Rezaei Nima, Pourpak Zahra, Aghamohammadi Asghar, Farhoudi Abolhassan, Movahedi Masoud, Gharagozlou Mohammad, Mirsaeid Ghazi Bahram, Atarod Lida, Abolmaali Kamran, Mahmoudi Maryam, Mansouri Davoud, Arshi Saba, Tarash Naser Javaher, Sherkat Roya, Amin Reza, Kashef Sara, Hosseini Reza Farid, Mohammadzadeh Iraj, Shabestari Mehrnaz Sadeghi, Nabavi Mohammad, Moin Mostafa
Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Iran.
Am J Reprod Immunol. 2006 Aug;56(2):145-51. doi: 10.1111/j.1600-0897.2006.00409.x.
Primary Immunodeficiency Disorders (PiD) are a heterogeneous group of genetic disorders, with different modes of inheritance. This study was accomplished in order to determine the frequency of consanguineous marriages in the families of patients with PiD.
In this study, the records 515 Iranian PiD patients were reviewed during a 25-year period.
The mean proportion of consanguineous marriages was 65.6% among PiD patients, while the overall rate was 38.6% in the country. The rate of consanguinity was 77.8% in cellular immunodeficiencies, 75.8% in combined immunodeficiencies, 72.5% in defects of phagocytic function, 58.6% in other immunodefiiencies, 54.1% in predominantly antibody deficiencies, and 50% in complement deficiencies. Moreover all patients with immunodeficiency associated with other diseases had consanguineous parents. Such marriages were most common in the parents of patients with Chediak-Higashi syndrome, severe combined immunodeficiencies, primary CD4 deficiency, ataxia-telangiectasia, selective IgG class deficiencies, chronic granulomatous disease, and Schwachman syndrome.
It is important to inform the general population about the dangers of consanguinity, which is very common in some areas such as Iran. Premarital examination to avoid genetic diseases could be suggested, especially in a community where the rate of consanguineous marriage is high.
原发性免疫缺陷病（PiD）是一组具有不同遗传方式的遗传性疾病。本研究旨在确定PiD患者家庭中近亲结婚的频率。
在本研究中，回顾了25年间515例伊朗PiD患者的记录。
PiD患者中近亲结婚的平均比例为65.6%，而该国的总体比例为38.6%。细胞免疫缺陷患者的近亲结婚率为77.8%，联合免疫缺陷患者为75.8%，吞噬功能缺陷患者为72.5%，其他免疫缺陷患者为58.6%，主要抗体缺陷患者为54.1%，补体缺陷患者为50%。此外，所有与其他疾病相关的免疫缺陷患者的父母均为近亲结婚。这种婚姻在患有切-东综合征、严重联合免疫缺陷、原发性CD4缺乏、共济失调-毛细血管扩张症、选择性IgG类缺陷、慢性肉芽肿病和施瓦茨曼综合征患者的父母中最为常见。
向公众宣传近亲结婚的危害非常重要，近亲结婚在伊朗等一些地区非常普遍。建议进行婚前检查以避免遗传疾病，尤其是在近亲结婚率较高的社区。
