Refine
Year of publication
Document Type
- Article (11)
- Conference Proceeding (5)
- Working Paper (2)
- Lecture (1)
- Report (1)
Language
- English (20) (remove)
Has Fulltext
- yes (20)
Is part of the Bibliography
- no (20)
Keywords
Institute
- Physik (18)
- Biowissenschaften (1)
- LOEWE-Schwerpunkt für Integrative Pilzforschung (1)
- Medizin (1)
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.
Despite an increasing demand for Burgundy truffles (Tuber aestivum), gaps remain in our understanding of the fungus’ overall lifecycle and ecology. Here, we compile evidence from three independent surveys in Hungary and Switzerland. First, we measured the weight and maturity of 2,656 T. aestivum fruit bodies from a three-day harvest in August 2014 in a highly productive orchard in Hungary. All specimens ranging between 2 and 755 g were almost evenly distributed through five maturation classes. Then, we measured the weight and maturity of another 4,795 T. aestivum fruit bodies harvested on four occasions between June and October 2015 in the same truffière. Again, different maturation stages occurred at varying fruit body size and during the entire fruiting season. Finally, the predominantly unrelated weight and maturity of 81 T. aestivum fruit bodies from four fruiting seasons between 2010 and 2013 in Switzerland confirmed the Hungarian results. The spatiotemporal coexistence of 7,532 small-ripe and large-unripe T. aestivum, which accumulate to ~182 kg, differs from species-specific associations between the size and ripeness that have been reported for other mushrooms. Although size-independent truffle maturation stages may possibly relate to the perpetual belowground environment, the role of mycelial connectivity, soil property, microclimatology, as well as other abiotic factors and a combination thereof, is still unclear. Despite its massive sample size and proof of concept, this study, together with existing literature, suggests consideration of a wider ecological and biogeographical range, as well as the complex symbiotic fungus-host interaction, to further illuminate the hidden development of belowground truffle fruit bodies.
The operation of a Free Electron Laser (FEL) in the ultraviolet or in the X-ray regime requires the acceleration of electron bunches with an rms length of 25 to 50 mikrometer. The wakefields generated by these sub picosecond bunches extend into the frequency range well beyond the threshold for Cooper pair breakup (about 750 GHz) in superconducting niobium at 2 K. It is shown, that the superconducting cavities can indeed be operated with 25 mikrometer bunches without suffering a breakdown of superconductivity (quench), however at the price of a reduced quality factor and an increased heat transfer to the superfluid helium bath. This was first shown by wakefield calculations based on the diffraction model [1]. In the meantime a more conventional method of computing wake fields in the time domain by numerical methods was developed and used for the wakefield calculations [2]. Both methods lead to comparable results: the operation of TESLA with 25 mikrometer bunches is possible but leads to an additional heat load due to the higher order modes (HOMs). Therefore HOM dampers for these high frequencies are under construction [3]. These dampers are located in the beam pipes between the 9-cell cavities. So it is of interest, if there are trapped modes in the cavity due to closed photon orbits. In this paper we investigate the existence of trapped modes and the distribution of heat load over the surface of the TESLA cavity by numerical photon tracking.
Accelerating cavities exchange HOM power through interconnecting beam pipes in case of signal frequencies above the cut-off of their propagating waveguide modes. This may lead either to improved HOM damping or - in the case most severe - to unwanted phase coherence of fields to the beam. Therefore the knowledge of the scattering properties of a cavity as a line element is needed to analyse all kinds of RF cavity-cavity interaction. Since there is a lack of measurement tools capable to provide a multidimensional scattering matrix at a given frequency point, we have been developing a method for this purpose. It uses a set of 2-port S-parameters of the device under test, embedded in a number of geometrically different RF environments. The application of the method is demonstrated with copper models of TESLA cavities.
To preserve the required beam quality in an e+/e- collider it is necessary to have a very precise beam position control at each accelerating cavity. An elegant method to avoid additional length and beam disturbance is the usage of signals from existing HOM-dampers. The magnitude of the displacement is derived from the amplitude of a dipole mode whereas the sign follows from the phase comparison of a dipole and a monopole HOM. To check the performance of the system, a measurement setup has been built with an antenna which can be moved with micrometer resolution to simulate the beam. Furthermore we have developed a signal processing to determine the absolute beam displacement. Measurements on the HOM-damper cell can be done in the frequency domain using a network analyser. Final measurements with the nonlinear time dependent signal processing circuit has to be done with very short electric pulses simulating electron bunches. Thus, we have designed a sub nanosecond pulse generator using a clipping line and the step recovery effect of a diode. The measurement can be done with a resolution of about 10 micrometers. Measurements and numerical calculations concerning the monitor design and the pulse generator are presented.
Due to the additional need of very short bunches for the FEL operation with the TESLA-machine strong wakefield effects are expected. One third of the total wakefield energy per bunch is radiated into the frequency region above the energy gap of Cooper pairs in superconducting niobium. The energy of the cooper pairs in superconducting niobium at 2 K corresponds to a frequency of 700 GHz. An analytical and experimental estimation for the overall energy loss of the FEL bunch above energy gap is presented. The analytical method is based on a study from R. B. Palmer [1]. The results of the wakefield estimations are used to calculate possible quality factor reduction of the TESLA cavities during FEL operation. Results are presented.
A new method for the determination of S-matrices of devices in multimoded waveguides and first experimental experiences are presented. The theoretical foundations are given. The scattering matrix of a TESLA copper cavity at a frequency above the cut-off of the second waveguide mode has been measured.
A new method of measuring quality factors in cavities is presented. This method is well suited to measure quality factors in undamped cavities as well as in heavily damped cavities, and in addition this method provides a possibility of separating modes and measuring quality factors especially in cases of overlapping modes. Measurements have been carried out on HOM-damped cavities for the DESY/THD linear collider project. Results are presented.
Damping cells for the higher order modes are necessary for the S-band linear collider to minimize BBU (Beam-Break-Up). The construction of the damper cells has to take into account the different field geometries of the higher order modes. So two different types of dampers have been designed: a wall slotted an an iris slotted cell. In order to optimize the two types of damping cells with respect to damping strength, impedance matching between coupling system and waveguide dampers and between damping cell and undamped cells and the tuning system, damping cells of both types have been built and examinated.