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Journal Articles Annals of Epidemiology Year : 2021

Prevalence and mortality in children with congenital diaphragmatic hernia: a multicountry study

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Mark Canfield
  • Function : Author
Amy Nance
  • Function : Author
Boris Groisman
  • Function : Author
Miriam Gatt
  • Function : Author
Adriana Benavides-Lara
  • Function : Author
Kärin Kallén
  • Function : Author
Danielle Landau
  • Function : Author
Jorge Lopez-Camelo
  • Function : Author
Margery Morgan
  • Function : Author
Osvaldo Mutchinick
  • Function : Author
Anna Pierini
  • Function : Author
Antonin Šípek
  • Function : Author
Wladimir Wertelecki
  • Function : Author
Pierpaolo Mastroiacovo
  • Function : Author

Abstract

Purpose: This study determined the prevalence, mortality, and time trends of children with congenital diaphragmatic hernia (CDH). Methods: Twenty-five hospital- and population-based surveillance programs in 19 International Clearinghouse for Birth Defects Surveillance and Research member countries provided birth defects mortality data between 1974 and 2015. CDH cases included live births, stillbirths, or elective termination of pregnancy for fetal anomalies. Prevalence, cumulative mortality rates, and 95% confidence intervals (CIs) were calculated using Poisson regression and a Kaplan-Meier product-limit method. Joinpoint regression analyses were conducted to assess time trends. Results: The prevalence of CDH was 2.6 per 10,000 total births (95% CI: 2.5-2.7), slightly increasing between 2001 and 2012 (average annual percent change = 0.5%; 95% CI:-0.6 to 1.6). The total percent mortality of CDH was 37.7%, with hospital-based registries having more deaths among live births than population-based registries (45.1% vs. 33.8%). Mortality rates decreased over time (average annual percent change = -2.4%; 95% CI: -3.8 to 1.1). Most deaths due to CDH occurred among 2- to 6-day-old infants for both registry types (36.3%, hospital-based; 12.1%, population-based). Conclusions: The mortality of CDH has decreased over time. Mortality remains high during the first week and varied by registry type.

Dates and versions

hal-03257883 , version 1 (11-06-2021)

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Cite

Maria Politis, Eva Bermejo-Sánchez, Mark Canfield, Paolo Contiero, Janet Cragan, et al.. Prevalence and mortality in children with congenital diaphragmatic hernia: a multicountry study. Annals of Epidemiology, 2021, 56, pp.61-69.e3. ⟨10.1016/j.annepidem.2020.11.007⟩. ⟨hal-03257883⟩
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