Gozal D
Constance S. Kaufman Pediatric Pulmonary Research Laboratory, Section of Pediatric Pulmonology, Department of Pediatrics, Tulane University School of Medicine, New Orleans, Louisiana, USA.
Pediatr Pulmonol. 2000 Feb;29(2):141-50. doi: 10.1002/(sici)1099-0496(200002)29:2<141::aid-ppul9>3.0.co;2-y.
Deterioration of respiratory function in patients with neuromuscular disorders is primarily responsible for the high mortality associated with these diseases. A review of Duchenne muscular dystrophy and spinal muscular atrophy, the leading neuromuscular disorders affecting children, will be followed by a critical analysis of the various pathophysiological mechanisms underlying respiratory manifestations in these patients. Among such mechanisms, the role of muscular weakness in preservation of lung function, mucociliary clearance, gas exchange at rest and during exercise, and respiratory control during wakefulness and sleep will be examined in detail. In addition, the potential benefits of respiratory muscle training and of early diagnosis and clinical intervention will be delineated. This review underscores the importance of periodic assessment of pulmonary function during wakefulness and sleep in children affected by neuromuscular diseases as an essential component of multidisciplinary care aimed at improving long-term morbidity, survival, and quality of life.
神经肌肉疾病患者呼吸功能的恶化是导致这些疾病高死亡率的主要原因。本文将首先回顾杜氏肌营养不良症和脊髓性肌萎缩症这两种影响儿童的主要神经肌肉疾病，随后对这些患者呼吸表现背后的各种病理生理机制进行批判性分析。在这些机制中，将详细研究肌肉无力在维持肺功能、黏液纤毛清除、静息和运动时的气体交换以及清醒和睡眠期间的呼吸控制方面所起的作用。此外，还将阐述呼吸肌训练以及早期诊断和临床干预的潜在益处。本综述强调了对受神经肌肉疾病影响的儿童在清醒和睡眠期间进行定期肺功能评估的重要性，这是旨在改善长期发病率、生存率和生活质量的多学科护理的重要组成部分。
