Cargando…

A survey: Understanding the health and perspectives of people with CF not benefiting from CFTR modulators

BACKGROUND: While the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator use has improved daily life and long‐term prognosis of CF for many with approved CFTR mutations, approximately 10% of people with CF (pwCF) have only symptomatic treatments available. METHODS: Betwee...

Descripción completa

Detalles Bibliográficos
Autores principales:	Kramer‐Golinkoff, Emily, Camacho, Amanda, Kramer, Liza, Taylor‐Cousar, Jennifer L.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	John Wiley and Sons Inc. 2022
Materias:	ORIGINAL ARTICLES
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9314897/ https://www.ncbi.nlm.nih.gov/pubmed/35170259 http://dx.doi.org/10.1002/ppul.25859

Ejemplares similares

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era
por: Jain, Raksha, et al.
Publicado: (2021)

Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease
por: Shteinberg, Michal, et al.
Publicado: (2020)

New therapies for people with CF in the CFTR modulator world
por: Wainwright, Claire E
Publicado: (2020)

CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis
por: Taylor-Cousar, Jennifer L.
Publicado: (2020)

CF Fungal Disease in the Age of CFTR Modulators
por: Bercusson, Amelia, et al.
Publicado: (2021)

Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages
por: Aridgides, Daniel S., et al.
Publicado: (2023)

Neutrophil Extracellular Trap (NET)-Mediated Killing of Pseudomonas aeruginosa: Evidence of Acquired Resistance within the CF Airway, Independent of CFTR
por: Young, Robert L., et al.
Publicado: (2011)

Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators
por: Muilwijk, Danya, et al.
Publicado: (2021)

Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation
por: Rowe, Steven M., et al.
Publicado: (2013)

Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function
por: Clancy, John P., et al.
Publicado: (2013)

Sweat rate analysis of ivacaftor potentiation of CFTR in non-CF adults
por: Kim, Jeeyeon, et al.
Publicado: (2018)

Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design
por: Jain, Raksha, et al.
Publicado: (2022)

Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene
por: Xu, Jie, et al.
Publicado: (2021)

CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France
por: Regard, Lucile, et al.
Publicado: (2022)

Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease
por: Centorame, Amanda, et al.
Publicado: (2022)

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles
por: Taylor-Cousar, Jennifer L., et al.
Publicado: (2019)

Mutations that permit residual CFTR function delay acquisition of multiple respiratory pathogens in CF patients
por: Green, Deanna M, et al.
Publicado: (2010)

Two novel and correlated CF-causing insertions in the (TG)mTn tract of the CFTR gene
por: Pierandrei, Silvia, et al.
Publicado: (2019)

Extensive CFTR Gene Analysis Revealed a Higher Occurrence of Cystic Fibrosis Transmembrane Regulator-Related Disorders (CFTR-RD) among CF Carriers
por: Esposito, Maria Valeria, et al.
Publicado: (2020)

239: Perspectives of people with CF and caregivers regarding medication access and telehealth in CF care during the COVID-19 pandemic
por: Phan, H., et al.
Publicado: (2021)

What can the CF registry tell us about rare CFTR-mutations? A Belgian study
por: De Wachter, E., et al.
Publicado: (2017)

Pharmacological Responses of the G542X-CFTR to CFTR Modulators
por: Fang, Xinxiu, et al.
Publicado: (2022)

CFTR modulator therapies – Effect on life expectancy in people with cystic fibrosis
por: Balfour-Lynn, I.M., et al.
Publicado: (2022)

Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype
por: Gillan, Jonathan L, et al.
Publicado: (2022)

Transcriptomic analysis of lung development in wildtype and CFTR(−/−) sheep suggests an early inflammatory signature in the CF distal lung
por: Kerschner, Jenny L., et al.
Publicado: (2023)

P256 Impact of COVID-19 pandemic and introduction of CFTR modulator therapy on sputum sampling in a large adult cystic fibrosis (CF) unit
por: Gaffney, K., et al.
Publicado: (2023)

Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation
por: Pohl, Kerstin, et al.
Publicado: (2018)

Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry
por: Yiallouros, Panayiotis K., et al.
Publicado: (2021)

Of muscle modulation and the CFTR gate
por: Adler, Elizabeth M.
Publicado: (2015)

The PROSPECT Is Bright for CFTR Modulators
por: Montemayor, Kristina, et al.
Publicado: (2021)

Survey of CF mutations in the clinical laboratory
por: Huber, Klaus Roland, et al.
Publicado: (2002)

Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes
por: Lurquin, Fabian, et al.
Publicado: (2023)

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells
por: Veltman, Mieke, et al.
Publicado: (2021)

Modulation of CFTR gating by permeant ions
por: Yeh, Han-I, et al.
Publicado: (2015)

CFTR Lifecycle Map—A Systems Medicine Model of CFTR Maturation to Predict Possible Active Compound Combinations
por: Vinhoven, Liza, et al.
Publicado: (2021)

492: CF innate immune defect affects CF intestinal microbiota
por: Scull, C., et al.
Publicado: (2021)

Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies
por: Gentzsch, Martina, et al.
Publicado: (2021)

G551D and G1349D, Two CF-associated Mutations in the Signature Sequences of CFTR, Exhibit Distinct Gating Defects
por: Bompadre, Silvia G., et al.
Publicado: (2007)

Beyond the Local Basic Panel: Full CFTR Gene Analysis Identifies Novel CF Mutation Missed on Standard Testing in an Arabic Child
por: Francis, Nader, et al.
Publicado: (2023)

CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity
por: Kramer, Elizabeth L., et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_0e56c3nd52poltn6pg61mok8am