Neuroblastoma: The Clinical Aspects

Neuroblastoma is a predominantly pediatric cancer, arising from the primordial neural crest cells that form the sympathetic nervous system. The prognosis for patients with neuroblastoma can vary from uniform survival in low risk patients to fatality in patients with high risk disease. This chapter gives a brief overview of the epidemiology, genetics, clinical presentation, diagnosis, and discussion of the various staging systems and risk classifications of neuroblastoma. We also briefly describe our understanding of the conventional and novel treatment modalities available and their effects on the current prognosis of patients with neuroblastoma. The purpose of this chapter is to serve as a brief overview of the clinical aspects of neuroblastoma, to serve as a foundation of knowledge for scientists aspiring to develop new therapeutic modalities for this dreadful pediatric disease

Can We Retain the Economy-Wide Benefits of Energy Efficiency While Reducing the Energy Rebound?

Economy-wide rebound is often presented as a necessary ‘evil’ accompanying economic expansion triggered by energy efficiency improvements. We challenge this position in two, inter-related ways. First, we question the emphasis on potential technical energy savings and losses due to rebound in energy efficiency policy evaluation. This abstracts from the wider economic and societal impacts of energy efficiency improvements that are often positive and valuable to policy makers. Second, we propose that economic expansion and economy-wide rebound need not be highly correlated. We argue that energy efficiency actions targeted at improving the competitiveness of less energy-intensive means of providing services, such as heat and transport, may provide opportunities to boost economic activity while minimising rebound effects. This perspective involves a change in current policy and research thinking, particularly in terms of the type of substitution possibilities that we should focus on in enhancing energy efficiency, economic expansion and rebound relations

Decarbonising domestic heating : the need for policy to help consumers respond to the benefits of technological progress change in electricity supply?

The UK Government’s acceptance of the Committee on Climate Change (CCC) advice on moving to a net zero carbon economy by 2050 makes the need to decarbonise how we heat our homes of even more pressing and strategic importance. Whatever the mix of solutions that is ultimately adopted, increased electrification of our energy system and economy is inevitable

Can the composition of energy use in an expanding economy be altered by consumers' responses to technological change?

Technological change is necessary for economies to grow and develop. This paper investigates how this technological change could be directed in order to simultaneously reduce carbon-intensive energy use and deliver a range of economic benefits. Using both partial and general equilibrium modelling we consider improvements in the efficiency in the delivery of electricity as an increasingly low carbon option in the UK. We demonstrate how linking this to policy action to assist and encourage households to substitute away from more carbon-intensive gas- to electricity-powered heating systems may change the composition of energy use, and implied emissions intensity, but not the level of the resulting economic expansion

Observational Evidence of For-Profit Delivery and Inferior Nursing Home Care: When Is There Enough Evidence for Policy Change?

This research was financially supported by the Social Sciences and Humanities Research Council

Medico-Legal Findings, Legal Case Progression, and Outcomes in South African Rape Cases: Retrospective Review

Rachel Jewkes and colleagues examine the processing of rape cases by South African police and courts and show an association between documentation of ano-genital injuries, trials commencing, and convictions in rape cases

Inherited germline TP53 mutation encodes a protein with an aberrant C-terminal motif in a case of pediatric adrenocortical tumor

Childhood adrenocortical tumor (ACT), a very rare malignancy, has an annual worldwide incidence of about 0.3 per million children younger than 15 years. The association between inherited germline mutations of the TP53 gene and an increased predisposition to ACT was described in the context of the Li-Fraumeni syndrome. In fact, about two-thirds of children with ACT have a TP53 mutation. However, less than 10% of pediatric ACT cases occur in Li-Fraumeni syndrome, suggesting that inherited low-penetrance TP53 mutations play an important role in pediatric adrenal cortex tumorigenesis. We identified a novel inherited germline TP53 mutation affecting the acceptor splice site at intron 10 in a child with an ACT and no family history of cancer. The lack of family history of cancer and previous information about the carcinogenic potential of the mutation led us to further characterize it. Bioinformatics analysis showed that the non-natural and highly hydrophobic C-terminal segment of the frame-shifted mutant p53 protein may disrupt its tumor suppressor function by causing misfolding and aggregation. Our findings highlight the clinical and genetic counseling dilemmas that arise when an inherited TP53 mutation is found in a child with ACT without relatives with Li-Fraumeni-component tumors