bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric Diamond‐Blackfan anemia in the Netherlands: An overview of clinical characteristics and underlying molecular defects
Authors: van Dooijeweert Birgit , van Ommen C. Heleen , Smiers Frans J. , Tamminga Rienk Y. J. , te Loo Maroeska W. , Donker Albertine E. , Peters Marjolein , Granzen Bernd , Gille Hans J. J. P. , Bierings Marc B. , MacInnes Alyson W. , Bartels Marije 
Published: November 2017

AbstractIntroductionDiamond‐Blackfan anemia (DBA) is characterized by hypoplastic anemia, congenital anomalies, and a predisposition for malignancies. Most of our understanding of this disorder stems from molecular studies combined with extensive data input from international patient registries.ObjectivesTo create an overview of the pediatric DBA population in the Netherlands. Methods: Forty‐three patients diagnosed with DBA from all Dutch university pediatric hospitals were included in this study, and their clinical and genetic characteristics were collected from patient records.ResultsCongenital malformations were present in 24 of 43 patients (55.8%). An underlying genetic defect was identified in 26 of 43 patients (60.5%), the majority of which were found in the RPS19 gene (12 of 43, 27.9%) with 1 patient carrying a mutation in a novel DBA candidate gene, RPL9. In 31 of 35 (88.6%) patients, an initial response to glucocorticoid treatment was observed. Six patients (14.0%) underwent hematopoietic stem cell transplantation, and eleven patients (11 of 43, 25.6%) became treatment‐independent spontaneously.ConclusionIn agreement with previous reports, the Dutch pediatric DBA population is both clinically and genetically heterogeneous. National and international registries, together with more extensive genetic testing, are crucial to increase our understanding of genotype and phenotype correlations of this intriguing disorder.
Tafamidis delays neurological progression comparably across Val30Met and non‐Val30Met genotypes in transthyretin familial amyloid polyneuropathy
Authors: Gundapaneni B. K. , Sultan M. B. , Keohane D. J. , Schwartz J. H. 
Published: November 2017

Background and purposeTo better characterize the effects of tafamidis in non‐Val30Met patients with transthyretin familial amyloid polyneuropathy, this post hoc analysis compared the neurological results from a 12‐month, open‐label study of non‐Val30Met versus Val30Met patients at month 12 from the 18‐month, double‐blind, placebo‐controlled registration study. A baseline covariate adjusted analysis was used to control for differences in baseline neurological severity.MethodsNeurological function was assessed using the Neuropathy Impairment Score – Lower Limbs (NIS‐LL) in three cohorts: Val30Met tafamidis (n = 64), Val30Met placebo (n = 61) and non‐Val30Met tafamidis (n = 21). The change in NIS‐LL from baseline to month 12 for Val30Met and non‐Val30Met tafamidis‐treated patients was compared with the change from baseline at month 12 for Val30Met placebo‐treated patients using a mixed‐effects model for repeated measures (MMRM).ResultsThe baseline adjusted mean (standard error) change in NIS‐LL values at month 12 was similar for Val30Met [1.60 (0.78)] and non‐Val30Met [1.62 (1.43)] tafamidis‐treated patients and less than that observed in the Val30Met placebo‐treated group [4.72 (0.77); P = 0.0055 for Val30Met and P = 0.0592 for non‐Val30Met]. Based on the MMRM, the magnitude of change in both tafamidis‐treated cohorts was similar across the range of observed baseline NIS‐LL values, and was consistently less than that observed in the Val30Met placebo‐treated group at month 12.ConclusionsThis baseline‐adjusted analysis demonstrated that tafamidis treatment delayed neurological progression comparably in Val30Met and non‐Val30Met patients across a range of baseline NIS‐LL values. Neurological progression in these two genotype groups may be more similar than previously considered.
Intracranial hypertension induced by internal jugular vein stenosis can be resolved by stenting
Authors: Zhou D. , Meng R. , Zhang X. , Guo L. , Li S. , Wu W. , Duan J. , Song H. , Ding Y. , Ji X. 
Published: November 2017

Background and purposeIdiopathic intracranial hypertension (IIH) is characterized by abnormally elevated intracranial pressure (ICP) without identifiable etiology. Recently, however, a subset of patients with presumed IIH have been found with isolated internal jugular vein (IJV) stenosis in the absence of intracranial abnormalities.MethodsFifteen consecutive patients were screened from 46 patients suspected as IIH and were finally confirmed as isolated IJV stenosis. The stenotic IJV was corrected with stenting when the trans‐stenotic mean pressure gradient (∆MPG) was equal to or higher than 5.44 cmH2O. Dynamic magnetic resonance venography, computed tomographic venography and digital subtraction angiography of the IJV, ∆MPG, ICP, Headache Impact Test 6 and the Frisén papilledema grade score before and after stenting were compared.ResultsAll the stenotic IJVs were corrected by stenting. ∆MPG decreased and the abnormal collateral veins disappeared or shrank immediately. Headache, tinnitus, papilledema and ICP were significantly ameliorated at 14 ± 3 days of follow‐up (all P < 0.01). At 12 ± 5.6 months of outpatient follow‐up, headache disappeared in 14 out of 15 patients (93.3%), visual impairments were recovered in 10 of 12 patients (83.3%) and tinnitus resolved in 10 out of 11 patients (90.9%). In 12 out of 15 cases, the Frisén papilledema grade scores declined to 1 (0–2). The stented IJVs in all 15 patients kept to sufficient blood flows on computed tomographic venography follow‐up without stenting‐related adverse events.ConclusionsNon‐thrombotic IJV stenosis may be a potential etiology of IIH. Stenting seems to be a promising option to address the issue of intracranial hypertension from the etiological level, particularly after medical treatment failure.
More extensive white matter hyperintensity is linked with higher risk of remote intracerebral hemorrhage after intravenous thrombolysis
Authors: Chen Y. , Yan S. , Xu M. , Zhong G. , Liebeskind D. S. , Lou M. 
Published: November 2017

Background and purposeRemote intracerebral hemorrhage (rICH) is infrequent after intravenous thrombolysis (IVT) and its mechanism remains poorly understood. We aimed to assess its frequency and possible relationship with the severity of white matter hyperintensity (WMH) in patients with acute ischemic stroke.MethodsWe prospectively analyzed data from consecutive patients with acute ischemic stroke with magnetic resonance imaging and IVT. WMH volume was quantitatively measured. rICH was defined as intracranial hemorrhage that appears in brain regions without visible ischemic changes on 24‐h follow‐up imaging. Unfavorable outcome was defined as a modified Rankin scale score of 3–6 at 3 months. Logistic regression analysis was used to determine the impact of WMH volume on hemorrhage, including rICH and local parenchymal hemorrhage, as well as clinical outcome.ResultsOf a total of 503 patients analyzed, 17 (3.4%) patients developed rICH. Logistic regression analysis indicated that patients with rICH had significantly larger whole‐brain corrected WMH volume (cWMHv) than those without rICH (22.90 vs. 4.42 mL; odds ratio, 1.562/10 mL; 95% confidence interval, 1.215–2.009; P = 0.001). Not only the corrected peri‐ventricular WMH volume (P = 0.001), but also the corrected deep WMH volume (P = 0.013) was associated with the occurrence of rICH. cWMHv was also independently associated with local parenchymal hemorrhage (P = 0.025). rICH was not related to unfavorable outcome (P = 0.323), whereas cWMHv (P = 0.006) was associated with unfavorable outcome.ConclusionAn increased occurrence rate of rICH after IVT is related to more extensive WMH, which may suggest underlying whole‐brain vascular injury in patients with WMH.
Kappa opioid receptors regulate hippocampal synaptic homeostasis and epileptogenesis
Authors: Queenan Bridget N. , Dunn Raymond L. , Santos Victor R. , Feng Yang , Huizenga Megan N. , Hammack Robert J. , Vicini Stefano , Forcelli Patrick A. , Pak Daniel T. S. 
Published: November 2017

SummaryObjectiveHomeostatic synaptic plasticity (HSP) serves as a gain control mechanism at central nervous system (CNS) synapses, including those between the dentate gyrus (DG) and CA3. Improper circuit control of DG‐CA3 synapses is hypothesized to underlie epileptogenesis. Here, we sought to (1) identify compounds that preferentially modulate DG‐CA3 synapses in primary neuronal culture and (2) determine if these compounds would delay or prevent epileptogenesis in vivo.MethodsWe previously developed and validated an in vitro assay to visualize the behavior of DG‐CA3 synapses and predict functional changes. We used this “synapse‐on‐chip” assay (quantification of synapse size, number, and type using immunocytochemical markers) to dissect the mechanisms of HSP at DG‐CA3 synapses. Using chemogenetic constructs and pharmacological agents we determined the signaling cascades necessary for gain control at DG‐CA3 synapses. Finally, we tested the implicated cascades (using kappa opioid receptor (OR) agonists and antagonists) in two models of epileptogenesis: electrical amygdala kindling in the mouse and chemical (pentylenetetrazole) kindling in the rat.ResultsIn vitro, synapses between DG mossy fibers (MFs) and CA3 neurons are the primary homeostatic responders during sustained periods of activity change. Kappa OR signaling is both necessary and sufficient for the homeostatic elaboration of DG‐CA3 synapses, induced by presynaptic DG activity levels. Blocking kappa OR signaling in vivo attenuates the development of seizures in both mouse and rat models of epilepsy.SignificanceThis study elucidates mechanisms by which synapses between DG granule cells and CA3 pyramidal neurons undergo activity‐dependent homeostatic compensation, via OR signaling in vitro. Modulation of kappa OR signaling in vivo alters seizure progression, suggesting that breakdown of homeostatic closed‐loop control at DG‐CA3 synapses contributes to seizures, and that targeting endogenous homeostatic mechanisms at DG‐CA3 synapses may prove useful in combating epileptogenesis.
Evaluating the utility of the Gulf Stream Index for predicting recruitment of Southern New England‐Mid Atlantic yellowtail flounder
Authors: Xu Haikun , Miller Timothy J. , Hameed Sultan , Alade Larry A. , Nye Janet A. 
Published: November 2017

AbstractThe justification for incorporating environmental effects into fisheries stock assessment models has been investigated and debated for a long time. Recently, a state‐space age‐structured assessment model which includes the stochastic change in the environmental covariate over time and its effect on recruitment was developed for Southern New England‐Mid Atlantic yellowtail flounder (Limanda ferruginea). In this paper, we first investigated the correlations of environmental covariates with Southern New England‐Mid Atlantic yellowtail flounder recruitment deviations. The covariate that was most strongly correlated with the recruitment deviations was then incorporated into the state‐space model and alternative effects on the stock‐recruit relationship were estimated and compared. For the model that performed best as measured by Akaike information criterion, we also compared the estimates and predictions of various population attributes and biological reference points with those from an otherwise identical model without the environmental covariate in the stock‐recruit function. We found that the estimates of population parameters are similar for the two models but the predictions differed substantially. To evaluate which model provided more reliable predictions, we quantitatively compared the prediction skill of the two models by generating two series of retrospective predictions. Comparison of the retrospective prediction pattern suggested that from an average point of view, the environmentally explicit model can provide more accurate near‐term recruitment predictions especially the one year ahead recruitment prediction. However, the accuracy of the near‐term recruitment prediction from the environmentally explicit model was largely determined by the accuracy of the corresponding environment prediction the model provides.
Big or Small Cities? On city size and economic growth
Authors: Frick Susanne A. , Rodríguez‐Pose Andrés 
Published: November 2017

AbstractPolicy makers and academics frequently emphasize a positive link between city size and economic growth. The empirical literature on the relationship, however, is scarce and uses rough indicators for the size of a country's cities, while ignoring factors that are increasingly considered to shape this relationship. In this paper, we employ a panel of 113 countries between 1980 and 2010 to explore whether 1) there are certain city sizes that are growth enhancing and 2) how additional factors highlighted in the literature impact the city size/growth relationship. The results suggest a nonlinear relationship which is dependent on the country's size. In contrast to the prevailing view that large cities are growth‐inducing, for a majority of countries relatively small cities of up to 3 million inhabitants are more conducive to economic growth. A large share of the urban population in cities of more than 10 million inhabitants is only growth promoting in countries with an urban population of 28.5 million and more. In addition, the relationship is highly context‐dependent: a high share of industries that benefit from agglomeration economies, a well‐developed urban infrastructure, and an adequate level of governance effectiveness allow countries to take advantage of agglomeration benefits from larger cities.
Risk factors for exacerbation of gastroesophageal varices and portosystemic encephalopathy during treatment with nucleos(t)ide analogs for hepatitis B virus‐related cirrhosis
Authors: Nagaoki Yuko , Aikata Hiroshi , Daijyo Kana , Teraoka Yuji , Honda Fumi , Nakamura Yuki , Hatooka Masahiro , Morio Kei , Fujino Hatsue , Nakahara Takashi , Kawaoka Tomokazu , Miki Daiki , Tsuge Masataka , Hiramatsu Akira , Imamura Michio , Kawakami Yoshiiku , Ochi Hidenori , Chayama Kazuaki 
Published: November 2017

AimThe aim of this study was to determine the risk factors for worsening of gastroesophageal varices (GEVs) and development of portosystemic encephalopathy in patients with hepatitis B virus (HBV)‐related cirrhosis during nucleos(t)ide analog (NA) treatment.MethodsOne hundred and thirty‐seven patients with HBV‐related cirrhosis were enrolled in this retrospective cohort study. Findings of portal hemodynamics with computed tomography, liver function, and endoscopic examinations during NA treatment were assessed.ResultsAmong 137 patients, feeding vessels for GEVs (left gastric vein, posterior gastric vein, and short gastric vein) were present in 56 (41%) patients, and extrahepatic portosystemic shunt (paraesophageal vein, paraumbilicul vein, and splenorenal shunt) were present in 36 (26%) patients at the start of NA treatment. Although NA treatment was successful, significant improvements were not observed in portosystemic collateral vessels 3 years after NA treatment and GEVs were exacerbated in 48 (35%) patients. The cumulative 5‐ and 10‐year exacerbation rate of GEVs was 27% and 50%, respectively. By multivariate analysis, the existence of feeding vessels for GEVs at the start of NA treatment was the independent predictive factor for the exacerbation of GEVs (P < 0.001). Eight patients who had extrahepatic portosystemic shunt at the start of NA treatment developed portosystemic encephalopathy during follow‐up. The 3‐ and 5‐year incidence of that was 5% and 8%, respectively.ConclusionsThe presence of portosystemic collateral vessels at the start of NA treatment increases the risk of GEVs worsening and development of portosystemic encephalopathy in patients with HBV‐related cirrhosis, despite improvement of liver function and success in reducing viral loads with NA treatment.
Commercial alkaline earth boroaluminosilicate glasses for sealing solid oxide cell stacks. Part I: Development of glass‐ceramic microstructure and thermomechanical properties
Authors: Agersted Karsten , Balic‐Zunic Tonci 
Published: November 2017

AbstractSealing performance in solid oxide cell (SOC) stacks and the devitrification process of commercially available alkaline earth boroaluminosilicate glasses containing 48‐61 mol% SiO2, 18‐28 mol% CaO, 1‐7 mol% MgO, 7‐10 mol% Al2O3, 1‐11 mol% B2O3 plus minor amounts of Na2O, K2O, FeO, and TiO2 were investigated and quantified through analysis of phase assemblages as function of heat treatments above the glass transition temperatures using the electron microprobe and powder X‐ray diffraction. For two of these glasses devitrification behavior was compared to the devitrification behavior of similar glasses produced in the laboratory. Glasses were characterized after annealing in air at 800°C and 850°C for up to 6 weeks. Even though the glasses lie within a relatively narrow compositional range, sealing performance and the resulting microstructures differed significantly. Best thermomechanical properties was developed in one of the laboratory‐produced glasses, MCAS, which may be applied in SOC‐stacks by allowing for a slow solidification in the range 750‐800°C followed by crystallization at or slightly above 800°C. The relatively high thermal expansion coefficient (CTE) from RT‐800°C, 11 × 10−6 K−1, which was developed over ~1000 hours at 800°C, depends mainly on the formation of cristobalite and quartz as well as the presence of a residual glass phase. The glass ceramic sealant appears relatively stable over time, except for a slow transition of cristobalite to quartz, and can possibly show self‐healing behavior if later brought close to 850°C. Devitrification led to increases of the thermal expansion coefficients in all other glasses tested, but did not reach levels interesting for SOC‐stack sealing.
Synthesis of modified catalyst and stabilization of CuO/NH4‐ZSM‐5 for conversion of methanol to gasoline
Authors: Kianfar Ehsan , Salimi Mahmoud , Pirouzfar Vahid , Koohestani Behnam 
Published: November 2017

AbstractIn this article, the catalytic conversion of methanol to gasoline range hydrocarbons has been studied over CuO/ NH4‐ZSM‐5(3,5,7,9%) catalysts prepared via sono‐chemistry methods. In order to improve, copper oxide can be used as a booster on NH4‐ZSM‐5 this catalyst property. Accordingly, the conversion process of Methanol to Gasoline (MTG) was conducted under a pressure of 1 atm and temperature of 400°C by a fixed‐bed reactor on copper oxide catalysts which were prepared based on synthetic NH4‐ZSM‐5. The synthetic catalyst was investigated by such analyses as BET, XRD, FT‐IR, and SEM. Formation of copper oxide phase and proper distribution of copper oxide were proven on the basic level of using XRD analysis. BET analysis showed the reduction in catalyst level and SEM images depicted the proper distribution of particles. The present investigation is to study the effect of CuO loading on NH4‐ZSM‐5 support for conversion of methanol to gasoline range hydrocarbons. A series of CuO/ NH4‐ZSM‐5 catalysts were prepared, characterized, and experimented for their performance on methanol conversion and hydrocarbon yield.
Relationship and variation of diabetes related symptoms, sleep disturbance and sleep‐related impairment in adults with type 2 diabetes
Authors: Zhu Bingqian , Quinn Laurie , Fritschi Cynthia 
Published: November 2017

AbstractAimThe objective of this study was to examine whether diabetes‐related symptoms (e.g. fatigue, neuropathic pain, diabetes distress and depressive symptoms) were related to sleep disturbance and sleep‐related impairment in adults with type 2 diabetes while controlling for potential covariates.BackgroundIn people with type 2 diabetes, sleep disturbance and sleep‐related impairment are common and likely associated with diabetes‐related symptoms. However, limited research has investigated the predictive ability of diabetes‐related symptoms on sleep.DesignA correlational, cross‐sectional design was used.MethodsData were collected at a large university in the Midwestern United States from September 2013–March 2014. Multiple linear regression analyses were used to examine the relationship of diabetes‐related symptoms (fatigue, neuropathic pain, distress and depressive symptoms) to sleep disturbance and sleep‐related impairment. The instruments included Patient‐Reported Outcomes Measurement Information System instruments, Diabetes Symptom Checklist and Diabetes Distress Scale.FindingsIn this study of adults with type 2 diabetes (N = 90; 52.2% female, mean age 57.4 years), gender, A1C, neuropathic pain and fatigue were significantly related to sleep disturbance when age, diabetes duration, depressive symptoms and distress were controlled. Those variables collectively explained 52% of the variation in sleep disturbance. Fatigue was significantly associated with sleep‐related impairment when the same covariates were controlled.ConclusionFindings suggested that diabetes‐related symptoms, including neuropathic pain and fatigue, are strongly related to sleep disturbance and sleep‐related impairment in adults with type 2 diabetes, underscoring the need to include detailed assessments of neuropathic pain and fatigue when evaluating sleep.
On culture, ethics, and hierarchy: How cultural variations in hierarchical relations are manifested in the code of ethics of British and Korean organizations
Authors: Moon Chanki , Uskul Ayse K. , Weick Mario 
Published: November 2017

AbstractThe present research examined if cultural differences in the extent to which hierarchical relations dictate individuals' behaviors are embedded in objective institutional regulations. Using quantitative and qualitative analysis, we examined codes of ethics of Korean and British organizations in relation to working relationships and corruptive behaviors. We found that, unlike British organizations, Korean organizations endorsed codes of ethics that place greater emphasis on hierarchical relations and contained prescriptions for individuals occupying senior or junior ranks. Ethical codes also appeared to be geared more towards preventing the abuse of power in Korean organizations compared with British organizations. Finally, unlike British organizations, Korean organizations often permitted top‐down exchanges (not bottom‐up exchanges), suggesting that in upper echelons benevolence may be more normative in Korean organizations than in British organizations.