bluuebunny/crossref_metadata_2025_split huggingface dataset:
Rediscovering Ducos du Hauron's Color Photography through a Review of His Three‐Color Printing Processes and Synchrotron Microanalysis of His Prints
Authors: Cotte Marine , Fabris Tiphaine , Langlois Juliette , Bellot‐Gurlet Ludovic , Ploye Françoise , Coural Natalie , Boust Clotilde , Gandolfo Jean‐Paul , Galifot Thomas , Susini Jean 
Published: March 2018

AbstractLouis Ducos du Huron (1837–1920) dedicated his entire life to the elaboration of physical–chemical processes for color photography. This study aimed at highlighting his unique contribution to three‐color printing through 1) an in‐depth review of the many protocols he published and 2) the synchrotron‐based IR and X‐ray microanalysis of fragments sampled in three artworks. Ducos du Hauron's method relied on the preparation and assembly of three monochromes (red, blue, yellow). This study brings to light complex multistep recipes based on photochemistry (carbon print), organic, and inorganic chemistry. The various ingredients involved (e.g., pigments, dichromate gelatin, collodion, resin) were identified and localized through their spectroscopic signature, confirming the relevance of synchrotron spectromicroscopy for the characterization of historical photographs. The impressive correlation between texts and chemical analyses calls for a wider application to the history of photography.
A family segregating lethal neonatal coenzyme Q10 deficiency caused by mutations in COQ9
Authors: Smith Amanda C. , Ito Yoko , Ahmed Afsana , Schwartzentruber Jeremy A. , Beaulieu Chandree L. , Aberg Erika , Majewski Jacek , Bulman Dennis E. , Horsting‐Wethly Karina , Koning Diana Vermunt‐de ,   Care4Rare Canada Consortium, Rodenburg Richard J. , Boycott Kym M. , Penney Lynette S. 
Published: March 2018

AbstractPrimary CoQ10 deficiency is a clinically and genetically heterogeneous, autosomal recessive disorder resulting from mutations in genes involved in the synthesis of coenzyme Q10 (CoQ10). To date, mutations in nine proteins required for the biosynthesis of CoQ10 cause CoQ10 deficiency with varying clinical presentations. In 2009 the first patient with mutations in COQ9 was reported in an infant with a neonatal‐onset, primary CoQ10 deficiency with multi‐system disease. Here we describe four siblings with a previously undiagnosed lethal disorder characterized by oligohydramnios and intrauterine growth restriction, variable cardiomyopathy, anemia, and renal anomalies. The first and third pregnancy resulted in live born babies with abnormal tone who developed severe, treatment unresponsive lactic acidosis after birth and died hours later. Autopsy on one of the siblings demonstrated brain changes suggestive of the subacute necrotizing encephalopathy of Leigh disease. Whole‐exome sequencing (WES) revealed the siblings shared compound heterozygous mutations in the COQ9 gene with both variants predicted to affect splicing. RT‐PCR on RNA from patient fibroblasts revealed that the c.521 + 2 T > C variant resulted in splicing out of exons 4–5 and the c.711 + 3G > C variant spliced out exon 6, resulting in undetectable levels of COQ9 protein in patient fibroblasts. The biochemical profile of patient fibroblasts demonstrated a drastic reduction in CoQ10 levels. An additional peak on the chromatogram may represent accumulation of demethoxy coenzyme Q (DMQ), which was shown previously to accumulate as a result of a defect in COQ9. This family expands our understanding of this rare metabolic disease and highlights the prenatal onset, clinical variability, severity, and biochemical profile associated with COQ9‐related CoQ10 deficiencies.
Treatment with chenodeoxycholic acid in cerebrotendinous xanthomatosis: clinical, neurophysiological, and quantitative brain structural outcomes
Authors: Amador Maria del Mar , Masingue Marion , Debs Rabab , Lamari Foudil , Perlbarg Vincent , Roze Emmanuel , Degos Bertrand , Mochel Fanny 
Published: March 2018

AbstractBackgroundCerebrotendinous xanthomatosis (CTX) is a rare neurodegenerative disease related to sterols metabolism. It affects both central and peripheral nervous systems but treatment with chenodeoxycholic acid (CDCA) has been reported to stabilize clinical scores and improve nerve conduction parameters. Few quantitative brain structural studies have been conducted to assess the effect of CDCA in CTX.Methods and resultsWe collected retrospectively clinical, neurophysiological, and quantitative brain structural data in a cohort of 14 patients with CTX treated by CDCA over a mean period of 5 years. Plasma cholestanol levels normalized under treatment with CDCA within a few months. We observed a significant clinical improvement in patients up to 25 years old, whose treatment was initiated less than 15 years after the onset of neurological symptoms. Conversely, patients whose treatment was initiated more than 25 years after neurological disease onset continued their clinical deterioration. Eleven patients presented with a length‐dependent peripheral neuropathy, whose electrophysiological parameters improved significantly under CDCA. Volumetric analyses in a subset of patients showed no overt volume loss under CDCA. Moreover, diffusion weighted imaging showed improved fiber integrity of the ponto‐cerebellar and the internal capsule with CDCA. CDCA was well tolerated in all patients with CTX.ConclusionCDCA may reverse the pathophysiological process in patients with CTX, especially if treatment is initiated early in the disease process. Besides tendon xanthoma, this study stresses the need to consider plasma cholestanol measurement in any patient with infantile chronic diarrhea and/or jaundice, juvenile cataract, learning disability and/or autism spectrum disorder, pyramidal signs, cerebellar syndrome or peripheral neuropathy.
Asymptotic shape and the speed of propagation of continuous-time continuous-space birth processes
Authors: Bezborodov Viktor , Di Persio Luca , Krueger Tyll , Lebid Mykola , Ożański Tomasz 
Published: March 2018

AbstractWe formulate and prove a shape theorem for a continuous-time continuous-space stochastic growth model under certain general conditions. Similar to the classical lattice growth models, the proof makes use of the subadditive ergodic theorem. A precise expression for the speed of propagation is given in the case of a truncated free-branching birth rate.
Establishing the Incidence and Prevalence of Multiple Sclerosis in Saskatchewan
Authors: Al-Sakran Lina H. , Marrie Ruth Ann , Blackburn David F. , Knox Katherine B. , Evans Charity D. 
Published: March 2018

AbstractObjective: To validate a case definition of multiple sclerosis (MS) using health administrative data and to provide the first province-wide estimates of MS incidence and prevalence for Saskatchewan, Canada. Methods: We used population-based health administrative data between January 1, 1996 and December 31, 2015 to identify individuals with MS using two potential case definitions: (1) ≥3 hospital, physician, or prescription claims (Marrie definition); (2) ≥1 hospitalization or ≥5 physician claims within 2 years (Canadian Chronic Disease Surveillance System [CCDSS] definition). We validated the case definitions using diagnoses from medical records (n=400) as the gold standard. Results: The Marrie definition had a sensitivity of 99.5% (95% confidence interval [CI] 92.3-99.2), specificity of 98.5% (95% CI 97.3-100.0), positive predictive value (PPV) of 99.5% (95% CI 97.2-100.0), and negative predictive value (NPV) of 97.5% (95% CI 94.4-99.2). The CCDSS definition had a sensitivity of 91.0% (95% CI 81.2-94.6), specificity of 99.0% (95% CI 96.4-99.9), PPV of 98.9% (95% CI 96.1-99.9), and NPV of 91.7% (95% CI 87.2-95.0). Using the more sensitive Marrie definition, the average annual adjusted incidence per 100,000 between 2001 and 2013 was 16.5 (95% CI 15.8-17.2), and the age- and sex-standardized prevalence of MS in Saskatchewan in 2013 was 313.6 per 100,000 (95% CI 303.0-324.3). Over the study period, incidence remained stable while prevalence increased slightly. Conclusion: We confirm Saskatchewan has one of the highest rates of MS in the world. Similar to other regions in Canada, incidence has remained stable while prevalence has gradually increased.
Calibration of EFOSC2 Broadband Linear Imaging Polarimetry
Authors: Wiersema K. , Higgins A. B. , Covino S. , Starling R. L. C. 
Published: March 2018

AbstractThe European Southern Observatory Faint Object Spectrograph and Camera v2 is one of the workhorse instruments on ESO’s New Technology Telescope, and is one of the most popular instruments at La Silla observatory. It is mounted at a Nasmyth focus, and therefore exhibits strong, wavelength and pointing-direction-dependent instrumental polarisation. In this document, we describe our efforts to calibrate the broadband imaging polarimetry mode, and provide a calibration for broadband B, V, and R filters to a level that satisfies most use cases (i.e. polarimetric calibration uncertainty ~0.1%). We make our calibration codes public. This calibration effort can be used to enhance the yield of future polarimetric programmes with the European Southern Observatory Faint Object Spectrograph and Camera v2, by allowing good calibration with a greatly reduced number of standard star observations. Similarly, our calibration model can be combined with archival calibration observations to post-process data taken in past years, to form the European Southern Observatory Faint Object Spectrograph and Camera v2 legacy archive with substantial scientific potential.
DNA barcoding of ancient parasites
Authors: WOOD JAMIE R. 
Published: March 2018

SUMMARYAncient samples present a number of technical challenges for DNA barcoding, including damaged DNA with low endogenous copy number and short fragment lengths. Nevertheless, techniques are available to overcome these issues, and DNA barcoding has now been used to successfully recover parasite DNA from a wide variety of ancient substrates, including coprolites, cesspit sediment, mummified tissues, burial sediments and permafrost soils. The study of parasite DNA from ancient samples can provide a number of unique scientific insights, for example: (1) into the parasite communities and health of prehistoric human populations; (2) the ability to reconstruct the natural parasite faunas of rare or extinct host species, which has implications for conservation management and de-extinction; and (3) the ability to view in ‘real-time’ processes that may operate over century- or millenial-timescales, such as how parasites responded to past climate change events or how they co-evolved alongside their hosts. The application of DNA metabarcoding and high-throughput sequencing to ancient specimens has so far been limited, but in future promises great potential for gaining empirical data on poorly understood processes such as parasite co-extinction.
The first multilocus genotype analysis of Giardia intestinalis in humans in the Czech Republic
Authors: Lecová L. , Weisz F. , Tůmová P. , Tolarová V. , Nohýnková E. 
Published: March 2018

AbstractTo date, genotyping data on giardiasis have not been available in the Czech Republic. In this study, we characterized 47 human isolates of Giardia intestinalis from symptomatic as well as asymptomatic giardiasis cases. Genomic DNA from trophozoites was tested by PCR-sequence analysis at three loci (β-giardin, glutamate dehydrogenase and triose phosphate isomerase). Sequence analysis showed assemblages A and B in 41 (87.2%) and six (12.8%) isolates, respectively. Two of the 41 assemblage A samples were genotyped as sub-assemblage AI, and 39 were genotyped as sub-assemblage AII. Four previously identified multilocus genotypes (MLGs: AI-1, AII-1, AII-4 and AII-9) and six likely novel variations of MLGs were found. In agreement with previous studies, sequences from assemblage B isolates were characterized by a large genetic variability and by the presence of heterogeneous positions, which prevent the definition of MLGs. This study also investigated whether there was a relationship between the assemblage and clinical data (including drug resistance). However, due to the large number of genotypes and the relatively small number of samples, no significant associations with the clinical data were found.
Forgiveness then satisfaction: why the order matters for a theory of the atonement
Authors: KINTZ JAMES S. 
Published: March 2018

AbstractCentral to a theory of the atonement is one's position on forgiveness and satisfaction. These two issues are interrelated, but it is unclear whether one takes precedence over the other. Specifically, must one make satisfaction to remove the guilt incurred by sin prior to forgiveness, or can a victim forgive a wrongdoer independently of any work of satisfaction? Richard Swinburne argues that satisfaction must precede forgiveness, but I defend the view that forgiveness is a manifestation of love, and as such satisfaction is not required prior to forgiveness. Instead, I argue that forgiveness can and should precede satisfaction, and I highlight important implications of this view for a theory of the atonement.
Between conformity and innovation: China’s and India’s quest for status as responsible nuclear powers
Authors: Leveringhaus Nicola , de Estrada Kate Sullivan 
Published: March 2018

AbstractChina and India, as rising powers, have been proactive in seeking status as nuclear responsibles. Since the 1990s they have sought to demonstrate conformity with intersubjectively accepted understandings of nuclear responsibility within the global nuclear order, and have also sought recognition on the basis of particularistic practices of nuclear restraint. This article addresses two puzzles. First, nuclear restraint is at the centre of the pursuit of global nuclear order, so why have China and India not received recognition from influential members of the nuclear order for the full spectrum of their restraint-based behaviours? Second, why do China and India nonetheless persist with these behaviours? We argue that the conferral of status as a nuclear responsible is a politicised process shaped by the interests, values, and perceptions of powerful stakeholder states in the global nuclear order. China’s and India’s innovations are not incorporated into the currently accepted set of responsible nuclear behaviours because, indirectly, they pose a strategic, political, and social challenge to these states. However, China’s and India’s innovations are significant as an insight into their identity-projection and preferred social roles as distinctive rising powers, and as a means of introducing new, if nascent, ideas into non-proliferation practice and governance.
Education and Attitudes toward Interpersonal and State-Sanctioned Violence
Authors: Schnabel Landon 
Published: March 2018

ABSTRACTThe link between education and liberal attitudes is among the most consistent findings in public-opinion research, but the theoretical explanations for this relationship warrant additional attention. Previous work suggested that the relationship is due to education socializing students to the “official culture” of the United States. This study uses the World Values Survey and General Social Survey to examine Americans’ attitudes toward the justifiability of violence. I find that Americans with more education are less likely to say that interpersonal violence—against women, children, and other individuals—can be justifiable. However, they are more likely to say that state-sanctioned violence—war and police violence—can be justifiable. These patterns are consistent with a modified socialization model of education and social attitudes. I conclude that American education socializes people to establishment culture, identity, and interests, which differentiate between unacceptable interpersonal violence and ostensibly acceptable state-sanctioned violence.
Sweetened beverages, snacks and overweight: findings from the Young Lives cohort study in Peru
Authors: Alviso-Orellana Claudia , Estrada-Tejada Dayna , Carrillo-Larco Rodrigo M , Bernabé-Ortiz Antonio 
Published: March 2018

AbstractObjectiveTo determine the association between consumption of snacks and sweetened beverages and risk of overweight among children.DesignSecondary analysis of the Young Lives cohort study in Peru.SettingTwenty sentinel sites from a total of 1818 districts available in Peru.SubjectsChildren in the younger cohort of the Young Lives study in Peru, specifically those included in the third (2009) and the fourth (2013) rounds.ResultsA total of 1813 children were evaluated at baseline; 49·2 % girls and mean age 8·0 (sd0·3) years. At baseline, 3·3 (95 % CI 2·5, 4·2) % reported daily sweetened beverage consumption, while this proportion was 3·9 (95 % CI 3·1, 4·9) % for snacks. Baseline prevalence of overweight was 22·0 (95 % CI 20·1, 23·9) %. Only 1414 children were followed for 4·0 (sd0·1) years, with an overweight incidence of 3·6 (95 % CI 3·1, 4·1) per 100 person-years. In multivariable analysis, children who consumed sweetened beverages and snacks daily had an average weight increase of 2·29 (95 % CI 0·62, 3·96) and 2·04 (95 % CI 0·48, 3·60) kg more, respectively, than those who never consumed these products, in approximately 4 years of follow-up. Moreover, there was evidence of an association between daily consumption of sweetened beverages and risk of overweight (relative risk=2·12; 95 % CI 1·05, 4·28).ConclusionsDaily consumption of sweetened beverages and snacks was associated with increased weight gainv. never consuming these products; and in the case of sweetened beverages, with higher risk of developing overweight.
A genome-scale RNAi screen for genetic interactors of the dynein co-factor nud-2 in Caenorhabditis elegans
Authors: Rocha Helder , Maia André F. , Gassmann Reto 
Published: March 2018

AbstractCytoplasmic dynein 1 (dynein) is the predominant microtubule minus end-directed motor in animals and participates in a wide range of cellular processes, including membrane trafficking, nuclear migration, and cell division. Dynein's functional diversity depends on co-factors that regulate its subcellular localization, interaction with cargo, and motor activity. The ubiquitous co-factor nuclear distribution gene E (NudE) is implicated in many of dynein's functions, and mutations in NudE cause the brain developmental disease microcephaly. To identify genetic interactors of the Caenorhabditis elegans NudE homolog nud-2, we performed a genome-wide RNAi screen with the null allele nud-2(ok949), which compromises dynein function but leaves animals viable and fertile. Using bacterial feeding to deliver dsRNAs in a 96-well liquid format and a semi-automated fluorescence microscopy approach for counting parents and progeny, we screened 19762 bacterial clones and identified 38 genes whose inhibition caused enhanced lethality in nud-2(ok949) relative to the nud-2(+) control. Further study of these genes, many of which participate in cell division, promises to provide insight into the function and regulation of dynein.
Compact, wideband impedance tuner using a three‐line‐microstrip structure
Authors: Heredia J. , Ribó M. , Pradell L. 
Published: March 2018

A novel three‐line‐microstrip compact impedance tuner is presented. It is based on a multimodal structure wherein six variable capacitances, implemented with two parallel‐connected varactors each, create multiple interactions among the three‐line‐microstrip modes in a reduced circuit area. Experimental results show better‐than‐70% coverage of the Smith chart in an 85% frequency bandwidth from 1.4 to 3.2 GHz.
Amiodarone-Induced Thyroid Dysfunction: A Clinical Update
Authors: Elnaggar Mohamed , Jbeili Kahtan , Nik-Hussin Nik , Kozhippally Mohandas , Pappachan Joseph 
Published: March 2018

AbstractAmiodarone is one of the most commonly prescribed antiarrhythmic agents in clinical practice owing to its efficacy, even with high toxicity profile. The high iodine content and the prolonged biological half-life of the drug can result in thyroid dysfunction in a high proportion of patients treated with amiodarone even after cessation of amiodarone. Both hypothyroidism and hyperthyroidism are common side effects that mandate regular monitoring of patients with thyroid function tests. Amiodarone-induced hypothyroidism (AIH) is diagnosed and managed in the same way as a usual case of hypothyroidism. However, differential diagnosis and clinical management of amiodarone-induced thyrotoxicosis (AIT) subtypes can be challenging. With the aid of a case snippet, we update the current evidence for the diagnostic work up and management of patients with amiodarone-induced thyroid dysfunction in this article.
TAFI Meets the Sticky Ends
Authors: Booth Nuala 
Published: March 2018

SummaryThe coagulation and fibrinolytic system are often considered as separate entities, despite the obvious connection that one produces fibrin, the substrate for the other. A potent example of a more subtle and yet important connection lies in thrombin’s ability to generate an inhibitor of fibrinolysis. This exemplifies the pivotal role of thrombin in haemostasis, a consequence of its broad range of activities. Thrombin activates the zymogen form of a basic carboxypeptidase, generating an active enzyme called carboxypeptidase U, carboxypeptidase R, plasma carboxypeptidase B or TAFIa, thrombin activable fibrinolysis inhibitor (reviewed in 1-4; references from the reviews are not cited here). The activated carboxypeptidase removes C-terminal basic residues from fibrin. C-terminal Lys residues are key to binding t-PA and plasminogen, and thus to generating plasmin activity, a mechanism by which partially-lysed fibrin amplifies plasmin formation. The removal of these C-terminal Lys residues therefore decreases local fibrinolytic activity (Fig. 1). Several recent studies, two of which appear in this issue of Thrombosis and Haemostasis (5, 6), add to our knowledge of this activity and its potential role in thromboembolic disease.
Regulation of Fibrinolysis in Plasma by TAFI and Protein C Is Dependent on the Concentration of Thrombomodulin
Authors: Meijers Joost , Bouma Bonno , Mosnier Laurent 
Published: March 2018

SummaryThrombin activatable fibrinolysis inhibitor (TAFI) is a carboxypeptidase B-like proenzyme, that after activation down regulates fibrinolysis. TAFI is activated by thrombin in the presence of the cofactor thrombomodulin (TM). By stimulation of TAFI activation TM down regulates fibrinolysis, however TM is also a cofactor in the activation of protein C. Activated protein C (APC) can up regulate fibrinolysis by limiting the activation of TAFI via the attenuation of thrombin production. We studied these counteracting fibrinolytic properties of TM in plasma by measuring the activation of TAFI during tissue factor induced coagulation. TAFI activation was stimulated at low concentrations of TM but decreased at higher concentrations of TM. Similarly, the clot lysis times increased at low concentrations of TM but decreased at higher concentrations of TM. The reduction of TAFI activation at high TM concentrations was found to be dependent on a functional protein C pathway. The concentration of TM is therefore an important factor in the regulation of TAFI activation and in the regulation of fibrinolysis. High concentrations of TM result in up regulation of fibrinolysis, whereas low concentrations of TM have a down regulatory effect on fibrinolysis. These results suggest that fibrinolysis might be differentially regulated by TM in different parts of the body depending on the local TM concentration in the vasculature.
Abbreviations: TAFI, thrombin activatable fibrinolysis inhibitor; TAFIa, activated TAFI; CPI, carboxypeptidase inhibitor from potato tubers; tPA, tissue-type plasminogen activator; TM, thrombo-modulin; TM4-6, Thrombomodulin fragment containing EGF domains 4 to 6; HMEC, human microvascular endothelial cell.