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Bcgitis As the Primary Manifestation of Chronic Granulomatous Disease Publisher



Khalili N1, 2 ; Mohammadzadeh I3 ; Khalili N1, 2 ; Heredia RJ4 ; Zoghi S4, 5, 6 ; Boztug K4, 5, 7 ; Rezaei N8, 9, 10
Authors
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Authors Affiliations
  1. 1. Cancer Immunology Project (CIP), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  2. 2. School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran
  4. 4. Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria
  5. 5. CeMM Research Centre for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
  6. 6. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Vienna, Austria
  7. 7. St Anna Children's Hospital, Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
  8. 8. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  9. 9. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  10. 10. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran

Source: IDCases Published:2021


Abstract

Patients with primary immunodeficiency disease (PID) are not only vulnerable to mycobacterial disease, but are also more likely to develop adverse events following BCG vaccination. These events can range from regional disease (BCGitis) to disseminated disease (BCGosis). Chronic granulomatous disease (CGD), which is characterized by impaired leukocyte phagocytic function, is one of the many inherited PIDs that increase the body's susceptibility to recurrent bacterial and fungal infections. Here, we report a 6-year-old boy with no significant past medical history who presented with progressive lymphadenopathy six years after BCG vaccination. He was later diagnosed with CGD on further evaluation. © 2020
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