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