bluuebunny/crossref_metadata_2025_split huggingface dataset:
A novel frameshift mutation of epithelial sodium channel β‐subunit leads to Liddle syndrome in an isolated case
Authors: Yang Kun‐Qi , Lu Chao‐Xia , Xiao Yan , Liu Ya‐Xin , Jiang Xiong‐Jing , Zhang Xue , Zhou Xian‐Liang 
Published: November 2014

SummaryObjectiveLiddle syndrome, an autosomal dominant form of monogenic hypertension, is attributed to mutations in the genes encoding β and γ subunits (SCNN1B and SCNN1G) of the epithelial sodium channel (ENaC). The aim of this study was to search for pathogenic mutations of SCNN1B and SCNN1G in an adolescent under the impression of Liddle syndrome and no family history of hypertension.Design and PatientsWe screened the C‐terminus of SCNN1B and SCNN1G in an adolescent with poorly controlled hypertension who was clinically diagnosed as having Liddle syndrome. We also screened for the mutation in his parents, 100 hypertensive patients and 100 controls.ResultsGenetic analysis of SCNN1B revealed a frameshift mutation induced by insertion of an additional cytosine into a string of six located between codons 617 and 618, which is predicted to introduce a new termination codon at position 621 and produce a protein truncated by 20 amino acids. This frameshift mutation was not detected in the patient's parents, the 100 hypertensive patients or the 100 controls, indicating that this is a de novo mutation and not a common genetic polymorphism. There was no mutation of SCNN1G in any of the individuals examined.ConclusionBased on direct DNA sequencing, we identified a novel frameshift mutation in the βENaC gene in an isolated case of Liddle syndrome. Confirmation of the diagnosis and effective tailored treatment in the patient were achieved, implying that genetic testing is a useful tool to diagnose Liddle syndrome.
Status of thousand cankers disease on eastern black walnut in the easternUnitedStates at two locations over 3 years
Authors: Griffin G. J. 
Published: November 2014

SummaryThousand cankers disease (TCD), a lethal fungal dieback of eastern black walnut (Juglans nigra), caused byGeosmithia morbida, and spread by the walnut twig beetle,Pityophthorus juglandis, was documented in 2009 to be very destructive in the western United States and was identified in the native range ofJ. nigraat Knoxville, Tennessee, in 2010, and in 2011 at Richmond, Virginia. Beginning late 2010, we studied branch dieback levels (per cent live crown) and newTCDsymptom development at these two quarantined locations monthly for 3 years. Of the 106 trees studied (53 at each location), 31 trees had low live crown ratings of 70 to 0% with little change over the 3 years of the study. One per cent of the trees developed new symptoms on a per‐year basis. Thus, a moderate level ofTCD(mean = 76% live crown) was present in these two locations, and most trees were in a quiescent or dormantTCDcondition for 3 years, an important finding not previously reported. We found newTCDsymptoms developed in Richmond in 2011 and 2012 when precipitation from January 1 to the end of August was low (60–64 cm), and not when the precipitation in Richmond was higher (99 cm). In late 2012, in Richmond, soil water potential assays indicated that some black walnut trees were under severe physiological stress (−15 bars). In contrast, in 2013, high precipitation levels (99 and 130 cm) and high soil water potentials (−0.1 to −3 bars) at both locations were associated with extensive new foliage and stem growth and recovery fromTCD. Further research is needed on water relationships in regard toTCDand black walnut health.
sec‐Butyl‐propylacetamide (SPD) and two of its stereoisomers rapidly terminate paraoxon‐induced status epilepticus in rats
Authors: Bar‐Klein Guy , Swissa Evyatar , Kamintsky Lyn , Shekh‐Ahmad Tawfeeq , Saar‐Ashkenazy Rotem , Hubary Yechiel , Shrot Shai , Stetlander Liran , Eisenkraft Arik , Friedman Alon , Bialer Meir 
Published: November 2014

SummaryObjectiveOrganophosphates (OPs) are commonly used insecticides for agriculture and domestic purposes, but may also serve as nerve agents. Exposure to OPs result in overstimulation of the cholinergic system and lead to status epilepticus (SE), a life‐threatening condition that is often resistant to treatment. SE is associated with significant neuronal damage, neurocognitive dysfunction, and the development of lifelong epilepsy. Therefore, rapid termination of SE and prevention of brain damage is of high interest. Here we tested the efficacy of sec‐butyl‐propylacetamide (SPD) and two of its individual stereoisomers, (2S,3S)‐SPD and (2R,3R)‐SPD, in discontinuing OP‐induced seizures. SPD is a one carbon homolog of valnoctamide, a central nervous system (CNS)–active constitutional isomer of valproic acid (VPA) corresponding amide valpromide.MethodsRats were implanted with epidural telemetric electrodes to allow electrocorticography (ECoG) recording 24 h prior, during and 24 h after poisoning with the OP paraoxon (at a dose equivalent to 1.4 LD50 Median lethal dose). All rats were provided with antidotal treatment of atropine and toxogonin. Epileptic activity was measured using a novel automated system to evaluate the different effects of midazolam, SPD, and its individual stereoisomers in comparison to nontreated controls.ResultsTreatment with SPD or its individual stereoisomer (2S,3S)‐SPD significantly shorten paraoxon‐induced SE and reduced the duration of recorded pathologic activity after SE was terminated. (2S,3S)‐SPD was superior to racemic‐SPD in diminishing delayed pathologic epileptiform activity within the first 8 h after SE.SignificanceThese results suggest SPD as an efficient drug for the rapid termination of SE and pathological epileptiform activity following OP poisoning, a strategy to reduce neuronal dysfunction and the risk for lifelong epilepsy.
Seizure expression, behavior, and brain morphology differences in colonies of Genetic Absence Epilepsy Rats from Strasbourg
Authors: Powell Kim L. , Tang Howard , Ng Caroline , Guillemain Isabelle , Dieuset Gabriel , Dezsi Gabi , Çarçak Nihan , Onat Filiz , Martin Benoît , O'Brien Terence J. , Depaulis Antoine , Jones Nigel C. 
Published: November 2014

SummaryObjectiveOriginally derived from a Wistar rat strain, a proportion of which displayed spontaneous absence‐type seizures, Genetic Absence Epilepsy Rats from Strasbourg (GAERS) represent the most widely utilized animal model of genetic generalized epilepsy. Here we compare the seizure, behavioral, and brain morphometric characteristics of four main GAERS colonies that are being actively studied internationally: two from Melbourne (MELB and STRAS‐MELB), one from Grenoble (GREN), and one from Istanbul (ISTAN).MethodsElectroencephalography (EEG) recordings, behavioral examinations, and structural magnetic resonance imaging (MRI) studies were conducted on GAERS and Non‐Epileptic Control (NEC) rats to assess and compare the following: (1) characteristics of spike‐and‐wave discharges, (2) anxiety‐like and depressive‐like behaviors, and (3) MRI brain morphology of regions of interest.ResultsSeizure characteristics varied between the colonies, with MELB GAERS exhibiting the least severe epilepsy phenotype with respect to seizure frequency, and GREN GAERS exhibiting four times more seizures than MELB. MELB and STRAS‐MELB colonies both displayed consistent anxiety and depressive‐like behaviors relative to NEC. MELB and GREN GAERS showed similar changes in brain morphology, including increased whole brain volume and increased somatosensory cortical width. A previously identified mutation in the Cacna1h gene controlling the CaV3.2 T‐type calcium channel (R1584P) was present in all four GAERS colonies, but absent in all NEC rats.SignificanceThis study demonstrates differences in epilepsy severity between GAERS colonies that were derived from the same original colony in Strasbourg. This multi‐institute study highlights the potential impact of environmental conditions and/or genetic drift on the severity of epileptic and behavioral phenotypes in rodent models of epilepsy.
Variable electrobehavioral patterns during focal nonconvulsive status epilepticus induced by unilateral intrahippocampal injection of kainic acid
Authors: Arcieri Salvatore , Velotti Rosa , Noè Francesco , Carriero Giovanni , Cattalini Alessandro , Galbardi Barbara , Gnatkovsky Vadym , de Curtis Marco 
Published: November 2014

SummaryObjectiveNonconvulsive status epilepticus (ncSE) is a severe condition that may result in neurologic sequelae and epilepsy resistant to pharmacologic treatment. We analyze here seizure and electroencephalography (EEG) patterns and their correlation to the development of a chronic epileptic condition in a guinea pig model of focal ncSEinduced by intrahippocampal injection of kainic acid (KA).MethodsElectrobehavioral patterns during ncSEinduced by unilateral injection of 1 μg ofKAin theCA1 region of the hippocampus were characterized by continuous video‐EEGmonitoring in 13 guinea pigs bilaterally implanted with recording electrodes in the hippocampus and neocortex.ResultsVideo‐EEGanalysis demonstrates a high variability of seizure type and duration duringKA‐induced ncSE. Seizures showed focal signs correlated with diverse epileptiformEEGdischarge distributions, either diffuse or localized. Nonfocal (bilateral motor) signs during seizures most likely correlated with a diffuseEEGpattern. The evolution into a chronic epileptic condition correlated neither with the severity of seizure pattern nor with the diffusion of theEEGdischarges observed during the ncSE.SignificanceVideo‐EEGmonitoring in a guinea pig model of ncSEinduced by unilateral hippocampal injection ofKAdemonstrates a high variability of electrobehavioral patterns. We demonstrate that the seizure severity score during focal ncSEis not a predictor of the evolution into a chronic epileptic condition of mesial temporal lobe epilepsy.
Intra‐articular treatment with triamcinolone compared with triamcinolone with hyaluronate: A randomised open‐label multicentre clinical trial in 80 lame horses
Authors: de Grauw J. C. , Visser‐Meijer M. C. , Lashley F. , Meeus P. , van Weeren P. R. 
Published: November 2014

SummaryReasons for performing studyIntra‐articular (IA) injection of corticosteroids with or without hyaluronate (HA) has been used for decades in equine practice for treatment of noninfectious synovitis and osteoarthritis. However, to date, no large‐scale randomised equine field trials have been reported that address the supposed superior clinical efficacy of the combination of corticosteroid +HAcompared withIAinjection of corticosteroid alone.ObjectivesTo compare the clinical efficacy ofIAtriamcinolone acetonide (TA, 12 mg) compared withIA TA(12 mg) + high molecular weightHA(20 mg) in horses with clinical joint disease.Study designProspective, randomised, parallel, open label, multicentre clinical trial.MethodsEighty client‐owned horses from 13 clinics were included. Lameness and effusion scores were assessed at baseline and 3 weeks afterIAtreatment. A standardised telephone questionnaire was completed between the owner and consulting veterinarian at 3 months. The primary outcome parameter was clinical success rate, defined as ≥2 grades lameness reduction (on a 0–5 scale) at 3 weeks. Chi‐square statistics and binary logistic regression were used to analyse data on an intention‐to‐treat basis for the 3 week outcome.ResultsThe success rate ofIA TA3 weeks after treatment was 87.8%, while that ofTA+HAwas 64.1% (P = 0.01). Age >13 years was associated with a reduced success rate for the combination treatment (P = 0.004) at 3 weeks. At 3 months, half the horses in each group had returned to their previous level of performance.ConclusionsThe combination of TA with HA was associated with a lower short‐term clinical success rate and a similar medium‐term outcome compared withIATA, with only half of the horses performing at their previous level of exercise after 3 months regardless of treatment group allocation.
Syringin may exert sleep‐potentiating effects through the NOS/NO pathway
Authors: Cui Yue , Zhang Ying , Liu Gang 
Published: November 2014

AbstractSleep is essential for basic survival as well as for optimal physical and cognitive performance in both human beings and animals. To investigate the effect of syringin on sleep of anesthetized mice and the potential mechanisms, 35 male Kunming mice were randomly divided into six experimental groups (n = 5) and one control group (n = 5). Sleep latency and sleep duration, as well as nitric oxide (NO) content and nitric oxide synthase (NOS) activity, were determined after syringin administration. The NO precursor l‐Arginine (l‐Arg) or NOS inhibitor NG‐Nitro‐l‐arginine methyl ester (l‐NAME) was administered alone or in combination with syringin, and time for sleep latency and duration was recorded. After intragastric administration of syringin, sleep latency decreased in a dose‐ and time‐dependent manner, concomitant with increased sleep duration. The optimal sleep performance was obtained when syringin was given at a dose of 80 mg/kg for eight consecutive days. Syringin significantly reduced NO concentration and NOS activity. Administration of l‐Arg prolonged sleep latency and shortened sleep duration, and the effects were fully reversed by syringin coadministration. Administration of L‐NAME induced a significant reduction in sleep latency and a corresponding increase in sleep duration, and coadministration of syringin further enhanced the effects. The finding of our study demonstrated that syringin could exert sleep‐potentiating effects on anesthetized mice in a time‐ and dose‐dependent manner, and these effects may be intimately correlated with the NO/NOS pathway.
The capacity to cope with climate warming declines from temperate to tropical latitudes in two widely distributed Eucalyptus species
Authors: Drake John E. , Aspinwall Michael J. , Pfautsch Sebastian , Rymer Paul D. , Reich Peter B. , Smith Renee A. , Crous Kristine Y. , Tissue David T. , Ghannoum Oula , Tjoelker Mark G. 
Published: November 2014

AbstractAs rapid climate warming creates a mismatch between forest trees and their home environment, the ability of trees to cope with warming depends on their capacity to physiologically adjust to higher temperatures. In widespread species, individual trees in cooler home climates are hypothesized to more successfully acclimate to warming than their counterparts in warmer climates that may approach thermal limits. We tested this prediction with a climate‐shift experiment in widely distributed Eucalyptus tereticornis and E. grandis using provenances originating along a ~2500 km latitudinal transect (15.5–38.0°S) in eastern Australia. We grew 21 provenances in conditions approximating summer temperatures at seed origin and warmed temperatures (+3.5 °C) using a series of climate‐controlled glasshouse bays. The effects of +3.5 °C warming strongly depended on home climate. Cool‐origin provenances responded to warming through an increase in photosynthetic capacity and total leaf area, leading to enhanced growth of 20–60%. Warm‐origin provenances, however, responded to warming through a reduction in photosynthetic capacity and total leaf area, leading to reduced growth of approximately 10%. These results suggest that there is predictable intraspecific variation in the capacity of trees to respond to warming; cool‐origin taxa are likely to benefit from warming, while warm‐origin taxa may be negatively affected.
Effect of Lactobacillus buchneri and Lactobacillus plantarum on the fermentation characteristics and aerobic stability of whipgrass silage in laboratory silos
Authors: Wang Musen , Yang Chunhua , Jia Lujie , Yu Kefei 
Published: November 2014

AbstractThe effect of Lactobacillus buchneri, alone or in combination with Lactobacillus plantarum, on the fermentation characteristics and aerobic stability of whipgrass silage was investigated under laboratory conditions. The inoculants were applied at 2 × 106 colony forming units g−1. Uninoculated silages served as control. After treatment, the chopped materials were ensiled in 1.5‐L anaerobic glass jars. Three jars per treatment were sampled on days 2, 4, 8, 15, 30, 45 and 90. After 90 days of fermentation, the silages were subjected to an aerobic stability test lasting 5 days, in which CO2 production, change in pH and microbial numbers were measured to determine the extent of aerobic deterioration. At the end of the fermentation period 90 days, inoculation with L. buchneri or in combination with L. plantarum significantly resulted in higher levels of acetic acid in the silages than other treatments (P < 0.05), thus the activities of yeasts and molds, especially yeasts, were impaired. The application of L. buchneri, alone or in combination of with L. plantarum, improved the aerobic stability of whipgrass silage as reflected by lower pH, CO2 production and yeast and mold counts during aerobic exposure. Combining L. buchneri with L. plantarum or applying L. plantarum alone improved the fermentation characteristics of whipgrass silage in comparison with the control. The application of L. buchneri alone or in combination with L. plantarum improved the aerobic stability of whipgrass silage under laboratory conditions.
A comparison of fibrinogen measurement using TEG® functional fibrinogen and Clauss in cardiac surgery patients
Authors: Agarwal S. , Johnson R. I. , Shaw M. 
Published: November 2014

SummaryIntroductionViscoelastic methods of estimating fibrinogen measure clot formation under platelet inhibition; these methods are gaining in popularity because of their convenience and speed. The aim of this study was to compare the results of the TEG® functional fibrinogen (FF) assay with the Clauss fibrinogen method in a group of patients presenting for cardiac surgery and to assess whether they gave equivalent results.MethodsAs part of a larger study evaluation a transfusion algorithm for use in cardiac surgery, 320 paired samples of TEG® FF and Clauss fibrinogen were compared. These were taken from 160 patients undergoing cardiac surgery at two time points – prior to heparinisation and 10 min after the administration of protamine. The results of the two fibrinogen measurements were also analysed in relation to the platelet count at the time.ResultsThere was no significant correlation between the TEG® FLEV and Clauss fibrinogen at baseline (R2 = 0.106) or 10 min postprotamine (R2 = 0.025) The TEG® FF maximal amplitude and the Clauss were also found to have no significant correlation (at baseline R2 = 0.061), at 10 min postprotamine (R2 = 0.260). Bland–Altman analysis showed no significant agreement between the two methods.ConclusionThe TEG® FF does not produce results equivalent to those of the Clauss fibrinogen regardless of platelet count.
Influence of Spark Plasma Sintering (SPS) Conditions on Transmission of MgAl2O4 Spinel
Authors: Morita Koji , Kim Byung‐Nam , Yoshida Hidehiro , Hiraga Keijiro , Sakka Yoshio 
Published: November 2014

The effects of the spark plasma sintering (SPS) parameters, such as heating rate α, temperature T and soak time ts, and impurities on in‐line transmission Tin were examined in MgAl2O4 spinel. The SPS processing at T = 1300°C for ts = 20 min with a low heating rate of α = 10°C/min is a preferable condition for attaining higher Tin. For the higher T or the longer ts, grain coarsening enhanced the coalescence of residual pores and second phase precipitation, resulting to the limited Tin even at the slow α. Although the lower T and longer ts attained fine and dense microstructure simultaneously, the maximum Tin was limited to about 50%. The limited transmission, particularly in the visible range, can be ascribed to the discoloration caused by the carbon contamination. The carbon contamination arose from the preexisting trace CO3 impurities, irrespective of the SPS conditions. For the higher α, which is the primary advantage of the SPS technique, the additional carbon contamination occurred from the paper/die and remained as glassy carbon in the matrix. To attain higher Tin by the SPS technique, the lower α and T, and the shorter ts should be utilized after removing the impurities.
A Coated Infiltration Growth Technique for Fabricating Single‐Grain Y–Ba–Cu–O Bulk Superconductor
Authors: Li Guo‐Zheng , Dong Lei , Deng Jian‐Hua , Deng Xiang‐Yun , Yang Wan‐Min 
Published: November 2014

A coated infiltration growth technique was proposed to fabricate single‐grain Y?Ba?Cu?O bulk superconductor, in which a liquid source coated Y2BaCuO5 (Y‐211) preform was employed and the liquid source composition was 3BaO + 5CuO. Experimental results indicated that, the sample exhibited a single‐grain morphology on the top surface, and the liquid source coating always existed surrounding the bulk which contributed to the complete growth of the sample. The homogeneous distribution of fine Y‐211 inclusions in microstructure and a satisfactory Jc performance of 5.67 × 104 A/cm2 in self‐field at 77 K have also been observed.
Computerized comprehension training for whom and under which conditions is it efficient?
Authors: Potocki A. , Ecalle J. , Magnan A. 
Published: November 2014

AbstractThe purpose of this study was to examine the differential impact of a computer‐based comprehension training programme according to second graders' comprehender profiles. These profiles differed in the children's abilities to process three types of textual information: literal information, text‐connecting inferences and gap‐filling inferences. The results showed that all the profiles improved significantly after the training on the question type that had caused them the greatest amount of difficulty on the pretest. In addition, factors that could a priori predict the effectiveness of the programme were investigated by comparing children who benefited from the instruction (responders) with those who did not benefit (nonresponders). Before instruction, these children differed in their lexical knowledge level and logical reasoning skills, but not in their memory or comprehension‐monitoring abilities. The results support the claim that children with comprehension difficulties form a heterogeneous group and emphasize the need to examine the differential impact of comprehension instruction according to the characteristics of poor comprehenders.
Bacteriophage Mixture Inactivation Kinetics against Escherichia Coli O157:H7 on Hard Surfaces
Authors: Viazis Stelios , Labuza Theodore P. , Diez‐Gonzalez Francisco 
Published: November 2014

AbstractThis study determined the effect of previously described phage cocktail (BEC8) on the inactivation kinetics of a mixture of Escherichia coli (EHEC) O157:H7 strains on food processing surface materials: stainless steel chip (SSC), ceramic tile chip (CTC) and high‐density polyethylene chip (HDPEC). EHEC strains were combined and spot‐inoculated on surfaces in dried or liquid form and BEC8 was applied and incubated at 12, 23, 30 and 37C. EHEC survival was determined on tryptic soy agar. D values ranged from 3.9 min at 37C to 46.7 min at 12C. Z values calculated for SSC, CTC and HDEPC resulted in 26.1, 23.7 and 26.7C for dry cells, respectively, and 23.2, 23.7 and 24.5C for liquid cells, respectively. There was a significant difference in the rate of bacterial inactivation between lower (12 and 23C) and higher temperatures (30 and 37C). These results indicated that BEC8 killed EHEC on hard surfaces rapidly following first‐order kinetics.Practical ApplicationsThe effect of a previously described phage cocktail (BEC8) on the inactivation kinetics of a mixture of Escherichia coli (EHEC) O157:H7 strains applied on hard food processing surface materials was studied. The results indicated that phages killed EHEC on hard surfaces relatively rapidly following a predictable rate. However, one must consider the long times to achieve a 5‐log reduction when initial cell counts are high. It would take approximately 2–4 h for a 5‐log cfu reduction of liquid cells at 23C. Both the time length and the temperature of application must fit within the industry time scales.