过敏性紫癜的诊断和治疗.ppt

泌尿系统疾病,苏州大学儿科学系肾内科,WorldKidneyDay,March9th2019wasthefirstWKDandthenthesecondThursdayonmarchwillbeanniversaryforkidneydiseases.About10%populationinvolvedChronicKidneydisease(CKD)inUSAAbout100millionCKDinchinaofthetotalmedicalcost,Introduction:background,Effectivemanagementofthepatientwithrenaldiseaseisdependentuponestablishinganaccuratediagnosis.TheclinicianmustbeawareofthepossiblepresentationsofrenaldiseasesandgatherthesesymptomsandsignswhichformrecognizeddiseasesandsyndromeWhatisthepossiblepresentationssuggestingrenalproblem?,Clinicalpresentationofrenaldisease,Thepatientisasymptomatic,butanabnormalityhasbeendetectedonclinicalorlaboratoryexaminationwhichindicatesanunderlyingrenaldisorder.ThepatientcomplainsofasymptomorhasaphysicalsignwhichdirectlyorindirectlyindicatesunderlyingrenaldiseasesThepatienthasasystemicdiseasewhichisknowntobeassociatedwithrenalinvolvementThepatienthasafamilyhistoryofaninheritedrenaldisorder.,Symptomsofurinarytractdisease,Afever;dysuria;frequency;loinpain/abdominalpain;urinaryinconsistency;offensivesmelling;cloudyurine;Frothyurine:Proteinuria;Smokeyurine:Haematuria,Signsofurinarytractdisease,Dysmorphicsyndromes;Anaemia;Oedema;Highbloodpressure;Renalmasses;Distendedbladder.,Acuteglomerulonephritis(AGN),急性肾小球肾炎,Acuteglomerulonephritis(AGN),概述病因发病机制病理生理病理临床表现实验室检查诊断鉴别诊断治疗预后,急性肾小球肾炎,目的要求,了解急性肾炎的病因及发病机理。掌握一般病例与严重病例的临床表现及治疗原则。掌握急性肾炎的实验室检查。了解急性肾炎与其他病原体引起急性肾炎、慢性肾炎急性发作、特发性肾病综合症、IgA肾病综合症、急进性肾炎等疾病的鉴别诊断。,重点和难点,重点：本病的一般病例及严重病例的临床表现及其产生机理。该病的尿液及血生化检查。小儿单纯性血尿的诊断要点和处理原则。难点：本症重症病例的治疗，高血压脑病急性肾功能不全，严重循环充血治疗及处理原则。,Acutepoststreptococcalglomerulonephritis急性链球菌感染后肾炎Hematuria血尿Oliguria尿少Edema浮肿,关键词：Keywords,概述,定义：急性起病，双侧性，弥漫性，前趋感染（非化脓性炎症），临床上以血尿为主，可有水肿、少尿、高血压，或肾功能不全等特点发病情况：年龄5-14岁、性别男女2：1、发生率下降,病因,细菌：A组溶血性链球菌的某些致肾炎菌株；其他细菌有。病毒：巨细胞、乙肝病毒等其他：支原体、霉菌、原虫等,发病机制病理生理,链球菌致肾炎菌株的抗原成分循环免疫复合物原位免疫复合物自身免疫激活补体系统释放生物活性因子肾素血管紧张素醛固酮肾小球基底膜断裂GRF水钠储留血尿、蛋白尿、管型尿水肿、少尿、高血压肾衰,病理,毛细血管内增生性肾小球肾炎光镜：内皮细胞、系膜细胞增生、肿胀，系膜基质增多，中性粒细胞浸润，严重时可有“新月体”形成电镜：上皮细胞下“驼峰样”电子致密物沉积免疫荧光：IgG、C3沉积,AA：入球小动脉EA：出球小动脉EGM：肾小球外系膜PE：壁层上皮细胞PO：足突细胞GBM：肾小球基底膜F：足突US：尿液腔M：系膜PT：近曲小管MD：致密斑G：颗粒细胞N：交感神经E：内皮细胞,PO：足突细胞podocyteGBM：肾小球基底膜glomerularbasementmembraneM：系膜mesangialcellMM：系膜基质mesangialmatrixE：内皮细胞fenestratedendothelium,新月体肾炎,临床表现（一）,前驱感染(Precedeinfection)秋冬季呼吸道感染为主夏秋季皮肤感染多见,临床表现（二）,典型表现(typicalmanifestation)水肿(部位,性质,时间)尿少（少尿，无尿）血尿（肾小球性）高血压(头晕,头痛),电镜扫描尿红细胞形态,临床表现（三）,严重表现(Seriousmanifestation)循环充血“心衰”征象高血压脑病剧烈头痛恶心呕吐惊厥昏迷急性肾功能不全少尿氮质血症电解质紊乱,呼吸困难,肺底湿罗音心脏扩大,心率增快肝肿大,临床表现（四）,不典型表现(Atypicalmanifestation)无症状病例无临床症状,有尿改变,补体C3下降肾外症状型水肿,高血压等表现明显尿改变轻微肾病综合征型大量蛋白尿肾活检病理改变类似典型病例,实验室检查,尿常规血常规肾功能血沉ASO血补体（C3下降）,诊断,前驱链球菌感染史临床症状：血尿、水肿、高血压等实验室检查：尿检、ASO、C3等,鉴别诊断,非典型病例（大量蛋白尿型）非链球菌感染肾炎（病毒性）IgA肾炎全身性疾病（SLE，APN，乙肝肾）慢肾急发急进性肾炎尿路感染肾病综合症,治疗（一）,一般治疗卧床休息（约2周）：水肿退，血压降，肉眼血尿消失；血沉正常上学；12小时尿沉渣正常恢复体力活动饮食：低盐：60mg/kg。d；低蛋白0。5mg/kg。d抗感染：青霉素对症治疗利尿（速尿，双氢克尿噻）降压（心痛定，ACEI：SQ14225）,治疗（二）,严重病例高血压脑病：止惊，降压，脱水（硝普钠，二氮嗪）严重循环充血：利尿为主（速尿）、扩血管、透析急性肾衰：液体，电解质，酸碱，感染关,预后和预防,95%完全恢复5%尿异常1%死亡预防：,总结,定义病理、生理临床表现诊断治疗,肾病综合征,（nephroticsyndrome),【目的要求】了解该病发病机理及病理生理。掌握原发性肾病综合征的分型（临床，激素治疗效应，病理分型）。掌握该病临床表现，并发症，诊断及治疗。【重点和难点】重点：本病的病理生理，临床表现，诊断治疗，并发症。难点：该病的临床与病理分型。,Nephroticsyndrome肾病综合征Proteinuria蛋白尿Hypoalbuminemia低蛋白血症,关键词：Keywords,（一）定义是由于肾小球滤过膜对血浆蛋白的通透性增高，导致大量血浆白蛋白自尿中丢失而引起的一种临床症侯群。,（二）病因和发病机制,病因不明发病机制：微小病变与T细胞功能紊乱有关，非微小病变与体液免疫与细胞免疫均相关细胞因子（IL-2，6，8）有遗传基础与HLA相关：SSNS：DR7FRNS：DR9裂隙膜分子的变化,（三）病理生理,致病因素分子屏障肾小球滤过膜通透性静电屏障大量蛋白尿高脂血症脂代谢紊乱心血管、小球硬化低蛋白血症IgG感染血浆胶体渗透压抗凝血酶,、高凝，血栓铁结合蛋白VitD3结合蛋白水血容量甲状腺素结合蛋白T3、T4分入(ADH，醛固酮、利钠因子、肾小球滤过率)间质水钠储留水肿,肾小球滤过膜通透性与分子大小的关系,不同的电荷其通透性不同,阳电荷,阴电荷,中性电荷,正常与肾病综合征情况下白蛋白代谢,（四）病理,肾病综合征常见病理改变,足突细胞,足突细胞的超微机构,MCD微小病变,FSGS（局灶性节段性肾小球硬化）,MN膜性肾病,MPGN（膜增生性肾小球肾炎）,（五）临床表现,病前常有感染水肿为主要表现,（六）并发症,感染：呼吸道感染，皮肤感染，腹膜炎，尿路感染低血容量休克：烦躁，四肢湿冷，皮肤花纹，心音低，血压下降电解质紊乱：低钠，低钾，低钙血栓形成：肾静脉血栓（腰痛，肉眼血尿）肾功能衰竭：休克所致的肾前性衰竭多见肾小管功能障碍,（七）实验室检查,尿常规：蛋白定性+24H尿蛋白定量：大于0.05/kg肝肾功能：血清白蛋白6.7mmol/L免疫学检测：ANA，抗-dsDNA抗体肾活检,（八）诊断,四大特征：三高一低（一高一低为主）大量蛋白尿：定性+定量24H尿蛋白大于0.05g/kg低蛋白血症：血浆白蛋白小于30g/L(儿童)高脂血症：胆固醇大于5.7mmol/L(儿童)不同程度的水肿,（九）分型,临床分型病理分型激素分型,临床分型,原发性90%单纯性肾病：三高一低肾炎性肾病：三高一低外，还有至少以下之一血尿：RBC大于10/HP高血压氮质血症：BUN大于10.7mmol/L血补体CH50，C3反复下降继发性：SLE，APN，乙肝肾先天性：较少见,病理分型,微小病变（MCD）局灶性节段性肾小球硬化（FsGs）膜增殖性肾炎（MPGN）系膜增生性肾炎（MsPGN）膜性肾病（MGN）,激素分型,激素敏感：8周内尿蛋白转阴激素部分敏感：8周内水肿退，尿蛋白+激素耐药：8周尿蛋白+（激素依赖，反复与复发，频复发）,（十）治疗,一般治疗：休息和饮食利尿：当水肿严重时，尤其有腹水时双克，速尿，氨苯喋啶低分子右旋糖酐激素治疗抗凝治疗免疫调节治疗中药,激素治疗(一),激素使用阶段诱导缓解：强的松1.5-2mg/kg/d4-8w巩固阶段：间歇用药或清晨顿服激素使用方法短程：强的松2mg/kg/d4w强的松1.5mg/kg/dqod4w共8周,激素治疗(二),激素使用方法中长程：强的松1.5-2mg/kg/d4w4w后蛋白转阴，改强的松2mg/kg,隔日早餐后顿服，继用4周，以后每2-4周减量一次，直致停药，总疗程6-9个月,激素治疗(三),复发和反复的治疗延长强的松使用时间加用免疫抑制剂：CTX，VCR激素耐药的治疗延长强的松诱导期甲基强的松龙冲击加用免疫抑制剂：CTX，环孢霉素A，酶芬酸酯,激素副作用,激素应用前注意事项：感染、胃肠道炎症、水肿、高血压代谢紊乱消化溃疡和精神欣快感白白内障，股骨头坏死高凝状态生长停止易发感染急性肾上腺功能不全,免疫抑制剂,化学制剂烷化剂（CTX）、抗代谢药（VCR、MMF）真菌代谢产物：环孢素A、FK-506中药及其有效成分：雷公藤掌握剂量、疗程注意副作用：骨髓抑制、胃肠道反应、性腺抑制,其它,抗凝：低分子肝素，潘生丁溶栓：尿激酶免疫调节：左旋咪唑控制病情发展：ACEIandARB中药,预后,微小病变好常常死于感染和激素严重副作用非微小病变肾病综合症：,总结,概念：三高一低病因及发病机制：了解病理生理：重要临床表现：重要诊断：分型治疗：激素的应用,谢谢,泌尿道感染,AnaphylactoidPurpura(AP)&APNephritis(APN)ORHenoch-SchonleinPurpura(HSP),XiaozhongLi,Maincontent,Introducedefinition,theetiology,PathophysiologyofHSPEmphasispathologicchangesandclinicalmanifestationsofHSPIntroducethetreatmentofHSP,Description,Definition:Henoch-SchonleinPurpuraSyndrome,HSP:Clinicalfeaturescharacterizedbyskinrash,jointpain,abdomensymptoms,renaldamage,etal.Pathologicchange:SystemicvasculitisCommonseenin2-8ysofchildinspringandautumn,boysmorethangirlsaboutratioof2:1.,Etiology：antigen,Infectionrelated:Arecenthistoryofanintercurrentinfection,particularlystreptococcalrespiratoryinfectionsFoodDrugVaccine&plasmaproductOthers:insectbite,cool,trauma,sexhormonesduringspecialphase,EnvironmentalagentswhichhavebeenimplicatedinthecausationofHenoch-schonleinpurpura,Micro-organismsb-HaemolyticstreptococcusMycobacteriumtuberculosisVaricellazosterVacciniaJim閚ezandDarringtonHaemophilusparainfluenzaeStreptococcuspneumoniaeRubellaMeaslesMycoplasmapneumoniaeYersiniaenterocoliticaHumanparvovirus)HumanimmunodeficiencyvirusStaphylococcussp.Legionellasp.InfluenzaevaccineSalmonellahirschfeldiiCampylobacterjejuni,DrugsAspirinErthromycinGriseofulvinPenicillinPhenacetinPhenothiazinesQuinidineSulphonamideTetracyclineThiazidediureticsChlorpromazineParacetamol-dihydrocodeineThiramCarbamazepineStreptokinaseEnalaprilLisinoprilFluoroquinolones,FoodsCrabshrampeNutsBlackberriesEggMilkPotatoWheatMeat(various)FishChocolateChickenTomatoAlcohol,Mechanism,Immunologicbasis:antigen,antibody,IgA-CiCTcells:ImpairedabilityofTcellstosuppressB-cellfunctionBcells:InvitroproductionofbothIgAandIgGbyBcellsisincreasedinpatientscomparedwithcontrolsIgAICdepositonthevascularwallofskinandglomerulicauseVasculitis.ProinflammatoryandinflammatoryfactorsHLArelated:HLADRB1orHLADRDW35,Pathology-skin,Leucocytoclasticvasculitis:inflammatorycells,mostlypolymorphonuclearleucocytesandmononuclearcellswithoccasionaleosinophils,surroundingthecapillaries,光镜,免疫荧光,CrescentGN,免疫病理分型根据肾小球内沉积的免疫复合物不同，分为四型：1、单纯IgA沉积型（IgA）2、IgA+IgG沉积型（IgA+G）3、IgA+IgM沉积型（IgA+M）4、IgA+IgG+IgM沉积型（IgA+G+M）其中IgAGM沉积型其病理为为IVVI者占41.7%。,Clinicfeatures,PurpurarashAbdomensymptoms:Arthralgia:Renaldiseases:,Skinrash,Distinctiveinbothitsdistributionandthenatureofthelesions.PalpablepurpuraAppearingontheextensorsurfacesofthearmsandlegsParticularlyroundtheanklesandoverthebuttocksandelbows.,Othermanifestationofskinrash,MayinfusionDermalnecrosisandscarringHaemorrhagicbullaeOtherarea:,AseverebutfairlytypicalpurpuricrashofHSPaffectingthebuttocksofan11-year-oldboy.Someofthelargerlesionsarebullous,whichisunusualinchildrenbutmaybeseenmorecommonlyinadults.,Thisisuncommoninolderchildren,butmaybeseenininfants,youngchildren,andadults.,TherashofsevereHenoch-Scholeinpurpuraaffectingtheface.,Skinlesionduration,Average:3daysto2yrsOnethird:within2weeksOnethird:2-4weeksOtherthird:morethan4weeks,Jointmanifestations,Jointpain:2thirdsofallcases,onequarteraspresentingsymptomAlwaysaffectlargejointsuchasankleandkneeJointswellingusually,butnotcommonX-rayshowsperiarticularedemawithouteffusionorenlargementofjointspaceNoresponsetosalicylateRecoveryearlierthanrash,nopermanentlydamage,Abdominalandgastrointestinalsymptoms(1),AbdominalsymptomsoccurinthemajorityofpatientsTheabdominalpainiscolicky,frequentlysevere,andmaymimicanabdominalemergencyIntestinalbleeding:melaena,haematemesis,Abdominalandgastrointestinalsymptoms(2),Intussusception:SerialultrasonographyoftheabdomenreliablydistinguishesintussusceptionfromothercausesofabdominalpainIleusOtherrareabdominalmanifestationsofthediseaseincludepancreatitis,intestinalperforationandmassivegastrichaemorrhage,Mustpayattentionto,Abdominalandgastrointestinalsymptomsmayproceedothersignsandsymptoms(particularskinrash)!,Renalinvolvement(1),haematuria,proteinuria,oliguriawithfluidretention,oedemaandhypertension,impairedglomerularfiltrationrate.,Renalinvolvement(2),Mostlymild,lessseveritythenephritiseitherappearslatercommonlywithin6monthsorispresentatonsetonlyintheformofurinaryabnormalities.severityofrenalinvolvementcannotbepredictedfromtheseverityofthenon-renalsymptomsandsigns.differentincidencefromdifferentcriteria,紫癜性肾炎的临床分型,1.孤立性血尿或蛋白尿2.血尿和蛋白尿3.急性肾炎型4.肾病综合征型5.急进性肾炎型6.慢性肾炎型,Pathologykidney,IMinimalchangesIIPuremesangialproliferationwithoutcrescents(a)Focal(b)DiffuseIIIMesangialproliferativeglomerulonephritiswithlessthan50%crescents(a)Focal(b)DiffuseIVMesangialproliferativeglomerulonephritiswith50-75%crescents(a)Focal(b)DiffuseVMesangialproliferativeglomerulonephritiswithmorethan75%crescents(a)Focal(b)DiffuseVIMembranoproliferative(mesangiocapillary)glomerulonephritis,Investigations,Fullbloodcount:normalplateletUrinetest:hematuriaandproteinuriaStooltest:positiveoccultbloodtestHematology:hypercoagulationBiochemistry:BUNandScr,liverfunctionandmyocardialenzymeImmunology:ESR,Ig,complementPathology:skinandrenalbiopsyImage:ultrasound,诊断,根据本病特征的临床表现典型的皮肤紫癜，又同时合并消化道、关节或肾脏症状以及反复发作史，即可诊断。同时应与ITP、急腹症、风湿性关节炎、流脑等鉴别。,AmericanCollegeofRheumatology(1990),1.20yearsatonset,2.Palpablepurpura,3.Acuteabdominalpain,Thepresenceofgranulocytesinthewallsofsmallarteriolesorvenulesinbiopsy,5.ThepresenceoftwoormoreofthesecriteriaidentifiedHenoch-scholeinpurpura,Treatment-no-special,SupportivetreatmentRestDiet:AvoidsomefoodanddrugsAntibioticsifneedFluidandelectrolytebalancewhendietrestricted,Treatment-Otherdrugs,Steroid:abdomensymptomsandrenaldiseaseImmunosuppressivedrugs:CTXAZA雷公藤Anti-allergicdrugs:Anticoagulationfibrolyticandanti-plateletdrugs,Others,Operation:severintestinalbleedingintussusceptionandperforation,紫癜性肾炎的治疗孤立性血尿或病理级予潘生丁和（或）清热活血中药血尿和蛋白尿或病理a级：雷公