[Effect of intermittent positive pressure ventilation on life-span and causes of death in Duchenne muscular dystrophy].

摘要: We have been introducing the intermittent positive pressure ventilation (IPPV) therapy in Duchenne muscular dystrophy (DMD) since 1990. Then, changes of life-span and causes mortality by IPPV were investigated this study. The subjects 157 patients long-term followed up DMD our hospital. Kaplan-Meier's survival curve 73 treated showed a median time 31.0 years age, whereas that 20.4 84 not IPPV. actual mean age death 29 was 25.68 +/- 5.18 years-old (M SD), which significantly higher than 74 with not-IPPV 19.76 3.47. treatment changed major death; 59.5% to 3.5% for respiratory failure, 12.2% 37.9% cardiac 0% 10.3% repiratory trouble. There certain number who died infection, tract disorder, digestive organ disorders sudden death. Thus, is effective prolongation DMD, it seems be necessary establish strategy failure other potentially fatal complications.