Sleep and Breathing Disturbances in Children With Leigh Syndrome: A Comparative Study.
Sleep and Breathing Disturbances in Children With Leigh Syndrome: A Comparative Study.
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DOI：
10.1016/j.pediatrneurol.2022.08.006
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发表时间：
2022-11
影响因子：
3.8
通讯作者：
Ramirez, Jan-Marino
中科院分区：
文献类型：
--
作者：
Wang, Jia-Der Ju;Chen, Maida;Zhang, Cristian;Parker, Jessica;Saneto, Russell;Ramirez, Jan-Marino
Leigh综合征（LS）是一种进行性神经退行性线粒体疾病，其特征是影响中枢神经系统不同部位的坏死性病变，特别是脑干和基底神经节。该区域的病变可能涉及呼吸和睡眠中枢，导致 LS 中出现临床显着的紊乱，但特征很少。本研究的目的是描述和比较年龄-性别和呼吸暂停低通气指数 (AHI) 匹配的 LS 儿童与阻塞性睡眠呼吸暂停 (OSA) 患者的多导睡眠图指标量化的呼吸紊乱的生理反应，这是一种具有类似睡眠呼吸障碍负担的常见临床人群。对 6 名 LS 患者的多导睡眠图数据进行回顾性比较研究，并将其与 18 名年龄、性别、AHI 匹配的 OSA 患者进行比较，特别关注心肺和睡眠结构指标。 LS 组和 OSA 组的睡眠结构和阶段持续时间保持不变，但第一组在入睡后觉醒时间增加。 LS组同时表现出阻塞性和中枢性睡眠呼吸暂停。与 OSA 组相比，该组的心率、氧饱和度指数≥3% 显着升高，睡眠效率、呼吸唤醒指数和总睡眠时间显着降低。与神经型 OSA 儿童相比，LS 患者表现出明显更多的与睡眠相关的心肺功能障碍和睡眠碎片化。鉴于这些发现可能不利于健康和发育，应鼓励对具有类似状况的患者进行睡眠评估，以进行早期管理。
Leigh syndrome (LS) is a progressive neurodegenerative mitochondrial disease characterized by necrotizing lesions affecting different parts of the central nervous system, especially in the brainstem and basal ganglia. Lesions in this area may involve respiratory and sleep centers, resulting in the clinically significant disturbances seen–but poorly characterized–in LS. The purpose of the present study is to characterize and compare the physiologic responses to respiratory disturbances quantified by polysomnography metrics of children with LS with age-sex- and apnea-hypopnea index (AHI)-matched patients with obstructive sleep apnea (OSA), a common clinical population with similar burden of sleep-disordered breathing. Retrospective comparative study of polysomnographic data from six patients with LS were reviewed and compared with 18 age-sex-AHI-matched patients with OSA, with particular attention to cardiorespiratory and sleep architecture metrics. Sleep architecture and stage duration were conserved in LS and OSA groups, but increased wake after sleep onset was seen among the first group. The LS group exhibited both obstructive and central sleep apnea. The group also had significantly greater values of heart rate, ≥3% oxygen desaturation index, and lower values of sleep efficiency, respiratory arousal index, and total sleep time when compared with the OSA group. Patients with LS exhibited significantly more sleep-related cardiorespiratory disturbances and sleep fragmentation when compared with neurotypical children with OSA. Given that these findings are plausibly detrimental to health and development, sleep evaluation in patients with similar conditions should be encouraged for early management.
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影响因子：
8
作者：
Montgomery-Downs, HE;O'Brien, LM;Gozal, D
通讯作者：
Gozal, D
影响因子：
3.5
作者：
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影响因子：
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通讯作者：
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影响因子：
4
作者：
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通讯作者：
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影响因子：
11.2
作者：
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通讯作者：
Zuccoli, Giulio