Identification of the First Iranian Family with “γArg275Cys” Mutation (Fibrinogen Tokyo II)

Background: Inherited fibrinogen deficiencies areclassified into two categories: quantitative, including afibrinogenemia and hypofibrinogenemia and qualitative, including dysfibrinogenemia. Any mutation in fibrinogen genes accounts for one of these disorders.Case report: This article reports an Iranian family with dysfibrinogenemia without any clinical signs accidentally diagnosed by routine coagulation tests with slightly elevated PT and APTT a few years ago. Fordeterminationof disease causing genetic aberration in fibrinogen genes, DNA sequencing of three hot spots of these genes (i.e. exon 2 of FGA,exon 2 of FGB and exon 8 of FGG)was performed. Analysis of sequencing results revealed a heterozygous missense mutation c.901 C>T (Arg275Cys) in exon 8 of FGG in mother and children.No mutationwas detected in father’s sample.Fibrinogen with this mutation is known as Tokyo II.Conclusion: γArg275Cys is a heterozygous mutation that impairs the function of fibrinogen andhas been solely reported in dysfibrinogenemic patients. Clinical findings in this family (no history of bleeding and thrombosis) were compatible with molecular results, because fibrinogen Tokyo II does not have a thrombotic or hemorrhagic nature and lack of clinical signs in this family is not unexpected.

Serum Vascular Endothelial Growth Factor (VEGF) levels in patients with alpha thalassemia

 Background: Alpha-thalassemia syndrome includes a group of hereditary anemia in which expression of alpha globin chains is decreased or absent. Impaired RBC in patients with thalassemia causes vessel involvement and endothelial cell vessel disturbance. Vascular Endothelial Growth Factor (VEGF) is the most important regulator for endothelial cell proliferation. So, the aim of this study is to compare the serum VEGF levels in patients with alpha thalassemia and normal control group.Materials and Methods: This case-control study was conducted on 17 patients with alpha thalassemia and 40 healthy people. Serum VEGF levels were measured by enzyme-linked immune sorbent assay (ELISA) kit. Then statistical analysis of results were performed using SPSS 16, value of P <0.05 was considered statistically significant.Results: Mean serum VEGF levels in case and control groups were 2294.19±1552.39 and 598.09±988.17pg/ml, respectively. Serum VEGF levels were higher in patients with alpha thalassemia (P <0.01). There was no significant correlation between serum VEGF levels and Hemoglobin. (P= 0.73).Conclusion: Our study revealed that patients with alpha thalassemia have elevated levels of serum VEGF than normal control group. Further studies with larger sample size are recommended to confirm these observations

Investigating the factors affecting the presence of women in behavioral Settings With an emphasis on environmental perception. Case study: Goldasht Ma'ali Abad neighborhood of Shiraz

Behavioral settings, as areas of urban life, can play an important role in the civic life experience of specific user groups, including women. This study seeks to investigate the factors and components affecting the presence of women with an emphasis on "environmental perception" in behavioral settings. The present research is of applied type and its research method is descriptive-analytical. After formulating the theoretical framework with the help of reviewing previous research, the results have been tested using the "quantitative survey" method and with the help of a questionnaire and using the Likert spectrum. A case study is the Goldasht Ma'ali Abad neighborhood, and in order to survey the status of women's presence in the neighborhood's behavioral settings and the factors influencing it, 376 questionnaires were distributed and collected among women present in the neighborhood spaces. Data were analyzed in SPSS software environment using Friedman tests, correlation and regression. The findings show that the desirability of the behavioral base and the optimal perception of the urban environment have a significant effect on the presence of women in the urban space and there is a significant correlation between the desirability of the behavioral settings and the perception of the environment with strong intensity

Exploring COVID-19 patient's dignity and satisfaction:a cross-sectional study

Aim With the outbreak of COVID-19 and associated challenges such as increased workload for health providers and shortage of equipment, it became more challenging to maintain patients' dignity and satisfaction. This study evaluated the patients' dignity and satisfaction with COVID-19 in Kashan, Iran, in 2021.Design A cross-sectional descriptive study.Methods In total, 385 patients recovered from COVID-19 were selected through sequential sampling method. Data were collected using demographic, patient dignity inventory, and patient satisfaction questionnaires. Data were analysed using descriptive analysis, independent t-test, ANOVA, and Spearman-Brown coefficient.Results The mean age of patients was 50.57. The mean scores of patients' dignity and satisfaction were 1.622 ± 0.653 and 3.851 ± 0.548 (out of 5), respectively. The dignity rating was associated with age, gender, education, underlying disease, and length of hospital stay (p < 0.05); but patient satisfaction was only associated with education (p = 0.002). The results indicated that dignity and satisfaction have a significant direct correlation (r = −0.23, p < 0.001).Patient or Public Contribution This study was designed based on the research priorities and needs in the field of clinical research and patients were involved in conducting the study via participating in data collection