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1、urinarysystemurinary systemUrinarysystemincludesthekidney、ureter、bladderandurethra.Thekidneyisanimportantexcretoryorgan,also,itisanessentialadjusterandendocrineorgantomaintainaprecisebalanceofinternalenvironment.anatomical characteranatomical characterneonateneonate largerlarger，inmatureinmature，sma
2、ll small glomerulus，short short tubuleinfantinfant Ureterismuchlongerandincurvater,tubemusclesandelasticfibersaredysontogenesis. Ureterclosetothebladderisstraighterandshorter,itispoortopreventurinereverseflow.femaleinfanthasashorterurethra,butmaleinfantoftenhascapistration physiologiccharacterglomer
3、ularfiltrationcommencesinthemetanephrickidneyatabout9to12weeksofgestation，thefullcomplementofnephronsispresentat36weeksofgestation.lowreservecapacity，inmatureregulatorymechanism，easytodysfunction。therenalfunction(calculatedbybodyweightorbodysurfacearea)reachesavaluecomparabletoadultat1to2yearsofage.
4、glomerular filtration rateglomerular filtration rateneonateneonatethefirstweekofbirth:glomerularfiltration2year-old:adultlevelpathologypathologyextramoistureandsolutecannotexcretionintimeedema the reabsorption and the excretory the reabsorption and the excretory function of renal tubule function of
5、renal tubule glomerulotubularbalanceneonatehasalreadypossessglomerulotubularbalance，butinmature。aminoacid/glucoseterminfant:normalreabsorptioncapacityprematureinfant:renaltubulefunctiondeficiency，glucosethresholdglucosuria(reabsorptglucose） natriumnatriumneonatealdosteronenatriumpositivebalanceglome
6、rularfiltrationratenatriumloadnatriumeexcretionedemainfantoflow-birthweightnatriumeductionifnatriumeintake（3mmol/kg/d）hyponatremiashockorconvulsionpotassiumpotassiumbecauseNa-K-ATPenzymesystemofrenaltubulesepitheliumisinmature，neonatepotassiumexcretionin10ayshyperkalemiatendency。acid-base balanceaci
7、d-base balancekidneyexcretH、reabsorbHCO3acid-basebalanceat2weeksafterbirththeurinePHgettheadultlevelat2yearsoldthecapabilityofammoniaexcretionclosetoadultlevelrenalthresholdofHCO3inneonateislow。urine concentration and durine concentration and di ilutionlutionurinedilutionabilityofnewbronorinfantsclo
8、setoadultglomerularfiltrationratebodyproteinanabolismactivelyureaexcretioncannotformenoughosmoticpressuregradientinkidneymedullaurineconcentrationrenal endosecretory functionrenal endosecretory function renin reninprostaglandinprostaglandinkallikreinkallikrein erythropoietin erythropoietin 1,25- 1,2
9、5-dihydroxycholecalciferoldihydroxycholecalciferolurinate characterurinate characterneonateurinationin24Hr(93%)urinationin48Hr(99%)pathology oliguria400ml/danuria50ml/ddailyurinaryvolume(ml)=400+100(age1)urine characterurine characteryellow,clarity，specificgravityofurineclosetoadultafter1year，24HrUr
10、ineprotein100mg/m2(150mg/d)、RBC、WBC、urinarycylinder、Addisscount、pHexaminationexamination ofofkidney disease kidney disease EXAMNATIONOFTHEKIDNEYDISEASEEXAMNATIONOFTHEKIDNEYDISEASEEVALUATIONOFTHEKEDNEYANDURINARYTRACT blood-vascularsystemrenalangiographynephrogramnephrogramrenal insufficiencyrenal ins
11、ufficiency EXAMNATIONOFTHEKIDNEYDISEASEEXAMNATIONOFTHEKIDNEYDISEASEEVALUATIONOFTHEKEDNEYANDURINARYTRACT Renal glomeruli Renal glomeruli proteinuria urinary sediment proteinuria urinary sediment blood urea nitrogen blood urea nitrogen（BUN） creatinine creatinine（Cr）bloodblood2 2MGMG creatinine clearan
12、ce creatinine clearance （Ccr）=Kheight(cm)Scr(mg/dl) (K：1岁岁infantoflow-birthweight=0.33；1岁岁matureinfant=0.45；212岁岁=0.55) CystatinC(Cys-C) proteinuria proteinuria quantitativeexamination150mg/dtubularproteinuria5%, 5%, diagnostic。CysCdiagnosis and differential diagnosis and differential diagnosisdiagn
13、osisdiagnosticcriteria：medicalhistorysymptoms&signslaboratoryexaminationdifferentialdiagnosis：IgAglomerulonephritismesangialproliferativenephritis,MSPGNrapidlyprogressiveglomerulonephritis,RPGNAcuteonsetofchronicnephritispurpuranephritisHBV-associatedglomerulonephritislupusnephritis,LNidiopathicneph
14、roticsyndrome,INStherapyRestDietanti-infectionsymptomatictreatmentDialysisChinesemedicineSeverecomplicationtreatmentprognosis95eusemia2007年尿液普查后转新华医院儿肾就诊情况年尿液普查后转新华医院儿肾就诊情况下级医院转诊病例下级医院转诊病例235例例/年年确诊肾炎随访一年肾活检共确诊肾炎随访一年肾活检共20例例1.女女10岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病伴肾小血管壁轻度纤维化轻微病变肾小球疾病伴肾小血管壁轻度纤维化MC32.女女10岁岁镜下血尿一
15、年镜下血尿一年局灶节段性肾小球透明变性局灶节段性肾小球透明变性C33.男男6岁岁镜下血尿一年镜下血尿一年薄基膜病薄基膜病G4.女女5岁岁镜下血尿一年镜下血尿一年薄基膜病薄基膜病C35.男男9岁岁镜下血尿镜下血尿10月月轻微病变肾小球疾病轻微病变肾小球疾病M6.女女7岁岁镜下血尿一年镜下血尿一年局灶节段增生性肾小球肾炎局灶节段增生性肾小球肾炎7.男男3岁岁镜下血尿一年镜下血尿一年IgA肾炎（轻微病变型）肾炎（轻微病变型）A8.男男4岁岁镜下血尿一年镜下血尿一年IgA肾炎（轻度局灶节段增生型）肾炎（轻度局灶节段增生型）AC3弱弱FN弱弱9.女女5岁岁镜下血尿一年镜下血尿一年肾小管轻微病变肾小管轻微
16、病变M弱弱FN弱弱10.女女10岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病G弱弱FN弱弱11.女女3岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病MFN12.男男6岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M13.女女3岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M14.女女4岁岁镜下血尿一年镜下血尿一年薄基膜病薄基膜病15.女女5岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M弱弱FN弱弱16.男男6岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M弱弱17.男男6岁岁镜下血尿
17、一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M18.女女4岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病M19.男男5岁岁镜下血尿一年镜下血尿一年轻微病变肾小球疾病轻微病变肾小球疾病C320.女女9岁岁蛋白尿一年蛋白尿一年轻微病变肾小球疾病轻微病变肾小球疾病M思考题思考题 1. 1. 怎样理解急性肾小球肾炎水肿、怎样理解急性肾小球肾炎水肿、 血尿、蛋白尿、高血压的发生内血尿、蛋白尿、高血压的发生内 在联系。在联系。2. 2. 急性肾炎的严重病例循环充血与急性肾炎的严重病例循环充血与 肺炎心衰在发病机理与处理上有肺炎心衰在发病机理与处理上有 何不同。何不同。urinar
18、ysystem上海交通大学医学院附属新华医院上海交通大学医学院附属新华医院儿内科教研室儿内科教研室卫敏江卫敏江nephroticsyndromeNSmass proteinuriamass proteinuria hypoproteinemiahypoproteinemiahypercholesterolemiahypercholesterolemiaedemaedema“三高一低三高一低”clinicalclassification congenitalnephritisprimarynephroticsyndromesimplenephropathynephrosonephritissec
19、ondarynephroticsyndromepathogenyprimarynephroticsyndrome-concerncellularimmunitycongenitalnephropathy-euchromosomerecessivehereditypathologysimpleNSMCDiscommon（about80%），），footprocessfusefusepathologynephritisNSmesangiumhyperplasiamembranatemembrano-proliferativetypeFSGS系膜细胞中度增生系膜细胞中度增生pathogenesisG
20、BMlesion，proteinleakagenegativenitrogenbalanceSerumlipidsserumALBeffectivebloodvolumeplasmacolloidosmoticpressureALD，ADHfluidenterinterstitialspacetubulereabsorbsodiumandwateredemaclinicalmanifestation Finlandcongenitalnephropathynon-Finlandclinicalmanifestation simpleNSmassproteinuria,hypoproteinem
21、ia,hypercholesterolemia&edemanephritisNSmassproteinuria,hypoproteinemia,hypercholesterolemia&edemahypertension、hematuria、 BUNScr,complementlaboratoryexamination routineurinetest24Hrproteinquantitativeurine(50mg/Kg/day)urineproteinelectrophoresis：smallmoleculeprotein:molecularweight16.9Mr，tubularprot
22、einuria;middlemoleculesprotein:molecularweight79.9Mr(albumin);macromoleculeprotein:molecularweight10Mr(IgG15Mr)，selectivealbuminuria/nonselectivealbuminuriaserumalbumin(30g/L)。cholesterin（5.7mmol/L)serumcomplementdeterminelaboratoryexaminationofsystemicdiseaselaboratoryexaminationofhypercoagulatives
23、tate&thrombosisbiopsysimpleNSnephritisNSAge167pathologicalchangeMCDMsPGN、MPGN、FSGSsymptomfourchiefsymptomsfourchiefsymptomshypertensionhematuriaAlbuminuriaselectivenonselectiveComplemennormalsomereduceRenalfunctionnormalsomereduceUrineFDPnegativepositiveReactiontogood，easytorelapsepoorglucocorticoid
24、courseofthediseaserelievein48weeksdelayedeasytorelapse,12yearcomplication 1.infectioninfectionURI,skin,urinarytract,peritonitis,etc.2.hypovolemic shockhypovolemic shock3.electrolyte disturbanceelectrolyte disturbancehyponatremia,lowpotassiumphosphate,hypocalcemia,etc.4.thrombogenesisthrombogenesis5.
25、acute renal failureacute renal failure6.renal tubules malfunctionrenal tubules malfunction7.delayed growthdelayed growthtreatmentgeneraltreatmentrest,diet,sodium12g/day，intakeVitD，calcium，protein1.52g/kg/dayinfectionpreventionvaccination、 theinfectionproblemduringtheglucocorticoidDetumescence&diures
26、ispayattentiontoacid-basebalance&electrolytedisturbanceGlucocorticoidtreatment mechanism:decreaseimmunoreaction，improvecapillarypermeability，reduceurineproteineduction.DecreaseADH&ALD，inncreaseGFR,diuresisshortrangealmostdesuetudeintermediaterange1.52mg/Kg/d,tid-qid,continuedosetill2weeksafterurinep
27、roteinischangetonegative(need4weekstobasicallycompleted)Thetwo-thirdsdoseofthetotaldoseof2days,qod，4weeks，thenreduce510mgper2-4weeks(need6monthstobasicallycompleted)longrangesameastheintermediaterange，thenreduce2.55mgper24weeks(need9monthstobasicallycompleted)Longtermlowdoseglucocorticoid(0.4mg/Kg/q
28、od),wouldnotleadtosideeffect.Enoughdoseatfirst,decreasedoseslowly,maintenancedoseshouldbelong.immunosuppressorindicationa.Frequentrelapse：relapse&reiteration1year3times、6monthstwice。b.Glucocorticoiddependence：sensitivetoglucocorticoid,can relieve，but relapse or reiteration in 2 weeks if dosereductio
29、n/drugwithdrawal，repeat23times。c.Glucocorticoid Resistant： regular glucocorticoidtreatment8weeks,urineprotein+immunosuppressorCyclophosphamideperos:2.53mg/Kg/d，accumulateddose200mg/Kg，course3monthsinveinstosstherapy:812mg/Kg/time，accumulateddose150mg/Kg,per2weeks，graduallyextendntermittenceaccording
30、toconditionhydrateatthefirstandlast3daysofstosstherapy。sexglandChlorambucilPeros：0.2mg/Kg/d,couse6months，accumulateddose10mg/Kg。Sideeffect：sexglandCyclosporinACsACy-AAzathioprineMycophenolate（FK506）TacrolimusAnticoagulant&fibrinolysisHeparinSodiumHeparinCalciumUrokinaseDipyridamoleangiotensin-conver
31、tingenzymeinhibitormechanism:improvelocalglomerulushemodynamics，reducealbuminuria，delayglomerulussclerosis Enalapril Maleate Tablets Enalapril Maleate Tablets Benazepril Hydrochloride ACEI Benazepril Hydrochloride ACEI Sodium Fosinopril Sodium Fosinopril Losartan Potassium ARB Losartan Potassium ARB
32、 chinesemedcine improvegeneralconditions，mitigatedrugside-effect雷雷公公滕滕多多甙甙片片 inhibit cell immunity，antiinflammator,reducealbuminuria，peros：11.5mg/kg/d,bidtid,couse36months。side-effect：sexglandprognosissimpleNS9095%sensitivetoglucocorticoid，buteasytorelapse。nephritisNSpartialevolvetoESRDin1015years思考
33、题思考题1.紧张性水肿与凹陷性水肿产生机理？紧张性水肿与凹陷性水肿产生机理？2.三岁单纯性肾病患儿，请开出医嘱。三岁单纯性肾病患儿，请开出医嘱。Pathologicaltypespathologicalchangesdistributinginglomerulardiseasefocal:partialglomerulusbeinconvenienceddiffuse:mostlyglomerulusbeinconveniencedpathologicalchangesdistributingincapillaryloopsofglomerulussegmental:partialbeinconveniencedglomerular:mostlybeinconveniencedmesangialcellinnormal,:mesangialproliferative
