临床测试1

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一个52岁的白人,具有轻度高血压和烟草的历史,使用了几个月的下肢肿胀。评估确认了24小时收集,广义水肿和1.8g / dl的血清白蛋白中12克蛋白尿的肾病综合征。他的血清肌酐是1.33mg / dl,EGFR为56ml / min。血清补体水平是正常的,ANA阴性和乙型肝炎和C血清素阴性。他略微贫血。WBC计数为6200,血小板计数为270,000 /μL。尿液沉积物显示6-10个RBCS / HPF。进行肾脏活检。什么是最可能的诊断?
新月状肾小球肾炎
不正确的。
最小的变化疾病
不正确的。
局灶节段性肾小球硬化(FSGS),尖端病变变异
正确的!石蜡切片含有保存完好的肾皮质,每张切片有48个肾小球。每节段有多达10个肾小球出现节段性硬化,局限于肾小球尖端,即近端小管起源的鲍曼囊区域(见下图A中的箭头)。一些尖端病变包含泡沫细胞(见箭头尖端)。大多数肾小球增大,但在光镜下无明显变化(见图B)。免疫荧光未发现免疫复合物沉积。电镜显示弥漫性足突消失,肾小球基底膜正常,无电子致密免疫型沉积(下图C)。肾小球尖端病变,虽然在哥伦比亚分类中被归类为FSGS的一种变体,但在临床上更类似于微小变异性疾病;孤立性肾小球尖端病变(无其他光镜异常,无免疫复合物沉积)的患者常表现为类固醇治疗反应性肾病综合征。其他形式的FSGS(门周、塌陷、细胞性和非特异性[NOS]变异体)更常见的是类固醇抵抗,预后更差。 Glomerular tip lesion is classified as a form of FSGS because, unlike minimal change disease, not all glomeruli are normal on light microscopy; some of them show segmental sclerosis/adhesion at the origin of the proximal tubule (the glomerular tip). Immune complex deposits are absent and there is widespread foot process effacement. The definition of the tip lesion vary between investigators; according to the Columbia classification of FSGS, up to 25% of the glomerular tuft can be obliterated by sclerosis at or close to the vascular pole. Others consider less extensive segmental lesions as true tip lesions. We believe that the glomerular tip lesion is a nonspecific reaction of the glomerulus that can be seen in a variety of glomerular diseases with heavy proteinuria, including minimal change disease and FSGS, but also membranous glomerulopathy and diabetic nephropathy. The pathogenesis of the tip lesion is unclear, but it is possible that it is related to herniation of the glomerular capillary tufts into the origin of the proximal tubules, podocyte injury, and subsequent adhesions of the glomerular capillaries to the glomerular tip. As pointed out above, most patients with isolated glomerular tip lesions and heavy proteinuria will respond to steroids just like minimal change disease. Steroid-resistant patients frequently develop progressive FSGS. Unfortunately, there are no consistent markers to predict which patient with glomerular tip lesion will behave like minimal change disease and which will have progressive FSGS.
IgA肾病伴继发性FSGS
不正确的。

浏览有关的分期付款FSGS尖端病变变异(免费提供),请浏览伟德是哪里的公司肾脏病理学的图谱II在ajkd.org。

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