特发性肌病的病理分型及其与患者功能障碍和预后的关系

背景：肌肉活检病理是诊断特发性炎症性肌病(inflammatory myopathies，IM)的重要手段。然而目前，对于肌肉病理改变与患的功能障碍及预后的联系，缺少大型系统性的研究。目的：提出并探讨IM的肌肉活检病理类型及其与患的功能障碍及预后的联系。设计：以诊断为依据，回顾性研究。单位：济南市中心医院神经内科及山东大学齐鲁医院神经内科。对象：山东大学齐鲁医院神经病理实验室的630例肌活检标本中检出病理确诊和拟诊的炎症性肌病。方法：回顾性分析IM的病理改变，归纳出各病理类型的特点。随访并评估IM患的预后情况。主要观察指标：①肌肉病理改变，主要包括纤维坏死的程度、坏死的分布特点和炎症细胞浸润的情况。②临床特点(吞咽、肌痛、肌无力、皮肤损害等)及其愈后。结果：共检出IMll9例，其中重、中、轻度坏死性肌炎分别为11例、19例和27例，束周坏死／萎缩性肌炎20例，无炎细胞浸润的坏死性肌炎22例，间质性肌炎11例，肌筋膜炎3例，包涵体肌炎4例，肉芽肿性肌炎和增殖性肌炎各1例。其中72例获得随访资料，轻度坏死性肌炎和束周坏死／萎缩性肌炎的好转和治愈的百分比高于中、重度坏死性肌炎。间质性肌炎和无炎细胞浸润的坏死性肌炎高于重度坏死性肌炎。结论：IM的病理类型对判断预后具有重要的参考价值。     

杆状体肌病的临床病理特征与预后分析

杆状体肌病(NM)为一种少见的先天性肌病,报告1例成人晚发肌病患者的临床表现、实验室及肌肉病理检查,电镜下观察到多数肌纤维中含有大量源于Z线的杆状体,确诊为NM。结合国内外文献探讨NM的临床与病理特点,对于有近端肢体无力特别是合并有免疫系统疾病的患者行MGT染色或电镜检查将避免NM的误诊,早期功能锻炼可改善此类患者的预后。     

经皮肾穿刺活检38例临床与病理关系分析

中国医科大学附属第二医院2006—02～2006—06共行肾活检38例，现分析如下。 1临床资料 1．1一般资料 本组男16例，女22例，年龄16～65（平均27）岁。临床诊断为急性及慢性肾炎共18例，原发性肾病综合征5例（其中2例合并慢性肾衰），糖尿病肾病1例，紫癜性肾炎3例，可疑乙型肝炎病毒相关性肾炎2例，IgA肾病1例，     

线粒体肌病与脑肌病2例报道

目的报道线粒体肌病和眼肌型线粒体脑肌病的临床和肌肉活检组织的病理诊断特点。方法应用常规、酶组织化学和电子显微镜对此病例进行形态学观察。结果改良GOMORI三色染色见不整边纤维或破碎红纤维。细胞色素氧化酶(CCO)的酶活性消失,琥珀酸脱氢酶(SDH)染色病变处酶活性增强。结论改良GOMORI三色染色应作为诊断线粒体疾病的常规染色。COX,SDH染色可定位阳性肌纤维的部位。     

关于混合性结缔组织病与多发性肌炎肌活检特点的对比性研究

混合性结缔组织病（ＭＣＴＤ）是一种少见病，本文作者通过对ＭＣＴＤ与多发性肌炎（ＰＭ）肌肉活检病理表现特点的对比研究，进一步寻找ＭＣＴＤ诊断的客观依据，以提高临床诊断的准确性。１资料与方法本组ＭＣＴＤ病人４例，全部为女性，年龄２３～５０岁，病史１～１５...     

线粒体肌病与脑肌病2例报道

目的 报道线粒体肌病和眼肌型线粒体脑肌病的临床和肌肉活检组织的病理诊断特点。方法 应用常规、酶组织化学和电子显徽镜对此病例进行形态学观察。结果 改良GOMORI三色染色见不整边纤维或破碎红纤维。细胞色素氧化酶(CCO)的酶活性消失，琥珀酸脱氢酶(SDH)染色病变处酶活性增强。结论 改良GOMORI三色染色应作为诊断线粒体疾病的常规染色。COX，SDH染色可定位阳性肌纤维的部位。     

肾小球疾病87例肾活检临床病理分析

肾活组织检查用于肾疾病的诊断已有50余年,对肾脏疾病具有较高的诊断价值[1],对肾脏疾病的治疗、判断预后以及对肾脏病学的发展也有重要意义[2].目前临床广泛采用经皮肾活检诊断不明原因弥漫性肾疾病[3],此项技术对疾病的治疗及判断预后具有重要意义.我院2008年6～12月对87例肾小球疾病行肾活检,现将结果报告如下.     

肝实性占位超声回声特性的病理基础

目的 了解决定肝实性占位病变超声回声特性的病理组织学基础.方法 120例患者的37个高回声结节、91个低回声结节在超声引导下接受穿刺组织活检,并进行病理组织学分析.结果 128个结节中包含了各种良、恶性病变.高回声的共同组织学特点为:含有非液化性坏死区、脂肪空泡、透明细胞、血窦或窦周隙扩张,上述结构的最小径>0.03 mm且大片融合形成直径>0.40 mm的集落簇.低回声病灶细胞排列致密无上述结构.结论 病灶内所含有的脂肪空泡、窦周隙的直径是构成回声特性的组织病理基础.     

肾穿刺活检病理956例临床分析

肾活检病理检查对各种肾病的病理诊断、指导治疗、评估预后有着重要意义。自从1951年瑞典、丹麦医师使用静脉肾盂造影定位,首次经皮肾穿刺活检（简称肾活检）成功,肾活检逐渐开展;1985年更加准确的超声实时定位及引导经皮肾穿刺活检,使肾活检相关并发症显著降低,肾活检得到广泛发展。     

623例膀胱镜活检病理分析

目的分析因膀胱病变行膀胱镜活检患者的病理类型构成,探讨膀胱镜检查的临床价值。方法复习我院623例膀胱病变患者经膀胱镜活检组织病理资料。结果良性肿瘤76例,占12．2％,均为尿路上皮乳头状瘤。恶性肿瘤291例,占46．7％,其中尿路上皮癌254例,腺癌17例,鳞癌6例,转移癌13例,横纹肌肉瘤1例。膀胱黏膜慢性炎症140例,上皮增生50例,分别占22．5％、8．0％;而腺性膀胱炎55例,正常膀胱黏膜8例,前列腺组织3例。结论尿路上皮性肿瘤仍是最常见的膀胱疾病,其次是膀胱炎性病变。膀胱恶性肿瘤以尿路上皮癌最常见,膀胱腺癌、鳞癌或转移癌等比较少见。膀胱镜活检安全、简便,具有重要的临床应用价值。     

严重脓毒症患者血管内皮生长因子水平及其与预后的关系

目的 通过检测严重脓毒症患者血清血管内皮生长因子(vascttlar endothelial growth Factor,VEGF)水平,探讨其与疾病严重程度及预后的关系.方法 采用对照研究方法,收集2006-07-2007-11期间天津医科大学第二医院ICU收治的严重脓毒症病例,根据28 d转归分为生存组和死亡组,对照组为健康人,严重脓毒症患者均于发病后1,3,7 d检测血常规、血气分析、血生化、G-反应蛋白、乳酸,计算APACHE Ⅱ评分,采用ELISA方法检测血清VEGF水平,统计学分析计量资料采用F检验,相关分析用spearman相关系数,死亡危险因素筛选采用Logistic回归分析.结果 共入选病例29例,生存16例,死亡13例,对照31例,对照组VECF为(78.77 4±8.15)pg/mL,生存组1,3,7 d VEGF为(210.47±59.40)pg/mL,(161.79±32.58)pg/mL,(85.33±12.13)pg/mL,其峰值出现在第1天,随病程进展VEGF逐渐下降,第7天与对照组比较差异无统计学意义(P>0.05);死亡组1,3,7 d VEGF为(324.12±44.35)pg/mL,(185.40±30.92)pg/mL,(273.32±55.23)pg/mL,其峰值亦出现在第1天,随病程进展第7天VEGF下降不明显,与对照组比较差异有统计学意义(P<0.01),VEGF与APACHE Ⅱ评分呈正相关(rs=0.510,P<0.01),均为影响预后的死亡危险冈素.结论 严重脓毒症患者VEGF水平在发病早期升高,其与APACHEⅡ评分有显著的相关性,是影响预后的死亡危险因素之一.     

病理诊断中与大肠癌预后密切相关的几个问题

大肠癌的首选治疗手段是手术切除,对切除的手术标本进行正确的病理诊断是进一步治疗及评估患者预后的重要依据.本文结合文献对与大肠癌预后密切相关的几个问题,如：组织学类型及分级、TNM分期病理诊断原位癌的概念、浆膜浸润、淋巴结转移、远隔转移及切缘等进行讨论.     

Endothelial dysfunction in Buerger's disease and its relation to markers of inflammation

BACKGROUND: Buerger's disease (BD) is a segmental occlusive vascular disease. The aim of this study was to detect functional changes in brachial artery and asymptomatic morphological changes in extra-cranial carotid arteries not affected by the disease process and to assess markers of inflammation and endothelial damage. MATERIALS AND METHODS: Fourteen patients in the remission phase of BD and the same number of age- and sex-matched healthy controls were included in the study. The capability of endothelium-dependent (flow-mediated) and endothelium-independent dilation of the brachial artery and intima-media thickness of the carotid arteries were measured using high-resolution ultrasound. Laboratory parameters of endogenous fibrinolytic activity, inflammation and endothelial dysfunction were also measured. RESULTS: Patients with BD had a diminished capability of endothelium-dependent vasodilation and higher levels of some circulating markers of inflammation, such as leukocytes, C-reactive protein, intercellular adhesion molecule-1 and E-selectin. Intercellular adhesion molecule-1 levels were related to some of the inflammatory markers (sedimentation rate, C-reactive protein, alpha2-globulins and fibrinogen), while E-selectin was correlated with decreased endogenous blood fibrinolytic activity. Endothelium-dependent vasodilation was in negative correlation with the relative share of neutrophil granulocytes. There were no significant differences in intima-media thickness between patients with BD and controls. CONCLUSIONS: Our study has expressed generalized functional arterial disorder in patients with BD not accompanied by any measurable morphological changes of the carotid arterial wall. Functional deterioration of brachial artery could be related to increased levels of various inflammatory markers--the process which is most probably the basic pathogenetic mechanism of the disease.     

An overview of podiatric biomechanics theory and its relation to selected gait dysfunction

This paper introduces the ‘foot function’ approach used by podiatrists in the treatment of lower limb musculoskeletal dysfunction. The aim is to demonstrate how podiatric theory has evolved its own perspective of mechanisms relating to normal and abnormal locomotion. Three individual podiatric paradigms are discussed, and a further theory allowing a working simplification of theory is introduced. Finally, an example of gait abnormality is discussed in relation to podiatric and physiotherapy perspectives.An insight into podiatric theory should enable therapists working within this field to develop a more holistic and multidisciplinary approach. It is the view of the authors that a closer working relationship between physiotherapists and podiatrists with an interest in movement dysfunction provides a better quality service for appropriate patients.     

Prevalence of ventricular discordance and its relation to functional capacity in idiopathic dilated cardiomyopathy

Introduction  Although left ventricular (LV) dilatation is the most distinguishing morphologic feature of idiopathic dilated cardiomyopathy (IDC), right ventricular (RV) dilatation may variably contribute to total cardiac enlargement. The prevalence and functional importance of the relative degree of left and right ventricular dilatation has not been comprehensively studied using cardiac magnetic resonance imaging (MRI). Methods  Our prospective study included 58 consecutive IDC patients with a LV ejection fraction <40% and NYHA functional class ≥2. MRI was performed with a 1.5 Tesla scanner for RV and LV dimensional and functional analysis. Cardiopulmonary exercise testing was used for evaluation of exercise capacity. Patients were grouped into tertiles based on the distribution of LV end-diastolic volume. Results  Compared to control subjects a considerable heterogeneity in the relative degree of left and right ventricular dilatation was noted in IDC patients. Within the entire patient group, a strong correlation between the degree of ventricular volume discordance and the extent of LV enlargement was observed (r = 0.8; P < 0.001). Tertile analysis revealed that the LV/RV volume ratio significantly differed in the three subgroups of patients (1.0 ± 0.3 vs. 1.5 ± 0.4 vs. 2.1 ± 0.9; P < 0.001). Only weak correlations between MRI data and patients’ functional capacity were found. LV ejection fraction was identified as the only independent predictor of maximum oxygen consumption in our setting. Conclusion  In IDC patients the degree of ventricular volume discordance is strongly related to the extent of left ventricular enlargement. However, comprehensive biventricular assessment of cardiac function by MRI adds little to our understanding of the cardiac mechanisms limiting exercise tolerance when compared to exclusive left ventricular measurements.     

骨桥蛋白在卵巢上皮癌组织中的表达及其与预后的关系

目的 探讨卵巢上皮癌组织中骨桥蛋白的表达及其与预后的关系.方法 应用免疫组织化学法测定骨桥蛋白在64例卵巢上皮癌组织、20例卵巢良性肿瘤组织及10例卵巢正常组织中的表达,分析其表达与临床病理分期及其与预后的关系.结果 64例卵巢上皮癌患者组织中骨桥蛋白表达阳性52例,骨桥蛋白在卵巢上皮癌组织中的阳性表达率[81.3%(52/64)明显高于卵巢良性肿瘤[15.0%(3/20)]及正常卵巢组织[10.0%(1/10)],差异有统计学意义(P均<0.01).骨桥蛋白的表达与卵巢上皮癌的分期、分化及有无腹水和淋巴结转移有关.骨桥蛋白阴性表达组患者的总生存时间(2年生存率为91.7%,3年生存率为83.3%)明显高于骨桥蛋白阳性表达组(2年为67.3%,3年为55.6%).结论 骨桥蛋白与卵巢上皮癌的发生、发展及其预后密切相关.     

隆突性皮肤纤维肉瘤病理诊断及预后的探讨

目的 探讨隆突性皮肤纤维肉瘤（DFSP）的病理诊断及临床病理参数，增殖细胞核抗原（PCNA）表达与预后的关系。方法 应用免疫组化方法对51例隆突性皮肤纤维肉瘤CD34、PCNA的表达进行检测。并结合病理形态学观察和随访分析。结果 所有DFSP均表达CD34，组织分级，浸润深度、局部切除范围与DFSP复合率显著相关，PCNA计数标记指数平均为41.7%，PCNA高表达组复发率显著高于PCNA低表达组。结论 CD34可作为DFSP与纤维肉瘤特异的鉴别诊断指标。组织分级，浸润深度及PCNA表达可作为判断DFSP预后的指标。扩大切除范围有助于减少复发。     

老年脑白质疏松与腔隙梗塞预后的关系

目的 :探讨脑白质疏松(LA)与腔隙梗塞(LI)预后的关系。方法 :将100例腔隙梗塞患者分为LI合并LA组(LI )及LI不合并LA组(LI-)。对两组患者卒中危险因素、卒中复发率、痴呆发生率、Barthel指数、病死率及死亡原因进行对比分析。结果 :随访结束时LI 组神经功能缺损评分、Barthel指数、卒中复发率、痴呆发生率及病死率与LI-组均有明显差异 ,P<0.01。结论 :LI合并LA者预后较差。