关键词: collapsing focal segmental glomerulosclerosis cyclosporin low-density lipoprotein apheresis nephrotic syndrome steroid

来  源:   DOI:10.2169/internalmedicine.8258-21

Abstract:
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.
摘要:
一名39岁的妇女因肾病综合征住院。实验室检查结果显示血清肌酐水平和尿排泄β-2-微球蛋白增加,和N-乙酰-β-D-氨基葡萄糖苷酶。肾活检显示局灶性节段肾小球硬化(FSGS)塌陷和急性间质性肾炎。尽管使用脉冲类固醇治疗,然后口服大剂量糖皮质激素和环孢菌素,重度蛋白尿持续存在。开始低密度脂蛋白单采(LDL-A)治疗后,她的蛋白尿逐渐减少,导致完全缓解。治疗后重复肾活检未发现肾小球塌陷。应立即进行LDL-A以治疗对其他治疗反应不佳的FSGS塌陷病例。
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