bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis
Authors: Sidana Surbhi , Tandon Nidhi , Gertz Morie A. , Dispenzieri Angela , Ramirez‐Alvarado Marina , Murray David L. , Kourelis Taxiarchis V. , Buadi Francis K. , Kapoor Prashant , Gonsalves Wilson , Warsame Rahma , Lacy Martha Q. , Kyle Robert A. , Rajkumar S. Vincent , Kumar Shaji K. , Leung Nelson 
Published: March 2019

SummaryThis study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10%, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5‐year OS in patients undergoing transplant was 90% vs. 75% vs. 64% (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.
No evidence of increased cerebrovascular involvement in adult neurologically‐asymptomatic β‐Thalassaemia. A multicentre multimodal magnetic resonance study
Authors: Tartaglione Immacolata , Russo Camilla , Elefante Andrea , Caiazza Martina , Casale Maddalena , Di Concilio Rosanna , Ciancio Angela , De Michele Elisa , Amendola Giovanni , Gritti Paolo , Carafa Pasquale A. , Ferrantino Teresa , Centanni Antonella , Ippolito Noemi , Caserta Violetta , Oliveto Tiziana , Granato Ilaria , Femina Gianluca , Esposito Fabrizio , Ponticorvo Sara , Russo Andrea G. , Canna Antonietta , Ermani Mario , Cirillo Mario , Perrotta Silverio , Manara Renzo 
Published: March 2019

SummaryMulti‐factorial causes jeopardize brain integrity in β‐thalassaemia. Intracranial parenchymal and vascular changes have been reported among young β‐thalassaemia patients but conventional magnetic resonance imaging (MRI) findings are contradictory making early MRI and magnetic resonance angiography (MRA)/venography monitoring a matter of debate.This study prospectively investigated 75 neurologically asymptomatic β‐thalassaemia patients (mean‐age 35·2 ± 10·7 years; 52/75 transfusion‐dependent; 41/75 splenectomised) using a 3T magnetic resonance scanner; clinical, laboratory and treatment data were also collected. White matter ischaemic‐like abnormalities, intracranial artery stenoses, aneurysms and sinus venous thrombosis were compared between patients and 56 healthy controls (mean‐age 33·9 ± 10·8 years). No patient or control showed silent territorial or lacunar strokes, intracranial artery stenoses or signs of sinus thrombosis. White matter lesions were found both in patients (35/75, 46·7%) and controls (28/56, 50·0%), without differences in terms of number (4·0 ± 10·6 vs. 4·6 ± 9·1, P = 0·63), size and Fazekas’ Score. Intracranial aneurysms did not differ between patients and controls for incidence rate (7/75, 9·3% vs. 5/56, 8·9%), size and site. Vascular and parenchymal abnormality rate did not differ according to treatments or clinical phenotype. According to this study, asymptomatic β‐thalassaemia patients treated according to current guidelines do not seem to carry an increased risk of brain and intracranial vascular changes, thus weakening recommendations for regular brain MRI monitoring.
A randomized controlled trial of internet‐delivered cognitive behaviour therapy for adolescent anxiety disorders in a routine clinical care setting with and without parent sessions
Authors: Waite Polly , Marshall Tamsin , Creswell Cathy 
Published: March 2019

BackgroundComputerized treatments have been shown to be effective in young people with anxiety disorders within research settings. The aims of this study were to evaluate a self‐completed, therapist‐supported online treatment for adolescent anxiety disorders in a routine clinical care setting and examine whether additional sessions for parents improved treatment outcome.MethodSixty adolescents (13–18 years) referred by primary and secondary care services for treatment of an anxiety disorder and their parent(s) were randomly allocated to begin treatment immediately or after a 16‐week waitlist. Half the parents (receiving treatment immediately or after a waitlist) were allocated to receive sessions themselves. Assessments were conducted pre‐ and posttreatment and at 6‐month follow‐up.ResultsThere was no significant difference posttreatment between the immediate treatment and waitlist groups in remission of primary anxiety disorder (Odds Ratio (OR) = 2.19, 95% CI: 0.72–6.70). Parent sessions did not significantly improve adolescent outcomes immediately or at 6‐month follow‐up (OR = 0.75, 95% CI: 0.26–2.15; OR = 1.14, 95% CI: 0.42–3.15).ConclusionsWithin a routine clinical care setting, a therapist‐supported online treatment failed to deliver significantly better outcomes for adolescents with anxiety disorders than a waitlist. Further research is needed to develop more effective treatments for this population.
Cognitive function and oral health among ageing adults
Authors: Kang Jing , Wu Bei , Bunce David , Ide Mark , Pavitt Sue , Wu Jianhua 
Published: March 2019

AbstractObjectivesThere is inconclusive evidence that cognitive function is associated with oral health in older adults. This study investigated the association between cognitive function and oral health among older adults in England.MethodsThis longitudinal cohort study included 4416 dentate participants aged 50 years or older in the English Longitudinal Study of Ageing during 2002‐2014. Cognitive function was assessed at baseline in 2002/2003 using a battery of cognitive function tests. The self‐reported number of teeth remaining and self‐rated general oral health status was reported in 2014/2015. Ordinal logistic regression was applied to model the association between cognitive function at baseline and tooth loss or self‐rated oral health.ResultsCognitive function at baseline was negatively associated with the risk of tooth loss (per each 1 standard deviation lower in cognitive function score, OR: 1.13, 95% CI: 1.05‐1.21). When cognitive function score was categorized into quintiles, there was a clear gradient association between cognitive function and tooth loss (P‐trend = 0.003); people in the lowest quintile of cognitive function had higher risk of tooth loss than those in the highest quintile (OR: 1.39, 95% CI: 1.12‐1.74). A similar magnitude and direction of association were evident between cognitive function and self‐rated oral health.ConclusionThis longitudinal study in an English ageing population has demonstrated that poor cognitive function at early stage was associated with poorer oral health and higher risk of tooth loss in later life. The gradient relationship suggests that an improvement in cognitive function could potentially improve oral health and reduce the risk of tooth loss in the ageing population.
Comparing and displaying phylogenetic trees using edge union networks
Authors: Miyagi Miriam , Wheeler Ward C. 
Published: March 2019

AbstractThe general problem of representing collections of trees as a single graph has led to many tree summary techniques. Many consensus approaches take sets of trees (either inferred as separate gene trees or gleaned from the posterior of a Bayesian analysis) and produce a single “best” tree. In scenarios where horizontal gene transfer or hybridization are suspected, networks may be preferred, which allow for nodes to have two parents, representing the fusion of lineages. One such construct is the cluster union network (CUN), which is constructed using the union of all clusters in the input trees. TheCUNhas a number of mathematically desirable properties, but can also present edges not observed in the input trees. In this paper we define a new network construction, the edge union network (EUN), which displays edges if and only if they are contained in the input trees. We also demonstrate that this object can be constructed with polynomial time complexity given arbitrary phylogenetic input trees, and so can be used in conjunction with network analysis techniques for further phylogenetic hypothesis testing.
Tracking genetic invasions: Genome‐wide single nucleotide polymorphisms reveal the source of pyrethroid‐resistant Aedes aegypti (yellow fever mosquito) incursions at international ports
Authors: Schmidt Thomas L. , van Rooyen Anthony R. , Chung Jessica , Endersby‐Harshman Nancy M. , Griffin Philippa C. , Sly Angus , Hoffmann Ary A. , Weeks Andrew R. 
Published: March 2019

AbstractBiological invasions are increasing globally in number and extent despite efforts to restrict their spread. Knowledge of incursion pathways is necessary to prevent new invasions and to design effective biosecurity protocols at source and recipient locations. This study uses genome‐wide single nucleotide polymorphisms (SNPs) to determine the origin of 115 incursive Aedes aegypti(yellow fever mosquito) detected at international ports in Australia and New Zealand. We also genotyped mosquitoes at three point mutations in the voltage‐sensitive sodium channel (Vssc) gene: V1016G, F1534C and S989P. These mutations confer knockdown resistance to synthetic pyrethroid insecticides, widely used for controlling invertebrate pests. We first delineated reference populations using Ae. aegypti sampled from 15 locations in Asia, South America, Australia and the Pacific Islands. Incursives were assigned to these populations using discriminant analysis of principal components (DAPC) and an assignment test with a support vector machine predictive model. Bali, Indonesia, was the most common origin of Ae. aegypti detected in Australia, while Ae. aegypti detected in New Zealand originated from Pacific Islands such as Fiji. Most incursives had the same allelic genotype across the three Vsscgene point mutations, which confers strong resistance to synthetic pyrethroids, the only insecticide class used in current, widely implemented aircraft disinsection protocols endorsed by the World Health Organization (WHO). Additionally, all internationally assigned Ae. aegypti had Vssc point mutations linked to pyrethroid resistance that are not found in Australian populations. These findings demonstrate that protocols for preventing introductions of invertebrates must consider insecticide resistance, and highlight the usefulness of genomic data sets for managing global biosecurity objectives.
Parent–Child Activities, Paid Work Interference, and Child Mental Health
Authors: Roeters Anne , van Houdt Kirsten 
Published: March 2019

ObjectiveTo examine the association between child mental health and (a) the amount of parent–child interaction and (b) the amount of interference in that interaction due to paid work.BackgroundAlthough some research findings suggest children do not always benefit from being with their parents full‐time, other studies suggest it is important for children to have their parents' undivided attention.MethodAnalyses are based on the 2013 New Families in the Netherlands dataset (639 fathers and 849 mothers of school‐aged children). Using structural equation modeling (SEM), we analyzed how child well‐being varied with the frequency of parent–child activities and the amount of interference due to paid work.ResultsChildren demonstrated better mental health when the frequency of father–child activities was higher and the amount of interference due to work was lower. We found no effects for mothers. Moreover, the association between the frequency of parent–child activities and child mental health was not moderated by the amount of interference due to paid work (neither for fathers nor mothers).ConclusionThe findings suggest that children attach great importance to the psychological availability of their fathers. We speculate that children respond more strongly to their fathers because their attention and involvement is less taken for granted than that of mothers.ImplicationsGiven the intrusive nature of smartphones and laptops in daily life, it is crucial that parents, employers, and family life educators understand how distractions during parent–child time due to paid work can affect children. With this knowledge, strategies to minimize unintended detrimental consequences for children can be developed.
A node release approach to estimate J‐R curve for single‐edge‐notched tension specimen under reversed loading
Authors: Liu Tianyao , Qian Xudong , Wang Wei , Chen Yiyi 
Published: March 2019

AbstractThis paper proposes a node release approach to estimate the fracture resistance curve, often known as the J‐R curve, for monotonic and cyclic fracture tests. The node release approach simulates the crack extension by releasing the constraints imposed on the node at the crack tip and estimates the J‐R curve by coupling the domain integral value with the corresponding crack extension. This proposed node release approach estimates closely the J‐R curve for SE(B) and SE(T) specimens subjected to monotonic loading. For SE(T) specimen under cyclic loading, this study implements the node release analysis in two approaches: (1) an equivalent monotonic analysis corresponding to the envelope of the cyclic load‐CMOD response and (2) a direct cyclic simulation. Both approaches lead to close estimations of the experimentally measured J‐R curve. The numerical analysis also confirms the path independence of the domain integral values in the direct cyclic simulation.
Influence of Fluctuating Groundwater Table on Volatile Organic Chemical Emission Flux at a Dissolved Chlorinated‐Solvent Plume Site
Authors: Guo Yuanming , Holton Chase , Luo Hong , Dahlen Paul , Johnson Paul C. 
Published: March 2019

AbstractGroundwater elevation fluctuation has been recognized as one mechanism causing temporal indoor air volatile organic chemical (VOC) impacts in vapor intrusion risk assessment guidance. For dissolved VOC sources, groundwater table fluctuation shortens/lengthens the transport pathway, and delivers dissolved contaminants to soils that are alternating between water saturated and variably saturated conditions, thereby enhancing volatilization potential. To date, this mechanism has not been assessed with field data, but enhanced VOC emission flux has been observed in lab‐scale and modeling studies. This work evaluates the impact of groundwater elevation changes on VOC emission flux from a dissolved VOC plume into a house, supplemented with modeling results for cyclic groundwater elevation changes. Indoor air concentrations, air exchange rates, and depth to groundwater (DTW) were collected at the study house during an 86‐d constant building underpressurization test. These data were used to calculate changes in trichloroethylene (TCE) emission flux to indoor air, during a period when DTW varied daily and seasonally from about 3.1 to 3.4 m below the building foundation (BF). Overall, TCE flux to indoor air varied by about 50% of the average, without any clear correlation to changes in DTW or its change rate. To complement the field study, TCE surface emission fluxes were simulated using a one‐dimensional model (HYDRUS 1D) for conditions similar to the field site. Simulation results showed time‐averaged surface TCE fluxes for cyclic water‐table elevations were greater than for stationary water‐table conditions at an equivalent time‐averaged water‐table position. The magnitudes of temporal TCE emission flux changes were generally less than 50% of the time‐averaged flux, consistent with the field site observations. Simulation results also suggested that TCE emission flux changes due to groundwater fluctuation are likely to be significant at sites with shallow groundwater (e.g., < 0.5 m BF) and permeable soil types (e.g., sand).
A molecular overview of the primary dystroglycanopathies
Authors: Brancaccio Andrea 
Published: March 2019

AbstractDystroglycan is a major non‐integrin adhesion complex that connects the cytoskeleton to the surrounding basement membranes, thus providing stability to skeletal muscle. In Vertebrates, hypoglycosylation of α‐dystroglycan has been strongly linked to muscular dystrophy phenotypes, some of which also show variable degrees of cognitive impairments, collectively termed dystroglycanopathies. Only a small number of mutations in the dystroglycan gene, leading to the so called primary dystroglycanopathies, has been described so far, as opposed to the ever‐growing number of identified secondary or tertiary dystroglycanopathies (caused by genetic abnormalities in glycosyltransferases or in enzymes involved in the synthesis of the carbohydrate building blocks). The few mutations found within the autonomous N‐terminal domain of α‐dystroglycan seem to destabilise it to different degrees, without influencing the overall folding and targeting of the dystroglycan complex. On the contrary other mutations, some located at the α/β interface of the dystroglycan complex, seem to be able to interfere with its maturation, thus compromising its stability and eventually leading to the intracellular engulfment and/or partial or even total degradation of the dystroglycan uncleaved precursor.
Dek42 encodes an RNA‐binding protein that affects alternative pre‐mRNA splicing and maize kernel development
Authors: Zuo Yi , Feng Fan , Qi Weiwei , Song Rentao 
Published: March 2019

AbstractRNA‐binding proteins (RBPs) play an important role in post‐transcriptional gene regulation. However, the functions of RBPs in plants remain poorly understood. Maize kernel mutant dek42 has small defective kernels and lethal seedlings. Dek42 was cloned by Mutator tag isolation and further confirmed by an independent mutant allele and clustered regularly interspaced short palindromic repeats (CRISPR)‐CRISPR‐associated protein 9 materials. Dek42 encodes an RRM_RBM48 type RNA‐binding protein that localizes to the nucleus. Dek42 is constitutively expressed in various maize tissues. The dek42 mutation caused a significant reduction in the accumulation of DEK42 protein in mutant kernels. RNA‐seq analysis showed that the dek42 mutation significantly disturbed the expression of thousands of genes during maize kernel development. Sequence analysis also showed that the dek42 mutation significantly changed alternative splicing in expressed genes, which were especially enriched for the U12‐type intron‐retained type. Yeast two‐hybrid screening identified SF3a1 as a DEK42‐interacting protein. DEK42 also interacts with the spliceosome component U1‐70K. These results suggested that DEK42 participates in the regulation of pre‐messenger RNA splicing through its interaction with other spliceosome components. This study showed the function of a newly identified RBP and provided insights into alternative splicing regulation during maize kernel development.
Use of computed tomography and radiation therapy planning software to develop a novel formula for body surface area calculation in dogs
Authors: Girens Renee , Bukoski Alex , Maitz Charles A. , Boston Sarah E. , Borgatti Antonella , Sprinkle Megan , Orrego Daniel , Kesl Shannon , Selting Kim 
Published: March 2019

BackgroundBody surface area (BSA) can reflect metabolic rate that might normalize dosing of chemotherapeutics across widely variable weights within a species. The current BSA formula for dogs lacks height, length, and body condition.HypothesisComputed tomography (CT) imaging will allow inclusion of morphometric variables in allometric modeling of BSA in dogs resulting in an improved formula for BSA estimation.AnimalsForty‐eight dogs from 4 institutions with whole‐body CT images.MethodsRetrospective and prospective case series. Body surface area was contoured using whole‐body CT scans and radiation therapy planning software. Body length and height were determined from CT images and also in 9 dogs by physical measurement. Nonlinear regression was used to model the BSA data sets using allometric equations. Goodness‐of‐fit criteria included average relative deviation, mean standard error, Akaike information criterion, and r2(derived from the r‐value generated by regression models).ResultsContoured BSA differed from the current formula by −9% to +19%. Nonlinear regression on untransformed data yielded BSA = 0.0134 × body weight [kg]∧0.4746 × length (cm)∧0.6393 as the best‐fit model. Heteroscedasticity (increasing morphometric variability with increasing BSA) was an important finding.Conclusions and Clinical ImportanceComputed tomography‐derived BSA was used to incorporate body length into a novel BSA formula. This formula can be applied prospectively to determine whether it correlates with adverse events attributed to chemotherapy.
Is Every Theory of Knowledge False?
Authors: Roeber Blake 
Published: March 2019

AbstractIs knowledge consistent with literally any credence in the relevant proposition, including credence 0? Of course not. But is credence 0 the only credence in p that entails that you don't know that p? Knowledge entails belief (most epistemologists think), and it's impossible to believe that p while having credence 0 in p. Is it true that, for every value of ‘x,’ if it's impossible to know that p while having credence x in p, this is simply because it's impossible to believe that p while having credence x in p? If so, is it possible to believe that p while having (say) credence 0.4 in p? These questions aren't standard epistemological fare—at least in part because many epistemologists think their answers are obvious—but they have unanticipated consequences for epistemology. Let ‘improbabilism’ name the thesis that it's possible to know that p while having a credence in p below 0.5. Improbabilism will strike many epistemologists as absurd, but careful reflection on these questions reveals that, if improbabilism is false, then all of the most plausible theories of knowledge are also false. Or so I shall argue in this paper. Since improbabilism is widely rejected by epistemologists (at least implicitly), this paper reveals a tension between all of the most plausible theories of knowledge and a widespread assumption in epistemology.
Allergic contact dermatitis to chlorhexidine in pediatric patients
Authors: Magdaleno‐Tapial Jorge , Martínez‐Doménech Alvaro , Valenzuela‐Oñate Cristian , Ferrer‐Guillén Blanca , Esteve‐Martínez Altea , Zaragoza‐Ninet Violeta 
Published: March 2019

AbstractChlorhexidine is one of the most widely used antiseptics in our environment. Allergic contact dermatitis to chlorhexidine is common in adults, but is quite rare in the pediatric population. Its diagnosis is important because it can mimic other dermatoses common during childhood, and it can also lead to acute immediate hypersensitivity reactions following successive exposures.