Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 17 2 2018 04 28 201 207 EN Shahrzad Fallah Emergency Department, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahboubeh Mansouri Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Delara Babaei Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Abdollah Karimi Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Seyed Alireza Fahimzad Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahnaz Armin Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sedigheh Rafiei Tabatabaei Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Roxana Azma Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Ghamartaj Khanbabaee Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Bahram Bashardoost Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mehrdad Amirmoeini Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Saeed Sadr Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Pulmonology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Rozita Jalilianhasanpour Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA Roxana Ghanaei Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Pediatric Infectious Disease, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA Zahra Chavoshzadeh Pediatric Infectious Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran AND Department of Allergy and Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2017 05 11 2017 09 25 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. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/1457 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/1457/824