158 research outputs found
Setleis Syndrome: Genetic and Clinical Findings in a New Case With Epilepsy
No full textFocal facial dermal dysplasias are a group of inherited ectodermal disorders characterized by congenital bitemporal or periauricular scar-like depressions as well as other facial and nonfacial developmental defects. Four subtypes have been delineated, and mutations in the TWIST2 gene have been identified in type III focal facial dermal dysplasia (Setleis syndrome).
PATIENTS:
We describe a sporadic patient with the hallmark bitemporal scar-like lesions, severe intellectual disability, and focal epilepsy.
RESULTS:
The boy has typical features of Setleis syndrome, and he developed focal epilepsy, a previously unreported feature of this syndrome. No mutations in the TWIST2 gene were found, and there were no pathologic copy number abnormalities.
CONCLUSIONS:
Epilepsy could represent a new manifestation, and the patient described broadens the spectrum of clinical features associated with Setleis syndrome, including central nervous system involvement
A Structurally Characterized Organometallic Plutonium(IV) Complex
No full textbibtex: ISI:000399384700030 bibtex\location:'POSTFACH 101161, 69451 WEINHEIM, GERMANY',publisher:'WILEY-V C H VERLAG GMBH',type:'Article',affiliation:'Walter, O (Reprint Author), European Commiss, Joint Res Ctr, Directorate Nucl Safety & Secur G, POB 2340, D-76125 Karlsruhe, Germany. Vogt, J; Edelmann, FT (Reprint Author), Otto von Guericke Univ, Inst Chem, Univ Pl 2, D-39106 Magdeburg, Germany. Maron, L (Reprint Author), Univ Toulouse, INSA, CNRS, Lab Phys & Chim Nanoobjets LPCNO,UMR5215, 135 Ave Rangueil, F-31077 Toulouse 4, France. Apostolidis, Christos; Walter, Olaf, European Commiss, Joint Res Ctr, Directorate Nucl Safety & Secur G, POB 2340, D-76125 Karlsruhe, Germany. Vogt, Jochen; Liebing, Phil; Edelmann, Frank T., Otto von Guericke Univ, Inst Chem, Univ Pl 2, D-39106 Magdeburg, Germany. Maron, Laurent, Univ Toulouse, INSA, CNRS, Lab Phys & Chim Nanoobjets LPCNO,UMR5215, 135 Ave Rangueil, F-31077 Toulouse 4, France.','author-email':'[email protected] [email protected] [email protected] [email protected]',da:'2018-12-05','doc-delivery-number':'ES2UZ',eissn:'1521-3773','funding-acknowledgement':'Otto-von-Guericke-Universitat Magdeburg','funding-text':'We thank the Otto-von-Guericke-Universitat Magdeburg for general financial support.','journal-iso':'Angew. Chem.-Int. Edit.','keywords-plus':'TRIS(ETA-5-CYCLOPENTADIENYL) ACTINIDE COMPLEXES; FOCK PERTURBATION-THEORY; BUILDING-BLOCK APPROACH; F-ORBITAL COVALENCY; SANDWICH COMPLEXES; ELECTRONIC-STRUCTURE; CRYSTAL-STRUCTURE; CYCLOOCTATETRAENYL COMPLEXES; TH-PU; ACTINOCENES','number-of-cited-references':'69',oa:'Other Gold, Green Published','orcid-numbers':'Edelmann, Frank T./0000-0001-5209-0018 walter, olaf/0000-0002-2679-1715 Liebing, Phil/0000-0002-4660-1691','research-areas':'Chemistry','times-cited':'8','unique-id':'ISI:000399384700030','usage-count-last-180-days':'2','usage-count-since-2013':'23','web-of-science-categories':'Chemistry, Multidisciplinary'\International audienceThe blood-red plutonocene complex Pu(1,3-COT `')(1,4-COT `') (4; COT `' = eta(8)-bis(trimethylsilyl) cyclooctatetraenyl) has been synthesized by oxidation of the anionic sandwich complex Li[Pu(1,4-COT `')(2)] (3) with anhydrous cobalt(II) chloride. The first crystal structure determination of an organoplutonium(IV) complex revealed an asymmetric sandwich structure for 4 where one COT `' ring is 1,3-substituted while the other retains the original 1,4-substitution pattern. The electronic structure of 4 has been elucidated by a computational study, revealing a probable cause for the unexpected silyl group migration
Business valuation in financial distress
No full textInvestoren und Sachverständige setzen sich täglich mit der Frage nach der Höhe des Unternehmenswertes auseinander. Die häufigste Methode zur Feststellung des Unternehmenswertes stellen die Diskontierungs- und Multiplikatorverfahren dar. Welches Verfahren für die Bewertung herangezogen wird, ist abhängig von Bewertungsanlass bzw. -zweck. Bei den Diskontierungsverfahren, bei denen der Unternehmenswert durch Kapitalisierung von Cashflows ermittelt wird, wird in der Praxis primär von der Going-Concern-Prämisse ausgegangen respektive eine unendliche Lebensdauer angenommen. Da Unternehmensinsolvenzen oftmals in den ersten Jahren nach Gründung eintreten, scheint es angebracht zu sein, das Insolvenzrisiko in die Bewertung miteinfließen zu lassen, um Verzerrungen im Ergebnis und folglich Fehlentscheidungen zu vermeiden. Für den Bewerter stellt sich daher die Frage nach der Ermittlung des Insolvenzrisikos und in weiterer Folge wie das Risiko in der Bewertung berücksichtigt werden kann. Die vorliegende Arbeit zeigt zunächst die Möglichkeiten auf, anhand deren das Insolvenzrisiko berechnet werden kann und auf welche Weise der ermittelte Parameter folglich in die Bewertung einfließen kann dies wird anhand eines hypothetischen Beispiels dargelegt und gewürdigt. Folglich wurde festgestellt, dass das Insolvenzrisiko im deutschsprachigen Raum grundsätzlich als Abschlag bei der Ermittlung der finanziellen Überschüsse zu berücksichtigen ist, sofern eine Berücksichtigung nicht bereits bei der Ermittlung der Cashflows bedacht wurde wie beispielsweise bei Anwendung eines simulationsbasierten Ratings, z. B. Monte-Carlo-Simulation. Aus den Ergebnissen der vorliegenden Arbeit kann geschlossen werden, dass die Berücksichtigung des Insolvenzrisikos bei Vorliegen eines bewertungsrelevanten Risikos erforderlich ist, da das Ergebnis ansonsten erheblich verzerrt würde. Ob eine Berücksichtigung zu erfolgen hat, muss allerdings individuell betrachtet werden.Investors and experts are confronted with the question of what the value of a company is on a daily basis. The most common methods in the valuation of a company are the discounted cashflow method and the multiplier method. Which method is used for the valuation depends on the purpose of the valuation. In case of the discounted cashflow method, in which the value of a firm is calculated by capitalizing future cashflows, the going concern principle is usually applied and thus, an infinite lifespan is assumed. Since corporate insolvencies often occur in the first years after formation of the company, it seems adequate to include an insolvency risk in the valuation in order to avoid a bias in the valuation of a firm and consequently wrong management decisions. Hence, the question of how to calculate and subsequently how to consider the risk in valuation arises. Therefore, this master thesis deals with the different ways of how the insolvency risk in valuation can be calculated and subsequently, how this parameter can successfully be incorporated in valuations. Therefore, a hypothetical example has been created where the methods of calculating and the necessity of considering the insolvency risk are assessed. This thesis shows that in the German-speaking area the insolvency risk must be considered as a discount on the cashflow, unless the insolvency risk has not already been taken into account when determining the cashflows, such as when using a simulation-based rating, for example the Monte-Carlo-Simulation. From the results of the hypothetical example it can be concluded that a consideration of the insolvency risk is necessary, given the case there is a relevant insolvency risk, since otherwise the result would be significantly distorted. Nevertheless, whether the insolvency risk has to be included in the valuation or not, has to be considered individually in any valuation case.Lisa Edelmann, BScZusammenfassungen in Deutsch und EnglischAbweichender Titel laut Übersetzung des Verfassers/der VerfasserinMasterarbeit Karl-Franzens-Universität Graz 2020 D1141
Comparison of EKF and UKF for vehicle state estimation by means of two-wheel vehicle model
No full textDiese Diplomarbeit handelt vom Thema Zustandsbeobachtung der Querdynamik eines Automobils. Als Fahrzeugmodell wird ein ebenes Zweiradmodell verwendet, bei dem die beiden Spuren des Autos auf eine Spur in der Symmetrieebene des Fahrzeugs zusammengefasst werden. Betrachtet wird ein fünfdimensionales Zustandsraumsystem mit der Längs- und Quergeschwindigkeit des Schwerpunkts, der Gierrate und den beiden Radwinkelgeschwindigkeiten als Zustandsgrößen. Als Eingangsgrößen fungieren der Lenkwinkel des Vorderrades, Antriebs- und Bremsmoment. Ziel dieser Arbeit ist ein Vergleich unterschiedlicher Zustandsbeobachter in Bezug auf deren Aufbau, Funktionsweise und Fähigkeiten. Verglichen werden sollen dabei drei unterschiedliche diskrete Kalman Filter, Linearer Kalman Filter, Extended Kalman Filter und Unscented Kalman Filter, bei unterschiedlichen Fahrmanövern und bei unterschiedlichen Abtastzeiten.This diploma thesis deals with the topic of state observation of the lateral dynamics of an automobile. A planar two-wheel vehicle model is used, where the two tracks of the car are grouped together on a single track in the plane of symmetry of the vehicle. A five dimensional state space system is considered. The state variables are the longitudinal and lateral velocity of the center of gravity, the yaw rate and the wheel angular velocities. The steering angle of the front wheel and the drive and braking torque act as input variables. The aim of this thesis is a comparison of different state observers in terms of their structure and functionality. This analysis compares three different discrete Kalman Filters, Linear Kalman Filter, Extended Kalman Filter and Unscented Kalman Filter, by means of different maneuvers and different sampling times
Shaping a Data Commoning Polity: Prospects and Challenges of a European Digital Sovereignty
No full textThe concept of “digital sovereignty” has gained momentum due to the emergence of a multipolar geopolitical scenario based upon different visions of today’s digital society. In this scenario, the United States, China, and the European Union are major players, each pursuing their understanding of digital sovereignty and their approach to digital transformation. The EU conceives of digital sovereignty as technological autonomy from other competitors, and to achieve this it has carved for itself the role of international regulator. De facto, however, the EU enacts an individual-centric and economic-driven digital strategy that hinders the possibility of a fully-fledged European digital sovereignty. Notably, the concept fails to embed the collective-level dimension proper to sovereignty as such. To tackle this, the paper explores data commoning as the basis for shaping a well-formed European polity, key to its digital sovereignty.Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Urban Data Scienc
Hack The Government!: Empowering Citizens to Make Meaningful Use of Open Data
No full textCitizens’ use of Open Data is often limited to the use of apps. The design of the (app based) public service as well as the interpretation of the data is done for them. The organization of local hackathons can promote citizens’ data literacy and better use of availableOpen Data for service transformation purposes. This promises to nicely combine citizen intelligence with participatory innovation with and by public service beneficiaries as the “next wave” of inclusive (e-)Government practice. In the proposed workshop, we simulate theorganization and dynamics of a Hackathon based on the evidence at hand.Design Conceptualization and Communicatio
Effects of wheelset flexibility on the simulation of vehicle-track interaction at crossings
No full textThough numerical models based on multi-body dynamics (MBD) are often used to simulate the vehicle-track interaction at crossing impact, their relative capabilities and accuracy were not discussed. This paper aims to investigate the influence of wheelset flexibility on the result of crossing impact simulation in the frequency range 0–500 Hz. Two models are used: a MBD model with rigid wheelset and a reference finite element model that could fully account for the structural flexibility. Results of the two models as well as in-situ measurement show three characteristic frequencies of the vehicle-track interaction system induced by crossing impact. Based on the characteristic frequencies, the MBD model is tuned to resemble the reference FE model in terms of track and contact representation through a parametric analysis so that the influence of these differences can be isolated. It is found that the major influence of the wheelset flexibility is on the second characteristic frequency of the system, reflecting the second order bending of the wheelset.Railway Engineerin
Citizens’ Motivations for Engaging in Open Data Hackathons
No full textEngaging citizens in open data hackathons provides opportunities for innovation and the generation of new services and products. This paper aims to explore the motivations of citizens who engage in open agriculture data hackathons. We conducted a case study and analyzed data collected from 161 participants of 11 farming hackathons held between 2016 and 2018 in the Netherlands. We found that participants of open agriculture data hackathons have different roles, including business developer, concept thinker, data analyst, data owner, developer, manager, marketer, problem owner, and student. Our analysis shows that citizens are predominantly motivated to engage in open agricultural data hackathons as part of their work. Furthermore, developers and problem owners are mainly motivated by fun and enjoyment. This indicates that it is important for open data policymakers and hackathon organizers to consider different approaches based on citizens’ roles when organizing open data hackathons. This paper contributes to the literature by providing insight in the motivations of citizens engaging in open agriculture data hackathons in comparison with hackathons in other sectors, and by mapping citizens’ roles to their motivations for engaging in such hackathons.Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Information and Communication Technolog
Clinical Utility of Array CGH for the Detection of Chromosomal Imbalances Associated with Mental Retardation and Multiple Congenital Anomalies
No full text- …
