Gur Michal, Manor Eynav, Hanna Moneera, Simaan Nadeen, Gut Guy, Toukan Yazeed, Hakim Fahed, Bar-Yoseph Ronen, Bentur Lea
Pediatric Pulmonary Institute and CF Center, Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Pediatr Pulmonol. 2023 Nov;58(11):3264-3270. doi: 10.1002/ppul.26655. Epub 2023 Aug 30.
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.
有效的呼吸工作和支气管卫生需要吸气和呼气肌肉的协同作用。吸气肌训练（IMT）是慢性阻塞性肺疾病（COPD）肺康复的一部分。有证据表明其在囊性纤维化（CF）中有效，最近在长期新冠病毒感染（long COVID-19）中也有效果。我们不知道有关原发性纤毛运动障碍（PCD）中IMT的研究。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。
一项单中心试点研究。在基线（第1次就诊）、使用®POWERbreathe进行一个月IMT后（第2次就诊）以及随访时（第3次就诊）测量肺活量、肺清除指数（LCI）、最大吸气压力（MIP）和最大呼气压力（MEP）。
该队列包括27名患者（19名PCD患者，8名CF患者）；平均年龄18.4±9.8岁。经过一个月的IMT后，MIP和MIP%显著增加（分别从6.19增加到7.44，p = 0.015；从81.85%增加到100.41%，p = 0.046），在第3次就诊时仍保持。依从性≥90%导致MIP有更高的改善。在亚组分析中，PCD患者的MIP和MIP%改善仍然显著（分别为p = 0.026和p = 0.049）。在肺活量、MEP或LCI方面未发现显著变化。
IMT耐受性良好，并导致PCD患者吸气肌力量改善。MIP改善的临床意义应进一步研究。需要更大规模的长期研究来评估IMT对肺功能、呼吸肌力量、肺部加重和生活质量的长期影响。
