1、精品PPT课件 浏览免费 下载后可以编辑修改。 http:/ http:/ http:/ http:/ http:/ 读分享，下载后可以编辑修改。,Nephrotic syndrome,Figure 1. Nephrotic edema.,Figure 2. Nephrotic edema.,Clinical Syndrome,肾脏及泌尿系疾病经常会引起一些临床症状、 体征和实验室表现相似的综合征。识别患者属于哪一种综合征对诊断很有帮助，因为导致每个综合征的病因较之其包含的个别临床症状和体征的致病原因要少，故识别患者属于哪一种综合征对诊断有帮助。,The most common syndrom
2、e of kidney disease,Nephrotic syndrome Nephritic syndrome Asymptomatic urinary abnormalities Acute renal failure or Rapidly progressive renal failure Chronic kidney disease(Table 1),(一)肾病综合征 (二)肾炎综合征 (三)无症状性尿检异常 (四)急性及急进性肾衰竭综合征 (五)慢性肾脏病(表1),肾脏疾病常见综合征,Table 1. STAGES OF CHRONIC KIDNEY DISEASE*,* Chro
3、nic kidney disease is defined as either kidney damage or GFR 60mL/min/1.73m2 for 3months. Kidney damage is defined as pathologic abnormalities or markers of damage, including abnormalities in blood or urine tests or image studies.,Nephrotic syndrome,This is characterized by proteinuria (Typically 3.
4、5g/24h),hypoalbuminemia ( less than 30g/dL ) and edema. Hyperlipidaemia is also present.Primary and secondary causes are summarized in Table 2, 3In practice, many clinicians refer to “nephrotic range” proteinuria regardless of whether their patients have the other manifestations of the full syndrome
5、 because the latter are consequences of the proteinuria.,NEPHROTIC SYNDROME,Pathophysiology Proteinuria Hypoalbuminemia Edema HyperlipidemiaCause (diagnosis and differential diagnosis) Systemic renal disease hepatitis B associated glomerulonephritis, Henoch-Schonlein purpura, systemic lupus erythema
6、tosus, diatetes mellitus, amyloidosis Idiopathic nephrotic syndromeComplications Infection Coagulation disorders Protein malnutrition and dyslipidemia Acute renal failure,Pathophysiology,Proteinuria,Proteinuria can be caused by systemic overproduction, tubular dysfunction, or glomerular dysfunction.
7、 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.,Heavy proteinuria (albuminuria),Figure 3.,Hypoalbuminemia,Hypoalbuminemia is in part a cons
8、equences 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
9、consequences of heavy albuminuria .,The salt and volume retention in the NS may occur through at least two different major mechanisms. In the classic theory, proteinuria leads to hypoalbuminemia, a low plasma oncotic pressure, and intravascular volume depletion. Subequent underperfusion of the kidne
10、y stimulates the priming of sodium-retentive hormonal systems such as the RAS axis, causing increased renal sodium and volume retention, In the peripheral capillaries with normal hydrostatic pressures and decreased oncotic pressure, the Starling forces lead to transcapillary fluid leakage and edema
11、.,Edema,In some patients, however, the intravascular volume has been measured and found to be increased along with suppression of the RAS axis. An animal model of unilateral proteinuria shows evidence of primary renal sodium retention at a distal nephron site, perhaps due to altered responsiveness t
12、o hormones such as atrial natriuretic factor. Here only the proteinuric kidney retains sodium and volume and at a time when the animal is not yet hypoalbuminemic. Thus, local factors within the kidney may account for the volume retention of the nephrotic patient as well.,Edema,Figure 4.,Hyperlipidem
13、ia,Most nephrotic patients have elevated levels of total and low-density lipoprotein (LDL) cholesterol with low or normal high-density lipoprotein (HDL) cholesterol . Lipoprotein (a) Lp(a) levels are elevated as well and return to normal with remission of the nephrotic syndrome. Nephrotic patients o
14、ften have a hypercoagulable state and are predisposed to deep vein thrombophlebitis, pulmonary emboli, and renal vein thrombosis.,Cause,Table 2 CAUSES OF THE NEPHROTIC SYNDROME,Table 3a NEPHROTIC SYNDROME ASSOCIATED WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME),Table 3b NEPHROTIC SYNDROME AS
15、SOCIATED WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME),Pathology patterns and clinical presentations of idiopathic nephrotic syndome,In adults, the nephrotic syndrome is a common condition leading to renal biopsy. In many studies, patients with heavy proteinuria and the nephrotic syndromes h
16、ave been a group highly likely to benefit from renal biopsy in terms of a change in specific diagnosis, prognosis, and therapy. Selected adult nephrotic patients such as the elderly have a slightly different spectrum of disease, but again the renal biopsy is the best guide to treatment and prognosis
17、 (Table 2, 3).,Renal biopsy,PRIMARY NEPHROTIC SYNDROME,Minimal Change DiseaseFocal Segmental GlomerulosclerosisMembranous NephropathyMembranoproliferative Glomerulonephritis (MPGN),Figure 5a. Pathology of glomerular disease. Light microscopy. (a) Normal glomerulus; minimal change disease.,Table 4,PR
18、IMARY NEPHROTIC SYNDROME,Minimal Change DiseaseFocal Segmental GlomerulosclerosisMembranous NephropathyMembranoproliferative Glomerulonephritis(MPGN),Figure 5b. Segmental sclerosis; focal segmental glomerulosclerosis.,Figure 6. Light microscopic appearances in focal segmental glomerulosclerosis. Seg
19、mental scars with capsular adhesions in otherwise normal glomeruli.,Table 5,PRIMARY NEPHROTIC SYNDROME,Minimal Change DiseaseFocal Segmental GlomerulosclerosisMembranous NephropathyMembranoproliferative Glomerulonephritis(MPGN),Figure 7a. Early MN: a glomerulus from a patient with severe nephrotic s
20、yndrome and early MN, exhibiting normal architecture and peripheral capillary basement membranes of normal thickness (Silvermethenamine 400).,Figure 7b morphologically advanced MN,Figure 7c. Morphologically more advanced MN (same patient as in (b),Table 6,PRIMARY NEPHROTIC SYNDROME,Minimal Change Di
21、seaseFocal Segmental GlomerulosclerosisMembranous NephropathyMembranoproliferative Glomerulonephritis(MPGN),Figure 8. Pathology of membranoproliferative glomerulonephritis type I. (a) Light microscopy shows a hypercellular glomerulus with accentuated lobular architecture and a small cellular crescen
22、t (methenamine silver).,Table 7,Diagnosis and Differential diagnosis,Initial evaluation of the nephrotic patient includes laboratory tests to define whether the patient has primary, idiopathic nephrotic syndrome or a secondary cause related to a systemic disease.,Common screening tests include the f
23、asting blood sugar and glycosylated hemoglobin tests for diabetes, and antinuclear antibody test for rheumatoid disease, and the serum complement, which screen for many immune complex-mediated disease (Table 3), In selected patients, cryoglobulins, hepatitis B and C serology, anti-neutrophil cytopla
24、smic antibodies (ANCAS), anti GBM antibodies, and other tests may be useful. Once secondary causes have been excluded, treating the adult nephrotic patient often requires a renal biopsy to define the pattern of glomerular involvement.,It leads to a multitude of other consequences , such as predispos
25、ition to infection and hypercoagulability. In general, the diseases associated with NS cause chronic kidney dysfunction, but rarely they can cause ARF. ARE may be seen with minimal change disease, and bilateral renal vein thrombosis.,Complications,Infection Coagulation disorders Protein malnutrition
26、 and dyslipidemia Acute renal failure,Treatment 治疗,1. General treatment 2. Symptomatic treatment (e.g.diuresis to relieve edema, treating dyslipidemias, anticoagulate treatment, etc.) 3. Immunosupressive treatment,一、一般治疗 二、利尿消肿 三、免疫抑制治疗 四、调脂药物 五、抗凝治疗,Thank you,急性肾小球肾炎的的病理改变是肾脏体积可较正常增大，病变主要累及肾小球。病理类型
27、为毛细血管内增生性肾小球肾炎。光镜下通常为弥漫性肾小球病变，以内皮细胞及系膜细胞增生为主要表现，急性期可伴有中性粒细胞和单核细胞浸润。病变严重时，增生和浸润的细胞可压迫毛细血管袢使毛细血管腔变窄、甚至闭塞，并损害肾小球滤过膜，可出现血尿、蛋白尿及管型尿等；并使肾小球滤过率下降，因而对水和各种溶质（包括含氮代谢产物、无机盐）的排泄减少，发生水钠潴留，继而引起细胞外液容量增加，因此临床上有水肿、尿少、全身循环充血状态如呼吸困难、肝大、静脉压增高等。肾小管病变多不明显，但肾间质可有水肿及灶状炎性细胞浸润。 急性肾小球肾炎治疗：本病治疗以休息及对症为主，少数急性肾功能衰竭病例应予透析，待其自然恢复。不
28、宜用激素及细胞毒素药物。 一、一般治疗 肉眼血尿消失、水肿消退及血压恢复正常前应卧床休息。予低盐（3g/d）饮食，尤其有水肿及高血压时。肾功能正常者蛋白质入量应保持正常（每日每公斤体重1g），但氮质血症时应限制蛋白质摄入，并予高质量蛋白（富含必需氨基酸的动物蛋白）。仅明显少尿的急性肾功能衰竭病例才限制液体入量。 二、治疗感染灶 首选青霉素（过敏者更换为对革兰氏阳性菌高度敏感的大环内酯类、头孢第一代抗生素）800万单位静脉滴注，1014天，但其必需性现有争议。反复发作的慢性扁桃体炎，待肾炎病情稳定后（尿蛋白小于（+），尿沉渣红细胞少于10个/高倍视野）可作扁桃体摘除，术前、后两周需注射青霉素。
29、三、对症治疗 利尿、消肿、降血压。常用噻嗪类利尿剂（如双氢氯噻嗪25mg,每日23次），必要时才予利尿剂如呋塞米2060mg/d,注射或分次口服。利尿后高血压值仍不满意时，可加用钙通道阻滞剂如硝苯啶2040mg/d,分次口服或血管扩张药如肼酞嗪25mg,每日3次。但保钾利尿药（如氨苯蝶啶及安体舒通）及血管紧张素转化酶抑制剂，少尿时应慎用，以防诱发高血钾。 慢性肾炎 图书四、中医药治疗 本病多属实证。根据辨证可分为风寒、风热、湿热，分别予以宣肺利尿，凉血解毒等疗法。本病恢复期脉证表现不很明确，辨证不易掌握，仍以清热利湿为主，佐以养阴，但不可温补。 五、透析治疗 少数发生急性肾功能衰竭而有透析指征时，应及时给予透析（血液透析或腹膜透析皆可）。由于本病具有自愈倾向，肾功能多可逐渐恢复，一般不需要长期维持透析。 ，它有力地推进了临床医学和预防医学。治疗和预防疾病的有效（df肺25s血液f369血小板t5172红血球gdf55m白血球fd2）手段在20世纪才开始出现。20世纪医学发展的主要原因是自然科学的进步。各学科专业间交叉融合，这形成现代医学的特点之一。 综合医学,
