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کاربرد نوع شرط:
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: STAT3,Hyper IgE syndromes,DOCK8
- چکیده:
- چکیده انگلیسی: Hyper IgE syndromes are groups of primary immunodeficiency diseases which present with a series of symptoms including recurrent infections accompanied by elevated serum IgE level and some atopic features. Both autosomal dominant and recessive mutations may cause hyper IgE syndrome. The autosomal dominant forms are mutations in signal transducer and activator of transcription3 (STAT3), ERBB2 and CARD11. The recessive forms are mutations in dedicator of cytokinesis8 (DOCK8) , phosphoglucomutase3 (PGM3) , thyrosin kinase2 (TYK2) and interleukin-6 ST. There are some features that help distinguish among different types of hyper IgE syndrome. In autosomal dominant form, connective tissue, skeletal and vascular abnormalities are prominent. In autosomal recessive form, viral infections, malignancies and neurological disorders are more prominent. The definite diagnosis is made by mutation analysis.
- انتشار مقاله: 28-04-1398
- نویسندگان: Marzieh Tavakol,Samin Sharafian,Fereshteh Salari,Sima Shokri
- 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