Semnan University of Medical Sciences

Research and Technology Vice Chancellor

REZAEI, NIMA and AGHAMOHAMMADI, ASGHAR and Moin, Mostafa and Pourpak, Zahra and Movahedi, Masoud and Gharagozlou, Mohammad and ATAROD, LIDA and MIRSAEID GHAZI, BAHRAM and ISAEIAN, ANNA and MAHMOUDI, MARYAM and ABOLMAALI, KAMRAN and MANSOURI, DAVOUD and ARSHI, SABA and JAVAHER TARASH, NASER and SHERKAT, ROYA and AKBARI, HEDAYAT and AMIN, REZA and ALBORZI, ABDOLVAHAB and KASHEF, SARA and FARID, REZA and MOHAMMADZADEH, IRAJ and SADEGHI SHABESTARI, MEHRNAZ and NABAVI, MOHAMMAD and FARHOUDI, ABOLHASSAN (2006) Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: Update from the Iranian primary immunodeficiency registry. Journal of Clinical Immunology. pp. 519-532.

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    Abstract

    Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections. A total of 930 patients (573 males and 357 females) are registered in Iranian PID Registry (IPIDR) during three decades. Predominantly antibody deficiencies were the most common (38.4%), followed by congenital defects of phagocyte number and/or function (28.3%), other well-defined immunodeficiency syndromes (17.7%), combined T- and B-cell immunodeficiencies (11.0%), complement deficiencies (2.4%), and diseases of immune dysregulation (2.3%). Common variable immunodeficiency was the most frequent disorder (20.8%), followed by chronic granulomatous disease, ataxia-telangiectasia, btk deficiency, selective IgA deficiency, and T-B-severe combined immunodeficiency. The frequency of other PID disorders was less than 50 in number (<5%). There is an increasing trend in recognition of more PID in the recent years. Construction of such registry is not only important for its epidemiological aspect but also for its role in increasing the physician's knowledge about such disorders.

    Item Type: Article
    Subjects: R Medicine > R Medicine (General)
    Divisions: Faculty of Medical Sciences > School of Medicine
    Depositing User: Mr Vahab Moshtaghi
    Date Deposited: 11 Feb 2017 10:59
    Last Modified: 11 Feb 2017 10:59
    URI: http://eprints.semums.ac.ir/id/eprint/834

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