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Assessment of stability and fluctuations of cultured lower airway bacterial communities in people with cystic fibrosis.

Sherrard LJ, Einarsson GG, Johnston E, O'Neill K, McIlreavey L, McGrath SJ, Gilpin DF, Downey DG, Reid A, McElvaney NG, Boucher RC, Muhlebach MS, Elborn JS, Tunney MM.

J Cyst Fibros. 2019 Mar 21. pii: S1569-1993(19)30052-9. doi: 10.1016/j.jcf.2019.02.012. [Epub ahead of print]


Diagnosis and management of α1-antitrypsin deficiency in Europe: an expert survey.

Horváth I, Canotilho M, Chlumský J, Chorostowska-Wynimko J, Corda L, Derom E, Ficker JH, Kneussl M, Miravitlles M, Sucena M, Thabut G, Turner AM, van 't Wout E, McElvaney NG.

ERJ Open Res. 2019 Mar 11;5(1). pii: 00171-2018. doi: 10.1183/23120541.00171-2018. eCollection 2019 Feb.


Circulating Truncated Alpha-1 Antitrypsin Glycoprotein in Patient Plasma Retains Anti-Inflammatory Capacity.

Reeves EP, Dunlea DM, McQuillan K, O'Dwyer CA, Carroll TP, Saldova R, Akepati PR, Wormald MR, McElvaney OJ, Shutchaidat V, Henry M, Meleady P, Keenan J, Liberti DC, Kotton DN, Rudd PM, Wilson AA, McElvaney NG.

J Immunol. 2019 Apr 15;202(8):2240-2253. doi: 10.4049/jimmunol.1801045. Epub 2019 Feb 22.


The European Alpha-1 Research Collaboration (EARCO): a new ERS Clinical Research Collaboration to promote research in alpha-1 antitrypsin deficiency.

Miravitlles M, Chorostowska-Wynimko J, Ferrarotti I, McElvaney NG, O'Hara K, Stolk J, Stockley RA, Turner A, Wilkens M, Greulich T; EARCO Clinical Research Collaboration; Members of the EARCO Clinical Research Collaboration.

Eur Respir J. 2019 Feb 14;53(2). pii: 1900138. doi: 10.1183/13993003.00138-2019. Print 2019 Feb. No abstract available.


GDPR: an impediment to research?

Clarke N, Vale G, Reeves EP, Kirwan M, Smith D, Farrell M, Hurl G, McElvaney NG.

Ir J Med Sci. 2019 Feb 8. doi: 10.1007/s11845-019-01980-2. [Epub ahead of print]


Prevalence of Smoking and Provision of Smoking Cessation Advice during Hospitalization

Najeeb N, Halawani M, McElvaney NG, Doyle F.

Ir Med J. 2018 Oct 11;111(9):821.


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

Kirwan L, Fletcher G, Harrington M, Jeleniewska P, Zhou S, Casserly B, Gallagher CG, Greally P, Gunaratnam C, Herzig M, Linnane B, McElvaney NG, McKone EF, McNally P, Mullane D, Ní Chróinín M, O'Mahony M, Plant BJ, Jackson AD.

Ann Am Thorac Soc. 2019 Feb;16(2):209-216. doi: 10.1513/AnnalsATS.201802-149OC.


Author Correction: α1-Antitrypsin deficiency.

Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG.

Nat Rev Dis Primers. 2018 Oct 29;4(1):40. doi: 10.1038/s41572-018-0043-2.


Safety of biweekly α1-antitrypsin treatment in the RAPID programme.

Greulich T, Chlumsky J, Wencker M, Vit O, Fries M, Chung T, Shebl A, Vogelmeier C, Chapman KR, McElvaney NG; RAPID Trial Group.

Eur Respir J. 2018 Nov 29;52(5). pii: 1800897. doi: 10.1183/13993003.00897-2018. Print 2018 Nov. No abstract available.


Emerging pharmacotherapies in cystic fibrosis.

McElvaney OJ, Gunaratnam C, McElvaney OF, Bagwe I, Reeves EP, McElvaney NG.

Expert Rev Respir Med. 2018 Oct;12(10):843-855. doi: 10.1080/17476348.2018.1512409. Epub 2018 Sep 19. Review.


Ataluren, a New Therapeutic for Alpha-1 Antitrypsin-Deficient Individuals with Nonsense Mutations.

Reeves EP, O'Dwyer CA, Dunlea DM, Wormald MR, Hawkins P, Alfares M, Kotton DN, Rowe SM, Wilson AA, McElvaney NG.

Am J Respir Crit Care Med. 2018 Oct 15;198(8):1099-1102. doi: 10.1164/rccm.201802-0338LE. No abstract available.


A specialized method of sputum collection and processing for therapeutic interventions in cystic fibrosis.

McElvaney OJ, Gunaratnam C, Reeves EP, McElvaney NG.

J Cyst Fibros. 2019 Mar;18(2):203-211. doi: 10.1016/j.jcf.2018.06.001. Epub 2018 Jun 28.


Anaerobic bacteria cultured from cystic fibrosis airways correlate to milder disease: a multisite study.

Muhlebach MS, Hatch JE, Einarsson GG, McGrath SJ, Gilipin DF, Lavelle G, Mirkovic B, Murray MA, McNally P, Gotman N, Davis Thomas S, Wolfgang MC, Gilligan PH, McElvaney NG, Elborn JS, Boucher RC, Tunney MM.

Eur Respir J. 2018 Jul 11;52(1). pii: 1800242. doi: 10.1183/13993003.00242-2018. Print 2018 Jul.


Hermansky-Pudlak syndrome with a novel genetic variant in HPS1 and subsequent accelerated pulmonary fibrosis: significance for phenocopy diseases.

McElvaney OJ, Huizing M, Gahl WA, O'Donovan P, Horan D, Logan PM, Reeves EP, McElvaney NG.

Thorax. 2018 Nov;73(11):1085-1088. doi: 10.1136/thoraxjnl-2018-211920. Epub 2018 Jun 25.


Targeting IL-8 in Cystic Fibrosis: Enough but Not Too Much.

McElvaney OJ, McElvaney NG.

Am J Respir Cell Mol Biol. 2018 Oct;59(4):401-402. doi: 10.1165/rcmb.2018-0145ED. No abstract available.


The Human Cathelicidin Antimicrobial Peptide LL-37 Promotes the Growth of the Pulmonary Pathogen Aspergillus fumigatus.

Sheehan G, Bergsson G, McElvaney NG, Reeves EP, Kavanagh K.

Infect Immun. 2018 Jun 21;86(7). pii: e00097-18. doi: 10.1128/IAI.00097-18. Print 2018 Jul.


The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven respiratory disease in deficient individuals.

Dunlea DM, Fee LT, McEnery T, McElvaney NG, Reeves EP.

J Inflamm Res. 2018 Mar 26;11:123-134. doi: 10.2147/JIR.S156405. eCollection 2018. Review.


Extracellular Neutrophil Proteases Are Efficient Regulators of IL-1, IL-33, and IL-36 Cytokine Activity but Poor Effectors of Microbial Killing.

Clancy DM, Sullivan GP, Moran HBT, Henry CM, Reeves EP, McElvaney NG, Lavelle EC, Martin SJ.

Cell Rep. 2018 Mar 13;22(11):2937-2950. doi: 10.1016/j.celrep.2018.02.062.


Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95).

Chen Q, Pandi SPS, Kerrigan L, McElvaney NG, Greene CM, Elborn JS, Taggart CC, Weldon S.

J Cyst Fibros. 2018 Sep;17(5):616-623. doi: 10.1016/j.jcf.2018.01.010. Epub 2018 Mar 2.


Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications.

Chapman KR, Chorostowska-Wynimko J, Koczulla AR, Ferrarotti I, McElvaney NG.

Int J Chron Obstruct Pulmon Dis. 2018 Jan 31;13:419-432. doi: 10.2147/COPD.S149429. eCollection 2018. Review.


Pulmonary alveolar proteinosis.

McElvaney OJ, Horan D, Franciosi AN, Gunaratnam C, McElvaney NG.

QJM. 2018 Mar 1;111(3):185-186. doi: 10.1093/qjmed/hcx235. No abstract available.


European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency.

Miravitlles M, Dirksen A, Ferrarotti I, Koblizek V, Lange P, Mahadeva R, McElvaney NG, Parr D, Piitulainen E, Roche N, Stolk J, Thabut G, Turner A, Vogelmeier C, Stockley RA.

Eur Respir J. 2017 Nov 30;50(5). pii: 1700610. doi: 10.1183/13993003.00610-2017. Print 2017 Nov. Review.


Glycosylation Repurposes Alpha-1 Antitrypsin for Resolution of Community-acquired Pneumonia.

McCarthy C, Dunlea DM, Saldova R, Henry M, Meleady P, McElvaney OJ, Marsh B, Rudd PM, Reeves EP, McElvaney NG.

Am J Respir Crit Care Med. 2018 May 15;197(10):1346-1349. doi: 10.1164/rccm.201709-1954LE. No abstract available.


Alpha-1 antitrypsin augmentation therapy decreases miR-199a-5p, miR-598 and miR-320a expression in monocytes via inhibition of NFκB.

Hassan T, de Santi C, Mooney C, McElvaney NG, Greene CM.

Sci Rep. 2017 Oct 23;7(1):13803. doi: 10.1038/s41598-017-14310-2.



McCarthy C, Reeves EP, Orr C, Fearon U, Veale DJ, McElvaney NG.

Arthritis Rheumatol. 2017 Dec;69(12):2404-2406. doi: 10.1002/art.40309. Epub 2017 Nov 8. No abstract available.


Neutrophil Membrane Cholesterol Content is a Key Factor in Cystic Fibrosis Lung Disease.

White MM, Geraghty P, Hayes E, Cox S, Leitch W, Alfawaz B, Lavelle GM, McElvaney OJ, Flannery R, Keenan J, Meleady P, Henry M, Clynes M, Gunaratnam C, McElvaney NG, Reeves EP.

EBioMedicine. 2017 Sep;23:173-184. doi: 10.1016/j.ebiom.2017.08.013. Epub 2017 Aug 16.


Risk factors for totally implantable venous access device-associated complications in cystic fibrosis.

McCarthy C, O'Carroll O, O'Brien ME, McEnery T, Franciosi A, Gunaratnam C, McElvaney NG.

Ir J Med Sci. 2018 May;187(2):429-434. doi: 10.1007/s11845-017-1672-2. Epub 2017 Aug 15.


X Chromosome-encoded MicroRNAs Are Functionally Increased in Cystic Fibrosis Monocytes.

McKiernan PJ, Molloy KP, Cryan SA, McElvaney NG, Greene CM.

Am J Respir Crit Care Med. 2018 Mar 1;197(5):668-670. doi: 10.1164/rccm.201707-1417LE. No abstract available.


Quantitative disease progression model of α-1 proteinase inhibitor therapy on computed tomography lung density in patients with α-1 antitrypsin deficiency.

Tortorici MA, Rogers JA, Vit O, Bexon M, Sandhaus RA, Burdon J, Chorostowska-Wynimko J, Thompson P, Stocks J, McElvaney NG, Chapman KR, Edelman JM.

Br J Clin Pharmacol. 2017 Nov;83(11):2386-2397. doi: 10.1111/bcp.13358. Epub 2017 Aug 11.


Brief Report: Genetic Variation of the α1 -Antitrypsin Gene Is Associated With Increased Autoantibody Production in Rheumatoid Arthritis.

McCarthy C, Orr C, Fee LT, Carroll TP, Dunlea DM, Hunt DJL, Dunne E, O'Connell P, McCarthy G, Kenny D, Fearon U, Veale DJ, Reeves EP, McElvaney NG.

Arthritis Rheumatol. 2017 Aug;69(8):1576-1579. doi: 10.1002/art.40127. Epub 2017 Jun 26.


5 Year Expression and Neutrophil Defect Repair after Gene Therapy in Alpha-1 Antitrypsin Deficiency.

Mueller C, Gernoux G, Gruntman AM, Borel F, Reeves EP, Calcedo R, Rouhani FN, Yachnis A, Humphries M, Campbell-Thompson M, Messina L, Chulay JD, Trapnell B, Wilson JM, McElvaney NG, Flotte TR.

Mol Ther. 2017 Jun 7;25(6):1387-1394. doi: 10.1016/j.ymthe.2017.03.029. Epub 2017 Apr 10.


Long-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE).

McElvaney NG, Burdon J, Holmes M, Glanville A, Wark PA, Thompson PJ, Hernandez P, Chlumsky J, Teschler H, Ficker JH, Seersholm N, Altraja A, Mäkitaro R, Chorostowska-Wynimko J, Sanak M, Stoicescu PI, Piitulainen E, Vit O, Wencker M, Tortorici MA, Fries M, Edelman JM, Chapman KR; RAPID Extension Trial Group.

Lancet Respir Med. 2017 Jan;5(1):51-60. doi: 10.1016/S2213-2600(16)30430-1. Epub 2016 Dec 2. Erratum in: Lancet Respir Med. 2017 Feb;5(2):e13.


The Role of Neutrophils in Alpha-1 Antitrypsin Deficiency.

McCarthy C, Reeves EP, McElvaney NG.

Ann Am Thorac Soc. 2016 Aug;13 Suppl 4:S297-304. doi: 10.1513/AnnalsATS.201509-634KV. Review.


α1-Antitrypsin deficiency.

Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG.

Nat Rev Dis Primers. 2016 Jul 28;2:16051. doi: 10.1038/nrdp.2016.51. Review. Erratum in: Nat Rev Dis Primers. 2018 Oct 29;4(1):40.


Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences.

Lavelle GM, White MM, Browne N, McElvaney NG, Reeves EP.

Biomed Res Int. 2016;2016:5258727. doi: 10.1155/2016/5258727. Epub 2016 Jun 1. Review.


Alpha-1 Antitrypsin Therapy in Cystic Fibrosis and the Lung Disease Associated with Alpha-1 Antitrypsin Deficiency.

McElvaney NG.

Ann Am Thorac Soc. 2016 Apr;13 Suppl 2:S191-6. doi: 10.1513/AnnalsATS.201504-245KV. Review.


Chitinase activation in patients with fungus-associated cystic fibrosis lung disease.

Hector A, Chotirmall SH, Lavelle GM, Mirković B, Horan D, Eichler L, Mezger M, Singh A, Ralhan A, Berenbrinker S, Mack I, Ensenauer R, Riethmüller J, Graepler-Mainka U, Murray MA, Griese M, McElvaney NG, Hartl D.

J Allergy Clin Immunol. 2016 Oct;138(4):1183-1189.e4. doi: 10.1016/j.jaci.2016.01.031. Epub 2016 Apr 4.



Lavelle GM, Mirković B, Chotirmall SH, McElvaney NG.

J Allergy Clin Immunol. 2016 Mar;137(3):969-70. doi: 10.1016/j.jaci.2015.11.013. Epub 2016 Jan 9. No abstract available.


Tumor necrosis factor-α driven inflammation in alpha-1 antitrypsin deficiency: a new model of pathogenesis and treatment.

Hurley K, Reeves EP, Carroll TP, McElvaney NG.

Expert Rev Respir Med. 2016 Feb;10(2):207-22. doi: 10.1586/17476348.2016.1127759. Epub 2015 Dec 18. Review.


Blood basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis in cystic fibrosis.

Gernez Y, Waters J, Mirković B, Lavelle GM, Dunn CE, Davies ZA, Everson C, Tirouvanziam R, Silver E, Wallenstein S, Chotirmall SH, McElvaney NG, Herzenberg LA, Moss RB.

Eur Respir J. 2016 Jan;47(1):177-85. doi: 10.1183/13993003.01068-2015. Epub 2015 Nov 19. Erratum in: Eur Respir J. 2016 Jun;47(6):1892.


miRNA-221 is elevated in cystic fibrosis airway epithelial cells and regulates expression of ATF6.

Oglesby IK, Agrawal R, Mall MA, McElvaney NG, Greene CM.

Mol Cell Pediatr. 2015 Dec;2(1):1. doi: 10.1186/s40348-014-0012-0. Epub 2015 Jan 7.


Unusual Acute Sequelae of α1-Antitrypsin Deficiency: A Myriad of Symptoms With One Common Cure.

Franciosiz AN, McCarthy C, Carroll TP, McElvaney NG.

Chest. 2015 Nov;148(5):e136-e138. doi: 10.1378/chest.15-0699.


The basophil surface marker CD203c identifies Aspergillus species sensitization in patients with cystic fibrosis.

Mirković B, Lavelle GM, Azim AA, Helma K, Gargoum FS, Molloy K, Gernez Y, Dunne K, Renwick J, Murphy P, Moss RB, Greene CM, Gunaratnam C, Chotirmall SH, McElvaney NG.

J Allergy Clin Immunol. 2016 Feb;137(2):436-443.e9. doi: 10.1016/j.jaci.2015.07.045. Epub 2015 Sep 18.


The BLT1 Inhibitory Function of α-1 Antitrypsin Augmentation Therapy Disrupts Leukotriene B4 Neutrophil Signaling.

O'Dwyer CA, O'Brien ME, Wormald MR, White MM, Banville N, Hurley K, McCarthy C, McElvaney NG, Reeves EP.

J Immunol. 2015 Oct 15;195(8):3628-41. doi: 10.4049/jimmunol.1500038. Epub 2015 Sep 14.


The Role of Short-Chain Fatty Acids, Produced by Anaerobic Bacteria, in the Cystic Fibrosis Airway.

Mirković B, Murray MA, Lavelle GM, Molloy K, Azim AA, Gunaratnam C, Healy F, Slattery D, McNally P, Hatch J, Wolfgang M, Tunney MM, Muhlebach MS, Devery R, Greene CM, McElvaney NG.

Am J Respir Crit Care Med. 2015 Dec 1;192(11):1314-24. doi: 10.1164/rccm.201505-0943OC.


Inhaled hypertonic saline for cystic fibrosis: Reviewing the potential evidence for modulation of neutrophil signalling and function.

Reeves EP, McCarthy C, McElvaney OJ, Vijayan MS, White MM, Dunlea DM, Pohl K, Lacey N, McElvaney NG.

World J Crit Care Med. 2015 Aug 4;4(3):179-91. doi: 10.5492/wjccm.v4.i3.179. eCollection 2015 Aug 4. Review.


The Ability of Secretory Leukocyte Protease Inhibitor to Inhibit Apoptosis in Monocytes Is Independent of Its Antiprotease Activity.

McGarry N, Greene CM, McElvaney NG, Weldon S, Taggart CC.

J Immunol Res. 2015;2015:507315. doi: 10.1155/2015/507315. Epub 2015 Jul 12.


miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production.

Oglesby IK, Vencken SF, Agrawal R, Gaughan K, Molloy K, Higgins G, McNally P, McElvaney NG, Mall MA, Greene CM.

Eur Respir J. 2015 Nov;46(5):1350-60. doi: 10.1183/09031936.00163414. Epub 2015 Jul 9.


Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

Chapman KR, Burdon JG, Piitulainen E, Sandhaus RA, Seersholm N, Stocks JM, Stoel BC, Huang L, Yao Z, Edelman JM, McElvaney NG; RAPID Trial Study Group.

Lancet. 2015 Jul 25;386(9991):360-8. doi: 10.1016/S0140-6736(15)60860-1. Epub 2015 May 27.


The impact of smoke exposure on the clinical phenotype of alpha-1 antitrypsin deficiency in Ireland: exploiting a national registry to understand a rare disease.

O'Brien ME, Pennycooke K, Carroll TP, Shum J, Fee LT, O'Connor C, Logan PM, Reeves EP, McElvaney NG.

COPD. 2015 May;12 Suppl 1:2-9. doi: 10.3109/15412555.2015.1021913.


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