Frequency and Clinical Manifestations of Patients with Primary Immunodeficiency Disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry

Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
Journal of Clinical Immunology (Impact Factor: 2.65). 11/2006; 26(6):519-32. DOI: 10.1007/s10875-006-9047-x
Source: PubMed

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.

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Available from: MOHAMMAD Nabavi, Jul 22, 2014
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    • "Common variable immunodeficiency (CVID) is the most symptomatic primary immunodeficiency characterized by defective immunoglobulin and specific antibody production by B lymphocytes [1] [2] [3] [4] [5] [6] [7]. As a result of this failure, recurrent infections particularly with encapsulated bacteria in respiratory tract are often seen in CVID patients [1] [8] [9]. The antibody response is affected at both early and late stages of B cell maturation including up-regulation of CD70 and CD86 in naïve B cells, somatic hypermutation and antibody affinity maturation [1] [10]. "
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