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Table 1 Baseline characteristics

From: Natural history of respiratory muscle strength in spinal muscular atrophy: a prospective national cohort study

 

Total number (n)

SMA type 1c (n)

SMA type 2a (n)

SMA type 2b (n)

SMA type 3a (n)

SMA type 3b (n)

Participants

      

Patients

80

6

32

22

16

4

Female gender

52

3

20

14

12

3

SMN2 copies

      

 2

4

1

1

1

1

 3

66

5

29

18

12

2

 4

10

2

3

3

2

Tests of respiratory muscle strength

      

Peak expiratory flow (PEF)

      

 Patients

79

6

31

22

16

4

 Tests

651

67

297

156

114

17

 Follow-up (years) [IQR]

6.7 [1.2–12]

6.8 [3.5–8.2]

7.3 [1.3–12.2]

6.2 [1.2–11.8]

2.1 [0.4–9.8]

11.1 [7.7–15.3]

Peak cough flow (PCF)

      

 Patients

61

4

27

19

9

2

 Tests

288

27

144

76

35

6

 Follow-up (years) [IQR]

3.6 [0.3–8.1]

6.6 [4.7–8.8]

5.6 [0.8–7.9]

3.4 [0.3–8.3]

0.9 [0.0–2.2]

2.0 [1.0–3.0]

Maximum expiratory pressure (PE)max

      

 Patients

75

6

28

22

15

4

 Tests

586

59

261

148

102

16

 Follow-up (years) [IQR]

5.8 [1.1–10.2]

4.4 [1.8–7.1]

6.1 [1.5–11.2]

6.2 [1.0–11.5]

3.0 [0.4–9.2]

7.3 [4.9–9.6]

Maximum inspiratory pressure (PI)max

      

 Patients

76

6

28

22

16

4

 Tests

590

60

263

148

103

16

 Follow-up (years) [IQR]

6.3 [1.1–10.6]

6.7 [3.5–7.6]

6.6 [1.5–13.0]

6.2 [1.0–11.5]

2.5 [0–9.2]

7.3 [4.9–9.6]

PEmax/PImax ratio

      

 Patients

75

6

28

22

15

4

 Tests

582

57

259

147

103

16

 Follow-up (years) [(IQR]

5.8 [1.1–10.2]

4.4 [1.8–7.1]

6.1 [1.5–11.2]

6.2 [1.0–11.5]

3.0 [0.4–9.2]

7.3 [4.9–9.6]

Sniff inspiratory pressure (SNIP)

      

 Patients

57

3

22

19

11

2

  1. IQR interquartile range, n number, PCF peak cough flow, PEF peak expiratory flow, PEmax maximal expiratory pressure, PImax maximal inspiratory pressure, SNIP sniff nasal inspiratory pressure