Skip to main content

Table 3 Characteristics of the three clusters

From: Muscle histological changes in a large cohort of patients affected with Becker muscular dystrophy

Variable

Cluster 1

(n = 6)

Cluster 2

(n = 22)

Cluster 3

(n = 17)

P-value

Age

28 (26–38)

42.5 (26.75–48)

38 (30.5–43.5)

0.29

Mutation

   

0.03

 Deletions 45-x

1 (17)

17 (77.27)

11 (64.44)

 

 Deletions distal to exon 45

0 (0)

1 (4.55)

2 (611.76)

 

 Mutations proximal to exon 45

5 (83)

4 (18.18)

4 (23.53)

 

BMI

21.40 (30.36–22.61)

22.10 (20.11–24.85)

24.02 (21.36–28.48)

0.2

Disease duration

25 (23.5–26.5)

22 (9.75–30.25)

23 (20–28.5)

0.77

MFMD1

13 (11–14.25)

19 (16–20)

22 (20–24.5)

 < 0.0001

6MWT

242 (237–306)

352 (286–386)

420 (388–446)

 < 0.0001

4-Stair climb score (* n=42)

    

 2

5 (100)

6 (28.57)

2 (11.76)

0.001

 5

0 (0)

15 (71.43)

13 (76.47)

 

 6

0 (0)

0 (0)

2 (11.76)

 

10 m score

   

0.21

 3

1 (16.67)

2 (9.09)

0 (0)

 

 4

5 (83.33)

19 (86.36)

12 (70.59)

 

 5

0 (0)

1 (4.55)

3 (17.65)

 

 6

0 (0)

0 (0)

2 (11.76)

 

Gowers Score (* n=38)

   

0.17

 1

2 (50)

1 (5.56)

0 (0)

 

 2

2 (50)

13 (72.22)

8 (47.06)

 

 3

0 (0)

2 (11.11)

3 (17.65)

 

 4

0 (0)

2 (11.11)

4 (23.53)

 

 5

0 (0)

0 (0)

1 (5.88)

 

 6

0 (0)

0 (0)

1 (5.88)

 

Right Biceps Brachial Strength

12.67 (7.75–34.58)

53.17 (34.50–73.67)

141.3 (91.33–160.7)

 < 0.0001

Left Biceps Brachial Strength

15 (7.08–40.25)

59.33 (36–75.5)

135 (107–155.8)

 < 0.0001

Nuclear Centralizations

4 (2–5)

5 (3–7)

4 (3–5)

0.25

MFA %

34.18 (10.73–52.82)

72.55 (60.89–79.86)

82.59 (79.96–88.25)

 < 0.0001

Fibrotic tissue %

53.05 (36.79–84.78)

25.40 (19.69–36.06)

15.90 (11.57–18.97)

 < 0.0001

Fatty tissue %

7.22 (2.12–13.49)

0 (0–0)

0 (0–0)

 < 0.0001

Other tissue %

0.34 (0–3.14)

0 (0–0)

0 (0–0.25)

0.12

CSA I

3007 (1711–4780)

3334 (2250–6006)

5234 (4023–6390)

0.07

CSA I Fibre size variability

4667 (3519–5254)

2668 (2018–4596)

3691 (2509–4651)

0.17

CSA II

2382 (1093–3042)

3555 (1720–4776)

5769 (5044–7574)

 < 0.0001

CSA II Fibre size variability

3868 (1528–4550)

4422 (3162–6079)

4726 (4067–7309)

0.17

Regenerating fibres %

0.61 (0.12–12.94)

4.18 (0–7.34)

3.4 (0.3–6.11)

0.88

Splitting fibres%

2.17 (0–4.17)

4.6 (2.27–6.71)

1.84 (0.83–5.5)

0.07

Necrosis fibres%

1.45 (0.66–1.61)

0.81 (0–2.19)

0 (0–1.09)

0.12

  1. Statistically significant p values are shown in bold
  2. Data are expressed as median (25th-75th percentiles) and number (frequencies), as appropriate. Statistics computed by Kruskal Wallis Test, except for categorical variables (Fisher’s exact test)
  3. BMI: Body Mass Index; MFMD Motor Function Measure Domain; 6MWT: 6 Minute Walking Test; 10mScore: 10 m score; MFA: muscle fibre area; CSA: cross sectional area