Hilton Nathan, Solis-Moya Arturo
Liverpool Heart and Chest Hospital NHS Foundation Trust, Thomas Drive, Liverpool, UK, L14 3PE.
Cochrane Database Syst Rev. 2018 May 24;5(5):CD006112. doi: 10.1002/14651858.CD006112.pub4.
Cystic fibrosis is the most common autosomal recessive disease in white populations, and causes respiratory dysfunction in the majority of individuals. Numerous types of respiratory muscle training to improve respiratory function and health-related quality of life in people with cystic fibrosis have been reported in the literature. Hence a systematic review of the literature is needed to establish the effectiveness of respiratory muscle training (either inspiratory or expiratory muscle training) on clinical outcomes in cystic fibrosis. This is an update of a previously published review.
To determine the effectiveness of respiratory muscle training on clinical outcomes in people with cystic fibrosis.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials register comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of most recent search: 17 April 2018.A hand search of the Journal of Cystic Fibrosis and Pediatric Pulmonology was performed, along with an electronic search of online trial databases up until 07 May 2018.
Randomised controlled studies comparing respiratory muscle training with a control group in people with cystic fibrosis.
Review authors independently selected articles for inclusion, evaluated the methodological quality of the studies, and extracted data. Additional information was sought from trial authors where necessary. The quality of the evidence was assessed using the GRADE system MAIN RESULTS: Authors identified 19 studies, of which nine studies with 202 participants met the review's inclusion criteria. There was wide variation in the methodological and written quality of the included studies. Four of the nine included studies were published as abstracts only and lacking concise details, thus limiting the information available. Seven studies were parallel studies and two of a cross-over design. Respiratory muscle training interventions varied dramatically, with frequency, intensity and duration ranging from thrice weekly to twice daily, 20% to 80% of maximal effort, and 10 to 30 minutes, respectively. Participant numbers ranged from 11 to 39 participants in the included studies; five studies were in adults only and four in a combination of children and adults.No significant improvement was reported in the primary outcome of pulmonary function (forced expiratory volume in one second and forced vital capacity) (very low-quality evidence). Although no change was reported in exercise capacity as assessed by the maximum rate of oxygen use, a 10% improvement in exercise duration was found when working at 60% of maximal effort in one study (n = 20) (very low-quality evidence). In a further study (n = 18), when working at 80% of maximal effort, health-related quality of life improved in the mastery and emotion domains (very low-quality evidence). With regards to the review's secondary outcomes, one study (n = 11) found a significant change in intramural pressure, functional residual capacity and maximal inspiratory pressure following training (low-quality evidence). A further study (n = 22) reported that respiratory muscle endurance was significantly longer in the training group (P < 0.01). No studies reported on any other secondary outcomes. Meta-analyses could not be performed due to a lack of consistency and insufficient detail in reported outcome measures.
AUTHORS' CONCLUSIONS: There is insufficient evidence to suggest whether this intervention is beneficial or not. Healthcare practitioners should consider the use of respiratory muscle training on a case-by-case basis. Further research of reputable methodological quality is needed to determine the effectiveness of respiratory muscle training in people with cystic fibrosis. Researchers should consider the following clinical outcomes in future studies; respiratory muscle function, pulmonary function, exercise capacity, hospital admissions, and health-related quality of life. Sensory-perceptual changes, such as respiratory effort sensation (e.g. rating of perceived breathlessness) and peripheral effort sensation (e.g. rating of perceived exertion) may also help to elucidate mechanisms underpinning the effectiveness of respiratory muscle training.
囊性纤维化是白种人群中最常见的常染色体隐性疾病，多数患者会出现呼吸功能障碍。文献报道了多种呼吸肌训练方法，旨在改善囊性纤维化患者的呼吸功能和与健康相关的生活质量。因此，需要对文献进行系统综述，以确定呼吸肌训练（吸气或呼气肌训练）对囊性纤维化患者临床结局的有效性。这是对先前发表综述的更新。
确定呼吸肌训练对囊性纤维化患者临床结局的有效性。
我们检索了Cochrane囊性纤维化和遗传疾病小组试验注册库，该注册库包含通过全面电子数据库检索以及对相关期刊和会议论文摘要集进行手工检索所确定的参考文献。最新检索日期：2018年4月17日。对《囊性纤维化杂志》和《儿科肺病学》进行了手工检索，并对在线试验数据库进行了电子检索，截至2018年5月7日。
比较囊性纤维化患者呼吸肌训练与对照组的随机对照研究。
综述作者独立选择纳入的文章，评估研究的方法学质量，并提取数据。必要时向试验作者寻求更多信息。使用GRADE系统评估证据质量。
作者识别出19项研究，其中9项研究共202名参与者符合综述的纳入标准。纳入研究的方法学和撰写质量差异很大。9项纳入研究中有4项仅以摘要形式发表，缺乏详细信息，因此限制了可用信息。7项研究为平行研究，2项为交叉设计。呼吸肌训练干预差异很大，频率、强度和持续时间分别从每周三次到每天两次、最大努力的20%到80%以及10到30分钟不等。纳入研究中的参与者人数从11人到39人不等；5项研究仅涉及成人，4项研究涉及儿童和成人。在肺功能的主要结局（一秒用力呼气量和用力肺活量）方面未报告有显著改善（极低质量证据）。虽然在通过最大耗氧率评估的运动能力方面未报告有变化，但在一项研究（n = 20）中，当以最大努力的60%进行运动时，运动持续时间提高了10%（极低质量证据）。在另一项研究（n = 18）中，当以最大努力的80%进行运动时，在掌握和情感领域与健康相关的生活质量有所改善（极低质量证据）。关于综述的次要结局，一项研究（n = 11）发现训练后壁内压、功能残气量和最大吸气压力有显著变化（低质量证据）。另一项研究（n = 22）报告训练组的呼吸肌耐力明显更长（P < 0.01）。没有研究报告任何其他次要结局。由于报告的结局测量缺乏一致性和细节不足，无法进行荟萃分析。
没有足够的证据表明这种干预是否有益。医疗从业者应根据具体情况考虑使用呼吸肌训练。需要进行更多方法学质量可靠的研究，以确定呼吸肌训练对囊性纤维化患者的有效性。研究人员在未来研究中应考虑以下临床结局；呼吸肌功能、肺功能、运动能力、住院次数以及与健康相关的生活质量。感觉 - 知觉变化，如呼吸努力感觉（如主观呼吸急促评分）和外周努力感觉（如主观用力评分）也可能有助于阐明呼吸肌训练有效性的潜在机制。
