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Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.

Abstract：

BACKGROUND:Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers. METHODS:This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006. RESULTS:A total number of 731 new PID patients (455 male and 276 female) from 14 medical centers were enrolled in the current study. Predominantly antibody deficiencies were the most common subcategory of PID (32.3 %) and were followed by combined immunodeficiencies (22.3 %), congenital defects of phagocyte number, function, or both (17.4 %), well-defined syndromes with immunodeficiency (17.2 %), autoinflammatory disorders (5.2 %), diseases of immune dysregulation (2.6 %), defects in innate immunity (1.6 %), and complement deficiencies (1.4 %). Severe combined immunodeficiency was the most common disorder (21.1 %). Other prevalent disorders were common variable immunodeficiency (14.9 %), hyper IgE syndrome (7.7 %), and selective IgA deficiency (7.5 %). CONCLUSIONS:Registration of Iranian PID patients increased the awareness of medical community of Iran and developed diagnostic and therapeutic techniques across more parts of the country. Further efforts must be taken by increasing the coverage of IPIDR via electronically registration and gradual referral system in order to provide better estimation of PID in Iran and reduce the number of undiagnosed cases.

journal_name

J Clin Immunol

journal_title

Journal of clinical immunology

authors

Aghamohammadi A,Mohammadinejad P,Abolhassani H,Mirminachi B,Movahedi M,Gharagozlou M,Parvaneh N,Zeiaee V,Mirsaeed-Ghazi B,Chavoushzadeh Z,Mahdaviani A,Mansouri M,Yousefzadegan S,Sharifi B,Zandieh F,Hedayat E,Nadjafi A,S

doi

10.1007/s10875-014-0001-z

subject

Has Abstract

pub_date

2014-05-01 00:00:00

pages

478-90

issue

eissn

0271-9142

issn

1573-2592

journal_volume

pub_type

杂志文章,多中心研究

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Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.