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摩洛哥北部的血红蛋白病:近亲婚配的初步研究。

Hemoglobinopathies in the North of Morocco: Consanguinity Pilot Study.

机构信息

Biomedical Genomics and Oncogenetics Research Laboratory, Faculty of Sciences and Techniques of Tangier, University Abdelmalek Essaâdi, Tangier 90000, Morocco.

出版信息

Biomed Res Int. 2019 Sep 26;2019:6857417. doi: 10.1155/2019/6857417. eCollection 2019.

Abstract

Consanguinity is a social behavior characterized by the arrangement of marriages between relatives. It coincides generally with the geographic distribution of recessive genetic diseases as it increases the likelihood of homozygosis and, consequently, the incidence of their pathologies in the population. In this pilot study, we assess the effect of inbreeding on the burden of hemoglobinopathies in Northern Morocco. From January 2016 to December 2018, 197 children born in the studied region to three ancestral generations and diagnosed with hemoglobinopathies were subject to investigation. The rate of consanguinity in the parents' generation of children with hemoglobinopathies was 50.25%, with first cousin marriages accounting for 68.69% of consanguineous unions (FI = 0.02). The corresponding rates in the general population, based on a sample of  = 900, were 29.67% and 82.02%, respectively. The marriages between first cousins are the most common among the other types of consanguineous unions. Our study propounds that consanguinity substantially contributes to the hemoglobinopathy burden in the studied region and has changed little over time. Refraining from consanguineous marriages and detecting couples at risk could contribute to the reduction of the incidence of genetic diseases in our country.

摘要

血缘关系是一种以亲属间婚姻安排为特征的社会行为。它通常与隐性遗传病的地理分布相吻合,因为它增加了同型合子的可能性,从而增加了其在人群中发病的可能性。在这项初步研究中,我们评估了近亲繁殖对摩洛哥北部血红蛋白病负担的影响。从 2016 年 1 月至 2018 年 12 月,对在研究区域出生并被诊断患有血红蛋白病的 197 名三代子女进行了调查。患有血红蛋白病的儿童父母一代的近亲结婚率为 50.25%,其中表亲婚姻占近亲结婚的 68.69%(FI=0.02)。根据 900 名样本的一般人群的相应比率分别为 29.67%和 82.02%。表亲之间的婚姻是其他类型的近亲婚姻中最常见的。我们的研究表明,近亲结婚在研究地区对血红蛋白病负担有很大影响,而且随着时间的推移变化不大。避免表亲婚姻和检测有风险的夫妇可以有助于减少我国遗传疾病的发病率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9cef/6791198/d6775a48b36b/BMRI2019-6857417.001.jpg

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