Reduced frequency of ALS in an ethnically mixed population
Reduced frequency of ALS in an ethnically mixed population
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DOI：
10.1212/wnl.0b013e3181a55f7b
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发表时间：
2009-05-12
期刊：
影响因子：
9.9
通讯作者：
Hardiman, O.
中科院分区：
文献类型：
--
作者：
Zaldivar, T.;Gutierrez, J.;Hardiman, O.
目的：描述6年来特征鲜明的古巴种族混合人群的ALS死亡率。背景：在非欧洲人中很少有基于人群的ALS流行病学研究。来自美国的初步数据表明，与欧洲血统的人相比，西班牙裔和非洲裔的ALS发病率较低。古巴1100万人口包括三个主要的祖先群体，按肤色分为白色（65%），混合（24%）和黑色（10%）。医疗服务是高标准的，而且是免费的。古巴是一个理想的地方，可以在不同种族血统的混合人口中建立ALS的频率。方法：检索2001 ~ 2006年古巴中央统计局的多因死亡率档案，查找ALS对应的编码。年龄调整死亡率按死亡时的性别、种族/民族、年龄和地理区域计算。结果：432例被诊断为ALS的患者。平均死亡年龄为63.7岁。男性有轻微优势（1.1:1）。15岁以上总人口中ALS的调整死亡率为0.83 / 10万。混合人群的校正死亡率（0.55，可信区间[CI] 0.4-0.72）明显低于白人（0.93，可信区间[CI] 0.83-1.03）和黑人（0.87，可信区间[CI] 0.62-1.17）。各地区神经科医师人数与ALS死亡率无相关性（r = 0.268, p = 0.335）。结论：古巴ALS的总死亡率与美国西班牙裔人群相似，低于北欧人群。ALS的死亡率在混合血统人群中最低。祖源可能在ALS易感性中起作用。神经病学(R) 2009；72:1640 - 1645
Objective: To describe ALS mortality rates in the well-characterized ethnically mixed Cuban population over a 6-year period. Background: There have been few population-based epidemiologic studies of ALS in non-Europeans. Preliminary data from the United States suggest a lower frequency of ALS in Hispanic and African groups compared with those of European descent. The Cuban population of 11 million comprises three main ancestral groups classified by skin color as white (65%), mixed (24%), and (black 10%). Medical care is of a high standard and is free. Cuba is ideally placed to establish the frequency of ALS in an admixed population of diverse ethnic origin.Methods: Multiple-cause mortality files from the Central Statistics office in Cuba for the years 2001 through to 2006 were searched for codes corresponding to ALS. Age-adjusted mortality rates were calculated by sex, race/ethnicity, age, and geographic region at time of death.Results: Four hundred thirty-two patients with a diagnosis of ALS were identified. The mean age at death was 63.7 years. There was a slight male predominance (1.1:1). The adjusted death rate from ALS for the total population older than 15 years was 0.83 per 100,000. The adjusted mortality rate per 100,000 was considerably lower in the mixed population ( 0.55; confidence interval [CI] 0.4-0.72) than in whites (0.93; CI 0.83-1.03) and blacks (0.87; CI 0.62-1.17). There was no correlation between the number of neurologists in each region and the mortality rate from ALS (r = 0.268, p = 0.335).Conclusions: The overall mortality rate from ALS in Cuba is similar to that described in Hispanic populations in the United States and is lower than in Northern European populations. Mortality from ALS is lowest in a population of mixed ancestry. Ancestral origin is likely to play a role in ALS susceptibility. Neurology (R) 2009;72:1640-1645