RARE-Bestpractices: a platform for sharing best practices for the management of rare diseases

No abstract available

RARE-Bestpractices: a platform for sharing best practices for the management of rare diseases

From 7th European Conference on Rare Diseases and Orphan Products (ECRD 2014).Rare diseases; clinical practice guidelines; recommendations. RARE-Bestpractices (http://www.rarebestpractices.eu) is a 4-year project (2013-2016) funded by the EC FP7. The project aims at improving clinical management of patients with rare diseases (RD) and at narrowing the existing gap in quality of healthcare among countries. Methods: RARE-Bestpractices (http://www.rarebestpractices.eu) involves 9 EU countries, including 15 partners from academic institutions, governmental bodies, patient organizations and networks, which will exploit the added value of integrating different contributions and viewpoints. The platform is developed involving both experts in RD research as well as experts in clinical practice guidelines (CPG) and systematic reviews. Results: Project expected outputs include: 1) identification of challenges to be considered in deriving high quality standards for CPG on RD; 2) transparent procedures and criteria for the evaluation of CPG and their collection in a publicly searchable database; 3) identification of notation criteria to improve user understandability and implementation of CPG; 4) production of mechanisms to assess RD clinical research needs; 5) development of training activities targeted to key stakeholders to disseminate process and tools for developing and evaluating CPG; 6) the publication of a new scientific journal (http://rarejournal.org). Discussion: RARE-Bestpractices addresses the demands from both patients and health care providers for updated and high quality CPG on RD. The project will meet the requirements laid down by to the Directive 2011/24/EU, which endorses EU MS to develop European Reference Networks (ERNs) for RD; in fact, one main criterion for ERNs should be the competence to produce CPG and actively disseminate them among Centers of Expertise.N

RARE-Bestpractices: a platform for sharing best practices for the management of rare diseases

No abstract available

Stabilisation of precipitates of pedogenic dissolved organic matter by multivalent cations

Purpose Precipitation of dissolved organic matter (DOM) by multivalent cations is important for biogeochemical cycling of organic carbon. We investigated to which extent cation bridges are involved in DOM precipitation and how cross-links by cations and water molecule bridges (WaMB) stabilise the matrix of precipitated DOM. Materials and methods DOM was precipitated from the aqueous extract of a forest floor layer adding solutions of Ca(NO3)2, Al(NO3)3 and Pb(NO3)2 with different initial metal cation/C (Me/C) ratios. Precipitates were investigated by differential scanning calorimetry before and after ageing to detect cation bridges, WaMB and restructuring of supramolecular structure. Results and discussion Twenty-five to sixty-seven per cent of the dissolved organic carbon was precipitated. The precipitation efficiency of cations increased in the order Ca  3 is explained by additional formation of small AlOOH particles. Thermal analysis indicated WaMB and their disruption at T* of 53–65 °C. Like cation content, T* increased with increasing Me/C ratio and in the order Ca < Pb < Al for low Me/C. This supports the general assumption that cross-linking ability increases in the order Ca < Pb < Al. The low T* for high initial Me/C suggests less stable and less cross-linked precipitates than for low Me/C ratios. Conclusions Our results suggest a very similar thermal behaviour of OM bound in precipitates compared with soil organic matter and confirms the relevance of WaMB in stabilisation of the supramolecular structure of cation-DOM precipitates. Thus, stabilisation of the supramolecular structure of the DOM precipitates is subjected to dynamics in soils

Stabilisation of precipitates of pedogenic dissolved organic matter by multivalent cations

Purpose Precipitation of dissolved organic matter (DOM) by multivalent cations is important for biogeochemical cycling of organic carbon. We investigated to which extent cation bridges are involved in DOM precipitation and how cross-links by cations and water molecule bridges (WaMB) stabilise the matrix of precipitated DOM. Materials and methods DOM was precipitated from the aqueous extract of a forest floor layer adding solutions of Ca(NO3)2, Al(NO3)3 and Pb(NO3)2 with different initial metal cation/C (Me/C) ratios. Precipitates were investigated by differential scanning calorimetry before and after ageing to detect cation bridges, WaMB and restructuring of supramolecular structure. Results and discussion Twenty-five to sixty-seven per cent of the dissolved organic carbon was precipitated. The precipitation efficiency of cations increased in the order Ca  3 is explained by additional formation of small AlOOH particles. Thermal analysis indicated WaMB and their disruption at T* of 53–65 °C. Like cation content, T* increased with increasing Me/C ratio and in the order Ca < Pb < Al for low Me/C. This supports the general assumption that cross-linking ability increases in the order Ca < Pb < Al. The low T* for high initial Me/C suggests less stable and less cross-linked precipitates than for low Me/C ratios. Conclusions Our results suggest a very similar thermal behaviour of OM bound in precipitates compared with soil organic matter and confirms the relevance of WaMB in stabilisation of the supramolecular structure of cation-DOM precipitates. Thus, stabilisation of the supramolecular structure of the DOM precipitates is subjected to dynamics in soils

Transformative Biodiversity Governance in Agricultural Landscapes: Taking Stock and Looking Forward

Agricultural land systems, covering about 40 percent of the world’s ice-free terrestrial surface, are the single largest contributor to biodiversity loss worldwide (Chapin et al., 2000; IPBES, 2018a; 2019). Agricultural practices have been linked to staggering losses in critical ecosystems such as tropical forests and ecologically functional species such as pollinators, raising concerns of losing biodiversity as both an intrinsic global value and as a central pillar of food security and ecosystem functions (IPBES, 2016; Laurance et al. 2014; Ramankutty et al., 2018)

Transformative Biodiversity Governance in Agricultural Landscapes: Taking Stock and Looking Forward

Agricultural land systems, covering about 40 percent of the world’s ice-free terrestrial surface, are the single largest contributor to biodiversity loss worldwide (Chapin et al., 2000; IPBES, 2018a; 2019). Agricultural practices have been linked to staggering losses in critical ecosystems such as tropical forests and ecologically functional species such as pollinators, raising concerns of losing biodiversity as both an intrinsic global value and as a central pillar of food security and ecosystem functions (IPBES, 2016; Laurance et al. 2014; Ramankutty et al., 2018)

RARE-Bestpractices: a platform for sharing best practices for the management of rare diseases

From 7th European Conference on Rare Diseases and Orphan Products (ECRD 2014).Rare diseases; clinical practice guidelines; recommendations. RARE-Bestpractices (http://www.rarebestpractices.eu) is a 4-year project (2013-2016) funded by the EC FP7. The project aims at improving clinical management of patients with rare diseases (RD) and at narrowing the existing gap in quality of healthcare among countries. Methods: RARE-Bestpractices (http://www.rarebestpractices.eu) involves 9 EU countries, including 15 partners from academic institutions, governmental bodies, patient organizations and networks, which will exploit the added value of integrating different contributions and viewpoints. The platform is developed involving both experts in RD research as well as experts in clinical practice guidelines (CPG) and systematic reviews. Results: Project expected outputs include: 1) identification of challenges to be considered in deriving high quality standards for CPG on RD; 2) transparent procedures and criteria for the evaluation of CPG and their collection in a publicly searchable database; 3) identification of notation criteria to improve user understandability and implementation of CPG; 4) production of mechanisms to assess RD clinical research needs; 5) development of training activities targeted to key stakeholders to disseminate process and tools for developing and evaluating CPG; 6) the publication of a new scientific journal (http://rarejournal.org). Discussion: RARE-Bestpractices addresses the demands from both patients and health care providers for updated and high quality CPG on RD. The project will meet the requirements laid down by to the Directive 2011/24/EU, which endorses EU MS to develop European Reference Networks (ERNs) for RD; in fact, one main criterion for ERNs should be the competence to produce CPG and actively disseminate them among Centers of Expertise.N