Improved Survival in Down Syndrome over the Last 60 Years and the Impact of Perinatal Factors in Recent Decades
Improved Survival in Down Syndrome over the Last 60 Years and the Impact of Perinatal Factors in Recent Decades
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DOI：
10.1016/j.jpeds.2015.10.083
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发表时间：
2016-02-01
影响因子：
5.1
通讯作者：
Leonard, Helen
中科院分区：
文献类型：
--
作者：
Glasson, Emma J.;Jacques, Angela;Leonard, Helen
目的利用关联人群数据计算近60年来唐氏综合征患者的生存率及主要围生期因素的影响。研究设计（发育异常和智力障碍探索答案登记数据库）用于识别1980年以来西澳772名出生时患有唐氏综合症的儿童2010.围产期和死亡率数据提取自西澳大利亚州助产士信息系统和西澳大利亚州死亡登记，并与同一时期出生的其余西澳大利亚州人口进行比较。1980年之前居住在西澳的另外606名唐氏综合征儿童可从残疾服务数据库中获得，并用于预测成年后的存活率。（95% CI 86%，90%）存活至5岁，87%（95% CI 85%，89%）存活至10年，83%（95% CI 80%，85%）存活至30年。活产唐氏综合征患儿的母亲年龄大于35岁（32.7% vs 13.4%）、胎龄小于37周（23.8% vs 7.9%）、剖腹产（28.9% vs 23.0%）和出生体重小于2500 g（20.4% vs 6.1%）的可能性更大（均P > 0.001）。唐氏综合征生存率降低的存在下，心血管缺陷，胎龄较低，低出生体重，或早出生years.Conclusions改善生存的唐氏综合征患儿在过去60年来已经发生递增，但差异仍然存在早产或低出生体重的儿童。
Objective To calculate the survival of people with Down syndrome over the past 60 years and the influence of major perinatal factors by using linked population-based data.Study design A data linkage between 2 Western Australian (WA) data sets (the Register for Developmental Anomalies and the Intellectual Disability Exploring Answers database) was used to identify 772 children born with Down syndrome in WA from 1980-2010. Perinatal and mortality data were extracted from the WA Midwives Information System and WA death registrations and compared with the remaining WA population born during that same era. An additional 606 children with Down syndrome living in WA prior to 1980 were available from a disability services database and were used for predicting survival into adulthood.Results Overall, for cases born 1953-2010, 88% (95% CI 86%, 90%) survived to 5 years of age, 87% (95% CI 85%, 89%) to 10 years, and 83% (95% CI 80%, 85%) to 30 years. Children live-born with Down syndrome were significantly more likely (all P > .001) to have mothers older than 35 years (32.7% vs 13.4%), a gestational age less than 37 weeks (23.8% vs 7.9%), a cesarean delivery (28.9% vs 23.0%), and a birth weight less than 2500 g (20.4% vs 6.1%). Down syndrome survival was reduced in the presence of a cardiovascular defect, younger gestational age, low birth weight, or earlier birth years.Conclusions Improved survival for children born with Down syndrome over the last 60 years has occurred incrementally, but disparities still exist for children who are preterm or have low birth weight.