https://orcid.org/0000-0002-1705-4780
References
- Sly PD, Gangell CL, Chen L, et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013;368(21):1963–1970. doi: 10.1056/NEJMoa1301725
- Roesch EA, Nichols DP, Chmiel JF. Inflammation in cystic fibrosis: an update. Pediatr Pulmonol. 2018;53(S3):S30–s50. doi: 10.1002/ppul.24129
- Schaupp L, Addante A, Völler M, et al. Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis. Eur Respir J. 2023;62(2):2202153. doi: 10.1183/13993003.02153-2022
- Casey M, Gabillard-Lefort C, McElvaney OF, et al. Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis. Thorax. 2023;78(8):835–839. doi: 10.1136/thorax-2022-219943
- Auerbach HS, Kirkpatrick J, Williams M, et al. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet. 1985;2(8457):686–688. doi: 10.1016/S0140-6736(85)92929-0
- Konstan MW, Döring G, Heltshe SL, et al. A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis. J Cyst Fibros. 2014;13(2):148–155. doi: 10.1016/j.jcf.2013.12.009
- Döring G, Bragonzi A, Paroni M, et al. BIIL 284 reduces neutrophil numbers but increases P. aeruginosa bacteremia and inflammation in mouse lungs. J Cyst Fibros. 2014;13(2):156–163. doi: 10.1016/j.jcf.2013.10.007
- Konstan MW, VanDevanter DR, Sawicki GS, et al. Association of high-dose ibuprofen use, lung function decline, and long-term survival in children with cystic fibrosis. Ann Am Thorac Soc. 2018;15(4):485–493. doi: 10.1513/AnnalsATS.201706-486OC
- Konstan MW, Byard PJ, Hoppel CL, et al. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 1995;332(13):848–854. doi: 10.1056/NEJM199503303321303
- Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003;290(13):1749–1756. doi: 10.1001/jama.290.13.1749
- Nichols DP, Odem-Davis K, Cogen JD, et al. Pulmonary outcomes associated with long-term azithromycin therapy in cystic fibrosis. Am J Respir Crit Care Med. 2020;201(4):430–437. doi: 10.1164/rccm.201906-1206OC
- Chmiel JF, Flume P, Downey DG, et al. Safety and efficacy of lenabasum in a phase 2 randomized, placebo-controlled trial in adults with cystic fibrosis. J Cyst Fibros. 2021;20(1):78–85. doi: 10.1016/j.jcf.2020.09.008
- Mayer-Hamblett N, Clancy JP, Jain R, et al. Advancing the pipeline of cystic fibrosis clinical trials: a new roadmap with a global trial network perspective. Lancet Respir Med. 2023;11(10):932–944. doi: 10.1016/S2213-2600(23)00297-7
- Elborn JS, Konstan MW, Taylor-Cousar JL, et al. Empire-CF study: a phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis. J Cyst Fibros. 2021;20(6):1026–1034. doi: 10.1016/j.jcf.2021.08.007
- McElvaney OJ, Heltshe SL, Odem-Davis K, et al. Adjunctive systemic corticosteroids for pulmonary exacerbations of cystic fibrosis. Ann Am Thorac Soc. 2023. doi: 10.1513/AnnalsATS.202308-673OC
- Keir HR, Fong CJ, Crichton ML, et al. Personalised anti-inflammatory therapy for bronchiectasis and cystic fibrosis: selecting patients for controlled trials of neutrophil elastase inhibition. ERJ Open Res. 2019;5(1). doi: 10.1183/23120541.00252-2018