E. McKone

发表

Relationship between pulmonary hyperinflation and dyspnoea severity during acute exacerbations of cystic fibrosis

C. Gallagher, T. Nicholson, P. Barry, 2017, Respirology.

Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalities.

J. Cavanaugh, E. Hoffman, M. Welsh, 2016, JCI insight.

Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.

M. Welsh, D. Stoltz, J. Zabner, 2018, JCI insight.

Macrophage migration inhibitory factor enzymatic activity, lung inflammation, and cystic fibrosis.

L. Leng, G. Fingerle-Rowson, R. Bucala, 2012, American journal of respiratory and critical care medicine.

Tocilizumab therapy in individuals with COVID‐19 infection and hyperinflammatory state

M. Butler, S. Ryan, M. Keane, 2020, Respirology.

Pleural effusion arising from a rare pancreatic neoplasm

J. Dodd, C. Gallagher, J. Geoghegan, 2012, European Respiratory Journal.

Targeting the PI3K/Akt/mTOR signalling pathway in Cystic Fibrosis

C. Behl, S. Keely, K. Wynne, 2017, Scientific Reports.

Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial

C. Teneback, P. Sosnay, R. Klingsberg, 2019, The Lancet.

Longitudinal Trends in Real‐World Outcomes after Initiation of Ivacaftor. A Cohort Study from the Cystic Fibrosis Registry of Ireland

B. Casserly, N. McElvaney, P. Greally, 2019, Annals of the American Thoracic Society.

Lung function decline is delayed but not decreased in patients with cystic fibrosis and the R117H gene mutation.

C. Goss, G. Sawicki, W. Morgan, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study.

C. Goss, G. Sawicki, D. Waltz, 2017, The Lancet. Respiratory medicine.

Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study

C. Goss, H. Rodgers, P. Burgel, 2022, Journal of Cystic Fibrosis.

Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T.

C. Gallagher, S. Carter, B. Grogan, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis

C. Haslett, R. Gray, D. Davidson, 2017, Thorax.

The global impact of SARS-CoV-2 in 181 people with cystic fibrosis

C. Goss, H. Rodgers, P. Burgel, 2020, Journal of Cystic Fibrosis.

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines.

J. Pilewski, A. Faro, Patrick J. Smith, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

VX‐445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

F. van Goor, D. Waltz, E. Tullis, 2018, The New England journal of medicine.

Patients with Cystic Fibrosis and a G551D or Homozygous F508del Mutation: Similar Lung Function Decline.

G. Sawicki, M. Konstan, D. Pasta, 2017, American journal of respiratory and critical care medicine.

CF pulmonary exacerbations-Steps in the right direction.

S. Carter, E. McKone, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Molecular detection of an atypical, highly resistant, clonal Pseudomonas aeruginosa isolate in cystic fibrosis patients.

C. Gallagher, K. Schaffer, B. Kennedy, 2013, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Pulmonary blastoma: a case report and review of the literature

A. Fabre, C. Gallagher, R. Smyth, 2014, BMC Research Notes.

Adrenal insufficiency and testicular failure secondary to megestrol acetate therapy in a patient with cystic fibrosis

M. Tonelli, M. Aitken, E. McKone, 2002, Pediatric pulmonology.

A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis

C. Goss, H. Rodgers, P. Burgel, 2020, Journal of Cystic Fibrosis.

Recall of provision of sun protection measures to patients with cystic fibrosis being prescribed voriconazole

L. Cunningham, A. Lally, E. McKone, 2015, Photodermatology, photoimmunology & photomedicine.

Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.

L. Lands, D. Waltz, P. Dřevínek, 2019, The New England journal of medicine.

The future of cystic fibrosis care: a global perspective.

C. Goss, P. Burgel, M. Macek, 2020, The Lancet. Respiratory medicine.

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Matthias Griese, P. Dřevínek, N. McElvaney, 2011, The New England journal of medicine.

Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study

S. Emerson, K. Edwards, M. Aitken, 2003, The Lancet.

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Xiaohong Huang, Michael P Boyle, Carla Colombo, 2015, The New England journal of medicine.

Pathway analysis of a genome-wide gene by air pollution interaction study in asthmatic children

J. Schwartz, D. Postma, T. Lumley, 2019, Journal of Exposure Science & Environmental Epidemiology.

Ambient air pollution, lung function, and airway responsiveness in asthmatic children.

Antonella Zanobetti, Joel Schwartz, Thomas Lumley, 2016, The Journal of allergy and clinical immunology.

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles

D. Waltz, B. Ramsey, E. Tullis, 2019, ERJ Open Research.

Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis

D. Matallanas, S. Donnelly, S. Carter, 2020, Thorax.

Lemierre’s syndrome

D. O’Dwyer, J. Lyons, L. Lavelle, 2011, Irish journal of medical science.

Aggressive prenatal care results in successful fetal outcomes in CF women.

C. Goss, V. Galic, M. Tonelli, 2006, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

ECFS Patient Registry : annual data Report : 2013 data

A. Zolin, E. McKone, J. V. Rens, 2016 .

Ivacaftor modifies cystic fibrosis neutrophil phenotype in subjects with R117H residual function CFTR mutations

R. Gray, Pradeep K. Singh, S. Carter, 2020, European Respiratory Journal.

Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del

E. Ingenito, E. Mckone, A. Munck, 2017, The New England journal of medicine.

Tracheomalacia in Adults with Cystic Fibrosis: Determination of Prevalence and Severity with Dynamic Cine CT.

H. Tiddens, P. D. de Jong, J. Dodd, 2015, Radiology.

Tracheomalacia in adults with cystic fibrosis: determination of prevalence and severity with dynamic cine CT.

H. Tiddens, P. D. de Jong, J. Dodd, 2009, Radiology.

Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.

Jing Shao, T. Kavanagh, M. Tonelli, 2006, American journal of respiratory and critical care medicine.

Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis

W. Liles, S. Carter, B. Grogan, 2020, ERJ Open Research.

Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe

M. Mei-Zahav, P. Yiallouros, P. Dřevínek, 2021, ERJ Open Research.

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections

L. Hoffman, M. Welsh, J. Bruce, 2017, American journal of respiratory and critical care medicine.

CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis

S. Gharib, W. Liles, S. Carter, 2020, Frontiers in Pharmacology.

VX‐659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

D. Prais, F. van Goor, D. Waltz, 2018, The New England journal of medicine.

Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).

Ying Jiang, Barry Plant, Matthias Griese, 2014, The Lancet. Respiratory medicine.

Correlation between Ivacaftor-induced CFTR Activation in Airway Epithelial Cells and Improved Lung Function: A Proof-of-Concept.

E. Mckone, Pradeep K. Singh, K. Barrow, 2020, Annals of the American Thoracic Society.

Position paper: Models of post-transplant care for individuals with cystic fibrosis.

M. Anstead, J. Pilewski, J. Blatter, 2023, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections

C. Goss, M. Welsh, S. Salipante, 2021, mBio.

Inflammatory cytokines TNFα and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators.

M. Welsh, D. Stoltz, Pradeep K. Singh, 2021, The Journal of clinical investigation.

Safety and efficacy of vanzacaftor-tezacaftor-deutivacaftor in adults with cystic fibrosis: randomised, double-blind, controlled, phase 2 trials.

P. Sosnay, F. van Goor, D. Waltz, 2023, The Lancet. Respiratory medicine.

A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele

R. Gibson, S. McColley, D. Waltz, 2021, American journal of respiratory and critical care medicine.

Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial.

R. Fischer, S. Mulrennan, D. Waltz, 2021, The Lancet. Respiratory medicine.

Efficacy of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease

C. Gallagher, B. Grogan, T. Nicholson, 2020, European Respiratory Journal.

Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.

D. Waltz, B. Ramsey, E. Tullis, 2021, The New England journal of medicine.

Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for 24 Weeks or Longer in People with Cystic Fibrosis and One or More F508del Alleles: Interim Results of an Open-Label Phase 3 Clinical Trial

M. Griese, S. Moskowitz, B. Quon, 2020, American journal of respiratory and critical care medicine.

565: Significant reduction in abdominal symptoms assessed with CFAbd score over 4 weeks of treatment with elexacaftor/tezacaftor/ivacaftor—First results from the RECOVER study

J. Mainz, J. Davies, B. Linnane, 2021, Journal of Cystic Fibrosis.

170 Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from an open-label extension study

K. Poplawska, E. Tullis, M. Chilvers, 2022, Journal of Cystic Fibrosis.

Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data.

G. Sawicki, M. Konstan, D. Pasta, 2015, American journal of respiratory and critical care medicine.

Association of sweat chloride concentration at time of diagnosis and CFTR genotype with mortality and cystic fibrosis phenotype.

C. Goss, P. Velentgas, E. McKone, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

M. Mei-Zahav, P. Yiallouros, P. Burgel, 2021, Journal of Cystic Fibrosis.

GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).

N. Derichs, P. Dřevínek, J. Davies, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Repeatability of transient elastography in children

D. Cox, P. Greally, M. Ní Chróinín, 2020, Pediatric Research.

absence of the vas deferens and their association with cystic fibrosis genetic modifiers Heterogeneous spectrum of mutations in CFTR gene from Indian patients with congenital

M. Drumm, K. Goddard, N. Kalsheker, 2015 .

EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.

R. Gibson, M. Drumm, K. Goddard, 2010, Physiological genomics.

Chest CT abnormalities and quality of life: relationship in adult cystic fibrosis.

H. Tiddens, J. Dodd, A. Kilcoyne, 2016, Annals of translational medicine.

Role of arterial hypoxemia and pulmonary mechanics in exercise limitation in adults with cystic fibrosis.

C. Gallagher, M. FitzGerald, S. Barry, 2005, Journal of applied physiology.

A treatment evaluator tool to monitor the real-world effectiveness of inhaled aztreonam lysine in cystic fibrosis.

E. Mckone, J. Eustace, D. Bilton, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Androgens, exercise capacity, and muscle function in cystic fibrosis.

C. Gallagher, T. Mckenna, P. Barry, 2008, Chest.

Validation and use of a parametric model for projecting cystic fibrosis survivorship beyond observed data: a birth cohort analysis

H. Quinton, P. Fitzpatrick, C. Kelleher, 2011, Thorax.

The role of supplemental oxygen during submaximal exercise in patients with cystic fibrosis

C. Gallagher, S. Barry, E. McKone, 2002, European Respiratory Journal.

Development, validation, and implementation of a questionnaire assessing disease knowledge and understanding in adult cystic fibrosis patients.

K. R. Siklosi, C. Gallagher, E. McKone, 2010, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Reproducibility of maximal exercise ergometer testing in patients with cystic fibrosis.

C. Gallagher, M. FitzGerald, S. Barry, 1999, Chest.

Macrophage migration inhibitory factor enhances Pseudomonas aeruginosa biofilm formation, potentially contributing to cystic fibrosis pathogenesis

J. Bernhagen, L. Leng, R. Bucala, 2017, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry cohort study

R. Keogh, M. Macek, C. Ariti, 2021, European Respiratory Journal.

Differentiating combined pulmonary fibrosis and emphysema from pure emphysema: utility of late gadolinium-enhanced MRI

D. Lynch, S. Abbara, M. Butler, 2020, European Radiology Experimental.

A population-level strain genotyping method to study pathogen strain dynamics in human infections

S. Salipante, M. Radey, S. Durfey, 2021, bioRxiv.

High-resolution CT of nontuberculous mycobacterium infection in adult CF patients: diagnostic accuracy

H. Tiddens, J. Dodd, C. Gallagher, 2012, European Radiology.

Estimating Direct Cost of Cystic Fibrosis Care Using Irish Registry Healthcare Resource Utilisation Data, 2008–2012

G. Doyle, F. Cullinane, A. Jackson, 2017, PharmacoEconomics.

Pharmacogenetics of cystic fibrosis treatment.

S. Carter, E. McKone, 2016, Pharmacogenomics.

CFTR genotype as a predictor of prognosis in cystic fibrosis.

C. Goss, E. Mckone, M. Aitken, 2006, Chest.

Experience using centralized spirometry in the phase 2 randomized, placebo-controlled, double-blind trial of denufosol in patients with mild to moderate cystic fibrosis.

C. Goss, J. Wanger, S. Millard, 2008, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Electrical Muscle Stimulation (EMS) In Cystic Fibrosis

C. Gallagher, L. Crowe, B. Caulfield, 2010, ATS 2010.

Ivacaftor-Induced Proteomic Changes Suggest Monocyte Defects May Contribute to the Pathogenesis of Cystic Fibrosis.

M. Welsh, C. Gallagher, Pradeep K. Singh, 2016, American journal of respiratory cell and molecular biology.

COVID-19 vaccine prioritisation for people with cystic fibrosis

C. Goss, P. Burgel, M. Salvatore, 2021, Journal of Cystic Fibrosis.

WS6.4 Long-term safety and efficacy of ivacaftor in subjects with cystic fibrosis who have the G551D-CFTR Mutation

J. Davies, Karl Yen, E. McKone, 2012 .

Use of the gas exchange threshold to noninvasively determine the lactate threshold in patients with cystic fibrosis.

P. McLoughlin, C. Gallagher, A. Thin, 2002, Chest.

WS3.1 The effect of ivacaftor on the rate of lung function decline in CF patients with a G551D-CFTR mutation

G. Sawicki, M. Konstan, D. Pasta, 2014 .

Personalised medicine in advanced cystic fibrosis.

E. McKone, 2016, The Lancet. Respiratory medicine.

Reduction in abdominal symptoms (CFAbd-Score), faecal M2-pyruvate-kinase and Calprotectin over one year of treatment with Elexacaftor-Tezacaftor-Ivacaftor in people with CF aged >=12 years - The RECOVER study

J. Mainz, D. Cox, J. Davis, 2023, medRxiv.

Rate of Lung Function Decline in People with Cystic Fibrosis Having a Residual Function Gene Mutation

C. Goss, G. Sawicki, W. Morgan, 2022, Pulmonary Therapy.

Obliterating Airway Bronchus Sign of Occult Malignancy in Sarcoid Conglomerate Fibrotic Masses

J. Dodd, D. Murphy, N. McVeigh, 2021, Journal of clinical imaging science.

Cystic fibrosis: disease mechanisms and therapeutic targets

M. Aitken, E. McKone, 2004 .

Early life nasal microbiota in infants with cystic fi brosis

E. McKone, 2016 .

Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2–5 Years with Cystic Fibrosis and at Least One F508del Allele

M. Rosenfeld, S. McColley, B. Ramsey, 2023, American journal of respiratory and critical care medicine.

Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: A retrospective registry cohort study.

L. Kirwan, A. Jackson, G. Fletcher, 2020, Journal of Cystic Fibrosis.

Tezacaftor – Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe 508 del Alleles

D. Prais, B. Ramsey, J. Davies, 2020 .

Acute Pulmonary Exacerbation Phenotypes in Patients with Cystic Fibrosis.

S. Coughlan, C. Gallagher, S. Carter, 2022, Annals of the American Thoracic Society.

Ambient air pollution

J. Schwartz, D. Postma, T. Lumley, 2016 .

Publications and Extramural Activities of General Internal Medicine and Medicine Subspecialty Clinician-Educators: A Multicenter Study

G. Rubenfeld, M. Tonelli, C. Scott, 2005, Academic medicine : journal of the Association of American Medical Colleges.

Comparison of Scholarly Productivity of General and Subspecialty Clinician-Educators in Internal Medicine

G. Rubenfeld, M. Tonelli, C. Scott, 2004, Teaching and learning in medicine.

Advancing the pipeline of cystic fibrosis clinical trials: a new roadmap with a global trial network perspective.

J. Pilewski, S. Donaldson, M. Konstan, 2023, The Lancet. Respiratory medicine.

EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.

R. Gibson, M. Drumm, K. Goddard, 2010, Physiological genomics.

Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.

M. Welsh, D. Stoltz, J. Zabner, 2018, JCI Insight.

Investigating serum extracellular vesicles in Cystic Fibrosis.

S. Carter, E. Dillon, J. Coppinger, 2023, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.

M. Wilschanski, J. Bouchara, S. van Koningsbruggen-Rietschel, 2024, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Elexacaftor/Tezacaftor/Ivacaftor Treatment and Depression-related Events

D. DeMaso, J. Taylor-Cousar, E. McKone, 2023, American Journal of Respiratory and Critical Care Medicine.

ePS03.4 Improved rate of decline in percent predicted FEV1 (ppFEV1) is not associated with acute improvement in ppFEV1 in patients with cystic fibrosis (CF) treated with ivacaftor

G. Sawicki, M. Konstan, D. Pasta, 2016 .

J. Mainz, D. Cox, B. Linnane, 2023, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.