Results 81 to 90 of about 6,985 (222)

Challenges to Assessing the Prevalence of Cystic Fibrosis in the Caribbean

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Cystic fibrosis (CF) is likely underdiagnosed in Caribbean populations due to non‐representative cystic fibrosis transmembrane conductance regulator (CFTR) variant screening panels, limited newborn screening programs, and structural healthcare barriers.
Krystal L. Rivera‐Figueroa, Cole L. Martin, Christina A. Le, Wilfredo De Jesús Rojas, Leandra Cordero Oñate, Stephen G. Aller   +5 more
wiley   +1 more source

Efficacy of Trikafta (ELX/TEZ/IVA) & Symdeko (TEZ/IVA) in Treating Cystic Fibrosis with F508del Allele: A Systematic Review and Meta-analysis

Thoracic Research and Practice
The objective of the study was to assess and compare the efficacy of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment with TEZ/IVA treatment in individuals diagnosed with cystic fibrosis (CF) and carrying the F508del allele.
Kainat Hussain, Sonali Karhana, Aakriti Garg, Mohd Ashif Khan   +3 more
doaj   +1 more source

Elexacaftor-Tezacaftor-Ivacaftor as a Final Frontier in the Treatment of Cystic Fibrosis: Definition of the Clinical and Microbiological Implications in a Case-Control Study

Pharmaceuticals, 2022
The use of modulator drugs that target the Cystic Fibrosis transmembrane conductance regulator (CFTR) is the final frontier in the treatment of Cystic Fibrosis (CF), a genetic multiorgan disease.
Giuseppe Migliorisi, Mirella Collura, Francesca Ficili, Tiziana Pensabene, Dafne Bongiorno, Antonina Collura, Francesca Di Bernardo, Stefania Stefani   +7 more
doaj   +1 more source

EPS6.03 Pulmonary pathogen prevalence 12 months after elexacaftor/tezacaftor/ivacaftor introduction: results from the Danish National Cystic Fibrosis Cohort [PDF]

, 2023
M. Jeppesen, S. Jensen-Fangel, Camilla Bjørn Jensen, Terese L. Katzenstein, M. Wang, M. Frahm Olesen, T. Pressler, Marianne Skov, Hanne Vebert Olesen, Tavs Qvist   +9 more
openalex   +1 more source

Utility of Fecal Elastase‐1 in Estimating Exocrine Pancreatic Function in Cystic Fibrosis: A Scoping Review

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Background In people with cystic fibrosis (pwCF), identification of exocrine pancreatic insufficiency (EPI) is essential to prevent steatorrhea and, if not managed actively, can lead to catastrophic consequences. Fecal elastase‐1 (FE‐1) is a widely used test to screen for EPI in cystic fibrosis (CF).
Senthilkumar Sankararaman, Sara Hendrix, Mandy Neudecker, Drucy Borowitz   +3 more
wiley   +1 more source

Elexacaftor/tezacaftor/ivacaftor as rescue therapy in a patient with the cystic fibrosis genotype F508DEL/G1244E

Clinical Case Reports, 2021
Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane regulator (CFTR) modulator. It is known to be efficacious in stable patients with severe pneumopathy, but there are few data concerning its effectiveness during acute ...
Donatello Salvatore, Carmela Colangelo, Michele D’Andria, Giovanni Marsicovetere, Domenica Passarella   +4 more
doaj   +1 more source

WS05.05 Randomised withdrawal of hypertonic saline in those with lower lung function after receiving elexacaftor/tezacaftor/ivacaftor; a sub-study of the SIMPLIFY Trial [PDF]

, 2023
David P. Nichols, Alex H. Gifford, Margaret Kloster, Richard Russell, J. Young, Rodolfo Amaro-Galvez, Joanne Billings, Zubin Mukadam, John P. Clancy, Nicole Mayer-Hamblett   +9 more
openalex   +1 more source

Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Rationale Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by neutrophilic airway inflammation but differ in clinical features. Objectives We investigated relationships of pulmonary and systemic inflammatory markers with functional and structural lung disease.
Teresa Fuchs, Jacqueline Donovan, Andrew Ives, Samantha Irving, Gwyneth Davies, Claire Hogg, Eric Alton, Gemma Wilson, Andrew Bush, Jane C. Davies   +9 more
wiley   +1 more source

Tezacaftor is a direct inhibitor of sphingolipid delta-4 desaturase enzyme (DEGS) [PDF]

Background: We recently demonstrated that 48 h exposure of primary human bronchial epithelial (hBE) cells, obtained from both CF (F508del homozygous) and non-CF subjects, to the triple drug combination Elexacaftor/Tezacaftor/Ivacaftor (ETI) results in a ...
Armirotti, Andrea, Aureli, Massimo, Bandiera, Tiziano, Bassi, Rosaria, Bertorelli, Rosalia, Bertozzi, Sine Mandrup, Capurro, Valeria, Ciobanu, Dinu Zinovie, Dobi, Dorina, Liessi, Nara, Loberto, Nicoletta, Nobbio, Lucilla, Pedemonte, Nicoletta, Summa, Maria, Tomati, Valeria   +14 more
core   +1 more source

Colorectal Cancer Complicated by Pulmonary Embolism and Potential Takotsubo Cardiomyopathy in a Person With Cystic Fibrosis: Diagnostic and Management Considerations

Respirology Case Reports, Volume 14, Issue 5, May 2026.
We describe a complex case of pulmonary embolism and dilated cardiomyopathy in a person with cystic fibrosis. These late complications occurred following laparoscopic left hemicolectomy for colonic adenocarcinoma. This case highlights the complexities of evaluating breathlessness in severe lung disease, and the management challenges that arise as life ...
Laura Frederiksen, James Brown, Vanessa Moore, David W. Reid   +3 more
wiley   +1 more source