Association of Fibroblast Growth Factor (Fgf-21) as a Screening Biomarker for Chronic Progressive External Ophthalmoplesia

Purpose: To investigate whether or not fibroblast growth factor (FGF-21) can be used as a screening biomarker in chronic progressive external ophthalmoplesia (CPEO) patients.Methods: FGF-21 concentration was measured in the serum of 24 patients with CEPO phenotype and 24 control samples by enzyme-linked immunosorbent assay (ELISA) and determined the deletion of mitochondrial genome by multiplex polymerase chain reaction (PCR).Results: FGF-21 concentration in 50 % of CPEO patients showed notable differences from that in control subjects. FGF-21 concentration ratio in patient group, 2 disorder control groups (mitochondrial and non-mitochondrial) and normal group, respectively, was 294.87 } 42.10 (p < 0.0001), 761.78} 75.07 (p < 0.0001), 124.26 } 12.27 (p = 0.1203), 69.27 } 10.09 (p = 0.2195). A statistically significant inverse correlation between FGF-21 concentration and age onset was found, with a significant difference (p < 0.05) in the age group . 19 years (mean FGF-21  concentration, 460.36 pg/mL) and for the age group . 51years (mean concentration FGF-21, 57.87 pg/mL. Surprisingly, there was no significant difference between FGF-21 concentration and age in the mid-age group (20 . 50 years) .Conclusion: These findings indicate that FGF-21 concentration significantly increases in CPEO patients like in other mitochondrial disorders and this factor can be used as a biomarker in primary diagnosis of mitochondrial disorders. In this regard, FGF-21 assay is only valid in teenagers and the >50 years age group who show acute symptoms.Keywords: Chronic progressive external ophthalmoplesia, Fibroblast growth factor-21, Mitochondrial disorders, Ophthalmoplesia, Biomarke

The effect of red lentil hydroalcoholic extract on retention and retrieval of memory in young and aged mice

Background: Since ancient times, people have believed that certain foods or plants can affect learning and memory potency in humans. The consumption of food and beverages rich in flavonoid compounds has been proposed as a way to restrict the neurodegeneration associated with many neurological complications and to reverse or prevent deterioration in cognitive performance. Objectives: In the present study, we have attempted to show the effect of red lentil extract (RLE), an edible legume with a high quantity of flavonoid, on retrieval and retention of memory in young and aged animals with the use of a passive avoidance apparatus. Materials and Methods: For the experiments, after coding, the animals (128 total) were weighted and classified into different groups as follows: Group 1 as a control received only electric shock, while group 2 as a blank received electric shock plus normal saline (1 mL/100 g). The test groups (groups 3 and 4) received electric shock plus 400 and 800 mg/kg ip. RLE, respectively. The delay in leaving the platform of avoidance apparatus was measured for both retrieval and retention tests of memory in all groups, whereas experiments were conducted on two age levels - young and aged mice. In the test of retention after getting electric shock, RLE immediately, whereas in the test of retrieval 23.5 hours after the shock was administered. Results: Our findings demonstrated where applying both400mg/kg and 800 mg/kg of RLE significantly increased (the latency time increased about 2- and 3-fold respectively in comparison with the control group) retention and retrieval (at least 7-fold compared to the control group) of the memory of young (P < 0.05) and aged (P < 0.01) mice. Conclusions: It can be concluded that the devisable memory-enhancing effects of red lentil (lens culinaris) are due to the antioxidant activity of its flavonoid, tannins, and terpenoids. © 2016, School of Pharmacy, Ahvaz Jundishapur University of Medical Sciences

A case report of congenital myasthenic syndrome caused by a mutation in the CHRNE gene in the Iranian population

Congenital myasthenic syndrome (CMS) refers to a heterogeneous group of inherited disorders, characterized by defective transmission at the neuromuscular junction (NMJ). Patients with CMS showed similar muscle weakness, while other clinical manifestations are mostly dependent on genetic factors. This disease, caused by different DNA mutations, is genetically inherited. It is also associated with mutations of genes at NMJ, involving the acetylcholine receptor (AChR) subunits. Here, we present the case of a five-year-old Iranian boy with CMS, undergoing targeted sequencing of a panel of genes, associated with arthrogryposis and CMS. The patient had six affected relatives in his genetic pedigree chart. The investigations indicated a homozygous single base pair deletion at exon 12 of the CHRNE gene (chr17:4802186delC). This region was conserved across mammalian evolution and was not submitted to the 1000 Genomes Project database. Overall, the CHRNE variant may be classified as a significant variant in the etiology of CMS. It can be suggested that the Iranian CMS population carry regional pathogenic mutations, which can be detected via targeted and whole genome sequencing

The Efficacy and Safety of Intrathecal Autologous Bone Marrow-Derived Mesenchymal Stromal Cells in Amyotrophic Lateral Sclerosis: A Pilot Study

Purpose: Amyotrophic lateral sclerosis (ALS) is an uncommon and aggressive neurodegenerative disorder that influences the lower and upper motor neurons. There are low eligible drugs for ALS treatment; in this regard, supplemental and replacement treatments are essential. There are relative studies in the field of mesenchymal stromal cells (MSCs) therapy in ALS, but the different methods, differently used medium, and difference in follow-up periods affect the outcome treatment. Methods: The current survey is a single-center, phase I clinical trial to evaluating the efficacy and safety of autologous bone marrow (BM)-derived MSCs through intrathecal administration in ALS patients. MNCs were isolated from BM specimens and cultured. The clinical outcome was evaluated based Revised Amyotrophic Lateral Sclerosis Functional Rating (ALSFRS-R) Scale. Results: Each patient received 15±3×106 cells through subarachnoid space. No adverse events (AEs) were detected. Just one patient experienced a mild headache after injection. Following injection, no new intradural cerebrospinal pathology transplant-related was observed. None of the patients’ pathologic disruptions following transplantation were detected by magnetic resonance imaging (MRI). The additional analyses have shown the average rate of ALSFRS-R score and forced vital capacity (FVC) reduction have decreased during 10 months following MSCs transplantation versus the pretreatment period, from -5.4±2.3 to -2±3.08 ALSFRS-R points/period (P=0.014) and -12.6±5.22% to -4.8±14.72%/period (P<0.001), respectively. Conclusion: These results have shown that autologous MSCs transplantation reduces the disease’s progression and has favorable safety. Trial Registration: This study performed as a phase I clinical trial (code IRCT20200828048551N1)

Occurrence of Dioctophyme renale (Goeze, 1782) in road-killed canids of Iran and its public health implication

Dioctophyme renale, is the largest of parasitic nematodes, which infects different species of fish-eating carnivores worldwide. The northern provinces of Iran (Guilan and Mazandaran) located in south of the Caspian Sea are suitable for parasitic infections due to the mild and humid climatic conditions. From separate surveys of road-killed canids in various parts of the Caspian Sea littoral area in Iran, 70 carcasses were collected along the roads of Guilan and Mazandaran from 2015 to 2017. Dioctophyme renale detected by direct observation and molecular methods based on Cytochrome c oxidase subunit 1 (COX1 gene) sequencing analysis. Molecular investigation was also performed to validate prevalence and reduce false negative concerns. Dioctophyme renale was found in eight of 70 carnivores, mostly in the right kidneys, as well as two cases in the abdominal cavity of a dog and a golden jackal. More carcasses on the roads were seen with lacerated internal organs. Given the frequent number of giant kidney worms in canids in the region, the transmission of this zoonotic helminth to humans seems possible, since the area is a tourism hub in the country. The infection burden of this helminth should be investigated using DNA analysis of kidney tissue of road-killed carnivores in Iran. Keywords: Giant kidney worm, COX1, gene Carnivores, Conventional PCR, One healt

Factors Affecting Dysfunctional Behavior and Turnover of Auditors in Audit Institutions

The main factor in the success of many service companies, including auditing institutions, is manpower. Without expert manpower, performance and effectiveness of these institutions will be essentially affected, because human resource is the most important asset of the institutions. Therefore, functional behavior and also durability of auditors have great importance to these institutions. In the other hand, the dysfunctional behavior and turnover of employees in these institutions will be a big blow. This study aims to examine the factors affecting the dysfunctional behavior at various levels (Including task complexity, auditor independence, time budget pressure and client’s importance) in order to answer the question whether dysfunctional behavior can influence the turnover of auditors in auditing institutions or not. So, 160 people (including technical managers, senior directors and supervisor) in the process of implementation of the study were surveyed using a questionnaire. The results of the analysis carried out using structural equation modeling and Smart PLS indicates that task complexity, auditor independence and time budget pressure impact dysfunctional behavior and this behavior has an effect on turnover in auditing institutions, while the role of the client’s importance on dysfunctional behavior is not supported

Brain Abscess and Keratoacanthoma Suggestive of Hyper IgE Syndrome

Hyper immunoglobulin-E (IgE) syndrome is an autosomal immune deficiency disease. It is characterized by an increase in IgE and eosinophil count with both T-cell and B-cell malfunction. Here, we report an 8-year-old boy whose disease started with an unusual skin manifestation. When 6 months old he developed generalized red, nontender nodules and pathologic report of the skin lesion was unremarkable (inflammatory). Then he developed a painless, cold abscess. At the age of 4 years, he developed a seronegative polyarticular arthritis. Another skin biopsy was taken which was in favor of Keratoacanthoma. Laboratory workup for immune deficiency showed high eosinophil count and high level of immunoglobulin-E, due to some diagnostic criteria (NIH sores: 41 in 9-year-olds), he was suggestive of hyper IgE syndrome. At the age of 8, the patient developed an abscess in the left inguinal region. While in hospital, the patient developed generalized tonic colonic convulsion and fever. Brain computed tomography scan revealed an abscess in the right frontal lobe. Subsequently magnetic resonance imaging (MRI) of the brain indicated expansion of the existing abscess to contralateral frontal lobe (left side). After evacuating the abscesses and administrating intravenous antibiotic, the patient’s condition improved dramatically and fever stopped

Study of the protective effect of hydroalcoholic extract microemulsion of Teucrium polium .L against bromobenzene -induced hepatotoxicity in mice

Background : Liver is a major organ of the body, which can be exposed to various chemicals, drugs and many other xenobiotics such as bromobenzene. The aim of this study was to find out the protective effect of hydroalcoholic extract microemulsion of Teucrium polium against hepatotoxicity induced by bromobenzene. Materials and Methods: Animals were divided into eight groups, with ten animals in each group. Group 1-3 received respectively normal saline base of microemulsion and extract microemulsions in dose of 400 mg/kg orally for 10 days. Group 4 received bromobenzene (0.36 ml/kg, ip) only on the 10th day groups 5-8 received extract microemulsions orally in doses of 50, 100, 200 and 400 mg/kg respectively, during 10 days and bromobenzene (0.36 ml/kg, ip) on the 10th day 1 hour after last dose of extract. 24 hours later, the animals were bled and enzymes ALT, AST and ALP were measured. Animal liver was removed for histological studies. Results: The results showed a significant increase in liver enzyme activity by bromobenzene. The treated groups with Teucrium polium showed a significant decrease in liver enzyme activity in doses of 100, 200 and 400 mg/kg (P<0.05). Histological observations also confirmed the results. Conclusion: The results revealed that hydroalcoholic extract microemulsion of Teucrium polium has protective effect on liver toxicity induced by bromobenzen

Antimicrobial, antioxidant, cytotoxic and apoptotic activities of Satureja khuzestanica

WOS: 000437529600029Objective: Natural products with antimicrobial effect are currently investigated in order to eliminate the use of synthetic antibiotics that cause the resistance of microorganisms. This study determines the antibacterial, antioxidant activity, and also the cytotoxic and apoptotic effect of Satureja khuzestanica on various cancer cell lines. Materials and methods: The individual constituents were identified by their identical retention indices referring to known compounds from the literature. Methanol and ethanol extracts of S. khuzestanica (25 and 50 mg/(mL)) were screened against four Gram-positive and four Gram-negative bacteria. The plant extracts were tested for their antioxidant activity against 2,2-diphenyl1- picrylhydrazyl (DPPH) radical. The interaction between the extracts and plasmid DNA were analyzed by agarose gel electrophoresis. In addition, the cytotoxic activity of the extracts was evaluated on MCF-7, DLD-1, osteosarcoma, and fibroblast cell line. Finally, gene expression of caspase-3, Bax and Bcl-2 were investigated by real-time PCR. Results: Our result indicated that both extracts showed good inhibitory activity against both Gram-negative (Escherichia coli ATCC 35218) and positive (Bacillus subtilis ATCC 6633) bacteria. In addition, the methanol extract of S. khuzestanica had strong antioxidant activity (IC50= 37.0 +/- 0.3 mu g/mL). The extracts showed a strong effect on plasmid DNA. The methanol extract of S. khuzestanica showed a good concentration-dependent cytotoxicity. Finally, IC50= 47.00a +/- 1.21 mu g/mL, ethanol, and water extract had apoptotic effect in MCF-7 cell line. Discussion: MCF-7 was detected as the most sensitive cell line therefore further studies should be done on this plant extract as a potential anticancer agent against breast cancer.Gazi UniversityGazi UniversityThis study was supported in a PhD program by Gazi Universit