天津醫(yī)科大學(xué)授課教案（共頁(yè)、第頁(yè)）課程名稱：內(nèi)科學(xué)課程內(nèi)容：急慢性腎小球腎炎教師姓名：韓鴻玲職稱：主任醫(yī)師授課日期：2013年2月28日10時(shí)授課對(duì)象：醫(yī)療系年級(jí)留學(xué)生本科教材版本：留學(xué)生教材授課方式：大課學(xué)時(shí)數(shù)：2聽課人數(shù)：80本單元或章節(jié)的教學(xué)目的與要求：1.掌握腎小球疾病的分類2.掌握急性腎炎、慢性腎炎、急進(jìn)腎炎的概念3。掌握急慢性腎炎的臨床表現(xiàn)和診斷方法治療原則4.了解急慢性腎炎的病理改變和發(fā)病機(jī)理授課主要內(nèi)容及學(xué)時(shí)分配：腎小球疾病的分類和急性腎小球腎炎1學(xué)時(shí)急進(jìn)性腎炎慢性腎炎1學(xué)時(shí)重點(diǎn)、難點(diǎn)及對(duì)學(xué)生要求（包括掌握、熟悉、了解、自學(xué)）重點(diǎn)：1.腎小球疾病的分類依據(jù)和臨床的應(yīng)用2.各種腎炎的臨床特點(diǎn)，難點(diǎn)：理解各種腎炎的發(fā)病機(jī)制不同，盡管臨床表現(xiàn)類似，在臨床中只能稱為綜合征掌握：各種腎小球綜合征的臨床表現(xiàn)特點(diǎn)和診斷依據(jù)及治療原則了解：各種腎小球炎癥的病理改變，但要相對(duì)重點(diǎn)了解RPGN的病理改變外語(yǔ)詞匯：輔助教學(xué)情況：幻燈片加上生動(dòng)的實(shí)際病例復(fù)習(xí)思考題：ClinicalfeaturesofPSGNWhatisRPGN？ClinicalfeaturesofIgAnephropathy參考資料：留學(xué)生教材腎臟病學(xué)主任簽字：教務(wù)處制天津醫(yī)科大學(xué)授課教案（共5頁(yè)、第1頁(yè)） AGNGrossormicroscopichematuriaismostcommon,andisoftendescribedbythepatientsassmoky-coffee-orcola-coloredurine.Theerythrocytesintheurinarysedimentaresmall,distored,fragmentedandhypochromicwhichiscalleddysmorphichematuria.Ingeneral,grosshematuriamaylastafewdaysto1or2weeksandthendisappear.B.ProteinuriaThedegreeofproteinuriavariesaccordingtothenatureandseverityoftheunderlyingglomerularlesion.Rarely,proteinexcretionratesarewithinthenormalrange,butgenerallytheyarebetween0.2and3g/dandnonselective.Ifproteinuriaismarkedandsustained,theNSmayappear.C.EdemaTheedemaappearsinareasoflowtissuepressure,suchasperiorbitalareas,especiallyinthemorning.Thisiscallednephriticfaces.Severeedemamayprogresstodependentportionofthebodyandleadtoascitesand/orpleuraleffusions.D．HypertensionAlmost80%ofcaseshaveamildtomoderatedegreeofhypertension,especiallyinoldpatients.E.OliguriaOliguriamaybepresentwhennephritisoccurs.Usuallylessthan500ml/d,whichleadstoazotemia.Twoweekslatertheamountofurinemaygraduallyincrease.F.RenalfunctionlesionGlomerularinflammationcanleadtoreducedglomerularfiltrationthatcouldleadtoazotemia.Usuallyafteradiuretic,azotemiamaygraduallydisappear,ifnot,acuterenalfailureoccurs.G.OthersOthersymptomsofPSGNmaybevomiting,nausea,sleeping,loinpainetc.ComplicationsThecomplicationsofPSGNareheartfailure,encephalopathyanduremia.LaboratoryfindingsRBCsandRBCcasts,leukocytecasts,WBCs,FDP,Cз,non-selectiveproteinuria(non-nephroticrange)canbedetectedinurine.Inmostchildrenandadults,proteinuriawillbecomenegativeafter4to6monthsofonsetofnephritis.Otherlaboratoryfeaturesincludepositivetestsforcirculatingimmunecomplexes,anelevatedantistreptolysisOtiter,alowserumcomplement(usuallyreturningtonormalat6to12weeks),azotemia,elevatederythrocytesedimentationrate(ESR)andmildanemia.DiagnosisanddifferentialdiagnosisThediagnosisofPSGNcanbebasedonthetypicalrenalpresentationfollowingstreptococcalinfection,hypocomplementemia,andserologicevidence.ThedifferentialdiagnosisisthatofAGNwithhypocomplementemiaandincludesotherformsofpostinfectiousGN,e.g.bacterialendocarditis,shuntnephritis,systemiclupuserythematosus(SLE),andmembranoproliferativeGN.Becausethediagnosisismostoftenstraightforward,arenalbiopsyisindicatedonlyofthediseasefollowsanatypicalcourseinchildren.Mostadultswithacutenephriticsyndromerequireakidneybiopsytoestablishthediagnosis.CourseandtreatmentCompleterecoveryoccursinatleast85to90%ofallpatients.However,minorurinarysedimentabnormalitiesmaycontinueforseveralyearsinsomepatients(<2%),butprogressiontochronicrenalfailureisrare,typicallyoccurringonlyinolderadults.Fewerthan5%ofpatientshaveoliguriaformorethan7to9days,andtheprognosisinthesepatientsislessfavorable.ThereisnospecifictherapyforPSGN.Thetreatmentissupportiveandsymptomaticuntilallacutesignshaveabated.It’sreasonabletorecommendedbedrestuntilthesignsofglomerularinflammationsubside.Mildproteinrestrictionisdesirableforazotemicpatients.Sevento10daysofpenicillinorothersuitableantimicrobialsshouldbegivenwithevidenceofstreptococcalinfection.Saltrestrictionand,insomecases,diureticsandantihypertensiveagentsmayberequiredtomanagesodiumretention(manifestedbyhypertension,edema,congestiveheartfailure,andothersigns).Steroidsandcytotoxicdrugsarenotofvalue.Rapidlyprogressiveglomerulonephritis(RPGN)RPGNischaracterizedclinicallybytherapiddeteriorationofrenalfunctionthatreachesendstagewithinaperiodofdaysorweeks,andhistologicallybyextensivecrescents.Itcanbeanidiopathicprimaryglomerulardiseaseorcanbesuperimposedonotherglomerulardiseases,eitherprimaryorsecondary.ClassificationandpathologyTheclassificationofRPGNisbasedonimmunofluorescencemicroscopicfindings.Thecategoriesareasfollows(shownintable-2):Table-2TypesofRPGNTypesAnti-GBMantibodyImmune-complexesNon-immune-complexesLightmicroscopyCrescentsNecrosisCrescentsProliferationCrescentsNecrosisImmunofluorenscenceMicroscopyLinear-IgGFibrinogenGranularIgGcomplementfibrinogenNegativePossiblePathogenesisAnti-GBMImmune-complexesANCAAssociationPulmonaryhemorrhageBacterialinfectionsSystemicsymptomsrash,fever·Glomerulonephritisduetoantibodiesdirectedtowardglomerularbasementmembraneantigens(anti-GBM).Itaccountsfor20%ofallcasesofRPGN.?Glomerulonephritisduetothedepositionorformationofimmunecomplexesintheglomeruli.Itaccountsfor40%ofallcasesofRPGN.?Glomerulonephritisinwhichnoimmunoglobulinsarefoundintheglomeruli(so-callednonimmune).Itaccountsfor40%ofcasesofRPGN.Bylightmicroscopy,extracapillaryproliferation(i.e.crescents)canbedetectedwhichisafeatureofRPGN.Usuallymorethan70%ofglomeruliareinvolvedwithcrescents(socalledcrescenticglomerulonephritisFigure-4).Endocapillaryproliferation,ifprominent,suggeststhepresenceofinfection.Segmentalordiffuseendocapillarynecrosissuggestsunderlyingsystemicnecrotizingvasculitis.NonstreptococcalacutepostinfectiousglomerulonephritisNonstreptococcalacutepostinfectiousglomerulonephritisincludesawidevarietybacterialstatesandvariousviralandparasiticdiseasese.g.infectiveendocarditis,sepsisofothertypes,visceralabscess,typhoidfever,infectiousmononucleosis,acuteviralhepatitisB,falciparummalaria,andtoxoplasmosisetc.CirculatingimmunecomplexesplayanimportantroleinthepathogenesisofAGNinthesediseases.Theclinicalandhistologicmanifestationmayvarysomewhat,still,mosthavefeaturessimilartothePSGN.Iftheunderlyinginfectioniseradicated,theprognosisisgood.Systemiclupuserythematous,Henoch-Sch?nleinpurpura,andmixedessentialcryoglobulinemiamaypresentasanacuteGN,buttheyareusuallyassociatedwithotherglomerularsyndromes.AsymptomaticurinaryabnormalitiesThisgroupofpatientshasproteinuriainthenonnephroticrangeand/orhematuria,unaccompaniedbyedema,reducedGFR,andhypertension.Abnormalitiesareoftendiscoveredincidentallyandmaybepersistentorrecurrent.Insome,thissyndromeisaphaseinthenaturalhistoryofotherglomerulopathicsyndromes,especiallyNSorchronicglomerulonephritis.AsymptomatichematuriaAvarietyofrenallesionmaypresentasasymptomatichematuria.IgAnephropathyIgAnephropathyorBerger’sdiseaseisthemostcommoncauseofrecurrenthematuriaofglomerularorigin.Itaccountsfor50%ofcaseswithasymptomatichematuriaand26to34%inprimaryglomerulopathy.Lightmicroscopicchangesarevariable,butdiffusemesangialproliferativeglomerulonephritisorfocalandsegmentalproliferativeglomerulonephritisisfoundmostoften.Insomecases,glomerularmorphologymaybenormal;uncommonly,crescentsmaybefound.ThediagnosisdependsonthefindingofprominentIgAdepositsinthemesangiumbyimmunofluorescencemicroscopy.Thetypicalpresentationisgrosshematuriafollowingaviralillnessorvigorousexercise,withmenaffectedtwotothreetimesmorefrequentlythanwomen.Mostotherpatientspresentwithasymptomatichematuriadiscoveredonanincidentalexamination,accompaniedbymildtomoderateproteinuria.Mostpatientsarebetweentheagesof15and35.Microscopichematuriausuallyremainsaftergrosshematuriaresolves.Mildproteinuriaoflessthan1g/discommon,buttheNSdevelopsoccasionally.Serumcomplementisnormal.SerumIgAlevelsareincreasedinabout50%ofcases.Atpresent,thereisnoevidencethattherapywillinfluencethenaturalhistory,althoughintermittentsteroidtherapymayreducethefrequencyofepisodesofgrossofhematuria.SteroidsmayalsoresultinremissionsofproteinuriainthosepatientswithNS.Theprognosisisvariable,butthediseasetendstoprogressslowly.Approximately50%ofpatientsdevelopendstagerenalfailurewithin25yearsofthetimeifdiagnosis.Poorprognosticindicatorsincludenephroticrangeproteinuria,hypertension,andazotemia.IgAnephropathyrecursinthetransplantedkidneyinapproximately30to40%ofcases,butwithminimallong-termeffectsonrenalfunction.Chronicglomerulonephritis(CGN