Análisis de la presencia en internet de castillos en la provincia de Valencia

[ES] El siguiente estudio hace análisis de la presencia en internet de los diferentes castillos de la provincia de Valencia. Este estudio se ha realizado en base a una lista extraída de Tripadvisor, la cual está ordenada según la opinión de los viajeros. Tras la definición de las variables más adecuadas, se realiza la elección de la muestra. Esa lista de castillos en la que se va a basar el estudio. Para finalizar según los resultados obtenidos, se realiza una serie de consejos de mejora que se consideren oportunos. Se ha llegado a la conclusión de que seria conveniente poder mostrar las webs en más idiomas con precaución de no crear falsas expectativas, crear un buzón de sugerencias donde los visitantes puedan dejar sus opiniones y propuestas de mejora, por ultimo deberían de hacer más uso de las redes sociales ya que se trata de una herramienta de promoción muy buena.[EN] The following study analyses the presence on the internet of the different castles in the province of Valencia. This study has been made based on a list drawn from Tripadvisor, which is ordered according to the opinion of travellers. After defining the most appropriate variables, the sample is chosen. That list of castles on which the study will be based. Finally, according to the results obtained, a series of improvement tips are considered as appropriate. It has been concluded that it would be convenient to be able to display the websites in more languages with caution not to create false expectations, create a suggestion box where visitors can leave their opinions and proposals for improvement and finally should make more use of social networks since it is a very good promotion tool.Seguí Torregrosa, JV. (2019). Análisis de la presencia en internet de castillos en la provincia de Valencia. Universitat Politècnica de València. http://hdl.handle.net/10251/130195TFG

Fabry Nephropathy: An Evidence-Based Narrative Review.

Fabry disease (FD) is a rare, X-linked disorder caused by mutations in the GLA gene encoding the enzyme α-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, endothelial cells, epithelial cells, and tubular cells contribute to the renal symptoms of FD, which manifest as proteinuria and reduced glomerular filtration rate leading to renal insufficiency. A correct diagnosis of FD, although challenging, has considerable implications regarding treatment, management, and counseling. The diagnosis may be confirmed by demonstrating the enzyme deficiency in males and by identifying the specific GLA gene mutation in male and female patients. Treatment with enzyme replacement therapy, as part of the therapeutic strategy to prevent complications of the disease, may be beneficial in stabilizing renal function or slowing its decline, particularly in the early stages of the disease. Emergent treatments for FD include the recently approved chaperone molecule migalastat for patients with amenable mutations. The objective of this report is to provide an updated overview on Fabry nephropathy, with a focus on the most relevant aspects of its epidemiology, diagnosis, pathophysiology, and treatment options.S

Adiciones y correcciones a la orquidoflora valenciana, VII

Se aportan datos sobre algunos táxones de Orchidaceae que resultan escasos en la Comunidad Valenciana o en determinadas de sus comarcas; a destacar la presencia de Ophrys santonica y O. × pseudospeculum en Alicante.It is shown some data about rare taxa of Orchidaceae at the Valencian Community (E Spain) or expansions of area to new shires; to emphasize the presence of Ophrys santonica and O. × pseudospeculum in Alicante

Osteoporosis, bone mineral density and CKD–MBD complex (I): Diagnostic considerations

Osteoporosis (OP) and chronic kidney disease (CKD) independently influence bone and cardiovascular health. A considerable number of patients with CKD, especially those with stages 3a to 5D, have a significantly reduced bone mineral density leading to a high risk of fracture and a significant increase in associated morbidity and mortality. Independently of classic OP related to age and/or gender, the mechanical properties of bone are also affected by inherent risk factors for CKD (“uraemic OP”). In the first part of this review, we will analyse the general concepts regarding bone mineral density, OP and fractures, which have been largely undervalued until now by nephrologists due to the lack of evidence and diagnostic difficulties in the context of CKD. It has now been proven that a reduced bone mineral density is highly predictive of fracture risk in CKD patients, although it does not allow a distinction to be made between the causes which generate it (hyperparathyroidism, adynamic bone disease and/or senile osteoporosis, etc.). Therefore, in the second part, we will analyse the therapeutic indications in different CKD stages. In any case, the individual assessment of factors which represent a higher or lower risk of fracture, the quantification of this risk (i.e. using tools such as FRAX®) and the potential indications for densitometry in patients with CKD could represent an important first step pending new clinical guidelines based on randomised studies which do not exclude CKD patients, all the while avoiding therapeutic nihilism in an area of growing importance. Resumen: Osteoporosis (OP) y enfermedad renal crónica (ERC) influyen de manera independiente en la salud ósea y cardiovascular. Un número significativo de pacientes con ERC, especialmente desde estadios 3a a 5D, presentan una disminución significativa de la densidad mineral ósea condicionando un alto riesgo de fractura y un incremento importante de la morbimortalidad asociada. Independientemente de la OP clásica asociada a edad y/o sexo, las propiedades mecánicas del hueso se encuentran afectadas adicionalmente por factores intrínsecos a la ERC («OP urémica»). En la primera parte de esta revisión, analizaremos conceptos generales sobre densidad mineral ósea, OP y fracturas, en gran parte infravalorados hasta ahora por los nefrólogos debido a la falta de evidencias y a las dificultades diagnósticas en el contexto de la ERC. Actualmente se ha demostrado que una densidad mineral ósea disminuida es realmente predictiva del riesgo de fracturas en pacientes con ERC, aunque no permite distinguir entre las causas que la originan (hiperparatiroidismo, enfermedad adinámica del hueso y/o osteoporosis senil, etc.). Por ello, en la segunda parte analizaremos las implicaciones terapéuticas en distintos estadios de la ERC. En cualquier caso, la valoración individualizada de los factores mayores y menores del riesgo de fractura, la cuantificación de dicho riesgo (i.e. con el uso de herramientas como el FRAX®) y las indicaciones potenciales de densitometría en pacientes con ERC podrían constituir un primer paso importante en espera de nuevas guías clínicas basadas en estudios aleatorizados que no excluyan a pacientes con ERC, evitando mientras tanto nihilismo terapéutico en un área de creciente importancia. Keywords: Osteoporosis, CKD–MBD, Bone mineral density, Fractures, FRAX, Chronic kidney disease, DEXA, Palabras clave: Osteoporosis, CKD-MBD, Densidad mineral ósea, Fracturas, FRAX, Enfermedad renal crónica, DEX

Osteoporosis, densidad mineral ósea y complejo CKD-MBD (I): consideraciones diagnósticas

Resumen: Osteoporosis (OP) y enfermedad renal crónica (ERC) influyen de manera independiente en la salud ósea y cardiovascular. Un número significativo de pacientes con ERC, especialmente desde estadios 3a a 5D, presentan una disminución significativa de la densidad mineral ósea condicionando un alto riesgo de fractura y un incremento importante de la morbimortalidad asociada. Independientemente de la OP clásica asociada a edad y/o sexo, las propiedades mecánicas del hueso se encuentran afectadas adicionalmente por factores intrínsecos a la ERC («OP urémica»). En la primera parte de esta revisión, analizaremos conceptos generales sobre densidad mineral ósea, OP y fracturas, en gran parte infravalorados hasta ahora por los nefrólogos debido a la falta de evidencias y a las dificultades diagnósticas en el contexto de la ERC. Actualmente se ha demostrado que una densidad mineral ósea disminuida es realmente predictiva del riesgo de fracturas en pacientes con ERC, aunque no permite distinguir entre las causas que la originan (hiperparatiroidismo, enfermedad adinámica del hueso y/o osteoporosis senil, etc.). Por ello, en la segunda parte analizaremos las implicaciones terapéuticas en distintos estadios de la ERC. En cualquier caso, la valoración individualizada de los factores mayores y menores del riesgo de fractura, la cuantificación de dicho riesgo (i.e. con el uso de herramientas como el FRAX®) y las indicaciones potenciales de densitometría en pacientes con ERC podrían constituir un primer paso importante en espera de nuevas guías clínicas basadas en estudios aleatorizados que no excluyan a pacientes con ERC, evitando mientras tanto nihilismo terapéutico en un área de creciente importancia. Abstract: Osteoporosis (OP) and chronic kidney disease (CKD) independently influence bone and cardiovascular health. A considerable number of patients with CKD, especially those with stages 3a to 5D, have a significantly reduced bone mineral density leading to a high risk of fracture and a significant increase in associated morbidity and mortality. Independently of classic OP related to age and/or gender, the mechanical properties of bone are also affected by inherent risk factors for CKD (“uraemic OP”). In the first part of this review, we will analyse the general concepts regarding bone mineral density, OP and fractures, which have been largely undervalued until now by nephrologists due to the lack of evidence and diagnostic difficulties in the context of CKD. It has now been proven that a reduced bone mineral density is highly predictive of fracture risk in CKD patients, although it does not allow a distinction to be made between the causes which generate it (hyperparathyroidism, adynamic bone disease and/or senile osteoporosis, etc.). Therefore, in the second part, we will analyse the therapeutic indications in different CKD stages. In any case, the individual assessment of factors which represent a higher or lower risk of fracture, the quantification of this risk (i.e. using tools such as FRAX®) and the potential indications for densitometry in patients with CKD could represent an important first step pending new clinical guidelines based on randomised studies which do not exclude CKD patients, all the while avoiding therapeutic nihilism in an area of growing importance. Palabras clave: Osteoporosis, CKD-MBD, Densidad mineral ósea, Fracturas, FRAX, Enfermedad renal crónica, DEXA, Keywords: Osteoporosis, CKD-MBD, Bone mineral density, Fractures, FRAX, Chronic kidney disease, DEX

Fabry nephropathy: an evidence-based narrative review

Fabry disease (FD) is a rare, X-linked disorder caused by mutations in the GLA gene encoding the enzyme α-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, endothelial cells, epithelial cells, and tubular cells contribute to the renal symptoms of FD, which manifest as proteinuria and reduced glomerular filtration rate leading to renal insufficiency. A correct diagnosis of FD, although challenging, has considerable implications regarding treatment, management, and counseling. The diagnosis may be confirmed by demonstrating the enzyme deficiency in males and by identifying the specific GLA gene mutation in male and female patients. Treatment with enzyme replacement therapy, as part of the therapeutic strategy to prevent complications of the disease, may be beneficial in stabilizing renal function or slowing its decline, particularly in the early stages of the disease. Emergent treatments for FD include the recently approved chaperone molecule migalastat for patients with amenable mutations. The objective of this report is to provide an updated overview on Fabry nephropathy, with a focus on the most relevant aspects of its epidemiology, diagnosis, pathophysiology, and treatment options

Fabry Nephropathy: An Evidence-based Narrative Review

Fabry disease (FD) is a rare, X-linked disorder caused by mutations in the GLA gene encoding the enzyme alpha-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, endothelial cells, epithelial cells, and tubular cells contribute to the renal symptoms of FD, which manifest as proteinuria and reduced glomerular filtration rate leading to renal insufficiency. A correct diagnosis of FD, although challenging, has considerable implications regarding treatment, management, and counseling. The diagnosis may be confirmed by demonstrating the enzyme deficiency in males and by identifying the specific GLA gene mutation in male and female patients. Treatment with enzyme replacement therapy, as part of the therapeutic strategy to prevent complications of the disease, may be beneficial in stabilizing renal function or slowing its decline, particularly in the early stages of the disease. Emergent treatments for FD include the recently approved chaperone molecule migalastat for patients with amenable mutations. The objective of this report is to provide an updated overview on Fabry nephropathy, with a focus on the most relevant aspects of its epidemiology, diagnosis, pathophysiology, and treatment options. (C) 2018 The Author(s) Published by S. Karger AG, Basel

Historia-historias de la lectura : XXIV Jornadas de Estudios Históricos Locales - XVII Jornadas de Historia de la Educación de los Países de Lengua Catalan

En el título, los términos 'Història' e 'Històries' están separados por una barra inclinadaLa temática de las jornadas se centra en el análisis de la dimensión educativa de la lectura a lo largo de la historia, y se desarrolla alrededor de los siguientes ejes temáticos: enseñar y aprender a leer; espacios y contextos para la lectura y su fomento; las lecturas educativas y la lectura y la educación como a elementos de debate y reflexión.La temàtica de les jornades se centra en l'anàlisi de la dimensió educativa de la lectura al llarg de la història, i es desenvolupa al voltant dels eixos temàtics següents: ensenyar i aprendre a llegir; espais i contextos per a la lectura i el seu foment; les lectures educatives i la lectura i l'educació com a elements de debat i reflexió.BalearesUniversitat de les Illes Balears. Redined Balears; Edifici Guillem Cifre de Colonya. Ctra. de Valldemossa, Km 7,5; 07122 Palma de Mallorca; +34971172792; +34971173190; [email protected]