Riconoscere e formalizzare le competenze trasversali nelle attivit\ue0 profossionali e artigianali: esperienze italiane a confronto

L'articolo discute della nozione di competenze trasversali. Esso problematizza tale nozione, facendo emergere le difficolt\ue0 di riconoscimento, formalizzazione e integrazione nei repertori di competenze professionali o tecniche. La presentazione di due esperienze di Ricerca Azione evidenzia la complessit\ue0 di implementare le competenze trasversali in repertori operativi all\u2019interno dei contesti professionali, ma mostra anche come il ruolo delle competenze trasversali nel mondo della formazione e nel mercato del lavoro sia imprescindibile

Comp\ue9tences transverses et savoirs professionnels. Etude de cat\ue9gorisation dans le secteur artisanal en Italie

\uc0 partir de l\u2019examen des r\ue9sultats de deux recherches-actions r\ue9alis\ue9es dans les secteurs de la chaussure de luxe et de l\u2019activit\ue9 artisanale de fabrication de dentelle, cet article interroge les processus d\u2019hybridation de deux logiques cat\ue9goriales pour l\u2019\ue9dification des r\ue9f\ue9rentiels de comp\ue9tences : le mod\ue8le des comp\ue9tences technico-professionnelles et celui des comp\ue9tences transverses. La recherche s\u2019int\ue9resse prioritairement aux proc\ue9d\ue9s de cat\ue9gorisation de l\u2019activit\ue9 et aux crit\ue8res de pertinence de ces cat\ue9gories dans la structure des r\ue9f\ue9rentiels. Par ces travaux, l\u2019article permet d\u2019appr\ue9hender les apports et usages du mod\ue8le des comp\ue9tences transverses en contextes professionnels, et tout sp\ue9cialement dans le monde du travail

V617F Jak2 mutation in children with essential thrombocythemia (ET)

none5noneRANDI M.L.; PUTTI MC; SCAPIN M; PACQUOLA E; FABRIS FRandi, MARIA LUIGIA; Putti, Mc; Scapin, M; Pacquola, E; Fabris, Fabrizi

Normal thrombopoietin and its receptor (c-mpl) genes in children with essential thrombocythemia.

BACKGROUND:Following the observation of thrombopoietin (TPO) gene abnormalities as the cause of familiar cases of thrombocythemia similar derangements of TPO and/or its receptor (c-mpl) might be surmised to be at the root of increased platelet count also in non-familiar (sporadic) cases. Although this was not demonstrated in adults, little data exist about childhood. PROCEDURES:We studied the molecular biology of TPO and c-mpl in seven children with non-familiar essential thrombocythemia (ET) and one child with secondary thrombocytosis (ST). Plasma TPO content was measured using a commercially available kit. Genomic DNA was extracted from whole blood by standard methods and TPO and c-mpl genes were amplified by polymerase chain reaction (PCR) and sequenced. RESULTS:Plasma TPO levels were normal in all our patients. No alteration was detected in either coding region, including the flanking intronic sequences of TPO and c-mpl genes. As compared to the published normal sequence of the TPO gene, one allelic base change in a non-coding region of intron 1 was found in all children with ET and ST, but this was reported as a common finding in normal subjects as well. CONCLUSIONS:High platelet count in our series of sporadic ET of childhood is not due to an abnormality either of TPO or c-mpl gene

Pediatric patients with essential thrombocythemia are mostly polyclonal and V617FJAK2 negative.

Essential thrombocythemia (ET) is rare in children, and little or no information is available about clonality or JAK2 mutations. However, the analyses in this work prove useful for the diagnosis of adult myeloproliferative disorders (MPDs). We evaluated the clonality status and V617FJAK2 mutation in 20 children affected by ET and compared them with 47 consecutive adult ET cases. Clonality was evaluated on the DNA of granulocytes and on the RNA of platelets. V617FJAK2 was analyzed by sequencing tests, allele-specific polymerase chain reaction (PCR), and digestion by BsaXI. A monoclonal pattern was found in 4 (28.5%) of 14 children and in 45% of informative adults. Heterozygous V617FJAK2 was found less frequently in children than in adults (P < .009). Only 2 girls showed both the V617FJAK2 mutation and a monoclonal pattern; one of them was the only child presenting a major thrombotic complication. In contrast to adults, most children with ET do not show either a clonal disorder or the V617FJAK2 mutation