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1、浙医二院 肾内科 胡颖,肾病综合征Nephrotic Syndrome,诊断和鉴别诊断,肾病综合征的诊断思路,步骤是不是肾病综合征?,Diagnostic standard,Marked proteinuria (albuminuria) 3.5 g/24hrHypoalbuminemia 30g/L EdemaHyperlipidemiaNot a disease but a symptom complex,步骤:排除继发性肾病综合征,感染,细菌性: 链球菌感染后肾炎 细菌性心内膜炎肾炎等病毒性: 乙肝相关性肾炎 有HBV感染的血清学证据; 有肾病综合征的临床表现; 肾组织有乙肝病毒抗原的沉
2、积寄生虫,狼疮性肾炎(lupus nephritis, LN),美国风湿病学会(ACR)1997年1颊部红斑 2盘状红斑3光过敏4口腔溃疡5关节炎6浆膜炎 7肾脏病变 8神经病变9血液学疾病10免疫学异常11抗核抗体,糖尿病肾病,除肾病综合征之外,有多年的糖尿病病史或糖尿病的其他器管的微血管病变(如眼底病变、周围神经炎、心肌病、动脉硬化及冠心病等),肿瘤相关性肾病,骨髓瘤性肾损害 好发于中老年; 骨痛; 蛋白电泳M带; 尿轻链增高 骨髓象显示浆细胞异常增生淋巴瘤肾损害: 淋巴结肿大 发热、消瘦、肝脾肿大 骨穿和淋巴结活检其他实体瘤,肾淀粉样变,好发于中老年多系统累及(心、肾、消化道、皮肤和神经
3、)继发者常继发于慢性感染、结核、恶性肿瘤等肾脏体积增大肾活检确诊,过敏性紫癜性肾炎,除有大量蛋白尿外,如下特征可考虑: 最常见于儿童,青年斑点状紫癜,常见于臀部和下肢,常有腹痛和关节痛 多数紫癜后48周出现肾损害; 血小板计数多正常,遗传性疾病(Alport综合征),除肾病综合征特点外, 符合: (1)有明显的家族病史; (2)30%50%病人有神经性耳聋, 3%20%有眼 部异常表现; (3) 肾活检电镜下肾小球基膜的致密层分离,破碎并有电子致密颗粒沉积,毒素、药物及过敏,有蜜蜂刺伤、毒蛇咬伤、花粉过敏史,或注射白喉、百日咳、破伤风疫苗等病史长期接触和服用汞、有机金、青霉素、海洛因、丙磺舒、
4、巯甲丙脯酸、非甾体抗炎药、华法令、利福平、造影剂等药物,也可引起肾病综合征,步骤3 确定原发性肾病综合征病理类型,原发性肾病综合征的病理类型,微小病变型肾病 (minimal change disease ,MCD) 局灶性节段性肾小球硬化 ( focal segmental glomerulosclerosis, FSGS)系膜增生性肾小球肾炎 (mesangioproliferative glomerulonephritis)系膜毛细血管性肾小球肾炎 (membranoproliferative glomerulonephritis, MPGN)膜性肾病 (membranous glome
5、rulonephritis,MN),A normal glomerulus is shown diagramatically. The normal anionic charge barrier of slit pores in overlying podocyte cytoplasm prevents protein molecules such as albumin from passing through the endothelium. The normal mesangium contains about 2 to 4 mesangial cells, which have a ma
6、crophage-like function.,This is a normal glomerulus by light microscopy. The glomerular capillary loops are thin and delicate. Endothelial and mesangial cells are normal in number. The surrounding tubules are normal. Life is good.,This normal glomerulus is stained with PAS to highlight basement memb
7、ranes of glomerular capillary loops and tubular epithelium. The capillary loops of this normal glomerulus are well-defined and thin,This is IgA nephropathy (Berger disease). The IgA is deposited mainly within the mesangium, which then increases mesangial cellularity as shown at the arrow.,This immun
8、ofluorescence pattern demonstrates positivity with antibody to IgA. Note that the pattern is that of mesangial deposition in the glomerulus.,This is focal segmental glomerulosclerosis (FSGS). An area of collagenous sclerosis runs across the middle of this glomerulus. In contrast to minimal change di
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