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- Sobas, Marta, et al.
(författare)
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Real-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs
- 2022
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Ingår i: BLOOD ADVANCES. - : ELSEVIER. - 2473-9529 .- 2473-9537. ; 6:17, s. 5171-5183
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Tidskriftsartikel (refereegranskat)abstract
- Myeloproliferative neoplasms (MPNs) are uncommon in children/young adults. Here, we present data on unselected patients diagnosed before 25 years of age included from 38 centers in 15 countries. Sequential patients were included. We identified 444 patients, with median follow-up 9.7 years (0-47.8). Forty-nine (11.1%) had a history of thrombosis at diagnosis, 49 new thrombotic events were recorded (1.16% patient per year [pt/y]), perihepatic vein thromboses were most frequent (47.6% venous events), and logistic regression identified JAK2V617F mutation (P = .016) and hyperviscosity symptoms (visual disturbances, dizziness, vertigo, headache) as risk factors (P = .040). New hemorrhagic events occurred in 44 patients (9.9%, 1.04% pt/y). Disease transformation occurred in 48 patients (10.9%, 1.13% pt/y), usually to myelofibrosis (7.5%) with splenomegaly as a novel risk factor for transformation in essential thrombocythemia (ET) (P= .000) in logistical regression. Eight deaths (1.8%) were recorded, 3 after allogeneic stem cell transplantation. Concerning conventional risk scores: International Prognostic Score for Essential Thrombocythemia-Thrombosis and new International Prognostic Score for Essential Thrombocythemia-Thrombosis differentiated ET patients in terms of thrombotic risk. Both scores identified high-risk patients with the same median thrombosis-free survival of 28.5 years. No contemporary scores were able to predict survival for young ET or polycythemia vera patients. Our data represents the largest real-world study of MPN patients age < 25 years at diagnosis. Rates of thrombotic events and transformation were higher than expected compared with the previous literature. Our study provides new and reliable information as a basis for prospective studies, trials, and development of harmonized international guidelines for the specific management of young patients with MPN.
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| 2. |
- Allard, Bert, 1945-, et al.
(författare)
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Mining waste management in the Baltic Sea Region : Min-Novation project
- 2013
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Bok (refereegranskat)abstract
- Mining waste and what to do about it is a common challenge facing companies, local authorities, environmental organizations, policymakers and increasingly other stakeholders in several countries of the Baltic Sea Region. From 2011 to 2013, a network of scientific and regional expertise brought together in the Min-Novation project has put this topic in the spotlight. The importance of the management of waste from extractive industries is due to the substantial share this waste has in the overall stream of waste generated in the EU. In 2010, 672 Mt or 28.3% of the total waste generated in the EU was attributable to the mining and quarrying industry, second only to construction (34.4%) and ahead of manufacturing (11.0%) and households (8.7%)1. Apart from this, mining waste is the raw material for one of the more visible man-made landmarks that surround us, with waste heaps of various shapes and sizes dotting the landscape up and down the Baltic Sea Region. Despite this dual prominence, mining waste is most often seen only as an environmental problem and in no way a resource. To move away from a one-sided view of mining waste, a life-cycle approach, which recognises that value can be recovered from waste and re-introduced into the product cycle is of the essence. It cannot be stressed enough that mining waste is a source of secondary raw materials, the use of which helps to protect the natural mineral deposits for future generations. Equally important is an appreciation of how the waste can be re-cycled in the excavation process (preventionand recovery) and adapted to create value for local communities (reclamation and revitalisation). However, for there to be effective mining waste management, both in the prevention stage, as well as in the recovery stage, and finally during land reclamation many conditions must be fulfilled. Of these the most important are access to appropriate technologies and methods and common sense legislation. Another condition not without importance is social acceptance for the recovery of waste located in old landfills. The Min-Novation Network over a span of 3 years has worked to understand and appreciate mining waste both as a corporate, community, regulatory and strategic issue. Set against the background of mining activity and waste management in the partner countries: Estonia, Finland, Germany, Norway, Poland and Sweden, both good practices and problem areas, which need to be addressed have been presented in this monograph. The purpose of this monograph is to show a cross-section of topics that affect how mining waste management works today, and which will play a decisive role in whether management of mining waste remains – an issue of primarily local relevance or whether it becomes a growth opportunity of national and EU-wide importance. The monograph focuses primarily on issues related to the management of waste from extractive industries in the countries whose representatives were involved in the Min-Novation project. Examples from outside the Baltic Sea Region of the use of waste heaps as an industrial heritage of the mining regions and also as attractions for local communities are presented as well. Indeed, every experience is valuable for the environment and socio-economic development of the Baltic Sea Region.
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