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A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies

This 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...

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Autores principales: Alonso-Ferreira, Verónica, Sánchez-Díaz, Germán, Villaverde-Hueso, Ana, Posada de la Paz, Manuel, Bermejo-Sánchez, Eva
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6121521/
https://www.ncbi.nlm.nih.gov/pubmed/30103420
http://dx.doi.org/10.3390/ijerph15081715
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author Alonso-Ferreira, Verónica
Sánchez-Díaz, Germán
Villaverde-Hueso, Ana
Posada de la Paz, Manuel
Bermejo-Sánchez, Eva
author_facet Alonso-Ferreira, Verónica
Sánchez-Díaz, Germán
Villaverde-Hueso, Ana
Posada de la Paz, Manuel
Bermejo-Sánchez, Eva
author_sort Alonso-Ferreira, Verónica
collection PubMed
description This 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.
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spelling pubmed-61215212018-09-07 A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies Alonso-Ferreira, Verónica Sánchez-Díaz, Germán Villaverde-Hueso, Ana Posada de la Paz, Manuel Bermejo-Sánchez, Eva Int J Environ Res Public Health Article This 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. MDPI 2018-08-10 2018-08 /pmc/articles/PMC6121521/ /pubmed/30103420 http://dx.doi.org/10.3390/ijerph15081715 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Alonso-Ferreira, Verónica
Sánchez-Díaz, Germán
Villaverde-Hueso, Ana
Posada de la Paz, Manuel
Bermejo-Sánchez, Eva
A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title_full A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title_fullStr A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title_full_unstemmed A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title_short A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies
title_sort nationwide registry-based study on mortality due to rare congenital anomalies
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6121521/
https://www.ncbi.nlm.nih.gov/pubmed/30103420
http://dx.doi.org/10.3390/ijerph15081715
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