Evaluation of interleukin-12 receptor Beta1 and interferon gamma receptor 1 deficiency in patients with disseminated BCG infection

Introduction: Disseminated BCG infections among other complications of Bacillus Calmette-Guérin (BCG) vaccine are rare and have occurred in children with immunodeficiency disorders such as mendelian susceptibility to mycobacterial disease (MSMD) which could be due to defects in some elements of IL-12/IFN-γ axis. MSMD-causing mutations have been identified in 10 genes during the last two decades. Among them, mutations in the IL12Rβ1 and IFN gamma R1 genes constitute about 80% of recorded cases of MSMD syndrome. The aim of this study was to investigate IL-12RBeta1 and IFN- gammaR1 deficiencies in patients with disseminated BCG infection. Methods: This study was performed on 31 children with disseminated BCG infections who referred to children's medical center. Whole blood cell culture was performed in presence of BCG, IL-12 and IFN- gamma stimulators. The supernatants were assayed for IFN-gamma and IL-12p70 by ELISA method. In order to evaluate IL12Rbeta1 and IFN- gammaR1 receptors expression, flow cytometry staining was performed on the patients’ T-cells stimulated with PHA. Results: Flow cytometry staining of 31 Iranian patients with disseminated BCG infections with the average age of 43 months showed lack of the expression of IL-12RBeta1 and IFN- gamma R1 genes in PHA-T-cells of the nine and one patients, respectively in whom the incomplete production of IFN- gamma and IL-12 was reported by ELISA. Among these 10 patients, eight cases had related parents (80%). Conclusion: It is recommended that to avoid BCG complications, screening be performed for MSMD before BCG inoculation in individuals with positive family history of primary immunodeficiency diseases and inhabitants of areas with high frequency of consanguinity

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Evaluation of interleukin-12 receptor ß1 and interferon gamma receptor 1 deficiency in patients with disseminated BCG infection.

INTRODUCTION: Disseminated BCG infections among other complications of Bacillus Calmette-Guérin (BCG) vaccine are rare and have occurred in children with immunodeficiency disorders such as mendelian susceptibility to mycobacterial disease (MSMD) which could be due to defects in some elements of IL-12/IFN-γ axis. MSMD-causing mutations have been identified in 10 genes during the last two decades. Among them, mutations in the IL12Rß1 and IFNγR1 genes constitute about 80% of recorded cases of MSMD syndrome. The aim of this study was to investigate IL-12Rß1 and IFN-γR1 deficiencies in patients with disseminated BCG infection. METHODS: This study was performed on 31 children with disseminated BCG infections who referred to children's medical center. Whole blood cell culture was performed in presence of BCG, IL-12 and IFN-γ stimulators. The supernatants were assayed for IFN-γ and IL-12p70 by ELISA method. In order to evaluate IL12Rß1 and IFN-γR1 receptors expression, flow cytometry staining was performed on the patients' T-cells stimulated with PHA. RESULTS: Flow cytometry staining of 31 Iranian patients with disseminated BCG infections with the average age of 43 months showed lack of the expression of IL-12Rß1 and IFN-γR1 genes in PHA-T-cells of the nine and one patients, respectively in whom the incomplete production of IFN-γ and IL-12 was reported by ELISA. Among these 10 patients, eight cases had related parents (80%). CONCLUSION: It is recommended that to avoid BCG complications, screening be performed for MSMD before BCG inoculation in individuals with positive family history of primary immunodeficiency diseases and inhabitants of areas with high frequency of consanguinity.

Pseudomonas aeruginosa infection among cystic fibrosis and ICU patients in the referral children medical hospital in Tehran, Iran.

INTRODUCTION: Pseudomonas aeruginosa is one of the important causes of hospital-acquired infections in Intensive Care Unit (ICU) and considered as a major determinant of morbidity and mortality in patients affected by cystic fibrosis (CF). The aim of this study was to investigate clonal diversity among randomly picked P. aeruginosa isolates of CF and the other hospitalized patients in ICU. METHODS: Cultivation, identification, and antimicrobial susceptibility testing of P. aeruginosa isolates were performed using standard techniques. The genetic similarity of the strains was investigated by amplification of the Enterobacterial Repetitive Intergenic Consensus-polymerase chain reaction (ERIC-PCR) sequence. RESULTS AND DISCUSSION: Among 49 isolates, sixteen were isolated from 11 patients affected by CF and 33 came from an epidemiological investigation of 25 P. aeruginosa infected patients of ICU. Five clusters were generated for all isolates analyzed through ERIC-PCR genotyping. Two major clusters (B and C) were discovered in P. aeruginosa isolates of ICU and CF patients during the whole period of this study. Fifteen unique antibiogram patterns obtained from all isolates and multi-resistant P. aeruginosa (MRPA) were identified in 23 isolates (47%). MRPA isolates were detected in all clusters (except A) while pan-resistant isolates were recovered only in cluster C. The high prevalence of related or identical isolates in CF and non-CF patients can be due to transmission of particular dominant clones in ICU ward. Therefore, enhanced infection-control may become necessary to prevent further spread of clonal strains.