Large critical current density improvement in Bi-2212 wires through groove-rolling process

Recently the interest about Bi-2212 round wire superconductor for high magnetic field use has been enhancing despite the fact that an increase of the critical current is still needed to boost its successful use in such applications. Recent studies have demonstrated that the main obstacle to current flow, especially in long wires, is the residual porosity inside these Powder-In-Tube processed conductors which develops in bubbles-agglomeration when the Bi-2212 melts. Through this work we tried to overcome this issue acting on the wire densification by changing the deformation process. Here we show the effects of groove-rolling versus drawing process on the critical current density JC and on the microstructure. In particular, groove-rolled multifilamentary wires show a JC increased by a factor of about 3 with respect to drawn wires prepared with the same Bi-2212 powder and architecture. We think that this approach in the deformation process is able to produce the required improvements both because the superconducting properties are enhanced and because it makes the fabrication process faster and cheaper

Inverse and Direct Energy Cascades in Three Dimensional Magnetohydrodynamic Turbulence at low Magnetic Reynolds Number

This experimental study analyzes the relationship between the dimensionality of turbulence and the upscale or downscale nature of its energy transfers. We do so by forcing low-$Rm$ magnetohydrodynamic (MHD) turbulence in a confined channel, while precisely controlling its dimensionality by means of an externally applied magnetic field. We first identify a specific lengthscale $\hat{l}_\perp^c$ that separates smaller three-dimensional structures from larger quasi-two-dimensional ones. We then show that an inverse energy cascade of horizontal kinetic energy along horizontal scales is always observable at large scales, but that it extends well into the region of 3D structures. At the same time, a direct energy cascade confined to the smallest and strongly 3D scales is observed. These dynamics therefore appear not to be simply determined by the dimensionality of individual scales, nor by the forcing scale, unlike in other studies. In fact, our findings suggest that the relationship between kinematics and dynamics is not universal and may strongly depend on the forcing and dissipating mechanisms at play

Experimental study of convection in a rotating tangent cylinder

This paper experimentally investigates the convection in a fast rotating Tangent Cylinder (TC), for Ekman numbers down to $E=3.36\times10^{-6}$, in a configuration relevant to the liquid core of the Earth. In the apparatus, the TC results from the Proudman-Taylor constraint incurred by rotating a hemispherical fluid vessel heated in its centre by a protruding heating element of cylindrical shape. The resulting convection that develops above the heater, i.e within the TC, is shown to set in for critical Rayleigh numbers and wavenumbers respectively scaling as $Ra_c\sim E^{4/3}$ and $a_c\sim E^{1/3}$ with the Ekman number $E$. Though exhibiting the same exponents as for plane rotating convection, these laws are indicative of much larger convective plumes at onset. The structure and dynamics of these plumes are in fact closer to those found in solid rotating cylinders heated from below, suggesting that the confinement within the TC induced by the Taylor-Proudman constraint influences convection in a similar way as solid walls would do. There is further similarity in that the critical modes in the TC all exhibit a slow retrograde precession at onset. In supercritical regimes, the precession evolves into a thermal wind with a complex structure featuring retrograde rotation at high latitude and either prograde or retrograde rotation at low latitudes (close to the heater), depending on the criticality and the Ekman number. Nevertheless the intensity of the thermal wind measured by the Rossby number scales as $Ro\sim 0.85(Ra_q^*)^{0.41}$ with the Rayleigh number based on the heat flux $Ra_q^*$. This scaling suggests that the convection in the TC is driven by quasi-geostrophic dynamics, a finding supported by the scaling for the rotation-normalised Nusselt number $Nu^{*} \sim (Ra_{q}^{*})^{5/9}$

Data-driven Modeling of Building Consumption Profile for Optimal Flexibility: Application to Energy Intensive Industry

International audienceDespite the vast research on the flexibility of buildings consumption, current literature is more about predicting the impacts of energy flexibility than fo-cusing on its modeling. In this paper, a methodology is provided to go from data-driven modeling of the load consumption to an optimization problem with a Mixed-Integer Linear Programming (MILP) formulation. Illustrated on an Energy Intensive Industries (EII) with an economic point of view, the methodology is suitable for any consumption site, allowing optimal energy planning studies at the district scale. Thus, it facilitates the definition of flexibility strategies to exploit the complementary of uses of the districts

Mécanique et thermique des aimants pour champs magnétiques intenses

Les aimants pour champs magnétiques intenses (c'est à dire au delà des champs fournis par les aimants supraconducteurs) sont réalisés à partir de bobinages d'alliage de cuivre soumis à des contraintes mécaniques et thermiques extrêmes. Ces structures mécaniques travaillent à des contraintes proches de leur limite élastique et les flux de chaleur surfacique à évacuer atteignent 500 W par cm2. Nous présentons les développements en cours pour la conception et la réalisation de ces échangeurs thermiques aux caractéristiques très particulières

BCKDK deficiency: a treatable neurodevelopmental disease amenable to newborn screening

There are few causes of treatable neurodevelopmental diseases described to date. Branched-chain ketoacid dehydrogenase kinase (BCKDK) deficiency causes branched-chain amino acid (BCAA) depletion and is linked to a neurodevelopmental disorder characterized by autism, intellectual disability and microcephaly. We report the largest cohort of patients studied, broadening the phenotypic and genotypic spectrum. Moreover, this is the first study to present newborn screening findings and mid-term clinical outcome. In this cross-sectional study, patients with a diagnosis of BCKDK deficiency were recruited via investigators’ practices through a MetabERN initiative. Clinical, biochemical and genetic data were collected. Dried blood spot (DBS) newborn screening (NBS) amino acid profiles were retrieved from collaborating centres and compared to a healthy newborn reference population. Twenty-one patients with BCKDK mutations were included from 13 families. Patients were diagnosed between 8 months and 16 years (mean: 5.8 years, 43% female). At diagnosis, BCAA levels (leucine, valine and isoleucine) were below reference values in plasma and in CSF. All patients had global neurodevelopmental delay; 18/21 had gross motor function (GMF) impairment with GMF III or worse in 5/18, 16/16 intellectual disability, 17/17 language impairment, 12/17 autism spectrum disorder, 9/21 epilepsy, 12/15 clumsiness, 3/21 had sensorineural hearing loss and 4/20 feeding difficulties. No microcephaly was observed at birth, but 17/20 developed microcephaly during follow-up. Regression was reported in six patients. Movement disorder was observed in 3/21 patients: hyperkinetic movements (1), truncal ataxia (1) and dystonia (2). After treatment with a high-protein diet (≥ 2 g/kg/day) and BCAA supplementation (100–250 mg/kg/day), plasma BCAA increased significantly (P < 0.001), motor functions and head circumference stabilized/ improved in 13/13 and in 11/15 patients, respectively. Among cases with follow-up data, none of the three patients starting treatment before 2 years of age developed autism at follow-up. The patient with the earliest age of treatment initiation (8 months) showed normal development at 3 years of age. NBS in DBS identified BCAA levels significantly lower than those of the normal population. This work highlights the potential benefits of dietetic treatment, in particular early introduction of BCAA. Therefore, it is of utmost importance to increase awareness about this treatable disease and consider it as a candidate for early detection by NBS programmes.A.G.C. is supported by FIS P118/00111, FI21/0073 ‘Instituto de Salud Carlos III (ISCIII)’ and ‘Fondo Europeo de desarrollo regional (FEDER)’

Incentivizing the Dynamic Workforce: Learning Contracts in the Gig-Economy

In principal-agent models, a principal offers a contract to an agent to perform a certain task. The agent exerts a level of effort that maximizes her utility. The principal is oblivious to the agent's chosen level of effort, and conditions her wage only on possible outcomes. In this work, we consider a model in which the principal is unaware of the agent's utility and action space. She sequentially offers contracts to identical agents, and observes the resulting outcomes. We present an algorithm for learning the optimal contract under mild assumptions. We bound the number of samples needed for the principal obtain a contract that is within $\epsilon$ of her optimal net profit for every $\epsilon>0$

Comprehensive study of 28 individuals with SIN3A-related disorder underscoring the associated mild cognitive and distinctive facial phenotype

Witteveen-Kolk syndrome (OMIM 613406) is a recently defined neurodevelopmental syndrome caused by heterozygous loss-of-function variants in SIN3A. We define the clinical and neurodevelopmental phenotypes related to SIN3A-haploinsufficiency in 28 unreported patients. Patients with SIN3A variants adversely affecting protein function have mild intellectual disability, growth and feeding difficulties. Involvement of a multidisciplinary team including a geneticist, paediatrician and neurologist should be considered in managing these patients. Patients described here were identified through a combination of clinical evaluation and gene matching strategies (GeneMatcher and Decipher). All patients consented to participate in this study. Mean age of this cohort was 8.2 years (17 males, 11 females). Out of 16 patients ≥ 8 years old assessed, eight (50%) had mild intellectual disability (ID), four had moderate ID (22%), and one had severe ID (6%). Four (25%) did not have any cognitive impairment. Other neurological symptoms such as seizures (4/28) and hypotonia (12/28) were common. Behaviour problems were reported in a minority. In patients ≥2 years, three were diagnosed with Autism Spectrum Disorder (ASD) and four with Attention Deficit Hyperactivity Disorder (ADHD). We report 27 novel variants and one previously reported variant. 24 were truncating variants; three were missense variants and one large in-frame gain including exons 10–12

Autoantibodies against type I IFNs in patients with life-threatening COVID-19

Interindividual clinical variability in the course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is vast. We report that at least 101 of 987 patients with life-threatening coronavirus disease 2019 (COVID-19) pneumonia had neutralizing immunoglobulin G (IgG) autoantibodies (auto-Abs) against interferon-w (IFN-w) (13 patients), against the 13 types of IFN-a (36), or against both (52) at the onset of critical disease; a few also had auto-Abs against the other three type I IFNs. The auto-Abs neutralize the ability of the corresponding type I IFNs to block SARS-CoV-2 infection in vitro. These auto-Abs were not found in 663 individuals with asymptomatic or mild SARS-CoV-2 infection and were present in only 4 of 1227 healthy individuals. Patients with auto-Abs were aged 25 to 87 years and 95 of the 101 were men. A B cell autoimmune phenocopy of inborn errors of type I IFN immunity accounts for life-threatening COVID-19 pneumonia in at least 2.6% of women and 12.5% of men

Rare predicted loss-of-function variants of type I IFN immunity genes are associated with life-threatening COVID-19

Background: We previously reported that impaired type I IFN activity, due to inborn errors of TLR3- and TLR7-dependent type I interferon (IFN) immunity or to autoantibodies against type I IFN, account for 15–20% of cases of life-threatening COVID-19 in unvaccinated patients. Therefore, the determinants of life-threatening COVID-19 remain to be identified in ~ 80% of cases. Methods: We report here a genome-wide rare variant burden association analysis in 3269 unvaccinated patients with life-threatening COVID-19, and 1373 unvaccinated SARS-CoV-2-infected individuals without pneumonia. Among the 928 patients tested for autoantibodies against type I IFN, a quarter (234) were positive and were excluded. Results: No gene reached genome-wide significance. Under a recessive model, the most significant gene with at-risk variants was TLR7, with an OR of 27.68 (95%CI 1.5–528.7, P = 1.1 × 10−4) for biochemically loss-of-function (bLOF) variants. We replicated the enrichment in rare predicted LOF (pLOF) variants at 13 influenza susceptibility loci involved in TLR3-dependent type I IFN immunity (OR = 3.70[95%CI 1.3–8.2], P = 2.1 × 10−4). This enrichment was further strengthened by (1) adding the recently reported TYK2 and TLR7 COVID-19 loci, particularly under a recessive model (OR = 19.65[95%CI 2.1–2635.4], P = 3.4 × 10−3), and (2) considering as pLOF branchpoint variants with potentially strong impacts on splicing among the 15 loci (OR = 4.40[9%CI 2.3–8.4], P = 7.7 × 10−8). Finally, the patients with pLOF/bLOF variants at these 15 loci were significantly younger (mean age [SD] = 43.3 [20.3] years) than the other patients (56.0 [17.3] years; P = 1.68 × 10−5). Conclusions: Rare variants of TLR3- and TLR7-dependent type I IFN immunity genes can underlie life-threatening COVID-19, particularly with recessive inheritance, in patients under 60&nbsp;years old