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- Delavari, S, et al.
(författare)
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Impact of SARS-CoV-2 Pandemic on Patients with Primary Immunodeficiency
- 2021
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Ingår i: Journal of clinical immunology. - : Springer Science and Business Media LLC. - 1573-2592 .- 0271-9142. ; 41:2, s. 345-355
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Tidskriftsartikel (refereegranskat)abstract
- Although it is estimated that COVID-19 life-threatening conditions may be diagnosed in less than 1:1000 infected individuals below the age of 50, but the real impact of this pandemic on pediatric patients with different types of primary immunodeficiency (PID) is not elucidated. The current prospective study on a national registry of PID patients showed that with only 1.23 folds higher incidence of infections, these patients present a 10-folds higher mortality rate compared to population mainly in patients with combined immunodeficiency and immune dysregulation. Therefore, further management modalities against COVID-19 should be considered to improve the survival rate in these two PID entities using hematopoietic stem cell transplantation and immunomodulatory agents.
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- Fatahi, R., et al.
(författare)
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Ventilation Prediction for an Industrial Cement Raw Ball Mill by BNN—A “Conscious Lab” Approach
- 2021
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Ingår i: Materials. - : MDPI. - 1996-1944. ; 14:12
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Tidskriftsartikel (refereegranskat)abstract
- In cement mills, ventilation is a critical key for maintaining temperature and material transportation. However, relationships between operational variables and ventilation factors for an industrial cement ball mill were not addressed until today. This investigation is going to fill this gap based on a newly developed concept named “conscious laboratory (CL)”. For constructing the CL, a boosted neural network (BNN), as a recently developed comprehensive artificial intelligence model, was applied through over 35 different variables, with more than 2000 records monitored for an industrial cement ball mill. BNN could assess multivariable nonlinear relationships among this vast dataset, and indicated mill outlet pressure and the ampere of the separator fan had the highest rank for the ventilation prediction. BNN could accurately model ventilation factors based on the operational variables with a root mean square error (RMSE) of 0.6. BNN showed a lower error than other traditional machine learning models (RMSE: random forest 0.71, support vector regression: 0.76). Since improving the milling efficiency has an essential role in machine development and energy utilization, these results can open a new window to the optimal designing of comminution units for the material technologies.
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| 30. |
- Sharifinejad, N, et al.
(författare)
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Autoimmunity in monogenic combined immune deficiencies with associated or syndromic features
- 2022
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Ingår i: Frontiers in immunology. - : Frontiers Media SA. - 1664-3224. ; 13, s. 1023127-
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Tidskriftsartikel (refereegranskat)abstract
- Combined immune deficiencies (CIDs) with associated or syndromic features are a highly heterogeneous subgroup of inherited immune disorders. These patients represent specific clinical complications with an increased risk of autoimmune conditions.MethodsWe analyzed data of monogenic patients with syndromic CIDs adopted from the Iranian inborn errors of immunity registry up to January 2022. A comprehensive comparison in terms of demographic, clinical, and immunological features was performed between patients with and without autoimmunity and also among four mutation groups with the most registered cases including ATM, STAT3 (AD-LOF), DNMT3B/ZBTB24, and WAS mutations.ResultsA total of 137 patients with monogenic syndromic CIDs were included. Most commonly mutated genes were the ATM [80 (58.4%)] and STAT3 (AD-LOF) [19 (13.9%)], followed by DNMT3B [11 (8%)], and WAS [11 (8%)]. More than 18% of all patients with syndromic CIDs, including most DNMT3B/ZBTB24 mutations patients, were clinically diagnosed with antibody deficiencies before genetic evaluation. Patients with ATM and WAS mutations had the latest age of onset and the lowest age of diagnosis, respectively. Autoimmune disorders were diagnosed in 24 patients at a median age of 3.5 (2.6-6.0) years, 70.6% of which were diagnosed prior to the diagnosis of immunodeficiency. Lymphoproliferation, particularly hepatosplenomegaly, was significantly higher in patients with autoimmunity (p=0.004). Syndromic CID patients with autoimmunity had significantly lower IgG levels. Hematologic autoimmunity mainly immune thrombocytopenic purpura was the most frequent autoimmunity among major groups of ATM, STAT3 (AD-LOF), DNMT3B/ZBTB24, and WAS mutations, however ATM-mutated patients present more diversified involved organs including rheumatologic, gastrointestinal and dermatologic autoimmunity.ConclusionAbout 18% of patients with monogenic syndromic CIDs developed autoimmunity, mainly in the form of hematological immune diseases. Autoimmunity could be an early-onset involvement with a potential diagnostic impact on suspicious cases of syndromic CIDs.
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