原发性肾小球疾病英文课件.pptVIP

第二章 原发性肾小球疾病Chapter 2 Primary Glomerular Disease 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）。 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 发病机制 (The mechanisms of glomerular injury) 免疫反应 肾小球免疫损伤的机制 肾小球疾病的进展 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 Nephrotic syndrome 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. Pathophysiology Proteinuria Proteinuria 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. Hypoalbuminemia Hypoalbuminemia 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 accounts for the inexact relationship between urinary protein loss, the level of the serum albumin, and other secondary consequences of heavy albuminuria . Edema The salt and volume retention in the NS may occur through at least two different major mechanisms. In the classic theory, proteinuria leads to hypoalbumin