Demographics | TSC–LAM | Sporadic LAM |
Age years | 40.1±9.9 | 39.9±8.1 |
Follow-up time years | 6.5±2.7 | 6.5±3.2 |
Deaths | 2 (5) | 3 (7.5) |
Lung function |
FEV1 L | 2.6±0.5 | 2.6±0.5 |
FEV1 % predicted | 96.8±18.9 | 93.8±15.9 |
DLCO mL·min-1·mmHg-1 | 20.4±3.9 | 20.5±4.2 |
DLCO % predicted | 95.4±19.1 | 94.8±16.7 |
Decline in FEV1 % predicted | 1.9±2.7 | 1.9±1.9 |
Decline in DLCO % predicted | 2.1±2.8 | 1.9±2.7 |
Cardiopulmonary exercise testing |
Work rate % predicted | 99±25 | 109±24 |
Peak heart rate % predicted | 89±8 | 91±8 |
V′Emax % predicted | 57±16 | 62±16 |
Breathing reserve % | 44±16 | 38±20 |
V′O2max % predicted | 80±15 | 86±21 |
V′E/V′CO2 at anaerobic threshold | 32±7 | 34±6 |
Volume occupied by cysts |
Initial cyst scores % | 5.3±5.9 | 5.6±5.2 |
Final cyst scores % | 11.8±9.6# | 15.3±9.8# |
Yearly change in cyst scores % | 1.0±1.3 | 1.4±1.7 |