儿科学课件-急性肾小球肾炎ppt.ppt

火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去ACUTE GLOMERULONEPHRITIS急性肾小球肾炎急性肾小球肾炎火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去要求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 火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Anatomy and physiology characteristics of urologic system解剖特点Anatomy characteristics 生理特点Physiology characteristics 肾功能的临床评估Estimate of renal function火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Physiology characteristics 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。火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Estimate of renal function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BUNSCrCCr胱抑素C肾小球滤过功能肾小管功能内分泌功能火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去肾小球疾病的临床分类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肾小球疾病遗传性进行性肾炎遗传性进行性肾炎 家族性血尿家族性血尿其他其他火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去General Information Acute glomerulonephritise Acute glomerulonephritise acute postinfectious acute postinfectious other secondary/primary other secondary/primary glomerulonephritis glomerulonephritis glomerulonephritisglomerulonephritis acute poststreptococcal acute poststreptococcal glomerulonephritis glomerulonephritis Non-poststreptococcalNon-poststreptococcalglomerulonephritisglomerulonephritisA group of glomerular diseases by different causesA group of glomerular diseases by different causes火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去病历摘要病历摘要患儿，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3.81012/L,网织红1.4%，白细胞11.3109/L,分叶82%，淋巴16%，单核2%，血小板207109 /L；尿常规：尿蛋白（+），红细胞30-40个/HP，比重1.010。火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去病例特点学龄儿童，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火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去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？火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Clinical manifestationIncubation:1-3 weeks after infection.r r r r：火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Clinical manifestation1.Typical cases(1)Edema and oliguria:mild degree edema only in eyelids in the morningsevere degree all over the body,edema is non pitting.urinary output decreased oliguria 250ml/m2 anuria 15%，提示肾小球源性血尿 严重变形红细胞包括：穿孔、环状、芽孢穿孔、环状、芽孢火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去肾小球性与非肾性血尿的鉴别肾小球性与非肾性血尿的鉴别 尿红细胞形态尿红细胞形态 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（带泡（1)1)细胞细胞肾小球性血尿的良好指标肾小球性血尿的良好指标 不受膀胱尿渗透压的影响不受膀胱尿渗透压的影响,形态相对固定形态相对固定,火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去肾小球性与非肾性血尿的鉴别肾小球性与非肾性血尿的鉴别尿流式细胞学检查尿流式细胞学检查检测原理：检测原理：-尿中有形成分荧光染色，通过计量细胞荧光强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定量检测尿中各类有形成分,并能对红细胞形态、并能对红细胞形态、大小进行分析大小进行分析 结果判定：非均一性小红细胞结果判定：非均一性小红细胞肾小球性肾小球性 均一性红细胞均一性红细胞非肾小球非肾小球火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去A kidney biopsy in a 34-year-old man with IgA nephropathy showed mesangial-cell proliferation and predominant A kidney biopsy in a 34-year-old man with IgA nephropathy showed mesangial-cell proliferation and predominant mesangial IgA deposition.At the time of the biopsy,the patients serum creatinine and blood urea nitrogen levels mesangial IgA deposition.At the time of the biopsy,the patients serum creatinine and blood urea nitrogen levels were 1.3 mg per deciliter(110 were 1.3 mg per deciliter(110 mmmol per liter)and 13 mg per deciliter(4.6 mmol per liter),respectively.mol per liter)and 13 mg per deciliter(4.6 mmol per liter),respectively.Microscopical examination of the urine revealed many dysmorphic urinary erythrocytes with spheroid surface Microscopical examination of the urine revealed many dysmorphic urinary erythrocytes with spheroid surface protrusions(Panel A,1450)indicative of glomerular hematuria.protrusions(Panel A,1450)indicative of glomerular hematuria.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去A glomerular erythrocyte ghost(Panel B,3250)has a small neck at the base of each surface protrusion,A glomerular erythrocyte ghost(Panel B,3250)has a small neck at the base of each surface protrusion,demonstrating its connection with the cytoplasm of the cell.Urinary erythrocytes with surface protrusions such as demonstrating its connection with the cytoplasm of the cell.Urinary erythrocytes with surface protrusions such as those shown here are present in large numbers only with pathologic glomerular bleeding.(Differential interference those shown here are present in large numbers only with pathologic glomerular bleeding.(Differential interference contrast microscopy,1000.)contrast microscopy,1000.)火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Clinical manifestation (3)Hypertension:cases have varying degrees of hypertension(120-150/80-110mmHg).the hypertension is attributed to expanded vascular volume or vasospasm.BP（mmHg）Preschool age120/80School age 130/90火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Frequency of clinical Manifestations in APSGNGross hematuria 50 70%Volume overload Edema 70%Hypertension 30-80%Circulatory congestion 20%CNS symptoms 10%Progressive GN 3/HP,malformation RBC60%.(2)Proteinuria:1+3+.(3)The urinary sediment:WBC and epithelial cells,hyaline,granular and red blood cell casts火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Laboratory findings2.Blood test(1)a mild anemia(3)ESR:the erythrocyte sedimentation rate(4)Hypocomplementemia:C3,CH50.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Laboratory findings2.Boold test(5)ASO:Antistreptolycin 0 titer elevated from 1 to 3 weeks after infection,the highest level from 3 to 5 weeks.Anti-DNAse B and anti hyaluronidase are elevated in patients with polyderma.(6)Renal function:SCr,BUNand GFR due to severe oliguria.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Pathology In gross observation the kidneys appear Symmetrically enlarged.By light microscopy -all glomeruli appear enlarged and relatively bloodless -show cells proliferation,such as epithelial cells,endothelial cells,mesangial cells.-polymorphonuclear leukocytes invade in glomeruli during the early stage of the disease,and increase in mesangialmatrix.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Pathology By immunofluorescent microscopy show lumpy-bumpy granular staining for IgG in peripheral capillary loops and mesangial areas By electron microscopy electron-dense deposits or humps are observed on the epithelial side of the glomerular bacementmembrane(GBM)火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去毛细血管内增生性肾小球肾炎，毛细血管内增生性肾小球肾炎，IgG IgG 粗大颗粒沿毛细血管粗大颗粒沿毛细血管 壁沉积壁沉积（荧光，（荧光，400 400 400 400）In AGN,immunofluorescent studies show In AGN,immunofluorescent studies show lumpy-bumpylumpy-bumpy granular staining for IgG in peripheral capillary loops granular staining for IgG in peripheral capillary loops and in mesangial areas and in mesangial areas火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Diagnosis3 steps1stClinical manifestation abruptly with hematuriaProteinuriaHypertensionazotemia asymptomatic oliguric acute renal failure latent period Pharyngitisskin infectionnephritis 2nd3rd evidence of recent group A streptococcal infection a positive skin or throat culture ASO(+)、Anti-SpeB火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去DiagnosisSpecial importantLow C3（90%）Returns to normal in 8 weeks in all patients with uncomplicated PSGNIs helpful for diagnosis subclinical APSGN（mild abnormalities of urine，transient drop in complement）火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Indications for renal biopsyMassive proteinuria Complement is persistently reduced after 2-3monthsAcute nephrosis syndrome with normal serum complementSerum creatinine increases progressively火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Differential diagnosis1.Other primary GN are frequently confused.IgA nephropathy Membrane proliferative disease 2.Secondary GN atypical cases resulted from systemic disease such purpura nephroitis,systemic lupus nephritis HBV associated GN 火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Differential diagnosis3.Chronic nephritis also may easily be mistaken for acute disease.4.RPGN Rapidly Progressive glomerulonephritis 5.Primary NS 火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去SummaryofPrimaryRenalDiseasesThatManifestasAcuteGlomerulonephritisDISEASESPOSTSTREPTOCOCCAL GLOMERULONEPHRITISIGA NEPHROPATHYGOODPASTURE SYNDROMEIDIOPATHIC RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS(RPGN)ClinicalmanifestationsAgeandsexAllages,mean7yr,2:1male1035yr,2:1male1530yr,6:1maleAdults,2:1maleAcutenephriticsyndrome90%50%90%90%AsymptomatichematuriaOccasionally50%RareRareNephroticsyndrome1020%RareRare1020%Hypertension70%3050%Rare25%Acuterenalfailure50%(transient)Veryrare50%60%OtherLatentperiodof13wkFollowsviralsyndromesPulmonaryhemorrhage;irondeficiencyanemiaNoneLaboratoryfindingsASOtiters(70%)SerumIgA(50%)Positiveanti-GBMantibodyPositiveANCAinsomePositivestreptozyme(95%)IgAindermalcapillariesC3C9;normalC1,C4火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去ImmunogeneticsHLA-B12,D“EN”(9)*HLA-Bw35,DR4(4)*HLA-DR2(16)*NoneestablishedRenalpathologyLightmicroscopyDiffuseproliferationFocalproliferationFocaldiffuseproliferationwithcrescentsCrescenticGNImmunofluorescenceGranularIgG,C3DiffusemesangialIgALinearIgG,C3NoimmunedepositsElectronmicroscopySubepithelialhumpsMesangialdepositsNodepositsNodepositsPrognosis95%resolvespontaneouslySlowprogressionin2550%75%stabilizeorimproveiftreatedearly75%stabilizeorimproveiftreatedearly5%RPGNorslowlyprogressiveTreatmentSupportiveUncertain(optionsincludesteroids,fishoil,andACEinhibitors)Plasmaexchange,steroids,cyclophosphamideSteroidpulsetherapyACE,angiotensin-convertingenzyme;ANCA,antineutrophilcytoplasmicantibody;ASO,anti-streptolysinO;GBM,glomerularbasementmembrane;GN,glomerulonephritis;HLA,humanleukocyteantigen;Ig,immunoglobulin.FromKliegmanRM,GreenbaumLA,LyePS:Practical Strategies in Pediatric Diagnosis and Therapy,2nded.Philadelphia,Elsevier,2004,p427.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Treatment 1.Nursing and diet Bed rest is indicated as long as there are clinical manifestations of active disease,such as edema,hypertension,or gross hematuria Protein restricted(intake 0.5g/kg.d)Salt restricted(60mg/kg.day)ESR normal go to school火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Treatment2 Antibiotics Penicillin is used for 10 to 14 days.3 Diuretic therapy Salt restriction,HCT 23mg/kgd PO,Furosemide 25mg/kg.d PO or l2mg/kgdose iv4 Antihypertensive therapy:Reserpine:O.07mg/kg.im Nifedipine:O.25 to 0.5mg/kg.d P.O.Captopril:for treating severe hypertension (O.3mg/kgd Tid Po).火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Treatment5.Treatment of severe complications Hypertensive encephalopathy:Sodium nitroprusside(l8g/kg)introvenously drop continually,regulating the dropping speed according to the level of BP.Anticonvulsantwater deprivation with Mannitoloxygen therapy火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Treatment5.Treatment of severe complicationsSevere Circulatory Congestion:Restriction of salt and water,diuretics.Sodium nitroprussideAcute renal failure:Maintaining balance of water,electrolytes and acid base,applying energy and preventing complications.Peritoneal and hemodialysis may be needed.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Course and prognosis2 weeks edema gross hematuriaHypertensionAbnormal urine usually lasts 1 years or longer.The prognosis of AGN is very good.火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去PrognosisThe early mortality is very low in childrenCardiovascular complications are the main causeIrreversible renal failure may fellow acute nephrisis with crescentic proliferation10-20 yrs later20%-abnormal urine analysis or creatinine clearence1%-azotaemia8-13%-proteinuria and hypertensionThe incidence of glomerular sclerosis and fibrosis is nearly 50%,but their relevance is uncertain火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去与急性链球菌感染后肾炎预后有关的因素与急性链球菌感染后肾炎预后有关的因素有关因素有关因素无关因素无关因素1发病（流行发病较散发者好）发病（流行发病较散发者好）1前驱感染史及严重程度前驱感染史及严重程度2年龄（儿童预后好，老年差）年龄（儿童预后好，老年差）2肉眼血尿的严重性肉眼血尿的严重性3严重而持续的高血压、肾病综严重而持续的高血压、肾病综3血补体下降程度血补体下降程度合征及肾功能损害（预后差）合征及肾功能损害（预后差）4光镜下广泛大新月体；电镜下光镜下广泛大新月体；电镜下4血血ASO滴度上升程度滴度上升程度不典型驼峰；荧光呈花环状改不典型驼峰；荧光呈花环状改变（预后差）变（预后差）火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去MicrohaematuriaIntermittent ProteinuriaGross HHypertentionLow C3Persistent Proteinuria2 weeks 4 weeks 2 months 6 months 1 years 2 yearsClinical and Laboratory featuresTime course to revolution of APSGN火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去SummeryASO+C3实验室检查临床表现诊断治疗火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去ReferenceBrenner and Rectors The Kidney Nelson Textbook of Pediatrics -18EdOxford Textbook of Clinical Nephrology(3rd Ed)火灾袭来时要迅速疏散逃生，不可蜂拥而出或留恋财物，要当机立断，披上浸湿的衣服或裹上湿毛毯、湿被褥勇敢地冲出去Thank You For Your Attention