Page updated: 16 October 2020


Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: a retrospective registry cohort study. 
Mckone EF, Jackson AD, Fletcher F, Kirwan L. 
J Cyst Fibros. 2020 August 21. DOI:
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. 
Kotnik Pirš A, Krivec U, Trebušak Podkrajšek K.
Acta Chim. Slov. 2020; 67: 666–673. DOI: 
Cystic Fibrosis Related Diabetes in Europe/ Prevalence, Risk Factors and Outcome. 
Olesen HV, et al on behalf of the ECFSPR Steering Group. 
J Cyst Fibros. 2019 Oct. 31. DOI:

Changing Epidemiology of the respiratory bacteriology of patients with cystic fibrosis from the European Cystic Fibrosis Society Patient Registry.
Hatziagorou E, Orenti A, Drevinek P, Kashirskaya N, Mei-Zahav M, de Boeck K, on behalf of the ECFSPR. 
J Cyst Fibros. 2019 Sept. 3. DOI:

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. 
Prinz N et al on behalf of the ECFS Patient Registry and the DPV Registry. 
Pediatr Diabetes. 2019;20(3):255-262. DOI:​​​​​​​
Cystic fibrosis mortality in childhood. Data from European Cystic Fibrosis Society Patient Registry. 
Zolin A , Bossi A, Cirilli N, Kashirskaya N, Padoan R.
Int. J. Environ. Res. Public Health 2018; 15; 2020 Link to Article
Creating longitudinal datasets and cleaning existing data identifiers in a cystic fibrosis registry using a novel Bayesian probabilistic approach from astronomy.
Hurley PD, Oliver S, Mehta A.  
PLoS ONE; 13 (7): e0199815 . Link to Article
Effect of allergic bronchopulmonary aspergillosis on FEV1 in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis. 
Kaditis AG, Miligkos M, Bossi A, Colombo C, Hatziagorou E, Kashirskaya N, de Monestrol I, Thomas M, Mei-Zahav M, Chrousos G, Zolin A. 
Arch Dis Child 2017; 102 (8): 742-747. Link to Article
Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes.
De Boeck K, Zolin A.  
J Cyst Fibros. 2017; 16 (2): 239-245. DOI:10.1016/j.jcf.2016.09.009
Epidemiology of nontuberculous mycobacteria (NTM) amongst individuals with cystic fibrosis (CF).
Viviani L, Harrison MJ,  Zolin A, Haworth CS, Floto RA. 
J Cyst Fibros. 2016; 15 (5): 619–623. Link to Article
International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome. 
Munck A, Alberti C, Colombo C, Kashirskaya N, Ellemunter H, Fotoulaki M, Houwen R, Robberecht E, Boizeau E, Wilschanski M et al, on behalf of the CF/Pancreas ESPGHAN Working Group and DIOS Study Group.
 J Cyst Fibros 2016; 15 (4): 531-539. Link to Article
Future trends in Cystic Fibrosis demography in 34 European countries. 
Burgel P-R,  Bellis G, Olesen HV, Viviani L, Zolin A, Blasi F and Elborn JS. on behalf of the ERS/ECFS Task Force on The Provision of Care for Adults with Cystic Fibrosis in Europe 2015.
Eur Respir J 2015; 46 (1): 133-141. 
European Respiratory Journal 2015 Mar 18. pii: ERJ-01963-2014. Link to Article
The relative frequency of CFTR mutation classes in European patients with cystic fibrosis. 
De Boeck K, Zolin A, Cuppens H, Olesen HV, Viviani L. 
J Cyst Fibros 2014;13(4):403-9. Link to Article
Multi-Country Estimate of Different Manifestations of Aspergillosis in Cystic Fibrosis.
Amstead J, Morris J, Denning DW.
PlosOne 2014; 9 (6): e98502. Link to Article
The European Cystic Fibrosis Society Patient Registry: valuable lessons learned on how to sustain a disease registry.
Laura Viviani, Anna Zolin, Anil Mehta, Hanne Vebert Olesen.
Orphanet J Rare Dis 2014, 9:81.    Link to Article
Factors associated with FEV1 decline in cystic fibrosis: analysis of the data of the ECFS Patient Registry.
Kerem E, Viviani L, Zolin A, Macneill S, Hatziagorou E, Ellemunter H, Drevinek P, Gulmans V, Krivec U, Olesen H; on behalf of the ECFS Patient Registry Steering Group.
Eur Respir J 2014 ;43(1):125-33.  Link to Article
Evidence of diminished FEV1 and FVC in 6-year-olds followed in the European cystic fibrosis patient registry, 2007-2009.
VanDevanter DR, Pasta DJ.
J Cyst Fibros 2013; 12(6): 786-789.  Link to Article
A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect
Boyle MP, De Boeck K.
The Lancet Respiratory Medicine 2013(1): 158-163. Link to Article
Epidemiology of Cystic Fibrosis Lung Disease progression in adolescents in ECFS Book on Healthcare Issues and challenges in Adolescents in CF, December 2012. 
Dutch VanDevanter. Link to Article
Reference percentiles for FEV(1) and BMI in European children and adults with cystic fibrosis.
Boëlle PY, Viviani L, Busson PF, Olesen HV, Ravilly S, Stern M, Assael BM, Barreto C, Drevinek P, Thomas M, Krivec U, Mei-Zahav M, Vibert JF, Clement A, Mehta A, Corvol H on behalf of the French CF Modifier Gene Study Investigators and the European CF Registry Working Group.
Orphanet J Rare Dis 2012; 7: 64.  Link to Article
Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. 
Mehta G, Macek M Jr, Mehta A on behalf of the European Registry Working Group.
J Cyst Fibros 2010; 9 (Suppl 2): S5-S21.  Link to Article
Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis.
McCormick J, Mehta G, Olesen HV, Viviani L, Macek M, Mehta A onbehalf of the European Registry Working Group.
Lancet 2010 (375): 1007-1013.  Link to Article