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Table 2 Main diseases that can benefit from NIPPV classified according to the cause and progressiveness of the respiratory impairment

From: Clinical review: Long-term noninvasive ventilation

Parietal disorders (PFT abnormal; ↓ VC, ↓ FEV1, → FEV1/VC, ↓ RV, ↓ TLC)
   Chest wall  
Kyphoscoliosis No worsening
Sequelae of tuberculosis Slow worsening
Obesity hypoventilation syndrome Depends on obesity
   Neuromuscular disorders  
Spinal muscular atrophy No worsening
Acid maltase deficit Slow worsening (>15 years)
Duchenne muscular dystrophy Intermediate worsening (5 to 15 years)
Myotonic myopathy Intermediate worsening (5 to 15 years)
Amyotrophic lateral sclerosis Rapid worsening (0 to 3 years)
Lung diseases (PFT abnormal; → or ↓ VC, ↓ FEV1, ↓ FEV1/VC, ↑ RV, ↑ TLC)
   COPD Continuous worsening
   Bronchiectasis, cystic fibrosis Continuous worsening
Predominant ventilatory control abnormalities (PFT normal)
   Ondine's curse Improvement?
   Cheyne-Stokes breathing Depends on heart failure
Upper airway abnormalities (PFT normal)
   Obstructive sleep apnea No worsening
  1. Symbols indicate actual compared to theoretical values: ↓, decrease; ↑, increase; →, normal. COPD, chronic obstructive pulmonary disease; FEV1, forced expiratory volume in 1 second; NIPPV, noninvasive positive pressure ventilation; PFT, pulmonary function test; RV, residual volume; TLC, total lung capacity; VC, vital capacity.