bluuebunny/crossref_metadata_2025_split huggingface dataset:
Peripheral neuropathy and parkinsonism: a large clinical and pathogenic spectrum
Authors: Vital Anne , Lepreux Sebastien , Vital Claude 
Published: January 2015

AbstractPeripheral neuropathy (PN) has been reported in idiopathic and hereditary forms of parkinsonism, but the pathogenic mechanisms are unclear and likely heterogeneous. Levodopa‐induced vitamin B12 deficiency has been discussed as a causal factor of PN in idiopathic Parkinson's disease, but peripheral nervous system involvement might also be a consequence of the underlying neurodegenerative process. Occurrence of PN with parkinsonism has been associated with a panel of mitochondrial cytopathies, more frequently related to a nuclear gene defect and mainly polymerase gamma (POLG1) gene. Parkin (PARK2) gene mutations are responsible for juvenile parkinsonism, and possible peripheral nervous system involvement has been reported. Rarely, an association of parkinsonism with PN may be encountered in other neurodegenerative diseases such as fragile X‐associated tremor and ataxia syndrome related to premutation CGG repeat expansion in the fragile X mental retardation (FMR1) gene, Machado–Joseph disease related to an abnormal CAG repeat expansion in ataxin‐3 (ATXN3) gene, Kufor–Rakeb syndrome caused by mutations in ATP13A2 gene, or in hereditary systemic disorders such as Gaucher disease due to mutations in the β‐glucocerebrosidase (GBA) gene and Chediak–Higashi syndrome due to LYST gene mutations. This article reviews conditions in which PN may coexist with parkinsonism.
Influence of birthweight on perinatal outcome in fetuses with antenatal diagnosis of congenital heart disease
Authors: Story Lisa , Pasupathy Dharmintra , Sankaran Srividhya , Sharland Gurleen , Kyle Pippa 
Published: January 2015

AbstractAimThe incidence of congenital heart disease (CHD) accounts for the largest proportion of infant mortality attributable to birth defects. Associations have previously been reported between CHD and low birthweight. Low birthweight is independently associated with adverse outcome and has characteristically been calculated using population‐based charts. This aim of this study was to determine the incidence of small for gestational age (SGA) in fetuses with CHD utilizing customized birthweight centiles and to determine the effect of SGA on adverse outcome.MethodsA retrospective cohort study was performed between 2006 and 2011. All singleton fetuses with CHD, with no associated karyotype or structural extra‐cardiac abnormalities, who delivered at St Thomas's Hospital, London, were included. Population and customized birthweight centiles were calculated and perinatal outcome data were recorded.ResultsA total of 17% of fetuses with CHD had a birthweight centile <10th when population centiles were used, and 25% when customized birthweight centiles were applied. There was no correlation between SGA and increased adverse perinatal outcome.ConclusionsA high proportion of fetuses with CHD are classified as SGA when both population and customized birthweight centiles are used. SGA does not correlate with adverse outcome in the perinatal period. The cardiac defect therefore appears to be the main determinant of outcome and not the size of the baby at delivery.
Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population‐based multicentre cohort study
Authors: Bartoloni E. , Baldini C. , Schillaci G. , Quartuccio L. , Priori R. , Carubbi F. , Bini V. , Alunno A. , Bombardieri S. , De Vita S. , Valesini G. , Giacomelli R. , Gerli R. 
Published: January 2015

AbstractObjectiveSystemic autoimmune diseases, in particular systemic lupus erythematosus and rheumatoid arthritis, are characterized by a high risk of premature cardiovascular (CV) events. Disease‐related characteristics and traditional CV disease risk factors may contribute to atherosclerotic damage. However, there are limited data on the risk of overt CV events in primary Sjögren's syndrome (pSS).MethodsWe retrospectively analysed a cohort of patients with 1343 pSS. Disease‐related clinical and laboratory data, traditional CV disease risk factors and overt CV events were recorded. Prevalence of traditional CV disease risk factors and of major CV events was compared between a subgroup of 788 female patients with pSS aged from 35 to 74 years and 4774 age‐matched healthy women.ResultsHypertension and hypercholesterolaemia were more prevalent, whereas smoking, obesity and diabetes mellitus were less prevalent, in women with pSS than in control subjects. Cerebrovascular events (2.5% vs. 1.4%, P = 0.005) and myocardial infarction (MI) (1.0% vs. 0.4%, P = 0.002) were more common in patients with pSS. In the whole population, central nervous system involvement (odds ratio (OR) 5.6, 95% confidence interval (CI) 1.35–23.7, P = 0.02) and use of immunosuppressive therapy (OR 1.9, 95% CI 1.04–3.70, P = 0.04) were associated with a higher risk of CV events. Patients with leucopenia had a higher risk of angina (P = 0.01).ConclusionspSS is associated with an increased risk of cerebrovascular events and MI. Disease‐related clinical and immunological markers may have a role in promoting CV events.
Successful treatment with telaprevir‐based regimens for chronic hepatitis C results in significant improvements to serum markers of liver fibrosis
Authors: Haseltine E. L. , Penney M. S. , George S. , Kieffer T. L. 
Published: January 2015

SummaryPatients infected with hepatitis C virus (HCV) have differing levels of liver health when they initiate treatment. We sought to quantify whether liver health improves following successful treatment with telaprevir‐based antiviral regimens. We performed a retrospective analysis of data generated from one Phase 2 and two Phase 3 telaprevir clinical studies. 1208 patients treated with a telaprevir‐based regimen were included in the analysis. Patients were grouped according to their baseline Metavir score (F0–F1, F2 and F3–F4) and whether or not they attained sustained virologic response (SVR). Scores from four biomarker tests, FibroTest, APRI, FIB‐4 and Forns' Score, were monitored both before and after HCV treatment. All four of these tests differentiated the fibrosis stage as determined by Metavir score at baseline. Consistent with previous studies, patients who attained SVR exhibited significant improvements in scores from each of these tests after treatment. These improvements remained significant even when patients were grouped according to their baseline Metavir score. On average, the scores from different tests exhibited differential improvements following SVR. Improvements in APRI scores corresponded to complete fibrosis regression (i.e. a Metavir stage of F0‐F1). In contrast, improvements in scores from Forns' Score, FIB‐4 and FibroTest were more modest (i.e. fibrosis regression of less than a Metavir stage). Overall, these results demonstrated that attaining SVR with a telaprevir‐based regimen led to significant improvements in liver health as determined by four biomarker tests. However, not all correlations observed between noninvasive markers and fibrosis stage at baseline hold after SVR is attained.
Pharmacokinetics and effects on thromboxane B2production following intravenous administration of flunixin meglumine to exercised thoroughbred horses
Authors: Knych H. K. , Arthur R. M. , McKemie D. S. , Chapman N. 
Published: January 2015

Flunixin meglumine is commonly used in horses for the treatment of musculoskeletal injuries. The current ARCI threshold recommendation is 20 ng/mLwhen administered at least 24 h prior to race time. In light of samples exceeding the regulatory threshold at 24 h postadministration, the primary goal of the study reported here was to update the pharmacokinetics of flunixin following intravenous administration, utilizing a highly sensitive liquid chromatography–mass spectrometry (LC‐MS). An additional objective was to characterize the effects of flunixin on COX‐1 and COX‐2 inhibition when drug concentrations reached the recommended regulatory threshold. Sixteen exercised adult horses received a single intravenous dose of 1.1 mg/kg. Blood samples were collected up to 72 h postadministration and analyzed using LC‐MS. Blood samples were collected from 8 horses for determination of TxB2and PGE2concentrations prior to and up to 96 h postflunixin administration. Mean systemic clearance, steady‐state volume of distribution and terminal elimination half‐life was 0.767 ± 0.098 mL/min/kg, 0.137 ± 0.12 L/kg, and 4.8 ± 1.59 h, respectively. Four of the 16 horses had serum concentrations in excess of the current ARCI recommended regulatory threshold at 24 h postadministration. TxB2suppression was significant for up to 24 h postadministration.
Evaluation of a new balloon occlusion catheter specifically designed for measurement of hepatic venous pressure gradient
Authors: Ferlitsch Arnulf , Bota Simona , Paternostro Rafael , Reiberger Thomas , Mandorfer Mattias , Heinisch Birgit , Salzl Petra , Schwarzer Remy , Sieghart Wolfgang , Peck‐Radosavljevic Markus , Ferlitsch Monika 
Published: January 2015

AbstractBackground & AimsDespite the important clinical value of hepatic venous pressure gradient (HVPG) and its increasing use, no specific balloon occlusion catheters have been designed to cannulate liver veins. The aim of the study was to evaluate the clinical applicability of a novel balloon (NC) occlusion catheter specifically designed for HVPG measurement.MethodsComparison of a new CE‐certified 7 French balloon occlusion catheter with a 150° angled tip and radiopaque markers (NC, Pejcl Medizintechnik, Austria), to a commonly used straight balloon catheter (SC; Boston Scientific, USA). Successful liver vein cannulation rate, need for extra equipment and total fluoroscopy time were recorded. Experts (>200) and novices (<20) in HVPG measurements were evaluated separately.Results566 HVPG measurements taken by 11 investigators (five experts and six novices) were analysed. Overall, HVPG could be successfully measured in 98.7% of cases. The rate of successful liver vein cannulation at first attempt was significantly higher among experts when compared to novices (87.3% vs 67.3%, P < 0.001). Moreover, the rate of successful liver vein cannulation without need for any additional equipment was higher when using the NC, both among experts (NC:91.9% vs SC:80.6%, P = 0.03) and novices (NC:73.3% vs SC:50.7%, P = 0.001). The mean fluoroscopy time needed to cannulate the hepatic vein was significantly shorter in experts as compared to novices [2.37(0.10–26) vs 5.2(0.6–30.2] min, P < 0.0001), but not significantly different between catheters.ConclusionsBoth novices and experts achieve higher liver vein cannulation rates using the new specifically designed catheter. The use of the novel catheter might increase rates of successful liver vein cannulation and reduce the need for additional equipment, especially in novices.
Permanent Pacemaker Insertion Following Isolated Aortic Valve Replacement before and after the Introduction of TAVI
Authors: KOGAN ALEXANDER , STERNIK LEONID , BEINART ROY , SHALABI AMJAD , GLIKSON MICHAEL , SPIEGELSTEIN DANNY , LEVIN SHANY , RAANANI EHUD 
Published: January 2015

BackgroundPermanent pacemaker (PPM) implantation is required in 3–12% of all patients undergoing surgical aortic valve replacement (AVR). Our aim was to evaluate the contemporary incidence and impact of the introduction of transcatheter aortic valve implantation (TAVI) for PPM insertion after isolated AVR.MethodsSince 2004, during a 10‐year period, a total of 858 patients underwent isolated AVR at our institution. Forty‐one patients with PPM before operation were excluded from the study and 817 patients were included in the statistical analysis. Of these, 20 patients (2.45%) developed significant conduction disorders, leading to PPM implantation within 120 days postoperatively. Patients were further divided into two groups. Before (Group I: June 2004 to September 2008) and after (Group II: October 2008 to May 2014) the introduction of the TAVI program.ResultsThere were 343 patients in Group I and 475 patients in Group II. The incidence of PPM implantation decreased from 3.79% (13 patients) in Group I to 1.47% (seven patients) in Group II (P < 0.001). Risk factors for permanent pacing identified by univariate analysis were: Group I (before introducing TAVI program), pulmonary hypertension, preoperative anemia, age older than 75 years, and previous myocardial infarction. Multivariate analysis identified Group I (before introducing TAVI program; P < 0.005; odds ratio [OR] 15.2, 95% confidence interval [CI] 6.3–19.9) and pulmonary hypertension (P < 0.005; OR 12.5, 95% CI 3.2–18.3) to be significant.ConclusionsIrreversible atrio‐ventricular block or symptomatic bradycardia requiring PPM implantation is a relatively rare complication. The incidence of PPM implantation after isolated surgical AVR decreased in a contemporary setting after the introduction of the TAVI program.
Expression of a bacterial 3‐dehydroshikimate dehydratase reduces lignin content and improves biomass saccharification efficiency
Authors: Eudes Aymerick , Sathitsuksanoh Noppadon , Baidoo Edward E. K. , George Anthe , Liang Yan , Yang Fan , Singh Seema , Keasling Jay D. , Simmons Blake A. , Loqué Dominique 
Published: January 2015

SummaryLignin confers recalcitrance to plant biomass used as feedstocks in agro‐processing industries or as source of renewable sugars for the production of bioproducts. The metabolic steps for the synthesis of lignin building blocks belong to the shikimate and phenylpropanoid pathways. Genetic engineering efforts to reduce lignin content typically employ gene knockout or gene silencing techniques to constitutively repress one of these metabolic pathways. Recently, new strategies have emerged offering better spatiotemporal control of lignin deposition, including the expression of enzymes that interfere with the normal process for cell wall lignification. In this study, we report that expression of a 3‐dehydroshikimate dehydratase (QsuB from Corynebacterium glutamicum) reduces lignin deposition in Arabidopsis cell walls. QsuB was targeted to the plastids to convert 3‐dehydroshikimate – an intermediate of the shikimate pathway – into protocatechuate. Compared to wild‐type plants, lines expressing QsuB contain higher amounts of protocatechuate, p‐coumarate, p‐coumaraldehyde and p‐coumaryl alcohol, and lower amounts of coniferaldehyde, coniferyl alcohol, sinapaldehyde and sinapyl alcohol. 2D‐NMR spectroscopy and pyrolysis‐gas chromatography/mass spectrometry (pyro‐GC/MS) reveal an increase of p‐hydroxyphenyl units and a reduction of guaiacyl units in the lignin of QsuB lines. Size‐exclusion chromatography indicates a lower degree of lignin polymerization in the transgenic lines. Therefore, our data show that the expression of QsuB primarily affects the lignin biosynthetic pathway. Finally, biomass from these lines exhibits more than a twofold improvement in saccharification efficiency. We conclude that the expression of QsuB in plants, in combination with specific promoters, is a promising gain‐of‐function strategy for spatiotemporal reduction of lignin in plant biomass.
Efficacy of hepatic T2* MRI values and serum ferritin concentration in predicting thalassemia major classification for hematopoietic stem cell transplantation
Authors: Hamidieh Amir Ali , Moeininia Fatemeh , Tayebi Sirous , Shamshiri Ahmad Reza , Behfar Maryam , Jalili Mahdi , Alimoghaddam Kamran , Ghavamzadeh Ardeshir 
Published: January 2015

AbstractLiver biopsy has been performed for many decades for classifying the patients with TM. Meanwhile, using non‐invasive methods such as T2* MRI technique has been recently much more considered to determine the hepatic iron overload. Ninety‐three pediatric HSCT candidates with TM who underwent liver biopsy were included in this study. Hepatic T2* MRI values and serum ferritin concentrations were assessed to investigate and determine the useful method in detection of patients with TM class III whom received different conditioning regimens, in comparison with class I and II. Twenty (21.5%) patients were categorized as class III. Hepatic T2* MRI could detect TM class III patients with 60% sensitivity and 87.67% specificity (LR+: 4.867, accuracy: 81.72%), while predictive feature of ferritin values for distinguishing patients with TM class III was not statistically significant (p‐value >0.01). Combination of T2*MRI with age (T2*‐age) could detect TM class III with 85% sensitivity and 72.6% specificity (LR+: 3.1, accuracy: 75.27%).T2*‐age may be considered as an alternative and non‐invasive method to liver biopsy for differentiation and classification of patients with TM before transplantation.
The LEADER Initiative has been a Victim of Its Own Success. The Decline of the Bottom‐Up Approach in Rural Development Programmes. The Cases of Wales and Andalusia
Authors: Navarro Francisco Antonio , Woods Michael , Cejudo Eugenio 
Published: January 2015

AbstractThe LEADER approach has been at the heart of European rural development policy for the last 20 years, encompassing the principles of bottom‐up endogenous development and community empowerment. Initially delivered through autonomous local action groups (LAGs), since the 2007–2013 programming period, LEADER has been integrated with other measures in broader regional rural development programmes. It has been claimed that these changes have diluted the participatory principles of this programme. We examine the extent and impact of participation in rural development through LEADER, how this has changed over time, and the factors driving changes, through surveys of LAG managers in two case study regions in Spain (Andalusia) and the UK (Wales). The findings show that LAG managers are very positive about the breadth of participation in their own group and its role in decentralising decision‐making, but critical of the structure, operation and management of LEADER in rural development programmes. In particular bureaucracy and the increased influence of regional and local government are perceived to have limited the autonomy of LAGs and to have deterred the participation of marginalised groups. The principles of this initiative are perceived to have been diluted and LEADER appears to have been a victim of its own success.
Firm Profitability and Agglomeration Economies: An Elusive Relationship
Authors: Stavropoulos Spyridon , Skuras Dimitris 
Published: January 2015

AbstractRecent empirical research has questioned the ability of agglomeration economies to stimulate profitability and healthy financial performance in firms. We devise a cross‐sectional sample of approximately 410,000 firms that were active in all subsectors of the manufacturing industry and that were located across 191 NUTS 2 regions of 15 European Union member states in 2005. For each firm, we calculate two profitability indicators and a labour efficiency indicator. We find that the part of the variance of the profitability and labour efficiency indicators that is attributed to regions is very small. Furthermore, fitted multilevel models show that the quantitative effect of regional specialisation, despite being statistically significant, is very small. The results are robust across more refined geographical scales and subsectors. However, the NUTS 2 level remains the major spatial scale of the design and implementation of European Union regional policy. Our results benefit from the power and safety of a large sample and challenge conventional approaches to agglomeration research and cluster policy.
Port Regionalisation from a Relational Perspective: The Rise of Venlo as Dutch International Logistics Hub
Authors: Raimbault Nicolas , Jacobs Wouter , van Dongen Frank 
Published: January 2015

AbstractThe rise of global supply chain systems and geographical dispersion of related inland logistics centres has led to a new phase in the evolution of port systems, referred to as port regionalisation. While this process largely results from the decisions of shippers and logistics providers, the scope of public policy can also shape it. This includes the strategic capacity of stakeholders to couple not only locally available assets with the needs of global flows, but also to provide meaning through the constitution of symbols, frames and discourses. We propose the application of a relational perspective to port regionalisation, which allows us to analyse how various actors engage strategically in actor‐networks and coalitions across scales to stimulate growth based on logistics. This is accomplished by presenting the rise of Venlo in the Netherlands as an inland logistics hub within the corridor of the port of Rotterdam.
Natural Resource Industries As a Platform for the Development of Knowledge Intensive Industries
Authors: Marin Anabel , Navas‐Alemán Lizbeth , Perez Carlota 
Published: January 2015

AbstractIn the innovation and development literature, natural resources (NR) are generally viewed as a curse for developing nations and NR‐based industries as having little potential to innovate and drive long‐term growth. This has led policy and development experts to opt for strategies to induce a shift in the pattern of specialization towards other sectors. This paper proposes a different approach. By exploring recent evidence from the Argentinean agricultural sector and the mining industry in Chile, it points to the window of opportunity that NR industries offer as a platform to develop knowledge intensive industries with which to support economic development in resource‐endowed countries. The lessons drawn from these findings suggest that development strategies can also promote more innovative knowledge intensive NR‐based industries rather than moving away from them.
Reframing HIV care: putting people at the centre of antiretroviral delivery
Authors: Duncombe Chris , Rosenblum Scott , Hellmann Nicholas , Holmes Charles , Wilkinson Lynne , Biot Marc , Bygrave Helen , Hoos David , Garnett Geoff 
Published: January 2015

SummaryThe delivery of HIV care in the initial rapid scale‐up of HIV care and treatment was based on existing clinic‐based models, which are common in highly resourced settings and largely undifferentiated for individual needs. A new framework for treatment based on variable intensities of care tailored to the specific needs of different groups of individuals across the cascade of care is proposed here. Service intensity is characterised by four delivery components: (i) types of services delivered, (ii) location of service delivery, (iii) provider of health services and (iv) frequency of health services. How these components are developed into a service delivery framework will vary across countries and populations, with the intention being to improve acceptability and care outcomes. The goal of getting more people on treatment before they become ill will necessitate innovative models of delivering both testing and care. As HIV programmes expand treatment eligibility, many people entering care will not be ‘patients’ but healthy, active and productive members of society . To take the framework to scale, it will be important to: (i) define which individuals can be served by an alternative delivery framework; (ii) strengthen health systems that support decentralisation, integration and task shifting; (iii) make the supply chain more robust; and (iv) invest in data systems for patient tracking and for programme monitoring and evaluation.