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Primary imunodeficiency in the practice of pediatric pneumology


Authors: M. Miškovská 1;  E. Mindžáková 1;  J. Fábry 1;  J. Melter 1;  P. Ferenc 1;  T. Strachan 1;  P. Bánovčin 2
Authors‘ workplace: Šrobárov ústav detskej tuberkulózy a respiračných chorôb, n. o., Dolný Smokovec a Klinika detskej tuberkulózy a respiračných chorôb JLF UK a ŠÚDTaRCH, n. o., Dolný Smokovec riaditeľ MUDr. J. Fábry, PhD. 1;  Klinika detí a dorastu JLF a UNM, Martin prednosta prof. MUDr. P. Bánovčin, CSc. 2
Published in: Čes-slov Pediat 2015; 70 (3): 146-151.
Category: Original Papers

Overview

BCG vaccine provides the protectivity mainly against the serious disseminated forms of tuberculosis, not against the most frequent pulmonary tuberculosis. One of the reasons of canceling the BCG vaccination were complications connected with vaccination. They are divided in local, regional and disseminated and are not serious in immunocompetent people, but could be fatal in people with serious immunodeficiency. The authors observed 178 patients with BCG lymphadenitis, abscess in the vaccination site and disseminated BCG-itis.

Results:
Ther were 160 patients with BCG lymphadenitis, 17 patients with the absces in th vaccination site and 1 patient with disseminated complication. The more frequent were lymphadenitis axillaris – 85%, 74.4% had 1 or 2 enlarged or suppurative lymph nodes. Authors didn´t confirm the serious disorder in phagocytosis, in the immunoglobulins levels there were significantly lower levels of IgA in patients with complications against the healthy children. The markers of cellular imunity didn´t confirmed the serious cellular imunodeficiency, but in comparison with healthy group we found out the significantly lower levels of CD19 B-lymphocytes. In all the examinated group we had one patients with extremely high levels of IFNγ, in this patients was the homozygous mutation 523delT of the gene for IFNGR1 confirmed.

Conclusion:
Most of the children with local BCG complications has no immunodeficiency, but in children with primary immunodeficiency the complication after BCG vaccination could have a fatal progression. Since we are now able to diagnose the IFNGR1 deficiency, we know to prevent one of the most serious complications..

Key words:
BCG vaccination, complication after BCG vaccination, mycobacterium, primary imunodeficiency, Mendelian susceptibility to mycobacterial diseases


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Labels
Neonatology Paediatrics General practitioner for children and adolescents
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