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首页> 外文期刊>Orphanet journal of rare diseases >Normal sleep on mechanical ventilation in adult patients with congenital central alveolar hypoventilation (Ondine’s curse syndrome)
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Normal sleep on mechanical ventilation in adult patients with congenital central alveolar hypoventilation (Ondine’s curse syndrome)

机译：成人先天性中央肺泡通气不足（Ondine的诅咒综合征）患者，机械通气可正常睡眠

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BackgroundThe purpose of this study was to describe the sleep structure (especially slow wave sleep) in adults with congenital central hypoventilation syndrome (CCHS), a rare genetic disease due to mutations in the PHOX2B gene. Fourteen patients aged 23 (19.0; 24.8) years old (median [1rst-3rd quartiles]) with CCHS underwent a sleep interview and night-time attended polysomnography with their ventilatory support. Their sleep variables were compared to those collected in 15 healthy control subjects matched for age, sex and body mass index. ResultsThe latency to N3 sleep was shorter in patients (26.3?min [24.0; 30.1]) than in controls (49.5?min [34.3; 66.9]; P =?0.005), and sleep onset latency tended to be shorter in patients (14.0?min [7.0; 20.5]) than in controls (33.0?min [18.0; 49.0]; P =?0.052). Total sleep time, sleep stage percentages, sleep fragmentation as well as respiratory and movement index were within normal ranges and not different between groups. ConclusionsNormal sleep in adult patients with CCHS and adequate ventilator support indicates that the PHOX2 gene mutations do not affect brain sleep networks. Consequently, any complaint of disrupted sleep should prompt clinicians to look for the usual causes of sleep disorders, primarily inadequate mechanical ventilation. Shorter N3 latency may indicate a higher need for slow wave sleep, to compensate for the abnormal respiratory-related cortical activity during awake quiet breathing observed in patients with CCH.
机译：背景本研究的目的是描述患有先天性中枢通气不足综合征（CCHS）的成年人的睡眠结构（特别是慢波睡眠），这是一种由于PHOX2B基因突变而引起的罕见遗传疾病。年龄为23岁（19.0; 24.8）岁（中位[1 -3rd四分位数]）的14例CCHS患者进行了睡眠访谈，并在通气支持下夜间进行了多导睡眠监测。将他们的睡眠变量与在年龄，性别和体重指数匹配的15名健康对照受试者中收集的睡眠变量进行比较。结果患者的N3睡眠潜伏期（26.3？min [24.0; 30.1]）比对照组（49.5？min [34.3; 66.9]; P = 0.005）短，并且患者的睡眠发作潜伏期较短（14.0）。 Δmin[7.0; 20.5]）比对照（33.0Δmin[18.0; 49.0]; P ＝Δ0.052）。总睡眠时间，睡眠阶段百分比，睡眠碎片以及呼吸和运动指数均在正常范围内，各组之间无差异。结论CCHS并有足够的呼吸机支持的成人患者正常睡眠表明，PHOX2基因突变不会影响脑部睡眠网络。因此，任何关于睡眠中断的抱怨都应促使临床医生寻找导致睡眠障碍的常见原因，主要是机械通气不足。 N3潜伏期越短，可能意味着对慢波睡眠的需求就越高，以补偿在CCH患者清醒的安静呼吸过程中异常呼吸相关的皮层活动。

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