TABLE 1

Study demographics

No current infectionInfected#p-valueNo NE activityFree NE activityp-value
Subjects n33313232
Age years2.28±2.24+3.53±1.92+0.02002.93±2.192.97±2.270.9497
Sex female/male13/2016/150.451518/1411/210.1313
Phe508del homozygous17 (51.52)16 (51.61)1.000017 (53.13)16 (50.00)1.0000
IL-8 ng·mL-11.80 (0.43–2.85)1.88 (0.93–4.68)0.48481.03 (0.28–1.87)§2.87 (1.83–4.92)§<0.0001
NE activity nM3.39 (3.39–39.66)+95.93 (3.39–179.80)+0.01963.39 (3.39–3.39)§102.50 (45.85–336.90)§<0.0001
Neutrophils ×103 cells·mL-1116.50 (16.83–163.04)160.92 (39.47–861.49)0.122434.71 (9.62–100.34)§240.45 (129.73–870.74)§<0.0001
Pseudomonas positive1 (3.03)+7 (22.58)+0.02452 (6.25)6 (18.75)0.2565
Bronchiectasis present16 (48.48)+25 (80.65)+0.009621 (65.63)20 (62.50)1.0000
Bronchiectasis score1.00 (0.00–1.75)+2.00 (1.00–10.00)+0.00071.00 (0.00–2.00)§2.00 (1.00–10.00)§0.0084
  • Data are presented as n, mean±sd, n (%) or median (interquartile range). Values in bold are statistically significant. NE: neutrophil elastase; IL: interleukin. #: bronchoalveolar lavage fluid (BALF) with >104 bacterial CFU·mL-1; : BALF with >200 ng·mL-1 (>6.78 nM); +: statistical significance between no current infection and infected; §: statistical significance between no NE activity and free NE activity.