ARC-TEC : acquisition, representation and compilation of technical knowledge

A global description of an expert system shell for the domain of mechanical engineering is presented. The ARC-TEC project constitutes an AI approach to realize the CIM idea. Along with conceptual solutions, it provides a continuous sequence of software tools for the acquisition, representation and compilation of technical knowledge. The shell combines the KADS knowledge-acquisition methodology, the KL-ONE representation theory and the WAM compilation technology. For its evaluation a prototypical expert system for production planning is developed. A central part of the system is a knowledge base formalizing the relevant aspects of common sense in mechanical engineering. Thus, ARC-TEC is less general than the CYC project but broader than specific expert systems for planning or diagnosis

Gesellschaftlicher Aufbruch, reale Utopien und die Arbeit am Sozialen

People around the world recollect socially responsible and desirable values, new forms of living and coexistence as well as eco-social economic activities. This volume is dedicated to these real utopias –positive ideas aiming at a better future and practical approaches combining notions of a more livable society with concrete practical implementations.Weltweit besinnen sich Menschen auf gesellschaftlich Verantwortbares und Wünschenswertes, auf neue Formen des Lebens und Zusammenlebens und des ökosozialen Wirtschaftens. Dieser Band widmet sich diesen realen Utopien, positiven, in eine bessere Zukunft gerichteten Vorstellungen und praktischen Ansätzen, die Ideen von einer lebenswerteren Gesellschaft mit konkreter Praxis verbinden

The German National Registry of Primary Immunodeficiencies (2012-2017)

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment