Extent of pulmonary fibrosis on high-resolution computed tomography is a prognostic factor in patients with pleuroparenchymal fibroelastosis

BackgroundSeveral prognostic factors for pleuroparenchymal fibroelastosis (PPFE) have recently been reported. However, detailed high-resolution computed tomography (HRCT) findings have not yet been evaluated as prognostic factors. This study retrospectively investigated whether HRCT findings are prognostic factors in patients with PPFE compared to those with idiopathic pulmonary fibrosis (IPF).MethodsPatients with PPFE and IPF diagnosed at our hospital between January 2008 and December 2016 were enrolled. Clinical and HRCT characteristics were obtained. In addition to our patients, we also analyzed data of PPFE patients whose cause of death had been identified in previous studies.ResultsWe enrolled 15 patients with PPFE and 75 patients with IPF. Consolidation and maximum pleural thickening were significantly higher in patients with PPFE than in those with IPF (both P < .001). Fibrosis score, honeycomb area, and traction bronchiectasis were not significantly different between these patient groups but were significant prognostic factors in patients with PPFE in univariate analysis (P = .021, P = .017, and P = .014, respectively). The proportions of deaths by acute exacerbation or lung cancer were significantly lower in patients with PPFE than in those with IPF (P < .001 and P = .001, respectively), whereas death by respiratory failure was significantly more frequent in PPFE patients (P < .001).ConclusionsHRCT findings, such as fibrosis score, honeycomb area, and traction bronchiectasis, were independent prognostic factors in patients with PPFE. Respiratory failure, but not acute exacerbation and lung cancer, was the main cause of death in patients with PPFE.     

经静脉左心室声学造影在超声运动试验中的应用

目的 :用东冠注射液声学造影剂进行经静脉左心室声学造影 (LCE) ,探讨其在超声运动试验中的应用价值。　　方法 :对 40名可疑冠心病患者行平板运动超声心动图检查 ,运动前后静脉注射声学造影剂各 0 2 2ml/kg ,观察左心室内膜边界分辨度的变化。　　结果 :运动前 ,注射造影剂后左心室内膜可分辨度计分≥ 2分的节段数较造影前提高了 2 1% (P≤ 0 0 1) ,运动后则提高了 2 6% (P≤ 0 0 0 1)。　　结论 :运动前后注射造影剂均可显著地提高左心室各个节段的内膜分辨度 ,从而利于分析室壁运动和室壁增厚情况 ,增加判断心肌缺血及其部位的准确性。     

Secondary endocardial fibroelastosis associated with Pompe disease and multicystic dysplastic kidney

An infant with secondary endocardial fibroelastosis (EFE) associated with glycogen storage disease II (Pompe disease) and multicystic dysplastic kidney (MCDK) is described. She had had refractory heart and renal failure from the early neonatal period. In spite of administration of cathecholamines and diuretics, ventilator support, and peritoneal dialysis, her heart failure due to reduction of left ventricular contractility progressively worsened. She died on the 40th day after admission. Histological examination of a left ventricular autopsy specimen showed prominent thickening of the endocardium due to fibroelastosis, and a lacework-like structure due to accumulation of glycogen in the cardiomyocytes. The EFE was derived from degeneration of the smooth muscle in the endocardium and cardiomyocytes due to glycogen storage. In addition, we supposed that the renal failure due to MCDK made the preload for the ventricles increase and accelerated her heart failure. Received: September 5, 2000 / Accepted: March 9, 2001     

早期后除极与再灌注心律失常机理的实验研究

再灌注心律失常已引起日益广泛的重视,但其发生机理尚未完全阐明。笔者应用心内膜接触电极导管,记录犬缺血及再灌注时在体心脏左室心内膜单相动作电位(MAPs)。10只犬,建立缺血(20分钟)—再灌注模型18次,66％(12/18)于再灌注时记录到早期后除极(EADs),其平均振幅为3.9±1.2mV,占MAPs振幅的22％。其中75％(9/12)发生与EADs有关的心律失常,EADs的耦联间期与室性早搏的耦联间期高度相关(r=0.71,P<0.01)。3只犬EADs振幅呈高低变化时,伴有左心腔电图T波电压交替变化。研究结果表明,EADs诱发的触发活动可能为再灌注心律失常的主要发生机理。MAPs技术是研究在体心脏触发活动的可靠方法。在某些特殊病例,EADs振幅的交替变化,可能是体表心电图T波电交替的发生机理。     

特发性左心室心动过速致心律失常基质的电解剖差异

目的 探索特发性左心室心动过速（ILVT）和房室折返性心动过速（AVRT）患者左心室传导系统和缓慢传导区电解剖变异情况.方法 选取2009年5月至2011年12月20例成功消融的ILVT患者（ILVT组）,年龄20～51（37±7）岁,男16例;26例AVRT患者（对照组）,年龄25～51（38±8）岁,男20例,窦性心律下分别建立左心室三维电解剖标测,标记左心室传导系统、缓慢传导区及其交汇区,分析变异情况.ILVT组患者依据消融关键区和拖带刺激进行消融.结果 根据浦肯野电位分布将传导系统变异分为3个亚型：两分支、三分支和扇形分布于左心室间隔,两组间各分支长度差异无统计学意义（P＞0.05）.缓慢传导区在ILVT中亦存在变异：17例位于后下间隔,1例位于下间隔近心尖处,2例于中、后间隔处.浦肯野电位和舒张期电位间存在面积约（1.5±0.4） cm2的交汇区,该处拖带和消融均取得成功.6例AVRT患者于后下间隔处记录到缓慢传导区,长度[12.0～28.7 （20.4±4.7） mm对11.8～ 20.3（16.1±3.3） mm,t=2.1,P=0.048]、面积[1.6～ 3.5（2.5±0.5） cm2对1.4～2.1 （1.8±0.3） cm2,t=3.0,P=0.006]显著小于ILVT组.结论 左心室传导系统和缓慢传导区存在多种变异,使ILVT折返机制更为复杂.     

大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症临床分析

目的:分析大剂量丙种球蛋白(IVIG)治疗婴幼儿特发性心内膜弹力纤维增生症的临床疗效。方法:回顾性分析1984年8月至2010年2月,在首都医科大学附属北京安贞医院小儿心脏科住院的53例特发性婴幼儿心内膜弹力纤维增生症患儿临床资料,应用大剂量丙种球蛋白治疗组患儿24例(2 g.kg-1.次-1,分2 d使用,每月1次连用3次,以后每3个月重复1次)未使用大剂量丙种球蛋白治疗组患儿29例作为对照。对比两组治疗前后1年的临床疗效。结果:两组患者发病月龄、发病时左心室射血分数、舒张末径之间差异无统计学意义(P>0.05),治疗后1年左心室射血分数、舒张末径的改善情况,两组之间差异无统计学意义(P>0.05),但IVIG组环磷酰胺的使用率明显低于非IVIG组,分别为(33.3%vs.62.1%,χ2=4.339,P=0.037)差异有统计学意义。结论:大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症有一定临床疗效,并能显著降低环磷酰胺的使用率。     

Regional uptake of iodine-125-metaiodobenzylguanidine in the rat heart

Regional uptake of iodine- 125-metaiodobenzylguanidine ([¹²⁵I]MIBG) was evaluated in normal (n=12) and reserpinized (n=12) rat hearts. At 15 min and 1, 3 and 6 h after injection of [¹²⁵I]MIBG, tissue activities were calculated for the right ventricular myocardium (RV), the whole left ventricular myocardium (whole LV), the epicardial layer of the left ventricular myocardium (Ep LV), the endocardial layer of the left ventricular myocardium (En LV), the basal segment of the left ventricular myocardium and the apical segment of the left ventricular myocardium. The uptake of [¹²⁵I]MIBG at 6 h after injection in the normal rat heart was higher in RV than in whole LV (0.45 ± 0.09% vs 0.30 ± 0.06% kg dose/g), and in Ep LV than in En LV (0.32 ± 0.07% vs 0.25 ± 0.05%). In the reserpinized rat heart, the difference in the uptake between Ep LV and En LV was smaller. This suggests that the difference in the regional [¹²⁵I] MIBG uptake might reflect different intravesicular uptake in the layers of the heart. To our knowledge, the low uptake in the endocardial layer was a new finding which seems to indicate a difference in innervation between the epicardial and endocardial layers of the left ventricle in the rat heart. Autoradiographic study also showed the low uptake of [¹²⁵I] MIBG in the endocardial layer, while homogeneous perfusion was observed with thallium-201, supporting the tissue uptake study. Thus, the endocardial and epicardial layers of the left ventricle in the rat heart were considered to be differently innervated.     

婴幼儿心内膜弹力纤维增生症的诊断(附43例报告)

目的：进一步认识心内膜弹力纤维增生症诊治的意义。方法：对本院儿科收治的心内膜弹力纤维增生症43例，于住院后作心电图、X线心脏摄片及二维和／或多普勒心脏超声检查。结果：本组43例均有心脏形态学改变，表现心脏增大以左心室增大为主；超声心动图尚显示心内膜呈弥漫或不规则增厚达3～6mm。结论：1岁以内患儿突发心力衰竭者，超声心动图检查必不可少，对临床诊断有肯定价值。     

A quantitative, randomized study evaluating three methods of mesenchymal stem cell delivery following myocardial infarction.

AIMS: Mesenchymal stem cells (MSCs), rare bone marrow-derived stem cell precursors of non-haematopoietic tissues, have shown promise in potentially repairing infarcted myocardium. These and similar cell types are being tested clinically, but understanding of delivery and subsequent biodistribution is lacking. This study was designed to quantitatively compare MSC engraftment rates after intravenous (IV), intracoronary (IC), or endocardial (EC) delivery in a porcine myocardial infarction (MI) model. METHODS AND RESULTS: Allogeneic, male MSCs were cultured from porcine bone marrow aspirates. Iridium nanoparticles were added during culturing and internalized by the MSCs. An MI was induced in female swine (27-40 kg in size) by prolonged balloon occlusion of the mid-left anterior descending artery. Animals (n = 6 per group) were randomized to one of three delivery methods. Cellular engraftment was determined 14+/-3 days post-delivery by measuring ex-vivo the iridium nanoparticle concentration in the infarct. Confirmation of cellular engraftment utilized both DiI and fluorescence in situ hybridization (FISH) labelling techniques. During MSC infusion, no adverse events were noted. However, following IC infusion, half of the pigs exhibited decreased blood flow distal to the infusion site. At 14 days, the mean number of engrafted cells within the infarct zone was significantly greater (P< or =0.01) following IC infusion than either EC injection or IV infusion and EC engraftment was greater than IV engraftment (P< or =0.01). There was less systemic delivery to the lungs following [EC vs. IV (P = 0.02), EC vs. IC (P = 0.06)]. Both DiI and FISH labelling demonstrated the presence of engrafted male MSCs within the female infarcted tissue. CONCLUSION: IC and EC injection of MSCs post-MI resulted in increased engraftment within infarcted tissue when compared with IV infusion, and IC was more efficient than EC. However, IC delivery was also associated with a higher incidence of decreased coronary blood flow. EC delivery into acutely infarcted myocardial tissue was safe and well tolerated and was associated with decreased remote organ engraftment with compared with IC and IV deliveries.