Objectives The primary aim of this systematic review is to objectively evaluate the test performance characteristics of three-dimensional echocardiography (3DE) in measuring left ventricular (LV) ...volumes and ejection fraction (EF). Background Despite its growing use in clinical laboratories, the accuracy of 3DE has not been studied on a large scale. It is unclear if this technology offers an advantage over traditional two-dimensional (2D) methods. Methods We searched for studies that compared LV volumes and EF measured by 3DE and cardiac magnetic resonance (CMR) imaging. A subset of those also compared standard 2D methods with CMR. We used meta-analyses to determine the overall bias and limits of agreement of LV end-diastolic volume (EDV), end-systolic volume (ESV), and EF measured by 3DE and 2D echocardiography (2DE). Results Twenty-three studies (1,638 echocardiograms) were included. The pooled biases ± 2 SDs for 3DE were −19.1 ± 34.2 ml, −10.1 ± 29.7 ml, and − 0.6 ± 11.8% for EDV, ESV, and EF, respectively. Nine studies also included data from 2DE, where the pooled biases were −48.2 ± 55.9 ml, −27.7 ± 45.7 ml, and 0.1 ± 13.9% for EDV, ESV, and EF, respectively. In this subset, the difference in bias between 3DE and 2D volumes was statistically significant (p = 0.01 for both EDV and ESV). The difference in variance was statistically significant (p < 0.001) for all 3 measurements. Conclusions Three-dimensional echocardiography underestimates volumes and has wide limits of agreement, but compared with traditional 2D methods in these carefully performed studies, 3DE is more accurate for volumes and more precise in all 3 measurements.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited genetic myocardial disease characterized by fibrofatty replacement of the myocardium and a predisposition to cardiac arrhythmias ...and sudden death. We evaluated the cardiomyopathy gene titin (TTN) as a candidate ARVC gene because of its proximity to an ARVC locus at position 2q32 and the connection of the titin protein to the transitional junction at intercalated disks.
All 312 titin exons known to be expressed in human cardiac titin and the complete 3' untranslated region were sequenced in 38 ARVC families. Eight unique TTN variants were detected in 7 families, including a prominent Thr2896Ile mutation that showed complete segregation with the ARVC phenotype in 1 large family. The Thr2896IIe mutation maps within a highly conserved immunoglobulin-like fold (Ig10 domain) located in the spring region of titin. Native gel electrophoresis, nuclear magnetic resonance, intrinsic fluorescence, and proteolysis assays of wild-type and mutant Ig10 domains revealed that the Thr2896IIe exchange reduces the structural stability and increases the propensity for degradation of the Ig10 domain. The phenotype of TTN variant carriers was characterized by a history of sudden death (5 of 7 families), progressive myocardial dysfunction causing death or heart transplantation (8 of 14 cases), frequent conduction disease (11 of 14), and incomplete penetrance (86%).
Our data provide evidence that titin mutations can cause ARVC, a finding that further expands the origin of the disease beyond desmosomal proteins. Structural impairment of the titin spring is a likely cause of ARVC and constitutes a novel mechanism underlying myocardial remodeling and sudden cardiac death.
This study analyses the presence of pesticides in 106 samples of raw propolis from Spain and Chile. The components detected and quantified using gas chromatography mass spectrometry were as follows: ...triamidefon present in 70.1% of the samples with concentrations between 0.35 and 42.17mg/kg, and dicofol detected in 7.5% of the samples with concentrations between 0.39 and 2.83mg/kg. Diclorofluanid, procymidone, folpet, propham and metazachlor were also detected but only in a small number of samples. Near infrared spectroscopy with a remote reflectance fibre-optic probe was used to detect samples contaminated with triadimefon via partial least squares discriminant, with a R2 value of 0.71. In addition, the presence of the pesticide triadimefon in propolis was quantified using the modified partial least squares (MPLS) regression method. The calibration results showed a coefficient of determination (R2) of 0.81, a root mean square error (RMSE) of 0.36, and a ratio of performance to deviation (RPD) of 2.5.
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The macrotidal Colorado River Delta at the northern end of the Gulf of California in Mexico is hydrologically complex. We review historical accounts, data, field notes and photographs to evaluate the ...hydrological processes active on the delta prior to the advent of upstream dams. We also employ satellite imagery as well as recent LIDAR data to illustrate the critical role played by headcut erosion in restoring the river's fluvial/tidewater connection during the 1979–1988 floods. Prior to human manipulation, the river's contribution of fresh water to the Gulf was periodically interrupted by natural overflowing, avulsing, and flooding into the sub‐sea level Salton Sink on the north slope of the delta plain. River flow south towards the Gulf was also subject to occasional overflow into Laguna Salada, another sub‐sea level basin. In the mid‐20th century, the Delta was disconnected from its fluvial supply following installation of upstream dams and reservoirs. A tidal sediment obstruction developed in the estuary channel, forming a final barrier to fluvial connectivity. Release of Colorado River floodwaters into Mexico between 1979 and 1988 provided a natural experiment on the hydrological response of a long‐disconnected macrotidal delta to restoration of fluvial supply.
During the 1980s, floodwaters reached Mexico's Colorado River Delta for the first time in a quarter century providing a natural experiment on the hydrological response of a long‐disconnected macrotidal delta to restoration of fluvial supply. We analysed historical aerial photos, satellite images, and LiDAR data to build a chronology of hydrological developments as the river restored its tidewater connection to the Gulf by the process of headcut migration and channel incision.
Objectives The aim of this study was to discern the role of the cardiac voltage-gated sodium ion channel SCN5A in the etiology of dilated cardiomyopathy (DCM). Background Dilated cardiomyopathy ...associates with mutations in the SCN5A gene, but the frequency, phenotype, and causative nature of these associations remain the focus of ongoing investigation. Methods Since 1991, DCM probands and family members have been enrolled in the Familial Cardiomyopathy Registry and extensively evaluated by clinical phenotype. Genomic deoxyribonucleic acid samples from 338 individuals among 289 DCM families were obtained and screened for SCN5A mutations by denaturing high-performance liquid chromatography and sequence analysis. Results We identified 5 missense SCN5A mutations among our DCM families, including novel mutations E446K, F1520L, and V1279I, as well as previously reported mutations D1275N and R222Q. Of 15 SCN5A mutation carriers in our study, 14 (93%) manifested arrhythmia: supraventricular arrhythmia (13 of 15), including sick sinus syndrome (5 of 15) and atrial fibrillation (9 of 15), ventricular tachycardia (5 of 15), and conduction disease (9 of 15). Conclusions Mutations in SCN5A were detected in 1.7% of DCM families. Two-thirds (6 of 9) of all reported DCM mutations in SCN5A localize to the highly conserved homologous S3 and S4 transmembrane segments, suggesting a shared mechanism of disruption of the voltage-sensing mechanism of this channel leading to DCM. Not surprisingly, SCN5A mutation carriers show a strong arrhythmic pattern that has clinical and diagnostic implications.
Los sistemas de pensiones en todo el mundo han evolucionado dinámicamente, es necesario alcanzar la sostenibilidad del sistema para proporcionar a sus aportantes y pensionistas confianza en el ...modelo. Para el sistema de pensiones del IESS, es importante obtener predicciones para prevenir a los interesados lo que podría suceder si no se toman las medidas correctivas. Las predicciones de sostenibilidad de pensiones del IESS se obtuvieron con el método de las Cadenas de Markov Monte Carlo (CMMC) y el de los Mínimos Cuadrados, utilizando datos desde el 1 de enero del 2012 hasta el 30 de junio de 2022 y su procesamiento con el Software GNU Octave. Los resultados de los modelos revelan que el sistema de pensiones sería sostenible hasta diciembre de 2023; por lo que las autoridades deberían aplicar las estrategias adecuadas para sostener el sistema.
Resumen Introducción La epilepsia benigna con puntas centro-temporales (EBPCT) es el síndrome epiléptico más frecuente de la infancia, tiene carácter edad-dependiente, elevada predisposición genética ...y curso benigno. El objetivo de este trabajo es describir el curso clínico y el pronóstico de 60 pacientes diagnosticados de EBPCT en nuestro centro. Pacientes y métodos Revisión retrospectiva de los pacientes diagnosticados de EBPCT en un hospital universitario (1995-2009). Se dividieron en 2 grupos: a) pacientes que cumplían todos los criterios clásicos de EBPCT, y b) cumplían los criterios excepto uno (menos de 4 años; crisis en vigilia; alteraciones EEG no típicas). Resultados Se seleccionó a 60 pacientes, 34 varones y 26 mujeres. Se incluyó a 31 pacientes en el grupo 1 y a 29 en el grupo 2. Edad media de inicio en el grupo 1: 7,45 años; grupo 2: 6,55 años. Se indicó tratamiento médico en 32,2% de pacientes del grupo 1, y en 41,3% del grupo 2. La evolución fue favorable en la mayoría: 58% en el grupo 1 y 62,1% en el 2 estaban libres de crisis tras un año. Edad media a la que desaparecieron: 8,54 años en el grupo 1 y 7,84 años en el grupo 2. No se encontraron diferencias estadísticamente significativas en ninguno de estos parámetros. Conclusiones A diferencia de lo que algunos autores habían publicado, en este trabajo no se han identificado factores clínicos de mala evolución en pacientes con EBPCT, de modo que su diagnóstico se correlaciona con una evolución favorable y un excelente pronóstico neurológico.
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