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Table 2 Lung function test results at start of mechanical ventilation (= intercept) and average annual change in the years prior to start mechanical ventilation (= slope)

From: Lung function decline preceding chronic respiratory failure in spinal muscular atrophy: a national prospective cohort study

Lung function test (LFT)

SMA type

n (obs)

Annual change LFT (SE)

95% CI

LFT at start MV (SE)

95% CI

Standardized FVC (%)

Total group

37 (260)

− 1.75 (0.45)

− 2.66; − 0.86

28.84 (2.69)

23.48; 34.17

1c

7 (69)

− 0.24 (0.94)

− 1.99; 1.53

23.69 (6.03)

12.19; 34.99

2a

20 (146)

− 2.34 (0.58)

− 3.42; − 1.26

27.57 (3.54)

20.89; 34.27

2b

7 (40)

− 1.70 (0.94)

− 3.49; 0.06

37.82 (5.93)

26.80; 49.27

3a

3 (5)

− 2.31 (2.40)

− 6.81; 2.19

31.40 (9.29)

13.78; 49.03

Standardized FEV1 (%)

Total group

38 (385)

− 1.72 (0.34)

− 2.40; − 1.04

28.82 (2.44)

23.99; 33.69

1c

8 (74)

− 0.27 (0.72)

− 1.62; 1.09

26.10 (5.31)

15.96; 36.15

2a

20 (243)

− 1.95 (0.43)

− 2.76; − 1.15

27.79 (3.24)

21.67; 33.99

2b

7 (47)

− 2.15 (0.78)

− 3.62; − 0.67

36.40 (5.79)

25.65; 47.73

3a

3 (21)

− 2.63 (1.26)

− 5.01; − 0.25

30.72 (8.56)

14.41; 47.01

Standardized PEF (%)

Total group

20 (247)

− 1.65 (0.46)

− 2.59; − 0.71

29.96 (3.31)

23.35; 36.67

1c

5 (60)

− 0.64 (0.89)

− 2.40; 1.11

33.88 (6.75)

20.78; 47.16

2a

15 (187)

− 1.99 (0.51)

− 3.00; − 0.97

28.71 (3.91)

21.12; 36.38

PEmax (cmH2O)

Total group

32 (279)

− 0.03 (0.34)

− 0.78; 0.66

35.30 (2.97)

29.41; 41.36

1c

6 (55)

0.02 (0.86)

− 1.58; 1.62

25.21 (6.44)

13.22; 37.36

2a

17 (175)

0.17 (0.44)

− 0.74; 0.99

36.40 (3.89)

29.08; 43.72

2b

6 (37)

− 0.35 (0.83)

− 1.93; 1.19

41.63 (7.61)

27.43; 55.99

3a

3 (12)

− 0.13 (1.95)

− 3.85; 3.52

40.03 (11.68)

17.92; 61.99

  1. CI = Confidence interval; FEV1 = Forced Expiratory Volume after 1 s; FVC = Forced Vital Capacity; MV = mechanical ventilation; n = number of patients; obs = number of observations; PEF = Peak Expiratory Flow; PEmax = Maximum Peak Expiratory Pressure; SE = standard error