Cystic fibrosis is a chronic genetic disease that can affect the function of the respiratory system. Previous reviews of the effects of respiratory muscle training in people with cystic fibrosis are uncertain and do not consider the effect of age on disease progression. This systematic review aims to determine the effectiveness of respiratory muscle training in the clinical outcomes of children and adolescents with cystic fibrosis. Up to July 2023, electronic databases and clinical trial registries were searched. Controlled clinical trials comparing respiratory muscle training with sham intervention or no intervention in children and adolescents with cystic fibrosis. The primary outcomes were respiratory muscle strength, respiratory muscle endurance, lung function, and cough. Secondary outcomes included exercise capacity, quality of life and adverse events. Two review authors independently extracted data and assessed study quality using the Cochrane Risk of Bias Tool 2. The certainty of the evidence was assessed according to the GRADE approach. Meta-analyses where possible; otherwise, take a qualitative approach. Six studies with a total of 151 participants met the inclusion criteria for this review. Two of the six included studies were published in abstract form only, limiting the available information. Four studies were parallel studies and two were cross-over designs. There were significant differences in the methods and quality of the methodology included in the studies. The pooled data showed no difference in respiratory muscle strength, lung function, and exercise capacity between the treatment and control groups. However, subgroup analyses suggest that inspiratory muscle training is beneficial in increasing maximal inspiratory pressure, and qualitative analyses suggest that respiratory muscle training may benefit respiratory muscle endurance without any adverse effects. This systematic review and meta-analysis indicate that although the level of evidence indicating the benefits of respiratory muscle training is low, its clinical significance suggests that we further study the methodological quality to determine the effectiveness of training. The protocol for this review was recorded in the International Prospective Register of Systematic Reviews (PROSPERO) under registration number CRD42023441829.

中文翻译：

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呼吸肌训练对囊性纤维化儿童和青少年的影响：系统评价和荟萃分析

囊性纤维化是一种慢性遗传性疾病，可影响呼吸系统的功能。先前关于呼吸肌训练对囊性纤维化患者的影响的评论尚不确定，并且没有考虑年龄对疾病进展的影响。本系统评价旨在确定呼吸肌训练对囊性纤维化儿童和青少年临床结果的有效性。截至2023年7月，检索了电子数据库和临床试验注册中心。对患有囊性纤维化的儿童和青少年进行比较呼吸肌训练与假干预或不干预的对照临床试验。主要结局是呼吸肌力量、呼吸肌耐力、肺功能和咳嗽。次要结果包括运动能力、生活质量和不良事件。两位综述作者独立提取数据并使用 Cochrane 偏倚风险工具 2 评估研究质量。根据 GRADE 方法评估证据的确定性。尽可能进行荟萃分析；否则，采取定性方法。共有 151 名参与者的六项研究符合本次审查的纳入标准。纳入的六项研究中有两项仅以摘要形式发表，限制了可用信息。四项研究是平行研究，两项是交叉设计。研究中所采用的方法和方法的质量存在显着差异。汇总数据显示治疗组和对照组之间的呼吸肌力量、肺功能和运动能力没有差异。然而，亚组分析表明，吸气肌训练有利于增加最大吸气压力，定性分析表明，呼吸肌训练可能有益于呼吸肌耐力，而没有任何副作用。这项系统评价和荟萃分析表明，尽管表明呼吸肌训练益处的证据水平较低，但其临床意义表明我们需要进一步研究方法学质量以确定训练的有效性。本次审查的方案已记录在国际前瞻性系统评价登记册（PROSPERO）中，登记号为 CRD42023441829。