Results of the RAMI analyses performed for the IFMIF accelerator facility in the engineering design phase

This paper presents a summary of the RAMI (Reliability Availability Maintainability Inspectability) analyses done for the IFMIF (International Fusion Materials Irradiation Facility) Accelerator facility in the Engineering Design Phase. The methodology followed, the analyses performed, the results obtained and the conclusions drawn are described. Moreover, the consequences of the incorporation of the RAMI studies in the IFMIF design are presented and the main outcomes of these analyses are shown. (C) 2015 Elsevier B.V. All rights reserved.Postprint (author's final draft

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

Ciencias Sociales: Economía y Humanidades HANDBOOK T-I

Se presenta un breve examen de la producción y comercialización de rosa en México; un estudio en México sobre el ingreso mínimo de las familias que identifica la línea de pobreza alimentaria en el área rural del sur de México, 2012; un pequeño estudio donde hablará sobre el análisis comparado del Sector Gubernamental y la Economía Mexicana desde la perspectiva de los eslabonamientos productivos Hirshman-Rasmuss; un estudio sobre los canales de comercialización de limón persa en el municipio de Martínez de la Torre, Veracruz; una análisis del comercio estratégico en el TLCAN: El Estado en la política agrícola de biocombustibles; también se expresan acerca de la importancia de la comercialización del café en México; un diagnóstico, retos del comercio electrónico en el Sector Agroindustrial Mexicano; trabajo nos muestra y habla sobre la inversión extranjera directa y su impacto en crecimiento de México, un análisis en prospectiva: 1999-2010; un estudio acerca sobre la importancia de la Banca en México; un trabajo acerca de la competitividad de la producción agrícola en México, un análisis regional; se analizan todo acerca de el SIAL productor de quesos en Poxtla, competividad y territorio; se habla acerca de la intermediación financiera al servicio de la comunidad indígena: el fondo regional indígena Tarhiata Keri; ademas un estudio acerca de la demanda de Importaciones de durazno (Prunus pérsica L. Batsch) en México procedentes de Estados Unidos de América (1982-2011); Loera y Sepúlveda analizan los parámetros de la productividad forestal en la producción de madera en rollo; un análisis de factores sociales, ambientales y económicos del territorio rural cercano a la ciudad de México; un estudio acerca de la crisis económica mundial y su efecto sobre los flujos migratorios de América Latina; Magadán, Hernández y Escalona presentan la tipología de los sujetos sociales que intervienen en el mercado campesino de Ocotlán Oaxaca; la normalización del proceso de compostaje: una opción para desarrollar el mercado de la composta; acerca de la reestructuración del capitalismo y crisis política en México; la rentabilidad de la producción de miel en el municipio de León, Guanjuato; la economía del maíz en la región metropolitana, Chiapas, 2014; análisis de los centros de educación y cultura ambiental, necesidad de profesionalización Pedagógica de facilitadores ambientales; los Costos y competitividad de la producción del limón persa en el municipio de Martínez de la Torre, Veracruz

Hardware availability calculations and results of the IFMIF accelerator facility

Hardware availability calculations have been done individually for each system of the deuteron accelerators of the International Fusion Materials Irradiation Facility (IFMIF). The principal goal of these analyses is to estimate the availability of the systems, compare it with the challenging IFMIF requirements and find new paths to improve availability performances.; Major unavailability contributors are highlighted and possible design changes are proposed in order to achieve the hardware availability requirements established for each system. In this paper, such possible improvements are implemented in fault tree models and the availability results are evaluated.; The parallel activity on the design and construction of the linear IFMIF prototype accelerator (LIPAc) provides detailed design information for the RAMI (reliability, availability, maintainability and inspectability) analyses and allows finding out the improvements that the final accelerator could have. Because of the R&D behavior of the LIPAc, RAMI improvements could be the major differences between the prototype and the IFMIF accelerator design.Peer ReviewedPostprint (published version

Results of the RAMI analyses performed for the IFMIF accelerator facility in the engineering design phase

This paper presents a summary of the RAMI (Reliability Availability Maintainability Inspectability) analyses done for the IFMIF (International Fusion Materials Irradiation Facility) Accelerator facility in the Engineering Design Phase. The methodology followed, the analyses performed, the results obtained and the conclusions drawn are described. Moreover, the consequences of the incorporation of the RAMI studies in the IFMIF design are presented and the main outcomes of these analyses are shown. (C) 2015 Elsevier B.V. All rights reserved

Hardware availability calculations and results of the IFMIF accelerator facility

Hardware availability calculations have been done individually for each system of the deuteron accelerators of the International Fusion Materials Irradiation Facility (IFMIF). The principal goal of these analyses is to estimate the availability of the systems, compare it with the challenging IFMIF requirements and find new paths to improve availability performances.; Major unavailability contributors are highlighted and possible design changes are proposed in order to achieve the hardware availability requirements established for each system. In this paper, such possible improvements are implemented in fault tree models and the availability results are evaluated.; The parallel activity on the design and construction of the linear IFMIF prototype accelerator (LIPAc) provides detailed design information for the RAMI (reliability, availability, maintainability and inspectability) analyses and allows finding out the improvements that the final accelerator could have. Because of the R&D behavior of the LIPAc, RAMI improvements could be the major differences between the prototype and the IFMIF accelerator design.Peer Reviewe

Availability simulation software adaptation to the IFMIF accelerator facility RAMI analyses

Several problems were found when using generic reliability tools to perform RAM! (Reliability Availability Maintainability Inspectability) studies for the IFMIF (International Fusion Materials Irradiation Facility) accelerator. A dedicated simulation tool was necessary to model properly the complexity of the accelerator facility.; AvailSim, the availability simulation software used for the International Linear Collider (ILC) became an excellent option to fulfill RAMI analyses needs. Nevertheless, this software needed to be adapted and modified to simulate the IFMIF accelerator facility in a useful way for the RAMI analyses in the current design phase. Furthermore, some improvements and new features have been added to the software. This software has become a great tool to simulate the peculiarities of the IFMIF accelerator facility allowing obtaining a realistic availability simulation. Degraded operation simulation and maintenance strategies are the main relevant features.; In this paper, the necessity of this software, main modifications to improve it and its adaptation to IFMIF RAMI analysis are described. Moreover, first results obtained with AvailSim 2.0 and a comparison with previous results is shown.Peer Reviewe