A Cyanotic Newborn with a Pink Right Upper Extremity

Aberrant origin of the subclavian artery (SCA) is a well-known vascular anomaly as part of congenital heart diseases with the left subclavian artery (LSCA) being more frequently affected than the right subclavian artery (RSCA). Complete isolation of the SCA is an even more infrequent aortic arch anomaly, occurring in less than 1% for the LSCA and even less for the RSCA. Isolation of the RSCA in patients with d-transposition of the great arteries (D-TGA) is even scanter with only a hand full of cases being reported in the literature. However, isolation of the RSCA has important implications on hemodynamics and surgical strategies. In this case report, we present a newborn patient with D-TGA which presented with distinct differential cyanosis. While the right upper extremity appeared pink with an oxygen saturation of 100%, the rest of the body was cyanotic. At first, this appearance was interpreted as the Harlequin phenomenon during primary care. However, detailed echocardiography revealed an aberrant origin of the RSCA from the right pulmonary artery, which led to the differential cyanosis. The patient underwent arterial switch operation on day of life two including dissection and reimplantation of the RSCA. The special hemodynamic situation of this is discussed in terms of pathophysiology and as well as its impact on perioperative and surgical management

Die österreichische EOSC Mandated Organisation / Das EOSC Support Office Austria

This report offers some reflections for a better understanding of the realization of the European Open Science Cloud (EOSC) at a local level, describing the implementation of the newly established Austrian EOSC Mandated Organisation and the EOSC Support Office Austria.Dieser Bericht bietet einige Überlegungen zum besseren Ver-ständnis der Umsetzung der European Open Science Cloud (EOSC) auf nationaler Ebene und schildert die Entstehung der neu gegründeten österreichischen EOSC Man-dated Organisation und des EOSC Support Office Austria.1431622

The Austrian EOSC Mandated Organisation / The EOSC Support Office Austria

Dieser Bericht bietet einige Überlegungen zum besseren Verständnis der Umsetzung der European Open Science Cloud (EOSC) auf nationaler Ebene und schildert die Entstehung der neu gegründeten österreichischen EOSC Mandated Organisation und des EOSC Support Office Austria.This report offers some reflections for a better understanding of the realization of the European Open Science Cloud (EOSC) at a local level, describing the implementation of the newly established Austrian EOSC Mandated Organisation and the EOSC Support Office Austria

Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome

Objective Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. Methods We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Results Eighteen patients were included (median age 55.5 years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0–4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8 months, range 17,25–108 months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. Conclusion AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.status: publishe

The High Brilliance neutron Source (HBS): A project for a next generation neutron research facility

The High Brilliance neutron Source (HBS) is a project for a next generation neutron research facility, based on new concepts and recent technological advancements. As elementary processes it uses neither fission nor spallation, but instead low energy nuclear reactions in a very compact Target-ModeratorReflector (TMR) assembly. Our facility design results in very efficient production of neutron beams with high brightness. Key features of HBS are: (i) very competitive instrument performance, (ii) comparatively low construction and operation costs, (iii) resilience, (iv) sustainability, (v) flexibility, (vi) accessibility and (vii) scalability. Here we present the basic layout of the facility, elaborate on the mentioned key features and report on the commissioning of a small test setup