Linfoma angiocéntrico centrofacial

El linfoma angiocéntrico centrofacial es una neoplasia linfoide rara, con un diagnóstico a menudo difícil, debido al cuadro clínico inespecífico y a que muchas veces son necesarias varias biopsias para llegar a un diagnóstico correcto. Se trata de un linfoma no Hodgkin(LNH) agresivo, de localización preferente en el tracto respiratorio superior (sobre todo en cavidad nasal), y con un pronóstico ominoso, ya que la supervivencia media es de 12-18 meses aproximadamente(1). Predomina en orientales y sudamericanos, entre los 50-60 años de edad, y con ligera preferencia por el sexo masculino (2:1). Se presenta el caso de una paciente ecuatoriana que acude a nuestro servicio con edema hemifacial, rinorrea achocolatada y obstrucción respiratoria nasal de 1 mes de evolución, sin respuesta al tratamiento antibiótico y antiinflamatorio, que tras realizar varias pruebas diagnósticas se evidenció histológicamente la presencia de un linfoma T extranodal de tipo nasal (también llamado linfoma de células T angiocéntrico).The centrofacial angiocentric lymphoma is a rare lymphoid neoplasm, with an often-difficult diagnosis due to the non-specific clinical picture. On many occasions it is necessary to perform various biopsies to reach the correct diagnosis. This lymphoma is an aggressive Non-Hodgkin's (NHL) type, which is normally found in the upper respiratory tract (predominantly in the nasal cavity), and has an ominous prognosis, as the average survival rate is between 12 and 18 months (1). It is predominantly found in subjects of oriental and South American extraction, who are between the ages of 50 and 60 years and with a slight tendency towards males (2:1). This is the case study of a female Ecuadorian patient who was referred to our department with a hemifacial edema, chocolate- like rhinorrhea and nasal respiratory obstruction, which had been treated with antibiotics and anti-inflammatories for a month without success. After performing a number of diagnostic tests, it was found histologically that the patient had an extranodal T-cell lymphoma of the nasal type (also known as T-cell angiocentric lymphoma)

Neural network controller for active demand side management with PV energy in the residential sector

In this paper, we describe the development of a control system for Demand-Side Management in the residential sector with Distributed Generation. The electrical system under study incorporates local PV energy generation, an electricity storage system, connection to the grid and a home automation system. The distributed control system is composed of two modules: a scheduler and a coordinator, both implemented with neural networks. The control system enhances the local energy performance, scheduling the tasks demanded by the user and maximizing the use of local generation

Speckle noise and dynamic range in coronagraphic images

This paper is concerned with the theoretical properties of high contrast coronagraphic images in the context of exoplanet searches. We derive and analyze the statistical properties of the residual starlight in coronagraphic images, and describe the effect of a coronagraph on the speckle and photon noise. Current observations with coronagraphic instruments have shown that the main limitations to high contrast imaging are due to residual quasi-static speckles. We tackle this problem in this paper, and propose a generalization of our statistical model to include the description of static, quasi-static and fast residual atmospheric speckles. The results provide insight into the effects on the dynamic range of wavefront control, coronagraphy, active speckle reduction, and differential speckle calibration. The study is focused on ground-based imaging with extreme adaptive optics, but the approach is general enough to be applicable to space, with different parameters.Comment: 31 pages, 18 figure

Frontal sinus mucocele with intracranial and intraorbital extension

ABSTRACT Introduction: Frontal sinus mucoceles can present with a multitude of different symptoms including ophthalmic disturbances. Even benign, they have a tendency to expand by eroding the surrounding bony walls that displaces and destroys structures by pressure and bony resorption. Case report: A 32-year-old man with diplopia, proptosis of the right eye and headache was presented. The diagnosis was frontal sinus mucocele with intracranial and intraorbital extension. Possible clinical manifestations of mucoceles, diagnostic imaging techniques and treatment used are discussed. Conclusion: Frontal mucoceles are benign and curable, early recognition and management of them is of paramount importance, because they can cause local, orbital or intracranial complications

Frontal sinus mucocele with intracranial and intraorbital extension

ABSTRACT Introduction: Frontal sinus mucoceles can present with a multitude of different symptoms including ophthalmic disturbances. Even benign, they have a tendency to expand by eroding the surrounding bony walls that displaces and destroys structures by pressure and bony resorption. Case report: A 32-year-old man with diplopia, proptosis of the right eye and headache was presented. The diagnosis was frontal sinus mucocele with intracranial and intraorbital extension. Possible clinical manifestations of mucoceles, diagnostic imaging techniques and treatment used are discussed. Conclusion: Frontal mucoceles are benign and curable, early recognition and management of them is of paramount importance, because they can cause local, orbital or intracranial complications

PV self-consumption optimization with storage and Active DSM for the residential sector

With the rising prices of the retail electricity and the decreasing cost of the PV technology, grid parity with commercial electricity will soon become a reality in Europe. This fact, together with less attractive PV feed-in-tariffs in the near future and incentives to promote self-consumption suggest, that new operation modes for the PV Distributed Generation should be explored; differently from the traditional approach which is only based on maximizing the exported electricity to the grid. The smart metering is experiencing a growth in Europe and the United States but the possibilities of its use are still uncertain, in our system we propose their use to manage the storage and to allow the user to know their electrical power and energy balances. The ADSM has many benefits studied previously but also it has important challenges, in this paper we can observe and ADSM implementation example where we propose a solution to these challenges. In this paper we study the effects of the Active Demand-Side Management (ADSM) and storage systems in the amount of consumed local electrical energy. It has been developed on a prototype of a self-sufficient solar house called “MagicBox” equipped with grid connection, PV generation, lead–acid batteries, controllable appliances and smart metering. We carried out simulations for long-time experiments (yearly studies) and real measures for short and mid-time experiments (daily and weekly studies). Results show the relationship between the electricity flows and the storage capacity, which is not linear and becomes an important design criterion

Projecting Climate Dependent Coastal Flood Risk With a Hybrid Statistical Dynamical Model

ABSTRACT: Numerical models for tides, storm surge, and wave runup have demonstrated ability to accurately define spatially varying flood surfaces. However these models are typically too computationally expensive to dynamically simulate the full parameter space of future oceanographic, atmospheric, and hydrologic conditions that will constructively compound in the nearshore to cause both extreme event and nuisance flooding during the 21st century. A surrogate modeling framework of waves, winds, and tides is developed in this study to efficiently predict spatially varying nearshore and estuarine water levels contingent on any combination of offshore forcing conditions. The surrogate models are coupled with a time-dependent stochastic climate emulator that provides efficient downscaling for hypothetical iterations of offshore conditions. Together, the hybrid statistical-dynamical framework can assess present day and future coastal flood risk, including the chronological characteristics of individual flood and wave-induced dune overtopping events and their changes into the future. The framework is demonstrated at Naval Base Coronado in San Diego, CA, utilizing the regional Coastal Storm Modeling System (CoSMoS; composed of Delft3D and XBeach) as the dynamic simulator and Gaussian process regression as the surrogate modeling tool. Validation of the framework uses both in-situ tide gauge observations within San Diego Bay, and a nearshore cross-shore array deployment of pressure sensors in the open beach surf zone. The framework reveals the relative influence of large-scale climate variability on future coastal flood resilience metrics relevant to the management of an open coast artificial berm, as well as the stochastic nature of future total water levels.This work was funded by the Strategic Environmental Research Development Program (DOD/SERDP RC-2644). Any use of trade, firm, or product names is for descriptive purposes only and does not imply endorsement by the U.S. Government. F. J. Mendez, A. Rueda, and L. Cagigal acknowledge the partial funding from the Spanish Ministry of Science and Innovation, project Beach4cast PID2019-107053RB-I00. The authors thank the Scripps Center for Coastal Studies for their efforts to deploy, recover, and process surf zone pressure sensor data used as validation in this study. The authors thank Melisa Menendez for sharing GOW2 hindcast data for Southern California. The authors thank the sea-level rise projection authors for developing and making the sea-level rise projections available, multiple funding agencies for supporting the development of the projections, and the NASA Sea-Level Change Team for developing and hosting the IPCC AR6 Sea-Level Projection Tool

A Semi-Distributed Electric Demand-Side Management System with PV Generation for Self-Consumption Enhancement

This paper presents the operation of an Electrical Demand-Side Management (EDSM) system in a real solar house. The use of EDSM is one of the most important action lines to improve the grid electrical efficiency. The combination between the EDSM and the PV generation performs a new control level in the local electric behavior and allows new energy possibilities. The solar house used as test-bed for the EDSM system owns a PV generator, a lead-acid battery storage system and a grid connection. The electrical appliances are controllable from an embedded computer. The EDSM is implemented by a control system which schedules the tasks commanded by the user. By using the control system, we define the house energy policy and improve the energy behavior with regard to a selected energy criterion, self-consumption. The EDSM system favors self-consumption with regard to a standard user behavior and reduces the energy load from the grid

Beneficial Effect of Ursodeoxycholic Acid in Patients with ACOX2 Deficiency-Associated Hypertransaminasemia

Background: A variant (p.Arg225Trp) of peroxisomal acyl-CoA oxidase 2 (ACOX2), involved in bile acid (BA) side-chain shortening, has been associated with unexplained persistent hypertransaminasemia and accumulation of C27-BAs, mainly trihydroxycholestanoic acid (THCA). Aims: To investigate the prevalence of ACOX2 deficiency-associated hypertransaminasemia (ADAH), its response to ursodeoxycholic acid (UDCA), elucidate its pathophysiological mechanism and identify other inborn errors that could cause this alteration. Methods & results: Among 33 patients with unexplained hypertransaminasemia from 11 hospitals, and 13 of their relatives, 7 individuals with abnormally high C27-BA levels (>50% of total BAs) were identified by HPLC-MS/MS. The p.Arg225Trp variant was found in homozygosity (exon amplification/sequencing) in 2 patients and 3 family members. Two additional non-related patients were heterozygous carriers of different alleles: c.673C>T (p.Arg225Trp) and c.456_459del (p.Thr154fs). In ADAH patients, impaired liver expression of ACOX2, but not ACOX3, was found (immunohistochemistry). Treatment with UDCA normalized transaminases levels. Incubation of HuH-7 liver cells with THCA, which was efficiently taken up, but not through BA transporters, increased ROS production (flow cytometry), ER stress biomarkers (GRP78, CHOP and XBP1-S/XBP1-U ratio), and BAX¿ expression (RT-qPCR and immunoblot), whereas cell viability was decreased (MTT). THCA-induced cell toxicity was higher than that of major C24-BAs and was not prevented by UDCA. Fourteen predicted ACOX2 variants were generated (site-directed mutagenesis) and expressed in HuH-7 cells. Functional tests to determine their ability to metabolize THCA identified six with the potential to cause ADAH. Conclusion: Dysfunctional ACOX2 has been found in several patients with unexplained hypertransaminasemia. This condition can be accurately identified by a non-invasive diagnostic strategy based on plasma BA profiling and ACOX2 sequencing. Moreover, UDCA treatment can efficiently attenuate liver damage in these patients.This study was supported by the following grants: CIBERehd (EHD15PI05/2016); Fondo de Investigaciones Sanitarias, Instituto de Salud Carlos III, Spain (PI19/00819 and PI20/00189), co-funded by European Regional Development Fund/European Social Fund, “Investing in your future”; “Junta de Castilla y León” (SA074P20); Fundació Marato TV3 (201916–31); AECC Scientific Foundation (2017/2020), Spain; and “Centro Internacional sobre el Envejecimiento” (OLD-HEPAMARKER, 0348_CIE_6_E), Spain. We also acknowledge support from grants PID2019-111669RBI- 100, PID2020-115055RB- I00 from Plan Nacional de I+D funded by the “Agencia Estatal de Investigación” (AEI) and the center grant P50AA011999 Southern California Research Center for ALPD and Cirrhosis funded by NIAAA/NIH, as well as support from AGAUR of the “Generalitat de Catalunya” SGR-2017- 1112, European Cooperation in Science & Technology (COST) ACTION CA17112 Prospective European Drug-Induced Liver Injury Network. Marta Alonso-Peña was the recipient of a predoctoral fellowship from “Ministerio de Educación, Cultura y Deporte” (BOE-A- 2015- 9456; FPU-14/ 00214) and a Mobility Grant for Short Stays from “Ministerio de Ciencia, Innovación y Universidades” (EST17/00186). Ricardo Espinosa-Escudero is the recipient of a predoctoral fellowship from “Junta de Castilla y León” and “Fondo Social Europeo” (EDU/574/2018). The funding sources were not involved in the research design or preparation of the articl

Beneficial effect of ursodeoxycholic acid in patients with acyl-CoA oxidase 2 (ACOX2) deficiency-associated hypertransaminasemia

Background and aims: A variant (p.Arg225Trp) of peroxisomal acyl-CoA oxidase 2 (ACOX2), involved in bile acid (BA) side-chain shortening, has been associated with unexplained persistent hypertransaminasemia and accumulation of C27-BAs, mainly 3?,7?,12?-trihydroxy-5?-cholestanoic acid (THCA). We aimed to investigate the prevalence of ACOX2 deficiency-associated hypertransaminasemia (ADAH), its response to ursodeoxycholic acid (UDCA), elucidate its pathophysiological mechanism and identify other inborn errors that could cause this alteration. Methods and results: Among 33 patients with unexplained hypertransaminasemia from 11 hospitals and 13 of their relatives, seven individuals with abnormally high C27-BA levels (>50% of total BAs) were identified by high-performance liquid chromatography-mass spectrometry. The p.Arg225Trp variant was found in homozygosity (exon amplification/sequencing) in two patients and three family members. Two additional nonrelated patients were heterozygous carriers of different alleles: c.673C>T (p.Arg225Trp) and c.456_459del (p.Thr154fs). In patients with ADAH, impaired liver expression of ACOX2, but not ACOX3, was found (immunohistochemistry). Treatment with UDCA normalized aminotransferase levels. Incubation of HuH-7 hepatoma cells with THCA, which was efficiently taken up, but not through BA transporters, increased reactive oxygen species production (flow cytometry), endoplasmic reticulum stress biomarkers (GRP78, CHOP, and XBP1-S/XBP1-U ratio), and BAX? expression (reverse transcription followed by quantitative polymerase chain reaction and immunoblot), whereas cell viability was decreased (tetrazolium salt-based cell viability test). THCA-induced cell toxicity was higher than that of major C24-BAs and was not prevented by UDCA. Fourteen predicted ACOX2 variants were generated (site-directed mutagenesis) and expressed in HuH-7 cells. Functional tests to determine their ability to metabolize THCA identified six with the potential to cause ADAH. Conclusions: Dysfunctional ACOX2 has been found in several patients with unexplained hypertransaminasemia. This condition can be accurately identified by a noninvasive diagnostic strategy based on plasma BA profiling and ACOX2 sequencing. Moreover, UDCA treatment can efficiently attenuate liver damage in these patients.Funding information: This study was supported by the following grants: CIBERehd (EHD15PI05/2016); Fondo de Investigaciones Sanitarias, Instituto de Salud Carlos III, Spain (PI19/00819 and PI20/00189), co-funded by European Regional Development Fund/European Social Fund, “Investing in your future”; “Junta de Castilla y León” (SA074P20); Fundació Marato TV3 (201916–31); AECC Scientific Foundation (2017/2020), Spain; and “Centro Internacional sobre el Envejecimiento” (OLD-HEPAMARKER, 0348_CIE_6_E), Spain. We also acknowledge support from grants PID2019-111669RBI-100, PID2020-115055RB-I00 from Plan Nacional de I+D funded by the “Agencia Estatal de Investigación” (AEI) and the center grant P50AA011999 Southern California Research Center for ALPD and Cirrhosis funded by NIAAA/NIH, as well as support from AGAUR of the “Generalitat de Catalunya” SGR-2017-1112, European Cooperation in Science & Technology (COST) ACTION CA17112 Prospective European Drug-Induced Liver Injury Network. Marta Alonso-Peña was the recipient of a predoctoral fellowship from “Ministerio de Educación, Cultura y Deporte” (BOE-A-2015-9456; FPU-14/00214) and a Mobility Grant for Short Stays from “Ministerio de Ciencia, Innovación y Universidades” (EST17/00186). Ricardo Espinosa-Escudero is the recipient of a predoctoral fellowship from “Junta de Castilla y León” and “Fondo Social Europeo” (EDU/574/2018). The funding sources were not involved in the research design or preparation of the article