High field brain proton magnetic resonance spectroscopy and volumetry in children with chronic, compensated liver disease - A pilot study.

There is increasing evidence that children or young adults having acquired liver disease in childhood display neurocognitive impairment which may become more apparent as they grow older. The molecular, cellular and morphological underpinnings of this clinical problem are incompletely understood. Therefore, we used the advantages of highly-resolved proton magnetic resonance spectroscopy at ultra-high magnetic field to analyze the neurometabolic profile and brain morphometry of children with chronic, compensated liver disease, hypothesizing that with high field spectroscopy we would identify early evidence of rising brain glutamine and decreased myoinositol, such as has been described both in animals and humans with more significant liver disease. Patients (n = 5) and age-matched controls (n = 19) underwent 7T MR scans and short echo time <sup>1</sup> H MR spectra were acquired using the semi-adiabatic SPECIAL sequence in two voxels located in gray and white matter dominated prefrontal cortex, respectively. A 3D MP2RAGE sequence was also acquired for brain volumetry and T <sub>1</sub> mapping. Liver disease had to have developed at least 6 months before entering the study. Subjects underwent routine blood analysis and neurocognitive testing using validated methods within 3 months of MRI and MRS. Five children aged 8-16 years with liver disease acquired in childhood were included. Baseline biological characteristics were similar among patients. There were no statistically significant differences between subjects and controls in brain metabolite levels or brain volumetry. Finally, there were minor neurocognitive fluctuations including attention deficit in one child, but none fell in the statistically significant range. Children with chronic, compensated liver disease did not display an abnormal neurometabolic profile, neurocognitive abnormalities, or signal intensity changes in the globus pallidus. Despite the absence of neurometabolic changes, it is an opportunity to emphasize that it is only by developing the use of <sup>1</sup> H MRS at high field in the clinical arena that we will understand the significance and generalizability of these findings in children with CLD. Healthy children displayed neurometabolic regional differences as previously reported in adult subjects

Congenital disorder of glycosylation type Id (CDG Id): phenotypic, biochemical and molecular characterization of a new patient

Summary: Congenital disorders of glycosylation (CDG) are a family of multisystem inherited disorders caused by defects in the biosynthesis of N- or O-glycans. Among the many different subtypes of CDG, the defect of a mannosyltransferase encoded by the human ALG3 gene (chromosome 3q27) is known to cause CDG Id. Six patients with CDG Id have been described in the literature so far. We further delineate the clinical, biochemical, neuroradiological and molecular features of CDG Id by reporting an additional patient bearing a novel missense mutation in the ALG3 gene. All patients with CDG Id display a slowly progressive encephalopathy with microcephaly, severe psychomotor retardation and epileptic seizures. They also share some typical dysmorphic features but they do not present the multisystem involvement observed in other CDG syndromes or any biological marker abnormalities. Unusually marked osteopenia is a feature in some patients and may remain undiagnosed until revealed by pathological fractures. Serum transferrin screening for CDG should be extended to all patients with encephalopathy of unknown origin, even in the absence of multisystem involvemen

Cultural engagement and the economic performance of the cultural and creative industries: an occupational critique

This article presents a new critical engagement with the concept of Cultural and Creative Industries (CCIs), focusing on the rationale for grouping occupations and industries under this label. We show how the definition of ‘creativity’ used to demonstrate CCIs’ economic performance remains contested and variable, particularly with regard to the inclusion of specific parts of the IT sector. In demonstrating the importance of IT to the economic narrative regarding CCIs, we then unfold a related critique, exploring patterns in cultural consumption within CCI occupations. We demonstrate how some CCI workers have distinctively high cultural consumption, others reflect their broader social class, and some, including IT workers, show lower than expected consumption. Overall, we question the coherence of the prevailing CCI category, particularly in government policy, and suggest a new mode of ‘cultural’ occupational analysis for the sociology of CCIs

The material role of digital media in connecting with, within, and beyond museums

The connective potentials of digital media have been positioned as a key part of a contemporary museum visitor experience. Using a sociology of translation, we construct a network of visitor experiences using data from a digital media engagement project at a large and multi-sited museum in the United Kingdom. These experiences relate to (dis)connections with the museum, museum objects, and other visitors. Through this analysis we disclose the often contradictory roles of the non-human, including and going beyond the digital, as contributors to the success and failure of attempts to change museum visitor experiences through engagement activities rooted in narratives of participation and connectivity

Exploring contemporary patterns of cultural consumption : offline and online film watching in the UK

This paper focuses on patterns of film consumption within cultural consumption more broadly to assess trends in consumerism such as eclectic consumption, individualised consumption and omnivorous/univorous consumption and whether economic background and status feature in shaping cultural consumption. We focus on film because it is widely consumed, online and offline, and has many genres that vary in terms of perceived artistic and entertainment value. In broad terms, film is differentiated between mainstream commercially driven film such as Hollywood blockbusters, middlebrow ‘feel good’ movies and independent arthouse and foreign language film. Our empirical statistical analysis shows that film consumers watch a wide range of genres. However, films deemed to hold artistic value such as arthouse and foreign language feature as part of broad and wide-ranging pattern of consumption of film that attracts its own dedicated consumers. Though we found that social and economic factors remain predictors of cultural consumption the overall picture is more complex than a simple direct correspondence and perceptions of other cultural forms also play a role. Those likely to consume arthouse and foreign language film consume other film genres and other cultural forms genres and those who ‘prefer’ arthouse and foreign language film have slightly more constrained socio-economic characteristics. Overall, we find that economic and cultural factors such income, education, and wider consumption of culture are significant in patterns of film consumption

Punishment in the Frame: Rethinking the History and Sociology of Art

Images of punishment have featured prominently in Western art and this article explores what might be learnt from studying such pictures of suffering. It seeks to develop an approach to the visual that avoids both the essentialism of art history and the reductionism of sociology by offering a rethinking of the relationships between the two. It begins by setting out the current state of the sociology of art, before discussing ‘new’ art histories that are inspired by social analysis. It then concentrates on how images of punishment have featured in Western art. This substantive material provides a rich resource to understand the force of representation and offers an opportunity to develop an aesthetic sociology that avoids some of the problems identified in the article. The approach developed in the second part is one that seeks to elaborate an aesthetic sociology that combines a historical sensitivity to images with the analytical concerns of social science. It strives to extend the art historian Michael Baxandall’s writings toward more sociological interpretations of visual analysis

The coming crisis of cultural engagement? Measurement, methods, and the nuances of niche activities

Do ticketing data and national survey data on attendance tell the same story? This question is particularly important in the context of debates over the power of new forms of data to supplant the “traditional” survey methods that have underpinned our understanding of the social stratification of culture. This paper compares three data sources on attendance: the Active Lives Survey, the Taking Part Survey, and Audience Finder. We first compare self-reported attendance at events in each English local authority from the Active Lives survey with ticket sales data, finding a close relationship. We follow up by comparing the distributions of ticket buyers across the Indices of Multiple Deprivation with those from Taking Part, finding that for widely-ticketed and widely-attended art forms they track closely together, providing support for existing trends. Ticketing data does not seem to offer more information on social stratification than traditional social science sources. However, we extend the comparison through more detailed analysis of subcategories within less well-researched forms – literature and dance events – where numbers of attendees are lower, with accompanying uncertainty in survey sources. We find that the audiences for dance vary widely, with ballet attendance being heavily socially stratified but attendance at contemporary dance much more similar to the general population. However, we find that audiences for literature events are more heavily socially stratified than almost any other art form, almost regardless of the subcategory. The power of new datasets is in offering specificity about artforms, rather than overturning what we know about culture and inequality

Virtual Touch (TM) Quantification to Diagnose and Monitor Liver Fibrosis in Hepatitis B and Hepatitis C: A NICE Medical Technology Guidance

King’s Technology Evaluation Centre is funded by NICE to act as an External Assessment Centre for the Medical Technologies Evaluation Programme. The NHS has a financial interest in the guidance issued by NICE as a result of this work and two authors are NHS employees (Prof. Keevil and Dr. Lewis

Digital Traces of Distinction? Popular Orientation and User-Engagement with Status Hierarchies in TripAdvisor Reviews of Cultural Organizations

Cultural organizations are categorized by cultural products (high or popular culture) and by organizational form (nonprofit or commercial). In sociology, these classifications are understood predominantly through a Bourdieusian lens, which links cultural consumption to habitus and a class-based struggle for distinction. However, people’s engagement with institutionalized cultural classifications may be expressed differently on the Internet, where a culture of hierarchy-free equality is (sometimes) idealized. Using digital trace data from a representative sample of 280 user-generated reviews of four London cultural organizations, we find that reviewers are concerned with practical issues over cultural content, displaying a popular orientation to cultural consumption (an “audience-focus” or an “embodied” approach). A very small minority of reviewers claim status honor on a variety of bases, including symbolic mastery of traditional cultural capital. Overall, we find an online space in the cultural sphere in which cultural hierarchies are not relevant

Contribution of magnetic resonance imaging to the prenatal diagnosis of common congenital vascular anomalies.

Screening ultrasound (US) has increased the detection of congenital vascular anomalies in utero. Complementary magnetic resonance imaging (MRI) may improve the diagnosis, but its real utility is still not well established. We aimed to describe the imaging findings on prenatal US and MRI of the most frequent congenital vascular anomalies (lymphatic malformations and congenital hemangiomas) to assess the accuracy of prenatal US and MRI exams for diagnosis and to evaluate the relevance of the additional information obtained by complementary fetal MRI. All confirmed postnatal congenital vascular anomalies detected in the last 10 years at 3 university hospitals were retrospectively identified. The prenatal diagnosis was compared with the final diagnosis for both methods and the clinical relevance of additional MRI information was evaluated. A second MRI in advanced pregnancy was performed in fetuses with lesions in a sensitive anatomical location and the clinical relevance of the additional information was evaluated. Twenty-four cases were included in the study, 20 lymphatic malformations and 4 hemangiomas. MRI slightly improved the diagnosis of lymphatic malformation, 85% vs. 80% at US, especially for abdominal lesions. Both methods had a low identification rate (25%) for tumors. MRI performed late in five fetuses with lymphatic malformation allowed optimized management at birth. MRI improves the diagnosis of congenital lymphatic malformations whereas hemangiomas remain difficult to identify in utero. The main role of MRI is to provide high-defined anatomical data to guide management at birth