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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.
Background: Tuberous sclerosis complex (TSC), a multisystem genetic disorder, affects many organs and systems, characterized by benign growths. This German multicenter study estimated the disease-specific costs and cost-driving factors associated with various organ manifestations in TSC patients. Methods: A validated, three-month, retrospective questionnaire was administered to assess the sociodemographic and clinical characteristics, organ manifestations, direct, indirect, out-of-pocket, and nursing care-level costs, completed by caregivers of patients with TSC throughout Germany. Results: The caregivers of 184 patients (mean age 9.8 ± 5.3 years, range 0.7–21.8 years) submitted questionnaires. The reported TSC disease manifestations included epilepsy (92%), skin disorders (86%), structural brain disorders (83%), heart and circulatory system disorders (67%), kidney and urinary tract disorders (53%), and psychiatric disorders (51%). Genetic variations in TSC2 were reported in 46% of patients, whereas 14% were reported in TSC1. Mean total direct health care costs were EUR 4949 [95% confidence interval (95% CI) EUR 4088–5863, median EUR 2062] per patient over three months. Medication costs represented the largest direct cost category (54% of total direct costs, mean EUR 2658), with mechanistic target of rapamycin (mTOR) inhibitors representing the largest share (47%, EUR 2309). The cost of anti-seizure drugs (ASDs) accounted for a mean of only EUR 260 (5%). Inpatient costs (21%, EUR 1027) and ancillary therapy costs (8%, EUR 407) were also important direct cost components. The mean nursing care-level costs were EUR 1163 (95% CI EUR 1027–1314, median EUR 1635) over three months. Total indirect costs totaled a mean of EUR 2813 (95% CI EUR 2221–3394, median EUR 215) for mothers and EUR 372 (95% CI EUR 193–586, median EUR 0) for fathers. Multiple regression analyses revealed polytherapy with two or more ASDs and the use of mTOR inhibitors as independent cost-driving factors of total direct costs. Disability and psychiatric disease were independent cost-driving factors for total indirect costs as well as for nursing care-level costs. Conclusions: This study revealed substantial direct (including medication), nursing care-level, and indirect costs associated with TSC over three months, highlighting the spectrum of organ manifestations and their treatment needs in the German healthcare setting.
Background: The approval of everolimus (EVE) for the treatment of angiomyolipoma (2013), subependymal giant cell astrocytoma (2013) and drug-refractory epilepsy (2017) in patients with tuberous sclerosis complex (TSC) represents the first disease-modifying treatment option available for this rare and complex genetic disorder. Objective: The objective of this study was to analyse the use, efficacy, tolerability and treatment retention of EVE in patients with TSC in Germany from the patient’s perspective. Methods: A structured cross-age survey was conducted at 26 specialised TSC centres in Germany and by the German TSC patient advocacy group between February and July 2019, enrolling children, adolescents and adult patients with TSC. Results: Of 365 participants, 36.7% (n = 134) reported the current or past intake of EVE, including 31.5% (n = 115) who were taking EVE at study entry. The mean EVE dosage was 6.1 ± 2.9 mg/m2 (median: 5.6 mg/m2, range 2.0–15.1 mg/m2) in children and adolescents and 4 ± 2.1 mg/m2 (median: 3.7 mg/m2, range 0.8–10.1 mg/m2) in adult patients. An early diagnosis of TSC, the presence of angiomyolipoma, drug-refractory epilepsy, neuropsychiatric manifestations, subependymal giant cell astrocytoma, cardiac rhabdomyoma and overall multi-organ involvement were associated with the use of EVE as a disease-modifying treatment. The reported efficacy was 64.0% for angiomyolipoma (75% in adult patients), 66.2% for drug-refractory epilepsy, and 54.4% for subependymal giant cell astrocytoma. The overall retention rate for EVE was 85.8%. The retention rates after 12 months of EVE therapy were higher among adults (93.7%) than among children and adolescents (88.7%; 90.5% vs 77.4% after 24 months; 87.3% vs 77.4% after 36 months). Tolerability was acceptable, with 70.9% of patients overall reporting adverse events, including stomatitis (47.0%), acne-like rash (7.7%), increased susceptibility to common infections and lymphoedema (each 6.0%), which were the most frequently reported symptoms. With a total score of 41.7 compared with 36.8 among patients not taking EVE, patients currently being treated with EVE showed an increased Liverpool Adverse Event Profile. Noticeable deviations in the sub-items ‘tiredness’, ‘skin problems’ and ‘mouth/gum problems’, which are likely related to EVE-typical adverse effects, were more frequently reported among patients taking EVE. Conclusions: From the patients’ perspective, EVE is an effective and relatively well-tolerated disease-modifying treatment option for children, adolescents and adults with TSC, associated with a high long-term retention rate that can be individually considered for each patient. Everolimus therapy should ideally be supervised by a centre experienced in the use of mechanistic target of rapamycin inhibitors, and adverse effects should be monitored on a regular basis.
We present calculations for the impact-parameter dependence of K-shell ionization rates in p¯-Cu and in p¯-Ag collisions at various projectile energies. We show that the effect of the attractive Coulomb potential on the Rutherford trajectory and the antibinding effect caused by the negative charge of the antiproton result in a considerable increase of the ionization probability. Total ionization cross sections for proton and antiproton projectiles are compared with each other and with experimental ionization cross sections for protons.
Atomic excitations are used to obtain information on the course of a nuclear reaction. Employing a semiclassical picture we calculate the emission of δ electrons and positrons in deep inelastic nuclear reactions for the example of U+U collisions incorporating nuclear trajectories resulting from two different nuclear friction models. The emission spectra exhibit characteristic deviations from those expected for elastic Coulomb scattering. The theoretical probabilities are compared with recent experimental data by Backe et al. A simple model is used to estimate the influence of a threebody breakup of the compound system upon atomic excitations.
A measurement of the transverse momentum spectra of jets in Pb-Pb collisions at sNN−−−√=2.76 TeV is reported. Jets are reconstructed from charged particles using the anti-kT jet algorithm with jet resolution parameters R of 0.2 and 0.3 in pseudo-rapidity |η|<0.5. The transverse momentum pT of charged particles is measured down to 0.15 GeV/c which gives access to the low pT fragments of the jet. Jets found in heavy-ion collisions are corrected event-by-event for average background density and on an inclusive basis (via unfolding) for residual background fluctuations and detector effects. A strong suppression of jet production in central events with respect to peripheral events is observed. The suppression is found to be similar to the suppression of charged hadrons, which suggests that substantial energy is radiated at angles larger than the jet resolution parameter R=0.3 considered in the analysis. The fragmentation bias introduced by selecting jets with a high pT leading particle, which rejects jets with a soft fragmentation pattern, has a similar effect on the jet yield for central and peripheral events. The ratio of jet spectra with R=0.2 and R=0.3 is found to be similar in Pb-Pb and simulated PYTHIA pp events, indicating no strong broadening of the radial jet structure in the reconstructed jets with R<0.3.
Weimar ist als Geburtsstätte der Klassik ein zentraler Ort unseres kulturellen Erbes. Goethe, Schiller, Herder und Wieland sind Namen, die der Stadt in der Provinz bis heute weltweite Beachtung sichern. Wie aber kam es dazu, dass Weimar zu dieser Metropole der deutschen Geistesgeschichte wurde? Welchen Anteil hatte im späten 18. Jahrhundert die Politik der Herzogin Anna Amalia und ihres Sohnes Herzog Carl August an der einzigartigen kulturellen Blüte, die sich um 1800 in ihrem Fürstentum entfaltete? Aus Anlass des 200. Todestages Anna Amalias und des 250. Geburtstages Carl Augusts gibt die Ausstellung »Ereignis Weimar – Anna Amalia, Carl August und das Entstehen der Klassik 1757–1807« Antworten auf diese Fragen. Sie wurde von der Klassik Stiftung Weimar in Zusammenarbeit mit dem Sonderforschungsbereich »Ereignis Weimar-Jena. Kultur um 1800« der Friedrich Schiller-Universität Jena konzipiert. Vor dem Hintergrund der Biographien beider Herrscherpersönlichkeiten beleuchtet die Ausstellung die Traditionen der Häuser Sachsen und Braunschweig, veranschaulicht die prekäre politische und wirtschaftliche Lage zur Zeit des Regierungsübergangs von Anna Amalia auf ihren Sohn Carl August und thematisiert die Versuche der Regierung, mit vielfältigen Reformen der drängenden Probleme Herr zu werden. Nach anfangs erfolglosem Engagement auf wirtschafts- und machtpolitischem Gebiet stellte sich Erfolg und Fortune in der Kulturpolitik ein. An die Stelle eines zunächst dilettantisch gepflegten »Genietreibens« trat eine zunehmend professionelle Lenkung von Wissenschaft und Kunst, die zu einer einzigartigen Konzentration geistig-kulturellen Kapitals in Sachsen-Weimar führte. Dieses »Ereignis Weimar« brachte dem kleinen Herzogtum seine Reputation als geistiges Zentrum Deutschlands ein. Am Ende rettete diese einzigartige Position das Fürstentum auch über die Fährnisse der napoleonischen Zeit hinweg. Zitiert nach: Gustav Seibt, Süddeutsche Zeitung vom 02. April 2007
Als Goethe im Oktober 1807 [...] an seinen Verleger Cotta schrieb, sah er sich genötigt, politische Schadensbegrenzung zu betreiben. Herabwürdigende Kolumnen aus der Feder seines Intimfeindes Karl August Böttiger in Cottas Augsburger "Allgemeiner Zeitung" waren in der für Weimar noch kritischen Situation nach dem gerade zu Ende gegangenen Krieg das letzte, dessen man bedurfte. Cotta möge, so bat Goethe dringend, "alles, was unsre politische Existenz betrifft und nicht von mir kommt", von seinen Blättern abweisen. Goethes Intervention zeigt nicht nur, daß die Rolle des Kulturellen damals weit wichtiger gewesen ist, als man mit den üblichen Vorstellungen über fürstliches Mäzenatentum und Forderung von Literatur, Kunst und Wissenschaften an Weimars "Musenhof" zu verbinden gewohnt ist. In einer Situation, in der die Katastrophe von Jena und Auerstedt 1806 die regulären Politikinstanzen nahezu blockiert hatte, erwiesen sich der Ruf Weimars und das internationale Ansehen seiner Dichter und Literaten als unschätzbares politisches Kapital, und die von ihnen in Jahrzehnten aufgebauten personellen Beziehungs- und Kommunikationsnetze bildeten in den chaotischen Tagen nach dem 14. Oktober 1806 den einzigen noch verfügbaren Handlungsraum, um Weichenstellungen einzuleiten, die den drohenden Untergang des kleinen Herzogtums abzuwenden und ihm einen Platz in Napoleons Rheinbund zu verschaffen vermochten. Verständlich also, daß Goethe diesen Nimbus unbeschädigt erhalten wollte. Mit anderen Worten: Die Kultur ersetzte in dieser besonderen historischen Situation gleichsam die Politik, genauer gesagt das, was man damals unter Staatspolitik verstand, das Handeln der Regenten, Regierungen und Diplomaten. Daß Weimar in der Krisensituation von 1806 in der Lage war, auf ein solches Potential zurückzugreifen, war das Ergebnis einer jahrzehntelangen Entwicklung. In den folgenden Ausführungen soll versucht werden, deren wichtigste Etappen und Aspekte nachzuzeichnen.
School psychologists are asked to systematically evaluate the effects of their work to ensure quality standards. Given the different types of methods applied to different users of school psychology measuring the effects of school psychological services is a complex task. Thus, the focus of our scoping review was to systematically investigate the state of past research on the measurement of the effects of school psychological services published between 1998 and 2018 in eight major school psychological journals. Of the 5,048 peer-reviewed articles published within this period, 623 were coded by two independent raters as explicitly refering to school psychology or counseling in the school context in their titles or abstracts. However, only 22 included definitions of effects of school psychological services or described outcomes used to evaluate school psychological services based on full text screening. These findings revealed that measurement of the effects of school psychological services has not been a focus of research despite its' relevance in guidelines of school psychological practice.
Yeast large ribosomal subunit (LSU) precursors are subject to substantial changes in protein composition during their maturation due to coordinated transient interactions with a large number of ribosome biogenesis factors and due to the assembly of ribosomal proteins. These compositional changes go along with stepwise processing of LSU rRNA precursors and with specific rRNA folding events, as revealed by recent cryo-electron microscopy analyses of late nuclear and cytoplasmic LSU precursors. Here we aimed to analyze changes in the spatial rRNA surrounding of selected ribosomal proteins during yeast LSU maturation. For this we combined a recently developed tethered tertiary structure probing approach with both targeted and high throughput readout strategies. Several structural features of late LSU precursors were faithfully detected by this procedure. In addition, the obtained data let us suggest that early rRNA precursor processing events are accompanied by a global transition from a flexible to a spatially restricted rRNA conformation. For intermediate LSU precursors a number of structural hallmarks could be addressed which include the fold of the internal transcribed spacer between 5.8S rRNA and 25S rRNA, the orientation of the central protuberance and the spatial organization of the interface between LSU rRNA domains I and III.