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Consanguinity in Primary Immunodeficiency Disorders; the Report from Iranian Primary Immunodeficiency Registry

Authors

  • Nima Rezaei,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Zahra Pourpak,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Asghar Aghamohammadi,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Abolhassan Farhoudi,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Masoud Movahedi,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Mohammad Gharagozlou,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Bahram MirSaeid Ghazi,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Lida Atarod,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Kamran Abolmaali,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Maryam Mahmoudi,

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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  • Davoud Mansouri,

    1. Division of Infectious Diseases and Clinical Immunology, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran;
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  • Saba Arshi,

    1. Department of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran;
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  • Naser Javaher Tarash,

    1. Department of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran;
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  • Roya Sherkat,

    1. Al-Zahra Hospital, Isfahan University of Medical Sciences, Tehran, Iran;
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  • Reza Amin,

    1. Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Tehran, Iran;
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  • Sara Kashef,

    1. Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Tehran, Iran;
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  • Reza Farid Hosseini,

    1. Department of Immunology and Allergy, Mashhad University of Medical Sciences, Tehran, Iran;
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  • Iraj Mohammadzadeh,

    1. Amirkola Hospital, Babol University of Medical Sciences, Tehran, Iran;
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  • Mehrnaz Sadeghi Shabestari,

    1. Tabriz University of Medical Sciences, Tehran, Iran;
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  • Mohammad Nabavi,

    1. Semnan University of Medical Sciences, Tehran, Iran
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  • Mostafa Moin

    1. Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;
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Zahra Pourpak, Immunology, Asthma and Allergy Research Institute, Children Medical Center, No. 62, Dr Gharib St, Keshavarz Blvd, PO Box 14185-863, Tehran 14194, Iran.
E-mail: rezaei_nima@hbi.ir; zpourpak@hbi.ir

Abstract

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, seletive 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.

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