Journal of Cystic Fibrosis最新文献_第2页

Factors associated with prescription of elexacaftor/tezacaftor/ivacaftor among people with cystic fibrosis aged 12 years or older with at least one F508del allele. 在至少有一个 F508del 等位基因的 12 岁或以上囊性纤维化患者中开具 elexacaftor/tezacaftor/ivacaftor 处方的相关因素。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-28 DOI: 10.1016/j.jcf.2024.10.006

Georgene E Hergenroeder, Jonathan V Todd, Josh S Ostrenga, Christopher H Goss, Raksha Jain, Wayne Morgan, Gregory S Sawicki, Michael S Schechter, Elizabeth A Cromwell, Clement L Ren

{"title":"Factors associated with prescription of elexacaftor/tezacaftor/ivacaftor among people with cystic fibrosis aged 12 years or older with at least one F508del allele.","authors":"Georgene E Hergenroeder, Jonathan V Todd, Josh S Ostrenga, Christopher H Goss, Raksha Jain, Wayne Morgan, Gregory S Sawicki, Michael S Schechter, Elizabeth A Cromwell, Clement L Ren","doi":"10.1016/j.jcf.2024.10.006","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.10.006","url":null,"abstract":"Background: This study aims to characterize the uptake of elexacaftor/tezacaftor/ivacaftor (ETI) following Food and Drug Administration (FDA) approval in October 2019.Methods: People with cystic fibrosis (PwCF) ≥12 years enrolled in the CF Foundation Patient Registry (CFFPR) from 2019-2022 with at least one copy of F508del were included. We calculated summary statistics according to ETI prescription status. We used a Kaplan-Meier estimator to determine median days to ETI prescription to identify differences in prescription uptake by lung function, race, and ethnicity and a Cox proportional hazards model to identify risk factors associated with timing of first ETI prescription.Results: A total of 17,183 people (91 %) were prescribed ETI. The median time to prescription was 121 days (95 % CI: 119, 122), with 75 % prescribed within 311 days (95 % CI: 301, 325). PwCF prescribed ETI were younger, had lower lung function, more pulmonary exacerbations in the prior year, earlier age of diagnosis, and were more likely to have been prescribed another CFTR modulator (if eligible). Public health insurance, ppFEV1 >90, Black race and Hispanic ethnicity were associated with lower hazards (e.g., later) of ETI prescription whereas prior modulator prescription, pancreatic insufficiency, increased exacerbation frequency and prior infections were associated with a higher hazard (earlier) of prescription.Conclusions: While over 90 % of eligible individuals were prescribed ETI within three years, time of first prescription was associated with demographic factors and disease severity. Further research should investigate the reasons for this delay and approaches to reduce time to initiation for ETI and future therapies.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142545741","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

Mental health, behaviour and sleep quality in children 6-11 years before and after elexacaftor/tezacaftor/ivacaftor initiation. 开始使用 elexacaftor/tezacaftor/ivacaftor 前后 6-11 岁儿童的心理健康、行为和睡眠质量。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-25 DOI: 10.1016/j.jcf.2024.10.002

Tonia Douglas, Maddison Deery, Hayley Kimball, Vanessa E Cobham, Sophia Panochini, Paul D Robinson, Claire E Wainwright, Peter D Sly, Tamara Blake

引用次数: 0

Pf bacteriophage is associated with decline in lung function in a longitudinal cohort of patients with cystic fibrosis and Pseudomonas airway infection. 在囊性纤维化和气道假单胞菌感染患者的纵向队列中，噬菌体Pf与肺功能下降有关。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-25 DOI: 10.1016/j.jcf.2024.09.018

Elizabeth B Burgener, Aditi Gupta, Kayo Nakano, Sophia L Gibbs, Maya E Sommers, Arya Khosravi, Michelle S Bach, Colleen Dunn, Jacquelyn Spano, Patrick R Secor, Lu Tian, Paul L Bollyky, Carlos E Milla

{"title":"Pf bacteriophage is associated with decline in lung function in a longitudinal cohort of patients with cystic fibrosis and Pseudomonas airway infection.","authors":"Elizabeth B Burgener, Aditi Gupta, Kayo Nakano, Sophia L Gibbs, Maya E Sommers, Arya Khosravi, Michelle S Bach, Colleen Dunn, Jacquelyn Spano, Patrick R Secor, Lu Tian, Paul L Bollyky, Carlos E Milla","doi":"10.1016/j.jcf.2024.09.018","DOIUrl":"10.1016/j.jcf.2024.09.018","url":null,"abstract":"Background: The Pseudomonas filamentous bacteriophage (Pf) infects Pseudomonas aeruginosa (Pa) and is abundant in the airways of many people with cystic fibrosis (CF) (pwCF). We previously demonstrated that Pf promotes biofilm growth, as well as generates liquid crystals that confer biofilms with adhesivity, viscosity and resistance to clearance. Consistent with these findings, the presence of Pf in sputum from pwCF has been linked to chronic Pa infection and more severe exacerbations in a cross-sectional cohort study.Methods: We examined the relationships between Pf and clinical outcomes in a longitudinal study of pwCF. Sputum Pa and Pf concentrations were measured by qPCR, as well cytokines and active neutrophil elastase by standardized assays. Recorded clinical data, including spirometry and microbiological results, were analyzed for associations with Pf. Finally, lung explants from pwCF in this cohort who underwent lung transplantation were examined for presence of liquid crystals within secretions.Results: In explanted lungs from pwCF with known Pf infection we demonstrate areas of birefringence consistent with liquid crystalline structures within the airways. We find that high concentration of Pf in sputum is associated with accelerated loss of lung function, suggesting a potential role for Pf in the pathogenesis of CF lung disease. We also find Pf to associate with increased airway inflammation and an anti-viral cytokine response.Conclusion: Pf may serve as a prognostic biomarker and potential therapeutic target for Pa infections in CF.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142566485","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

Evidence for secondary ciliary dyskinesia in patients with cystic fibrosis. 囊性纤维化患者继发性睫状肌运动障碍的证据。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-19 DOI: 10.1016/j.jcf.2024.10.003

Romane Bonhiver, Noemie Bricmont, Maud Pirotte, Marc-Antoine Wuidart, Justine Monseur, Lionel Benchimol, Anne-Lise Poirrier, Catherine Moermans, Doriane Calmés, Florence Schleich, Renaud Louis, Marie-Christine Seghaye, Céline Kempeneers

{"title":"Evidence for secondary ciliary dyskinesia in patients with cystic fibrosis.","authors":"Romane Bonhiver, Noemie Bricmont, Maud Pirotte, Marc-Antoine Wuidart, Justine Monseur, Lionel Benchimol, Anne-Lise Poirrier, Catherine Moermans, Doriane Calmés, Florence Schleich, Renaud Louis, Marie-Christine Seghaye, Céline Kempeneers","doi":"10.1016/j.jcf.2024.10.003","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.10.003","url":null,"abstract":"Background: Mucociliary clearance (MCC) impairment can be due to mucus abnormalities or to a ciliary dysfunction, which can be innate, or secondary to infection and/or inflammation. In cystic fibrosis (CF), it is well documented that MCC is impaired due to mucus abnormalities, but little is known concerning ciliary beating. This study aimed to confirm that ciliary dyskinesia is present in CF, and if this might be innate or secondary to the chronic infection and/or inflammation.Methods: Ciliated epithelial samples were obtained by nasal brushing from 51 CF patients, and from 30 healthy subjects. Ciliary beating was evaluated using digital high-speed videomicroscopy at 37 °C, allowing to evaluate ciliary beat frequency (CBF) and the percentage of abnormal beat pattern (CBP); this was repeated after air-liquid interface (ALI) cell culture.Results: Ciliary dyskinesia was higher in CF patients than in healthy subjects, with a lower CBF and a higher percentage of abnormal CBP. Ciliary dyskinesia, already present in childhood, normalized after ALI cell culture. A chronic airway colonization did not worsen ciliary dyskinesia.Conclusions: We showed that, in CF, a ciliary dyskinesia, present from childhood, might contribute to the impaired MCC. Our results also found that the abnormal ciliary beating was not associated with a chronic infection, and resolved after ALI cell culture, suggesting that ciliary dyskinesia in CF is not innate, and might be secondary to chronic inflammation.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466566","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

Elevated liver function tests in infants exposed to elexacaftor-tezacaftor-ivacaftor in utero and while breastfeeding - Case reports. 在子宫内和母乳喂养期间暴露于 elexacaftor-tezacaftor-ivacaftor 的婴儿肝功能检测升高 - 病例报告。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-17 DOI: 10.1016/j.jcf.2024.09.025

Theresa Jane Kolaczkowski, Amanda Bevan, Julian Legg, Jay Self, Mark Allenby

引用次数: 0

Development of metabolic syndrome in people with Cystic Fibrosis one year after exposure to elexacaftor-tezacaftor-ivacaftor. 囊性纤维化患者在使用 elexacaftor-tezacaftor-ivacaftor 一年后出现代谢综合征。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-16 DOI: 10.1016/j.jcf.2024.09.022

Gregory A Ratti, Hannah Smith, Sasan Mirfakhraee, Joan Reisch, Leah Cohen, Raksha Jain, James D Finklea

{"title":"Development of metabolic syndrome in people with Cystic Fibrosis one year after exposure to elexacaftor-tezacaftor-ivacaftor.","authors":"Gregory A Ratti, Hannah Smith, Sasan Mirfakhraee, Joan Reisch, Leah Cohen, Raksha Jain, James D Finklea","doi":"10.1016/j.jcf.2024.09.022","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.09.022","url":null,"abstract":"Background: The constellation of hypertension, truncal obesity, impaired fasting glucose, low high-density lipoprotein, and hypertriglyceridemia is known as metabolic syndrome (MetSyn) and is associated with cardiovascular and other diseases. Elexacaftor-tezacaftor-ivacaftor (ETI) in people with cystic fibrosis (pwCF) is associated with weight gain but effects on cardiovascular risk are unknown. This study sought to investigate ETI exposure and risk for development of MetSyn in pwCF.Methods: A prospective cohort study including pwCF ≥ 18 years old exposed to ETI was performed. All data for calculating MetSyn was collected from the electronic medical record at initiation and 1 year ± 3 months after starting ETI. A total of 152 pwCF exposed to ETI and 34 pwCF never exposed to CF transmembrane conductance regulator modulators were included in the analysis. Changes to hypertension classification was also examined over this period.Results: After 1 year of ETI there was an increase in MetSyn from 13 to 30 pwCF, p < 0.0001. No new cases of MetSyn were seen in the group not exposed to ETI. After 1 year of ETI, more people met criteria for class 1 (BP 130-139/90-99 mm Hg) or class 2 hypertension (BP ≥140/≥90 mm Hg) regardless of prior modulator exposure, p < 0.0001.Conclusions: Exposure to ETI for 1 year resulted in an increased number of cases of MetSyn. There was an increased incidence of hypertension associated with ETI exposure. Additional studies are needed to further examine this trend and to determine if these changes will translate to cardiovascular complications over time.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466564","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

Calibrating sweat chloride levels to CFTR activity via ETI effects on CF subjects with one or two F508DEL mutations. 通过 ETI 对有一个或两个 F508DEL 突变的 CF 受试者的影响，校准汗液氯化物水平和 CFTR 活性。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-14 DOI: 10.1016/j.jcf.2024.09.004

Jeffrey J Wine

{"title":"Calibrating sweat chloride levels to CFTR activity via ETI effects on CF subjects with one or two F508DEL mutations.","authors":"Jeffrey J Wine","doi":"10.1016/j.jcf.2024.09.004","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.09.004","url":null,"abstract":"Background: It is difficult to determine CFTR activity following highly effective CFTR modulator therapies (HEMT). The sweat gland provides two biomarkers of CFTR activity: a linear readout via the β-sweat rate and a logarithmic readout via sweat chloride concentration (SCC). In prior work, different logarithmic functions were generated to calibrate SCC with the percent of healthy control CFTR activity (HCCFTR). Two functions, A and B, were fit to SCC means from healthy controls set = 100 % and CF carriers measured as 50 % HCCFTR. A and B differ in the % HCCFTR activity assigned to SCC for minimal function mutations = 0.01 % for A and 1 % for B.Methods: Here, the functions are evaluated based on retrospective analysis of three multi-center studies of CF subjects with one or two F508del mutations treated with Elexacaftor/Tezacaftor/Ivacaftor (ETI). Predictions of the percent HCCFTR activity for one vs two mutations were compared for the two functions. The expectation is that after ETI treatment, subjects with two responsive mutations will have 2-fold higher HCCFTR activity than subjects with only one. The hypothesis is that the SCCHCCFTR function that most closely fits that expectation provides the more accurate prediction of CFTR activity.Results: In two separate comparisons, function B most accurately predicted a 2-fold (1.9, 2.3-fold) higher level of HCCFTR activity in subjects on ETI with two vs. one responsive mutation. Function A predicted a 4, 5.5-fold higher level.Conclusions: Function B predicts that 60 mmol/L SCC, the cutoff for a CF diagnosis, is associated with 10 % HCCFTR activity. Comparing HEMT effects on subjects with one or two mutations provides an additional tool for calibrating SCC to CFTR activity.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466563","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

Suicidal behaviour and CFTR modulators: A case series and WHO database disproportionality analysis. 自杀行为与 CFTR 调节剂：病例系列和世卫组织数据库比例失调分析。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-14 DOI: 10.1016/j.jcf.2024.09.020

Inès Nidegger, Julie Macey, Marine Ferey, Allison Singier, Marie Tournier, Justine Perino, Francesco Salvo

{"title":"Suicidal behaviour and CFTR modulators: A case series and WHO database disproportionality analysis.","authors":"Inès Nidegger, Julie Macey, Marine Ferey, Allison Singier, Marie Tournier, Justine Perino, Francesco Salvo","doi":"10.1016/j.jcf.2024.09.020","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.09.020","url":null,"abstract":"Background: A highly effective therapy involving elexacaftor, tezacaftor, and ivacaftor (ETI) for cystic fibrosis (CF) patients has recently raised safety concerns regarding potential psychiatric disorders. The manuscript reports cases of suicide attempts in patients receiving ETI and investigates putative causality using the WHO spontaneous reporting database.Methods: First, four cases of suicide attempts/self-injury are described. Second, a disproportionality analysis was conducted using spontaneous reports collected in Vigibase through the standardised MedDRA Query (narrow version) \"Suicide/Self-injury\" and ETI exposure. Reporting Odds Ratio (ROR) was calculated for the main and subgroup (i/suicide attempt, ii/suicidal ideation) analyses. Sensitivity analyses were performed with variations in exposure, to ivacaftor/lumacaftor to assess the intrinsic psychiatric risk of CF patients, and paracetamol as a positive control for suicide attempt and a negative one for suicidal ideation. Exposure to reduced-dose ETI was studied to evaluate the dose-gradient effect.Results: Four cases of suicide attempt/self-injury occurred 3 to 13 months after ETI initiation in CF patients and were reported to the Bordeaux Pharmacovigilance centre. Aside, in Vigibase, ETI is associated with an increased likelihood of reporting suicidal behaviour (ROR 2.5, 95 % CI[2.1; 2.8]). A signal of disproportionate reporting was found for the subgroup of suicide attempts (1.4, 95 % CI[1.2; 1.8]), unlike ivacaftor/lumacaftor, which was associated only with the risk of reporting suicidal ideation. Significant ROR values were also found for reduced-dose ETI for all psychiatric effects studied except suicide attempt.Conclusions: ETI exposure is related with increased reporting of suicidal behaviour. A potential dose-dependent effect merits further investigation.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466568","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

Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on the oropharyngeal metagenome in adolescents with cystic fibrosis. elexacaftor/tezacaftor/ivacaftor 对囊性纤维化青少年口咽部元基因组的纵向影响。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-14 DOI: 10.1016/j.jcf.2024.10.001

Ruth Steinberg, Alexander Moeller, Amanda Gisler, Nadja Mostacci, Markus Hilty, Jakob Usemann

{"title":"Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on the oropharyngeal metagenome in adolescents with cystic fibrosis.","authors":"Ruth Steinberg, Alexander Moeller, Amanda Gisler, Nadja Mostacci, Markus Hilty, Jakob Usemann","doi":"10.1016/j.jcf.2024.10.001","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.10.001","url":null,"abstract":"Background: Triple modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and impacts upon the respiratory microbiome in people with Cystic fibrosis (pwCF) with advanced lung disease. However, adolescents with cystic fibrosis (CF) are less colonized with bacterial pathogens than adult pwCF but their microbiota already differs from healthy individuals. The aim of this study was to longitudinally analyze the impact of ETI on the respiratory metagenome in adolescents with predominantly mild CF lung disease.Methods: In this prospective observational study, we included pwCF aged 12-20 years with at least one F508del mutation, who collected oropharyngeal swabs before and after initiation of ETI therapy twice per week to biweekly over three months. We performed whole metagenome shotgun sequencing, followed by host DNA filtering and taxonomic profiling. We used linear and additive mixed effects models adjusted for known confounders and corrected for multiple testing to study longitudinal development of the microbiome. We analyzed bacterial diversity, abundance, and strain-level phylogeny.Results: We analyzed the metagenomic data of 297 swabs of 20 pwCF. Microbiome composition changed after initiation of ETI therapy. We observed a slight diversification of the microbiome over time (Inv Simpson, Coef 0.085, 95 %CI 0.003, 0.17, p = 0.04). Strain-level analysis and clustering showed that strain retention of the most frequent bacterial species is predominant even during ETI therapy.Conclusions: During three months of ETI therapy, commensal bacteria increased, which may help to prevent overgrowth of bacterial pathogens.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466567","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

Changes in factors associated with inhaled antibiotic prescriptions for people with cystic fibrosis over time in the U.S. 美国囊性纤维化患者吸入抗生素处方相关因素随时间推移的变化。

IF 5.4 2区医学

Journal of Cystic Fibrosis Pub Date : 2024-10-09 DOI: 10.1016/j.jcf.2024.09.017

Marianne S Muhlebach, Jane She, Eric Y Zhang, Jonathan D Cogen, Michael R Kosorok

{"title":"Changes in factors associated with inhaled antibiotic prescriptions for people with cystic fibrosis over time in the U.S.","authors":"Marianne S Muhlebach, Jane She, Eric Y Zhang, Jonathan D Cogen, Michael R Kosorok","doi":"10.1016/j.jcf.2024.09.017","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.09.017","url":null,"abstract":"Rationale: CF care guidelines recommend chronic inhaled antibiotics for chronic Pseudomonas aeruginosa (Pa) lung infection. These medications are costly, time consuming and prescription needs may change with improved outcomes.Objectives: We determined the proportion of pwCF with chronic, intermittent or negative Pa infection categories, their clinical and demographic characteristics, factors associated with inhaled antibiotic prescription and changes between 2011 and 2019.Methods: This cohort study using the U.S. CF Foundation patient registry for pwCF >2 years, no prior lung transplant, and with ≥3 respiratory cultures/year determined chronic inhaled antibiotics (≥3 months per calendar year) and Pa infection status from encounter level data. Outcomes and odds of prescription for relevant clinical factors were evaluated using generalized estimating equation models with additional interaction between the predictor and the calendar year to examine changes of predictors over time.Results: Proportion of pwCF with chronic and intermittent Pa decreased and antibiotic prescription rates increased for these groups and decreased for Pa negative pwCF. Hispanic ethnicity, female sex, pancreatic insufficiency, CF diabetes, and ivacaftor/lumacaftor were associated with higher antibiotic prescriptions for each Pa status. Among Pa-negative pwCF prescriptions were higher with Burkholderia spp. (1.17, (CI95 1.03,1.34)) or MRSA (OR 1.45, (1.26,1.68)) but decreased between 2011 and 2019. For Aspergillus OR increased to 1.6,(1.3,1.8) in 2019. Prescriptions for pwCF on ivacaftor decreased, becoming lower in 2019 for chronic (OR 0.7, (0.5,0.8)) and Pa-negative pwCF (OR 0.7, (0.5,0.8)).Conclusions: Factors predicting inhaled antibiotic prescription differed between 2011 and 2019 indicating changes in health and care for pwCF even prior to triple-modulators.","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400430","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