Venous Malformations in Childhood: Clinical, Histopathological and Genetics Update

Our knowledge in vascular anomalies has grown tremendously in the past decade with the identification of key molecular pathways and genetic mutations that drive the development of vascular tumors and vascular malformations. This has led us to better understand the pathogenesis of vascular lesions, refine their diagnosis and update their classification while also exploring the opportunity for a targeted molecular treatment. This paper aims to provide an overview of venous malformations (VM) in childhood. Specific entities include common VMs, cutaneo-mucosal VM, blue rubber bleb nevus syndrome or Bean syndrome, glomuvenous malformation, cerebral cavernous malformation, familial intraosseous vascular malformation and verrucous venous malformation. The clinicopathological features and the molecular basis of each entity are reviewed

Rapid Differential Diagnosis between Extrapulmonary Tuberculosis and Focal Complications of Brucellosis Using a Multiplex Real-Time PCR Assay

BACKGROUND: Arduous to differ clinically, extrapulmonary tuberculosis and focal complications of brucellosis remain important causes of morbidity and mortality in many countries. We developed and applied a multiplex real-time PCR assay (M RT-PCR) for the simultaneous detection of Mycobacterium tuberculosis complex and Brucella spp. METHODOLOGY: Conventional microbiological techniques and M RT-PCR for M. tuberculosis complex and Brucella spp were performed on 45 clinical specimens from patients with focal complications of brucellosis or extrapulmonary tuberculosis and 26 control samples. Fragments of 207 bp and 164 bp from the conserved region of the genes coding for an immunogenic membrane protein of 31 kDa of B. abortus (BCSP31) and the intergenic region SenX3-RegX3 were used for the identification of Brucella and M. tuberculosis complex, respectively. CONCLUSIONS: The detection limit of the M RT-PCR was 2 genomes per reaction for both pathogens and the intra- and inter-assay coefficients of variation were 0.44% and 0.93% for Brucella and 0.58% and 1.12% for Mycobacterium. M RT-PCR correctly identified 42 of the 45 samples from patients with tuberculosis or brucellosis and was negative in all the controls. Thus, the overall sensitivity, specificity, PPV and NPV values of the M RT PCR assay were 93.3%, 100%, 100% and 89.7%, respectively, with an accuracy of 95.8% (95% CI, 91.1%-100%). Since M RT-PCR is highly reproducible and more rapid and sensitive than conventional microbiological tests, this technique could be a promising and practical approach for the differential diagnosis between extrapulmonary tuberculosis and focal complications of brucellosis

Las medidas de gestión de mercados en la nueva PAC

Históricamente, los instrumentos de gestión de mercados constituían el núcleo de la PAC, de tal forma que medidas como las compras de intervención o la protección en frontera proporcionaban un elevado grado de sostenimiento de ingresos a gran parte de las producciones agrarias europeas

Actitud y contexto ante los quehaceres escolares: el estudio de un caso en primaria en España

A lo largo de los últimos cursos académicos, el profesorado del centro escolar en el que hemos enfocado nuestro estudio ha ido detectando en el alumnado una significativa falta de motivación con respecto a la escuela y las tareas escolares, observable tanto en aquellos estudiantes que tienen "éxito escolar" como en los que tienen ciertas dificultades y precisan apoyos o refuerzos educativos. Partiendo de esta realidad, y desde una vocación aplicada que trata de mitigar esta carencia para atenuar asimismo sus efectos, el propósito de nuestro trabajo ha sido conocer cuál es la percepción que los distintos colectivos implicados en el proceso educativo tienen acerca de la motivación de los alumnos del centro y cuáles son las condiciones contextuales y los usos o rutinas de los estudiantes que pueden incidir en su actitud frente a las tareas escolares

Five years survival of women diagnosed with breast cancer during the period 1997-1999 in Toledo-Centro and Mancha Area, Spain

Fundamentos: el cáncer de mama representa la primera causa de mortalidad prematura en las mujeres de la provincia de Toledo. El objetivo es conocer el estadío al diagnóstico para cada grupo de edad y la supervivencia relativa (SR) del cáncer de mama a los 5 años, en el Área de Toledo-Centro y Mancha de los tumores diagnosticados durante 1997-1999. Métodos: se utilizó la información del Registro Poblacional de Cáncer de Toledo. Se incluyó a 366 mujeres con tumores clasificados por estadio y grupo de edad (en función de su inclusión en el programa de detección precoz de cáncer de mama, 64). La SR e intervalo de confianza al 95% (IC) fueron calculados mediante el método de Hakulinen utilizando la aplicación WAERS. Resultados: la SR global fue 78,4% (IC: 73,6-83,6), siendo 93,3% (IC: 87,0-99,4) en 64 años. La SR fue 99,3% (IC: 94,4-104,5) para tumores localizados; 81,9% (IC: 74,0-90,8) para tumores con afectación ganglionar; y 20,1% (IC: 9,7-41,6) para tumores con metástasis. Al diagnóstico, el 52,3% de los tumores en 64 años presentaba metástasis. Conclusiones: la SR es similar a la media del estudio EUROCARE-4. Estos resultados son un punto de partida para valorar, mediante el seguimiento de estos indicadores, el impacto de las actividades de detección precoz y terapéuticas en la SR del cáncer de mama en nuestra área

Reforma de la PAC de 2003. Evaluación del impacto sobre la superficie, los rendimientos de secano y la diversidad de cultivos en España

Este artículo evalúa los impactos de la Reforma de la PAC de 2003 y la implementación del Pago Único (PU) sobre superficies, rendimientos de secano y diversidad de cultivo en España. Empleando datos de superficie, se hace un análisis estadístico para aislar el efecto del PU con respecto a la tendencia del periodo 1995-2009. Adicionalmente, se analiza el impacto sobre los rendimientos de las principales producciones de secano. Finalmente, se analiza la diversidad productiva. Desde la introducción del PU en secano se ha producido una mayor respuesta al mercado, caracterizada por una concentración e intensificación de la producción. También produjo un efecto marcado de reducción en la SAU total, y las SAUs de secano y regadío

The histological and immunohistochemical features of the skin lesions in CANDLE syndrome

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a newly characterized autoinflammatory disorder, caused by mutations in PSMB8. It is characterized by early-onset fevers, accompanied by a widespread, violaceous and often annular, cutaneous eruption. While the exact pathogenesis of this syndrome is still obscure, it is postulated that the inflammatory disease manifestations stem from excess secretion of interferons. Based on preliminary blood cytokine and gene expression studies, the signature seems to come mostly from type I interferons, which are proposed to lead to the recruitment of immature myeloid cells into the dermis and subcutis. In this study, we systematically analyzed skin biopsies from 6 CANDLE syndrome patients by routine histopathology and immunohistochemistry methods. Skin lesions showed the presence of extensive mixed dermal and subcutaneous inflammatory infiltrate, composed of mononuclear cells, atypical myeloid cells, neutrophils, eosinophils and some mature lymphocytes. Positive LEDER and myeloperoxidase staining supported the presence of myeloid cells. Positive CD68/PMG1 and CD163 staining confirmed the existence of histiocytes and monocytic macrophages in the inflammatory infiltrate. CD123 staining was positive, demonstrating the presence of plasmacytoid dendritic cells. Uncovering the unique histopathologic and immunohistochemical features of CANDLE syndrome provides tools for rapid and specific diagnosis of this disorder as well as further insight into the pathogenesis of this severe, life-threatening conditionThis work was supported in part by the NIAMS Intramural Research Program (IRP) at the National Institutes of Health (NIH); The Authority for Research and Development, Hebrew University of Jerusalem (to A.Z.), and the Young clinician’s grant, Hadassah – Hebrew University Medical Center (to Y.R.

Disentangling Component Dynamics in an All-Polymer Nanocomposite Based on Single-Chain Nanoparticles by Quasielastic Neutron Scattering

[EN] We have investigated an all-polymer nanocomposite (NC) consisting of single-chain nanoparticles (SCNPs) immersed in a matrix of linear chains of their precursors (25/75% composition in weight). The SCNPs were previously synthesized via "click" chemistry, which induces intramolecular cross-links in the individual macromolecules accompanied by a slight shift (5-8 K) of the glass transition temperature toward higher values and a broadening of the dynamic response with respect to the raw precursor material. The selective investigation of the dynamics of the NC components has been possible by using properly isotopically labeled materials and applying quasielastic neutron scattering techniques. Results have been analyzed in the momentum transfer range where the coherent scattering contribution is minimal, as determined by complementary neutron diffraction experiments with polarization analysis. We observe the development of dynamic heterogeneity in the intermediate scattering function of the NC components, which grows with increasing time. Local motions in the precursor matrix of the NC are accelerated with respect to the reference bulk behavior, while the displacements of SCNPs' hydrogens show enhanced deviations from Gaussian and exponential behavior compared with the pure melt of SCNPs. The resulting averaged behavior in the NC coincides with that of the pure precursor, in accordance with the macroscopic observations by differential scanning calorimetry (DSC) experiments.We acknowledge the Grant PGC2018-094548-B-I00 funded by MCIN/AEI/10.13039/501100011033 and by "ERDF A way of making Europe". We also acknowledge the financial support of Eusko Jaurlaritza, codes: IT-1175-19 and IT1566-22 and the Open Access funding provided by University of Basque Country

Variation of the phenolic composition and a-glucosidase inhibition potential of seeds, soaked seeds, and sprouts of four wild forms and four varieties of common bean (Phaseolus vulgaris)

The determination of the changes in the composition of bioactive phenolic compounds of germinating seeds which accumulate high levels of these compounds could contribute to the understanding of the germination mechanism and the development of markers for the selection of plant genotypes. In the current study, the changes in the phenolic composition and a-glucosidase inhibition activity, taking place during the germination of four wild forms and four varieties of common bean (Phaseolus vulgaris L.) from Durango Mexico, were determined. A total of 66 phenolic compounds (19 phenolic acids, 18 isoflavones, 18 flavonol glycosides, 3 flavonol aglycones, 3 flavones, 2 dihydroflavonoids, 2 chalcones and one non-identified type) were found by HPLC-DAD, which were differentially accumulated by the seeds, 24 h-soaked seeds, and 4 day-sprouts of each genotype. The accumulation of the flavonol aglycones, myricetin, quercetin and kaempferol was distinctive of the wild seeds. Soaking not only caused leaching and degradation but also triggered the synthesis of new phenolic compounds whereas germination diversified the composition of isoflavones and flavonol glycosides. The seeds of all genotypes analyzed were important inhibitors of a-glucosidase, improving their potential after soaking and germination. The results suggested that the structure rather than the concentration of the flavonoids and phenolic acids determined the inhibitory potential of a-glucosidase of samples. The principal component analysis and cluster analysis revealed HPLC-DAD phenolic profiles as genotype-specific chemomarkers at any of the states (seeds, soaked seeds, and sprouts). The results have wide implications on agronomy and food quality