Page updated: 15 May 2024


Kim C, Higgins M, Liu L, Volkova N, Zolin A, Naehrlich L, on behalf of the ECFS Patient Registry Study Group.  Effectiveness if lumacaftor/ivacaftor initiation in children with CF aged 2 through 5 years on disease progression: interim results from an ongoing registry-based study. J Cyst Fibros, 2024;1569-1993. doi: 10.1016/j.jcf.2024.02.004
Kerem E, Orenti A, Adamoli A, Hatziagorou E, Naehrlich L, Sermet-Gaudelus I, on behalf of the  ECFS Patient Registry Steering Group, CCystic fibrosis in Europe: improved lung function & longevity: reasons for cautious optimism, but challenges remain. Eur Respir J, 2004;63(3): 2301241. doi: 10.1183/13993003.01241-2023.
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. J Cyst Fibros, 2023; S1569-1993(23)01666-1. doi: 10.1016/j.jcf.2023.10.017.
Orenti A, Pranke I, Faucon C, et al. on behalf of the ECFS Patient Registry Steering Group. Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children. J Cyst Fibros, 2023;22(6):1070-1079. doi: 10.1016/j.jcf.2023.06.005
Gambazza S, Orenti A, Pizzamiglio G, et al. on behalf of the ECFS Patient Registry. 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;19:255-267. doi: 10.2147/TCRM.S391476
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. Eur Respir J Open Research, 2023;9(3): 00449-2022. doi: 10.1183/23120541.00449-2022
Kerem E, Orenti A, Zolin A, Annicchiarico L, Drevinek P, on behalf of the ECFS Patient Registry. Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis. J Cyst Fibros, 2022;22(2):334-343. doi: 10.1016/j.jcf.2022.11.001
Duursma SS, Verkade HJ, Bodewes FAJA. Cirrhosis and portal hypertension in cystic fibrosis in compound heterozygous people with harboring one F508del CFTR gene mutation. J Gastroenterol, 2022;163(4):1098-1100. doi: 10.1053/j.gastro.2022.06.019
Orenti A, Mei-Zahav M, Boracchi P, Lindbald A, Shteinberg M., on behalf of the ECFS Patient Registry Scientific Committee. 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. J Cyst Fibros, 2022;22(1):103-111. doi: 10.1016/j.jcf.2022.08.010.

Mei Zahav M, Orenti A, Jung A, Hatziagorou E, Olesen HV, Kerem E., on behalf of the ECFS Patient Registry.  Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry. J Cyst Fibros, 2022;22(2):234-247. doi: 10.1016/j.jcf.2022.07.015

Mondejar P, Zolin A, Garcia-Marcos PW, et al.  Geographic distribution and phenotype of European people with Cystic Fibrosis carrying A1006E mutation. Respiratory Medicine 2022;192:106736. doi: 10.1016/j.rmed.2022.106736
Jung A, Orenti A, Dunlevy F, et al. on behalf of the ECFS Patient Registry. Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe.  Eur Respir J Open Research, 2021;7(4):00411-2021. doi: 10.1183/23120541.00411-2021
Naehrlich L, Orenti A, Dunlevy F, et al. on behalf of the ECFS COVID project group. Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020.  J Cyst Fibros, 2021;20(4):566-577. doi: 10.1016/j.jcf.2021.03.017
McKone E.F, Ariti C, Zolin A, et al. on behalf of the ECFS Patient Registry. Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study. Eur Respir J, 2021;58(3):2002288. doi: 10.1183/13993003.02288-2020
Mckone E.F, Jackson A.D, Fletcher F, Kirwan L. Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: a retrospective registry cohort study.  J Cyst Fibros, 2020;20(3):552-554. doi: 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. cta Chim Slov, 2020;67(2):666-673. doi: 10.17344/acsi.2019.5677
Olesen H.V, Drevinek P, Gulmans V.A, et al. on behalf of the ECFS Patient Registry Steering Group. Cystic Fibrosis Related Diabetes in Europe/ Prevalence, Risk Factors and Outcome. J Cyst Fibros, 2019;19(2):321-327. doi: 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.  J Cyst Fibros, 2019;19(3):376-383. doi: 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. Pediatr Diabetes, 2019;20(3)255-262. doi: 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. Nurs Rep, 2018;15(9):2020. doi: 10.3390/ijerph15092020

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):0199815. doi: 10.1371/journal.pone.0199815

Kaditis A.G, Miligkos M, Bossi A, et al. Effect of allergic bronchopulmonary aspergillosis on FEV1 in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis. Arch Dis Child Educ Pract Ed, 2017;102(8):742-747. doi: 10.1136/archdischild-2016-311132

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

Link to Article

Viviani L, Harrison M.J, Zolin A, Haworth C.S, Floto R.A. Epidemiology of nontuberculous mycobacteria (NTM) amongst individuals with cystic fibrosis (CF). J Cyst Fibros, 2016;15(5):619-623. doi: 10.1016/j.jcf.2016.03.002   Link to Article
Munck A, Alberti C, Colombo C, 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. J Cyst Fibros, 2016;15(4):531-539. doi: 10.1016/j.jcf.2016.02.002  Link to Article
Burgel P-R,  Bellis G, Olesen H.V, 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. Eur Respir J, 2015;63(4). doi: 10.1183/09031936.00196314. 
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. 
J Cyst Fibros, 2014;13(4):403-409. doi: 10.1016/j.jcf.2013.12.003
Amstead J, Morris J, Denning DW. Multi-Country Estimate of Different Manifestations of Aspergillosis in Cystic Fibrosis.  PloS One, 2014;9(6):98502. doi: 10.1371/journal.pone.0098502 
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 J Rare Dis, 2014;9:81. doi: 10.1186/1750-1172-9-81
Kerem E, Viviani L, Zolin A, 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. Eur Respir J, 2014;43(1):125-133. doi: 10.1183/09031936.00166412.
VanDevanter D.R, Pasta D.J. Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European cystic fibrosis patient registry, 2007-2009.  J Cyst Fibros, 2013;12(6):786-789. doi: 10.1016/j.jcf.2013.04.005
Boyle MP, De Boeck K. A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect.  Lancet Respir Med, 2013;1(2):158-163. doi: 10.1016/S2213-2600(12)70057-7 
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 P.Y, Viviani L, Busson P.F, 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 J Rare Dis, 2012;7(64). 
doi: 10.1186/1750-1172-7-64 
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. J Cyst Fibros, 2010;9(Suppl 2):S5-S21. doi: 10.1016/j.jcf.2010.08.002.
McCormick J, Mehta G, Olesen H.V, 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(9719):1007-1013.  doi: 10.1016/S0140-6736(09)62161-9