TY - JOUR T1 - <em>In vivo</em> monitoring of cystic fibrosis-like lung disease in mice by volumetric computed tomography JF - European Respiratory Journal JO - Eur Respir J SP - 1060 LP - 1070 DO - 10.1183/09031936.00149810 VL - 38 IS - 5 AU - M.O. Wielpütz AU - M. Eichinger AU - Z. Zhou AU - K. Leotta AU - S. Hirtz AU - S.H. Bartling AU - W. Semmler AU - H-U. Kauczor AU - M. Puderbach AU - M.A. Mall Y1 - 2011/11/01 UR - http://erj.ersjournals.com/content/38/5/1060.abstract N2 - The onset and spontaneous development of cystic fibrosis (CF) lung disease remain poorly understood. In the present study, we used volumetric computed tomography (VCT) as a new method for longitudinal in vivo monitoring of early lesions and disease progression in CF-like lung disease in β-epithelial Na+ channel (ENaC)-transgenic (TG) mice. Using a VCT scanner prototype (80 kV, 50 mA·s, scan time 19 s and spatial resolution 200 μm), βENaC-TG mice and wild-type (WT) littermates were examined longitudinally at 10 time-points from neonatal to adult ages, and VCT images were assessed by qualitative and quantitative morphological parameters. We demonstrate that VCT detected early-onset airway mucus obstruction, diffuse infiltrates, atelectasis and air trapping as characteristic abnormalities in βENaC-TG mice. Furthermore, we show that early tracheal mucus obstruction predicted mortality in βENaC-TG mice and that the density of lung parenchyma was significantly reduced at all time-points in βENaC-TG compared with WT mice (median±sem -558±8 HU in WT versus -686±16 HU in βENaC-TG at 6 weeks of age; p&lt;0.005). Our study demonstrates that VCT is a sensitive, noninvasive technique for early detection and longitudinal monitoring of morphological abnormalities of CF-like lung disease in mice, and may thus provide a useful tool for pre-clinical in vivo evaluation of novel treatment strategies for CF. ER -