Table 2 Anthropometric characteristics and cardiopulmonary exercise test variables in patients with metabolic myopathies and in healthy controls.

From: Exercise efficiency impairment in metabolic myopathies

 

Control

Non-metabolic myalgia

MAD Decreased

MAD Absent

Glycogenoses

Respiratory Chain Deficiency

Anthropometric data

Number (n)

28

73

22

7

8

5

Sex (f/m)

13/15

20/53

14/8

4/3

5/3

2/3

Age (years)

40.4 ± 13.7

38.1 ± 13.1

43.0 ± 14.1

38.6 ± 18.4

39.5 ± 26.3

31.0 ± 11.2

BMI (kg.m−2)

22.7 ± 3.2

24.3 ± 4.8

23.1 ± 3.8

27.2 ± 8.7

23.4 ± 4.1

23.1 ± 8.0

Exercise test

Duration (min)

9.9 ± 1.0

10.3 ± 1.7

9.5 ± 1.1

9.5 ± 2.4

8.7 ± 1.3

8.8 ± 1.4

Maximal power (W)

198.6 ± 67.3

191.3 ± 62.5

151.5 ± 65.9

133.6 ± 63.2

66.6 ± 21.1a,b

111.8 ± 67.6

% Predicted maximal power

110.6 ± 21.5

99.8 ± 18.1

95.5 ± 20.1

87.3 ± 27.3

48.4 ± 9.9a,b,c,d

55.8 ± 26.5a,b,c

Peak V’O2 (ml.min−1.kg−1)

35.1 ± 7.8

35.0 ± 10.0

29.9 ± 9.7

28.6 ± 14.7

20.1 ± 7.8a,b

27.8 ± 11.2

% Predicted peak V’O2

106.4 ± 23.5

100.0 ± 15.9

97.3 ± 15.5

91.5 ± 23.8

62.8 ± 10.2a,b,c

70.8 ± 23.3a,b

∆V’O2/∆WR (mLO2.min−1.W−1)

10.2 ± 0.7

11.3 ± 1.3A

11.3 ± 1.1A

12.6 ± 1.5A

14.0 ± 2.5A

13.1 ± 1.1A

HRmax (beat/min)

172.5 ± 11.5

170.6 ± 16.7

155.6 ± 19.5A,B

142.7 ± 23.5

164.0 ± 25.9

162.0 ± 30.1

% Predicted HRmax

96.3 ± 6.0

94.7 ± 11.1

88.0 ± 9.9

78.6 ± 9.8a,b

90.8 ± 5.1

85.7 ± 14.8

Peak O2 pulse (ml/beat)

14.4 ± 5.1

15.0 ± 4.3

12.9 ± 3.9

13.7 ± 4.6

7.1 ± 1.9a,b

10.6 ± 4.5

MCR

0.91 ± 0.21

0.92 ± 0.20

0.78 ± 0.15

0.58 ± 0.18a,b

1.50 ± 0.29a,b,c,d

1.15 ± 0.19c,d

Resting RER

0.78 ± 0.05

0.76 ± 0.05

0.75 ± 0.04

0.78 ± 0.08

0.74 ± 0.04

0.76 ± 0.03

Peak RER

1.27 ± 0.06 C,D

1.22 ± 0.09

1.21 ± 0.07

1.11 ± 0.10

0.91 ± 0.03A,B,C,D,E

1.25 ± 0.12

Peak V’E/V’O2

37.6 ± 5.4

36.7 ± 5.9

37.5 ± 4.4

32.9 ± 5.6

33.1 ± 5.1

41.6 ± 13.4

OUES

2.4 ± 0.8

2.4 ± 0.7

1.9 ± 0.7

2.1 ± 0.7

1.4 ± 0.6

1.8 ± 0.9

Peak V’E/V’CO2

29.7 ± 4.4

30.2 ± 4.6

31.5 ± 3.4

30.1 ± 5.0

35.3 ± 6.1

35.0 ± 12.9

V’E/V’CO2 slope

28.3 ± 4.8

29.8 ± 6.1

30.3 ± 3.8

28.3 ± 5.5

34.6 ± 4.51

35.5 ± 18.6

Resting lactate (mM)

1.3 ± 0.4

1.3 ± 0.6

1.1 ± 0.5

1.3 ± 0.3

0.8 ± 0.3A,B,D

2.6 ± 2.4

Peak lactate (mM)

6.6 ± 2.1

6.0 ± 2.7 C,D

4.3 ± 1.4A,B

3.1 ± 1.6A,B

0.9 ± 0.3A,B,C,E

5.2 ± 1.3

Resting ammonia (µM)

21.9 ± 11.4

25.5 ± 12.9

20.1 ± 10.5

29.0 ± 17.8

45.5 ± 25.8

21.4 ± 14.3

Peak ammonia (µM)

35.6 ± 18.7

43.5 ± 30.9 C

28.1 ± 14.0

34.1 ± 14.8

112.5 ± 50.5A,B,C,D,E

31.2 ± 13.3

  1. Data are reported as mean ± SD. BMI, Body Mass Index Peak; HRmax, maximal heart rate; Peak O2 pulse, oxygen pulse at peak exercise; MCR, Metabolic-Chronotropic Relationship; RER, Respiratory Exchange Ratio; OUES, Optimal Uptake Efficiency Slope; V’E, minute ventilation; V’O2, oxygen consumption; V’CO2, carbon dioxide production. Data were analyzed by a one-way ANOVA, followed by post-hoc Games-Howell or Scheffé’s pairwise comparison tests according to Levene’s test results for homogeneity of variance. Lower case letter superscripts (a-c) and upper case letter superscripts (A-D) represent statistical significance with Scheffé’s or Games-Howell post-hoc tests, respectively. a,A significantly different from Control (p < 0.05). b,B significantly different from Non-metabolic myalgia (p < 0.05). c,C significantly different from MAD Decreased (p < 0.04); d,D significantly different from MAD Absent (p < 0.05). E, significantly different from Respiratory Chain Deficiency (p < 0.03).
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