References
1. Stoltz DA, Meyerholz DK, Welsh MJ. Origins of cystic fibrosis lung
disease. The New England journal of medicine.2015;372(4):351-362.
2. Hoegger MJ, Fischer AJ, McMenimen JD, et al. Cystic fibrosis.
Impaired mucus detachment disrupts mucociliary transport in a piglet
model of cystic fibrosis. Science. 2014;345(6198):818-822.
3. Birket SE, Chu KK, Liu L, et al. A Functional Anatomic Defect of the
Cystic Fibrosis Airway. American journal of respiratory and
critical care medicine. 2014.
4. Regnis JA, Robinson M, Bailey DL, et al. Mucociliary clearance in
patients with cystic fibrosis and in normal subjects. American
journal of respiratory and critical care medicine. 1994;150(1):66-71.
5. Flume PA, Robinson KA, O’Sullivan BP, et al. Cystic fibrosis
pulmonary guidelines: airway clearance therapies. Respiratory
care. 2009;54(4):522-537.
6. Ward N, Stiller K, Holland AE, Australian Cystic Fibrosis Exercise
Survey g. Exercise is commonly used as a substitute for traditional
airway clearance techniques by adults with cystic fibrosis in Australia:
a survey. J Physiother. 2019;65(1):43-50.
7. Hoo ZH, Daniels T, Wildman MJ, Teare MD, Bradley JM. Airway clearance
techniques used by people with cystic fibrosis in the UK.Physiotherapy. 2015;101(4):340-348.
8. Dwyer TJ, Daviskas E, Zainuldin R, et al. Effects of exercise and
airway clearance (positive expiratory pressure) on mucus clearance in
cystic fibrosis: a randomised crossover trial. The European
respiratory journal. 2019;53(4).
9. McIlwaine MP, Lee Son NM, Richmond ML. Physiotherapy and cystic
fibrosis: what is the evidence base? Current opinion in pulmonary
medicine. 2014;20(6):613-617.
10. Oates GR, Stepanikova I, Rowe SM, Gamble S, Gutierrez HH, Harris WT.
Objective Versus Self-Reported Adherence to Airway Clearance Therapy in
Cystic Fibrosis. Respiratory care. 2019;64(2):176-181.
11. Mikesell CL, Kempainen RR, Laguna TA, et al. Objective Measurement
of Adherence to Out-Patient Airway Clearance Therapy by High-Frequency
Chest Wall Compression in Cystic Fibrosis. Respiratory care.2017;62(7):920-927.
12. Benoit CM, Christensen E, Nickel AJ, et al. Objective Measures of
Vest Therapy Adherence Among Pediatric Subjects With Cystic Fibrosis.Respiratory care. 2020;65(12):1831-1837.
13. Szczesniak RD, Li D, Su W, et al. Phenotypes of Rapid Cystic
Fibrosis Lung Disease Progression during Adolescence and Young
Adulthood. American journal of respiratory and critical care
medicine. 2017;196(4):471-478.
14. O’Sullivan KJ, Collins L, McGrath D, Linnane B, O’Sullivan L, Dunne
CP. Oscillating Positive Expiratory Pressure Therapy May Be Performed
Poorly by Children With Cystic Fibrosis. Respiratory care.2019;64(4):398-405.
15. Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in
patients with cystic fibrosis and the G551D mutation. The New
England journal of medicine. 2011;365(18):1663-1672.
16. Rowe SM, Daines C, Ringshausen FC, et al. Tezacaftor-Ivacaftor in
Residual-Function Heterozygotes with Cystic Fibrosis. The New
England journal of medicine. 2017;377(21):2024-2035.
17. Taylor-Cousar JL, Munck A, McKone EF, et al. Tezacaftor-Ivacaftor in
Patients with Cystic Fibrosis Homozygous for Phe508del. The New
England journal of medicine. 2017;377(21):2013-2023.
18. Heijerman HGM, McKone EF, Downey DG, et al. Efficacy and safety of
the elexacaftor plus tezacaftor plus ivacaftor combination regimen in
people with cystic fibrosis homozygous for the F508del mutation: a
double-blind, randomised, phase 3 trial. Lancet.2019;394(10212):1940-1948.
19. Middleton PG, Mall MA, Drevinek P, et al.
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single
Phe508del Allele. The New England journal of medicine.2019;381(19):1809-1819.
20. Donaldson SH, Laube BL, Corcoran TE, et al. Effect of ivacaftor on
mucociliary clearance and clinical outcomes in cystic fibrosis patients
with G551D-CFTR. JCI insight. 2018;3(24).
21. Adam RJ, Hisert KB, Dodd JD, et al. Acute administration of
ivacaftor to people with cystic fibrosis and a mutation reveals smooth
muscle abnormalities. JCI insight. 2016;1(4):e86183.
22. Gifford AH, Mayer-Hamblett N, Pearson K, Nichols DP. Answering the
call to address cystic fibrosis treatment burden in the era of highly
effective CFTR modulator therapy. Journal of cystic fibrosis :
official journal of the European Cystic Fibrosis Society.2020;19(5):762-767.
23. Sole A, Olveira C, Perez I, et al. Development and electronic
validation of the revised Cystic Fibrosis Questionnaire (CFQ-R
Teen/Adult): New tool for monitoring psychosocial health in CF.Journal of cystic fibrosis : official journal of the European
Cystic Fibrosis Society. 2018;17(5):672-679.
24. Schneiderman-Walker J, Wilkes DL, Strug L, et al. Sex differences in
habitual physical activity and lung function decline in children with
cystic fibrosis. The Journal of pediatrics. 2005;147(3):321-326.
25. Valencia-Peris A, Lizandra J, Moya-Mata I, Gomez-Gonzalvo F,
Castillo-Corullon S, Escribano A. Comparison of Physical Activity and
Sedentary Behaviour between Schoolchildren with Cystic Fibrosis and
Healthy Controls: A Gender Analysis. International journal of
environmental research and public health. 2021;18(10).
26. Rowbotham NJ, Smith SJ, Davies G, et al. Can exercise replace airway
clearance techniques in cystic fibrosis? A survey of patients and
healthcare professionals. Journal of cystic fibrosis : official
journal of the European Cystic Fibrosis Society. 2020;19(4):e19-e24.
27. Doull I. Cystic fibrosis 2019: Year in review. Paediatric
respiratory reviews. 2020;35:95-98.
28. Ding S, Zhong C. Exercise and Cystic Fibrosis. Advances in
experimental medicine and biology. 2020;1228:381-391.
29. Ward N, Morrow S, Stiller K, Holland AE. Exercise as a substitute
for traditional airway clearance in cystic fibrosis: a systematic
review. Thorax. 2020.
30. Morrison L, Milroy S. Oscillating devices for airway clearance in
people with cystic fibrosis. The Cochrane database of systematic
reviews. 2020;4:CD006842.
31. Wilson LM, Morrison L, Robinson KA. Airway clearance techniques for
cystic fibrosis: an overview of Cochrane systematic reviews. The
Cochrane database of systematic reviews. 2019;1:CD011231.
32. Fischer AJ, Singh SB, LaMarche MM, et al. Sustained Coinfections
with Staphylococcus aureus and Pseudomonas aeruginosa in Cystic
Fibrosis. American journal of respiratory and critical care
medicine. 2020.
33. Porterfield HS, Maakestad LJ, LaMarche MM, et al. MRSA Strains with
Distinct Accessory Genes Predominate at Different Ages in Cystic
Fibrosis. Pediatric pulmonology. 2021.