A multi-country study of prevalence and early childhood mortality among children with omphalocele.
mortality
omphalocele
prevalence
registry
surveillance
Journal
Birth defects research
ISSN: 2472-1727
Titre abrégé: Birth Defects Res
Pays: United States
ID NLM: 101701004
Informations de publication
Date de publication:
12 2020
12 2020
Historique:
received:
02
07
2020
revised:
30
09
2020
accepted:
05
10
2020
pubmed:
18
10
2020
medline:
19
8
2021
entrez:
17
10
2020
Statut:
ppublish
Résumé
Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies. We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95% confidence intervals (CI) to calculate cumulative mortality and joinpoint regression for time trend analyses. The prevalence of omphalocele was 2.6 per 10,000 births (95% CI: 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = -0.19%, p = .52). The overall mortality rate was 32.1% (95% CI: 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA. The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period.
Sections du résumé
BACKGROUND
Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies.
METHODS
We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95% confidence intervals (CI) to calculate cumulative mortality and joinpoint regression for time trend analyses.
RESULTS
The prevalence of omphalocele was 2.6 per 10,000 births (95% CI: 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = -0.19%, p = .52). The overall mortality rate was 32.1% (95% CI: 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA.
CONCLUSIONS
The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period.
Identifiants
pubmed: 33067932
doi: 10.1002/bdr2.1822
pmc: PMC7722785
mid: NIHMS1597106
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1787-1801Subventions
Organisme : NCBDD CDC HHS
ID : 5U01DD000491
Pays : United States
Organisme : Instituto de Salud Carlos III
Organisme : Arkansas Biosciences Institute
ID : 037062
Organisme : Czech Ministry of Health
ID : AZV 17-29622A
Organisme : Intramural CDC HHS
ID : CC999999
Pays : United States
Organisme : Ministry of Science and Innovation, of Spain
Organisme : Fundación 1000 sobre Defectos Congénitos, of Spain
Organisme : Direzione Diritti di cittadinanza e coesione sociale-Regione Toscana
Organisme : Dutch Ministry of Welfare, Health and Sports
Organisme : Dutch Ministry of Welfare, Health and Sports.
Organisme : Public Health Wales
Informations de copyright
© 2020 Wiley Periodicals LLC.
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