平滑肌肉瘤的临床病理研究─—病理组织学、免疫组化及超微结构分析

本文报道对２６例全身多个部位的平滑肌肉瘤（ＬＭｓ）附病理组织学、ＨＨＦ３５及Ｄｅｓｍｉｎ免疫组化及部分病例还进行了超微结构特征研究。结果表明ＬＭＳ具有较典型的组织学特征：瘤细胞呈杆状，平行排列，胞浆内有肌原纤维。低分化的ＬＭＳ细胞异型性大，但仍具有上述特征。Ｄｅｓｍｉｎ标记ＬＭＳ的阳性率是６５％，ＨＨＦ３５的阳性率是９６％。两者有显著性差异。其它对照的多种梭形细胞肉瘤中，除恶性纤维组织细胞瘤中可有少数、单个的细胞呈ＨＨＦ３５阳性外，其它均不表达ＨＨＦ３５和Ｄｅｓｍｉｎ。因此ＨＨＦ３５是标记ＬＭＳ的敏感性高、特异性强的抗体。透射电镜观察ＬＭＳ具有特征性的密体和密斑存在。透射电镜观察也是确诊ＬＭＳ的途径之一。     

Primary restrictive cardiomyopathy in childhood

Restrictive cardiomyopathy is one of the rarest forms of cardiomyopathy in childhood and is associated with very poor prognosis (median transplant-free survival approximately 2 years). Little progress has been made in our understanding of the etiology of this condition, and most cases are considered ‘idiopathic’ in nature. No strong predictors of outcome have been identified and sudden death is common, even among those with few symptoms. Disappointingly, no effective therapies have emerged other than heart transplantation. Studies of familial cases suggest genetic and phenotypic overlap with other forms of cardiomyopathy, especially hypertrophic cardiomyopathy. Future research priorities include more careful delineation of phenotype(s), search for genetic etiologies and molecular mechanisms of disease, and studies to identify prognostic factors. Given the rarity of this condition, future studies will require collaboration among large numbers of centers following the models developed by the US Pediatric Cardiomyopathy Registry and the National Australian Childhood Cardiomyopathy Study.     

高脂血症家兔肾小球病变及绞股蓝总苷的干预作用

目的观察高脂血症早期家兔肾小球病变及绞股蓝总苷干预作用。方法高脂饮食法复制家兔高脂血症模型,8周后检查肾脏病理改变,免疫组化法检测肾小球中Desmin,α-SMA含量。结果正常组Desmin,α-SMA蛋白无明显表达;模型组两种蛋白表达均增强,肾脏系膜细胞轻微增多;绞股蓝总苷组表达高于正常组,低于模型组。结论高脂血症早期肾小球足细胞和系膜细胞受损,最终导致肾小球硬化。绞股蓝总苷可降低这种损伤。起到保护肾脏的作用。     

心房颤动犬心房肌细胞骨架重构及贝那普利的干预作用

目的 观察快速心房起搏心房颤动(房颤)犬心房肌细胞骨架重构及贝那普利的干预作用.方法 采用慢件快速左心房起搏建立房颤犬模型,分为假手术组(6只)、房颤组(7只)、干预组(6只).房颤组应用固定频率型起搏器,600次/min起搏6周;干预组于起搏前1周开始每日服贝那普利(1 mg/kg).实验结束时取材,HE染色测定心房肌细胞直径;Masson染色进行心房肌纤维化定量分析;免疫组织化学法检测结蛋白心脏原位蛋白分布及表达;RT-PCR方法测定心房肌组织β-微管蛋白、结蛋白mRNA表达水平.结果 假手术组、房颤组、干预组左心房肌细胞直径分别为(19.6 ±2.9)μm、(27.9 ±3.8)μm、(25.1 ±3.4)μm,右心房分别为(18.7 ±2.6)μm、(26.8 ±3.2)μm、(25.2 ±3.5)μm,房颤组、干预组左、右心房肌细胞直径均明显长于假手术组(均P＜0.01);左心房胶原容积分数(CVF)分别为(9.2±0.9)%、(16.9 ±1.1)%、(11.3 ±0.8)%,右心房CVF分别为(9.3±0.8)%、(15.7 ±2.3)%、(10.9 ±0.8)%,房颤组左、右心房CVF均高于假手术组(均P＜0.01),干预组均明显低于房颤组(均P＜O.01).免疫组化示房颤组结蛋自在细胞质内表达增多,而闰盘处表达不明显,房颤组左、右心房肌结蛋白吸光度值均高于假手术组(均P＜0.01),干预组均明显低于房颤组(均P＜0.01).房颤组左、右心房肌结蛋白、微管蛋白mRNA表达均高于假手术组(均P＜0.01);干预组房颤组(均P＜0.01).结论 快速心房起搏房颤犬心房肌细胞骨架发生重构且贝那普利对其有干预作用.     

SMA、Desmin、CD10和Vimentin在特殊类型子宫肿瘤诊断中的应用

[目的]探讨SMA、Desmin、CD10和Vimentin在特殊类型子宫肿瘤诊断中的应用价值。[方法]收集特殊类型子宫肿瘤76例（子宫内膜间质肉瘤20例,癌肉瘤17例,平滑肌肉瘤10例,非典型平滑肌瘤16例,上皮样平滑肌瘤13例）,免疫组化SP法检测各组织中平滑肌肌动蛋白（SMA）、结蛋白（Desmin）、CD10和波形蛋白（Vimentin）的表达。[结果]CD10与Vimentin在子宫内膜间质肉瘤和癌肉瘤中高表达,SMA和Desmin在非典型平滑肌瘤和上皮样平滑肌瘤中高表达,Vimentin在平滑肌肉瘤中高表达。[结论]SMA、Desmin、CD10和Vi-mentin在特殊类型子宫肿瘤的诊断中有重要意义。     

肌源性肿瘤的结蛋白,波型蛋白及肌动蛋白表达特性

采用ＡＢＣ－ＤＡＢ免疫组织化学方法分析３５问各种类型肌源性肿瘤的结蛋白、波型蛋白及肌动蛋白表达特性．结果显示恶性肿瘤细胞表达结蛋白能力下阶，可呈结蛋白表达阴性、波型蛋白阳性，或共同表达结蛋白与波型蛋白，此与恶性肿瘤细胞低分化或未分化密切相关。血管平滑肌瘤的中间丝表达呈现３种不问类型。肌动蛋白（Ａｌｐｈａ－Ｓｒ－１）显示对胚胎型横纹肌肉瘤的良好染色反应。     

Expression of desmin in normal and laser (HeNe) irradiated regenerating skeletal muscles in the toad (Bufo viridis) and the white rat

The expression of desmin was investigated using immunohistochemical methods in normal and low-energy laser (HeNe) irradiated regenerating rat and toad gastrocnemius muscles following partial excision in the former and cold injury in the latter. During the initial stages of regeneration, presumptive myoblasts immunoreacted to desmin antibodies while other mononucleated cells remained unstained. At a later stage the cytoplasm of the myotubes was intensely stained with anti-desmin. At all time intervals there were more mature myogenic structures in the injured zones of the laser irradiated rat or toad muscles than non-irradiated muscles as indicated by the positive immunoreactivity to desmin. It is concluded that desmin immunostaining provides additional information on the role of histological structures during the regeneration process. The process of skeletal muscle regeneration follwing injury is markedly promoted by low-energy direct laser irradiation during the regeneration process. The results also indicate that the rate of differentiation of myoblasts from undifferentiated stem cells is enhanced by the laser irradiation.     

大鼠肝大部切除后再生时贮脂细胞的动态变化

目的 研究大鼠肝大部切除后再生时贮脂细胞的动态变化。方法 用结蛋白免疫组织化学法显示肝贮脂细胞并计量，以图像分析仪测灰度值。结果 正常肝小叶内贮脂细胞呈网架状分布，肝大部切除后再生时贮脂细胞数量递减，至第５ｄ时为正常肝的１／３。结论肝大部切除后再生时贮脂细胞动态变化明显不同于肝中毒等损伤后的增殖变化。     

Muscle fibre type composition in distal myopathy (Welander). An analysis with enzyme- and immuno-histochemical, gel-electrophoretic and ultrastructural techniques

The myopathic muscle of distal myopathy (Welander's disease), the dominantly inherited neuromuscular disorder which occurs frequently in Sweden, has been characterized by electron microscopy, enzyme- and immuno-histochemistry (using antibodies against embryonic, neonatal, fast and slow myosin, and against the muscle-specific intermediate filament protein, desmin), and with gel electrophoretic techniques. Of special interest is the fact that the ultrastructural appearance of the fibres with regard to M- and Z-band structures does not fit the proposed classification criteria for ultrastructural fibre typing of normal human muscle. Furthermore, contrary to previous results, we conclusively demonstrate that the predominating fibres are of a slow-twitch type. Unexpectedly, we also observed that embryonic and neonatal myosin was expressed in some residual fibres. This emphasises the importance of supplementing stains to demonstrate activity of ATPase with myosin immuno-histochemistry in order to improve understanding of fibre type characteristics in myopathic muscles. The origin of the myopathic muscle fibres in distal myopathy could not be definitely determined, but it is suggested that neurogenic disturbances play an important part in the pathophysiology of Welander's disease.