Background Wolcott-Rallison syndrome is a rare autosomal recessive disorder characterized by neonatal/early-onset non-autoimmune insulin-dependent diabetes, multiple epiphyseal dysphasia and growth retardation. It is caused by mutations in the gene encoding eukaryotic translation initiation factor 2α kinase 3 (EIF2AK3). We aimed to study the clinical characteristics and frequency of the disease in the Iranian population. Methods We recruited 42 patients who referred to the endocrine and metabolism clinic at Mashhad Imam Reza Hospital with neonatal diabetes. Molecular screening of KCNJ11, INS, ABCC8 and EIF2AK3 was performed at the Exeter Molecular Genetics Laboratory, UK. We calculated the frequency of the disease in 124 patients referred from Iran to the Exeter Molecular Genetics Laboratory for genetic screening and compared it to other countries worldwide. Results We identified seven patients as having Wolcott-Rallison syndrome. Genetic testing confirmed the clinical diagnosis and indicated five novel mutations. Only two patients developed clinical features of the syndrome by 6 months of age. Of all 124 cases of Iranian neonatal diabetes referred to the Exeter Molecular Genetics Laboratory for genetic screening, 28 patients (22.58%) had a recessive mutation in EIF2AK3. Conclusions The results of this study raises awareness of the condition and provides further accurate data on the genetic and clinical presentation of Wolcott-Rallison syndrome in the Iranian population. Our study highlights the importance of genetic testing in patients from consanguineous families with diabetes diagnosed within the first 6 months of life.

Surgical excision using cardiopulmonary bypass combined with medical therapy provides the most optimal treatment for cardiac echinococcosis.

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Objective: Clinical trial research have provided evidence that omega-3 may have larger potential benefits for treating cystic fibrosis (CF). This study's objective was to assess the impact of three supplementation on pediatric CF patients.Methods: Scopus, PubMed/Medline, Web of Science, Cochrane, and Embase databases were searched from commencement until July 20, 2022 using standard keywords to identify all randomized controlled studies (RCTs) examining the effects of omega-3 supplementation on young patients with CF. The eligible studies were subjected to a random-effects model meta-analysis.Results: A meta-analysis of 12 the eligible studies was performed. Findings of the study showed that omega-3 supplementation significantly increased the levels of docosahexaenoic acid (weighted mean [WMD]: 2.06%, 95% confidence interval [CI]:1.29, 2.82, p < 0.001) and eicosapentaenoic acid (WMD: 0.32%, 95% CI: 0.15, 0.48, p < 0.001) as well as decreased arachidonic acid (WMD: −0.78%, 95% CI: −1.50, −0.05, p = 0.035) and C-receptive protein (CRP) (WMD: −3.76 mg/L, 95% CI: −7.42, −0.10, p = 0.044) especially when used in higher doses and for a longer period of time compared to the control group. However, no significant effect was observed on other factors including forced expiratory volume 1, forced vital capacity as well as anthropometric parameters. In addition, high heterogeneity was reported for all fatty acids, but heterogeneity was low and nonsignificant for other variables. Conclusion:The finding showed that in pediatric patients with CF, omega-3 supplementation showed benefits only in plasma fatty acid profile and serum CRP.