LoMauro Antonella, D'Angelo Maria Grazia, Aliverti Andrea
Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, 20133, Milan, Italy.
Istituto di Ricovero e Cura a Carattere Scientifico "E. Medea", Bosisio Parini, Italy.
Curr Neurol Neurosci Rep. 2017 May;17(5):44. doi: 10.1007/s11910-017-0750-1.
This review aims to explain the inevitable imbalance between respiratory load, drive, and muscular force that occurs in the natural aging of Duchenne muscular dystrophy and that predisposes these patients to sleep disordered breathing (SDB). In DMD, SDB is characterized by oxygen desaturation, apneas, hypercapnia, and hypoventilation during sleep and ultimately develops into respiratory failure during wakefulness. It can be present in all age groups. Young patients risk obstructive apneas because of weight gain, secondary to progressive physical inactivity and prolonged corticosteroid therapy; older patients hypoventilate and desaturate because of respiratory muscle weakness, in particular the diaphragm. These conditions are further exacerbated during REM sleep, the phase of maximal muscle hypotonia during which the diaphragm has to provide most of the ventilation. Evidence is given to the daytime predictors of early symptoms of SDB, important indicators for the proper time to initiate mechanical ventilation.
本综述旨在解释杜氏肌营养不良症自然衰老过程中呼吸负荷、驱动力和肌肉力量之间不可避免的失衡，这种失衡使这些患者易患睡眠呼吸障碍（SDB）。在杜氏肌营养不良症中，睡眠呼吸障碍的特征是睡眠期间出现氧饱和度下降、呼吸暂停、高碳酸血症和通气不足，最终在清醒时发展为呼吸衰竭。它可出现在所有年龄组。年轻患者由于体重增加而有发生阻塞性呼吸暂停的风险，这继发于进行性身体活动减少和长期使用皮质类固醇治疗；老年患者则由于呼吸肌无力，尤其是膈肌无力，出现通气不足和氧饱和度下降。这些情况在快速眼动睡眠期间会进一步恶化，快速眼动睡眠是肌肉张力最低的阶段，在此期间膈肌必须提供大部分通气。文中还给出了睡眠呼吸障碍早期症状的日间预测指标，这些指标是启动机械通气的合适时机的重要指标。
