過敏性紫癜的診斷與治療ppt課件

1、泌尿系統(tǒng)疾病蘇州大學(xué)兒科學(xué)系 腎內(nèi)科 World Kidney Day March 9th 2006 was the first WKD and then the second Thursday on march will be anniversary for kidney diseases.About 10% population involved Chronic Kidney disease (CKD) in USAAbout 100 million CKD in china of the total medical cost Introduction :background Effect

2、ive management of the patient with renal disease is dependent upon establishing an accurate diagnosis.The clinician must be aware of the possible presentations of renal diseases and gather these symptoms and signs which form recognized diseases and syndrome What is the possible presentations suggest

3、ing renal problem ? Clinical presentation of renal diseaseThe patient is asymptomatic, but an abnormality has been detected on clinical or laboratory examination which indicates an underlying renal disorder.The patient complains of a symptom or has a physical sign which directly or indirectly indica

4、tes underlying renal diseasesThe patient has a systemic disease which is known to be associated with renal involvementThe patient has a family history of an inherited renal disorder. Symptoms of urinary tract disease A fever; dysuria; frequency; loinpain/abdominal pain; urinary inconsistency;offensi

5、ve smelling;cloudy urine; Frothy urine :Proteinuria;Smokey urine : Haematuria Signs of urinary tract disease Dysmorphic syndromes; Anaemia; Oedema; High blood pressure; Renal masses; Distended bladder. Acute glomerulonephritis(AGN)急性腎小球腎炎Acute glomerulonephritis(AGN)概述病因發(fā)病機(jī)制 病理生理病理臨床表現(xiàn)實(shí)驗(yàn)室檢查診斷 鑒別診斷治療

6、 預(yù)后急性腎小球腎炎目的要求了解急性腎炎的病因及發(fā)病機(jī)理。掌握普通病例與嚴(yán)重病例的臨床表現(xiàn)及治療原那么。掌握急性腎炎的實(shí)驗(yàn)室檢查。了解急性腎炎與其他病原體引起急性腎炎、慢性腎炎急性發(fā)作、特發(fā)性腎病綜合癥、IgA腎病綜合癥、急進(jìn)性腎炎等疾病的鑒別診斷。重點(diǎn)和難點(diǎn)重點(diǎn)：本病的普通病例及嚴(yán)重病例的臨床表現(xiàn)及其產(chǎn)活力理。該病的尿液及血生化檢查。小兒單純性血尿的診斷要點(diǎn)和處置原那么。難點(diǎn)：本癥重癥病例的治療，高血壓腦病急性腎功能不全，嚴(yán)重循環(huán)充血治療及處置原那么。Acute poststreptococcal glomerulonephritis 急性鏈球菌感染后腎炎Hematuria 血尿Oligur

7、ia 尿少 Edema 浮腫 關(guān)鍵詞：Key words概 述定義：急性起病，雙側(cè)性，彌漫性，前趨感染非化膿性炎癥，臨床上以血尿?yàn)橹?，可有水腫、少尿、高血壓，或腎功能不全等特點(diǎn)發(fā)病情況：年齡5-14歲、性別男女2：1、發(fā)生率下降 病 因細(xì)菌：A組溶血性鏈球菌的某些致 腎炎菌株；其他細(xì)菌有。病毒：巨細(xì)胞、乙肝病毒等其他：支原體、霉菌、原蟲等發(fā)病機(jī)制 病理生理鏈球菌致腎炎菌株的抗原成分 循環(huán)免疫復(fù)合物 原位免疫復(fù)合物 本身免疫激活補(bǔ)體系統(tǒng) 釋放生物活性因子 腎素血管緊張素醛固酮 腎小球基底膜斷裂 GRF 水鈉儲留 血尿、蛋白尿、管型尿 水腫、少尿、高血壓 腎衰病 理 毛細(xì)血管內(nèi)增生性腎小球腎炎光鏡

8、：內(nèi)皮細(xì)胞、系膜細(xì)胞增生、腫脹，系膜基質(zhì)增多，中性粒細(xì)胞浸潤，嚴(yán)重時(shí)可有“新月體構(gòu)成電鏡：上皮細(xì)胞下“駝峰樣電子致密物堆積免疫熒光：IgG、C3堆積AA：入球小動脈EA：出球小動脈EGM：腎小球外系膜PE：壁層上皮細(xì)胞PO：足突細(xì)胞GBM：腎小球基底膜F：足突US：尿液腔M：系膜PT：近曲小管MD：致密斑G：顆粒細(xì)胞N：交感神經(jīng)E：內(nèi)皮細(xì)胞 PO： 足突細(xì)胞 podocyte GBM：腎小球基底膜 glomerular basement membrane M：系膜 mesangial cell MM：系膜基質(zhì) mesangial matrix E：內(nèi)皮細(xì)胞 fenestrated endoth

9、elium 新月體腎炎臨床表現(xiàn)一前驅(qū)感染 ( Precede infection)秋冬季呼吸道感染為主夏秋季皮膚感染多見臨床表現(xiàn)二 典型表現(xiàn) ( typical manifestation )水腫 (部位, 性質(zhì), 時(shí)間)尿少 少尿，無尿血尿 腎小球性高血壓 (頭暈, 頭痛)電鏡掃描 尿紅細(xì)胞形狀臨床表現(xiàn)三嚴(yán)重表現(xiàn)(Serious manifestation)循環(huán)充血 “心衰征象 高血壓腦病猛烈頭痛 惡心嘔吐 驚厥昏迷 急性腎功能不全 少尿 氮質(zhì)血癥 電解質(zhì)紊亂呼吸困難, 肺底濕羅音心臟擴(kuò)展, 心率增快肝腫大臨床表現(xiàn)四不典型表現(xiàn) (Atypical manifestation)無病癥病例無臨床

10、病癥,有尿改動,補(bǔ)體C3下降腎外病癥型水腫,高血壓等表現(xiàn)明顯尿改動細(xì)微腎病綜合征型大量蛋白尿腎活檢病理改動類似典型病例實(shí) 驗(yàn) 室 檢 查尿常規(guī)血常規(guī) 腎功能血沉ASO血補(bǔ)體C3下降診 斷前驅(qū)鏈球菌感染史臨床病癥：血尿、水腫、高血壓等實(shí)驗(yàn)室檢查：尿檢、ASO 、C3等鑒 別 診 斷 非典型病例大量蛋白尿型 非鏈球菌感染腎炎病毒性 IgA腎炎 全身性疾病SLE，APN，乙肝腎 慢腎急發(fā) 急進(jìn)性腎炎 尿路感染 腎病綜合癥 治 療一普通治療臥床休憩約2周：水腫退，血壓降，肉眼血尿消逝；血沉正常上學(xué)；12小時(shí)尿沉渣正常恢復(fù)膂力活動飲食：低鹽：60mg/kg。d；低蛋白0。5mg/kg。d抗感染： 青霉素

11、對癥治療利尿速尿，雙氫克尿噻降壓 心痛定， ACEI：SQ14225治 療二嚴(yán)重病例 高血壓腦?。褐贵@，降壓，脫水 硝普鈉，二氮嗪 嚴(yán)重循環(huán)充血：利尿?yàn)橹魉倌颉U(kuò)血管、透析 急性腎衰： 液體 ，電解質(zhì)，酸堿，感染關(guān) 預(yù)后和預(yù)防95%完全恢復(fù)5%尿異常1%死亡預(yù)防：總結(jié)定義病理、生理臨床表現(xiàn)診斷治療腎病綜合征nephrotic syndrome)【目的要求】了解該病發(fā)病機(jī)理及病理生理。掌握原發(fā)性腎病綜合征的分型臨床，激素治療效應(yīng)，病理分型。掌握該病臨床表現(xiàn)，并發(fā)癥，診斷及治療。【重點(diǎn)和難點(diǎn)】重點(diǎn)：本病的病理生理，臨床表現(xiàn)，診斷治療，并發(fā)癥。難點(diǎn)：該病的臨床與病理分型。Nephrotic synd

12、rome 腎病綜合征Proteinuria 蛋白尿 Hypoalbuminemia 低蛋白血癥 關(guān)鍵詞：Key words一定義 是由于腎小球?yàn)V過膜對血漿蛋白的通透性增高，導(dǎo)致大量血漿白蛋白自尿中喪失而引起的一種臨床癥侯群。二病因和發(fā)病機(jī)制病因不明發(fā)病機(jī)制： 微小病變與T細(xì)胞功能紊亂有關(guān)，非微小病變與體液免疫與細(xì)胞免疫均相關(guān)細(xì)胞因子IL-2，6，8有遺傳根底與HLA相關(guān)：SSNS：DR7 FRNS：DR9裂隙膜分子的變化三病理生理 致病要素 分子屏障 腎小球?yàn)V過膜通透性 靜電屏障 大量蛋白尿 高脂血癥 脂代謝紊亂 心血管、小球硬化 低蛋白血癥 IgG感染 血漿膠體浸透壓 抗凝血酶, 、高凝，血

13、栓 鐵結(jié)合蛋白VitD3結(jié)合蛋白水 血容量 甲狀腺素結(jié)合蛋白T3、T4 分 入 (ADH，醛固酮、利鈉因子、腎小球?yàn)V過率)間質(zhì) 水 鈉儲留 水腫腎小球?yàn)V過膜通透性與分子大小的關(guān) 系不同的電荷其通透性不同陽電荷陰電荷中性電荷正常與腎病綜合征情況下白蛋白代謝四 病 理腎病綜合征常見病理改動足突細(xì)胞足突細(xì)胞的超微機(jī)構(gòu)MCD 微小病變FSGS局灶性節(jié)段性腎小球硬化MN 膜性腎病MPGN膜增生性腎小球腎炎五臨床表現(xiàn)病前常有感染水腫為主要表現(xiàn)六并 發(fā) 癥感染：呼吸道感染，皮膚感染，腹膜炎，尿路感染低血容量休克：焦躁，四肢濕冷，皮膚花紋，心 音低，血壓下降電解質(zhì)紊亂：低鈉，低鉀，低鈣血栓構(gòu)成：腎靜脈血栓腰痛

14、，肉眼血尿腎功能衰竭：休克所致的腎前性衰竭多見腎小管功能妨礙七實(shí)驗(yàn)室檢查尿常規(guī)：蛋白定性 + +24H尿蛋白定量：大于0.05 /kg肝腎功能：血潔白蛋白 6.7mmol/L免疫學(xué)檢測：ANA，抗-dsDNA抗體腎活檢八診 斷 四大特征：三高一低一高一低為主大量蛋白尿：定性 + + 定量 24H尿蛋白 大于0.05g/kg低蛋白血癥：血漿白蛋白小于30g/L(兒童) 高脂血癥： 膽固醇大于5.7mmol/L(兒童) 不同程度的水腫九分 型 臨床分型 病理分型 激素分型臨 床 分 型原發(fā)性 90%單純性腎病 ：三高一低腎炎性腎病：三高一低外，還有至少以下之一血尿：RBC大于10/HP高血壓氮質(zhì)血

15、癥：BUN大于10.7mmol/L血補(bǔ)體CH50，C3反復(fù)下降繼發(fā)性：SLE，APN，乙肝腎先天性：較少見 病 理 分 型微小病變MCD局灶性節(jié)段性腎小球硬化FsGs膜增殖性腎炎MPGN系膜增生性腎炎MsPGN膜性腎病MGN 激 素 分 型激素敏感：8周內(nèi)尿蛋白轉(zhuǎn)陰激素部分敏感：8周內(nèi)水腫退，尿蛋白+激素耐藥：8周尿蛋白+激素依賴，反復(fù)與復(fù)發(fā)，頻復(fù)發(fā)十 治 療普通治療：休憩和飲食利尿：當(dāng)水腫嚴(yán)重時(shí)，尤其有腹水時(shí)雙克，速尿，氨苯喋啶低分子右旋糖酐激素治療抗凝治療免疫調(diào)理治療中藥 激素治療(一)激素運(yùn)用階段誘導(dǎo)緩解：強(qiáng)的松1.5-2mg/kg/d 4-8w穩(wěn)定階段：間歇用藥或清晨頓服激素運(yùn)用方法短

16、程：強(qiáng)的松2mg/kg/d 4w 強(qiáng)的松1.5mg/kg/d qod 4w 共8周 激素治療(二)激素運(yùn)用方法中長程：強(qiáng)的松1.5-2mg/kg/d 4w 4w后蛋白轉(zhuǎn)陰，改強(qiáng)的松2mg/kg,隔日早餐后頓服，繼用4周，以后每2-4周減量一次，直致停藥，總療程6-9個(gè)月 激素治療(三)復(fù)發(fā)和反復(fù)的治療延伸強(qiáng)的松運(yùn)用時(shí)間加用免疫抑制劑：CTX，VCR激素耐藥的治療延伸強(qiáng)的松誘導(dǎo)期甲基強(qiáng)的松龍沖擊加用免疫抑制劑：CTX，環(huán)孢霉素A，酶芬酸酯激素副作用 激素運(yùn)用前本卷須知：感染 、胃腸道炎癥、水腫、高血壓代謝紊亂消化潰瘍和精神欣快感白白內(nèi)障，股骨頭壞死高凝形狀生長停頓易發(fā)感染急性腎上腺功能不全 免

17、疫 抑 制 劑化學(xué)制劑 烷化劑CTX、 抗代謝藥VCR、MMF真菌代謝產(chǎn)物：環(huán)孢素A、FK-506中藥及其有效成分： 雷公藤掌握劑量、療程 留意副作用：骨髓抑制、胃腸道反響、性腺抑制 其 它抗凝：低分子肝素，潘生丁溶栓：尿激酶免疫調(diào)理：左旋咪唑控制病情開展：ACEI and ARB中藥預(yù)后微小病變好經(jīng)常死于感染和激素嚴(yán)重副作用非微小病變腎病綜合癥：總結(jié)概念：三高一低病因及發(fā)病機(jī)制：了解 病理生理：重要臨床表現(xiàn) ：重要診斷：分型治療：激素的運(yùn)用謝謝泌 尿 道 感 染 Anaphylactoid Purpura (AP) & AP Nephritis (APN) OR Henoch-Schonle

18、in Purpura (HSP) Xiao zhong Li Main content Introduce definition, the etiology, Pathophysiology of HSPEmphasis pathologic changes and clinical manifestations of HSPIntroduce the treatment of HSP Description Definition : Henoch-Schonlein Purpura Syndrome, HSP: Clinical features characterized by skin

19、rash, joint pain , abdomen symptoms, renal damage, et al. Pathologic change: Systemic vasculitis Common seen in 2-8 ys of child in spring and autumn , boys more than girls about ratio of 2:1.Etiology：antigenInfection related: A recent history of an intercurrent infection, particularly streptococcal

20、respiratory infectionsFoodDrugVaccine & plasma productOthers: insect bite, cool, trauma , sex hormones during special phase Environmental agents which have been implicated in the causation of Henoch-schonlein purpuraMicro-organismsb-Haemolytic streptococcus Mycobacterium tuberculosis Varicella zoste

21、r VacciniaJim閚ez and Darrington Haemophilus parainfluenzae Streptococcus pneumoniae Rubella Measles Mycoplasma pneumoniae Yersinia enterocolitica Human parvovirus )Human immunodeficiency virusStaphylococcus sp. Legionella sp. Influenzae vaccine Salmonella hirschfeldii Campylobacter jejuni Drugs Aspi

22、rin Erthromycin Griseofulvin Penicillin Phenacetin Phenothiazines Quinidine Sulphonamide Tetracycline Thiazide diuretics Chlorpromazine Paracetamol-dihydrocodeine Thiram Carbamazepine Streptokinase Enalapril Lisinopril Fluoroquinolones FoodsCrabshrampeNuts Blackberries Egg Milk Potato Wheat Meat (va

23、rious) Fish Chocolate Chicken Tomato Alcohol Mechanism Immunologic basis: antigen, antibody, IgA-CiCT cells: Impaired ability of T cells to suppress B-cell functionB cells: In vitro production of both IgA and IgG by B cells is increased in patients compared with controls IgA IC deposit on the vascul

24、ar wall of skin and glomeruli cause Vasculitis.Proinflammatory and inflammatory factors HLA related: HLA DRB1 or HLA DR DW35Pathology-skin Leucocytoclastic vasculitis : inflammatory cells, mostly polymorphonuclear leucocytes and mononuclear cells with occasional eosinophils, surrounding the capillar

25、ies 光鏡免疫熒光 Crescent GN免疫病理分型根據(jù)腎小球內(nèi)堆積的免疫復(fù)合物不同，分為四型：1、單純IgA堆積型IgA2、IgA+IgG堆積型IgA+G3、IgA+IgM堆積型IgA+M4、IgA+IgG+IgM堆積型IgA+G+M其中IgAGM堆積型其病理為為IVVI者占41.7%。Clinic features Purpura rashAbdomen symptoms: Arthralgia : Renal diseases: Skin rash Distinctive in both its distribution and the nature of the lesions.P

26、alpable purpuraAppearing on the extensor surfaces of the arms and legsParticularly round the ankles and over the buttocks and elbows.Other manifestation of skin rashMay infusion Dermal necrosis and scarringHaemorrhagic bullaeOther area: A severe but fairly typical purpuric rash of HSP affecting the

27、buttocks of an 11-year-old boy. Some of the larger lesions are bullous, which is unusual in children but may be seen more commonly in adults. This is uncommon in older children, but may be seen in infants, young children, and adults.The rash of severe Henoch-Scholein purpura affecting the face.Skin

28、lesion durationAverage: 3 days to 2 yrsOne third : within 2 weeksOne third : 2-4 weeksOther third : more than 4 weeksJoint manifestationsJoint pain: 2 thirds of all cases, one quarter as presenting symptom Always affect large joint such as ankle and knee Joint swelling usually , but not commonX-ray

29、shows periarticular edema without effusion or enlargement of joint spaceNo response to salicylateRecovery earlier than rash, no permanently damage Abdominal and gastrointestinal symptoms(1)Abdominal symptoms occur in the majority of patientsThe abdominal pain is colicky, frequently severe, and may m

30、imic an abdominal emergencyIntestinal bleeding:melaena, haematemesisAbdominal and gastrointestinal symptoms(2)Intussusception: Serial ultrasonography of the abdomen reliably distinguishes intussusception from other causes of abdominal painIleus Other rare abdominal manifestations of the disease incl

31、ude pancreatitis, intestinal perforation and massive gastric haemorrhageMust pay attention to Abdominal and gastrointestinal symptoms may proceed other signs and symptoms (particular skin rash) !Renal involvement (1)haematuria, proteinuria, oliguriawith fluid retention, oedema and hypertension, impa

32、ired glomerular filtration rate.Renal involvement (2) Mostly mild , less severity the nephritis either appears later commonly within 6 months or is present at onset only in the form of urinary abnormalities.severity of renal involvement cannot be predicted from the severity of the non-renal symptoms

33、 and signs. different incidence from different criteria紫癜性腎炎的臨床分型1. 孤立性血尿或蛋白尿2. 血尿和蛋白尿3. 急性腎炎型4. 腎病綜合征型5. 急進(jìn)性腎炎型6. 慢性腎炎型Pathologykidney I Minimal changes IIPure mesangial proliferation without crescents(a) Focal(b) DiffuseIIIMesangial proliferative glomerulonephritis with less than 50% crescents(a)

34、Focal(b) DiffuseIVMesangial proliferative glomerulonephritis with 50-75% crescents(a) Focal(b) DiffuseVMesangial proliferative glomerulonephritis with more than 75% crescents(a) Focal(b) DiffuseVIMembranoproliferative (mesangiocapillary) glomerulonephritisInvestigationsFull blood count: normal plate

35、let Urine test: hematuria and proteinuria Stool test: positive occult blood test Hematology: hyper coagulation Biochemistry: BUN and Scr, liver function and myocardial enzyme Immunology: ESR , Ig, complement Pathology: skin and renal biopsy Image : ultrasound 診斷根據(jù)本病特征的臨床表現(xiàn)典型的皮膚紫癜，又 同時(shí)合并消化道、關(guān)節(jié)或腎臟病癥以及

36、反復(fù)發(fā)作史，即可診斷。同時(shí)應(yīng)與ITP、急腹癥、風(fēng)濕性關(guān)節(jié)炎、流腦等鑒別。 American College of Rheumatology(1990) 1. 20 years at onset, 2. Palpable purpura, 3. Acute abdominal pain, The presence of granulocytes in the walls of small arterioles or venules in biopsy ,5. The presence of two or more of these criteria identified Henoch-schol

37、ein purpura Treatment- no-special Supportive treatment RestDiet: Avoid some food and drugsAntibiotics if needFluid and electrolyte balance when diet restrictedTreatment- Other drugs Steroid : abdomen symptoms and renal disease Immunosuppressive drugs: CTX AZA 雷公藤Anti-allergic drugs:Anticoagulation fibrolytic and anti-platelet drugs Others Operation: sever intestinal bleeding intussusception and perforation 紫癜性腎炎的治療孤立性血尿或病理級予潘生丁和或清熱活血中藥血尿和蛋白尿或病理a級：雷公藤1mg/kg/d每日最大量45mg，療程3月