Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review

Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations

Remote Control Unit of the LHC Injector Complex Beam Loss Monitoring System

The strategy for beam set-up and machine protection of the accelerators at the European Organization for Nuclear Research (CERN) relies strongly on their Beam Loss Monitoring (BLM) systems. The BLM system of the CERN PS complex (PS, PS Booster and Linac4) was recently renovated. The upgrade aimed to extend the dynamic range in order to accept more types of detectors and to obtain a highly reconfigurable system. The new acquisition system has shown very promising results, and development is now concentrated to provide advanced remote diagnostics, set-up and monitoring features. In this context, a new remotely controlled module is under design, which will continuously survey a set of status parameters (power consumption, temperature, etc.). Additionally, by controlling an embedded current source, it will allow the analogue circuit of each channel to be compensated for component tolerances, noise and ageing

The Beam Loss Monitoring System after LHC Injectors Upgrade at CERN

The LHC Injector Upgrade project aims to increase the brightness of the beams available and improve the efficiency of the whole accelerator chain. The Beam Loss Monitoring (BLM) system is a key element of CERN’s accelerator instrumentation for beam optimisation and machine protection by producing continuous and reliable beam loss measurements while ensuring safe operation. The new BLM system for the LHC Injectors aimed to provide faster measures with a higher dynamic range, to install more detectors along the beamlines and to give the operator more flexible use. A review will be given on the versatility provided by the system to cover requirements from various accelerators and their transfer lines, focusing on the measurements and the operational scenarios

Summary of LHC MD 369: DOROS vs WBTN in IR Stripline BPMs

The aim of this MD is to quantify the impact of the stripline beam position monitor (BPM) directivity with two acquisition chain electronics systems, WBTN (Wide Band Time Normalizer) and DOROS (Diode ORbit and Oscillation System). This impact depends on the relative position and intensity of the two beams at the location of the monitor. This note explains all the procedures of the LHC MD 369, which took place on 20/07/2015 and presents the obtained results

The Diamond Beam Loss Monitoring System at CERN LHC and SPS

The Large Hadron Collider (LHC) and the Super Proton Synchrotron (SPS) accelerators are equipped with 17 pCVD diamond based Beam Loss detectors at strategical locations where their nanosecond resolution can provide insights into the loss mechanisms and complement the information of the standard ionization chamber type detectors. They are used at the injection and extraction lines of the LHC and SPS, to analyse the injection or extraction efficiency, and to verify the timing alignment of other elements like kicker magnets. They are used at the betatron collimation region and are being also explored as detectors to analyse slow extractions. The acquisition chain was fully renovated during the second LHC long shutdown period (from December 2018 to July 2022) to provide higher resolution measurements, real-time data processing and data reduction at the source as well as to integrate seamlessly to the controls infrastructure. This paper presents the new hardware platform, the different acquisition modes implemented, the system capabilities and initial results obtained during the commissioning and operation at the beginning of the LHC’s Run 3

Linearity and Response Time of the LHC Diamond Beam Loss Monitors in the CLEAR Beam Test Facility at CERN

Chemical Vapour Deposition (CVD) diamond detectors have been tested during the Run 2 operation period (2015-2018) as fast beam loss monitors for the Beam Loss Monitoring (BLM) system of the Large Hadron Collider (LHC) at CERN. However, the lack of raw data recorded during this operation period restrains our ability to perform a deep analysis of their signals. For this reason, a test campaign was carried out at the CLEAR beam test facility at CERN with the aim of studying the linearity and response time of the diamond detectors against losses from electron beams of different intensities. The signal build-up from multi-bunched electron beams was also analyzed. The conditions and procedures of the test campaign are explained, as well as the most significant results obtained

Fast Spill Monitor Studies for the SPS Fixed Target Beams

At the CERN Super Proton Synchrotron (SPS) the proton beam is supplied to the fixed target experiments in the North Area facility (NA) via a slow extraction process, taking place at 400 GeV. The monitoring of the spill quality during the extraction, lasting 4.8 seconds with the present SPS setup, is of high interest for minimising beam losses and providing the users with uniform proton-on-target rates. The monitor development challenges include the need for detecting, sampling, processing and publishing the data at rates ranging from few hundred Hz to support the present operation to several hundreds of MHz to serve future experiments proposed within the Physics Beyond Collider (PBC) programme. This paper will give an overview of the ongoing studies for optimizing the existing monitors performances and of the R&D; dedicated to future developments. Different techniques are being explored, from Secondary Emission Monitors to Optical Transition Radiation (OTR), Gas Scintillation and Cherenkov detectors. Expected ultimate limitations from the various methods will be presented, together with 2022 experimental results, for example with a recently refurbished OTR detector

La biblioteca en todos los sentidos

El trabajo obtuvo un premio de la modalidad B de los Premios Tomás García Verdejo a las buenas prácticas educativas en la Comunidad Autónoma de Extremadura para el curso académico 2012/2013Se describe el proyecto llevado a cabo en el IES Gonzalo Torrente Ballester (Miajadas, Cáceres) para dinaminzar la biblioteca del centro. Entre los objetivos del proyecto estaban: convertir a la biblioteca en un centro cultural, implicar a los alumnos en su funcionamiento, ofrecer sus servicios al resto de la localidad donde se ubica, promover la formación de usuarios y fomentar entre los alumnos las destrezas necesarias para localizar y utilizar las fuentes documentalesExtremaduraES

Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review

Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations

Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review.

Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients.Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent.Forty-one patients (26women/15 men) were recruited. They had a mean age of 34.414 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n=8), mild leukopenia (n=3), myopathy (n=1), and infections (n=5).ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations