Funciones básicas y competencias del orientador para una educación de calidad

El presente proyecto, ha sido elaborado a partir de dos trabajos desarrollados en el Máster de Profesorado de Secundaria, en la especialidad de Orientación Educativa. Uno de ellos, dedicado a establecer las funciones que realiza el orientador en el centro educativo, y el otro, que muestra las competencias que un orientador debe tener y llevar a cabo en su práctica diaria. El objetivo de este Trabajo Fin de Máster, es mostrar desde un marco teórico y legal, las funciones que realiza el orientador en sus tareas diarias y las competencias que debe tener para una educación de calidad, propuestas por la Asociación Internacional de Orientación Educativa y Profesional, y el Proyecto Tuning. De la misma manera, se expone un estudio que muestra la importancia de las competencias profesionales actitudinales que se llevan a cabo en los centros de la Comunidad Autónoma de Galicia, y las que se deberían adoptar

La opinión del auditor y la contabilidad creativa: una aplicación al contexto español

El objetivo de este trabajo es analizar en qué medida la opinión que manifiesta el auditor en el informe de auditoría, referido a las cuentas consolidadas de grupos de empresas españolas, está relacionada con la contabilidad creativa, medida a través de los ajustes por devengo discrecionales. Para ello, hemos clasificado las salvedades e incertidumbres expresadas en los informes de auditoría en dos categorías: las relacionadas con el principio de empresa en funcionamiento y las que se derivan de otros motivos diferentes a este principio. Los resultados indican que para el primer caso, la opinión del auditor no se asocia con la manipulación contable; sin embargo, para el segundo tipo de salvedades sí que existe relación entre la opinión del auditor y la contabilidad creativa. Además, se observa que la rentabilidad, el resultado del ejercicio anterior, la situación de concurso de acreedores y el sector donde opera la empresa son factores determinantes de la opinión del auditor acerca de la continuidad de las empresas. En la decisión de los auditores de emitir opiniones relacionadas con otras razones influyen, entre otras cosas, la rentabilidad de la empresa y el sector en el que opera ésta

Mitochondrial aconitase enzymatic activity: a potential long-term survival biomarker in the blood of ALS patients

Background: Amyotrophic lateral sclerosis (ALS) is a multisystemic, progressive, neurodegenerative disorder. Despite it being generally fatal within a period of 2–4 years, it is highly heterogeneous; as a result, survival periods may vary greatly among individual patients. Biomarkers can serve as tools for diagnosis, prognosis, indicators of therapeutic response, and future therapeutics. Free-radical-dependent mitochondrial damage is believed to play a crucial role in neurodegeneration in ALS. Mitochondrial aconitase, which is also known as aconitase 2 (Aco2), is a key Krebs cycle enzyme and is involved in the regulation of cellular metabolism and iron homeostasis. Aco2 is very sensitive to oxidative inactivation and can aggregate and accumulate in the mitochondrial matrix, causing mitochondrial dysfunction. Loss of Aco2 activity may therefore reflect increased levels of mitochondrial dysfunction due to oxidative damage and could be relevant to ALS pathogenesis. The aim of our study was to confirm changes in mitochondrial aconitase activity in peripheral blood and to determine whether such changes are dependent on, or independent of, the patient’s condition and to propose the feasibility of using them as possible valid biomarkers to quantify the progression of the disease and as a predictor of individual prognosis in ALS. Methods: We measured the Aco2 enzymatic activity in the platelets of blood samples taken from 22 controls and 26 ALS patients at different stages of disease development. We then correlated antioxidant activity with clinical and prognostic variables. Results: Aco2 activity was significantly lower in the 26 ALS patients than in the 22 controls (p < 0.05). Patients with higher levels of Aco2 activity survived longer than those with lower levels (p < 0.05). Aco2 activity was also higher in patients with earlier onset (p < 0.05) and in those with predominantly upper motor neuron signs. Conclusions: Aco2 activity seems to be an independent factor that could be used in the long-term survival prognosis of ALS. Our findings suggest that blood Aco2 could be a leading candidate for use as a biomarker to improve prognosis. More studies are needed to confirm these results

DREAM-dependent activation of astrocytes in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of unknown origin and characterized by a relentless loss of motor neurons that causes a progressive muscle weakness until death. Among the several pathogenic mechanisms that have been related to ALS, a dysregulation of calcium-buffering proteins in motor neurons of the brain and spinal cord can make these neurons more vulnerable to disease progression. Downstream regulatory element antagonist modulator (DREAM) is a neuronal calcium-binding protein that plays multiple roles in the nucleus and cytosol. The main aim of this study was focused on the characterization of DREAM and glial fibrillary acid protein (GFAP) in the brain and spinal cord tissues from transgenic SOD1G93A mice and ALS patients to unravel its potential role under neurodegenerative conditions. The DREAM and GFAP levels in the spinal cord and different brain areas from transgenic SOD1G93A mice and ALS patients were analyzed by Western blot and immunohistochemistry. Our findings suggest that the calcium-dependent excitotoxicity progressively enhanced in the CNS in ALS could modulate the multifunctional nature of DREAM, strengthening its apoptotic way of action in both motor neurons and astrocytes, which could act as an additional factor to increase neuronal damage. The direct crosstalk between astrocytes and motor neurons can become vulnerable under neurodegenerative conditions, and DREAM could act as an additional switch to enhance motor neuron loss. Together, these findings could pave the way to further study the molecular targets of DREAM to find novel therapeutic strategies to fight ALS

Collagen XIX Alpha 1 improves prognosis in amyotrophic lateral sclerosis

The identification of more reliable diagnostic or prognostic biomarkers in age-related neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS), is urgently needed. The objective in this study was to identify more reliable prognostic biomarkers of ALS mirroring neurodegeneration that could be of help in clinical trials. A total of 268 participants from three cohorts were included in this study. The muscle and blood cohorts were analyzed in two cross-sectional studies, while the serial blood cohort was analyzed in a longitudinal study at 6-monthly intervals. Fifteen target genes and fourteen proteins involved in muscle physiology and differentiation, metabolic processes and neuromuscular junction dismantlement were studied in the three cohorts. In the muscle biopsy cohort, the risk for a higher mortality in an ALS patient that showed high Collagen type XIX, alpha 1 (COL19A1) protein levels and a fast progression of the disease was 70.5% (P < 0.05), while in the blood cohort, this risk was 20% (P < 0.01). In the serial blood cohort, the linear mixed model analysis showed a significant association between increasing COL19A1 gene levels along disease progression and a faster progression during the follow-up period of 24 months (P < 0.05). Additionally, higher COL19A1 levels and a faster progression increased 17.9% the mortality risk (P < 0.01). We provide new evidence that COL19A1 can be considered a prognostic biomarker that could help the selection of homogeneous groups of patients for upcoming clinical trial and may be pointed out as a promising therapeutic target in ALS

Síndrome de Guillain-Barré: Características epidemiológicas, inmunológicas y clínico-evolutivas de una serie hospitalaria.

1. RESUMEN 1.1 Introducción El Síndrome de Guillain-Barré (SGB) es una poliradiculoneuropatía inflamatoria de características autoinmunes, caracterizada por la presencia de parálisis fláccida con arreflexia, parestesias y dolor. Los mecanismos fisiopatológicos conducen a un daño desmielinizante, axonal o axono-desmielinizante. El diagnóstico de la enfermedad es clínico, pero el estudio de LCR y las pruebas neurofisiológicas apoyan el diagnóstico con mayor especificidad. 1.2 Metodología Estudio descriptivo , observacional, y retrospectivo de las historias clínicas de una serie de pacientes diagnosticados de SGB en el Hospital Clínico Universitario de Zaragoza, desde enero de 2009 hasta diciembre de 2016, y con seguimiento posterior en consultas de neurología. 1.3 Resultados Observamos una incidencia anual similar a la esperada, con predominio estacional en invierno y en periodos post-vacunales coincidentes con la pandemia de gripe A. Es destacable el predominio femenino, el elevado porcentaje de formas sensitivas con la variante Miller Fisher, así como la presencia especifica de anticuerpos AntiGQ1B. Con excelente respuesta a tratamiento inmunomodulador, destacamos una evidente resolución del déficit funcional y mínima sintomatología residual. Encontramos como factor pronóstico con significación estadística, la disminución de amplitud motora en estudios neurofisiológicos, que destaca como marcador de daño axonal. 1.4 Conclusiones Las características encontradas son similares a las descritas en la literatura, lo cual otorga de mayor validez al estudio. Palabras clave: Polineuropatías agudas autoinmunes. Síndrome Guillain-Barré. Epidemiología Síndrome Guillain-Barré. Estudios neurofisiológicos en polineuropatías autoinmunes. Estudios inmunológicos en polineuropatías autoinmunes. Características clínico-evolutivas. Inmunoterapia.<br /

Severe infantile-onset cardiomyopathy associated with a homozygous deletion in desmin

Desminopathy is a genetically heterogeneous disorder with autosomal dominant pattern of inheritance in most affected families; the age of disease onset is on average 30 years. We studied a patient with a history of recurrent episodes of syncope from infancy who later developed second-degree AV block and restrictive cardiomyopathy; she subsequently suffered several episodes of ventricular tachyarrhythmia requiring implantation of bicameral defibrillator. Neurological examination revealed rapidly progressive bilateral facial weakness, winging of the scapulae, symmetric weakness and atrophy of the trunk muscles, shoulder girdle and distal muscles of both upper and lower extremities. Muscle biopsy demonstrated signs of myofibrillar myopathy with prominent subsarcolemmal desmin-reactive aggregates. Molecular analysis identified a homozygous deletion in DES resulting in a predicted in-frame obliteration of seven amino acids (p.R173_E179del) in the 1B domain of desmin. We describe the youngest known desminopathy patient with severe cardiomyopathy and aggressive course leading to the devastation of cardiac, skeletal and smooth musculature at an early age

Severe infantile-onset cardiomyopathy associated with a homozygous deletion in desmin

Desminopathy is a genetically heterogeneous disorder with autosomal dominant pattern of inheritance in most affected families; the age of disease onset is on average 30 years. We studied a patient with a history of recurrent episodes of syncope from infancy who later developed second-degree AV block and restrictive cardiomyopathy; she subsequently suffered several episodes of ventricular tachyarrhythmia requiring implantation of bicameral defibrillator. Neurological examination revealed rapidly progressive bilateral facial weakness, winging of the scapulae, symmetric weakness and atrophy of the trunk muscles, shoulder girdle and distal muscles of both upper and lower extremities. Muscle biopsy demonstrated signs of myofibrillar myopathy with prominent subsarcolemmal desmin-reactive aggregates. Molecular analysis identified a homozygous deletion in DES resulting in a predicted in-frame obliteration of seven amino acids (p.R173_E179del) in the 1B domain of desmin. We describe the youngest known desminopathy patient with severe cardiomyopathy and aggressive course leading to the devastation of cardiac, skeletal and smooth musculature at an early age.</p

Type XIX collagen: a promising biomarker from the basement membranes

Among collagen members in the collagen superfamily, type XIX collagen has raised increasing interest in relation to its structural and biological roles. Type XIX collagen is a Fibril-Associated Collagen with Interrupted Triple helices member, one main subclass of collagens in this superfamily. This collagen contains a triple helix composed of three polypeptide segments aligned in parallel and it is associated with the basement membrane zone in different tissues. The molecular structure of type XIX collagen consists of five collagenous domains, COL1 to COL5, interrupted by six non-collagenous domains, NC1 to NC6. The most relevant domain by which this collagen exerts its biological roles is NC1 domain that can be cleavage enzymatically to release matricryptins, exerting anti-tumor and anti-angiogenic effect in murine and human models of cancer. Under physiological conditions, type XIX collagen expression decreases after birth in different tissues although it is necessary to keep its basal levels, mainly in skeletal muscle and hippocampal and telencephalic interneurons in brain. Notwithstanding, in amyotrophic lateral sclerosis, altered transcript expression levels show a novel biological effect of this collagen beyond its structural role in basement membranes and its anti-tumor and anti-angiogenic properties. Type XIX collagen can exert a compensatory effect to ameliorate the disease progression under neurodegenerative conditions specific to amyotrophic lateral sclerosis in transgenic SOD1G93A mice and amyotrophic lateral sclerosis patients. This novel biological role highlights its nature as prognostic biomarker of disease progression in and as promising therapeutic target, paving the way to a more precise prognosis of amyotrophic lateral sclerosis