Articles

Page updated: 08 February 2024

Kerem E , Orenti A , Adamoli A ,Hatziagorou E , Naehrlich L, Sermet-Gaudelus I; ECFS Patient Registry Steering Group, Cystic fibrosis in Europe: improved lung function & longevity: reasons for cautious optimism, but challenges remain. Eur Respir J. 2024 Feb 1:2301241. Doi: 10.1183/13993003.01241-2023. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/38302155/

Bradbury J.D, Chesshyre E, Orenti A, Jung A, Warris A, European Cystic Fibrosis COVID project group. A multinational report on SARS-COV-2 infection outcomes in people with CF and Aspergillus infection or ABPA. Journal of Cystic Fibrosis; 2023; 03 Nov. Doi: https://doi.org/10.1016/j.jcf.2023.10.017

Orenti A, Pranke I, Faucon C, Varilh J, Hatton A, Golec A, Dehillotte C, Durieu I, Reix P, Burgel PR, Grenet D, Tasset C, Gachelin E, Perisson C, Lepissier A, Dreano E, Tondelier D, Chevalier B, Weiss L, Kiefer S, Sermet-Gaudelus I. Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children. Journal of Cystic Fibrosis; 2023; 06 Jul. Doi: https://doi.org/10.1016/j.jcf.2023.06.005.

www.sciencedirect.com/science/article/abs/pii/S1569199323008196?dgcid=coauthor

Gambazza S, Orenti A, Pizzamiglio G, Zolin A, Colombo C, Laquintana D, Ambrogi F. Association of Oxygen Therapy with the Natural Disease Progression of Cystic Fibrosis: A Multi-State Model of the European Cystic Fibrosis Society Patient Registry. Ther Clin Risk Manag. 2023 Mar 13;19:255-267. doi: 10.2147/TCRM.S391476. eCollection 2023.
www.dovepress.com/association-of-oxygen-therapy-with-the-natural-disease-progression-of--peer-reviewed-fulltext-article-TCRM

Hatziagorou E, Fieuws S, Orenti A, et al. Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry. ERJ Open Research 2023 9: 00449-2022; DOI: 10.1183/23120541.00449-2022.
https://openres.ersjournals.com/content/9/3/00449-2022

Kerem E, Orenti A, Zolin A, Annicchiarico L, Drevinek P. Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis. Journal of Cystic Fibrosis; 2022.
www.sciencedirect.com/science/article/pii/S1569199322014102?via%3Dihub

Duursma SS, Verkade HJ, Bodewes FAJA. Cirrhosis and portal hypertension in cystic fibrosis in compound heterozygous people with harboring one F508del CFTR gene mutation. Journal of Gastroenterology; 2022, June 09. Doi: https://doi.org/10.1053/j.gastro.2022.06.019.

Orenti A, Mei-Zahav M, Boracchi P, Lindbald A, Shteinberg M. Prevalence, trends and outcomes of long-term inhaled antibiotic treatment in people with cystic fibrosis without chronic Pseudomonas aeruginosa infection – A European cystic fibrosis patient registry data analysis. Journal of Cystic Fibrosis 2022 ; 28 Aug. Doi: https://doi.org/10.1016/j.jcf.2022.08.010.
www.sciencedirect.com/science/article/pii/S1569199322006476?dgcid=author

Mei Zahav M, Orenti A, Jung A, Hatziagorou E, Olesen HV, Kerem E. Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry. Journal of Cystic Fibrosis 2022; Aug.
www.sciencedirect.com/science/article/pii/S1569199322006373#aep-article-footnote-id1

Mondejar P, Zolin A, Garcia-Marcos PW, et al. Geographic distribution and phenotype of European people with Cystic Fibrosis carrying A1006E mutation. Respiratory Medicine Journal, 2022 Feb;192:106736. doi: 10.1016/j.rmed.2022.106736.
www.resmedjournal.com/article/S0954-6111(22)00001-4/fulltext

Jung A, Orenti A, …., Downey DG, Naehrlich L. Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe. ERJ Open Research 2021; DOI: 10.1183/23120541.00411-2021. https://doi.org/10.1183/23120541.00411-2021

Naehrlich L, Orenti A, Dunlevy F, …., van Koningsbruggen-Rietschel S, Jung A. Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020. Journal of Cystic Fibrosis 2021; April 17. DOI: https://www.cysticfibrosisjournal.com/article/S1569-1993(21)00099-0/fulltext#seccesectitle0022

McKone EF, Ariti C, Zolin A, on behalf of the ECFSPR, Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study. https://erj.ersjournals.com/content/58/3/2002288.

Mckone EF, Jackson AD, Fletcher F, Kirwan L. Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: a retrospective registry cohort study. Journal of Cystic Fibrosis 2020. https://doi.org/10.1016/j.jcf.2020.08.004.

Kotnik Pirš A, Krivec U, Trebušak Podkrajšek K. The c.3140-26A>G Variant of the CFTR Gene in homozygous state causes mild cystic fibrosis Overview of longitudinal clinical data of the patient managed in our centre and review of the literature. Acta Chimica Slovenica 2020;67:666-673. https://dx.doi.org/10.17344/acsi.2019.5677.

Olesen HV, Drevinek P, Gulmans VA, Thomas M, Zolin A, on behalf of the ECFSPR Steering Group. Cystic Fibrosis Related Diabetes in Europe/ Prevalence, Risk Factors and Outcome. Journal of Cystic Fibrosis 2019;19(2):321-327. DOI: https://doi.org/10.1016/j.jcf.2019.10.009.

Hatziagorou E, Orenti A, Drevinek P, Kashirskaya N, Mei-Zahav M, de Boeck K, on behalf of the ECFSPR. Changing Epidemiology of the respiratory bacteriology of patients with cystic fibrosis from the European Cystic Fibrosis Society Patient Registry. Journal of Cystic Fibrosis 2019. https://doi.org/10.1016/j.jcf.2019.08.006

Prinz N, Zolin A, Konrad K, Nährlich L, Laubner K, Olesen HV et al. on behalf of the ECFS Patient Registry and the DPV Registry. Characteristics of Cystic Fibrosis-related diabetes: Data from two different sources, the European Cystic Fibrosis Society Patient registry and German/Austrian diabetes prospective follow-up registry. Pediatric Diabetes 2019;20(3):255-262. https://doi.org/10.1111/pedi.12831

Zolin A , Bossi A, Cirilli N, Kashirskaya N, Padoan R. Cystic fibrosis mortality in childhood. Data from European Cystic Fibrosis Society Patient Registry. International Journal of Environmental Research and Public Health 2018;15:2020. DOI:10.3390/ijerph15092020

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Hurley PD, Oliver S, Mehta A. Creating longitudinal datasets and cleaning existing data identifiers in a cystic fibrosis registry using a novel Bayesian probabilistic approach from astronomy. PLOS ONE 2019;13(7): e0199815. https://doi.org/10.1371/journal.pone.0199815.

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Kaditis AG, Miligkos M, Bossi A, Colombo C, Hatziagorou E, Kashirskaya N, et al. Effect of allergic bronchopulmonary aspergillosis on FEV1 in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis. Archives of Disease in Childhood 2017;102(8):742-747. Link to Article

De Boeck K, Zolin A. Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes. Journal of Cystic Fibrosis 2017;16(2):239-245. DOI:10.1016/j.jcf.2016.09.009

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Viviani L, Harrison MJ, Zolin A, Haworth CS, Floto RA. Epidemiology of nontuberculous mycobacteria (NTM) amongst individuals with cystic fibrosis (CF). Journal of Cystic Fibrosis 2016;15(5):619–623. Link to Article

Munck A, Alberti C, Colombo C, Kashirskaya N, Ellemunter H, Fotoulaki M et al. on behalf of the CF/Pancreas ESPGHAN Working Group and DIOS Study Group. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome. Journal of Cystic Fibrosis 2016;15(4):531-539. Link to Article

Burgel P-R, Bellis G, Olesen HV, Viviani L, Zolin A, Blasi F et al. on behalf of the ERS/ECFS Task Force on The Provision of Care for Adults with Cystic Fibrosis in Europe 2015. Future trends in Cystic Fibrosis demography in 34 European countries.
European Respiratory Journal 2015;46(1):133-141. Link to Article

De Boeck K, Zolin A, Cuppens H, Olesen HV, Viviani L. The relative frequency of CFTR mutation classes in European patients with cystic fibrosis.
Journal of Cystic Fibrosis 2014;13(4):403-9. Link to Article

Amstead J, Morris J, Denning DW. Multi-Country Estimate of Different Manifestations of Aspergillosis in Cystic Fibrosis. PLOS ONE 2014;9(6):e98502. Link to Article

Viviani L, Zolin A, Mehta A, Vebert Olesen H.The European Cystic Fibrosis Society Patient Registry: valuable lessons learned on how to sustain a disease registry. Orphanet Journal of Rare Diseases 2014,9:81. Link to Article

Kerem E, Viviani L, Zolin A, Macneill S, Hatziagorou E, Ellemunter H, et al. on behalf of the ECFS Patient Registry Steering Group. Factors associated with FEV1 decline in cystic fibrosis: analysis of the data of the ECFS Patient Registry. European Respiratory Journal 2014;43(1):125-33. Link to Article

VanDevanter DR, Pasta DJ. Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European cystic fibrosis patient registry, 2007-2009. Journal of Cystic Fibrosis 2013;12(6):786-789. Link to Article

Boyle MP, De Boeck K. A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect. The Lancet Respiratory Medicine 2013(1):158-163. Link to Article

VanDevanter D. Epidemiology of Cystic Fibrosis Lung Disease progression in adolescents. In: Castellani C, Elborn, S Heijerman H, editors. Healthcare Issues and challenges in Adolescents in CF Denmark, ECFS 2012. Link to Article

Boëlle PY, Viviani L, Busson PF, Olesen HV, Ravilly S, Stern M, et al. on behalf of the French CF Modifier Gene Study Investigators and the European CF Registry Working Group. Reference percentiles for FEV(1) and BMI in European children and adults with cystic fibrosis. Orphanet Journal of Rare Diseases 2012;7:64. Link to Article

Mehta G, Macek Jr M, Mehta A, on behalf of the European Registry Working Group. Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. Journal of Cystic Fibrossis 2010;9(Suppl 2):S5-S21. Link to Article

McCormick J, Mehta G, Olesen HV, Viviani L, Macek M, Mehta A, on behalf of the European Registry Working Group. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet 2010(375):1007-1013. Link to Article