Results 91 to 100 of about 5,299 (208)

Epithelial responses to CFTR modulators are improved by inflammatory cytokines and impaired by antiinflammatory drugs

JCI Insight
Cystic fibrosis (CF) is a genetic disorder that disrupts CF transmembrane conductance regulator (CFTR) anion channels and impairs airway host defenses. Airway inflammation is ubiquitous in CF, and suppressing it has generally been considered to improve ...
Tayyab Rehman, Alejandro A. Pezzulo, Andrew L. Thurman, Rachel L. Zemans, Michael J. Welsh   +4 more
doaj   +1 more source

Clinical Outcomes in Patients with Cystic Fibrosis Receiving CFTR Modulators: A Comparison of Childhood Versus Adolescent Initiation

Children
Background/objectives: Cystic fibrosis (CF) is a life-limiting genetic disorder affecting multiple organ systems. This study compared clinical outcomes, hospitalization rates, and survival between children and adolescents with CF who received CFTR ...
Eman A. Toraih, Hassan A. Malik, Rahib K. Islam, Humza A. Pirzadah, Ahmed Abdelmaksoud, Rami M. Elshazli, Paul Antwi Boasiako, Shehab Ahmed Alenazi, Angelique Dabel, Jessan A. Jishu, Bandar T. Alenezi, Hani Aiash, Manal S. Fawzy   +12 more
doaj   +1 more source

Polymicrobial Extracellular Vesicles Reduce the Innate Immune Response of Human Cystic Fibrosis Bronchial Epithelial Cells

Journal of Extracellular Biology, Volume 5, Issue 8, August 2026.
ABSTRACT Chronic antibiotic‐resistant cystic fibrosis (CF) lung infections are the leading cause of death in adults with CF. Despite advances in highly effective modulator therapies, microbial communities persist in the CF lung. The pathogenesis of CF airway infections can be exacerbated by pathogens such as Pseudomonas aeruginosa, which communicates ...
Lily A. Charpentier, Roxanna L. Barnaby, Carolyn T. Roche, Byoung‐Kyu Cho, Prashant Kaushal, Young Ah Goo, Brad Vietje, Douglas J. Taatjes, Alix Ashare, Fabrice Jean‐Pierre, Bruce A. Stanton   +10 more
wiley   +1 more source

MITF Regulates CFTR Expression to Participate in Myocardial Ischemia–Reperfusion Injury

The Journal of Gene Medicine, Volume 28, Issue 8, August 2026.
MITF regulates CFTR expression in MIRI, and disruption of the MITF‐CFTR axis is associated with aggravated injury in cellular and animal models. Restoring CFTR partially mitigates MITF deficiency‐related damage. These findings support the involvement of MITF‐CFTR regulation in MIRI and provide a basis for further mechanistic investigation.
Baoxin Tang, Chenying Zhu, Heqing Wang, Mingkui Gao, Tieyan Li   +4 more
wiley   +1 more source

Obstructive Sleep Apnea: Epidemiology, Pathophysiology, Complications, Diagnosis, Management, and Emerging Fibrosis‐Linked Remodeling

MedComm, Volume 7, Issue 8, August 2026.
Obstructive sleep apnea (OSA) is characterized by recurrent upper‐airway collapse, which generates key nocturnal stressors including intermittent hypoxia, sleep fragmentation, intrathoracic pressure stress, and sympathetic activation. These physiological disturbances converge on shared biological mechanisms, including oxidative stress, inflammation ...
Nhi Ho Thi Thuy, Thị Hằng Giang Phan, Ali Hussein Eid, Paola Maccioccu, Stefania Camboni, Gavino Casu, Anna Maria Posadino, Roberta Giordo, Gianfranco Pintus   +8 more
wiley   +1 more source

Prediction of Antimicrobial Resistance in People Living With Cystic Fibrosis Using Machine Learning

MedComm, Volume 7, Issue 8, August 2026.
Using routinely collected electronic healthcare record data from patients with cystic fibrosis, machine learning models predicted antimicrobial resistance in sputum cultures before susceptibility results were available. Extreme gradient boosting showed consistent discrimination across five commonly tested antibiotics, with AUCs of 0.75–0.80.
Junrong Jiang, Akhil Naik, Dilip Nazareth, Dennis Wat, Graham Hyde, Jo Fothergill, Sarah Benabidallah, Gregory Y. H. Lip, Sandra Ortega‐Martorell, Ivan Olier, Freddy Frost   +10 more
wiley   +1 more source

Lumacaftor-ivacaftor in the treatment of cystic fibrosis: design, development and place in therapy

Drug Design, Development and Therapy, 2019
GJ ConnettNational Institute for Health Research, Southampton Respiratory Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust, Southampton, SO16 6YD, UKAbstract: Lumacaftor-ivacaftor is a combination of two small molecule ...
Connett GJ
doaj  

CFTR mediates Cl- transport in osteocytes to sustain cell viability and skeletal homeostasis

Nature Communications
Osteocytes are long-lived with underlying mechanisms largely unknown. Here, we report that osteocyte-specific knockout of cystic fibrosis transmembrane conductance regulator (CFTR) results in excessive osteocyte death, proinflammatory cytokine surge ...
Peijie Hu, Wanting Du, Muyan Chu, Junjiang Chen, Xiaotian Zhang, Ziyi Chen, Jun Hu, Lei Qin, Wayne Yuk-Wai Lee, Jinghui Guo, Hui Chen, Ruiyao Xu, Xiaojun Cai, Xiaohua Jiang, Hsiao Chang Chan, Ling Qin, Jiankun Xu, Ye Chun Ruan   +17 more
doaj   +1 more source

Utilization of Des‐Gamma‐Carboxy‐Prothrombin in Estimating Vitamin K Status in People With Cystic Fibrosis: A Single Center Retrospective Review

Pediatric Pulmonology, Volume 61, Issue 8, August 2026.
ABSTRACT Background People with cystic fibrosis (CF) are at increased risk of fat‐soluble vitamin (A, D, E, and K) deficiencies secondary to exocrine pancreatic insufficiency (EPI). Estimation of vitamin K deficiency in routine clinical practice is challenging, and no uniform consensus exists due to lack of an accurate clinical test for the evaluation ...
Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A. McBennett, Erica Roesch, Alex H. Gifford   +7 more
wiley   +1 more source

Human Nasal and Bronchial Epithelium in Cystic Fibrosis: Differences in Expression of Airway pH Regulatory Proteins

Pediatric Pulmonology, Volume 61, Issue 8, August 2026.
ABSTRACT Background Decreased apical bicarbonate transport into the airway surface liquid (ASL) has been associated with decreased ASL pH, which can have adverse respiratory effects. However, the human CF epithelium can normalize ASL pH. Thus, we hypothesized that pH regulatory proteins other than the CFTR could be altered in the CF epithelium ...
Michael D. Davis, Laura Smith, Yi Zhao, Rania Abdul Rahman, Faiza Khalid, James Chmiel, Katie Boyne, Ivana A. Daniels, Benjamin Gaston, Nadzeya Marozkina   +9 more
wiley   +1 more source