1、 Acute Glomerulonephritis Nephrotic syndromeMo Wang,Associate professorNephrology Department Childrens Hospital ofChongqing Medical UniversityCase 1 A 7-year-old boy,got periorbital edema and oliguria for 5 days,urine analysis showed proteinuria+，RBC 10-15/HP，cylinduria 1-2/HP，physical examination s
2、howed P:95/min,R:28/min,BP140/100mmHg,mild edema in eyelids and both legs，congestion in throat,heart,lung and abdomen were normal,he had felt blurred vision for 2-4 mins after hospitalized.1.Whats the diagnosis?2.Lav investigations?3.How to manage?Symptoms of urinary tract diseasenDysuria排尿困难nPolyur
3、ia多尿nMicturition尿频nIncontinence of urine尿失禁nHematuria血尿nProteinuria蛋白尿nHypertention高血压nOliguria少尿nEdema水肿kidneyureterbladderCauses of hematuriaNon-glomerular:nInfectionnTrauma外伤nTumours肿瘤nSickle diseasenBleeding disorder出血性疾病nRenal vein thrombosis肾静脉血栓nHypercalciuria高钙尿症Glomerular diseases:nAcute gl
4、omerulonephritisnChronic glomerulonephritisnIgA nephropathynFamilial nephritisnThin basement membrane disease薄基底膜病Acute glomerulonephritis:n Acute-onsetn A panel of diseases with different etiology and pathogenesisn Typical manifestation:Hematuria Edema Hypertention Renal insufficiency(Acute nephrit
5、ic syndrome)NSAGNEpidemiological Investigation Of Urine System In HospitalizationAcute post-streptococcal glomerulonephritis (APSGN)急性链球菌感染后肾小球肾炎急性链球菌感染后肾小球肾炎Outline:definitionn Immune associated disease nFollows infection of the throat or skin with group A-hemalytic streptococci.nClinical features:
6、Hematuria/Proteinuria Oliguria Edema Hypertension Renal insufficiency(Bun,Cr,GFR)n Most common cause for gross hematuria（肉眼血尿）in ChildrennMorbidity:0-20%after streptococcal infectionn Seasonality:Spring and Autumnn Age:5-14 yo,rare under 2 yon Gender:M:F=2:1Outline:general infomationEtiologyn Follow
7、s infection of the throat or skin with certain“nephritogenic”strains 致肾炎菌株of group A betta-hemolytic streptococci Pathway:Winter:streptococcal pharyngitis (serotype 12)咽峡炎 Summer:streptococcal skin infection pyoderma(serotype 49)脓皮病Pathogenesisn The precise mechanisms remain unclear.n Immune complex
8、es mediated:PathogenesisImmune complex play the most important roleAntigens of nephritogenic strain of streptococciImmune complex in situ type Anti-GBM-antibodiesCICActivated complments and inflammatory mediatorsInflammation of glomeruli 原位免疫复合物原位免疫复合物诱发自身免疫诱发自身免疫链球菌致肾炎菌株抗原成分链球菌致肾炎菌株抗原成分循环免疫复合物循环免疫复
9、合物激活补体及其他炎症介质激活补体及其他炎症介质肾小球免疫损伤肾小球免疫损伤Pathogenesis免疫复合物是最主要原因！免疫复合物是最主要原因！PathologyFeatures:diffuse,弥漫性proliferative,增生性exudative，渗出性glomerulonephritisendothelial cellpodocytemesangial cellendothelial cellepithelial cellmesangial cellleukocytediagram of glomeruliproliferation of endothelial cells an
10、d mesangial cellseffusion of leukocytes in lumenPAS 400Normal glomeruliPAS 400Glomerular nephritisLight Microscope:capillaries proliferative glomerulonephritis毛细血管内增生性肾小球肾炎 Enlarged,hypercellular glomeruli with endothelial and mesangial cell proliferation.EM 10,000Glomerular nephritisEM 10,000Normal
11、 glomeruliElectron Microscope electron-dense deposits on the epithelial side of the GBM驼峰样电子致密物沉积在上皮下Immune Fluorescence lumpy-bumpy deposits of IgG and C3 on GBM or in mesangium IF 400Glomerular nephritisPathophysiologyrenal corpuscle肾小体The glomerular capillary loops肾小球毛细血管袢肾小球炎症病变肾小球炎症病变内皮细胞肿胀、系膜内
12、皮细胞肿胀、系膜细胞增生细胞增生 毛细血管管腔狭窄毛细血管管腔狭窄 肾小球血流量肾小球血流量 滤过面积滤过面积 肾小球滤过率肾小球滤过率 球管失衡球管失衡 钠、水潴留，血容量扩张钠、水潴留，血容量扩张 肾小球基底膜肾小球基底膜破坏破坏血血 尿尿蛋白尿蛋白尿管型尿管型尿 氮质血症氮质血症 少尿、无尿少尿、无尿 水肿水肿高血压高血压 循环充血循环充血 PathophysiologyPathophysiologyInflammation of glomeruli Impairment of GBMEffusion of leukocytes ,proliferation of mesangial c
13、ells and endothelial cells GFR BUN and SCrImbalance of glomeruli-tubulus oliguriaWater and salt retentionEdema,hypertension and Circulatory CongestionHematuria and proteinuriaClinical manifestations:n Antecedent infection:90%APSGN with antecedent streptococcal infection Pharyngitis:6-12 days(average
14、 10 days)before onset Skin infections:14-28 days(average 20 days)before onsetDifferent features of APSGN caused by throat or skin infectionAPSGN caused by PharyngitisAPSGN caused by PyodermaSerotype 12(1,3,4)49(2,5,47)Climate Spring Summer or autumnAgeSchool agePre-school ageIntermissionAbout 10 day
15、sAbout 20 daysClinical manifestations:nTypical manifestation典型病例nSevere manifestation重症病例nAtypical manifestation 不典型病例Clinical manifestations:Typical manifestationsn Hematuria:50-70%gross hematuria肉眼血尿,with RBC casts,sometimes microscopic hematuria镜下血尿NormalPinkRedDark grayBrownn Proteinuria:Usually
16、+-+,24-h urine protein quantitation24小时尿蛋白定量：130/90mmHg for school children 120/80mmHg for pre-school childrenClinical manifestations:Typical manifestationsnsevere circulatory congestion严重循环充血 n Encephalopathy due to hypertention高血压脑病n Acute renal failure急性肾功能衰竭Clinical manifestations:Severe manifes
17、tationsn severe circulatory congestion dyspnea呼吸困难,orthopnea端坐呼吸,cough with pink frothy sputum粉红色泡沫痰 heartbests acceleraging心跳加速Clinical manifestations:Severe manifestationsn Encephalopathy due to hypertention headache,dizziness,photophobia,transient blindness,even coma,convulsionClinical manifestat
18、ions:Severe manifestationsn Acute renal failure BUN、Scr nAsymptomatic（无症状性）n Slight UA&severe symptoms（肾外症状型）n Nephrosis-like（肾病型）Clinical manifestations:Atypical manifestationsAntecedent infection，ASO、C3Laboratory findingsn UA:RBC,proteinn Blood routine:diluted anemia(due to water and salt retensio
19、n)n Renal function:BUN,SCr n Evidence of streptococci infection:ASO nComplement:dynamic decreased C3 2W 4W 6W 8W-C31 2 3 4 5 6(g/L)dynamic change of C3,recover in 8 weeksDiagnosisTypical APSGNn History of antecedent infectionn Clinical manifestationsn Laboratory findings:evidence of streptococcal in
20、fection and decreased C3 nNon streptococcal infection glomerulonephritisnRapidly progressive glomerulonephritis(RPGN)急性性肾炎nChronic glomerulonephritis(CGN)慢性肾炎nSecondary glomerulonephritis(SGN)继发性肾炎nNephrotic syndrome(NS)肾炎（肾病型）Differential diagnosisTreatment：No specific treatmentGeneral treatmentnRe
21、st in bed while hematuria and hypertension nDiet:Water and sodium:Restrict sodium intake while severe edema and hypertension Protein:no strict limitation nElectrolyte:must be closely monitored,keep balancenRenal function:must be closely monitornAntibiotics:Penicillin,Cephalosporin,7-10 daysSymptomat
22、ic treatmentnOliguria and edema management-Diuresis利尿:with intravenous administration of Hydrochlorothiazide双氢克尿噻 1-2mg/kg each time Furosemide呋塞米 1-2mg/kg each timenHypertension-Diuresis,antihypertensive降压(ACEI/ARB)nHypertensive encephalopathy-diazepam安定(antispastic treatment)nitroprusside sodium硝普
23、钠(first choice for antihypertension)TreatmentTreatmentSymptomatic treatmentnAcute renal failure-Restrict liquids Blood Purification(BP)血液净化 Hemodialysis(HD)血液透析 Hemofiltration(HF)血液滤过PrognosisnClinical Course 2 weeks-symptoms release 4-8 weeks-urine analysis is normal4-8 months-Addis count（addis计数）i
24、s normalnMore than 95%of APGSN patients recover Excellent prognosisCase 1 A 7-year-old boy,got periorbital edema and oliguria for 5 days,urine analysis showed proteinuria+，RBC 10-15/HP，cylinduria 1-2/HP，physical examination showed P:95/min,R:28/min,BP140/100mmHg,mild edema in eyelids and both legs，c
25、ongestion in throat,heart,lung and abdomen were normal,he had felt blurred vision for 2-4 mins after hospitalized.1.diagnosis:APSGN,hypertensive encephalopathy2.Lav investigations:ASO,C3,BUN,SCr,UA 3.Management:water and salt restriction,diuresis SummarynDefinitionnEtiology and pathogensisnPathologynPathophysiologynClinical manifestation nLab examination nDiagnosisnPrinciple of treatmentnPrognosis
