应用多普勒组织成像评价肥厚型心肌病左室收缩功能

目的 :应用多普勒组织成像脉冲速度模式测量二尖瓣环收缩速度 ,以评价肥厚型心肌病患者左室收缩功能的改变。材料和方法 :对肥厚型心肌病及正常人分别测量二尖瓣环各点收缩期峰值速度。结果 :肥厚型心肌病患者与正常组二尖瓣环收缩期峰值速度有显著性差异 ,均较正常组减低。结论 :肥厚型心肌病患者左室长轴收缩功能减低。     

595nm激光对兔耳瘢痕成纤维细胞增殖与凋亡的影响

目的：探讨595nmVbeam激光照射对增生性瘢痕动物模型伤口愈合过程中成纤维细胞增殖与凋亡的影响。方法：成年大耳白兔20只,建立兔耳腹侧面增生性瘢痕模型,对比研究在瘢痕形成过程中595nmVbeam激光照射对兔耳瘢痕成纤维细胞的影响,采用免疫组化方法检测增殖细胞核抗原（PCNA）蛋白表达和细胞凋亡的原位检测。结果：兔耳增生性瘢痕经595nmVbeam激光照射后,按不同时间段取材进行免疫组化染色并与对照组比较,高倍镜下观察结果,显示PCNA蛋白表达明显减弱,细胞凋亡增加。结论：595nmVbeam激光照射可抑制兔耳增生性瘢痕成纤维细胞的增殖过程,诱导细胞凋亡。应用595nmVbeam激光预防和治疗瘢痕是可行的。     

Holmes-like tremor of the lower extremity following brainstem hemorrhage.

Holmes tremor is an arrhythmic, 2- to 5-Hz resting, postural, and kinetic upper extremity movement disorder that occurs weeks to months after acute mesencephalic pathology. We present a patient who developed tremor in three body parts postbrainstem hemorrhage with subsequent hypertrophic olivary degeneration and discuss the relevant clinical evolution. Our case is unique because in addition to expected upper extremity and cervical dystonic head tremors, the patient also developed a severe lower extremity movement disorder, which we believe to be a form of Holmes tremor. Tremor involving the lower extremity in this setting has not been previously reported.     

肥厚型心肌病预后评估的研究进展

肥厚型心肌病是最常见的遗传性心脏病,其临床表现及预后极富多样性。该病患者的不良转归主要有:猝死、心力衰竭以及心房颤动所致的栓塞事件等。现就影响肥厚型心肌病预后的因素作一综述。     

肥大肝叶内胆管结石的处理

目的探讨在肝肥大-萎缩征中肥大肝叶内胆管结石的处理方法。方法回顾性分析我科1990年6月～2004年12月收治的103例肥大肝叶内胆管结石病人的临床资料，总结其手术治疗的原则和方法。结果全体病例均经手术治愈，术后残石率17．5％，效果优良率83．7％。结论肥大肝叶内的胆管结石，手术难度大，应根据病情选择手术方式，既要遵循肝胆管结石的治疗原则，又要保护赖以生存的肥大肝叶（段）。     

腹腔镜与开腹幽门环肌切开术的前瞻性比较研究

目的研究比较腹腔镜幽门环肌切开术（LP）和开腹幽门环肌切开术（OP）治疗先天性幽门肥厚性狭窄的疗效及免疫功能的变化。方法自2003年4月-2006年7月将72例先天性幽门肥厚性狭窄患儿随机分成二组（LP组及OP组各36例），比较二组麻醉时间、手术时间、术后进食时间及术后并发症，监测二组术前、术后第一天、术后第三天的外周血T淋巴细胞亚群、C反应蛋白（CRP）及白细胞介素-6（IL-6）和肿瘤坏死因子（TNF）的变化并行对比研究。结果二组麻醉时间、手术时间、术后进食时间差异无统计学意义，OP组术后并发症要略多于LP组，比较二组术前、术后第一天、术后第三天的外周血T淋巴细胞亚群、CRP及IL-6和TNF的变化差异无统计学意义。结论腹腔镜幽门环肌切开术（LP）和开腹幽门环肌切开术（OP）治疗先天性幽门肥厚性狭窄的临床效果相近，二组患儿免疫功能的变化无显著性差异。腹腔镜幽门环肌切开术是一种稳定、可靠的手术，对于治疗先天性肥厚性幽门狭窄的效果满意。     

Anti-mitochondrial antibodies in patients with dilated cardiomyopathy (anti-M7) are directed against flavoenzymes with covalently bound FAD

Anti-mitochondrial antibodies (anti-M7) in sera from patients with dilated cardiomyopathy and myocarditis recognize, besides mitochondrial antigens, bacterial sarcosine dehydrogenase. The common target antigen was identified as the covalently bound FAD of mitochondrial and bacterial flavoenzymes. Thus, anti-M7-positive serum reacted on Western blots exclusively with covalently flavinylated enzymes. The antigenic specificity of anti-M7 sera was reproduced by an antiserum raised in rabbits with 6-hydroxy- D -nicotine oxidase. The heart mitochondrial membrane antigen recognized by anti-M7 serum was identified as the flavoprotein subunit of succinate dehydrogenase, the antigens in rat liver mitochondrial matrix as the flavoenzymes dimethylglycine dehydrogenase and sarcosine dehydrogenase. Anti-M7 serum contained a specific anti-flavoenzyme antibody fraction. Nanomolar concentrations of FAD and riboflavin inhibited the immune reaction on Western blots and in ELISA, and incubation with FAD-agarose depleted the anti-M7 activity of the serum. N-terminally deleted dimethylglycine dehydrogenase proteins were only immunoprecipitated by anti-M7 sera when the FAD was covalently incorporated. An affinity constant (K_D) of 10^?8 M was established for the anti-flavoenzyme antibodies by competitive ELISA. Of patients with cardiomyopathy and myocarditis, 36% and 25%, respectively, were anti-flavoenzyme-positive by Western blot and ELISA, but only two of 15 patients with other heart diseases and none of 50 healthy controls.     

Incidence and Clinical Significance of Ventricular Late Potentials in Idiopathic Dilated Cardiomyopathy Compared to Coronary Artery Disease

Objective: This prospective study was designed to compare incidence and clinical significance of ventricular late potentials between patients with idiopathic dilated cardiomyopathy (IDC) and postinfarct patients (CAD) using exactly the same method of signal-averaged electrocardiography (SAECG) in both patient groups. Methods: Time-domain analysis of SAECG was performed in 120 consecutive patients with IDC, 120 patients with CAD, and 60 healthy controls. Ventricular late potentials were detected in 27 of 120 patients with IDC (23%) compared to 41 of 120 patients with CAD (34%; P < 0.05). Results: Ventricular late potentials were found in 2 of 60 controls (3%). During 15 ± 7 months follow-up, serious arrhythmic events occurred in 17 of 120 patients with IDC (14%) and in 13 of 120 patients with CAD (11%). The sensitivity of ventricular late potentials for future arrhythmic events was 35% for IDC compared to 77% for CAD (P < 0.05). The positive predictive value of late potentials detected by time-domain analysis was 22% for IDC versus 24% for CAD (P = ns). Conclusion: In this selected patient population with IDC and CAD, time-domain analysis of SAECG revealed a lower incidence of ventricular ate potentials in patients with IDC as compared to postinfarct patients. Whereas ventricular late potentials had a high sensitivity but a low positive predictive value for identification of postinfarct patients with serious arrhythmic events during follow-up, both sensitivity and positive predictive value of ventricular late potentials for future serious arrhythmic events were low in the setting of IDC.     

Isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium is a recently described anomaly. We report the first case of this anomaly presenting as a restrictive cardiomyopathy, and the first association of this entity with endocardial fibrosis.     

中国肥厚型心肌病患者血栓栓塞事件风险预测模型的构建研究

背景 血栓栓塞（TE）事件是肥厚型心肌病（HCM）的重要并发症。目前针对HCM患者TE事件的风险预测,仅国外学者构建了两个模型：HCM Risk-CVA及French HCM score,然而,现有研究发现HCM Risk-CVA模型对于中国HCM患者的临床价值较为有限。目的 本研究拟构建适合中国HCM患者的TE事件风险预测模型。方法本研究系回顾性队列研究,收集2010—2018年在四川大学华西医院就诊的537例HCM患者的病例资料。本研究通过电话随访或电子病历系统查询患者就诊记录,每6～12个月随访1次,直至出现终点事件或死亡或研究拟定的评估日期（2019-12-31）,终点事件定义为复合性TE事件。采用单因素和多因素Cox回归分析构建风险预测模型,并使用自助重抽样的方法进行内部验证。结果 537例患者中,24例患者有不同程度的数据缺失,最终纳入513例患者。中位随访时间为4.2(1.3,6.2)年,随访过程中42例（8.18%）发生TE事件,年发病率为2.10%[95%CI(1.47%,2.73%)]。根据多因素Cox回归模型构建TE事件风险预测模型,最终纳入年龄、既往TE事件、心...