Figures
- FIGURE 1
Trial profile. ALS: amyotrophic lateral sclerosis; NIV: noninvasive ventilation; NBS: Norris scale bulbar subscore.
- FIGURE 2
Survival without tracheostomy a) from disease onset and b) from noninvasive ventilation (NIV) indication. Total population.
- FIGURE 3
Survival without tracheostomy. a) Patients with no or moderate bulbar dysfunction from disease onset. b) Patients with no or moderate bulbar dysfunction from noninvasive ventilation (NIV) indication. c) Patients with severe bulbar dysfunction from disease onset. d) Patients with severe bulbar dysfunction from NIV indication.
Tables
- TABLE 1
Demographic, pulmonary function and gas exchange parameters in noninvasive ventilation (NIV) group and no-NIV group
NIV No NIV p-value Patients 120 20 Age years 64.05±9.11 66.05±10.27 0.373 Males/females 56/64 6/14 0.164 Spinal/bulbar ALS onset 81/39 13/7 0.826 Time from ALS onset to NIV months 22.30±10.51 23.35±10.27 0.680 BMI kg·m−2 26.15±4.23 24.28±3.56 0.055 ALSFRS-R 29.01±7.84 28.94±7.22 0.973 NBS 26.75±10.33 18.84±8.30 0.001 No or moderate/severe bulbar dysfunction 105/15 14/6 0.052 FVC L 1.45±0.80 1.24±1.01 0.387 FVC % predicted 49.28±23.05 44.75±26.85 0.484 MIC L 1.96±0.95 1.58±1.21 0.240 PCF L·s−1 3.48±1.69 2.88±1.49 0.114 PCFMIC L·s−1 4.07±1.94 3.22±1.88 0.108 PCFMI−E L·s−1 3.70±1.07 3.26±0.64 0.090 PImax cmH2O −42.37±22.82 −35.33±21.90 0.220 PEmax cmH2O 65.54±42.28 58.72±40.95 0.519 PaO2 mmHg 74.32±11.95 81.41±11.34 0.027 PaCO2 mmHg 50.34±8.68 45.52±7.42 0.023 HCO3− mmol·L−1 29.02±3.09 20.70±3.65 0.740 %sleepSpO2<90 30.96±28.74% 18.94±31.04% 0.162 SpO2mean 91.68±3.03% 92.62±2.84% 0.239 SpO2min 79.76±9.30% 86.93±6.48% 0.004 Data are presented as n or mean±sd unless otherwise stated. ALS: amyotrophic lateral sclerosis; ALSFRS-R: revised amyotrophic lateral sclerosis functional rating scale; BMI: body mass index; NBS: Norris scale bulbar subscore; FVC: forced vital capacity; MIC: maximum insufflation capacity; PCF: peak cough flow; PCFMIC: manually assisted peak cough flow; PCFMI-E: mechanically assisted peak cough flow; PImax: maximum inspiratory pressure; PEmax: maximum expiratory pressure; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; %sleepSpO2<90: time spent with oxygen saturation measured by pulse oximetry (SpO2) <90%; SpO2mean: mean SpO2; SpO2min: minimum SpO2.
- TABLE 2
Blood gas and nocturnal gas exchange parameters at baseline situation and while using noninvasive ventilation (NIV) in NIV-group patients
Baseline Using NIV p-value PaO2 mmHg 74.32±11.95 84.06±11.42 <0.001 PaCO2 mmHg 50.34±8.68 42.11±4.32 <0.001 %sleepSpO2<90 30.96±28.74% 1.50±3.5% <0.001 SpO2mean 91.68±3.03% 95.56±1.50% <0.001 SpO2min 79.76±9.30% 92.41±3.39% <0.001 Data are presented as mean±sd unless otherwise stated. PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; %sleepSpO2<90: time spent with oxygen saturation measured by pulse oximetry (SpO2) <90%; SpO2mean: mean SpO2; SpO2min: minimum SpO2.
- TABLE 3
Blood gas, nocturnal gas exchange and set ventilator parameters under noninvasive ventilation (NIV) in the no or moderate bulbar dysfunction (BD) and severe BD groups in those patients who agreed to treatment with NIV
No or moderate BD Severe BD p-value PaO2 mmHg 84.21±1.00 85.80±14.50 0.817 PaCO2 mmHg 41.87±4.54 43.13±2.50 0.287 %sleepSpO2<90 1.26±3.35% 1.40±2.72% 0.855 SpO2mean 95.73±1.46% 95.49±1.05% 0.294 SpO2min 92.94±3.32% 91.53±2.69% 0.082 VT mL 790.09±154.41 727.14±124.67 0.102 RR breaths·min−1 14.50±1.14 14.80±1.01 0.306 Data are presented as mean±sd unless otherwise stated. PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; %sleepSpO2<90: time spent with oxygen saturation measured by pulse oximetry (SpO2) <90%; SpO2mean: mean SpO2; SpO2min: minimum SpO2; VT: tidal volume; RR: respiratory rate.
- TABLE 4
Survival data
All patients# No or moderate BD¶ Severe BD+ NIV No NIV p-value NIV No NIV p-value NIV No NIV p-value Patients 120 20 105 14 15 6 Survival from ALS onset months 43.00 (38.13–47.86) 28.00 (21.40–34.57) 0.001 43.00 (39.01–46.98) 33.00 (26.64–39.35) 0.002 40.00 (29.64–50.35) 16.00 (5.19–26.80) 0.002 Survival from NIV months 18.50 (12.62–24.38) 3.00 (0.82–5.18) 0.001 20.00 (13.71–26.28) 3.00 (0.55–5.44) 0.0001 13.00 (9.49–16.50) 3.00 (0.85–5.14) 0.001 Data are presented as mean (95% CI) unless otherwise stated. NIV: noninvasive ventilation; BD: bulbar dysfunction; ALS: amyotrophic lateral sclerosis. #: n=140; ¶: n=119; +: n=21.
- TABLE 5
Prognostic factors for survival related to noninvasive ventilation (NIV) failure: univariate analysis
Factor (mean) HR (95% CI) p-value Age (64.08 years) 1.01 (0.98–1.03) 0.600 Male sex 1.43 (0.91–2.24) 0.114 Bulbar onset 2.13 (1.35–3.36) 0.001 Time from ALS onset to NIV (22.60 months) 0.99 (0.97–1.01) 0.320 BMI (26.06 kg·m−2) 0.95 (0.89–1.01) 0.070 FVC (1.48 L) 0.51 (0.36–0.72) 0.001 FVC % predicted (49.33%) 0.98 (0.97–0.99) 0.001 MIC (1.91 L) 0.58 (0.43–0.78) 0.001 PCF (3.55 L·s−1) 0.73 (0.62–0.86) 0.001 PCFMIC (4.13 L·s−1) 0.77 (0.67–0.89) 0.001 PCFMI-E (3.73 L·s−1) 0.71 (0.56–0.90) 0.005 PImax (−44.73 cmH2O) 1.01 (1.00–1.02) 0.018 PEmax (66.37 cmH2O) 0.99 (0.98–1.00) 0.040 ALSFRS-R (20.01) 0.95 (0.92–0.98) 0.002 NBS (26.67) 0.95 (0.93–0.97) 0.001 PaO2 under NIV (83.13 mmHg) 0.98 (0.97–1.01) 0.286 PaCO2 under NIV (42.12 mmHg) 1.04 (0.99–1.09) 0.134 %sleepSpO2<90 under NIV (1.20%) 1.11 (1.04–1.19) 0.002 HR: hazard ratio; ALS: amyotrophic lateral sclerosis; BMI: body mass index; FVC: forced vital capacity; MIC: maximum insufflation capacity; PCF: peak cough flow; PCFMIC: manually assisted peak cough flow; PCFMI-E: mechanically assisted peak cough flow; PImax: maximum inspiratory pressure; PEmax: maximum expiratory pressure; ALSFRS-R: revised amyotrophic lateral sclerosis functional rating scale; NBS: Norris scale bulbar subscore; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension; %sleepSpO2<90: time spent with oxygen saturation measured by pulse oximetry <90%.
