7 research outputs found
Una panorámica de la ganadería industrial en el Estado español
En el Estado español el número de cabezas de ganado no deja de crecer, pero cada vez hay menos personas dedicadas a la ganadería. La industria cárnica ha convertido la ganadería en una actividad industrial más: deslocalizada, desligada del entorno y altamente dependiente de insumos externos. La parte productora está a merced de una industria que impone los precios y modos de producción mientras que la consumidora paga un alto precio en salud y medio ambiente por adquirir carne y productos lácteos baratos
Association of metreleptin treatment and dietary intervention with neurological outcomes in Celia's encephalopathy
Celia’s encephalopathy (progressive encephalopathy with/without lipodystrophy, PELD) is a recessive neurodegenerative disease that is fatal in childhood. It is caused by a c.985C>T variant in the BSCL2/seipin gene that results in an aberrant seipin protein. We evaluated neurological development before and during treatment with human recombinant leptin (metreleptin) plus a dietary intervention rich in polyunsaturated fatty acids (PUFA) in the only living patient. A 7 years and 10 months old girl affected by PELD was treated at age 3 years with metreleptin, adding at age 6 omega-3 fatty acid supplementation. Her mental age was evaluated using the Battelle Developmental Inventory Screening Test (BDI), and brain PET/MRI was performed before treatment and at age 5, 6.5, and 7.5 years. At age 7.5 years, the girl remains alive and leads a normal life for her mental age of 30 months, which increased by 4 months over the last 18 months according to BDI. PET images showed improved glucose uptake in the thalami, cerebellum, and brainstem. This patient showed a clear slowdown in neurological regression during leptin replacement plus a high PUFA diet. The aberrant BSCL2 transcript was overexpressed in SH-SY5Y cells and was treated with docosahexaenoic acid (200 µM) plus leptin (0.001 mg/ml) for 24 h. The relative expression of aberrant BSCL2 transcript was measured by qPCR. In vitro studies showed significant reduction (32%) in aberrant transcript expression. This therapeutic approach should be further studied in this devastating disease.This work was supported by the Fundación Mutua Madrileña and by the Instituto de Salud Carlos III and the European Regional Development Fund, FEDER (PI13/00314).S
A new seipin-associated neurodegenerative syndrome
Background: Seipin/BSCL2 mutations can cause type
2 congenital generalised lipodystrophy (BSCL) or
dominant motor neurone diseases. Type 2 BSCL is
frequently associated with some degree of intellectual
impairment, but not to fatal neurodegeneration. In order
to unveil the aetiology and pathogenetic mechanisms of
a new neurodegenerative syndrome associated with a
novel BSCL2 mutation, six children, four of them
showing the BSCL features, were studied.
Methods: Mutational and splicing analyses of BSCL2
were performed. The brain of two of these children was
examined postmortem. Relative expression of BSCL2
transcripts was analysed by real-time reverse
transcription-polymerase chain reaction (RT-PCR) in
different tissues of the index case and controls.
Overexpressed mutated seipin in HeLa cells was analysed
by immunofluorescence and western blotting.
Results: Two patients carried a novel homozygous
c.985C>T mutation, which appeared in the other four
patients in compound heterozygosity. Splicing analysis
showed that the c.985C>T mutation causes an aberrant
splicing site leading to skipping of exon 7. Expression of
exon 7-skipping transcripts was very high with respect to
that of the non-skipped transcripts in all the analysed
tissues of the index case. Neuropathological studies
showed severe neurone loss, astrogliosis and intranuclear
ubiquitin(+) aggregates in neurones from multiple
cortical regions and in the caudate nucleus.
Conclusions: Our results suggest that exon 7 skipping
in the BSCL2 gene due to the c.985C>T mutation is
responsible for a novel early onset, fatal
neurodegenerative syndrome involving cerebral cortex
and basal ganglia.Instituto de Salud Carlos III (grant number PI 10/02873) and European
Regional Development Fund, FEDER (grant number 10PXIB208013PR) and
Consellería de Industria, Xunta de Galicia.S
No molestar, se negocia (lo que vas a comer) : el tratado de libre comercio entre Europa y los Estados Unidos
Crece la oposición de organizaciones campesinas, sociales y ecologistas así como de sindicatos de varios países europeos y de EEUU a un peligroso tratado que generaría más desempleo y supondría un ataque sin precedentes a los derechos sociales y la democracia, privatizando aún más los servicios públicos, como la salud y el agua. Además las negociaciones tienen como objetivo bajar los salarios y eliminar los niveles de protección todavía existentes en amplias áreas, como en la salud pública, el medio ambiente y los derechos laborales. Y desde luego, las negociaciones secretas del Tratado Transatlántico de Comercio e Inversiones entre EEUU y la UE, son una grave amenaza para la Soberanía Alimentaria, como veremos en este artículo
Pla d’acció educativa per a l’atenció d’un infant amb altes capacitats: tutoria entre iguals
Des del punt de vista d’escola inclusiva, són igual d’importants els alumnes que tenen
dificultats per seguir el ritme com aquells que se senten fora del que l’escola proporciona.
L’alumnat amb altes capacitats no sol mostrar mancances en la participació,
l’aprenentatge i l’èxit i, per tant, sovint rep intervenció específica però passa
desapercebut dins de l’aula ordinària. Si no s’intervé de forma adequada, pot mostrar
factors de risc com l’avorriment a l’aula, poca motivació vers l’aprenentatge, desajustos
emocionals i socials i, fins i tot, baix rendiment acadèmic.
A partir de les necessitats detectades mitjançant l’estudi d’un infant amb AC i la interacció
amb la seva realitat actual, s’ha dissenyat un pla d’acció com a mesura universal,
destinada al 100% de l’alumnat, per millorar la resposta educativa dins de l’aula ordinària.
El resultat ha estat l’aplicació de la tutoria entre iguals a una aula de 6è de primària i la
posterior valoració del procés, en general, i de l’impacte a les necessitats específiques de
l’alumna.From the point of view of inclusive education, students who have difficulty keeping up
with the rhythm of the classroom are as important as students who do not feel satisfied
with what school provides. Gifted pupils do not often show a lack of participation, learning
and success and, therefore, they often receive specific intervention but go unnoticed in
the classroom. If the intervention is not appropriate, they can show risk factors as
boredom at class, little motivation towards learning, emotional and social imbalances and
even a low academic performance.
Based on the needs identified through the study of a gifted child and the interaction with
her current reality, it has been designed an action plan as a universal measure, aimed at
100% of the students, to improve educational response in the classroom.
The result has consisted in the implementation of a peer tutoring at a 6th grade classroom
and the following evaluation of the process, in general and of the impact on the specific
needs of the gifted student