The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease

Neuronal intermediate filament inclusion disease (NIFID), a rare form of frontotemporal lobar degeneration (FTLD), is characterized neuropathologically by focal atrophy of the frontal and temporal lobes, neuronal loss, gliosis, and neuronal cytoplasmic inclusions (NCI) containing epitopes of ubiquitin and neuronal intermediate filament proteins. Recently, the 'fused in sarcoma' (FUS) protein (encoded by the FUS gene) has been shown to be a component of the inclusions of familial amyotrophic lateral sclerosis with FUS mutation, NIFID, basophilic inclusion body disease, and atypical FTLD with ubiquitin-immunoreactive inclusions (aFTLD-U). To further characterize FUS proteinopathy in NIFID, and to determine whether the pathology revealed by FUS immunohistochemistry (IHC) is more extensive than a-internexin, we have undertaken a quantitative assessment of ten clinically and neuropathologically well-characterized cases using FUS IHC. The densities of NCI were greatest in the dentate gyrus (DG) and in sectors CA1/2 of the hippocampus. Anti-FUS antibodies also labeled glial inclusions (GI), neuronal intranuclear inclusions (NII), and dystrophic neurites (DN). Vacuolation was extensive across upper and lower cortical layers. Significantly greater densities of abnormally enlarged neurons and glial cell nuclei were present in the lower compared with the upper cortical laminae. FUS IHC revealed significantly greater numbers of NCI in all brain regions especially the DG. Our data suggest: (1) significant densities of FUS-immunoreactive NCI in NIFID especially in the DG and CA1/2; (2) infrequent FUS-immunoreactive GI, NII, and DN; (3) widely distributed vacuolation across the cortex, and (4) significantly more NCI revealed by FUS than a-internexin IHC.

Direct current ripple compensation for multi-phase fault-tolerant machines

A second-harmonic direct current (DC) ripple compensation technique is presented for a multi-phase, fault-tolerant, permanent magnet machine. The analysis has been undertaken in a general manner for any pair of phases in operation with the remaining phases inactive. The compensation technique determines the required alternating currents in the machine to eliminate the second-harmonic DC-link current, while at the same time minimising the total rms current in the windings. An additional benefit of the compensation technique is a reduction in the magnitude of the electromagnetic torque ripple. Practical results are included from a 70 kW, five-phase generator system to validate the analysis and illustrate the performance of the compensation technique.

A tree-based decision model to support prediction of the severity of asthma exacerbations in children

This paper describes the development of a tree-based decision model to predict the severity of pediatric asthma exacerbations in the emergency department (ED) at 2 h following triage. The model was constructed from retrospective patient data abstracted from the ED charts. The original data was preprocessed to eliminate questionable patient records and to normalize values of age-dependent clinical attributes. The model uses attributes routinely collected in the ED and provides predictions even for incomplete observations. Its performance was verified on independent validating data (split-sample validation) where it demonstrated AUC (area under ROC curve) of 0.83, sensitivity of 84%, specificity of 71% and the Brier score of 0.18. The model is intended to supplement an asthma clinical practice guideline, however, it can be also used as a stand-alone decision tool.

A series-dynamic-resistor-based converter protection scheme for doubly-fed induction generator during various fault conditions

This paper proposes a new converter protection method, primarily based on a series dynamic resistor (SDR) that avoids the doubly-fed induction generator (DFIG) control being disabled by crowbar protection during fault conditions. A combined converter protection scheme based on the proposed SDR and conventional crowbar is analyzed and discussed. The main protection advantages are due to the series topology when compared with crowbar and dc-chopper protection. Various fault overcurrent conditions (both symmetrical and asymmetrical) are analyzed and used to design the protection in detail, including the switching strategy and coordination with crowbar, and resistance value calculations. PSCAD/EMTDC simulation results show that the proposed method is advantageous for fault overcurrent protection, especially for asymmetrical faults, in which the traditional crowbar protection may malfunction.

Multiterminal DC wind farm collection grid internal fault analysis and protection design

The multiterminal dc wind farm is a promising topology with a voltage-source inverter (VSI) connection at the onshore grid. Voltage-source converters (VSCs) are robust to ac-side fault conditions. However, they are vulnerable to dc faults on the dc side of the converter. This paper analyzes dc faults, their transients, and the resulting protection issues. Overcurrent faults are analyzed in detail and provide an insight into protection system design. The radial wind farm topology with star or string connection is considered. The outcomes may be applicable for VSCs in the multi-VSC dc wind farm collection grid and VSC-based high-voltage direct current (HVDC) offshore transmission systems.

Short-circuit and ground fault analyses and location in VSC-based DC network cables

The application of high-power voltage-source converters (VSCs) to multiterminal dc networks is attracting research interest. The development of VSC-based dc networks is constrained by the lack of operational experience, the immaturity of appropriate protective devices, and the lack of appropriate fault analysis techniques. VSCs are vulnerable to dc-cable short-circuit and ground faults due to the high discharge current from the dc-link capacitance. However, faults occurring along the interconnecting dc cables are most likely to threaten system operation. In this paper, cable faults in VSC-based dc networks are analyzed in detail with the identification and definition of the most serious stages of the fault that need to be avoided. A fault location method is proposed because this is a prerequisite for an effective design of a fault protection scheme. It is demonstrated that it is relatively easy to evaluate the distance to a short-circuit fault using voltage reference comparison. For the more difficult challenge of locating ground faults, a method of estimating both the ground resistance and the distance to the fault is proposed by analyzing the initial stage of the fault transient. Analysis of the proposed method is provided and is based on simulation results, with a range of fault resistances, distances, and operational conditions considered.

Nonlinear speech analysis algorithms mapped to a standard metric achieve clinically useful quantification of average Parkinson's disease symptom severity

The standard reference clinical score quantifying average Parkinson's disease (PD) symptom severity is the Unified Parkinson's Disease Rating Scale (UPDRS). At present, UPDRS is determined by the subjective clinical evaluation of the patient's ability to adequately cope with a range of tasks. In this study, we extend recent findings that UPDRS can be objectively assessed to clinically useful accuracy using simple, self-administered speech tests, without requiring the patient's physical presence in the clinic. We apply a wide range of known speech signal processing algorithms to a large database (approx. 6000 recordings from 42 PD patients, recruited to a six-month, multi-centre trial) and propose a number of novel, nonlinear signal processing algorithms which reveal pathological characteristics in PD more accurately than existing approaches. Robust feature selection algorithms select the optimal subset of these algorithms, which is fed into non-parametric regression and classification algorithms, mapping the signal processing algorithm outputs to UPDRS. We demonstrate rapid, accurate replication of the UPDRS assessment with clinically useful accuracy (about 2 UPDRS points difference from the clinicians' estimates, p < 0.001). This study supports the viability of frequent, remote, cost-effective, objective, accurate UPDRS telemonitoring based on self-administered speech tests. This technology could facilitate large-scale clinical trials into novel PD treatments.

Low severity Fischer-Tropsch synthesis for the production of synthetic hydrocarbon fuels

Currently, the main source for the production of liquid transportation fuels is petroleum, the continued use of which faces many challenges including depleting oil reserves, significant oil price rises, and environmental concerns over global warming which is widely believed to be due to fossil fuel derived CO2 emissions and other greenhouse gases. In this respect, lignocellulosic or plant biomass is a particularly interesting resource as it is the only renewable source of organic carbon that can be converted into liquid transportation fuels. The gasification of biomass produces syngas which can then be converted into synthetic liquid hydrocarbon fuels by means of the Fischer-Tropsch (FT) synthesis. This process has been widely considered as an attractive option for producing clean liquid hydrocarbon fuels from biomass that have been identified as promising alternatives to conventional fossil fuels like diesel and kerosene. The resulting product composition in FT synthesis is influenced by the type of catalyst and the reaction conditions that are used in the process. One of the issues facing this conversion process is the development of a technology that can be scaled down to match the scattered nature of biomass resources, including lower operating pressures, without compromising liquid composition. The primary aims of this work were to experimentally explore FT synthesis at low pressures for the purpose of process down-scaling and cost reduction, and to investigate the potential for obtaining an intermediate FT synthetic crude liquid product that can be integrated into existing refineries under the range of process conditions employed. Two different fixed-bed micro-reactors were used for FT synthesis; a 2cm3 reactor at the University of Rio de Janeiro (UFRJ) and a 20cm3 reactor at Aston University. The experimental work firstly involved the selection of a suitable catalyst from three that were available. Secondly, a parameter study was carried out on the 20cm3 reactor using the selected catalyst to investigate the influence of reactor temperature, reactor pressure, space velocity, the H2/CO molar ratio in the feed syngas and catalyst loading on the reaction performance measured as CO conversion, catalyst stability, product distribution, product yields and liquid hydrocarbon product composition. From this parameter study a set of preferred operating conditions was identified for low pressure FT synthesis. The three catalysts were characterized using BET, XRD, TPR and SEM. The catalyst selected was an unpromoted Co/Al2O3 catalyst. FT synthesis runs on the 20cm3 reactor at Aston were conducted for 48 hours. Permanent gases and light hydrocarbons (C1-C5) were analysed in an online GC-TCD/FID at hourly intervals. The liquid hydrocarbons collected were analyzed offline using GC-MS for determination of fuel composition. The parameter study showed that CO conversion and liquid hydrocarbon yields increase with increasing reactor pressure up to around 8 bar, above which the effect of pressure is small. The parameters that had the most significant influence on CO conversion, product selectivity and liquid hydrocarbon yields were reactor temperature and catalyst loading. The preferred reaction conditions identified for this research were: T = 230ºC, P = 10 bar, H2/CO = 2.0, WHSV = 2.2 h-1, and catalyst loading = 2.0g. Operation in the low range of pressures studied resulted in low CO conversions and liquid hydrocarbon yields, indicating that low pressure BTL-FT operation may not be industrially viable as the trade off in lower CO conversions and once-through liquid hydrocarbon product yields has to be carefully weighed against the potential cost savings resulting from process operation at lower pressures.

"It's not my fault - but only I can change it":counterfactual and prefactual thoughts of managers

In testing for the self-serving bias in performance evaluation, the authors propose that comparing managers' counterfactual and prefactual thoughts about subordinates' performance is more conclusive than the attributional approach and also offers practical advantages. In a study with 120 managers, a 4-way interaction of subordinate performance, temporal perspective, direction, and reference confirmed the predicted pattern. Managers' thoughts about how a weak performance could have been enhanced had external references, but thoughts about how such a performance could be enhanced in the future focused on the leader. This asymmetry was only observed for weak performance. Results are discussed with regard to biases in leaders' performance evaluations and to how counter- and prefactual thoughts could be used for leadership research and practice.

Cardiac dysfunction and cell damage across clinical stages of severity in growth-restricted fetuses

Objective - The purpose of this study was to assess cardiac function and cell damage in intrauterine growth-restricted (IUGR) fetuses across clinical Doppler stages of deterioration. Study Design - One hundred twenty appropriate-for-gestational-age and 81 IUGR fetuses were classified in stages 1/2/3 according umbilical artery present/absent/reversed end-diastolic blood flow, respectively. Cardiac function was assessed by modified-myocardial performance index, early-to-late diastolic filling ratios, cardiac output, and cord blood B-type natriuretic peptide; myocardial cell damage was assessed by heart fatty acid–binding protein, troponin-I, and high-sensitivity C-reactive protein. Results - Modified-myocardial performance index, blood B-type natriuretic peptide, and early-to-late diastolic filling ratios were increased in a stage-dependent manner in IUGR fetuses, compared with appropriate-for-gestational-age fetuses. Heart fatty acid–binding protein levels were higher in IUGR fetuses at stage 3, compared with control fetuses. Cardiac output, troponin-I, and high-sensitivity C-reactive protein did not increase in IUGR fetuses at any stage. Conclusion - IUGR fetuses showed signs of cardiac dysfunction from early stages. Cardiac dysfunction deteriorates further with the progression of fetal compromise, together with the appearance of biochemical signs of cell damage.

Single-phase active rectifier selection for fault tolerant machine

A fault tolerant, 5-phase PM generator has been developed for use on the low pressure (LP) shaft of an aircraft gas turbine engine. The machine operates at variable speed and therefore has a variable voltage, variable frequency electrical output (VVVF). The generator is to be used to provide a 350V DC bus for distribution throughout the aircraft, and a study has been carried out that identifies the most suitable AC-DC converter topology for this machine in terms of losses, electrical component ratings, filtering requirements and circuit complexity.

Visual field severity indices demonstrate dose-dependent visual loss from Vigabatrin therapy

Purpose: The aims of this study were to develop an algorithm to accurately quantify Vigabatrin (VGB)-induced central visual field loss and to investigate the relationship between visual field loss and maximum daily dose, cumulative dose and duration of dose. Methods: The sample comprised 31 patients (mean age 37.9 years; SD 14.4 years) diagnosed with epilepsy and exposed to VGB. Each participant underwent standard automated static visual field examination of the central visual field. Central visual field loss was determined using continuous scales quantifying severity in terms of area and depth of defect and additionally by symmetry of defect between the two eyes. A simultaneous multiple regression model was used to explore the relationship between these visual field parameters and the drug predictor variables. Results: The regression model indicated that maximum VGB dose was the only factor to be significantly correlated with individual eye severity (right eye: p = 0.020; left eye: p = 0.012) and symmetry of visual field defect (p = 0.024). Conclusions: Maximum daily dose was the single most reliable indicator of those patients likely to exhibit visual field defects due to VGB. These findings suggest that high maximum dose is more likely to result in visual field defects than high cumulative doses or those of long duration.

The Matrix Method of Determining the Fault Tolerance Degree of a Computer Network Topology

This work presents a theoretical-graph method of determining the fault tolerance degree of the computer network interconnections and nodes. Experimental results received from simulations of this method over a distributed computing network environment are also presented.

Protection issue discussion of DC network development:circuit breaker or fault-tolerant converter

This paper provides a discussion on future direct current (DC) network development in terms of system protection under DC-side fault scenarios. The argument between appropriate DC circuit breaker and new DC fault-tolerant converters is discussed after a review on DC technology development and bottleneck issues that require proper solutions. The overcurrent/cost curve of power-electronic DC circuit breakers (CB) superimposed to voltage-source converter (VSC) systems is derived and compared with other possible fault-tolerant power conversion options. This in-advance planning of protection capability is essential for the future development of DC networks.

Permanent magnet synchronous generator based wind power generation system fault protection schemes

The PMSG-based wind power generation system protection is presented in this paper. For large-scale systems, a voltagesource converter rectifier is included. Protection circuits for this topology are studied with simulation results for cable permanent fault conditions. These electrical protection methods are all in terms of dumping redundant energy resulting from disrupted path of power delivery. Pitch control of large-scale wind turbines are considered for effectively reducing rotor shaft overspeed. Detailed analysis and calculation of damping power and resistances are presented. Simulation results including fault overcurrent, DC-link overvoltage and wind turbine overspeed are shown to illustrate the system responses under different protection schemes to compare their application and effectiveness.