Nephrology Unit, Department of Clinical Medicine and Prevention, University of Milano-Bicocca, Ospedale San Gerardo, Via Pergolesi 23, Monza, Italy.
J Nephrol. 2010 Nov-Dec;23(6):717-24.
Only few cases of acute renal failure (ARF) requiring dialysis have been reported in patients with idiopathic nephrotic syndrome (NS). This study aims to better define the clinical outcome and treatment of this condition.
A pilot enquiry regarding the occurrence of ARF requiring dialysis in patients with NS and biopsy proven minimal changes (MC) or focal segmental glomerulosclerosis (FSGS) was conducted among 5 nephrology centers.
From 1996-2006, 6 patients with idiopathic NS (4 MC, 2 FSGS) developed ARF requiring dialysis early after onset of NS. At presentation all but 1 patient had elevated blood pressure. Patients were treated with dialysis from 7-40 days. All achieved complete or partial remission after 4-8 weeks of steroids. Recovery of renal function paralleled with the reduction of proteinuria. At renal biopsy proximal tubules showed a large amount of protein droplets, flattening of epithelial cells, and focal detachment of cells from the basal membrane. After a follow-up of 24-60 months, 5 patients had a relapse. Of these 4 were responsive to steroids, while one progressed to dialysis after an episode of hemolytic uremic syndrome related to cyclosporine treatment. ARF did not recur.
ARF requiring dialysis is a rare and unexpected complication of idiopathic NS occurring in most cases early after presentation. These patients are sensitive to steroids that should be administered as promptly as possible in view of the potential noxious effect of protein overload on proximal tubular cells.
仅有少数几例特发性肾病综合征（NS）患者出现需要透析的急性肾衰竭（ARF）的报道。本研究旨在更好地定义这种疾病的临床结局和治疗方法。
在 5 个肾病中心进行了一项关于 NS 患者出现需要透析的 ARF 的病例调查，这些患者的病理活检结果证实为微小病变（MC）或局灶节段性肾小球硬化（FSGS）。
1996 年至 2006 年期间，6 例特发性 NS（4 例 MC，2 例 FSGS）患者在 NS 发病后早期出现需要透析的 ARF。在就诊时，除 1 例患者外，所有患者的血压均升高。患者接受透析治疗 7-40 天。所有患者在接受类固醇治疗 4-8 周后均完全或部分缓解。肾功能的恢复与蛋白尿的减少平行。在肾活检中，近端小管显示大量蛋白滴、上皮细胞变平以及细胞从基底膜局部脱落。在 24-60 个月的随访中，5 例患者复发。其中 4 例对类固醇有反应，而 1 例在环孢素治疗相关的溶血尿毒综合征发作后进展为透析。ARF 未再复发。
需要透析的 ARF 是特发性 NS 的一种罕见且意外的并发症，在大多数情况下，这种并发症在发病后早期出现。这些患者对类固醇敏感，鉴于蛋白过载对近端肾小管细胞的潜在有害影响，应尽快给予类固醇治疗。
