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Jornal Brasileiro de Pneumologia :... 2024To evaluate the effect of treatment with the combination of three cystic fibrosis transmembrane conductance regulator (CFTR) modulators-elexacaftor+tezacaftor+ivacaftor... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To evaluate the effect of treatment with the combination of three cystic fibrosis transmembrane conductance regulator (CFTR) modulators-elexacaftor+tezacaftor+ivacaftor (ETI)-on important clinical endpoints in individuals with cystic fibrosis.
METHODS
This was a systematic review and meta-analysis of randomized clinical trials that compared the use of ETI in individuals with CF and at least one F508del allele with that of placebo or with an active comparator such as other combinations of CFTR modulators, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations and the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) methodology. We searched the following databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov from their inception to December 26th, 2022. The risk of bias was assessed using the Cochrane risk-of-bias tool, and the quality of evidence was based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE).
RESULTS
We retrieved 54 studies in the primary search. Of these, 6 met the inclusion criteria and were analyzed (1,127 patients; 577 and 550 in the intervention and control groups, respectively). The meta-analysis revealed that the use of ETI increased FEV1% [risk difference (RD), +10.47%; 95% CI, 6.88-14.06], reduced the number of acute pulmonary exacerbations (RD, -0.16; 95% CI, -0.28 to -0.04), and improved quality of life (RD, +14.93; 95% CI, 9.98-19.89) and BMI (RD, +1.07 kg/m2; 95% CI, 0.90-1.25). Adverse events did not differ between groups (RD, -0.03; 95% CI, -0.08 to 0.01), and none of the studies reported deaths.
CONCLUSIONS
Our findings demonstrate that ETI treatment substantially improves clinically significant, patient-centered outcomes.
Topics: Humans; Alleles; Cystic Fibrosis Transmembrane Conductance Regulator; Cystic Fibrosis; Quality of Life; Pyridines; Indoles; Pyrazoles; Aminophenols; Quinolones; Pyrrolidines; Benzodioxoles
PubMed: 38198345
DOI: 10.36416/1806-3756/e20230187 -
BMC Pediatrics Apr 2024Cystic fibrosis is a chronic genetic disease that can affect the function of the respiratory system. Previous reviews of the effects of respiratory muscle training in... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Cystic fibrosis is a chronic genetic disease that can affect the function of the respiratory system. Previous reviews of the effects of respiratory muscle training in people with cystic fibrosis are uncertain and do not consider the effect of age on disease progression. This systematic review aims to determine the effectiveness of respiratory muscle training in the clinical outcomes of children and adolescents with cystic fibrosis.
METHODS
Up to July 2023, electronic databases and clinical trial registries were searched. Controlled clinical trials comparing respiratory muscle training with sham intervention or no intervention in children and adolescents with cystic fibrosis. The primary outcomes were respiratory muscle strength, respiratory muscle endurance, lung function, and cough. Secondary outcomes included exercise capacity, quality of life and adverse events. Two review authors independently extracted data and assessed study quality using the Cochrane Risk of Bias Tool 2. The certainty of the evidence was assessed according to the GRADE approach. Meta-analyses where possible; otherwise, take a qualitative approach.
RESULTS
Six studies with a total of 151 participants met the inclusion criteria for this review. Two of the six included studies were published in abstract form only, limiting the available information. Four studies were parallel studies and two were cross-over designs. There were significant differences in the methods and quality of the methodology included in the studies. The pooled data showed no difference in respiratory muscle strength, lung function, and exercise capacity between the treatment and control groups. However, subgroup analyses suggest that inspiratory muscle training is beneficial in increasing maximal inspiratory pressure, and qualitative analyses suggest that respiratory muscle training may benefit respiratory muscle endurance without any adverse effects.
CONCLUSIONS
This systematic review and meta-analysis indicate that although the level of evidence indicating the benefits of respiratory muscle training is low, its clinical significance suggests that we further study the methodological quality to determine the effectiveness of training.
TRIAL REGISTRATION
The protocol for this review was recorded in the International Prospective Register of Systematic Reviews (PROSPERO) under registration number CRD42023441829.
Topics: Child; Adolescent; Humans; Cystic Fibrosis; Quality of Life; Breathing Exercises; Chronic Disease; Respiratory Muscles
PubMed: 38622583
DOI: 10.1186/s12887-024-04726-x -
European Respiratory Review : An... Sep 2023Oxygen therapy is prescribed to treat hypoxaemia in people with interstitial lung disease (ILD); however, uptake and adherence remain an ongoing challenge. This... (Review)
Review
BACKGROUND
Oxygen therapy is prescribed to treat hypoxaemia in people with interstitial lung disease (ILD); however, uptake and adherence remain an ongoing challenge. This systematic review aimed to identify the barriers to and facilitators of use of oxygen therapy in people with ILD, caregivers and health professionals.
METHODS
A systematic search for qualitative literature was undertaken using five electronic databases (MEDLINE, CINAHL, Embase, PsycINFO, PubMed). Qualitative analysis identified themes that were mapped to the Theoretical Domains Framework and the Consolidated Framework for Implementation Research and classified as barriers, facilitators or both.
RESULTS
A total of 13 studies were eligible for inclusion. Commonly represented domains were associated with the design of the oxygen delivery system, the associated cost, financing, stigmatisation, the physical environment and the individual needs that acted as barriers to and facilitators of the optimisation of oxygen therapy.
CONCLUSION
Effective implementation of oxygen therapy in ILD requires more robust evidence to strengthen international guidelines, sustainable and equitable funding models, and improved oxygen delivery systems that meet the needs of users. Increased information and support for users will be critical to optimise the uptake and outcomes of this important therapy.
Topics: Humans; Oxygen Inhalation Therapy; Lung Diseases, Interstitial; Environment; Health Personnel; Oxygen
PubMed: 37611946
DOI: 10.1183/16000617.0066-2023 -
Journal of Personalized Medicine Nov 2023Idiopathic pulmonary fibrosis (IPF) is one of the most aggressive forms of interstitial lung diseases (ILDs), marked by an ongoing, chronic fibrotic process within the... (Review)
Review
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is one of the most aggressive forms of interstitial lung diseases (ILDs), marked by an ongoing, chronic fibrotic process within the lung tissue. IPF leads to an irreversible deterioration of lung function, ultimately resulting in an increased mortality rate. Therefore, the focus has shifted towards the biomarkers that might contribute to the early diagnosis, risk assessment, prognosis, and tracking of the treatment progress, including those associated with epithelial injury.
METHODS
We conducted this review through a systematic search of the relevant literature using established databases such as PubMed, Scopus, and Web of Science. Selected articles were assessed, with data extracted and synthesized to provide an overview of the current understanding of the existing biomarkers for IPF.
RESULTS
Signs of epithelial cell damage hold promise as relevant biomarkers for IPF, consequently offering valuable support in its clinical care. Their global and standardized utilization remains limited due to a lack of comprehensive information of their implications in IPF.
CONCLUSIONS
Recognizing the aggressive nature of IPF among interstitial lung diseases and its profound impact on lung function and mortality, the exploration of biomarkers becomes pivotal for early diagnosis, risk assessment, prognostic evaluation, and therapy monitoring.
PubMed: 38003922
DOI: 10.3390/jpm13111607 -
Diagnostics (Basel, Switzerland) Mar 2024While ground-glass opacity, consolidation, and fibrosis in the lungs are some of the hallmarks of acute SAR-CoV-2 infection, it remains unclear whether these pulmonary... (Review)
Review
While ground-glass opacity, consolidation, and fibrosis in the lungs are some of the hallmarks of acute SAR-CoV-2 infection, it remains unclear whether these pulmonary radiological findings would resolve after acute symptoms have subsided. We conducted a systematic review and meta-analysis to evaluate chest computed tomography (CT) abnormalities stratified by COVID-19 disease severity and multiple timepoints post-infection. PubMed/MEDLINE was searched for relevant articles until 23 May 2023. Studies with COVID-19-recovered patients and follow-up chest CT at least 12 months post-infection were included. CT findings were evaluated at short-term (1-6 months) and long-term (12-24 months) follow-ups and by disease severity (severe and non-severe). A generalized linear mixed-effects model with random effects was used to estimate event rates for CT findings. A total of 2517 studies were identified, of which 43 met the inclusion (N = 8858 patients). Fibrotic-like changes had the highest event rate at short-term (0.44 [0.3-0.59]) and long-term (0.38 [0.23-0.56]) follow-ups. A meta-regression showed that over time the event rates decreased for any abnormality (β = -0.137, = 0.002), ground-glass opacities (β = -0.169, < 0.001), increased for honeycombing (β = 0.075, = 0.03), and did not change for fibrotic-like changes, bronchiectasis, reticulation, and interlobular septal thickening ( > 0.05 for all). The severe subgroup had significantly higher rates of any abnormalities ( < 0.001), bronchiectasis ( = 0.02), fibrotic-like changes ( = 0.03), and reticulation ( < 0.001) at long-term follow-ups when compared to the non-severe subgroup. In conclusion, significant CT abnormalities remained up to 2 years post-COVID-19, especially in patients with severe disease. Long-lasting pulmonary abnormalities post-SARS-CoV-2 infection signal a future public health concern, necessitating extended monitoring, rehabilitation, survivor support, vaccination, and ongoing research for targeted therapies.
PubMed: 38535041
DOI: 10.3390/diagnostics14060621 -
Frontiers in Pharmacology 2023At present, there is a lack of effective treatment for pulmonary fibrosis (PF), and a number of studies have confirmed that curcumin (CUR) has a good effect on PF. Is...
At present, there is a lack of effective treatment for pulmonary fibrosis (PF), and a number of studies have confirmed that curcumin (CUR) has a good effect on PF. Is CUR effective in preclinical trials for PF and what is its mechanism of action? Animal reports of PF treated with CUR were searched from Pubmed, Embase, Web of Science and Cochrane Library from 1 January 2000 to 19 April 2023 to compare CUR treatment of PF with a no-intervention model group. A previous registration (nsply registration number: INPLASY202360084) of this review protocol was undertaken. The meta-analysis included 27 publications and 29 studies involving 396 animals. CUR significantly improved the degree of fibrosis, levels of inflammation, and oxidative imbalances in lung tissue in animal models of PF. In terms fibrosis, such as HYP content (SMD = -4.96; 95% CI = -6.05 to -3.87; = 0.000).In terms of inflammatory indicators, such as MPO activity (SMD = -2.12; 95% CI = -4.93 to 0.69; = 0.000). In terms of oxidation index, such as MDA (SMD = -5.63; 95% CI = -9.66 to -1.6; = 0.000). CUR significantly improved the degree of fibrosis, levels of inflammation, and oxidative imbalances in lung tissue in animal models of PF. Due to the quantitative and qualitative limitations of current research, more high-quality studies are needed to verify the above conclusion.
PubMed: 37900163
DOI: 10.3389/fphar.2023.1258885 -
Molecular Genetics & Genomic Medicine Nov 2023Among present reports, the T/G allelic variation at the rs2609255 locus of the family sequence similarity gene 13A (FAM13A) was considerable associated with... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Among present reports, the T/G allelic variation at the rs2609255 locus of the family sequence similarity gene 13A (FAM13A) was considerable associated with susceptibility to interstitial lung diseases (ILDs). In this study, we summarized relevant studies and applied a meta-analysis to explore whether the polymorphism of rs2609255 site of the FAM13A gene can be utilized to predict susceptibility to idiopathic pulmonary fibrosis (IPF) patients or rheumatoid arthritis-associated interstitial lung disease (RA-ILD) or silicosis patients in different populations for the first time.
METHODS
We compared the frequency of G allele on rs2609255 site of FAM13A between the control subjects and IPF or RA-ILD or silicosis patients from different races by using meta-analysis. Nine studies were involved in this meta-analysis, including five IPF studies, two RA-ILD studies, and two silicosis studies, and containing 14 subgroups. We conducted separate meta-analyses for different races.
RESULTS
In all individuals, a substantial link between the G allele of the FAM13A rs2609255 polymorphism and IPF (OR: 1.47, 95% CI: 1.33-1.63, p < 0.00001) was indicated. After dividing by ethnicity, the G allele was illustrated to be considerable correlation with IPF in Asian (OR: 2.63, 95% CI: 1.81-3.81, p < 0.00001) and with RA-ILD individuals (OR: 3.27, 95% CI: 1.26-8.49, p = 0.01). Conversely, there was no correlation with the G allele and IPF in European individuals (OR: 1.27, 95% CI: 0.89-1.83, p = 0.13) or silicosis in Chinese individuals (OR: 1.20, 95% CI: 0.99-1.46, p = 0.07).
CONCLUSION
This is the first meta-analysis that provides evidence that the rs2609255 of FAM13A might increase susceptibility to RA-ILD, and IPF especially in Asian but not in European individuals, and not be correlated with silicosis in Chinese individuals, which indicated the differences in susceptibility to disease by race were noteworthy.
Topics: Humans; Lung Diseases, Interstitial; Idiopathic Pulmonary Fibrosis; Polymorphism, Genetic; Arthritis, Rheumatoid; Silicosis; GTPase-Activating Proteins
PubMed: 37786320
DOI: 10.1002/mgg3.2279 -
BMJ Open Oct 2023Very few studies and limited information are available regarding the mechanism of fibrosis in tuberculosis (TB). This study aimed to identify, describe and synthesise... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Very few studies and limited information are available regarding the mechanism of fibrosis in tuberculosis (TB). This study aimed to identify, describe and synthesise potential biomarkers of the development of tissue fibrosis induced by TB through a systematic method and meta-analysis.
METHODS
A literature search was performed using keywords according to the topic from electronic databases (ScienceDirect and PubMed) and other methods (websites, organisations and citations). Studies that matched predetermined eligibility criteria were included. The quality assessment tool used was the Quality Assessment of Diagnostic Accuracy Score 2, and the data obtained were processed using Review Manager V.5.3.
RESULTS
Of the 305 studies, 7 met the eligibility criteria with a total sample of 365. The results of the meta-analysis showed that the post-TB group of patients with pulmonary parenchymal fibrosis had a higher transforming growth factor (TGF)-β level (6.09) than the control group (1.82), with a 4.27 (95% CI: 0.92 to 7.61) mean difference. Moreover, patients with residual pleural thickening post-TB had a higher mean of TGF-β (0.61) than the control group (0.56), with a 0.05 (95% CI: 0.04 to 0.06) mean difference. Besides TGF-β, our qualitative synthesis also found that matrix metalloproteinase-1 might have a role in forming and developing pulmonary tissue fibrosis, thus, could be used as a predictor marker in the formation of fibrotic lesions in patients with TB. In addition, several other biomarkers were assessed in the included studies, such as tumour necrosis factor-α, interleukin (IL)-4, IL-8, IL-10, plasminogen activator inhibitor-1 and platelet-derived growth factor. However, this study is not intended to examine these biomarkers.
CONCLUSIONS
There were differences in the results of TGF-β levels in patients with fibrotic lesions compared with controls. TGF-β might be a biomarker of fibrotic tissue formation or increased pulmonary tissue fibrosis in post-TB patients. However, further studies are needed on a larger scale.
Topics: Humans; Transforming Growth Factor beta; Tuberculosis; Fibrosis; Pulmonary Fibrosis; Biomarkers; Matrix Metalloproteinases; Transforming Growth Factors
PubMed: 37827747
DOI: 10.1136/bmjopen-2022-070377 -
Immunity, Inflammation and Disease Nov 2023Respiratory disease (RD) is one of the most common diseases characterized by lung dysfunction. Many diagnostic mechanisms have been used to identify the pathogenic... (Review)
Review
AIM
Respiratory disease (RD) is one of the most common diseases characterized by lung dysfunction. Many diagnostic mechanisms have been used to identify the pathogenic agents of responsible for RD. Among these, proteomics emerges as a valuable diagnostic method for pinpointing the specific proteins involved in RD pathogenesis. Therefore, in this study, for the first time, we examined the protein markers involved in the pathogenesis of chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), asthma, bronchiolitis obliterans (BO), and chemical warfare victims exposed to mustard gas, using the proteomics method as a systematic study.
MATERIALS AND METHODS
A systematic search was performed up to September 2023 on several databases, including PubMed, Scopus, ISI Web of Science, and Cochrane. In total, selected 4246 articles were for evaluation according to the criteria. Finally, 119 studies were selected for this systematic review.
RESULTS
A total of 13,806 proteins were identified, 6471 in COPD, 1603 in Asthma, 5638 in IPF, three in BO, and 91 in mustard gas exposed victims. Alterations in the expression of these proteins were observed in the respective diseases. After evaluation, the results showed that 31 proteins were found to be shared among all five diseases.
CONCLUSION
Although these 31 proteins regulate different factors and molecular pathways in all five diseases, they ultimately lead to the regulation of inflammatory pathways. In other words, the expression of some proteins in COPD and mustard-exposed patients increases inflammatory reactions, while in IPF, they cause lung fibrosis. Asthma, causes allergic reactions due to T-cell differentiation toward Th2.
Topics: Humans; Lung; Mustard Gas; Proteomics; Pulmonary Disease, Chronic Obstructive; Asthma; Biomarkers
PubMed: 38018577
DOI: 10.1002/iid3.1090 -
Cureus Feb 2024The aim of this meta-analysis was to scrutinize the prevalence, characteristics, and outcomes of obstructive sleep apnea (OSA) in individuals with ideopathic pulmonary... (Review)
Review
The aim of this meta-analysis was to scrutinize the prevalence, characteristics, and outcomes of obstructive sleep apnea (OSA) in individuals with ideopathic pulmonary fibrosis (IPF). We carried out this systematic review and meta-analysis in accordance with the guidelines outlined by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Statement (PRISMA). Two independent researchers systematically searched major databases, including MEDLINE/PubMed, EMBASE, and the Cochrane Library, from January 1, 2000, until December 31, 2023. We included all studies involving adult patients (age >18 years) with IPF that assessed the prevalence and characteristics of OSA in IPF patients. A total of seven studies involving a pooled sample of 411 patients were included in this meta-analysis. The pooled prevalence of OSA among individuals with IPF was found to be 70% (95% CI: 59 to 82%). Individuals with OSA exhibited a significantly higher mean body mass index (BMI) compared to their counterparts. While individuals with both IPF and OSA exhibited higher scores on the Epworth Sleepiness Scale (ESS) compared to those with IPF alone, the OSA group also showed lower oxygen saturation during sleep in comparison to non-OSA patients. In summary, OSA is a prevalent coexisting condition among individuals with IPF. This presence could worsen the nighttime oxygen saturation. Consequently, there is a need for more extensive studies involving more uniform participant groups.
PubMed: 38516439
DOI: 10.7759/cureus.54562