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Background The Quality of Life-Bronchiectasis (QOL-B), a self-administered, patient-reported outcome measure assessing symptoms, functioning and health-related quality of life for patients with non-cystic fibrosis (CF) bronchiectasis, contains 37 items on 8 scales (Respiratory Symptoms, Physical, Role, Emotional and Social Functioning, Vitality, Health Perceptions and Treatment Burden). Methods Psychometric analyses of QOL-B V.3.0 used data from two double-blind, multicentre, randomised, placebo-controlled, phase III trials of aztreonam for inhalation solution (AZLI) in 542 patients with non-CF bronchiectasis and Gram-negative endobronchial infection. Results Excellent internal consistency (Cronbach's α ≥0.70) and 2-week test–retest reliability (intraclass correlation coefficients ≥0.72) were demonstrated for each scale. Convergent validity with 6 min walk test was observed for Physical and Role Functioning scores. No floor or ceiling effects (baseline scores of 0 or 100) were found for the Respiratory Symptoms scale (primary endpoint of trials). Baseline Respiratory Symptoms scores discriminated between patients based on baseline FEV1% predicted in only one trial. The minimal important difference score for the Respiratory Symptoms scale was 8.0 points. AZLI did not show efficacy in the two phase III trials. QOL-B responsivity to treatment was assessed by examining changes from baseline QOL-B scores at study visits at which protocol-defined pulmonary exacerbations were reported. Mean Respiratory Symptoms scores decreased 14.0 and 14.2 points from baseline for placebo-treated and AZLI-treated patients with exacerbations, indicating that worsening respiratory symptoms were reflected in clinically meaningful changes in QOL-B scores. Conclusions Previously established content validity, reliability and responsivity of the QOL-B are confirmed by this final validation study. The QOL-B is available for use in clinical trials and routine clinical practice.

Background Exercise training is recommended for non-cystic fibrosis (CF) bronchiectasis, but the long-term effects are unclear. This randomised controlled trial aimed to determine the effects of exercise training and review of airway clearance therapy (ACT) on exercise capacity, health related quality of life (HRQOL) and the incidence of acute exacerbations in people with non-CF bronchiectasis. Methods Participants were randomly allocated to 8 weeks of supervised exercise training and review of ACT, or control. Primary outcomes of exercise capacity and HRQOL (Chronic respiratory disease questionnaire) and secondary outcomes of cough-related QOL (Leicester cough questionnaire) and psychological symptoms (Hospital anxiety and depression scale) were measured at baseline, following completion of the intervention period and at 6 and 12 months follow up. Secondary outcomes of the exacerbation rate and time to first exacerbation were analysed over 12 months. Results Eighty-five participants (mean FEV 1 74% predicted; median Modified Medical Research Council Dyspnoea grade of 1 (IQR [1–3]) were included. Exercise training increased the incremental shuttle walk distance (mean difference to control 62 m, 95% CI 24 to 101 m) and the 6-minute walking distance (mean difference to control 41 m, 95% CI 19 to 63 m), but these improvements were not sustained at 6 or 12 months. Exercise training reduced dyspnoea (p = 0.009) and fatigue (p = 0.01) but did not impact on cough-related QOL or mood. Exercise training reduced the frequency of acute exacerbations (median 1[IQR 1–3]) compared to the control group (2[1–3]) over 12 months follow up (p = 0.012), with a longer time to first exacerbation with exercise training of 8 months (95% CI 7 to 9 months) compared to the control group (6 months [95% CI 5 to 7 months], p = 0.047). Conclusions Exercise training in bronchiectasis is associated with short term improvement in exercise capacity, dyspnoea and fatigue and fewer exacerbations over 12 months. Trial registry ClinicalTrials.gov ( NCT00885521 ).

Background There has been comparatively little patient information about bronchiectasis, a chronic lung disease with rising prevalence. Patients want more information, which could improve their understanding and self-management. A novel information resource meeting identified needs has been co-developed in prior work. We sought to establish the feasibility of conducting a multi-centre randomised controlled trial to determine effect of the information resource on understanding, self-management and health outcomes. Methods/design We conducted an unblinded, single-centre, randomised controlled feasibility trial with two parallel groups (1:1 ratio), comparing a novel patient information resource with usual care in adults with bronchiectasis. Integrated qualitative methods allowed further evaluation of the intervention and trial process. The setting was two teaching hospitals in North East England. Participants randomised to the intervention group received the information resource (website and booklet) and instructions on its use. Feasibility outcome measures included willingness to enter the trial, in addition to recruitment and retention rates. Secondary outcome measures (resource use and satisfaction, quality of life, unscheduled healthcare presentations, exacerbation frequency, bronchiectasis knowledge and lung function) were recorded at baseline, 2 weeks and 12 weeks. Results Sixty-two participants were randomised (control group = 30; intervention group = 32). Thirty-eight (61%) were female, and the participants’ median age was 65 years (range 15–81). Median forced expiratory volume in 1 s percent predicted was 68% (range 10–120). Sixty-two of 124 (50%; 95% CI, 41–59%) of potentially eligible participants approached were recruited. Sixty (97%) of 62 participants completed the study (control group, 29 of 30 [97%]; 95% CI, 83–99%; 1 unrelated death; intervention group, 31 [97%] of 32; 95% CI, 84–99%; 1 withdrawal). In the intervention group, 27 (84%) of 32 reported using the information provided, and 25 (93%) of 27 of users found it useful, particularly the video content. Qualitative data analysis revealed acceptability of the trial and intervention. Web analytics recorded over 20,000 page views during the 16-month study period. Conclusion The successful recruitment process, high retention rate and study form completion rates indicate that it appears feasible to conduct a full trial based on this study design. Worldwide demand for online access to the information resource was high. Trial registration ISRCTN Registry, ISRCTN84229105 . Registered on 25 July 2014.

Background: Non-cystic-fibrosis bronchiectasis (non-CF BE) continues to be a problem in developing countries and it is therefore important to examine and assess this disease. Objectives: The aims of this prospective study were to evaluate the health-related quality of life (HRQOL) in non-CF BE children and also to assess the risk factors associated with HRQOL. Methods: Forty-two non-CF BE patients between the ages of 9 and 18 years were enrolled in the study. All recruited patients completed the generic Short-Form-36 (SF-36), the St. George's Respiratory Questionnaire (SGRQ) for disease-specific QOL scale and forms on socioeconomic status (SES). The extent and severity of CT abnormalities were evaluated by using the modified Bhalla scoring system. Association between HRQOL questionnaires and demographic variables, pulmonary function test, high-resolution CT scores and SES were evaluated. Results: SF-36 and SGRQ subscales all correlated inversely with each other (SF-36 physical component summary with SGRQ symptoms score: r = -0.466, p = 0.001, activity score: r = -0.666, p = 0.000 and impact score: r = -0.667, p = 0.000. SF-36 mental component summary with SGRQ symptoms score: r = -0.396, p = 0.005, activity score: r = -0.533, p = 0.000 and impact score: r = -0.512, p = 0.000). There was an inverse correlation between SGRQ symptoms scores and the duration of regular follow-up (r = -0.3, p = 0.04). The symptoms subscale of SGRQ correlated positively with low values for pulmonary function testing (r = -0.417, p = 0.003) and frequent antibiotic requirements (r = 0.303, p = 0.035). Conclusions: Early diagnosis and regular follow-up of children with non-CF BE is important for improving their QOL. As expected, the severity and frequency of symptoms are inversely related to the pulmonary function and the QOL scores. A disease-specific questionnaire should be developed to monitor QOL in children with non-CF BE.

Background Despite bronchiectasis being increasingly recognised as an important cause of chronic respiratory morbidity in both indigenous and non-indigenous settings globally, high quality evidence to inform management is scarce. It is assumed that antibiotics are efficacious for all bronchiectasis exacerbations, but not all practitioners agree. Inadequately treated exacerbations may risk lung function deterioration. Our study tests the hypothesis that both oral azithromycin and amoxicillin-clavulanic acid are superior to placebo at improving resolution rates of respiratory exacerbations by day 14 in children with bronchiectasis unrelated to cystic fibrosis. Methods We are conducting a b ronchiectasis e xacerbation st udy (BEST), which is a multicentre, randomised, double-blind, double-dummy, placebo-controlled, parallel group trial, in five centres (Brisbane, Perth, Darwin, Melbourne, Auckland). In the component of BEST presented here, 189 children fulfilling inclusion criteria are randomised (allocation-concealed) to receive amoxicillin-clavulanic acid (22.5 mg/kg twice daily) with placebo-azithromycin; azithromycin (5 mg/kg daily) with placebo-amoxicillin-clavulanic acid; or placebo-azithromycin with placebo-amoxicillin-clavulanic acid for 14 days. Clinical data and a paediatric cough-specific quality of life score are obtained at baseline, at the start and resolution of exacerbations, and at day 14. In most children, blood and deep nasal swabs are also collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 14. The main secondary outcome is the paediatric cough-specific quality of life score. Other outcomes are time to next exacerbation; requirement for hospitalisation; duration of exacerbation; and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood markers will also be reported. Discussion Effective, evidence-based management of exacerbations in people with bronchiectasis is clinically important. Yet, there are few randomised controlled trials (RCTs) in the neglected area of non-cystic fibrosis bronchiectasis. Indeed, no published RCTs addressing the treatment of bronchiectasis exacerbations in children exist. Our multicentre, double-blind RCT is designed to determine if azithromycin and amoxicillin-clavulanic acid, compared with placebo, improve symptom resolution on day 14 in children with acute respiratory exacerbations. Our planned assessment of the predictors of antibiotic response, the role of antibiotic-resistant respiratory pathogens, and whether early treatment with antibiotics affects duration and time to the next exacerbation, are also all novel. Trial registration Australia and New Zealand Clinical Trials Register (ANZCTR) number ACTRN12612000011886.

Bronchiectasis guidelines are inconsistent with regard to the effectiveness of mucoactive agents, and their use varies geographically. Large trials are needed to assess safety and effectiveness. For this open-label, randomized, two-by-two factorial trial at 20 sites in the United Kingdom, we enrolled participants with non-cystic fibrosis bronchiectasis who had frequent pulmonary exacerbations and daily sputum production. Current smokers and persons who had recently received mucoactive treatments were excluded. All participants received standard care and were also assigned either to one of three mucoactive-drug groups - hypertonic saline (the hypertonic-saline group), hypertonic saline and carbocisteine (the combination group), or carbocisteine (the carbocisteine group) - or to standard care alone. The comparisons were between hypertonic saline and no hypertonic saline and between carbocisteine and no carbocisteine, with each category consisting of two groups. The primary outcome was the number of pulmonary exacerbations over a 52-week period. Key secondary outcomes were scores on disease-specific health-related quality-of-life assessments, time to next pulmonary exacerbation, and safety. A total of 288 participants underwent randomization. No treatment interactions were found. The mean number of adjudicated fully qualifying pulmonary exacerbations over the 52-week period was 0.76 (95% confidence interval [CI], 0.58 to 0.95) with hypertonic saline as compared with 0.98 (95% CI, 0.78 to 1.19) with no hypertonic saline (adjusted between-group difference in the means, -0.25 [95% CI, -0.57 to 0.07; P = 0.12]) and 0.86 (95% CI, 0.66 to 1.06) with carbocisteine as compared with 0.90 (95% CI, 0.70 to 1.09) with no carbocisteine (adjusted between-group difference in the means, -0.04 [95% CI, -0.36 to 0.28; P = 0.81]). Secondary outcomes and the incidence of adverse events, including serious adverse events, were similar across the groups. In participants with bronchiectasis, neither hypertonic saline nor carbocisteine significantly reduced the mean incidence of pulmonary exacerbations over a period of 52 weeks. (Funded by the National Institute for Health and Care Research Health Technology Assessment Programme and others; ISRCTN Registry number, ISRCTN89040295.).

Background The Bronchiectasis Health Questionnaire (BHQ), a 10-question instrument designed to assess quality of life and disease-specific issues, was developed in 11 languages, including English. The present study aims to translate the original version of the BHQ into Turkish using a standardised methodology and to evaluate the validity and reliability of the Turkish version of the BHQ. Methods This cross-sectional study was conducted at Giresun Training and Research Hospital between August 2024 and January 2025, with ethical approval (decision number 03.07.2024/03) and informed consent from all participants. A total of 132 clinically stable participants with bronchiectasis were enrolled. The study was performed in two stages: (1) cross-cultural adaptation of the original Bronchiectasis Health Questionnaire (BHQ) into Turkish (T-BHQ) according to established guidelines, and (2) psychometric validation of the T-BHQ. Alongside the T-BHQ, data on socio-demographics, clinical characteristics, mMRC dyspnea score, pulmonary function tests, Bronchiectasis Severity Index (BSI), SF-12 quality of life questionnaire, and exacerbation history were collected. Reliability was assessed using Cronbach’s alpha and test–retest analysis, while construct validity was evaluated through exploratory and confirmatory factor analyses, convergent validity with clinical measures, and Bland–Altman analysis. Results The validity and reliability of the T-BHQ were evaluated in present study involving a total of 134 participants with bronchiectasis, and a Cronbach’s alpha of 0.729 was obtained. In the exploratory factorial analysis, factorial loadings of > 0.40 were determined for all items and were evaluated as appropriate. Utilising the Bland-Altman analysis to assess the test’s repeatability, (mean difference: 0,059, β = 0.03, p:0.840). The convergent validity of the T-BHQ with mMRC was strong ( r  = − 0.648, p:< 0.001), with BSI were moderate (r: -0,437, p:<0,001), while concurrent validity with SF-12 subtypes mainly were at a moderate level (Physical role functioning: r  = − 0.661 p:<0.001, other subtypes at a moderate level). Conclusions The T-BHQ has been demonstrated to possess both reliability and validity in evaluating the disease-related quality of life and health status of participants with bronchiectasis in Turkey.

BackgroundA range of questionnaires have been used to assess health-related quality of life (HRQOL) in bronchiectasis. A systematic review was conducted to evaluate their psychometric properties and assess associations between HRQOL and clinical measures.MethodsFive electronic databases were searched. Studies eligible for inclusion were those that investigated the validity of HRQOL questionnaires and/or their association with other outcomes in adults with bronchiectasis. Patients with cystic fibrosis were excluded. The identified questionnaires were assessed for convergent, discriminant and cross-cultural translation validity; missing data, floor and ceiling effects, internal consistency, responsiveness and test-retest reliability. A meta-analysis was conducted to estimate the strength of associations between HRQOL and clinical measures.ResultsFrom 1918 studies identified, 43 studies were included in the systematic review, of which 38 were suitable for the meta-analysis. Nine HRQOL questionnaires were identified, with the most widely used being: St George's Respiratory Questionnaire, Leicester Cough Questionnaire, Quality of Life–Bronchiectasis and Short Form-36. HRQOL questionnaires had moderate to good internal consistency and good test-retest reliability. Only 8 of 18 studies that used translated HRQOL questionnaires reported or referred to the validity of the translated questionnaire. There was a stronger correlation (mean r (95% CI)) between HRQOL and subjective outcome measures, such as dyspnoea (0.55 (0.41 to 0.68)) and fatigue (0.42 (0.23 to 0.58)) compared with objective measures; exercise capacity (−0.41 (−0.54 to −0.24)), FEV1% predicted (−0.31 (−0.40 to −0.23)) and extent of bronchiectasis on CT scan (0.35 (0.03 to 0.61)); all p<0.001.ConclusionsThis review supports most HRQOL questionnaires used in bronchiectasis have good psychometric properties. There was a weak to moderate association between HRQOL and objective outcome measures. This suggests that HRQOL questionnaires assess a unique aspect of health not captured by objective measures.

ObjectivesTreatable traits are identifiable and treatable features of disease. The primary objective was to evaluate whether a bronchiectasis clinic in a regional setting, implementing a treatable traits approach, would lead to improvements in quality of life. Secondary objectives were to evaluate clinic implementation outcomes, annual exacerbations and hospitalisation.MethodsAn implementation study. Adults with bronchiectasis attending an outpatient clinic in Rockhampton, Australia between 2021 and 2023 were recruited. Clinical care was implemented by a physiotherapist and nurses according to traits identified. Quality of life (QoL-Bronchiectasis respiratory symptom score (QoL-B rs), Leicester Cough Questionnaire (LCQ) total score) was evaluated at baseline, three and 12-months from first attendance. Implementation outcomes were determined by clinic appointment uptake and attendance and pulmonary rehabilitation uptake and completion at 12-months. Exacerbation frequency and hospitalisation for the 12-months before and after enrolment were compared.Results50 participants were recruited [mean (SD) age 71 (12) years; Bronchiectasis Severity Index (%) mild (22) moderate (44) severe (34)]. There was a significant improvement in QoL at three and 12-months from baseline (mean difference, 95% CI) [3 months: QoL-B rs 9.2 (3.2-15.2), LCQ 1.7 (0.6-2.8); 12-months: QoL-B rs 10.1 (3.9-16.3), LCQ 1.8 (0.8-2.8)]. Clinic uptake (91%), attendance (97%) and pulmonary rehabilitation uptake (74%) and completion (84%) were high. Exacerbation frequency [median (IQR) 1.0 (3.0) to 0.0 (1.0), p = 0.007], and hospitalisation decreased (18% vs 0%, p = 0.005).DiscussionA treatable traits approach improved quality of life, was feasible, including the achievement of high pulmonary rehabilitation uptake and completion, and reduced exacerbation frequency and hospitalisation.

aim: Cough and airway secretions are part of daily life for people living with bronchiectasis. During the COVID-19 pandemic, infections associated with airway inflammation and cough amplified the health-related stigma and social unacceptability of coughing. This study explored the experiences and perceptions of adults with bronchiectasis during the pandemic to better understand the holistic impact of cough on their lives. method: A qualitative, interpretive descriptive study was undertaken using semi-structured interviews with 15 adults living with bronchiectasis resident in Counties Manukau, Aotearoa New Zealand. results: Insights into the lives of adults living with bronchiectasis during the pandemic highlighted how they were impacted on multiple levels. Four key themes were developed that described participants’ struggle: “feeling vulnerable but keeping safe”; “being treated differently”; adjusting to “becoming a virtual patient”; and participants articulating an increased focus on “self-care and supportive communities” as key strategies. Communication with health teams became crucial, offering essential support for respiratory health, medication access, reassurance and social connectivity. conclusions: Health professionals play a key role in increasing public awareness around bronchiectasis and cough, helping to reduce stigma. While it is unknown when another disease outbreak mirroring that of COVID-19 will occur, the stigma of cough continues and warrants improved understanding.