Population-based cohort study that linked mortality data to data on children born with T13 or T18, including translocations and mosaicisms, from 13 member registries of EUROCAT, a European network for the surveillance of congenital anomalies.
13 regions in nine Western European countries.
252 live births with T13 and 602 with T18.
Survival at 1 week, 4 weeks and 1, 5 and 10 years of age estimated by random-effects meta-analyses of registry-specific Kaplan-Meier survival estimates.
Survival estimates of children with T13 were 34% (95% CI 26% to 46%), 17% (95% CI 11% to 29%) and 11% (95% CI 6% to 18%) at 4 weeks, 1 and 10 years, respectively. The corresponding survival estimates were 38% (95% CI 31% to 45%), 13% (95% CI 10% to 17%) and 8% (95% CI 5% to 13%) for children with T18. The 10-year survival conditional on surviving to 4 weeks was 32% (95% CI 23% to 41%) and 21% (95% CI 15% to 28%) for children with T13 and T18, respectively.
This multi-registry European study found that despite extremely high neonatal mortality in children with T13 and T18, 32% and 21%, respectively, of those who survived to 4 weeks were likely to survive to age 10 years. These reliable survival estimates are useful to inform counselling of parents after prenatal diagnosis.
方法:基于人群的队列研究,将死亡率数据与T13或T18出生儿童的数据联系起来,包括易位和镶嵌,来自欧洲CAT的13个成员注册管理机构,欧洲先天性异常监测网络。
方法:9个西欧国家的13个地区。
方法:252例T13的活产和602例T18的活产。
方法:1周生存率,4周和1、5和10岁的年龄通过随机效应荟萃分析的注册特异性Kaplan-Meier生存估计估计。
结果:T13儿童的生存率估计为34%(95%CI26%至46%),4周时分别为17%(95%CI11%至29%)和11%(95%CI6%至18%),1年和10年,分别。相应的生存率估计为38%(95%CI31%至45%),T18患儿为13%(95%CI10%至17%)和8%(95%CI5%至13%)。对于T13和T18患儿,以存活至4周为条件的10年生存率分别为32%(95%CI23%至41%)和21%(95%CI15%至28%)。
结论:这项欧洲多注册研究发现,尽管T13和T18儿童的新生儿死亡率极高,分别为32%和21%,分别,那些存活到4周的人可能存活到10岁。这些可靠的生存估计有助于在产前诊断后为父母提供咨询。