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Introducción y objetivos: La coartación aórtica es una de las enfermedades congénitas cardiovasculares más frecuentes, y su resolución quirúrgica se acompaña de un 11% de complicaciones a largo plazo, entre las cuales se encuentran los aneurismas y pseudoaneurismas. El objetivo del presente trabajo consiste en reportar la experiencia de nuestro centro en el manejo quirúrgico de los aneurismas de aorta descendente poscirugía de coartación aórtica. Métodos: Trabajo retrospectivo de 6 casos intervenidos desde enero de 2006 hasta abril de 2014, con antecedentes de corrección quirúrgica de coartación aórtica con parche en la infancia y diagnóstico de aneurisma de aorta descendente en el seguimiento. Resultados: De 6 pacientes, 4 eran varones y 2 mujeres; la edad promedio al momento de la primera cirugía fue de 8,2 ± 2,9 años, y la de la corrección del aneurisma de aorta descendente fue de 34,3 ± 5,1 años; el tiempo transcurrido desde la primera cirugía a la reintervención fue de 26 ± 5 años. Se realizó el reemplazo de la aorta descendente con prótesis de PTRC y se emplearon 2 estrategias de perfusión: en 5 pacientes se utilizó circulación extracorpórea y en uno se llevó a cabo derivación auriculofemoral con centrífuga. Cuatro pacientes registraron complicaciones menores (hemotórax, neumotórax, quilotórax, síndrome de Horner, disfonía y cefalea), y un paciente falleció. Los pacientes se encontraron asintomáticos durante un seguimiento promedio de 44,8 meses. Conclusiones: En pacientes jóvenes y con escasas comorbilidades, la cirugía ofrece una resolución definitiva para los aneurismas poscirugía de coartación aórtica, presentando una morbimortalidad aceptable

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The presence of light-absorbing particles, including black carbon in glaciers leads to a reduction in albedo (light reflection), leading to further melting of snow ice, increased amount of shortwave solar radiation and leads to the glacier. The objective has been to determine the variation in the temporal space of black carbon, the amount of light-absorbing particles and the decrease of albedo on the surface of the Ampay glacier. 10 snow samples were selected at various locations on the glacier during 2017. The light absorption heating method has been applied to measure light-absorbing particles, a technique that measures the temperature increase of the particle charge in a filter applying visible light that estimates the amount of light energy. The results show in terms of effective black carbon, the highest of 65,224 nanograms of black carbon per gram of water in the month of October and the minimum value of 20,941 nanograms of black carbon per gram of water in the month of February, typically associated with the rain. The energy absorbed by the light-absorbing particles in the Ampay glacier, the highest corresponds to the month of November with 8,952.92 J s/m² and lowest in February with 2,747.26 J s/m². In April, the amount of snow melted due to light-absorbing particles has been approximately 13.57 kg/m². Melting has increased considerably in other months with the largest melting, with a value of 26.65 kg/m2, almost 7.0 kW/m² of snow turned into water in the month of November. It is concluded that the technique of the Light absorption heating method is adequate because it is optimal for the achievement of the research objectives, it is economical, effective and has allowed quantifying light-absorbing particles in snow. [ABSTRACT FROM AUTHOR]

Introducción y objetivos: El tratamiento quirúrgico de la enfermedad renal vascular sigue teniendo resultados favorables cuando se realiza con criterio. El objetivo de este trabajo consiste en presentar 10 casos con distinta afección vascular renal y sus resultados a corto y a largo plazo. Métodos: Estudio retrospectivo de todos los pacientes con diagnóstico de aneurisma y/o estenosis de las arterias renales (AR) cuya enfermedad requirió resolución quirúrgica desde junio de 2000 a julio de 2015 en nuestro centro. Resultados: De 10 pacientes el 90% eran mujeres, edad media de 52 ± 17 años. El 90% tenía hipertensión arterial, 2 insuficiencia renal crónica y un paciente insuficiencia renal aguda. Cinco pacientes tenían un diagnóstico previo de fibrodisplasia muscular. El 30% había sido intervenido previamente con stents en las AR, y uno de ellos tenía como antecedente una cirugía de bypass aortorrenal. En 6 casos se realizó bypass a las AR con autoinjerto de safena, en otro paciente se colocó un tubo protésico de politetrafluoroetileno expandido, además se realizó una plastia de arteria renal y, en 2 casos, se llevó a cabo una nefrectomía para poder resolver la enfermedad que luego se autotrasplantó. La estancia hospitalaria fue de 9,2 ± 3,3 días y el tiempo de seguimiento fue de 5,8 ± 4,5 años. No se registró ninguna muerte intra ni perioperatoria. Conclusiones: A pesar de que en la actualidad el tratamiento no invasivo está indicado en un gran número de escenarios, la resolución quirúrgica sigue siendo la terapéutica de elección en pacientes seleccionados, teniendo buenos resultados.

The presence of light-absorbing particles, including black carbon in glaciers leads to a reduction in albedo (light reflection), leading to further melting of snow ice, increased amount of shortwave solar radiation and leads to the glacier. The objective has been to determine the variation in the temporal space of black carbon, the amount of light-absorbing particles and the decrease of albedo on the surface of the Ampay glacier. 10 snow samples were selected at various locations on the glacier during 2017. The light absorption heating method has been applied to measure light-absorbing particles, a technique that measures the temperature increase of the particle charge in a filter applying visible light that estimates the amount of light energy. The results show in terms of effective black carbon, the highest of 65,224 nanograms of black carbon per gram of water in the month of October and the minimum value of 20,941 nanograms of black carbon per gram of water in the month of February, typically associated with the rain. The energy absorbed by the light-absorbing particles in the Ampay glacier, the highest corresponds to the month of November with 8,952.92 J s/m2 and lowest in February with 2,747.26 J s/m2. In April, the amount of snow melted due to light-absorbing particles has been approximately 13.57 kg/m2. Melting has increased considerably in other months with the largest melting, with a value of 26.65 kg/m2, almost 7.0 kW/m2 of snow turned into water in the month of November. It is concluded that the technique of the Light absorption heating method is adequate because it is optimal for the achievement of the research objectives, it is economical, effective and has allowed quantifying light-absorbing particles in snow.

Anterior gradient 2 (AGR2) is a protein disulfide isomerase that is important for protein processing in the endoplasmic reticulum and is essential for mucin production in the digestive and respiratory tracts. Bi-allelic AGR2 variants were recently found to cause recurrent respiratory infections and failure to thrive with or without diarrhea (RIFTD; MIM # 620233), although the mechanisms behind this condition remain unclear. To date, at least 15 patients with homozygous AGR2 variants have been reported. Here, we report two affected siblings in a consanguineous family who had recurrent respiratory infections and digestive symptoms, one of whom needed lung transplantation. To identify the genetic cause of their symptoms, we performed exome sequencing and identified a novel homozygous missense variant in AGR2 (NM_006408.4, c.250A>C, p.(Ser84Arg)) in both affected siblings. Both parents had the identical variant in a heterozygous state. This variant is quite rare in the general population and is clinically compatible with RIFTD, substituting a highly conserved CXXS motif with CXXR. We performed structural modeling and functional studies to investigate the effect of this variant. Through transient overexpression, Ser84Arg AGR2 decreased protein stability, and promoted aberrant dimerization under non-reducing conditions. AGR2 functions in a monomer-dimer equilibrium. Size-exclusion chromatography showed that the Ser84Arg mutant had a larger molecular size than the wild-type protein under non-reducing, but not reducing conditions, indicating that Ser84Arg enhanced intermolecular disulfide bonds. In conclusion, we identified a novel pathogenic AGR2 variant and indicated its abnormal monomer-dimer equilibrium as a possible mechanism involved in the pathogenesis of RIFTD., Competing Interests: Declarations. Ethical Approval: This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the institution review board of the National Center for Global Health and Medicine (NCGM-A-004163-10). Consent to Participate: Written consent was obtained from the patients and their parents. Consent for Publication: Not applicable. Competing Interests: The authors declare no competing interests., (© 2024. The Author(s).)

Embedding optical fibers sensors into composite structures for Structural Health Monitoring purposes is not just one of the most attractive solutions contributing to smart structures, but also the optimum integration approach that insures maximum protection and integrity of the fibers. Nevertheless this intended integration level still remains an industrial challenge since today there is no mature integration process in composite plants matching all necessary requirements. This article describes the process developed to integrate optical fiber sensors in the Production cycle of a test specimen. The sensors, Bragg gratings, were integrated into the laminate during automatic tape lay-up and also by a secondary bonding process, both in the Airbus Composite Plant. The test specimen, completely representative of the root joint of the lower wing cover of a real aircraft, is comprised of a structural skin panel with the associated stringer run out. The ingress-egress was achieved through the precise design and integration of miniaturized optical connectors compatible with the manufacturing conditions and operational test requirements. After production, the specimen was trimmed, assembled and bolted to metallic plates to represent the real triform and buttstrap, and eventually installed into the structural test rig. The interrogation of the sensors proves the effectiveness of the integration process; the analysis of the strain results demonstrate the good correlation between fiber sensors and electrical gauges in those locations where they are installed nearby, and the curvature and load transfer analysis in the bolted stringer run out area enable demonstration of the consistency of the fiber sensors measurements. In conclusion, this work presents strong evidence of the performance of embedded optical sensors for structural health monitoring purposes, where in addition and most importantly, the fibers were integrated in a real production environment and the ingress-egress issue was solved by the design and integration of miniaturized connectors compatible with the manufacturing and structural test phases., Competing Interests: The authors declare no conflicts of interest.

The deep ocean is the largest and least known ecosystem on Earth. It hosts numerous pelagic organisms, most of which are able to emit light. Here we present a unique data set consisting of a 2.5-year long record of light emission by deep-sea pelagic organisms, measured from December 2007 to June 2010 at the ANTARES underwater neutrino telescope in the deep NW Mediterranean Sea, jointly with synchronous hydrological records. This is the longest continuous time-series of deep-sea bioluminescence ever recorded. Our record reveals several weeks long, seasonal bioluminescence blooms with light intensity up to two orders of magnitude higher than background values, which correlate to changes in the properties of deep waters. Such changes are triggered by the winter cooling and evaporation experienced by the upper ocean layer in the Gulf of Lion that leads to the formation and subsequent sinking of dense water through a process known as "open-sea convection". It episodically renews the deep water of the study area and conveys fresh organic matter that fuels the deep ecosystems. Luminous bacteria most likely are the main contributors to the observed deep-sea bioluminescence blooms. Our observations demonstrate a consistent and rapid connection between deep open-sea convection and bathypelagic biological activity, as expressed by bioluminescence. In a setting where dense water formation events are likely to decline under global warming scenarios enhancing ocean stratification, in situ observatories become essential as environmental sentinels for the monitoring and understanding of deep-sea ecosystem shifts.