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This ICB Research Report constitutes the proceedings of the following four workshops which were held on Tuesday, 29th June 2010 as part of the Requirements Engineering: Foundation for Software Quality (REFSQ) conference 2010 at the University of Duisburg-Essen. First Workshop on Creativity in Requirements Engineering (CreaRE). First International Workshop on Product Line Requirements Engineering and Quality (PLREQ). First Workshop on Requirements Prioritization for customer-oriented Software-Development (RePriCo). First Workshop on Requirements Engineering in Small Companies (RESC).
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene
(2003)
2-methylacetoacetyl-coenzyme A thiolase (beta-ketothiolase) deficiency: one disease - two pathways
(2019)
Background: 2-methylacetoacetyl-coenzyme A thiolase deficiency (MATD; deficiency of mitochondrial acetoacetyl-coenzyme A thiolase T2/ “beta-ketothiolase”) is an autosomal recessive disorder of ketone body utilization and isoleucine degradation due to mutations in ACAT1.
Methods: We performed a systematic literature search for all available clinical descriptions of patients with MATD. 244 patients were identified and included in this analysis. Clinical course and biochemical data are presented and discussed.
Results: For 89.6 % of patients at least one acute metabolic decompensation was reported. Age at first symptoms ranged from 2 days to 8 years (median 12 months). More than 82% of patients presented in the first two years of life, while manifestation in the neonatal period was the exception (3.4%). 77.0% (157 of 204 patients) of patients showed normal psychomotor development without neurologic abnormalities.
Conclusion: This comprehensive data analysis provides a systematic overview on all cases with MATD identified in the literature. It demonstrates that MATD is a rather benign disorder with often favourable outcome, when compared with many other organic acidurias.
20 Jahre Hochschul- und Kreisbibliothek Bonn-Rhein-Sieg: Eine ungewöhnliche Erfolgsgeschichte
(2018)
Als am 28. September 1998 Oberkreisdirektor Frithjof Kühn (der spätere Landrat) und Kreisdirektorin Monika Lohr für den Rhein-Sieg-Kreis sowie Gründungsrektor Prof. Dr. Hubert Severin und Kanzler Hans Stender für die Fachhochschule Rhein-Sieg (heute Hochschule Bonn-Rhein-Sieg) einen Kooperationsvertrag unterschrieben, taten sie etwas sehr Ungewöhnliches: Die wissenschaftliche Bibliothek der Fachhochschule und die öffentliche Bibliothek des Rhein-Sieg-Kreises sollten organisatorisch und räumlich zusammengelegt werden.
Innovative Technologien, veränderte Anbieterstrukturen, stets neue Leistungsangebote und wandelnde Kundenanforderungen prägen und verändern von Beginn an das Erscheinungsbild des TK-Marktes. Unter Berücksichtigung einer Betrachtung im Zeitablauf lässt sich der Markt in drei aufeinanderfolgende Phasen einteilen: 1. Marktöffnung, 2. Konsolidierung und Optimierung, 3. Automatisierung. Die Phasen sind selbstverständlich nicht Überschneidungsfrei, geben jedoch einen Hinweis auf die strukturellen Veränderungen des Marktes.
Sharing economies enabled by technical platforms have been studied regarding their economic, legal, and social effects, as well as with regard to their possible influences on CSCW topics such as work, collaboration, and trust. While a lot current research is focusing on the sharing economy and related communities, there is little work addressing the phenomenon from a socio-technical point of view. Our workshop is meant to address this gap. Building on research themes and discussion from last year’s ECSCW, we seek to engage deeper with topics such as novel socio-technical approaches for enabling sharing communities, discussing issues around digital consumer and worker protection, as well as emerging challenges and opportunities of existing platforms and approaches.
Sie sind im Bereich Qualitätsmanagement tätig und haben die Aufgabe bekommen, ein Problem systematisch zu untersuchen und methodisch zu lösen? Sie haben zu viele Aufgaben und wissen nicht, wie Sie diese priorisieren sollen? Oder haben Sie zu begrenzte Ressourcen, um alle Reklamationen gleichzeitig bearbeiten zu können? Oder wissen nicht, wie Sie einen bestimmten Prozess in seinen Grenzen zielführend verbessern können?
Background 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMGCLD) is an autosomal recessive disorder of ketogenesis and leucine degradation due to mutations in HMGCL .
Method We performed a systematic literature search to identify all published cases. 211 patients of whom relevant clinical data were available were included in this analysis. Clinical course, biochemical findings and mutation data are highlighted and discussed. An overview on all published HMGCL variants is provided.
Results More than 95% of patients presented with acute metabolic decompensation. Most patients manifested within the first year of life, 42.4% already neonatally. Very few individuals remained asymptomatic. The neurologic long-term outcome was favorable with 62.6% of patients showing normal development.
Conclusion This comprehensive data analysis provides a systematic overview on all published cases with HMGCLD including a list of all known HMGCL mutations.