Neuropathies optiques héréditaires

Les neuropathies optiques héréditaires sont un groupe hétérogène de pathologies affectant les nerfs optiques et qui peuvent se transmettre selon un mode autosomique dominant, autosomique récessif, lié à l’X ou selon une hérédité mitochondriale. Les deux pathologies héréditaires non syndromiques les plus fréquentes (neuropathie optique héréditaire de Leber et l’atrophie optique dominante) ont des manifestations cliniques et des modes de transmission très différents, aboutissant néanmoins à une atrophie optique non spécifique. Au-delà des manifestations purement visuelles, il n’est pas exceptionnel de constater chez des patients atteints, un nombre croissant de manifestations neurologiques associées

Dietary Vitamin D and AMD

International audienc

Impaired saccadic eye movement in primary open-angle glaucoma

PURPOSE: Our study aimed at investigating the extent to which saccadic eye movements are disrupted in patients with primary open-angle glaucoma (POAG). This approach followed upon the discovery of differences in the eye-movement behavior of POAG patients during the exploration of complex visual scenes. METHODS: The eye movements of 8 POAG patients and 4 healthy age-matched controls were recorded. Four of the patients had documented visual field scotoma, and 4 had no identifiable scotoma on visual field testing. The eye movements were monitored as the observers watched static and kinetic targets. The gain, latency, and velocity-peak latency of the saccades recorded were then analyzed. RESULTS: In POAG patients, with abnormal visual fields, watching a static target, the saccades were delayed and their accuracy was reduced, compared with those of normal observers. In POAG patients, with normal and abnormal visual fields, watching a kinetic target, a task involving precise motion analysis, the latency and accuracy of the saccades were impaired, compared with those of normal observers. CONCLUSIONS: Our findings suggest that POAG alters saccade programming and execution particularly in the case of moving targets

CONSIDERATIONS ON THE TECHNOLOGIES FOR SEPARATING FRUIT SEEDS WITH APPLICATIONS ON THE SEPARATION OF SEABUCKTHORN PULP FROM SEEDS

Sea-buckthorn is a shrub fruit whose benefits have been known since antiquity. The whole plant is considered medicinal by specialists in the field, but the most active part is the sea-buckthorn fruit because it has a beneficial effect on the entire human body and more recently on animal breeding. A high-value by-product is sea-buckthorn oil, which is currently gaining traders’ attention at international level and which is obtained from both fruit and pomace resulting from the extraction of juice (shells and seeds). This paper presents some representative technolo-gies and installations used for fruit processing in general, technologies that will be the basis for the realization by INMA Bucharest of a technical equipment for separating sea-buckthorn pulp from the seeds

Association Between Hypovitaminosis D and Late Stages of Age-Related Macular Degeneration: A Case-Control Study

International audienc

Association Between Serum 25-Hydroxyvitamin D Concentration and Optic Chiasm Volume

International audienc

Chelating Agents for the Sequestration of Mercury(II) and Monomethyl Mercury(II)

Both mercury(II) and monomethyl mercury(II) poisonings are of great concern for several reasons. As it happens for other metals, chelation therapy is the most indicated treatment for poisoned patients. The efficacy of the therapy and the reduction of side-effects can be sensibly enhanced by an accurate knowledge of all the physiological mechanisms involved in metal uptake, transport within and between various tissues, and (possibly) clearance. All these aspects, however, are strictly dependent on the chemical speciation (i.e., the distribution of the chemical species of a component in a given system) of both the metal and the chelating agent in the systems where they are present. In this light, this review analyzes the state of the art of research performed in this field for mercury(II) and methylmercury(II). After a brief summary of their main sources, the physiological patterns for the treatment of mercury poisoning have also been considered. The binding ability of various chelating agents toward mercury has been then analyzed by modeling the behavior of the main classes of ligands present in biological fluids and/or frequently used in chelation therapy. Their sequestering ability has been successively evaluated by means of a semiempirical parameter already proposed for its objective quantification, and the main characteristics of an efficient chelating agent have been evaluated on this basis

EXPERIMENTAL RESEARCHES ON EXTRUSION PROCESSING OF SOYBEAN SEEDS FOR THEIR SUPERIOR CAPITALIZATION IN ANIMAL FEEDING

The extrusion technology is one of the perspective and efficiency processes, combining hydrothermal and mechanical processing of raw material (soybeans seeds), allowing obtaining new-generation products and components with predetermined properties, with a new structure called instant products, which are successfully used in animals fodder ration. This paper presents the installation for soybean seeds superior capitalization -IVSS (consist of screw conveyor, supply system, extruder, cooling system, mobile belt conveyor and a unit for command and control) and the experimental research in the establishment of its optimal operating parameters. Taking into consideration all this aspects, this project designed at INMA Bucharest tries to transform typical farmer in professional livestock farmer which capitalize on the innovative technologies established by research

Activated Signaling Pathways and Targeted Therapies in Desmoid-Type Fibromatosis: A Literature Review

Desmoid-type fibromatosis (DTF) is a rare, soft tissue tumor of mesenchymal origin which is characterized by local infiltrative growth behavior. Besides “wait and see,” surgery and radiotherapy, several systemic treatments are available for symptomatic patients. Recently, targeted therapies are being explored in DTF. Unfortunately, effective treatment is still hampered by the limited knowledge of the molecular mechanisms that prompt DTF tumorigenesis. Many studies focus on Wnt/β-catenin signaling, since the vast majority of DTF tumors harbor a mutation in the CTNNB1 gene or the APC gene. The established role of the Wnt/β-catenin pathway in DTF forms an attractive therapeutic target, however, drugs targeting this pathway are still in an experimental stage and not yet available in the clinic. Only few studies address other signaling pathways which can drive uncontrolled growth in DTF such as: JAK/STAT, Notch, PI3 kinase/AKT, mTOR, Hedgehog, and the estrogen growth regulatory pathways. Evidence for involvement of these pathways in DTF tumorigenesis is limited and predominantly based on the expression levels of key pathway genes, or on observed clinical responses after targeted treatment. No clear driver role for these pathways in DTF has been identified, and a rationale for clinical studies is often lacking. In this review, we highlight common signaling pathways active in DTF and provide an up-to-date overview of their therapeutic potential

A SURVEY REGARDING THE NEWEST SEEDLINGS PLANTING TECHNOLOGIES THAT CAN BE USED IN AGROFORESTY, LANDSCAPE ECOLOGY AND FOREST REGENERATION FIELDS

Nowadays the seedlings planting technologies are design to raise mechanization performance on forestry and pomiculture works. Their impact is manly on soil remediation, lowering the surface and depth erosion degree, capitalization of rainwater, lowering the dust pollution and natural calamity risk and the rehabilitation of inland flora. In this paper will be presented some of the most performant planting technologies, which can be used in the environmental management process that assures high establishment rate. In some cases, the ecological benefits can occur after a shorter period of time reducing considerably the calamity risks, encouraged the inland flora, forest and degraded the site restauration, but also landscape ecology.Â