591 research outputs found
ReprÊsentations d'Êlèves de 4e et de 5e secondaire à propos des pratiques de lecture et d'Êcriture en français et de leurs compÊtences en lecture et en Êcriture
Si des recherches ont montrĂŠ que plusieurs ĂŠlèves ont des difficultĂŠs avec la maitrise des aspects formels de lâĂŠcrit, peu dâentre elles documentent ce qui est fait en classe pour dĂŠvelopper leurs compĂŠtences en lecture et en ĂŠcriture et informent sur leurs reprĂŠsentations sur ces compĂŠtences. Notre recherche vise dâabord Ă brosser un portrait des pratiques de lecture et dâĂŠcriture en français telles que dĂŠcrites par les ĂŠlèves, puis Ă dĂŠcrire et comprendre leurs reprĂŠsentations sur leurs compĂŠtences Ă lâĂŠcrit. Pour ce faire, nous avons analysĂŠ et croisĂŠ des donnĂŠes issues de lâenquĂŞte Ătat des lieux de lâenseignement du français avec celles issues de sept entretiens avec des ĂŠlèves de 4e secondaire. Lâanalyse rĂŠvèle que nos sujets ont une conscience ĂŠtonnante des processus cognitifs qui sous-tendent la lecture et lâĂŠcriture et quâils suggèrent des pistes intĂŠressantes pour lâenseignement qui sâapparentent souvent Ă ce que proposent les recherches en didactique du français
La langue française au QuĂŠbec : perceptions du prĂŠsent et visions dâavenir dâĂŠlèves de 4e et de 5e secondaire
La MinienquĂŞte sur le français au QuĂŠbec poursuivait trois objectifs : comprendre les reprĂŠsentations sur la langue française de jeunes QuĂŠbĂŠcois âgĂŠs de 15 Ă 17 ans, dĂŠcrire leurs pratiques culturelles et observer lâinfl uence du milieu socioculturel et de la langue parlĂŠe Ă la maison sur ces deux aspects. De cette recherche mettant Ă contribution des donnĂŠes issues des rĂŠponses de 349 ĂŠlèves Ă un questionnaire anonyme, et des propos de 23 jeunes en groupes de discussion, il ressort que les ĂŠlèves utilisent le français dans certaines activitĂŠs de dĂŠtente; ont des opinions mitigĂŠes quant Ă lâusage du français dansleur milieu et entretiennent une vision plutĂ´t pessimiste de lâavenir de cette langue quâils disent vouloir utiliser dans leur vie future, quâils imaginent caractĂŠrisĂŠe par la mobilitĂŠ et le bilinguisme
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ARHGEF9 disease: Phenotype clarification and genotype-phenotype correlation.
ObjectiveWe aimed to generate a review and description of the phenotypic and genotypic spectra of ARHGEF9 mutations.MethodsPatients with mutations or chromosomal disruptions affecting ARHGEF9 were identified through our clinics and review of the literature. Detailed medical history and examination findings were obtained via a standardized questionnaire, or if this was not possible by reviewing the published phenotypic features.ResultsA total of 18 patients (including 5 females) were identified. Six had de novo, 5 had maternally inherited mutations, and 7 had chromosomal disruptions. All females had strongly skewed X-inactivation in favor of the abnormal X-chromosome. Symptoms presented in early childhood with delayed motor development alone or in combination with seizures. Intellectual disability was severe in most and moderate in patients with milder mutations. Males with severe intellectual disability had severe, often intractable, epilepsy and exhibited a particular facial dysmorphism. Patients with mutations in exon 9 affecting the protein's PH domain did not develop epilepsy.ConclusionsARHGEF9 encodes a crucial neuronal synaptic protein; loss of function of which results in severe intellectual disability, epilepsy, and a particular facial dysmorphism. Loss of only the protein's PH domain function is associated with the absence of epilepsy
MYH7 p.(Arg1712Gln) is pathogenic founder variant causing hypertrophic cardiomyopathy with overall relatively delayed onset
Introduction: The MYH7 c.5135GâŻ> A p.(Arg1712Gln) variant has been identified in several patients worldwide and is classified as pathogenic in the ClinVar database. We aimed to delineate its associated phenotype and evaluate a potential founder effect.Methods: We retrospectively collected clinical and genetic data of 22 probands and 74 family members from an international cohort.Results: In total, 53 individuals carried the MYH7 p.(Arg1712Gln) variant, of whom 38 (72%) were diagnosed with hypertrophic cardiomyopathy (HCM). Mean age at HCM diagnosis was 48.8 years (standard deviation: 18.1; range: 8â74). The clinical presentation ranged from asymptomatic HCM to arrhythmias (atrial fibrillation and malignant ventricular arrhythmias). Aborted sudden cardiac death (SCD) leading to the diagnosis of HCM occurred in one proband at the age of 68 years, and a family history of SCD was reported by 39% (5/13) probands. Neither heart failure deaths nor heart transplants were reported. Women had a generally later-onset disease, with 14% of female carriers diagnosed with HCM at age 50 years compared with 54% of male carriers. In both sexes, the disease was fully penetrant by age 75 years. Haplotypes were reconstructed for 35 patients and showed a founder effect in a subset of patients.Conclusion: MYH7 p.(Arg1712Gln) is a pathogenic founder variant with a consistent HCM phenotype that may present with delayed penetrance. This suggested that clinical follow-up should be pursued after the seventh decade in healthy carriers and that longer intervals between screening may be justified in healthy women <âŻ30 years.</p
Search for the standard model Higgs boson in the H to ZZ to 2l 2nu channel in pp collisions at sqrt(s) = 7 TeV
A search for the standard model Higgs boson in the H to ZZ to 2l 2nu decay
channel, where l = e or mu, in pp collisions at a center-of-mass energy of 7
TeV is presented. The data were collected at the LHC, with the CMS detector,
and correspond to an integrated luminosity of 4.6 inverse femtobarns. No
significant excess is observed above the background expectation, and upper
limits are set on the Higgs boson production cross section. The presence of the
standard model Higgs boson with a mass in the 270-440 GeV range is excluded at
95% confidence level.Comment: Submitted to JHE
Combined search for the quarks of a sequential fourth generation
Results are presented from a search for a fourth generation of quarks
produced singly or in pairs in a data set corresponding to an integrated
luminosity of 5 inverse femtobarns recorded by the CMS experiment at the LHC in
2011. A novel strategy has been developed for a combined search for quarks of
the up and down type in decay channels with at least one isolated muon or
electron. Limits on the mass of the fourth-generation quarks and the relevant
Cabibbo-Kobayashi-Maskawa matrix elements are derived in the context of a
simple extension of the standard model with a sequential fourth generation of
fermions. The existence of mass-degenerate fourth-generation quarks with masses
below 685 GeV is excluded at 95% confidence level for minimal off-diagonal
mixing between the third- and the fourth-generation quarks. With a mass
difference of 25 GeV between the quark masses, the obtained limit on the masses
of the fourth-generation quarks shifts by about +/- 20 GeV. These results
significantly reduce the allowed parameter space for a fourth generation of
fermions.Comment: Replaced with published version. Added journal reference and DO
Search for a W' boson decaying to a bottom quark and a top quark in pp collisions at sqrt(s) = 7 TeV
Results are presented from a search for a W' boson using a dataset
corresponding to 5.0 inverse femtobarns of integrated luminosity collected
during 2011 by the CMS experiment at the LHC in pp collisions at sqrt(s)=7 TeV.
The W' boson is modeled as a heavy W boson, but different scenarios for the
couplings to fermions are considered, involving both left-handed and
right-handed chiral projections of the fermions, as well as an arbitrary
mixture of the two. The search is performed in the decay channel W' to t b,
leading to a final state signature with a single lepton (e, mu), missing
transverse energy, and jets, at least one of which is tagged as a b-jet. A W'
boson that couples to fermions with the same coupling constant as the W, but to
the right-handed rather than left-handed chiral projections, is excluded for
masses below 1.85 TeV at the 95% confidence level. For the first time using LHC
data, constraints on the W' gauge coupling for a set of left- and right-handed
coupling combinations have been placed. These results represent a significant
improvement over previously published limits.Comment: Submitted to Physics Letters B. Replaced with version publishe
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