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The Cochrane Database of Systematic... Jul 2016Pseudomonas aeruginosa is the most common bacterial pathogen causing lung infections in people with cystic fibrosis and appropriate antibiotic therapy is vital.... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Pseudomonas aeruginosa is the most common bacterial pathogen causing lung infections in people with cystic fibrosis and appropriate antibiotic therapy is vital. Antibiotics for pulmonary exacerbations are usually given intravenously, and for long-term treatment, via a nebuliser. Oral anti-pseudomonal antibiotics with the same efficacy and safety as intravenous or nebulised antibiotics would benefit people with cystic fibrosis due to ease of treatment and avoidance of hospitalisation. This is an update of a previous review.
OBJECTIVES
To determine the benefit or harm of oral anti-pseudomonal antibiotic therapy for people with cystic fibrosis, colonised with Pseudomonas aeruginosa, in the:1. treatment of a pulmonary exacerbation; and2. long-term treatment of chronic infection.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.We contacted pharmaceutical companies and checked reference lists of identified trials.Date of last search: 08 July 2016.
SELECTION CRITERIA
Randomised or quasi-randomised controlled trials comparing any dose of oral anti-pseudomonal antibiotics, to other combinations of inhaled, oral or intravenous antibiotics, or to placebo or usual treatment for pulmonary exacerbations and long-term treatment.
DATA COLLECTION AND ANALYSIS
Two authors independently selected the trials, extracted data and assessed quality. We contacted trial authors to obtain missing information.
MAIN RESULTS
We included three trials examining pulmonary exacerbations (171 participants) and two trials examining long-term therapy (85 participants). We regarded the most important outcomes as quality of life and lung function. The analysis did not identify any statistically significant difference between oral anti-pseudomonal antibiotics and other treatments for these outcome measures for either pulmonary exacerbations or long-term treatment. One of the included trials reported significantly better lung function when treating a pulmonary exacerbation with ciprofloxacin when compared with intravenous treatment; however, our analysis did not confirm this finding. We found no evidence of difference between oral anti-pseudomonal antibiotics and other treatments regarding adverse events or development of antibiotic resistance, but trials were not adequately powered to detect this. None of the studies had a low risk of bias from blinding which may have an impact particularly on subjective outcomes such as quality of life. The risk of bias for other criteria could not be clearly stated across the studies.
AUTHORS' CONCLUSIONS
We found no conclusive evidence that an oral anti-pseudomonal antibiotic regimen is more or less effective than an alternative treatment for either pulmonary exacerbations or long-term treatment of chronic infection with P. aeruginosa. Until results of adequately-powered future trials are available, treatment needs to be selected on a pragmatic basis, based upon any available non-randomised evidence, the clinical circumstances of the individual, the known effectiveness of drugs against local strains and upon individual preference.
Topics: Administration, Oral; Adult; Anti-Bacterial Agents; Child; Chronic Disease; Cystic Fibrosis; Humans; Pseudomonas Infections; Pseudomonas aeruginosa; Randomized Controlled Trials as Topic; Respiratory Tract Infections; Treatment Outcome
PubMed: 27412131
DOI: 10.1002/14651858.CD005405.pub4 -
Journal of Clinical Medicine Aug 2022The prevalence of obstructive sleep apnea (OSA) has greatly increased in recent years. Recent data suggest that severe and moderate forms of OSA affect between 6 and 17%... (Review)
Review
The prevalence of obstructive sleep apnea (OSA) has greatly increased in recent years. Recent data suggest that severe and moderate forms of OSA affect between 6 and 17% of adults in the general population. Many papers are reporting the significantly increased prevalence of OSA in patients suffering from fibrotic diseases, including idiopathic pulmonary fibrosis (IPF). Therefore, we performed a systematic review and meta-analysis regarding the dependency between IPF and OSA. Due to the lack of papers focusing on IPF among OSA patients, we focused on the prevalence of OSA among IPF patients. In the search strategy, a total of 684 abstracts were identified, 496 after the removal of duplicates. After the screening of titles and abstracts, 31 studies were qualified for further full-text analysis for eligibility criteria. The final analysis was performed on 614 IPF patients from 18 studies, which met inclusion criteria. There were 469 (76.38%) IPF patients with OSA and 145 (23.62%) without. The mean age varied from 60.9 ± 8.1 up to 70.3 ± 7.9. The obtained prevalence was 76.4 (95% CI: 72.9-79.7) and 75.7 (95% CI: 70.1-80.9) for fixed and random effects, respectively. The median prevalence of OSA among non-IPF patients for all the ethnics groups included in this study was 16,4% (IQR: 3.4%-26.8%). The study provides strong evidence for the increased prevalence of OSA in IPF patients when comparing with the general OSA prevalence.
PubMed: 36078938
DOI: 10.3390/jcm11175008 -
The Clinical Respiratory Journal Mar 2018Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic disease limited to the lungs. The course of disease varies widely, with some patients... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic disease limited to the lungs. The course of disease varies widely, with some patients experiencing acute respiratory deterioration, a condition called acute exacerbations of IPF (AE-IPF). The risk factors contributing to AE-IPF are unclear. This systematic review and meta-analysis investigated the risk factors for AE-IPF.
METHODS
Studies of risk factors for AE-IPF were identified in Medline, EMBASE and Cochrane databases. Fixed effects models were used to calculate pooled relative risks and weighted mean differences (WMD).The meta-analysis included seven articles involving 14 risk factors for AE-IPF.
RESULTS
Risk factors for AE included reductions in vital capacity (VC; WMD - 10.58, 95% confidence interval (CI) -17.17 to - 3.99), forced vital capacity (FVC; WMD -6.02, 95%CI - 8.58 to - 3.47), total lung capacity (TLC; WMD -4.88, 95%CI -7.59 to - 2.17), and PaO (WMD -4.19, 95%CI -7.66 to -0.71) and a higher alveolar-arterial oxygen difference (AaDO ; WMD 4.4, 95%CI 0.24 to 8.57). Mechanical procedures, higher serum KL-6 concentration and secondary pulmonary hypertension, might be risk factors for AE-IPF. In contrast, age, sex, body mass index (BMI), differences in diffusing lung capacity for carbon monoxide (DLCO), exposure to seasonal variations and air pollution, and virus infection might be unrelated to AE-IPF.
CONCLUSIONS
Poor pulmonary function, mechanical procedures, higher serum KL-6 and secondary pulmonary hypertension were associated with increased risks of AE-IPF.
Topics: Acute Disease; Blood Gas Analysis; Disease Progression; Humans; Idiopathic Pulmonary Fibrosis; Lung; Prognosis; Pulmonary Diffusing Capacity; Risk Factors; Vital Capacity
PubMed: 28332341
DOI: 10.1111/crj.12631 -
European Review For Medical and... Nov 2022The incidence of idiopathic pulmonary fibrosis is increasing year by year in the world, which has a greater impact on the quality of life of patients. In the past,... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The incidence of idiopathic pulmonary fibrosis is increasing year by year in the world, which has a greater impact on the quality of life of patients. In the past, symptomatic treatment was used in clinical practice, but the overall effect is still not good. Multiple clinical studies have demonstrated the efficacy of pirfenidone in the treatment of idiopathic pulmonary fibrosis; however, adverse reactions have been reported. We, therefore, systematically evaluated the effectiveness and safety of pirfenidone in patients with idiopathic pulmonary fibrosis.
PATIENTS AND METHODS
Relevant studies were retrieved from the Embase, PubMed, Web of Science, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chinese Biomedical Literature (CBM), Wanfang and Weipu databases between January 1999 and May 2020, including the keywords "pirfenidone" and "idiopathic pulmonary fibrosis", were included in our systematic review. Review Manager 5.4 software was used for data synthesis, and analyses of publication bias and sensitivity.
RESULTS
Our systematic review included 13 studies involving a total of 13247 patients with idiopathic pulmonary fibrosis. Pirfenidone was associated with reduced declines in vital capacity (VC) and forced vital capacity (FVC) from baseline in patients with hermansky-pudlak syndrome (HPS)-related pulmonary fibrosis and to moderate idiopathic pulmonary fibrosis (IPF). Pirfenidone treatment was associated with lower reductions in FVC, lower reductions in 6-minute walking test distance, lower decreases in minimum oxygen saturation during the 6-minute walking test, lower all-cause death, lower relative risk of IPF-related death and increased progression-free survival compared to placebo. Progression-free survival was significantly longer in the pirfenidone group. The incidence of gastrointestinal, skin, nervous system, and liver function-related adverse events was significantly higher in the pirfenidone group compared to the control group.
CONCLUSIONS
Pirfenidone has efficacy in delaying the progression of idiopathic pulmonary fibrosis. Pirfenidone is well-tolerated by the majority of patients; however, mild adverse reactions related to the gastrointestinal tract, skin, nervous system, and liver function are common. Overall, Pirfenidone may be an effective and well-tolerated treatment option for idiopathic pulmonary fibrosis.
Topics: Humans; Hermanski-Pudlak Syndrome; Quality of Life; Idiopathic Pulmonary Fibrosis; Vital Capacity; Skin
PubMed: 36459024
DOI: 10.26355/eurrev_202211_30377 -
Digestive Diseases and Sciences May 2023Cystic Fibrosis (CF) is associated with gut dysbiosis, local and systemic inflammation, and impaired immune function. Gut microbiota dysbiosis results from changes in...
BACKGROUND AND AIMS
Cystic Fibrosis (CF) is associated with gut dysbiosis, local and systemic inflammation, and impaired immune function. Gut microbiota dysbiosis results from changes in the complex gut milieu in response to CF transmembrane conductance regulator (CFTR) dysfunction, pancreatic malabsorption, diet, medications, and environmental influences. In several diseases, alteration of the gut microbiota influences local and systemic inflammation and disease outcomes. We conducted a systematic review of the gut microbiota in CF and explored factors influencing dysbiosis.
METHODS
An electronic search of three databases was conducted in January 2019, and re-run in June 2021. Human, animal, and in vitro studies were included. The primary outcome was differences in the gut microbiota between people with CF (pwCF) and healthy controls. Secondary outcomes included the relationship between the gut microbiota and other factors, including diet, medication, inflammation, and pulmonary function in pwCF.
RESULTS
Thirty-eight studies were identified. The literature confirmed the presence of CF-related gut dysbiosis, characterized by reduced diversity and several taxonomic changes. There was a relative increase of bacteria associated with a pro-inflammatory response coupled with a reduction of those considered anti-inflammatory. However, studies linking gut dysbiosis to systemic and lung inflammation were limited. Causes of gut dysbiosis were multifactorial, and findings were variable. Data on the impact of CFTR modulators on the gut microbiota were limited.
CONCLUSIONS
CF-related gut dysbiosis is evident in pwCF. Whether this influences local and systemic disease and is amenable to interventions with diet and drugs, such as CFTR modulators, requires further investigation.
Topics: Animals; Humans; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dysbiosis; Bacteria; Inflammation
PubMed: 36600119
DOI: 10.1007/s10620-022-07812-1 -
The Cochrane Database of Systematic... Jun 2015Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of previously published reviews. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of previously published reviews.
OBJECTIVES
To determine the effects of physical exercise training compared to no training on aerobic exercise capacity, forced expiratory volume in one second, health-related quality of life and other patient-relevant (secondary) outcomes in cystic fibrosis.
SEARCH METHODS
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.Date of the most recent search: 10 March 2015.
SELECTION CRITERIA
All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and duration with conventional care in people with cystic fibrosis.
DATA COLLECTION AND ANALYSIS
Two authors independently selected studies for inclusion, assessed methodological quality and extracted data.
MAIN RESULTS
Of the 48 studies identified, 13 studies which included 402 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; one study was in adults, six were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and nine studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows limited evidence from both short- and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training or a combination of both has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. Although improvements are not consistent between studies and ranged from no effects to clearly positive effects, the most consistent effects of the heterogeneous exercise training modalities and durations were found for maximal aerobic exercise capacity (in four out of six studies) with unclear effects on FEV1 (in two out of 10 studies) and health-related quality of life (in two out of five studies).
AUTHORS' CONCLUSIONS
Evidence about the efficacy of physical exercise training in cystic fibrosis from 13 small studies with low to moderate methodological quality is limited. Exercise training is already part of regular outpatient care offered to most people with cystic fibrosis, and since there is some evidence for beneficial effects on aerobic fitness and no negative side effects exist, there is no reason to actively discourage this. The benefits from including physical exercise training in an individual's regular care may be influenced by the type and duration of the training programme. High quality randomised controlled trials are needed to comprehensively assess the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical exercise training to the care of people with cystic fibrosis.
Topics: Cystic Fibrosis; Exercise Therapy; Exercise Tolerance; Humans; Randomized Controlled Trials as Topic
PubMed: 26116828
DOI: 10.1002/14651858.CD002768.pub3 -
Respirology (Carlton, Vic.) Jan 2018This systematic review synthesizes published articles investigating the prevalence, severity and impact of urinary incontinence (UI), a condition associated with cystic... (Review)
Review
This systematic review synthesizes published articles investigating the prevalence, severity and impact of urinary incontinence (UI), a condition associated with cystic fibrosis (CF). References were identified through searching Medline, Embase and PubMed using the medical subject headings 'cystic fibrosis' AND 'urinary incontinence'. Articles were included if UI prevalence was investigated as an outcome. Twelve studies met selection criteria. The prevalence of UI ranged from 5% to 76%. Age and gender contributed to this variability. When assessed, UI commonly limited airway clearance, exercise and/or spirometry, and had a variable impact on patients' lives. Worry and embarrassment were features for many; others were less affected. In CF, UI is common and can interfere with respiratory care and social well-being. The prevalence, characteristics and impact are poorly understood, which is made worse by inconsistent definitions across studies. Future research is needed to improve approaches to prevention, identification, management and education.
Topics: Age Factors; Anxiety; Cystic Fibrosis; Exercise; Humans; Prevalence; Sex Factors; Shame; Spirometry; Urinary Incontinence
PubMed: 28718995
DOI: 10.1111/resp.13125 -
BMC Pulmonary Medicine Aug 2014Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and... (Review)
Review
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall, between single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and summarize wait-list survival.
METHODS
A systematic review of English-language studies published in Medline or Embase between 1990 and 2013 was performed. Eligible studies were those of observational design reporting survival post-lung transplantation or while on the wait list among IPF patients.
RESULTS
Median survival post-transplantation among IPF patients is estimated at 4.5 years. From ISHLT and OPTN data, one year survival ranged from 75% - 81%; 3-year: 59% - 64%; and 5-year: 47% - 53%. Post-transplant survival is lower for IPF vs. other underlying pre-transplant diagnoses. The proportion of IPF patients receiving BLT has steadily increased over the last decade and a half. Unadjusted analyses suggest improved long-term survival for BLT vs. SLT; after adjustment for patient characteristics, the differences tend to disappear. IPF patients account for the largest proportion of patients on the wait list and while wait list time has decreased, the number of transplants for IPF patients has increased over time. OPTN data show that wait list mortality is higher for IPF patients vs. other diagnoses. The proportion of IPF patients who died while awaiting transplantation ranged from 14% to 67%. While later transplant year was associated with increased survival, no significant differences were noted pre vs. post LAS implementation; however a high LAS vs low LAS was associated with decreased one-year survival.
CONCLUSIONS
IPF accounts for the largest proportion of patients awaiting lung transplants, and IPF is associated with higher wait-list and post-transplant mortality vs. other diagnoses. Improved BLT vs. SLT survival may be the result of selection bias. Survival pre- vs. post LAS appears to be similar except for IPF patients with high LAS, who have lower survival compared to pre-LAS. Data on post-transplant morbidity outcomes are sparse.
Topics: Graft Survival; Humans; Idiopathic Pulmonary Fibrosis; Lung Transplantation; Patient Acuity; Survival Rate; Waiting Lists
PubMed: 25127540
DOI: 10.1186/1471-2466-14-139 -
Archives of Medical Research Jun 2023A better capacity to identify patients with idiopathic pulmonary fibrosis (IPF) at risk of acute exacerbation (AEIPF) might improve outcomes and reduce healthcare costs. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
A better capacity to identify patients with idiopathic pulmonary fibrosis (IPF) at risk of acute exacerbation (AEIPF) might improve outcomes and reduce healthcare costs.
AIMS
We critically appraised the available evidence of the differences in clinical, respiratory, and biochemical parameters between AEIPF and IPF patients with stable disease (SIPF) by conducting a systematic review and meta-analysis.
METHODS
PubMed, Web of Science and Scopus were reviewed up until August 1, 2022, for studies reporting differences in clinical, respiratory, and biochemical parameters (including investigational biomarkers) between AEIPF and SIPF patients. The Joanna Briggs Institute Critical Appraisal Checklist was used to assess the risk of bias.
RESULTS
Twenty-nine cross-sectional studies published between 2010 and 2022 were identified (all with a low risk of bias). Of the 32 meta-analysed parameters, significant differences were observed between groups, assessed through standard mean differences or relative ratios, with age, forced vital capacity, vital capacity, carbon monoxide diffusion capacity, total lung capacity, oxygen partial pressure, alveolar-arterial oxygen gradient, P/F ratio, 6 min walk test distance, C-reactive protein, lactate dehydrogenase, white blood cell count, albumin, Krebs von den Lungen 6, surfactant protein D, high mobility group box 1 protein, and interleukin-1β, 6, and 8.
CONCLUSIONS
We identified significant differences between AEIPF and SIPF patients in age and specific parameters of respiratory function, inflammation, and epithelial lung damage. Prospective studies are warranted to determine the capacity of these parameters to predict AEIPF more accurately (PROSPERO registration number: CRD42022356640).
Topics: Humans; Cross-Sectional Studies; Disease Progression; Idiopathic Pulmonary Fibrosis; Risk Factors; Oxygen
PubMed: 37137756
DOI: 10.1016/j.arcmed.2023.04.002 -
The Cochrane Database of Systematic... Apr 2022Chronic rhinosinusitis frequently occurs in people with cystic fibrosis. Several medical interventions are available for treating chronic rhinosinusitis in people with... (Review)
Review
BACKGROUND
Chronic rhinosinusitis frequently occurs in people with cystic fibrosis. Several medical interventions are available for treating chronic rhinosinusitis in people with cystic fibrosis; for example, different concentrations of nasal saline irrigations, topical or oral corticosteroids, antibiotics - including nebulized antibiotics - dornase alfa and modulators of the cystic fibrosis transmembrane conductance regulator (CFTR) (such as lumacaftor, ivacaftor or tezacaftor). However, the efficacy of these interventions is unclear. This is an update of a previously published review.
OBJECTIVES
The objective of this review is to compare the effects of different medical interventions in people diagnosed with cystic fibrosis and chronic rhinosinusitis.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and hand searching of journals and conference abstract books. Date of last search of trials register: 09 September 2021. We also searched ongoing trials databases, other medical databases and the reference lists of relevant articles and reviews. Date of latest additional searches: 22 November 2021.
SELECTION CRITERIA
Randomized and quasi-randomized trials of different medical interventions compared to each other or to no intervention or to placebo.
DATA COLLECTION AND ANALYSIS
Two review authors independently assessed trials identified for potential inclusion in the review. We planned to conduct data collection and analysis in accordance with Cochrane methods and to independently rate the quality of the evidence for each outcome using the GRADE guidelines.
MAIN RESULTS
We identified no trials that met the pre-defined inclusion criteria. The most recent searches identified 44 new references, none of which were eligible for inclusion in the current version of this review; 12 studies are listed as excluded and one as ongoing.
AUTHORS' CONCLUSIONS
We identified no eligible trials assessing the medical interventions in people with cystic fibrosis and chronic rhinosinusitis. High-quality trials are needed which should assess the efficacy of different treatment options detailed above for managing chronic rhinosinusitis, preventing pulmonary exacerbations and improving quality of life in people with cystic fibrosis.
Topics: Anti-Bacterial Agents; Chronic Disease; Cystic Fibrosis; Humans; Quality of Life; Sinusitis
PubMed: 35390177
DOI: 10.1002/14651858.CD012979.pub3