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Sökning: WFRF:(Will Stefan)

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1.
  • Richardson, Katherine, et al. (författare)
  • Earth beyond six of nine planetary boundaries
  • 2023
  • Ingår i: Science Advances. - 2375-2548. ; 9:37
  • Tidskriftsartikel (refereegranskat)abstract
    • This planetary boundaries framework update finds that six of the nine boundaries are transgressed, suggesting that Earth is now well outside of the safe operating space for humanity. Ocean acidification is close to being breached, while aerosol loading regionally exceeds the boundary. Stratospheric ozone levels have slightly recovered. The transgression level has increased for all boundaries earlier identified as overstepped. As primary production drives Earth system biosphere functions, human appropriation of net primary production is proposed as a control variable for functional biosphere integrity. This boundary is also transgressed. Earth system modeling of different levels of the transgression of the climate and land system change boundaries illustrates that these anthropogenic impacts on Earth system must be considered in a systemic context.
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2.
  • Budde, U, et al. (författare)
  • Detailed von Willebrand factor multimer analysis in patients with von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (MCMDM-1VWD)
  • 2008
  • Ingår i: Journal of Thrombosis and Haemostasis. - : Elsevier BV. - 1538-7933 .- 1538-7836. ; 6:5, s. 762-771
  • Tidskriftsartikel (refereegranskat)abstract
    • Background: Type 1 von Willebrand disease (VWD) is a congenital bleeding disorder characterized by a partial quantitative deficiency of plasma von Willebrand factor (VWF) in the absence of structural and/or functional VWF defects. Accurate assessment of the quantity and quality of plasma VWF is difficult but is a prerequisite for correct classification. Objective: To evaluate the proportion of misclassification of patients historically diagnosed with type 1 VWD using detailed analysis of the VWF multimer structure. Patients and methods: Previously diagnosed type 1 VWD families and healthy controls were recruited by 12 expert centers in nine European countries. Phenotypic characterization comprised plasma VWF parameters and multimer analysis using low- and intermediate-resolution gels combined with an optimized visualization system. VWF genotyping was performed in all index cases (ICs). Results: Abnormal multimers were present in 57 out of 150 ICs; however, only 29 out of these 57 (51%) had VWF ristocetin cofactor to antigen ratio below 0.7. In most cases multimer abnormalities were subtle, and only two cases had a significant loss of the largest multimers. Conclusions: Of the cases previously diagnosed as type 1 VWD, 38% showed abnormal multimers. Depending on the classification criteria used, 22 out of these 57 cases (15% of the total cohort) may be reclassified as type 2, emphasizing the requirement for multimer analysis compared with a mere ratio of VWF functional parameters and VWF:Ag. This is further supported by the finding that even slightly aberrant multimers are highly predictive for the presence of VWF mutations.
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4.
  • Eadson, Will, et al. (författare)
  • Decarbonising industry : A places-of-work research agenda
  • 2023
  • Ingår i: The Extractive Industries and Society. - : Elsevier. - 2214-790X .- 2214-7918. ; 15
  • Forskningsöversikt (refereegranskat)abstract
    • Deep decarbonisation of extractive and foundational industries will involve widespread social and economic change. Research on previous industrial restructuring has demonstrated that resultant changes will be geographically uneven, especially without countervailing state intervention. Such change has been shown to matter for both the nature and location of work in those industries as well as for the wider wellbeing of places. Concentrations of economic activity create place-based economic and sociocultural dependencies. As such in-dustries and industrial work often become entwined with workers' and communities' cultural identities. It is important to understand implications of industrial change for work, for place, and - as we argue here - relations between work and place. Building from a semi-systematic review of existing literature on industrial decarbon-isation, work and place, we extend prevailing political economic approaches to economic change, to also set out an original approach to decarbonising extractive and foundational industries, which we term 'places-of-work'. This approach is embedded in acknowledgement of the deep economic and cultural relations between work and place, which also plays out in processes of industrial decarbonisation. The approach builds from cultural and feminist approaches to economic change to emphasise sets of interrelations important to study of industrial decarbonisation as geographic phenomenon. Such an approach means extending the role of the state not as solely, or even primarily, focused on provision of training or employment opportunities, but as requiring adoption of a place-based approach to remaking economic and cultural characteristics of a location and its people. In setting out our alternative agenda, we seek to develop new insights that enable us to understand how industrial transitions potentially act within, and impact upon, places and their cultural identities, and the role of the state in reinforcing and disrupting these to support just transitions.
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5.
  • Feigin, Valery L., et al. (författare)
  • Global, regional, and national burden of neurological disorders, 1990–2016 : a systematic analysis for the Global Burden of Disease Study 2016
  • 2019
  • Ingår i: Lancet Neurology. - : Elsevier. - 1474-4422 .- 1474-4465. ; 18:5, s. 459-480
  • Tidskriftsartikel (refereegranskat)abstract
    • Background: Neurological disorders are increasingly recognised as major causes of death and disability worldwide. The aim of this analysis from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2016 is to provide the most comprehensive and up-to-date estimates of the global, regional, and national burden from neurological disorders.Methods: We estimated prevalence, incidence, deaths, and disability-adjusted life-years (DALYs; the sum of years of life lost [YLLs] and years lived with disability [YLDs]) by age and sex for 15 neurological disorder categories (tetanus, meningitis, encephalitis, stroke, brain and other CNS cancers, traumatic brain injury, spinal cord injury, Alzheimer's disease and other dementias, Parkinson's disease, multiple sclerosis, motor neuron diseases, idiopathic epilepsy, migraine, tension-type headache, and a residual category for other less common neurological disorders) in 195 countries from 1990 to 2016. DisMod-MR 2.1, a Bayesian meta-regression tool, was the main method of estimation of prevalence and incidence, and the Cause of Death Ensemble model (CODEm) was used for mortality estimation. We quantified the contribution of 84 risks and combinations of risk to the disease estimates for the 15 neurological disorder categories using the GBD comparative risk assessment approach.Findings: Globally, in 2016, neurological disorders were the leading cause of DALYs (276 million [95% UI 247–308]) and second leading cause of deaths (9·0 million [8·8–9·4]). The absolute number of deaths and DALYs from all neurological disorders combined increased (deaths by 39% [34–44] and DALYs by 15% [9–21]) whereas their age-standardised rates decreased (deaths by 28% [26–30] and DALYs by 27% [24–31]) between 1990 and 2016. The only neurological disorders that had a decrease in rates and absolute numbers of deaths and DALYs were tetanus, meningitis, and encephalitis. The four largest contributors of neurological DALYs were stroke (42·2% [38·6–46·1]), migraine (16·3% [11·7–20·8]), Alzheimer's and other dementias (10·4% [9·0–12·1]), and meningitis (7·9% [6·6–10·4]). For the combined neurological disorders, age-standardised DALY rates were significantly higher in males than in females (male-to-female ratio 1·12 [1·05–1·20]), but migraine, multiple sclerosis, and tension-type headache were more common and caused more burden in females, with male-to-female ratios of less than 0·7. The 84 risks quantified in GBD explain less than 10% of neurological disorder DALY burdens, except stroke, for which 88·8% (86·5–90·9) of DALYs are attributable to risk factors, and to a lesser extent Alzheimer's disease and other dementias (22·3% [11·8–35·1] of DALYs are risk attributable) and idiopathic epilepsy (14·1% [10·8–17·5] of DALYs are risk attributable).Interpretation: Globally, the burden of neurological disorders, as measured by the absolute number of DALYs, continues to increase. As populations are growing and ageing, and the prevalence of major disabling neurological disorders steeply increases with age, governments will face increasing demand for treatment, rehabilitation, and support services for neurological disorders. The scarcity of established modifiable risks for most of the neurological burden demonstrates that new knowledge is required to develop effective prevention and treatment strategies.Funding: Bill & Melinda Gates Foundation.
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6.
  • Goodeve, Anne, et al. (författare)
  • Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD)
  • 2007
  • Ingår i: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 109:1, s. 112-121
  • Tidskriftsartikel (refereegranskat)abstract
    • Type 1 von Willebrand disease (VWD) is characterized by a personal and family history of bleeding coincident with reduced levels of normal plasma von Willebrand factor (VWF). The molecular basis of the disorder is poorly understood. The aims of this study were to determine phenotype and genotype and their relationship in patients historically diagnosed with type 1 VWD. Families were recruited in 9 European countries based on previous type 1 VWD diagnosis. Bleeding symptoms were recorded, plasma phenotype analyzed, and VWF mutation analysis performed in all index cases (ICs). Phenotypic and molecular analysis stratified patients into those with or without phenotypes suggestive of qualitative VWF defects (abnormal multimers) and with or without mutations. A total of 105 of 150 ICs (70%) had mutations identified. A subgroup with abnormal multimers (38% of ICs, 57 of 150) showed a high prevalence of VWF gene mutations (95% of ICs, 54 of 57), whereas in those with qualitatively normal VWF, fewer mutations were identified (55% of ICs, 51 of 93). About one third of the type I VWD cases recruited could be reconsidered as type 2. The remaining group could be considered "true" type 1 VWD, although mutations were found in only 55%.
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7.
  • Koegl, Matthias, et al. (författare)
  • 3D LIF/Mie planar droplet s izing in IC engine sprays using single-droplet calibration data
  • 2020
  • Konferensbidrag (refereegranskat)abstract
    • This paper reports on the quantitative 2D and 3D droplet sizing based on the LIF/Mie ratio in ethanol DISI (Direct Injection Spark Ignition) sprays, where the required calibration is performed using quantitative micro and macro LIF/Mie ratio imaging of individual microdroplets. The droplets are generated from a monodisperse droplet generator in a diameter range from 16 µm to 60 µm. First, the liquid LIF (laser-induced fluorescence) and Mie scattered light (Mie) intensity from the dye-doped ethanol droplet illuminated by the 532 nm laser sheet are simultaneously recorded using a microscopic and a macroscopic imaging objective. The light intensity of the LIF and Mie optical signals are evaluated as a function of droplet diameters (d). The LIF and Mie intensity is found in a good agreement with volumetric (d3) and surface (d2) dependencies, respectively. Both micro and macro LIF/Mie intensity ratio show a linear dependence on d. The deduced calibration curve showing a correlation between LIF/Mie intensity ratio and droplet diameter is then used for the calibration of LIF/Mie ratio for 3D droplet size (Sauter Mean Diameter: SMD) in sprays. For 3D sizing, two-phase SLIPI-(Structured Laser Illumination Planar Imaging) based droplet sizing is applied in combination with laser sheet scanning for probing the spray in several two-dimensional (2D) slices. These 2D layers are combined together to reconstruct a quantitative 3D map of droplet SMD. Droplet SMD in the spray is found in the range between 10 µm and 50 µm.
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8.
  • Koegl, Matthias, et al. (författare)
  • Analysis of ethanol and butanol direct-injection spark-ignition sprays using two-phase structured laser illumination planar imaging droplet sizing
  • 2018
  • Ingår i: International Journal of Spray and Combustion Dynamics. - : SAGE Publications. - 1756-8277 .- 1756-8285. ; 11
  • Tidskriftsartikel (refereegranskat)abstract
    • This paper reports on the spray structure of the biofuels, ethanol, and butanol generated by a multihole direct-injection spark-ignition injector, which is studied in a constant volume chamber. The spray shape and structure are analyzed using two-phase structured laser illumination planar imaging where both laser-induced fluorescence and Mie-scattering light are recorded simultaneously for the extraction of instantaneous laser-induced fluorescence/Mie-scattering ratio images. Quantitative planar measurements of the droplet Sauter mean diameter are conducted, using calibration data from phase-Doppler anemometry. The resulting Sauter mean diameters are presented for ethanol and butanol at various fuel temperatures at different times after the start of injection. It is found that an increase in fuel temperature results in a faster atomization and higher evaporation rate, which leads to reduced spray tip penetration and smaller droplet Sauter mean diameter. At equivalent conditions, butanol consistently showed larger spray tip penetration in comparison to ethanol. This behavior is due to the higher surface tension and viscosity of butanol resulting in the formation of larger droplets and larger Sauter mean diameters in the whole spray region. Finally, the butanol injection also shows larger cyclic variations in the spray shape from injection to injection which is explained by the internal nozzle flow that is influenced by larger fuel viscosity as well. The Sauter mean diameter distribution is also compared to phase-Doppler anemometry data showing good agreement and an uncertainty analysis of the structured laser illumination planar imaging-laser-induced fluorescence/Mie-scattering technique for planar droplet sizing in direct-injection spark-ignition sprays is presented.
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9.
  • Koegl, Matthias, et al. (författare)
  • Analysis of LIF and Mie signals from single micrometric droplets for instantaneous droplet sizing in sprays
  • 2018
  • Ingår i: Optics Express. - 1094-4087. ; 26:24, s. 31750-31766
  • Tidskriftsartikel (refereegranskat)abstract
    • Planar droplet sizing (PDS) is a technique relying on the assumption that laser-induced fluorescence (LIF) and Mie scattering optical signals from spherical droplets depend on their volume and surface area, respectively. In this article, we verify the validity of this assumption by experimentally analyzing the light intensity of the LIF and Mie optical signals from micrometric droplets as a function of their diameter. The size of the droplets is controlled using a new flow-focusing monodisperse droplet generator capable of producing droplets of the desired size in the range of 21 µm to 60 µm. Ethanol droplets doped with eosin dye and excited at 532 nm are considered in this study, and the individual droplets were imaged simultaneously at microscopic and macroscopic scale. The effects of laser power, dye concentration, and temperature variation are systematically studied as a function of LIF/Mie ratio in the whole range of droplet sizes. Finally, a calibration curve at tracer concentration of 0.5 vol% is deduced and used to extract the droplet Sauter mean diameter (SMD) from instantaneous images of a transient ethanol spray. This droplet size mapping is done using structured laser illumination planar imaging (SLIPI), in order to suppress the artifacts induced by multiple light scattering.
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10.
  • Koegl, Matthias, et al. (författare)
  • Analysis of the LIF/Mie ratio from individual droplets for planar droplet sizing : Application to gasoline fuels and their mixtures with ethanol
  • 2019
  • Ingår i: Applied Sciences (Switzerland). - : MDPI AG. - 2076-3417. ; 9:22
  • Tidskriftsartikel (refereegranskat)abstract
    • In this work, the possibility of using planar droplet sizing (PDS) based on laser-induced fluorescence (LIF) and Mie scattering was investigated within the framework of measuring the droplet Sauter mean diameter (SMD) of direct-injection spark-ignition (DISI) spray systems. For this purpose, LIF and Mie signals of monodisperse fuel droplets produced by a droplet generator were studied at engine relevant diameters (20-50 μm). The surrogate gasoline fuel Toliso (consisting of 65 vol. % isooctane, 35 vol. % toluene) and the biofuel blend E20 (consisting of 80 vol. % Toliso, 20 vol. % ethanol) were used and which were doped with the fluorescence dye "nile red". The effects of ethanol admixture, dye concentration, laser power, and temperature variation on the LIF/Mie ratio were studied simultaneously at both macroscopic and microscopic scale. The deduced calibration curves of the LIF and Mie signals of both fuels showed volumetric and surface dependent behaviors, respectively, in accordance with the assumptions in the literature. The existence of glare points and morphology-dependent resonances (MDRs) lead to slightly higher LIF and Mie exponents of E20 in comparison to Toliso. In principle, these calibration curves enable the determination of the SMD from LIF/Mie ratio images of transient fuel sprays.
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