TABLE 1

patient characteristics and computed tomography (CT) and microCT results

controlLAMp-value
Subjects n55
Age years51±846±120.42
Sex female n (%)5 (100)5 (100)1.00
Height cm166±4163±40.35
Weight kg68±1466±81.00
Smoking history (non-/ex smoker) n4/14/11.00
Time between diagnosis and LTx yearsNA8.5±5.0
Pulmonary function
 FEV1 LNA1.0±0.43
 FEV1 %NA37±14
 FVC LNA2.6±0.7
 FVC %NA83±17
 FEV1/FVCNA0.38±0.11
 TLC LNA6.0±1.0
 TLC %NA120±19
DLCO LNA2.4±0.6
DLCO %NA28±7
CT measurement
 Lung volume L2.47±0.503.13±0.440.21
 Lung density g·L−1128±21189±570.095
 Lung weight g318±38600±2350.095
MicroCT measurement
 TB/mL5.06±1.891.06±0.160.0079
 TB/lung12 510±42683296±3630.0079
 Diameter TB µm386±35368±680.84
 Surface density 1/µm0.11±0.020.03±0.010.0079
 Tissue percentage %37.8±2.623.4±4.70.0079
 Wall thickness µm71.8±5.9154.1±45.90.0079

Data are presented as mean±sd, unless otherwise stated. LAM: lymphangioleiomyomatosis; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; TLC: Total lung capacity; DLCO: diffusing capacity of the lung for carbon monoxide; CT: computed tomography; TB: terminal bronchiole. Bold text indicates statistical significance.