Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience

摘要: Chronic respiratory insufficiency is inevitable in the course of disease progression patients with Duchenne muscular dystrophy (DMD). Without mechanical ventilation (MV), morbidity and mortality are highly likely towards end second decade life. The present review reports evidence clinical implications regarding DMD treated MV. There no doubt that nocturnal hypercapnia precedes daytime hypercapnia. Historical comparisons have provided non-invasive intermittent positive pressure (NIPPV) at night effective improves quality life survival by 5-10 years. By contrast, optimal criteria timing for initiation NIPPV inconsistent. A recent randomized study however demonstrated benefits commencing as soon hypoventilation detected (Ward S, et al., Randomised controlled trial (NIV) neuromuscular chest wall normocapnia. Thorax 2005; 60: 1019-24). respective role three hypotheses indirect action on blood gases may be complimentary; main improvement due to improved ventilatory response CO2. ultimate time offer full most advantageous interface lacking evidence. Full NIV possible a combination nasal mask during mouthpiece day, tracheostomy when techniques cough-assistance useless treat chronic cough insufficiency.