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Table 6 Outcome measures physically active 2011 training cohort (continued training) vs. physically active controls

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 2011 training cohort (n = 9)

Physically active controls (n = 7)

p value

FVC upright (% of normal)

82 [55;100]

59 [54;89]

.133

FVC supine (% of normal)

56 [32;84]

34 [26;79]

.262

VO2peak (ml/min/kg)

21.2 [14.6;35.6]a

19.4 [12.9;29.8]b

.825

VO2peak (% of normal)

67 [36;116]a

59.5 [40;89]b

.395

Wmax (Watt)

107 [47;226]a

108 [41;162]b

.950

Wmax/kg (Watt/kg)

1.5 [0.7;2.9]a

1.3 [0.6;1.8]b

.640

6MWT (m)

460.5 [289.0;601.0]a

366.0 [225.0;584.0]

.613

MRC sum score (% of normal)

87.7 [69.2;98.5]

83.8 [74.4;97.7]

.939

HHD sum score (% of normal)

88.4 [60.9;96.7]

92.7 [64.2;97.7]b

.607

QMFT sum score

37 [31;62]

43 [29;60]

1.000

ActiGraph activity score (counts/hour)

47150.1 [20768.3;97689.5]

39771.1 [34180.0;49131.0]

.252

  1. All values, if not stated otherwise, are presented as a median with a minimum and maximum range; a n = 8, b n = 6, 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