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[拉齐奥地区地中海贫血的预防:伊达·比安科档案]

[Prevention of mediterranean anemia in Latium: Ida Bianco archives].

作者信息

Falchetti Mario, Lupi Ramona, Ottini Laura

机构信息

Dipartimento di Medicina Sperimentale, Università "La Sapienza", Roma, I.

出版信息

Med Secoli. 2006;18(1):241-61.

Abstract

Mediterranean anemia or beta-thalassemia is a hereditary syndrome characterized by a severe defect in haemoglobin production and an altered morphology of red blood cells. Homozygous condition for beta-thalassemia is characterized by short survival. Heterozygous condition is clinically found in adolescence and is characterized by a less aggressive phenotype. Ida Bianco, with her husband Ezio Silvestroni, has conducted a long struggle for beta-thalassemia prevention in Italy. They were the first to draw up an accurate map of the distribution of thalassemia in Italy and to conceive and implement a campaign against this genetic disease by the development of annual screening on at-school teenagers and pre-marriage prevention. Here we focused on the analysis of Ida Bianco's archives concerning screenings conducted on middle-schools in the Latium by the "Centro Studi della Microcitemia" of Rome from 1975 up to today. The results of the thirty-years prevention work in the Latium will be described.

摘要

地中海贫血或β地中海贫血是一种遗传性综合征,其特征是血红蛋白生成严重缺陷和红细胞形态改变。β地中海贫血的纯合子状态特征为生存期短。杂合子状态在临床上于青春期发现,其特征为表型侵袭性较小。伊达·比安科与她的丈夫埃齐奥·西尔韦斯特罗尼在意大利为预防β地中海贫血进行了长期斗争。他们率先绘制了意大利地中海贫血分布的精确地图,并通过对在校青少年进行年度筛查和婚前预防来构思和开展一场对抗这种遗传病的运动。在此,我们重点分析了伊达·比安科的档案,这些档案涉及罗马“小红细胞贫血症研究中心”自1975年至今在拉齐奥大区的中学所进行的筛查。将描述在拉齐奥大区三十年预防工作的成果。

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