Table 3 Pulmonary function test data for patients with late onset Pompe disease
From: Characteristics of Pompe disease in China: a report from the Pompe registry
Variable | China (n = 59) | Rest of the world (n = 1180) |
|---|---|---|
Patients with ≥1 PFT, n (%) | 27 (45.8) | 984 (83.4) |
FVC % predicted normal (upright) (%) | ||
n | 27 | 925 |
Mean (SD) | 37.2 (14.00) | 63.5 (26.71) |
Median (min, max) | 35.7 (15.1, 72.3) | 62.0 (3.0, 137.0) |
FVC % predicted normal (supine) (%) | ||
n | 10 | 558 |
Mean (SD) | 29.2 (14.41) | 55.3 (26.63) |
Median (min, max) | 23.3 (12.8, 59.4) | 53.3 (4.0, 137.0) |
MIP (PImax) (cm H2O) | ||
n | 17 | 474 |
Mean (SD) | 27.9 (13.54) | 51.0 (38.66) |
Median (min, max) | 24.5 (12.6, 60.9) | 45.0 (0.4, 611.8) |
MEP (PEmax) (cm H2O) | ||
n | 16 | 466 |
Mean (SD) | 29.4 (12.04) | 70.5 (52.78) |
Median (min, max) | 26.1 (14.3, 56.7) | 62.0 (0.2, 641.4) |