References
[1] Tiddens HAMW, de Jong PA. Update on the Application of Chest
Computed Tomography Scanning to Cystic Fibrosis. Curr Opin Pulm Med 2006
Nov;12(6):433-9
[2] De Boeck K, Zolin A, Cuppens H, Olesen HV, Viviani L. The
relative frequency of CFTR mutation classes in European patients with
cystic fibrosis. J Cyst Fibros 13 (2014) 403–409
[3] Elborn JS. Cystic Fibrosis. Lancet 2016; 388: 2519–31
[4] Bell SC, De Boeck K, Amaral MD. New pharmacological approaches
for cystic fibrosis: promises, progress, pitfalls. Pharmacol Ther. 2015
Jan;145:19-34
[5] Wainwright C, Elborn J, Ramsey BW, Marigowda G, Huang X, Cipolli
M, Colombo C, Davies JC, De Boeck K, Flume PA, et al.
Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for
Phe508del CFTR. N Engl J Med. 2015 Jul 16;373(3):220-31
[6] Konstan MW, McKone EF, Moss RB, Marigowda G, Tian S, Waltz D,
Huang X, Lubarsky B, Rubin J, Millar SJ, Pasta DJ, Mayer-Hamblett N,
Goss CH, Morgan W, Sawicki GS. Assessment of safety and efficacy of
long-term treatment with combination lumacaftor and ivacaftor therapy in
patients with cystic fibrosis homozygous for the F508del-CFTR mutation
(PROGRESS): a phase 3 , extension study. Lancet Respir Med. 2017
Feb;5(2):107-118
[7] Whiting P, Al M, Burgers L, Westwood M, Ryder S, Hoogendoorn M,
Armstrong N, Allen A, Severens H, Kleijnen J. Ivacaftor for the
treatment of patients with cystic fibrosis and the G551D mutation: a
systematic review and cost-effectiveness analysis. Health Technol
Assess. 2014 Mar;18(18):1-106
[8] Hubert D, Chiron R, Camara B, Grenet D, Prévotat A, Bassinet L,
Dominique S, Rault G, Macey J, Honoré I, et al. Real-life initiation of
lumacaftor/ivacaftor combination in adults with cystic fibrosis
homozygous for the Phe508del CFTR mutation and severe lung disease. J
Cyst Fibros 16 (2017) 388–391
[9] Brody AS, Kosorok MR, Li Z, Broderick LS, Foster JL, Laxova A,
Bandla H, Farrell PM. Reproducibility of a scoring system for Computed
Tomography scanning in Cystic Fibrosis. J Thorac Imaging 2006 21;1:14-21
[10] Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R,
Coates A, Crapo R, Enright P, van der Grinten CP, Gustafsson P, et al.
Standardisation of spirometry. Eur Resp J 2005 26: 319-338
[11] Quanjer PH, Stanojevic S, Cole TJ, Baur X, Hall GL, Culver BH,
Enright PL, Hankinson JL, Ip MS, Zheng J, Stocks J. Multi-ethnic
reference values for spirometry for the 3–95-yr age range: the global
lung function 2012 equations. Eur Respir J 2012 40: 1324-1343
[12] Helbich TH, Heinz-Peer G, Eichler I, Wunderbaldinger P, Götz M,
Wojnarowski C, Brasch RC, Herold CJ. Cystic fibrosis: CT assessment of
lung involvement in children and adults. Radiology. 1999
Nov;213(2):537-44
[13] Brody AS, Klein JS, Molina PL, Quan J, Bean JA, Wilmott RW.
High-resolution computed tomography in young patients with cystic
fibrosis: distribution of abnormalities and correlation with pulmonary
function tests. J Pediatr. 2004 Jul;145(1):32-8.
[14] Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR,
Sagel SD, Hornick DB, Konstan MW, Donaldson S, et al. Effect of VX-770
in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J
Med. 2010 Nov 18;363(21):1991-2003
[15] de Jong PA, Nakano Y, Lequin MH, Mayo JR, Woods R, Paré PD,
Tiddens HA. Progressive damage on high resolution computed tomography
despite stable lung function in cystic fibrosis. Eur Respir J
2004;23:93-97