过敏性紫癜的诊断与治疗

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,泌尿系统,疾,疾病,苏州大学,儿,儿科学系,肾,肾内科,World Kidney Day,March 9th 2006wasthefirstWKDandthenthe secondThursday onmarch willbe anniversary for kidney diseases.,About 10% population involved Chronic Kidney disease (CKD)in USA,About 100 million CKD inchina, ofthetotal medical cost,Introduction:background,Effective managementofthepatientwithrenal disease is dependentuponestablishing anaccurate diagnosis.,Theclinician must be awareof the possiblepresentations ofrenal diseasesandgather thesesymptoms and signswhich form recognized diseases and syndrome,Whatisthepossiblepresentations suggesting renalproblem?,Clinicalpresentation ofrenal disease,Thepatientis asymptomatic,butanabnormalityhasbeendetected onclinical orlaboratoryexaminationwhich indicatesan underlying renaldisorder.,Thepatientcomplains ofa symptom or has aphysicalsign whichdirectlyorindirectly indicatesunderlyingrenal diseases,Thepatienthasa systemic disease whichisknown tobeassociated withrenal involvement,Thepatienthasa familyhistoryofan inheritedrenal disorder.,Symptomsofurinarytractdisease,Afever;dysuria;frequency;loinpain/abdominalpain;urinaryinconsistency;offensivesmelling;cloudyurine;,Frothyurine:,Proteinuria;,Smokeyurine:,Haematuria,Signsofurinarytractdisease,Dysmorphicsyndromes;,Anaemia;,Oedema;,Highbloodpressure;,Renalmasses;,Distendedbladder.,Acuteglomerulonephritis(AGN),急,性,性,肾,肾,小,小,球,球,肾,肾,炎,炎,Acuteglomerulonephritis(AGN),概,述,述,病,因,因,发,病,病,机,机,制,制,病,病,理,理,生,生,理,理,病,理,理,临,床,床,表,表,现,现,实,验,验,室,室,检,检,查,查,诊,断,断,鉴,鉴,别,别,诊,诊,断,断,治,疗,疗,预,预,后,后,急,性,性,肾,肾,小,小,球,球,肾,肾,炎,炎,目,的,的,要,要,求,求,了,解,解,急,急,性,性,肾,肾,炎,炎,的,的,病,病,因,因,及,及,发,发,病,病,机,机,理,理,。,。,掌,握,握,一,一,般,般,病,病,例,例,与,与,严,严,重,重,病,病,例,例,的,的,临,临,床,床,表,表,现,现,及,及,治,治,疗,疗,原,原,则,则,。,。,掌,握,握,急,急,性,性,肾,肾,炎,炎,的,的,实,实,验,验,室,室,检,检,查,查,。,。,了,解,解,急,急,性,性,肾,肾,炎,炎,与,与,其,其,他,他,病,病,原,原,体,体,引,引,起,起,急,急,性,性,肾,肾,炎,炎,、,、,慢,慢,性,性,肾,肾,炎,炎,急,急,性,性,发,发,作,作,、,、,特,特,发,发,性,性,肾,肾,病,病,综,综,合,合,症,症,、,、IgA,肾,肾,病,病,综,综,合,合,症,症,、,、,急,急,进,进,性,性,肾,肾,炎,炎,等,等,疾,疾,病,病,的,的,鉴,鉴,别,别,诊,诊,断,断,。,。,重,点,点,和,和,难,难,点,点,重,点,点,：,：,本,本,病,病,的,的,一,一,般,般,病,病,例,例,及,及,严,严,重,重,病,病,例,例,的,的,临,临,床,床,表,表,现,现,及,及,其,其,产,产,生,生,机,机,理,理,。,。,该,病,病,的,的,尿,尿,液,液,及,及,血,血,生,生,化,化,检,检,查,查,。,。,小,儿,儿,单,单,纯,纯,性,性,血,血,尿,尿,的,的,诊,诊,断,断,要,要,点,点,和,和,处,处,理,理,原,原,则,则,。,。,难,点,点,：,：,本,本,症,症,重,重,症,症,病,病,例,例,的,的,治,治,疗,疗,，,，,高,高,血,血,压,压,脑,脑,病,病,急,急,性,性,肾,肾,功,功,能,能,不,不,全,全,，,，,严,严,重,重,循,循,环,环,充,充,血,血,治,治,疗,疗,及,及,处,处,理,理,原,原,则,则,。,。,Acutepoststreptococcalglomerulonephritis,急,性,性,链,链,球,球,菌,菌,感,感,染,染,后,后,肾,肾,炎,炎,Hematuria,血,血,尿,尿,Oliguria,尿,尿,少,少,Edema,浮,浮,肿,肿,关,键,键,词,词,：,：Keywords,概,述,述,定,义,义,：,：,急,急,性,性,起,起,病,病,，,，,双,双,侧,侧,性,性,，,，,弥,弥,漫,漫,性,性,，,，,前,前,趋,趋,感,感,染,染,（,（,非,非,化,化,脓,脓,性,性,炎,炎,症,症,）,）,，,，,临,临,床,床,上,上,以,以,血,血,尿,尿,为,为,主,主,，,，,可,可,有,有,水,水,肿,肿,、,、,少,少,尿,尿,、,、,高,高,血,血,压,压,，,，,或,或,肾,肾,功,功,能,能,不,不,全,全,等,等,特,特,点,点,发,病,病,情,情,况,况,：,：,年,年,龄,龄5-14,岁,岁,、,、,性,性,别,别,男,男,女,女2,：,：1,、,、,发,发,生,生,率,率,下,下,降,降,病,因,因,细,菌,菌,：,：A,组,组,溶,溶,血,血,性,性,链,链,球,球,菌,菌,的,的,某,某,些,些,致,致,肾,肾,炎,炎,菌,菌,株,株,；,；,其,其,他,他,细,细,菌,菌,有,有,。,。,。,。,。,。,。,。,。,。,病,毒,毒,：,：,巨,巨,细,细,胞,胞,、,、,乙,乙,肝,肝,病,病,毒,毒,等,等,其,他,他,：,：,支,支,原,原,体,体,、,、,霉,霉,菌,菌,、,、,原,原,虫,虫,等,等,发,病,病,机,机,制,制,病,病,理,理,生,生,理,理,链,球,球,菌,菌,致,致,肾,肾,炎,炎,菌,菌,株,株,的,的,抗,抗,原,原,成,成,分,分,循,环,环,免,免,疫,疫,复,复,合,合,物,物,原,原,位,位,免,免,疫,疫,复,复,合,合,物,物,自,自,身,身,免,免,疫,疫,激,活,活,补,补,体,体,系,系,统,统,释,放,放,生,生,物,物,活,活,性,性,因,因,子,子,肾,肾,素,素,血,血,管,管,紧,紧,张,张,素,素,醛,醛,固,固,酮,酮,肾,小,小,球,球,基,基,底,底,膜,膜,断,断,裂,裂GRF,水,钠,钠,储,储,留,留,血,尿,尿,、,、,蛋,蛋,白,白,尿,尿,、,、,管,管,型,型,尿,尿,水,水,肿,肿,、,、,少,少,尿,尿,、,、,高,高,血,血,压,压,肾,衰,衰,病,理,理,毛,细,细,血,血,管,管,内,内,增,增,生,生,性,性,肾,肾,小,小,球,球,肾,肾,炎,炎,光镜：内皮,细,细胞、系膜,细,细胞增生、,肿,肿胀，系膜,基,基质增多，,中,中性粒细胞,浸,浸润，严重,时,时可有“新,月,月体”形成,电镜：上皮,细,细胞下“驼,峰,峰样”电子,致,致密物沉积,免疫荧光：IgG、C,3,沉积,AA：入球,小,小动脉,EA：出球,小,小动脉,EGM：肾,小,小球外系膜,PE：壁层,上,上皮细胞,PO：足突,细,细胞,GBM：肾,小,小球基底膜,F：足突,US：尿液,腔,腔,M：系膜,PT：近曲,小,小管,MD：致密,斑,斑,G：颗粒细,胞,胞,N：交感神,经,经,E：内皮细,胞,胞,PO： 足,突,突细胞,podocyte,GBM：肾,小,小球基底膜,glomerularbasementmembrane,M：系膜,mesangialcell,MM：,系膜,基质,mesangialmatrix,E：内皮细,胞,胞,fenestrated endothelium,新月体肾炎,临床表现,（一）,前驱感染( Precedeinfection),秋冬季呼吸,道,道感染为主,夏秋季皮肤,感,感染多见,临床表现,（二）,典型表现,(,typical manifestation,),水肿(部位,性,性质, 时,间,间),尿少 （少,尿,尿，无尿）,血尿 （肾,小,小球性）,高血压(头晕,头,头痛),电镜扫描,尿,尿红,细,细胞形态,临床表现,（三）,严重表现(Serious manifestation),循环充血,“心衰”征,象,象,高血压脑病,剧烈头痛,恶,恶心呕,吐,吐 惊,厥,厥昏迷,急性肾功能,不,不全,少尿,氮,氮质血症,电,电解质,紊,紊乱,呼吸困难,肺,肺底湿罗,音,音,心脏扩大,心,心率增快,肝肿大,临床表现,（四）,不典型表现(Atypical manifestation),无症状病例,无临床症状,有尿改变,补体C3,下,下降,肾外症状型,水肿,高血,压,压等表现明,显,显,尿改变轻微,肾病综合征,型,型,大量蛋白尿,肾活检病理,改,改变类似典,型,型病例,实 验 室,检,检 查,尿常规,血常规,肾功能,血沉,ASO,血补体（C,3,下降）,诊 断,前驱链球菌,感,感染史,临床症状：,血,血尿,、,水肿、高血,压,压等,实验室检查,：,：尿检,、ASO,、,、C,3等,鉴 别 诊,断,断,非典型病例,（,（大量蛋白,尿,尿型）,非链球菌感,染,染肾炎（病,毒,毒性）,IgA肾炎,全身性疾病,（,（SLE，APN，乙,肝,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,约2周）：,水,水肿退，血,压,压降，肉眼,血,血尿消失；,血,血沉正常上,学,学；12小,时,时尿沉渣正,常,常恢复体力,活,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,噻）,降压 （心,痛,痛定， ACEI：SQ,14225,）,治 疗,（二）,严重病例,高血压脑病,：,：,止惊，降压,，,，脱水,（硝普钠，,二,二氮嗪）,严重循环充,血,血：,利尿为主（,速,速尿）、扩,血,血管、透析,急性肾衰：,液体 ，电,解,解质，酸碱,，,，感染关,预后和预防,95%完全,恢,恢复,5%尿异,常,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,病机理及病,理,理生理。,掌握原发性,肾,肾病综合征,的,的分型（临,床,床，激素治,疗,疗效应，病,理,理分型）。,掌握该病临,床,床表现，并,发,发症，诊断,及,及治疗。,【重点和难,点,点】,重点：本病,的,的病理生理,，,，临床表现,，,，诊断治疗,，,，并发症。,难点：该病,的,的临床与病,理,理分型。,Nephroticsyndrome,肾,肾病,综,综合征,Proteinuria,蛋,蛋白尿,Hypoalbuminemia,低,低蛋,白,白血症,关键词：Key words,（一）,定义,是由于肾小,球,球滤过膜对,血,血浆蛋白的,通,通透性增高,，,，导致大量,血,血浆白蛋白,自,自尿中丢失,而,而引起的一,种,种临床症侯,群,群。,（二）,病因和发病,机,机制,病因不明,发病机制：,微小病变与T细胞功能,紊,紊乱有关，,非,非微小病变,与,与体液免疫,与,与细胞免疫,均,均相关,细胞因子（IL-2，6，8）,有遗传基础,与,与HLA相,关,关：SSNS：DR7,FRNS：DR9,裂隙膜分子,的,的变化,（三）病理,生,生理,致病因素,分子屏障,肾小球滤过,膜,膜通透性,静电屏障,大量蛋白尿,高,高脂血,症,症,脂代谢紊乱,心,心血管、小,球,球硬化,低蛋白血症,IgG,感,感染,血浆胶体渗,透,透压,抗,抗凝血,酶,酶,、,高,高凝，血栓,铁结合蛋白,VitD,3,结合蛋白,水,血,血容量,甲,甲状,腺,腺素结合蛋,白,白T,3,、T,4,分,入(ADH,，,，醛,固,固酮,、,利,利钠,因,因子,、,肾,肾小,球,球滤,过,过率,),间,质,水,水,钠,钠储,留,留,水肿,肾小球滤过膜通透性与分子,大小的关 系,不同,的,的电,荷,荷其,通,通透,性,性不,同,同,阳电,荷,荷,阴电,荷,荷,中性,电,电荷,正常,与,与肾,病,病综,合,合征,情,情况,下,下白,蛋,蛋白,代,代谢,（四,）,）,病,理,理,肾病,综,综合,征,征常,见,见病,理,理改,变,变,足,突,细,胞,足,突,细,胞,的,超,微,机,构,MCD,微,微,小,小病,变,变,FSGS,（,局灶,性,性节,段,段性,肾,肾小,球,球硬,化,化）,MN,膜,膜,性,性肾,病,病,MPGN,（膜,增,增生,性,性肾,小,小球,肾,肾炎,）,）,（五,）,）,临床,表,表现,病前,常,常有,感,感染,水肿,为,为主,要,要表,现,现,（六,）,）,并,发,发,症,症,感染,：,：,呼吸,道,道感,染,染，,皮,皮肤,感,感染,，,，腹,膜,膜炎,，,，尿,路,路感,染,染,低血,容,容量,休,休克,：,：,烦躁,，,，四,肢,肢湿,冷,冷，,皮,皮肤,花,花纹,，,，心,音低,，,，血,压,压下,降,降,电解,质,质紊,乱,乱：,低钠,，,，低,钾,钾，,低,低钙,血栓,形,形成,：,：,肾静,脉,脉血,栓,栓（,腰,腰痛,，,，肉,眼,眼血,尿,尿）,肾功,能,能衰,竭,竭：,休克,所,所致,的,的肾,前,前性,衰,衰竭,多,多见,肾小,管,管功,能,能障,碍,碍,（七,）,）实,验,验室,检,检查,尿常,规,规：,蛋白,定,定性+,24H尿,蛋,蛋白,定,定量,：,：,大于0.05/kg,肝肾,功,功能,：血,清,清白,蛋,蛋白6.7mmol/L,免疫,学,学检,测,测：,ANA，,抗,抗-dsDNA抗,体,体,肾活,检,检,（八,）,）,诊,断,断,四大,特,特征,：三,高,高一,低,低（,一,一高,一,一低,为,为主,）,）,大量,蛋,蛋白,尿,尿,：定性+,定量24H,尿,尿蛋,白,白,大,大于0.05g/kg,低蛋,白,白血,症,症,：血浆,白,白蛋,白,白小,于,于30,g/L(,儿童),高脂,血,血症,：胆固,醇,醇大,于,于5.7mmol/L(,儿童),不同,程,程度,的,的水,肿,肿,（九,）,）,分,型,型,临床,分,分型,病理,分,分型,激素,分,分型,临,床,床,分,分,型,型,原发,性,性90%,单纯,性,性肾,病,病,：,：,三高,一,一低,肾炎,性,性肾,病,病：,三高,一,一低,外,外，,还,还有,至,至少,以,以下,之,之一,血尿,：,：RBC,大,大于10/HP,高血,压,压,氮质,血,血症,：,：BUN,大,大于10.7mmol/L,血补,体,体CH,50,，,，,C,3,反复,下,下降,继发,性,性：SLE，APN，,乙,乙肝,肾,肾,先天,性,性：,较,较少,见,见,病,理,理,分,分,型,型,微小,病,病变,（,（MCD,）,）,局灶,性,性节,段,段性,肾,肾小,球,球硬,化,化（FsGs,）,）,膜增,殖,殖性,肾,肾炎,（,（MPGN）,系膜,增,增生,性,性肾,炎,炎（MsPGN）,膜性,肾,肾病,（,（MGN,）,）,激,素,素,分,分,型,型,激素,敏,敏感,：,：,8周,内,内尿,蛋,蛋白,转,转阴,激素,部,部分,敏,敏感,：,：,8周,内,内水,肿,肿退,，,，尿,蛋,蛋白+,激素,耐,耐药,：,：,8周,尿,尿蛋,白,白+,（激,素,素依,赖,赖，,反,反复,与,与复,发,发，,频,频复,发,发）,（十,）,）,治,疗,疗,一般,治,治疗,：,：休,息,息和,饮,饮食,利尿,：,：当,水,水肿,严,严重,时,时，,尤,尤其,有,有腹,水,水时,双克,，,，速,尿,尿，,氨,氨苯,喋,喋啶,低分,子,子右,旋,旋糖,酐,酐,激素治疗,抗凝治疗,免疫调节,治,治疗,中药,激素治疗(一),激素使用,阶,阶段,诱导缓解,：,：强的松1.5-2mg/kg/d4-8w,巩固阶段,：,：间歇用,药,药或清晨,顿,顿服,激素使用,方,方法,短程：强,的,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,方法,中长程：,强,强的松1.5-2mg/kg/d4w,4w,后蛋白转,阴,阴，改强,的,的松2mg/kg,隔日早,餐,餐后顿服,，,，继用4,周,周，以后,每,每2-4,周,周减量一,次,次，直致,停,停药，总,疗,疗程6-9个月,激素治疗(三),复发和反,复,复的治疗,延长强的,松,松使用时,间,间,加用免疫,抑,抑制剂：CTX，VCR,激素耐药,的,的治疗,延长强的,松,松诱导期,甲基强的,松,松龙冲击,加用免疫,抑,抑制剂：CTX，,环,环孢霉素A，酶芬,酸,酸酯,激素副作,用,用,激素应用,前,前注意事,项,项：感染,、,、胃肠,道,道炎症、,水,水肿、高,血,血压,代谢紊乱,消化溃疡,和,和精神欣,快,快感,白白内障,，,，股骨头,坏,坏死,高凝状态,生长停止,易发感染,急性肾上,腺,腺功能不,全,全,免 疫,抑,抑 制,剂,剂,化学制剂,烷化剂（CTX）,、,、,抗代谢药,（,（VCR,、,、MMF,）,）,真菌代谢,产,产物：,环孢素A,、,、FK-506,中药及其,有,有效成分,：,：,雷公藤,掌握剂量,、,、疗程,注意副作,用,用,：骨髓抑,制,制、胃肠,道,道反应、,性,性腺抑制,其 它,抗凝：低分子肝,素,素，潘生,丁,丁,溶栓：尿激酶,免疫调节,：,：左旋咪唑,控制病情,发,发展：ACEIandARB,中药,预后,微小病变,好,好,常常死于,感,感染和激,素,素严重副,作,作用,非微小病,变,变肾病综,合,合症：,总结,概念：三,高,高一低,病因及发,病,病机制：,了,了解,病理生理,：,：重要,临床表现,：,：重要,诊断：分,型,型,治疗：激,素,素的应用,谢谢,泌 尿,道,道 感,染,染,Anaphylactoid Purpura (AP)&AP Nephritis(APN)OR Henoch-SchonleinPurpura(HSP),XiaozhongLi,Maincontent,Introduce definition, the etiology,PathophysiologyofHSP,Emphasispathologicchangesandclinicalmanifestationsof HSP,Introduce the treatmentofHSP,Description,Definition :Henoch-Schonlein Purpura Syndrome,HSP:,Clinicalfeatures characterizedby skinrash, jointpain, abdomen symptoms,renal damage, et al.,Pathologic change: Systemicvasculitis,Commonseenin2-8ys of childin spring and autumn ,boys more thangirls aboutratio of2:1.,Etiology,：,：antigen,Infection related: Arecenthistoryof an intercurrent infection, particularly streptococcalrespiratoryinfections,Food,Drug,Vaccine& plasmaproduct,Others:insect bite,cool, trauma ,sexhormonesduringspecialphase,Environmental agentswhich havebeenimplicatedin the causationofHenoch-schonleinpurpura,Micro-organisms,b-Haemolyticstreptococcus,Mycobacterium tuberculosis,Varicella zoster,VacciniaJim,閚,閚ezandDarrington,Haemophilusparainfluenzae,Streptococcus pneumoniae,Rubella,Measles,Mycoplasma pneumoniae,Yersiniaenterocolitica,Human parvovirus),Human immunodeficiency,virus,Staphylococcus sp.,Legionella sp.,Influenzae vaccine,Salmonella hirschfeldii,Campylobacter jejuni,Drugs,Aspirin,Erthromycin,Griseofulvin,Penicillin,Phenacetin,Phenothiazines,Quinidine,Sulphonamide,Tetracycline,Thiazidediuretics,Chlorpromazine,Paracetamol-dihydrocodeine,Thiram,Carbamazepine,Streptokinase,Enalapril,Lisinopril,Fluoroquinolones,Foods,Crab,shrampe,Nuts,Blackberries,Egg,Milk,Potato,Wheat,Meat(various),Fish,Chocolate,Chicken,Tomato,Alcohol,Mechanism,Immunologicbasis: antigen,antibody, IgA-CiC,T cells:Impairedabilityof Tcells to suppress B-cell function,B cells:,In vitro,production of both IgA and IgG by Bcells isincreased in patients comparedwithcontrols,IgAIC deposit on the vascular wallof skinandglomeruli causeVasculitis.,Proinflammatoryandinflammatoryfactors,HLArelated:HLADRB1 orHLADRDW35,Pathology-skin,Leucocytoclasticvasculitis:,inflammatorycells,mostly polymorphonuclearleucocytesandmononuclearcells with occasional eosinophils, surrounding the capillaries,光镜,免疫荧光,CrescentGN,免疫病理,分,分型,根据肾小,球,球内沉积,的,的免疫复,合,合物不同,，,，分为四,型,型：,1、单纯IgA沉,积,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,积型其病,理,理为为IVVI,者,者占41.7%。,Clinic features,Purpurarash,Abdomensymptoms:,Arthralgia :,Renal diseases:,Skinrash,Distinctivein bothitsdistributionandthenatureof the lesions.,Palpablepurpura,Appearing ontheextensor surfaces of the arms and legs,Particularlyround the ankles and over the buttocksandelbows.,Other manifestationofskinrash,Mayinfusion,Dermal necrosisandscarring,Haemorrhagicbullae,Other area:,A severebutfairlytypicalpurpuricrash ofHSPaffecting the buttocksof an 11-year-old boy. Someof the larger lesions are bullous, whichisunusualin childrenbutmaybe seenmorecommonly inadults.,Thisisuncommoninolder children,butmaybe seenin infants,young children,andadults.,Therashofsevere Henoch-Scholein purpura affectingtheface.,Skinlesionduration,Average:3daysto2 yrs,Onethird :within2 weeks,Onethird :2-4weeks,Other third: more than4 weeks,Joint manifestations,Joint pain:2 thirdsofallcases, one quarter as presentingsymptom,Always affect largejoint such as ankleandknee,Joint swelling usually ,butnotcommon,X-ray showsperiarticular edemawithouteffusionorenlargementof jointspace,No responseto salicylate,Recoveryearlierthan rash,no permanently damage,Abdominal and gastrointestinal symptoms(1),Abdominal symptoms occurinthemajorityofpatients,Theabdominal pain is colicky, frequently severe, and may mimican abdominalemergency,Intestinal bleeding:melaena, haematemesis,Abdominaland gastrointestinalsymptoms(2),Intussusception: Serialultrasonographyofthe abdomenreliablydistinguishesintussusceptionfromothercausesofabdominal pain,Ileus,Otherrare abdominalmanifestationsofthediseaseinclude pancreatitis,intestinalperforationandmassivegastric haemorrhage,Must payattentionto,Abdominaland gastrointestinalsymptomsmay proceedother signs andsymptoms(particularskin rash)!,Renalinvolvement(1),haematuria,proteinuria,oliguria,with fluid retention,oedema andhypertension,impairedglomerular filtrationrate.,Renalinvolvement(2),Mostlymild, lessseverity,thenephritis eitherappears later commonly within6 monthsorispresent at onset onlyinthe formofurinaryabnormalities.,severityofrenalinvolvementcannotbepredicted fromthe severity of thenon-renalsymptomsand signs.,differentincidence fromdifferent criteria,紫癜性,肾,肾炎的,临,临床分,型,型,1.,孤,孤立性,血,血尿或,蛋,蛋白尿,2.,血,血尿和,蛋,蛋白尿,3.,急,急性肾,炎,炎型,4.,肾,肾病综,合,合征型,5.,急,急进性,肾,肾炎型,6.,慢,慢性肾,炎,炎型,Pathology,kidney,IMinimalchanges,IIPure mesangialproliferation withoutcrescents,(a)Focal,(b)Diffuse,IIIMesangial proliferativeglomerulonephritis withlessthan 50%crescents,(a)Focal,(b)Diffuse,IVMesangialproliferativeglomerulonephritiswith50-75%crescents,(a)Focal,(b)Diffuse,VMesangialproliferative glomerulonephritiswith morethan75%crescents,(a)Focal,(b)Diffuse,VIMembranoproliferative(mesangiocapillary)glomerulonephritis,Investigations,Full blood count:normalplatelet,Urinetest:hematuriaand proteinuria,Stooltest:positiveoccultbloodtest,Hematology:hypercoagulation,Biochemistry: BUNandScr,liverfunctionandmyocardialenzyme,Immunology:ESR,Ig,complement,Pathology: skinand renal biopsy,Image: ultrasound,诊断,根据本,病,病特征,的,的临床,表,表现典,型,型的皮,肤,肤紫癜,，,，又,同,同时合,并,并消化,道,道、关,节,节或肾,脏,脏症状,以,以及反,复,复发作,史,史，即,可,可诊断,。,。,同时应,与,与ITP、急,腹,腹症、,风,风湿性,关,关节炎,、,、流脑,等,等鉴别,。,。,AmericanCollege of Rheumatology(1990),1.,20yearsatonset,2.Palpable purpura,3.Acute abdominalpain,Thepresenceofgranulocytes in thewalls,ofsmallarterioles or venulesinbiopsy ,5.Thepresenceoftwo or moreofthesecriteriaidentifiedHenoch-scholeinpurpura,Treatment-no-special,Supportive treatment,Rest,Diet:Avoid some food anddrugs,Antibiotics ifneed,Fluidandelectrolyte balancewhendietrestricted,Treatment-,Otherdrugs,Steroid :abdomen symptoms andrenal disease,Immunosuppressive drugs:,CTXAZA,雷,雷公藤,Anti-allergic drugs:,Anticoagulationfibrolytic and anti-plateletdrugs,Others,Operation:sever intestinal bleeding intussusceptionand perforation,紫癜性肾炎,的,的治疗,孤立性血尿,或,或病理级,予,予潘生丁和,（,（或）清热,活,活血中药,血尿和蛋白,尿,尿或病理a级：雷公,藤,藤1mg/kg/d（,每,每日最大量,45mg,，,，疗程3月,急性肾炎型,（,（尿蛋尿1g/d）,或,或 病理b、a,级,级：雷公藤,，,，疗程36月,肾病综合征,型,型病理b、级：,强,强的松雷,公,公藤或环磷,酰,酰胺强的,松,松冲击疗法,急进性肾炎,型,型或病理,、,、级：,甲,甲强龙冲击,（,（1530mg/kg）CTX肝素,潘,潘生丁四联,疗,疗法,The greatmajority of patients, recovercompletely from Henoch-schonlein purpura with nomeasurableimpairment ofrenalfunction,In large studies ofunselectedchildren:death or endstagerenal diseaseensued insevenof 245 patients andpersisting urinaryabnormalities were presentin5 to15per centafterfollow-upforseveral years.,Predictionof renaloutcome,Classificationof clinical status at follow-up ofpatients withHenoch-schonlein purpuranephritis-Outcomegroup,ANormal,Normal physicalexamination, no urinaryabnormality,normal renal function,BMinor urinaryabnormality,Normal physicalexamination, haematuria,(microscopic-intermittentmacroscopic) and/or,proteinuria 1g/24h and/or hypertension, normalrenal,function,DRenal insufficiency,Glomerularfiltration rate 60ml/min/1.73 m2, death or,renalreplacement therapy,Whats thefocus today ?,Definition,Pathologyof renal involvement,Clinical features,Treatment,Key words,AP,APN,Aimsand Requirements,Getting hold ofdefinition , pathologicchanges and clinicalmanifestationsof HSP,Understanding the etiology, pathophysiology of HSP,Familiarizing with the treatment ofHSPN,Thankyour attention,