Schnitzler-Syndrom mit Urtikaria-Vaskulitis

Zusammenfassung: Das Schnitzler-Syndrom ist eine seltene Erkrankung, welche mit Urtikaria, periodischem Fieber, Knochenschmerzen bei Hyperostosis, Arthritiden und einer monoklonalen IgM-Gammopathie einhergeht. Es wurde 1974 durch die französische Dermatologin Liliane Schnitzler beschrieben. Aufgrund der sehr unterschiedlichen Symptome werden die Betroffenen oft von verschiedenen Fachärzten gesehen und sind insbesondere für Internisten, Rheumatologen, Hämatologen und Dermatologen von Interesse. Bislang war die Therapie oft schwierig und enttäuschend. Ein neuer therapeutischer Ansatz ist die Gabe eines Interleukin-1-Rezeptorantagoniste

Towards net zero CO2 in 2050: an emission reduction pathway for organic soils in Germany

The Paris Agreement reflects the global endeavour to limit the increase of global average temperature to 2 °C, better 1.5 °C above pre-industrial levels to prevent dangerous climate change. This requires that global anthropogenic net carbon dioxide (CO2) emissions are reduced to zero around 2050. The German Climate Protection Plan substantiates this goal and explicitly mentions peatlands, which make up 5 % of the total area under land use and emit 5.7 % of total annual greenhouse gas emissions in Germany. Based on inventory reporting and assumptions of land use change probability, we have developed emission reduction pathways for organic soils in Germany that on a national level comply with the IPCC 1.5 °C pathways. The more gradual pathway 1 requires the following interim (2030, 2040) and ultimate (2050) milestones: Cropland use stopped and all Cropland converted to Grassland by 2030; Water tables raised to the soil surface on 15 % / 60 % / 100 % of all Grassland, on 50 % / 75 % / 100 % of all Forest land, and ultimately on 2/3 of all Settlements and on 100 % of all Wetlands. Also a more direct pathway 2 without interim ‘moist’ water tables and the climate effect (radiative forcing) of different scenarios is presente

Combustibility of biomass from wet fens in Belarus and its potential as a substitute for peat in fuel briquettes

Peatland drainage has caused enormous environmental problems at global scale; in particular, ongoing greenhouse gas emissions and soil degradation. In Belarus, which is rich in peatlands and a hotspot of emissions from drained peatlands, several thousand hectares have already been re-wetted but are not used productively. Moreover, vast areas of wet (undrained) peatland that are designated for nature conservation are in need of mowing and biomass removal. Plants such as common reed (Phragmites australis), reed canary grass (Phalaris arundinacea) and sedges (Carex spp.) which frequently dominate these areas could be harvested and used as fuel, potentially as a substitute for peat. In this study we analysed the yield and combustibility of late harvests in March/April 2009 and 2010. The yields of 3.7–11.7 t DM ha-1 were within the range reported from other studies on wetland plants. Concentrations of Cl, S, N, P, C, Ca, K, Mg and Na, as well as water and ash contents, indicated similar or better combustibility when compared to other straw-like (graminaceous) plants such as Miscanthus. The full replacement of peat fuel by biomass from wet peatlands in Belarus would require an area of 680,000 ha, i.e. 'only' half of the peatland that has been drained for agriculture

High-fidelity simulations of the mixing and combustion of a technically premixed hydrogen flame

Numerical simulations are used here to obtain further understanding on the flashback mechanism of a technically premixed hydrogen flame operated in lean conditions. Recent work from the authors (Mira et al., 2020) showed that the hydrogen momentum strongly influences the flame dynamics and plays a fundamental role on the stability limits of the combustor. The axial injection influences the vortex breakdown position and therefore, the propensity of the burner to produce flashback. This work is an extension of our previous work where we include a detailed description of the mixing process and the influence of equivalence ratio fluctuations and heat loss on the flame dynamics

Integrating ecosystem markets to co-ordinate landscape-scale public benefits from nature

Ecosystem markets are proliferating around the world in response to increasing demand for climate change mitigation and provision of other public goods. However, this may lead to perverse outcomes, for example where public funding crowds out private investment or different schemes create trade-offs between the ecosystem services they each target. The integration of ecosystem markets could address some of these issues but to date there have been few attempts to do this, and there is limited understanding of either the opportunities or barriers to such integration. This paper reports on a comparative analysis of eleven ecosystem markets in operation or close to market in Europe, based on qualitative analysis of 25 interviews, scheme documentation and two focus groups. Our results indicate three distinct types of markets operating from the regional to national scale, with different modes of operation, funding and outcomes: regional ecosystem markets, national carbon markets and green finance. The typology provides new insights into the operation of ecosystem markets in practice, which may challenge traditionally held notions of Payment for Ecosystem Services. Regional ecosystem markets, in particular, represent a departure from traditional models, by using a risk-based funding model and aggregating both supply and demand to overcome issues of free-riding, ecosystem service trade-offs and land manager engagement. Central to all types of market were trusted intermediaries, brokers and platforms to aggregate supply and demand, build trust and lower transaction costs. The paper outlines six options for blending public and private funding for the provision of ecosystem services and proposes a framework for integrating national carbon markets and green finance with regional ecosystem markets. Such integration may significantly increase funding for regenerative agriculture and conservation across multiple habitats and services, whilst addressing issues of additionality and ecosystem service trade-offs between multiple schemes

Towards the development of a UK Peatland Code: Payments for Ecosystem Services (PES) Pilot Research Project

This Final Report describes research into the rationale and basis for the development of a UK Peatland Code. The development of the Code was supported by the latest relevant research evidence, via an international expert workshop, and by feedback from the Code’s Steering Group and 17 individuals/organisations from across the UK. The Code was launched in September 2013, at the start of an 18-month pilot phase of operation, in which the Code will be further developed, and is available online: http://www.iucn-uk-peatlandprogramme.org/peatland-code. There is growing interest in the creation of new markets to facilitate private investment in the provision of ecosystem services, and this was a key emphasis of Defra’s 2011 Natural Environment White Paper. The White Paper led to the formation of an Ecosystem Markets Taskforce to identify business opportunities in the natural environment that recommended the development of a UK Peatland Code in its report in March 2013. Subsequently in May 2013, Defra published an action plan for developing the potential for payments for ecosystem services in which it committed to work in partnership with the IUCN and others to support the testing, development and launch later in 2013 of a pilot UK Peatland Code. UK peatlands are a relevant place to explore the potential to pay for ecosystem services, given the range and importance of ecosystem services that they supply, and that fact that many of these service are not widely or fully paid for via agricultural support payments or by markets. This has led to the degradation of many peatlands through inappropriate burning, overWgrazing and drainage, leading to reductions in carbon storage, water quality and biodiversity. Investing in conserving and restoring peatlands is therefore a key tool to help deliver the UK’s climate change obligations, whilst helping meet other national and international obligations on biodiversity and water quality. Although there is growing interest from the private sector in paying for some of these ecosystem services, and there have been a small number of bilateral agreements to pay for peatland restoration via the Corporate Social Responsibility market, there is a need to develop guidance, frameworks and monitoring to provide sponsors with the confidence necessary to restore peatlands on any significant scale. This Payment for Ecosystem Service (PES) pilot research project therefore conducted the research necessary to develop and launch a UK pilot Peatland Code, designed to support markets that could pay for the restoration and reWwetting of degraded peatlands across the UK. The research project and subsequent Code drew significantly on Defra’s PES Best Practice Guide, first identifying a saleable ecosystem service and prospective buyers and sellers, based on previous work and new market research conducted as part of this project (Phase 1 in the PES Guide). The Code establishes the principles for a peatland PES scheme, and resolves a number of technical issues, to make it possible for buyers and sellers to work together for peatland restoration (Phase 2 in the PES Guide). The Code also provides guidance on contracts and monitoring (Phases 3 and 4 of the PES Guide), and takes a bundled approach to PES (a ‘premium carbon’ scheme that provides a range of coWbenefits), whilst not ruling out the possibility of layered schemes (e.g. using the Code to elicit payments for climate mitigation benefits on top of existing schemes that pay for water quality benefits) (Phase 5 of the PES Guide). The research offers a number of insights into the creation of markets for ecosystem services linked to peatland restoration. It also offers insights of more general relevance to the creation and implementation of new markets for ecosystem services, which may be of relevance to other habitats and ecosystems in the UK, and to the development of new PES schemes internationally

Active afforestation of drained peatlands is not a viable option under the EU Nature Restoration Law

The EU Nature Restoration Law (NRL) is critical in restoring degraded ecosystems. However, active afforestation of degraded peatlands has been suggested by some as a restoration measure under the NRL. Here, we discuss the current state of scientific evidence on the climate mitigation effects of peatlands under forestry and its limitations, uncertainties and evidence gaps. Based on this discussion we conclude: Afforestation of drained peatlands, while maintaining their drained state, is not equivalent to ecosystem restoration. This approach will not restore the peatland ecosystem's flora, fauna, and functions. There is insufficient evidence to support the long-term climate change mitigation benefits of active afforestation of drained peatlands. Most studies only focus on the short-term gains in standing biomass and rarely explore the full life cycle emissions associated with afforestation of drained peatlands. Thus, it is unclear whether the CO2 sequestration of a forest on drained peatland can offset the carbon loss from the peat over the long term. In some ecosystems, such as abandoned or certain cutaway peatlands, afforestation may provide short-term benefits for climate change mitigation compared to taking no action. However, this approach violates the concept of sustainability by sacrificing the most space-effective carbon store of the terrestrial biosphere, the long-term peat store, for a shorter-term, less space-effective, and more vulnerable carbon store, namely tree biomass. Consequently, active afforestation of drained peatlands is not a viable option for climate mitigation under the EU Nature Restoration Law and might even impede future rewetting/restoration efforts. To restore degraded peatlands, hydrological conditions must first be improved, primarily through rewetting

Predicting Concentrations of Organic Chemicals in Fish by Using Toxicokinetic Models

Quantification of chemical toxicity continues to be generally based on measured external concentrations. Yet, internal chemical concentrations have been suggested to be a more suitable parameter. To better understand the relationship between the external and internal concentrations of chemicals in fish, and to quantify internal concentrations we compared three. toxicokinetic (TK) models with each other and with literature data of measured concentrations of 39 chemicals. Two one, compartment models, together with the physiologically based toxicokinetic (PBTK) model, in which we improved the treatment of lipids, were used to predict concentrations of organic chemicals in two fish species: rainbow trout (Oncorhynchus mykiss) and fathead minnow (Pimephales promelas). All models predicted the measured internal concentrations in fish within I order of magnitude for at least 68% of the chemicals. Furthermore, the PBTK model outperformed the one-compartment models with respect to simulating chemical concentrations in the whole body (at least 88% of internal concentrations were predicted within 1 order of magnitude using the PBTK model). All the models can be used to predict concentrations in different fish species without additional experiments. However, further development of TK models is required for polar, ionizable, and easily biotransformed compounds

The Schnitzler syndrome

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenström disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections