"Being in a Knowledge Space": information behaviour of cult media fan communities

This article describes the first two parts of a three-stage study investigating the information behaviour of fans and fan communities, focusing on fans of cult media. A literature analysis shows that information practices are an inherent and major part of fan activities, and that fans are practitioners of new forms of information consumption and production, showing sophisticated activities of information organisation and dissemination. A subsequent Delphi study, taking the novel form of a 'serious leisure' Delphi, in which the participants are not experts in the usual sense, identifies three aspects of fan information behaviour of particular interest beyond the fan context: information gatekeeping; classifying and tagging; and entrepreneurship and economic activity

Pediatric ventricular assist device use as a bridge to transplantation does not affect long-term quality of life

ObjectiveThe present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL.MethodsThe present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated.ResultsAt follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the survey's Core Module, nor were any differences found in the outcomes assessed using the survey's Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores.ConclusionsOver the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation

Pediatric Hypertrophic Cardiomyopathy: Exploring the Genotype‐Phenotype Association

Pediatric hypertrophic cardiomyopathy (HCM) is the most common form of cardiomyopathy in children and a leading cause of sudden cardiac death. Yet, the association between genotype variation, phenotype expression, and adverse events in pediatric HCM has not been fully elucidated. Although the literature on this topic is evolving in adult HCM, the evidence in children is lacking. Solidifying our understanding of this relationship could improve risk stratification as well as improve our comprehension of the underlying pathophysiological characteristics of pediatric HCM. In this state‐of‐the‐art review, we examine the current literature on genetic variations in HCM and their association with outcomes in children, discuss the current approaches to identifying cardiovascular phenotypes in pediatric HCM, and explore possible avenues that could improve sudden cardiac death risk assessment