SELECTED PEER-REVIEWED PUBLICATIONS

  • Chilvers MA, Davies JC, Milla C, Tian S, Han Z, Cornell AG, Owen CA, Ratjen F. Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study. Lancet Respir Med. 2021 Jan 28:S2213-2600(20)30517-8. doi: 10.1016/S2213-2600(20)30517-8. Epub ahead of print. PMID: 33516285.
  • Stanojevic S, Davis SD, Perrem L, Shaw M, Retsch-Bogart G, Davis M, Jensen R, Clem CC, Isaac SM, Guido J, Jara S, France L, McDonald N, Solomon M, Sweezey N, Grasemann H, Waters V, Sanders DB, Ratjen FA. Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis. Eur Respir J. 2021 Feb 4:2003380. doi: 10.1183/13993003.03380-2020. Epub ahead of print. PMID: 33542049.
  • Nichols DP, Donaldson SH, Frederick CA, Freedman SD, Gelfond D, Hoffman LR, Kelly A, Narkewicz MR, Pittman JE, Ratjen F, Sagel SD, Rosenfeld M, Schwarzenberg SJ, Singh PK, Solomon GM, Stalvey MS, Kirby S, VanDalfsen JM, Clancy JP, Rowe SM. PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy. J Cyst Fibros. 2021 Feb 19:S1569-1993(21)00038-2. doi: 10.1016/j.jcf.2021.02.003. Epub ahead of print. PMID: 33619012.
  • Pollak M, Shaw M, Wilson D, Solomon M, Ratjen F, Grasemann H. Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations. Pediatr Pulmonol. 2021 Apr 8. doi: 10.1002/ppul.25409. Epub ahead of print. PMID: 33830642.
  • Bayfield KJ, Douglas TA, Rosenow T, Davies JC, Elborn SJ, Mall M, Paproki A, Ratjen F, Sly PD, Smyth AR, Stick S, Wainwright CE, Robinson PD. Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis. Thorax. 2021 Apr 29:thoraxjnl-2020-216085. doi: 10.1136/thoraxjnl-2020-216085. Epub ahead of print. PMID: 33927017.
  • Anagnostopoulou P, Latzin P, Jensen R, Stahl M, Harper A, Yammine S, Usemann J, Foong RE, Spycher B, Hall GL, Singer F, Stanojevic S, Mall MA, Ratjen F, Ramsey KA. Normative Data for Multiple Breath Washout Outcomes in School-Aged Caucasian Children. Eur Respir J. 2020 Apr 3;55(4):1901302. doi: 10.1183/13993003.01302-2019. [abstract]
  • Pollak M, Shaw M, Wilson D, Grasemann H, Ratjen F. Bronchodilator Responsiveness in Children With Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis. Eur Respir J. 2020 Apr 8:2000175. doi: 10.1183/13993003.00175-2020. Online ahead of print.
  • Bogart G, Ratjen F. Changes in the parent cystic fibrosis questionnaire-revised (CFQ-R) with respiratory symptoms in preschool children with cystic fibrosis J Cyst Fibros. 2020 May: 19(3):492-498. Doi: 10.1016/j.jcf.2020.02.017.
  • Shaw M, Khan U, Clancy JP, Donaldson SH, Sagel SD, Rowe SM, Ratjen F; PROSPECT Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study. J Cyst Fibros. 2020 Jun 6:S1569-1993(20)30160-0. doi: 10.1016/j.jcf.2020.05.010. Online ahead of print.
  • Mayer-Hamblett N, van Koningsbruggen-Rietschel S, Nichols DP, VanDevanter DR, Davies JC, Lee T, Durmowicz AG, Ratjen F, Konstan MW, Pearson K, Bell SC, Clancy JP, Taylor-Cousar JL, De Boeck K, Donaldson SH, Downey DG, Flume PA, Drevinek P, Goss CH, Fajac I, Magaret AS, Quon BS, Singleton SM, VanDalfsen JM, Retsch-Bogart GZ. Building Global Development Strategies for Cf Therapeutics During a Transitional CFTR Modulator Era. J Cyst Fibros. 2020 Jun 7:S1569-1993(20)30161-2. doi: 10.1016/j.jcf.2020.05.011. Online ahead of print. (Co-A)
  • Saunders C, Jensen R, Robinson PD, Stanojevic S, Klingel M, Short C, Davies JC, Ratjen F. Integrating the multiple breath washout test into international multicentre trials. J Cyst Fibros. 2020 Jul;19(4):602-607. doi: 10.1016/j.jcf.2019.11.006.
  • Sagel SD, Khan U, Heltshe SL, Clancy JP, Borowitz D, Gelfond D, Donaldson SH, Moran A, Ratjen F, VanDalfsen JM, Rowe SM; PROSPECT Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR. Ann Am Thorac Soc. 2020 Jul 9. doi: 10.1513/AnnalsATS.202002-144OC. Online ahead of
  • Stanojevic S, Fillipow N, Ratjen F. Paediatric Reproducibility Limits for the Forced Expiratory Volume in 1 s. Thorax. 2020 Aug 11:thoraxjnl-2020-214817. doi: 10.1136/thoraxjnl-2020-214817. Online ahead of print. PMID: 32788261.
  • Eber E, Trawinska-Bartnicka M, Sands D, Bellon G, Mellies U, Bolbás K, Quattrucci S, Mazurek H, Widmann R, Schoergenhofer C, Jilma B, Ratjen F. Aerosolized lancovutide in adolescents (≥12 years) and adults with cystic fibrosis – a randomized trial. J Cyst Fibros. 2020 Sep 1:S1569-1993(20)30830-4. doi: 10.1016/j.jcf.2020.08.014. Epub ahead of print. PMID: 32888826.
  • Faughnan ME, Mager JJ, Hetts SW, Palda VA, Lang-Robertson K, Buscarini E, Deslandres E, Kasthuri RS, Lausman A, Poetker D, Ratjen F, Chesnutt MS, Clancy M, Whitehead KJ, Al-Samkari H, Chakinala M, Conrad M, Cortes D, Crocione C, Darling J, de Gussem E, Derksen C, Dupuis-Girod S, Foy P, Geisthoff U, Gossage JR, Hammill A, Heimdal K, Henderson K, Iyer VN, Kjeldsen AD, Komiyama M, Korenblatt K, McDonald J, McMahon J, McWilliams J, Meek ME, Mei-Zahav M, Olitsky S, Palmer S, Pantalone R, Piccirillo JF, Plahn B, Porteous MEM, Post MC, Radovanovic I, Rochon PJ, Rodriguez-Lopez J, Sabba C, Serra M, Shovlin C, Sprecher D, White AJ, Winship I, Zarrabeitia R. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med. 2020 Sep 8. doi: 10.7326/M20-1443. Epub ahead of print. PMID: 32894695.
  • Perrem L, Ratjen F. Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis. Front Pharmacol. 2020 Sep 11;11:576293. doi: 10.3389/fphar.2020.576293. PMID: 33013419; PMCID: PMC7516261.
  • Perrem L, Stanojevic S, Shaw M, Jensen R, McDonald N, Isaac SM, Davis M, Clem C, Guido J, Jara S, France L, Solomon M, Grasemann H, Waters V, Sweezey N, Sanders DB, Davis SD, Ratjen F. Lung Clearance Index to Track Acute Respiratory Events in School-age Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2020 Oct 8. doi: 10.1164/rccm.202006-2433OC. Epub ahead of print. PMID: 33030967.
  • O’Neill K, Ferguson K, Cosgrove D, Tunney MM, De Soyza A, Carroll M, Chalmers JD, Gatheral T, Hill AT, Hurst JR, Johnson C, Loebinger MR, Angyalosi G, Haworth CS, Jensen R, Ratjen F, Saunders C, Short C, Davies JC, Elborn JS, Bradley JM. Multiple breath washout in bronchiectasis clinical trials: is it feasible? ERJ Open Res. 2020 Oct 13;6(4):00363-2019. doi: 10.1183/23120541.00363-2019. PMID: 33083441; PMCID: PMC7553113.
  • Bernasconi N, Kieninger E, Shaw M, Kurz J, Moeller A, Ratjen F, Rochat I, Stanojevic S, Singer F. CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis. J Cyst Fibros. 2020 Dec 18:S1569-1993(20)30941-3. doi: 10.1016/j.jcf.2020.12.009. Epub ahead of print. PMID: 33349584.
  • Filipow N, Davies G, Main E, Sebire NJ, Wallis C, Ratjen F, Stanojevic S. Unsupervised Phenotypic Clustering for Determining Clinical Status in Children with Cystic Fibrosis. Eur Respir J. 2021 Jan 14:2002881. doi: 10.1183/13993003.02881-2020. Epub ahead of print. PMID: 33446607.
  • Mayer-Hamblett N, Nichols DP, Odem-Davis K, Riekert KA, Sawicki GS, Donaldson SH, Ratjen F, Konstan MW, Simon N, Rosenbluth DB, Retsch-Bogart G, Clancy JP, VanDalfsen JM, Buckingham R, Gifford AH; Cystic Fibrosis Therapeutics Development Network and SIMPLIFY Investigators. Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Study Design. Ann Am Thorac Soc. 2021 Jan 19. doi: 10.1513/AnnalsATS.202010-1336SD. Epub ahead of print. PMID: 33465316.
  • Lu Z, Dai R, Kowalik K, Dubeau A, Lefebvre DL, Balkovec S, Becker AB, Mandhane PJ, Turvey SE, Lou W, Ratjen F, Moraes TJ, Sears MR, Gustafsson PM, Subbarao P. Newly developed multiple-breath washout reference equations from the CHILD Cohort Study: implications of poorly fitting equations. ERJ Open Res. 2021 Jan 25;7(1):00301-2020. doi: 10.1183/23120541.00301-2020. PMID: 33532481; PMCID: PMC7836672.
  • Bell, SC., Mall, MA., Gutierrez, H., Ratjen, F. et al. (2020). The future of cystic fibrosis care: a global perspective. Lancet Respir Med Volume 8, Issue 1, January 2020, Pages 65-124. [abstract]
  • Shaw, M., Oppelaar, M. C., Jensen, R., Stanojevic, S., Davis, S. D., Retsch‐Bogart, G., & Ratjen, F. A. (2020). The utility of moment ratios and abbreviated endpoints of the multiple breath washout test in preschool children with cystic fibrosis. Pediatric Pulmonology55(3), 649–653. doi: 10.1002/ppul.24618 [abstract]
  • Anagnostopoulou, P., et al. “Normative Data for Multiple Breath Washout Outcomes in School-Aged Caucasian Children.” European Respiratory Journal, 2019, p. 1901302., doi:10.1183/13993003.01302-2019. [abstract]
  • Grasemann, H., Klingel, M., Avolio, J., Prentice, C., Gonska, T., Tullis, E., & Ratjen, F. (2019). Long-term effect of CFTR modulator therapy on airway nitric oxide. European Respiratory Journal55(1), 1901113. doi: 10.1183/13993003.01113-2019 [abstract]
  • Saunders, C., Jensen, R., Robinson, P. D., Stanojevic, S., Klingel, M., Short, C., … Ratjen, F. (2019). Integrating the multiple breath washout test into international multicentre trials. Journal of Cystic Fibrosis. doi: 10.1016/j.jcf.2019.11.006 [abstract]
  • Ratjen, F., Jensen, R., Klingel, M., Mcdonald, R., Moore, C., Benseler, N., . . . Stanojevic, S. (2019). Effect of changes in tidal volume on multiple breath washout outcomes. Plos One, 14(7). doi:10.1371/journal.pone.0219309 [abstract]
  • Klingel, M., Stanojevic, S., Tullis, E., Ratjen, F., & Waters, V. (2019). Oral Azithromycin and Response to Pulmonary Exacerbations Treated with Intravenous Tobramycin in Children with Cystic Fibrosis. Annals of the American Thoracic Society, 16(7), 861-867. doi:10.1513/annalsats.201811-774oc [abstract]
  • Ratjen, F., Davis, SD., Stanojevic S., Kronmal RA., Hinckley Stukovsky KD. (2019). Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Respir Med, S2213-2600(19)30187-0. doi: 10.1016/S2213-2600(19)30187-0. [abstract]
  • Perrem, L., Stanojevic, S., Solomon, M., Carpenter, S., & Ratjen, F. (2019). Incidence and risk factors of paediatric cystic fibrosis-related diabetes. Journal of Cystic Fibrosis. doi:10.1016/j.jcf.2019.04.015 [abstract]
  • Rayment, J. H., Couch, M. J., Mcdonald, N., Kanhere, N., Manson, D., Santyr, G., & Ratjen, F. (2019). Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis. European Respiratory Journal, 53(5), 1802188. doi:10.1183/13993003.02188-2018 [abstract]
  • Perrem, L., Rayment, J. H., & Ratjen, F. (2018). The lung clearance index as a monitoring tool in cystic fibrosis. Current Opinion in Pulmonary Medicine, 1. doi:10.1097/mcp.0000000000000515 [abstract]
  • Ratjen, F., Klingel, M., Black, P., Powers, M. R., Grasemann, H., Solomon, M., . . . Rosenfeld, M. (2018). Changes in Lung Clearance Index in Preschool Cystic Fibrosis Patients Treated with Ivacaftor. American Journal of Respiratory and Critical Care Medicine. doi:10.1164/rccm.201802-0243le [abstract]
  • Robinson, PD., et al. “Comparison of Facemask and Mouthpiece Interfaces for Multiple Breath Washout Measurements.” Journal of Cystic Fibrosis, 2018, doi:10.1016/j.jcf.2018.01.003. [abstract]
  • Robinson, PD., et al. “Preschool Multiple-Breath Washout Testing. An Official American Thoracic Society Technical Statement.” American Journal of Respiratory and Critical Care Medicine, vol. 197, no. 5, 2018, doi:10.1164/rccm.201801-0074st. [abstract]
  • Rayment JH, Stanojevic S, Davis SD, Retsch-Bogart G, Ratjen F. Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis. Thorax Published Online First: 15 February 2018. doi: 10.1136/thoraxjnl-2017-210979. [abstract]
  • Stanojevic, S., Davis, SD., Retsch-Bogart, G., Webster, H., Davis, M., Johnson, RC., Jensen, R., Pizarro, ME., Kane, M., Clem, CC., Schornick, L., Subbarao, P., Ratjen, F. (2017). Progression of lung disease in preschool patients with cystic fibrosis. Am J Respir Crit Care Med, 195(9), 1216–1225. [abstract]
  • Kane M, Rayment JH, Jensen R, McDonald R, Stanojevic S, Ratjen F (2017) Correcting for tissue nitrogen excretion in multiple breath washout measurements. PLoS ONE 12(10): e0185553. [abstract]
  • Engberink, E.O., Ratjen, F., Davis, SD., Retsch-Bogart, G., Amin, R., Stanojevic, S. (2017).  Inter-test reproducibility of the lung clearance index measured by multiple breath washout. European Respiratory Journal, Oct 5;50(4) 1700433. [abstract]
  • Rayment, J., Ratjen, F. Constrictive bronchiolitis: A distinct phenotype of CF lung disease? Am J Resp Crit Care Med. Accepted September 21.
  • Ratjen F. How best to capture early CF lung disease? Am J Resp Crit Care Med. Accepted September 17.
  • Stanojevic, S., Mcdonald, A., Waters, V., Macdonald, S., Horton, E., Tullis, E., & Ratjen, F. (2016). Effect of pulmonary exacerbations treated with oral antibiotics on clinical outcomes in cystic fibrosis.Thorax. [abstract]
  • Stanojevic, S., & Ratjen, F. (2016). Physiologic endpoints for clinical studies for cystic fibrosis. Journal of Cystic Fibrosis, 15(4), 416-423.[abstract]
  • Engberink, E.O., Stanojevic, S., & Ratjen, F. (2016). Clinimetric Properties of the Lung Clearance Index in Adults and Children With Cystic Fibrosis. Chest, 150(6), 1412-1413. doi:10.1016/j.chest.2016.08.1478 [abstract]
  • Wettlaufer, J., Klingel, M., Yau, Y., Stanojevic, S., Tullis, E., Ratjen, F., Waters V. (2016) Longitudinal Study of Stenotrophomonas Maltophilia antibody levels and outcomes in cystic fibrosis patients. Journal of Cystic Fibrosis [abstract]
  • Jensen, R., Stanojevic, S., Klingel, M., Pizarro, M. E., Hall, G. L., Ramsey, K., . . . Ratjen, F. (2016). A Systematic Approach to Multiple Breath Nitrogen Washout Test Quality. PLOS ONE PLoS ONE, 11(6).[abstract]
  • Amin, R., Stanojevic, S., Kane, M., Webster, H., Ratjen, F. (2016) A randomized controlled trial to evaluate the lung clearance index as an outcome measure for early phase studies in patients with cystic fibrosis. Respiratory Medicine; (112):59-64. [abstract]
  • Ratjen, F. (2016). Pulmonary artery hypertension: An underrated disease manifestation in cystic fibrosis? The Lancet Respiratory Medicine, 4(8), 596-598. [abstract]
  • Ratjen, F., Waters, V., Klingel, M., Mcdonald, N., Dell, S., Leahy, T. R., . . . Grasemann, H. (2015). Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia. Eur Respir J European Respiratory Journal, 47(3), 829-836. [abstract]
  • Kane, M., Gonska, T., Jensen, R., Avolio, J., Klingel, M., Stanojevic, S., Ratjen, F. (2016) Lung clearance index response in CF patients with class III CFTR mutations. Thorax; 71(5):476-7. [abstract]
  • Waters, V., Stanojevic, S., Klingel, M., Chiang, J., Sonneveld, N., & Kukkar, R. et al. (2015). Prolongation of antibiotic treatment for cystic fibrosis pulmonary exacerbations. Journal Of Cystic Fibrosis, 14(6), 770-776. [abstract]
  • Sonneveld, N., *Stanojevic, S., Amin, R., Aurora, P., Davies, J., Elborn, JS., Horsely, A., Latzin, P., O’Neill, K., Robinson, P., Scrase, E., Selvadurai, H., Subbarao, P., Welsh, L., Yammine, S., Ratjen, F. (2015) Lung Clearance Index in cystic fibrosis subjects treated for pulmonary exacerbations. European Respiratory Journal; 46(4):1055-64. [abstract]

SELECTED CONFERENCE ABSTRACTS

  • Kane, M., Stanojevic, S., Jensen, R., Ratjen, F. 2016 Effect of Tissue Nitrogen Excretion on Multiple Breath Washout Measurements. European Respiratory Society, London, UK.
  • Stanojevic, S., Graham, B., Cooper, B., Thompson, B., Carter, K., Hall, G. 2016 Global lung function initiative: Reference equations for the transfer factor for carbon monoxide (TLCO). European Respiratory Society, London, UK.
  • Ratien F, Alon N,Maykut R, Liu C, Angyalosi TOBI® for eradication of early P. aeruginosa infection in paediatric cystic fibrosis patients: the EARLY study. J Cyst Fibros. 15; Suppl 1. S1.
  • Stanojevic, S., Jensen, R., Webster, H., Kane M., Pizarro, M., Davis, S., Retsch-Bogart, G., Subbarao, P., Ratjen, F. 2016 Tracking pulmonary exacerbations in preschool children using the lung clearance index. European Respiratory Society, London, UK.
  • Klingel, M., Isaac, S., Raaijmakers, L., Jensen, R., Stanojevic, S., Ratjen, F. 2016 O2 concentration based definition of wait times between multiple breath nitrogen washout trials. European Respiratory Society, London, UK.
  • Jensen, R., Pizarro, M. Klingel, M., Stanojevic, S., Webster, H., McDonald, N., Subbarao, P., Davis, S.D., Retsch-Bogart, G.Z., Pittman, J.E., Ratjen, F. 2015 Progression and variability of LCI in preschool children with Cystic Fibrosis. North American Cystic Fibrosis Conference, Phoenix, USA.