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Table 1 Demographic description

From: Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis

Country

CF patients

Estimated Coverage*

Number of measurements

Sex-ratio (F)

p.Phe508del homozygosity

Age (yrs)

Adults**

FEV1 percent predicted (%)***

BMI z-score

 

(n)

(%)

(n)

(%)

(%)

Mean [range]

(%)

Mean ± SD

Mean ± SD

ECFS Patient Registry (2004-2007)

France

5147

89%

14674

47.9

48%

16.7 [0 - 78.4]

34%

74.4 ± 31.8

-0.63 ± 1.07

Germany

5039

74%

19272

48.1

64%

18.1 [0 - 68.2]

39%

75.0 ± 31.6

-0.54 ± 1.09

High coverage countries

3671

93%

9213

47.4

53%

18.6 [0 - 77.4]

41%

80.1 ± 28.4

-0.35 ± 1.08

Low coverage countries

1347

18%

3774

48.3

44%

18.3 [0 - 69.0]

40%

81.32 ± 28.0

-0.21 ± 1.08

MUCONAT (2008-2010)

France

1577

39%

3549

47.9

51.8%

20.3 [6.0 - 40.0]

47%

72.9 ± 31.2

-0.53 ± 1.00

Overall

16781

-

50482

47.9

47.8

18.0 [0 - 78.4]

39%

75.7 ± 31.1

-0.52 ± 1.07

  1. * Estimated Coverage derived from prevalence estimates reported by Farell et al., Efrati et al. and the French CF national registry.
  2. ** Age > 20 years.
  3. *** FEV1 percent predicted calculated with Knudson equation.