Baird P A, Sadovnick A D
J Pediatr. 1987 Jun;110(6):849-54. doi: 10.1016/s0022-3476(87)80395-5.
This study is part of a collaborative effort between the Division of Vital Statistics, Ministry of Health, Province of British Columbia and the Department of Medical Genetics, University of British Columbia. Permission to use the provincial vital and health records was conditional on the strict observance of the oath of secrecy regarding the nonstatistical information contained in the records. To develop life expectancy data for Down syndrome, we studied 1341 patients with Down syndrome identified by the British Columbia Health Surveillance Registry from more than one million consecutive live births from 1952-1981 inclusive. Our results indicate that life expectancy is much better than generally believed. For patients with Down syndrome with congenital heart anomalies, survival to age 1 year is 76.3%; to age 5, 61.8%; to age 10, 57.1%; to age 20, 53.1%; and to age 30, 49.9%. For patients with Down syndrome without congenital heart anomalies, survival to the same ages is 90.7%; 87.2%, 84.9%, 81.9%, and 79.2%, respectively. Survival for these patients without congenital heart defects is still significantly lower than that for a comparison group of mentally retarded persons without congenital heart defects.
本研究是不列颠哥伦比亚省卫生部生命统计司与英属哥伦比亚大学医学遗传学系合作项目的一部分。使用该省生命与健康记录的许可条件是严格遵守对记录中所含非统计信息保密的誓言。为了得出唐氏综合征患者的预期寿命数据，我们研究了不列颠哥伦比亚省健康监测登记处从1952年至1981年（含）超过100万例连续活产中确定的1341例唐氏综合征患者。我们的结果表明，预期寿命比普遍认为的要好得多。对于患有先天性心脏异常的唐氏综合征患者，1岁时的生存率为76.3%；5岁时为61.8%；10岁时为57.1%；20岁时为53.1%；30岁时为49.9%。对于没有先天性心脏异常的唐氏综合征患者，到相同年龄的生存率分别为90.7%、87.2%、84.9%、81.9%和79.2%。这些没有先天性心脏缺陷的患者的生存率仍显著低于没有先天性心脏缺陷的智障对照人群。
