肝豆状核变性肝组织的电镜检查

目的：研究早期肝豆状核变性肝细胞超微结构改变及其诊断价值．方法：经皮穿刺活检肝组织进行常规透射电镜标本制备，用JEM-2000EX透射电镜观察．结果：肝豆状核变性肝细胞中线粒体可见明显异常，除常见的非特异性改变(线粒体肿胀、多形性、空泡化、基质颗粒数量增加或缺失)外，显而有特征性的改变包括线粒体的形态大小不一，线粒体体积增大形成巨大线粒体，并可见奇异线粒体．基质电子密度增高．由于膜间腔的不规则扩大造成外膜冗余，线粒体的嵴不规则，也可扩张，偶尔可见线粒体基质致密颗粒的空泡化．基质内可见结晶样包涵物，呈平行排列的纤维样杆状物．局灶性的肝细胞脂滴增多，溶酶体和颗粒状包涵物数目增多，可见特征性的多泡状包涵体．并可出现灶性肝细胞坏死和炎细胞浸润．结论：在早期溶酶体改变不明显时，多表现为线粒体的改变．因此，重视线粒体的改变对肝豆状核变性有着特殊的诊断意义．     

缺血性心肌病患者的救治

缺血性心肌病是指由于长期心肌缺血导致心肌局限性或弥漫性纤维化,从而产生心脏收缩和(或)舒张功能受损,引起心脏扩大或僵硬、充血性心力衰竭、心律失常等一系列临床表现的临床综合征。
　　缺血性心肌病的发病基础主要是由于冠状动脉粥样硬化性狭窄闭塞、痉挛甚至心肌内毛细血管网的病变,引起心肌供氧和需氧之间的不平衡而导致心肌细胞变性、坏死心肌纤维化心肌瘢痕形成,出现心力衰竭、心律失常和心腔的扩大,表现为充血性心肌病样的临床综合征,另外有少部分缺血性心肌病患者主要表现为心室肌舒张功能改变,心室壁僵硬度异常。缺血性心肌病患者,尤其是充血型缺血性心肌病,往往有多支冠状动脉发生显著性粥样硬化性狭窄。     

缺血性心肌病的临床分析

缺血性心肌病(Ischemic Cardiomgopathy，ICM)是由于冠状动脉病变引起充血性心力衰竭和某些临床状态组成的综合在。以心脏扩大和充血性心力衰竭为主要表现。虽然按国际规定的心肌病定义并不包括缺血性心肌病，但这一新疾病名称在临床上逐渐被采用，其临床表现与原发性扩张型心肌病(DCM)有很多相似之处，常易混淆，本文就此进行对比分析。     

内质网应激在糖尿病心肌病发病机制中的作用

糖尿病心肌病是由Rubler等首先提出的,随着研究的深入,人们逐步认识到糖尿病心肌病是由糖尿病引起的心脏结构和功能的改变,在病因上独立于缺血性疾病、高血压及其它可能原因引起的心肌病变.临床上,早期表现为心肌的顺应性下降和舒张功能不全,晚期表现为心肌的收缩功能不全,进而发展成充血性心力衰竭[1].糖尿病心肌病发病机制多样,主要包括胰岛素抵抗、能量代谢紊乱、氧化应激、线粒体损伤等.最近的研究表明[2-3],内质网应激(ERS)贯穿于糖尿病心肌病的发生、发展过程,与诸多病理因素、病理过程存在密切联系.本文从内质网应激角度对糖尿病心肌病发病机制中所涉及到的病理因素及病理过程做一综述,以期增强对糖尿病心肌病发病机制的认识.     

酒精性心肌病的诊治研究

酒精性心肌病（alcoholic cardiomyopathy,ACM）是指长期嗜酒引起的心肌病变,以心脏扩大、心律失常和充血性心力衰竭为特征的心肌病,属继发性心肌病之一。     

糖尿病合并心肌病20例临床分析

糖尿病心肌病(DCM)是指糖尿病患者心冠状动脉及心脏瓣膜病变,无先天性心脏病,高血压心脏病及酗酒情况下,罹患充血性心力衰竭和心律失常。考虑存在一种特异心肌病。1974年Hamby等首次提出糖尿病心肌病的概念,该病由糖尿     

小儿充血型原发性心肌病25例临床分析

近年来,临床上把主要累及心肌的非血管性、非瓣膜性心脏病统归为“心肌病”。根据病因是否明确又分为原发性(特发性)心肌病和继发性心肌病。依据心脏病理生理改变原发性心肌病再分为充血型、梗阻型和限制型。本文选择了我院近十年来所见25例小儿充血型原发性心肌病加以报告。一、病例选择标准 (一)持续性心脏增大,尤其是X线示心影呈球形增大,而无其他原因可寻者;     

糖尿病与高血压性心脏病

糖尿病与高血压两种病变经常同时存在于同一病人身上,导致心脏的结构和功能受损,加速心血管病的发展,造成心血管性死亡。临床和实验研究证实同一病人患有糖尿病和高血压发生心肌病比单独一个病变时严重。糖尿病性心脏病长期患糖尿病可引起心脏结构及功能损伤,导致缺血性心脏病,心肌病,和充血性心力衰竭。缺血性心脏病:糖尿病对于冠心病的发展是一个独立的危险因素。糖尿病人的冠脉血管     

糖尿病心肌病发病机制的研究进展

正糖尿病心肌病(DCM)是一种独立的糖尿病并发症,目前研究已证实它是一类区别于其他心肌病的特殊的心肌病,是指患者有明确的糖尿病病史,在排除心血管疾病(如冠心病、高血压病、心脏瓣膜病变以及其他原发性的心肌病等)后,因糖尿病而引起的以心肌细胞功能代谢紊乱、心脏微血管病变、心肌纤维化及心肌间质增生等病理生理改变的一种广泛的心肌结构异常。该病早期表现为舒张功能不全,晚期则以收缩期的功能障碍、左     

糖尿病性心肌病

糖尿病引起的心脏病变,以往都被当作是大血管病变引起的冠心病来处理,而忽视了微血管病变的存在。本文将以微血管病变和心肌代谢障碍为中心,对糖尿病性心肌病进行论述。1　概念1960年,Blumenthal[1]等人在对糖尿病人的心脏进行解剖时,首次发现心肌内有微血管病变。1972年Rubler[2]等人在4例有充血性心衰冠状动脉而排除硬化,心瓣膜病、高血压、先天性心脏病及酒精性心脏病的糖尿病患者进行尸解时,发现其心肌内小血管狭窄心室壁及小血管壁增厚,管腔狭窄,间质呈弥漫性纤维化,而室壁外的冠状血管无明显狭窄,Rubler称这改变为糖尿病情心肌病(di…     

Histological and ultrastructural evaluation of cardiac lesions in idiopathic cardiomyopathy in dogs

Described are pathological studies of eight dogs which died in congestive heart failure, with a clinical diagnosis of congestive cardiomyopathy. Examination revealed marked dilatation and enlargement of all four chambers of the heart. The ventricular walls were thin with small atrophic papillary muscles. Histological studies on the myocardium revealed scattered areas of myocardial necrosis, especially around the papillary muscles of the left ventricle and random scattered areas of fibrosis. Electron microscopic studies revealed fewer and disoriented myofibrils, myocytolysis, increased numbers of mitochondria with swelling and inclusions, increased glycogen granules and increased numbers of lysosomes, lipofuscin granules and lipid droplets. Mild Z-band abnormalities were found throughout the myofibers.     

小儿克山病心肌病变的超微结构改变

本文报告10例小儿克山病心肌病变的电镜观察结果。看到受累的细胞器主要是心肌线粒体、外膜系统和肌原纤维等。其中以线粒体的形态改变最为突出。线粒体的主要变化是肿胀、基质清亮、嵴严重破坏、基质电子致密物质沉着和线粒体数量增多等。线粒体改变具有广泛性、严重性和早发性的特点。还描述了两种形态改变的线粒体。认为心肌线粒体的改变在克山病发病机制中可能具有原发性意义。     

MITOCHONDRIAL MYOPATHY AND MITOCHONDRIAL ENCEPHALOMYOPATHY

This paper is the first report of mitochondrial encephalomyopathy and mitochondrial myopathy diagnosed in China. It includes l case of mitochondrial encephalomyopathy with lactic acidemia and stroke-Iike episodes (MELAS), 5 cases of mitochondrial myopathy with skeletal muscles predominantly involved, and 2 cases of mitochondrial myopathy with external ocular muscles predominantly involved. The diagnosis was confirmed by muscle biopsies which revealed the "ragged-red-fiber" CRRF) in modified Gomori trichrome CMGT) stain, accumulation of lipie droplets in oil-red-O stain in frozen sections, and aggregates of abnormal mitochondria with complex paracrystalline inclusions and distorted cristae and osmiophilic dense bodies in their matrix in electron microscopy.     

心肌缺血-再灌注损伤的超微结构变化

目的：观察犬急性心肌缺血－再灌注损伤的早期超微结构改变,探讨急性心肌缺血－再灌注损伤所致急死的形态学基础。方法：复制犬急性心肌缺血－再灌注损伤动物模型,在透射电镜下观察心肌纤维、线粒体、内质网和微血管结构变化。结果：缺血30min再灌注120min,心肌细胞线粒体轻度肿胀,基质内可见絮状致密体;缺血60～90min再灌注120min,心肌细胞线粒体嵴断裂,基质内出现絮状致密体和颗粒状致密体。微血管内皮细胞肿胀,血管腔狭窄。结论：再灌注可使缺血30min心肌超微结构得到部分恢复,加重缺血60～90min心肌超微结构改变。     

Immunological results in myocardial diseases.

Immunological studies have shown new diagnostically important changes in alcoholic and viral myocarditis, as well as in congestive cardiomyopathy. Increased heart size correlated with the degree of congestive heart failure, as well as with negative immunofluorescence and an increased IgA concentration in the serum. These findings may serve as a diagnostic aid in patients with myocardial disease due to alcohol abuse. Viral heart disease is characterized by a variety of symptoms and nuclear antibodies (IgM) can be of help in the differential diagnosis. Heart muscle tissue of patients with congestive cardiomyopathy preferentially binds IgG and IgA. In addition to the other changes these findings are of diagnostic importance. It seems likely that results similar to those obtained for humoral antibodies in congestive cardiomyopathy will apply in the correlation of the haemodynamic status of the patients. The pathophysiological implication of these findings is not clear at present, but the evolution of congestive cardiomyopathy appears to be associated with binding of immunoglobulin to the myocardium, as well as with humoral antiheart antibodies.     

Electron microscopic studies of microvasculature and sympathetic nerve fibers in dilated cardiomyopathy

OBJECTIVE: To investigate the ultrastructural pathological alterations of the microvasculature and nerve fibers in the endomyocardial biopsied specimens of the left ventricular myocardium obtained from patients with idiopathic dilated cardiomyopathy and chronic heart failure. METHODS: Transmission electron microscopic observations of endomyocardial biopsied specimens of the left ventricular myocardium were carried out in 10 patients with idiopathic dilated cardiomyopathy and chronic heart failure. RESULTS: Various degrees of ultrastructural pathological alterations in the microvessels and sympathetic nerves in the diseased myocardium were consistently demonstrated in idiopathic dilated cardiomyopathy. In addition, abnormal accumulation of collagen tissue and edematous fluid were often seen in the interspace between myocardial cells and nerve endings and capillaries. CONCLUSIONS: Based on the ultrastructural pathological findings in this study, we consider that all the structures forming the muscle cells and the tissues around them, namely the microvessels and nerves may participate in the pathological process in the course of dilated cardiomyopathy. The damage of microvasculature and sympathetic nerves resulting from the underlying disease processes are considered to be an important pathogenetic mechanism responsible for progressive development of myocardial degeneration and dysfunction throughout the course of the disease. It is hoped that our data may provide some insights into the understanding of the role of microcirculation and sympathetic nerves in the etiopathogenesis of dilated cardiomyopathy.     

心内膜心肌活检诊断价值的临床研究

对90例心脏病患者进行心内膜心肌活检(EMB)95例次,成功率96.8%,临床诊断原因不明心律失常44例,各型心肌病24例,疑诊心肌炎10例,先心并心律失常4例,缩窄性心包炎5例,二尖瓣脱垂3例,经光、电镜检查确定诊断72例(80%)。其中心肌炎44例,扩张型心肌病19例、肥厚型1例,限制型4例,炎性心肌病4例。排除诊断3例(3.4%),无助于诊断15例(16.6%)。全组均无严重并发症,作者认为EMB对心肌疾病(尤其对心内膜心肌纤维化病)及心肌炎有较大诊断价值的新技术,值得推广。     

用透射电镜和免疫电镜的方法研究人类乳头瘤病毒与尖锐湿疣的关系

用透射电镜观察了30例尖锐湿疣的超微病理变化。多数尖锐湿疣存在空泡样变、双核、异型核、基质团及大量肿胀线粒体现象。但没有找到成熟的HPV颗粒。空泡样变可能是肿胀的线粒体破裂或胞浆基质团损耗所致。用金标免疫电镜法检出尖锐湿疣的HPV抗原阳性率为60％,而用酶标免疫电镜法其阳性率为47％。前法较后法敏感。大多数HPV抗原阳性细胞位子颗粒层和角化层。     

阻断心淋巴干后心肌亚微结构变化的研究

本文对阻断狗心淋巴干后心肌亚微結构和心电图改变进行了研究。主要结果如下:心电图:心率加快,T波倒置与ST段抬高;透射电镜显示心肌间质水肿、胶原纤维增生、毛细血管壁和闰增厚、心肌细胞膜破裂、线粒体肿胀、肌原纤维扭曲、肌丝断裂,模糊甚至溶解消失、肌浆网扩张、溶酶体增多,但糖原颗粒明显减少。本研究结果进一步证实了Solti(1989)提出的淋巴郁滞性心肌病的病因学概念是正确的。     

家兔心肌自溶的超微结构变化

作者对离体家兔心肌自溶的超微结构进行了观察。结果表明糖原颗粒减少,核和线粒体均出现变化、线粒体表现肿胀,基质密度降低,基质内出现絮状致密体。随自溶时间延长,絮状致密体体积增大,数量增多。上述变化有助于死亡时间的判定。