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Radiotherapy is an important modality of cancer therapy in childhood. To ensure reliable positioning during the course of therapy, in very young patients sedation is often required. The objective of this study was to evaluate safety and feasibility of repeated sedation during PBT at our institution treating a high number of very young children. We retrospectively analyzed sedation-related data of children receiving PBT at our institution. All patients were enrolled in the prospective registry study KiProReg (DRKS00005363). We investigated the association between sedation-related complications and possible risk factors as head mask, positioning, preexisting dysphagia, concurrent chemotherapy, and age. Complications were categorized as respiratory, cardiovascular, and other. Pearson's chi-squared test or Fisher's exact test was used to quantify associations. Between 2013 and 2018, in total 10,187 sedations in 340 children (152 male, 188 female) were performed. The median age of patients was 3.38 years (range 0.7-16.4 years). For sedation, either propofol only (n=9597) or propofol plus adjuncts (n=590) were applied. Complications occurred in 120 sedations (1.2%) and concerned 66 patients with respiratory (n=105), cardiovascular (n=3), and other (n=12) events. Four patients required hospitalization for respiratory complications (n=2) or complications due to tumor progression (n=2). Head mask (p=0.0001) and preexisting dysphagia were significantly associated with the risk of complication (p=<0.0001). Prone position was not showing any impact on complications. Our study proved safe application of repeated sedation in young patients during radiotherapy with PBT. Evaluating a high number of procedures the complication rate was low. Still, to ensure safety, a multidisciplinary, experienced team is mandatory. [ABSTRACT FROM AUTHOR]

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.