Table 2

Cross-sectional overview of CF disease progression for the cohort, including the upper reference limit of normal LCI (ULN) derived from 140 individuals with no lung disease. LCI values for the CF cohort are also presented as age adjusted LCI (LCIadj)

VariableAge 2 years (n=27)Age 5 years (n=41)Age 7 years (n=44)Age 12 years (n=44)
LCI7.7 (6.3–13.8)7.4 (6.2–12.2)7.5 (5.7–12.4)7.9 (5.6–11.0)
LCIadj 7.2 (5.8–13.3)7.3 (6.1–12.1)7.5 (5.7–12.4)7.9 (5.6–11.0)
ULN LCI7.47.07.07.0
FEV1 (z-score)−0.1 (-2.0–2.1)*−0.1 (-3.1–2.7)−0.4 (-3.8–2.6)
Chest CT (%Dis)3.8 (0–17.5) 5.4 (0.9–32.7)
Chest CT (%Be)0.8 (0–7.6) 2.0 (0–17.5)
BMI (z-score)−0.1 (−1.7–1.8)−0.3 (−2.8–1.4)−0.2 (−1.9–2.65)−0.4 (−2.0–1.2)
  • Data are presented as median (range).

  • *30 of 41 subjects performed technically acceptable spirometry.

  • †35 of 44 subjects had a chest CT between age 6 and 8 years.

  • ‡30 of 44 subjects had a chest CT between age 11 and 13 years.

  • %Be, bronchiectasis (%); BMI, body mass index; %Dis, total airway disease (%); FEV, forced expiratory volume; LCI, lung clearance index; LCIadj, age adjusted lung clearance index; ULN, upper limit of normal.