University of Hassan II Casablanca

Author(s): Collaboration, The ATLAS; Aad, G; Abat, E; Abdallah, J; Abdelalim, AA; Abdesselam, A; Abdinov, O; Abi, BA; Abolins, M; Abramowicz, H; Acerbi, E; Acharya, BS; Achenbach, R; Ackers, M; Adams, DL; Adamyan, F; Addy, TN; Aderholz, M; Adorisio, C; Adragna, P; Aharrouche, M; Ahlen, SP; Ahles, F; Ahmad, A; Ahmed, H; Aielli, G; Åkesson, PF; Åkesson, TPA; Akimov, AV; Alam, SM; Albert, J; Albrand, S; Aleksa, M; Aleksandrov, IN; Aleppo, M; Alessandria, F; Alexa, C; Alexander, G; Alexopoulos, T; Alimonti, G; Aliyev, M; Allport, PP; Allwood-Spiers, SE; Aloisio, A; Alonso, J; Alves, R; Alviggi, MG; Amako, K; Amaral, P; Amaral, SP; Ambrosini, G; Ambrosio, G; Amelung, C; Ammosov, VV; Amorim, A; Amram, N; Anastopoulos, C; Anderson, B; Anderson, KJ; Anderssen, EC; Andreazza, A; Andrei, V; Andricek, L; Andrieux, M-L; Anduaga, XS; Anghinolfi, F; Antonaki, A; Antonelli, M; Antonelli, S; Apsimon, R; Arabidze, G; Aracena, I; Arai, Y; Arce, ATH; Archambault, JP; Arguin, J-F; Arik, E; Arik, M; Arms, KE; Armstrong, SR; Arnaud, M; Arnault, C; Artamonov, A; Asai, S; Ask, S

The Large Hadron Collider (LHC) at CERN will extend the frontiers of particle physics with its
\nunprecedented high energy and luminosity. Inside the LHC, bunches of up to 1011 protons (p)
\nwill collide 40 million times per second to provide 14 TeV proton-proton collisions at a design
\nluminosity of 1034 cm􀀀2s􀀀1. The LHC will also collide heavy ions (A), in particular lead nuclei, at
\n5.5 TeV per nucleon pair, at a design luminosity of 1027 cm􀀀2s􀀀1.
\nThe high interaction rates, radiation doses, particle multiplicities and energies, as well as the
\nrequirements for precision measurements have set new standards for the design of particle detectors.
\nTwo general purpose detectors, ATLAS (A Toroidal LHC ApparatuS) and CMS (Compact
\nMuon Solenoid) have been built for probing p-p and A-A collisions.
\nThis paper presents a comprehensive overview of the ATLAS detector prior to the first LHC
\ncollisions, written as the installation of the ATLAS detector is nearing completion. This detector
\nrepresents the work of a large collaboration of several thousand physicists, engineers, technicians,
\nand students over a period of fifteen years of dedicated design, development, fabrication, and installation.

A measurement of the Higgs boson mass is presented based on the combined data samples of the ATLAS and CMS experiments at the CERN LHC in the H→γγ and H→ZZ→4ℓ decay channels. The results are obtained from a simultaneous fit to the reconstructed invariant mass peaks in the two channels and for the two experiments. The measured masses from the individual channels and the two experiments are found to be consistent among themselves. The combined measured mass of the Higgs boson is m_{H}=125.09±0.21 (stat)±0.11 (syst) GeV.

We report the updated classification of Inborn Errors of Immunity/Primary Immunodeficiencies, compiled by the International Union of Immunological Societies Expert Committee. This report documents the key clinical and laboratory features of 430 inborn errors of immunity, including 64 gene defects that have either been discovered in the past 2 years since the previous update (published January 2018) or were characterized earlier but have since been confirmed or expanded upon in subsequent studies. The application of next-generation sequencing continues to expedite the rapid identification of novel gene defects, rare or common; broaden the immunological and clinical phenotypes of conditions arising from known gene defects and even known variants; and implement gene-specific therapies. These advances are contributing to greater understanding of the molecular, cellular, and immunological mechanisms of disease, thereby enhancing immunological knowledge while improving the management of patients and their families. This report serves as a valuable resource for the molecular diagnosis of individuals with heritable immunological disorders and also for the scientific dissection of cellular and molecular mechanisms underlying inborn errors of immunity and related human diseases.

We report the updated classification of primary immunodeficiencies compiled by the Primary Immunodeficiency Expert Committee (PID EC) of the International Union of Immunological Societies (IUIS). In the two years since the previous version, 34 new gene defects are reported in this updated version. For each disorder, the key clinical and laboratory features are provided. In this new version we continue to see the increasing overlap between immunodeficiency, as manifested by infection and/or malignancy, and immune dysregulation, as manifested by auto-inflammation, auto-immunity, and/or allergy. There is also an increased number of genetic defects that lead to susceptibility to specific organisms which reflects the finely tuned nature of immune defense systems. This classification is the most up to date catalogue of all known and published primary immunodeficiencies and acts as a current reference of the knowledge of these conditions and is an important aid for the genetic and molecular diagnosis of patients with these rare diseases.

We report the updated classification of inborn errors of immunity, compiled by the International Union of Immunological Societies Expert Committee. This report documents the key clinical and laboratory features of 55 novel monogenic gene defects, and 1 phenocopy due to autoantibodies, that have either been discovered since the previous update (published January 2020) or were characterized earlier but have since been confirmed or expanded in subsequent studies. While variants in additional genes associated with immune diseases have been reported in the literature, this update includes only those that the committee assessed that reached the necessary threshold to represent novel inborn errors of immunity. There are now a total of 485 inborn errors of immunity. These advances in discovering the genetic causes of human immune diseases continue to significantly further our understanding of molecular, cellular, and immunological mechanisms of disease pathogenesis, thereby simultaneously enhancing immunological knowledge and improving patient diagnosis and management. This report is designed to serve as a resource for immunologists and geneticists pursuing the molecular diagnosis of individuals with heritable immunological disorders and for the scientific dissection of cellular and molecular mechanisms underlying monogenic and related human immune diseases.

Combined ATLAS and CMS measurements of the Higgs boson production and decay rates, as well as constraints on its couplings to vector bosons and fermions, are presented. The combination is based on the analysis of five production processes, namely gluon fusion, vector boson fusion, and associated production with a W or a Z boson or a pair of top quarks, and of the six decay modes H → ZZ, W W , γγ, ττ, bb, and μμ. All results are reported assuming a value of 125.09 GeV for the Higgs boson mass, the result of the combined measurement by the ATLAS and CMS experiments. The analysis uses the CERN LHC proton-proton collision data recorded by the ATLAS and CMS experiments in 2011 and 2012, corresponding to integrated luminosities per experiment of approximately 5 fb$^{−1}$ at $\sqrt{s}$=7 TeV and 20 fb−1 at $\sqrt{s}$=8 TeV. The Higgs boson production and decay rates measured by the two experiments are combined within the context of three generic parameterisations: two based on cross sections and branching fractions, and one on ratios of coupling modifiers. Several interpretations of the measurements with more model-dependent parameterisations are also given. The combined signal yield relative to the Standard Model prediction is measured to be 1.09 ± 0.11. The combined measurements lead to observed significances for the vector boson fusion production process and for the H → ττ decay of 5.4 and 5.5 standard deviations, respectively. The data are consistent with the Standard Model predictions for all parameterisations considered.

Beginning in 1970, a committee was constituted under the auspices of the World Health Organization (WHO) to catalog primary immunodeficiencies. Twenty years later, the International Union of Immunological Societies (IUIS) took the remit of this committee. The current report details the categorization and listing of 354 (as of February 2017) inborn errors of immunity. The growth and increasing complexity of the field have been impressive, encompassing an increasing variety of conditions, and the classification described here will serve as a critical reference for immunologists and researchers worldwide.

We report the updated classification of primary immunodeficiencies (PIDs) compiled by the Expert Committee of the International Union of Immunological Societies. In comparison to the previous version, more than 30 new gene defects are reported in this updated version. In addition, we have added a table of acquired defects that are phenocopies of PIDs. For each disorder, the key clinical and laboratory features are provided. This classification is the most up-to-date catalog of all known PIDs and acts as a current reference of the knowledge of these conditions and is an important aid for the molecular diagnosis of patients with these rare diseases.

During 2015 the ATLAS experiment recorded [Formula: see text] of proton-proton collision data at a centre-of-mass energy of [Formula: see text]. The ATLAS trigger system is a crucial component of the experiment, responsible for selecting events of interest at a recording rate of approximately 1 kHz from up to 40 MHz of collisions. This paper presents a short overview of the changes to the trigger and data acquisition systems during the first long shutdown of the LHC and shows the performance of the trigger system and its components based on the 2015 proton-proton collision data.

Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification into ten tables. This phenotypic classification is user-friendly and serves as a resource for clinicians at the bedside. There are now 430 single-gene IEI underlying phenotypes as diverse as infection, malignancy, allergy, autoimmunity, and autoinflammation. We herein report the 2019 phenotypic classification, including the 65 new conditions. The diagnostic algorithms are based on clinical and laboratory phenotypes for each of the ten broad categories of IEI.

Circulating autoantibodies (auto-Abs) neutralizing high concentrations (10 ng/mL, in plasma diluted 1 to 10) of IFN-α and/or -ω are found in about 10% of patients with critical COVID-19 pneumonia, but not in subjects with asymptomatic infections. We detect auto-Abs neutralizing 100-fold lower, more physiological, concentrations of IFN-α and/or -ω (100 pg/mL, in 1/10 dilutions of plasma) in 13.6% of 3,595 patients with critical COVID-19, including 21% of 374 patients > 80 years, and 6.5% of 522 patients with severe COVID-19. These antibodies are also detected in 18% of the 1,124 deceased patients (aged 20 days-99 years; mean: 70 years). Moreover, another 1.3% of patients with critical COVID-19 and 0.9% of the deceased patients have auto-Abs neutralizing high concentrations of IFN-β. We also show, in a sample of 34,159 uninfected subjects from the general population, that auto-Abs neutralizing high concentrations of IFN-α and/or -ω are present in 0.18% of individuals between 18 and 69 years, 1.1% between 70 and 79 years, and 3.4% >80 years. Moreover, the proportion of subjects carrying auto-Abs neutralizing lower concentrations is greater in a subsample of 10,778 uninfected individuals: 1% of individuals <70 years, 2.3% between 70 and 80 years, and 6.3% >80 years. By contrast, auto-Abs neutralizing IFN-β do not become more frequent with age. Auto-Abs neutralizing type I IFNs predate SARS-CoV-2 infection and sharply increase in prevalence after the age of 70 years. They account for about 20% of both critical COVID-19 cases in the over-80s, and total fatal COVID-19 cases.

Since their discovery in patients with autosomal dominant (AD) chronic mucocutaneous candidiasis (CMC) in 2011, heterozygous STAT1 gain-of-function (GOF) mutations have increasingly been identified worldwide. The clinical spectrum associated with them needed to be delineated. We enrolled 274 patients from 167 kindreds originating from 40 countries from 5 continents. Demographic data, clinical features, immunological parameters, treatment, and outcome were recorded. The median age of the 274 patients was 22 years (range, 1-71 years); 98% of them had CMC, with a median age at onset of 1 year (range, 0-24 years). Patients often displayed bacterial (74%) infections, mostly because of Staphylococcus aureus (36%), including the respiratory tract and the skin in 47% and 28% of patients, respectively, and viral (38%) infections, mostly because of Herpesviridae (83%) and affecting the skin in 32% of patients. Invasive fungal infections (10%), mostly caused by Candida spp. (29%), and mycobacterial disease (6%) caused by Mycobacterium tuberculosis, environmental mycobacteria, or Bacille Calmette-Guérin vaccines were less common. Many patients had autoimmune manifestations (37%), including hypothyroidism (22%), type 1 diabetes (4%), blood cytopenia (4%), and systemic lupus erythematosus (2%). Invasive infections (25%), cerebral aneurysms (6%), and cancers (6%) were the strongest predictors of poor outcome. CMC persisted in 39% of the 202 patients receiving prolonged antifungal treatment. Circulating interleukin-17A-producing T-cell count was low for most (82%) but not all of the patients tested. STAT1 GOF mutations underlie AD CMC, as well as an unexpectedly wide range of other clinical features, including not only a variety of infectious and autoimmune diseases, but also cerebral aneurysms and carcinomas that confer a poor prognosis.

collision data, measurements of the reconstruction efficiency, as well as of the momentum scale and resolution, are presented and compared to Monte Carlo simulations. The reconstruction efficiency is measured to be close to [Formula: see text] over most of the covered phase space ([Formula: see text] and [Formula: see text] GeV). The isolation efficiency varies between 93 and [Formula: see text] depending on the selection applied and on the momentum of the muon. Both efficiencies are well reproduced in simulation. In the central region of the detector, the momentum resolution is measured to be [Formula: see text] ([Formula: see text]) for muons from [Formula: see text] ([Formula: see text]) decays, and the momentum scale is known with an uncertainty of [Formula: see text]. In the region [Formula: see text], the [Formula: see text] resolution for muons from [Formula: see text] decays is [Formula: see text] while the precision of the momentum scale for low-[Formula: see text] muons from [Formula: see text] decays is about [Formula: see text].

Two-particle correlations in relative azimuthal angle ($\ensuremath{\Delta}\ensuremath{\phi}$) and pseudorapidity ($\ensuremath{\Delta}\ensuremath{\eta}$) are measured in $\sqrt{{s}_{\mathrm{NN}}}=5.02\text{ }\text{ }\mathrm{TeV}$ $p+\mathrm{Pb}$ collisions using the ATLAS detector at the LHC. The measurements are performed using approximately $1\text{ }\text{ }\ensuremath{\mu}{\mathrm{b}}^{\ensuremath{-}1}$ of data as a function of transverse momentum (${p}_{\mathrm{T}}$) and the transverse energy ($\ensuremath{\Sigma}{E}_{\mathrm{T}}^{\mathrm{Pb}}$) summed over $3.1&lt;\ensuremath{\eta}&lt;4.9$ in the direction of the Pb beam. The correlation function, constructed from charged particles, exhibits a long-range ($2&lt;|\ensuremath{\Delta}\ensuremath{\eta}|&lt;5$) ``near-side'' ($\ensuremath{\Delta}\ensuremath{\phi}\ensuremath{\sim}0$) correlation that grows rapidly with increasing $\ensuremath{\Sigma}{E}_{\mathrm{T}}^{\mathrm{Pb}}$. A long-range ``away-side'' ($\ensuremath{\Delta}\ensuremath{\phi}\ensuremath{\sim}\ensuremath{\pi}$) correlation, obtained by subtracting the expected contributions from recoiling dijets and other sources estimated using events with small $\ensuremath{\Sigma}{E}_{\mathrm{T}}^{\mathrm{Pb}}$, is found to match the near-side correlation in magnitude, shape (in $\ensuremath{\Delta}\ensuremath{\eta}$ and $\ensuremath{\Delta}\ensuremath{\phi}$) and $\ensuremath{\Sigma}{E}_{\mathrm{T}}^{\mathrm{Pb}}$ dependence. The resultant $\ensuremath{\Delta}\ensuremath{\phi}$ correlation is approximately symmetric about $\ensuremath{\pi}/2$, and is consistent with a dominant $\mathrm{cos}2\ensuremath{\Delta}\ensuremath{\phi}$ modulation for all $\ensuremath{\Sigma}{E}_{\mathrm{T}}^{\mathrm{Pb}}$ ranges and particle ${p}_{\mathrm{T}}$.

Since the 1990s, the International Union of Immunological Societies (IUIS) PID expert committee (EC), now called Inborn Errors of Immunity Committee, has published every other year a classification of the inborn errors of immunity. This complete catalog serves as a reference for immunologists and researchers worldwide. However, it was unadapted for clinicians at the bedside. For those, the IUIS PID EC is now publishing a phenotypical classification since 2013, which proved to be more user-friendly. There are now 320 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. We herein propose the revised 2017 phenotypic classification, based on the accompanying 2017 IUIS Inborn Errors of Immunity Committee classification.

Atomically 2D thin-layered structures, such as graphene nanosheets, graphitic carbon nitride nanosheets (g-C3N4), hexagonal boron nitride, and transition metal dichalcogenides are emerging as fascinating materials for a good array of domains owing to their rare physicochemical characteristics. In particular, graphitic carbon nitride has turned into a hot subject in the scientific community due to numerous qualities such as simple preparation, electrochemical properties, high adsorption capacity, good photochemical properties, thermal stability, and acid-alkali chemical resistance, etc. Basically, g-C3N4 is considered as a polymeric material consisting of N and C atoms forming a tri-s-triazine network connected by planar amino groups. In comparison with most C-based materials, g-C3N4 possesses electron-rich characteristics, basic moieties, and hydrogen-bonding groups owing to the presence of hydrogen and nitrogen atoms; therefore, it is taken into account as an interesting nominee to further complement carbon in applications of functional materials. Nevertheless, g-C3N4 has some intrinsic limitations and drawbacks mainly related to a relatively poor specific surface area, rapid charge recombination, a limited light absorption range, and a poor dispersibility in both aqueous and organic mediums. To overcome these shortcomings, numerous chemical modification approaches have been conducted with the aim of expanding the range of application of g-C3N4 and enhancing its properties. In the current review, the comprehensive survey is conducted on g-C3N4 chemical functionalization strategies including covalent and noncovalent approaches. Covalent approaches consist of establishing covalent linkage between the g-C3N4 structure and the chemical modifier such as oxidation/carboxylation, amidation, polymer grafting, etc., whereas the noncovalent approaches mainly consist of physical bonding and intermolecular interaction such as van der Waals interactions, electrostatic interactions, π–π interactions, and so on. Furthermore, the preparation, characterization, and diverse applications of functionalized g-C3N4 in various domains are described and recapped. We believe that this work will inspire scientists and readers to conduct research with the aim of exploring other functionalization strategies for this material in numerous applications.

Rationale: In addition to the overwhelming lung inflammation that prevails in coronavirus disease 2019 (COVID-19), hypercoagulation and thrombosis contribute to the lethality of subjects infected with severe acute respiratory syndrome coronavirus 2. Platelets are chiefly implicated in thrombosis. Moreover, they can interact with viruses and are an important source of inflammatory mediators. While a lower platelet count is associated with severity and mortality, little is known about platelet function during COVID-19. Objective: To evaluate the contribution of platelets to inflammation and thrombosis in patients with COVID-19. Methods and Results: Blood was collected from 115 consecutive patients with COVID-19 presenting nonsevere (n=71) and severe (n=44) respiratory symptoms. We document the presence of severe acute respiratory syndrome coronavirus 2 RNA associated with platelets of patients with COVID-19. Exhaustive assessment of cytokines in plasma and in platelets revealed the modulation of platelet-associated cytokine levels in both patients with nonsevere and severe COVID-19, pointing to a direct contribution of platelets to the plasmatic cytokine load. Moreover, we demonstrate that platelets release their alpha- and dense-granule contents in both nonsevere and severe forms of COVID-19. In comparison to concentrations measured in healthy volunteers, phosphatidylserine-exposing platelet extracellular vesicles were increased in nonsevere, but not in severe cases of COVID-19. Levels of D-dimers, a marker of thrombosis, failed to correlate with any measured indicators of platelet activation. Functionally, platelets were hyperactivated in COVID-19 subjects presenting nonsevere and severe symptoms, with aggregation occurring at suboptimal thrombin concentrations. Furthermore, platelets adhered more efficiently onto collagen-coated surfaces under flow conditions. Conclusions: Taken together, the data suggest that platelets are at the frontline of COVID-19 pathogenesis, as they release various sets of molecules through the different stages of the disease. Platelets may thus have the potential to contribute to the overwhelming thrombo-inflammation in COVID-19, and the inhibition of pathways related to platelet activation may improve the outcomes during COVID-19.

Differential measurements of charged particle azimuthal anisotropy are presented for lead-lead collisions at s NN = 2.76 TeV with the ATLAS detector at the LHC, based on an integrated luminosity of approximately 8 b -1 . This anisotropy is characterized via a Fourier expansion of the distribution of charged particles in azimuthal angle relative to the reaction plane, with the coefficients v n denoting the magnitude of the anisotropy. Significant v 2 -v 6 values are obtained as a function of transverse momentum (0.5 < p T < 20 GeV), pseudorapidity (|| < 2.5), and centrality using an event plane method. The v n values for n 3 are found to vary weakly with both and centrality, and their p T dependencies are found to follow an approximate scaling relation,

Combined measurements of Higgs boson production and decay using up to 80