Total | Western Europe | British Isles | Southern Europe | Northern Europe | Eastern Europe | |||||||||||||
n | % | 95% CI | n | % | 95% CI | n | % | 95% CI | n | % | 95% CI | n | % | 95% CI | n | % | 95% CI | |
Screening tests | ||||||||||||||||||
Nasal nitric oxide | 89 | 45.9 | 38.8–53.0 | 31 | 50.8 | 37.9–63.7 | 11 | 33.3 | 16.4–50.3 | 23 | 48.9 | 34.1–63.78 | 15 | 68.2 | 47.0–89.3 | 9 | 29.0 | 12.1–46.0 |
Saccharine/variant of saccharine | 70 | 36.1 | 29.3–42.9 | 18 | 29.5 | 17.7–41.3 | 6 | 18.2 | 4.3–32.1 | 24 | 51.1 | 36.2–65.9 | 8 | 36.4 | 14.5–58.2 | 14 | 45.2 | 26.6–63.7 |
Biopsy# | 175 | 90.2 | 86.0–94.4 | 58 | 95.1 | 99.5–100 | 24 | 72.7 | 56.7–88.7 | 43 | 91.5 | 83.2–100 | 22 | 100 | 28 | 90.3 | 79.3–100 | |
Bronchial mucosa | 124 | 63.9 | 57.1–70.7 | 46 | 75.4 | 64.3–86.5 | 14 | 42.4 | 24.6–60.2 | 32 | 68.1 | 54.3–81.9 | 15 | 68.2 | 47.0–81.9 | 17 | 54.8 | 36.3–73.4 |
Nasal brush/biopsy | 164 | 84.5 | 79.4–89.7 | 52 | 85.2 | 76.1–94.4 | 24 | 72.7 | 56.7–88.8 | 40 | 85.1 | 74.5–95.6 | 21 | 95.4 | 86.0–100 | 27 | 87.1 | 74.6–100 |
Analytic methods | ||||||||||||||||||
Electron microscopy | 150 | 77.3 | 71.4–83.3 | 50 | 82.0 | 72.0–91.9 | 23 | 69.7 | 53.1–86.2 | 35 | 74.4 | 61.5–87.4 | 18 | 81.8 | 64.3–99.3 | 24 | 77.4 | 61.8–93.0 |
Any ciliary function (native)¶ | 111 | 57.2 | 50.2–64.2 | 39 | 63.9 | 51.5–76.3 | 24 | 72.7 | 56.7–88.7 | 25 | 53.2 | 38.4–68.0 | 10 | 45.5 | 22.9–68.1 | 13 | 41.9 | 23.5–60.3 |
Ciliary visualisation | 95 | 49.0 | 41.9–56.1 | 37 | 60.6 | 48.0–73.3 | 16 | 48.5 | 30.5–66.5 | 21 | 44.7 | 29.9–59.4 | 8 | 36.4 | 14.5–58.2 | 13 | 41.9 | 23.5–60.3 |
Ciliary beat frequency | 71 | 36.6 | 29.8–43.4 | 28 | 45.9 | 33.0–58.8 | 17 | 51.5 | 33.5–69.5 | 14 | 29.8 | 16.2–43.4 | 4 | 18.2 | 0.6–35.6 | 8 | 25.8 | 9.4–42.1 |
Ciliary beat frequency and pattern | 53 | 27.3 | 21.0–33.6 | 17 | 27.8 | 16.2–39.4 | 17 | 51.5 | 33.5–69.5 | 12 | 25.5 | 12.6–38.5 | 5 | 22.7 | 3.7–41.7 | 2 | 6.5 | 0.0–15.6 |
Culture of ciliated epithelium | 42 | 21.6 | 15.8–27.5 | 18 | 29.5 | 17.7–41.3 | 13 | 39.4 | 21.8–56.9 | 4 | 8.5 | 0.0–16.8 | 4 | 18.2 | 0.6–35.7 | 3 | 9.7 | 0.0–20.7 |
Genetic testing | 58 | 29.9 | 23.4–36.4 | 29 | 47.5 | 34.6–60.4 | 9 | 27.3 | 11.2–43.3 | 10 | 21.3 | 9.1–33.4 | 3 | 13.6 | 0.0–29.2 | 7 | 22.6 | 7.0–38.2 |
European regions are defined as follows. Western Europe: Austria, Belgium, France, Germany, the Netherlands and Switzerland (n=61); British Isles: Ireland and UK (n=33); southern Europe: Cyprus, Greece, Italy, Portugal and Spain (n=47); northern Europe: Denmark, Finland, Norway and Sweden (n=22); eastern Europe: Bulgaria, Czech Republic, Estonia, Hungary, Israel, Romania, Serbia, Slovakia and Turkey (n=31) (total n=194). Tests available to a centre were either carried out in-house or by referral of the patient or specimen to another hospital. CI: confidence interval. #: bronchial mucosa or nasal brush/biopsy; ¶: ciliary visualisation, ciliary beat frequency or ciliary beat frequency and pattern.