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- Abolhassani, H, et al.
(författare)
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Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021
- 2022
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Ingår i: Frontiers in immunology. - : Frontiers Media SA. - 1664-3224. ; 13, s. 1032358-
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Tidskriftsartikel (refereegranskat)abstract
- The J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.
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| 4. |
- Mayer, M., et al.
(författare)
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Ion beam analysis of fusion plasma-facing materials and components : Facilities and research challenges
- 2020
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Ingår i: Nuclear Fusion. - : Institute of Physics Publishing. - 0029-5515 .- 1741-4326. ; 60:2
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Tidskriftsartikel (refereegranskat)abstract
- Following the IAEA Technical Meeting on 'Advanced Methodologies for the Analysis of Materials in Energy Applications Using Ion Beam Accelerators', this paper reviews the current status of ion beam analysis (IBA) techniques and some aspects of ion-induced radiation damage in materials for the field of materials relevant to fusion. Available facilities, apparatus development, future research options and challenges are presented and discussed. The analysis of beryllium and radioactivity-containing samples from future experiments in JET or ITER represents not only an analytical but also a technical challenge. A comprehensive list of the facilities, their current status, and analytical capabilities comes alongside detailed descriptions of the labs. A discussion of future issues of sample handling and the current status of facilities at JET complete the technical section. To prepare the international IBA community for these challenges, the IAEA technical meeting concludes the necessity for determining new nuclear reaction cross-sections and improving the inter-laboratory comparability by defining international standards and testing these via a round-robin test.
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| 5. |
- Pegourie, B., et al.
(författare)
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Deuterium inventory in Tore Supra : Coupled carbon-deuterium balance
- 2013
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Ingår i: Journal of Nuclear Materials. - : Elsevier BV. - 0022-3115 .- 1873-4820. ; 438:Suppl., s. S120-S125
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Tidskriftsartikel (refereegranskat)abstract
- This paper presents an analysis of the carbon-deuterium circulation and the resulting balance in Tore Supra over the period 2002-2007. Carbon balance combines the estimation of carbon gross erosion from spectroscopy, net erosion and deposition using confocal microscopy, lock-in thermography and SEM, and a measure of the amount of deposits collected in the vacuum chamber. Fuel retention is determined from post-mortem (PM) analyses and gas balance (GB) measurements. Special attention was paid to the deuterium outgassed during the nights and weekends of the experimental campaign (vessel under vacuum, Plasma Facing Components at 120 degrees C) and during vents (vessel at atmospheric pressure, PFCs at room temperature). It is shown that this outgassing is the main process reconciling the PM and GB estimations of fuel retention, closing the coupled carbon-deuterium balance. In particular, it explains why the deuterium concentration in deposits decreases with increasing depth.
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| 7. |
- Pelicon, P., et al.
(författare)
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Fuel retention study in fusion reactor walls by micro-NRA deuterium mapping
- 2011
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Ingår i: Nuclear Instruments and Methods in Physics Research Section B. - : Elsevier BV. - 0168-583X .- 1872-9584. ; 269:20, s. 2317-2321
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Tidskriftsartikel (refereegranskat)abstract
- Nuclear Reaction Analysis (NRA) with a (3)He ion beam is a powerful analytical technique for analysis of light elements in thin films. The main motivation for 3He focused beam applications is lateral mapping of deuterium using the nuclear reaction D((3)He,p)(4)He in surfaces exposed to a tokamak plasma, where a lateral resolution in the pm-range provides unique information for fuel retention studies. At the microprobe at the Jozef Stefan Institute typical helium ion currents of 300 pA and beam dimensions of 4 x 4 mu m(2) can be obtained. This work is focused on micro-NRA studies of plasma-facing materials using a set-up consisting of a silicon partially depleted charge particle detector for NRA spectroscopy applied in parallel with a permanently installed X-ray detector, an RBS detector and a beam chopper for ion dose monitoring. A method for absolute deuterium quantification is described. In addition, plasma-deposited amorphous deuterated carbon thin films (a-C:D) with known D content were used as a reference. The method was used to study deuterium fuel retention in carbon fibre composite materials exposed to a deuterium plasma in the Tore Supra and TEXTOR tokamaks. The high lateral resolution of micro-NRA allowed us to make a detailed study of the influence of topography on the fuel retention process. We demonstrated that the surface topography plays a dominant role in the retention of deuterium. The deep surfaces inside the castellation gaps showed approximately two orders of magnitude lower deuterium concentrations than in areas close to the exposed surface.
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