Cryopyrin-Associated Periodic Syndrome: An Update on Diagnosis and Treatment Response

Cryopyrin-associated periodic syndrome (CAPS) is a rare hereditary inflammatory disorder encompassing a continuum of three phenotypes: familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease. Distinguishing features include cutaneous, neurological, ophthalmologic, and rheumatologic manifestations. CAPS results from a gain-of-function mutation of the NLRP3 gene coding for cryopyrin, which forms intracellular protein complexes known as inflammasomes. Defects of the inflammasomes lead to overproduction of interleukin-1, resulting in inflammatory symptoms seen in CAPS. Diagnosis is often delayed and requires a thorough review of clinical symptoms. Remarkable advances in our understanding of the genetics and the molecular pathway that is responsible for the clinical phenotype of CAPS has led to the development of effective treatments. It also has become clear that the NLRP3 inflammasome plays a critical role in innate immune defense and therefore has wider implications for other inflammatory disease states

Is the meiofauna a good indicator for climate change and anthropogenic impacts?

Our planet is changing, and one of the most pressing challenges facing the scientific community revolves around understanding how ecological communities respond to global changes. From coastal to deep-sea ecosystems, ecologists are exploring new areas of research to find model organisms that help predict the future of life on our planet. Among the different categories of organisms, meiofauna offer several advantages for the study of marine benthic ecosystems. This paper reviews the advances in the study of meiofauna with regard to climate change and anthropogenic impacts. Four taxonomic groups are valuable for predicting global changes: foraminifers (especially calcareous forms), nematodes, copepods and ostracods. Environmental variables are fundamental in the interpretation of meiofaunal patterns and multistressor experiments are more informative than single stressor ones, revealing complex ecological and biological interactions. Global change has a general negative effect on meiofauna, with important consequences on benthic food webs. However, some meiofaunal species can be favoured by the extreme conditions induced by global change, as they can exhibit remarkable physiological adaptations. This review highlights the need to incorporate studies on taxonomy, genetics and function of meiofaunal taxa into global change impact research

Denial of racism and its implications for local action

Literature on modern racism identifies denial as one of its key features. This article examines the discourses of denial that feature in the talk of local anti-racism actors in Australia, and asks what drives these discourses. The research draws on qualitative interviews undertaken with participants involved in local anti-racism in two case study areas, one in South Australia and the other in New South Wales. This article explores the way local participants in the case study areas deployed four discourses to deny or minimise racism: temporal deflections; spatial deflections; deflections from the mainstream; and absence discourses. Place defending and the desire to protect one's local area from being branded a racist space is discussed as a driver of those local denial discourses. Local denial of racism is also linked to national politics of racism and anti-racism. In particular, the Australian government's retreat from multiculturalism, and the preference for 'harmony' rather than 'anti-racism' initiatives, was linked to the avoidance of the language of racism within participants' responses. The way denial discourses narrow the range of possibilities for local anti-racism is discussed, as is the importance of acknowledgement of racism, particularly institutional and systemic racism. Public acknowledgement of these forms of racism will broaden the scope of local anti-racism. © The Author(s) 2013