Abstract：
Effective work of breathing and bronchial hygiene requires synergy of inspiratory and expiratory muscles. Inspiratory muscle training (IMT) is a part of pulmonary rehabilitation in chronic obstructive pulmonary disease (COPD). There is some evidence of its efficacy in cystic fibrosis (CF) and, recently, in long COVID-19. We are not aware of studies on IMT in primary ciliary dyskinesia (PCD). Our aim was to assess the effect of IMT on respiratory muscle strength and pulmonary function in PCD and CF patients.
A single center pilot study. Spirometry, lung clearance index (LCI), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured at baseline (visit 1), after a month of IMT with ®POWERbreathe (visit 2), and at follow-up (visit 3).
The cohort included 27 patients (19 PCD, 8 CF); mean age 18.4 ± 9.8 years. After a month of IMT, there was a significant increase in MIP and MIP% (6.19-7.44, p = .015; and 81.85%-100.41%, p = .046, respectively), which was sustained at visit 3. Compliance ≥90% led to higher improvement in MIP. In sub-group analysis, improvement in MIP and MIP% remained significant for PCD patients (p = .026 and p = .049, respectively). No significant changes were found in spirometry, MEP or LCI.
IMT was well-tolerated and led to improved inspiratory muscle strength in PCD patients. The clinical implication of improved MIP should be further investigated. Larger, long-term studies are needed to evaluate long-term effects of IMT on pulmonary function, respiratory muscle strength, pulmonary exacerbations, and quality of life.
A single center pilot study. Spirometry, lung clearance index (LCI), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured at baseline (visit 1), after a month of IMT with ®POWERbreathe (visit 2), and at follow-up (visit 3).
The cohort included 27 patients (19 PCD, 8 CF); mean age 18.4 ± 9.8 years. After a month of IMT, there was a significant increase in MIP and MIP% (6.19-7.44, p = .015; and 81.85%-100.41%, p = .046, respectively), which was sustained at visit 3. Compliance ≥90% led to higher improvement in MIP. In sub-group analysis, improvement in MIP and MIP% remained significant for PCD patients (p = .026 and p = .049, respectively). No significant changes were found in spirometry, MEP or LCI.
IMT was well-tolerated and led to improved inspiratory muscle strength in PCD patients. The clinical implication of improved MIP should be further investigated. Larger, long-term studies are needed to evaluate long-term effects of IMT on pulmonary function, respiratory muscle strength, pulmonary exacerbations, and quality of life.
摘要：
背景：有效的呼吸和支气管卫生工作需要吸气和呼气肌的协同作用。吸气肌训练（IMT）是慢性阻塞性肺疾病（COPD）肺康复的一部分。有一些证据表明其在囊性纤维化（CF）和，最近,在长长的COVID-19中。我们不知道原发性纤毛运动障碍（PCD）的IMT研究。我们的目的是评估IMT对PCD和CF患者呼吸肌力量和肺功能的影响。
方法：单中心试点研究。肺活量测定,肺清除指数（LCI），最大吸气压力(MIP),和最大呼气压（MEP）测量在基线（第1次访问），在使用®POWER呼吸进行IMT一个月后(第2次访问)，和随访（第3次访问）。
结果：该队列包括27例患者（19例PCD，8CF）；平均年龄18.4±9.8岁。经过一个月的IMT，MIP和MIP%显著增加(6.19-7.44，p=0.015；81.85%-100.41%，分别为p=.046)，在访问3时持续。合规性≥90%导致MIP有更高的改善。在分组分析中，PCD患者的MIP和MIP%改善仍然显著(分别为p=.026和p=.049).肺活量没有发现明显变化，MEP或LCI。
结论：在PCD患者中，IMT具有良好的耐受性，并导致吸气肌强度改善。改善MIP的临床意义有待进一步研究。较大,需要长期研究来评估IMT对肺功能的长期影响，呼吸肌力量,肺加重,和生活质量。
方法：单中心试点研究。肺活量测定,肺清除指数（LCI），最大吸气压力(MIP),和最大呼气压（MEP）测量在基线（第1次访问），在使用®POWER呼吸进行IMT一个月后(第2次访问)，和随访（第3次访问）。
结果：该队列包括27例患者（19例PCD，8CF）；平均年龄18.4±9.8岁。经过一个月的IMT，MIP和MIP%显著增加(6.19-7.44，p=0.015；81.85%-100.41%，分别为p=.046)，在访问3时持续。合规性≥90%导致MIP有更高的改善。在分组分析中，PCD患者的MIP和MIP%改善仍然显著(分别为p=.026和p=.049).肺活量没有发现明显变化，MEP或LCI。
结论：在PCD患者中，IMT具有良好的耐受性，并导致吸气肌强度改善。改善MIP的临床意义有待进一步研究。较大,需要长期研究来评估IMT对肺功能的长期影响，呼吸肌力量,肺加重,和生活质量。
