Forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) of primary ciliary dyskinesia (PCD) patients of the international PCD Cohort with available ultrastructural defect information compared to Global Lung Function Initiative 2012 reference values
| Subjects | FEV1 | p-value# | Subjects | FVC | p-value# | |
| Total | 689 | −1.52 (−1.65 to −1.40) | 683 | −0.79 (−0.92 to −0.66) | ||
| Sex | 0.25 | 0.52 | ||||
| Male | 344 | −1.46 (−1.62 to −1.29) | 341 | −0.76 (−0.93 to −0.58) | ||
| Female | 345 | −1.59 (−1.75 to −1.43) | 342 | −0.83 (−1.00 to −0.66) | ||
| Age group | <0.001 | <0.001 | ||||
| 6–9 years | 127 | −0.77 (−1.04 to −0.49) | 126 | −0.25 (−0.54 to 0.03) | ||
| 10–13 years | 148 | −1.19 (−1.44 to −0.94) | 147 | −0.65 (−0.91 to −0.39) | ||
| 14–17 years | 152 | −1.51 (−1.76 to −1.26) | 151 | −0.84 (−1.10 to −0.58) | ||
| 18–21 years | 69 | −1.53 (−1.89 to −1.17) | 68 | −0.62 (−1.00 to −0.23) | ||
| 22–25 years | 51 | −1.66 (−2.09 to −1.23) | 50 | −0.61 (−1.05 to −0.16) | ||
| 26–29 years | 35 | −2.14 (−2.66 to −1.63) | 34 | −1.17 (−1.71 to −0.63) | ||
| 30–33 years | 23 | −2.47 (−3.10 to −1.84) | 22 | −1.42 (−2.09 to −0.76) | ||
| 34–37 years | 20 | −2.67 (−3.34 to −1.99) | 21 | −1.74 (−2.43 to −1.06) | ||
| 38–41 years | 10 | −2.37 (−3.33 to −1.41) | 10 | −1.00 (−2.00 to −0.01) | ||
| 42–45 years | 10 | −3.24 (−4.20 to −2.28) | 11 | −2.86 (−3.81 to −1.91) | ||
| 46–49 years | 17 | −3.00 (−3.74 to −2.25) | 16 | −2.05 (−2.84 to −1.25) | ||
| ≥50 years | 27 | −2.34 (−2.93 to −1.75) | 27 | −1.29 (−1.90 to −0.68) | ||
| Country | <0.001 | <0.001 | ||||
| Australia | 31 | −1.82 (−2.36 to −1.28) | 31 | −1.18 (−1.74 to −0.62) | ||
| Belgium | 66 | −1.20 (−1.59 to −0.82) | 66 | 0.03 (−0.36 to 0.43) | ||
| Cyprus | 27 | −2.02 (−2.62 to −1.43) | 27 | −1.79 (−2.40 to −1.17) | ||
| Denmark | 23 | −1.36 (−1.99 to −0.72) | 23 | −0.60 (−1.26 to 0.05) | ||
| France | 59 | −1.28 (−1.68 to −0.87) | 59 | −0.45 (−0.87 to −0.03) | ||
| Germany | 83 | −1.21 (−1.54 to −0.87) | 79 | −0.69 (−1.05 to −0.33) | ||
| Israel | 67 | −1.46 (−1.83 to −1.08) | 64 | −0.89 (−1.28 to −0.49) | ||
| Italy | 35 | −1.46 (−1.98 to −0.95) | 35 | −0.65 (−1.19 to −0.12) | ||
| The Netherlands | 30 | −0.45 (−1.01 to 0.11) | 30 | 0.66 (0.08 to 1.24) | ||
| Norway | 13 | −1.43 (−2.27 to −0.60) | 13 | −0.84 (−1.71 to 0.03) | ||
| Poland | 39 | −1.80 (−2.30 to −1.30) | 39 | −0.89 (−1.41 to −0.37) | ||
| Serbia | 2 | −1.77 (−3.97 to 0.44) | 2 | −2.39 (−4.68 to −0.11) | ||
| Switzerland | 24 | −1.45 (−2.10 to −0.81) | 24 | −0.83 (−1.50 to −0.16) | ||
| Turkey | 7 | −2.12 (−3.28 to −0.96) | 7 | −2.13 (−3.33 to −0.92) | ||
| UK | 183 | −1.90 (−2.13 to −1.67) | 184 | −1.19 (−1.43 to −0.96) | ||
| Ultrastructural defects¶ | 0.01 | 0.18 | ||||
| Nondiagnostic | 123 | −1.19 (−1.48 to −0.91) | 120 | −0.74 (−1.04 to −0.44) | ||
| Dynein arm defects | 425 | −1.50 (−1.65 to −1.35) | 422 | −0.73 (−0.88 to −0.57) | ||
| Microtubular defects | 134 | −1.91 (−2.20 to −1.63) | 134 | −1.08 (−1.37 to −0.78) | ||
| Acilia | 7 | −1.44 (−2.64 to −0.24) | 7 | −0.40 (−1.64 to 0.85) |
Data are presented as n or mean z-scores (95% CI) for each group after adjusting for the remaining characteristics, unless otherwise stated. #: likelihood ratio test p-value indicating whether the characteristic explains differences in FEV1 or FVC within the study population; ¶: dynein arm defects: outer and/or inner dynein arm defects; microtubular defects: central pair, tubulus disorganisation, tubular transposition and/or nexin link defect.