最新同济大学附属同济医院儿科31PPT课件.ppt

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1、概念nA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 pe

2、rmission 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

3、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

4、 病理n急性渗出期：34Wn增生期： 风湿小体，34Mo Aschoff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

5、，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 verrucaeFigure 8: Stenotic mitral valve seen from left atrium. Both commissures are fused; the cusps are severely thickened. The left atrium is huge. The

6、 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

7、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-dimens

8、ional 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: Colo

9、r 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 = righ

10、t atrium; AO = aorta -心包炎 少量积液：心包摩擦音 大量积液：心音遥远，肝大，颈静脉怒张， 奇脉 X线：心脏搏动减弱或消失，心影扩大，立 位烧瓶形，卧位心腰增宽 EKG: 早期低电压，ST段抬高 晚期ST段下降，T波平坦或倒置n舞蹈病 -好发812岁女孩 -不自主运动 -兴奋或注意力集中时加剧，入睡消失 -部分早期以情绪和性格变化为主 -病程13月，个别12年内可反复n皮肤症状 -皮下结节 关节伸面骨质隆起和肌腱附着处 0.11cm，圆形，质硬，活动无压痛 24W消失 -环形红斑 躯干和四肢屈侧处 时隐时现Figure 11: Subcutaneous nodule on

11、 the extensor surface of elbow of a patient with acute RF Figure 12a: Erythema marginatum on the trunk, showing erythematous lesions with pale centers and rounded or serpiginous marginsFigure 12b: Closer view of erythema marginatum in the same patient试验室检查n链球菌感染证据 ASO,ASK,AHn风湿热活动指标 发热，乏力，苍白，脉搏增快 ES

12、R,CRP,WBC,中性粒细胞，P-R间期延长，贫血，（ASO持续，无风湿热临床表现）Jones诊断标准主要表现次要表现链球菌感染证据1.心脏炎2.多关节炎3.舞蹈病4.环形红斑5.皮下结节临床表现 1.发热2.关节痛3.风湿热史实验室检查1.ESR 2.CRP 3.WBC 4.P-R 1.近期猩红热史2.咽拭培养+3.抗体滴度n注意 主要症状为关节炎，关节痛不能作为次要症状 主要症状为心脏炎，P-R间期延长不能作为次要症状n三种特殊情况 舞蹈病 ：排出其他病因者 隐匿性心脏炎：无其他情况者 风湿热复发：风心出现一项症状，+近期链球菌感染证据鉴别诊断n与风湿性关节炎鉴别 幼年类风湿关节炎 急性

13、化脓性关节炎 白血病 生长痛鉴别诊断n与心脏炎鉴别 感染性心内膜炎 病毒性心肌炎治疗n治疗原则 早期诊断，合理治疗，防止进展至不可逆改变 据病情轻重，合理选用抗风湿药，避免危重儿死亡，控制一般病例 症状 防治链球菌感染，防止疾病复发 注意药物副作用治疗n休息（卧床）急性期：2W 急性期有心脏炎：4W 心脏炎伴心功能不全：8w，23月内逐渐增加活动n饮食 高营养 少量多餐 适当限盐n控制链球菌感染: 青霉素 80万U ，im ，bid2w 苄星青霉素120万u，1次 红霉素 3040mg/kg/d，po，分34次/d10dn抗风湿治疗 -急性病例未合并心脏炎 阿斯匹林 ：80100mg/kg.d

14、2w 75mg/kg.d46w qid 疗程812w -合并心脏炎 强的松：2mg/kg.d24w，逐渐减量，疗程812w -严重心脏炎氢化可的松 DXM: 0.51mg/kg23d强的松：1030mg/kg，qd充血性心力衰竭：慎用或不用洋地黄，以免中毒；正常洋地黄剂量的1/21/3低盐、吸氧、利尿、扩管 -反跳现象 停激素后：低热，关节痛，ESR 一般23天内 消失 激素减量同时合用阿司匹林，最终代替激素 总疗程812Wn舞蹈病治疗 -激素和阿司匹林无效 -可用镇静剂n关节肿痛 制动 预防n苄星青霉素120万u，1次/34w 5。最好持续至25yr，有风心宜终身预防n红霉素每月口服67d，

15、持续时间同前n手术前后预防感染性心内膜炎病毒性心肌炎同济大学附属同济医院儿科魏东概念n各种病毒侵犯心肌n心肌局灶性或弥漫性炎症n心包或心内膜炎症（少）n临床轻重不一：心衰，休克，严重心律失常，猝死（少）n预后大多良好病因n各种病毒（20余种）n柯萨奇病毒B组（43.6%） 腺病毒（21.2%） 埃可病毒（10.9%）发病机制n直接侵犯心脏n自身免疫反应 潜伏期 抗心肌抗体n生化机制 自由基病理n心肌细胞水肿，溶解，坏死n心肌间质和血管周围炎性细胞侵润n心包和心内膜炎症n侵犯传导系统致心律失常n慢性病例有心脏扩大Note infiltration of lymphocytes and plasm

16、a cells in the interstitial space (between muscle fibers). The nuclei and cross striations of the cytoplasm are well preserved. In order to be classified as a myocarditis, Dallas criteria must be met. Specifically, the presence of necrotic myocytes with lymphocyte infiltrationMyocardial interstitium

17、 with abundant edema and inflammatory infiltrate, rich in lymphocytes and macrophages. Focal destruction of myocytes. (H&E, ob. x10)Active myocarditis (case 29; EV PCR positive). (a) Severe and diffuse inflammatory cell infiltration with evident necrosis of adjacent myocytes (original magnification

18、40). (b) Lymphocytes show strong CD45RO immunoreactivity (original magnification 40). 临床表现n急性心肌炎 -呼吸道和消化道感染的前驱症状 -心脏症状 -心脏体征：心脏扩大，心音低钝，奔马律， 心包摩擦音，心率失常 -心源性休克n慢性心肌炎 -超过1年 -反复发作的心律失常或心力衰竭 -进行性心脏扩大 -EKG改变持续不恢复 -X线心影不缩小辅助检查nEKG ST段下移，T波低平，双向或倒置，QRS低电压，Q-T间期延长nX线 心影增大，心脏搏动减弱，肺充血，肺水肿，胸腔积液，心包积液n血清检查 AST，CK

19、，CK-MB，LDH，-HBDHn病原学检查 -病毒抗体检测 -抗心肌抗体 -病毒分离 -PCRn心肌活检n心超诊断及鉴别诊断n病原学诊断依据确诊指标：自心内膜、心肌、心包或心包穿刺液发现以下之一者可确诊 分离到病毒 探针检测病毒核酸 特异性病毒抗体阳性 参考依据：有以下一项结合临床可考虑心肌炎由病毒引起 自粪便、咽拭子或血液中分离到病毒，且恢复期同型抗体4倍以上升高或降低 早期特异性IgM阳性 血中探针检测到病毒核酸n临床诊断依据 -主要指标 急慢性心功能不全或心脑综合症、心源性休克 心脏扩大（X线、心超） EKG严重心律失常 CK-MB升高或心肌肌钙蛋白阳性n确诊依据：临床诊断依据2项可临

20、床诊断；发病时或前13w有病毒感染证据支持者： 同时具备病因学确诊依据之一者，可确诊 具备病因学参考依据之一者，可临床诊断 不具备确诊依据，治疗随访 除外其他疾病治疗n休息：36月以上 急性期卧床休息至热退后34Wn改善心肌营养及代谢 -能量合剂 -大剂量VitC -辅酶Q10 -FDP n抗病毒治疗：-干扰素，病毒唑n激素；强的松，DXMn大剂量丙种球蛋白n对症治疗 -镇静，镇痛 -控制心力衰竭 -抢救心源性休克n中医中药：黄芪，丹参n心律紊乱的治疗 x-A2D5G8KbNfQiTlXo#r%v(y0B3E6I9LcOgRjVmYp!t&w)z1C4F7JaMePhSkWnZq$u*x+A2

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30、UmYp!t&w)z1C4F7JaMePhSkWnZq$u*x+A2D5H8KbNfQiUlXo#s%v(y0B3F6I9LdOgRjq$t*x-A2D5G8KbNeQiTlXo#r%v(y+B3E6I9LcOgRjUmYp!t&w)z1C4F7JaMePhSkWnZq$u*x+A2D5H8KbNfQiTlXo#s%v(y0B3E6I9LdOgRjVmYp!t&w-z1C4G7JaMePhTkWnZr$u*x+A2E5H8KcNfQiUlXp#s%v)y0B3F6IaLdOgSjVmYq!t*w-z1D4G7JbMePhTkWoZr$u(x+A2E5H9KcNfRiUlXp#s&v)y0C3F

31、6IaLdPgSjVnYq!t*w-A1D4G8JbMeQhTlWoZr%u(x+B2E6H9KcOfRiUmXp#s&v)z0C3F7IaLdPgSkVnYq$t*w-A1D5G8JbNeQhTlWo#r%u(y+B2E6H9LcOfRjUmXp!s&w)z0C4F7IaMdPhSkVnZq$t*x-A2D5G8KbRiUmXp#s&v)z0C3F7IaLdPgSkVnYq$t*w-A1D5G8JbNeQhTlWo#r%u(y+B2E6H9LcOfRjUmXp!s&w)z0C4F7IaMdPgSkVnZq$t*x-A1D5G8KbNeQiTlWo#r%v(y+B3E6H9LcOgRjUmYp

32、!s&w)z1C4F7JaMdPhSkWnZq$u*x-A2D5H8KbNfQiTlXo#s%v(y0B3E6I9LcOgRjVmYp!t&w)z1C4G7JaMePhSkWnZr$u*x+A2D5H8KcNfQiUlXo#s%v)y0B3F6I9LdOgSjVmYq!t&w-z1D4G7JbMePhTkWnZr$u(x+A2E5H8KcNfRiUlXp#s%v)y0C3F6IaLdOgSjVnYq!t*w-z1D4G8JbMeQhTkWoZr%u(x+B2E5H9KcOfRiUmXp#s&v)y0C3F7IaLdPgSjVn$u(x+A2E5H8KcNfRiUlXp#s%v)y0C3F6Ia

33、LdOgSjVnYq!t*w-z1D4G8JbMeQhTkWoZr%u(x+B2E5H9KcNfRiUmXp#s&v)y0C3F7IaLdPgSjVnYq$t*w-A1D4G8JbNeQhTlWoZr%u(y+B2E6H9KcOfRjUmXp!s&v)z0C4F7IaMdPgSkVnYq$t*x-A1D5G8JbNeQiTlWo#r%u(y+B3E6H9LcOfRjUmYp!s&w)z0C4F7JaMdPhSkVnZq$u*x-A2D5G8fRjUmXp!s&v)z0C3F7IaMdPgSkVnYq$t*x-A1D5G8JbNeQiTlWo#r%u(y+B3E6H9LcOfRjUmYp!s&w

34、)z0C4F7JaMdPhSkVnZq$u*x-A2D5G8KbNeQiTlXo#r%v(y+B3E6I9LcOgRjUmYp!t&w)z1C4F7JaMePhSkWnZq$u*x+A2D5H8KbNfUmYp!s&w)z0C4F7JaMdPhSkVnZq$t*x-A2D5G8KbNeQiTlXo#r%v(y+B3E6I9LcOgRjUmYp!t&w)z1C4F7JaMePhSkWnZq$u*x+A2D5H8KbNfQiUlXo#s%v(y0B3E6I9LdOgRjVmYp!t&w-z1C4G7JaMePhTkWnZr$u*x+A2E5H8KcNfQiUlXp#s%v)y0B3F6IaLdOg

35、SjVmYq!t*w-z1D4G7JbMePhTkWoZr$u(x+A2E5dOgRjVmYp!t&w-z1C4G7JaMePhTkWnZr$u*x+A2E5H8KcNfQiUlXp#s%v)y0B3F6IaLdOgSjVmYq!t&w-z1D4G7JbMePhTkWoZr$u(x+A2E5H9KcNfRiUlXp#s&v)y0C3F6IaLdPgSjVnYq!t*w-A1D4GcNfQiUlXo#s%v)y0B3F6I9LdOgSjVmYq!t&w-z1D4G7JbMePhTkWoZr$u(x+A2E5H9KcNfRiUlXp#s&v)y0C3F6IaLdPgSjVnYq!t*w-A1D4G

36、8JbMeQhTkWoZr%u(x+B2E5H9KcOfRiUmXp#s&v)z0C3F7IaLdPgSkVnYq$t*w-A1D5G8JbNeQhTlWo#r%u(y+B2E6H9LcOfRjUmXp!s&v)z0C4F7IaMdPgSkVnZq$t*x-A1D5G8KbNeQiTlWo#r%v(y+B3E6H9LcOgRjUmYp!s&w)z1C4F7JaMdPhSkWnZq$u*x-A2D5H8KbNfQiTlXo#r%v(y0B3E6I9LcOgRjVmYp!-A1D5G8KbNeQiTlWo#r%v(y+B3E6H9LcOgRjUmYp!s&w)z1C4F7JaMdPhSkVnZq$u*x-A2D5G8KbNfQiTlXo#r%v(y0B3E6I9LcOgRjVmYp!t&w)z1C4G7JaMePhSkWnZr$u*x+A2D5H8KcNfQiUlXo#s%v)y0B3F6I9LdOgRjVmYq!t&w-z1C4G7JbMePhTk69 结束语结束语