病理学——泌尿系统ppt课件

1、泌尿系统疾病 The Kidney and its collecting system,第十二章,肾脏功能,排泄代谢产物 内分泌功能,调节体液平衡 维持酸碱平衡,Normal adult kidney,150 grams 1 million nephrons 14 calyces,Normal adult kidney,4 basic morphologic components,肾小球 Glomeruli 肾小管 Tubules 肾间质 Interstitium 血管 Blood vessels,流行病学,美国40万终末肾病人 Hypertension Diabetes Chronic in

2、fection Cystic disease 透析和肾移植,47,000 per year for peritoneal dialysis $76,000 per year for hemodialysis,52,000 per procedure 70,000 people awaiting,血 透,腹 透,肾小球疾病 Glomerular diseases,原发性肾小球肾炎 Primary Glomerulonephritis 继发性肾小球疾病 Glomerulopathies Secondary to Systemic Diseases 遗传性肾小球疾病 Hereditary disea

3、se,Visceral epithelial cells (podocytes) scanning electron micrograph,水和离子自由通过，蛋白不能通过 size-dependent (3.5 nm) charge-dependent (the polyanions,滤过膜,肾小球损伤的机制,大部分与免疫复合物沉积有关 抗体在滤过膜与固有/植入抗体反应 循环免疫复合物沉积 Mediated by complement activation, polys, perhaps also macrophages, coagulation system, etc,肾穿刺 Renal p

4、uncture,Electron microscopy,Normal,电子致密物沉积,Granular, characteristic of circulating and in situ immune complex deposition,Immunofluorescence microscopy,Linear, characteristic of classic anti-GBM antibody GN,Normal glomerulus stained with HE,Normal glomerulus stained with PAS,how does glomerular damag

5、e ensue,1,Podocyte injury,急性弥漫增生性肾小球肾炎毛细血管内增生性肾小球肾炎(链球菌)感染后性肾小球肾炎,一)（五,Acute post-streptococcal GN,2 weeks later,Acute diffuse proliferative GN,Normal,Acute diffuse proliferative GN,EM,Immunofluorescence (Ig and complement,内皮细胞和系膜细胞弥漫性肿胀，增生,Choke off their blood supply,毛细血管损伤,血尿,蛋白尿,高血压,水 肿,肾炎综合征,Pa

6、thologyClinical feature,oliguria,azotemia,GFR,预 后,儿童病例多数康复，少数进展为快速进行型GN或慢性肾病 15%-25% 成人病例发展为终末肾,Goodpastures GN, anti IgG,Crescentic GN (PAS stain,Oliguria Anuric Azotemia,Nephritic syndrome,预 后,肾小球受累少于80%者略好于超过80%者 部分病人血浆置换有效 (Goodpasture syndrome,膜性肾小球肾炎/膜性肾病,30y-50y好发,缓慢进展 Heymann nephritis (动物模型

7、) Ag in situ Action of C5b-C9 on podocytes 病理变化：GBM弥漫性增厚 足突消失,三-1)（三,Normal,Membranous glomerulopathy (Silver stain,Membranous glomerulopathy (EM,Membranous glomerulopathy (EM,Immunofluorescence (Ig and complement,GBM damaged,Nephrin lost,低蛋白血症,大量蛋白尿,高脂血症和脂尿,高度水肿,肾病综合征,PathologyClinical feature,预 后,

8、差异大，起病和进展隐匿 60% 病例持续蛋白尿 40% 2-20年进展为终末肾 10-30% 有部分或完全蛋白尿缓解,轻微肾小球病变/脂性肾病,光镜下肾小球正常 1-7 岁儿童 Disorders of T-cell 肾病综合症,三-2)（一,临床表现及预后,选择性蛋白尿 90% 对短程激素疗法有效，但其中2/3蛋白尿复发 204 cases 10years follow-up Complete remission 71% Persist proteinuria 10% Chronic renal faliure 2,膜性增生性肾小球肾炎,C3, IgG C1q, C4,C3,三-4)（六,发

9、病机制,Type I 循环免疫复合物 Type II 致密物沉积病 C3Nef (低补体血症,PAS stain,Silver stain,Silver stain,NPGN, Type II,临床表现及预后,肾病综合征 起病可为急性肾炎或轻微蛋白尿 预后差，II型更差 60 years 20 years follow-up Complete remission 0 End-stage renal faliure 40% Renal insufficiency 30% Persistent nephrotic syndrome 30,IgA肾病 Berger disease,青年和儿童多见 反

10、复发作的镜下和肉眼血尿 系膜区IgA沉积,四)（八,发病机制,Increased production,Reduced clearance,activation of the alternative complement pathway,IgA deposition,临床表现及预后,50%肉眼血尿，30-40% 镜下血尿，伴或不伴蛋白尿 5-10% 发展为典型的急性肾炎综合征 25-50% 病例20年缓慢进展为终末肾,慢性肾小球肾炎,Unfortunate outcomes of glomerular diseases 30-50% 病人需要透析或换肾 20% 无肾病史 大量肾小球玻璃样变和硬

11、化 严重的不可逆的瘢痕形成,五)（九,Hyaline obliteration of glomeruli Tubules are lost, vessel walls are thickened,Masson trichrome stain,临床病理联系,部分有明确肾病史，部分隐匿起病 慢性肾炎综合征 蛋白尿、高血压 多尿、夜尿、低比重尿 氮质血症、尿毒症 预后均很差,Clinical manifestations of renal disease,Azotemia and uremia Renal syndromes Acute nephritic syndrome Nephrotic sy

12、ndrome Asymptomatic hematuria or proteinuria Rapidly progressive glomerulonephritis Acute renal failure Chronic renal failure Urinary tract infection Nephrolithiasis (renal stones,急性肾盂肾炎 Acute pyelonephritis,化脓菌感染引起的肾盂、肾小管和肾间质的急性化脓性炎症 血源性感染 Hematogenous infection 上行性感染 Ascending infection,Predisposi

13、ng conditions,插管和膀胱镜 catheterization and cystoscopy 女性 Female，妊娠 Pregnancy 尿储留 Urine stasis 糖尿病 Diabetes mellitus 膀胱输尿管返流 Vesicoureteral reflux,膀胱输尿管返流,急性肾盂肾炎,急性肾盂肾炎,急性肾盂肾炎,急性肾盂肾炎,肾乳头坏死,临床表现,症状和体征 脊肋角疼痛 全身症状 脓尿和菌尿 膀胱尿道刺激症 (尿频尿急尿痛) 预后 自限性 有危险因素者可转为慢性 肾乳头坏死者预后差,慢性肾盂肾炎 Chronic pyelonephritis,A morphologic entity in which predominantly interstitial infl