Repetitive Fistulizing Lymphadenopathy and Hematochezia

  • Mahboubeh Mansouri


  • Three major categories of primary immunodeficiency can predispose to BCG infection: 1) severe combined immunodeficiency, 2) innate phagocytic defects as in chronic granulomatous disease, and 3) Mendelian susceptibility to mycobacterial diseases (MSMD)

  • IL-12Rβ1 deficiency is the most common underlying defect in MSMD

  • MSMD confers susceptibility to non-tuberculous mycobacteria, as well as Mycobacterium bovis in BCG, Salmonellae, and selected viral and Candida infections

  • Sever salmonella infections might be the initial or sole presenting feature in MSMD


Disseminated BCG Mendelian susceptibility to mycobacterial diseases Salmonellae 


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Mahboubeh Mansouri
    • 1
  1. 1.Department of Immunology and AllergyMofid Children’s Hospital, Shahid Beheshti University of Medical SciencesTehranIran

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