Description
Articles de périodiques scientifiques
Général
- Cystic fibrosis-associated amyloid a amyloidosis: a rare but devastating complication with poor prognosison 11 août 2026
Eur J Pediatr. 2026 Aug 6;185(8):645. doi: 10.1007/s00431-026-07312-8.ABSTRACTAmyloid A (AA) amyloidosis is a rare but life-threatening complication of cystic fibrosis (CF) driven by chronic […]
- Cystic fibrosis: insights from zebrafish modelson 11 août 2026
Dis Model Mech. 2026 Jul 1;19(7):dmm052856. doi: 10.1242/dmm.052856. Epub 2026 Aug 5.ABSTRACTCystic fibrosis (CF) is a severe, life-limiting genetic disorder caused by mutations in the CFTR gene, […]
- Population Pharmacokinetic and Exposure-Response Analysis of Vancomycin Nephrotoxicity in Cystic Fibrosis Patientson 11 août 2026
Pediatr Pulmonol. 2026 Aug;61(8):e71748. doi: 10.1002/ppul.71748.ABSTRACTINTRODUCTION: Acute pulmonary exacerbations (APE) in persons with cystic fibrosis (PwCF) are associated with a reduction in […]
- Current diabetes therapy and use of diabetes technology in people living with cystic fibrosis-related diabetes (CFRD): Insights from the DPV networkon 11 août 2026
J Cyst Fibros. 2026 Jul 24:S1569-1993(26)01658-9. doi: 10.1016/j.jcf.2026.07.002. Online ahead of print.ABSTRACTBACKGROUND: Cystic fibrosis-related diabetes (CFRD) is a common comorbidity in cystic […]
- Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Studyon 11 août 2026
Adv Respir Med. 2026 Jul 14;94(4):47. doi: 10.3390/arm94040047.ABSTRACTCystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene. The sequential approval of CFTR […]
- Gastrointestinal and Pancreatic Manifestations of Cystic Fibrosis in the Era of Highly Effective Modulatorson 11 août 2026
Adv Pediatr. 2026 Aug;73(1):179-190. doi: 10.1016/j.yapd.2025.11.001. Epub 2026 May 19.ABSTRACTThis review synthesizes emerging evidence on gastrointestinal (GI) disorders, specifically luminal and […]
Diagnostic et évaluation
- Clinical characteristics and outcomes of people with cystic fibrosis with homozygous minimal function genotypes compared to those with F508del/minimal function genotypeson 11 août 2026
Respir Med. 2026 Aug 4:109079. doi: 10.1016/j.rmed.2026.109079. Online ahead of print.ABSTRACTThis observational, retrospective study compared clinical characteristics and outcomes in people with […]
- DNA sequencing for microbial surveillance in cystic fibrosis airways: advances, challenges, and clinical translationon 11 août 2026
Clin Microbiol Rev. 2026 Aug 3:e0035225. doi: 10.1128/cmr.00352-25. Online ahead of print.ABSTRACTSUMMARYDNA sequencing has revolutionized microbial surveillance in cystic fibrosis (CF), transforming […]
- Extracellular vesicle-based delivery to airway basal cells for durable gene therapy in cystic fibrosison 11 août 2026
Front Bioeng Biotechnol. 2026 Jul 15;14:1855907. doi: 10.3389/fbioe.2026.1855907. eCollection 2026.ABSTRACTAirway basal cells are an appealing target for durable genetic therapy in cystic fibrosis […]
- Differential Metabolite Production Underlies Disruption of the Cystic Fibrosis Airway Microbiota by Pathogenson 11 août 2026
bioRxiv [Preprint]. 2026 Jul 17:2026.07.16.738945. doi: 10.64898/2026.07.16.738945.ABSTRACTCystic fibrosis (CF) is a multisystem disease characterized by the accumulation of mucus in the airways that […]
- Cystic Fibrosis: Modern Diagnostic and Therapeutic Advances from Molecular Pathogenesis to Multidisciplinary Managementon 11 août 2026
Genes (Basel). 2026 Jun 27;17(7):743. doi: 10.3390/genes17070743.ABSTRACTCystic fibrosis (CF) is an autosomal recessive disorder caused by pathogenic variants in the cystic fibrosis transmembrane […]
- Abnormalities of the distal terminal ileum in people with cystic fibrosis assessed using magnetic resonance imagingon 11 août 2026
J Cyst Fibros. 2026 Jul 25:S1569-1993(26)01662-0. doi: 10.1016/j.jcf.2026.07.009. Online ahead of print.ABSTRACTBACKGROUND: The terminal ileum (TI) is commonly affected in people with cystic fibrosis […]
- Are Sputum High Mobility Group Box 1 and D-Dimer Changes Relevant Markers of Tissue Damage and Fibrinolysis in Cystic Fibrosis?on 11 août 2026
Pathophysiology. 2026 Jul 21;33(3):53. doi: 10.3390/pathophysiology33030053.ABSTRACTBACKGROUND/OBJECTIVES: Cystic fibrosis (CF) is a genetic disease whose hallmarks include chronic inflammation of […]
Interventions et traitements
- Correlation between pulmonary outcomes and nutritional status following treatment with elexacaftor/tezacaftor/ivacaftor in people with Cystic fibrosison 11 août 2026
Respir Med. 2026 Aug 6:109096. doi: 10.1016/j.rmed.2026.109096. Online ahead of print.ABSTRACTBACKGROUND: Elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and nutritional status in […]
- Pulmonary Outcomes in People with Cystic Fibrosis who Stop Chronic Daily Therapies: The HERO-2 Studyon 11 août 2026
Ann Am Thorac Soc. 2026 Aug 5:aaoag234. doi: 10.1093/annalsats/aaoag234. Online ahead of print.ABSTRACTRATIONALE: Given improvement in symptoms and lung function in people with cystic fibrosis (CF) […]
- Long-Term Clinical Efficacy of Elexacaftor-Tezacaftor-Ivacaftor in People With Cystic Fibrosis and Preexisting Advanced Lung Disease at Treatment Initiationon 11 août 2026
CHEST Pulm. 2024 Sep 7;3(1):100099. doi: 10.1016/j.chpulm.2024.100099. eCollection 2025 Mar.ABSTRACTBACKGROUND: Elexacaftor-tezacaftor-ivacaftor (ETI) is associated with increased FEV1, decreased […]
- A Multicenter Retrospective Study Evaluating IL-5-Targeted Biologic Therapies for the Treatment of Asthma and Allergic Bronchopulmonary Aspergillosis in Adults With Cystic Fibrosison 11 août 2026
CHEST Pulm. 2025 Mar 12;3(2):100163. doi: 10.1016/j.chpulm.2025.100163. eCollection 2025 Jun.ABSTRACTBACKGROUND: Biologic therapies targeting T-helper cell type 2 (Th2) eosinophilic inflammation have […]
- Real-world population pharmacokinetic modeling of elexacaftor, tezacaftor, and ivacaftoron 11 août 2026
J Cyst Fibros. 2026 Jul 30:S1569-1993(26)03659-3. doi: 10.1016/j.jcf.2026.07.1984. Online ahead of print.ABSTRACTBACKGROUND: Cystic fibrosis transmembrane conductance regulator modulators have […]
- The Role of ETI Therapy in Managing Sinusitis in Cystic Fibrosis pediatric population: A Study on Clinical Outcomeson 11 août 2026
Respir Med. 2026 Jul 28:109065. doi: 10.1016/j.rmed.2026.109065. Online ahead of print.ABSTRACTBACKGROUND: Chronic rhinosinusitis (CRS) is a common extrapulmonary manifestation of cystic fibrosis […]
- Evolving Cystic Fibrosis Therapy: The Good, the Sad, and the Hopefulon 11 août 2026
Children (Basel). 2026 Jun 30;13(7):878. doi: 10.3390/children13070878.ABSTRACTCystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane […]
- FT-IR Spectroscopy to Discriminate Old from New Pseudomonas aeruginosa Infections in People with Cystic Fibrosison 11 août 2026
Int J Mol Sci. 2026 Jul 20;27(14):6452. doi: 10.3390/ijms27146452.ABSTRACTChronic Pseudomonas aeruginosa colonisation leads to lung deterioration and poor prognosis in people with cystic fibrosis […]
- Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single-center cohort studyon 11 août 2026
JPGN Rep. 2026 Apr 27. doi: 10.1002/jpr3.70186. Online ahead of print.ABSTRACTOBJECTIVES: The efficacy of cystic fibrosis transmembrane conductance regulator (CFTR)-modulator therapies in preventing […]
- Nucleic acid-based therapies for people with cystic fibrosison 11 août 2026
Cochrane Database Syst Rev. 2026 Jul 23;7:CD016370. doi: 10.1002/14651858.CD016370.ABSTRACTThis is a protocol for a Cochrane Review (intervention). The objectives are as follows: To assess the […]
- Polymicrobial Extracellular Vesicles Reduce the Innate Immune Response of Human Cystic Fibrosis Bronchial Epithelial Cellson 11 août 2026
J Extracell Biol. 2026 Aug 3;5(8):e70176. doi: 10.1002/jex2.70176. eCollection 2026 Aug.ABSTRACTChronic antibiotic-resistant cystic fibrosis (CF) lung infections are the leading cause of death in […]
Transplantation
- Divergent Management of the Foregut Between Pulmonologists and Gastroenterologists in Advanced Cystic Fibrosis Lung Diseaseon 20 juillet 2026
Pediatr Pulmonol. 2026 Jul;61(7):e71740. doi: 10.1002/ppul.71740.ABSTRACTBACKGROUND: Advanced cystic fibrosis lung disease (ACFLD) remains a major cause of morbidity, particularly in individuals […]
- A Single-Center Retrospective Cohort Review of Pancreatic Cysts and Malignancy in Cystic Fibrosis Lung Transplant Recipientson 20 juillet 2026
Dig Dis Sci. 2026 Jul 15. doi: 10.1007/s10620-026-10110-9. Online ahead of print.ABSTRACTBACKGROUND: Individuals with cystic fibrosis (CF) develop pancreatic cysts and cancer more frequently than the […]
Mode de vie et approches complémentaires
- Mental health, sleep, and quality of life following CFTR modulator therapy: a longitudinal study of children with cystic fibrosis and caregiverson 11 août 2026
Eur J Pediatr. 2026 Aug 11;185(9):651. doi: 10.1007/s00431-026-07331-5.ABSTRACTModulator therapies have been shown to improve clinical outcomes, particularly respiratory outcomes, in children with […]
- Nutrition and cardiometabolic health in cystic fibrosis: Emerging evidence and research gapson 11 août 2026
J Cyst Fibros. 2026 Aug 7:S1569-1993(26)03658-1. doi: 10.1016/j.jcf.2026.07.1983. Online ahead of print.ABSTRACTCardiometabolic risk is increasingly recognised as a significant and evolving health […]
- Shifting Nutrition Paradigms: Changing Attitudes Towards Diet and Body Perceptions Among People With CF in the Era of Highly Effective Modulatorson 11 août 2026
J Hum Nutr Diet. 2026 Aug;39(4):e70323. doi: 10.1111/jhn.70323.ABSTRACTINTRODUCTION: People with cystic fibrosis (PwCF) have historically been counselled to eat a high-energy, high-fat diet to […]
- Integrating Pain Management in a Patient With Cystic Fibrosis and Fibromyalgia: A Case Reporton 11 août 2026
Pain Med Case Rep. 2026 Jul;10(5):395-400.ABSTRACTBACKGROUND: Chronic pain is increasingly recognized in individuals with cystic fibrosis (CF), especially as survival improves. However, the […]
- EASE-CF: dietitian-led weight-loss intervention for adults with cystic fibrosis and excess weight: protocol for a feasibility randomised controlled trialon 11 août 2026
BMJ Open Respir Res. 2026 Jul 27;13(1):e004328. doi: 10.1136/bmjresp-2026-004328.ABSTRACTINTRODUCTION: Advances in cystic fibrosis (CF) care have coincided with an increasing prevalence of excess […]
Expériences des patients et des institutions
- Policy in practice: Cystic Fibrosis Australia and Phage Australia surveys: understanding clinical needs and attitudes towards phage therapy in the cystic fibrosis communityon 11 août 2026
Sustain Microbiol. 2025 Jan 24;2(1):qvae036. doi: 10.1093/sumbio/qvae036. eCollection 2025.ABSTRACTCystic fibrosis (CF) is the most prevalent serious inherited disease in Australia, imposing […]
- Multidisciplinary Cystic Fibrosis Teams: First Steps to Improve Professional Quality of Life and Address Burnouton 11 août 2026
Chest. 2026 Aug;170(2):283-284. doi: 10.1016/j.chest.2026.04.033.NO ABSTRACTPMID:42575631 | DOI:10.1016/j.chest.2026.04.033
- Impact of High-Frequency Chest Wall Oscillation on Health Care Resource Use and Economic Outcomes in Adult Patients With Non-Cystic Fibrosis Bronchiectasis in the United States: A Pre-Post Cohort Analysison 11 août 2026
CHEST Pulm. 2023 Aug 19;2(1):100013. doi: 10.1016/j.chpulm.2023.100013. eCollection 2024 Mar.ABSTRACTBACKGROUND: Airway clearance therapy is prescribed to patients with bronchiectasis, but limited […]
- Using Real-World Research to Study the Impact of Chronic Daily Therapy Discontinuation in Cystic Fibrosis: The Home-Reported Outcomes in Cystic Fibrosis 2 Study Designon 11 août 2026
CHEST Pulm. 2024 Jun 29;2(4):100080. doi: 10.1016/j.chpulm.2024.100080. eCollection 2024 Dec.ABSTRACTBACKGROUND: Chronic daily therapies (CDTs) are the foundation of clinical care for people with […]
- Mental Health Changes in Adolescents and Adults With Cystic Fibrosis After Initiation of Elexacaftor/Tezacaftor/Ivacaftor Therapy: Insights From the Longitudinal Resilience Impacted by Positive Stressful Events (RISE) Studyon 11 août 2026
CHEST Pulm. 2025 Feb 7;3(3):100146. doi: 10.1016/j.chpulm.2025.100146. eCollection 2025 Sep.ABSTRACTBACKGROUND: Starting elexacaftor/tezacaftor/ivacaftor (ETI) is considered a positive life event for […]
- Health State Utilities Associated With Treatment Burden in Cystic Fibrosis: A Patient Valuation Studyon 11 août 2026
CHEST Pulm. 2024 Aug 23;3(1):100097. doi: 10.1016/j.chpulm.2024.100097. eCollection 2025 Mar.ABSTRACTBACKGROUND: Although recent advancements in the treatment of cystic fibrosis (CF) have improved […]
- Stories, Systems, and Survival: Photovoice Insights on Structural Racism and Cystic Fibrosis Careon 11 août 2026
Health Equity. 2026 Jun 2;10:24731242261451646. doi: 10.1177/24731242261451646. eCollection 2026 Jan-Dec.ABSTRACTINTRODUCTION: People who identify as Black, Indigenous, and People of Color (BIPOC) […]
- Exploring the lived experiences of individuals with cystic fibrosis after the implementation of elexacaftor/tezacaftor/ivacaftor for treatmenton 11 août 2026
Ther Adv Respir Dis. 2026 Jan-Dec;20:17534666261469021. doi: 10.1177/17534666261469021. Epub 2026 Jul 25.ABSTRACTBACKGROUND: CFTR modulators are a class of medications prescribed to people with […]
- EnVision CF: Building the Next Generation of Leaders in Cystic Fibrosis Endocrinology. A Model for Physician-Scientist Workforce Development in Advanced Subspecialty Careon 11 août 2026
AACE Endocrinol Diabetes. 2026 May 14;13(4):662-666. doi: 10.1016/j.aed.2026.04.018. eCollection 2026 Jul-Aug.ABSTRACTINTRODUCTION: As people with cystic fibrosis (CF) live longer, the burden of […]
- Timing and rate of reimbursement for cystic fibrosis modulator therapies in EU countrieson 11 août 2026
J Cyst Fibros. 2026 Jul 21:S1569-1993(26)01663-2. doi: 10.1016/j.jcf.2026.07.008. Online ahead of print.ABSTRACTBACKGROUND: In the EU, Cystic Fibrosis modulator therapies (CFTR modulators) are […]
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