Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation
Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation
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DOI：
10.1016/s0960-8966(02)00140-2
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发表时间：
2002-12-01
影响因子：
2.8
通讯作者：
Bushby, K
中科院分区：
文献类型：
--
作者：
Eagle, M;Baudouin, SV;Bushby, K
我们回顾了自1967-2002年间在纽卡斯尔肌肉中心接受治疗的197例Duchenne肌营养不良症患者的笔记，以确定几十年来存活率是否有所改善，以及夜间通风的影响是否改变了存活率模式。根据死亡十年和是否使用呼吸机对患者进行分组。Kaplan Meier生存分析显示，十年来存活率显著提高。20世纪60年代的平均死亡年龄为14.4岁，而自1990年以来呼吸机的平均死亡年龄为25.3岁。心肌病显著缩短了预期寿命，从19岁缩短到平均16.9岁。更好的协调护理可能会将存活率从20世纪60年代的0%提高到25年，从20世纪60年代的0%提高到70年代的4%和80年代的12%，但自1990年以来接受通气者的夜间通风的影响进一步将这一机会提高到53%。(C)2002 Elsevier Science B.V.保留所有权利。
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. (C) 2002 Elsevier Science B.V. All rights reserved.