105 research outputs found

    Malignant pheochromocytoma in a 16-year-old patient with neurofibromatosis type 1

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    The Impact of Specific Viruses on Clinical Outcome in Children Presenting with Acute Heart Failure

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    Abstract: The presence and type of viral genomes have been suggested as the main etiology for inflammatory dilated cardiomyopathy. Information on the clinical implication of this finding in a large population of children is lacking. We evaluated the prevalence, type, and clinical impact of specific viral genomes in endomyocardial biopsies (EMB) collected between 2001 and 2013 among 63 children admitted to our hospital for acute heart failure (median age 2.8 years). Viral genome was searched by polymerase chain reaction (PCR). Patients underwent a complete two-dimensional echocardiographic examination at hospital admission and at discharge and were followed-up for 10 years. Twenty-seven adverse events (7 deaths and 20 cardiac transplantations) occurred during the follow-up. Viral genome was amplified in 19/63 biopsies (35%); PVB19 was the most commonly isolated virus. Presence of specific viral genome was associated with a significant recovery in ejection fraction, compared to patients without viral evidence (p < 0.05). In Cox-regression analysis, higher survival rate was related to virus-positive biopsies (p < 0.05). When comparing long-term prognosis among different viral groups, a trend towards better prognosis was observed in the presence of isolated Parvovirus B19 (PVB19) (p = 0.07). In our series, presence of a virus-positive EMB (mainly PVB19) was associated with improvement over time in cardiac function and better long-term prognosis

    Mineralogical, geochemical, and isotopic characteristics of the ejecta from the 5 April 2003 paroxysm at Stromboli, Itlay: Inferences on the Preeruptive Magma Dynamics

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    The 5 April 2003 explosive eruption at Stromboli emplaced typical basaltic scoria, pumice, and lithic blocks. This paper reports a detailed set of mineralogical, geochemical, and isotopic data on the juvenile ejecta and fresh subvolcanic blocks, including micro-Sr isotope analyses and major and dissolved volatile element contents in olivine-hosted melt inclusions. The juvenile ejecta have compositions similar to those of their analogs from previous paroxysms; the 2003 pumice, however, does not contain stable high-MgO olivine, usually typical of large-scale paroxysms and has lower compatible element contents. Texture, composition, and Sr isotope disequilibrium of crystals in pumice indicate that most of them are inherited from the shallow crystal-rich magma and/or crystal mush. The most primitive magma is recorded as rare melt inclusion in olivine Fo85–86. It has a typical S/Cl (1.1) and a total volatile content of 3.1 wt % from which the total fluid pressure was evaluated ≥240 MPa. Hence, moderate pressure conditions can be envisaged for the mechanism triggering the April 2003 paroxysm. The subvolcanic blocks are shoshonitic basalts with 45–50 vol % of phenocrysts (plagioclase + clinopyroxene + olivine). The late-stage crystallization of the crystal-rich magma lead to the formation of Na-sanidine with plagioclase An60–25 + olivine Fo68–49 + Timagnetite ± apatite ± phlogopite ± ilmenite assemblage. Mineralogy, chemistry, and Sr–Nd isotopic signatures of the subvolcanic blocks indicate they represent the slowly cooled equivalents of batches of crystal-rich basaltic magma stored in the uppermost subvolcanic feeding system. Cooling might be facilitated by short breaks in the summit crater activity

    Pre-eruptive magmatic processes re-timed using a non-isothermal approach to magma chamber dynamics

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    Open Source PaperThis work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/. The attached file is the published version of the article

    Evaluating Process Quality Based on Change Request Data – An Empirical Study of the Eclipse Project

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    Abstract. The information routinely collected in change request management systems contains valuable information for monitoring of the process quality. However this data is currently utilized in a very limited way. This paper presents an empirical study of the process quality in the product portfolio of the Eclipse project. It is based on a systematic approach for the evaluation of process quality characteristics using change request data. Results of the study offer insights into the development process of Eclipse. Moreover the study allows assessing applicability and limitations of the proposed approach for the evaluation of process quality

    Problemática actual de los intereses moratorios en los préstamos hipotecarios

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    El trabajo aborda la problemática en cuanto a la fijación de un límite para la declaración de abusividad de la cláusula que establece los intereses moratorios en el marco de un contrato de préstamo hipotecario para la adquisición de la vivienda actual. Para ello, primero hace un análisis de la regulación existente así como de la jurisprudencia tanto del TS como del TJUE, de cuáles son sus convergencias y divergencias. Concluyendo con el análisis de la evolución de la regulación de esta materia desde anteproyecto de ley a la aprobación de la ley 5/2019, pasando por las diversas enmiendas y el informe emitido por el Consejo del Poder Judicial. Fianalmente, la citada nueva ley parece poner un límite claro a estos intereses, fijándolos en el interés remuneratorio más tres puntos porcentuales.Grado en Derecho y Grado en Dirección y Administración de Empresa

    KRIT1 Regulates the Homeostasis of Intracellular Reactive Oxygen Species

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    KRIT1 is a gene responsible for Cerebral Cavernous Malformations (CCM), a major cerebrovascular disease characterized by abnormally enlarged and leaky capillaries that predispose to seizures, focal neurological deficits, and fatal intracerebral hemorrhage. Comprehensive analysis of the KRIT1 gene in CCM patients has suggested that KRIT1 functions need to be severely impaired for pathogenesis. However, the molecular and cellular functions of KRIT1 as well as CCM pathogenesis mechanisms are still research challenges. We found that KRIT1 plays an important role in molecular mechanisms involved in the maintenance of the intracellular Reactive Oxygen Species (ROS) homeostasis to prevent oxidative cellular damage. In particular, we demonstrate that KRIT1 loss/down-regulation is associated with a significant increase in intracellular ROS levels. Conversely, ROS levels in KRIT1−/− cells are significantly and dose-dependently reduced after restoration of KRIT1 expression. Moreover, we show that the modulation of intracellular ROS levels by KRIT1 loss/restoration is strictly correlated with the modulation of the expression of the antioxidant protein SOD2 as well as of the transcriptional factor FoxO1, a master regulator of cell responses to oxidative stress and a modulator of SOD2 levels. Furthermore, we show that the KRIT1-dependent maintenance of low ROS levels facilitates the downregulation of cyclin D1 expression required for cell transition from proliferative growth to quiescence. Finally, we demonstrate that the enhanced ROS levels in KRIT1−/− cells are associated with an increased cell susceptibility to oxidative DNA damage and a marked induction of the DNA damage sensor and repair gene Gadd45α, as well as with a decline of mitochondrial energy metabolism. Taken together, our results point to a new model where KRIT1 limits the accumulation of intracellular oxidants and prevents oxidative stress-mediated cellular dysfunction and DNA damage by enhancing the cell capacity to scavenge intracellular ROS through an antioxidant pathway involving FoxO1 and SOD2, thus providing novel and useful insights into the understanding of KRIT1 molecular and cellular functions

    Reconstruction of the eruptive activity on the NE sector of Stromboli volcano: timing of flank eruptions since 15 ka

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    A multidisciplinary geological and compositional investigation allowed us to reconstruct the occurrence of flank eruptions on the lower NE flank of Stromboli volcano since 15 ka. The oldest flank eruption recognised is Roisa, which occurred at ~15 ka during the Vancori period, and has transitional compositional characteristics between the Vancori and Neostromboli phases. Roisa was followed by the San Vincenzo eruption that took place at ~12 ka during the early stage of Neostromboli period. The eruptive fissure of San Vincenzo gave rise to a large scoria cone located below the village of Stromboli, and generated a lava flow, most of which lies below sea level. Most of the flank eruptions outside the barren Sciara del Fuoco occurred in a short time, between ~9 and 7 ka during the Neostromboli period, when six eruptive events produced scoria cones, spatter ramparts and lava flows. The Neostromboli products belong to a potassic series (KS), and cluster in two differently evolved groups. After an eruptive pause of ~5,000 years, the most recent flank eruption involving the NE sector of the island occurred during the Recent Stromboli period with the formation of the large, highly K calc-alkaline lava flow field, named San Bartolo. The trend of eruptive fissures since 15 ka ranges from N30°E to N55°E, and corresponds to the magma intrusions radiating from the main feeding system of the volcano
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