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Understanding amateur beekeepers' perception of risks affecting bee health and mortality is essential to analyse the reasons for adopting or rejecting good management practices. A perception survey on how beekeepers perceive and manage factors related to climate change, Varroa infestation, management practices, and pesticide exposure was designed and launched online. This unpreceded sociological survey involved 355 beekeepers spread all over Belgium. A two-sample t test with unequal variances comparing beekeepers with colony loss rates below or exceeding the acceptable level, that is

Background & Aims: The gastrointestinal epithelium plays a crucial role in maintaining homeostasis with the gut microbiome. Mucins are essential for intestinal barrier function and serve as a scaffold for antimicrobial factors. Mucin 2 (MUC2) is the major intestinal gel-forming mucin produced predominantly by goblet cells. Goblet cells express anterior gradient 2 (AGR2), a protein disulfide isomerase that is crucial for proper processing of gel-forming mucins. Here, we investigated 2 siblings who presented with severe infantile-onset inflammatory bowel disease. Methods: We performed whole-genome sequencing to identify candidate variants. We quantified goblet cell numbers using H&E histology and investigated the expression of gel-forming mucins, stress markers, and goblet cell markers using immunohistochemistry. AGR2-MUC2 binding was evaluated using co-immunoprecipitation. Endoplasmic reticulum (ER) stress regulatory function of mutant AGR2 was examined by expression studies in Human Embryonic Kidney 293T (HEK293T) using tunicamycin to induce ER stress. Results: Both affected siblings were homozygous for a missense variant in AGR2. Patient biopsy specimens showed reduced goblet cells; depletion of MUC2, MUC5AC, and MUC6; up-regulation of AGR2; and increased ER stress. The mutant AGR2 showed reduced capacity to bind MUC2 and alleviate tunicamycin-induced ER stress. Conclusions: Phenotype–genotype segregation, functional experiments, and the striking similarity of the human phenotype to AGR2-/- mouse models suggest that the AGR2 missense variant is pathogenic. The Mendelian deficiency of AGR2, termed “Enteropathy caused by AGR2 deficiency, Goblet cell Loss, and ER Stress” (EAGLES), results in a mucus barrier defect, the inability to mitigate ER stress, and causes infantile-onset inflammatory bowel disease.

The I48TN is one of the 60 International Monitoring System (IMS) stations of the Comprehensive nuclear Test Ban Treaty Organization (CTBTO), characterized by its location in the heart of the IMS Infrasound network. The ability of the International Monitoring System (IMS) infrasound network to detect atmospheric nuclear explosions and other signals of interest is strongly dependent on station-specific ambient noise. This ambient noise, includes both incoherent wind noise and real coherent infrasonic waves. Infrasound analysis software detects tens to hundreds of events per day which consume a lot of time for the Infrasound analysts, to define and categorize events where around 90% of the detections are coherent noise. This study analyzed the importance of the synergy between infrasound and seismic data, and provided the infrasound data analyst with the most important local coherent infrasound sources in the region as recorded by the IMS station I48TN, in order to reduce the workload of the analysts and give them a clear view on the coherent noise affecting this station for better discrimination between events of interest like nuclear explosions and coherent sources. DTK_GPMCC and DIVA software were used to perform this study. Geotool software from the International Data Centre (IDC) was used in analysing seismic data from the Tunisian IMS station KEST. The result of this study allowed the characterization of the most important coherent local infrasound sources (Mines and Quarries) which are considered as coherent noise to I48TN station and correct parameters in some reference events in the Reference Event Database source of the International Data Centre.

Hamrouni Salwa, Tabei Mouna, Agrebi Meriem. Tunisie. In: Annuaire international de justice constitutionnelle, 36-2020, 2021. L'état d'exception, nouveau régime de droit commun des droits et libertés? Du terrorisme à l'urgence sanitaire- L'opinion publique aujourd'hui. Regards pluridisciplinaires. pp. 1123-1136.

International audience; Local mechanical properties of submicron features are of particular interest due to their influence on macroscopic material performance and behaviour. This study is focused on local nanomechanical measurements, based on the latest Atomic Force Microscopy (AFM) mode, where the peak force set point is finely controlled at each pixel. After probe calibration, we evaluate the impact of spring constant of two AFM hand-crafted natural full diamond tips with steel cantilevers, used for mapping. Based on the fast capture of the cantilever deflection at each pixel and real time force curve analysis in the elastic region, AFM local measured contact moduli mappings of the silica beads ('50 GPa) incorporated in an epoxy resin matrix, are compared with those determined using classical instrumented nanoindentation tests. Our analyses show that with the two AFM probes, without local residual deformation, the high moduli of the silica beads measured with this advanced AFM mode are within the standard deviation of the values determined by classical nanoindentation. Lay Description: The knowledge of material properties at the nanometer scale is a key parameter for well understanding and determining the behavior of material at macroscopic scale. In this paper, we compare two methods (an advanced mode and a classical one) based on the analysis of probes in interaction with the surface of studied material. We focus on a latest developed mode for determining local mechanical properties with a very high spatial resolution. For the advanced mode, we also consider two different hand-crafted probes. Our analyses show that with the high spatial resolution advanced mode, local mechanical properties are well determined. We also highlight the impact of the properties of the used probes for this advanced mode. In a final step, the power of the presented investigation lies in the fact that it does not modify the topography of the surface.

BACKGROUND AND AIMS Systemic AL amyloidosis is a rare pathology associated with the precipitation of monoclonal immunoglobulin light chains in the form of fibrils in various organs. Clinical presentations are extremely variable and diagnosis is often delayed. The two most affected organs are the kidney and the heart. The treatment is based on chemotherapy to eliminate the medullary clone that produces the monoclonal light chain. However, the outcome is still poor. The aim of our study was to describe the demographic, clinicobiological, histological, therapeutic and evolutionary characteristics of AL amyloidosis in our population. METHOD We carried out a single-center retrospective descriptive trial of patients who were diagnosed with histologically confirmed AL amyloidosis in our Nephrology Department during the period from 2014 to 2021. RESULTS We enrolled 12 patients with a mean age of 61.25 ± 8.17years. The sex ratio (M/F) was 3. The histological confirmation of AL amyloidosis was based on biopsies: kidney (n = 7) and salivary glands (n = 5). The immunoglobulin type was Ig G Lambda in 75% of cases (n = 9), IgG kappa in 16.6% of cases (n = 2) and IgM in 8.3% of cases (n = 1). The cause of amyloidosis was multiple myeloma in 66.6% of cases (n = 8) and Waldenström disease in 8.3% of cases (n = 1). The mean creatinine at diagnosis was 441.33 ± 334.47 umol/L, i.e. a median creatinine clearance (MDRD) of 36.67 ± 46.77 mL/min. At the time of diagnosis, 50% of the patients (n = 6) had end-stage renal disease and 25% (n = 3) had reached it after a few more months. The mean proteinuria was 6.33 g/24 h within 83.3% of cases (n = 10) with the presence of a nephrotic syndrome. The cardiac involvement was found in 58% of the patients (n = 7) at the time of diagnosis with a Mayo Clinic prognostic score of III. The neurological involvement was observed in 8.3% of cases (n = 1) and was confirmed by spinal MRI as polyradiculoneuritis. As for the therapeutical management, 75% of the patients (n = 9) received first-line treatment with Bortezomib Cyclophosphamide Dexamethasone, and 16.6 of the patients (n = 2) were treated with corticosteroids and Melphalan (the 12th patient died early). The outcome was fatal in 41.5% (n = 5) and the cause of death was a state of septic shock, and the rest of the patients are alive with a mean follow-up of 1 year. CONCLUSION AL Amyloidosis is one of the most severe complications of monoclonal lymphocyte proliferation. This overgrowth is most frequently related to myeloma, and rarely to another malignant B-cell disease. The prognosis is related to cardiac and renal impairment. It requires urgent management to improve its outcome and to reduce mortality.

This paper focuses on the control of permanent magnet synchronous motors (PMSMs) utilizing a non-linear adaptive PI-Backstepping design and a model of harmonics reduction approach that uses an active shunt filter followed by a cascade bandpass filter. While conventional backstepping may assure the system's stability, it is often imprecise. It creates a significant amount of static error, which has a detrimental effect on the system's behavior, such as disruptions and loads that might arise in industrial settings. We can assure minimum fixed errors and considerable interaction with uncertainty by integrating the PI controller with adaptive backstepping through robust Lyapunov functions. Numerical simulations are used to demonstrate the proposed controller's effectiveness.

Store-operated Ca2+ entry (SOCE) represents a predominant Ca2+ influx pathway in non-excitable cells. SOCE is required for immune cell activation and is mediated by the plasma membrane (PM) channel ORAI1 and the endoplasmic reticulum (ER) Ca2+ sensor STIM1. Mutations in the Orai1 or STIM1 genes abolish SOCE leading to combined immunodeficiency (CID), muscular hypotonia, and anhidrotic ectodermal dysplasia. Here, we identify a novel autosomal recessive mutation in ORAI1 in a child with CID. The patient is homozygous for p.C126R mutation in the second transmembrane domain (TM2) of ORAI1, a region with no previous loss-of-function mutations. SOCE is suppressed in the patient’s lymphocytes, which is associated with impaired T cell proliferation and cytokine production. Functional analyses demonstrate that the p.C126R mutation does not alter protein expression but disrupts ORAI1 trafficking. Orai1-C126R does not insert properly into the bilayer resulting in ER retention. Insertion of an Arg on the opposite face of TM2 (L135R) also results in defective folding and trafficking. We conclude that positive side chains within ORAI1 TM2 are not tolerated and result in misfolding, defective bilayer insertion, and channel trafficking thus abolishing SOCE and resulting in CID.Other Information Published in: Journal of Clinical Immunology License: https://creativecommons.org/licenses/by/4.0See article on publisher's website: http://dx.doi.org/10.1007/s10875-021-01004-8

The behaviors of spray, in Reactivity Controlled Combustion Ignition (RCCI) dual fuel engine and subsequent emissions formation, are numerically addressed. Five spray cone angles ranging between 5° and 25° with an advanced injection timing of 22° Before Top Dead Center (BTDC) are considered. The objective of this paper is twofold: (a) to enhance engine behaviors in terms of performances and consequent emissions by adjusting spray cone angle and (b) to outcome the exergy efficiency for each case. The simulations are conducted using the Ansys-forte tool. The turbulence model is the Renormalization Group (RNG) K-epsilon, which is selected for its effectiveness in strongly sheared flows. The spray breakup is governed by the hybrid model Kelvin–Helmholtz and Rayleigh–Taylor spray models. A surrogate of n-heptane, which contains 425 species and 3128 reactions, is used for diesel combustion modeling. The obtained results for methane/diesel engine combustion, under low load operating conditions, include the distribution of heat transfer flux, pressure, temperature, Heat Release Rate (HRR), and Sauter Mean Diameter (SMD). An exergy balance analysis is conducted to quantify the engine performances. Output emissions at the outlet of the combustion chamber are also monitored in this work. Investigations show a pressure decrease for a cone angle θ = 5° of roughly 8%, compared to experimental measurement (θ = 10°). A broader cone angle produces a higher mass of NOₓ. The optimum spray cone angle, in terms of exergy efficiency, performance, and consequent emissions is found to lie at 15° ≤ θ ≤ 20°.

In this paper, the analytical modeling of a wind turbine conversion chain is carried out according to multi-physical disciplines of the system. The chain represents a 3 bladed horizontal axis wind turbine coupled directly to a permanent magnet synchronous generator PMSG delivering power in a battery through diode rectifier. For the considered chain, four models are developed. Mechanical model describes the power generation from kinetic movement of the wind to its transmission to the generator ...