Skip to main content

Table 2 Participant characteristics

From: Chest MRI to diagnose early diaphragmatic weakness in Pompe disease

  Pompe patients with decreased spirometry results
(FVC supine
 < 80%) (n = 22)
Pompe patients with normal spirometry results
(FVC supine
 ≥ 80%) (n = 13)
Healthy controls (n = 18) p value
Sex, number of males (%) 11 (50%) 7 (54%) 8 (44%) 0.869
Age, years 45 ± 16 31 ± 14 43 ± 14 0.030
Height, cm 178 ± 11 174 ± 9 178 ± 12 0.761
Weight, kg 76 ± 16 70 ± 13 79 ± 14 0.291
BMI, kg/m2 24 ± 4 23 ± 4 25 ± 3 0.262
Disease duration, years 16 ± 8 9 ± 9 0.018
Patients on ERT, n (%) 18 (82%) 6 (46%) 0.028
Duration of ERT, years 6 ± 5 6 ± 4 0.811
Non-invasive ventilation, n (%) 1 (3%) 0 (0%) 0.435
FVC upright, % of predicted 81 ± 11 97 ± 9 106 ± 8  < 0.001
FVC supine, % of predicted 60 ± 12 94 ± 9 102 ± 8  < 0.001
Δ FVC, % of predicted 21 ± 10 4 ± 4 4 ± 4  < 0.001
MIP, % of predicted 72 ± 23 88 ± 28 106 ± 25 0.001
MEP, % of predicted 80 ± 28 81 ± 28 112 ± 28 0.002
  1. Characteristics and outcomes of pulmonary function tests of Pompe patients and healthy controls. Continues values are presented as mean ± standard deviation and were tested using the Mann Whitney or Kruskal Wallis tests. Categorical values are presented as number with percentage and were tested with the chi square test. P-values indicate overall differences between groups
  2. BMI = body mass index, ERT = enzyme replacement therapy, FVC = forced vital capacity, Δ FVC = FVC upright – FVC supine, MIP = maximum inspiratory pressure, MEP = maximum expiratory pressure