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- Lilleker, JB, et al.
(författare)
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Cytosolic 5'-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis
- 2017
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Ingår i: Annals of the rheumatic diseases. - : BMJ. - 1468-2060 .- 0003-4967. ; 76:5, s. 862-868
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Tidskriftsartikel (refereegranskat)abstract
- Autoantibodies directed against cytosolic 5′-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5′-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis.Materials and methodsData from various European inclusion body myositis registries were pooled. Anticytosolic 5′-nucleotidase 1A status was determined by an established ELISA technique. Cases were stratified according to antibody status and comparisons made. Survival and mobility aid requirement analyses were performed using Kaplan-Meier curves and Cox proportional hazards regression.ResultsData from 311 patients were available for analysis; 102 (33%) had anticytosolic 5′-nucleotidase 1A antibodies. Antibody-positive patients had a higher adjusted mortality risk (HR 1.89, 95% CI 1.11 to 3.21, p=0.019), lower frequency of proximal upper limb weakness at disease onset (8% vs 23%, adjusted OR 0.29, 95% CI 0.12 to 0.68, p=0.005) and an increased prevalence of excess of cytochrome oxidase deficient fibres on muscle biopsy analysis (87% vs 72%, adjusted OR 2.80, 95% CI 1.17 to 6.66, p=0.020), compared with antibody-negative patients.InterpretationDifferences were observed in clinical and histopathological features between anticytosolic 5′-nucleotidase 1A antibody positive and negative patients with inclusion body myositis, and antibody-positive patients had a higher adjusted mortality risk. Stratification of inclusion body myositis by anticytosolic 5′-nucleotidase 1A antibody status may be useful, potentially highlighting a distinct inclusion body myositis subtype with a more severe phenotype.
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| 12. |
- Pinty, B, et al.
(författare)
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An Operational Anthropogenic CO₂ Emissions Monitoring & Verification Support Capacity : Needs and High Level Requirements for in situ Measurements
- 2019
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Rapport (övrigt vetenskapligt/konstnärligt)abstract
- This is the third report form the CO2 Monitoring Task Force on the multiple input streams of in-situ observations that are requirement for the Copernicus CO2 Monitoring and Verification Support capacity to: (i) calibrated and validate the space component, (ii) assimilate data in the models and integrate information in the core of the system, and (iii) evaluate the output generated by the system for its end users. The availability of sustained in situ networks is currently a significant factor of risk that needs to be mitigated to establish a European CO 2 support capacity which is fit-for-purpose. The current status of existing networks may be the source of large uncertainties in anthropogenicCO2 emission estimates as well as limited capability in meeting the requirements for country, large city and point source scale assessments. This conclusion results from a risk analysis formulated for four scenarios: 1) maintaining the status quo, 2) assuring sustained funding for the status quo, 3) enhancing network capabilities at European scale with sustained funding and 4) with a significantly improved in situ infrastructure in Europe and beyond. This report substantiates the multifaceted needs and requirements of the European CO2 support capacity with respect to in situ observations. The analysis concerns all core elements of the envisaged integrated system with a particular attention on the impact of such observations in achieving the proposed objectives. The specific needs for the validation of products delivered by the space component that is, the Copernicus Sentinels CO2 monitoring constellation, are addressed as another prerequisite for the success of the CO2 support capacity. This European asset will represent a significant contribution to the virtual constellation proposed by the Committee on Earth Observation Satellites (CEOS) and, accordingly, complementary requirements are elaborated in that international frame.The report highlights that although high measurement standards are present within existing networks such as ICOS, in the context of the needs for targeted in situ data for the realization of the operational system, these data are not fully fit-for-purpose. A fundamental prerequisite is to have a good geographical coverage over Europe for evaluating the data assimilation and modeling system over a large variety of environmental conditions such as, for instance, urban areas, agricultural regions, forested zones and industrial complexes. The in situ observations need to be extended under a coordinated European lead with sustained infrastructure and targeted additional and maintained long-term funding.It has been clearly understood from the onset that the international dimension of the European CO2 support capacity would be critical and that these aspects should be developed in parallel to, and in synergy with the definition and implementation of a European contributing system. It was also understood that this international dimension had both strategic, policy relevant and technical dimensions and the Commission and the relevant European institutional partners have started since several years to engage both bilaterally and multilaterally with the relevant stakeholders and counterparts to develop these relations. Specifically, CEOS will undertake, over the next few years,dedicated preparatory work in a coordinated international context, to provide cumulative added value to the specific programmatic activities of their member agencies. Concerted efforts have already begun in the context of the European Commission's Chairmanship of CEOS in 2018. It is recognized in the context of the European efforts, and increasingly by our international counterparts that a broad and holistic system approach is required to address the requirements which are represented by the climate policy, of which the satellite component, whilst important, cannot effectively be developed in isolation. This system indeed includes the satellite observing capability but in addition, the required modelling component and data integration elements, prior information, ancillary data and in situ observations delivered by essential networks.Acknowledging the need for an efficient coordination at international level for instance via the Global Atmosphere Watch programme of the World Meteorological Organisation is a key towards a successful implementation of appropriate actions to ensure the sustainability of essential networks, to enhance current network capabilities with new observations and to propose adequate governance schemes. Such actions to mitigate current network limitations are deemed critical to implementing the Copernicus CO 2 Monitoring & Verification Support capacity in its full strength.
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