6 research outputs found

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

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    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

    Phytoremediation Efficiency of Sorghum bicolor (L.) Moench in Removing Cadmium, Lead and Arsenic

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    Heavy metals are a significant problem in municipal wastewater, in soil accumulation and are costly to remove in order to facilitate water use in irrigation. Wastewater, with three heavy metal cations and an anion, was employed in irrigation during the Sorghum bicolor (L.) Moench growth period. Results show that the absorption coefficient or bioaccumulation ability of Sorghum bicolor (L.) Moench is relegated to certain heavy metals and their concentrations, but not at all to plant tissues in dry ash weight (DAW) scale. Heavy metals absorption was determined in the following order: cadmium = lead &gt; arsenic, and while tissue accumulation based on DAW was equal for cadmium and lead, based on dry weight (DW) all three ions differed. The soil’s ability to accumulate heavy metal ranked as follows: cadmium &gt; lead&gt; arsenic by wastewater quality in this experimvent. These results change many previous ideas about decreasing the transportation of heavy metals from root to shoot or other organs in plants. With low cadmium and lead concentration in irrigation, sorghum is a good plant for remediation; however, in high concentration this plant benefits from arsenic remediation. Soil is a critical parameter for wastewater phytoremediation. This topic merits further research.</p

    Fourth Update on the Iranian National Registry of Primary Immunodeficiencies: Integration of Molecular Diagnosis

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