Farhoodi A, Ahangari Gh, Chavoshzadeh Z, Ramyar A, Movahedi M, Ghareghozlou M, Heydarzadeh M, Fazlolahi M, Bemanian M H, Zandieh F, Mansori M,
Volume 65, Issue 7 (10-2007)
Abstract
Background: Mutations of ELA2, the gene encoding neutrophil elastase (NE) are known to be associated with cyclic neutropenia (CN) and severe congenital neutropenia (SCN). However, high variability of these mutations has been reported. This study was designed to describe the analysis of the ELA2 gene, clinical manifestations and demographic characteristics in patients with CN and SCN.
Methods: A series of 21 patients with CN or SCN were selected, based on SCINR criteria, from the immunology ward of the Pediatric Medicine Center, Tehran, Iran, from March 2004 to August 2005. The ELA2 gene, isolated from blood samples, was analyzed using RT-PCR and automated capillary sequencing. Informed consent was obtained under the tenets of the Helsinki Declaration and the Ethical Committee of the Tehran University of Medical Sciences.
Results: Kostmann's syndrome and CN was diagnosed in three and 18 patients respectively. Of all the patients, one or two mutations were found in 18 cases (85.7%), including all three patients with SCN and 15 of the patients with CN. Exons two and four had the most mutations (eight and seven cases, respectively). Seven patients had double mutations in two distinct exons. Overall, 16 different mutations were found. At the time of presentation, the mean age of patients was 13.4 ±17.6 months, ranging from one month to seven years. Overall, 61.9% of patients had consanguineous parents. The mean absolute neutrophil count was 830.5 ±419.4 (150-2000)/mm3. On average, each patient had been admitted to the hospital 2.2 ±1.6 times. The neutrophil counts of the SCN patients were significantly higher than those of the CN patients. However, there was no significant difference in the neutrophil counts between patients with mutations and those without mutations. All patients with SCN had two or more infectious complications, although the prevalence of infectious or non-infectious complications did not correlate with ELA2 mutations or the neutropenic disorders.
Conclusion: Mutations in ELA2 appear to play an important role in the phatogenetic mechanisms of CN and SCN. Patients with CN had significantly higher neutrophil counts than SCN patients with CN. Although it possible for the gene encoding neutrophil elastase to have more than one mutation in distinct exons, we found no association between the mutations in ELA2 and their complications in CN and SCN patients.
Batoul Kavyani , Mohammad Yousef Alikhani , Mohammad Reza Arabestani , Shirin Moradkhani , Mohammad Taheri ,
Volume 74, Issue 8 (11-2016)
Abstract
Background: Multidrug-resistant bacteria make many problems in clinical therapy, design and manufacture of synthetic drugs. Pseudomonas aeruginosa is one of the most important multidrug-resistance bacteria leads to variety infections in human especially in immunocompromised, patients with severe burns, and nosocomial infections. It Recent years, this organism makes a big challenge in clinical treatment of infections using a wide range of antibiotics. Medicinal herbs for thousands of years to prevent or treat infectious diseases were considered. Today, pharmacists have high interest of using medicinal herbs to prepare a new antimicrobial compounds. The goal of this study was to investigation the effect of aqueous and alcoholic extract of fresh garlic on the expression of genes encoding elastase and exotoxin A virulence factors, in P. aeruginosa PAO1 strain.
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Methods: Present study was an experimental study and performed from 2015 to 2016 in Hamadan University of Medical Science, Iran. The minimal inhibitory concentration (MIC) and minimal bactericidal concentration (MBC) of aqueous and alcoholic extract of garlic was determined. Then in order to investigation the gene expression of elastase and exotoxin A genes, quantitative real-time polymerase chain reaction (qPCR) method was performed at sub-MBC concentrations.
Results: According to the results aqueous extracts of garlic had better impact in comparison with alcoholic alone. At concentration of 64 and 8 mg/ml of aqueous extract the expression of both elastase and exotoxin A genes were decreased. Although, the expression of elastase gene was most affected by garlic at different concentrations than exotoxin A.
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Conclusion: The results suggested that the compositions of garlic extracts can inhibit the production of virulence factors in P. aeruginosa. So in order to treat infectious diseases in the near future, medicinal plants known as new antimicrobial drugs can be used alone or with antibiotic drugs against pathogenic bacteria.
