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کاربرد نوع شرط:
- جایگاه : پژوهشی
- مجله: Iranian Journal of Immunology
- نوع مقاله: Journal Article
- کلمات کلیدی: Diagnosis,Prevalence,Primary Immunodeficiency Disorders
- چکیده:
- چکیده انگلیسی: Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy.
Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital.
Method: All PID cases with a confirmed diagnosis, according to the criteria of International Union of Immunological Societies, who were referred to the Children’s Medical Center in Tehran, Iran, between March 2006 and March 2013 were enrolled in this retrospective cohort study.
Results: Three-hundred and seven PID patients were investigated. Predominantly antibody deficiencies were the most common group of PID observed in 118 cases (38.4%), followed by the well-defined syndromes with immunodeficiency in 52 (16.9%), congenital defects of phagocyte in 45 (14.7%), combined immunodeficiencies in 36 (11.7%), autoinflammatory disorders in 34 (11.4%), immune dysregulation in 11 (3.6%), complement deficiencies in 7 (2.3%), and defects in innate immunity in 3 (1%). Selective IgA deficiency was the most prevalent disorder which affected 46 individuals (14.9%). The median diagnostic delay was 15 months.
Conclusion: Increased awareness and availability of diagnostic tests could result in the better recognition of more undiagnosed PID cases and a decrease in diagnostic delay.- انتشار مقاله: 15-05-1395
- نویسندگان: Payam Mohammadinejad,Babak Mirminachi,Bamdad Sadeghi,Masoud Movahedi,Mohammad Gharagozlou,Javad Mohammadi,Hassan Abolhassani,Nima Rezaei,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: Iranian Journal of Immunology
- نوع مقاله: Journal Article
- کلمات کلیدی: Class Switch Recombination,Hyper IgM Syndrome,Clinical Manifestation
- چکیده:
- چکیده انگلیسی: Background: Defects in B cell class switch recombination (CSR) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper IgM (HIgM) syndrome. Due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients.
Objective: To shed some light on the morbidity and mortality regarding a relatively large cohort of diagnosed CSR defective Iranian patients.
Methods: This study was performed using demographic information, laboratory findings and clinical data obtained from an observation of 33 Iranian patients of different ethnicities referred from all medical centers of Iran to the Children’s Medical Center Hospital, pediatrics center of excellence, Tehran, Iran; of which 28 were males and 5 were females.
Results: Our patients mean age at the onset of symptoms was 1.8 ± 0.2 years; they were diagnosed with a mean delay of 4.4 ± 3.3 years and followed for a mean time of 5.7 ± 4.8 years. The most prominent clinical features observed were multi-organ infections, affecting mostly the respiratory system, followed by lymphoproliferative and autoimmune disorders, the latter being of much higher frequency (44%) in our study than the reported frequency in previous reports. The three year survival rate for our enrolled patients was 67.9%.
Conclusions: Based on our findings, the most common cause of death in HIgM patients is respiratory failure. The molecular mechanism behind the nature of the CSR defective patients in Iran is more compatible with autosomal recessive mutations rather than X-linked HIgM syndrome which is in contrast with other large cohorts of patients with CSR defect.- انتشار مقاله: 14-05-1395
- نویسندگان: Hassan Abolhassani,Fatemeh Akbari,Babak Mirminachi,Saeed Bazregari,Ehsan Hedayat,Nima Rezaei,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Consensus,management,Symptoms,guideline,Primary immunodeficiency
- چکیده:
- چکیده انگلیسی: Primary immunodeficiency (PID) is a group of more than 400 distinct genetic disorders affecting both children and adults. As signs and symptoms of PID are usually heterogeneous and unspecific, based on the available human resources and laboratory facilities, diagnosis and follow-up of these patients can be challenging. In line with this notion, management and treatment of PID are practiced divergently in different PID centers. In order to reach a distinct national protocol and given the little evidence on appropriate or universal guidelines to improve the current status of management of the disease, the Iranian PID network designed a consensus suitable for regional resources. This review summarizes this PID guideline based on the importance of different clinical complications and the level of medical authority visiting those at the first line. Further, for each complication, appropriate interventions are mentioned to improve the approach.
- انتشار مقاله: 29-10-1397
- نویسندگان: Hassan Abolhassani,Marzieh Tavakol,Zahra Chavoshzadeh,Seyed Alireza Mahdaviani,Tooba Momen,Reza Yazdani,Gholamreza Azizi,Masoud Movahedi,Amir Ali Hamidieh,Nasrin Behniafard,Mohammamd Nabavi,Saba Arshi,Mohammad Hassan Bemanian,Morteza Fallahpour,Sima Shokri,Rasol Molatefi,Roya Sherkat,Afshin Shirkani,Reza Amin,Soheila Aleyasin,Reza Faridhosseini,Farahzad Jabbari-Azad,Iraj Mohammadzadeh,Javad Ghaffari,Alireza Shafiei,Arash Kalantari,Mahboubeh Mansouri,Mehrnaz Mesdaghi,Delara Babaie,Hamid Ahanchian,Maryam Khoshkhui,Habib Soheili,Mohammad Hossein Eslamian,Taher Cheraghi,Abbas Dabbaghzadeh,Mahmoud Tavassoli,Rasoul Nasiri Kalmarzi,Seyed Hamidreza Mortazavi,Sara Kashef,Hossein Esmaeilzadeh,Javad Tafaroji,Abbas Khalili,Fariborz Zandieh,Mahnaz Sadeghi-Shabestari,Sepideh Darougar,Fatemeh Behmanesh,Hedayat Akbari,Mohammadreza Zandkarimi,Farhad Abolnezhadian,Abbas Fayezi,Mojgan Moghtaderi,Akefeh Ahmadiafshar,Behzad Shakerian,Vahid Sajedi,Behrang Taghvaei,Mojgan Safari,Marzieh Heidarzadeh,Babak Ghalehbaghi,Seyed Mohammad Fathi,Behzad Darabi,Saeed Bazregari,Nasrin Bazargan,Alireza Khayatzadeh,Naser Javahertrash,Bahram Bashardoust,Mohammadali Zamani,Azam Mohsenzadeh,Sarehsadat Ebrahimi,Samin Sharafian,Ahmad Vosughimotlagh,Mitra Tafakoridelbari,Maziar Rahimi,Parisa Ashournia,Anahita Razaghian,Fatemeh Aghaeimeybodi,Setareh Mamishi,Nima Parvaneh,Nima Rezaei,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Iran,Education,Research,Registry,Immunodeficiency diseases,National network
- چکیده:
- چکیده انگلیسی: Although comprehension of the molecularbasis of primary immunodeficiency diseases (PID) provides unique insight into the functioning of the immune system, translational research is also needed to provide better care to affected individuals. Many institutions and academic departments have been established to provide training and encourage collaborative research on the immune system and related disorders. In Iran, one of the frontiers of PIDs in the Middle Eastern region, considerable progress in basic and clinical immunology has been achieved during the last three decades. During this period, massive improvements have revolutionized the management of PIDs in the country, from educational and research related aspects to diagnostic procedures and treatments available to Iranian PID patients. In this review, we seek to elucidate the current status of PIDs in Iran from different angles and to speculate upon the opportunities that the future may bring.
- انتشار مقاله: 28-01-1397
- نویسندگان: Hassan Abolhassani,Nima Rezaei,Asghar Aghamohammadi
- مشاهده