Unit of Epidemiology of Rare diseases and Congenital anomalies, Institute of Clinical Physiology, National Research Council, Pisa, Italy.
Fondazione Toscana Gabriele Monasterio, Pisa, Italy.
Paediatr Perinat Epidemiol. 2022 Nov;36(6):792-803. doi: 10.1111/ppe.12884. Epub 2022 Jun 8.
Congenital anomalies are a major cause of perinatal, neonatal and infant mortality.
The aim was to investigate temporal changes and geographical variation in survival of children with major congenital anomalies (CA) in different European areas.
In this population-based linkage cohort study, 17 CA registries members of EUROCAT, the European network for the surveillance of CAs, successfully linked data on 115,219 live births with CAs to mortality records. Registries estimated Kaplan-Meier survival at 28 days and 5 years of age and fitted Cox's proportional hazards models comparing mortality at 1 year and 1-9 years of age for children born during 2005-2014 with those born during 1995-2004. The hazard ratios (HR) from each registry were combined centrally using a random-effects model. The 5-year survival conditional on having survived to 28 days of age was calculated.
The overall risk of death by 1 year of age for children born with any major CA in 2005-2014 decreased compared to 1995-2004 (HR 0.68, 95% confidence interval [CI] 0.53, 0.89). Survival at 5 years of age ranged between registries from 97.6% to 87.0%. The lowest survival was observed for the registry of OMNI-Net (Ukraine) (87.0%, 95% CI 86.1, 87.9).
Survival of children with CAs improved for births in 2005-2014 compared with 1995-2004. The use of CA registry data linked to mortality data enables investigation of survival of children with CAs. Factors such as defining major CAs, proportion of terminations of pregnancy for foetal anomaly, source of mortality data and linkage methods are important to consider in the design of future studies and in the interpretation of the results on survival of children with CAs.
先天性畸形是围产儿、新生儿和婴儿死亡的主要原因。
旨在研究不同欧洲地区患有重大先天性异常（CA）的儿童生存率的时间变化和地域差异。
在这项基于人群的联系队列研究中，欧洲先天性异常监测网络 EUROCAT 的 17 个 CA 登记处成功地将 115219 例活产 CA 数据与死亡率记录联系起来。登记处估计了 28 天和 5 岁时的 Kaplan-Meier 生存率，并使用 Cox 比例风险模型比较了 2005-2014 年出生的儿童与 1995-2004 年出生的儿童在 1 岁和 1-9 岁时的死亡率。每个登记处的危险比（HR）使用随机效应模型集中组合。计算了在 28 天龄时存活下来的儿童在 5 岁时的生存条件。
与 1995-2004 年相比，2005-2014 年出生的任何重大 CA 患儿在 1 岁时死亡的总体风险下降（HR 0.68，95%置信区间 [CI] 0.53，0.89）。5 岁时的生存率在登记处之间的范围为 97.6%至 87.0%。OMNI-Net（乌克兰）登记处的生存率最低（87.0%，95%CI 86.1，87.9）。
与 1995-2004 年相比，2005-2014 年出生的 CA 患儿的生存率有所提高。使用与死亡率数据相关联的 CA 登记处数据可以调查 CA 患儿的生存率。在设计未来的研究和解释 CA 患儿生存率的结果时，重要的是要考虑诸如定义重大 CA、因胎儿异常终止妊娠的比例、死亡率数据的来源和联系方法等因素。
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