Table 1 Demographic data of the five groups of patients with Duchenne muscular dystrophy: morning measurements
Group 1Group 2Group 3Group 4Group 5
Age (years)***17 (2.9 ) (14.9 to 19.1)18.6 (2.8) (16.4 to 20.7)21.6 (4.6) (18.6 to 24.7)22.2 (3.3) (19.9 to 24.4)29.1 (6.2) (24.5 to 33.8)
BMI (kg/m2)21.7 (6.5) (13 to 28.2)20.7 (6.2) (16 to 25.3)20.9 (8.8) (15 to 26.8)18.3 (6.8) (13.7 to 22.8)18.8 (5.4) (14.8 to 22.8)
VC (%)***44.5 (16) (33.1 to 55.9)23.3 (5.4) (19.2 to 27.5)25.9 (8.4) (20.3 to 31.6)14.8 (4.2) (12 to 17.6)12.1 (2.4) (10.2 to 14)
MVV (l/min)***60.8 (18.1) (47.9 to 73.7)34.7 (8.2) (28.4 to 41)41.4 (11.7) (33.5 to 49.2)26.4 (13.6) (17.2 to 35.4)18.2 (4.0) (15.2 to 21.3)
VT (ml)**425 (188) (291 to 559)252 (88) (184 to 320)305 (74) (256 to 355)274 (84) (218 to 331)241 (44) (208 to 275)
RR (breath/min)**17.5 (5) (13.9 to 21.1)22.4 (6.4) (17.6 to 27.3)19.3 (4.1) (16.6 to 21.9)19.6 (2.5) (17.9 to 21.2)24.8 (4.8) (21.1 to 28.5)
VE (l/min)6.9 (2.2) (5.3 to 8.5)5.3 (1.7) (4 to 6.6)5.8 (1.4) (4.8 to 6.7)5.4 (1.7) (4.2 to 6.6)5.9 (1.1) (5.0 to 6.8)
Ti/Ttot0.46 (0.04) (0.44 to 0.49)0.44 (0.08) (0.39 to 0.5)0.41 (0.08) (0.36 to 0.46)0.44 (0.04) (0.41 to 0.47)0.43 (0.05) (0.40 to 0.47)
MIP (cm H2O)***46.7 (14.8) (36.1 to 57.2)24.1 (8.5) (17.6 to 30.6)26.9 (10.1) (20.1 to 33.7)14 (5.6) (10.2 to 17.7)11.1 (3.2) (8.7 to 13.5)
P0.11.84 (0.78) (1.28 to 2.4)1.61 (0.37) (1.32 to 1.9)1.42 (0.4) (1.14 to 1.69)1.35 (0.5) (1.02 to 1.67)1.48 (0.71) (0.93 to 2.02)
TT0.1*0.019 (0.008) (0.013 to 0.025)0.034 (0.022) (0.017 to 0.051)0.024 (0.01) (0.017 to 0.031)0.046 (0.021) (0.031 to 0.06)0.059 (0.026) (0.039 to 0.079)
Tlim (s)**422 (186) (288 to 555)429 (249) (237 to 620)256 (195) (125 to 387)202 (197) (70 to 334)121 (81) (59 to 183)
  • Data are presented as mean (SD) with 95% confidence interval.

  • BMI, body mass index; VC, vital capacity; MVV, maximal voluntary ventilation; VT, tidal volume; RR, respiratory rate; VE, minute ventilation; Ti, inspiratory time; Ttot, total breath time; Ti/Ttot, duty cycle; MIP, maximum inspiratory pressure; P0,1, occlusion pressure; TT0.1, tension-time index; Tlim, muscle endurance time.

  • Group 1, no assisted ventilation (NIPPV) and 24/24 h normocapnia; group 2, nocturnal hypercapnia prior to start of NIPPV implementation; group 3, nocturnal NIPPV with no breathlessness; group 4, nocturnal NIPPV with breathlessness; group 5, full-time NIPPV.

  • *p<0.05; **p<0.01; ***p<0.001 (ANOVA).