Síndrome de lisis tumoral espontáneo

Tumor lysis syndrome is a complication that usually occurs at the beginning of antineoplastic treatment, being the cases of spontaneous appearance very rare and even less associated with solid tumors.We present the case of a 69-year-old male with the constitutional syndrome and the presence of multiple hepatic micronodules, evolving rapidly and progressively to a spontaneous tumor lysis syndrome.El síndrome de lisis tumoral es una complicación que habitualmente ocurre al inicio del tratamiento antineoplásico, siendo los casos de aparición espontánea muy poco frecuentes y menos aún, asociado a tumores sólidos. Presentamos el caso de un varón de 69 años con síndrome constitucional y presencia de múltiples micronódulos hepáticos, evolucionando de forma rápida y progresiva a un síndrome de lisis tumoral espontáneo

Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry

Common variable immunodeficiency (CVID) constitutes a heterogenic group of primary immunodeficiency disorders with a wide-ranging clinical spectrum. CVID-associated non-infectious morbidity constitutes a major challenge requiring a full understanding of its pathophysiology and its clinical importance and global variability, especially considering the broad clinical, genetic, and regional heterogeneity of CVID disorders. This work aimed to develop a nationwide, multicenter, retrospective study over a 3-year period describing epidemiological, clinical, laboratory, therapeutic, and prognostic features of 250 CVID patients in Spain. The mean diagnostic delay was around 10 years and most patients initially presented with infectious complications followed by non-infectious immune disorders. However, infectious diseases were not the main cause of morbimortality. Non-infectious lung disease was extraordinarily frequent in our registry affecting approximately 60% of the patients. More than one-third of the patients in our cohort showed lymphadenopathies and splenomegaly in their follow-up, and more than 33% presented immune cytopenias, especially Evans' syndrome. Gastrointestinal disease was observed in more than 40% of the patients. Among biopsied organs in our cohort, benign lymphoproliferation was the principal histopathological alteration. Reaching 15.26%, the global prevalence of cancer in our registry was one of the highest reported to date, with non-Hodgkin B lymphoma being the most frequent. These data emphasize the importance of basic and translational research delving into the pathophysiological pathways involved in immune dysregulation and diffuse lymphocytic infiltration. This would reveal new tailored strategies to reduce immune complications, and the associated healthcare burden, and ensure a better quality of life for CVID patients

Natural History of MYH7-Related Dilated Cardiomyopathy

BACKGROUND Variants in myosin heavy chain 7 (MYH7) are responsible for disease in 1% to 5% of patients with dilated cardiomyopathy (DCM); however, the clinical characteristics and natural history of MYH7-related DCM are poorly described. OBJECTIVES We sought to determine the phenotype and prognosis of MYH7-related DCM. We also evaluated the influence of variant location on phenotypic expression. METHODS We studied clinical data from 147 individuals with DCM-causing MYH7 variants (47.6% female; 35.6 +/- 19.2 years) recruited from 29 international centers. RESULTS At initial evaluation, 106 (72.1%) patients had DCM (left ventricular ejection fraction: 34.5% +/- 11.7%). Median follow-up was 4.5 years (IQR: 1.7-8.0 years), and 23.7% of carriers who were initially phenotype-negative developed DCM. Phenotypic expression by 40 and 60 years was 46% and 88%, respectively, with 18 patients (16%) first diagnosed at <18 years of age. Thirty-six percent of patients with DCM met imaging criteria for LV noncompaction. During follow-up, 28% showed left ventricular reverse remodeling. Incidence of adverse cardiac events among patients with DCM at 5 years was 11.6%, with 5 (4.6%) deaths caused by end-stage heart failure (ESHF) and 5 patients (4.6%) requiring heart transplantation. The major ventricular arrhythmia rate was low (1.0% and 2.1% at 5 years in patients with DCM and in those with LVEF of <= 35%, respectively). ESHF and major ventricular arrhythmia were significantly lower compared with LMNA-related DCM and similar to DCM caused by TTN truncating variants. CONCLUSIONS MYH7-related DCM is characterized by early age of onset, high phenotypic expression, low left ventricular reverse remodeling, and frequent progression to ESHF. Heart failure complications predominate over ventricular arrhythmias, which are rare. (C) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation

Short-term changes in klotho and FGF23 in heart failure with reduced ejection fraction—a substudy of the DAPA-VO2 study

The klotho and fibroblast growth factor 23 (FGF-23) pathway is implicated in cardiovascular pathophysiology. This substudy aimed to assess the changes in klotho and FGF-23 levels 1-month after dapagliflozin in patients with stable heart failure and reduced ejection fraction (HFrEF). The study included 29 patients (32.2% of the total), with 14 assigned to the placebo group and 15 to the dapagliflozin, as part of the double-blind, randomized clinical trial [DAPA-VO2 (NCT04197635)]. Blood samples were collected at baseline and after 30 days, and Klotho and FGF-23 levels were measured using ELISA Kits. Between-treatment changes (raw data) were analyzed by using the Mann-Whitney test and expressed as median (p25%–p75%). Linear regression models were utilized to analyze changes in the logarithm (log) of klotho and FGF-23. The median age was 68.3 years (60.8–72.1), with 79.3% male and 81.5% classified as NYHA II. The baseline medians of left ventricular ejection fraction, glomerular filtration rate, NT-proBNP, klotho, and FGF-23 were 35.8% (30.5–37.8), 67.4 ml/min/1.73 m2 (50.7–82.8), 1,285 pg/ml (898–2,305), 623.4 pg/ml (533.5–736.6), and 72.6 RU/ml (62.6–96.1), respectively. The baseline mean peak oxygen uptake was 13.1 ± 4.0 ml/kg/min. Compared to placebo, patients on dapagliflozin showed a significant median increase of klotho [Δ+29.5, (12.9–37.2); p = 0.009] and a non-significant decrease of FGF-23 [Δ−4.6, (−1.7 to −5.4); p = 0.051]. A significant increase in log-klotho (p = 0.011) and a decrease in log-FGF-23 (p = 0.040) were found in the inferential analysis. In conclusion, in patients with stable HFrEF, dapagliflozin led to a short-term increase in klotho and a decrease in FGF-23

Impact of COVID-19 on cardiovascular testing in the United States versus the rest of the world

Objectives: This study sought to quantify and compare the decline in volumes of cardiovascular procedures between the United States and non-US institutions during the early phase of the coronavirus disease-2019 (COVID-19) pandemic. Background: The COVID-19 pandemic has disrupted the care of many non-COVID-19 illnesses. Reductions in diagnostic cardiovascular testing around the world have led to concerns over the implications of reduced testing for cardiovascular disease (CVD) morbidity and mortality. Methods: Data were submitted to the INCAPS-COVID (International Atomic Energy Agency Non-Invasive Cardiology Protocols Study of COVID-19), a multinational registry comprising 909 institutions in 108 countries (including 155 facilities in 40 U.S. states), assessing the impact of the COVID-19 pandemic on volumes of diagnostic cardiovascular procedures. Data were obtained for April 2020 and compared with volumes of baseline procedures from March 2019. We compared laboratory characteristics, practices, and procedure volumes between U.S. and non-U.S. facilities and between U.S. geographic regions and identified factors associated with volume reduction in the United States. Results: Reductions in the volumes of procedures in the United States were similar to those in non-U.S. facilities (68% vs. 63%, respectively; p = 0.237), although U.S. facilities reported greater reductions in invasive coronary angiography (69% vs. 53%, respectively; p < 0.001). Significantly more U.S. facilities reported increased use of telehealth and patient screening measures than non-U.S. facilities, such as temperature checks, symptom screenings, and COVID-19 testing. Reductions in volumes of procedures differed between U.S. regions, with larger declines observed in the Northeast (76%) and Midwest (74%) than in the South (62%) and West (44%). Prevalence of COVID-19, staff redeployments, outpatient centers, and urban centers were associated with greater reductions in volume in U.S. facilities in a multivariable analysis. Conclusions: We observed marked reductions in U.S. cardiovascular testing in the early phase of the pandemic and significant variability between U.S. regions. The association between reductions of volumes and COVID-19 prevalence in the United States highlighted the need for proactive efforts to maintain access to cardiovascular testing in areas most affected by outbreaks of COVID-19 infection

Efecto del herbivorismo en la conservación de la especie amenazada Androcymbium europaeum (Lange) K. Richt = Effect of herbivorism on the conservation of the endangered species Androcymbium europaeum (Lange) K. Richt

RESUMENA. europaeum es un endemismo ibero-magrebí incluido en la Lista Roja de la Flora Vascular Española (Moreno et al., 2008). Los objetivos del trabajo han sido evaluar el efecto del ganado ovino doméstico y del conejo sobre la densidad de individuos de A. europaeum, así como analizar la evolución de ésta especie durante cinco años de seguimiento en el Parque Natural de Cabo de Gata-Níjar. Se realizó un seguimiento durante los años 2010 a 2014, con un diseño en bloques aleatorizados (seis) con tres tratamientos (tipo de manejo) por bloque: 1) con herbivorismo de ganado y conejo (G+C+), 2) excluidas sólo al ganado (G-C+) y, 3) excluidas al conejo y ganado (G-C-); en el año 2010 no hubo exclusión. Analizando sólo las zonas con herbivorismo, se observa un aumento paulatino de la densidad desde 2010 a 2014, con efecto significativo de los años y los bloques. Durante los cuatro años de exclusión (2011-2014) nuevamente se advierte la misma respuesta para años y bloques. Sin embargo, el factor tipo de manejo no muestra efecto significativo sobre la densidad, aunque presenta mayor densidad media (individuos 0,25 m-2) el tratamiento G+C+, 67,30, respecto a G-C+, 53,85 y G-C- 60, 84. Este aumento también puede observarse si consideramos el incremento de la densidad del último año en relación a 2010: 241 % (G+C+) respecto a 200 % (G-C+) y 229 % (G-C-). Estos datos sugieren que la actual gestión de la zona de estudio es sostenible para la supervivencia de A. europaeum y que el herbivorismo, mayoritariamente ganado y conejo, no está afectando significativamente a la supervivencia de la especie, percibiéndose una tendencia a incrementar la densidad en las zonas no excluidas. Además, parece que la especie se ve influenciada más por las variaciones interanuales que por las debidas al tipo de manejo. ABSTRACTA. europaeum is an ibero-maghreb endemism which is included in the Red List of the Spanish Vascular Flora (Moreno et al., 2008). The aims of this work have been to evaluate the effect of domestic livestock and rabbit on the density of individuals of A. europaeum as well as to analyze the evolution of this species during a five-year survey in Cabo de Gata-Níjar Natural Park. Monitoring was performed from 2010 until 2014, following a randomized block design (six blocks) with three treatments (type of management) per block: 1) with herbivorism of sheep and rabbit (G+C+), only excluded to livestock (G-C+) and, 3) excluded to rabbit and livestock (G-C-).When analyzing only the areas with herbivorism, a gradual increase in density from 2010 to 2014 is observed, with significant effects of years and blocks. During the four years with exclusions (2011-2014) the same response of years and blocks is detected, but no significant differences among the three types of management are observed. Nevertheless the mean density (individual 0.25 m-2) was higher for G+C+, 67.30, with respect to G-C+, 53.85and G-C-60.84. This increment can be also detected if we compare the density of 2014 with respect to that of 2010: 241 % (G+C+), 200 % (G-C+) and 229 % (G-C+). These data suggest that the current management of A. europaeum in the study area is adequate and that herbivorism,mainly by livestock and rabbit, is not significantly affecting the survivorship of this species, since an increasing trend for density in non-excluded areas has been detected. Furthermore, it seems that the species responds more rapidly to interanual climatic variations than to those caused by the type of management

Tierra de Campos, nuestra tierra

Proyecto realizado por siete profesores del colegio público comarcal 'Conde de Vallellano' de Ampudia (Palencia). Tiene como objetivo: valorar y apreciar la comarca y respetar el entorno, así como conocer el legado de nuestros antepasados. El proceso de desarrollo seguido: 1) Acuerdo del claustro de acometer el proyecto. 2) Profesores y alumnos de 2õ y 3õ ciclo de primaria responsables de elaborar la letra de las canciones. 3) Participación del resto de los alumnos, padres y comunidad educativa en la realización final. 4) Consenso sobre tipo de música a poner en las canciones. 5) Grabación final de la cinta. Este proyecto de innovación ha incidido en: Aspectos organizativos del centro; Participación de la comunidad educativa; Metodología; Participación de la comunidad educativa; Metodología; Actitud en actividades relacionadas con el medio ambiente. Los materiales aportados son una cinta en musicasette con dos canciones grabadas 'Tierra de Campos', dedicada a la comarca y 'Tú decides para la región de Castilla y León. El trabajo no ha sido publicado.Comunidad Autónoma de Castilla y León. Consejería de Educación y Cultura. Dirección General de EducaciónCastilla y LeónES

Talleres en la escuela : medio didáctico para la adaptación de niños con problemas de integración social

Se plantea la organización de talleres como la tarea educativa más adecuada al tipo de alumnos de este centro: gitanos y payos de escaso nivel económico y social. Los objetivos son: desarrollar la formación integral del alumno mediante actividades prácticas, llevar a cabo medidas de adaptación e integración social mediante hábitos de convivencia, intentar nivelar las carencias culturales, y enseñar habilidades y destrezas básicas. Los talleres organizados son: Huerto, Educación Física, Psicomotricidad, etc. Se adjuntan fichas de evaluación del proyecto, donde se detallan las particularidades de cada taller y cada ciclo..Madrid (Comunidad Autónoma). Consejería de Educación y CulturaMadridMadrid (Comunidad Autónoma). Subdirección General de Formación del Profesorado. CRIF Las Acacias; General Ricardos 179 - 28025 Madrid; Tel. + 34915250893ES