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Ausgangspunkt dieses Beitrags ist die Beobachtung (cf. Seiler 1984), daß komplexe Sätze, insbesondere Komplementsätze, einerseits die Dimension PARTIZIPATION 'abschließen', andererseits aber auch über sie hinausgehen. Hier treffen offensichtlich zwei Dimensionen (im Sinne von UNITYP) aufeinander: die Dimension der PARTIZIPATION einerseits und die Dimension der NEKTION andererseits, deren generelle Funktion etwa bestimmt werden könnte als die sprachliche Darstellung von Relationen zwischen Sachverhalten bzw. Propositionen (bzw. den entsprechenden Konzeptualisierungen der Relationen zwischen Sachverhalten). Die 'Nahtstelle' zwischen den beiden Dimensionen soll hier anhand von einigen Beispielen aus dem Baskischen diskutiert werden.
The approach outlined in the present paper is based on observations made with African languages. Although the 1000-odd African languages display a remarkable extent of structural variation, there are certain structures that do not seem to occur in Africa. Thus, to our knowledge, an African language having anything that could be called an ergative case or a numeral classifier system has not been discovered so far. It may turn out that our approach can, in a modified form, be made applicable to languages outside Africa. This , however, is a possibility that has not been considered here. The present approach is based essentially on diachronic findings in that it uses observations on language evolution in order to account for structural differences between languages. Thus, it has double potential: apart from describing and explaining typological diversity it can also be material to reconstructing language history.
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.