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Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

P.M. Gustafsson, P. Aurora, A. Lindblad
European Respiratory Journal 2003 22: 972-979; DOI: 10.1183/09031936.03.00049502
P.M. Gustafsson
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P. Aurora
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A. Lindblad
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  • Fig. 1.—
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    Fig. 1.—

    a) Forced expiratory volume in one second (FEV1) % predicted versus age in 43 patients with cystic fibrosis (CF) (•) and 28 healthy controls (○). b) Maximum expiratory flow at 25% of forced vital capacity (MEF25) % pred versus age in 34 patients with CF (•) and 26 healthy controls (○). c) Mixing ratio from multiple-breath washout (MBW) versus age in 43 patients with CF (•) and 28 healthy controls (○). d) Lung clearance index (LCI) from MBW versus age in 43 patients with CF (•) and 28 healthy controls (○). The dashed horizontal lines in figures c and d denote the upper limits of normality for mixing ratio (1.35) and LCI (7,17), respectively.

  • Fig. 2.—
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    Fig. 2.—

    a) Mixing ratio (MR) versus forced expiratory volume in one second (FEV1) % predicted in 43 patients with cystic fibrosis (CF) (•) and 28 healthy controls (○). The dashed horizontal line denotes the upper normal limit for MR (1.35). b) MR versus maximum expiratory flow at 25% of forced vital capacity (MEF25) % pred in 34 patients with CF (•) and 26 healthy controls (○) aged ≥7 yrs. The dashed horizontal line denotes the upper normal limit for MR (1.35).

Tables

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  • Table 1

    Clinical data of the 43 cystic fibrosis (CF) patients and the 28 healthy controls

    CF patientsControls
    Patients4328
    Males/females23/2014/14
    Age yrs11.5 (3.0–18.2)11.4 (4.5–18.7)
    Weight kg35.5 (16.5–92.0)38.3 (20.5–85.5)
    Height cm146 (101–181)150 (113–189)
    Genotype
     ΔF508/ΔF50824
     ΔF508/other17
     Other/other3
    Pancreatic function
     PI36
     PS7
    Chronically colonised
     P. aeruginosa10
     S. maltophilia2
     B. cepacia2
    • Data are presented as n or median (range)

    • PI: pancreatic insufficiency

    • PS: pancreatic sufficiency

    • P. aeruginosa: Pseudomonas aeruginosa

    • S.maltophilia: Stenotrophomonas maltophilia

    • B. cepacia: Burkholderia cepacia

  • Table 2

    Comparison of lung function tests results from children with cystic fibrosis (CF) and the healthy controls

    CFControlsCF‐controls
    Patients n4328
    FEV1 % pred89.1 (19.8)96.9 (10.3)−15.9–0.3#
    MEF50 % pred¶92.3 (41.2)107.0 (30.2)−33.9–4.6
    MEF25 % pred¶64.5 (33.5)86.5 (28.2)−38.3–−5.7**
    RV/TLC+26.5 (10.6)22.8 (4.3)−0.7–8.1
    MR1.61 (0.49)1.19 (0.08)0.23–0.61***
    LCI8.33 (2.48)6.33 (0.43)1.06–2.95***
    • Data are presented as mean (SD) and 95% confidence interval for the difference between the groups unless otherwise stated

    • FEV1: forced expiratory volume in one second

    • % pred: % predicted

    • MEF50: maximum expiratory flow at 50% of forced vital capacity

    • MEF25: maximum expiratory flow at 25% of forced vital capacity

    • RV: residual volume

    • TLC: total lung capacity

    • MR: mixing ratio

    • LCI: lung clearance index

    • #: p=0.06

    • ¶: MEF50 and MEF25 results were available for analyses in 34 CF patients and 26 controls

    • +: RV/TLC results were available for analyses in 35 CF patients and 27 controls

    • **: p<0.01

    • ***: p<0.001

  • Table 3

    Comparison of age and lung function tests results in the cystic fibrosis (CF) patients with or without chronic bacterial colonisation

    ColonisedYes-no
    YesNo
    Patients n1429
    Age yrs13.7 (2.8)9.3 (3.9)2.0–6.7***
    FEV1 % pred74.7 (22.8)96.1 (13.9)−32.3–−10.1***
    MEF50 % pred#67.1 (36.0)110.0 (36.0)−68.3–−17.6**
    MEF25 % pred#43.7 (30.9)79.1 (27.5)−55.9–−14.9**
    RV/TLC %¶33.0 (14.4)22.7 (5.4)3.4–17.1**
    MR1.91 (0.69)1.47 (0.28)0.14–0.74**
    LCI9.85 (3.49)7.60 (1.37)0.76–3.74**
    • Data are presented as mean (sd) and 95% confidence interval for thedifference between the groups unless otherwise stated

    • FEV1: forced expiratory volume in one second

    • % pred: % predicted

    • MEF50: maximum expiratory flow at 50% of forced vital capacity

    • MEF25: maximum expiratory flow at 25% of forced vital capacity

    • RV: residual volume

    • TLC: total lung capacity

    • MR: mixing ratio

    • LCI: lung clearance index

    • #: MEF50 and MEF25 results were available for analyses in 20 noncolonised patients

    • ¶: RV/TLC results were available for analyses in 22 noncolonised patients and 13 colonised

    • **: p<0.01

    • ***: p<0.001

  • Table 4

    Comparison of age and lung function in the children with cystic fibrosis (CF) who did not show chronic colonisation and the healthy control children

    CF (noncolonised)ControlsCF‐controls
    Patients n2928
    Age yrs9.3 (3.9)11.5 (3.2)−4.0–−0.2*
    FEV1 % pred96.1 (13.9)96.9 (10.3)−7.3–5.7
    MEF50 % pred#110.0 (36.0)107.0 (30.2)16.5–22.6
    MEF25 % pred#79.1 (27.5)86.5 (28.2)−24.1–9.3
    RV/TLC¶22.7 (5.4)22.8 (4.3)−2.9–2.7
    MR1.47 (0.28)1.19 (0.08)0.17–0.39***
    LCI7.6 (1.37)6.33 (0.43)0.73–1.81***
    • Data are presented as mean (sd) and 95% confidence interval for the difference between the groups unless otherwise stated

    • FEV1: forced expiratory volume in one second

    • % pred: % predicted

    • MEF50: maximum expiratory flow at 50% of forced vital capacity

    • MEF25: maximum expiratory flow at 25% of forced vital capacity

    • RV: residual volume

    • TLC: total lung capacity

    • MR: mixing ratio

    • LCI: lung clearance index

    • #: MEF50 and MEF25 results were available for analyses in 20 CF patients and 26 control subjects

    • ¶: RV/TLC results were available for analyses in 22 CF patients and 27 control subjects

    • *: p<0.05

    • ***: p<0.001

  • Table 5

    Number of subjects with abnormal (+) or normal (−) mixing ratios (MR) and lung clearance index (LCI) findings in relation to spirometry and plethysmography results

    CFControls
    MRLCIMRLCI
    +−+−+−+−
    FEV1
     +91910000
     −22111815028028
    MEF50
     +1011010202
     −1211914024024
    MEF25
     +1221220404
     −1010713022022
    RV/TLC
     +1101100303
     −1212915024024
    LCI
     +27000
     −412028
    • FEV1: forced expiratory volume in one second

    • MEF50: maximum expiratory flow at 50% of forced vital capacity

    • MEF25: maximum expiratory flow at 25% of forced vital capacity

    • RV: residual volume

    • TLC: total lung capacity

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Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis
P.M. Gustafsson, P. Aurora, A. Lindblad
European Respiratory Journal Dec 2003, 22 (6) 972-979; DOI: 10.1183/09031936.03.00049502

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Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis
P.M. Gustafsson, P. Aurora, A. Lindblad
European Respiratory Journal Dec 2003, 22 (6) 972-979; DOI: 10.1183/09031936.03.00049502
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