原发性肾小球疾病-英文.ppt
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原发性肾小球疾病-英文.ppt
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第二章原发性肾小球疾病Chapter2PrimaryGlomerularDisease概述概述肾小球疾病(Glomerulardisease)是指病变位于肾小球的一类以血尿,蛋白尿,水肿和髙血压等为主要表现的肾脏疾病,可导致终末期肾脏病(EndStageRenalDisease,ESRD),分原发性,继发性和遗传性。
原发性病因常不明确,故又称之为特发性,肾小球病变是唯一或主要病变。
继发性病因常比较明确(如系统性红斑狼疮和糖尿病等),肾小球病变为全身性疾病的一个部分,病理变化类型并不单一。
而遗传性肾小球疾病,是遗传基因变异所致的肾小球疾病(如Alport综合征和Fabry病等)。
分类分类原发性肾小球疾病病理分型(根据WHO1995年制定的标准):
n微小病变性肾小球病n局灶节段性肾小球肾炎/肾小球硬化n弥漫性肾小球肾炎膜性肾病增生性肾炎系膜增生性肾小球肾炎毛细血管内增生性肾小球肾炎腹增生性肾小球肾炎/系膜毛细血管性肾小球肾炎新月体(和坏死)性肾小球肾炎增生硬化性/硬化性肾小球肾炎n免疫反应n肾小球免疫损伤的机制n肾小球疾病的进展发病机制发病机制(Themechanismsofglomerularinjury)临床表现临床表现n蛋白尿Proteinurian血尿Hematurian水肿Edeman高血压Hypertensionn肾功能损害RenalInsufficiencyNephroticsyndromeFigure1.Nephroticedema.Figure2.Nephroticedema.NephroticsyndromeThisischaracterizedbyproteinuria(Typically3.5g/24h),hypoalbuminemia(lessthan30g/dL)andedema.Hyperlipidaemiaisalsopresent.PrimaryandsecondarycausesaresummarizedinTable2,3a,bInpractice,manycliniciansreferto“nephroticrange”proteinuriaregardlessofwhethertheirpatientshavetheothermanifestationsofthefullsyndromebecausethelatterareconsequencesoftheproteinuria.PathophysiologyProteinuriaProteinuriacanbecausedbysystemicoverproduction,tubulardysfunction,orglomerulardysfunction.Itisimportanttoidentifypatientsinwhomtheproteinuriaisamanifestationofsubstantialglomerulardiseaseasopposedtothosepatientswhohavebenigntransientorpostural(orthostatic)proteinuria.Heavyproteinuria(albuminuria)Figure3.Zandi-NejadKetal.KidneyInt.Suppl2004;92:
S76-S89图1、导致蛋白尿肾病的小管间质炎症及肾硬化的某些事件图2、肾脏对滤过白蛋白处理(回吸收)的旁路RussoLMetal.KidneyInt.Suppl2004;92:
S67-S68HypoalbuminemianHypoalbuminemiaisinpartaconsequencesofurinaryproteinloss.Itisalsoduetothecatabolismoffilteredalbuminbytheproximaltubuleaswellastoredistributionofalbuminwithinthebody.Thisinpartaccountsfortheinexactrelationshipbetweenurinaryproteinloss,theleveloftheserumalbumin,andothersecondaryconsequencesofheavyalbuminuria.ThesaltandvolumeretentionintheNSmayoccurthroughatleasttwodifferentmajormechanisms.nIntheclassictheory,proteinurialeadstohypoalbuminemia,alowplasmaoncoticpressure,andintravascularvolumedepletion.Subequentunderperfusionofthekidneystimulatestheprimingofsodium-retentivehormonalsystemssuchastheRASaxis,causingincreasedrenalsodiumandvolumeretention,Intheperipheralcapillarieswithnormalhydrostaticpressuresanddecreasedoncoticpressure,theStarlingforcesleadtotranscapillaryfluidleakageandedema.EdemanInsomepatients,however,theintravascularvolumehasbeenmeasuredandfoundtobeincreasedalongwithsuppressionoftheRASaxis.Ananimalmodelofunilateralproteinuriashowsevidenceofprimaryrenalsodiumretentionatadistalnephronsite,perhapsduetoalteredresponsivenesstohormonessuchasatrialnatriureticfactor.Hereonlytheproteinurickidneyretainssodiumandvolumeandatatimewhentheanimalisnotyethypoalbuminemic.Thus,localfactorswithinthekidneymayaccountforthevolumeretentionofthenephroticpatientaswell.EdemaFigure4.HyperlipidemianMostnephroticpatientshaveelevatedlevelsoftotalandlow-densitylipoprotein(LDL)cholesterolwithlowornormalhigh-densitylipoprotein(HDL)cholesterol.Lipoprotein(a)Lp(a)levelsareelevatedaswellandreturntonormalwithremissionofthenephroticsyndrome.Nephroticpatientsoftenhaveahypercoagulablestateandarepredisposedtodeepveinthrombophlebitis,pulmonaryemboli,andrenalveinthrombosis.CauseTable2CAUSESOFTHENEPHROTICSYNDROMETable3aNEPHROTICSYNDROMEASSOCIATEDWITHSPECIFICCAUSES(“SECONDARY”NEPHROTICSYNDROME)Table3bNEPHROTICSYNDROMEASSOCIATEDWITHSPECIFICCAUSES(“SECONDARY”NEPHROTICSYNDROME)PathologypatternsandclinicalpresentationsofidiopathicnephroticsyndomenInadults,thenephroticsyndromeisacommonconditionleadingtorenalbiopsy.Inmanystudies,patientswithheavyproteinuriaandthenephroticsyndromeshavebeenagrouphighlylikelytobenefitfromrenalbiopsyintermsofachangeinspecificdiagnosis,prognosis,andtherapy.nSelectedadultnephroticpatientssuchastheelderlyhaveaslightlydifferentspectrumofdisease,butagaintherenalbiopsyisthebestguidetotreatmentandprognosis(Table2,3).RenalbiopsyPRIMARYNEPHROTICSYNDROMEnMinimalChangeDiseasenFocalSegmentalGlomerulosclerosisnMembranousNephropathynMembranoproliferativeGlomerulonephritis(MPGN)Figure5a.Pathologyofglomerulardisease.Lightmicroscopy.(a)Normalglomerulus;minimalchangedisease.Table4PRIMARYNEPHROTICSYNDROMEnMinimalChangeDiseasenFocalSegmentalGlomerulosclerosisnMembranousNephropathynMembranoproliferativeGlomerulonephritis(MPGN)Figure5b.Segmentalsclerosis;focalsegmentalglomerulosclerosis.Figure6.Lightmicroscopicappearancesinfocalsegmentalglomerulosclerosis.Segmentalscarswithcapsularadhesionsinotherwisenormalglomeruli.Table5PRIMARYNEPHRO
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