Phospholamban p.Arg14del Cardiomyopathy: A Japanese Case Series

Phospholamban p.Arg14del is reported to cause hereditary cardiomyopathy with malignant ventricular tachycardia (VT) and advanced heart failure. However, the clinical courses of Japanese cardiomyopathy patients with phospholamban p.Arg14del remain uncharacterized. We identified five patients with this variant. All patients were diagnosed with dilated cardiomyopathy (DCM), developed end-stage heart failure and experienced VT requiring implantable cardioverter defibrillator discharge. Four patients survived after implantation of a left ventricular assist device (LVAD), while one patient who refused LVAD implantation died of heart failure. Based on the severe course of the disease, we propose genetic screening for phospholamban p.Arg14del in DCM patients.     

Effects of transcranial direct current stimulation over the right dorsolateral prefrontal cortex on fairness-related decision-making

Neuroimaging studies suggest that the right dorsolateral prefrontal cortex (rDLPFC) is an important brain area involved in fairness-related decision-making. In the present study, we used transcranial direct current stimulation (tDCS) over the rDLPFC to investigate the effects of changed cortical excitability on fairness norm enforcement in social decision-making. Participants received anodal, cathodal or sham stimulation before performing a modified ultimatum game task, in which participants were asked to accept or reject the proposer’s offer and self-rate the intensity of their anger at offers on a 7-point scale. The results showed that the rejection rate of unfair offers and anger level were higher in the anodal compared to the sham and cathodal groups and that the level of anger at unfair offers can predict the rejection rate. Furthermore, the fairness effect of RTs was more prominent in the anodal group than in the sham and cathodal groups. Our findings validate the causal role of the rDLPFC in fairness-related decision-making through tDCS, suggesting that strengthening the rDLPFC increases individuals’ reciprocal fairness in social decision-making, both in subjective rating and behaviors.     

持续非卧床腹膜透析患者左心室肥厚与血清N-末端脑钠素原水平的关系

目的探讨持续非卧床腹膜透析（CAPD）患者左心室肥厚（LVH）与血清N-末端脑钠素原（NT-proBNP）水平的关系。方法连续入选32例接受CAPD治疗1年以上患者,超声心动图检查测算左心室心肌质量指数（LVMI）;根据LVMI将入选者分为左心室肥厚组和左心室正常组,双抗体夹心酶标免疫法测定两组患者血清NT-proBNP水平。结果入选CAPD患者中,左心室肥厚组17例,左心室正常组15例,左心室肥厚组血清NT-proBNP（6234.21±854.43）pg/ml较左心室正常组血清NT-proBNP（2456.23±576.98）pg/ml水平明显升高（P〈0.01）,且NT-proBNP水平与LVMI呈正相关（r=0.625,P〈0.01）,NT-proBNP诊断LVH的ROC曲线下面积为0.912。结论 CAPD合并LVH患者血清NT-proBNP水平明显升高,与LVMI呈正相关且有诊断价值,NT-proBNP可能是预测CAPD患者合并LVH的一个新指标。     

用Amplatzer导管封堵器介入闭合先天性膜周部室间隔缺损

目的 研究Amplatzer导管封堵器(ADO)介入闭合先天性膜周部室间隔缺损(VSD)的可行性、安全性和有效性，确定其适应证和并发症。方法 2001年5月至2002年12月，经导管闭合先天性膜周部VSD4l例，VSD直径4～12．8(平均6．0)mm，其上缘距离主动脉右冠瓣4．5～12(平均6．6)mm。心导管资料示QP／QS 1．4～2，6(平均1．7)，肺动脉收缩压19～34(平均25)mmHg。4l例膜周部VSD中，18例伴有膜部膨出瘤。结果 4l例VSD均顺利闭合成功，选用的ADO尺寸为6／4～16／14mm。术后心脏杂音立即消失，选择性左心室造影及经胸超声心动图均无明显残余分流。胸片示肺血管影较术前减少。心电图有12例出现不完全性右束支传导阻滞，l例出现完全性左束支传导阻滞．均在l～2周内自行消失。随访2～21个月，临床及实验室资料进一步改善，未见ADO移位、主动脉瓣或三尖瓣返流及其他并发症。结论 用ADO介入闭合先天性膜周部VSD是完全可行的，而且具有操作简便、安全高效、并发症少和费用较低等优点。     

运动员心动时相及左室顺应性观察

作者认为,对运动员左室功能的全面评价,应包括收缩和舒张功能两个方面,而不应该只是前者。文章提出XJY-6型心脏机能诊断仪可一机多用,尤以对LVEdp和ΔV/ΔP的测试,能为医务监督提供有价值的参据。     

Assessment of left ventricular mechanical dyssynchrony by phase analysis of ECG-gated SPECT myocardial perfusion imaging

Cardiac resynchronization therapy (CRT) has shown benefits in patients with severe heart failure. However, at least 30% of patients selected for CRT by use of traditional criteria (New York Heart Association class III or IV, depressed left ventricular [LV] ejection fraction, and prolonged QRS duration) do not respond to CRT. Recent studies with tissue Doppler imaging have shown that the presence of LV dyssynchrony is an important predictor of response to CRT. Phase analysis has been developed to allow assessment of LV dyssynchrony by gated single photon emission computed tomography myocardial perfusion imaging. This technique uses Fourier harmonic functions to approximate regional wall thickness changes over the cardiac cycle and to calculate the regional onset-of-mechanical contraction phase. Once the onset-of-mechanical contraction phases are obtained 3-dimensionally over the left ventricle, a phase distribution map is formed that represents the degree of LV dyssynchrony. This technique has been compared with other methods of measuring LV dyssynchrony and shown promising results in clinical evaluations. In this review the phase analysis methodology is described, and its up-to-date validations are summarized.     

MMP-1、MMP-3对高动力血流状态肺血管重建的影响

目的:通过检测大鼠肺动脉高压模型肺动脉壁内金属基质蛋白酶鄄1(MMP鄄1)、金属基质蛋白酶鄄3(MMP鄄3)的表达水平,探讨MMP鄄1和MMP鄄3对肺动脉高压肺血管重建的影响。方法:采用套管连接法建立大鼠肺动脉高压模型。将60只健康雄性Wistar大鼠随机分为实验组穴S1、S2雪各20只和对照组(C1、C2)各10只,实验组用套管连接右侧颈总动脉近心端和右侧颈外静脉第1属支的近心端,对照组只在相同部位作假手术。于实验后第8周和第16周测定肺动脉收缩压和右心室收缩压,留取肺组织行免疫组化法染色,应用IA1型自动图像分析系统测定血管壁中MMP鄄1和MMP鄄3的含量相对值。结果:术后8周S1组PAP和RVP均显著升高,与C1组相比有显著差异(P<0.005),术后16周S2组PAP和RVP升高,与C2组相比有显著差异(P<0.001);S1组与S2组比较亦有显著差异(P<0.001);术后8、16周S1组大鼠肺血管大中动脉中膜弹力纤维和平滑肌胞浆及细胞外基质中有较多MMP鄄1和MMP鄄3棕褐色颗粒沉积,同时可见血管内膜增厚,管腔狭窄,平滑肌肌层增厚,无肌型小动脉肌化;而对照组大鼠肺血管无明显MMP鄄1和MMP鄄3棕褐色颗粒沉积和肺高压病理改变。IA1型自动图像分析量化结果显示,术后8、16周实验组和对照组均差异显著(P<0.001);S2组和S1组有显著差异(P<0.001)。结论:MMP鄄1和MM     

医疗保护制度与患者知情同意权中的伦理关系

知情同意权作为患者拥有的基本权利和医生应履行的起码的义务,在沟通医患关系中起着重要作用。由于认知程度的不同,患方主体对病情告知会产生不同的反应。而医生在履行这一义务时会面临法律、伦理和医疗保护制度之间的矛盾。正确认识和处理这些矛盾,对避免医疗纠纷有重要作用。     

医师的行医权与告知义务

医师的行医权和履行医师的告知义务紧密联系、不可分割的。行医权的行使必须以履行法定义务为前提,只有在充分履行告知义务的基础上,行医权才能有保障。文章通过以下几个方面来浅析一下医疗活动中医师的行医权与告知义务：（1）行医权的概念及法律保障。（2）医师的告知义务。（3）医疗服务中的行医权与告知义务的履行。其目的是明确医师的权利和义务,提高医务人员依法行医,切实履行告知义务,才能恰当实施行医权。维护医患双方合法权益,构建和谐的医患关系。     

C反应蛋白对急性心肌梗死后左室重塑的相关性研究

目的:探讨C反应蛋白(CRP)与急性心肌梗死(AMI)后左室重塑的关系.方法:选54例首次确诊单纯前壁AMI患者分别于0、1、2、3、4、5、6天抽血查CRP计算CRp曲线下面积(CRPAUC),3～4天抽血查脑钠肽(BNP),心肌梗死(MI)后2月做超声心动图检查观察左室舒张末期容积指数(LVEDVi),左室收缩末期容积指数(LVESVi),室壁运动指数(WMSI)和左室射血分数(LVEF),根据CRP峰值分为CRP增高组和CRP正常组.设对照组35例,应用回归分析观察CRP峰值(CRRpeak),CRPAUC,BNP与左室重塑性指标的关系.结果:与对照组比较CRP峰值、BNP以及EDVi、ESVi、WMSi、和EF均有显著性差异,P＜0.05.CRP增高组与CRP正常组比较,除WMSi外BNP、EDVi、ESVi、EF均有显著性差异.单因素相关分析显示,CRPpeak,CRPAUC和BNP与ESVi、EDVi呈正相关(r值分别为0.738,0.573:0.876,0.714;0.624,0.577),而与EF呈负相关(r值分别为-0.625,-0.824,-0.531),尤以CRPAUC为显著.结论:CRP可以对AMI后左室重塑和左室功能不全强有力的预测因子,是影响预后的指标,CRP易于临床检测,可以为MI危险分层提供有价值的依据.