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This paper investigates the effect of voltage sensors on the measurement of transient voltages for power semiconductors in a Double Pulse Test (DPT) environment.We adapt previously published models that were developed for current sensors and apply them to voltage sensors to evaluate their suitability for DPT applications. Similarities and differences between transient current and voltage sensors are investigated and the resulting methodology is applied to commercially available and experimental voltage sensors. Finally, a selection aid for given measurement tasks is derived that focuses on the measurement of fast-switching power semiconductors.
Grailog embodies a systematics to visualize knowledge sources by graphical elements. Its main benefit is that the resulting visual presentations are easier to read for humans than the original symbolic source code. In this paper we introduce a methodology to handle the mapping from Datalog RuleML, serialized in XML, to an SVG representation of Grailog, also serialized in XML, via eXtensible Stylesheet Language Transformations (XSLT) 2.0/XML; the SVG is then rendered visually by modern Web browsers. This initial mapping is realized to target Grailog's "fully node copied" normal form. Elements can thus be translated one at a time, separating the fundamental Datalog-to-SVG translation concern from the concern of merging node copies for optimal (hyper)graph layout and avoiding its high computational complexity in this online tool. The resulting open source Grailog Knowledge-Source Visualizer (Grailog KS Viz) supports Datalog RuleML with positional relations of arity n>1. The on-the-fly transformation was shown to run on all recent major Web browsers and should be easy to understand, use, and extend.
BACKGROUND
Propionic acidemia is an inherited disorder caused by deficiency of propionyl-CoA carboxylase. Although it is one of the most frequent organic acidurias, information on the outcome of affected individuals is still limited.
STUDY DESIGN/METHODS
Clinical and outcome data of 55 patients with propionic acidemia from 16 European metabolic centers were evaluated retrospectively. 35 patients were diagnosed by selective metabolic screening while 20 patients were identified by newborn screening. Endocrine parameters and bone age were evaluated. In addition, IQ testing was performed and the patients' and their families' quality of life was assessed.
RESULTS
The vast majority of patients (>85%) presented with metabolic decompensation in the neonatal period. Asymptomatic individuals were the exception. About three quarters of the study population was mentally retarded, median IQ was 55. Apart from neurologic symptoms, complications comprised hematologic abnormalities, cardiac diseases, feeding problems and impaired growth. Most patients considered their quality of life high. However, according to the parents' point of view psychic problems were four times more common in propionic acidemia patients than in healthy controls.
CONCLUSION
Our data show that the outcome of propionic acidemia is still unfavourable, in spite of improved clinical management. Many patients develop long-term complications affecting different organ systems. Impairment of neurocognitive development is of special concern. Nevertheless, self-assessment of quality of life of the patients and their parents yielded rather positive results.
