Volume 4, Issue 1 (1-2016)                   J. Pediatr. Rev 2016, 4(1): 0-0 | Back to browse issues page

XML Print

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

Golpour M, Ghaffari J. Hyperimmunoglobulin-D Syndrome in Children: A Review Article . J. Pediatr. Rev 2016; 4 (1)
URL: http://jpr.mazums.ac.ir/article-1-119-en.html
1- Associated Professor of Dermatology, Department of Dermatology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran
2- Professor of Allergy and Clinical Immunology, Infectious Disease Research Center with Focus on Nosocomial Infection, Mazandaran University of Medical Sciences, Sari, IR Iran , javadneg@yahoo.com
Abstract:   (3738 Views)
Hyperimmunoglobulin-D syndrome (HIDS) is a rare, autosomal recessively inherited autoinflammatory disease caused by mutations in the mevalonate kinase gene. HIDS usually starts in infancy with recurrent fever episodes lasting three to seven days and recurring every three to six weeks, with only partial symptom decrease in adulthood. Fever is typically accompanied by abdominal pain, vomiting, diarrhea and cervical lymphadenopathy, and sometimes by skin and joint symptoms. Blood leukocytes and serum C-reactive protein (CRP) are elevated during the episode, and in addition, high levels of interleukine-1 (IL-1), IL-6 and tumor necrosis factor (TNF) and respective soluble receptors are measured. Currently, there is no established treatment for HIDS. So far, four children have been successfully treated by TNF-alpha inhibitor (etanercept) and three children with IL-1 receptor antagonist (anakinra). The current study is a narrative review about the updates of HIDS.
Full-Text [PDF 930 kb]   (1821 Downloads)    
Type of Study: Narrative Review |
Received: 2015/11/3 | Accepted: 2016/01/21 | Published: 2016/01/26

Add your comments about this article : Your username or Email:

Send email to the article author

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

© 2023 CC BY-NC 4.0 | Journal of Pediatrics Review

Designed & Developed by : Yektaweb