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.     

Immune Checkpoint Inhibitors: A Promising Treatment Option for Metastatic Castration-Resistant Prostate Cancer?

Since 2010, several treatment options have been available for men with metastatic castration-resistant prostate cancer (mCRPC), including immunotherapeutic agents, although the clinical benefit of these agents remains inconclusive in unselected mCRPC patients. In recent years, however, immunotherapy has re-emerged as a promising therapeutic option to stimulate antitumor immunity, particularly with the use of immune checkpoint inhibitors (ICIs), such as PD-1/PD-L1 and CTLA-4 inhibitors. There is increasing evidence that ICIs may be especially beneficial in specific subgroups of patients with high PD-L1 tumor expression, high tumor mutational burden, or tumors with high microsatellite instability/mismatch repair deficiency. If we are to improve the efficacy of ICIs, it is crucial to have a better understanding of the mechanisms of resistance to ICIs and to identify predictive biomarkers to determine which patients are most likely to benefit. This review focuses on the current status of ICIs for the treatment of mCRPC (either as monotherapy or in combination with other drugs), mechanisms of resistance, potential predictive biomarkers, and future challenges in the management of mCRPC.     

陶瓷/金属钎焊与扩散连接的研究现状

工程陶瓷由于具有优异的综合性能,在许多领域得到广泛应用,但其加工性能差,通常需要与金属组成复合结构.实现陶瓷与金属之间的可靠连接是推进陶瓷材料应用的关键,钎焊和扩散连接被认为是陶瓷/金属连接中较为适合的方法.文中对近年来国内外陶瓷/金属钎焊和扩散连接技术领域的研究现状进行了综述,认为活性金属钎焊和部分瞬间液相连接发展比较成熟,部分瞬间液相连接充分结合了活性钎焊和固相扩散连接两者的优点,将成为未来陶瓷/金属连接的发展方向.     

等误差直线逼近非圆曲线节点计算新方法

传统的等误差直线逼近法从被逼曲线的端点开始计算节点.文章在此基础上提出一种非圆曲线节点计算新方法,以被逼曲线曲率半径最小点开始,按等误差法求取节点,再根据曲线规律对节点重新进行排序,输出节点坐标值.用该种方法逼近曲线,在逼近误差等于等误差法逼近误差的条件下,节点数少于等误差法求得的节点数,文中用C语言编写新方法求取节点程序,并与传统方法进行比较,最后用曲线方程进行验证,结论正确,具有实际意义.     

负热膨胀材料立方ZrMo2O8的制备研究

以ZrO(NO3)2和N6H24Mo7O24·4H2O等为原料,采用湿化学法制备出立方相ZrMo2O8粉体.对前驱体在室温～800℃进行了TG-DSC测试,采用XRD、SEM对立方相ZrMo2O8粉体进行了分析观测.结果表明亚稳相立方ZrMo2O8的制备对热处理工艺十分敏感,采用湿化学法在450℃制各出单一立方相ZrMo2O8粉体,在室温～400℃热膨胀系数为-4.337×10-6K-1.     

Detection of water ingress in composite sandwich structures: a magnetic resonance approach

Water ingress inside honeycomb sandwich panels during service has been linked to in-flight failure in some aircraft. There is an ongoing effort to develop nondestructive testing methods to detect the presence of water within the panels. Magnetic resonance (MR) represents an attractive approach in that it is sensitive to moisture. Using a unilateral MR sensor, testing can be applied directly to the surface of the panel. The viability of MR is demonstrated through laboratory imaging of both water within sandwich panels, as well as the adhesive itself. The detection of water using a one-sided handheld MR sensor is presented. It is shown that simple detection, as well as spatial localization of water within sandwich panels is possible.     

Isobaric vapor—liquid equilibria for binary systems composed of methyl butanoate with ethanol and with propan-1-ol at 114.66 and 127.99 kpa

Vapor-liquid equilibrium data for the binary systems methyl butanoate/ethanol and methyl butanoate/propan-l-ol at pressures of 114.66 and 127.99 kPa are presented. Both mixtures yield an azeotrope, the compositions (ester) of which decrease quasi-linearly as the pressure increases. The experimental data for all the binary mixtures were fitted to a suitable equation and then used for comparison with predictions by ASOG and UNIFAC methods. These group contribution models give a good estimation of activity coefficients with an overall mean error less than 5% for all cases.     

含水量对渠道保温板保温耐久性的影响及保温板厚度设计

北方渠道大多使用保温板防止混凝土衬砌受冻胀破坏。在计算保温板厚度时为了降低成本,容易忽略保温板使用过程中受各种因素的影响导致保温效果变差的情况,使混凝土衬砌达不到设计使用年限便提前被破坏。内蒙古河套灌区试验结果表明,由于受酸雨、冻融作用、含水量等因素的影响,保温板保温效果平均每年降低0.9℃,其中受含水量变化的影响最大。经试验测得:聚苯乙烯保温板(EPS)含水量每增加1%,会导致导热系数增大5%。基于多层材料串联热阻叠加原理和热阻等效原理,对保温板厚度计算公式进行完善,并提出相应的铺设方法和施工技术要求。该设计对保温板在季节性冻土区的推广应用具有一定参考价值。     

Comparison of a contactor catalytic membrane reactor with a conventional reactor: example of wet air oxidation

A wet air oxidation reaction was carried out in a gas/liquid catalytic membrane reactor of the contactor type. The oxidation of formic acid was used as a model reaction. The mesoporous top-layer of a ceramic tubular membrane was used as catalyst (Pt) support, and was placed at the interface of the gas (air) and liquid (HCOOH solution) phases.

A similar reaction was carried out in a conventional batch reactor, using a steering rate high enough to avoid gas-diffusion limitations, and exactly identical conditions than for the CMR (amount of catalyst, pressure, etc.). At room temperature, the CMR showed an initial activity three to six times higher than the conventional reactor. This activity increase was attributed to an easier oxygen access to the catalytic sites. Nevertheless, the catalytic membrane gradually deactivated after a few hours of operation. Different deactivation mechanisms are presented.     

非水溶剂回滴法测定低镍甲烷化催化剂的表面酸性

本文采用非水溶剂回滴法测定了低镍甲烷化催化剂和载体γ—Al2O3的表面酸性，并与催化剂的活性、组成、比表面积关联。实验结果说明，助剂的种类、含量对表面酸性有明显影响，催化剂催化活性与表面酸性关系不大。