Intimal Sarcoma of the Infrarenal Aorta: Report of a Rare Single Case and Review of the Literature

AbstractPrimary malignant tumours of the abdominal aorta are rare and they are usually associated with a poor one-year survival rate. The histological classification of aortic sarcomas (angiosarcoma/intimal sarcoma) is complex and does not appear to affect the prognosis. We present a case of a 68-year-old man with an infrarenal intimal sarcoma with peripheral embolization. The world literature on primary intimal malignancies of the abdominal aorta is reviewed

Spent Fuel Transportation Package Response to the Baltimore Tunnel Fire Scenario

On July 18, 2001, a freight train carrying hazardous (non-nuclear) materials derailed and caught fire while passing through the Howard Street railroad tunnel in downtown Baltimore, Maryland. The United States Nuclear Regulatory Commission (USNRC), one of the agencies responsible for ensuring the safe transportation of radioactive materials in the United States, undertook an investigation of the train derailment and fire to determine the possible regulatory implications of this particular event for the transportation of spent nuclear fuel by railroad. Shortly after the accident occurred, the USNRC met with the National Transportation Safety Board (NTSB, the U.S. agency responsible for determining the cause of transportation accidents), to discuss the details of the accident and the ensuing fire. Following these discussions, the USNRC assembled a team of experts from the National Institute of Standards and Technology (NIST), the Center for Nuclear Waste Regulatory Analyses (CNWRA), and Pacific Northwest National Laboratory (PNNL) to determine the thermal conditions that existed in the Howard Street tunnel fire and analyze the effects of this fire on various spent fuel transportation package designs. The Fire Dynamics Simulator (FDS) code, developed by NIST, was used to determine the thermal environment present in the Howard Street tunnel during the fire. The FDS results were used as boundary conditions in the COBRA-SFS and ANSYS® computer codes to evaluate the thermal performance of different package designs. The staff concluded that larger transportation packages resembling the HOLTEC Model No. HI STAR 100 and TransNuclear Model No. TN-68 would withstand a fire with thermal conditions similar to those that existed in the Baltimore tunnel fire event with only minor damage to peripheral components. This is due to their sizable thermal inertia and design specifications in compliance with currently imposed regulatory requirements. The staff also concluded that some components of smaller transportation packages resembling the NAC Model No. LWT, despite placement within an ISO container, could degrade. USNRC staff evaluated the radiological consequences of the package responses to the Baltimore tunnel fire. Though components in some packages heated up beyond their service temperatures, the staff determined that there would be no significant dose as a result of the fire for any of these and similar packages

Selection of Oncogenic Mutant Clones in Normal Human Skin Varies with Body Site

Skin cancer risk varies substantially across the body, yet how this relates to the mutations found in normal skin is unknown. Here we mapped mutant clones in skin from high- and low-risk sites. The density of mutations varied by location. The prevalence of NOTCH1 and FAT1 mutations in forearm, trunk, and leg skin was similar to that in keratinocyte cancers. Most mutations were caused by ultraviolet light, but mutational signature analysis suggested differences in DNA-repair processes between sites. Eleven mutant genes were under positive selection, with TP53 preferentially selected in the head and FAT1 in the leg. Fine-scale mapping revealed 10% of clones had copy-number alterations. Analysis of hair follicles showed mutations in the upper follicle resembled adjacent skin, but the lower follicle was sparsely mutated. Normal skin is a dense patchwork of mutant clones arising from competitive selection that varies by location. / Significance: Mapping mutant clones across the body reveals normal skin is a dense patchwork of mutant cells. The variation in cancer risk between sites substantially exceeds that in mutant clone density. More generally, mutant genes cannot be assigned as cancer drivers until their prevalence in normal tissue is known

The CDK inhibitor CR8 acts as a molecular glue degrader that depletes cyclin K

Molecular glue compounds induce protein-protein interactions that, in the context of a ubiquitin ligase, lead to protein degradation1. Unlike traditional enzyme inhibitors, these molecular glue degraders act substoichiometrically to catalyse the rapid depletion of previously inaccessible targets2. They are clinically effective and highly sought-after, but have thus far only been discovered serendipitously. Here, through systematically mining databases for correlations between the cytotoxicity of 4,518 clinical and preclinical small molecules and the expression levels of E3 ligase components across hundreds of human cancer cell lines3-5, we identify CR8-a cyclin-dependent kinase (CDK) inhibitor6-as a compound that acts as a molecular glue degrader. The CDK-bound form of CR8 has a solvent-exposed pyridyl moiety that induces the formation of a complex between CDK12-cyclin K and the CUL4 adaptor protein DDB1, bypassing the requirement for a substrate receptor and presenting cyclin K for ubiquitination and degradation. Our studies demonstrate that chemical alteration of surface-exposed moieties can confer gain-of-function glue properties to an inhibitor, and we propose this as a broader strategy through which target-binding molecules could be converted into molecular glues

PV self-consumption optimization with storage and Active DSM for the residential sector

With the rising prices of the retail electricity and the decreasing cost of the PV technology, grid parity with commercial electricity will soon become a reality in Europe. This fact, together with less attractive PV feed-in-tariffs in the near future and incentives to promote self-consumption suggest, that new operation modes for the PV Distributed Generation should be explored; differently from the traditional approach which is only based on maximizing the exported electricity to the grid. The smart metering is experiencing a growth in Europe and the United States but the possibilities of its use are still uncertain, in our system we propose their use to manage the storage and to allow the user to know their electrical power and energy balances. The ADSM has many benefits studied previously but also it has important challenges, in this paper we can observe and ADSM implementation example where we propose a solution to these challenges. In this paper we study the effects of the Active Demand-Side Management (ADSM) and storage systems in the amount of consumed local electrical energy. It has been developed on a prototype of a self-sufficient solar house called “MagicBox” equipped with grid connection, PV generation, lead–acid batteries, controllable appliances and smart metering. We carried out simulations for long-time experiments (yearly studies) and real measures for short and mid-time experiments (daily and weekly studies). Results show the relationship between the electricity flows and the storage capacity, which is not linear and becomes an important design criterion

The Mu3e Data Acquisition

The Mu3e experiment aims to find or exclude the lepton flavor violating decay μ+→e+e−e+ with a sensitivity of one in 10 16 muon decays. The first phase of the experiment is currently under construction at the Paul Scherrer Institute (PSI, Switzerland), where beams with up to 10 8 muons per second are available. The detector will consist of an ultra-thin pixel tracker made from High-Voltage Monolithic Active Pixel Sensors (HV-MAPS) , complemented by scintillating tiles and fibers for precise timing measurements. The experiment produces about 100Gbit/s of zero-suppressed data, which are transported to a filter farm using a network of field programmable gate arrays (FPGAs) and fast optical links. On the filter farm, tracks and three-particle vertices are reconstructed using highly parallel algorithms running on graphics processing units, leading to a reduction of the data to 100 Mbyte/s for mass storage and offline analysis. This article introduces the system design and hardware implementation of the Mu3e data acquisition and filter farm

Civil Aircraft for the regular investigation of the atmosphere based on an instrumented container: The new CARIBIC system

An airfreight container with automated instruments for measurement of atmospheric gases and trace compounds was operated on a monthly basis onboard a Boeing 767-300 ER of LTU International Airways during long-distance flights from 1997 to 2002 (CARIBIC, Civil Aircraft for Regular Investigation of the Atmosphere Based on an Instrument Container, http://www.caribic-atmospheric.com). Subsequently a more advanced system has been developed, using a larger capacity container with additional equipment and an improved inlet system. CARIBIC phase #2 was implemented on a new long-range aircraft type Airbus A340-600 of the Lufthansa German Airlines (Star Alliance) in December 2004, creating a powerful flying observatory. The instrument package comprises detectors for the measurement of O3, total and gaseous H2O, NO and NOy, CO, CO2, O2, Hg, and number concentrations of sub-micrometer particles (>4 nm, >12 nm, and >18 nm diameter). Furthermore, an optical particle counter (OPC) and a proton transfer mass spectrometer (PTR-MS) are incorporated. Aerosol samples are collected for analysis of elemental composition and particle morphology after flight. Air samples are taken in glass containers for laboratory analyses of hydrocarbons, halocarbons and greenhouse gases (including isotopic composition of CO2) in several laboratories. Absorption tubes collect oxygenated volatile organic compounds. Three differential optical absorption spectrometers (DOAS) with their telescopes mounted in the inlet system measure atmospheric trace gases such as BrO, HONO, and NO2. A video camera mounted in the inlet provides information about clouds along the flight track. The flying observatory, its equipment and examples of measurement results are reported