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Frequency and Clinical Manifestations of Patients with Primary Immunodeficiency Disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry
Authors:NIMA REZAEI  ASGHAR AGHAMOHAMMADI  MOSTAFA MOIN  ZAHRA POURPAK  MASOUD MOVAHEDI  MOHAMMAD GHARAGOZLOU  LIDA ATAROD  BAHRAM MIRSAEID GHAZI  ANNA ISAEIAN  MARYAM MAHMOUDI  KAMRAN ABOLMAALI  DAVOUD MANSOURI  SABA ARSHI  NASER JAVAHER TARASH  ROYA SHERKAT  HEDAYAT AKBARI  REZA AMIN  ABDOLVAHAB ALBORZI  SARA KASHEF  REZA FARID  IRAJ MOHAMMADZADEH  MEHRNAZ SADEGHI SHABESTARI  MOHAMMAD NABAVI  ABOLHASSAN FARHOUDI
Institution:(1) Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran;(2) Department of Infectious Diseases, Masih Daneshvari Hospital, Beheshti University of Medical Sciences, Tehran, Iran;(3) Department of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran;(4) Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran;(5) Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran;(6) Department of Immunology and Allergy, Mashhad University of Medical Sciences, Mashhad, Iran;(7) Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran;(8) Tabriz University of Medical Sciences, Tabriz, Iran;(9) Semnan University of Medical Sciences, Semnan, Iran;(10) Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, No. 62, Dr. Gharib Street, Keshavarz Boulevard, P.O. Box 14185-863, 14194 Tehran, Iran
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.
Keywords:Epidemiology  immunological deficiency syndromes  infection  Iran
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