bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment strategies for lysosomal storage disorders
Authors: Beck Michael 
Published: November 2017

Over the past several years the number of treatments available for patients with lysosomal storage disorders has rapidly increased. Haematopoietic stem cell transplantation, enzyme replacement therapy, substrate reduction, and chaperone therapies are currently available, and gene therapies and other treatments are rapidly advancing. Despite remarkable advances, the efficacy of most of these therapies is limited, particularly because the treatments are usually initiated when organ damage has already occurred. To circumvent this limitation, screening in newborn infants for lysosomal storage disorders has been introduced in many countries. However, this screening is complicated by the broad clinical variability of the disorders and the fact that many individuals who will be detected as having an enzyme deficiency will develop symptoms very late or never in their life. This paper provides an overview of available therapies for lysosomal storage disorders and describes those treatments that are under development.What this paper adds

For a few lysosomal storage disorders, new therapies are available or under development.

These therapies include enzyme replacement therapy, small molecules, and gene therapy.

The new therapies cannot cure patients, but can stabilize organ function or slow progression.
Nesting aggregation as a predictor of brood parasitism in mason bees (Osmia spp.)
Authors: GROULX ADAM F. , FORREST JESSICA R. K. 
Published: November 2017

1. Parasitism can be an important source of mortality for insect populations; however, we know little about the factors influencing vulnerability of wild bees to parasites. Mason bees (genus Osmia; Hymenoptera: Megachilidae) are important pollinators of crops and wild plants and are vulnerable to attack by brood parasites. High nest densities may increase rates of brood parasitism by attracting disproportionate numbers of parasites.2. Three years of field observations from multiple sites were analysed to assess whether mason bee brood parasitism increased with host density. Mason bees were allowed to nest in artificial nesting blocks and establish natural variation in nesting density. Nest cells constructed by bees were checked for the presence of parasite eggs.3. Parasitism of nest cells strongly increased with the number of actively nesting bees at a nesting block. Mason bees showed no preference for nesting in blocks that were occupied or unoccupied by other mason bees. Parasitism also increased with the number of days a nest was provisioned and decreased over the course of the season. Nest cells constructed last in a nest were significantly more parasitised than inner cells, despite being sealed against invasions.4. These findings show positively density‐dependent parasitism in mason bees. They also suggest that bees terminate parasitised nests, causing parasitised cells to become outermost nest cells – a behaviour that may represent a defence against parasites. Our results have implications for the management of mason bees as agricultural pollinators, as cultivating them at high densities could reduce offspring survival.
A review of Dynamic Data Envelopment Analysis: state of the art and applications
Authors: Mariz Fernanda B.A.R. , Almeida Mariana R. , Aloise Daniel 
Published: November 2017

AbstractThis article reports the evolution of the literature on Dynamic Data Envelopment Analysis (DDEA) models from 1996 to 2016. Systematic searches in the databases Scopus and Web of Science were performed to outline the state of the art. The results enabled the establishment of DDEA studies as the scope of this article, analyzing the transition elements to represent temporal interdependence. The categorization of these studies enabled the mapping of the evolution of the DDEA literature and identification of the relationships between models. The three most widely adopted studies to conduct DDEA research were classified as structuring models. Mapping elucidated the literature behavior through three phases and showed an increase in publications with applications in recent years. The analysis of applications indicated that most studies address evaluations in the agriculture and farming, banking and energy sectors and consider the facilities as transition elements between analysis periods.
Ranking corporate sustainability: a flexible multidimensional approach based on linguistic variables
Authors: Liern V. , Pérez‐Gladish B. 
Published: November 2017

AbstractCorporate sustainability implies a compromise between the present environmental, social, and economic needs of a firm's stakeholders and their future needs. Corporate sustainability is therefore a multidimensional concept. Nowadays, several independent rating agencies rate firms in terms of environmental, social, and governance (ESG) criteria. These ratings are usually used by main sustainability indices such as the Dow Jones Sustainability Index, FTSE4 Good, Stoxx Sustainability Index, or Euronext Vigeo Family to select companies to invest in. Only those firms performing better than the average of their sector are selected. However, although providing linguistic ratings about the performance of the firms in individual ESG criteria with respect to their sector, rating agencies do not usually provide overall ESG rates describing the global performance of the firms in terms of ESG. In this paper, we propose a flexible operator, linguistic ordered weighted geometric aggregating operator (LOWGA), which will allow us to define the fuzzy ESG performance of the firms based on the linguistic labels provided by the rating agencies. Once overall ESG scores have been obtained, we will use them together with financial criteria to rank the firms in terms of their sustainability using a suitable multiple criteria decision aid (MCDA) approach, namely, TOPSIS (technique for order preference by similarity to ideal solution).
Annual Research Review: Suicide among youth – epidemiology, (potential) etiology, and treatment
Authors: Cha Christine B. , Franz Peter J. , M. Guzmán Eleonora , Glenn Catherine R. , Kleiman Evan M. , Nock Matthew K. 
Published: November 2017

BackgroundSuicide is a leading cause of death and a complex clinical outcome. Here, we summarize the current state of research pertaining to suicidal thoughts and behaviors in youth. We review their definitions/measurement and phenomenology, epidemiology, potential etiological mechanisms, and psychological treatment and prevention efforts.ResultsWe identify key patterns and gaps in knowledge that should guide future work. Regarding epidemiology, the prevalence of suicidal thoughts and behaviors among youth varies across countries and sociodemographic populations. Despite this, studies are rarely conducted cross‐nationally and do not uniformly account for high‐risk populations. Regarding etiology, the majority of risk factors have been identified within the realm of environmental and psychological factors (notably negative affect‐related processes), and most frequently using self‐report measures. Little research has spanned across additional units of analyses including behavior, physiology, molecules, cells, and genes. Finally, there has been growing evidence in support of select psychotherapeutic treatment and prevention strategies, and preliminary evidence for technology‐based interventions.ConclusionsThere is much work to be done to better understand suicidal thoughts and behaviors among youth. We strongly encourage future research to: (1) continue improving the conceptualization and operationalization of suicidal thoughts and behaviors; (2) improve etiological understanding by focusing on individual (preferably malleable) mechanisms; (3) improve etiological understanding also by integrating findings across multiple units of analyses and developing short‐term prediction models; (4) demonstrate greater developmental sensitivity overall; and (5) account for diverse high‐risk populations via sampling and reporting of sample characteristics. These serve as initial steps to improve the scientific approach, knowledge base, and ultimately prevention of suicidal thoughts and behaviors among youth.
Characterisation of lattice damage formation in tantalum irradiated at variable temperatures
Authors: IPATOVA I. , WADY P.T. , SHUBEITA S.M. , BARCELLINI C. , IMPAGNATIELLO A. , JIMENEZ‐MELERO E. 
Published: November 2017

SummaryThe formation of radiation‐induced dislocation loops and voids in tantalum at 180(2), 345(3) and 590(5)°C was assessed by 3MeV proton irradiation experiments and subsequent damage characterisation using transmission electron microscopy. Voids formed at 345(3)°C and were arranged into a body centred cubic lattice at a damage level of 0.55 dpa. The low vacancy mobility at 180(2)°C impedes enough vacancy clustering and therefore the formation of voids visible by TEM. At 590(5)°C the Burgers vector of the interstitial‐type dislocation loops is a<100>, instead of the a/2 <111> Burgers vector characteristic of the loops at 180(2) and 345(3)°C. The lower mobility of a<100> loops hinders the formation of voids at 590(5)°C up to a damage level of 0.55 dpa.
Trust in organization as a moderator of the relationship between self‐efficacy and workplace outcomes: A social cognitive theory‐based examination
Authors: Ozyilmaz Adnan , Erdogan Berrin , Karaeminogullari Aysegul 
Published: November 2017

Drawing on a social cognitive theory perspective, we contend that an employee's trust in oneself, or self‐efficacy, will interact with the individual's trust in the system, or trust in organization, to predict job attitudes and behaviours. Specifically, we expected that self‐efficacy would have stronger effects on job attitudes (job satisfaction and turnover intentions) and behaviours (task performance and organizational citizenship behaviours) to the degree to which employees perceive high levels of trust in organization. Using data collected from 300 employees and their respective supervisors at a manufacturing organization in Turkey across three waves, we found that self‐efficacy had more positive effects on job satisfaction, task performance, and citizenship behaviours when trust in organization was high. Interestingly, self‐efficacy had a positive effect on turnover intentions when trust in organization was low, indicating that high trust in organization buffered the effects of self‐efficacy on intentions to leave. The results suggest that the motivational value of trust in oneself is stronger to the degree to which employees also have high trust in the system, whereas low trust in system neutralizes the motivational benefits of self‐efficacy.Practitioner points

Practicing managers should not only invest in increasing self‐efficacy of their employees, but also invest in building trust to improve employees’ attitudes, behaviours, and performance. This is because when employee trust in organization is high, employee self‐efficacy has greater potential to have a positive influence over job satisfaction, task performance, and organizational citizenship behaviours.

Self‐efficacy may actually increase an employee's desire to leave the organization when organizational conditions are unfavourable, such as in the case of low trust in the organization. Practicing managers should be aware that employees who have high levels of confidence may be at higher risk of turnover when they are unhappy with the organization.
Reliability of an adherence monitoring sensor embedded in an oral appliance used for treatment of obstructive sleep apnoea
Authors: Gjerde K. , Lehmann S. , Naterstad I. F. , Berge M. E. , Johansson A. 
Published: November 2017

SummaryThe aim of this study was to test whether digitally registered use of a mandibular advancement device (MAD) by a built‐in thermal sensor was reliable compared to a self‐reported diary of MAD use. Eighty consecutive patients referred to a specialist outpatient sleep medicine clinic (HUS) were recruited. Patients of both genders, aged from 25 to 70 years with a diagnosis of mild, moderate or severe, were included. All participants signed a written informed consent when they received the MAD. For the purpose of this reliability study, we found it sufficient to include the first 30 nights of MAD use in the reliability analysis. At the 30th night follow‐up visit, the self‐reported diary with duration of MAD use was returned and data on the duration of MAD use with the built‐in sensor were retrieved. From a total of 2400 nights, complete data from both methods were retrieved for 2108 nights (84.6%). Missing data were largely a result of missing self‐reported diaries, whereas technical failure occurred in 6 nights (0.002%). The relative reliability was very high with ICC3,1 0.847, and the absolute reliability for digitally registered MAD usage was calculated to −0.17 (95% CI: 1.47 to −1.81) hours in decimal conversion. Objectively collected data from built‐in thermal sensors in MADs are as reliable as those of the self‐report assessments. This opens new possibilities for more accurate measurements of MAD adherence.
ePTFE‐TIPS vs repetitive LVP plus albumin for the treatment of refractory ascites in patients with cirrhosis
Authors: Bucsics Theresa , Hoffman Sophie , Grünberger Johanna , Schoder Maria , Matzek Wolfgang , Stadlmann Alexander , Mandorfer Mattias , Schwabl Philipp , Ferlitsch Arnulf , Peck‐Radosavljevic Markus , Trauner Michael , Karner Josef , Karnel Franz , Reiberger Thomas 
Published: November 2017

AbstractBackground & AimsReduction in portal pressure by self‐expandable polytetrafluoroethylene (ePTFE)‐covered transjugular intrahepatic portosystemic shunts (TIPS) is a treatment option for refractory ascites. Data on clinical outcomes after ePTFE‐TIPS vs repetitive large‐volume paracentesis (LVP) plus albumin (A) administration for the treatment of patients with refractory ascites are limited.MethodsRetrospective comparison of ePTFE‐TIPS vs LVP+A in terms of (i) control of ascites, (ii) occurrence of overt hepatic encephalopathy (HE) and (iii) transplant‐free survival in cirrhotic patients with refractory ascites.ResultsAmong n = 221 patients with cirrhosis and refractory ascites, n = 140 received ePTFE‐TIPS and were compared to n = 71 patients undergoing repetitive LVP+A. After ePTFE‐TIPS, ascites was controlled without any further need for paracentesis in n = 76 (54%; n = 7 without and n = 69 with diuretics). The need for frequent large‐volume paracentesis was significantly higher in the LVP+A group than with ePTFE‐TIPS (median 0.67 (IQR: 0.23‐2.63) months vs 49.5 (IQR: 5.07‐102.60) months until paracentesis, log‐rank P < .001). De‐novo incidence of HE was similar in ePTFE‐TIPS and LVP+A patients (log‐rank P = .361). Implantation of ePTFE‐TIPS was associated with improved 1‐year survival as compared to LVP+A (65.6% vs 48.4%, log‐rank P = .033). Age (odds ratio (OR):1.05; 95% confidence interval (95% CI):1.03‐1.07; P < .001), serum albumin (OR: 0.95; 95% CI: 0.92‐0.99; P = .013) and hepatocellular carcinoma (OR: 1.66; 95% CI: 1.06‐2.58; P = .026) emerged as independent predictors of survival.ConclusionsePTFE‐TIPS results in superior control of ascites without increasing the risk for overt HE as compared to LVP+A. Although ePTFE‐TIPS improved 1‐year survival in cirrhotic patients with refractory ascites, its use was not independently associated with transplant‐free survival.
Ledipasvir/sofosbuvir for treatment of hepatitis C virus in sofosbuvir‐experienced, NS5A treatment‐naïve patients: Findings from two randomized trials
Authors: Tam Edward , Luetkemeyer Anne F. , Mantry Parvez S. , Satapathy Sanjaya K. , Ghali Peter , Kang Minhee , Haubrich Richard , Shen Xianlin , Ni Liyun , Camus Gregory , Copans Amanda , Rossaro Lorenzo , Guyer Bill , Brown Robert S. ,   the RESCUE and ACTG A5348 study investigators
Published: November 2017

AbstractBackground & AimsWe report data from two similarly designed studies that evaluated the efficacy, safety, and optimal duration of ledipasvir/sofosbuvir (LDV/SOF) ± ribavirin (RBV) for retreatment of chronic hepatitis C virus (HCV) in individuals who failed to achieve sustained virological response (SVR) with prior SOF‐based, non‐NS5A inhibitor‐containing regimens.MethodsThe RESCUE study enrolled HCV mono‐infected adults with genotype (GT) 1 or 4. Non‐cirrhotic participants were randomized to 12 weeks of LDV/SOF or LDV/SOF + RBV. Compensated cirrhotic participants were randomized to LDV/SOF + RBV (12 weeks) or LDV/SOF (24 weeks). The AIDS Clinical Trials Group A5348 study randomized genotype 1 adults with HCV/HIV co‐infection to LDV/SOF + RBV (12 weeks) or LDV/SOF (24 weeks). Both studies used SVR at 12 weeks post‐treatment (SVR12) as the primary endpoint.ResultsIn the RESCUE study, 82 participants were randomized and treated, and all completed treatment. Overall, SVR12 was 88% (72/82); 81‐100% in non‐cirrhotic participants treated with LDV/SOF or LDV/SOF + RBV for 12 weeks and 80‐92% in cirrhotic participants treated with LDV/SOF + RBV for 12 weeks or LDV/SOF for 24 weeks. Adverse events (AEs), mostly mild‐to‐moderate in severity, were experienced by 78% of participants, with headache and fatigue most frequently reported. One serious AE, not related to treatment, was observed. No premature discontinuations of study drug, or deaths occurred. In the A5348 study, seven participants were randomized (cirrhotic n = 1; GT1a n = 5) and all attained SVR12, with no serious AEs or premature discontinuations.ConclusionsIn this SOF‐experienced, NS5A inhibitor‐naïve population, which included participants with cirrhosis or HCV/HIV co‐infection, high SVR12 rates were achieved.
Successive evolutionary steps drove Pooideae grasses from tropical to temperate regions
Authors: Zhong Jinshun , Robbett Meghan , Poire Alfonso , Preston Jill C. 
Published: November 2017

Summary

Angiosperm adaptations to seasonally cold climates have occurred multiple times independently. However, the observation that less than half of all angiosperm families are represented in temperate latitudes suggests internal constraints on the evolution of cold tolerance/avoidance strategies. Similar to angiosperms as a whole, grasses are primarily tropical, but one major clade, subfamily Pooideae, radiated extensively within temperate regions. It is posited that this Pooideae niche transition was facilitated by an early origin of long‐term cold responsiveness around the base of the subfamily, and that a set of more ancient pathways enabled evolution of seasonal cold tolerance.

To test this, we compared transcriptome‐level responses of disparate Pooideae to short‐/long‐term cold and with those previously known in the subtropical grass rice (Ehrhartoideae).

Analyses identified several highly conserved cold‐responsive ‘orthogroups’ within our focal Pooideae species that originated successively during the diversification of land plants, predominantly via gene duplication. The majority of conserved Pooideae cold‐responsive genes appear to have ancient roles in stress responses, with most of the orthogroups also being sensitive to cold in rice. However, a subgroup of genes was likely co‐opted de novo early in the Pooideae.

These results highlight a plausible stepwise evolutionary trajectory for cold adaptations across Pooideae.
Clinical safety and performance of a MRI conditional pacing system in patients undergoing cardiac MRI
Authors: Ching Chi Keong , Chakraborty Rabindra Nath , Kler Tarlochan Singh , Pumprueg Satchana , Ngarmukos Tachapong , Chan Joseph Yat Sun , Anand Sumit , Yadav Rakesh , Sitthisook Surapun , Yim Ka Wing , Jaswal Rakesh K. , Bhargava Kartikeya 
Published: November 2017

AbstractBackgroundUtilization of cardiac magnetic resonance imaging (cMRI) as an imaging modality in clinical practice is rapidly increasing. More evidence from randomized studies establishing clinical safety and performance of pacing systems in patients undergoing a cMRI scan is needed.ObjectivesThe purpose of this prospective, multicenter, randomized study was to demonstrate safety and efficacy of the Accent MRI™ conditional pacing systems (St. Jude Medical, St. Paul, MN, USA) in patients undergoing cMRI scan.MethodsPatients (n = 283) indicated for dual‐chamber pacemaker implant were randomized to either the MRI Group (MG) (n = 140) or the Control Group (CG) (n = 143) after successful device implantation of the Accent MRI™ system. Clinical evaluation and device interrogation were performed at pre‐ and post‐MRI scan, and 1 month post‐MRI for all patients. At 9–12 weeks postimplant, patients in MG underwent a nondiagnostic cMRI scan at 1.5 Tesla (T), while patients in CG underwent device interrogation and clinical evaluation twice with a 45‐minute waiting period in between. The safety endpoint was freedom from MRI scan‐related complications and that for efficacy was significant changes in right atrial/ventricular capture threshold and sensing amplitude between right before MRI, immediately after MRI, and 1 month post‐MRI.ResultsResults showed 100% freedom from MRI scan‐related complications. There were no significant changes in device performance between pre‐MRI scan and 1 month post‐MRI scan time points in both study groups.ConclusionscMRI scanning with 1.5 T scanners is safe in patients implanted with the Accent MRI™ conditional pacing system and has no significant effect on the electrical parameters of the device and leads.
The former presence of organic matter caused its later absence: Burn‐down of organic matter in oceanic red beds enhanced by bioturbation (Eocene Variegated Shale, Carpathians)
Authors: Wetzel Andreas , Uchman Alfred 
Published: November 2017

AbstractEocene oceanic red beds that formed in a well‐oxygenated setting at low sedimentation rates below the calcite compensation depth are effectively barren of organic carbon in the present state. Recurrent distal low‐erosive turbidites preserve the bioturbated zone underneath that documents seasonal and long‐term fluctuating accumulation of considerable amounts of organic matter on the sea floor as evidenced by Scolicia; the producers of this trace fossil exploited nutritious organic matter conserved in turbidite‐buried sea floor deposits. Over the long‐term, slow average sedimentation of (hemi)pelagic oxic (red) mud led to long oxygen exposure times and low burial of organic matter. Consequently, trace fossils representing persistent sediment‐feeding modes are of small size. Although the food‐limited setting appears appropriate for producers of graphoglyptids, such ‘stationary’ burrows have not been encountered because seasonal deposition of organic matter fostered at least temporary surface layer feeding organisms, for instance producers of Nereites irregularis that intensively reworked the sediment and, hence, hindered graphoglyptid production. These findings confirm palaeoceanographic modelling results that suggest upwelling in the study area during the Eocene.