La démocratie fatiguée

La pandemia de COVID-19 puso en evidencia varias faltas o carencias. Inicialmente, algunas estructurales y otras, coyunturales. Las medidas restrictivas fueron decididas por los ejecutivos. Los estados se plantean nuevas agendas para enfrentar la postpandemia. Pero, ¿cuál es el rol reservado a la democracia? Entre las democracias iliberales y las democracias fatigadas hay diferencias de naturaleza sustanciales.La pandémie de la COVID 19 a mis en évidence plusieurs “manques ou lacunes”, au départ, certains structurels et d’autres, circonstanciels. Les mesures restrictives ont été décidées par les exécutifs. Les états envisagent de nouveaux agendas pour faire face á l’après-pandémie. Mais quel est le rôle réservé á la démocratie? Entre les démocraties illégitimes et les démocraties fatiguées, il existe des différences substantielles de nature.Facultad de Ciencias Jurídicas y Sociale

Prevalencia de factores de riesgo de enfermedad cardiovascular en estudiantes universitarios de Santa Fe (Argentina)

Introduction: Cardiovascular diseases (CVD) cause 25% world's deaths. In America, Argentina ranks fourth. Factors such as sedentary lifestyle, smoking habits, eating habits and genetic predisposition, would be responsible. The aim of this study was to estimate the prevalence of CVD risk factors (RF) in Nutrition students. Material and methods: a cross-sectional descriptive study was performed in 185 students, aged between 19 and 30 years. Weight, height, and waist circumference (WC) were measured. In addition, they were questioned about family history of chronic noncommunicable diseases (CNCDs), smoking, alcohol and physical activity (PA). Total cholesterol (TC), HDL-cholesterol (HDL-c), LDL-cholesterol (LDL-c), triglycerides (TG), glucose, Castelli index (CI) and TG/HDL-c ratio were quantified. Differences in continuous variables between groups were evaluated with t test of Student. The statistical significance level assumed was 5%. Minitab 16 program was used. Results: A 75.7% history of CNCDs was found. The body mass index showed 10.3% of overweight and 2.2% of obesity, and cardiovascular risk according to WC was 9.7%. The most altered parameter was LDL-c (46.9%). 21.1% presented hypercholesterolemia and 21.6%, HDL-c decreased. 13.5% presented CI >4.5, whereas when the suggested cut-off point was considered for the Argentine population (CIA>3.25), 39.5% surpassed it. 11.4% had high TG/HDL-c. 8.1% smoked, 56.2% took alcoholic beverages and half did not engage in PA. The RF of CVD that presented statistically significant differences according to sex were overweight, HDL-c and CI, resulting in all the cases most unfavorable in men.Conclusions: Family history of CNCDs, alcohol consumption, physical inactivity, hypercholesterolemia with decreased HDL-c, marked elevation of LDL-c and elevated CI was remarkable. As the most of these cardiovascular RF are modifiable, it is suggested to establish nutritional education programs and promotion of healthy lifestyles.Resumen  Introducción: Las enfermedades cardiovasculares (ECV) causan el 25% de las muertes en el mundo. En América, Argentina ocupa el cuarto lugar. Factores como sedentarismo, tabaquismo, hábitos alimentarios y predisposición genética, serían responsables. El objetivo fue estimar la prevalencia de factores de riesgo (FR) de ECV en estudiantes de Nutrición.Material y métodos: Se realizó un estudio descriptivo-transversal, en 185 estudiantes, entre 19-30 años. Se midieron peso, talla y circunferencia de cintura (CC). Se interrogó sobre antecedentes familiares de enfermedades crónicas no transmisibles (ECNT), tabaquismo, alcohol y actividad física (AF). Se cuantificó colesterol total (CT), HDL-colesterol (HDL-c), LDL-colesterol (LDL-c), triglicéridos (TG), glucosa, índice de Castelli (IC) y la relación TG/HDL-col. Los datos se analizaron estadísticamente con el programa Minitab 16.Resultados: En la población estudiada (21,9 ± 2,9 años) se halló un 75,7% de antecedentes de ECNT. El 8,1% fumaba, el 56,2% tomaba bebidas alcohólicas y la mitad no realizaba AF. El índice de masa corporal mostró 10,3% de sobrepeso y 2,2% de obesidad, y el riesgo cardiovascular según CC fue 9,7%. El parámetro más alterado fue LDL-c (48,1%). El 21,1% presentó hipercolesterolemia y el 22,2%, HDL-col disminuido. El IC fue 3,43 ± 0,96, cuando se evaluó la cifra de riesgo, el 33,3% de los varones y el 18,8% de las mujeres, la superaba. El 11,4% tuvo TG/HDL-col elevado. Hubo asociación entre CC-TG y CC-TG/HDL-col.Conclusiones: Como la mayoría de los FR cardiovascular son modificables, se sugiere establecer programas de educación alimentaria nutricional y promoción de estilos de vida saludable

Pheno-genotyping of inherited thrombocytopenias: our experience in 50 families

Dada la heterogeneidad de las entidades comprendi- das en las trombocitopenias hereditarias y la escasez de marcadores distintivos, su diagnóstico constituye un verdadero desafío. El abordaje clásico se basa en la caracterización fenotípica seguida del estudio mo- lecular de genes candidatos, orientado según la sos- pecha clínica. La introducción de la secuenciación de nueva generación (NGS), que permite evaluar múltiples genes simultáneamente, constituye una al- ternativa diagnóstica de alto costo, siendo de acceso limitado en nuestro medio. Nos propusimos evaluar la utilidad del abordaje clásico en una cohorte conse- cutiva de 50 familias y describir la aplicación de NGS en un subgrupo de pacientes sin diagnóstico etioló- gico luego del enfoque clásico. Mediante el abordaje clásico se efectuó el diagnóstico en 27 (54%) familias. Posteriormente, 8 familias que quedaron sin diag- nóstico luego del algoritmo clásico, se evaluaron me- diante NGS, identificando el gen causal en 4 de ellas. Considerando ambos abordajes, el rédito diagnóstico fue 31/50 (62%) familias, con la siguiente distribu- ción: 38% desorden relacionado a MYH9, 8% síndro- me de Bernard-Soulier (4% clásico, 4% monoalélico), 4% síndrome de plaquetas grises, 4% desorden pla- quetario con predisposición a leucemia, 6% trom- bocitopenia relacionada a ANKRD26, 2% síndrome Wiskott-Aldrich. Los pacientes en los que no se pudo efectuar un diagnóstico etiológico presentaban trom- bocitopenia aislada leve, con aumento moderado del tamaño plaquetario y sangrado escaso.En conclusión, la aplicación de NGS permitió au- mentar el rédito diagnóstico, si bien sería necesa- rio ampliar la población estudiada para establecer el valor real de este abordaje en nuestro medio. Por lo tanto, el uso inicial del abordaje clásico, reserván- dose la aplicación posterior de NGS a los casos que permanecen sin diagnóstico luego de este enfoque, constituiría una alternativa útil en países con pocos recursos, apuntando a un diagnóstico adecuado que posibilite la pesquisa de complicaciones sindrómicas, oriente al tratamiento y consejo genético acertado.Diagnosis of inherited thrombocytopenias represents a true challenge owing to heterogeneity of these disorders and the absence of distinctive features in a substantial proportion of patients. Classical diagnostic approach is based on phenotypic characterization followed by molecular analysis of candidate genes guided by clinical suspicion. The introduction of next generation sequencing (NGS), that allows multiple genes analysis, is a high-cost alternative with limited access in our country. The aim of this work was to evaluate the utility of the classical approach in a consecutive cohort of 50 families and to describe the application of NGS in a subgroup of patients without an etiological diagnosis after the initial approach. Through the conventional approach, an etiologic diagnosis was made in 27 (54%) families. NGS was performed in 8 that remained without diagnosis after initial characterization, attaining a diagnosis in 4. Combining both approaches, the diagnostic yield was 31/50 (62%) families: 38% MYH9-related disorder, 8% Bernard-Soulier syndrome, 4% gray platelet syndrome, 4% familial platelet disorder with predisposition to leukemia, 6% ANKRD26-related thrombocytopenia, 2% Wiskott-Aldrich syndrome. Most patients without diagnosis had isolated macrothrombocytopenia and mild bleeding. NGS increased the diagnostic rate in this cohort, although it would be necessary to expand the population to establish its actual value in our setting. Therefore, the use of the classical approach and subsequent application of NGS in undiagnosed patients would represent a useful alternative in low-income countries, pointing out that a correct etiological diagnosis enables the detection of syndromic complications, appropriate treatment and adequate genetic counseling.Fil: Heller, Paula Graciela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Goette, Nora Paula. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Marin Oyarzún, Cecilia Paola. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Baroni Pietto, Maria Constanza. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Ayala, Daniela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Altuna, Diana R.. Instituto Universidad Escuela de Medicina del Hospital Italiano; ArgentinaFil: Arrieta, Maria Elizabeth. Hospital Público Descentralizado Dr. Guillermo Rawson.; ArgentinaFil: Arbesú, Guillermo. Hospital Dr. Humberto Notti; ArgentinaFil: Basqueira, Ana L.. Hospital Privado Universitario de Cordoba.; ArgentinaFil: Bazack, Nora. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez"; ArgentinaFil: Bonacorso, Silvina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; ArgentinaFil: Brodsky, Andrés. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; ArgentinaFil: Castro Rios, Miguel. No especifíca;Fil: Cosentini, María L.. Hospital Materno Infantil Doctor Hector Quintana ; Gobierno de la Provincia de Jujuy;Fil: Donato, Hugo Sebastian. Hospital Municipal del Niño de San Justo ; Municipalidad de la Matanza (buenos Aires);Fil: Korin, Jorge D.. No especifíca;Fil: Gomez, Silvina. No especifíca;Fil: Guglielmone, Hugo. Sanatorio Allende; ArgentinaFil: Lagrotta, Pablo. Hospital Nacional Profesor Alejandro Posadas.; ArgentinaFil: Marti, Alejandra. Provincia de Buenos Aires. Ministerio de Salud. Hospital Alta Complejidad en Red El Cruce Dr. Néstor Carlos Kirchner Samic; ArgentinaFil: Negro, Fernando Javier. Sanatorio Sagrado Corazon; ArgentinaFil: Rapetti, María C.. Hospital Municipal del Niño de San Justo ; Municipalidad de la Matanza (buenos Aires);Fil: Rosso, Diego. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños Pedro Elizalde (ex Casa Cuna); Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Ponzinibbio, Carlos. Hospital Italiano de La Plata; ArgentinaFil: Veber, Ernesto. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños Pedro Elizalde (ex Casa Cuna); ArgentinaFil: Zerga, Marta Elisa. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Oncología "Ángel H. Roffo"; ArgentinaFil: Molinas, Felisa Concepción. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Savoia, Anna. Instituto para la Salud Materna e Infancia; Italia. Università degli Studi di Trieste; ItaliaFil: Pecci, Alessandro. Universita Degli Studi Di Pavia; ItaliaFil: Marta, Rosana Fernanda. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; ArgentinaFil: Glembotsky, Ana Claudia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentin

Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2

We observed a syndrome of intermittent fevers, early-onset lacunar strokes and other neurovascular manifestations, livedoid rash, hepatosplenomegaly, and systemic vasculopathy in three unrelated patients. We suspected a genetic cause because the disorder presented in early childhood

Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

Funder: European Society of Intensive Care Medicine; doi: http://dx.doi.org/10.13039/501100013347Funder: Flemish Society for Critical Care NursesAbstract: Purpose: Intensive care unit (ICU) patients are particularly susceptible to developing pressure injuries. Epidemiologic data is however unavailable. We aimed to provide an international picture of the extent of pressure injuries and factors associated with ICU-acquired pressure injuries in adult ICU patients. Methods: International 1-day point-prevalence study; follow-up for outcome assessment until hospital discharge (maximum 12 weeks). Factors associated with ICU-acquired pressure injury and hospital mortality were assessed by generalised linear mixed-effects regression analysis. Results: Data from 13,254 patients in 1117 ICUs (90 countries) revealed 6747 pressure injuries; 3997 (59.2%) were ICU-acquired. Overall prevalence was 26.6% (95% confidence interval [CI] 25.9–27.3). ICU-acquired prevalence was 16.2% (95% CI 15.6–16.8). Sacrum (37%) and heels (19.5%) were most affected. Factors independently associated with ICU-acquired pressure injuries were older age, male sex, being underweight, emergency surgery, higher Simplified Acute Physiology Score II, Braden score 3 days, comorbidities (chronic obstructive pulmonary disease, immunodeficiency), organ support (renal replacement, mechanical ventilation on ICU admission), and being in a low or lower-middle income-economy. Gradually increasing associations with mortality were identified for increasing severity of pressure injury: stage I (odds ratio [OR] 1.5; 95% CI 1.2–1.8), stage II (OR 1.6; 95% CI 1.4–1.9), and stage III or worse (OR 2.8; 95% CI 2.3–3.3). Conclusion: Pressure injuries are common in adult ICU patients. ICU-acquired pressure injuries are associated with mainly intrinsic factors and mortality. Optimal care standards, increased awareness, appropriate resource allocation, and further research into optimal prevention are pivotal to tackle this important patient safety threat

Correction to: Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

The original version of this article unfortunately contained a mistake

Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

Funder: European Society of Intensive Care Medicine; doi: http://dx.doi.org/10.13039/501100013347Funder: Flemish Society for Critical Care NursesAbstract: Purpose: Intensive care unit (ICU) patients are particularly susceptible to developing pressure injuries. Epidemiologic data is however unavailable. We aimed to provide an international picture of the extent of pressure injuries and factors associated with ICU-acquired pressure injuries in adult ICU patients. Methods: International 1-day point-prevalence study; follow-up for outcome assessment until hospital discharge (maximum 12 weeks). Factors associated with ICU-acquired pressure injury and hospital mortality were assessed by generalised linear mixed-effects regression analysis. Results: Data from 13,254 patients in 1117 ICUs (90 countries) revealed 6747 pressure injuries; 3997 (59.2%) were ICU-acquired. Overall prevalence was 26.6% (95% confidence interval [CI] 25.9–27.3). ICU-acquired prevalence was 16.2% (95% CI 15.6–16.8). Sacrum (37%) and heels (19.5%) were most affected. Factors independently associated with ICU-acquired pressure injuries were older age, male sex, being underweight, emergency surgery, higher Simplified Acute Physiology Score II, Braden score 3 days, comorbidities (chronic obstructive pulmonary disease, immunodeficiency), organ support (renal replacement, mechanical ventilation on ICU admission), and being in a low or lower-middle income-economy. Gradually increasing associations with mortality were identified for increasing severity of pressure injury: stage I (odds ratio [OR] 1.5; 95% CI 1.2–1.8), stage II (OR 1.6; 95% CI 1.4–1.9), and stage III or worse (OR 2.8; 95% CI 2.3–3.3). Conclusion: Pressure injuries are common in adult ICU patients. ICU-acquired pressure injuries are associated with mainly intrinsic factors and mortality. Optimal care standards, increased awareness, appropriate resource allocation, and further research into optimal prevention are pivotal to tackle this important patient safety threat

ErbB2 is central to the integration of multiple signaling pathways involved in cardiac function

ErbB2 is a member of the epidermal growth factor receptor (EGFR/erbB1) family of tyrosine kinase receptors. Mutations resulting in the over-expression of erbB2 exhibit ligand-independent constitutive activation of its kinase domain that contributes to the pathogenesis of a variety of human cancers. The important role of erbB2 function in the adult heart was first observed in heart- specific erbB2 mutant mice (1, 2), which develop a phenotype consistent with that of dilated cardiomyopathy. The requirement of erbB2 in adult cardiac physiology was further demonstrated in clinical studies of breast cancer patients treated with Herceptin (Trastuzumab), a humanized monoclonal antibody targeting human erbB2 (Her2). A subset of these patients developed a similar cardiomyopathy to that observed in the heart-specific erbB2 mutant mice (3). In an effort to establish how the loss of erbB2-dependent signaling results in cardiac dysfunction, we developed a method for the isolation and culture of adult cardiac myocytes from wild type and erbB2 heart-specific mutant mice. In a combination of in vitro and in vivo studies, we show that erbB2 is required for key cardioprotective signaling pathways important for adult cardiac function. The present studies confirm the concept that the erbB receptor ligand, Neuregulin (NRG), requires erbB2 for NRG- dependent signaling by demonstrating that NRG-induced activation of the extracellular signal-regulated kinase 1/ 2 (Erk1/2) mitogen activated protein kinase (MAPK) survival pathway is absent in heart-specific erbB2 mutant mice. Interestingly, we show that erbB2 is not required for Erk1/2 activation in response to epidermal growth factor, another erbB receptor ligand. We extended our studies of the requirement of erbB2 for Erk1/2 activation elicited by other classes of ligands. We established that erbB2 is critical for G-protein coupled receptor (GPCR) agonists-, Leukemia Inhibitory Factor- (LIF), and Insulin- induced Erk1/2 activation. We established that erbB2 is required for both NRG- and LIF-induced signal transducer and activator of transcription 3 (STAT3) activation, another key cardioprotective signaling pathway. We extended our observations for the requirement of erbB2 in the cross-talk and signal integration in response to multiple classes of ligands by demonstrating that erbB2 forms a ligand-induced complex with GPCRs in vivo and in vitro and, importantly, is transactivated by a GPCR in adult mouse cardiac myocytes. We further showed that EGFR is not required for cross-talk with GPCR for GPCR agonist- induced Erk1/2 activation, as confirmed by our studies utilizing EGFR null cell lines. The ability of erbB2 to complex with multiple classes of receptors suggests a potential mechanism for erbB2 in integrating signaling network

La percepción del tiempo en la comunidad mapuche Millain Currical

Resumen temporalmente no disponible. La presente obra no cuenta con resumen provisto por el autor.Fil. Negro, Alejandra Lorena. Universidad de Buenos Aires. Facultad de Filosofía y Letras

Are the quality and stability of democracy “strengthened” in the Mercosur scenario?

La democracia se ha extendido y ha sido revalorizada a partir de la década de 1980. Sin embargo, hay un creciente desencanto con ella y se producen fuertes inestabilidades en numerosos casos, ya sea en el plano institucional o bien en el reconocimiento a la legitimidad del ganador electoral. ¿Es posible que el Mercosur provea de herramientas para fortalecer y proteger la calidad y estabilidad democrática de los Estados parte?Democracy has spread and has been revalued since the 1980s. However, there is a growing disenchantment with it and strong instability occurs in many cases, either at the institutional level or in the recognition of the legitimacy of the electoral winner. Is it possible for Mercosur to provide tools to strengthen and protect the democratic quality and stability of the member States?Facultad de Ciencias Jurídicas y Sociale