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先天性畸形和孟德尔疾病对以色列婴儿死亡率的影响。

The impact of congenital malformations and Mendelian diseases on infant mortality in Israel.

作者信息

Zlotogora Joël, Leventhal Alex, Amitai Yona

机构信息

Public Health Services, Ministry of Health, Tel Hashomer, Israel.

出版信息

Isr Med Assoc J. 2003 Jun;5(6):416-8.

Abstract

BACKGROUND

Infant mortality in Israel is twofold higher among non-Jews than Jews.

OBJECTIVES

To determine the impact of congenital malformations and Mendelian diseases on infant mortality.

METHODS

We compared the causes of infant mortality in a 4 year period among Jewish and non-Jewish Israeli citizens. Classification was done by analyzing all the death reports according to whether or not the child had any known major malformation, Mendelian disease and/or a syndrome, irrespective of the immediate cause of death.

RESULTS

The infant mortality among non-Jews was double that among Jews (9 versus 4.4 per 1,000 live births). The rate of children with malformations/genetic syndromes was 3.1 times higher among non-Jews than among Jews (2.94 vs. 1.25 per 1,000 live births). The most significant difference was in the rate of Mendelian diseases, which were 8.3 times more frequent in non-Jewish children (0.16 vs. 1.33 per 1,000 live births respectively). A Mendelian disease was diagnosed in almost 15% of the non-Jewish infants and in less than 5% of the Jewish infants.

CONCLUSIONS

The most striking difference between the Jewish and non-Jewish infants was the incidence of congenital malformations and Mendelian diseases parallel to the differences in the consanguinity rates between the two populations.

摘要

背景

以色列非犹太人群的婴儿死亡率比犹太人群高出两倍。

目的

确定先天性畸形和孟德尔疾病对婴儿死亡率的影响。

方法

我们比较了4年期间以色列犹太和非犹太公民的婴儿死亡原因。分类是通过分析所有死亡报告来进行的,依据是孩子是否患有任何已知的严重畸形、孟德尔疾病和/或综合征,而不考虑直接死因。

结果

非犹太人的婴儿死亡率是犹太人的两倍(每1000例活产中分别为9例和4.4例)。非犹太人中患有畸形/遗传综合征的儿童比例比犹太人高3.1倍(每1000例活产中分别为2.94例和1.25例)。最显著的差异在于孟德尔疾病的发病率,非犹太儿童中的发病率比犹太儿童高8.3倍(每1000例活产中分别为0.16例和1.33例)。近15%的非犹太婴儿被诊断患有孟德尔疾病,而犹太婴儿中这一比例不到5%。

结论

犹太和非犹太婴儿之间最显著的差异在于先天性畸形和孟德尔疾病的发病率,这与两个人群近亲结婚率的差异相一致。

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