954 resultados para BASE-LINE SYNDROME


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Acquired Fanconi syndrome is characterized by inappropriate urinary loss of amino acids, bicarbonate, electrolytes, and water. It has recently been described in dogs fed chicken jerky treats from China, a new differential diagnosis to the classical inciting infectious diseases (e.g. leptospirosis, pyelonephritis) and toxins. A dog fed exclusively chicken jerky treats purchased in Switzerland was presented to our clinic with severe polyuria, polydipsia and profound electrolyte and acid base disturbances. Other inciting causes of Fanconi syndrome were ruled out. The requirement of a very intensive supportive treatment in this dog stands in contrast to treatment of chronic forms of Fanconi syndrome as described in the Basenji. This intensive therapy and the associated monitoring can be a real challenge and a limiting factor for the prognosis of acquired Fanconi syndrome. Veterinarians should be aware of the risk of excessive feeding of chicken jerky treats.

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Close similarities of various physiological parameters makes the pig one of the preferred animal models for the study of human diseases, especially those involving the cardiovascular system. Unfortunately, the use of pig models to study diseases such as viral hemorrhagic fevers and endotoxic shock syndrome have been hampered by the lack of the necessary immunological tools to measure important immunoregulatory cytokines such as tumor necrosis factor (TNF). Here we describe a TNF-bioassay which is based on the porcine kidney cell line PK(15). Compared to the widely used murine fibroblastoid cell line L929, the PK(15) cell line displays a 100-1000-fold higher sensitivity for porcine TNF-alpha, a higher sensitivity for human TNF-alpha, and a slightly lower sensitivity for murine TNF-alpha. Using a PK(15) bioassay we can detect recombinant TNF-alpha as well as cytotoxic activity in the supernatants of lipopolysaccharide (LPS)-activated porcine monocytes at high dilutions. This suggests that the sensitivity of the test should permit the detection of TNF in biological specimens such as pig serum.

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BACKGROUND Glatiramer acetate is worldwide used as first line treatment in relapsing remitting multiple sclerosis. Local skin reactions associated with glatiramer acetate are common, however, only isolated cases of severe local injection site reactions known as Nicolau Syndrome have been reported so far. CASE PRESENTATION We describe the case of a recurrent Nicolau Syndrome occurred during longstanding glatiramer acetate treatment in a woman with multiple sclerosis. The haemorrhagic patch necrotized and was treated locally as a deep second degree burn with excision of dead skin tissue and was healed. Treatment with glatiramer acetate was definitely suspended. CONCLUSIONS GA injections can be complicated by isolated or recurrent Nicolau Syndrome, a potentially life-threatening condition of which neurologists should be aware.

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Lynch syndrome, is caused by inherited germ-line mutations in the DNA mismatch repair genes resulting in cancers at an early age, predominantly colorectal (CRC) and endometrial cancers. Though the median age at onset for CRC is about 45 years, disease penetrance varies suggesting that cancer susceptibility may be modified by environmental or other low-penetrance genes. Genetic variation due to polymorphisms in genes encoding metabolic enzymes can influence carcinogenesis by alterations in the expression and activity level of the enzymes. Variation in MTHFR, an important folate metabolizing enzyme can affect DNA methylation and DNA synthesis and variation in xenobiotic-metabolizing enzymes can affect the metabolism and clearance of carcinogens, thus modifying cancer risk. ^ This study examined a retrospective cohort of 257 individuals with Lynch syndrome, for polymorphisms in genes encoding xenobiotic-metabolizing enzymes-- CYP1A1 (I462V and MspI), EPHX1 (H139R and Y113H), GSTP1 (I105V and A114V), GSTM1 and GSTT1 (deletions) and folate metabolizing enzyme--MTHFR (C677T and A1298C). In addition, a series of 786 cases of sporadic CRC were genotyped for CYP1A1 I462V and EPHX1 Y113H to assess gene-gene interaction and gene-environment interaction with smoking in a case-only analysis. ^ Prominent findings of this study were that the presence of an MTHFR C677T variant allele was associated with a 4 year later age at onset for CRC on average and a reduced age-associated risk for developing CRC (Hazard ratio: 0.55; 95% confidence interval: 0.36–0.85) compared to the absence of any variant allele in individuals with Lynch syndrome. Similarly, Lynch syndrome individuals heterozygous for CYP1A1 I462V A>G polymorphism developed CRC an average of 4 years earlier and were at a 78% increased age-associated risk (Hazard ratio for AG relative to AA: 1.78; 95% confidence interval: 1.16-2.74) than those with the homozygous wild-type genotype. Therefore these two polymorphisms may be additional susceptibility factors for CRC in Lynch syndrome. In the case-only analysis, evidence of gene-gene interaction was seen between CYP1A1 I462V and EPHX1 Y113H and between EPHX1 Y113H and smoking suggesting that genetic and environmental factors may interact to increase sporadic CRC risk. Implications of these findings are the ability to identify subsets of high-risk individuals for targeted prevention and intervention. ^

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Hyper IgE syndrome (HIES) is a multisystem disorder resulting in bone and immune system abnormalities. It is associated with mutations in STAT3, which disrupt protein domains responsible for transcriptional function. Patients with HIES display osteoporosis and enhanced inflammatory cytokine production similar to hematopoietic Stat3-deficient mice. Since osteoclast and inflammatory cytokine genes are NFκB targets, these observations indicate a possible deregulation of NFκB signaling in both mice and humans with STAT3-deficiency. Here, we sought to examine the role of STAT3 in the regulation of NFκB-mediated gene expression through analysis of three HIES STAT3 point mutations in both hematopoietic and non- hematopoietic cells. We found that IL-6-induced tyrosine phosphorylation of STAT3 was partially or completely abrogated by HIES mutations in the transactivation domain (V713L) or SH2 domain (V637M), respectively, in both hematopoietic and non- hematopoietic cells. By contrast, IL-6-induced tyrosine phosphorylation of an HIES mutant in the STAT3 DNA-binding domain (R382W) was intact. The R382W and V713L mutants significantly reduced IL-6-dependent STAT3 transcriptional activity in reporter gene assays. Moreover, the R382W and V637M mutants significantly diminished IL-6-responsive expression of the endogenous STAT3 target gene, Socs3, as assessed by quantitative real-time PCR (qPCR) in the RAW macrophage cell line. These observations indicate the HIES mutants dominantly suppress the transcriptional activity of wild type STAT3, albeit to varying degrees. All three HIES mutants enhanced LPS-induced expression of the NFκB target genes IL6 (IL-6), Cxcl10 (IP- 10), and Tnf (TNFα) in RAW cells, as indicated by qPCR. Furthermore, overexpression of wild type STAT3 in Stat3-deficient murine embryonic fibroblasts significantlyreduced LPS-stimulated expression of IL6, Cxcl10, and IL12p35. In addition, in aprimary murine osteoclast differentiation assay, a STAT3-specific SH2 domain inhibitor led to significantly increased levels of osteoclast-specific gene expression. These results suggest that STAT3 serves as a negative regulator of NFκB-mediated gene expression, and furthermore imply that STAT3 mutations associated with HIES contribute to the osteopenia and inflammation observed in HIES patients.

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Iceberg calving has been assumed to be the dominant cause of mass loss for the Antarctic ice sheet, with previous estimates of the calving flux exceeding 2,000 gigatonnes per year. More recently, the importance of melting by the ocean has been demonstrated close to the grounding line and near the calving front. So far, however, no study has reliably quantified the calving flux and the basal mass balance (the balance between accretion and ablation at the ice-sheet base) for the whole of Antarctica. The distribution of fresh water in the Southern Ocean and its partitioning between the liquid and solid phases is therefore poorly constrained. Here we estimate the mass balance components for all ice shelves in Antarctica, using satellite measurements of calving flux and grounding-line flux, modelled ice-shelf snow accumulation rates and a regional scaling that accounts for unsurveyed areas. We obtain a total calving flux of 1,321 ± 144 gigatonnes per year and a total basal mass balance of -1,454 ± 174 gigatonnes per year. This means that about half of the ice-sheet surface mass gain is lost through oceanic erosion before reaching the ice front, and the calving flux is about 34 per cent less than previous estimates derived from iceberg tracking. In addition, the fraction of mass loss due to basal processes varies from about 10 to 90 per cent between ice shelves. We find a significant positive correlation between basal mass loss and surface elevation change for ice shelves experiencing surface lowering and enhanced discharge. We suggest that basal mass loss is a valuable metric for predicting future ice-shelf vulnerability to oceanic forcing.

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Software Product Line Engineering (SPLE) is becoming widely used due to the improvement it means when developing software products of the same family. However, SPLE demands long-term investment on a product-line platform that might not be profitable due to rapid changing business settings. Since Agile Software Development (ASD) approaches are being successfully applied in volatile markets, several companies have suggested the idea of integrating SPLE and ASD when a family product has to be developed. Agile Product Line Engineering (APLE) advocates the integration of SPLE and ASD to address their lacks when they are individually applied to software development. A previous literature re-view of experiences and practices on APLE revealed important challenges about how to fully put APLE into practice. Our contribution address several of these challenges by tailoring the agile method Scrum by means of three concepts that we have defined: plastic partial components, working PL-architectures, and reactive reuse.

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Desde la explosión de crecimiento de internet que comenzó en los años 90, se han ido creando y poniendo a disposición de los usuarios diversas herramientas para compartir información y servicios de diversas formas, desde el nacimiento del primer navegador hasta nuestros días, donde hay infinidad de lenguajes aplicables al ámbito web. En esta fase de crecimiento, en primer lugar, de cara a usuarios individuales, saldrían herramientas que permitirían a cada cual hacer su web personal, con sus contenidos expuestos. Más adelante se fue generando el fenómeno “comunidad”, con, por ejemplo, foros, o webs en las que había múltiples usuarios que disfrutaban de contenidos o servicios que la web ofreciese. Este crecimiento del mundo web en lo comunitario ha avanzado en muchas ramas,entre ellas, por supuesto, la educacional, surgiendo plataformas como la que es base del proyecto que a continuación se presenta, y herramienta básica y prácticamente ya imprescindible en la enseñanza universitaria: Moodle. Moodle es una herramienta diseñada para compatir recursos y diseñar actividades para el usuario potencial, complementando su aprendizaje en aula, o incluso siendo una vía autónoma de aprendizaje en sí misma. Se ha realizado un estudio sobre el estado de saludo de los contenidos que se exponen en Moodle, y se ha encontrado que una gran mayoría de los cursos que se pueden visitar tienen un gran número de carencias. Por un lado, hay pocos con material original explotado exclusivamente para el curso, y, si tienen material original, no se ha observado una especial atención por la maquetación. Por otro lado, hay muchos otros sin material original, y, en ambos casos, no se ha encontrado ningún curso que ofrezca material audiovisual exclusivo para el curso, presentando algunos en su lugar material audiovisual encontrado en la red (Youtube, etc). A la vista de estos hechos, se ha realizado un proyecto que intenta aportar soluciones ante estas carencias, y se presenta un curso procedente de diversas referencias bibliográficas, para la parte textual, y material audiovisual original e inédito que también se ha explotado específicamente para este curso. Este material ha sido por un lado vídeo, que se ha visionado, editado y subtitulado con software de libre distribución, y por otro lado, audio, que complementa un completo glosario que se ha añadido como extra al curso y cuyo planteamiento no se ha encontrado en ningún curso online de los revisados. Todo esto se ha envuelto en una maquetación cuidada que ha sido fruto del estudio de los lenguajes web html y CSS, de forma que, por un lado, el curso sea un lugar agradable en el que aprender dentro de internet, y por otro, se pudiesen realizar ciertas operaciones que sin estos conocimientos habrían sido imposibles, como la realización del glosario o la incrustación de imágenes y vídeos. A su vez, se ha tratado de dar un enfoque didáctico a toda la memoria del proyecto, de forma que pueda ser de utilidad a un usuario futuro que quisiese profundizar en los usos de Moodle, introducirse en el lenguaje web, o introducirse en el mundo de la edición de vídeo. ABSTRACT: Since the explosion of Internet growth beginning in the 90s, many tools have been created and made available for users to share information and services in various ways, from the birth of the first browser until today, where there are plenty of web programming languages. This growth stage would give individual users tools that would allow everyone to make an own personal website, with their contents exposed. Later, the "community" phenomenon appeared with, for example, forums, or websites where multiple users enjoyed the content or web services that those websites offered. Also, this growth in the web community world has progressed in many fields, including education, with the emerge of platforms such as the one that this project uses as its basis, and which is the basic and imperative tool in college education: Moodle. Moodle is a tool designed to share resources and design activities for the potential user, completing class learning, or even letting this user learn in an autonomous way. In this project a study on the current situation of the content present in Moodle courses around the net has been carried out, and it has been found that most of them lack of original material exploited exclusively for the courses, and if they have original material, there has been not observed concern on the layout where that material lies. On the other hand, there are many other with non original material, and in both cases, there has not been found any course that offers audio- visual material made specifically for the course, instead of presenting some audiovisual material found on the net (Youtube, etc). In view of these facts, the project presented here seeks to provide solutions to these shortcomings, presenting a course with original material exploited from various references, and unpublished audioevisual material which also has been exploited specifically for this course. This material is, on one hand, video, which has been viewed, edited and subtitled with free software, and on the other, audio, which complements a comprehensive glossary that has been added as an extra feature to the course and whose approach was not found in any of the online courses reviewed. All of this has been packaged in a neat layout that has been the result of the study of web languages HTML and CSS, so that first, the course was a pleasant place to learn on the internet, and second, certain operations could be performed which without this knowledge would have been impossible, as the glossary design or embedding images and videos. Furthermore, a didactic approach has been adopted to the entire project memory, so it can be useful to a future user who wanted to go deeper on the uses of Moodle, containing an intro into the web language, or in the world video editing.

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The aim of this thesis is to study the mechanisms of instability that occur in swept wings when the angle of attack increases. For this, a simplified model for the a simplified model for the non-orthogonal swept leading edge boundary layer has been used as well as different numerical techniques in order to solve the linear stability problem that describes the behavior of perturbations superposed upon this base flow. Two different approaches, matrix-free and matrix forming methods, have been validated using direct numerical simulations with spectral resolution. In this way, flow instability in the non-orthogonal swept attachment-line boundary layer is addressed in a linear analysis framework via the solution of the pertinent global (Bi-Global) PDE-based eigenvalue problem. Subsequently, a simple extension of the extended G¨ortler-H¨ammerlin ODEbased polynomial model proposed by Theofilis, Fedorov, Obrist & Dallmann (2003) for orthogonal flow, which includes previous models as particular cases and recovers global instability analysis results, is presented for non-orthogonal flow. Direct numerical simulations have been used to verify the stability results and unravel the limits of validity of the basic flow model analyzed. The effect of the angle of attack, AoA, on the critical conditions of the non-orthogonal problem has been documented; an increase of the angle of attack, from AoA = 0 (orthogonal flow) up to values close to _/2 which make the assumptions under which the basic flow is derived questionable, is found to systematically destabilize the flow. The critical conditions of non-orthogonal flows at 0 _ AoA _ _/2 are shown to be recoverable from those of orthogonal flow, via a simple analytical transformation involving AoA. These results can help to understand the mechanisms of destabilization that occurs in the attachment line of wings at finite angles of attack. Studies taking into account variations of the pressure field in the basic flow or the extension to compressible flows are issues that remain open. El objetivo de esta tesis es estudiar los mecanismos de la inestabilidad que se producen en ciertos dispositivos aerodinámicos cuando se aumenta el ángulo de ataque. Para ello se ha utilizado un modelo simplificado del flujo de base, así como diferentes técnicas numéricas, con el fin de resolver el problema de estabilidad lineal asociado que describe el comportamiento de las perturbaciones. Estos métodos; sin y con formación de matriz, se han validado utilizando simulaciones numéricas directas con resolución espectral. De esta manera, la inestabilidad del flujo de capa límite laminar oblicuo entorno a la línea de estancamiento se aborda en un marco de análisis lineal por medio del método Bi-Global de resolución del problema de valores propios en derivadas parciales. Posteriormente se propone una extensión simple para el flujo no-ortogonal del modelo polinomial de ecuaciones diferenciales ordinarias, G¨ortler-H¨ammerlin extendido, propuesto por Theofilis et al. (2003) para el flujo ortogonal, que incluye los modelos previos como casos particulares y recupera los resultados del analisis global de estabilidad lineal. Se han realizado simulaciones directas con el fin de verificar los resultados del análisis de estabilidad así como para investigar los límites de validez del modelo de flujo base utilizado. En este trabajo se ha documentado el efecto del ángulo de ataque AoA en las condiciones críticas del problema no ortogonal obteniendo que el incremento del ángulo de ataque, de AoA = 0 (flujo ortogonal) hasta valores próximos a _/2, en el cual las hipótesis sobre las que se basa el flujo base dejan de ser válidas, tiende sistemáticamente a desestabilizar el flujo. Las condiciones críticas del caso no ortogonal 0 _ AoA _ _/2 pueden recuperarse a partir del caso ortogonal mediante el uso de una transformación analítica simple que implica el ángulo de ataque AoA. Estos resultados pueden ayudar a comprender los mecanismos de desestabilización que se producen en el borde de ataque de las alas de los aviones a ángulos de ataque finitos. Como tareas pendientes quedaría realizar estudios que tengan en cuenta variaciones del campo de presión en el flujo base así como la extensión de éste al caso de flujos compresibles.

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Firm location patterns emerge as a consequence of multiple factors, including firm considerations, labor force availability, market opportunities, and transportation costs. Many of these factors are influenced by changes in accessibility wrought by new transportation infrastructure. In this paper we use spatial statistical techniques and a micro-level data base to evaluate the effects of Madrid?s metro line 12 (known as Metrosur) expansion on business location patterns. The case study is the municipality of Alcorcon, which is served by the new metro line since 2003. Specifically, we explore the location patterns by different industry sectors, to evaluate if the new metro line has encouraged the emergence of a ?Metrosur spatial economy?. Our results indicate that the pattern of economic activity location is related to urban accessibility and that agglomeration, through economies of scale, also plays an important role. The results presented in this paper provide evidence useful to inform efficient transportation, urban, and regional economic planning.

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Characteristics of the impacts su!ered by the fruit on a transfer point of an experimental fruit packing line were analysed. The transfer is made up by two transporting belts at di!erent heights forming an angle of 903. These transfer points are very common in fruit packing lines, in which fruits receive two impacts: the "rst onto the belt base and the second into the lateral plate. Diferent tests were carried out to study the e!ect of transfer height, velocity, belt structure and padding on the acceleration values recorded by an instrumental sphere (IS 100). Results showed that transfer height and belt structure a!ect mainly impact values on the belt base, and padding a!ects mainly impact values registered for lateral contact. The elect of belt velocity in both impacts is less important when compared to the rest of the variables. Additionally, two powered transfer decelerators were tested at the same point with the aim of decreasing impacts su!ered by the fruit. Comparing impacts registered using these decelerators to those analysed in the first part of the study without decelerators, a high reduction of the impact values was observed.

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Basándonos en la recopilación inicial de preposiciones, locuciones preposicionales, términos con preposición dependiente y phrasal verbs utilizados en el texto técnico realizada en otros proyectos anteriores del Departamento de Lingüística Aplicada a la Ciencia y a la Tecnología, el objetivo de este trabajo es completar, organizar, actualizar y dar visibilidad a esta información inicial. Tras realizar un proceso exhaustivo de verificación, unificación, clasificación y ampliación de la información existente, en caso necesario, el listado resultante se utiliza para elaborar un glosario de términos con preposición. El objetivo final de este proyecto es que este glosario esté a disposición de los usuarios, a través de una consulta on-line, en la página del ILLLab (http://illlab.euitt.upm.es/wordpress/), dependiente del Departamento de Lingüística Aplicada a la Ciencia y a la Tecnología. Para incluir en el glosario ejemplos actualizados de textos técnicos, se ha recopilado un corpus lingüístico de textos técnicos, tomando como base diferentes números de la revista IEEE Spectrum, en su edición digital, publicados entre los años 2009 y 2012. El objetivo de esta recopilación es la de ofrecer al consultante diferentes ejemplos de uso en el texto técnico de los distintos términos con preposición que componen el glosario, de manera que pueda acceder de manera rápida y sencilla a ejemplos de uso real de los términos que está buscando, con objeto de clarificar aspectos relacionados con su uso o, en su caso, facilitar su aprendizaje. Toda esta información, tanto el listado de términos con preposición como las frases pertenecientes al corpus recopilado, se incorpora a una base de datos, alojada dentro de la misma página web del ILLLab. A través de un formulario de consulta, a disposición del usuario en dicha página, se pueden obtener todos los términos recopilados que coincidan con los criterios de búsqueda introducidos. El usuario puede realizar dos tipos de búsqueda principales: por preposición o por término completo. Además, puede elegir una búsqueda global (entre todos los términos que integran el glosario) o parcial (en una sola de las categorías en las que se han dividido los diferentes términos, de acuerdo con su función gramatical). Por último, se presentan unas estadísticas de uso de los términos recopilados dentro de los diferentes textos que integran el corpus lingüístico, de manera que pueda establecerse una relación de los que aparecen con más frecuencia en el texto técnico. ABSTRACT. Based on the initial collection of prepositions, prepositional phrases, dependent prepositions and phrasal verbs used in technical texts collected on previous projects in the Department of Applied Linguistics to Science and Technology, the aim of this project is to improve, organize, update and provide visibility to this initial information. Following a process of verification, unification, classification and extension of existing information, if necessary, a glossary of terms with preposition is built. The ultimate objective of this project is to make this glossary available to users through an online consultation in the ILLLab webpage (http://illlab.euitt.upm.es/wordpress/). The administration of tis webpage depends of the Department of Applied Linguistics in Science and Technology. A linguistic corpus of technical texts has been compiled, based on different numbers of the IEEE Spectrum magazine, in its online edition, published between the years 2009 and 2012. The aim of this collection is to provide different examples of use in the technical text for the terms included in the glossary, so that examples of the actual use of the terms consulted can be easily and quickly accessed, in order to clarify doubts regarding their meaning or translation into Spanish and facilitate learning. All this information, both the list of terms with prepositional phrases as well as the corpus developed, is incorporated in a database. Through a searching form, the ILLLab's user may obtain all the terms matching the search criteria entered. The user can perform two types of main search: by preposition or by full term. Additionally, a global search can be selected (including all terms included in the glossary) or a partial one (including only one of the glossary's categories). Finally, some statistics of use are presented according to the various texts included in the corpus, so a relation of the most frequent prepositions in the technical text can be established.

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Characteristics of the impacts suffered by the fruit on a transfer point of an experimental fruit packing line were analysed. The transfer is made up by two transporting belts at different heights forming an angle of 90°. These transfer points are very common in fruit packing lines, in which fruits receive two impacts: the first onto the belt base and the second into the lateral plate. Different tests were earned out to study the effect of transfer height, velocity, belt structure and padding on tire acceleration values recorded by an instrumental sphere (IS 100). Results showed that transfer height and belt structure affect mainly impact values on the belt base, and padding affects mainly impact values registered in lateral impact. The effect of belt velocity in both impacts is less important when compared to the rest of the variables. Additionally, two powered transfer decelerators were tested at the same point with the aim of decreasing impacts suffered by the fruit. Comparing impacts registered using these decelerators to those analysed in the first part of the study without decelerators a high reduction of the impact values was observed.

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The banking industry is observing how new competitors threaten its millennial business model by targeting unbanked people, offering new financial services to their customer base, and even enabling new channels for existing services and customers. The knowledge on users, their behaviour, and expectations become a key asset in this new context. Well aware of this situation, the Center for Open Middleware, a joint technology center created by Santander Bank and Universidad Politécnica de Madrid, has launched a set of initiatives to allow the experimental analysis and management of socio-economic information. PosdataP2P service is one of them, which seeks to model the economic ties between the holders of university smart cards, leveraging on the social networks the holders are subscribed to. In this paper we describe the design principles guiding the development of the system, its architecture and some implementation details.