸的是，患有杜氏肌营养不良症的小男孩的睡眠评估经常被忽视，导致人们对该人群的睡眠健康知之甚少。本研究描述了年龄较小（4-9 岁）和较大（10-17 岁）的患有杜氏肌营养不良症的男性的睡眠量和质量，并将这些特征与未受影响的同龄人的睡眠特征进行了比较（n=17）。
本研究为纵向观察性研究。使用父母代理的儿童睡眠习惯问卷简化版（Childrens Sleep Habits Questionnaire-Abbreviated version）收集睡眠指标，并使用活动记录仪收集 30 天内的所有参与者的客观睡眠指标（睡眠效率、觉醒和觉醒持续时间）。检查平均值和标准差，并计算效应量，以量化杜氏肌营养不良症组和未受影响组之间差异的大小。
总体而言，与未受影响的同龄人相比，通过父母报告和活动记录仪测量，患有杜氏肌营养不良症的男孩睡眠质量较差。两种测量方法的效应量都表明，在许多睡眠变量中存在中度到较大的差异。患有杜氏肌营养不良症男孩的父母在儿童睡眠习惯问卷简化版的所有小节和总分中报告的分数更高（表明睡眠质量更差）。活动记录仪数据表明，杜氏肌营养不良症组的睡眠效率百分比较低，夜间觉醒次数较多，夜间觉醒持续时间较长。
我们的研究结果提供了一个新颖的视角来了解患有杜氏肌营养不良症的年轻男孩的睡眠情况。与年龄匹配的未受影响的同龄人相比，父母报告和活动记录仪数据都表明该人群的睡眠健康状况不佳。在该人群中，活动记录仪与父母对睡眠的报告一致，支持使用这两种不同的方法来测量杜氏肌营养不良症的睡眠。本研究的结果应鼓励临床医生和研究人员进一步探索睡眠及其对患有杜氏肌营养不良症的年轻男孩的疾病的影响。
