Repository of Research and Investigative Information

Repository of Research and Investigative Information

Zahedan University of Medical Sciences

Inherited disorders of the IL-12-IFN-gamma axis in patients with disseminated BCG infection

(2005) Inherited disorders of the IL-12-IFN-gamma axis in patients with disseminated BCG infection. European Journal of Pediatrics. pp. 753-757. ISSN 0340-6199

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Official URL: <Go to ISI>://WOS:000233247900008


Disseminated BCG infection is a rare complication of vaccination that occurs in patients with impaired immunity. In recent years, a series of inherited disorders of the IL-12-IFN-gamma axis have been described that predispose affected individuals to disseminated disease caused by BCG, environmental Mycobacteria, and non-typhoidal Salmonella. The routine immunological work-up of these patients is normal and the diagnosis requires specific investigation of the IL-12-IFN-gamma circuit. We report here the first two such patients originating from and living in Iran. The first child is two years old and suffers from complete IFN-gamma receptor 2 deficiency and disseminated BCG infection. He is currently in clinical remission thanks to prolonged multiple antibiotic therapy. The other, a 28-year-old adult, suffers from IL-12p40 deficiency and presented with disseminated BCG infection followed by recurrent episodes of systemic salmonellosis. He is now doing well. A third patient of Iranian descent, living in North America, was reported elsewhere to suffer from IL-12R beta 1 deficiency. These three patients thus indicate that various inherited defects of the IL-12-IFN-gamma circuit can be found in Iranian people. In conclusion we recommend to consider the disorders of the IL-12-IFN-gamma circuit in all patients with severe BCG infection, disseminated environmental mycobacterial disease, or systemic non-typhoidal salmonellosis, regardless of their ethnic origin and country of residence.

Item Type: Article
Keywords: BCG immunodeficiency interferon-beta interleukin-12 Salmonella bacille calmette-guerin receptor beta-1 deficiency interferon-gamma-receptor interleukin-12 deficiency mycobacterial infection salmonella-enteritidis t-cells resistance susceptibility mutation Pediatrics
Page Range: pp. 753-757
Journal or Publication Title: European Journal of Pediatrics
Journal Index: ISI
Volume: 164
Number: 12
Identification Number:
ISSN: 0340-6199
Depositing User: خانم مهدیه رضائی پور

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