Table 1

Patient characteristics

First HRCTSecond HRCTMean annual change
Age yrs11.05±3.3013.04±3.30Interval 1.99
Body height m1.37±0.211.47±0.200.047*
Body weight kg32.5±12.138.6±13.93.46*
FEV1 % pred74.3±18.276.0±21.01.24
FVC % pred85.5±15.785.1±16.2−0.11
FEF25–75% pred58.8±31.259.9±32.0−1.30
FEV1/FVC %75.4±10.677.6±9.80.39
Raw % pred136.1±61.8143.4±76.00.10
RV % pred136.6±42.1129.9±40.2−8.49*
TLC % pred98.0±13.296.8±11.2−1.06
RV/TLC %33.2±9.431.6±9.7−2.11*
Castile score 217.7±9.722.4±11.42.39*
Brody score 312.1±8.716.3±10.22.21*
Helbich score 48.4±3.710.1±3.80.88*
Santamaria score 58.9±4.010.7±4.10.91*
Bhalla score 68.2±3.410.0±3.60.87*
  • Data are presented as mean±sd

  • HRCT: high-resolution computed tomography

  • FEV1: forced expiratory volume in one second

  • % pred: % predicted

  • FVC: forced vital capacity

  • FEF25–75%: forced mid-expiratory flow

  • Raw: airway resistance

  • RV: residual volume

  • TLC: total lung capacity

  • *: p<0.05 in 1 yr with the one-sample t–test (unpaired)