Characteristics of included studies describing spirometry indices of primary ciliary dyskinesia patients
Total studies | 24 (100) |
Study size | |
≤20 patients | 8 (33) |
21–50 patients | 10 (42) |
51–100 patients | 2 (8) |
>100 patients | 4 (17) |
Number of centres | |
Single centre | 17 (71) |
Multicentre | 7 (29) |
Publication period | |
≤1996 | 2 (8) |
1997–2007 | 7 (29) |
≥2008 | 15 (63) |
Study region | |
Europe | 15 (62.5) |
Asia | 6 (25) |
North and South America | 3 (12.5) |
Age of participants | |
Children <18 years | 7 (29) |
Adults ≥18 years | 3 (13) |
Children and adults | 14 (58) |
Study design | |
Retrospective | 11 (46) |
Prospective | 13 (54) |
Diagnostics performed | |
Diagnosis based only on strong clinical suspicion | 2 (8) |
Diagnosis based on EM or a combination of tests in most patients; few only on strong clinical suspicion# | 3 (13) |
Diagnosis based on EM or a combination of tests in all patients# | 19 (79) |
Spirometry indices reported | |
FEV1 | 24 (100) |
FVC | 12 (50) |
Information for different age groups | 6 (43)¶ |
Longitudinal information | 8 (33) |
Data are presented as n (%). EM: electron microscopy; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity. #: combination of the following: EM, nasal nitric oxide, high-frequency video-microscopy or light microscopy, or genetic analysis. ¶: of 14 studies including children and adults.