bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pre-emptive rituximab in focal and segmental glomerulosclerosis patients at risk of recurrence after kidney transplantation
Authors: Auñón Pilar , Polanco Natalia , Pérez-Sáez María José , Rodrigo Emilio , Sancho Asunción , Pascual Julio , Andrés Amado , Praga Manuel 
Published: August 2019

AbstractBackgroundThe recurrence of proteinuria after kidney transplantation (KT) is a characteristic complication of focal segmental glomerulosclerosis (FSGS). It has been suggested that pre-emptive rituximab might prevent recurrences in patients at risk, but there is no agreement about which factors might help to identify such patients.MethodsWe studied 93 kidney transplants with biopsy-proven idiopathic FSGS in order to analyse if preventive rituximab treatment could decrease recurrences in patients at risk.ResultsFifteen patients (16.1%) presented a recurrence after KT, but when we restricted the analysis to the 34 patients presenting nephrotic syndrome at primary disease onset, the recurrence diagnosis rate increased to 44.1%. All patients with recurrence had complete nephrotic syndrome at the time of diagnosis. After multivariate adjustment, the only significant risk factor for recurrence was the presence of complete nephrotic syndrome at diagnosis. Twelve of the 34 patients at risk for recurrence received rituximab at the time of transplantation. Clinical and analytical characteristics were similar in all patients at risk. The number of recurrences was similar among treated (50%) and non-treated patients (40.9%).ConclusionsNephrotic syndrome with hypoalbuminaemia at diagnosis is the most important feature to identify patients at risk of recurrence. Our data suggest that pre-emptive rituximab is not effective to prevent FSGS recurrences.
The Transnational Counter-Terrorism Order: A Problématique
Authors: de Londras Fiona 
Published: August 2019

Abstract
We live our lives in an often-unseen transnational counter-terrorism order. For almost two decades now, counter-terrorist hegemons have been acting on multiple transnational levels, using a mixture of legal, institutional, technical and political manoeuvres to develop laws, policies and practices of counter-terrorism that undervalue rights, exclude civil society, limit dissent and disagreement, and expand greatly the reach of national and transnational security. The assemblage of laws, institutions, forums, processes, bureaucracies, and cooperative networks that have emerged from these machinations should be understood as a transnational counter-terrorism order that is intended to instantiate on a global level ‘an arrangement of social life…[that]…promotes certain goals or values’ (Bull), whether or not they conflict with rights, whether or not they emerge from legitimate and participatory processes. This paper brings together various seemingly-technical or esoteric strands of law, institutions, policy and politics to show their connections, interdependencies and interactions and, thereby, to illustrate the emergence of this transnational counter-terrorism order. It argues that unless we recognise the connections between and multi-scalar implications of the seemingly disparate, sometimes opaque, and often bureaucratic elements that make up the transnational counter-terrorism order, its scale and implications will remain hidden in plain sight and we may find ourselves unable effectively to insist on fidelity to the constitutionalist values of rights, accountability, and democratic legitimacy.
Recoarctation of the aorta after the Norwood procedure may be treated during the second stage of the surgical palliation
Authors: Mroczek Tomasz , Czerżyńska Magdalena , Sacharczuk Julita , Żurek Rafał , Wójcik Elżbieta , Morka Aleksandra , Kuźma Jacek , Skalski Janusz H 
Published: August 2019

Abstract

OBJECTIVES
Recoarctation of the aorta (re-CoA) after the Norwood procedure is traditionally treated during catheter-based aortoplasty (CB-A) performed as a separate procedure preceding stage II surgical palliation (S II SP). Our goal was to determine the efficacy of the protocol according to which re-CoA after the Norwood procedure in patients with hypoplastic left heart syndrome is treated during S II SP using hybrid catheter-based aortoplasty.

METHODS
We compared 2 groups of infants who developed re-CoA after the Norwood procedure and were treated at the same institution: In group I (n = 18), CB-A was traditionally performed before S II SP; in group II (n = 15), CB-A was performed during S II SP using a hybrid procedure (catheter access was through an aortic cannula routinely used for cardiopulmonary bypass). The right ventricular fractional area change was analysed.

RESULTS
The CB-A was performed effectively in both groups. S II SP was performed at a younger age in group II (5.4 ± 0.3 vs 6.0 ± 0.4 months; P = 0.003), with lower body weight (5.6 ± 0.5 vs 6.0 ± 0.4; P = 0.03, respectively). The duration of hospital stay did not differ between the groups (10.6 ± 6.2 vs 11.6 ± 6.4 days; P = 0.91). The right ventricular fractional area change measured before S II SP was higher in group I (39.7 ± 4.2% vs 36.8 ± 3.6%, respectively; P = 0.009), but the difference was not seen 1 month after S II SP (41.0 ± 5.6 vs 39.8 ± 4.1; P > 0.05). The total radiation dose was significantly lower in group II.

CONCLUSIONS
re-CoA after the Norwood procedure in patients with hypoplastic left heart syndrome can be treated effectively during S II SP using a hybrid procedure. The strategy allows for reduction of the total radiation dose and of the number of procedures and does not prolong the postoperative course, even in patients with decreased right ventricular systolic function.
Coursebooks: Is there more than meets the eye?
Authors: Hughes Stacey Holliday 
Published: August 2019

Abstract
This article responds to Jordan and Gray’s critique of the global coursebook and the call to have open, critical discussions about coursebook use in light of SLA research (Jordan and Gray 2019). I make a case for why the assumptions made about what constitutes a global coursebook are too narrow. I also dismiss the idea of one type of global coursebook and that the two coursebooks mentioned as examples of a typical global coursebook follow a wholly synthetic syllabus with an explicit instruction model. Most modern global coursebooks, I posit, have both synthetic and analytic characteristics. I point to research which gives evidence in support of the benefit of explicit instruction within the context of communicative language practice. In defence of publishers, I present an alternative viewpoint showing that major publishers do not simply publish coursebooks with little regard to educational research in pursuit of profit. I end by addressing the issue of why a coursebook—or course package—can be a valuable set of resources for teachers.
A proof of principle study of atomoxetine for the prevention of vasovagal syncope: the Prevention of Syncope Trial VI
Authors: Sheldon Robert S , Lei Lucy , Guzman Juan C , Kus Teresa , Ayala-Paredes Felix A , Angihan Jesse , Safdar Shahana , Maxey Connor , Bennett Graham , Raj Satish R 
Published: August 2019

Abstract

Aims
There are few effective therapies for vasovagal syncope (VVS). Pharmacological norepinephrine transporter (NET) inhibition increases sympathetic tone and decreases tilt-induced syncope in healthy subjects. Atomoxetine is a potent and highly selective NET inhibitor. We tested the hypothesis that atomoxetine prevents tilt-induced syncope.

Methods and results
Vasovagal syncope patients were given two doses of study drug [randomized to atomoxetine 40 mg (n = 27) or matched placebo (n = 29)] 12 h apart, followed by a 60-min drug-free head-up tilt table test. Beat-to-beat heart rate (HR), blood pressure (BP), and cardiac haemodynamics were recorded using non-invasive techniques and stroke volume modelling. Patients were 35 ± 14 years (73% female) with medians of 12 lifetime and 3 prior year faints. Fewer subjects fainted with atomoxetine than with placebo [10/29 vs. 19/27; P = 0.003; risk ratio 0.49 (confidence interval 0.28–0.86)], but equal numbers of patients developed presyncope or syncope (23/29 vs. 21/27). Of patients who developed only presyncope, 87% (13/15) had received atomoxetine. Patients with syncope had lower nadir mean arterial pressure than subjects with only presyncope (39 ± 18 vs. 69 ± 18 mmHg, P < 0.0001), and this was due to lower trough HRs in subjects with syncope (67 ± 30 vs. 103 ± 32 b.p.m., P = 0.006) and insignificantly lower cardiac index (2.20 ± 1.36 vs. 2.84 ± 1.05 L/min/m2, P = 0.075). There were no significant differences in stroke volume index (32 ± 6 vs. 35 ± 5 mL/m2, P = 0.29) or systemic vascular resistance index (2156 ± 602 vs. 1790 ± 793 dynes*s/cm5*m2, P = 0.72).

Conclusion
Norepinephrine transporter inhibition significantly decreased the risk of tilt-induced syncope in VVS subjects, mainly by blunting reflex bradycardia, thereby preventing final falls in cardiac index and BP.
Impact of overweightness and critical weight loss on overall survival in patients with hepatocellular carcinoma initially treated with chemoembolization
Authors: Chen Zhen-Xin , Jian Zhi-Wei , Wu Xi-Wen , Wang Jun-Cheng , Peng Jing-Yuan , Huang Chun-Yu , Lao Xiang-Ming 
Published: August 2019

AbstractBackgroundThe effects of overweightness and weight loss on the development and prognosis of hepatocellular carcinoma (HCC) remain unclear. In this study, we aimed to evaluate the impact of overweightness and weight loss on the survival of patients with intermediate/advanced HCC receiving chemoembolization as initial treatment.MethodsWe examined 1,170 patients who underwent chemoembolization as initial treatment for Barcelona-Clínic Liver Cancer stages B and C HCC at Sun Yat-sen University Cancer Center (Guangzhou, China) between December 2009 and May 2015. A baseline body mass index (BMI) of ≥23 kg/m2 was defined as overweight, and body-weight loss of ≥5.0% from baseline was defined as critical weight loss (CWL). Cox regression analysis was used to determine the association between overweightness or CWL and overall survival (OS).ResultsThe median survival time was 16.8 (95% confidence interval, 13.9–19.7) months and 11.1 (95% confidence interval, 10.0–12.2) months in the overweight and non-overweight groups (log-rank test, P &lt; 0.001), respectively. Cox multivariate analysis identified overweightness as an independent protective prognostic factor for OS (P &lt; 0.001). Subgroup stratification analysis revealed a significant association between overweightness and survival among patients receiving further treatment (P = 0.005), but not in those not receiving further treatment (P = 0.683). Multivariate analysis showed that both overweightness and CWL were independent prognostic factors for OS among patients receiving further treatment.ConclusionAmong patients with intermediate- or advanced-stage HCC initially treated with chemoembolization, overweightness was associated with longer OS. Furthermore, CWL was an independent adverse prognostic factor for OS in patients receiving additional treatment.
Pharmacological treatment of hepatorenal syndrome: a network meta-analysis
Authors: Wang Li , Long Yin , Li Ke-Xin , Xu Gao-Si 
Published: August 2019

Abstract

Background
Observational studies suggest that hepatorenal syndrome (HRS) patients who receive pharmacological therapy before orthotopic liver transplantation display a post-transplant outcome similar to those without HRS. The aim of this study was to comprehensively compare and rank the pharmacological therapies for HRS.

Methods
We reviewed PubMed, Elsevier, Medline, and the Cochrane Central Register of Controlled Trials (CENTRAL) for studies that were published between 1 January 1999 and 24 February 2018. The primary endpoint was reversal of HRS. The secondary endpoints were the changes in serum creatinine (Scr) and serum sodium. We evaluated the different therapeutic strategies using network meta-analysis on the basis of Bayesian methodology.

Results
The study included 24 articles with 1,419 participants evaluating seven different therapeutic strategies for HRS. The most effective treatments to induce reversal of HRS were terlipressin plus albumin, noradrenaline plus albumin, and terlipressin, which had a surface under the cumulative ranking curve (SUCRA) of 0.086, 0.151, and 0.451, respectively. The top two treatments for decreasing Scr were dopamine plus furosemide plus albumin (rank probability: 0.620) and terlipressin plus albumin (rank probability: 0.570). For increasing serum sodium, the optimal treatment was octreotide plus midodrine plus albumin (rank probability: 0.800), followed by terlipressin plus albumin (rank probability: 0.544).

Conclusions
Terlipressin plus albumin and dopamine plus furosemide plus albumin should be prioritized for decreasing Scr in HRS, and octreotide plus midodrine plus albumin was the most effective at increasing serum sodium. Terlipressin plus albumin showed a comprehensive effect in both decreasing Scr and increasing serum sodium.
Architecture of Parallel Adaptation in Ten Lacustrine Threespine Stickleback Populations from the White Sea Area
Authors: Terekhanova Nadezhda V , Barmintseva Anna E , Kondrashov Alexey S , Bazykin Georgii A , Mugue Nikolai S 
Published: August 2019

AbstractAdaptation of threespine stickleback to freshwater involves parallel recruitment of freshwater alleles in clusters of closely linked sites, or divergence islands (DIs). However, it remains unclear to what extent the DIs and the alleles that constitute them coincide between populations that underwent adaptation to freshwater independently. We examine threespine sticklebacks from ten freshwater lakes that emerged 500–1500 years ago in the White Sea basin, with the emphasis on repeatability of genomic patterns of adaptation among the lake populations and the role of local recombination rate in the distribution and structure of DIs. The 65 detected DIs are clustered in the genome, forming 12 aggregations, and this clustering cannot be explained by the variation of the recombination rate. Only 21 of the DIs are present in all the freshwater populations, likely being indispensable for successful colonization of freshwater environment by the ancestral marine population. Within most DIs, the same set of single nucleotide polymorphisms (SNPs) distinguish marine and freshwater haplotypes in all the lake populations; however, in some DIs, freshwater alleles differ between populations, suggesting that they could have been established by recruitment of different haplotypes in different populations.
Phylogenomics Reveals Clear Cases of Misclassification and Genus-Wide Phylogenetic Markers for Acinetobacter
Authors: Mateo-Estrada Valeria , Graña-Miraglia Lucía , López-Leal Gamaliel , Castillo-Ramírez Santiago 
Published: August 2019

AbstractThe Gram-negative Acinetobacter genus has several species of clear medical relevance. Many fully sequenced genomes belonging to the genus have been published in recent years; however, there has not been a recent attempt to infer the evolutionary history of Acinetobacter with that vast amount of information. Here, through a phylogenomic approach, we established the most up-to-date view of the evolutionary relationships within this genus and highlighted several cases of poor classification, especially for the very closely related species within the Acinetobacter calcoaceticus–Acinetobacter baumannii complex (Acb complex). Furthermore, we determined appropriate phylogenetic markers for this genus and showed that concatenation of the top 13 gives a very decent reflection of the evolutionary relationships for the genus Acinetobacter. The intersection between our top markers and previously defined universal markers is very small. In general, our study shows that, although there seems to be hardly any universal markers, bespoke phylogenomic approaches can be used to infer the phylogeny of different bacterial genera. We expect that ad hoc phylogenomic approaches will be the standard in the years to come and will provide enough information to resolve intricate evolutionary relationships like those observed in the Acb complex.
Post-critical SsPmp and its applications to Virtual Deep Seismic Sounding (VDSS) – 2: 1-D imaging of the crust/mantle and joint constraints with receiver functions
Authors: Liu Tianze , Klemperer Simon L , Ferragut Gabriel , Yu Chunquan 
Published: August 2019

SUMMARY
Virtual Deep Seismic Sounding (VDSS) has emerged as a novel method to image the crust–mantle boundary (CMB) and potentially other lithospheric boundaries. In Part 1, we showed that the arrival time and waveform of post-critical SsPmp, the post-critical reflection phase at the CMB used in VDSS, is sensitive to several different attributes of the crust and upper mantle. Here, we synthesize our methodology of deriving Moho depth, average crustal Vp and uppermost-mantle Vp from single-station observations of post-critical SsPmp under a 1-D assumption. We first verify our method with synthetics and then substantiate it with a case study using the Yellowknife and POLARIS arrays in the Slave Craton, Canada. We show good agreement of crustal and upper-mantle properties derived with VDSS with those given by previous active-source experiments and our own P receiver functions (PRF) in our study area. Finally, we propose a PRF-VDSS joint analysis method to constrain average crustal Vp/Vs ratio and composition. Our PRF-VDSS joint analysis shows that the southwest Slave Craton has an intermediate crustal composition, most consistent with a Mesoarchean age.
Generalized earthquake frequency–magnitude distribution described by asymmetric Laplace mixture modelling
Authors: Mignan A 
Published: August 2019

SUMMARYThe complete part of the earthquake frequency–magnitude distribution, above the completeness magnitude mc, is well described by the Gutenberg–Richter law. On the other hand, incomplete data does not follow any specific law, since the shape of the frequency–magnitude distribution below max(mc) is function of mc heterogeneities that depend on the seismic network spatiotemporal configuration. This paper attempts to solve this problem by presenting an asymmetric Laplace mixture model, defined as the weighted sum of Laplace (or double exponential) distribution components of constant mc, where the inverse scale parameter of the exponential function is the detection parameter κ below mc, and the Gutenberg–Richter β-value above mc. Using a variant of the Expectation-Maximization algorithm, the mixture model confirms the ontology proposed by Mignan [2012, https://doi.org/10.1029/2012JB009347], which states that the shape of the earthquake frequency–magnitude distribution shifts from angular (in log-linear space) in a homogeneous space–time volume of constant mc to rounded in a heterogeneous volume corresponding to the union of smaller homogeneous volumes. The performance of the proposed mixture model is analysed, with encouraging results obtained in simulations and in eight real earthquake catalogues that represent different seismic network spatial configurations. We find that k = κ/ln(10) ≈ 3 in most earthquake catalogues (compared to b = β/ln(10) ≈ 1), suggesting a common detection capability of different seismic networks. Although simpler algorithms may be preferred on pragmatic grounds to estimate mc and the b-value, other methods so far fail to model the angular distributions observed in homogeneous space-time volumes. Mixture modelling is a promising strategy to model the full earthquake magnitude range, hence potentially increasing seismicity data availability tenfold, since ca. 90 per cent of earthquake catalogue events are below max(mc).
Spp1 (osteopontin) promotes TGFβ processing in fibroblasts of dystrophin-deficient muscles through matrix metalloproteinases
Authors: Kramerova Irina , Kumagai-Cresse Chino , Ermolova Natalia , Mokhonova Ekaterina , Marinov Masha , Capote Joana , Becerra Diana , Quattrocelli Mattia , Crosbie Rachelle H , Welch Ellen , McNally Elizabeth M , Spencer Melissa J 
Published: August 2019

AbstractDuchenne muscular dystrophy (DMD) is caused by mutations in the gene encoding dystrophin. Prior work has shown that DMD progression can vary, depending on the genetic makeup of the patient. Several modifier alleles have been identified including LTBP4 and SPP1. We previously showed that Spp1 exacerbates the DMD phenotype in the mdx mouse model by promoting fibrosis and by skewing macrophage polarization. Here, we studied the mechanisms involved in Spp1's promotion of fibrosis by using both isolated fibroblasts and genetically modified mice. We found that Spp1 upregulates collagen expression in mdx fibroblasts by enhancing TGFβ signaling. Spp1's effects on TGFβ signaling are through induction of MMP9 expression. MMP9 is a protease that can release active TGFβ ligand from its latent complex. In support for activation of this pathway in our model, we showed that treatment of mdx fibroblasts with MMP9 inhibitor led to accumulation of the TGFβ latent complex, decreased levels of active TGFβ and reduced collagen expression. Correspondingly, we found reduced active TGFβ in Spp1−/−mdxB10 and Mmp9−/−mdxB10 muscles in vivo. Taken together with previous observations of reduced fibrosis in both models, these data suggest that Spp1 acts upstream of TGFβ to promote fibrosis in mdx muscles. We found that in the context of constitutively upregulated TGFβ signaling (such as in the mdxD2 model), ablation of Spp1 has very little effect on fibrosis. Finally, we performed proof-of-concept studies showing that postnatal pharmacological inhibition of Spp1 reduces fibrosis and improves muscle function in mdx mice.
Molecular genetic investigations identify new clinical phenotypes associated with BCS1L-related mitochondrial disease
Authors: Oláhová Monika , Berti Camilla Ceccatelli , Collier Jack J , Alston Charlotte L , Jameson Elisabeth , Jones Simon A , Edwards Noel , He Langping , Chinnery Patrick F , Horvath Rita , Goffrini Paola , Taylor Robert W , Sayer John A 
Published: August 2019

Abstract
BCS1L encodes a homolog of the Saccharomyces cerevisiae bcs1 protein, which has a known role in the assembly of Complex III of the mitochondrial respiratory chain. Phenotypes reported in association with pathogenic BCS1L variants include growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis and early death (GRACILE syndrome), and Björnstad syndrome, characterized by abnormal flattening and twisting of hair shafts (pili torti) and hearing problems. Here we describe two patients harbouring biallelic variants in BCS1L; the first with a heterozygous variant c.166C&gt;T, p.(Arg56*) together with a novel heterozygous variant c.205C&gt;T, p.(Arg69Cys) and a second patient with a novel homozygous c.325C&gt;T, p.(Arg109Trp) variant. The two patients presented with different phenotypes; the first patient presented as an adult with aminoaciduria, seizures, bilateral sensorineural deafness and learning difficulties. The second patient was an infant who presented with a classical GRACILE syndrome leading to death at 4 months of age. A decrease in BCS1L protein levels was seen in both patients, and biochemical analysis of Complex III revealed normal respiratory chain enzyme activities in the muscle of both patients. A decrease in Complex III assembly was detected in the adult patient’s muscle, whilst the paediatric patient displayed a combined mitochondrial respiratory chain defect in cultured fibroblasts. Yeast complementation studies indicate that the two missense variants, c.205C&gt;T, p.(Arg69Cys) and c.325C&gt;T, p.(Arg109Trp), impair the respiratory capacity of the cell. Together, these data support the pathogenicity of the novel BCS1L variants identified in our patients.