肾炎肾病幻灯课件.ppt

1、Acute GlomerulonephritisBeijing Childrens Hospital affiliated to Capital University of Medical SciencesMeng QunAcute GlomerulonephritisnDefinition nEtiology and PathogenesisnPathologynClinical featuresnLaboratory findingsnDiagnosis and differential diagnosisnTreatmentDefinitionnacute onsetnpostinfec

2、tious glomerulonephritis:pharyngitis or pyodermanoften belong to acute poststreptococcal glomerulonephritis(APSGN)nresult of inflammatory glomerular injuryDefinitionnhematuria nproteinurianedema nhypertension nrenal insufficiencyncommon age:514y 2y:rare nmale/female2/1nself-limitednprognosis:fine nm

3、orbidity:on the decline Etiologynbacteria,viruses,mycoplasma ngroup A-hemolytic streptococcintype 12:pharyngitisntypes 2,49,50,55,60:pyoderma ntype 49:pharyngitis or pyodermaPathogenesis nsecondary to a direct toxic effect on the glomerulus of a streptococcal proteinnthe streptococcal product induce

4、 an immune complex-mediated injury 1)introducing an antigen to the glomrulus:planted antigen 2)deposition of circulating immune complexes 3)altering a normal renal antigen to a self-antigen inducing an autoimmune response to the self-antigen streptococcal antigenCICin situ ICtriggerautoimmunitycompl

5、ement activitedinflammatory glomerular injuryGBM damagedhematuriaproteinuria proliferative mesangial and endothelial cellsGFRoliguria,edema,hypertentionPathologyndiffuse global hypercellularitynproliferative mesangial and endothelial cellsncrescent formation nsubepithelial depositionninterstitial ed

6、ema ninterstitial infiltration of leukocyteClinical featuresnpresent from asymptomatic to oliguric acute renal failurenlatent period:after pharyngitis:612 days after skin infection:1428 daysNonspecific symptoms and signs tiredness headache appetite slightly fever vomiting the sign of infectionClassi

7、cal manifestation(1)hematuria gross hematuria:5070%patients coffee-colored or tea-colored turn to microhematuria:after 12wClassical manifestation(2)edema:70%patients reason:sodium and fluid retention presents:face periorbital upper extremitiesClassical manifestation(3)hypertention:3080%patients mild

8、 to moderate school age patients130/90mmHg pre-school age patients120/80mmHgClassical manifestation(4)proteinuria:the extent is various most 3g/d nephrotic-range proteinuria:20%patients urine volume:Severe case(1)nsevere hypervolemia:often occurs 150160/100110mmHg reason:CNS vasculitis manifestation

9、s:headache vomiting confusion somnolence convulsion coma Severe case(3)nacute renal failure:last 35d,10d mesangial and endothelial cells proliferation blood volume of capillary glomerular filtration rate(GFR)Non-Classical manifestation(1)nasymptomatic AGN:microhematuria without other clinical manife

10、station contacting AGN patients or when streptococci is epidemicNon-Classical manifestation(2)nextrarenal AGN:edema hypertention C3 ASO urine test:nomal or slightly abnomalNon-Classical manifestation(3)nnephrotic syndrome like AGN:severe edema hematuria proteinuria hypoalbuminimia hypercolesteralimi

11、a Laboratory findings(1)urine:dysmorphic red blood cell red blood cell castscasts of leukocytesrenal tubule epithelial cellshyaline and granular castsproteinuriaerythrocyte sedimentation rate:antistreptolysin O(ASO):pharyngitis 1014 days of phase reach the hightest titer in 35weeks,return to nomal i

12、n 36 monthantideoxyribonuclease B(+):pyoderma Laboratory findings(2)ncomplement:return to normal 8 weeksncirculationg immune complexes:(+)nBUN,Cr:correlate with disease activity Laboratory findings(3)Diagnosisnacute onset+abnomal urine test or edema,oliguria,hypertentionnAPSGN:above +streptococcal i

13、nfectial disease +increased ASO +decreased C3Differential diagnosis(1)nAGN caused by other etiologies and pathogenic mechanisms:such as virusesDifferential diagnosis(2)nIgA nephropathy latent:24-48h no edema,hypertention C3:normal Differential diagnosis(3)Chronic GN with acute episode:latent:short(1

14、2 days)patients:malnutrition anemia growth retarded renal function:usually abnormal proteinuria:severe gravity of urine:Differential diagnosis(4)nIdiopathic nephrotic syndrome:ASO:normal Pathology Differential diagnosis(5)rapidly progressive glomerulonephritis(crescentic glomerulonephritis)oliguria

15、or anuria rapid loss of renal funtionsecondary GN:Lupus nephritis Purpura nephritis HBV-associated glomerulonephritis Treatment nno specific treatmentnsupportive care General treatment nstay in bed:23 weeks nsalt intake:60mg/Kg.dnlimit protein intake:0.5g/Kg.d(azotemia period)Appropriate antibiotic

16、therapynpenicillin G:1014 days nother antibiotics:according to sensitivity test Other treatmentEdema:control liquid and salt diuretics Hypertention:rest,control liquid and salt Nifedipine CaptoprilTreatment of severe cases(1)nSevere hypervolemia:restrict the intake of liquid and salt loop diuretics:

17、furosemide sodium nitroprusside dialytic supportnHypertensive encephalopathy:sodium nitroprusside control convulsionnAcute renal failure:comprehensive therapyTreatment of severe cases(2)Prognosis and preventionnrecovery:95%nsevere cases may progressed to chronic GN and CRFnprevention:prevent infecti

18、onNephrotic SyndromeDefinition(NS)massive proteinuria hypoproteinemia edema hyperlipidemianmay occur as a result of any form of glomerular disease nmay be associated with a variety of extrarenal conditionsnaccording to etiology:primary NS:simple and nephritic type NS secondary NS congenital NS nusua

19、lly affects pre-school childrennthe most common age:35 yearsnmale/female3.7/1nmost common form of nephropathynracial and genomic and enviromental background:nephrin-NPHS1,podocinnsteroid responsive NS:associate with HLA-DR7nfrequently relapse:correlate with HLA-DR9nsteroid resistant NS:NPHS2 Etiolog

20、y and Pathogenesis nunclear the change of formation and electron of capillary glomerular permeability to proteinminimal change NS:no immune complex deposition negative charge of barrier:injury (cell-mediated immunity)non-minimal change NS:Ig and/or C deposit at glomerruli damage the filtrated barrie

21、rPhysiopathology nProteinuria:the most basic clinical character the fusion of foot processes of the visceral epithelium of GBM the negative charge glomrular permeability to proteinHypoproteinemianreason:the loss of albumin in urine synthesis of albumin no proper intaking proteinnserum albumin is neg

22、atively correlated with the severity of proteinuriaEdema the most common symptoms:n1)hypoproteinemia plasma oncotic pressure hypovolemian 2)serum albumin 25g/L fluid into the interstitial space 15g/L asitesn3)tubular sodium reabsorptionn4)the defect of Na arrangementn5)serum ADHHyperlipidemia hyperc

23、holesterolemia hypertriglyceridemia synthesis low-density lipoproteins catabolism very low density lipoproteinsdamage vasculature induce glomerulosclerosisncorrelated with hypoalbuminemia proteinuriaPathologynminimal change NS:76.4%nmembranoproliferative GN:7.5%nfocal segmental glomerulosclerosis:6.

24、9%nmesangial proliferative GN:2.3%nfocal glomerulosclerosis:1.7%nmembranous nephropathy:1.5%nMCNS:Light Microscopynno glomerular lesions nminimal focal segmental mesangial prominence nthe matrix:no expanded to the extent that capillary lumens are compromisedncapillary walls:thin and capillary lumens

25、 patentnprotein and lipid resorption droplets in tubular epithelial cellninterstitial edema:rareMCNS:Electron Microscopynthe effacement of visceral epithelial cell foot processesnmicrovillous transformationnglomerular and proximal tubular epithelial cells have increased clear and dense cytoplasmic d

26、ropletsMCNS:Immunofluoresenses Microscopynno remarkable findings nlow-level mesangial staining for IgMmembranoproliferative GNmesangial proliferative GNmembranous nephropathyClinical Manifestation(1)cardinal clinical feature:abrupt onset of edema heavy proteinuria hypoalbuminemia hyperlipidemiaClini

27、cal Manifestation(2)hematuria:unusualhypertention:not commontransient GFR:30%patients caused by hypovolemiathe function of renal:nomalARF:rareComplicationsnInfections nElectrolite disturbance and hypovolemianHypercoagulability and thrombosisnAcute renal failurenTubular function lesionnGrowth retarde

28、d Laboratory findings nurine analysis:proteinuria:40mg/h.m2 or 50mg/kg.d Upro/Ucr 3.5 microscopic hematuria:5.72mmol/L or 220mg/dl edemaDifferential diagnosis(1)Nephritic type NS above points+at least as followsnurine test:dysmorphic red blood cell RBC10/HP 3 times 2 weeksnhypertention:school age ch

29、ildren 130/90mmHg pre-school age children 120/80mmHgnrenal function insufficiency npersistent complementnSecondary NS:APSGN Lupus nephritis Purpura nephritis HBV-associated glomerulonephritis Differential diagnosis(2)Treatment:General treatment nrest in bedndietary:salt 12g/d high quality protein 1.

30、52g/kg.d Vit D 400u/d,Cansevere edema and hypertension:limit liquid and salt antihypertension diuretics ncontrol and prevent infectionnknowledge education Corticosteroid therapynPrednisone:2mg/kg.d maximum 60mg/dnprinciple:enough dosage slowly taper long remain VitD,CalciumNew patients:nshort term t

31、herapy:8 weeks prednisone 2mg/kg.d4w 1.5mg/kg.qod 4wnmiddlelong term therapy:69 months prednisone 1.52mg/kg.d48w QOD4w slowly taper Side effects of steroidnAltered glucose metabolism:hyperglycemia and glycosurianCessation of growthnCushingoid habitusnElevated blood pressurenBehavior and personality

32、changesnHypercoagulatory state,thrombosisnAdrenal insufficiencynInfectionnOsteoporosisnSome conceptsnSteroid-responsive NS:pred8w,Upro(-)nSteroid-resistant NS:pred8w,Upro(+)nSteroid-dependent NS:response to steroid taper or discontinued1month,relapse 2 timesnRelapse:Upro(-)Upro(+)2wnFrequently relap

33、se:6mon relapse 2times 1 year relapse 3 timesFrequently relapse or steroid dependent patients:adjust the steroid dosage adjust period of steroid change to other steroid:dexamethasonemethylprednisoloneImmunosuppressive therapy nfrequently relapse nsteroid dependent nsteroid resistant nserious side ef

34、fects with steroid therapy Cyclophosphamide,cyclosporin A,Cyclophosphamiden22.5mg/kg.d Tid po812w 200mg/kgn1012mg/kg.d iv 2d/2w 150mg/kgn500mg/m2 iv/mon 68m 200mg/kgnSide effects:bone marrow suppression(WBC PLT )liver function hemorrhagic cystitis gonadal dysfunction Anticoagulant nSodium Heparin:1m

35、g/kg.d 24weeks ivnUrikinase:360000u/d 12weeks ivnDipyridamole:510mg/kg.d po Tid6mEnhance immune functionnLevamisole:2.5mg/kg,d Qod6mnImmunogloblin:400mg/kg.d 5 daysnAngiotensin converting enzyme inhibitor(ACEI)Captopril Enalapril Fosinopril nTraditional herbs OthersCriteria of recoverynclinical cure

36、d:stop treatment for 3 years total remission no relapsentotal remission:laboratory test is nomal.npart remission:proteinuria+Prognosisnclosely correlated with pathologynminimal change NS:fineClinical features of the various types of pathology of NSMCNS FSGS MPGN MNAgeMale/femaleHematuriaBPSCrC3Sensi

37、tive to steriodPrognosisRecurrence after transplantation 16 every age 616 114 2/1 3/2 1/1 3/1 A few many many many A few some many some A few some many some -68%+-93%25%-Good bad bad not so bad -+someDifferences between children and adults with NS children adults24h Upro 50mg/kg 3.5gpathology MCNS M

38、Netiolgy primary NS secondary NSresponse to steroid most well some wellcase12 years maleEarly morning facial edema 5 days generalized edema with ascites 3 days urine output diminishedCurrent immunizationPhysical examination uncomfortable-appearing obvious anasarca BP95/58mmHg periorbital edema+breat

39、h sounds diminished heart tones normal abdomen distended fluid wave+pitting edema of the lower extremities+no rash joints normal No family history of renal disease w 15kgUrinalysis:pro+1.030 PH 5.5 RBC-cast Urine culture:-Hemogram:normalComplement:normalANA:-Hepatitis B surface antigen:-Albumin:25g/

40、LBUN:normalCholesterol:8.9mmol/L24-hr urine total protein:1.8gTuberculin test:-questionsnWhat is the diagnosis?nMake differential diagnosisnHow to treat the patient？case28 years maleEarly morning facial edema 5 days generalized edema with ascites 3 days coffee-colored urine oliguriaHad upper respira

41、tory infection 2 weeks agoCurrent immunizationPhysical examination uncomfortable-appearing BP 105/70mmHg periorbital edema+breath sounds diminished heart tones normal abdomen distended fluid wave+pitting edema of the lower extremities+no rash joints normalNo family history of renal disease w 30kgUrinalysis:pro+1.030 PH 5.5 RBC+Urine culture:-Hemogram:Hb10g/LANA:-Hepatitis B surface antigen:-Albumin:20g/LBUN:normalCholesterol:8.5mmol/L24-hr urine total protein:3.0gTuberculin test:-ASO C