作者
Gholamreza Azizi, Marzieh Tavakol, Reza Yazdani, Samaneh Delavari, Tannaz Moeini Shad, Seyed Erfan Rasouli, Mahnaz Jamee, Salar Pashangzadeh, Arash Kalantari, Mansoureh Shariat, Alireza Shafiei, Javad Mohammadi, Gholamreza Hassanpour, Zahra Chavoshzadeh, Seyed Alireza Mahdaviani, Tooba Momen, Nasrin Behniafard, Mohammad Nabavi, Mohammad Hassan Bemanian, Saba Arshi, Rasol Molatefi, Roya Sherkat, Afshin Shirkani, Soheila Alyasin, Farahzad Jabbari‐Azad, Javad Ghaffari, Mehrnaz Mesdaghi, Hamid Ahanchian, Maryam Khoshkhui, Mohammad Hossein Eslamian, Taher Cheraghi, Abbas Dabbaghzadeh, Rasoul Nasiri Kalmarzi, Hossein Esmaeilzadeh, Javad Tafaroji, Abbas Khalili, Mahnaz Sadeghi‐Shabestari, Sepideh Darougar, Mojgan Moghtaderi, Akefeh Ahmadiafshar, Behzad Shakerian, Marzieh Heidarzadeh, Babak Ghalebaghi, Seyed Mohammad Fathi, Behzad Darabi, Morteza Fallahpour, Azam Mohsenzadeh, Sarehsadat Ebrahimi, Samin Sharafian, Ahmad Vosughimotlagh, Mitra Tafakoridelbari, Maziyar Rahimi Haji‐Abadi, Parisa Ashournia, Anahita Razaghian, Arezou Rezaei, Fereshte Salami, Paniz Shirmast, Nasrin Bazargan, Setareh Mamishi, Hossein Ali Khazaei, Babak Negahdari, Sima Shokri, Seyed Hesamedin Nabavizadeh, Saeed Bazregari, Ramin Ghasemi, Shiva Bayat, Hamid Eshaghi, Nima Rezaei, Hassan Abolhassani, Asghar Aghamohammadi
发表日期
2021/8
期刊
Pediatric Allergy and Immunology
卷号
32
期号
6
页码范围
1335-1348
简介
Background
The inborn errors of immunity (IEIs) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections besides other complications including autoimmune and inflammatory diseases. In this study, we aim to evaluate clinical, immunologic, and molecular data of monogenic IEI patients with and without autoimmune manifestations.
Methods
We have retrospectively screened cases of monogenic IEI in the Iranian PID registry for the occurrence of autoimmunity and immune dysregulation. A questionnaire was filled for all qualified patients with monogenic defects to evaluate demographic, laboratory, clinical, and molecular data.
Results
A total of 461 monogenic IEI patients (290 male and 171 female) with a median (IQR) age of 11.0 (6.0‐20.0) years were enrolled in this study. Overall, 331 patients (72.1%) were born to consanguineous parents. At the time of the study, 330 …
学术搜索中的文章
G Azizi, M Tavakol, R Yazdani, S Delavari… - Pediatric Allergy and Immunology, 2021