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丹麦的进行性肌营养不良症。自然病史、患病率和发病率。

Progressive muscular dystrophy in Denmark. Natural history, prevalence and incidence.

作者信息

Leth A, Wulff K, Corfitsen M, Elmgreen J

出版信息

Acta Paediatr Scand. 1985 Nov;74(6):881-5. doi: 10.1111/j.1651-2227.1985.tb10052.x.

Abstract

Prevalence of progressive muscular dystrophy in 1965 and incidence in Denmark for the period 1965 to 1975 was studied by collection of data from hospital departments, nursery homes and general practitioners. This material was supplemented with information from death certificates and disablement pension records. Total patient group included 445 patients with progressive muscular dystrophy alive January 1st 1965. Prevalence was 69.4 per 10(6) male inhabitants in the Duchenne type, whereas the prevalences in the limb-girdle and facioscapulohumeral types were 36.5 and 18.6 per 10(6) inhabitants, respectively. Incidence was 222 per 10(6) male newborns in the Duchenne type, 66 and 26 per 10(6) newborns for the limb-girdle and the facioscapulohumeral types, respectively. Figures for the Duchenne type are in agreement with previous results. Both prevalence and incidence rates for the limb-girdle and the facioscapulohumeral types exceed published figures by a factor 3 to 6. These high Danish rates seem to reflect the true prevalence and incidence in the less serious types of progressive muscular dystrophy, probably because the Danish health system with free medical care and easy access to specialized hospital departments makes it possible to identify all cases of progressive muscular dystrophy.

摘要

通过收集医院科室、养老院和全科医生的数据,对1965年进行性肌营养不良的患病率以及1965年至1975年丹麦的发病率进行了研究。这些资料还补充了死亡证明和残疾抚恤金记录中的信息。总的患者群体包括1965年1月1日还活着的445例进行性肌营养不良患者。杜氏型每10⁶男性居民中的患病率为69.4,而肢带型和面肩肱型每10⁶居民中的患病率分别为36.5和18.6。杜氏型的发病率为每10⁶男性新生儿222例,肢带型和面肩肱型分别为每10⁶新生儿66例和26例。杜氏型的数据与之前的结果一致。肢带型和面肩肱型的患病率和发病率均比已发表的数据高出3至6倍。丹麦的这些高发病率似乎反映了不太严重类型的进行性肌营养不良的真实患病率和发病率,这可能是因为丹麦的医疗系统提供免费医疗且能方便地进入专科医院,使得所有进行性肌营养不良病例都能被识别出来。

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