Search results for "Hunter"
showing 10 items of 61 documents
Studienbegleitender und studienvorbereitender Deutschunterricht international: Einführung
2023
Studienbegleitender und studienvorbereitender Deutschunterricht an Universitäten und Hochschulen steht weltweit vor Herausforderungen, sich den Anforderungen des globalen Wissenschaftsbetriebs zu stellen. Zum einen ist die Bedeutung des Deutschen in den letzten Jahren dramatisch gesunken, zum anderen sind innovative didaktische Ansätze für den Deutschunterricht gefragt. In welcher Weise sich studienbegleitender und studienvorbereitender Deutschunterricht diesen Anforderungen im In- und Ausland stellen, wird in diesem Themaheft beleuchtet. An zehn Beiträgen aus unterschiedlichen Ländern wird unter anderem deutlich, wie aktive Sprachenpolitik durch strategische Investitionen einerseits und En…
Vorbereitung von Austauschstudierenden auf ein Studium in einem deutschsprachigen Land : Ein textsortenorientiertes Blended-Learning-Konzept
2023
Deutschland ist nach wie vor das beliebteste Zielland finnischer Studierender, und das wichtigste Motiv für die Wahl eines deutschsprachigen Gastlandes ist, Deutsch zu lernen. In diesem Beitrag soll das Konzept eines Blended-Learning-Kurses zur Vorbereitung auf ein Austauschstudium in einem deutschsprachigen Land vorgestellt werden. Der Kurs war handlungs- und textsortenorientiert auf den Erwerb mündlicher studienbezogener Fertigkeiten ausgerichtet. Für das E-Learning wurde das deutsche EUROMOBIL-Programm (Euromobil o.J.) verwendet. In den Präsenzphasen hielten die Studierenden mündliche Referate, die im Kurs diskutiert und von den Lehrenden und Vortragenden selbst evaluiert wurden. Im empi…
Characteristics of patients with mucopolysaccharidosis type II who have received a bone marrow transplant: Data from the Hunter Outcome Survey
2019
Mucopolysaccharidosis type II (MPS II Hunter syndrome) is a rare, life-limiting, X-linked lysosomal storage disease. The Hunter Outcome Survey (HOS) is a Shire-sponsored, global, observational registry initiated in 2005 that collects real-world data on the natural history of MPS II and long-term treatment with enzyme replacement therapy (ERT) with idursulfase. Patients receiving other forms of pharmacological ERT are excluded from HOS but individuals who have received a bone marrow transplant (BMT) may be enrolled. This analysis examined the characteristics of 36 male patients in HOS from Europe and North America who had received a BMT (March 2018 data). In total, 22 patients (61.1%) were E…
Comment: Why are females with Fabry disease affected?
2019
29 Clinical benefit of enzyme replacement therapy (ERT) in mucopolysaccharidosis II (MPS II, Hunter syndrome)
2007
Orthopedic manifestations in patients with mucopolysaccharidosis type II (Hunter syndrome) enrolled in the Hunter Outcome Survey.
2010
Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare, inherited disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulfatase. As a result of this deficiency, glycosaminoglycans accumulate in lysosomes in many tissues, leading to progressive multisystemic disease. The cardiopulmonary and neurological problems associated with MPS II have received considerable attention. Orthopedic manifestations are common but not as well characterized. This study aimed to characterize the prevalence and severity of orthopedic manifestations of MPS II and to determine the relationship of these signs and symptoms with cardiovascular, pulmonary and central nervous system involvemen…
Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome.
2011
Purpose: This study evaluated the safety and effectiveness of long-term enzyme replacement therapy with idursulfase (recombinant human iduronate-2-sulfatase) in patients with Hunter syndrome. Methods: All 94 patients who completed a 53-week double-blinded study of idursulfase enrolled in this open-labeled extension study and received intravenous idursulfase at a dose of 0.5 mg/kg weekly for 2 years, and clinical outcomes and safety were assessed. Results: No change in percent predicted forced vital capacity was seen, but absolute forced vital capacity demonstrated sustained improvement and was increased 25.1% at the end of the study. Statistically significant increases in 6-minute walking t…
Typification of five plant names described based on specimens collected by Józef Warszewicz in Central and South America.
2022
Józef Warszewicz (1812–1864) was one of the first Polish naturalists to explore the flora of the tropical New World. During two expeditions to Central and South America (1844–1850 and 1850–1853) he collected and delivered to Europe up to twenty thousand plant specimens. To honour his service and his achievements in plant collections, different taxonomists described more than 100 taxa using the surname Warszewicz, for example in the genus name (Warszewiczia) and the species epithets (warszewiczii, warscewiczii, warszewicziana). Unfortunately, a large part of Warszewicz’s collection of plant species deposited in the Berlin Herbarium (B), including many type specimens was destroyed during Worl…
Supplementary material 1 from: Nobis M, Klichowska E, Wolanin M, Nobis A, Nowak A (2022) Typification of five plant names described based on specimen…
2022
Table S1