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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