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

Frontiers in Physiology / Comparison of Bleaching Products With Up to 6% and With More Than 6% Hydrogen Peroxide: Whitening Efficacy Using BI and WID and Side Effects An in vitro Study

The aim of this study was to evaluate the effect of bleaching agents containing different concentrations of hydrogen peroxide (HP) on color-change and on enamel-surface in bovine teeth. Furthermore the influence on cell viability and proliferation was investigated. Two hundred and forty teeth were randomly assigned into four groups (home bleaching 6%, in-office bleaching 6%, in-office bleaching > 6% HP, and control group). Bleaching was performed after artificial staining and the bleached index (BI) as well as the whiteness index (WID) was measured at several time points. Chemical analysis for HP concentrations and the pH of the bleaching products was done. Furthermore, enamel surfaces of randomly selected specimens were analyzed using scanning electron microscopy (SEM) and cytotoxicity of the tested bleaching products was evaluated in vitro using dental pulp cells (DPCs) and L929 cells. A statistically significant whitening effect was observed in almost all products. As expected all investigated products resulted in decreased cell viability, however, with different values of LC50 (median lethal concentration). SEM analysis showed an analog of enamel alterations with decreasing pH, increasing exposure time, and increasing HP concentration. Bleaching agents containing a low HP concentration are considered to be effective and to have less damaging effects on enamel and tested cells.(VLID)490832