Table 1 Patient characteristics and study datasets for each study group. NMD neuromuscular disease, DMD Duchenne Muscular Dystrophy, SMA Spinal Muscular Atrophy, uNMD uncategorized NMD, SD standard deviation, FVC functional vital capacity, pp percent predicted, PCF peak cough flow, NIV non-invasive ventialation, LCI lung clearance index, N₂ nitrogen, SF₆ sulfur hexaflouride ¹Two patients of this group did not perform this analysis ²One patient of this subgroup did not perform this analysis

n

24

12

8

4

15

Age [months]

mean (SD; range)

198 (33.75;

121–239)

198.5 (15;

153–239)

170 (78.5;

121–220)

197.5 (28.25;

178–222)

205 (51;

105–224)

Female Sex

[n (%)]

4 (16;7)

0 (0)

2 (25)

2 (50)

5 (33.3)

FVC [pp]

mean (SD; range)

35.5 (19.25;

10–100)

40 (12;

24–100)

20 (9;

10–36)

36.5 (10.25;

22–48)

95 (18;

76–120)

PCF [L/min]

mean (SD; range)

180 (145;

70–350)

235 (72.5;

140–350)

100 (15;

70–280)

160(70;

110–330)

470 (200;

180–750)

NIV n (%)

21 (87.5)

10 (83.3)

8 (100)

3 (75)

0 (0)

LCI N₂

mean (SD; range)

9.67 (2.05;

7.66–13.27)

8.64 (1.14;

7.66–10.56)

11.36 (2.54;

7.73–13.27)

9.84 (1.45;

8.44–10.52)

7.03 (0.37;

6.32–7.43)

LCI SF₆ pre mean (SD;

range)

8.658.71 (1.52;

6.41–12.36)

8.09 (1.17;

6.41–9.54)

9.80 (2.65;

6.88–12.36)

8.20 (0.71;

6.69–9.20)

7.05 (0.67;

6.23–8.61)

LCI SF₆ post mean (SD;

range)

8.79 (2.03;

6.81–15.82)¹

7.94 (1.11;

6.86–15.82)²

10.37 (2.57;

6.93–11.20)²

8.27 (0.51;

7.03–8.81)

na