TABLE 1

Characteristics of stable primary ciliary dyskinesia patients

Measuren
Demographics
 Age years3513.7 (5.8–17.9)
 Male:female n3520:15
Inflammatory markers
 IL-8 ng·mL−135181.4 (4.27–990.9)
 NE activity nmol·min−13512.4 (0.25–308.7)
 Total cell count ×106351.6 (0.02–34.2)
 Neutrophils %1695.5 (65–99.8)
Pulmonary function#
 FEV1 % predicted3383 (53–132)
 FVC % predicted3394 (58–119)
 FEF25–75% % predicted3366 (24–170)
Microbiology#
Haemophilus influenzae3514 (40)
Staphylococcus aureus3510 (29)
Pseudomonas aeruginosa355 (14)
Streptococcus spp.352 (6)
 Usual flora10 (29)
  • Data are presented as median (range) or n (%), unless otherwise stated. IL: interleukin; NE: neutrophil elastase; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; FEF25–75%: forced expiratory flow at 25–75% of FVC. #: patients could be infected with more than one pathogen.