Cystic fibrosis.

Lancet. 2016; 388: 2519-2531Clancy J.P. Cotton C.U. Donaldson S.H. Solomon G.M. VanDevanter D.R. Boyle M.P. et al.

CFTR modulator theratyping: current status, gaps and future directions.

J Cyst Fibros. 2019; 18: 22-34

Cystic Fibrosis Foundation. Cystic fibrosis foundation patient registry 2018 patient registry annual data report. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2018-Patient-Registry-Annual-Data-Report.pdf. Accessed April 20, 2021.

European Cystic Fibrosis Society. Cystic fibrosis in Europe facts and figures. 2017. At-a-glance report. https://www.ecfs.eu/sites/default/files/general-content-files/working-groups/ecfs-patient-registry/At-a-Glance_2017_ECFSPR.pdf. Accessed April 20, 2021.

Cystic Fibrosis Worldwide. What is cystic fibrosis? https://www.cfww.org/what-is-cystic-fibrosis. Accessed June 7, 2021.

Acute exacerbation in interstitial lung disease.

Front Med. 2017; 4: 176Parkins M.D. Somayaji R. Waters V.J.

Epidemiology, biology, and impact of clonal Pseudomonas aeruginosa infections in cystic fibrosis.

Clin Microbiol Rev. 2018; 31 (-18): e00019Crull M.R. Ramos K.J. Caldwell E. Mayer-Hamblett N. Aitken M.L. Goss C.H.

Change in Pseudomonas aeruginosa prevalence in cystic fibrosis adults over time.

BMC Pulm Med. 2016; 16: 176Van Daele S.G. Franckx H. Verhelst R. Schelstraete P. Haerynck F. Van Simaey L. et al.

Epidemiology of Pseudomonas aeruginosa in a cystic fibrosis rehabilitation centre.

Eur Respir J. 2005; 25: 474-481Hatziagorou E. Orenti A. Drevinek P. Kashirskaya N. Mei-Zahav M. De Boeck K. et al.

Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.

J Cyst Fibros. 2019; 19: 376-383

The changing microbial epidemiology in cystic fibrosis.

Clin Microbiol Rev. 2010; 23: 299-323Razvi S. Quittell L. Sewall A. Quinton H. Marshall B. Saiman L.

Respiratory microbiology of patients with cystic fibrosis in the United States, 1995 to 2005.

Chest. 2009; 136: 1554-1560

Zolin, A., Orenti, A., Naehrlich, L., van Rens, J., Fox, A., Krasnyk, M., et al. on behalf of the ECFSPR. ECFS Patient Registry annual data report 2017. https://www.ecfs.eu/sites/default/files/general-content-images/working-groups/ecfs-patient-registry/ECFSPR_Report2017_v1.3.pdf. Accessed April 20, 2021.

Doring G. Flume P. Heijerman H. Elborn J.S.

Consensus Study G. Treatment of lung infection in patients with cystic fibrosis: current and future strategies.

J Cyst Fibros. 2012; 11: 461-479

Interactions between neutrophils and Pseudomonas aeruginosa in cystic fibrosis.

Pathogens. 2017; 6: 10Smyth A.R. Bell S.C. Bojcin S. Bryon M. Duff A. Flume P. et al.

European Cystic Fibrosis Society standards of care: best practice guidelines.

J Cyst Fibros. 2014; 13: S23-S42Mogayzel Jr., P.J. Naureckas E.T. Robinson K.A. Mueller G. Hadjiliadis D. Hoag J.B. et al.

Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health.

Am J Respir Crit Care Med. 2013; 187: 680-689Castellani C. Duff A.J.A. Bell S.C. Heijerman H.G.M. Munck A. Ratjen F. et al.

ECFS best practice guidelines: the 2018 revision.

J Cyst Fibros. 2018; 17: 153-178Meers P. Neville M. Malinin V. Scotto A.W. Sardaryan G. Kurumunda R. et al.

Biofilm penetration, triggered release and in vivo activity of inhaled liposomal amikacin in chronic Pseudomonas aeruginosa lung infections.

J Antimicrob Chemother. 2008; 61: 859-868Clancy J.P. Dupont L. Konstan M.W. Billings J. Fustik S. Goss C.H. et al.

Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection.

Thorax. 2013; 68: 818-825Bilton D. Pressler T. Fajac I. Clancy J.P. Sands D. Minic P. et al.

Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.

J Cyst Fibros. 2020; 19: 284-291Sole A. Olveira C. Perez I. Hervas D. Valentine V. Baca Yepez A.N. et al.

Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult): new tool for monitoring psychosocial health in CF.

J Cyst Fibros. 2018; 17: 672-679Kerem E. Viviani L. Zolin A. MacNeill S. Hatziagorou E. Ellemunter H. et al.

Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry.

Eur Respir J. 2014; 43: 125-133

Preserving lung function: the holy grail in managing cystic fibrosis.

Ann Am Thorac Soc. 2017; 14: 833-835Keating C. Poor A.D. Liu X. Chiuzan C. Backenroth D. Zhang Y. et al.

Reduced survival in adult cystic fibrosis despite attenuated lung function decline.

J Cyst Fibros. 2017; 16: 78-84Maliniak M.L. Stecenko A.A. McCarty N.A.

A longitudinal analysis of chronic MRSA and Pseudomonas aeruginosa co-infection in cystic fibrosis: a single-center study.

J Cyst Fibros. 2016; 15: 350-356Volkova N. Moy K. Evans J. Campbell D. Tian S. Simard C. et al.

Disease progression in patients with cystic fibrosis treated with ivacaftor: data from national US and UK registries.

J Cyst Fibros. 2020; 19: 68-79https://doi.org/10.1016/j.jcf.2019.05.015Gibson R.L. Emerson J. Mayer-Hamblett N. Burns J.L. McNamara S. Accurso F.J. et al.

Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis.

Pediatr Pulmonol. 2007; 42: 610-623Oermann C.M. Retsch-Bogart G.Z. Quittner A.L. Gibson R.L. McCoy K.S. Montgomery A.B. et al.

An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis.

Pediatr Pulmonol. 2010; 45: 1121-1134Ratjen F. Munck A. Kho P. Angyalosi G. Group E.S.

Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis: the ELITE trial.

Thorax. 2010; 65: 286-291Schuster A. Haliburn C. Doring G. Goldman M.H.

Freedom Study G. Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study.

Thorax. 2013; 68: 344-350Retsch-Bogart G.Z. Quittner A.L. Gibson R.L. Oermann C.M. McCoy K.S. Montgomery A.B. et al.

Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis.

Chest. 2009; 135: 1223-1232