bluuebunny/crossref_metadata_2025_split huggingface dataset:
La formation à distance sélectionne un public d'autodidactes : résultats réflexifs à partir d'une enquête à visée exploratoire
Authors: Albero Brigitte , Kaiser Arnim 
Published: October 2010

Résumé La formation ouverte et à distance est généralement perçue comme une modalité hybride, susceptible de s’adapter à tout type de public. Une enquête auprès d’usagers adultes a mis en valeur un phénomène de sélection implicite dû aux caractéristiques mêmes du dispositif. Cette contribution présente : 1) les conditions méthodologiques de l’enquête et l’analyse de ses résultats ; 2) le cadre théorique qui permet de comprendre le phénomène et d’envisager d’autres conceptions de cette réalité sociale ; 3) les propositions dégagées par les auteurs en termes de principes d’action pour le terrain de la formation.
L'activité des dirigeants de la fonction publique territoriale, une recherche en analyse de l'activité
Authors: Durat Laurence 
Published: October 2010

Résumé Un programme d’études original a été mis sur pied pour analyser l’activité des dirigeants de plusieurs secteurs de la sphère publique (Cnam-Resp). Cette recherche s’est donné pour objectif de collecter les données par l’observation de l’activité des dirigeants en cours d’activité, in situ , par le recueil de traces et de documents ainsi que le recueil des discours. Une partie des résultats de l’étude sur des dirigeants de la fonction publique territoriale est présentée ici. Il en ressort notamment que le dirigeant opère un travail constant de recherche d’équilibres relatifs aux situations qu’il rencontre et à ses partenaires dans l’activité, et élabore la structure conceptuelle de la situation. Pour agir sur autrui et transformer les processus en cours, il intervient grâce à deux concepts organisateurs : l’appréciation du processus managérial et la mesure de turbulence potentielle.
Les applications du sentiment d'efficacité personnelle
Authors: Lecomte Jacques 
Published: October 2010

Résumé La théorie du sentiment d’efficacité personnelle présente une grande ampleur, tant sur le plan théorique, que sur celui de ses applications pratiques. Après avoir présenté les principales composantes de cette théorie, l’auteur en décrit les applications possibles dans trois domaines : la réussite scolaire, le travail professionnel, la psychothérapie. Il propose, en conclusion, une voie de recherche encore inexplorée.
Analysis and Interpretation of Microstructures after Liquid Oxide Corrosion
Authors: Poirier Jacques 
Published: October 2010

Corrosion by liquid oxides is one of the most severe modes of degradations which limit the lifetime of the refractory linings. The study of the microstructures of corroded refractories provides essential information. However, the interpretation of the microscopic observations is difficult :
-	The refractories are multi-component and heterogeneous ceramics,
-	The microscopic observations are carried out at room temperature. They are not representative of the mineral and vitreous phases existing at high temperature,
-	During cooling, new solid phases appear by crystallization of liquid oxides. The composition of the vitreous phases also evolves with the temperature.
Consequently, the information obtained is often limited.
In this paper , we will present a method to analyse and interpret the microstructures of refractories after use. The concept of local thermodynamic equilibrium and the use of the phase rule make it possible to interpret the microstructures of corroded refractories, to explain the observed mineral zonation and to quantify the composition of the liquid phase at high temperature from chemical profiles established by S.E.M.
Experimental data from corrosion of MgO-C, Al2O3-MgO and high alumina refractories will illustrate and validate this theoretical approach.
Hepatitis C virus–associated polyarteritis nodosa
Authors: Saadoun D. , Terrier B. , Semoun O. , Sene D. , Maisonobe T. , Musset L. , Amoura Z. , Rigon M. Resche , Cacoub P. 
Published: October 2010

AbstractObjectiveTo analyze the main characteristics and outcome of polyarteritis nodosa (PAN)–type vasculitis associated with hepatitis C virus (HCV).MethodsWe reported the characteristics and outcome of 31 patients chronically infected with HCV who satisfied the American College of Rheumatology and Chapel Hill criteria for PAN, seen between 1990 and 2009 in a university center.ResultsAmong a cohort of 161 patients with HCV‐related vasculitis, 31 (19.3%) were diagnosed as having PAN. The median age was 64.5 years (interquartile range 49.5–70.5 years), with 54.8% women. Compared with HCV‐associated mixed cryoglobulinemia (HCV‐MC) vasculitis, HCV‐PAN displayed a more severe and acute clinical presentation with more frequent fever and weight loss (P < 0.0001), severe hypertension (P = 0.0006), gastrointestinal tract involvement (P < 0.0001), severe acute sensory‐motor multifocal mononeuropathy (P < 0.0001), kidney and liver microaneurysms (P = 0.002), and increased C‐reactive protein level (P < 0.0001). Complete clinical remission of vasculitis was achieved in 79.3% of HCV‐PAN patients compared to 57.5% of HCV‐MC patients (P = 0.05). In multivariate analysis, skin involvement (odds ratio [OR] 2.81, 95% confidence interval [95% CI] 1.27–6.33) and PAN‐type vasculitis (OR 3.01, 95% CI 1.16–8.96) were independently associated with a complete clinical response of HCV vasculitis. A glomerular filtration rate <70 ml/minute (OR 0.54, 95% CI 0.24–1.21) was negatively associated with a complete clinical response of HCV vasculitis. The 5‐year survival rate was 86% in the entire cohort, regardless of the vasculitis type.ConclusionHCV‐PAN accounts for 19.3% of our cohort of HCV vasculitis. HCV‐PAN displays a more severe and acute clinical presentation and a higher rate of clinical remission.
Towards Greener Fluorine Organic Chemistry: Direct Electrophilic Fluorination of Carbonyl Compounds in Water and Under Solvent‐Free Reaction Conditions
Authors: Stavber Gaj , Stavber Stojan 
Published: October 2010

AbstractSelective and efficient fluorination of organic 1,3‐dicarbonyl compounds was achieved using the electrophilic fluorinating reagentsSelectfluorTMF‐TEDA‐BF4(1‐chloromethyl‐4‐fluoro‐1,4‐diazoniabicyclo[2.2.2]octane bis‐tetrafluoroborate) in aqueous medium orAccufluorTMNFSi(N‐fluorobenzenesulfonimide) under solvent‐free reaction conditions (SFRC). Under both reaction conditions cyclic 1,3‐dicarbonyl compounds were transformed into 2‐fluoro‐substituted derivatives and acyclic analogues into 2,2‐difluoro‐substituted compounds, while the reactions of 1‐trifluoromethyl‐substituted 1,3‐dicarbonyls in water resulted in the formation of 2,2‐difluoro‐3,3‐dihydroxy‐1‐one derivatives. The reactivity of the starting material in water was found to be dependent on its enolizability, hydrophobic interactions and aggregate state at the reaction temperature. Reactions under SFRC proceeded in the molten eutectic phase of the reactants. The technique of competitive reactivity was used in order to evaluate and better understand the effects of reaction conditions on the course of these reactions.
Recyclable Polymer‐Supported Iodobenzene‐Mediated Electrocatalytic Fluorination in Ionic Liquid
Authors: Sawamura Takahiro , Kuribayashi Shunsuke , Inagi Shinsuke , Fuchigami Toshio 
Published: October 2010

AbstractThe electrochemical fluorination of organosulfur compounds in triethylamine/hydrofluoric acid (Et3N‐5 HF) with polystyrene‐supported iodobenzene (PSIB) and tetraethylammonium chloride (Et4NCl) was performed successfully in an undivided cell under constant current conditions to afford the corresponding fluorinated compounds in moderate to good yields. Recycle use of the PSIB could be achieved due to its easy separation. Notably, the mediatory activity of the iodobenzene derivative was not appreciably changed even after 10 recycle uses.
Costello syndrome with severe cutis laxa and mosaic HRAS G12S mutation
Authors: Girisha Katta M. , Lewis Leslie E. , Phadke Shubha R. , Kutsche Kerstin 
Published: October 2010

AbstractCostello syndrome is a rare developmental disorder characterized by coarse face, postnatal growth retardation, skin and musculoskeletal anomalies, cardiovascular abnormalities, mental retardation, and tumor predisposition. Dermatological manifestations usually include redundant, soft and thickened skin. Loose skin is especially present over the neck, hands, and feet. Heterozygous missense mutations in HRAS are causative for Costello syndrome, with the c.34G > A (p.G12S) mutation as the most commonly found alteration. In the majority of affected individuals pathogenic sequence changes appeared de novo, however, two individuals with somatic mosaicism for the HRAS mutation have been reported. Here, we describe a boy with somatic mosaicism for the c.34G > A mutation in HRAS. Allelic quantitation revealed the mutation in approximately 58% of his lymphocytes; however, in DNA derived from buccal cells we could not detect the sequence change. The patient presented with the typical clinical findings of Costello syndrome such as increased birth weight, severe failure to thrive, characteristic facial appearance, and skin abnormalities. The dermatological anomalies were remarkable as he showed severe skin laxity with wrinkling of skin on all parts of the body due to loss of subcutaneous fat that decreased significantly by age 13 months. This case further adds to the phenotypic variability seen in patients with somatic mosaicism for an HRAS mutation and highlights the awareness of mosaic mutations in Costello syndrome when molecular testing is performed. © 2010 Wiley‐Liss, Inc.
Marfan syndrome with neonatal progeroid syndrome‐like lipodystrophy associated with a novel frameshift mutation at the 3′ terminus of the FBN1‐gene
Authors: Graul‐Neumann Luitgard M. , Kienitz Tina , Robinson Peter N. , Baasanjav Sevjidmaa , Karow Benjamin , Gillessen‐Kaesbach Gabriele , Fahsold Raimund , Schmidt Hartmut , Hoffmann Katrin , Passarge Eberhard 
Published: October 2010

AbstractWe report on a 25‐year‐old woman with pronounced generalized lipodystrophy and a progeroid aspect since birth, who also had Marfan syndrome (MFS; fulfilling the Ghent criteria) with mild skeletal features, dilated aortic bulb, dural ectasia, bilateral subluxation of the lens, and severe myopia in addition to the severe generalized lipodystrophy. She lacked insulin resistance, hypertriglyceridemia, hepatic steatosis, and diabetes. Mutation analysis in the gene encoding fibrillin 1 (FBN1) revealed a novel de novo heterozygous deletion, c.8155_8156del2 in exon 64. The severe generalized lipodystrophy in this patient with progeroid features has not previously been described in other patients with MFS and FBN1 mutations. We did not find a mutation in genes known to be associated with congenital lipodystrophy (APGAT2, BSCL2, CAV1, PTRF‐CAVIN, PPARG, LMNB2) or with Hutchinson–Gilford progeria (ZMPSTE24, LMNA/C). Other progeria syndromes were considered unlikely because premature greying, hypogonadism, and scleroderma‐like skin disease were not present. Our patient shows striking similarity to two patients who have been published in this journal by O'Neill et al. [O'Neill et al. (2007); Am J Med Genet Part A 143A:1421–1430] with the diagnosis of neonatal progeroid syndrome (NPS). This condition also known as Wiedemann–Rautenstrauch syndrome is a rare disorder characterized by accelerated aging and lipodystrophy from birth, poor postnatal weight gain, and characteristic facial features. The course is usually progressive with early lethality. However this entity seems heterogeneous. We suggest that our patient and the two similar cases described before represent a new entity, a subgroup of MFS with overlapping features to NPS syndrome. © 2010 Wiley‐Liss, Inc.
Autism spectrum features in Smith–Magenis syndrome
Authors: Laje Gonzalo , Morse Rebecca , Richter William , Ball Jonathan , Pao Maryland , Smith Ann C.M. 
Published: October 2010

AbstractSmith–Magenis syndrome (SMS; OMIM 182290) is a neurodevelopmental disorder characterized by a well‐defined pattern of anomalies. The majority of cases are due to a common deletion in chromosome 17p11.2 that includes the RAI1 gene. In children with SMS, autistic‐like behaviors and symptoms start to emerge around 18 months of age. This study included 26 individuals (15 females and 11 males), with a confirmed deletion (del 17p11.2). Parents/caregivers were asked to complete the Social Responsiveness Scale (SRS) and the Social Communication Questionnaire (SCQ) both current and lifetime versions. The results suggest that 90% of the sample had SRS scores consistent with autism spectrum disorders. Moreover, females showed more impairment in total T‐scores (P = 0.02), in the social cognition (P = 0.01) and autistic mannerisms (P = 0.002) subscales. The SCQ scores are consistent to show that a majority of individuals may meet criteria for autism spectrum disorders at some point in their lifetime. These results suggest that SMS needs to be considered in the differential diagnosis of autism spectrum disorders but also that therapeutic interventions for autism are likely to benefit individuals with SMS. The mechanisms by which the deletion of RAI1 and contiguous genes cause psychopathology remain unknown but they provide a solid starting point for further studies of gene–brain–behavior interactions in SMS and autism spectrum disorders. Published 2010 Wiley‐Liss, Inc.
Mouse models of cognitive disabilities in trisomy 21 (Down syndrome)
Authors: Roubertoux Pierre L. , Carlier Michèle 
Published: October 2010

AbstractTrisomy 21 (TRS21), also referred to as Down syndrome, occurs once in every 800–1,000 live births. It is the consequence of an extra copy of HSA21 that causes an imbalanced gene dose effect. TRS21 is the first known genetic cause of cognitive disability. The syndrome is complex, and includes various cardiac, immune, and bone disorders. Most of these signs are highly variable in expression but cognitive disability is the most constant characteristic of persons with TRS21. The syntenies that exist between HSA21 and three mouse chromosomes (MMU10, MMU16, and MMU17) offer the opportunity for a genotype–phenotype correlation. We present here the segmental trisomies available in the mouse and we discuss their contribution to the brain and cognitive phenotypes of TRS21. © 2010 Wiley‐Liss, Inc.
The behavioral phenotype of the idic(15) syndrome
Authors: Battaglia Agatino , Parrini Barbara , Tancredi Raffaella 
Published: October 2010

AbstractIdic(15) syndrome is a neurogenetic disorder clinically delineated by early central hypotonia, developmental delay and intellectual disability (ID), epilepsy, absent or very poor speech, and autistic or autistic‐like behavior. It is due to the presence of a supernumerary marker chromosome formed by the inverted duplication of proximal chromosome 15, resulting in tetrasomy 15p and partial tetrasomy 15q, and containing the Prader–Willi/Angelman syndrome critical region (PWS/ASCR). The vast majority of these idic(15) derives from the two homologous maternal chromosomes at meiosis. To better define the behavior profile, we studied 22 idic(15) children (15 males and 7 females) observed at our institute between 1986 and 2010, and present, in detail, case studies of five of them. We have been able to perform standardized and semi‐standardized measures of intelligence, and psychopathology in only 13 of our 22 patients, due to the limitations of chronological age, and to the severity of ID (ranging from mild–moderate, in 15%, to severe–profound, in 85% of our sample). The results show a distinct developmental profile in idic(15) patients, that may provide a behavioral signature for autism spectrum disorder (ASD)/ASD‐like arising from the susceptibility locus on proximal 15q; and suggest that idic(15) individuals are not “true autistic,” but distinct “autistic‐like” persons with high score in the third ADOS‐G and ADI‐R area. © 2010 Wiley‐Liss, Inc.
A new species of snub‐nosed monkey, genus Rhinopithecus Milne‐Edwards, 1872 (Primates, Colobinae), from northern Kachin state, northeastern Myanmar
Authors: Geissmann Thomas , Lwin Ngwe , Aung Saw Soe , Aung Thet Naing , Aung Zin Myo , Hla Tony Htin , Grindley Mark , Momberg Frank 
Published: October 2010

AbstractWe describe a snub‐nosed monkey that is new to science from the high altitudes of northeastern Kachin state, northeastern Myanmar, the Burmese snub‐nosed monkey, Rhinopithecus strykeri sp. nov. Descriptions are based on a skin and skulls of four specimens obtained from local hunters. The new species is geographically isolated from other snub‐nosed monkeys and separated from them by two major barriers—the Mekong and the Salween (Thanlwin) rivers. The species is chiefly diagnosed by its almost entirely blackish fur coloration with white fur only on ear tufts, chin beard, and perineal area, and its relatively long tail (140% of head and body length in the adult male). Preliminary surveys and interviews with hunters indicate that the new species is limited in distribution to the Maw River area, a small region of the Salween‐N'mai Hka divide in northeastern Kachin state, northeastern Myanmar. The distribution area appears to cover about 270 km2, and the species may consist of only three groups with a total population of approximately 260–330 individuals. Our data on hunting pressure suggest that the species is Critically Endangered. Am. J. Primatol. 73:96–107, 2011. © 2010 Wiley‐Liss, Inc.
Systematic review and meta-analysis of intraperitoneal local anaesthetic for pain reduction after laparoscopic gastric procedures
Authors: Kahokehr A , Sammour T , Srinivasa S , Hill A G 
Published: October 2010

AbstractBackgroundWith the advent of minimally invasive gastric surgery, visceral nociception has become an important area of investigation as a potential cause of postoperative pain. A systematic review and meta-analysis was carried out to investigate the clinical effects of intraperitoneal local anaesthetic (IPLA) in laparoscopic gastric procedures.MethodsComprehensive searches were conducted independently without language restriction. Studies were identified from the following databases from inception to February 2010: Cochrane Central Register of Controlled Trials, the Cochrane Library, MEDLINE, PubMed, Embase and CINAHL. Relevant meeting abstracts and reference lists were searched manually. Appropriate methodology according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement was adhered to.ResultsFive randomized controlled trials in laparoscopic gastric procedures were identified for review. There was no significant heterogeneity between the trials (χ2 = 10·27, 10 d.f., P = 0·42, I2 = 3 per cent). Based on meta-analysis of trials, there appeared to be reduced abdominal pain intensity (overall mean difference in pain score − 1·64, 95 per cent confidence interval (c.i.) − 2·09 to − 1·19; P &lt; 0·001), incidence of shoulder tip pain (overall odds ratio 0·15, 95 per cent c.i. 0·05 to 0·44; P &lt; 0·001) and opioid use (overall mean difference − 3·23, − 4·81 to − 1·66; P &lt; 0·001).ConclusionThere is evidence in favour of IPLA in laparoscopic gastric procedures for reduction of abdominal pain intensity, incidence of shoulder pain and postoperative opioid consumption.
Mouse granulosa cells contribute more to the mRNA synthesis of mZP2 than oocyte does
Authors: Xie Yan , Yu Yanhong , Nie Chuncheng , Cao Zuowu 
Published: October 2010

AbstractIn order to elucidate the biogenesis of mouse zona pellucida 2 (mZP2) protein, RT‐PCR, and in situ hybridization were carried out to localize the expression of mouse ZP2 mRNA. Cumulus cells of the OCC (Oocyte‐Cumulus cell Complex) were isolated from the oocytes after superovulation for the RNA extraction. The frozen sections of ovaries from adolescent and aged mice were prepared to hybridize with RNA probe of mouse ZP2. mRNA of ZP2 was detected in isolated cumulus cells by RT‐PCR. Results of in situ hybridization showed that the mRNA of ZP2 was synthesized in both oocyte and granulosa cells at different folliculogenesis stages; and the expression of ZP2 mRNA in granulosa cells was stronger than that in oocyte; much weaker expression of mZP2 was detected in the follicles of aged mouse. These suggest that the entire amount of ZP2 mRNA generated in the granulosa cells layer should be much more than that in oocyte. Therefore, we think that the granulosa cells contribute more to the mZP2 mRNA synthesis than oocyte does. Copyright © 2010 John Wiley & Sons, Ltd.
A Fluorescence Selection Method for Accurate Large‐Gene Synthesis
Authors: Kim Hwangbeom , Han Hyojun , Shin Dongyun , Bang Duhee 
Published: October 2010

AbstractThe fundamental problem for low‐cost gene synthesis is errors that occur during the synthetic process. To address this problem, we developed a practical method that exploits the fact that the predominant errors are deletions. In this method, a simple fluorescence‐based readout was used to distinguish error‐free synthetic DNA molecules. To do this, we constructed vectors that contained multiple cloning sites and GFP. In the vectors, the GFP gene is designed to be out‐of‐frame, but insertion of an in‐framed synthetic DNA construct into the appropriate cloning site will lead to fluorescent cell colonies. We successfully used this method to synthesize five genes and improved the bp per error from 629 to 6552 by selecting green fluorescent colonies.
Semiparametric median residual life model and inference
Authors: Ma Yanyuan , Yin Guosheng 
Published: October 2010

AbstractFor randomly censored data, the authors propose a general class of semiparametric median residual life models. They incorporate covariates in a generalized linear form while leaving the baseline median residual life function completely unspecified. Despite the non‐identifiability of the survival function for a given median residual life function, a simple and natural procedure is proposed to estimate the regression parameters and the baseline median residual life function. The authors derive the asymptotic properties for the estimators, and demonstrate the numerical performance of the proposed method through simulation studies. The median residual life model can be easily generalized to model other quantiles, and the estimation method can also be applied to the mean residual life model.The Canadian Journal of Statistics38: 665–679; 2010 © 2010 Statistical Society of Canada