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关键词： 亚急性脊髓联合变性   维生素B12缺乏   脑白质变性  

摘要： 目的探讨亚急性脊髓联合变性(SCD)伴脑白质变性(WMC)的发病机制和临床特点。方法回顾性分析1例SCD伴WMC患者的临床资料并复习文献。结果患者以意识障碍及舞蹈症起病。实验室及影像学检查显示重度维生素B_(12)缺乏(146.00 pg/mL)、胸髓后索脱髓鞘及脑白质对称性病变。治疗后,患者维生素B_(12)水平恢复,舞蹈症改善,但遗留共济失调。结论维生素B12缺乏可致广泛中枢损害,包括脑白质及基底节-小脑通路病变,早期治疗可逆转部分症状,但轴索损伤不可逆。需重视非典型表现的代谢性病因筛查。

关键词： GLP-1 receptor agonists   Graft rejection   Heart failure   Heart transplant   Mortality  

摘要： Glucagon-like peptide 1 receptor agonists (GLP-1RAs) have demonstrated survival benefits and cardioprotective effects in diabetic and heart failure (HF) patients. However, the association of GLP-1RAs use with mortality and morbidity in heart transplant (HT) recipients has not been previously investigated.

关键词： Neuromuscular disease   Outcome measures   Reliability   Rehabilitation   Validity  

摘要： To examine the psychometric properties of the Motor Function Measure 32 (MFM32) in adults with myotonic dystrophy type 1 (DM1). MFM32 is an instrument for evaluating function in neuromuscular diseases that have not been adequately assessed in DM1.

关键词： Biologically active molecules   Cyclic-1,3-diketones   Heterocyclic compounds   Michael addition   Styrylisoxazole derivatives  

摘要： A facile synthesis of styrylisoxazole derivatives via Michael addition reaction of 3-methyl-4-nitro-5-styrylisoxazoles to cyclic-1,3-diketones were reported. This method has several additional advantages such as simple operation, mild reaction conditions, broad substrate scope, good functional group tolerance, very good yields without column purification and gram scale synthesis.

关键词： Crank-Nicolson scheme   Finite difference method   Logarithmic Schr & ouml   dinger equation   Regularized logarithmic Schr & ouml   dinger equation   Nonlinear damping   2D soliton  

摘要： We propose and analyze a Crank-Nicolson finite difference scheme for the (2+1)-dimensional regularized logarithmic nonlinear Schr & ouml;dinger equation with general nonlinear damping. The singular logarithmic term ln(|u|2)\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\ln (|u|<^>2)$$\end{document} is handled through a suitable regularization. We prove existence, uniqueness, and uniform boundedness of the discrete solution in the discrete L2\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$L<^>2$$\end{document} and H01\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$H_0<^>1$$\end{document} norms. A sharp Lipschitz-type estimate enables rigorous error analysis, yielding optimal convergence of order O(tau 2+h2)\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$O( au <^>2 + h<^>2)$$\end{document} in L2\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$L<^>2$$\end{document} norm. Numerical experiments confirm the theoretical results and demonstrate accurate resolution of dissipative soliton dynamics under varying damping and regularization parameters.

关键词： Heart catheterization   Neonate   Operability   Outcome   Pulmonary hypertension   Pulmonary pressures  

摘要： Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) can potentially be reversed by early shunt closure, but its efficacy decreases over time. This study provides valuable descriptive data on: which factors were associated with clinical decision-making of operability and the outcomes of those decisions in infants under 1 year with PAH-CHD and an open shunt.

关键词： Paramylon   β-Glucan   Euglena gracilis   IgA   Macrophage   Mucosal immunity  

摘要： Paramylon (PM) is an insoluble dietary fiber derived from Euglena gracilis. Mechanisms underlying the health-promoting effects of Paramylon ingestion remain unclear. Hence, this study aimed to investigate the effect of PM derived from E. gracilis strain EOD-1 (EOD1PM) intake on mucosal antibody production, elucidate its mechanism, and clarify its effect on infection prevention. EOD1PM ingestion in mice increased anti- β-glucan IgA antibody titer. The anti-Candida β-glucan IgA antibody titer in bronchoalveolar lavage was higher in the EOD1PM group than that in the control. EOD1PM intake also increased interleukin-6- and inducible nitric oxide synthase-producing cells involved in IgA class switching. EOD1PM is suggested to induces IgA production following recognition by Dectin-1. Collectively, EOD1PM induces IgA-producing cells in the intestine and promotes IgA production in the respiratory mucosa, potentially via immune cell activation. The findings suggested that EOD1PM activated mucosal immunity, potentially contributing to prevention of respiratory infections.

关键词： Tirbanibulin   Efficacy   Safety   PRO   ClinRO  

摘要： To assess patient- and clinician-reported outcomes, efficacy, and safety of tirbanibulin 1% administered to patients with actinic keratoses (AKs) in Spain and Italy.

关键词： copper binding homodimeric enzyme   destabilization sod protein structureincreasing   stabilization   superoxide dismutase   lateral sclerosis als   translation   degradation   breaking down superoxide radicalsmore  

摘要： Superoxide dismutase 1(SOD1)is a thermodynamically stable,zinc and copper binding homodimeric enzyme responsible for breaking down superoxide radicals.More than 200,mostly missense,mutations spread throughout the SOD1 gene are associated with the fatal neurodegenerative disease,amyotrophic lateral sclerosis(ALS).A unifying feature of ALS-associated SOD1 mutations is the destabilization of the SOD1 protein structure,increasing the propensity for misfolding and subsequent pathological aggregation.Post-mortem analysis of SOD1-associated ALS tissue shows the accumulation of misfolded SOD1 protein and ubiquitinated SOD1 inclusions within motor neurons.Misfolded SOD1 accumulation and aggregates are implicated in cellular dysfunction via a number of disparate but critical processes,including endoplasmic reticulum stress,oxidative damage,proteasome dysfunction,axonal transport abnormalities and synaptic dysfunction;culminating in motor neuron degeneration associated with ALS.