Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany

Background: Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany. Methods: We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany. Subsequently, via online surveys and teleconferences among pediatric rheumatologists with a special expertise in the treatment of SJIA, we identified current diagnostic and treatment approaches in Germany. Those were harmonized via the formulation of statements and, supported by findings from a literature search. Finally, an in-person consensus conference using nominal group technique was held to further modify and consent the statements. Results: Up to 50% of patients diagnosed with SJIA in Germany do not fulfill the International League of Associations for Rheumatology (ILAR) classification criteria, mostly due to the absence of chronic arthritis. Our findings suggest that chronic arthritis is not obligatory for the diagnosis and treatment of SJIA, allowing a diagnosis of probable SJIA Malignant, infectious and hereditary autoinflammatory diseases should be considered before rendering a diagnosis of probable SJIA There is substantial variability in the initial treatment of SJIA. Based on registry data, most patients initially receive systemic glucocorticoids, however, increasingly substituted or accompanied by biological agents, i.e. interleukin (IL)-1 and IL-6 blockade (up to 27.2% of patients). We identified preferred initial therapies for probable and definitive SJIA, including step-up patterns and treatment targets for the short-term (resolution of fever, decrease in C-reactive protein by 50% within 7 days), the mid-term (improvement in physician global and active joint count by at least 50% or a JADAS-10 score of maximally 5.4 within 4 weeks) and the long-term (glucocorticoid-free clinically inactive disease within 6 to 12 months), and an explicit treat-to-target strategy. Conclusions: We developed consensus-based strategies regarding the diagnosis and treatment of probable or definitive SJIA in Germany

Cushing-Syndrom mit konsekutiver tertiärer Nebennierenrindeninsuffizienz nach simultaner multipler intraartikulärer Lokaltherapie mit Glukokortikoiden

After simultaneous multiple local treatment with glucocorticoids at 46 sites a 4‑year-old female patient with newly diagnosed polyarticular juvenile idiopathic arthritis (JIA) initially developed Cushing's syndrome followed by a gradual worsening of her condition and finally an acute high fever urinary tract infection. Iatrogenic adrenocortical insufficiency after multiple intra-articular glucocorticoid administration was diagnosed. The possibility of severe systemic glucocorticoid side effects after extensive local treatment should be included in the regular management of JIA patients

Design and first cold test of BNL superconducting 112 MHz QWR for electron gun applications

Brookhaven National Laboratory and Niowave, Inc. have designed, fabricated, and performed the first cold test of a superconducting 112 MHz quarter-wave resonator (QWR) for electron gun experiments. The first cold test of the QWR cryomodule has been completed at Niowave. The paper discusses the cryomodule design, presents the cold test results, and outline plans to upgrade the cryomodule for future experiments. A quarter-wave resonator concept of superconducting RF (SRF) electron gun was proposed at BNL for electron cooling ion/proton beams at RHIC. QWRs can be made sufficiently compact even at low RF frequencies (long wavelengths). The long wavelength allows to produce long electron bunches, thus minimizing space charge effects and enabling high bunch charge. Also, such guns should be suitable for experiments requiring high average current electron beams. A 112 MHz QWR gun was designed, fabricated, and cold-tested in collaboration between BNL and Niowave. This is the lowest frequency SRF gun ever tested successfully. In this paper we describe the gun design and fabrication, present the cold test results, and outline plans for the cryomodule upgrade for future experiments

Superconducting 112 MHz QWR electron gun DISCLAIMER SUPERCONDUCTING 112 MHZ QWR ELECTRON GUN*

Abstract Brookhaven National Laboratory and Niowave, Inc. have designed and fabricated a superconducting 112 MHz quarter-wave resonator (QWR) electron gun. The first cold test of the QWR cryomodule has been completed at Niowave. The paper describes the cryomodule design, presents the cold test results, and outline plans to upgrade the cryomodule. Future experiments include studies of different photocathodes and use for the coherent electron cooling proof-of-principle experiment. Two cathode stalk options, one for multi-alkali photocathodes and the other one for a diamond-amplified photocathode, are discussed

Superconducting 112 MHz QWR electron gun

Brookhaven National Laboratory and Niowave, Inc. have designed and fabricated a superconducting 112 MHz quarter-wave resonator (QWR) electron gun. The first cold test of the QWR cryomodule has been completed at Niowave. The paper describes the cryomodule design, presents the cold test results, and outline plans to upgrade the cryomodule. Future experiments include studies of different photocathodes and use for the coherent electron cooling proof-of-principle experiment. Two cathode stalk options, one for multi-alkali photocathodes and the other one for a diamond-amplified photocathode, are discussed. A quarter-wave resonator concept of superconducting RF (SRF) electron gun was proposed at BNL for electron cooling hadron beams in RHIC. QWRs can be made sufficiently compact even at low RF frequencies (long wavelengths). The long wavelength allows to produce long electron bunches, thus minimizing space charge effects and enabling high bunch charge. Also, such guns should be suitable for experiments requiring high average current electron beams. A 112 MHz QWR gun was designed, fabricated, and cold-tested in collaboration between BNL and Niowave. This is the lowest frequency SRF gun ever tested successfully. In this paper we describe the gun design and fabrication, present the cold test results, and outline our plans. This gun will also serve as a prototype for a future SRF gun to be used for coherent electron cooling of hadrons in eRHIC

Visually-guided Attention Enhances Target Identification in a Complex Auditory Scene

In auditory scenes containing many similar sound sources, sorting of acoustic information into streams becomes difficult, which can lead to disruptions in the identification of behaviorally relevant targets. This study investigated the benefit of providing simple visual cues for when and/or where a target would occur in a complex acoustic mixture. Importantly, the visual cues provided no information about the target content. In separate experiments, human subjects either identified learned birdsongs in the presence of a chorus of unlearned songs or recalled strings of spoken digits in the presence of speech maskers. A visual cue indicating which loudspeaker (from an array of five) would contain the target improved accuracy for both kinds of stimuli. A cue indicating which time segment (out of a possible five) would contain the target also improved accuracy, but much more for birdsong than for speech. These results suggest that in real world situations, information about where a target of interest is located can enhance its identification, while information about when to listen can also be helpful when targets are unfamiliar or extremely similar to their competitors