Results 91 to 100 of about 6,598 (226)

Home Microbiological Sampling in a Pediatric Cystic Fibrosis Population: Pandemic Implementation and Ongoing Use

Pediatric Pulmonology, Volume 60, Issue 11, November 2025.
ABSTRACT Background During the coronavirus disease 2019 (COVID19) pandemic, restrictions on in‐person care threatened to disrupt in‐clinic airway sampling for microbiological surveillance, a vital aspect of cystic fibrosis (CF) care. In response, institutions developed home airway sampling strategies to allow continued guidelines‐based microbiological ...
Valerie Swanston, Jeffrey N. Bone, Melissa Richmond, David Goldfarb, Jonathan H. Rayment   +4 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

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

Standards for the care of people with cystic fibrosis; establishing and maintaining health [PDF]

, 2023
This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health.
Addy, C, Bell, S, Bertrand, DP, Bevan, A, Borawska, U, Brown, C, Burgel, PR, Button, B, Castellani, C, Chansard, A, Chilvers, M, Davies, G, Davies, J, De Boeck, K, Declercq, D, Doumit, M, Drevinek, P, Fajac, I, Gartner, S, Georgiopoulos, A, Gramegna, A, Gursli, S, Hansen, CM, Hug, M, Lammertyn, E, Landau, EC, Langley, R, Mayer-Hamblett, N, Middleton, A, Middleton, P, Mielus, M, Morrison, L, Munck, A, Plant, B, Ploeger, M, Pressler, T, Quon, BS, Radtke, T, Saynor, ZL, Shufer, I, Smith, C, Smyth, AR, Southern, K, Van Koningsbruggen-Rietschel, S   +43 more
core  

Mejoría de la inflamación intestinal tras tratamiento con moduladores de la proteína CFTR en pacientes con fibrosis quística

Anales de Pediatría
Resumen: Introducción: Los tratamientos con moduladores de la proteína CFTR han mejorado la salud respiratoria y digestiva de los pacientes con fibrosis quística.
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   +14 more
doaj   +1 more source

Functional Pulmonary Imaging

Journal of Magnetic Resonance Imaging, Volume 62, Issue 4, Page 986-1008, October 2025.
ABSTRACT The aging of the world population gave rise to an increased prevalence of many lung diseases, with chronic obstructive pulmonary disease now ranking as the third‐leading cause of death according to the World Health Organization. To diagnose lung disease, a thorough assessment of lung function is essential since it may reveal unique signatures ...
Agilo L. Kern, Filip Klimeš, Andreas Voskrebenzev, Hoen‐Oh Shin, Jens Vogel‐Claussen   +4 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

A pediatric cystic fibrosis arthropathy case who responded to Elexacaftor/Tezacaftor/Ivacaftor therapy [PDF]

, 2023
Meliksah Arslan, Zeynep Bahadır, Matthew L. Basiaga, Sarah Chalmers, Nadir Demirel   +4 more
openalex   +1 more source

Phenotype‐Genotype Correlations in ABCA3 Patients—The RespiRare Cohort

Pediatric Pulmonology, Volume 60, Issue 10, October 2025.
ABSTRACT Background ATP‐binding cassette transporter A3 (ABCA3) deficiency is one of the most severe causes of childhood interstitial lung diseases (chILD). This study aims to report the RespiRare ABCA3 cohort and to establish phenotype‐genotype correlations. Methods Phenotypic and genotypic data of patients under 18 years were retrospectively included
Manon Fleury, Céline Delestrain, Alice Hadchouel, Julie Mazenq, Myriam Benhamida, Anne‐Sophie Bernard, Raphaël Borie, Jacques Brouard, Harriet Corvol, Pierrick Cros, Christophe Delacourt, Tifenn Desroziers, Jean‐Christophe Dubus, Carole Egron, Ralph Epaud, Michael Fayon, Aude Forgeron, Lisa Giovannini‐Chami, Christophe Marguet, Alexandra Masson‐Rouchaud, Hortense Petat, Marie‐Catherine Renoux, Léa Roditis, Caroline Thumerelle, Clémentine Vigier, Aurore Coulomb L'Herminé, Hubert Ducou le Pointe, Pascale Fanen, Camille Fletcher, Chiara Sileo, Laureline Berteloot, Camille Louvrier, Alix de Becdelièvre, Marie Legendre, Nadia Nathan   +34 more
wiley   +1 more source

Cystic Fibrosis Care in South Africa: Facing the Challenge of Diversity and Inequality

Pediatric Pulmonology, Volume 60, Issue 10, October 2025.
ABSTRACT Low‐and‐middle‐income countries (LMIC) like South Africa (SA) were left behind with advancements in cystic fibrosis (CF) care that followed re‐imbursement agreements in high‐income countries of CF transmembrane regulator protein modulators (CFTRm) for the treatment of CF.
Marco Zampoli, Cathy Baird, Brenda Morrow, Janine Verstraete, Greg Calligaro, Mark Seale   +5 more
wiley   +1 more source