Results 61 to 70 of about 3,103 (172)

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

Longitudinal profiling of the intestinal microbiome in children with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor

mBio
The intestinal microbiome influences growth and disease progression in children with cystic fibrosis (CF). Elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA), the newest pharmaceutical modulator for CF, restores the function of the pathogenic mutated CF ...
Seth A. Reasoner, Rachel Bernard, Adam Waalkes, Kelsi Penewit, Janessa Lewis, Andrew G. Sokolow, Rebekah F. Brown, Kathryn M. Edwards, Stephen J. Salipante, Maria Hadjifrangiskou, Maribeth R. Nicholson   +10 more
doaj   +1 more source

A 3-Week Inpatient Rehabilitation Programme Improves Body Composition in People with Cystic Fibrosis with and Without Elexacaftor/Tezacaftor/Ivacaftor Therapy [PDF]

Background: The introduction of cystic fibrosis transmembrane conductance regulator modulators, especially the triple therapy elexacaftor, tezacaftor, ivacaftor (ETI), has improved outcomes in people with cystic fibrosis (pwCF), reducing underweight but ...
Christian Falkenberg, Gruber, Wolfgang, Koop, Jana, Stefan Dewey, Olaf Schnabel, Falkenberg, Christian, Schnabel, Olaf, Hägele, Franziska Anna, Norman, Kristina, Franziska A. Hägele, Herpich, Catrin, Hasler, Mario, Jana Koop, Wolfgang Gruber, Anja Bosy-Westphal, Weisser, Burkhard, Kristina Norman, Mario Hasler, Burkhard Weisser, Dewey, Stefan, Bosy-Westphal, Anja, Catrin Herpich   +21 more
core   +1 more source

Efficacy of Elexacaftor–Tezacaftor–Ivacaftor on chronic rhinosinusitis in cystic fibrosis

Purpose: Our work aims to add evidence on the effectiveness of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis. Materials and methods: We conducted an observational retrospective cohort study at the Cystic Fibrosis Center ...
Molteni, Gabriele, Tommasi, Nicola, Di Gioia, Stefano, Lucca, Francesca, Venditto, Laura, Cipolli, Marco, Sandri, Giulio   +6 more
core   +1 more source

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

ERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen, Daniel Faurholt-Jepsen, Tavs Qvist, Christine Højte, Bibi U. Nielsen, Thomas Bryrup, Esben H. Henriksen, Terese Katzenstein, Marianne Skov, Inger H.M. Mathiesen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne V. Olesen, Frederik Fouirnaies Buchvald, Kim Gjerum Nielsen, Espen Jimenez-Solem, Christian Ritz, Tacjana Pressler, Mette F. Olsen, the TransformCF Study Group   +19 more
doaj   +1 more source

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

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

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

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

The Impact of Cystic Fibrosis‐Related Diabetes on Barriers to Self‐Management

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Background Cystic fibrosis (CF) is a complex disease requiring adherence to an intensive medical regimen to maintain health. For those who develop CF‐related diabetes (CFRD, 20% adolescents, 30%–50% adults), daily self‐management is additionally complex and burdensome.
Rebecca J. Vitale, Christine Ford, Stephanie S. Filigno, Liam Sweeney, Carolyn Snell, Melissa S. Putman, Shipra Rai, Kristin A. Riekert   +7 more
wiley   +1 more source