| 1. |
- Baumgartner, T., et al.
(författare)
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A survey of the European Reference Network EpiCARE on clinical practice for selected rare epilepsies
- 2021
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Ingår i: Epilepsia Open. - : Wiley. - 2470-9239. ; 6:1, s. 160-170
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Clinical care of rare and complex epilepsies is challenging, because evidence-based treatment guidelines are scarce, the experience of many physicians is limited, and interdisciplinary treatment of comorbidities is required. The pathomechanisms of rare epilepsies are, however, increasingly understood, which potentially fosters novel targeted therapies. The objectives of our survey were to obtain an overview of the clinical practice in European tertiary epilepsy centers treating patients with 5 arbitrarily selected rare epilepsies and to get an estimate of potentially available patients for future studies. Methods: Members of the European Reference Network for rare and complex epilepsies (EpiCARE) were invited to participate in a web-based survey on clinical practice of patients with Dravet syndrome, tuberous sclerosis complex (TSC), autoimmune encephalitis, and progressive myoclonic epilepsies including Unverricht Lundborg and Unverricht-like diseases. A consensus-based questionnaire was generated for each disease. Results: Twenty-six of 30 invited epilepsy centers participated. Cohorts were present in most responding centers for TSC (87%), Dravet syndrome (85%), and autoimmune encephalitis (71%). Patients with TSC and Dravet syndrome represented the largest cohorts in these centers. The antiseizure drug treatments were rather consistent across the centers especially with regard to Dravet syndrome, infantile spasms in TSC, and Unverricht Lundborg / Unverricht-like disease. Available, widely used targeted therapies included everolimus in TSC and immunosuppressive therapies in autoimmune encephalitis. Screening for comorbidities was routinely done, but specific treatment protocols were lacking in most centers. Significance: The survey summarizes the current clinical practice for selected rare epilepsies in tertiary European epilepsy centers and demonstrates consistency as well as heterogeneity in the treatment, underscoring the need for controlled trials and recommendations. The survey also provides estimates for potential participants of clinical trials recruited via EpiCARE, emphasizing the great potential of Reference Networks for future studies to evaluate new targeted therapies and to identify novel biomarkers. © 2020 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy
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| 2. |
- Tobin, J. J., et al.
(författare)
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Deuterium-enriched water ties planet-forming disks to comets and protostars
- 2023
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Ingår i: Nature. - : Springer Science and Business Media LLC. - 0028-0836 .- 1476-4687. ; 615:7951, s. 227-230
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Tidskriftsartikel (refereegranskat)abstract
- Water is a fundamental molecule in the star and planet formation process, essential for catalysing the growth of solid material and the formation of planetesimals within disks1,2. However, the water snowline and the HDO:H2O ratio within proto-planetary disks have not been well characterized because water only sublimates at roughly 160 K (ref. 3), meaning that most water is frozen out onto dust grains and that the water snowline radii are less than 10 AU (astronomical units)4,5. The sun-like protostar V883 Ori (M* = 1.3 M⊙)6 is undergoing an accretion burst7, increasing its luminosity to roughly 200 L⊙ (ref. 8), and previous observations suggested that its water snowline is 40-120 AU in radius6,9,10. Here we report the direct detection of gas phase water (HDO and [Formula: see text]) from the disk of V883 Ori. We measure a midplane water snowline radius of approximately 80 AU, comparable to the scale of the Kuiper Belt, and detect water out to a radius of roughly 160 AU. We then measure the HDO:H2O ratio of the disk to be (2.26 ± 0.63) × 10-3. This ratio is comparable to those of protostellar envelopes and comets, and exceeds that of Earth's oceans by 3.1σ. We conclude that disks directly inherit water from the star-forming cloud and this water becomes incorporated into large icy bodies, such as comets, without substantial chemical alteration.
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| 3. |
- Vidal, Antoni, et al.
(författare)
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Modelling and simulations of reactor neutron noise induced by mechanical vibrations
- 2022
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Ingår i: Annals of Nuclear Energy. - : Elsevier BV. - 0306-4549 .- 1873-2100. ; 177
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Tidskriftsartikel (refereegranskat)abstract
- Mechanical vibrations of core internals are among the main perturbations that induce oscillations in the neutron flux field, also known as neutron noise. In this work, different simulation models for the study of the influence of the mechanical vibrations of fuel assemblies on the neutron flux in the reactor core have been discussed. These methodologies employ the diffusion approximation, with or without a previous homogenization model, to simulate the neutron noise in the time or the frequency domain. The diffusion-based approach is expected to be less accurate in the vicinity of the vibrating fuel assemblies, but correct when considering distances larger than a few diffusion lengths away from the perturbation. All methodologies provide consistent results and can reproduce typical features of the neutron noise induced by mechanical vibrations of core components. First, FEMFFUSION can perform simulations in both the time and frequency domains. Second, CORE SIM + can be used to study various neutron noise scenarios in realistic three-dimensional reactor configurations. The third methodology is centred on using commercial codes as CASMO-5, SIMULATE-3 and SIMULATE-3K. This methodology allows time domain simulations of the neutron noise induced by different neutron noise sources in a nuclear reactor. Finally, a model for time-dependent geometry is implemented for the code system ATHLET/QUABOX-CUBBOX employing a cross-section-based approach for encoding water gap width variations at the reflector.
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