Moran Fidelma, Bradley Judy M, Piper Amanda J
Institute of Nursing and Health Research and School of Health Sciences, Ulster University, Shore Road, Newtownabbey, Northern Ireland, UK, BT37 0QB.
The Wellcome Trust-Wolfson Northern Ireland Clinical Research Facility U Floor, Queen's University Belfast, Belfast City Hospital, Lisburn Road, Belfast, Northern Ireland, UK, BT9 7AB.
Cochrane Database Syst Rev. 2017 Feb 20;2(2):CD002769. doi: 10.1002/14651858.CD002769.pub5.
Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review.
To compare the effect of non-invasive ventilation versus no non-invasive ventilation in people with cystic fibrosis for airway clearance, during sleep and during exercise.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We searched the reference lists of each trial for additional publications possibly containing other trials.Most recent search: 08 August 2016.
Randomised controlled trials comparing a form of pressure preset or volume preset non-invasive ventilation to no non-invasive ventilation used for airway clearance or during sleep or exercise in people with acute or chronic respiratory failure in cystic fibrosis.
Three reviewers independently assessed trials for inclusion criteria and methodological quality, and extracted data.
Ten trials met the inclusion criteria with a total of 191 participants. Seven trials evaluated single treatment sessions, one evaluated a two-week intervention, one evaluated a six-week intervention and one a three-month intervention. It is only possible to blind trials of airway clearance and overnight ventilatory support to the outcome assessors. In most of the trials we judged there was an unclear risk of bias with regards to blinding due to inadequate descriptions. The six-week trial was the only one judged to have a low risk of bias for all other domains. One single intervention trial had a low risk of bias for the randomisation procedure with the remaining trials judged to have an unclear risk of bias. Most trials had a low risk of bias with regard to incomplete outcome data and selective reporting.Six trials (151 participants) evaluated non-invasive ventilation for airway clearance compared with an alternative chest physiotherapy method such as the active cycle of breathing techniques or positive expiratory pressure. Three trials used nasal masks, one used a nasal mask or mouthpiece and one trial used a face mask and in one trial it is unclear. Three of the trials reported on one of the review's primary outcome measures (quality of life). Results for the reviews secondary outcomes showed that airway clearance may be easier with non-invasive ventilation and people with cystic fibrosis may prefer it. We were unable to find any evidence that non-invasive ventilation increases sputum expectoration, but it did improve some lung function parameters.Three trials (27 participants) evaluated non-invasive ventilation for overnight ventilatory support compared to oxygen or room air using nasal masks (two trials) and nasal masks or full face masks (one trial). Trials reported on two of the review's primary outcomes (quality of life and symptoms of sleep-disordered breathing). Results for the reviews secondary outcome measures showed that they measured lung function, gas exchange, adherence to treatment and preference, and nocturnal transcutaneous carbon dioxide. Due to the small numbers of participants and statistical issues, there were discrepancies in the results between the RevMan and the original trial analyses. No clear differences were found between non-invasive ventilation compared with oxygen or room air except for exercise performance, which significantly improved with non-invasive ventilation compared to room air over six weeks.One trial (13 participants) evaluated non-invasive ventilation on exercise capacity (interface used was unclear) and did not reported on any of the review's primary outcomes. The trial found no clear differences between non-invasive ventilation compared to no non-invasive ventilation for any of our outcomes.Three trials reported on adverse effects. One trial, evaluating non-invasive ventilation for airway clearance, reported that a participant withdrew at the start of the trial due to pain on respiratory muscle testing. One trial evaluating non-invasive ventilation for overnight support reported that one participant could not tolerate an increase in inspiratory positive airway pressure. A second trial evaluating non-invasive ventilation in this setting reported that one participant did not tolerate the non-invasive ventilation mask, one participant developed a pneumothorax when breathing room air and two participants experienced aerophagia which resolved when inspiratory positive airway pressure was decreased.
AUTHORS' CONCLUSIONS: Non-invasive ventilation may be a useful adjunct to other airway clearance techniques, particularly in people with cystic fibrosis who have difficulty expectorating sputum. Non-invasive ventilation, used in addition to oxygen, may improve gas exchange during sleep to a greater extent than oxygen therapy alone in moderate to severe disease. The effect of NIV on exercise is unclear. These benefits of non-invasive ventilation have largely been demonstrated in single treatment sessions with small numbers of participants. The impact of this therapy on pulmonary exacerbations and disease progression remain unclear. There is a need for long-term randomised controlled trials which are adequately powered to determine the clinical effects of non-invasive ventilation in cystic fibrosis airway clearance and exercise.
无创通气可能是一种通过提供通气支持并避免气管插管来暂时逆转或减缓囊性纤维化患者呼吸衰竭进展的方法。在适当的情况或个体中使用无创通气，可通过增加气流和气体交换以及减少呼吸功来改善肺力学。因此，无创通气起到了外部呼吸肌的作用。这是对先前发表的综述的更新。
比较无创通气与不进行无创通气对囊性纤维化患者气道清除、睡眠期间及运动期间的影响。
我们检索了Cochrane囊性纤维化和遗传疾病小组试验注册库，其中包括通过全面电子数据库检索、手工检索相关期刊以及会议论文摘要集确定的参考文献。我们检索了每项试验的参考文献列表，以查找可能包含其他试验的额外出版物。最新检索日期：2016年8月8日。
随机对照试验，比较压力预设或容量预设形式的无创通气与不进行无创通气在囊性纤维化急性或慢性呼吸衰竭患者气道清除、睡眠或运动期间的效果。
三位综述作者独立评估试验的纳入标准和方法学质量，并提取数据。
10项试验符合纳入标准，共有191名参与者。7项试验评估了单次治疗，1项评估了为期两周的干预，1项评估了为期六周的干预，1项评估了为期三个月的干预。对于气道清除和夜间通气支持试验，仅可能对结果评估者实施盲法。在大多数试验中，由于描述不充分，我们判断在盲法方面存在不明确的偏倚风险。为期六周的试验是唯一在所有其他领域被判定为低偏倚风险的试验。一项单次干预试验在随机化程序方面具有低偏倚风险，其余试验被判定为具有不明确的偏倚风险。大多数试验在不完整结果数据和选择性报告方面具有低偏倚风险。6项试验（151名参与者）评估了无创通气用于气道清除，并与替代胸部物理治疗方法（如主动呼吸循环技术或呼气末正压）进行比较。3项试验使用鼻罩，1项试验使用鼻罩或口含器，1项试验使用面罩，还有1项试验情况不明。其中3项试验报告了综述的一项主要结局指标（生活质量）。综述次要结局的结果表明，无创通气可能使气道清除更容易，囊性纤维化患者可能更喜欢它。我们未找到任何证据表明无创通气可增加痰液咳出，但它确实改善了一些肺功能参数。3项试验（27名参与者）评估了无创通气用于夜间通气支持，并与使用鼻罩（2项试验）和鼻罩或全面罩（1项试验）的吸氧或室内空气进行比较。试验报告了综述的两项主要结局（生活质量和睡眠呼吸障碍症状）。综述次要结局指标的结果表明，这些指标包括肺功能、气体交换、治疗依从性和偏好以及夜间经皮二氧化碳。由于参与者数量少和统计问题，RevMan与原始试验分析的结果存在差异。与吸氧或室内空气相比，无创通气除运动表现外未发现明显差异，与室内空气相比，无创通气在六周内可显著改善运动表现。1项试验（13名参与者）评估了无创通气对运动能力的影响（使用的接口不明），未报告综述的任何主要结局。该试验发现，对于我们的任何结局，无创通气与不进行无创通气相比均无明显差异。3项试验报告了不良反应。1项评估无创通气用于气道清除的试验报告，1名参与者在试验开始时因呼吸肌测试疼痛而退出。1项评估无创通气用于夜间支持的试验报告，1名参与者无法耐受吸气气道正压增加。第二项评估此情况下无创通气的试验报告，1名参与者无法耐受无创通气面罩，1名参与者在呼吸室内空气时发生气胸，2名参与者出现吞气症，当吸气气道正压降低时症状缓解。
无创通气可能是其他气道清除技术的有用辅助手段，特别是对于咳痰困难的囊性纤维化患者。在中重度疾病中，除吸氧外使用无创通气可能比单纯吸氧在更大程度上改善睡眠期间的气体交换。无创通气对运动的影响尚不清楚。无创通气的这些益处大多在参与者数量较少的单次治疗中得到证实。这种疗法对肺部加重和疾病进展的影响仍不清楚。需要进行长期随机对照试验，并有足够的样本量来确定无创通气在囊性纤维化气道清除和运动方面的临床效果。
