References:
1. Wagener JS, Elkin EP, Pasta DJ, et al. Pulmonary function outcomes for assessing cystic fibrosis care. J Cyst Fibros . 2015;14(3):376-383. doi:10.1016/j.jcf.2014.11.008
2. Stylemans D, Verbanck S, Vincken S, Vincken W, De Wachter E, Vanderhelst E. Pulmonary function patterns and their association with genotype and phenotype in adult cystic fibrosis patients. Acta Clin Belg . 2019;74(6):386-392. doi:10.1080/17843286.2018.1533716
3. Levine H, Cohen-Cymberknoh M, Klein N, et al. Reversible airway obstruction in cystic fibrosis: Common, but not associated with characteristics of asthma. J Cyst Fibros . 2016;15(5):652-659. doi:10.1016/j.jcf.2016.01.003
4. Bennett WD. Effect of beta-adrenergic agonists on mucociliary clearance. J Allergy Clin Immunol . 2002;110(6 Suppl):S291-297. doi:10.1067/mai.2002.129704
5. Wong LB, Miller IF, Yeates DB. Stimulation of ciliary beat frequency by autonomic agonists: in vivo. J Appl Physiol . 1988;65(2):971-981. doi:10.1152/jappl.1988.65.2.971
6. Wood RE, Wanner A, Hirsch J, Farrell PM. Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am Rev Respir Dis . 1975;111(6):733-738. doi:10.1164/arrd.1975.111.6.733
7. Delavoie F, Molinari M, Milliot M, et al. Salmeterol restores secretory functions in cystic fibrosis airway submucosal gland serous cells. Am J Respir Cell Mol Biol . 2009;40(4):388-397. doi:10.1165/rcmb.2008-0037OC
8. Taouil K, Hinnrasky J, Hologne C, Corlieu P, Klossek J-M, Puchelle E. Stimulation of beta 2-adrenergic receptor increases cystic fibrosis transmembrane conductance regulator expression in human airway epithelial cells through a cAMP/protein kinase A-independent pathway.J Biol Chem . 2003;278(19):17320-17327. doi:10.1074/jbc.M212227200
9. Barry PJ, Flume PA. Bronchodilators in cystic fibrosis: a critical analysis. Expert Review of Respiratory Medicine . 2017;11(1):13-20. doi:10.1080/17476348.2017.1246358
10. Balfour-Lynn IM, Elborn JS. “CF asthma”: what is it and what do we do about it? Thorax . 2002;57(8):742-748. doi:10.1136/thorax.57.8.742
11. Kent BD, Lane SJ, van Beek EJ, Dodd JD, Costello RW, Tiddens HAWM. Asthma and cystic fibrosis: a tangled web. Pediatr Pulmonol . 2014;49(3):205-213. doi:10.1002/ppul.22934
12. Graham BL, Steenbruggen I, Miller MR, et al. Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement. Am J Respir Crit Care Med . 2019;200(8):e70-e88. doi:10.1164/rccm.201908-1590ST
13. Enright PL, Lebowitz MD, Cockroft DW. Physiologic measures: pulmonary function tests. Asthma outcome. Am J Respir Crit Care Med . 1994;149(2 Pt 2):S9-18; discussion S19-20. doi:10.1164/ajrccm/149.2_Pt_2.S9
14. Pollak M, Shaw M, Wilson D, Grasemann H, Ratjen F. Bronchodilator Responsiveness in Children with Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis. Eur Respir J . Published online April 8, 2020. doi:10.1183/13993003.00175-2020
15. Pellegrino R. Interpretative strategies for lung function tests.European Respiratory Journal . 2005;26(5):948-968. doi:10.1183/09031936.05.00035205
16. Quanjer PH, Stanojevic S, Cole TJ, et al. Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations. Eur Respir J . 2012;40(6):1324-1343. doi:10.1183/09031936.00080312
17. Mellis CM, Levison H. Bronchial reactivity in cystic fibrosis.Pediatrics . 1978;61(3):446-450.
18. Eggleston PA, Rosenstein BJ, Stackhouse CM, Alexander MF. Airway hyperreactivity in cystic fibrosis. Clinical correlates and possible effects on the course of the disease. Chest . 1988;94(2):360-365. doi:10.1378/chest.94.2.360
19. Mitchell I, Corey M, Woenne R, Krastins IR, Levison H. Bronchial hyperreactivity in cystic fibrosis and asthma. J Pediatr . 1978;93(5):744-748. doi:10.1016/s0022-3476(78)81070-1
20. Matusovsky OS, Kachmar L, Ijpma G, et al. Contractile Properties of Intrapulmonary Airway Smooth Muscle in Cystic Fibrosis. Am J Respir Cell Mol Biol . 2019;60(4):434-444. doi:10.1165/rcmb.2018-0005OC
21. Balfour-Lynn IM, Welch K, Smith S. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev . 2019;7:CD001915. doi:10.1002/14651858.CD001915.pub6
22. Balfour-Lynn IM, Lees B, Hall P, et al. Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis. Am J Respir Crit Care Med . 2006;173(12):1356-1362. doi:10.1164/rccm.200511-1808OC
23. Bruce Marshall, Albert Faro, Alexander Elbert, et al. Cystic Fibrosis Foundation Patient Registry 2018 Annual Data Report Bethesda, Maryland.
24. Maturu VN, Agarwal R. Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta-analysis. Clin Exp Allergy . 2015;45(12):1765-1778. doi:10.1111/cea.12595