Efficacy of eculizumab in a patient with immunoadsorption- dependent catastrophic antiphospholipid syndrome: A case report

Catastrophic antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with antiphospholipid syndrome (APS) with a high morbidity and mortality. We describe a case of a 30-year old female patient with immunoglobulin A (IgA) deficiency who underwent splenectomy because of idiopathic thrombocytopenic thrombocytopenia. Subsequently, an APS and finally systemic lupus erythematosus was diagnosed. After an uncomplicated pregnancy that was terminated by cesarean section, the patient developed severe CAPS with cerebral, myocardial, renal, and pulmonary involvement. Because of IgA deficiency, standard therapy consisting of plasmapheresis and intravenous immunoglobulins in addition to steroids was not tolerated. After 8 sessions of immunoadsorption (IAS), massive pulmonary hemorrhage was controlled but relapsed twice whenever IAS was terminated. As other immunosuppressive agents were considered dangerous because of the risk of infections in the face of severe hypogammaglobulinemia, we administered eculizumab, an inhibitor of the terminal complement pathway, which led to a persistent control of her disease. Interestingly, eculizumab therapy was associatedwith a further decline of complement C3 and C4 serumlevels. The patient developed a subsequent flare of her systemic lupus erythematosus, potentially indicating that complement inhibition by eculizumab is not effective in preventing lupus flares. Taken together, we describe a unique case of life-threatening and difficult-to-treat CAPS with a good clinical response after terminal complement complex inhibition with eculizumab. Further controlled trials are necessary to investigate the value of eculizumab in patients with CAPS

Biomechanical Properties of the Internal Limiting Membrane after Intravitreal Ocriplasmin Treatment

Purpose: To assess the stiffness of the human internal limiting membrane (ILM) and evaluate potential changes of mechanical properties following intravitreal ocriplasmin injection for vitreomacular traction. Methods: This is an interventional comparative case series of 12 surgically excised ILM specimens consecutively obtained from 9 eyes of 9 patients after unsuccessful pharmacologic vitreolysis with ocriplasmin. During the same time period, 16 specimens from 13 other eyes without ocriplasmin treatment were harvested during vitrectomy and served as controls. All patients presented with macular holes or vitreomacular traction and underwent vitrectomy with ILM peeling either with or without brilliant blue (BB) staining. All specimens were analyzed using atomic force microscopy with scan regions of 25 x 25 mu m. In all specimens, both the retinal side and vitreal side of the ILM were analyzed. Results: Atomic force microscopy revealed no significant differences in elasticity of ILM specimens removed from eyes with or without ocriplasmin treatment. Undulated areas of the retinal side presented stiffer than the vitreal side of the ILM. Topographical mapping of both the vitreal and retinal side of the ILM showed no apparent alteration of the morphology in ocriplasmin-treated eyes compared to untreated eyes. Staining with BB resulted in an increase of tissue stiffness. Conclusions: Intravitreal injection of ocriplasmin does not change biomechanical properties of the human ILM. There is no evidence of a potential enzymatic effect of ocriplasmin interfering with the stiffness of this basement membrane. (C) 2016 S. Karger AG, Base

Networking in regional development. The experience of the LEADER project "Wissbegierige Bildungsregion" (inquisitive education region) in the Weinviertel region

Die "Wissbegierige Bildungsregion" ist ein Bildungsprojekt der Region Weinviertel-Manhartsberg mit dem Ziel, Bildung in die Gemeinden und damit näher zur Bevölkerung des Weinviertels zu bringen. Dazu wurden in Dorfzentren sog. "Bildungsräume" eingerichtet und lernwilligen Gemeinde-Bewohner*innen zur Verfügung gestellt. Um diese Bildungsräume aktiv zu betreiben, brauchte es in jeder beteiligten Gemeinde ein Netzwerk an Personen, die sie betreuen und laufend aktivieren - mit verschiedenen Netzwerkaktivitäten. Dazu gab es regelmäßige Vernetzungstreffen, Schulungen der Bildungsraum-Verantwortlichen und Bildungsbörsen. Um aus Erfahrungen zu lernen und bestehende Bildungskonzepte für die Region zu adaptieren, wurde zudem ein Bildungs-Netzwerk mit regionalen und überregionalen Akteur*innen rund um den Bildungsbereich eingerichtet. Es entstand ein regionales Bildungsprogramm, das gemeinsam vermarktet wurde. Darüber hinaus gab es Kooperationen mit bestehenden Initiativen wie der "Dorfuni" oder der Montags-Akademie, beides Initiativen mit dem Ziel, Bildung für alle zu ermöglichen. Die Erfahrung zeigte: Die Motivation, in einem Netzwerk ehrenamtlich mitzuwirken, entsteht durch erlebte Sinnhaftigkeit, aber sie nimmt im Laufe der Zeit ab. Es ist leichter, neue Mitwirkende zu finden, als Mitwirkende dauerhaft zu halten. Und es braucht einen sehr klar formulierten persönlichen Nutzen, damit Personen an Weiterbildungen oder Veranstaltungen teilnehmen. Zusammengefasst: Es braucht Feingefühl für die richtigen Maßnahmen und das richtige Ausmaß und es bedarf vertrauensvoller Beziehungen sowie Lern-, Bildungs- und Netzwerkprozesse, die als sinnvoll und lebendig empfunden werden. (DIPF/Orig.)"Wissbegierige Bildungsregion" (Inquisitive Education Region) is an education project in the Weinviertel-Manhartsberg region that aims to bring education to municipalities and thus closer to the inhabitants of the Weinviertel. "Educational rooms" that are accessible to municipality residents interested in learning have been set up in the centre of villages. Each participating municipality needed a network of individuals responsible for different network activities and who actively operate these educational rooms. To this end, regular networking meetings, training for those responsible for the educational rooms and educational exchanges were organized. To learn from these experiences and adapt existing educational concepts to the region, an educational network with regional and transregional actors in the field of education was also set up. A regional educational programme was created that was jointly marketed. In addition, there was cooperation with existing initiatives such as the "Dorfuni" (Village Uni) or the Monday Academy, two initiatives whose goal is to facilitate education for everyone. The experience revealed that the commitment to being involved in a network on a voluntary basis arises through the experience of a sense of purpose, but it decreases over time. It is easier to find new volunteers than to retain volunteers permanently. For people to participate in continuing education or events, their personal benefit must be very clearly formulated. To sum up: Sensitivity to the right measures and the right number of activities is required, as are trusting relationships as well as learning, education and networking processes that are perceived as being meaningful and dynamic. (DIPF/Orig.

Automated Synthesis of Tableau Calculi

This paper presents a method for synthesising sound and complete tableau calculi. Given a specification of the formal semantics of a logic, the method generates a set of tableau inference rules that can then be used to reason within the logic. The method guarantees that the generated rules form a calculus which is sound and constructively complete. If the logic can be shown to admit finite filtration with respect to a well-defined first-order semantics then adding a general blocking mechanism provides a terminating tableau calculus. The process of generating tableau rules can be completely automated and produces, together with the blocking mechanism, an automated procedure for generating tableau decision procedures. For illustration we show the workability of the approach for a description logic with transitive roles and propositional intuitionistic logic.Comment: 32 page

Long-Term Socio-Ecological Research in Practice: Lessons from Inter- and Transdisciplinary Research in the Austrian Eisenwurzen

Long-Term Socio-Ecological Research (LTSER) is an inter- and transdisciplinary research field addressing socio-ecological change over time at various spatial and temporal scales. In the Austrian Eisenwurzen region, an LTSER platform was founded in 2004. It has fostered and documented research projects aiming at advancing LTSER scientifically and at providing regional stakeholders with relevant information for sustainable regional development. Since its establishment, a broad range of research activities has been pursued in the region, integrating information from long-term ecological monitoring sites with approaches from social sciences and the humanities, and in cooperation with regional stakeholders. Based on the experiences gained in the Eisenwurzen LTSER platform, this article presents current activities in the heterogeneous field of LTSER, identifying specific (inter-)disciplinary contributions of three research strands of LTSER: long-term ecological research, socio-ecological basic research, and transdisciplinary research. Given the broad array of diverse contributions to LTSER, we argue that the platform has become a relevant "boundary organization", linking research to its regional non-academic context, and ensuring interdisciplinary exchange among the variety of disciplines. We consider the diversity of LTSER approaches an important resource for future research. Major success criteria of LTSER face specific challenges: (1) existing loose, yet stable networks need to be maintained and extended; (2) continuous generation of and access to relevant data needs to be secured and more data need to be included; and (3) consecutive research projects that have allowed for capacity building in the past may be threatened in the future if national Austrian research funders cease to provide resources

Signal transduction in light-oxygen-voltage receptors lacking the active-site glutamine

In nature as in biotechnology, light-oxygen-voltage photoreceptors perceive blue light to elicit spatiotemporally defined cellular responses. Photon absorption drives thioadduct formation between a conserved cysteine and the flavin chromophore. An equally conserved, proximal glutamine processes the resultant flavin protonation into downstream hydrogen-bond rearrangements. Here, we report that this glutamine, long deemed essential, is generally dispensable. In its absence, several light-oxygen-voltage receptors invariably retained productive, if often attenuated, signaling responses. Structures of a light-oxygen-voltage paradigm at around 1 Å resolution revealed highly similar light-induced conformational changes, irrespective of whether the glutamine is present. Naturally occurring, glutamine-deficient light-oxygen-voltage receptors likely serve as bona fide photoreceptors, as we showcase for a diguanylate cyclase. We propose that without the glutamine, water molecules transiently approach the chromophore and thus propagate flavin protonation downstream. Signaling without glutamine appears intrinsic to light-oxygen-voltage receptors, which pertains to biotechnological applications and suggests evolutionary descendance from redox-active flavoproteins

Ergänzung zur Stellungnahme des Zentrums für Lehrer*innenbildung der Universität Wien zum Lehrplan Digitale Grundbildung

Das neue Schulfach Digitale Grundbildung (DGB) wurde als Pflichtfach in der Sekundarstufe I mit überarbeitetem Lehrplan verankert – ein Grund zur Freude, dass der Digitalisierung auch innerhalb der schulische Bildung endlich die nötige Aufmerksamkeit gegeben wird. Das Fach stellt, besonders vor dem Hintergrund der Initiativen, die im Rahmen des 8-Punkte Plans der Bundesregierung gesetzt wurden, die notwendige Brücke zwischen Infrastruktur-Maßnahmen, Inhalten und der tatsächlichen Nutzung dieser Ressourcen dar. Das neue Schulfach Digitale Grundbildung – eine Projektionsfläche für mannigfaltige Bedürfnislagen. Aus Informatikperspektive und Sicht der Wirtschaft bietet es eine Gelegenheit, kompetenzorientierten Informatikunterricht schon in der Sekundarstufe I beginnen zu lassen. Aus medienpädagogischem Betrachtungswinkel ist das Fach ein lang gewünschtes Gefäß, um jungen Menschen ein besseres Verständnis von Medien und eine kompetente Mediennutzung nahezubringen. Aus der im Folgenden abgebildeten Perspektive des Zentrums für Lehrer*innenbildung der Universität Wien bietet das Schulfach den notwendigen Rahmen, um zumindest ansatzweise inklusive, diversitätsgerechte und interdisziplinäre Perspektiven auf die Digitalisierung und ihre sozialen, kulturellen, politschen und gesellschaftlichen Aus- und Wechselwirkungen zusammenzuführen