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Table 1 Participant characteristics, ordered by V̇O2peak (from highest to lowest)

From: Aerobic capacity and skeletal muscle characteristics in glycogen storage disease IIIa: an observational study

Participant

Patient demographics

Clinical Characteristics

Dietary information and medications

Gender

Age (years)

Height (cm)

Body Mass (kg)

Cardiac hypertrophy (Y/N)

hepatomegaly (Y/N)

Creatine kinase (RR: 26–140 IU/L)

Triglycerides (mmol/L)

Higher protein diet

Protein supplement

UCCS

Medication

1

M

27

181.0

81.4

Y*

Y

6592

1.2

Y

N

Y

A

2

F

28

173.1

73.3

N

Y

641

1.1

N

N

N

None

3

M

26

193.2

80.6

N

Y

3842

1.6

Y

Y

N

A, D

4

M

48

180.3

69.5

N

Y

1756

2.2

Y

Y (Int)

N

B, D

5

F

50

173.9

81.1

N

Y

1266

1.4

Y

N

N

None

6

F

45

162.3

78.9

Y#

N

2442

1.1

N

N

N

None

7

M

32

188.0

112

N

Y

2682

1.5

Y

Y

Y

D

Mean (SD)

–

37 (11)

179 (10)

 

–

–

2746 (1987)

1.4 (0.4)

–

–

–

–

Median (IQ range)

–

  

80.6 (8.0)

–

–

 

–

–

–

–

–

  1. Int, Intermittent; Cardiac hypertrophy: *Y1, Maximal wall thickness 14 mm (asymmetric septal), #Y6, Maximal wall thickness 13 mm (basal septal); UCCS: uncooked cornstarch: Y, 50 g nightly; A, Allopurinol; D, Vitamin D; B, Bisphosphonate