泌尿系統(tǒng)教學(xué)課件：3泌尿病理

1、Urinary Syetem- Pathology The normal structureThe normal structure ( one nephron) ( one nephron) endothelial cells endothelial cells 內(nèi)皮細胞內(nèi)皮細胞 glomerular basementglomerular basement membranemembrane (GBM)(GBM) 基底膜基底膜 GlomusGlomus visceral epithelial cellsvisceral epithelial cells (podocytes)(podocyte

2、s) 臟層上皮細胞臟層上皮細胞 血管球血管球 mesangial cellsmesangial cells (2 / lobe)(2 / lobe) 系膜細胞系膜細胞 GlomeruliGlomeruli mesangial matrix:mesangial matrix: 系膜基質(zhì)系膜基質(zhì) has three functionshas three functions 腎小球腎小球 CapsuleCapsule parietal epithelial cells and its GBMparietal epithelial cells and its GBM 腎球囊腎球囊 壁層上皮細胞及其基底

3、膜壁層上皮細胞及其基底膜 proximal convoluted tublesproximal convoluted tubles Tuble Tuble looploop 腎小管腎小管 distal convoluted tublesdistal convoluted tubles A normal glomerulus by light microscopy. Endothelial and mesangial cells are normal in number. The surrounding tubules are normal. hypercellularity HE Staini

4、ng ： normal glomerulus The normal FM by electron microscopy. Shows endothelial cells, GBM , and podocyte. EM: FUSION OF FOOT PROCESSES Clinical manifestations： Changes of urine 1. oliguria (少尿）少尿）or anuria（無尿）（無尿） 2. polyuria（多尿），（多尿），nocturia（夜尿）（夜尿） 3. hematuria (血尿）血尿） 4. proteinuria (蛋白尿）蛋白尿） 5.

5、 cylindruria（管型尿）（管型尿） 1. nephrotic syndrome腎病綜合征腎病綜合征 2. nephritic syndrome腎炎綜合征腎炎綜合征 acute nephritic syndrome 急性腎炎綜合征急性腎炎綜合征 rapidly progressive nephritic syndrome 快速進行性腎炎綜合征快速進行性腎炎綜合征 chronic nephritic syndrome 慢性腎炎綜合征慢性腎炎綜合征 3. occult nephritis syndrome 隱匿性腎炎綜合癥隱匿性腎炎綜合癥 4. recurrent or continuou

6、s haematuria 反復(fù)發(fā)作性或持續(xù)性血尿反復(fù)發(fā)作性或持續(xù)性血尿 5. renal failure 腎功能衰竭腎功能衰竭 6. uremia 尿毒癥尿毒癥 Systemic changes: 1. renal edema 2. renal hypertension 3. creatinine (肌酐值）肌酐值） 4. Renal anemia 1. diffuse endocapillary proliferative GN 2. rapidly progressive GN / diffuse cresentic GN /diffuse extracapillary prolifera

7、tive GN 3. membranous GN / mbranous nephropathy 4. minimal change GN / lipoid nephrosis 5. focal segmental GN 6. membranoproliferative GN 7. diffuse mesangial proliferative GN 8. IgA nephropathy 9. chronic GN diffuse or focal; global or segmental The rules of nomenclature lIn the kidney： 50% glomeru

8、li involved Diffuse: Focal: lIn one glomerulus： Global: Segmental: lInner or outer of GBM: Endocapillary : Extracapillary ： lProliferation or not: 50% 內(nèi)皮細胞內(nèi)皮細胞 系膜細胞系膜細胞 上皮細胞上皮細胞 Inner or outer of GBM: Endocapillary : 毛細血管內(nèi)毛細血管內(nèi) Extracapillary ：毛細血管外：毛細血管外 Renal biopsy- 1.the changes of alteration 2.

9、 the changes of exudation 3. the changes of proliferation 4. the changes of tubulus and interstitial HE染色、染色、 PAS 染色、過碘酸六安銀染色、過碘酸六安銀(PASM)染色、染色、Masson三色染色三色染色 免疫熒光免疫熒光: IgA IgG IgM C3 C1q Fibrin 透射電鏡透射電鏡 6. Proteinuria and Nephrotic Syndrome What is Proteinuria? What is Proteinuria? Normal: 1-2g/24h

10、: glomerular disease 3.5g/24h: nephrotic syndrome 1.proteinuria (3.5g/day), 2.hypoalbuminemia (30g/L) 3.edema 4.hyperlipidemia Etiology oIn children nMinimal change disease is predominant oIn adults nSystemic disease related: 30% nPrimary renal disorders: 70% oMembranous nephropathy oFocal glomerulo

11、sclerosis oMinimal change disease oAmyloidosis oIn elderly nIncreased incidence of amyloidosis and decreased incidence of SLE Causes of NS in Children Primary glomerular disease (95%) MCD: 65% FSGS: 10% MPGN: 10% MGN: 5% Other proliferative GN: 10% Systemic disease (5%) Causes of NS in Adult Primary

12、 glomerular disease (70%) FSGS: 35% MGN: 30% MCD: 10% MPGN: 10% Other proliferative GN: 15% Systemic diseases (30%) Diabetes; SLE; Amyloidosis; Drugs; Infections; Malignancy Minimal change disease (Minimal Change GN) Characteristic: 1. it is characterized by diffuse loss of foot processes of epithel

13、ial cells in glomeruli that appear normal by light microscopy. 2. children : 26 year-old children 3. are the most common cause of the nephrotic syn. in children. Etiology and Pathogenesis an immunologic disease? gene mutation? Pathological changes Grossly: Light M: 1. the glomeruli are normal by lig

14、ht microscopy. 2. the cells of proximal tubules are often laden with lipid. (lipoid nephrosis) Immunofluorescence M: negative Minimal Change Disease Minimal Change Disease Electron M: 1.GBM is normal, no electrondense deposits. 2.The principal lesion is in the visceral epithelial cells, which show a

15、 uniform and diffuse effacement of foot processes, termed “fusion” or loss of foot processes. (foot process disease) Minimal Change Disease EM: FUSION OF FOOT PROCESSES This is minimal change disease which is characterized by effacement of the epithelial cell (podocyte) foot processes and loss of th

16、e normal charge barrier such that albumin selectively leaks out and proteinuria ensues. Clinical presentations: nephrotic syndrome： proteinuria; edema; hypolbuminemia; hyperlipidemia and lipiduria Prognosis not bad, because it is usually dramatic response to corticosteroid therapy. (membranous nephr

17、opathy) Membranous GN Introduction l 1. Is the most common cause of the nephrotic syn. in adults. l 2. Diffuse thickening of cap. wall and the accumulation of electron-dense. Etiology and pathogenesis Ag-Ab-mediated disease Pathological changes Grossly: enlarged , pale kidney Light M: 1. Uniform dif

18、fuse thickening of glomerular cap. wall . 2. sclerosis of glomeruli. Ehrenreich-Churg Staging System for MGN I: GBM normal in thickness with no/minimal spikes II: Spikes extending between deposits; +/- broadening of the end of spikes III: Electron-dense deposits fully enclosed by GBM material and in

19、corporated within a thickened GBM IV: Deposits incorporated within the GBM and become rarified Membranous Glomerulonephropathy-LM A silver stain. There are characteristic spikes seen with membranous GN seen here in which the black basement membrane material appears as projections around the capillar

20、y loops. Membranous Glomerulonephropathy-LM Membranous GN: Uniform diffuse thickening of glomerular cap. wall . Immunofluorescence M: IgG,C3,IgG,C3,granular deposits on surface of the GBM. Membranous Glomerulonephropathy-IF Granular capillary wall staining (fine to coarse) for Ig and complement IgG

21、(100%), C3 (75%), IgM and IgA (minority) Mesangial deposits (-). If prominent, suggestive of lupus Membranous GN is an immunologically mediated disease in which deposts of mainly IgG and C3 collect in the GBM and appear in a diffuse granular pattern by immunofluorescence, as seen here. Electron M: t

22、hickening is caused by irregular dense deposits. Four stages: By EM in membranous GN, the darker electron dense immune deposits are seen scattered within the thickened GBM. The spikes represent the intervening matrix of GBM between the deposits. Clinical presentations: nephrotic syndrome proteinuria

23、; edema; hypolbuminemia; hyperlipidemia and lipiduria Prognosis: 10% die or progressive to renal failure within 10 years; 50% cases: gross hematuria; 30-40% have only microscopic hematuria; or/and proteinuria. 8. Diabetic Nephrophaty and Chronic Kidney Disease Nodular formationNodular formation：PAS

24、positivePAS positive，K-W nodulesK-W nodules Diabetic Nephrophaty Hyaline thickening of arteriolar wall Fibrosis of the glomeruli DN - IF Linear stain along the GBM DN - EM Homogenized thickening of GBM 9. End-Stage Kinday (Chronic GN) Introduction end-stage kidney Pathological changes: Grossly: “Gra

25、nular-contracted kidney” 1. symmetrically contracted and have diffusely granular surface. 2.The cortex is thinned,and there is an increase in peripelvic fat. Microscopically: complicated 1, fibrosis and hyalinization of glomeruli; 2, marked atrophy of associated tubules; 3, irregular interstitial fi

26、brosis; 4, lymphocytic infiltration; 5, arteriolar sclerosis; 6, the other nephrons are hypertrophy. Fibrosis of glomeruli CGNCGN Clinical presentations: chronic renal failure 1, diuresis, 2, anemia 3, hypertention 4, azotemia 5, uremia Prognosis if not maintained with continued dialysis or receive

27、a renal transplant, the outcome is invariable death. Pyelonephritis Urinary Tract infection Definition Pyelonephritis is a suppurative inflammation affecting tubules, interstitium, and renal pelvis . it is caused by pyogenic bacteritic infection directly (one of the most common disease of the kidney

28、) Etiology and Pathogenesis Microorganism gram-negative bacilli that are normal inhabitants of the intestinal tract. By far the most common is Escherichia coli (大腸桿菌大腸桿菌). Acute : one type of B. infection. Chronic : is more complex. Mechanisms: two routes: 1. hematogenous inf. -less common bacteria

29、reach the kidney through the bloodstream. 2. ascending inf. -most common bacteria come from the lower urinary tract. Inducements: (1) urinary obstruction（pregnancy） (2) instrumentation (使用儀器使用儀器) of the urinary tract (3) vesicoureteral ruflux (膀胱輸尿管反膀胱輸尿管反 流流) (4) immuno deficiency Acute pyelonephri

30、tis Definition is an acute suppurative inflammation(化化 膿性炎膿性炎) of the kidney caused by B. inf. 1. interstitial suppurative inflammation 2. tubular necrosis. Pathological changes Grossly: focal abscesses involving one or both kidneys or as large, wedge-shaped areas of coalescent suppuration. focal ab

31、scesses on the surface of the kidney. Microscopically: 1.early stage: the neutrophilic infiltration is limited to the interstitial tissue. 2. then: abscess focus destruct the tubules. 3. finally: the glomeruli may be implicated. acute pyelonephritis This is an ascending bacterial infection leading t

32、o acute pyelonephritis. At high magnification, many neutrophils are seen in the tubules and interstitium in a case of acute pyelonephritis. Clinical presentations: pain, fever, and malaise. Bladder irritation(激惹激惹): dysuria, frequency and urgency. Syndromes 1. papillary necrosis 壞死性乳頭炎壞死性乳頭炎 2. pyon

33、ephrosis 腎盂積膿腎盂積膿 3. perinephric abscess 腎周圍膿腫腎周圍膿腫 Prognosis: is a benign course. acute pyelonephritis Chronic pyelonephritis Introduction: is a chronic tubulointerstitial renal disorder. can be divided into two forms: chronic obstructive chronic reflux-associated : more common Pathological changes

34、 Grossly: kidneys are usually large, wedge-shaped areas irregularly scarred, asymmetric(不對稱不對稱). 大體：瘢痕凹陷性固縮腎大體：瘢痕凹陷性固縮腎 瘢痕瘢痕 凹陷凹陷 Microscopically: 1. Tubules show : a. Atrophy in some areas and hypertrophy in others. b. Dilation. Dilated tubules may be filled with colloid casts (thyroidization). 2.

35、Interstition: chronic inf. and fibrosis in the cortex and medulla. 3. Glomeruli: may normal. But in severe cases, may fibrous obliteration. The large collection of chronic inflammatory cells here is in a patient with a history of multiple recurrent urinary tract infections. This is chronic pyeloneph

36、ritis. 腎小球纖維化腎小球纖維化 玻璃樣變玻璃樣變 管型管型 慢慢 性性 腎腎 盂盂 腎腎 炎炎 腎小管管腔內(nèi)蛋白腎小管管腔內(nèi)蛋白 管型管型, ,似甲狀腺濾泡似甲狀腺濾泡 慢慢 性性 腎腎 盂盂 腎腎 炎炎 Clinical presentations: 1. loss of tubular function- concentrating ability polyuria, nocturia. 2. bacturia may be present, but often absent in the late stage. 3. hypertension , heart failure 4.

37、 azotemia, renal failure Prognosis: 13. Renal Masses and Urinary Tract Tumors Renal Mass Renal cell carcinoma Introduction account for 85% of renal cancers in adults, 13% of all visceral cancers. 6070 year-old man. M: F 2-3 : 1 arise in tubular epithelium also called renal adenocarcinoma Epidemiolog

38、y Risk factors: tobacco, obesity, petroleum products, heavy metals, and so on. Pathological changes Grossly: Nodular, might have a pseudo capsula, usually on the upper pole of the kidney; yellow colored, hemorrhagic and necrotic. spherical masses, 3-15cm 3-15cm in diameter, composed of bright yellow

39、-gray-white tissue that distort the renal outline. The cut surface demonstrates a variegated app- earance with yellowish areas, white areas, brown areas, and hemorrhagic red areas. This renal cell carcinoma is very large, as indicated by the 15cm ruler. A portion of normal kidney protrudes at the lo

40、wer center. This patient was a physician himself and just didnt have any early symptoms. Microscopically: Clear cell (nonpapillary) carcinoma: Papillary carcinoma: Chromophobe renal carcinoma: This is the classic histologic appearance of a renal cell carcinoma. This appearance is why they are often

41、called clear cell carcinomas. Clear cell type Papillary type Chromophobe type Clinical presentations: three classic diagnostic features: pain, palpable mass (可觸及的包塊可觸及的包塊), hematuria. Associated syn. Metastases Hematogenic metastasis: early stage , lung, bone, liver, adrenal gland, brain Prognosis T

42、ransitional cell carcinoma of the bladder Introduction is the most common cancer occurred in urinary tract and account for 90% of all bladder cancers. 5070 year-old man. M: F 3 : 1 arise in transitional epithelium Epidemiology Cigarette smoking; industrial exposure to arylamines(芳基胺芳基胺) Pathological

43、 changes Grossly: Usually it is a papillary tumor with slender or broad pedicle, sometime it show cauliflower-like or polypous, sometime it is flat. invasive or noninvasive The key is to decide whether the tumor has invaded the underline tissue or not and how deep is it. A male who had a long histor

44、y of smoking. He had presented with hematuria. The opened bladder reveals masses of a neoplasm that histologically proved to be transitional cell carcinoma (TCC). Microscopically: WHO classification grades tumors into a rare totally benign papilloma and three TCC: grade 0 : : papilloma grade, , : : 1. grade : Low malignancy, the papilla is covered by orderl