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Infantile malignant osteopetrosis is a devastating disorder of early childhood that is frequently fatal and for which there are only limited therapeutic options. Gene therapy utilizing autologous hematopoietic stem and progenitor cells represents a potentially advantageous therapeutic alternative for this multisystemic disease. Gene therapy can be performed relatively rapidly following diagnosis,

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A recent literature emphasizes that gender differences in the labor market may in part be driven by a gender gap in willingness to compete. However, whereas experiments in this literature typically investigate willingness to compete in private environments, real world competitions often have a more public nature, which introduces potential social image concerns. If such image concerns are importan

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(1) Background: Although several neighborhood environmental factors have been identified to be associated with older adults’ physical activity, little research has been done in rural areas where the population is aging. This study aimed to investigate neighborhood environmental factors and the longitudinal change of physical activity status among rural older adults in Japan. (2) Meth-ods: The stud

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We introduce a new method to mitigate numerical diffusion in adaptive mesh refinement (AMR) simulations of cosmological galaxy formation, and study its impact on a simulated dwarf galaxy as part of the 'EDGE' project. The target galaxy has a maximum circular velocity of 21 km s-1 but evolves in a region that is moving at up to 90 km s-1 relative to the hydrodynamic grid. In the absence of any miti

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BACKGROUND: A hydraulic force generated by blood moving the atrio-ventricular plane is a novel mechanism of diastolic function. The direction and magnitude of the force is dependent on the geometrical relationship between the left atrium and ventricle and is measured as the short-axis atrio-ventricular area difference (AVAD). In short, the net hydraulic force acts from a larger area towards a smal

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Since the dawn of its existence, and at times thanks to ambitious interventions, Venice and its lagoon have needed to be constantly protected from the various ways in which water has reclaimed its existence. This article asserts that the ways in which Venice approached the watery world imply a tendency to relate to the natural environment as if it was something humans ought to separate themselves

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We present a new analog-to-digital converter (ADC)-based architecture of a phase-tracking receiver (PT-RX) optimized for ultra-low-power (ULP) and ultra-low-voltage (ULV) operations for the Internet of Things (IoT). The RX employs a type-II loop configuration that offers improved stability compared with the previous type-I PT-RX solutions. In addition, the type-II loop is also very tolerant of lon

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Objectives: To assess whether vessel morphology depicted by three-dimensional (3D) power Doppler ultrasound improves discrimination between benignity and malignancy if used as a second-stage test in adnexal masses that are difficult to classify. Methods: This was a prospective observational international multicenter diagnostic accuracy study. Consecutive patients with an adnexal mass underwent sta

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The US has experienced a substantial decline in social trust in recent decades. Surprisingly few studies analyze whether individual-level explanations can account for this decrease. We use three-wave panel data from the General Social Survey (2006–2014) to study the effects of four possible individual-level sources of changes in social trust: job loss, social ties, income, and confidence in politi

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OBJECTIVE: We investigated the processes underlying glycemic deterioration in type 2 diabetes (T2D). RESEARCH DESIGN AND METHODS: A total of 732 recently diagnosed patients with T2D from the Innovative Medicines Initiative Diabetes Research on Patient Stratification (IMI DIRECT) study were extensively phenotyped over 3 years, including measures of insulin sensitivity (OGIS), β-cell glucose sensiti

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Climate change is increasingly acknowledged as a threat to states and societies, and several international organizations are now seeking to respond to climate-related security risks. The EU’s comprehensive approach to security suggests that the Union is particularly well-suited to respond to such risks, but the EU has not yet developed a coherent policy. This article addresses the gap between disc

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In the report we analyse the concept of capability and compare it to relatedconcepts such as resilience. With starting point in a sociotechnical systemperspective as well as existing research on capability and vulnerability wedevelop an analytical framework that could be used to study the capability indifferent energy systems. We suggest that capability could be seen as composedof five “building b

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Under 2014 har det skett ett antal stora förändringar inom EU. Vissa är av mergenerell betydelse, såsom en ny sammansättning i Europaparlamentet ellertillsättningen av nya kommissionärer, medan andra reformer är av mer strukturellkaraktär i syfte att stärka EU:s ställning inom olika politikområden, däriblandEU:s strukturer för civil krishantering. Syftet med denna studie är dels attbeskriva dessa

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Att tala om energipolitik är ingen helt enkel uppgift. Beroende på vilkadimensioner av energifrågan som sätts i fokus kan diskussionerna handla omsåväl tillväxtpolitik, miljömål, tekniska innovationer – och säkerhet. För attnärma sig begreppet energisäkerhet krävs en förståelse av å ena sidanenergisystemet, vad det är som ska säkras, och å andra sidan säkerhetspolitik,vilka strategier som fungerar

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The impacts of climate change will not be evenly spread around the globe. Instead, several studies point to some specific regions that are argued to be particularly vulnerable or prone to climate change-related conflict (‘hotspots’) The overarching aim of this report is to describe these regions and to look at the underlying methodology used by those studies when identifying the regional ‘hotspots

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The focal and progressive degeneration of dopaminergic (DA) neurons in ventral midbrain has made Parkinson's disease (PD) a particularly interesting target of cell-based therapies. However, ethical issues and limited tissue availability have so far hindered the widespread use of human fetal tissue in cell-replacement therapy. DA neurons derived from human pluripotent stem cells (hPSCs) offer unpre