bluuebunny/crossref_metadata_2025_split huggingface dataset:
Les interventions comportementales intensives et précoces auprès des enfants avec autisme : une revue critique de la littérature récente
Authors: Cruveiller Virginie 
Published: December 2014

Les interventions comportementales intensives et précoces (méthode aba , ou eibi , Early Intensive Behavioral Intervention ) font aujourd’hui l’objet d’un apparent consensus ; leur validité serait scientifiquement prouvée. Or, les données de la littérature scientifique sont bien plus divergentes et controversées qu’il n’y paraît. Cet article se propose de faire le point sur l’état actuel des connaissances en la matière, à travers une revue de la littérature récente (essais cliniques et méta-analyses parus depuis 2009). Si ce type d’intervention est probablement efficace sous certaines formes et pour certains enfants, comme l’attestent plusieurs essais cliniques, de nombreuses questions restent sans réponse, et le niveau de preuve général de ces études est grevé par un certain nombre de biais méthodologiques potentiels. De plus, certaines études montrent que l’intensivité d’une prise en charge n’est pas nécessairement corrélée à l’importance de ses effets. Les données actuellement disponibles – par ailleurs souvent en faveur de l’ eibi – restent donc lacunaires et discutables.
Remarques à propos du troisième Plan autisme (2013-2017)
Authors: Trouvé Jean-Noël 
Published: December 2014

Le récent « plan autisme » du gouvernement a désagréablement surpris, non pas tant par son manque de moyens que par son univocité autoritaire en faveur des interventions éducatives, et par son exclusion de fait du service public de pédopsychiatrie des soins aux autistes ; loin de la logique de complémentarité intégrative que défendent beaucoup de professionnels et de familles.
Pulsion invocante avec les bébés à risque d'autisme
Authors: Laznik Marie-Christine 
Published: December 2014

En confrontant la clinique avec des enfants autistes et les textes de Freud et ceux de Lacan, Marie-Christine Laznik a fait l’hypothèse que le troisième temps du circuit pulsionnel serait absent chez les enfants autistes. Testant cette hypothèse sur des films familiaux de bébés en voie de devenir autistes, elle s’est heurtée à une disparité étonnante chez ces bébés, habituellement fermés mais capables par moments d’entrer dans des interactions réussies. Imprégnée des travaux des psycholinguistes, elle a émis une autre hypothèse : celle de l’importance du mamanais dans les interactions avec ce type de bébé. Le mamanais , trace dans la voix de la mère du plaisir émerveillé suscité par son bébé, renvoie au troisième temps du circuit pulsionnel. Les bébés en sont avides dès la naissance, sans aucun étayage sur un objet du besoin. L’analyse acoustique des films familiaux de bébés qui sont devenus autistes, puis celle de séances de thérapie avec un bébé à risque autistique, ont montré que ces bébés peuvent répondre à l’appel de cette pulsion invoquante, lorsqu’un adulte est capable de ce type de prosodie. Cependant, cela reste rare car ces bébés ne jouent pas leur rôle dans cette interaction avec l’adulte.
A phreatic explosion model inferred from a very long period seismic event at Mayon Volcano, Philippines
Authors: Maeda Yuta , Kumagai Hiroyuki , Lacson Rudy , Figueroa Melquiades S. , Yamashina Tadashi , Ohkura Takahiro , Baloloy Alejo V. 
Published: December 2014

AbstractDuring a phreatic explosion at Mayon Volcano, Philippines, on 7 May 2013, a very long period seismic event with a peak frequency of 0.4 Hz was observed. Our frequency‐domain waveform inversion solution of the event in the frequency range 0.1–0.6 Hz is consistent with a subhorizontal tensile crack and a vertical single force at a shallow location beneath the summit crater. The source time functions obtained by the waveform inversion are band‐passed forms. We estimated the deconvolved forms of the source time functions (DSTFs), which are source time functions corrected for the effects of the band‐pass filter. The DSTF of the crack can be approximated by an impulse‐type function composed of inflation followed by deflation, whereas the DSTF of the single force can be approximated by a downward impulse. The inflation of the crack may be attributed to boiling of underground water and its deflation can be attributed to discharge of water vapor, whereas the downward force may be understood as the counterforce of the explosion. Our results suggest that only a portion of the crack wall was destroyed by the explosion. We could not find clear precursors in seismic, thermography, geothermal, geodetic, and meteorological data. We present a model of repeated explosions in which an explosion can occur once the fragmented portion of the crack is sealed by precipitation of clay minerals or hydrothermal secondary deposits. This model may explain the absence of clear precursory signals before the 2013 explosion.
Intermediate Filaments
Authors: Cheng Fang , Eriksson John E 
Published: December 2014

Abstract

Intermediate filaments (IFs) represent a diverse group of evolutionary conserved cytoskeletal structures, with context‐, tissue‐ and cell type‐dependent expression patterns and properties. Initially, IFs were assumed to provide only structural reinforcement in cells and tissues. However, recent research has rapidly expanded the knowledge of the functions of IF proteins, and the view of this complex gene family is progressively changing, as a multitude of diseases have been revealed to be associated with IF mutations. The molecular mechanisms underlying these disease conditions reflect disturbances in molecular processes reaching far beyond structural support. IFs are now known to be multifunctional organisers of cellular processes during all stages of life, from development to ageing, with critical functions in both homoeostasis and stress.

Key Concepts:

Intermediate filaments are flexible cytoskeletal and nucleoskeletal structures that assemble hierarchically.

Intermediate filaments comprise a diverse protein family expressed in a differentiation stage‐, cell‐ and tissue‐specific manner.

Intermediate filaments are functionally regulated by posttranslational modifications (PTMs).

Intermediate filaments integrate cell fate control with mechanosensing and three‐dimensional arrangement of cells and tissues.

Intermediate filaments are modulators of cell signalling and transcription.

Intermediate filaments are important cellular stress proteins that help in maintaining the cellular organisation and homoeostasis on injury.

Intermediate filaments act at the border between health and disease as critical guardian biopolymers, the failing of which contributes to a diverse range of pathologies.
Nucleotide Synthesis via Salvage Pathway
Authors: Nyhan William L 
Published: December 2014

AbstractThe biosynthesis of purine and pyrimidine nucleotides takes place overde novosynthetic pathways from small molecules and by salvage pathways from preformed purine or pyrimidine bases or nucleosides. The pathways ofde novosynthesis are the same in animals and microorganisms. Salvage pathways are considerably more energy‐efficient thande novopathways, which require 5 (pyrimidine) or 6 (purine) moles of ATP for each mole of nucleotide produced. Salvage pathways are integral to the cause or treatment of a number of human diseases of purine or pyrimidine metabolism. Among disorders of purine metabolism, the Lesch–Nyhan disease is characterised by overproduction of uric acid, clinical gout, nephropathy, neurologic disease, and unusual self‐injurious behaviours.Key Concepts:Salvage of purines is catalysed by adenine phosphoribosyltransferase (APRT) and hypoxanthine guanine phosphoribosyltransferase (HGPRT).Pyrimidine salvage is catalysed by thymidine kinase.Pyrimidine salvage is effective in the treatment of orotic aciduria, a disorder of pyrimidine nucleotide synthesis.Deficiency of APRT leads to renal calculi.Deficiency of HGPRT is the cause of Lesch–Nyhan disease.
Varicella Zoster: Virus and Disease
Authors: Dotzler Steven M , Grose Charles 
Published: December 2014

Abstract

Varicella‐zoster virus
(VZV) is a human‐specific virus that causes varicella (chickenpox) upon primary infection of children. During the convalescent stage of infection, VZV establishes latency in dorsal root ganglia of the nervous system and reactivates decades later in adults, causing herpes zoster (shingles). In the early 1970s, a live attenuated varicella vaccine was developed; subsequent varicella vaccination has greatly reduced VZV‐related hospitalisations and fatalities, although VZV remains commonplace in areas of the world that have not achieved high degrees of vaccination. VZV research continues to study viral mechanisms and pathogenesis. Recent experiments have discovered that VZV induces the cellular process called autophagy, which can aid in prolonging cell lifespan. Phylogenetic analyses of sequenced genomes indicate that the VZV genome originated in Africa and has coevolved with its human hosts during their migration out‐of‐Africa throughout the world.

Key Concepts:

Varicella‐zoster virus
causes chickenpox initially, but remains latent in the body and can be reactivated, usually in the elderly, to cause the infection known as shingles.

VZV is one of nine human herpesviruses; in 2012,
human herpesvirus type 6
was separated into two species called A and B. VZV is evolutionarily closely most related to the
herpes simplex virus
(HSV); however, it causes chickenpox and shingles and cannot cause herpes.

The double‐stranded DNA genome of VZV has been fully sequenced and has more than 70 open reading frames that encode nearly 70 unique proteins.

A highly geometrical and complex layer of proteins known as the capsid serves as a protective housing for the viral DNA; the capsid is then covered by a tegument and a lipid bilayer called the envelope.

Glycoproteins – proteins with bound sugars – are embedded within the envelope and serve functions from attachment to the host cell and cell‐to‐cell spread of the infection.

VZV is thought to have originated in Africa and has spread throughout the world by coevolving with its human host.

VZV is spread only during active infection; among the human herpesviruses, VZV is the only one that is spread by aerosolizing into airborne particles.

Autophagy, the recycling of damaged organelles, is induced by VZV as a method of prolonging cell lifespan, which gives the virus more time to replicate.

A live attenuated varicella vaccine was developed in the early 1970s and has dramatically reduced the number of VZV‐related hospitalisations and fatalities.

Antiviral drugs such as aciclovir are very effective in the treatment of VZV infections.
Molecular Genetics of Gitelman Syndrome
Authors: Colussi Giacomo , Tedeschi Silvana , Bettinelli Alberto , Syrén Marie Louise 
Published: December 2014

AbstractGitelman syndrome (OMIM263800) is an autosomal recessive renal tubular disorder due to loss‐of‐function mutations ofSLC12A3gene, encoding the thiazide‐inhibitable, electroneutral Na+‐Cl−cotransporter (NCC) of the distal convoluted tubule. Clinical consequences include chronic normotensive hypokalemic alkalosis, hypomagnesemia, hypocalciuria, polyuria/nocturia, chronic asthenia, muscular cramps, chondrocalcinosis and rarely cardiac arrhythmias.Impaired reabsorption of glomerular filtrate through NCC drives preferential reabsorption of Na+in more distal tubular segments via the ‘electrogenic’ channel ENaC, enhancing tubular secretion of potassium and protons, explaining the hypokalemic alkalosis.There exists wide variability and severity of symptoms between subjects, ranging from an almost asymptomatic disease to a severely disabling one. More than 400SLC12A3mutations have been so far described, evenly distributed along the protein sequence and without any hot spot. Mutation detection rate actually is approximately 80–90%. There are no genotype–phenotype correlations.Commonly considered a benign condition, Gitelman syndrome may be associated with reduced quality of life, increased medicalisation and high hospitalisation rate.Key Concepts:Gitelman syndrome is the clinical and biochemical manifestation of impaired function of the electroneutral Na‐Cl cotransporter of the renal distal convoluted tubule, the target of the thiazide class of diuretics.Homozygous and compound heterozygous loss‐of‐function mutations of theSLC12A3gene cause the disease; more than 400 inactivating mutations (mostly missense mutations) have been identified, without any preferential target.Main biochemical abnormalities include hypokalemia, mild metabolic alkalosis, hypomagnesemia, hyperreninemia and hypocalciuria.Clinical manifestations include muscular cramps/tetanic crisis, asthenia, polyuria/nocturia and chondrocalcinosis; normal/low blood pressure is an important feature in the differential diagnosis with hypertensive hypokalemic disorders.Gitelman syndrome cannot be presently cured, and therapy aims at correcting plasma potassium and possibly magnesium levels with supplemental oral potassium and magnesium, aldosterone receptor antagonists and the sodium channel blocker amiloride. Intravenous infusions of potassium and magnesium are needed in acute crisis and stressful contexts.Long‐term prognosis appears good and long‐term renal function preserved, but quality of life may be somehow impaired and medicalisation/hospitalisation rate increased compared to the general population.
Exploring the Relationship Between Mathematics Anxiety and Performance: An Eye‐Tracking Approach
Authors: Hunt Thomas E. , Clark‐Carter David , Sheffield David 
Published: December 2014

SummaryThe mechanisms underpinning the relationship between math anxiety and arithmetic performance are not fully understood. This study used an eye‐tracking approach to measure a range of eye movements of 78 undergraduate students in response to performance on an arithmetic verification task. Results demonstrated a significant positive relationship between self‐reported math anxiety and response time, indicating reduced processing efficiency. Analysis of eye‐movement data reinforced the utility of an eye‐tracking approach in studying arithmetic performance; specific digit fixations, dwell time, saccades, and regressions all significantly predicted response time. Furthermore, findings highlighted significant positive correlations between math anxiety and fixations, dwell time, and saccades. Despite there being little evidence that eye movements mediate the math anxiety‐to‐performance relationship, relationships observed between math anxiety and eye movements provide a useful starting point for research using an eye‐tracking methodology in studying math anxiety and performance; the present findings suggest future work should focus on calculation strategy. Copyright © 2014 John Wiley & Sons, Ltd.
Contribution of the Subjective Components of the Disease Activity Score to the Response to Biologic Treatment in Rheumatoid Arthritis
Authors: Jurgens Maud S. , Overman Cécile L. , Jacobs Johannes W. G. , Geenen Rinie , Cuppen Bart V. J. , Marijnissen Anne C. A. , Bijlsma Johannes W. J. , Welsing Paco M. J. , Lafeber Floris P. J. G. , van Laar Jacob M. ,   on behalf of the Utrecht Arthritis Cohort Study Group
Published: December 2014

ObjectiveA significant proportion of patients with rheumatoid arthritis do not respond adequately to biologic treatment. We hypothesized that lack of response to (biologic) disease‐modifying antirheumatic drugs (DMARDs) is high in patients in whom the subjective, patient‐reported component of the Disease Activity Score 28 (DAS28) is high at baseline. The primary aim of our present study was to investigate the contribution of the more subjective versus the objective components of the DAS28 to response to biologic agents in RA patients, as well as the changes in this contribution over time. The secondary aim was to examine whether the value of this subjective contribution at baseline affects the response to treatment.MethodsThe DAS28‐P (the subjective components of the DAS28 relative to the total DAS28) was calculated. Patients were derived from the computer‐assisted Management in Early Rheumatoid Arthritis Trial‐II and the Biologicals and Outcome Compared and Predicted in Utrecht Region in Rheumatoid Arthritis Study. Ordinal logistic regression analyses were performed.ResultsThe DAS28‐P score at baseline was not associated with the level of response according to European League Against Rheumatism criteria at 3 months. Overall, a significant reduction in the DAS28‐P score was observed 3 months after start of treatment, showing a greater reduction of the combined subjective components in good responders.ConclusionThe results reject the hypothesis that the lack of response to biologic DMARDs is especially high in patients in whom the patient‐reported component of the DAS28 is high at baseline; these subjective components are not linked to treatment response.
Endoscopic direct vessel closure in carotid artery injury
Authors: Padhye Vikram , Murphy Jae , Bassiouni Ahmed , Valentine Rowan , Wormald Peter‐John 
Published: December 2014

BackgroundInternal carotid artery (ICA) injury represents one of the most challenging management scenarios for the endoscopic skull base surgeon. Techniques developed through use of an animal model of carotid injury have shown direct vessel closure techniques to be effective in gaining hemostasis and preventing subsequent complications. The aim of this study was to investigate the effectiveness of the novel AnastoClip vessel closure system in ICA injury.MethodsNine sheep underwent ICA dissection/isolation followed by the artery placement within a modified “sinus model otorhino neuro trainer” (SIMONT) model. Standardized linear injuries were made and treated endoscopically with the AnastoClip device. Specific outcome measures included attainment of primary hemostasis, procedure time, blood loss, pseudoaneurysm formation and carotid patency on follow‐up magnetic resonance imaging (MRI).ResultsPrimary hemostasis was achieved in all cases. No instances of secondary bleeding. Procedure times averaged 5 minutes and 25 seconds. Average blood loss was 146 mL. All 9 sheep reached the 3‐month end point. Eight showed normal carotid flow on MRI, with 1 case of pseudoaneurysm.ConclusionDirect vessel closure is an effective endoscopic technique in ICA injury. It allows for visual confirmation of hemostasis and carotid flow and permits the surgeon to continue with surgery despite the complication. The novel design of the AnastoClip can maintain normal vessel patency as well as accommodate pulsatile flow.
Ionic Liquids as Precursors for Efficient Mesoporous Iron‐Nitrogen‐Doped Oxygen Reduction Electrocatalysts
Authors: Li Zelong , Li Guanglan , Jiang Luhua , Li Jinlei , Sun Gongquan , Xia Chungu , Li Fuwei 
Published: December 2014

AbstractA ferrocene‐based ionic liquid (Fe‐IL) is used as a metal‐containing feedstock with a nitrogen‐enriched ionic liquid (N‐IL) as a compatible nitrogen content modulator to prepare a novel type of non‐precious‐metal–nitrogen–carbon (M‐N‐C) catalysts, which feature ordered mesoporous structure consisting of uniform iron oxide nanoparticles embedded into N‐enriched carbons. The catalyst Fe10@NOMC exhibits comparable catalytic activity but superior long‐term stability to 20 wt % Pt/C for ORR with four‐electron transfer pathway under alkaline conditions. Such outstanding catalytic performance is ascribed to the populated Fe (Fe3O4) and N (N2) active sites with synergetic chemical coupling as well as the ordered mesoporous structure and high surface area endowed by both the versatile precursors and the synthetic strategy, which also open new avenues for the development of M‐N‐C catalytic materials.
Decarbonylative Radical Coupling of α‐Aminoacyl Tellurides: Single‐Step Preparation of γ‐Amino and α,β‐Diamino Acids and Rapid Synthesis of Gabapentin and Manzacidin A
Authors: Nagatomo Masanori , Nishiyama Hayato , Fujino Haruka , Inoue Masayuki 
Published: December 2014

AbstractA new radical‐based coupling method has been developed for the single‐step generation of various γ‐amino acids and α,β‐diamino acids from α‐aminoacyl tellurides. Upon activation by Et3B and O2 at ambient temperature, α‐aminoacyl tellurides were readily converted into α‐amino carbon radicals through facile decarbonylation, which then reacted intermolecularly with acrylates or glyoxylic oxime ethers. This mild and powerful method was effectively incorporated into expeditious synthetic routes to the pharmaceutical agent gabapentin and the natural product (−)‐manzacidin A.
A Method for Dynamic Nuclear Polarization Enhancement of Membrane Proteins
Authors: Smith Adam N. , Caporini Marc A. , Fanucci Gail E. , Long Joanna R. 
Published: December 2014

AbstractDynamic nuclear polarization (DNP) magic‐angle spinning (MAS) solid‐state NMR (ssNMR) spectroscopy has the potential to enhance NMR signals by orders of magnitude and to enable NMR characterization of proteins which are inherently dilute, such as membrane proteins. In this work spin‐labeled lipid molecules (SL‐lipids), when used as polarizing agents, lead to large and relatively homogeneous DNP enhancements throughout the lipid bilayer and to an embedded lung surfactant mimetic peptide, KL4. Specifically, DNP MAS ssNMR experiments at 600 MHz/395 GHz on KL4 reconstituted in liposomes containing SL‐lipids reveal DNP enhancement values over two times larger for KL4 compared to liposome suspensions containing the biradical TOTAPOL. These findings suggest an alternative sample preparation strategy for DNP MAS ssNMR studies of lipid membranes and integral membrane proteins.
Synergetic Spin Crossover and Fluorescence in One‐Dimensional Hybrid Complexes
Authors: Wang Chun‐Feng , Li Ren‐Fu , Chen Xue‐Yuan , Wei Rong‐Jia , Zheng Lan‐Sun , Tao Jun 
Published: December 2014

AbstractHybrid materials integrated with a variety of physical properties, such as spin crossover (SCO) and fluorescence, may show synergetic effects that find applications in many fields. Herein we demonstrate a promising post‐synthetic approach to achieve such materials by grafting fluorophores (1‐pyrenecarboxaldehyde and Rhodamine B) on one‐dimensional SCO FeII structures. The resulting hybrid materials display expected one‐step SCO behavior and fluorescent properties, in particular showing a coupling between the transition temperature of SCO and the temperature where the fluorescent intensity reverses. Consequently, synergetic effect between SCO and fluorescence is incorporated into materials despite different fluorophores. This study provides an effective strategy for the design and development of novel magnetic and optical materials.