三种自身免疫性肝病的临床特点比较

目的分析比较三种自身免疫性肝病(ALD)的临床表现、血清学和病理学特点及诊断意义。方法回顾性总结北京协和医院1998—2002年78例自身免疫性肝病患者的临床资料。结果1．临床表现：ALD以中年女性多见，在自身免疫性肝炎(Am)和原发性硬化性胆管炎(PSC)患者中发热明显多于原发性胆汁性肝硬化(PBC)，皮肤瘙痒在PSC最为突出；2．实验室检查：PBC和PSC以ALP、GGT显著升高为主，AIH以ALT、AST升高为主，血清抗体检测在AIH中抗核抗体(ANA)阳性占80％，抗线粒体抗体M：亚型(AMA-M：)对PBC最具特异性；3．影像学检查：PSC患者80％有肝内外胆管的僵硬、狭窄、变细及局部扩张；4．病理学：共有41例行肝穿刺病理活检，病理结果提出明确诊断者17例(41．5％)。结论ALD临床表现复杂多样但也各有特点，血清抗体检测有助于AIH与PBC的诊断与鉴别诊断。胰胆管影像检查有助于PSC的确诊。病理学检查的诊断价值有一定的局限性。     

自身免疫性肝病临床及免疫学特征

目的 分析自身免疫性肝病的临床特征和免疫学特点,以提高对该类疾病的认识和诊断水平.方法 对164例自身免疫性肝病患者的临床症状、体征及免疫学检查资料进行回顾性分析.结果 原发性硬化性胆管炎(PSC)多发于青年男性,自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及其重叠综合征(AIH-PBC)多发于中年女性;临床表现上AIH、PBC、PSC、AIH-PBC黄疸发生率分别为84%、78%、90%和67%,皮肤瘙痒的发生率分别为43%、56%、81%和60%.但AIH、PBC和PBC-AIH患者之间的年龄、性别、黄疸、皮肤瘙痒等症状无显著性差异(P均＞0.05).AIH首诊正确诊断率为8%(6/77),PSC为9%(1/11),PBC为13%(6/46).从发病到确诊PBC平均为38月,AIH为46月,PSC为31月,PBC-AIH重叠综合征确诊最难,平均需51月,常误诊为可能的AIH或单纯PBC.AIH、PBC、PSC和PBC-AIH肝外自身免疫性疾病的发生率分别为47%、11%、27%和24%,各自身免疫性肝病患者均有较高的自身抗体发生率.结论 自身免疫性肝病在临床上并不少见,患者常伴发肝外自身免疫性疾病及较高的自身抗体发生率;诊断需综合临床、生化、自身抗体和病理组织学等指标.     

自身免疫性肝病的临床及病理研究现状

自身免疫性肝病(autoimmune liver disease,ALD)包括自身免疫性肝炎(autoimmune hepatits,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC),以及这三种疾病中任何两者之间的重叠综合征(overlap syndrome,OS)。肝穿刺组织病理检查是目前诊断各种慢性肝损伤的重要依据,在对自身免疫性肝病的诊断认识过程中,肝脏的组织病理学起重要作用。     

自身免疫性肝病活检组织病理学特征分析109例

目的:分析比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)及其重叠综合征的组织病理学变化,提高对自身免疫性肝病(AILD)的认识.方法:对27例AIH、67例PBC、4例PSC、1例AIH-PSC重叠综合征和10例AIH-PBC重叠综合征患者的肝穿组织病理资料进行回顾性分析.结果:AILD患者多发于中年女性(73.3%),肝组织病理变化以界面性肝炎为主(77.7%),在重度患者则出现重度界面性肝炎、桥样坏死等.PBC患者早期(Ⅰ、Ⅱ)占28.3%,而晚期(Ⅲ、Ⅳ)占71.7%,肝组织病理变化以小胆管减少甚至消失为主(62.6%).AIH-PBC重叠综合征患者并非罕见,他的肝组织病理学具有AIH和PBC的双重特征.结论:AILD是非病毒性肝病的重要组成部分,其诊断需综合临床表现、生化、免疫指标和组织学变化.     

自身免疫性肝病20例临床特点分析

目的　比较自身免疫性肝炎 (AIH)、原发性胆汁性肝硬化 (PBC)和原发性硬化性胆管炎 (PSC)的临床特点 ,以利于临床早期鉴别诊断。方法　分析我院 1998～ 2 0 0 1年 2 0例自身免疫性肝病 (AIH 9例 ,PBC 10例 ,PSC 1例 )的临床资料 ,并对其肝功能特点及自身抗体进行比较。结果　 2 0例患者中 ,男女之比为 2∶18,平均年龄 (5 4.7± 11.0 )岁。AIH与PBC患者各项指标比较 ,PBC肝功能异常以碱性磷酸酶升高为特点 ,其余指标比较差异无显著性 ;AIH免疫学指标以γ 球蛋白、主要是免疫球蛋白IgG升高为特点 ,而PBC以γ 球蛋白、主要是IgM升高为特点 ;AIH循环自身抗体以抗核抗体、抗平滑肌抗体为主 ,而PBC患者以抗线粒体抗体及其分型M2 为主。结论　AIH及PBC临床表现、肝功能检查有许多相似之处 ,但仍然有其各自特异的临床诊断依据     

自身免疫性肝病重叠综合征诊治进展

自身免疫性肝病包括自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC)、原发性硬化性胆管炎(PSC)等。同时存在AIH、PBC或PSC特征者称为重叠综合征,若不及时接受治疗常可迅速进展至肝硬化和肝功能衰竭。早期诊断和治疗可显著改善预后。本文就近年自身免疫性肝病重叠综合征的诊治进展作一阐述。     

自身免疫性肝病自身抗体检测的临床意义

自身免疫性肝病(autoimmuneliverdisease,ALD)是指一组病因尚不明确,但普遍认为与自身免疫有关的肝脏疾病。通常包括自身免疫性肝炎(autoimmunehepatitis,AIH)、原发性胆汁性肝硬化(primarybiliarycirrhosis,PBC)及原发性硬化性胆管炎(primarysclerosingcholangi-tis,PSC)。AIH     

原发性胆汁性肝硬化／自身免疫性肝炎重叠综合征患者的临床和病理学研究

目的分析原发性胆汁性肝硬化（PBC）、自身免疫性肝炎（AIH）和PBC／AIH重叠综合征患者的临床和病理学特点。方法对105例自身免疫性肝病患者的临床资料进行分析,比较PBC／AIH重叠综合征和单纯PBC或AIH患者的临床表现和肝组织病理学变化。结果在105例患者中,包括11例PBC／AIH重叠综合征、60例PBC、33例AIH和1例原发性硬化性胆管炎（PSC）。PBC／AIH重叠综合征与PBC或AIH患者的性别、年龄、症状和并发症无明显差异（P〉0．05）,其实验室检查具有AIH的特点,如血清丙氨酸氨基转移酶、天冬氨酸氨基转移酶、免疫球蛋白IgG的明显升高,同时具有PBC的特点,如GGT、ALP、免疫球蛋白IgM的显著增高,但与PBC或AIH相比,无统计学差异（P〉0．05）;自身抗体检测可见抗核抗体、抗线粒体M2抗体和抗核心蛋白gP210抗体阳性,后两项抗体检出率明显高于AIH患者（P〈0．01）;肝组织病理学检查结果显示,PBC／AIH重叠综合征兼有PBC和AIH的特点,如界面炎和碎屑样坏死,汇管区浆细胞浸润,胆管不同程度的病变等。结论PBC／AIH重叠综合征的临床表现和肝组织病理学具有PBC和AIH的双重特征,应对此病充分认识,并探索有效的治疗方案。     

自身免疫性肝病及其重叠综合征的诊断及治疗

自身免疫性肝病主要包括自身免疫性肝炎( autoimmune hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)及其相互重叠的综合征,但就相互重叠关联而言尚没有明确的定义.在重叠综合征中,以AIH - PBC最为多见,在AIH或PBC患者中占10％[1].近年来由于相关临床经验的累积、实验室诊断技术的发展以及肝活检的普及,使得我国自身免疫性肝病检出率明显增高.     

原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征30例诊断和治疗分析

背景:自身免疫性肝病包括自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)和原发性硬化性胆管炎(PSC)等。这组疾病的临床表现、生化、免疫和组织学变化常常交叉重叠,使得临床鉴别相当困难。目的:探讨PBC鄄AIH重叠综合征患者的临床特点、诊断和治疗情况。方法:从164例自身免疫性肝病患者中选取PBC鄄AIH重叠综合征、AIH和PBC患者各30例进行配比,重点对PBC鄄AIH重叠综合征患者的诊断和治疗应答情况进行分析。结果:PBC鄄AIH重叠综合征患者占本组自身免疫性肝病病例的18%,三组患者的性别、年龄无显著差异,黄疸、皮肤瘙痒等症状、体征的发生率亦无差异。PBC鄄AIH组患者的天冬氨酸氨基转移酶(AST)和丙氨酸氨基转移酶(ALT)水平显著高于AIH和PBC组;碱性磷酸酶(ALP)和γ鄄谷氨酰转肽酶(GGT)水平显著高于AIH组,但低于PBC组(P<0.05或P<0.01)。经免疫抑制剂联合治疗后,AIH组的上述肝功能指标均显著改善(P<0.05或P<0.01),PBC鄄AIH组的胆红素、AST和ALT水平亦显著降低(P<0.05或P<0.01),但改善时间慢于AIH组(P<0.05或P<0.01)。结论:在本组病例中,PBC鄄AIH重叠综合征并不少见,其诊断需综合临床表现、生化、免疫指标和组织学变化,联合应用皮质激素、硫唑嘌呤和熊去氧胆酸(UDCA)并维持治疗,可获得较好的疗效。     

鞍钢职工心脑血管疾病现状与发病趋势

目的分析鞍钢职工大样本人群心脑血管疾病的发病率及易患因素分布情况。方法通过对鞍钢集团95 912例职工的心脑血管疾病既往史、吸烟、血压、糖尿病、血清总胆固醇、高密度脂蛋白、体重指数、总胆固醇与高密度脂蛋白胆固醇的比值等数据的分析,前瞻性分析受检人群心脑血管疾病发病的危险分层。结果冠心病1 910例(2.00%),脑血管病607例(0.63%),吸烟19 453例(20.28%),糖尿病788例(0.82%),高血压31 698例(33.05%),高脂血症8 347例(8.70%),体重超重55 462例(57.83%),总胆固醇(TC)/高密度脂蛋白胆固醇(HDL-C)≥3.5的10 487例(10.93%);缺血性心血管病发病概率多分布于极低危(68.14%)和低危(5.75%),中危(0.865%)占很小的比例,颈动脉粥样硬化斑块发生概率多分布于高危(44.23%)、中危(23.21%)和极高危(10.51%),无低危和极低危。结论目前鞍钢职工的健康状况不容乐观,应进一步加大健康知识宣教力度。     

Impacts of biodiversity and biodiversity loss on zoonotic diseases

Zoonotic diseases are infectious diseases of humans caused by pathogens that are shared between humans and other vertebrate animals. Previously, pristine natural areas with high biodiversity were seen as likely sources of new zoonotic pathogens, suggesting that biodiversity could have negative impacts on human health. At the same time, biodiversity has been recognized as potentially benefiting human health by reducing the transmission of some pathogens that have already established themselves in human populations. These apparently opposing effects of biodiversity in human health may now be reconcilable. Recent research demonstrates that some taxa are much more likely to be zoonotic hosts than others are, and that these animals often proliferate in human-dominated landscapes, increasing the likelihood of spillover. In less-disturbed areas, however, these zoonotic reservoir hosts are less abundant and nonreservoirs predominate. Thus, biodiversity loss appears to increase the risk of human exposure to both new and established zoonotic pathogens. This new synthesis of the effects of biodiversity on zoonotic diseases presents an opportunity to articulate the next generation of research questions that can inform management and policy. Future studies should focus on collecting and analyzing data on the diversity, abundance, and capacity to transmit of the taxa that actually share zoonotic pathogens with us. To predict and prevent future epidemics, researchers should also focus on how these metrics change in response to human impacts on the environment, and how human behaviors can mitigate these effects. Restoration of biodiversity is an important frontier in the management of zoonotic disease risk.     

Paediatric aspects of coeliac disease: old challenges and new ones…

The concept of coeliac disease has expanded from a gastrointestinal disease with malabsorption to a systemic immunological disease with a genetic basis. Epidemiological studies indicate that environmental factors, like the infant feeding pattern, affect the clinical presentation while population-screening studies indicate that the prevalence, at least in Caucasian populations, is similar. Secondary complications, like malignancies, osteopenia - osteoporosis, gynaecological and obstetrical problems and autoimmune diseases, are common. The risk is reduced or prevented by treatment with a gluten-free diet. The basis for such a secondary prevention is: 1. early case-finding by a) knowledge about different presentations of the disease and factors affecting that, b) generous serological testing in patients with vague symptoms, c) screening of risk groups, and, 2. support for children and adolescents with coeliac disease to comply with the gluten-free diet.     

Simultaneous operation of concomitant diseases in the esophagus and lung

The aim of this study was to retrospectively review our experience of performing simultaneous operations on concomitant diseases in the esophagus and lungs. From January 1998 to July 2009, simultaneous operations were performed on 13 patients with concomitant esophageal and pulmonary diseases, using coordinated surgical approaches. Among the 13 patients, six had primary cancers in the esophagus and lungs, five had primary esophageal cancer accompanied by a benign pulmonary disease, one had benign diseases in both esophagus and lung, and one had primary esophageal cancer with metastasis to the left lower lung. All patients survived the operations. Two major complications occurred postoperatively. One complication was bronchopleural fistula and the other was intrathoracic gastric laceration. Both patients recovered after additional treatments. Simultaneous operation of concomitant diseases in the esophagus and lungs is feasible and safe in selected patients who have received careful preoperative assessment, well‐designed surgical approach, and proper perioperative management.     

Concerted action of wild-type and mutant TNF receptors enhances inflammation in TNF receptor 1-associated periodic fever syndrome

TNF, acting through p55 tumor necrosis factor receptor 1 (TNFR1), contributes to the pathogenesis of many inflammatory diseases. TNFR-associated periodic syndrome (TRAPS, OMIM 142680) is an autosomal dominant autoinflammatory disorder characterized by prolonged attacks of fevers, peritonitis, and soft tissue inflammation. TRAPS is caused by missense mutations in the extracellular domain of TNFR1 that affect receptor folding and trafficking. These mutations lead to loss of normal function rather than gain of function, and thus the pathogenesis of TRAPS is an enigma. Here we show that mutant TNFR1 accumulates intracellularly in peripheral blood mononuclear cells of TRAPS patients and in multiple cell types from two independent lines of knockin mice harboring TRAPS-associated TNFR1 mutations. Mutant TNFR1 did not function as a surface receptor for TNF but rather enhanced activation of MAPKs and secretion of proinflammatory cytokines upon stimulation with LPS. Enhanced inflammation depended on autocrine TNF secretion and WT TNFR1 in mouse and human myeloid cells but not in fibroblasts. Heterozygous TNFR1-mutant mice were hypersensitive to LPS-induced septic shock, whereas homozygous TNFR1-mutant mice resembled TNFR1-deficient mice and were resistant to septic shock. Thus WT and mutant TNFR1 act in concert from distinct cellular locations to potentiate inflammation in TRAPS. These findings establish a mechanism of pathogenesis in autosomal dominant diseases where full expression of the disease phenotype depends on functional cooperation between WT and mutant proteins and also may explain partial responses of TRAPS patients to TNF blockade.     

Prevalence of Coronary Artery Disease in Patients with Valvular Heart Disease

ABSTRACT. To evaluate the usefulness of preoperative coronary angiography in patients undergoing preoperative investigation because of valvular heart disease, we performed coronary angiography in a consecutive series of 329 patients. The prevalence of significant coronary artery disease was 32%. Asymptomatic coronary artery disease was present in 13%. Angina pectoris proved to be a poor predictor of coronary artery disease in aortic valve disease. In mitral valve disease, however, the specificity was high. A cost-benefit calculation was carried out in order to assess what advantage routine coronary angiography might have. According to this, coronary angiography should be performed in all patients suffering from valvular heart disease with angina pectoris, whereas it can be omitted in younger patients without angina. A cut-off point of 60 years seems appropriate for aortic valve disease and 65 years for mitral valve disease.     

川崎病的心血管病变观察

目的　观察川崎病 ( KD)的心血管病变。方法　对 2 6例 KD的住院患儿 ,于急性期或亚急性期行心电图、超声心动图、血清肌钙蛋白 I( c Tn I)及肌酸磷酸激酶 ( CK)及其同工酶 ( CK-MB)检查 ,并于病程不同时期 ( 6个月～ 2年 )进行复查。结果　冠状动脉病变 10例 ,占 3 8.5 % ,其中冠状动脉扩大 7例 ,冠状动脉瘤 3例 ,发生于急性期或亚急性期 9例 ,发生于病程第 9个月 1例。心肌炎 2例 ,主动脉瓣、二尖瓣反流各 1例。心电图异常 8例 ,占 3 0 .8%。血清 c Tn I阳性 12例 ,占 46.2 % ,CK-MB升高 7例 ,占 2 6.9%。结论　KD的心血管并发症表现形式多样 ,主要为冠状动脉扩大和冠状动脉瘤 ,其次是心电图异常 ,c Tn I阳性 ,CK-MB升高 ,主动脉瓣及二尖瓣反流。     

克山病.大骨节病区18种食物总脂质.总磷脂和主要磷脂含量的研究

对克山病、大骨节病区及非病区18种主要日常食物中总脂质、总磷脂和主要磷脂含量进行了分析测试。评价了病区主要食品的磷脂营养价值。结果表明:同一种食品病区与非病区之间无显著差异;病区粮食的磷脂营养价值,以大豆最高、小麦次之、玉米和籼米最低;叶菜类高于根茎类。文中对改善病区村民膳食磷脂供应问题,进行了初步探讨。     

IgG4相关性肺疾病1例及文献复习

目的提高对IgG4相关性肺疾病的临床特征、胸部影像学和病理组织学的认识。方法对1例经病理证实的IgG4相关性肺疾病的临床资料进行分析,并结合文献进行回顾总结。结果患者男,62岁,以胸腔积液起病,在外院先后行胸膜活检、胸腔镜肺活检等均未能明确诊断,后至我院行CT引导下经皮肺穿刺活检术,结果示活检组织内纤维组织增生伴淋巴细胞、浆细胞等炎症细胞浸润,免疫组化示大量浆细胞(+),IgG4阳性,浆细胞最密集计数约为40个/高倍视野,血清IgG4浓度示4.07 g/L(0.03 g/L~2 g/L)。诊断IgG4相关性肺疾病,给予糖皮质激素治疗,2个月后复查胸部CT示肺部病灶较前局部吸收、好转。结论IgG4相关性疾病是一种累及多器官、以血清IgG4水平升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病。目前国内IgG4相关性肺疾病的报道很少,报道这一病例并进行文献复习有助于提高对IgG4相关性肺疾病的认识。