9-12016-5-26-北京医院-肾炎英.ppt

1、,ACUTE GLOMERULONEPHRITIS 急性肾小球肾炎,首都儿科研究所附属儿童医院 陈朝英,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,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,Physiology characteristics,出生后一段时期内呈动态成熟

2、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。,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 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,General Information,Acute g

3、lomerulonephritise acute postinfectious other secondary /primary glomerulonephritis glomerulonephritis,acute poststreptococcal glomerulonephritis,Non-poststreptococcal glomerulonephritis,A group of glomerular diseases by different causes,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

4、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20710

5、9 /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？,Clinical manifestation,Incubation: 1-3 weeks after infection., ,r ：,Clinical manifestation,1.Typical cases (1) Edema

6、 and oliguria: mild degree edema only in eyelids in the morning severe degree all over the body, edema is non pitting. urinary output decreased oliguria 250ml/m2 anuria 305Oml/d,Clinical manifestation,(2) Hematuria: almost all patients have microscopical hematuria, and about 50-70%cases have gross h

7、ematuria.,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（1)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

8、小球,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 were 1.3 mg per deciliter (110 mmol per liter) and 13 mg per deciliter (4

9、.6 mmol per liter), respectively. Microscopical examination of the urine revealed many dysmorphic urinary erythrocytes with spheroid surface protrusions (Panel A, 1450) indicative of glomerular hematuria.,A glomerular erythrocyte ghost (Panel B, 3250) has a small neck at the base of each surface pro

10、trusion, 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 contrast microscopy, 1000.),Clinical manifestation,(3) Hypert

11、ension: cases have varying degrees of hypertension (120-150/80-110mmHg). the hypertension is attributed to expanded vascular volume or vasospasm.,BP（mmHg） Preschool age120/80 School age 130/90,Frequency of clinical Manifestations in APSGN,Gross hematuria 50 70 % Volume overload Edema 70% Hypertensio

12、n 30-80% Circulatory congestion 20% CNS symptoms 10% Progressive GN 1%,Etiology,cases of GN follow infection with group A hemolytic streptococci, such as upper respiratory tract infection, Scarlet fever, skin infection.,Pathogenesis,It is now generally accepted that glomerulonephritis is a form of i

13、mmune -complex disease. The antigenic component of the immune complex be related somehow to the streptococcus.,Some strains of A group hemolytic streptococcus URI skin infection antibody production circulating in site antigen-antibody complexes immune complexes deposit in the GBM activate the comple

14、ment system incite media of inflammation,Pathogenesis,Autoimmune reaction,immune injuries (Ag-AbIC C3 ) Cells proliferation media of inflammation capillary lumen narrowed hematuria protineruria GB M injury Glomerular blood flow azotemia GFR oliguria /anuria Retention of water and natrium edema hyper

15、tension blood volume hypertensive encephalopath circulatory congestion,Pathogenesis,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,Clinical manifestation,2. Severe cases (1) Circulatory congestion the cause is attributable to an increase in blood

16、volume secondary to retention of sodium and water. It is not a real cardiac failure in the early stage of the disease, but circulatory congestion can develop to real heart failure.,Manifestations of Circulatory congestion,paroxysmal or persistent dyspnea, orthopnea, moist rales being audible in the

17、bases of the lungs, heart enlargement, HR, galloping rhythm. liver enlarged,Clinical manifestation,2. Severe cases (2) Hypertensive encephalopathy The cerebral symptoms are caused by cerebral edema ,vasospasm, cerebral anoxia Headache, vomiting, irritability/apathy, convulsion , even temporary compl

18、ete blindness. .,2. Severe cases (3) Acute renal failure: Oliguria even anuria, azotemia, hyperkalemia metabolic acidosis,Clinical manifestation,Clinical manifestation,3.Atypical cases (1) Acute nephritis with minimal urinary findings The patient may have generalized edema, hypertension, even circul

19、atory congestion, but no urinary abnormalities or minor abnormalities. Measurement of C3 and needle biopsy is needed for diagnosis.,Clinical manifestation,3.Atypical cases (2) Acute nephritis with severe proteinuria: Apart from the characteristics of acute nephritis the patient has massive proteinur

20、ia.,Clinical manifestation,3.Atypical cases (3)Asymptomatic acute nephritis (or subclinical form): the patient has mild abnormalities of urine, no other symptoms, measurement of C3 is helpful in diagnosis.,Laboratory findings,1.Urinalysis: (1) Gross or microscopic hematuria: Urine appears reddish br

21、own or smoky, RBC 3/HP , malformation RBC60%. (2) Proteinuria:1+ 3+. (3) The urinary sediment: WBC and epithelial cells, hyaline, granular and red blood cell casts,Laboratory findings,2.Blood test (1) a mild anemia (3) ESR: the erythrocyte sedimentation rate (4) Hypocomplementemia: C3, CH50.,Laborat

22、ory findings,2.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.,Pathology, In gross ob

23、servation 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 dis

24、ease, and increase in mesangialmatrix.,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 bacementmembran

25、e (GBM),毛细血管内增生性肾小球肾炎， IgG 粗大颗粒沿毛细血管 壁沉积 （荧光， 400 400）,In AGN, immunofluorescent studies show lumpy-bumpy granular staining for IgG in peripheral capillary loops and in mesangial areas,Diagnosis3 steps,1st,Clinical manifestation,abruptly with hematuria Proteinuria Hypertension azotemia,asymptomatic,

26、oliguric acute renal failure,latent period,Pharyngitis skin infection,nephritis,2nd,3rd evidence of recent group A streptococcal infection a positive skin or throat culture ASO(+)、Anti-SpeB,Diagnosis,Special important Low C3（90%） Returns to normal in 8 weeks in all patients with uncomplicated PSGN I

27、s helpful for diagnosis subclinical APSGN（ mild abnormalities of urine，transient drop in complement）,Indications for renal biopsy,Massive proteinuria Complement is persistently reduced after 2-3months Acute nephrosis syndrome with normal serum complement Serum creatinine increases progressively,Diff

28、erential diagnosis,1.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,Differential diagnosis,3. Chronic nephritis also may easily

29、be mistaken for acute disease. 4. RPGN Rapidly Progressive glomerulonephritis 5. Primary NS,Summary of Primary Renal Diseases That Manifest as Acute Glomerulonephritis,Treatment,1.Nursing and diet Bed rest is indicated as long as there are clinical manifestations of active disease, such as edema, hy

30、pertension, or gross hematuria Protein restricted( intake 0.5g/kg.d) Salt restricted ( 60mg/kg.day) ESR normal go to school,Treatment,2 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 iv 4 Antihypertensiv

31、e 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).,Treatment,5.Treatment of severe complications Hypertensive encephalopathy: Sodium nitroprusside( l8g/kg ) introvenously drop continually, regulating the dropp

32、ing speed according to the level of BP. Anticonvulsant water deprivation with Mannitol oxygen therapy,Treatment,5.Treatment of severe complications Severe Circulatory Congestion: Restriction of salt and water, diuretics. Sodium nitroprusside Acute renal failure: Maintaining balance of water, electro

33、lytes and acid base,applying energy and preventing complications. Peritoneal and hemodialysis may be needed.,Course and prognosis,2 weeks edema gross hematuria Hypertension Abnormal urine usually lasts 1 years or longer. The prognosis of AGN is very good.,Prognosis,The early mortality is very low in

34、 children Cardiovascular complications are the main cause Irreversible renal failure may fellow acute nephrisis with crescentic proliferation 10-20 yrs later 20% -abnormal urine analysis or creatinine clearence 1% -azotaemia 8-13%-proteinuria and hypertension The incidence of glomerular sclerosis an

35、d fibrosis is nearly 50%,but their relevance is uncertain,与急性链球菌感染后肾炎预后有关的因素 有关因素 无关因素 发病（流行发病较散发者好） 1 前驱感染史及严重程度 年龄（儿童预后好，老年差） 2 肉眼血尿的严重性 严重而持续的高血压、肾病综 3 血补体下降程度 合征及肾功能损害（预后差） 光镜下广泛大新月体；电镜下 4 血ASO滴度上升程度 不典型驼峰；荧光呈花环状改 变（预后差）,Microhaematuria,Intermittent Proteinuria,Gross H,Hypertention,Low C3,Persis

36、tent Proteinuria,2 weeks 4 weeks 2 months 6 months 1 years 2 years,Clinical and Laboratory features,Time course to revolution of APSGN,Summery,ASO+,C3,实验室检查,临床表现,诊断,治疗,Reference,Brenner and Rectors The Kidney Nelson Textbook of Pediatrics - 18Ed Oxford Textbook of Clinical Nephrology(3rd Ed),Thank You For Your Attention,