McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing syndrome, as well as other less common features. The combination of any of these findings, with or without FD, is known as McCune-Albright syndrome (MAS). The broad spectrum of involved tissues and the unpredictable combination of findings owe to the fact that molecular defect is due to dominant activating mutations in the widely expressed signaling protein, Gsα, and the fact these mutations arises sporadically, often times early in development, prior to gastrulation, and can distribute across many or few tissues

Radar signal categorization using a neural network

Neural networks were used to analyze a complex simulated radar environment which contains noisy radar pulses generated by many different emitters. The neural network used is an energy minimizing network (the BSB model) which forms energy minima - attractors in the network dynamical system - based on learned input data. The system first determines how many emitters are present (the deinterleaving problem). Pulses from individual simulated emitters give rise to separate stable attractors in the network. Once individual emitters are characterized, it is possible to make tentative identifications of them based on their observed parameters. As a test of this idea, a neural network was used to form a small data base that potentially could make emitter identifications

In situ propellant production: Alternatives for Mars exploration

Current planning for the Space Exploration Initiative (SEI) recognizes the need for extraterrestrial resources to sustain long-term human presence and to attain some degree of self-sufficiency. As a practical matter, reducing the need to carry large supplies of propellant from Earth will make space exploration more economical. For nearly every round trip planned with conventional propulsion, the actual payload is only a small fraction - perhaps 10-15 percent - of the mass launched from Earth. The objective of this study was to analyze the potential application for SEI missions of propellants made exclusively from lunar or martian resources. Using such propellants could minimize or eliminate the cost of carrying propellant for surface excursion vehicles and return transfers through two high-energy maneuvers: Earth launch and trans-Mars injection. Certain chemical mono- and bipropellants are candidates for this approach; they could be recovered entirely from in situ resources on the Moon and Mars, without requiring a continuing Earth-based resupply of propellant constituents (e.g., fuel to mix with a locally obtained oxidizer) and, perhaps, with minimal need to resupply consumables (e.g., reagents or catalyst for process reactions). A complete assessment of the performance potential of these propellants must include the requirements for installation, operations, maintenance, and resupply of the chemical processing facility

Electrostatic Modulation of the Electronic Properties of Dirac Semimetal Na3Bi

Large-area thin films of topological Dirac semimetal Na$_3$Bi are grown on amorphous SiO$_2$:Si substrates to realise a field-effect transistor with the doped Si acting as back gate. As-grown films show charge carrier mobilities exceeding 7,000 cm$^2$/Vs and carrier densities below 3 $\times$10$^{18}$ cm$^{-3}$, comparable to the best thin-film Na$_3$Bi. An ambipolar field effect and minimum conductivity are observed, characteristic of Dirac electronic systems. The results are quantitatively understood within a model of disorder-induced charge inhomogeneity in topological Dirac semimetals. Due to the inverted band structure, the hole mobility is significantly larger than the electron mobility in Na$_3$Bi, and when present, these holes dominate the transport properties.Comment: 5 pages, 4 figures; minor corrections and revisions for readabilit

Dental Perspectives in Fibrous Dysplasia and McCune-Albright Syndrome

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by the triad of polyostotic fibrous dysplasia (PFD), endocrine disorders and café-au-lait skin pigmentation. Ninety percent of MAS patients have FD lesions in the craniofacial area, resulting in significant orofacial deformity, dental disorders, bone pain and compromised oral health. Maxillo-mandibular FD is also associated with dental developmental disorders, malocclusion, and high caries index. There is limited data on the outcomes of dental treatments in maxillo-mandibular FD/MAS patients, because clinicians and researchers have limited access to patients, and there are concerns that dental surgery may activate quiescent jaw FD lesions to grow aggressively. This report highlights current perspectives on dental management issues associated with maxillo-mandibular FD within the context of MAS

Thermal Resistance of a Window with an Enclosed Venetian Blind: A Simplified Model

©2008, American Society of Heating, Refrigerating and Air-Conditioning Engineers, Inc. (www.ashrae.org). Published in ASHRAE Transactions, Volume 114, Part 1. For personal use only. Additional reproduction, distribution, or transmission in either print or digital form is not permitted without ASHRAE’s prior written permission.Solar gain has a strong influence on building energy consumption and peak cooling load. Venetian blinds are routinely used to control solar gain. Software based on 1-D models is available to accurately predict the thermal performance of glazing systems but the development of models for shading devices is at a very early stage. An accurate model has been formulated to quantify the thermal resistance of a glazing system with an enclosed venetian blind. It is possible to account for pane spacing, slat angle, alternate fill gases and the presence of a low-emissivity coating. Effective longwave optical properties are assigned to the blind layer in order to calculate radiant heat transfer. An exceptionally simple model for convective heat transfer, the reduced slat length (RSL) model, has been developed on the basis of guarded heater plate measurements. CFD results reveal reasons for the very close agreement between measurement and the RSL model. The new simulation capability can be applied to the quantification of U-factor and Solar Heat Gain Coefficient. The simplicity of the RSL model is particularly valuable in the context of building energy simulation where CPU time must be used sparingly.Natural Sciences and Engineering Research Council (NSERC) || The American Society of Heating, Refrigerating and Air-Conditioning Engineers (ASHRAE)

Thermal Resistance of a Window with an Enclosed Venetian Blind: Guarded Heater Plate Measurements

©2006, American Society of Heating, Refrigerating and Air-Conditioning Engineers, Inc. (www.ashrae.org). Published in ASHRAE Transactions, Volume 112, Part 2. For personal use only. Additional reproduction, distribution, or transmission in either print or digital form is not permitted without ASHRAE’s prior written permission.Window solar gain is known to have a strong influence on building energy consumption and peak cooling load. Venetian blinds are routinely used to control solar gain. Software based on one-dimensional models is available to accurately predict the thermal performance of glazing systems, but the develop-ment of models for windows with shading devices is at a very early stage. A guarded heater plate apparatus has been used to measure center-glass heat transfer rates through a double-glazed window with a venetian blind in the glazing cavity. Vari-ables examined include pane spacing, temperature difference, slat angle, and the presence of a low-emissivity coating. Results were compared with earlier measurements. The data collected provide direct guidance in the development of models to predict U-factor and solar gain for this type of glazing/shad-ing system, leading to a more structured and quantitative design procedure.Natural Sciences and Engineering Research Council || Golden Windows Ltd. (Kitchener, Ontario

An Examination of Keyes Universal Chart: 50 Years Later

© 2016 ASHRAE (www.ashrae.org). Published in ASHRAE Conference Papers, Winter Conference, Orlando, FL. For personal use only. Additional reproduction, distribution, or transmission in either print or digital form is not permitted without ASHRAE's prior written permission.From the late 1940's to the late 1960's, significant efforts were made by ASHVE and then ASHRAE to evaluate and quantify the impact of window shading. In the context of the now defunct Shading Coefficient, well known researchers such as Parmelee, Ozisik, Schutrum, Farber, Yellott, and Keyes laid the groundwork for much of the work that followed decades later. Of particular interest are the efforts of Keyes. In his work, he produced a method of classifying fabric based either on visible inspection, or on property measurements. The result was the Keyes Universal Chart, which was first published in the 1965 ASHRAE Guide and Data Book, and has been part of the Fenestration Chapter of the ASHRAE Handbook of Fundamentals since its inception. The chart compares fabric transmittance, reflectance, and openness. It also permits estimation of these properties by making generalized fabric classifications based on a subjective analysis of how light or dark the fabric is, and how open or closed the fabric weave is. More recently, significant efforts have been made to produce window shading models for use in building simulation and daylighting analysis. As part of this research, shading materials have been analyzed using modern and highly accurate spectrophotometric equipment. Unfortunately, that data has revealed inaccuracy in Keyes Universal Chart. The present work examines this inaccuracy.Natural Sciences and Engineering Research Council of Canada (NSERC