Familial hypercholesterolaemia in children and adolescents from 48 countries: a cross-sectional study

Background: Approximately 450 000 children are born with familial hypercholesterolaemia worldwide every year, yet only 2·1% of adults with familial hypercholesterolaemia were diagnosed before age 18 years via current diagnostic approaches, which are derived from observations in adults. We aimed to characterise children and adolescents with heterozygous familial hypercholesterolaemia (HeFH) and understand current approaches to the identification and management of familial hypercholesterolaemia to inform future public health strategies. Methods: For this cross-sectional study, we assessed children and adolescents younger than 18 years with a clinical or genetic diagnosis of HeFH at the time of entry into the Familial Hypercholesterolaemia Studies Collaboration (FHSC) registry between Oct 1, 2015, and Jan 31, 2021. Data in the registry were collected from 55 regional or national registries in 48 countries. Diagnoses relying on self-reported history of familial hypercholesterolaemia and suspected secondary hypercholesterolaemia were excluded from the registry; people with untreated LDL cholesterol (LDL-C) of at least 13·0 mmol/L were excluded from this study. Data were assessed overall and by WHO region, World Bank country income status, age, diagnostic criteria, and index-case status. The main outcome of this study was to assess current identification and management of children and adolescents with familial hypercholesterolaemia. Findings: Of 63 093 individuals in the FHSC registry, 11 848 (18·8%) were children or adolescents younger than 18 years with HeFH and were included in this study; 5756 (50·2%) of 11 476 included individuals were female and 5720 (49·8%) were male. Sex data were missing for 372 (3·1%) of 11 848 individuals. Median age at registry entry was 9·6 years (IQR 5·8-13·2). 10 099 (89·9%) of 11 235 included individuals had a final genetically confirmed diagnosis of familial hypercholesterolaemia and 1136 (10·1%) had a clinical diagnosis. Genetically confirmed diagnosis data or clinical diagnosis data were missing for 613 (5·2%) of 11 848 individuals. Genetic diagnosis was more common in children and adolescents from high-income countries (9427 [92·4%] of 10 202) than in children and adolescents from non-high-income countries (199 [48·0%] of 415). 3414 (31·6%) of 10 804 children or adolescents were index cases. Familial-hypercholesterolaemia-related physical signs, cardiovascular risk factors, and cardiovascular disease were uncommon, but were more common in non-high-income countries. 7557 (72·4%) of 10 428 included children or adolescents were not taking lipid-lowering medication (LLM) and had a median LDL-C of 5·00 mmol/L (IQR 4·05-6·08). Compared with genetic diagnosis, the use of unadapted clinical criteria intended for use in adults and reliant on more extreme phenotypes could result in 50-75% of children and adolescents with familial hypercholesterolaemia not being identified. Interpretation: Clinical characteristics observed in adults with familial hypercholesterolaemia are uncommon in children and adolescents with familial hypercholesterolaemia, hence detection in this age group relies on measurement of LDL-C and genetic confirmation. Where genetic testing is unavailable, increased availability and use of LDL-C measurements in the first few years of life could help reduce the current gap between prevalence and detection, enabling increased use of combination LLM to reach recommended LDL-C targets early in life

Challenges and Opportunities for International Trade in Forest Biomass

In an effort to reduce fossil fuel consumption, the use of woody biomass for heat and power generation is growing. Key destination markets will be countries within the European Union, particularly the United Kingdom, the Netherlands, Denmark and Belgium. While demand from Asia (particularly South Korea and Japan) will also increase, it will continue to play a secondary role. Across the EU, adoption of sustainability criteria for forest biomass based on the Renewable Energy Directive (RED) 2009/28/EC or similar criteria is likely. In the United Kingdom, the largest market for traded wood pellets, such criteria have already been proposed. As of 2015, only forest biomass that achieves at least a 60% reduction in GHG emissions, relative to the EU fossil fuel electricity average, can be used for bioenergy production and proof of SFM is required. In the Netherlands, the energy industry and non-governmental organisations achieved principal agreement on sustainability criteria for solid biomass, but issues of compliance testing and monitoring must still be addressed. In Flemish Belgium, the sustainability requirements for bioliquids may soon be applied to woody biomass. In Denmark, a voluntary industry agreement is set to ensure that all bioenergy production by 2019 is conducted sustainably. At present, internationally traded wood pellets from temperate and boreal biomes originate primarily from the United States, Canada and Russia. Among these, Canada offers large stretches of SFM-certified forests, but the US Southeast has seen the strongest increase in wood pellet production and export in recent years. The expansion of pellet production in the Southeast United States is mainly linked to available forest inventory (pulpwood in particular) and the competitive advantage gained by the relative proximity to demand markets in the EU. In addition to the mobilisation barriers observed in supply chains at the local and national scale, limitations in the supply of forest biomass for international trade will be influenced by regulatory measures that could restrict the trade of specific feedstock fractions

Charting global position and vision of stakeholders towards sustainable bioenergy

Background: Stakeholder's position of bioenergy sustainability is important for the deployment and contribution of bioenergy to sustainable development. Existing publications are usually limited to specific geographical contexts and focuses. This paper aims more broadly to examine the position and vision of a wider range of stakeholder groups towards bioenergy and its development at a global level. Method: The applied methodology includes six steps: (1) identification of stakeholders as belonging to one of seven groups; (2) describing the role of each group in relation to bioenergy; (3) data collection via an online questionnaire, roundtable dialogues and interviews to examine their stated awareness and opinions of bioenergy development, driver and barriers to such development; (4) data analysis; (5) comparison of interests and influence as a basis for expressing position and vision; and (6) recommendations for gaining support for sustainable bioenergy development. Results: The stakeholders state awareness of bioenergy development and have in general a positive view of the sector. They also inform that the general public is less aware of and not sufficiently involved in bioenergy development. Internet and social media are the most consulted sources of information but least trusted, while scientific information is most trusted but least used. Agricultural residues, energy crops cultivated on marginal or degraded land and forestry residues are widely accepted as feedstocks for bioenergy production, whereas use of agricultural land is viewed critically. The stakeholders generally support bioenergy development when jointly agreed sustainability requirements are met. Conclusions: The stakeholders acknowledge the important role of effectively disseminating scientific information as an influencing factor on the position towards bioenergy. They also find that enhancing support for the bioenergy sector relies on mandatory sustainability requirements covering social, economic and environmental aspects, applied to all types of biomass regardless of end use. Some also emphasise that all relevant sectors should work on market conditions to create a level playing field and that this is crucial to change stakeholders' position to gain more social acceptance of bioenergy. Transparency in demonstrating compliance with sustainability criteria is also an expected pre-condition to enhance support for bioenergy (and ultimately the bioeconomy) in the long term

Effective sustainability criteria for bioenergy: Towards the implementation of the european renewable directive II

Sustainability criteria and verification through national legislations and voluntary certification schemes are important tools to ensure sustainable supply and bioenergy development in the European Union. The Renewable Energy Directive Recast (RED II) sets the framework for renewable energy support for the period 2021–2030 with updated and new sustainability criteria. This study reviews the sustainability criteria in the RED II and in existing national legislations and voluntary schemes. The aim is to identify possible gaps and good practices in certification to propose a set of sustainability criteria that are effective in their coverage of the most urgent sustainability concerns, and that are practically applicable to the whole bioenergy sector. The proposed set of effective sustainability criteria was validated through stakeholder interviews. The results show that the RED II is a major step forward in safeguarding sustainable bioenergy supply; however, it still entails sustainability risks in forest management and lacks clarifications and criteria for imported biomass feedstocks. The proposed effective sustainability criteria in this study are more extensive than in the RED II and help to assure sustainable land use, to protect biodiversity, and to conserve ecosystems, whilst also addressing rights for workers and local communities, and the efficient use of resources. These criteria are already implemented in some comprehensive and stringent national support schemes and voluntary schemes. It is recommended that policy makers, scheme owners and sustainability practitioners coordinate discussions and agreements on the various sustainability aspects. A clear definition of waste and residues, measurement of indirect land use change, and recognition of competent voluntary schemes to demonstrate sustainability compliance should be considered at EU level

Assessment of sustainable lignocellulosic biomass export potentials from Brazil to the European Union

The main aim of BioTrade2020plus is to provide guidelines for the development of a European Bioenergy Trade Strategy for 2020 and beyond ensuring that imported biomass feedstock is sustainably sourced and used in an efficient way, while avoiding distortion of other (non-energy) markets. This will be accomplished by analyzing the potentials (technical, economical and sustainable) and assessing key sustainability risks of current and future lignocellulosic biomass and bioenergy carriers. Focus will be placed on wood chips, pellets, torrefied biomass and pyrolysis oil from current and potential future major sourcing regions of the world (US-SE, Ukraine, South America, Asia and Sub-Saharan Africa). BioTrade2020plus will thus provide support to the use of stable, sustainable, competitively priced and resource-efficient flows of imported biomass feedstock to the EU – a necessary pre-requisite for the development of the bio-based economy in Europe. In order to achieve this objective close cooperation will be ensured with current international initiatives such as IEA Bioenergy Task 40 on “Sustainable International Bioenergy Trade - Securing Supply and Demand” and European projects such as Biomass Policies, S2BIOM, Biomass Trade Centers, DIA-CORE, and PELLCERT

Charting global position and vision of stakeholders towards sustainable bioenergy

Background: Stakeholder's position of bioenergy sustainability is important for the deployment and contribution of bioenergy to sustainable development. Existing publications are usually limited to specific geographical contexts and focuses. This paper aims more broadly to examine the position and vision of a wider range of stakeholder groups towards bioenergy and its development at a global level. Method: The applied methodology includes six steps: (1) identification of stakeholders as belonging to one of seven groups; (2) describing the role of each group in relation to bioenergy; (3) data collection via an online questionnaire, roundtable dialogues and interviews to examine their stated awareness and opinions of bioenergy development, driver and barriers to such development; (4) data analysis; (5) comparison of interests and influence as a basis for expressing position and vision; and (6) recommendations for gaining support for sustainable bioenergy development. Results: The stakeholders state awareness of bioenergy development and have in general a positive view of the sector. They also inform that the general public is less aware of and not sufficiently involved in bioenergy development. Internet and social media are the most consulted sources of information but least trusted, while scientific information is most trusted but least used. Agricultural residues, energy crops cultivated on marginal or degraded land and forestry residues are widely accepted as feedstocks for bioenergy production, whereas use of agricultural land is viewed critically. The stakeholders generally support bioenergy development when jointly agreed sustainability requirements are met. Conclusions: The stakeholders acknowledge the important role of effectively disseminating scientific information as an influencing factor on the position towards bioenergy. They also find that enhancing support for the bioenergy sector relies on mandatory sustainability requirements covering social, economic and environmental aspects, applied to all types of biomass regardless of end use. Some also emphasise that all relevant sectors should work on market conditions to create a level playing field and that this is crucial to change stakeholders' position to gain more social acceptance of bioenergy. Transparency in demonstrating compliance with sustainability criteria is also an expected pre-condition to enhance support for bioenergy (and ultimately the bioeconomy) in the long term

Effective sustainability criteria for bioenergy: Towards the implementation of the european renewable directive II

Sustainability criteria and verification through national legislations and voluntary certification schemes are important tools to ensure sustainable supply and bioenergy development in the European Union. The Renewable Energy Directive Recast (RED II) sets the framework for renewable energy support for the period 2021–2030 with updated and new sustainability criteria. This study reviews the sustainability criteria in the RED II and in existing national legislations and voluntary schemes. The aim is to identify possible gaps and good practices in certification to propose a set of sustainability criteria that are effective in their coverage of the most urgent sustainability concerns, and that are practically applicable to the whole bioenergy sector. The proposed set of effective sustainability criteria was validated through stakeholder interviews. The results show that the RED II is a major step forward in safeguarding sustainable bioenergy supply; however, it still entails sustainability risks in forest management and lacks clarifications and criteria for imported biomass feedstocks. The proposed effective sustainability criteria in this study are more extensive than in the RED II and help to assure sustainable land use, to protect biodiversity, and to conserve ecosystems, whilst also addressing rights for workers and local communities, and the efficient use of resources. These criteria are already implemented in some comprehensive and stringent national support schemes and voluntary schemes. It is recommended that policy makers, scheme owners and sustainability practitioners coordinate discussions and agreements on the various sustainability aspects. A clear definition of waste and residues, measurement of indirect land use change, and recognition of competent voluntary schemes to demonstrate sustainability compliance should be considered at EU level