Elexacaftor–tezacaftor–ivacaftor for cystic fibrosis with a single Phe508del allele PG Middleton, MA Mall, P Dřevínek, LC Lands, EF McKone, D Polineni, ... New England Journal of Medicine 381 (19), 1809-1819, 2019 | 1725 | 2019 |
Lumacaftor–Ivacaftor in patients with Cystic Fibrosis homozygous for Phe508del CFTR. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M,Colombo ... N Engl J Med 373, 220-231, 2015 | 1581* | 2015 |
Liposome-mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis NJ Caplen, EWFW Alton, PG Mddleton, JR Dorin, BJ Stevenson, X Gao, ... Nature medicine 1 (1), 39-46, 1995 | 979 | 1995 |
VX-445-Tezacaftor-Ivacaftor in patients with Cystic Fibrosis and one or two Phe508del alleles Keating D, Marigowda G, Burr L, Daines C, Mall MA, McKone EF, Ramsey BW ... New Engl J Med 379, 1612-20, 2018 | 673 | 2018 |
Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice E Alton, PG Middleton, NJ Caplen, SN Smith, DM Steel, FM Munkonge, ... Nature genetics 5 (2), 135-142, 1993 | 537 | 1993 |
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised … MP Boyle, SC Bell, MW Konstan, SA McColley, SM Rowe, E Rietschel, ... Lancet Respiratory Medicine 11, 539-549, 2014 | 531 | 2014 |
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis DR Gill, KW Southern, KA Mofford, T Seddon, L Huang, F Sorgi, ... Gene therapy 4 (3), 199-209, 1997 | 300 | 1997 |
The safety and efficacy of inhaled dry powder mannitol as a bronchial provocation test for airway hyperresponsiveness: a phase 3 comparison study with hypertonic (4.5%) saline JD Brannan, SD Anderson, CP Perry, R Freed-Martens, AR Lassig, ... Respiratory research 6, 1-12, 2005 | 261 | 2005 |
Triple therapy for Cystic Fibrosis Phe508del –gating and -residual function genotypes. PD Barry PJ, Mall MA, Alvarez A, Colombo C, de Winter-Groot KM, Fajac I ... New England Journal of Medicine 385, 815, 2021 | 208 | 2021 |
Sleep disordered breathing and pregnancy N Edwards, PG Middleton, DM Blyton, CE Sullivan Thorax 57 (6), 555-558, 2002 | 194 | 2002 |
Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium PG Middleton, DM Geddes, EW Alton European Respiratory Journal 7 (11), 2050-2056, 1994 | 189 | 1994 |
Treatable traits can be identified in a severe asthma registry and predict future exacerbations VM McDonald, SA Hiles, K Godbout, ES Harvey, GB Marks, M Hew, ... Respirology 24 (1), 37-47, 2019 | 183 | 2019 |
Efficacy and safety of lumacaftor / ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis VXTT Elborn JS, Ramsey BW, Boyle MP, Konstan MW, Huang X, Marigowda G, Waltz ... Lancet 4, 617-26, 2016 | 165* | 2016 |
A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis R Cosgriff, S Ahern, SC Bell, K Brownlee, PR Burgel, C Byrnes, H Corvol, ... Journal of cystic fibrosis 19 (3), 355-358, 2020 | 152 | 2020 |
New clinical diagnostic procedures for cystic fibrosis in Europe K De Boeck, N Derichs, I Fajac, HR De Jonge, I Bronsveld, I Sermet, ... Journal of Cystic Fibrosis 10, S53-S66, 2011 | 142 | 2011 |
Long term effects of denufosol tetrasodium in patients with cystic fibrosis Ratjen F, Durham T, Navratil T, Schaberg A, Accurso FJ, Wainwright C, Barnes ... Journal of Cystic Fibrosis 11, 539-49, 2012 | 124 | 2012 |
CFTR biomarkers: time for promotion to surrogate end-point K De Boeck, L Kent, J Davies, N Derichs, M Amaral, SM Rowe, ... European Respiratory Journal 41 (1), 203-216, 2013 | 123 | 2013 |
ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases PG Middleton, EJ Gade, C Aguilera, L MacKillop, BM Button, C Coleman, ... European Respiratory Journal 55 (2), 2020 | 119 | 2020 |
Clinical associations and prevalence of Scedosporium spp. in Australian cystic fibrosis patients: identification of novel risk factors CC Blyth, PG Middleton, A Harun, TC Sorrell, W Meyer, SC Chen Medical Mycology 48 (Suppl 1), S37-44, 2010 | 119 | 2010 |
Cystic fibrosis infections: treatment strategies and prospects AM George, PM Jones, PG Middleton FEMS microbiology letters 300 (2), 153-164, 2009 | 113 | 2009 |