Rapella Silvia, Piazza Caterina, Morandi Francesco, Carcano Alessandro, Arzilli Cinzia, Nassi Niccolò, Catalano Igor, Formica Francesca, Biffi Emilia
Scientific Institute, IRCCS Eugenio Medea, 23842 Bosisio Parini, LC, Italy.
Italian Association for Congenital Central Hypoventilation Syndrome (AISICC), 50126 Florence, FI, Italy.
Sensors (Basel). 2025 Mar 12;25(6):1759. doi: 10.3390/s25061759.
Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder that impairs autonomic breathing control, leading to alveolar hypoventilation and sometimes to central apnoea, predominantly during sleep. Patients typically require nocturnal ventilatory support and alarms to prevent life-threatening desaturation events. However, current alarm systems integrated into pulse oximeters do not provide adequate assistance at home. To address these limitations, we developed an assistive device with customizable multisensory stimulation that activates based on the severity and duration of desaturation episodes. In a multicenter clinical trial involving 4 children and 11 young adults with CCHS, we assessed the device's effectiveness and the role of arousals over three nights: one baseline and two test nights. The results showed that the device significantly improved awakening rates and enabled faster recovery from desaturations in young adults. However, no such improvements were observed in children compared to the baseline. Arousal events and sleep efficiency were unaffected by the device in both groups. These findings suggest that the device can enhance the safety and autonomy of young adults with CCHS but may be more effective in alerting caregivers in pediatric cases than directly waking children. Further studies are needed to refine its application across different age groups, given the limited sample size.
先天性中枢性低通气综合征（CCHS）是一种罕见的疾病，会损害自主呼吸控制，导致肺泡通气不足，有时还会导致中枢性呼吸暂停，主要发生在睡眠期间。患者通常需要夜间通气支持和警报装置，以防止危及生命的血氧饱和度下降事件。然而，目前集成在脉搏血氧仪中的警报系统在家庭环境中并不能提供足够的帮助。为了解决这些局限性，我们开发了一种具有可定制多感官刺激功能的辅助设备，该设备可根据血氧饱和度下降发作的严重程度和持续时间启动。在一项涉及4名儿童和11名患有CCHS的年轻人的多中心临床试验中，我们在三个晚上评估了该设备的有效性以及觉醒的作用：一个基线夜晚和两个测试夜晚。结果表明，该设备显著提高了年轻人的觉醒率，并使他们从血氧饱和度下降中恢复得更快。然而，与基线相比，儿童组未观察到此类改善。两组的觉醒事件和睡眠效率均未受该设备影响。这些发现表明，该设备可以提高患有CCHS的年轻人的安全性和自主性，但在提醒儿科病例中的护理人员方面可能比直接唤醒儿童更有效。鉴于样本量有限，需要进一步研究以完善其在不同年龄组中的应用。
