Table 1 Patient characteristics and PFT results in patients and volunteers
From: Lung MRI and impairment of diaphragmatic function in Pompe disease
| Â | Patients | Volunteers | P-value |
|---|---|---|---|
Age (years) | 46 (32-66) | 43 (27-55) | 0.25 |
Gender, (% males) | 5 (50) | 3 (50) | 1.0 |
Height (cm) | 178 (154-196) | 177 (175-190) | 0.39 |
Weight (kg) | 73 (61-88) | 85 (65-94) | 0.13 |
BMI (kg/m2) | 23.4 (20.6-25.4) | 24.9 (21-25.7) | 0.18 |
Duration of the disease (years) | 16 (9-30) | - | - |
Duration of ERT (years) | 5.5 (0-7) | - | - |
Wheelchair-dependent (%) | 1 (10) | 0 (0) | 0.79 |
Ventilator-dependent (%) | 3 (30) | 0 (0) | 0.37 |
Pulmonary function test | |||
FVCsitting (%) | 60 (45-84) | 102 (92-111) | 0.001 |
FVCsupine (%) | 43 (27-70) | 102 (87-113) | 0.001 |
ΔFVC (%)a | 33 (11-44) | 0 (0-10) | 0.001 |
FEV1sitting (l/s) | 59 (42-80) | 98 (85-117) | 0.001 |
FEV1supine (l/s) | 40 (30-63) | 91 (76-112) | 0.001 |
MIP (kPa) | 6.9 (3.9-8.3) | 8.6 (6.4-11.8) | 0.07 |
MEP (kPa) | 10.0 (6.4-11.8) | 12.5 (10.3-14.2) | 0.02 |