原发性肾小球疾病英文.pptxVIP

原发性肾小球疾病英文第1页/共49页 概述肾小球疾病（Glomerular disease）是指病变位于肾小球的一类以血尿，蛋白尿，水肿和髙血压等为主要表现的肾脏疾病，可导致终末期肾脏病(End Stage Renal Disease, ESRD)，分原发性，继发性和遗传性。原发性病因常不明确，故又称之为特发性，肾小球病变是唯一或主要病变。继发性病因常比较明确（如系统性红斑狼疮和糖尿病等），肾小球病变为全身性疾病的一个部分，病理变化类型并不单一。而遗传性肾小球疾病，是遗传基因变异所致的肾小球疾病（如Alport综合征和Fabry病等）。第2页/共49页 分类原发性肾小球疾病病理分型(根据WHO 1995年制定的标准)：微小病变性肾小球病局灶节段性肾小球肾炎/肾小球硬化弥漫性肾小球肾炎膜性肾病增生性肾炎 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腹增生性肾小球肾炎/系膜毛细血管性肾小球肾炎 ④新月体(和坏死)性肾小球肾炎增生硬化性/硬化性肾小球肾炎第3页/共49页 免疫反应肾小球免疫损伤的机制肾小球疾病的进展 发病机制 (The mechanisms of glomerular injury)第4页/共49页 第5页/共49页 第6页/共49页 临床表现蛋白尿 Proteinuria血尿 Hematuria水肿 Edema高血压 Hypertension肾功能损害 Renal Insufficiency第7页/共49页 Nephrotic syndrome第8页/共49页 Figure 1. Nephrotic edema. 第9页/共49页 Figure 2. Nephrotic edema. 第10页/共49页 Nephrotic syndrome This is characterized by proteinuria (Typically 3.5g/24h), hypoalbuminemia ( less than 30g/dL ) and edema. Hyperlipidaemia is also present. Primary and secondary causes are summarized in Table 2, 3a,b In practice, many clinicians refer to “nephrotic range” proteinuria regardless of whether their patients have the other manifestations of the full syndrome because the latter are consequences of the proteinuria.第11页/共49页 Pathophysiology第12页/共49页 ProteinuriaProteinuria can be caused by systemic overproduction, tubular dysfunction, or glomerular dysfunction. It is important to identify patients in whom the proteinuria is a manifestation of substantial glomerular disease as opposed to those patients who have benign transient or postural (orthostatic) proteinuria.第13页/共49页 Heavy proteinuria (albuminuria)Figure 3.第14页/共49页 Zandi-Nejad K et al. Kidney Int. Suppl 2004;92:S76-S89图1、导致蛋白尿肾病的小管间质炎症及肾硬化的某些事件第15页/共49页 图2、肾脏对滤过白蛋白处理(回吸收) 的旁路Russo LM et al. Kidney Int. Suppl 2004;92:S67-S68第16页/共49页 HypoalbuminemiaHypoalbuminemia is in part a consequences of urinary protein loss. It is also due to the catabolism of filtered albumin by the proximal tubule as well as to redistribution of albumin within the body. This in part