TABLE 4.
Basic characteristics of the 3013 primary ciliary dyskinesia (PCD) patients included in the international PCD cohort (iPCD Cohort) (April 2016)
Patients | 3013 (100) |
Sex | |
Male | 1490 (49) |
Female | 1523 (51) |
Location of country of residence# | |
Australia | 109 (4) |
Northern Europe | 515 (17) |
Western Europe | 1146 (39) |
Eastern Europe | 105 (4) |
Southern Europe | 341 (11) |
Western Asia | 278 (9) |
Northern America | 418 (14) |
Southern America | 101 (3) |
Diagnostic information | |
Definite PCD diagnosis¶ | 1718 (56) |
Probable PCD diagnosis+ | 420 (14) |
Clinical diagnosis only | 875 (30) |
Age years | |
0–9 | 517 (17) |
10–19 | 1156 (38) |
20–29 | 553 (18) |
30–39 | 316 (10) |
40–49 | 205 (7) |
50–59 | 137 (5) |
≥60 | 129 (4) |
Data are presented as n (%). #: based on geographical region definitions of the United Nations Statistic Division (August 2016) (Northern Europe: Denmark, Norway, UK; Western Europe: Belgium, France, Germany, Switzerland, the Netherlands; Eastern Europe: Poland; Southern Europe: Italy, Serbia; Western Asia: Cyprus, Israel, Turkey; Northern America: Canada, USA; Southern America: Argentina); ¶: defined as hallmark PCD electron microscopy findings and/or bi-allelic gene mutation identified based on the European Respiratory Society PCD Diagnostics Task Force guidelines [11]; +: abnormal light or high-frequency video microscopy finding and/or low (≤77 nL·min−1) nasal nitric oxide value.