Journal of Cystic Fibrosis最新文献

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The treatment with Elexacaftor/Tezacaftor/Ivacaftor significantly increases serum bilirubin and decreases blood platelets in children and adolescents with cystic fibrosis homozygous or double heterozygous for the F508del CFTR variant. Elexacaftor/Tezacaftor/Ivacaftor治疗F508del CFTR变异纯合子或双杂合子囊性纤维化儿童和青少年患者,可显著提高血清胆红素,降低血小板。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-21 DOI: 10.1016/j.jcf.2025.10.001
Alice Castaldo, Chiara Cimbalo, Cristina Fevola, Valeria Raia, Vito Terlizzi, Monica Gelzo, Angela Sepe, Antonella Tosco
{"title":"The treatment with Elexacaftor/Tezacaftor/Ivacaftor significantly increases serum bilirubin and decreases blood platelets in children and adolescents with cystic fibrosis homozygous or double heterozygous for the F508del CFTR variant.","authors":"Alice Castaldo, Chiara Cimbalo, Cristina Fevola, Valeria Raia, Vito Terlizzi, Monica Gelzo, Angela Sepe, Antonella Tosco","doi":"10.1016/j.jcf.2025.10.001","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.001","url":null,"abstract":"<p><p>The Elexacaftor/Tezacaftor/Ivacaftor (ETI) combination of cystic fibrosis transmembrane regulator modulators is safe and effective even in children with at least one F508del variant. However, cases of liver damage have been reported, and we observed an increase of serum bilirubin and alanine aminotransferase (ALT) in Cystic Fibrosis (CF) adults homozygous or compound heterozygous for the F508del after one year of ETI treatment. In the present study we followed 106 people with CF (pwCF) aged 8-15 years, who were homozygous or compound heterozygous for the F508del variant and treated with ETI, monitoring liver biochemical indices, lipid profile, and circulating cells. Treatment significantly improved sweat chloride, body mass index and forced expiratory volume in 1 s in both pwCF homozygous and compound heterozygous for the F508del variant (p < 0.001). On the other hand, ETI treatment caused a significant increase in total and conjugated bilirubin in both subgroups (p < 0.001). These changes were mostly found after six months of therapy but not continued after one and two years. Furthermore, we observed a significant reduction in the number of blood platelets after ETI treatment. These data suggest to further investigate the effects of ETI therapy on bilirubin metabolism and to search for biomarkers that can predict its increase, whereas we hypothesize that the reduction in platelet count may depend on the reduced systemic inflammation due to ETI treatment.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145345643","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}
引用次数: 0
Nasal irrigation as an alternate method to monitor airway microbiology in cystic fibrosis. 鼻冲洗作为监测囊性纤维化患者气道微生物学的替代方法。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-21 DOI: 10.1016/j.jcf.2025.10.003
Raynuka Lazarus, Cassandra Thompson, Jennifer Bishop, Nicholas Perry, Jackson Foster, Catherine Banks, Peter Middleton, Michael Doumit
{"title":"Nasal irrigation as an alternate method to monitor airway microbiology in cystic fibrosis.","authors":"Raynuka Lazarus, Cassandra Thompson, Jennifer Bishop, Nicholas Perry, Jackson Foster, Catherine Banks, Peter Middleton, Michael Doumit","doi":"10.1016/j.jcf.2025.10.003","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.003","url":null,"abstract":"<p><strong>Background: </strong>The sinus cavity may be an alternative sampling site for microbial pathogens in people with cystic fibrosis. However, the congruence between sinus and cough-based sputum sampling is unknown. This study aimed to determine the diagnostic accuracy of nasal irrigation sampling to detect microbial pathogens present in the lower airway.</p><p><strong>Methods: </strong>People with CF (pwCF) suspected of having chronic rhinosinusitis (CRS) provided an expectorated sputum and a nasal irrigation sample on the same day. Sensitivity and specificity with 95 % confidence intervals were calculated, using the expectorated sputum sample as the reference sample. A non-parametric test of equivalence was used to assess non-inferiority of the nasal irrigation procedure to the standard cough method.</p><p><strong>Results: </strong>103 paired samples were collected. Nasal irrigation had a sensitivity and specificity for Pseudomonas aeruginosa of 84 % (95 % CI, 70.9-91.4 %) and 91 % (95 % CI, 80.1-95.6 %), respectively, and for Staphylococcus aureus of 79 % (95 % CI, 61.6-90.2 %) and 85 % (95 % CI, 75.3-91.5 %), respectively. Nasal irrigation demonstrated poor diagnostic accuracy for detecting fungal pathogens [Sensitivity, 0.4 % (95 % CI, 0.08-2.27); Specificity, 99 % (95 % CI, 93.8-99.8)] CONCLUSIONS: Bacterial pathogens common in pwCF can be isolated from nasal irrigation samples, indicating nasal irrigation as a potential alternative diagnostic tool to sputum samples in those suspected of having CRS. Nasal irrigation was not an accurate method to diagnose lower respiratory tract fungal infections. Further research is needed to determine the diagnosis yield of nasal irrigation sampling in pwCF who are asymptomatic for CRS and in a paediatric CF population.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145345582","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}
引用次数: 0
Impact of glucagon-like-peptide-1 receptor agonist therapy on pulmonary function in people with cystic fibrosis who achieve normal body mass index. 胰高血糖素样肽-1受体激动剂治疗对达到正常体重指数的囊性纤维化患者肺功能的影响
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-17 DOI: 10.1016/j.jcf.2025.10.006
Andrew Horvit, Katie Kaput, Amber Neece, Jessica Abramowitz, Marconi Abreu, Gregory A Ratti, James D Finklea, Raksha Jain, Sasan Mirfakhraee
{"title":"Impact of glucagon-like-peptide-1 receptor agonist therapy on pulmonary function in people with cystic fibrosis who achieve normal body mass index.","authors":"Andrew Horvit, Katie Kaput, Amber Neece, Jessica Abramowitz, Marconi Abreu, Gregory A Ratti, James D Finklea, Raksha Jain, Sasan Mirfakhraee","doi":"10.1016/j.jcf.2025.10.006","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.006","url":null,"abstract":"<p><strong>Background: </strong>Glucagon-like-peptide-1 receptor agonists (GLP-1 RA) and glucose-dependent insulinotropic polypeptide/glucagon-like peptide-1 receptor agonists (GIP/GLP-1 RA) are being explored for use in people with CF (pwCF) and CF-related diabetes. In pwCF who are overweight or obese, GLP-1 RA and GIP/GLP-1 RA may offer additional benefits beyond glycemic control, including potential benefits in lung function. However, there is a paucity of evidence regarding the impact of these medications on pulmonary function in pwCF and whether the associated weight loss adversely affects lung function.</p><p><strong>Methods: </strong>This study is the first to describe the impact of GLP-1 RA and GIP/GLP-1 RA on lung function in pwCF who experienced weight loss and had a normal-range body mass index (BMI) of 18.5-24.9 kg/m<sup>2</sup>. Our cohort consists of 13 pwCF (12 females, 1 male; aged 23-46 years) treated with GLP-1 RA or GIP/GLP-1 RA for a median duration of 16 months (range 3-50 months).</p><p><strong>Results: </strong>Subjects experienced 11.1 kg median weight loss (-15.5 % median weight reduction); median BMI reduced from 26.2 kg/m<sup>2</sup> to 21.7 kg/m<sup>2</sup>. Eleven of thirteen subjects had an increase in FEV1 (median, 10.8 %) and all subjects had an increase in FVC (median, 10.6 %). There was no difference in median number of pulmonary exacerbations between groups.</p><p><strong>Conclusions: </strong>Future multi-center studies are needed to investigate the efficacy and tolerability of GLP-1 RA therapy in pwCF. If these agents are shown to be safe and effective in pwCF with normal-range BMI, then current BMI targets in pwCF may need to be revisited.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318282","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}
引用次数: 0
Global prevalence of CFTR variants with respect to their responsiveness to elexacaftor-tezacaftor-ivacaftor. CFTR变异的全球流行程度与它们对基因因子-基因因子-基因因子的响应性有关。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-17 DOI: 10.1016/j.jcf.2025.10.007
P-R Burgel, A Orenti, E Cromwell, M Macek, H H Gutierrez, B Karadag, A Faro, J G van Rens, L Naehrlich, E Bakkeheim, S B Carr, A Lindblad, A Zolin, E Lammertyn, R Ruseckaite, M Zampoli, C A Byrnes, Lvrf da Silva-Filho, A Elbert, S Y Cheng, A L Stephenson
{"title":"Global prevalence of CFTR variants with respect to their responsiveness to elexacaftor-tezacaftor-ivacaftor.","authors":"P-R Burgel, A Orenti, E Cromwell, M Macek, H H Gutierrez, B Karadag, A Faro, J G van Rens, L Naehrlich, E Bakkeheim, S B Carr, A Lindblad, A Zolin, E Lammertyn, R Ruseckaite, M Zampoli, C A Byrnes, Lvrf da Silva-Filho, A Elbert, S Y Cheng, A L Stephenson","doi":"10.1016/j.jcf.2025.10.007","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.007","url":null,"abstract":"<p><strong>Background: </strong>Elexacaftor-tezacaftor-ivacaftor (ETI) is mostly approved in people with cystic fibrosis (pwCF) with a p.Phe508del CFTR variant. The US Food and Drug Administration (FDA) approved ETI for an additional 177 rare CFTR variants and studies identified 7 non-FDA approved variants also responsive to ETI. The global impact of expanding the ETI label to rare responsive CFTR variants on the number of eligible pwCF is unknown.</p><p><strong>Methods: </strong>Data were obtained from CF registries and individual CF clinics in countries without registries. Individuals were classified according to five mutually-exclusive categories (1) at least one p.Phe508del variant (2) at least one of the 177 FDA-approved variants (3) at least one non-FDA-approved variant responsive to ETI (4) two variants resulting in no CFTR protein (5) all other variants. The first 3 groups were considered responsive to ETI, group 4 was nonresponsive and group 5 of undetermined responsiveness.</p><p><strong>Results: </strong>Data were obtained from 95,838 pwCF living in 69 countries: 78,566 (82.0 %) had at least one p.Phe508del, 6175 (6.4 %) at least one FDA-approved variants, and 2981 (3.1 %) at least one non-FDA-approved responsive variants. Two variants resulting in no CFTR protein were found in 3574 (3.7 %) and other variant combinations in 4490 (4.7 %). The prevalence of p.Phe508del ranged from 7 % to 98 % in individual countries and expanding the eligibility to responsive non-p.Phe508del variants resulted in greatest eligibility increase in countries with low p.Phe508del prevalence.</p><p><strong>Conclusion: </strong>Expanding the label of ETI to rare responsive CFTR variants will make at least 91.5 % of pwCF eligible to this disease-modifying therapy.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318243","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}
引用次数: 0
Limited impact of Elexacaftor/Tezacaftor/Ivacaftor on CPET outcomes in an Italian cohort of people with Cystic Fibrosis: reinforcing the essential role of exercise training. elexaftor /Tezacaftor/Ivacaftor对意大利囊性纤维化患者CPET结果的有限影响:强化运动训练的重要作用
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-16 DOI: 10.1016/j.jcf.2025.10.005
Mariangela Retucci, Andrea Gramegna, Simone Gambazza, Martina Santambrogio, Gianmarco Putti, Alessandra Mariani, Maria Chiara Russo, Marco Vicenzi, Valeria Daccò, Francesco Blasi
{"title":"Limited impact of Elexacaftor/Tezacaftor/Ivacaftor on CPET outcomes in an Italian cohort of people with Cystic Fibrosis: reinforcing the essential role of exercise training.","authors":"Mariangela Retucci, Andrea Gramegna, Simone Gambazza, Martina Santambrogio, Gianmarco Putti, Alessandra Mariani, Maria Chiara Russo, Marco Vicenzi, Valeria Daccò, Francesco Blasi","doi":"10.1016/j.jcf.2025.10.005","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.005","url":null,"abstract":"<p><strong>Background: </strong>Elexacaftor/Tezacaftor/Ivacaftor (ETI) is changing life quality and expectancy of people with Cystic Fibrosis (pwCF). Exercise has a pivotal role in improving health of pwCF and proved to be a reliable indicator of health status. This study aims at describing the effects of 6 months of ETI therapy on pwCF exercise-related health and giving insight on the conditions predisposing to better treatment response.</p><p><strong>Methods: </strong>In this observational prospective multicentric study were consecutively enrolled pwCF aged ≥16 who started ETI between May 2021 and April 2022 at Milan Children and Adult CF centres. Exercise performance at Cardiopulmonary Exercise Test (CPET), indicated by maximum workload (Wmax), and other functional outcomes were assessed before starting and after 6 months of ETI.</p><p><strong>Results: </strong>101 pwCF were enrolled in the study, mean age 28.4 (8.7) years, 38.6 % females. 20.8 % were affected by CF-related diabetes (CFRD) and 46.5 % had a previous exposure to CFTR modulators. Wmax significantly improved across timepoints (151.0 [38.6] W to 156.6 [41.1] W, p = 0.008), baseline characteristics associated with such improvement were being more physically active, male sex, CFRD, higher lung clearance index, baseline exercise limitation. No significant change in peak oxygen consumption was observed, while patients experienced an overall improvement in functional outcomes. Fitted regression model showed male subjects and those affected by CFRD are expected to have better exercise performance.</p><p><strong>Conclusion: </strong>While participants who started ETI treatment experienced some improvement in exercise capacity, the gains were limited. Therefore, integrating physical exercise training remains an essential and impactful strategy to enhance physical fitness.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313036","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}
引用次数: 0
Stool and symptom testing in ColoREctal Evaluation for Neoplasia in Cystic Fibrosis (SCREEN-CF). 粪便和症状检测在结肠直肠囊性纤维化肿瘤评估中的应用(SCREEN-CF)。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-16 DOI: 10.1016/j.jcf.2025.09.008
Nicole A Taylor, Sheila Sivam, Josie van Dorst, Michael J Coffey, Simone Visser, Paul Haber, Anastasia Volovets, Chee Y Ooi
{"title":"Stool and symptom testing in ColoREctal Evaluation for Neoplasia in Cystic Fibrosis (SCREEN-CF).","authors":"Nicole A Taylor, Sheila Sivam, Josie van Dorst, Michael J Coffey, Simone Visser, Paul Haber, Anastasia Volovets, Chee Y Ooi","doi":"10.1016/j.jcf.2025.09.008","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.09.008","url":null,"abstract":"<p><strong>Background: </strong>People with cystic fibrosis (pwCF) have increased colorectal cancer (CRC) risk. Colonoscopy is recommended, yet CF comorbidities increase complexity and risk.</p><p><strong>Methods: </strong>We conducted a prospective, observational study of pwCF meeting colonoscopy screening guidelines at an Australian centre (2019 - 2023). Immunochemical faecal occult blood test (iFOBT), faecal calprotectin (FC), and faecal tumour pyruvate kinase isoenzyme type M2 (TuM2-PK) were evaluated for detecting adenomatous polyps and malignant ileocolonic lesions in pwCF. Stools were collected within 3 months of colonoscopy. Diagnostic performance and optimal cut-offs were calculated.</p><p><strong>Results: </strong>Among 49 participants [mean (SD) age 47.8 (8.2) years; 53 % female], 12 (24.5 %) were post-solid organ transplant, 10 (20.4 %) had > 3 months of triple modulator therapy at stool testing, 12 (24.5 %) had adenomatous polyps and 2 (4 %) had ileocolonic malignancy. Malignancies were in non-transplanted individuals, in the terminal ileum (age 43) and hepatic flexure/ascending colon (age 48). Higher BMI (>23.5 kg/m²) was associated with abnormal colonoscopy (p = 0.03). iFOBT, FC and TuM2PK demonstrated excellent predictive performance for malignancy (AUC 0.93, 1.00, 0.83; all p < 0.05). Only FC had acceptable predictive performance for pre-malignant lesions (AUC 0.73; p = 0.008). For adenomatous polyps, FC ≤100 µg/g achieved a sensitivity of 91.7 % and an NPV of 95.5 %. For ileocolonic malignancy, FC ≥1000 µg/g showed 100 % sensitivity and specificity (p = 0.0009).</p><p><strong>Conclusion: </strong>CRC screening in pwCF is critical given the high prevalence of neoplasia. Alternative non-invasive screening may support risk stratification among individuals with comorbidities, or reluctance, though performance could be influenced by CFTR modulator therapy.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313039","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}
引用次数: 0
Economic impact of elexacaftor/tezacaftor/ivacaftor on healthcare expenditure in Canada. 医疗保健行业/医疗保健行业/医疗保健行业对加拿大医疗支出的经济影响。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-14 DOI: 10.1016/j.jcf.2025.10.004
Stephen E Congly, Ranjani Somayaji, Michael D Parkins, Christina S Thornton
{"title":"Economic impact of elexacaftor/tezacaftor/ivacaftor on healthcare expenditure in Canada.","authors":"Stephen E Congly, Ranjani Somayaji, Michael D Parkins, Christina S Thornton","doi":"10.1016/j.jcf.2025.10.004","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.004","url":null,"abstract":"<p><p>The introduction of elexacaftor/tezacaftor/ivacaftor (ETI) has led to improved outcomes and survival in patients living with cystic fibrosis (PwCF) although imposes a substantial economic burden. Despite the reduced healthcare utilization that follows ETI initiation, the economic impact on healthcare spending is not well understood. To try and better understand this, the estimated economic impact on healthcare spending of ETI was calculated in Canada. A treatment naïve cohort of PwCF receiving their first ETI prescription during the 2021-2022 fiscal year from 7 provinces had their healthcare utilization and costs collected one year prior and one year following the initiation of ETI for each patient. Data available included physician visits, emergency department presentations, hospitalizations, drug utilization and laboratory and other diagnostic charges. In the year prior to the first ETI prescription, there was an estimated direct health care cost of $17.6 million CDN. The spending decreased significantly in the year post ETI by $6.9 million with the majority attributed to a 75% reduction in hospitalization-associated costs. When the list price of ETI is accounted for, up to an additional $203 million was spent in the first year after ETI. Irrespective of improvements in life quality brought about by ETI, a price of approximately $10,000/year would be required for it to be cost neutral.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145300949","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}
引用次数: 0
Lung transplant for CF: Low lung bacterial burden and immune mediators in year one associate with clad development. CF肺移植:低肺细菌负荷和免疫介质在第一年与包膜发育有关。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-09 DOI: 10.1016/j.jcf.2025.10.002
Samantha A Whiteside, John E McGinniss, Rebecca A Deek, Carter Merenstein, Noel Britton, Aurea Simon-Soro, Michelle Oyster, Laurel Kalman, Melanie C Brown, Jevon Graham-Wooten, John F McDyer, Pali Shah, Franco D'Alessio, Edward Cantu, Emily S Clausen, Hongzhe Li, Joshua M Diamond, Frederic D Bushman, Jason D Christie, Ronald G Collman
{"title":"Lung transplant for CF: Low lung bacterial burden and immune mediators in year one associate with clad development.","authors":"Samantha A Whiteside, John E McGinniss, Rebecca A Deek, Carter Merenstein, Noel Britton, Aurea Simon-Soro, Michelle Oyster, Laurel Kalman, Melanie C Brown, Jevon Graham-Wooten, John F McDyer, Pali Shah, Franco D'Alessio, Edward Cantu, Emily S Clausen, Hongzhe Li, Joshua M Diamond, Frederic D Bushman, Jason D Christie, Ronald G Collman","doi":"10.1016/j.jcf.2025.10.002","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.10.002","url":null,"abstract":"<p><strong>Background: </strong>Lung transplantation is commonly required for advanced lung disease in cystic fibrosis (CF). Long-term lung allograft survival is limited primarily by chronic lung allograft dysfunction (CLAD), and microbial factors have been implicated in CLAD development. However studies have not specifically investigated CF patients despite the unique microbe-rich nature of the CF respiratory tract. We investigated whether early post-transplantation lung microbiome features associate with CLAD development.</p><p><strong>Methods: </strong>We investigated a longitudinal cohort of 23 CF patients undergoing lung transplantation. Lung lavage was collected from donor lungs, and from recipient allografts serially during the first year post-transplantation. Patients were followed for a median of 4.9 years. This was complemented by a case-control study of 8 CF patients sampled at incident CLAD along with non-CLAD CF transplant controls. Lung bacteria were enumerated by 16S rRNA gene sequencing and quantified by qPCR, and immune mediators investigated by multiplex assay.</p><p><strong>Results: </strong>Cohort patients who developed CLAD had lower lung bacterial burden, lower relative abundances of classic CF lung microbiota, and lower mediator levels during the first-year post-transplantation than those remaining CLAD-free. In contrast, incident CLAD showed elevated lung immune mediators but no microbiome differences.</p><p><strong>Conclusions: </strong>Low lung bacterial content and immune mediators during the first year post-transplantation for CF associate CLAD, whereas CLAD onset is characterized by elevated immune mediators but no lung microbiome differences. Whether airway bacteria early after transplantation for CF may protect against CLAD or serve as a biomarker merits further study.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145274817","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}
引用次数: 0
Sexual health experiences and care utilization of males with cystic fibrosis. 男性囊性纤维化患者的性健康经历及护理利用。
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-06 DOI: 10.1016/j.jcf.2025.09.010
Traci M Kazmerski, Olivia M Stransky, Catherine E Wright, Ishaan Jathal, Asher Prangley, Vin Tangpricha, Raksha Jain, Sigrid Ladores Barrett, Kara S Hughan, Natalie E West, Jennifer L Taylor-Cousar, Gregory S Sawicki
{"title":"Sexual health experiences and care utilization of males with cystic fibrosis.","authors":"Traci M Kazmerski, Olivia M Stransky, Catherine E Wright, Ishaan Jathal, Asher Prangley, Vin Tangpricha, Raksha Jain, Sigrid Ladores Barrett, Kara S Hughan, Natalie E West, Jennifer L Taylor-Cousar, Gregory S Sawicki","doi":"10.1016/j.jcf.2025.09.010","DOIUrl":"https://doi.org/10.1016/j.jcf.2025.09.010","url":null,"abstract":"<p><strong>Background: </strong>Males with cystic fibrosis (MwCF) face many sexual health concerns that have been understudied. We compared sexual health experiences and care utilization of MwCF to the general population and defined CF-related considerations and care preferences.</p><p><strong>Methods: </strong>We surveyed MwCF aged ≥15 years and compared results to the United States National Survey of Family Growth (NSFG;n = 5206 15-49-year-old males). We used descriptive statistics and adjusted linear and logistic regression analyses for comparisons.</p><p><strong>Results: </strong>A total of 532 MwCF (mean age 35.3 years) participated. Over 75 % of MwCF reported never using a condom. Among those sexually active with ≥2 partners in the prior year, 36 % of 15-49-year-old MwCF reported never using condoms vs. 16 % of NSFG males (p < 0.001). Thirty-two percent of MwCF reported having ever been tested for sexually transmitted infections(STIs). Among MwCF, 8 % had ever received information from a healthcare provider about HIV/AIDS, 14 % about other STIs, and 18 % about condom use. MwCF reported an average pubertal onset of 13.2 ± 1.7 years with the majority reporting undergoing puberty at the same time or earlier than their peers. MwCF reported minimal hypogonadism symptoms; 36 % ever had their testosterone measured and 10 % were diagnosed with hypogonadism. Eighty-two percent of MwCF considered their CF team their main health provider and 45 % reported no primary care provider.</p><p><strong>Conclusion: </strong>MwCF report suboptimal STI prevention and counseling compared to males in the general population, highlighting an urgent need to encourage sexual health counseling and care in the CF model and partnerships with primary care providers and other specialists.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":6.0,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145244217","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}
引用次数: 0
71 The CF home sputum-induction trial (CF-HomeSpIT): Primary outcome data for early morning sputum-induction at home compared to same- day sputum-induction in clinic CF家庭诱导痰试验(CF- homespit):清晨在家诱导痰与当日临床诱导痰的主要结局数据
IF 6 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-10-01 DOI: 10.1016/S1569-1993(25)01690-X
J. Forton, K. Ronchetti, J. Tame, H. Morgan, S. Mitchell, R. Dhillon, A. Aseeri, L. Thia, E. Mahenthiralingam
{"title":"71 The CF home sputum-induction trial (CF-HomeSpIT): Primary outcome data for early morning sputum-induction at home compared to same- day sputum-induction in clinic","authors":"J. Forton,&nbsp;K. Ronchetti,&nbsp;J. Tame,&nbsp;H. Morgan,&nbsp;S. Mitchell,&nbsp;R. Dhillon,&nbsp;A. Aseeri,&nbsp;L. Thia,&nbsp;E. Mahenthiralingam","doi":"10.1016/S1569-1993(25)01690-X","DOIUrl":"10.1016/S1569-1993(25)01690-X","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 ","pages":"Pages S36a-S37"},"PeriodicalIF":6.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145189689","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}
引用次数: 0
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