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1.
目的 分析比较自身免疫性肝炎(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 cholangltis)及其重叠综合征的临床特点、生化特征和治疗反应,提高对自身免疫性肝病的认识。方法对77例AIH患者、46例PBC患者、11例PSC患者和30例PBC-AIH重叠综合征患者的临床及实验室检查资料进行回顾性分析。结果除PSC外,大多数自身免疫性肝病多发于中年女性,从出现症状到明确诊断平均需要2.5年。AIH、PBC-AIH重叠患者具有较高的转氨酶,PBC、PSC具有较明显的GGT、ALP升高。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%。PSC和AIH-PBC具有较高的AIH评分,27%的PSC患者和33%AIH-PBC的评分达到可能的AIH。合理应用UDCA和免疫抑制剂可使90%的PBC和AIH患者症状在六个月内得到缓解、肝功能恢复明显改善。结论 AIH、PBC-AIH的肝功能异常以转氨酶升高为主,PBC、PSC以胆汁淤积为主。应用AIH评分系统诊断可能的AIH时应注意鉴别PSC及其它自身免疫性肝病。UDCA和免疫抑制剂可改善绝大多数患者的症状和肝功能异常。  相似文献   

2.
目的: 分析自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及原发性硬化性胆管炎(PSC)患者的临床特点及影像学特征.方法: 2003-10/2008-02在烟台毓璜顶医院消化内科住院的自身免疫性肝病患者43例, 排除其他原因引起肝脏损害. 其中AIH 12例, PBC 19例, PSC 12例. 对患者的临床及影像学资料进行回顾性分析, 观察药物治疗的效果.结果: 除PSC外, AIH多发于中年女性, 患者具有较高的转氨酶, PBC、PSC患者具有较明显的GGT、ALP升高. 临床表现上AIH、PBC、PSC黄疸的发生率分别为42%、80%、91%,皮肤瘙痒的发生率分别为38%、68%、79%.合理应用熊去氧胆酸和免疫抑制剂可使68%的PBC和AIH患者症状在6 mo内得到缓解、肝功能明显改善.结论: AIH的肝功能异常以转氨酶升高为主,PBC、PSC以胆汁淤积为主. 熊去氧胆酸和免疫抑制剂可改善绝大多数患者的症状和肝功能.  相似文献   

3.
自身免疫性肝病(AILD)包括原发性胆汁性胆管炎(PBC)、自身免疫性肝炎(AIH)、原发性硬化性胆管炎(PSC)。患者可在初诊时或随访的过程中出现2种AILD的特征,通常将这种情况称为“重叠综合征”,其中以PBC重叠AIH最为常见。与单纯PBC或AIH相比,PBC-AIH重叠综合征门静脉高压、消化道出血、腹水、死亡及肝移植发生率明显升高,病情进展也更迅速,因此,其早诊早治显得尤为重要。对近年PBC-AIH重叠综合征的诊治进展进行综述。  相似文献   

4.
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.  相似文献   

5.
自身免疫性肝病是一组以异常自身免疫反应为基础的慢性肝、胆疾病综合征,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)和原发性硬化性胆管炎(primary sclerosing eholangitis,PSC).由于本组疾病确切的发病机制尚不清楚,各个疾病的临床表现、生化、免疫学检查和组织学变化有交叉重叠,故而提出了"重叠综合征"一词,临床上以AIH-PBC、AIH-PSC和PBC-PSC重叠常见.本文现将自身免疫性肝病及其诊断重叠综合征的诊断介绍于下.  相似文献   

6.
自身免疫性肝病主要包括原发性胆汁性胆管炎(PBC)、自身免疫性肝炎(AIH)和原发性硬化性胆管炎(PSC)。同时或相继出现以上任意两种单独自身免疫性肝病特征者称为重叠综合征。其中,以PBC重叠AIH(PBC-AIH)相对最为常见。若不及时接受治疗可迅速进展至肝硬化和肝衰竭。就近年自身免疫性肝病重叠综合征的研究进展进行了总结。  相似文献   

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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AIH重叠综合征的临床特点、生化特征和组织学变化,以提高对自身免疫性肝病(AILD)的认识.方法 收集2004年1月-2008年6月肝穿刺病理学检查确诊的AILD患者共109例,其中AIH 27例、PBC 67例、PSC 4例、AIH-PSC重叠综合征1例和AIH-PBC重叠综合征10例,对患者的临床及实验室检查资料进行回顾性分析.结果 AILD患者多发于中年女性(73.3%,80/109),常见症状为黄疸、乏力、纳差和皮肤瘙痒.AIH患者的发病年龄高峰在50岁左右,肝功能检查结果显示为肝炎样异常,丙种球蛋白和免疫球蛋白G均明显高于正常值,62.9%的患者(17/27)抗核抗体(ANA)阳性.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件肝炎、桥样坏死等.PBC患者主要表现为碱性磷酸酶、γ-谷氨酰转肽酶和胆红素明显升高,伴免疫球蛋白M升高,74.6%的患者(50/67)线粒体抗体(AMA)和(或)AMA-M2亚型阳性.所有PBC患者行肝脏病理学检查,早期(Ⅰ、Ⅱ)占28.3%,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 0A).AIH-PBC重叠综合征患者的临床表现和肝组织病理学具有AlH和PBC的双重特征,其中有3例患者同时检测到ANA和AMA/AMA-M2阳性.结论 AILD在中国人中并非少见,其诊断需综合临床表现、生化、免疫指标和组织学变化.  相似文献   

8.
自身免疫性肝病临床病理特征分析28例   总被引:4,自引:0,他引:4  
目的:探讨并比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及其重叠综合征(AIH-PBC CS)的临床、生化、影象学以及组织学特征.方法:收集在我院自身免疫性肝病住院患者28例,其中v型AIH 13例、PBC 6例和AIH-PBCOS9例,回顾性分析患者临床资料.结果:AIH-PBC OS患者血清谷草转氨酶(AST)和γ-球蛋白(GLO)水平(分别为和)均明显高于AIH和PBC患者(132.67±35.05 vs 81.15±39.26,55.17±24.38;44.33±8.92 vs 34.23±9.89,33.17±6.79,均P<0.05),而碱性磷酸酶(ALP)和γ-谷氨酰转肽酶(GGT)水平明显高于AIH患者(218.11±107.79 vs 106.85±91.57;280.33±133.51 vs 82.07±36.67,均P<0.05),低于PBC患者(245.83±88.60,315.67±160.01,均P<0.05).AIH-PBC OS和PBC患者的IgM水平均明显高于AIH患者(793.00±528.57 vs127.33±30.14;538.50±349.43 vs 127.33±30.14,均P<0.05),而AIH-PBC OS患者的IgG水平明显高于PBC患者(2036.00±457.03 vs1121.25±313.8,P<0.05).AIH-PBC OS患者界面性肝炎和破坏性胆管炎的发生率分别为88.9%和66.7%.腹部BUS提示三者常伴有脾大和腹腔淋巴结肿大.结论:AIH和PBC是典型的分别选择性损伤肝细胞及肝内小胆管的自身免疫性肝病,AIH-PBC OS同时具有AIH和PBC的临床病理学特征,与PBC相比,AIH-PBC OS更倾向于AIH.  相似文献   

9.
目的观察自身免疫性肝炎和原发性胆汁性肝硬化(AIH-PBC)重叠综合征的临床特征及治疗效果。方法研究1:回顾分析124例PBC、57例AIH、39例AIH-PBC重叠综合征患者的临床特征;研究2:根据不同治疗方案对39例AIH-PBC重叠综合征患者进行分组疗效分析。结果在220例自身免疫性肝病患者中,AIH-PBC重叠综合征占17.73%。3组患者的性别组成差异无统计学意义,但发病年龄AIH组相似文献   

10.
AIH-PBC重叠综合征的诊断及治疗   总被引:1,自引:0,他引:1  
温小凤  蒋忠胜 《肝脏》2009,14(5):426-427
自身免疫性肝病是一组与自身免疫异常有关的肝胆疾病,其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)以及这三种疾病中任两者之间的综合征。AIH-PBC重叠综合征(AIHPBCOS)在PBC患者中的发生率为2%~20%.其定义为一个患者同时具有这二种疾病的主要特征。  相似文献   

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Our study examined the efficacy of four treatment modalities in controlling hemorrhage and achieving hemodynamic stabilization in hemorrhagic shock: intravenous fluid replacement (IV); military antishock trousers used concomitantly with fluids (MAST); balloon occlusion at the level of the diaphragm with concomitant fluid replacement (balloon); and a combination of MAST inflation, balloon occlusion, and fluid resuscitation (MAST and balloon). Twenty-eight mongrel dogs were anesthetized, and the spleen was exposed and completely crushed. The abdomen was closed, and treatment was initiated and continued for four hours or until the dog died. For all conditions the hematocrit dropped during the course of the experiment; balloon occlusion was effective at slowing this drop (P less than .0001), but MAST had no statistically significant effect. Animals with balloons bled more slowly into the abdominal cavity than did animals in the other two groups (P less than .0001). MAST also were effective at slowing the bleeding (P less than .05). Of the balloon and the MAST and balloon dogs, all except one survived the entire four hours; this difference between balloon and nonballoon dogs is significant (P = .002). MAST did not have a statistically significant effect on survival. Perfusion pressure (PP) declined during the course of the experiment, and the balloon was effective at slowing this decline (P less than .0001); none of the other comparisons was statistically significant.  相似文献   

13.
Paul Roddy 《Viruses》2014,6(10):3699-3718
The frequency and magnitude of recognized and declared filovirus-disease outbreaks have increased in recent years, while pathogenic filoviruses are potentially ubiquitous throughout sub-Saharan Africa. Meanwhile, the efficiency and effectiveness of filovirus-disease outbreak preparedness and response efforts are currently limited by inherent challenges and persistent shortcomings. This paper delineates some of these challenges and shortcomings and provides a proposal for enhancing future filovirus-disease outbreak preparedness and response. The proposal serves as a call for prompt action by the organizations that comprise filovirus-disease outbreak response teams, namely, Ministries of Health of outbreak-prone countries, the World Health Organization, Médecins Sans Frontières, the Centers for Disease Control and Prevention—Atlanta, and others.  相似文献   

14.
Sun Y  Han M  Kim C  Calvert JG  Yoo D 《Viruses》2012,4(4):424-446
Innate immunity is the first line of defense against viral infection, and in turn, viruses have evolved to evade host immune surveillance. As a result, viruses may persist in host and develop chronic infections. Type I interferons (IFN-α/β) are among the most potent antiviral cytokines triggered by viral infections. Porcine reproductive and respiratory syndrome (PRRS) is a disease of pigs that is characterized by negligible induction of type I IFNs and viral persistence for an extended period. For IFN production, RIG-I/MDA5 and JAK-STAT pathways are two major signaling pathways, and recent studies indicate that PRRS virus is armed to modulate type I IFN responses during infection. This review describes the viral strategies for modulation of type I IFN responses. At least three non-structural proteins (Nsp1, Nsp2, and Nsp11) and a structural protein (N nucleocapsid protein) have been identified and characterized to play roles in the IFN suppression and NF-κB pathways. Nsp's are early proteins while N is a late protein, suggesting that additional signaling pathways may be involved in addition to the IFN pathway. The understanding of molecular bases for virus-mediated modulation of host innate immune signaling will help us design new generation vaccines and control PRRS.  相似文献   

15.
Virus disease pandemics and epidemics that occur in the world’s staple food crops pose a major threat to global food security, especially in developing countries with tropical or subtropical climates. Moreover, this threat is escalating rapidly due to increasing difficulties in controlling virus diseases as climate change accelerates and the need to feed the burgeoning global population escalates. One of the main causes of these pandemics and epidemics is the introduction to a new continent of food crops domesticated elsewhere, and their subsequent invasion by damaging virus diseases they never encountered before. This review focusses on providing historical and up-to-date information about pandemics and major epidemics initiated by spillover of indigenous viruses from infected alternative hosts into introduced crops. This spillover requires new encounters at the managed and natural vegetation interface. The principal virus disease pandemic examples described are two (cassava mosaic, cassava brown streak) that threaten food security in sub-Saharan Africa (SSA), and one (tomato yellow leaf curl) doing so globally. A further example describes a virus disease pandemic threatening a major plantation crop producing a vital food export for West Africa (cacao swollen shoot). Also described are two examples of major virus disease epidemics that threaten SSA’s food security (rice yellow mottle, groundnut rosette). In addition, brief accounts are provided of two major maize virus disease epidemics (maize streak in SSA, maize rough dwarf in Mediterranean and Middle Eastern regions), a major rice disease epidemic (rice hoja blanca in the Americas), and damaging tomato tospovirus and begomovirus disease epidemics of tomato that impair food security in different world regions. For each pandemic or major epidemic, the factors involved in driving its initial emergence, and its subsequent increase in importance and geographical distribution, are explained. Finally, clarification is provided over what needs to be done globally to achieve effective management of severe virus disease pandemics and epidemics initiated by spillover events.  相似文献   

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Barrett''s esophagus (BE) is a precursor for esophageal adenocarcinoma, which has an increased incidence rate over the last few decades. Its importance stems from the poor five-year survival of esophageal adenocarcinoma and current data that suggest a survival benefit when surveillance programs are implemented. In this review, we will cover the pathophysiology and natural history of BE and the different endoscopic findings. The prevalence of BE in different geographic areas and the incidence of high-grade dysplasia and adenocarcinoma in this patient population is reviewed. Recent recommendation for screening and surveillance of BE has been covered in this review as well as the efficacy of nonconventional imaging modalities and endoscopic ablation therapies.  相似文献   

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Two masterpieces of the Qing Dynasty (1644–1912 CE), one in gilded brass (incense burner) decorated with cloisonné enamels stylistically attributed to the end of the Kangxi Emperor’s reign, the other in gold (ewer offered by Napoleon III to the Empress as a birthday present), decorated with both cloisonné and painted enamels bearing the mark of the Qianlong Emperor, were non-invasively studied by optical microscopy, Raman microspectroscopy and X-ray microfluorescence spectroscopy (point measurements and mapping) implemented on-site with mobile instruments. The elemental compositions of the metal substrates and enamels are compared. XRF point measurements and mappings support the identification of the coloring phases and elements obtained by Raman microspectroscopy. Attention was paid to the white (opacifier), blue, yellow, green, and red areas. The demonstration of arsenic-based phases (e.g., lead arsenate apatite) in the blue areas of the ewer, free of manganese, proves the use of cobalt imported from Europe. The high level of potassium confirms the use of smalt as the cobalt source. On the other hand, the significant manganese level indicates the use of Asian cobalt ores for the enamels of the incense burner. The very limited use of the lead pyrochlore pigment (European Naples yellow recipes) in the yellow and soft green cloisonné enamels of the Kangxi incense burner, as well as the use of traditional Chinese recipes for other colors (white, turquoise, dark green, red), reinforces the pioneering character of this object in technical terms at the 17th–18th century turn. The low level of lead in the cloisonné enamels of the incense burner may also be related to the use of European recipes. On the contrary, the Qianlong ewer displays all the enameling techniques imported from Europe to obtain a painted decoration of exceptional quality with the use of complex lead pyrochlore pigments, with or without addition of zinc, as well as cassiterite opacifier.  相似文献   

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