Table 1– Clinical and demographic characteristics of the study population
Patients n14 732
Participating countries
 Austria90 (0.6)
 Belgium#757 (5.1)
 Bulgaria23 (0.2)
 Czech Republic#313 (2.1)
 Denmark#320 (2.2)
 France#3422 (23.2)
 Germany#4133 (28.0)
 Greece56 (0.4)
 Israel390 (2.6)
 Italy632 (4.3)
 The Netherlands#871 (5.9)
 Portugal78 (0.5)
 Slovenia37 (0.2)
 UK#3610 (24.5)
Male7772 (52.8)
Age at FEV1 measurement years
 Median (range)19.0 (6–76.7)
 ≥18 years8001 (54.3)
Age at diagnosis months¶,+
 Median (range)7.2 (0–73.3)
 ≥18 years668 (6.1)
Genotype§
 Patients with DNA analysis13613 (92.4)
 Severe/severe8624 (63.3)
 Severe/mild731 (5.4)
 Mild/mild27 (0.2)
 At least one allele unclassified4231 (31.1)
 Unknown alleles2344 (8.6)
BMI ≤ -2 SDSƒ
 6–9 years111 (5.1)
 10–14 years191 (6.7)
 15–19 years316 (11.4)
 20–24 years340 (14.9)
 25–29 years225 (13.3)
 30–34 years104 (9.3)
 35–39 years65 (8.0)
 40–44 years21 (4.2)
 ≥45 years18 (4.3)
Pancreatic status##
 Pancreatic insufficient11567 (86.7)
Chronic Pseudomonas aeruginosa infection¶¶
 Infected patients3631 (37.2)
CFRD++
 0–9 years14 (0.7)
 10–19 years409 (7.6)
 20–29 years697 (18.2)
 ≥30 years619 (22.7)
FEV1 % predicted
 6–9 years91.2 (90.4–91.9)
 10–14 years86.5 (85.7–87.2)
 15–19 years72.3 (71.4–73.1)
 20–24 years63.0 (62.0–64.0)
 25–29 years58.5 (57.4–59.7)
 30–34 years56.4 (55.0–57.8)
 35–39 years56.9 (55.3–58.6)
 40–44 years55.5 (53.4–57.6)
 ≥45 years57.7 (55.3–60.0)
  • Data are presented as n (%) or mean (95% CI), unless otherwise stated. FEV1: forced expiratory volume in 1 s; BMI: body mass index; SDS: standard deviation score; CFRD: cystic fibrosis-related diabetes. #: data sent by a national registry; : data available for 10 994 patients; +: for prenatal diagnoses, age at diagnosis was set to 0 months; §: alleles classified according to McKone et al. [11]: severe (alleles that belong to either class I, II or III), mild (alleles that belong either to class IV or V) and unclassified (alleles that could not be classified into any of the previous classes); ƒ: data available for 14 625 patients; ##: data available for 13 343 patients; ¶¶: data available for 9748 patients; ++: data available for 14 013 patients.