TABLE 2

Characteristics of included studies describing spirometry indices of primary ciliary dyskinesia patients

Total studies24 (100)
Study size
 ≤20 patients8 (33)
 21–50 patients10 (42)
 51–100 patients2 (8)
 >100 patients4 (17)
Number of centres
 Single centre17 (71)
 Multicentre7 (29)
Publication period
 ≤19962 (8)
 1997–20077 (29)
 ≥200815 (63)
Study region
 Europe15 (62.5)
 Asia6 (25)
 North and South America3 (12.5)
Age of participants
 Children <18 years7 (29)
 Adults ≥18 years3 (13)
 Children and adults14 (58)
Study design
 Retrospective11 (46)
 Prospective13 (54)
Diagnostics performed
 Diagnosis based only on strong clinical suspicion2 (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
 FEV124 (100)
 FVC12 (50)
Information for different age groups6 (43)
Longitudinal information8 (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.