Results 51 to 60 of about 2,330 (177)

Cystic fibrosis modulator therapy can reverse cystic bronchiectasis

Respirology Case Reports, 2023
Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms.
Peter G. Middleton, Nicholas J. Simmonds
doaj   +1 more source

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

Frontiers in Physiology, 2021
A deficiency in cystic fibrosis transmembrane conductance regulator (CFTR) function in CF leads to chronic lung disease. CF is associated with abnormalities in fatty acids, ceramides, and cholesterol, their relationship with CF lung pathology is not ...
Mieke Veltman, Mieke Veltman, Juan B. De Sanctis, Marta Stolarczyk, Nikolai Klymiuk, Nikolai Klymiuk, Andrea Bähr, Andrea Bähr, Rutger W. Brouwer, Rutger W. Brouwer, Edwin Oole, Edwin Oole, Juhi Shah, Tomas Ozdian, Jie Liao, Carolina Martini, Danuta Radzioch, John W. Hanrahan, John W. Hanrahan, Bob J. Scholte, Bob J. Scholte   +20 more
doaj   +1 more source

Comparison of Single‐Breath and Multi‐Breath Xe‐MRI in the Longitudinal Assessment of Treatment in Children With Cystic Fibrosis

Journal of Magnetic Resonance Imaging, Volume 63, Issue 6, Page 1652-1661, June 2026.
ABSTRACT Background Elexacaftor/tezacaftor/ivacaftor (ETI) is a current standard therapy for pediatric cystic fibrosis (CF). Multiple‐breath washout 129Xe MRI (MBW Xe‐MRI) is improved following 1 month of treatment. However, the utility of MBW Xe‐MRI over extended ETI treatment and its comparison to single‐breath Xe‐MRI and pulmonary function tests ...
Faiyza S. Alam, Samal Munidasa, Brandon Zanette, Daniel Li, Sharon Braganza, Felix Ratjen, Giles Santyr   +6 more
wiley   +1 more source

Improvement of intestinal inflammation after treatment with CFTR modulators in cystic fibrosis patients

Anales de Pediatría (English Edition)
Introduction: Treatments with CFTR protein modulators have improved respiratory and digestive health in patients with cystic fibrosis. Objective: To assess changes in intestinal inflammation through the analysis of fecal calprotectin in patients with ...
Ruth García Romero, Concepción López Cárdenes, Elena Crehuá Gaudiza, Marina Álvarez Beltrán, Mercedes Murray Hurtado, Carlos Tutau Gómez, Inés Loverdos Eseverri, Encarni Torcuato Rubio, Camila García Volpe, Enrique Salcedo Lobato, María Medina Martínez, Carmen Martin Fernández, Ana Moreno Álvarez, Ana Reyes Domínguez, David González Jiménez, Saioa Vicente Santamaría, Ana Tabares González, Jose Ramon Gutierrez Martinez, Sara Sierra San Nicolás, Pilar Ortiz Pérez, Luis Peña Quintana   +20 more
doaj   +1 more source

Clinical Use of Home Spirometry in Children With Cystic Fibrosis

Pediatric Pulmonology, Volume 61, Issue 6, June 2026.
ABSTRACT Background The use of home spirometry (HSPIR) has increased in pediatric cystic fibrosis (CF) care, but how it has been used clinically and its impact on clinical care have not been described. The purpose of this study was to address this knowledge gap through a secondary analysis of data from a HSPIR quality improvement project to ...
Lucy Tan, Andrew Borowiec, James A. Reed, Mark Rath, Reid Masi, Laura Bennett, Don B. Sanders, Daniel J. Weiner, Clement L. Ren   +8 more
wiley   +1 more source

Divergent neurobehavioral effects of CFTR modulators elexacaftor and ivacaftor in mice

Acta Psychologica
Recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator combination therapies have markedly improved survival and quality of life for people with cystic fibrosis (CF).
Qian Ge, Amy Fagan, Hui Lu, Jianyang Du, Leah R. Reznikov   +4 more
doaj   +1 more source

Performance Evaluation of a Novel Cystic Fibrosis Caregiver Burden Measure Demonstrates Significant Challenges for Parents of Children With CF During the Early Years: The Irish Comparative Outcomes Study (ICOS)

Pediatric Pulmonology, Volume 61, Issue 6, June 2026.
ABSTRACT Background As a part of the Irish Comparative Outcome national cohort study of childhood CF, this cross‐sectional study investigated challenges faced by parents of children with CF (CWCF) in the Republic of Ireland using a newly validated modified tool. Methods Parents completed the modified “Challenge of Living with Cystic Fibrosis‐Short Form”
Rini Bhatnagar, Nancy Bhardwaj, Gwinyai Masukume, Ricardo Segurado, Barry Linnane, Mary Herzig, Muireann Ni Chronin, David Mullane, Des W. Cox, Paul McNally, Sheila Javadpour, Ruth O'Reilly, Basil Elnazir, Peter Greally, Fiona Healy, Michael Williamson, Dubhfeasa Slattery, Gareth McCray, Gillian Lancaster, Claire Glasscoe, Kevin W. Southern, Laura Kirwan, Patricia Fitzpatrick   +22 more
wiley   +1 more source

Organic Synthesis and Current Understanding of the Mechanisms of CFTR Modulator Drugs Ivacaftor, Tezacaftor, and Elexacaftor

Molecules
The monogenic rare disease Cystic Fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance (CFTR) protein, an anion channel expressed at the apical plasma membrane of epithelial cells.
Filipa C. Ferreira, Camilla D. Buarque, Miquéias Lopes-Pacheco   +2 more
doaj   +1 more source

Elexacaftor-Tezacaftor-Ivacaftor in 2 cystic fibrosis adults homozygous for M1101K with end-stage lung disease

Respiratory Medicine Case Reports, 2023
Elexacaftor-tezacaftor-ivacaftor (ETI) therapy is shown to improve the health of individuals with cystic fibrosis (CF) who have the F508del variant. There are in vitro studies showing benefit with ETI for select rare CF variants.
Winnie M. Leung, Parastoo Molla Davoodi, Ashten Langevin, Clare Smith, Michael D. Parkins   +4 more
doaj   +1 more source

Improved Quality of Life in Children With Cystic Fibrosis Who Received Transmembrane Conductance Regulator Modulators

Acta Paediatrica, Volume 115, Issue 6, Page 1283-1289, June 2026.
ABSTRACT Aim Children with cystic fibrosis (CF) face substantial daily treatment burdens and the effects of transmembrane conductance regulator modulators on these have not been sufficiently described. We evaluated changes in treatment burden after elexacaftor tezacaftor ivacaftor (ETI) was initiated.
Marcus Svedberg, Frida Lundqvist, Huda Abdulahi Östrand, Christina Krantz, Per Ekman, Henrik Imberg   +5 more
wiley   +1 more source