WorldWideScience

Sample records for highly penetrant autosomal

  1. A three-generation family with idiopathic facial palsy suggesting an autosomal dominant inheritance with high penetrance

    DEFF Research Database (Denmark)

    Larsen, Christian Grønhøj; Gyldenløve, Mette; Jønch, Aia Elise

    2015-01-01

    Idiopathic facial palsy (IFP), also known as Bell's palsy, is a common neurologic disorder, but recurrent and familial forms are rare. This case series presents a three-generation family with idiopathic facial palsy. The mode of inheritance of IFP has previously been suggested as autosomal dominant...

  2. [Congenital valvular heart disease with high familial penetrance].

    Science.gov (United States)

    Dattilo, Giuseppe; Lamari, Annalisa; Tulino, Viviana; Scarano, Michele; De Luca, Eleonora; Mutone, Daniela; Busacca, Paolo

    2012-12-01

    Bicuspid valve aortic (BVA) is one of the most common congenital malformations. Only 20% of patients preserves a normal valve function throughout life. There are sporadic and familial forms, the latter to autosomal dominant. We present a case of familiarity of BVA high penetrance. Patient with aortic stenosis by BVA, is the father of two children with BVA.

  3. Assessing high wind energy penetration

    DEFF Research Database (Denmark)

    Tande, J.O.

    1995-01-01

    In order to convincingly promote installing wind power capacity as a substantial part of the energy supply system, a set of careful analyses must be undertaken. This paper applies a case study concentrated on assessing the cost/benefit of high wind energy penetration. The case study considers...... expanding the grid connected wind power capacity in Praia, the capital of Cape Verde. The currently installed 1 MW of wind power is estimated to supply close to 10% of the electric energy consumption in 1996. Increasing the wind energy penetration to a higher level is considered viable as the project...... with the existing wind power, supply over 30% of the electric consumption in 1996. Applying the recommended practices for estimating the cost of wind energy, the life-cycle cost of this 2.4 MW investment is estimated at a 7% discount rate and a 20 year lifetime to 0.26 DKK/kW h....

  4. Assessing high wind energy penetration

    International Nuclear Information System (INIS)

    Tande, J.O.

    1995-01-01

    In order to convincingly promote installing wind power capacity as a substantial part of the energy supply system, a set of careful analyses must be undertaken. This paper applies a case study concentrated on assessing the cost/benefit of high wind energy penetration. The case study considers expanding the grid connected wind power capacity in Praia, the capital of Cape Verde. The currently installed 1 MW of wind power is estimated to supply close to 10% of the electric energy consumption in 1996. Increasing the wind energy penetration to a higher level is considered viable as the project settings are close to ideal, including a very capable national utility company, Electra, a conventional power supply system based on imported heavy fuel and gas oil, and favourable wind conditions with an estimated annual average of 9.3 m/s at the hub height of the wind turbines. With the applied case study assumptions, simulations with WINSYS over the lifetime of the assessed wind power investment show that investments up to 4.2 MW are economically viable. The economic optimum is found at 2.4 MW reaching an internal rate of return of almost 8% p.a. This 2.4 MW of wind power would, together with the existing wind power, supply over 30% of the electric consumption in 1996. Applying the recommended practices for estimating the cost of wind energy, the life-cycle cost of this 2.4 MW investment is estimated at a 7% discount rate and a 20 year lifetime to 0.26 DKK/kW h. (Author)

  5. Opportunities for high wind energy penetration

    DEFF Research Database (Denmark)

    Tande, J.O.; Hansen, J.C.

    1997-01-01

    Wind power is today a mature technology, which at windy locations, is economically competitive to conventional power generation technologies. This and growing global environmental concerns have led governments to encourage and plan for wind energy development, a typical aim being 10% of electricity...... consumption. The successful operation of the three major power systems of Cape Verde, with a total wind energy penetration of about 15% since December 1994, demonstrates that power systems can be operated with high penetration of wind energy by adding simple control and monitoring systems only. Thorough...... analyses conclude that expanding to even above 15% wind energy penetration in the Cape Verde power systems is economical. Worldwide, numerous locations with favorable wind conditions and power systems similar to the Capeverdean provide good opportunities for installing wind farms and achieving high wind...

  6. High-Penetration Photovoltaic Planning Methodologies

    Energy Technology Data Exchange (ETDEWEB)

    Gao, David Wenzhong [Alternative Power Innovations, LLC, Broomfield, CO (United States); Muljadi, Eduard [National Renewable Energy Lab. (NREL), Golden, CO (United States); Tian, Tian [National Renewable Energy Lab. (NREL), Golden, CO (United States); Miller, Mackay [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2017-02-24

    The main objective of this report is to provide an overview of select U.S. utility methodologies for performing high-penetration photovoltaic (HPPV) system planning and impact studies. This report covers the Federal Energy Regulatory Commission's orders related to photovoltaic (PV) power system interconnection, particularly the interconnection processes for the Large Generation Interconnection Procedures and Small Generation Interconnection Procedures. In addition, it includes U.S. state interconnection standards and procedures. The procedures used by these regulatory bodies consider the impacts of HPPV power plants on the networks. Technical interconnection requirements for HPPV voltage regulation include aspects of power monitoring, grounding, synchronization, connection to the overall distribution system, back-feeds, disconnecting means, abnormal operating conditions, and power quality. This report provides a summary of mitigation strategies to minimize the impact of HPPV. Recommendations and revisions to the standards may take place as the penetration level of renewables on the grid increases and new technologies develop in future years.

  7. Eddy current testing with high penetration

    International Nuclear Information System (INIS)

    Becker, R.; Kroening, M.

    1999-01-01

    The low-frequency eddy current testing method is used when penetration into very deep layers is required. The achievable penetration depth is determined among other parameters by the lowest testing frequency that can be realised together with the eddy current sensor. When using inductive sensors, the measuring effect declines proportional to the lowering frequency (induction effect). Further reduction of testing frequency requires other types of sensors, as e.g. the GMR (Giant Magnetic Resistance), which achieves a constant measuring sensitivity down to the steady field. The multi-frequency eddy current testing method MFEC 3 of IZFP described here can be operated using three different scanning frequencies at a time. Two variants of eddy current probes are used in this case. Both have an inductive winding at their emitters, of the type of a measuring probe. The receiver end is either also an inductive winding, or a magnetic field-responsive resistance (GMR). (orig./CB) [de

  8. Penetrative convection at high Rayleigh numbers

    Science.gov (United States)

    Toppaladoddi, Srikanth; Wettlaufer, John S.

    2018-04-01

    We study penetrative convection of a fluid confined between two horizontal plates, the temperatures of which are such that a temperature of maximum density lies between them. The range of Rayleigh numbers studied is Ra=[0.01 ,4 ]106,108 and the Prandtl numbers are Pr=1 and 11.6. An evolution equation for the growth of the convecting region is obtained through an integral energy balance. We identify a new nondimensional parameter, Λ , which is the ratio of temperature difference between the stable and unstable regions of the flow; larger values of Λ denote increased stability of the upper stable layer. We study the effects of Λ on the flow field using well-resolved lattice Boltzmann simulations and show that the characteristics of the flow depend sensitively upon it. For the range Λ = , we find that for a fixed Ra the Nusselt number, Nu, increases with decreasing Λ . We also investigate the effects of Λ on the vertical variation of convective heat flux and the Brunt-Väisälä frequency. Our results clearly indicate that in the limit Λ →0 the problem reduces to that of the classical Rayleigh-Bénard convection.

  9. Review of Power System Stability with High Wind Power Penetration

    DEFF Research Database (Denmark)

    Hu, Rui; Hu, Weihao; Chen, Zhe

    2015-01-01

    analyzing methods and stability improvement approaches. With increasing wind power penetration, system balancing and the reduced inertia may cause a big threaten for stable operation of power systems. To mitigate or eliminate the wind impacts for high wind penetration systems, although the practical......This paper presents an overview of researches on power system stability with high wind power penetration including analyzing methods and improvement approaches. Power system stability issues can be classified diversely according to different considerations. Each classified issue has special...... and reliable choices currently are the strong outside connections or sufficient reserve capacity constructions, many novel theories and approaches are invented to investigate the stability issues, looking forward to an extra-high penetration or totally renewable resource based power systems. These analyzing...

  10. Southern California Edison High Penetration Photovoltaic Project - Year 1

    Energy Technology Data Exchange (ETDEWEB)

    Mather, B.; Kroposki, B.; Neal, R.; Katiraei, F.; Yazdani, A.; Aguero, J. R.; Hoff, T. E.; Norris, B. L.; Parkins, A.; Seguin, R.; Schauder, C.

    2011-06-01

    This report discusses research efforts from the first year of a project analyzing the impacts of high penetration levels of photovoltaic (PV) resources interconnected onto Southern California Edison's (SCE's) distribution system. SCE will be interconnecting a total of 500 MW of commercial scale PV within their service territory by 2015. This Year 1 report describes the need for investigating high-penetration PV scenarios on the SCE distribution system; discusses the necessary PV system modeling and distribution system simulation advances; describes the available distribution circuit data for the two distribution circuits identified in the study; and discusses the additional inverter functionality that could be implemented in order to specifically mitigate some of the undesirable distribution system impacts caused by high-penetration PV installations.

  11. Fault Analysis and Detection in Microgrids with High PV Penetration

    Energy Technology Data Exchange (ETDEWEB)

    El Khatib, Mohamed [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Hernandez Alvidrez, Javier [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Ellis, Abraham [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2017-05-01

    In this report we focus on analyzing current-controlled PV inverters behaviour under faults in order to develop fault detection schemes for microgrids with high PV penetration. Inverter model suitable for steady state fault studies is presented and the impact of PV inverters on two protection elements is analyzed. The studied protection elements are superimposed quantities based directional element and negative sequence directional element. Additionally, several non-overcurrent fault detection schemes are discussed in this report for microgrids with high PV penetration. A detailed time-domain simulation study is presented to assess the performance of the presented fault detection schemes under different microgrid modes of operation.

  12. Assessment of high penetration of solar photovoltaics in Wisconsin

    International Nuclear Information System (INIS)

    Myers, Kevin S.; Klein, Sanford A.; Reindl, Douglas T.

    2010-01-01

    This paper provides an assessment of the large-scale implementation of distributed solar photovoltaics in Wisconsin with regard to its interaction with the utility grid, economics of varying levels of high penetration, and displaced emissions. These assessment factors are quantified using simulations with measured hourly solar radiation and weather data from the National Solar Radiation Database as primary inputs. Hourly utility load data for each electric utility in Wisconsin for a complete year were used in combination with the simulated PV output to quantify the impacts of high penetration of distributed PV on the aggregate Wisconsin electric utility load. As the penetration rate of distributed PV systems increases, both economic and environmental benefits experience diminishing returns. At penetration rates exceeding 15-20% of the aggregate utility load peak, less of the PV-energy is utilized and the contribution of the aggregate electricity generated from PV approaches a practical limit. The limit is not affected by costs, but rather by the time-distribution of available solar radiation and mismatch with the coincidence of aggregate utility electrical loads. The unsubsidized levelized cost of electricity from PV is more than four times greater than the current market price for electricity, based on time-of-use rates, in Wisconsin. At the present time, the investment in solar PV as a cost-effective means to reduce emissions from traditional electricity generation sources is not justified. (author)

  13. Are high penetrations of commercial cogeneration good for society?

    Science.gov (United States)

    Keen, Jeremy F.; Apt, Jay

    2016-12-01

    Low natural gas prices, market reports and evidence from New York State suggest that the number of commercial combined heat and power (CHP) installations in the United States will increase by 2%-9% annually over the next decade. We investigate how increasing commercial CHP penetrations may affect net emissions, the distribution network, and total system energy costs. We constructed an integrated planning and operations model that maximizes owner profit through sizing and operation of CHP on a realistic distribution feeder in New York. We find that a greater penetration of CHP reduces both total system energy costs and network congestion. Commercial buildings often have low and inconsistent heat loads, which can cause low fuel utilization efficiencies, low CHP rates-of-return and diminishing avoided emissions as CHP penetration increases. In the northeast, without policy intervention, a 5% penetration of small commercially owned CHP would increase CO2 emissions by 2% relative to the bulk power grid. Low emission CHP installations can be encouraged with incentives that promote CHP operation only during times of high heat loads. Time-varying rates, such as time-of-day and seasonal rates, are one option and were shown to reduce customer emissions without reducing profits. In contrast, natural gas rate discounts, a common incentive for industrial CHP in some states, can encourage CHP operation during low heat loads and thus increase emissions.

  14. High Penetrated Wind Farm Impacts on the Electricity Price

    DEFF Research Database (Denmark)

    Haji Bashi, Mazaher; Yousefi, G. R.; Bak, Claus Leth

    2016-01-01

    of the high penetrated wind farm integration into electricity markets. Then, stochastic programming approach is employed to compare the volume of trades for a typical wind farm in a high and low wind penetrated market. Although increasing price spikes and volatility was reported in the literature......Energy trading policies, intermittency of wind farm output power, low marginal cost of the production, are the key factors that cause the wind farms to be effective on the electricity price. In this paper, the Danish electricity market is studied as a part of Nord Pool. Considering the completely...... fossil fuel free overview in Danish energy policies, and the currently great share of wind power (more than 100% for some hours) in supplying the load, it is an interesting benchmark for the future electricity markets. Negative prices, price spikes, and price volatility are considered as the main effects...

  15. Microgrid optimal scheduling considering impact of high penetration wind generation

    Science.gov (United States)

    Alanazi, Abdulaziz

    The objective of this thesis is to study the impact of high penetration wind energy in economic and reliable operation of microgrids. Wind power is variable, i.e., constantly changing, and nondispatchable, i.e., cannot be controlled by the microgrid controller. Thus an accurate forecasting of wind power is an essential task in order to study its impacts in microgrid operation. Two commonly used forecasting methods including Autoregressive Integrated Moving Average (ARIMA) and Artificial Neural Network (ANN) have been used in this thesis to improve the wind power forecasting. The forecasting error is calculated using a Mean Absolute Percentage Error (MAPE) and is improved using the ANN. The wind forecast is further used in the microgrid optimal scheduling problem. The microgrid optimal scheduling is performed by developing a viable model for security-constrained unit commitment (SCUC) based on mixed-integer linear programing (MILP) method. The proposed SCUC is solved for various wind penetration levels and the relationship between the total cost and the wind power penetration is found. In order to reduce microgrid power transfer fluctuations, an additional constraint is proposed and added to the SCUC formulation. The new constraint would control the time-based fluctuations. The impact of the constraint on microgrid SCUC results is tested and validated with numerical analysis. Finally, the applicability of proposed models is demonstrated through numerical simulations.

  16. Pumped storage in systems with very high wind penetration

    International Nuclear Information System (INIS)

    Tuohy, A.; O'Malley, M.

    2011-01-01

    This paper examines the operation of the Irish power system with very high levels of wind energy, with and without pumped storage. A unit commitment model which accounts for the uncertainty in wind power is used. It is shown that as wind penetration increases, the optimal operation of storage depends on wind output as well as load. The main benefit from storage is shown to be a decrease in wind curtailment. The economics of the system are examined to find the level at which storage justifies its capital costs and inefficiencies. It is shown that the uncertainty of wind makes the option of storage more attractive. The size of the energy store has an impact on results. At lower levels of installed wind (up to approximately 50% of energy from wind in Ireland), the reduction in curtailment is insufficient to justify building storage. At greater levels of wind, storage reduces curtailment sufficiently to justify the additional capital costs. It can be seen that if storage replaces OCGTs in the plant mix instead of CCGTs, then the level at which it justifies itself is lower. Storage increases the level of carbon emissions at wind penetration below 60%. - Research highlights: → Examines operation of pumped storage unit in a system with levels of wind from 34%-68% of energy. → High capital cost of storage is not justified until system has high (approx. 45%) wind penetration. → Results are driven by the amount of wind curtailment avoided and plant mix of system. → Other flexible options (e.g. interconnection) offer many of the same benefits as storage.

  17. Net load forecasting for high renewable energy penetration grids

    International Nuclear Information System (INIS)

    Kaur, Amanpreet; Nonnenmacher, Lukas; Coimbra, Carlos F.M.

    2016-01-01

    We discuss methods for net load forecasting and their significance for operation and management of power grids with high renewable energy penetration. Net load forecasting is an enabling technology for the integration of microgrid fleets with the macrogrid. Net load represents the load that is traded between the grids (microgrid and utility grid). It is important for resource allocation and electricity market participation at the point of common coupling between the interconnected grids. We compare two inherently different approaches: additive and integrated net load forecast models. The proposed methodologies are validated on a microgrid with 33% annual renewable energy (solar) penetration. A heuristics based solar forecasting technique is proposed, achieving skill of 24.20%. The integrated solar and load forecasting model outperforms the additive model by 10.69% and the uncertainty range for the additive model is larger than the integrated model by 2.2%. Thus, for grid applications an integrated forecast model is recommended. We find that the net load forecast errors and the solar forecasting errors are cointegrated with a common stochastic drift. This is useful for future planning and modeling because the solar energy time-series allows to infer important features of the net load time-series, such as expected variability and uncertainty. - Highlights: • Net load forecasting methods for grids with renewable energy generation are discussed. • Integrated solar and load forecasting outperforms the additive model by 10.69%. • Net load forecasting reduces the uncertainty between the interconnected grids.

  18. Potential of ultrafine grained materials as high performance penetrator materials

    Directory of Open Access Journals (Sweden)

    Lee C.S.

    2012-08-01

    Full Text Available The shear formability and the metal jet formability are important for the kinetic energy penetrator and the chemical energy penetrator, respectively. The shear formability of ultrafine grained (UFG steel was examined, mainly focusing on the effects of the grain shape on the shear characteristics. For this purpose, UFG 4130 steel having the different UFG structures, the lamellar UFG and the equiaxed UFG, was prepared by equal channel angular pressing (ECAP. The lamellar UFG steel exhibited more sharper and localized shear band formation than the equiaxed UFG steel. This is because a lamellar UFG structure was unfavourable against grain rotation which is a main mechanism of the band propagation in UFG materials. Meanwhile, the metal jet formability of UFG OFHC Cu also processed by ECAP was compared to that of coarse grained (CG one by means of dynamic tensile extrusion (DTE tests. CG OFHC Cu exhibited the higher DTE ductility, i.e. better metal jet stability, than UFG OFHC Cu. The initial high strength and the lack of strain hardenability of UFG OFHC Cu were harmful to the metal jet formability.

  19. Wind power bidding in electricity markets with high wind penetration

    International Nuclear Information System (INIS)

    Vilim, Michael; Botterud, Audun

    2014-01-01

    Highlights: • We analyze the pricing systems and wind power trading in electricity markets. • We propose a model that captures the relation between market prices and wind power. • A probabilistic bidding model can increase profits for wind power producers. • Profit maximizing bidding strategies carry risks for power system operators. • We conclude that modifications of current market designs may be needed. - Abstract: Objective: The optimal day-ahead bidding strategy is studied for a wind power producer operating in an electricity market with high wind penetration. Methods: A generalized electricity market is studied with minimal assumptions about the structure of the production, bidding, or consumption of electricity. Two electricity imbalance pricing schemes are investigated, the one price and the two price scheme. A stochastic market model is created to capture the price effects of wind power production and consumption. A bidding algorithm called SCOPES (Supply Curve One Price Estimation Strategy) is developed for the one price system. A bidding algorithm called MIMICS (Multivariate Interdependence Minimizing Imbalance Cost Strategy) is developed for the two price system. Results: Both bidding strategies are shown to have advantages over the assumed “default” bidding strategy, the point forecast. Conclusion: The success of these strategies even in the case of high deviation penalties in a one price system and the implicit deviation penalties of the two price system has substantial implications for power producers and system operators in electricity markets with a high level of wind penetration. Practice implications: From an electricity market design perspective, the results indicate that further penalties or regulations may be needed to reduce system imbalance

  20. High-Penetration PV Integration Handbook for Distribution Engineers

    Energy Technology Data Exchange (ETDEWEB)

    Seguin, Rich [Electrical Distribution Design, Blacksburg, VA (United States); Woyak, Jeremy [Electrical Distribution Design, Blacksburg, VA (United States); Costyk, David [Electrical Distribution Design, Blacksburg, VA (United States); Hambrick, Josh [Electrical Distribution Design, Blacksburg, VA (United States); Mather, Barry [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2016-01-01

    This handbook has been developed as part of a five-year research project which began in 2010. The National Renewable Energy Laboratory (NREL), Southern California Edison (SCE), Quanta Technology, Satcon Technology Corporation, Electrical Distribution Design (EDD), and Clean Power Research (CPR) teamed together to analyze the impacts of high-penetration levels of photovoltaic (PV) systems interconnected onto the SCE distribution system. This project was designed specifically to leverage the experience that SCE and the project team would gain during the significant installation of 500 MW of commercial scale PV systems (1-5 MW typically) starting in 2010 and completing in 2015 within SCE’s service territory through a program approved by the California Public Utility Commission (CPUC).

  1. Risk assessment of electric generation systems with high wind penetration

    International Nuclear Information System (INIS)

    Salgado Duarte, Yorlandys; Castillo Serpa, Alfredo M. del

    2017-01-01

    The research evaluates the risk function of an Electric Generation System (SGE) with high wind power penetration using the Sequential Monte Carlo Simulation (SMCS) method, which allows calculating indicators that characterize the performance of the SGE with expected average values. The research uses a Markov model of two states or four states according to the characteristics of the generator to simulate the instantaneous capacity. The primary sources of each conventional generator are assumed to be always available; however, wind power depends on the wind behavior in each analyzed region. In this research, the Chronological Series and Weibull models are used to model the wind behavior, and the analyzes are performed in the IEEE-RTS system. The work shows that the behavior of the probabilistic indicators used to analyze the static capacity of the SGE is determined by the model used to simulate the stochastic of the generators and by the primary energy source. (author)

  2. Final Technical Report: Distributed Controls for High Penetrations of Renewables

    Energy Technology Data Exchange (ETDEWEB)

    Byrne, Raymond H. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Neely, Jason C. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Rashkin, Lee J. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Trudnowski, Daniel J. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Wilson, David G. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2015-12-01

    The goal of this effort was to apply four potential control analysis/design approaches to the design of distributed grid control systems to address the impact of latency and communications uncertainty with high penetrations of photovoltaic (PV) generation. The four techniques considered were: optimal fixed structure control; Nyquist stability criterion; vector Lyapunov analysis; and Hamiltonian design methods. A reduced order model of the Western Electricity Coordinating Council (WECC) developed for the Matlab Power Systems Toolbox (PST) was employed for the study, as well as representative smaller systems (e.g., a two-area, three-area, and four-area power system). Excellent results were obtained with the optimal fixed structure approach, and the methodology we developed was published in a journal article. This approach is promising because it offers a method for designing optimal control systems with the feedback signals available from Phasor Measurement Unit (PMU) data as opposed to full state feedback or the design of an observer. The Nyquist approach inherently handles time delay and incorporates performance guarantees (e.g., gain and phase margin). We developed a technique that works for moderate sized systems, but the approach does not scale well to extremely large system because of computational complexity. The vector Lyapunov approach was applied to a two area model to demonstrate the utility for modeling communications uncertainty. Application to large power systems requires a method to automatically expand/contract the state space and partition the system so that communications uncertainty can be considered. The Hamiltonian Surface Shaping and Power Flow Control (HSSPFC) design methodology was selected to investigate grid systems for energy storage requirements to support high penetration of variable or stochastic generation (such as wind and PV) and loads. This method was applied to several small system models.

  3. Update on the Management of High-Risk Penetrating Keratoplasty.

    Science.gov (United States)

    Jabbehdari, Sayena; Rafii, Alireza Baradaran; Yazdanpanah, Ghasem; Hamrah, Pedram; Holland, Edward J; Djalilian, Ali R

    2017-03-01

    In this article, we review the indications and latest management of high-risk penetrating keratoplasty. Despite the immune-privilege status of the cornea, immune-mediated graft rejection still remains the leading cause of corneal graft failure. This is particularly a problem in the high-risk graft recipients, namely patients with previous graft failure due to rejection and those with inflamed and vascularized corneal beds. A number of strategies including both local and systemic immunosuppression are currently used to increase the success rate of high-risk corneal grafts. Moreover, in cases of limbal stem cell deficiency, limbal stem cells transplantation is employed. Corticosteroids are still the top medication for prevention and treatment in cases of corneal graft rejection. Single and combined administration of immunosuppressive agents e.g. tacrolimus, cyclosporine and mycophenolate are promising adjunctive therapies for prolonging graft survival. In the future, cellular and molecular therapies should allow us to achieve immunologic tolerance even in high-risk grafts.

  4. Development of high-performance concrete having high resistance to chloride penetration

    International Nuclear Information System (INIS)

    Oh, Byung Hwan; Cha, Soo Won; Jang, Bong Seok; Jang, Seung Yup

    2002-01-01

    The resistance to chloride penetration is one of the simplest measures to determine the durability of concrete, e.g. resistance to freezing and thawing, corrosion of steel in concrete and other chemical attacks. Thus, high-performance concrete may be defined as the concrete having high resistance to chloride penetration as well as high strength. The purpose of this paper is to investigate the resistance to chloride penetration of different types of concrete and to develop high-performance concrete that has very high resistance to chloride penetration, and thus, can guarantee high durability. A large number of concrete specimens have been tested by the rapid chloride permeability test method as designated in AASHTO T 277 and ASTM C 1202. The major test variables include water-to-binder ratios, type of cement, type and amount of mineral admixtures (silica fume, fly ash and blast-furnace slag), maximum size of aggregates and air-entrainment. Test results show that concrete containing optimal amount of silica fume shows very high resistance to chloride penetration, and high-performance concrete developed in this study can be efficiently employed to enhance the durability of concrete structures in severe environments such as nuclear power plants, water-retaining structures and other offshore structures

  5. Mastering wireless penetration testing for highly secured environments

    CERN Document Server

    Johns, Aaron

    2015-01-01

    This book is intended for security professionals who want to enhance their wireless penetration testing skills and knowledge. Since this book covers advanced techniques, you will need some previous experience in computer security and networking.

  6. Improved Modeling Tools Development for High Penetration Solar

    Energy Technology Data Exchange (ETDEWEB)

    Washom, Byron [Univ. of California, San Diego, CA (United States); Meagher, Kevin [Power Analytics Corporation, San Diego, CA (United States)

    2014-12-11

    One of the significant objectives of the High Penetration solar research is to help the DOE understand, anticipate, and minimize grid operation impacts as more solar resources are added to the electric power system. For Task 2.2, an effective, reliable approach to predicting solar energy availability for energy generation forecasts using the University of California, San Diego (UCSD) Sky Imager technology has been demonstrated. Granular cloud and ramp forecasts for the next 5 to 20 minutes over an area of 10 square miles were developed. Sky images taken every 30 seconds are processed to determine cloud locations and cloud motion vectors yielding future cloud shadow locations respective to distributed generation or utility solar power plants in the area. The performance of the method depends on cloud characteristics. On days with more advective cloud conditions, the developed method outperforms persistence forecasts by up to 30% (based on mean absolute error). On days with dynamic conditions, the method performs worse than persistence. Sky Imagers hold promise for ramp forecasting and ramp mitigation in conjunction with inverter controls and energy storage. The pre-commercial Sky Imager solar forecasting algorithm was documented with licensing information and was a Sunshot website highlight.

  7. High-pressure-induced water penetration into 3-isopropylmalate dehydrogenase

    International Nuclear Information System (INIS)

    Nagae, Takayuki; Kawamura, Takashi; Chavas, Leonard M. G.; Niwa, Ken; Hasegawa, Masashi; Kato, Chiaki; Watanabe, Nobuhisa

    2012-01-01

    Structures of 3-isopropylmalate dehydrogenase were determined at pressures ranging from 0.1 to 650 MPa. Comparison of these structures gives a detailed picture of the swelling of a cavity at the dimer interface and the generation of a new cleft on the molecular surface, which are accompanied by water penetration. Hydrostatic pressure induces structural changes in proteins, including denaturation, the mechanism of which has been attributed to water penetration into the protein interior. In this study, structures of 3-isopropylmalate dehydrogenase (IPMDH) from Shewanella oneidensis MR-1 were determined at about 2 Å resolution under pressures ranging from 0.1 to 650 MPa using a diamond anvil cell (DAC). Although most of the protein cavities are monotonically compressed as the pressure increases, the volume of one particular cavity at the dimer interface increases at pressures over 340 MPa. In parallel with this volume increase, water penetration into the cavity could be observed at pressures over 410 MPa. In addition, the generation of a new cleft on the molecular surface accompanied by water penetration could also be observed at pressures over 580 MPa. These water-penetration phenomena are considered to be initial steps in the pressure-denaturation process of IPMDH

  8. Economic viability of transmission capacity expansion at high wind penetrations

    DEFF Research Database (Denmark)

    Østergaard, Poul Alberg

    2005-01-01

    investments and analyses of the Nord Pool price variations. The analyses are done for varying degrees of wind power penetrations ranging from 20% of the West Danish electricity demand up to 100% of the demand. The analyses demonstrate, that while there is an economic potential for some expansion in some years......With growing wind power penetrations in many countries, grid and system integration becomes more and more important issues. This is particularly the case in countries or regions with good wind resources as well as substantial installed wind power capacity as found in e.g. Northern Europe. At 20......% penetration in Western Denmark, the issue is pertinent here in relation to future plans of further expansion which is planned in accordance with the Danish Government’s climate change mitigation initiatives. This paper analyses the potential economic benefit of selling excess electricity production...

  9. Distribution Networks Management with High Penetration of Photovoltaic Panels

    DEFF Research Database (Denmark)

    Morais, Hugo

    2013-01-01

    The photovoltaic solar panels penetration increases significantly in recent years in several European countries, mainly in the low voltage and medium voltage networks supported by governmental policies and incentives. Consequently, the acquisition and installation costs of PV panels decrease...... and the know–how increase significantly. Presently is important the use of new management methodologies in distribution networks to support the growing penetration of PV panels. In some countries, like in Germany and in Italy, the solar generation based in photovoltaic panels supply 40% of the demand in some...

  10. Operation of Power Grids with High Penetration of Wind Power

    Science.gov (United States)

    Al-Awami, Ali Taleb

    The integration of wind power into the power grid poses many challenges due to its highly uncertain nature. This dissertation involves two main components related to the operation of power grids with high penetration of wind energy: wind-thermal stochastic dispatch and wind-thermal coordinated bidding in short-term electricity markets. In the first part, a stochastic dispatch (SD) algorithm is proposed that takes into account the stochastic nature of the wind power output. The uncertainty associated with wind power output given the forecast is characterized using conditional probability density functions (CPDF). Several functions are examined to characterize wind uncertainty including Beta, Weibull, Extreme Value, Generalized Extreme Value, and Mixed Gaussian distributions. The unique characteristics of the Mixed Gaussian distribution are then utilized to facilitate the speed of convergence of the SD algorithm. A case study is carried out to evaluate the effectiveness of the proposed algorithm. Then, the SD algorithm is extended to simultaneously optimize the system operating costs and emissions. A modified multi-objective particle swarm optimization algorithm is suggested to identify the Pareto-optimal solutions defined by the two conflicting objectives. A sensitivity analysis is carried out to study the effect of changing load level and imbalance cost factors on the Pareto front. In the second part of this dissertation, coordinated trading of wind and thermal energy is proposed to mitigate risks due to those uncertainties. The problem of wind-thermal coordinated trading is formulated as a mixed-integer stochastic linear program. The objective is to obtain the optimal tradeoff bidding strategy that maximizes the total expected profits while controlling trading risks. For risk control, a weighted term of the conditional value at risk (CVaR) is included in the objective function. The CVaR aims to maximize the expected profits of the least profitable scenarios, thus

  11. High frequency of potentially pathogenic SORL1 mutations in autosomal dominant early-onset Alzheimer disease.

    Science.gov (United States)

    Pottier, C; Hannequin, D; Coutant, S; Rovelet-Lecrux, A; Wallon, D; Rousseau, S; Legallic, S; Paquet, C; Bombois, S; Pariente, J; Thomas-Anterion, C; Michon, A; Croisile, B; Etcharry-Bouyx, F; Berr, C; Dartigues, J-F; Amouyel, P; Dauchel, H; Boutoleau-Bretonnière, C; Thauvin, C; Frebourg, T; Lambert, J-C; Campion, D

    2012-09-01

    Performing exome sequencing in 14 autosomal dominant early-onset Alzheimer disease (ADEOAD) index cases without mutation on known genes (amyloid precursor protein (APP), presenilin1 (PSEN1) and presenilin2 (PSEN2)), we found that in five patients, the SORL1 gene harbored unknown nonsense (n=1) or missense (n=4) mutations. These mutations were not retrieved in 1500 controls of same ethnic origin. In a replication sample, including 15 ADEOAD cases, 2 unknown non-synonymous mutations (1 missense, 1 nonsense) were retrieved, thus yielding to a total of 7/29 unknown mutations in the combined sample. Using in silico predictions, we conclude that these seven private mutations are likely to have a pathogenic effect. SORL1 encodes the Sortilin-related receptor LR11/SorLA, a protein involved in the control of amyloid beta peptide production. Our results suggest that besides the involvement of the APP and PSEN genes, further genetic heterogeneity, involving another gene of the same pathway is present in ADEOAD.

  12. Epidemiologic analysis of families with isolated anorectal malformations suggests high prevalence of autosomal dominant inheritance.

    Science.gov (United States)

    Dworschak, Gabriel C; Zwink, Nadine; Schmiedeke, Eberhard; Mortazawi, Kiarasch; Märzheuser, Stefanie; Reinshagen, Konrad; Leonhardt, Johannes; Gómez, Barbara; Volk, Patrick; Rißmann, Anke; Jenetzky, Ekkehart; Reutter, Heiko

    2017-12-13

    Anorectal malformations (ARM) are rare abnormalities that occur in approximately 1 in 3000 live births with around 40% of patients presenting with isolated forms. Multiple familial cases reported, suggest underlying genetic factors that remain largely unknown. The recurrence in relatives is considered rare, however transmission rates of ARM by affected parents have never been determined before. The inheritance pattern of ARM was investigated in our database of patients with isolated ARM. Within our cohort of 327 patients with isolated ARM we identified eight adult patients from eight families who had in total 16 children with their healthy spouse. Of these ten had ARM, resulting in a recurrence risk of approximately one in two live births (10 of 16; 62%). From 226 families with 459 siblings we found two affected siblings in five families. Hence, the recurrence risk of ARM among siblings is approximately one in 92 live births (5 of 459; 1.0%). Comparing the observed recurrence risk in our cohort with the prevalence in the general population, we see a 1500-fold increase in recurrence risk for offspring and a 32-fold increase if a sibling is affected. The recurrence risk of approximately 62% indicates an autosomal dominant mode of inheritance. Reliable figures on recurrence of ARM are becoming increasingly important since improved surgical techniques are able to maintain sexual function resulting in more offspring of patients with ARM. These data allow more precise counseling of families with ARM and support the need for genetic studies.

  13. Storage Application in Smart Grid with High PV and EV Penetration

    DEFF Research Database (Denmark)

    Hashemi Toghroljerdi, Seyedmostafa; Yang, Guangya; Østergaard, Jacob

    2013-01-01

    grids with residential PVs and Electric Vehicles (EVs). The effect of EV home charging on EESS capacity in high PV penetration is also addressed. The results indicate that increasing the EV penetration in the network can decrease the EESS capacity need. This decrease is highest in situations with low PV...

  14. Retaining the Value of PV at High Penetration Workshop

    Energy Technology Data Exchange (ETDEWEB)

    Kurtz, Sarah; Bolen, Michael

    2017-01-19

    PV prices have dropped and are now attractive without incentives for peaking applications in some locations. Modeling suggests and, empirically, some regions demonstrate that as PV penetration increases its value decreases, predominantly due to a decrease in energy and capacity value. It is not apparent what technologies and price may be needed for PV to supply tens of percent of electricity in the most economically efficient manner. A 1-day workshop was co-sponsored by EPRI and NREL with support from ASU. A dozen presentations and discussions introduced how the interplay of various technologies impact the value of PV, identified technical challenges and gaps impeding implementation, and discussed future R&D needs and opportunities.

  15. Optimal Operation of Plug-In Electric Vehicles in Power Systems with High Wind Power Penetrations

    DEFF Research Database (Denmark)

    Hu, Weihao; Su, Chi; Chen, Zhe

    2013-01-01

    in the power systems with high wind power penetrations. In this paper, the integration of plug-in electric vehicles in the power systems with high wind power penetrations is proposed and discussed. Optimal operation strategies of PEV in the spot market are proposed in order to decrease the energy cost for PEV......The Danish power system has a large penetration of wind power. The wind fluctuation causes a high variation in the power generation, which must be balanced by other sources. The battery storage based Plug-In Electric Vehicles (PEV) may be a possible solution to balance the wind power variations...... owners. Furthermore, the application of battery storage based aggregated PEV is analyzed as a regulation services provider in the power system with high wind power penetrations. The western Danish power system where the total share of annual wind power production is more than 27% of the electrical energy...

  16. Price Forecasting of Electricity Markets in the Presence of a High Penetration of Wind Power Generators

    OpenAIRE

    Saber Talari; Miadreza Shafie-khah; Gerardo J. Osório; Fei Wang; Alireza Heidari; João P. S. Catalão

    2017-01-01

    Price forecasting plays a vital role in the day-ahead markets. Once sellers and buyers access an accurate price forecasting, managing the economic risk can be conducted appropriately through offering or bidding suitable prices. In networks with high wind power penetration, the electricity price is influenced by wind energy; therefore, price forecasting can be more complicated. This paper proposes a novel hybrid approach for price forecasting of day-ahead markets, with high penetration of wind...

  17. High Penetration of Electrical Vehicles in Microgrids: Threats and Opportunities

    Science.gov (United States)

    Khederzadeh, Mojtaba; Khalili, Mohammad

    2014-10-01

    Given that the microgrid concept is the building block of future electric distribution systems and electrical vehicles (EVs) are the future of transportation market, in this paper, the impact of EVs on the performance of microgrids is investigated. Demand-side participation is used to cope with increasing demand for EV charging. The problem of coordination of EV charging and discharging (with vehicle-to-grid (V2G) functionality) and demand response is formulated as a market-clearing mechanism that accepts bids from the demand and supply sides and takes into account the constraints put forward by different parts. Therefore, a day-ahead market with detailed bids and offers within the microgrid is designed whose objective is to maximize the social welfare which is the difference between the value that consumers attach to the electrical energy they buy plus the benefit of the EV owners participating in the V2G functionality and the cost of producing/purchasing this energy. As the optimization problem is a mixed integer nonlinear programming one, it is decomposed into one master problem for energy scheduling and one subproblem for power flow computation. The two problems are solved iteratively by interfacing MATLAB with GAMS. Simulation results on a sample microgrid with different residential, commercial and industrial consumers with associated demand-side biddings and different penetration level of EVs support the proposed formulation of the problem and the applied methods.

  18. Highly penetrative liposome nanomedicine generated by a biomimetic strategy for enhanced cancer chemotherapy.

    Science.gov (United States)

    Jia, Yali; Sheng, Zonghai; Hu, Dehong; Yan, Fei; Zhu, Mingting; Gao, Guanhui; Wang, Pan; Liu, Xin; Wang, Xiaobing; Zheng, Hairong

    2018-04-25

    Liposome nanomedicine has been successfully applied for cancer chemotherapy in patients. However, in general, the therapeutic efficacy is confined by its limited accumulation and penetration in solid tumors. Here, we established a biomimetic strategy for the preparation of highly penetrative liposome nanomedicine for enhanced chemotherapeutic efficacy. By applying this unique type of nanomedicine, membrane proteins on the cancer cells are used as highly penetrative targeting ligands. Biomimetic liposomes are highly stable, exhibiting a superior in vitro homologous targeting ability, and a 2.25-fold deeper penetration in 3D tumor spheroids when compared to conventional liposome nanomedicine. The fluorescence/photoacoustic dual-modal imaging approach demonstrated enhanced tumor accumulation and improved tumor penetration of the biomimetic liposome in C6 glioma tumor-bearing nude mice. Following the intravenous administration of biomimetic liposome nanomedicine, the tumor inhibition rate reached up to 93.3%, which was significantly higher when compared to that of conventional liposome nanomedicine (69.3%). Moreover, histopathological analyses demonstrated that biomimetic liposome nanomedicine has limited side effects. Therefore, these results suggested that a cancer cell membrane-based biomimetic strategy may provide a breakthrough approach for enhancing drug penetration and improving treatment efficacy, holding a great promise for further clinical studies.

  19. High prevalence of mutations affecting the splicing process in a Spanish cohort with autosomal dominant retinitis pigmentosa

    Science.gov (United States)

    Ezquerra-Inchausti, Maitane; Barandika, Olatz; Anasagasti, Ander; Irigoyen, Cristina; López de Munain, Adolfo; Ruiz-Ederra, Javier

    2017-01-01

    Retinitis pigmentosa is the most frequent group of inherited retinal dystrophies. It is highly heterogeneous, with more than 80 disease-causing genes 27 of which are known to cause autosomal dominant RP (adRP), having been identified. In this study a total of 29 index cases were ascertained based on a family tree compatible with adRP. A custom panel of 31 adRP genes was analysed by targeted next-generation sequencing using the Ion PGM platform in combination with Sanger sequencing. This allowed us to detect putative disease-causing mutations in 14 out of the 29 (48.28%) families analysed. Remarkably, around 38% of all adRP cases analysed showed mutations affecting the splicing process, mainly due to mutations in genes coding for spliceosome factors (SNRNP200 and PRPF8) but also due to splice-site mutations in RHO. Twelve of the 14 mutations found had been reported previously and two were novel mutations found in PRPF8 in two unrelated patients. In conclusion, our results will lead to more accurate genetic counselling and will contribute to a better characterisation of the disease. In addition, they may have a therapeutic impact in the future given the large number of studies currently underway based on targeted RNA splicing for therapeutic purposes. PMID:28045043

  20. Sub-hourly impacts of high solar penetrations in the Western United States

    Energy Technology Data Exchange (ETDEWEB)

    Lew, Debra; Brinkman, Greg; Florita, Anthony; Heaney, Michael; Hodge, Bri-Mathias; Hummon, Marissa; Ibanez, Eduardo [National Renewable Energy Lab. (NREL), Golden, CO (United States); King, Jack [RePPAE, Wexford, PA (United States)

    2012-07-01

    Until recently, it has been difficult to study the impacts of significant penetrations of hypothetical, utility-scale solar photovoltaic (PV) plants over large geographic regions. This was because of the lack of credible data to simulate the output of these plants with appropriate spatial and temporal correlation, especially on a sub-hourly basis. In the Western Wind and Solar Integration Study Phase 2 (WWSIS2), we used new technigues to synthesize sub-hourly high-resolution solar output for PV rooftops, utility-scale PV, and concentrating solar power (CSP). This allowed us to examine implications of 25 % solar (60/40 split of PV and CSP) and 8 % wind. In this paper, we present results of analysis on the sub-hourly impacts of high solar penetrations. Extreme event analysis showed that most of the large ramps were because of sunrise and sunset events, which have a significant predictability component. Variability in general was much higher with high penetrations of solar than with high penetrations of wind. Reserve methodologies that had already been developed for wind were therefore modified to take into account the predictability component of solar variability. Significantly less transmission was required for high solar penetrations than wind and significantly less curtailment occurred in the high solar cases. (orig.)

  1. High filler concrete using fly ash. Chloride penetration and microstructure

    NARCIS (Netherlands)

    Valcke, S.L.A.; Polder, R.B.; Nijland, T.G.; Leegwater, G.A.; Visser, J.H.M.; Bigaj-van Vliet, A.J.

    2012-01-01

    Most high filler concrete studies are based on relatively high contents of powder (cement + filler) (>400 kg m-3). This paper aims to increase the total fly ash content relative to the clinker content, while simultaneously minimizing the total powder content in the concrete to values lower than 300

  2. High filler concrete using fly ash : Chloride penetration and microstructure

    NARCIS (Netherlands)

    Valcke, S.L.A.; Polder, R.B.; Nijland, T.G.; Leegwater, G.A.; Visser, J.H.M.; Bigaj-van Vliet, A.J.

    2012-01-01

    Most high filler concrete studies are based on relatively high contents of powder (cement + filler) (>400 kg m-3). This paper aims to increase the total fly ash content relative to the clinker content, while simultaneously minimizing the total powder content in the concrete to values lower than 300

  3. High-velocity Penetration of Concrete Targets with Three Types of Projectiles: Experiments and Analysis

    Directory of Open Access Journals (Sweden)

    Shuang Zhang

    Full Text Available Abstract This study conducted high-velocity penetration experiments using conventional ogive-nose, double-ogive-nose, and grooved-tapered projectiles of approximately 2.5 kg and initial velocities between 1000 and 1360 m/s to penetrate or perforate concrete targets with unconfined compressive strengths of nominally 40MPa. The penetration performance data of these three types of projectiles with two different types of materials (i.e., AerMet100 and DT300 were obtained. The crater depth model considering both the projectile mass and the initial velocity was proposed based on the test results and a theoretical analysis. The penetration ability and the trajectory stability of these three projectile types were compared and analyzed accordingly. The results showed that, under these experimental conditions, the effects of these two different kinds of projectile materials on the penetration depth and mass erosion rate of projectile were not obvious. The existing models could not reflect the crater depths for projectiles of greater weights or higher velocities, whereas the new model established in this study was reliable. The double-ogive-nose has a certain effect of drag reduction. Thus, the double-ogive-nose projectile has a higher penetration ability than the conventional ogive-nose projectile. Meanwhile, the grooved-tapered projectile has a better trajectory stability, because the convex parts of tapered shank generated the restoring moment to stabilize the trajectory.

  4. Final Technical Report: Integrated Distribution-Transmission Analysis for Very High Penetration Solar PV

    Energy Technology Data Exchange (ETDEWEB)

    Palmintier, Bryan [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Hale, Elaine [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Hansen, Timothy M. [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Jones, Wesley [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Biagioni, David [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Baker, Kyri [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Wu, Hongyu [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Giraldez, Julieta [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Sorensen, Harry [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Lunacek, Monte [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Merket, Noel [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Jorgenson, Jennie [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States)); Hodge, Bri-Mathias [NREL (National Renewable Energy Laboratory (NREL), Golden, CO (United States))

    2016-01-29

    Transmission and distribution simulations have historically been conducted separately, echoing their division in grid operations and planning while avoiding inherent computational challenges. Today, however, rapid growth in distributed energy resources (DERs)--including distributed generation from solar photovoltaics (DGPV)--requires understanding the unprecedented interactions between distribution and transmission. To capture these interactions, especially for high-penetration DGPV scenarios, this research project developed a first-of-its-kind, high performance computer (HPC) based, integrated transmission-distribution tool, the Integrated Grid Modeling System (IGMS). The tool was then used in initial explorations of system-wide operational interactions of high-penetration DGPV.

  5. Electricity demand profile with high penetration of heat pumps in Nordic area

    DEFF Research Database (Denmark)

    Liu, Zhaoxi; Wu, Qiuwei; Nielsen, Arne Hejde

    2013-01-01

    This paper presents the heat pump (HP) demand profile with high HP penetration in the Nordic area in order to achieve the carbon neutrality power system. The calculation method in the European Standard EN14825 was used to estimate the HP electricity demand profile. The study results show...... there will be high power demand from HPs and the selection of supplemental heating for heat pumps has a big impact on the peak electrical power load of heating. The study in this paper gives an estimate of the scale of the electricity demand with high penetration of heat pumps in the Nordic area....

  6. LCA of electricity systems with high wind power penetration

    DEFF Research Database (Denmark)

    Turconi, Roberto; O' Dwyer, C. O.; Flynn, D.

    Electricity systems are shifting from being based on fossil fuels towards renewable sources to enhance energy security and mitigate climate change. However, by introducing high shares of variable renewables - such as wind and solar - dispatchable power plants are required to vary their output...... to fulfill the remaining electrical demand, potentially increasing their environmental impacts [1,2]. In this study the environmental impacts of potential short-term future electricity systems in Ireland with high shares of wind power (35-50% of total installed capacity) were evaluated using life cycle...... considered: while not outweighing the benefits from increasing wind energy, cycling emissions are not negligible and should thus be systematically included (i.e. by using emission factors per unit of fuel input rather than per unit of power generated). Cycling emissions increased with the installed wind...

  7. High-Penetration Photovoltaics Standards and Codes Workshop, Denver, Colorado, May 20, 2010: Workshop Proceedings

    Energy Technology Data Exchange (ETDEWEB)

    Coddington, M.; Kroposki, B.; Basso, T.; Lynn, K.; Herig, C.; Bower, W.

    2010-09-01

    Effectively interconnecting high-level penetration of photovoltaic (PV) systems requires careful technical attention to ensuring compatibility with electric power systems. Standards, codes, and implementation have been cited as major impediments to widespread use of PV within electric power systems. On May 20, 2010, in Denver, Colorado, the National Renewable Energy Laboratory, in conjunction with the U.S. Department of Energy (DOE) Office of Energy Efficiency and Renewable Energy (EERE), held a workshop to examine the key technical issues and barriers associated with high PV penetration levels with an emphasis on codes and standards. This workshop included building upon results of the High Penetration of Photovoltaic (PV) Systems into the Distribution Grid workshop held in Ontario California on February 24-25, 2009, and upon the stimulating presentations of the diverse stakeholder presentations.

  8. Chloride Penetration through Cracks in High-Performance Concrete and Surface Treatment System for Crack Healing

    Directory of Open Access Journals (Sweden)

    In-Seok Yoon

    2012-01-01

    Full Text Available For enhancing the service life of concrete structures, it is very important to minimize crack at surface. Even if these cracks are very small, the problem is to which extend these cracks may jeopardize the durability of these decks. It was proposed that crack depth corresponding with critical crack width from the surface is a crucial factor in view of durability design of concrete structures. It was necessary to deal with chloride penetration through microcracks characterized with the mixing features of concrete. This study is devoted to examine the effect of high strength concrete and reinforcement of steel fiber on chloride penetration through cracks. High strength concrete is regarded as an excellent barrier to resist chloride penetration. However, durability performance of cracked high strength concrete was reduced seriously up to that of ordinary cracked concrete. Steel fiber reinforcement is effective to reduce chloride penetration through cracks because steel fiber reinforcement can lead to reduce crack depth significantly. Meanwhile, surface treatment systems are put on the surface of the concrete in order to seal the concrete. The key-issue is to which extend a sealing is able to ensure that chloride-induced corrosion can be prevented. As a result, penetrant cannot cure cracks, however, coating and combined treatment can prevent chloride from flowing in concrete with maximum crack width of 0.06 mm and 0.08 mm, respectively.

  9. Hydrodynamic analysis and design of high-level radioactive waste disposal model penetrators

    International Nuclear Information System (INIS)

    Visintini, L.; Mazazzi, R.; Murray, C.N.

    1991-01-01

    The Commission of the European Communities is studying in the framework of the NEA/OECD Internationally Co-ordinational Seabed Programme the feasibility of using deep ocean sedimentary geological formations as a final disposal medium for vitrified high level waste and fuel elements. At present, two options are being considered for the embedment of such wastes in the sediment column, drilling and free fall penetrators. In the second case, the high level waste would be contained in specially designed drums which would be placed into torpedo-shaped projectiles. These penetrators would then be launched from a semi-submersible platform or ship and allowed to fall freely through the water column (≅ 5 km depth) and to bury themselves within the sediment column. The present article reports some work which has been carried out by the Joint Research Centre, Ispra Establishment on designing large model penetrators for tests at two sites in the North Atlantic

  10. Environmental impacts of high penetration renewable energy scenarios for Europe

    Science.gov (United States)

    Berrill, Peter; Arvesen, Anders; Scholz, Yvonne; Gils, Hans Christian; Hertwich, Edgar G.

    2016-01-01

    The prospect of irreversible environmental alterations and an increasingly volatile climate pressurises societies to reduce greenhouse gas emissions, thereby mitigating climate change impacts. As global electricity demand continues to grow, particularly if considering a future with increased electrification of heat and transport sectors, the imperative to decarbonise our electricity supply becomes more urgent. This letter implements outputs of a detailed power system optimisation model into a prospective life cycle analysis framework in order to present a life cycle analysis of 44 electricity scenarios for Europe in 2050, including analyses of systems based largely on low-carbon fossil energy options (natural gas, and coal with carbon capture and storage (CCS)) as well as systems with high shares of variable renewable energy (VRE) (wind and solar). VRE curtailments and impacts caused by extra energy storage and transmission capabilities necessary in systems based on VRE are taken into account. The results show that systems based largely on VRE perform much better regarding climate change and other impact categories than the investigated systems based on fossil fuels. The climate change impacts from Europe for the year 2050 in a scenario using primarily natural gas are 1400 Tg CO2-eq while in a scenario using mostly coal with CCS the impacts are 480 Tg CO2-eq. Systems based on renewables with an even mix of wind and solar capacity generate impacts of 120-140 Tg CO2-eq. Impacts arising as a result of wind and solar variability do not significantly compromise the climate benefits of utilising these energy resources. VRE systems require more infrastructure leading to much larger mineral resource depletion impacts than fossil fuel systems, and greater land occupation impacts than systems based on natural gas. Emissions and resource requirements from wind power are smaller than from solar power.

  11. Environmental impacts of high penetration renewable energy scenarios for Europe

    International Nuclear Information System (INIS)

    Berrill, Peter; Arvesen, Anders; Hertwich, Edgar G; Scholz, Yvonne; Gils, Hans Christian

    2016-01-01

    The prospect of irreversible environmental alterations and an increasingly volatile climate pressurises societies to reduce greenhouse gas emissions, thereby mitigating climate change impacts. As global electricity demand continues to grow, particularly if considering a future with increased electrification of heat and transport sectors, the imperative to decarbonise our electricity supply becomes more urgent. This letter implements outputs of a detailed power system optimisation model into a prospective life cycle analysis framework in order to present a life cycle analysis of 44 electricity scenarios for Europe in 2050, including analyses of systems based largely on low-carbon fossil energy options (natural gas, and coal with carbon capture and storage (CCS)) as well as systems with high shares of variable renewable energy (VRE) (wind and solar). VRE curtailments and impacts caused by extra energy storage and transmission capabilities necessary in systems based on VRE are taken into account. The results show that systems based largely on VRE perform much better regarding climate change and other impact categories than the investigated systems based on fossil fuels. The climate change impacts from Europe for the year 2050 in a scenario using primarily natural gas are 1400 Tg CO 2 -eq while in a scenario using mostly coal with CCS the impacts are 480 Tg CO 2 -eq. Systems based on renewables with an even mix of wind and solar capacity generate impacts of 120–140 Tg CO 2 -eq. Impacts arising as a result of wind and solar variability do not significantly compromise the climate benefits of utilising these energy resources. VRE systems require more infrastructure leading to much larger mineral resource depletion impacts than fossil fuel systems, and greater land occupation impacts than systems based on natural gas. Emissions and resource requirements from wind power are smaller than from solar power. (letter)

  12. High-resolution mapping, modeling, and evolution of subsurface geomorphology using ground-penetrating radar techniques

    Digital Repository Service at National Institute of Oceanography (India)

    Loveson, V.J.; Gujar, A.R.

    subsurface. It has been useful to decipher shallow geomorphic structures having various options to use different antennas for different depth penetration (0-30 m) with higher resolution.   7.2 Principles of GPR  Ground Penetrating Radar (GPR) was invented... about 90m. Flat and plain land is being used, at present, for agriculture (paddy cultivation) practice. Sand dunes are low lying and highly reworked due to social forestry plantation (acacia) activities. 13    7.8.6 Paleo­Lagoon  GPR data shows two...

  13. Penetration of Liquid Jets into a High-velocity Air Stream

    Science.gov (United States)

    Chelko, Louis J

    1950-01-01

    Data are presented showing the penetration characteristics of liquid jets directed approximately perpendicular to a high-velocity air stream for jet-nozzle-throat diameters from 0.0135 to 0.0625 inch, air stream densities from 0.0805 to 0.1365 pound per cubic foot, liquid jet velocities from 168.1 to 229.0 feet per second and a liquid jet density of approximately 62 pounds per cubic foot. The data were analyzed and a correlation was developed that permitted the determination of the penetration length of the liquid jet for any operation condition within the range of variables investigated.

  14. High performance ground penetrating radar survey of TA-49/Area 2. Final report

    International Nuclear Information System (INIS)

    Hoeberling, R.F.; Rangel, M.J. III

    1994-09-01

    The results of high performance ground penetrating radar study of Area 2 at Technical Area 49 are presented. The survey was commissioned as part of Los Alamos Laboratory's continuing Environmental Remediation program and was completed and analyzed before borehole studies in Area 2 were started. Based upon the ground penetrating radar results, the location of one of the planned boreholes was moved to assure the drilling area was as safe as possible. While earlier attempts to use commercial radar devices at this facility had not been successful, the radar and digital processing system developed at Los Alamos were able to significantly improve the buried physical detail of the site

  15. Integration of electric drive vehicles in the Danish electricity network with high wind power penetration

    DEFF Research Database (Denmark)

    Chandrashekhara, Divya K; Østergaard, Jacob; Larsen, Esben

    2010-01-01

    /conventional) which are likely to fuel these cars. The study was carried out considering the Danish electricity network state around 2025, when the EDV penetration levels would be significant enough to have an impact on the power system. Some of the interesting findings of this study are - EDV have the potential......This paper presents the results of a study carried out to examine the feasibility of integrating electric drive vehicles (EDV) in the Danish electricity network which is characterised by high wind power penetration. One of the main aims of this study was to examine the effect of electric drive...... vehicles on the Danish electricity network, wind power penetration and electricity market. In particular the study examined the effect of electric drive vehicles on the generation capacity constraints, load curve, cross border transmission capacity and the type of generating sources (renewable...

  16. A Dynamic Programming based method for optimizing power system restoration with high wind power penetration

    DEFF Research Database (Denmark)

    Hu, Rui; Hu, Weihao; Li, Pengfei

    2016-01-01

    and relatively low cost. Thus, many countries are increasing the wind power penetration in their power system step by step, such as Denmark, Spain and Germany. The incremental wind power penetration brings a lot of new issues in operation and programming. The power system sometimes will operate close to its...... stable limits. Once the blackout happens, a well-designed restoration strategy is significant. This paper focuses on how to ameliorate the power system restoration procedures to adapt the high wind power penetration and how to take full advantages of the wind power plants during the restoration....... In this paper, the possibility to exploit the stochastic wind power during restoration was discussed, and a Dynamic Programming (DP) method was proposed to make wind power contribute in the restoration rationally as far as possible. In this paper, the method is tested and verified by a modified IEEE 30 Buses...

  17. Managing high penetration of renewable energy in MV grid by electric vehicle storage

    DEFF Research Database (Denmark)

    Kordheili, Reza Ahmadi; Bak-Jensen, Birgitte; Pillai, Jayakrishnan Radhakrishna

    2015-01-01

    This paper proposes an intelligent algorithm for dealing with high penetration of renewable energy sources (RESs) in the medium voltage by intelligently managing electric vehicles (EVs), as one of the grid flexible loads. The MV grid used in this work is a CIGRE benchmark grid. Different...... residential and industrial loads are considered in this grid. The connection of medium voltage wind turbines to the grid is investigated. The solar panels in this study are residential panels. Also, EVs are located among the buses with residential demand. The study is done for different winter and summer...... scenarios, considering typical load profiles in Denmark. Different scenarios have been studied with different penetration level of RESs in the grid. The results show the capability of the proposed algorithm to reduce voltage deviations among the grid buses, as well as to increase the RES penetration...

  18. Energy Storage Options for Voltage Support in Low-Voltage Grids with High Penetration of Photovoltaic

    DEFF Research Database (Denmark)

    Marra, Francesco; Tarek Fawzy, Y.; Bülo, Thorsten

    2012-01-01

    to be established. In the long term, these solutions should also aim to allow further more PV installed capacity, while meeting the power quality requirements. In this paper, different concepts of energy storage are proposed to ensure the voltage quality requirements in a LV grid with high PV penetration...

  19. Developing High PV Penetration Cases for Frequency Response Study of U.S. Western Interconnection: Preprint

    Energy Technology Data Exchange (ETDEWEB)

    Tan, Jin; Zhang, Yingchen; Veda, Santosh; Elgindy, Tarek; Liu, Yilu

    2017-04-11

    Recent large penetrations of solar photovoltaic (PV) generation and the inertial characteristics of inverter-based generation technologies have caught the attention of those in the electric power industry in the United States. This paper presents a systematic approach to developing test cases of high penetrations of PV for the Western Interconnection. First, to examine the accuracy of the base case model, the Western Electricity Coordinating Council (WECC) model is validated by using measurement data from synchronized phasor measurement units. Based on the 2022 Light Spring case, we developed four high PV penetration cases for the WECC system that are of interest to the industry: 5% PV+15 % wind, 25% PV+15% wind, 45% PV+15% wind, 65% PV+15% wind). Additionally, a method to project PV is proposed that is based on collected, realistic PV distribution information, including the current and future PV power plant locations and penetrations in the WECC system. Both the utility-scale PV plant and residential rooftop PV are included in this study.

  20. Differences in Femoral Head Penetration Between Highly Cross-Linked Polyethylene Cemented Sockets and Uncemented Liners.

    Science.gov (United States)

    Morita, Daigo; Seki, Taisuke; Higuchi, Yoshitoshi; Takegami, Yasuhiko; Ishiguro, Naoki

    2017-12-01

    This study aimed at investigating differences in femoral head penetration between highly cross-linked polyethylene (HXLPE) cemented sockets and uncemented liners during 5 years postoperatively. Ninety-six patients (106 hips) with a mean age of 64.4 (range, 35-83) years underwent total hip arthroplasty using a HXLPE cemented socket or liner and were respectively divided into cemented (35 patients [37 hips]) and uncemented (61 patients [69 hips]) groups. Femoral head penetrations were evaluated on both anteroposterior (AP)-view and Lauenstein-view radiographs, and mean polyethylene (PE) wear rates were calculated based on femoral head penetration from 2 to 5 years. Multivariate analyses were performed to assess risk factors for PE wear. At 5 years postoperatively, the cemented and uncemented groups exhibited proximal direction femoral head penetrations of 0.103 mm and 0.124 mm (P = .226) and anterior direction penetrations of 0.090 mm and 0.151 mm (P = .002), respectively. The corresponding mean PE wear rates were 0.004 mm/y and 0.009 mm/y in the AP-view (P = .286) and 0.005 mm/y and 0.012 mm/y in the Lauenstein-view (P = .168), respectively. Left-side operation and high activity were independent risk factors for PE wear on AP-view. When HXLPE was used, all mean PE wear rates were very low and those of cemented sockets and uncemented liners were very similar. PE particle theory suggests that the occurrence of osteolysis and related aseptic loosening might consequently decrease. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Outlook and application analysis of energy storage in power system with high renewable energy penetration

    Science.gov (United States)

    Feng, Junshu; Zhang, Fuqiang

    2018-02-01

    To realize low-emission and low-carbon energy production and consumption, large-scale development and utilization of renewable energy has been put into practice in China. And it has been recognized that power system of future high renewable energy shares can operate more reliably with the participation of energy storage. Considering the significant role of storage playing in the future power system, this paper focuses on the application of energy storage with high renewable energy penetration. Firstly, two application modes are given, including demand side application mode and centralized renewable energy farm application mode. Afterwards, a high renewable energy penetration scenario of northwest region in China is designed, and its production simulation with application of energy storage in 2050 has been calculated and analysed. Finally, a development path and outlook of energy storage is given.

  2. High penetration wind generation impacts on spot prices in the Australian national electricity market

    International Nuclear Information System (INIS)

    Cutler, Nicholas J.; Boerema, Nicholas D.; MacGill, Iain F.; Outhred, Hugh R.

    2011-01-01

    This paper explores wind power integration issues for the South Australian (SA) region of the Australian National Electricity Market (NEM) by assessing the interaction of regional wind generation, electricity demand and spot prices over 2 recent years of market operation. SA's wind energy penetration has recently surpassed 20% and it has only a limited interconnection with other regions of the NEM. As such, it represents an interesting example of high wind penetration in a gross wholesale pool market electricity industry. Our findings suggest that while electricity demand continues to have the greatest influence on spot prices in SA, wind generation levels have become a significant secondary influence, and there is an inverse relationship between wind generation and price. No clear relationship between wind generation and demand has been identified although some periods of extremely high demand may coincide with lower wind generation. Periods of high wind output are associated with generally lower market prices, and also appear to contribute to extreme negative price events. The results highlight the importance of electricity market and renewable policy design in facilitating economically efficient high wind penetrations. - Highlights: → In South Australia (SA) wind generation is having an influence on market prices. → Little or no correlation is found between wind generation and demand. → Wind farms in SA are receiving a lower average price than in other States. → The results highlight the importance of appropriate electricity market design.

  3. Facing the challenges of distribution systems operation with high wind power penetration

    DEFF Research Database (Denmark)

    Das, Kaushik; Altin, Müfit; Hansen, Anca Daniela

    2017-01-01

    power flow in 60kV distribution networks through controlling the ability of wind power plants (WPPs) to generate or absorb reactive power. This paper aims to understand the characteristics of a distribution network with high penetration of distributed generation. A detailed analysis of the active...... and reactive power flows in a real distribution network under different wind and load conditions based on actual measurements is performed in order to understand the correlation between the consumption, wind power production, and the network losses. Conclusive remarks are presented, briefly expressing......This paper addresses the challenges associated with the operation of a distribution system with high penetration of wind power. The paper presents some preliminary investigations of an ongoing Danish research work, which has as main objective to reduce the network losses by optimizing the reactive...

  4. Penetration of hydrogen isotopes through EhI 698 alloy at high pressure and temperature

    International Nuclear Information System (INIS)

    Bystritskij, V.M.; Voznyak, Ya.; Granovskij, V.B.

    1986-01-01

    The paper deals with investigations of the process of hydrogen and deuterium penetration through the high-temperature alloy EhI-698 at a pressure up to 1 kbar and temperature up to 1050 K. Parameters of the process obey Sieverts's law and can be described by Arrenius's and Vant-Goff's equations. The obtained results lead to a conclusion that the alloy EhI-698 is good for vessels to be employed in hydrogen media

  5. Electric Vehicles for Improved Operation of Power Systems with High Wind Power Penetration

    OpenAIRE

    Larsen, Esben; Chandrashekhara, Divya K; Østergaard, Jacob

    2008-01-01

    In a power system with a high share of wind energy the wind fluctuation causes a variation in the power generation, which must be compensated from other sources. The situation in Denmark with a penetration of more than 20% wind in yearly average is presented. The introduction of electric drive vehicles (EDV) as flexible loads can improve the system operation. Bidirectional power exchange through batteries (vehicle to grid) can be seen as a storage system in the grid. An analysis of possible e...

  6. Review of Congestion Management Methods for Distribution Networks with High Penetration of Distributed Energy Resources

    DEFF Research Database (Denmark)

    Huang, Shaojun; Wu, Qiuwei; Liu, Zhaoxi

    2014-01-01

    This paper reviews the existing congestion management methods for distribution networks with high penetration of DERs documented in the recent research literatures. The congestion management methods for distribution networks reviewed can be grouped into two categories – market methods and direct...... control methods. The market methods consist of dynamic tariff, distribution capacity market, shadow price and flexible service market. The direct control methods are comprised of network reconfiguration, reactive power control and active power control. Based on the review of the existing methods...

  7. High Penetration Solar PV Deployment Sunshine State Solar Grid Initiative (SUNGRIN)

    Energy Technology Data Exchange (ETDEWEB)

    Meeker, Rick [Nhu Energy, Inc., Tallahassee, FL (United States); Florida State Univ., Tallahassee, FL (United States); Steurer, Mischa [Florida State Univ., Tallahassee, FL (United States); Faruque, MD Omar [Florida State Univ., Tallahassee, FL (United States); Langston, James [Florida State Univ., Tallahassee, FL (United States); Schoder, Karl [Florida State Univ., Tallahassee, FL (United States); Ravindra, Harsha [Florida State Univ., Tallahassee, FL (United States); Hariri, Ali [Florida State Univ., Tallahassee, FL (United States); Moaveni, Houtan [New York Power Authority (NYPA), New York (United States); University of Central Florida, Florida Solar Energy Center, Cocoa, FL (Unitied States); Click, Dave [ESA Renewables, LLC, Sanford, FL (United States); University of Central Florida, Florida Solar Energy Center, Cocoa, FL (United States); Reedy, Bob [University of Central Florida, Florida Solar Energy Center, Cocoa, FL (United States)

    2015-05-31

    The report provides results from the Sunshine State Solar Grid Initiative (SUNGRIN) high penetration solar PV deployment project led by Florida State University’s (FSU) Center for Advanced Power Systems (CAPS). FSU CAPS and industry and university partners have completed a five-year effort aimed at enabling effective integration of high penetration levels of grid-connected solar PV generation. SUNGRIN has made significant contributions in the development of simulation-assisted techniques, tools, insight and understanding associated with solar PV effects on electric power system (EPS) operation and the evaluation of mitigation options for maintaining reliable operation. An important element of the project was the partnership and participation of six major Florida utilities and the Florida Reliability Coordinating Council (FRCC). Utilities provided details and data associated with actual distribution circuits having high-penetration PV to use as case studies. The project also conducted foundational work supporting future investigations of effects at the transmission / bulk power system level. In the final phase of the project, four open-use models with built-in case studies were developed and released, along with synthetic solar PV data sets, and tools and techniques for model reduction and in-depth parametric studies of solar PV impact on distribution circuits. Along with models and data, at least 70 supporting MATLAB functions have been developed and made available, with complete documentation.

  8. Effect of Using Metakaolin on Chloride Ion Penetration in High Performance Steel Fiber Reinforced Concrete

    Directory of Open Access Journals (Sweden)

    Adnan Mohammed Shihab

    2016-03-01

    Full Text Available This paper attempts to reduce the penetrability of high performance steel fiber reinforced concrete to chloride ions originating from external sources, by using High Reactivity Metakaolin (HRM as a highly active pozzolanic material, in order to prolong the time to initiation of the steel fibers corrosion and to minimize concrete damage that may occur due to the exposure to chloride ion penetration. According to pozzolanic activity index (P.A.I., 8% content of HRM was used as a partial replacement by weight of cement with 2% steel fibers by volume of concrete. During the exposure period of 300 days in 4.5% of NaCl solution, the total and free chloride contents (Cltotal, Clfree with the chloride profiles at the ages of 28 and 300 days were investigated. Also the rapid chloride penetrability test (RCPT, compressive and flexural strengths tests were conducted at the ages of 28, 90, 180 and 300 days. Results showed that the incorporation of 8% HRM caused a reduction in the (Clfree/Cltota ratio, the chloride penetration depth and the electrical conductivity with percentages of 21%, 40% and 43% respectively after 300 days exposure to chloride solution in comparing with the mix of 0% HRM. Results also indicated that the losses in compressive and flexural strengths after exposure of 300 days to chloride solution for the mix incorporating 8% HRM were by 5% and 5.8% respectively while they reached 9.5% and 11% respectively for the mix without HRM in relation to the correspondent test specimens cured in tap water.

  9. Production price of hydrogen from grid connected electrolysis in a power market with high wind penetration

    International Nuclear Information System (INIS)

    Joergensen, Claus; Ropenus, Stephanie

    2008-01-01

    In liberalized power markets, there are significant power price fluctuations due to independently varying changes in demand and supply, the latter being substantial in systems with high wind power penetration. In such systems, hydrogen production by grid connected electrolysis can be cost optimized by operating an electrolyzer part time. This paper presents a study on the minimization of the hydrogen production price and its dependence on estimated power price fluctuations. The calculation of power price fluctuations is based on a parameterization of existing data on wind power production, power consumption and power price evolution in the West Danish power market area. The price for hydrogen is derived as a function of the optimal electrolyzer operation hours per year for four different wind penetration scenarios. It is found to amount to 0.41-0.45 EUR/Nm 3 . The study further discusses the hydrogen price sensitivity towards investment costs and the contribution from non-wind power sources. (author)

  10. Production price of hydrogen from grid connected electrolysis in a power market with high wind penetration

    Energy Technology Data Exchange (ETDEWEB)

    Joergensen, Claus [Materials Research Department, Risoe National Laboratory for Sustainable Energy, Technical University of Denmark, P.O. Box 49, Frederiksborgvej 399, DK-4000 Roskilde (Denmark); Ropenus, Stephanie [Systems Analysis Department, Risoe National Laboratory for Sustainable Energy, Technical University of Denmark, P.O. Box 49, Frederiksborgvej 399, DK-4000 Roskilde (Denmark)

    2008-10-15

    In liberalized power markets, there are significant power price fluctuations due to independently varying changes in demand and supply, the latter being substantial in systems with high wind power penetration. In such systems, hydrogen production by grid connected electrolysis can be cost optimized by operating an electrolyzer part time. This paper presents a study on the minimization of the hydrogen production price and its dependence on estimated power price fluctuations. The calculation of power price fluctuations is based on a parameterization of existing data on wind power production, power consumption and power price evolution in the West Danish power market area. The price for hydrogen is derived as a function of the optimal electrolyzer operation hours per year for four different wind penetration scenarios. It is found to amount to 0.41-0.45 EUR/Nm{sup 3}. The study further discusses the hydrogen price sensitivity towards investment costs and the contribution from non-wind power sources. (author)

  11. Production price of hydrogen from grid connected electrolysis in a power market with high wind penetration.

    Energy Technology Data Exchange (ETDEWEB)

    Joergensen, Claus [Materials Research Department, Risoe National Laboratory for Sustainable Energy, Technical University of Denmark, P.O. Box 49, Frederiksborgvej 399, DK-4000 Roskilde (Denmark); Ropenus, Stephanie [Systems Analysis Department, Risoe National Laboratory for Sustainable Energy, Technical University of Denmark, P.O. Box 49, Frederiksborgvej 399, DK-4000 Roskilde (Denmark)

    2008-10-15

    In liberalized power markets, there are significant power price fluctuations due to independently varying changes in demand and supply, the latter being substantial in systems with high wind power penetration. In such systems, hydrogen production by grid connected electrolysis can be cost optimized by operating an electrolyzer part time. This paper presents a study on the minimization of the hydrogen production price and its dependence on estimated power price fluctuations. The calculation of power price fluctuations is based on a parameterization of existing data on wind power production, power consumption and power price evolution in the West Danish power market area. The price for hydrogen is derived as a function of the optimal electrolyzer operation hours per year for four different wind penetration scenarios. It is found to amount to 0.41-0.45 EUR/Nm{sup 3}. The study further discusses the hydrogen price sensitivity towards investment costs and the contribution from non-wind power sources. (author)

  12. High-accuracy defect sizing for CRDM penetration adapters using the ultrasonic TOFD technique

    International Nuclear Information System (INIS)

    Atkinson, I.

    1995-01-01

    Ultrasonic time-of-flight diffraction (TOFD) is the preferred technique for critical sizing of throughwall orientated defects in a wide range of components, primarily because it is intrinsically more accurate than amplitude-based techniques. For the same reason, TOFD is the preferred technique for sizing the cracks in control rod drive mechanism (CRDM) penetration adapters, which have been the subject of much recent attention. Once the considerable problem of restricted access for the UT probes has been overcome, this inspection lends itself to very high accuracy defect sizing using TOFD. In qualification trials under industrial conditions, depth sizing to an accuracy of ≤ 0.5 mm has been routinely achieved throughout the full wall thickness (16 mm) of the penetration adapters, using only a single probe pair and without recourse to signal processing. (author)

  13. Penetrating bladder trauma: a high risk factor for associated rectal injury.

    Science.gov (United States)

    Pereira, B M; Reis, L O; Calderan, T R; de Campos, C C; Fraga, G P

    2014-01-01

    Demographics and mechanisms were analyzed in prospectively maintained level one trauma center database 1990-2012. Among 2,693 trauma laparotomies, 113 (4.1%) presented bladder lesions; 51.3% with penetrating injuries (n = 58); 41.3% (n = 24) with rectal injuries, males corresponding to 95.8%, mean age 29.8 years; 79.1% with gunshot wounds and 20.9% with impalement; 91.6% arriving the emergence room awake (Glasgow 14-15), hemodynamically stable (average systolic blood pressure 119.5 mmHg); 95.8% with macroscopic hematuria; and 100% with penetrating stigmata. Physical exam was not sensitive for rectal injuries, showing only 25% positivity in patients. While 60% of intraperitoneal bladder injuries were surgically repaired, extraperitoneal ones were mainly repaired using Foley catheter alone (87.6%). Rectal injuries, intraperitoneal in 66.6% of the cases and AAST-OIS grade II in 45.8%, were treated with primary suture plus protective colostomy; 8.3% were sigmoid injuries, and 70.8% of all injuries had a minimum stool spillage. Mean injury severity score was 19; mean length of stay 10 days; 20% of complications with no death. Concomitant rectal injuries were not a determinant prognosis factor. Penetrating bladder injuries are highly associated with rectal injuries (41.3%). Heme-negative rectal examination should not preclude proctoscopy and eventually rectal surgical exploration (only 25% sensitivity).

  14. Economic Justification of Concentrating Solar Power in High Renewable Energy Penetrated Power Systems

    Energy Technology Data Exchange (ETDEWEB)

    Hodge, Brian S [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Kroposki, Benjamin D [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Du, Ershun [Tsinghua University; Zhang, Ning [Tsinghua University; Kang, Chongqing [Tsinghua University; Xia, Qing [Tsinghua University

    2018-04-24

    Concentrating solar power (CSP) plants are able to provide both renewable energy and operational flexibility at the same time due to its thermal energy storage (TES). It is ideal generation to power systems lacking in flexibility to accommodate variable renewable energy (VRE) generation such as wind power and photovoltaics. However, its investment cost currently is too high to justify its benefit in terms of providing renewable energy only. In this paper we evaluate the economic benefit of CSP in high renewable energy penetrated power systems from two aspects: generating renewable energy and providing operational flexibility to help accommodating VRE. In order to keep the same renewable energy penetration level during evaluation, we compare the economic costs between the system with a high share of VRE and another in which some part of the VRE generation is replaced by CSP generation. The generation cost of a power system is analyzed through chronological operation simulation over a whole year. The benefit of CSP is quantified into two parts: (1) energy benefit - the saving investment of substituted VRE generation and (2) flexibility benefit - the reduction in operating cost due to substituting VRE with CSP. The break-even investment cost of CSP is further discussed. The methodology is tested on a modified IEEE RTS-79 system. The economic justifications of CSP are demonstrated in two practical provincial power systems with high penetration of renewable energy in northwestern China, Qinghai and Gansu, where the former province has massive inflexible thermal power plants but later one has high share of flexible hydro power. The results suggest that the CSP is more beneficial in Gansu system than in Qinghai. The levelized benefit of CSP, including both energy benefit and flexibility benefit, is about 0.177-0.191 $/kWh in Qinghai and about 0.238-0.300 $/kWh in Gansu, when replacing 5-20% VRE generation with CSP generation.

  15. High-Resolution En Face Images of Microcystic Macular Edema in Patients with Autosomal Dominant Optic Atrophy

    Directory of Open Access Journals (Sweden)

    Kiyoko Gocho

    2013-01-01

    Full Text Available The purpose of this study was to investigate the characteristics of microcystic macular edema (MME determined from the en face images obtained by an adaptive optics (AO fundus camera in patients with autosomal dominant optic atrophy (ADOA and to try to determine the mechanisms underlying the degeneration of the inner retinal cells and RNFL by using the advantage of AO. Six patients from 4 families with ADOA underwent detailed ophthalmic examinations including spectral domain optical coherence tomography (SD-OCT. Mutational screening of all coding and flanking intron sequences of the OPA1 gene was performed by DNA sequencing. SD-OCT showed a severe reduction in the retinal nerve fiber layer (RNFL thickness in all patients. A new splicing defect and two new frameshift mutations with premature termination of the Opa1 protein were identified in three families. A reported nonsense mutation was identified in one family. SD-OCT of one patient showed MME in the inner nuclear layer (INL of the retina. AO images showed microcysts in the en face images of the INL. Our data indicate that AO is a useful method to identify MME in neurodegenerative diseases and may also help determine the mechanisms underlying the degeneration of the inner retinal cells and RNFL.

  16. Renewable Electricity Futures Study. Volume 1. Exploration of High-Penetration Renewable Electricity Futures

    Energy Technology Data Exchange (ETDEWEB)

    Hand, M. M. [National Renewable Energy Lab. (NREL), Golden, CO (United States); Baldwin, S. [U.S. Dept. of Energy, Washington, DC (United States); DeMeo, E. [Renewable Energy Consulting, Chicago, IL (United States); Reilly, J. M. [Massachusetts Inst. of Technology (MIT), Cambridge, MA (United States); Mai, T. [National Renewable Energy Lab. (NREL), Golden, CO (United States); Arent, D. [Joint Inst. for Strategic Energy Analysis, Boulder, CO (United States); Porro, G. [National Renewable Energy Lab. (NREL), Golden, CO (United States); Meshek, M. [National Renewable Energy Lab. (NREL), Golden, CO (United States); Sandor, D. [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2012-06-15

    The Renewable Electricity Futures (RE Futures) Study investigated the challenges and impacts of achieving very high renewable electricity generation levels in the contiguous United States by 2050. The analysis focused on the sufficiency of the geographically diverse U.S. renewable resources to meet electricity demand over future decades, the hourly operational characteristics of the U.S. grid with high levels of variable wind and solar generation, and the potential implications of deploying high levels of renewables in the future. RE Futures focused on technical aspects of high penetration of renewable electricity; it did not focus on how to achieve such a future through policy or other measures. Given the inherent uncertainties involved with analyzing alternative long-term energy futures as well as the multiple pathways that might be taken to achieve higher levels of renewable electricity supply, RE Futures explored a range of scenarios to investigate and compare the impacts of renewable electricity penetration levels (30%–90%), future technology performance improvements, potential constraints to renewable electricity development, and future electricity demand growth assumptions. RE Futures was led by the National Renewable Energy Laboratory (NREL) and the Massachusetts Institute of Technology (MIT). Learn more at the RE Futures website. http://www.nrel.gov/analysis/re_futures/

  17. Renewable Electricity Futures Study. Volume 1: Exploration of High-Penetration Renewable Electricity Futures

    Energy Technology Data Exchange (ETDEWEB)

    Mai, T.; Wiser, R.; Sandor, D.; Brinkman, G.; Heath, G.; Denholm, P.; Hostick, D.J.; Darghouth, N.; Schlosser, A.; Strzepek, K.

    2012-06-01

    The Renewable Electricity Futures (RE Futures) Study investigated the challenges and impacts of achieving very high renewable electricity generation levels in the contiguous United States by 2050. The analysis focused on the sufficiency of the geographically diverse U.S. renewable resources to meet electricity demand over future decades, the hourly operational characteristics of the U.S. grid with high levels of variable wind and solar generation, and the potential implications of deploying high levels of renewables in the future. RE Futures focused on technical aspects of high penetration of renewable electricity; it did not focus on how to achieve such a future through policy or other measures. Given the inherent uncertainties involved with analyzing alternative long-term energy futures as well as the multiple pathways that might be taken to achieve higher levels of renewable electricity supply, RE Futures explored a range of scenarios to investigate and compare the impacts of renewable electricity penetration levels (30%-90%), future technology performance improvements, potential constraints to renewable electricity development, and future electricity demand growth assumptions. RE Futures was led by the National Renewable Energy Laboratory (NREL) and the Massachusetts Institute of Technology (MIT).

  18. Extended-Term Dynamic Simulations with High Penetrations of Photovoltaic Generation.

    Energy Technology Data Exchange (ETDEWEB)

    Concepcion, Ricky James [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Elliott, Ryan Thomas [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Donnelly, Matt [Montana Tech., Butte, MT (United States); Sanchez-Gasca, Juan [GE Energy, Schenectady, NY (United States)

    2016-01-01

    The uncontrolled intermittent availability of renewable energy sources makes integration of such devices into today's grid a challenge. Thus, it is imperative that dynamic simulation tools used to analyze power system performance are able to support systems with high amounts of photovoltaic (PV) generation. Additionally, simulation durations expanding beyond minutes into hours must be supported. This report aims to identify the path forward for dynamic simulation tools to accom- modate these needs by characterizing the properties of power systems (with high PV penetration), analyzing how these properties affect dynamic simulation software, and offering solutions for po- tential problems. We present a study of fixed time step, explicit numerical integration schemes that may be more suitable for these goals, based on identified requirements for simulating high PV penetration systems. We also present the alternative of variable time step integration. To help determine the characteristics of systems with high PV generation, we performed small signal sta- bility studies and time domain simulations of two representative systems. Along with feedback from stakeholders and vendors, we identify the current gaps in power system modeling including fast and slow dynamics and propose a new simulation framework to improve our ability to model and simulate longer-term dynamics.

  19. Voltage magnitude and margin controller for remote industrial microgrid with high wind penetration

    DEFF Research Database (Denmark)

    Cai, Yu; Lin, Jin; Song, Yonghua

    2013-01-01

    It is well known that the remote industrial microgrid is located at the periphery of the grid, which is weakly connected to the main grid. In order to enhance the voltage stability and ensure a good power quality for industries, a voltage magnitude and margin controller based on wind turbines...... is proposed in this paper. This controller includes two parts to improve voltage stability in different time scales by using local measurements. Case studies conducted for a remote microgrid with high wind penetration have proved the effectiveness of the proposed control scheme....

  20. Optimal LFC SMC for three – area power system with high penetration of PV

    Directory of Open Access Journals (Sweden)

    Maksymilian Klimontowicz

    2016-03-01

    Full Text Available Electrical power systems are subjected to new trends appearing in grid structuring, electrical power sources, new control strategies, etc. The introduction of inverterbased distributed energy resources to replace conventional synchronous machines depletes the mechanical inertia, and causing the system to become more sensitive to disturbances. This paper proposed a simple and reliable solution to assure sufficient frequency stability of electrical power systems when subjected to high penetration of decoupled distributed generation. To achieve this, a decentralized sliding mode control was designed to operate as a compensator for conventional load frequency controllers. Subsequently, PV farms and battery energy storage systems were interconnected. Simulated network under different configurations were conducted using MATLAB.

  1. High Kinetic Energy Penetrator Shielding and High Wear Resistance Materials Fabricated with Boron Nitride Nanotubes (BNNTS) and BNNT Polymer Composites

    Science.gov (United States)

    Kang, Jin Ho (Inventor); Sauti, Godfrey (Inventor); Smith, Michael W. (Inventor); Jordan, Kevin C. (Inventor); Park, Cheol (Inventor); Bryant, Robert George (Inventor); Lowther, Sharon E. (Inventor)

    2015-01-01

    Boron nitride nanotubes (BNNTs), boron nitride nanoparticles (BNNPs), carbon nanotubes (CNTs), graphites, or combinations, are incorporated into matrices of polymer, ceramic or metals. Fibers, yarns, and woven or nonwoven mats of BNNTs are used as toughening layers in penetration resistant materials to maximize energy absorption and/or high hardness layers to rebound or deform penetrators. They can be also used as reinforcing inclusions combining with other polymer matrices to create composite layers like typical reinforcing fibers such as Kevlar.RTM., Spectra.RTM., ceramics and metals. Enhanced wear resistance and usage time are achieved by adding boron nitride nanomaterials, increasing hardness and toughness. Such materials can be used in high temperature environments since the oxidation temperature of BNNTs exceeds 800.degree. C. in air. Boron nitride based composites are useful as strong structural materials for anti-micrometeorite layers for spacecraft and space suits, ultra strong tethers, protective gear, vehicles, helmets, shields and safety suits/helmets for industry.

  2. High-resolution geophysical profiling using a stepped-frequency ground penetrating radar

    Energy Technology Data Exchange (ETDEWEB)

    Noon, D; Longstaff, D [The University of Queensland, (Australia)

    1996-05-01

    This paper describes the results of a ground penetrating radar (GPR) system which uses stepped-frequency waveforms to obtain high-resolution geophysical profiles. The main application for this system is the high-resolution mapping of thin coal seam structures, in order to assist surface mining operations in open-cut coal mines. The required depth of penetration is one meter which represents the maximum thickness of coal seams that are designated `thin`. A resolution of five centimeters is required to resolve the minimum thickness of coal (or shale partings) which can be economically recovered in an open-cut coal mine. For this application, a stepped-frequency GPR system has been developed, because of its ultrawide bandwidth (1 to 2 GHz) and high external loop sensitivity (155 dB). The field test results of the stepped-frequency GPR system on a concrete pavement and at two Australian open-cut coal mines are also presented. 7 refs., 5 figs.

  3. Price Forecasting of Electricity Markets in the Presence of a High Penetration of Wind Power Generators

    Directory of Open Access Journals (Sweden)

    Saber Talari

    2017-11-01

    Full Text Available Price forecasting plays a vital role in the day-ahead markets. Once sellers and buyers access an accurate price forecasting, managing the economic risk can be conducted appropriately through offering or bidding suitable prices. In networks with high wind power penetration, the electricity price is influenced by wind energy; therefore, price forecasting can be more complicated. This paper proposes a novel hybrid approach for price forecasting of day-ahead markets, with high penetration of wind generators based on Wavelet transform, bivariate Auto-Regressive Integrated Moving Average (ARIMA method and Radial Basis Function Neural Network (RBFN. To this end, a weighted time series for wind dominated power systems is calculated and added to a bivariate ARIMA model along with the price time series. Moreover, RBFN is applied as a tool to correct the estimation error, and particle swarm optimization (PSO is used to optimize the structure and adapt the RBFN to the particular training set. This method is evaluated on the Spanish electricity market, which shows the efficiency of this approach. This method has less error compared with other methods especially when it considers the effects of large-scale wind generators.

  4. The role of hydrogen in high wind energy penetration electricity systems: the Irish case

    International Nuclear Information System (INIS)

    Gonzalez, A.; McKeogh, E.; Gallachoir, B.O.

    2004-01-01

    The deployment of wind energy is constrained by wind uncontrollability, which poses operational problems on the electricity supply system at high penetration levels, lessening the value of wind-generated electricity to a significant extent. This paper studies the viability of hydrogen production via electrolysis using wind power that cannot be easily accommodated on the system. The potential benefits of hydrogen and its role in enabling a large penetration of wind energy are assessed, within the context of the enormous wind energy resource in Ireland. The exploitation of this wind resource may in the future give rise to significant amounts of surplus wind electricity, which could be used to produce hydrogen, the zero-emissions fuel that many experts believe will eventually replace fossil fuels in the transport sector. In this paper the operation of a wind powered hydrogen production system is simulated and optimised. The results reveal that, even allowing for significant cost-reductions in electrolyser and associated balance-of-plant equipment, low average surplus wind electricity cost and a high hydrogen market price are also necessary to achieve the economic viability of the technology. These conditions would facilitate the installation of electrolysis units of sufficient capacity to allow an appreciable increase in installed wind power in Ireland. The simulation model was also used to determine the CO 2 abatement potential associated with the wind energy/hydrogen production. (author)

  5. A Distributed Robust Dispatch Approach for Interconnected Systems with a High Proportion of Wind Power Penetration

    Directory of Open Access Journals (Sweden)

    Jianwen Ren

    2018-04-01

    Full Text Available This paper proposes a distributed robust dispatch approach to solve the economic dispatch problem of the interconnected systems with a high proportion of wind power penetration. First of all, the basic principle of synchronous alternating direction method of multipliers (SADMM is introduced to solve the economic dispatch problem of the two interconnected regions. Next, the polyhedron set of the robust optimization method is utilized to describe the wind power output. To adjust the conservativeness of the polyhedron set, an adjustment factor of robust conservativeness is introduced. Subsequently, considering the operation characteristics of the DC tie line between the interconnected regions, an economic dispatch model with a high proportion of wind power penetration is established and parallel iteration based on SADMM is used to solve the model. In each iteration, the optimized power of DC tie lines is exchanged between the regions without requiring the participation of the superior dispatch center. Finally, the validity of the proposed model is verified by the examples of the 2-area 6-node interconnected system and the interconnection of several modified New England 39-node systems. The results show that the proposed model can meet the needs of the independent dispatch of regional power grids, effectively deal with the uncertainty of wind power output, and maximize the wind power consumption under the condition of ensuring the safe operation of the interconnected systems.

  6. Environmental acceptability of high-performance alternatives for depleted uranium penetrators

    Energy Technology Data Exchange (ETDEWEB)

    Kerley, C.R.; Easterly, C.E.; Eckerman, K.F. [and others

    1996-08-01

    The Army`s environmental strategy for investigating material substitution and management is to measure system environmental gains/losses in all phases of the material management life cycle from cradle to grave. This study is the first in a series of new investigations, applying material life cycle concepts, to evaluate whether there are environmental benefits from increasing the use of tungsten as an alternative to depleted uranium (DU) in Kinetic Energy Penetrators (KEPs). Current military armor penetrators use DU and tungsten as base materials. Although DU alloys have provided the highest performance of any high-density alloy deployed against enemy heavy armor, its low-level radioactivity poses a number of environmental risks. These risks include exposures to the military and civilian population from inhalation, ingestion, and injection of particles. Depleted uranium is well known to be chemically toxic (kidney toxicity), and workplace exposure levels are based on its renal toxicity. Waste materials containing DU fragments are classified as low-level radioactive waste and are regulated by the Nuclear Regulatory Commission. These characteristics of DU do not preclude its use in KEPs. However, long-term management challenges associated with KEP deployment and improved public perceptions about environmental risks from military activities might be well served by a serious effort to identify, develop, and substitute alternative materials that meet performance objectives and involve fewer environmental risks. Tungsten, a leading candidate base material for KEPS, is potentially such a material because it is not radioactive. Tungsten is less well studied, however, with respect to health impacts and other environmental risks. The present study is designed to contribute to the understanding of the environmental behavior of tungsten by synthesizing available information that is relevant to its potential use as a penetrator.

  7. Highly accurate and fast optical penetration-based silkworm gender separation system

    Science.gov (United States)

    Kamtongdee, Chakkrit; Sumriddetchkajorn, Sarun; Chanhorm, Sataporn

    2015-07-01

    Based on our research work in the last five years, this paper highlights our innovative optical sensing system that can identify and separate silkworm gender highly suitable for sericulture industry. The key idea relies on our proposed optical penetration concepts and once combined with simple image processing operations leads to high accuracy in identifying of silkworm gender. Inside the system, there are electronic and mechanical parts that assist in controlling the overall system operation, processing the optical signal, and separating the female from male silkworm pupae. With current system performance, we achieve a very highly accurate more than 95% in identifying gender of silkworm pupae with an average system operational speed of 30 silkworm pupae/minute. Three of our systems are already in operation at Thailand's Queen Sirikit Sericulture Centers.

  8. Highly prevalent LIPH founder mutations causing autosomal recessive woolly hair/hypotrichosis in Japan and the genotype/phenotype correlations.

    Directory of Open Access Journals (Sweden)

    Kana Tanahashi

    Full Text Available Mutations in LIPH cause of autosomal recessive woolly hair/hypotrichosis (ARWH, and the 2 missense mutations c.736T>A (p.Cys246Ser and c.742C>A (p.His248Asn are considered prevalent founder mutations for ARWH in the Japanese population. To reveal genotype/phenotype correlations in ARWH cases in Japan and the haplotypes in 14 Japanese patients from 14 unrelated Japanese families. 13 patients had woolly hair, and 1 patient had complete baldness since birth. An LIPH mutation search revealed homozygous c.736T>A mutations in 10 of the patients. Compound heterozygous c.736T>A and c.742C>A mutations were found in 3 of the patients, and homozygous c.742C>A mutation in 1 patient. The phenotype of mild hypotrichosis with woolly hair was restricted to the patients with the homozygous c.736T>A mutation. The severe phenotype of complete baldness was seen in only 1 patient with homozygous c.742C>A. Haplotype analysis revealed that the alleles containing the LIPH c.736T>A mutation had a haplotype identical to that reported previously, although 4 alleles out of 5 chromosomes containing the LIPH c.742C>A mutation had a different haplotype from the previously reported founder allele. These alleles with c.742C>A are thought to be the third founder LIPH mutation causing ARWH. To accurately determine the prevalence of the founder mutations, we investigated allele frequencies of those mutations in 819 Japanese controls. Heterozygous c.736T>A mutations were found in 13 controls (allele frequency: 0.0079; carrier rate: 0.016, and heterozygous c.742C>A mutations were found in 2 controls (allele frequency: 0.0012; carrier rate: 0.0024. In conclusion, this study confirms the more accurate allele frequencies of the pathogenic founder mutations of LIPH and shows that there is a third founder mutation in Japan. In addition, the present findings suggest that the mutation patterns of LIPH might be associated with hypotrichosis severity in ARWH.

  9. High-strength uranium-0.8 weight percent titanium alloy penetrators

    International Nuclear Information System (INIS)

    Northcutt, W.G.

    1978-09-01

    Long-rod kinetic-energy penetrators, produced from a uranium-0.8 titanium (U-0.8 Ti) alloy, are normally water quenched from the gamma phase (approximately 800 0 C) and aged to the desired hardness and strength levels. High cooling rates from 800 0 C in U-0.8 Ti alloy cylindrical bodies larger than about 13 mm in diameter cause internal voids, while slower rates of cooling can produce material that is unresponsive to aging. For the present study, elimination of quenching voids was of paramount importance; therefore, a process including the quenching of plate was explored. Vacuum-induction-cast ingots were forged and rolled into plate and cut into blanks from which the penetrators were obtained. Quenched U-0.8 Ti alloy blanks were aged at 350 to 500 0 C to determine the treatment that would provide maximum tensile and impact strengths. Both tensile and impact strengths were maximized by aging in vacuum for six hours at 450 0 C

  10. No novel, high penetrant gene might remain to be found in Japanese patients with unknown MODY.

    Science.gov (United States)

    Horikawa, Yukio; Hosomichi, Kazuyoshi; Enya, Mayumi; Ishiura, Hiroyuki; Suzuki, Yutaka; Tsuji, Shoji; Sugano, Sumio; Inoue, Ituro; Takeda, Jun

    2018-07-01

    MODY 5 and 6 have been shown to be low-penetrant MODYs. As the genetic background of unknown MODY is assumed to be similar, a new analytical strategy is applied here to elucidate genetic predispositions to unknown MODY. We examined to find whether there are major MODY gene loci remaining to be identified using SNP linkage analysis in Japanese. Whole-exome sequencing was performed with seven families with typical MODY. Candidates for novel MODY genes were examined combined with in silico network analysis. Some peaks were found only in either parametric or non-parametric analysis; however, none of these peaks showed a LOD score greater than 3.7, which is approved to be the significance threshold of evidence for linkage. Exome sequencing revealed that three mutated genes were common among 3 families and 42 mutated genes were common in two families. Only one of these genes, MYO5A, having rare amino acid mutations p.R849Q and p.V1601G, was involved in the biological network of known MODY genes through the intermediary of the INS. Although only one promising candidate gene, MYO5A, was identified, no novel, high penetrant MODY genes might remain to be found in Japanese MODY.

  11. Revisiting long-run relations in power markets with high RES penetration

    International Nuclear Information System (INIS)

    Gianfreda, Angelica; Parisio, Lucia; Pelagatti, Matteo

    2016-01-01

    Electricity generation from renewable energy resources (RES) has become increasingly significant to reach EU and emissions reduction targets. At the same time, one of the main EU policy goals has been the creation of a common internal energy market for Europe. In this paper, we focus on these two issues previously studied separately, considering their possible interactions. We first analyze the long-run relationship between day-ahead electricity prices and fuel prices (natural gas and coal) looking at two samples of years characterized by low and high RES penetration, then we explore the integration of EU markets. We show that the electricity–fuel nexus found over 2006–2008 changed dramatically over 2010–2014 for the majority of countries considered. In particular, the long-run dependence of electricity from gas and coal prices is much lower in recent years. Furthermore, our results confirm that the considered EU countries are becoming less integrated as RES-E increases. Our findings suggest that nationally implemented policies to support renewables are successful in increasing RES penetration, but they have lessened the linkage among EU markets, then making integration more difficult to obtain. - Highlights: •RES lower the intra-daily electricity dependence from coal and natural gas. •RES affect the EU wholesale electricity market integration, studied at hourly level. •Overlapping national and supranational policies tackle the achievement of EU targets. •Strong policy coordination is required to avoid that the “greens” promote the “dirtiest”.

  12. Climate and Water Vulnerability of the US Electricity Grid Under High Penetrations of Renewable Energy

    Science.gov (United States)

    Macknick, J.; Miara, A.; O'Connell, M.; Vorosmarty, C. J.; Newmark, R. L.

    2017-12-01

    The US power sector is highly dependent upon water resources for reliable operations, primarily for thermoelectric cooling and hydropower technologies. Changes in the availability and temperature of water resources can limit electricity generation and cause outages at power plants, which substantially affect grid-level operational decisions. While the effects of water variability and climate changes on individual power plants are well documented, prior studies have not identified the significance of these impacts at the regional systems-level at which the grid operates, including whether there are risks for large-scale blackouts, brownouts, or increases in production costs. Adequately assessing electric grid system-level impacts requires detailed power sector modeling tools that can incorporate electric transmission infrastructure, capacity reserves, and other grid characteristics. Here, we present for the first time, a study of how climate and water variability affect operations of the power sector, considering different electricity sector configurations (low vs. high renewable) and environmental regulations. We use a case study of the US Eastern Interconnection, building off the Eastern Renewable Generation Integration Study (ERGIS) that explored operational challenges of high penetrations of renewable energy on the grid. We evaluate climate-water constraints on individual power plants, using the Thermoelectric Power and Thermal Pollution (TP2M) model coupled with the PLEXOS electricity production cost model, in the context of broader electricity grid operations. Using a five minute time step for future years, we analyze scenarios of 10% to 30% renewable energy penetration along with considerations of river temperature regulations to compare the cost, performance, and reliability tradeoffs of water-dependent thermoelectric generation and variable renewable energy technologies under climate stresses. This work provides novel insights into the resilience and

  13. A novel drug delivery gel of terbinafine hydrochloride with high penetration for external use.

    Science.gov (United States)

    Yang, Yan; Ou, Rujing; Guan, Shixia; Ye, Xiaoling; Hu, Bo; Zhang, Yi; Lu, Shufan; Zhou, Yubin; Yuan, Zhongwen; Zhang, Jun; Li, Qing-Guo

    2015-12-01

    Terbinafine hydrochloride is an antifungal drug for onychomycosis. Poor permeability of its external preparation leads to poor curative effect. Transfersomes, also known as flexible liposome, could improve transmission of drug for local external use. Terbinafine hydrochloride-loaded liposome is expected to become a breakthrough on the treatment of onychomycosis. This study is aimed to prepare high skin penetration terbinafine hydrochloride transfersomes with high encapsulation efficiency, appropriate drug loading and good stability. Taking entrapment efficiency as the main indicator, the formulations and the processes of preparation were investigated. Transfersomes with different surfactants were prepared in the optimization processes, and the formulations were optimized through the transdermal test in vitro. As a result, a gel contained transfersomes was obtained with a brief evaluation. Its pharmacokinetic properties of going through the skin were studied by using the micro dialysis technology and liquid chromatography-mass spectrometry to assay the penetration behavior of terbinafine. Mean particle size of the terbinafine hydrochloride transfersomes was 69.6 ± 1.23 nm, and the entrapment efficiency was 95.4% ± 0.51. The content of the gel was 4.45 ± 0.15 mg/g. The accumulated permeation of the transfersomes gel in 12 h was 88.52 ± 4.06 µg cm -2 and the intracutaneous drug detention was 94.38 ± 5.26 µg cm -2 . The results of pharmacokinetic studies showed the C max and area under the curve (AUC) were apparently higher than the commercial cream. The terbinafine hydrochloride transfersomes was highly absorbed by the skin. The absorption rate was significantly higher than that of the commercial cream either in the transdermal test in vitro or in the pharmacokinetic studies in vivo.

  14. Optimal Operation and Dispatch of Voltage Regulation Devices Considering High Penetrations of Distributed Photovoltaic Generation

    Energy Technology Data Exchange (ETDEWEB)

    Mather, Barry A [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Hodge, Brian S [National Renewable Energy Laboratory (NREL), Golden, CO (United States); Cho, Gyu-Jung [Sungkyunkwan University; Oh, Yun-Sik [Sungkyunkwan University; Kim, Min-Sung [Sungkyunkwan University; Kim, Ji-Soo [Sungkyunkwan University; Kim, Chul-Hwan [Sungkyunkwan University

    2017-06-29

    Voltage regulation devices have been traditionally installed and utilized to support distribution voltages. Installations of distributed energy resources (DERs) in distribution systems are rapidly increasing, and many of these generation resources have variable and uncertain power output. These generators can significantly change the voltage profile for a feeder; therefore, in the distribution system planning stage of the optimal operation and dispatch of voltage regulation devices, possible high penetrations of DERs should be considered. In this paper, we model the IEEE 34-bus test feeder, including all essential equipment. An optimization method is adopted to determine the optimal siting and operation of the voltage regulation devices in the presence of distributed solar power generation. Finally, we verify the optimal configuration of the entire system through the optimization and simulation results.

  15. Cost allocation model for distribution networks considering high penetration of distributed energy resources

    DEFF Research Database (Denmark)

    Soares, Tiago; Pereira, Fábio; Morais, Hugo

    2015-01-01

    The high penetration of distributed energy resources (DER) in distribution networks and the competitive environment of electricity markets impose the use of new approaches in several domains. The network cost allocation, traditionally used in transmission networks, should be adapted and used...... in the distribution networks considering the specifications of the connected resources. The main goal is to develop a fairer methodology trying to distribute the distribution network use costs to all players which are using the network in each period. In this paper, a model considering different type of costs (fixed......, losses, and congestion costs) is proposed comprising the use of a large set of DER, namely distributed generation (DG), demand response (DR) of direct load control type, energy storage systems (ESS), and electric vehicles with capability of discharging energy to the network, which is known as vehicle...

  16. NREL/SCE High Penetration PV Integration Project: FY13 Annual Report

    Energy Technology Data Exchange (ETDEWEB)

    Mather, B. A.; Shah, S.; Norris, B. L.; Dise, J. H.; Yu, L.; Paradis, D.; Katiraei, F.; Seguin, R.; Costyk, D.; Woyak, J.; Jung, J.; Russell, K.; Broadwater, R.

    2014-06-01

    In 2010, the National Renewable Energy Laboratory (NREL), Southern California Edison (SCE), Quanta Technology, Satcon Technology Corporation, Electrical Distribution Design (EDD), and Clean Power Research (CPR) teamed to analyze the impacts of high penetration levels of photovoltaic (PV) systems interconnected onto the SCE distribution system. This project was designed specifically to benefit from the experience that SCE and the project team would gain during the installation of 500 megawatts (MW) of utility-scale PV systems (with 1-5 MW typical ratings) starting in 2010 and completing in 2015 within SCE's service territory through a program approved by the California Public Utility Commission (CPUC). This report provides the findings of the research completed under the project to date.

  17. Electric Vehicles for Improved Operation of Power Systems with High Wind Power Penetration

    DEFF Research Database (Denmark)

    Larsen, Esben; Chandrashekhara, Divya K; Østergaard, Jacob

    2008-01-01

    In a power system with a high share of wind energy the wind fluctuation causes a variation in the power generation, which must be compensated from other sources. The situation in Denmark with a penetration of more than 20% wind in yearly average is presented. The introduction of electric drive...... vehicles (EDV) as flexible loads can improve the system operation. Bidirectional power exchange through batteries (vehicle to grid) can be seen as a storage system in the grid. An analysis of possible economical incentives for the vehicle owners will be shown. By control of EDV charging through a price...... signal from the day ahead market the economical incentives for an EDV-owner will be small. If the EDV's can participate in the regulation of the grid through ancillary services the incentives will be increased to an attractive level....

  18. Impacts of reserve methodology on production cost in high-penetration renewable scenarios

    Energy Technology Data Exchange (ETDEWEB)

    Brinkman, G.; Lew, D.; Hummon, M.; Ibanez, E.; Ela, E.; Hodge, B.M. [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2012-07-01

    Prior to wind and solar penetration, electric power systems were designed to handle variability in system load, uncertainty in load forecasts, and contingency events. Frequency regulations reserve typically handles high frequency (less than 5-minute time scale) variability. Contingency reserves supply energy in the case of the loss of a generator or transmission line. Wind and solar photovoltaic generation and variability to electric power system generation that must be balanced by the system operator. New ancillary service products may be necessary to minimize the cost of integrating these variable renewable generators. For example, California ISO is studying incorporating a flexible ramping product to ensure sufficient ramping capability. A flexibility reserve product could help ensure that sufficient capacity is online to handle unexpected variability in wind and solar generation. (orig.)

  19. Analysis of distribution systems with a high penetration of distributed generation

    DEFF Research Database (Denmark)

    Lund, Torsten

    Since the mid eighties, a large number of wind turbines and distributed combined heat and power plants (CHPs) have been connected to the Danish power system. Especially in the Western part, comprising Jutland and Funen, the penetration is high compared to the load demand. In some periods the wind...... power alone can cover the entire load demand. The objective of the work is to investigate the influence of wind power and distributed combined heat and power production on the operation of the distribution systems. Where other projects have focused on the modeling and control of the generators and prime...... movers, the focus of this project is on the operation of an entire distribution system with several wind farms and CHPs. Firstly, the subject of allocation of power system losses in a distribution system with distributed generation is treated. A new approach to loss allocation based on current injections...

  20. Static Equivalent of Distribution Grids With High Penetration of PV Systems

    DEFF Research Database (Denmark)

    Samadi, Afshin; Söder, Lennart; Shayesteh, Ebrahim

    2015-01-01

    High penetrations of photovoltaic (PV) systems within load pockets in distribution grids have changed pure consumers to prosumers. This can cause technical challenges in distribution and transmission grids, such as overvoltage and reverse power flow. Embedding voltage support schemes into PVs...... equivalent that can fairly capture the dominant behavior of the distribution grids. The aim of this paper is to use gray-box modeling concepts to develop a static equivalent of distribution grids comprising a large number of PV systems embedded with voltage support schemes. In the proposed model, the PV...... systems are aggregated as a separate entity, and not as a negative load, which is traditionally done. The results demonstrate the superior quality of the proposed model compared with the model with PV systems as the negative load....

  1. Demand side resource operation on the Irish power system with high wind power penetration

    DEFF Research Database (Denmark)

    Keane, A.; Tuohy, A.; Meibom, Peter

    2011-01-01

    part of the power system plant mix and contribute to the flexible operation of a power system. A model for demand side resources is proposed here that captures its key characteristics for commitment and dispatch calculations. The model is tested on the all island Irish power system, and the operation...... of the functions of conventional peaking plant. Demand side resources are also shown to be capable of improving the reliability of the system, with reserve capability identified as a key requirement in this respect....... of the model is simulated over one year in both a stochastic and deterministic mode, to illustrate the impact of wind and load uncertainty. The results illustrate that demand side resources can contribute to the efficient, flexible operation of systems with high penetrations of wind by replacing some...

  2. Capacity Payments in Restructured Markets under Low and High Penetration Levels of Renewable Energy

    Energy Technology Data Exchange (ETDEWEB)

    Jenkin, Thomas [National Renewable Energy Lab. (NREL), Golden, CO (United States); Beiter, Philipp [National Renewable Energy Lab. (NREL), Golden, CO (United States); Margolis, Robert [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2016-02-11

    There is considerable debate about the degree to which restructured markets perform successfully in their use of capacity markets. In providing appropriate incentives for new and existing generation to meet reliability requirements, a variety of capacity market designs have developed across RTOs and ISOs in the United States and internationally. Growing levels of variable renewable energy (VRE) resources arguably create new challenges for capacity market designs, because VREs suppress energy prices while providing relatively little capacity, with these effects increase with VRE penetration. The purpose of this report is threefold. First, we provide a brief outline of the purpose and design of various capacity markets under consideration using variable resource requirement (VRR) demand curves. Second, we discuss some of the main challenges raised in existing literature and a set of interviews that we conducted with market participants, regulators, and observers, including where there substantive differences in opinion. Third, we consider some of the challenges that may be specific to higher penetration levels of VRE. While the well known 'merit order' effect from VRE can be expected to suppress wholesale energy prices and revenue, this may be partly mitigated by increased capacity payments and the greater importance of AS payments for flexible capacity. The potential for greater reliance on capacity markets for generator revenues may amplify any inefficiency and costs associated with capacity price volatility and other suboptimal market design choices. Regulatory intervention to ensure adequate capacity payments and ancillary service revenue may become more prevalent under current market designs as the timescale for market signals shifts increasingly from near term (e.g., day-ahead in wholesale electricity markets) to longer term (annual intervals in capacity markets). Our review and discussion with market participants suggest substantive challenges may

  3. Optimizing the U.S. Electric System with a High Penetration of Renewables

    Science.gov (United States)

    Corcoran, B. A.; Jacobson, M. Z.

    2013-12-01

    As renewable energy generators are increasingly being installed throughout the U.S., there is growing interest in interconnecting diverse renewable generators (primarily wind and solar) across large geographic areas through an enhanced transmission system. This reduces variability in the aggregate power output, increases system reliability, and allows for the development of the best overall group of renewable technologies and sites to meet the load. Studies are therefore needed to determine the most efficient and economical plan to achieve large area interconnections in a future electric system with a high penetration of renewables. This research quantifies the effects of aggregating electric load together with diverse renewable generation throughout the ten Federal Energy Regulatory Commission (FERC) regions in the contiguous U.S. A deterministic linear program has been built in AMPL (A Mathematical Programming Language) to solve for the least-cost organizational structure and system (generators, transmission, and storage) for a highly renewable electric grid. The analysis will 1) examine a highly renewable 2006 electric system, including various sensitivity cases and additional system components such as additional load from electric vehicles, and 2) create a 'roadmap' from the existing 2006 system to a highly renewable system in 2030, accounting for projected price and demand changes and generator retirements based on age and environmental regulations. Ideally, results from this study will offer insight for a federal renewable energy policy (such as a renewable portfolio standard) and how to best organize U.S. regions for transmission planning.

  4. [Autosomal dominant polycystic kidney].

    Science.gov (United States)

    Jorge Adad, S; Estevão Barbosa, M; Fácio Luíz, J M; Furlan Rodrigues, M C; Iwamoto, S

    1996-01-01

    A 48-year-old male had autosomic dominant polycystic kidneys with dimensions, to the best of our knowledge, never previously reported; the right kidney weighed 15,100 g and measured 53 x 33 x 9cm and the left one 10.200 g and 46 x 21 x 7cm, with cysts measuring up to 14cm in diameter. Nephrectomy was done to control persistent hematuria and to relief disconfort caused by the large kidneys. The renal function is stable four years after transplantation.

  5. Ultra-rare mutation in long-range enhancer predisposes to thyroid carcinoma with high penetrance.

    Directory of Open Access Journals (Sweden)

    Huiling He

    Full Text Available Thyroid cancer shows high heritability but causative genes remain largely unknown. According to a common hypothesis the genetic predisposition to thyroid cancer is highly heterogeneous; being in part due to many different rare alleles. Here we used linkage analysis and targeted deep sequencing to detect a novel single-nucleotide mutation in chromosome 4q32 (4q32A>C in a large pedigree displaying non-medullary thyroid carcinoma (NMTC. This mutation is generally ultra-rare; it was not found in 38 NMTC families, in 2676 sporadic NMTC cases or 2470 controls. The mutation is located in a long-range enhancer element whose ability to bind the transcription factors POU2F and YY1 is significantly impaired, with decreased activity in the presence of the C- allele compared with the wild type A-allele. An enhancer RNA (eRNA is transcribed in thyroid tissue from this region and is greatly downregulated in NMTC tumors. We suggest that this is an example of an ultra-rare mutation predisposing to thyroid cancer with high penetrance.

  6. Correction of High Astigmatism after Penetrating Keratoplasty with Toric Multifocal Intraocular Lens Implantation

    Directory of Open Access Journals (Sweden)

    Raffaele Nuzzi

    2017-07-01

    Full Text Available After penetrating keratoplasty (PK, high astigmatism is often induced, being frequently about 4–6 dpt. According to the entity and typology of astigmatism, different methods of correction can be used. Selective suture removal, relaxing incisions, wedge resections, compression sutures, photorefractive keratectomy, and laser-assisted in situ keratomileusis can reduce corneal astigmatism and ametropia, but meanwhile they can cause a reduction in the corneal integrity and cause an over- or undercorrection. In case of moderate-to-high regular astigmatisms, the authors propose a toric multifocal intraocular lens (IOL implantation to preserve the corneal integrity (especially in PK after herpetic corneal leukoma keratitis. We evaluated a 45-year-old patient who at the age of 30 was subjected to PK in his left eye due to corneal leukoma herpetic keratitis, which led to high astigmatism (7.50 dpt cyl. 5°. The patient was subjected to phacoemulsification and customized toric multifocal IOL implantation in his left eye. The correction of PK-induced residual astigmatism with a toric IOL implantation is an excellent choice but has to be evaluated in relation to patient age, corneal integrity, longevity graft, and surgical risk. It seems to be a well-tolerated therapeutic choice and with good results.

  7. Quantifying snow and vegetation interactions in the high arctic based on ground penetrating radar (GPR)

    DEFF Research Database (Denmark)

    Gacitúa, G.; Bay, C.; Tamstorf, M.

    2013-01-01

    Arctic in Northeast Greenland. We used ground penetrating radar (GPR) for snow thickness measurements across the Zackenberg valley. Measurements were integrated to the physical conditions that support the vegetation distribution. Descriptive statistics and correlations of the distribution of each...

  8. Hermetic cable penetrations for containments of nuclear power reactors meet high safety standards

    International Nuclear Information System (INIS)

    Kusserow, J.; Gurr, W.; Pflug, H.

    1985-05-01

    Different types of cable penetrations for containments of nuclear power reactors have been developed and fabricated in the GDR. The technical parameters achieved are in accordance with the radiation protection requirements

  9. Melting Penetration Simulation of Fe-U System at High Temperature Using MPS-LER

    International Nuclear Information System (INIS)

    Mustari, A P A; Irwanto, Dwi; Yamaji, A

    2016-01-01

    Melting penetration information of Fe-U system is necessary for simulating the molten core behavior during severe accident in nuclear power plants. For Fe-U system, the information is mainly obtained from experiment, i.e. TREAT experiment. However, there is no reported data on SS304 at temperature above 1350°C. The MPS-LER has been developed and validated to simulate melting penetration on Fe-U system. The MPS-LER modelled the eutectic phenomenon by solving the diffusion process and by applying the binary phase diagram criteria. This study simulates the melting penetration of the system at higher temperature using MPS-LER. Simulations were conducted on SS304 at 1400, 1450 and 1500°C. The simulation results show rapid increase of melting penetration rate. (paper)

  10. Technical impacts of high penetration levels of wind power on power system stability

    OpenAIRE

    Flynn, Damian; Rather, Z.; Ardal, Atle; Darco, Salvatore; Hansen, Anca Daniela; Cutululis, Nicolaos Antonio; Sørensen, Poul Ejnar; Estanqueiro, Ana; Gomez, Emilio; Menemenlis, Nickie; Smith, Charlie; Wang, Ye

    2017-01-01

    With increasing penetrations of wind generation, based on power-electronic converters, power systems are transitioning away from well-understood synchronous generator-based systems, with growing implications for their stability. Issues of concern will vary with system size, wind penetration level, geographical distribution and turbine type, network topology, electricity market structure, unit commitment procedures, and other factors. However, variable-speed wind turbines, both onshore and con...

  11. Clinical Application of Screening for GJB2 Mutations before Cochlear Implantation in a Heterogeneous Population with High Rate of Autosomal Recessive Nonsyndromic Hearing Loss

    Directory of Open Access Journals (Sweden)

    Masoud Motasaddi Zarandy

    2011-01-01

    Full Text Available Clinical application of mutation screening and its effect on the outcome of cochlear implantation is widely debated. We investigated the effect of mutations in GJB2 gene on the outcome of cochlear implantation in a population with a high rate of consanguineous marriage and autosomal recessive nonsyndromic hearing loss. Two hundred and one children with profound prelingual sensorineural hearing loss were included. Forty-six patients had 35delG in GJB2. Speech awareness thresholds (SATs and speech recognition thresholds (SRTs improved following implantation, but there was no difference in performance between patients with GJB2-related deafness versus control (all >0.10. Both groups had produced their first comprehensible words within the same period of time following implantation (2.27 months in GJB2-related deaf versus 2.62 months in controls, =0.22. Although our findings demonstrate the need to uncover unidentified genetic causes of hereditary deafness, they do not support the current policy for genetic screening before cochlear implantation, nor prove a prognostic value.

  12. Analysis of Carbon Policies for Electricity Networks with High Penetration of Green Generation

    Science.gov (United States)

    Feijoo, Felipe A.

    In recent decades, climate change has become one of the most crucial challenges for humanity. Climate change has a direct correlation with global warming, caused mainly by the green house gas emissions (GHG). The Environmental Protection Agency in the U.S. (EPA) attributes carbon dioxide to account for approximately 82% of the GHG emissions. Unfortunately, the energy sector is the main producer of carbon dioxide, with China and the U.S. as the highest emitters. Therefore, there is a strong (positive) correlation between energy production, global warming, and climate change. Stringent carbon emissions reduction targets have been established in order to reduce the impacts of GHG. Achieving these emissions reduction goals will require implementation of policies like as cap-and-trade and carbon taxes, together with transformation of the electricity grid into a smarter system with high green energy penetration. However, the consideration of policies solely in view of carbon emissions reduction may adversely impact other market outcomes such as electricity prices and consumption. In this dissertation, a two-layer mathematical-statistical framework is presented, that serves to develop carbon policies to reduce emissions level while minimizing the negative impacts on other market outcomes. The bottom layer of the two layer model comprises a bi-level optimization problem. The top layer comprises a statistical model and a Pareto analysis. Two related but different problems are studied under this methodology. The first problem looks into the design of cap-and-trade policies for deregulated electricity markets that satisfy the interest of different market constituents. Via the second problem, it is demonstrated how the framework can be used to obtain levels of carbon emissions reduction while minimizing the negative impact on electricity demand and maximizing green penetration from microgrids. In the aforementioned studies, forecasts for electricity prices and production cost

  13. Dynamic voltage stability of a distribution system with high penetration of grid-connected photovoltaic type solar generators

    Directory of Open Access Journals (Sweden)

    Zetty Adibah Kamaruzzaman

    2016-06-01

    Full Text Available This paper presents the impact of grid-connected photovoltaic (PV generator on dynamic voltage stability of a power distribution system by considering solar intermittency, PV penetration level, and contingencies such as line outage and load increase. The IEEE 13 node test feeder is used as a test system, and a solar PV of 0.48 kV/0.5 MVA is integrated into the test system. Test results show that system voltage is stable at high PV penetration levels. Increase in load causes voltage instability, in which voltage drops below its allowable operating limit. Thus, increase in PV penetration level does not improve system voltage stability because the system experiences voltage collapse during line outage.

  14. Changes in the Economic Value of Variable Generation at High Penetration Levels: A Pilot Case Study of California

    Energy Technology Data Exchange (ETDEWEB)

    Mills, Andrew [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Wiser, Ryan [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2012-05-18

    We estimate the long-run economic value of variable renewable generation with increasing penetration using a unique investment and dispatch model that captures long-run investment decisions while also incorporating detailed operational constraints and hourly time resolution over a full year. High time resolution and the incorporation of operational constraints are important for estimating the economic value of variable generation, as is the use of a modeling framework that accommodates new investment decisions. The model is herein applied with a case study that is loosely based on California in 2030. Increasing amounts of wind, photovoltaics (PV), and concentrating solar power (CSP) with and without thermal energy storage (TES) are added one at a time. The marginal economic value of these renewable energy sources is estimated and then decomposed into capacity value, energy value, day-ahead forecast error cost, and ancillary services. The marginal economic value, as defined here, is primarily based on the combination of avoided capital investment cost and avoided variable fuel and operations and maintenance costs from other power plants in the power system. Though the model only captures a subset of the benefits and costs of renewable energy, it nonetheless provides unique insights into how the value of that subset changes with technology and penetration level. Specifically, in this case study implementation of the model, the marginal economic value of all three solar options is found to exceed the value of a flat-block of power (as well as wind energy) by \\$20--30/MWh at low penetration levels, largely due to the high capacity value of solar at low penetration. Because the value of CSP per unit of energy is found to be high with or without thermal energy storage at low penetration, we find little apparent incremental value to thermal storage at low solar penetration in the present case study analysis. The marginal economic value of PV and CSP without thermal

  15. Highly variable penetrance of abnormal phenotypes in embryonic lethal knockout mice

    Science.gov (United States)

    Wilson, Robert; Geyer, Stefan H.; Reissig, Lukas; Rose, Julia; Szumska, Dorota; Hardman, Emily; Prin, Fabrice; McGuire, Christina; Ramirez-Solis, Ramiro; White, Jacqui; Galli, Antonella; Tudor, Catherine; Tuck, Elizabeth; Mazzeo, Cecilia Icoresi; Smith, James C.; Robertson, Elizabeth; Adams, David J.; Mohun, Timothy; Weninger, Wolfgang J.

    2017-01-01

    Background: Identifying genes that are essential for mouse embryonic development and survival through term is a powerful and unbiased way to discover possible genetic determinants of human developmental disorders. Characterising the changes in mouse embryos that result from ablation of lethal genes is a necessary first step towards uncovering their role in normal embryonic development and establishing any correlates amongst human congenital abnormalities. Methods: Here we present results gathered to date in the Deciphering the Mechanisms of Developmental Disorders (DMDD) programme, cataloguing the morphological defects identified from comprehensive imaging of 220 homozygous mutant and 114 wild type embryos from 42 lethal and subviable lines, analysed at E14.5. Results: Virtually all mutant embryos show multiple abnormal phenotypes and amongst the 42 lines these affect most organ systems. Within each mutant line, the phenotypes of individual embryos form distinct but overlapping sets. Subcutaneous edema, malformations of the heart or great vessels, abnormalities in forebrain morphology and the musculature of the eyes are all prevalent phenotypes, as is loss or abnormal size of the hypoglossal nerve. Conclusions: Overall, the most striking finding is that no matter how profound the malformation, each phenotype shows highly variable penetrance within a mutant line. These findings have challenging implications for efforts to identify human disease correlates. PMID:27996060

  16. Transactive Demand Side Management Programs in Smart Grids with High Penetration of EVs

    Directory of Open Access Journals (Sweden)

    Poria Hasanpor Divshali

    2017-10-01

    Full Text Available Due to environmental concerns, economic issues, and emerging new loads, such as electrical vehicles (EVs, the importance of demand side management (DSM programs has increased in recent years. DSM programs using a dynamic real-time pricing (RTP method can help to adaptively control the electricity consumption. However, the existing RTP methods, particularly when they consider the EVs and the power system constraints, have many limitations, such as computational complexity and the need for centralized control. Therefore, a new transactive DSM program is proposed in this paper using an imperfect competition model with high EV penetration levels. In particular, a heuristic two-stage iterative method, considering the influence of decisions made independently by customers to minimize their own costs, is developed to find the market equilibrium quickly in a distributed manner. Simulations in the IEEE 37-bus system with 1141 customers and 670 EVs are performed to demonstrate the effectiveness of the proposed method. The results show that the proposed method can better manage the EVs and elastic appliances than the existing methods in terms of power constraints and cost. Also, the proposed method can solve the optimization problem quick enough to run in real-time.

  17. Demand side resource operation on the Irish power system with high wind power penetration

    International Nuclear Information System (INIS)

    Keane, A.; Tuohy, A.; Meibom, P.; Denny, E.; Flynn, D.; Mullane, A.; O'Malley, M.

    2011-01-01

    The utilisation of demand side resources is set to increase over the coming years with the advent of advanced metering infrastructure, home area networks and the promotion of increased energy efficiency. Demand side resources are proposed as an energy resource that, through aggregation, can form part of the power system plant mix and contribute to the flexible operation of a power system. A model for demand side resources is proposed here that captures its key characteristics for commitment and dispatch calculations. The model is tested on the all island Irish power system, and the operation of the model is simulated over one year in both a stochastic and deterministic mode, to illustrate the impact of wind and load uncertainty. The results illustrate that demand side resources can contribute to the efficient, flexible operation of systems with high penetrations of wind by replacing some of the functions of conventional peaking plant. Demand side resources are also shown to be capable of improving the reliability of the system, with reserve capability identified as a key requirement in this respect. - Highlights: → Demand side resource model presented for use in unit commitment and dispatch calculations. → Benefits of demand side aggregation demonstrated specifically as a peaking unit and provider of reserve. → Potential to displace or defer construction of conventional peaking units.

  18. Bremsstrahlung-induced highly penetrating probes for nondestructive assay and defect analysis

    CERN Document Server

    Selim, F A; Harmon, J F; Kwofie, J; Spaulding, R; Erickson, G; Roney, T

    2002-01-01

    Nondestructive assay and defect analysis probes based on bremsstrahlung-induced processes have been developed to identify elements and probe defects in large volume samples. Bremsstrahlung beams from (electron accelerators) with end-point energies both above and below neutron emission threshold have been used. Below neutron emission threshold these beams (from 6 MeV small pulsed linacs), which exhibit high penetration, create positrons via pair production inside the material and produce X-ray fluorescence (XRF) radiation. Chemical assays of heavy elements in thick samples up to 10 g/cm sup 2 thick are provided by energy dispersive XRF measurements. The pair-produced positrons annihilate within the material, thereby emitting 511 keV gamma radiation. Doppler broadening spectroscopy of the 511 keV radiation can be performed to characterize the material and measure defects in samples of any desired thickness. This technique has successfully measured induced strain due to tensile stress in steel samples of 0.64 cm...

  19. High-velocity penetrating thoracic trauma with suspected cardiac involvement in a combat support hospital

    International Nuclear Information System (INIS)

    Dominguez, F.; Gentlesk, P.J.; Eckart, R.E.; Beekley, A.C.; Huffer, L.L.

    2011-01-01

    The most common cardiac injuries in the United States are blunt trauma from motor vehicle accidents or low-velocity trauma from stabbings. During military conflict, high-velocity injuries, including gunshot wounds (GSW) and fragment injury from improvised explosive devices (IED), are relatively more common. This is a retrospective review of cases with high-velocity penetrating injury and suspected myocardial involvement during a 6-month period in Baghdad, Iraq, at a United States Army hospital during Operation Iraqi Freedom. Eleven cases survived to admission (GSW in 5, IED in 6). The mean age of the all-male cohort was 27 years (range, 3-54 years). Eight of the 11 patients (73%) were victims of polytrauma. The entrance involved the right ventricle (n=3), right atrium (n=2), left ventricle (n=1), or mediastinum and pericardial reflections (n=5). Echocardiography was performed in all 11 patients. In 7 patients, no foreign body was identifiable, and in 2 patients the foreign body was identified within the pericardial fat pad. Three patients were identified as having a suspected ventricular septal defect, ranging in size from 2 to 8 mm. The most common electrocardiographic abnormality was atrioventricular block and right bundle branch block. In 4 patients, the management of the chest injury was nonsurgical, and in 1 patient the treatment was a chest tube only. Four of the patients underwent median sternotomy, 1 underwent emergent lateral thoracotomy, and 1 underwent an infradiaphragmatic approach. This case series is too small to draw definitive conclusions; however, a multidisciplinary approach to high-velocity injuries with potential for cardiac involvement augments preoperative assessment for myocardial injury and may allow selective nonoperative management. (author)

  20. Exome Sequencing and Directed Clinical Phenotyping Diagnose Cholesterol Ester Storage Disease Presenting as Autosomal Recessive Hypercholesterolemia

    NARCIS (Netherlands)

    Stitziel, Nathan O.; Fouchier, Sigrid W.; Sjouke, Barbara; Peloso, Gina M.; Moscoso, Alessa M.; Auer, Paul L.; Goel, Anuj; Gigante, Bruna; Barnes, Timothy A.; Melander, Olle; Orho-Melander, Marju; Duga, Stefano; Sivapalaratnam, Suthesh; Nikpay, Majid; Martinelli, Nicola; Girelli, Domenico; Jackson, Rebecca D.; Kooperberg, Charles; Lange, Leslie A.; Ardissino, Diego; McPherson, Ruth; Farrall, Martin; Watkins, Hugh; Reilly, Muredach P.; Rader, Daniel J.; de Faire, Ulf; Schunkert, Heribert; Erdmann, Jeanette; Samani, Nilesh J.; Charnas, Lawrence; Altshuler, David; Gabriel, Stacey; Kastelein, John J. P.; Defesche, Joep C.; Nederveen, Aart J.; Kathiresan, Sekar; Hovingh, G. Kees

    2013-01-01

    Objective Autosomal recessive hypercholesterolemia is a rare inherited disorder, characterized by extremely high total and low-density lipoprotein cholesterol levels, that has been previously linked to mutations in LDLRAP1. We identified a family with autosomal recessive hypercholesterolemia not

  1. Groundwater penetrating radar and high resolution seismic for locating shallow faults in unconsolidated sediments

    International Nuclear Information System (INIS)

    Wyatt, D.E.

    1993-01-01

    Faults in shallow, unconsolidated sediments, particularly in coastal plain settings, are very difficult to discern during subsurface exploration yet have critical impact to groundwater flow, contaminant transport and geotechnical evaluations. This paper presents a case study using cross-over geophysical technologies in an area where shallow faulting is probable and known contamination exists. A comparison is made between Wenner and dipole-dipole resistivity data, ground penetrating radar, and high resolution seismic data. Data from these methods were verified with a cone penetrometer investigation for subsurface lithology and compared to existing monitoring well data. Interpretations from these techniques are compared with actual and theoretical shallow faulting found in the literature. The results of this study suggests that (1) the CPT study, combined with the monitoring well data may suggest that discontinuities in correlatable zones may indicate that faulting is present (2) the addition of the Wenner and dipole-dipole data may further suggest that offset zones exist in the shallow subsurface but not allow specific fault planes or fault stranding to be mapped (3) the high resolution seismic data will image faults to within a few feet of the surface but does not have the resolution to identify the faulting on the scale of our models, however it will suggest locations for upward continuation of faulted zones (4) offset 100 MHz and 200 MHz CMP GPR will image zones and features that may be fault planes and strands similar to our models (5) 300 MHz GPR will image higher resolution features that may suggest the presence of deeper faults and strands, and (6) the combination of all of the tools in this study, particularly the GPR and seismic may allow for the mapping of small scale, shallow faulting in unconsolidated sediments

  2. The population genetics of Quechuas, the largest native South American group: autosomal sequences, SNPs, and microsatellites evidence high level of diversity.

    Science.gov (United States)

    Scliar, Marilia O; Soares-Souza, Giordano B; Chevitarese, Juliana; Lemos, Livia; Magalhães, Wagner C S; Fagundes, Nelson J; Bonatto, Sandro L; Yeager, Meredith; Chanock, Stephen J; Tarazona-Santos, Eduardo

    2012-03-01

    Elucidating the pattern of genetic diversity for non-European populations is necessary to make the benefits of human genetics research available to individuals from these groups. In the era of large human genomic initiatives, Native American populations have been neglected, in particular, the Quechua, the largest South Amerindian group settled along the Andes. We characterized the genetic diversity of a Quechua population in a global setting, using autosomal noncoding sequences (nine unlinked loci for a total of 16 kb), 351 unlinked SNPs and 678 microsatellites and tested predictions of the model of the evolution of Native Americans proposed by (Tarazona-Santos et al.: Am J Hum Genet 68 (2001) 1485-1496). European admixture is Quechua or Melanesian populations, which is concordant with the African origin of modern humans and the fact that South America was the last part of the world to be peopled. The diversity in the Quechua population is comparable with that of Eurasian populations, and the allele frequency spectrum based on resequencing data does not reflect a reduction in the proportion of rare alleles. Thus, the Quechua population is a large reservoir of common and rare genetic variants of South Amerindians. These results are consistent with and complement our evolutionary model of South Amerindians (Tarazona-Santos et al.: Am J Hum Genet 68 (2001) 1485-1496), proposed based on Y-chromosome data, which predicts high genomic diversity due to the high level of gene flow between Andean populations and their long-term effective population size. Copyright © 2012 Wiley Periodicals, Inc.

  3. Influence of Antenna Characteristics on Elevation Dependence of Building Penetration Loss for High Elevation Links

    Directory of Open Access Journals (Sweden)

    M. Kvicera

    2012-12-01

    Full Text Available Building penetration loss models presented in our previous paper [1] were valid for various scenarios, propagation conditions, frequency bands and hemispherical receiving antenna pointing towards zenith. These models had a significantly rising trend of penetration loss with increasing elevation angle of the link in common. In this paper we show that when working with non-isotropic terminal antennas, this trend relates primarily to the elevation trend of the corresponding reference level dependent on the receiving antenna radiation pattern. This is demonstrated by the results of single-input multiple-output (SIMO measurement trials performed at L-band in an office building and a brick building in the city of Prague. Further, based on the detailed analysis, a method to modify the elevation trend of a particular penetration loss model for different receiving antenna radiation patterns is derived and experimentally validated.

  4. Transport mean free path related to trajectory patterns: Comparison of nonrelativistic and highly relativistic electron penetration through matter

    International Nuclear Information System (INIS)

    Liljequist, D.; Ismail, M.

    1987-01-01

    This analysis is based on the similarity between multiple scattering and slowing down (random walk) processes described by the same transport mean-free-path function λ/sub tr/(s) (s = path length). We discuss the connection between λ/sub tr/(s) and the characteristic appearance and scale of the trajectory pattern. Straggling is considered by means by stochastically discontinuous λ/sub tr/(s) functions. In the application to electron penetration, we show that while nonrelativistic electron penetration is modeled by λ/sub tr/ = (r-s)/α, where r is the range and α is a material-dependent dimensionless constant, highly relativistic electron penetration is modeled by λ/sub tr/proportionalexp(-s/Λ), where Λ is a length characteristic for the penetrated material. The respective trajectory patterns are distinctly different. The effect of straggling on the trajectory pattern in the highly relativistic case is demonstrated by means of a simple model of the stochastic λ/sub tr/(s) behavior

  5. Analysis Methodology for Balancing Authority Cooperation in High Penetration of Variable Generation

    Energy Technology Data Exchange (ETDEWEB)

    Makarov, Yuri V.; Etingov, Pavel V.; Zhou, Ning; Ma, Jian; Samaan, Nader A.; Diao, Ruisheng; Malhara, Sunita V.; Guttromson, Ross T.; Du, Pengwei; Sastry, Chellury

    2010-02-01

    With the rapidly growing penetration level of wind and solar generation, the challenges of managing variability and the uncertainty of intermittent renewable generation become more and more significant. The problem of power variability and uncertainty gets exacerbated when each balancing authority (BA) works locally and separately to balance its own subsystem. The virtual BA concept means various forms of collaboration between individual BAs must manage power variability and uncertainty. The virtual BA will have a wide area control capability in managing its operational balancing requirements in different time frames. This coordination results in the improvement of efficiency and reliability of power system operation while facilitating the high level integration of green, intermittent energy resources. Several strategies for virtual BA implementation, such as ACE diversity interchange (ADI), wind only BA, BA consolidation, dynamic scheduling, regulation and load following sharing, extreme event impact study are discussed in this report. The objective of such strategies is to allow individual BAs within a large power grid to help each other deal with power variability. Innovative methods have been developed to simulate the balancing operation of BAs. These methods evaluate the BA operation through a number of metrics — such as capacity, ramp rate, ramp duration, energy and cycling requirements — to evaluate the performances of different virtual BA strategies. The report builds a systematic framework for evaluating BA consolidation and coordination. Results for case studies show that significant economic and reliability benefits can be gained. The merits and limitation of each virtual BA strategy are investigated. The report provides guidelines for the power industry to evaluate the coordination or consolidation method. The application of the developed strategies in cooperation with several regional BAs is in progress for several off-spring projects.

  6. Application of a high-throughput genotyping method for loci exclusion in non-consanguineous Australian pedigrees with autosomal recessive retinitis pigmentosa.

    Science.gov (United States)

    Paterson, Rachel L; De Roach, John N; McLaren, Terri L; Hewitt, Alex W; Hoffmann, Ling; Lamey, Tina M

    2012-01-01

    Retinitis pigmentosa (RP) is the most common form of inherited blindness, caused by progressive degeneration of photoreceptor cells in the retina, and affects approximately 1 in 3,000 people. Over the past decade, significant progress has been made in gene therapy for RP and related diseases, making genetic characterization increasingly important. Recently, high-throughput technologies have provided an option for reasonably fast, cost-effective genetic characterization of autosomal recessive RP (arRP). The current study used a single nucleotide polymorphism (SNP) genotyping method to exclude up to 28 possible disease-causing genes in 31 non-consanguineous Australian families affected by arRP. DNA samples were collected from 59 individuals affected with arRP and 74 unaffected family members from 31 Australian families. Five to six SNPs were genotyped for 28 genes known to cause arRP or the related disease Leber congenital amaurosis (LCA). Cosegregation analyses were used to exclude possible causative genes from each of the 31 families. Bidirectional sequencing was used to identify disease-causing mutations in prioritized genes that were not excluded with cosegregation analyses. Two families were excluded from analysis due to identification of false paternity. An average of 28.9% of genes were excluded per family when only one affected individual was available, in contrast to an average of 71.4% or 89.8% of genes when either two, or three or more affected individuals were analyzed, respectively. A statistically significant relationship between the proportion of genes excluded and the number of affected individuals analyzed was identified using a multivariate regression model (pA) and USH2A in two families (c.2276 G>T). This study has shown that SNP genotyping cosegregation analysis can be successfully used to refine and expedite the genetic characterization of arRP in a non-consanguineous population; however, this method is effective only when DNA samples are

  7. Self-association of a highly charged arginine-rich cell-penetrating peptide

    Czech Academy of Sciences Publication Activity Database

    Tesei, G.; Vazdar, M.; Jensen, M. R.; Cragnell, C.; Mason, Philip E.; Heyda, J.; Skepö, M.; Jungwirth, Pavel; Lund, M.

    2017-01-01

    Roč. 114, č. 43 (2017), s. 11428-11433 ISSN 0027-8424 R&D Projects: GA ČR(CZ) GA16-01074S Institutional support: RVO:61388963 Keywords : cell-penetrating peptide * self-association * MD simulations * SAXS * NMR Subject RIV: CF - Physical ; Theoretical Chemistry OBOR OECD: Physical chemistry Impact factor: 9.661, year: 2016

  8. Size-Resolved Penetration Through High-Efficiency Filter Media Typically Used for Aerosol Sampling

    Czech Academy of Sciences Publication Activity Database

    Zíková, Naděžda; Ondráček, Jakub; Ždímal, Vladimír

    2015-01-01

    Roč. 49, č. 4 (2015), s. 239-249 ISSN 0278-6826 R&D Projects: GA ČR(CZ) GBP503/12/G147 Institutional support: RVO:67985858 Keywords : filters * size-resolved penetration * atmospheric aerosol sampling Subject RIV: CF - Physical ; Theoretical Chemistry Impact factor: 1.953, year: 2015

  9. Study on Penetration Characteristics of Tungsten Cylindrical Penetrator

    Energy Technology Data Exchange (ETDEWEB)

    Jo, Jong Hyun; Lee, Young Shin; Kim, Jae Hoon [Chungnam Nat' l Univ., Daejeon (Korea, Republic of); Bae, Yong Woon [Agency for Defense Development, Daejeon (Korea, Republic of)

    2013-09-15

    The design of missile require extremely small warheads that must be highly efficient and lethal. The penetration characteristics of each penetrator and the total number of penetrators on the warhead are obvious key factors that influence warhead lethality. The design of the penetrator shape and size are directly related to the space and weight of the warhead. The design of the penetrator L/D was directly related to the space and weight of the warhead. L and D are the length and the diameter of the projectile, respectively. The AUTODYN-3a code was used to study the effect of penetrator penetration. The objective of numerical analysis was to determine the penetration characteristics of penetrator produced by hypervelocity impacts under different initial conditions such as initial velocity, obliquity angle and L/D of penetrator. The residual velocity and residual mass were decreased with increasing initial impact velocity under L/D{<=}4.

  10. Large-scale, high-definition Ground Penetrating Radar prospection in archaeology

    Science.gov (United States)

    Trinks, I.; Kucera, M.; Hinterleitner, A.; Löcker, K.; Nau, E.; Neubauer, W.; Zitz, T.

    2012-04-01

    The future demands on professional archaeological prospection will be its ability to cover large areas in a time and cost efficient manner with very high spatial resolution and accuracy. The objective of the 2010 in Vienna established Ludwig Boltzmann Institute for Archaeological Prospection and Virtual Archaeology (LBI ArchPro) in collaboration with its eight European partner organisations is the advancement of state-of-the-art archaeological sciences. The application and specific further development of remote sensing, geophysical prospection and virtual reality applications, as well as of novel integrated interpretation approaches dedicated to non-invasive spatial archaeology combining near-surface prospection methods with advanced computer science is crucial for modern archaeology. Within the institute's research programme different areas for distinct case studies in Austria, Germany, Norway, Sweden and the UK have been selected as basis for the development and testing of new concepts for efficient and universally applicable tools for spatial, non-invasive archaeology. In terms of geophysical prospection the investigation of entire archaeological landscapes for the exploration and protection of Europe's buried cultural heritage requires new measurement devices, which are fast, accurate and precise. Therefore the further development of motorized, multichannel survey systems and advanced navigation solutions is required. The use of motorized measurement devices for archaeological prospection implicates several technological and methodological challenges. Latest multichannel Ground Penetrating Radar (GPR) arrays mounted in front off, or towed behind motorized survey vehicles permit large-scale GPR prospection surveys with unprecedented spatial resolution. In particular the motorized 16 channel 400 MHz MALÅ Imaging Radar Array (MIRA) used by the LBI ArchPro in combination with latest automatic data positioning and navigation solutions permits the reliable high

  11. Technical impacts of high penetration levels of wind power on power system stability

    DEFF Research Database (Denmark)

    Flynn, Damian; Rather, Z.; Ardal, Atle

    2017-01-01

    With increasing penetrations of wind generation, based on power-electronic converters, power systems are transitioning away from well-understood synchronous generator-based systems, with growing implications for their stability. Issues of concern will vary with system size, wind penetration level......, geographical distribution and turbine type, network topology, electricity market structure, unit commitment procedures, and other factors. However, variable-speed wind turbines, both onshore and connected offshore through DC grids, offer many control opportunities to either replace or enhance existing...... capabilities. Achieving a complete understanding of future stability issues, and ensuring the effectiveness of new measures and policies, is an iterative procedure involving portfolio development and flexibility assessment, generation cost simulations, load flow, and security analysis, in addition...

  12. Capacity Payments in Restructured Markets under Low and High Penetration Levels of Renewable Energy

    Energy Technology Data Exchange (ETDEWEB)

    Thomas Jenkin, Philipp Beiter, and Robert Margolis

    2016-02-01

    Growing levels of variable renewable energy resources arguably create new challenges for capacity market designs, because variable renewable energy suppresses wholesale energy prices while providing relatively little capacity. This effect becomes more pronounced the higher the variable renewable energy penetration in a market. The purpose of this report is threefold. First, we provide a brief outline of the purpose and design of various capacity markets using administratively determined capacity demand curves. Second, we discuss some of the main challenges raised in existing literature and a set of interviews that we conducted with market participants, regulators, and observers. Third, we consider some of the challenges to capacity markets that arise with higher variable renewable energy penetration.

  13. Projectile Nose Mass Abrasion of High-Speed Penetration into Concrete

    Directory of Open Access Journals (Sweden)

    Haijun Wu

    2012-01-01

    Full Text Available Based on the dynamic spherical cavity expansion theory of concrete and the analysis of experimental data, a mass abrasion model of projectile considering the hardness of aggregates, the relative strength of target and projectile, and the initial impact velocity is constructed in this paper. Furthermore, the effect of mass abrasion on the penetration depth of projectile and the influence of hardness of aggregates and strength of projectile on penetration depth and mass loss are also analyzed. The results show that, for the ogive-nose projectile with the CRH of 3 and aspect ratio of 7 penetrating the concrete of 35 MPa, the “rigid-body penetration” model is available when the initial impact velocity is lower than 800 m/s. However, when the initial impact velocity is higher than 800 m/s, the “deforming/eroding body penetration” model should be adopted. Through theoretical analysis and numerical calculation, the results indicate that the initial impact velocity is the most important factor of mass abrasion. The hardness of aggregates and the strength of projectile are also significant factors. But relatively speaking, the sensitivity of strength of projectile to mass abrasion is higher, which indicates that the effect of projectile material on mass abrasion is more dramatic than the hardness of aggregates.

  14. Quantitative assessment of liquid Ga penetration into an aluminium alloy by high-resolution X-ray tomography

    International Nuclear Information System (INIS)

    Ohgaki, T.; Toda, H.; Sinclair, I.; Buffiere, J.-Y.; Ludwig, W.; Kobayashi, T.; Niinomi, M.; Akahori, T.

    2005-01-01

    We have evaluated the liquid Ga penetration into an aluminium alloy by high-resolution X-ray tomography. The 3D visualization of a crack together with its surrounding grain structure was performed with the help of the Ga penetration technique. It is found that the advance directions of the crack-tip were strongly influenced by the grain microstructure and the branching of the crack is affected by grain distribution. In this study, the liquid Ga not only acts as a contrast agent for grain boundaries, but also expands the volume of the Al alloy due to Ga diffusion and associated processes. The 3D strain between the grains has been determined by microstructural gauging technique, which uses micropores as marker points. The 3D expansion of the sample volume, the volume reduction of micropores and the brittle fracture were evidently observed

  15. [Waardenburg syndrome. A heterogenic disorder with variable penetrance].

    Science.gov (United States)

    Apaydin, F; Bereketoglu, M; Turan, O; Hribar, K; Maassen, M M; Günhan, O; Zenner, H-P; Pfister, M

    2004-06-01

    Waardenburg syndrome (WS) is an autosomal dominant disorder characterised by pigmentary anomalies of the skin, hairs, eyes and various defects of other neural crest derived tissues. It accounts for over 2% of congenital hearing impairment. At least four types are recognized on the basis of clinical and genetic criteria. Based on a screening of congenitally hearing impaired children, 12 families with WS type II were detected. Of special interest was the phenotype of these families, in particular the reduced penetrance of hearing impairment within the families. In all cases a high variability of the disease phenotype was detected and the penetrance of the clinical traits varied accordingly. Therefore, it is not possible to predict the clinical phenotype even in a single family. Based on these studies, we plan to identify the pathogenetic cause of the disease in order to perform a detailed genotype/phenotype analysis.

  16. The impact of high penetration of wind energy on the vulnerability of power systems

    Energy Technology Data Exchange (ETDEWEB)

    EL-Arroudi, K.; Joos, G.; McGillis, D. [McGill Univ., Montreal, PQ (Canada). Dept. of Electrical and Computer Engineering

    2006-07-01

    This paper examined the impact of higher penetrations of wind energy installations on the vulnerability of power systems. Vulnerability was defined in terms of migration of system states based on the contingencies that might occur. It was noted that both the penetration levels and physical locations of wind energy installations in power systems have a strong influence on system vulnerability. A methodology was proposed to analyze the impacts of wind energy on power system vulnerability through the construction of a decision-tree classification model at the point of common coupling (PCC) bus. The aim of the model was to determine planning criteria for wind power interconnection and to ensure that design concepts are adequate and secure. The model was built by simulating a pre-specified range of system contingencies to generate patterns at the PCC bus. Actual measurements were then compared against known patterns, from which the stress levels of disturbances were estimated. Stress levels were defined in terms of the performance level measures delineated by National Electricity Reliability Council (NERC) planning standards. The methodology is a non-parametric learning technique able to produce classifiers about given problems in order to deduce information from new, unobserved cases. A case study consisting of a 4-machine system with a total generation of 2295 MW was presented where wind-based generation accounted for 450 MW. The decision-tree classifier was constructed by simulating 120 events generated by combinations of contingencies; seasonal wind patterns and different wind production levels per season. Results showed that with a knowledge of the total penetration level and location of wind power installations, it is possible to estimate the effect of wind energy on the vulnerability of a power system. 12 refs., 6 figs.

  17. Customized photorefractive keratectomy to correct high ametropia after penetrating keratoplasty: A pilot study

    OpenAIRE

    De Rosa, Giuseppe; Boccia, Rosa; Santamaria, Carmine; Fabbozzi, Lorenzo; De Rosa, Luigi; Lanza, Michele

    2014-01-01

    Purpose: To evaluate preliminarily the safety and efficacy of customized photorefractive keratectomy (PRK) to correct ametropia and irregular astigmatism after penetrating keratoplasty (PK). Methods: This pilot study included five eyes of five patients with a mean spherical equivalent of −5.1 ± 1.46 D (range from −2.75 to −6.50 D). In all cases, ametropia and irregular astigmatism was corrected with topography-guided customized PRK. Ocular examinations with topographic analysis were perfor...

  18. Index-Based Assessment of Voltage Rise and Reverse Power Flow Phenomena in a Distribution Feeder Under High PV Penetration

    DEFF Research Database (Denmark)

    Hasheminamin, Maryam; Agelidis, Vassilios G.; Salehi, Vahid

    2015-01-01

    -based methodology for assessing the impact of high solar PV generation, considering the reverse power flow and voltage rise phenomena. Indices are defined that link these two phenomena and their impact on the voltage profile across the feeder. This assessment relies on detailed modeling of the network and the solar......The proliferation of photovoltaic (PV) generation in low- and medium-voltage distribution networks is expected to continue. Qualified studies can quantify adverse impacts of high PV penetration on distribution networks and assist utilities in decision making. This paper proposes an index...

  19. Participation of Flexible Loads in Load Frequency Control to Support High Wind Penetration

    DEFF Research Database (Denmark)

    Uslu, Umur; Zhang, Boyang; Pillai, Jayakrishnan Radhakrishna

    2016-01-01

    The increasing amount of fluctuating wind power penetration in power systems presents many challenges to its operation and control. The new wind power plants are replacing many of the conventional large power plants that ensure power balancing and ancillary services for stable and reliable...... operation of the grid. Therefore, new solutions for power balancing reserves have to be explored and utilized by the grid utilities. To meet these challenges, large sizable loads like alkaline electrolysers, heat pumps and electric vehicles which are gaining popularity can provide system support to the grid...... through their inherent flexibility and energy storage characteristics. This paper investigates the possibilities and potential of such flexible loads to participate in power system frequency regulation in a wind dominated power system. The results show that these consumption units provide better...

  20. Eddy current testing with high penetration; WS-Pruefungen mit grosser Eindringtiefe

    Energy Technology Data Exchange (ETDEWEB)

    Becker, R.; Kroening, M. [Fraunhofer Inst. fuer Zerstoerungsfreie Pruefverfahren, Saarbruecken (Germany)

    1999-08-01

    The low-frequency eddy current testing method is used when penetration into very deep layers is required. The achievable penetration depth is determined among other parameters by the lowest testing frequency that can be realised together with the eddy current sensor. When using inductive sensors, the measuring effect declines proportional to the lowering frequency (induction effect). Further reduction of testing frequency requires other types of sensors, as e.g. the GMR (Giant Magnetic Resistance), which achieves a constant measuring sensitivity down to the steady field. The multi-frequency eddy current testing method MFEC 3 of IZFP described here can be operated using three different scanning frequencies at a time. Two variants of eddy current probes are used in this case. Both have an inductive winding at their emitters, of the type of a measuring probe. The receiver end is either also an inductive winding, or a magnetic field-responsive resistance (GMR). (orig./CB) [Deutsch] Das Niederfrequenz(NF)-Wirbelstrom(WS)-Verfahren wird eingesetzt, um eine grosse Eindringtiefe zu erzielen. Die erreichbare Tiefenreichweite wird u.a. durch die niedrigste Prueffrequenz bestimmt, die zusammen mit dem Wirbelstrom-Sensor realisiert werden kann. Bei Einsatz von induktiven Sensoren geht mit abnehmender Prueffrequenz der Messeffekt proportional zurueck (Induktionswirkung). Eine weitere Absenkung der Prueffrequenzen macht den Einsatz von andersartigen Sensoren notwendig, z.B. den GMR (Giant Magnetic Resistance), der eine gleichmaessige Messempfindlichkeit bis zum Gleichfeld besitzt. Das eingesetzte Mehrfrequenz-Wirbelstrom-Pruefverfahren MFEC 3 des IZFP arbeitet mit drei gleichzeitig eingespeisten Prueffrequenzen. Dabei werden zwei Varianten von WS-Sensoren eingesetzt. Beide besitzen auf der Senderseite eine induktive Wicklung in der Art einer Tastsonde. Die Empfaengerseite ist entweder ebenfalls eine induktive Wicklung oder ein magnetfeldempfindlicher Widerstand (GMR). (orig./DGE)

  1. Stability analysis of transmission system with high penetration of distributed generation

    Energy Technology Data Exchange (ETDEWEB)

    Reza, M.

    2006-12-21

    Nowadays, interest in generating electricity using decentralized generators of relatively small scale ('distributed generation', DG) is increasing. This work deals with the impact of implementing DG on the transmission system transient stability, with the emphasis on a potential transition from a 'vertical power system' to a 'horizontal power system. A problem in power systems is maintaining synchronous operation of all (centralized) synchronous machines. This stability problem associated is called rotor angle stability. In this work, the impact of the DG implementation on this is investigated. The impact of DG levels on the system transient stability when the increasing DG level is followed by a reduction of centralized generators in service resulting in a 'vertical to horizontal' transformation of the power system is also investigated. Furthermore, a stochastic analysis is used to study the transient stability of the power systems. The results show that including the stochastic behavior of DG leads to a more complete and detailed view of the system performance. Finally, the situation when the power system is pushed towards a scenario, where DG penetration reaches a level that covers the total load of the original power system (100% DG level) is investigated. The research performed in this work indicates that from the transmission system stability point of view, if higher DG penetration levels are coming up, sufficient inertia and voltage support must be installed. Furthermore, one should be aware of the fact that the system behaves stochastically, especially with DG. To a certain extent regional balancing of power can be performed by local voltage control.

  2. A Scenario-Based Approach for Energy Storage Capacity Determination in LV Grids with High PV Penetration

    DEFF Research Database (Denmark)

    Hashemi Toghroljerdi, Seyedmostafa; Østergaard, Jacob; Yang, Guangya

    2014-01-01

    In this paper a new method is proposed to determine the minimum energy storage required to be installed at different locations of a low voltage (LV) grid in order to prevent the overvoltage due to high residential photovoltaic (PV) penetration. The method is based on the voltage sensitivity...... with different occurrence probabilities without involving the time-series studies problems. The proposed method is capable of modeling output power of PV panels with different orientations as well as different electric vehicle (EV) charging patterns....

  3. Evidence of weak pair coupling in the penetration depth of bi-based high-Tc superconductors

    International Nuclear Information System (INIS)

    Thompson, J.R.; Sun, Yang Ren; Ossandon, J.G.; Christen, D.K.; Chakoumakos, B.C.; Sales, B.C.; Kerchner, H.R.; Sonder, E.

    1990-01-01

    The magnetic penetration depth λ(T) has been investigated in Bi(Pb)SrCaCuO high-T c compounds having 2- and 3-layers of copper-oxygen per unit cell. Studies of the magnetization in the vortex state were employed and the results were compared with weak and strong coupling calculations. The temperature dependence of λ is described well by BCS theory in the clean limit, giving evidence for weak pair coupling in this family of materials. For the short component of the λ tensor, we obtain values of 292 and 220 nm (T = 0) for Bi-2212 and (BiPb)-2223, respectively

  4. Efficiency of low versus high airline pressure in stunning cattle with a pneumatically powered penetrating captive bolt gun.

    Science.gov (United States)

    Oliveira, Steffan Edward Octávio; Gregory, Neville George; Dalla Costa, Filipe Antonio; Gibson, Troy John; Paranhos da Costa, Mateus José Rodrigues

    2017-08-01

    The efficiency of stunning cattle was assessed in 443 animals (304 pure Zebu and 139 crossbred cattle), being mainly mature bulls and cows. Cattle were stunned using a Jarvis pneumatically powered penetrating captive bolt gun operating with low (160-175psi, N=82) and high (190psi, N=363) airline pressure, which was within the manufactures specifications. Signs of brain function and the position of the shots on the heads were recorded after stunning. Velocity of the captive bolt and its physical parameters were calculated. Cattle shot with low pressures showed more rhythmic respiration (27 vs. 8%, P<0.001), less tongue protrusion (4 vs. 12%, P=0.03) and less masseter relaxation (22 vs. 48%, P<0.001). There was an increased frequency of shots in the ideal position when cattle were shot with the low compared to high airline pressures (15.3 vs. 3.1%). Bolt velocity and its physical parameters were significantly (P<0.01) higher when using high pressure. Airline pressures below 190psi are inappropriate when shooting adult Zebu beef cattle with pneumatically powered penetrating captive bolt guns. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Autosomal Dominant Polycystic Kidney Disease

    Science.gov (United States)

    ... NIH Director Organization Budget History NIH Almanac Public Involvement Outreach & Education Visitor Information RePORT NIH Fact Sheets Home > Autosomal ... other than the observation that 50 percent of children born to an affected parent would develop the disease. Diagnosis of well-established ...

  6. Winds of change: How high wind penetrations will affect investment incentives in the GB electricity sector

    International Nuclear Information System (INIS)

    Steggals, Will; Gross, Robert; Heptonstall, Philip

    2011-01-01

    Wind power is widely expected to expand rapidly in Britain over the next decade. Large amounts of variable wind power on the system will increase market risks, with prices more volatile and load factors for conventional thermal plant lower and more uncertain. This extra market risk may discourage investment in generation capacity. Financial viability for thermal plant will be increasingly dependent on price spikes during periods of low wind. Increased price risk will also make investment in other forms of low-carbon generation (e.g. nuclear power) more challenging. A number of policies can reduce the extent to which generators are exposed to market risks and encourage investment. However, market risks play a fundamental role in shaping efficient investment and dispatch patterns in a liberalised market. Therefore, measures to improve price signals and market functioning (such as a stronger carbon price and developing more responsive demand) are desirable. However, the scale of the investment challenge and increased risk mean targeted measures to reduce (although not eliminate) risk exposure, such as capacity mechanisms and fixed price schemes, may have increasing merit. The challenge for policy is to strike the right balance between market and planned approaches. - Research highlights: → Analyses how increases penetrations of wind power effect electricity market functioning. → Assesses the impacts of this on investment incentives for different technologies. → Discusses implications for policy and market design.

  7. Penetration of High Intensity Radiated Fields (HIRF) Into General Aviation Aircraft

    Science.gov (United States)

    Balanis, Constantine A.; Birtcher, Craig R.; Georgakopoulos, Stavros V.; Panaretos, Anastasios H.

    2004-01-01

    The ability to design and achieve electromagnetic compatibility is becoming more challenging with the rapid development of new electronic products and technologies. The importance of electromagnetic interference (EMI) and electromagnetic compatibility (EMC) issues stems from the fact that the ambient electromagnetic environment has become very hostile; that is, it increases both in density and intensity, while the current trend in technology suggests the number of electronic devices increases in homes, businesses, factories, and transportation vehicles. Furthermore, the operating frequency of products coming into the market continuously increases. While cell phone technology has exceeded 1 GHz and Bluetooth operates at 2.4 GHz, products involving satellite communications operate near 10 GHz and automobile radar systems involve frequencies above 40 GHz. The concern about higher frequencies is that they correspond to smaller wavelengths, therefore electromagnetic waves are able to penetrate equipment enclosure through apertures or even small cracks more easily. In addition, electronic circuits have become small in size, and they are usually placed on motherboards or housed in boxes in very close proximity. Cosite interference and coupling in all electrical and electronic circuit assemblies are two essential issues that have to be examined in every design.

  8. Incorporating Charging/Discharging Strategy of Electric Vehicles into Security-Constrained Optimal Power Flow to Support High Renewable Penetration

    Directory of Open Access Journals (Sweden)

    Kyungsung An

    2017-05-01

    Full Text Available This research aims to improve the operational efficiency and security of electric power systems at high renewable penetration by exploiting the envisioned controllability or flexibility of electric vehicles (EVs; EVs interact with the grid through grid-to-vehicle (G2V and vehicle-to-grid (V2G services to ensure reliable and cost-effective grid operation. This research provides a computational framework for this decision-making process. Charging and discharging strategies of EV aggregators are incorporated into a security-constrained optimal power flow (SCOPF problem such that overall energy cost is minimized and operation within acceptable reliability criteria is ensured. Particularly, this SCOPF problem has been formulated for Jeju Island in South Korea, in order to lower carbon emissions toward a zero-carbon island by, for example, integrating large-scale renewable energy and EVs. On top of conventional constraints on the generators and line flows, a unique constraint on the system inertia constant, interpreted as the minimum synchronous generation, is considered to ensure grid security at high renewable penetration. The available energy constraint of the participating EV associated with the state-of-charge (SOC of the battery and market price-responsive behavior of the EV aggregators are also explored. Case studies for the Jeju electric power system in 2030 under various operational scenarios demonstrate the effectiveness of the proposed method and improved operational flexibility via controllable EVs.

  9. Decitabine improves progression-free survival in older high-risk MDS patients with multiple autosomal monosomies: results of a subgroup analysis of the randomized phase III study 06011 of the EORTC Leukemia Cooperative Group and German MDS Study Group.

    Science.gov (United States)

    Lübbert, Michael; Suciu, Stefan; Hagemeijer, Anne; Rüter, Björn; Platzbecker, Uwe; Giagounidis, Aristoteles; Selleslag, Dominik; Labar, Boris; Germing, Ulrich; Salih, Helmut R; Muus, Petra; Pflüger, Karl-Heinz; Schaefer, Hans-Eckart; Bogatyreva, Lioudmila; Aul, Carlo; de Witte, Theo; Ganser, Arnold; Becker, Heiko; Huls, Gerwin; van der Helm, Lieke; Vellenga, Edo; Baron, Frédéric; Marie, Jean-Pierre; Wijermans, Pierre W

    2016-01-01

    In a study of elderly AML patients treated with the hypomethylating agent decitabine (DAC), we noted a surprisingly favorable outcome in the (usually very unfavorable) subgroup with two or more autosomal monosomies (MK2+) within a complex karyotype (Lübbert et al., Haematologica 97:393-401, 2012). We now analyzed 206 myelodysplastic syndrome (MDS) patients (88 % of 233 patients randomized in the EORTC/GMDSSG phase III trial 06011, 61 of them with RAEBt, i.e. AML by WHO) with cytogenetics informative for MK status.. Endpoints are the following: complete/partial (CR/PR) and overall response rate (ORR) and progression-free (PFS) and overall survival (OS). Cytogenetic subgroups are the following: 63 cytogenetically normal (CN) patients, 143 with cytogenetic abnormalities, 73 of them MK-negative (MK-), and 70 MK-positive (MK+). These MK+ patients could be divided into 17 with a single autosomal monosomy (MK1) and 53 with at least two monosomies (MK2+). ORR with DAC in CN patients: 36.1 %, in MK- patients: 16.7 %, in MK+ patients: 43.6 % (MK1: 44.4 %, MK2+ 43.3 %). PFS was prolonged by DAC compared to best supportive care (BSC) in the CN (hazard ratio (HR) 0.55, 99 % confidence interval (CI), 0.26; 1.15, p = 0.03) and MK2+ (HR 0.50; 99 % CI, 0.23; 1.06, p = 0.016) but not in the MK-, MK+, and MK1 subgroups. OS was not improved by DAC in any subgroup. In conclusion, we demonstrate for the first time in a randomized phase III trial that high-risk MDS patients with complex karyotypes harboring two or more autosomal monosomies attain encouraging responses and have improved PFS with DAC treatment compared to BSC.

  10. Potential Effect and Analysis of High Residential Solar Photovoltaic (PV Systems Penetration to an Electric Distribution Utility (DU

    Directory of Open Access Journals (Sweden)

    Jeffrey Tamba Dellosa

    2016-11-01

    Full Text Available The Renewable Energy Act of 2008 in the Philippines provided an impetus for residential owners to explore solar PV installations at their own rooftops through the Net-Metering policy. The Net-Metering implementation through the law however presented some concerns with inexperienced electric DU on the potential effect of high residential solar PV system installations. It was not known how a high degree of solar integration to the grid can possibly affect the operations of the electric DU in terms of energy load management. The primary objective of this study was to help the local electric DU in the analysis of the potential effect of high residential solar PV system penetration to the supply and demand load profile in an electric distribution utility (DU grid in the province of Agusan del Norte, Philippines. The energy consumption profiles in the year 2015 were obtained from the electric DU operating in the area. An average daily energy demand load profile was obtained from 0-hr to the 24th hour of the day based from the figures provided by the electric DU. The assessment part of the potential effect of high solar PV system integration assumed four potential total capacities from 10 Mega Watts (MW to 40 MW generated by all subscribers in the area under study at a 10 MW interval. The effect of these capacities were measured and analyzed with respect to the average daily load profile of the DU. Results of this study showed that a combined installations beyond 20 MWp coming from all subscribers is not viable for the local electric DU based on their current energy demand or load profile. Based from the results obtained, the electric DU can make better decisions in the management of high capacity penetration of solar PV systems in the future, including investment in storage systems when extra capacities are generated. Article History: Received July 15th 2016; Received in revised form Sept 23rd 2016; Accepted Oct 1st 2016; Available online How to Cite

  11. BECCS Market Launch Strategy Aiming to Help Ensure Reliable Grid Power at High Penetrations of IRE (Intermittent Renewable Electricity)

    Science.gov (United States)

    WIlliams, R. H.

    2017-12-01

    Despite its recognized importance for carbon (C)-mitigation, progress in advancing biomass energy with CO2 capture and sequestration (BECCS) has been slow. A BECCS market launch strategy based on technologies ready for commercial-scale demonstration is discussed—based on co-gasification of coal and biomass to make H2 with CCS. H2 so produced would be a key element of a H2 balancing capacity (H2-BC) strategy for ensuring reliable grid power at high IRE penetrations. High grid penetrations of IRE must be complemented by fast-ramping balancing (backup and/or storage) capacity (BC) to ensure reliable grid power. BC provided now by natural gas-fired gas turbine combined cycle and combustion turbine units would eventually have to be decarbonized to realize C-mitigation goals, via CCS or other means. Capital-intensive CCS energy systems require baseload operation to realize favourable economics, but at high IRE penetrations, BC plants must be operated at low capacity factors. A H2-BC strategy is a promising way to address this challenge. The elements of a H2-BC system are: (a) H2 production from carbonaceous feedstocks in baseload plants with CCS; (b) H2 consumption in fast-ramping BC units that operate at low capacity factors; (c) Buffer underground H2 storage to enable decoupling baseload H2 production from highly variable H2 consumption by BC units. The concept is likely to "work" because underground H2 storage is expected to be inexpensive. A H2 production analysis is presented for a negative GHG-emitting H2-BC system based on cogasification of corn stover and coal, with captured CO2 used for enhanced oil recovery. The technical readiness of each system component is discussed, and preliminary insights are offered as to the conditions under which the corresponding H2-BC system might compete with natural gas in providing backup for IRE on US electric grids. Public policy to help advance this strategy might be forthcoming, because 2 US Senate bills with broad

  12. Congenital non-syndromal autosomal recessive deafness in Bengkala, an isolated Balinese village.

    Science.gov (United States)

    Winata, S; Arhya, I N; Moeljopawiro, S; Hinnant, J T; Liang, Y; Friedman, T B; Asher, J H

    1995-01-01

    Bengkala is an Indonesian village located on the north shore of Bali that has existed for over 700 years. Currently, 2.2% of the 2185 people in this village have profound congenital deafness. In response to the high incidence of deafness, the people of Bengkala have developed a village specific sign language which is used by many of the hearing and deaf people. Deafness in Bengkala is congenital, sensorineural, non-syndromal, and caused by a fully penetrant autosomal recessive mutation at the DFNB3 locus. The frequency of the DFNB3 mutation is estimated to be 9.4% among hearing people who have a 17.2% chance of being heterozygous for DFNB3. PMID:7616538

  13. New autosomal recessive faciodigitogenital syndrome.

    OpenAIRE

    Teebi, A S; Naguib, K K; Al-Awadi, S; Al-Saleh, Q A

    1988-01-01

    Most pedigrees of Aarskog's faciodigitogenital syndrome have suggested X linked inheritance. However, sex influenced autosomal dominant inheritance is also a possibility in some families. We describe an Arab family of normal consanguineous parents with five children (three males and two females) with some features of Aarskog syndrome in addition to some unusual hair changes. The possibility that this family represents a distinct previously unrecognised faciodigitogenital syndrome with short s...

  14. Electricity markets evolution with the changing generation mix: An empirical analysis based on China 2050 High Renewable Energy Penetration Roadmap

    International Nuclear Information System (INIS)

    Zou, Peng; Chen, Qixin; Yu, Yang; Xia, Qing; Kang, Chongqing

    2017-01-01

    Highlights: • How electricity markets are evolving with the changing generation mix is studied. • China 2050 High Renewable Energy Penetration Roadmap are empirically analysed. • A multi-period Nash-Cournot model is established to study the market equilibrium. • Energy storages are analysed and compared to reveal their impacts on the equilibrium. - Abstract: The power generation mix are significantly changing due to the growth of stricter energy policies. The renewables are increasingly penetrating the power systems and leading to more clean energy and lower energy prices. However, they also require much more flexibilities and ancillary services to handle their uncertainties and variabilities. Thus, the requirements for regulation and reserve services may dramatically increase while the supplies of these services, which are mainly from the traditional thermal plants, remain almost invariant. This changing situation will cause higher regulation and reserve prices and impact the profit models and revenue structures of the traditional plants. How electricity markets are actually evolving with the changing generation mix? Can enough backup power plants be given adequate economic incentives and thus remained with the increasing renewables and the decreasing energy prices and productions? Can de-carbonization be fully performed in power systems? To explicitly answer the question, this paper uses a multi-period Nash-Cournot equilibrium model to formulate the evolution of power markets incorporating different types of generators, including thermal units, hydro units, wind farms, solar stations and energy storage systems. The price changes in the co-optimized energy, regulation and reserve markets, and the profit changes of various generators are studied. And the variabilities and uncertainties of renewable generation sources are considered in dynamically determining the requirements of regulation and reserve services. Based on the China 2050 High Renewable Energy

  15. Long range surface plasmon resonance with ultra-high penetration depth for self-referenced sensing and ultra-low detection limit using diverging beam approach

    Energy Technology Data Exchange (ETDEWEB)

    Isaacs, Sivan, E-mail: sivan.isaacs@gmail.com; Abdulhalim, Ibrahim [Department of Electro-Optical Engineering and TheIlse Katz Institute for Nanoscale Science and Technology, Ben Gurion University of the Negev, Beer Sheva 84105 (Israel); NEW CREATE Programme, School of Materials Science and Engineering, 1 CREATE Way, Research Wing, #02-06/08, Singapore 138602 (Singapore)

    2015-05-11

    Using an insulator-metal-insulator structure with dielectric having refractive index (RI) larger than the analyte, long range surface plasmon (SP) resonance exhibiting ultra-high penetration depth is demonstrated for sensing applications of large bioentities at wavelengths in the visible range. Based on the diverging beam approach in Kretschmann-Raether configuration, one of the SP resonances is shown to shift in response to changes in the analyte RI while the other is fixed; thus, it can be used as a built in reference. The combination of the high sensitivity, high penetration depth and self-reference using the diverging beam approach in which a dark line is detected of the high sensitivity, high penetration depth, self-reference, and the diverging beam approach in which a dark line is detected using large number of camera pixels with a smart algorithm for sub-pixel resolution, a sensor with ultra-low detection limit is demonstrated suitable for large bioentities.

  16. Short-Term and Medium-Term Reliability Evaluation for Power Systems With High Penetration of Wind Power

    DEFF Research Database (Denmark)

    Ding, Yi; Singh, Chanan; Goel, Lalit

    2014-01-01

    reliability evaluation techniques for power systems are well developed. These techniques are more focused on steady-state (time-independent) reliability evaluation and have been successfully applied in power system planning and expansion. In the operational phase, however, they may be too rough......The expanding share of the fluctuating and less predictable wind power generation can introduce complexities in power system reliability evaluation and management. This entails a need for the system operator to assess the system status more accurately for securing real-time balancing. The existing...... an approximation of the time-varying behavior of power systems with high penetration of wind power. This paper proposes a time-varying reliability assessment technique. Time-varying reliability models for wind farms, conventional generating units, and rapid start-up generating units are developed and represented...

  17. Implementation and Assessment of a Decentralized Load Frequency Control: Application to Power Systems with High Wind Energy Penetration

    Directory of Open Access Journals (Sweden)

    Irene Muñoz-Benavente

    2017-01-01

    Full Text Available This paper describes and assesses a decentralized solution based on a wireless sensor-actuator network to provide primary frequency control from demand response in power systems with high wind energy penetration and, subsequently, with relevant frequency excursions. The proposed system is able to modify the electrical power demand of a variety of thermostatically-controlled loads, maintaining minimum comfort levels and minimizing both infrastructure requirements and primary reserves from the supply side. This low-cost hardware solution avoids any additional wiring, extending the wireless sensor-actuator network technology towards small customers, which account for over a 30% share of the current power demand. Frequency excursions are collected by each individual load controller, considering not only the magnitude of the frequency deviation, but also their evolution over time. Based on these time-frequency excursion characteristics, controllers are capable of modifying the power consumption of thermostatically-controlled loads by switching them off and on, thus contributing to primary frequency control in power systems with higher generation unit oscillations as a consequence of relevant wind power integration. Field tests have been carried out in a laboratory environment to assess the load controller performance, as well as to evaluate the electrical and thermal response of individual loads under frequency deviations. These frequency deviations are estimated from power systems with a high penetration of wind energy, which are more sensitive to frequency oscillations and where demand response can significantly contribute to mitigate these frequency excursions. The results, also included in the paper, evaluate the suitability of the proposed load controllers and their suitability to decrease frequency excursions from the demand side in a decentralized manner.

  18. Control of Grid Interactive PV Inverters for High Penetration in Low Voltage Distribution Networks

    OpenAIRE

    Demirok, Erhan

    2012-01-01

    Regarding of high density deployment of PV installations in electricity grids, new technical challenges such as voltage rise, thermal loading of network components, voltage unbalance, harmonic interaction and fault current contributions are being added to tasks list of distribution system operators (DSOs) in order to maintain at least the same power quality as before PVs were not revealed. Potential problems caused by high amount of PV installations can be avoided with technical study of both...

  19. Enhanced Local Grid Voltage Support Method for High Penetration of Distributed Generators

    DEFF Research Database (Denmark)

    Demirok, Erhan; Sera, Dezso; Rodriguez, Pedro

    2011-01-01

    Grid voltage rise and thermal loading of network components are the most remarkable barriers to allow high number of distributed generator (DG) connections on the medium voltage (MV) and low voltage (LV) electricity networks. The other barriers such as grid power quality (harmonics, voltage...

  20. Swiss pumped hydro storage potential for Germany's electricity system under high penetration of intermittent renewable energy

    NARCIS (Netherlands)

    van Meerwijk, Aagje J. H.; Benders, Reinerus; Davila-Martinez, Alejandro; Laugs, Gideon A. H.

    2016-01-01

    In order to cut greenhouse-gas emissions and increase energy security, the European Commission stimulates the deployment of intermittent renewable energy sources (IRES) towards 2050. In an electricity system with high shares of IRES implemented in the network, energy balancing like storage is needed

  1. Storage coordination and peak-shaving operation in urban areas with high renewable penetration

    NARCIS (Netherlands)

    Voulis, N.; Warnier, Martijn; Brazier, F.M.

    2017-01-01

    As renewable power generation gains importance, balancing of power demand and supply becomes more and more challenging. This paper addresses this challenge by exploring the potential of individually-owned storage units in decentralised power systems with a high share of renewables. The focus is

  2. Control of Grid Interactive PV Inverters for High Penetration in Low Voltage Distribution Networks

    DEFF Research Database (Denmark)

    Demirok, Erhan

    Regarding of high density deployment of PV installations in electricity grids, new technical challenges such as voltage rise, thermal loading of network components, voltage unbalance, harmonic interaction and fault current contributions are being added to tasks list of distribution system operators...... of these inverters may depend on grid connection rules which are forced by DSOs. Minimum requirement expected from PV inverters is to transfer maximum power by taking direct current (DC) form from PV modules and release it into AC grid and also continuously keep the inverters synchronized to the grid even under...... for this problem but PV inverters connected to highly capacitive networks are able to employ extra current and voltage harmonics compensation to avoid triggering network resonances at low order frequencies. The barriers such as harmonics interaction, flicker, fault current contribution and dc current injections...

  3. Storage coordination and peak-shaving operation in urban areas with high renewable penetration

    OpenAIRE

    Voulis, N.; Warnier, Martijn; Brazier, F.M.

    2017-01-01

    As renewable power generation gains importance, balancing of power demand and supply becomes more and more challenging. This paper addresses this challenge by exploring the potential of individually-owned storage units in decentralised power systems with a high share of renewables. The focus is on the influence of coordination and peak-shaving operation of these individual units in realistic urban areas. Currently extensive amount of research exits on specific applications related to storage ...

  4. The penetration, diffusion and energy deposition of high-energy photon in layered media

    International Nuclear Information System (INIS)

    Zhengming, Luo; Chengjun, Gou; Laub, Wolfram

    2002-01-01

    This paper presents a new theory for calculating the transport of high-energy photons and their secondary charged particles. We call this new algorithm characteristic line method, which is completely analytic. Using this new method we can not only accurately calculate the transport behavior of energetic photons, but also precisely describes the transport behavior and energy deposition of secondary electrons, photoelectrons, Compton recoil electrons and positron-electron pairs. Its calculation efficiency is much higher than the Monte Carlo method's. The theory can be directly applied to layered media situation and obtain a pencil-beam-modeled solution. Therefore, it may be applied to clinical applications for radiation therapy

  5. Anomalously deep penetration of hydrogen into niobium under action of pulse high temperature hydrogen plasma

    International Nuclear Information System (INIS)

    Didyk, A.Yu.

    2011-01-01

    The method of elastic recoil detection (ERD) has been used for the study of storage and redistribution processes of hydrogen atoms under the influence of pulse high temperature hydrogen plasma obtained using the 'Plasma Focus' PF-4 set-up in three high purity niobium foils. It was established that with an increase of number of PF-4 set-up pulses there occur spreading and transfer of implanted hydrogen atoms to large depths in three Nb-foils which are significantly larger than the projected range of hydrogen ions (with the velocity ∼ 10 8 cm/s). The maximum hydrogen concentration up to 60 at. % is reached in the nearest to Ph-4 surface of the third Nb-foil at 20 impulses of the Ph-4 set-up. The observed phenomenon can be described by transfer of implanted hydrogen atoms under the action of powerful shock waves, created by pulse hydrogen plasma and (or) by accelerating hydrogen atom diffusion under the influence of compression straining wave at the front of the shock wave at redistribution of hydrogen atoms at large depths. Similar behavior was discovered and described also in series of nickel, vanadium, niobium and tantalum foils (two or three foils and more in a series) including series of foils from heterogeneous (different) materials, which were studied, too

  6. Constitutional and acquired autosomal aneuploidy.

    Science.gov (United States)

    Jackson-Cook, Colleen

    2011-12-01

    Chromosomal imbalances can result from numerical or structural anomalies. Numerical chromosomal abnormalities are often referred to as aneuploid conditions. This article focuses on the occurrence of constitutional and acquired autosomal aneuploidy in humans. Topics covered include frequency, mosaicism, phenotypic findings, and etiology. The article concludes with a consideration of anticipated advances that might allow for the development of screening tests and/or lead to improvements in our understanding and management of the role that aneuploidy plays in the aging process and acquisition of age-related and constitutional conditions.

  7. Control Architecture for Intentional Island Operation in Distribution Network with High Penetration of Distributed Generation

    DEFF Research Database (Denmark)

    Chen, Yu

    , the feasibility of the application of Artificial Neural Network (ANN) to ICA is studied, in order to improve the computation efficiency for ISR calculation. Finally, the integration of ICA into Dynamic Security Assessment (DSA), the ICA implementation, and the development of ICA are discussed....... to utilize them for maintaining the security of the power supply under the emergency situations, has been of great interest for study. One proposal is the intentional island operation. This PhD project is intended to develop a control architecture for the island operation in distribution system with high...... amount of DGs. As part of the NextGen project, this project focuses on the system modeling and simulation regarding the control architecture and recommends the development of a communication and information exchange system based on IEC 61850. This thesis starts with the background of this PhD project...

  8. Locational Pricing to Mitigate Voltage Problems Caused by High PV Penetration

    Directory of Open Access Journals (Sweden)

    Sam Weckx

    2015-05-01

    Full Text Available In this paper, a locational marginal pricing algorithm is proposed to control the voltage in unbalanced distribution grids. The increasing amount of photovoltaic (PV generation installed in the grid may cause the voltage to rise to unacceptable levels during periods of low consumption. With locational prices, the distribution system operator can steer the reactive power consumption and active power curtailment of PV panels to guarantee a safe network operation. Flexible loads also respond to these prices. A distributed gradient algorithm automatically defines the locational prices that avoid voltage problems. Using these locational prices results in a minimum cost for the distribution operator to control the voltage. Locational prices can differ between the three phases in unbalanced grids. This is caused by a higher consumption or production in one of the phases compared to the other phases and provides the opportunity for arbitrage, where power is transferred from a phase with a low price to a phase with a high price. The effect of arbitrage is analyzed. The proposed algorithm is applied to an existing three-phase four-wire radial grid. Several simulations with realistic data are performed.

  9. Autosomal dominant distal myopathy: Linkage to chromosome 14

    Energy Technology Data Exchange (ETDEWEB)

    Laing, N.G.; Laing, B.A.; Wilton, S.D.; Dorosz, S.; Mastaglia, F.L.; Kakulas, B.A. [Australian Neuromuscular Research Institute, Perth (Australia); Robbins, P.; Meredith, C.; Honeyman, K.; Kozman, H.

    1995-02-01

    We have studied a family segregating a form of autosomal dominant distal myopathy (MIM 160500) and containing nine living affected individuals. The myopathy in this family is closest in clinical phenotype to that first described by Gowers in 1902. A search for linkage was conducted using microsatellite, VNTR, and RFLP markers. In total, 92 markers on all 22 autosomes were run. Positive linkage was obtained with 14 of 15 markers tested on chromosome 14, with little indication of linkage elsewhere in the genome. Maximum two-point LOD scores of 2.60 at recombination fraction .00 were obtained for the markers MYH7 and D14S64 - the family structure precludes a two-point LOD score {ge} 3. Recombinations with D14S72 and D14S49 indicate that this distal myopathy locus, MPD1, should lie between these markers. A multipoint analysis assuming 100% penetrance and using the markers D14S72, D14S50, MYH7, D14S64, D14S54, and D14S49 gave a LOD score of exactly 3 at MYH7. Analysis at a penetrance of 80% gave a LOD score of 2.8 at this marker. This probable localization of a gene for distal myopathy, MPD1, on chromosome 14 should allow other investigators studying distal myopathy families to test this region for linkage in other types of the disease, to confirm linkage or to demonstrate the likely genetic heterogeneity. 24 refs., 3 figs., 1 tab.

  10. Kita driven expression of oncogenic HRAS leads to early onset and highly penetrant melanoma in zebrafish.

    Directory of Open Access Journals (Sweden)

    Cristina Santoriello

    2010-12-01

    Full Text Available Melanoma is the most aggressive and lethal form of skin cancer. Because of the increasing incidence and high lethality of melanoma, animal models for continuously observing melanoma formation and progression as well as for testing pharmacological agents are needed.Using the combinatorial Gal4-UAS system, we have developed a zebrafish transgenic line that expresses oncogenic HRAS under the kita promoter. Already at 3 days transgenic kita-GFP-RAS larvae show a hyper-pigmentation phenotype as earliest evidence of abnormal melanocyte growth. By 2-4 weeks, masses of transformed melanocytes form in the tail stalk of the majority of kita-GFP-RAS transgenic fish. The adult tumors evident between 1-3 months of age faithfully reproduce the immunological, histological and molecular phenotypes of human melanoma, but on a condensed time-line. Furthermore, they show transplantability, dependence on mitfa expression and do not require additional mutations in tumor suppressors. In contrast to kita expressing melanocyte progenitors that efficiently develop melanoma, mitfa expressing progenitors in a second Gal4-driver line were 4 times less efficient in developing melanoma during the three months observation period.This indicates that zebrafish kita promoter is a powerful tool for driving oncogene expression in the right cells and at the right level to induce early onset melanoma in the presence of tumor suppressors. Thus our zebrafish model provides a link between kita expressing melanocyte progenitors and melanoma and offers the advantage of a larval phenotype suitable for large scale drug and genetic modifier screens.

  11. A highly penetrant form of childhood apraxia of speech due to deletion of 16p11.2.

    Science.gov (United States)

    Fedorenko, Evelina; Morgan, Angela; Murray, Elizabeth; Cardinaux, Annie; Mei, Cristina; Tager-Flusberg, Helen; Fisher, Simon E; Kanwisher, Nancy

    2016-02-01

    Individuals with heterozygous 16p11.2 deletions reportedly suffer from a variety of difficulties with speech and language. Indeed, recent copy-number variant screens of children with childhood apraxia of speech (CAS), a specific and rare motor speech disorder, have identified three unrelated individuals with 16p11.2 deletions. However, the nature and prevalence of speech and language disorders in general, and CAS in particular, is unknown for individuals with 16p11.2 deletions. Here we took a genotype-first approach, conducting detailed and systematic characterization of speech abilities in a group of 11 unrelated children ascertained on the basis of 16p11.2 deletions. To obtain the most precise and replicable phenotyping, we included tasks that are highly diagnostic for CAS, and we tested children under the age of 18 years, an age group where CAS has been best characterized. Two individuals were largely nonverbal, preventing detailed speech analysis, whereas the remaining nine met the standard accepted diagnostic criteria for CAS. These results link 16p11.2 deletions to a highly penetrant form of CAS. Our findings underline the need for further precise characterization of speech and language profiles in larger groups of affected individuals, which will also enhance our understanding of how genetic pathways contribute to human communication disorders.

  12. Determination of penetration depth at high velocity impact using finite element method and artificial neural network tools

    Directory of Open Access Journals (Sweden)

    Namık KılıÇ

    2015-06-01

    Full Text Available Determination of ballistic performance of an armor solution is a complicated task and evolved significantly with the application of finite element methods (FEM in this research field. The traditional armor design studies performed with FEM requires sophisticated procedures and intensive computational effort, therefore simpler and accurate numerical approaches are always worthwhile to decrease armor development time. This study aims to apply a hybrid method using FEM simulation and artificial neural network (ANN analysis to approximate ballistic limit thickness for armor steels. To achieve this objective, a predictive model based on the artificial neural networks is developed to determine ballistic resistance of high hardness armor steels against 7.62 mm armor piercing ammunition. In this methodology, the FEM simulations are used to create training cases for Multilayer Perceptron (MLP three layer networks. In order to validate FE simulation methodology, ballistic shot tests on 20 mm thickness target were performed according to standard Stanag 4569. Afterwards, the successfully trained ANN(s is used to predict the ballistic limit thickness of 500 HB high hardness steel armor. Results show that even with limited number of data, FEM-ANN approach can be used to predict ballistic penetration depth with adequate accuracy.

  13. Flexible fault ride through strategy for wind farm clusters in power systems with high wind power penetration

    International Nuclear Information System (INIS)

    Wang, Songyan; Chen, Ning; Yu, Daren; Foley, Aoife; Zhu, Lingzhi; Li, Kang; Yu, Jilai

    2015-01-01

    Highlights: • A flexible fault ride through strategy is proposed. • The strategy comprises of grid code requirements and power restrictions. • Slight faults and moderate faults are the main defending objectives. • Temporary overloading capability of the doubly fed induction generator is considered. - Abstract: This paper investigates a flexible fault ride through strategy for power systems in China with high wind power penetration. The strategy comprises of adaptive fault ride through requirements and maximum power restrictions of the wind farms with weak fault ride through capabilities. The slight faults and moderate faults with high probability are the main defending objective of the strategy. The adaptive fault ride through requirement in the strategy consists of two sub fault ride through requirements, a temporary slight voltage ride through requirement corresponding to a slight fault incident, with a moderate voltage ride through requirement corresponding to a moderate fault. The temporary overloading capability of the wind farm is reflected in both requirements to enhance the capability to defend slight faults and to avoid tripping when the crowbar is disconnected after moderate faults are cleared. For those wind farms that cannot meet the adaptive fault ride through requirement, restrictions are put on the maximum power output. Simulation results show that the flexible fault ride through strategy increases the fault ride through capability of the wind farm clusters and reduces the wind power curtailment during faults

  14. Application of Phasor Measurement Units for Protection of Distribution Networks with High Penetration of Photovoltaic Sources

    Science.gov (United States)

    Meskin, Matin

    The rate of the integration of distributed generation (DG) units to the distribution level to meet the growth in demand increases as a reasonable replacement for costly network expansion. This integration brings many advantages to the consumers and power grids, as well as giving rise to more challenges in relation to protection and control. Recent research has brought to light the negative effects of DG units on short circuit currents and overcurrent (OC) protection systems in distribution networks. Change in the direction of fault current flow, increment or decrement of fault current magnitude, blindness of protection, feeder sympathy trip, nuisance trip of interrupting devices, and the disruption of coordination between protective devices are some potential impacts of DG unit integration. Among other types of DG units, the integration of renewable energy resources into the electric grid has seen a vast improvement in recent years. In particular, the interconnection of photovoltaic (PV) sources to the medium voltage (MV) distribution networks has experienced a rapid increase in the last decade. In this work, the effect of PV source on conventional OC relays in MV distribution networks is shown. It is indicated that the PV output fluctuation, due to changes in solar radiation, causes the magnitude and direction of the current to change haphazardly. These variations may result in the poor operation of OC relays as the main protective devices in the MV distribution networks. In other words, due to the bi-directional power flow characteristic and the fluctuation of current magnitude occurring in the presence of PV sources, a specific setting of OC relays is difficult to realize. Therefore, OC relays may operate in normal conditions. To improve the OC relay operation, a voltage-dependent-overcurrent protection is proposed. Although, this new method prevents the OC relay from maloperation, its ability to detect earth faults and high impedance faults is poor. Thus, a

  15. Autosomal dominant adult neuronal ceroid lipofuscinosis

    NARCIS (Netherlands)

    Nijssen, Peter C.G.

    2011-01-01

    this thesis investigates a family with autosomal dominant neuronal ceroid lipofuscinosis, with chapters on clinical neurology, neuropathology, neurogenetics, neurophysiology, auditory and visual aspects.

  16. The effect of high-pressure devitrification and densification on ballistic-penetration resistance of fused silica

    Science.gov (United States)

    Avuthu, Vasudeva Reddy

    perhaps alpha-quartz) within fused silica during ballistic impact. To rationalize the findings obtained, the all-atom molecular-level computational analysis is complemented by a series of quantum-mechanics density functional theory (DFT) computations. The latter computations enable determination of the relative potential energies of the fused silica, alpha-quartz and stishovite under ambient pressure (i.e. under their natural densities) as well as under imposed (as high as 50 GPa) pressures (i.e. under higher densities) and shear strains. In addition, the transition states associated with various fused-silica devitrification processes were identified. In the second part of the present work, the molecular-level computational results obtained in the first portion of the work are used to enrich a continuum-type constitutive model (that is, the so-called Johnson-Holmquist-2, JH2, model) for fused silica. Since the aforementioned devitrification and permanent-densification processes modify the response of fused silica to the pressure as well as to the deviatoric part of the stress, changes had to be made in both the JH2 equation of state and the strength model. To assess the potential improvements with respect to the ballistic-penetration resistance of this material brought about by the fused-silica devitrification and permanent-densification processes, a series of transient non-linear dynamics finite element analyses of the transverse impact of a fused-silica test plate with a solid right-circular cylindrical steel projectile was conducted. The results obtained revealed that, provided the projectile incident velocity and, hence, the attendant pressure, is sufficiently high, fused silica can undergo impact-induced energy-consuming devitrification, which improves its ballistic-penetration resistance.

  17. Three-Phase Short-Circuit Current Calculation of Power Systems with High Penetration of VSC-Based Renewable Energy

    Directory of Open Access Journals (Sweden)

    Niancheng Zhou

    2018-03-01

    Full Text Available Short-circuit current level of power grid will be increased with high penetration of VSC-based renewable energy, and a strong coupling between transient fault process and control strategy will change the fault features. The full current expression of VSC-based renewable energy was obtained according to transient characteristics of short-circuit current. Furtherly, by analyzing the closed-loop transfer function model of controller and current source characteristics presented in steady state during a fault, equivalent circuits of VSC-based renewable energy of fault transient state and steady state were proposed, respectively. Then the correctness of the theory was verified by experimental tests. In addition, for power grid with VSC-based renewable energy, superposition theorem was used to calculate AC component and DC component of short-circuit current, respectively, then the peak value of short-circuit current was evaluated effectively. The calculated results could be used for grid planning and design, short-circuit current management as well as adjustment of relay protection. Based on comparing calculation and simulation results of 6-node 500 kV Huainan power grid and 35-node 220 kV Huaisu power grid, the effectiveness of the proposed method was verified.

  18. Highly variable penetrance of abnormal phenotypes in embryonic lethal knockout mice [version 2; referees: 1 approved, 2 approved with reservations

    Directory of Open Access Journals (Sweden)

    Robert Wilson

    2017-02-01

    Full Text Available Background: Identifying genes that are essential for mouse embryonic development and survival through term is a powerful and unbiased way to discover possible genetic determinants of human developmental disorders. Characterising the changes in mouse embryos that result from ablation of lethal genes is a necessary first step towards uncovering their role in normal embryonic development and establishing any correlates amongst human congenital abnormalities. Methods: Here we present results gathered to date in the Deciphering the Mechanisms of Developmental Disorders (DMDD programme, cataloguing the morphological defects identified from comprehensive imaging of 220 homozygous mutant and 114 wild type embryos from 42 lethal and subviable lines, analysed at E14.5. Results: Virtually all mutant embryos show multiple abnormal phenotypes and amongst the 42 lines these affect most organ systems. Within each mutant line, the phenotypes of individual embryos form distinct but overlapping sets. Subcutaneous edema, malformations of the heart or great vessels, abnormalities in forebrain morphology and the musculature of the eyes are all prevalent phenotypes, as is loss or abnormal size of the hypoglossal nerve. Conclusions: Overall, the most striking finding is that no matter how profound the malformation, each phenotype shows highly variable penetrance within a mutant line. These findings have challenging implications for efforts to identify human disease correlates.

  19. Optimal Operation and Management for Smart Grid Subsumed High Penetration of Renewable Energy, Electric Vehicle, and Battery Energy Storage System

    Science.gov (United States)

    Shigenobu, Ryuto; Noorzad, Ahmad Samim; Muarapaz, Cirio; Yona, Atsushi; Senjyu, Tomonobu

    2016-04-01

    Distributed generators (DG) and renewable energy sources have been attracting special attention in distribution systems in all over the world. Renewable energies, such as photovoltaic (PV) and wind turbine generators are considered as green energy. However, a large amount of DG penetration causes voltage deviation beyond the statutory range and reverse power flow at interconnection points in the distribution system. If excessive voltage deviation occurs, consumer's electric devices might break and reverse power flow will also has a negative impact on the transmission system. Thus, mass interconnections of DGs has an adverse effect on both of the utility and the customer. Therefore, reactive power control method is proposed previous research by using inverters attached DGs for prevent voltage deviations. Moreover, battery energy storage system (BESS) is also proposed for resolve reverse power flow. In addition, it is possible to supply high quality power for managing DGs and BESSs. Therefore, this paper proposes a method to maintain voltage, active power, and reactive power flow at interconnection points by using cooperative controlled of PVs, house BESSs, EVs, large BESSs, and existing voltage control devices. This paper not only protect distribution system, but also attain distribution loss reduction and effectivity management of control devices. Therefore mentioned control objectives are formulated as an optimization problem that is solved by using the Particle Swarm Optimization (PSO) algorithm. Modified scheduling method is proposed in order to improve convergence probability of scheduling scheme. The effectiveness of the proposed method is verified by case studies results and by using numerical simulations in MATLAB®.

  20. System behaviour of compressed-air energy-storage in Denmark with a high penetration of renewable energy sources

    International Nuclear Information System (INIS)

    Salgi, Georges; Lund, Henrik

    2008-01-01

    In 2005, wind power supplied 19% of the 36 TWh annual electricity demand in Denmark, while 50% was produced at combined heat-and-power plants (CHP). The installed wind-turbine capacity in Western Denmark exceeds the local demand at certain points in time. So far, excess production has been exported to neighbouring countries. However, plans to expand wind power both in Denmark and in its neighbouring countries could restrain the export option and create transmission congestion challenges. This results in a need to increase the flexibility of the local electricity-system. Compressed-Air Energy-Storage (CAES) has been proposed as a potential solution for levelling fluctuating wind-power production and maintaining a system balance. This paper analyses the energy-balance effects of adding CAES to the Western Danish energy-system. Results show that even with an unlimited CAES plant capacity, excess power production is not eliminated because of the high percentage of CHP production. The optimal wind-power penetration for maximum CAES operation is found to be around 55%. The minimum storage size for CAES to fully eliminate condensing power plants operation in the optimized system is over 500 GWh, which corresponds to a cavern volume of around 234 Mm 3 at an average pressure of 60 bar. Such a storage size would be technically and economically unfeasible. The analysis, however, did not include the potential role of a CAES plant in regulating the power services. (author)

  1. A Hierarchical Optimal Operation Strategy of Hybrid Energy Storage System in Distribution Networks with High Photovoltaic Penetration

    Directory of Open Access Journals (Sweden)

    Jian Chen

    2018-02-01

    Full Text Available In this paper, a hierarchical optimal operation strategy for a hybrid energy storage system (HESS is proposed, which is suitable to be utilized in distribution networks (DNs with high photovoltaic (PV penetration to achieve PV power smoothing, voltage regulation and price arbitrage. Firstly, a fuzzy-logic based variable step-size control strategy for an ultracapacitor (UC with the improvement of the lifetime of UC and tracking performance is adopted to smooth PV power fluctuations. The impact of PV forecasting errors is eliminated by adjusting the UC power in real time. Secondly, a coordinated control strategy, which includes centralized and local controls, is proposed for lithium-ion batteries. The centralized control is structured to determine the optimal battery unit for voltage regulation or price arbitrage according to lithium-ion battery performance indices. A modified lithium-ion battery aging model with better accuracy is proposed and the coupling relationship between the lifetime and the effective capacity is also considered. Additionally, the local control of the selected lithium-ion battery unit determines the charging/discharging power. A case study is used to validate the operation strategy and the results show that the lifetime equilibrium among different lithium-ion battery units can be achieved using the proposed strategy.

  2. Hybrid Genetic Algorithm Fuzzy-Based Control Schemes for Small Power System with High-Penetration Wind Farms

    Directory of Open Access Journals (Sweden)

    Mohammed Elsayed Lotfy

    2018-03-01

    Full Text Available Wind is a clean, abundant, and inexhaustible source of energy. However, wind power is not constant, as windmill output is proportional to the cube of wind speed. As a result, the generated power of wind turbine generators (WTGs fluctuates significantly. Power fluctuation leads to frequency deviation and voltage flicker inside the system. This paper presents a new methodology for controlling system frequency and power. Two decentralized fuzzy logic-based control schemes with a high-penetration non-storage wind–diesel system are studied. First, one is implemented in the governor of conventional generators to damp frequency oscillation, while the other is applied to control the pitch angle system of wind turbines to smooth wind output power fluctuations and enhance the power system performance. A genetic algorithm (GA is employed to tune and optimize the membership function parameters of the fuzzy logic controllers to obtain optimal performance. The effectiveness of the suggested controllers is validated by time domain simulation for the standard IEEE nine-bus three-generator test system, including three wind farms. The robustness of the power system is checked under normal and faulty operating conditions.

  3. Convective transport of highly plasma protein bound drugs facilitates direct penetration into deep tissues after topical application

    Science.gov (United States)

    Dancik, Yuri; Anissimov, Yuri G; Jepps, Owen G; Roberts, Michael S

    2012-01-01

    AIMS To relate the varying dermal, subcutaneous and muscle microdialysate concentrations found in man after topical application to the nature of the drug applied and to the underlying physiology. METHODS We developed a physiologically based pharmacokinetic model in which transport to deeper tissues was determined by tissue diffusion, blood, lymphatic and intersitial flow transport and drug properties. The model was applied to interpret published human microdialysis data, estimated in vitro dermal diffusion and protein binding affinity of drugs that have been previously applied topically in vivo and measured in deep cutaneous tissues over time. RESULTS Deeper tissue microdialysis concentrations for various drugs in vivo vary widely. Here, we show that carriage by the blood to the deeper tissues below topical application sites facilitates the transport of highly plasma protein bound drugs that penetrate the skin, leading to rapid and significant concentrations in those tissues. Hence, the fractional concentration for the highly plasma protein bound diclofenac in deeper tissues is 0.79 times that in a probe 4.5 mm below a superficial probe whereas the corresponding fractional concentration for the poorly protein bound nicotine is 0.02. Their corresponding estimated in vivo lag times for appearance of the drugs in the deeper probes were 1.1 min for diclofenac and 30 min for nicotine. CONCLUSIONS Poorly plasma protein bound drugs are mainly transported to deeper tissues after topical application by tissue diffusion whereas the transport of highly plasma protein bound drugs is additionally facilitated by convective blood, lymphatic and interstitial transport to deep tissues. PMID:21999217

  4. Modelling the effects of penetrance and family size on rates of sporadic and familial disease.

    Science.gov (United States)

    Al-Chalabi, Ammar; Lewis, Cathryn M

    2011-01-01

    Many complex diseases show a diversity of inheritance patterns ranging from familial disease, manifesting with autosomal dominant inheritance, through to simplex families in which only one person is affected, manifesting as apparently sporadic disease. The role of ascertainment bias in generating apparent patterns of inheritance is often overlooked. We therefore explored the role of two key parameters that influence ascertainment, penetrance and family size, in rates of observed familiality. We develop a mathematical model of familiality of disease, with parameters for penetrance, mutation frequency and family size, and test this in a complex disease: amyotrophic lateral sclerosis. Monogenic, high-penetrance variants can explain patterns of inheritance in complex diseases and account for a large proportion of those with no apparent family history. With current demographic trends, rates of familiality will drop further. For example, a variant with penetrance 0.5 will cause apparently sporadic disease in 12% of families of size 10, but 80% of families of size 1. A variant with penetrance 0.9 has only an 11% chance of appearing sporadic in families of a size similar to those of Ireland in the past, compared with 57% in one-child families like many in China. These findings have implications for genetic counselling, disease classification and the design of gene-hunting studies. The distinction between familial and apparently sporadic disease should be considered artificial. Copyright © 2011 S. Karger AG, Basel.

  5. Autosomal dominant hereditary ataxia in Sri Lanka

    OpenAIRE

    Sumathipala, Dulika S; Abeysekera, Gayan S; Jayasekara, Rohan W; Tallaksen, Chantal ME; Dissanayake, Vajira HW

    2013-01-01

    Background Spinocerebellar ataxias (SCA) are a group of hereditary neurodegenerative disorders. Prevalence of SCA subtypes differ worldwide. Autosomal dominant ataxias are the commonest types of inherited ataxias seen in Sri Lanka. The aim of the study is to determine the genetic etiology of patients with autosomal dominant ataxia in Sri Lanka and to describe the clinical features of each genetic subtype. Methods ...

  6. Autosomal-dominant osteopetrosis: An incidental finding

    Directory of Open Access Journals (Sweden)

    Rajathi Maria

    2010-01-01

    Full Text Available Osteopetrosis is a descriptive term that refers to a group of rare, heritable disorders of the skeleton. Osteopetrotic conditions vary greatly in their presentation and severity, from just as an incidental finding on radiographs to causing life-threatening complications such as bone marrow suppression. It is caused by failure of osteoclast development and function. Osteopetrosis can be inherited as autosomal-recessive, autosomal-dominant or as X-linked traits, with the most severe forms being the autosomal-recessive ones. The severity of the disease is mild to moderate in the autosomal-dominant forms, with normal life expectancy. Diagnosis is largely based on clinical and radiographic evaluation. The present paper reports a case of autosomal-dominant osteopetrosis complicated by osteomyelitis with a short review of the condition.

  7. Spinning reserve quantification by a stochastic–probabilistic scheme for smart power systems with high wind penetration

    International Nuclear Information System (INIS)

    Khazali, Amirhossein; Kalantar, Mohsen

    2015-01-01

    Highlights: • A stochastic–probabilistic approach is proposed for spinning reserve quantification. • A new linearized formulation integrating reliability metrics is presented. • The framework manages the reserve provided by responsive loads and storage systems. • The proposed method is capable of detaching the spinning reserve for different uses. - Abstract: This paper introduces a novel spinning reserve quantification scheme based on a hybrid stochastic–probabilistic approach for smart power systems including high penetration of wind generation. In this research the required spinning reserve is detached into two main parts. The first part of the reserve is procured to overcome imbalances between load and generation in the system. The second part of the required spinning reserve is scheduled according to the probability of unit outages. In order to overcome uncertainties caused by wind generation and load forecasting errors different scenarios of wind generation and load uncertainties are generated. For each scenario the reserve deployed by different components are taken account as the first part of the required reserve which is used to overcome imbalances. The second part of the required reserve is based on reliability constraints. The total expected energy not supplied (TEENS) is the reliability criterion which determines the second part of the required spinning reserve to overcome unit outage possibilities. This formulation permits the independent system operator to purchase the two different types of reserve with different prices. The introduced formulation for reserve quantification is also capable of managing and detaching the reserve provided by responsive loads and energy storage devices. The problem is formulated as a mixed integer linear programming (MILP) problem including linearized formulations for reliability metrics. Obtained results show the efficiency of the proposed approach compared with the conventional stochastic and deterministic

  8. Adaptation and penetration of resin-based root canal sealers in root canals irradiated with high-intensity lasers

    Science.gov (United States)

    Moura-Netto, Cacio; Mello-Moura, Anna Carolina Volpi; Palo, Renato Miotto; Prokopowitsch, Igor; Pameijer, Cornelis H.; Marques, Marcia Martins

    2015-03-01

    This research analyzed the quality of resin-based sealer adaptation after intracanal laser irradiation. Extracted teeth (n=168) were root canal treated and divided into four groups, according to dentin surface treatment: no laser; Nd:YAG laser (1.5 W, 100 mJ, 15 Hz) diode laser (2.5 W in CW), and Er:YAG laser (1 W, 100 mJ, 10 Hz). The teeth were divided into four subgroups according to the sealer used: AH Plus, EndoREZ, Epiphany, and EpiphanySE. For testing the sealing after root canal obturation, the penetration of silver nitrate solution was measured, whereas to evaluate the adaptation and penetration of the sealer into the dentin, environmental scanning electron microscopy (ESEM) was used. The ESEM images were analyzed using a four-grade criteria score by three evaluators. The inter-examiner agreement was confirmed by Kappa test and the scores statistically compared by the Kruskal-Wallis' test (p<0.05). Both adaptation and sealer penetration in root canals were not affected by the laser irradiation. Nd:YAG and diode laser decreased the tracer penetration for AH Plus, whereas EndoREZ and EpiphanySE performances were affected by Nd:YAG irradiation (p<0.05). It can be concluded that intracanal laser irradiation can be used as an adjunct in endodontic treatment; however, the use of hydrophilic resin sealers should be avoided when root canals were irradiated with Nd:YAG laser.

  9. High-resolution, real-time mapping of surface soil moisture at the field scale using ground penetrating radar

    Science.gov (United States)

    Lambot, S.; Minet, J.; Slob, E.; Vereecken, H.; Vanclooster, M.

    2008-12-01

    Measuring soil surface water content is essential in hydrology and agriculture as this variable controls important key processes of the hydrological cycle such as infiltration, runoff, evaporation, and energy exchanges between the earth and the atmosphere. We present a ground-penetrating radar (GPR) method for automated, high-resolution, real-time mapping of soil surface dielectric permittivity and correlated water content at the field scale. Field scale characterization and monitoring is not only necessary for field scale management applications, but also for unravelling upscaling issues in hydrology and bridging the scale gap between local measurements and remote sensing. In particular, such methods are necessary to validate and improve remote sensing data products. The radar system consists of a vector network analyzer combined with an off-ground, ultra-wideband monostatic horn antenna, thereby setting up a continuous-wave steeped-frequency GPR. Radar signal analysis is based on three-dimensional electromagnetic inverse modelling. The forward model accounts for all antenna effects, antenna-soil interactions, and wave propagation in three-dimensional multilayered media. A fast procedure was developed to evaluate the involved Green's function, resulting from a singular, complex integral. Radar data inversion is focused on the surface reflection in the time domain. The method presents considerable advantages compared to the current surface characterization methods using GPR, namely, the ground wave and common reflection methods. Theoretical analyses were performed, dealing with the effects of electric conductivity on the surface reflection when non-negligible, and on near-surface layering, which may lead to unrealistic values for the surface dielectric permittivity if not properly accounted for. Inversion strategies are proposed. In particular the combination of GPR with electromagnetic induction data appears to be promising to deal with highly conductive soils

  10. Europa Kinetic Ice Penetrator (EKIP)

    Data.gov (United States)

    National Aeronautics and Space Administration — The goal of the proposed work is to validate an initial design for a Europa penetrator that can withstand the high g load associated with the expected hypervelocity...

  11. The Norwegian PMS2 founder mutation c.989-1G > T shows high penetrance of microsatellite instable cancers with normal immunohistochemistry.

    Science.gov (United States)

    Grindedal, Eli Marie; Aarset, Harald; Bjørnevoll, Inga; Røyset, Elin; Mæhle, Lovise; Stormorken, Astrid; Heramb, Cecilie; Medvik, Heidi; Møller, Pål; Sjursen, Wenche

    2014-01-01

    Using immunohistochemistry (IHC) to select cases for mismatch repair (MMR) genetic testing, we failed to identify a large kindred with the deleterious PMS2 mutation c.989-1G > T. The purpose of the study was to examine the sensitivity of IHC and microsatellite instability-analysis (MSI) to identify carriers of the mutation, and to estimate its penetrance and expressions. All carriers and obligate carriers of the mutation were identified. All cancer diagnoses were confirmed. IHC and MSI-analysis were performed on available tumours. Penetrances of cancers included in the Amsterdam and the Bethesda Criteria, for MSI-high tumours and MSI-high and low tumours were calculated by the Kaplan-Meier algorithm. Probability for co-segregation of the mutation and cancers by chance was 0.000004. Fifty-six carriers or obligate carriers were identified. There was normal staining for PMS2 in 15/18 (83.3%) of tumours included in the AMS1/AMS2/Bethesda criteria. MSI-analysis showed that 15/21 (71.4%) of tumours were MSI-high and 4/21 (19.0%) were MSI-low. Penetrance at 70 years was 30.6% for AMS1 cancers (colorectal cancers), 42.8% for AMS2 cancers, 47.2% for Bethesda cancers, 55.6% for MSI-high and MSI-low cancers and 52.2% for MSI-high cancers. The mutation met class 5 criteria for pathogenicity. IHC was insensitive in detecting tumours caused by the mutation. Penetrance of cancers that displayed MSI was 56% at 70 years. Besides colorectal cancers, the most frequent expressions were carcinoma of the endometrium and breast in females and stomach and prostate in males.

  12. ANTHEM simulation of the early time magnetic field penetration of the plasma surrounding a high density Z-pinch

    International Nuclear Information System (INIS)

    Mason, R.J.

    1989-01-01

    The early time penetration of magnetic field into the low density coronal plasma of a Z-pinch fiber is studied with the implicit plasma simulation code ANTHEM. Calculations show the emission of electrons from the cathode, pinching of the electron flow, magnetic insulation of the electrons near the anode, and low density ion blow off. PIC-particle ion calculations show a late time clumping of the ion density not seen with a fluid ion treatment. 4 refs., 4 figs

  13. Systematic Multi-variable H-infinity Control Design for Primary Frequency Regulation in Stand-alone Microgrids with High Penetration of Renewable Energy Sources

    OpenAIRE

    Lam , Quang Linh; Bratcu , Antoneta Iuliana; Riu , Delphine

    2016-01-01

    International audience; In this paper, a systematic design of a robust multi-variable control structure for primary frequency regulation in microgrids with high rate of renewable source penetration is proposed. The considered microgrid represents a diesel-photovoltaic-supercapacitor hybrid power generation system operating in stand-alone mode. The proposed control structure relies on a two-level architecture: classical PI-based current tracking controllers are placed on the low control level ...

  14. Andhidrotic ectodermal dysplasia-autosomal recessive form

    Directory of Open Access Journals (Sweden)

    Inamadar Arun

    1994-01-01

    Full Text Available Anhidrotic ectodermal dysplasia with classical features in 2 sisters is reported. The mode of inheritance in these seems to be autosomal recessive; which is a very rare occurrence.

  15. Autosomal dominant inheritance of Weaver syndrome.

    OpenAIRE

    Fryer, A; Smith, C; Rosenbloom, L; Cole, T

    1997-01-01

    Most report of Weaver syndrome have been sporadic cases and the genetic basis of the syndrome is uncertain. This report of an affected father and daughter provides evidence for autosomal dominant inheritance.

  16. Genetics Home Reference: autosomal dominant hypocalcemia

    Science.gov (United States)

    ... individuals have features of a kidney disorder called Bartter syndrome in addition to hypocalcemia. These features can include ... sometimes referred to as autosomal dominant hypocalcemia with Bartter syndrome or Bartter syndrome type V. There are two ...

  17. Adaptation and penetration of resin-based root canal sealers in root canals irradiated with high-intensity lasers.

    Science.gov (United States)

    Moura-Netto, Cacio; Mello-Moura, Anna Carolina Volpi; Palo, Renato Miotto; Prokopowitsch, Igor; Pameijer, Cornelis H; Marques, Marcia Martins

    2015-03-01

    This research analyzed the quality of resin-based sealer adaptation after intracanal laser irradiation. Extracted teeth (n = 168) were root canal treated and divided into four groups, according to dentin surface treatment: no laser; Nd:YAG laser (1.5 W, 100 mJ, 15 Hz); diode laser (2.5 W in CW), and Er:YAG laser (1 W, 100 mJ, 10 Hz). The teeth were divided into four subgroups according to the sealer used: AH Plus, EndoREZ, Epiphany, and EpiphanySE. For testing the sealing after root canal obturation, the penetration of silver nitrate solution was measured, whereas to evaluate the adaptation and penetration of the sealer into the dentin, environmental scanning electron microscopy (ESEM) was used. The ESEM images were analyzed using a four-grade criteria score by three evaluators. The inter-examiner agreement was confirmed by Kappa test and the scores statistically compared by the Kruskal-Wallis' test (p laser irradiation. Nd:YAG and diode laser decreased the tracer penetration for AH Plus, whereas EndoREZ and EpiphanySE performances were affected by Nd:YAG irradiation (p laser irradiation can be used as an adjunct in endodontic treatment; however, the use of hydrophilic resin sealers should be avoided when root canals were irradiated with Nd:YAG laser.

  18. Genotype-phenotype correlation in FMF patients: A "non classic" recessive autosomal or "atypical" dominant autosomal inheritance?

    Science.gov (United States)

    Procopio, V; Manti, S; Bianco, G; Conti, G; Romeo, A; Maimone, F; Arrigo, T; Cutrupi, M C; Salpietro, C; Cuppari, C

    2018-01-30

    Uncertainty remains on the pathogenetic mechanisms, model of inheritance as well as genotype-phenotype correlation of FMF disease. To investigate the impact of genetic factors on the FMF phenotype and the disease inheritance model. A total of 107 FMF patients were enrolled. Patients were diagnosed clinically. All patients underwent genetic analysis of the FMF locus on 16p13.3. 9 distinct mutations were detected. Specifically, the 85.98% of patients showed a heterozygous genotype. The most common genotypes were p.Met680Ile/wt and p.Met694Val/wt. The most frequent clinical findings were fever, abdominal pain, joint pain, thoracic pain, and erysipelas-like erythema. Analysis of clinical data did not detect any significant difference in clinical phenotype among heterozygous, homozygous as well as compound homozygous subjects, further supporting the evidence that, contrary to the recessive autosomal inheritance, heterozygous patients fulfilled the criteria of clinical FMF. Moreover, subjects with p.Met694Val/wt and p.Met680Ile/wt genotype reported the most severe clinical phenotype. p.Ala744Ser/wt, p.Glu148Gln/Met680Ile, p.Met680Ile/Met680Ile, p.Met680Ile/Met694Val, p.Pro369Ser/wt, p.Met694Ile/wt, p.Glu148Gln/Glu148Gln, p.Lys695Arg/wt resulted in 100% pathogenicity. The existence of a "non classic" autosomal recessive inheritance as well as of an "atypical" dominant autosomal inheritance with incomplete penetrance and variable expressivity cannot be excluded in FMF. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. The population genetics of X-autosome synthetic lethals and steriles.

    Science.gov (United States)

    Lachance, Joseph; Johnson, Norman A; True, John R

    2011-11-01

    Epistatic interactions are widespread, and many of these interactions involve combinations of alleles at different loci that are deleterious when present in the same individual. The average genetic environment of sex-linked genes differs from that of autosomal genes, suggesting that the population genetics of interacting X-linked and autosomal alleles may be complex. Using both analytical theory and computer simulations, we analyzed the evolutionary trajectories and mutation-selection balance conditions for X-autosome synthetic lethals and steriles. Allele frequencies follow a set of fundamental trajectories, and incompatible alleles are able to segregate at much higher frequencies than single-locus expectations. Equilibria exist, and they can involve fixation of either autosomal or X-linked alleles. The exact equilibrium depends on whether synthetic alleles are dominant or recessive and whether fitness effects are seen in males, females, or both sexes. When single-locus fitness effects and synthetic incompatibilities are both present, population dynamics depend on the dominance of alleles and historical contingency (i.e., whether X-linked or autosomal mutations occur first). Recessive synthetic lethality can result in high-frequency X-linked alleles, and dominant synthetic lethality can result in high-frequency autosomal alleles. Many X-autosome incompatibilities in natural populations may be cryptic, appearing to be single-locus effects because one locus is fixed. We also discuss the implications of these findings with respect to standing genetic variation and the origins of Haldane's rule.

  20. Grid of the Future: Quantification of Benefits from Flexible Energy Resources in Scenarios With Extra-High Penetration of Renewable Energy

    Energy Technology Data Exchange (ETDEWEB)

    Bebic, Jovan [General Electric International, Inc., Schenectady, NY (United States). Energy Consulting; Hinkle, Gene [General Electric International, Inc., Schenectady, NY (United States). Energy Consulting; Matic, Slobodan [General Electric International, Inc., Schenectady, NY (United States). Energy Consulting; Schmitt, William [General Electric International, Inc., Schenectady, NY (United States). Energy Consulting

    2015-01-15

    The main objective of this study is to quantify the entitlement for system benefits attainable by pervasive application of flexible energy resources in scenarios with extra-high penetration of renewable energy. The quantified benefits include savings in thermal energy and reduction of CO2 emissions. Both are primarily a result of displacement of conventional thermal generation by renewable energy production, but there are secondary improvements that arise from lowering operating reserves, removing transmission constraints, and by partially removing energy-delivery losses due to energy production by distributed solar. The flexible energy resources in the context of this study include energy storage and adjustable loads. The flexibility of both was constrained to a time horizon of one day. In case of energy storage this means that the state of charge is restored to the starting value at the end of each day, while for load this means that the daily energy consumed is maintained constant. The extra-high penetration of renewable energy in the context of this study means the level of penetration resulting in significant number of hours where instantaneous power output from renewable resources added to the power output from baseload nuclear fleet surpasses the instantaneous power consumption by the load.

  1. Autosomal dominant arteriopathy with sub cortical infarcts and leucoencephalopathy (CADASIL)

    International Nuclear Information System (INIS)

    Ojeda, Adriana; Tiezzi, Gerardo; Uriarte, Ana M.; Eguren, Leonor

    2002-01-01

    Cerebral autosomal dominant arteriopathy with sub cortical infarcts and leucoencephalopathy (CASADIL) is a systemic hereditary, vascular disease that involves small arteries. Recurrent ischemia, pseudo bulbar paralysis and dementia are characteristic. Other manifestations include migraine and depression. We report an Argentine family with VI generations with evidence of disease in IV. MR examinations were performed on 21 family members (both symptomatic and asymptomatic). The main findings on MR on symptomatic and asymptomatic patients were small lesions with high signal on T2 localised in periventricular white matter, brain stem, basal ganglia and thalamus, and confluent patches on white matter although with high signal on T2 images, usually symmetric. In conclusion we can assess that diffuse myelin loss and small infarcts occurring in cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy well demonstrated with MR. In addition, some of the abnormalities in pre symptomatic patients can be identified on MR images. (author)

  2. Decitabine improves progression-free survival in older high-risk MDS patients with multiple autosomal monosomies: results of a subgroup analysis of the randomized phase III study 06011 of the EORTC Leukemia Cooperative Group and German MDS Study Group

    NARCIS (Netherlands)

    Lubbert, M.; Suciu, S.; Hagemeijer, A.; Ruter, B.; Platzbecker, U.; Giagounidis, A.; Selleslag, D.; Labar, B.; Germing, U.; Salih, H.R.; Muus, P.; Pfluger, K.H.; Schaefer, H.E.; Bogatyreva, L.; Aul, C.; Witte, T.J.M. de; Ganser, A.; Becker, H.; Huls, G.A.; Helm, L. van der; Vellenga, E.; Baron, F.; Marie, J.P.; Wijermans, P.W.; Group, E.L.; German, M.D.S.S.G. the

    2016-01-01

    In a study of elderly AML patients treated with the hypomethylating agent decitabine (DAC), we noted a surprisingly favorable outcome in the (usually very unfavorable) subgroup with two or more autosomal monosomies (MK2+) within a complex karyotype (Lubbert et al., Haematologica 97:393-401, 2012).

  3. Measurement and modeling of the low-temperature penetration-depth anomaly in high-quality MgB{sub 2} thin films

    Energy Technology Data Exchange (ETDEWEB)

    Agassi, Y.D. [Naval Surface Warfare Center, Carderock Division, Bethesda, MD 20817 (United States); Oates, D.E., E-mail: OATES@LL.MIT.EDU [MIT-Lincoln Laboratory, Lexington, MA 02420 (United States); Moeckly, B.H. [STI, Inc. Santa Barbara, CA 93111 (United States)

    2012-10-15

    Based on our measurements of intermodulation distortion in MgB{sub 2}, we have previously proposed that the {pi} energy-gap in MgB{sub 2} entails six nodal lines [Y.D. Agassi, D.E. Oates, and B.H. Moeckly, Phys. Rev. B 80 (2009) 174522]. Here we report high-precision measurements in MgB{sub 2} stripline resonators that show an increase of the penetration depth as the temperature is decreased below 5 K. This increase is consistent with the Script-Small-L = 6 symmetry of the {pi} energy gap that we have proposed. We interpret the increase as a manifestation of Andreev surface-attached states that are associated with the nodal lines of the {pi} energy gap. Penetration-depth calculations are in good agreement with our data. To reconcile the present interpretation with existing literature, we review other penetration-depth data, magnetic-impurity and tunneling experiments, and data on the paramagnetic Meissner effect. We conclude that these data do not rule out the interpretation of our experimental data based on a nodal {pi} energy gap.

  4. Frozen-thawed rhesus sperm retain normal morphology and highly progressive motility but exhibit sharply reduced efficiency in penetrating cervical mucus and hyualuronic acid gel

    Science.gov (United States)

    Tollner, Theodore L.; Dong, Qiaoxiang; VandeVoort, Catherine A.

    2011-01-01

    The preservation of the genetic diversity of captive populations of rhesus monkeys is critical to the future of biomedical research. Cryopreservation of rhesus macaque sperm is relatively simple to perform, yields high post-thaw motility, and theoretically, provides via artificial insemination (AI) a way to easily transfer genetics among colonies of animals. In the interest of optimizing semen cryopreservation methods for use with vaginal AI, we evaluated the ability of frozen-thawed rhesus sperm to penetrate periovulatory cervical mucus (CM). Motile sperm concentration of pre–freeze (“fresh”) and post-thawed (“thawed”) samples from 5 different males were normalized for both computer assisted sperm motion analysis and CM penetration experiments. Sperm samples were deposited into slide chambers containing CM or gel composed of hyaluronic acid (HA) as a surrogate for CM and numbers of sperm were recorded as they entered a video field a preset distance from the sperm suspension-CM (or HA) interface. Fresh and thawed sperm were dried on glass slides, “Pap”-stained, and assessed for changes in head dimensions and head and flagellar shape. While retaining better than 80% of fresh sperm progressive motility, thawed sperm from the same ejaculate retained on average only 18.6% of the CM penetration ability. Experiments using HA gel yielded similar results only with reduced experimental error and thus improved detection of treatment differences. Neither the percentage of abnormal forms nor head dimensions differed between fresh and thawed sperm. While findings suggests that sperm-CM interaction is a prominent factor in previous failures of vaginal AI with cryopreserved macaque sperm, neither sperm motility nor morphology appears to account for changes in the ability of cryopreserved sperm to penetrate CM. Our data points to a previously unidentified manifestation of cryodamage which may have implications for assessment of sperm function beyond the cervix and

  5. Lipid shell-enveloped polymeric nanoparticles with high integrity of lipid shells improve mucus penetration and interaction with cystic fibrosis-related bacterial biofilms

    DEFF Research Database (Denmark)

    Wan, Feng; Nylander, Tommy; Klodzinska, Sylvia Natalie

    2018-01-01

    , we describe facile methods to prepare Lipid@NPs with high integrity of lipid shells and demonstrate the potential of Lipid@NPs in effective mucus penetration and interaction with cystic fibrosis-related bacterial biofilms. Lipid shell-enveloped polystyrene NPs with high integrity of lipid shells (c...... mediated layer-by layer approach. Our results suggest that the integrity of the lipid envelopes is crucial for enabling the diffusion of Lipid@PSNPs into the mucus layer and promoting the interaction of Lipid@PSNPs with a bacterial biofilm....

  6. Genetic diagnosis of high-penetrance susceptibility for colorectal cancer (CRC) is achievable for a high proportion of familial CRC by exome sequencing.

    Science.gov (United States)

    Chubb, Daniel; Broderick, Peter; Frampton, Matthew; Kinnersley, Ben; Sherborne, Amy; Penegar, Steven; Lloyd, Amy; Ma, Yussanne P; Dobbins, Sara E; Houlston, Richard S

    2015-02-10

    Knowledge of the contribution of high-penetrance susceptibility to familial colorectal cancer (CRC) is relevant to the counseling, treatment, and surveillance of CRC patients and families. To quantify the impact of germline mutation to familial CRC, we sequenced the mismatch repair genes (MMR) APC, MUTYH, and SMAD4/BMPR1A in 626 early-onset familial CRC cases ascertained through a population-based United Kingdom national registry. In addition, we evaluated the contribution of mutations in the exonuclease domain (exodom) of POLE and POLD1 genes that have recently been reported to confer CRC risk. Overall mutations (pathogenic, likely pathogenic) in MMR genes make the highest contribution to familial CRC (10.9%). Mutations in the other established CRC genes account for 3.3% of cases. POLE/POLD1 exodom mutations were identified in three patients with family histories consistent with dominant transmission of CRC. Collectively, mutations in the known genes account for 14.2% of familial CRC (89 of 626 cases; 95% CI = 11.5, 17.2). A genetic diagnosis is feasible in a high proportion of familial CRC. Mainstreaming such analysis in clinical practice should enable the medical management of patients and their families to be optimized. Findings suggest CRC screening of POLE and POLD1 mutation carriers should be comparable to that afforded to those at risk of HNPCC. Although the risk of CRC associated with unexplained familial CRC is in general moderate, in some families the risk is substantive and likely to be the consequence of unidentified genes, as exemplified by POLE and POLD1. Our findings have utility in the design of genetic analyses to identify such novel CRC risk genes. © 2015 by American Society of Clinical Oncology.

  7. On the synergy between large electric vehicle fleet and high wind penetration – An analysis of the Danish case

    DEFF Research Database (Denmark)

    Krog Ekman, Claus

    2011-01-01

    Increasing the level of wind power penetration beyond the present level in the Danish power system implies large challenges when it comes to energy management and system stability. Plug-in electric vehicles promise to contribute to the flexibility of the energy system by creating a link between...... the power system and the transportation sector and provide the possibility to make use of the inherent energy storage of a large electric vehicle (EV) fleet. The present work investigates the effects of different EV charging strategies on the balance between wind power production and consumption in a future...... batteries are used as backup at times with little wind power production) will have very limited effects on the overall energy management and is more likely to be used only for regulation and reserve services, also in the longer perspective....

  8. Penetrating ureteral trauma

    Directory of Open Access Journals (Sweden)

    Gustavo P. Fraga

    2007-04-01

    Full Text Available OBJECTIVE: The purpose of this series is to report our experience in managing ureteral trauma, focusing on the importance of early diagnosis, correct treatment, and the impact of associated injuries on the management and morbid-mortality. MATERIALS AND METHODS: From January 1994 to December 2002, 1487 laparotomies for abdominal trauma were performed and 20 patients with ureteral lesions were identified, all of them secondary to penetrating injury. Medical charts were analyzed as well as information about trauma mechanisms, diagnostic routine, treatment and outcome. RESULTS: All patients were men. Mean age was 27 years. The mechanisms of injury were gunshot wounds in 18 cases (90% and stab wounds in two (10%. All penetrating abdominal injuries had primary indication of laparotomy, and neither excretory urography nor computed tomography were used in any case before surgery. The diagnosis of ureteric injury was made intra-operatively in 17 cases (85%. Two ureteral injuries (10% were initially missed. All patients had associated injuries. The treatment was dictated by the location, extension and time necessary to identify the injury. The overall incidence of complications was 55%. The presence of shock on admission, delayed diagnosis, Abdominal Trauma Index > 25, Injury Severity Score > 25 and colon injuries were associated to a high complication rate, however, there was no statistically significant difference. There were no mortalities in this group. CONCLUSIONS: A high index of suspicion is required for diagnosis of ureteral injuries. A thorough exploration of all retroperitoneal hematoma after penetrating trauma should be an accurate method of diagnosis; even though it failed in 10% of our cases.

  9. Autosomal recessive Charcot-Marie-Tooth neuropathy.

    Science.gov (United States)

    Espinós, Carmen; Calpena, Eduardo; Martínez-Rubio, Dolores; Lupo, Vincenzo

    2012-01-01

    Charcot-Marie-Tooth (CMT) disease, a hereditary motor and sensory neuropathy that comprises a complex group of more than 50 diseases, is the most common inherited neuropathy. CMT is generally divided into demyelinating forms, axonal forms and intermediate forms. CMT is also characterized by a wide genetic heterogeneity with 29 genes and more than 30 loci involved. The most common pattern of inheritance is autosomal dominant (AD), although autosomal recessive (AR) forms are more frequent in Mediterranean countries. In this chapter we give an overview of the associated genes, mechanisms and epidemiology of AR-CMT forms and their associated phenotypes.

  10. When Physics Meets Biology: Low and High Velocity Penetration, Blunt Trauma and Blast Injuries to the Brain

    Directory of Open Access Journals (Sweden)

    Leanne eYoung

    2015-05-01

    Full Text Available The incidence of TBI in the US has reached epidemic proportions with well over 2 million new cases reported each year. TBI can occur in both civilians and warfighters, with head injuries occurring in both combat and non-combat situations from a variety of threats, including ballistic penetration, acceleration, blunt impact, and blast. Most generally, TBI is a condition in which physical loads exceed the capacity of brain tissues to absorb without injury. More specifically, TBI results when sufficient external force is applied to the head and is subsequently converted into stresses that must be absorbed or redirected by protective equipment. If the stresses are not sufficiently absorbed or redirected, they will lead to damage of extracranial soft tissue and the skull. Complex interactions and kinematics of the head, neck and jaw cause strains within the brain tissue, resulting in structural, anatomical damage that is characteristic of the inciting insult. This mechanical trauma then initiates a neuro-chemical cascade that leads to the functional consequences of TBI, such as cognitive impairment. To fully understand the mechanisms by which TBI occurs, it is critically important to understand the effects of the loading environments created by these threats. In the following, a review is made of the pertinent complex loading conditions and how these loads cause injury. Also discussed are injury thresholds and gaps in knowledge, both of which are needed to design improved protective systems.

  11. Management of Extreme Ametropia after Penetrating Keratoplasty: A Series of Surgical Procedures for High Myopia and Astigmatism

    Directory of Open Access Journals (Sweden)

    Jorge E. Valdez-Garcia

    2014-08-01

    Full Text Available A series of surgical interventions - relaxing corneal incisions, intraocular lens, and intrastromal rings - were used to correct a case of extreme ametropia in a thin cornea after a penetrating keratoplasty in an 18-year-old patient who presented with a -10.25 -8.50 × 120 preoperative refraction and 20/200 best-corrected visual acuity (BCVA. After a series of surgical procedures, the patient's BCVA in his left eye improved to 20/30 with +0.50 -1.00 × 170, the slit lamp examination showed no significant findings, and the patient's visual complaints disappeared. At the 1-year follow-up, the BCVA was 20/25, without visual complaints. The process of individualizing the surgical procedure in the present case was employed in an outcome-based approach, that is, the next surgical procedure was defined after the surgery and postoperative evaluation. The patient did not present complications during the follow-up period of 2.5 years.

  12. Operational Simulation Tools and Long Term Strategic Planning for High Penetrations of PV in the Southeastern United States

    Energy Technology Data Exchange (ETDEWEB)

    Tuohy, Aidan [Electric Power Research Institute, Knoxville, TN (United States); Smith, Jeff [Electric Power Research Institute, Knoxville, TN (United States); Rylander, Matt [Electric Power Research Institute, Knoxville, TN (United States); Singhvi, Vikas [Electric Power Research Institute, Knoxville, TN (United States); Enbar, Nadav [Electric Power Research Institute, Knoxville, TN (United States); Coley, Steven [Electric Power Research Institute, Knoxville, TN (United States); Roark, Jeff [Electric Power Research Institute, Knoxville, TN (United States); Ela, Erik [Electric Power Research Institute, Knoxville, TN (United States); Lannoye, Eamonn [Electric Power Research Institute, Knoxville, TN (United States); Pilbrick, Charles Russ [Electric Power Research Institute, Knoxville, TN (United States); Rudkevich, Alex [Electric Power Research Institute, Knoxville, TN (United States); Hansen, Cliff [Electric Power Research Institute, Knoxville, TN (United States)

    2016-07-11

    Increasing levels of distributed and utility scale Solar Photovoltaics (PV) will have an impact on many utility functions, including distribution system operations, bulk system performance, business models and scheduling of generation. In this project, EPRI worked with Southern Company Services and its affiliates and the Tennessee Valley Authority to assist these utilities in their strategic planning efforts for integrating PV, based on modeling, simulation and analysis using a set of innovative tools. Advanced production simulation models were used to investigate operating reserve requirements. To leverage existing work and datasets, this last task was carried out on the California system. Overall, the project resulted in providing useful information to both of the utilities involved and through the final reports and interactions during the project. The results from this project can be used to inform the industry about new and improved methodologies for understanding solar PV penetration, and will influence ongoing and future research. This report summarizes each of the topics investigated over the 2.5-year project period.

  13. Quasi-static time-series simulation using OpenDSS in IEEE distribution feeder model with high PV penetration and its impact on solar forecasting

    Science.gov (United States)

    Mohammed, Touseef Ahmed Faisal

    Since 2000, renewable electricity installations in the United States (excluding hydropower) have more than tripled. Renewable electricity has grown at a compounded annual average of nearly 14% per year from 2000-2010. Wind, Concentrated Solar Power (CSP) and solar Photo Voltaic (PV) are the fastest growing renewable energy sectors. In 2010 in the U.S., solar PV grew over 71% and CSP grew by 18% from the previous year. Globally renewable electricity installations have more than quadrupled from 2000-2010. Solar PV generation grew by a factor of more than 28 between 2000 and 2010. The amount of CSP and solar PV installations are increasing on the distribution grid. These PV installations transmit electrical current from the load centers to the generating stations. But the transmission and distribution grid have been designed for uni-directional flow of electrical energy from generating stations to load centers. This causes imbalances in voltage and switchgear of the electrical circuitry. With the continuous rise in PV installations, analysis of voltage profile and penetration levels remain an active area of research. Standard distributed photovoltaic (PV) generators represented in simulation studies do not reflect the exact location and variability properties such as distance between interconnection points to substations, voltage regulators, solar irradiance and other environmental factors. Quasi-Static simulations assist in peak load planning hour and day ahead as it gives a time sequence analysis to help in generation allocation. Simulation models can be daily, hourly or yearly depending on duty cycle and dynamics of the system. High penetration of PV into the power grid changes the voltage profile and power flow dynamically in the distribution circuits due to the inherent variability of PV. There are a number of modeling and simulations tools available for the study of such high penetration PV scenarios. This thesis will specifically utilize OpenDSS, a open source

  14. Regional PV power estimation and forecast to mitigate the impact of high photovoltaic penetration on electric grid.

    Science.gov (United States)

    Pierro, Marco; De Felice, Matteo; Maggioni, Enrico; Moser, David; Perotto, Alessandro; Spada, Francesco; Cornaro, Cristina

    2017-04-01

    The growing photovoltaic generation results in a stochastic variability of the electric demand that could compromise the stability of the grid and increase the amount of energy reserve and the energy imbalance cost. On regional scale, solar power estimation and forecast is becoming essential for Distribution System Operators, Transmission System Operator, energy traders, and aggregators of generation. Indeed the estimation of regional PV power can be used for PV power supervision and real time control of residual load. Mid-term PV power forecast can be employed for transmission scheduling to reduce energy imbalance and related cost of penalties, residual load tracking, trading optimization, secondary energy reserve assessment. In this context, a new upscaling method was developed and used for estimation and mid-term forecast of the photovoltaic distributed generation in a small area in the north of Italy under the control of a local DSO. The method was based on spatial clustering of the PV fleet and neural networks models that input satellite or numerical weather prediction data (centered on cluster centroids) to estimate or predict the regional solar generation. It requires a low computational effort and very few input information should be provided by users. The power estimation model achieved a RMSE of 3% of installed capacity. Intra-day forecast (from 1 to 4 hours) obtained a RMSE of 5% - 7% while the one and two days forecast achieve to a RMSE of 7% and 7.5%. A model to estimate the forecast error and the prediction intervals was also developed. The photovoltaic production in the considered region provided the 6.9% of the electric consumption in 2015. Since the PV penetration is very similar to the one observed at national level (7.9%), this is a good case study to analyse the impact of PV generation on the electric grid and the effects of PV power forecast on transmission scheduling and on secondary reserve estimation. It appears that, already with 7% of PV

  15. A curious fact: Photic sneeze reflex. Autosomical dominant compelling helio-ophthalmic outburst syndrome.

    Science.gov (United States)

    Sevillano, C; Parafita-Fernández, A; Rodriguez-Lopez, V; Sampil, M; Moraña, N; Viso, E; Cores, F J

    2016-07-01

    To assess ocular involvement in the pathophysiology of autosomal dominant compelling helio-ophthalmic outburst syndrome (ACHOOs). An interview was conducted with a Caucasian family that showed clinical features of ACHOOs. Twelve of them had photic reflex and were recruited. A complete eye evaluation was made. A dominant autosomal inheritance with mild penetrance was demonstrated, with 67% of the studied subjects showing some degree of prominent corneal nerves. No other eye changes were found. Prominent corneal nerves may be associated with ACHOOs. The other eye structures studied do not seem to play a role in ACHOOs. Further studies are needed to understand the physiology of the ACHOOs. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  16. Evaluation of the effect of media velocity on filter efficiency and most penetrating particle size of nuclear grade high-efficiency particulate air filters.

    Science.gov (United States)

    Alderman, Steven L; Parsons, Michael S; Hogancamp, Kristina U; Waggoner, Charles A

    2008-11-01

    High-efficiency particulate air (HEPA) filters are widely used to control particulate matter emissions from processes that involve management or treatment of radioactive materials. Section FC of the American Society of Mechanical Engineers AG-1 Code on Nuclear Air and Gas Treatment currently restricts media velocity to a maximum of 2.5 cm/sec in any application where this standard is invoked. There is some desire to eliminate or increase this media velocity limit. A concern is that increasing media velocity will result in higher emissions of ultrafine particles; thus, it is unlikely that higher media velocities will be allowed without data to demonstrate the effect of media velocity on removal of ultrafine particles. In this study, the performance of nuclear grade HEPA filters, with respect to filter efficiency and most penetrating particle size, was evaluated as a function of media velocity. Deep-pleat nuclear grade HEPA filters (31 cm x 31 cm x 29 cm) were evaluated at media velocities ranging from 2.0 to 4.5 cm/sec using a potassium chloride aerosol challenge having a particle size distribution centered near the HEPA filter most penetrating particle size. Filters were challenged under two distinct mass loading rate regimes through the use of or exclusion of a 3 microm aerodynamic diameter cut point cyclone. Filter efficiency and most penetrating particle size measurements were made throughout the duration of filter testing. Filter efficiency measured at the onset of aerosol challenge was noted to decrease with increasing media velocity, with values ranging from 99.999 to 99.977%. The filter most penetrating particle size recorded at the onset of testing was noted to decrease slightly as media velocity was increased and was typically in the range of 110-130 nm. Although additional testing is needed, these findings indicate that filters operating at media velocities up to 4.5 cm/sec will meet or exceed current filter efficiency requirements. Additionally

  17. Influence of jet thrust on penetrator penetration when studying the structure of space object blanket

    Directory of Open Access Journals (Sweden)

    N. A. Fedorova

    2014-01-01

    Full Text Available The article presents the calculation-and-theory-based research results to examine the possibility for using the jet thrust impulse to increase a penetration depth of high-velocity penetrator modules. Such devices can be used for studies of Earth surface layer composition, and in the nearest future for other Solar system bodies too. Research equipment (sensors and different instruments is housed inside a metal body of the penetrator with a sharpened nose that decreases drag force in soil. It was assumed, that this penetrator is additionally equipped with the pulse jet engine, which is fired at a certain stage of penetrator motion into target.The penetrator is considered as a rigid body of variable mass, which is subjected to drag force and reactive force applied at the moment the engine fires. A drag force was represented with a binomial empirical law, and penetrator nose part was considered to be conical. The jet thrust force was supposed to be constant during its application time. It was in accordance with assumption that mass flow and flow rate of solid propellant combustion products were constant. The amount of propellant in the penetrator was characterized by Tsiolkovsky number Z, which specifies the ratio between the fuel mass and the penetrator structure mass with no fuel.The system of equations to describe the penetrator dynamics was given in dimensionless form using the values aligned with penetration of an equivalent inert penetrator as the time and penetration depth scales. Penetration dynamics of penetrator represented in this form allowed to eliminate the influence of penetrator initial mass and its cross-section diameter on the solution results. The lack of such dependency is convenient for comparing the calculation results since they hold for penetrators of various initial masses and cross-sections.To calculate the penetration a lunar regolith was taken as a soil material. Calculations were carried out for initial velocities of

  18. Autosomal dominant spastic paraplegia with peripheral neuropathy maps to chr12q23-24.

    Science.gov (United States)

    Schüle, R; Bonin, M; Dürr, A; Forlani, S; Sperfeld, A D; Klimpe, S; Mueller, J C; Seibel, A; van de Warrenburg, B P; Bauer, P; Schöls, L

    2009-06-02

    Hereditary spastic paraplegias (HSP) are genetically exceedingly heterogeneous. To date, 37 genetic loci for HSP have been described (SPG1-41), among them 16 loci for autosomal dominant disease. Notwithstanding, further genetic heterogeneity is to be expected in HSP, as various HSP families do not link to any of the known HSP loci. In this study, we aimed to map the disease locus in a German family segregating autosomal dominant complicated HSP. A genome-wide linkage analysis was performed using the GeneChip Mapping 10Kv2.0 Xba Array containing 10,204 SNP markers. Suggestive loci were further analyzed by mapping of microsatellite markers. One locus on chromosome 12q23-24, termed SPG36, was confirmed by high density microsatellite fine mapping with a significant LOD score of 3.2. SPG36 is flanked by markers D12S318 and D12S79. Linkage to SPG36 was excluded in >20 additional autosomal dominant HSP families. Candidate genes were selected and sequenced. No disease-causing mutations were identified in the coding regions of ATXN2, HSPB8, IFT81, Myo1H, UBE3B, and VPS29. SPG36 is complicated by a sensory and motor neuropathy; it is therefore the eighth autosomal dominant subtype of complicated HSP. We report mapping of a new locus for autosomal dominant hereditary spastic paraplegia (HSP) (SPG36) on chromosome 12q23-24 in a German family with autosomal dominant HSP complicated by peripheral neuropathy.

  19. MO-FG-CAMPUS-IeP2-05: Feasibility Demonstration of High-Voltage Clinical CT and Impact On X-Ray Penetration Through Metal Objects

    International Nuclear Information System (INIS)

    Jin, Y; De Man, B; Robinson, V; Gjesteby, L; Wang, G; Verburg, J; Giantsoudi, D; Paganetti, H

    2016-01-01

    Purpose: To demonstrate the possibility and quantify the impact of operating a clinical CT scanner at exceptionally high x-ray tube voltage for better penetration through metal objects and facilitating metal artifact reduction. Methods: We categorize metal objects according to the data corruption severeness (level of distortion and complete photon starvation fraction). To demonstrate feasibility and investigate the impact of high voltage scanning we modified a commercial GE LightSpeed VCT scanner (generator and software) to enable CT scans with x-ray tube voltages as high as 175 kVp. A 20 cm diameter water phantom with two metal rods (10 mm stainless and 25 mm titanium) and a water phantom with realistic metal object (spine cage) were used to evaluate the data corruption and image artifacts in the absence of any algorithm correction. We also performed simulations to confirm our understanding of the transmitted photon levels through metal objects with different size and composition. Results: The reconstructed images at 175 kVp still have significant dark shading artifacts, as expected since no special scatter correction or beam hardening was performed but show substantially lower noise and photon starvation than at lower kVp due to better beam penetration. Analysis of the raw data shows that the photon starved data is reduced from over 4% at 140 kVp to below 0.2% at 175 kVp. The simulations indicate that for clinically relevant titanium and stainless objects a 175 kVp tube voltage effectively avoids photon starvation. Conclusion: The use of exceptionally high tube voltage on a clinical CT system is a practical and effective solution to avoid photon starvation caused by certain metal implants. Sparse and hybrid high-voltage protocols are being considered to maintain low patient dose. This opens the door to algorithmic physics-based corrections rather than treating the data as missing and relying on missing data algorithms. Some of the authors are employees of General

  20. MO-FG-CAMPUS-IeP2-05: Feasibility Demonstration of High-Voltage Clinical CT and Impact On X-Ray Penetration Through Metal Objects

    Energy Technology Data Exchange (ETDEWEB)

    Jin, Y; De Man, B [GE Global Research, Niskayuna, NY (United States); Robinson, V [GE, Global Research, Niskayuna, NY (United States); Gjesteby, L [Rensselaer Polytechnic Institute, Troy, NY (United States); Wang, G [Rensselaer Polytechnic Institute Troy, NY (United States); Verburg, J [Massachusetts, General Hospital, Boston, MA (United States); Giantsoudi, D; Paganetti, H [Massachusetts General Hospital, Boston, MA (United States)

    2016-06-15

    Purpose: To demonstrate the possibility and quantify the impact of operating a clinical CT scanner at exceptionally high x-ray tube voltage for better penetration through metal objects and facilitating metal artifact reduction. Methods: We categorize metal objects according to the data corruption severeness (level of distortion and complete photon starvation fraction). To demonstrate feasibility and investigate the impact of high voltage scanning we modified a commercial GE LightSpeed VCT scanner (generator and software) to enable CT scans with x-ray tube voltages as high as 175 kVp. A 20 cm diameter water phantom with two metal rods (10 mm stainless and 25 mm titanium) and a water phantom with realistic metal object (spine cage) were used to evaluate the data corruption and image artifacts in the absence of any algorithm correction. We also performed simulations to confirm our understanding of the transmitted photon levels through metal objects with different size and composition. Results: The reconstructed images at 175 kVp still have significant dark shading artifacts, as expected since no special scatter correction or beam hardening was performed but show substantially lower noise and photon starvation than at lower kVp due to better beam penetration. Analysis of the raw data shows that the photon starved data is reduced from over 4% at 140 kVp to below 0.2% at 175 kVp. The simulations indicate that for clinically relevant titanium and stainless objects a 175 kVp tube voltage effectively avoids photon starvation. Conclusion: The use of exceptionally high tube voltage on a clinical CT system is a practical and effective solution to avoid photon starvation caused by certain metal implants. Sparse and hybrid high-voltage protocols are being considered to maintain low patient dose. This opens the door to algorithmic physics-based corrections rather than treating the data as missing and relying on missing data algorithms. Some of the authors are employees of General

  1. [National and regional market penetration rates of generic's high dosage buprenorphine: its evolution from 2006 to 2008, using reimbursed drug database].

    Science.gov (United States)

    Boczek, Christelle; Frauger, Elisabeth; Micallef, Joëlle; Allaria-Lapierre, Véronique; Reggio, Patrick; Sciortino, Vincent

    2012-01-01

    To assess the national market penetration rate (PR) of generic high-dosage buprenorphine (HDB) in 2008 and its evolution since their marketing (2006), and making a point for each dosage and at regional level. Retrospective study over data using national and regional health reimbursement database over three years (2006-2008). In 2008, the generic HDB's national MPR was 31%. The PR for each dosage were 45% for 0.4 mg, 36% for 2 mg and 19% for 8 mg. The (PR) based on Defined Daily Dose (DDD) was 23% in 2008, 15% in 2007 and 4% in 2006. In 2008, at the regional level, disparities were observed in the adjusted penetration rate from 15% in Île de France to 39% in Champagne Ardennes Lorraine. The national PR of generic HDB has increased. There are differences in MPR in terms of dosage and area. However, this PR is still low (in 2008, 82% of the delivered drugs are generics). © 2012 Société Française de Pharmacologie et de Thérapeutique.

  2. A review of penetration mechanisms and dynamic properties of tungsten and depleted uranium penetrators

    International Nuclear Information System (INIS)

    Andrew, S.P.; Caligiuri, R.D.; Eiselstein, L.E.

    1991-01-01

    Kinetic energy penetrators must posses the best possible combination of hardness, stiffness, strength, and fracture toughness characteristics to be effective against modern armor systems. Over the last decade, depleted uranium (DU) and tungsten alloys have been the materials of choice for kinetic energy penetrators. Du and tungsten perform abut the same against semi-infinite targets, and DU outperforms tungsten penetrators in oblique, spaced array targets, but because of environmental and subsequent cost concerns, effort has focused on improving the performance of tungsten penetrators over the last few years. However, despite recent improvements in material properties, the penetration performance of tungsten still lags behind that of DU. One possible reason is the difference in deformation mechanisms at the leading edge of the penetrator during the penetration process-DU alloys tend to shear band and sharpen as they penetrate the target material, whereas tungsten penetrators tend to mushroom and blunt. As a first step to determine whether shear banding is truly the reason for superior DU performance, a review of the fabrication, high strain-rate properties, and penetration phenomena of penetrators manufactured from both tungsten and DU alloys. Specifically, the effects of composition, processing, and heat treatment on material properties and penetration mechanisms of these alloys are discussed

  3. Penetrating Osseous Spicules Causing High-Flow Ventral CSF Leaks in the Setting of Relatively Low BMI : A Preliminary Study.

    Science.gov (United States)

    Rosebrock, Richard E; Diehn, Felix E; Luetmer, Patrick H; Wald, John T; Lane, John I; Morris, Jonathan M; Lehman, Vance T; Carr, Carrie M; Mokri, Bahram; Thielen, Kent R

    2017-05-16

    We have anecdotally observed patients with high-flow ventral cerebrospinal fluid (CSF) leaks resulting from penetrating osseous spicules or calcified discs to be relatively thin. The purpose of this study was to explore the validity of this observation and determine if a potential association exists between low body mass index (BMI) and high-flow spinal ventral CSF leaks resulting from such dura-penetrating lesions. Sixteen consecutive patients with precisely localized high-flow ventral spinal CSF leaks on dynamic myelography were identified. The cause of the CSF leak was determined. The BMI on the date nearest to and within 2 weeks of myelography was recorded. Utilizing exact sign test, the body mass index was compared to the average BMI from the National Health and Nutrition Examination Survey (Centers for Disease Control), matched to sex and age-range. The cohort consisted of 10 males (63%) and 6 females with a mean age of 54 years (range 37-72 years). In all patients, a spiculated osteophyte/calcified disc was identified at the site of the leak. Fourteen patients (88%) had a BMI below the matched national average, while only two patients (13%) had values above the national average (p = 0.004). Patients with high-flow ventral CSF leaks resulting from spiculated osteophyte or calcified disc as identified by dynamic myelography are more likely to have a BMI below the U.S. national average, matched for gender and age-range. This exploratory analysis requires confirmation as well as further characterization of potential pathophysiologic mechanisms and impact on radiographic and clinical assessments.

  4. Renewable Energy and Efficiency Modeling Analysis Partnership: An Analysis of How Different Energy Models Addressed a Common High Renewable Energy Penetration Scenario in 2025

    Energy Technology Data Exchange (ETDEWEB)

    Blair, N.; Jenkin, T.; Milford, J.; Short, W.; Sullivan, P.; Evans, D.; Lieberman, E.; Goldstein, G.; Wright, E.; Jayaraman, K.; Venkatech, B.; Kleiman, G.; Namovicz, C.; Smith, B.; Palmer, K.; Wiser, R.; Wood, F.

    2009-09-30

    and/or different answers in response to a set of focused energy-related questions. The focus was on understanding reasons for model differences, not on policy implications, even though a policy of high renewable penetration was used for the analysis. A group process was used to identify the potential question (or questions) to be addressed through the project. In late 2006, increasing renewable energy penetration in the electricity sector was chosen from among several options as the general policy to model. From this framework, the analysts chose a renewable portfolio standard (RPS) as the way to implement the required renewable energy market penetration in the models. An RPS was chosen because it was (i) of interest and represented the group's consensus choice, and (ii) tractable and not too burdensome for the modelers. Because the modelers and analysts were largely using their own resources, it was important to consider the degree of effort required. In fact, several of the modelers who started this process had to discontinue participation because of other demands on their time. Federal and state RPS policy is an area of active political interest and debate. Recognizing this, participants used this exercise to gain insight into energy model structure and performance. The results are not intended to provide any particular insight into policy design or be used for policy advocacy, and participants are not expected to form a policy stance based on the outcomes of the modeling. The goals of this REMAP project - in terms of the main topic of renewable penetration - were to: (1) Compare models and understand why they may give different results to the same question, (2) Improve the rigor and consistency of assumptions used across models, and (3) Evaluate the ability of models to measure the impacts of high renewable-penetration scenarios.

  5. Customer-economics of residential photovoltaic systems (Part 1): The impact of high renewable energy penetrations on electricity bill savings with net metering

    International Nuclear Information System (INIS)

    Darghouth, Naïm R.; Barbose, Galen; Wiser, Ryan H.

    2014-01-01

    Residential photovoltaic (PV) systems in the US are often compensated at the customer's underlying retail electricity rate through net metering. Given the uncertainty in future retail rates and the inherent links between rates and the customer–economics of behind-the-meter PV, there is growing interest in understanding how potential changes in rates may impact the value of bill savings from PV. In this article, we first use a production cost and capacity expansion model to project California hourly wholesale electricity market prices under two potential electricity market scenarios, including a reference and a 33% renewables scenario. Second, based on the wholesale electricity market prices generated by the model, we develop retail rates (i.e., flat, time-of-use, and real-time pricing) for each future scenario based on standard retail rate design principles. Finally, based on these retail rates, the bill savings from PV is estimated for 226 California residential customers under two types of net metering, for each scenario. We find that high renewable penetrations can drive substantial changes in residential retail rates and that these changes, together with variations in retail rate structures and PV compensation mechanisms, interact to place substantial uncertainty on the future value of bill savings from residential PV. - Highlights: • We investigate the impact of high renewables on customer economics of solar. • We model three types of residential retail electricity rates. • Based on the rates, we calculate the bill savings from photovoltaic (PV) generation. • High renewables penetration can lead to lower bill savings with time-varying rates. • There is substantial uncertainty in the future bill savings from residential PV

  6. Penetrating abdominal trauma.

    Science.gov (United States)

    Henneman, P L

    1989-08-01

    The management of patients with penetrating abdominal trauma is outlined in Figure 1. Patients with hemodynamic instability, evisceration, significant gastrointestinal bleeding, peritoneal signs, gunshot wounds with peritoneal violation, and type 2 and 3 shotgun wounds should undergo emergency laparotomy. The initial ED management of these patients includes airway management, monitoring of cardiac rhythm and vital signs, history, physical examination, and placement of intravenous lines. Blood should be obtained for initial hematocrit, type and cross-matching, electrolytes, and an alcohol level or drug screen as needed. Initial resuscitation should utilize crystalloid fluid replacement. If more than 2 liters of crystalloid are needed to stabilize an adult (less in a child), blood should be given. Group O Rh-negative packed red blood cells should be immediately available for a patient in impending arrest or massive hemorrhage. Type-specific blood should be available within 15 minutes. A patient with penetrating thoracic and high abdominal trauma should receive a portable chest x-ray, and a hemo- or pneumothorax should be treated with tube thoracostomy. An unstable patient with clinical signs consistent with a pneumothorax, however, should receive a tube thoracostomy prior to obtaining roentgenographic confirmation. If time permits, a nasogastric tube and Foley catheter should be placed, and the urine evaluated for blood (these procedures can be performed in the operating room). If kidney involvement is suspected because of hematuria or penetrating trauma in the area of a kidney or ureter in a patient requiring surgery, a single-shot IVP should be performed either in the ED or the operating room. An ECG is important in patients with possible cardiac involvement and in patients over the age of 40 going to the operating room. Tetanus status should be updated, and appropriate antibiotics covering bowel flora should be given. Operative management should rarely be delayed

  7. Optimal placement, sizing, and daily charge/discharge of battery energy storage in low voltage distribution network with high photovoltaic penetration

    DEFF Research Database (Denmark)

    Jannesar, Mohammad Rasol; Sedighi, Alireza; Savaghebi, Mehdi

    2018-01-01

    when photovoltaic penetration is increased in low voltage distribution network. Local battery energy storage system can mitigate these disadvantages and as a result, improve the system operation. For this purpose, battery energy storage system is charged when production of photovoltaic is more than...... consumers’ demands and discharged when consumers’ demands are increased. Since the price of battery energy storage system is high, economic, environmental, and technical objectives should be considered together for its placement and sizing. In this paper, optimal placement, sizing, and daily (24 h) charge......Proper installation of rooftop photovoltaic generation in distribution networks can improve voltage profile, reduce energy losses, and enhance the reliability. But, on the other hand, some problems regarding harmonic distortion, voltage magnitude, reverse power flow, and energy losses can arise...

  8. Comparison of high speed movie and flash x-ray measurement of the translational and rotational motions of projectiles penetrating gelatin

    International Nuclear Information System (INIS)

    Roecker, E.T.

    1979-01-01

    Projectiles penetrating a gelatin block were simultaneously measured by a high speed movie camera, Dynafax, and by a sequential, orthogonal, flash x-ray system. The eight orthogonal views of the x-ray system provided position and orientation of the projectiles vs. time. From onset of tumble in the gelatin, owing to gyroscopic instability, the growth of yaw was the same for each round in a replicated set. This phenomenon provided a legitimate procedure for pooling the x-ray data, giving well determined curves of velocity decay and yaw growth. The movie camera observed the progress of the cavity formed by the projectile. The resulting velocity decay of the cavity tip was compared to that of the projectile as measured by the x-ray technique. (author)

  9. Impact of High Levels of Wind Penetration on the Exercise of Market Power in the Multi-Area Systems

    NARCIS (Netherlands)

    Moiseeva, E.

    2017-01-01

    New European energy policies have set a goal of a high share of renewable energy in electricity markets. In the presence of high levels of renewable generation, and especially wind, there is more uncertainty in the supply. It is natural, that volatility in energy production induces the volatility in

  10. Autosomal dominant cyclic hematopoiesis: Genetics, phenotype, and natural history

    Energy Technology Data Exchange (ETDEWEB)

    Palmer, S.E.; Stephens, K.; Dale, D.C. [Univ. of Washington, Seattle, WA (United States)

    1994-09-01

    Autosomal dominant cyclic hematopoiesis (ADCH; cyclic neutropenia) is a rare disorder manifested by transient neutropenia that recurs every three weeks. To facilitate mapping the ADCH gene by genetic linkage analysis, we studied 9 ADCH families with 42 affected individuals. Pedigrees revealed AD inheritance with no evidence for decreased penetrance. Similar intra- and interfamilial variable expression was observed, with no evidence to support heterogeneity. At least 3 families displayed apparent new mutations. Many adults developed chronic neutropenia, while offspring always cycled during childhood. Children displayed recurrent oral ulcers, gingivitis, lymphadenopathy, fever, and skin and other infections with additional symptoms. Interestingly, there were no cases of neonatal infection. Some children required multiple hospitalizations for treatment. Four males under age 18 died of Clostridium sepsis following necrotizing enterocolitis; all had affected mothers. No other deaths due to ADCH were found; most had improvement of symptoms and infections as adults. Adults experienced increased tooth loss prior to age 30 (16 out of 27 adults, with 9 edentulous). No increase in myelodysplasia, malignancy, or congenital anomalies was observed. Recombinant G-CSF treatment resulted in dramatic improvement of symptoms and infections. The results suggest that ADCH is not a benign disorder, especially in childhood, and abdominal pain requires immediate evaluation. Diagnosis of ADCH requires serial blood counts in the proband and at least one CBC in relatives to exclude similar disorders. Genetic counseling requires specific histories as well as CBCs of each family member at risk to determine status regardless of symptom history, especially to assess apparent new mutations.

  11. Development of an Extraterrestrial Organic Analyzer (EOA) for Highly Sensitive Organic Detection on an Ice Shell Impact Penetrator (IceShIP)

    Science.gov (United States)

    Stockton, A. M.; Duca, Z. A.; Cato, M.; Cantrell, T.; Kim, J.; Putman, P.; Schmidt, B. E.

    2016-12-01

    Kinetic penetrators have the potential to enable low cost in situ measurements of the ice of worlds including Europa and Enceladus [1]. Their small size and mass, critical to limiting their kinetic energy, makes them ideal small landers riding on primarily orbiter missions, while enabling sampling at several m depth due to burial and excavation. In situ microfluidic-based organic analysis systems are a powerful, miniaturized approach for detecting markers of habitability and recent biological activity. Development of microfluidic technology, like that of the Mars Organic Analyzer (MOA) [2,3] and Enceladus Organic Analyzer (EOA), has led to an instrument capable of in situ organic chemical analysis compatible with a kinetic penetrator platform. This technology uses an integrated microfluidic processor to prepare samples for analysis via fluorescent derivatization prior to highly sensitive laser-induced fluorescence (LIF) detection. Selective derivatization in the presence of a chiral selector enables distinction between amino acid enantiomers. Finite element analysis of the core microfluidic processing and analytical device indicated that the device itself is more than capable of surviving the stresses associated with an impact acceleration of >50,000g. However, a number of developments were still required to enable a flight-ready system. Preliminary experiments indicated that moving from a pneumatically-actuated to a hydraulically-actuated microvalve system may provide better impact resistance. A hydraulically-actuated microvalve system was developed and tested. A modification of an established microfabricated LIF detection system would use indium bump bonding to permanently weld optical components using standard microfabrication techniques with perfect alignment. Recent work has also focused on developing and characterizing impact-resistant electronics. This work shows the low-TRL development of EOA's LIF and microfluidic subsystems for future planetary impact

  12. In-place HEPA filter penetration test

    International Nuclear Information System (INIS)

    Bergman, W.; Wilson, K.; Elliott, J.; Bettencourt, B.; Slawski, J.W.

    1997-01-01

    We have demonstrated the feasibility of conducting penetration tests on high efficiency particulate air (HEPA) filters as installed in nuclear ventilation systems. The in-place penetration test, which is designed to yield equivalent penetration measurements as the standard DOP efficiency test, is based on measuring the aerosol penetration of the filter installation as a function of particle size using a portable laser particle counter. This in-place penetration test is compared to the current in-place leak test using light scattering photometers for single HEPA filter installations and for HEPA filter plenums using the shroud method. Test results show the in-place penetration test is more sensitive than the in-place leak test, has a similar operating procedure, but takes longer to conduct. Additional tests are required to confirm that the in-place penetration test yields identical results as the standard dioctyl phthalate (DOP) penetration test for HEPA filters with controlled leaks in the filter and gasket and duct by-pass leaks. Further development of the procedure is also required to reduce the test time before the in- place penetration test is practical

  13. In-place HEPA filter penetration test

    Energy Technology Data Exchange (ETDEWEB)

    Bergman, W.; Wilson, K.; Elliott, J. [Lawrence Livermore National Lab., CA (United States)] [and others

    1997-08-01

    We have demonstrated the feasibility of conducting penetration tests on high efficiency particulate air (HEPA) filters as installed in nuclear ventilation systems. The in-place penetration test, which is designed to yield equivalent penetration measurements as the standard DOP efficiency test, is based on measuring the aerosol penetration of the filter installation as a function of particle size using a portable laser particle counter. This in-place penetration test is compared to the current in-place leak test using light scattering photometers for single HEPA filter installations and for HEPA filter plenums using the shroud method. Test results show the in-place penetration test is more sensitive than the in-place leak test, has a similar operating procedure, but takes longer to conduct. Additional tests are required to confirm that the in-place penetration test yields identical results as the standard dioctyl phthalate (DOP) penetration test for HEPA filters with controlled leaks in the filter and gasket and duct by-pass leaks. Further development of the procedure is also required to reduce the test time before the in-place penetration test is practical. 14 refs., 14 figs., 3 tabs.

  14. Ballistic-type field penetration into metals illustrated by high- and low-frequency size-effect measurements in silver

    DEFF Research Database (Denmark)

    Gantmakher, V. F.; Lebech, Jens; Bak, Christen Kjeldahl

    1979-01-01

    Radio-frequency size-effect experiments were performed on silver plane-parallel plates at high, 45 GHz, and low, 3 MHz, frequencies. By investigation of size-effect structures we show the influence of frequency on the field distribution inside the metal. When the frequency increases, the splash...

  15. Single-point reactive power control method on voltage rise mitigation in residential networks with high PV penetration

    DEFF Research Database (Denmark)

    Hasheminamin, Maryam; Agelidis, Vassilios; Ahmadi, Abdollah

    2018-01-01

    Voltage rise (VR) due to reverse power flow is an important obstacle for high integration of Photovoltaic (PV) into residential networks. This paper introduces and elaborates a novel methodology of an index-based single-point-reactive power-control (SPRPC) methodology to mitigate voltage rise by ...... system with high r/x ratio. Efficacy, effectiveness and cost study of SPRPC is compared to droop control to evaluate its advantages.......Voltage rise (VR) due to reverse power flow is an important obstacle for high integration of Photovoltaic (PV) into residential networks. This paper introduces and elaborates a novel methodology of an index-based single-point-reactive power-control (SPRPC) methodology to mitigate voltage rise...... by absorbing adequate reactive power from one selected point. The proposed index utilizes short circuit analysis to select the best point to apply this Volt/Var control method. SPRPC is supported technically and financially by distribution network operator that makes it cost effective, simple and efficient...

  16. Metasploit penetration testing cookbook

    CERN Document Server

    Agarwal, Monika

    2013-01-01

    This book follows a Cookbook style with recipes explaining the steps for penetration testing with WLAN, VOIP, and even cloud computing. There is plenty of code and commands used to make your learning curve easy and quick.This book targets both professional penetration testers as well as new users of Metasploit, who wish to gain expertise over the framework and learn an additional skill of penetration testing, not limited to a particular OS. The book requires basic knowledge of scanning, exploitation, and the Ruby language.

  17. Geometry of power flows and convex-relaxed power flows in distribution networks with high penetration of renewables

    DEFF Research Database (Denmark)

    Huang, Shaojun; Wu, Qiuwei; Zhao, Haoran

    2016-01-01

    Renewable energies are increasingly integrated in electric distribution networks and will cause severe overvoltage issues. Smart grid technologies make it possible to use coordinated control to mitigate the overvoltage issues and the optimal power flow (OPF) method is proven to be efficient...... in the applications such as curtailment management and reactive power control. Nonconvex nature of the OPF makes it difficult to solve and convex relaxation is a promising method to solve the OPF very efficiently. This paper investigates the geometry of the power flows and the convex-relaxed power flows when high...

  18. Investigations into the penetration and pressure drop of HEPA filter media during loading with submicron particle aerosols at high concentrations

    International Nuclear Information System (INIS)

    Leibold, H; Wilhelm, J.G.

    1991-01-01

    High Efficiency Particulate Air (HEPA) filters are typically employed in particle removal and retention within the air cleaning systems of clean rooms in the pharmaceutical, nuclear and semiconductor industries for dust concentrations of some μg/m 3 . Their extremely high removal efficiencies for submicron particles make them attractive candidates in complying with increasingly lower emission limits for industrial processes that involve dust concentrations of up to several g/m 3 . Cost-effective operation under such conditions requires the filter units to be recleanable. The recleanability of HEPA filter media depends not only on the operating conditions during the cleaning process but also on the filtration conditions during particle loading. The structure and location of the particles captured by the glass fiber matrix greatly affect the degree to which they can be subsequently dislodged and removed from the filter medium. Changes in filtration efficiency with service time for various particle diameters in the critical submicron size range, as well as the effects of filtration velocity on the increase in pressure drop, are important criteria with regard to recleaning HEPA filter units. Of special significance for the recleanability of HEPA filter media is knowledge of how operating conditions affect dust cake formation. (author)

  19. Influence of structural disorder on low-temperature behavior of penetration depth in electron-doped high-TC thin films

    International Nuclear Information System (INIS)

    Lanfredi, A.J.C.; Sergeenkov, S.; Araujo-Moreira, F.M.

    2006-01-01

    To probe the influence of structural disorder on low-temperature behavior of magnetic penetration depth, λ(T), in electron-doped high-T C superconductors, a comparative study of high-quality Pr 1.85 Ce 0.15 CuO 4 (PCCO) and Sm 1.85 Ce 0.15 CuO 4 (SCCO) thin films is presented. The λ(T) profiles are extracted from conductance-voltage data using a highly-sensitive home-made mutual-inductance technique. The obtained results confirm a d-wave pairing mechanism in both samples (with nodal gap parameter Δ 0 /k B T C =2.0 and 2.1 for PCCO and SCCO films, respectively), substantially modified by impurity scattering (which is more noticeable in less homogeneous SCCO films) at the lowest temperatures. More precisely, Δλ(T)=λ(T)-λ(0) is found to follow the Goldenfeld-Hirschfeld interpolation formulae Δλ(T)/λ(0)=AT 2 /(T+T 0 ) with T 0 =ln(2)k B Γ 1/2 Δ 0 1/2 being the crossover temperature which demarcates pure and impure scattering processes (T 0 /T C =0.13 and 0.26 for PCCO and SCCO films, respectively). The value of the extracted impurity scattering rate Γ correlates with the quality of our samples and is found to be much higher in less homogeneous films with lower T C

  20. High Resolution ground penetrating radar (GPR) measurements at the laboratory scale to model porosity and permeability in the Miami Limestone in South Florida.

    Science.gov (United States)

    Mount, G. J.; Comas, X.

    2015-12-01

    Subsurface water flow within the Biscayne aquifer is controlled by the heterogeneous distribution of porosity and permeability in the karst Miami Limestone and the presence of numerous dissolution and mega-porous features. The dissolution features and other high porosity areas can create preferential flow paths and direct recharge to the aquifer, which may not be accurately conceptualized in groundwater flow models. As hydrologic conditions are undergoing restoration in the Everglades, understanding the distribution of these high porosity areas within the subsurface would create a better understanding of subsurface flow. This research utilizes ground penetrating radar to estimate the spatial variability of porosity and dielectric permittivity of the Miami Limestone at centimeter scale resolution at the laboratory scale. High frequency GPR antennas were used to measure changes in electromagnetic wave velocity through limestone samples under varying volumetric water contents. The Complex Refractive Index Model (CRIM) was then applied in order to estimate porosity and dielectric permittivity of the solid phase of the limestone. Porosity estimates ranged from 45.2-66.0% from the CRIM model and correspond well with estimates of porosity from analytical and digital image techniques. Dielectric permittivity values of the limestone solid phase ranged from 7.0 and 13.0, which are similar to values in the literature. This research demonstrates the ability of GPR to identify the cm scale spatial variability of aquifer properties that influence subsurface water flow which could have implications for groundwater flow models in the Biscayne and potentially other shallow karst aquifers.

  1. Penetration testing with Perl

    CERN Document Server

    Berdeaux, Douglas

    2014-01-01

    If you are an expert Perl programmer interested in penetration testing or information security, this guide is designed for you. However, it will also be helpful for you even if you have little or no Linux shell experience.

  2. penetrating abdominal trauma

    African Journals Online (AJOL)

    gender, mechanism of injury, injury severity scores (ISS), penetrating ... ileus, reduced pulmonary function and loss of muscle mass and function, all of .... pathophysiology and rehabilitation. ... quality of life after surgery for colorectal cancer.

  3. Autosomal dominant craniometaphyseal dysplasia with atypical features.

    Science.gov (United States)

    McKay, D R; Fialkov, J A

    2002-03-01

    Craniometaphyseal dysplasia (CMD) is a rare genetic disorder of bone modelling characterised by hyperostosis and sclerosis of the craniofacial bones, and abnormal modelling of the metaphyses. Clinically, autosomal dominant (AD) CMD is characterised by facial distortion and cranial-nerve compression. The goals of surgical treatment for AD CMD are cosmetic recontouring of the sclerotic craniofacial bones, correction of nasal obstruction and correction or prevention of neurological manifestations. We describe the successful correction of AD CMD craniofacial manifestations in an individual with atypical findings, and outline an approach for correcting the craniofacial deformities associated with this rare disorder. Copyright 2002 The British Association of Plastic Surgeons.

  4. Clinical neurogenetics: autosomal dominant spinocerebellar ataxia.

    Science.gov (United States)

    Shakkottai, Vikram G; Fogel, Brent L

    2013-11-01

    The autosomal dominant spinocerebellar ataxias are a diverse and clinically heterogeneous group of disorders characterized by degeneration and dysfunction of the cerebellum and its associated pathways. Clinical and diagnostic evaluation can be challenging because of phenotypic overlap among causes, and a stratified and systematic approach is essential. Recent advances include the identification of additional genes causing dominant genetic ataxia, a better understanding of cellular pathogenesis in several disorders, the generation of new disease models that may stimulate development of new therapies, and the use of new DNA sequencing technologies, including whole-exome sequencing, to improve diagnosis. Copyright © 2013 Elsevier Inc. All rights reserved.

  5. Barrier penetration database

    International Nuclear Information System (INIS)

    Fainberg, A.; Bieber, A.M. Jr.

    1978-11-01

    This document is intended to supply the NRC and nuclear power plant licensees with basic data on the times required to penetrate forcibly the types of barriers commonly found in nuclear plants. These times are necessary for design and evaluation of the physical protection system required under 10CFR73.55. Each barrier listed is described in detail. Minor variations in basic barrier construction that result in the same penetration time, are also described

  6. Stochastic scheduling of local distribution systems considering high penetration of plug-in electric vehicles and renewable energy sources

    International Nuclear Information System (INIS)

    Tabatabaee, Sajad; Mortazavi, Seyed Saeedallah; Niknam, Taher

    2017-01-01

    This paper investigates the optimal scheduling of electric power units in the renewable based local distribution systems considering plug-in electric vehicles (PEVs). The appearance of PEVs in the electric grid can create new challenges for the operation of distributed generations and power units inside the network. In order to deal with this issue, a new stochastic optimization method is devised to let the central controll manage the power units and charging behavior of PEVs. The problem formulation aims to minimize the total cost of the network including the cost of power supply for loads and PEVs as well as the cost of energy not supplied (ENS) as the reliability costs. In order to make PEVs as opportunity for the grid, the vehicle-2-grid (V2G) technology is employed to reduce the operational costs. To model the high uncertain behavior of wind turbine, photovoltaics and the charging and discharging pattern of PEVs, a new stochastic power flow based on unscented transform is proposed. Finally, a new optimization algorithm based on bat algorithm (BA) is proposed to solve the problem optimally. The satisfying performance of the proposed stochastic method is tested on a grid-connected local distribution system. - Highlights: • Introduction of stochastic method to assess Plug-in Electric Vehicles effects on the microgrid. • Assessing the role of V2G technology on battery aging and degradation costs. • Use of BA for solving the proposed problem. • Introduction of a new modification method for the BA.

  7. Genome-wide misexpression of X-linked versus autosomal genes associated with hybrid male sterility.

    Science.gov (United States)

    Lu, Xuemei; Shapiro, Joshua A; Ting, Chau-Ti; Li, Yan; Li, Chunyan; Xu, Jin; Huang, Huanwei; Cheng, Ya-Jen; Greenberg, Anthony J; Li, Shou-Hsien; Wu, Mao-Lien; Shen, Yang; Wu, Chung-I

    2010-08-01

    Postmating reproductive isolation is often manifested as hybrid male sterility, for which X-linked genes are overrepresented (the so-called large X effect). In contrast, X-linked genes are significantly under-represented among testis-expressing genes. This seeming contradiction may be germane to the X:autosome imbalance hypothesis on hybrid sterility, in which the X-linked effect is mediated mainly through the misexpression of autosomal genes. In this study, we compared gene expression in fertile and sterile males in the hybrids between two Drosophila species. These hybrid males differ only in a small region of the X chromosome containing the Ods-site homeobox (OdsH) (also known as Odysseus) locus of hybrid sterility. Of genes expressed in the testis, autosomal genes were, indeed, more likely to be misexpressed than X-linked genes under the sterilizing action of OdsH. Since this mechanism of X:autosome interaction is only associated with spermatogenesis, a connection between X:autosome imbalance and the high rate of hybrid male sterility seems plausible.

  8. Novel autosomal dominant TNNT1 mutation causing nemaline myopathy.

    Science.gov (United States)

    Konersman, Chamindra G; Freyermuth, Fernande; Winder, Thomas L; Lawlor, Michael W; Lagier-Tourenne, Clotilde; Patel, Shailendra B

    2017-11-01

    Nemaline myopathy (NEM) is one of the three major forms of congenital myopathy and is characterized by diffuse muscle weakness, hypotonia, respiratory insufficiency, and the presence of nemaline rod structures on muscle biopsy. Mutations in troponin T1 (TNNT1) is 1 of 10 genes known to cause NEM. To date, only homozygous nonsense mutations or compound heterozygous truncating or internal deletion mutations in TNNT1 gene have been identified in NEM. This extended family is of historical importance as some members were reported in the 1960s as initial evidence that NEM is a hereditary disorder. Proband and extended family underwent Sanger sequencing for TNNT1. We performed RT-PCR and immunoblot on muscle to assess TNNT1 RNA expression and protein levels in proband and father. We report a novel heterozygous missense mutation of TNNT1 c.311A>T (p.E104V) that segregated in an autosomal dominant fashion in a large family residing in the United States. Extensive sequencing of the other known genes for NEM failed to identify any other mutant alleles. Muscle biopsies revealed a characteristic pattern of nemaline rods and severe myofiber hypotrophy that was almost entirely restricted to the type 1 fiber population. This novel mutation alters a residue that is highly conserved among vertebrates. This report highlights not only a family with autosomal dominant inheritance of NEM, but that this novel mutation likely acts via a dominant negative mechanism. © 2017 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

  9. MDCT diagnosis of penetrating diaphragm injury

    Energy Technology Data Exchange (ETDEWEB)

    Bodanapally, Uttam K.; Shanmuganathan, Kathirkamanathan; Mirvis, Stuart E.; Sliker, Clint W.; Fleiter, Thorsten R.; Sarada, Kamal; Miller, Lisa A. [University of Maryland School of Medicine, Department of Diagnostic Radiology, Baltimore, MD (United States); Stein, Deborah M. [University of Maryland, Department of Surgery, Shock Trauma Center, Baltimore, MD (United States); Alexander, Melvin [National Study Center for Trauma and Emergency Medical Systems, Baltimore, MD (United States)

    2009-08-15

    The purpose of the study was to determine the diagnostic sensitivity and specificity of multidetector CT (MDCT) in detection of diaphragmatic injury following penetrating trauma. Chest and abdominal CT examinations performed preoperatively in 136 patients after penetrating trauma to the torso with injury trajectory in close proximity to the diaphragm were reviewed by radiologists unaware of surgical findings. Signs associated with diaphragmatic injuries in penetrating trauma were noted. These signs were correlated with surgical diagnoses, and their sensitivity and specificity in assisting the diagnosis were calculated. CT confirmed diaphragmatic injury in 41 of 47 injuries (sensitivity, 87.2%), and an intact diaphragm in 71 of 98 patients (specificity, 72.4%). The overall accuracy of MDCT was 77%. The most accurate sign helping the diagnosis was contiguous injury on either side of the diaphragm in single-entry penetrating trauma (sensitivity, 88%; specificity, 82%). Thus MDCT has high sensitivity and good specificity in detecting penetrating diaphragmatic injuries. (orig.)

  10. MDCT diagnosis of penetrating diaphragm injury

    International Nuclear Information System (INIS)

    Bodanapally, Uttam K.; Shanmuganathan, Kathirkamanathan; Mirvis, Stuart E.; Sliker, Clint W.; Fleiter, Thorsten R.; Sarada, Kamal; Miller, Lisa A.; Stein, Deborah M.; Alexander, Melvin

    2009-01-01

    The purpose of the study was to determine the diagnostic sensitivity and specificity of multidetector CT (MDCT) in detection of diaphragmatic injury following penetrating trauma. Chest and abdominal CT examinations performed preoperatively in 136 patients after penetrating trauma to the torso with injury trajectory in close proximity to the diaphragm were reviewed by radiologists unaware of surgical findings. Signs associated with diaphragmatic injuries in penetrating trauma were noted. These signs were correlated with surgical diagnoses, and their sensitivity and specificity in assisting the diagnosis were calculated. CT confirmed diaphragmatic injury in 41 of 47 injuries (sensitivity, 87.2%), and an intact diaphragm in 71 of 98 patients (specificity, 72.4%). The overall accuracy of MDCT was 77%. The most accurate sign helping the diagnosis was contiguous injury on either side of the diaphragm in single-entry penetrating trauma (sensitivity, 88%; specificity, 82%). Thus MDCT has high sensitivity and good specificity in detecting penetrating diaphragmatic injuries. (orig.)

  11. Akon - A Penetrator for Europa

    Science.gov (United States)

    Jones, Geraint

    2016-04-01

    Jupiter's moon Europa is one of the most intriguing objects in our Solar System. This 2000km-wide body has a geologically young solid water ice crust that is believed to cover a global ocean of liquid water. The presence of this ocean, together with a source of heating through tidal forces, make Europa a conceivable location for extraterrestrial life. The science case for exploring all aspects of this icy world is compelling. NASA has selected the Europa Mission (formerly Europa Clipper) to study Europa in detail in the 2020s through multiple flybys, and ESA's JUICE mission will perform two flybys of the body in the 2030s. The US agency has extended to the European Space Agency an invitation to provide a contribution to their mission. European scientists interested in Europa science and exploration are currently organizing themselves, in the framework of a coordinated Europa M5 Inititative to study concurrently the main options for this ESA contribution, from a simple addition of individual instruments to the NASA spacecraft, to a lander to investigate Europa's surface in situ. A high speed lander - a penetrator - is by far the most promising technology to achieve this latter option within the anticipated mass constraints, and studies of such a hard lander, many funded by ESA, are now at an advanced level. An international team to formally propose an Europa penetrator to ESA in response to the anticipated ESA M5 call is growing. The working title of this proposal is Akon (Άκων), named after the highly accurate javelin gifted to Europa by Zeus in ancient Greek mythology. We present plans for the Akon penetrator, which would impact Europa's surface at several hundred metres per second, and travel up to several metres into the moon's subsurface. To achieve this, the penetrator would be delivered to the surface by a dedicated descent module, to be destroyed on impact following release of the penetrator above the surface. It is planned that the instruments to be

  12. Estimating porosity and solid dielectric permittivity in the Miami Limestone using high-frequency ground penetrating radar (GPR) measurements at the laboratory scale

    Science.gov (United States)

    Mount, Gregory J.; Comas, Xavier

    2014-10-01

    Subsurface water flow in South Florida is largely controlled by the heterogeneous nature of the karst limestone in the Biscayne aquifer and its upper formation, the Miami Limestone. These heterogeneities are amplified by dissolution structures that induce changes in the aquifer's material and physical properties (i.e., porosity and dielectric permittivity) and create preferential flow paths. Understanding such patterns are critical for the development of realistic groundwater flow models, particularly in the Everglades, where restoration of hydrological conditions is intended. In this work, we used noninvasive ground penetrating radar (GPR) to estimate the spatial variability in porosity and the dielectric permittivity of the solid phase of the limestone at centimeter-scale resolution to evaluate the potential for field-based GPR studies. A laboratory setup that included high-frequency GPR measurements under completely unsaturated and saturated conditions was used to estimate changes in electromagnetic wave velocity through Miami Limestone samples. The Complex Refractive Index Model was used to derive estimates of porosity and dielectric permittivity of the solid phase of the limestone. Porosity estimates of the samples ranged between 45.2 and 66.0% and showed good correspondence with estimates of porosity using analytical and digital image techniques. Solid dielectric permittivity values ranged between 7.0 and 13.0. This study shows the ability of GPR to image the spatial variability of porosity and dielectric permittivity in the Miami Limestone and shows potential for expanding these results to larger scales and other karst aquifers.

  13. Penetration portion shielding structure

    International Nuclear Information System (INIS)

    Hayashi, Katsumi; Narita, Hitoshi; Handa, Hiroyuki; Takeuchi, Jun; Tozuka, Fumio.

    1994-01-01

    Openings of a plurality of shieldings for penetration members are aligned to each other, and penetration members are inserted from the openings. Then, the openings of the plurality of shielding members are slightly displaced with each other to make the penetration portions into a helical configuration, so that leakage of radiation is reduced. Upon removal of the members, reverse operation is conducted. When a flowable shielding material is used, the penetration portions are constituted with two plates having previously formed openings and pipes for connecting the openings with each other and a vessel covering the entire of them. After passing the penetration members such as a cable, the relative position of the two plates is changed by twisting, to form a helical configuration which reduces radiation leakage. Since they are bent into the helical configuration, shielding performance is extremely improved compared with a case that radiation leakage is caused from an opening of a straight pipe. In addition, since they can be returned to straight pipes, attachment, detachment and maintenance can be conducted easily. (N.H.)

  14. AutoSOME: a clustering method for identifying gene expression modules without prior knowledge of cluster number

    Directory of Open Access Journals (Sweden)

    Cooper James B

    2010-03-01

    Full Text Available Abstract Background Clustering the information content of large high-dimensional gene expression datasets has widespread application in "omics" biology. Unfortunately, the underlying structure of these natural datasets is often fuzzy, and the computational identification of data clusters generally requires knowledge about cluster number and geometry. Results We integrated strategies from machine learning, cartography, and graph theory into a new informatics method for automatically clustering self-organizing map ensembles of high-dimensional data. Our new method, called AutoSOME, readily identifies discrete and fuzzy data clusters without prior knowledge of cluster number or structure in diverse datasets including whole genome microarray data. Visualization of AutoSOME output using network diagrams and differential heat maps reveals unexpected variation among well-characterized cancer cell lines. Co-expression analysis of data from human embryonic and induced pluripotent stem cells using AutoSOME identifies >3400 up-regulated genes associated with pluripotency, and indicates that a recently identified protein-protein interaction network characterizing pluripotency was underestimated by a factor of four. Conclusions By effectively extracting important information from high-dimensional microarray data without prior knowledge or the need for data filtration, AutoSOME can yield systems-level insights from whole genome microarray expression studies. Due to its generality, this new method should also have practical utility for a variety of data-intensive applications, including the results of deep sequencing experiments. AutoSOME is available for download at http://jimcooperlab.mcdb.ucsb.edu/autosome.

  15. Plasma lipoprotein(a) levels in patients with homozygous autosomal dominant hypercholesterolemia

    NARCIS (Netherlands)

    Sjouke, Barbara; Yahya, Reyhana; Tanck, Michael W. T.; Defesche, Joep C.; de Graaf, Jacqueline; Wiegman, Albert; Kastelein, John J. P.; Mulder, Monique T.; Hovingh, G. Kees; Roeters van Lennep, Jeanine E.

    2017-01-01

    Patients with autosomal dominant hypercholesterolemia (ADH), caused by mutations in either low-density lipoprotein receptor (LDLR), apolipoprotein B (APOB), or proprotein convertase subtilisin-kexin type 9 (PCSK9) are characterized by high low-density lipoprotein cholesterol levels and in some

  16. Plasma lipoprotein(a) levels in patients with homozygous autosomal dominant hypercholesterolemia

    NARCIS (Netherlands)

    Sjouke, B.; Yahya, R.; Tanck, M.W.T.; Defesche, J.C.; Graaf, J. de; Wiegman, A.; Kastelein, J.J.; Mulder, M.T.; Hovingh, G.K.; Roeters van Lennep, J.E.

    2017-01-01

    BACKGROUND: Patients with autosomal dominant hypercholesterolemia (ADH), caused by mutations in either low-density lipoprotein receptor (LDLR), apolipoprotein B (APOB), or proprotein convertase subtilisin-kexin type 9 (PCSK9) are characterized by high low-density lipoprotein cholesterol levels and

  17. New parkin mutations and atypical phenotypes in families with autosomal recessive parkinsonism.

    NARCIS (Netherlands)

    Rawal, N.; Periquet, M.; Lohmann, E.; Lucking, C.B.; Teive, H.; Ambrosio, G.; Raskin, S.; Lincoln, S.; Hattori, N.; Guimaraes, J.; Horstink, M.W.I.M.; Santos Bele, W. Dos; Brousolle, E.; Destee, A.; Mizuno, Y.; Farrer, M.; Deleuze, J.F.; Michele, G. de; Agid, Y.; Durr, A.; Brice, A.

    2003-01-01

    The frequency of parkin mutations was evaluated in 30 families of highly diverse geographic origin with early-onset autosomal recessive parkinsonism. Twelve different mutations, six of which were new, were found in 10 families from Europe and Brazil. Patients with parkin mutations had significantly

  18. Ground penetrating radar

    CERN Document Server

    Daniels, David J

    2004-01-01

    Ground-penetrating radar has come to public attention in recent criminal investigations, but has actually been a developing and maturing remote sensing field for some time. In the light of recent expansion of the technique to a wide range of applications, the need for an up-to-date reference has become pressing. This fully revised and expanded edition of the best-selling Surface-Penetrating Radar (IEE, 1996) presents, for the non-specialist user or engineer, all the key elements of this technique, which span several disciplines including electromagnetics, geophysics and signal processing. The

  19. Skull penetrating wound

    International Nuclear Information System (INIS)

    Gonzalez Orlandi, Yvei; Junco Martin, Reinel; Rojas Manresa, Jorge; Duboy Limonta, Victor; Matos Herrera, Omar; Saez Corvo, Yunet

    2011-01-01

    The cranioencephalic trauma is common in the emergence centers to care for patients with multiple traumata and it becames in a health problem in many countries. Skull penetrating trauma is located in a special place due to its low frequency. In present paper a case of male patient aged 52 severely skull-injured with penetrating wound caused by a cold steel that remained introduced into the left frontotemporal region. After an imaging study the emergence surgical treatment was applied and patient evolves adequately after 25 days of hospitalization. Nowadays, she is under rehabilitation treatment due to a residual right hemiparesis.(author)

  20. Autosomal dominant cortical tremor, myoclonus and epilepsy.

    Science.gov (United States)

    Striano, Pasquale; Zara, Federico

    2016-09-01

    The term 'cortical tremor' was first introduced by Ikeda and colleagues to indicate a postural and action-induced shivering movement of the hands which mimics essential tremor, but presents with the electrophysiological findings of cortical reflex myoclonus. The association between autosomal dominant cortical tremor, myoclonus and epilepsy (ADCME) was first recognized in Japanese families and is now increasingly reported worldwide, although it is described using different acronyms (BAFME, FAME, FEME, FCTE and others). The disease usually takes a benign course, although drug-resistant focal seizures or slight intellectual disability occur in some cases. Moreover, a worsening of cortical tremor and myoclonus is common in advanced age. Although not yet recognized by the International League Against Epilepsy (ILAE), this is a well-delineated epilepsy syndrome with remarkable features that clearly distinguishes it from other myoclonus epilepsies. Moreover, genetic studies of these families show heterogeneity and different susceptible chromosomal loci have been identified.

  1. MRI of autosomal dominant pure spastic paraplegia

    DEFF Research Database (Denmark)

    Krabbe, K.; Nielsen, J.E.; Fallentin, E.

    1997-01-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus...... callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a "corpus-callosum index" expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord...... at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and "corpus-callosum index" than controls. This finding, not reported previously...

  2. A review of penetration mechanisms and dynamic properties of tungsten and depleted uranium penetrators

    International Nuclear Information System (INIS)

    Andrew, S.P.; Caligiuri, R.D.; Eiselstein, L.E.

    1991-01-01

    Over the last decade, depleted uranium (DU) and tungsten alloys have been the materials of choice for kinetic energy penetrators. However, despite improvements in mechanical properties in recent years, the penetration performance of tungsten still lags behind that of DU. One possible reason is the difference in deformation mechanisms- DU alloys tend to shear band as they penetrate the target material, whereas tungsten penetrators tend to mushroom. As a first step to determining whether shear banding is truly the reason for superior DU performance, a review and summary of the available information was performed. This paper presents a state-of-the-art review of the formulation, high strain- rate properties, and penetration phenomena of penetrators manufactured from both tungsten and DU alloys. Specifically, the effects of composition, processing, and heat treatment on mechanical properties and penetration mechanisms of these alloys are discussed. Penetration data and models for penetration mechanisms (in particular shear banding) are also presented, as well as the applicability of these models and their salient features

  3. Evidence for nonallelic genetic heterogeneity in autosomal recessive retinitis pigmentosa

    NARCIS (Netherlands)

    Bleeker-Wagemakers, L. M.; Gal, A.; Kumar-Singh, R.; van den Born, L. I.; Li, Y.; Schwinger, E.; Sandkuijl, L. A.; Bergen, A. A.; Kenna, P.; Humphries, P.

    1992-01-01

    Recent evidence suggesting the involvement of mutant rhodopsin proteins in the pathogenesis of autosomal recessive retinitis pigmentosa has prompted us to investigate whether this form of the disease shows non-allelic genetic heterogeneity, as has previously been shown to be the case in autosomal

  4. Allele frequency distribution for 21 autosomal STR loci in Bhutan.

    Science.gov (United States)

    Kraaijenbrink, Thirsa; van Driem, George L; Tshering of Gaselô, Karma; de Knijff, Peter

    2007-07-20

    We studied the allele frequency distribution of 21 autosomal STR loci contained in the AmpFlSTR Identifiler (Applied Biosystems), the Powerplex 16 (Promega) and the FFFL (Promega) multiplex PCR kits among 936 individuals from the Royal Kingdom of Bhutan. As such these are the first published autosomal DNA results from this country.

  5. Management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation.

    Science.gov (United States)

    Tellman, Matthew W; Bahler, Clinton D; Shumate, Ashley M; Bacallao, Robert L; Sundaram, Chandru P

    2015-05-01

    Chronic pain is a prominent feature of autosomal dominant polycystic kidney disease that is difficult to treat and manage, often resulting in a decrease in quality of life. Understanding the underlying anatomy of renal innervation and the various etiologies of pain that occur in autosomal dominant polycystic kidney disease can help guide proper treatments to manage pain. Reviewing previously studied treatments for pain in autosomal dominant polycystic kidney disease can help characterize treatment in a stepwise fashion. We performed a literature search of the etiology and management of pain in autosomal dominant polycystic kidney disease and the anatomy of renal innervation using PubMed® and Embase® from January 1985 to April 2014 with limitations to human studies and English language. Pain occurs in the majority of patients with autosomal dominant polycystic kidney disease due to renal, hepatic and mechanical origins. Patients may experience different types of pain which can make it difficult to clinically confirm its etiology. An anatomical and histological evaluation of the complex renal innervation helps in understanding the mechanisms that can lead to renal pain. Understanding the complex nature of renal innervation is essential for surgeons to perform renal denervation. The management of pain in autosomal dominant polycystic kidney disease should be approached in a stepwise fashion. Acute causes of renal pain must first be ruled out due to the high incidence in autosomal dominant polycystic kidney disease. For chronic pain, nonopioid analgesics and conservative interventions can be used first, before opioid analgesics are considered. If pain continues there are surgical interventions such as renal cyst decortication, renal denervation and nephrectomy that can target pain produced by renal or hepatic cysts. Chronic pain in patients with autosomal dominant polycystic kidney disease is often refractory to conservative, medical and other noninvasive treatments

  6. Site-specific epsilon-NH2 monoacylation of pancreatic phospholipase A2. 2. Transformation of soluble phospholipase A2 into a highly penetrating "membrane-bound" form.

    Science.gov (United States)

    Van der Wiele, F C; Atsma, W; Roelofsen, B; van Linde, M; Van Binsbergen, J; Radvanyi, F; Raykova, D; Slotboom, A J; De Haas, G H

    1988-03-08

    Long-chain lecithins present in bilayer structures like vesicles or membranes are only very poor substrates for pancreatic phospholipases A2. This is probably due to the fact that pancreatic phospholipases A2 cannot penetrate into the densely packed bilayer structures. To improve the weak penetrating properties of pancreatic phospholipases A2, we prepared and characterized a number of pancreatic phospholipase A2 mutants that have various long acyl chains linked covalently to Lys116 in porcine and to Lys10 in bovine phospholipase A2 [Van der Wiele, F.C., Atsma, W., Dijkman, R., Schreurs, A.M.M., Slotboom, A.J., & De Haas, G.H. (1988) Biochemistry (preceding paper in this issue)]. When monomolecular surface layers of L- and D-didecanoyllecithin were used, it was found that the introduction of caprinic, lauric, palmitic, and oleic acid at Lys116 in the porcine enzyme increases its penetrating power from 13 to about 17, 20, 32, and 22 dyn/cm, respectively, before long lag periods were obtained. Incorporation of a palmitoyl moiety at Lys10 in the bovine enzyme shifted the penetrating power from 11 to about 25 dyn/cm. Only the best penetrating mutant, viz., porcine phospholipase A2 having a palmitoyl moiety at Lys116, was able to cause complete leakage of 6-carboxyfluorescein entrapped in small unilamellar vesicles of egg lecithin under nonhydrolytic conditions. Similarly, only this latter palmitoylphospholipase A2 completely hydrolyzed all lecithin in the outer monolayer of the human erythrocyte at a rate much faster than Naja naja phospholipase A2, the most powerful penetrating snake venom enzyme presently known.

  7. Tumor penetrating peptides

    Directory of Open Access Journals (Sweden)

    Tambet eTeesalu

    2013-08-01

    Full Text Available Tumor-homing peptides can be used to deliver drugs into tumors. Phage library screening in live mice has recently identified homing peptides that specifically recognize the endothelium of tumor vessels, extravasate, and penetrate deep into the extravascular tumor tissue. The prototypic peptide of this class, iRGD (CRGDKGPDC, contains the integrin-binding RGD motif. RGD mediates tumor homing through binding to αv integrins, which are selectively expressed on various cells in tumors, including tumor endothelial cells. The tumor-penetrating properties of iRGD are mediated by a second sequence motif, R/KXXR/K. This C-end Rule (or CendR motif is active only when the second basic residue is exposed at the C-terminus of the peptide. Proteolytic processing of iRGD in tumors activates the cryptic CendR motif, which then binds to neuropilin-1 activating an endocytic bulk transport pathway through tumor tissue. Phage screening has also yielded tumor-penetrating peptides that function like iRGD in activating the CendR pathway, but bind to a different primary receptor. Moreover, novel tumor-homing peptides can be constructed from tumor-homing motifs, CendR elements and protease cleavage sites. Pathologies other than tumors can be targeted with tissue-penetrating peptides, and the primary receptor can also be a vascular zip code of a normal tissue. The CendR technology provides a solution to a major problem in tumor therapy, poor penetration of drugs into tumors. The tumor-penetrating peptides are capable of taking a payload deep into tumor tissue in mice, and they also penetrate into human tumors ex vivo. Targeting with these peptides specifically increases the accumulation in tumors of a variety of drugs and contrast agents, such as doxorubicin, antibodies and nanoparticle-based compounds. Remarkably the drug to be targeted does not have to be coupled to the peptide; the bulk transport system activated by the peptide sweeps along any compound that is

  8. Penetrance of breast cancer, ovarian cancer and contralateral breast cancer in BRCA1 and BRCA2 families : high cancer incidence at older age

    NARCIS (Netherlands)

    van der Kolk, Dorina M.; de Bock, Geertruida H.; Leegte, Beike K.; Schaapveld, Michael; Mourits, Marian J. E.; de Vries, J; van der Hout, Annemieke H.; Oosterwijk, Jan C.

    Accurate estimations of lifetime risks of breast and ovarian cancer are crucial for counselling women from BRCA1/2 families. We therefore determined breast and ovarian cancer penetrance in BRCA1/2 mutation families in the northern Netherlands and compared them with the incidence of cancers in the

  9. Monozygotic twins with CAPN5 autosomal dominant neovascular inflammatory vitreoretinopathy

    Directory of Open Access Journals (Sweden)

    Rowell HA

    2012-12-01

    Full Text Available Hannah A Rowell,1,2 Alexander G Bassuk,3,4 Vinit B Mahajan1,21Omics Laboratory, 2Department of Ophthalmology and Visual Sciences, 3Department of Pediatrics, 4Department of Neurology, University of Iowa, Iowa City, IA, USABackground: The purpose of this study was to describe the clinical findings in a set of monozygotic twins with autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV over a 23-year period.Methods: A pair of female twins were examined between 26 and 49 years of age. The concordance and discordance of their clinical features were determined. The CAPN5 gene was sequenced using genomic DNA.Results: Both twins of an affected father demonstrated Stage I ADNIV with mild vitreous cells and a negative b-wave on electroretinography. Genetic analysis confirmed a guanine to thymine nucleotide (c.728G>T, pArg243Leu mutation in the CAPN5 gene. Over the course of 23 years, each twin progressed to stage III disease, showing posterior uveitis, cystoid macular edema, intraocular fibrosis, early retinal neovascularization, retinal degeneration, and cataract. Disease progression varied moderately between each twin and was asymmetrical between eyes. Twin A had 20/70 and 20/125 in the right and left eye, respectively, and underwent vitrectomy surgery and intravitreal injections with bevacizumab for recurrent cystoid macular edema. Twin B maintained 20/20 and 20/40 in the right and left eye, respectively without intervention.Conclusion: There was asymmetry between the eyes and some discordance in the rate of disease progression in these monozygotic twins with ADNIV. The overall high disease concordance suggests genetic factors play a major role in clinical manifestations in CAPN5 vitreoretinopathy.Keywords: autosomal dominant neovascular inflammatory vitreoretinopathy, ADNIV, CAPN5, calpain-5, monozygotic twins

  10. Development Of Very-High-Strength and High-Performance Concrete Materials for Improvement of Barriers Against Blast and Projectile Penetration

    National Research Council Canada - National Science Library

    O'Neill, E. F., III; Cummins, T. K; Durst, B. P; Kinnebrew, P. G; Boone, R. N; Torres, R. X

    2004-01-01

    .... S. Army Engineer Research and Development Center (ERDC) is developing several high-performance concretes to mitigate the effects of blast and ballistic threats from conventional and asymmetric weapons...

  11. Antibody tumor penetration

    Science.gov (United States)

    Thurber, Greg M.; Schmidt, Michael M.; Wittrup, K. Dane

    2009-01-01

    Antibodies have proven to be effective agents in cancer imaging and therapy. One of the major challenges still facing the field is the heterogeneous distribution of these agents in tumors when administered systemically. Large regions of untargeted cells can therefore escape therapy and potentially select for more resistant cells. We present here a summary of theoretical and experimental approaches to analyze and improve antibody penetration in tumor tissue. PMID:18541331

  12. Python penetration testing essentials

    CERN Document Server

    Mohit

    2015-01-01

    If you are a Python programmer or a security researcher who has basic knowledge of Python programming and want to learn about penetration testing with the help of Python, this book is ideal for you. Even if you are new to the field of ethical hacking, this book can help you find the vulnerabilities in your system so that you are ready to tackle any kind of attack or intrusion.

  13. Pregnancy in autosomal recessive polycystic kidney disease.

    Science.gov (United States)

    Banks, Nicole; Bryant, Joy; Fischer, Roxanne; Huizing, Marjan; Gahl, William A; Gunay-Aygun, Meral

    2015-03-01

    Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. As treatments improve, more women are reaching reproductive age, but little is known about ARPKD and pregnancy. In our ongoing study on ARPKD and other ciliopathies, 12 females over 18 years of age were identified and systematically evaluated. Six had children; four carried pregnancies and delivered, one used assisted reproductive technology and had a surrogate carry the pregnancy, and one adopted. We report the outcomes of four pregnancies with live birth deliveries and two women who chose alternate family building options. Patient one was diagnosed at 6 months, and at age 21 had a pregnancy complicated by transient worsening of renal function (creatinine increase from 1.15 to 1.78 mg/dL). Patient two was diagnosed with ARPKD at age seven and had an uncomplicated pregnancy at age 23. Patient three was diagnosed incidentally with ARPKD at age 23, 3 months after completion of an uncomplicated pregnancy. Patient four who had an uncomplicated pregnancy at age 33 was diagnosed with ARPKD at age 46. Women with ARPKD face reproductive decisions largely bereft of information about the pregnancies of other ARPKD patients. We report four cases of pregnancy and ARPKD to expand current knowledge and encourage further research.

  14. MRI of autosomal dominant pure spastic paraplegia

    International Nuclear Information System (INIS)

    Krabbe, K.; Fallentin, E.; Herning, M.; Nielsen, J.E.; Fenger, K.

    1997-01-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a ''corpus-callosum index'' expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and ''corpus-callosum index'' than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination. (orig.). With 1 fig., 3 tabs

  15. MRI of autosomal dominant pure spastic paraplegia

    Energy Technology Data Exchange (ETDEWEB)

    Krabbe, K.; Fallentin, E.; Herning, M. [Danish Research Center of Magnetic Resonance, Hvidovre Hospital, Kettegaard alle 30, DK-2650 Hvidovre (Denmark); Nielsen, J.E.; Fenger, K. [Institute of Medical Biochemistry and Genetics, Laboratory of Medical Genetics, Section of Neurogenetics, University of Copenhagen (Denmark)

    1997-10-01

    We examined 16 patients with autosomal dominant pure spastic paraplegia (HSP) and 15 normal controls matched for age and sex using MRI of the brain and spinal cord. Images were assessed qualitatively by two independent radiologists, blinded to the clinical diagnosis. Areas of the brain and corpus callosum on one midsagittal slice and the area of the brain on one axial slice were measured and a ``corpus-callosum index`` expressing the size of the corpus callosum relative to that of the brain was calculated. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C 2, C 5, T 3, T 6, T 9 and T 11 were measured. No significant differences between patients and controls were found on qualitative evaluation of the images. The patients had a significantly smaller corpus callosum and ``corpus-callosum index`` than controls. This finding, not reported previously, might indicate that the disease process in pure HSP is not confined to the spinal cord. The anteroposterior diameters of the spinal cord at T 3 and T 9 were significantly smaller in patients than in controls. This might correspond to the degeneration of the pyramidal tracts and the dorsal columns described at neuropathological examination. (orig.). With 1 fig., 3 tabs.

  16. Autosomal Dominant Growth Hormone Deficiency (Type II).

    Science.gov (United States)

    Alatzoglou, Kyriaki S; Kular, Dalvir; Dattani, Mehul T

    2015-06-01

    Isolated growth hormone deficiency (IGHD) is the commonest pituitary hormone deficiency resulting from congenital or acquired causes, although for most patients its etiology remains unknown. Among the known factors, heterozygous mutations in the growth hormone gene (GH1) lead to the autosomal dominant form of GHD, also known as type II GHD. In many cohorts this is the commonest form of congenital isolated GHD and is mainly caused by mutations that affect the correct splicing of GH-1. These mutations cause skipping of the third exon and lead to the production of a 17.5-kDa GH isoform that exerts a dominant negative effect on the secretion of the wild type GH. The identification of these mutations has clinical implications for the management of patients, as there is a well-documented correlation between the severity of the phenotype and the increased expression of the 17.5-kDa isoform. Patients with type II GHD have a variable height deficit and severity of GHD and may develop additional pituitary hormone defiencies over time, including ACTH, TSH and gonadotropin deficiencies. Therefore, their lifelong follow-up is recommended. Detailed studies on the effect of heterozygous GH1 mutations on the trafficking, secretion and action of growth hormone can elucidate their mechanism on a cellular level and may influence future treatment options for GHD type II.

  17. Unilateral Autosomal Recessive Anophthalmia in a Patient with Cystic Craniopharyngioma

    Science.gov (United States)

    Kumar, Amandeep; Bansal, Ankit; Garg, Ajay; Sharma, Bhawani S.

    2014-01-01

    Abstract Anophthalmia is a rare ocular malformation. It is a genetically determined disorder and is typically associated with syndromes. However, sporadic nonsyndromic familial as well as non-familial cases of anophthalmia have also been reported. Non-syndromic familial cases are usually bilateral and have been attributed to autosomal recessive, autosomal dominant, and X-linked inheritance patterns. The authors hereby report a rare case of autosomal recessive unilateral anophthalmia in a patient with no other associated congenital anomaly. Patient was operated for craniopharyngioma. The clinical, radiological and intraoperative findings are discussed. PMID:27928292

  18. Low Force Penetration of Icy Regolith

    Science.gov (United States)

    Mantovani, J. G.; Galloway, G. M.; Zacny, K.

    2016-01-01

    A percussive cone penetrometer measures the strength of granular material by using percussion to deliver mechanical energy into the material. A percussive cone penetrometer was used in this study to penetrate a regolith ice mixture by breaking up ice and decompacting the regolith. As compared to a static cone penetrometer, percussion allows low reaction forces to push a penetrometer probe tip more easily into dry regolith in a low gravity environment from a planetary surface rover or a landed spacecraft. A percussive cone penetrates icy regolith at ice concentrations that a static cone cannot penetrate. In this study, the percussive penetrator was able to penetrate material under 65 N of down-force which could not be penetrated using a static cone under full body weight. This paper discusses using a percussive cone penetrometer to discern changes in the concentration of water-ice in a mixture of lunar regolith simulant and ice to a depth of one meter. The rate of penetration was found to be a function of the ice content and was not significantly affected by the down-force. The test results demonstrate that this method may be ideal for a small platform in a reduced gravity environment. However, there are some cases where the system may not be able to penetrate the icy regolith, and there is some risk of the probe tip becoming stuck so that it cannot be retracted. It is also shown that a percussive cone penetrometer could be used to prospect for water ice in regolith at concentrations as high as 8 by weight.

  19. High Penetration Photovoltaic Power Electronics and Energy Management Technology Research, Development and Demonstration: Cooperative Research and Development Final Report, CRADA Number CRD-13-517

    Energy Technology Data Exchange (ETDEWEB)

    Hudgins, Andrew P. [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2018-01-25

    Advanced Energy Industries, Inc., will partner with DOE's National Renewable Energy Laboratory (NREL) to conduct research and development to demonstrate technologies that will increase the penetration of photovoltaic (PV) technologies for commercial and utility applications. Standard PV power control systems use simple control techniques that only provide real power to the grid. A focus of this partnership is to demonstrate how state of the art control and power electronic technologies can be combined to create a utility interactive control platform.

  20. Preparation of Power Distribution System for High Penetration of Renewable Energy Part I. Dynamic Voltage Restorer for Voltage Regulation Pat II. Distribution Circuit Modeling and Validation

    Science.gov (United States)

    Khoshkbar Sadigh, Arash

    other analyses. In this study, ETAP, OpenDSS, and GridLab-D are considered, and PSCMD transfer applications written in MATLAB have been developed for each of these to read the circuit model data provided in the UP spreadsheet. In order to test the developed PSCMD transfer applications, circuit model data of a test circuit and a power distribution circuit from Southern California Edison (SCE) - a utility company - both built in CYME, were exported into the spreadsheet file according to the UP format. Thereafter, circuit model data were imported successfully from the spreadsheet files into above mentioned software using the PSCMD transfer applications developed for each software. After the SCE studied circuit is transferred into OpenDSS software using the proposed UP scheme and developed application, it has been studied to investigate the impacts of large-scale solar energy penetration. The main challenge of solar energy integration into power grid is its intermittency (i.e., discontinuity of output power) nature due to cloud shading of photovoltaic panels which depends on weather conditions. In order to conduct this study, OpenDSS time-series simulation feature, which is required due to intermittency of solar energy, is utilized. In this study, the impacts of intermittency of solar energy penetration, especially high-variability points, on voltage fluctuation and operation of capacitor bank and voltage regulator is provided. In addition, the necessity to interpolate and resample unequally spaced time-series measurement data and convert them to equally spaced time-series data as well as the effect of resampling time-interval on the amount of error is discussed. Two applications are developed in Matlab to do interpolation and resampling as well as to calculate the amount of error for different resampling time-intervals to figure out the suitable resampling time-interval. Furthermore, an approach based on cumulative distribution, regarding the length for lines/cables types

  1. Genetics Home Reference: autosomal dominant leukodystrophy with autonomic disease

    Science.gov (United States)

    ... have muscle stiffness (spasticity) or weakness and involuntary rhythmic shaking, called intention tremor because it worsens during ... Hobson G, Brusco A, Brussino A, Padiath QS. Analysis of LMNB1 duplications in autosomal dominant leukodystrophy provides ...

  2. Genetics Home Reference: autosomal dominant congenital stationary night blindness

    Science.gov (United States)

    ... collapse boxes. Description Autosomal dominant congenital stationary night blindness is a disorder of the retina , which is the specialized tissue at the back of the eye that detects light and color. People with this condition typically have difficulty seeing ...

  3. Genetics Home Reference: autosomal recessive congenital stationary night blindness

    Science.gov (United States)

    ... collapse boxes. Description Autosomal recessive congenital stationary night blindness is a disorder of the retina , which is the specialized tissue at the back of the eye that detects light and color. People with this condition typically have difficulty seeing ...

  4. Genetics Home Reference: autosomal dominant nocturnal frontal lobe epilepsy

    Science.gov (United States)

    ... with ADNFLE have experienced psychiatric disorders (such as schizophrenia), behavioral problems, or intellectual disability. It is unclear ... Epilepsy Society Citizens United for Research in Epilepsy (CURE) GeneReviews (1 link) Autosomal Dominant Nocturnal Frontal Lobe ...

  5. Market penetration of ethanol

    International Nuclear Information System (INIS)

    Szulczyk, Kenneth R.; McCarl, Bruce A.; Cornforth, Gerald

    2010-01-01

    This research examines in detail the technology and economics of substituting ethanol for gasoline. This endeavor examines three issues. First, the benefits of ethanol/gasoline blends are examined, and then the technical problems of large-scale implementation of ethanol. Second, ethanol production possibilities are examined in detail from a variety of feedstocks and technologies. The feedstocks are the starch/sugar crops and crop residues, while the technologies are corn wet mill, dry grind, and lignocellulosic fermentation. Examining in detail the production possibilities allows the researchers to identity the extent of technological change, production costs, byproducts, and GHG emissions. Finally, a U.S. agricultural model, FASOMGHG, is updated which predicts the market penetration of ethanol given technological progress, variety of technologies and feedstocks, market interactions, energy prices, and GHG prices. FASOMGHG has several interesting results. First, gasoline prices have a small expansionary impact on the U.S. ethanol industry. Both agricultural producers' income and cost both increase with higher energy prices. If wholesale gasoline is $4 per gallon, the predicted ethanol market penetration attains 53% of U.S. gasoline consumption in 2030. Second, the corn wet mill remains an important industry for ethanol production, because this industry also produces corn oil, which could be converted to biodiesel. Third, GHG prices expand the ethanol industry. However, the GHG price expands the corn wet mill, but has an ambiguous impact on lignocellulosic ethanol. Feedstocks for lignocellulosic fermentation can also be burned with coal to generate electricity. Both industries are quite GHG efficient. Finally, U.S. government subsidies on biofuels have an expansionary impact on ethanol production, but may only increase market penetration by an additional 1% in 2030, which is approximately 6 billion gallons. (author)

  6. SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis

    OpenAIRE

    Orlacchio, Antonio; Babalini, Carla; Borreca, Antonella; Patrono, Clarice; Massa, Roberto; Basaran, Sarenur; Munhoz, Renato P.; Rogaeva, Ekaterina A.; St George-Hyslop, Peter H.; Bernardi, Giorgio; Kawarai, Toshitaka

    2010-01-01

    The mutation of the spatacsin gene is the single most common cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum. Common clinical, pathological and genetic features between amyotrophic lateral sclerosis and hereditary spastic paraplegia motivated us to investigate 25 families with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival for mutations in the spatascin gene. The inclusion criterion was a diagnosis of clinically definite ...

  7. Study of Penetration Technology

    Science.gov (United States)

    1976-11-01

    srecimens fabricated at the AFATL, AISI-01 oil quenched bar stock was used. Three of the projectiles used in the Eglin penetration experiments are shown...in the Mathema- tical Laboratory at Eglin AFB, is essencially a fourth order Runge-Kutta numerical method for solving simultaneous differential...C9G. VEL. X-COMPo (M4/5): d!10. I oil 140. 107. 82. RmCC~kr’ED TIME OF MAXIftjM/MINIM94U COIL VOLTAGE tSI MAX 0040 A 55 *.03J04A 0 0 05ji6 .000634 MIN

  8. Deep penetration calculations

    International Nuclear Information System (INIS)

    Thompson, W.L.; Deutsch, O.L.; Booth, T.E.

    1980-04-01

    Several Monte Carlo techniques are compared in the transport of neutrons of different source energies through two different deep-penetration problems each with two parts. The first problem involves transmission through a 200-cm concrete slab. The second problem is a 90 0 bent pipe jacketed by concrete. In one case the pipe is void, and in the other it is filled with liquid sodium. Calculations are made with two different Los Alamos Monte Carlo codes: the continuous-energy code MCNP and the multigroup code MCMG

  9. Type II autosomal dominant osteopetrosis (Albers-Schönberg disease): clinical and radiological manifestations in 42 patients.

    Science.gov (United States)

    Bénichou, O D; Laredo, J D; de Vernejoul, M C

    2000-01-01

    Type II autosomal dominant osteopetrosis (ADO II, Albers-Schonberg disease) is a genetic condition characterized by generalized osteosclerosis predominating in some skeletal sites such as the spine and pelvis. ADO II is rare, and most available clinical descriptions are based on small numbers of patients. We report the clinical and radiological manifestations in 42 ADO II patients. To our knowledge, this is the largest series reported so far. Our inclusion criterion was presence on radiographs of the spine of vertebral endplate thickening, producing the classic sandwich vertebra appearance. We found various patterns of sandwich vertebra, of which we provide a description to assist physicians in diagnosing ADO II. The classic bone-within-bone appearance was present in most but not all skeletal sites. The radiological penetrance of the disease was high (90%) and increased after 20 years of age. As many as 81% of our patients experienced clinical manifestations. Fractures were common (78% of patients) and healed slowly. Hip osteoarthritis developed in 27% of patients and required arthroplasty in 9 of the 16 affected hips. Nonmandibular osteomyelitis occurred in 4 cases (11%). Twenty-four percent of patients had thoracic or lumbar scoliosis. Orthopedic surgery was performed in 52.8% of patients, of whom half had at least three surgical procedures for internal fracture fixation, arthroplasty, limb deformity correction, or treatment of surgical complications. There was a high rate of surgical complications including nonunion, infection, prosthesis loosening, and intraoperative fractures. Nearly two-thirds of patients (64%) had stomatologic manifestations, including mandibular osteomyelitis in 4 patients (11%). Cranial nerve involvement responsible for hearing loss, bilateral optic atrophy, and/or facial palsy was present in 14 patients but was clearly attributable to ADO II in only 6 cases (16%). This large series sheds new light on several aspects of ADO II, most

  10. Penetrating cardiothoracic war wounds.

    Science.gov (United States)

    Biocina, B; Sutlić, Z; Husedzinović, I; Rudez, I; Ugljen, R; Letica, D; Slobodnjak, Z; Karadza, J; Brida, V; Vladović-Relja, T; Jelić, I

    1997-03-01

    Penetrating cardiothoracic war wounds are very common among war casualties. Those injuries require prompt and specific treatment in an aim to decrease mortality and late morbidity. There are a few controversies about the best modality of treatment for such injuries, and there are not many large series of such patients in recent literature. We analysed a group of 259 patients with penetrating cardiothoracic war wounds admitted to our institutions between May 1991 and October 1992. There were 235 (90.7%) patients with thoracic wounds, 14 (5.4%) patients with cardiac, wounds and in 10 (3.7%) patients both heart and lungs were injured. The cause of injury was shrapnel in 174 patients (67%), bullets in 25 patients (9.7%), cluster bomb particles in 45 patients (17.3%) and other (blast etc.) in 15 patients (6%). Patients, 69, had concomitant injuries of various organs. The initial treatment in 164 operated patients was chest drainage in 76 (46.3%) patients, thoracotomy and suture of the lung in 71 (43.2%) patients, lobectomy in 12 (7.3%) patients and pneumonectomy in 5 (3%) patients. Complications include pleural empyema and/or lung abscess in 20 patients (8.4%), incomplete reexpansion of the lung in 10 patients (4.2%), osteomyelitis of the rib in 5 patients (2.1%) and bronchopleural fistula in 1 patient (0.4%). Secondary procedures were decortication in 12 patients, rib resection in 5 patients, lobectomy in 2 patients, pneumonectomy in 4 patients, reconstruction of the chest wall in 2 patients and closure of the bronchopleural fistula in 1 patient. The cardiac chamber involved was right ventricle in 12 patients, left ventricular in 6 patients, right atrium in 7 patients, left atrium in 3 patients, ascending aorta in 2 patients and 1 patient which involved descending aorta, right ventricle and coronary artery (left anterior descending) and inferior vena cava, respectively. The primary procedure was suture in 17 patients (in 10 patients with the additional suture of the

  11. [Autosomal-recessive renal cystic disease and congenital hepatic fibrosis: clinico-anatomic case].

    Science.gov (United States)

    Rostol'tsev, K V; Burenkov, R A; Kuz'micheva, I A

    2012-01-01

    Clinico-anatomic observation of autosomal-recessive renal cystic disease and congenital hepatic fibrosis at two fetuses from the same family was done. Mutation of His3124Tyr in 58 exon of PKHD1 gene in heterozygous state was found out. The same pathomorphological changes in the epithelium of cystic renal tubules and bile ducts of the liver were noted. We suggest that the autopsy research of fetuses with congenital abnormalities, detected after prenatal ultrasonic screening, has high diagnostic importance.

  12. Penetration Tester's Open Source Toolkit

    CERN Document Server

    Faircloth, Jeremy

    2011-01-01

    Great commercial penetration testing tools can be very expensive and sometimes hard to use or of questionable accuracy. This book helps solve both of these problems. The open source, no-cost penetration testing tools presented do a great job and can be modified by the user for each situation. Many tools, even ones that cost thousands of dollars, do not come with any type of instruction on how and in which situations the penetration tester can best use them. Penetration Tester's Open Source Toolkit, Third Edition, expands upon existing instructions so that a professional can get the most accura

  13. Genes and Mutations Causing Autosomal Dominant Retinitis Pigmentosa

    Science.gov (United States)

    Daiger, Stephen P.; Bowne, Sara J.; Sullivan, Lori S.

    2015-01-01

    Retinitis pigmentosa (RP) has a prevalence of approximately one in 4000; 25%–30% of these cases are autosomal dominant retinitis pigmentosa (adRP). Like other forms of inherited retinal disease, adRP is exceptionally heterogeneous. Mutations in more than 25 genes are known to cause adRP, more than 1000 mutations have been reported in these genes, clinical findings are highly variable, and there is considerable overlap with other types of inherited disease. Currently, it is possible to detect disease-causing mutations in 50%–75% of adRP families in select populations. Genetic diagnosis of adRP has advantages over other forms of RP because segregation of disease in families is a useful tool for identifying and confirming potentially pathogenic variants, but there are disadvantages too. In addition to identifying the cause of disease in the remaining 25% of adRP families, a central challenge is reconciling clinical diagnosis, family history, and molecular findings in patients and families. PMID:25304133

  14. Preimplantation Genetic Diagnosis Counseling in Autosomal Dominant Polycystic Kidney Disease.

    Science.gov (United States)

    Murphy, Erin L; Droher, Madeline L; DiMaio, Miriam S; Dahl, Neera K

    2018-03-30

    Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary forms of chronic kidney disease. Mutations within PKD1 or PKD2 lead to innumerable fluid-filled cysts in the kidneys and in some instances, end-stage renal disease (ESRD). Affected individuals have a 50% chance of passing the mutation to each of their offspring. Assisted reproductive technology using preimplantation genetic diagnosis (PGD) allows these individuals to reduce this risk to 1% to 2%. We assess the disease burden of 8 individuals with ADPKD who have undergone genetic testing in preparation for PGD. Clinical features that predict high risk for progression to ESRD in patients with ADPKD include genotype, early onset of hypertension, a urologic event before age 35 years, and a large height-adjusted total kidney volume. Patients may have a family history of intracranial aneurysms or complications involving hepatic cysts, which may further influence the decision to pursue PGD. We also explore the cost, risks, and benefits of using PGD. All patients with ADPKD of childbearing potential, regardless of risk for progression to ESRD or risk for a significant disease burden, will likely benefit from genetic counseling. Copyright © 2018 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  15. Monozygotic twins with CAPN5 autosomal dominant neovascular inflammatory vitreoretinopathy.

    Science.gov (United States)

    Rowell, Hannah A; Bassuk, Alexander G; Mahajan, Vinit B

    2012-01-01

    The purpose of this study was to describe the clinical findings in a set of monozygotic twins with autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) over a 23-year period. A pair of female twins were examined between 26 and 49 years of age. The concordance and discordance of their clinical features were determined. The CAPN5 gene was sequenced using genomic DNA. Both twins of an affected father demonstrated Stage I ADNIV with mild vitreous cells and a negative b-wave on electroretinography. Genetic analysis confirmed a guanine to thymine nucleotide (c.728G>T, pArg243Leu) mutation in the CAPN5 gene. Over the course of 23 years, each twin progressed to stage III disease, showing posterior uveitis, cystoid macular edema, intraocular fibrosis, early retinal neovascularization, retinal degeneration, and cataract. Disease progression varied moderately between each twin and was asymmetrical between eyes. Twin A had 20/70 and 20/125 in the right and left eye, respectively, and underwent vitrectomy surgery and intravitreal injections with bevacizumab for recurrent cystoid macular edema. Twin B maintained 20/20 and 20/40 in the right and left eye, respectively without intervention. There was asymmetry between the eyes and some discordance in the rate of disease progression in these monozygotic twins with ADNIV. The overall high disease concordance suggests genetic factors play a major role in clinical manifestations in CAPN5 vitreoretinopathy.

  16. Penetrating ocular trauma from trampoline spring.

    Science.gov (United States)

    Spokes, David; Siddiqui, Salina; Vize, Colin

    2010-02-01

    The case is presented of a 12-year old boy who sustained severe penetrating ocular trauma while playing on a domestic trampoline. A main spring broke under tension and the hook had struck the eye at high velocity and penetrated the sclera. Primary repair was undertaken but on review it became apparent the eye could not be salvaged. Evisceration was carried out and an orbital implant was placed. Post-operative cosmesis is acceptable. This type of injury has not been reported before. Adult supervision of children on trampolines is recommended to minimise the chance of serious injury.

  17. Canine disorder mirrors human disease: exonic deletion in HES7 causes autosomal recessive spondylocostal dysostosis in miniature Schnauzer dogs.

    Directory of Open Access Journals (Sweden)

    Cali E Willet

    Full Text Available Spondylocostal dysostosis is a congenital disorder of the axial skeleton documented in human families from diverse racial backgrounds. The condition is characterised by truncal shortening, extensive hemivertebrae and rib anomalies including malalignment, fusion and reduction in number. Mutations in the Notch signalling pathway genes DLL3, MESP2, LFNG, HES7 and TBX6 have been associated with this defect. In this study, spondylocostal dysostosis in an outbred family of miniature schnauzer dogs is described. Computed tomography demonstrated that the condition mirrors the skeletal defects observed in human cases, but unlike most human cases, the affected dogs were stillborn or died shortly after birth. Through gene mapping and whole genome sequencing, we identified a single-base deletion in the coding region of HES7. The frameshift mutation causes loss of functional domains essential for the oscillatory transcriptional autorepression of HES7 during somitogenesis. A restriction fragment length polymorphism test was applied within the immediate family and supported a highly penetrant autosomal recessive mode of inheritance. The mutation was not observed in wider testing of 117 randomly sampled adult miniature schnauzer and six adult standard schnauzer dogs; providing a significance of association of Praw = 4.759e-36 (genome-wide significant. Despite this apparently low frequency in the Australian population, the allele may be globally distributed based on its presence in two unrelated sires from geographically distant locations. While isolated hemivertebrae have been observed in a small number of other dog breeds, this is the first clinical and genetic diagnosis of spontaneously occurring spondylocostal dysostosis in a non-human mammal and offers an excellent model in which to study this devastating human disorder. The genetic test can be utilized by dog breeders to select away from the disease and avoid unnecessary neonatal losses.

  18. Canine disorder mirrors human disease: exonic deletion in HES7 causes autosomal recessive spondylocostal dysostosis in miniature Schnauzer dogs.

    Science.gov (United States)

    Willet, Cali E; Makara, Mariano; Reppas, George; Tsoukalas, George; Malik, Richard; Haase, Bianca; Wade, Claire M

    2015-01-01

    Spondylocostal dysostosis is a congenital disorder of the axial skeleton documented in human families from diverse racial backgrounds. The condition is characterised by truncal shortening, extensive hemivertebrae and rib anomalies including malalignment, fusion and reduction in number. Mutations in the Notch signalling pathway genes DLL3, MESP2, LFNG, HES7 and TBX6 have been associated with this defect. In this study, spondylocostal dysostosis in an outbred family of miniature schnauzer dogs is described. Computed tomography demonstrated that the condition mirrors the skeletal defects observed in human cases, but unlike most human cases, the affected dogs were stillborn or died shortly after birth. Through gene mapping and whole genome sequencing, we identified a single-base deletion in the coding region of HES7. The frameshift mutation causes loss of functional domains essential for the oscillatory transcriptional autorepression of HES7 during somitogenesis. A restriction fragment length polymorphism test was applied within the immediate family and supported a highly penetrant autosomal recessive mode of inheritance. The mutation was not observed in wider testing of 117 randomly sampled adult miniature schnauzer and six adult standard schnauzer dogs; providing a significance of association of Praw = 4.759e-36 (genome-wide significant). Despite this apparently low frequency in the Australian population, the allele may be globally distributed based on its presence in two unrelated sires from geographically distant locations. While isolated hemivertebrae have been observed in a small number of other dog breeds, this is the first clinical and genetic diagnosis of spontaneously occurring spondylocostal dysostosis in a non-human mammal and offers an excellent model in which to study this devastating human disorder. The genetic test can be utilized by dog breeders to select away from the disease and avoid unnecessary neonatal losses.

  19. Molecular and clinical study of a cohort of 110 Algerian patients with autosomal recessive ataxia.

    Science.gov (United States)

    Hamza, Wahiba; Ali Pacha, Lamia; Hamadouche, Tarik; Muller, Jean; Drouot, Nathalie; Ferrat, Farida; Makri, Samira; Chaouch, Malika; Tazir, Meriem; Koenig, Michel; Benhassine, Traki

    2015-06-12

    Autosomal recessive cerebellar ataxias (ARCA) are a complex group of neurodegenerative disorders with great genetic and phenotypic heterogeneity, over 30 genes/loci have been associated with more than 20 different clinical forms of ARCA. Genetic heterogeneity combined with highly variable clinical expression of the cerebellar symptoms and overlapping features complicate furthermore the etiological diagnosis of ARCA. The determination of the most frequent mutations and corresponding ataxias, as well as particular features specific to a population, are mandatory to facilitate and speed up the diagnosis process, especially when an appropriate treatment is available. We explored 166 patients (115 families) refered to the neurology units of Algiers central hospitals (Algeria) with a cerebellar ataxia phenotype segregating as an autosomal recessive pattern of inheritance. Genomic DNA was extracted from peripheral blood samples and mutational screening was performed by PCR and direct sequencing or by targeted genomic capture and massive parallel sequencing of 57 genes associated with inherited cerebellar ataxia phenotypes. In this work we report the clinical and molecular results obtained on a large cohort of Algerian patients (110 patients/76 families) with genetically determined autosomal recessive ataxia, representing 9 different types of ARCA and 23 different mutations, including 6 novel ones. The five most common ARCA in this cohort were Friedreich ataxia, ataxia with isolated vitamin E deficiency, ataxia with oculomotor apraxia type 2, autosomal recessive spastic ataxia of Charlevoix-Saguenay and ataxia with oculomotor apraxia type 1. We report here a large cohort of patients with genetically determined autosomal recessive ataxia and the first study of the genetic context of ARCA in Algeria. This study showed that in Algerian patients, the two most common types of ataxia (Friedreich ataxia and ataxia with isolated vitamin E deficiency) coexist with forms that may be

  20. Penetrating chest injury: A miraculous life salvage

    Directory of Open Access Journals (Sweden)

    Santosh B Dalavi

    2013-01-01

    Full Text Available An unusual penetrating chest injury was caused by high velocity road traffic accident. An 18-year-old had a four wheeler accident and was brought in emergency department with a ′bamboo′ stick on the left side chest exiting through back. After the stabilization of vital parameters, an inter-costal tube drainage was done on the left side. Except the minor brochopleural fistula which healed by 10 th day, his recovery was uneventful. The outcome was consistent with current aggressive management of penetrating chest injuries. Management of penetrating chest injury involving pulmonary trauma is based on three principles. One is stabilization of hemodynamics of patient with proper clinical evaluation. Second, a mere intercostal tube drainage sufficient for majority of the cases. Third, post-operative active as well as passive physiotherapy is necessary for speedy recovery.

  1. Physical Penetration Testing: A Whole New Story in Penetration Testing

    NARCIS (Netherlands)

    Dimkov, T.; Pieters, Wolter

    2011-01-01

    Physical penetration testing plays an important role in assuring a company that the security policies are properly enforced and that the security awareness of the employees is on the appropriate level. In physical penetration tests the tester physically enters restricted locations and directly

  2. Influence of fillers on hydrogen penetration properties and blister fracture of rubber composites for O-ring exposed to high-pressure hydrogen gas

    Energy Technology Data Exchange (ETDEWEB)

    Yamabe, Junichiro; Nishimura, Shin [Department of Mechanical Science Engineering, Kyushu University, 744 Motooka, Nishi-ku, Fukuoka 819-0395 (Japan); Research Center for Hydrogen Industrial Use and Storage (HYDROGENIUS), National Institute of Advanced Industrial Science and Technology (AIST), 744 Motooka, Nishi-ku, Fukuoka 819-0395 (Japan)

    2009-02-15

    Ethylene-propylene rubber (EPDM) and nitrile-butadiene rubber (NBR) composites having carbon black, silica, and no fillers were exposed to hydrogen gas at a maximum pressure of 10 MPa; then, blister tests and the measurement of hydrogen content were conducted. The hydrogen contents of the composites were proportional to the hydrogen pressure, i.e., the behavior of their hydrogen contents follows Henry's law. This implies that hydrogen penetrates into the composite as a hydrogen molecule. The addition of carbon black raised the hydrogen content of the composite, while the addition of silica did not. Based on observations, the blister damages of composites with silica were less pronounced, irrespective of the hydrogen pressures. This may be attributed to their lower hydrogen content and relatively better tensile properties than the others. (author)

  3. Molecular evolution of a Y chromosome to autosome gene duplication in Drosophila.

    Science.gov (United States)

    Dyer, Kelly A; White, Brooke E; Bray, Michael J; Piqué, Daniel G; Betancourt, Andrea J

    2011-03-01

    In contrast to the rest of the genome, the Y chromosome is restricted to males and lacks recombination. As a result, Y chromosomes are unable to respond efficiently to selection, and newly formed Y chromosomes degenerate until few genes remain. The rapid loss of genes from newly formed Y chromosomes has been well studied, but gene loss from highly degenerate Y chromosomes has only recently received attention. Here, we identify and characterize a Y to autosome duplication of the male fertility gene kl-5 that occurred during the evolution of the testacea group species of Drosophila. The duplication was likely DNA based, as other Y-linked genes remain on the Y chromosome, the locations of introns are conserved, and expression analyses suggest that regulatory elements remain linked. Genetic mapping reveals that the autosomal copy of kl-5 resides on the dot chromosome, a tiny autosome with strongly suppressed recombination. Molecular evolutionary analyses show that autosomal copies of kl-5 have reduced polymorphism and little recombination. Importantly, the rate of protein evolution of kl-5 has increased significantly in lineages where it is on the dot versus Y linked. Further analyses suggest this pattern is a consequence of relaxed purifying selection, rather than adaptive evolution. Thus, although the initial fixation of the kl-5 duplication may have been advantageous, slightly deleterious mutations have accumulated in the dot-linked copies of kl-5 faster than in the Y-linked copies. Because the dot chromosome contains seven times more genes than the Y and is exposed to selection in both males and females, these results suggest that the dot suffers the deleterious effects of genetic linkage to more selective targets compared with the Y chromosome. Thus, a highly degenerate Y chromosome may not be the worst environment in the genome, as is generally thought, but may in fact be protected from the accumulation of deleterious mutations relative to other nonrecombining

  4. Population data of 19 autosomal STR loci in the Li population from Hainan Province in southernmost China.

    Science.gov (United States)

    Fan, Haoliang; Wang, Xiao; Ren, Zheng; He, Guanglin; Long, Ren; Liang, Anwen; Song, Tao; Deng, Jianqiang

    2018-03-20

    In the present study, population data of 19 autosomal STR loci included in the Goldeneye™ DNA ID System 20A in 653 Li individuals was obtained and population genetic relationships among 13 populations were investigated. MDS and phylogenetic analysis suggested that the Hainan Li population kept a close genetic relationship with the Chinese Han populations, especially for Southern Han populations (Guangdong Han, Sichuan Han, and Hunan Han). Our results indicated that the 19 autosomal STRs are highly discriminative and polymorphic in the Hainan Li population suitable for personal forensic identification and paternity testing.

  5. Autosomal Recessive Polycystic Kidney Disease: Antenatal Diagnosis and Histopathological Correlation

    Directory of Open Access Journals (Sweden)

    Dayananda Kumar Rajanna

    2013-01-01

    Full Text Available Autosomal recessive polycystic kidney disease (ARPKD is one of the most common inheritable disease manifesting in infancy and childhood with a frequency of 1:6,000 to 1:55,000 births. The patient in her second trimester presented with a history of amenorrhea. Ultrasound examination revealed bilateral, enlarged, hyperechogenic kidneys, placentomegaly, and severe oligohydramnios. The pregnancy was terminated. An autopsy was performed on the fetus. Both the kidneys were found to be enlarged and the cut surface showed numerous cysts. The liver sections showed changes due to fibrosis. The final diagnosis of autosomal recessive polycystic kidney disease was made based on these findings. In this article, we correlate the ante-natal ultrasound and histopathological findings in autosomal recessive polycystic kidney disease.

  6. The penetrating depth analysis of Lunar Penetrating Radar onboard Chang’e-3 rover

    Science.gov (United States)

    Xing, Shu-Guo; Su, Yan; Feng, Jian-Qing; Dai, Shun; Xiao, Yuan; Ding, Chun-Yu; Li, Chun-Lai

    2017-04-01

    Lunar Penetrating Radar (LPR) has successfully been used to acquire a large amount of scientific data during its in-situ detection. The analysis of penetrating depth can help to determine whether the target is within the effective detection range and contribute to distinguishing useful echoes from noise. First, this study introduces two traditional methods, both based on a radar transmission equation, to calculate the penetrating depth. The only difference between the two methods is that the first method adopts system calibration parameters given in the calibration report and the second one uses high-voltage-off radar data. However, some prior knowledge and assumptions are needed in the radar equation and the accuracy of assumptions will directly influence the final results. Therefore, a new method termed the Correlation Coefficient Method (CCM) is provided in this study, which is only based on radar data without any a priori assumptions. The CCM can obtain the penetrating depth according to the different correlation between reflected echoes and noise. To be exact, there is a strong correlation in the useful reflected echoes and a random correlation in the noise between adjacent data traces. In addition, this method can acquire a variable penetrating depth along the profile of the rover, but only one single depth value can be obtained from traditional methods. Through a simulation, the CCM has been verified as an effective method to obtain penetration depth. The comparisons and analysis of the calculation results of these three methods are also implemented in this study. Finally, results show that the ultimate penetrating depth of Channel 1 and the estimated penetrating depth of Channel 2 range from 136.9 m to 165.5 m ({\\varepsilon }r=6.6) and from 13.0 m to 17.5 m ({\\varepsilon }r=2.3), respectively.

  7. Autosomal recessive anhidrotic ectodermal dysplasia: A rare entity

    Directory of Open Access Journals (Sweden)

    Sangita Ghosh

    2014-01-01

    Full Text Available We describe a case of anhidrotic ectodermal dysplasia (AED with an autosomal recessive mode of inheritance, a very rare entity, in a 2-year-old female child of two asymptomatic, consanguineous parents. Their previous child also had a similar condition. Autosomal recessive AED (AR-AED can have its full expression both in males and females and it is clinically indistinguishable from the x-linked recessive AED (XL-AED, which is the most common type of ectodermal dysplasia. Unlike the partially symptomatic carriers of XL-AED, the heterozygotes of AR-AED are phenotypically asymptomatic.

  8. Penetration of Photovoltaics in Greece

    Directory of Open Access Journals (Sweden)

    Eugenia Giannini

    2015-06-01

    Full Text Available Recently, an interesting experiment was completed in Greece concerning photovoltaic penetration into the electricity production sector. Based on the relevant laws and in accordance to the related European directives, an explosive penetration process was completed in less than three years, resulting in a 7% share of photovoltaics in electricity production instead of the previous negligible share. The legislation was based on licensing simplification and generous feed-in-tariffs. This approach transformed photovoltaic technology from a prohibitively expensive to a competitive one. This work aims to summarize the relevant legislation and illustrate its effect on the resulting penetration. A sigmoid-shape penetration was observed which was explained by a pulse-type driving force. The return on investment indicator was proposed as an appropriate driving force, which incorporates feed-in-tariffs and turnkey-cost. Furthermore, the resulting surcharge on the electricity price due to photovoltaic penetration was also analyzed.

  9. Development Of The Nuclear Optical Penetration

    Science.gov (United States)

    Inoue, K.; Koike, K.; Imada, Y.

    1984-10-01

    We have developed the nuclear optical penetration to be incorporated in the wall penetration of the shell to introduce a data transmission system using optical fibers into a nuclear power plant with a pressurized water reactor. Radiation-induced coloration in optical glass seriously affects transmission characteristics of optical fibers, whereas it has been revealed that the pure-silica core optical fiber without any dopant in the core has wide applicability in radiation fields thanks to its very low radiation-induced attenuation. The wall penetration of the shell should have airtightness and resistivity to heat, vibration, and pressure, let alone radiation, excellent enough to be invariable in data transmission efficiency even when subjected to severe environmental tests. The sealing modules of this newly developed nuclear optical penetration are hermetically sealed. The gap between the optical fiber rod (100 pm in core diameter and 5 mm in rod diameter) and stainless steel tube is sealed with lamingted glass layer. As the result of He gas leakage test, high airtightness of less than 10 cc/sec was achieved. No thermal deformation of the core was caused by sealing with laminated glass layer, nor was observed transmission loss. Then the sealiing modules were subjected to the irradiation test using 60 Co gamma ray exposure of 2 x 10 rads. Though silica glass layer supporting the fiber rod and sealing glass portion turned blackish purple, transparency of the fiber was not affected. Only less than 0.5 dB of connecting loss was observed at the connecting point with the optical fiber cable. The sealing modules were also found to have resistivity to vibration and pressure as excellent as that of existing nuclear electric penetrations. We expect the nuclear optical fiber penetration will be much effective in improving reliability of data transmission systems using optical fibers in radiation fields.

  10. A comparison of base deficit and vital signs in the early assessment of patients with penetrating trauma in a high burden setting.

    Science.gov (United States)

    Dunham, Mark Peter; Sartorius, Benn; Laing, Grant Llewellyn; Bruce, John Lambert; Clarke, Damian Luiz

    2017-09-01

    An assessment of physiological status is a key step in the early assessment of trauma patients with implications for triage, investigation and management. This has traditionally been done using vital signs. Previous work from large European trauma datasets has suggested that base deficit (BD) predicts clinically important outcomes better than vital signs (VS). A BD derived classification of haemorrhagic shock appeared superior to one based on VS derived from ATLS criteria in a population of predominantly blunt trauma patients. The initial aim of this study was to see if this observation would be reproduced in penetrating trauma patients. The power of each individual variable (BD, heart rate (HR), systolic blood pressure (SBP), shock index(SI) (HR/SBP) and Glasgow Coma Score (GCS)) to predict mortality was then also compared. A retrospective analysis of adult trauma patients presenting to the Pietermaritzburg Metropolitan Trauma Service was performed. Patients were classified into four "shock" groups using VS or BD and the outcomes compared. Receiver Operator Characteristic (ROC) curves were then generated to compare the predictive power for mortality of each individual variable. 1863 patients were identified. The overall mortality rate was 2.1%. When classified by BD, HR rose and SBP fell as the "shock class" increased but not to the degree suggested by the ATLS classification. The BD classification of haemorrhagic shock appeared to predict mortality better than that based on the ATLS criteria. Mortality increased from 0.2% (Class 1) to 19.7% (Class 4) based on the 4 level BD classification. Mortality increased from 0.3% (Class 1) to 12.6% (Class 4) when classified based by VS. Area under the receiver operator characteristic (AUROC) curve analysis of the individual variables demonstrated that BD predicted mortality significantly better than HR, GCS, SBP and SI. AUROC curve (95% Confidence Interval (CI)) for BD was 0.90 (0.85-0.95) compared to HR 0

  11. Variation of autosomes and X chromosome STR in breast cancer and gynecological cancer tissues

    Directory of Open Access Journals (Sweden)

    Hou Youxiang

    2017-04-01

    Full Text Available This study analyses 1000 cases of patients with breast cancer and 2000 cases of patients with gynecological cancer (1000 cases of malignant tumor, 1000 cases of benign tumors, where breast cancer and malignant tumor patients comprise the observation group, while patients with benign tumors comprise the control group. Through DNA extraction, STR genotyping and variation verification, microdissection, individual STR mutation rate and loci STR mutation rate of the two groups of patients were calculated. Results show that there are no significant (P > 0.05 differences in the STR variation of autosomes and X chromosome between patients in the observation group and those in the reference group. However, significant (P < 0.05 intergroup differences were found for STR variation typing between patients with malignant and benign tumors. Using STR genotyping for autosomes and X chromosomes, gynecological cancer patients were found to be more likely to mutate, with a clear relationship between STR variation and tumor differentiation degrees. The study on the variation analysis of autosomes and X chromosome STR in breast and gynecological cancer tissues is expected to have a high application value when applied to medical research and identification processes.

  12. Further evidence for P59L mutation in GJA3 associated with autosomal dominant congenital cataract

    Directory of Open Access Journals (Sweden)

    Li Wang

    2016-01-01

    Full Text Available Context: Congenital cataracts are one of the common eye disorders leading to visual impairment or blindness in children worldwide. We found a Chinese family with autosomal dominant pulverulent cataract. Aims: To identify the pathogenic gene mutation in a Chinese family with autosomal dominant inherited pulverulent cataract. Subjects and Methods: After obtained informed consent, detailed ophthalmic examinations were carried out; genomic DNAs were obtained from seven family members in a three-generation Chinese family with three affected. All exons of candidate genes were amplified by polymerase chain reaction and were sequenced performed by bidirectional sequencing. Results: By sequencing the encoding regions of the candidate genes, a missense mutation (c. 176C>T was detected in gap junction protein alpha 3 genes (GJA3, which resulted in the substitution of highly conserved proline by leucine at codon 59 (p.P59L. The mutation co-segregated with all patients and was absent in 100 normal Chinese controls. Conclusions: The study identified a missense mutation (c. 176C>T in GJA3 gene associated with autosomal dominant congenital pulverulent cataract in a Chinese family. It gave further evidence of phenotype heterogeneity for P59L mutation in GJA3 associated with congenital cataract.

  13. Genetic architecture of autosome-mediated hybrid male sterility in Drosophila.

    Science.gov (United States)

    Marín, I

    1996-04-01

    Several estimators have been developed for assessing the number of sterility factors in a chromosome based on the sizes of fertile and sterile introgressed fragments. Assuming that two factors are required for producing sterility, simulations show that one of these, twice the inverse of the relative size of the largest fertile fragment, provides good average approximations when as few as five fertile fragments are analyzed. The estimators have been used for deducing the number of factors from previous data on several pairs of species. A particular result contrasts with the authors' interpretations: instead of the high number of sterility factors suggested, only a few per autosome are estimated in both reciprocal crosses involving Drosophila buzzatii and D. koepferae. It has been possible to map these factors, between three and six per chromosome, in the autosomes 3 and 4 of these species. Out of 203 introgressions of different fragments or combinations of fragments, the outcome of at least 192 is explained by the mapped zones. These results suggest that autosome-mediated sterility in the male hybrids of these species is mediated by a few epistatic factors, similarly to X-mediated sterility in the hybrids of other Drosophila species.

  14. Deep ocean model penetrator experiments

    International Nuclear Information System (INIS)

    Freeman, T.J.; Burdett, J.R.F.

    1986-01-01

    Preliminary trials of experimental model penetrators in the deep ocean have been conducted as an international collaborative exercise by participating members (national bodies and the CEC) of the Engineering Studies Task Group of the Nuclear Energy Agency's Seabed Working Group. This report describes and gives the results of these experiments, which were conducted at two deep ocean study areas in the Atlantic: Great Meteor East and the Nares Abyssal Plain. Velocity profiles of penetrators of differing dimensions and weights have been determined as they free-fell through the water column and impacted the sediment. These velocity profiles are used to determine the final embedment depth of the penetrators and the resistance to penetration offered by the sediment. The results are compared with predictions of embedment depth derived from elementary models of a penetrator impacting with a sediment. It is tentatively concluded that once the resistance to penetration offered by a sediment at a particular site has been determined, this quantity can be used to sucessfully predict the embedment that penetrators of differing sizes and weights would achieve at the same site

  15. Autosomal dominant frontometaphyseal dysplasia : Delineation of the clinical phenotype

    NARCIS (Netherlands)

    Wade, Emma M.; Jenkins, Zandra A.; Daniel, Philip B.; Morgan, Tim; Addor, Marie C.; Ades, Lesley C.; Bertola, Debora; Bohring, Axel; Carter, Erin; Cho, Tae-Joon; de Geus, Christa M.; Duba, Hans-Christoph; Fletcher, Elaine; Hadzsiev, Kinga; Hennekam, Raoul C. M.; Kim, Chong A.; Krakow, Deborah; Morava, Eva; Neuhann, Teresa; Sillence, David; Superti-Furga, Andrea; Veenstra-Knol, Hermine E.; Wieczorek, Dagmar; Wilson, Louise C.; Markie, David M.; Robertson, Stephen P.

    Frontometaphyseal dysplasia (FMD) is caused by gain-of-function mutations in the X-linked gene FLNA in approximately 50% of patients. Recently we characterized an autosomal dominant form of FMD (AD-FMD) caused by mutations in MAP3K7, which accounts for the condition in the majority of patients who

  16. Autosomal dominant frontometaphyseal dysplasia: Delineation of the clinical phenotype

    NARCIS (Netherlands)

    Wade, Emma M.; Jenkins, Zandra A.; Daniel, Philip B.; Morgan, Tim; Addor, Marie C.; Adés, Lesley C.; Bertola, Debora; Bohring, Axel; Carter, Erin; Cho, Tae-Joon; de Geus, Christa M.; Duba, Hans-Christoph; Fletcher, Elaine; Hadzsiev, Kinga; Hennekam, Raoul C. M.; Kim, Chong A.; Krakow, Deborah; Morava, Eva; Neuhann, Teresa; Sillence, David; Superti-Furga, Andrea; Veenstra-Knol, Hermine E.; Wieczorek, Dagmar; Wilson, Louise C.; Markie, David M.; Robertson, Stephen P.

    2017-01-01

    Frontometaphyseal dysplasia (FMD) is caused by gain-of-function mutations in the X-linked gene FLNA in approximately 50% of patients. Recently we characterized an autosomal dominant form of FMD (AD-FMD) caused by mutations in MAP3K7, which accounts for the condition in the majority of patients who

  17. Genetics Home Reference: autosomal dominant partial epilepsy with auditory features

    Science.gov (United States)

    ... for This Condition ADLTE ADPEAF Autosomal dominant lateral temporal lobe epilepsy Epilepsy, partial, with auditory features ETL1 Related Information ... W, Nakken KO, Fischer C, Steinlein OK. Familial temporal lobe epilepsy with aphasic seizures and linkage to chromosome 10q22- ...

  18. Two novel mutations in ILDR1 gene cause autosomal recessive ...

    Indian Academy of Sciences (India)

    In a recent screening programme on hearing loss (HL), we examined 17 common autosomal recessive nonsyndromic hearing loss (ARNSHL) genes in every consanguineous Ira- nian family with ARNSHL that was referred to our centre. We first screened GJB2 mutations and then utilized a panel of three to four short ...

  19. A further patient with Pai syndrome with autosomal dominant inheritance?

    OpenAIRE

    Rudnik-Schöneborn, S; Zerres, K

    1994-01-01

    We report a patient with median cleft of the upper lip, cutaneous facial polyps, and lipoma of the corpus callosum who represents a further case of Pai syndrome. The father of the patient showed coloboma of the right iris and shared some facial dysmorphism with his son, thus raising the question of autosomal dominant inheritance.

  20. 3. Pattern of Inheritance of Autosome and Sex. Chromosome Linked ...

    Indian Academy of Sciences (India)

    Home; Journals; Resonance – Journal of Science Education; Volume 4; Issue 10. Teaching and Learning Genetics with Drosophila – Pattern of Inheritance of Autosome and Sex Chro-mosome Linked Genes/Characters. H A Ranganath M T Tanuja. Classroom Volume 4 Issue 10 October 1999 pp 78-87 ...

  1. SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis.

    Science.gov (United States)

    Orlacchio, Antonio; Babalini, Carla; Borreca, Antonella; Patrono, Clarice; Massa, Roberto; Basaran, Sarenur; Munhoz, Renato P; Rogaeva, Ekaterina A; St George-Hyslop, Peter H; Bernardi, Giorgio; Kawarai, Toshitaka

    2010-02-01

    The mutation of the spatacsin gene is the single most common cause of autosomal recessive hereditary spastic paraplegia with thin corpus callosum. Common clinical, pathological and genetic features between amyotrophic lateral sclerosis and hereditary spastic paraplegia motivated us to investigate 25 families with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival for mutations in the spatascin gene. The inclusion criterion was a diagnosis of clinically definite amyotrophic lateral sclerosis according to the revised El Escorial criteria. The exclusion criterion was a diagnosis of hereditary spastic paraplegia with thin corpus callosum in line with an established protocol. Additional pathological and genetic evaluations were also performed. Surprisingly, 12 sequence alterations in the spatacsin gene (one of which is novel, IVS30 + 1 G > A) were identified in 10 unrelated pedigrees with autosomal recessive juvenile amyotrophic lateral sclerosis and long-term survival. The countries of origin of these families were Italy, Brazil, Canada, Japan and Turkey. The variants seemed to be pathogenic since they co-segregated with the disease in all pedigrees, were absent in controls and were associated with amyotrophic lateral sclerosis neuropathology in one member of one of these families for whom central nervous system tissue was available. Our study indicates that mutations in the spatascin gene could cause a much wider spectrum of clinical features than previously recognized, including autosomal recessive juvenile amyotrophic lateral sclerosis.

  2. Case report: Autosomal dominant non-epidermolytic palmoplantar ...

    African Journals Online (AJOL)

    Palmoplantar keratoderma (PPK) is a hereditary cutaneous disorder characterized by a marked hyperkeratosis of the palms and soles. A variant that was inherited in an autosomal dominant form was highlighted in a 20-month-old girl-child. The proband was brought to the Paediatric Outpatient Department by her mother ...

  3. Penetration of Photovoltaics in Greece

    OpenAIRE

    Eugenia Giannini; Antonia Moropoulou; Zacharias Maroulis; Glykeria Siouti

    2015-01-01

    Recently, an interesting experiment was completed in Greece concerning photovoltaic penetration into the electricity production sector. Based on the relevant laws and in accordance to the related European directives, an explosive penetration process was completed in less than three years, resulting in a 7% share of photovoltaics in electricity production instead of the previous negligible share. The legislation was based on licensing simplification and generous feed-in-tariffs. This approach ...

  4. Projectile penetration into ballistic gelatin.

    Science.gov (United States)

    Swain, M V; Kieser, D C; Shah, S; Kieser, J A

    2014-01-01

    Ballistic gelatin is frequently used as a model for soft biological tissues that experience projectile impact. In this paper we investigate the response of a number of gelatin materials to the penetration of spherical steel projectiles (7 to 11mm diameter) with a range of lower impacting velocities (projectile velocity are found to be linear for all systems above a certain threshold velocity required for initiating penetration. The data for a specific material impacted with different diameter spheres were able to be condensed to a single curve when the penetration depth was normalised by the projectile diameter. When the results are compared with a number of predictive relationships available in the literature, it is found that over the range of projectiles and compositions used, the results fit a simple relationship that takes into account the projectile diameter, the threshold velocity for penetration into the gelatin and a value of the shear modulus of the gelatin estimated from the threshold velocity for penetration. The normalised depth is found to fit the elastic Froude number when this is modified to allow for a threshold impact velocity. The normalised penetration data are found to best fit this modified elastic Froude number with a slope of 1/2 instead of 1/3 as suggested by Akers and Belmonte (2006). Possible explanations for this difference are discussed. © 2013 Published by Elsevier Ltd.

  5. HMO penetration and the geographic mobility of practicing physicians.

    Science.gov (United States)

    Polsky, D; Kletke, P R; Wozniak, G D; Escarce, J J

    2000-09-01

    In this study, we assessed the influence of changes in health maintenance organization (HMO) penetration on the probability that established patient care physicians relocated their practices or left patient care altogether. For physicians who relocated their practices, we also assessed the impact of HMO penetration on their destination choices. We found that larger increases in HMO penetration decreased the probability that medical/surgical specialists in early career stayed in patient care in the same market, but had no impact on generalists, hospital-based specialists, or mid career medical/surgical specialists. We also found that physicians who relocated their practices were much more likely to choose destination markets with the same level of HMO penetration or lower HMO penetration compared with their origin markets than they were to choose destination markets with higher HMO penetration. The largely negligible impact of changes in HMO penetration on established physicians' decisions to relocate their practices or leave patient care is consistent with high relocation and switching costs. Relocating physicians' attraction to destination markets with the same level of HMO penetration as their origin markets suggests that, while physicians' styles of medical practice may adapt to changes in market conditions, learning new practice styles is costly.

  6. A Study on Factors Affecting Airborne LiDAR Penetration

    Directory of Open Access Journals (Sweden)

    Wei-Chen Hsu

    2015-01-01

    Full Text Available This study uses data from different periods, areas and parameters of airborne LiDAR (light detection and ranging surveys to understand the factors that influence airborne LiDAR penetration rate. A discussion is presented on the relationships between these factors and LiDAR penetration rate. The results show that the flight height above ground level (AGL does not have any relationship with the penetration rate. There are some factors that should have larger influence. For example, the laser is affected by a wet ground surface by reducing the number of return echoes. The field of view (FOV has a slightly negative correlation with the penetration rate, which indicates that the laser incidence angle close to zero should achieve the best penetration. The vegetation cover rate also shows a negative correlation with the penetration rate, thus bare ground and reduced vegetation in the aftermath of a typhoon also cause high penetration rate. More return echoes could be extracted from the full-waveform system, thereby effectively improving the penetration rate. This study shows that full-waveform LiDAR is an effective method for increasing the number of surface reflected echoes. This study suggests avoiding LiDAR survey employment directly following precipitation to prevent laser echo reduction.

  7. Large scale wind power penetration in Denmark

    DEFF Research Database (Denmark)

    Karnøe, Peter

    2013-01-01

    he Danish electricity generating system prepared to adopt nuclear power in the 1970s, yet has become the world's front runner in wind power with a national plan for 50% wind power penetration by 2020. This paper deploys a sociotechnical perspective to explain the historical transformation...... of "networks of power" via the interactions of politics, the techno-physics of electrons, and the market setting. The Danish case is about how an assemblage of new agencies has reorganized and reshaped society by building a new sociotechnical network. This has rendered developments highly unpredictable...... and highly experimental. The transformation process can be followed through the way successive technical engineering reports have represented the challenges associated with the penetration of wind power. The iteration shows how novel technical phenomena emerge and are assimilated, and how new engineering...

  8. Validation of a combined autosomal/Y-chromosomal STR approach for analyzing typical biological stains in sexual-assault cases.

    Science.gov (United States)

    Purps, Josephine; Geppert, Maria; Nagy, Marion; Roewer, Lutz

    2015-11-01

    DNA testing is an established part of the investigation and prosecution of sexual assault. The primary purpose of DNA evidence is to identify a suspect and/or to demonstrate sexual contact. However, due to highly uneven proportions of female and male DNA in typical stains, routine autosomal analysis often fails to detect the DNA of the assailant. To evaluate the forensic efficiency of the combined application of autosomal and Y-chromosomal short tandem repeat (STR) markers, we present a large retrospective casework study of probative evidence collected in sexual-assault cases. We investigated up to 39 STR markers by testing combinations of the 16-locus NGMSElect kit with both the 23-locus PowerPlex Y23 and the 17-locus Yfiler kit. Using this dual approach we analyzed DNA extracts from 2077 biological stains collected in 287 cases over 30 months. To assess the outcome of the combined approach in comparison to stand-alone autosomal analysis we evaluated informative DNA profiles. Our investigation revealed that Y-STR analysis added up to 21% additional, highly informative (complete, single-source) profiles to the set of reportable autosomal STR profiles for typical stains collected in sexual-assault cases. Detection of multiple male contributors was approximately three times more likely with Y-chromosomal profiling than with autosomal STR profiling. In summary, 1/10 cases would have remained inconclusive (and could have been dismissed) if Y-STR analysis had been omitted from DNA profiling in sexual-assault cases. Copyright © 2015 The Authors. Published by Elsevier Ireland Ltd.. All rights reserved.

  9. Autosomal recessive osteopetrosis with a unique imaging finding: multiple encephaloceles

    International Nuclear Information System (INIS)

    Saglam, Dilek; Bilgici, Meltem Ceyhan; Bekci, Tuemay; Albayrak, Canan; Albayrak, Davut

    2017-01-01

    Osteopetrosis is a hereditary form of sclerosing bone dysplasia with various radiological and clinical presentations. The autosomal recessive type, also known as malignant osteopetrosis, is the most severe type, with the early onset of manifestations. A 5-month-old infant was admitted to our hospital with recurrent respiratory tract infections. Chest X-ray and skeletal survey revealed the classic findings of osteopetrosis, including diffuse osteosclerosis and bone within a bone appearance. At follow-up, the patient presented with, thickened calvarium, multiple prominent encephaloceles, and dural calcifications leading to the intracranial clinical manifestations with bilateral hearing and sight loss. Autosomal recessive osteopetrosis is one of the causes of encephaloceles and this finding may become dramatic if untreated. (orig.)

  10. Autosomal recessive osteopetrosis with a unique imaging finding: multiple encephaloceles

    Energy Technology Data Exchange (ETDEWEB)

    Saglam, Dilek; Bilgici, Meltem Ceyhan; Bekci, Tuemay [Ondokuz Mayis University, Department of Radiology, School of Medicine, Kurupelit, Samsun (Turkey); Albayrak, Canan; Albayrak, Davut [Ondokuz Mayis University, Department of Pediatrics, School of Medicine, Kurupelit, Samsun (Turkey)

    2017-05-15

    Osteopetrosis is a hereditary form of sclerosing bone dysplasia with various radiological and clinical presentations. The autosomal recessive type, also known as malignant osteopetrosis, is the most severe type, with the early onset of manifestations. A 5-month-old infant was admitted to our hospital with recurrent respiratory tract infections. Chest X-ray and skeletal survey revealed the classic findings of osteopetrosis, including diffuse osteosclerosis and bone within a bone appearance. At follow-up, the patient presented with, thickened calvarium, multiple prominent encephaloceles, and dural calcifications leading to the intracranial clinical manifestations with bilateral hearing and sight loss. Autosomal recessive osteopetrosis is one of the causes of encephaloceles and this finding may become dramatic if untreated. (orig.)

  11. Evidence for autosomal dominant inheritance of ablepharon-macrostomia syndrome.

    Science.gov (United States)

    Rohena, Luis; Kuehn, Devon; Marchegiani, Shannon; Higginson, Jason D

    2011-04-01

    Ablepharon-macrostomia syndrome (AMS) is characterized by absent or short eyelids, macrostomia, ear anomalies, absent lanugo and hair, redundant skin, abnormal genitalia, and developmental delay in two-thirds of the reported patients. Additional anomalies include dry skin, growth retardation, hearing loss, camptodactyly, hypertelorism, absent zygomatic arches, and umbilical abnormalities. We present the second familial case of ablepharon-macrostomia syndrome in a newborn female and her 22-year-old father making autosomal dominant inheritance more likely than the previously proposed autosomal recessive transmission for this disorder. These cases likely represent the 16th and 17th reported cases of AMS and the first case suspected on prenatal ultrasound. Additionally, the child shows more prominent features of the disorder when compared to her father documenting variable expression and possible anticipation. This article is a US Government work and, as such, is in the public domain in the United States of America. Published 2011 Wiley-Liss, Inc.

  12. Autosomal recessive progressive myoclonus epilepsy due to impaired ceramide synthesis.

    Science.gov (United States)

    Ferlazzo, Edoardo; Striano, Pasquale; Italiano, Domenico; Calarese, Tiziana; Gasparini, Sara; Vanni, Nicola; Fruscione, Floriana; Genton, Pierre; Zara, Federico

    2016-09-01

    Autosomal recessive progressive myoclonus epilepsy due to impaired ceramide synthesis is an extremely rare condition, so far reported in a single family of Algerian origin presenting an unusual, severe form of progressive myoclonus epilepsy characterized by myoclonus, generalized tonic-clonic seizures and moderate to severe cognitive impairment, with probable autosomal recessive inheritance. Disease onset was between 6 and 16 years of age. Genetic study allowed to identify a homozygous nonsynonymous mutation in CERS1, the gene encoding ceramide synthase 1, a transmembrane protein of the endoplasmic reticulum (ER), catalyzes the biosynthesis of C18-ceramides. The mutation decreased C18-ceramide levels. In addition, downregulation of CerS1 in neuroblastoma cell line showed activation of ER stress response and induction of proapoptotic pathways. This observation demonstrates that impairment of ceramide biosynthesis underlies neurodegeneration in humans.

  13. Plutonium in depleted uranium penetrators

    International Nuclear Information System (INIS)

    McLaughlin, J.P.; Leon-Vintro, L.; Smith, K.; Mitchell, P.I.; Zunic, Z.S.

    2002-01-01

    Depleted Uranium (DU) penetrators used in the recent Balkan conflicts have been found to be contaminated with trace amounts of transuranic materials such as plutonium. This contamination is usually a consequence of DU fabrication being carried out in facilities also using uranium recycled from spent military and civilian nuclear reactor fuel. Specific activities of 239+240 Plutonium generally in the range 1 to 12 Bq/kg have been found to be present in DU penetrators recovered from the attack sites of the 1999 NATO bombardment of Kosovo. A DU penetrator recovered from a May 1999 attack site at Bratoselce in southern Serbia and analysed by University College Dublin was found to contain 43.7 +/- 1.9 Bq/kg of 239+240 Plutonium. This analysis is described. An account is also given of the general population radiation dose implications arising from both the DU itself and from the presence of plutonium in the penetrators. According to current dosimetric models, in all scenarios considered likely ,the dose from the plutonium is estimated to be much smaller than that due to the uranium isotopes present in the penetrators. (author)

  14. Mutations in DNMT1 cause autosomal dominant cerebellar ataxia, deafness and narcolepsy

    DEFF Research Database (Denmark)

    Winkelmann, Juliane; Lin, Ling; Schormair, Barbara

    2012-01-01

    to HDAC2. It is also highly expressed in immune cells and required for the differentiation of CD4+ into T regulatory cells. Mutations in exon 20 of this gene were recently reported to cause hereditary sensory neuropathy with dementia and hearing loss (HSAN1). Our mutations are all located in exon 21......Autosomal dominant cerebellar ataxia, deafness and narcolepsy (ADCA-DN) is characterized by late onset (30-40 years old) cerebellar ataxia, sensory neuronal deafness, narcolepsy-cataplexy and dementia. We performed exome sequencing in five individuals from three ADCA-DN kindreds and identified DNMT...

  15. Genetic polymorphisms of 20 autosomal STR loci in the Vietnamese population from Yunnan Province, Southwest China.

    Science.gov (United States)

    Zhang, Xiufeng; Hu, Liping; Du, Lei; Nie, Aiting; Rao, Min; Pang, Jing Bo; Nie, Shengjie

    2017-05-01

    The genetic polymorphisms of 20 autosomal short tandem repeat (STR) loci included in the PowerPlex® 21 kit were evaluated in 522 healthy unrelated Vietnamese from Yunnan, China. All of the loci reached the Hardy-Weinberg equilibrium. These loci were examined to determine allele frequencies and forensic statistical parameters. The combined discrimination power and probability of excluding paternity of the 20 STR loci were 0.999999999999999999999991 26 and 0.999999975, respectively. Results suggested that the 20 STR loci are highly polymorphic, which is suitable for forensic personal identification and paternity testing.

  16. Mutations in AAGAB underlie autosomal dominant punctate palmoplantar keratoderma.

    Science.gov (United States)

    Dinani, N; Ali, M; Liu, L; McGrath, J; Mellerio, J

    2017-04-01

    Punctate palmoplantar keratoderma type 1 (PPPK1) is a rare autosomal dominant inherited skin disease, characterized by multiple hyperkeratotic lesions on the palms and soles. The causative gene for PPPK1 has been identified as AAGAB, which encodes α- and γ-adaptin-binding protein p34. We describe the clinical features in three unrelated families with PPPK1, and report three recurrent causative mutations in AAGAB. © 2017 British Association of Dermatologists.

  17. Allele frequency distribution for 21 autosomal STR loci in Nepal.

    Science.gov (United States)

    Kraaijenbrink, T; van Driem, G L; Opgenort, J R M L; Tuladhar, N M; de Knijff, P

    2007-05-24

    The allele frequency distributions of 21 autosomal loci contained in the AmpFlSTR Identifiler, the Powerplex 16 and the FFFL multiplex PCR kits, was studied in 953 unrelated individuals from Nepal. Several new alleles (i.e. not yet reported in the NIST Short Tandem Repeat DNA Internet DataBase [http://www.cstl.nist.gov/biotech/strbase/]) have been detected in the process.

  18. Arrestin gene mutations in autosomal recessive retinitis pigmentosa.

    Science.gov (United States)

    Nakazawa, M; Wada, Y; Tamai, M

    1998-04-01

    To assess the clinical and molecular genetic studies of patients with autosomal recessive retinitis pigmentosa associated with a mutation in the arrestin gene. Results of molecular genetic screening and case reports with DNA analysis and clinical features. University medical center. One hundred twenty anamnestically unrelated patients with autosomal recessive retinitis pigmentosa. DNA analysis was performed by single strand conformation polymorphism followed by nucleotide sequencing to search for a mutation in exon 11 of the arrestin gene. Clinical features were characterized by visual acuity slitlamp biomicroscopy, fundus examinations, fluorescein angiography, kinetic visual field testing, and electroretinography. We identified 3 unrelated patients with retinitis pigmentosa associated with a homozygous 1-base-pair deletion mutation in codon 309 of the arrestin gene designated as 1147delA. All 3 patients showed pigmentary retinal degeneration in the midperipheral area with or without macular involvement. Patient 1 had a sibling with Oguchi disease associated with the same mutation. Patient 2 demonstrated pigmentary retinal degeneration associated with a golden-yellow reflex in the peripheral fundus. Patients 1 and 3 showed features of retinitis pigmentosa without the golden-yellow fundus reflex. Although the arrestin 1147delA has been known as a frequent cause of Oguchi disease, this mutation also may be related to the pathogenesis of autosomal recessive retinitis pigmentosa. This phenomenon may provide evidence of variable expressivity of the mutation in the arrestin gene.

  19. Autosomal dominant syndrome resembling Coffin-Siris syndrome.

    Science.gov (United States)

    Flynn, Maureen A; Milunsky, Jeff M

    2006-06-15

    Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature. Copyright 2006 Wiley-Liss, Inc.

  20. Long-rod penetration: the transition zone between rigid and hydrodynamic penetration modes

    Directory of Open Access Journals (Sweden)

    Jian-feng Lou

    2014-06-01

    Full Text Available Long-rod penetration in a wide range of velocity means that the initial impact velocity varies in a range from tens of meters per second to several kilometers per second. The long rods maintain rigid state when the impact velocity is low, the nose of rod deforms and even is blunted when the velocity gets higher, and the nose erodes and fails to lead to the consumption of long projectile when the velocity is very high due to instantaneous high pressure. That is, from low velocity to high velocity, the projectile undergoes rigid rods, deforming non-erosive rods, and erosive rods. Because of the complicated changes of the projectile, no well-established theoretical model and numerical simulation have been used to study the transition zone. Based on the analysis of penetration behavior in the transition zone, a phenomenological model to describe target resistance and a formula to calculate penetration depth in transition zone are proposed, and a method to obtain the boundary velocity of transition zone is determined. A combined theoretical analysis model for three response regions is built by analyzing the characteristics in these regions. The penetration depth predicted by this combined model is in good agreement with experimental result.

  1. Ureteric transection secondary to penetrating handlebar injury

    Directory of Open Access Journals (Sweden)

    K.P. Debbink

    2017-08-01

    Full Text Available Ureteric trauma is rare, occurring in <1% of all traumas. We present a unique case of a 13 year old female who sustained a penetrating abdominal injury from a bicycle handlebar. Upon initial examination there was herniation of bowel through the abdominal wound, so exploratory laparotomy was performed. A serosal injury of the colon and bleeding mesenteric veins were encountered; the retroperitoneum was not explored at that time. Postoperative course was remarkable for a doubling of the serum creatinine, increasing abdominal distention and pain. Computed tomography on postoperative day five demonstrated a large amount of intra-abdominal fluid. The patient was taken for re-exploration. The left ureter was found to be completely transected. It was repaired over a double-J stent. This case demonstrates the need for a high index of suspicion in the diagnosis of ureteric injury. Keywords: Ureter, Bicycle, Handlebar, Penetrating

  2. Containment penetration design and analysis by finite element methods

    International Nuclear Information System (INIS)

    Perry, R.F.; Rigamonti, G.; Dainora, J.

    1975-01-01

    Containment penetration designs which provide complete support to process piping containing high pressure and high temperature fluids and which do not employ cooling coils, require special provisions to sustain loadings associated with normal/abnormal conditions and to limit maximum temperature transmitted to the containment concrete wall. In order to accomodate piping loads and fluid temperatures within code and regulatory limitations, the containment penetration designs require careful analysis of two critical regions: 1) the portion of the penetration sleeve which is exposed to containment ambient conditions and 2) the portion of the penetration which connects the sleeve to process piping (flued head). Analytical models using finite element representation of process piping, penetration flued head, and exposed sleeve were employed to investigate the penetration assembly design. By application of flexible multi-step analyses, different penetration configurations were evaluated to determine the effects of key design parameters. Among the parameters studied were flued head angles with the process piping, sleeve length and wall thickness. Special designs employing fins welded to the sleeve to further lower the temperature at the concrete wall interface were also investigated and fin geometry effects reported. (Auth.)

  3. Penetration shielding applications of CYLSEC

    International Nuclear Information System (INIS)

    Dexheimer, D.T.; Hathaway, J.M.

    1985-01-01

    Evaluation of penetration and discontinuity shielding is necessary to meet 10CFR20 regulations for ensuring personnel exposures are as low as reasonably achievable (ALARA). Historically, those shielding evaluations have been done to some degree on all projects. However, many early plants used conservative methods due to lack of an economical computer code, resulting in costly penetration shielding programs. With the increased industry interest in cost effectively reducing personnel exposures to meet ALARA regulations and with the development of the CYLSEC gamma transport computer code at Bechtel, a comprehensive effort was initiated to reduce penetration and discontinuity shielding but still provide a prudent degree of protection for plant personnel from radiation streaming. This effort was more comprehensive than previous programs due to advances in shielding analysis technology and increased interest in controlling project costs while maintaining personnel exposures ALARA. Methodology and resulting cost savings are discussed

  4. A novel missense mutation in the CLCN7 gene linked to benign autosomal dominant osteopetrosis: a case series

    Directory of Open Access Journals (Sweden)

    Rashid Ban Mousa

    2013-01-01

    channel 7 gene in this patient (Case 2. The missense mutation (CGG>TGG located in exon 15 (c.1225C>T of the Chloride channel 7 gene changed the amino acid position 409 from arginine to tryptophan (p.R409W, c.1225C>T. In addition to the clinical diagnosis of both cases, the missense mutation we identified in one allele of the Chloride channel 7 gene could be linked to autosomal dominant osteopetrosis-II because the symptoms appear in late childhood or adolescence. Conclusion In this family, the molecular diagnosis was confirmed after identification of the same mutation in the older son (sibling. Furthermore, we detected that the father and his brother (the uncle are carriers of the same mutation, whereas the mother and her sister (the aunt do not carry any mutation of the Chloride channel 7 gene. Thus, the disease penetrance is at least 60% in the family. The mother and father are cousins and a further consanguineous marriage between the aunt and the uncle is not recommended because the dominant allele of the Chloride channel 7 gene will be transferred to the progeny. However, a similar risk is also expected following a marriage between the uncle and an unrelated woman. The p.R409W mutation in the Chloride channel 7 gene has not yet been described in the literature and it possibly has a dominant-negative impact on the protein.

  5. A novel missense mutation in the CLCN7 gene linked to benign autosomal dominant osteopetrosis: a case series.

    Science.gov (United States)

    Rashid, Ban Mousa; Rashid, Nawshirwan Gafoor; Schulz, Ansgar; Lahr, Georgia; Nore, Beston Faiek

    2013-01-09

    mutation (CGG>TGG) located in exon 15 (c.1225C>T) of the Chloride channel 7 gene changed the amino acid position 409 from arginine to tryptophan (p.R409W, c.1225C>T).In addition to the clinical diagnosis of both cases, the missense mutation we identified in one allele of the Chloride channel 7 gene could be linked to autosomal dominant osteopetrosis-II because the symptoms appear in late childhood or adolescence. In this family, the molecular diagnosis was confirmed after identification of the same mutation in the older son (sibling). Furthermore, we detected that the father and his brother (the uncle) are carriers of the same mutation, whereas the mother and her sister (the aunt) do not carry any mutation of the Chloride channel 7 gene. Thus, the disease penetrance is at least 60% in the family. The mother and father are cousins and a further consanguineous marriage between the aunt and the uncle is not recommended because the dominant allele of the Chloride channel 7 gene will be transferred to the progeny. However, a similar risk is also expected following a marriage between the uncle and an unrelated woman. The p.R409W mutation in the Chloride channel 7 gene has not yet been described in the literature and it possibly has a dominant-negative impact on the protein.

  6. Transconjunctival penetration of mitomycin C

    Directory of Open Access Journals (Sweden)

    Velpandian T

    2008-01-01

    Full Text Available Aims: The study was performed to estimate transconjunctival penetration of mitomycin C (MMC to Tenon′s tissue following application over the intact conjunctiva before routine trabeculectomy. Settings and Design: Institution-based case series. Materials and Methods: In 41 eyes of 41 patients, MMC (0.4 mg/ml for 3 min was applied over the intact conjunctiva before beginning trabeculectomy. Tenon′s capsule directly beneath the site of application was excised during trabeculectomy and was homogenized, centrifuged and MMC concentrations were analyzed using high-performance liquid chromatography (HPLC. Statistical Analysis Used: Statistical analysis was performed using stata0 8.0 version software (STATA Corporation, Houston, TX, USA. In this study, P -values less than 0.05 were considered as statistically significant. Results: The average weight of the sample of Tenon′s tissue excised was 5.51 ± 4.42 mg (range: 0.9-17.1 and the average estimated MMC concentration found to be present in Tenon′s tissue using HPLC was 18.67 ± 32.36 x 10−6 moles/kg of the tissue (range: 0.38-197.05 x 10−6 . In 36 of the 41 patients (87.80%, the MMC concentration reached above 2 x 10−6 moles/kg of the tissue concentration required to inhibit human conjunctival fibroblasts. Conclusions: Mitomycin C does permeate into the subconjunctival tissue after supraconjunctival application for 3 min. Application of MMC over the conjunctiva may be a useful alternative to subconjunctival or subscleral application during routine trabeculectomy and as an adjunct for failing blebs.

  7. Mitigation of Power Quality Issues Due to High Penetration of Renewable Energy Sources in Electric Grid Systems Using Three-Phase APF/STATCOM Technologies: A Review

    Directory of Open Access Journals (Sweden)

    Wajahat Ullah Khan Tareen

    2018-06-01

    Full Text Available This study summarizes an analytical review on the comparison of three-phase static compensator (STATCOM and active power filter (APF inverter topologies and their control schemes using industrial standards and advanced high-power configurations. Transformerless and reduced switch count topologies are the leading technologies in power electronics that aim to reduce system cost and offer the additional benefits of small volumetric size, lightweight and compact structure, and high reliability. A detailed comparison of the topologies, control strategies and implementation structures of grid-connected high-power converters is presented. However, reducing the number of power semiconductor devices, sensors, and control circuits requires complex control strategies. This study focuses on different topological devices, namely, passive filters, shunt and hybrid filters, and STATCOMs, which are typically used for power quality improvement. Additionally, appropriate control schemes, such as sinusoidal pulse width modulation (SPWM and space vector PWM techniques, are selected. According to recent developments in shunt APF/STATCOM inverters, simulation and experimental results prove the effectiveness of APF/STATCOM systems for harmonic mitigation based on the defined limit in IEEE-519.

  8. Intrapulmonary penetration of linezolid.

    Science.gov (United States)

    Honeybourne, David; Tobin, Caroline; Jevons, Gail; Andrews, Jenny; Wise, Richard

    2003-06-01

    This study was designed to measure the concentrations of linezolid in bronchial mucosa, pulmonary macrophages and epithelial lining fluid and to compare them with simultaneous blood levels. Ten adult patients undergoing bronchoscopy for diagnostic purposes were given oral linezolid at a dosage of 600 mg twice a day for a total of six doses. Patients with active lung infection were excluded from the study. Flexible bronchoscopy was carried out between 2 and 8 h after the last dose of linezolid. Bronchial biopsies and bronchoalveolar lavage were carried out and a simultaneous blood sample obtained. Linezolid levels were measured using high-performance liquid chromatography (HPLC). Mean concentrations of linezolid were 13.4 mg/L in serum, 10.7 mg/kg in mucosa, 8.1 mg/L in alveolar macrophages and 25.1 mg/L in epithelial lining fluid. The mean site/serum concentration ratios were 0.79 for bronchial mucosa, 0.71 for macrophages and 8.35 for epithelial lining fluid. The MIC90 (< or =4 mg/L) of linezolid for Staphylococcus aureus and Streptococcus pneumoniae was exceeded in serum and bronchial mucosa in all subjects, in epithelial lining fluid in nine subjects and in macrophages in six subjects.

  9. Cutaneous mucormycosis secondary to penetrative trauma.

    Science.gov (United States)

    Zahoor, Bilal; Kent, Stephen; Wall, Daryl

    2016-07-01

    Mucormycosis is a rare but serious sequelae of penetrating trauma [1-5]. In spite of aggressive management, mortality remains high due to dissemination of infection. We completed a review of literature to determine the most optimal treatment of cutaneous mucormycosis which occurs secondary to penetrating trauma. We completed a review regarding the management of mucormycosis in trauma patients. We selected a total of 36 reports, of which 18 were case-based, for review. Surgical debridement is a primary predictor of improved outcomes in the treatment of mucormycosis [3,6,7]. Anti-fungal therapy, especially lipid soluble formulation of Amphotericin B, is helpful as an adjunct or when surgical debridement has been maximally achieved. Further research is needed to fully evaluate the impact of topical dressings; negative pressure wound therapy is helpful. An aggressive and early surgical approach, even at the expense of disfigurement, is necessary to reduce mortality in the setting of cutaneous mucormycosis that results from penetrating trauma [4,8,9]. Anti-fungal therapy and negative pressure wound therapy are formidable adjuncts. Copyright © 2016 Elsevier Ltd. All rights reserved.

  10. Prediction of electric vehicle penetration.

    Science.gov (United States)

    2017-05-01

    The object of this report is to present the current market status of plug-in-electric : vehicles (PEVs) and to predict their future penetration within the world and U.S. : markets. The sales values for 2016 show a strong year of PEV sales both in the...

  11. FAA Fluorescent Penetrant Laboratory Inspections

    Energy Technology Data Exchange (ETDEWEB)

    WINDES,CONNOR L.; MOORE,DAVID G.

    2000-08-02

    The Federal Aviation Administration Airworthiness Assurance NDI Validation Center currently assesses the capability of various non-destructive inspection (NDI) methods used for analyzing aircraft components. The focus of one such exercise is to evaluate the sensitivity of fluorescent liquid penetrant inspection. A baseline procedure using the water-washable fluorescent penetrant method defines a foundation for comparing the brightness of low cycle fatigue cracks in titanium test panels. The analysis of deviations in the baseline procedure will determine an acceptable range of operation for the steps in the inspection process. The data also gives insight into the depth of each crack and which step(s) of the inspection process most affect penetrant sensitivities. A set of six low cycle fatigue cracks produced in 6.35-mm thick Ti-6Al-4V specimens was used to conduct the experiments to produce sensitivity data. The results will document the consistency of the crack readings and compare previous experiments to find the best parameters for water-washable penetrant.

  12. Industrial Penetration and Internet Intensity

    NARCIS (Netherlands)

    C-L. Chang (Chia-Lin); M.J. McAleer (Michael); Y-C. Wu (Yu-Chieh)

    2016-01-01

    textabstractThis paper investigates the effect of industrial penetration and internet intensity for Taiwan manufacturing firms, and analyses whether the relationships are substitutes or complements. The sample observations are based on 153,081 manufacturing plants, and covers 26 two-digit industry

  13. Import market penetration in services

    OpenAIRE

    Langhammer, Rolf J.

    1991-01-01

    The EC-1992 programme foresees the complete liberalization of trade in services among member countries. To what extent has import market penetration in the Community already begun in selected service industries? Which EC member countries have been the forerunners to date? The following paper uses a new data base released recently by EUROSTAT in an attempt to answer these and other related questions.

  14. Diverging longitudinal changes in astrocytosis and amyloid PET in autosomal dominant Alzheimer's disease.

    Science.gov (United States)

    Rodriguez-Vieitez, Elena; Saint-Aubert, Laure; Carter, Stephen F; Almkvist, Ove; Farid, Karim; Schöll, Michael; Chiotis, Konstantinos; Thordardottir, Steinunn; Graff, Caroline; Wall, Anders; Långström, Bengt; Nordberg, Agneta

    2016-03-01

    mixed-effects models, fibrillar amyloid-β plaque deposition was first observed in the striatum of presymptomatic autosomal dominant Alzheimer's disease carriers from 17 years before expected symptom onset; at about the same time, astrocytosis was significantly elevated and then steadily declined. Diverging from the astrocytosis pattern, amyloid-β plaque deposition increased with disease progression. Glucose metabolism steadily declined from 10 years after initial amyloid-β plaque deposition. Patients with sporadic mild cognitive impairment who were (11)C-Pittsburgh compound B-positive at baseline showed increasing amyloid-β plaque deposition and decreasing glucose metabolism but, in contrast to autosomal dominant Alzheimer's disease carriers, there was no significant longitudinal decline in astrocytosis over time. The prominent initially high and then declining astrocytosis in autosomal dominant Alzheimer's disease carriers, contrasting with the increasing amyloid-β plaque load during disease progression, suggests astrocyte activation is implicated in the early stages of Alzheimer's disease pathology. © The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain.

  15. Emissions from cycling of thermal power plants in electricity systems with high penetration of wind power: Life cycle assessment for Ireland

    DEFF Research Database (Denmark)

    Turconi, Roberto; O'Dwyer, C.; Flynn, D.

    2014-01-01

    demand. The environmental impacts related to potential future energy systems in Ireland for 2025 with high shares of wind power were evaluated using life cycle assessment (LCA), focusing on cycling emissions (due to part-load operation and start-ups) from dispatchable generators. Part-load operations...... significantly affect the average power plant efficiency, with all units seeing an average yearly efficiency noticeably less than optimal. In particular, load following units, on average, saw an 11% reduction. Given that production technologies are typically modeled assuming steady-state operation at full load...

  16. Premium Efficiency Motors And Market Penetration Policy

    Energy Technology Data Exchange (ETDEWEB)

    Benhaddadi, Mohamed; Olivier, Guy

    2010-09-15

    This paper illustrates the induced enormous energy saving potential, permitted by using high-efficiency motors. Furthermore, the most important barriers to larger high-efficiency motors utilization are identified, and some incentives recommendations are given to overcome identified impediments. The authors consider that there is a strong case to enhance incentives policies for larger market penetration. The US Energy Policy Act and the Canadian Energy Efficient Act have lead to North American leadership on motor efficiency implementation. North America is not on the leading edge for energy saving and conservation. Motor efficiency is an exception that should be at least maintained.

  17. Implications of high renewable electricity penetration in the U.S. for water use, greenhouse gas emissions, land-use, and materials supply

    International Nuclear Information System (INIS)

    Arent, Doug; Pless, Jacquelyn; Mai, Trieu; Wiser, Ryan; Hand, Maureen; Baldwin, Sam; Heath, Garvin; Macknick, Jordan; Bazilian, Morgan; Schlosser, Adam; Denholm, Paul

    2014-01-01

    Highlights: • Renewable electricity generation could supply 80% of U.S. generation in 2050. • GHGs are reduced proportionally and water use is reduced by 50%. • Gross land-use impacts total less than 3% of land area of the contiguous U.S. • Some clean energy technologies rely on materials that face short-term risks. • No insurmountable long-term constraints to materials supply were identified. - Abstract: Recent work found that renewable energy could supply 80% of electricity demand in the contiguous United States in 2050 at the hourly level. This paper explores some of the implications of achieving such high levels of renewable electricity for supply chains and the environment in scenarios with renewable supply up to such levels. Expanding the renewable electricity supply at this scale by 2050 implies annual capacity additions of roughly 20 gigawatts per year (GW/year) over the next decade, rising to roughly 40 GW/year from 2040 to 2050. Given total 2012 renewable electricity capacity additions of slightly more than 16 GW, this suggests moderate growth of the related supply chains, averaging overall roughly 4% annual growth to 2040. Transitioning to high renewable electricity supply would lead to significant reductions in greenhouse gas emissions and water use, with only modest land-use implications. While renewable energy expansion implies moderate growth of the renewable electricity supply chains, no insurmountable long-term constraints to renewable electricity technology manufacturing capacity or materials supply are identified

  18. Containment penetration design and analysis by finite element methods

    International Nuclear Information System (INIS)

    Perry, R.F.; Rigamonti, G.; Dainora, J.

    1975-01-01

    Containment penetration designs which provide complete support to process piping containing high pressure and high temperature fluids and which do not employ cooling coils, require special provisions to sustain loadings associated with normal/abnormal conditions and to limit maximum temperature transmitted to the containment concrete wall. In order to accommodate piping imposed loads and fluid temperatures within code and regulatory limitations, the containment penetration designs require careful analysis of two critical regions: the portion of the penetration sleeve which is exposed to containment ambient conditions and the portion of the penetration which connects the sleeve to process piping (flued head). The length and thickness of the sleeve must be designed to provide maximum heat dissipation to the atmosphere and minimum heat conduction through the sleeve to meet concrete temperature limitations. The sleeve must have the capability to transmit the postulated piping loads to concrete embedments in the containment shell. The penetration flued head design must be strong enough to transfer high mechanical loads and be flexible enough to accommodate the thermal stresses generated by the high temperature fluid. Analytical models using finite element representations of process piping, penetration flued head, and exposed sleeve were employed to investigate the penetration assembly design. By application of flexible multi-step analyses, different penetration configurations were evaluated to determine the effects of key design parameters. Among the parameters studied were flued head profiles, flued head angles with the process piping, sleeve length and wall thickness. Special designs employing fins welded to the sleeve to lower the temperature at the concrete wall interface were investigated and fin geometry effects reported

  19. Smart Solar Grid. Integration of high penetration of photovoltaic in municipal low voltage distribution grids; Smart Solar Grid. Integration hoher Anteile von Photovoltaik in kommunalen Niederspannungsverteilnetzen

    Energy Technology Data Exchange (ETDEWEB)

    Ruf, Holger; Heilscher, Gerd [Hochschule Ulm (Germany); Meier, Florian [SWU Netze GmbH, Ulm (Germany)

    2012-07-01

    The high rate of decentralized generation in low voltage grids especially photovoltaic (PV) put the distribution grid operators to new challenges. Grid operation and grid planning have to respect the volatility and dynamic of decentralized generation now and in the future and adapt their previous proceedings. In the frame of the project Smart Solar Grid was a test site defined in the grid area of the DSO Stadtwerke Ulm/Neu-Ulm GmbH (SWU) to analyze the impact of the PV rise and possible solutions for the grid planning in the future. The first analysis based upon secondly measurements of the first test site. From this were statistical evaluation of the load flows and power variations done. Furthermore were the roof potential analysis results of the test site validated. These data are the base for the development of a forecast system for grid condition parameter. (orig.)

  20. [Professor WU Zhongchao's experience of penetration needling].

    Science.gov (United States)

    Zhang, Ning; Wang, Bing; Zhou, Yu

    2016-08-12

    Professor WU Zhongchao has unique application of penetration needling in clinical treatment. Professor WU applies penetration needling along meridians, and the methods of penetration needling include self-meridian penetration, exterior-interior meridian penetration, identical-name meridian penetration, different meridian penetration. The meridian differentiation is performed according to different TCM syndromes, locations and natures of diseases and acupoint nature, so as to make a comprehensive assessment. The qi movement during acupuncture is focused. In addition, attention is paid on anatomy and long-needle penetration; the sequence and direction of acupuncture is essential, and the reinforcing and reducing methods have great originality, presented with holding, waiting, pressing and vibrating. Based on classical acupoint, the acupoint of penetration needling is flexible, forming unique combination of acupoints.

  1. An experimental study on inhibiting graft rejection following high-risk penetrating keratoplasty by CD25 siRNA nanocarrier in rats

    Directory of Open Access Journals (Sweden)

    Yun-jie SHI

    2015-06-01

    Full Text Available Objective To investigate the effects of CD25 siRNA nanoparticles against immune rejection and prolongation of corneal graft survival time after high-risk corneal grafting in rats. Methods Orthotopic corneal transplantation was performed in SD rats with alkali burned corneas to mimic high-risk rat models. Donor cornea (Wistar rats was grafted into the right cornea of SD recipients on day 14 after alkali burn. The grafted rats were randomly divided into control group (Group A, EntransterTM-control CD25siRNA instillation treatment (Group B, EntransterTM-CD25siRNA instillation treatment (Group C and EntransterTM-CD25siRNA twice instillation treatment (Group D, first administration at 2-hour post-surgery and second on day 7 post-surgery. The recipient eyes were examined using a slit lamp microscope. Then, the mean survival time and rejection index (RI were calculated. The morphologies of grafts were microscopically examined with HE staining, and TEM. CD25 expression after operation was determined by quantitative RT-PCR and immunohistochemistry. Results The survival curves of transplanted cornea showed that the mean survival time in rats of groups C and D was significantly longer than that in groups A and B (P<0.05. No significant difference was found in survival time between group A and group B, and the same between group C and group D. The grafts in groups A and B showed obvious edema and thickening, with irregular arrangement of collagen fibers and infiltration of a large amount of inflammatory cells. Immunohistochemical results showed that expression of CD25 was found in the corneal epithelium, stroma and endothelium in all rats, and higher CD25 expression was observed in groups A and B. Transmission electron microscopy revealed that the degree of stromal fibroblast apoptosis and necrosis in corneal graft was obviously lower in groups C and D than that of groups A and B, with a significant statistical difference. The expression of CD25 m

  2. Control of penetration zone GMAW

    Directory of Open Access Journals (Sweden)

    Віталій Петрович Iванов

    2016-11-01

    Full Text Available Thermal properties of the base metal, shielding medium and the nature of the electrode metal transfer to a great extent determine the penetration area formation in gas-arc welding. It is not always possible to take into account the influence of these factors on penetration front forming within the existing models. The aim of the work was to research the penetration area forming in gas-arc welding. The research of the penetration area forming in gas-arc welding of CrNi austenitic steels was made. The parameters of the regime as well as the kind of the gaseous medium influence on the formation of the penetration zone were studied. The article shows a linear proportional relationship between the electrode feed rate and the size of the base metal plate. The penetration area formation mode for welding in argon and carbon dioxide have been worked out. Diameter, feed rate and the speed of the electrode movement have been chosen as the main input parameters. Multiple regression analysis method was used to make up the modes. The relations of the third order that make it possible to take into account the electrode metal transfer and thermal properties change of the materials to be welded were used. These relationships show quite good agreement with the experimental measurements in the calculation of the fusion zone shape with consumable electrode in argon and carbon dioxide. It was determined that the shape of the melting front curve can be shown as a generalized function in which the front motion parameters depend on feed rate and the diameter of the electrode. Penetration zone growth time is determined by the welding speed and is calculated as a discrete function of the distance from the electrode with the spacing along the movement coordinate. The influence of the mode parameters on the formation of the fusion zone has been investigated and the ways to manage and stabilize the weld pool formation have been identified. The modes can be used to develop

  3. Characterization of Large Structural Genetic Mosaicism in Human Autosomes

    Science.gov (United States)

    Machiela, Mitchell J.; Zhou, Weiyin; Sampson, Joshua N.; Dean, Michael C.; Jacobs, Kevin B.; Black, Amanda; Brinton, Louise A.; Chang, I-Shou; Chen, Chu; Chen, Constance; Chen, Kexin; Cook, Linda S.; Crous Bou, Marta; De Vivo, Immaculata; Doherty, Jennifer; Friedenreich, Christine M.; Gaudet, Mia M.; Haiman, Christopher A.; Hankinson, Susan E.; Hartge, Patricia; Henderson, Brian E.; Hong, Yun-Chul; Hosgood, H. Dean; Hsiung, Chao A.; Hu, Wei; Hunter, David J.; Jessop, Lea; Kim, Hee Nam; Kim, Yeul Hong; Kim, Young Tae; Klein, Robert; Kraft, Peter; Lan, Qing; Lin, Dongxin; Liu, Jianjun; Le Marchand, Loic; Liang, Xiaolin; Lissowska, Jolanta; Lu, Lingeng; Magliocco, Anthony M.; Matsuo, Keitaro; Olson, Sara H.; Orlow, Irene; Park, Jae Yong; Pooler, Loreall; Prescott, Jennifer; Rastogi, Radhai; Risch, Harvey A.; Schumacher, Fredrick; Seow, Adeline; Setiawan, Veronica Wendy; Shen, Hongbing; Sheng, Xin; Shin, Min-Ho; Shu, Xiao-Ou; VanDen Berg, David; Wang, Jiu-Cun; Wentzensen, Nicolas; Wong, Maria Pik; Wu, Chen; Wu, Tangchun; Wu, Yi-Long; Xia, Lucy; Yang, Hannah P.; Yang, Pan-Chyr; Zheng, Wei; Zhou, Baosen; Abnet, Christian C.; Albanes, Demetrius; Aldrich, Melinda C.; Amos, Christopher; Amundadottir, Laufey T.; Berndt, Sonja I.; Blot, William J.; Bock, Cathryn H.; Bracci, Paige M.; Burdett, Laurie; Buring, Julie E.; Butler, Mary A.; Carreón, Tania; Chatterjee, Nilanjan; Chung, Charles C.; Cook, Michael B.; Cullen, Michael; Davis, Faith G.; Ding, Ti; Duell, Eric J.; Epstein, Caroline G.; Fan, Jin-Hu; Figueroa, Jonine D.; Fraumeni, Joseph F.; Freedman, Neal D.; Fuchs, Charles S.; Gao, Yu-Tang; Gapstur, Susan M.; Patiño-Garcia, Ana; Garcia-Closas, Montserrat; Gaziano, J. Michael; Giles, Graham G.; Gillanders, Elizabeth M.; Giovannucci, Edward L.; Goldin, Lynn; Goldstein, Alisa M.; Greene, Mark H.; Hallmans, Goran; Harris, Curtis C.; Henriksson, Roger; Holly, Elizabeth A.; Hoover, Robert N.; Hu, Nan; Hutchinson, Amy; Jenab, Mazda; Johansen, Christoffer; Khaw, Kay-Tee; Koh, Woon-Puay; Kolonel, Laurence N.; Kooperberg, Charles; Krogh, Vittorio; Kurtz, Robert C.; LaCroix, Andrea; Landgren, Annelie; Landi, Maria Teresa; Li, Donghui; Liao, Linda M.; Malats, Nuria; McGlynn, Katherine A.; McNeill, Lorna H.; McWilliams, Robert R.; Melin, Beatrice S.; Mirabello, Lisa; Peplonska, Beata; Peters, Ulrike; Petersen, Gloria M.; Prokunina-Olsson, Ludmila; Purdue, Mark; Qiao, You-Lin; Rabe, Kari G.; Rajaraman, Preetha; Real, Francisco X.; Riboli, Elio; Rodríguez-Santiago, Benjamín; Rothman, Nathaniel; Ruder, Avima M.; Savage, Sharon A.; Schwartz, Ann G.; Schwartz, Kendra L.; Sesso, Howard D.; Severi, Gianluca; Silverman, Debra T.; Spitz, Margaret R.; Stevens, Victoria L.; Stolzenberg-Solomon, Rachael; Stram, Daniel; Tang, Ze-Zhong; Taylor, Philip R.; Teras, Lauren R.; Tobias, Geoffrey S.; Viswanathan, Kala; Wacholder, Sholom; Wang, Zhaoming; Weinstein, Stephanie J.; Wheeler, William; White, Emily; Wiencke, John K.; Wolpin, Brian M.; Wu, Xifeng; Wunder, Jay S.; Yu, Kai; Zanetti, Krista A.; Zeleniuch-Jacquotte, Anne; Ziegler, Regina G.; de Andrade, Mariza; Barnes, Kathleen C.; Beaty, Terri H.; Bierut, Laura J.; Desch, Karl C.; Doheny, Kimberly F.; Feenstra, Bjarke; Ginsburg, David; Heit, John A.; Kang, Jae H.; Laurie, Cecilia A.; Li, Jun Z.; Lowe, William L.; Marazita, Mary L.; Melbye, Mads; Mirel, Daniel B.; Murray, Jeffrey C.; Nelson, Sarah C.; Pasquale, Louis R.; Rice, Kenneth; Wiggs, Janey L.; Wise, Anastasia; Tucker, Margaret; Pérez-Jurado, Luis A.; Laurie, Cathy C.; Caporaso, Neil E.; Yeager, Meredith; Chanock, Stephen J.

    2015-01-01

    Analyses of genome-wide association study (GWAS) data have revealed that detectable genetic mosaicism involving large (>2 Mb) structural autosomal alterations occurs in a fraction of individuals. We present results for a set of 24,849 genotyped individuals (total GWAS set II [TGSII]) in whom 341 large autosomal abnormalities were observed in 168 (0.68%) individuals. Merging data from the new TGSII set with data from two prior reports (the Gene-Environment Association Studies and the total GWAS set I) generated a large dataset of 127,179 individuals; we then conducted a meta-analysis to investigate the patterns of detectable autosomal mosaicism (n = 1,315 events in 925 [0.73%] individuals). Restricting to events >2 Mb in size, we observed an increase in event frequency as event size decreased. The combined results underscore that the rate of detectable mosaicism increases with age (p value = 5.5 × 10−31) and is higher in men (p value = 0.002) but lower in participants of African ancestry (p value = 0.003). In a subset of 47 individuals from whom serial samples were collected up to 6 years apart, complex changes were noted over time and showed an overall increase in the proportion of mosaic cells as age increased. Our large combined sample allowed for a unique ability to characterize detectable genetic mosaicism involving large structural events and strengthens the emerging evidence of non-random erosion of the genome in the aging population. PMID:25748358

  4. Pathogenesis and potential therapy of autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    O.O. Melnyk

    2017-10-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is a hereditary disease characterized by progressive growth of the cyst and an increase in the total volume of the kidneys which leads to kidney failure. The main causes of ADPKD are mutations in the genes PKD1 and PKD2 which encode the formation of polycystin-1 and polycystin-2 proteins. There is a connection between structural and functional defects in the primary cilia with the ADPKD. The most promising drugs for the treatment of ADPKD today are vasopressin-2 receptor antagonists, m-TOR and c-AMP inhibitors.

  5. Gene therapy in animal models of autosomal dominant retinitis pigmentosa

    Science.gov (United States)

    Rossmiller, Brian; Mao, Haoyu

    2012-01-01

    Gene therapy for dominantly inherited genetic disease is more difficult than gene-based therapy for recessive disorders, which can be treated with gene supplementation. Treatment of dominant disease may require gene supplementation partnered with suppression of the expression of the mutant gene either at the DNA level, by gene repair, or at the RNA level by RNA interference or transcriptional repression. In this review, we examine some of the gene delivery approaches used to treat animal models of autosomal dominant retinitis pigmentosa, focusing on those models associated with mutations in the gene for rhodopsin. We conclude that combinatorial approaches have the greatest promise for success. PMID:23077406

  6. Evidence for autosomal recessive inheritance in cerebral gigantism

    Science.gov (United States)

    Nevo, S.; Zeltzer, M.; Benderly, A.; Levy, J.

    1974-01-01

    Three cases of cerebral gigantism, two sibs and their double first cousin, are described in a large inbred family from Israel. Two of the three were observed and diagnosed at birth and two were followed for two years. They all presented the signs and symptoms considered typical of this syndrome, as well as some of the less frequent findings. Generalized oedema and flexion contractures of the feet were observed in two of the three at birth. This has not hitherto been reported in cases of cerebral gigantism, of whom only a few have been observed and diagnosed at birth. Autosomal recessive inheritance is clearly implied in this family. Images PMID:4841084

  7. Investigation on the Combined Use of Ground Penetrating Radar, Cone Penetrometer and High Resolution Seismic Data for Near Surface and Vadose Zone Characterization in the A/M Area of the Savannah River Site, South Carolina

    International Nuclear Information System (INIS)

    Wyatt, D.E.; Cumbest, R.J.; Aadland, R.K.; Syms, F.H.; Stephenson, D.E.; Sherrill, J.C.

    1997-06-01

    This study compares data from Cone Penetrometer Tests (CPT), high resolution surface reflection seismic (HRS) data and ground penetrating radar (GPR) data in the upper 120 feet (40 meters) of the A/M Area, Upper Three Runs Watershed at the Savannah River Site in South Carolina. The CPT, GPR, and HRS data were obtained along the Silverton Road in the western sector of the A/M Area groundwater plume, and adjacent to Geophysical Correlation Boring number-sign 1 (GCB-1). This location allows for multiple correlations to be made between the various data sources, and supports shallow investigations for near surface affects of the Crackerneck Fault, a major structural feature in the area. Borehole geophysical data from GCB-1 were used to provide subsurface constraints on the CPT, GPR, and HRS data. core data, natural gamma ray, spectral gamma data, multi-level induction resistivity, density and sonic data were utilized to distinguish clays, sands and silts. The CPT data provided tip bearing and sleeve stress, as an indicator of stratigraphy. Reflection seismic data provided continuous subsurface profiles of key marker horizons. Ground Penetrating Radar provided information on shallow subsurface geological features. Conclusions from this study suggest that there is a high degree of correlation between the CPT and borehole geophysical data, specifically, the Friction Ratio and gamma/spectral gamma curves. The Upland/Tobacco Road, Tobacco Road/Dry Branch, Dry Branch/Santee, Santee/Warley Hill and the Warley Hill/Congaree contacts are discernible. From these contacts it is possible to map structural relationships in the shallow subsurface that are tied to regional data. Because formation contacts are discernible, CPT, HRS, GPR, and geophysical log intra-formational anomalies are mappable. These features allow for stratigraphic and facies mapping using the GPR and HRS data for continuity and the CPT and geophysical data for lithofacies analysis. It is possible to use the

  8. Penetrating Cardiac and Hepatic Injury; Polytrauma of a Child After Bombing

    Directory of Open Access Journals (Sweden)

    Baris Akca

    2013-10-01

    Full Text Available After a bombing attack, patients were brought into hospital suffering from a combination of injuries caused by the blast, penetrating injuries and burns which as a case of polytrauma. In penetrating thoracoabdominal injuries due to bombing possibility of cardiac injury should be kept in mind. Penetrating cardiac injuries in children are rare but has a high mortality and morbidity. In some cases there may be difficulty in diagnosis of penetrating cardiac injury. In this case we want to share the diagnosis, treatment and follow-up processes of penetrating cardiac and hepatic injury with burns of a politrauma child due to bombing.

  9. Genetic diversity of 21 autosomal STR loci in the Han population from Sichuan province, Southwest China.

    Science.gov (United States)

    He, Guanglin; Li, Ye; Wang, Zheng; Liang, Weibo; Luo, Haibo; Liao, Miao; Zhang, Ji; Yan, Jing; Li, Yingbi; Hou, Yiping; Wu, Jin

    2017-11-01

    Exploration of the ethnic origin and genetic differentiation of 56 Chinese officially recognized nationalities populations played a fundamental role in the research field of population genetics, forensic science, linguistics, anthropology, and archaeology. In the present study, population data of 21 autosomal STR loci (CSF1PO, D10S1248, D12S391, D13S317, D16S539, D18S51, D19S433, D21S11, D2S1338, D2S441, D3S1358, D5S818, D6S1043, D7S820, D8S1179, FGA, Penta D, Penta E, TH01, TPOX, and vWA) included in the AGCU EX22 kit in 2793 Southwest Han Chinese individuals was obtained and population genetic relationships among 28 Chinese populations were investigated. Our study indicated that the twenty-one autosomal STRs are highly polymorphic in the Sichuan Han population and can be used as a powerful tool in the routine forensic usage. MDS and phylogenetic analysis suggested that the Sichuan Han population kept a close genetic relationship with the southwest populations. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Substructure of a Tunisian Berber population as inferred from 15 autosomal short tandem repeat loci.

    Science.gov (United States)

    Khodjet-El-Khil, Houssein; Fadhlaoui-Zid, Karima; Gusmão, Leonor; Alves, Cíntia; Benammar-Elgaaied, Amel; Amorim, Antonio

    2008-08-01

    Currently, language and cultural practices are the only criteria to distinguish between Berber autochthonous Tunisian populations. To evaluate these populations' possible genetic structure and differentiation, we have analyzed 15 autosomal short tandem repeat loci (CSF1PO, D3S1358, D5S818, D7S820, D8S1179, D13S317, D16S539, D18S51, D21S11, FGA, TH01, TPOX, VWA, D2S1338, and D19S433) in three southern Tunisian Berber groups: Sened, Matmata, and Chenini-Douiret. The exact test of population differentiation based on allele frequencies at the 15 loci shows significant P values at 7 loci between Chenini-Douiret and both Sened and Matmata, whereas just 5 loci show significant P values between Sened and Matmata. Comparative analyses between the three Berber groups based on genetic distances show that P values for F(ST) distances are significant between the three Berber groups. Population analysis performed using Structure shows a clear differentiation between these Berber groups, with strong genetic isolation of Chenini-Douiret. These results confirm at the autosomal level the high degree of heterogeneity of Tunisian Berber populations that had been previously reported for uniparental markers.

  11. Mobile Termination and Mobile Penetration

    OpenAIRE

    Hurkens, Sjaak; Jeon, Doh-Shin

    2009-01-01

    In this paper, we study how access pricing affects network competition when subscription demand is elastic and each network uses non-linear prices and can apply termination-based price discrimination. In the case of a fixed per minute termination charge, we find that a reduction of the termination charge below cost has two oppos- ing effects: it softens competition but helps to internalize network externalities. The former reduces mobile penetration while the latter boosts it. We find that fi...

  12. Mobile termination and mobile penetration

    OpenAIRE

    Hurkens, Sjaak

    2009-01-01

    In this paper, we study how access pricing affects network competition when subscription demand is elastic and each network uses non-linear prices and can apply termination-based price discrimination. In the case of a fixed per minute termination charge, we find that a reduction of the termination charge below cost has two opposing effects: it softens competition but helps to internalize network externalities. The former reduces mobile penetration while the latter boosts it. We find that firm...

  13. Fluorescent penetration crack testing method

    International Nuclear Information System (INIS)

    Roth, A.

    1979-01-01

    The same cleaning, penetration, washing, development and evaluation agents are used in this method as for known methods. In order to accelerate or shorten the testing, the drying process is performed only to optical dryness of the material surface by blowing on it with pressurized air, the development by simple pressing into or dusting of the material with the developer and the temperature of the washing water is selected within room temperature range. (RW) [de

  14. Penetration of relativistic heavy ions through matter

    International Nuclear Information System (INIS)

    Scheidenberger, C.; Geissel, H.

    1997-07-01

    New heavy-ion accelerators covering the relativistic and ultra-relativistic energy regime allow to study atomic collisions with bare and few-electron projectiles. High-resolution magnetic spectrometers are used for precise stopping-power and energy-loss straggling measurements. Refined theories beyond the Born approximation have been developed and are confirmed by experiments. This paper summarizes the large progress in the understanding of relativistic heavy-ion penetration through matter, which has been achieved in the last few years. (orig.)

  15. HMO penetration and physicians' earnings.

    Science.gov (United States)

    Hadley, J; Mitchell, J M

    1999-11-01

    The goal of this study is to estimate whether cross-sectional variations in enrollment in health maintenance organizations (HMOs) affected physicians' earnings and hourly income in 1990. Using data from a nationally representative sample of 4,577 younger physicians (penetration is endogenous and used the instrumental variables approach to obtain unbiased estimates. HMO penetration had a negative and statistically significant impact on physicians earnings in 1990. A doubling of the average level of HMO penetration in the market is estimated to reduce annual earnings by 7% to 10.7%, and hourly earnings by approximately 6% to 9%. It appears that HMOs were successful in reducing physicians' annual and per hour earnings in 1990, presumably through a combination of fewer visits and lower payment rates for people covered by HMOs. Although these results cannot be generalized to all physicians, the experience of a younger cohort of physicians may still be a good indicator of the future effects of HMOs because younger physicians may be more susceptible to market forces than older and more established physicians. Moreover, these results may be somewhat conservative because they reflect market behavior in 1990, several years before the rapid growth and more aggressive market behavior of HMOs in recent years.

  16. Penetrating eye injury in war.

    Science.gov (United States)

    Biehl, J W; Valdez, J; Hemady, R K; Steidl, S M; Bourke, D L

    1999-11-01

    The percentage of penetrating eye injuries in war has increased significantly in this century compared with the total number of combat injuries. With the increasing use of fragmentation weapons and possibly laser weapons on the battle-field in the future, the rate of eye injuries may exceed the 13% of the total military injuries found in Operations Desert Storm/Shield. During the Iran-Iraq War (1980-1988), eye injuries revealed that retained foreign bodies and posterior segment injuries have an improved prognosis in future military ophthalmic surgery as a result of modern diagnostic and treatment modalities. Compared with the increasing penetrating eye injuries on the battlefield, advances in ophthalmic surgery are insignificant. Eye armor, such as visors that flip up and down and protect the eyes from laser injury, needs to be developed. Similar eye protection is being developed in civilian sportswear. Penetrating eye injury in the civilian sector is becoming much closer to the military model and is now comparable for several reasons.

  17. Autosomal dominant cutis laxa with progeroid features due to a novel, de novo mutation in ALDH18A1.

    Science.gov (United States)

    Bhola, Priya T; Hartley, Taila; Bareke, Eric; Boycott, Kym M; Nikkel, Sarah M; Dyment, David A

    2017-06-01

    De novo dominant mutations in the aldehyde dehydrogenase 18 family member A1 (ALDH18A1) gene have recently been shown to cause autosomal dominant cutis laxa with progeroid features (MIM 616603). To date, all de novo dominant mutations have been found in a single highly conserved amino acid residue at position p.Arg138. We report an 8-year-old male with a clinical diagnosis of autosomal dominant cutis laxa (ADCL) with progeroid features and a novel de novo missense mutation in ALDH18A1 (NM_002860.3: c.377G>A (p.Arg126His)). This is the first report of an individual with ALDH18A1-ADCL due to a substitution at a residue other than p.Arg138. Knowledge of the complete spectrum of dominant-acting mutations that cause this rare syndrome will have implications for molecular diagnosis and genetic counselling of these families.

  18. Autosomal recessive Noonan syndrome associated with biallelic LZTR1 variants.

    Science.gov (United States)

    Johnston, Jennifer J; van der Smagt, Jasper J; Rosenfeld, Jill A; Pagnamenta, Alistair T; Alswaid, Abdulrahman; Baker, Eva H; Blair, Edward; Borck, Guntram; Brinkmann, Julia; Craigen, William; Dung, Vu Chi; Emrick, Lisa; Everman, David B; van Gassen, Koen L; Gulsuner, Suleyman; Harr, Margaret H; Jain, Mahim; Kuechler, Alma; Leppig, Kathleen A; McDonald-McGinn, Donna M; Can, Ngoc Thi Bich; Peleg, Amir; Roeder, Elizabeth R; Rogers, R Curtis; Sagi-Dain, Lena; Sapp, Julie C; Schäffer, Alejandro A; Schanze, Denny; Stewart, Helen; Taylor, Jenny C; Verbeek, Nienke E; Walkiewicz, Magdalena A; Zackai, Elaine H; Zweier, Christiane; Zenker, Martin; Lee, Brendan; Biesecker, Leslie G

    2018-02-22

    PurposeTo characterize the molecular genetics of autosomal recessive Noonan syndrome.MethodsFamilies underwent phenotyping for features of Noonan syndrome in children and their parents. Two multiplex families underwent linkage analysis. Exome, genome, or multigene panel sequencing was used to identify variants. The molecular consequences of observed splice variants were evaluated by reverse-transcription polymerase chain reaction.ResultsTwelve families with a total of 23 affected children with features of Noonan syndrome were evaluated. The phenotypic range included mildly affected patients, but it was lethal in some, with cardiac disease and leukemia. All of the parents were unaffected. Linkage analysis using a recessive model supported a candidate region in chromosome 22q11, which includes LZTR1, previously shown to harbor mutations in patients with Noonan syndrome inherited in a dominant pattern. Sequencing analyses of 21 live-born patients and a stillbirth identified biallelic pathogenic variants in LZTR1, including putative loss-of-function, missense, and canonical and noncanonical splicing variants in the affected children, with heterozygous, clinically unaffected parents and heterozygous or normal genotypes in unaffected siblings.ConclusionThese clinical and genetic data confirm the existence of a form of Noonan syndrome that is inherited in an autosomal recessive pattern and identify biallelic mutations in LZTR1.Genet Med advance online publication, 22 February 2018; doi:10.1038/gim.2017.249.

  19. Microcephaly-chorioretinopathy syndrome, autosomal recessive form. A case report

    Directory of Open Access Journals (Sweden)

    Rafael Fabiano Machado Rosa

    Full Text Available CONTEXT: The autosomal recessive form of microcephaly-chorioretinopathy syndrome is a rare genetic condition that is considered to be an important differential diagnosis with congenital toxoplasmosis.CASE REPORT: Our patient was a seven-year-old white boy who was initially diagnosed with congenital toxoplasmosis. However, his serological tests for congenital infections, including toxoplasmosis, were negative. He was the first child of young, healthy and consanguineous parents (fourth-degree relatives. The parents had normal head circumferences and intelligence. The patient presented microcephaly and specific abnormalities of the retina, with multiple diffuse oval areas of pigmentation and patches of chorioretinal atrophy associated with diffuse pigmentation of the fundus. Ophthalmological evaluations on the parents were normal. A computed tomography scan of the child's head showed slight dilation of lateral ventricles and basal cisterns without evidence of calcifications. We did not find any lymphedema in his hands and feet. He had postnatal growth retardation, severe mental retardation and cerebral palsy.CONCLUSIONS: The finding of chorioretinal lesions in a child with microcephaly should raise suspicions of the autosomal recessive form of microcephaly-chorioretinopathy syndrome, especially in cases with an atypical pattern of eye fundus and consanguinity. A specific diagnosis is essential for an appropriate clinical evaluation and for genetic counseling for the patients and their families.

  20. NDST1 missense mutations in autosomal recessive intellectual disability.

    Science.gov (United States)

    Reuter, Miriam S; Musante, Luciana; Hu, Hao; Diederich, Stefan; Sticht, Heinrich; Ekici, Arif B; Uebe, Steffen; Wienker, Thomas F; Bartsch, Oliver; Zechner, Ulrich; Oppitz, Cornelia; Keleman, Krystyna; Jamra, Rami Abou; Najmabadi, Hossein; Schweiger, Susann; Reis, André; Kahrizi, Kimia

    2014-11-01

    NDST1 was recently proposed as a candidate gene for autosomal recessive intellectual disability in two families. It encodes a bifunctional GlcNAc N-deacetylase/N-sulfotransferase with important functions in heparan sulfate biosynthesis. In mice, Ndst1 is crucial for embryonic development and homozygous null mutations are perinatally lethal. We now report on two additional unrelated families with homozygous missense NDST1 mutations. All mutations described to date predict the substitution of conserved amino acids in the sulfotransferase domain, and mutation modeling predicts drastic alterations in the local protein conformation. Comparing the four families, we noticed significant overlap in the clinical features, including both demonstrated and apparent intellectual disability, muscular hypotonia, epilepsy, and postnatal growth deficiency. Furthermore, in Drosophila, knockdown of sulfateless, the NDST ortholog, impairs long-term memory, highlighting its function in cognition. Our data confirm NDST1 mutations as a cause of autosomal recessive intellectual disability with a distinctive phenotype, and support an important function of NDST1 in human development. © 2014 Wiley Periodicals, Inc.

  1. Real-Time Penetrating Particle Analyzer (PAN)

    Science.gov (United States)

    Wu, X.; Ambrosi, G.; Bertucci, B.

    2018-02-01

    The PAN can measure penetrating particles with great precision to study energetic particles, solar activities, and the origin and propagation of cosmic rays. The real-time monitoring of penetrating particles is crucial for deep space human travel.

  2. Network Penetration Testing and Research

    Science.gov (United States)

    Murphy, Brandon F.

    2013-01-01

    This paper will focus the on research and testing done on penetrating a network for security purposes. This research will provide the IT security office new methods of attacks across and against a company's network as well as introduce them to new platforms and software that can be used to better assist with protecting against such attacks. Throughout this paper testing and research has been done on two different Linux based operating systems, for attacking and compromising a Windows based host computer. Backtrack 5 and BlackBuntu (Linux based penetration testing operating systems) are two different "attacker'' computers that will attempt to plant viruses and or NASA USRP - Internship Final Report exploits on a host Windows 7 operating system, as well as try to retrieve information from the host. On each Linux OS (Backtrack 5 and BlackBuntu) there is penetration testing software which provides the necessary tools to create exploits that can compromise a windows system as well as other operating systems. This paper will focus on two main methods of deploying exploits 1 onto a host computer in order to retrieve information from a compromised system. One method of deployment for an exploit that was tested is known as a "social engineering" exploit. This type of method requires interaction from unsuspecting user. With this user interaction, a deployed exploit may allow a malicious user to gain access to the unsuspecting user's computer as well as the network that such computer is connected to. Due to more advance security setting and antivirus protection and detection, this method is easily identified and defended against. The second method of exploit deployment is the method mainly focused upon within this paper. This method required extensive research on the best way to compromise a security enabled protected network. Once a network has been compromised, then any and all devices connected to such network has the potential to be compromised as well. With a compromised

  3. Ethical Dilemmas and Dimensions in Penetration Testing

    OpenAIRE

    Faily, Shamal; McAlaney, John; Iacob, C.

    2015-01-01

    Penetration testers are required to attack systems to evaluate their security, but without engaging in unethical behaviour while doing so. Despite work on hacker values and studies into security practice, there is little literature devoted to the ethical pressures associated with penetration testing. This paper presents several ethical dilemmas and dimensions associated with penetration testing;\\ud these shed light on the ethical positions taken by Penetration testers, and help identify poten...

  4. Kali Linux wireless penetration testing essentials

    CERN Document Server

    Alamanni, Marco

    2015-01-01

    This book is targeted at information security professionals, penetration testers and network/system administrators who want to get started with wireless penetration testing. No prior experience with Kali Linux and wireless penetration testing is required, but familiarity with Linux and basic networking concepts is recommended.

  5. Effect of compressibility on the hypervelocity penetration

    Science.gov (United States)

    Song, W. J.; Chen, X. W.; Chen, P.

    2018-02-01

    We further consider the effect of rod strength by employing the compressible penetration model to study the effect of compressibility on hypervelocity penetration. Meanwhile, we define different instances of penetration efficiency in various modified models and compare these penetration efficiencies to identify the effects of different factors in the compressible model. To systematically discuss the effect of compressibility in different metallic rod-target combinations, we construct three cases, i.e., the penetrations by the more compressible rod into the less compressible target, rod into the analogously compressible target, and the less compressible rod into the more compressible target. The effects of volumetric strain, internal energy, and strength on the penetration efficiency are analyzed simultaneously. It indicates that the compressibility of the rod and target increases the pressure at the rod/target interface. The more compressible rod/target has larger volumetric strain and higher internal energy. Both the larger volumetric strain and higher strength enhance the penetration or anti-penetration ability. On the other hand, the higher internal energy weakens the penetration or anti-penetration ability. The two trends conflict, but the volumetric strain dominates in the variation of the penetration efficiency, which would not approach the hydrodynamic limit if the rod and target are not analogously compressible. However, if the compressibility of the rod and target is analogous, it has little effect on the penetration efficiency.

  6. Development of penetrant materials from used oil

    International Nuclear Information System (INIS)

    Mohamad Pauzi Ismail; Azhar Azmi

    2014-01-01

    This paper described the results of experiment to produce penetrant for nondestructive testing using used engine oil. The used engine oil was obtained from motor vehicle. It was mixed with kerosene at several mix proportion. The penetrability of these mixing were measured and compared with the penetrant available on the market. The results of measurement were explained and discussed. (author)

  7. Videofluoroscopic Predictors of Penetration-Aspiration in Parkinson's Disease Patients.

    Science.gov (United States)

    Argolo, Natalie; Sampaio, Marília; Pinho, Patrícia; Melo, Ailton; Nóbrega, Ana Caline

    2015-12-01

    Parkinson's disease (PD) patients show a high prevalence of swallowing disorders and tracheal aspiration of food. The videofluoroscopic study of swallowing (VFSS) allows clinicians to visualize the visuoperceptual and temporal parameters associated with swallowing disorders in an attempt to predict aspiration risk. However, this subject remains understudied in PD populations. Our aim was to identify the predictors of penetration-aspiration in PD patients using the VFSS. Consecutive patients were evaluated using VFSS with different consistencies and volumes of food. A speech-language pathologist measured the type of intra-oral bolus organization, loss of bolus control, bolus location at the initiation of the pharyngeal swallow, the presence of multiple swallows, piecemeal deglutition, bolus residue in the pharyngeal recesses and temporal measures. Scores ≥3 on the penetration-aspiration scale (PAS) indicated the occurrence of penetration-aspiration. Using logistic marginal regression, we found that residue in the vallecula, residue in the upper esophageal sphincter and piecemeal deglutition were associated with penetration-aspiration (odds ratio (OR) = 4.09, 2.87 and 3.83; P = 0.0040, 0.0071 and 0.0009, respectively). Penetration/aspiration occurred only with fluids (both of thin and thick consistency), and no significant differences were observed between fluid types or food volumes. The mechanisms underlying dysphagia and penetration/aspiration in PD patients and indications for further studies are discussed.

  8. Penetrance and clinical consequences of a gross SDHB deletion in a large family.

    Science.gov (United States)

    Solis, D C; Burnichon, N; Timmers, H J L M; Raygada, M J; Kozupa, A; Merino, M J; Makey, D; Adams, K T; Venisse, A; Gimenez-Roqueplo, A-P; Pacak, K

    2009-04-01

    Mutations in the gene encoding subunit B of the mitochondrial enzyme succinate dehydrogenase (SDHB) are inherited in an autosomal dominant manner and are associated with hereditary paraganglioma (PGL) and pheochromocytoma. The phenotype of patients with SDHB point mutations has been previously described. However, the phenotype and penetrance of gross SDHB deletions have not been well characterized as they are rarely described. The objective was to describe the phenotype and estimate the penetrance of an exon 1 large SDHB deletion in one kindred. A retrospective and prospective study of 41 relatives across five generations was carried out. The main outcome measures were genetic testing, clinical presentations, plasma catecholamines and their O-methylated metabolites. Of the 41 mutation carriers identified, 11 were diagnosed with PGL, 12 were found to be healthy carriers after evaluation, and 18 were reportedly healthy based on family history accounts. The penetrance of PGL related to the exon 1 large SDHB deletion in this family was estimated to be 35% by age 40. Variable expressivity of the phenotype associated with a large exon 1 SDHB deletion was observed, including low penetrance, diverse primary PGL tumor locations, and malignant potential.

  9. Double ionization chamber survey meter for the separate measurement of penetrating and non-penetrating dose

    International Nuclear Information System (INIS)

    Lucas, A.C.

    1987-01-01

    The full capabilities of an advanced 8-bit microprocessor have been utilized in construction of a wide range, multiplexing survey meter based on dual electrometers and ionization chambers. The ionization chambers are constructed of modular conducting and non-conducting parts in such a way that the angular dependence for measurement of beta radiation is controlled by design. Display functions for the high range instrument include logarithmic or linear analog display, digital display of rate or dose, SI or English units, optionally for either total, penetrating, or non-penetrating dose. The instrument is presently configured to operate in the range 0.1 R/hr to 50,000 R/hr in support of the requirements of Regulatory Guide 19.7

  10. Characteristics of rain penetration through a gravity ventilator used for natural ventilation.

    Science.gov (United States)

    Kim, Taehyeung; Lee, Dong Ho; Ahn, Kwangseog; Ha, Hyunchul; Park, Heechang; Piao, Cheng Xu; Li, Xiaoyu; Seo, Jeoungyoon

    2008-01-01

    Gravity ventilators rely simply on air buoyancy to extract air and are widely used to exhaust air contaminants and heat from workplaces using minimal energy. They are designed to maximize the exhaust flow rate, but the rain penetration sometimes causes malfunctioning. In this study, the characteristics of rain penetration through a ventilator were examined as a preliminary study to develop a ventilator with the maximum exhaust capacity while minimizing rain penetration. A model ventilator was built and exposed to artificial rain and wind. The paths, intensities and amounts of penetration through the ventilator were observed and measured in qualitative and quantitative fashions. In the first phase, the pathways and intensities of rain penetration were visually observed. In the second phase, the amounts of rain penetration were quantitatively measured under the different configurations of ventilator components that were installed based on the information obtained in the first-phase experiment. The effects of wind speed, grill direction, rain drainage width, outer wall height, neck height and leaning angle of the outer wall from the vertical position were analyzed. Wind speed significantly affected rain penetration. Under the low crosswind conditions, the rain penetration intensities were under the limit of detection. Under the high crosswind conditions, grill direction and neck height were the most significant factors in reducing rain penetration. The installation of rain drainage was also important in reducing rain penetration. The experimental results suggest that, with proper configurations of its components, a gravity ventilator can be used for natural ventilation without significant rain penetration problems.

  11. FAA Fluorescent Penetrant Activities - An Update

    Energy Technology Data Exchange (ETDEWEB)

    Moore, D.G.

    1998-10-20

    The Federal Aviation Administration's Airworthiness Assurance NDI Validation Center (AANC) is currently characterizing low cycle fatigue specimens that will support the needs of penetrant manufacturers, commercial airline industry and the Federal Aviation Administration. The main focus of this characterization is to maintain and enhance the evaluation of penetrant inspection materials and apply resources to support the aircraft community needs. This paper discusses efforts to-date to document the Wright Laboratory penetrant evaluation process and characterize penetrant brightness readings in the initial set of sample calibration panels using Type 1 penetrant.

  12. Web penetration testing with Kali Linux

    CERN Document Server

    Muniz, Joseph

    2013-01-01

    Web Penetration Testing with Kali Linux contains various penetration testing methods using BackTrack that will be used by the reader. It contains clear step-by-step instructions with lot of screenshots. It is written in an easy to understand language which will further simplify the understanding for the user.""Web Penetration Testing with Kali Linux"" is ideal for anyone who is interested in learning how to become a penetration tester. It will also help the users who are new to Kali Linux and want to learn the features and differences in Kali versus Backtrack, and seasoned penetration testers

  13. A novel IMPDH1 mutation (Arg231Pro) in a family with a severe form of autosomal dominant retinitis pigmentosa.

    Science.gov (United States)

    Grover, Sandeep; Fishman, Gerald A; Stone, Edwin M

    2004-10-01

    To define ophthalmic findings in a family with autosomal dominant retinitis pigmentosa and a novel IMPDH1 gene mutation. Genetic and observational family study. Sixteen affected members of a family with autosomal dominant retinitis pigmentosa. Ophthalmic examination, including best-corrected visual acuity (VA), slit-lamp biomicroscopy, direct and indirect ophthalmoscopy, Goldmann kinetic perimetry, and electroretinography were performed. Deoxyribonucleic acid single-strand conformation polymorphism (SSCP) analysis was done. Abnormal polymerase chain reaction products identified by SSCP analysis were sequenced bidirectionally. All affected patients had the onset of night blindness within the first decade of life. Ocular findings were characterized by diffuse retinal pigmentary degenerative changes, marked restriction of peripheral visual fields, severe loss of VA, nondetectable electroretinography amplitudes, and a high frequency of posterior subcapsular lens opacities. Affected members were observed to harbor a novel IMPDH1 gene mutation. A novel IMPDH1 gene mutation (Arg231Pro) was associated with a severe form of autosomal dominant retinitis pigmentosa. Families affected with a severe form of this genetic subtype should be investigated for a mutation in the IMPDH1 gene.

  14. Genomic portrait of population of Jharkhand, India, drawn with 15 autosomal STRs and 17 Y-STRs.

    Science.gov (United States)

    Imam, Jahangir; Reyaz, Romana; Singh, Rama Shankar; Bapuly, Arun Kumar; Shrivastava, Pankaj

    2018-01-01

    We analysed 15 autosomal STRs in 200 unrelated individuals (102 males and 98 females) and 17 Y-STRs in 102 unrelated males living in Jharkhand, India, to establish parameters of forensic interest. The examined autosomal STRs revealed high combined power of exclusion (CPE) and combined power of discrimination (CPD) as equal to 0.9999 and greater than 0.99999, respectively. The combined probability of match (CP m ) and combined paternity index (CPI) for all 15 autosomal STR loci were found to be 5.15 × 10 -18 and 6.83 × 10 5 , respectively. A total of 97 unique haplotypes were obtained, of which 93 were observed only once. The haplotype diversity, discrimination capacity and matching probability for 17 Y-STR loci were 0.999, 0.951 and 1.09 × 10 -2 , respectively. The highest gene diversity values at the single copy locus DYS635 and multi-copy locus DYS385 a/b were 0.785 and 0.823, respectively.

  15. Evolutionary history of continental southeast Asians: "early train" hypothesis based on genetic analysis of mitochondrial and autosomal DNA data.

    Science.gov (United States)

    Jinam, Timothy A; Hong, Lih-Chun; Phipps, Maude E; Stoneking, Mark; Ameen, Mahmood; Edo, Juli; Saitou, Naruya

    2012-11-01

    The population history of the indigenous populations in island Southeast Asia is generally accepted to have been shaped by two major migrations: the ancient "Out of Africa" migration ∼50,000 years before present (YBP) and the relatively recent "Out of Taiwan" expansion of Austronesian agriculturalists approximately 5,000 YBP. The Negritos are believed to have originated from the ancient migration, whereas the majority of island Southeast Asians are associated with the Austronesian expansion. We determined 86 mitochondrial DNA (mtDNA) complete genome sequences in four indigenous Malaysian populations, together with a reanalysis of published autosomal single-nucleotide polymorphism (SNP) data of Southeast Asians to test the plausibility and impact of those migration models. The three Austronesian groups (Bidayuh, Selatar, and Temuan) showed high frequencies of mtDNA haplogroups, which originated from the Asian mainland ∼30,000-10,000 YBP, but low frequencies of "Out of Taiwan" markers. Principal component analysis and phylogenetic analysis using autosomal SNP data indicate a dichotomy between continental and island Austronesian groups. We argue that both the mtDNA and autosomal data suggest an "Early Train" migration originating from Indochina or South China around the late-Pleistocene to early-Holocene period, which predates, but may not necessarily exclude, the Austronesian expansion.

  16. Penetration through the Skin Barrier

    DEFF Research Database (Denmark)

    Nielsen, Jesper Bo; Benfeldt, Eva; Holmgaard, Rikke

    2016-01-01

    The skin is a strong and flexible organ with barrier properties essential for maintaining homeostasis and thereby human life. Characterizing this barrier is the ability to prevent some chemicals from crossing the barrier while allowing others, including medicinal products, to pass at varying rates......-through diffusion cells) as well as in vivo methods (microdialysis and microperfusion). Then follows a discussion with examples of how different characteristics of the skin (age, site and integrity) and of the penetrants (size, solubility, ionization, logPow and vehicles) affect the kinetics of percutaneous...

  17. Penetrating abdominal injuries: management controversies

    Science.gov (United States)

    Butt, Muhammad U; Zacharias, Nikolaos; Velmahos, George C

    2009-01-01

    Penetrating abdominal injuries have been traditionally managed by routine laparotomy. New understanding of trajectories, potential for organ injury, and correlation with advanced radiographic imaging has allowed a shift towards non-operative management of appropriate cases. Although a selective approach has been established for stab wounds, the management of abdominal gunshot wounds remains a matter of controversy. In this chapter we describe the rationale and methodology of selecting patients for non-operative management. We also discuss additional controversial issues, as related to antibiotic prophylaxis, management of asymptomatic thoracoabdominal injuries, and the use of colostomy vs. primary repair for colon injuries. PMID:19374761

  18. Penetrating abdominal injuries: management controversies

    Directory of Open Access Journals (Sweden)

    Velmahos George C

    2009-04-01

    Full Text Available Abstract Penetrating abdominal injuries have been traditionally managed by routine laparotomy. New understanding of trajectories, potential for organ injury, and correlation with advanced radiographic imaging has allowed a shift towards non-operative management of appropriate cases. Although a selective approach has been established for stab wounds, the management of abdominal gunshot wounds remains a matter of controversy. In this chapter we describe the rationale and methodology of selecting patients for non-operative management. We also discuss additional controversial issues, as related to antibiotic prophylaxis, management of asymptomatic thoracoabdominal injuries, and the use of colostomy vs. primary repair for colon injuries.

  19. Penetration testing with Raspberry Pi

    CERN Document Server

    Muniz, Joseph

    2015-01-01

    If you are looking for a low budget, small form-factor remotely accessible hacking tool, then the concepts in this book are ideal for you. If you are a penetration tester who wants to save on travel costs by placing a low-cost node on a target network, you will save thousands by using the methods covered in this book. You do not have to be a skilled hacker or programmer to use this book. It will be beneficial to have some networking experience; however, it is not required to follow the concepts covered in this book.

  20. Correlation Between Cone Penetration Rate And Measured Cone Penetration Parameters In Silty Soils

    DEFF Research Database (Denmark)

    Poulsen, Rikke; Nielsen, Benjaminn Nordahl; Ibsen, Lars Bo

    2013-01-01

    This paper shows, how a change in cone penetration rate affects the cone penetration measurements, hence the cone resistance, pore pressure, and sleeve friction in silty soil. The standard rate of penetration is 20 mm/s, and it is generally accepted that undrained penetration occurs in clay while...... drained penetration occurs in sand. When lowering the penetration rate, the soil pore water starts to dissipate and a change in the drainage condition is seen. In intermediate soils such as silty soils, the standard cone penetration rate may result in a drainage condition that could be undrained......, partially or fully drained. However, lowering the penetration rate in silty soils has a great significance because of the soil permeability, and only a small change in penetration rate will result in changed cone penetration measurements. In this paper, analyses will be done on data from 15 field cone...

  1. Autism in siblings with autosomal dominant nocturnal frontal lobe epilepsy.

    Science.gov (United States)

    Miyajima, Tomoko; Kumada, Tomohiro; Saito, Keiko; Fujii, Tatsuya

    2013-02-01

    In 1999, Hirose et al. reported a Japanese family with autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) associated with a neuronal nicotinic acetylcholine receptor α4 subunit mutation (S252L). We followed the siblings of this family, and found that the elder brother had Asperger's disorder without mental retardation (MR) and the younger brother had autistic disorder with profound MR. The clinical epileptic features of the siblings were very similar, and both had deficits in socialization, but their cognitive development differed markedly. It thus seems that epilepsy is the direct phenotype of the S252L mutation, whereas other various factors modulate the cognitive and social development. No patients with ADNFLE have previously been reported to have autism spectrum disorder or profound MR. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  2. Autosomal dominant inheritance Caffey-Silverman disease hyperostosis corticalis infantum

    International Nuclear Information System (INIS)

    Rogoyski, A.; Jakubowska, K.; Tronowska, T.D.

    1984-01-01

    A case of Caffey-Silverman disease is described in an infant aged 4.5 months. The case was erroneously diagnosed in the initial stage of the disease as osteitis. The correct diagnosis was established after radiological examination of the skeleton. The pathological lesions involved the mandible, both clavicles, all ribs, left shoulder blade, both radial bones and left ulna. Follow-up radiological examination after 12 months demonstrated nearly complete disappearance of the previously observed skeletal changes. At the age of 18 months the condition of the child was good and its development was normal. Radiological changes indicating past Caffey-Silverman disease were disclosed in the mother and maternal grandmother of the child. This indicates an autosomal dominant type of inheritance of the disease. (Author)

  3. A Japanese Family With Autosomal Dominant Oculocutaneous Albinism Type 4.

    Science.gov (United States)

    Oki, Ryoko; Yamada, Kisaburo; Nakano, Satoko; Kimoto, Kenichi; Yamamoto, Ken; Kondo, Hiroyuki; Kubota, Toshiaki

    2017-02-01

    We report the clinical characteristics of a Japanese family with autosomal dominant oculocutaneous albinism and a SLC45A2 gene mutation. A total of 16 members of a Japanese family with general hypopigmentation and foveal hypoplasia underwent detailed clinical examinations. We evaluated the severity of foveal hypoplasia using spectral-domain optical coherence tomography (SD-OCT) and graded it according to the criteria of Thomas et al. DNA was extracted from 17 family members and used for genome-wide single nucleotide polymorphism genotyping and linkage analysis. Mutational search was performed for the SLC45A2 gene responsible for oculocutaneous albinism type 4 (OCA4). All 16 patients exhibited hypopigmentation of their hair and/or iris. They showed foveal hypoplasia, including 3 patients with grade 1 foveal hypoplasia, 7 with grade 2, and 6 with grade 3. No patient had grade 4 foveal hypoplasia. Optical coherence tomography showed macular ganglion cell complex thinning in the temporal area, and a slight reduction of visual field sensitivity in the centrotemporal area. A maximum multipoint parametric logarithm of the odds (LOD) score of approximately 2.00 to 3.56 was obtained on chromosome 5, spanning approximately 7.2 Mb between rs13187570 and rs395967 that included the SLC45A2 gene. All affected members showed a novel heterozygous variant, c.208T>C (p.Y70H), in the SLC45A2 gene, which supported a diagnosis of OCA4. The present study reports a very rare family with autosomal dominant OCA4 whose diagnosis was confirmed by a mutational analysis. Most family members exhibited mild general hypopigmentation and low-grade foveal hypoplasia.

  4. Linguistic Intuitions and Cognitive Penetrability

    Directory of Open Access Journals (Sweden)

    Michael Devitt

    2014-12-01

    Full Text Available Metalinguistic intuitions play a very large evidential role in both linguistics and philosophy. Linguists think that these intuitions are products of underlying linguistic competence. I call this view “the voice of competence” (“VoC”. Although many philosophers seem to think that metalinguistic intuitions are a priori many may implicitly hold the more scientifically respectable VoC. According to VoC, I argue, these intuitions can be cognitively penetrated by the central processor. But, I have argued elsewhere, VoC is false. Instead, we should hold “the modest explanation” (“ME” according to which these intuitions are fairly unreflective empirical theory-laden central-processor responses to phenomena. On ME, no question of cognitive penetration arises. ME has great methodological significance for the study of language. Insofar as we rely on intuitions as evidence we should prefer those of linguists and philosophers because they are more expert. But, more importantly, we should be seeking other evidence in linguistic usage.

  5. Prenatal diagnosis of recurrent autosomal dominant osteogenesis imperfecta associated with unaffected parents and paternal gonadal mosaicism

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2013-03-01

    Conclusion: Recurrent autosomal dominant OI may occur in the offspring of unaffected parents with parental gonadal mosaicism. Genetic counseling of recurrent autosomal dominant OI should include a thorough mutational analysis of the family members, and mutational analysis of the sperm may detect paternal gonadal mosaicism for the mutation.

  6. Chorioretinal dysplasia-microcephaly-mental retardation syndrome : Another family with autosomal dominant inheritance

    NARCIS (Netherlands)

    Hordijk, R; VandeLogt, F; Houtman, WA; VanEssen, AJ

    1996-01-01

    We describe a boy and his father with the chorioretinal dysplasia-microcephaly-mental retardation syndrome (CDMMS). Our report extends the phenotypic spectrum of autosomal dominant CDMMS by describing microphthalmia for the first time in an autosomal dominant family. The boy was also severely

  7. Autosomal recessive mode of inheritance of a Coffin-Siris like syndrome.

    Science.gov (United States)

    Bonioli, E; Palmieri, A; Bertola, A; Bellini, C

    1995-01-01

    Autosomal recessive mode of inheritance of a Coffin-Siris like syndrome: Coffin-Siris syndrome is a rare mental retardation/multiple congenital anomalies syndrome; so far its pattern of inheritance is under debate. We report a child affected by this syndrome, the pedigree of which is consistent with autosomal recessive inheritance.

  8. Autosomal recessive retinitis pigmentosa with RP1 mutations is associated with myopia

    NARCIS (Netherlands)

    Chassine, T.; Bocquet, B.; Daien, V.; Avila-Fernandez, A.; Ayuso, C.; Collin, R.W.J.; Corton, M.; Hejtmancik, J.F.; Born, L.I. van den; Klevering, B.J.; Riazuddin, S.A.; Sendon, N.; Lacroux, A.; Meunier, I.; Hamel, C.P.

    2015-01-01

    OBJECTIVE: To determine the refractive error in patients with autosomal recessive retinitis pigmentosa (arRP) caused by RP1 mutations and to compare it with that of other genetic subtypes of RP. METHODS: Twenty-six individuals had arRP with RP1 mutations, 25 had autosomal dominant RP (adRP) with RP1

  9. Progeria (Hutchison - Gilford syndrome in siblings: In an autosomal recessive pattern of inheritance

    Directory of Open Access Journals (Sweden)

    Raghu Tanjore

    2001-09-01

    Full Text Available Progeria is an autosomal dominant, premature aging syndrome. Six and three year old female siblings had sclcrodermatous changes over the extremities, alopecia, beaked nose, prominent veins and bird-like facies. Radiological features were consistent with features of progeria. The present case highlights rarity of progeria in siblings with a possible autosomal recessive pattern.

  10. Novel LMNA Mutation in a Taiwanese Family with Autosomal Dominant Emery-Dreifuss Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Wen-Chen Liang

    2007-01-01

    Full Text Available Emery-Dreifuss muscular dystrophy (EDMD is characterized by early-onset contractures, slowly progressive weakness, and muscle wasting in humeroperoneal muscles, and adult-onset cardiomyopathy with conduction block. We analyzed blood samples from an EDMD family, including a mother and two daughters, and found a novel mutation in codon 520 in exon 9 of the lamin A/C (LMNA gene, resulting in a substitution of tryptophan (W by glycine (G in all three patients. The mother died after a stroke-like episode at the age of 43. The elder sister received pacemaker implantation, which improved symptoms of exercise intolerance and dizziness. These cases illustrate the necessity of correct diagnosis, evaluation, and follow-up of cardiac problems due to the wide clinical spectrum and high prevalence of cardiac conduction block in patients with autosomal dominant EDMD. [J Formos Med Assoc 2007;106(2 Suppl:S27-S31

  11. Follicular and percutaneous penetration pathways of topically applied minoxidil foam.

    Science.gov (United States)

    Blume-Peytavi, Ulrike; Massoudy, Lida; Patzelt, Alexa; Lademann, Jürgen; Dietz, Ekkehart; Rasulev, Utkur; Garcia Bartels, Natalie

    2010-11-01

    In the past, it was assumed that the intercellular route was the only relevant penetration pathway for topically applied substances. Recent results on follicular penetration emphasize that the hair follicles represent a highly relevant and efficient penetration pathway and reservoir for topically applied substances. This study investigates a selective closure technique of hair follicle orifices in vivo assessing interfollicular and follicular absorption rates of topical minoxidil foam in humans. In delimited skin area, single hair orifices or interfollicular skin were blocked with a microdrop of special varnish-wax-mixture in vivo. Minoxidil foam (5%) was topically applied, and transcutaneous absorption was measured by a new surface ionization mass spectrometry technique in serum. Different settings (open, closed or none of both) enabled to clearly distinguish between interfollicular and follicular penetration of the topically applied minoxidil foam. Five minutes after topical application, minoxidil was detected in blood samples when follicles remained open, whereas with closed follicles 30 min were needed. Highest levels were found first when both pathways were open, followed by open follicles and subsequently by closed follicles. These results demonstrate the high importance of the follicular penetration pathway. Hair follicles are surrounded by a dense network of blood capillaries and dendritic cells and have stem cells in their immediate vicinity, making them ideal targets for drug delivery. Copyright © 2010 Elsevier B.V. All rights reserved.

  12. Cable Braid Electromagnetic Penetration Model.

    Energy Technology Data Exchange (ETDEWEB)

    Warne, Larry K. [Sandia National Laboratories (SNL-NM), Albuquerque, NM (United States); Langston, William L. [Sandia National Laboratories (SNL-NM), Albuquerque, NM (United States); Basilio, Lorena I. [Sandia National Laboratories (SNL-NM), Albuquerque, NM (United States); Johnson, W. A. [Sandia National Laboratories (SNL-NM), Albuquerque, NM (United States)

    2015-06-01

    The model for penetration of a wire braid is rigorously formulated. Integral formulas are developed from energy principles and reciprocity for both self and transfer immittances in terms of potentials for the fields. The detailed boundary value problem for the wire braid is also setup in a very efficient manner; the braid wires act as sources for the potentials in the form of a sequence of line multipoles with unknown coefficients that are determined by means of conditions arising from the wire surface boundary conditions. Approximations are introduced to relate the local properties of the braid wires to a simplified infinite periodic planar geometry. This is used in a simplified application of reciprocity to be able to treat nonuniform coaxial geometries including eccentric interior coaxial arrangements and an exterior ground plane.

  13. Microstructured barbs on the North American porcupine quill enable easy tissue penetration and difficult removal

    OpenAIRE

    Cho, Woo Kyung; Ankrum, James A.; Guo, Dagang; Chester, Shawn A.; Yang, Seung Yun; Kashyap, Anurag; Campbell, Georgina A.; Wood, Robert J.; Rijal, Ram K.; Karnik, Rohit; Langer, Robert; Karp, Jeffrey M.

    2012-01-01

    North American porcupines are well known for their specialized hairs, or quills that feature microscopic backward-facing deployable barbs that are used in self-defense. Herein we show that the natural quill’s geometry enables easy penetration and high tissue adhesion where the barbs specifically contribute to adhesion and unexpectedly, dramatically reduce the force required to penetrate tissue. Reduced penetration force is achieved by topography that appears to create stress concentrations al...

  14. Measurement of Skin Permeation/Penetration of Nanoparticles for Their Safety Evaluation

    OpenAIRE

    木村, 恵理子; 河野, 雄一郎; 藤堂, 浩明; 五十嵐, 良明; 杉林, 堅次

    2012-01-01

    The aim of the present study was to quantitatively evaluate the skin permeation/penetration of nanomaterials and to consider their penetration pathway through skin. Firstly, penetration/permeation of a model fluorescent nanoparticle, Fluoresbrite?, was determined through intact rat skin and several damaged skins. Fluoresbrite? permeated through only needle-punctured skin. The permeation profiles of soluble high molecular compounds, fluorescein isothiocyanate-dextrans (FITC-dextrans, FDs), wit...

  15. Bodily action penetrates affective perception

    Science.gov (United States)

    Rigutti, Sara; Gerbino, Walter

    2016-01-01

    Fantoni & Gerbino (2014) showed that subtle postural shifts associated with reaching can have a strong hedonic impact and affect how actors experience facial expressions of emotion. Using a novel Motor Action Mood Induction Procedure (MAMIP), they found consistent congruency effects in participants who performed a facial emotion identification task after a sequence of visually-guided reaches: a face perceived as neutral in a baseline condition appeared slightly happy after comfortable actions and slightly angry after uncomfortable actions. However, skeptics about the penetrability of perception (Zeimbekis & Raftopoulos, 2015) would consider such evidence insufficient to demonstrate that observer’s internal states induced by action comfort/discomfort affect perception in a top-down fashion. The action-modulated mood might have produced a back-end memory effect capable of affecting post-perceptual and decision processing, but not front-end perception. Here, we present evidence that performing a facial emotion detection (not identification) task after MAMIP exhibits systematic mood-congruent sensitivity changes, rather than response bias changes attributable to cognitive set shifts; i.e., we show that observer’s internal states induced by bodily action can modulate affective perception. The detection threshold for happiness was lower after fifty comfortable than uncomfortable reaches; while the detection threshold for anger was lower after fifty uncomfortable than comfortable reaches. Action valence induced an overall sensitivity improvement in detecting subtle variations of congruent facial expressions (happiness after positive comfortable actions, anger after negative uncomfortable actions), in the absence of significant response bias shifts. Notably, both comfortable and uncomfortable reaches impact sensitivity in an approximately symmetric way relative to a baseline inaction condition. All of these constitute compelling evidence of a genuine top-down effect on

  16. Bodily action penetrates affective perception

    Directory of Open Access Journals (Sweden)

    Carlo Fantoni

    2016-02-01

    Full Text Available Fantoni & Gerbino (2014 showed that subtle postural shifts associated with reaching can have a strong hedonic impact and affect how actors experience facial expressions of emotion. Using a novel Motor Action Mood Induction Procedure (MAMIP, they found consistent congruency effects in participants who performed a facial emotion identification task after a sequence of visually-guided reaches: a face perceived as neutral in a baseline condition appeared slightly happy after comfortable actions and slightly angry after uncomfortable actions. However, skeptics about the penetrability of perception (Zeimbekis & Raftopoulos, 2015 would consider such evidence insufficient to demonstrate that observer’s internal states induced by action comfort/discomfort affect perception in a top-down fashion. The action-modulated mood might have produced a back-end memory effect capable of affecting post-perceptual and decision processing, but not front-end perception. Here, we present evidence that performing a facial emotion detection (not identification task after MAMIP exhibits systematic mood-congruent sensitivity changes, rather than response bias changes attributable to cognitive set shifts; i.e., we show that observer’s internal states induced by bodily action can modulate affective perception. The detection threshold for happiness was lower after fifty comfortable than uncomfortable reaches; while the detection threshold for anger was lower after fifty uncomfortable than comfortable reaches. Action valence induced an overall sensitivity improvement in detecting subtle variations of congruent facial expressions (happiness after positive comfortable actions, anger after negative uncomfortable actions, in the absence of significant response bias shifts. Notably, both comfortable and uncomfortable reaches impact sensitivity in an approximately symmetric way relative to a baseline inaction condition. All of these constitute compelling evidence of a genuine top

  17. Varieties of cognitive penetration in visual perception.

    Science.gov (United States)

    Vetter, Petra; Newen, Albert

    2014-07-01

    Is our perceptual experience a veridical representation of the world or is it a product of our beliefs and past experiences? Cognitive penetration describes the influence of higher level cognitive factors on perceptual experience and has been a debated topic in philosophy of mind and cognitive science. Here, we focus on visual perception, particularly early vision, and how it is affected by contextual expectations and memorized cognitive contents. We argue for cognitive penetration based on recent empirical evidence demonstrating contextual and top-down influences on early visual processes. On the basis of a perceptual model, we propose different types of cognitive penetration depending on the processing level on which the penetration happens and depending on where the penetrating influence comes from. Our proposal has two consequences: (1) the traditional controversy on whether cognitive penetration occurs or not is ill posed, and (2) a clear-cut perception-cognition boundary cannot be maintained. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Percutaneous penetration studies for risk assessment

    DEFF Research Database (Denmark)

    Sartorelli, Vittorio; Andersen, Helle Raun; Angerer, Jürgen

    2000-01-01

    . In order to predict the systemic risk of dermally absorbed chemicals and to enable agencies to set safety standards, data is needed on the rates of percutaneous penetration of important chemicals. Standardization of in vitro tests and comparison of their results with the in vivo data could produce...... internationally accepted penetration rates and/or absorption percentages very useful for regulatory toxicology. The work of the Percutaneous Penetration Subgroup of EC Dermal Exposure Network has been focussed on the standardization and validation of in vitro experiments, necessary to obtain internationally...... accepted penetration rates for regulatory purposes. The members of the Subgroup analyzed the guidelines on percutaneous penetration in vitro studies presented by various organizations and suggested a standardization of in vitro models for percutaneous penetration taking into account their individual...

  19. Ethical hacking and penetration testing guide

    CERN Document Server

    Baloch, Rafay

    2014-01-01

    Requiring no prior hacking experience, Ethical Hacking and Penetration Testing Guide supplies a complete introduction to the steps required to complete a penetration test, or ethical hack, from beginning to end. You will learn how to properly utilize and interpret the results of modern-day hacking tools, which are required to complete a penetration test. The book covers a wide range of tools, including Backtrack Linux, Google reconnaissance, MetaGooFil, dig, Nmap, Nessus, Metasploit, Fast Track Autopwn, Netcat, and Hacker Defender rootkit. Supplying a simple and clean explanation of how to effectively utilize these tools, it details a four-step methodology for conducting an effective penetration test or hack.Providing an accessible introduction to penetration testing and hacking, the book supplies you with a fundamental understanding of offensive security. After completing the book you will be prepared to take on in-depth and advanced topics in hacking and penetration testing. The book walks you through each ...

  20. Application of Uintah-MPM to shaped charge jet penetration of aluminum

    International Nuclear Information System (INIS)

    Burghardt, J; Leavy, B; Brannon, R; Guilkey, J; Xue, Z

    2010-01-01

    The capability of the generalized interpolation material point (GIMP) method in simulation of penetration events is investigated. A series of experiments was performed wherein a shaped charge jet penetrates into a stack of aluminum plates. Electronic switches were used to measure the penetration time history. Flash x-ray techniques were used to measure the density, length, radius and velocity of the shaped charge jet. Simulations of the penetration event were performed using the Uintah MPM/GIMP code with several different models of the shaped charge jet being used. The predicted penetration time history for each jet model is compared with the experimentally observed penetration history. It was found that the characteristics of the predicted penetration were dependent on the way that the jet data are translated to a discrete description. The discrete jet descriptions were modified such that the predicted penetration histories fell very close to the range of the experimental data. In comparing the various discrete jet descriptions it was found that the cumulative kinetic energy flux curve represents an important way of characterizing the penetration characteristics of the jet. The GIMP method was found to be well suited for simulation of high rate penetration events.

  1. Initial response of a rock penetrator

    International Nuclear Information System (INIS)

    Longcope, D.B.; Grady, D.E.

    1977-12-01

    An analysis based on elastic rod theory is given for the early-time axisymmetric response of pointed penetrators. Results of measurements by laser interferometry of the back surface particle velocity of laboratory scale penetrators impacted by sandstone targets are presented. Values of the initial pressure on the penetrator tip are determined which give good agreement between the analytical and experimental results. These initial tip pressures are found to be approximated by the stress-particle velocity Hugoniot for the target material

  2. Longitudinal Assessment of Left Ventricular Mass in Autosomal Dominant Polycystic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Taimur Dad

    2018-05-01

    Full Text Available Introduction: The high burden of cardiovascular morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD is related to development of hypertension and left ventricular hypertrophy. Blood pressure reduction has been shown to reduce left ventricular mass in ADPKD; however, moderators and predictors of response to lower blood pressure are unknown. Methods: This was a post hoc cohort analysis of HALT PKD study A, a randomized placebo controlled trial examining the effect of low blood pressure and single versus dual renin−angiotensin blockade in early ADPKD. Participants were hypertensive ADPKD patients 15 to 49 years of age with estimated glomerular filtration rate (eGFR > 60 ml/min per 1.73 m2 across 7 centers in the United States. Predictors included age, sex, baseline eGFR, systolic blood pressure, total kidney volume, serum potassium, and urine sodium, potassium, albumin, and aldosterone. Outcome was left ventricular mass index (LVMI measured using 1.5-T magnetic resonance imaging at months 0, 24, 48, and 60. Results: Reduction in LVMI was associated with higher baseline systolic blood pressure and larger kidney volume regardless of blood pressure control group assignment (P < 0.001 for both. Male sex and baseline eGFR were associated with a positive annual slope in LVMI (P < 0.001 and P = 0.07, respectively. Conclusion: Characteristics associated with higher risk of progression in ADPKD, including higher systolic blood pressure, larger kidney volume, and lower eGFR are associated with improvement in LVMI with intensive blood pressure control, whereas male sex is associated with a smaller slope of reduction in LVMI. Keywords: autosomal dominant polycystic kidney disease, hypertension, left ventricular hypertrophy, left ventricular mass index

  3. Autosomal recessive retinitis pigmentosa with RP1 mutations is associated with myopia.

    Science.gov (United States)

    Chassine, Thomas; Bocquet, Béatrice; Daien, Vincent; Avila-Fernandez, Almudena; Ayuso, Carmen; Collin, Rob Wj; Corton, Marta; Hejtmancik, J Fielding; van den Born, L Ingeborgh; Klevering, B Jeroen; Riazuddin, S Amer; Sendon, Nathacha; Lacroux, Annie; Meunier, Isabelle; Hamel, Christian P

    2015-10-01

    To determine the refractive error in patients with autosomal recessive retinitis pigmentosa (arRP) caused by RP1 mutations and to compare it with that of other genetic subtypes of RP. Twenty-six individuals had arRP with RP1 mutations, 25 had autosomal dominant RP (adRP) with RP1 mutation, 8 and 33 had X-linked RP (xlRP) with RP2 and RPGR mutations, respectively, 198 and 93 had Usher syndrome and arRP without RP1 mutations, respectively. The median of the spherical equivalent (SE) and the IQR (Q25-Q75) was determined and multiple comparisons were performed. arRP patients with RP1 mutations had SE median at -4.0 dioptres (D) OD (Ocula Dextra); -3.88 D OS (Ocula Sinistra), whereas arRP patients without RP1 mutations (-0.50 D OD; -0.75 D OS) and Usher syndrome patients (-0.50 D OD; -0.38 D OS) were significantly less myopic (pUsher syndrome and adRP with RP1 mutation had a narrow IQR (-9.06 to -1.13 D), whereas arRP with RP1 mutations and xlRP with RP2 or RPGR mutations had a larger range (-9.06; -1.13 D). arRP patients with RP1 mutations have myopia not different from patients with xlRP with RP2 or RPGR mutations, while RP patients from other genetic subgroups were emmetropic or mildly myopic. We suggest that arRP patients with high myopic refractive error should be preferentially analysed for RP1 mutations. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  4. Autosomal recessive primary microcephaly (MCPH): clinical manifestations, genetic heterogeneity and mutation continuum

    Science.gov (United States)

    2011-01-01

    Autosomal Recessive Primary Microcephaly (MCPH) is a rare disorder of neurogenic mitosis characterized by reduced head circumference at birth with variable degree of mental retardation. In MCPH patients, brain size reduced to almost one-third of its original volume due to reduced number of generated cerebral cortical neurons during embryonic neurogensis. So far, seven genetic loci (MCPH1-7) for this condition have been mapped with seven corresponding genes (MCPH1, WDR62, CDK5RAP2, CEP152, ASPM, CENPJ, and STIL) identified from different world populations. Contribution of ASPM and WDR62 gene mutations in MCPH World wide is more than 50%. By and large, primary microcephaly patients are phenotypically indistinguishable, however, recent studies in patients with mutations in MCPH1, WDR62 and ASPM genes showed a broader clinical and/or cellular phenotype. It has been proposed that mutations in MCPH genes can cause the disease phenotype by disturbing: 1) orientation of mitotic spindles, 2) chromosome condensation mechanism during embryonic neurogenesis, 3) DNA damage-response signaling, 4) transcriptional regulations and microtubule dynamics, 5) certain unknown centrosomal mechanisms that control the number of neurons generated by neural precursor cells. Recent discoveries of mammalian models for MCPH have open up horizons for researchers to add more knowledge regarding the etiology and pathophysiology of MCPH. High incidence of MCPH in Pakistani population reflects the most probable involvement of consanguinity. Genetic counseling and clinical management through carrier detection/prenatal diagnosis in MCPH families can help reducing the incidence of this autosomal recessive disorder. PMID:21668957

  5. Market penetration rates of new energy technologies

    International Nuclear Information System (INIS)

    Lund, Peter

    2006-01-01

    The market penetration rates of 11 different new energy technologies were studied covering energy production and end-use technologies. The penetration rates were determined by fitting observed market data to an epidemical diffusion model. The analyses show that the exponential penetration rates of new energy technologies may vary from 4 up to over 40%/yr. The corresponding take-over times from a 1% to 50% share of the estimated market potential may vary from less than 10 to 70 years. The lower rate is often associated with larger energy impacts. Short take-over times less than 25 years seem to be mainly associated with end-use technologies. Public policies and subsides have an important effect on the penetration. Some technologies penetrate fast without major support explained by technology maturity and competitive prices, e.g. compact fluorescent lamps show a 24.2%/yr growth rate globally. The penetration rates determined exhibit some uncertainty as penetration has not always proceeded close to saturation. The study indicates a decreasing penetration rate with increasing time or market share. If the market history is short, a temporally decreasing functional form for the penetration rate coefficient could be used to anticipate the probable behavior

  6. Use of ground-penetrating radar techniques in archaeological investigations

    Science.gov (United States)

    Doolittle, James A.; Miller, W. Frank

    1991-01-01

    Ground-penetrating radar (GPR) techniques are increasingly being used to aid reconnaissance and pre-excavation surveys at many archaeological sites. As a 'remote sensing' tool, GPR provides a high resolution graphic profile of the subsurface. Radar profiles are used to detect, identify, and locate buried artifacts. Ground-penetrating radar provides a rapid, cost effective, and nondestructive method for identification and location analyses. The GPR can be used to facilitate excavation strategies, provide greater areal coverage per unit time and cost, minimize the number of unsuccessful exploratory excavations, and reduce unnecessary or unproductive expenditures of time and effort.

  7. Tungsten versus depleted uranium for armour-piercing penetrators

    International Nuclear Information System (INIS)

    Johnson, P.K.

    1983-01-01

    Tungsten alloys have been widely used in the production of armour-piercing (AP) penetrators for defense purposes for the past 40 years. In recent years, however, depleted uranium (DU) has also been utilised for this application. Both materials exhibit high density and strength, two properties necessary for kinetic-energy projectiles to penetrate armour on tanks and other vehicles. The facts, however, support the view that tungsten can and should be utilised as the primary material for most armour-defeating ordnance applications. (author)

  8. Wind tunnel tests of a deep seabed penetrator model

    International Nuclear Information System (INIS)

    Visintini, L.; Murray, C.N.

    1991-01-01

    C.C.R. Euratom Ispra are currently involved in studies on the possibility of storing radioactive wastes in deep ocean sediment beds. The report summarizes the results of wind tunnel tests performed in March 1985 on a 1:2.5 scale model of a European Standard Penetrator in Aermacchi low speed wind tunnel. Tests covered the measurement of overall fluid dynamic forces at varying angle of attack and measurement of unsteady pressures acting on the instrumentation head protruding in the penetrator's wake. Overall force coefficients were found to be in good agreement with predictions. Unsteady pressures were found to be much smaller than expected so that no mechanical damage to instrumentation is to be foreseen even at the high dynamic pressures typical of the penetrator moving into water. The present work has been undertaken under contract 2450-84-08 ED ISP I of C.C.R. EURATOM ISPRA

  9. Cell-penetrating peptides for drug delivery across membrane barriers

    DEFF Research Database (Denmark)

    Foged, Camilla; Nielsen, Hanne Moerck

    2008-01-01

    During the last decade, cell-penetrating peptides have been investigated for their ability to overcome the plasma membrane barrier of mammalian cells for the intracellular or transcellular delivery of cargoes as diverse as low molecular weight drugs, imaging agents, oligonucleotides, peptides......, proteins and colloidal carriers such as liposomes and polymeric nanoparticles. Their ability to cross biological membranes in a non-disruptive way without apparent toxicity is highly desired for increasing drug bioavailability. This review provides an overview of the application of cell......-penetrating peptides as transmembrane drug delivery agents, according to the recent literature, and discusses critical issues and future challenges in relation to fully understanding the fundamental principles of the cell-penetrating peptide-mediated membrane translocation of cargoes and the exploitation...

  10. A case of an intrahepatic fish bone penetration

    International Nuclear Information System (INIS)

    Tsuboi, Kazuhiko; Nakajima, Yoshiro; Yamamoto, Shunji; Nagao, Masatoshi; Nishimura, Kazumasa.

    1981-01-01

    A 56-year-old man was admitted to our hospital because of epigastric discomfort, appetite loss and body weight loss. A gallstone, signs of chronic inflammation and CEA-Z: 12.5 ng/ml were found. Abdominal CT scan revealed an intrahepatic low density nodule and an intra-and-extrahepatic high-dense, needle-like foreign body. By laparotomy a fish bone penetrating into the left lateral segment of the liver from the anterior wall of the prepyloric region of the stomach was found. Cholecystectomy was performed. The penetrating fish bone was withdrawn from the liver easily. The Postoperative course was smooth. The possibility of the definitive preoperative diagnosis of the intestinal fish bone penetration by abdominal CT scan was suggested. (author)

  11. Convective penetration in a young sun

    Science.gov (United States)

    Pratt, Jane; Baraffe, Isabelle; Goffrey, Tom; MUSIC developers group

    2018-01-01

    To interpret the high-quality data produced from recent space-missions it is necessary to study convection under realistic stellar conditions. We describe the multi-dimensional, time implicit, fully compressible, hydrodynamic, implicit large eddy simulation code MUSIC. We use MUSIC to study convection during an early stage in the evolution of our sun where the convection zone covers approximately half of the solar radius. This model of the young sun possesses a realistic stratification in density, temperature, and luminosity. We approach convection in a stellar context using extreme value theory and derive a new model for convective penetration, targeted for one-dimensional stellar evolution calculations. This model provides a scenario that can explain the observed lithium abundance in the sun and in solar-like stars at a range of ages.

  12. A novel approach identifying hybrid sterility QTL on the autosomes of Drosophila simulans and D. mauritiana.

    Science.gov (United States)

    Dickman, Christopher T D; Moehring, Amanda J

    2013-01-01

    When species interbreed, the hybrid offspring that are produced are often sterile. If only one hybrid sex is sterile, it is almost always the heterogametic (XY or ZW) sex. Taking this trend into account, the predominant model used to explain the genetic basis of F1 sterility involves a deleterious interaction between recessive sex-linked loci from one species and dominant autosomal loci from the other species. This model is difficult to evaluate, however, as only a handful of loci influencing interspecies hybrid sterility have been identified, and their autosomal genetic interactors have remained elusive. One hindrance to their identification has been the overwhelming effect of the sex chromosome in mapping studies, which could 'mask' the ability to accurately map autosomal factors. Here, we use a novel approach employing attached-X chromosomes to create reciprocal backcross interspecies hybrid males that have a non-recombinant sex chromosome and recombinant autosomes. The heritable variation in phenotype is thus solely caused by differences in the autosomes, thereby allowing us to accurately identify the number and location of autosomal sterility loci. In one direction of backcross, all males were sterile, indicating that sterility could be entirely induced by the sex chromosome complement in these males. In the other direction, we identified nine quantitative trait loci that account for a surprisingly large amount (56%) of the autosome-induced phenotypic variance in sterility, with a large contribution of autosome-autosome epistatic interactions. These loci are capable of acting dominantly, and thus could contribute to F1 hybrid sterility.

  13. A novel approach identifying hybrid sterility QTL on the autosomes of Drosophila simulans and D. mauritiana.

    Directory of Open Access Journals (Sweden)

    Christopher T D Dickman

    Full Text Available When species interbreed, the hybrid offspring that are produced are often sterile. If only one hybrid sex is sterile, it is almost always the heterogametic (XY or ZW sex. Taking this trend into account, the predominant model used to explain the genetic basis of F1 sterility involves a deleterious interaction between recessive sex-linked loci from one species and dominant autosomal loci from the other species. This model is difficult to evaluate, however, as only a handful of loci influencing interspecies hybrid sterility have been identified, and their autosomal genetic interactors have remained elusive. One hindrance to their identification has been the overwhelming effect of the sex chromosome in mapping studies, which could 'mask' the ability to accurately map autosomal factors. Here, we use a novel approach employing attached-X chromosomes to create reciprocal backcross interspecies hybrid males that have a non-recombinant sex chromosome and recombinant autosomes. The heritable variation in phenotype is thus solely caused by differences in the autosomes, thereby allowing us to accurately identify the number and location of autosomal sterility loci. In one direction of backcross, all males were sterile, indicating that sterility could be entirely induced by the sex chromosome complement in these males. In the other direction, we identified nine quantitative trait loci that account for a surprisingly large amount (56% of the autosome-induced phenotypic variance in sterility, with a large contribution of autosome-autosome epistatic interactions. These loci are capable of acting dominantly, and thus could contribute to F1 hybrid sterility.

  14. Caffeine intake by patients with autosomal dominant polycystic kidney disease

    International Nuclear Information System (INIS)

    Vendramini, L.C.; Nishiura, J.L.; Baxmann, A.C.; Heilberg, I.P.

    2012-01-01

    Because caffeine may induce cyst and kidney enlargement in autosomal dominant polycystic kidney disease (ADPKD), we evaluated caffeine intake and renal volume using renal ultrasound in ADPKD patients. Caffeine intake was estimated by the average of 24-h dietary recalls obtained on 3 nonconsecutive days in 102 ADPKD patients (68 females, 34 males; 39 ± 12 years) and compared to that of 102 healthy volunteers (74 females, 28 males; 38 ± 14 years). The awareness of the need for caffeine restriction was assessed. Clinical and laboratory data were obtained from the medical records of the patients. Mean caffeine intake was significantly lower in ADPKD patients versus controls (86 vs 134 mg/day), and 63% of the ADPKD patients had been previously aware of caffeine restriction. Caffeine intake did not correlate with renal volume in ADPKD patients. There were no significant differences between the renal volumes of patients in the highest and lowest tertiles of caffeine consumption. Finally, age-adjusted multiple linear regression revealed that renal volume was associated with hypertension, chronic kidney disease stage 3 and the time since diagnosis, but not with caffeine intake. The present small cross-sectional study indicated a low level of caffeine consumption by ADPKD patients when compared to healthy volunteers, which was most likely due to prior awareness of the need for caffeine restriction. Within the range of caffeine intake observed by ADPKD patients in this study (0-471 mg/day), the renal volume was not directly associated with caffeine intake

  15. Caffeine intake by patients with autosomal dominant polycystic kidney disease

    Energy Technology Data Exchange (ETDEWEB)

    Vendramini, L.C.; Nishiura, J.L.; Baxmann, A.C.; Heilberg, I.P. [Disciplina de Nefrologia, Departamento de Medicina, Universidade Federal de São Paulo, São Paulo, SP (Brazil)

    2012-07-20

    Because caffeine may induce cyst and kidney enlargement in autosomal dominant polycystic kidney disease (ADPKD), we evaluated caffeine intake and renal volume using renal ultrasound in ADPKD patients. Caffeine intake was estimated by the average of 24-h dietary recalls obtained on 3 nonconsecutive days in 102 ADPKD patients (68 females, 34 males; 39 ± 12 years) and compared to that of 102 healthy volunteers (74 females, 28 males; 38 ± 14 years). The awareness of the need for caffeine restriction was assessed. Clinical and laboratory data were obtained from the medical records of the patients. Mean caffeine intake was significantly lower in ADPKD patients versus controls (86 vs 134 mg/day), and 63% of the ADPKD patients had been previously aware of caffeine restriction. Caffeine intake did not correlate with renal volume in ADPKD patients. There were no significant differences between the renal volumes of patients in the highest and lowest tertiles of caffeine consumption. Finally, age-adjusted multiple linear regression revealed that renal volume was associated with hypertension, chronic kidney disease stage 3 and the time since diagnosis, but not with caffeine intake. The present small cross-sectional study indicated a low level of caffeine consumption by ADPKD patients when compared to healthy volunteers, which was most likely due to prior awareness of the need for caffeine restriction. Within the range of caffeine intake observed by ADPKD patients in this study (0-471 mg/day), the renal volume was not directly associated with caffeine intake.

  16. Autosomal dominant familial erythrocytosis due to autonomous erythropoietin production

    International Nuclear Information System (INIS)

    Distelhorst, C.W.; Wagner, D.S.; Goldwasser, E.; Adamson, J.W.

    1981-01-01

    A family is described in which four members spanning three consecutive generations have erythrocytosis associated with a normal hemoglobin oxygen affinity. When bone marrow from one affected family member was cultured in vitro, erythroid colonies formed only when erythropoietin was added to the culture. Serum erythropoietin, measured by radioimmunoassay, was significantly elevated above normal in each of the affected family members. Bioassayable erythropoietin was detected in the urine of two of the three affected family members. In two of the affected family members, erythropoietin was measured in serum by radioimmunoassay and in urine by bioassay before and for 4 days following an isovolemic phlebotomy, which reduced the red cell mass by 20%. Neither serum nor urinary erythropoietin levels changed following phlebotomy. The erythrocytosis in this family appears to be secondary to inappropriately increased erythropoietin production unassociated with a decrease in the blood oxygen-carrying capacity. This is the first instance in which autonomous erythropoietin production appears to be inherited on an autosomal dominant basis

  17. Spectrum of Autosomal Recessive Congenital Ichthyosis in Scandinavia

    DEFF Research Database (Denmark)

    Hellström Pigg, Maritta; Bygum, Anette; Gånemo, Agneta

    2016-01-01

    Autosomal recessive congenital ichthyosis (ARCI) represents a heterogeneous group of rare disorders of cornification with 3 major subtypes: harlequin ichthyosis (HI), lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE). A 4th subtype has also been proposed: pleomorphic...... ichthyosis (PI), characterized by marked skin changes at birth and subsequently mild symptoms. In nationwide screenings of suspected cases of ARCI in Denmark and Sweden, we identified 132 patients (age range 0.1-86 years) classified as HI (n = 7), LI (n = 70), CIE (n = 17) and PI (n = 38). At birth......-100%). A scoring (0-4) of ichthyosis/ery-thema past infancy showed widely different mean values in the subgroups: HI (3.2/3.1), LI (2.4/0.6), CIE (1.8/1.6), PI (1.1/0.3). Novel or recurrent mutations were found in 113 patients: TGM1 (n = 56), NIPAL4 (n = 15), ALOX12B (n = 15), ABCA12 (n = 8), ALOXE3 (n = 9), SLC27...

  18. Is Autosomal Dominant Polycystic Kidney Disease Becoming a Pediatric Disorder?

    Directory of Open Access Journals (Sweden)

    Stéphanie De Rechter

    2017-12-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD affects 1 in 400 to 1,000 live births, making it the most common monogenic cause of renal failure. Although no definite cure is available yet, it is important to affect disease progression by influencing modifiable factors such as hypertension and proteinuria. Besides this symptomatic management, the only drug currently recommended in Europe for selected adult patients with rapid disease progression, is the vasopressin receptor antagonist tolvaptan. However, the question remains whether these preventive interventions should be initiated before extensive renal damage has occurred. As renal cyst formation and expansion begins early in life, frequently in utero, ADPKD should no longer be considered an adult-onset disease. Moreover, the presence of hypertension and proteinuria in affected children has been reported to correlate well with disease severity. Until now, it is controversial whether children at-risk for ADPKD should be tested for the presence of the disease, and if so, how this should be done. Herein, we review the spectrum of pediatric ADPKD and discuss the pro and contra of testing at-risk children and the challenges and unmet needs in pediatric ADPKD care.

  19. Subjective memory complaints in preclinical autosomal dominant Alzheimer disease.

    Science.gov (United States)

    Norton, Daniel J; Amariglio, Rebecca; Protas, Hillary; Chen, Kewei; Aguirre-Acevedo, Daniel C; Pulsifer, Brendan; Castrillon, Gabriel; Tirado, Victoria; Munoz, Claudia; Tariot, Pierre; Langbaum, Jessica B; Reiman, Eric M; Lopera, Francisco; Sperling, Reisa A; Quiroz, Yakeel T

    2017-10-03

    To cross-sectionally study subjective memory complaints (SMC) in autosomal dominant Alzheimer disease (ADAD). We examined self-reported and study partner-based SMC in 52 young, cognitively unimpaired individuals from a Colombian kindred with early-onset ADAD. Twenty-six carried the PSEN-1 E280A mutation, averaging 7 years of age younger than the kindred's expected clinical onset. Twenty-six were age-matched noncarriers. Participants also underwent structural MRI and cognitive testing. Self-reported SMC were greater in carriers than noncarriers ( p = 0.02). Study partner-based SMC did not differ between groups ( p = 0.21), but in carriers increased with age ( r = 0.66, p < 0.001) and decreased with hippocampal volume ( r = -0.35, p = 0.08). Cognitively unimpaired PSEN-1 carriers have elevated SMC. Self-reported SMC may be a relatively early indicator of preclinical AD, while partner- reported SMC increases later in preclinical AD, closer to clinical onset. © 2017 American Academy of Neurology.

  20. Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children.

    Science.gov (United States)

    Martín-Santiago, A; Rodríguez-Pascual, M; Knöpfel, N; Hernández-Martín, Á

    2015-11-01

    Few studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). To assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. We prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients' medical records. Ear examination and hearing tests were performed in all cases. Ten patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. The involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients. Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.

  1. Novel FAM20A mutation causes autosomal recessive amelogenesis imperfecta.

    Science.gov (United States)

    Volodarsky, Michael; Zilberman, Uri; Birk, Ohad S

    2015-06-01

    To relate the peculiar phenotype of amelogenesis imperfecta in a large Bedouin family to the genotype determined by whole genome linkage analysis. Amelogenesis imperfecta (AI) is a broad group of inherited pathologies affecting enamel formation, characterized by variability in phenotypes, causing mutations and modes of inheritance. Autosomal recessive or compound heterozygous mutations in FAM20A, encoding sequence similarity 20, member A, have been shown to cause several AI phenotypes. Five members from a large consanguineous Bedouin family presented with hypoplastic amelogenesis imperfecta with unerupted and resorbed permanent molars. Following Soroka Medical Center IRB approval and informed consent, blood samples were obtained from six affected offspring, five obligatory carriers and two unaffected siblings. Whole genome linkage analysis was performed followed by Sanger sequencing of FAM20A. The sequencing unravelled a novel homozygous deletion mutation in exon 11 (c.1523delC), predicted to insert a premature stop codon (p.Thr508Lysfs*6). We provide an interesting case of novel mutation in this rare disorder, in which the affected kindred is unique in the large number of family members sharing a similar phenotype. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. Quantitative penetration testing with item response theory

    NARCIS (Netherlands)

    Pieters, W.; Arnold, F.; Stoelinga, M.I.A.

    2013-01-01

    Existing penetration testing approaches assess the vulnerability of a system by determining whether certain attack paths are possible in practice. Therefore, penetration testing has thus far been used as a qualitative research method. To enable quantitative approaches to security risk management,

  3. Generic penetration in the retail antidepressant market.

    Science.gov (United States)

    Ventimiglia, Jeffrey; Kalali, Amir H

    2010-06-01

    In this article, we explore the accelerated penetration of generic antidepressants in the United States market following the availability of generic citalopram and sertraline. Analysis suggests that overall, generic penetration into the antidepressant market has grown from approximately 41 percent in January 2004 to over 73 percent in January 2010. Similar trends are uncovered when branded and generic prescriptions are analyzed by specialty.

  4. Quantitative Penetration Testing with Item Response Theory

    NARCIS (Netherlands)

    Arnold, Florian; Pieters, Wolter; Stoelinga, Mariëlle Ida Antoinette

    2014-01-01

    Existing penetration testing approaches assess the vulnerability of a system by determining whether certain attack paths are possible in practice. Thus, penetration testing has so far been used as a qualitative research method. To enable quantitative approaches to security risk management, including

  5. Quantitative penetration testing with item response theory

    NARCIS (Netherlands)

    Arnold, Florian; Pieters, Wolter; Stoelinga, Mariëlle

    2013-01-01

    Existing penetration testing approaches assess the vulnerability of a system by determining whether certain attack paths are possible in practice. Thus, penetration testing has so far been used as a qualitative research method. To enable quantitative approaches to security risk management, including

  6. Chemical Penetration Enhancers for Transdermal Drug Delivery ...

    African Journals Online (AJOL)

    for transdermal administration. The permeation of drug through skin can be enhanced by both chemical penetration enhancement and physical methods. In this review, we have discussed the chemical penetration enhancement technology for transdermal drug delivery as well as the probable mechanisms of action.

  7. Oculopharyngeal Weakness, Hypophrenia, Deafness, and Impaired Vision: A Novel Autosomal Dominant Myopathy with Rimmed Vacuoles

    Directory of Open Access Journals (Sweden)

    Ting Chen

    2016-01-01

    Conclusions: We reported a novel autosomal dominant myopathy with rimmed vacuoles characterized by dysarthria, dysphagia, external ophthalmoplegia, limb weakness, hypophrenia, deafness, and impaired vision, but the causative gene has not been found and needs further study.

  8. A newly recognized autosomal dominant limb girdle muscular dystrophy with cardiac involvement

    NARCIS (Netherlands)

    van der Kooi, A. J.; Ledderhof, T. M.; de Voogt, W. G.; Res, C. J.; Bouwsma, G.; Troost, D.; Busch, H. F.; Becker, A. E.; de Visser, M.

    1996-01-01

    Sixty-five members of three families with limb girdle muscular dystrophy (LGMD) underwent neurological, cardiological, and ancillary investigations. Thirty-five individuals were diagnosed as having slowly progressive autosomal dominant LGMD. Symmetrical weakness started in the proximal lower limb

  9. Imaging of the Macula Indicates Early Completion of Structural Deficit in Autosomal-Dominant Optic Atrophy

    DEFF Research Database (Denmark)

    Rönnbäck, Cecilia; Milea, Dan; Larsen, Michael

    2013-01-01

    Optical coherence tomography (OCT) enables 3-dimensional imaging of the retina, including the layer of ganglion cells that supplies the optic nerve with its axons. We tested OCT as means of diagnosing and phenotyping autosomal-dominant optic atrophy (ADOA)....

  10. Orientation dependence of deformation and penetration behavior of tungsten single-crystal rods

    International Nuclear Information System (INIS)

    Bruchey, W.J.; Horwath, E.J.; Kingman, P.W.

    1991-01-01

    This paper reports on the performance of tungsten single crystals as kinetic energy penetrator materials that was investigated in a high length-to-diameter (L/D) rod geometry at sub-scale (1/4 geometric scale). The [111]. [110], and [100] crystal orientations were tested in this 74-g LD = 15 geometry penetrator (6.90-mm diameter x 102.5-mm length). Several 93% tungsten alloy and uranium 3/4 titanium rod geometries were also tested to baseline expected performance of typical penetrator material/geometry combinations. Performance was determined for semi-infinite penetration into RHA steel and finite penetration into 76.20-mm RHA steel. Of the orientation tested, the [100] orientation provided the best ballistic results, with superior performance to mass and geometric equivalent 93% tungsten alloy rods. The [100] orientation also provided similar performance to geometric equivalent uranium 3/4 titanium rods. Favorable slip/cleavage during the compressive loading of the penetration process to allow penetrator material flow without large scale plastic deformation, and final shear localization at a favorable angle for easy material flow away from the penetration interface, contribute to the [100] orientation crystals' excellent performance. The net result was less energy expenditure during penetrator flow and, therefore, more energy for deformation of RHA

  11. Molecular and cellular pathogenesis of autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    A.P. Bastos

    2011-07-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is one of the most common human life-threatening monogenic disorders. The disease is characterized by bilateral, progressive renal cystogenesis and cyst and kidney enlargement, often leading to end-stage renal disease, and may include extrarenal manifestations. ADPKD is caused by mutation in one of two genes, PKD1 and PKD2, which encode polycystin-1 (PC1 and polycystin-2 (PC2, respectively. PC2 is a non-selective cation channel permeable to Ca2+, while PC1 is thought to function as a membrane receptor. The cyst cell phenotype includes increased proliferation and apoptosis, dedifferentiation, defective planar polarity, and a secretory pattern associated with extracellular matrix remodeling. The two-hit model for cyst formation has been recently extended by the demonstration that early gene inactivation leads to rapid and diffuse development of renal cysts, while inactivation in adult life is followed by focal and late cyst formation. Renal ischemia/reperfusion, however, can function as a third hit, triggering rapid cyst development in kidneys with Pkd1 inactivation induced in adult life. The PC1-PC2 complex behaves as a sensor in the primary cilium, mediating signal transduction via Ca2+ signaling. The intracellular Ca2+ homeostasis is impaired in ADPKD, being apparently responsible for the cAMP accumulation and abnormal cell proliferative response to cAMP. Activated mammalian target for rapamycin (mTOR and cell cycle dysregulation are also significant features of PKD. Based on the identification of pathways altered in PKD, a large number of preclinical studies have been performed and are underway, providing a basis for clinical trials in ADPKD and helping the design of future trials.

  12. Mutations in REEP6 Cause Autosomal-Recessive Retinitis Pigmentosa.

    Science.gov (United States)

    Arno, Gavin; Agrawal, Smriti A; Eblimit, Aiden; Bellingham, James; Xu, Mingchu; Wang, Feng; Chakarova, Christina; Parfitt, David A; Lane, Amelia; Burgoyne, Thomas; Hull, Sarah; Carss, Keren J; Fiorentino, Alessia; Hayes, Matthew J; Munro, Peter M; Nicols, Ralph; Pontikos, Nikolas; Holder, Graham E; Asomugha, Chinwe; Raymond, F Lucy; Moore, Anthony T; Plagnol, Vincent; Michaelides, Michel; Hardcastle, Alison J; Li, Yumei; Cukras, Catherine; Webster, Andrew R; Cheetham, Michael E; Chen, Rui

    2016-12-01

    Retinitis pigmentosa (RP) is the most frequent form of inherited retinal dystrophy. RP is genetically heterogeneous and the genes identified to date encode proteins involved in a wide range of functional pathways, including photoreceptor development, phototransduction, the retinoid cycle, cilia, and outer segment development. Here we report the identification of biallelic mutations in Receptor Expression Enhancer Protein 6 (REEP6) in seven individuals with autosomal-recessive RP from five unrelated families. REEP6 is a member of the REEP/Yop1 family of proteins that influence the structure of the endoplasmic reticulum but is relatively unstudied. The six variants identified include three frameshift variants, two missense variants, and a genomic rearrangement that disrupts exon 1. Human 3D organoid optic cups were used to investigate REEP6 expression and confirmed the expression of a retina-specific isoform REEP6.1, which is specifically affected by one of the frameshift mutations. Expression of the two missense variants (c.383C>T [p.Pro128Leu] and c.404T>C [p.Leu135Pro]) and the REEP6.1 frameshift mutant in cultured cells suggest that these changes destabilize the protein. Furthermore, CRISPR-Cas9-mediated gene editing was used to produce Reep6 knock-in mice with the p.Leu135Pro RP-associated variant identified in one RP-affected individual. The homozygous knock-in mice mimic the clinical phenotypes of RP, including progressive photoreceptor degeneration and dysfunction of the rod photoreceptors. Therefore, our study implicates REEP6 in retinal homeostasis and highlights a pathway previously uncharacterized in retinal dystrophy. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  13. Autosomal mutations affecting Y chromosome loops in Drosophila melanogaster

    Directory of Open Access Journals (Sweden)

    Petrucci Romano

    2008-04-01

    Full Text Available Abstract Background The Y chromosome of Drosophila melanogaster harbors several genes required for male fertility. The genes for these fertility factors are very large in size and contain conspicuous amounts of repetitive DNA and transposons. Three of these loci (ks-1, kl-3 and kl-5 have the ability to develop giant lampbrush-like loops in primary spermatocytes, a cytological manifestation of their active state in these cells. Y-loops bind a number of non-Y encoded proteins, but the mechanisms regulating their development and their specific functions are still to be elucidated. Results Here we report the results of a screen of 726 male sterile lines to identify novel autosomal genes controlling Y-loop function. We analyzed mutant testis preparations both in vivo and by immunofluorescence using antibodies directed against Y-loop-associated proteins. This screen enabled us to isolate 17 mutations at 15 loci whose wild-type function is required for proper Y-loop morphogenesis. Six of these loci are likely to specifically control loop development, while the others display pleiotropic effects on both loops and meiotic processes such as spermiogenesis, sperm development and maturation. We also determined the map position of the mutations affecting exclusively Y-loop morphology. Conclusion Our cytological screening permitted us to identify novel genetic functions required for male spermatogenesis, some of which show pleiotropic effects. Analysis of these mutations also shows that loop development can be uncoupled from meiosis progression. These data represent a useful framework for the characterization of Y-loop development at a molecular level and for the study of the genetic control of heterochromatin.

  14. Thermo-chemical Ice Penetrator for Icy Moons

    Science.gov (United States)

    Arenberg, J. W.; Lee, G.; Harpole, G.; Zamel, J.; Sen, B.; Ross, F.; Retherford, K. D.

    2016-12-01

    The ability to place sensors or to take samples below the ice surface enables a wide variety of potential scientific investigations. Penetrating an ice cap can be accomplished via a mechanical drill, laser drill, kinetic impactor, or heated penetrator. This poster reports on the development of technology for the latter most option, namely a self-heated probe driven by an exothermic chemical reaction: a Thermo-chemical ice penetrator (TChIP). Our penetrator design employs a eutectic mix of alkali metals that produce an exothermic reaction upon contact with an icy surface. This reaction increases once the ice starts melting, so no external power is required. This technology is inspired by a classified Cold-War era program developed at Northrop Grumman for the US Navy. Terrestrial demonstration of this technology took place in the Arctic; however, this device cannot be considered high TRL for application at the icy moons of the solar system due to the environmental differences between Earth's Arctic and the icy moons. These differences demand a TChIP design specific to these cold, low mass, airless worlds. It is expected that this model of TChIP performance will be complex, incorporating all of the forces on the penetrator, gravity, the thermo-chemistry at the interface between penetrator and ice, and multi-phase heat and mass transport, and hydrodynamics. Our initial efforts are aimed at the development of a validated set of tools and simulations to predict the performance of the penetrator for both the environment found on these icy moons and for a terrestrial environment. The purpose of the inclusion of the terrestrial environment is to aid in model validation. Once developed and validated, our models will allow us to design penetrators for a specific scientific application on a specific body. This poster discusses the range of scientific investigations that are enabled by TChIP. We also introduce the development plan to advance TChIP to the point where it can be

  15. Current Understanding of Physicochemical Mechanisms for Cell Membrane Penetration of Arginine-rich Cell Penetrating Peptides: Role of Glycosaminoglycan Interactions.

    Science.gov (United States)

    Takechi-Haraya, Yuki; Saito, Hiroyuki

    2018-01-01

    Arginine-rich cell penetrating peptides (CPPs) are very promising drug carriers to deliver membrane-impermeable pharmaceuticals, such as siRNA, bioactive peptides and proteins. CPPs directly penetrate into cells across cell membranes via a spontaneous energy-independent process, in which CPPs appear to interact with acidic lipids in the outer leaflet of the cell membrane. However, acidic lipids represent only 10 to 20% of the total membrane lipid content and in mammalian cell membranes they are predominantly located in the inner leaflet. Alternatively, CPPs favorably bind in a charge density- dependent manner to negatively charged, sulfated glycosaminoglycans (GAGs), such as heparan sulfate and chondroitin sulfate, which are abundant on the cell surface and are involved in many biological functions. We have recently demonstrated that the interaction of CPPs with sulfated GAGs plays a critical role in their direct cell membrane penetration: the favorable enthalpy contribution drives the high-affinity binding of arginine-rich CPPs to sulfated GAGs, initiating an efficient cell membrane penetration. The favorable enthalpy gain is presumably mainly derived from a unique property of the guanidino group of arginine residues forming multidentate hydrogen bonding with sulfate and carboxylate groups in GAGs. Such interactions can be accompanied with charge neutralization of arginine-rich CPPs, promoting their partition into cell membranes. This review summarizes the current understanding of the physicochemical mechanism for lipid membrane penetration of CPPs, and discusses the role of the GAG interactions on the cell membrane penetration of CPPs. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  16. Autosomal recessive type II hereditary motor and sensory neuropathy with acrodystrophy.

    Science.gov (United States)

    Thomas, P K; Claus, D; King, R H

    1999-02-01

    A family is described with presumed autosomal recessive inheritance in which three siblings developed a progressive neuropathy that combined limb weakness and severe distal sensory loss leading to prominent mutilating changes. Electrophysiological and nerve biopsy findings indicated an axonopathy. The disorder is therefore classifiable as type II hereditary motor and sensory neuropathy (HMSN II). The clinical features differ from those reported in previously described cases of autosomal recessive HMSN II. This disorder may therefore represent a new variant.

  17. An 11-Year-Old Child with Autosomal Dominant Polycystic Kidney Disease Who Presented with Nephrolithiasis

    Directory of Open Access Journals (Sweden)

    Fatih Firinci

    2012-01-01

    Full Text Available Patients with autosomal dominant polycystic kidney disease become symptomatic and are diagnosed usually at adulthood. The rate of nephrolithiasis in these patients is 5–10 times the rate in the general population, and both anatomic and metabolic abnormalities play role in the formation of renal stones. However, nephrolithiasis is rare in childhood age group. In this paper, an 11-year-old child with autosomal dominant polycystic kidney disease presenting with nephrolithiasis is discussed.

  18. Cellular Energy Pathways as Novel Targets for the Therapy of Autosomal Dominant Polycystic Kidney Disease

    Science.gov (United States)

    2017-09-01

    AWARD NUMBER: W81XWH-15-1-0419 TITLE: Cellular Energy Pathways as Novel Targets for the Therapy of Autosomal Dominant Polycystic Kidney Disease...COVERED 1 Sep 2016 - 31 Aug 2017 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Cellular Energy Pathways as Novel Targets for the Therapy of Autosomal...inappropriate cell growth, fluid secretion, and dysregulation of cellular energy metabolism. The enzyme AMPK regulates a number of cellular pathways, including

  19. Autosomal and uniparental portraits of the native populations of Sakha (Yakutia): implications for the peopling of Northeast Eurasia.

    Science.gov (United States)

    Fedorova, Sardana A; Reidla, Maere; Metspalu, Ene; Metspalu, Mait; Rootsi, Siiri; Tambets, Kristiina; Trofimova, Natalya; Zhadanov, Sergey I; Hooshiar Kashani, Baharak; Olivieri, Anna; Voevoda, Mikhail I; Osipova, Ludmila P; Platonov, Fedor A; Tomsky, Mikhail I; Khusnutdinova, Elza K; Torroni, Antonio; Villems, Richard

    2013-06-19

    Sakha--an area connecting South and Northeast Siberia--is significant for understanding the history of peopling of Northeast Eurasia and the Americas. Previous studies have shown a genetic contiguity between Siberia and East Asia and the key role of South Siberia in the colonization of Siberia. We report the results of a high-resolution phylogenetic analysis of 701 mtDNAs and 318 Y chromosomes from five native populations of Sakha (Yakuts, Evenks, Evens, Yukaghirs and Dolgans) and of the analysis of more than 500,000 autosomal SNPs of 758 individuals from 55 populations, including 40 previously unpublished samples from Siberia. Phylogenetically terminal clades of East Asian mtDNA haplogroups C and D and Y-chromosome haplogroups N1c, N1b and C3, constituting the core of the gene pool of the native populations from Sakha, connect Sakha and South Siberia. Analysis of autosomal SNP data confirms the genetic continuity between Sakha and South Siberia. Maternal lineages D5a2a2, C4a1c, C4a2, C5b1b and the Yakut-specific STR sub-clade of Y-chromosome haplogroup N1c can be linked to a migration of Yakut ancestors, while the paternal lineage C3c was most likely carried to Sakha by the expansion of the Tungusic people. MtDNA haplogroups Z1a1b and Z1a3, present in Yukaghirs, Evens and Dolgans, show traces of different and probably more ancient migration(s). Analysis of both haploid loci and autosomal SNP data revealed only minor genetic components shared between Sakha and the extreme Northeast Siberia. Although the major part of West Eurasian maternal and paternal lineages in Sakha could originate from recent admixture with East Europeans, mtDNA haplogroups H8, H20a and HV1a1a, as well as Y-chromosome haplogroup J, more probably reflect an ancient gene flow from West Eurasia through Central Asia and South Siberia. Our high-resolution phylogenetic dissection of mtDNA and Y-chromosome haplogroups as well as analysis of autosomal SNP data suggests that Sakha was colonized by

  20. The effect of piracetam on ataxia: clinical observations in a group of autosomal dominant cerebellar ataxia patients.

    Science.gov (United States)

    Ince Gunal, D; Agan, K; Afsar, N; Borucu, D; Us, O

    2008-04-01

    Autosomal dominant cerebellar ataxias are clinically and genetically heterogeneous neurodegenerative disorders. There is no known treatment to prevent neuronal cell death in these disorders. Current treatment is purely symptomatic; ataxia is one of the most disabling symptoms and represents the main therapeutic challenge. A previous case report suggesting benefit from administration of high dose piracetam inspired the present study of the efficacy of this agent in patients with cerebellar ataxia. Piracetam is a low molecular weight derivative of gamma-aminobutyric acid. Although little is known of its mode of action, its efficacy has been documented in a wide range of clinical indications, such as cognitive disorders, dementia, vertigo and dyslexia, as well as cortical myoclonus. The present report investigated the role of high dose piracetam in patients with cerebellar ataxia. Eight patients with autosomal dominant cerebellar ataxia were given intravenous piracetam 60 g/day by a structured protocol for 14 days. The baseline and end-of-the study evaluations were based on the International Cooperative Ataxia Rating Scale. Statistical analysis demonstrated a significant improvement in the patients' total score (P = 0.018) and a subscale analysis showed statistical significance for only the posture and gait disturbances item (P = 0.018). This study is providing good clinical observation in favour of high dose piracetam infusion to reduce the disability of the patients by improving their gait ataxia.

  1. Enabling kinetic micro-penetrator technology for Solar System research

    Science.gov (United States)

    Gowen, R. A.

    2008-09-01

    Whilst the concept of high speed impacting penetrator probes is not new, recent highly successful ground test results have considerably improved the perception that these can be a viable and useful addition to the current toolbox of planetary probes. Previous developments only led to a single deployment (Deep Space-2 to Mars on the ill fated NASA Mars Polar Lander mission in 1999) where neither the soft lander nor penetrator was ever heard from, which is not a logical basis for dismissing penetrator technology. Other space penetrator programmes have included the Russian Mars'96 ~80m/s penetrators for which the whole mission was lost before the spacecraft left Earth orbit, and the Japanese Lunar-A program which was cancelled after a lengthy development program which however saw multiple successful ground trials. The Japanese penetrators were designed for ~300m/s impact. The current UK penetrator developments are actively working towards full space qualification for a Lunar penetrators (MoonLITE mission), which would also provide a significant technical demonstration towards the development of smaller, shorter lived penetrators for exploring other solar system objects. We are advocating delivered micro-penetrators in the mass range ~4-10Kg, (preceded by ~13Kg Lunar penetrator MoonLITE development program), impacting at around 100-500m/s and carrying a scientific payload of around 2Kg. Additional mass is required to deliver the probes from `orbit' to surface which is dependent upon the particular planetary body in question. The mass per descent module therefore involves and additional element which, for a descent through an atmosphere could be quite modest, while for a flyby deployment, can be substantial. For Europa we estimate a descent module mass of ~13 Kg, while for Enceladus the value is ~40Kg for Enceladus since a deceleration of ~3.8 kms-1 is needed from a Titan orbit. The delivery system could consist of a rocket deceleration motor and attitude control system

  2. Miniature Ground Penetrating Radar, CRUX GPR

    Science.gov (United States)

    Kim, Soon Sam; Carnes, Steven R.; Haldemann, Albert F.; Ulmer, Christopher T.; Ng, Eddie; Arcone, Steven A.

    2006-01-01

    Under NASA instrument development programs (PIDDP 2000-2002, MIPD 2003-2005, ESR and T, 2005) we have been developing miniature ground penetrating radars (GPR) for use in mapping subsurface stratigraphy from planetary rovers for Mars and lunar applications. The Mars GPR is for deeper penetration (up to 50 m depth) into the Martian subsurface at moderate resolution (0.5 m) for a geological characterization. As a part of the CRUX (Construction and Resource Utilization Explorer) instrument suite, the CRUX GPR is optimized for a lunar prospecting application. It will have shallower penetration (5 m depth) with higher resolution (10 cm) for construction operations including ISRU (in-situ resource utilization).

  3. Development of coring, consolidating, subterrene penetrators

    International Nuclear Information System (INIS)

    Murphy, H.D.; Neudecker, J.W.; Cort, G.E.; Turner, W.C.; McFarland, R.D.; Griggs, J.E.

    1976-02-01

    Coring penetrators offer two advantages over full face-melting penetrators, i.e., formation of larger boreholes with no increase in power and the production of glass-lined, structurally undisturbed cores which can be recovered with conventional core-retrieval systems. These cores are of significant value in geological exploratory drilling programs. The initial design details and fabrication features of a 114-mm-diam coring penetrator are discussed; significant factors for design optimization are also presented. Results of laboratory testing are reported and compared with performance predictions, and an initial field trial is described

  4. Sphere impact and penetration into wet sand

    KAUST Repository

    Marston, J. O.

    2012-08-07

    We present experimental results for the penetration of a solid sphere when released onto wet sand. We show, by measuring the final penetration depth, that the cohesion induced by the water can result in either a deeper or shallower penetration for a given release height compared to dry granular material. Thus the presence of water can either lubricate or stiffen the granular material. By assuming the shear rate is proportional to the impact velocity and using the depth-averaged stopping force in calculating the shear stress, we derive effective viscosities for the wet granular materials.

  5. Sphere impact and penetration into wet sand

    KAUST Repository

    Marston, J. O.; Vakarelski, Ivan Uriev; Thoroddsen, Sigurdur T

    2012-01-01

    We present experimental results for the penetration of a solid sphere when released onto wet sand. We show, by measuring the final penetration depth, that the cohesion induced by the water can result in either a deeper or shallower penetration for a given release height compared to dry granular material. Thus the presence of water can either lubricate or stiffen the granular material. By assuming the shear rate is proportional to the impact velocity and using the depth-averaged stopping force in calculating the shear stress, we derive effective viscosities for the wet granular materials.

  6. Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

    Directory of Open Access Journals (Sweden)

    Susan Sommerlad

    2010-10-01

    speckling. Fine mapping was then performed on 45 of these 50 dogs and a further 48 dogs (n = 93. Sequencing candidate gene Sox10 in 6 hearing ASCD, 2 unilaterally deaf ASCD and 2 bilaterally deaf ASCD did not reveal any disease-associated mutations.Deafness in ASCD is an incompletely penetrant autosomal recessive inherited disease that maps to CFA10.

  7. Persistent enhancement of bacterial motility increases tumor penetration.

    Science.gov (United States)

    Thornlow, Dana N; Brackett, Emily L; Gigas, Jonathan M; Van Dessel, Nele; Forbes, Neil S

    2015-11-01

    Motile bacteria can overcome the transport limitations that hinder many cancer therapies. Active bacteria can penetrate through tissue to deliver treatment to resistant tumor regions. Bacterial therapy has had limited success, however, because this motility is heterogeneous, and within a population many individuals are non-motile. In human trials, heterogeneity led to poor dispersion and incomplete tumor colonization. To address these problems, a swarm-plate selection method was developed to increase swimming velocity. Video microscopy was used to measure the velocity distribution of selected bacteria and a microfluidic tumor-on-a-chip device was used to measure penetration through tumor cell masses. Selection on swarm plates increased average velocity fourfold, from 4.9 to 18.7 μm/s (P < 0.05) and decreased the number of non-motile individuals from 51% to 3% (P < 0.05). The selected phenotype was both robust and stable. Repeating the selection process consistently increased velocity and eliminated non-motile individuals. When selected strains were cryopreserved and subcultured for 30.1 doublings, the high-motility phenotype was preserved. In the microfluidic device, selected Salmonella penetrated deeper into cell masses than unselected controls. By 10 h after inoculation, control bacteria accumulated in the front 30% of cell masses, closest to the flow channel. In contrast, selected Salmonella accumulated in the back 30% of cell masses, farthest from the channel. Selection increased the average penetration distance from 150 to 400 μm (P < 0.05). This technique provides a simple and rapid method to generate high-motility Salmonella that has increased penetration and potential for greater tumor dispersion and clinical efficacy. © 2015 Wiley Periodicals, Inc.

  8. A Mass Loss Penetration Model to Investigate the Dynamic Response of a Projectile Penetrating Concrete considering Mass Abrasion

    Directory of Open Access Journals (Sweden)

    NianSong Zhang

    2015-01-01

    Full Text Available A study on the dynamic response of a projectile penetrating concrete is conducted. The evolutional process of projectile mass loss and the effect of mass loss on penetration resistance are investigated using theoretical methods. A projectile penetration model considering projectile mass loss is established in three stages, namely, cratering phase, mass loss penetration phase, and remainder rigid projectile penetration phase.

  9. Synthesis and processing of composites by reactive metal penetration

    Energy Technology Data Exchange (ETDEWEB)

    Loehman, R.E.; Ewsuk, K.G. [Sandia National Laboratories, Albuquerque, NM (United States); Tomsia, A.P. [Pask Research and Engineering, Berkeley, CA (United States)] [and others

    1995-05-01

    Ceramic-metal composites are being developed because their high stiffness-to weight ratios, good fracture toughness, and variable electrical and thermal properties give them advantages over more conventional materials. However, because ceramic-metal composite components presently are more expensive than monolithic materials, improvements in processing are required to reduce manufacturing costs. Reactive metal penetration is a promising new method for making ceramic- and metal-matrix composites that has the advantage of being inherently a net-shape process. This technique, once fully developed, will provide another capability for manufacturing the advanced ceramic composites that are needed for many light-weight structural and wear applications. The lower densities of these composites lead directly to energy savings in use. Near-net-shape fabrication of composite parts should lead to additional savings because costly and energy intensive grinding and machining operations are significantly reduced, and the waste generated from such finishing operations is minimized. The goals of this research program are: (1) to identify feasible compositional systems for making composites by reactive metal penetration; (2) to understand the mechanism(s) of composite formation by reactive metal penetration; and (3) to learn how to control and optimize reactive metal penetration for economical production of composites and composite coatings.

  10. Analytical review of 664 cases of penetrating buttock trauma

    Science.gov (United States)

    2011-01-01

    A comprehensive review of data has not yet been provided as penetrating injury to the buttock is not a common condition accounting for 2-3% of all penetrating injuries. The aim of the study is to provide the as yet lacking analytical review of the literature on penetrating trauma to the buttock, with appraisal of characteristics, features, outcomes, and patterns of major injuries. Based on these results we will provide an algorithm. Using a set of terms we searched the databases Pub Med, EMBASE, Cochran, and CINAHL for articles published in English between 1970 and 2010. We analysed cumulative data from prospective and retrospective studies, and case reports. The literature search revealed 36 relevant articles containing data on 664 patients. There was no grade A evidence found. The injury population mostly consists of young males (95.4%) with a high proportion missile injury (75.9%). Bleeding was found to be the key problem which mostly occurs from internal injury and results in shock in 10%. Overall mortality is 2.9% with significant adverse impact of visceral or vascular injury and shock (P colon, or rectum injuries leading in shot wounds, whilst vascular injury leads in stab wounds (P trauma. In conclusion, penetrating buttock trauma should be regarded as a life-threatening injury with impact beyond the pelvis until proven otherwise. PMID:21995834

  11. Abdominal penetrating trauma and organ damage and its prognosis

    Directory of Open Access Journals (Sweden)

    Babak Abri

    2016-12-01

    Full Text Available Introduction: Abdominal penetrating trauma is still a serious problem in the world, due to its high prevalence in young people that impose a heavy economic burden on our country, we decided to evaluate the epidemiologic and demographic status and patient’s prognosis. Methods: This is a descriptive cross-sectional study; all patients with abdominal penetrating trauma referred to the emergency department of Imam Reza Hospital between March 2012 and March 2014 were enrolled to study. Age, sex, injured organs, mechanism of trauma and the prognosis was documented in the checklist. Data was analyzed by SPSS 15.0, and descriptive analysis was run. Results: In this study, 137 patients were enrolled; 103 patients (75.20% did not have visceral damage. In visceral injury, the spleen was most commonly injured abdominal organs (8.03%. the damage to small intestine seen in 8 patients (5.84%, kidney and liver damage seen in 6 patients (4.38% and 5 patients, respectively, and finally diaphragm and colon injury was seen in 2 patients (1.46%. Conclusion: According to a recent study, abdominal penetrating trauma mostly occurred in men. The most common cause of abdominal penetrating trauma was related to knife damage; spleen was commonly damaged organ caused by knives and other sharp objects.

  12. A study of the penetration of projectiles into marine sediments

    International Nuclear Information System (INIS)

    Boisson, J.Y.

    1985-01-01

    The work described in this document consists of three main parts: - Application, after having reviewed calculation methods and known codes, of a dynamic plasticity model based on the upper bound-method (with dissipated energy calculations by plastic deformations). The soil model used for this calculation is the Cambridge Clay Model. - Carrying out a programme of tests with instrumented small scale penetrators in centrifuge on a consolidated clay-target. The trials are done under 50 g, with projectiles, fired with an airgun at high impact velocity (50 m/s). The penetrators' instrumentation consists of either measuring acceleration, or tip force with strain gauges. - The mounting of a small instrumented penetrator for shallow water depth experimentations, with an accelerometer, and a local cell for tip resistance. A rapid electronic data acquisition system has been developed for these experimentations. The preliminary tests are done in a large tank filled with clay. The geotechnical characteristics of the clay are perfectly controlled. The tests performed under these conditions have shown the following observations: the rapid electronic data acquisition system works perfectly and could be used as a basic component for a deep water instrumentated penetrator; the results, obtained in these particular test conditions, are in a perfect agreement with the predictions of the model developed in the frame of this contract

  13. Neutron imaging of water penetration into cracked steel reinforced concrete

    International Nuclear Information System (INIS)

    Zhang, P.; Wittmann, F.H.; Zhao, T.; Lehmann, E.H.

    2010-01-01

    Service life and durability of reinforced concrete structures have become a crucial issue because of the economical and ecological implications. Service life of reinforced concrete structures is often limited by penetration of water and chemical compounds dissolved in water into the porous cement-based material. By now it is well-known that cracks in reinforced concrete are preferential paths for ingress of aggressive substances. Neutron radiography was successfully applied to study the process of water penetration into cracked steel reinforced concrete. In addition, the effectiveness of integral water repellent concrete to prevent ingress of water and salt solutions was investigated. Results are described in detail in this contribution. It will be shown that neutron radiography is a powerful method to visualize the process of water penetration into cracked and uncracked cement-based materials. On the basis of the obtained experimental data, it is possible to quantify the time-dependent water distributions in concrete with high accuracy and spatial resolution. It is of particular interest that penetration of water and salt solutions into damaged interfaces between concrete and steel can be visualized by means of neutron radiography. Deteriorating processes in cracked reinforced concrete structures can be studied in a completely new way. This advanced technology will help and find adequate ways to improve durability and service life of reinforced concrete structures. This will mean at the same time an essential contribution to improved sustainability.

  14. Left frontal hub connectivity delays cognitive impairment in autosomal-dominant and sporadic Alzheimer’s disease

    Science.gov (United States)

    Franzmeier, Nicolai; Düzel, Emrah; Jessen, Frank; Buerger, Katharina; Levin, Johannes; Duering, Marco; Dichgans, Martin; Haass, Christian; Suárez-Calvet, Marc; Fagan, Anne M; Paumier, Katrina; Benzinger, Tammie; Masters, Colin L; Morris, John C; Perneczky, Robert; Janowitz, Daniel; Catak, Cihan; Wolfsgruber, Steffen; Wagner, Michael; Teipel, Stefan; Kilimann, Ingo; Ramirez, Alfredo; Rossor, Martin; Jucker, Mathias; Chhatwal, Jasmeer; Spottke, Annika; Boecker, Henning; Brosseron, Frederic; Falkai, Peter; Fliessbach, Klaus; Heneka, Michael T; Laske, Christoph; Nestor, Peter; Peters, Oliver; Fuentes, Manuel; Menne, Felix; Priller, Josef; Spruth, Eike J; Franke, Christiana; Schneider, Anja; Kofler, Barbara; Westerteicher, Christine; Speck, Oliver; Wiltfang, Jens; Bartels, Claudia; Araque Caballero, Miguel Ángel; Metzger, Coraline; Bittner, Daniel; Weiner, Michael; Lee, Jae-Hong; Salloway, Stephen; Danek, Adrian; Goate, Alison; Schofield, Peter R; Bateman, Randall J; Ewers, Michael

    2018-01-01

    Abstract Patients with Alzheimer’s disease vary in their ability to sustain cognitive abilities in the presence of brain pathology. A major open question is which brain mechanisms may support higher reserve capacity, i.e. relatively high cognitive performance at a given level of Alzheimer’s pathology. Higher functional MRI-assessed functional connectivity of a hub in the left frontal cortex is a core candidate brain mechanism underlying reserve as it is associated with education (i.e. a protective factor often associated with higher reserve) and attenuated cognitive impairment in prodromal Alzheimer’s disease. However, no study has yet assessed whether such hub connectivity of the left frontal cortex supports reserve throughout the evolution of pathological brain changes in Alzheimer’s disease, including the presymptomatic stage when cognitive decline is subtle. To address this research gap, we obtained cross-sectional resting state functional MRI in 74 participants with autosomal dominant Alzheimer’s disease, 55 controls from the Dominantly Inherited Alzheimer’s Network and 75 amyloid-positive elderly participants, as well as 41 amyloid-negative cognitively normal elderly subjects from the German Center of Neurodegenerative Diseases multicentre study on biomarkers in sporadic Alzheimer’s disease. For each participant, global left frontal cortex connectivity was computed as the average resting state functional connectivity between the left frontal cortex (seed) and each voxel in the grey matter. As a marker of disease stage, we applied estimated years from symptom onset in autosomal dominantly inherited Alzheimer’s disease and cerebrospinal fluid tau levels in sporadic Alzheimer’s disease cases. In both autosomal dominant and sporadic Alzheimer’s disease patients, higher levels of left frontal cortex connectivity were correlated with greater education. For autosomal dominant Alzheimer’s disease, a significant left frontal cortex connectivity

  15. Economic Operation of Power Systems with Significant Wind Power Penetration

    DEFF Research Database (Denmark)

    Farashbashi-Astaneh, Seyed-Mostafa

    This dissertation addresses economic operation of power systems with high penetration of wind power. Several studies are presented to address the economic operation of power systems with high penetration of variable wind power. The main concern in such power systems is high variability...... and unpredictability. Unlike conventional power plants, the output power of a wind farm is not controllable. This brings additional complexity to operation and planning of wind dominant power systems. The key solution in face of wind power uncertainty is to enhance power system flexibility. The enhanced flexibility......, cooperative wind-storage operation is studied. Lithium-Ion battery units are chosen as storage units. A novel formulation is proposed to investigate optimal operation of a storage unit considering power system balancing conditions and wind power imbalances. An optimization framework is presented to increase...

  16. Allele frequency distribution for 15 autosomal STR loci in Afridi Pathan population of Uttar Pradesh, India.

    Science.gov (United States)

    Noor, Sabahat; Ali, Shahnaz; Eaaswarkhanth, Muthukrishnan; Haque, Ikramul

    2009-11-01

    Allele frequencies of the 15 autosomal short tandem repeat (STR) loci D8S1179, D21S11, D7S820, CSF1PO D19S433, vWA, TPOX, D18S51, D3S1358, THO1, D13S317, D16S539, D2S1338, D5S818 and FGA were determined in Afridi Pathan population of Uttar Pradesh, India. All the 15 STR loci studied were found to be highly polymorphic with respect to observed heterozygosity values. Adherence to the expectations of the Hardy-Weinberg equilibrium (HWE) was confirmed for all the loci with an exception of TPOX and FGA. The allele 12 in CSF1PO was found to be most frequent. The power of discrimination was found to be high ranging from a minimum of 0.858 for the locus CSFIPO to maximum of 0.962 for the locus FGA, thereby facilitating the validation and efficiency of these STR markers in human identification. Population differentiation test between the studied and neighboring populations revealed significant differences at several loci suggesting the endogamous nature of the studied population. To the best of our knowledge, Afridi Pathan population has not been explored genetically for generating forensic data on STR markers. Therefore, STR allele frequency data of this unique population is a valuable contribution to the existing DNA database on Indian populations.

  17. Autosomal dominant polycystic kidney disease caused by somatic and germline mosaicism.

    Science.gov (United States)

    Tan, A Y; Blumenfeld, J; Michaeel, A; Donahue, S; Bobb, W; Parker, T; Levine, D; Rennert, H

    2015-04-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a heterogeneous genetic disorder caused by loss of function mutations of PKD1 or PKD2 genes. Although PKD1 is highly polymorphic and the new mutation rate is relatively high, the role of mosaicism is incompletely defined. Herein, we describe the molecular analysis of ADPKD in a 19-year-old female proband and her father. The proband had a PKD1 truncation mutation c.10745dupC (p.Val3584ArgfsX43), which was absent in paternal peripheral blood lymphocytes (PBL). However, very low quantities of this mutation were detected in the father's sperm DNA, but not in DNA from his buccal cells or urine sediment. Next generation sequencing (NGS) analysis determined the level of this mutation in the father's PBL, buccal cells and sperm to be ∼3%, 4.5% and 10%, respectively, consistent with somatic and germline mosaicism. The PKD1 mutation in ∼10% of her father's sperm indicates that it probably occurred early in embryogenesis. In ADPKD cases where a de novo mutation is suspected because of negative PKD gene testing of PBL, additional evaluation with more sensitive methods (e.g. NGS) of the proband PBL and paternal sperm can enhance detection of mosaicism and facilitate genetic counseling. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. The penetration of aerosols through fine orifices

    International Nuclear Information System (INIS)

    Marshall, I.A.; Latham, L.J.; Ball, M.H.E.; Mitchell, J.P.

    1991-07-01

    A novel experimental technique has been extended to study the migration of gas-borne glass microspheres in the size range from about 1 to 15 μm volume equivalent diameter through orifices with bores and thicknesses in the range from 2 to 100 μm and 12.7 to 509 μm respectively. The penetration of these particles was significant with all orifices greater than 10 μm bore at a constant driving pressure of 100 kPa. However, few particles penetrated the 5 μm bore orifice, while virtually no particles penetrated the 2 μm bore orifice. Particle size distributions determined after penetration through the orifices were very similar to that of the upstream aerosol except when significant attenuation occurred. (author)

  19. Temporary fire sealing of penetrations on TFTR

    International Nuclear Information System (INIS)

    Hondorp, H.L.

    1981-02-01

    The radiation shielding provided for TFTR for D-D and D-T operation will be penetrated by numerous electrical and mechanical services. Eventually, these penetrations will have to be sealed to provide the required fire resistance, tritium sealability, pressure integrity and radiation attenuation. For the initial hydrogen operation, however, fire sealing of the penetrations in the walls and floor is the primary concern. This report provides a discussion of the required and desirable properties of a temporary seal which can be used to seal these penetrations for the hydrogen operation and then subsequently be removed and replaced as required for the D-D and D-T operations. Several candidate designs are discussed and evaluated and recommendations are made for specific applications

  20. Identifying structural damage with ground penetrating radar

    CSIR Research Space (South Africa)

    Van Schoor, Abraham M

    2008-07-01

    Full Text Available Ground penetrating radar (GPR) and electrical resistance tomography (ERT) surveys were conducted in an urban environment in an attempt to identify the cause of severe structural damage to a historically significant residential property...

  1. Thyroid Emphysema Following Penetrating Neck Trauma

    Directory of Open Access Journals (Sweden)

    Demet Karadağ

    2011-03-01

    Full Text Available Although traumatic thyroid gland rupture or hemorrhage is usually seen in goitrous glands, injuries of the normal thyroid gland after neck trauma have rarely been described in the literature. We describe a 44-year-old man who presented with thyroid emphysema and subcutaneous emphysema (SCE that occurred after penetrating neck trauma. CT images showed complete resolution of thyroid emphysema and subcutaneous emphysema at follow-up examination. Neck injuries can be life threatening. After penetrating neck traumas, physicians should consider subtle esophageal or tracheal laceration. Thyroid emphysema can occur as the result of penetrating neck trauma. The mechanism of emphysema of the thyroid parenchyma can be explained by the thyroid gland’s presence in a single visceral compartment that encompasses the larynx, trachea and thyroid gland. We describe an unusual case of thyroid emphysema of a normal thyroid gland following a penetrating neck injury.

  2. Kali Linux wireless penetration testing beginner's guide

    CERN Document Server

    Ramachandran, Vivek

    2015-01-01

    If you are a security professional, pentester, or anyone interested in getting to grips with wireless penetration testing, this is the book for you. Some familiarity with Kali Linux and wireless concepts is beneficial.

  3. Penetration testing protecting networks and systems

    CERN Document Server

    Henry, Kevin M

    2012-01-01

    This book is a preparation guide for the CPTE examination, yet is also a general reference for experienced penetration testers, ethical hackers, auditors, security personnel and anyone else involved in the security of an organization's computer systems.

  4. The penetration of aerosols through fine capillaries

    International Nuclear Information System (INIS)

    Mitchell, J.P.; Edwards, R.T.; Ball, M.H.E.

    1989-10-01

    A novel experimental technique has been developed to study the penetration of aerosol particles ranging from about 1 to 15 μm aerodynamic diameter through capillaries varying from 20 to 80 μm bore and from 10 to 50 mm in length. When the driving pressure was 100 kPa, the penetration of the airborne particles was considerably smaller than expected from a simple comparison of particle diameter with the bore of the capillary. Particle size distributions determined after penetration through the capillaries were in almost all cases similar to the particle size distribution of the aerosol at the capillary entrance. This lack of size-selectivity can be explained in terms of the capillary behaving as a conventional suction-based sampler from a near still (calm) air environment. The resulting particle penetration data are important in assessing the potential for the leakage of aerosols through seals in containers used to transport radioactive materials. (author)

  5. In vitro penetration of bleaching agents into the pulp chamber

    DEFF Research Database (Denmark)

    Benetti, Ana Raquel; Valera, M C; Mancini, M N G

    2004-01-01

    To investigate pulp chamber penetration of bleaching agents in teeth following restorative procedures.......To investigate pulp chamber penetration of bleaching agents in teeth following restorative procedures....

  6. Ground Penetrating Radar Technologies in Ukraine

    Science.gov (United States)

    Pochanin, Gennadiy P.; Masalov, Sergey A.

    2014-05-01

    Transient electromagnetic fields are of great interest in Ukraine. The following topics are studied by research teams, with high-level achievements all over the world: (i) Ultra-Wide Band/Short-pulse radar techniques (IRE and LLC "Transient Technologies", for more information please visit http://applied.ire.kharkov.ua/radar%20systems_their%20components%20and%20relevant%20technologies_e.html and http://viy.ua); (ii) Ground Penetrating Radar (GPR) with stepped frequency sounding signals (IRE); (iii) Continuous-Wave (CW) radar with phase-shift keying signals (IRE); and (iv) Radio-wave interference investigation (Scientific and Technical Centre of The Subsurface Investigation, http://geophysics.ua). GPR applications are mainly in search works, for example GPR is often used to search for treasures. It is also used to identify leaks and diffusion of petroleum in soil, in storage areas, as well as for fault location of pipelines. Furthermore, GPR is used for the localization of underground utilities and for diagnostics of the technical state of hydro dams. Deeper GPR probing was performed to identify landslides in Crimea. Rescue radar with CW signal was designed in IRE to search for living people trapped under the rubble of collapsed buildings. The fourth version of this radar has been recently created, showing higher stability and noise immunity. Radio-wave interference investigation allows studying the soil down to tens of meters. It is possible to identify areas with increased conductivity (moisture) of the soil. LLC "Transient Technologies" is currently working with Shevchenko Kyiv University on a cooperation program in which the construction of a test site is one of the planned tasks. In the framework of this program, a GPR with a 300 MHz antenna was handed to the geological Faculty of the University. Employees of "Transient Technologies" held introductory lectures with a practical demonstration for students majoring in geophysics. The authors participated to GPR

  7. Adsorption of polymer chains at penetrable interfaces

    International Nuclear Information System (INIS)

    Gerasimchuk, I. V.; Sommer, J.-U.; Gerasimchuk, V. S.

    2011-01-01

    We investigate the problem of adsorption (localization) of polymer chains in the system of two penetrable interfaces within the mean-field approximation. The saturation of the polymer system in the limit case of zero bulk concentration is studied. We find the exact solution of this mean-field polymer adsorption problem that opens the possibility to treat various localization problems for polymer chains in such environments using appropriate boundary conditions. The exact solution is controlled by a single scaling variable that describes the coupling between the interfaces due to the polymer chains. We obtain a nonmonotonic behavior of the amount of adsorbed polymers as a function of the distance between the interfaces. This leads to a high-energy and a low-energy phase for the double layer with respect to the amount of polymers localized. At the saturation point, we find the total energy of the system and determine the force acting between the interfaces to be strictly attractive and to monotonically decay to zero when the interface distance increases.

  8. Penetration of tritium into the Tropical Pacific

    International Nuclear Information System (INIS)

    Fine, R.A.; Peterson, W.H.; Ostlund, H.G.

    1987-01-01

    The persistence of subsurface tritium maxima coincident with the Equatorial Currents is used to show that advection along isopycnals by the mean wind-driven circulation is the dominant process in the at most 14-year time scale for the penetration of high northern latitude water to the equator (above 26.2 sigma-theta). Ventilation of the equatorial Pacific thermocline from the north contrasts sharply with the equatorial Atlantic thermocline which is ventilated from the south. The most striking manifestation of the North Pacific circulation is evidenced by a tritium maximum and salinity minimum at the equator between 145 0 and 125 0 W located above 25.6 sigma-theta. It shows that regardless of time of sampling the easter/central equator has received the highest latitude water, probably as a consequence of recirculation by the Equatorial Currents. Between the same meridians there is a tritium maximum on and north of the equator at the surface, which is interpreted as an expression of upwelling. Its coincidence with the cool tongue (Wyrtki) provides direct evidence that the upwelling process plays a dominant role in its maintenance on a decadal time scale

  9. Genetic polymorphisms in 18 autosomal STR loci in the Tibetan population living in Tibet Chamdo, Southwest China.

    Science.gov (United States)

    Li, Zhenghui; Zhang, Jian; Zhang, Hantao; Lin, Ziqing; Ye, Jian

    2018-05-01

    Short tandem repeats (STRs) play a vitally important role in forensics. Population data is needed to improve the field. There is currently no large population data-based data set in Chamdo Tibetan. In our study, the allele frequencies and forensic statistical parameters of 18 autosomal STR loci (D5S818, D21S11, D7S820, CSF1PO, D2S1338, D3S1358, VWA, D8S1179, D16S539, PentaE, TPOX, TH01, D19S433, D18S51, FGA, D6S1043, D13S317, and D12S391) included in the DNATyper™19 kit were investigated in 2249 healthy, unrelated Tibetan subjects living in Tibet Chamdo, Southwest China. The combined power of discrimination and the combined probability of exclusion of all 18 loci were 0.9999999999999999999998174 and 0.99999994704, respectively. Furthermore, the genetic relationship between our Tibetan group and 33 previously published populations was also investigated. Phylogenetic analyses revealed that the Chamdo Tibetan population is more closely related genetically with the Lhasa Tibetan group. Our results suggest that these autosomal STR loci are highly polymorphic in the Tibetan population living in Tibet Chamdo and can be used as a powerful tool in forensics, linguistics, and population genetic analyses.

  10. Rapid penetration into granular media visualizing the fundamental physics of rapid earth penetration

    CERN Document Server

    Iskander, Magued

    2015-01-01

    Rapid Penetration into Granular Media: Visualizing the Fundamental Physics of Rapid Earth Penetration introduces readers to the variety of methods and techniques used to visualize, observe, and model the rapid penetration of natural and man-made projectiles into earth materials. It provides seasoned practitioners with a standard reference that showcases the topic's most recent developments in research and application. The text compiles the findings of new research developments on the subject, outlines the fundamental physics of rapid penetration into granular media, and assembles a com

  11. Distribution of skeletal muscle involvement in autosomal recessive distal muscular dystrophy

    International Nuclear Information System (INIS)

    Mizusawa, Hidehiro; Nakanishi, Takao; Kobayashi, Fumie.

    1987-01-01

    Distribution of skeletal muscle involvement in 5 cases with autosomal recessive distal muscular dystrophy was studied clinically and by computed tomography (CT). Manual muscle test showed muscle involvement with a predilection for flexors in the lower leg and adductors in the thigh. Flexion and extension of the thigh and the lower leg was impaired to similar degree. In progressed cases, neck flexors and trunk muscles were also affected mildly. CT disclosed more clearly the preferential involvement of flexors in the lower leg, and involvement of both hamstrings · adductors group and extensors group of the thigh to similar degree. However, m. popliteus was curiously well preserved. In addition, there was a stage showing high density and hypertrophy of m. sartorius, m. gracilis, m. adductor, m. biceps femoris, m. semimenbranosus, m. semitendinosus or m. rectus femoris, which in thought to be compensatory hypertrophy. M. gluteus minimus in the pelvic girdle and m. dorsi proprii in the trunk were also liable to be affected. The CT findings are regarded as characteristic features noted clearly before muscle weakness and atrophy become apparent clinically. CT is very useful for distinguishing distal muscular dystrophy from rimmed vacuolar distal myopathy in which m. quadriceps femoris and flexors of the lower leg are usually well preserved without compensatory hypertrophy on CT. (author)

  12. Genetic distribution of 15 autosomal STR markers in the Punjabi population of Pakistan.

    Science.gov (United States)

    Shan, Muhammad Adnan; Hussain, Manzoor; Shafique, Muhammad; Shahzad, Muhammad; Perveen, Rukhsana; Idrees, Muhammad

    2016-11-01

    Genetic diversity of 15 autosomal short tandem repeat (STR) loci was evaluated in 713 unrelated individual samples of a Punjabi population of Pakistan. These loci were scrutinized to establish allelic frequencies and statistical parameters of forensic and paternity interests. A total of 165 alleles were observed with the corresponding allele frequencies ranging from 0.001 to 0.446. D2S1338 was found as the most informative locus while TPOX (0.611) was the least discriminating locus. The combined power of discrimination (CPD), the combined probability of exclusion (CPE), and cumulative probability of matching (CPM) were found equaled to 0.999999999999999998606227424808, 0.999995777557989, and 1.37543 × 10-18, respectively. All the loci followed the Hardy-Weinberg equilibrium after the Bonferroni correction (p < 0.0033) except one locus D3S1358. The study revealed that these STR loci are highly polymorphic, suitable for forensic and parentage analyses. In comparison to different populations (Asians and non-Asians), significant differences were recorded for these loci.

  13. Genetic Counselors' Experiences Regarding Communication of Reproductive Risks with Autosomal Recessive Conditions found on Cancer Panels.

    Science.gov (United States)

    Mets, Sarah; Tryon, Rebecca; Veach, Patricia McCarthy; Zierhut, Heather A

    2016-04-01

    The development of hereditary cancer genetic testing panels has altered genetic counseling practice. Mutations within certain genes on cancer panels pose not only a cancer risk, but also a reproductive risk for autosomal recessive conditions such as Fanconi anemia, constitutional mismatch repair deficiency syndrome, and ataxia telangiectasia. This study aimed to determine if genetic counselors discuss reproductive risks for autosomal recessive conditions associated with genes included on cancer panels, and if so, under what circumstances these risks are discussed. An on-line survey was emailed through the NSGC list-serv. The survey assessed 189 cancer genetic counselors' experiences discussing reproductive risks with patients at risk to carry a mutation or variant of uncertain significance (VUS) in a gene associated with both an autosomal dominant cancer risk and an autosomal recessive syndrome. Over half (n = 82, 55 %) reported having discussed reproductive risks; the remainder (n = 66, 45 %) had not. Genetic counselors who reported discussing reproductive risks primarily did so when patients had a positive result and were of reproductive age. Reasons for not discussing these risks included when a patient had completed childbearing or when a VUS was identified. Most counselors discussed reproductive risk after obtaining results and not during the informed consent process. There is inconsistency as to if and when the discussion of reproductive risks is taking place. The wide variation in responses suggests a need to develop professional guidelines for when and how discussions of reproductive risk for autosomal recessive conditions identified through cancer panels should occur with patients.

  14. Enamelin/ameloblastin gene polymorphisms in autosomal amelogenesis imperfecta among Syrian families.

    Science.gov (United States)

    Dashash, Mayssoon; Bazrafshani, Mohamed Riza; Poulton, Kay; Jaber, Saaed; Naeem, Emad; Blinkhorn, Anthony Stevenson

    2011-02-01

      This study was undertaken to investigate whether a single G deletion within a series of seven G residues (codon 196) at the exon 9-intron 9 boundary of the enamelin gene ENAM and a tri-nucleotide deletion at codon 180 in exon 7 (GGA vs deletion) of ameloblastin gene AMBN could have a role in autosomal amelogenesis imperfecta among affected Syrian families.   A new technique - size-dependent, deletion screening - was developed to detect nucleotide deletion in ENAM and AMBN genes. Twelve Syrian families with autosomal-dominant or -recessive amelogenesis imperfecta were included.   A homozygous/heterozygous mutation in the ENAM gene (152/152, 152/153) was identified in affected members of three families with autosomal-dominant amelogenesis imperfecta and one family with autosomal-recessive amelogenesis imperfecta. A heterozygous mutation (222/225) in the AMBN gene was identified. However, no disease causing mutations was found. The present findings provide useful information for the implication of ENAM gene polymorphism in autosomal-dominant/-recessive amelogenesis imperfecta.   Further investigations are required to identify other genes responsible for the various clinical phenotypes. © 2010 Blackwell Publishing Asia Pty Ltd.

  15. Elastic behavior of MFI-type zeolites: Compressibility of H-ZSM-5 in penetrating and non-penetrating media

    International Nuclear Information System (INIS)

    Quartieri, Simona; Montagna, Gabriele; Arletti, Rossella; Vezzalini, Giovanna

    2011-01-01

    The elastic behavior of H-ZSM-5 was investigated by in-situ synchrotron X-ray powder diffraction, using both silicone oil (s.o.) and (16:3:1) methanol:ethanol:water (m.e.w.) as 'non-penetrating' and 'penetrating' pressure transmitting media, respectively. From P amb to 6.2 GPa the volume reduction observed in s.o. is 16.6%. This testifies that H-ZSM-5 is one of the most flexible microporous materials up to now compressed in s.o. Volume reduction observed in m.e.w. up to 7.6 GPa is 14.6%. A strong increase in the total electron number of the extraframework system, due to the penetration of water/alcohol molecules in the pores, is observed in m.e.w. This effect is the largest up to now observed in zeolites undergoing this phenomenon without cell volume expansion. The higher compressibility in s.o. than in m.e.w. can be ascribed to the penetration of the extra-water/alcohol molecules, which stiffen the structure and contrast the channel deformations. - Graphical abstract: High-pressure behavior of H-ZSM-5 compressed in (16:3:1) methanol:ethanol:water: (a) projection of the structure along the [0 1 0] direction at P amb , 2 GPa and after pressure release to original ambient conditions (P amb (rev)), and (b) P-dependence of the extraframework content expressed as total number of electrons (gray square represents the number of the extraframework electrons at P amb after decompression). Highlights: → X-ray powder diffraction study of H-ZSM-5 compressibility. → H-ZSM-5 is one of the softest porous material compressed in silicon oil. → Penetration of additional water/alcohol molecules upon compression in m.e.w. → Extra molecules contribute to stiffen the structure and to contrast HP effects.

  16. Directional migration in the Hindu castes: inferences from mitochondrial, autosomal and Y-chromosomal data.

    Science.gov (United States)

    Wooding, Stephen; Ostler, Christopher; Prasad, B V Ravi; Watkins, W Scott; Sung, Sandy; Bamshad, Mike; Jorde, Lynn B

    2004-08-01

    Genetic, ethnographic, and historical evidence suggests that the Hindu castes have been highly endogamous for several thousand years and that, when movement between castes does occur, it typically consists of females joining castes of higher social status. However, little is known about migration rates in these populations or the extent to which migration occurs between caste groups of low, middle, and high social status. To investigate these aspects of migration, we analyzed the largest collection of genetic markers collected to date in Hindu caste populations. These data included 45 newly typed autosomal short tandem repeat polymorphisms (STRPs), 411 bp of mitochondrial DNA sequence, and 43 Y-chromosomal single-nucleotide polymorphisms that were assayed in more than 200 individuals of known caste status sampled in Andrah Pradesh, in South India. Application of recently developed likelihood-based analyses to this dataset enabled us to obtain genetically derived estimates of intercaste migration rates. STRPs indicated migration rates of 1-2% per generation between high-, middle-, and low-status caste groups. We also found support for the hypothesis that rates of gene flow differ between maternally and paternally inherited genes. Migration rates were substantially higher in maternally than in paternally inherited markers. In addition, while prevailing patterns of migration involved movement between castes of similar rank, paternally inherited markers in the low-status castes were most likely to move into high-status castes. Our findings support earlier evidence that the caste system has been a significant, long-term source of population structuring in South Indian Hindu populations, and that patterns of migration differ between males and females. Copyright 2004 Springer-Verlag

  17. Deformation analysis of shallow penetration in clay

    Science.gov (United States)

    Sagaseta, C.; Whittle, A. J.; Santagata, M.

    1997-10-01

    A new method of analysis is described for estimating the deformations and strains caused by shallow undrained penetration of piles and caissons in clay. The formulation combines previous analyses for steady, deep penetration, with methods used to compute soil deformations due to near-surface ground loss, and is referred to as the Shallow Strain Path Method (SSPM). Complete analytical solutions for the velocity and strain rates are given for a planar wall, an axisymmetric, closed-ended pile and unplugged, open-ended pile geometries. In these examples, the analyses consider a single source penetrating through the soil at a constant rate, generating a family of penetrometers with rounded tips, referred to as simple wall, pile and tube geometries. Soil deformations and strains are obtained by integrating the velocity and strain rates along the particle paths.The transition from shallow to deep penetration is analysed in detail. Shallow penetration causes heave at the ground surface, while settlements occur only in a thin veneer of material adjacent to the shaft and in a bulb-shaped region around the tip. The size of this region increases with the embedment depth. Deformations inside an open-ended pile/caisson are affected significantly by details of the simple tube wall geometry.

  18. Clinical and Radiological Findings of Autosomal Dominant Osteopetrosis Type II: A Case Report

    Directory of Open Access Journals (Sweden)

    Priyanka Kant

    2013-01-01

    Full Text Available Osteopetrosis is a rare inherited genetic disease characterized by sclerosis of the skeleton caused by the absence or malfunction of osteoclasts. Three distinct forms of the disease have been recognized, autosomal dominant osteopetrosis being the most common. Autosomal dominant osteopetrosis exhibits a heterogeneous trait with milder symptoms, often at later childhood or adulthood. The aim of this case report is to present the clinical and radiographic features of a 35-year-old female patient with autosomal dominant osteopetrosis type II who exhibited features of chronic generalised periodontitis, and the radiographs revealed generalised osteosclerosis and hallmark radiographic features of ADO type II, that is, “bone-within-bone appearance” and “Erlenmeyer-flask deformity.”

  19. Market penetration of biodiesel and ethanol

    Science.gov (United States)

    Szulczyk, Kenneth Ray

    This dissertation examines the influence that economic and technological factors have on the penetration of biodiesel and ethanol into the transportation fuels market. This dissertation focuses on four aspects. The first involves the influence of fossil fuel prices, because biofuels are substitutes and have to compete in price. The second involves biofuel manufacturing technology, principally the feedstock-to-biofuel conversion rates, and the biofuel manufacturing costs. The third involves prices for greenhouse gas offsets. The fourth involves the agricultural commodity markets for feedstocks, and biofuel byproducts. This dissertation uses the Forest and Agricultural Sector Optimization Model-Greenhouse Gas (FASOM-GHG) to quantitatively examine these issues and calculates equilibrium prices and quantities, given market interactions, fossil fuel prices, carbon dioxide equivalent prices, government biofuel subsidies, technological improvement, and crop yield gains. The results indicate that for the ranges studied, gasoline prices have a major impact on aggregate ethanol production but only at low prices. At higher prices, one runs into a capacity constraint that limits expansion on the capacity of ethanol production. Aggregate biodiesel production is highly responsive to gasoline prices and increases over time. (Diesel fuel price is proportional to the gasoline price). Carbon dioxide equivalent prices expand the biodiesel industry, but have no impact on ethanol aggregate production when gasoline prices are high again because of refinery capacity expansion. Improvement of crop yields shows a similar pattern, expanding ethanol production when the gasoline price is low and expanding biodiesel. Technological improvement, where biorefinery production costs decrease over time, had minimal impact on aggregate ethanol and biodiesel production. Finally, U.S. government subsidies have a large expansionary impact on aggregate biodiesel production. Finally, U.S. government

  20. [Filtering facepieces: effect of oily aerosol load on penetration through the filtering material].

    Science.gov (United States)

    Plebani, Carmela; Listrani, S; Di Luigi, M

    2010-01-01

    Electrostatic filters are widely used in applications requiring high filtration efficiency and low pressure drop. However various studies showed that the penetration through electrostatic filters increases during exposure to an aerosol flow. This study investigates the effects of prolonged exposure to an oily aerosol on the penetration through filtering facepieces available on the market. Some samples of FFP1, FFP2 and FFP3 filtering facepieces were exposed for 8 hours consecutively to a paraffin oil polydisperse aerosol. At the end of the exposure about 830 mg of paraffin oil were deposited in the facepiece. All the examined facepieces showed penetration values that increased with paraffin oil load while pressure drop values were substantially the same before and after exposure. The measured maximum penetration values did not exceed the maximum penetration values allowed by the European technical standards, except in one case. According to the literature, 830 mg of oil load in a facepiece is not feasible in workplaces over an eight- hour shift. However, the trend of the penetration versus exposure mass suggests that if the load increases, the penetration may exceed the maximum allowed values. For comparison a mechanical filter was also studied. This showed an initial pressure drop higher than FFP2 filtering facepieces characterized by comparable penetration values. During exposure the pressure drop virtually doubled while penetration did not change. The increase in penetration with no increase in pressure drop in the analyzed facepieces indicates that it is necessary to comply with the information supplied by the manufacturer that restricts their use to a single shift.