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Introduction: The prognosis of people diagnosed with cystic fibrosis (CF) has dramatically improved over the past decade in France, largely due to advances in CF care management, including an emphasis on chronic maintenance medications. Currently, the majority of French CF patients are adults, which means that they went through a transition process from receiving care at a pediatric CF center to receiving care at an adult CF center. To determine the impact of the transfer on clinical evolution, we report the transition procedure of our CF center in Lyon.
Materials And Methods: From January 2006 to December 2016, 97 CF patients underwent a standardized process of transitioning from the pediatric to the adult CF center in Lyon. We compared the clinical evolution of these patients during three periods, starting the year before transition and ending the year after transition. Clinical data taken into account were forced expiratory volume in 1 s (FEV1 in liters), body mass index (BMI in kg/m ), pulmonary colonization, number of antibiotic courses, number of days of hospitalization per year, and outpatient visits per year.
Results: No significant differences were observed between respiratory and nutritional status, respiratory microbiome, number of antibiotic courses, or number of hospitalizations or visits when comparing the threeperiods of observation around transition (the year before, the first year after, and the second year after transfer).
Conclusion: The standardized transition procedure used in Lyon is associated with the clinical stability of our CF patients.
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http://dx.doi.org/10.1002/ppul.25364 | DOI Listing |
Scand J Caring Sci
September 2025
Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences (ESSUA) and Institute of Biomedicine (iBiMED), University of Aveiro, Aveiro, Portugal.
Background: Cystic fibrosis imposes a significant treatment burden on children and their informal caregivers, who have to change their routines to carefully adhere to medication and exercise as treatment regimes. Although informal caregivers are known to be key players in the daily management of these children, their own voice is scarcely explored, often hindering personalisation of care. The main objective of the study was to map the multifactorial impact of cystic fibrosis, as well as identify barriers and facilitators perceived by parental caregivers in managing the disease in the paediatric age.
View Article and Find Full Text PDFAllergy
September 2025
Department of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, Lydia Becker Institute of Immunology and Inflammation, The University of Manchester, Manchester, UK.
Mast cells (MCs) rapidly adapt to the microenvironment due to the plethora of cytokine receptors expressed. Understanding microenvironment-primed immune responses is essential to elucidate the phenotypic/functional changes MCs undergo, and thus understand their contribution to diseases and predict the most effective therapeutic strategies. We exposed primary human MCs to cytokines mimicking a T1/pro-inflammatory (IFNγ), T2/allergic (IL-4 + IL-13), alarmin-rich (IL-33) and pro-fibrotic/pro-tolerogenic (TGFβ) microenvironment.
View Article and Find Full Text PDFMol Ther
September 2025
Sorbonne Université, Inserm, Centre de Recherche Saint-Antoine, CRSA, F-75012 Paris, France. Electronic address:
Patients with cystic fibrosis (CF) who are non-responsive to treatments due to specific mutations need alternative CFTR-independent therapies. This study aims to assess the impact of TMEM16a expression by a specific oligonucleotide (TMEM16a ASO) on dysregulated parameters in CF, which will help prepare for preclinical studies. In this study, we analyzed the effects of TMEM16a oligonucleotide within a CF context by evaluating the impact, optimal administration route, toxicity, and specificity in primary cells and various mouse models.
View Article and Find Full Text PDFImmunol Lett
September 2025
Department of Clinical and Translational Science, College of Graduate Health Science, University of Tennessee Health Science Center, Memphis, Tennessee. Electronic address:
Background: Patients with chronic lung diseases often suffer from pulmonary aspergillosis, caused by Aspergillus fumigatus (AF). Alveolar macrophages play a key role in the initial immune response to AF. Azithromycin (AZM), commonly known for its immunomodulatory properties in reducing exacerbations and improving lung function, has mixed effects on the development of aspergillosis.
View Article and Find Full Text PDFTurk J Pediatr
September 2025
Department of Cardiorespiratory Physiotherapy and Rehabilitation, Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Türkiye.
Background: Vascular changes are observed in children with cystic fibrosis (cwCF), and gender-specific differences may impact arterial stiffness. We aimed to compare arterial stiffness and clinical parameters based on gender in cwCF and to determine the factors affecting arterial stiffness in cwCF.
Methods: Fifty-eight cwCF were included.