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dc.contributor.authorAlonso-Ferreira, Verónica 
dc.contributor.authorSanchez-Diaz, German 
dc.contributor.authorVillaverde-Hueso, Ana 
dc.contributor.authorPosada De la Paz, Manuel 
dc.contributor.authorBermejo-Sanchez, Eva
dc.identifier.citationInt J Environ Res Public Health. 2018 Aug 10;15(8). pii: E1715.es_ES
dc.description.abstractThis study aimed to analyse population-based mortality attributed to rare congenital anomalies (CAs) and assess the associated time trends and geographical differences in Spain. Data on CA-related deaths were sourced from annual mortality databases kept by the National Statistics Institute of Spain (1999⁻2013). Based on the ICD-10, only CAs corresponding to rare diseases definition were included in this study. Annual age-adjusted mortality rates were calculated and time trends were evaluated by joinpoint regression analysis. Geographical differences were assessed using standardised mortality ratios and cluster detection. A total of 13,660 rare-CA-related deaths (53.4% males) were identified in the study period. Annual age-adjusted mortality rates decreased by an average of -5.2% (-5.5% males, -4.8% females, p < 0.001). Geographical analysis showed a higher risk of rare-CA-related mortality in regions largely located in the south of the country. Despite their limitations, mortality statistics are essential and useful tools for enhancing knowledge of rare disease epidemiology and, by extension, for designing and targeting public health actions. Monitoring rare-CA-related mortality in Spain has shown a 15-year decline and geographical differences in the risk of death, all of which might well be taken into account by the health authorities in order to ensure equality and equity, and to adopt appropriate preventive measures.es_ES
dc.description.sponsorshipThis research was funded by Instituto de Salud Carlos III, Spanish Strategy Action for Health (AESI), project TPY1238/15. The author G.S.D. received a research grant from the Spanish Ministry of Education, Culture and Sport, FPU14/03914.es_ES
dc.publisherMultidisciplinary Digital Publishing Institute (MDPI) es_ES
dc.subjectCongenital anomalieses_ES
dc.subjectGeographical analysises_ES
dc.subjectPopulation-based mortalityes_ES
dc.subjectRare diseaseses_ES
dc.subjectTime trendes_ES
dc.subject.meshCongenital Abnormalities es_ES
dc.subject.meshFemale es_ES
dc.subject.meshHumans es_ES
dc.subject.meshMale es_ES
dc.subject.meshMortality es_ES
dc.subject.meshRare Diseases es_ES
dc.subject.meshRegistries es_ES
dc.subject.meshSpain es_ES
dc.subject.meshSpatial Regression es_ES
dc.subject.meshPopulation Surveillance es_ES
dc.titleA Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalieses_ES
dc.typejournal articlees_ES
dc.rights.licenseAtribución 4.0 Internacional*
dc.contributor.funderInstituto de Salud Carlos III 
dc.contributor.funderMinisterio de Educación, Cultura y Deporte (España) 
dc.identifier.journalInternational journal of environmental research and public healthes_ES
dc.repisalud.centroISCIII::Instituto de Investigación de Enfermedades Rarases_ES
dc.rights.accessRightsopen accesses_ES

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