Impact of SARS-CoV-2 (COVID-19) pandemic on patients with lysosomal storage disorders and restoration of services: experience from a specialist centre

This study aims to evaluate the impact of the COVID-19 pandemic on the lysosomal disorders unit (LSDU) at Royal Free London NHS Foundation Trust (RFL), a highly specialised national service for diagnosis and management of adults with lysosomal storage disorders (LSD). Review of home care enzyme replacement therapy (ERT) and emergency care, and COVID-19 shielding categories as per UK government guidance. New clinical pathways were developed to manage patients safely during the pandemic; staff well-being initiatives are described. LSDU staff were redeployed and/or had additional roles to support increased needs of hospitalised COVID-19 patients. During the first lockdown in March 2020, 286 of 602 LSD patients were shielding; 72 of 221 had home care ERT infusions interrupted up to 12 weeks. During the pandemic, there was a 3% reduction in home care nursing support required, with patients learning to self-cannulate or require support for cannulation only. There were no increased adverse clinical events during this period. Twenty-one contracted COVID-19 infection, with one hospitalised and no COVID-19 related deaths. In 2020, virtual clinics were increased by 88% (video and/or telephone) compared to 2019. RFL well-being initiatives supported all staff. We provide an overview of the impact of the COVID-19 pandemic on staff and patients attending a highly specialised rare disease service. As far as we are aware, this is the first detailed narrative on the challenges and subsequent rapid adaptations made, both as part of a large organisation and as a specialist centre. Lessons learnt could be translated to other rare disease services and ensure readiness for any future pandemic

Identification of Novel Cetacean Poxviruses in Cetaceans Stranded in South West England

Poxvirus infections in marine mammals have been mainly reported through their clinical lesions and electron microscopy (EM). Poxvirus particles in association with such lesions have been demonstrated by EM and were previously classified as two new viruses, cetacean poxvirus 1 (CePV-1) and cetacean poxvirus 2 (CePV-2). In this study, epidermal pox lesions in cetaceans stranded in South West England (Cornwall) between 2008 and 2012 were investigated by electron microscopy and molecular analysis. PCR and sequencing of a highly conserved region within the viral DNA polymerase gene ruled out both parapoxand orthopoxviruses. Moreover, phylogenetic analysis of the PCR product clustered the sequences with those previously described as cetacean poxviruses. However, taking the close genetic distance of this gene fragment across the family of poxviridae into account, it is reasonable to postulate further, novel cetacean poxvirus species. The nucleotide similarity within each cluster (tentative species) detected ranged from 98.6% to 100%, whilst the similarity between the clusters was no more than 95%. The detection of several species of poxvirus in different cetacean species confirms the likelihood of a heterogeneous cetacean poxvirus genus, comparable to the heterogeneity observed in other poxvirus genera

High-Pressure Structural Systematics in Neodymium to 302 GPa

Angle-dispersive x-ray powder diffraction experiments have been performed on neodymium metal to a pressure of 302 GPa. Up to 70 GPa we observe the $hP4 → cF4 → hR24 → oI16 → hP3$ transition sequence reported previously. At 71(2) GPa we find a transition to a phase which has an orthorhombic structure (oF8) with eight atoms in the unit cell, space group Fddd. This structure is the same as that recently observed in samarium above 93 GPa, and is isostructural with high-pressure structures found in the actinides Am, Cf, and Cm. We see a further phase transition at 98(1) GPa to a phase with the orthorhombic α-U (oC4) structure, which remains stable up to 302 GPa, the highest pressure reached in this study. Electronic structure calculations find the same structural sequence, with calculated transition pressures of 66 and 88 GPa, respectively, for the $hP3 → F8$ and $oF8 → oC4$ transitions. The calculations further predict that oC4-Nd loses its magnetism at 100 GPa, in agreement with previous experimental results, and it is the accompanying decrease in enthalpy and volume that results in the transition to this phase. Comparison calculations on the oF8 and oC4 phases of Sm show that they both retain their magnetism to at least 240 GPa, with the result that oC4-Sm is calculated to have the lowest enthalpy over a narrow pressure region near 200 GPa at 0 K

The Capaciousness of No: Affective Refusals as Literacy Practices

© 2020 The Authors. Reading Research Quarterly published by Wiley Periodicals, Inc. on behalf of International Literacy Association The authors considered the capacious feeling that emerges from saying no to literacy practices, and the affective potential of saying no as a literacy practice. The authors highlight the affective possibilities of saying no to normative understandings of literacy, thinking with a series of vignettes in which children, young people, and teachers refused literacy practices in different ways. The authors use the term capacious to signal possibilities that are as yet unthought: a sense of broadening and opening out through enacting no. The authors examined how attention to affect ruptures humanist logics that inform normative approaches to literacy. Through attention to nonconscious, noncognitive, and transindividual bodily forces and capacities, affect deprivileges the human as the sole agent in an interaction, thus disrupting measurements of who counts as a literate subject and what counts as a literacy event. No is an affective moment. It can signal a pushback, an absence, or a silence. As a theoretical and methodological way of thinking/feeling with literacy, affect proposes problems rather than solutions, countering solution-focused research in which the resistance is to be overcome, co-opted, or solved. Affect operates as a crack or a chink, a tiny ripple, a barely perceivable gesture, that can persist and, in doing so, hold open the possibility for alternative futures

Understanding Anthropological Understanding: for a merological anthropology

In this paper I argue for a merological anthropology in which ideas of ‘partiality’ and ‘practical adequacy’ provide a way out of the impasse of relativism which is implied by post-modernism and the related abandonment of a concern with ‘truth’. Ideas such as ‘aptness’ and ‘faithfulness’ enable us to re-establish empirical foundations without having to espouse a simple realism which has been rightly criticised. Ideas taken from ethnomethodology, particularly the way we bootstrap from ‘practical adequacy’ to ‘warrants for confidence’ point to a merological anthropology in which we recognize that we do not and cannot know everything, but that we can have reasons for being confident in the little we know

Structural phase transitions in yttrium up to 183 GPa

Angle-dispersive x-ray powder diffraction experiments have been performed on yttrium metal up to 183 GPa.We find that the recently discoveredoF16 structure observed in the high-Ztrivalent lanthanides is also adoptedby yttrium above 106 GPa, pressures where it has a superconducting temperature of∼20 K. We have also refinedboth tetragonal and rhombohedral structures against the diffraction data from the preceding “distorted-fcc” phaseand we are unable to state categorically which of these is the true structure of this phase. Finally, analysis ofyttrium’s equation of state reveals a marked change in the compressibility upon adoption of theoF16 structure,after which the compression is that of a “regular” metal. Electronic structure calculations ofoF16-Y confirm itsstability overoF8 structure seen in Nd and Sm, and provide insight into the nature of the shift of orbital characterfromstodunder compression