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Table 4 Outcome measures physically active vs. inactive patients (2010 WHO norm)

From: Long-term benefits of physical activity in adult patients with late onset Pompe disease: a retrospective cohort study with 10 years of follow-up

 

Physically active (n = 16)

Physically inactive (n = 13)

p value

FVC upright (% of normal)

81 [54;100]

65.5 [43;85]a

.107

FVC supine (% of normal)

52 [26;84]

41 [17;58]a

.124

VO2peak (ml/min/kg)

19.4 [12.9;35.6]b

16.6 [10.0;21.4]c

.026

VO2peak (% of normal)

65 [36;116]b

58 [38;108]c

.798

Wmax (Watt)

107 [41;226]b

54 [26;91]c

.007

Wmax/kg (Watt/kg)

1.4 [0.6;2.9]b

0.7 [0.4;1.3]c

.003

6MWT (m)

453.0 [225.0;601.0]d

365.0 [237.0;544.0]e

.338

MRC sum score (% of normal)

85.8 [69.2;98.5]

80.0 [65.0;94.6]a

.142

HHD sum score (% of normal)

92.5 [60.9;97.7]d

80.3 [40.4;93.4]c

.027

QMFT sum score

42.5 [29;62]

29.5 [19;47]a

.005

ActiGraph activity score (counts/hour)

40483.1 [20768.3;97689.5]

31209.6 [12047.3;42385.2]c

.001

  1. All values, if not stated otherwise, are presented as a median with a minimum and maximum range; a n = 12, b n = 14, c n = 11, d n = 15, e n = 10, missings due to inadequate technical performance or not performed due to logistic reasons; Mann–Whitney U test (Exact significance 2-tailed) is used
  2. FVC Forced vital capacity, VO2peak Peak Oxygen Uptake, Wmax Maximum Workload, 6MWT 6 Minute Walking Test (maximum walking distance in 6 min), MRC Medical Research Council (manual muscle strength test), HHD Hand Held Dynamometer (quantitative muscle strength test), QMFT Quick Motor Function Test
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172