Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Aghamohammadi Asghar, Mohammadinejad Payam, Abolhassani Hassan, Mirminachi Babak, Movahedi Masoud, Gharagozlou Mohammad, Parvaneh Nima, Zeiaee Vaheid, Mirsaeed-Ghazi Bahram, Chavoushzadeh Zahra, Mahdaviani Alireza, Mansouri Mahboubeh, Yousefzadegan Sedigheh, Sharifi Bahareh, Zandieh Fariborz, Hedayat Ehsan, Nadjafi Ali, Sherkat Roya, Shakerian Behzad, Sadeghi-Shabestari Mahnaz, Hosseini Reza Farid, Jabbari-Azad Farahzad, Ahanchian Hamid, Behmanesh Fatemeh, Zandkarimi Mohammadreza, Shirkani Afshin, Cheraghi Taher, Fayezi Abbas, Mohammadzadeh Iraj, Amin Reza, Aleyasin Soheila, Moghtaderi Mojgan, Ghaffari Javad, Arshi Saba, Javahertrash Naser, Nabavi Mohammad, Bemanian Mohammad Hassan, Shafiei Alireza, Kalantari Najmedin, Ahmadiafshar Akefeh, Khazaei Hossein Ali, Atarod Lida, Rezaei Nima
Аннотация
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.