365 research outputs found
Dysferlinopathy course and sportive activity: clues for possible treatment
LGMD2B is a frequent proximo-distal myopathy with rapid evolution after age 20. Exacerbating factors may be physical exercise and inflammation. There is very little information about the effect of sportive activity in LGMD2B, since eccentric exercise frequently results in muscle damage. LGMD2B has often an onset with myalgia and MRI imaging (STIR-sequences) shows myoedema. In a prolonged observational study of a series of 18 MM/LGMD2B patients we have studied the pattern of clinical and radiological evolution. The disease has an abrupt onset in the second decade and most patients perform sports before definite disease onset. On the basis of Gardner-Medwin and Walton scale, grade 4 is reached two years faster in patients who performed sports (over 1000 hours). Other considerations regarding pathogenetic mechanism and response to treatment show a poor response to immunosuppressive treatment of muscle inflammation. Preventing a strenuous physical activity should be recommended in patients with high CK and diagnosed or suspected to have dysferlin deficiency
THE PALLADIOLIBRARY GEO-MODELS: AN OPEN 3D ARCHIVE TO MANAGE AND VISUALIZE INFORMATION-COMMUNICATION RESOURCES ABOUT PALLADIO
Abstract. The paper describes objectives, methods, procedures and outcomes of the development of the digital archive of Palladio works and documentation: the PALLADIOLibrary of Centro Internazionale di Studi di Architettura Andrea Palladio di Vicenza (CISAAP). The core of the application consists of fifty-one reality-based 3D models usable and navigable within a system grounded on GoogleEarth. This information system, a collaboration of four universities bearers of specific skills returns a comprehensive, structured and coherent semantic interpretation of Palladian landscape through shapes realistically reconstructed from historical sources and surveys and treated for GE with Ambient Occlusion techniques, overcoming the traditional display mode
5'-deiodinase activity and circulating thyronines in lactating cows.
To investigate the correlation between lactation and thyroid hormone metabolism, the authors studied concentrations of total and free thyroxine (T4 and fT4), triiodothyronine (T3 and fT3), and reverse triiodothyronine (rT3) in plasma and milk, as well as liver and mammary gland 5'-deiodinase (5'D) activity in dry, early, middle, and late lactating dairy cows. Cows in early lactation show lower plasma levels of T4 and rT3 than dry, middle, and late lactating animals, whereas T3 shows the lowest plasma levels in the dry period; free T4 and T3 show a similar pattern. In early lactation there is a clear decrease in liver 5'D associated with a notable increase in mammary 5'D. Concentrations of T4 and T3 in milk drop significantly in the first few days after delivery, whereas rT3 increases up to the fourth month. The findings suggest a relationship between the hypothyroid status of lactating cows and the rearrangement of organ-specific 5'-deiodinase activity related to the maintenance of the udder's function
Relationship between Secondary Metabolites, Antiradical Activities, and Colour Characteristics of Cochlospermum Tinctorium A. Rich. (Bixaceae) Root
Cochlospermum tinctorium A. Rich. (Bixaceae) is a widespread herbaceous plant in the African sahelian and soudanian zones. Its root is used as food and medicinal plant. Studies of secondary metabolites, functional activities and trichromatic parameters were realized according to standard methods of laboratory. At a threshold of α = 0.05, significant correlations (p < 0.05) were found between secondary metabolites contents and functional activities such as radical scavenging activity of antioxidant component or hydroxyl radical scavenging activity. The value of DPPHH was 95.56 ± 0.94% for CT inner Matrix with variation of 0.12% for CT Soaked inner Matrix and 1.63% for CT Peeler. But HSRA was 37.14 ± 5.71 for CT inner Matrix with variation of -38.46% for CT Soaked inner Matrix and -71.79% for CT Peeler. Cochlospermum tinctorium root has exhibited efficiency to heal many diseases thanks to multiple bioactive compounds. In addition, the colour of powders depended upon chemical compounds. Finally, Cochlospermum tinctorium root was found to neutralize organic radicals and reactive oxygen species
A General Approach for Predicting the Filtration of Soft and Permeable Colloids: The Milk Example
Membrane filtration operations (ultra-, microfiltration) are now extensively used for concentrating or separating an ever-growing variety of colloidal dispersions. However, the phenomena that determine the efficiency of these operations are not yet fully understood. This is especially the case when dealing with colloids that are soft, deformable, and permeable. In this paper, we propose a methodology for building a model that is able to predict the performance (flux, concentration profiles) of the filtration of such objects in relation with the operating conditions. This is done by focusing on the case of milk filtration, all experiments being performed with dispersions of milk casein micelles, which are sort of ″natural″ colloidal microgels. Using this example, we develop the general idea that a filtration model can always be built for a given colloidal dispersion as long as this dispersion has been characterized in terms of osmotic pressure Π and hydraulic permeability k. For soft and permeable colloids, the major issue is that the permeability k cannot be assessed in a trivial way like in the case for hard-sphere colloids. To get around this difficulty, we follow two distinct approaches to actually measure k: a direct approach, involving osmotic stress experiments, and a reverse-calculation approach, that consists of estimating k through well-controlled filtration experiments. The resulting filtration model is then validated against experimental measurements obtained from combined milk filtration/SAXS experiments. We also give precise examples of how the model can be used, as well as a brief discussion on the possible universality of the approach presented here
Joint Transmission and Sensing in Few-Mode Fibre
We experimentally demonstrate the coexistence of transmission and sensing using an innovative commercial 15-mode few-mode fibre system. With respect to previous works where the cascade of single-few-single mode fibres has been used, our system features a mode multiplexer and demultiplexer. By transmitting a telecommunication signal over the fibre fundamental mode, the sensing capabilities of the few-mode fibre are demonstrated analysing the power variation of the higher-order modes directly excited by the fundamental mode itself owing to the intermodal crosstalk perturbation
Maitotoxin-4, a Novel MTX Analog Produced by Gambierdiscus excentricus
Maitotoxins (MTXs) are among the most potent toxins known. These toxins are produced by epi-benthic dinoflagellates of the genera Gambierdiscus and Fukuyoa and may play a role in causing the symptoms associated with Ciguatera Fish Poisoning. A recent survey revealed that, of the species tested, the newly described species from the Canary Islands, G. excentricus, is one of the most maitotoxic. The goal of the present study was to characterize MTX-related compounds produced by this species. Initially, lysates of cells from two Canary Island G. excentricus strains VGO791 and VGO792 were partially purified by (i) liquid-liquid partitioning between dichloromethane and aqueous methanol followed by (ii) size-exclusion chromatography. Fractions from chromatographic separation were screened for MTX toxicity using both the neuroblastoma neuro-2a (N2a) cytotoxicity and Ca2+ flux functional assays. Fractions containing MTX activity were analyzed using liquid chromatography coupled to high-resolution mass spectrometry (LC-HRMS) to pinpoint potential MTX analogs. Subsequent non-targeted HRMS analysis permitted the identification of a novel MTX analog, maitotoxin-4 (MTX4, accurate mono-isotopic mass of 3292.4860 Da, as free acid form) in the most toxic fractions. HRMS/MS spectra of MTX4 as well as of MTX are presented. In addition, crude methanolic extracts of five other strains of G. excentricus and 37 other strains representing one Fukuyoa species and ten species, one ribotype and one undetermined strain/species of Gambierdiscus were screened for the presence of MTXs using low resolution tandem mass spectrometry (LRMS/MS). This targeted analysis indicated the original maitotoxin (MTX) was only present in one strain (G. australes S080911_1). Putative maitotoxin-2 (p-MTX2) and maitotoxin-3 (p-MTX3) were identified in several other species, but confirmation was not possible because of the lack of reference material. Maitotoxin-4 was detected in all seven strains of G. excentricus examined, independently of their origin (Brazil, Canary Islands and Caribbean), and not detected in any other species. MTX4 may therefore serve as a biomarker for the highly toxic G. excentricus in the Atlantic area
Long-term effectiveness and tolerability of apremilast in patients with moderate-to- severe plaque psoriasis: A 5-year multicentre retrospective study—IL PSO (Italian landscape psoriasis)
Apremilast is an oral inhibitor of phosphodiesterase (PDE)-4
that has been approved for the treatment of patients with
moderate-to-
severe
plaque psoriasis who have contraindications
or inadequate response to conventional systemic treatments.
1,2 It is also indicated for the management of adults
with active psoriatic arthritis (PsA) who have failed or are
intolerant to other disease-modifying
antirheumatic drugs
(DMARDs).1,2 In Italy, apremilast is reimbursed for patients
with moderate-to-
severe
plaque psoriasis in case of inadequate
response/intolerance to systemic treatments and those
with contraindications to biological drugs.3 Despite the wide
use of apremilast for the treatment of plaque psoriasis, long-term
real-world
experiences are still limited.4,
Drug survival of IL-12/23, IL-17 and IL-23 inhibitors for moderate-to-severe plaque psoriasis: a retrospective multicenter real-world experience on 5932 treatment courses – IL PSO (Italian landscape psoriasis)
Introduction: The development of several effective biological drugs for moderate-to-severe plaque psoriasis has dramatically changed the lives of patients. Despite the wide use of interleukin (IL) inhibitors, limited data are available to date regarding long-term treatment persistence. Method: This multicenter retrospective real-world study evaluated 5932 treatment courses across 5300 patients, all treated with interleukin inhibitors. Drug survival was expressed by using the Kaplan-Meier estimator for each biological drug at 6, 12, 24, 36 and 48 months. We also stratified by discontinuation associated with primary or secondary ineffectiveness. Results: In our study, the most prescribed drugs were secukinumab (1412), ixekizumab (1183), and risankizumab (977). After four years of follow-up, risankizumab emerged as the treatment with the highest drug survival overall, as 91.6% of patients were still on treatment. The overall probability of drug survival at four years was comparable for tildrakizumab (83.5%), ixekizumab (82.6%), guselkumab (82.4%) and brodalumab (81.8%). When evaluating only patients who discontinued the treatment because of ineffectiveness, once again risankizumab was the molecule with the highest drug survival at 4 years (93.4%), this time followed by ixekizumab (87%). Our study, in which all IL inhibitors were adequately represented, confirmed a slightly better treatment persistence for IL-23 inhibitors, consistent with other real-world studies. Conclusion: Our experience showed that IL-23 inhibitors, and risankizumab in particular, had a higher probability of drug survival overall during a 4-year follow-up. Risankizumab and ixekizumab were less likely to be discontinued because of ineffectiveness after four years
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