Corticosteroid treatment exacerbates nephrotic syndrome in a zebrafish model of magi2a knockout

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局灶节段性肾小球硬化患者常见并发症的分析

目的:观察局灶节段性肾小球硬化(FSGS)患者急性肾损伤(AKI)、感染及血栓栓塞的发生率,探讨并发症发生的危险因素。方法:观察70例经临床及肾脏病理确诊的特发性FSGS患者的AKI、感染及血栓栓塞的发生率,分析并发症的临床特征及高危因素。结果:(1)70例特发性FSGS中,34例(48.6%)并发AKI、14例(20.0%)合并感染、11例(15.7%)出现血栓栓塞。(2)AKI以男性多见,其尿蛋白水平较高,血白蛋白水平较低,小管间质损伤更重,小管急性损伤的程度与AKI的分期有关;随访过程中AKI患者完全缓解率及有效率显著低于未合并AKI者。(3)感染以呼吸道感染多见,院内感染占50%;与非感染组相比,感染组患者的尿蛋白水平更高,血白蛋白、球蛋白、IgG水平则较低;随住院时间延长,感染发生率升高,院内感染所占比例增加;Logistic回归分析显示白蛋白低于20g/L和住院时间延长是感染的独立危险因素。(4)血栓栓塞包括颅内静脉窦血栓2例、颈内静脉血栓1例、右肾静脉血栓1例、股静脉血栓2例、腘静脉血栓2例和肺栓塞3例;血栓栓塞患者的循环内皮细胞计数(CECs)、血管性血友病因子(vWF)高于未合并血栓栓塞者,Logistic回归分析显示CECs和血红蛋白水平升高与血栓栓塞独立相关。结论:FSGS患者肾小管损伤、低白蛋白血症和住院时间延长、血管内皮细胞损伤和血液浓缩分别在AKI、感染、血栓栓塞的发生中起重要作用。     

Effect of post-transplant double filtration plasmapheresis on recurrent focal and segmental glomerulosclerosis in renal transplant recipients

In the present study, we reviewed the effect of post-transplant double filtration plasmapheresis (DFPP) on recurrent focal segmental glomerulosclerosis (FSGS) in the transplanted kidney allograft. Sixteen patients with post-transplant recurrent FSGS were enrolled in this study. Out of 16 patients with recurrent FSGS after transplantation, five did not receive DFPP and lost their grafts, while 11 did receive DFPP and four of these patients lost their grafts. Seven patients were able to maintain normal renal function for an average observation period of 57.1 +/- 40.7 months (range 7-125 months). In five patients who had a significant reduction in urinary protein after DFPP, the urinary protein level decreased from 26.60 +/- 23.05 g/day (range 3.34-62.6 g/day) to 2.95 +/- 3.42 g/day (range 0.02-8.64 g/day) and renal function was maintained. The beneficial effects of DFPP on graft outcome were more likely to occur if the patients experienced a marked drop in urinary excretion. Thus, post-transplant DFPP appears to be effective for reducing urinary protein levels and improving long-term graft survival. With the small numbers in this trial, however, none of the findings were statistically significant. We recommend the use of post-transplant DFPP to prevent the progression of recurrent FSGS.     

End-Stage Renal Disease and Mortality Outcomes Across Different Glomerulonephropathies in a Large Diverse US Population

Objective

To compare renal function decline, incident end-stage renal disease (ESRD), and mortality among patients with 5 common glomerular diseases in a large diverse population.

Focal and segmental glomerulosclerosis in murine models: a histological and ultrastructural characterization with immunohistochemistry correlation of glomerular CD44 and WT1 expression

Aim: Focal segmental glomerulosclerosis (FSGS) is a common progressive chronic renal disease. Podocyte injury and loss are the postulated pivotal events that trigger FSGS. In this study, the authors aim to examine the evolution of FSGS in murine models histologically, ultrastructurally and immunohistochemically with special emphasis on podocytes and parietal epithelial cells (PECs). Material and methods: FSGS resembling primary FSGS in humans was initiated in Wistar rats using intravenous Adriamycin injections. Blood and urine analysis were performed at 0, 8, and 12 weeks. Both the control kidneys and the test kidneys were harvested at 8 and 12 weeks, examined histologically and ultrastructurally and the findings correlated with the glomerular expression of immunostains specific for podocytes (WT-1) and for activated PECs (CD44). Results: FSGS developed in both 8 and 12 weeks test groups showing progressive proteinuria, podocytopathy and segmental glomerular scarring. There was a decrease in the glomerular expression of WT-1 with a concurrent increase in the glomerular expression of CD44, indicating podocyte loss with synchronous increase in activated PECs. The evolving FSGS correlated negatively with podocytes and positively with activated PECs. Conclusion: Our study shows that with podocyte injury there is podocyte effacement and loss, proteinuria, glomerular segmental adhesion and scarring, all culminating in FSGS. In addition, there is activation, hyperplasia and hypertrophy of PECs. This demonstrates that both podocyte loss and PEC activation promote FSGS. Our findings are consistent with recent investigations. More studies are required to further understand the role of these cells in the evolution of FSGS and subsequently introduce new targeted treatment modalities.     

Strumpell's Disease in a Family with Hereditary Focal Segmental Glomerulosclerosis

Strumpell's familial spastic paraplegia is a rare hereditary disease, clinically characterized by progressive disturbance of gait. Focal Segmental Glomerulosclerosis (FSGS) is a frequent glomerulopathy, with an extremely rare familial subtype. The cases of two brothers with Strumpell's disease are reported, who also developed glomerular renal disease, most probably familial FSGS. The genetics of the two disorders, Strumpell's paraplegia and familial FSGS, are discussed, together with the possibility of a parallel transmission.     

来氟米特联合泼尼松治疗表现为肾病综合征局灶节段性肾小球硬化的临床观察

目的观察来氟米特联合泼尼松治疗表现为肾病综合征局灶节段性肾小球硬化（focalsegmental glomerulosclerosis,FSGS）患者的疗效及安全性。方法将60例局灶节段性肾小球硬化肾病综合征患者随机分为2组：来氟米特（1eflunomicle,LEF）治疗组30例、环孢素（cyclosporinA,CsA）组30例。LEF组开始治疗后,前3d予LEF50mg／d,3d后改为维持剂量30mg／d）CsA组予以CsA1．5～2．5mg·kg-1·d-1,根据血药浓度逐渐增加剂量。2组患者同时使用泼尼松0．5mg·kg-1·d-1,并逐渐减量。观察治疗前、治疗后第4、8、12周及第6、12个月的24h尿蛋白定量、肝功能、肾功能、血脂等临床指标以及不良反应。结果LEF组30例患者均完成了一年的治疗观察,CsA组30例患者有一例因药物副反应退出治疗,其余29例患者完成了治疗。2组患者治疗后24h尿蛋白定量明显减少,血白蛋白升高,与治疗前相比,差异均有统计学意义（P〈O．05）。LEF组治疗后第6个月时总有效率70．00％（21／30）,治疗后第12个月时总有效率76．67％（23／30）;CsA组治疗后第6个月时总有效率75．86％（22／29）,治疗后第12个月时总有效率68．97％（20／29）,2组比较差异无统计学意义（P〉0．05）。LEF组不良反应轻微,患者耐受性良好。结论LEF是治疗表现为肾病综合征的FSGS患者的一种安全有效药物。