Story in health and social care

This paper offers a brief consideration of how narrative, in the form of people‟s own stories, potentially figures in health and social care provision as part of the impulse towards patient-centred care. The rise of the epistemological legitimacy of patients‟ stories is sketched here. The paper draws upon relevant literature and original writing to consider the ways in which stories can mislead as well as illuminate the process of making individual treatment care plans

Impact of long-term elosulfase alfa treatment on clinical and patient-reported outcomes in patients with mucopolysaccharidosis type IVA: results from a Managed Access Agreement in England

BACKGROUND: We present baseline characteristics and follow-up data of a Managed Access Agreement (MAA), including patients with mucopolysaccharidosis IVA (MPS IVA) receiving elosulfase alfa enzyme replacement therapy (ERT) in England on a conditional basis. Patients enrolled in the MAA programme are reviewed on an annual basis. Therapy can be continued if patients are compliant, able to tolerate infusions, and meet four out of five pre-defined clinical and patient-reported outcomes (PRO) criteria. Baseline and follow-up clinical and PRO data are presented for all participants who completed ≥ 1 year of assessments in the MAA. RESULTS: The analysis included data from 55 patients, including 26 patients previously enrolled in clinical trials and 29 who started ERT after enrolling in the MAA. In patients with both baseline and follow-up data, mean 6-min walk test distance increased from 217 m at baseline to 244 m after a mean follow-up of 4.9 years. Improvement or stabilisation was seen regardless of age at treatment initiation or duration of treatment. Mean forced vital capacity and forced expiratory volume in 1 s were 0.87 L and 0.78 L, respectively at baseline and 1.05 L and 0.88 L after a mean follow-up of 5.5 years. PRO data showed overall improvements over time in Mobility, Self-care, and Caregiver assistance scores of the MPS-Health Assessment Questionnaire, relatively stable quality of life, and some improvements in pain scores. CONCLUSIONS: The MAA data confirm the effects of elosulfase alfa on clinical and PRO results observed in the clinical trials and provide real-world evidence for long-term stabilisation in these measures, suggesting a positive impact on the natural history of MPS IVA

Comparison of sapwood invasion by three Phytophthora spp.in different hosts

Many Phytophthora spp. have recently been isolated from native vegetation in Western Australia. As their pathogenicity is often unknown, it is not possible to provide advice to land managers on the impact of site infestation on native plants and how these infestations should be managed. We describe a rapid screening method based on sapwood invasion that has been used to compare the pathogenicity of Phytophthora arenaria, P. cinnamomi and P. multivora. Radial invasion into the xylem of six banksias and three eucalypts was assessed in an excised branch assay in summer and winter. Branches were wound inoculated and invasion was assessed by plating from a strip of tissue cut across the stem at the inoculation point and at 40 mm above and below. A symptomless infection had established in both the bark and sapwood within 6 days. P. arenaria was only isolated from the strip of tissue at the inoculation point. P. cinnamomi was isolated from the sapwood of Banksia attenuata, B. burdettii, B. menziesii and B. speciosa 40 mm above or below the inoculation point in some experiments. P. multivora was isolated from B. speciosa 40 mm below the inoculation point in one experiment. Hyphae of both species were seen in both ray parenchyma cells and xylem vessels. The invasiveness of the Phytophthora spp. was compared on the two groups of hosts using scores for sapwood invasion at the inoculation point. For banksias, P. cinnamomi and P. multivora had significantly higher invasion scores on banksias than P. arenaria but were not significantly different to one another. There was no significant difference between the three Phytophthora spp. on the eucalypt hosts. Assessing sapwood invasion provides a rapid, inexpensive and biologically meaningful way of screening the many Phytophthora spp. that have been isolated from native vegetation

Exploring Profitable, Sustainable Livestock Businesses in an Increasingly Variable Climate

Livestock production systems will need to change in many regions of the world to meet the dual challenges of adaptation to a changing climate and reducing net greenhouse gas (GHG) emissions. The ‘Nexus project’ is exploring these options using seven case studies in eastern Australia spanning a range of production systems from semi-arid beef production in northern Australia to mixed beef and sheep production in cool temperate climates of southern Australia. Regional reference groups, made up of 4-6 local farmers and advisers, identified a series of farm systems changes that could be made to meet these challenges. Options were categorised under four themes: feedbase, animal genetics and management, management and technology, and transformational options. More options for adaptation were identified compared to options to mitigate greenhouse gas emission of sequestered carbon. Adaptation options focussed on maximising pasture production in variable climates, and having efficient and flexible animal production systems. A focus on infrastructure for water and feed storage and use was also observed. Options to reduce GHG emissions focussed on reducing feed demand and sequestering carbon in soils and trees, and limited possibilities for feed additives were noted in these pasture-based systems. Further work will explore how combinations of these options will impact production, profit and greenhouse gas emissions from these production systems in current and future climate scenarios

Presynaptic partner selection during retinal circuit reassembly varies with timing of neuronal regeneration in vivo

Whether neurons can restore their original connectivity patterns during circuit repair is unclear. Taking advantage of the regenerative capacity of zebrafish retina, we show here the remarkable specificity by which surviving neurons reassemble their connectivity upon regeneration of their major input. H3 horizontal cells (HCs) normally avoid red and green cones, and prefer ultraviolet over blue cones. Upon ablation of the major (ultraviolet) input, H3 HCs do not immediately increase connectivity with other cone types. Instead, H3 dendrites retract and re-extend to contact new ultraviolet cones. But, if regeneration is delayed or absent, blue-cone synaptogenesis increases and ectopic synapses are made with red and green cones. Thus, cues directing synapse specificity can be maintained following input loss, but only within a limited time period. Further, we postulate that signals from the major input that shape the H3 HC's wiring pattern during development persist to restrict miswiring after damage

Immunohistochemical localization of fibronectin as a tool for the age determination of human skin wounds

We analyzed the distribution of fibronectin in routinely embedded tissue specimens from 53 skin wounds and 6 postmortem wounds. In postmortem wounds a faint but focal positive staining was exclusively found at the margin of the specimens which dit not extend into the adjacent stroma. Vital wounds were classified into 3 groups. The first comprising lesions with wound ages ranging from a few seconds to 30 min, the second comprising those with wound ages upt to 3 weeks, and the third group with lesions more than 3 weeks old. Ten out of 17 lesions with a wound age up to 30 min showed a clear positive reaction within the wound area. Three specimens in this group were completely negative, while in 4 additional cases the result was not significantly different from postmortem lesions. These 7 cases were characterized by acute death with extremely short survival times (only seconds). In wounds up to 3 weeks old fibronectin formed a distinct network containing an increasing number of inflammatory cells corresponding to the wound age. In 2 cases with a survival time of 17 days and in all wounds older than 3 weeks fibronectin was restricted to the surface of fibroblasts and to parallel arranged fibers in the granulation tissue without any network structures. We present evidence that fibronectin is a useful marker for vital wounds with a survival time of more than a few minutes. Fibronectin appears before neutrophilic granulocytes migrate into the wound area. Since a faint positive fibronectin staining is seen in postmortem lesions and bleedings, we propose that only those wounds which show strong positive fibronectin staining also extending into the adjacent stroma should be regarded as vital

Carbon myopia: the urgent need for integrated social, economic and environmental action in the livestock sector

Livestock have long been integral to food production systems, often not by choice but by need. While our knowledge of livestock greenhouse gas emissions (GHG) mitigation has evolved, the prevailing focus has been - somewhat myopically on technology applications associated with mitigation.Here, we (1) examine the global distribution of livestock GHG emissions, (2) explore social, economic and environmental co-benefits and trade-offs associated with mitigation interventions, and (3) critique approaches for quantifying GHG emissions.This review uncovered many insights. First, while GHG emissions from ruminant livestock are greatest in low and middle-income countries (LMIC; globally, 66% of emissions are produced by Latin America and the Caribbean, East and southeast Asia, and south Asia), the majority of mitigation strategies are designed for developed countries. This serious concern is heightened by the fact that 80% of growth in global meat production over the next decade will occur in LMIC. Second, few studies concurrently assess social, economic and environmental aspects of mitigation. Of the 54 interventions reviewed, only 16 had triple-bottom line benefit with medium-high mitigation potential. Third, while efforts designed to stimulate adoption of strategies allowing both emissions reduction (ER) and carbon sequestration (CS) would achieve the greatest net emissions mitigation, CS measures have greater potential mitigation and co-benefits.The scientific community must shift attention away from the prevailing myopic lens on carbon, towards more holistic, systems-based, multi-metric approaches that carefully consider the raison d'être for livestock systems. Consequential life-cycle assessments and systems-aligned socio-economic planetary boundaries offer useful starting points that may uncover leverage points and cross-scale emergent properties. Derivation of harmonised, globally-reconciled sustainability metrics requires iterative dialogue between stakeholders at all levels. Greater emphasis on the simultaneous characterisation of multiple sustainability dimensions would help avoid situations where progress made in one area causes maladaptive outcomes in other areas

Nuclear reprocessing-related radiocarbon (¹⁴C) uptake in UK marine mammals

To evaluate the transfer of Sellafield-derived radiocarbon (14C) to top predators in the UK marine environment, 14C activities were examined in stranded marine mammals. All samples of harbour porpoise (Phocoena phocoena) obtained from the Irish Sea showed 14C enrichment above background. Mammal samples obtained from the West of Scotland, including harbour porpoise, grey seals (Halichoerus grypus) and harbour seals (Phoca vitulina) showed 14C enrichment but to a lesser extent. This study demonstrates, for the first time, enriched 14C is transferred through the marine food web to apex predators as a consequence of ongoing nuclear reprocessing activities at Sellafield. Total Sellafield 14C discharge activity 24 months prior to stranding and, in particular, distance of animal stranding site from Sellafield are significant variables affecting individual 14C activity. 14C activities of West of Scotland harbour porpoises suggest they did not forage in the Irish Sea prior to stranding, indicating a high foraging fidelity

Expanding the phenotype in argininosuccinic aciduria: need for new therapies

OBJECTIVES: This UK-wide study defines the natural history of argininosuccinic aciduria and compares long-term neurological outcomes in patients presenting clinically or treated prospectively from birth with ammonia-lowering drugs. METHODS: Retrospective analysis of medical records prior to March 2013, then prospective analysis until December 2015. Blinded review of brain MRIs. ASL genotyping. RESULTS: Fifty-six patients were defined as early-onset (n = 23) if symptomatic < 28 days of age, late-onset (n = 23) if symptomatic later, or selectively screened perinatally due to a familial proband (n = 10). The median follow-up was 12.4 years (range 0-53). Long-term outcomes in all groups showed a similar neurological phenotype including developmental delay (48/52), epilepsy (24/52), ataxia (9/52), myopathy-like symptoms (6/52) and abnormal neuroimaging (12/21). Neuroimaging findings included parenchymal infarcts (4/21), focal white matter hyperintensity (4/21), cortical or cerebral atrophy (4/21), nodular heterotopia (2/21) and reduced creatine levels in white matter (4/4). 4/21 adult patients went to mainstream school without the need of additional educational support and 1/21 lives independently. Early-onset patients had more severe involvement of visceral organs including liver, kidney and gut. All early-onset and half of late-onset patients presented with hyperammonaemia. Screened patients had normal ammonia at birth and received treatment preventing severe hyperammonaemia. ASL was sequenced (n = 19) and 20 mutations were found. Plasma argininosuccinate was higher in early-onset compared to late-onset patients. CONCLUSIONS: Our study further defines the natural history of argininosuccinic aciduria and genotype-phenotype correlations. The neurological phenotype does not correlate with the severity of hyperammonaemia and plasma argininosuccinic acid levels. The disturbance in nitric oxide synthesis may be a contributor to the neurological disease. Clinical trials providing nitric oxide to the brain merit consideration