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

On the luminescence signals of empty sample carriers

International audienceLuminescence dating is a leading technique for a large spectrum of Quaternary dating applications. Since the development of automated reader systems, handling great amounts of samples has become possible. A large quantity of data is produced in a short time and a detailed check of every single curve is often impractical. Therefore, it is important to be confident in excluding any kind of unwanted signal contributions, such as those from sample carriers. For commonly used types of steel and aluminium (Al) carriers from three laboratories, luminescence characteristics of spurious and radiation-induced signals are presented. TL and OSL emissions of discs show natural (Al) and regenerated thermally stable signals in the UV, UV-blue and red detection range. These signals have characteristic saturation doses of several hundred Gy. Furthermore, we demonstrate light insensitive signal components and phototransferred thermoluminescence (PTTL). Due to high scatter between discs, the proportion of unwanted disc signal contribution to the entire signal is difficult to predict, without direct measurement. The sources of these signals are possibly chemical compounds acting as luminophores or oxide layers (Al2O3 layers in case of Al discs)

Stepwise, earthquake-driven coastal subsidence in the Ganges-Brahmaputra Delta (Sundarbans) since the eighth century deduced from submerged in situ kiln and mangrove remnants

This study reconstructs the coastal subsidence over the past 1300 years in a mangrove region along the coast of the Ganges-Brahmaputra Delta, an area not affected by anthropogenic interference. The relative sea level (RSL) history is based on radiocarbon and luminescence ages measured on 108 submerged kilns and in situ mangrove stumps. While the regional, long-term average subsidence rate is calculated to be 2.7 +/- 0.3 mm/yr, modern RSL (including eustacy, isostasy, ocean level, and subsidence) rises by 8.7 +/- 0.4 mm/yr. This rate has been balanced by natural sediment accretion so far. A reduction in sediment supply by engineering projects along rivers and coasts may, however, accelerate coastal inundation and retreat. Subsidence has accelerated during specific episodes since the eighth century. A major land-sinking event happened in the eighteenth century, with a downward displacement of 1-2 m, depending on geographic area. We propose that the subduction-related 1762 Arakan earthquake caused this sudden lowering. Prior to this event, RSL was nearly stable for 900 years. An earlier major subsidence event occurred around 900 CE, when the land suddenly sank by about 1 m, which also coincided with a major earthquake along the Arakan coast. Event-driven, sudden, significant subsidence, thus, needs to be considered a potential major hazard for coastal Bangladesh

Single-grain feldspar luminescence chronology of historical extreme wave event deposits recorded in a coastal lowland, Pacific coast of central Japan

The Shirasuka lowlands, located on the Enshu-nada coast of central Japan, record evidence for numerous extreme wave events. Here we test the applicability of using the luminescence signal from feldspars to date these young (<1000 a) extreme wave event deposits. The signal used for dating is the IRSL signal (measured at 50 °C) as part of a post-IR IRSL130 procedure. We demonstrate that this IRSL50 (pre-IR130) signal results in reliable ages when corrected for fading, and the post-IR IRSL130 stimulation functions as an optical wash for both the natural/regenerated luminescence signal (Lx) and the test dose signal (Tx), lowering the recuperation and removing any remaining charge from previous steps in the protocol. The single grain IRSL50 (pre-IR130) ages generated, cover the historical record of the past 800 years and correlate well with past earthquakes and tsunamis in AD 1361, AD 1498 and AD 1605. Another identified tsunami deposit may correlate with the AD 1707 earthquake and tsunami. A slope failure deposit, probably caused by the earthquake in AD 1944, is also identified. This study demonstrates that accurate ages can be determined for the young, extreme wave events at this site using the luminescence signal from feldspars