儿科学：肾病综合征

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 UniversityCentral South UniversityP

2、reface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）增加，导增加，导致血浆内大量蛋白质从尿中丢失的临床综合征。致血浆内大量蛋白质从尿中丢失的临床综合征。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)高脂血症高脂血症(hyperchole

3、sterolemia)(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 diseases one of the commonest renal diseases in childhood, An increased in childhood, An increased glomerular permeability result

4、ing glomerular permeability resulting in proteinuria is the primary renal in proteinuria is the primary renal abnormality in NS.abnormality in NS.PrefaceCentral South UniversityCentral South University 肾病综合症肾病综合症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

5、niversity Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeCentral South UniversityCentral South University 肾病综合症肾病综合症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 UniversityPathologyNephrotic SyndromeCentral South Universit

6、yCentral South UniversityNephrotic SyndromePathologyCentral South UniversityCentral South UniversityPathology正常肾小球结构模式图正常肾小球结构模式图Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeHE染色染色PAS染色染色Masson染色染色PASM染色染色Pathology HE 染色染色Central South UniversityCentral South U

7、niversityPathology （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）上皮细胞足突广泛融合、消失胞足突广泛融合、消失Nephrotic SyndromeCentral South UniversityCentral South UniversityPathology PASM-HE400 正常肾小球正常肾小球 PAS400 肾小球结构基本正常肾小球结构基本正常Nephrotic SyndromeCentral South UniversityCentral South UniversityPathology （EM）EM13000 肾小球

8、上皮细胞足突广泛融合变平，这是肾小球上皮细胞足突广泛融合变平，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系膜增生性肾小球肾炎，（左）正，（左）正常，（右）系膜细胞和基质增生，常，（右）系膜细胞和基质增生，电子致密物（电子致密物（D）沉积）沉积C

9、entral South UniversityCentral South UniversityNephrotic Syndrome肾小球系膜增生模式图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 South UniversityNephrotic SyndromePath

10、ologyPAS400 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）上皮下免疫复合物沉积（下免疫复合物沉积（D），），GBM增厚，增厚，钉突形成（钉突形成（S）Central South Un

11、iversityCentral 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,基底膜弥漫基底膜弥漫增厚，钉突形成增厚，钉突形成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，链环状结构形成增厚，链环状结构形成Central South Universi

12、tyCentral 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）沉积）沉积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 UniversityCen

13、tral South UniversityNephrotic SyndromePathologyCentral South UniversityCentral South University 肾病综合症肾病综合症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/m100mg/m2 2 d d； 150mg/d150mg/d为异常为异常 ( Normal 100mg/m2 d, Abnorma

14、l 150mg/d ) NS NS：定性：定性+；定量；定量50mg/kg50mg/kg d dMassive ProteinuiaNephrotic SyndromeCentral South UniversityCentral South UniversityMassive ProteinuiaNephrotic SyndromeCentral South UniversityCentral South UniversityMassive ProteinuiaNephrotic 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。一般认为尚不完全明了。一般认为蛋白尿起因于肾

15、小球毛细蛋白尿起因于肾小球毛细血管壁电化学或结构的改血管壁电化学或结构的改变。正常肾小球滤过膜对变。正常肾小球滤过膜对血浆蛋白具有血浆蛋白具有静电屏障静电屏障和和分子滤过屏障分子滤过屏障作用。作用。Central South UniversityCentral South UniversityNephrotic SyndromeMassive ProteinuiaCentral South UniversityCentral South UniversityNephrotic SyndromeMassive ProteinuiaCentral South UniversityCentral S

16、outh University HypoproteinemiaNephrotic SyndromeCentral South UniversityCentral South University)HypoproteinemiaNephrotic SyndromeCentral South UniversityCentral South University ) ) on the pharmacokinetics) HypoproteinemiNephrotic SyndromeCentral South UniversityCentral South University Hypoprotei

17、nemiNephrotic SyndromeCentral South UniversityCentral South University（From up to down）Pleural effusion,Ascites)EdemaNephrotic SyndromeCentral South UniversityCentral South University）HyperlipemiaNephrotic SyndromeCentral South UniversityCentral South University 肾病综合症肾病综合症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

18、治疗治疗并发症并发症诊断诊断Central South UniversityCentral South University ) Urinary tract infection Nephrotic SyndromeInfectionsCentral South UniversityCentral South University) ) Proteinuria and Reduce the level of vitamin D) Nephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic Syndrome

19、Central South UniversityCentral South University )Nephrotic Syndrome ) )Complication Central South UniversityCentral South University 肾病综合症肾病综合症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 Nephrotic SyndromeNephrotic SyndromeCentral South UniversityCe

20、ntral South UniversitySimple NS)Nephritic NS)Clinical diagnosisNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeClinical diagnosis Simple NS Nephritic NS Pathol MCD non-MCDClinic Hollow edema Simple NS+ Massive proteinuria Persistent hematuria， Hypoproteinemia RBC1

21、0/HP Hyperlipemia Azotemia Hypertension Persistent low complementCentral South UniversityCentral South University 肾病综合症肾病综合症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 UniversityTherapyNephrotic SyndromeCentral South UniversityCentral South UniversityRest)Diet)

22、Salt restriction)Protein)Diuresis)TherapyNephrotic SyndromeCentral South UniversityCentral South UniversityNephrotic SyndromeKey points of therapy) :Enough, Full course of treatment.TherapyCentral South UniversityCentral South University Use of dexamethasone after 4 months Before illness Central Sou

23、th UniversityCentral South University )Prednisone 2mg/kg/d（Max dosage 60mg/d）treat for 4w：Steroid sensitiveSteroid resistanceTherapyNephrotic SyndromeCentral South UniversityCentral South UniversitySteroid dependence) (Frequent relapses)TherapyNephrotic Syndrome )Central South UniversityCentral Sout

24、h UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityNephrotic SyndromeTherapyCentral South UniversityCentral South UniversityN