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Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry

Eitan Kerem, Laura Viviani, Anna Zolin, Stephanie MacNeill, Elpis Hatziagorou, Helmut Ellemunter, Pavel Drevinek, Vincent Gulmans, Uros Krivec, Hanne Olesen on behalf of the ECFS Patient Registry Steering Group
European Respiratory Journal 2014 43: 125-133; DOI: 10.1183/09031936.00166412
Eitan Kerem
1Dept of Pediatrics and CF Center, Hadassah Hebrew University Hospital, Jerusalem, Israel
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  • For correspondence: kerem@hadassah.org.il
Laura Viviani
2Dept of Clinical Sciences and Community Health, University of Milan, Milan, Italy
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Anna Zolin
2Dept of Clinical Sciences and Community Health, University of Milan, Milan, Italy
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Stephanie MacNeill
3Dept of Occupational and Environmental Medicine, National Heart and Lung Institute, Imperial College London, London, UK
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Elpis Hatziagorou
43rd Dept of Paediatrics, CF Centre of Aristotelian University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece
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Helmut Ellemunter
5Dept of Pediatrics, Univ.-Klinik für Pädiatrie III, Cystic Fibrosis Center, Medical University Innsbruck, Austria
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Pavel Drevinek
6Dept of Paediatrics, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague
7Dept of Medical Microbiology, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic
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Vincent Gulmans
8Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands
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Uros Krivec
9Unit for Pulmonary Diseases, University Children's Hospital, Ljubljana, Slovenia
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Hanne Olesen
10Dept of Paediatrics, Aarhus University Hospital, Aarhus, Denmark
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  • Figure 1–
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    Figure 1–

    Prevalence of chronic Pseudomonas aeruginosa infection and total number of patients by age.

  • Figure 2–
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    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.

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    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.

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  • 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.

  • 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 countryAdjustment for age, country and all other factors
    Differencep-valueDifferencep-value
    Genotype#<0.00010.0005
     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/mild3.8 (-5.0–12.6)4.5 (-6.8–15.9)
     Severe/mild – unclassified5.2 (3.4–7.0)1.0 (-1.1–3.1)
     Mild/mild – unclassified1.4 (-7.2–10.0)-3.5 (-14.8–7.7)
    Sex
     Male – female1.1 (0.3–1.8)0.00512.7 (1.8–3.6)<0.0001
    Pancreatic status
     Sufficient – insufficient12.9 (11.8–14.2)<0.00016.7 (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 – poor23.1 (21.8–24.3)21.6 (20.0–23.2)
     Above normal – poor38.5 (32.8–44.1)29.1 (21.9–36.2)
     Above normal – normal15.4 (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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Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry
Eitan Kerem, Laura Viviani, Anna Zolin, Stephanie MacNeill, Elpis Hatziagorou, Helmut Ellemunter, Pavel Drevinek, Vincent Gulmans, Uros Krivec, Hanne Olesen
European Respiratory Journal Jan 2014, 43 (1) 125-133; DOI: 10.1183/09031936.00166412

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Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry
Eitan Kerem, Laura Viviani, Anna Zolin, Stephanie MacNeill, Elpis Hatziagorou, Helmut Ellemunter, Pavel Drevinek, Vincent Gulmans, Uros Krivec, Hanne Olesen
European Respiratory Journal Jan 2014, 43 (1) 125-133; DOI: 10.1183/09031936.00166412
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