Eagle Michelle, Baudouin Simon V, Chandler Colin, Giddings David R, Bullock Robert, Bushby Kate
Newcastle Muscle Centre, International Centre for Life, Central Parkway, Newcastle upon Tyne NE1 3BZ, UK.
Neuromuscul Disord. 2002 Dec;12(10):926-9. doi: 10.1016/s0960-8966(02)00140-2.
We reviewed the notes of 197 patients with Duchenne muscular dystrophy whose treatment was managed at the Newcastle muscle centre from 1967 to 2002, to determine whether survival has improved over the decades and whether the impact of nocturnal ventilation altered the pattern of survival. Patients were grouped according to the decade of death and whether or not they were ventilated. Kaplan Meier survival analyses showed significant decade on decade improvement in survival. Mean age of death in the 1960s was 14.4 years, whereas for those ventilated since 1990 it was 25.3 years. Cardiomyopathy significantly shortened life expectancy from 19 years to a mean age of 16.9 years. Better coordinated care probably improved the chances of survival to 25 years from 0% in the 1960s to 4% in the 1970s and 12% in the 1980s, but the impact of nocturnal ventilation has further improved this chance to 53% for those ventilated since 1990.
我们回顾了1967年至2002年在纽卡斯尔肌肉中心接受治疗的197例杜氏肌营养不良症患者的病历，以确定数十年来生存率是否有所提高，以及夜间通气的影响是否改变了生存模式。患者根据死亡年代以及是否接受通气进行分组。Kaplan Meier生存分析显示，生存率逐十年有显著提高。20世纪60年代的平均死亡年龄为14.4岁，而自1990年以来接受通气的患者平均死亡年龄为25.3岁。心肌病显著缩短了预期寿命，从19岁降至平均16.9岁。更好的协调护理可能提高了活到25岁的几率，从20世纪60年代的0%提高到70年代的4%和80年代的12%，但夜间通气的影响进一步将自1990年以来接受通气患者活到25岁的几率提高到了53%。
