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引用次数: 0
摘要
新生儿筛查是一项重大的公共卫生问题。法国于 1972 年建立了新生儿筛查制度,对苯丙酮尿症进行系统筛查。随后,又增加了其他筛查项目,包括先天性甲状腺功能减退症、先天性肾上腺皮质增生症、囊性纤维化和镰状细胞病。2020 年,筛查实验室引入串联质谱技术,新增了八种遗传代谢病:氨基酸病(酪氨酸血症 I 型、枫糖尿病和高胱氨酸尿症)、有机酸尿症(异戊酸和戊二酸 I 型酸尿症)和脂肪酸代谢紊乱(MCADD、长链 3- 羟基乙酰-CoA 脱氢酶缺乏症(LCHADD)和原发性肉碱缺乏症)。我们将简要介绍这些新增加的疾病,公众对这些疾病的认识尚不全面。
[Newborn screening in France: news and perspectives].
Newborn screening is a major public health concern. In France, it was established in 1972 with systematic screening for phenylketonuria. Subsequently, other screenings, including congenital hypothyroidism, congenital adrenal hyperplasia, cystic fibrosis, and sickle cell disease, were added. The introduction of tandem mass spectrometry in screening laboratories in 2020 enabled the inclusion of eight additional inherited metabolic diseases: aminoacidopathies (tyrosinemia type I, maple syrup urine disease, and homocystinuria), organic acidurias (isovaleric and glutaric type I acidurias), and disorders of fatty acid metabolism (MCADD, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD), and primary carnitine deficiency). We briefly present these newly added diseases, of which public awareness is still incomplete.