儿科学课件-急性肾小球肾炎

要求掌握急性肾小球肾炎典型表现、重症表现及非典型表现掌握急性肾小球肾炎的诊断掌握急性肾小球肾炎的治疗原则Anatomyandphysiologycharacteristicsofurologicsystem解剖特点Anatomycharacteristics生理特点Physiologycharacteristics肾功能的临床评估EstimateofrenalfunctionPhysiologycharacteristics出生后一段时期内呈动态成熟过程：贮备能力差，调节机制不成熟，肾功能1-1岁半接近成人。Estimateofrenalfunction肾功能检查检查结果的临床意义应考虑年龄、身长、体重等因素BUNSCrCCr胱抑素C肾小球滤过功能肾小管功能内分泌功能肾小球疾病的临床分类原发性肾小球疾病肾小球肾炎急性肾小球肾炎急进性肾炎迁延性肾炎慢性肾炎肾病综合征单纯性肾病肾炎性肾病无症状性血尿或蛋白尿继发性肾小球疾病紫癜性肾炎狼疮性肾炎乙型肝炎病毒相关肾炎药物中毒其他遗传性肾小球疾病•遗传性进行性肾炎•家族性血尿•其他GeneralInformationAcuteglomerulonephritiseacutepostinfectiousothersecondary/primaryglomerulonephritisglomerulonephritisacutepoststreptococcalglomerulonephritisNon-poststreptococcalglomerulonephritisAgroupofglomerulardiseasesbydifferentcauses病历摘要患儿，女，8岁，因“双眼睑浮肿、血尿6天”入院。6天前出现双眼睑浮肿，排全程茶色尿，伴尿频、尿急，尿中无血块，尿量无减少。当地医院予抗生素治疗（具体不详）。4天前，患儿浮肿加重，渐波及全身，尿量每日130-150ml，血压130/90mmHg，予利尿、降压治疗，患儿病情无好转，转来我院。病前2周曾出现发热，并伴有咳嗽。过去史、家族史、生长发育及喂养史、预防接种史无特殊。查体：血压140/95mmHg，双眼睑、颜面浮肿，睑结膜苍白，双肺音粗，可闻及细湿罗音。心率120次/分，律齐，心音有力，未闻杂音。腹软，肾区叩击痛（+），双下肢非可凹性水肿。实验室及影像学检查：血常规：血色素93g/L，红细胞3.8×1012/L,网织红1.4%，白细胞11.3×109/L,分叶82%，淋巴16%，单核2%，血小板207×109/L；尿常规：尿蛋白（++），红细胞30-40个/HP，比重1.010。病例特点学龄儿童，起病急，病程短；表现为：血尿、尿量减少、浮肿、血压增高；实验室检查提示：轻度贫血；尿蛋白（++），红细胞30-40个/HP起病前2周有前驱感染病史心率120次/分，双肺可闻及细湿罗音该患儿的初步诊断是什么？诊断依据是什么？还需补充哪些症状、体征及实验室检查？ClinicalmanifestationIncubation:1-3weeksafterinfection.６－２１ｄａｙＷｉｎｔｅｒ３ｗｅｅｋｓＳｕｍｍｅｒ５－１０ｙｅａrｓＭａｌｅ／ｆｅｍａｌｅ２：１Clinicalmanifestation1.Typicalcases(1)Edemaandoliguria:milddegreeedemaonlyineyelidsinthemorningseveredegreealloverthebody,edemaisnonpitting.urinaryoutputdecreasedoliguria250ml/m2anuria30~5Oml/dClinicalmanifestation(2)Hematuria:almostallpatientshavemicroscopicalhematuria,andabout50-70%caseshavegrosshematuria.尿红细胞形态检查尿红细胞形态鉴别肾小球源性或非肾小球源性血尿严重变形RBC15%，提示肾小球源性血尿严重变形红细胞包括：穿孔、环状、芽孢肾小球性与非肾性血尿的鉴别——尿红细胞形态严重变形非严重变形环形粗刺穿孔细刺带泡大淡染残骸带泡（Ｇ1)细胞——肾小球性血尿的良好指标不受膀胱尿渗透压的影响,形态相对固定,肾小球性与非肾性血尿的鉴别——尿流式细胞学检查检测原理：-尿中有形成分荧光染色，通过计量细胞荧光强度、前向散射光强度和细胞电阻,-定量检测尿中各类有形成分,并能对红细胞形态、大小进行分析结果判定：非均一性小红细胞——肾小球性均一性红细胞——非肾小球Akidneybiopsyina34-year-oldmanwithIgAnephropathyshowedmesangial-cellproliferationandpredominantmesangialIgAdeposition.Atthetimeofthebiopsy,thepatient’sserumcreatinineandbloodureanitrogenlevelswere1.3mgperdeciliter(110mmolperliter)and13mgperdeciliter(4.6mmolperliter),respectively.Microscopicalexaminationoftheurinerevealedmanydysmorphicurinaryerythrocyteswithspheroidsurfaceprotrusions(PanelA,1450)indicativeofglomerularhematuria.Aglomerularerythrocyteghost(PanelB,3250)hasasmallneckatthebaseofeachsurfaceprotrusion,demonstratingitsconnectionwiththecytoplasmofthecell.Urinaryerythrocyteswithsurfaceprotrusionssuchasthoseshownherearepresentinlargenumbersonlywithpathologicglomerularbleeding.(Differentialinterferencecontrastmicroscopy,1000.)Clinicalmanifestation(3)Hypertension:caseshavevaryingdegreesofhypertension(120-150/80-110mmHg).thehypertensionisattributedtoexpandedvascularvolumeorvasospasm.BP（mmHg）Preschoolage120/80Schoolage130/90FrequencyofclinicalManifestationsinAPSGNGrosshematuria50–70%VolumeoverloadEdema70%Hypertension30-80%Circulatorycongestion20%CNSsymptoms10%ProgressiveGN1%EtiologycasesofGNfollowinfectionwithgroupAβhemolyticstreptococci,suchasupperrespiratorytractinfection,Scarletfever,skininfection.PathogenesisItisnowgenerallyacceptedthatglomerulonephritisisaformofimmune-complexdisease.Theantigeniccomponentoftheimmunecomplexberelatedsomehowtothestreptococcus.SomestrainsofAgrouphemolyticstreptococcusURIskininfectionantibodyproductioncirculatinginsiteantigen-antibodycomplexesimmunecomplexesdepositintheGBMactivatethecomplementsystemincitemediaofinflammationPathogenesisAutoimmunereactionimmuneinjuries(Ag-Ab→IC→C3)CellsproliferationmediaofinflammationcapillarylumennarrowedhematuriaprotineruriaGBMinjuryGlomerularbloodflow↓azotemiaGFR↓oliguria/anuriaRetentionofwaterandnatrium→edemahypertensionbloodvolume↑hypertensiveencephalopathcirculatorycongestionPathogenesis病例特点起病急，病程短；表现为：血尿、尿量减少、浮肿、血压增高；实验室检查提示：轻度贫血；尿蛋白（++），红细胞30-40个/HP起病前2周有前驱感染病史心率120次/分，双肺可闻及细湿罗音Clinicalmanifestation2.Severecases(1)Circulatorycongestionthecauseisattributabletoanincreaseinbloodvolumesecondarytoretentionofsodiumandwater.Itisnotarealcardiacfailureintheearlystageofthedisease,butcirculatorycongestioncandeveloptorealheartfailure.ManifestationsofCirculatorycongestionparoxysmalorpersistentdyspnea,orthopnea,moistralesbeingaudibleinthebasesofthelungs,heartenlargement,HR↑,gallopingrhythm.liverenlargedClinicalmanifestation2.Severecases(2)HypertensiveencephalopathyThecerebralsymptomsarecausedbycerebraledema,vasospasm,cerebralanoxiaHeadache,vomiting,irritability/apathy,convulsion,eventemporarycompleteblindness..2.Severecases(3)Acuterenalfailure:Oliguriaevenanuria,azotemia,hyperkalemiametabolicacidosisClinicalmanifestationClinicalmanifestation3.Atypicalcases(1)AcutenephritiswithminimalurinaryfindingsThepatientmayhavegeneralizededema,hypertension,evencirculatorycongestion,butnourinaryabnormalitiesorminorabnormalities.MeasurementofC3andneedlebiopsyisneededfordiagnosis.Clinicalm