Giant Bilateral Renal Angiomyolipomas and Lymphangioleiomyomatosis Presenting after Two Successive Pregnancies Successfully Treated with Surgery and Rapamycin

We report the case of a 25-year-old woman who presented with abdominal and flank pain with two successive pregnancies and was diagnosed of giant bilateral renal AMLs and pulmonary LAM associated with TSC in the post-partum of her second pregnancy. This case illustrates that in women with TSC rapid growth from renal AMLs and development of LAM may occur with successive pregnancies. It also stresses the potential for preservation of renal function despite successive bilateral renal surgery of giant AMLs. Moreover, the treatment with a low-dose rapamycin may be an option for LAM treatment. Finally, a low-dose rapamycin may be considered as an adjuvant treatment together to kidney-sparing conservative surgery for renal AMLs

Los resultados de la implantación de la Normativa ISO 9001 en el sector de la construcción español

JEL: L15, M10[EN] The ISO 9001 Standard – a quality assurance standard for products and services – is well established in the construction industry, which is one of the major industries in the world, including Spain. The objectives of this paper are, on the one hand, to analyse the results of adhering to this standard for construction companies in Spain, and on the other, to examine whether these results are affected by the size of the organization and the seniority of the certification. To this end, a questionnaire was sent to quality managers of 302 companies in the industry, and the responses of 126 valid questionnaires received were treated by descriptive analysis and ANOVA. From the answers gathered is concluded that obtaining the ISO 9001 certification has positive effects leading to benefits which are both “internal” and “external”. Some of the most significant internal benefits mentioned were improvements to the organisational processes, while among the external benefits, commercial aspects, such as better chances of competing on the market, were mentioned most frequently. It was also observed that the size of the organization does not significantly modify the positive effects of adherence to the standard, while the age of the certification itself, has a significant influence on the positive results of the adherence.[ES] El sector de la construcción es uno de los más importantes tanto a nivel mundial como español, y presenta una amplia implantación de la Norma ISO 9001 como norma de aseguramiento de la calidad de sus productos y servicios. Los objetivos del presente trabajo son, por un lado, analizar los resultados derivados de la adhesión a dicha Norma por parte de las empresas del sector de la construcción español, y por otro, verificar si dichos resultados están influenciados por el tamaño de dichas empresas y la antigüedad de la certificación. Para ello se ha remitido un cuestionario a los directores de calidad de 302 empresas de dicho sector, recibiéndose 126 cuestionarios válidos, cuyas respuestas fueron tratadas mediante un análisis descriptivo y ANOVA. De las respuestas recogidas se concluye que la implantación de ISO 9001 tiene una influencia positiva para la obtención de beneficios tanto de índole “interna” como “externa”. Dentro de los beneficios “internos”, destacan las mejoras organizativas, mientras que en lo relativo a los “externos” destacan los aspectos comerciales, es decir, la posibilidad de competir en el mercado. Así mismo, se verifica que el tamaño de las empresas no provoca diferencias significativas en la influencia positiva de la adhesión a la Norma, mientras que la antigüedad de la certificación sí influye de forma significativa en los resultados positivos de dicha adhesión

Posible interrupción de la autovía GC-2 (Gran Canaria) por deslizamientos y desprendimientos rocosos: análisis de estabilidad del acantilado de el Rincón

En este trabajo se han identificado los deslizamientos y desprendimientos que se pueden desarrollar en el acantilado de El Rincón (Gran Canaria) y sus repercusiones sobre la Autovía GC-2, en la base del acantilado. El análisis de estabilidad realizado para las condiciones actuales indica que la ladera es estable. El análisis a largo plazo considera el sustrato saturado en agua y se limita a los dos bloques rocosos más susceptibles de experimentar deslizamiento: uno a media ladera y otro en coronación. El deslizamiento del bloque de coronación parece más probable ya que sólo requiere la saturación de los piroclastos y está favorecido por la progresiva apertura de la grieta subvertical. Por último, la evaluación de los desprendimientos rocosos que afectan al acantilado indica que los bloques no alcanzarían la calzada de la autovía GC-2; sino que se acumularían en los conos de deyección presentes al pie de la ladera

El consumidor español y su utilización de las tarjetas bancarias: líneas de actuación para su desarrollo en el futuro.

El objetivo de este trabajo es analizar cual es el nivel actual de utilización de las tarjetas bancarias por parte de los consumidores españoles, así como los motivos que lo justifican, de tal forma que las entidades financieras españolas puedan identificar las posibilidades de desarrollo futuro de las situaciones de uso de dichas tarjetas, logrando un incremento de ingresos que compense la reducción de los mismos que se viene produciendo desde el año 2005, fruto de la reducción en las tasas de intercambio que se aplican en este negocio. Para ello se ha desarrollado una investigación sobre dichas entidades.The aim of this project is to analyze the current level of use of bank cards by Spanish consumers, as well as the reasoning behind it, in order to allow Spanish financial entities to identify possibilities for the future implementation of bank cards in new situations, thus allowing them to increase their revenues and counter the reduction that has characterized them since 2005, as a result of the fall in the interchange rates applied in this business. Research on these entities has been carried out to this end.post-print621 K

Severe congenital nephrogenic diabetes insipidus in a compound heterozygote with a new large deletion of the AQP2 gene: A case report

Background: Congenital nephrogenic diabetes insipidus (NDI) is a rare condition characterized by severe polyuria, due to the inability of the kidneys to concentrate urine in response to arginine vasopressin (AVP). In the majority of the cases, the disease shows an X‐linked inherited pattern, although an autosomal recessive inheritance was also observed. Methods: We report a patient with a severe NDI diagnosed during the neonatal period. Because the patient was female without a family history of congenital NDI, her disease was thought to exhibit an autosomal recessive form. Results: A full mutation analysis of AVP receptor 2 (AVPR2; MIM#300538) gene showed no mutations. However, direct Sanger sequencing of the aquaporin 2 (AQP2) revealed an apparently homozygous mutation at nucleotide position NM_000486.5:c.374C>T (p.Thr125Met) in exon 2. Further customized multiplex ligation‐dependent probe amplification (MLPA), single‐nucleotide polymorphism (SNP) array analysis, and long‐range polymerase chain reaction (PCR) followed by Sanger sequencing showed a heterozygous exonic deletion comprising exons 2, 3, and partially 4 of AQP2. Conclusion: This is the first case of a compound heterozygote patient with a missense mutation involving NM_000486.5:exon2:c.374C>T (p.Thr125Met) and a gross deletion of at least exons 2, 3, and partially 4 on the AQP2 to present with a severe NDI phenotypeThis study was supported in part by grants from the Research Activity Intensification Program (Programa Intensificación Actividad Investigadora) (IdiPAZ and Agencia Laín‐Entralgo/CM) to R.P. ISCIII RETIC REDINREN RD16/0009 FEDER FUNDS, and (Programa Intensificación Actividad Investigadora) (IdiPAZ and FIBHULP) to J.N

Intangible assets and business results of large companies

Purpose: To analyze the main types of intangible assets as sources of economic and social outcomes inlarge corporations.Design/methodology: We create a sample of large corporations with data from two secondarydatabases: Fortune Most Admired Companies and Interbrand. We study business’ legitimacy throughtheir presence on Google.com. We perform quantitative analysis by testing the proposed hypothesesagainst a structural equation model built using the PLS method. Findings: We confirm the value of brands and legitimacy as sources of economic outcomes. Weobserve that perceived quality and brand equity influence social results. We conclude that perceivedquality alone does not improve brand or market value.Research limitations/implications: The main limitation is related to the characteristics of thecompanies in the sample: large, highly institutionalized multinational corporations.Practical implications: The results offer several considerations for managers seeking to improveorganizational outcomes. Intangible assets must be managed along with tangible assets in order fororganizations to survive and thrive in the marketplace. Originality/value: The principal contribution of this study is to demonstrate the importance ofmanaging intangible assets so that the organization can maintain or improve its legitimacy and itseconomic outcomesPeer Reviewe

NUEVO ENFOQUE DEL NEGOCIO DE LAS TARJETAS BANCARIAS EN ESPAÑA Y EL NUEVO PAPEL A DESARROLLAR POR EL COMERCIANTE

El objetivo de este trabajo es analizar los elementos básicos del nuevo enfoque de las entidades financieras españolas para su negocio de tarjetas bancarias, así como el nuevo papel que podría tener el comerciante para su desarrollo. Para ello se envió un cuestionario a 60 responsables de este negocio en dichas entidades. De las respuestas recogidas en los 44 cuestionarios recibidos, se concluye que este nuevo enfoque deberá hacer más hincapié en las operaciones de financiación al consumo mediante tarjeta bancaria. Así mismo, se verifica que el comerciante deberá tener un papel relevante y retribuido como prescriptor de estas operaciones

Co-occurrence of neurofibromatosis type 1 and optic nerve gliomas with autosomal dominant polycystic kidney disease type 2

Background: Autosomal dominant polycystic kidney disease (ADPKD) and neurofibromatosis type 1 (NF1) are both autosomal dominant disorders with a high rate of novel mutations. However, the two disorders have distinct and well-delineated genetic, biochemical, and clinical findings. Only a few cases of coexistence of ADPKD and NF1 in a single individual have been reported, but the possible implications of this association are unknown. Methods: We report an ADPKD male belonging to a family of several affected members in three generations associated with NF1 and optic pathway gliomas. The clinical diagnosis of ADPKD and NF1 was performed by several image techniques. Results: Linkage analysis of ADPKD family was consistent to the PKD2 locus by a nonsense mutation, yielding a truncated polycystin-2 by means of next-generation sequencing. The diagnosis of NF1 was confirmed by mutational analysis of this gene showing a 4-bp deletion, resulting in a truncated neurofibromin, as well. The impact of this association was investigated by analyzing putative genetic interactions and by comparing the evolution of renal size and function in the proband with his older brother with ADPKD without NF1 and with ADPKD cohorts. Conclusion: Despite the presence of both conditions there was not additive effect of NF1 and PKD2 in terms of the severity of tumor development and/or ADPKD progression.This study was financed in part by the Instituto de Salud Carlos III, the Ministerio de Ciencia y Innovación (EC08/00236) and the program for intensifying research activities (IdiPAZ and Agencia Lain Entralgo/CM) to R.P. or the program for intensifying (IdiPAZ and FIBHULP) to J.N. NF1 studies are supported by grants from Fundación Mutua Madrileña de Investigación Biomédica (FMM) and Asociación Española de Afectados de Neurofibromatosis. ISCIII RETIC REDINREN RD16/0009 FEDER Fund

An error based mathematical module to enhance learning in signals and systems

During the last years, the lecturers at the Circuits and Systems Engineering Department at the E.U.I.T. de Telecomunicación at the Universidad Politécnica de Madrid are observing more and more serious mathematical errors in the different exams and exercises taken by the students. Although some of these mistakes can be considered unacceptable in engineering disciplines, it is possible for a student to pass the final exam regardless of these mistakes. In this scenario, and aware that results were getting worse and worse year after year, it was considered convenient, and almost indispensable, to develop math exercises that students must practice if they want to progress following a continuous and formative assessment method along their engineering studies. The first part of this work is to analyze basic mathematical errors in final exam exercises of the course “Signals and Systems”. We present and illustrate the most relevant errors detected during the last two years final exams of that course. The information obtained permits us to identify the main lacks, difficulties and defaults of the students. The second part of this work is to develop a training module in order to the students can practice as many times as they want with simple exercises dealing with the topics where frequent errors are detected. After practicing they must pass an initial test to make sure that students have acquired the adequate basic mathematical background and skills to progress successfully in the mentioned course. The questions and exercises have been written using different formats, most of them to be compatible with Moodle platform requirements

Reading virtual slide using web viewers: results of subjective experience with three different solutions

<p>Abstract</p> <p>Background</p> <p>Virtual slides are viewed using interactive software that enables the user to simulate the behaviour of a conventional optical microscope, like adjusting magnifications and navigating to any portion of the image. Nowadays, information about the performance and features of web-based solutions for reading slides in real environments is still scarce. The objective of this study is analyzing the subjective experience of pathologists with virtual slides, comparing the time needed to read slides using different web viewers and different network connections.</p> <p>Methods</p> <p>Eight slides were randomly selected (4 biopsies and 2 cytologies) from Hospital General de Ciudad Real (HGCR) archives. Three different virtual slide web-viewing solutions were analyzed: Aperio web server, Olympus NetImage Server, and Aurora mScope. Five pathologists studied to time needed to access images of each virtual slide, selecting a panoramic view, 10 low magnification fields, and 20 high magnification fields.</p> <p>Results</p> <p>Aperio viewer is very efficient in overview images. Aurora viewer is especially efficient in lower magnifications (10×). For larger magnifications (20× and 40×) no significant differences were found between different vendors. Olympus was found to be the most user-friendly interface. When comparing Internet with intranet connections, despite being slower, users also felt comfortable using virtual slides through Internet connection.</p> <p>Conclusion</p> <p>Available web solutions for virtual slides have different advantages, mainly in functionalities and optimization for different magnifications. Pathologist should select the solutions adapted to their needs.</p