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Friedreich ataxia (FRDA) is a multisystemic, autosomal recessive disorder caused by homozygous GAA expansion mutation in the first intron of frataxin (FXN) gene. FXN is a mitochondrial protein critical for iron-sulfur cluster biosynthesis and deficiency impairs mitochondrial electron transport chain functions and iron homeostasis within the organelle. Currently, there is no effective treatment for FRDA. We have previously demonstrated that single infusion of wild-type hematopoietic stem and progenitor cells (HSPCs) resulted in prevention of neurologic and cardiac complications of FRDA in YG8R mice, and rescue was mediated by FXN transfer from tissue engrafted, HSPC-derived microglia/macrophages to diseased neurons/myocytes. For a future clinical translation, we developed an autologous stem cell transplantation approach using CRISPR/Cas9 for the excision of the GAA repeats in FRDA patients CD34+ HSPCs; this strategy leading to increased FXN expression and improved mitochondrial functions. The aim of the current study is to validate the efficiency and safety of our gene editing approach in a disease-relevant model. We generated a cohort of FRDA patient-derived iPSCs and isogenic lines that were gene edited with our CRISPR/Cas9 approach. iPSC derived FRDA neurons displayed characteristic apoptotic and mitochondrial phenotype of the disease, such as non-homogenous microtubule staining in neurites, increased caspase-3 expression, mitochondrial superoxide levels, mitochondrial fragmentation, and partial degradation of the cristae compared to healthy controls. These defects were fully prevented in the gene edited neurons. RNASeq analysis of FRDA and gene edited neurons demonstrated striking improvement in gene clusters associated with endoplasmic reticulum (ER) stress in the isogenic lines. Gene edited neurons demonstrated improved ER-calcium release, normalization of ER stress response gene, XBP-1, and significantly increased ER-mitochondrial contacts that are critical fo

La investigación se centra en la posible influencia que han tenido los artistas de Land Art Christo y Jeanne-Claude en los desfiles de moda de Jacquemus. Para ello se lleva a cabo un análisis comparativo de sus obras, y se plantea el interrogante de si la estética de Jacquemus se forma a partir de la inspiración de la obra de Christo y Jeanne-Claude. Para comenzar se establece un contexto histórico, en el que se exploran diferentes casos en los que el mundo del arte y el mundo de la moda han llegado a confluir a lo largo de la historia. La obra de Christo y Jeanne-Claude se desglosa en cuatro escalas de trabajo diferentes, desde la escala del objeto hasta la escala del paisaje, que posteriormente van a servir para mirar la obra de Jacquemus bajo esos mismos criterios. La investigación concluye reflexionando sobre la posible influencia de los artistas de Land Art en la estética de Jacquemus, y abre puertas a posibles análisis e investigaciones más profundos sobre su obra y su impacto en la moda.

Fullerene derivatives have been used as electron acceptor and transport materials in organic photovoltaics as well as in perovskite solar cells. Among them, [6,6]-phenyl-C61-butyric acid methyl ester (PC61BM) has been one of the most widely used, in combination with poly-3-hexylthiophene (P3HT) as an electron donor semiconducting polymer, for the fabrication of organic bulk heterojunction solar cells (BHJ-OSC). In this work, a steroid-fullerene hybrid was synthesized through the Bingel–Hirsch reaction to compare its properties with PC61BM as the reference structure. Different spectroscopic techniques were employed to characterize the new fullerene derivative, namely 1D and 2D nuclear magnetic resonance (NMR), Fourier Transform Infrared Spectroscopy (FTIR), and high-resolution mass spectrometry (HRMS). In addition, thermogravimetric analysis (TGA), and cyclic voltammetry were also performed. The characterization was completed by transmission electron microscopy (TEM), which explore the hybrid‘s morphology, where the presence of the steroid moiety increases the solubility of the fullerene material. DFT/PBE−D3(BJ)/6-311G(d,p) calculations for both the steroid-fullerene hybrid and PC61BM allowed to obtain geometry optimizations and electronic structure data. A comparative study to analyze the nature of the electronic properties of the synthesized steroid-[60]fullerene hybrid and PC61BM with an oligomer of ten thiophene units, used in the active layer of organic solar cells, was carried out. Electron density maps using the [CIS|CNDOL/21] approach illustrate the photoexcitation of the donor followed by an electron-charge transfer to the acceptor fullerene., Ministerio de Ciencia e Innovación (España), Depto. de Química Orgánica, Fac. de Ciencias Químicas, TRUE, pub, Descuento UCM

Omidubicel is an umbilical cord blood (UCB)-derived ex vivo-expanded cellular therapy product that has demonstrated faster engraftment and fewer infections compared with unmanipulated UCB in allogeneic hematopoietic cell transplantation. Although the early benefits of omidubicel have been established, long-term outcomes remain unknown. We report on a planned pooled analysis of 5 multicenter clinical trials including 105 patients with hematologic malignancies or sickle cell hemoglobinopathy who underwent omidubicel transplantation at 26 academic transplantation centers worldwide. With a median follow-up of 22 months (range, .3 to 122 months), the 3-year estimated overall survival and disease-free survival were 62.5% and 54.0%, respectively. With up to 10 years of follow-up, omidubicel showed durable trilineage hematopoiesis. Serial quantitative assessments of CD3+, CD4+, CD8+, CD19+, CD116+CD56+, and CD123+ immune subsets revealed median counts remaining within normal ranges through up to 8 years of follow-up. Secondary graft failure occurred in 5 patients (5%) in the first year, with no late cases reported. One case of donor-derived myeloid neoplasm was reported at 40 months post-transplantation. This was also observed in a control arm patient who received only unmanipulated UCB. Overall, omidubicel demonstrated stable trilineage hematopoiesis, immune competence, and graft durability in extended follow-up.

Rentals United és una empresa de gestió de canals, de manera que ofereix solucions de programari per a propietaris i gestors de propietats enfocades al seu lloguer per vacances. Ja que opera en un sector molt volàtil, és una prioritat per a l'empresa mantenir els clients existents i aprofitar les noves oportunitats que sorgeixen. L'objectiu principal d'aquest treball és partir de l'actual sistema de Rentals United per a la retenció de clients i construir un nou conjunt de processos de transformació de les dades. El seu origen radica en els sistemes d'emmagatzematge de dades de l'empresa i, mitjançant mètodes i algoritmes basats en dades, dóna lloc a una taula final de dades on cada client hi és representat. D'una banda, en aquesta taula es poden trobar indicacions per tal de prevenir la pèrdua de clients, per mitjà d'accions construïdes a partir d'arbres de decisió. D'altra banda, hi ha oportunitats de creixement personalitzades i quantificades per tal que els clients puguin ampliar el seu catàleg de productes actual (augmentant així els ingressos de l'empresa). Les mètriques es divideixen en diferents àrees i es calculen cada dia, en qüestió de minuts. Al mateix temps, es reforcen processos paral·lels, destinats a incrementar la fiabilitat global de les dades i a trobar possibles inconsistències. Aquest treball permet l'extracció d'una llista de clients amb un risc de pèrdua estimat elevat. A més, s'obtenen estadístiques individuals i agregades, les quals donen lloc a diferents informes gràfics que els membres de Rentals United ja estan utilitzant a diari per a entendre el comportament dels clients. S'avaluen diferents sistemes de processament del llenguatge per a aprofundir en la integració dels resultats del projecte en l'operació habitual de l'empresa. De fet, es millora un bot de conversa existent per a incorporar les propostes del projecte. A més, es plantegen i defineixen les bases d'un model supervisat que faci servir aquestes dades, pendent de ser implement, Rentals United is a channel management company that offers software solutions for property managers and owners to handle their vacation rental properties. It operates in a very volatile sector; thereby it is a priority for the company to maintain existing clients and to leverage new opportunities. The main objective of this thesis is to depart from Rentals United's current approaches to customer retention in order to build a solid new pipeline. It originates in the company's data warehouses and, through data-driven methods and algorithms, gives rise to a final data table where each client is represented. On the one hand, guidance to prevent churns may be found in this table by means of a set of decision tree - based actionable insights. On the other hand, there are customized and quantified growth opportunities for customers to expand their current portfolio (increasing the company's revenue). Metrics are segmented into different areas and re-computed every day, in a matter of minutes. At the same time, parallel data processes are reinforced to boost data reliability and find inconsistences. This work allows the extraction of a shortlist of clients with estimated high churn risk at present. Moreover, user-by-user and aggregated statistics are obtained and feed reports that Rentals United members are already using to understand clients' behaviour. Different approaches to language processing are tested to further integrate the results into the company's operation flow. In fact, an existing chat bot is strengthened to incorporate the insights of the project. Furthermore, the groundworks for a supervised model to exploit the new data are laid and well-defined to be implemented in the future. In summary, the company's knowledge about the customers' operation is widened and made more usable.

This project presents a Linear Parameter Varying (LPV) Linear Quadratic Regulator (LQR) approach for the control of semi-active dampers. A LPV LQR controller is designed and implemented to a simple quarter car vehicle model and to a more complex vehicle model defined in Vi-CarRealTime (Vi-CRT) software. A methodology for the evaluation of comfort and roadholding performance is presented, and vehicle model performance with semi-active damper control is compared to that achievable with a passive system. To conduct the study, a semi-active damper model is defined based on the Guo formulation, together with a vehicle model, providing a realistic case study of a sedan car equipped with semi-active (SA) dampers. Results obtained from the quarter car plant model underscore the active system’s capacity to improve the achievable passive roadholding and comfort performance. It has also proven to be a versatile solution, as different results can be obtained by modifying the controller tuning parameters. Vi-CarRealTime plant model results show certain improvement areas. Semi-active damper system can notably improve the passive roadholding achievable performance but cannot achieve better comfort levels. Suggestions to improve the controller performance with the Vi-CRT model plant are provided for future research, Aquest projecte presenta un enfocament de Linear Quadratic Regulator (LQR) Linear Parameter Varying (LPV) per al control d'amortidors semiactius. Un controlador LPV LQR és dissenyat i implementat per a un model simple de tipus ‘quart de vehicle' i per a un model de vehicle més complex definit en el programari Vi-CarRealTime (Vi-CRT). Es presenta una metodologia per a l'avaluació del rendiment en comoditat i adherència a la carretera, i es compara el rendiment del model de vehicle amb control d'amortidors semiactius amb el que es pot aconseguir amb un sistema passiu. Per dur a terme l'estudi, es defineix un model d'amortidor semiactiu basat en la formulació de Guo juntament amb un model de vehicle, proporcionant un cas d'estudi realista d'un cotxe del tipus Sedan equipat amb amortidors semiactius (SA). Els resultats obtinguts amb el model de 'quart de vehicle' com a planta subratllen la capacitat del sistema actiu per millorar el rendiment passiu que es pot aconseguir en adherència i comoditat. També ha demostrat ser una solució versàtil, ja que es poden aconseguir diferents resultats modificant els paràmetres del controlador. Els resultats obtinguts amb el model de planta de Vi-CarRealTime mostren certes àrees de millora. El sistema d'amortidors semiactius és capaç de millorar notablement el rendiment en adherència, però no pot aconseguir nivells de comoditat superiors als del sistema passiu. Es proporcionen suggeriments per millorar el rendiment del controlador en futures investigacions, Este proyecto presenta un enfoque de Linear Quadratic Regulator (LQR) Linear Parameter Varying (LPV) para el control de amortiguadores semiactivos. Se diseña e implementa un controlador LPV LQR para un modelo de tipo ‘cuarto de vehículo’ más simple y para un modelo de vehículo más complejo definido en el software Vi- CarRealTime (Vi-CRT). Se presenta una metodología para la evaluación del rendimiento en comfort y adherencia, y se compara el rendimiento del modelo de vehículo con control de amortiguadores semiactivos con lo alcanzable mediante un sistema pasivo. Para llevar a cabo el estudio, se define un modelo de amortiguador semiactivo basado en la formulación de Guo, junto con un modelo de vehículo, proporcionando un caso de estudio realista de un sedán equipado con amortiguadores semiactivos (SA). Los resultados obtenidos del modelo de cuarto de vehículo subrayan la capacidad del sistema activo para mejorar el rendimiento pasivo alcanzable en adherencia y comodidad. También ha demostrado ser una solución versátil, ya que se pueden lograr diferentes comportamientos mediante la modificación de los parámetros de ajuste del controlador. Los resultados con la planta de Vi-CarRealTime muestran ciertas áreas de mejora. El sistema de amortiguación semiactivo puede mejorar notablemente el rendimiento alcanzable en adherencia, pero no logra niveles de comodidad superiores. Así pues, se proporcionan sugerencias para mejorar el rendimiento del controlador con la planta de Vi- CRT en futuras investigaciones

La arquitectura y la moda van de la mano en distintos aspectos, pero se encuentran especialmente en nuestras calles a través de la arquitectura comercial y el escaparatismo. Por ello, en este trabajo se profundiza en las diferentes maneras en las que la moda y las marcas, especialmente en el caso de Loewe, hacen uso de la arquitectura para representar unos valores e imagen de la marca. Se realiza un acercamiento a través del estudio de las relaciones entre la arquitectura y la imagen y, a su vez entre la arquitectura y la moda, para después realizar una cronología de Loewe relacionando la primera con los distintos factores encontrados en el estudio previo. Esto y las diversas visitas y diálogos en el archivo histórico de la firma, permiten la elaboración de análisis gráficos que conforman gran parte del trabajo. Para llevarlos a cabo, se identifican distintas capas y elementos que permiten a la arquitectura desarrollar esa imagen: desde el análisis de la situación estratégica en la ciudad hasta la disposición de elementos expositivos. Por último una comparativa entre los casos elegidos para concluir y entender de manera global la evolución de las tiendas a lo largo de la historia.

El interés por la moda hoy en día es incuestionable. Desde el punto de vista económico, en España cubre casi un 3% del PIB y a nivel mundial es considerada la segunda industria con mayor impacto en nuestro medioambiente. Desde el punto de vista cultural, constituye un medio de expresión incomparable, y psicólogos, sociólogos, historiadores o teóricos del arte, se basan en la moda para conocer y analizar nuestras costumbres y orden social. Hoy, la moda es un fenómeno extremadamente complejo, a nivel industrial es un sector global, interdisciplinar e internacional. A nivel creativo, hace tiempo que los modistos han dejado paso a los directores creativos, la comunicación ha ocupado el lugar de la confección, y en esa construcción de aspiraciones, la arquitectura juega un papel decisivo para las grandes marcas. Este trabajo explora la idea de que la arquitectura es un recurso fundamental para el desarrollo de la estrategia corporativa y transmisión de la filosofía de las firmas de moda. Investiga las relaciones interdisciplinares que existen dentro del triángulo que conforman la moda, la marca y la arquitectura, en la firma Balenciaga. El análisis se estructura en dos fases. La primera recoge información del proceso de ideación, diseño y desarrollo evolutivo de la casa Balenciaga. La segunda parte de esta investigación aborda en mayor profundidad, tres momentos significantes de la dirección creativa de la firma, que posteriormente se compararán entre sí, de forma que se obtendrá como resultado tres situaciones, provenientes de momentos espaciales distintos, en las que se presenta la arquitectura como elemento fundamental, con el mismo peso y en distintas escalas, a la hora del desarrollo de la imagen de Balenciaga. La metodología utilizada en este estudio, se apoya en un formato gráfico visual que permite la expresión de las conclusiones de una forma clara y dinámica.

Purpose: The risk of secondary zoonotic transmission of SARS-CoV-2 from pet animals remains unclear. Here, we report on a 44 year old Caucasian male presenting to our clinic with COVID-19 pneumonia, who reported that his dog displayed respiratory signs shortly prior to his infection. The dog tested real-time-PCR (RT-PCR) positive for SARS-CoV-2 RNA and the timeline of events suggested a transmission from the dog to the patient. Methods: RT-PCR and serological assays were used to confirm SARS-CoV-2 infection in the nasopharyngeal tract in the dog and the patient. We performed SARS-CoV-2-targeted amplicon-based next generation sequencing of respiratory samples from the dog and patient for sequence comparisons. Results: SARS-CoV-2 infection of the dog was confirmed by three independent PCR-positive pharyngeal swabs and subsequent seroconversion. Sequence analysis identified two separate SARS-CoV-2 lineages in the canine and the patient’s respiratory samples. The timeline strongly suggested dog-to-human transmission, yet due to the genetic distance of the canine and the patient’s samples paired-transmission was highly unlikely. Conclusion: The results of this case support current knowledge about the low risk of secondary zoonotic dog-to-human transmissions of SARS-CoV-2 and emphasizes the strength of genomic sequencing in deciphering viral transmission chains.

Con el objetivo principal de vender ropa y promover la industria de la moda, los desfiles se van sintonizando más con el espectáculo y la creación de escenarios extraordinarios. Son espectáculos que buscan formas seductoras de presentar la moda y sorprender al público. Ya sea en el teatro, la ópera, los conciertos o en las instalaciones artísticas, la luz cobra un gran protagonismo en la puesta en escena. Y en las pasarelas de moda, como en todo espectáculo, la iluminación puede llegar a ser el elemento principal entorno al cual se articula todo. El estudio de la obra de algunos artistas que trabajan con este recurso, nos hará entender la importancia que puede llegar a adquirir antes de centrarnos en el estudio de Stefan Beckman, un escenógrafo especializado en moda. Stefan Beckman es uno de los profesionales que todo buen desfile necesita. Estos incluyen desde diseñadores de moda, modelos y equipo técnico a diseñadores de producción y, en los últimos tiempos, arquitectos. Beckman lleva años organizando algunos de los desfiles más icónicos de la semana de la moda de Nueva York. A través su trabajo, crea entornos diseñados para provocar una reacción en el espectador y esto lo consigue con la ayuda de algunos elementos organizadores pero, principalmente, con la iluminación. Para analizar su extenso trabajo nos vamos a centrar en el análisis de tres escenografías de pasarelas con características lumínicas y organizaciones espaciales diferentes, como son: Marc Jacobs Otoño/Invierno (o/i) 2013, Alexander Wang Primavera/Verano (p/v) 2016 y Marc Jacobs Primavera/Verano (p/v) 2017. El análisis se llevará a cabo desde varios aspectos: organización espacial; público, recorrido y luces y la organización temporal de la iluminación en relación con el resto de los elementos.

Vivimos en una época donde la necesidad de delinear horizontes sobre los sucesos futuros es cada vez más necesaria y posible. Este escrito abordará algunos caminos que pueden articular y retroalimentar las relaciones entre arte, diseño y educación para proponer posibles cruces y anticipar escenarios, explorando los cambios interdisciplinarios que se observan hoy en la aldea global. El planteo analizará cómo sería posible construir modelos dinámicos, discutirá acerca de las nociones de hiperrealidad y singularidad y el aporte que estas pueden dar al desarrollo de la inteligencia y el conocimiento humano, explorando sus posibilidades innovadoras y el impacto que podrían tener sobre la educación., This article will explore the singularity notion and how to improve the human education, will analyse the leaderships limitations to resolve exponential issues. Explores how the human singularity, open an interactive and interdisciplinary new hole on the cyberspace. It will discuss also about the poetics to explore a new frontier of the real world through singularity, the relationships between art, design and education, considering the innovative possibilities in many professional areas., Vivemos um tempo em que a necessidade de traçar horizontes sobre eventos futuros é cada vez mais necessária e possível. Esta escrita abordará alguns caminhos que podem articular e retroalimentar as relações entre arte, design e educação para propor possíveis intersecções e antecipar cenários, explorando as mudanças interdisciplinares que se observam hoje na aldeia global. A proposta analisará como seria possível construir mo-delos dinâmicos, discutirá as noções de hiper-realidade e singularidade e a contribuição que estas podem dar ao desenvolvimento da inteligência e do conhecimento humano, explorando suas possibilidades inovadoras e o impacto que podem ter na educação.

Introducció Els pacients pediàtrics sotmesos a un trasplantament de progenitors hematopoètics (TPH) al·logènic que precisen ingrés en la Unitat de Cures Intensives Pediàtriques (UCIP) tenen una alta mortalitat. L'objectiu general d'aquest estudi és descriure i analitzar factors de risc a l'ingrés a la UCIP dels pacients sotmesos a un TPH i investigar quins s'associen a pitjor pronòstic. Com a objectius secundaris es determinen les causes d'ingrés, s'estudien les característiques clíniques i l'evolució dels pacients ingressats i s'investiguen quins paràmetres de l'evolució associen a pitjor pronòstic. Material i mètodes S'avaluen dades referents a la filiació del pacient, al trasplantament de progenitors hematopoètics, dades de l'ingrés a la UCIP i dades de l'estat del pacient durant l'evolució i l'alta de la UCIP. Entre Gener del 2008 i Desembre 2017 es van realitzar a l'Hospital de Sant Pau 132 trasplantaments al·logènics de progenitors hematopoètics en 112 pacients dels que han precisat ingrés a la UCIP 54 (40,9%). Els 54 pacients que van precisar ingrés a la UCIP van ser sotmesos a 68 trasplantaments. Resultats La supervivència actuarial global és del 41,7% a 11 anys. El 40,9% dels pacients sotmesos a un TPH han precisat ingrés a UCIP. La principal causa d'ingrés a la UCIP és la insuficiència respiratòria (41,1%), seguit de la patologia neurològica (22,7%), el trastorn hemodinàmic (21,2%), la insuficiència renal (6,0%) i la fallida hepàtica (6,0%). La causa d'ingrés està associada a un procés infecciós en el 71,2% dels ingressos. En l'anàlisi univariant, els pacients tenen a l'ingrés a la UCIP un augment significatiu del pes (p=0,007), de la xifra de creatinina (p=0,007), de la bilirubina (p< 0,001) i del valor de l'INR (p< 0,001) i tenen un descens significatiu en el filtrat glomerular segons la fórmula de Schwartz (p< 0,001). Les característiques associades amb menor supervivència són la puntuació O-PRISM (p=0,018), pSOFA (p=0,018), PELOD (p=0,010) i PIM-3 (p=, Introducción Los pacientes pediátricos sometidos a un trasplante de progenitores hematopoyéticos (TPH) alogénico que precisan ingreso en la Unidad de Cuidados Intensivos Pediátricos (UCIP) tienen una alta mortalidad. El objetivo general de este estudio es describir y analizar factores de riesgo al ingreso en la UCIP de los pacientes sometidos a un TPH e investigar cuáles se asocian a peor pronóstico. Como objetivos secundarios se determinan las causas de ingreso, se estudian las características clínicas y la evolución de los pacientes ingresados, y se investigan qué parámetros de la evolución asocian un peor pronóstico. Material y métodos Se evalúan datos referentes a la filiación del paciente, al trasplante de progenitores hematopoyéticos, datos del ingreso en la UCIP y datos del estado del paciente durante la evolución y el alta de la UCIP. Entre Enero de 2008 y Diciembre 2017 se realizaron en el Hospital de Sant Pau 132 trasplantes alogénicos de progenitores hematopoyéticos en 112 pacientes de los que precisaron ingreso en la UCIP 54 (40,9%). Los 54 pacientes que precisaron ingreso en la UCIP fueron sometidos a 68 trasplantes. Resultados La supervivencia actuarial global es del 41,7% a 11 años. El 40,9% de los pacientes sometidos a un TPH precisaron ingreso en UCIP. La principal causa de ingreso es la insuficiencia respiratoria (41,1%), seguido de la patología neurológica (22,7%), el trastorno hemodinámico (21,2%), la insuficiencia renal (6,0% ) y el fallo hepático (6,0%). La causa de ingreso está asociada a un proceso infeccioso en el 71,2% de los ingresos. En el análisis univariante, los pacientes tienen al ingreso en la UCIP un aumento significativo del peso (p=0,007), de la cifra de creatinina (p=0,007), de la bilirrubina (p<0,001) y del valor del INR (p<0,001) y tienen un descenso significativo en el filtrado glomerular según la fórmula de Schwartz (p<0,001). Las características asociadas con menor supervivencia son la puntuación O-PRISM (p=0,018), pSOFA (p=, Introduction Paediatric patients undergoing an allogenic hematopoietic stem cell transplant (HSCT) who require admission to the Paediatric Intensive Care Unit (PICU) have a high mortality rate. The general objective of this study is to describe and analyce risk factors upon admission to the PICU of patients undergoing HSCT and to investigate which ones are associated with a worse prognosis. As secondary objectives, the causes of admission are determined, the clinical characteristics and evolution of admitted patients are studied, and the parameters of the evolution associated with a worse prognosis are investigated. Material and methods Data regarding the patient's affiliation, hematopoietic stem cell transplantation, data on admission to the PICU, and data on the patient's condition during the evolution and discharge from the PICU are evaluated. Between January 2008 and December 2017, 132 allogeneic hematopoietic stem cell transplants were performed at the Hospital de Sant Pau in 112 patients of whom 54 (40.9%) required admission to the intensive care unit. The 54 patients who required admission to PICU underwent 68 transplants. Results Overall survival is 41.7% at 11 years. 40.9% of patients undergoing TPH required admission to the UCIP. The main cause of admission is respiratory failure (41.1%), followed by neurological pathology (22.7%), hemodynamic disorder (21.2%), renal failure (6.0%) and failure liver (6.0%). The cause of admission is associated with an infectious process in 71.2% of admissions. In the univariate analysis, the patients admitted to the PICU had a significant increase in weight (p=0.007), creatinine (p=0.007), bilirubin (p<0.001) and INR value (p<0.001) and have a significant decrease in glomerular filtration according to the Schwartz formula (p<0.001). The characteristics associated with lower survival are O-PRISM score (p=0.018), pSOFA (p=0.018), PELOD (p=0.010) and PIM-3 (p=0.015), as well as, the SatO2/FiO2ratio (p=0.004) and invasive mech, Universitat Autònoma de Barcelona. Programa de Doctorat en Pediatria, Obstetrícia i Ginecologia

A fundamental necessity in mobile robotics is the ability of knowing the robot own location. In many applications, a map of the environment is not available or it does not have the accuracy or the information necessary for robot localization. In this cases, it is necessary to build a map simultaneously (SLAM). In applications where the GPS signal is not reliable, like in indoor environments, the localization has to be based in estimating robot position with the information of the robot movement. This estimation is obtained in local coordinates and it implies that a drift in the estimation accumulates error over time. In order to reduce the drift effects, it is necessary the capacity to recognize places that had already been visited and readjust the localization. This search of loop closures provides global reference points to the localization system. This field of study is already a very discussed topic, but finding an equilibrium between efficiency and robustness in order to be able to work in large environments is still a challenge. The aim of this work is to equip the WOLF SLAM library with an state-of-art loop closure for 2D LIDAR sensors. WOLF SLAM library is integrated with ROS and proposes an interesting environment to manage SLAM and localization problems based in graph-SLAM. In this work, we first performed a review of the state of the art for loop closure that lead us to FALKO. It is an open source project focused in loop closure that implements feature detection and scene matching using descriptors. Furthermore, the FALKO loop closure algorithm has been integrated along with an Iterative Closest Point method for robustness and accuracy purposes. ICP is a point to line metric optimized for range-finder scan matching. The integration and developed algorithms has been tested with experimental data from a real robot

Type I and III leukocyte adhesion deficiencies (LADs) are primary immunodeficiency disorders resulting in early death due to infections and additional bleeding tendency in LAD-III. The curative treatment of LAD-I and LAD-III is allogeneic hematopoietic stem cell transplantation (allo-HSCT). In this retrospective multicenter study, data were collected using the European Society for Blood and Marrow Transplantation registry; we analyzed data from 84 LAD patients from 33 centers, all receiving an allo-HSCT from 2007 to 2017. The 3-year overall survival estimate (95% confidence interval [CI]) was 83% (74-92) for the entire cohort: 84% (75-94) and 75% (50-100) for LAD-I and LAD-III, respectively. We observed cumulative incidences (95% CI) of graft failure (GF) at 3 years of 17% (9%-26%) and grade II to IV acute graft-versus-host disease (aGVHD) at 100 days of 24% (15%-34%). The estimate (95% CI) at 3 years for GF- and GVHD-II to IV-free survival as event-free survival (EFS) was 56% (46-69) for the entire cohort; 58% (46-72) and 56% (23-88) for LAD-I and LAD-III, respectively. Grade II to IV acute GVHD was a relevant risk factor for death (hazard ratio 3.6; 95% CI 1.4-9.1; P 5.006). Patients' age at transplant $13 months, transplantation from a nonsibling donor, and any serological cytomegalovirus mismatch in donor-recipient pairs were significantly associated with severe acute GVHD and inferior EFS. The choice of busulfan- or treosulfan-based conditioning, type of GVHD prophylaxis, and serotherapy did not impact overall survival, EFS, or aGVHD. An intrinsic inflammatory component of LAD may contribute to inflammatory complications during allo-HSCT, thus providing the rationale for considering anti-inflammatory therapy pretreatment.

Inherited bone marrow failure syndromes (IBMFSs) are a group of congenital rare diseases characterized by bone marrow failure, congenital anomalies, high genetic heterogeneity, and predisposition to cancer. Appropriate treatment and cancer surveillance ideally depend on the identification of the mutated gene. A next-generation sequencing (NGS) panel of genes could be 1 initial genetic screening test to be carried out in a comprehensive study of IBMFSs, allowing molecular detection in affected patients. We designed 2 NGS panels of IBMFS genes: version 1 included 129 genes and version 2 involved 145 genes. The cohort included a total of 204 patients with suspected IBMFSs without molecular diagnosis. Capture-based targeted sequencing covered > 99% of the target regions of 145 genes, with more than 20 independent reads. No differences were seen between the 2 versions of the panel. The NGS tool allowed a total of 91 patients to be diagnosed, with an overall molecular diagnostic rate of 44%. Among the 167 patients with classified IBMFSs, 81 patients (48%) were diagnosed. Unclassified IBMFSs involved a total of 37 patients, of whom 9 patients (24%) were diagnosed. The preexisting diagnosis of 6 clinically classified patients (6%) was amended, implying a change of therapy for some of them. Our NGS IBMFS gene panel assay is a useful tool in the molecular diagnosis of IBMFSs and a reasonable option as the first tier genetic test in these disorders.

A fundamental necessity in mobile robotics is the ability of knowing the robot own location. In many applications, a map of the environment is not available or it does not have the accuracy or the information necessary for robot localization. In this cases, it is necessary to build a map simultaneously (SLAM). In applications where the GPS signal is not reliable, like in indoor environments, the localization has to be based in estimating robot position with the information of the robot movement. This estimation is obtained in local coordinates and it implies that a drift in the estimation accumulates error over time. In order to reduce the drift effects, it is necessary the capacity to recognize places that had already been visited and readjust the localization. This search of loop closures provides global reference points to the localization system. This field of study is already a very discussed topic, but finding an equilibrium between efficiency and robustness in order to be able to work in large environments is still a challenge. The aim of this work is to equip the WOLF SLAM library with an state-of-art loop closure for 2D LIDAR sensors. WOLF SLAM library is integrated with ROS and proposes an interesting environment to manage SLAM and localization problems based in graph-SLAM. In this work, we first performed a review of the state of the art for loop closure that lead us to FALKO. It is an open source project focused in loop closure that implements feature detection and scene matching using descriptors. Furthermore, the FALKO loop closure algorithm has been integrated along with an Iterative Closest Point method for robustness and accuracy purposes. ICP is a point to line metric optimized for range-finder scan matching. The integration and developed algorithms has been tested with experimental data from a real robot

BACKGROUND: Corynebacterium diphtheriae, the agent of diphtheria, is a genetically diverse bacterial species. Although antimicrobial resistance has emerged against several drugs including first-line penicillin, the genomic determinants and population dynamics of resistance are largely unknown for this neglected human pathogen. METHODS: Here, we analyzed the associations of antimicrobial susceptibility phenotypes, diphtheria toxin production, and genomic features in C. diphtheriae. We used 247 strains collected over several decades in multiple world regions, including the 163 clinical isolates collected prospectively from 2008 to 2017 in France mainland and overseas territories. RESULTS: Phylogenetic analysis revealed multiple deep-branching sublineages, grouped into a Mitis lineage strongly associated with diphtheria toxin production and a largely toxin gene-negative Gravis lineage with few toxin-producing isolates including the 1990s ex-Soviet Union outbreak strain. The distribution of susceptibility phenotypes allowed proposing ecological cutoffs for most of the 19 agents tested, thereby defining acquired antimicrobial resistance. Penicillin resistance was found in 17.2% of prospective isolates. Seventeen (10.4%) prospective isolates were multidrug-resistant (≥ 3 antimicrobial categories), including four isolates resistant to penicillin and macrolides. Homologous recombination was frequent (r/m = 5), and horizontal gene transfer contributed to the emergence of antimicrobial resistance in multiple sublineages. Genome-wide association mapping uncovered genetic factors of resistance, including an accessory penicillin-binding protein (PBP2m) located in diverse genomic contexts. Gene pbp2m is widespread in other Corynebacterium species, and its expression in C. glutamicum demonstrated its effect against several beta-lactams. A novel 73-kb C. diphtheriae multiresistance plasmid was discovered. CONCLUSIONS: This work uncovers the dynamics of antimicrobial resistance in C

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative procedure in patients with Shwachman–Diamond syndrome (SDS) with bone marrow abnormalities. The results of 74 patients with SDS (6 acute myeloid leukemia, 7 myelodysplastic syndrome, and 61 bone marrow failure) treated with HSCT between 1988 and 2016 are reported. The donor source was: 24% sibling, 8% parent, and 68% unrelated donor. The stem cell source was: 70% bone marrow, 19% peripheral blood stem cells, and 11% cord blood. The conditioning regimen was myeloablative in 54% and reduced intensity in 46%. Neutrophil engraftment was achieved in 84% of patients after a median time of 17.5 days. Graft failure occurred in 15% of HSCTs. Grades I–IV acute and chronic GVHD were observed in 55% and 20% of patients, respectively. After a median follow-up of 7.3 years (95% CI 4.8–10.2), 28 patients died for progression/relapse (7) or toxicity (21). The 5-year overall survival and nonrelapse mortality were 63.3% (95% CI 50.8–73.4) and 19.8% (95% CI 10.8–30.8), respectively. In conclusion, this is the largest series so far reported and confirms that HSCT is a suitable option for patients with SDS. Further efforts are needed to lower transplant-related toxicity and reduce graft failure.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

This article will explore the cyberspace and how to enhance the human imagination, investigating the problem of the virtual and immersive environments and how these contribute to increasing a perceptual level of the student’s in the classroom. Will analyze the limitations of the teaching strategies into the conventional classroom and reflect on the treatment of the spatial notion, contributing to develop the vision of digital ecosystem, analyzing how open an interactive and interdisciplinary window from cyberspace to the aulic environment to expand it, considering the way we access the virtual knowledge, to open ourselves to a universe of creative and innovative possibilities., Em tempos em que a dissolução das fronteiras entre o que foi chamado de “realidade” e a influência da virtualidade em sua construção, este trabalho explorará as delimitações, as áreas intangíveis que, do ciberespaço, aprimoram o desenvolvimento da Imaginação humana, abordando o problema de como os ambientes virtuais e imersivos podem contribuir em um nível perceptivo para o desenvolvimento dos alunos em um ambiente convencional. A abordagem analisará as limitações dos métodos de ensino em sala de aula e refletirá sobre o tratamento da noção espacial, contribuindo para desenvolver a visão do ecossistema digital, que venho investigando há algum tempo, tentando abrir uma janela interativa e interdisciplinar, que constrói uma viagem de ida e volta do ciberespaço ao ambiente aulico, para expandir a maneira como acessamos o conhecimento e nos abrimos para um universo de possibilidades criativas e inovadoras., En tiempos en donde la disolución de los límites entre aquello que se ha denominado “realidad” y la influencia de la virtualidad en la construcción de la misma, este trabajo explorara las delimitaciones, las zonas intangibles que desde el ciberespacio permiten potenciar el desarrollo de la imaginación humana, abordando la problemática acerca de cómo los entornos virtuales e inmersivos pueden contribuir a nivel perceptivo al desarrollo de los alumnos dentro de un ámbito convencional. El planteo analizará las limitaciones de las formas de enseñanza dentro de un aula y reflexionara acerca del tratamiento de la noción espacial, contribuyendo a desarrollar la visión de ecosistema digital, que he venido investigando hace tiempo, intentando abrir una ventana interactiva e interdisciplinaria, que construya una avenida de ida y vuelta desde el ciberespacio hacia el entorno áulico, para expandir la forma en que accedemos al conocimiento y abrirnos a un universo de posibilidades creativas e innovadoras.

[EN] Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available., [ES] Las inmunodeficiencias primarias (IDP) son unas enfermedades raras, frecuentemente infradiagnosticadas y potencialmente fatales. Las manifestaciones clínicas de las IDP pueden ser muy graves y ocasionar secuelas que empeoran la calidad de vida de los pacientes. Tradicionalmente, el tratamiento de las IDP ha sido fundamentalmente de soporte, con excepción del trasplante de progenitores hematopoyéticos y, más recientemente, la terapia génica. El descubrimiento de nuevos mecanismos patogénicos, el desarrollo de nuevas moléculas y fármacos biológicos y los avances en el conocimiento de las bases moleculares de estas enfermedades han abierto oportunidades para el tratamiento de esta afección. El objetivo de este documento es revisar el conocimiento actual y aportar recomendaciones para el diagnóstico y el tratamiento clínico de los pacientes adultos y pediátricos con IDP basado en la evidencia científica disponible y teniendo en cuenta la actual práctica y los retos futuros. Se realizó una revisión sistemática, que justifica los niveles de evidencia para cada recomendación.

Aquest treball tracta sobre el disseny cinemàtic i dinàmic d’una suspensió davantera de tipus independent de dobles triangles encarada al monoplaça de l’equip ETSEIB Motorsport de la categoria Formula Student. La memòria es pot dividir en dues parts. La primera defineix un marc teòric sobre la cinemàtica i dinàmica d’aquest tipus de suspensions. Respecte a la cinemàtica, s’expliquen els conceptes necessaris per a realitzar una anàlisi de la suspensió a partir de les vistes frontal i lateral d’aquesta. En la part dinàmica, es defineix la teoria que permet definir els elements dinàmics de la suspensió i s’exposen una sèrie d’eines que són útils de cara al seu disseny. La segona part del document exposa el procés seguit per al disseny de la suspensió. Pel que fa a la part cinemàtica del disseny, es comprova la precisió de les equacions definides en la primera part de la memòria i es defineix un procediment per tal d’escollir els punts de la suspensió. Aquest, es basa en el model del pneumàtic escollit per al vehicle, que és de perfil més baix del que s’ha estat utilitzant fins ara en els vehicles de l’equip. Finalment, es defineixen uns punts de suspensió que compleixen amb els objectius establerts. En la part dinàmica del disseny, es defineixen els components dinàmics de la suspensió a partir dels conceptes teòrics exposats en la primera part.

La malaltia de Parkinson (MP) és un trastorn neurodegeneratiu crònic que es caracteritza per una pèrdua progressiva de les neurones dopaminèrgiques de la substància nigra pars compacta (SNpc). Tot i que s'han dut a terme diverses estratègies per intentar aturar la progressió de la MP, cap d'elles ha demostrat ser de suficient eficàcia. El possible paper del factor de transcripció EB (TFEB) com a diana terapèutica en la MP va guanyar importància quan es va descobrir que el TFEB controla la biogènesi lisosomal i l'autofàgia, i que la seva activació podria contrarestar el defecte lisosomal i l'agregació de proteïnes, dos fenòmens descrits des de fa temps que estan implicats en les malalties neurodegeneratives, incloent la MP. Malgrat això, la majoria de les presumptes dianes del TFEB que s'han descrit fins a dia d'avui estan implicades en diversos processos biològics que no estan relacionats amb el sistema autofàgic-lisosomal. Per tant, aprofundir el nostre coneixement sobre la funció del TFEB en neurones podria ser crucial per desenvolupar una bona estratègia terapèutica per la MP i altres trastorns neurològics. En aquesta tesi, s'ha determinat l'efecte de sobreexpressar el TFEB per mitjà d'un vector viral adeno-associat en les neurones dopaminèrgiques de la substància nigra de ratolí i s'han estudiat els processos moleculars activats en condicions de sobreexpressió de TFEB que podrien oferir beneficis importants en el context de la MP. En aquest sentit, s'ha demostrat que la sobreexpressió de TFEB induïa un efecte neurotròfic, el qual no estava descrit, i que alhora anava acompanyat d'una millora en la funcionalitat dopaminèrgica, de l'activació de vies de senyalització implicades en supervivència i de canvis mitochondrials que, tots plegats, podrien contribuir en fer més resistents a les neurones front la mort cel·lular. Per explorar en més profunditat aquest tema, es va estudiar el potencial terapèutic del TFEB en un context parkinsonià, l'induït per la neurotoxina, Parkinson's disease (PD) is a chronic neurodegenerative disorder mainly characterized by progressive loss of dopaminergic neurons in the substantia nigra pars compacta (SNpc). Albeit diverse efforts have been carried out to halt the progression of PD, none of them have been fully satisfactory. The possible implication of transcription factor EB (TFEB) as a therapeutic target in PD has gained momentum since it was discovered that TFEB controls lysosomal biogenesis and autophagy and that its activation might counteract lysosomal impairment and protein aggregation, which have long been described in neurodegenerative diseases, including PD. However, the majority of putative direct targets of TFEB described to date is linked to a range of biological processes that are not related to the autophagy-lysosomal system. Therefore, to deepen our knowledge on TFEB function in neurons may be crucial to develop a potential therapeutic strategy for PD and other related neurological disorders. In this thesis, we assessed the effect of overexpressing TFEB by means of an adeno-associated viral vector in mouse substantia nigra dopaminergic neurons and studied several molecular processes activated upon TFEB overexpression that may offer potential benefits in the context of PD. In this line, we demonstrated that TFEB overexpression drove a previously unknown bona fide neurotrophic effect accompanied by an enhanced dopaminergic function, activation of pro-survival signaling pathways and mitochondrial changes that altogether may contribute to render neurons less prone to cell death. To delve further into this concept, we studied the therapeutic potential of TFEB in a parkinsonian context, that induced by the neurotoxin MPTP. In this regard, we showed that TFEB overexpression was indeed able to block MPTP-induced neurodegeneration both at the cell body level as well as striatal dopaminergic terminals and restored neuronal activity/function and phenotype in the MPTP mouse model of PD. Moreov

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[Background]: Telomeres are nucleoprotein structures present at the terminal region of the chromosomes. Mutations in genes coding for proteins involved in telomere maintenance are causative of a number of disorders known as telomeropathies. The genetic origin of these diseases is heterogeneous and has not been determined for a significant proportion of patients., [Methods]: This article describes the genetic characterization of a cohort of patients. Telomere length was determined by Southern blot and quantitative PCR. Nucleotide variants were analyzed either by high-resolution melting analysis and Sanger sequencing of selected exons or by massive sequencing of a panel of genes., [Results]: Forty-seven patients with telomere length below the 10% of normal population, affected with three telomeropathies: dyskeratosis congenita (4), aplastic anemia (22) or pulmonary fibrosis (21) were analyzed. Eighteen of these patients presented known pathogenic or novel possibly pathogenic variants in the telomere-related genes TERT, TERC, RTEL1, CTC1 and ACD. In addition, the analyses of a panel of 188 genes related to haematological disorders indicated that a relevant proportion of the patients (up to 35%) presented rare variants in genes related to DNA repair or in genes coding for proteins involved in the resolution of complex DNA structures, that participate in telomere replication. Mutations in some of these genes are causative of several syndromes previously associated to telomere shortening., [Conclusion]: Novel variants in telomere, DNA repair and replication genes are described that might indicate the contribution of variants in these genes to the development of telomeropathies. Patients carrying variants in telomere-related genes presented worse evolution after diagnosis than the rest of patients analyzed.

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Objective College entrance may be a strategically well-placed “point of capture” for detecting late adolescents with suicidal thoughts and behaviors (STB). However, a clear epidemiological picture of STB among incoming college students is lacking. We present the first cross-national data on prevalence as well as socio-demographic and college-related correlates for STB among first-year college students. Method Web-based self-report surveys were obtained from 13,984 first-year students (response rate 45.5%) across 19 colleges in 8 countries (Australia, Belgium, Germany, Mexico, Northern Ireland, South Africa, Spain, and the United States). Results Lifetime prevalence of suicidal ideation, plans, and attempts was 32.7%, 17.5%, and 4.3%, respectively. The 12-month prevalence was 17.2%, 8.8%, and 1.0%, respectively. About three-fourths of STB cases had onset before the age of 16 years (Q3 = 15.8), with persistence figures in the range of 41% to 53%. About one-half (53.4%) of lifetime ideators transitioned to a suicide plan; 22.1% of lifetime planners transitioned to an attempt. Attempts among lifetime ideators without plan were less frequent (3.1%). Significant correlates of lifetime STB were cross-nationally consistent and generally modest in effect size (median adjusted odds ratio [aOR] = 1.7). Nonheterosexual orientation (aOR range 3.3−7.9) and heterosexual orientation with some same-sex attraction (aOR range 1.9−2.3) were the strongest correlates of STB, and of transitioning from ideation to plans and/or attempts (aOR range 1.6−6.1). Conclusion The distribution of STB in first-year students is widespread, and relatively independent of socio-demographic risk profile. Multivariate risk algorithms based on a high number of risk factors are indicated to efficiently link high-risk status with effective preventive interventions.

El presente texto tiene como objetivo analizar el mecenazgo promovido por mujeres dentro del ámbito de la arquitectura, subrayando la importancia que las promotoras han tenido en la esfera residencial. Se han seleccionado los casos de estudio de tres mujeres: Truss Schröder, Edith Farnsworth y Manorama Sarabhai. A través de sus experiencias con los arquitectos que contrataron, Gerrit Rietveld, Mies van der Rohe y Le Corbusier respectivamente, se analizarán las tres viviendas. En todos los ejemplos seleccionados hubo una intensa relación profesional entre cliente y arquitecto, y la relevancia de las propuestas desarrolladas consiguió elevar el papel clientelar al de mecenas. Mediante un análisis comparativo desde una perspectiva de género, se destacarán los significados implícitos de unas propuestas que superan consideraciones arquitectónicas-espaciales para subrayar cómo gracias a la visión de las mencionadas mujeres se promueve una sostenibilidad aplicada a la arquitectura que puede ser medioambiental, económica y social., The purpose of this text is to analyse the patronage promoted by women in the field of architecture, underlining the importance of these actions in the residential sphere. Three study cases have been selected; Truss Schröder, Edith Farnsworth and Manorama Sarabhai. Through their experiences with the architects they hired, Gerrit Rietveld, Mies van der Rohe and Le Corbusier respectively, three houses will be analysed. In all of them there was an intense professional relationship between client and architect, and the relevance of the developed proposals turned their role as clients into patronage. Through a comparative analysis from a gender perspective, the implicit meanings of proposals that go beyond spatial architectural considerations will be highlighted to underline how, thanks to the vision of these women, a sustainability applied to architecture can be promoted, which can be environmental, economic or social.

El presente texto tiene como objetivo analizar el mecenazgo promovido por mujeres dentro del ámbito de la arquitectura, subrayando la importancia que las promotoras han tenido en la esfera residencial. Se han seleccionado los casos de estudio de tres mujeres: Truss Schröder, Edith Farnsworth y Manorama Sarabhai. A través de sus experiencias con los arquitectos que contrataron, Gerrit Rietveld, Mies van der Rohe y Le Corbusier respectivamente, se analizarán las tres viviendas. En todos los ejemplos seleccionados hubo una intensa relación profesional entre cliente y arquitecto, y la relevancia de las propuestas desarrolladas consiguió elevar el papel clientelar al de mecenas. Mediante un análisis comparativo desde una perspectiva de género, se destacarán los significados implícitos de unas propuestas que superan consideraciones arquitectónicas-espaciales para subrayar cómo gracias a la visión de las mencionadas mujeres se promueve una sostenibilidad aplicada a la arquitectura que puede ser medioambiental, económica y social. ---------- ABSTRACT---------- The purpose of this text is to analyse the patronage promoted by women in the field of architecture, underlining the importance of these actions in the residential sphere. Three study cases have been selected; Truss Schröder, Edith Farnsworth and Manorama Sarabhai. Through their experiences with the architects they hired, Gerrit Rietveld, Mies van der Rohe and Le Corbusier respectively, three houses will be analysed. In all of them there was an intense professional relationship between client and architect, and the relevance of the developed proposals turned their role as clients into patronage. Through a comparative analysis from a gender perspective, the implicit meanings of proposals that go beyond spatial architectural considerations will be highlighted to underline how, thanks to the vision of these women, a sustainability applied to architecture can be promoted, which can be environmental, economic or social.

[Introduction] Haematopoietic stem cell transplantation (HSCT) involves implanting cellular elements capable of generating a new and healthy haematopoietic system. Reduced intensity conditioning (RIC) consists of an immunosuppressive treatment to facilitate a progressive implant with lower morbidity. This type of conditioning can also lead to myelosuppression, which is potentially reversible over time. Reduced intensity conditioning enables HSCT to be performed on patients with genetic diseases for whom added comorbidity is undesirable due to the high doses of chemotherapy that accompanies conventional myeloablative regimens., [Patients and methods] An analysis was performed on the outcomes of 68 paediatric patients with genetic diseases who underwent HSCT with RIC between 2005 and 2013 in the of Paediatric Haematopoietic Stem Cell Transplantation Units that are part of the Spanish Working Group for Bone Marrow Transplantation in Children. A multicentre study was conducted including 68 patients, of whom 43 had Primary Immunodeficiency, 21 with congenital haematological diseases, and 4 with metabolic diseases. RIC in patients with metabolic diseases need to be assessed on an individual basis., [Results] Fifty (73.5%) of the 68 patients were still alive. The Overall Survival (OS) at nine years was 0.74. Twenty-three (33.8%) had some event during the course of the HSCT, with an event-free survival rate of 0.66. The OS in patients with haematological diseases was 0.81, being 0.7 in primary immunodeficiencies, and 0.4 in metabolic diseases. No significant difference was observed between the 3 groups of diseases. As regards the source of haematopoietic progenitors, there was an OS rate of 0.74 in patients transplanted with peripheral blood, 0.70 with bone marrow, and 0.70 and with cord blood, with no statistically significant differences., [Conclusions] Favourable results have been obtained in HSCT with reduced intensity conditioning in genetic diseases. It should be noted that the risks and benefits of the RIC in patients with metabolic diseases need to be assessed on an individual basis., [Introducción] El trasplante de progenitores hematopoyéticos (TPH) consiste en implantar elementos celulares capaces de generar un sistema hematopoyético nuevo y sano. El régimen de intensidad reducida (RIR) consiste en un tratamiento predominantemente inmunosupresor, para facilitar un implante progresivo con menor morbilidad. Este tipo de acondicionamiento puede también provocar mielosupresión, aunque potencialmente reversible en el tiempo. El acondicionamiento RIR permite aplicar TPH a pacientes con enfermedad genética en los que no es deseable añadir comorbilidad por las altas dosis de quimioterapia que conlleva el régimen mieloablativo convencional., [Pacientes y métodos] Se analiza la evolución de 68 pacientes pediátricos con enfermedades genéticas que entre los años 2005-2013 se han sometido a un TPH con RIR en las Unidades pediátricas de Trasplante Hematopoyético de los hospitales españoles integrantes del Grupo Español para Trasplante de Médula Ósea en niños. Se trata de un estudio multicéntrico que incluye a 68 pacientes, de los cuales 43 presentan inmunodeficiencia primaria, 21 presentan hemopatía congénita y 4 están afectados de metabolopatía., [Resultados] Cincuenta de los 68 pacientes se encuentran vivos (73,5%). La supervivencia global (SG) a 9 años es de 0,74. Veintitrés (33,8%) han presentado en el transcurso del TPH algún evento. Supervivencia libre de evento de 0,66. La SG en los pacientes con hemopatía es de 0,81; en las inmunodeficiencias primarias es de 0,70 y en las metabolopatías es de 0,4. No se observa diferencia significativa entre los 3 grupos de enfermedades. Respecto a la fuente de progenitores hematopoyéticos, la SG en los pacientes trasplantados con sangre periférica es de 0,74; con médula ósea es de 0,70 y con la sangre de cordón umbilical es de 0,70. No se observa tampoco diferencia estadística significativa., [Conclusiones] En nuestro trabajo, de ámbito nacional, hemos evidenciado unos resultados favorables en TPH con régimen de intensidad reducida en las enfermedades genéticas. Cabe destacar que las metabolopatías requieren una consideración individualizada para sopesar en cada paciente los riesgos y beneficios que comporta el RIR.

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En aquest treball estudiarem un cas particular del problema de 4 cossos, el problema colineal i simètric dos a dos respecte l'origen. Ens centrarem en el cas que les quatre masses col·lisionen a l'origen i analitzarem, mitjançant simulacions amb MATLAB, com afecta la variació de la massa a les dinàmiques dels cossos. Primer plantejarem el model i el trnasformarem per evitar la singularitat en la col·lisió quàdruple. Després trobarem la varietat de col·lisió total i calcularem els punts d'equilibri, així com les varietats invariants, que viuen en la varietat de col·lisió quàdruple. Finalment, trobarem per quines masses tindrem òrbites que connectin els punts d'equilibri calculats prèviament.

El servicio de búnker consiste en suministrar combustible a los buques mediante tubería, surtidor, camión o gabarra. En el puerto de Barcelona hay distintos operadores petrolíferos que distribuyen combustible a los buques mediante gabarras, siendo los fuelóleos IFO 380, IFO 380 LS y RMK 500 los ofrecidos en el puerto barcelonés. Algunas de las terminales del puerto cuentan con tanques de almacenamiento destinados a estos productos, pero ninguna de ellas dispone de grandes cantidades de almacenamiento para este propósito. Este trabajo estudia la normativa española que regula el diseño y construcción de parques de almacenamiento de líquidos petrolíferos, aprobada por el Real Decreto 1562/1998, y se plantea el diseño de construcción de una terminal en Barcelona dedicada a la recepción de combustibles marinos, almacenamiento y carga en gabarras para su posterior distribución. En primer lugar, se deben establecer las capacidades de almacenamiento de los tanques y la disposición de estos en un terreno. Por otra parte, el diseño debe tener en cuenta muchos otros factores, como son las distancias que se deben dejar entre instalaciones, las capacidades de los tanques o las medidas de protección contra incendios. También se realiza un estudio del impacto ambiental que supondría la construcción del parque, tanto durante su construcción como en su fase posterior de operación, donde se analizan los riesgos que supone construir una terminal para el medio y donde se proponen medidas correctivas y de mejora.

We investigated prospectively factors influencing the safety of hematopoietic stem cell (HSC) collection in 453 pediatric donors. The children in the study donated either BM or peripheral blood stem cells (PBSCs) according to center policy. A large variability in approach to donor issues was observed between the participating centers. Significant differences were observed between BM and PBSC donors regarding pain, blood allotransfusion, duration of hospital stay, and iron supplementation; however, differences between the groups undergoing BM vs PBSC donation preclude direct risk comparisons between the 2 procedures. The most common adverse event was pain, reported mainly by older children after BM harvest, but also observed after central venous catheter (CVC) placement for PBSC collection. With regard to severe adverse events, one patient (0.7%) developed a pneumothorax with hydrothorax after CVC placement for PBSC collection. The risk of allotransfusion after BM harvest was associated with a donor age of < 4 years and a BM harvest volume of > 20 mL/kg. Children < 4 years were at higher risk than older children for allotransfusion after BM harvest and there was a higher risk of complications from CVC placement before apheresis. We conclude that PBSC and BM collection are safe procedures in children. (Blood. 2012;119(12):2935-2942), We investigated prospectively factors influencing the safety of hematopoietic stem cell (HSC) collection in 453 pediatric donors. The children in the study donated either BM or peripheral blood stem cells (PBSCs) according to center policy. A large variability in approach to donor issues was observed between the participating centers. Significant differences were observed between BM and PBSC donors regarding pain, blood allotransfusion, duration of hospital stay, and iron supplementation; however, differences between the groups undergoing BM vs PBSC donation preclude direct risk comparisons between the 2 procedures. The most common adverse event was pain, reported mainly by older children after BM harvest, but also observed after central venous catheter (CVC) placement for PBSC collection. With regard to severe adverse events, one patient (0.7%) developed a pneumothorax with hydrothorax after CVC placement for PBSC collection. The risk of allotransfusion after BM harvest was associated with a donor age of < 4 years and a BM harvest volume of > 20 mL/kg. Children < 4 years were at higher risk than older children for allotransfusion after BM harvest and there was a higher risk of complications from CVC placement before apheresis.We conclude that PBSC and BM collection are safe procedures in children. © 2012 by The American Society of Hematology.

Viajar es en sí mismo una aventura en la que, cada vez que descubrimos una nueva ciudad, nos sorprendemos al comprobar que está muy lejos de ser lo que habíamos esperado. De hecho, contrariamente a lo que podríamos suponer, cuanto más famoso es un destino, más expectativas tenemos acerca del mismo y mayor puede ser la sorpresa. El principio de “anclaje y ajuste” explica, desde el ámbito del Marketing, cómo los decisores necesitan siempre alguna referencia inicial antes de hacer cualquier valoración. Para tomar una mejor decisión, ese “anclaje” debe adaptarse, “ajustarse” durante el proceso. Este principio sirve entonces, para explicar por qué las ciudades pueden servir de referencia para comunicar un determinado mensaje, y para introducir una reflexión acerca de qué simboliza realmente Roma y cómo ese símbolo se utiliza actualmente en comunicación de moda. ----------ABSTRACT---------- Traveling is an adventure in itself in which, every time we reach a new city, we are surprised checking that reality does not correspond to our expectations. In fact, and contrary to common sense, the more a city is famous, the bigger we would be surprised by visiting it. “Anchoring and adjustment” in Marketing, explains how decision makers needs some reference before doing any valuation. To take better decisions, the previous “anchor” must be adapted, “adjusted” during the process. Therefore, anchor and adjustment principle can explain why cities serve as reference in marketing strategies. Moreover, this marketing principle introduces a reflection on what Rome actually symbolizes, and how this symbol is used in fashion communication.

Aquest estudi té per objectiu fer un anàlisi exhaustiu de les maniobres més habituals realitzades per grans iots al port de Vilanova i la Geltrú per tal de poder demostrar posteriorment la seva viabilitat amb diferents condicions donades pels factors meteorològics externs. Es pretén buscar, doncs, els límits meteorològics i operatius en la maniobra a port. Per poder-ho dur a terme s’han utilitzat documents i informació cedits per “Vilanova Grand Marina” que han permès elaborar una descripció detallada de les instal·lacions portuàries i els serveis oferts per la marina. També s’ha comptat amb la col·laboració del Capità de Port, que m’ha permès conèixer les instal·lacions a través de converses i visites a la marina. A més, per a la realització de la part pràctica de l’estudi s’ha utilitzat el simulador de navegació de la Facultat Nàutica de Barcelona (Transas NTPro 5000 v 5.35). La següent tesi exposa quines són les característiques dels iots i els factors que influeixen en la maniobra a port per tal de comprovar-ne els efectes utilitzant el simulador de navegació amb un model de vaixell similar. Finalment, s’estableixen uns límits meteorològics concrets en les maniobres simulades que es podran utilitzar com a indicadors en les que es realitzin de forma real en endavant. Main target of this study is to make a thorough analysis of the most common manoeuvres large yachts perform in the harbour of Vilanova i la Geltrú in order to confirm its feasibility with different weather conditions. The aim is seeking for operational and meteorological boundaries to manoeuvre safely in the port. To do so, I have been working on documents and information supplied by Vilanova Grand Marina which permitted to develop a detailed description of the port facilities and services provided by the company. I also had a great support from the Harbour Master, who fully open his facilities to me, with visits to the marina and deep explanations about their functionality . For the practice part of

Este trabajo intenta profundizar en el mundo del tráfico marítimo de contenedores, dando a conocer el día a día de una terminal puntera y pionera en el mundo como es la Terminal BEST. Está enfocado principalmente a la operativa de una terminal de contenedores, pero para ello, es necesario hablar un poco sobre el estado actual del comercio marítimo. En España el puerto de Barcelona se sitúa en tercer lugar en cuanto a volumen de carga manipulado (más de 47 millones de toneladas en 2016), siendo el primero, el puerto de Algeciras, que movió en el 2016 más de 100 millones de toneladas y seguido del puerto de Valencia con más de 71 millones de toneladas. Barcelona Europe South Terminal, es una terminal perteneciente a la multinacional HUTCHINSON PORT HOLDINGS, la cual dispone de 48 terminales en todo el mundo y fue capaz de mover el año pasado 81,4 millones de TEU’s.

ResumenEl contexto climático y paisajístico es esencial para estudiar las raíces de cualquier sociedad y su identidad cultural. En la génesis de las primeras sociedades, su relación con el entorno natural definió las bases de su idiosincrasia y religión, todo ello reflejado en sus manifestaciones artísticas. A través de un análisis de base antropológica, aplicado al caso del mediterráneo, se estudiarán las consecuencias de del clima y el paisaje. La Europa clásica, con sociedades como la romana y la griega, evolucionaron a lo largo del tiempo partiendo de condiciones climatológicas cercanas a las regiones monzónicas, sin embargo terminó importando una religión del desierto. Este tema es básico para poder hacer una lectura de sus manifestaciones artísticas posteriores entendiendo su origen estrechamente ligado a su contexto natural. El Panteón de Roma servirá como estudio de caso para analizarlo desde una nueva perspectiva recogiendo todas las variables previamente estudiadas. AbstractClimate and landscape context is essential to study the roots of any society and its cultural identity. In the genesis of the first societies, their relationship with the natural environment defined the bases of its idiosyncrasy and religion, all reflected in its art. Through an anthropological analysis based on monsoon and desert climate contexts, the impact of weather conditions and the landscape will be studied in order to apply it to the Mediterranean case. During the Classical Europe period, Romans and Greeks evolved over time based on weather conditions closer to the monsoon regions and ended up importing a religion of the desert. This topic is basic to analyse later artistic examples and the origin closely linked to their natural context. The Pantheon will serve as a study case to analyze it from a new perspective by collecting all the variables previously studied.