Konagaya Masaaki, Sakai Motoko, Wakayama Tadashi, Kimura Seigo, Kuru Satoshi, Yasuma Fumihiko
Department of Neurology, Suzuka National Hospital.
Rinsho Shinkeigaku. 2005 Sep;45(9):643-6.
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.
自1990年以来，我们一直在杜氏肌营养不良症（DMD）患者中引入间歇性正压通气（IPPV）疗法。在此项研究中，我们调查了接受IPPV治疗后的患者寿命变化及死亡原因。研究对象为我院157例长期随访的DMD患者。73例接受IPPV治疗患者的Kaplan-Meier生存曲线显示，中位生存时间为31.0岁，而84例未接受IPPV治疗患者的中位生存时间为20.4岁。29例接受IPPV治疗患者的实际平均死亡年龄为25.68±5.18岁（均值±标准差），显著高于74例未接受IPPV治疗患者的19.76±3.47岁。IPPV治疗改变了主要死亡原因；呼吸衰竭导致的死亡比例从59.5%降至3.5%，心力衰竭导致的死亡比例从12.2%升至37.9%，呼吸问题导致的死亡比例从0%升至10.3%。有一定数量的患者死于呼吸道感染、呼吸道疾病、消化器官疾病和猝死。因此，IPPV疗法对延长DMD患者的寿命具有显著效果，似乎有必要制定针对心力衰竭和其他潜在致命并发症的治疗策略。
