Figures
- Figure 1–
Prevalence of chronic Pseudomonas aeruginosa infection and total number of patients by age.
- Figure 2–
Forced expiratory volume in 1 s (FEV1) and number of patients by age. Data are presented as mean (95% CI) unless otherwise stated. % pred: % predicted.
- Figure 3–
Multiple logistic model: effect of age, body mass index (BMI), chronic Pseudomonas aeruginosa infection, pancreatic status and cystic fibrosis-related diabetes (CFRD) on the odds of having forced expiratory volume in 1 s (FEV1) <40% predicted. Estimates are controlled for country. Model estimated on 8650 patients from Austria, Belgium, Bulgaria, France, Greece, Italy, the Netherlands, Slovenia and UK. % pred: % predicted; SDS: standard deviation scores. #: chosen as reference as most numerous group.
Tables
- Table 1– Clinical and demographic characteristics of the study population
Patients n 14 732 Participating countries Austria 90 (0.6) Belgium# 757 (5.1) Bulgaria 23 (0.2) Czech Republic# 313 (2.1) Denmark# 320 (2.2) France# 3422 (23.2) Germany# 4133 (28.0) Greece 56 (0.4) Israel 390 (2.6) Italy 632 (4.3) The Netherlands# 871 (5.9) Portugal 78 (0.5) Slovenia 37 (0.2) UK# 3610 (24.5) Male 7772 (52.8) Age at FEV1 measurement years Median (range) 19.0 (6–76.7) ≥18 years 8001 (54.3) Age at diagnosis months¶,+ Median (range) 7.2 (0–73.3) ≥18 years 668 (6.1) Genotype§ Patients with DNA analysis 13613 (92.4) Severe/severe 8624 (63.3) Severe/mild 731 (5.4) Mild/mild 27 (0.2) At least one allele unclassified 4231 (31.1) Unknown alleles 2344 (8.6) BMI ≤ -2 SDSƒ 6–9 years 111 (5.1) 10–14 years 191 (6.7) 15–19 years 316 (11.4) 20–24 years 340 (14.9) 25–29 years 225 (13.3) 30–34 years 104 (9.3) 35–39 years 65 (8.0) 40–44 years 21 (4.2) ≥45 years 18 (4.3) Pancreatic status## Pancreatic insufficient 11567 (86.7) Chronic Pseudomonas aeruginosa infection¶¶ Infected patients 3631 (37.2) CFRD++ 0–9 years 14 (0.7) 10–19 years 409 (7.6) 20–29 years 697 (18.2) ≥30 years 619 (22.7) FEV1 % predicted 6–9 years 91.2 (90.4–91.9) 10–14 years 86.5 (85.7–87.2) 15–19 years 72.3 (71.4–73.1) 20–24 years 63.0 (62.0–64.0) 25–29 years 58.5 (57.4–59.7) 30–34 years 56.4 (55.0–57.8) 35–39 years 56.9 (55.3–58.6) 40–44 years 55.5 (53.4–57.6) ≥45 years 57.7 (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.
- Table 2– Differences in the estimated marginal means of forced expiratory volume in 1 s (FEV1) % predicted computed from the linear regression models; representing the estimated effect of each factor on FEV1
Adjustment for age and country Adjustment for age, country and all other factors Difference p-value Difference p-value Genotype# <0.0001 0.0005 Severe/severe – severe/mild -10.9 (-12.6− -9.1) -3.8 (-5.9− -1.6) Severe/severe – mild/mild -7.1 (-15.7–1.6) 0.7 (-10.5–12.0) Severe/severe – unclassified -5.7 (-6.5− -4.8) -2.8 (-3.9− -1.7) Severe/mild – mild/mild 3.8 (-5.0–12.6) 4.5 (-6.8–15.9) Severe/mild – unclassified 5.2 (3.4–7.0) 1.0 (-1.1–3.1) Mild/mild – unclassified 1.4 (-7.2–10.0) -3.5 (-14.8–7.7) Sex Male – female 1.1 (0.3–1.8) 0.0051 2.7 (1.8–3.6) <0.0001 Pancreatic status Sufficient – insufficient 12.9 (11.8–14.2) <0.0001 6.7 (5.2–8.2) <0.0001 CFRD Yes – no -11.6 (-12.8− -10.4) <0.0001 -8.0 (-9.5− -6.6) <0.0001 Pseudomonas aeruginosa infection Yes – no -15.7 (-16.6− -14.7) <0.0001 -13.0 (-14.0− -12.0) <0.0001 BMI¶ <0.0001 <0.0001 Normal – poor 23.1 (21.8–24.3) 21.6 (20.0–23.2) Above normal – poor 38.5 (32.8–44.1) 29.1 (21.9–36.2) Above normal – normal 15.4 (9.9–21.0) 7.4 (0.5–14.4) Data are presented as mean (95% CI), unless otherwise stated. CFRD: cystic fibrosis-related diabetes; BMI: body mass index. #: 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); ¶: normal: -2–2 standard deviation scores (SDS); poor: ≤ -2 SDS; above normal: >2 SDS.
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