Fabry Disease and the Heart: A Comprehensive Review

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations of the GLA gene that result in a deficiency of the enzymatic activity of α-galactosidase A and consequent accumulation of glycosphingolipids in body fluids and lysosomes of the cells throughout the body. GB3 accumulation occurs in virtually all cardiac cells (cardiomyocytes, conduction system cells, fibroblasts, and endothelial and smooth muscle vascular cells), ultimately leading to ventricular hypertrophy and fibrosis, heart failure, valve disease, angina, dysrhythmias, cardiac conduction abnormalities, and sudden death. Despite available therapies and supportive treatment, cardiac involvement carries a major prognostic impact, representing the main cause of death in FD. In the last years, knowledge has substantially evolved on the pathophysiological mechanisms leading to cardiac damage, the natural history of cardiac manifestations, the late-onset phenotypes with predominant cardiac involvement, the early markers of cardiac damage, the role of multimodality cardiac imaging on the diagnosis, management and follow-up of Fabry patients, and the cardiac efficacy of available therapies. Herein, we provide a comprehensive and integrated review on the cardiac involvement of FD, at the pathophysiological, anatomopathological, laboratory, imaging, and clinical levels, as well as on the diagnosis and management of cardiac manifestations, their supportive treatment, and the cardiac efficacy of specific therapies, such as enzyme replacement therapy and migalastat.     

In vivo aging of gutta‐percha dental cone

Gutta‐percha cone is the most widely used material for root canal filling. The in vivo aging of this cone focus on the degradation of its main organic component, trans‐1,4‐polyisoprene, was studied. Aged cones (25 samples) from 2 to 30 years of root canal filling were extracted from different patients in the occasion of retreatment by mechanical way. The information about the aging time was given by the patients. Gel‐permeation chromatography (GPC) and infrared spectroscopy (FTIR) were the analytical techniques used. Polyisoprene degrades with time of aging, but in a slow process. Decrease in polymer molar mass from 5.7 × 10⁵ to 1.7 × 10⁵ g/mol was observed in polyisoprene from cone after 30 years of root canal filling and inside a noninfected tooth. In tooth with caries and periodontal infection, the decrease in molar mass is higher (4.6 × 10⁴ g/mol in cone with 10 years of aging). The production of carbonyl and hydroxyl groups in the aged material indicates that the process is oxidative, even in closed teeth. In these cases, the oxygen could be provided from tissue fluid. The degradation mechanism is complex and depends on many factors, besides time of root canal filling. The dental problem caused by the aging could be the production and migration of cytotoxic substances to periodontal ligament and the reduction on the canal sealing property due to the polymer weight loss. Both of them could contribute to the root canal treatment failure. © 2006 Wiley Periodicals, Inc. J Appl Polym Sci 100:4082–4088, 2006     

The effect of grazing intensity and the presence of a forage legume on nitrogen dynamics in Brachiaria pastures in the Atlantic forest region of the south of Bahia, Brazil

It has been shown that with careful grazing management and addition of Pand K, but not N, fertilisers Brachiaria pastures are ableto maintain sustainable live weight gains over many years. However, standardon-farm practice, which generally involves high stocking rates, leads after afew years to pasture decline due mainly to N deficiency for grass regrowth. Togenerate an understanding of the mechanism of pasture decline and possiblemanagement options to mitigate this process, a study was performed in theAtlantic forest region of the south of Bahia state to study the N dynamics inpastures of Brachiaria humidicola subject to threedifferent stocking rates of beef cattle, with and without the presence of theforage legume Desmodium ovalifolium. Despite the fact thatthe C:N ratio of the deposited litter was high (60 to 70) the rate ofdecomposition was very rapid (k –0.07 gg^–1 day^–1) and annual rates of Nturnover through the litter pathway were between 105 and 170 kg Nha^–1 year^–1. In the grass-onlypasturesas stocking rate increased from 2 to 3 head ha^–1, N recycledinthe litter decreased by 11%, but a further increase to 4 headha^–1 decreased N recycling by 30% suggesting thatbeyonda certain critical level higher grazing stocking rates would lead to pasturedecline if there was no N addition. High stocking rates decreased theproportionof the legume in the sward, but at all rates the concentration of N in both thegreen and dead grass in the forage on offer and in the litter was higher in themixed sward. The presence of the legume caused a decrease in the C:N ratio ofthe microbial biomass while both soil N mineralisation and nitrificationincreased. This increased rate of turnover of the microbial biomass and thecontribution of N₂ fixation to the legume resulted in largeincreasesin the N recycled via litter deposition ranging from 42 to 155 kg Nha^–1 year^–1.     

Comparative electrochemical studies of zinc chromate and zinc phosphate as corrosion inhibitors for zinc

The anticorrosive performance of two inhibitive pigments, zinc chromate and zinc phosphate, was compared using electrochemical impedance spectroscopy (EIS) and the scanning vibrating electrode technique (SVET) in pigment extracts in 0.1 M NaCl. It was observed that zinc was protected from corrosion in both extracts. In tests using hot dip galvanised steel painted with an epoxy primer incorporating the pigments, the SVET detected the anodic and cathodic distribution along the scribes, although no significant differences were observed among the various primers. On the contrary, EIS was able to distinguish processes occurring on the metal surface exposed by the scribe in different samples. For primers with anticorrosive pigment, a time constant at high frequencies was attributed to a layer of protective nature, probably formed by metal ions from the substrate and inhibitive ions leached from the anticorrosive pigments.     

Improving process models by mining mappings of low-level events to high-level activities

While it is possible to analyze the run-time behavior of a business process through process mining techniques, in practice there is often a gap between the low-level nature of the events recorded in an event log and the high-level of abstraction at which the process is modeled. This makes it difficult to understand the recorded behavior in terms of the high-level activities in the process model. Also, it makes it difficult to improve the model based on run-time data about the process. In this work we present an approach to mine mappings between the events in the log and the activities in the model. These mappings can be used to generate suggestions of how the process model can be extended in order to capture the behavior recorded in the event log. Using a real-world and publicly available event log, we show how the approach can improve the model in a stepwise manner, until it covers all the behavior recorded in the event log.     

Development of ceramic floor tile compositions based on quartzite and granite sludges

In the present work, industrial sludges derived from cutting and polishing natural stones (granite and quartzite) were characterised in terms of particles size distribution, chemical and mineralogical composition, and thermal behaviour and their potential to be incorporated as main components in red-clay-based stoneware tiles was evaluated. High levels (60–70 wt.%) of incorporation were attempted aiming at designing new formulations intended to be less expensive and possess better final properties (lower water absorption and higher flexural strength) in comparison to an industrial reference body used in the production of rustic tiles by extrusion, characterised 8–9% water absorption and a flexural strength of 17–18 MPa. Extruded rods of different formulations were produced and fired at 1100 °C, 1125 °C, 1150 °C and 1200 °C. The experimental results showed that all the new formulations performed better with the most significant improvements being obtained with incorporation of granite sludge. Flexural strength values more than triplicate and water absorption decreased by more than one order of magnitude in comparison to the reference paste. The new products fulfil the requirements of the ISO 13006 standard, group BIa (porcelain tiles).     

Degradation of cellulosic and hemicellulosic substrates using a chelator‐mediated Fenton reaction

The involvement of catechol and hydroxamate chelators, along with hydrogen peroxide and Fe³⁺, in the degradation of cellulosic and hemicellulosic substrates was examined with the purpose of improving our current knowledge of the non‐enzymatic mechanisms involved in wood biodegradation by fungi. It could be demonstrated that a catechol chelator‐mediated Fenton reaction not only clearly degraded hemicellulosic substrates but also significantly accelerated and increased the effectiveness of degradation reactions. On the other hand, when a hydroxamate chelator‐mediated Fenton reaction was used, an inhibitory effect was observed. When cellulosic substrates underwent a chelator‐mediated Fenton reaction, no significant difference in degradation was observed between catechol and hydroxamate chelator‐mediated reactions. However, a catechol‐mediated reaction did accelerate the degradation of cellulosic substrates at the beginning of reactions. In addition, it was observed that with a chelator‐mediated Fenton reaction, oxidation of cellulose proceeds depolymerization. Copyright © 2005 Society of Chemical Industry     

Dielectric behavior of PVDF/POMA blends that have a low doped POMA content

The real (ε′) and imaginary (ε″) components of the complex permittivity of blends of PVDF [poly(vinylidene fluoride)] with POMA [poly(o‐methoxyaniline)] doped with toluenosulfonic acid (TSA) containing 1, 2.5, and 5 wt % POMA–TSA were determined in the frequency interval between 10² and 3 × 10⁶ Hz and in the temperature range from ?120 up to 120°C. It was observed that the values of ε′ and ε″ had a greater increase with the POMA–TSA content and with a temperature in the region of frequencies below 10 kHz. This effect decreased with frequency and it was attributed to interfacial polarization. This polarization was caused by the blend heterogeneity, formed by conductive POMA–TSA agglomerates dispersed in an insulating matrix of PVDF. The equation of Maxwell–Garnett, modified by Cohen, was used to evaluate the permittivity and conductivity behavior of POMA–TSA in the blends. A strong decrease was observed in POMA–TSA conductivity in the blend, which was bigger the lower the POMA–TSA content in the blend. This decrease could have been caused either by the POMA dedoping during the blend preparation process or by its dispersion into the insulating matrix. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 87: 752–758, 2003     

Evolutionary robustness analysis for multi-objective optimization: benchmark problems

This paper presents a new approach to robustness analysis in multi-objective optimization problems aimed at obtaining the most robust Pareto front solutions and distributing the solutions along the most robust regions of the optimal Pareto set. A new set of test problems accounting for the different types of robustness cases is presented in this study. Non-dominated solutions are classified according to their degree of robustness and are distributed along the Pareto front according to specific algorithm parameter values. Verification of the proposed method is carried out using the developed test problems and artificial and real world benchmark test problems present in the literature.     

Comparative study of homogeneous and heterogeneous styrene polymerizations with Ni(acac)2/MAO catalytic system

Styrene polymerization was carried out with Ni(acac)₂/MAO and Ni(acac)₂/SiO₂/MAO. The influence of reaction parameters (Al/Ni mole ratio, catalyst concentration, temperature and time polymerization) on styrene polymerization was evaluated. It was observed that both catalytic systems were affected by reaction parameters and that the heterogeneous catalyst presented higher activity than the homogeneous one. Polystyrenes with different molecular weight, stereoregularity and polydispersity were obtained. These results suggest that different active catalyst species could have been present. In addition, two types of methylaluminoxane (MAO) with different molecular weights were also evaluated as cocatalyst. As a result, the catalyst activity and stereospecificity were strongly affected by the MAO type.