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. 2017 Feb 16:91:e201701020.

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

[Article in Spanish]
Mercedes Cáffaro Rovira  1 , M Magdalena Salom Castell  2
Affiliations
  • PMID: 28206985
Free article

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

[Article in Spanish]
Mercedes Cáffaro Rovira et al. Rev Esp Salud Publica. .
Free article

Abstract
in English, Spanish

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.

Objetivo: La enfermedad de Huntington (EH) es una enfermedad hereditaria de baja prevalencia, por lo que se incluye en los registros de enfermedades raras. El registro poblacional de enfermedades raras de las Islas Baleares se inició en el año 2010. Previamente no existían datos de prevalencia y mortalidad de la EH en las Islas Baleares. El objetivo de este estudio fue determinar la prevalencia y la mortalidad de la enfermedad de Huntington en las Islas Baleares durante el periodo 2010-2013.

Metodos: Se utilizaron como fuentes de información el registro poblacional de enfermedades raras de las Islas Baleares, del que se obtuvieron los casos diagnosticados, el Registro de mortalidad de las Islas Baleares, del que se obtuvieron los casos fallecidos, el registro del Servicio de Salud de las Islas Baleares del que se obtuvieron el número de tarjetas sanitarias y del Instituto Nacional de Estadística se obtuvieron los datos de población. Se calcularon las tasas de prevalencia y de mortalidad.

Resultados: El registro poblacional de enfermedades raras de las Islas Baleares durante el periodo 2010-2013 registró 27 casos de EH. El 63% fueron mujeres. La tasa de prevalencia fue de 2,6 por 105 y la tasa de mortalidad de 1,1 por 105. La isla de Menorca fue la más afectada con una prevalencia de 5,9 por 105 y una mortalidad de 2,1 por 105.

Conclusiones: La prevalencia y mortalidad de la EH en las Islas Baleares son bajas en comparación con zonas del entorno.

Keywords: Huntington’s Disease; Mortality; Prevalence; Rare Diseases; Registries; Spain.

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Conflict of interest statement

Disclosure: The authors report no conflicts of interest in this work.

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