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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.
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.
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.
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.
OBJECTIVE: To compare efficacy, safety, and tolerability of an oral enzyme combination (OEC) containing proteolytic enzymes and bioflavonoid vs diclofenac (DIC), a nonselective nonsteroidal anti-inflammatory drug in the treatment of osteoarthritis of the knee.
MATERIALS AND METHODS: This was an individual patient-level pooled reanalysis of patient-reported data from prospective, randomized, double-blind, parallel-group studies in adult patients with moderate-to-severe osteoarthritis of the knee treated for at least 3 weeks with OEC or DIC. Appropriate trials were identified with a systemic literature and database search. Data were extracted from the original case-report forms and reanalyzed by a blinded evaluation committee. The primary end point was the improvement of the Lequesne algofunctional index (LAFI) score at study end vs baseline. Secondary end points addressed LAFI response rates, treatment-related pain-intensity changes, adverse events, and laboratory parameters.
RESULTS: Six trials were identified that enrolled in total 774 patients, of whom 759 had post-baseline data for safety analysis, 697 (n=348/349 with OEC/DIC) for intent to treat, 524 for per protocol efficacy analysis, and 500 for laboratory evaluation. LAFI scores - the primary efficacy end point - decreased comparably with both treatments and improved with both treatments significantly vs baseline (OEC 12.6±2.4 to 9.1±3.9, DIC 12.7±2.4 to 9.1±4.2, effect size 0.9/0.88; P<0.001 for each). In parallel, movement-related 11-point numeric rating-scale pain intensity improved significantly (P<0.001) and comparably with both treatments from baseline (6.4±1.9/6.6±1.8) to study end (3.8±2.7/3.9±2.5). Overall, 55/81 OEC/DIC patients of the safety-analysis population (14.7%/21.1%, P=0.022) reported 90/133 treatment-emergent adverse events, followed by premature treatment discontinuations in 22/39 patients (5.9%/10.2%, P=0.030). Changes in laboratory parameters were significantly less with OEC vs DIC: on average 18.8% vs 86.3% of patients presented a decrease with respect to hemoglobin, hematocrit, or erythrocyte count (P<0.001), and 28.2% vs 72.6% showed an increase in AST, ALT, or GGT (P<0.001).
CONCLUSION: When compared with DIC, OEC showed comparable efficacy and a superior tolerability/safety profile associated with a significantly lower risk of treatment-emergent adverse events, related study discontinuations, and changes in laboratory parameters.
We can conclude that the Dughwede calendar lasts for two seasonal years, marked by the bull festival as a culminating and turning point. All ritual and agricultural activities are interlinked and need to be seen comprehensively together with the social and cosmological order to understand the underlying cultural pattern. The year is dramatized throughout the seasons to keep the communication between the natural and spiritual forces, both creatively reflected in the individual person. The traditional world was kept in balance as a functional equilibrium over a period of time not known to us, but is now moving towards a process of transformation initiated by structural historical change. The first step towards change is the change of moral values which affects possibly first individuals and then groups. This encourages them to give up the traditional way of interacting with their environment. This process can be described as secularisation and leads to another quality of relationship between man and his natural environment. The same process can also be described as socio-economic change.
The Way of the Beer analyses how Mafa re-enact their history in the ritual transfer of sorghum beer from junior to senior members of their society. Beer is the ‘Eucharist’ of Mafa religion, standing for the linkage between God, the ancestors, the fertility of the living and the agricultural land. The ritual sequences in which beer is exchanged and offered at family and community shrines are an encoding of settlement history. The CD-ROM version of the "Way of the Beer" not only contains everything found in the printed version of this work, but also a digital map (figure 8) whichis too large to be printed. The key to the digital map (figure 9) is electronically linked to the map. Please note that figure 9 is displayed when the option "key", found under each ward name (in the bookmark section of figure 8), is activated. If this is done for the first time, figure 8 needs to be brought up again in order to be tiled next to figure 9. However, the best option for exploring the map is to print out the key. Please refer to "The Way of the Beer"(pages 142-144) for further information on how the large digital map needs to be read. The text, maps and images can be viewed in Word (please install the linguistic fonts before using the Word version) or Acrobat Reader (version 4.0 has beencopied on to the CD-ROM). The CD-ROM also contains version 1.03 of the Northern Mandaras Homepage which must be viewed with Microsoft Explorer. The CD-ROM is organised in five main folders which are labelled "Text", "Figures", "Tables", "Plates" and "Homepage". Each folder contains a Word 97 as well as an Adobe Acrobat version of "The Way of the Beer". It is only the large digital map which does not exist as a Word but only as an Adobe Acrobat version. The page numbering apart from figure 8) continues through the sections, beginning with the text and ending with the plates.
Binding energies and wave functions of inner-shell electronic states in superheavy quasimolecules with (Zp+Zt)α>1 are calculated. Ionization during a collision of very heavy ions is investigated within a molecular basis generated by the solutions of the two-center Dirac equation. Transitions to vacant bound states as well as direct excitation to the continuum are taken into account. We present theoretical values for the ionization probability as a function of impact parameter, bombarding energy, and combined nuclear charge. Our computed results are compared with recent experimental data. It is suggested that relativistic binding energies of electrons in superheavy quasimolecules can be determined experimentally via the impact-parameter dependence of ionization and the anisotropy of quasimolecular radiation.
The energy shift of K electrons in heavy atoms due to the self-energy correction has been calculated. This process is treated to all orders in Zα, where Z denotes the nuclear charge. For the superheavy system Z=170, where the K-shell binding energy reaches the pair-production threshold (E1sb∼2mc2), a shift of +11.0 keV is found. This shift is almost cancelled by the vacuum polarization, leaving a negligible effect for all quantum-electrodynamical corrections of order α but all orders of Zα.