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

`Football Remembers':The Collective Memory of Football in the Spectacle of British Military Commemoration

This article examines two major rituals of contemporary national life in the UK: association football and military commemoration. It explores the ways in which remembering is enacted and performed within UK football and how these processes are related to issues of power, agency and identity in Britain today. Employing the concepts of collective memory and spectacle, this article argues that ‘memory entrepreneurs’ have sought to embed football as ‘site of memory’ in the performance of military commemoration. It concludes that this has contributed to the transformation of military commemoration, from a ritual that is observed to a spectacle that is consumed. This paper thus contributes to emergent debates on the militarization of civilian space, the shifting nature of civil–military relations in the twenty-first century, and the role of military remembrance in the reproduction of Britishness

A Possible Alignment Between the Orbits of Planetary Systems and their Visual Binary Companions

Astronomers do not have a complete picture of the effects of wide-binary companions (semimajor axes greater than 100 au) on the formation and evolution of exoplanets. We investigate these effects using new data from Gaia Early Data Release 3 and the Transiting Exoplanet Survey Satellite mission to characterize wide-binary systems with transiting exoplanets. We identify a sample of 67 systems of transiting exoplanet candidates (with well-determined, edge-on orbital inclinations) that reside in wide visual binary systems. We derive limits on orbital parameters for the wide-binary systems and measure the minimum difference in orbital inclination between the binary and planet orbits. We determine that there is statistically significant difference in the inclination distribution of wide-binary systems with transiting planets compared to a control sample, with the probability that the two distributions are the same being 0.0037. This implies that there is an overabundance of planets in binary systems whose orbits are aligned with those of the binary. The overabundance of aligned systems appears to primarily have semimajor axes less than 700 au. We investigate some effects that could cause the alignment and conclude that a torque caused by a misaligned binary companion on the protoplanetary disk is the most promising explanation

A Life Cycle for the Development of Autonomic Systems: The e-Mobility Showcase

Abstract—Component ensembles are a promising way of building self-aware autonomic adaptive systems. This approach has been promoted by the EU project ASCENS, which develops the core idea of ensembles by providing rigorous semantics as well as models and methods for the whole development life cycle of an ensemble-based system. These methods specifically address adaptation, self-awareness, self-optimization, and continuous system evolution. In this paper, we demonstrate the key concepts and benefits of the ASCENS approach in the context of intelligent navigation of electric vehicles (e-Mobility), which itself is one of the three key case studies of the project. I

Results of the Wilcoxon signed rank test for the baseline adjusted SEF difference.

<p>Results of the Wilcoxon signed rank test for the baseline adjusted SEF difference.</p

Overview of the participant flow.

<p>* The study was originally designed for 12 patients with balanced randomization of 3 patients to each of the 4 study arms. One patient in treatment group BoNT/A (right gland) was not treated according to the study protocol and therefore excluded from the study. An additional patient was recruited and assigned to the treatment group of the excluded patient to retain the balanced allocation. The assignment was only known to the Coordination Center for Clinical Trials and double-blindness was maintained. The original patient refused radiochemotherapy and did not undergo post-treatment scintigraphy and therefore was excluded from the analysis. † One patient originally allocated to group BoNT/A (right gland) was actually treated with BoNT/A in the left gland and placebo in the right gland due to mix-up of the injections designated for the two sides. According to actual treatment, this patient was assigned to treatment group BoNT/A (left gland) and a new patient was allocated to group BoNT/A (right gland) to retain the balance. The actual group assignment was only known to the Coordination Center for Clinical Trials and double-blindness was maintained. Because of the double-blinded nature of the trial, it can be assumed that the treatment error occurred accidentally and therefore did not lead to any selection bias.</p

Results of the ANCOVA analyses for the SEF difference of the submandibular glands after radiotherapy.

<p>Results of the ANCOVA analyses for the SEF difference of the submandibular glands after radiotherapy.</p

Dosimetric parameters of the left and right submandibular glands for each patient in Gray.

<p>D_min = minimum dose, D_max = maximum dose, D_mean = mean dose, SD = standard deviation. For an explanation of treatment group numbers see legend of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0151316#pone.0151316.t001" target="_blank">Table 1</a>.</p

Results of the Wilcoxon signed rank test for the baseline adjusted uptake difference.

<p>n: number of patients in each dose group.</p

Salivary gland scintigraphy before (left) and after (right) radiotherapy of patient number 11.

<p>A consistently higher uptake level of the right submandibular gland area treated with BoNT compared to the control gland on the opposite side was demonstrated. Time activity curves on quantitative scintigraphy of the submandibular gland before (left) and after (right) radiotherapy are shown. Red: right gland, blue: left gland, yellow: background activity.</p