bluuebunny/crossref_metadata_2025_split huggingface dataset:
The mild phenotype in severe hemophilia A with Arg1781His mutation is associated with enhanced binding affinity of factor VIII for factor X
Authors: Yada Koji , Wakabayashi Hironao , Fay Philip J. , Shima Midori , Nogami Keiji 
Published: March 2013

SummaryThe clinical severity in some patients with haemophilia A appears to be unrelated to the levels of factor (F)VIII activity (FVIII:C), but mechanisms are poorly understood. We have investigated a patient with a FVIII gene mutation at Arg1781 to His (R1781H) presenting with a mild phenotype despite FVIII:C of 0.9 IU/dl. Rotational thromboelastometry using the patient’s whole blood demonstrated that the clot time and clot firmness were comparable to those usually observed at FVIII:C 5–10 IU/dl. Thrombin and FXa assays using plasma samples also showed that the peak levels of thrombin formation and the initial rate of FXa generation were comparable to those observed at FVIII:C 5–10 IU/dl. The results suggested a significantly greater haemostatic potential in this individual than in those with severe phenotype. The addition of incremental amounts of FX to control plasma with FVIII:C 0.9 IU/dl in clot waveform analyses suggested that the enhanced functional tenase assembly might have been related to changes in association between FVIII and FX. To further investigate this mechanism, we prepared a stably expressed, recombinant, B-domainless FVIII R1781H mutant. Thrombin generation assays using mixtures of control plasma and FVIII revealed that the coagulation function observed with the R1781H mutant (0.9 IU/dl) was comparable to that seen with wild-type FVIII:C at ∼5 IU/dl. In addition, the R1781H mutant demonstrated an ∼1.9-fold decrease in K m for FX compared to wild type. These results indicated that relatively enhanced binding affinity of FVIII R1781H for FX appeared to moderate the severity of the haemophilia A phenotype.Note: An account of this work was presented, in part, at the 23rd Congress of the International Society of Thrombosis and Haemostasis, July 27, 2011, Kyoto, Japan.
TAFI deficiency promotes liver damage in murine models of liver failure through defective down-regulation of hepatic inflammation
Authors: Hugenholtz Greg C. G. , Meijers Joost C. M. , Adelmeijer Jelle , Porte Robert J. , Lisman Ton 
Published: March 2013

SummaryEmerging evidence indicates that various haemostatic components can regulate the progression of liver disease. Thrombin-activatable fibrinolysis inhibitor (TAFI) possesses anti-inflammatory properties besides its anti-fibrinolytic function. Here, we investigated the contribution of TAFI to the progression of disease in murine models of chronic and acute liver failure. Chronic carbon tetrachloride (CCL4) administration induced liver damage and fibrosis both in TAFI knockout (TAFI−/−) mice and wild-type controls. Smooth muscle actin-α (α-SMA) content of liver tissue was significantly increased after 1 and 3 weeks, and pro-collagen α1 expression was significantly increased after 3 and 6 weeks in TAFI−/− mice. TAFI−/− mice showed significantly elevated levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) after 3 weeks of CCL4. Neutrophil influx was significantly increased in TAFI−/− mice after 6 weeks of CCL4. No difference in hepatic fibrin deposition between TAFI−/− and wild-types was observed. After acetaminophen intoxication, necrosis was significantly increased in TAFI−/− mice at 24 hours (h) after injection. AST and ALT levels were decreased at 2 and 6 h after acetaminophen injection in TAFI−/− mice, but were significantly higher in the TAFI−/− mice at 24 h. Similarly, hepatic fibrin deposition was decreased at 6 h in TAFI−/− mice, but was comparable to wild-types at 24 h after injection. In conclusion, TAFI deficiency results in accelerated fibrogenesis and increased liver damage in murine models of chronic and acute liver disease, which may be related to increased inflammation.
Vena cava filters in hospitalised patients with chronic obstructive pulmonary disease and pulmonary embolism
Authors: Matta Fadi , Stein Paul D. 
Published: March 2013

SummaryIn view of the high case fatality rates of patients with chronic obstructive pulmonary disease (COPD) who have pulmonary embolism (PE) we speculated that such patients might benefit from vena cava filters. To test this hypothesis we assessed the database of the Nationwide Inpatient Sample. From 1998–2009, 440,370 patients were hospitalised with PE and COPD who were not in shock or ventilator-dependent and did not receive thrombolytic therapy or pulmonary embolectomy. In-hospital all-cause case fatality rate among those with filters was 5,890 of 68,800 (8.6%) (95% confidence interval [CI] = 8.4–8.8) compared with 38,960 of 371,570 (10.5%) (95% CI = 10.4–10.6) (p<0.0001) who did not receive filters. Case fatality rate was age-dependent. Only those who were older than aged 50 years had a lower in-hospital all-cause case fatality rate with filters. Among such patients, absolute risk reduction was 2.1% (95% CI = 1.9–2.3). The greatest reduction of case fatality rate with vena cava filters was shown in patients >aged 80 years, 11,720 of 81,600 (14.4%) compared with 1,570 of 17,220 (9.1%) (p<0.0001). In conclusion, a somewhat lower in-hospital all-cause case fatality rate was shown with vena filters in stable patients with PE >aged 50 years who also had COPD. The benefit was greatest in elderly patients. The benefit in terms of a decreased case fatality rate would seem to outweigh the risks of vena cava filters in such patients.
Effect of Adaptation on Object Representation Accuracy in Macaque Inferior Temporal Cortex
Authors: Kaliukhovich Dzmitry A. , De Baene Wouter , Vogels Rufin 
Published: March 2013

Abstract
Stimulus repetition produces a decrease of the response in many cortical areas and different modalities. This adaptation is highly prominent in macaque inferior temporal (IT) neurons. Here we ask how these repetition-induced changes in IT responses affect the accuracy by which IT neurons encode objects. This question bears on the functional consequences of adaptation, which are still unclear. We recorded the responses of single IT neurons to sequences of familiar shapes, each shown for 300 msec with an ISI of the same duration. The difference in shape between the two successively presented stimuli,that is, adapter and test, varied parametrically. The discriminability of the test stimuli was reduced for repeated compared with nonrepeated stimuli. In some conditions for which adapter and test shapes differed, the cross-adaptation resulted in an enhanced discriminability. These single cell results were confirmed in a second experiment in which we recorded multiunit spiking activity using a laminar microelectrode in macaque IT. Two familiar stimuli were presented successively for 500 msec each and separated with an ISI of the same duration. Trials consisted either of a repetition of the same stimulus or of their alternation. Small neuronal populations showed decreased classification accuracy for repeated compared with nonrepeated test stimuli, but classification was enhanced for the test compared with adapter stimuli when the test stimulus differed from recently seen stimuli. These findings suggest that short-term, stimulus-specific adaptation in IT supports efficient coding of stimuli that differ from recently seen ones while impairing the coding of repeated stimuli.
Working Memory Processes Are Mediated by Local and Long-range Synchronization of Alpha Oscillations
Authors: Crespo-Garcia Maite , Pinal Diego , Cantero Jose L. , Díaz Fernando , Zurrón Montserrat , Atienza Mercedes 
Published: March 2013

Abstract
Different cortical dynamics of alpha oscillations (8–13 Hz) have been associated with increased working memory load, which have been mostly interpreted as a neural correlate of functional inhibition. This study aims at determining whether different manifestations of load-dependent amplitude and phase dynamics in the alpha band can coexist over different cortical regions. To address this question, we increased information load by manipulating the number and spatial configuration of domino spots. Time–frequency analysis of EEG source activity revealed (i) load-independent increases of both alpha power and interregional alpha-phase synchrony within task-irrelevant, posterior cortical regions and (ii) load-dependent decreases of alpha power over areas of the left pFC and bilateral posterior parietal cortex (PPC) preceded in time by load-dependent decreases of alpha-phase synchrony between the left pFC and the left PPC. The former results support the role of alpha oscillations in inhibiting irrelevant sensorimotor processing, whereas the latter likely reflect release of parietal task-relevant areas from top–down inhibition with load increase. This interpretation found further support in a significant latency shift of 15 msec from pFC to the PPC. Together, these results suggest that amplitude and phase alpha dynamics in both local and long-range cortical networks reflect different neural mechanisms of top–down control that might be crucial in mediating the different working memory processes.
Functional Neuroimaging and Psychology: What Have You Done for Me Lately?
Authors: Moran Joseph M. , Zaki Jamil 
Published: March 2013

Abstract
Functional imaging has become a primary tool in the study of human psychology but is not without its detractors. Although cognitive neuroscientists have made great strides in understanding the neural instantiation of countless cognitive processes, commentators have sometimes argued that functional imaging provides little or no utility for psychologists. And indeed, myriad studies over the last quarter century have employed the technique of brain mapping—identifying the neural correlates of various psychological phenomena—in ways that bear minimally on psychological theory. How can brain mapping be made more relevant to behavioral scientists broadly? Here, we describe three trends that increase precisely this relevance: (i) the use of neuroimaging data to adjudicate between competing psychological theories through forward inference, (ii) isolating neural markers of information processing steps to better understand complex tasks and psychological phenomena through probabilistic reverse inference, and (iii) using brain activity to predict subsequent behavior. Critically, these new approaches build on the extensive tradition of brain mapping, suggesting that efforts in this area—although not initially maximally relevant to psychology—can indeed be used in ways that constrain and advance psychological theory.
Visual Sensitivity Shifts with Perceived Eye Position
Authors: Odoj Bartholomäus , Balslev Daniela 
Published: March 2013

AbstractSpatial attention can be defined as the selection of a location for privileged stimulus processing. Most oculomotor structures, such as the superior colliculus or the FEFs, play an additional role in visuospatial attention. Indeed, electrical stimulation of these structures can cause changes in visual sensitivity that are location specific. We have proposed that the recently discovered ocular proprioceptive area in the human postcentral gyrus (S1EYE) may have a similar function. This suggestion was based on the observation that a reduction of excitability in this area with TMS causes not only a shift in perceived eye position but also lateralized changes in visual sensitivity. Here we investigated whether these shifts in perceived gaze position and visual sensitivity are spatially congruent. After continuous theta burst stimulation over S1EYE, participants underestimated own eye rotation, so that saccades from a lateral eye rotation undershoot a central sound (Experiment 1). They discriminated letters faster if they were presented nearer the orbit midline (Experiment 2) and spent less time looking at locations nearer the orbit midline when searching for a nonexistent target in a letter array (Experiment 3). This suggests that visual sensitivity increased nearer the orbit midline, in the same direction as the shift in perceived eye position. This spatial congruence argues for a functional coupling between the cortical eye position signal in the somatosensory cortex and visuospatial attention.
Resentment and Renewal: Toward a Theory of the History of Judaism
Authors: Neusner Jacob 
Published: March 2013

Abstract
Diverse Judaic religious systems, from the Pentateuchal statement to the Zionist reiteration of that statement, endure and will define future Judaisms too. A single paradigm defines those systems, however varied they are in detail. I propose to explain the endurance of the Judaic paradigm, which is realized in the myth of exile and return.
Climate Simulation and Change in the Brazilian Climate Model
Authors: Nobre Paulo , Siqueira Leo S. P. , de Almeida Roberto A. F. , Malagutti Marta , Giarolla Emanuel , Castelão Guilherme P. , Bottino Marcus J. , Kubota Paulo , Figueroa Silvio N. , Costa Mabel C. , Baptista Manoel , Irber Luiz , Marcondes Gabriel G. 
Published: March 2013

AbstractThe response of the global climate system to atmospheric CO2 concentration increase in time is scrutinized employing the Brazilian Earth System Model Ocean–Atmosphere version 2.3 (BESM-OA2.3). Through the achievement of over 2000 yr of coupled model integrations in ensemble mode, it is shown that the model simulates the signal of recent changes of global climate trends, depicting a steady atmospheric and oceanic temperature increase and corresponding marine ice retreat. The model simulations encompass the time period from 1960 to 2105, following the phase 5 of the Coupled Model Intercomparison Project (CMIP5) protocol. Notwithstanding the accurate reproduction of large-scale ocean–atmosphere coupled phenomena, like the ENSO phenomena over the equatorial Pacific and the interhemispheric gradient mode over the tropical Atlantic, the BESM-OA2.3 coupled model shows systematic errors on sea surface temperature and precipitation that resemble those of other global coupled climate models. Yet, the simulations demonstrate the model’s potential to contribute to the international efforts on global climate change research, sparking interest in global climate change research within the Brazilian climate modeling community, constituting a building block of the Brazilian Framework for Global Climate Change Research.
Comparing Cyclone Life Cycle Characteristics and Their Interannual Variability in Different Reanalyses
Authors: Tilinina Natalia , Gulev Sergey K. , Rudeva Irina , Koltermann Peter 
Published: March 2013

Abstract
Characteristics of Northern Hemisphere extratropical cyclone activity were compared for five concurrent reanalyses: the NCEP–U.S. Department of Energy (DOE) reanalysis (herein NCEP–DOE), the Japanese 25-year Reanalysis Project (JRA-25), the ECMWF Interim Re-Analysis (ERA-Interim), the National Aeronautics and Space Administration's Modern-Era Retrospective Analysis for Research and Applications (NASA-MERRA), and the NCEP Climate Forecast System Reanalysis (NCEP-CFSR), for the period 1979–2010 using a single cyclone tracking algorithm. The total number of cyclones, ranging from 1400 to more than 1800 yr−1, was found to depend strongly on the spatial resolution of the respective reanalysis. The largest cyclone population was identified using NASA-MERRA data, which also showed the highest occurrence of very deep cyclones. Of the reanalyses, two (NCEP–DOE and ERA-Interim) are associated with statistically significant positive trends in the total number of cyclones from 1% to 2% decade−1. These trends result from moderate and shallow cyclones contributing to approximately 90% of the total cyclone count on average. The number of very deep cyclones (&lt;960 hPa) in the North Atlantic increased in most reanalyses until 1990 and then declined during the last decade. In the North Pacific, the number of these events reached a peak in 2000 and then decreased during the last decade. The winter pattern is characterized by robust trends in cyclone numbers, with an enhancement of the North Atlantic storm track and a weakening of the North Pacific subtropical storm track. In the summer, there is a robust intensification of the Mediterranean storm track and a decrease in counts over the North Atlantic. Interannual variability and decadal-scale variations of the cyclone counts are highly correlated among the reanalyses, with the greatest agreement in moderate and deep cyclones.
Determination of Planetary Boundary Layer Height on Short Spatial and Temporal Scales: A Demonstration of the Covariance Wavelet Transform in Ground-Based Wind Profiler and Lidar Measurements*
Authors: Compton Jaime C. , Delgado Ruben , Berkoff Timothy A. , Hoff Raymond M. 
Published: March 2013

Abstract
This article explores the application of the covariance wavelet transform (CWT) to lidar and, for the first time to the authors' knowledge, wind profiler data to examine the possibility of accurate and continuous planetary boundary layer (PBL) height measurements on short temporal resolution (1- and 15-min averages, respectively). Determining the mixing in the PBL was one goal of a study of the spatial and diurnal variations of the PBL height over Maryland for July 2011, during NASA's Earth Venture mission DISCOVER-AQ. The PBL heights derived from ground-based lidars [at University of Maryland, Baltimore County (UMBC); 39.25°N, 76.70°W], a 915-MHz wind profiler, and radiosondes (at Beltsville, Maryland; 38.92°N, 77.02°W) were compared. Results from the comparison show an R2 = 0.89, 0.92, and 0.94 correlation between the radiosonde PBL heights and two lidars and wind profiler PBL heights, respectively. Accurate determination of the PBL height by applying the CWT to lidar and wind profilers will allow for improved air quality forecasting and understanding of regional pollution dynamics.
Direct Calculation of Thermodynamic Wet-Bulb Temperature as a Function of Pressure and Elevation
Authors: Sadeghi Sayed-Hossein , Peters Troy R. , Cobos Douglas R. , Loescher Henry W. , Campbell Colin S. 
Published: March 2013

Abstract
A simple analytical method was developed for directly calculating the thermodynamic wet-bulb temperature from air temperature and the vapor pressure (or relative humidity) at elevations up to 4500 m above MSL was developed. This methodology was based on the fact that the wet-bulb temperature can be closely approximated by a second-order polynomial in both the positive and negative ranges in ambient air temperature. The method in this study builds upon this understanding and provides results for the negative range of air temperatures (−17° to 0°C), so that the maximum observed error in this area is equal to or smaller than −0.17°C. For temperatures ≥0°C, wet-bulb temperature accuracy was ±0.65°C, and larger errors corresponded to very high temperatures (Ta ≥ 39°C) and/or very high or low relative humidities (5% &lt; RH &lt; 10% or RH &gt; 98%). The mean absolute error and the root-mean-square error were 0.15° and 0.2°C, respectively.
Assessing Ensemble Forecasts of Low-Level Supercell Rotation within an OSSE Framework
Authors: Potvin Corey K. , Wicker Louis J. 
Published: March 2013

Abstract
Under the envisioned warn-on-forecast (WoF) paradigm, ensemble model guidance will play an increasingly critical role in the tornado warning process. While computational constraints will likely preclude explicit tornado prediction in initial WoF systems, real-time forecasts of low-level mesocyclone-scale rotation appear achievable within the next decade. Given that low-level mesocyclones are significantly more likely than higher-based mesocyclones to be tornadic, intensity and trajectory forecasts of low-level supercell rotation could provide valuable guidance to tornado warning and nowcasting operations. The efficacy of such forecasts is explored using three simulated supercells having weak, moderate, or strong low-level rotation. The results suggest early WoF systems may provide useful probabilistic 30–60-min forecasts of low-level supercell rotation, even in cases of large radar–storm distances and/or narrow cross-beam angles. Given the idealized nature of the experiments, however, they are best viewed as providing an upper-limit estimate of the accuracy of early WoF systems.
Motivational Goals and School Achievement: Lebanese-Background Students in South-Western Sydney
Authors: Suliman Rosemary , McInerney Dennis M 
Published: March 2013

There is a general belief that Lebanese-background students in Australia do not usually perform well at school. This study examines the school achievement of Lebanese-background students and the major motivational factors related to their achievement. A total of 271 Year Nine students (117 Lebanese-background and 154 non-Lebanese background) in three high schools in southwestern Sydney completed the Inventory of School Motivation and the Facilitating Conditions Questionnaire and this information was used to examine the students' motivational profiles and the salience of motivational and other background variables in predicting school achievement. The study indicates that the Lebanese-background group is lower on variables which predict both their English and Science results, such as their valuing of education, and their perceived family and teacher support. The strongest predictors for achievement for the Lebanese-background group were competition, social concern, recognition, and token reward; however, these variables were not the strongest motivators for the students of Lebanese background. The variable most strongly valued by the Lebanese-background group was power, and that was found to be negatively related to their school achievement. The significance of these findings is that the Lebanese-background group does not strongly value the positive predictors for school achievement and attention needs to be paid to enhance these among this group; and that the Lebanese-background group shows higher levels of the negative predictors and hence attention needs to be given to diminish the students' negative perspectives which predict lower academic performance.
The Reliability, Discrimination and Difficulty of Word-Knowledge Tests Employing Multiple Choice Items Containing Three, Four, or Five Alternatives
Authors: Hogben Donald 
Published: March 2013

The multiple-choice item found on standardized and teacher-made tests typically requires the examinee to choose among either four or five alternative answers. The present study offers some evidence favouring three-option over four-option items. Word knowledge tests containing three, four, and five-option items were developed and administered to pupils in ten upper primary classes from schools in the Adelaide metropolitan area. In each of the classes, item discrimination was better and reliability was higher for the three-option test compared to the test employing four-option items. The five-option test was also superior to the four-option test. Differences between the three and five-option tests on reliability and item discrimination were not significant.
An Economic Approach to Teacher Loss and Retention
Authors: Smith C. Selby 
Published: March 2013

In the late 1960's the imbalance between the supply and demand for teacher services was fundamental to the problems of Australian education. This article outlines an analytical framework which helps in understanding some important features of that imbalance. The paper comes to three main conclusions: that high levels of teacher resignation were probably a rational response to the conditions governing their recruitment and the level and career structure of their earnings; that if resignation rates for teachers are to be reduced in a full employment labour market, changes are required in their salary structure and career prospects; and that the statistical data available on a consistent national basis are seriously inadequate for satisfactory economic analysis.