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Objectives: Myotonic Dystrophy type I (DM1) is a dominantly inherited disorder with a multisystemic pattern affecting skeletal muscle, heart, eye, endocrine and central nervous system. DM1 is associated with the expansion and instability of CTG repeat in the 3' untranslated region of the myotonic dystrophy protein kinase (DMPK) gene located on chromosome 19q13.3. The aim of this study was to determine clinical and genetic characteristic of DM1 in Iranian patients. Genotype-phenotype correlation was also assessed in a small group of studied patients.

Methods: Twenty six DM1 patients belonging to seventeen families were analyzed. Clinical assessment was based on the muscular disability rating scale (MDRS) and a sum of symptoms score (SSS). Molecular analysis (PCR and Southern blot) was used to clarify uncertain clinical diagnosis and in order to confirm clinical findings.

Results: There was an inverse and significant correlation between age of onset  and expanded allele  length (P=0.026, tau-b=-0.360) based on Kendall's tau-b correlation coefficient, while there was no significant correlation between age of onset and severity of the clinical symptoms (P<0.05). Also no significant correlation was observed between the two severity scales of the disease (MDRS and SSS) and expanded allele length (P<0.05). Expanded allele length was correlated with hypogonadism (P=0.007) and cognitive impairment (P=0.034).

Discussion: There was no correlation between cataract and endocrine dysfunction with the expansion size in DM1 patients. Generally it seems there is discordant correlation between clinical symptoms and expanded allele length.

Objectives: Because of the high rates of accidents, the construction industry has become a hazardous industry in Iran and therefore paying more attention is required. This paper investigates safety management in construction companies for improving workers’ safety as one of the ergonomics aims.

Methods: In order to survey the safety management in construction companies a questionnaire was prepared and all aspects in construction safety management structure were included.

Results: The main important problems of safety management in construction include: lack of safety training, lack of provision of personal protection equipment, absence of safety and health specialized staff in construction sites, disuse of safety regulations and lack of updated rules, traditional management attitudes toward safety as a cost.

Discussion: Despite other industries, safety, health, and environment management have not found its true position in the construction sector however some construction companies have established some provisional measures recently. Among the most important problems in safety management in the construction sector, these items can be mentioned: regulations need to be reviewed considering the type of project, low cultural level of the working class, and safety leadership do not perform well in the construction sites.

Objectives: Emotional regulation refers to strategies that reduce, maintain or increase an emotion in people. The purpose of this study is to standard the Cognitive Emotion Regulation Questionnaire in Tehran aged population.

Methods: The sample was selected randomly from 20 Jahandidhgan associations and consisted of 500 aged people from whom 338 people remained till the end of study. In order to define the dimensional structure a Principal Component Analysis with Varimax-rotation on item level was performed.

Results: The alpha coefficients of the various subscales across the diverse populations was 0/798 but the items 5, 7, 31, 6, 8, 32, 30, 29 and 12 had week co efficiency (0.84) which was consider as subscale.

Discussion: After some steps of analysis with Varimax-rotation, 7 factors was created and the 8th factors which was categorized as subscale was named as "acceptance of negative happenings". These results prove that the cognitive coping strategies can be used in Iranian aging population.

Occupational therapy in Iran is relatively young-established in 1971-with a fluctuated development due to the Iranian revolution in 1979, and eight years war from 1981 to 1989. Today, there are ten Bachelor programs across the country approved by World Federation of Occupational Therapists (WFOT). Furthermore, occupational therapists have opportunities for further postgraduate training in both Master and PhD levels. Fast growing of occupational therapy due to social demands necessitated therapists to develop a professional organization. Therefore, Iranian Occupational Therapy Association (IROTA) was formally established in 1994. This paper aims to present an overview about occupational therapy in Iran. Iranian context including population, health status and culture is also reviewed. This follows with explanation about occupational therapy background, education and development. Finally, conception, development, purposes, and achievements of Iranian Occupational Therapy Association present situation and future perspectives of occupational therapy are discussed.

The aim of this review article is describing a research on spiritual and religious interventions in Iran. An integrative review was conducted to determine the state of the science in Iran. Iranmedex, Scientific Information Database, Irandoc, Noormags, Magiran and Google scholar were searched to find articles published in peer-reviewed journals from August 2002 to August 2012. A qualitative approach utilizing content analysis was used in the review. Overall, 21 articles on spiritual and religious interventions in Iran's health care system which met the search criteria were included from 800,000 records in 438 journals. The review shows that there are at least four overarching themes of spiritual and religious interventions: spiritual and religious behaviours, spiritual care as part of a holistic caring approach, spiritual/religious therapy as an effective healing technique, and patients’ spiritual needs. These themes are linked and interrelated. The main concern for caregivers was “hanging on to spirituality” in spite of the eroding effects on spiritual beliefs caused by different factors in the health care system. Spirituality plays an important role in the way people live and die. The majority of the research on spiritual and religious interventions in Iran’s health care system focuses on patients’ need toward spiritual care and health professionals’ spiritual approach, as well as factors that influence their spirituality. More research is needed on the factors that influence patients’ spiritual needs, spirituality among health care providers, and interventions to engender spiritual and religious interventions in the health care system.

Objectives: Emotion regulations refer to the ability of experiencing emotions as a basic human capacity and the experience of the basic emotions happiness, anger, sadness and fear are considered as reular characteristics among nations. In school-age children, problems in socioemotional development typically shows themselves as challenging, socially disruptive paterns of behavior. The purpose of the present study was to understand Emotion Regulation characteristics in Iranian primary school pupils and whether Iranian children enable to identify common emotions.

Methods: Participants included 900 children, 9 to 10 years, from elementary schools from 21 provinces in Iran. In pilot work we presented 200 children with four hypothetical vignettes of the kind typically used in display rule research. In the main study children’s knowledge regarding hiding their emotions was assessed through a structured interview. The participants were presented with the interview questions after the vignettes. The answers were coded by two people and the interrater reliability was high. The children were assessed on the basis of four common emotions:Happiness, Anger,Fear,and, Sadness.

Results: The analysis of the data indicated that: 1) all children were enabled to identify and differentiate all four emotions from each other, most of students hide their happiness, anger, fear and sadness, they hide their emotions in specific situation such as school and home, hide happiness and anger against peers and hide fear and sadness in front of adults.

Discussion: The study indicates that Iranian children not only differ from other culture peers in the amount of display rule use in daily life, but also in the situations they report using it and their motives for doing so they suppress their overall emotions more frequently, especially in presence of family and for pro-social and self-protective reasons. These findings provides the basic knowledge about Iranian children emotional expression development which can be used in cultural, educational and therapeutic contexts.

Objectives: Hearing loss (HL) is the most common sensory disorder, and affects 1 in 1000 newborns. About 50% of HL is due to genetics and 70% of them are non-syndromic with a recessive pattern of inheritance. Up to now, more than 50 genes have been detected which are responsible for autosomal recessive non-syndromic hearing loss, (ARNSHL). In  Iran, HL is one of the most common disabilities due to consanguineous marriages. The aim was to investigate the prevalence of three new ARHL genes (GJB4, GJC3, and SLITRK6) reported in neighboring countries among Iranian families with ARNSHL.

Methods: One hundred unrelated families with at least two affected siblings in consanguineous marriage, who were negative for GJB2 gene mutations, were selected. By using three STR markers for each gene, homozygosity mapping was performed.

Results: Two families showed linkage to GJB4, six families were linked to GJC3 and only one family linked to SLITRK6. The samples of these families who showed linkage were sent for Sanger sequencing to detect the causative mutations. However, after analyzing the sequencing results, no mutation could be detected in either of the families. Molecular analysis for these nine families is underway in order to determine the pathogenic mutations using whole exome sequencing.

Discussion: These data demonstrate a very low prevalence of mutation in these three genes (GJB4, GJC3, and SLITRK6) in the Iranian population, since no mutation was detected in our study group of 100 families.

Objectives: The aim of this study was to determine the type and priority needs of people with multiple sclerosis in Khuzestan using Persian version of the Southampton Needs Assessment Questionnaire (SNAQ) and their relationship to age, gender, and physical disability scale, respectively.
Methods: In this cross-sectional study, the priority needs of 100 patients with multiple sclerosis (aged below 18 years) were studied, whose diagnosis had crossed over a period of one year. This study was covered by the MS Society of Khuzestan Province. Out of 100 patients, 25 were men and 75 were women. The data were analyzed to calculate the dispersion index of the unmet needs after the determination of initial priority needs by using the chi-square statistics.
Results: Based on the patients’ responses, around eight unmet needs were located. Treatment needs, rehabilitation, and financial assistance were the first priority, the need for affordable housing and optimizing environment were at the second place, and employment, information, and fun were at the third place. With the increasing scale of physical disability, the dependence of the individuals on basic needs like treatment, rehabilitation, financial service and, accessibility for an optimizing environmental increases, and people’s satisfaction with the quality of health services reduces (P<0.05). The findings of the study also highlighted that need for information increases with increase in age (P<0.05).
Discussion: In order to meet the needs of people with multiple sclerosis in Khuzestan province, attempts should be made by planners and authorities to fix the therapeutic, rehabilitation, and financial issues at first, followed by the issues of affordable housing. Appropriate measures should be implemented for the success of these programs, and proper assessments of its functionality should be done in a periodic manner.

Objectives: Polymorphisms in α-actinin-3 (ACTN3) gene are considered to be important in the genetic predisposition to human athletic performance. Most of the activities in soccer such as jumping, striking the ball, and running are considered plyometric and explosive. Performance power during such activities depends on the strength of the muscles involved. Therefore, high muscle strength is considered crucial for soccer players. The primary objective of this study was to ascertain the association between ACTN3 R577X (rs1815739) polymorphism and sports athletism in Iranian elite male soccer players.
Methods: In total, 90 top-level professional Iranian male soccer players and 200 nonathletic Iranian men from the general population participated in this case–control study. Exon 16 of ACTN3 was genotyped throughout polymerase chain reaction (PCR) followed by Restriction Fragment Length Polymorphism (RFLP) using the restriction enzyme DdeI and direct sequencing. The genotypic and allelic frequencies of R577X polymorphism in athletes were compared to the frequencies in the general population (nonathletes).
Results: According to the results, the percentage distributions of 577RR and 577RX genotypes (41% and 37%, respectively) were significantly higher and lower, respectively, than that of controls (25% and 57%, respectively) (P<0.001). However, no statistically significant difference was found between allelic frequencies (P=0.20).
Discussion: Our findings showed a significant association between ACTN3 genotypes and elite sport performance among Iranian male soccer players, which agrees with several previous studies.

Objectives: To date, there is no specific scale to assess the quality of life in Bipolar Disorder (BD). The Quality of Life in Bipolar Disorder (QoL.BD) is the first questionnaire designed for this purpose. In this study, we aimed to examine the psychometric properties of the Persian Brief Quality of Life in Bipolar Disorder (Brief QoL.BD).
Methods: After forward and backward translation of the questionnaire and comparing the two translations, the Persian version of the questionnaire was prepared to be tested. The statistical population comprised all patients and normal individuals in Tehran . The sample population included 118 patients with BD and 132 healthy individuals without the history of mental disorder; they were selected using a convenience sampling method. The study instruments included the Beck Depression Inventory-II, Bech–Rafaelsen Mania Rating Scale, the 36-Item Short-Form Health Survey (SF-36), and the Brief QoL.BD. Data were analyzed using SPSS and Lisrel statistical programs.
Results: The confirmatory factor analysis indices indicated a relatively good model fit. Content validity estimates were also acceptable. Convergent validity was examined by calculating the correlation between the scores on the Brief QoL.BD and those on the SF-36 questionnaire; the results were found to be significant (r=0.36, P<0.01). Discriminant validity was assessed by comparing the Brief QoL.BD total scores between the groups of patients with BD and healthy individuals; the results indicated a significant difference between the two groups (t=65.09, P<0.01). The Cronbach’s alpha of 0.70 indicated an internal consistency. A test–retest reliability estimate of 0.89 indicated a high reliability of the scale.
Discussion: The Brief QoL.BD has high validity and reliability in assessing the QoL of patients with BD in Iran.