El Gran París: el reto de superar las oscuras realidades de exclusión social en la ciudad de las luces

La exclusión social en París ha alcanzado niveles alarmantes generando un malestar social importante en los franceses, llegando incluso a transformarse en protestas callejeras como en el caso de los disturbios de 2005. Existe una segregación espacial tácita, vinculada directamente con el origen socio-cultural, el nivel económico e incluso con la orientación sexual de sus habitantes. Este reportaje apunta a resaltar esta grave situación y las iniciativas tomadas por el gobierno de Nicolás Sarkozy y los desafíos que le esperan al nuevo mandatario galo François Hollande. A lo largo de esta investigación periodística se evidencian el origen de las ampliaciones urbanas en la capital francesa, los retos sociales que persisten en el París actual y la multiplicidad de actores sociales que están involucrados. Lejos de haber un consenso, los diferentes actores implicados difieren en las medidas a adoptar para paliar esta segregación. Estas medidas van desde potenciar el transporte público hasta la construcción de viviendas de interés social.

Análisis del Espacio Schengen en materia de seguridad como base para la implementación del Protocolo sobre la Facilitación del Movimiento de Personas de la Comunidad para el Desarrollo de África Austral (SADC)

El fin de la Guerra Fría supuso no sólo el triunfo del capitalismo y de la democracia liberal, sino un cambio significativo en el Sistema Internacional; siendo menos centralizado y más regionalizado, como consecuencia de la proximidad y relaciones de interdependencia entre sus actores (no sólo Estados) y permitiendo la formación de Complejos Regionales de Seguridad (CRS). Los CRS son una forma efectiva de relacionarse y aproximarse a la arena internacional pues a través de sus procesos de securitización y desecuritización consiguen lograr objetivos específicos. Partiendo de ello, tanto la Unión Europea (UE) como la Comunidad para el Desarrollo de África Austral (SADC) iniciaron varios procesos de securitización relacionados con la integración regional; siendo un ejemplo de ello la eliminación de los controles en sus fronteras interiores o libre circulación de personas; pues consideraron que de no hacerse realidad, ello generaría amenazas políticas (su influencia y capacidad de actuación estaban amenazadas), económicas (en cuanto a su competitividad y niveles básicos de bienestar) y societales (en cuanto a la identidad de la comunidad como indispensable para la integración) que pondrían en riesgo la existencia misma de sus CRS. En esta medida, la UE creó el Espacio Schengen, que fue producto de un proceso de securitización desde inicios de la década de los 80 hasta mediados de la década de los 90; y la SADC se encuentra inmersa en tal proceso de securitización desde 1992 hasta la actualidad y espera la ratificación del Protocolo para la Facilitación del Movimiento de personas como primer paso para lograr la eliminación de controles en sus fronteras interiores. Si bien tanto la UE como la SADC consideraron que de no permitir la libre circulación de personas, su integración y por lo tanto, sus CRS estaban en riesgo; la SADC no lo ha logrado. Ello hace indispensable hacer un análisis más profundo de sus procesos de securitización para así encontrar sus falencias con respecto al éxito de la UE. El análisis está basado en la Teoría de los Complejos de Seguridad de Barry Buzan, plasmada en la obra Security a New Framework for Analysis (1998) de Barry Buzan, Ole Waever y Jaap de Wilde y será dividido en cada una de las etapas del proceso de securitización: la identificación de una amenaza existencial a un objeto referente a través de un acto discursivo, la aceptación de una amenaza por parte de una audiencia relevante y las acciones de emergencia para hacer frente a las amenazas existenciales; reconociendo las diferencias y similitudes de un proceso de securitización exitoso frente a otro que aún no lo ha sido.

Aproximación al manejo de la seguridad ambiental canadiense y estadounidense frente a la degradación del sistema de la los Grandes Lagos. Periodo 1995-2000

El propósito de la presente monografía es determinar la relación entre la degradación y navegación en los Grandes Lagos en la noción de seguridad ambiental de Estados Unidos y Canadá en un entorno de interdependencia entre 1995 - 2000. En ese sentido, se busca determinar como los recursos de poder de Canadá y Estados Unidos en la relación degradación-navegación transforma la noción de seguridad ambiental. De este modo, se analiza el concepto de seguridad ambiental desde la navegación, elemento esencial para entender la relación bilateral dentro del sistema de los Grandes Lagos. Esta investigación de tipo cualitativo que responde a las variables de la seguridad ambiental planteadas por Barry Buzan, Thomas Homer-Nixon, y Stephan Libiszewski, y a la teoría de la Interdependencia Compleja por Robert Keohane y Joseph Nye, pretende avanzar hacia la complejización de la dimensión ambiental lejos de la tradicional definición antropocéntrica.

El papel que desempeña la Comisión Europea a través de la línea de desarraigados y la ayuda oficial al desarrollo en materia de la atención al desplazamiento como consecuencia de la violencia en Colombia.

El fuerte impacto que el fenómeno del desplazamiento forzado tiene para Colombia y para los actores de la Comunidad Internacional, resulta interesante analizar el trabajo conjunto que han desarrollado las instituciones del Gobierno nacional encargadas de la atención al desplazamiento

Actividad educativa, cultural y profesional relacionada con el conocimiento de Castilla y León.

Realizado en el I.E.S Ramón y Cajal de Valladolid, complementando un intercambio cultural y profesional con alumnos del Centre de Formation d'apprendits de Salon de Provençe (Francia) y del Lycée Sainte-Victoire de Aix-en-Provence, participantes en el Programa europeo PETRA II, con el objetivo de visitar Empresas y Organismos de ese país y que los alumnos franceses realicen esas visitas en nuestra provincia. Realizan un viaje a Salamanca con un triple objetivo: educativo, cultural y profesional en diferentes enseñanzas profesionales, aprovechando la exposición 'Las Edades del Hombre' y relacionarla con su profesión, la estética, elaborando trabajos y estudiando algunas obras. En Ledesma visitan un balneario donde contemplan distintas técnicas de hidromasaje y otros tratamientos, ampliando lo visto en clase. En Aldeadávila y Almendra descubren los saltos de agua, generadores de energia hidroeléctrica. Presentan monográficos de estas visitas-estudio elaborados por los alumnos. Con una evaluación continua, formativa e integradora, valoran positivamente la actividad en general, teniendo en cuenta el interés, colaboración, actitud, puntualidad. El trabajo no está publicado..

L'éducation au développement durable : de l'école au campus. 'La educación en el desarrollo sostenible : de la escuela al campus'.

Basado en un Coloquio Internacional con el mismo título celebrado en L'École des Mines d'Albi, 25-27 de junio de 2008

Dos maneras de erudición.

Existen dos clases de escritores y de conversadores eruditos: los que poseen realmente erudición y los que aparentan tenerla. Hay en las letra erudición de primera y de segunda mano. Aquélla es útil; ésta, no, por lo menos cuando no se maneja con arte. Modelos de eruditos de primera mano son, entre otros, Sainte Beuve y Menéndez y Pelayo. Falsos eruditos hay y hubo muchos en todos los tiempos y países. Chateaubriand y Victor Hugo, pertenecen a este segundo grupo. No obstante, Victor Hugo fue escritor de primera magnitud, pero erudito de segunda mano. Los conocimientos científicos, literarios, artísticos, que cada uno guarda han de acudir a prestarnos fama de eruditos en momento oportuno y sin que se esperen. De lo contrario, los más despiertos enseguida se darán cuenta de que se les engaña con una erudición ficticia. Todo hombre, y sobretodo el escritor, necesita tener cultura, haber leído mucho y todos los conocimientos adquiridos ha de saber aplicarlos con sentido común, oportunamente, cuando venga al caso. Sólo así podrá llegar a ser un verdadero erudito.

Missionary children: The French Holy Childhood Association in European context, 1843-c.1914

In the mid-nineteenth century, thousands of children in Europe and beyond were organized into battalions of fundraisers for overseas missions. By the end of the century these juvenile missionary organizations had become a global movement, generating millions of pounds in revenue each year. While the transnational nature of the children’s missions and publications has been well-documented by historians, the focus has tended to be on the connections that were established by encounters between the young western donors, missionaries overseas and the non-western ‘other’ constructed by their work. A full exploration of the European political, social and cultural concerns that produced the juvenile missionaries movement and the trans-European networks that sustained it are currently missing from historical accounts of the phenomenon. This article looks at the largest of these organizations, the Catholic mission for children, the French Holy Childhood Association (L’Œuvre de la sainte enfance), to understand how the principles this mission sought to impose abroad were above all an expression of anxieties at home about the role of religion in the family, childhood and in civil society as western polities were modernizing and secularizing in the nineteenth century.

Prevalence and nonrandom distribution of exonic mutations in interferon regulatory factor 6 in 307 families with Van der Woude syndrome and 37 families with popliteal pterygium syndrome

Purpose: Interferon regulatory factor 6 encodes a member of the IRF family of transcription factors. Mutations in interferon regulatory factor 6 cause Van der Woude and popliteal pterygium syndrome, two related orofacial clefting disorders. Here, we compared and contrasted the frequency and distribution of exonic Mutations in interferon regulatory factor 6 between two large geographically distinct collections of families with Van der Woude and between one collection of families with popliteal pterygium syndrome. Methods: We performed direct sequence analysis of interferon regulatory factor 6 exons oil samples from three collections, two with Van der Woude and one with popliteal pterygium syndrome. Results: We identified mutations in interferon regulatory factor 6 exons in 68% of families in both Van der Woude collections and in 97% of families with popliteal pterygium syndrome. In sum, 106 novel disease-causing variants were found. The distribution of mutations in the interferon regulatory factor 6 exons in each collection was not random; exons 3, 4, 7, and 9 accounted for 80%. In the Van der Woude collections, the mutations were evenly divided between protein truncation and missense, whereas most mutations identified in the popliteal pterygium syndrome collection were missense. Further, the missense mutations associated with popliteal pterygium syndrome were localized significantly to exon 4, at residues that are predicted to bind directly to DNA. Conclusion: The nonrandom distribution of mutations in the interferon regulatory factor 6 exons suggests a two-tier approach for efficient mutation screens for interferon regulatory factor 6. The type and distribution of mutations are consistent with the hypothesis that Van der Woude is caused by haploinsufficiency of interferon regulatory factor 6. Oil the other hand, the distribution of popliteal pterygium syndrome-associated mutations suggests a different, though not mutually exclusive, effect oil interferon regulatory factor 6 function. Genet Med 2009:11(4):241-247.

Nonrecurrent MECP2 duplications mediated by genomic architecture-driven DNA breaks and break-induced replication repair

Recurrent submicroscopic genomic copy number changes are the result of nonallelic homologous recombination (NAHR). Nonrecurrent aberrations, however, can result from different nonexclusive recombination-repair mechanisms. We previously described small microduplications at Xq28 containing MECP2 in four male patients with a severe neurological phenotype. Here, we report on the fine-mapping and breakpoint analysis of 16 unique microduplications. The size of the overlapping copy number changes varies between 0.3 and 2.3 Mb, and FISH analysis on three patients demonstrated a tandem orientation. Although eight of the 32 breakpoint regions coincide with low-copy repeats, none of the duplications are the result of NAHR. Bioinformatics analysis of the breakpoint regions demonstrated a 2.5-fold higher frequency of Alu interspersed repeats as compared with control regions, as well as a very high GC content (53%). Unexpectedly, we obtained the junction in only one patient by long-range PCR, which revealed nonhomologous end joining as the mechanism. Breakpoint analysis in two other patients by inverse PCR and subsequent array comparative genomic hybridization analysis demonstrated the presence of a second duplicated region more telomeric at Xq28, of which one copy was inserted in between the duplicated MECP2 regions. These data suggest a two-step mechanism in which part of Xq28 is first inserted near the MECP2 locus, followed by breakage-induced replication with strand invasion of the normal sister chromatid. Our results indicate that the mechanism by which copy number changes occur in regions with a complex genomic architecture can yield complex rearrangements.

Screening of ARHSP-TCC Patients Expands the Spectrum of SPG11 Mutations and Includes a Large Scale Gene Deletion

Autosomal recessive spastic paraplegia with thinning of corpus callosum (ARHSP-TCC) is a complex form of HSP initially described in Japan but subsequently reported to have a worldwide distribution with a particular high frequency in multiple families from the Mediterranean basin. We recently showed that ARHSP-TCC is commonly associated with mutations in SPG11/KIAA1840 on chromosome 15q. We have now screened a collection of new patients mainly originating from Italy and Brazil, in order to further ascertain the spectrum of mutations in SPG11, enlarge the ethnic origin of SPG11 patients, determine the relative frequency at the level of single Countries (i.e., Italy), and establish whether there is one or more common mutation. In 25 index cases we identified 32 mutations; 22 are novel, including 9 nonsense, 3 small deletions, 4 insertions, 1 in/del, 1 small duplication, 1 missense, 2 splice-site, and for the first time a large genomic rearrangement. This brings the total number of SPG11 mutated patients in the SPATAX collection to 111 cases in 44 families and in 17 isolated cases, from 16 Countries, all assessed using homogeneous clinical criteria. While expanding the spectrum of mutations in SPG11, this larger series also corroborated the notion that even within apparently homogeneous population a molecular diagnosis cannot be achieved without full gene sequencing. (C) 2008 Wiley-Liss, Inc.

Cistos subependimários diagnosticados pelo ultra-som transfontanelar

Objetivos: avaliar a evolução ultra-sonográfica de cistos subependimários (CSE) do ângulo externo dos ventrículos laterais, e a evolução clínica dos pacientes. Comparar os pacientes com CSE isolados e os com CSE associados a outras lesões. Métodos: foram diagnosticados um a vários cistos no ângulo externo de um ou de ambos os ventrículos laterais, por meio de ultra-som transfontanelar (US) de rotina, realizado nos primeiros dias de vida, caracterizando os CSE. Durante o período de 1981-2000, 66 recém-nascidos tiveram CSE evidenciados na UTI neonatal do Hospital de Port-Royal. Foram constituídos dois grupos: G-I, com CSE isolados (n=21), e G-II, com CSE associados a outras lesões (n=45). Resultados: os recém-nascidos do GI apresentaram maior maturidade, melhores condições de nascimento e menor morbidade respiratória em relação a GII. A incidência de malformações congênitas foi elevada em ambos os grupos. Houve baixa taxa de infecção bacteriana e ausência de infecção congênita. Os CSE foram uni ou bilaterais, únicos ou múltiplos (colar de pérolas), sem diferença entre os grupos estudados, e predominaram à esquerda. US seriados foram realizados em 49/66 pacientes (74%), mostrando aumento no tamanho do cisto em 21/49 (45%), no primeiro mês de vida, enquanto 12 CSE (24%) desapareceram. O óbito ocorreu em dez recém-nascidos com lesões neurológica graves (quatro leucomalácias periventriculares, cinco hemorragias peri e intraventriculares), e somente um com hérnia diafragmática não apresentava outras lesões ao US transfontanelar. Conclusões: as características dos CSE não diferiram quando esses estavam associados a outras lesões. O nítido predomínio no lado esquerdo sugere uma etiologia vascular. Foi encontrada uma alta taxa de malformações associadas, alertando para a possibilidade de uma etiologia malformativa. Ambas hipóteses sugerem um desvio de desenvolvimento, e não de uma fetopatia viral.

Prevalence and nonrandom distribution of exonic mutations in interferon regulatory factor 6 in 307 families with Van der Woude syndrome and 37 families with popliteal pterygium syndrome

Purpose: Interferon regulatory factor 6 encodes a member of the IRF family of transcription factors. Mutations in interferon regulatory factor 6 cause Van der Woude and popliteal pterygium syndrome, two related orofacial clefting disorders. Here, we compared and contrasted the frequency and distribution of exonic Mutations in interferon regulatory factor 6 between two large geographically distinct collections of families with Van der Woude and between one collection of families with popliteal pterygium syndrome. Methods: We performed direct sequence analysis of interferon regulatory factor 6 exons oil samples from three collections, two with Van der Woude and one with popliteal pterygium syndrome. Results: We identified mutations in interferon regulatory factor 6 exons in 68% of families in both Van der Woude collections and in 97% of families with popliteal pterygium syndrome. In sum, 106 novel disease-causing variants were found. The distribution of mutations in the interferon regulatory factor 6 exons in each collection was not random; exons 3, 4, 7, and 9 accounted for 80%. In the Van der Woude collections, the mutations were evenly divided between protein truncation and missense, whereas most mutations identified in the popliteal pterygium syndrome collection were missense. Further, the missense mutations associated with popliteal pterygium syndrome were localized significantly to exon 4, at residues that are predicted to bind directly to DNA. Conclusion: The nonrandom distribution of mutations in the interferon regulatory factor 6 exons suggests a two-tier approach for efficient mutation screens for interferon regulatory factor 6. The type and distribution of mutations are consistent with the hypothesis that Van der Woude is caused by haploinsufficiency of interferon regulatory factor 6. Oil the other hand, the distribution of popliteal pterygium syndrome-associated mutations suggests a different, though not mutually exclusive, effect oil interferon regulatory factor 6 function. Genet Med 2009:11(4):241-247.

A new probabilistic canopy dynamics model (SLCD) that is suitable for evergreen and deciduous forest ecosystems

There are strong uncertainties regarding LAI dynamics in forest ecosystems in response to climate change. While empirical growth & yield models (G&YMs) provide good estimations of tree growth at the stand level on a yearly to decennial scale, process-based models (PBMs) use LAI dynamics as a key variable for enabling the accurate prediction of tree growth over short time scales. Bridging the gap between PBMs and G&YMs could improve the prediction of forest growth and, therefore, carbon, water and nutrient fluxes by combining modeling approaches at the stand level.Our study aimed to estimate monthly changes of leaf area in response to climate variations from sparse measurements of foliage area and biomass. A leaf population probabilistic model (SLCD) was designed to simulate foliage renewal. The leaf population was distributed in monthly cohorts, and the total population size was limited depending on forest age and productivity. Foliage dynamics were driven by a foliation function and the probabilities ruling leaf aging or fall. Their formulation depends on the forest environment.The model was applied to three tree species growing under contrasting climates and soil types. In tropical Brazilian evergreen broadleaf eucalypt plantations, the phenology was described using 8 parameters. A multi-objective evolutionary algorithm method (MOEA) was used to fit the model parameters on litterfall and LAI data over an entire stand rotation. Field measurements from a second eucalypt stand were used to validate the model. Seasonal LAI changes were accurately rendered for both sites (R-2 = 0.898 adjustment, R-2 = 0.698 validation). Litterfall production was correctly simulated (R-2 = 0.562, R-2 = 0.4018 validation) and may be improved by using additional validation data in future work. In two French temperate deciduous forests (beech and oak), we adapted phenological sub-modules of the CASTANEA model to simulate canopy dynamics, and SLCD was validated using LAI measurements. The phenological patterns were simulated with good accuracy in the two cases studied. However, IA/max was not accurately simulated in the beech forest, and further improvement is required.Our probabilistic approach is expected to contribute to improving predictions of LAI dynamics. The model formalism is general and suitable to broadleaf forests for a large range of ecological conditions. (C) 2014 Elsevier B.V. All rights reserved.