67 research outputs found
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- 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
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
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
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 , 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 and
with the Ekman number . 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
with the Rayleigh number based on the heat flux
. 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
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 of her optimal net profit for every
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
Contribution au développement des aimants pour champs magnétiques intenses dans le domaine des matériaux, de la thermique et de la gestion de l’énergie.
Habilitation à diriger les recherche soutenu le 12 juillet 202
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