Abstract：
Respiratory failure is a significant concern in neuromuscular diseases (NMDs). This CHEST guideline examines the literature on the respiratory management of patients with NMD to provide evidence-based recommendations.
An expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations. A modified Delphi technique was used to reach a consensus on the recommendations.
Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement.
Evidence of best practices for respiratory management in NMD is limited and is based primarily on observational data in amyotrophic lateral sclerosis. The panel found that pulmonary function testing every 6 months may be beneficial and may be used to initiate noninvasive ventilation (NIV) when clinically indicated. An individualized approach to NIV settings may benefit patients with chronic respiratory failure and sleep-disordered breathing related to NMD. When resources allow, polysomnography or overnight oximetry can help to guide the initiation of NIV. The panel provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. The guideline panel emphasizes that NMD pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function. The clinician\'s role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making.
An expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations. A modified Delphi technique was used to reach a consensus on the recommendations.
Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement.
Evidence of best practices for respiratory management in NMD is limited and is based primarily on observational data in amyotrophic lateral sclerosis. The panel found that pulmonary function testing every 6 months may be beneficial and may be used to initiate noninvasive ventilation (NIV) when clinically indicated. An individualized approach to NIV settings may benefit patients with chronic respiratory failure and sleep-disordered breathing related to NMD. When resources allow, polysomnography or overnight oximetry can help to guide the initiation of NIV. The panel provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. The guideline panel emphasizes that NMD pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function. The clinician\'s role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making.
摘要：
背景：呼吸衰竭是神经肌肉疾病（NMD）的重要关注点。本CHEST指南审查了NMD患者呼吸管理的文献，以提供循证建议。
方法：专家小组针对NMD的呼吸管理进行了系统评价，并应用GRADE方法评估证据的确定性并制定和分级建议。使用改进的Delphi技术就建议达成共识。
结果：基于128项研究，小组提出了15项分级建议，良好的实践声明，一个基于共识的声明。
结论：NMD呼吸管理最佳实践证据有限，主要基于肌萎缩侧索硬化症的观察数据。小组发现，每六个月进行一次肺功能检查可能是有益的，并在有临床指征时用于启动NIV。对NIV设置的个性化方法可能会使患有与NMD相关的慢性呼吸衰竭和睡眠呼吸障碍的患者受益。当资源允许时，多导睡眠图或夜间血氧测定可以帮助指导NIV的开始。小组提供了烟嘴通风的指南，过渡到家庭机械通风，唾液分泌管理，和气道清除疗法。指南小组强调，NMD病变代表了一组不同的疾病，具有不同的肺功能下降率。临床医生的作用是在床边增加评估，与患者和家属共同决策，包括尊重患者的偏好和治疗目标，考虑生活质量，以及在决策中适当使用可用资源。
方法：专家小组针对NMD的呼吸管理进行了系统评价，并应用GRADE方法评估证据的确定性并制定和分级建议。使用改进的Delphi技术就建议达成共识。
结果：基于128项研究，小组提出了15项分级建议，良好的实践声明，一个基于共识的声明。
结论：NMD呼吸管理最佳实践证据有限，主要基于肌萎缩侧索硬化症的观察数据。小组发现，每六个月进行一次肺功能检查可能是有益的，并在有临床指征时用于启动NIV。对NIV设置的个性化方法可能会使患有与NMD相关的慢性呼吸衰竭和睡眠呼吸障碍的患者受益。当资源允许时，多导睡眠图或夜间血氧测定可以帮助指导NIV的开始。小组提供了烟嘴通风的指南，过渡到家庭机械通风，唾液分泌管理，和气道清除疗法。指南小组强调，NMD病变代表了一组不同的疾病，具有不同的肺功能下降率。临床医生的作用是在床边增加评估，与患者和家属共同决策，包括尊重患者的偏好和治疗目标，考虑生活质量，以及在决策中适当使用可用资源。
