32 research outputs found

    Design Optimization, Cost and Risk Analysis of CNG Vessels Transportation

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    The GASVESSEL Project, financed by the EU under H2020, aims to prove the technoeconomic feasibility of a new CNG (Compressed Natural Gas) transport concept, enabled by a novel patented Pressure Vessel manufacturing technology and a new conceptual ship design including safe on- and off-loading solution. In this paper we illustrate how the process automation and web-based collaboration software from ESTECO have been used by the partners in the different phases of the Project for the design of the system. In function of each different geographical scenarios (which includes East Mediterranean, Barents Sea and Black Sea) and gas demand, parameters such as ship size and number, storage and facilities units at the ports have been optimized in order to reduce the transportation costs and therefore gas tariff. Components of the gas vessels, in particular material and type of the fibers that wraps the liner, have been then optimized to minimize weights while respecting high value of safety factors. Finally, a CFD study is performed to analyze the risks related to gas leakages and explosion

    Oral manifestation of Goltz-Gorlin syndrome in a young girl

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    Introduction Focal dermal hypoplasia (Goltz-Gorlin syndrome) is a multi-system disorder characterized by involvement of skin, skeletal system, eyes and face. It is caused by loss-of-function mutations in the PORCN gene. We report the case of a young female, focusing on the dental features. Aim To describe the oral manifestation of a rare disorder that resembles ectodermal dysplasia (ED). Case report Clinical, radiological and genetic findings revealed common features of Goltz-Gorlin syndrome and pure ED. Oro-dental characteristics of the patient mostly corresponded to those described in the literature. However, previously unreported oro-dental findings such as taurodontism, peg-shaped teeth and microdontia are considered unusual for Goltz-Gorlin syndrome, but similar to the dental features of hypohidrotic ED. Clinical characterization of the patient by a multidisciplinary approach is described and a comprehensive review of the literature is presented

    A novel INDEL mutation in the EDA gene resulting in a distinct X- linked hypohidrotic ectodermal dysplasia phenotype in an Italian family

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    A novel INDEL mutation in theEDA gene resulting in a distinctX- linked hypohidroticectoder mal dysplasia phenotypein an Italian familyEditorX-Linked Hypohidrotic Ectodermal Dysplasia (XL-HED; MIM305100) is characterized by hypodontia, misshaped teeth, hypo-hidrosis, sparse hair, peculiar facial features,1,2and occurs in lessthan 1 in every 100.000 individuals.1XL-HED is caused bymutations in the Ectodysplasin-A (EDA) gene located at Xq12-q13 with more than 100 causative mutations reported todate.1,3,4The identification of disease-causing mutations con-firms the diagnosis, however, does not automatically imply agenotype\u2013phenotype correlation

    Access to administrative documents and to public sector information in Italy

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    Law No. 241 of 1990 on administrative procedure (Italian APA) established general rules on the right of access to administrative documents for the first time in the Italian legal system, which partly reproduced rules defined in sectorial legislations. From such very restrictive regime of access to administrative documents\u2014lately accompanied by a rather demagogical obligation imposed on public administrations to disclose a set of information in the context of the so-called open data policies\u2014Italy has recently moved forth to public access to data and documents held by public administrations

    Commentario al codice dei contratti pubblici

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    "Commentario al codice dei contratti pubblici", a cura di Marcello Clarich, analizza la disciplina dei contratti pubblici che \ue8 stata oggetto negli ultimi anni di ripetuti interventi legislativi fino al recentissimo Decreto legge \u201cSblocca cantieri\u201d (convertito nella legge 14 giugno 2019, n. 55) e che ha modificato numerose disposizioni del Codice dei contratti pubblici (d.lgs. 18 aprile 2016, n. 50). Stazioni appaltanti, imprese e professionisti troveranno nel Commentario al Codice un quadro completo e aggiornato della disciplina vigente con i principali orientamenti della giurisprudenza e dell\u2019Autorit\ue0 nazionale anticorruzione, nonch\ue9 con riferimenti essenziali alla dottrina. "Commentario al codice dei contratti pubblici" \ue8 suddiviso in capitoli dedicati al commento degli articoli relativi ai singoli istituti e ale fasi delle procedure di affidamento (bandi e avvisi di gara, prequalifica, selezione delle offerte, subappalto, ecc.). In questo modo, il Commentario d\ue0 un ordine sistematico che consente ai lettori di orientarsi in una disciplina complessa e frammentaria. Il volume tratta anche i profili penali, tributari ed economici del public procurement la cui conoscenza \ue8 indispensabile per ben inquadrare e applicare il Codice. I lettori potranno seguire l\u2019evoluzione della normativa tramite una newsletter periodica. Il volume \ue8 aggiornato alla sentenza della Corte di Giustizia dell\u2019Unione europea 26 settembre 2019 C-63/18 che ha dichiarato illegittimo il limite del 30% del subappalto

    Commentario al Codice dei contratti pubblici

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    Il volume offre un commento sistematico al codice dei contratti pubblici, approvato con il d.lgs. n. 163/2006. Il testo è integrato anche con un'analisi economico del mercato dei contratti pubblic

    Commentario al Codice dei Contratti Pubblici - seconda edizione

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    Il volume commenta il Codice dei Contratti Pubblici di cui al d.lgs. 50/201
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