Advertisement

Repetitive Fistulizing Lymphadenopathy and Hematochezia

  • Mahboubeh Mansouri
Chapter

Abstract

  • 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

Keywords

Disseminated BCG Mendelian susceptibility to mycobacterial diseases Salmonellae 

References

  1. 1.
    Norouzi S, Aghamohammadi A, Mamishi S, Rosenzweig SD, Rezaei N. Bacillus Calmette-Guerin (BCG) complications associated with primary immunodeficiency diseases. J Infect. 2012;64(6):543–54.CrossRefGoogle Scholar
  2. 2.
    Lee WI, Huang JL, Yeh KW, Jaing TH, Lin TY, Huang YC, Chiu CH. Immune defects in active mycobacterial diseases in patients with primary immunodeficiency diseases (PIDs). J Formos Med Assoc Taiwan yi zhi. 2011;110(12):750–8.CrossRefGoogle Scholar
  3. 3.
    Haerynck F, Holland SM, Rosenzweig SD, Casanova JL, Schelstraete P, De Baets F. Disseminated Mycobacterium avium infection in a patient with a novel mutation in the interleukin-12 receptor-beta1 chain. J Pediatr. 2008;153(5):721–2.CrossRefGoogle Scholar
  4. 4.
    Bustamante J, Boisson-Dupuis S, Abel L, Casanova JL. Mendelian susceptibility to mycobacterial disease: genetic, immunological, and clinical features of inborn errors of IFN-gamma immunity. Semin Immunol. 2014;26(6):454–70.CrossRefGoogle Scholar
  5. 5.
    Dorman SE, Picard C, Lammas D, Heyne K, van Dissel JT, Baretto R, Rosenzweig SD, Newport M, Levin M, Roesler J, Kumararatne D, Casanova JL, Holland SM. Clinical features of dominant and recessive interferon gamma receptor 1 deficiencies. Lancet (London, England). 2004;364(9451):2113–21.CrossRefGoogle Scholar

Copyright information

© 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

Personalised recommendations