Improved 6-year overall survival in AT/RT - results of the registry study Rhabdoid 2007

Atypical teratoid rhabdoid tumors (AT/RT) are characterized by mutations and subsequent inactivation of SMARCB1 (INI1, hSNF5), a predilection for very young children and an unfavorable outcome. The European Registry for rhabdoid tumors (EU-RHAB) was established to generate a common European database and to establish a standardized treatment regimen as the basis for phase I/II trials. Thus, genetic analyses, neuropathologic and radiologic diagnoses, and a consensus treatment regimen were prospectively evaluated. From 2005 to 2009, 31 patients with AT/RT from four countries were recruited into the registry study Rhabdoid 2007 and treated with systemic and intraventricular chemotherapy. Eight patients received high-dose chemotherapy, 23 radiotherapy, and 17 maintenance therapy. Reference evaluations were performed in 64% (genetic analyses, FISH, MLPA, sequencing) up to 97% (neuropathology, INI1 stain). Germ-line mutations (GLM) were detected in 6/21 patients. Prolonged overall survival was associated with age above 3years, radiotherapy and achievement of a complete remission. 6-year overall and event-free survival rates were 46% (+/- 0.10) and 45% (+/- 0.09), respectively. Serious adverse events and one treatment-related death due to insufficiency of a ventriculo peritoneal shunt (VP-shunt) and consecutive herniation were noted. Acquisition of standardized data including reference diagnosis and a standard treatment schedule improved data quality along with a survival benefit. Treatment was feasible with significant but manageable toxicity. Although our analysis is biased due to heterogeneous adherence to therapy, EU-RHAB provides the best available basis for phase I/II clinical trials

Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients

BACKGROUND: The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity. OBJECTIVE: To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus. METHODS: Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%). RESULTS: Seropositive patients were found to be predominantly female (p 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome. CONCLUSION: This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients

Seasonality, resource stress, and food sharing in so-called "egalitarian" foraging societies

Most discussions of food sharing among so-called "egalitarian" hunters and gatherers implicitly assume that, because all adult members of a group participate in the network of sharing, all must therefore be receiving portions of more or less equivalent nutritional worth. This assumption is questioned and five basic points are raised: (1) because fat is not uniformly distributed over the carcass of an animal and because it is depleted sequentially when an animal is stressed, certain individuals may receive nutritionally inferior portions of meat, with potentially serious health consequences for the recipients during seasonal or interannual periods when other food resources are in short supply; (2) even when sharing is quantitatively and nutritionally equitable, food taboos may block certain individuals from access to meat and/or fat, particularly children, women at critical stages in their reproductive life, and the elderly (however, in the case of pregnant women, such food taboos and seemingly inequitable sharing practices may have positive as well as negative consequences for the health and survivorship of the fetus or newborn infant by keeping maternal protein consumption below about 20% of total calories and by reducing the mother's risk of exposure to potentially teratogenic substances that may accumulate in animal tissues); (3) skilled hunters may acquire nutritionally more valuable parts than do other males by "snacking" at kill sites and through differential sharing; (4) food-sharing practices and food taboos vary widely among foragers, and this diversity may contribute to observed differences among groups in fertility and infant mortality patterns; and (5) the focus of anthropologists on the sharing of food, especially meat, as opposed to the sharing of a broad spectrum of social, political, economic, and sexual rights and privileges, is an overly narrow and potentially misleading perspective. In closing, the paper briefly discusses the utility of the term "egalitarian," concluding that the concept, by conflating ideology with actual behavior, may obscure rather than enhance our understanding of the origins and adaptations of foraging societies.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/28530/1/0000327.pd

Impact of mandatory testing system and school context factors on teachers’ acceptance and usage of school performance feedback data

In Deutschland wurden standardbasierte Vergleichsarbeiten eingeführt, um die Qualität im öffentlichen Schulsystem zu sichern und zu verbessern. Die Wirkung dieser Tests hängt jedoch wesentlich von der schulinternen Rezeption und Nutzung der Leistungsrückmeldungen durch Lehrkräfte ab. Ein theoretisches Rahmenmodell legt nahe, dass sowohl Merkmale des Testsystems als auch der Implementation dabei eine Rolle spielen und institutionelle Kontextfaktoren auf Ebene des Schulsystems und Ebene der Schule den Effekt moderieren. In einer quantitativen Vorstudie zu Vergleichsarbeiten in Baden-Württemberg und Thüringen konnte gezeigt werden, dass sich Unterschiede im Testsystem und der Testimplementation auf die Akzeptanz und Nutzung der Testrückmeldungen durch Lehrkräfte auswirken können. Das Ziel dieser Studie war eine weiterführende, vertiefte Analyse der Befunde auf der Basis einer wesentlich größeren Lehrerstichprobe (n = 1777) und unter Kontrolle weiterer Kontextfaktoren auf Ebene der Einzelschule (Lehrerkooperation, Diskussion von Testrückmeldungen in Gremien). In multiplen Regressionsanalysen und einer multivariaten Varianzanalyse erwiesen sich die theoriekonformen Differenzen in der Wahrnehmung der curricularen Validität, der diagnostischen Hinweise und der Einschätzung negativer Folgen von Vergleichsarbeiten zwischen den beiden Bundesländern als stabil. Es wird diskutiert, in welchem Maße die Unterschiede im Testsystem und andere institutionelle Kontextfaktoren für diesen Befund verantwortlich gemacht werden können. (DIPF/Orig.The states of the Federal Republic of Germany implemented educational standards and mandatory testing for controlling and improving the quality of public schooling. The success of this reform highly depends on teachers’ acceptance and sensemaking of external feedback data. A theoretical model suggests that features of the testing system and institutional context factors on the system level and the school level moderate teachers’ interpretation and usage of school performance feedback. A preliminary cross-country teacher survey revealed differences in teacher acceptance of external testing feedback between two German states with different approaches to mandatory testing. This paper aimed at testing if the country difference remains stable when analyzing a larger sample size (n = 1,777) and controlling for more contextual factors on the school level (test subjects, tracking, teacher cooperation, discussion of performance feedback in faculty meetings). Multiple regression analyses and a multivariate analysis of variance and covariance approved the difference in teachers’ perspective on curricular validity, diagnostic usage and negative consequences of mandatory testing between the two German states. The paper eventually discusses to what extent the states’ testing system and other institutional context variables account for the differences in teachers’ view on performance feedback data. (DIPF/Orig.

Ein allgemeindidaktisches Kategoriensystem zur Analyse des kognitiven Potenzials von Aufgaben

Die Diskussion über Aufgabenkultur ist ein wichtiges Element der aktuellen Schul- und Unterrichtsreform. Vor allem in den Fachdidaktiken wurden in den letzten Jahren differenzierte Klassifikationssysteme zur Analyse von Lern- und Testaufgaben entwickelt. Im Rahmen der Lehrerbildung und der Unterrichtsentwicklung sind jedoch fächerübergreifende Kriterien für die Beurteilung von Aufgaben von Bedeutung. Ziel dieses Beitrags ist es deshalb, ein allgemeindidaktisches Kategoriensystem für die Analyse des kognitiven Potenzials von Aufgaben vorzuschlagen. Unter Rückgriff auf allgemeindidaktische Lernzieltaxonomien und Befunde der Kognitionspsychologie wurde ein einfaches Analyseraster entwickelt.Recent debates on powerful teaching practices pinpoint the importance of cognitively demanding, higher-order student assignments. Educational researchers have developed complex, domain-specific systems for categorizing the cognitive demand of large scale test items. However, most of these extensive, domain-specific taxonomies are scientific tools but they are not very useful for teachers\u27 daily job and teacher education. We therefore developed a clear and modest taxonomy for analyzing the cognitive level of student assignments or assessment tasks across all subjects. Our work is based on Bloom\u27s revised taxonomy of educational objectives and some core features of powerful teaching practices

Das kognitive Anforderungsniveau von Aufgaben analysieren und modifizieren können: Eine wichtige Fähigkeit von Lehrkräften bei der Planung eines kompetenzorientierten Unterrichts

Das Ziel kompetenzorientierter Bildungspläne ist die Fokussierung von Unterricht auf den Wissenserwerb der einzelnen Schülerinnen und Schüler. Hierfür müssen Lehrkräfte eine klare Vorstellung von dem zu erwerbenden Wissen, dem Weg dorthin und den Möglichkeiten der Überprüfung der Zielerreichung haben. Aufgaben spielen in diesem Zusammenhang eine zentrale Rolle. Um entscheiden zu können, ob eine Aufgabe den Erwerb einer bestimmten Kompetenz fördern kann oder sich für die Diagnostik einer bestimmten Kompetenz eignet, sollten Lehrkräfte genau analysieren können, welches Wissen auf welchem Niveau für die Bearbeitung zu aktivieren ist. In diesem Beitrag wird anhand von Beispielen ein überfachliches Kategoriensystem für die Analyse des kognitiven Anforderungsniveaus von Lern- und Diagnoseaufgaben vorgestellt und dessen Bedeutung für die Diagnose und Förderung von Kompetenzen diskutiert.Competency-oriented curricula aim at focusing classroom instruction on knowledge acquisition of individual students. For teachers it is thus necessary to understand learning trajectories as well as methods of supporting students, and to be able to measure learning progress in their respective domain of teaching. Instructional assignments play an important role in planning competency-oriented teaching. Teachers need to have a clear understanding of the cognitive demands of instructional and diagnostic assignments. What kind of knowledge must be applied by students to solve a problem? How can you modify instructional assignments for students on a lower level of the knowledge acquisition trajectory? This paper presents seven categories for analyzing and modifying the cognitive complexity of instructional assignments. The categories are applied to examples in the subjects mathematics and German. Furthermore, this paper discusses the relevance of the categories to competency-oriented teaching