GJB2 mutation analysis is used routinely as a first step in genetic testing for autosomal recessive non-syndromic sensorineural hearing loss. Although most GJB2 mutations can be detected by sequencing of the exon 2 of this gene, a prevalent splice mutation, c.-23+1G>A (IVS1+1G>A), is not usually included in the analyzed region. In this study, we have developed an ARMS-PCR strategy for detection of this mutation among Iranian deaf individuals. A total of 418 Iranian individuals with hearing loss consistent with autosomal recessive non-syndromic sensorineural hearing loss based on audiological test result, medical history, physical examination and pedigree of the family, were included in this study. c.35delG and c.-23+1G>A mutations were detected by using ARMS-PCR. Direct sequencing of the exon 2 of the GJB2 gene was performed for mutation analysis of the coding region of this gene. Among 418 investigated cases, a total of 81 patients (~19.4 %) with biallelic pathogenic mutations in the GJB2 gene and 13 cases with only one pathogenic mutant allele were identified. The total allele frequencies of the two most frequent mutations, c.35delG and c.-23+1G>A, among mutated alleles were found to be around 59 and 15.7 %, respectively. High frequency of the c.35delG and c.-23+1G>A mutations among Iranian deaf individuals shows the importance of developing rapid and cost-effective methods for primary mutation screening methods before performing direct sequencing.

Purpose The purpose of this study is to carry out the demographic evaluation and analysis of the entrepreneurial orientation of librarians in Iranian public libraries using the Lumpkin and Dess model. Design/methodology/approach In this study, a structured questionnaire of entrepreneurial orientation was used. The research population consisted of 5,000 librarians of Iranian public libraries who were selected using Krejcie and Morgan table (95% confidence level and p-value of 5%) and stratified the random sampling method. A total of 365 individuals were selected as the study sample. Data were analyzed by SPSS software using descriptive statistics (frequency, percent, mean, standard deviation and standard error mean) as well as inferential statistics (Pearson correlation, one-sample t-test, independent samples t-test and ANOVA. Findings The results showed that the mean level of entrepreneurial orientation of librarians was 2.92 with an average level (µ = 3). Librarians were at an average level (µ = 3) in terms of components of innovativeness (2.98), risk-taking (2.93) and proactiveness (3.02). The status of the autonomy component (2.53) was at an undesirable level (3 < µ), and only the competitive aggressiveness status (3.12) was at the desirable level (3 < µ). The analysis of the librarian’s entrepreneurial orientation based on demographic characteristics indicated that the male librarian’s entrepreneurial orientation was higher than the female librarian’s (p-value = 0.039). Meantime, librarians with library and information science degrees have less entrepreneurial orientation than the librarians with other (non-librarian and information science) degrees (p-value = 0.01). Also, the higher the librarian’s degree, the lower their entrepreneurial orientation will be (p-value = 0.000). In addition, there was no significant relationship between librarians’ age (p-value = 0.14), marital status (p-value = 0.15) or work experience (p-value = 0.388) and their entrepreneurial orientation. Practical implications Entrepreneurial orientation is one of the significant concepts in entrepreneurship of organizations. Accordingly, studying the concept of entrepreneurial orientation and formulating an appropriate strategy is of great importance in improving the performance of libraries because libraries can be more useful and appealing to library members by enhancing the entrepreneurial orientation of librarians, delivering new, unique and tailor-made services to users’ needs. Originality/value Not much research has been carried out on entrepreneurship in library and information sciences. To the best of the authors’ knowledge, this is the first study to examine the entrepreneurial orientation of librarians based on their demographic characteristics.

The present study was conducted with the aim of analyzing the bibliometrics of Koomesh, as one of the oldest and most reputable Iranian medical journals.Materials and Methods: This study was conducted using a bibliometric method on the articles published in Koomesh during the years 2006-2017. For this purpose, through advanced search in the Scopus database, 764 papers were extracted and bibliometric and Visualization analysis was carried out using Excel and VOSviewer software.Results: The publication process of articles and citations received by Koomesh is ascending. The number of articles reached from 14 to 100 articles in 2017 and the number of citations reached from zero citations in 2006 to 289 citations in 2017. The co-authorship map of the journal's authors showed that Raheb Ghorbani, with 88 articles and 27 links, was the most active author and had the most co-authorship. Considerably, evaluation of organizational affiliation and contributing countries in the journal articles indicated that Semnan University of Medical Sciences was ranked first with 310 organizational affiliations and authors from nine countries participated in the articles received by the journal. According to the co-occurrence map of all 5424 keywords in the articles published in Koomesh, 127 keywords, which were the most frequent keywords in three clusters, had the frequency of 10 and more than 10. The article by Abdolreza Raja'i Fard, Abolfazl Mohammadbeygi and Narges Mohammad Salehi, "Estimation of natural age of menopause in Iranian women: A meta-analysis study" with 20 citations was the most cited article of Komosh in Scopus database. Conclusion:This study presents a general perspective on Koomesh performance during the years 2006-2017. The findings of this study can serve as a roadmap to journalists and assist them in continuing their way.