Abstract：
To investigate the survival to 10 years of age of children with trisomy 13 (T13) and children with trisomy 18 (T18), born 1995-2014.
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.
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.
摘要：
目的：调查13三体（T13）儿童和18三体（T18）儿童的10岁生存率。出生于1995-2014年。
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。
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。
