Journal of Cystic Fibrosis最新文献

{"title":"Pregnancy and neonatal outcome following in utero exposure to CFTR modulators: A multicentre prospective case series.","authors":"Sophie Gautier, Bénédicte Coulm, Marie-Andrée Thompson-Bos, Laurent Chouchana, Camille Audousset, Elisabeth Elefant, Julie Mankikian, Pierre-Regis Burgel, Charles Garabedian, Benoît Marin, Annie-Pierre Jonville-Bera","doi":"10.1016/j.jcf.2025.06.001","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.06.001","url":null,"abstract":"<p><strong>Background: </strong>Clinical data regarding in utero exposure to CFTR modulators (CFTRm) are limited. Our objective was to describe pregnancy outcomes, with particular attention to malformations, and neonatal adverse outcomes among prospective pregnancies exposed to CFTRm, using clinical data from the French Network of Pharmacovigilance Centres and the Teratology Information Service CRAT (Centre de Reference sur les Agents Tératogènes).</p><p><strong>Methods: </strong>An observational multicentre study was performed on the reported CFTRm-exposed pregnancies with an expected delivery date between 2018 and 2023. We described prospective cases, defined as pregnancies for which the outcome was unknown and no adverse prenatal outcome was diagnosed at the time of first contact with the healthcare professional. Major congenital anomalies (MCA) were classified according to criteria of the European Registration of Congenital Anomalies and Twins.</p><p><strong>Results: </strong>Fifty-eight pregnancies were included, mainly exposed to elezacaftor/tezacaftor/ivacaftor throughout pregnancy (87.9 %).There were 53 live births, four spontaneous abortions and one medical abortion (fetus with cystic fibrosis). One atrial septal defect and one acrania/anencephaly were observed, resulting in a MCA prevalence of 3.4 % (IC95 %: 0.4- 11.9). There were three neonatal adverse outcomes without a clearly identified cause: one sudden massive alveolar hemorrhage, one delayed respiratory distress and one delayed transient hypotonia. No cataract was found in children who had an ophthalmological examination (n = 10).</p><p><strong>Conclusions: </strong>This prospective case series - the largest to date - does not suggest a high rate of MCA or neonatal adverse outcomes in CFTRm-exposed pregnancies. Further studies including long-term follow-up of in utero exposed children are needed to confirm these findings.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144340160","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":"Tobacco smoke exposure is associated with diminished longitudinal benefit of elexacaftor/tezacaftor/ivacaftor in cystic fibrosis.","authors":"William T Harris, Elizabeth H Baker, Jennifer S Guimbellot, Joseph M Collaco, Michael S Schechter, Gabriela R Oates","doi":"10.1016/j.jcf.2025.05.002","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.002","url":null,"abstract":"<p><strong>Background: </strong>Tobacco smoke exposure (TSE) is associated with diminished benefit of ivacaftor and tezacaftor/ivacaftor in people with CF (PwCF). This study assessed the association of TSE with clinical benefit from elexacaftor/tezacaftor/ivacaftor (E/T/I), focusing on lung function (ppFEV₁) and pulmonary exacerbations (PEx).</p><p><strong>Methods: </strong>We conducted a retrospective longitudinal analysis of data from the Cystic Fibrosis Foundation Patient Registry (2019-2021) on PwCF aged ≥ 12 years with documented prescription of E/T/I. TSE was defined by self-report of daily or weekly exposure, including living with a smoker or active smoking. Outcome measures were change in ppFEV₁ and PEx after E/T/I initiation. Mixed effects modeling adjusted for sociodemographic and clinical characteristics quantified the interaction between TSE and E/T/I response.</p><p><strong>Results: </strong>In 15,005 PwCF (mean age 27.7 years, 51.9 % F508del homozygous), TSE was associated with a 2.7 % lower ppFEV₁ (69.1 % vs 71.8 %, p < 0.001) before E/T/I therapy. After E/T/I initiation, ppFEV₁ increased in both groups, with a similar peak ΔppFEV₁ of 8.5 % at 6 months. However, after 6 months on E/T/I therapy, TSE was associated with an additional 0.03 % monthly decrease in ppFEV₁, resulting in a 3.4 % lower ppFEV₁ (76.3 % vs 79.7 %, p < 0.001) at the end of 2021. Smoke-exposed PwCF on E/T/I had twice the odds of a PEx compared to unexposed counterparts (OR 2.2, p < 0.001).</p><p><strong>Conclusion: </strong>E/T/I increases ppFEV₁ and decreases PEx in all PwCF, but this benefit is not sustained past 6 months of E/T/I therapy among those with TSE, thus widening long-term disparities in pulmonary outcomes.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144284468","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":"A standard operating procedure for reducing risk from medications prohibited during clinical trials in cystic fibrosis.","authors":"M Le Sayec, G Davies, M D Waller, J C Davies, N J Simmonds, A Jones, R Brugha, C Dawson, P Macedo, G Ruiz, D Hughes, V Shah, C Pao, N Shafi, S Brown, D Watson, L Baker, A Lillis, T Ruffles, Y Needham, S Safavi, I Bokobza, L Holt, J Longmate, S Sketchley, L Cameron, C J Bossley","doi":"10.1016/j.jcf.2025.05.007","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.007","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266362","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":"Fecal microbiota changes in people with cystic fibrosis after 6 months of elexacaftor/tezacaftor/ivacaftor: Findings from the promise study.","authors":"Jennifer T Duong, Hillary S Hayden, Adrian J Verster, Christopher E Pope, Carson Miller, Kelsi Penewit, Stephen J Salipante, Steven M Rowe, George M Solomon, David Nichols, Andrea Kelly, Sarah Jane Schwarzenberg, Steven D Freedman, Lucas R Hoffman","doi":"10.1016/j.jcf.2025.05.006","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.006","url":null,"abstract":"<p><strong>Background: </strong>People with cystic fibrosis (PwCF) often have fecal dysbioses relative to those without CF, characterized by increased pro-inflammatory microbiota and gastrointestinal (GI) inflammation as measured by fecal calprotectin, suggesting that inflammation contributes to CF GI disease. The multicenter observational PROMISE study (NCT04038047) found that calprotectin decreased in PwCF treated with elexacaftor/tezacaftor/ivacaftor (ETI). To better understand the dynamics between fecal dysbiosis and GI inflammation, we characterized the microbiomes of fecal samples from PROMISE and the relationships with calprotectin before, 1-month post, and 6-months post ETI.</p><p><strong>Methods: </strong>Fecal microbiota from participants ≥12 y/o were determined by shotgun metagenomic sequencing with random forest modeling and multivariate linear regression analysis to define relationships between microbiota, calprotectin, and deltaF508 genotype before and after ETI.</p><p><strong>Results: </strong>We analyzed 345 samples from 124 participants. At baseline, we observed community-level differences in the fecal microbiota among participants with abnormal compared to normal calprotectin. With ETI, the relative abundances of 7 bacterial species - Escherichia coli, Staphylococcus aureus, Clostridium scindens, Enterocloster clostridioformis, Clostridium butyricum, Anaeroglobus geminatus, and Ruminococcus gnavus - decreased significantly, correlating with calprotectin decrease. We detected community-level differences in the fecal microbiota based on CFTR genotype and a distinct pattern of microbiota change in F508del homozygous compared to heterozygous participants after ETI.</p><p><strong>Conclusions: </strong>We identified 7 species for which fecal abundances decreased with ETI and correlated with calprotectin decrease, supporting a close relationship between fecal microbiota and inflammation in PwCF. Future work will define these relationships with metabolites and GI symptoms during long-term ETI therapy.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248095","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":"Air travel and cystic fibrosis: An algorithm to assess the risk of In-Flight Hypoxemia.","authors":"A Akerø, E Edvardsen, P L Finstad, B Skrede, M Østerhaug, S Backman, A Edvardsen, O H Skjønsberg","doi":"10.1016/j.jcf.2025.05.004","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.004","url":null,"abstract":"<p><strong>Background: </strong>Air travel may cause significant hypoxemia in patients with cystic fibrosis (CF). A pre-flight algorithm has previously been validated for patients with chronic obstructive pulmonary disease (COPD). No such tools are available for CF patients. The aim of this study was to evaluate if the pre-flight algorithm for COPD patients can be used by CF patients.</p><p><strong>Methods: </strong>In this prospective cross-sectional study, oxygen saturation at sea level (SpO_{2 SL}) and during exercise (SpO_{2 6MWT}) were used to evaluate whether CF patients a) are fit for flight without further assessment, b) require in-flight supplemental oxygen, or c) need further evaluation with hypoxia-altitude simulation test (HAST). HAST was used as reference method, and SpO_{2 HAST} ≤85 % was the criterion for recommending in-flight supplemental oxygen.</p><p><strong>Results: </strong>79 CF patients (41 men), age 38.0 ± 13.4 years, with FEV₁ of 71±23 % of predicted underwent HAST (SpO_{2 HAST} 89.2 ± 4.0 %). Categories for SpO_{2 SL} were >95 % (N = 53), 92-95 % (N = 25), and <92 %, (N = 1), and the cut-off value for SpO_{2 6MWT} was <84 %. HAST showed that CF patients with SpO_{2 SL} >95 % combined with SpO_{2 6MWT} ≥84 % can travel by air without further assessment. Supplemental oxygen is recommended if SpO_{2 SL} is 92-95 % combined with SpO_{2 6MWT} <84 %, or if SpO_{2 SL}<92 %. Otherwise, HAST should be performed. Only 21 patients (27 %) would have needed referral to HAST. The algorithm correctly identified those who needed and those did not need in-flight supplemental oxygen.</p><p><strong>Conclusions: </strong>The algorithm for COPD patients may be used in the pre-flight evaluation of adult CF patients.</p><p><strong>Clinicaltrials: </strong>gov (NCT03843723).</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144225620","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":"Evolution of hepatobiliary involvement in cystic fibrosis children on CFTR modulators.","authors":"Mélanie Auvray, Nolwenn Laborde, Marie Mittaine, Frédérick Barreau, Géraldine Labouret, Léa Roditis, Clara Flumian, Amélie Arrouy, Emmanuel Mas","doi":"10.1016/j.jcf.2025.05.003","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.003","url":null,"abstract":"<p><strong>Background: </strong>There are great changes in cystic fibrosis (CF) disease following introduction of modulator treatments. We aimed to focus on the evolution of hepatobiliary involvement following lumacaftor-ivacaftor (LI) and elexacaftor-tezacaftor-ivacaftor (ETI) initiation.</p><p><strong>Methods: </strong>A retrospective monocentric observational study included 62 CF children treated with CFTR modulators. Data were collected at initiation and after one year of treatment. The primary objective was to describe the evolution of hepatobiliary involvement under CFTR modulator treatment.</p><p><strong>Results: </strong>We identified hepatobiliary involvement before treatment in 37 patients (59.7 %). Fifteen had persistently (during >6 months) elevated liver enzymes (mostly ALT); 17 had abnormal ultrasound including 3 with nodular liver and 3 with pathological elastography; 5 had isolated splenomegaly. Biliary involvement was found in 19 patients. The evolution of hepatic parameters in the overall population was not significant (p > 0.05). However, we observed a trend towards improvement in laboratory values under treatment. There was only one inaugural diagnosis of nodular liver under LI and none under ETI. All patients had preserved liver function (PT>50 %).</p><p><strong>Conclusions: </strong>We did not find a significant improvement or worsening of hepatobiliary involvement under CFTR modulators. We hypothesize that it could be stabilized with these treatments, but this will need confirmation through further studies with longer follow-up and larger cohorts. The other hypothesis proposed is that biological monitoring may not be an accurate assessment of the hepatobiliary response to modulators. This study supports the safety of CFTR modulator use.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144225621","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":"Long-term outcomes in people with CF lacking FEV₁ response to elexacaftor/tezacaftor/ivacaftor therapy.","authors":"Mohamad Hadhud, Dan Halevy, Joel Reiter, Elad Ben Meir, Alex Gileles-Hillel, Ido Sadras, Eitan Kerem, Malena Cohen-Cymberknoh, Oded Breuer","doi":"10.1016/j.jcf.2025.05.005","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.05.005","url":null,"abstract":"<p><strong>Background: </strong>Real-world data demonstrate variability in the response to elexacaftor/tezacaftor/ivacaftor (ETI) treatment among people with CF (pwCF). The aim of this study was to evaluate long-term outcomes in pwCF that had not shown early improvement in the percentage of predicted FEV₁ (ppFEV₁) following ETI treatment.</p><p><strong>Methods: </strong>A single-center prospective study in pwCF who initiated ETI. Patients were categorized as 'early responders' if showing an improvement of at least 10 % in ppFEV₁ within three months of treatment or 'non-early responders' if not. Patients with pretreatment ppFEV₁ of above 99 % predicted were excluded. Respiratory and non-respiratory outcomes 18 to 24 months after ETI initiation were evaluated.</p><p><strong>Results: </strong>A total of 52 pwCF (median age 30 (22-34), 22 (42 %) female) were included, of whom 21 (40 %) were 'non-early responders'. In a multivariable analysis, previous CFTR modulator therapy (p = 0.002), higher pretreatment ppFEV₁ (p = 0.002), and higher pretreatment BMI (p = 0.018), were negatively associated with early change in ppFEV₁. After 18 to 24 months of ETI therapy, ppFEV₁ did not significantly improve in the 'non-early responders' group (p = 0.29). However, rates of ppFEV₁ decline (p < 0.001), BMI (p < 0.005), number of pulmonary exacerbations (p < 0.02), days of intravenous antibiotic treatment (p < 0.01), and chest CT scores (p < 0.05), all significantly improved in both patient groups.</p><p><strong>Conclusions: </strong>This study provides evidence for the long-term clinical benefits of ETI in pwCF lacking an early ppFEV₁ response. The data suggest that a lack of early improvement should not deter clinicians from treatment continuation.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144215977","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":"WS13.01Characterization of two novel F508del-CFTR complex alleles and their impact on drug responsiveness","authors":"V. Tomati ,&nbsp;V. Capurro ,&nbsp;E. Pesce ,&nbsp;C. Pastorino ,&nbsp;F. Cresta ,&nbsp;M. Lena ,&nbsp;A. Dighero ,&nbsp;A. Mantero ,&nbsp;M. Ambroni ,&nbsp;L. Claut ,&nbsp;V. Daccò ,&nbsp;C. Castellani ,&nbsp;R. Bocciardi ,&nbsp;N. Pedemonte","doi":"10.1016/j.jcf.2025.03.564","DOIUrl":"10.1016/j.jcf.2025.03.564","url":null,"abstract":"<div><h3>Objectives</h3><div>In cystic fibrosis (CF), a complete and correct genetic diagnosis is essential for appropriate therapeutic intervention with CFTR modulating drugs. The triple combination of modulators Elexacaftor, Tezacaftor, and Ivacaftor (ETI) successfully overcome molecular defects of the most frequent variant F508del-CFTR. In this scenario, complex alleles of F508del-CFTR can modify both clinical manifestations and the response to treatment. Indeed, each variant may impact on the structure, folding and activity of the CFTR channel [doi: 10.3390/ijms23063175]. In the frame of a large theratyping project of the Italian CF population, we recruited two unrelated CF patients, compound heterozygous for F508del and a minimal function variant, who were referred as having limited or negligible beneficial effects following treatment with ETI.</div></div><div><h3>Methods</h3><div>We performed molecular, functional and biochemical studies on primary cultures of nasal epithelial cells isolated from patients with CF (pwCF) and on immortalized bronchial cells.</div></div><div><h3>Results</h3><div><em>Ex vivo</em> functional studies on patient-derived nasal epithelia confirmed the lack of response to ETI. In-depth molecular characterization highlighted the presence of additional amino acid substitutions <em>in cis</em> with the F508del variant. The first patient carried a rare poorly characterized variant, while the second patient carried a variant not listed in any database. The double mutant CFTR proteins produced by the complex alleles display reduced activity with negligible rescue by CFTR modulators.</div></div><div><h3>Conclusion</h3><div>This study suggests that in pwCF carrying F508del variant and not responding to modulators, the genotype should be re-evaluated in search of possible complex alleles; it also provides new information regarding poorly or not yet described CFTR variants.</div><div>Supported by The Italian Ministry of Health (PNRR-MR1-2023-12378412), CFF (PEDEMO20G0), FFC Ricerca (FFC#10/2021, “Kaftrio nella vita reale – substudy 1”).</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 ","pages":"Page S25"},"PeriodicalIF":5.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144203904","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":"WS10.02Proteomic analysis of nasal lavage fluid and sputum samples in people with cystic fibrosis during 24 months of treatment with elexecaftor/tezacaftor/ivacaftor - The RECOVER study","authors":"S. Nolan ,&nbsp;S. Weldon ,&nbsp;R. Cregan ,&nbsp;B. Elnazir ,&nbsp;M. Williamson ,&nbsp;D. Cox ,&nbsp;B. Linane ,&nbsp;E. McKone ,&nbsp;A. Reid ,&nbsp;J. Davies ,&nbsp;P. McNally ,&nbsp;D. Downey ,&nbsp;C. Taggart","doi":"10.1016/j.jcf.2025.03.547","DOIUrl":"10.1016/j.jcf.2025.03.547","url":null,"abstract":"<div><h3>Objectives</h3><div>Elexecaftor/Tezacaftor/Ivacaftor (ETI) has been shown to modulate the microbial and inflammatory microenvironment in the airways of people with CF. ETI treatment is associated with a markedly reduced sputum production in many people, prompting the need for a surrogate sample type to allow measurement of indices of infection and inflammation. We sought to characterise the changes in the sputum and nasal lavage fluid (NLF) proteome in PwCF in response to ETI and determine the similarity of the responses in both sample types.</div></div><div><h3>Methods</h3><div>124 PwCF over 12y/o were recruited to the study and samples were collected prior to treatment and at subsequent visits up to 2 years. 26 sputum samples and 111 nasal lavage samples underwent proteomic analysis. Significant differences in protein enrichment were calculated and pathway analysis performed.</div></div><div><h3>Results</h3><div>Principal component analysis revealed differences in the proteome of patient sputum before and after treatment, with little clustering in NLF samples. Differential enrichment analysis of sputum prior to and after treatment showed significant pathway enrichment of over 700 proteins - complement cascade proteins (p&lt;0.001) and peptidase inhibitors (p&lt;0.001) with decreases in proteins involved in the regulation of pathogen clearance (p&lt;0.001). Differential analysis of NLF samples in the same fashion revealed 117 significantly different proteins, with pathway enrichment for regulators of proteases and the antimicrobial response (p&lt;0.001). 31 proteins were found to be significantly enriched and 9 decreased in NLF <em>and</em> sputum.</div></div><div><h3>Conclusions</h3><div>ETI treatment is associated with differential expression of proteins involved in immune responses, particularly the complement cascade and proteins implicated in the protease-antiprotease imbalance. Sputum samples displayed very clear distinctions in proteome profiles before and after treatment, unlike NLF samples. NLF and sputum proteomes displayed limited similarity.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 ","pages":"Page S20"},"PeriodicalIF":5.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144203920","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":"Publication Information","authors":"","doi":"10.1016/S1569-1993(25)01494-8","DOIUrl":"10.1016/S1569-1993(25)01494-8","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 ","pages":"Page iii"},"PeriodicalIF":5.4,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144204921","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}