16 research outputs found

    PReS-FINAL-2255: Primary pyomiositis in children: a challenging diagnosis

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    DTT - Divertor Tokamak Test facility: A testbed for DEMO

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    The effective treatment of the heat and power exhaust is a critical issue in the road map to the realization of the fusion energy. In order to provide possible, reliable, well assessed and on-time answers to DEMO, the Divertor Tokamak Test facility (DTT) has been conceived and projected to be carried out and operated within the European strategy in fusion technology. This paper, based on the invited plenary talk at the 31st virtual SOFT Conference 2020, provides an overview of the DTT scientific proposal, which is deeply illustrated in the 2019 DTT Interim Design Report

    DTT - Divertor Tokamak Test facility - Interim Design Report

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    The “Divertor Tokamak Test facility, DTT” is a milestone along the international program aimed at demonstrating – in the second half of this century – the feasibility of obtaining to commercial electricity from controlled thermonuclear fusion. DTT is a Tokamak conceived and designed in Italy with a broad international vision. The construction will be carried out in the ENEA Frascati site, mainly supported by national funds, complemented by EUROfusion and European incentive schemes for innovative investments. The project team includes more than 180 high-standard researchers from ENEA, CREATE, CNR, INFN, RFX and various universities. The volume, entitled DTT Interim Design Report (“Green Book” from the colour of the cover), briefly describes the status of the project, the planning of the design future activities and its organizational structure. The publication of the Green Book also provides an occasion for thorough discussions in the fusion community and a broad international collaboration on the DTT challenge

    From thyroid to duodenum: round-trip

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    La malattia celiaca molto spesso (2%) si associa ad altre malattie autoimmuni come la tiroidite di Hashimoto, questa \ue8 una relazione biunivoca e non \ue8 soggetta a vincoli temporali (pu\uf2 presentarsi prima la celiachia e poi la tiroidite o viceversa). Non solo la celiachia \ue8 spesso asintomatica, ma pu\uf2 capitare anche di diagnosticarla in bambini sovrappeso che con l\u2019idea classica di celiachia hanno ben poco a che fare

    Biological and clinical changes in a pediatric series treated with off-label jak inhibitors

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    Off-label use of medications is still a common practice in pediatric rheumatology. JAK inhibitors are authorized in adults in the treatment of rheumatoid arthritis, psoriatic arthritis and ulcerative colitis. Although their use is not authorized yet in children, JAK inhibitors, based on their mechanism of action and on clinical experiences in small series, have been suggested to be useful in the treatment of pediatric interferon-mediated inflammation. Accordingly, an increased interferon score may help to identify those patients who might benefit of JAK inhibitors. We describe the clinical experience with JAK inhibitors in seven children affected with severe inflammatory conditions and we discuss the correlation between clinical features and transcriptomic data. Clinical improvements were recorded in all cases. A reduction of interferon signaling was recorded in three out of seven subjects at last follow-up, irrespectively from clinical improvements. Other signal pathways with significant differences between patients and controls included upregulation of DNA repair pathway and downregulation of extracellular collagen homeostasis. Two patients developed drug-related adverse events, which were considered serious in one case. In conclusion, JAK inhibitors may offer a valuable option for children with severe interferon-mediated inflammatory disorders reducing the interferon score as well as influencing other signal pathways that deserve future studies

    PReS-FINAL-2088: Risk of severe adverse events in juvenile idiopathic arthritis and pediatric-onset inflammatory bowel disease, treated with anti-tnf drugs

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    Introduction: Severe adverse events have been described in children affected by Juvenile Idiopathic Arthritis (JIA) and Inflammatory Bowel Disease (IBD) treated with anti-tnf drugs. Objectives: To define the risk of severe adverse events in patients with JIA and IBD treated with anti-tnf drugs. Methods: This is a retrospective cohort study. All patients with JIA and IBD attending the "IRCCS Burlo Garofolo" of Trieste from 2000 to 2012 were enrolled. They were divided into 2 groups on the basis of the presence or absence of anti-tnf exposure. Severe adverse events were considered the followings: a) infections needing anti-tnf permanent suspension and/or hospitalization; b) autoimmune diseases with present or potential organ damage, except for hepatitis and cholangitis during IBD; c) anaphylaxis; d) malignancies. Univariate analysis testing the effect of anti-tnf exposure on adverse events appearance was realized. Results: 323 patients were enrolled (159 with JIA and 164 with IBD). 120 patients were exposed to anti-tnf and 203 were not. Infliximab was the most used anti-tnf (73 patients), followed by etanercept (56 patients) and adalimumab (21 patients). Mean total duration of anti-tnf therapy was 26 months (min.1, max.127). The two cohorts were comparable for sex, age, diagnosis and other therapies assumed. Severe adverse events occurred in 38 anti-tnf-exposed patients (31.7%) and 22 of the not-exposed group (10.8%), with a statistically significant difference (p = 0.000) and a relative risk (RR) of 2.9 (95% confidence interval, CI, 1.8 to 4.7). Anaphylaxis occured in 11 patients (9.2% of the anti-tnf-treated), all assuming infliximab; in the not-treated group none presented reactions (p = 0.000). Infection rate was 6.7% in the anti-tnf-treated group (8 patients) and 3.5% in the not-exposed group (7 patients) (p = 0.273, RR = 1.9, 95% CI: 0.7 to 5.2). Incidence rate of autoimmune diseases in patients treated with anti-tnf was 18.3% (22 patients) vs 7.9% in not-exposed cohort (p = 0.007, RR = 2.3, 95% CI: 1.3 to 4.2). Uveitis was the most frequent autoimmune disease. Both uveitis and lupus-like syndrome were more likely in the subgroup of patients treated with anti-tnf (p = 0.005, RR = 2.5, 95% CI: 1.1 to 6.0 for uveitis and p = 0.050 for lupus-like syndrome). No patients developed malignancies. The outcome of severe anti-tnf drug reactions was as follows: 2 out of 3 uveitis, all anaphylactic reactions, severe infections and lupus-like syndromes healed without organ damage, whereas the other autoimmune complications have been still treating with a good clinical outcome. Conclusion: The patients with JIA or IBD treated with anti-tnf have a higher risk of severe adverse events, like anaphylaxis and autoimmune diseases (in particular uveitis and lupus-like syndrome), whereas it seems that this risk does not exist for severe infections. No malignancies were observed during follow up. Our data suggest that, although the risk of severe adverse reactions to anti-tnf therapy is significant, the occurrence of a permanent damage results very low
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