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Simultaneous sulfur hexafluoride and nitrogen multiple-breath washout (MBW) to examine inherent differences in MBW outcomes.

Bayfield KJ, Horsley A, Alton E, Irving S, Bush A, Davies JC.

ERJ Open Res. 2019 Nov 4;5(4). pii: 00234-2018. doi: 10.1183/23120541.00234-2018. eCollection 2019 Oct.


Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

Kyrilli S, Henry T, Wilschanski M, Fajac I, Davies JC, Jais JP, Sermet-Gaudelus I.

J Cyst Fibros. 2019 Nov 4. pii: S1569-1993(19)30899-9. doi: 10.1016/j.jcf.2019.09.015. [Epub ahead of print]


Cephalosporin nitric oxide-donor prodrug DEA-C3D disperses biofilms formed by clinical cystic fibrosis isolates of Pseudomonas aeruginosa.

Soren O, Rineh A, Silva DG, Cai Y, Howlin RP, Allan RN, Feelisch M, Davies JC, Connett GJ, Faust SN, Kelso MJ, Webb JS.

J Antimicrob Chemother. 2019 Sep 17. pii: dkz378. doi: 10.1093/jac/dkz378. [Epub ahead of print]


The future of cystic fibrosis care: a global perspective.

Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel PR, Tullis E, Castaños C, Castellani C, Byrnes CA, Cathcart F, Chotirmall SH, Cosgriff R, Eichler I, Fajac I, Goss CH, Drevinek P, Farrell PM, Gravelle AM, Havermans T, Mayer-Hamblett N, Kashirskaya N, Kerem E, Mathew JL, McKone EF, Naehrlich L, Nasr SZ, Oates GR, O'Neill C, Pypops U, Raraigh KS, Rowe SM, Southern KW, Sivam S, Stephenson AL, Zampoli M, Ratjen F.

Lancet Respir Med. 2019 Sep 27. pii: S2213-2600(19)30337-6. doi: 10.1016/S2213-2600(19)30337-6. [Epub ahead of print] Review. Erratum in: Lancet Respir Med. 2019 Oct 25;:.


Quantitative Analysis of Surgical Working Space During Endoscopic Skull Base Surgery.

Davies JC, Chan HHL, Yao CMKL, Cusimano MD, Irish JC, Lee JM.

J Neurol Surg B Skull Base. 2019 Oct;80(5):469-473. doi: 10.1055/s-0038-1675591. Epub 2018 Nov 26.


Training dogs to differentiate Pseudomonas aeruginosa from other cystic fibrosis bacterial pathogens: not to be sniffed at?

Davies JC, Alton E, Simbo A, Murphy R, Seth I, Williams K, Somerville M, Jolly L, Morant S, Guest C.

Eur Respir J. 2019 Nov 7;54(5). pii: 1900970. doi: 10.1183/13993003.00970-2019. Print 2019 Nov. No abstract available.


Fair selection of participants in clinical trials: The challenge to push the envelope further.

Davies JC, Scott S, Dobra R, Brendell R, Brownlee K, Carr SB, Cosgriff R, Simmonds NJ; London Network of Clinical Trials Accelerator Platform sites, Jahan R, Jones A, Matthews J, Brown S, Galono K, Miles K, Pao C, Shafi N, Watson D, Orchard C, Davies G, Pike K, Shah S, Bossley CJ, Fong T, Macedo P, Ruiz G, Waller M, Baker L.

J Cyst Fibros. 2019 Sep;18(5):e48-e50. doi: 10.1016/j.jcf.2019.07.004. Epub 2019 Aug 9. No abstract available.


Speeding up access to new drugs for CF: Considerations for clinical trial design and delivery.

Davies JC, Drevinek P, Elborn JS, Kerem E, Lee T; European CF Society (ECFS) Strategic Planning Task Force on ‘Speeding up access to new 4 drugs for CF’, Amaral MD, de Boeck K, Davies JC, Drevinek P, Elborn JS, Kerem E, Lee T.

J Cyst Fibros. 2019 Sep;18(5):677-684. doi: 10.1016/j.jcf.2019.06.011. Epub 2019 Jul 11.


ONRAB® oral rabies vaccine is shed from, but does not persist in, captive mammals.

Sobey KG, Jamieson SE, Walpole AA, Rosatte RC, Donovan D, Fehlner-Gardiner C, Nadin-Davis SA, Davies JC, Kyle CJ.

Vaccine. 2019 Jul 18;37(31):4310-4317. doi: 10.1016/j.vaccine.2019.06.046. Epub 2019 Jun 24.


Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis.

Turnbull AR, Pyle CJ, Patel DF, Jackson PL, Hilliard TN, Regamey N, Tan HL, Brown S, Thursfield R, Short C, Mc Fie M, Alton EWFW, Gaggar A, Blalock JE, Lloyd CM, Bush A, Davies JC, Snelgrove RJ.

J Cyst Fibros. 2019 Jun 5. pii: S1569-1993(19)30769-6. doi: 10.1016/j.jcf.2019.05.017. [Epub ahead of print]


Trials and tribulations: The highs and lows of running cystic fibrosis drug studies.

Davies JC.

Paediatr Respir Rev. 2019 Aug;31:25-27. doi: 10.1016/j.prrv.2019.03.002. Epub 2019 Mar 14. Review.


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

Davies JC, Van de Steen O, van Koningsbruggen-Rietschel S, Drevinek P, Derichs N, McKone EF, Kanters D, Allamassey L, Namour F, de Kock H, Conrath K.

J Cyst Fibros. 2019 Sep;18(5):693-699. doi: 10.1016/j.jcf.2019.05.006. Epub 2019 May 27.


Who and why; sharing our experiences of developing a standard operating procedure (SOP) to allocate screening slots for highly competitive cystic fibrosis trials.

Dobra R, Scott S, Davies JC, Simmonds NJ.

J Cyst Fibros. 2019 Sep;18(5):e45-e46. doi: 10.1016/j.jcf.2019.04.008. Epub 2019 May 3. No abstract available.


An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB).

Rosenfeld M, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Chilvers M, Higgins M, Tian S, Cooke J, Davies JC; KLIMB study group.

J Cyst Fibros. 2019 Apr 30. pii: S1569-1993(19)30061-X. doi: 10.1016/j.jcf.2019.03.009. [Epub ahead of print]


Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition.

Southern KW, Barben J, Gartner S, Munck A, Castellani C, Mayell SJ, Davies JC, Winters V, Murphy J, Salinas D, McColley SA, Ren CL, Farrell PM.

J Cyst Fibros. 2019 Apr 24. pii: S1569-1993(19)30070-0. doi: 10.1016/j.jcf.2019.04.010. [Epub ahead of print] No abstract available.


"Fortunate are those who take the first steps"? The psychosocial impact of novel drug development.

Dobra R, Madge S, Martin I, Weldon P, Simmonds N, Davies JC.

Paediatr Respir Rev. 2019 Aug;31:9-11. doi: 10.1016/j.prrv.2019.02.005. Epub 2019 Feb 27. Review.


Longitudinal development of the airway microbiota in infants with cystic fibrosis.

Ahmed B, Cox MJ, Cuthbertson L, James P, Cookson WOC, Davies JC, Moffatt MF, Bush A.

Sci Rep. 2019 Mar 26;9(1):5143. doi: 10.1038/s41598-019-41597-0.


Chronic infection by controlling inflammation.

Filloux A, Davies JC.

Nat Microbiol. 2019 Mar;4(3):378-379. doi: 10.1038/s41564-019-0397-6. No abstract available.


Analysis of simulated mandibular reconstruction using a segmental mirroring technique.

Davies JC, Chan HHL, Jozaghi Y, Goldstein DP, Irish JC.

J Craniomaxillofac Surg. 2019 Mar;47(3):468-472. doi: 10.1016/j.jcms.2018.12.016. Epub 2018 Dec 30.


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

Davies JC, Moskowitz SM, Brown C, Horsley A, Mall MA, McKone EF, Plant BJ, Prais D, Ramsey BW, Taylor-Cousar JL, Tullis E, Uluer A, McKee CM, Robertson S, Shilling RA, Simard C, Van Goor F, Waltz D, Xuan F, Young T, Rowe SM; VX16-659-101 Study Group.

N Engl J Med. 2018 Oct 25;379(17):1599-1611. doi: 10.1056/NEJMoa1807119. Epub 2018 Oct 18.


Potentiators and Correctors in Paediatric Cystic Fibrosis Patients: A Narrative Review.

Dobra R, Edmondson C, Hughes D, Martin I, Davies JC.

Paediatr Drugs. 2018 Dec;20(6):555-566. doi: 10.1007/s40272-018-0315-z. Review.


New anti-pseudomonal agents for cystic fibrosis- still needed in the era of small molecule CFTR modulators?

Davies JC, Martin I.

Expert Opin Pharmacother. 2018 Aug;19(12):1327-1336. doi: 10.1080/14656566.2018.1505864. Epub 2018 Aug 12. Review.


Comparison of the upper and lower airway microbiota in children with chronic lung diseases.

Ahmed B, Cox MJ, Cuthbertson L, James PL, Cookson WOC, Davies JC, Moffatt MF, Bush A.

PLoS One. 2018 Aug 2;13(8):e0201156. doi: 10.1371/journal.pone.0201156. eCollection 2018.


Metabolic Phenotyping and Strain Characterisation of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients Using Rapid Evaporative Ionisation Mass Spectrometry.

Bardin EE, Cameron SJS, Perdones-Montero A, Hardiman K, Bolt F, Alton EWFW, Bush A, Davies JC, Takáts Z.

Sci Rep. 2018 Jul 19;8(1):10952. doi: 10.1038/s41598-018-28665-7.


Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study.

Rosenfeld M, Wainwright CE, Higgins M, Wang LT, McKee C, Campbell D, Tian S, Schneider J, Cunningham S, Davies JC; ARRIVAL study group.

Lancet Respir Med. 2018 Jul;6(7):545-553. doi: 10.1016/S2213-2600(18)30202-9. Epub 2018 Jun 7. Erratum in: Lancet Respir Med. 2018 Jul;6(7):e35. Lancet Respir Med. 2019 Apr;7(4):e15.


Outdoor air pollution and cystic fibrosis.

Brugha R, Edmondson C, Davies JC.

Paediatr Respir Rev. 2018 Sep;28:80-86. doi: 10.1016/j.prrv.2018.03.005. Epub 2018 Apr 11.


Predicting the Future of Cystic Fibrosis Lung Disease: Gene Expression Holds Some of the Answers.

Edmondson C, Davies JC.

Ann Am Thorac Soc. 2018 May;15(5):556-557. doi: 10.1513/AnnalsATS.201802-098ED. No abstract available.


Orbital Floor Reconstruction: 3-Dimensional Analysis Shows Comparable Morphology of Scapular and Iliac Crest Bone Grafts.

Davies JC, Chan HHL, Bernstein JM, Goldstein DP, Irish JC, Gilbert RW.

J Oral Maxillofac Surg. 2018 Sep;76(9):2011-2018. doi: 10.1016/j.joms.2018.03.034. Epub 2018 Mar 28.


Children with cystic fibrosis demonstrate no respiratory immunological, infective or physiological, consequences of vitamin D deficiency.

Thursfield RM, Naderi K, Leaver N, Rosenthal M, Alton EWFW, Bush A, Davies JC.

J Cyst Fibros. 2018 Sep;17(5):657-665. doi: 10.1016/j.jcf.2018.02.011. Epub 2018 Apr 7.


Impact of T2R38 Receptor Polymorphisms on Pseudomonas aeruginosa Infection in Cystic Fibrosis.

Turnbull AR, Murphy R, Behrends V, Lund-Palau H, Simbo A, Mariveles M, Alton EWFW, Bush A, Shoemark A, Davies JC.

Am J Respir Crit Care Med. 2018 Jun 15;197(12):1635-1638. doi: 10.1164/rccm.201711-2365LE. No abstract available.


Pooling of bronchoalveolar lavage in children with cystic fibrosis does not adversely affect the microbiological yield or sensitivity in detecting pulmonary inflammation.

McNally P, O'Rourke J, Fantino E, Chacko A, Pabary R, Turnbull A, Grant T, O'Sullivan N, Wainwright C, Linnane B, Davies JC, Sly PD.

J Cyst Fibros. 2018 May;17(3):391-399. doi: 10.1016/j.jcf.2017.10.016. Epub 2017 Nov 20.


The Immunomodulatory Drug Glatiramer Acetate is Also an Effective Antimicrobial Agent that Kills Gram-negative Bacteria.

Christiansen SH, Murphy RA, Juul-Madsen K, Fredborg M, Hvam ML, Axelgaard E, Skovdal SM, Meyer RL, Sørensen UBS, Möller A, Nyengaard JR, Nørskov-Lauritsen N, Wang M, Gadjeva M, Howard KA, Davies JC, Petersen E, Vorup-Jensen T.

Sci Rep. 2017 Nov 15;7(1):15653. doi: 10.1038/s41598-017-15969-3.


Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis.

Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, Nair N, Simard C, Han L, Ingenito EP, McKee C, Lekstrom-Himes J, Davies JC.

N Engl J Med. 2017 Nov 23;377(21):2024-2035. doi: 10.1056/NEJMoa1709847. Epub 2017 Nov 3.


Where are we with transformational therapies for patients with cystic fibrosis?

De Boeck K, Davies JC.

Curr Opin Pharmacol. 2017 Jun;34:70-75. doi: 10.1016/j.coph.2017.09.005. Epub 2017 Oct 6. Review.


A Cell-Free Biosensor for Detecting Quorum Sensing Molecules in P. aeruginosa-Infected Respiratory Samples.

Wen KY, Cameron L, Chappell J, Jensen K, Bell DJ, Kelwick R, Kopniczky M, Davies JC, Filloux A, Freemont PS.

ACS Synth Biol. 2017 Dec 15;6(12):2293-2301. doi: 10.1021/acssynbio.7b00219. Epub 2017 Oct 5.


Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.

Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, Davies JC, Lekstrom-Himes JA, Wang LT; VX11-661-101 Study Group.

Am J Respir Crit Care Med. 2018 Jan 15;197(2):214-224. doi: 10.1164/rccm.201704-0717OC.


Current and future therapies for Pseudomonas aeruginosa infection in patients with cystic fibrosis.

Smith WD, Bardin E, Cameron L, Edmondson CL, Farrant KV, Martin I, Murphy RA, Soren O, Turnbull AR, Wierre-Gore N, Alton EW, Bundy JG, Bush A, Connett GJ, Faust SN, Filloux A, Freemont PS, Jones AL, Takats Z, Webb JS, Williams HD, Davies JC.

FEMS Microbiol Lett. 2017 Aug 1;364(14). doi: 10.1093/femsle/fnx121. Review.


Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.

Flume PA, Wainwright CE, Elizabeth Tullis D, Rodriguez S, Niknian M, Higgins M, Davies JC, Wagener JS.

J Cyst Fibros. 2018 Jan;17(1):83-88. doi: 10.1016/j.jcf.2017.06.002. Epub 2017 Jun 24.


Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial.

Ratjen F, Hug C, Marigowda G, Tian S, Huang X, Stanojevic S, Milla CE, Robinson PD, Waltz D, Davies JC; VX14-809-109 investigator group.

Lancet Respir Med. 2017 Jul;5(7):557-567. doi: 10.1016/S2213-2600(17)30215-1. Epub 2017 Jun 9. Erratum in: Lancet Respir Med. 2017 Aug;5(8):e28.


Disease-modifying drug therapy in cystic fibrosis.

Harman K, Dobra R, Davies JC.

Paediatr Respir Rev. 2018 Mar;26:7-9. doi: 10.1016/j.prrv.2017.03.008. Epub 2017 Mar 14. Review.


Visualising early lung disease in CF: the emergence of MRI.

Davies JC.

Thorax. 2017 Aug;72(8):682. doi: 10.1136/thoraxjnl-2017-210297. Epub 2017 Apr 14. No abstract available.


Diagnosis of Cystic Fibrosis in Screened Populations.

Farrell PM, White TB, Howenstine MS, Munck A, Parad RB, Rosenfeld M, Sommerburg O, Accurso FJ, Davies JC, Rock MJ, Sanders DB, Wilschanski M, Sermet-Gaudelus I, Blau H, Gartner S, McColley SA.

J Pediatr. 2017 Feb;181S:S33-S44.e2. doi: 10.1016/j.jpeds.2016.09.065.


Developments in multiple breath washout testing in children with cystic fibrosis.

Saunders C, Bayfield K, Irving S, Short C, Bush A, Davies JC.

Curr Med Res Opin. 2017 Apr;33(4):613-620. doi: 10.1080/03007995.2016.1268999. Epub 2017 Feb 2. Review.


Cystic fibrosis in 2016: considerable progress, but much more to do.

Davies JC.

Lancet Respir Med. 2016 Dec;4(12):943-945. doi: 10.1016/S2213-2600(16)30378-2. Epub 2016 Nov 22. No abstract available.


Preparation for a first-in-man lentivirus trial in patients with cystic fibrosis.

Alton EW, Beekman JM, Boyd AC, Brand J, Carlon MS, Connolly MM, Chan M, Conlon S, Davidson HE, Davies JC, Davies LA, Dekkers JF, Doherty A, Gea-Sorli S, Gill DR, Griesenbach U, Hasegawa M, Higgins TE, Hironaka T, Hyndman L, McLachlan G, Inoue M, Hyde SC, Innes JA, Maher TM, Moran C, Meng C, Paul-Smith MC, Pringle IA, Pytel KM, Rodriguez-Martinez A, Schmidt AC, Stevenson BJ, Sumner-Jones SG, Toshner R, Tsugumine S, Wasowicz MW, Zhu J.

Thorax. 2017 Feb;72(2):137-147. doi: 10.1136/thoraxjnl-2016-208406. Epub 2016 Nov 16.


Genetic medicines for CF: Hype versus reality.

Alton EW, Boyd AC, Davies JC, Gill DR, Griesenbach U, Harrison PT, Henig N, Higgins T, Hyde SC, Innes JA, Korman MS.

Pediatr Pulmonol. 2016 Oct;51(S44):S5-S17. doi: 10.1002/ppul.23543. Review.


Cystic fibrosis gene therapy: a mutation-independent treatment.

Griesenbach U, Davies JC, Alton E.

Curr Opin Pulm Med. 2016 Nov;22(6):602-9. doi: 10.1097/MCP.0000000000000327. Review.


A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral cystic fibrosis transmembrane conductance regulator (CFTR) gene therapy in patients with cystic fibrosis.

Alton EWFW, Armstrong DK, Ashby D, Bayfield KJ, Bilton D, Bloomfield EV, Boyd AC, Brand J, Buchan R, Calcedo R, Carvelli P, Chan M, Cheng SH, Collie DS, Cunningham S, Davidson HE, Davies G, Davies JC, Davies LA, Dewar MH, Doherty A, Donovan J, Dwyer NS, Elgmati HI, Featherstone RF, Gavino J, Gea-Sorli S, Geddes DM, Gibson JSR, Gill DR, Greening AP, Griesenbach U, Hansell DM, Harman K, Higgins TE, Hodges SL, Hyde SC, Hyndman L, Innes JA, Jacob J, Jones N, Keogh BF, Limberis MP, Lloyd-Evans P, Maclean AW, Manvell MC, McCormick D, McGovern M, McLachlan G, Meng C, Montero MA, Milligan H, Moyce LJ, Murray GD, Nicholson AG, Osadolor T, Parra-Leiton J, Porteous DJ, Pringle IA, Punch EK, Pytel KM, Quittner AL, Rivellini G, Saunders CJ, Scheule RK, Sheard S, Simmonds NJ, Smith K, Smith SN, Soussi N, Soussi S, Spearing EJ, Stevenson BJ, Sumner-Jones SG, Turkkila M, Ureta RP, Waller MD, Wasowicz MY, Wilson JM, Wolstenholme-Hogg P; on behalf of the UK Cystic Fibrosis Gene Therapy Consortium.

Southampton (UK): NIHR Journals Library; 2016 Jul.


Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications.

Edmondson C, Davies JC.

Ther Adv Chronic Dis. 2016 May;7(3):170-83. doi: 10.1177/2040622316641352. Epub 2016 May 1. Review.


Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches.

Lund-Palau H, Turnbull AR, Bush A, Bardin E, Cameron L, Soren O, Wierre-Gore N, Alton EW, Bundy JG, Connett G, Faust SN, Filloux A, Freemont P, Jones A, Khoo V, Morales S, Murphy R, Pabary R, Simbo A, Schelenz S, Takats Z, Webb J, Williams HD, Davies JC.

Expert Rev Respir Med. 2016 Jun;10(6):685-97. doi: 10.1080/17476348.2016.1177460. Epub 2016 May 13. Review.

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