Journal of Cystic Fibrosis最新文献

{"title":"Association between the childhood opportunity index and healthcare utilization in cystic fibrosis.","authors":"Ashley Han, Matt Hall, Elizabeth Cromwell, Ronald L Gibson, Thida Ong, A Jay Freeman, Clement L Ren, Don B Sanders, Jonathan D Cogen","doi":"10.1016/j.jcf.2026.08.009","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.009","url":null,"abstract":"<p><strong>Background: </strong>Among people with cystic fibrosis (CF), limited data exists describing how area-level socioeconomic factors influence in-hospital, emergency room, and ambulatory surgery healthcare utilization. Using the Childhood Opportunity Index (COI) as an area-level composite measure, we aimed to describe the healthcare utilization patterns among children with CF (CwCF) by COI and determine if COI is associated with increased healthcare utilization.</p><p><strong>Methods: </strong>Retrospective cohort study that utilized the CF Foundation Patient Registry-Pediatric Health Information System dataset. The COI version 3.0 was used. Primary study outcomes included the number of hospital encounters (hospitalizations, emergency room visits, and ambulatory surgeries) between 2009-2022, compared among five COI quintiles. Generalized estimating equations were used to regress hospital encounter on COI quintile with a Poisson distribution and clustered on hospital.</p><p><strong>Results: </strong>8177 CwCF <22 years contributed 65,038 hospital encounters for analysis. Compared to the very high quintile, CwCF in the very low, low, and moderate quintiles had a higher hospitalization risk (very low Relative Risk (RR) 1.31, 95% Confidence Interval (CI) 1.17-1.47; p < 0.001, low RR 1.16, 95%CI 1.04-1.30; p = 0.008, and moderate RR 1.15, 95%CI 1.05-1.25; p = 0.001). In addition, CwCF in the very low group had a higher respiratory-related hospitalization risk (RR 1.60, 95%CI 1.30-1.90; p < 0.01).</p><p><strong>Conclusions: </strong>CwCF in the lower COI quintiles had a higher hospitalization risk. Future research should focus on underlying area-level drivers of hospitalizations to reduce inpatient utilization.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Microbial content versus microbial interaction: the impact of medications on CF airway microbial ecosystems.","authors":"Pok-Man Ho, Rahan Rudland Nazeer, Isabel Askenasy, Robert A Quinn, Martin Welch","doi":"10.1016/j.jcf.2026.08.011","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.011","url":null,"abstract":"<p><strong>Background: </strong>The airways of people with cystic fibrosis (pwCF) are often colonized by a variety of different microbes. Although much effort has been put into cataloguing the impact of medication on the identities and abundances of these microbes, far less has been directed towards examining this from an ecological perspective, i.e., examining how medications affect the network and types of interactions between microbes.</p><p><strong>Methods: </strong>In the current work, we generated an ecological model of the CF airway microbiome and examined how medications affect interactions between co-habiting airway microbiota in six pwCF. Ecological interactions were inferred from a generalized Lotka-Volterra model, and the impact of medications was determined by principal component(s) regression analysis.</p><p><strong>Results: </strong>For the majority of the subjects studied, antimicrobial interventions had relatively little impact on the CF airway microbial ecology, and even appeared to stabilize ecological interactions between the microbiota. However, the microbial ecosystem in some individuals was more sensitive to external perturbations. More surprisingly, we found that some non-antimicrobial medications, and also certain carriers and excipients affect the ecosystem.</p><p><strong>Conclusions: </strong>Medications affect the ecology of the CF airway microbiota. These impacts appear to be very patient-specific. We also note that some nominally non-bioactive ingredients in medications can also potentially impact the CF airway ecosystem. Our data highlight the importance of collecting patient-specific data and in employing suitable computational frameworks for disentangling medication-microbiota interactions in vivo.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148891886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The cystic fibrosis gut microbial dysbiosis index (CF-GMDI): a quantitative measure of gut microbial imbalance in children with cystic fibrosis.","authors":"B L D Uthpala Pushpakumara, Michael J Coffey, Jennifer Hudson, Jessica Halim, Sandra Chuang, Bernadette Prentice, Adam Jaffe, Robert Edwards, Andrew S Day, Mark Oliver, Sarath Ranganathan, Claire Wainwright, Hiran Selvadurai, Josie van Dorst, Chee Y Ooi","doi":"10.1016/j.jcf.2026.08.007","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.007","url":null,"abstract":"<p><strong>Background: </strong>Gut dysbiosis is a hallmark of cystic fibrosis (CF), typically characterised using non-specific diversity metrics and study-specific taxonomic lists, limiting comparability across studies. We aimed to develop a metagenomic, species-level CF Gut Microbial Dysbiosis Index (CF-GMDI) to standardise measurement of gut microbial imbalance in children with CF (cwCF) METHODS: CF-GMDI was derived using stool metagenomic data from the PEARL-CF study (70 cwCF; 67 healthy controls (HC); 0-6 years). Differentially abundant taxa were identified between cwCF and HC using MaAsLin2. The index was calculated as the log₁₀ ratio of the summed relative abundances of taxa enriched in CF vs HC to those depleted in CF vs HC. Reproducibility was assessed in the independent EARTH cohort (56 cwCF; 56 HC; 0-18 years). Responsiveness to therapy was evaluated using publicly available metagenomic data from an Elexacaftor/Tezacaftor/Ivacaftor (ETI) study (39 cwCF; 6-18 years).</p><p><strong>Results: </strong>CF-GMDI was significantly higher in cwCF than HC (p < 0.001), inversely correlated with species richness (ρ = -0.74, p < 0.001), and higher in pancreatic-insufficient vs pancreatic-sufficient cwCF in the PEARL-CF cohort (p = 0.01). Key ecological and clinical associations were replicated in the EARTH cohort. In the ETI study, CF-GMDI decreased significantly at 6 and 12 months post-treatment, whereas alpha diversity remained unchanged.</p><p><strong>Conclusions: </strong>CF-GMDI is a CF-associated metric that captures clinically relevant gut microbiome restructuring not detected by standard diversity measures in cwCF (0-18 years). It differentiates disease and pancreatic status and tracks therapeutic modulation, supporting its use as a novel endpoint in CF intervention studies.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148887679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peripheral muscle dysfunction persists in the era of highly effective CFTR modulators in cystic fibrosis.","authors":"Antoine Bronstein, Elise Zitta, Oussama Saidi, Nadège Goriot-Raynaud, Laurent Mely, Mathieu Gruet","doi":"10.1016/j.jcf.2026.08.008","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.008","url":null,"abstract":"<p><strong>Background: </strong>Peripheral muscle dysfunction is a well-established extrapulmonary manifestation of cystic fibrosis (CF), contributing to exercise intolerance and reduced quality of life. However, its persistence in the era of highly effective CFTR modulator therapy (HEMT) remains unclear.</p><p><strong>Methods: </strong>Fifty-six people with CF (age: 31 ± 13 years, BMI: 22 [20-24] kg·m^-2; FEV₁%: 77 ± 24; 95% treated with elexacaftor/tezacaftor/ivacaftor for 31 ± 11 months) were compared with 46 healthy controls of comparable age and sex distribution. Muscle function was assessed following international recommendations, including quadriceps strength (primary outcome), endurance and thickness, handgrip strength, and squat jump performance. Physical activity was assessed using accelerometry.</p><p><strong>Results: </strong>Compared with controls, people with CF exhibited lower quadriceps strength (203 [161-304] vs. 284 [238-335] N·m, p = 0.001, rrb = 0.35) and endurance (p < 0.001, rrb = 0.47). Quadriceps thickness (p = 0.002, rrb = 0.34) and handgrip strength (p = 0.009, d = 0.48) were also lower. After normalisation to muscle thickness, quadriceps strength remained lower (p = 0.01, rrb = 0.27), whereas habitual physical activity levels did not differ significantly between groups.</p><p><strong>Conclusion: </strong>This is the first large controlled study to comprehensively assess peripheral muscle function in people with CF following long-term HEMT exposure. Despite this prolonged exposure, clinically relevant impairments remained evident across multiple dimensions of muscle function. Altered muscle performance cannot be explained solely by muscle deconditioning and lower muscle size, suggesting intrinsic muscle alterations. These findings support the need for comprehensive assessment of peripheral muscle function and the continued prioritisation of interventions aiming to optimise muscle health.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148865012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-invasive colorectal cancer screening in cystic fibrosis: Promise, pitfalls, and future directions.","authors":"Liron Birimberg-Schwartz, Eitan H Zlotnick, Lior H Katz","doi":"10.1016/j.jcf.2026.08.001","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.001","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prenatal CFTR modulator therapy and fetal meconium ileus in cystic fibrosis: a systematic review and individual patient data meta-analysis.","authors":"Vincenzo G Menditto, Giovanni Pomponio, Nicole Caporelli, Marica Bordicchia, Alessandra Boni, Marco Bruno Luigi Rocchi, Giacomo Menditto, Benedetta Fabrizzi","doi":"10.1016/j.jcf.2026.08.005","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.005","url":null,"abstract":"<p><strong>Background: </strong>Fetal meconium ileus (MI) is a clinically relevant manifestation of cystic fibrosis (CF). We conducted a systematic review with meta-analysis to evaluate the efficacy of prenatal CFTRm therapy, administered to carrier mothers or mothers with CF, for the prevention or treatment of fetal MI. The primary outcome was the occurrence of MI requiring surgical intervention.</p><p><strong>Methods: </strong>A systematic search was conducted in PubMed/MEDLINE, Embase, SCOPUS and the Cochrane Library. In-vitro and animal studies were excluded. Reporting quality and the risk of bias of the cohort studies was assessed using the NOS Scale and the CARE checklist, as appropriate, and the ROBINS-I V2, respectively. An individual patient data meta-analysis and meta-regression were performed.</p><p><strong>Results: </strong>We included 18 studies. Among 42 fetuses antenatally diagnosed with MI and exposed to CFTRm, 12% (95% CI, 6%-25%) required surgery after birth. Comparison with a historical cohort suggested a possible 59.5% absolute reduction in the risk of surgery (95% CI, 44%-69%). None of the nine asymptomatic fetuses exposed to treatment developed MI. On meta-regression, the diagnosis of MI in second trimester was associated with lower odds of surgery (OR 0.05, 0.01-0.50; p = 0.011), whereas maternal treatment duration shorter than 5 weeks was associated with increased odds (OR 15.15, 1.41-162.73; p = 0.025).</p><p><strong>Discussion: </strong>Prenatal exposure to CFTRm appears to be effective in treating or preventing MI in fetuses with CF. However, the evidence is limited to case reports or small observational studies and prospective controlled studies are needed.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Once-daily hypertonic saline inhalation and airway clearance techniques in children with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor: a prospective multicentre study.","authors":"Marcus Svedberg, Christina Krantz, Henrik Imberg","doi":"10.1016/j.jcf.2026.08.006","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.006","url":null,"abstract":"<p><strong>Background: </strong>Highly effective CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) have improved outcomes in cystic fibrosis (CF), with many children now experiencing minimal respiratory symptoms. This raises questions about the need for continued hypertonic saline inhalation and airway clearance techniques (ACTs).</p><p><strong>Methods: </strong>In this prospective multicentre observational study, children with CF aged 6-17 years treated with ETI for ≥9 months reduced the prescribed frequency of hypertonic saline inhalation and ACTs from twice daily to once daily over 12 months. The primary outcome was change in lung clearance index (LCI). Secondary outcomes included spirometry, airway microbiology, antibiotic use, and respiratory symptoms. Analyses of longitudinal outcomes were performed using mixed-effects models RESULTS: Forty-six children were included from two Swedish CF centres. Mean (SD) LCI and FEV₁% predicted at baseline were 6.6 (1.1) and 97.2 (6.1), respectively. During the 12 months following treatment reduction, LCI showed a small, non-significant improvement of -0.32 units (95% CI -0.67 to 0.03; P = 0.075), while FEV₁% predicted remained stable (mean change 0.02%-points, 95% CI -2.74 to 2.77; P = 0.99). Respiratory symptoms were stable (mean change -2.9 points, 95% CI -9.8 to 4.1; P = 0.41). Antibiotic use decreased from 47 to 29 days per person-year, corresponding to a 39% reduction (95% CI 25%-50%; P < 0.001), with no clinically relevant changes in airway microbiology.</p><p><strong>Conclusions: </strong>Reduction to once-daily hypertonic saline inhalation and ACTs in children with CF treated with ETI was not associated with clinical deterioration over 12 months and was accompanied by reduced antibiotic use.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Variants of varying clinical consequence (VVCCs) are associated with differing penetrance and expressivity in children with CRMS/CFSPID.","authors":"MacKenzie L Wyatt, Karen S Raraigh, Anna V Faino, Meghan E McGarry","doi":"10.1016/j.jcf.2026.08.004","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.004","url":null,"abstract":"<p><strong>Background: </strong>An unintended consequence of cystic fibrosis (CF) newborn bloodspot screening (NBS) is the identification of infants who have an abnormal NBS but do not meet the clinical diagnostic criteria for CF, known as CFTR-related metabolic syndrome (CRMS) in the US or CF screen positive, inconclusive diagnosis (CFSPID) in other countries. Many children with CRMS/CFSPID harbor at least one CFTR variant of varying clinical consequences (VVCC), a category associated with varying penetrance for CF. We sought to determine whether particular VVCCs are associated with differing risks of diagnostic transition (or conversion) from CRMS/CFSPID to CF.</p><p><strong>Methods: </strong>We performed a meta-analysis of published CRMS/CFSPID cases and examined CF penetrance (i.e. conversion from CRMS/CFSPID to CF) associated with specific VVCCs. We examined expressivity by evaluating the symptoms associated with CF conversion for each individual VVCC and by overall group.</p><p><strong>Results: </strong>There were 606 children with CRMS/CFSPID in 25 manuscripts. Of 268 children with CRMS/CFSPID harboring one VVCC and one CF-causing variant, 78 (28%) converted to CF. There was wide variability in VVCC penetrance; some variants (5T;TG13, Q1476X, P5L) were associated with a high risk of CF conversion (≥50%) whereas others (F1052V, R117H;7T, D1270N) were associated with a low risk. Expressivity associated with VVCCs varied substantially, with elevated sweat chloride concentration most commonly observed in CF conversions.</p><p><strong>Conclusions: </strong>There is significant variability in the penetrance and expressivity associated with VVCCs. Results can inform clinicians and families and guide policy regarding inclusion of VVCCs in CF NBS.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence, prevalence, and survival of persons living with advanced cystic fibrosis lung disease in the United States.","authors":"Jonathan V Todd, Kathleen J Ramos, Erin Tallarico, Abigail Thaxton, Chris Beres, Whitney Brown, Albert Faro, Elizabeth A Cromwell","doi":"10.1016/j.jcf.2026.08.003","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.003","url":null,"abstract":"<p><strong>Background: </strong>Persons with advanced cystic fibrosis lung disease (ACFLD) are a subpopulation with specific care needs and unique clinical challenges. In 2019, the Cystic Fibrosis Foundation Patient Registry (CFFPR) introduced new case report forms related to ACFLD. The objective of this study was to estimate the prevalence and incidence of ACFLD 2015-2023.</p><p><strong>Methods: </strong>ACFLD cases were identified by summarizing individuals with report of supplemental oxygen use, pneumothorax, massive hemoptysis, ppFEV₁ < 40, and transplant status. We estimated the incidence of ALD annually, and quantified prevalence accounting for deaths and transplants, compared to case identification using the additional ACFLD case report forms. Kaplan-Meier estimates were used to calculate survival proportions by calendar period and ACFLD qualifying condition.</p><p><strong>Results: </strong>There were 19,117 people who met inclusion criteria; 3,590 incident cases were identified between 2015-2018 and 2,161 incident cases between 2019-2023. Prevalence of ACFLD fell from 6.1% in 2015 to 1.5% in 2023. There were 745 deaths among people meeting ACFLD criteria in 2015-2018 and 158 deaths among incident ACFLD cases 2019-2022. People identified as ACFLD with multiple qualifying conditions had worse survival in both time periods.</p><p><strong>Conclusions: </strong>Incidence of ACFLD and risk of death has declined from 2015-2023, but 1- and 2-year survival estimates reveal an ongoing need for attention to risk factors for death without lung transplant in this vulnerable population, as those with both ppFEV₁ <40 and complications at ACFLD onset have the highest risk of death.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of geographical and environmental exposures in respiratory infections among individuals with cystic fibrosis: A systematic review and meta-analysis.","authors":"Brittnee Bryer, Jialu Wang, David Reid, Gail Williams, Darsy Darssan","doi":"10.1016/j.jcf.2026.08.002","DOIUrl":"https://doi.org/10.1016/j.jcf.2026.08.002","url":null,"abstract":"<p><strong>Background & aims: </strong>Chronic, recurrent respiratory infections are a hallmark of Cystic Fibrosis (CF). Previous reviews concluded that environmental and community risk factors likely interact with the respiratory tract in CF. However, these studies do not include a systematic search, quality and risk of bias assessment, or meta-analysis. This study aimed to: (i) systematically identify and summarise evidence on associations between environmental and geographical exposures and respiratory infections in CF from epidemiological studies, (ii) assess methodological quality and risk of bias of identified studies, (iii) synthesise evidence via meta-analyses.</p><p><strong>Methods: </strong>Three electronic databases were searched to identify studies conducted in humans and published in English between 2014 and 2024 inclusive. All stages of study screening, data extraction, quality assessment, and risk of bias assessment were conducted in duplicate by independent reviewers. Meta-analysis was conducted when three or more estimates were available for an exposure.</p><p><strong>Results: </strong>A total of 43 studies were included in this review. Weather season, vapour pressure, water and soil constituents, and air pollution were associated with bacterial respiratory infections but showed mixed associations with fungal and viral infections. Geographical exposures, including treatment centre location and residential location, were associated with both bacterial and fungal infections.</p><p><strong>Conclusion: </strong>Environmental exposures showed stronger associations with bacterial than fungal or viral infections, whereas geographical exposures were linked to both bacterial and fungal pathogens. These patterns suggest external factors act differently across infection types in CF. Acknowledging this may improve clinical risk stratification, but more standardised and geographically diverse research is needed.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148793598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}