16 resultados para Lucio Mansilla

em BORIS: Bern Open Repository and Information System - Berna - Suiça


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This paper addresses the novel notion of offering a radio access network as a service. Its components may be instantiated on general purpose platforms with pooled resources (both radio and hardware ones) dimensioned on-demand, elastically and following the pay-per-use principle. A novel architecture is proposed that supports this concept. The architecture's success is in its modularity, well-defined functional elements and clean separation between operational and control functions. By moving much processing traditionally located in hardware for computation in the cloud, it allows the optimisation of hardware utilization and reduction of deployment and operation costs. It enables operators to upgrade their network as well as quickly deploy and adapt resources to demand. Also, new players may easily enter the market, permitting a virtual network operator to provide connectivity to its users.

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Basato su interviste con i principali attori tedeschi e su un’analisi della letteratura, l’articolo analizza lo sviluppo recente dell’economia tedesca e la strategia tedesca nell’affrontare la crisi dell’eurozona. La Germania è uno stato commerciale (trading state), la cui crescita è fortemente trainata dalle esportazioni. Fino agli anni novanta, rigidità istituzionali forti, nel sistema di relazioni industriali e nel sistema di protezione sociale, contribuivano a conciliare lo sviluppo delle esportazioni con una crescita armonica dei consumi interni, contribuendo cosi a ingabbiare la «tigre» tedesca. A partire dagli anni novanta, sia le relazioni industriali sia la protezione sociale sono state fortemente liberalizzate, stimolando ulteriormente la competitività estera e indebolendo i consumi interni. Il modello economico tedesco, cosi come è venuto profilandosi negli ultimi dieci anni, è alla base delle politiche di austerità che la Germania impone all’Europa. Tali politiche sono fortemente condivise dai partiti politici, dagli attori sociali e dall’opinione pubblica, e le probabilità che la strategia tedesca cambi sono minime.

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Based on interviews with the main German actors and on secondary sources, the article examines the recent development of the German political economy, and the German strategy vis-à-vis the Euro zone. Germany is a trading state whose economic growth is strongly export-led. Until the years 1990s, strong institutional rigidities, both in industrial relations and in the welfare state, contributed to reconcile export growth with household consumption, thus keeping the German “tiger” on a leash. From the early 1990s on, however, both industrial relations and social protections have been strongly liberalized, thus further stimulating external competitiveness and reducing the role of consumption in the German growth model. The unleashed trading state shapes the German response to the Euro crisis and the austerity policies that Germany imposes to Europe. These policies are strongly supported by political parties, social actors, and public opinion in Germany, and the likelihood that they change in the near future is minimal.

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OBJECTIVE To give a comprehensive overview of the phenotypic and genetic spectrum of STXBP1 encephalopathy (STXBP1-E) by systematically reviewing newly diagnosed and previously reported patients. METHODS We recruited newly diagnosed patients with STXBP1 mutations through an international network of clinicians and geneticists. Furthermore, we performed a systematic literature search to review the phenotypes of all previously reported patients. RESULTS We describe the phenotypic features of 147 patients with STXBP1-E including 45 previously unreported patients with 33 novel STXBP1 mutations. All patients have intellectual disability (ID), which is mostly severe to profound (88%). Ninety-five percent of patients have epilepsy. While one-third of patients presented with Ohtahara syndrome (21%) or West syndrome (9.5%), the majority has a nonsyndromic early-onset epilepsy and encephalopathy (53%) with epileptic spasms or tonic seizures as main seizure type. We found no correlation between severity of seizures and severity of ID or between mutation type and seizure characteristics or cognitive outcome. Neurologic comorbidities including autistic features and movement disorders are frequent. We also report 2 previously unreported adult patients with prominent extrapyramidal features. CONCLUSION De novo STXBP1 mutations are among the most frequent causes of epilepsy and encephalopathy. Most patients have severe to profound ID with little correlation among seizure onset, seizure severity, and the degree of ID. Accordingly, we hypothesize that seizure severity and ID present 2 independent dimensions of the STXBP1-E phenotype. STXBP1-E may be conceptualized as a complex neurodevelopmental disorder rather than a primary epileptic encephalopathy.