Development and Evaluation of SENSE-ational Mealtimes: a Book for Families with Mealtime Difficulties

Many families with young children experience mealtime difficulties whereby the child eats a limited range of foods and/or refuses new food. Clinical interventions typically include behaviour training, enhancement of parenting skills and nutrition education. Clinical experience and a review of the literature across several domains suggested that interventions that optimise reflective functioning and understandings about sensory preferences at mealtimes are needed for both mild and complex mealtime difficulties. This study describes the development of the SENSE-ational Mealtimes book for families with mealtime difficulties and reports the findings of the initial evaluation. A questionnaire was used to assess the change in the frequency of difficult mealtimes, level of concern, understandings, feelings and goals of mothers 2 months after the book was distributed in a community setting. Mothers also provided feedback regarding helpfulness of the book, needs of families and recommendations. There was a statistically significant improvement in all aspects, namely frequency of mealtime difficulties, level of concern, understandings, feelings and goals. The subjective data indicated that the concepts instrumental in enhancing most mothers' understandings were how sensory preferences and past experiences of all members of the family had an impact on mealtime interactions. Initial evaluation suggests that wide-spread access to the SENSE-ational Mealtimes book could be an inexpensive approach to reduce the costs of adverse effects of mealtime difficulties on the emotional well-being of families and dietary intake of children. Mothers unanimously recommended the SENSE-ational Mealtimes book for both targeted prevention of and early intervention with mealtime difficulties in families

Atypical hemolytic uremic syndrome in children: complement mutations and clinical characteristics

Item does not contain fulltextBACKGROUND: Mutations in complement factor H (CFH), factor I (CFI), factor B (CFB), thrombomodulin (THBD), C3 and membrane cofactor protein (MCP), and autoantibodies against factor H (alphaFH) with or without a homozygous deletion in CFH-related protein 1 and 3 (CFHR1/3) predispose development of atypical hemolytic uremic syndrome (aHUS). METHODS: Different mutations in genes encoding complement proteins in 45 pediatric aHUS patients were retrospectively linked with clinical features, treatment, and outcome. RESULTS: In 47% of the study participants, potentially pathogenic genetic anomalies were found (5xCFH, 4xMCP, and 4xC3, 3xCFI, 2xCFB, 6xalphaFH, of which five had CFHR1/3); four patients carried combined genetic defects or a mutation, together with alphaFH. In the majority (87%), disease onset was preceeded by a triggering event; in 25% of cases diarrhea was the presenting symptom. More than 50% had normal serum C3 levels at presentation. Relapses were seen in half of the patients, and there was renal graft failure in all except one case following transplant. CONCLUSIONS: Performing adequate DNA analysis is essential for treatment and positive outcome in children with aHUS. The impact of intensive initial therapy and renal replacement therapy, as well as the high risk of recurrence of aHUS in renal transplant, warrants further understanding of the pathogenesis, which will lead to better treatment options.01 augustus 201

Advances in our understanding of the pathogenesis of glomerular thrombotic microangiopathy

Glomerular thrombotic microangiopathy is a hallmark feature of haemolytic uraemic syndrome, the leading cause of acute renal failure in childhood. This paper is a review of the different mechanistic pathways that lead to this histological picture in the kidney. It will focus on atypical HUS and complement dysregulation, but will also highlight some other recent advances in our understanding of this condition, including the potential role of the molecule vascular endothelial growth factor- A (VEGF-A)

Genetics and complement in atypical HUS

Central to the pathogenesis of atypical hemolytic uremic syndrome (aHUS) is over-activation of the alternative pathway of complement. Following the initial discovery of mutations in the complement regulatory protein, factor H, mutations have been described in factor I, membrane cofactor protein and thrombomodulin, which also result in decreased complement regulation. Autoantibodies to factor H have also been reported to impair complement regulation in aHUS. More recently, gain of function mutations in the complement components C3 and Factor B have been seen. This review focuses on the genetic causes of aHUS, their functional consequences, and clinical effect

aHUS caused by complement dysregulation: new therapies on the horizon

Atypical hemolytic uremic syndrome (aHUS) is a heterogeneous disease that is caused by defective complement regulation in over 50% of cases. Mutations have been identified in genes encoding both complement regulators [complement factor H (CFH), complement factor I (CFI), complement factor H-related proteins (CFHR), and membrane cofactor protein (MCP)], as well as complement activators [complement factor B (CFB) and C3]. More recently, mutations have also been identified in thrombomodulin (THBD), an anticoagulant glycoprotein that plays a role in the inactivation of C3a and C5a. Inhibitory autoantibodies to CFH account for an additional 5–10% of cases and can occur in isolation or in association with mutations in CFH, CFI, CFHR 1, 3, 4, and MCP. Plasma therapies are considered the mainstay of therapy in aHUS secondary to defective complement regulation and may be administered as plasma infusions or plasma exchange. However, in certain cases, despite initiation of plasma therapy, renal function continues to deteriorate with progression to end-stage renal disease and renal transplantation. Recently, eculizumab, a humanized monoclonal antibody against C5, has been described as an effective therapeutic strategy in the management of refractory aHUS that has failed to respond to plasma therapy. Clinical trials are now underway to further evaluate the efficacy of eculizumab in the management of both plasma-sensitive and plasma-resistant aHUS

Seeing versus Doing: How Businesses Manage Tensions in Pursuit of Sustainability

Management of organizational tensions can facilitate the simultaneous advancement of economic, social, and environmental priorities. The approach is based on managers identifying and managing tensions between the three priorities, by employing one of the three strategic responses. Although recent work has provided a theoretical basis for such tension acknowledgment and management, there is a dearth of empirical studies. We interviewed 32 corporate sustainability managers across 25 forestry and wood-products organizations in Australia. Study participants were divided into two groups: (1) those considered effective at corporate sustainability and (2) a status-quo group. Contrary to current theory, our findings showed that acknowledgment of organizational tensions was widespread in the Australian forestry and wood-products industry and not limited to those managers who are effective at managing corporate sustainability. What differed was the degree to which managers did something about the perceived tensions—with the effective group more consistently acting to manage and resolve paradoxical scenarios. Our findings suggest that existing theoretical constructs of tension management may not adequately capture the individual-level complexity involved with managing tensions in practice

Anarchist education and the paradox of pedagogical authority

This paper interrogates a key feature of anarchist education; focusing on a problem with implications not only for anarchist conceptions of education, but for anarchist philosophy and practice more broadly. The problem is this: if anarchism consists in the principled opposition to all forms of coercive authority, then how is this to be reconciled with situations where justice demands the use of coercion in order to protect some particular good? It seems that anarchist educators are forced to deny coercive authority in principle, whilst at the same time affirming it in practice. This is the paradox of pedagogical authority in anarchist education. Coercive authority is simultaneously impossible and indispensable. Exploring this paradox through a reading of Jacques Derrida’s later work, and, in particular, his conception of justice as requiring openness to the singular situation (Derrida, 1990), I argue that in exercising their authority anarchist educators encounter the aporetic moment in anarchism, experiencing what Derrida calls ‘the ordeal of the undecidable’ (Ibid.). Understood this way, the paradox becomes less an indication of anarchism’s limitations than it does its value. For it is here that the problem of pedagogical authority is treated with the gravity that all questions of justice deserve