[The silent yet successful revolution of mechanical ventilation in patients with neuromuscular disorders, in particular Duchenne muscular dystrophy].
While developments in molecular biology have been giving rise to breakthroughs in diagnosis, management and promising treatment approaches for neuromuscular disorders, there has been a silent revolution in ventilatory support of patients with a range of neuromuscular disorders with respiratory insufficiency. In a Dutch retrospective study on ventilatory support in 48 boys with Duchenne muscular dystrophy, a 5-year survival rate of 75% was found, with 67% of the patients still living with their relatives. These data are in line with reports from Great Britain. The significant impact of ventilatory support on the natural history of various neuromuscular disorders with respiratory insufficiency is a major breakthrough.
van Engelen
B G M
BG
Universitair Medisch Centrum St Radboud, Neuromusculair Centrum, Instituut voor Neurologie, HP 935, Postbus 9101, 6500 HB Nijmegen. b.vanengelen@neuro.umcn.nl
D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology* / Q000628:therapy; D009468:Neuromuscular Diseases / Q000503:physiopathology* / Q000628:therapy; D012121:Respiration, Artificial / Q000379:methods*; D012131:Respiratory Insufficiency / Q000628:therapy*