Edward F McKone

Edward F McKone

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Edward F McKone

Edward F McKone

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

N Engl J Med 2019 11 31;381(19):1809-1819. Epub 2019 Oct 31.

From the Department of Respiratory and Sleep Medicine, Westmead Hospital and CF Research Group, Ludwig Engel Centre for Respiratory Research, Westmead Institute for Medical Research, University of Sydney, Westmead, NSW, Australia (P.G.M.); the Department of Pediatric Pulmonology, Immunology, and Intensive Care Medicine, Charité-Universitätsmedizin Berlin, the Berlin Institute of Health, and the German Center for Lung Research, Berlin (M.A.M.); the Department of Medical Microbiology, Department of Pediatrics, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic (P.D.); the Pediatric Respiratory Medicine and Pediatric Cystic Fibrosis Clinic, McGill University Health Centre, Montreal (L.C.L.); St. Vincent's University Hospital and University College Dublin School of Medicine, Dublin (E.F.M.); the Department of Internal Medicine, University of Kansas Medical Center, Kansas City (D.P.); the Department of Pediatrics, University of Washington School of Medicine, and Seattle Children's Research Institute, Seattle (B.W.R.); the Departments of Medicine and Pediatrics, National Jewish Health, Denver (J.L.T.-C.); the Division of Respirology, St. Michael's Hospital, University of Toronto, Toronto (E.T.); the Cystic Fibrosis Reference Center, Department of Pediatrics, Catholic University of Leuven, Leuven, Belgium (F.V.); Vertex Pharmaceuticals, Boston (G.M., C.M.M., S.M.M., N.N., J.S., C.S., S.T., D.W., F.X.); the Departments of Medicine, Pediatrics, and Cell, Developmental, and Integrative Biology, University of Alabama at Birmingham, Birmingham (S.M.R.); and the Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas (R.J.).

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http://dx.doi.org/10.1056/NEJMoa1908639DOI Listing
November 2019

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

J Expo Sci Environ Epidemiol 2019 Jun 26;29(4):539-547. Epub 2019 Apr 26.

Channing Division of Network Medicine, Brigham and Women's Hospital, Department of Medicine, Harvard Medical School, Boston, MA, United States.

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http://dx.doi.org/10.1038/s41370-019-0136-3DOI Listing
June 2019

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

J Cyst Fibros 2019 May 27;18(3):321-333. Epub 2019 Mar 27.

Division of Pulmonary, Allergy, Critical Care Medicine and Sleep, Dept of Medicine, University of Minnesota, Minneapolis, MN, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2019.03.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6545264PMC
May 2019

VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.

N Engl J Med 2018 10 18;379(17):1612-1620. Epub 2018 Oct 18.

From Alfred Hospital, Melbourne, VIC (D.K.), and Mater Hospital, Brisbane, QLD (L.B.) - both in Australia; Vertex Pharmaceuticals, Boston (G.M., C.M.M., S.M.M., S.R., J.S., C.S., F.V.G., D.W., F.X., T.Y.); Banner University Medical Center, Tucson, AZ (C.D.); Universitätsmedizin Berlin and Berlin Institute of Health, Berlin, and the German Center for Lung Research, Giessen - both in Germany (M.A.M.); St. Vincent's University Hospital and University College Dublin School of Medicine, Dublin (E.F.M.); Seattle Children's Hospital, Seattle (B.W.R.); University of Alabama at Birmingham, Birmingham (S.M.R.); Children's Hospital of the King's Daughters, Norfolk, VA (L.A.S.); St. Michael's Hospital, Toronto (E.T.); and National Jewish Health, Denver (J.L.T.-C.).

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http://www.nejm.org/doi/10.1056/NEJMoa1807120
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http://dx.doi.org/10.1056/NEJMoa1807120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289290PMC
October 2018

VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.

N Engl J Med 2018 10 18;379(17):1599-1611. Epub 2018 Oct 18.

From Imperial College London and Royal Brompton and Harefield NHS Foundation Trust, London (J.C.D.), and the Manchester Adult Cystic Fibrosis Centre, Manchester (A.H.) - both in the United Kingdom; Vertex Pharmaceuticals (S.M.M., C.M.M., S.R., R.A.S., C.S., F.V.G., D.W., F.X., T.Y.) and Boston Children's Hospital and Brigham and Women's Hospital (A.U.) - all in Boston; Indiana University School of Medicine, Indianapolis (C.B.); Universitätsmedizin Berlin and Berlin Institute of Health, Berlin, and the German Center for Lung Research, Giessen - all in Germany (M.A.M.); St. Vincent's University Hospital and University College Dublin School of Medicine, Dublin (E.F.M.), and Cork University Hospital and University College Cork, Cork (B.J.P.) - all in Ireland; Schneider Children's Medical Center of Israel, Petah Tikva, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv (D.P.) - both in Israel; Seattle Children's Hospital, Seattle (B.W.R.); National Jewish Health, Denver (J.L.T.-C.); St. Michael's Hospital, Toronto (E.T.); and the University of Alabama at Birmingham, Birmingham (S.M.R.).

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http://dx.doi.org/10.1056/NEJMoa1807119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277022PMC
October 2018

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

JCI Insight 2018 08 9;3(15). Epub 2018 Aug 9.

Department of Internal Medicine and Pappajohn Biomedical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.

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http://dx.doi.org/10.1172/jci.insight.121468DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6129116PMC
August 2018

Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.

N Engl J Med 2017 11 3;377(21):2013-2023. Epub 2017 Nov 3.

From National Jewish Health, Denver (J.L.T.-C.); Hôpital Robert Debré, Assistance Publique-Hopitaux de Paris, Paris (A. Munck); University College Dublin School of Medicine, St. Vincent's University Hospital, Dublin (E.F.M.); University Medical Center, Utrecht, the Netherlands (C.K.E.); University Children's Hospital Zurich, Zurich, Switzerland (A. Moeller); Vertex Pharmaceuticals, Boston (C.S., L.T.W., E.P.I., C.M., Y.L., J.L.-H.); and Imperial College and Royal Brompton Hospital and Harefield NHS Foundation Trust, London, and Queens University, Belfast - all in the United Kingdom (J.S.E.).

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http://dx.doi.org/10.1056/NEJMoa1709846DOI Listing
November 2017

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

Respirology 2017 01 11;22(1):141-148. Epub 2016 Sep 11.

Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland.

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http://dx.doi.org/10.1111/resp.12885DOI Listing
January 2017

Personalised medicine in advanced cystic fibrosis.

Authors:
Edward F McKone

Lancet Respir Med 2016 08 10;4(8):594-595. Epub 2016 Jun 10.

St Vincent's University Hospital, Dublin 4, Ireland. Electronic address:

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http://dx.doi.org/10.1016/S2213-2600(16)30156-4DOI Listing
August 2016

Pharmacogenetics of cystic fibrosis treatment.

Pharmacogenomics 2016 08 4;17(13):1453-63. Epub 2016 Aug 4.

National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Elm Park, Dublin 4, Ireland.

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http://dx.doi.org/10.2217/pgs.16.25DOI Listing
August 2016

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

J Allergy Clin Immunol 2016 Feb 14;137(2):390-9. Epub 2015 Jul 14.

Environmental Epidemiology and Risk Program, Harvard T.H. Chan School of Public Health, Boston, Mass; Channing Laboratory, Brigham and Women's Hospital, Department of Medicine, Harvard Medical School, Boston, Mass. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2015.05.028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4742428PMC
February 2016

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

Am J Respir Crit Care Med 2015 Oct;192(7):836-42

6 Case Western Reserve University School of Medicine Rainbow Babies and Children's Hospital, Cleveland, Ohio.

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http://dx.doi.org/10.1164/rccm.201503-0578OCDOI Listing
October 2015

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

J Cyst Fibros 2015 Sep 3;14(5):580-6. Epub 2015 Feb 3.

Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA, USA.

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http://dx.doi.org/10.1016/j.jcf.2015.01.005DOI Listing
September 2015

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

N Engl J Med 2015 07 17;373(3):220-31. Epub 2015 May 17.

From Queensland Children's Medical Research Institute, Royal Children's Hospital, Lady Cilento Children's Hospital, and University of Queensland School of Medicine, Brisbane, Australia (C.E.W.); Queens University of Belfast, Belfast (J.S.E.), and Royal Brompton and Harefield NHS Foundation Trust and Imperial College London, London (J.C.D.) - all in the United Kingdom; Seattle Children's Hospital and University of Washington School of Medicine, Seattle (B.W.R.); Vertex Pharmaceuticals, Boston (G.M., X.H., D.W.); Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata, Verona (M.C.), and Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, University of Milan, Milan (C.C.) - both in Italy; University Hospital Gasthuisberg, Leuven, Belgium (K.D.B.); Medical University of South Carolina, Charleston (P.A.F.); Case Western Reserve University School of Medicine, Rainbow Babies and Children's Hospital, Cleveland (M.W.K.), and the Department of Pediatrics, Pulmonary Division, Nationwide Children's Hospital and Ohio State University, Columbus (K.M.) - both in Ohio; Stanley Manne Children's Research Institute, Northwestern University Feinberg School of Medicine, Chicago (S.A.M.); St. Vincent's University Hospital and University College Dublin School of Medicine, Dublin (E.F.M.); Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Center, Assistance Publique-Hôpitaux de Paris, Université Paris 7, Paris (A.M.); Division of Respiratory Medicine, Department of Pediatrics, Physiology, and Experimental Medicine, Hospital for Sick Children, University of Toronto, Toronto (F.R.); University of Alabama at Birmingham, Birmingham (S.M.R.); and Johns Hopkins Medicine, Baltimore (M.P.B.).

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http://dx.doi.org/10.1056/NEJMoa1409547DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4764353PMC
July 2015

Pulmonary blastoma: a case report and review of the literature.

BMC Res Notes 2014 May 13;7:294. Epub 2014 May 13.

St, Vincent's University Hospital, Elm Park, Dublin 4, Ireland.

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http://dx.doi.org/10.1186/1756-0500-7-294DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4026592PMC
May 2014

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

J Cyst Fibros 2013 Mar 16;12(2):141-6. Epub 2012 Aug 16.

Department of Medical Microbiology, St. Vincent's University Hospital, Elm Park, Dublin 4, Ireland. Electronic address:

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http://dx.doi.org/10.1016/j.jcf.2012.07.007DOI Listing
March 2013

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

J Cyst Fibros 2010 Dec 14;9(6):400-5. Epub 2010 Aug 14.

National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Dublin, Ireland.

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http://dx.doi.org/10.1016/j.jcf.2010.07.001DOI Listing
December 2010

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

Radiology 2009 Aug 9;252(2):577-86. Epub 2009 Jun 9.

Department of Radiology, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland.

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http://dx.doi.org/10.1148/radiol.2522081956DOI Listing
August 2009

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

Chest 2008 Dec 8;134(6):1258-1264. Epub 2008 Aug 8.

National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Dublin, Ireland. Electronic address:

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http://dx.doi.org/10.1378/chest.08-1091DOI Listing
December 2008

CFTR genotype as a predictor of prognosis in cystic fibrosis.

Chest 2006 Nov;130(5):1441-7

Division of Pulmonary and Critical Care Medicine and Adult Cystic Fibrosis Center, University of Washington Medical Center, Seattle, WA 98195-6522, USA.

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http://dx.doi.org/10.1378/chest.130.5.1441DOI Listing
November 2006

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

Am J Respir Crit Care Med 2006 Aug 11;174(4):415-9. Epub 2006 May 11.

Department of Environmental and Occupational Health Sciences, University of Washington Mail Box 354695, Seattle, WA 98195, USA.

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http://dx.doi.org/10.1164/rccm.200508-1281OCDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2648118PMC
August 2006

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

J Cyst Fibros 2006 May;5(2):85-91

Department of Obstetrics and Gynecology, University of Washington, Seattle, WA, USA.

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http://dx.doi.org/10.1016/j.jcf.2006.01.002DOI Listing
May 2006

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

J Appl Physiol (1985) 2005 Sep 28;99(3):1012-8. Epub 2005 Apr 28.

Dept. of Respiratory Medicine, St. Vincent's Univ. Hospital, Elm Park, Dublin 4, Republic of Ireland.

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http://www.physiology.org/doi/10.1152/japplphysiol.00475.200
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http://dx.doi.org/10.1152/japplphysiol.00475.2004DOI Listing
September 2005

Publications and extramural activities of general internal medicine and medicine subspecialty clinician-educators: a multicenter study.

Acad Med 2005 Mar;80(3):238-43

Department of Internal Medicine, University of Minnesota School of Medicine, Minneapolis, MN 55455, USA.

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http://dx.doi.org/10.1097/00001888-200503000-00007DOI Listing
March 2005

Comparison of scholarly productivity of general and subspecialty clinician-educators in internal medicine.

Teach Learn Med 2004 ;16(4):323-8

Department of Internal Medicine, University of Washington School of Medicine, Seattle, Washington, USA.

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http://dx.doi.org/10.1207/s15328015tlm1604_4DOI Listing
January 2005

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

Lancet 2003 May;361(9370):1671-6

Division of Pulmonary and Critical Care Medicine and Adult Cystic Fibrosis Center, University of Washington, Seattle, WA, USA.

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http://dx.doi.org/10.1016/S0140-6736(03)13368-5DOI Listing
May 2003

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

Pediatr Pulmonol 2002 Nov;34(5):381-3

Adult Cystic Fibrosis Center, University of Washington Medical Center, Seattle, Washington, USA.

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http://dx.doi.org/10.1002/ppul.10184DOI Listing
November 2002

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

Chest 2002 Jun;121(6):1761-70

Department of Physiology, Conway Institute of Biomedical and Biomolecular Research, University College, Dublin, Ireland.

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http://dx.doi.org/10.1378/chest.121.6.1761DOI Listing
June 2002