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Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014)
BACKGROUND: The literature suggests that 0.9 to 6% of infants who die unexpectedly may have had a metabolic disorder. At least 43 different inborn errors of metabolism (IEMs) have been associated with sudden death (SUDI). To date, the frequency of IEM-associated SUDI has not been studied in Brazil....
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367785/ https://www.ncbi.nlm.nih.gov/pubmed/30736757 http://dx.doi.org/10.1186/s12887-019-1421-y |
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author | de Bitencourt, F. H. Schwartz, I. V. D. Vianna, F. S. L. |
author_facet | de Bitencourt, F. H. Schwartz, I. V. D. Vianna, F. S. L. |
author_sort | de Bitencourt, F. H. |
collection | PubMed |
description | BACKGROUND: The literature suggests that 0.9 to 6% of infants who die unexpectedly may have had a metabolic disorder. At least 43 different inborn errors of metabolism (IEMs) have been associated with sudden death (SUDI). To date, the frequency of IEM-associated SUDI has not been studied in Brazil. The present study sought to characterize infant mortality related to IEMs known to cause SUDI disaggregated by each of the regions of Brazil. METHODS: This was a descriptive, cross-sectional, population-based study of data obtained from the Brazilian Ministry of Health Mortality Information System (SIM). Death records were obtained for all infants (age < 1 year) who died in Brazil in 2002–2014 in whom the underlying cause of death was listed as ICD-10 codes E70 (Disorders of aromatic amino-acid metabolism), E71 (Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), E72 (Other disorders of amino-acid metabolism), or E74 (Other disorders of carbohydrate metabolism), which are known to be associated with SUDI. RESULTS: From 2002 to 2014, 199 deaths of infants aged < 1 year were recorded in the SIM with an underlying cause corresponding to one of the IEMs of interest. The prevalence of IEM-related deaths was 0.67 per 10,000 live births (0.58–0.77). Of these 199 deaths, 18 (9.0%) occurred in the North of Brazil, 43 (21.6%) in the Northeast, 80 (40.2%) in the Southeast, 46 (23.1%) in the South, and 12 (6.0%) in the Center-West region. Across all regions of the country, ICD10-E74 was predominant. CONCLUSIONS: This 13-year time-series study provides the first analysis of the number of infant deaths in Brazil attributable to IEMs known to be associated with sudden death. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12887-019-1421-y) contains supplementary material, which is available to authorized users. |
format | Online Article Text |
id | pubmed-6367785 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-63677852019-02-15 Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) de Bitencourt, F. H. Schwartz, I. V. D. Vianna, F. S. L. BMC Pediatr Research Article BACKGROUND: The literature suggests that 0.9 to 6% of infants who die unexpectedly may have had a metabolic disorder. At least 43 different inborn errors of metabolism (IEMs) have been associated with sudden death (SUDI). To date, the frequency of IEM-associated SUDI has not been studied in Brazil. The present study sought to characterize infant mortality related to IEMs known to cause SUDI disaggregated by each of the regions of Brazil. METHODS: This was a descriptive, cross-sectional, population-based study of data obtained from the Brazilian Ministry of Health Mortality Information System (SIM). Death records were obtained for all infants (age < 1 year) who died in Brazil in 2002–2014 in whom the underlying cause of death was listed as ICD-10 codes E70 (Disorders of aromatic amino-acid metabolism), E71 (Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), E72 (Other disorders of amino-acid metabolism), or E74 (Other disorders of carbohydrate metabolism), which are known to be associated with SUDI. RESULTS: From 2002 to 2014, 199 deaths of infants aged < 1 year were recorded in the SIM with an underlying cause corresponding to one of the IEMs of interest. The prevalence of IEM-related deaths was 0.67 per 10,000 live births (0.58–0.77). Of these 199 deaths, 18 (9.0%) occurred in the North of Brazil, 43 (21.6%) in the Northeast, 80 (40.2%) in the Southeast, 46 (23.1%) in the South, and 12 (6.0%) in the Center-West region. Across all regions of the country, ICD10-E74 was predominant. CONCLUSIONS: This 13-year time-series study provides the first analysis of the number of infant deaths in Brazil attributable to IEMs known to be associated with sudden death. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12887-019-1421-y) contains supplementary material, which is available to authorized users. BioMed Central 2019-02-08 /pmc/articles/PMC6367785/ /pubmed/30736757 http://dx.doi.org/10.1186/s12887-019-1421-y Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Article de Bitencourt, F. H. Schwartz, I. V. D. Vianna, F. S. L. Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title | Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title_full | Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title_fullStr | Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title_full_unstemmed | Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title_short | Infant mortality in Brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
title_sort | infant mortality in brazil attributable to inborn errors of metabolism associated with sudden death: a time-series study (2002–2014) |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367785/ https://www.ncbi.nlm.nih.gov/pubmed/30736757 http://dx.doi.org/10.1186/s12887-019-1421-y |
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