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Airway clearance in patients with neuromuscular disease
Paediatric Respiratory Reviews ( IF 5.8 ) Pub Date : 2023-02-23 , DOI: 10.1016/j.prrv.2023.02.002
Alexander Ilan Gipsman 1 , Nicole Christine Lapinel 2 , Oscar Henry Mayer 1
Affiliation  

Airway clearance is a critical component of both maintenance of respiratory health and management of acute respiratory illnesses. The process of effective airway clearance begins with the recognition of secretions in the airway and culminates in expectoration or swallowing. There are multiple points on this continuum at which neuromuscular disease causes impaired airway clearance. This can result in an otherwise mild upper respiratory illness progressing unabated from an easily managed condition to a severe, life-threatening lower respiratory illness requiring intensive therapy for patient recovery. Even during periods of relative health, airway protective mechanisms can be compromised, and patients may have difficulty managing average quantities of secretions. This review summarizes airway clearance physiology and pathophysiology, mechanical and pharmacologic treatment modalities, and provides a practical approach for managing secretions in patients with neuromuscular disease. Neuromuscular disease is an umbrella term used to describe disorders that involve dysfunction of peripheral nerves, the neuromuscular junction, or skeletal muscle. Although this paper specifically reviews airway clearance pertaining to those with neuromuscular diseases (e.g., muscular dystrophy, spinal muscular atrophy, myasthenia gravis), most of its content is relevant to the management of patients with central nervous system disorders such as chronic static encephalopathy caused by trauma, metabolic or genetic abnormalities, congenital infection, or neonatal hypoxic-ischemic injury.



中文翻译:

神经肌肉疾病患者的气道清除率

气道清除是维持呼吸道健康和管理急性呼吸道疾病的重要组成部分。有效的气道清除过程始于识别气道中的分泌物,最终于咳出或吞咽。在这个连续体上有多个点神经肌肉疾病会导致气道清除功能受损。这可能导致原本轻微的上呼吸道疾病从易于控制的病情有增无减地发展为严重的、危及生命的下呼吸道疾病,需要强化治疗才能使患者康复。即使在相对健康的时期,气道保护机制也可能受到损害,患者可能难以控制平均分泌物量。本综述总结了气道清除生理学和病理生理学、机械和药物治疗方式,并为神经肌肉疾病患者的分泌物管理提供了实用方法。神经肌肉疾病是一个总称术语,用于描述涉及周围神经、神经肌肉接头或骨骼肌功能障碍的疾病。虽然本文专门回顾了与神经肌肉疾病(例如,肌营养不良症、脊髓性肌萎缩症、重症肌无力)相关的气道清除,但其大部分内容与中枢神经系统疾病患者的治疗相关,例如由神经肌肉疾病引起的慢性静态性脑病。外伤、代谢或遗传异常、先天性感染或新生儿缺氧缺血性损伤。

更新日期:2023-02-23
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