Table 1.

Demographics, Primary outcomes: Questionnaires for functional capacity in Affected, Carrier and Unaffected relatives, Muscle strength measurement: 6 min walk test.

M/F	Affected 17/11 (61% M, 39% F) Mean (SD)	Carriers 2/5 (29% M, 71%F) Mean (SD)	Unaffected relatives 7/7 (50% M, 50%F) Mean (SD)	p-value*
Age (year) (N = 28, 7, 14)	52.40 (8.96)	43.31 (7.53)	48.79 (14.86)	0.57, 0.13, 0.52
BMI (kg/m2) (N = 24, 5, 9)	35.13 (26.17)	32.56 (10.95)	29.32 (4.89)	0.77, 0.97, 0.96
IBMFRS (N = 26,6,8) (out of 40)	27.31 (9.54)	38.00 (2.45)	39.88 (0.35)	0.01, 0.02, 0.90
Fatigue scale (N = 24,4,9) (out of 63)	45.29 (12.72)	34.50 (24.77)	26.89 (11.86)	0.02**
6 min walk test (m) (N = 14,6,13)	390.86 (168.64)	458.13 (95.33)	550.82 (98.23)	0.02, 0.65, 0.49

Sample sizes (N) are listed in order of affected, carriers, and unaffected relatives.

^*p-values are indicated in the order of differences between affected and unaffected individuals, differences between affected individuals and carriers, and differences between carriers and unaffected individuals. p-values ≤ 0.05 indicate significant differences

^**Indicates difference between affected and unaffected. The small sample size of the group precluded statistical analysis of the carriers.