Complicazioni Scheletriche Dopo Trapianto Renale

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THU0601 REFRACTORY ACUTE CORONARY SYNDROME AND CARDIOGENIC SHOCK AS ATYPICAL ONSET OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

Background:Eosinophilic Granulomatosis with Polyangiitis (EGPA) is an ANCA-associated vasculitis, characterized by eosinophilic infiltration in tissues, intravascular and extravascular granuloma formation. It is a rare disease, affecting between 0 and 4 per million population per year. The cardiac involvement occurs in 15-60% of EGPA patients (generally ANCA-negative); it is often insidious, underestimated and it has a poor prognosis. The disease usually shows a prodromal phase characterized by asthma and allergic manifestations.Objectives:We report the case of a young patient with acute coronary syndrome (ACS) complicated by cardiogenic shock as the first manifestation of EGPA.Methods:A 36 year old Indian male patient, with a previous history of asthma, rhinitis, Raynaud syndrome and allergy to ketoprofen, presented to the emergency department with a complaint of chest pain and dyspnea. Cardiac troponin was elevated and he was admitted to intensive care unit with a diagnosis of ACS. The patient conditions rapidly deteriorated due to acute cardiogenic shock and an urgent coronary angiogram was performed. An occlusion of the left main coronary artery was treated with angioplasty and two drug-eluting stents. Echocardiography showed severe left ventricular dysfunction requiring inotropic and intra-aortic balloon pump support. A mild dermatitis after salicylic acid administration resolved with intravenous hydrocortisone 1 g. The thrombophilia screening was negative, as well as cardiovascular risk factors. Over the next days, the clinical conditions rapidly improved with recovery of normal ventricular function on discharge. However, two weeks later he was readmitted with recurrent mandibular and chest pain. Troponin levels were elevated and fluctuated, suggesting recurrent ischemic events. Repeated ECG during angina crisis showed ischemic alterations in different coronary territories. The coronary angiogram detected coronary vasospasm of the circumflex artery, reversible after nitroglycerin (Figure 1). Nitroglycerin and calcium channel blockers were initiated, but did not resolve the vasospastic angina crisis, occurring daily. Laboratory tests revealed eosinophilia (4390 cells/mcl), increased C reactive protein (9.4 mg/l) and positive antinuclear antibodies (1:320). The other serological and immunological tests were negative, including MPO-ANCA and PR3-ANCA. An abdomen and chest CT scan was negative.Figure 1.Coronary angiogram showing coronary vasospasm in the left circumflex artery (panel A, yellow arrow), reversible after nytroglicerin infusion (panel B, yellow arrow)Results:The eosinophilia and the history of asthma rose the suspect of EGPA vasculitis. The patient was treated with intravenous methylprednisolone 250 mg once daily for 3 days, followed by oral prednisone 1 mg/kg/day, with rapid and complete resolution of the recurrent angina episodes. Intravenous cyclophosphamide 10 mg/kg was administered every 2 weeks for 2 times, then 12 mg/kg every 4 weeks. Oral corticosteroid was tapered, with the persistence of a complete remission of the symptoms, after 2 months of immunosuppressive therapy.Conclusion:Coronary involvement in EGPA can mimic atherosclerotic artery disease and can be life threatening, if not promptly recognized. An accurate medical history and complete serological and immunological tests are crucial to detect an atypical onset of EGPA, prompting early immunosuppressive therapy which is pivotal for the patient survival.References:[1]MR Hazebroek et al. Prevalence and Prognostic Relevance of Cardiac Involvement in ANCA-associated Vasculitis: Eosinophilic Granulomatosis With Polyangiitis and Granulomatosis With Polyangiitis. Int J Cardiol 2015, 199: 170-9.Disclosure of Interests:None declaredDOI: 10.1136/annrheumdis-2020-eular.648

Chemotherapy rechallenge after regorafenib treatment in metastatic colorectal cancer. Still hope after the last hope?

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The measurement of the noise-equivalent spectral radiance of SIMBIO-SYS/VIHI spectrometer

We report about the measurement of the Noise- Equivalent Spectral Radiance (NESR) of the VIHI imaging spectromter aboard ESA's Bepi Colombo mission to Mercury. The knowledge of the NESR allows to determine the capability of an optical detector to measure faint signals. A description of the setup used to determine the NESR during the prelaunch calibration campaign is given. The processing of the data col- lected at various operative temperatures and integration times is described. The sensitivity study of the NESR has been performed at the expected detector's temperatures and integration times with the goal to determine the minimum spectral radiance at which VIHI is sensitive during the different observation phases of the mission. A simulation of the expected Signal-to-Noise Ratio (SNR) of VIHI during the different orbital phases is provided

Integrated Genomic, Functional, and Prognostic Characterization of Atypical Chronic Myeloid Leukemia

Atypical chronic myeloid leukemia (aCML) is a BCR-ABL1-negative clonal disorder, which belongs to the myelodysplastic/myeloproliferative group. This disease is characterized by recurrent somatic mutations in SETBP1, ASXL1 and ETNK1 genes, as well as high genetic heterogeneity, thus posing a great therapeutic challenge. To provide a comprehensive genomic characterization of aCML we applied a high-throughput sequencing strategy to 43 aCML samples, including both whole-exome and RNA-sequencing data. Our dataset identifies ASXL1, SETBP1, and ETNK1 as the most frequently mutated genes with a total of 43.2%, 29.7 and 16.2%, respectively. We characterized the clonal architecture of 7 aCML patients by means of colony assays and targeted resequencing. The results indicate that ETNK1 variants occur early in the clonal evolution history of aCML, while SETBP1 mutations often represent a late event. The presence of actionable mutations conferred both ex vivo and in vivo sensitivity to specific inhibitors with evidence of strong in vitro synergism in case of multiple targeting. In one patient, a clinical response was obtained. Stratification based on RNA-sequencing identified two different populations in terms of overall survival, and differential gene expression analysis identified 38 significantly overexpressed genes in the worse outcome group. Three genes correctly classified patients for overall survival

Children’s rights and digital technologies

Digital technologies have reshaped children’s lives, resulting in new opportunities for and risks to their well-being and rights. This chapter investigates the impact of digital technologies on children’s rights through the lens of the United Nations Convention on the Rights of the Child. Up until now, not all rights have received the same level of attention in the digital context. Legal and policy discourse in the area of children and digital media predominantly focuses on ‘protection’ rights, albeit with a growing awareness of the tension between ‘protection’ and ‘participation’ rights. ‘Provision’ rights are not often emphasised, other than in the important domain of education. However, all children’s rights should be supported, valued and developed in both online and offline spheres of engagement. Governments, parents, educators, industry, civil society and children’s rights commissioners or ombudspersons should all take up their responsibility to enhance children’s rights in relation to digital technologies, while actively listening and taking account of children’s views when developing laws, policies, programmes and other measures in this field

Radiometric calibration of the SIMBIO-SYS STereo imaging Channel

The STereo imaging Channel (STC) is a double wide-angle camera developed to be one of the channels of the SIMBIOSYS instrument onboard of the ESA BepiColombo mission to Mercury. STC main goal is to map in 3D the whole Mercury surface. The geometric and radiometric responses of the STC Proto Flight model have been characterized on-ground during the calibration campaign. The derived responses will be used to calibrate the STC images that will be acquired in flight. The aim is to determine the functions linking the detected signal in digital number to the radiance of the target surface in physical units. The result of the radiometric calibration consists in the determination of well-defined quantities: (1) the dark current as a function of the integration time and of the detector temperature, settled and controlled to be stable at 268 K; (2) the read out noise, which is associated with the noise signal of the read-out electronic; and (3) the fixed pattern noise, which is generated by the different response of each pixel. Once these quantities are known, the photon response and the photoresponse non-uniformity, which represents the variation of the photon responsivity of a pixel in an array, can be derived. The final result of the radiometric calibration is the relation between the radiance of an accurately known and uniform source, and the digital numbers measured by the detector

SIMBIO-SYS : Scientific Cameras and Spectrometer for the BepiColombo Mission

The SIMBIO-SYS (Spectrometer and Imaging for MPO BepiColombo Integrated Observatory SYStem) is a complex instrument suite part of the scientific payload of the Mercury Planetary Orbiter for the BepiColombo mission, the last of the cornerstone missions of the European Space Agency (ESA) Horizon + science program. The SIMBIO-SYS instrument will provide all the science imaging capability of the BepiColombo MPO spacecraft. It consists of three channels: the STereo imaging Channel (STC), with a broad spectral band in the 400-950 nm range and medium spatial resolution (at best 58 m/px), that will provide Digital Terrain Model of the entire surface of the planet with an accuracy better than 80 m; the High Resolution Imaging Channel (HRIC), with broad spectral bands in the 400-900 nm range and high spatial resolution (at best 6 m/px), that will provide high-resolution images of about 20% of the surface, and the Visible and near-Infrared Hyperspectral Imaging channel (VIHI), with high spectral resolution (6 nm at finest) in the 400-2000 nm range and spatial resolution reaching 120 m/px, it will provide global coverage at 480 m/px with the spectral information, assuming the first orbit around Mercury with periherm at 480 km from the surface. SIMBIO-SYS will provide high-resolution images, the Digital Terrain Model of the entire surface, and the surface composition using a wide spectral range, as for instance detecting sulphides or material derived by sulphur and carbon oxidation, at resolutions and coverage higher than the MESSENGER mission with a full co-alignment of the three channels. All the data that will be acquired will allow to cover a wide range of scientific objectives, from the surface processes and cartography up to the internal structure, contributing to the libration experiment, and the surface-exosphere interaction. The global 3D and spectral mapping will allow to study the morphology and the composition of any surface feature. In this work, we describe the on-ground calibrations and the results obtained, providing an important overview of the instrument performances. The calibrations have been performed at channel and at system levels, utilizing specific setup in most of the cases realized for SIMBIO-SYS. In the case of the stereo camera (STC), it has been necessary to have a validation of the new stereo concept adopted, based on the push-frame. This work describes also the results of the Near-Earth Commissioning Phase performed few weeks after the Launch (20 October 2018). According to the calibration results and the first commissioning the three channels are working very well.Peer reviewe