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[巴利阿里群岛罕见病人群登记中的亨廷顿舞蹈病:2010 - 2013年期间的患病率和死亡率。西班牙]

[Huntington's Disease in Balearic Islands Population-Based Registry of Rare Diseases: Prevalence and Mortality during the Period 2010-2013. Spain].

作者信息

Cáffaro Rovira Mercedes, Salom Castell M Magdalena

机构信息

Registro poblacional de enfermedades raras de las Islas Baleares. Servicio de Epidemiología. Dirección General de Salud Pública y Participación del Gobierno de las Islas Baleares. Palma de Mallorca. España.

Registro de mortalidad de las Islas Baleares. Servicio de Epidemiología. Dirección General de Salud Pública y Participación del Gobierno de las Islas Baleares.Palma de Mallorca. España.

出版信息

Rev Esp Salud Publica. 2017 Feb 16;91:e201701020.

Abstract

OBJECTIVE

Huntington's disease is a hereditary disease with low prevalence. The low frequency of Huntington's disease leads to its inclusion as one of the pathologies in the Registry of Rare Diseases. The Balearic Islands Population-based Registry of Rare Diseases began in 2010. Previously, there had been no prevalence or mortality data for Huntington's disease in the Balearic Islands. The aim of this study was to determine the prevalence and mortality of Huntington's disease in the Balearic Islands between 2010 and 2013.

METHODS

The data sources were the Balearic Islands Population-based Registry of Rare Diseases, from which the diagnosed cases were obtained; the Balearic Islands Mortality Register, from which the deceased cases were obtained; the Balearic Islands Health Service, from which the number of Health Cards was obtained; and the National Institute for Statistics, from which population data were obtained. Prevalence and mortality rates were calculated.

RESULTS

The Balearic Islands Population-based Registry of Rare Diseases registered 27 cases of Huntington's disease between 2010-2013. 63% of these were women. The period prevalence rate was 2.6 per 100,000 and the period mortality rate was 1.1 per 100,000. Menorca was the island with the highest rates, the prevalence rate was 5,9 per 100,000 and the mortality rate was 2,1 per 100,000.

CONCLUSIONS

Prevalence and mortality of Huntington's disease in the Balearic Islands are low compared to similar areas.

摘要

目的

亨廷顿舞蹈症是一种患病率较低的遗传性疾病。因其低发病率,它被纳入罕见病登记册中的疾病之一。巴利阿里群岛罕见病人群登记始于2010年。此前,巴利阿里群岛没有关于亨廷顿舞蹈症的患病率或死亡率数据。本研究的目的是确定2010年至2013年间巴利阿里群岛亨廷顿舞蹈症的患病率和死亡率。

方法

数据来源包括巴利阿里群岛罕见病人群登记册(从中获取确诊病例)、巴利阿里群岛死亡登记册(从中获取死亡病例)、巴利阿里群岛卫生服务机构(从中获取健康卡数量)以及国家统计局(从中获取人口数据)。计算患病率和死亡率。

结果

巴利阿里群岛罕见病人群登记册在2010 - 2013年间登记了27例亨廷顿舞蹈症病例。其中63%为女性。期间患病率为每10万人2.6例,期间死亡率为每10万人1.1例。梅诺卡岛是发病率最高的岛屿,患病率为每10万人5.9例,死亡率为每10万人2.1例。

结论

与类似地区相比,巴利阿里群岛亨廷顿舞蹈症的患病率和死亡率较低。

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