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Table 2 Outcome measures 2011 training cohort vs. comparable control patients

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

 

2011 Training cohort (n = 19)

Control patients (n = 10)

p value

FVC upright (% of normal)

75 [43;100]

59 [52;89]a

.290

FVC supine (% of normal)

48 [17;84]

34 [23;79]a

.475

VO2peak (ml/min/kg)

17.3 [10.0;35.6]b

18.7 [12.9;29.8]c

.679

VO2peak (% of normal)

64 [36;116]b

59.5 [38;95]c

.466

Wmax (Watt)

84 [26;226]b

89.5 [41;162]c

.966

Wmax/kg (Watt/kg)

1.1 [0.4;2.9]b

1.1 [0.6;1.8]c

.831

6MWT (m)

402.0 [289.0;601.0]d

362.0 [225.0;584.0]a

.329

MRC sum score (% of normal)

83.1 [69.2;98.5]

83.3 [65.0;97.7]a

.933

HHD sum score (% of normal)

82.6 [40.4;96.7]e

90.0 [44.1;97.7]c

.683

QMFT sum score

35 [19;62]

42 [23;60]a

.856

ActiGraph activity score (counts/hour)

36834.8 [12047.3;97689.5]e

38492.4 [17206.7;49131.0]a

.781

  1. All values, if not stated otherwise, are presented as a median with a minimum and maximum range; a n = 9, b n = 17, c n = 8, d n = 16, e n = 18, 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