Ultrastructural Changes Suggestive of Focal Segmental Glomerulosclerosis in Atypical Minimal Change Nephrotic Syndrome

In an attempt to recognize early stages of focal segmental glomerulosclerosis (FSGS) in patients with a clinical course suggesting a diagnosis other than minimal change disease (MCD) and normal histology, or minor, nondiagnostic changes on light microscopy (LM), we used a protocol for systematic and extensive electron microscopy (EM) examination of kidney biopsies obtained from such patients. By this method ultrastructural pathology was found in 8 patients. These changes were localized, involving only portions of single glomerular segments. The findings included mild to moderate increase of the mesangial matrix, focal wrinkling of the capillary basement membrane, and early obliteration of the normal architecture of individual capillary loops, as well as electron-dense deposits in a mesangial and subendothelial distribution. Of these 8 patients, 2 are at present in remission without therapy (in 1, following therapy with cyclophosphamide); 3 are in remission on steroid therapy; 1 developed massive proteinuria during pregnancy, after a spontaneous remission lasting almost 2 years; 1 patient advanced to terminal renal failure 3 1/2 years after biopsy; and 1 died of sepsis 1 month after biopsy. We believe that the ultrastructural changes found may represent early or mild FSGS and that the protocol described can add valuable information in clinically worrisome patients in whom renal histology appears normal.     

皮质激素和间断环磷酰胺冲击治疗局灶节段性肾小球硬化疗效观察

目的 观察激素及同断环磷酰胺(CTX)冲击治疗局灶节段性肾小球硬化-肾病综合征(FSGS-NS)的疗效。方法回顾性分析1993～1997年间,63例FSGS-NS患者对激素治疗的反应,疗效与病理的关系及随访结果。结果以NS为表现的]SGS占同期肾活检FSGS的43.45％,平均发病年龄(31±14)岁,男女之比为2.15∶1,平均随访43个月。对初治疗有反应的患者(Ⅰ组)完全缓解率(CR)高,为65.79％,无进展至慢性肾功能衰竭(CRF)者;对初始治疗无反应者(Ⅱ组)CR低,为12％,延长激素及CTX治疗可使CR或部分缓解率(PR)增加至48％,进展至CRF者为6.35％。治疗的反应及CR的高低与肾小球病变范围及肾小管间质病变严重程度有关,肾小球病变范围大,小管间质病变严重者,对初始治疗反应差,CR低。药物不良反应以感染和肝损为主。结论延长皮质激素及间断CTX冲击治疗FSGS-NS使NS的治疗缓解率增加(总CR44.44％),进展至CRF少(6.35％),患者预后改善,临床上应根据肾脏病理,在严密监测药物不良反应的情况下,对其积极治疗。     

灯盏花治疗椎-基底动脉缺血性眩晕64例

灯盏花治疗椎－基底动脉缺血性眩晕６４例吕云利，傅学锋，姚向荣，廖军，路逵自１９９５年１１月～１９９６年７月，我们用云南灯盏花注射液治疗椎一基底动脉缺血性眩晕（ＶＢＩＶ），并与传统中药丹参注射液作对照观察，疗效满意，报告如下。临床资料诊断接文献（中风与...     

儿童局灶节段性肾小球硬化

局灶节段性肾小球硬化（FSGS）近年来有增多趋势,FSGS不仅是一种形态学描述,而被视为一种临床病理综合征,表现为蛋白尿,常为肾病水平蛋白尿,并有局灶节段分布的肾小球硬化和足突融合。FSGS可为原发性（特发性）、继发性和遗传家族性。最近FSGS被区分为5种变异型,提示其不同的发病机制和预后,这5型包括特异FSGS、门周型、细胞型、顶端病变和塌陷型。该文还就FSCS的治疗和预后进行了讨论。     

Successful Treatment of Nephrotic Syndrome Due to Collapsing Focal Segmental Glomerulosclerosis Accompanied by Acute Interstitial Nephritis

A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.     

原发性局灶节段性肾小球硬化预后相关因素的研究进展

近年来局灶节段性肾小球硬化（FSGS）发病率增加,且治疗困难,预后较差,是导致终末期肾疾病的主要原因之一。肾脏病学家试图探索某些指标来拟诊和预测预后,该文简述了原发性FSGS预后相关因素如蛋白尿程度、血肌酐水平、肾小管间质病变、病理类型、治疗方法、治疗反应、基因、足细胞及足细胞蛋白等的研究进展。     

家族性局灶节段性肾小球硬化的基因研究

目的:探讨家族性局灶节段性肾小球硬化(familial focal segmental glomerulosclerosis, FFSGS)基因与染色体1q25-31区域的连锁关系.方法:1个中国皖北地区3代FFSGS家系,采集本家系中27名成员的外周血样,选择位于1q25-31上的10微卫星标志:D19S49、D1S452、D1S242、D1S416、D1S240、D1S254、D1S202、D1S222、D1S238和D1S413,应用聚合酶链式反应(PCR)得到扩增产物片断,采用ABI PRISMTM310 Genetic Analyze 测定 PCR产物片断大小.利用Genescan 软件( 3.1 版)、Genetyper ( 3.7版) 处理后得到检测片断大小,根据相应微卫星标记的产物片断大小不同,得到每个样本的基因型.对基因型数据进行校对后,用连锁分析软件 LINKAGE 的 MLINK 程序计算每个标记的两点间LOD(log odds)值.根据两点LOD值判断连锁关系.结果:连锁分析结果显示所有微卫星标记两点间LOD值在不同重组率时均小于0,所有LOD值在重组率为0.0时均小于-2,说明该FFSGS家系疾病基因与1q25-31区域没有连锁关系.结论:该家族疾病基因与已报道的FFSGS 定位区域(1q25-31区域)没有连锁关系.     

口服爱若华联合甲基强的松龙肾囊注射治疗原发性局灶节段性肾小球硬化9例报告

目的探讨新型免疫抑制剂来氟米特(商品名:爱若华)和肾囊注射甲基强的松龙联合治疗局灶节段性肾小球硬化症(FSGS)的疗效和安全性。方法从我院2002年前经肾活检患者中确诊为FSGS的16例患者中随机选取10例(均采用常规类固醇口服治疗)患者作为对照组,从2002年始所有肾穿刺确诊为FSGS的9例患者作为治疗组,采用口服爱若华,同时肾囊注射甲基强的松龙长期治疗1年。在治疗前及治疗6个月、1年后观察24h尿蛋白定量、血尿素(Urea)、血肌酐(Scr)、内生肌酐清除率(Ccr)等指标变化,按照疗效评定标准对比分析联合治疗实验组与常规治疗对照组的疗效和安全性。结果联合治疗组显著降低24h尿蛋白定量,其中治疗6个月蛋白尿完全缓解率为34.5%、部分缓解率为44.5%,明显高于对照组(17.5%、25.4%)(P<0.01);12个月完全缓解率累加为67%(对照组:29.8%),部分缓解率累加为21.3%(对照组:38.5%)(P<0.01)。疗程中未发现糖皮质激素的副作用,有2例发现一过性来氟米特致肝酶升高,减量和对症治疗后恢复正常。结论爱若华联合肾囊注射甲基强的松龙治疗FSGS较常规治疗有效,副作用少,值得进一步研究与观察。