OBJECTIVE: To describe the clinical findings in a patient who developed a neurofibroma, meningioma, and choroidal melanoma. METHODS: Clinical and histopathological findings of the case are reviewed and presented. RESULTS: The patient had a right superolateral periorbital neurofibroma, a right sphenoid wing meningioma, and a left choroidal juxtapapillary malignant melanoma. All 3 tumors are derived from neural crest cells. CONCLUSIONS: To our knowledge, this is the first report of a patient with this combination of 3 neural crest-derived tumors. This case is most appropriately classified as a complex neurocristopathy, a disorder involving the aberrant and pathological proliferation of multiple tissues derived from neural crest cells. 相似文献
Jennifer Bernhard welcomed the new AdCom members: Peter de Maagt, George Elefiheraides, Jay Kralovec, Giuliano Manara, and Prabhakar Pathak; and the new President Elect, Makoto Ando. She also thanked the out-going Past President, L. Wilson Pearson and the out-going AdComn members: Mike Jensen, Leo Kempel, Brian Kent, Eric Michielssen, and Tapan Sarkar for their four years of service. Several committee chairs have been changed: Jeff Young will be replaced as Chapter Activities Chair by Leo Kempel; Wilson Pearson will be replaced as Publications Chair by Allen Glisson; Lance Griffiths will be replaced by Ryan Adams as the GOLD representative; Magdy Iskander will be replaced by Andy Peterson as Awards Committee Chair; Dan Weile has resigned as co-Chair of the Membership Committee; and Ozlem. Kilic is replacing Jennifer Bernhard as Chair of the Constitution and Bylaws Committee. 相似文献
Emerging pharmacogenetics research may improve clinical outcomes for common complex conditions typically treated in primary care settings. Physicians' willingness to offer genetically-tailored treatments to their patients will be critical to realizing this potential. According to recent research, it is likely that genotypes used to tailor smoking will have pleiotropic associations with other addictions and diseases, and may have different frequencies across populations. These additional features may pose an additional barrier to adoption. To assess physicians' willingness to offer a new test to individually tailor smoking treatment according to specific test characteristics, we conducted a national mailed survey of 2,000 U.S. primary care physicians (response rate: 62.3%). Physicians responded to a baseline scenario describing a new test to tailor smoking treatment, and three additional scenarios describing specific test characteristics based on published research; there was random assignment to one of two survey conditions in which the test was described as a genetic or non-genetic test. Our findings indicate physicians' self-reported likelihood (0-100 scale) that they would offer a new test to tailor smoking cessation treatment ranged from 69%-78% across all scenarios. Relative to baseline scenario responses, physicians were significantly less likely to offer the test when informed that the same genotypes assessed for treatment tailoring: (1) may also identify individuals predisposed to become addicted to nicotine (p<.001), (2) differ in frequency by race (p<.004), and (3) may have associations with other conditions (e.g., alcohol and cocaine addiction, attention deficit hyperactivity disorder ADHD and Tourette Syndrome) (p<.01). Describing a new test to individually tailor smoking treatment as a "genetic" versus non-genetic test significantly reduced physicians' likelihood of offering the test across all scenarios, regardless of specific test characteristics (p<.0007). Effective education of primary care physicians will be critical to successful integration of promising new pharmacogenetic treatment strategies for smoking. 相似文献
Herein, we describe a reduced‐scale test (“Cube” test), measuring the fire performance of specimens including a fire barrier (FB) and a flammable core material, which acts as the main fuel load. The specimen is intended to reproduce a cross‐section of a composite product where heat/mass transfer occurs primarily in a direction perpendicular to the FB. The Cube test procedure and benefits are discussed in this work by adopting residential upholstery furniture as an exemplary study. One flexible polyurethane foam, one polypropylene cover fabric, and 10 commercially available FBs were selected. They were used to compare the fire performance of FBs, measured in terms of peak of heat release rate, in the ASTM E1474‐14 standard test and the newly developed Cube test. Edge effects severely affected the performance of FBs in the ASTM E1474‐14 standard test but not in the Cube test. Furthermore, appropriate test conditions were determined in the Cube test to measure the so‐called “wetting point,” that is, the time and value of heat release rate measured when flammable liquid products were first observed on the bottom of the specimen. The relevance of the “wetting point” in terms of full‐scale fire performance and failure mechanism of FBs is discussed. 相似文献
In this work, an adaptive simplex stochastic collocation method is introduced in which sample refinement is informed by variability in the solution of the system. The proposed method is based on the concept of multi-element stochastic collocation methods and is capable of dealing with very high-dimensional models whose solutions are expressed as a vector, a matrix, or a tensor. The method leverages random samples to create a multi-element polynomial chaos surrogate model that incorporates local anisotropy in the refinement, informed by the variance of the estimated solution. This feature makes it beneficial for strongly nonlinear and/or discontinuous problems with correlated non-Gaussian uncertainties. To solve large systems, a reduced-order model (ROM) of the high-dimensional response is identified using singular value decomposition (higher-order SVD for matrix/tensor solutions) and polynomial chaos is used to interpolate the ROM. The method is applied to several stochastic systems of varying type of response (scalar/vector/matrix) and it shows considerable improvement in performance compared to existing simplex stochastic collocation methods and adaptive sparse grid collocation methods. 相似文献
Leukodystrophies are progressive disorders involving the development and maintenance of myelin in the central and peripheral nervous systems. Although relatively uncommon, leukodystrophic disorders may be undiagnosed or misdiagnosed during life, and may appear as "sudden death." In such instances, these victims may be referred to a forensic pathologist. In general, leukodystrophies are inherited in an autosomal recessive manner so that proper postmortem diagnosis by the forensic pathologist is extremely important to the decendant's family for future family planning. 相似文献
We propose a novel hybrid model that exploits the strength of discriminative classifiers along with the representation power of generative models. Our focus is on detecting multimodal events in time varying sequences as well as generating missing data in any of the modalities. Discriminative classifiers have been shown to achieve higher performances than the corresponding generative likelihood-based classifiers. On the other hand, generative models learn a rich informative space which allows for data generation and joint feature representation that discriminative models lack. We propose a new model that jointly optimizes the representation space using a hybrid energy function. We employ a Restricted Boltzmann Machines (RBMs) based model to learn a shared representation across multiple modalities with time varying data. The Conditional RBMs (CRBMs) is an extension of the RBM model that takes into account short term temporal phenomena. The hybrid model involves augmenting CRBMs with a discriminative component for classification. For these purposes we propose a novel Multimodal Discriminative CRBMs (MMDCRBMs) model. First, we train the MMDCRBMs model using labeled data by training each modality, followed by training a fusion layer. Second, we exploit the generative capability of MMDCRBMs to activate the trained model so as to generate the lower-level data corresponding to the specific label that closely matches the actual input data. We evaluate our approach on ChaLearn dataset, audio-mocap, as well as the Tower Game dataset, mocap-mocap as well as three multimodal toy datasets. We report classification accuracy, generation accuracy, and localization accuracy and demonstrate its superiority compared to the state-of-the-art methods. 相似文献
The escape potential of occupants in a building and the effectiveness of existing escape strategies can be described as a function of the time required for escape and the time available for escape.
The formalisation of these two concepts within the contextual framework of equivalency is addressed in this paper. Existing fire simulation and people evacuation models (ASET and EVACNET+, respectively) are used to assess equivalent fire safety provision in public assembly buildings.
In each of the examples chosen, a safety index is derived for deemed-to-satisfy designs and used as a benchmark against which other prototype designs may be compared for the purpose of establishing code equivalency.
A methodology that is capable of assisting in the determination of code equivalency with respect to escape route provision while accommodating trade-off is postulated. 相似文献
A unique experimental apparatus has been constructed in order to generate a controlled and repeatable size and mass distribution of glowing firebrands. The present study reports on a series of experiments conducted in order to characterize the performance of this firebrand generator. Firebrand generator characterization experiments were performed at the Fire Research Wind Tunnel Facility (FRWTF) at the Building Research Institute (BRI) in Tsukuba, Japan. The firebrand generator was fed with three different initial firebrand geometries, two different sized cylinders and one size of disks. Cylinders were used to simulate firebrand fluxes from vegetation, such as trees, while disks were used to simulate a firebrand flux from burning structures. Samples of these geometries were constructed from wood dowels, fed into the firebrand generator, ignited, and the glowing firebrands generated were collected using an array of water filled pans. The pans were filled with water in order to quench combustion. The collected firebrands were subsequently dried and the size and mass distribution was measured. These experiments were performed over a range of wind tunnel speeds, with no wind speed present to 9 m/s, to determine the lofting distance of the firebrands generated. Finally, the size and mass distribution produced from the firebrand generator are compared to those produced from burning trees. Results of the study are presented and discussed. 相似文献
The Institute of Fire Safety Engineering Research and Technology Centre (FireSERT) at the University of Ulster in Northern Ireland has been a major provider of higher technical fire safety education, fire related research and services to industry for over 20 years. Its internationally recognised research spans the fields of fire dynamics, structural fire engineering, human behaviour in fire and more recently fire (CFD) modelling. Since 1991, it has offered the only PG Dip/MSc Fire Safety Engineering programme in the United Kingdom. In September 2000 FireSERT was the recipient of a £5.7 m Joint Infrastructure Fund (JIF) Award to build new fire safety engineering laboratories on the University's Jordanstown campus. These new facilities unparallel in the University sector in the UK offer greater scope and depth to the FireSERT's research portfolios. The new facilities will be extensively used in support of FireSERT's suite of MSc Fire Safety Engineering and DPhil programmes. This paper presents a brief history of FireSERT and its achievements, together with an update on the new facility, current research themes, taught programmes, research opportunities and visions for the future. 相似文献