Hyper-IgG4 disease: report and characterisation of a new disease

BACKGROUND: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. METHODS: We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS: Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION: We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good

COVID-19 symptoms at hospital admission vary with age and sex: results from the ISARIC prospective multinational observational study

Background: The ISARIC prospective multinational observational study is the largest cohort of hospitalized patients with COVID-19. We present relationships of age, sex, and nationality to presenting symptoms. Methods: International, prospective observational study of 60 109 hospitalized symptomatic patients with laboratory-confirmed COVID-19 recruited from 43 countries between 30 January and 3 August 2020. Logistic regression was performed to evaluate relationships of age and sex to published COVID-19 case definitions and the most commonly reported symptoms. Results: ‘Typical’ symptoms of fever (69%), cough (68%) and shortness of breath (66%) were the most commonly reported. 92% of patients experienced at least one of these. Prevalence of typical symptoms was greatest in 30- to 60-year-olds (respectively 80, 79, 69%; at least one 95%). They were reported less frequently in children (≤ 18 years: 69, 48, 23; 85%), older adults (≥ 70 years: 61, 62, 65; 90%), and women (66, 66, 64; 90%; vs. men 71, 70, 67; 93%, each P < 0.001). The most common atypical presentations under 60 years of age were nausea and vomiting and abdominal pain, and over 60 years was confusion. Regression models showed significant differences in symptoms with sex, age and country. Interpretation: This international collaboration has allowed us to report reliable symptom data from the largest cohort of patients admitted to hospital with COVID-19. Adults over 60 and children admitted to hospital with COVID-19 are less likely to present with typical symptoms. Nausea and vomiting are common atypical presentations under 30 years. Confusion is a frequent atypical presentation of COVID-19 in adults over 60 years. Women are less likely to experience typical symptoms than men

Design and optimization of low frequency electric field sensor using Pockels effect

International audienc

First Industrial Tests of a Matrix Monitor Correction for the Differential Die-away Technique of Historic Waste Drums

International audienceAbstract The fissile mass in radioactive waste drums filled with compacted metallic residues (spent fuel hulls and nozzles) produced at AREVA NC La Hague reprocessing plant is measured by neutron interrogation with the Differential Die-away measurement Technique (DDT). In the next years, old hulls and nozzles mixed with Ion-Exchange Resins will be measured. The ion-exchange resins increase neutron moderation in the matrix, compared to the waste measured in the current process. In this context, the Nuclear Measurement Laboratory (LMN) of CEA Cadarache has studied a matrix effect correction method, based on a drum monitor, namely a $^3$He proportional counter located inside the measurement cavity. After feasibility studies performed with LMN's PROMETHEE 6 laboratory measurement cell and with MCNPX simulations, this paper presents first experimental tests performed on the industrial ACC (hulls and nozzles compaction facility) measurement system. A calculation vs. experiment benchmark has been achieved by performing dedicated calibration measurements with a representative drum and $^{235}$U samples. The comparison between calculation and experiment shows a satisfactory agreement for the drum monitor. The final objective of this work is to confirm the reliability of the modeling approach and the industrial feasibility of the method, which will be implemented on the industrial station for the measurement of historical wastes

Reduction of the uncertainty due to fissile clusters in radioactive waste characterization with the Differential Die-away Technique

International audienceAREVA NC is preparing to process, characterize and compact old used fuel metallic waste stored at La Hague reprocessing plant in view of their future storage (“Haute Activité Oxyde” HAO project). For a large part of these historical wastes, the packaging is planned in CSD-C canisters (“Colis Standard de Déchets Compacté s”) in the ACC hulls and nozzles compaction facility (“Atelier de Compactage des Coques et embouts”).. This paper presents a new method to take into account the possible presence of fissile material clusters, which may have a significant impact in the active neutron interrogation (Differential Die-away Technique) measurement of the CSD-C canisters, in the industrial neutron measurement station “P2-2”. A matrix effect correction has already been investigated to predict the prompt fission neutron calibration coefficient (which provides the fissile mass) from an internal “drum flux monitor” signal provided during the active measurement by a boron-coated proportional counter located in the measurement cavity, and from a “drum transmission signal” recorded in passive mode by the detection blocks, in presence of an AmBe point source in the measurement cell. Up to now, the relationship between the calibration coefficient and these signals was obtained from a factorial design that did not consider the potential for occurrence of fissile material clusters. The interrogative neutron self-shielding in these clusters was treated separately and resulted in a penalty coefficient larger than 20% to prevent an underestimation of the fissile mass within the drum. In this work, we have shown that the incorporation of a new parameter in the factorial design, representing the fissile mass fraction in these clusters, provides an alternative to the penalty coefficient. This new approach finally does not degrade the uncertainty of the original prediction, which was calculated without taking into consideration the possible presence of clusters. Consequently, the accuracy of the fissile mass assessment is improved by this new method, and this last should be extended to similar DDT measurement stations of larger drums, also using an internal monitor for matrix effect correctio

Performance assessment of amplification and discrimination electronic devices for passive neutron measurements

The knowledge of the fissile material mass is a key challenge to enhance radioactive waste management and to ensure a high level of safety in nuclear industry. Data is analyzed according to the principles of the neutron measurement techniques. As proportional counters filled with 3He gas display high neutron detection efficiency and a good gamma-ray discrimination, they are the reference detector for passive neutron coincidence counting. A charge preamplifier or a current amplifier, depending on applications, collects the electric pulse produced by neutron interaction in the 3He gas and a threshold discriminator produces a logic pulse used for neutron counting. This paper describes the performance assessment of different commercially available electronics from Mirion Technologies, Precision Data Technology (PDT), Mesytec, as well as MONACO electronics originally developed by CEA LIST for fission chamber measurements in experimental reactors. Comparative passive neutron measurements are carried out with these electronics at CEA/DEN Nuclear Measurement Laboratory in Cadarache. Overall, PDT and Mesytec electronics show similar detection efficiency as the ACH-NA98 charge amplifier, which is commonly used in our laboratory for such applications. However, MONACO electronics have a lower detection efficiency, similar to Mirion 7820 current amplifier used in specific high-count rate applications. An optimisation of MONACO settings would probably be necessary to adapt to 3He counters instead of fission chambers

Catalytic and thermal characterisations of nanosized PdPt / Al₂O₃ for hydrogen detection

Palladium platinum (PdPt) has been intensively studied these last decades due to high conversion rate in hydrogen oxidation at room temperature with significant exothermic effects. These remarkable properties have been studied by measuring the temperature variations of alumina (Al₂O₃) supported nanosized PdPt nanoparticles exposed to different hydrogen concentrations in dry air. This catalyst is expected to be used as a sensing material for stable and reversible ultrasensitive hydrogen sensors working at room temperature (low power consumption). Structural and gas sensing characterisations and catalytic activity of PdPt / Al₂O₃ systems synthesised by co-impregnation will be presented. Catalytic characterisations show that the system is already active at room temperature and that this activity sharply increases with rise in temperature. Moreover, the increase of the PdPt proportion in the co-impregnation process improves the activity, and very high conversion can be reached even at room temperature. The thermal response (about 3 °C) of only 1 mg of PdPt / Al₂O₃ is reversible, and the time response is about 5 s. The integration of PdPt / Al₂O₃ powder on a flat substrate has been realised by the deposition onto the powder of a thin porous hydrophobic layer of parylene. The possibility of using PdPt in gas sensors will be discussed

Oxygen Reduction Catalysis at a Dicobalt Center: The Relationship of Faradaic Efficiency to Overpotential

The selective four electron, four proton, electrochemical reduction of O₂ to H₂O in the presence of a strong acid (TFA) is catalyzed at a dicobalt center. The faradaic efficiency of the oxygen reduction reaction (ORR) is furnished from a systematic electrochemical study by using rotating ring disk electrode (RRDE) methods over a wide potential range. We derive a thermodynamic cycle that gives access to the standard potential of O₂ reduction to H₂O in organic solvents, taking into account the presence of an exogenous proton donor. The difference in ORR selectivity for H₂O vs H₂O₂ depends on the thermodynamic standard potential as dictated by the p<i>K</i>_a of the proton donor. The model is general and rationalizes the faradaic efficiencies reported for many ORR catalytic systems