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

Identifying sustainable warehouse management system indicators and proposing new weighting method

The performance of a sustainable warehouse management system is a multidimensional concept based on the triple bottom line approach. It has been a challenge to identify the key performance indicators for a sustainable warehouse management system and to develop a model for evaluating the direct and indirect indicators. In order to overcome this challenge, this paper describes a method to identify and weight indicators that assess sustainability in a sustainable warehouse management system using structural equation modeling. A comprehensive literature review has been conducted and a questionnaire survey involving experts in the field has been undertaken. A list of 33 key performance indicators for a sustainable warehouse management system has been proposed, and this can be used by policymakers to appraise the sustainability performance of a sustainable warehouse management system. The proposed robust model can weight indicators and evaluate the total effect of each indicator in incorporating sustainability in a sustainable warehouse management system. The developed method could also be applied to weighting indicators for other industries

Dynamic failure behavior of glass/epoxy composites under low temperature using Charpy impact test method

211-220This paper demonstrates results of an experimental study on glass/epoxy laminated composites subjected to low velocity impact at energy levels equal to 10, 15 and 30 J under variable temperatures in the range of -30°C to 23°C. The configuration of specimens is quasi-isotropic. The low temperature and its influence on the maximum absorbed energy, elastic energy, crack length and delamination are highlighted. Also, the effects of geometry index (span-to-depth) and notch orientation are studied. Failure mechanisms of specimens are examined using microscopic examinations. Results indicate that impact performance of these composites is affected over the range of temperature considered. Failure mechanism is changed from matrix cracking at room temperature to delamination and fiber breakage at low temperatures

Status of estrogene, progesterone receptors and HER-2/neu expression in invasive breast cancer

Background&Objective: The breast cancer is the most common malignancy in women. The normal and malignant breast tissue are under the regulatory effects of esteroid hormones and growth factors including HER-2/neu. The purpose of this study, is to determine the expression of estrogene receptor (ER) progesterone receptor(PR) and HER-2/neu among Iranian women with invasive breast cancer. Materials&Methods: The study is descriptive and cross-sectional that was performed on 50 samples of patients with invasive breast cancer in Imam-Khomeini Hospital-Sari (2005-06). After preparing the samples four tissue sections from each sample was obtained then H&E and IHC staining were performed. Results: In our study, the patients were between 28-88 yrs, (mean:52/6). ER and PR and HER-2/neu were positive in 80%, 72% and 57.1% of cases, respectively. Concurrent positive ER and PR was 70%, meanwhile 62.9% of these patient were also positive for HER-2/neu. 20% of the cases showed negativity for both ER and PR and of these 83.4% were also negative for HER-2/neu. Conclusion: In our study ER expression by itself is equal to previous studies in textbooks but PR expression, HER-2/neu and concurrent expression of ER&PR are higher in our study. Interestingly HER-2/neu expression is near to what was previously proposed by Iranian Blood Transfusion Organization and Kerman University of Medical science. It seems that racial and geographic factors are contributed for these diffrences

A new method for evaluation of mechanical properties of glass/epoxy composites at low temperatures

Для расчета прочности однонаправленного композита при различных видах нагружения в условиях комнатной температуры и -60°C использованы аналитические модели, учитывающие микромеханические характеристики композитов. В отличие от стандартных методов, базирующихся на результатах испытаний образцов из однонаправленного композита, в данной работе измеряются микромеханические характеристики стекловолокон и эпоксидной матрицы. С помощью разных аналитических моделей выполнен расчет четырех различных модулей упругости и характеристик прочности при комнатной температуре и -60°C.С целью верификации результатов расчета проведены экспериментальные исследования. Показано, что наилучший расчет модулей упругости однонаправленного композита при комнатной и низкой температурах обеспечивает применение упругой модели. Получено хорошее соответствие между расчетными и экспериментальными данными по механическим характеристикам этого материала при исследованных температурах.Розрахунок міцності однонаправленого композита при різних типах навантаження в умовах кімнатної температури і -60°С проводиться за допомогою аналітичних моделей з урахуванням мікромеханічних характеристик композита. На відміну від стандартних методів, що базуються на результатах випробувань зразків з однонаправленого композита, в даній роботі проводиться вимірювання мікромеханічних характеристик скловолокон і епоксидної матриці. За допомогою різних аналітичних моделей виконано розрахунок чотирьох різних модулей пружності і характеристик міцності за кімнатної температури і-60°С. Із метою верифікації результатів розрахунків проведено експериментальні дослідження. Показано, що найкращий розрахунок модулей пружності однонаправленого композита за кімнатної і низької температур забезпечує використання пружної моделі. Отримано хорошу відповідність між розрахунковими й експериментальними даними щодо механічних характеристик цього матеріалу при досліджуваних температурах