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乌拉圭的伦理、遗传学与公共政策:以新生儿和婴儿筛查为例

Ethics, genetics and public policies in Uruguay: newborn and infant screening as a paradigm.

作者信息

Larrandaburu Mariela, Matte Ursula, Noble Ana, Olivera Zully, Sanseverino Maria Teresa V, Nacul Luis, Schuler-Faccini Lavinia

机构信息

Ministry of Public Health of Uruguay, 18 de Julio 1892, 11200, Montevideo, Uruguay,

出版信息

J Community Genet. 2015 Jul;6(3):241-9. doi: 10.1007/s12687-015-0236-2. Epub 2015 May 29.

Abstract

Uruguay is a middle-income country and the smallest in South America. Its population is under 3.3 million. The demographic and epidemiological characteristics are similar to those of developed countries, with a high burden associated with congenital anomalies. Infant mortality rate (IMR) decreased from 37/1000 live births, in 1980, to 8.8/1000, in 2013. This is largely explained by medical and social policies. IMR related to congenital anomalies, however, remained unchanged for the last 30 years. Therefore, programmes for prevention of congenital disorders were developed, such as the National Newborn Screening Programme. Mandatory, universal, free infant screening was implemented two decades ago. The Ministry of Public Health created the Comprehensive Plan on Birth Defects and Rare Diseases (PIDCER), to develop a strategic public policy tool enabling comprehensive, universal, quality care during their entire lifetime. Recent national legislation created provisions for newborn and infant screening, including for congenital hypothyroidism, phenylketonuria, congenital adrenal hyperplasia, cystic fibrosis and medium-chain acyl-CoA dehydrogenase, via blood spot test, otoacoustic emissions, systematic physical examination and hip ultrasound. We discuss how this programme was implemented, the current situation of rare diseases, the institution managing disability in Uruguay and the development of new laws based on the MPH's PIDCER. It illustrates how Uruguay is developing public policies in the genomic era, based both on science and bioethics.

摘要

乌拉圭是一个中等收入国家,也是南美洲最小的国家。其人口不到330万。人口统计学和流行病学特征与发达国家相似,先天性异常负担较高。婴儿死亡率从1980年的每1000例活产37例降至2013年的每1000例8.8例。这在很大程度上归因于医疗和社会政策。然而,与先天性异常相关的婴儿死亡率在过去30年中保持不变。因此,制定了先天性疾病预防计划,如国家新生儿筛查计划。二十年前实施了强制性、普遍性、免费的婴儿筛查。公共卫生部制定了出生缺陷和罕见病综合计划(PIDCER),以制定一项战略性公共政策工具,在其整个生命周期内提供全面、普遍、高质量的护理。最近的国家立法规定了新生儿和婴儿筛查,包括通过血斑试验、耳声发射、系统体格检查和髋关节超声对先天性甲状腺功能减退症、苯丙酮尿症、先天性肾上腺皮质增生症、囊性纤维化和中链酰基辅酶A脱氢酶进行筛查。我们讨论了该计划的实施情况、罕见病的现状、乌拉圭管理残疾的机构以及基于公共卫生部的PIDCER制定的新法律。它说明了乌拉圭如何在基因组时代基于科学和生物伦理制定公共政策。

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