Table 1 Characteristics of LOPD patients and healthy volunteers
HV n = 10 | LOPD n = 10 | |
|---|---|---|
Female, (%) | 5 (50) | 5 (50) |
Male (%) | 5 (50) | 5 (50) |
Age, years | 41.2 ± 14.2 | 40.6 ± 12.1 |
Weight, kg | 73.9 ± 11.9 | 65.0 ± 17.3 |
Height, cm | 176.2 ± 0.1 | 172.3 ± 0.1 |
BMI (kg/m2) | 23.6 ± 2.2 | 21.6 ± 4.0 |
Ambulatory (%) | 10 (100) | 9 (90) |
ERT (%) | 0 (0) | 8 (80) |
Nocturnal ventilation support | 0 (0) | 4 (40) |
Lung function | ||
FEV1 (%) | 100.8 ± 11.8 | 68.2 ± 23.5 |
FVC (%) | 104.9 ± 9.7 | 62.6 ± 21.4 |
Functional testing | ||
R-PACT | 36.0 ± 0.0 | 29.1 ± 10.4 |
QMFT | 64 ± 0.0 | 46.7 ± 16.1 |
6MWT (m) | 671.3 ± 116.6 | 560.3 ± 87.2 |
TUAG (s) | 1.7 ± 0.4 | 3.7 ± 3.0 |
MRC | ||
UB | ||
Proximal | 5.0 ± 0.0 | 4.3 ± 0.1 |
Medial | 5.0 ± 0.0 | 4.5 ± 0.1 |
Distal | 5.0 ± 0.0 | 4.7 ± 0.0 |
LW | ||
Proximal | 5.0 ± 0.0 | 3.9 ± 0.1 |
Medial | 5.0 ± 0.0 | 4.1 ± 0.1 |
Distal | 5.0 ± 0.0 | 4.5 ± 0.1 |
