TABLE 3

Patient characteristics at the time of pulmonary exacerbation

Cystic fibrosisPrimary ciliary dyskinesiap-value
Subjects n1711
Age years12.0 (6.9–16.7)11.0 (6.8–15.3)0.94
Male6 (35)6 (54)0.32
Body mass index centile56.5 (4–90)46 (0.2–91)0.75
Pancreatic insufficient11 (65)0 (0)0.001
Pulmonary function
 FEV1 % predicted74 (45–104)65 (39–86)0.10
 FVC % predicted90 (51–109)77 (59–102)0.09
 Change in FEV1 from baseline %−18 (−36–6)−22 (−50–−4)0.66
 Change in FVC from baseline %−14 (−36–7)−15 (−36–8)0.69
 Pulmonary exacerbation score4.2 (2.7–7.1)4.1 (2.7–8.7)0.32
Inflammatory markers
 Bacterial density log10 CFU·mL−17.4 (5.4–8.5)5.1 (0.04–35.4)0.02
 Total cell count ×1061.6 (0.04–15.3)6.4 (0.04–35.4)0.06
 Neutrophils %96.1 (43.3–100)96.4 (88.3–98.8)0.86
 Neutrophils absolute ×1062.5 (0.2–15.2)11.4 (0.8–34.4)0.02
 IL-8 ng·mL−173.5 (4.4–365.9)90.8 (64.8–825.9)0.10
 NE activity nmol·min−146.3 (4.5–224.5)91.4 (11.8–166.4)0.23
  • Data are presented as median (range) or n (%), unless otherwise stated. FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; IL: interleukin; NE: neutrophil elastase.