Design and performance of the multiplexing spectrometer CAMEA

The cold neutron multiplexing secondary spectrometer CAMEA (Continuous Angle Multiple Energy Analysis) was commissioned at the Swiss spallation neutron source SINQ at the Paul Scherrer Institut at the end of 2018. The spectrometer is optimised for an efficient data collection in the horizontal scattering plane, allowing for detailed and rapid mapping of excitations under extreme conditions. The novel design consists of consecutive, upward scattering analyzer arcs underneath an array of position sensitive detectors mounted inside a low permeability stainless-steel vacuum vessel. The construction of the world's first continuous angle multiple energy analysis instrument required novel solutions to many technical challenges, including analyzer mounting, vacuum connectors, and instrument movement. These were solved by extensive prototype experiments and in-house developments. Here we present a technical overview of the spectrometer describing in detail the engineering solutions and present our first experimental data taken during the commissioning. Our results demonstrate the tremendous gains in data collection rate for this novel type of spectrometer design

Accumulation of mutations in antibody and CD8 T cell epitopes in a B cell depleted lymphoma patient with chronic SARS-CoV-2 infection

Antibodies against the spike protein of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) can drive adaptive evolution in immunocompromised patients with chronic infection. Here we longitudinally analyze SARS-CoV-2 sequences in a B cell-depleted, lymphoma patient with chronic, ultimately fatal infection, and identify three mutations in the spike protein that dampen convalescent plasma-mediated neutralization of SARS-CoV-2. Additionally, four mutations emerge in non-spike regions encoding three CD8 T cell epitopes, including one nucleoprotein epitope affected by two mutations. Recognition of each mutant peptide by CD8 T cells from convalescent donors is reduced compared to its ancestral peptide, with additive effects resulting from double mutations. Querying public SARS-CoV-2 sequences shows that these mutations have independently emerged as homoplasies in circulating lineages. Our data thus suggest that potential impacts of CD8 T cells on SARS-CoV-2 mutations, at least in those with humoral immunodeficiency, warrant further investigation to inform on vaccine design

The German National Registry of Primary Immunodeficiencies (2012-2017)

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment

EU budget support policy of the euro area. The attempt of the political concept’s legal delimitation

Perturbacje w strefie euro uzasadniają pytanie nie tylko o stan finansów publicznych państw tej strefy, ale i zmuszają do zastanowienia się nad kształtem uprawianej ze strony Unii Europejskiej polityki wsparcia budżetów krajów należących do strefy euro. Trudno bowiem nie zauważyć, że wspomniana polityka stanowi od maja 2010 roku zarówno obiekt krytyki, w tym rzecz jasna naukowej[1], ale i źródło (aczkolwiek nie jedyne) obecnych kłopotów finansowych Unii Europejskiej. Wprawdzie kłopoty te nie zagrażają (jeszcze) praktykowaniu europejskiego wspólnego rynku, to tym niemniej jednak nie sposób przeoczyć, że osłabiają w poważnym stopniu wspólnotowe spoiwo, które stanowi europejska unia gospodarcza i walutowa. Chcąc zatem ukazać implikacje prawne i polityczne tego procesu, konieczne staje się najpierw przypomnienie stosownych uregulowań prawa wspólnotowego. Wszystko po to, aby odwołując się do bon motJohna Kennetha Galbraitha, iż jedną z korzyści płynących z recesji jest to, że ujawnia co przegapił księgowy, móc spróbować pokazać nowe kontury polityczno-prawne wskazanego problemu. Kontury, ale i powiedzmy wyraźnie – wyzwania polityczno-prawne, jakie wyłaniają się z analizy obecnej polityki Unii Europejskiej wsparcia budżetów państw strefy euro.Perturbations in the eurozone justify the question concerning not only the condition of public finances in the countries belonging to this zone, but also force us to think about the form of budget support policy of the eurozone countries implemented by the European Union. It is not difficult to realize that the aforementioned policy is, since May 2010, both an object of criticism, also in a scientific aspect, and the (not only) source of the current financial difficulties of the European Union. Although these problems do not (yet) threaten the common European market, it is impossible to ignore that they weaken to a great extent the union binder, which consists both of the economic and monetary European union. With the purpose of showing the legal and political implications of this process, it becomes necessary to remind first of all of the adequate regulations of the European Union law. All this, referring to the bon mot of John Kenneth Galbraith that one of the recession’s benefits is that it reveals what the accountant has overlooked, in order to be able to present the new political and legal shapes of the indicated problem. The shapes and also, specifically speaking, the political and legal challenges, which emerge from the analysis of the present budget support policy of the eurozone countries implemented by the European Union

Solar Photocatalytic Detoxification of Rocket Test Facility Waste Water with a Non Concentrating Tubular Receiver (NCTR) Pilot Plant

A new type of non concentrating tubular receiver (NCTR) for solar photocatalytic applications is presented and the decontamination efficiency is compared with the well known CPC type technology. The NCTR receiver consists mainly of Borosilicate type glass tubes in a compact arrangement without any reflector material. Only cheap, rugged and simple parts are used for the construction of the NCTR receiver and the support structure to enhance the cost-effectiveness and weather resistance of the receiver system. Comparative detoxification tests are carried out with pre-pilot plant reactors of the CPC and the NCTR type using heterogeneous and homogenous photocatalysis for the detoxification of artificial waste water containing cyanide or hydrazine. Based on the results of the tests with the pre-pilot plant scale reactor a NCTR pilot plant has been installed for the treatment of waste water containing hydrazine from rocket test facilities at DLR site Lampoldshausen, Germany. The NCTR pilot plant consists of a 32 m² NCTR receiver and a pumping unit for batch processing of 1 m³ of the rocket test facility waste water. First results of the pilot plant treatment tests will be presented. The new NCTR type solar collector seems to be a promising design for solar water technology on the way to achieve an economically applicable solar photocatalytic waste water treatment system