Division of Pulmonology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Division of Pulmonary and Sleep Medicine, Department of Pediatrics, University of Missouri-Kansas City, Kansas City, MO, USA.
Clin Chest Med. 2024 Sep;45(3):729-747. doi: 10.1016/j.ccm.2024.02.021. Epub 2024 Apr 4.
Respiratory sequelae are a frequent cause of morbidity and mortality in children with NMD. Impaired cough strength and resulting airway clearance as well as sleep disordered breathing are the two main categories of respiratory sequelae. Routine clinical evaluation and diagnostic testing by pulmonologists is an important pillar of the multidisciplinary care required for children with NMD. Regular surveillance for respiratory disease and timely implementation of treatment including pulmonary clearance techniques as well as ventilation can prevent respiratory related morbidity including hospital admissions and improve survival. Additionally, novel disease modifying therapies for some NMDs are now available which has significantly improved the clinical trajectories of patients resulting in a paradigm shift in clinical care. Pulmonologists are 'learning' the new natural history for these diseases and adjusting clinical management accordingly.
呼吸后遗症是导致神经肌肉疾病患儿发病率和死亡率居高不下的一个主要原因。咳嗽力量减弱和由此导致的气道清除功能障碍以及睡眠呼吸紊乱是呼吸后遗症的两个主要类型。肺病专家进行的常规临床评估和诊断性检测是神经肌肉疾病患儿多学科治疗所必需的重要支柱。对呼吸疾病进行定期监测，并及时实施治疗，包括肺部清除技术和通气，可预防与呼吸相关的发病率，包括住院治疗，并提高生存率。此外，一些神经肌肉疾病的新型疾病修饰疗法现已面世，这极大地改善了患者的临床轨迹，从而使临床护理发生了范式转变。肺病专家正在“了解”这些疾病的新自然病史，并相应地调整临床管理。
