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Table 3 Clinical manifestations, medications and intervention characteristics in the registrants with DMD and BMD

From: Characteristics of Japanese Duchenne and Becker muscular dystrophy patients in a novel Japanese national registry of muscular dystrophy (Remudy)

 

DMD patients

BMD patients

 

No. of case

% of cases

No. of case

% of cases

Walking capability

    

Normal walking

254

43.6%

80

76.2%

Not able to walk, and sit without support

184

31.6%

19

18.1%

Not able to sit without support

145

24.9%

6

5.7%

 

583

100.0%

105

100.0%

Cardiac function

    

Normal

392

67.2%

76

72.4%

Dysfunction

180

30.9%

28

26.7%

Not performed

11

1.9%

1

1.0%

 

583

100.0%

105

100.0%

Respiratory function

    

Normal

65

11.1%

45

42.9%

Dysfunction

202

34.6%

18

17.1%

Not performed

316

54.2%

42

40.0%

 

583

100.0%

105

100.0%

Steroid use

    

Current

171

29.3%

6

5.7%

Used to

69

11.8%

6

5.7%

Never

343

58.8%

93

88.6%

 

583

100.0%

105

100.0%

Cardiac medication

    

Prescribed

172

29.5%

25

23.8%

Not prescribed

411

70.5%

80

76.2%

 

583

100.0%

105

100.0%

Drug

    

β-blocker

94

54.7%

16

64.0%

ACE-inhibitor

140

81.4%

19

76.0%

ARB

12

7.0%

5

20.0%

Diuretics

43

25.0%

7

28.0%

Other

29

16.9%

7

28.0%

 

172*1

100%

25*1

100%

Mechanical ventilation

    

No

455

78.0%

103

98.1%

Yes

128

22.0%

2

1.9%

 

583

100.0%

105

100.0%

Invasive

20

15.6%

2

100.0%

Non-invasive

108

84.4%

0

0.0%

 

128*2

100.0%

2*2

100.0%

Temporal

71

55.5%

0

0.0%

Continuous

57

44.5%

2

100.0%

 

128*2

100.0%

2*2

100.0%

Scoliosis surgery

    

Yes

23

3.9%

1

1.0%

No

560

96.1%

103

98.1%

Not described

0

0.0%

1

1.0%

 

583

100.0%

105

100.0%

  1. *1; The number of registrants who were prescribed with cardiovascular medicines.
  2. *2; The number of registrants using ventilator support.