@article{OrtizAbioseBichetetal.2016, author = {Ortiz, Alberto and Abiose, Ademola and Bichet, Daniel G. and Cabrera, Gustavo and Charrow, Joel and Germain, Dominique P. and Hopkin, Robert J. and Jovanovic, Ana and Linhart, Aleš and Maruti, Sonia S. and Mauer, Michael and Oliveira, Jo{\~a}o P. and Patel, Manesh R. and Politei, Juan and Waldek, Stephen and Wanner, Christoph and Yoo, Han-Wook and Warnock, David G.}, title = {Time to treatment benefit for adult patients with Fabry disease receiving agalsidase beta: data from the Fabry Registry}, series = {Journal of Medical Genetics}, volume = {53}, journal = {Journal of Medical Genetics}, number = {7}, doi = {10.1136/jmedgenet-2015-103486}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-188241}, pages = {495-502}, year = {2016}, abstract = {Background Agalsidase beta is a form of enzyme replacement therapy for Fabry disease, a genetic disorder characterised by low alpha-galactosidase A activity, accumulation of glycosphingolipids and life-threatening cardiovascular, renal and cerebrovascular events. In clinical trials, agalsidase beta cleared glycolipid deposits from endothelial cells within 6 months; clearance from other cell types required sustained treatment. We hypothesised that there might be a 'lag time' to clinical benefit after initiating agalsidase beta treatment, and analysed the incidence of severe clinical events over time in patients receiving agalsidase beta. Methods The incidence of severe clinical events (renal failure, cardiac events, stroke, death) was studied in 1044 adult patients (641 men, 403 women) enrolled in the Fabry Registry who received agalsidase beta (average dose 1 mg/kg every 2 weeks) for up to 5 years. Results The incidence of all severe clinical events was 111 per 1000 person-years (95\% CI 84 to 145) during the first 6 months. After 6 months, the incidence decreased and remained stable within the range of 40-58 events per 1000 patient-years. The largest decrease in incidence rates was among male patients and those aged >= 40 years when agalsidase beta was initiated. Conclusions Contrary to the expected increased incidence of severe clinical events with time, adult patients with Fabry disease had decreased incidence of severe clinical events after 6 months treatment with agalsidase beta 1 mg/kg every 2 weeks.}, language = {en} } @inproceedings{OPUS4-13940, title = {Neue Materialien des Bayerischen Neolithikums - Tagung im Kloster Windberg vom 21. bis 23. November 2014}, series = {W{\"u}rzburger Studien zur Vor- und Fr{\"u}hgeschichtlichen Arch{\"a}ologie}, booktitle = {W{\"u}rzburger Studien zur Vor- und Fr{\"u}hgeschichtlichen Arch{\"a}ologie}, editor = {Pechtl, Joachim and Link, Thomas and Husty, Ludwig}, publisher = {W{\"u}rzburg University Press}, address = {W{\"u}rzburg}, isbn = {978-3-95826-044-3 (print)}, issn = {2367-0681 (print)}, doi = {10.25972/WUP-978-3-95826-045-0}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-139407}, pages = {148}, year = {2016}, abstract = {Unter dem Titel "Neue Materialien des Bayerischen Neolithikums" fand vom 21. bis 23. November 2014 im Kloster Windberg bei Straubing eine Tagung statt, bei der neue, bislang unpublizierte jung-steinzeitliche Fundkomplexe vorgestellt und diskutiert wurden, die aus allen Landesteilen des Freistaats Bayern stammten und zeitlich den Bogen von der Linienbandkeramik bis zu den Becherkulturen spannten. Mit einer diachronen und Regionen {\"u}bergreifenden Betrachtung charakteristischer Inventare aus den verschiedenen Landschaftsr{\"a}umen und Zeitabschnitten wurde das Ziel verfolgt, neues Material zu erschließen und damit die Quellenbasis f{\"u}r die arch{\"a}ologische Forschung zu erweitern sowie der Neolithforschung in Bayern neue Impulse zu geben. Im vorliegenden Band sind neun der Vortr{\"a}ge dieser Tagung zusammen gestellt.}, subject = {Bayern}, language = {de} }