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Background Hereditary fructose intolerance (HFI) is a rare inborn error of fructose metabolism caused by the deficiency of aldolase B. Since treatment consists of a fructose-, sucrose- and sorbitol-restrictive diet for life, patients are at risk of presenting vitamin deficiencies. Although there is no published data on the status of these vitamins in HFI patients, supplementation with vitamin C and folic acid is common. Therefore, the aim of this study was to assess vitamin C and folate status and supplementation practices in a nationwide cohort of HFI patients. Methods Vitamin C and folic acid dietary intake, supplementation and circulating levels were assessed in 32 HFI patients and 32 age- and sex-matched healthy controls. Results Most of the HFI participants presented vitamin C (96.7%) and folate (90%) dietary intake below the recommended population reference intake. Up to 69% received vitamin C and 50% folic acid supplementation. Among HFI patients, 15.6% presented vitamin C and 3.1% folate deficiency. The amount of vitamin C supplementation and plasma levels correlated positively (R = 0.443; p = 0.011). Interestingly, a higher percentage of non-supplemented HFI patients were vitamin C deficient when compared to supplemented HFI patients (30% vs. 9.1%; p = 0.01) and to healthy controls (30% vs. 3.1%; p < 0.001). Conclusions Our results provide evidence for the first time supporting vitamin C supplementation in HFI. There is great heterogeneity in vitamin supplementation practices and, despite follow-up at specialised centres, vitamin C deficiency is common. Further research is warranted to establish optimal doses of vitamin C and the need for folic acid supplementation in HFI.

Hereditary Fructose Intolerance (HFI) is an autosomal recessive inborn error of metabolism characterised by the deficiency of the hepatic enzyme aldolase B. Its treatment consists in adopting a fructose-, sucrose-, and sorbitol (FSS)-restrictive diet for life. Untreated HFI patients present an abnormal transferrin (Tf) glycosylation pattern due to the inhibition of mannose-6-phosphate isomerase by fructose-1-phosphate. Hence, elevated serum carbohydrate-deficient Tf (CDT) may allow the prompt detection of HFI. The CDT values improve when an FSS-restrictive diet is followed; however, previous data on CDT and fructose intake correlation are inconsistent. Therefore, we examined the complete serum sialoTf profile and correlated it with FSS dietary intake and with hepatic parameters in a cohort of paediatric and adult fructosemic patients. To do so, the profiles of serum sialoTf from genetically diagnosed HFI patients on an FSS-restricted diet (n = 37) and their age-, sex- and body mass index-paired controls (n = 32) were analysed by capillary zone electrophoresis. We found that in HFI patients, asialoTf correlated with dietary intake of sucrose (R = 0.575, p < 0.001) and FSS (R = 0.475, p = 0.008), and that pentasialoTf+hexasialoTf negatively correlated with dietary intake of fructose (R = −0.386, p = 0.024) and FSS (R = −0.400, p = 0.019). In addition, the tetrasialoTf/disialoTf ratio truthfully differentiated treated HFI patients from healthy controls, with an area under the ROC curve (AUROC) of 0.97, 92% sensitivity, 94% specificity and 93% accuracy.

Mucopolysaccharidosis type IVA (MPS IVA) is a lysosomal storage disease caused by mutations in the N-acetylgalactosamine-6-sulfatase (GALNS) gene. Skeletal dysplasia and the related clinical features of MPS IVA are caused by disruption of the cartilage and its extracellular matrix, leading to a growth imbalance. Enzyme replacement therapy (ERT) with recombinant human GALNS has yielded positive results in activity of daily living and endurance tests. However, no data have demonstrated improvements in bone lesions and bone grow thin MPS IVA after ERT, and there is no correlation between therapeutic efficacy and urine levels of keratan sulfate, which accumulates in MPS IVA patients. Using qualitative and quantitative proteomics approaches, we analyzed leukocyte samples from healthy controls (n = 6) and from untreated (n = 5) and ERT-treated (n = 8, sampled before and after treatment) MPS IVA patients to identify potential biomarkers of disease. Out of 690 proteins identified in leukocytes, we selected a group of proteins that were dysregulated in MPS IVA patients with ERT. From these, we identified four potential protein biomarkers, all of which may influence bone and cartilage metabolism: lactotransferrin, coronin 1A, neutral alpha-glucosidase AB, and vitronectin. Further studies of cartilage and bone alterations in MPS IVA will be required to verify the validity of these proteins as potential biomarkers of MPS IVA

[EN] During 2011, CEOPAN, FEAGRA-CCOO, FITAG-UGT and IBV developed a manual for technical assessment to prevent ergonomic risks in the bakery sector, thanks to the funds of the FPRL (IS-0099/2010). This project allowed the development of a simple, rapid and participatory tool for ergonomic risk assessment. This tool answered one of the great difficulties in the sector for the implementation of preventive measures: having simple tools available for entrepreneurs and employees in the context of microSMEs (more than 90% of companies of the bakery sector). In 2012 the same entities have developed a project to overcome another challenge of the sector: the difficulty to access to training caused by work schedules and because so small companies don¿t hire any prevention consultant for technical assessment. Specifically, partners have developed a set of audiovisual podcasts based on real environment, available through a web portal or mobile apps, in order to explain risk factors for employees and good practices to reduce them., [ES] En el marco de la asignación para los panaderos de recursos de la Fundación para la Prevención de Riesgos Laborales (Convocatoria de asignación de recursos del ejercicio 2011, IS-0110/2011, IS-0113/2011 e IS-0136/2011), CEOPAN, las federaciones sindicales FEAGRACCOO y FITAG-UGT y el Instituto de Biomecánica (IBV) han desarrollado un proyecto dirigido a presentar recomendaciones y buenas prácticas ergonómicas mediante mensajes audiovisuales disponibles a través de la web o de los dispositivos móviles de los trabajadores y empresarios del sector de panadería.

El siguiente trabajo presenta un estudio geoestadístico de las principales barrancas del volcán Popocatépetl para obtener la aceleración de la gravedad, la pendiente por distancia-elevación, la altura-gravedad y la fuerza de un líquido. También, se presentan los resultados hipotéticos de un modelo eruptivo para el volcán Popocatépetl., This article presents a geostatistical study of the main canyons from Popocatepetl volcano for obtaining the gravity acceleration, distance-elevation by slope, height-gravity and the force of a liquid. Also, it shows the hypothetical results of an eruptive model for Popocatepelt volcano.

Inhibition of circulating immune cell activation: a molecular antiinflammatory effect of the Mediterranean diet, BACKGROUND: Adherence to the Mediterranean diet (Med-Diet) is associated with a reduced risk of cardiovascular disease (CVD). However, the molecular mechanisms involved are not fully understood. OBJECTIVE: The objective was to compare the effects of 2 Med-Diets with those of a low-fat diet on immune cell activation and soluble inflammatory biomarkers related to atherogenesis in subjects at high risk of CVD. DESIGN: In a controlled study, we randomly assigned 112 older subjects with diabetes or > or =3 CVD risk factors to 3 dietary intervention groups: Med-Diet with supplemental virgin olive oil (VOO), Med-Diet with supplemental nuts, and low-fat diet. Changes from baseline in cellular and serum inflammatory biomarkers were assessed at 3 mo. RESULTS: One hundred six participants (43% women; average age: 68 y) completed the study. At 3 mo, monocyte expression of CD49d, an adhesion molecule crucial for leukocyte homing, and of CD40, a proinflammatory ligand, decreased (P < 0.05) after both Med-Diets but not after the low-fat diet. Serum interleukin-6 and soluble intercellular adhesion molecule-1, inflammatory mediators crucial in firm adhesion of leukocytes to endothelial surfaces, decreased (P < 0.05) in both Med-Diet groups. Soluble vascular cellular adhesion molecule-1 and C-reactive protein decreased only after the Med-Diet with VOO (P < 0.05), whereas interleukin-6, soluble vascular cellular adhesion molecule-1, and soluble intercellular adhesion molecule-1 increased (P < 0.05) after the low-fat diet. CONCLUSIONS: Med-Diets supplemented with VOO or nuts down-regulate cellular and circulating inflammatory biomarkers related to atherogenesis in subjects at high risk of CVD. The results support the recommendation of the Med-Diet as a useful tool against

Si bien actualmente el cálculo de la dosis al paciente en un punto determinado, puede especificarse con precisión suficiente, el problema que se plantea es garantizar que la dosis prescrita por el radioterapeuta y calculada por el radiofísico se corresponda con la recibida realmente por el paciente. Actualmente la única forma de conocerlo es mediante la dosimetría en vivo durante las sesiones de tratamiento. En este trabajo se ha elegido como sistema de dosimetría en vivo los detectores de semiconductor y, la contribución principal del mismo ha consistido en el desarrollo de modelos semiempíricos que permitan dado un espesor y una profundidad, conocer la dosis en cualquier punto del organismo a partir de la dosis medida a la entrada y a la salida del haz en el paciente. Esto supone una mejora a los modelos ya existentes (Sánchez-Doblado y col., 1995-a), puesto que hasta ahora lo más habitual es determinar la dosis en plano medio del paciente, a partir de las medidas realizadas con detectores colocados simultáneamente a la entrada y a la salid de la superficie del mismo, en condiciones de tratamiento. En principio este algoritmo se ha desarrollado para el cálculo de la dosis a pacientes sometidos a irradiación corporal total (ICT), ya que habitualmente no se dispone de sistema de planificación (Sánchez-Nieto, 1995). Por otra parte el sistema se ha implementado en la práctica clínica desde Septiembre de 1993, habiendo acumulado desde entonces una gran experiencia que ha redundado en una mejora del mismo. La realización de este trabajo ha comportado una serie de pasos que pueden considerarse como objetivos particulares del mismo: (i) Estudio exhaustivo de la respuesta de un conjunto de detectores semiconductores de nueva generación, frente a las variables de las cuales ésa depende (tasa de dosis, temperatura, envejecimiento, direccionalidad, etc.). Estos detectores están diseñados para utilizarlos en el ámbito clínico e interesa establecer sus limitaciones. (ii) Puesta

Entre los días 19 y 21 de febrero de 1997, se dio cita en Santiago de Compostela un considerable número de especialistas llegados desde todos los puntos de España para hablar de Filología Francesa. A estas jornadas organizadas por el Departamento de Filología Francesa e Italiana de la Universidad de Santiago de Compostela en colaboración con la Asociación de Profesores de Filología Francesa de la Universidad Española (APFFUE), se presentaron cerca de un centenar de comunicaciones, agrupadas temáticamente en las secciones de Lingüística, Literatura, Metodología de la enseñanza del francés y traducción e Historia de la lengua.