56 research outputs found

    2nd Annual Workshop Proceedings of the Collaborative Project "Fast / Instant Release of Safety Relevant Radionuclides from Spent Nuclear Fuel" (7th EC FP CP FIRST-Nuclides), Antwerp 05 - 07 November 2013 (KIT Scientific Reports ; 7676)

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    This proceedings document the 2nd Annual Workshop of the EURATOM FP7 Collaborative Project FIRST-Nuclides (Fast / Instant Release of Safety Relevant Radionuclides from Spent Nuclear Fuel). 10 beneficiaries and 11 associated groups participated in the 2nd Annual Workshop, as well as interested externals. The key purpose of the proceedings is documenting the scientific outcome of the FIRST-Nuclides project. This is covered by scientific/technical contributions of the partners. It includes reports by the Associated Groups and the Topical Session

    1st Annual Workshop Proceedings of the Collaborative Project "Fast / Instant Release of Safety Relevant Radionuclides from Spent Nuclear Fuel" (7th EC FP CP FIRST-Nuclides), Budapest 09 - 11 October 2012 (KIT Scientific Reports ; 7639)

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    These proceedings document the scientific-technical progress of the 1st year of the European project FIRST-Nuclides. The project is established with the overall objective to provide for improved understanding of the fast / instantly released radionuclides from high burn-up spent nuclear fuel (HBU-SNF) under conditions of geological disposal. 14 HBU-SNF samples, discharged from various European and US American light water reactors, are characterized and prepared for experimental investigations

    4th Annual Workshop Proceedings of the Collaborative Project "Redox Phenomena Controlling Systems" (7th EC FP CP RECOSY) (KIT Scientific Reports ; 7626)

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    These are the proceedings of the 4th Annual Workshop of the EURATOM FP7 Collaborative Project \"Redox Phenomena Controlling System\", held in Karlsruhe (Germany) 23rd to 26th January 2012. The project deals with the impact of redox processes on the long-term safety of nuclear waste disposal. The proceedings have five workpackage overview contributions, and 24 reviewed scientific-technical short papers. The proceedings document the scientific-technical progress of the fourth project year

    3rd Annual Workshop Proceedings of the Collaborative Project "Redox Phenomena Controlling Systems" (7th EC FP CP RECOSY) (KIT Scientific Reports ; 7603)

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    These are the proceedings of the 3rd Annual Workshop of the EURATOM FP7 Collaborative Project "Redox Phenomena Controlling System", held in Balaruc-les-Bains (France) 21st to 24th March 2011. The project deals with the impact of redox processes on the long-term safety of nuclear waste disposal. The proceedings have five workpackage overview contributions, and 17 reviewed scientific-technical short papers. The proceedings document the scientific-technical progress of the third project year

    ICEM07-7109 ON THE ROLE OF H 2 AS AN INHIBITOR OF UO 2 MATRIX DISSOLUTION

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    ABSTRACT The study of spent fuel behaviour under disposal conditions is usually based on conservative approaches assuming oxidising conditions produced by water radiolysis at the fuel/water interface. However, the presence of H 2 from container corrosion can inhibit the dissolution of the UO 2 matrix and enhance its long-term stability. Several studies have confirmed the decrease in dissolution rates when H 2 is present in the system, although the exact mechanisms of interaction have not been fully established. This paper deals with a radiolytic modelling exercise to explore the consequences of the interaction of H 2 with radicals generated by radiolysis in the homogeneous phase. The main conclusion is that in all the modelled cases the presence of H 2 in the system leads to a decrease in matrix dissolution. The extent of the inhibition, and the threshold partial pressure for the inhibition to take place, both depend in a complex way on the chemical composition of the water and the type of radiation present in the syste

    2nd Annual Workshop Proceedings of the Collaborative Project "Redox Phenomena Controlling Systems" (7th EC FP CP RECOSY) (KIT Scientific Reports ; 7557)

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    These are proceedings of the 2nd Annual Workshop of the EURATOM FP7 Collaborative Project "Redox Phenomena Controlling System", held in Larnaca (Cyprus) 16th to 19th March 2010. The project deals with the impact of redox processes on the long-term safety of nuclear waste disposal. The proceedings have six workpackage overview contributions, and 21 reviewed scientific-technical short papers. The proceedings document the scientific-technical progress of the second project year

    The results of different labour induction approaches: A Cross sectional study

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    Background and Purpose: To evaluate the use of prostaglandins and oxytocin in labour induction according to different indications. Perinatal outcomes, rate of vaginal delivery and complation of labour were studied and compared. Methods: Cross-sectional descriptive study from January 2012 to December 2012. 530 women who required labour induction were included. Seven groups were created according to the methods of induction. Women with twin pregnancies, induction of dead foetus, two previous caesarean sections or an incomplete clinical history were excluded. Results: The rate of vaginal deliveries in women that only received prostaglandins the first day was 84.6%; similar in women with prolonged pregnancies, 85.2%. The induction with oxytocin directly showed the highest rate of caesarean section. The rate of vaginal deliveries was 50% in women with previous caesarean section. Conclusions: A high rate of vaginal deliveries with a single dose of prostaglandin and within 24 hours of beginning induction. Administration of prostaglandins must be used when cervix is unfavorable and previous to oxytocin stymulation

    Truncating FLNC Mutations Are Associated With High-Risk Dilated and Arrhythmogenic Cardiomyopathies

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    BACKGROUND: Filamin C (encoded by the FLNC gene) is essential for sarcomere attachment to the plasmatic membrane. FLNC mutations have been associated with myofibrillar myopathies, and cardiac involvement has been reported in some carriers. Accordingly, since 2012, the authors have included FLNC in the genetic screening of patients with inherited cardiomyopathies and sudden death. OBJECTIVES: The aim of this study was to demonstrate the association between truncating mutations in FLNC and the development of high-risk dilated and arrhythmogenic cardiomyopathies. METHODS: FLNC was studied using next-generation sequencing in 2,877 patients with inherited cardiovascular diseases. A characteristic phenotype was identified in probands with truncating mutations in FLNC. Clinical and genetic evaluation of 28 affected families was performed. Localization of filamin C in cardiac tissue was analyzed in patients with truncating FLNC mutations using immunohistochemistry. RESULTS: Twenty-three truncating mutations were identified in 28 probands previously diagnosed with dilated, arrhythmogenic, or restrictive cardiomyopathies. Truncating FLNC mutations were absent in patients with other phenotypes, including 1,078 patients with hypertrophic cardiomyopathy. Fifty-four mutation carriers were identified among 121 screened relatives. The phenotype consisted of left ventricular dilation (68%), systolic dysfunction (46%), and myocardial fibrosis (67%); inferolateral negative T waves and low QRS voltages on electrocardiography (33%); ventricular arrhythmias (82%); and frequent sudden cardiac death (40 cases in 21 of 28 families). Clinical skeletal myopathy was not observed. Penetrance was >97% in carriers older than 40 years. Truncating mutations in FLNC cosegregated with this phenotype with a dominant inheritance pattern (combined logarithm of the odds score: 9.5). Immunohistochemical staining of myocardial tissue showed no abnormal filamin C aggregates in patients with truncating FLNC mutations. CONCLUSIONS: Truncating mutations in FLNC caused an overlapping phenotype of dilated and left-dominant arrhythmogenic cardiomyopathies complicated by frequent premature sudden death. Prompt implantation of a cardiac defibrillator should be considered in affected patients harboring truncating mutations in FLNC.Instituto de Salud Carlos III [PI11/0699, PI14/0967, PI14/01477, RD012/0042/0029, RD012/0042/0049, RD012/0042/0066, RD12/0042/0069]; Spanish Ministry of Economy and Competitiveness [SAF2015-71863-REDT]; Plan Nacional de I+D+I; Plan Estatalde I+D+I, European Regional Development Fund; Health in Code SLS
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