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- Title
Inherited disorders of the IL-12-IFN-gamma axis in patients with disseminated BCG infection.
- Authors
Mansouri, Davood; Adimi, Parisa; Mirsaeidi, Mehdi; Mansouri, Nahal; Khalilzadeh, Soheila; Masjedi, Mohammad R; Adimi, Parvaneh; Tabarsi, Payam; Naderi, Mohammad; Filipe-Santos, Orchidée; Vogt, Guillaume; de Beaucoudrey, Ludovic; Bustamante, Jacinta; Chapgier, Ariane; Feinberg, Jacqueline; Velayati, Ali A; Casanova, Jean-Laurent
- Abstract
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-12Rbeta1 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.
- Publication
European journal of pediatrics, 2005, Vol 164, Issue 12, p753
- ISSN
0340-6199
- Publication type
Journal Article
- DOI
10.1007/s00431-005-1689-9