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Jirakova A.,Charles University | Rajska L.,Charles University | Rob F.,Charles University | Dzambova M.,Charles University | And 6 more authors.
Dermatologic Therapy | Year: 2015

Pachyonychia congenita (PC) is a rare autosomal dominant skin disorder characterized predominantly by hypertrophic nail dystrophy, oral leukokeratosis, and painful palmoplantar keratoderma. It is associated with a mutation in one of five keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17. The International PC Research Registry (IPCRR) confirms that as of January 2014 there have been 547 cases of PC genetically confirmed. It is estimated that there are between 2000 and 10,000 cases of PC in the world. However, the exact prevalence of PC is not yet established. We report a case of PC-K6a, p.Arg164Pro, in a 40-year-old man. Initially he was diagnosed with onychomycosis and was treated with systemic antifungals. This is the first genetically confirmed case of PC in the Czech Republic. © 2014 Wiley Periodicals, Inc.


Flora A.,University of Naples Federico II | Gargano S.,University of Naples Federico II | Lirer S.,University of Guglielmo Marconi | Mele L.,University of Naples Federico II
Procedia Engineering | Year: 2016

The management of the huge amount of dredged sediments is an important issue to be solved worldwide, and dewatering is by far the most critical step when fine grained sediments are involved. Different technologies have been proposed in time to speed up the process. Even though electro-kinetic treatment is in principle one of them, it has not been implemented yet at an industrial scale, and only few trial applications are known. For such a reason, a multidisciplinary research activity is ongoing at the University of Napoli Federico II in the framework of the EU commitment ROSE with the aim to analyse the effectiveness and feasibility of such a technology from the lab to the site scale. In this paper, some evidences stemming from lab tests are presented. The results indicate that the application of low voltages improves the mechanical behaviour of the tested soil. In this case, however, the improvement is due more to a change in microstructure than to a decrease in void ratio. © 2016 Published by Elsevier Ltd.


Fiori C.,University of Rome La Sapienza | Dell'Era A.,University of Rome La Sapienza | Dell'Era A.,University of Guglielmo Marconi | Zuccari F.,University of Rome La Sapienza | And 6 more authors.
International Journal of Hydrogen Energy | Year: 2015

This study identifies possible alternatives in the currently use of hydrides in submarine applications in order to increase the storage capacity and, therefore, the autonomy (Air Independent Propulsion) of the boat. The study proposes a plug and play solution which requires no changes for what concerning the current propulsion system, nor the auxiliary systems of absorption and desorption of hydrogen, nor the power supply system of fuel cells. Different constraints have been taken into account, as the storage volume density, the operating conditions (pressure and temperature), the number of charge and discharge cycles. Taking into account that it is desirable to maintain the set-up of the actual systems (for what concerning the spaces, the pressure and temperature) the solution obtained in the present study ranges in between the following target values: 120g/dm3 for the storage capacity in volume; 20-50°C for the operating temperature, 2-3bar for the hydrogen desorption pressure and 150 charge and discharge cycles that can ensure 5 cycles per year for 30 years. Under these constraints, a significant increase of the autonomy of the boat has been evaluated. © 2015 Hydrogen Energy Publications, LLC.


Grande E.,University of Guglielmo Marconi | Imbimbo M.,University of Cassino and Southern Lazio
Mechanical Systems and Signal Processing | Year: 2016

The paper proposes a fusion approach for damage detection in structural applications in the case of multiple damage locations and three-dimensional systems. Based on the Dempster-Shafer evidence theory, a multi-stage approach is proposed with the mode shapes assumed as primary sources and local decisions based on a flexibility method. The proposed approach has been applied to two case studies, a a fixed end beam analyzed in other papers and a three dimensional structures codified in a Benchmark problem. Both the case studies have shown the ability and the efficiency of the proposed approach to detect damage also in the case of multiple damage, limited number of identified parameters and noise measurements. © 2016 Elsevier Ltd.


Fiori C.,University of Rome La Sapienza | Dell'Era A.,University of Rome La Sapienza | Dell'Era A.,University of Guglielmo Marconi | Zuccari F.,University of Rome La Sapienza | And 6 more authors.
International Journal of Hydrogen Energy | Year: 2015

Hydrogen is considered one of the most important energy vector of the future and fuel in transport sector. The Fuel Cells (FCs) Traction System present some advantages respect to the traditional traction engine, consisting in lower emissions and noise. The more suitable Fuel Cells in automotive applications are those that use Polymer Electrolyte Membrane (PEM). The main obstacles to the commercialization of PEM fuel cells are largely concerning the cost, mechanical weakness and low durability of the membranes with increasing temperature. This latter aspect in particular referring to the fact that water is present in the membranes, thereby limiting the operating temperature of a fuel cell, which on average is about 80 °C. This in turn results in lower performance of the fuel cells due to a slower kinetics of electrodes and essentially no CO tolerance. It can groped to improve the performance of a PEM increasing the temperature above 100 °C, changing the membrane type making it resistant to the natural increase in temperature of the system so as to improve the electrodes kinetics. The present work has the purpose of highlighting the orientation of the current research towards the development of specific types of membrane for the FC performance improvement. © 2015 Hydrogen Energy Publications, LLC. All rights reserved.


Fiori C.,University of Rome La Sapienza | Dell'Era A.,University of Guglielmo Marconi | Zuccari F.,University of Guglielmo Marconi | Santiangelia A.,University of Guglielmo Marconi | And 2 more authors.
20th World Hydrogen Energy Conference, WHEC 2014 | Year: 2014

Hydrogen is considered one of the most important energy vector of the future and fuel in transport sector .The FuelCells (FC)present some advantages respect to the traditional traction engine, consisting in lower emissions and noise. The more suitable Fuel Cells in automotive applications are those that use Polymer Electrolyte Membrane (PEM).The main obstacles to the commercialization of PEM fuel cells are largely concerning the cost, mechanical weakness and low durability of the membranes with increasing temperature. This latter aspect in particular referring to the fact that water is present in the membranes, thereby limiting the operating temperature of a fuel cell, which on average is about 80 °C. This in turn results in lower performance of the fuel cells due to a slower kinetics of electrodes and essentially no CO tolerance. It can groped to improve the performance of a PEM increasing the temperature above 100 °C, changing the membrane type making it resistant to the natural increase in temperature of the system so as to improve the electrodes kinetics. The present work has the purpose of highlighting the orientation of the current research towards the development of specific types of membrane for the FC performance improvement. Copyright © (2014) by the Committee of WHEC2014 All rights reserved.


Santiangeli A.,University of Guglielmo Marconi | Fiori C.,University of Rome La Sapienza | Zuccari F.,University of Guglielmo Marconi | Dell'Era A.,University of Guglielmo Marconi | And 2 more authors.
Energy Procedia | Year: 2014

The purpose of this study is to evaluate on a real urban driving cycle the performance of a plug-in hybrid-electric vehicle, which is part of a series of pre-marketing vehicles, taking into account the real driving conditions. For this purpose have been considered the driving style influence, the traffic level, the path slope and the comfort level requested inside the vehicle by the driver. In particular the influence of auxiliary systems consumption on the total consumption of the vehicle has been evaluated. Attention has been paid to assess the effect of auxiliary systems consumption on the autonomy of the vehicle on electric mode. The data were collected under real driving cycles in the city of Rome. The importance and innovation of this work consist of two principal concepts: (i) the auxiliary systems consumption and its influences on total consumption and (ii) the acquisition analysis under real driving cycle. Different drivers were involved during the acquisition campaign along several itineraries characterized by the features of a real urban cycle (length, slope, traffic light posts, etc.). The data acquisition was carried out on-board in real time using a measurement chain which included: OBDII (On - Board Diagnostic systems - second version), hardware, laptops and software. Acquired parameters were: traveled distance, speed, air flow, equivalence ratio, RPM, voltage, SOC (State Of Charge) of the batteries. The calculated variables from acquired data were: fuel consumption, consumption and incidence of auxiliaries on the autonomy in electric drive. © 2013 The Authors.

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