La reconnaissance des acquis expérientiels: étude de cas de la politique d'admission des adultes à l'Université Laval

Has the inclusion of experience as a criterion of admission for adult students raised significantly the chances of these students of being admitted? The authors analyse the new admission policy at Laval University (1980) and, from a sample of400 candidates' dossiers, its practice age and a fair amount of experience appear to be important factors opening the door of university. However, the effects of these factors are particularly enhanced if experience is close to academic or cognitive learning or combined with a CEGEP diploma and a grade higher than 65%. Thus, experience does not seem to be used as a basis of admission of its own, but mainly to corroborate academic ability already expressed by good grades.Les auteurs évaluent formellement la politique et le processus d'évaluation de la clientèle adulte qui ne rencontre pas les critères "normaux" d'admission aux programmes de premier cycle à l'Université Laval en 1980. L'étude de l'application de cette politique, tenant compte de l'expérience de vie ou des acquis expérientiels des candidats adultes en plus de leurs acquis scolaires antérieurs et l'analyse d'un échantillon de 400 dossiers de candidats adultes nous révèlent que l'âge et la durée de l'expérience constituent des facteurs favorisant l'admissibilité des candidats adultes. Elles démontrent aussi clairement que le profil de référence, lors de ce processus d'évaluation, est principalement la for- mation scolaire et sa dimension cognitive. En effet, le fait de posséder des expé- riences liées à l'apprentissage, au moins un diplôme de niveau collégial et des résultats scolaires supérieurs à 65%, augmente les chances d'un candidat d'être reconnu admissible. Le concept d'expérience de vie semble donc être utilisé de façon secondaire, comme confirmation au renforcement des succès scolaires antérieurs, plutôt que comme critère autonome

Prenatal growth restriction, retinal dystrophy, diabetes insipidus and white matter disease: expanding the spectrum of PRPS1-related disorders

PRPS1 codes for the enzyme phosphoribosyl pyrophosphate synthetase-1 (PRS-1). The spectrum of PRPS1-related disorders associated with reduced activity includes Arts syndrome, Charcot-Marie-Tooth disease-5 (CMTX5) and X-linked non-syndromic sensorineural deafness (DFN2). We describe a novel phenotype associated with decreased PRS-1 function in two affected male siblings. Using whole exome and Sanger sequencing techniques, we identified a novel missense mutation in PRPS1. The clinical phenotype in our patients is characterized by high prenatal maternal α-fetoprotein, intrauterine growth restriction, dysmorphic facial features, severe intellectual disability and spastic quadraparesis. Additional phenotypic features include macular coloboma-like lesions with retinal dystrophy, severe short stature and diabetes insipidus. Exome sequencing of the two affected male siblings identified a shared putative pathogenic mutation c.586C>T p.(Arg196Trp) in the PRPS1 gene that was maternally inherited. Follow-up testing showed normal levels of hypoxanthine in urine samples and uric acid levels in blood serum. The PRS activity was significantly reduced in erythrocytes of the two patients. Nucleotide analysis in erythrocytes revealed abnormally low guanosine triphosphate and guanosine diphosphate. This presentation is the most severe form of PRPS1-deficiency syndrome described to date and expands the spectrum of PRPS1-related disorder