Primary Immunodeficiency Disorders in Iran: Update and New Insights from the Third Report of the National Registry

被引：109

作者：

Aghamohammadi, Asghar ^{[1
]}

Mohammadinejad, Payam ^{[1
]}

Abolhassani, Hassan ^{[1
]}

Mirminachi, Babak ^{[1
]}

Movahedi, Masoud ^{[2
]}

Gharagozlou, Mohammad ^{[2
]}

Parvaneh, Nima ^{[1
]}

Zeiaee, Vaheid ^{[3
]}

Mirsaeed-Ghazi, Bahram ^{[4
]}

Chavoushzadeh, Zahra ^{[5
]}

Mahdaviani, Alireza ^{[5
]}

Mansouri, Mahboubeh ^{[5
]}

Yousefzadegan, Sedigheh ^{[1
]}

Sharifi, Bahareh ^{[1
]}

Zandieh, Fariborz ^{[4
]}

Hedayat, Ehsan ^{[1
]}

Nadjafi, Ali ^{[1
]}

Sherkat, Roya ^{[6
]}

Shakerian, Behzad ^{[6
]}

Sadeghi-Shabestari, Mahnaz ^{[7
]}

Hosseini, Reza Farid ^{[8
]}

Jabbari-Azad, Farahzad ^{[8
]}

Ahanchian, Hamid ^{[8
]}

Behmanesh, Fatemeh ^{[8
]}

Zandkarimi, Mohammadreza ^{[8
]}

Shirkani, Afshin ^{[8
]}

Cheraghi, Taher ^{[9
]}

Fayezi, Abbas ^{[10
]}

Mohammadzadeh, Iraj ^{[11
]}

Amin, Reza ^{[12
]}

Aleyasin, Soheila ^{[12
]}

Moghtaderi, Mojgan ^{[12
]}

Ghaffari, Javad ^{[13
]}

Arshi, Saba ^{[14
]}

Javahertrash, Naser ^{[14
]}

Nabavi, Mohammad ^{[14
]}

Bemanian, Mohammad Hassan ^{[14
]}

Shafiei, Alireza ^{[14
]}

Kalantari, Najmedin ^{[15
]}

Ahmadiafshar, Akefeh ^{[16
]}

Khazaei, Hossein Ali ^{[17
]}

Atarod, Lida ^{[18
]}

Rezaei, Nima ^{[1
,19
,20
]}

机构：

[1] Univ Tehran Med Sci, Childrens Med Ctr, Pediat Ctr Excellence, Res Ctr Immunodeficiencies, Tehran 14194, Iran

[2] Univ Tehran Med Sci, Childrens Med Ctr, Pediat Ctr Excellence, Dept Allergy & Clin Immunol, Tehran 14194, Iran

[3] Univ Tehran Med Sci, Childrens Med Ctr, Pediat Ctr Excellence, Dept Rheumatol, Tehran 14194, Iran

[4] Univ Tehran Med Sci, Bahrami Hosp, Dept Immunol, Tehran 14194, Iran

[5] Shahid Beheshti Univ Med Sci, Masih Daneshvari Hosp, Dept Immunol & Allergy, Tehran, Iran

[6] Isfahan Univ Med Sci, Al Zahra Hosp, Dept Immunol & Allergy, Esfahan, Iran

[7] Tabriz Univ Med Sci, Dept Immunol & Allergy, Tabriz, Iran

[8] Mashhad Univ Med Sci, Dept Immunol & Allergy, Mashhad, Iran

[9] Guilan Univ Med Sci, Shahrivar Childrens Hosp 17, Dept Pediat, Rasht, Iran

[10] Ahvaz Jundishapur Univ Med Sci, Dept Immunol & Allergy, Ahvaz, Iran

[11] Babol Univ Med Sci, Amirkola Hosp, Dept Immunol & Allergy, Babol, Iran

[12] Shiraz Univ Med Sci, Namazi Hosp, Dept Immunol & Allergy, Shiraz, Iran

[13] Mazandaran Univ Med Sci, Dept Immunol & Allergy, Sari, Iran

[14] Yazd Univ Med Sci, Dept Immunol & Allergy, Yazd, Iran

[15] Golestan Univ Med Sci, Dept Immunol & Allergy, Gorgan, Iran

[16] Ghazvin Univ Med Sci, Dept Immunol & Allergy, Ghazvin, Iran

[17] Zahedan Med Sci Univ, Dept Immunol & Hematol, Zahedan, Iran

[18] Univ Tehran Med Sci, Imam Khomeini Hosp, Dept Pediat, Tehran, Iran

[19] Univ Tehran Med Sci, Mol Immunol Res Ctr, Tehran, Iran

[20] Univ Tehran Med Sci, Sch Med, Dept Immunol, Tehran, Iran

来源：

JOURNAL OF CLINICAL IMMUNOLOGY | 2014年 / 34卷 / 04期

关键词：

Epidemiology; infection; Iran; primary; immunodeficiency; PROPHYLACTIC INTRAVENOUS IMMUNOGLOBULIN; QUALITY-OF-LIFE; SUBCUTANEOUS IMMUNOGLOBULIN; PARENTAL CONSANGUINITY; PEDIATRIC-PATIENTS; ADVERSE-REACTIONS; IGA DEFICIENCY; 1ST REPORT; DISEASES; CHILDREN;

D O I：

10.1007/s10875-014-0001-z

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页码：478 / 490

页数：13

共 58 条

[21] Population prevalence of diagnosed primary immunodeficiency diseases in the United States [J].