Volume 2, Number 1 (2-2014)                   JPR 2014, 2(1): 39-46 | Back to browse issues page


XML Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Ghaffari J, Ahanchian H, Zandieh F. Update on Hyper IgE syndrome (HIES). JPR. 2014; 2 (1) :39-46
URL: http://jpr.mazums.ac.ir/article-1-65-en.html

Assistant Professor
Abstract:   (11688 Views)
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.
Full-Text [PDF 242 kb]   (3251 Downloads)    
Type of Study: Narrative Review | Subject: Pediatric Allergology
Received: 2013/11/9 | Accepted: 2014/01/7 | Published: 2014/01/7

Add your comments about this article : Your username or email:
Write the security code in the box

Send email to the article author


© 2015 All Rights Reserved | Journal of Pediatrics Review

Designed & Developed by : Yektaweb