Background: Variants in recombination-activating genes (RAG) are common genetic causes of autosomal recessive forms of combined immunodeficiencies (CID) ranging from severe combined immunodeficiency (SCID), Omenn syndrome (OS), leaky SCID, and CID with granulomas and/or autoimmunity (CID-G/AI), and even milder presentation with antibody deficiency. Objective: We aim to estimate the incidence, clinical presentation, genetic variability, and treatment outcome with geographic distribution of patients with the RAG defects in populations inhabiting South, West, and East Slavic countries. Methods: Demographic, clinical, and laboratory data were collected from RAG-deficient patients of Slavic origin via chart review, retrospectively. Recombinase activity was determined in vitro by flow cytometry-based assay. Results: Based on the clinical and immunologic phenotype, our cohort of 82 patients from 68 families represented a wide spectrum of RAG deficiencies, including SCID (n = 20), OS (n = 37), and LS/CID (n = 25) phenotypes. Sixty-seven (81.7%) patients carried RAG1 and 15 patients (18.3%) carried RAG2 biallelic variants. We estimate that the minimal annual incidence of RAG deficiency in Slavic countries varies between 1 in 180,000 and 1 in 300,000 live births, and it may vary secondary to health care disparities in these regions. In our cohort, 70% (n = 47) of patients with RAG1 variants carried p.K86Vfs*33 (c.256_257delAA) allele, either in homozygous (n = 18, 27%) or in compound heterozygous (n = 29, 43%) form. The majority (77%) of patients with homozygous RAG1 p.K86Vfs*33 variant originated from Vistula watershed area in Central and Eastern Poland, and compound heterozygote cases were distributed among all Slavic countries except Bulgaria. Clinical and immunological presentation of homozygous RAG1 p.K86Vfs*33 cases was highly diverse (SCID, OS, and AS/CID) suggestive of strong influence of additional genetic and/or epigenetic factors in shaping the final phenotype. Conclusion: We propose that RAG1 p.K86Vfs*33 is a founder variant originating from the Vistula watershed region in Poland, which may explain a high proportion of homozygous cases from Central and Eastern Poland and the presence of the variant in all Slavs. Our studies in this cohort of RAG1 founder variants confirm that clinical and immunological phenotypes only partially depend on the underlying genetic defect. As access to HSCT is improving among RAG-deficient patients in Eastern Europe, we anticipate improvements in survival.

The aim of this research was to determine the influence of motor abilities on the success of performing a freestyle swimming, pre and post the completed swimming training program. The study was conducted on a sample of 90 female students, aged 11-12 years, fifth graders from primary schools in Novi Grad Municipality in Sarajevo, using 6 motor variables and 1 criterion. Regression analysis was used to determine the effect of balance and flexibility on the freestyle swimming performance. Analyzing the presented results of the regression analysis, it was found that after the pre-test measurement of the variables for estimating the balance, the following predictor motor variable had the greatest and statistically significant influence on the criterion variable: standing upright on the balance beam with eyes open (BETA) = .581, which is significant at the level of p<.001. For flexibility, the following predictor motor variable is: upper body bent forward while sitting with legs side by side (BETA) = .411, which is significant at the p<.001 level. After the post-test measurement of the variables for the assessment of motor abilities (balance and flexibility), the same variables as for the pre-test measurement had the statistically significant influence on the criterion variable (freestyle swimming of the sections 25 m long – crawl, chest or back swimming). At standing on the right leg longitudinally, open-eyed, on the balance bench (BETA) = .448, which is significant at the level of p<.001, and in flexibility the variable - bench ab with legs side by side (BETA) = .463, which is significant at the level of p<.001. All movements in swimming require well-developed flexibility (mobility), and in particular the mobility of the torso and upper extremities.

COVID-19 pandemic: implications on the surgical treatment of gastrointestinal and hepatopancreatobiliary tumours in Europe Editor In the context of the ongoing COVID19 pandemic, the field of surgical oncology demands careful crisis management considering the overall importance of timely treatment1,2. Based on an online survey (Google Forms; Google LLC, Mountain View, California, USA) conducted among university-affiliated surgical departments throughout Europe, we offer a multicentre perspective on the instantaneous performance of gastrointestinal (GI) and hepatopancreatobiliary (HPB) tumour surgery. Ninety-five surgeons from 79 surgical departments in 20 European countries completed the survey. The survey revealed an alarming status quo. Some 59 per cent of participants stated that the COVID-19 pandemic is having a strong or very strong impact on ongoing treatment of their patients (Fig. 1a). A decrease in patient referral since onset of the viral outbreak was observed by 90 per cent of respondents. Twentynine per cent reported a decrease of more than 60 per cent compared to the pre-pandemic standard (Fig. 1b). In addition to a re-evaluation of hospital capacities, the excessive workload of general practitioners, the role of the media and general recommendations to avoid person-to-person contact, including medical appointments, have to be reconsidered3. It can be assumed that telemedical options are not offered sufficiently (only 12 per cent of respondents reported telemedical options for all outpatient appointments)4. Moreover, almost one-third reported that Fig 1 Impact of the COVID-19 pandemic

In this paper we report results from an online study conducted in Bosnia and Herzegovina during the ongoing COVID-19 pandemic (May 2020). The study examined a range of social and behavioural responses by youth from different ethnic backgrounds and across 63 cities (N = 569). More specifically, the study focused on investigating the relationship between threat perceptions, public health behaviours, stress and social cohesion. As expected, results indicate that higher perceptions of threat were related to higher compliance to safety and health measures despite extremely extremely low levels of political trust. Surprisingly, participants reported relatively low levels of stress despite high social isolation and physical restrictions. These results could partially be explained by an increased level of family interactions. Furthermore, participants reported relatively high levels of social cohesion and common-ingroup identification in a usually segregated and conflict-ridden context.

ABSTRACT The digital printing process contributes to the quality of indoor air through the emission of chemical stressors such as total volatile organic compounds (TVOCs) and suspended particulate matter (SPM). The concentration levels of SPM and TVOCs were determined using the gravimetric and gas chromatography method, respectively. The results show that the digital printing machines and the cutting machine do not produce PM2.5 particles. The significant cumulative mass concentrations of PM10 suspended particles of 9.91 μg/m3 for a single-colour digital printing machine, 4.45 μg/m3 for a four-colour digital printing machine, and 2.02 μg/m3 for a cutting machine, were detected. By comparing the cumulative values of TVOC concentration levels measured, it can be concluded that the values have been 4 to 12 times higher for the four-colour digital printing machine than for the single-colour digital printing machine. Namely, the TVOC concentration levels measured for the four-colour digital printing machine ranged from 28.69 to 57.30 mg/m3, while the values for the single-colour digital printing machine ranged from 2.31 to 13.51 mg/m3. Considering the fact that there are no standards of quality of indoor air in printing offices defined in the Republic of Serbia, the results obtained could represent a valid basis and a worthy guideline for the definition of values necessary for the indoor air quality assessment.

The success of any activity and process depends fundamentally on the possibility of balancing (symmetry) needs and their satisfaction. That is, the ability to properly define a set of success indicators. The application of the developed new multi-criteria decision-making (MCDM) methods can be eliminated or decreased by decision-makers’ subjectivity, which leads to consistency or symmetry in the weight values of the criteria. In this Special Issue, 40 research papers and one review study co-authored by 137 researchers from 23 different countries explore aspects of multi-criteria modeling and optimization in crisp or uncertain environments. The papers proposing new approaches and elaborate case studies in the following areas of applications: MCDM optimization in sustainable engineering, environmental sustainability in engineering processes, sustainable multi-criteria production and logistics processes planning, integrated approach for modeling processes in engineering, new trends in the multi-criteria evaluation of sustainable processes, multi-criteria decision-making in strategic management based on sustainable criteria.

Este artigo relata experiencias de profissionais e usuarios de servicos da Rede de Atencao Psicossocial (RAPS), nos municipios de Ilheus e Itabuna, os quais estao entre os que lideram as estatisticas em numero de casos da COVID-19 no estado da Bahia, Brasil. Descreve-se a criacao de um grupo de WhatsApp para usuarios de um Centro de Atencao Psicossocial (CAPS), com impactos na proximidade das interacoes entre eles, e entre eles e a equipe e a comunidade. A experiencia e encorajadora, na medida em que a mutua ajuda nasce e produz autonomia e protagonismo. A outra experiencia baseia-se em movimentos de uma gestora da RAPS, para articular usuarios do CAPS e Atencao Primaria a Saude, para apoio emocional e respostas as demandas em momentos de pandemia e isolamento social. Nesses cenarios, as experiencias tem criado e fortalecido novas estrategias de saude mental, ampliando atores sociais, ferramentas e setores, na producao do cuidado na comunidade.

The COVID-19 pandemic and the social distancing implemented shortly after influence physical activity levels (PALs). The purpose of this investigation was to evaluate the changes in PAL and factors associated with PALs among Croatian adolescents while considering the impact of community (urban vs. rural living environment). The sample included 823 adolescents (mean age: 16.5 ± 2.1 years) who were tested on baseline (from October 2019 to March 2020; before COVID-19 pandemic in Croatia) and follow-up (in April 2020; during the COVID-19 pandemic and imposed rules of social distancing). Baseline testing included anthropometrics, physical fitness status, and evaluation of PALs, while follow-up included only PALs (evaluated by a standardized questionnaire through an internet application). The results showed a significant influence of the living environment on the decrease of PAL, with a larger decrease in urban adolescents. Logistic regression showed a higher likelihood for normal PALs at baseline in adolescents who had better fitness status, with no strong confounding effect of the urban/rural environment. The fitness status of urban adolescents predicted their PALs at follow-up. The differences between urban and rural adolescents with regard to the established changes in PALs and relationships between the predictors and PALs are explained by the characteristics of the living communities (lack of organized sports in rural areas), and the level of social distancing in the studied period and region/country.

PurposeThe purpose of this paper is to investigate whether there is a difference in career development programs between family and nonfamily companies. Moreover, the paper explores the relationships between career development dimensions and affective commitment in a family business setting.Design/methodology/approachA cross-sectional questionnaire-based survey was used to collect data from 506 employees in Bosnia and Herzegovina across the different industries. Independentt-test and hierarchical regression were used to test the hypotheses developed in the study.FindingsThe findings supported our assumptions that there is a significant difference in career development opportunities between family and nonfamily companies. Moreover, career development has a higher influence on affective commitment in the family business setting.Research limitations/implicationsThe data for this study was collected by using convenience sampling, as well as a cross-sectional survey method, which limits the generalization of results. Due to the unavailability of a public database, we relied on employees’ perceptions when it comes to the ownership of a company.Practical implicationsTo keep key employees, family business owners and managers have to invest in career development programs. The study shows that by aligning employee and organizational goals, offering professional development and remunerating the positive performance helps in keeping the most valuable assets within the company.Originality/valueSo far, the research about career development in family businesses was insensible. This study provides an important contribution to the understanding of career development and its outcomes in family businesses.