ECFS 2020 - Optimizing pharmaceutical care in cystic fibrosis

150 w w ww.ecfs.eu OPTIMIZING PHARMACEUTICAL CARE IN CYSTIC FIBROSIS CF MEDICINES IN CHILDREN CHAPTER 8 [21] Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, et al. Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis. Am J Respir Crit Care Med. 2014;190(2):175-84. [22] Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. A CFTR Potentiator in Patients with Cystic Fibrosis and the G551DMutation. N Engl J Med. 2011;365(18):1663-72. [23] Davies JC, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, et al. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Lancet Respir Med. 2016;4(2):107-15. [24] De Boeck K, Munck A, Walker S, Faro A, Hiatt P, Gilmartin G, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros. 2014;13(6):674-80. [25] Moss RB, Flume PA, Elborn JS, Cooke J, Rowe SM, McColley SA, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His- CFTR muta- tion: a double-blind, randomised controlled trial. Lancet Respir Med. 2015;3(7):524-33. [15] Stelmach I, Korzeniewska A, Stelmach W, Majak P, Grzelewski T, Jerzynska J. Effects of montelukast treatment on clinical and inflammatory variables in patients with cystic fibrosis. Annals of Allergy, Asthma & Immunology. 2005;95(4):372-80. [16] Konstan MW, Döring G, Heltshe SL, Lands LC, Hilliard KA, Koker P, 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):148-55. [17] Konstan MW, Schluchter MD, Xue W, Davis PB. Clinical Use of Ibuprofen Is Associated with Slower FEV 1 Decline in Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2007;176(11):1084-9. [18] Konstan MW, VanDevanter DR, Sawicki GS, Pasta DJ, Foreman AJ, Neiman EA, 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):485-93. [19] Yang C, Chilvers M, Montgomery M, Nolan SJ. Dornase alpha for cystic fibrosis. Cochrane Database of Systematic Reviews 206, Issue 4. Art. No.: CD001127. [20] O’Riordan TG, Donn KH, Hodsman P, Ansede JH, Newcomb T, Lewis SA, et al. Acute Hyperkalemia Associated with Inhalation of a Potent ENaC Antagonist: Phase 1 Trial of GS-9411. J Aerosol Med Pulm Drug Deliv. 2014;27(3):200-8.