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Table 1 Basic information of patients with dystrophinopathy

From: Serum creatinine as a biomarker for dystrophinopathy: a cross-sectional and longitudinal study

  

Number (%)

 

Total number (%)

Age (y)

0–4 y

5–9 y

10–14 y

15–19 y

20–25 y

Ethnic

101

208

47

11

10

377

 

 East Asian

101 (100.00)

208 (100.00)

47 (100.00)

11 (100.00)

10 (100.00)

377 (100.00)

7 (4–9)

Clinical phenotype

101

208

47

11

10

377

 

 BMD

19 (18.81)

44 (21.15)

15 (31.91)

7 (63.64)

9 (90.00)

94 (24.93)

7 (5–12)

 DMD

82 (81.19)

164 (78.85)

32 (68.09)

4 (36.36)

1 (10.00)

283 (75.07)

7 (4–8)

Vignos scale

78 (77.23)

205 (98.56)

46 (97.87)

11 (100.00)

9 (90.00)

349 (92.57)

7 (5–9)

Brooke scale

78 (77.23)

202 (97.12)

37 (78.72)

11 (100.00)

9 (90.00)

337 (89.39)

7 (5–9)

complete GSGC scale

18 (17.82)

45 (21.63)

19 (40.43)

5 (45.45)

3 (30.00)

90 (23.87)

7 (5–10)

 Gait

42 (41.58)

107 (51.44)

33 (70.21)

7 (63.64)

4 (40.00)

193 (51.19)

7 (5–9)

 Time to walk 10 m

8 (7.92)

20 (9.62)

0 (0.00)

0 (0.00)

2 (20.00)

30 (7.96)

6.5 (4–8)

 Climbing stairs

24 (23.76)

54 (25.96)

27 (57.45)

6 (54.55)

3 (30.00)

114 (30.24)

7 (5–10.25)

 Time to climb four standard steps

14 (13.86)

20 (9.62)

0 (0.00)

0 (0.00)

2 (20.00)

36 (9.55)

5 (4–7)

 Gowers’ manoeuvre

46 (45.54)

111 (53.37)

33 (70.21)

8 (72.73)

5 (50.00)

203 (53.85)

7 (5–9)

 Time to stand up from ground

41 (40.59)

66 (31.73)

7 (14.89)

2 (18.18)

3 (30.00)

119 (31.56)

6 (4–8)

 Arising from a chair

18 (17.82)

51 (24.52)

24 (51.06)

5 (45.45)

3 (30.00)

101 (26.79)

8 (5–10)

 Time to arise from sitting

10 (9.90)

27 (12.98)

3 (6.38)

0 (0.00)

2 (20.00)

42 (11.14)

7 (4.75–8)

 Loss of ambulation

0 (0.00)

7 (3.37)

16 (34.04)

4 (36.37)

1 (10.00)

28 (7.43)

11 (9.5–13.5)

MRI in legs

5 (4.95)

21 (10.10)

4 (8.51)

0 (0.00)

0 (0.00)

30 (7.96)

6.9 ± 2.71

Muscle biopsy

3 (2.97)

12 (5.77)

3 (6.38)

1 (9.09)

2 (20.00)

21 (5.57)

8(6–10)

Glucocorticoid administration

 Oral administration

10 (9.90)

74 (35.58)

20 (42.55)

0 (0.00)

2 (20.00)

106 (28.12)

7 (6–9)

 None

68 (67.33)

131 (62.98)

20 (42.55)

2 (18.18)

2 (20.00)

223 (59.15)

6 (4–8)

 Unknown

23 (22.77)

3 (1.44)

7 (14.89)

9 (81.82)

6 (60.00)

48 (12.73)

5.5 (3–15)

Mutation analysis

101

204

45

11

9

370

 

 Deletion

68 (67.33)

138 (67.65)

27 (60.00)

6 (54.55)

6 (66.67)

245 (66.22)

6.5 (4–9)

 Duplication

11 (10.89)

22 (10.78)

7 (15.56)

0 (0.00)

0 (0.00)

40 (10.81)

7.23 ± 3.18

 Point mutation

22 (21.78)

44 (21.57)

11 (24.44)

5 (45.45)

3 (33.33)

85 (22.97)

7 (4–9)

  1. BMD Becker muscular dystrophy, DMD Duchenne muscular dystrophy, GSGC Gait, Stairs, Gowers’ manoeuvre, Chair, y year. Age with normal distributions and without normal distributions were presented as mean ± standard deviation and median (interquartile range: P25–P75), respectively