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Variable outcome of experimental interferon-γ therapy of disseminated Bacillus Calmette-Guerin infection in two unrelated interleukin-12Rβ1-deficient Slovakian children

Authors
  • Ulrichs, Timo1, 2
  • Fieschi, Claire3
  • Nevicka, Eva4
  • Hahn, Helmut2
  • Brezina, Martin4
  • Kaufmann, Stefan H. E.1
  • Casanova, Jean-Laurent3
  • Frecerova, Klara4
  • 1 Max-Planck-Institute for Infection Biology, Department of Immunology, Berlin, Germany , Berlin
  • 2 Charité, Campus Benjamin Franklin, Department of Medical Microbiology, Institute for Infection Medicine, Berlin, Germany , Berlin
  • 3 Université de Paris René Descartes, Laboratoire de Génetique Humaine des Maladies Infectieuses, Paris, France , Paris
  • 4 National Institute of Tuberculosis and Respiratory Diseases (NUTRCH), Paediatric Pulmonology Department, Krajinska 101, Bratislava, 825 56, Slovak Republic , Bratislava
Type
Published Article
Journal
European Journal of Pediatrics
Publisher
Springer-Verlag
Publication Date
Jan 05, 2005
Volume
164
Issue
3
Pages
166–172
Identifiers
DOI: 10.1007/s00431-004-1599-2
Source
Springer Nature
Keywords
License
Yellow

Abstract

Mycobacterium bovis Bacillus Calmette-Guerin (BCG) is an attenuated live vaccine that may cause life-threatening clinical disease in children with impaired immunity. In particular, patients with any of the nine known inherited disorders of the interleukin-12/23 interferon-γ (IL-12/23-IFNγ) axis are highly vulnerable to BCG. We describe two unrelated young Slovakian children suffering from disseminated BCG infection which developed shortly after routine BCG vaccination after birth. During treatment with selected anti-BCG antibiotics, resistance against several of these drugs developed. In both children, interleukin-12/23 receptor β1 (IL-12/23Rβ1) deficiency was diagnosed. Thus, in addition to chemotherapy, immunomodulatory treatment with recombinant IFN-γ was performed as the pathogenesis of BCG disease in IL-12Rβ1 deficiency involves impaired IL-12- and IL-23-dependent IFN-γ production by lymphocytes. One child responded to treatment and is presently doing well whereas the second patient died. Conclusion:The marked variability of outcome of disseminated Bacillus Calmette-Guerin disease in interleukin-12/23 receptor β1-deficient children sharing the same ethnic origin and exposed to a similar environment as presented in these case reports has to be taken into consideration for diagnosis and treatment of infections due to this genetic defect.

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