《版肾病综合征》ppt课件

1、Central South UniversityCentral South UniversityCentral South UniversityCentral South University肾病综合征肾病综合征Nephrotic SyndromeNephrotic Syndrome，NSNSCentral South UniversityCentral South University 肾病综合症肾病综合症nephrotic syndrome，NS病理生理与临床病理生理与临床前言前言发病机制发病机制病理病理 治疗治疗并发症并发症诊断诊断Central South UniversityCent

2、ral South UniversityPreface由于肾小球基膜的通透性由于肾小球基膜的通透性glomerular glomerular basement membrane permeabilitybasement membrane permeability添加，导添加，导致血浆内大量蛋白质从尿中丧失的临床综合征。致血浆内大量蛋白质从尿中丧失的临床综合征。Clinical characterClinical character大量蛋白尿大量蛋白尿(proteinuria) (proteinuria) 低蛋白血症低蛋白血症(hypoalbuminemia)(hypoalbuminemia)高

3、脂血症高脂血症(hypercholesterolemia)(hypercholesterolemia)不同程度水肿不同程度水肿(edema)(edema)Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic Syndrome one of the commonest renal one of the commonest renal diseases in childhood, An diseases in childhood, An increased glomerular increased g

4、lomerular permeability resulting in permeability resulting in proteinuria is the primary proteinuria is the primary renal abnormality in NS.renal abnormality in NS.PrefaceCentral South UniversityCentral South University 肾病综合症肾病综合症nephrotic syndrome，NS病理生理与临床病理生理与临床前言前言发病机制发病机制病理病理 治疗治疗临床并发症临床并发症诊断诊断

5、Central South UniversityCentral South UniversityNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeCentral South UniversityCentral South University 肾病综合症肾病综合症nephrotic syndrome，NS病理生理与临床病理生理与临床前言前言发病机制发病机制病理病理 治疗治疗临床并发症临床并发症诊断诊断Central South UniversityCentral South U

6、niversityPathologyNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromePathologyCentral South UniversityCentral South UniversityPathology正常肾小球构造方式图正常肾小球构造方式图Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeHE染色染色PAS染色染色Masson染色染色PASM染

7、色染色Pathology HE 染色染色Central South UniversityCentral South UniversityPathology Model微小病变性肾病，左正常，右上皮细微小病变性肾病，左正常，右上皮细胞足突广泛交融、消逝胞足突广泛交融、消逝Nephrotic SyndromeCentral South UniversityCentral South UniversityPathology LMPASM-HE400 正常肾小球正常肾小球 PAS400 肾小球构造根本正常肾小球构造根本正常Nephrotic SyndromeCentral South Universi

8、tyCentral South UniversityPathology EMEM13000 肾小球上皮细胞足突广泛交融变平，这是肾小球上皮细胞足突广泛交融变平，这是该病的特征性病变该病的特征性病变EM8400 正常肾小球正常肾小球 显示正常的肾小球基底膜显示正常的肾小球基底膜(BM)，内皮细胞内皮细胞(En)，足细胞，足细胞(P)及足突及足突(FP)Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromePathology系膜增生性肾小球肾炎，左正系膜增生性肾小球肾炎，左正常，右系膜

9、细胞和基质增生，常，右系膜细胞和基质增生，电子致密物电子致密物D堆积堆积Central South UniversityCentral South UniversityNephrotic Syndrome肾小球系膜增生方式图肾小球系膜增生方式图自左至右：轻度、中度和重度系膜增生自左至右：轻度、中度和重度系膜增生PathologyCentral South UniversityCentral South UniversityNephrotic SyndromePathologyPAS400 系膜增生性肾小球肾炎系膜增生性肾小球肾炎Central South UniversityCentral S

10、outh UniversityNephrotic SyndromePathologyPAS400 局灶节段性肾小球硬化局灶节段性肾小球硬化Central South UniversityCentral South UniversityNephrotic SyndromePathologyPASM400 肾小球缺血性硬化症肾小球缺血性硬化症Central South UniversityCentral South UniversityNephrotic SyndromePathology膜性肾病，左正常，右上皮膜性肾病，左正常，右上皮下免疫复合物堆积下免疫复合物堆积D，GBM增厚，增厚，钉突构成

11、钉突构成SCentral South UniversityCentral South UniversityNephrotic SyndromePathologyMasson800膜性肾病膜性肾病,基底膜弥漫基底膜弥漫增厚，上皮下大量嗜复红蛋白沉淀增厚，上皮下大量嗜复红蛋白沉淀PASM800膜性肾病膜性肾病,基底膜弥基底膜弥漫漫增厚，钉突构成增厚，钉突构成Central South UniversityCentral South UniversityNephrotic SyndromePathologyPASM400膜性肾病膜性肾病,基底膜弥漫基底膜弥漫增厚，链环状构造构成增厚，链环状构造构成C

12、entral South UniversityCentral South UniversityNephrotic SyndromePathology膜增生性肾小球肾炎，左正常，右膜增生性肾小球肾炎，左正常，右系膜增生系膜增生M，广泛插入，广泛插入I，电子致，电子致密物密物D堆积堆积Central South UniversityCentral South UniversityNephrotic SyndromePathologyPASM400膜增生性肾小球肾炎膜增生性肾小球肾炎,系膜细胞系膜细胞和基质重度增生，广泛插入和基质重度增生，广泛插入Central South UniversityCe

13、ntral South UniversityNephrotic SyndromePathologyCentral South UniversityCentral South University 肾病综合症肾病综合症nephrotic syndrome，NS病生与临床病生与临床前言前言发病机制发病机制病理病理 治疗治疗临床并发症临床并发症诊断诊断Central South UniversityCentral South University 正常尿中有微量蛋白质：正常尿中有微量蛋白质： 通常通常100mg/m2 d100mg/m2 d； 150mg/d150mg/d为异为异常常 ( Norma

14、l 100mg/m2 d, ( Normal 100mg/m2 d, Abnormal 150mg/d )Abnormal 150mg/d ) NS NS：定性：定性+；定量；定量50mg/kg d50mg/kg dMassive ProteinuiaNephrotic SyndromeCentral South UniversityCentral South UniversityMassive ProteinuiaNephrotic SyndromeCentral South UniversityCentral South UniversityMassive ProteinuiaNephro

15、tic Syndrome 本病的发病机制迄今本病的发病机制迄今尚不完全明了。普通以为尚不完全明了。普通以为蛋白尿原因于肾小球毛细蛋白尿原因于肾小球毛细血管壁电化学或构造的改血管壁电化学或构造的改动。正常肾小球滤过膜对动。正常肾小球滤过膜对血浆蛋白具有静电屏障和血浆蛋白具有静电屏障和分子滤过屏障作用。分子滤过屏障作用。Central South UniversityCentral South UniversityNephrotic SyndromeMassive ProteinuiaCentral South UniversityCentral South UniversityNephrotic

16、 SyndromeMassive ProteinuiaCentral South UniversityCentral South UniversityJ gastrointestinal tract gastrointestinal tract HypoproteinemiaNephrotic SyndromeCentral South UniversityCentral South UniversityHypoproteinemiaNephrotic SyndromeCentral South UniversityCentral South UniversityHypoproteinemiN

17、ephrotic SyndromeCentral South UniversityCentral South UniversityHypoproteinemiNephrotic SyndromeCentral South UniversityCentral South UniversityEdemaNephrotic SyndromeCentral South UniversityCentral South UniversityHyperlipemiaNephrotic SyndromeCentral South UniversityCentral South University 肾病综合症

18、肾病综合症nephrotic syndrome，NS病生与临床病生与临床前言前言发病机制发病机制病理病理 治疗治疗并发症并发症诊断诊断Central South UniversityCentral South UniversityNephrotic SyndromeInfectionsCentral South UniversityCentral South University Proteinuria and Reduce the level of Proteinuria and Reduce the level of vitamin D) vitamin D) JJ白蛋白白蛋白20g/L2

19、0g/L难以维持正常血容量难以维持正常血容量 (Albumin (Albumin 20g/L)10/HP Hyperlipemia Azotemia Hypertension Persistent low complementCentral South UniversityCentral South University 肾病综合症肾病综合症nephrotic syndrome，NS病生与临床病生与临床前言前言发病机制发病机制病理病理 治疗治疗临床并发症临床并发症诊断诊断Central South UniversityCentral South UniversityNephrotic Synd

20、romeCentral South UniversityCentral South UniversityNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityUse of dexamethasone after 4 months Before illness Central South UniversityCentral South UniversityNephrotic

21、SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic Syndrome TherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeT-B-体构成，最终引起免疫抑制。体构成，最终引起免疫抑