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希腊的血红蛋白病:过去 35 年的预防计划。

Haemoglobinopathies in Greece: prevention programme over the past 35 years.

机构信息

University of Athens & Biomedical Research Foundation of the Academy of Athens, Athens, Greece.

出版信息

Indian J Med Res. 2011 Oct;134(4):572-6.

Abstract

At present, prevention of thalassaemia and sickle cell disease is the only realistic approach to control the birth of new patients in countries having high numbers of carriers. This is fully justified because avoiding the birth of an ever increasing number of patients may allow a more effective use of the available resources in improving the management of the patients surviving today and alleviate the already overloaded public health system from the inevitable tremendous and ever increasing cost. Moreover, prenatal diagnosis may help couples at risk to have non-thalassaemic children. Greece is one of the countries where the mean frequency of carriers is approximately 7.5 per cent (population 11 million) and has set up a nationwide programme for carrier identification in the early seventies; this is provided through a dozen of specific Units attached to the major Blood Transfusion Services of the country, on a voluntary basis and free of charge. Spread of information through mass media, the schools, and other groups has greatly contributed in creating the necessary sensitization; obstetricians and antenatal Clinics are also instrumental to this effect. Prenatal diagnosis is offered centrally (Athens) and covers satisfactorily the estimated needs (500-600 annually); the total number has already exceeded 35,000. According to information obtained from the major paediatric hospitals all over the country, the number of thalassaemia major or SCD admitted for treatment over the last ten years has been around 15 yearly (instead of an estimate of 120-130).

摘要

目前,预防地中海贫血症和镰状细胞病是控制高携带率国家新患者出生的唯一现实途径。这是完全合理的,因为避免越来越多的患者出生,可以更有效地利用现有资源,改善今天幸存患者的管理,并减轻已经不堪重负的公共卫生系统因不可避免的巨大和不断增加的成本而带来的负担。此外,产前诊断可以帮助有风险的夫妇生育非地中海贫血症的孩子。希腊是携带者平均频率约为 7.5%(人口 1100 万)的国家之一,早在 70 年代就建立了全国范围内的携带者识别计划;这是通过该国十几个主要输血服务机构的十几个特定单位自愿和免费提供的。通过大众媒体、学校和其他团体传播信息,极大地促进了必要的宣传;妇产科医生和产前诊所也为此做出了贡献。产前诊断是在雅典进行的,并且能够很好地满足估计的需求(每年 500-600 例);总数已经超过 35000 例。根据从全国各地主要儿科医院获得的信息,过去十年中因治疗而入院的重型地中海贫血症或镰状细胞病患者每年约有 15 例(而不是估计的 120-130 例)。

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