Prevalence and mortality in children with congenital diaphragmatic hernia: a multicountry study.
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Politis Maria D, Bermejo-Sánchez Eva, Canfield Mark A, Contiero Paolo, Cragan Janet D, Dastgiri Saeed, de Walle Hermien E K, Feldkamp Marcia L, Nance Amy, Groisman Boris, Gatt Miriam, Benavides-Lara Adriana, Hurtado-Villa Paula, Kallén Kärin, Landau Danielle, Lelong Nathalie, Lopez-Camelo Jorge, Martinez Laura, Morgan Margery, Mutchinick Osvaldo M, Pierini Anna, Rissmann Anke, Šípek Antonin, Szabova Elena, Wertelecki Wladimir, Zarante Ignacio, Bakker Marian K, Kancherla Vijaya, Mastroiacovo Pierpaolo, Nembhard Wendy N, International Clearinghouse for Birth Defects Surveillance and Research
Аннотация
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.