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

Strong Activity Changes Observable during the First Pretreatment Cycles of Trimetallic PtNiMo/C Catalysts

Pt‐based alloy catalysts supported on carbon are commonly characterized for oxygen reduction reaction (ORR) activity using the rotating disk electrode technique (RDE). Within this study, we show exemplarily for PtNiMo/C catalysts that the applied pretreatment influences strongly the determined activity. The classically employed descriptor of unchanged cyclic voltammetry response is insufficient to portrait completed surface restructuring, and gives an incorrect impression that stable activity can be determined. This might be one of the reasons for the strongly deviating activities reported in literature. Following the changes in activity during pretreatment also with in‐situ FTIR and online dissolution measurements gives insights to an up to now largely overseen high activity of the trimetallic catalysts. A maximum activity of 0.57 mA cmPt⁻² at 0.95 VRHE is reached quickly during the first six cycles and decreases slowly subsequently. The maximum activity and change of activity over the cycle number is affected by the scan rate and electrolyte refreshing, while the gas atmosphere plays only a minor role. This exemplary study might be important for Pt alloy catalysts in general