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The comprehension and production of single words involve a variety of processing stages. Which stages need to be accessed differs depending on whether objects (pictures in an experimental environment) or words are supposed to be named. Naming tasks are often employed in psycholinguistic studies in order to provide an insight into the function of mental processes during word production. Differences in naming latencies and naming accuracy between words suggest that the retrieval of some lexical items is easier or more difficult in contrast to others. The relative ease of word retrieval has been found to be strongly influenced by properties of these words, such as familiarity and written or spoken frequency.
Exploring which variables affect naming speed and accuracy will allow gaining more information about the storage and processing of words in general. If a variable has a discernable effect on a specific experimental task, the localization of this effect is of interest for psycholinguistic research. This is because finding the locus of the effect can help specify models of speech production with respect to what processes occur at which stage of lexical retrieval. Additionally, identifying which variables influence language processing is inevitable in order to control for these variables when necessary. Otherwise variance in naming latencies could not be explained by the variable that was to be tested because other, uncontrolled variables could have altered the results.
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.