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Severe Combined Immunodeficiency: A Case Series and Review From a Tertiary Pediatric Hospital Pubmed



Fallah S1, 2 ; Mesdaghi M2, 3 ; Mansouri M2 ; Babaei D2, 3 ; Karimi A2, 4 ; Fahimzad SA2, 4 ; Armin S2, 4 ; Tabatabaei SR2, 4 ; Azma R2, 4 ; Khanbabaee G5 ; Bashardoost B3 ; Amirmoeini M3 ; Sadr S2, 5 ; Jalilianhasanpour R6, 7 Show All Authors
Authors
  1. Fallah S1, 2
  2. Mesdaghi M2, 3
  3. Mansouri M2
  4. Babaei D2, 3
  5. Karimi A2, 4
  6. Fahimzad SA2, 4
  7. Armin S2, 4
  8. Tabatabaei SR2, 4
  9. Azma R2, 4
  10. Khanbabaee G5
  11. Bashardoost B3
  12. Amirmoeini M3
  13. Sadr S2, 5
  14. Jalilianhasanpour R6, 7
  15. Ghanaei R2, 4
  16. Rezaei N6, 7
  17. Chavoshzadeh Z2, 3
Show Affiliations
Authors Affiliations
  1. 1. Emergency Department, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  2. 2. Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  3. 3. Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  4. 4. Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, United States

Source: Iranian Journal of Allergy# Asthma and Immunology Published:2018


Abstract

Severe combined immunodeficiency syndrome (SCID) is a life-threatening condition leading to early infant death as a result of severe infection, due to impaired cellular and humoral immune systems. Various forms of SCID are classified based on the presence or absence of T cells, B cells and natural killer cells. Patients usually present with recurrent infections and failure to thrive. Definitive treatment is hematopoietic stem cell transplantation. To achieve the best outcome, it should be performed prior to the development of severe infection. In This study, we described 10 patients (6 male and 4 female) with SCID who were admitted to Mofid Children Hospital, Tehran, Iran, from 2006 to 2013. We reviewed patients’ clinical manifestation, laboratory data, family history and outcome. The mean age at the time of diagnosis was 131.8 days. One patient had non-consanguineous parents. Seven patients received BCG vaccine before the diagnosis of SCID, three of them showed disseminated BCG infection. One patient presented with invasive pulmonary aspergillosis. Flow cytometric analysis showed T-B+NK- in three patients, T-B-NK+ in five patients, T-B-NK-in one patient, and T-B+NK+ in one patient. This study highlights the importance of early diagnosis and patient referral before the occurrence of serious infection. Copyright© April 2018, Iran J Allergy Asthma Immunol. All rights reserved.
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