Consensus Middle East and North Africa Registry on Inborn Errors of Immunity

Background: Inborn errors of immunity (IEIs) are a heterogeneous group of genetic defects of immunity, which cause high rates of morbidity and mortality mainly among children due to infectious and non-infectious complications. The IEI burden has been critically underestimated in countries from middle- and low-income regions and the majority of patients with IEI in these regions lack a molecular diagnosis. Methods: We analyzed the clinical, immunologic, and genetic data of IEI patients from 22 countries in the Middle East and North Africa (MENA) region. The data was collected from national registries and diverse databases such as the Asian Pacific Society for Immunodeficiencies (APSID) registry, African Society for Immunodeficiencies (ASID) registry, Jeffrey Modell Foundation (JMF) registry, J Project centers, and International Consortium on Immune Deficiency (ICID) centers. Results: We identified 17,120 patients with IEI, among which females represented 39.4%. Parental consanguinity was present in 60.5% of cases and 27.3% of the patients were from families with a confirmed previous family history of IEI. The median age of patients at the onset of disease was 36 months and the median delay in diagnosis was 41 months. The rate of registered IEI patients ranges between 0.02 and 7.58 per 100,000 population, and the lowest rates were in countries with the highest rates of disability-adjusted life years (DALY) and death rates for children. Predominantly antibody deficiencies were the most frequent IEI entities diagnosed in 41.2% of the cohort. Among 5871 patients genetically evaluated, the diagnostic yield was 83% with the majority (65.2%) having autosomal recessive defects. The mortality rate was the highest in patients with non-syndromic combined immunodeficiency (51.7%, median age: 3.5 years) and particularly in patients with mutations in specific genes associated with this phenotype (RFXANK, RAG1, and IL2RG). Conclusions: This comprehensive registry highlights the importance of a detailed investigation of IEI patients in the MENA region. The high yield of genetic diagnosis of IEI in this region has important implications for prevention, prognosis, treatment, and resource allocation

Comparing the effect of progressive muscle relaxation exercise and support group therapy on the happiness of nursing students: A randomized clinical trial study

Background: Some nursing students might experience lower and median levels of happiness; hence, providing some interventions would be crucial for them. Objective: This study aimed to compare the effects of Progressive Muscle Relaxation (PMR) exercise and support group therapy on happiness amongst nursing students. Design: This was a randomized controlled trial study. Participants: In this study, 150 nursing students participated. They were selected from nursing and midwifery school of Shiraz University of Medical Sciences. Methods: 150 nursing students were divided into three groups (PMR exercise (n = 50), support group therapy (n = 50), and control (n = 50)). PMR exercise and support group therapy were carried out over five consecutive 45-minute sessions per week for 50 participants in groups of 10. Oxford Happiness Inventory was used before and after the interventions. In order to compare the mean score of happiness between two and three groups, t-test, and ANCOVA were used, respectively. Results: After the intervention, the mean scores of happiness were 55.26 (SD = 16.26), 35.16 (SD = 12.69), and 34.22 (SD = 11.83) in the PMR exercise, support group therapy, and control groups, respectively. The results indicated a significant difference amongst the three groups regarding happiness after the intervention (p < 0.05). A significant difference was observed between the PMR exercise and the control group regarding happiness (p < 0.05). In addition, the mean score of happiness was significantly higher in the PMR exercise group compared to the support group therapy after the intervention (p < 0.05). In the PMR exercise group, a significant difference was found regarding the mean change of students� happiness before and after the intervention (t = -4.29, p < 0.001). Conclusion: Considering the positive effects of PMR exercise and support group therapy in increasing nursing students� happiness, educational authorities and educators are recommended to use these therapies during the course of nursing� Bachelor's degree to increase happiness within students and positive outlooks amongst groups. © 202

Nurses' perspectives on technical skill requirements in primary and tertiary healthcare services

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