bluuebunny/crossref_metadata_2025_split huggingface dataset:
Critical care for patients with congenital abnormalities of the coronary arteries
Authors: Flores Saul , Moore Ryan A. , Statile Christopher J. , Michelfelder Erik C. , Wanstrath Shawna G. , Knilans Timothy K. , Morales David L. , Cooper David S. 
Published: December 2015

AbstractCongenital abnormalities of the coronary arteries in the absence of structural heart disease account for a small but interesting percentage of cardiac lesions in children. Their presentation may vary from incidental identification to aborted/sudden cardiac death. Patients with aborted sudden death episodes will require significant support if they develop extensive ischaemic myocardial injury. Ultimately, surgical repair should be carried out as soon as haemodynamic stability is attained and the neurological status is evaluated. The aims of this article were to provide a review of congenital abnormalities of the coronary arteries most commonly seen in children in the ICU as well as to review the current critical-care management thereof.
The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes
Authors: Anand Vijay , Cave Dominic , McCrady Heather , Al-Aklabi Mohammed , Ross David B. , Rebeyka Ivan M. , Adatia Ian 
Published: December 2015

AbstractIn 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children’s Hospital.
Primary-care management of patients with congenital anomalies of the coronary arteries
Authors: Boris Jeffrey R. , Brothers Julie A. 
Published: December 2015

AbstractCongenital anomalies of the coronary arteries, although uncommon, have the potential to cause serious myocardial damage, ischaemic cardiomyopathy, and sudden cardiac death. This article summarises aspects of care for these patients in the outpatient setting, including clinical history and physical examination findings, ancillary testing, decision-making regarding the need for surgical intervention, and recommendations for physical activity. Although there are limited data regarding some of these recommendations, it is hoped that these can be used as an initial benchmark against which further data will lead to a more evidence-based approach.
Characterising adequacy or inadequacy of the borderline left ventricle: what tools can we use?
Authors: Kaplinski Michelle , Cohen Meryl S. 
Published: December 2015

AbstractBorderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.
Introduction: December 2015 HeartWeek Issue of Cardiology in the Young – Highlights of HeartWeek 2015: Challenges and Dilemmas of Pediatric Cardiac Care including Heart Failure in Children and Congenital Abnormalities of the Coronary Arteries
Authors: Jacobs Jeffrey P. 
Published: December 2015

AbstractThis December Issue of Cardiology in the Young represents the 13th annual publication in Cardiology in the Young generated from the two meetings that composed “HeartWeek in Florida”. “HeartWeek in Florida”, the joint collaborative project sponsored by the Cardiac Centre at the Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, together with Johns Hopkins All Children’s Heart Institute of Saint Petersburg, Florida, averages over 1000 attendees every year and is now recognised as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the foetus, neonate, infant, child, and adult. “HeartWeek in Florida” combines the International Symposium on Congenital Heart Disease, organised by All Children’s Hospital and Johns Hopkins Medicine, and entering its 16th year, with the Annual Postgraduate Course in Paediatric Cardiovascular Disease, organised by The Children’s Hospital of Philadelphia entering its 19th year.This December 2015 Issue of Cardiology in the Young features highlights of the two meetings that compose HeartWeek. Johns Hopkins All Children’s Heart Institute’s 15th Annual International Symposium on Congenital Heart Disease was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from Friday, 6 February, 2015, to Monday, 9 February, 2015. This Symposium was co-sponsored by The American Association for Thoracic Surgery and its special focus was “Congenital Abnormalities of the Coronary Arteries”. The Children’s Hospital of Philadelphia’s annual meeting – Cardiology 2015, the 18th Annual Update on Paediatric and Congenital Cardiovascular Disease: “Challenges and Dilemmas” – was held at the Hyatt Regency Scottsdale Resort and Spa at Gainey Ranch, Scottsdale, Arizona, from Wednesday, 11 February, 2015, to Sunday, 15 February, 2015.We would like to acknowledge the tremendous contributions made to paediatric and congenital cardiac care by Juan Valentín Comas, MD, PhD (13 May, 1960 to 16 June, 2015) and Donald Nixon Ross, FRCS (4 October, 1922 to 7 July, 2014); and therefore, we dedicate this December 2015 HeartWeek Issue of Cardiology in the Young to them.
Ars Moriendi: Coping with death in the Late Middle Ages
Authors: Espi Forcén Fernando , Espi Forcén Carlos 
Published: December 2015

ABSTRACTObjective:TheArs moriendiwas a book written in the early 15th century with the goal of assisting friars in their work of helping the dying. The aim of our study was to review the current literature on theArs Moriendiconcerning the field of medicine, to analyze the psychological mechanisms for coping with death anxiety withinArs Moriendi, and to explore parallels between the strategies used in the medieval book and in contemporary literature about death and dying.Method:A review of literature using Pubmed, EMBASE, JSTOR, Project MUSE, and the New York Public Library was undertaken first. The primary source was then interpreted from a medical/psychological point of view.Results:Seven articles were selected by literature review. These works comment on the importance of theArs Moriendiin its historical context and explore the possibility of retrieving the principles of the text in contemporary society. The original text ofArs Moriendi, the primary source, presents death as a relief from the sufferings of earthly life and a gateway to eternal glory. According to the author, a good death implied the triumph over five demonic temptations in agonizing people: a lack of faith, despair, impatience, pride and greed.Significance of Results:Analyzed from a modern psychiatric perspective, theArs Moriendioffers descriptions of behavioral manifestations compatible with delirium, mood and anxiety disorders that characterize people with terminal illnesses. Moreover, we also explored parallels between the strategies used to cope with death anxiety in the Late Middle Ages and in contemporary society.
MBNL1-mediated regulation of differentiation RNAs promotes myofibroblast transformation and the fibrotic response
Authors: Davis Jennifer , Salomonis Nathan , Ghearing Natasha , Lin Suh-Chin J. , Kwong Jennifer Q. , Mohan Apoorva , Swanson Maurice S. , Molkentin Jeffery D. 
Published: December 2015

AbstractThe differentiation of fibroblasts into myofibroblasts mediates tissue wound healing and fibrotic remodelling, although the molecular programme underlying this process remains poorly understood. Here we perform a genome-wide screen for genes that control myofibroblast transformation, and identify the RNA-binding protein muscleblind-like1 (MBNL1). MBNL1 overexpression promotes transformation of fibroblasts into myofibroblasts, whereas loss of Mbnl1 abrogates transformation and impairs the fibrotic phase of wound healing in mouse models of myocardial infarction and dermal injury. Mechanistically, MBNL1 directly binds to and regulates a network of differentiation-specific and cytoskeletal/matrix-assembly transcripts to promote myofibroblast differentiation. One of these transcripts is the nodal transcriptional regulator serum response factor (SRF), whereas another is calcineurin Aβ. CRISPR-Cas9-mediated gene-editing of the MBNL1-binding site within the Srf 3′UTR impairs myofibroblast differentiation, whereas in vivo deletion of Srf in fibroblasts impairs wound healing and fibrosis. These data establish a new RNA-dependent paradigm for myofibroblast formation through MBNL1.
All-photonic intercity quantum key distribution
Authors: Azuma Koji , Tamaki Kiyoshi , Munro William J. 
Published: December 2015

AbstractRecent field demonstrations of quantum key distribution (QKD) networks hold promise for unconditionally secure communication. However, owing to loss in optical fibres, the length of point-to-point links is limited to a hundred kilometers, restricting the QKD networks to intracity. A natural way to expand the QKD network in a secure manner is to connect it to another one in a different city with quantum repeaters. But, this solution is overengineered unless such a backbone connection is intercontinental. Here we present a QKD protocol that could supersede even quantum repeaters for connecting QKD networks in different cities below 800 km distant. Nonetheless, in contrast to quantum repeaters, this protocol uses only a single intermediate node with optical devices, requiring neither quantum memories nor quantum error correction. Our all-photonic ‘intercity’ QKD protocol bridges large gaps between the conventional intracity QKD networks and the future intercontinental quantum repeaters, conceptually and technologically.
Toxic tau oligomer formation blocked by capping of cysteine residues with 1,2-dihydroxybenzene groups
Authors: Soeda Yoshiyuki , Yoshikawa Misato , Almeida Osborne F. X. , Sumioka Akio , Maeda Sumihiro , Osada Hiroyuki , Kondoh Yasumitsu , Saito Akiko , Miyasaka Tomohiro , Kimura Tetsuya , Suzuki Masaaki , Koyama Hiroko , Yoshiike Yuji , Sugimoto Hachiro , Ihara Yasuo , Takashima Akihiko 
Published: December 2015

AbstractNeurofibrillary tangles, composed of hyperphosphorylated tau fibrils, are a pathological hallmark of Alzheimer’s disease; the neurofibrillary tangle load correlates strongly with clinical progression of the disease. A growing body of evidence indicates that tau oligomer formation precedes the appearance of neurofibrillary tangles and contributes to neuronal loss. Here we show that tau oligomer formation can be inhibited by compounds whose chemical backbone includes 1,2-dihydroxybenzene. Specifically, we demonstrate that 1,2-dihydroxybenzene-containing compounds bind to and cap cysteine residues of tau and prevent its aggregation by hindering interactions between tau molecules. Further, we show that orally administered DL-isoproterenol, an adrenergic receptor agonist whose skeleton includes 1,2-dihydroxybenzene and which penetrates the brain, reduces the levels of detergent-insoluble tau, neuronal loss and reverses neurofibrillary tangle-associated brain dysfunction. Thus, compounds that target the cysteine residues of tau may prove useful in halting the progression of Alzheimer’s disease and other tauopathies.
Single injection of small-molecule amyloid accelerator results in cell death of nigral dopamine neurons in mice
Authors: Chermenina Maria , Chorell Erik , Pokrzywa Małgorzata , Antti Henrik , Almqvist Fredrik , Strömberg Ingrid , Wittung-Stafshede Pernilla 
Published: December 2015

AbstractThe assembly process of α-synuclein toward amyloid fibers is linked to neurodegeneration in Parkinson’s disease. In the present study, we capitalized on thein vitrodiscovery of a small-molecule accelerator of α-synuclein amyloid formation and assessed its effects when injected in brains of normal mice. An accelerator and an inhibitor of α-synuclein amyloid formation, as well as vehicle only, were injected into the striatum of normal mice and followed by behavioral evaluation, immunohistochemistry, and metabolomics up to six months later. The effects of molecules injected into the substantia nigra of normal and α-synuclein knock-out mice were also analyzed. When accelerator or inhibitor was injected into the brain of normal mice no acute compound toxicity was found. However, 6 months after single striatal injection of accelerator, mice sensorimotor functions were impaired, whereas mice injected with inhibitor had no dysfunctions. Injection of accelerator (but not inhibitor or vehicle) into the substantia nigra revealed significant loss of tyrosine hydroxylase (TH)-positive neurons after 3 months. No loss of TH-positive neurons was found in α-synuclein knock-out mice injected with accelerator into the substantia nigra. Metabolic serum profiles from accelerator-injected normal mice matched those of newly diagnosed Parkinson’s disease patients, whereas the profiles from inhibitor-injected normal mice matched controls. Single inoculation of a small-molecule amyloid accelerator may be a new approach for studies of early events during dopamine neurodegeneration in mice.
Reduced pupillary reward sensitivity in Parkinson’s disease
Authors: Manohar S G , Husain M 
Published: December 2015

AbstractAbnormalities in reward processing may be a critical part of understanding nonmotor manifestations of Parkinson’s disease (PD). Dysfunction in dopaminergic pathways, which signal upcoming rewards, might result in altered motivation by incentives. To examine this proposal, we studied 16 patients with PD, both ON and OFF their normal dopaminergic medication, comparing them with healthy controls. Participants performed a speeded saccade task to obtain monetary rewards. Crucially, we manipulated the reward available from trial to trial, by presenting an auditory incentive precue before each saccade. The effects of incentives on pupil dilatation (an index of autonomic response) were measured. Individuals with PD showed diminished autonomic reward effects, compared with age-matched controls. When tested ON medication, pupil responses to reward increased, demonstrating that dopaminergic drugs can restore reward sensitivity. These findings reveal blunted autonomic responses to incentives in PD, which can be modulated by dopaminergic drugs.
Dysfunctional vestibular system causes a blood pressure drop in astronauts returning from space
Authors: Hallgren Emma , Migeotte Pierre-François , Kornilova Ludmila , Delière Quentin , Fransen Erik , Glukhikh Dmitrii , Moore Steven T. , Clément Gilles , Diedrich André , MacDougall Hamish , Wuyts Floris L. 
Published: December 2015

AbstractIt is a challenge for the human body to maintain stable blood pressure while standing. The body’s failure to do so can lead to dizziness or even fainting. For decades it has been postulated that the vestibular organ can prevent a drop in pressure during a position change – supposedly mediated by reflexes to the cardiovascular system. We show – for the first time – a significant correlation between decreased functionality of the vestibular otolith system and a decrease in the mean arterial pressure when a person stands up. Until now, no experiments on Earth could selectively suppress both otolith systems; astronauts returning from space are a unique group of subjects in this regard. Their otolith systems are being temporarily disturbed and at the same time they often suffer from blood pressure instability. In our study, we observed the functioning of both the otolith and the cardiovascular system of the astronauts before and after spaceflight. Our finding indicates that an intact otolith system plays an important role in preventing blood pressure instability during orthostatic challenges. Our finding not only has important implications for human space exploration; they may also improve the treatment of unstable blood pressure here on Earth.
Non-local Optical Topological Transitions and Critical States in Electromagnetic Metamaterials
Authors: Ishii Satoshi , Narimanov Evgenii 
Published: December 2015

AbstractJust as the topology of the Fermi surface defines the properties of the free electrons in metals and semiconductors, the geometry of the iso-frequency surface in the phase space of the propagating electromagnetic waves, determines the optical properties of the corresponding optical materials. Furthermore, in the direct analog to the Lifshitz transition in condensed matter physics, a change in the topology of iso-frequency surface has a dramatic effect on the emission, propagation and scattering of the electromagnetic waves. Here, we uncover a new class of such optical topological transitions in metamaterials, induced by the non-locality of the electromagnetic response inherent to these composites.
Familiarity affects other-regarding preferences in pet dogs
Authors: Quervel-Chaumette Mylene , Dale Rachel , Marshall-Pescini Sarah , Range Friederike 
Published: December 2015

AbstractOther-regarding preferences are considered to be the foundation of human cooperation. However, the evolutionary origin of this behavior in humans remains poorly understood. So far, comparative studies in primates have led to mixed conclusions probably due to methodological differences relating to both task complexity and the types of control conditions used. Moreover, no clear link between phylogenetic relatedness and prosociality has been found, suggesting that other convergent selection pressures may play a role in the evolution of such behaviors. Here, using one of the cognitively less demanding tasks, we show for the first time, that dogs can behave pro-socially by donating food to a conspecific partner, but only if the partner is familiar. This highlights the importance of considering the social relationships between individuals when testing animals for other-regarding behaviors. Moreover, by including a social control condition, we show that the dogs’ prosocial response was not due to a simple social facilitation effect. The current findings support recent proposals that other convergent selection pressures, such as dependence on cooperative activities, rather than genetic relatedness to humans, may shape a species’ propensity for other-regarding behaviors.
Biotechnological production of 1,2,4-butanetriol: An efficient process to synthesize energetic material precursor from renewable biomass
Authors: Cao Yujin , Niu Wei , Guo Jiantao , Xian Mo , Liu Huizhou 
Published: December 2015

Abstract1,2,4-Butanetriol (BT) is a valuable chemical with extensive applications in many different fields. The traditional chemical routes to synthesize BT suffer from many drawbacks, e.g., harsh reaction conditions, multiple steps and poor selectivity, limiting its industrial production. In this study, an engineered Escherichia coli strain was constructed to produce BT from xylose, which is a major component of the lignocellulosic biomass. Through the coexpression of a xylose dehydrogenase (CCxylB) and a xylonolactonase (xylC) from Caulobacter crescentus, native E. coli xylonate dehydratase (yjhG), a 2-keto acid decarboxylase from Pseudomonas putida (mdlC) and native E. coli aldehyde reductase (adhP) in E. coli BL21 star(DE3), the recombinant strain could efficiently convert xylose to BT. Furthermore, the competitive pathway responsible for xylose metabolism in E. coli was blocked by disrupting two genes (xylA and EcxylB) encoding xylose isomerase and xyloluse kinase. Under fed-batch conditions, the engineered strain BL21ΔxylAB/pE-mdlCxylBC&pA-adhPyjhG produced up to 3.92 g/L of BT from 20 g/L of xylose, corresponding to a molar yield of 27.7%. These results suggest that the engineered E. coli has a promising prospect for the large-scale production of BT.
Hierarchical deconstruction of mouse olfactory sensory neurons: from whole mucosa to single-cell RNA-seq
Authors: Saraiva Luis R. , Ibarra-Soria Ximena , Khan Mona , Omura Masayo , Scialdone Antonio , Mombaerts Peter , Marioni John C. , Logan Darren W. 
Published: December 2015

AbstractThe mouse olfactory mucosa is a complex chemosensory tissue composed of multiple cell types, neuronal and non-neuronal. We have here applied RNA-seq hierarchically, in three steps of decreasing cellular heterogeneity: starting with crude tissue samples dissected from the nose, proceeding to flow-cytometrically sorted pools of mature olfactory sensory neurons (OSNs) and finally arriving at single mature OSNs. We show that 98.9% of intact olfactory receptor (OR) genes are expressed in mature OSNs. We uncover a hitherto unknown bipartition among mature OSNs. We find that 19 of 21 single mature OSNs each express a single intact OR gene abundantly, consistent with the one neuron-one receptor rule. For the 9 single OSNs where the two alleles of the abundantly expressed OR gene exhibit single-nucleotide polymorphisms, we demonstrate that monoallelic expression of the abundantly expressed OR gene is extremely tight. The remaining two single mature OSNs lack OR gene expression but express Trpc2 and Gucy1b2. We establish these two cells as a neuronal cell type that is fundamentally distinct from canonical, OR-expressing OSNs and that is defined by the differential, higher expression of 55 genes. We propose this tiered experimental approach as a paradigm to unravel gene expression in other cellularly heterogeneous systems.