• Home
  • Browse
    • Current Issue
    • By Issue
    • By Author
    • By Subject
    • Author Index
    • Keyword Index
  • Journal Info
    • About Journal
    • Aims and Scope
    • Editorial Board
    • Advisory Editorial Board
    • Editorial Staff
    • Publication Ethics
    • Indexing and Abstracting
    • Related Links
    • FAQ
    • Peer Review Process
    • News
  • Guide for Authors
  • Submit Manuscript
  • Reviewers
  • Contact Us
 
  • Login
  • Register
Home Articles List Article Information
  • Save Records
  • |
  • Printable Version
  • |
  • Recommend
  • |
  • Export Citation Export to
    RIS EndNote BibTeX APA MLA Harvard Vancouver
  • |
  • Share Share
    CiteULike Mendeley Facebook Google LinkedIn Twitter Telegram
Iranian Journal of Immunology
Articles in Press
Current Issue
Journal Archive
Volume Volume 15 (2018)
Volume Volume 14 (2017)
Volume Volume 13 (2016)
Volume Volume 12 (2015)
Volume Volume 11 (2014)
Issue Issue 4
Issue Issue 3
Issue Issue 2
Issue Issue 1
Volume Volume 10 (2013)
Volume Volume 9 (2012)
Volume Volume 8 (2011)
Volume Volume 7 (2010)
Volume Volume 6 (2009)
Volume Volume 5 (2008)
Volume Volume 4 (2007)
Volume Volume 3 (2006)
Volume Volume 2 (2005)
Volume Volume 1 (2004)
Abolhassani, H., Akbari, F., Mirminachi, B., Bazregari, S., Hedayat, E., Rezaei, N., Aghamohammadi, A. (2014). Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis. Iranian Journal of Immunology, 11(2), 123-133.
Hassan Abolhassani; Fatemeh Akbari; Babak Mirminachi; Saeed Bazregari; Ehsan Hedayat; Nima Rezaei; Asghar Aghamohammadi. "Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis". Iranian Journal of Immunology, 11, 2, 2014, 123-133.
Abolhassani, H., Akbari, F., Mirminachi, B., Bazregari, S., Hedayat, E., Rezaei, N., Aghamohammadi, A. (2014). 'Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis', Iranian Journal of Immunology, 11(2), pp. 123-133.
Abolhassani, H., Akbari, F., Mirminachi, B., Bazregari, S., Hedayat, E., Rezaei, N., Aghamohammadi, A. Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis. Iranian Journal of Immunology, 2014; 11(2): 123-133.

Morbidity and Mortality of Iranian Patients with Hyper IgM Syndrome: a Clinical Analysis

Article 7, Volume 11, Issue 2, Spring 2014, Page 123-133  XML PDF (325 K)
Document Type: Original Article
Authors
Hassan Abolhassani1, 2; Fatemeh Akbari2; Babak Mirminachi2; Saeed Bazregari2; Ehsan Hedayat2; Nima Rezaei2; Asghar Aghamohammadi 2
1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
2Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
Abstract
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.
Keywords
Class Switch Recombination; Clinical Manifestation; Hyper IgM Syndrome
Statistics
Article View: 568
PDF Download: 472
Home | Glossary | News | Aims and Scope | Sitemap
Top Top

Journal Management System. Designed by sinaweb.