伪阻塞性睡眠呼吸障碍 - - 脊柱肌肉缩的定义和进展
Federica Trucco1, Sakina Dastagir2, Hui-Leng Tan2
1Department Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK; Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London, UK.
伪阻塞性睡眠呼吸障碍 (SDB) 在脊髓肌缩 (SMA) 2型中很常见,并且随着疾病的进展而恶化. 非侵入性通风 (NIV) 可以帮助在SMA患者中管理这些伪阻塞事件.
科学领域:
- 神经学 神经学
- 肺部病理学 肺部病理学
- 儿科 儿科 儿科
背景情况:
- 阻塞性睡眠呼吸障碍 (SDB) 在脊髓肌肉缩 (SMA) 患者中很常见.
- 像nusinersen这样的疾病修饰治疗 (DMT) 可能会影响SDB.
- 假设:SMA中的一些阻塞性事件可能是由于胸壁软弱而造成的伪阻塞性,而不是真正的呼吸道阻塞.
研究的目的:
- 报告2型儿科SMA患者的伪阻塞性SDB模式.
- 为了定义伪阻碍性SDB特征.
- 为了评估伪阻塞性SDB随着疾病进展和治疗的变化.
主要方法:
- 从6名2型小儿性SMA患者的18个呼吸道多重检查 (PG) 的回顾性审查.
- 在研究开始时,患者从未接受过DMT治疗.
- 在三个时间点收集的数据:初始研究,一年的随访和最新的研究.
主要成果:
- 伪阻塞性SDB特征包括矛盾的呼吸和缺乏补偿呼吸.
- 伪阻塞事件的发生率随着时间的推移在自呼吸的患者中增加.
- 开始或调整非侵入性通风 (NIV) 导致伪阻塞事件的减少.
结论:
- 伪阻塞性SDB在SMA2型中是普遍且渐进的.
- 非侵入性通风 (NIV) 是这种人群中伪阻塞性SDB的有效治疗方法.
- 需要在较大的SMA队列中进行进一步的前性研究.
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