病理学——泌尿系统

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HypertensionDiabetesChronic infection 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 disease,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 puncture,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 damage 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,PathologyClinical 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),OliguriaAnuricAzotemia,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) Ag in situAction 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,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-upComplete remission 71%Persist proteinuria 10%Chronic renal faliure 2%,膜性增生性肾小球肾炎,C3, IgGC1q, C4,C3,(三-4)（六）,发病机制,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-upComplete remission 0End-stage renal faliure 40%Renal insufficiency 30%Persistent nephrotic syndrome 30%,IgA肾病 Berger disease,青年和儿童多见反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 diseases30-50% 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化 严重的不可逆的瘢痕形成,(五)（九）,Hyaline obliteration of glomeruliTubules 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 uremiaRenal syndromes Acute nephritic syndromeNephrotic syndromeAsymptomatic 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,Predisposing 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 inflam