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    Top 10 research priorities for people living with pulmonary fibrosis, their caregivers, healthcare professionals and researchers. Tikellis Gabriella,Tong Allison,Lee Joanna Y T,Corte Tamera J,Hey-Cunningham Alison J,Bartlett Michael,Crawford Tonia,Glaspole Ian,Price John,Maloney Jamie,Holland Anne E Thorax INTRODUCTION:People with pulmonary fibrosis (PF) experience a high symptom burden, reduced quality of life and a shortened lifespan. Treatment options are limited and little is known about what patients, caregivers and healthcare professionals (HCPs)/researchers consider as the most important research priorities. This study aimed to identify the top 10 research priorities for PF across all stakeholders. METHODS:Participants included people with PF, caregivers and HCPs/researchers involved with PF. The research priority setting exercise involved three stages: (1) identifying priorities using an open-ended questionnaire and thematic analysis, (2) development of specific research questions at a face-to-face workshop, and (3) online ranking of research questions to identify the top 10 research priorities using nominal group ranking method. RESULTS:196 participants completed stage 1 generating 560 questions and 14 research themes were identified. Stage 2 involved 32 participants and generated 53 indicative questions from which 39 were used for the final ranking. Stage 3 was completed by 270 participants. The top ranked priorities focussed on medications to reverse scarring in the lungs (ranked 1), improving lung function (ranked 2, 6 and 8), interventions aimed at alleviating symptoms (ranked 5 and 7), prevention of PF (ranked 3 and 4) and the best exercise programme for PF (ranked 10). There was good consensus among patients/carers and HCPs/researchers on the top 10 priorities, however, causes of acute exacerbations and early diagnosis for improving survival, was ranked higher by HCPs/researchers. CONCLUSION:Interventions for preserving lung health and alleviation of symptom burden were top research priorities for PF stakeholders. 10.1136/thoraxjnl-2020-215731
    Impact of Pulmonary Rehabilitation on Exercise Tolerance and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A SYSTEMATIC REVIEW AND META-ANALYSIS. Gomes-Neto Mansueto,Silva Cassio Magalhães,Ezequiel Diego,Conceição Cristiano Sena,Saquetto Micheli,Machado Adelmir Souza Journal of cardiopulmonary rehabilitation and prevention BACKGROUND:Patients with pulmonary fibrosis are living longer and present with an increasing number of comorbidities over time. Pulmonary rehabilitation, as a nonpharmacological approach, may be promising in these patients, although there is limited information on the impact of pulmonary rehabilitation on exercise tolerance and quality of life. Thus, conducting a systematic review and meta-analysis, the purpose of this study was to determine the effects of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis. METHODS:We searched MEDLINE, Cochrane Library, Embase, Scielo, PEDro, and CINAHL (from the earliest date available to June 2016) for trials. Study selection included randomized controlled trials (RCTs) that examined the effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Two reviewers selected studies independently. Data were extracted from published RCTs. Study quality was evaluated using the PEDro scale. Weighted mean differences, standard mean differences, and 95% CIs were calculated. RESULTS:We analyzed data from 5 RCTs comparing a pulmonary rehabilitation group with a control group. Pulmonary rehabilitation improved exercise tolerance weighted mean differences (44 m; 95% CI, 5.3-82.8) compared with no exercise. The meta-analyses also showed significant improvement in symptoms, impact, and total score from the St George's Respiratory Questionnaire for participants in pulmonary rehabilitation compared with control. No serious adverse events were reported. CONCLUSION:Pulmonary rehabilitation is effective in increasing exercise tolerance and improving quality of life in patients with idiopathic pulmonary fibrosis. 10.1097/HCR.0000000000000273
    Clinical evidence for improving exercise tolerance and quality of life with pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A systematic review and meta-analysis. Clinical rehabilitation OBJECTIVE:To evaluate the efficacy and safety of pulmonary rehabilitation for exercise tolerance and quality of life improvement in idiopathic pulmonary fibrosis. METHODS:We searched PubMed, Cochrane Library, Embase, Web of Science, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, Wanfang Database, and Chongqing VIP for randomized controlled trials that compared pulmonary rehabilitation with usual care for idiopathic pulmonary fibrosis. The risk of bias and certainty of evidence were assessed using Cochrane Collaboration's Risk of Bias tool and the Grading of Recommendations, Assessment, Development and Evaluation criteria. RESULTS:Eleven trials in total with 549 participants. Compared with usual care, pulmonary rehabilitation significantly increased 6-minute walking distance (mean difference: 35.2m, 95% confidence intervals: 25.4m-44.9m; ten trials; 447 participants; moderate), decreased the St George's Respiratory Questionnaire total scores (mean difference: -9.11, 95% confidence intervals: -10.78 to -7.43; six trials; 303 participants; moderate), and reduced the modified Medical Research Council scores were lower (mean difference: -0.76, 95% confidence intervals: -1.25 to -0.27; three trials; 196 participants; low). Improvements were noted in forced vital capacity percent-predicted (mean difference: 4.88, 95% confidence intervals: 2.67 to 7.10; four trials; 214 participants; moderate) and diffusing capacity for carbon monoxide (mean difference: 4.71, 95% confidence intervals: 0.96 to 8.46; six trials; 358 participants; low). CONCLUSIONS:Pulmonary rehabilitation may significantly improve exercise tolerance and quality of life in idiopathic pulmonary fibrosis patients, but the quality of evidence was low to moderate. Large sample, multicenter, randomized controlled trials are needed to verify the efficacy and safety of pulmonary rehabilitation. 10.1177/02692155221095481