Open Access

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.

In German, the subject usually precedes the object (SO order), but, under certain discourse conditions, the object is allowed to precede the subject (OS order). This paper focuses on main clauses in which either the subject or a discourse-given object occurs in clause-initial position. Two acceptability experiments show that OS sentences with a given object are generally acceptable, but the precise degree of acceptability varies both with the object‘s referential form (demonstrative objects leading to higher acceptability than other types of objects) and with formal properties of the subject (pronominal subjects leading to higher acceptability than non-pronominal subjects). For SO sentences, acceptability was reduced when the object was a d-pronoun, which contrasts with the high acceptability of OS sentences with a d-pronoun object. This finding was explored in a third acceptability experiment comparing d-pronouns in subject and object function. This experiment provides evidence that a reduction in acceptability due to a prescriptive bias against d-pronouns is suspended when the d-pronoun occurs as object in the prefield. We discuss the experimental results with respect to theories of German clause structure that claim that OS sentences with different information-structural properties are derived by different types of movement.

This paper presents three acceptability experiments investigating German verb-final clauses in order to explore possible sources of sentence complexity during human parsing. The point of departure was De Vries et al.'s (2011) generalization that sentences with three or more crossed or nested dependencies are too complex for being processed by the human parsing mechanism without difficulties. This generalization is partially based on findings from Bach et al. (1986) concerning the acceptability of complex verb clusters in German and Dutch. The first experiment tests this generalization by comparing two sentence types: (i) sentences with three nested dependencies within a single clause that contains three verbs in a complex verb cluster; (ii) sentences with four nested dependencies distributed across two embedded clauses, one center-embedded within the other, each containing a two-verb cluster. The results show that sentences with four nested dependencies are judged as acceptable as control sentences with only two nested dependencies, whereas sentences with three nested dependencies are judged as only marginally acceptable. This argues against De Vries et al.'s (2011) claim that the human parser can process no more than two nested dependencies. The results are used to refine the Verb-Cluster Complexity Hypothesis of Bader and Schmid (2009a). The second and the third experiment investigate sentences with four nested dependencies in more detail in order to explore alternative sources of sentence complexity: the number of predicted heads to be held in working memory (storage cost in terms of the Dependency Locality Theory [DLT], Gibson, 2000) and the length of the involved dependencies (integration cost in terms of the DLT). Experiment 2 investigates sentences for which storage cost and integration cost make conflicting predictions. The results show that storage cost outweighs integration cost. Experiment 3 shows that increasing integration cost in sentences with two degrees of center embedding leads to decreased acceptability. Taken together, the results argue in favor of a multifactorial account of the limitations on center embedding in natural languages.

In this article we present experimental findings on the acceptability of different argument orders in the German middle field. Our study pursues two goals: First, to evaluate a number of surface constraints on German argument order that have been proposed in the literature, and second, to shed new light on how gradient constraints jointly determine sentence acceptability. In four experiments, we investigated the impact of surface constraints relating to animacy, thematic roles, definiteness and case. While we are able to confirm an influence of most constraints under investigation, the resulting constraint hierarchy does not coincide with any hierarchy put forward so far in the literature, to the best of our knowledge. With regard to gradience, our results can be accounted for either by an OT variant incorporating a notion of markedness, or by a fully quantified model using constraint weights. For the latter, however, we provide evidence against uniform penalties associated with constraint violations.