Journal of Cystic Fibrosis最新文献

{"title":"Theranostics vs theratyping or theranostics plus theratyping?","authors":"Margarida D. Amaral,&nbsp;Ines Pankonien","doi":"10.1016/j.jcf.2024.09.013","DOIUrl":"10.1016/j.jcf.2024.09.013","url":null,"abstract":"<div><div>Treating all people with Cystic Fibrosis (pwCF) to the level of benefit achieved by highly efficient CFTR modulator therapies (HEMT) remains a significant challenge. Theratyping and theranostics are two distinct approaches to advance CF treatment. Both theratyping in cell lines and pwCF-derived biomaterials theranostics have unique strengths and limitations in the context of studying and treating CF. The challenges, advantages and disadvantages of both approaches are discussed here. While theratyping in cell lines offers ease of use, cost-effectiveness, and standardized platforms for experimentation, it misses physiological relevance and patient-specificity. Theranostics, on the other hand, provides a more human-relevant model for personalized medicine approaches but requires specialized expertise, resources, and access to patient samples. Integrating these two approaches in parallel and leveraging their respective strengths may enhance our understanding of CF and facilitate the development of more effective therapies for all pwCF.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 1","pages":"Pages 10-15"},"PeriodicalIF":5.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"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 ,&nbsp;Jonathan V Todd ,&nbsp;Josh S. Ostrenga ,&nbsp;Christopher H Goss ,&nbsp;Raksha Jain ,&nbsp;Wayne Morgan ,&nbsp;Gregory S. Sawicki ,&nbsp;Michael S Schechter ,&nbsp;Elizabeth A Cromwell ,&nbsp;Clement L Ren","doi":"10.1016/j.jcf.2024.10.006","DOIUrl":"10.1016/j.jcf.2024.10.006","url":null,"abstract":"<div><h3>Background</h3><div>This study aims to characterize the uptake of elexacaftor/tezacaftor/ivacaftor (ETI) following Food and Drug Administration (FDA) approval in October 2019.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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 <em>CFTR</em> modulator (if eligible). Public health insurance, ppFEV₁ &gt;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.</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 1","pages":"Pages 135-141"},"PeriodicalIF":5.4,"publicationDate":"2025-01-01","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}

{"title":"Impact of elexacaftor/tezacaftor/ivacaftor on utilization of routine therapies in cystic fibrosis: Danish nationwide register study","authors":"Hans Kristian Råket ,&nbsp;Camilla Bjørn Jensen ,&nbsp;Joanna Nan Wang ,&nbsp;Tacjana Pressler ,&nbsp;Hanne Vebert Olesen ,&nbsp;Marianne Skov ,&nbsp;Søren Jensen-Fangel ,&nbsp;Janne Petersen ,&nbsp;Espen Jimenez-Solem ,&nbsp;for the TransformCF study group","doi":"10.1016/j.jcf.2024.11.004","DOIUrl":"10.1016/j.jcf.2024.11.004","url":null,"abstract":"<div><h3>Background</h3><div>Elexacaftor/tezacaftor/ivacaftor (ETI) has been effective in improving several outcomes in people living with cystic fibrosis (pwCF). Although clinical guidance regarding maintenance therapies has not changed, staff reports indicate that individuals reduce some therapies. This study aimed to evaluate ETI's effect on utilization of routine therapies among pwCF in Denmark.</div></div><div><h3>Methods</h3><div>We included all pwCF initiating ETI between 1 September 2020 and 31 October 2022. Utilization of routine therapies was analysed by drug class (e.g., gastrointestinal medications) and individual treatments (e.g., pancreatic enzymes) before and after ETI initiation using national registry data. Odds ratios (ORs) for prescription redemptions pre- and post-ETI were calculated to assess ETIs impact on the use of routine therapies.</div></div><div><h3>Results</h3><div>The study population consisted of 351 individuals. Median age was 23 years (IQR 14–32) and mean ppFEV₁ was 76 (SD 22) at index. Two-year follow-up was available for 205 individuals. Two years post ETI initiation, the one-year prevalence was reduced for airway medications, (89.5 % to 75.1 %) and inhaled antibiotics (59.5 % to 42.9 %.). OR for redeeming a prescription two years post-ETI initiation (95 % CI) was reduced for four out of five drug classes: airway medications (OR: 0.24 [0.19; 0.29]), inhaled antibiotics (OR: 0.28 [0.2; 0.39]), oral antibiotics (OR: 0.49 [0.41; 0.58]), gastrointestinal medications (OR: 0.66 [0.57; 0.77]).</div></div><div><h3>Conclusion</h3><div>Two years after ETI initiation, reductions in the use of several routine therapies were observed in a national cohort of pwCF, with the largest declines in airway medications and antibiotics. These findings highlight ETI's real-world impact beyond conventional clinical metrics.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 1","pages":"Pages 105-111"},"PeriodicalIF":5.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142709547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of UPLIFT, a group telehealth intervention, on symptoms of depression and anxiety in adults with CF.","authors":"Michael S Schechter, Andrea Molzhon, Robin S Everhart, Le Kang, Rachel Weiskittle, Brittany Castleberry, Nancy J Thompson","doi":"10.1016/j.jcf.2024.11.008","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.008","url":null,"abstract":"<p><strong>Background: </strong>Despite high rates of anxiety and depression, research regarding the effect of psychological interventions on people with CF (pwCF) is limited. We evaluated the impact of UPLIFT (Using Practice and Learning to Increase Favorable Thoughts), a group telehealth intervention using mindfulness-based cognitive behavioral therapy (MBCT), on symptoms of anxiety and depression in pwCF.</p><p><strong>Methods: </strong>This multicenter randomized trial compared changes in symptoms of anxiety and/or depression in adult pwCF who participated in the 8-week UPLIFT intervention to a treatment-as-usual (TAU) group. Follow up assessments occurred immediately after and 6- and 12-months post-intervention. Primary outcome measures were change in Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder (GAD-7) scores modeled in separate linear mixed-effects models.</p><p><strong>Results: </strong>Sixty-six pwCF participated. At baseline, 43 (65.15%) had some minimal symptoms of depression (PHQ-9≥5) and 44 (66.67%) had some minimal symptoms of anxiety (GAD-7≥5). During the 12 month follow up period, the overall change in PHQ-9 was greater in the UPLIFT group compared to TAU (p = .049). Analysis of individual time points showed a statistically significant difference between groups in change from baseline immediately post-treatment (-2.321, SD 0.684 vs 0.362, SD 0.656, p = .005); differences persisted but were not statistically significant at 6 and 12 months. Similar trends for changes in GAD-7 were non-significant.</p><p><strong>Conclusions: </strong>Participation in UPLIFT, a group telehealth intervention using MBCT, provides short-term improvement in symptoms of depression, as measured by changes in PHQ9. Improvement in symptoms of anxiety were suggested but could not be statistically confirmed.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818290","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":"Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines.","authors":"E P Dellon, G Allada, S J Allgood, A M Georgiopoulos, J L Goggin, D Hadjiliadis, J D Lowman, S Madge, B Middour-Oxler, C Muirhead, M Noel, P Wilson, Hempstead Se, A Faro, D Kavalieratos","doi":"10.1016/j.jcf.2024.11.012","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.012","url":null,"abstract":"<p><p>Even as many outcomes for people living with cystic fibrosis (PLwCF) improve, individuals still experience extensive symptom burdens. From birth, many PLwCF experience both pain as a symptom of their CF disease and procedural pain, posing detriments to health, functioning, and quality of life. Despite its prevalence and impact, there is no CF-specific guidance for the assessment and management of pain. Similarly, no guidance exists regarding communication with PLwCF about their pain experiences or its impact on their lives. Therefore, the Cystic Fibrosis Foundation (CFF) assembled an expert panel of clinicians, researchers, PLwCF, and caregivers to develop consensus recommendations for pain management in CF. We utilized literature review and expert opinion to develop 13 recommendations addressing pain assessment, management, and communication. Recommendations are centered on guiding principles of utilizing a multimodal approach to pain management, offering age and developmentally appropriate assessment and interventions, concurrently treating underlying conditions causing, contributing to, and/or exacerbated by pain, considering societal stigma of the pain experience, particularly for minoritized and marginalized people, and sensitivity to issues of access and cost. These recommendations are intended to guide clinicians in managing pain and improving quality of life for PLwCF with pain at all stages of illness and development.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142791733","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":"Outcomes of prenatal use of elexacaftor/tezacaftor/ivacaftor in carrier mothers to treat meconium ileus in fetuses with cystic fibrosis.","authors":"Angela Metcalf, Stacey L Martiniano, Scott D Sagel, Michael V Zaretsky, Edith T Zemanick, Jordana E Hoppe","doi":"10.1016/j.jcf.2024.11.011","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.011","url":null,"abstract":"<p><p>As cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies including elexacaftor/tezacaftor/ivacaftor (ETI) have become widely used in eligible patients with cystic fibrosis (CF), the use of these medications in pregnant people has become a critical area of investigation. Since these medications appear generally safe to both mother and fetus when taken by pregnant people with CF, interest has pivoted to the use of ETI in CF carrier mothers to decrease morbidity and mortality from meconium ileus (MI) in fetuses with cystic fibrosis. Here we discuss three infants at our institution with ultrasound findings of MI who were exposed to prenatal ETI through CF carrier mothers for the purposes of treating MI and lowering risk of intestinal complications from this severe manifestation of CF. These cases differ in the timing of ETI initiation, severity of outcome, and accessibility of this off-label medication use to families depending on their insurance. All infants and mothers tolerated the medication well without significant side effects. One infant had complete MI resolution, one had persistent MI at birth with easy clearance with minimally invasive therapies, and one had persistent MI requiring jejunostomy. The infant with the most severe outcome had the shortest duration of ETI exposure and may have been able to receive this medication sooner had a referral to a CF center been made. These cases highlight the potentially life-altering effects of prenatal ETI use and the need for awareness of this clinical situation among fetal care providers.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142791745","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":"Treatment effects of elexacaftor/tezacaftor/ivacaftor on people with cystic fibrosis heterozygous for 3849+10kbC->T and a class I variant.","authors":"Moshe Heching, Michal Shteinberg, Inbal Golan-Tripto, Galit Livnat-Levanon, Karin Yaacoby-Bianu, Liora Boehm Cohen, Guy Hazan, Liora Slomianski, Dario Prais, Huda Mussaffi, Joel Weinberg, Mordechai R Kramer","doi":"10.1016/j.jcf.2024.11.010","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.010","url":null,"abstract":"<p><strong>Background: </strong>The splice variant 3849+10kbC->T (c.3717+12191C>T) (3849 variant) is a residual function CFTR variant, characterized by insertion of an in-frame stop codon into most CFTR transcripts. Both ivacaftor (Iva) and tezacaftor/ivacaftor (Tez/Iva) have been approved for people with CF (pwCF) carrying the 3849 variant. In-vitro studies for elexacaftor/tezacaftor/ivacaftor (ETI) did not include the 3849 variant as responsive to ETI. We present the clinical effectiveness of ETI in pwCF homozygous for the 3849 variant or heterozygous for 3849 and class I variants previously treated with Iva or Tez/Iva.</p><p><strong>Methods: </strong>We conducted a multi-center observational study of pwCF homozygous for the 3849 variant or heterozygous for 3849 and class I variants who were transitioned from Iva or Tez/Iva to ETI. We collected clinical data, including sweat chloride concentrations, pulmonary function tests, BMI and intravenous antibiotic treatments.</p><p><strong>Results: </strong>We identified nine pwCF heterozygous for 3849 and class I variants and one pwCF homozygous for the 3849 variant. Prior to transitioning to ETI, nine pwCF were treated with Tez/Iva and one with Iva. Compared to baseline, median sweat chloride concentration declined from 48 to 35 mEq/L (p = 0.009). Median FEV₁ increased from 53 % to 65 % (p = 0.006). Pulmonary exacerbations requiring intravenous antibiotics declined from mean 1.4 to 0.6 in the twelve months before and after ETI.</p><p><strong>Conclusions: </strong>We demonstrate the clinical effectiveness of ETI in pwCF carrying the 3849 variant, in excess of the response to Iva or Iva/Tez. Our results provide preliminary support for clinical use of ETI in pwCF carrying the 3849+10kbC->T variant.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142791748","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":"Daily variability of Pseudomonas aeruginosa density in cystic fibrosis sputum.","authors":"Lisa A Carmody, Lindsay J Caverly, Linda M Kalikin, Christina S Thornton, Richard H Simon, Donald R VanDevanter, John J LiPuma","doi":"10.1016/j.jcf.2024.11.013","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.013","url":null,"abstract":"<p><p>Treatment-associated differences in Pseudomonas aeruginosa (Pa) density in sputum have been used as a response biomarker in clinical trials of cystic fibrosis (CF) therapies. Although most studies have included placebo-treated groups as comparators, variability of Pa density in untreated individuals has rarely been reported. We measured day-to-day differences in Pa density in 267 sputum sample pairs collected from 13 adults with CF during days in which no changes in antibiotic therapy occurred. Although the mean sputum Pa density change across all sample pairs was modest (-0.09 log₁₀ 16S rRNA gene copies/mL), variability in day-to-day changes were substantial (SD = 1.09) with one-quarter of sample pairs having >1 log₁₀ differences in Pa density; approximately 8 % of pairs had >2 log₁₀ differences in density. Day-to-day variability in Pa density differed substantially between study participants (p = .001). These results will support the design and interpretation of studies using sputum Pa density change as an efficacy biomarker.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142769462","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":"Measurement of treatment burden in cystic fibrosis: A systematic review.","authors":"Rana Altabee, Martin J Mwamba, David Turner, Gwyneth Davies, Janice Abbott, Nicholas J Simmonds, Jennifer A Whitty, Siobhán B Carr, Garry Barton, Rory A Cameron","doi":"10.1016/j.jcf.2024.11.005","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.005","url":null,"abstract":"<p><strong>Background: </strong>Cystic fibrosis (CF) is a chronic condition that requires complex and long-term treatments. While substantial research has explored treatment burden associated with CF; its impact remains complex to quantify. This review aims to identify the different methods used in the literature to measure treatment burden in people with CF (pwCF).</p><p><strong>Method: </strong>Five databases were searched for interventional and observational studies that focused primarily on treatment burden. The studies were presented using narrative synthesis structured around the perspective of treatment burden (subjective vs. objective).</p><p><strong>Results: </strong>This review synthesised 17 articles, which utilised subjective and objective measures separately or collectively. Twelve studies used subjective treatment burden measures (CF-specific and generic scales), while 14 studies used objective measures (treatment time, volume and complexity, and cost). Eight studies investigated treatment burden reported by proxy on behalf of children with CF. The most used measures were treatment time (9/17) and CF questionnaire-revised (CFQ-R) treatment burden subscale (6/17). Older age and lower lung function were associated with greater burden, treatment time, and complexity. Caregivers/parents reported worse treatment burden compared to children with CF (6-13 y/o) when completing the same measure.</p><p><strong>Conclusion: </strong>No single measure used in the reviewed studies fully the multidimensional nature of treatment burden and summarised it in a single score. Given the rapidly evolving landscape of CF care a pragmatic approach to capture a broader array of treatment burden dimensions may be to routinely complement subjective measures with objective measures.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142769463","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":"Active parents, active youth? Exploring the association between physical activity of youth with Cystic Fibrosis and their parents.","authors":"Manon Kinaupenne, Stephanie Van Biervliet, Kim Van Hoorenbeeck, Heidi Schaballie, Kristof Vandekerckhove, Marieke De Craemer, Heleen Demeyer","doi":"10.1016/j.jcf.2024.11.007","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.11.007","url":null,"abstract":"<p><strong>Background: </strong>Parents play a major role in shaping their children's physical activity (PA) behaviour. This study aimed to investigate the association between PA of youth with Cystic Fibrosis (YwCF) and their parents.</p><p><strong>Methods: </strong>PA was measured by an ActiGraph GT3x-BT for seven consecutive days. Data were processed by GGIR and PA intensities were based on the age-specific Hildebrand equations. Moderate-to-vigorous PA was chosen as primary outcome.</p><p><strong>Results: </strong>26 YwCF-parent dyads participated. A significant positive association was found between parental PA behaviour and YwCF's PA behaviour for both moderate-to-vigorous PA and total PA (R² = 0.60; p = 0.001; R² = 0.64; p < 0.001). Furthermore, YwCF with less active parents perform 16 min/day less moderate-to-vigorous PA compared to YwCF with more active parents (p = 0.004).</p><p><strong>Conclusion: </strong>Higher parental PA levels are strongly associated with higher YwCF's PA levels. This association needs to be confirmed in a larger cohort to explore whether parental behaviour is an effective strategy to improve YwCF's PA levels.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142754934","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}