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杜氏肌营养不良症的咳嗽和气道清除

Cough and airway clearance in Duchenne muscular dystrophy.

机构信息

Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138 Bologna, Italy.

Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138 Bologna, Italy.

出版信息

Paediatr Respir Rev. 2019 Aug;31:35-39. doi: 10.1016/j.prrv.2018.11.001. Epub 2018 Nov 24.

Abstract

People with Duchenne muscular dystrophy (DMD), develop a respiratory muscle weakness that results in weakened cough, airway clearance impairment and over time respiratory failure and death. Assessment of cough effectiveness through vital capacity, peak cough flow and maximal inspiratory and expiratory pressures has been used to identify the optimal timing of cough augmentation techniques initiation. The choice of therapies depends on physician knowledge, and patient/care giver abilities. The purpose of this review is to clarify mechanisms of action, benefits and disadvantages of available techniques, such as manual cough-assisting manoeuvres, glossopharyngeal breathing, air stacking by resuscitator bag or by volume-cycle ventilator, and mechanical insufflator-exsufflator. Mechanisms of mucus mobilization, like intrapulmonary percussive ventilation, may have a therapeutic role in the case of persistent atelectasis. It is also crucial to recognize the initial phase of an acute respiratory exacerbation, increase the use of these techniques which may reduce morbidity and mortality.

摘要

杜氏肌营养不良症(DMD)患者会出现呼吸肌无力,导致咳嗽无力、气道清除功能受损,随着时间的推移还会导致呼吸衰竭和死亡。通过肺活量、峰值咳嗽流量和最大吸气和呼气压力评估咳嗽效果,已用于确定开始增强咳嗽技术的最佳时机。治疗方法的选择取决于医生的知识和患者/护理人员的能力。本综述的目的是阐明可用技术的作用机制、益处和缺点,例如手动咳嗽辅助手法、舌咽呼吸、复苏气囊或容量循环呼吸机进行空气堆积以及机械吹入-吹出气。像肺内叩击通气这样的黏液移动机制在持续肺不张的情况下可能具有治疗作用。认识急性呼吸恶化的初始阶段也很重要,增加这些技术的使用可能会降低发病率和死亡率。

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