Semnan University of Medical Sciences

Research and Technology Vice Chancellor

Rezaei, Nima and Pourpak, Zahra and Aghamohammadi, Asghar and Farhoudi, Abolhassan and Movahedi, Masoud and Gharagozlou, Mohammad and MirSaeid Ghazi, Bahram and Atarod, Lida and Abolmaali, Kamran and Mahmoudi, Maryam and Mansouri, Davoud and Arshi, Saba and Javaher Tarash, Naser and Sherkat, Roya and Amin, Reza and Kashe, Sara and Hosseini, Reza Farid and Mohammadzadeh, Iraj and Sadeghi Shabestari, Mehrnaz and Nabavi, Mohammad and Moin, Mostafa (2006) Consanguinity in primary immunodeficiency disorders; the report from Iranian primary immunodeficiency registry. American Journal of Reproductive Immunology. pp. 145-151.

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    PROBLEM: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. METHOD:In this study, the records 515 Iranian PiD patients were reviewed during a 25-year period. RESULTS: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. CONCLUSIONS: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.

    Item Type: Article
    Subjects: R Medicine > R Medicine (General)
    Divisions: Faculty of Medical Sciences > School of Medicine
    Depositing User: Mr Vahab Moshtaghi
    Date Deposited: 13 Feb 2017 07:26
    Last Modified: 13 Feb 2017 07:26

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