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婚前地中海贫血预防:伊斯法罕10万例病例经验报告。

Pre-marriage prevention of thalassaemia: report of a 100,000 case experience in Isfahan.

作者信息

Ghanei M, Adibi P, Movahedi M, Khami M A, Ghasemi R L, Azarm T, Zolfaghari B, Jamshidi H R, Sadri R

机构信息

Isfahan University of Medical Sciences and Health Services, Iran.

出版信息

Public Health. 1997 May;111(3):153-6. doi: 10.1016/s0033-3506(97)00574-x.

Abstract

BACKGROUND

Iran like other middle east countries has a large number of major thalassaemics. Due to religious restrictions on abortion, the routine prevention of the birth of thalassaemic children by this means is not possible. The aim of this study is to describe an alternative means to prevent the birth of thalassaemic children.

METHODS

From January 1993 to January 1996, 10,000 people preparing for marriage were screened for the thalassaemia trait, using CBC and HbA2 level measurement. High risk couples were referred for further consultation regarding the disease and the means of its prevention. The proposed actions of the couples regarding thalassaemia prevention were evaluated immediately after consultation and then re-evaluated three months later.

RESULT

After the project had been running for three years the average of high risk couple initially deciding not to marry was 90% and no new cases of thalassemia were detected in the children of the screened population.

CONCLUSION

Where both members of the couple were trait-positive their preferred choice was not to marry, rather than to marry and use other or no methods of preventing a thalassemia affected child being born to them. Cultural and religious ideas can affect such decisions and in some Islamic countries the establishment and use of a genetic counselling centre can help prevent most of new thalassaemia cases.

摘要

背景

伊朗与其他中东国家一样,有大量重型地中海贫血患者。由于宗教对堕胎的限制,通过这种方式常规预防地中海贫血患儿的出生是不可能的。本研究的目的是描述一种预防地中海贫血患儿出生的替代方法。

方法

从1993年1月至1996年1月,对10000名准备结婚的人进行地中海贫血特征筛查,采用全血细胞计数和HbA2水平测量。高危夫妇被转介进行关于该疾病及其预防方法的进一步咨询。夫妇双方关于地中海贫血预防的建议行动在咨询后立即进行评估,然后在三个月后重新评估。

结果

该项目运行三年后,最初决定不结婚的高危夫妇平均比例为90%,在筛查人群的子女中未检测到新的地中海贫血病例。

结论

当夫妇双方均为特征阳性时,他们更倾向的选择是不结婚,而不是结婚并采用其他或不采用任何方法来防止他们生育受地中海贫血影响的孩子。文化和宗教观念会影响此类决定,在一些伊斯兰国家,建立和使用遗传咨询中心有助于预防大多数新的地中海贫血病例。

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