The Differences Between Childhood and Adult Onset Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is a rare autoimmune disease of unknown etiology that represents a leading cause of acquired thromboembolism and recurrent miscarriage. It is characterized by the persistent elevated presence of pathogenic antiphospholipid auto-antibodies directed against cardiolipin, ß2-glycoprotein-I, and/or a positive lupus anticoagulant test. As with many autoimmune disorders, the pathogenesis of APS is believed to be the result of a complex interaction between environmental triggers and genetic predisposition. Although more common in adults, APS occasionally manifests in the neonatal period and throughout childhood. Adut-onset APS classification criteria are poorly validated to the pediatric population (in which pregnancy related complications are seldom seen) and as a result, assessment of the prevalence of the disease in childhood is difficult. Thromboembolic events seen in children include deep venous thrombosis in addition to stroke and pulmonary embolism, which can lead to significant long-term disability. The disease can be classified as either primary (when occurring in isolation) or secondary, in which the disease is diagnosed in the context of another underlying disease, most commonly systemic lupus erythematosus. A variety of laboratory and clinical difference are seen between pediatric and adult-onset APS. The marked female predominance seen in adult-onset disease is less evident in childhood where the gender split is more evenly spread. In addition, children with APS are at a higher risk of recurrent thromboembolism than adults. The treatment of childhood-onset APS is challenging due to a lack of large-scale prospective studies in the pediatric population. Therapeutic options are often based upon treatment guidelines that have been based upon literature from the adult-onset form of the disease. In the majority of cases, treatment is focused on the prevention of further thrombosis through treatment with long-term anti-coagulation therapy. The evidence for the use of antiplatelet agents (such as aspirin) and hydroxychloroquine is inconclusive. It is important to remember that anti-coagulation can have significant lifestyle implications for the child with APS and it is essential to consider potential implications relating to school and recreational activities, with contact sports often discouraged due to the increased risk of bleeding

Cuisine chypriote et cuisine grecque

Dans l’article qui suit, nous allons tenter d’approcher un phénomène très récent qui s’attache aux réalités culinaires de l’île de Chypre et qui n’a pas encore été étudié. Il s’agit de la présence à Chypre d’un nombre croissant de restaurants et de bistrots grecs, notamment depuis une quinzaine d’années, phénomène lié en partie à la prospérité et au niveau de vie plus élevés à Chypre qu’en Grèce, du moins jusqu’en 2013, ainsi qu’à la crise durable de l’économie grecque depuis la fin de la dernière décennie. Les conclusions de cette approche pourraient être utiles pour une analyse plus approfondie de la question

Les rêves d’Elytis

Strongly influenced by the Surrealist Movement, and particularly by its psychoanalytic dimensions, Elytis attaches the utmost importance to the dream as a space of total freedom beyond any form of prohibition, therefore as a space of innocence and authentic creativity. This article studies the role of the thematic of the dream in the works of the Greek Nobelist

La réinvention de Rimbaud par la poésie chypriote

This article studies the reception of Rimbaud´s two-period stay in Cyprus (1878-79, 1880), by the 20th century Cypriot poetry. It attempts to show that, despite the fact that Rimbaud´s activities during his stay, had nothing to do with literature or anything heroic, Cypriot poetry has presented the poet as a semaphore of individual and national revolution as well as an idealised example of contestation and rebelliousness

L’identité insulaire et le Paradis retrouvé d’Odysseus Elytis

In this article, we study the way in which the geocultural, insular space of diachronic Greece transforms itself into a clearly poetic space in Odysseas Elytis’s poetry and subsequently, the way in which the discovery of this space becomes one of the decisive factors of the poet’s esthetic and ideological identity. The insular space appears as a source of inspiration and, at the same time, as a place of life as well as a creative universe which results in the sanctification of the senses as well as the means of interpretation of the world.  This sanctification leads, in its turn, to the abolition of the biological temporality and to the adoption of a poetic temporality which results from the incessant penetration into the sensation.  In this liberator Earthly Paradise, lives again in the form of an eternally present sensation, the stunning synthesis of all stages of the Greek history and civilization, as a place of magic and innocence

PKC Activation in Niemann Pick C1 Cells Restores Subcellular Cholesterol Transport

Activation of protein kinase C (PKC) has previously been shown to ameliorate the cholesterol transport defect in Niemann Pick Type C1 (NPC1) cells, presumably by increasing the soluble levels of one of its substrates, vimentin. This activity would then restore the vimentin cycle in these cells and allow vimentin-dependent retrograde transport to proceed. Here, we further investigate the effects of PKC activation in NPC1 cells by evaluating different isoforms for their ability to solubilize vimentin and correct the NPC1 cholesterol storage phenotype. We also examine the effects of PKC activators, including free fatty acids and the PKC-specific activator diazoxide, on the NPC1 disease phenotype. Our results indicate that PKC isoforms α, βII, and ε have the greatest effects on vimentin solubilization. Furthermore, expression or activation of PKCε in NPC1 cells dramatically reduces the amount of stored cholesterol and restores cholesterol transport out of endocytic vesicles. These results provide further support for the contribution of PKCs in NPC1 disease pathogenesis and suggest that PKCs may be targeted in future efforts to develop therapeutics for NPC1 disease

The evolution of labour law in the new member states of the European Union : 1995-2005 - country studies on Cyprus and Malta

This Report traces the development of Labour Law and the implications for Industrial Relations, as well as social and employment policy more generally, in the two small Mediterranean countries of Cyprus and Malta during the period 1995–2005. This period was particularly important for the two countries as it coincided with their efforts for accession to the European Union (EU) and the process of harmonisation with the Acquis Communautaire. Since their independence in 1960 and 1964 respectively for Cyprus and Malta, successive Governments in each country – working with the social partners – had sought to steer a policy of social cohesion to underpin their development efforts. Whilst these strategies were successful in fostering a long period of economic growth and peaceful labour relations, a major outcome was the existence of relatively inflexible labour markets. Liberalisation and globalisation of international markets, coupled with the pressure exerted by the accession process, which required the implementation of the Acquis Communautaire necessitated a series of changes with far reaching implications in social and economic affairs. Naturally the framework of Labour Law – and labour practices thereof – came under increasing pressure to adapt and reform. The Executive Summary describes the main aims and objectives of the Report on the evolution of Labour Law in Cyprus and Malta in the period 1995-2005, and provides an outline of the component chapters. Specifically the Report is divided into three chapters. The first and second chapters consist of the individual Reports on Cyprus and Malta respectively. These constitute the main body of the Report and investigate the evolution of Labour Law in the two countries separately and the implications for Industrial Relations, employment and social policy. The third chapter provides a concluding overview of the two countries’ experiences and an evaluation of the state of implementation of the Acquis Communautaire in the fields examined.peer-reviewe

A novel expression system of domain I of human beta2 glycoprotein I in Escherichia coli

BACKGROUND: The antiphospholipid syndrome (APS), characterised by recurrent miscarriage and thrombosis, is a significant cause of morbidity and mortality. Domain I (DI) of human beta 2 glycoprotein I (β(2)GPI) is thought to contain crucial antibody binding epitopes for antiphospholipid antibodies (aPL), which are critical to the pathogenesis of APS. Expressing this protein in bacteria could facilitate studies investigating how this molecule interacts with aPL. METHODS: Using a computer programme called Juniper, sequentially overlapping primers were designed to be used in a recursive polymerase chain reaction (PCR) to produce a synthetic DI gene. Specifically Juniper incorporates 'major' codons preferred by bacteria altering 41 codons out of 61. This was cloned into the expression plasmid pET(26b) and expressed in BL21(DE3) Escherichia coli (E. coli). By virtue of a pelB leader sequence, periplasmic localisation of DI aided disulphide bond formation and toxicity was addressed by tightly regulating expression through the high stringency T7lac promoter. RESULTS: Purified, soluble his-tagged DI in yields of 750 μg/L bacterial culture was obtained and confirmed on Western blot. Expression using the native human cDNA sequence of DI in the same construct under identical conditions yielded significantly less DI compared to the recombinant optimised sequence. This constitutes the first description of prokaryotic expression of soluble DI of β(2)GPI. Binding to murine monoclonal antibodies that recognise conformationally restricted epitopes on the surface of DI and pathogenic human monoclonal IgG aPL was confirmed by direct and indirect immunoassay. Recombinant DI also bound a series of 21 polyclonal IgG samples derived from patients with APS. CONCLUSION: By producing a synthetic gene globally optimised for expression in E. coli, tightly regulating expression and utilising periplasmic product translocation, efficient, soluble E. coli expression of the eukaryotic protein DI of β(2)GPI is possible. This novel platform of expression utilising pan-gene prokaryote codon optimisation for DI production will aid future antigenic studies. Furthermore if DI or peptide derivatives of DI are eventually used in the therapeutic setting either as toleragen or as a competitive inhibitor of pathogenic aPL, then an E. coli production system may aid cost-effective production