Hyperferritinemia -cataract syndrome: Long-term ophthalmic observations in an Italian family

Background Hyperferritinemia-cataract syndrome (HCS) is a rare Mendelian condition characterized by bilateral cataract and high levels of serum ferritin in the absence of iron overload. Methods HCS was diagnosed in three adult siblings. In two of them it was possible to assess lens changes initially in 1995 and again in 2013. Serum ferritin, iron, transferrin concentrations and transferrin saturation percentage were also measured, and the Iron Responsive Element (IRE) region of the L-ferritin gene (FTL) was studied. Results Serum ferritin concentrations were considerably elevated while serum iron, transferrin and transferrin saturation levels were within the normal range in each sibling. Cataract changes in our patients were consistent with those previously reported in the literature. Progression of the cataract, an aspect of few studies in this syndrome, appeared to be quite limited in extent. The heterozygous +32G to T (-168G>T) substitution in the IRE of the FTL gene was detected in this family. Conclusions Ophthalmic and biochemical studies together with genetic testing confirmed HCS in three family members. Although the disorder has been extensively described in recent years, little is known regarding cataract evolution over time. In our cases, lens evaluations encompassed many years, identified bilateral cataract of typical morphology and supported the hypothesis that this unique clinical feature of the disease tends to be slowly progressive in nature, at least in adults.

Open City, Rossellini and neorealism : sixty years on

For Bakhtin, it is always important to know from where one speaks. The place from which I speak is that of a person who grew up in Italy during the economic miracle (pre-1968) in a working class family, watching film matinees on television during school holidays. All sort of films and genres were shown: from film noir to westerns, to Jean Renoir's films, German expressionism, Italian neorealism and Italian comedy. Cinema has come to represent over time a sort of memory extension that supplements lived memory of events, and one which, especially, mediates the intersection of many cultural discourses. When later in life I moved to Australia and started teaching in film studies, my choice of a film that was emblematic of neorealism went naturally to Roma città aperta (Open city hereafter) by Roberto Rossellini (1945), and not to Paisan or Sciuscà or Bicycle Thieves. My choice was certainly grounded in my personal memory - especially those aspects transmitted to me by my parents, who lived through the war and maintained that Open City had truly made them cry. With a mother who voted for the Christian Democratic Party and a father who was a unionist, I thought that this was normal in Italian families and society. In the early 1960s, the Resistance still offered a narrative of suffering and redemption, shared by Catholics or Communists. This construction of psychological realism is what I believe Open City continues to offer in time.

The new Golden Legend: Patrick Nicholas's secular saints

The work of Italian-based photo-artist Patrick Nicholas is analysed to show how his re-workings of classic ‘old-master’ paintings can be seen as the art of ‘redaction,’ shedding new light on the relationship between originality and copying. I argue that redactional creativity is both highly productive of new meanings and a reinvention of the role of the medieval Golden Legend. (Lives of the Saints).

Chi viaggia ha delle storie da raccontare : those who travel have stories to tell

This review article uses the work of Italian scholar Milly Buonanno to review the state and future of television scholarship, given that the ‘age of television’ has been overtaken by the age of computer-based media. In particular, it discusses the role of open-ended narrative through which we collectively explore the human condition.

EAP essentials : a teacher's guide to principles and practice

Book review - Ancient scholars once journeyed across the Alps to study Italian law while some sailed the Mediterranean to learn Greek philosophy or examine Arabic scripts on science, medicine and mathematics. Devotees of philosophical and religious thought migrated to the Orient in search of transcendental wisdom. Today, the quest for knowledge has not changed as English-medium universities experience unprecedented internationalisation. This book is a publication for such a time as this. The authors invite readers to "join the tribe" (Becher, 1998) and learn the specific academic discourse and culture of English for Academic Purposes (EAP).

QUT speaker identity verification system for EVALITA 2009

This document outlines the system submitted by the Speech and Audio Research Laboratory at the Queensland University of Technology (QUT) for the Speaker Identity Verication: Application task of EVALITA 2009. This submission consisted of a score-level fusion of three component systems, a joint-factor GMM system and two SVM systems using GLDS and GMM supervector kernels. Development and evaluation results are presented, demonstrating the effectiveness of this fused system approach.

The influence of family ownership on the quality of accounting information

This article explores the quality of accounting information in listed family firms. The authors exploit the features of the Italian equitymarket characterizd by high ownership concentration across all tpes of firms to disentangle the effects of family ownership from other major block holders on the quality of accounting information. The findings document that family firms convey financial information of higher quality compared to the nonfamily peers. Furthermore the authors provide evidence that the determinants of accounting quality differ across family and nonfamily firms.

Ownership structure and business evaluation practices in family SMEs : evidence from empirical research in Italy

This paper presents early results from a pilot project which aims to investigate the relationship between proprietary structure of small and medium- sized Italian family firms and their owners’ orientation towards a “business evaluation process”. Evidence from many studies point out the importance of family business in a worldwide economic environment: in Italy 93% of the businesses are represented by family firms; 98% of them have less than 50 employees (Italian Association of Family Firms, 2004) so we judged family SMEs as a relevant field of investigation. In this study we assume a broad definition of family business as “a firm whose control (50% of shares or voting rights) is closely held by the members of the same family” (Corbetta,1995). “Business evaluation process” is intended here both as “continuous evaluation process” (which is the expression of a well developed managerial attitude) or as an “immediate valuation” (i.e. in the case of new shareholder’s entrance, share exchange among siblings, etc). We set two hypotheses to be tested in this paper: the first is “quantitative” and aims to verify whether the number of owners (independent variable) in a family firm is positively correlated to the business evaluation process. If a family firm is led by only one subject, it is more likely that personal values, culture and feelings may affect his choices more than “purely economic opportunities”; so there is less concern about monitoring economic performance or about the economic value of the firm. As the shareholders’ number increases, economic aspects in managing the firm grow in importance over the personal values and "value orientation" acquires a central role. The second hypothesis investigates if and to what extent the presence of “non- family members” among the owners affects their orientation to the business evaluation process. The “Cramer’s V” test has been used to test the hypotheses; both were not confirmed from these early results; next steps will lead to make an inferential analysis on a representative sample of the population.

Le vie dell'Impero. Le strade coloniali italiane e il loro rapporto con la manualistica del Ventennio

Imperial roads. The Italian colonial routes and their relationship with 1920-40 manuals

An FPGA-based approach to multi-objective evolutionary algorithm for multi-disciplinary design optimisation

This paper investigates the field programmable gate array (FPGA) approach for multi-objective and multi-disciplinary design optimisation (MDO) problems. One class of optimisation method that has been well-studied and established for large and complex problems, such as those inherited in MDO, is multi-objective evolutionary algorithms (MOEAs). The MOEA, nondominated sorting genetic algorithm II (NSGA-II), is hardware implemented on an FPGA chip. The NSGA-II on FPGA application to multi-objective test problem suites has verified the designed implementation effectiveness. Results show that NSGA-II on FPGA is three orders of magnitude better than the PC based counterpart.

User-representative feature selection for keystroke dynamics

Continuous user authentication with keystroke dynamics uses characters sequences as features. Since users can type characters in any order, it is imperative to find character sequences (n-graphs) that are representative of user typing behavior. The contemporary feature selection approaches do not guarantee selecting frequently-typed features which may cause less accurate statistical user-representation. Furthermore, the selected features do not inherently reflect user typing behavior. We propose four statistical based feature selection techniques that mitigate limitations of existing approaches. The first technique selects the most frequently occurring features. The other three consider different user typing behaviors by selecting: n-graphs that are typed quickly; n-graphs that are typed with consistent time; and n-graphs that have large time variance among users. We use Gunetti’s keystroke dataset and k-means clustering algorithm for our experiments. The results show that among the proposed techniques, the most-frequent feature selection technique can effectively find user representative features. We further substantiate our results by comparing the most-frequent feature selection technique with three existing approaches (popular Italian words, common n-graphs, and least frequent ngraphs). We find that it performs better than the existing approaches after selecting a certain number of most-frequent n-graphs.

High speed rail and regional competitiveness

The presence of High Speed Rail (HSR) systems influences market shares of road and air transport, and the development of cities and regions they serve. With the deployment of HSR infrastructure, changes in accessibility have occurred. These changes have lead researchers to investigate effects on the economic and spatial derived variables. Contention exists when managing the trade off between efficiency, and access points which are usually in the range of hundreds of kilometres apart. In short, it is argued that intermediate cities, bypassed by HSR services, suffer a decline in their accessibility and developmental opportunities. The present Chapter will analyse possible impacts derived from the presence of HSR infrastructure. In particular, it will consider small and medium agglomerations in the vicinity of HSR corridors, not always served by HSR stations. Thus, a methodology is developed to quantify accessibility benefits and their distribution. These benefits will be investigated in relation to different rail transit strategies integrating HSR infrastructure where a HSR station cannot be positioned. These strategies are selected principally for the type of service offered: (i) cadenced, (ii) express, (iii) frequent or (iv) non-stopping. Furthermore, to ground the theoretical approach linking accessibility and competitiveness, a case study in the North-Eastern Italian regions will be used for the application of the accessibility distributive patterns between the HSR infrastructure and the selected strategies. Results indicate that benefits derive from well informed decisions on HSR station positioning and the appropriate blend of complementary services in the whole region to interface HSR infrastructure. The results are significant for all countries in Europe and worldwide, not only for investing in HSR infrastructure, but mostly in terms of building territorial cohesion, while seeking international recognition for developing successful new technology and systems.

Investigation to enhance high speed rail accessibility

High Speed Rail (HSR) is rapidly gaining popularity worldwide as a safe and efficient transport option for long-distance travel. Designed to win market shares from air transport, HSR systems optimise their productivity between increasing speeds and station spacing to offer high quality service and gain ridership. Recent studies have investigated the effects that the deployment of HSR infrastructure has on spatial distribution and the economic development of cities and regions. Findings appear mostly positive at higher geographical scales, where HSR links connect major urban centres several hundred kilometres apart and already well positioned within a national or international context. Also, at the urban level, studies have shown regeneration and concentration effects around HSR station areas with positive returns on city’s image and economy. However, doubts persist on the effects of HSR at an intermediate scale, where the accessibility trade off on station spacing limits access to many small and medium agglomerations. Thereby, their ability to participate in the development opportunities facilitated by HSR infrastructure is significantly reduced. The locational advantages deriving from transport improvements appear contrasting especially in regions that tend to have a polycentric structure, where cities may present greater accessibility disparities between those served by HSR and those left behind. This thesis fits in this context where intermediate and regional cities do not directly enjoy the presence of an HSR station while having an existing or planned proximate HSR corridor. With the aim of understanding whether there might be a solution to this apparent incongruity, the research investigates strategies to integrate HSR accessibility at the regional level. While current literature recommends to commit with ancillary investments to the uplift of station areas and the renewal of feeder systems, I hypothesised the interoperability between the HSR and the conventional networks to explore the possibilities offered by mixed traffic and infrastructure sharing. Thus, I developed a methodology to quantify the exchange of benefits deriving from this synergistic interaction. In this way, it was possible to understand which level of service quality offered by alternative transit strategies best facilitates the distribution of accessibility benefits for areas far from actual HSR stations. Therefore, strategies were selected for their type of service capable of regional extensions and urban penetrations, while incorporating a combination of specific advantages (e.g. speed, sub-urbanity, capacity, frequency and automation) in order to emulate HSR quality with increasingly efficient services. The North-eastern Italian macro region was selected as case study to ground the research offering concurrently a peripheral polycentric metropolitan form, the presence of a planned HSR corridor with some portions of HSR infrastructure implementation, and the project to develop a suburban rail service extended regionally. Results show significant distributive potential, in terms of network effects produced in relation with HSR, in increasing proportions for all the strategies considered: a regional metro rail strategy (abbreviated RMR), a regional high speed rail strategy (abbreviated RHSR), a regional light rail transit (abbreviated LRT) strategy, and a non-stopping continuous railway system (abbreviated CRS) strategy. The provision of additional tools to value HSR infrastructure against its accessibility benefits and their regional distribution through alternative strategies beyond the actual HSR stations, would have great implications, both politically and technically, in moving towards new dimensions of HSR evaluation and development.