Paul McNally

Paul McNally

Associate Professor of Paediatrics

Paul graduated from the UCD School of Medicine in 1998. He completed his paediatric medical training in Ireland, and subsequently completed a 2-year MD in Cystic Fibrosis (CF) lung disease in the Royal College of Surgeons in Ireland. Paul undertook pulmonology fellowship training in the Children’s Hospital of Philadelphia from 2007 to 2009. He was appointed as a consultant in Paediatric Respiratory Medicine at Children’s Health Ireland at Crumlin (CHI at Crumlin) in 2009. In August 2015, Paul was appointed as Associate Professor of Paediatrics at RCSI. Paul is director of the CF centre in CHI at Crumlin and leads the CF research group at NCRC. Paul is clinical lead for Paediatric Respiratory Medicine in the Dublin Paediatric Hospitals. He is a member of the medical and scientific council of CF Ireland and the Scientific Advisory Committee of the CF Registry of Ireland. Paul is a member of the working group of the National Clinical Programme for Cystic Fibrosis and is a member of the advisory board for the National Clinical Programme for Paediatrics. Paul’s main research interest is early CF lung disease, in particular around the question of why some children have more severe lung disease, and how we can detect these children earlier and modify their treatment.

Paul McNally

Associate Professor of Paediatrics

The Study of Host Immunity and Early Lung Disease in CF (SHIELD CF)

We are currently conducting a long term ongoing research study looking at the evolution of infection and inflammation in the airways of children with cystic fibrosis (CF). Every year, children with CF undergo a procedure called a bronchoscopy. A small camera is inserted into the patients airways which allows us to look inside a patient’s lungs and identify any damage present. Biological samples such as bronchoalveolar lavage (BAL) fluid from the lung as well as urine, blood and samples from the throat and nose are taken during the procedure and stored in a specialised ‘biobank’ . We also collect some of the patient’s clinical information. SHIELD CF is in place across three sites; OLCH Crumlin, Tallaght hospital and University hospital Limerick. There are over 8000 samples in the biobank already and these have contributed to more than 25 peer reviewed publications, helping us to understand various different aspects of CF lung disease.

The airway microbiome in children with CF (AIM CF)

In recent years we have come to understand that a vast array of bacteria lives in our airways – even in healthy people. It has become clear that the interaction between the healthy and harmful bacteria in the lungs is complex. We are conducting a long term and very wide-ranging study in children with CF looking at how these bacteria vary over time and whether they are related to lung inflammation, changes in antibiotic use and lung function at school age.

Inflammatory and Microbiological Implications of Pooling of Bronchoalveolar Lavage samples in Preschool Children with Cystic Fibrosis (the POOL study)

Bronchoalveolar lavage fluid is collected when salt water is instilled temporarily in the lungs during a bronchoscopy procedure. It collects the proteins and bacteria from the lower airways that otherwise could not be collected. This is potentially a very useful test for clinical trials of new medications in children with CF, but there are several different approaches internationally to the way the test is performed. This multicentre study seeks to determine if different ways of handling and processing the fluid impact on its utility as a marker of lung pathology.

CF Urinary biomarker study (CUBS)

The purpose of the CUBS study is to see if we can find proteins or ‘biomarkers’ in the urine that will help us to identify lung disease in children with CF. Biomarkers are biological molecules found in blood, other body fluids, or tissues that can be indicative of a particular disease or abnormal process such as inflammation. Lots of substances that are produced in the body are excreted, at least partially, in the urine. This has been shown to be true of biomarkers of lung damage in people with lung diseases. In some instances, urinary biomarkers have been shown to correlate well with lung infection or lung damage. This is of significant interest to doctors looking after children with CF as the collection of urine is a painless and harmless procedure, urine is always accessible and it is an easy fluid to work with. In the first part of the study, parents of children with CF will collect urine on a regular basis for 3 months at home and sent it to our partner lab in the UK (Mologic) to allow us to determine what the ‘normal’ range is (parents keep a symptom diary so we could determine when the children were well or sick). In the second part of this study, we will match urine collected with the corresponding bronchoalveolar lavage (BAL) fluid collected as part of the SHIELD CF project. We are trying to determine if there are substances in urine which are typically present at the time that we see a marker of lung damage called neutrophil elastase (NE) in the lung fluid. Earlier detection of lung damage and earlier intervention could prevent or slow down the progression of lung disease in children with CF.

Protease activity: Lung Measurement in Children with Cystic Fibrosis (PALM)

The presence of certain compounds called ‘proteases’ have been shown to be a risk factor for lung damage in children with CF. Proteases are enzymes that can degrade healthy lung tissue if present in abnormal amounts. There are lots of different types of proteases, and little is known about some of these in children with CF. It is not yet known if particular proteases are associated with infection or damage to the lungs. The study is designed to establish the degree and pattern of proteases in the lungs of preschool children with CF, and to determine if specific proteases are associated with either pulmonary infection or clinical disease parameters. This study might help us to discover meaningful tests in due course to determine whether harmful proteins are present in young babies and children with CF.

Children's Follow up Orkambi Real Word MBW Study (C-FORMS)

Cystic fibrosis is a common inherited life threatening disease which is particularly common in Ireland. People with CF have a mutation in a gene that produces a protein called cystic fibrosis transmembrane conductance protein or CFTR. CFTR regulates the movement of salt in and out of cells. When CFTR doesn’t work well the result is thick, sticky mucus in the respiratory, digestive and reproductive systems, and inability to fight lung infections. Medications have now been designed to specifically target the CFTR protein. These are called CTR modulators. One such modulator is Orkambi, which has recently been approved to treat children over 6 years in Ireland. In this study we are using a new type of specialised lung function technique called ‘multiple breath washout’ (MBW) to look at the lung function of children aged 6-11 years with CF. Children will undergo MBW and a new form of low dose CT scanning called ‘spirometrically controlled CT’ before they commence on Orkmabi. In the first study of its kind in the world, we will be working with colleagues in Rotterdam, London and Cincinnati to assess the effects of Orkambi on the lung structure and lung function of these Irish children for two years following commencement of Orkambi in a real world setting.

Predicting REsponses in Disease outcomes in CF using iPSCs for new CFTR Therapies (PREDiCT)

The introduction of promising new medications to treat the basic defect in people with CF holds great promise to improve the survival and health of our patients with CF. New CF treatment such as Orkambi are currently only available for CF patients with the most common mutations. Furthermore only individuals with common or well-recognised mutations are being recruited to clinical trials of these new investigational medications. There are likely to be a significant group of children and adults with rarer mutations who may be left behind, with no prospect of having new drugs licensed for use in these people. What is required here is a patient specific ‘test’ to determine whether an individual may benefit from treatment with a given drug. The purpose of this research is to create a repository or “bank” of a type of stem cell called “induced pluripotent stem cells” or iPSCs. Stem cells are special kinds of cells that can renew themselves. Under certain experimental conditions, iPSCs can be directed to grow into any type of tissue or organ. In the case of this study, we are working with colleagues in RCSI and Boston University to develop a special type of lung cell which can readily be used to determine whether the child’s own lung cells may respond to new CF drugs. Ultimately, we would like to develop a test that can be offered to all patients with cystic fibrosis, particularly those with rare mutations not currently able to avail of approved drugs, to determine whether or not they will respond to a given drug.

Role of Exosomes in CF Airway Pathogenesis (RECAP)

RECAP is a new joint project between our CF clinical team and our CF Basic Science core in NCRC led by Dr Judith Coppinger. The team has discovered that there are small capsules within cells called exosomes. These exosomes are released by lung cells from people with CF and can help regulate inflammation. Exosomes form part of a cell-to-cell communication system used by most cells to send messages across the human body. Understanding their role promises to improve our understanding of many diseases and how we treat them. These small exosomes are carriers of proteins and other signals that can impact neighbouring inflammatory cells in the fight against infection and may offer the potential to be markers of lung disease in CF. From our research we have discovered that CF cells produce more of these exosomes than healthy cells. Using state of the art scientific technologies, we examined what protein molecules are contained in these exosomes and found many proteins are involved in immune cell function and inflammation. We are now using samples collected through SHIELD CF in children of different ages, some of whom are on new CF modifier drugs to understand this phenomenon in greater detail and how exosomes may cause disease in CF and respond to treatment strategies. We hope to determine in the future whether certain exosomes will help us to non-invasively determine how patients are doing in terms of lung inflammation and damage.

Baseline LCI OF Children With OR without CF (BLOC WORC)

Understanding early lung disease in CF is an international focus to try and tailor treatment to slow or prevent decline in lung function. In Ireland, we currently have limited capacity to formally assess early lung disease in this group. In recent years, there has been a move towards closer monitoring and active treatment in younger patients with CF, in an effort to improve long term survival. Lung Clearance Index (LCI) is a lung function measurement performed using the multiple breath washout (MBW) method. The MBW technique is an effort independent, low-risk, non-invasive procedure performed on spontaneously breathing children. The LCI is a value that is used to indicate the performance of the lungs, particularly the smaller airways. LCI is much more sensitive than other established tests for CF lung disease and can be performed in younger children. It is therefore a very helpful test for doctors looking after children with CF. We have recently acquired the equipment for MBW testing (N2) and wish to validate the test in a population of healthy Irish children and in children with CF, both when well and unwell and to compare our results with established values using this equipment. We are working with our colleage Dr Barry Linnane in Limerick, who has been using this device for a number of years.

The list of publications below is automatically derived from MEDLINE/PubMed. As a result, there may be incorrect or missing publications.

McNally P, Davies J, Ciet P, Tiddens H, 2024 Feb 14, Reply to: Hierarchical CT Scoring Systems Cannot Discriminate Between Reversible Bronchiectasis and Mucus Plugs. Am J Respir Crit Care Med, DOI: 10.1164/rccm.202311-2199LE
Mainz JG, Lester K, Elnazir B, Williamson M, McKone E, Cox D, Linnane B, Zagoya C, Duckstein F, Barucha A, Davies JC, McNally P, RECOVER Study Group, 2023 Oct 7, Reduction in abdominal symptoms (CFAbd-Score), faecal M2-pyruvate-kinase and Calprotectin over one year of treatment with Elexacaftor-Tezacaftor-Ivacaftor in people with CF aged ≥12 years - The RECOVER study. J Cyst Fibros, DOI: 10.1016/j.jcf.2023.10.001
McNally P, Lester K, Stone G, Elnazir B, Williamson M, Cox D, Linnane B, Kirwan L, Rea D, O'Regan P, Semple T, Saunders C, Tiddens HAWM, McKone E, Davies JC, RECOVER Study Group, 2023 Nov 1, Improvement in Lung Clearance Index and Chest Computed Tomography Scores with Elexacaftor/Tezacaftor/Ivacaftor Treatment in People with Cystic Fibrosis Aged 12 Years and Older - The RECOVER Trial. Am J Respir Crit Care Med, DOI: 10.1164/rccm.202308-1317OC
Stone RG, Short C, Davies JC, McNally P, 2023 Sep 8, Chronic rhinosinusitis in the era of CFTR modulator therapy. J Cyst Fibros, DOI: 10.1016/j.jcf.2023.08.009
McNally P, Linnane B, Williamson M, Elnazir B, Short C, Saunders C, Kirwan L, David R, Kemner-Van de Corput MPC, Tiddens HAWM, Davies JC, Cox DW, 2023 Aug 11, The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with cystic fibrosis in a real-world setting. Respir Res, DOI: 10.1186/s12931-023-02497-0
Wainwright C, McColley SA, McNally P, Powers M, Ratjen F, Rayment JH, Retsch-Bogart G, Roesch E, Ahluwalia N, Chin A, Chu C, Lu M, Menon P, Waltz D, Weinstock T, Zelazoski L, Davies JC, 2023 Jul 1, Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged ⩾6 Years with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Clinical Trial. Am J Respir Crit Care Med, DOI: 10.1164/rccm.202301-0021OC
Trappe A, Lakkappa N, Carter S, Dillon E, Wynne K, McKone E, McNally P, Coppinger JA, 2023 Jul, Investigating serum extracellular vesicles in Cystic Fibrosis. J Cyst Fibros, DOI: 10.1016/j.jcf.2023.02.005
Gifford AH, Taylor-Cousar JL, Davies JC, McNally P, 2022 Dec, Update on Clinical Outcomes of Highly Effective Modulator Therapy. Clin Chest Med, DOI: 10.1016/j.ccm.2022.06.009
Berical A, Lee RE, Lu J, Beermann ML, Le Suer JA, Mithal A, Thomas D, Ranallo N, Peasley M, Stuffer A, Bukis K, Seymour R, Harrington J, Coote K, Valley H, Hurley K, McNally P, Mostoslavsky G, Mahoney J, Randell SH, Hawkins FJ, 2022 Jul 29, A multimodal iPSC platform for cystic fibrosis drug testing. Nat Commun, DOI: 10.1038/s41467-022-31854-8
Rowland M, McNally P, Fitzpatrick E, Bourke B, 2022 Jul, Transient elastography lacks precision in children. Pediatr Res, DOI: 10.1038/s41390-021-01694-1
Perrem LM, McNally P, Ratjen F, 2022 Feb, Apples to apples? Comparative analyses of national CF registries. Thorax, DOI: 10.1136/thoraxjnl-2021-217532
Rowland M, McGee A, McNally P, Bourke B, 2022 Jan 1, Performance Characteristics, Intra- and Inter-Operator Agreement of Transient Elastography in Pediatric Nonalcoholic Fatty Liver Disease. J Pediatr Gastroenterol Nutr, DOI: 10.1097/MPG.0000000000003254
McNally P, Butler D, Karpievitch YV, Linnane B, Ranganathan S, Stick SM, Hall GL, Schultz A, 2021 Sep 1, Ivacaftor and Airway Inflammation in Preschool Children with Cystic Fibrosis. Am J Respir Crit Care Med, DOI: 10.1164/rccm.202012-4332LE
Zemanick ET, Taylor-Cousar JL, Davies J, Gibson RL, Mall MA, McKone EF, McNally P, Ramsey BW, Rayment JH, Rowe SM, Tullis E, Ahluwalia N, Chu C, Ho T, Moskowitz SM, Noel S, Tian S, Waltz D, Weinstock TG, Xuan F, Wainwright CE, McColley SA, 2021 Jun 15, A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele. Am J Respir Crit Care Med, DOI: 10.1164/rccm.202102-0509OC
MacDonagh L, Farrell L, O'Reilly R, McNally P, Javadpour S, Cox DW, 2021 Jun, Efficacy and adherence of noninvasive ventilation treatment in children with Down syndrome. Pediatr Pulmonol, DOI: 10.1002/ppul.25308
Trappe A, Donnelly SC, McNally P, Coppinger JA, 2021 Oct, Role of extracellular vesicles in chronic lung disease. Thorax, DOI: 10.1136/thoraxjnl-2020-216370
Linnane B, Walsh AM, Walsh CJ, Crispie F, O'Sullivan O, Cotter PD, McDermott M, Renwick J, McNally P, 2021 Feb 26, The Lung Microbiome in Young Children with Cystic Fibrosis: A Prospective Cohort Study. Microorganisms, DOI: 10.3390/microorganisms9030492
Hutchinson I, McNally P, 2021 Jan, Appearance of Pancreatic Sufficiency and Discontinuation of Pancreatic Enzyme Replacement Therapy in Children with Cystic Fibrosis on Ivacaftor. Ann Am Thorac Soc, DOI: 10.1513/AnnalsATS.202006-614RL
Rowland M, McGee A, Broderick A, Drumm B, Connolly L, Daly LE, Drummond J, Fitzpatrick E, Linnane B, McCormick PA, McNally P, Rainford L, Bourke B, Cystic Fibrosis Registry of Ireland, Cystic Fibrosis Liver Disease Research Group, 2020 Oct, Repeatability of transient elastography in children. Pediatr Res, DOI: 10.1038/s41390-020-0916-4
Useckaite Z, Ward MP, Trappe A, Reilly R, Lennon J, Davage H, Matallanas D, Cassidy H, Dillon ET, Brennan K, Doyle SL, Carter S, Donnelly S, Linnane B, McKone EF, McNally P, Coppinger JA, 2020 Jun, Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis. Thorax, DOI: 10.1136/thoraxjnl-2019-214027
De Santi C, Fernández Fernández E, Gaul R, Vencken S, Glasgow A, Oglesby IK, Hurley K, Hawkins F, Mitash N, Mu F, Raoof R, Henshall DC, Cutrona MB, Simpson JC, Harvey BJ, Linnane B, McNally P, Cryan SA, MacLoughlin R, Swiatecka-Urban A, Greene CM, 2020 Apr 8, Precise Targeting of miRNA Sites Restores CFTR Activity in CF Bronchial Epithelial Cells. Mol Ther, DOI: 10.1016/j.ymthe.2020.02.001
Hulme KM, Linnane B, McNally P, 2020 Mar 15, Lower Airway Infection in Preschool Children with Cystic Fibrosis: An International Comparison. Am J Respir Crit Care Med, DOI: 10.1164/rccm.201910-2064LE
Lynch A, Nicholson A, Bush A, McNally P, 2021 Feb, Pattern recognition in acute wheeze. Arch Dis Child Educ Pract Ed, DOI: 10.1136/archdischild-2018-314823
Greene ZM, Davenport J, Fitzgerald S, Russell JD, McNally P, 2019 Apr, Tracheostomy speaking valve modification in children: A standardized approach leads to widespread use. Pediatr Pulmonol, DOI: 10.1002/ppul.24209
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, 2019 Feb, Longitudinal Trends in Real-World Outcomes after Initiation of Ivacaftor. A Cohort Study from the Cystic Fibrosis Registry of Ireland. Ann Am Thorac Soc, DOI: 10.1513/AnnalsATS.201802-149OC
Linnane B, McNally P, 2018 Oct, Six-lobe bronchoalveolar lavage in children with cystic fibrosis. Lancet Respir Med, DOI: 10.1016/S2213-2600(18)30295-9
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, 2018 Jul, Anaerobic bacteria cultured from cystic fibrosis airways correlate to milder disease: a multisite study. Eur Respir J, DOI: 10.1183/13993003.00242-2018
Ringholz FC, Higgins G, Hatton A, Sassi A, Moukachar A, Fustero-Torre C, Hollenhorst M, Sermet-Gaudelus I, Harvey BJ, McNally P, Urbach V, 2018 Sep, Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways. J Cyst Fibros, DOI: 10.1016/j.jcf.2017.11.017
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, 2018 May, Pooling of bronchoalveolar lavage in children with cystic fibrosis does not adversely affect the microbiological yield or sensitivity in detecting pulmonary inflammation. J Cyst Fibros, DOI: 10.1016/j.jcf.2017.10.016
Higgins G, Fustero Torre C, Tyrrell J, McNally P, Harvey BJ, Urbach V, 2016 Jun 1, Lipoxin A4 prevents tight junction disruption and delays the colonization of cystic fibrosis bronchial epithelial cells by Pseudomonas aeruginosa. Am J Physiol Lung Cell Mol Physiol, DOI: 10.1152/ajplung.00368.2015
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, 2015 Dec 1, The Role of Short-Chain Fatty Acids, Produced by Anaerobic Bacteria, in the Cystic Fibrosis Airway. Am J Respir Crit Care Med, DOI: 10.1164/rccm.201505-0943OC
McGovern E, McNally P, O'Sullivan M, Phelan E, Sumner K, Best DH, McMahon CJ, 2016 Apr, Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension. Cardiol Young, DOI: 10.1017/S1047951115001006
Oglesby IK, Vencken SF, Agrawal R, Gaughan K, Molloy K, Higgins G, McNally P, McElvaney NG, Mall MA, Greene CM, 2015 Nov, miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production. Eur Respir J, DOI: 10.1183/09031936.00163414
Linnane B, Clarke D, Murray P, O'Sullivan N, Dunne C, McNally P, 2015 Oct, The benefit of taking a control sample when performing bronchoalveolar lavage. Thorax, DOI: 10.1136/thoraxjnl-2015-207319
Gaffney C, McNally P, 2015 Jun, Successful use of acetazolamide for central apnea in a child with Pitt-Hopkins syndrome. Am J Med Genet A, DOI: 10.1002/ajmg.a.37034
Higgins G, Ringholz F, Buchanan P, McNally P, Urbach V, 2015, Physiological impact of abnormal lipoxin A₄ production on cystic fibrosis airway epithelium and therapeutic potential. Biomed Res Int, DOI: 10.1155/2015/781087
Glasgow AM, Small DM, Scott A, McLean DT, Camper N, Hamid U, Hegarty S, Parekh D, O'Kane C, Lundy FT, McNally P, Elborn JS, McAuley DF, Weldon S, Taggart CC, 2015 May, A role for whey acidic protein four-disulfide-core 12 (WFDC12) in the regulation of the inflammatory response in the lung. Thorax, DOI: 10.1136/thoraxjnl-2014-206488
Renwick J, McNally P, John B, DeSantis T, Linnane B, Murphy P, SHIELD CF, 2014, The microbial community of the cystic fibrosis airway is disrupted in early life. PLoS One, DOI: 10.1371/journal.pone.0109798
Linnane B, Vaish S, Clarke D, O'Sullivan N, McNally P, 2015 Apr, The findings of a clinical surveillance bronchoalveolar lavage programme in pre-school patients with cystic fibrosis. Pediatr Pulmonol, DOI: 10.1002/ppul.23118
Small DM, Zani ML, Quinn DJ, Dallet-Choisy S, Glasgow AM, O'Kane C, McAuley DF, McNally P, Weldon S, Moreau T, Taggart CC, 2015 Jan, A functional variant of elafin with improved anti-inflammatory activity for pulmonary inflammation. Mol Ther, DOI: 10.1038/mt.2014.162
Al-Alawi M, Buchanan P, Verriere V, Higgins G, McCabe O, Costello RW, McNally P, Urbach V, Harvey BJ, 2014 Aug 1, Physiological levels of lipoxin A4 inhibit ENaC and restore airway surface liquid height in cystic fibrosis bronchial epithelium. Physiol Rep, DOI: 10.14814/phy2.12093
Weldon S, McNally P, McAuley DF, Oglesby IK, Wohlford-Lenane CL, Bartlett JA, Scott CJ, McElvaney NG, Greene CM, McCray PB Jr, Taggart CC, 2014 Jul 15, miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. Am J Respir Crit Care Med, DOI: 10.1164/rccm.201311-1986OC
Ringholz FC, Buchanan PJ, Clarke DT, Millar RG, McDermott M, Linnane B, Harvey BJ, McNally P, Urbach V, 2014 Aug, Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis. Eur Respir J, DOI: 10.1183/09031936.00106013
Higgins G, Buchanan P, Perriere M, Al-Alawi M, Costello RW, Verriere V, McNally P, Harvey BJ, Urbach V, 2014 Aug, Activation of P2RY11 and ATP release by lipoxin A4 restores the airway surface liquid layer and epithelial repair in cystic fibrosis. Am J Respir Cell Mol Biol, DOI: 10.1165/rcmb.2012-0424OC
O'Donovan C, Greally P, Canny G, McNally P, Hussey J, 2014 May, Active video games as an exercise tool for children with cystic fibrosis. J Cyst Fibros, DOI: 10.1016/j.jcf.2013.10.008
Ringholz F, Devins M, McNally P, 2014 Mar, Managing end stage lung disease in children. Paediatr Respir Rev, DOI: 10.1016/j.prrv.2013.07.002
Buchanan PJ, McNally P, Harvey BJ, Urbach V, 2013 Jul 15, Lipoxin A₄-mediated KATP potassium channel activation results in cystic fibrosis airway epithelial repair. Am J Physiol Lung Cell Mol Physiol, DOI: 10.1152/ajplung.00058.2013
Palfreeman A, Nyatsanza F, Farn H, McKinnon G, Schober P, McNally P, 2013 Jun, HIV testing for acute medical admissions: evaluation of a pilot study in Leicester, England. Sex Transm Infect, DOI: 10.1136/sextrans-2011-050401
Verrière V, Higgins G, Al-Alawi M, Costello RW, McNally P, Chiron R, Harvey BJ, Urbach V, 2012, Lipoxin A4 stimulates calcium-activated chloride currents and increases airway surface liquid height in normal and cystic fibrosis airway epithelia. PLoS One, DOI: 10.1371/journal.pone.0037746
Linnane B, McNally P, 2011 Oct 26, Bronchoalveolar lavage-directed therapy in children with cystic fibrosis and Pseudomonas aeruginosa infection. JAMA, DOI: 10.1001/jama.2011.1518
Vega-Carrascal I, Reeves EP, Niki T, Arikawa T, McNally P, O'Neill SJ, Hirashima M, McElvaney NG, 2011 Mar 1, Dysregulation of TIM-3-galectin-9 pathway in the cystic fibrosis airways. J Immunol, DOI: 10.4049/jimmunol.1003187
Weldon S, McNally P, McElvaney NG, Elborn JS, McAuley DF, Wartelle J, Belaaouaj A, Levine RL, Taggart CC, 2009 Dec 15, Decreased levels of secretory leucoprotease inhibitor in the Pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degradation. J Immunol, DOI: 10.4049/jimmunol.0901716
Nino G, McNally P, Miske LJ, Hickey E, Panitch HB, 2009 Nov, Use of intrapulmonary percussive ventilation (IPV) in the management of pulmonary complications of an infant with osteogenesis imperfecta. Pediatr Pulmonol, DOI: 10.1002/ppul.21112
Bergsson G, Reeves EP, McNally P, Chotirmall SH, Greene CM, Greally P, Murphy P, O'Neill SJ, McElvaney NG, 2009 Jul 1, LL-37 complexation with glycosaminoglycans in cystic fibrosis lungs inhibits antimicrobial activity, which can be restored by hypertonic saline. J Immunol, DOI: 10.4049/jimmunol.0803959
Guyot N, Butler MW, McNally P, Weldon S, Greene CM, Levine RL, O'Neill SJ, Taggart CC, McElvaney NG, 2008 Nov 21, Elafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosis. J Biol Chem, DOI: 10.1074/jbc.M803707200
McNally PG, Dean JD, Morris AD, Wilkinson PD, Compion G, Heller SR, 2007 May, Using continuous glucose monitoring to measure the frequency of low glucose values when using biphasic insulin aspart 30 compared with biphasic human insulin 30: a double-blind crossover study in individuals with type 2 diabetes. Diabetes Care, DOI: 10.2337/dc06-1328
McNally P, McNicholas F, Oslizlok P, 2007 Feb, The QT interval and psychotropic medications in children: recommendations for clinicians. Eur Child Adolesc Psychiatry, DOI: 10.1007/s00787-006-0573-0
Kong MF, Jogia R, Jackson S, Quinn M, McNally P, Davies M, 2005 Nov 12, Malignant melanoma presenting as a foot ulcer. Lancet, DOI: 10.1016/S0140-6736(05)67701-X

Funding Agency:National Children’s Research Centre
Project Title:The Study of Host Immunity and Early Lung Disease in Children with CF (SHIELD CF)
Start Date/End Date:2009 – present
Total Awarded:€350k
Funding Agency:National Children’s Research Centre / National Children’s Hospital foundation
Project Title:Polymicrobial Communities in The Lower Airways of Children with Cystic Fibrosis: Development, Diversity and Consequences on Lung InflammationStart Date/End
Start Date/End Date:2010 – present
Total Awarded:€280k
Funding Agency:National Children’s Research Centre
Project Title:Physiological Impact of Lipoxins on the Function of Lung Epithelium in Children with Cystic Fibrosis
Start Date/End Date:2010 – 2012
Total Awarded:€385k
Funding Agency:Health Research Board
Project Title:Lipoxin synthesis in children with cystic fibrosis and pro-resolution actions in airway epithelium
Start Date/End Date:2011 – 2013
Total Awarded:€350k
Funding Agency:Health Research Board Project
Project Title:The role of mucus and mucins in mediating P aeruginosa colonisation in the CF lung.
Start Date/End Date:2011-2013
Total Awarded: €172k
Funding Agency:National Children’s Research Centre
Project Title:MicroRNA modulation of aberrant interleukin-8 expression in cystic fibrosis bronchial epithelial cells.
Start Date/End Date:2013-2016
Total Awarded:€195k
Name:Dr Barry Linnane
Position:Consultant in Paediatric Respiratory Medicine
Role:Dr Linnane is paediatric CF centre director in University Hospital Limerick and co-founder of SHIELD CF. He is an active clinical researcher and local principle investigator on several international clinical trials.
Name:Dr Des Cox
Position:Consultant in Paediatric Respiratory Medicine
Role:Dr Cox is head of the Department of Respiratory Medicine in OLCHC, and an active lead researcher in the CF Research group. He is local principal investigator on several international clinical trials.
Name:Dr Judith Coppinger
Position: Senior Lecturer, RCSI
Role:Judith is an accomplished scientist who has worked in leading institutions in Ireland and North America, and published extensively in the areas of CF and cancer. Judith heads up the basic science research core in NCRC.
Name:Dr Peter Greally
Position:Consultant in Paediatric Respiratory Medicine
Role:Dr Greally is CF centre director in Tallaght Hospital. He is an active clinical researcher and local principle investigator on several international clinical trials.
Name:Dr David Rea
Position:Consultant Paediatric Radiologist
Role:Dr Rea is a consultant paediatric radiologist in OLCHC. He is lead radiologist for the SHIELD CF network. Dr Rea leads the imaging analysis aspects of SHIELD CF. OLCHC is part of the SciFi network – an international imaging network for children with CF
Name:Dr Michael McDermott
Position:Consultant Paediatric Pathologist
Role:Dr McDermott is a consultant paediatric pathologist in OLCHC. He is lead pathologist for the SHIELD CF network, and leads the optimization of techniques for cell imaging and analysis for SHIELD CF.
Name:Professor Ivo Leuschner
DepartmentPathology, Division of Paediatric Pathology, Institution: Christian-Albrechts
Institution:University Kiel,
Name:Dr. Christian Vokuhl
DepartmentPathology Institution
Institution:Christian-Albrechts University, Kiel
Name:Professor Manfred Gessler
Institution:Developmental Biochemistry, and Comprehensive Cancer Center Mainfranken, Wuerzburg University, 97074 Wuerzburg.
Name:Professor Norbert Graf
DepartmentPaediatric Oncology and Hematology
Institution:Saarland University Hospital, Homburg
Name:Professor Adrian Bracken
Institution:Smurfit Institute, Trinity College, Dublin
Name:Dr. Gerard Cagney
DepartmentConway Institute
Institution:University College Dublin
Name:Professor Catherine Greene
DepartmentDepartment of Microbiology
Institution:Royal College of Surgeons in Ireland
Name:Dr Killian Hurley
DepartmentDepartment of Medicine
Institution:Royal College of Surgeons in Ireland
Name:Professor Marianne Muhlebach
DepartmentDepartment of Paediatric Pulmonology
Institution:University of North Carolina
Name:Dr Finn Hawkins
DepartmentDepartment of Medicine
Institution:Boston University
Name:Professor Cliff Taggart
DepartmentDepartment of Medicine
Institution:Queens University Belfast
Country:Northern Ireland
Name:Professor Gerry McElvaney
DepartmentDepartment of Medicine
Institution:Royal College of Surgeons in Ireland
Name:Professor Peter Sly
DepartmentDepartment of Paediatrics
Institution:University of Queensland
Name:Professor Claire Wainwright
DepartmentDepartment of Paediatrics
Institution:University of Queensland
Name:Professor Jane Davies
DepartmentDepartment of Gene Therapy
Institution:Imperial College London
Name:Professor Colum Dunne
DepartmentDepartment of Medicine
Institution:University of Limerick
Name:Dr Paul Cotter
Name:Dr Gita Parekh
Institution:Mologic Ltd

Current Trials & Studies

Study NameStudy TitleStudy TypeSponsorSponsor Type
SHIELD CFThe Study of Host Immunity and Early Lung Disease in Children with CFNon-Interventional Study
VX14-661-110A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
VOICE: VX14-CFR-107Observational Registry of Cystic Fibrosis Patients in EuropeNon-interventional studyVertexIndustry Sponsored
PALM StudyPALM study (Protease activity: Lung measurement) – a longitudinal study in children with CF to establish spectrum and influence of protease antiprotease activity in CF Starting in JulyNon-Interventional Study
VX15-770-124A Phase 3, 2-Part, Open-label Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have a CFTR Gating MutationClinical TrialVertexIndustry Sponsored
VX15-770-126A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have a CFTR Gating Mutation.Clinical TrialVertexIndustry Sponsored
VX18-445-106A Phase 3 Study Evaluating the Pharmacokinetics, Safety, and Tolerability of VX-445/TEZ/IVA Triple Combination Therapy in Cystic Fibrosis Subjects 6 Through 11 Years of Age.Clinical TrialVertexIndustry Sponsored
VX18-445-113A Phase 3, Open-label Study evaluating the long-term Safety of VX-445 Combination Therapy in Subjects with Cystic Fibrosis.Clinical TrialVertexIndustry Sponsored
RECOVERReal World Clinical Outcomes with Novel Modifier Therapy Combinations in People with CF.Non-Interventional StudyRCSIAcademic- Investigator Led
SeagullsStudy to Evaluate the Additional Gains of Upper and Lower Lobe Sampling in Children with CF.Non-Interventional Study

Previous Trials & Studies

Study NameStudy TitleStudy TypeSponsorSponsor Type
VX15-371-101A Phase 2a, Randomized, Double-blind, Placebo-controlled, Incomplete Block, Crossover Study to Evaluate the Safety and Efficacy of VX-371 in Subjects Aged 12 Years or Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation, and Being Treated With OrkambiClinical TrialVertexIndustry Sponsored
VX14-661-106A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With IvacaftorClinical TrialVertexIndustry Sponsored
VX14-661-109A Phase 3 Study to Evaluate the Efficacy and Safety of Ivacaftor and VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
VX12-809-103A Phase 3, Randomised, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination with Ivacaftor in Subjects Aged 12 years and older with Cystic Fibrosis, Homozygous for the F508del CFTR Mutation (TRAFFIC)Clinical TrialVertexIndustry Sponsored
VX12-809-105A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR MutationClinical TrialVertexIndustry Sponsored
MPEX-209A Phase 3, Open-label, Randomised Trial to Evaluate the Safety and Efficacy of MP-376 Inhalation solution (Aeroquin) versus Tobramycin Inhalation Solution in stable Cystic Fibrosis patientsClinical TrialMpex Pharmaceuticals Inc.Industry Sponsored
VX08-770-103A Phase 3, 2-Part, Randomised, Double-Blind, Placebo-controlled, Parallel Group Study to Evaluate the Pharmacokinetics, Efficacy and Safety of VX-770 in Subjects Aged 6 -11 years with Cystic Fibrosis and G551D mutationClinical TrialVertexIndustry Sponsored
VX08-770-105An Open Label, Rollover Study to Evaluate the Long-Term Safety and Efficacy of VX-770 in Subjects with Cystic FibrosisClinical TrialVertexIndustry Sponsored
TR02-108Randomised, Open Label, Active controlled, multicentre study to assess the efficacy, safety and tolerability of ArikaceTM in Cystic Fibrosis Patients with Chronic Infection due to Pseudomonas AeruginosaClinical TrialInsmed IncIndustry Sponsored
TR02-110Long term safety and tolerability study of open-label liposomal amikacin for inhalation (Arikace) in Cystic Fibrosis patients with chronic infection due to Pseudomonas AeruginosaClinical TrialInsmed IncIndustry Sponsored
PsAer-IgYProspective randomised, placebo controlled, double-blind, multicentre study (Phase III) to evaluate clinical efficacy and safety of avian polyclonal anti-Pseudomonas antibodies (IgY) in prevention of recurrence of Pseudomonas aeruginosa infection in cystic fibrosis patientsClinical TrialMukoviszidosAcademic - Collaborative Group
Caregiver Burden StudyCaregiver Burden Study in Cystic Fibrosis in the United States, Germany, United Kingdom and IrelandNon-Interventional StudyVertexIndustry Sponsored
The POOL studyInflammatory and Microbiological Implications of Pooling of Bronchoalveolar Lavage samples in Preschool Children with Cystic FibrosisNon-Interventional Study
Rhinovirus pilot studyThe Role of Human Rhinovirus Infections in Young Children with Cystic Fibrosis - A Pilot StudyNon-Interventional Study
CUBS (CF Urinary biomarker study)CF Urinary biomarker studyNon-interventional study