文獻回顧問題
與不使用呼吸道清除技術清除囊腫性纖維化患者肺部多餘黏液相比，使用任何一種呼吸道清除技術有何影響？
研究背景
囊腫性纖維化患者的肺部會產生過多的黏液。這導致肺部反覆的感染和組織損傷。使用藥物和呼吸道清除技術（物理治療）清除黏液很重要。有各種不同的呼吸道清除技術可以用於清除黏液，其中一些可能包括使用機械設備。每天的物理治療費時且費力，所以了解它是否有效很重要。我們搜索了研究，在這些研究中，參與者有同等機會進入使用呼吸道清除技術或沒有使用呼吸道清除技術的組別。本文是先前已出版文獻的更新版。
文獻搜尋日期
證據更新至 2022 年 10 月 17 日。
研究特徵
我們納入 12 項研究，包含 194 名囊腫性纖維化患者。這些研究非常不同，有些研究將多種治療與不治療進行比較。1 項研究使用自體引流（一種控制呼吸技術，使用不同的呼氣速度和深度將黏液向上移動到呼吸道，以便透過咳嗽將其清除）；5 項研究使用了傳統的胸部物理治療（通常在物理治療師或親屬的幫助下，對胸壁進行敲擊和振動的手動技術）；9 項使用正壓呼氣（透過面罩或喉舌抵抗阻力呼氣，這會導致肺部積聚壓力以移動黏液），其中 1 項使用標準和高壓正壓呼氣；3 項研究使用振盪正壓呼氣（正壓呼氣與呼吸道內的振動相結合以鬆動黏液）；2 項使用運動（在跑步機上），和 2 項使用的高頻胸壁振盪（透過充氣服裝在胸壁外施加高頻振動）。我們無法合併任何結果來進行統合分析。
主要結果
總結這 12 項研究的結果，我們發現對肺功能短期影響的證據有限。只有 1 項研究報告了一些治療組的肺功能有所改善，而其他 6 項研究則沒有發現任何改善。
這篇文獻回顧發現，清理呼吸道的方法可能對移動黏液有短期好處。4 項研究發現使用呼吸道清除技術的人咳出更多痰，但 1 項研究報告使用呼吸道清除技術與否並無差異。5 項研究報告了使用呼吸道清除技術時，放射性標記物清除率增加（1 項測試，在該測試中，人們吸入放射性氣溶膠後連續成像，以評估其從肺部清除的時間），但 1 項正壓呼氣研究沒有發現差異。目前，沒有明確的證據表明進行呼吸道清除技術對生活品質或生存的長期有影響。
證據的限制性
基於許多理由，我們對於此證據幾乎沒有信心。大多數納入的研究都存在設計問題，而且在接近一半的研究中，並不清楚是否報告了所有結果。此外，在物理治療研究中，接受治療的人和他們的物理治療師都知道他們正在接受哪種治療，這可能會影響某些研究結果。例如，如果一個人意識到他們正在接受的治療，咳出的黏液量和肺功能測試（由一半的納入研究被測量）以及一個人對特定技術的看法（在四分之一的納入研究中被記錄）可能會受到影響。最後，在大多數研究中，並不清楚每個人是否對他們所使用的技術有經驗。
我們無法找到任何關於呼吸道清除技術對接受新的囊腫性纖維化跨膜電導調節劑 (CFTR) 治療的相關研究。囊腫性纖維化是由突變的 CFTR 基因在細胞表面產生的錯誤蛋白質所引起的；這些新藥被設計來校正有缺陷的蛋白質功能。
The evidence from this review shows that ACTs may have short-term effects on increasing mucus transport in people with CF. All included studies had short-term follow-up; consequently, we were unable to draw any conclusions on the long-term effects of ACTs compared to no ACTs in people with CF.
The evidence in this review represents the use of airway clearance techniques in a CF population before widespread use of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Further research is needed to determine the effectiveness and acceptability of airway clearance in those treated with highly effective CFTR modulators.
Cystic fibrosis (CF) is an inherited progressive life-limiting disease characterised by the build-up of abnormally thick, sticky mucus affecting mostly the lungs, pancreas, and digestive system. Airway clearance techniques (ACTs), traditionally referred to as chest physiotherapy, are recommended as part of a complex treatment programme for people with CF. The aim of an ACTs is to enhance mucociliary clearance and remove viscous secretions from the airways within the lung to prevent distal airway obstruction. This reduces the infective burden and associated inflammatory effects on the airway epithelia.
There are a number of recognised ACTs, none of which have shown superiority in improving short-term outcomes related to mucus transport. This systematic review, which has been updated regularly since it was first published in 2000, considers the efficacy of ACTs compared to not performing any ACT in adults and children with CF. It is important to continue to review this evidence, particularly the long-term outcomes, given the recent introduction of highly effective modulator therapies and the improved health outcomes and potential changes to CF management associated with these drugs.
To determine the effectiveness and acceptability of airway clearance techniques compared to no airway clearance techniques or cough alone in people with cystic fibrosis.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings, to 17 October 2022. We searched ongoing trials registers (Clinicaltrials.gov and the WHO International Clinical Trials Registry Platform) to 7 November 2022.
We included randomised or quasi-randomised studies that compared airway clearance techniques (chest physiotherapy) with no airway clearance techniques or spontaneous cough alone in people with CF.
Both review authors independently assessed study eligibility, extracted data, and assessed the risk of bias of the included studies. We used GRADE methodology to assess the certainty of the evidence.
We included 11 cross-over studies (153 participants) and one parallel study (41 participants). There were differences between studies in how the interventions were delivered, with several intervention groups combining more than one ACT. One study used autogenic drainage; five used conventional chest physiotherapy; nine used positive expiratory pressure (PEP), with one study varying the water pressure between arms; three studies used oscillating PEP; two used exercise; and two used high-frequency chest wall oscillation (HFCWO). Of the 12 included studies, 10 were single-treatment studies, and two delivered the intervention over two consecutive days (once daily in one study, twice daily in the second). This substantial heterogeneity in the treatment interventions precluded pooling of data for meta-analysis. Blinding of participants, caregivers, and clinicians is impossible in airway clearance studies; we therefore judged all studies at unclear risk of performance bias. Lack of information in eight studies made assessment of risk of bias unclear for most other domains.
We rated the certainty of evidence as low or very low due to the short-term cross-over trial design, small numbers of participants, and uncertain risk of bias across most or all domains.
Six studies (84 participants) reported no effect on pulmonary function variables following intervention; but one study (14 participants) reported an improvement in pulmonary function following the intervention in some of the treatment groups. Two studies reported lung clearance index: one (41 participants) found a variable response to treatment with HFCWO, whilst another (15 participants) found no effect on lung clearance index with PEP therapy (low-certainty evidence). Five studies (55 participants) reported that ACTs, including coughing, increased radioactive tracer clearance compared to control, while a further study (eight participants) reported no improvement in radioactive tracer clearance when comparing PEP to control, although coughing was discouraged during the PEP intervention. We rated the certainty of evidence on the effect of ACTs on radioactive tracer clearance as very low.
Four studies (46 participants) investigated the weight of mucus cleared from the lungs and reported greater secretions during chest physiotherapy compared to a control. One study (18 participants) reported no differences in sputum weight (very low-certainty evidence).
翻譯者：洪峻澤 (臺北醫學大學藥學系臨床藥學組六年級) 【本翻譯計畫由臺北醫學大學考科藍臺灣研究中心 (CochraneTaiwan) 及東亞考科藍聯盟 (EACA）統籌執行。 聯絡 E-mail:cochranetaiwan@tmu.edu.tw】