Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center

AIM: To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever (UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever score can help predicting the response to treatment and the outcome at follow-up. METHODS: Clinical and therapeutic information of patients with recurrent fever who presented at a single pediatric rheumatology center from January 2006 through April 2016 were retrospectively collected. Patients with a clinical suspicion of hereditary periodic fever (HPF) syndrome and patients with clinical picture of periodic fever, aphthae, pharingitis, adenitis (PFAPA) who were refractory to tonsillectomy underwent molecular analysis of five HPF-related genes: MEFV (NM_000243.2), MVK (NM_000431.3), TNFRSF1A (NM_001065.3), NLRP3 (NM_001079821.2), NLRP12 (NM_001277126.1). All patients who had a negative genetic result were defined as UPF and further investigated. PRINTO-Eurofever score for clinical diagnosis of HPF was calculated in all cases. RESULTS: Of the 221 patients evaluated for periodic fever, twelve subjects with a clinical picture of PFAPA who were refractory to tonsillectomy and 22 subjects with a clinical suspicion of HPF underwent genetic analysis. Twenty-three patients (10.4%) resulted negative and were classified as UPF. The median age at presentation of patients with UPF was 9.5 mo (IQR 4-24). Patients with UPF had a higher frequency of aphthae (52.2% vs 0%, P = 0.0026) and musculoskeletal pain (65.2% vs 18.2%, P = 0.0255) than patients with genetic confirmed HPF. Also, patients with UPF had a higher frequency of aphthous stomatitis (52.2% vs 10.7%, P < 0.0001), musculoskeletal pain (65.2% vs 8,0%, P < 0.0001), and abdominal pain (52.2% vs 4.8%, P < 0.0001) and a lower frequency of pharyngitis (56.6% vs 81.3%, P = 0.0127) compared with typical PFAPA in the same cohort. Twenty-one of 23 patients with UPF (91.3%) received steroids, being effective in 16; 13 (56.2%) were given colchicine, which was effective in 6. Symptoms resolution occurred in 2 patients with UPF at last follow-up. Classification according to the PRINTO-Eurofever score did not correlate with treatment response and prognosis. CONCLUSION: UPF is not a rare diagnosis among patients with periodic fever. Clinical presentation place UPF half way on a clinical spectrum between PFAPA and HPF. The PRINTO-Eurofever score is not useful to predict clinical outcome and treatment response in these patients

A complicated case of pulmonary embolism in a patient with renal failure

We describe a case of pulmonary embolism with instable hemodynamics in a patient with renal failure; the case is complicated by heparin-induced thrombocytopenia (HIT). Renal failure has a high prevalence in hospitalized patients and is a restriction on administration of low molecular weight heparins (LMWH) and fondaparinux. HIT is a potentially life-threatening complication, therefore a precocious diagnosis is essential. Therapy consists in the immediate stop of heparin’s administration and in the administration of non-heparin antithrombotic drugs

Impatto del microbioma (polmonare e intestinale) sull’asma

L’asma è una delle patologie croniche più diffuse e rappresenta la malattia respiratoria cronica più frequente nell’età pediatrica. Sono sempre più numerosi gli studi volti a individuare delle strategie preventive per ridurne l’incidenza: negli ultimi decenni è stato dimostrato che esiste una “finestra” temporale che si apre già durante la vita intrauterina e nella quale vari fattori ambientali possono interagire con il substrato genetico per favorire l’insorgenza dell’asma e, più in generale, delle malattie allergiche. Negli ultimi anni è stato ampiamente studiato il ruolo del microbioma intestinale dimostrandone la capacità di modulare la risposta immunitaria. Una disbiosi intestinale in epoca precoce, con sbilanciamento della composizione del microbioma a favore di Escherichia coli e Clostridium difficile e a discapito dei Bifidobacteria, può predisporre allo sviluppo delle allergopatie. Più recentemente è stato dimostrato che esiste un microbioma anche a livello delle vie aeree inferiori, la cui composizione può essere influenzata dalle infezioni virali e che, nei soggetti asmatici, è caratterizzata dalla prevalenza del phylum Proteobacteria. Non è stato ancora dimostrato se sia possibile ridurre l’insorgenza dell’asma agendo sul microbioma, mentre è necessario tenere a mente la necessità di ridurre l’impiego degli antibiotici per limitare le interferenze sul microbioma, soprattutto nei neonati e nei lattanti

Il link asma-obesità: aspetti patogenetici, clinico-funzionali e diagnostico-terapeutici

L’asma e l’obesità sono considerate un problema primario di salute pubblica dell’età infantile, che sta assumendo proporzioni globalmente “epidemiche”. Diversi studi epidemiologici hanno chiaramente evidenziato la presenza di un’associazione tra le due patologie. Tale complessa interazione patogenetica vede coinvolti fattori genetici, di sviluppo, di funzione polmonare, immunologici e comportamentali; alcuni di essi sono ad oggi ancora poco studiati e conosciuti. Per tale motivo, non è possibile identificare un meccanismo prevalente sugli altri che sia alla base della relazione causale tra le due patologie. Il crescente interesse scientifico nei confronti dell’associazione tra asma e obesità ha contribuito a delineare diversi fenotipi di patologia presenti nelle varie epoche della vita. La caratterizzazione clinica dei soggetti asmatici obesi è presupposto fondamentale per identificare terapie mirate a raggiungere il controllo dell’asma e contemporaneamente a ridurre il peso del soggetto prevenendo le complicanze legate all’obesità

I farmaci biologici nell’asma del bambino

I farmaci biologici hanno recentemente rivoluzionato l’approccio terapeutico al paziente con asma grave. Attraverso l’inibizione di precisi target molecolari implicati nella patogenesi della malattia asmatica, questi farmaci trovano un’innovativa applicazione nell’ambito della medicina moderna che, sempre più spesso, si indirizza verso un trattamento personalizzato sulla base delle specifiche caratteristiche infiammatorie (endotipi con relativi biomarcatori) che la patologia presenta nel singolo paziente. Tra i farmaci biologici disponibili per l’età pediatrica, ad oggi omalizumab è il farmaco di scelta per la terapia aggiuntiva dell’asma grave, con significative evidenze di efficacia e sicurezza e con maggiore esperienza clinica in “real life”. Le nuove molecole, come mepolizumab, reslizumab e dupilumab, sono attualmente in fase di sperimentazione clinica per l’età pediatrica, sulla base dei risultati positivi ottenuti negli studi sulla popolazione adulta

D5.3 Resilience assessment of current farming systems across the European Union

For improving sustainability and resilience of EU farming system, the current state needs to be assessed, before being able to move on to future scenarios. Assessing sustainability and resilience of farming systems is a multi-faceted research challenge in terms of the scientific domains and scales of integration (farm, household, farming system level) that need to be covered. Hence, in SURE-Farm, multiple approaches are used to evaluate current sustainability and resilience and its underlying structures and drivers. To maintain consistency across the different approaches, all approaches are connected to a resilience framework which was developed for the unique purposes of SURE-Farm. The resilience framework follows five steps: 1) the farming system (resilience of what?), 2) challenges (resilience to what?), 3) functions (resilience for what purpose?), 4) resilience capacities, 5) resilience attributes (what enhances resilience?). The framework was operationalized in 11 case studies across the EU. Applied approaches differ in disciplinary orientation and the farming system process they focus on. Three approaches focus on risk management: 1) a farm survey with a main focus on risk management and risk management strategies, 2) interviews on farmers’ learning capacity and networks of influence, and 3) Focus Groups on risk management. Two approaches address farm demographics: 4) interviews on farm demographics, and 5) AgriPoliS Focus Group workshops on structural change of farming systems from a (farm) demographics perspective. One approach applied so far addresses governance: 6) the Resilience Assessment Tool that evaluates how policies and legislation support resilience of farming systems. Two methods address agricultural production and delivery of public and private goods: 7) the Framework of Participatory Impact Assessment for sustainable and resilient farming systems (FoPIA-SURE-Farm), aiming to integrate multiple perspectives at farming system level, and 8) the Ecosystem Services assessment that evaluates the delivery of public and private goods. In a few case studies, additional methods were applied. Specifically, in the Italian case study, additional statistical approaches were used to increase the support for risk management options (Appendix A and Appendix B). Results of the different methods were compared and synthesized per step of the resilience framework. Synthesized results were used to determine the position of the farming system in the adaptive cycle, i.e. in the exploitation, conservation, release, or reorganization phase. Dependent on the current phase of the farming system, strategies for improving sustainability and resilience were developed. Results were synthesized around the three aspects characterizing the SURE-Farm framework, i.e. (i) it studies resilience at the farming system level, (ii) considers three resilience capacities, and (iii) assesses resilience in the context of the (changing) functions of the system. (i) Many actors are part of the farming system. However, resilience-enhancing strategies are mostly defined at the farm level. In each farming system multiple actors are considered to be part of the system, such as consultants, neighbors, local selling networks and nature organizations. The number of different farming system actors beyond the focal farmers varies between 4 (in French beef and Italian hazelnut systems) and 14 (large-scale arable systems in the UK). These large numbers of actors illustrate the relevance of looking at farming system level rather than at farm level. It also suggests that discussions about resilience and future strategies need to embrace all of these actors. (ii) At system level there is a low perceived capacity to transform. Yet, most systems appear to be at the start of a period in which (incremental) transformation is required. At system level, the capacity to transform is perceived to be relatively low, except in the Romanian mixed farming system. The latter may reflect a combination of ample room to grow and a relatively stable environment (especially when compared to the past 30 to 50 years). The relatively low capacity to transform in the majority of systems is not in line with the suggestion that most systems are at the start of (incremental) transformation, or, at least, reached a situation in which they can no longer grow. Further growth is only deemed possible in the Belgium dairy, Italian hazelnut, Polish fruit and Romanian mixed farming systems. (iii) System functions score well with regard to the delivery of high-quality and safe food but face problems with quality of rural life and protecting biodiversity. Resilience capacities can only be understood in the context of the functions to be delivered by a farming system. We find that across all systems required functions are a mix of private and public goods. With regard to the capacity to deliver private goods, all systems perform well with respect to high-quality and safe food. Viability of farm income is regarded moderate or low in the livestock systems in Belgium (dairy), France (beef) and Sweden (broilers), and the fruit farming system in Poland. Across all functions, attention is especially needed for the delivery of public goods. More specifically the quality of rural life and infrastructure are frequently classified as being important, but currently performing bad. Despite the concerns about the delivery of public goods, many future strategies still focus on improving the delivery of private goods. Suggestions in the area of public goods include among others the implementation of conservation farming in the UK arable system, improved water management in the Italian hazelnut system, and introduction of technologies which reduce the use of herbicides in Polish fruit systems. It is questionable whether these are sufficient to address the need to improve the maintenance of natural resources, biodiversity and attractiveness of rural areas. With regard to the changing of functions over time, we did not find evidence for this in our farming systems

Printability, microstructure, and flow dynamics of phase-separated edible 3D inks

Personalizing the nutrition and sensorial attributes of 3D printed foods primarily requires various multiscale properties to be individually tailored. Herein, multiscale inks are produced by segregative phase separation, a candidate for further 3D inks texture control, of gellan gum (GG), and whey protein isolate (WPI). The inks microstructure, rheological properties, flow dynamics, their impact on printability, and properties-variables interactions are analyzed using experimental design and clustering. The gels are a GG matrix structured with WPI beads or fibers ranging from 100??m in diameter. A straightforward, six-step printability test determines that high-quality prints require increasing viscosity, which is obtained by reducing the size and length of the WPI beads. Also, flow dynamics and rheology models predict the shear stress and extrusion force, according to the print settings and food-inks fluid properties. The phase-separated inks enable printing at high speed (>25/50?mm/s) upon low extrusion forces (<50?N) and low shear stresses (<500?Pa), according to the calculations and model validation. These printability evaluation methodologies and fabrication of phase-separated inks are particularly interesting for 3D food printing, bioprinting, or biomaterials applications.Nanotechnology-based functional solutions project, funded by ERDF and CCDR-N, under the call Norte2020 (Ref. NORTE-01-0145-FEDER-000019) and Enhance Microalgae (High added-value industrial opportunities for microalgae in the Atlantic Area), funded by ERDF, under the Call Interreg Atlantic Area 2014–2020 (Ref. EAPA_338/2016)info:eu-repo/semantics/publishedVersio

Measurements of Higgs boson production and couplings in diboson final states with the ATLAS detector at the LHC

Measurements are presented of production properties and couplings of the recently discovered Higgs boson using the decays into boson pairs, H →γ γ, H → Z Z∗ →4l and H →W W∗ →lνlν. The results are based on the complete pp collision data sample recorded by the ATLAS experiment at the CERN Large Hadron Collider at centre-of-mass energies of √s = 7 TeV and √s = 8 TeV, corresponding to an integrated luminosity of about 25 fb−1. Evidence for Higgs boson production through vector-boson fusion is reported. Results of combined fits probing Higgs boson couplings to fermions and bosons, as well as anomalous contributions to loop-induced production and decay modes, are presented. All measurements are consistent with expectations for the Standard Model Higgs boson

Standalone vertex finding in the ATLAS muon spectrometer

A dedicated reconstruction algorithm to find decay vertices in the ATLAS muon spectrometer is presented. The algorithm searches the region just upstream of or inside the muon spectrometer volume for multi-particle vertices that originate from the decay of particles with long decay paths. The performance of the algorithm is evaluated using both a sample of simulated Higgs boson events, in which the Higgs boson decays to long-lived neutral particles that in turn decay to bbar b final states, and pp collision data at √s = 7 TeV collected with the ATLAS detector at the LHC during 2011

Measurement of the top quark-pair production cross section with ATLAS in pp collisions at \sqrt{s}=7\TeV

A measurement of the production cross-section for top quark pairs(\ttbar) in $pp$ collisions at \sqrt{s}=7 \TeV is presented using data recorded with the ATLAS detector at the Large Hadron Collider. Events are selected in two different topologies: single lepton (electron $e$ or muon $\mu$) with large missing transverse energy and at least four jets, and dilepton ($ee$, $\mu\mu$ or $e\mu$) with large missing transverse energy and at least two jets. In a data sample of 2.9 pb-1, 37 candidate events are observed in the single-lepton topology and 9 events in the dilepton topology. The corresponding expected backgrounds from non-\ttbar Standard Model processes are estimated using data-driven methods and determined to be $12.2 \pm 3.9$ events and $2.5 \pm 0.6$ events, respectively. The kinematic properties of the selected events are consistent with SM \ttbar production. The inclusive top quark pair production cross-section is measured to be \sigmattbar=145 \pm 31 ^{+42}_{-27} pb where the first uncertainty is statistical and the second systematic. The measurement agrees with perturbative QCD calculations.Comment: 30 pages plus author list (50 pages total), 9 figures, 11 tables, CERN-PH number and final journal adde