当前位置： SCI文献检索 > EUROPEAN JOURNAL OF PEDIATRICS期刊下所有文献 > Variable outcome of experimental interferon-gamma therapy of disseminated Bacillus Calmette-Guerin infection in two unrelated interleukin-12Rbeta1-deficient Slovakian children.

Variable outcome of experimental interferon-gamma therapy of disseminated Bacillus Calmette-Guerin infection in two unrelated interleukin-12Rbeta1-deficient Slovakian children.

Abstract：

UNLABELLED: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-gamma (IL-12/23-IFNgamma) 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 beta1 (IL-12/23Rbeta1) deficiency was diagnosed. Thus, in addition to chemotherapy, immunomodulatory treatment with recombinant IFN-gamma was performed as the pathogenesis of BCG disease in IL-12Rbeta1 deficiency involves impaired IL-12- and IL-23-dependent IFN-gamma 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 beta1-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.

journal_name

Eur J Pediatr

journal_title

European journal of pediatrics

authors

Ulrichs T,Fieschi C,Nevicka E,Hahn H,Brezina M,Kaufmann SH,Casanova JL,Frecerova K

doi

10.1007/s00431-004-1599-2

keywords:

subject

Has Abstract

pub_date

2005-03-01 00:00:00

pages

166-72

issue

3

eissn

0340-6199

issn

1432-1076

journal_volume

164

pub_type

杂志文章

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