Search Results - "McKone, E. F."

Targeting the PI3K/Akt/mTOR signalling pathway in Cystic Fibrosis by Reilly, R., Mroz, M. S., Dempsey, E., Wynne, K., Keely, S. J., McKone, E. F., Hiebel, C., Behl, C., Coppinger, J. A.

“…Deletion of phenylalanine 508 of the cystic fibrosis transmembrane conductance regulator (ΔF508 CFTR) is a major cause of cystic fibrosis (CF), one of the most…”
Get full text

Changes in fecal microbiota with CFTR modulator therapy: A pilot study by Pope, CE, Vo, AT, Hayden, HS, Weiss, EJ, Durfey, S, McNamara, S, Ratjen, A, Grogan, B, Carter, S, Nay, L, Parsek, MR, Singh, PK, McKone, EF, Aitken, ML, Rosenfeld, MR, Hoffman, LR

“…•People with CF often have exocrine pancreatic insufficiency (PI) and altered fecal microbiomes.•Whether the PI and resulting nutrient malabsorption causes the…”
Get full text

Reproducibility of maximal exercise ergometer testing in patients with cystic fibrosis by McKone, E F, Barry, S C, FitzGerald, M X, Gallagher, C G

“…Exercise testing in patients with cystic fibrosis (CF) has become an important tool in assessing disease severity and predicting overall outcome. The…”
Get more information

The role of supplemental oxygen during submaximal exercise in patients with cystic fibrosis by McKone, E.F, Barry, S.C, FitzGerald, M.X, Gallagher, C.G

“…Repeated bouts of submaximal exercise are an important part of most pulmonary rehabilitation programmes. Patients with moderate-to-severe cystic fibrosis (CF)…”
Get full text

Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T by Carter, S, Kelly, S, Caples, E, Grogan, B, Doyle, J, Gallagher, C.G, McKone, E.F

“…Abstract Ivacaftor is a novel CFTR potentiator that increases CFTR activity and improves clinical outcomes in cystic fibrosis (CF) patients with at least one…”
Get full text

Experience using centralized spirometry in the phase 2 randomized, placebo-controlled, double-blind trial of denufosol in patients with mild to moderate cystic fibrosis by Goss, C.H, McKone, E.F, Mathews, D, Kerr, D, Wanger, J.S, Millard, S.P

“…Abstract Background Centralized spirometry may significantly improve quality of spirometry and reduce variability of this outcome measure in clinical trials in…”
Get full text

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele by Middleton, Peter G, Mall, Marcus A, Dřevínek, Pavel, Lands, Larry C, McKone, Edward F, Polineni, Deepika, Ramsey, Bonnie W, Taylor-Cousar, Jennifer L, Tullis, Elizabeth, Vermeulen, François, Marigowda, Gautham, McKee, Charlotte M, Moskowitz, Samuel M, Nair, Nitin, Savage, Jessica, Simard, Christopher, Tian, Simon, Waltz, David, Xuan, Fengjuan, Rowe, Steven M, Jain, Raksha

“…Triple treatment with elexacaftor, tezacaftor, and ivacaftor in patients with cystic fibrosis who had one Phe508del allele and a minimal-function mutation…”
Get full text

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR by Wainwright, Claire E, Elborn, J. Stuart, Ramsey, Bonnie W, Marigowda, Gautham, Huang, Xiaohong, Cipolli, Marco, Colombo, Carla, Davies, Jane C, De Boeck, Kris, Flume, Patrick A, Konstan, Michael W, McColley, Susanna A, McCoy, Karen, McKone, Edward F, Munck, Anne, Ratjen, Felix, Rowe, Steven M, Waltz, David, Boyle, Michael P

“…This study identified a new combination therapy for patients with cystic fibrosis homozygous for the Phe508del CFTR mutation. Treatment with ivacaftor, a CFTR…”
Get full text

VX-659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles by Davies, Jane C, Moskowitz, Samuel M, Brown, Cynthia, Horsley, Alexander, Mall, Marcus A, McKone, Edward F, Plant, Barry J, Prais, Dario, Ramsey, Bonnie W, Taylor-Cousar, Jennifer L, Tullis, Elizabeth, Uluer, Ahmet, McKee, Charlotte M, Robertson, Sarah, Shilling, Rebecca A, Simard, Christopher, Van Goor, Fredrick, Waltz, David, Xuan, Fengjuan, Young, Tim, Rowe, Steven M

“…This companion article to the VX-445 report shows that VX-659, a new CFTR potentiator, when administered with tezacaftor and ivacaftor improved lung function,…”
Get full text

B16 CYSTIC FIBROSIS (CF) AND NON-CF BRONCHIECTASIS: Short-Term And Long-Term Effects Of Ivacaftor Treatment On Sputum Microbiota In Cf Patients With The G551d Cftr Mutation by Hoffman, L, Pope, C, Jorth, P, Hisert, K B, Welsh, M, Singh, P K, McKone, E F

Get full text

B54 CASE REPORTS IN THE PULMONARY CIRCULATION: Pneumonectomy For The Management Of Unilateral Pulmonary Artery Agenesis by O'Reilly, A, Sharma, K, Franciosi, A, Dodd, J D, Fabre, A, Healy, D, McKone, E F, Gallagher, C G

Get full text

C60 ALL ABOUT CYSTIC FIBROSIS: Acute And Long Term Effects Of Ivacaftor On Air Trapping In Adults With Cystic Fibrosis And The G551d Mutation by Adam, R J, Dodd, J D, Hisert, K B, Grogan, B, Launspach, J L, Gallagher, C G, Sieren, J, Hoffman, E A, Welsh, M, Singh, P K, McKone, E F, Stoltz, D A

Get full text

Corrigendum to “WS04.1 The effect of Orkambi® on exercise capacity and muscle strength” [J Cyst Fibros, volume 16, supplement 1, June 2017, pages S6–S7] by Buckley, R., Reilly, C.M., Kelly, S., Ward, E., O'Connor, C., Carter, S., Gallagher, C.G., McKone, E.F.

Get full text

Validation and use of a parametric model for projecting cystic fibrosis survivorship beyond observed data: a birth cohort analysis by Jackson, Abaigeal D, Daly, Leslie, Jackson, Andrew L, Kelleher, Cecily, Marshall, Bruce C, Quinton, Hebe B, Fletcher, Godfrey, Harrington, Mary, Zhou, Shijun, McKone, Edward F, Gallagher, Charles, Foley, Linda, Fitzpatrick, Patricia

“…The current lifetable approach to survival estimation is favoured by CF registries. Recognising the limitation of this approach, we examined the utility of a…”
Get more information

A13 THE GENOME AND ASTHMA IN 2014: Pathway Analysis Of A Genome Wide Gene By Air Pollution Interaction Study In Asthma by Ierodiakonou, D, Coull, B A, Zanobetti, A, Melly, S, Postma, D, Boezen, H M, Vonk, J M, Williams, P V, Shapiro, G G, McKone, E F, Hallstrand, T S, Koenig, J Q, Jonathan, S S, Koppelman, G H, Lumley, T, Croteau-Chonka, D D C, Fuhlbrigge, A, Koutrakis, P, Schwartz, J, Gold, D R, Weiss, S T

Get full text

Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study by McKone, Edward F, Emerson, Scott S, Edwards, Karen L, Aitken, Moira L

“…Over 1000 mutations of the cystic fibrosis transmembrane conductance regulator gene (CFTR) that cause cystic fibrosis have been identified. We examined the…”
Get full text

Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe by Jung, Andreas, Orenti, Annalisa, Dunlevy, Fiona, Aleksejeva, Elina, Bakkeheim, Egil, Bobrovnichy, Vladimir, Carr, Siobhán B, Colombo, Carla, Corvol, Harriet, Cosgriff, Rebecca, Daneau, Géraldine, Dogru, Deniz, Drevinek, Pavel, Vukic, Andrea Dugac, Fajac, Isabelle, Fox, Alice, Fustik, Stojka, Gulmans, Vincent, Harutyunyan, Satenik, Hatziagorou, Elpis, Kasmi, Irena, Kayserová, Hana, Kondratyeva, Elena, Krivec, Uroš, Makukh, Halyna, Malakauskas, Kestutis, McKone, Edward F, Mei-Zahav, Meir, de Monestrol, Isabelle, Olesen, Hanne Vebert, Padoan, Rita, Parulava, Tsitsino, Pastor-Vivero, Maria Dolores, Pereira, Luísa, Petrova, Guergana, Pfleger, Andreas, Pop, Liviu, van Rens, Jacqui G, Rodic, Milan, Schlesser, Marc, Storms, Valérie, Turcu, Oxana, Woz Niacki, Lukasz, Yiallouros, Panayiotis, Zolin, Anna, Downey, Damian G, Naehrlich, Lutz

“…Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis (pwCF) can lead to severe outcomes. In this observational…”
Get full text

Role of arterial hypoxemia and pulmonary mechanics in exercise limitation in adults with cystic fibrosis by McKone, Edward F, Barry, Sinead C, FitzGerald, Muiris X, Gallagher, Charles G

“…Department of Respiratory Medicine and National Referral Center for Adult Cystic Fibrosis, St. Vincent's University Hospital, Dublin, Ireland Submitted 4 May…”
Get full text

Publications and extramural activities of general internal medicine and medicine subspecialty clinician-educators: a multicenter study by Kempainen, Robert R, McKone, Edward F, Rubenfeld, Gordon D, Scott, Craig S, Tonelli, Mark R

“…Generalist clinician-educators may have more difficulty than specialists satisfying common promotion criteria (peer-reviewed publication and extramural…”
Get full text

Use of the gas exchange threshold to noninvasively determine the lactate threshold in patients with cystic fibrosis by Thin, Alasdair G, Linnane, Seamus J, McKone, Edward F, Freaney, Rosemarie, FitzGerald, Muiris X, Gallagher, Charles G, McLoughlin, Paul

“…The anaerobic threshold (AT) is a submaximal index related to endurance exercise performance, which is usually determined by the measurement of blood lactate…”
Get more information