|
|
Journal of Pediatrics Review، جلد ۲، شماره ۱، صفحات ۳۹-۴۶
|
|
|
| عنوان فارسی |
|
|
| چکیده فارسی مقاله |
|
|
| کلیدواژه‌های فارسی مقاله |
|
|
| عنوان انگلیسی |
Update on Hyper IgE syndrome (HIES) |
|
| چکیده انگلیسی مقاله |
Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency disease. Most of HIES cases are sporadic. HIES type AD is caused by mutation in signal transducer and activator of transcription-3 (STAT-3). A number of mosaicism HIES has been reported that is associated with intermediate phenotype. Autosomal recessive HIES (AR-HIES) is due to mutation in Dock-8 or cytokine sis 8 and TYK2 or tyrosine kinase 2. The common manifestations are atopic eczema, staphylococcal dermatitis, cellulitis and folliculitis (cold dermal abscesses that are not warm, painful and without redness), recurrent pneumonia and pulmonary abscesses, osteopenia and recurrent bone fracture. The diagnosis of standard HIES is based on clinical suspicion. There is no specific treatment for HIES. The treatment should be based on the prevention of developing infections. Prophylactic antibiotics such as cotrimoxazole and IVIG are administered. Hematopoietic stem cell transplantation was done for all types of HIES, but there is a little information and experience about the long term results of this therapy. |
|
| کلیدواژه‌های انگلیسی مقاله |
Hyper IgE Syndrome, infection, Immunodeficiency |
|
| نویسندگان مقاله |
جواد غفاری | javad ghaffari
antimicrobial nosocomial research center, mazandaran university of medical sciences, sari, iran
سازمان اصلی تایید شده: دانشگاه علوم پزشکی مازندران (Mazandaran university of medical sciences)
حمید آهنچیان | hamid ahanchian
allergy research center, mashhad university of medical sciences, mashhad, iran
سازمان اصلی تایید شده: دانشگاه علوم پزشکی مشهد (Mashhad university of medical sciences)
فریبرز زندیه | fariborz zandieh
faculty of medicine, tehran university of medical sciences, tehran, iran
سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)
|
|
| نشانی اینترنتی |
http://jpr.mazums.ac.ir/browse.php?a_code=A-10-30-15&slc_lang=en&sid=en |
| فایل مقاله |
فایلی برای مقاله ذخیره نشده است |
| کد مقاله (doi) |
|
| زبان مقاله منتشر شده |
en |
| موضوعات مقاله منتشر شده |
آلرژی کودکان |
| نوع مقاله منتشر شده |
مرور نقلی |
|
|
|
برگشت به:
صفحه اول پایگاه |
نسخه مرتبط |
نشریه مرتبط |
فهرست نشریات
|