bluuebunny/crossref_metadata_2025_split huggingface dataset:
Risk of Death in Heart Disease is Associated With Elevated Urinary Globotriaosylceramide
Authors: Schiffmann Raphael , Forni Sabrina , Swift Caren , Brignol Nastry , Wu Xiaoyang , Lockhart David J. , Blankenship Derek , Wang Xuan , Grayburn Paul A. , Taylor Matthew R. G. , Lowes Brian D. , Fuller Maria , Benjamin Elfrida R. , Sweetman Lawrence 
Published: February 2014

Background

Elevated urinary globotriaosylceramide (Gb
3
) has been considered a hallmark of
F
abry disease, an X‐linked lysosomal disorder that is a risk factor for most types of heart disease.

Methods and Results

We screened 1421 consecutive patients with common forms of heart disease for
F
abry disease by measuring urinary Gb
3
in whole urine using tandem mass spectrometry, α‐galactosidase A activity in dried blood spots, and we looked for
GLA
mutations by parallel sequencing of the whole gene (exons and introns) in pooled genomic
DNA
samples followed by Sanger sequencing verification.

GLA

variants were found in 13 patients. In the 1408 patients without

GLA

mutations, urinary Gb
3
levels were significantly higher in heart disease patients compared to 116 apparently healthy controls (median difference=10.0 ng/mL and
P
<0.001). Urinary lipid profiling showed that levels of 5 other lipids significantly distinguished between urine of patients with
F
abry disease (n=7) and heart disease patients with elevated urinary Gb
3
(n=6). Sphingomyelin and Gb
3
levels were abnormal in the left ventricular wall of patients with ischemic heart failure. Elevated levels of urinary Gb
3
were independently associated with increased risk of death in the average follow‐up of 17 months (hazard ratio=1.59 for increase in Gb
3
of 200, 95%
CI
=1.36 and 1.87, and
P
<0.0001).

Conclusions

In heart disease patients who do not have
F
abry disease or

GLA

gene mutations, a higher level of urinary Gb
3
is positively associated with near‐term mortality. The elevation of urinary Gb
3
and that of other lipids suggests that heart disease is associated with multiorgan lipid abnormalities.

Clinical Trial Registration

URL: clinicaltrials.gov. Unique Identifier:
NCT
01019629.
Evaluation of Computed Tomography Angiography Plaque Thickness Measurements in High-Grade Carotid Artery Stenosis
Authors: Gupta Ajay , Baradaran Hediyeh , Kamel Hooman , Pandya Ankur , Mangla Atul , Dunning Allison , Marshall Randolph S. , Sanelli Pina C. 
Published: February 2014

Background and Purpose—
Increasing evidence suggests that carotid artery imaging can identify vulnerable plaque elements that increase stroke risk. We correlated recently proposed markers, soft and hard plaque thickness measurements on axial computed tomography angiography source images, with symptomatic disease status (ipsilateral stroke or transient ischemic attack) in high-grade carotid disease.

Methods—
Soft plaque and hard plaque thickness were measured with a recently validated technique using computed tomography angiography source images in subjects with ≥70% extracranial carotid artery stenosis. Logistic regression analyses were used to assess the strength of association between soft and hard plaque thickness measurements and previous stroke or transient ischemic attack. Receiver operating characteristic analysis was also performed.

Results—
Compared with asymptomatic subjects, those with symptomatic carotid disease had significantly larger soft plaque and total plaque thickness measurements and smaller hard plaque thickness measurements. Each 1-mm increase in soft plaque resulted in a 2.7 times greater odds of previous stroke or transient ischemic attack. Soft plaque thickness measurements provided excellent discrimination between symptomatic and asymptomatic disease, with receiver operating characteristic analysis showing an area under the curve of 0.90. A cutoff of 3.5-mm maximum soft plaque thickness provided a sensitivity of 81%, specificity of 83%, positive predictive value of 85%, and a negative predictive value of 78%.

Conclusions—
Increasing maximum soft plaque thickness measurements are strongly associated with symptomatic disease status in carotid artery stenosis. Prospective validation of these results may translate into a widely accessible stroke risk stratification tool in high-grade carotid artery atherosclerotic disease.
Clinical Associations and Causes of Convexity Subarachnoid Hemorrhage
Authors: Khurram Ashan , Kleinig Timothy , Leyden James 
Published: February 2014

Background and Purpose—
It has been previously found noted that ≈15% to 20% of subarachnoid hemorrhage (SAH) is nonaneurysmal. Nontraumatic convexity SAH (cSAH) is increasingly recognized. Data concerning incidence and associations are scant.

Methods—
We identified all SAH-coded cases from South Australian public hospitals between January 2005 and July 2011. Electronic discharge summaries were reviewed, and cases of cSAH were ascertained. Clinical and radiological features were recorded, and pathogenesis was assigned.

Results—
Of 742 cases with SAH, 41 (6%) cases were cSAH, giving a minimum population annual incidence of 5.1 per million (95% confidence interval, 3.7–7.0). Median age was 70 years (interquartile range, 48–79). Commonest causes were cerebral amyloid angiopathy (39%), reversible cerebral vasoconstriction syndrome (17%), cerebral venous sinus thrombosis (10%), large-vessel stenotic atherosclerosis (10%), and posterior reversible encephalopathy syndrome (5%). No cause was identified in 20% (mostly elderly patients with incomplete evaluation). Most (63%) presented with transient neurological symptoms. Many (49%) were misdiagnosed as transient ischemic attacks and treated inappropriately with antithrombotics.

Conclusions—
cSAH comprises a significant proportion of SAH. Commonest causes are cerebral amyloid angiopathy in the elderly and reversible cerebral vasoconstriction syndrome in the young, but differential diagnosis is broad. Misdiagnosis is common and leads to potentially harmful treatments.
Modeling the Impacts of a Man-Made Lake on the Meteorological Conditions of the Surrounding Areas
Authors: Klaić Zvjezdana B. , Kvakić Marko 
Published: February 2014

AbstractThe possible modifications of the surface meteorological conditions due to construction of a small, man-made lake in midlatitudes (surface area of 11.55 km2 and maximum fetch distance L ≈ 5 km) were investigated using the Weather Research and Forecasting (WRF) model (version 3.3). The model was applied to four real typical synoptic situations for which the area of interest was under the influence of a wintertime anticyclone (WA), a wintertime cyclone (WC), a summertime anticyclone (SA), and a summertime cyclone (SC). Each of these four typical synoptic setups was simulated twice—once assuming the present state (“NO-LAKE” experiment) and again assuming the existence of the new lake (“LAKE” experiment). The differences between 36-h average LAKE and NO-LAKE simulation results show noticeable mean changes in the surface temperature and relative humidity as well as a small increase of the mean surface wind speeds in the air above the newly constructed lake. At other portions of the investigated area (distances up to ~4L–6L downstream of the new lake), the 36-h mean differences produced by the new lake are below the order of magnitude of accuracy of operational meteorological measurement instruments. In individual hours, however, these differences are occasionally very high, particularly for cyclonic episodes. In addition, results obtained for SA suggest an existence of a lake circulation cell associated with small differences between the lake and land temperatures (at most up to ~3°–5°C) and consequently, a slight enhancement of slope winds in future (LAKE) conditions.
What Controls the Mean East–West Sea Surface Temperature Gradient in the Equatorial Pacific: The Role of Cloud Albedo
Authors: Burls N. J. , Fedorov A. V. 
Published: February 2014

Abstract
The mean east–west sea surface temperature gradient along the equator is a key feature of tropical climate. Tightly coupled to the atmospheric Walker circulation and the oceanic east–west thermocline tilt, it effectively defines tropical climate conditions. In the Pacific, its presence permits the El Niño–Southern Oscillation phenomenon. What determines this temperature gradient within the fully coupled ocean–atmosphere system is therefore a central question in climate dynamics, critical for understanding past and future climates. Using a comprehensive coupled model [Community Earth System Model (CESM)], the authors demonstrate how the meridional gradient in cloud albedo between the tropics and midlatitudes (Δα) sets the mean east–west sea surface temperature gradient in the equatorial Pacific. To change Δα in the numerical experiments, the authors change the optical properties of clouds by modifying the atmospheric water path, but only in the shortwave radiation scheme of the model. When Δα is varied from approximately −0.15 to 0.1, the east–west SST contrast in the equatorial Pacific reduces from 7.5°C to less than 1°C and the Walker circulation nearly collapses. These experiments reveal a near-linear dependence between Δα and the zonal temperature gradient, which generally agrees with results from the Coupled Model Intercomparison Project phase 5 (CMIP5) preindustrial control simulations. The authors explain the close relation between the two variables using an energy balance model incorporating the essential dynamics of the warm pool, cold tongue, and Walker circulation complex.
A Framework for Dynamical Seasonal Prediction of Precipitation over the Pacific Islands
Authors: Annamalai H. , Hafner J. , Kumar A. , Wang H. 
Published: February 2014

Abstract
A three-step approach to develop a framework for dynamical seasonal prediction of precipitation over the U.S. Affiliated Pacific Islands (USAPI) is adopted. First, guided by the climatological features of basic variables, a view that climates of the USAPI are connected by large-scale phenomena involving the warm pool, South Pacific convergence zone, tropical monsoons, and subtropical anticyclone is proposed. Second, prediction skill in ensemble hindcasts performed with the Climate Forecast System, version 2 (CFSv2), is evaluated with the hypothesis that ENSO is the leading candidate for large and persisting precipitation departures. Third, moist static energy budget diagnostics are performed to identify physical processes responsible for precipitation anomalies.
At leads of 0–6 months, CFSv2 demonstrates useful skill in predicting Niño-3.4 SST and equatorial Pacific precipitation anomalies. During El Niño, positive precipitation anomalies along the central (eastern) equatorial Pacific are anchored by net radiative flux (Frad) and moist advection (evaporation and Frad). The model’s skill in predicting precipitation anomalies over South Pacific (Hawaiian) islands is highest (lowest). Over the west Pacific islands, the skill is low during the rainy season. During El Niño, skill over the USAPI, in particular predicting dryness persistence at long leads is useful. Suppressed precipitation over the Hawaiian and South Pacific (west Pacific) islands are determined by anomalous dry and cold air advection (reduced evaporation and Frad). These processes are local, but are dictated by circulation anomalies forced by ENSO. Model budget estimates are qualitatively consistent with those obtained from reanalysis, boosting confidence for societal benefits. However, observational constraints, as well as budget residuals, pose limitations.
Benchmarking a Soil Moisture Data Assimilation System for Agricultural Drought Monitoring
Authors: Han Eunjin , Crow Wade T. , Holmes Thomas , Bolten John 
Published: February 2014

Abstract
Despite considerable interest in the application of land surface data assimilation systems (LDASs) for agricultural drought applications, relatively little is known about the large-scale performance of such systems and, thus, the optimal methodological approach for implementing them. To address this need, this paper evaluates an LDAS for agricultural drought monitoring by benchmarking individual components of the system (i.e., a satellite soil moisture retrieval algorithm, a soil water balance model, and a sequential data assimilation filter) against a series of linear models that perform the same function (i.e., have the same basic input/output structure) as the full system component. Benchmarking is based on the calculation of the lagged rank cross correlation between the normalized difference vegetation index (NDVI) and soil moisture estimates acquired for various components of the system. Lagged soil moisture/NDVI correlations obtained using individual LDAS components versus their linear analogs reveal the degree to which nonlinearities and/or complexities contained within each component actually contribute to the performance of the LDAS system as a whole. Here, a particular system based on surface soil moisture retrievals from the Land Parameter Retrieval Model (LPRM), a two-layer Palmer soil water balance model, and an ensemble Kalman filter (EnKF) is benchmarked. Results suggest significant room for improvement in each component of the system.
Evaluation of CLASS 2.7 and 3.5 Simulations of Snow Properties from the Canadian Regional Climate Model (CRCM4) over Québec, Canada*
Authors: Langlois A. , Bergeron J. , Brown R. , Royer A. , Harvey R. , Roy A. , Wang L. , Thériault N. 
Published: February 2014

Abstract
Snow cover simulations from versions 2.7 and 3.5 of the Canadian Land Surface Scheme (CLASS) coupled to the Canadian Regional Climate Model, version 4 (CRCM4), are evaluated over northern Québec and the larger Québec domain using in situ and remotely sensed datasets. Version 2.7 of CLASS has been used in the operational version of CRCM4 at Ouranos since 2006. Version 3.5 includes a number of improvements to the snow processes as well as a more realistic parameterization of snow thermal conductivity. The evaluation shows that version 3.5 provides improved simulations of snow water equivalent, density, depth, and snowpack temperature values. However, snowpack density still contains systematic biases during the snow season that need to be addressed. The snow albedo parameterization in CLASS was found to be very sensitive to an empirical snowfall rate threshold for albedo refreshment and does not keep track of the snow accumulation history in estimating the snow surface albedo. A modified albedo scheme based on snow-specific surface areas is proposed to address this problem.
A New Method for Generating Initial Condition Perturbations in a Regional Ensemble Prediction System: Blending
Authors: Wang Yong , Bellus Martin , Geleyn Jean-Francois , Ma Xulin , Tian Weihong , Weidle Florian 
Published: February 2014

Abstract
A blending method for generating initial condition (IC) perturbations in a regional ensemble prediction system is proposed. The blending is to combine the large-scale IC perturbations from a global ensemble prediction system (EPS) with the small-scale IC perturbations from a regional EPS by using a digital filter and the spectral analysis technique. The IC perturbations generated by blending can well represent both large-scale and small-scale uncertainties in the analysis, and are more consistent with the lateral boundary condition (LBC) perturbations provided by global EPS. The blending method is implemented in the regional ensemble system Aire Limitée Adaptation Dynamique Développement International-Limited Area Ensemble Forecasting (ALADIN-LAEF), in which the large-scale IC perturbations are provided by the European Centre for Medium-Range Weather Forecasts (ECMWF-EPS), and the small-scale IC perturbations are generated by breeding in ALADIN-LAEF. Blending is compared with dynamical downscaling and breeding over a 2-month period in summer 2007. The comparison clearly shows impact on the growth of forecast spread if the regional IC perturbations are not consistent with the perturbations coming through LBC provided by the global EPS. Blending can cure the problem largely, and it performs better than dynamical downscaling and breeding.
Assessing the Predictability of Convection Initiation in the High Plains Using an Object-Based Approach
Authors: Burghardt Brock J. , Evans Clark , Roebber Paul J. 
Published: February 2014

Abstract
This study investigates the short-range (0–12 h) predictability of convection initiation (CI) using the Advanced Research Weather Research and Forecasting (WRF) Model (ARW) with a horizontal grid spacing of 429 m. A unique object-based method is used to evaluate model performance for 25 cases of CI across the west-central high plains of the United States from the 2010 convective season. In the aggregate, there exists a high probability of detection but, due to the significant overproduction of CI events by the model, high false alarm and bias ratios that lead to modestly skillful forecasts. Model CI objects that are matched with observed CI objects show, on average, an early bias of about 3 min and distance errors of around 38 km. The operational utility and inherent biases of such high-resolution simulations are discussed.
Immulite 2000 Parathyroid Hormone Assay: Stability of Parathyroid Hormone in EDTA Blood Kept at Room Temperature for 48 h
Authors: Omar H , Chamberlin A , Walker V , Wood P J 
Published: February 2014

Parathyroid hormone (PTH) concentrations were compared in serum and EDTA plasma from 36 patients attending a renal stone clinic. Serum PTH concentrations ranged from 0·9 to 10·9pmol/L, with a mean of 4·6pmol/L. When serum and EDTA plasma results were compared, in samples frozen within 30 min of collection, EDTA plasma results were found to be significantly higher than those in serum ( P <0·0001; Wilcoxon test), with an average increase of 19·5% over the serum result. Results from EDTA-preserved blood left to stand at room temperature for 48 h were on average 14·8% lower than results from the corresponding EDTA plasma samples frozen within 30 min, with a highly significant difference ( P <0·0001). Freshly frozen serum and 48h EDTA plasma PTH results were not significantly different. Parathyroid hormone in EDTA-preserved blood is not completely stable, and this could lead to misclassification of results for samples which are not frozen quickly.
Contamination by Iodine in the AutoAnalyzer Method for Serum Protein-Bound Iodine
Authors: Wilding P. , Rollason J. G. 
Published: February 2014

A phenomenon is described whereby iodine was shown to be adsorbed from aqueous standards by precipitated protein, to be subsequently ‘leached-off’ this protein by the first serum sample. The excessive protein precipitation was thought to be due to the water as supplied by a mixed-bed automatic deioniser. A simple method for detecting this phenomenon is to follow the range of aqueous standards with three identical serum samples. It is probable that the sensitivity of the standard curve is diminished due to the phenomenon described if the first of the replicate samples has a peak height greater than the subsequent samples.
Evaluation of a Kit for Serum Calcium Determination
Authors: Cossar J. M. , Fitzpatrick J. 
Published: February 2014

A kit for serum calcium determination (Calcium Rapid Stat Kit), based on a methyl thymol blue method, has been investigated and the results compared with those obtained from an AutoAnalyzer cresolphthalein complexone method. The kit technique is simple, requires a small amount of serum and has a better precision than the AutoAnalyzer method, with which it correlates well. The standards supplied should be checked before use.
An Evaluation of the “CentrifiChem” Parallel Fast Analyser
Authors: Henry P. , Saunders R. A. 
Published: February 2014

The operating principles of the CentrifiChem are briefly described and the results of the evaluation are summarised. The pipettor, the temperature control unit, and the photometer were tested separately, but the remainder of the instrument is interdependent. Up to 10 different tests were used to measure within-batch and between-day precision, and accuracy. Carryover was found to be small, and for the six tests investigated the linearity was good. Estimates are given of the running costs, and also included are sections on the reliability and safety of the instrument. Since it requires only a small volume of serum, it is ideally suited to paediatric requirements. When used as an enzyme analyser it can work at about 200 specimens per hour.
Interference by Polar Metabolites in a Direct Radioimmunoassay for Plasma Aldosterone
Authors: Jones Julia C , Carter Graham D , Macgregor Graham A 
Published: February 2014

The CIS Aldoctk-125 kit, a direct radioimmunoassay for plasma aldosterone, has been compared with a conventional technique involving solvent extraction. Results given by these two methods were poorly correlated (r = 0·445, n = 103), the direct assay giving higher values, particularly in patients being dialysed for renal failure. When the kit was modified to include an extraction step, results correlated well with those of the standard method (r = 0·952, n = 60). These observations suggested interference from polar metabolites. The possibility that glucuronides were responsible was investigated by measuring plasma aldosterone before and after hydrolysis with β-glucuronidase. Higher post-hydrolysis values confirmed the presence of glucuronides in plasma from normal subjects and patients with renal failure. Preliminary chromatographic studies on plasma from nine dialysis patients indicated the presence of tetrahydroaldosterone 3-glucuronide, and it is thought that this metabolite might contribute to the high values obtained with the direct assay.
Gel Isoelectric Focusing and IgG Index for Demonstration of Intrathecal IgG Synthesis in Neurological Disorders
Authors: Van Bergh Frank A J T M , Roos Raymund A C 
Published: February 2014

Cerebrospinal fluid (CSF) and serum were obtained from 37 patients with multiple sclerosis and from 112 patients with various other neurological diseases, and the concentrations of IgG and albumin were quantitated immunochemically. Simultaneously, isoelectric focusing (IF) was carried out for the detection of CSF-specific oligoclonal bands in the alkaline region. CSF-specific components were found in 51 patients by IF, the estimated number of which correlated significantly (Rs = 0·733, p<0·0001) with the value of the IgG index. In addition, six patients without CSF-specific abnormalities on IF had an increased IgG index (>0·70). In two of them this increase is, in all probability, false-positive; three others showed fully identical IF bands in both CSF and serum. On the other hand, 13 of the same 51 patients showed a normal IgG index (<0·70) although CSF-specific components were detectable on IF. These results confirm the superiority of IF over the immunochemical quantitation of albumin and total IgG for the evaluation of immune reactivity in the central nervous system.
Improved Performance of Plasma Gonadotrophin Assays Using Common Reagents and Assay Protocols: Evidence from the UK External Quality Assessment Scheme
Authors: Hayes Margaret , Ferguson K M , Jeffcoate S L , Bacon R R A , Seth J 
Published: February 2014

Radioimmunoassay kits prepared in the Chelsea Hospital for Women for follicle stimulating hormone (FSH) and luteinising hormone (LH) have been used in 26 UK laboratories for over 2 years. Data from the UK External Quality Assessment Schemes for FSH and LH have been used to provide an independent assessment of the performance of these kits over a 12-month period. For both analytes, users of the kits were found to have: a low variability of the bias, implying good within-laboratory, between-assay precision; a lower between-laboratory, within-sample geometric coefficient of variation than users of ‘own’ methods, implying better between-laboratory agreement; method bias that did not differ significantly from laboratories using ‘own’ method protocols. This survey indicates that a non-commercial organisation can produce immunoassay kits that improve the quality of FSH and LH assays generally available.