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泌尿系统教学资料 慢性肾小球肾炎及肾病综合征王成玉课件

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泌尿系统教学资料 慢性肾小球肾炎及肾病综合征
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单击此处编辑 母版标题样 式 单击 此处编辑 母版副标题样 式 *1 肾小球疾病 Renal glomerular disease 肾内科 王成玉 学时数:2学时 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 概 念 肾小球疾病(Renal glomerular disease)是一组 临床表现相似,病因、发病机制及病理表现不尽 相同,但病变皆主要侵犯双侧肾小球的疾病, 可分为原发性、继发性及遗传性。 继发性肾小球疾病系指全身性疾病(如SLE、DM) 的肾小球损害; 遗传性肾小球疾病是遗传基因病变导致的肾小球疾 病(如遗传性进行肾炎); 原发性肾小球 疾病多数病因不清,需要除外继发性 及遗传性肾小球疾病后才能诊断。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 原发性肾小球疾病临床分型 1.急性肾小球肾炎(glomerular nephritis;GN) 2.急进性肾小球肾炎(rapidly progressive glomerulonephritis) 3.慢性肾小球肾炎(CGN) 4.隐匿性肾小球肾炎 5.肾病综合征 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二、病理分型(WHO 1995制定) (一)肾小球轻微病变 (二)局灶性节段性病变(局灶性肾小球肾炎、局灶性节段性肾 小球硬化) (三)弥漫性肾小球肾炎 1、膜性肾病 2、增生性肾炎 (1)系膜增生性肾小球肾炎 (2)毛细血管内增生性肾小球肾炎 (3)系膜毛细血管性肾小球肾炎 (4)新月体和坏死性肾小球肾炎 3、硬化性肾小球肾炎 (四)未分类的肾小球肾炎 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n同一病理类型可呈现不同的临床表现类型 ,而相同的一种临床类型可来自多种不同 的病理类型,因此肾活检是确定肾小球病 理类型和病变程度的必需手段,而正确的 病理诊断又必须与临床密切结合。 单击此处编辑 母版标题样 式 单击 此处编辑 母版副标题样 式 *6 慢性肾小球肾炎 CGN With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 慢性肾小球肾炎 定义:是以蛋白尿、血尿、高血压、水 肿为基本临床表现,起病方式各有不同, 病情迁延,病变缓慢进展、最终将发展成 CRF的一组肾小球疾病。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 病 理 1.系膜增生性肾炎 2.系膜毛细血管增生性肾炎 3.膜性肾病 4.局灶节段性肾小球硬化 5.硬化性肾小球肾炎 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 临床表现及实验室检查 1.好发于青、中年,男性居多。 2.多数起病缓慢、隐袭 3.起病急者,常有前驱症状(系膜毛细血管 肾炎、系膜增生性肾炎)。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 一.蛋白尿: 1.多为1~3g/d。 2.肾小球性蛋白尿。 二.血尿: 1.肾小球源性 2.以增生或局灶病理→可肉眼血尿 。 三.水肿:眼睑、下肢、一般无体腔积液。 四.高血压:中、晚期常有 五.肾功能损害:程度不等 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 诊断 1.临床及尿检异常:蛋白尿、血尿、 管型尿、水肿、高血压。 2.除外继发性、遗传性肾小球肾炎。 3.病史>1年。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 鉴别诊断 一、继发性肾小球肾炎:狼疮肾炎,紫 癜性肾炎 二、Alport综合征:起病于青少年(多 在10岁之前),有眼(球形晶状体等 )耳(神经性耳聋)肾(血尿、轻、 中度蛋白尿及进行性肾功能损害)异 常,并有阳性家族史。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三、其它原发性肾小球疾病: 1、隐匿型肾炎 2、感染后急性肾炎 潜伏期1~3周 100%镜下血尿 补体C3下降(<8周) 病理为毛细血管内增生性肾小球肾 炎。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 四、原发性高血压肾损害: 先有较长期高血压病后有肾脏的病变 ,肾小管功能较肾小球功能损伤早,且常 有高血压其它靶器官(心、脑、眼底)并 发症。贫血不如原发性肾小球肾炎严重。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 治 疗 以防止或延缓肾功能进行性减退、改善或 缓解临床症状及防治严重合并症为主要目的 ,不以消除蛋白和血尿为目标。 一.限制食物中蛋白及磷摄入量: 氮质血症时: 1.0.6~0.8g/kg/日 2.可辅以α-酮酸及必需氨基酸 3.低磷:600~800mg/日 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二.积极平稳地控制高血压: 高血压是与慢性肾脏疾病进展有关的一个关键机制, 其中肾小球毛细血管内压增高是介导进行性肾硬化 的关键因素。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 降压原则: ①目标值:尿Pr≥1g/d,BP < 125/75mmHg 尿Pr<1g/d,BP < 130/80mmHg ②选择具有肾保护作用、能延缓肾功能恶化的 降压药物 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 具体措施: 1.限盐<3g/天 2.利尿 3.ACEI或ARB 4.β受体阻滞剂 5.钙通道阻滞剂 6. 血管扩张药 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 降低肾小球内“三高” 间接效应 直接效应 改善肾小球滤过膜选 择通透性 减少细胞外基质蓄积 血压 依赖 非血压 依赖 非肾小球 血液动力 学效应 肾小球血 液动力学 效应 抑制AⅡ的肾脏保护作用机制 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society ①减低肾小球内“三 高” ②改善肾小球滤过膜 选 择通透性 ③减少细胞外基质蓄 积 抑制AⅡ的肾脏保护作用机制 延缓 肾损 害进 展 减少 尿蛋 白排 泄 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三.血小板解聚药: 大剂量的潘生丁 小剂量的阿斯匹林 四.避免有害于肾的因素: 感染、劳累、妊娠、肾毒性药。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 预后 n取决于病理类型,也与是否正 确治疗及养护有关。 单击此处编辑 母版标题样 式 单击 此处编辑 母版副标题样 式 *23 肾病综合征 nephrotic syndrome With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 诊断标准: 1.大量蛋白尿:>3.5g/天 2.低白蛋白血症:<30g/L 3.水肿 4.高脂血症 必备条件 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 肾病综合征的分类和常见病因 分类儿童青少年中老年 原发性MCDMsPGN MPGN FSGS MN MCD 继发性过敏性紫癜肾炎 乙肝病毒相关肾炎 先天性肾病综合征 SLE肾炎 过敏性紫癜肾炎 乙肝病毒相关肾炎 糖尿病肾病 肾淀粉样变性 骨髓瘤性肾病 淋巴瘤或实体 肿瘤性肾病 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 病理生理 大量蛋白尿 血浆蛋白减低 水肿 高脂血症 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 原发性肾病综合征的病理类型及 临床表现 一.微小病变病:(MCD) 1.病理 光镜: 肾小球无病变或仅轻微病变,近曲 小管上皮空泡变性和脂肪变性。 免疫荧光:免疫球蛋白和补体均阴性 电镜:肾小球上皮细胞足突广泛融合。无 ED↓ With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MCNS,(左)正常(右)上皮细胞足突广泛融合、消失 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 微小病变性肾病(PASM,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MCNS,上皮细胞足突广泛融合 、消失(电镜,×5000) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 2.临床表现: (1)好发于儿童2-8岁、老人。 (2)血尿:10%~20%镜下血尿,无肉 眼血尿。 (3)一过性高血压; (4)激素敏感(90%),复发率高(60%) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二.系膜增生性肾小球肾炎 1.病理: 光镜:系膜细胞和基质弥漫增生,可分轻、中、重度。 免疫荧光:IgA肾病: IgA沉积在系膜区 非IgA肾病:lgG或lgM为主,伴C3在Ms或 GCW呈团块状(偏小)和颗粒状沉积 电镜:在系膜区,有时还可在内皮下ED↓。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MsPGN,(左)正常(右)系膜细胞和基质增生, 电子致密物(D)沉积 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 肾小球系膜增生模式图 自左至右:轻度、中度和重度系膜增生 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 2.临床表现: ( 1)发病率高(30%)、好发青少年、男多于女。 (2)血尿:IgA肾病几乎100%,非IgA肾病约70%。 (3)随着肾病变程度由轻至重,肾功能不全及高Bp的 发生率 逐渐增加。 (4)对激素及细胞毒类药敏感与病理改变轻重有关。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三.系膜毛细血管性肾小球肾炎 1.病理: LM:系膜C和基质重度弥漫性增生,广泛插 入,GBM弥漫增厚,双轨征形成。 IIF:lgG、C3颗粒和团块状呈花瓣样沉积于 Ms和GCW。 EM:GBM的内皮下可见插入的系膜C和基质 并伴大块状ED↓。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MPGN,(左)正常,右系膜增生(M),电子致 密物沉积(D),广泛插入(I) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MPGN,系膜重度增生,广泛插入,GBM增厚,内皮 下嗜复红蛋白沉积(F)(Masson,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MPGN,IgG呈花瓣状颗粒状沉积于GCW及 系膜区(荧光,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 2.临床表现: (1)好发于青壮年,男多于女。 (2)急性起病者,多有前驱症状(60%~70%), 常伴急性肾炎综合征。 亦可隐匿起病。 (3)血尿: 100%血尿,肉眼血尿常见。 (4)常持续进展,肾功能、高血压、贫血出现早。 (5)C3↓:50-70%持续降低。 (6)激素、细胞毒药物成人无效,仅对部分儿童有 效。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 四.膜性肾病 1.病理 LM:①早期在GBM上皮侧见到多数排列整齐的 嗜复红小颗粒(Masson染色) ②钉突形成(PASM染色),GBM增厚。 EM:GBM上皮侧有ED沉积、有钉突、 足突广泛融合。 IIF: lgG、C3沿GCW呈高亮度细颗粒状, 均匀一致↓为为此病特征。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MN,(左)正常(右)上皮下IC沉积(D),GBM增厚, 钉突形成(S),上皮细胞足突融合 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society Ⅰ期MN,GBM空泡变性(V)(PASM,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society Ⅰ期MN,基底膜上皮侧少量电子致密物沉积(D),上 皮细胞足突融合(电镜,×5000) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society Ⅱ期MN,GBM弥漫性增厚,钉突形成(PASM,×800) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society MN,IgG呈细颗粒状GCW沉积(荧光,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 2.临床表现: (1)好发于中老年,男多于女 (2)隐匿起病:80%表现为 NS (3)血尿: 20-40%伴镜下血尿,无肉眼血尿 (4)进展缓慢: 5~10年→肾功能减退及高血压 (5)极易发生血栓栓塞并发症 (6)约1/4自行缓解; 未出现钉突→激素、细胞毒药敏感, 出现钉突→激素、细胞毒药疗效差。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 五.局灶性、节段性肾小球硬化 1.病理: LM:多在皮髓质交界处,局灶节段性分布。 性质为硬化(高度结节样系膜增生)+玻璃样变。 相应的肾小管萎缩,肾间质纤维化。 IIF:lgM、C3大团块状沉积于病变节段上。 EM:病变或非病变区上皮C足突广泛融合, 病变节段系膜基质增生,ED↓。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society FSGS(PASM,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society FSGS,IgM团块状沉积于病灶区 (荧光,×400) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 2.临床表现: (1)好发于青少年,男略多 (2)多隐匿起病 (3)血尿发生率高(约75%),可见肉眼血尿(约20%) (4)确诊时常有肾功能减退(30%)及高血压(50%) (5)常出现近端小管功能障碍:肾性糖尿、AA尿、 磷酸盐尿。 肾功能多进行性下降。 (6)对激素、细胞毒类药疗效不佳,少数尚可。 顶端型FSGS-激素可,塌陷型-差,经典型、细胞 型、非特殊型介于两者之间 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 推测病理诊断小结(一) n①发病年龄: n 发病高峰年龄从小至老依次为MCD(儿童、少年) n MsPGN及FSGS(青少 年) n MCGN(青壮年) n MN(中老年) n②起病情况: n 起病急、临床呈单纯NS者--MCD; n 感染后急性起病并呈现急性肾炎综合征者 n -MsPGN及MCGN; n 隐袭起病--多为MN及FSGS; n MsPGN及MCGN在无前驱感染情况下也可隐袭起病。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 推测测病理诊诊断小结结(二) n③血尿: 感染后3日内出现现肉眼血尿者,多为为IgA肾肾病; 无肉眼血尿,乃至无血尿者,主要为为MCD及MN。 n④肾肾功能不全: MCGN--肾肾功能不全出现现早、进进展快; MN肾肾功能不全出现现晚、进进展慢; FSGS及重度MsPGN确诊时诊时 已多有肾肾功能损损害; MCD及轻轻度MsPGN常肾肾功能正常。 n⑤其它: IgA肾肾病血清IgA水平可能增高; MCGN病人血清补补体常持续续降低。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 并发症 一.感染: ①原因:蛋白质营养不良、免疫功能下 降、激素治疗。 ②常见部位:呼吸道、泌尿系、皮肤、 腹腔等感染。 ③后果:影响疗效或者NS复发,甚至死亡。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二.血栓、栓塞并发症: 1.原因: (1)凝血、抗凝、纤溶系统失衡: 大分子凝血因子合成大于丢失、 小分子抗凝因子则丢失大于合成。 (2)血小板功能亢进 (3)血粘度增加(血液浓缩、高血脂) (4)激素加重高凝状态 2.肾V、肢体V、下腔V、肺、脑血栓 。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三.急性肾功能衰竭: 原因:1.胶体渗透压下降→肾前性 氮质血症。 2.肾间质水肿、大量管型 阻塞肾小管。 四.蛋白质和脂肪代谢紊乱 营养不良、生长发育障碍等 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 诊断和鉴别诊断 肾病综合征 原发 继发 病理类型 有无并发症 yes With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 诊断和鉴别诊断 继发性肾病综合征: 一.过敏性紫癜肾炎:好发于儿童,典 型皮疹、关节痛、腹痛及黑便。 二.系统性红斑狼疮性肾炎:好发于女性, 有多系统和器官受损表现,免疫学 检查多种自身抗体(+)。 With the improving quality
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