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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)
  1. FVC forced vital capacity, MEP maximal expiratory pressure, MIP maximal inspiratory pressure, PFT pulmonary function test