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.

Saga land: Richard Fidler and Kári Gíslason in Iceland

This is a four-part radio documentary produced for the Australian Broadcasting Corporation's Radio National. Richard Fidler and Kári Gíslason travel to Iceland to tell the stories of the ancient Vikings, and to settle a longstanding family mystery.

The prankster’s passion: Using theatrical terms to unpack players motivations in popular social performance

Amongst social players, the prank, as a social performance form, holds a lot of potential to impact on personal, relational and social status within a group or between one group and another group. More than simply showing off, a prank in the strictest definition of the term, is a social performance in which one player, a prankster, deploys mischief, trickery or deceit, to cause a moment of anxiety, fear or anger about a happening for another spectator-become-collaborating-player, a prankee – to enhance social bonds, entertain, or comment on a social, cultural or political phenomenon. During a prank, the prankster’s ability to be creative, clever or culturally astute, and the prankee’s ability to be duped, be a good sport, play along, or even play/pay the prankster back, both become fodder for other spectators and society to scrutinize. In Australia, pranking traditions are popular with many social groups, from the community-building pranks of footballers, bucks parties and ‘drop bear’ tales told to tourists, to the more controversial pranks of radio shock jocks, activists and artists. In this paper, I consider whether theatrical terms – theoretical terms from the stage such as actor, acting, objective, arc, performance, audience and emotion, such as those offered by Joseph Roach – are useful in understanding the passion some social players show for pranksterism. Are theatrical terms such as Roach’s as useful as analysts of social self-performance such as Erving Goffman suggest they are? Do they assist in understanding the personal actions, reactions and emotions of prankster and prankee? Do they assist in understanding the power relations between prankster and prankee? Do they assist in understanding the relation between the prank – be it an everyday prank amongst families, friends and coworkers, an entertainment program prank of the sort seen on Prank Patrol, Punked or Scare Tactics, or an activist pranks perpetrated by a guerrilla artist, ‘jammers’ or ‘hackers’ intent on turning dominant social systems back on themselves – the social players, and the public sphere in which the prank takes place? I reflect on how reading pranks as performances, by players, for highly participatory audiences, helps understand why they are so prevalent, and so recurrent across times, cultures and contexts, and also so controversial when not performed well enough – or when performed too well – prompting outrage from the prankster, prankee or society as passionate as any debate about a performance by players in a theatre.

Creative Labor in Cinema and Media Industries

Introduction The rapidly burgeoning popularity of cinema at the beginning of the 20th century favored industrialized modes of creativity organized around large production studios that could churn out a steady stream of narrative feature films. By the mid-1910s, a handful of Hollywood studios became leaders in the production, distribution, and exhibition of popular commercial movies. In order to serve incessant demand for new titles, the studios relied on a set of conventions that allowed them to regularize production and realize workplace efficiencies. This entailed a socialized mode of creativity that would later be adopted by radio and television broadcasters. It would also become a model for cinema and media production around the world, both for commercial and state-supported institutions. Even today the core tenets of industrialized creativity prevail in most large media enterprises. During the 1980s and 1990s, however, media industries began to change radically, driven by forces of neoliberalism, corporate conglomeration, globalization, and technological innovation. Today, screen media are created both by large-scale production units and by networked ensembles of talent and skilled labor. Moreover, digital media production may take place in small shops or via the collective labor of media users or fans who have attracted attention due to their hyphenated status as both producers and users of media (i.e., “prosumers”). Studies of screen media labor fall into five conceptual and methodological categories: historical studies of labor relations, ethnographically inspired investigations of workplace dynamics, critical analyses of the spatial and social organization of labor, and normative assessments of industrialized creativity.