【精品】同济大学附属同济医院儿科4

风湿热同济大学附属同济医院儿科魏东概念n A组溶血性链球菌n 心脏炎n 游走性关节炎n 舞蹈病n 盘形红斑和皮下结节病因和发病机理n A组乙型溶血性链球菌咽峡炎 咽峡存留时间 菌株： M血清型、粘液样型 遗传学背景病因和发病机理n 分子模拟 夹膜透明质酸：关节、滑膜 细胞壁 M及相关蛋白、 N-乙酰葡萄糖胺和鼠李糖：心肌、心瓣膜 细胞膜脂蛋白：心肌肌膜、丘脑下核、尾状核FIG. 9. Reactivity of antistreptococcal-antimyosin MAb with human myocardium in an immunofluorescence assay. (Reprinted from reference 173 with permission from the publisher. Copyright 1989. The American Association of Immunologists.) FIG. 10. Reactivity of antistreptococcal-antimyosin MAb 36.2.2 with the surface or extracellular matrix of rat myocardial cells in culture. MAb 36.2.2 exhibits cytotoxicity against rat heart cells in the presence of complement. (Reprinted from reference 14 with permission from the publisher. Copyright 1997. The American Association of Immunologists.) 病因和发病机理n 自身免疫反应 免疫复合物 病 细胞免疫反应异常 T细胞反应增强、淋巴细胞母细胞化和增值反应减弱、 NK细胞功能增加 白细胞移动抑制试验增强 扁桃体单核细胞反应异常FIG. 2. How the immune system recognizes group A streptococci and uses opsonization by complement and type-specific antibody against M protein or any other surface molecule capable of generating opsonic antibody. Fc receptors shown on macrophages bind to the antibody Fc region, inducing phagocytosis and killing of the streptococci 病因和发病机理n 遗传背景HLA-B35、 DR2、 DR4、淋巴细胞表面标记D8/17+n 毒素Figure 2. Pathogenetic pathway for ARF and RHD 病理n 急性渗出期： 34Wn 增生期：风湿小体， 34MoAschoff细胞。此细胞体积大，圆形、多边形，边界清楚而不整。胞浆丰富均质而微嗜双色。核大，圆形或卵圆形，核膜清晰，染色质集中于中央，横切面呈枭眼状，纵切面呈毛虫状，稍后则核变的浓染，结构不清。除单核外，亦可见双核或多核。 n 硬化期：瓣膜受累，持续 34月Figure 3: Myocardial Aschoff body the cells are large, elongated, with large nuclei; some are multinucleate 在纤维素样坏死基础上，出现巨噬细胞吞噬纤维素样坏死物所形成的阿少夫细胞，胞界清而不整齐，略嗜双色，呈枭眼或毛虫状且有少量淋巴细胞，浆细胞 Figure 2: Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations “verrucae“Figure 8: Stenotic mitral valve seen from left atrium. Both commissures are fused; the cusps are severely thickened. The left atrium is huge. The valve is both incompetent and stenoticFigure 9: Opened stenotic mitral valve showing thickening distorted cusps, adherent commissures with calcification and thrombus deposition, and thickening, fusion and shortening of chordae tendinaeFigure 10: Stenotic mitral valve seen from left atrium, showing fusion of commissures, thickening and calcification of the cusps临床表现n 一般表现发热，咽峡炎，扁桃体炎，猩红热n 关节炎游走性大关节不留关节畸形34Wn 心脏炎-心肌炎HR，奔马律心脏扩大，心尖搏动弥漫，第一心音低钝心尖区 / SM，吹风样X线：心脏扩大，心脏搏动减弱EKG: 传导阻滞（ ）， ST-T波改变心力衰竭-心内膜炎二尖瓣：心尖区 级，吹风样，全收缩期舒张期隆隆样杂音主动脉瓣：舒张期吹风样杂音二尖瓣关闭不全和狭窄：半年和 2年急性期：心脏扩大和瓣膜充血致杂音Figure 5a: Two-dimensional color flow Doppler image of the left ventricular inflow of a patient with mitral regurgitation in the four-chamber view (top panel) and two-dimensional parasternal long-axis view (lower panel), showing lack of apposition of the leaflets of the mitral valve during systole (arrow)Figure 5b: Color flow Doppler study of a patient with aortic regurgitation, as viewed from the parasternal long-axis view (top panel) and two-dimensional four-chamber view, showing hypertrophy and dilatation of the left ventricle (lower panel). LV = left ventricle; LA = left atrium; RV = right ventricle; RA = right atrium; AO = aorta -心包炎少量