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 |