Zaldivar T, Gutierrez J, Lara G, Carbonara M, Logroscino G, Hardiman O
Institute of Neurology and Neurosurgery, Havana, Cuba.
Neurology. 2009 May 12;72(19):1640-5. doi: 10.1212/WNL.0b013e3181a55f7b.
To describe ALS mortality rates in the well-characterized ethnically mixed Cuban population over a 6-year period.
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.
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.
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).
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.
描述在6年期间特征明确的种族混合古巴人群中肌萎缩侧索硬化症(ALS)的死亡率。
针对非欧洲人群的基于人群的ALS流行病学研究较少。来自美国的初步数据表明,西班牙裔和非洲裔群体中ALS的发病率低于欧洲裔群体。古巴有1100万人口,由三个主要的祖先群体组成,按肤色分类为白人(65%)、混血(24%)和黑人(10%)。医疗保健水平高且免费。古巴非常适合确定不同种族混合人群中ALS的发病率。
在古巴中央统计局2001年至2006年的多病因死亡率档案中搜索与ALS对应的编码。按死亡时的性别、种族/民族、年龄和地理区域计算年龄调整死亡率。
共识别出432例诊断为ALS的患者。平均死亡年龄为63.7岁。男性略占优势(1.1:1)。15岁以上总人口的ALS调整死亡率为每10万人0.83例。混血人群中每10万人的调整死亡率(0.55;置信区间[CI]0.4 - 0.72)显著低于白人(0.93;CI 0.83 - 1.03)和黑人(0.87;CI 0.62 - 1.17)。各地区神经科医生数量与ALS死亡率之间无相关性(r = 0.268,p = 0.335)。
古巴ALS的总体死亡率与美国西班牙裔人群中描述的相似,低于北欧人群。ALS死亡率在混血人群中最低。祖先起源可能在ALS易感性中起作用。