IRCCS Fondazione Don Carlo Gnocchi - ONLUS, Milan, Italy.
Respiratory Unit and Cystic Fibrosis Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy.
Acta Myol. 2024 Jun;43(2):78-82. doi: 10.36185/2532-1900-506.
Neuromuscular diseases (NMDs) comprise a heterogeneous group of conditions characterized by extreme progressive muscle weakness leading to respiratory failure. Noninvasive mechanical ventilation (NIV) has emerged as a cornerstone in the management of respiratory complications associated with NMDs. This review aims to elucidate the role of NMV in respiratory function, improving quality of life, and prolonging survival in individuals with NMD. The physiological basis of respiratory impairment in NMDs, principles of NMV application, evidence supporting its efficacy, patient selection criteria, and potential challenges in its application are discussed.
神经肌肉疾病（NMDs）包括一组表现为极度进行性肌肉无力导致呼吸衰竭的异质性疾病。无创机械通气（NIV）已成为治疗与 NMD 相关的呼吸并发症的基石。本综述旨在阐明 NMV 在呼吸功能、提高生活质量和延长 NMD 患者生存方面的作用。讨论了 NMD 中呼吸功能障碍的生理学基础、NMV 应用的原则、支持其疗效的证据、患者选择标准以及在其应用中潜在的挑战。
